PASTEST Flashcards
A 45-year-old man is admitted with right-sided abdominal pain and his investigations include an abdominal ultrasound scan. The ultrasound reveals right-sided hydronephrosis with a dilated right ureter.
Which of the following inflammatory processes might impinge upon the right ureter and cause obstruction?
1) Acute appendicitis
2) A perforated caecal carcinoma
3) Crohn’s disease affecting the terminal ileum
4) All of the above
5) None of the above
All of the above
As stated, given the ultrasound findings, it is important to consider acute appendicitis, Crohn’s disease and a perforated caeceal carcinoma as a possible cause of his symptoms, due to their close proximity anatomically to the right distal ureter.
Acute appendicitis
The appendix lies anterior to the right ureter. As such, acute appendicitis and associated inflammatory processes can cause hydronephrosis and a hydroureter.
A perforated caecal carcinoma
Inflammatory processes, including local and generalised peritonitis, could cause obstruction, as the caecum lies anterior to the right ureter. This question refers specifically to inflammatory processes causing obstruction. However, tumour bulk could also cause obstruction of the right ureter.
Crohn’s disease affecting the terminal ileum
Again, the terminal ileum is found just anterior to the right ureter, and inflammation of Crohn’s disease affecting the terminal ileum could cause obstruction.
None of the above
Although a calculus in the distal right ureter would be included in the differential diagnosis for this patient, local anatomy and associated pathologies must be considered, given the ultrasound findings. Anteriorly, the right ureter is related to the terminal ileum, caecum, appendix, and ascending colon and their mesenteries.
A 17-year-old male presents with fever, malaise and severe anorectal pain with swelling. This is on a background of six month history of diarrhoea and weight loss.
What is the most likely diagnosis?
1) Intersphincteric abscess
2) Ischiorectal abscess
3) Necrotising fasciitis
4) Perianal abscess
5) Supralevator abscess
Explanation
Perianal abscess
The most common type of anorectal sepsis is a perianal abscess (60%), and it is more commonly seen in men than women. Crohn’s disease is associated with an increased incidence of anorectal sepsis.
Intersphincteric abscess
Intersphincteric abscesses only account for a small number (around 5%) of anorectal abscesses.
Ischiorectal abscess
Ischiorectal abscesses account for around 20% of anorectal abscesses. Therefore, a perianal abscess is more likely, given the clinical scenario.
Necrotising fasciitis
If untreated, perianal sepsis can lead to necrotising fasciitis, so prompt incision and drainage of abscesses is required. However, there are no features of necrotising fasciitis described in the question stem. Necrotising fasciitis is a surgical emergency caused by bacterial infection of the fascia and subsequent necrosis of the subcutaneous tissues. It requires emergency debridement of affected areas with wide margins and intravenous antibiotics. Even with prompt treatment, mortality is still approximately 25–35%.
Supralevator abscess
Supralevator abscesses can develop from abdominopelvic pathology and can produce lower abdominal or rectal pain.
A 3-week-old boy presents with vomiting, jaundice and dehydration. Investigations reveal hypokalaemia and metabolic alkalosis.
What is the most appropriate initial management?
1) Correction of metabolic derangements
2) Feeding jejunostomy
3) Ramstedt’s pyloromyotomy
4) Total parenteral nutrition
5) Upper GI endoscopy
Explanation
Correction of metabolic derangements
The classical electrolyte abnormality of infantile pyloric stenosis (IHPS) is hypokalaemic hypochloraemic alkalosis. Surgery should be undertaken only after careful correction of the abnormalities, with the consult of an experienced paediatrician and anaesthetist.
Feeding jejunostomy
This would not be appropriate initial management of IHPS. Furthermore, pyloromyotomy is required as a curative procedure.
Ramstedt’s pyloromyotomy
This is the definitive surgical procedure required. However, correction of electrolytes before surgery is paramount. Of note, the umbilicus should be excluded from the operative field because of the risk of Staphylococcus aureus infection.
Total parenteral nutrition
Correction of electrolyte abnormalities is required as initial management. Given the significant electrolyte derangements, total parenteral nutrition would be challenging and ill-advised.
Upper GI endoscopy
The tumour is most commonly diagnosed clinically as a palpable tumour on test feed, alongside a history of projectile vomiting and hungry feeding, with no bile in the vomitus. This prevents the need for a diagnostic endoscopy, while correction of electrolyte abnormalities should be the first priority.
The incidence of IHPS is 3–4/1000 (1 in 300–900) live births. It is more common in males and has a slightly higher incidence in first borns. If the mother had IHPS, the risk is 20%, and if the father had IHPS, the risk is 5%. The pylorus is increased in length and diameter with hypertrophy of the circular muscle layer and autonomic nerves. There may be jaundice in 5–10% of cases due to a reduction of glucuronyl transferase.
A 60-year-old gentleman with a past history of atrial fibrillation attends the Emergency Department, complaining of a 3-day history of progressively worsening generalised abdominal pain and bloody diarrhoea. He has had intermittent colicky postprandial abdominal pain for the last 6 months. There are no exacerbating factors, and his weight has gradually declined, presumably due to reduced oral intake from apprehension to eat. Clinical examination reveals sinus tachycardia and hypotension. He has severe generalised abdominal pain and distension, but no specific tenderness. What is the most likely diagnosis?
1) Acute diverticulitis
2) Gastrointestinal ischaemia
3) Perforated viscus
4) Ruptured abdominal aortic aneurysm
5) Ulcerative colitis
Explanation
Gastrointestinal ischaemia
The history suggests a gastrointestinal cause, for which the most likely answer is gastrointestinal ischaemia, mostly likely ischaemic colitis. It frequently presents as severe abdominal pain that is out of proportion to the clinical signs, bloody diarrhoea and a significantly raised serum lactate level that is poorly responsive to fluid resuscitation. The concurrent atrial fibrillation is also a potential thromboembolic source that may trigger such acute ischaemia.
Acute diverticulitis
If the causative pathology were acute diverticulitis, signs of sepsis would be expected. Additionally, diverticulitis presents with localised abdominal pain, usually the left iliac fossa, as diverticular disease most commonly affects the sigmoid colon.
Perforated viscus
A perforated viscus is merely likely to present with localisable signs and symptoms. For example, a perforated gastric ulcer would produce localised tenderness and guarding within the upper abdomen. Moreover, the history of colicky postprandial pain and atrial fibrillation is suggestive of abdominal ischaemia.
Ruptured abdominal aortic aneurysm
A ruptured abdominal aortic aneurysm is unlikely, as this patient has no risk factors suggesting the diagnosis.
Ulcerative colitis
Although ulcerative colitis is a possible cause, in the acute setting, it is more likely in a younger individual.
A 70-year-old gentleman undergoes a difficult laparoscopic cholecystectomy where the cystic duct is found to be wide. Before this procedure, he had undergone an endoscopic retrograde cholangiopancreatography (ERCP) and a sphincterotomy for common bile duct stones. In the post-operative period, he develops a subdiaphragmatic collection which is drained. The fluid is bile, and so a stent is placed within the common bile duct by ERCP. Within 24 hours, he becomes tachycardic and hypotensive, passes black stools and develops severe abdominal pain.
What is the most likely diagnosis?
1) Acute pancreatitis
2) Enterocutaneous fistula
3) Gastrointestinal haemorrhage
4) Small bowel obstruction
5) Small bowel perforation
Gastrointestinal haemorrhage
Division of the sphincter of Oddi with sphincterotomy may cause pancreatitis, duodenal perforation or bleeding. The black stools suggest the passage of melaena, and therefore upper gastrointestinal bleeding post-ERCP.
Acute pancreatitis
Pancreatitis is one of the most common complications post-ERCP, with an incidence of around 20%, and can also present with severe abdominal pain (localised to the epigastric region, with radiation to the back), hypotension and tachycardia. However, black stools are not consistent with acute pancreatitis.
Enterocutaneous fistula
An enterocutaneous fistula would not cause the passage of black stools.
Small bowel obstruction
Gallstone ileus as a form of small bowel obstruction post-ERCP is rare. Again, passage of black stools is more indicative of upper gastrointestinal bleeding which would not be present in small bowel obstruction.
Small bowel perforation
Perforation of the duodenum (usually periampullary or ductal perforation due to sphincterotomy or guidewire manipulation) is a recognised complication of ERCP. However, it is rare, with a reported rate of 0.6%. Therefore, gastrointestinal haemorrhage would be more likely.
A 46-year-old male presents with an acutely painful left groin. He is tender over the affected area and you notice that his swelling originates inferior and lateral to the pubic tubercle.
What is the most likely diagnosis?
1) Direct inguinal hernia
2) Femoral hernia
3) Indirect inguinal hernia
4) Obturator hernia
5) Spigelian hernia
Explanation
Femoral hernia
Femoral hernias always arise inferior and lateral to the pubic tubercle.
Direct inguinal hernia
Inguinal hernias originate superior and medial to the pubic tubercle, not inferior and lateral. Although clinical assessment can classify inguinal hernias as direct (through weakness in the abdominal wall) or indirect (through the inguinal canal), inguinal hernias can only be truly classified into ‘direct’ or ‘indirect’ at operation, when their relation to the inferior epigastric artery can be observed (direct – medial, indirect – lateral).
Indirect inguinal hernia
As above, inguinal hernias originate superior and medial to the pubic tubercle. Obturator hernia
Obturator hernias are rare and usually found in women.
Spigelian hernia
Spigelian hernias originate at the linea semilunaris of the abdominal wall.
A 54-year-old woman presents to the Emergency Department with severe upper abdominal pain which is constant and localised to the right upper quadrant. She is tachycardic and pyrexial, with a positive Murphy’s sign. A diagnosis of cholecystitis is made. She is treated with antibiotics and is offered a ‘hot’ cholecystectomy.
Concerning this diagnosis and its treatment, which of the following statements is correct?
1) Boas’ sign is pain in the right upper quadrant which radiates to the right iliac fossa as inflammatory fluid tracks down the right pericolic gutter
2) Hartmann’s pouch may be found at the junction of the cystic duct and common hepatic duct
3) The cystic duct is a boundary of the cystohepatic triangle
4) The bile duct lies in the free edge of the greater omentum
5) Ultrasound is more sensitive than computerised tomography (CT) in the diagnosis of cholecystitis
Explanation
The cystic duct is a boundary of the cystohepatic triangle
The boundaries of the cystohepatic triangle (Calot’s) are the cystic duct, the common hepatic duct and the inferior surface of the liver. The triangle’s contents include the right hepatic artery, cystic artery, cystic lymph node, connective tissue and lymphatics.
Boas’ sign is pain in the right upper quadrant which radiates to the right iliac fossa as inflammatory fluid tracks down the right pericolic gutter
Boas’ sign is hypersensitivity below the right scapula and can also be caused by phrenic irritation.
Hartmann’s pouch may be found at the junction of the cystic duct and common hepatic duct
Hartmann’s pouch is found at the junction of the gall bladder neck and the cystic duct. It is a pathological variant where a stone may become impacted.
The bile duct lies in the free edge of the greater omentum
The common hepatic duct is anterior to the portal vein, and the bile duct runs in the free edge of the lesser omentum.
Ultrasound is more sensitive than computerised tomography (CT) in the diagnosis of cholecystitis
Although CT scanning has a higher sensitivity and specificity, when compared with ultrasound scanning, it is more expensive and cannot visualise non-calcified gallstones. Additionally, the patient is not exposed to ionising radiation.
A patient undergoes intramedullary nailing for a mid-shaft fracture of the tibia three hours ago. The ward nurses are concerned as he is in a lot of pain. On examination, he has no neurovascular deficit. He complains of excruciating pain on passive plantar flexion of the big toe, but not on passive dorsiflexion.
What is the likely diagnosis?
1) Compartment syndrome of the deep posterior compartment of the leg
2) Compartment syndrome of the superficial posterior compartment of the leg
3) Compartment syndrome of the anterior compartment of the leg
4) Compartment syndrome of the lateral compartment of the leg
5) Compartment syndrome of the medial compartment of the leg
Explanation
Compartment syndrome of the anterior compartment of the leg
In compartment syndrome, pain is worsened by passive stretching (ie extension) of the affected compartment. In this case, the muscle being stretched is the extensor hallucis longus. This muscle is in the anterior compartment which also contains the tibialis anterior, the extensor digitorum longus and the peroneus tertius. This is a surgical emergency, as increasing pressure within the compartment exceeds perfusion pressure, causing hypoxia and ischaemia. It requires urgent fasciotomy, in which all osseofascial compartments are opened.
Compartment syndrome of the deep posterior compartment of the leg
The deep posterior compartment of the leg contains the flexor hallucis longus, along with the flexor digitorum longus and the tibialis posterior.
Compartment syndrome of the superficial posterior compartment of the leg
The superficial posterior compartment contains the gastrocnemius, the plantaris and the soleus.
Compartment syndrome of the lateral compartment of the leg The lateral compartment contains the peroneus longus and brevis. Compartment syndrome of the medial compartment of the leg There is no medial compartment of the leg.
A 45-year-old man has been sent to the accident and emergency department by his general practitioner who suspects that he is suffering from cauda equina syndrome. There is no history of spinal surgery in the patient.
Which of the following clinical findings supports this diagnosis?
1) Magnetic resonance imaging (MRI) of the spine which reveals a disc prolapse at T11
2) There is a positive Babinski sign
3) The patient’s chief complaint is of a shooting pain radiating down the back of his legs
4) The patient is in urinary retention, with reduced anal tone and bilateral lower limb weakness
5) The patient has purely sensory loss in all of the lumbar dermatomes
The patient is in urinary retention, with reduced anal tone and bilateral lower limb weakness As above, the signs of cauda equina syndrome can include urinary retention, reduced anal tone and bilateral lower limb weakness. Following clinical assessment, investigation with an MRI spine would be appropriate and surgical decompression may be required.
Magnetic resonance imaging (MRI) of the spine which reveals a disc prolapse at T11
Cauda equina syndrome affects the cauda equina which is situated below the level of the termination of the cord, at around the L1/2 disc space. As such, the disc prolapse at T11 would cause compression of the spinal cord.
There is a positive Babinski sign
A positive Babinski sign represents an upper motor neurone defect.
The patient’s chief complaint is of a shooting pain radiating down the back of his legs
Below the conus medullaris, the spinal canal contains the cauda equina which branches off the lower end of the spinal cord and contains the nerve roots from L1 to 5 and S1 to 5. These roots from L4 to S4 join in the sacral plexus. Compression to this area results in cauda equina syndrome. Signs include weakness of the muscles of the lower extremities, detrusor weaknesses causing urinary retention and post-void residual incontinence. There may be decreased anal tone and faecal incontinence, saddle anaesthesia, bilateral leg pain and weakness and bilateral absence of ankle reflexes. Pain may be absent.
The patient has purely sensory loss in all of the lumbar dermatomes
Sensory loss can be present in cauda equina syndrome, but primarily in the sacral dermatomes (S4–5) affecting the perianal region.
A 56-year-old man presents to the Accident and Emergency Department with severe vomiting and chest discomfort. His chest X-ray shows air in the mediastinum.
Which one of the following disease processes may cause this appearance?
1) Aortic rupture
2) Aortic dissection
3) Cardiac tamponade
4) Oesophageal perforation
5) Pericarditis
Explanation
Oesophageal perforation
Pneumomediastinum is the presence of air in the mediastinal tissues and can be readily seen on a chest radiograph. It is a hallmark of oesophageal perforation and large airway (trachea or bronchus) injury, and therefore must be taken seriously.
Aortic rupture
Isolated aortic rupture may cause mediastinal widening on the chest radiograph, but will not present as pneumomediastinum. Additionally, a patient with aortic rupture will likely present in extremis due to sudden circulatory collapse.
Aortic dissection
An aortic dissection occurs when there is an intimal tear of the aortic endothelium creating a false lumen. This can cause mediastinal widening on the chest radiograph, however there will be no air within the mediastinum.
Cardiac tamponade
Cardiac tamponade causes compression of the heart due to the accumulation of fluid, usually blood, impairing diastolic filling and so cardiac output. This may cause an enlarged cardiac shadow on the chest radiograph but no air would be visible within the mediastinum.
Pericarditis
Pericarditis is inflammation of the pericardium and has a number of aetiologies but it will not cause pneumomediastinum.
Acute limb compartment syndrome is characterised by increased pressure within an unyielding osteo-fascial compartment, resulting in local tissue hypoxia. Urgent surgery to decompress with responsible compartment(s) is necessary to reduce longer term morbidity and mortality.
Of the following symptoms, which one is a late sign of compartment syndrome and indicates the poorest prognosis?
1) Anaesthesia
2) Paraesthesia within distribution of sensory nerves
3) Pulses present
4) Severe pain on passive muscle stretch
5) Swollen limb
Anaesthesia
Complete anaesthesia is a late sign due to myoneural necrosis and indicates a poor prognosis.
Compartment syndrome is the term used to describe the condition in which the tissue pressure in an enclosed fascial compartment rises above the capillary pressure, so reducing blood flow to the distal tissues. Although direct measurement of compartmental pressures can be made, the condition should be treated on clinical grounds with removal of any occlusive dressings and elevation followed by fasciotomy if needed.
Paraesthesia within distribution of sensory nerves
Paraesthesia is a relatively late sign, however, progression to complete anaesthesia indicates a worse prognosis.
Pulses present
Distal pulses and capillary refill may be present even in the presence of significant increases in compartmental pressure.
Severe pain on passive muscle stretch
The earliest sign is pain out of proportion with the injury, particularly severe pain on passive muscle stretch. Other early signs are pink shiny skin and a feeling of pressure.
Swollen limb
This is an early sign of compartment syndrome and if diagnosis and treatment at this stage are swift then the prognosis would be good.
A 54-year-old man presents as an emergency to casualty following a crush injury to his left femur sustained on a building site. On examination a diagnosis of compartment syndrome is suspected.
Which one of the following early signs might be expected on examination of this man’s left lower limb to support this presumptive diagnosis?
1) Absent dorsalispedis pulse
2) Motor loss before sensory loss
3) Severe pain on passive stretch of the affected group of muscles
4) Greatly prolonged capillary refill
5) Blue or grey extremities
Explanation
Severe pain on passive stretch of the affected group of muscles
Severe pain in response to passive stretch of the ischaemic muscles is by far the most dramatic and reliable clinical sign.
Compartment syndrome is defined as an increase in the interstitial fluid pressure within an osteofascial compartment of sufficient magnitude to cause microcirculatory compromise and later myoneural necrosis. The limb becomes tense and swollen, and if not treated, the muscle weakness progresses to paralysis. Alternatively, areas of muscle may infarct, giving rise to rhabdomyolysis, hyperkalaemia, hyperphosphataemia, high uric acid levels and metabolic acidosis. It is a devastating early complication seen after long-bone fractures and crush injuries. It can also be caused by deep thermal burns, electrical injuries, restricting tourniquets and fluid extravasation (eg caused by iv regional anaesthesia).
Classically, compartment pressures are measured using a slit-catheter device. The normal resting pressure within the compartment tissues is approximately 3–4 mmHg. Compartment pressures in excess of 30–35 mmHg in a normally perfused patient have previously been taken to indicate the need for open-compartment fasciotomy. Recent evidence, however, suggests that fasciotomy should be undertaken if the difference between the diastolic pressure and the measured compartment pressure is < 30 mmHg.
Absent dorsalispedis pulse
Early in its development, the peripheral pulses are normal, as are fingertip/toe colour, temperature and capillary refill, as it is the microvasculature that is initially affected. Loss of peripheral pulses is usually a late sign and the diagnosis should be made before this progression of signs.
Motor loss before sensory loss
Thin cutaneous nerve fibres are more susceptible to ischaemia than the motor fibres, and distal paraesthesias occurs before motor loss.
Greatly prolonged capillary refill
Capillary refill may be normal in the early stages, so waiting until capillary refill time is prolonged is not appropriate as this will lead to a poor outcome for the patient. As such, prolonged capillary refill should not be used to support the presumptive diagnosis as ideally treatment would have taken place before this sign developing.
Blue or grey extremities
This would indicate significant disruption to the vasculature of the limb and ideally the diagnosis and subsequent treatment should have occurred before colour changes in the affected limb.
You are completing your paediatric surgical rotation and discussing with your consultant the indications for urgent surgery in an infant. Certain presentations must be dealt with swiftly to reduce future morbidity and mortality.
For which one of the following would a 4-month-old infant need urgent treatment?
1) A 6 cm strawberry naevus over the sacrum
2) Bat ears
3) Bilateral hydroceles
4) Metatarsus varus
5) Redcurrant coloured stool
Explanation
Redcurrant coloured stool
Redcurrant coloured stool is a late presentation suggesting a severe intussusception, and urgent treatment is required with a surgical opinion as soon as possible. Intussusception occurs when one section of the intestines invaginates another, the most common form is ileocolic. Surgical treatment is aimed at reducing the intussusception manually, usually an air enema or, if severe, resection of the affected bowel.
A 6 cm strawberry naevus over the sacrum
Strawberry naevus only requires treatment if the lesion is impairing sight, as failure to develop stereoscopic vision is a possibility. As this naevus is located on the sacrum, no treatment is required.
Bat ears
This is primarily a cosmetic concern for parents and is not an indication for urgent surgery.
Bilateral hydroceles
Bilateral hydroceles do not require urgent treatment as usually they resolve spontaneously.
Metatarsus varus
Metatarsus varus may correct with physiotherapy in due course and is not urgent.
You admit an elderly man through the Emergency Department with a 10-day history of abdominal distension, crampy abdominal pain and absolute constipation for the past 24 h. He has also been vomiting large amounts of faeculant material.
Which one of the following is the most common cause of colonic obstruction?
1) Endometriosis
2) Gallstone ileus
3) Irritable bowel syndrome
4) Sigmoid volvulus
5) Solitary rectal ulcer
Explanation
Sigmoid volvulus
The most common causes of colonic obstruction include carcinoma of the colon (approximately 65% incidence), diverticulitis (10% incidence), volvulus (approximately 5% incidence), others including pseudo-obstruction, radiational and inflammatory stricture, external hernias and ischaemic hernias approximately 20% incidence. A sigmoid volvulus may be resolved simply by passing a flatus tube rectally, if unsuccessful the patient will require surgery and manual reduction or resection of the volvulus.
Endometriosis
Endometriosis is a condition in which functioning endometrial tissue is found outside of the uterus, most commonly in the abdominal or pelvic cavity. If endometrial tissue is found on the bowel wall it can cause a stenosis and subsequent obstruction, however it is uncommon.
Gallstone ileus
Gallstone ileus is a rare form of bowel obstruction and occurs when a gallstone becomes lodged in the terminal ileum. It is a rare, but recognised complication of endoscopic retrograde cholangio-pancreatography (ERCP).
Irritable bowel syndrome
Irritable bowel syndrome does not cause bowel obstruction but may cause functional constipation.
Solitary rectal ulcer
Solitary rectal ulcers can cause obstruction if severe due to pain, however it is uncommon.
A 45-year-old man presents to the Acute Surgical Admissions Unit with a 1-day history of worsening epigastric pain, radiating through to the back, and vomiting. He is not able to find any comfortable position and analgesia is not helpful. Clinical examination reveals generalised abdominal guarding and rigidity.
What is the most definitive investigation to confirm diagnosis?
1) Amylase of 450 international units is suggestive of acute pancreatitis
2) Computed tomography (CT) scan of the abdomen
3) Ultrasound scan
4) Normal serum amylase excludes acute pancreatitis
5) The modified Glasgow score
Explanation
Computed tomography (CT) scan of the abdomen
In this context, a CT scan of the abdomen is the best investigative modality in confirming acute pancreatitis. It is also useful when the diagnosis is not clear and other conditions such as perforation and peritonitis are being considered.
Amylase of 450 international units is suggestive of acute pancreatitis
Amylase is often used to confirm the diagnosis of acute pancreatitis in conjunction with clinical symptoms and signs. However, the use of arbitrary values (eg >1000 or 3–4 times the upper limit of normal) are dependent upon the half-life of amylase. When available, plasma lipase has more accuracy than amylase. Lipase is produced by the pancreas alone and persists for longer than amylase. It is therefore more sensitive and specific in the diagnosis of acute pancreatitis.
Ultrasound scan
The pancreas is poorly visualised by ultrasonography but is useful to assess the aetiology (eg gallstones) or to detect other pathologies (eg abdominal aortic aneurysm). Normal serum amylase excludes acute pancreatitis
Amylase level gradually returns to normal over 3–4 days and delay in performing the test can lead to a false-negative result. A raised amylase can also occur in upper gastrointestinal (GI) perforation, mesenteric infarction, small bowel obstruction, tubo- ovarian disease, renal failure, or macroamylasaemia.
The modified Glasgow score
This is clinical severity and prognosis score, is not an investigation for acute pancreatitis.
A 55-year-old man with severe epigastric pain radiating through to the back presents with the following results:
Amylase (urine test): 500 IU, Total bilirubin: 50 mmol/l, Total bilirubin: 50 mmol/l, Alkaline phosphatase: 250 mmol/l, Aspartate transaminase: 50 mmol/l, Alanine transaminase: 95 mmol/l
Severity can be predicted with which one of the following?
1) APACHE I score
2) C-reactive protein
3) Glasgow Score
4) White-cell count
5) Serum amylase
Explanation
Glasgow Score
The Glasgow score can be used to predict severity and takes into consideration a number of parameters including; age, white-cell count, p (O ), serum glucose, calcium, albumin, LDH and urea. The British Society of Gastroenterology recommends that the Glasgow score be used to predict severity within 48 h following admission. It is only applicable when the presumed cause is either alcohol or gallstones. Computed tomography (CT) scan can also be used to assess prognosis by grading the extent of pancreatic necrosis (Balthazar score). Other CT features such as necrosis of the head of pancreas, mesenteric oedema and intraperitoneal fluid are also associated with a poorer outcome.
APACHE I score
The APACHE II score, not APACHE I, is a general severity of disease tool and mortality predictor used for the acutely unwell patient admitted to intensive care. It is not specific to pancreatitis. An APACHE II score > 8 could be used to be predictive of severe acute pancreatitis. It takes into consideration the physiological state of the patient, including oxygenation, haemodynamic stability, biochemical abnormalities, and neurological status.
C-reactive protein
C-reactive protein (CRP), an acute phase reactant, can be used in isolation as a predictor of severity. A CRP > 150 mg/l more than 48 h after the onset of symptoms is predictive of severe acute pancreatitis, however, there are more reliable indicators and scoring systems as CRP can be raised with any inflammatory process.
White-cell count
White-cell count is one of the parameters included in the Glasgow score, which can be used to predict severity, however in isolation it is not useful.
Serum amylase
Levels of serum amylase and lipase are not predictive of severity.
A 6-year-old boy is brought to the Accident and Emergency department with a 12-h history of vomiting, severe abdominal pain and being generally unwell. His parents say that he also had two episodes of convulsions during this period. On examination, he appears pale and dehydrated. The abdomen is rigid and tender, and his pain is worse over the right iliac fossa. His temperature is 40.2°C, blood pressure 82/64 mmHg, and pulse rate 172 beats/min. There is no discoloration over the anterior abdominal wall. Bowel sounds are absent.
From the options below choose the one that you think is the most likely pathological process in this child.
1) Necrotising enterocolitis
2) Uncomplicated acute appendicitis
3) Volvulus neonatorum
4) Meckel’s diverticulitis
5) Bacterial Peritonitis
Explanation
Bacterial Peritonitis
The signs and symptoms in this child are suggestive of spreading/established infection in the peritoneal cavity. Bacterial peritonitis in children may occur as a result of a ruptured viscus such as ruptured appendicitis or ruptured Meckel’s diverticulitis, or as a complication of abdominal surgery. The child may present with classical signs of peritonitis such as abdominal pain, pyrexia, nausea, vomiting, tachycardia, low blood pressure and decreased urine output. High pyrexia may result in febrile convulsions. Abdominal examination may reveal a board-like rigidity, guarding and rebound tenderness. Bowel sounds are absent if the peritonitis becomes established. Plain abdominal X-rays should be performed in both supine and upright positions to identify the presence of free gas beneath the diaphragm, which suggests perforation of a viscus. The common organisms responsible for bacterial peritonitis in children
include Escherichia coli, Klebsiella pneumoniae and Pseudomonas species.
Necrotising enterocolitis
This condition is primarily seen in premature neonates in which the bowel necroses and is a common cause of morbidity. As the child described in the case history is 6 years old, this answer is unlikely.
Uncomplicated acute appendicitis
Acute appendicitis would result in many of the signs and symptoms described in the case history, however, given the child is in extremis, it would suggest bacterial peritonitis. A ruptured appendix as a sequelae of appendicitis would cause bacterial peritonitis but this answer does not specify that the appendicitis has progressed to rupture.
Volvulus neonatorum This is a condition seen in newborns and would present in the neonatal period, therefore this child is too old to present with volvulus neonatorum. Additionally, the high grade fever, febrile convulsions and cardiac instability are suggestive of an extensive infective process which would not be seen with volvulus neonatorum.
Meckel’s diverticulitis
Merkel’s diverticulum is a congenital diverticulum most commonly found in the distal ileum, if bowel contents become trapped then diverticulitis can occur producing infective symptoms. As above however, only if there was perforation of a viscus would the severity of symptoms described in the case history be seen.
A 54-year-old man presents to the Accident and Emergency department with a 6 h history of gradual onset severe epigastric and central abdominal pain radiating through to his back. The pain reduces when he sits forward. He has also had three episodes of vomiting, mostly bilious.
He admits to drinking up to 40–50 units of alcohol per week. He has experienced similar episodes in the past but less severe. On examination, his pulse rate is 94 beats/min and respiratory rate is 18 breaths/min. Abdominal examination reveals that he is very tender over the epigastric region with moderate degree of guarding. Plain radiographs of the chest (erect) and abdomen (supine) are unremarkable.
From the options below choose the one that you think is the most likely diagnosis in this patient.
1) Intestinal obstruction
2) Mesenteric ischaemia
3) Acute pancreatitis
4) Perforated peptic ulcer
5) Ruptured abdominal aortic aneurysm
Explanation
Acute pancreatitis
The signs and symptoms in this patient are very suggestive of acute pancreatitis, with the most common causes being alcohol and gallstones. Pancreatitis is thought to result from early activation of pancreatic enzymes, producing auto-digestion of the pancreas and surrounding tissues. The severity of acute pancreatitis is validated using various prognostic scoring systems. Currently in the UK, the Glasgow–Imrie (modified Glasgow score) scoring system is widely used for assessing the severity, while the APACHE II is useful predicating the prognosis in acute pancreatitis. Serum C-reactive protein concentration, although not part of the Glasgow criteria, has an independent prognostic value if the peak level is >210 mg/litre in the first 4 days of the attack. Serum amylase is a useful indicator to diagnose acute pancreatitis; a diagnosis of acute pancreatitis is likely if the level is three times the upper limit of normal although this may vary between laboratories depending on the hospital policy/guidelines. An ultrasound of the abdomen is indicated in all patients with acute pancreatitis to determine the presence/absence of biliary calculi. A computed tomography (CT) scan of the abdomen should be performed on all patients with severe acute pancreatitis between the third and tenth days following the onset of symptoms to rule out pancreatic necrosis, in addition to use of the Balthazar score, which examines the radiographic features of acute pancreatitis.
Intestinal obstruction
Intestinal obstruction would more commonly present with generalised abdominal pain and distension, vomiting and absolute constipation. If intestinal obstruction was the causative pathology in this case history abnormalities would likely be seen on the abdominal X-ray, such as dilated loops of bowel.
Mesenteric ischaemia Intermittent, colicky, generalised abdominal pain, worse in the post prandial period, is the most common presentation of mesenteric ischaemia. A history of atrial fibrillation is a common co-morbidity. Furthermore, mesenteric ischaemia usually has a chronic, progressive history with the avoidance of food (due to pain) and weight loss.
Perforated peptic ulcer
This presentation could also be consistent with a perforated abdominal viscus. However, it is slightly less likely than pancreatitis in this scenario given that chest radiograph excluded free air. This patient has an unremarkable chest radiograph. It should be noted that air is not always seen under the diaphragm in hollow viscus rupture, but given that this patient has also had previous less severe episodes and drinks alcohol heavily, pancreatitis is more likely. It is also more likely given the gradual onset – patients with a perforated viscus often describe sudden onset abdominal pain. Guarding itself may indicate peritoneal irritation.
Ruptured abdominal aortic aneurysm
A ruptured abdominal aortic aneurysm is likely to result in a haemodynamically unstable patient due to circulatory collapse. Given that neither the patient’s heart rate or respiratory rate are raised, this diagnosis is less likely. Furthermore, the history is often of sudden onset pain, not of a gradual course over 6 h.
A 47-year-old barmaid presents to the Accident and Emergency department with a 12 h history of right upper quadrant pain and vomiting. She says that the pain is radiating to her right scapula and is exacerbated by breathing. She appears pale but not jaundiced. On examination, her pulse rate is 98 beats/min, blood pressure is 126/84 mmHg and temperature is 37.6°C. Abdominal examination reveals tenderness over the right hypochondrium but no mass is palpable. Plain radiographs of the abdomen (supine) and chest (erect) are unremarkable.
From the options below choose the one that you think is the most likely diagnosis in this patient.
1) Perforated peptic ulcer
2) Acute pancreatitis
3) Acute biliary cholangitis
4) Acute cholecystitis
5) Infective hepatitis
Explanation
Acute cholecystitis
The history, signs and symptoms in this patient are suggestive of acute cholecystitis. Acute cholecystitis is more common in women over the age of 40 and with high body mass index (BMI). Gallstones are the commonest cause for acute cholecystitis. Obstruction of the common bile duct due to stones leads to accumulation of bile and inflammation, resulting in an acutely inflamed gall bladder. Other risk factors for acute cholecystitis include alcohol abuse and tumours of the gall bladder. The signs and symptoms of acute cholecystitis include: severe right hypochondrial pain exacerbated by respiration, nausea and vomiting, and increase in temperature. The rise in temperature is frequently mild to moderate; a very high temperature with or without chills and rigours may point to a diagnosis of acute cholangitis. A tender, inflamed gall bladder may be palpable in some patients. Likewise, jaundice may or may not be present.
Perforated peptic ulcer
A perforated abdominal viscus would be likely to be identified on an erect chest radiograph with the presence of free air under the diaphragm, and cause more centralised pain. This patient has an unremarkable chest radiograph.
Acute pancreatitis
Pancreatitis more commonly presents with epigastric pain radiating through to the back.
Acute biliary cholangitis
Ascending infection of the biliary tree and ducts requires urgent treatment, but it generally presents with high grade fever, rigours and jaundice. Charcot’s triad of jaundice, fever (often with rigours) and right upper quadrant pain is often used to diagnose cholangitis.
Infective hepatitis Hepatitis broadly refers to the inflammation of the liver, and infective causes most commonly include the hepatitis viruses (A, B, C, D, E). Infective hepatitis is not often an acute presentation as described in the case history.
A 51-year-old woman presents to the Surgical Emergency Assessment Unit with a 12 h history of central colicky abdominal pain and vomiting. She has undergone a subtotal colectomy and formation of an end ileostomy for ulcerative colitis 7 years ago. Her ileostomy has not functioned for 2 days. On examination, she is tender over the upper abdomen and the abdomen is mildly distended. Plain abdominal radiograph reveals a number of small loops in the centre of the abdomen.
From the options below choose the one that you think is the most likely diagnosis in this patient.
1) Acute colonic pseudo-obstruction
2) Incarcerated incisional hernia
3) Bacterial peritonitis
4) Adhesional small bowel obstruction
5) Sigmoid volvulus
Explanation
Adhesional small bowel obstruction
The cardinal features of small bowel obstruction are pain, vomiting and abdominal distension; untreated, this leads to constipation with reduction in flatus which then becomes absolute. The pain is usually colicky due to excessive peristalsis, but may become continuous if strangulation or perforation occurs. Vomiting is early in high small bowel obstruction, late in low small bowel obstruction and delayed or absent in large bowel obstruction. The management involves appropriate resuscitation of the patient and surgical exploration of the abdomen to relieve the obstruction. Small bowel obstructions make up the majority of intestinal obstructions. Of these, adhesions following laparotomy and/or surgery to the bowel is the leading cause of small bowel obstruction. It can occur as a sequelae of ‘minor’ abdominal surgeries such as appendicectomies or ‘major’ surgeries such as resection of large sections of the bowel. In women, gynaecological procedures are an important cause. In addition, pelvic inflammatory disease can also lead to adhesions even in the absence of a surgical intervention in the abdomen.
Acute colonic pseudo-obstruction
This is obstruction and massive dilation of the colon in the absence of a mechanical blockage. As this patient has undergone a subtotal colectomy, this is not a possibility.
Incarcerated incisional hernia
Obstruction in a patient with known hernia can occur when a section of bowel becomes incarcerated within a hernial orifice. Abdominal examination would reveal localised tenderness over the incisional hernia, and there is not suggestion of a hernia in the case history.
Bacterial peritonitis
Bacterial peritonitis can occur as a result of a perforated abdominal viscus with infection of the peritoneal cavity. The patient may present with classical signs of peritonitis such as abdominal pain, pyrexia, nausea, vomiting, tachycardia, low blood pressure and decreased urine output. Abdominal examination may reveal a board-like rigidity, guarding and rebound tenderness.
Sigmoid volvulus This patient has previously undergone a subtotal colectomy with formation of an end ileostomy in which the sigmoid colon would have been resected.
A 75-year-old woman is admitted with small bowel obstruction and
pain radiating down the medial aspect of the right thigh to the knee. There is no palpable abnormality in the groin but the inner aspect of the groin is tender.
Which one of the following is the most likely diagnosis?
1) Femoral hernia
2) Gluteal hernia
3) Lumbar hernia
4) Obturator hernia
5) Sciatic hernia
Explanation
Obturator hernia
An obturator hernia is six times more common in women and twice as common on the right side. It particularly affects elderly women who have had recent rapid weight loss. The hernial sac protrudes through the obturator canal potentially compressing the geniculate branch of the obturator nerve causing referred pain.
Femoral hernia
Strangulated femoral hernias are generally palpable within the groin, lateral and inferior to the pubic tubercle.
Gluteal hernia
Gluteal hernias are very rare and are suggested by the presence of a painful swelling in the buttock or pain.
Lumbar hernia
Lumbar hernias are associated with paralysed muscles especially by poliomyelitis or spina bifida.
Sciatic hernia
Again, sciatic hernias are very rare but would be suggested by pain in the distribution of the sciatic nerve.
As a CT2 surgical trainee you are asked to perform a tracheostomy on a 52-year-old man currently on the Intensive Care Unit (ITU). The supervising consultant asks you where on the trachea would you normally perform the tracheostomy.
Selecting from the following, how would you answer?
1) Cricoid cartilage
2) Crycothyroid membrane
3) First tracheal cartilage
4) Second tracheal cartilage
5) Thyroid isthmus
In a typical surgical tracheostomy, the preferred site is usually between the second and third tracheal cartilages. Based on the given options, the most appropriate answer would be:
4) Second tracheal cartilage
Explanation:
• Cricoid cartilage (1): A tracheostomy is not performed at this level because it is part of the larynx and performing the procedure here can damage the larynx. • Cricothyroid membrane (2): This is the site for an emergency cricothyrotomy, not a tracheostomy. A cricothyrotomy is usually performed in acute situations where airway access is needed immediately. • First tracheal cartilage (3): This is too high and close to the cricoid cartilage, posing a risk of laryngeal damage. • Second tracheal cartilage (4): This is the most common site for a surgical tracheostomy, as it allows for secure placement of the tracheostomy tube while minimizing risks to the laryngeal structures. • Thyroid isthmus (5): The thyroid isthmus often lies over the second and third tracheal rings, and while the tracheostomy may involve moving or dividing the isthmus, the actual tracheal opening is made in the second or third tracheal cartilage.
Conclusion:
The most appropriate site for performing a tracheostomy is at the level of the second tracheal cartilage.
A 56-year-old woman with known metastatic breast cancer presents to the Emergency Department with a calcium concentration of 3.22 mmol/l (normal corrected Ca2+ 2.20– 2.60 mmol/l).
Which one of the following is the most appropriate initial management?
1) Intravenous hydrocortisone
2) Intravenous infusion of 0.9% sodium chloride
3) Intravenous infusion of sodium phosphate
4) Oral bisphosphonate
5) Oral thiazide diuretic
Explanation
Intravenous infusion of 0.9% sodium chloride
The priority in symptomatic hypercalcaemia is to rehydrate the patient and establish diuresis; 0.9% sodium chloride is the fluid of choice, and patients often require around 4–6 litres over a 24 h period.
Intravenous hydrocortisone
Corticosteroids may occasionally be helpful but are not first-line treatment. Intravenous infusion of sodium phosphate
Sodium phosphate infusion is dangerous: it lowers the calcium concentration rapidly, but risks causing metastatic calcification.
Oral bisphosphonate
Bisphosphonates are effective, but must be given intravenously to have a rapid effect. Oral thiazide diuretic
Diuretics are only helpful for management of fluid overload in patients receiving rehydration therapy, and so copious volumes of intravenous fluids. However, they are not useful for reducing serum calcium levels. Loop diuretics would be the diuretic of choice, not thiazide diuretics.
A 71-year-old man is taken to theatre as an emergency following perforation of his colon resulting in generalised peritonitis. A subtotal colectomy and end ileostomy is performed.
What is the most common cause of peritonitis?
1) Acute appendicitis
2) Acute cholecystitis
3) Perforated peptic ulcer
4) Post-operative complications
5) Secondary to an initial infection Explanation
Explanation
Post-operative complications
The most common cause of peritonitis is post-operative complications, accounting for approximately 30% of cases. Peritonitis is inflammation of the peritoneum that may be localised (peritonism) or generalised. It is classified according to the causative agent, examples including bacterial, chemical or biliary.
Acute appendicitis
Acute appendicitis accounts for around 20% of cases.
Acute cholecystitis
Acute cholecystitis rarely leads to biliary peritonitis because the inflamed gall-bladder rarely becomes gangrenous or perforates.
Perforated peptic ulcer
Perforated peptic ulcers account for around 20% of cases of peritonitis. Secondary to an initial infection
If an infection is severe causing a bacteraemia then peritonitis can occur as a consequence of the initial infection, however this is uncommon.
An 81-year-old woman is admitted with bowel obstruction, pain and dehydration. An abdominal radiograph is performed by the admitting doctor with features suggesting volvulus of the large bowel.
What is the commonest form of volvulus in the elderly?
1) Caecal
2) Sigmoid
3) Small bowel
4) Stomach
5) Transverse colon
Explanation
Sigmoid
Volvulus occurs when a segment of bowel twists on its mesentery. Sigmoid volvulus is the commonest form of volvulus in the elderly and occurs in an anticlockwise direction. It is seen in approximately 5% of cases of large bowel obstruction in developed countries. The characteristic radiological appearance is known as a ‘coffee bean’ which arises from the left iliac fossa. The volvulus may be resolved by passing a flatus tube and, if unsuccessful, more aggressive surgical management.
Caecal
Caecal volvulus is less common than volvulus of the sigmoid colon.
Small bowel
A small bowel volvulus is rare; more commonly obstruction of the small bowel is due to adhesions from previous surgery.
Stomach
Gastric volvulus is uncommon and can occur at any age but a higher incidence is seen in the elderly.
Transverse colon
Again, this is an uncommon form of volvulus.
A 55-year-old obese man with known diabetes is admitted to an acute medical ward with cellulitis of the lower limb. Over the next few hours he becomes unwell and complains of increased pain. His cellulitis spreads, he becomes
haemodynamically unstable and he requires inotropic support to maintain his blood pressure (BP). You do an arterial blood gas.
Which one of the following would you most expect to find?
1) Metabolic acidosis
2) Metabolic alkalosis
3) Mixed metabolic alkalosis with respiratory compromise
4) Respiratory acidosis
5) Respiratory alkalosis
Explanation
Metabolic acidosis
Metabolic acidosis can be due to impaired hydrogen excretion, increased hydrogen ion production or ingestion, or loss of bicarbonate. In this patient, the rapidly spreading cellulitis coupled with significant cardiovascular compromise could indicate necrotising fasciitis and should be suspected. The acidosis is likely due to a diabetic ketoacidosis; diabetes is also a risk factor for necrotising fasciitis. Other causes of metabolic acidosis include renal disease, lactic acidosis, salicylate poisoning and chronic diarrhoea and intestinal fistulas.
Metabolic alkalosis
Given the severe infection and diabetes a metabolic acidosis, not alkalosis, is likely. Mixed metabolic alkalosis with respiratory compromise
Metabolic alkalosis with respiratory compensation would be unlikely. Respiratory compensation would be through hypoventilation, and given this patient is haemodynamically unstable and septic it is likely he would be tachypneic. Additionally, sepsis causes a metabolic acidosis due to anaerobic respiration.
Respiratory acidosis
A respiratory acidosis would occur in the presence of hypoventilation. This patient is likely to have a high respiratory rate due to sepsis.
Respiratory alkalosis
This occurs during hyperventilation as more carbon dioxide is ‘blown off’ causing a reduction in hydrogen ions. Carbonic anhydrase in erythrocytes facilitates a reaction between carbon dioxide and water that results in hydrogen ions. As such, a reduction in carbon dioxide, through hyperventilation, will reduce the number of hydrogen ions and so acidity. The primary issues for this patient however is a metabolic acidosis due to sepsis.
A patient awaiting surgery for a parathyroid adenoma is admitted with confusion and drowsiness. The diagnosis of hypercalcaemic crisis is made.
What should be the initial treatment?
1) Intravenous magnesium
2) Intravenous fluids
3) Steroids
4) Calcitonin
5) Intravenous phosphate
Explanation
Intravenous fluids
Hypercalcaemic crisis occurs when a patient with mild hypercalcaemia has a rapid rise in plasma calcium. This is associated with rapid deterioration in the patient’s condition, in the form of confusion, drowsiness, vomiting and dehydration. The first-line treatment of hypercalcaemia is intravenous fluid replacement with 0.9% saline. Crises may be precipitated by intercurrent illness, operation or infection.
Intravenous magnesium
This has no role in the treatment of a hypercalcaemic crisis.
Steroids
Hypercalcaemic crises due to hyperparathyroidism do not respond to treatment with steroids. Even in hypercalcaemia of different aetiologies, corticosteroids are considered second-line treatment.
Calcitonin
The condition may be controlled pre-operatively by the careful use of calcitonin or mithramycin but these would not be initial treatment.
Intravenous phosphate
Sodium phosphate infusion is dangerous; it lowers the calcium concentration rapidly, but risks causing metastatic calcification.
A patient is admitted with diverticulitis. On assessment they are found to have a high fever, are tachycardic and have localised peritonism in the left iliac fossa.
Which one of the following statements fulfils the criteria for the correct definition of sepsis?
1) The presence of bacteria in the bloodstream
2) The presence of micro-organisms within a normally sterile viscus
3) Hypotension refractory to resuscitation in the presence of demonstrable infection
4) Sepsis is a life-threatening organ dysfunction due to a dysregulated host response to infection.
5) Low blood pressure, signs of fluid overload and the presence of a proven source of infection.
Explanation
Sepsis is a life-threatening organ dysfunction due to a dysregulated host response to infection.
Sepsis is a life-threatening condition associated with significant morbidity and mortality, yet with early recognition and management these can be improved greatly. As such, in recent years there has been a concerted effort to improve the recognition and management of sepsis which includes increasing public awareness and producing a clear definition of sepsis. Recent National Institute of Clinical Excellence (NICE) Guidance was released in 2016 in response to a UK Parliamentary inquiry examining sepsis. The ‘Time to Act’ report found failures in the recognition, diagnosis, and early management of those who died from sepsis. Part of this guidance was clarifying the definition of sepsis as previously there was a confusing mix of terminology being used to describe the disease process, including; septicaemia, sepsis, septic shock and systemic inflammatory response syndrome (SIRS). Efforts are now being made to improve the recognition, diagnosis and management of sepsis throughout the UK.
The presence of bacteria in the bloodstream
This simply describes a bacteraemia that may or may not be related to sepsis depending if there is organ dysfunction or a dysregulated host response to infection.
The presence of micro-organisms within a normally sterile viscus
Sepsis is a systemic dysregulated response to infection and is not limited to a viscus. Hypotension refractory to resuscitation in the presence of demonstrable infection
In the past this would have been referred to as septic shock. A universal definition of sepsis is now in use and terminology such as septic shock should be avoided and replaced simply with sepsis.
Low blood pressure, signs of fluid overload and the presence of a proven source of infection. This is not the universally agreed definition of sepsis.
A 69-year-old immunosuppressed man presents to the medical ward with cellulitis of the leg. Over the next 24 h he becomes systemically unwell. He is anuric and develops acute renal failure. His systolic blood pressure does not respond to fluids and he requires noradrenaline support. He has radiological signs of acute respiratory distress syndrome (ARDS) on his chest X-ray.
Which one of the following is true of multi-organ dysfunction syndrome?
1) Jaundice is usually due to gallstones
2) Mortality rates are low
3) Sepsis is a well recognised cause
4) There is a decreasing alveolar–arterial oxygen gradient
5) There is increased lung compliance
Explanation
Sepsis is a well recognised cause
There are several causes of multi-organ dysfunction syndrome, however infections (resulting in sepsis) and injuries (either trauma or surgery) are the most common causes. If sepsis is left untreated, or unrecognised, then this can progress to septic shock and multi-organ dysfunction syndrome.
Jaundice is usually due to gallstones
Intrahepatic cholestasis and hepatocyte necrosis is the usual cause of jaundice in these patients.
Mortality rates are low
Multi-organ dysfunction syndrome unsurprisingly is associated with a high mortality rate, approximately 30–100%.
There is a decreasing alveolar–arterial oxygen gradient
A decreasing alveolar–arterial (A–a) gradient implies there is a reduced diffusion barrier to Ocrossing from the alveolus to 2 blood and would enhance gas transfer. In ARDS the A–a gradient increases due to alveolar–capillary membrane thickening caused by endothelial leakage and accumulation of interstitial fluid.
There is increased lung compliance
Increased lung compliance means the lung is more distensible so that a smaller fall of intrapleural pressure would be required for a given tidal volume during inspiration and respiratory work would decrease (in emphysema this is a problem as expiratory recoil is reduced). In ARDS lung compliance is reduced. In multiple organ dysfunction syndrome there is an uncontrolled inflammatory response and mal-distribution of blood flow, as a result of microembolic phenomenon, excessive release of various vasoactive compounds (vasoconstrictors, vasodilators and those that increase capillary permeability) which leads to interstitial oedema and a mismatch between supply and demand for oxygen within the microcirculation. Other hypotheses include inappropriate and uncontrolled release of cytotoxic mediators from bacteria.
A 40-year-old woman with acute onset abdominal pain and fever is found to be tachycardic and hypotensive with localised guarding and tenderness in the epigastric region. Despite initiating therapy for her pancreatitis, she develops refractory hypoxaemia secondary to acute respiratory distress syndrome (ARDS).
Which one of the following is the most appropriate initial management considering her current clinical state?
1) Close monitoring within a level 1 care facility
2) Computed tomography (CT) scan
3) Dopamine therapy to support renal function
4) Early endoscopic retrograde cholangiopancreatography (ERCP)
5) Initiate nursing in a prone position
Explanation
Initiate nursing in a prone position
Nursing in a prone or seated position has been shown to improve refractory
hypoxaemia caused by ARDS.
Close monitoring within a level 1 care facility
Patients should be managed in a level 2 or level 3 facility, which refers to an acute district hospital (level 2) or level 3 which is a tertiary referral centre (usually a teaching hospital). A level 1 care facility refers to a community hospital.
Computed tomography (CT) scan
This patient is critically unwell and a CT scan is unlikely to change current management; she primary requires treatment for ARDS and pancreatitis. Furthermore, transfer to the radiology department carries inherent risk for limited benefit.
Dopamine therapy to support renal function
Dopamine is not recommended as it has been shown to cause arrhythmias, so should be avoided.
Early endoscopic retrograde cholangiopancreatography (ERCP)
This patient is critically unwell and in their current condition ERCP is extremely risky. Early ERCP is only recommended for patients with co-existing cholangitis or biliary obstruction.
A 19-year-old girl is admitted to the Emergency Department with a severe anaphylaxisreaction following a bee sting. She becomes severely hypotensive and her airway may be threatened.
Which one of the following management options would you be most appropriate?
1) Intramuscular adrenaline
2) Nebulised bronchocdilators
3) Beta blockers
4) Intravenous nitrates
5) Intravenous morphine
Explanation
Intramuscular adrenaline
The principles of management of severe anaphylactic shock include: airway maintenance and oxygen; intravenous fluids to provide circulatory support; and subcutaneous or intramuscular adrenaline (adrenaline). The dose for an adult is 0.5 mg adrenaline, repeated after 5 min as required. Subsequent treatment is with an anti- histamine (chlorphenamine 4 mg) and corticosteroid (hydrocortisone 100 mg), however, adrenaline should be given first as this is the life-saving treatment.
Nebulised bronchocdilators
Nebulised bronchodilators can help counteract the bronchospasm, however, intramuscular adrenaline is the primary treatment.
Beta blockers
Beta blockers are contraindicated as they lower blood pressure and may cause bronchoconstriction, so have no role in the treatment of anaphylaxis.
Intravenous nitrates
Intravenous nitrates cause vasodilation and have no role in the treatment of anaphylaxis.
Intravenous morphine
Intravenous is primarily reserved for severe pain management, therefore would not be indicated in the treatment of anaphylaxis.
A 25-year-old woman exhibiting features of septic shock is brought to your
attention. She has been admitted with acute appendicitis, unfortunately there has been a delay with CEPOD and she has deteriorated.
Which one of the following is correct?
1) Tissue uptake of oxygen by cells is normal
2) Peripheral vasoconstriction is seen
3) Blood cultures are commonly positive
4) Endotoxins act as a negative inotrope
5) Gram-negative organisms are the most common cause
Explanation
Endotoxins act as a negative inotrope
Endotoxins act as a negative inotrope and can initiate both ARDS and disseminated intravascular coagulation (DIC).
Tissue uptake of oxygen by cells is normal
Tissue uptake of oxygen by cells is reduced, contributing to the metabolic acidosis of sepsis due to anaerobic respiration and so increased lactic acid production.
Peripheral vasoconstriction is seen
Peripheral vasodilation is seen throughout the systemic circulation causing reduced tissue perfusion and organ dysfunction if not treated.
Blood cultures are commonly positive
Blood cultures are rarely positive, however, if the causative bacteria is grown from blood cultures then this significantly aids treatment as targeted antimicrobial therapy is possible.
Gram-negative organisms are the most common cause
Septicaemic shock is most commonly due to Gram-positive organisms. While Gram- negative bacteria were previously considered the most common cause of septic shock, recent epidemiological studies revealed approximately 200 000 cases of Gram-positive sepsis annually, compared with 150 000 cases of Gram-negative sepsis in recent years.
Martin GS, Mannino DM, Eaton S, Moss M. The epidemiology of sepsis in the United States from 1979 through 2000. N Engl J Med. 2003;348:1546–54.
A 68-year-old woman is admitted to the Intensive Care Unit (ITU) having sustained 40% burns in a house fire. Over the next 3 days she develops increasing respiratory needs , wheeze and reduced pa (O2 ) level . You suspect she has developed ARDS.
Which one of the following statements regarding ARDS is correct?
1) It has an increased pa (O2 ) (in mmHg) to FI O 2 ratio
2) It has < 20% mortality in established cases
3) It is caused due to collapse of the alveoli
4) It is characterised by decreased lung compliance
5) It usually develops in the first 24 h after injury
Explanation
It is characterised by decreased lung compliance. Pulmonary fibrosis in the later stages of the disease leads to a decrease in the functional residual capacity, further decrease in lung compliance, and an increase in the shunt effect. It has an increased pa (O2 ) (in mmHg) to FI O 2 ratio. ARDS is characterised by refractory hypoxemia (pa (O2 ) < 8 kPa at FI O 2 > 0.4), alveolar
inflammation and oedema, reduced total compliance (< 30 ml/cmH2 O) and a pa (O2 ) (in mmHg) to FI O 2 ratio of < 200 (normal is approximately 500). It has < 20% mortality in established cases. The mortality of this condition is as high as 50–70%.
It is caused due to collapse of the alveoli. Indirect or direct lung injury initiates abnormal behaviour and movement of neutrophils, platelets and macrophages. Neutrophils and platelets attach to capillary endothelium causing capillary leak. This leads to oedema of lung tissue and movement of neutrophils and erythrocytes into the lung parenchyma. Lung lymph flow is increased and there is thickening of the alveolar–capillary membrane. This results in impaired oxygen diffusion and reduced lung compliance as the alveolus is surrounded by fluid. In addition, some of the fluid in the pulmonary parenchyma may leak into the alveoli, giving the characteristic appearance of a hyaline membrane.
It usually develops in the first 24 h after injury. ARDS can present as soon as 2 h following injury. ARDS is an acute, diffuse inflammatory process resulting from direct or indirect pulmonary injury. It is most commonly seen in sepsis but can also occur after trauma, burns, inhalation injuries, shock and pancreatitis. In post-operative surgical patients, abdominal sepsis or central- line sepsis should be considered. Pulmonary signs are often minimal or non-specific, the patient simply being breathless, progressively tachypneic, hypoxic and then cyanotic.
Chest X-ray may be normal in the early stages but later shows bilateral diffuse pulmonary infiltration. Treatment, in addition to eliminating the precipitating cause, involves ventilating the patient in an Intensive Care Unit. Patients are usually nursed in
the prone position. The tidal volume should be kept low (approximately 6 ml/kg) and so should the pulmonary capillary wedge pressure (high pressures exacerbate pulmonary oedema).
You are treating a patient who underwent emergency laparotomy for faecal peritonitis 2 days ago. The patient is tachycardic and hypotensive and has developed acute respiratory distress syndrome (ARDS). What is the most likely cause of this?
1) Abdominal sepsis
2) Fat embolism
3) Pancreatitis
4) Pulmonary fibrosis
5) Trauma
Explanation
Abdominal sepsis
ARDS is an acute diffuse inflammatory process resulting from direct or indirect
pulmonary injury. It is most commonly seen in sepsis but can also occur after trauma,
burns, inhalation injuries, shock and pancreatitis. In post-operative surgical patients,
abdominal sepsis or central-line sepsis should be considered. Pulmonary signs are often
minimal or non-specific in the early stages, the patient simply being breathless, but, if
untreated, they could soon become hypoxic and cyanotic. Chest X-ray may be normal in
the early stages but later shows bilateral diffuse pulmonary infiltration. The mortality of
this condition is as high as 50–70%.
Fat embolism
Fat embolism is a recognised complication of long bone fractures which are associated
with major trauma. As described above, major trauma can cause ARDS, however, given
this patient has been treated for peritonitis, abdominal sepsis is the most likely cause.
Pancreatitis
Pancreatitis is a recognised cause of ARDS, however abdominal sepsis is more likely
given the case history.
Pulmonary fibrosis
Pulmonary fibrosis occurs as a result of ARDS. Fibrosis in the later stages of the disease
leads to a decrease in the functional residual capacity, further decrease in lung
compliance and an increase in the shunt effect.
Trauma
As mentioned, major trauma is a recognised cause of ARDS, however, abdominal sepsis
is the likely cause in this patient. ARDS is characterised by refractory hypoxaemia
(pa (O2 ) < 8 kPa at Fi O2 > 0.4), alveolar inflammation and oedema, reduced total
compliance (< 30 ml/cm water) and a pa (O2 ) (in mm Hg):Fi O 2 ratio of < 200 (normal is
approximately 500). Indirect or direct lung injury initiates abnormal behaviour and
movement of neutrophils, platelets and macrophages. Neutrophils and platelets attach
to capillary endothelium causing capillary leakage. This leads to oedema of lung tissue
and movement of neutrophils and erythrocytes into the lung parenchyma. Lung lymph
flow is increased with thickening of the alveolar–capillary membrane. This results in
impaired oxygen diffusion and reduced lung compliance as the alveolus is surrounded
by fluid. In addition, some of the fluid in the pulmonary parenchyma may leak into the alveoli, giving the characteristic appearance of a hyaline membrane. Treatment, in
addition to eliminating the precipitating aetiology, involves ventilating the patient in
intensive care. Patients are usually nursed in the prone position. The tidal volume
should be kept low (approximately 6 ml/kg), as should the pulmonary capillary wedge
pressure (high exacerbates pulmonary oedema).
You review a patient who underwent emergency laparotomy for abdominal sepsis 36 h
ago. The patient clearly has acute respiratory distress syndrome (ARDS).
Which one of the following would you expect in ARDS?
1) Focal infiltration on chest X-ray
2) Leakage of protein rich oedema fluid into the lung tissue
3) Only late occurrence of tachypnoea and tachycardia
4) Steroids promote recovery in the late phase
5) Thinning of the alveolar membrane
Explanation
Leakage of protein rich oedema fluid into the lung tissue
The underlying pathological process is leakage of cells and fluid through the capillary
walls of the alveoli. The alveolar membrane thickens and ultimately fibroses.
Focal infiltration on chest X-ray
Typically the chest X-ray shows diffuse infiltration.
Only late occurrence of tachypnoea and tachycardia
In the early stages, tachycardia and tachypnoea may be the only features. Later there is
cyanosis and worsening dyspnoea.
Steroids promote recovery in the late phase
Steroids have been used in the treatment of ARDS but there is no good evidence to
support their use. The cornerstones of treatment are support of the circulation,
respiratory support by ventilation and the use of appropriate antibiotics.
Thinning of the alveolar membrane
As mentioned thickening of the alveolar membrane occurs, and in the later stages of the
disease, fibrosis occurs.
Acute respiratory distress syndrome (ARDS) is a well recognised complication of sepsis,
trauma and other pulmonary insults, both direct and indirect. The commonest clinical
signs are tachypnoea, tachycardia, cyanosis and high-pitched coarse crepitations on
auscultation. The alveolar membrane becomes thickened. Differential diagnoses include
left ventricular failure, bronchitis, pneumonia and asthma.
Systemic Inflammatory Response Syndrome (SIRS) is an uncontrolled immune reaction, and can be classified by aetiology as either infectious or non-infectious SIRS has a
defined criteria for diagnosis. Recognition is essential as early intervention is more likely to prevent severe morbidity and mortality associated with pathologies such as sepsis.
Which one of the following would meet the requirements for SIRS?
1) Temperature of 37.5°C, a leucocyte count of 11 000/mm 3 , heart rate 150 beats/min
2) Temperature of 38°C, a leucocyte count of 5x10 9 /l, respiratory rate 15 breaths/min
3) Temperature of 35.5°C, a leucocyte count of 8x10 9 /l, pa (CO2 ) 50 mmHg
4) Temperature of 38.0°C, a leucocyte count of 2x10 9 /l, respiratory rate 30 breaths/min
5) Temperature of 37.5°C, a leucocyte count of 11x10 9 /l, heart rate 100 beats/min
Temperature of 38.0°C, a leucocyte count of 2x10 9 /l, respiratory rate 30 breaths/min
Pyrexia, leucopenia and tachypnoea are all consistent with SIRS. A patient is said to have SIRS if they satisfy two or more of the following criteria:
*temperature > 38°C or < 36°C
*heart rate > 90 beats/min
*respiratory rate > 20/min (or pa (CO2 ) < 32 mmHg)
*leucocyte count > 12 000/mm 3 or < 4000/mm 3 [or > 10% immature (band) cells].
*Temperature of 37.5°C, a leucocyte count of 11 000/mm 3 , heart rate 150 beats/min
This patient is normothermic, has a leucocyte count in the higher range of normal, and a significant tachycardia, as such they do not fit the criteria for SIRS.
Temperature of 38°C, a leucocyte count of 5x10 9 /l, respiratory rate 15 breaths/min
These parameters do not fill the criteria for SIRS.
Temperature of 35.5°C, a leucocyte count of 8x10 9 /l, pa (CO2 ) 50 mmHg
The low temperature fulfils the criteria, however, the leucocyte count is within the normal range (leucocyte count: 4–11 × 10 9 /l), and the pa (CO2 ) is satisfactory.
Temperature of 37.5°C, a leucocyte count of 11x10 9 /l, heart rate 100 beats/min Only the tachycardia would satisfy the SIRS criteria.
15-year-old post-splenectomy patient is admitted to hospital with sepsis. Since becoming a teenager she has been inconsistent in taking her prophylactic antibiotics.
Which one of the following is a sign indicative of septic shock ?
1) Confirmed or suspected infection
2) Core temperature < 36°C or > 38.3°C
3) Heart rate higher than 90 beats per min
4) Respiratory rate higher than 20 breaths per min
5) Urine output < 0.5ml/kg/h for more than 2 h
Explanation
Urine output < 0.5ml/kg/h for more than 2 h
Decreased urine output is an indicator of reduced renal perfusion, which can be used as a surrogate marker for reduced generalised tissue perfusion. This is therefore maybe a sign of septic shock as it represents renal dysfunction which occurs in severe circulatory abnormalities or circulatory collapse. Post-splenectomy sepsis can present 20 years after splenectomy and carries a high mortality. It is prevented by penicillin prophylaxis.
The micro-organisms most likely to lead to infection include pneumococcus and meningococcus species and H. influenzae. Other infections include those due to Escherichia coli and Plasmodium (malaria). The Surviving Sepsis Campaign has published international guidelines on the recognition and management of severe sepsis in an attempt to decrease the high mortality rates. Although NICE guidance lists ‘red- flags’ for sepsis (see below), the National Early Warning System (NEWS) introduced by the Royal College of Physicians is an alternative.
Red-flags for sepsis according to NICE Guidelines:
*systolic blood pressure <90 mmHg (or >40 mmHg fall from baseline)
*heart rate >130 beats per min
*oxygen saturations <91%
*respiratory rate >25 breaths per min
*responds only to voice or pain/unresponsive
*lactate >2.0 mmol.
Confirmed or suspected infection
A patient can have an ongoing infection but may not have sepsis which is defined as ‘life-threatening organ dysfunction due to a dysregulated host response to infection’.
Core temperature < 36°C or > 38.3°C
Although temperature is commonly used as an indicator of infection, it is not a helpful indicator of either severe sepsis or septic shock. Septic shock is defined as ‘a subset of sepsis in which underlying circulatory and cellular/metabolic abnormalities are profound enough to substantially increase mortality’ – The Third International Consensus Definitions for Sepsis and Septic Shock (Sepsis-3). It indicates a patient who is septic with refractory hypotension when given an intravenous fluid challenge/bolus.
While sepsis is referred to as life-threatening organ dysfunction due to a dysregulated host response to infection, so a patient may have an infection causing pyrexia but may not have sepsis.
Heart rate higher than 90 beats per min
Heart rate can be used as a ‘red-flag’ sign for sepsis according to NICE guidance, however, it suggests a tachycardia above 130 beats per min.
Respiratory rate higher than 20 breaths per min
Again, NICE guidance suggests a ‘red-flag’ sign for sepsis is a respiratory rate above 25 breaths per min.
A 76-year-old patient is admitted to the acute admission unit with septic shock. Pulse is 106 beats per minute and blood pressure 90/40 mmHg. Urinary catheterisation produces 75 ml of concentrated urine (normal 800–2000 ml in 24 h).
Which one of the following principles applies to the choice of an appropriate intravenous fluid for resuscitation?
1) Certain intravenous solutions, which would be hypo-osmolar, have dextrose added to ensure they are iso-osmolar
2) Hartmann’s solution contains sodium, potassium, chloride, calcium and bicarbonate
3) An advantage of crystalloid solutions is that relatively small volumes have to be infused to restore an intravascular volume deficit
4) The normal colloid oncotic pressure is 70 mmHg
5) Albumin is indicated
The most appropriate principle regarding the choice of intravenous fluid for resuscitation in a patient with septic shock is:
1) Certain intravenous solutions, which would be hypo-osmolar, have dextrose added to ensure they are iso-osmolar
Explanation:
In the context of intravenous fluid resuscitation for septic shock, the principles involve understanding the properties and functions of different types of fluids. Here’s a breakdown of the options:
1. Certain intravenous solutions, which would be hypo-osmolar, have dextrose added to ensure they are iso-osmolar: This is correct. Solutions like 5% dextrose are isotonic and provide free water once dextrose is metabolized, helping to ensure fluids remain iso-osmolar to avoid causing cellular dehydration or overhydration. 2. Hartmann’s solution contains sodium, potassium, chloride, calcium, and bicarbonate: This is incorrect. Hartmann’s solution (or Ringer’s lactate) contains sodium, potassium, chloride, calcium, and lactate (which is metabolized to bicarbonate), but not bicarbonate directly. 3. An advantage of crystalloid solutions is that relatively small volumes have to be infused to restore an intravascular volume deficit: This is incorrect. Crystalloids generally require larger volumes compared to colloids to achieve the same intravascular volume expansion due to their distribution across the extracellular space. 4. The normal colloid oncotic pressure is 70 mmHg: This is incorrect. Normal colloid oncotic pressure (oncotic pressure) is around 25-30 mmHg, not 70 mmHg. 5. Albumin is indicated: This is incorrect as a blanket statement. Albumin is a colloid and can be used in specific situations, but it is not the first-line treatment for fluid resuscitation in septic shock due to its cost and lack of evidence showing superiority over crystalloids.
Correct Answer: 1) Certain intravenous solutions, which would be hypo-osmolar, have dextrose added to ensure they are iso-osmolar
You are treating a 48-year-old man with acute severe pancreatitis. He has been in hospital for 48 h and you are assessing his modified Glasgow score. His modified Glasgow score is 5.
What is his mortality risk?
1) 2%
2) 15%
3) 30%
4) 40%
5) 100%
Explanation
40%
The mortality risk for a patient scoring 5 on the modified Glasgow score is 40%. The following criteria are used to calculate the score.
2%
This would represent the mortality risk for a patient with a modified Glasgow score of
0–2.
15%
This would represent the mortality risk for a patient with a modified Glasgow score of
3–4.
30%
The mortality risk for a patient scoring 5 on the modified Glasgow score is greater than
30%.
100%
Mortality risk reaches 100% when the patient scores between 7 and 8 on the modified
Glasgow score. Pancreatitis is a common presentation in the UK, of which gallstones and alcohol are the two primary aetiologies. Gallstones account for up to 50% of pancreatitis
in the UK, while alcohol accounts for 20–25%. The modified Glasgow score takes into account a number of parameter taken within the first 48 h following admission which can then be used to assess mortality risk These criteria are as follows; pa (O2 ) < 60 mmHg; age > 55 years; neutrophils > 15 × 10 9 /l; calcium < 2 mmol/l; raised urea > 16 mmol/l; enzyme (LDH) > 600 units/l; albumin, 32 g/l; sugar (glucose) > 10 mmol/l.
A 42-year-old man with type 1 diabetes mellitus, which was diagnosed 23 years ago, is admitted to hospital with diabetic ketoacidosis (DKA). He is complaining of epigastric abdominal pain and vomiting.
Which one of the following findings on admission would most specifically suggest the precipitating cause of this illness?
1) Serum amylase activity of 1244 U/l (upper limit of normal, 150 U/l)
2) Serum creatinine concentration 140 µmol/l (normal value creatinine: 50–120 µmol/l)
3) Serum lactate concentration 4.3 mmol/l (normal value lactate: 0.5–2.2 mmol/l)
4) Serum triglyceride concentration 12.2 mmol/l (normal value triglycerides: 0–1.5 mmol/l)
5) White cell count of 15 × 10 9 /l (normal value WCC: 4–11 × 10 9 /l)
Explanation
Serum amylase activity of 1244 U/l (upper limit of normal, 150 U/l)
Patients in DKA often have elevated serum amylase activities as a result of decreased renal excretion of the enzyme, however, a level this high is very suggestive of pancreatitis and coupled with the epigastric pain, pancreatitis is the likely precipitant cause of his DKA.
Serum creatinine concentration 140 µmol/l (normal value creatinine: 50–120 µmol/l)
Serum creatinine concentration is often slightly raised in patients with DKA (even in the absence of diabetic nephropathy) as a result of dehydration and subsequently a
decreased glomerular filtration rate.
Serum lactate concentration 4.3 mmol/l (normal value lactate: 0.5–2.2 mmol/l)
Decreased tissue perfusion as a result of dehydration often leads to an element of lactic acidosis secondary to anaerobic respiration, in addition to the ketoacidosis.
Serum triglyceride concentration 12.2 mmol/l (normal value triglycerides: 0–1.5 mmol/l)
Hypertriglyceridaemia is common in uncontrolled diabetes.
White cell count of 15 × 10 9 /l (normal value WCC: 4–11 × 10 9 /l)
An increased white cell count can occur in any acute illness and does not specifically indicate infection.
Two hours after sustaining major trauma in a road traffic accident, a 22-year-old man, not known to have diabetes, is found to have a high blood glucose concentration. They have sustained significant injuries
Increased secretion of which one of the following substances is most likely to be responsible?
1) Adrenaline (epinephrine)
2) Cortisol
3) C-reactive protein
4) Growth hormone
5) Insulin
Explanation
Adrenaline (epinephrine)
During the metabolic response to trauma, there is increased secretion of catecholamines (such as adrenaline), cortisol, glucagon and growth hormone. Adrenaline acts most rapidly through stimulation of glycogenolysis causing blood glucose concentration to rise
Cortisol
Increased secretion of cortisol following trauma will increase blood glucose concentration, however, this occurs more slowly through the stimulation of gluconeogenesis.
C-reactive protein
C-reactive protein is an acute phase reactant and a marker of inflammation, but does not affect glucose homeostasis.
Growth hormone
Growth hormone is also released in response to major trauma. It can increase blood glucose concentration indirectly as it appears to potentiate the action of cortisol and opposes the action of insulin.
Insulin
Insulin is a hypoglycaemic hormone, therefore would lower blood glucose concentration.
An 18-year-old man presents to Accident and Emergency after vomiting blood following a night out for his birthday. On further questioning he admits to consuming a large amount of alcohol and then vomiting heavily. After vomiting around 12 times he noticed streaks of blood in his vomit.
What is the most likely diagnosis?
1) Aortic-enteric fistula
2) Boerhaave syndrome
3) Mallory-Weiss tear
4) Oesophageal varices
5) Peptic ulcer disease
Explanation
Mallory-Weiss tear
A Mallory–Weiss tea is a tear in the mucosal lining of the oesophagus that classically occurs after heavily vomiting (often following an alcohol binge as described in the case
history). Most cases of bleeding from this are self-limiting and usually minor.
Aortic-enteric fistula
Aortic-enteric fistulae occur mostly following abdominal aortic aneurysm (AAA) repair in which communication has developed between the aorta and the small bowel. They
result in very heavy blood loss into the small bowel and are often fatal.
Boerhaave syndrome
Boerhaave syndrome is perforation of the oesophagus secondary to vomiting. It is associated with a high morbidity and mortality, and would present with severe chest pain, fever and shock. The patient described in the case is not significantly unwell.
Oesophageal varices
Oesophageal varices are the result of portal hypertension as blood is shunted through arteriovenous anastomosis due to increased vascular resistance within the liver, most commonly as a result of cirrhosis secondary to alcohol. It would be rare for an 18-year- old to have significant liver cirrhosis to cause varices. Furthermore, variceal bleeding
would be profuse, and unlikely to present with just streaks of blood in the vomit.
Peptic ulcer disease
A peptic ulcer can cause upper gastrointestinal bleeding, however, it would be unlikely in a young adult, and does not fit with the case history.
An 82-year-old man is recovering in the Intensive Care Unit
seven days after an emergency repair of a difficult juxta-renal leaking abdominal aortic aneurysm. The nurse bleeps you because he has become tachycardic and his blood pressure has dropped to 80/50 mmHg. On assessment he is very pale and the nurse reports he has just had a large, fresh per rectal (PR) bleed.
What is the most likely cause for this?
1) Angiodysplasia
2) Aortic-enteric fistula
3) Diverticular disease
4) Gastric Cancer
5) Peptic ulcer disease
Explanation
Aortic-enteric fistula
Aortic-enteric fistulae occur mostly following abdominal aortic aneurysm (AAA) repair in which a communication has developed between the aorta and the small bowel. They result in very heavy blood loss into the small bowel and are often fatal.
Angiodysplasia
Angiodysplasia is the most common vascular lesion of the gut. Lesions are often multiple and presentation insidious, and may simply present as anaemia.
Diverticular disease
Diverticular disease may present with small amount of fresh PR bleeding, but given the proximity to surgery, an aortic-enteric fistula is more likely.
Gastric Cancer
Gastric cancer is likely to have a more insidious presentation. Again, as the bleeding would be upper gastrointestinal in nature, it would present as melaena not fresh blood.
Peptic ulcer disease
Peptic ulcer can cause bleeding, however this is likely to present as upper gastrointestinal bleeding. Occasionally, they can present as lower gastrointestinal bleeding but this will present as melena as opposed to fresh PR blood.
A 35-year-old male with known ulcerative colitis (UC) reports generalised itching, fatigue and abdominal pain. On examination, he is found to be jaundiced and his blood tests show a markedly raised bilirubin and alkaline phosphatase. A magnetic resonance cholangiopancreatography (MRCP) test shows multiple strictures in the biliary tree.
What is the most likely diagnosis?
1) Cholangitis
2) Cholecystitis
3) Primary biliary cirrhosis
4) Primary sclerosing cholangitis
5) Wilson’s disease
Explanation
Primary sclerosing cholangitis
Primary sclerosing cholangitis (PSC) is a condition in which inflammation, fibrosis and Strictures of the intrahepatic and extra-hepatic bile ducts occur. MRCP shows multiple strictures in the biliary tree and a characteristic ‘beaded’ appearance. Around 80% of patients with PSC will have a diagnosis of UC.
Cholangitis
Cholangitis is an ascending infection of the biliary tree, given this patient has no signs of infection this is unlikely. Furthermore, strictures would not be seen in cholangitis.
Cholecystitis
This refers to inflammation of the gallbladder, most common caused by gallstones. If the gallstones become lodged in the common bile duct then obstructive jaundice may be seen, however, the finding of strictures on MRCP is more suggestive of primary sclerosing cholangitis.
Primary biliary cirrhosis
Primary biliary cholangitis is an autoimmune disorder causing destruction of the small interlobular bile ducts, subsequent intrahepatic cholestasis causes fibrosis and
ultimately cirrhosis of the liver. The patient has a history of UC, so primary sclerosing cholangitis is a more likely, furthermore, strictures in the biliary tree would not be seen
on MRCP.
Wilson’s disease
Wilson’s disease is a rare inherited disorder of copper metabolism leading to a pathological accumulation of copper within various organ, particularly affecting the liver and brain. Presentation is usually in teenager years with either neuropsychiatric conditions due to accumulation of copper in the brain, or with coagulopathy and hepatic encephalopathy from accumulation in the liver. As such, this does not fit with the case history given.
An 18-year-old A-level student is referred from his GP with
right iliac fossa (RIF) pain and nausea. On examination, she has guarding and tenderness in the RIF. On deep palpation in the left iliac fossa (LIF) she still complains of pain in the RIF.
What is this sign called?
1) Aaron’s sign
2) Dunphy’s sign
3) Murphy’s sign
4) Rigler’s sign
5) Rovsing’s sign
Explanation
Rovsing’s sign
Rovsing’s sign describes pain in the RIF more than the LIF when the LIF is pressed, and is a sign of appendicitis.
Aaron’s sign
Aaron’s sign is pain referred to the epigastrium upon continuous firm pressure over
McBurney’s point and is indicative of appendicitis.
Dunphy’s sign
Dunphy’s sign is a medical sign in which increased abdominal pain occurs with coughing and is often present in appendicitis.
Murphy’s sign
Murphy’s sign occurs when pressing over the right upper quadrant (RUQ) with two fingers and asking the patient to breathe in, causes pain and arrest of inspiration as the
inflamed gall-bladder impinges on the examiner’s fingers. It is only positive if repeating the test on the left does not result in a similar pain.
Rigler’s sign
Rigler’s sign is a radiological sign in which air is seen on an abdominal X-ray on both sides of the intestine.
A previously well 83-year-old woman is brought into Accident and Emergency with abdominal pain and vomiting. On examination, she has a distended abdomen, and denies any previous surgery. On assessment of her groin, a tenderlump
is palpable on the left. It is below and lateral to the pubic tubercle.
What is the most likely cause of her symptoms?
1) Epigastric hernia
2) Femoral hernia
3) Direct inguinal hernia
4) Indirect inguinal hernia
5) Obturator hernia
Explanation
Femoral hernia
Femoral hernias classically emerge below and lateral to the pubic tubercle, and are
more common in women due to the wider pelvis. As the neck for femoral hernias is
relatively narrow and stiff, they are more likely to obstruct and strangulate.
Epigastric hernia
Given the patient has a lump in their groin, an epigastric hernia would be unlikely.
Direct inguinal hernia
Inguinal hernias emerge from above and medial to the pubic tubercle. Direct inguinal
hernias pass through Hasselbach’s triangle directly through a defect in the anterior
abdominal wall.
Indirect inguinal hernia
Again, inguinal hernias emerge from above and medial to the pubic tubercle, however,
indirect inguinal hernias pass through the inguinal canal.
Obturator hernia
An obturator hernia is rare but is more common in women and twice as common on the
right side. It particularly affects elderly women who have had recent rapid weight loss.
The hernial sac protrudes through the obturator canal potentially compressing the
geniculate branch of the obturator nerve causing referred pain. Given their anatomical
location they are difficult to diagnose clinically and does not present as a lump in the
groin.
A 17-year-old girl is brought in with a history of abdominal and left-sided chest pain. She gives a history of excessive vomiting earlier after a large meal, followed by sudden
onset of the pain. On examination she looks very unwell, is febrile and has a chest X-ray that shows a left pleural effusion. She is peripherally shutdown, hypotensive and
tachycardic. Her only past medical history of note is eating disorders for which she is known to the adolescent psychiatry team.
What is the most likely diagnosis?
1) Achalasia
2) Hiatus hernia
3) Mallory–Weiss tear
4) Oesophageal cancer
5) Oesophageal rupture
Explanation
Oesophageal rupture
Oesophageal rupture (Boerhaave syndrome) occurs mostly either secondary to trauma eg during endoscopy or due to forceful vomiting. Excessive vomiting may be due to
alcohol or in patients with a history of excessive eating and forced vomiting eg due to bulimia as suggested in this case. Perforation of the oesophagus causes a mediastinitis and an early diagnosis is crucial. Patients will deteriorate rapidly with systemic inflammatory response syndrome, unless the perforation is minimal. In patients with a significant perforation, resuscitation with surgery to debride the mediastinum and place a T-tube within the oesophagus is needed in most cases.
Achalasia
This refers to oesophageal dysmotility secondary to failure of smooth muscle of the lower oesophageal sphincter to relax during swallowing. It causes progressive dysphagia and regurgitation. The acute history of excessive vomiting is more suggestive of oesophageal rupture, achalasia would likely present with a longer history.
Hiatus hernia
The cardia protrudes into the thoracic cavity through the phrenoesophageal membrane into the thoracic cavity in a hiatus hernia. The most common is a sliding hiatus hernia,
which causes symptoms of reflux. It is unlikely to present with a patient in extremis.
Mallory–Weiss tear
A Mallory–Weiss tear is a tear in the mucosal lining of the oesophagus that classically occurs after heavily vomiting (often following an alcohol binge as described in the case
history). Given the patient is in extremis, this is not likely to be the cause as most cases of bleeding from this are self-limiting and usually minor.
Oesophageal cancer
Oesophageal cancer in a young patient would be rare.
A 24-year-old man is taken to the Emergency Department by his friends as they are concerned regarding blood in his vomit. The patient is very inebriated and a collateral history from them reveals he has been on a stag night and drinking heavily. After arriving home he had been vomiting excessively and recent vomitus has shown streaks of blood. He is haemodynamically stable, with unremarkable blood results, no past medical history and no change in bowel habits with normal stool.
Which one of the following conditions is most likely?
1) Achalasia
2) Diffuse oesophageal spasm
3) Mallory–Weiss tear
4) Para-oesophageal hernia
5) Oesophageal Ca
Explanation
Mallory–Weiss tear
A Mallory–Weiss tear occurs after excessive vomiting, often secondary to alcohol and involves a mucosal tear in the mucosa and submucosa. In most cases the bleeding is minor and self-limiting, and no intervention is required.
Achalasia
Achalasia refers to oesophageal dysmotility secondary to failure of smooth muscle of the lower oesophageal sphincter to relax during swallowing. It causes progressive dysphagia and regurgitation. The acute history of excessive vomiting and alcohol binge is more suggestive of a Mallory–Weiss tear, achalasia would likely present with a longer history.
Diffuse oesophageal spasm
This is a disorder of oesophageal dysmotility causing uncoordinated contractions of the oesophagus. Diagnosis can be difficult but it usually presents with intermittent non-
cardiac retrosternal pain and dysphagia.
Para-oesophageal hernia
The acute history given would not support the diagnosis of a para-oesophageal hernia as this tends to present with reflux symptoms and dysphagia.
Oesophageal Ca
This would present with weight loss and dysphagia for which the patient has no history. Additionally, oesophageal cancer in a 24-year-old would be rare.
An overweight 41-year-old woman presents to the Emergency department with central abdominal pain which is colicky in nature, and vomiting. On further questioning it
emerges she has not opened her bowels for 4 days, and on examination her abdomen is distendedand tender centrally. A plain abdominal radiograph shows dilated loops of small bowel, however, no air is seen in the large bowel or rectum. The radiograph also suggests evidence of pneumobilia.
What is most likely the cause of her symptoms?
1) Caecal volvulus
2) Crohn’s disease
3) Diverticular stricture
4) Gallstone ileus
5) Sigmoid volvulus
Explanation
Gallstone ileus
It is unusual for a gallstone to cause problems outside of the biliary tree, but sometimes a gallstone can pass into the small bowel and cause an obstruction known as a gallstone
ileus. This occurs after formation of a cholecystoduodenal fistula between the gall-bladder and duodenum. This causes the presence of air in the biliary tree, (ie pneumobilia), which can be seen on imaging.
Caecal volvulus
A caecal volvulus would cause dilated loops of small bowel, however, it would not cause pneumobilia, which is presence of air in the biliary tree, and is more indicative of gallstone ileus.
Crohn’s disease
Crohn’s disease is an inflammatory bowel disease and can affect the entire length of the gastrointestinal tract with transmural inflammation noted and although Crohn’s disease can cause obstruction, pneumobilia is unlikely. Additionally, the history is more indicative of cholecystitis secondary to gallstones.
Diverticular stricture
The lack of air in the rectum suggests an obstructive picture and the absence of dilated large bowel loops goes against the diagnosis of large bowel obstruction caused by a diverticular stricture.
Sigmoid volvulus
A sigmoid volvulus causes a classic coffee-bean sign on abdominal X-ray.
A 20-year-old man is admitted as an emergency with suspected appendicitis. This is the most common emergency surgical operation and approximately 80 000 people a year
are admitted with this diagnosis in the UK. How may the symptoms present?
1) Colicky central abdominal pain shifting to right iliac fossa
2) Dysuria, frequency of micturition and fever
3) Nausea and vomiting
4) Sharp localised pain, worse on movement and found at McBurney’s point
5) All of the above
Explanation
All of the above
The possibilities of presentation of appendicitis are numerous; good knowledge of the
signs and symptoms of appendicitis is paramount as it is often a clinical diagnosis,
supported with evidence of inflammation in the blood results, and occasionally with
imaging, primarily ultrasound or in older patients computed tomography (CT) scan.
Colicky central abdominal pain shifting to right iliac fossa
The usual presenting history is of colicky and vague central abdominal pain shifting to
the right iliac fossa after 24 h and becoming constant with associated nausea and
pyrexia.
Dysuria, frequency of micturition and fever
History may mimic urinary tract infection with dysuria, frequency and fever. This
generally occurs when the appendix lies on or adjacent to the bladder.
Nausea and vomiting
Nausea and vomiting are common associated symptoms of appendicitis, and are usually
seen as the condition progresses.
Sharp localised pain, worse on movement and found at McBurney’s point
The classical area of maximal tenderness is one-third of the distance from the anterior
superior iliac spine (ASIS) to the umbilicus. This is McBurney’s point. Specific signs on
abdominal examination are as follows:
Psoas stretch sign – passive extension or hyperextension of the hip increases abdominal
pain due to the psoas muscle being in contact with the inflamed appendix.
Rovsing’s sign – Palpation in the left iliac fossa may produce pain at the site of
tenderness in the right iliac fossa due to movement of the inflamed parietal peritoneum.
A 64-year-old woman presents with severe abdominal pain
and a history of vomiting initially that has now settled, although she continues to retch. On assessment she has widespread abdominal tenderness and looks unwell. A blood gas confirms a metabolic acidosis and the nursing staff report they are unable to pass a nasogastric (NG) tube.
What is the likely diagnosis?
1) Gastric volvulus
2) Oesophageal perforation
3) Perforated duodenal ulcer
4) Sigmoid volvulus
5) Small bowel obstruction
Explanation
Gastric volvulus
Gastric volvulus classically involves Borchardt’s triad; epigastric pain, retching without
vomiting and the inability to pass an NG tube. The twisting can occur in two ways: along
the long axis of the stomach (organoaxial) or around the axis perpendicular to the
stomach (mesenteroaxial).
Oesophageal perforation
Oesophageal rupture or perforation occurs mostly either secondary to trauma eg during
endoscopy or due to prolonged forceful vomiting. It is likely to also cause retrosternal
pain and would not prevent passage of an NG tube.
Perforated duodenal ulcer
A perforated ulcer will cause epigastric abdominal pain with peritonitis and possibly
guarding depending on the extent of the perforation. Additionally, it would not obstruct
the passage of a nasogastric tube.
Sigmoid volvulus
Sigmoid volvulus is more likely to present with abdominal pain and complete
constipation. If vomiting were to occur, it would be faeculent in nature.
Small bowel obstruction
This would not prevent the passage of an NG tube.
A 3-year-old boy presents to the Emergency Department with a severe sore throat, temperature of 38.7 °C and noisy breathing. The child is sat forwards and drooling.
What is the most important first step to perform in the management of this child?
1) Examination of the oropharynx
2) Flexible laryngoscopy
3) IV access and blood cultures
4) IV antibiotics
5) Urgently call the paediatric anaesthetist
Explanation
Urgently call the paediatric anaesthetist
The child has acute epiglottitis. Acute epiglottitis tends to present with a rapidly
progressive sore throat and in the late stages, may be associated with inspiratory
stridor. The child will be toxic with a raised temperature and tends to sit forwards and
drool. The loose connective tissue swelling in the epiglottis can occlude the airway and
care must be taken to avoid this. Any attempts to examine the child, including simple
cannulation can precipitate laryngospasm and airway occlusion. Therefore the most
important step in managing this patient is to get the help of someone with experience in
managing paediatric airways.
Examination of the oropharynx
Any attempts to examine the child, including examination of the oropharynx can
precipitate laryngospasm and airway occlusion, and therefore is ill advised and should
be avoided.
Flexible laryngoscopy
As mentioned, any attempt to examine this child may trigger life-threatening airway
occlusion and so should be avoided.
IV access and blood cultures
While this child is likely to be septic given the history, no attempt to cannulate the
patient should be undertaken as this may cause airway obstruction.
IV antibiotics
This would require cannulation, which should not be undertaken.
A 22-year-old woman student presents with epistaxis. This
fails to improve with pressure. Which one of the following methods would be attempted next?
1) Embolisation
2) External carotid artery ligation
3) Sphenopalatine artery ligation
4) Trans-sphenoidal ligation
5) Use of silver nitrate
Explanation
Use of silver nitrate
Application of sustained pressure for epistaxis is the primary first aid measure. If
unsuccessful, silver nitrate or chemical cautery can be utilised and can be very effective
in stemming epistaxis. However, in higher risk patients (particularly the elderly on
anticoagulation), nasal packing, both anterior and posterior, may be required.
Progression to surgery and artery ligation would then be the next management method.
Embolisation
Maxillary artery embolisation is becoming a more frequently used alternative to artery
ligation, however, this is reserved for specialist interventional radiology centres, and
would only be indicated for refractory bleeding despite cautery and nasal packing.
External carotid artery ligation
Ligation of the external carotid artery is reserved for life-threatening epistaxis, other
measures should be trialled to stem bleeding before artery ligation.
Sphenopalatine artery ligation
Ligation of local arteries, such as the sphenopalatine, may be required and are not
uncommon, however, more conservative measures should be
trialled before progression to surgical ligation.
Trans-sphenoidal ligation
This is not indicated for epistaxis.
An 18-year-old gap-year student presents to the Accident and Emergency Department (A&E) with right-sided abdominal pain and rigours. On examination, he is clammy to touch, febrile and tender in the right upper quadrant. His only medical history of note is dysentery while on a backpacking trip to Mexico 2 months ago. What is the most likely diagnosis?
1) Amoebic liver abscess
2) Cholangiocarcinoma
3) Gallstones
4) Hydatid disease
5) Pancreatitis
Explanation
Amoebic liver abscess
Amoebic liver abscesses often occur secondary to Entamoeba histolytica infection in the
gut entering the portal circulation and spreading to the liver. The condition occurs
mostly in endemic areas and affects travellers visiting them. Clinical features include
abdominal pain (most commonly in the right upper quadrant), fever, rigours and
nausea/vomiting. Some patients will report a history of dysentery recently. Imaging
with ultrasound scan or computed tomography (CT) is useful in the diagnosis, and can
be combined with aspiration which produces a thick fluid resembling anchovy sauce.
Treatment mostly consists of metronidazole sometimes with percutaneous drainage.
Cholangiocarcinoma
Cholangiocarcinoma would be rare in an 18-year-old adult.
Gallstones
Gallstones presents with colicky, right upper quadrant abdominal pain. It may be
associated with pyrexia if there is cholecystitis. It would be uncommon in young men,
more often presenting in overweight, middle-aged women. As such, the case history
does not fit with this diagnosis.
Hydatid disease
Hydatid disease is caused by parasitic Echinococcus tapeworms, it causes the formation
of slow growing cysts, usually in the liver or lungs, containing hydatid fluid. Patients can
often be asymptomatic. Intermediate hosts are grazing animals, with dog and cats often
acting as definitive hosts. Humans are only accidental hosts. Given this patient has a
history of dysentery while travelling, this supports the diagnosis of amoebic abscess
formation, as opposed to hydatid disease.
Pancreatitis
This commonly presents with epigastric abdominal pain radiating through to the back.
The most common aetiology in the UK are gallstones or alcohol, both of which would be
unlikely in a young adult.
A 75-year-old man who has had a prolonged intensive care unit admission following an emergency abdominal aortic aneurysm repair is referred to the surgical team because of
increased abdominal pain and discomfort noted when he is moved. His bloods and urine show normal amylase and lipase levels respectively. A computed tomography (CT) scan showed no leak from the anastomosis, but an ultrasound scan showed a dilated gall-bladder with oedema in the wall. However, no gallstones were noted. What is the most likely diagnosis?
1) Aorta-enteric fistulae
2) Biliary colic
3) Cholecystitis
4) Ileus
5) Pancreatitis
Explanation
Cholecystitis
Acalculous cholecystitis is rare, but can occur in critically ill patients often
on the intensive care unit. The mechanism for this is thought to involve gall-
bladder stasis due to analgesia or parenteral nutrition. Imaging includes
ultrasound, which can show a dilated gall-bladder with oedema in the wall.
Aorta-enteric fistulae
Aortic-enteric fistulae occur mostly following abdominal aortic aneurysm (AAA) repair
in which a communication has developed between the aorta and the small bowel. They
result in very heavy blood loss into the small bowel and are often fatal.
Biliary colic
Biliary colic secondary to gallstones is common and presents as colicky abdominal pain.
Given that no gallstones were identified on ultrasound and the prolonged stay in
intensive care, this is less likely than acalculous cholecystitis.
Ileus
Ileus is common in patients who have undergone major abdominal surgery and is often
due to handling of the bowel during the operation. It usually resolves after a few days
and ‘bowel rest’. The findings of a distended gall-bladder suggest a more likely
pathology.
Pancreatitis
Pancreatitis usually presents with epigastric pain, and the most common causes are
gallstones or alcohol. Given no gallstones were identified on ultrasound but the gall-
bladderis distended and oedematous, acalculous cholecystitis must be considered.
Moreover, this patient has normal lipase and amylase levels.
A 57-year-old woman is admitted under the general surgical team with severe epigastric pain radiating to her back. She has a history of profuse vomiting and a serum amylase comes back as 1200 U/ml. She is started on treatment for acute pancreatitis and further tests are done.
Result Normal
Serum amylase
1200 U/l
<200 U/l
White-cell count
12 × 10 9 /l
4–11 × 10 9 /l
Calcium
1.9 mml/l
2.20–2.60 mmol/l
Urea
17 mmol/l
2.5–6.5 mmol/l
LDH
672 IU/l
100–190 IU/l
Albumin
31 g/l
35–55 g/l
Blood glucose
11 mmol/l
3.5–5.5 mmol/l
Arterial blood gas pa (O2 )
13.1 kPa
10.5–13.5 kPa
How severe is her pancreatitis using the modified Glasgow criteria?
1) Mild
2) Moderate
3) Severe
4) Haemorrhagic
5) Necrotic
Explanation
Severe
The modified Glasgow criteria can be used to assess the severity of acute pancreatitis by
using parameters taken within the first 48 h following admission. The most common
aetiologies in the UK are either gallstones or alcohol. This patient has significantly
deranged physiological and biochemical markers, increasing their Glasgow score,
indicating a severe pancreatitis.
Mild = 1 positive factor, moderate = 2 factors, severe = 3 positive factor.
Mild
The patient has significant derangements of the physiological and biochemical markers
used to assess severity of pancreatitis, increasing the Glasgow score above the mild
category.
Moderate
As above, the patient has significant derangements in the parameters used to calculate
the Glasgow score, causing the severity to be categorised as severe.
Haemorrhagic
The terms haemorrhagic and necrotic describe the computed tomography
(CT) scan appearance of the pancreas, and influence the Balthazar score.
Necrotic
Again, this term is reserved for describing the appearance of the pancreas on CT.
A 45-year-old woman with known gallstones is admitted with epigastric pain and vomiting. Her serum amylase is 1194 U/1ml. An abdominal ultrasound shows gallstones in the common bile duct (CBD) with a dilated CBD and it is thought she has acute gallstone pancreatitis. Chest X-ray (CXR) and abdominal X-ray (AXR) are unremarkable and her other test results are as follows:
Result Normal
White-cell count. 9 × 10 9 /l. 4–11 × 10 9 /l
Calcium. 2.3 mml/l. 2.20–2.60 mmol/l
Urea. 9 mmol/l. 2.5–6.5 mmol/l
LDH. 352 IU/l. 100–190 IU/l
Albumin. 35 g/l. 35–55 g/l
Blood glucose. 7 mmol/l. 3.5–5.5 mmol/l
Arterial blood gas pa (O2 ) 12 kPa. 10.5–13.5 kPa
With this information, which scoring system can be best used to assess the
severity of her pancreatitis?
1) APACHE
2) Balthazar
3) Glasgow
4) Hinchey
5) Ranson
Explanation
Glasgow
The modified Glasgow criteria can be used to assess the severity of acute pancreatitis by
using parameters taken within the first 48 h following admission.
APACHE
The APACHE score requires additional information to those provided eg blood pressure,
urine output and is used to assess mortality risk for patients admitted to intensive care
regardless of causative pathology. Balthazar
The Balthazar grade is based on the computed tomography (CT) scan appearance of the
pancreas.
Hinchey
The Hinchey classification grades diverticulitis not pancreatitis.
Ranson
Ranson’s criteria are valid for alcohol-induced pancreatitis and can only be applied after
48 h.
The severity of acute pancreatitis is typically classified based on various criteria, including clinical, biochemical, and radiological parameters. Here are some commonly used criteria:
1. Ranson’s Criteria: Developed by Dr. John Ranson in the 1970s, this system includes 11 criteria—5 assessed at admission and 6 during the first 48 hours. The presence of 3 or more of these criteria indicates severe pancreatitis and a poorer prognosis. 2. APACHE II Score (Acute Physiology and Chronic Health Evaluation II): This scoring system evaluates physiological parameters within the first 24 hours of admission. A higher score indicates a more severe condition and a worse prognosis. 3. Balthazar Score: This system assesses the severity of pancreatitis based on CT imaging findings. It uses a grading system from A to E, with E indicating the most severe form of pancreatitis. 4. Imrie Score: Similar to Ranson’s criteria, the Imrie scoring system evaluates clinical and laboratory parameters to predict the severity of pancreatitis. A score of 3 or more indicates severe pancreatitis. 5. CT Severity Index (CTSI): This index evaluates the extent of pancreatic and peripancreatic inflammation on CT imaging. It helps in predicting the severity of pancreatitis and guiding management decisions. 6. Bedside Index for Severity in Acute Pancreatitis (BISAP): This simplified scoring system assesses five parameters at admission—blood urea nitrogen, impaired mental status, systemic inflammatory response syndrome, age, and pleural effusion—to predict the severity of acute pancreatitis. 7. CRP (C-reactive protein): Elevated levels of CRP have been associated with severe acute pancreatitis and can be used as a marker for assessing severity. 8. Modified Glasgow Criteria: Similar to Glasgow Coma Scale, this criteria assesses various clinical parameters and organ dysfunction to predict the severity of pancreatitis.
These criteria help clinicians in assessing the severity of acute pancreatitis, guiding treatment decisions, and predicting patient outcomes.
Sources:
• https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5734411/ • https://www.ncbi.nlm.nih.gov/books/NBK482291/
A 38-year-old woman with a 1-year history of intermittent epigastric pain presents with vomiting and abdominal distension. A plain abdominal radiograph reveals dilated loops of small bowel and pneumobilia. What is the most appropriate management?
1) Emergency laparotomy
2) Endoscopic retrograde cholangiopancreatography (ERCP)
3) Laparoscopic cholecystectomy
4) Percutaneous drainage of the gall bladder
5) Surgical closure of a cholecystoduodenal fistula
Explanation
Emergency laparotomy
This patient has a small bowel obstruction secondary to gallstone ileus. This is a surgical
emergency requiring urgent laparotomy. The gallstone usually impacts proximal to the
ileocaecal valve, and so it should be massaged through into the large intestine or
extracted via an enterotomy, so relieving the obstruction.
Endoscopic retrograde cholangiopancreatography (ERCP)
This intervention is used for the diagnosis and treatment of gallstones located in the
common bile duct. Again, this will not solve the small bowel obstruction which requires
emergency intervention.
Laparoscopic cholecystectomy
Laparoscopic cholecystectomy is indicated for those with recurrent cholecystitis with
evidence of gallstones. This does not, however, address the small bowel obstruction
which is a surgical emergency requiring laparotomy.
Percutaneous drainage of the gall bladder
Small bowel obstruction is a surgical emergency and requires intervention.
Percutaneous drainage of the gall bladder will not resolve the bowel obstruction and
therefore would be inappropriate.
Surgical closure of a cholecystoduodenal fistula
The gallstone will have passed into the small bowel through a biliary-enteric fistula, but
this does not usually require surgical repair. Such fistulae often lead to pneumobilia,
which can be seen on plain abdominal radiographs.
A previously fit and well 54-year-old man attends the General Practice Surgery, as his family have told him he is ‘looking yellow’. On examination, he is aundiced, but his abdomen is soft and non-tender. In the right upper quadrant (RUQ), his
gall bladder is palpable. A triple-phase computerised tomography (CT) reveals a tumour at the head of
the pancreas.
Which of the following phenomena is demonstrated?
1) Cope sign
2) Courvoisier’s law
3) Murphy’s sign
4) Psoas sign
5) Rovsing’s sign
Explanation
Courvoisier’s law
Courvoisier’s law states that in the event of painless jaundice and a palpable gall
bladder, the cause is unlikely to be gallstones. This is because the presence of gallstones
tends to make the gall bladder fibrosed, and therefore it is unlikely to become distended
in the event of an obstruction of the biliary tree.
Cope sign
Cope sign involves flexion and internal rotation of the right hip, causing pain if the
appendix is lying in close relation to the obturator internus.
Murphy’s sign
Murphy’s sign occurs when, upon pressing over the RUQ with two fingers, asking the
patient to breathe in causes pain and arrest of inspiration, as the inflamed gall bladder
impinges on the examiner’s fingers.
Psoas sign
Psoas sign involves extension of the hip, causing pain if there is a retrocaecal appendix.
Rovsing’s sign
Rovsing’s sign describes pain in the right iliac fossa more than in the left iliac fossa
when the left iliac fossa is pressed.
A 37-year-old woman presents to the acute surgical take. She is
postpartum and on the oral contraceptive pill, she complains of right upper quadrant pain, nausea and vomiting. On examination she has
hepatosplenomegaly and ascites.
What is the most likely cause for this?
1) Alcoholic cirrhosis
2) Budd–Chiari syndrome
3) Pylephlebitis after acute appendicitis
4) Splenectomy
5) Tricuspid valve incompetence
Explanation
Budd–Chiari syndrome
Portal hypertension with a pressure of over 20 mmHg is commonly caused by pre-hepatic problems such as portal vein thrombosis, hepatic disease such as cirrhosis and post-hepatic problems such as tricuspid valve incompetence and Budd–Chiari syndrome of hepatic vein thrombosis. Budd–Chiari syndrome commonly results in
hepatosplenomegaly and ascites. It is associated with pregnancy and being postpartum.
Use of the oral contraceptive pill can also increase the risk for venous thromboembolic disease.
Alcoholic cirrhosis
There is no history of alcohol excess in the history given, so alcoholic cirrhosis would be unlikely.
Pylephlebitis after acute appendicitis
Pylephlebitis is an uncommon thrombophlebitis of the portal vein, caused by an infection such as appendicitis. The case history given, however, is not consistent with appendicitis, which commonly presents with vague, centralised abdominal pain, localising to the right iliac fossa over 24 hours.
Splenectomy
Palpable splenomegaly is noted on examination; therefore, splenectomy cannot be the cause.
Tricuspid valve incompetence
Although tricuspid valve incompetence can cause portal hypertension, and subsequently hepatosplenomegaly, as described in the case history, as the patient is postpartum, Budd–Chiari syndrome is the more likely diagnosis.
An elderly, confused man presents with a
grossly distended tympanic abdomen
and absolute constipation. A plain abdominal radiograph reveals a grossly
dilated large intestine , but no evidence of pneumoperitoneum. What is the most appropriate initial management?
1) Computerised tomographic scan of the abdomen and pelvis
2) Laparotomy and sigmoid colectomy
3) Laxatives
4) Sigmoidoscopy
5) Trephine ileostomy
Explanation
Computerised tomographic scan of the abdomen and pelvis
For this scenario, the differential diagnosis is pseudo-obstruction, sigmoid volvulus and carcinoma of the distal colon/rectum. Computerised tomography of the abdomen and pelvis is the best option, as it permits the diagnosis to be obtained and the correct treatment instituted. Pseudo-obstruction may be associated with an underlying precipitating condition such as renal failure, electrolyte abnormalities, myxoedema, stroke, myocardial infarction, chest infection and retroperitoneal malignancy (pseudo- obstruction in the presence of retroperitoneal malignancy is known as Ogilvie syndrome). Medical management comprises correction of electrolyte abnormalities, treatment of the underlying cause and use of the anticholinergic neostigmine (with heart monitor surveillance).
Laparotomy and sigmoid colectomy
At present, the diagnosis is not clear and further imaging is required, so the most
appropriate treatment can be initiated. Sigmoid colectomy is indicated in an elective setting for recurrent sigmoid volvulus. Sigmoid volvulus often has the characteristic ‘coffee bean’ sign on plain abdominal radiograph. Obstructed colon caused by a distal neoplasm is open to several surgical options following adequate resuscitation. These include stenting, defunctioning stoma, primary resection and anastomosis following on-
table colonic lavage or Hartmann’s procedure. Note if the patient was too unstable for the computerised tomography scanner, then exploratory laparotomy may indeed be indicated first.
Laxatives
Initiating treatment before identifying the causative pathology could cause further harm to the patient. For example, if the obstruction is caused by a carcinoma of the sigmoid colon, use of laxatives could precipitate a perforation.
Sigmoidoscopy
Sigmoidoscopy may be beneficial in decompressing the colon with a flatus tube when a sigmoid volvulus is the causative pathology. However, the diagnosis is not clear in this case, so further imaging is required before intervention. Fixation procedures for
recurrent sigmoid volvulus, such as sigmoidopexy, have been performed but are associated with high recurrence rates.
Trephine ileostomy
This is an operative technique to create a permanent end-colostomy without undergoing a laparotomy. Again, this would not be indicated until the diagnosis is clarified with further imaging.
An 82-year-old man presents with intermittent abdominal pain and vomiting. On further questioning, it is noted he has
not opened his bowels for 5 days and not passed wind for 2 days. On examination, he looks uncomfortable and his abdomen is grossly distended. His only past medical history of note is an open repair of an
abdominal aortic aneurysm (AAA) 4 months ago, from which he recovered well.
What is the most likely cause of his symptoms?
1) Adhesional small bowel obstruction
2) Aorto-enteric fistula
3) Incisional hernia
4) Diverticular disease
5) Sigmoid tumour
Explanation
Adhesional small bowel obstruction
This patient has a number of characteristic features of small bowel obstruction:
vomiting, constipation and abdominal distension. Adhesions are the most common
cause of small bowel obstruction in the developed world and can occur following any
form of abdominal surgery.
Aorto-enteric fistula
Aortic-enteric fistulae occur mostly following AAA repair, in which a communication has
developed between the aorta and the small bowel. They result in very heavy blood loss
into the small bowel, causing rectal bleeding, and are often fatal.
Incisional hernia
There is nothing in the history of the case to suggest the patient has an incisional hernia
which could be causing obstruction.
Diverticular disease
There are no features in the case history that suggest diverticular disease as the cause
for obstruction.
Sigmoid tumour
Carcinoma of the colon, particularly the sigmoid colon, is a common cause for large
bowel obstruction. However, there are no features in the history that point to a sigmoid
tumour as the cause for obstruction, and given this gentleman has had previous major
abdominal surgery, adhesional bowel obstruction is more likely.
An 88-year-old woman, who has a past medical history of dementia and is bed-bound in a nursing home, presents with a history of constipation for the last 7 days. Her carers have noted her abdomen has become grossly distended and is tender to touch. An abdominal X-ray demonstrates the coffee bean sign.
What would be an appropriate treatment option to manage this patient initially ?
1) Antibiotics
2) Computerised tomography (CT) scan
3) Exploratory laparotomy
4) Insertion of flatus tube
5) Sigmoid colectomy
Explanation
Insertion of flatus tube
The coffee bean sign and clinical history are highly suggestive of sigmoid volvulus,
which commonly occurs in elderly patients who have a chronic history of constipation.
Insertion of a flatus tube represents a relatively low-risk procedure that could
potentially resolve the patient’s symptoms and therefore would be a good first-line
option. If this fails, the next step would be flexible sigmoidoscopy.
Antibiotics
Antibiotics are not indicated, as there are no features in the case history suggestive of
infection.
Computerised tomography (CT) scan
A CT scan would confirm the diagnosis but offers no therapeutic benefit to the patient.
Exploratory laparotomy
Given the patient’s pre-morbid status of being a bed-bound nursing home patient, the
risks of any major surgery, eg laparotomy, would be great. Depending on the severity of
the patient’s dementia, they may also lack capacity to consent to surgery, and it is likely
to be against the patient’s best interests to undergo major surgery.
Sigmoid colectomy
Again, major surgery for this patient would be ill-advised.
An 85-year-old man is recovering 2 days after a hemiarthroplasty for a fractured neck of femur. The nurses call you as he has started vomiting profusely and his
abdomen is distended. He has not opened his bowels for 3 days and on examination, he has a distended, tender abdomen. He has never undergone any abdominal surgery and has no palpable herniae. His rectum is empty. His blood tests show a potassium level of 2.6 and a creatinine level of 144.
What is the most likely diagnosis?
1) Adhesional small bowel obstruction
2) Constipation secondary to opioids
3) Incisional hernia
4) Obstruction hernia
5) Pseudo-obstruction
Explanation
Pseudo-obstruction
The history of vomiting, constipation and a distended abdomen is suggestive of an
obstruction. In this case, this gentleman has developed pseudo-obstruction, which often
resembles bowel obstruction and can occur in any post-operative patients and can also
be triggered by deranged electrolytes, eg hypokalaemia. It is best managed
conservatively through correcting the underlying causes.
Adhesional small bowel obstruction
The most common cause for small bowel obstruction, namely adhesions, is unlikely in
this case, given the patient has never had abdominal surgery.
Constipation secondary to opioids
Although the patient is likely to have been prescribed opioid-based medication before
and following the hemi-arthroplasty, his empty rectum points away from constipation.
Incisional hernia
No hernias are palpable on examination and he has not previously undergone
abdominal surgery; as such, this is incorrect.
Obstruction hernia
This is unlikely, as there are no palpable hernias on examination and no history of
previous abdominal surgery.
A frail 87-year-old woman is brought in by her concerned family as she has started vomiting and not opened her bowels or passed wind for 3 days. On examination, she looks unwell with a distended abdomen, but no previous incisions, and on further questioning, she reports the pain radiates along the right upper medial thigh. Vaginal examination reveals a swelling in the right side wall of the vagina.
What is the most likely diagnosis?
1) Adhesional small bowel obstruction
2) Epigastric hernia
3) Para-umbilical hernia
4) Obturator hernia
5) Sigmoid volvulus
Explanation
Obturator hernia
Pain which radiates to the thigh is suggestive of pressure on the obturator nerve which supplies sensation to the medial thigh. This can be present in around 50% of patients with an obturator hernia. Obturator hernias occur most commonly in frail old women and often there are no signs. If pressure is present over the obturator nerve, patients often hold the leg flexed to reduce pain. Rectal, and more often vaginal, examination can reveal a swelling in the region of the obturator foramen.
Adhesional small bowel obstruction
Although the most common cause of small bowel obstruction is adhesions in the developed world, the lack of previous surgery makes this very unlikely.
Epigastric hernia
An epigastric hernia would not cause pain in the medial thigh and vaginal examination would be unremarkable. Furthermore, an epigastric hernia was not identified on examination of the abdomen.
Para-umbilical hernia
There were no hernias identified on abdominal examination, and a lack of previous abdominal surgery makes this diagnosis unlikely.
Sigmoid volvulus
Sigmoid volvulus is a common cause for bowel obstruction, particularly in the elderly.
However, it would not produce pain in the medial thigh and vaginal examination would be unremarkable.
A 32-year-old man develops an acutely painful arm after having been immobilised in a plaster cast, following a closed fracture of his distal radius 8 hours previously.
What is the most likely underlying diagnosis?
1) Compartment syndrome
2) Deep vein thrombosis
3) Early infection
4) Inadequate analgesia
5) Necrotising fascitis
Explanation
Compartment syndrome
Compartment syndrome is characterised by increased pressure within an unyielding osseofascial compartment, resulting in local tissue hypoxia. The earliest sign is pain out of proportion with the injury, particularly severe pain on passive muscle stretch. Pallor, paraesthesiae and pulselessness occur in late stages. Diagnosis is on clinical grounds, although compartment pressures can be measured. Raised creatine kinase (CK) levels are indicative of muscle necrosis. Fasciotomies to release the deep and superficial compartments should be performed early. Although more common in the lower limb, it is possible in the upper limb as well.
Deep vein thrombosis
While venous thromboembolic disease should be considered in those with prolonged immobilisation of a limb, development of a deep vein thrombosis within 8 hours is
improbable.
Early infection
Infection of the limb may be painful. However, there are no other signs of infection in the case history. Moreover, compartment syndrome should always be considered in an
acutely painful limb which has recently been immobilised in a plaster cast.
Inadequate analgesia
Adequate analgesia is important. However, effective immobilisation should provide sufficient pain relief. The history described is highly suggestive of compartment syndrome.
Necrotising fascitis
Necrotising fasciitis is a surgical emergency caused by bacterial infection of the fascia and subsequent necrosis of the subcutaneous tissues. It requires emergency debridement of affected areas with wide margins and intravenous antibiotics. In the case history, there is no suggestion of sepsis with haemodynamic compromise, which
would be seen with necrotising fasciitis.
A 2-year-old child is admitted to the Paediatric Emergency Department with malaise, rash, vomiting and fever. Mum reports that she has been off her food for the preceding 24 hours and has been running a low-grade temperature. She also points to a partial-thickness burn over her arm that has been de-roofed and treated with dressings by her general practitioner.
What is the likely diagnosis?
Select one answer only.
1) Anaphylaxis
2) Haemophilus influenza
3) Meningococcal septicaemia
4) Salmonella
5) Toxic shock syndrome
Explanation
Toxic shock syndrome
An unwell child with an unhealed burn must be treated for toxic shock syndrome until proven otherwise. Treatment will require management in the Intensive Care Department, with input from a paediatric consultant and the plastic surgery team. Circulatory support with cryoprecipitate may be necessary.
Anaphylaxis
This is a paediatric airway emergency which requires emergency administration of intramuscular adrenaline to prevent airway occlusion from a hypersensitivity reaction.
The history described is not consistent with anaphylaxis, which presents rapidly with lip and tongue swelling, airway compromise and difficulty breathing.
Haemophilus influenzae
There is no suggestion of respiratory signs or symptoms in the case history, so this is unlikely, despite being a common paediatric presentation.
Meningococcal septicaemia
Meningococcal meningitis leading to septicaemia is life-threatening and should always be considered in an acutely unwell child. However, given the unhealed burn, toxic shock
syndrome is more probable.
Salmonella
Salmonella infection would present with gastrointestinal symptoms and signs, of which there are none in the case history, other than anorexia which is a common and non-
specific symptom for an unwell child.
A 17-year-old man presents with right-sided abdominal pain, which is worse on movement, and anorexia. He is listed for an open appendicectomy which reveals a normal appendix. Further exploration intra-operatively reveals a normal caecum, and a terminal ileum with no obvious inflammation. However, an outpouching is noted on the terminal ileum around 60 cm from the ileocaecal valve.
What is the most likely diagnosis?
1) Abdominal tuberculosis (TB)
2) Appendicitis
3) Caecal carcinoma
4) Crohn’s disease
5) Meckel’s diverticulum
Explanation
Meckel’s diverticulum
The description of an outpouching in this location on the antimesenteric border is consistent with a Meckel’s diverticulum. These are embryological remnants of the vitello-intestinal duct and are a free diverticulum of the terminal ileum. They occur in 2% of the population, are commonly 2 feet (60 cm) from the ileocaecal valve, often 2
inches (5 cm) in length and twice as common in men than women. Acute inflammation may mimic appendicitis and it is important when performing an appendicectomy to search for a Meckel’s diverticulum if the appendix is normal.
Abdominal tuberculosis (TB)
The case history is not consistent with abdominal TB and no risk factor for TB is described.
Appendicitis
The normal appendix goes against a diagnosis of acute appendicitis.
Caecal carcinoma
The normal caecum makes a caecal tumour causes the symptoms very unlikely, furthermore, a caecal carcinoma in a 17-year-old adolescent would be rare.
Crohn’s disease
The lack of inflammation in the terminal ileum makes Crohn’s unlikely.
An 89-year-old woman is seen on the post-take ward round following CT scanning. She presented with tenderness in the left iliac fossa for 7 days. She has localised tenderness but feels better than on admission yesterday. Past medical history reveals a cerebrovascular accident (CVA) 5 years ago which has left her bed bound with chronic obstructive pulmonary disease (COPD), requiring multiple admissions. Her CT scan shows diverticulitis with a localised perforation. Blood results show a white-cell count of 18 × 10 9 /l (normal 4–11 × 10 9 /l) and C-reactive protein 160 mg/l (normal 0–10
mg/l).
What is the best initial management?
1) Discharge
2) Intravenous antibiotics and close monitoring
3) Laparotomy and primary anastomosis
4) Laparoscopic resection
5) Laxatives
Explanation
Intravenous antibiotics and close monitoring This elderly patient has a localised diverticular perforation with absence of peritonitis and is showing signs of improvement. She has significant past medical history that would make her a high risk candidate for surgery, therefore conservative management with IV antibiotics and close monitoring for signs of deterioration is the best management path.
Discharge
Discharge is obviously not appropriate at this point.
Laparotomy and primary anastomosis
A primary anastomosis would not be advisable in an acute case with potential contamination leading to a higher chance of anastomotic leak. An anastomotic leak could be disastrous in this patient and so a colostomy would be best surgical practice if she were to be taken to theatre.
Laparoscopic resection
If surgical intervention were required, laparoscopy may be difficult due to her COPD, particularly as carbon dioxide insufflation of the abdominal cavity will splint the diaphragm, making ventilation more difficult.
Laxatives
Laxatives are not the primary concern in this patient’s acute management.
A 78-year-old nursing home resident is admitted with severe diarrhoea and left iliac fossa pain. At flexible sigmoidoscopy diffuse pseudomembranes are seen. Biopsy is neutrophil rich.
What is the most likely diagnosis?
1) Crohn’s disease
2) Melanosis Coli
3) Malignancy
4) Pseudomembranous colitis
5) Ulcerative Colitis
Explanation
Pseudomembranous colitis
The appearance of pseudomembranes are typical for pseudomembranous colitis caused by Clostridium difficile. This pathogen is more common in nursing home or hospitalised patients, and is often associated with antibiotic use. High risk antibiotics include; fluoroquinolones, cephalosporins and clindamycin. Prevention of the spread of C. difficileinvolves isolation of infected patients, judicious hand hygiene and measured antibiotic use.
Crohn’s disease
Crohn’s disease is an inflammatory bowel condition affecting the entire length of the gastrointestinal tract. Biopsy would demonstrate transmural inflammation and presentation of Crohn’s disease is usually in early 20s.
Melanosis Coli
This is a benign condition identified on colonoscopy in which there is a disorder of pigmentation of the endothelium of the colon. As the condition is benign, it would not produce the symptoms described in the case history.
Malignancy
Malignancy can present with a change in bowel habit, although it is usually less dramatic than severe diarrhoea, while malignancies are usually painless until complications such as perforation or obstruction occur.
Ulcerative Colitis
Ulcerative colitis is an inflammatory bowel disease in which inflammation is limited to the colon. It presents in the early 20s, so a diagnosis of ulcerative colitis in the elderly is uncommon.
A 22-year-old right-hand-dominant woman is referred to the hand surgery unit with pain and swelling of her right index finger. She thinks she has sustained a small puncture wound
to the finger while helping her dad with gardening 2 days ago. On examination the finger is diffusely swollen, erythematous and held in a slightly flexed position. It is exquisitely painful to passively extend the finger , and most tender on the volar aspect.
What is the most likely diagnosis?
1) Cellulitis
2) Flexor tendon sheath infection
3) Foreign body
4) Mallet finger
5) Paronychia
Explanation
Flexor tendon sheath infection
A flexor tendon sheath infection is an infection of the sheath that forms the lubricating surface around the flexor tendons to the hand. This constitutes an emergency as severe infection can destroy these surfaces resulting in a painful and stiff finger. In most cases there will be a history of penetrating trauma before the infection.
There are four main signs of a flexor tendon sheath infection:
*diffuse swelling of the digit
*the digit will be partially flexed
*tenderness along the flexor tendon sheath
*pain on passive extension of the digit.
Cellulitis
Cellulitis would certainly be included in the differential diagnosis given the case history, however, cellulitis would not cause severe pain on passive extension of the finger.
Foreign body
This will likely produce localised inflammation and swelling around the site of the foreign body. Additionally, pain on passively extending the finger suggests tendon sheath infection.
Mallet finger
Mallet finger describes the deformity of disruption of the extensor digitorum tendon at the distal interphalangeal joint with blunt force trauma being the causative injury. The deformity is not described and the patient has not suffered blunt force trauma in the case history.
Paronychia
Paronychia is a local infection around a fingernail that often begins as a cellulitis before developing into an abscess which requires drainage. This is not described in the case history above.
Question:
A 60-year-old woman who had undergone a successful left renal transplant 1 week earlier presents with fever, oliguria, and rising serum creatinine. She has been referred back to the transplant team for ongoing care. Which is the best management option in such a scenario?
Options:
1. It is a picture of acute rejection characterized by preformed antibodies to donor human leukocyte antigen (HLA) and should be treated with intravenous hydrocortisone 2. Tacrolimus should be the first-line treatment 3. The woman has chronic graft rejection 4. Treatment is with prednisolone 5. Urinary obstruction, infection, reduced blood supply and drug toxicity should be considered before treating for acute graft rejection
Correct Answer:
5. Urinary obstruction, infection, reduced blood supply and drug toxicity should be considered before treating for acute graft rejection
Explanation:
In the immediate post-transplant period, a rise in serum creatinine accompanied by fever and oliguria can be due to several potential causes. While acute rejection is a possibility, it is crucial to first rule out other common causes such as:
• Urinary obstruction: Check for any blockages in the urinary tract. • Infection: Infections can cause fever and impact kidney function, and must be treated appropriately. • Reduced blood supply: Ensure that the transplanted kidney is receiving adequate blood flow. • Drug toxicity: Medications used post-transplant can sometimes be nephrotoxic.
It is important to conduct a thorough evaluation to rule out these causes before initiating treatment for acute graft rejection. This approach helps to avoid unnecessary immunosuppressive therapy and addresses any reversible causes of graft dysfunction. Therefore, the correct management strategy in this scenario is to consider and investigate other potential causes of the symptoms before treating for acute graft rejection.
Question:
A 71-year-old diabetic man presents with cellulitis of his lower limb and pyrexia. He has pain on flexion of his ankle and it is tender to palpation. He develops a metabolic acidosis, and his blood pressure and urine output decline steadily throughout the day. Further examination reveals a reddish-bronze discoloration of the skin and decreased sensation, with the margin of infection advancing rapidly along the fascial planes. What is the diagnosis?
Options:
1. Cellulitis 2. Compartment syndrome 3. Myositis 4. Necrotising fasciitis 5. Rhabdomyolysis
Correct Answer:
4. Necrotising fasciitis
Explanation:
Necrotising fasciitis is a severe, rapidly progressing soft tissue infection characterized by widespread necrosis of the fascia and subcutaneous tissue. The key features in this case that point towards necrotising fasciitis include:
• Rapid progression of infection: The infection is advancing rapidly along the fascial planes. • Severe systemic signs: The patient has metabolic acidosis, declining blood pressure, and urine output, indicating severe sepsis or septic shock. • Characteristic skin changes: The reddish-bronze discoloration and decreased sensation are typical signs of necrotising fasciitis. • Tenderness and pain: Severe pain and tenderness beyond what is expected for cellulitis.
These findings distinguish necrotising fasciitis from other conditions such as cellulitis, compartment syndrome, myositis, and rhabdomyolysis, which do not typically present with such rapid progression and systemic deterioration. Immediate surgical intervention and broad-spectrum antibiotics are critical in the management of necrotising fasciitis to prevent further tissue destruction and systemic complications.
Explanation
Necrotising fasciitis
The progressive symptoms and signs with progressive haemodynamic compromise is
highly suggestive of necrotising fasciitis. It is a surgical emergency requiring
debridement with wide margins and treatment with high dose intravenous antibiotics.
Necrotising fasciitis often involves an initial focus such as a puncture wound, insect bite,
or scratch, and usually begins like cellulitis with hot, red and tender skin. However,
there may be an initial dissemination in the deeper tissue planes in the absence of
surface changes. At the leading edge of infection, there is reddish or
bronze discoloration of the skin and decreased sensation. The margin of infection
advances rapidly along fascial planes with thrombosis of perforating vessels, oedema,
necrosis and sloughing of the skin occur. Haemorrhagic bullae are relatively common.
Clinical features can be divided into early findings, which include pain, cellulitis, pyrexia,
tachycardia, swelling, induration and skin anaesthesia. Late findings may include severe
pain, purple or black skin discoloration, blistering, haemorrhagic bullae, crepitus,
discharge of ‘dishwater’ fluid, severe sepsis or systemic inflammatory response
syndrome and multiorgan failure.
Cellulitis
The condition has progressed from a simple cellulitis to necrotising fasciitis given the
worsening condition of the patient and clinical signs of a rapidly spreading fascial
infection.
Compartment syndrome
Compartment syndrome is characterised by increased pressure within an unyielding
osseofascial compartment, resulting in local tissue hypoxia. It is often an early
complication of long bone fractures. The case history given is not consistent with
compartment syndrome.
Myositis
Myositis is a broad term used to describe inflammation of the muscles, of which there
can be many causes including infection, medications, trauma or be immune mediated. It would not be associated with rapidly advancing skin changes or significant
haemodynamic compromise.
Rhabdomyolysis
This is condition causing skeletal muscle to break down rapidly following either direct
or indirect injury, and is a common pathology following a crush injury. A rapid
accumulation of myoglobin can cause significant renal failure. Again, rhabdomyolysis
would not produce skin changes and the history is consistent with necrotising fasciitis.
Question:
A 22-year-old man presents with altered behaviour, fever, and sore throat. His GP started him on a course of amoxicillin. His condition worsened and he was admitted to the Emergency Department with reduced consciousness and a rash over his torso that spread to his limbs together with neck stiffness. What is his likely diagnosis?
Options:
1. Disseminating intravascular coagulopathy (DIC) 2. HIV seroconversion 3. Meningococcal sepsis 4. Staphylococcus scalded skin syndrome 5. Viral infection
Correct Answer:
3. Meningococcal sepsis
Explanation:
The patient’s presentation is highly suggestive of meningococcal sepsis, which is a life-threatening condition caused by the bacterium Neisseria meningitidis. The key features pointing towards this diagnosis include:
• Altered behaviour and reduced consciousness: Suggest central nervous system involvement. • Fever and sore throat: Common initial symptoms of an infection. • Rash: The rash described, which spreads from the torso to the limbs, is characteristic of the petechial or purpuric rash seen in meningococcal sepsis. • Neck stiffness: A sign of meningitis, which often accompanies meningococcal infection.
Other Options:
1. Disseminating intravascular coagulopathy (DIC): A serious condition that involves widespread blood clotting and bleeding, but it does not typically present with the combination of symptoms described. 2. HIV seroconversion: Can present with fever and rash, but the acute presentation of reduced consciousness and neck stiffness is not typical. 3. Staphylococcus scalded skin syndrome: Characterized by widespread redness and skin peeling, typically without the severe systemic symptoms described. 4. Viral infection: While it can present with fever and rash, the severity of symptoms and rapid progression is more indicative of a bacterial cause such as meningococcal sepsis.
Given the critical nature of meningococcal sepsis and the need for urgent treatment, this is the most likely diagnosis.
Question:
An 83-year-old woman is admitted to the hospital with bloody diarrhea, painful abdominal cramps, fever, and leucocytosis. She has recently been discharged from the hospital having been treated for community-acquired pneumonia. What is her likely diagnosis?
Options:
1. Bowel cancer 2. C. difficile 3. Campylobacter jejuni 4. Inflammatory bowel disease 5. Peptic ulcer
Correct Answer:
2. C. difficile
Explanation:
The patient’s presentation is highly suggestive of a Clostridium difficile (C. difficile) infection, which is a common cause of antibiotic-associated colitis. The key features include:
• Recent antibiotic use: She was recently treated for pneumonia, likely with antibiotics, which is a major risk factor for developing C. difficile infection. • Bloody diarrhea and abdominal cramps: These are classic symptoms of C. difficile colitis. • Fever and leucocytosis: Indicate an infectious process, commonly seen in severe C. difficile infection.
Other Options:
1. Bowel cancer: While it can cause changes in bowel habits and bleeding, the acute presentation with fever and leukocytosis is more indicative of an infectious cause. 2. Campylobacter jejuni: Typically causes gastroenteritis with diarrhea, but the association with recent antibiotic use makes C. difficile more likely. 3. Inflammatory bowel disease (IBD): Can present with bloody diarrhea and abdominal pain, but the acute onset after antibiotic use and presence of fever and leukocytosis point more towards an infectious etiology. 4. Peptic ulcer: Usually presents with upper abdominal pain and possibly bleeding, but not with bloody diarrhea and systemic symptoms like fever and leukocytosis.
Given the patient’s history of recent hospitalization and antibiotic treatment, C. difficile infection is the most likely diagnosis.
Question:
A 45-year-old previously well man presents with a 3-day history of constant right groin pain and nausea. He has been limping for 2 days. Physical examination is unremarkable other than slight tenderness in the right groin on palpation. Urine dipstick is positive for blood and his white cells are raised. What is the likely diagnosis?
Options:
1. Cystitis 2. Incarcerated femoral hernia 3. Psoas abscess 4. Pyelonephritis 5. Renal calculi
Correct Answer:
3. Psoas abscess
Explanation:
While renal calculi can present with many of these symptoms, the correct diagnosis in this context is a psoas abscess. The key features supporting this diagnosis include:
• Constant right groin pain and limping: The psoas muscle, located in the retroperitoneal space, can cause referred pain to the groin and hip, leading to limping. • Nausea: Can occur due to systemic infection and inflammation. • Tenderness in the right groin: Psoas abscess often presents with pain that can be elicited on palpation or during movements that stretch the psoas muscle, such as hip flexion. • Raised white cells: Indicative of an infection. • Positive urine dipstick for blood: Can occur due to adjacent structures being affected or due to irritation/inflammation from the abscess.
Other Options:
1. Cystitis: Typically presents with urinary symptoms like dysuria, frequency, and urgency, which are not highlighted in this case. 2. Incarcerated femoral hernia: Would likely present with a palpable mass and more acute, localized symptoms. 3. Pyelonephritis: Usually presents with systemic symptoms like fever, flank pain, and dysuria, and less commonly with significant groin pain and limping. 4. Renal calculi: Although possible, the presence of limping and the specific description of the groin pain suggests a muscular or soft tissue source, such as a psoas abscess.
Given the clinical presentation, a psoas abscess is the most appropriate diagnosis.
A 68-year-old male diabetic patient presents with foot pain. He has long-standing peripheral neuropathy and has been gardening wearing sandals. He reports 3 days of increasing pain and swelling of his calf. His legs are covered in blackened sores.
Which one of the following causes gas gangrene?
1) Clostridium perfringens
2) Staphylococcus aureus
3) Clostridium botulinum
4) Staphylococcus haemolyticus
5) Pseudomonas aeruginosa
Explanation
Clostridium perfringens
Gas gangrene can cause myonecrosis, gas production, and sepsis. It may progress to toxic shock rapidly. Features are large, blackened sores and crepitus caused by gas escaping the necrotic tissue. Clostridia are saprophytes; they live in soil and require a spore to protect them from dehydration. Clostridium perfringens, formerly C. welchii, can cause gas gangrene. There is gas production due to the proteolytic enzymes released by the organism. It also causes food poisoning at about 12–18 h after ingestion. This is caused by exotoxin production.
Staphylococcus aureus
Staphylococcus aureus is the commonest causative pathogen for cellulitis, however the case does not describe a simple cellulitis and another cause should be sought.
Clostridium botulinum
Also a member of the Clostridium genus, C. botulinum produces the exotoxin botulinum,which causes a flaccid paralysis as opposed to gas gangrene.
Staphylococcus haemolyticus
This bacteria is part of normal commensal skin flora, and is often the causative organism in opportunistic infections following a breach in the natural barrier of the skin, such as following cannulation.
Pseudomonas aeruginosa
Pseudomonas is an important pathogen with regard to hospital-acquired infections, multi-drug resistance and infections in immunocompromised patients. Diabetic patients with lower limb ulcers can often become colonised with Pseudomonas causing chronic non-healing ulcers, which are troublesome to treat. It does not however produce gas
gangrene.
A 35-year-old man presents to the Emergency department with a 24-h history of severevthrobbing pain in the finger tip
of his right index finger. He injured his hand while gardening 3 days before and thinks he cut his hand on a rose thorn. The fingertip is erythematous and swollen up to the distal interphalangeal joint (DIP) joint, but not proximal to it.
What is the most likely diagnosis?
1) Apical infection
2) Felon
3) Herpetic whitlow
4) Paronychia
5) Flexor tendon sheath infection
Explanation
Felon
A felon is an abscess in the compartments of the pulp. This is usually more painful than
a paronychia. The swelling does not extend proximal to the distal interphalangeal joint.
The infection often follows a penetrating trauma and is most common in the thumb and
index finger.
Apical infection
An apical infection involves the apical space, ie between the distal part of the nail and
the bone of the distal phalanx, infections of this space may occur when a splinter runs
under the nail. Tenderness is greatest at or just under the free edge of the nail.
Herpetic whitlow
Herpetic whitlow is caused by the herpes simplex virus and small clear vesicles would
be seen.
Paronychia
Paronychia is an infection beside or proximal to the nail, it usually begins as a simple
cellulitis before progressing into a definite abscess which requires incision and
drainage.
Flexor tendon sheath infection
Flexor tendon sheath infection is a surgical emergency with a sausage-shaped digit,
flexed position, tenderness over the flexor tendon sheath and pain on passive extension.
A 10-month-old boy presents with colicky abdominal pain, vomiting and redcurrant stools. On examination there is a
sausage-shaped mass palpable within an otherwise soft abdomen.
What is the most appropriate initial management?
1) Emergency laparotomy
2) Air enema
3) Lower gastrointestinal (GI) endoscopy
4) Rigid sigmoidoscopy
5) Stool softeners
Explanation
Air enema
The clinical picture is of intussusception which occurs in 0.4% of children. It is more common in boys, usually younger than 1 year old. Intussusception occurs when one section of the bowel invaginates into another, the most common form is ileocolic. Blood and mucus or ‘redcurrant jelly’ stool may be passed after the first 24 h as a late manifestation. Sausage-shaped mass may be felt in upper abdomen. Rectal examination is very important as occasionally the apex of intussusception is palpable. The majority of cases are successfully treated conservatively with an air enema.
Emergency laparotomy
Emergency operative management is only indicated in established peritonitis, perforation or failure of enema reduction.
Lower gastrointestinal (GI) endoscopy
Lower GI endoscopy would not be indicated given the clinical scenario, the initial management of choice would be an air enema.
Rigid sigmoidoscopy
Again, rigid sigmoidoscopy is not indicated.
Stool softeners
Stool softeners have no role in the treatment of intussusception.
You are examining an 18-month-old infant who is day 1 post-operation. The infant has a pulse of 150 beats/min, respiratory rate 30 breaths/min, urine output 1.6 ml/kg/h,
haemoglobin 11 g/dl and white-cell count 12 × 10 9 /l.
Which one of these options is abnormal for an infant of this age?
1) Haemoglobin
2) Pulse rate
3) Respiratory rate
4) Urine output
5) White-cell count
Explanation
Pulse rate
The normal ranges for physiological variables and blood test results vary with age in children. The normal value for pulse rate in a infant aged between 1–2 is 100–120 beats per minute, therefore, this infant is tachycardic, which warrants further investigation.
Haemoglobin
The normal range for haemoglobin is 10.5–13.5 g/dl.
Respiratory rate
A normal respiratory rate for an infant aged 1–2 years old is 25–35. This will reduce with age.
Urine output
Normal urine output would be more than 1.5 ml/kg/h.
White-cell count
A normal white-cell count will range from 6–15 × 10 9 /l.
A 1-day-old newborn girl has a distended abdomen and bilious vomiting. Per rectal (PR) examination reveals an empty rectum, and meconium has not been passed.
What is the most likely underlying associated condition that the patient may have?
1) Cerebral palsy
2) Cystic fibrosis
3) Down syndrome
4) Spina bifida
5) Turner’s syndrome
Explanation
Cystic fibrosis
The newborn has meconium ileus. Signs of this include a distended abdomen, bilious
vomiting and an empty rectum. The commonest association is with cystic fibrosis, and
15% of children with cystic fibrosis have meconium ileus.
Cerebral palsy
Cerebral palsy is a broad diagnosis covering a number of neurodevelopmental
conditions affecting movement and coordination. It is not associated with meconium
ileus.
Down syndrome
Down syndrome is a genetic disorder resulting from trisomy of chromosome 21. It is
associated with a number of conditions including congenital heart disease, epilepsy,
hypothyroidism and leukaemia.
Spina bifida
This is a birth defect resulting in failure of closure of the meninges. While there may be
bladder and bowel pathology due to failure of closure of the spinal cord, cystic fibrosis
has the commonest associated with meconium ileus.
Turner’s syndrome
This is a genetic disorder seen in women in which there complete, or partial loss, of the
X chromosome. It is not associated with meconium ileus.
An 8-year-old child presents with vague central abdominal pain and nausea. He is pyrexial (38.7 °C), and has a headache. On examination he has shifting abdominal tenderness and cervical lymphadenopathy.
What is the most likely diagnosis?
1) Acute appendicitis
2) Constipation
3) Crohn’s disease
4) Meckel’s diverticulum
5) Mesenteric adenitis
Explanation
Mesenteric adenitis
Mesenteric adenitis is an inflammation of the mesenteric lymph node accompanied by a
mild peritoneal reaction. It is most common between ages 5–10 years and often
follows/accompanies an URTI.
Acute appendicitis
Acute appendicitis can be distinguished from mesenteric adenitis by lack of cervical
lymphadenopathy, headache, mild abdominal pain, shifting tenderness and pyrexia
greater than 38 °C.
Constipation
Constipation would not cause pyrexia or cervical lymphadenopathy. There are a
number of causes of constipation in children including hypothyroidism, hypercalcaemia,
and Hirschsprung’s disease.
Crohn’s disease
The incidence of Crohn’s disease in children is increasing, presentation is similar to
adults, but children more commonly have extra-gastro-intestinal symptoms, none of
which has been described in the case history.
Meckel’s diverticulum
Meckel’s diverticulum can present with similar symptoms to appendicitis, or with
bleeding, perforation, intussusception, volvulus or obstruction.
A 9-month-old baby boy is brought to the Paediatric Surgical Emergency unit by his parents with a 24-hour history of intermittent episodes of crying, vomiting, and refusal to feed. The parents have noticed that the baby’s stools are mixed with blood. On examination, a ‘sausage-shaped’ mass is palpable over the right side of the abdomen. A per rectal examination reveals an empty rectum, but blood is noticed on the examining glove.
From the options below choose the one which you think is the most likely diagnosis in this patient:
1) Intussusception
2) Infantile hypertrophic pyloric stenosis
3) Duodenal atresia
4) Meconium ileus
5) Hirschsprung’s disease
The most likely diagnosis for this patient is:
Intussusception
Explanation:
1. Intussusception: This is a condition where a part of the intestine telescopes into an adjacent part, causing a blockage. It typically presents with intermittent abdominal pain, vomiting, bloody stools (often described as “currant jelly” stools), and a palpable sausage-shaped mass in the abdomen. The presence of blood on rectal examination supports this diagnosis. 2. Infantile Hypertrophic Pyloric Stenosis: This condition presents with projectile vomiting in infants but does not typically cause bloody stools or a palpable abdominal mass. 3. Duodenal Atresia: This congenital condition causes bilious vomiting in newborns and is usually diagnosed shortly after birth. It does not typically present with bloody stools or a palpable mass. 4. Meconium Ileus: Often associated with cystic fibrosis, this condition presents with bowel obstruction in newborns and meconium impaction but not with the acute symptoms described in this case. 5. Hirschsprung’s Disease: This condition involves a lack of nerve cells in parts of the colon, leading to severe constipation and abdominal distension. It does not typically present with acute episodes of crying, vomiting, or bloody stools.
Given the clinical presentation, intussusception is the most likely diagnosis.
A mother presents her 1-year-old son to the hospital with a history of absence of faeces production for 48 hours. The infant has a distended abdomen with a palpable sausage-shaped mass. His mother thinks he is constipated. What is the most common cause of obstruction in the first 2 years of life?
1) Hirschsprung’s disease
2) Hyperthyroidism
3) Coeliac disease
4) Intussusception
5) Hypercalcaemi
The most common cause of obstruction in the first 2 years of life is:
Intussusception
Explanation:
1. Intussusception: This is the most common cause of intestinal obstruction in young children, particularly between the ages of 6 months and 2 years. It occurs when a part of the intestine telescopes into an adjacent segment, leading to obstruction, reduced blood flow, and potentially severe complications. The classic presentation includes episodic crying, abdominal pain, vomiting, and the passage of “currant jelly” stools. A palpable sausage-shaped mass is a key clinical finding. 2. Hirschsprung’s Disease: While this condition can cause chronic constipation and abdominal distension due to a lack of ganglion cells in the colon, it is not the most common cause of acute intestinal obstruction in this age group. 3. Hyperthyroidism: This condition is rare in infants and does not typically present with acute intestinal obstruction. 4. Coeliac Disease: This autoimmune disorder affects the small intestine due to gluten sensitivity and can cause chronic symptoms such as diarrhea, malabsorption, and failure to thrive, but it is not typically associated with acute intestinal obstruction in infants. 5. Hypercalcaemia: Elevated calcium levels can lead to constipation but are not a common cause of intestinal obstruction in infants.
Given the clinical presentation of a distended abdomen, absence of stool production, and a palpable sausage-shaped mass, intussusception is the most likely diagnosis.
An 80-year-old man is referred to the Emergency Department with abdominal pain and vomiting. His pain
radiates to his back and slightly improves on leaning forwards. Examination reveals a soft abdomen with a very tender epigastrium . His past medical history consists of hypertension for which he takes bendroflumethiazide. Systolic blood pressure is 110 mmHg on both arms. X-rays are unremarkable apart from a prominent central loop.
What is the most likely diagnosis?
1) Aortic dissection
2) Biliary Colic
3) Pancreatitis
4) Renal Colic
5) Ruptured aortic aneurysm
Explanation
Pancreatitis
This patient is most likely to have pancreatitis possibly caused by thiazide diuretic use.
However, the most common cause is alcohol and gallstones in the UK and evidence of this must be sought on history and ultrasound scan. A significantly raised amylase should be apparent on the blood results, >1000 U/l (normal <200 U/l) is virtually diagnostic but beware that peritonitis and perforated duodenal ulcer for example can give similar appearances and a raised. Some units now use serum lipase which can be more sensitive. Pancreatitis can lead to vomiting but the X-ray appearance alludes to a sentinel loop – a paralytic ileus of duodenal or jejunal small bowel caused by local inflammation of the pancreas.
Aortic dissection
Of course in this age group, ruptured abdominal aortic aneurysm (AAA) and aortic dissection must be a consideration and vitally ruled out. Look for evidence of a pulsatile epigastric mass and difference in blood pressure in both arms. Computed tomography (CT) aortogram is suggested if there is any doubt, to rule out these other diagnoses.
Biliary Colic
Biliary colic more commonly presents with intermittent, colicky pain in the right upper quadrant and does not radiate through to the back.
Renal Colic
In renal colic, patients usually cannot get comfortable due to the spasmodic nature of the pain and urine dip may show blood.
Ruptured aortic aneurysm
As above, this is an important diagnosis to exclude, however, the case history is more consistent with acute pancreatitis.
At the request of the nursing staff, you agree to assess a patient who is being treated for a post-operative chest infection. You diagnose septic shock as per recent NICE guidance. On what parameters would you make that diagnosis?
1. Temperature 37°C, pulse rate 110/min, 25 mmHg improvement in systolic blood pressure following fluid bolus 2. Temperature greater than 38.3°C, pulse rate 100/min, blood pressure 134/82 mmHg without intravenous fluids 3. Temperature greater than 38°C and urine output less than 30 ml/h 4. Temperature 37.3°C and pulse rate 80/min 5. Temperature 38.4°C, urine output less than 30 ml/h, no improvement in blood pressure despite fluid challenge
To diagnose septic shock according to the recent NICE guidelines, you should look for the following key parameters:
1. Persistent hypotension requiring vasopressors to maintain mean arterial pressure (MAP) ≥ 65 mmHg despite adequate fluid resuscitation. 2. Serum lactate level > 2 mmol/L despite adequate fluid resuscitation.
Given these criteria, the correct answer is:
5. Temperature 38.4°C, urine output less than 30 ml/h, no improvement in blood pressure despite fluid challenge
Explanation:
• A temperature of 38.4°C indicates a fever, which is often present in septic patients. • Urine output less than 30 ml/h indicates significant hypoperfusion, a common feature in septic shock. • No improvement in blood pressure despite a fluid challenge suggests persistent hypotension, which is critical for diagnosing septic shock.
This scenario matches the definition of septic shock as outlined by NICE guidelines, involving persistent hypotension and evidence of organ dysfunction despite fluid resuscitation.
Sepsis is a potentially life-threatening emergency that requires early recognition and management. Prompt intervention subsequently reduces future morbidity and
mortality. According to National Institute of Clinical Excellence (NICE) Guidance, which of the following is a ‘red flag’ for sepsis?
1) Systolic blood pressure <100 mmHg
2) Heart rate >100 beats/min
3) Respiratory rate >25 breaths/min
4) Lactate of 1.9 mmol
5) White blood-cell count of >12 × 10 9 /l
Explanation
Respiratory rate >25 breaths/min
A raised respiratory rate is one of the first parameters to change in the presence of physiological stress. As such, the National Institute of Clinical Excellence (NICE) guidance identifies a raised respiratory rate as an important red flag for sepsis. Recent National Institute of Clinical Excellence (NICE) Guidance was released in 2016 in response to a UK Parliamentary inquiry examining sepsis. The ‘Time to Act’ report found failures in the recognition, diagnosis, and early management of those who died from sepsis. Efforts are now being made to improve the recognition and diagnosis of
sepsis, and close monitoring and identification of abnormal physiological parameters is an important aspect of this.
Systolic blood pressure <100 mmHg
Systolic blood pressure <90 mmHg is identified as a red flag for sepsis. Consideration of baseline blood pressure is also an important factor and therefore a >40 mmHg fall from
patient’s baseline should also be considered as a red flag in the context of sepsis.
Heart rate >100 beats/min
Tachycardia is an important indicator of physiological stress, however, the National Institute of Clinical Excellence (NICE) states that a heart rate >130 beats/min is a red flag for sepsis.
Lactate of 1.9 mmol
An elevated lactate is an indicator of reduce tissue perfusion as a result of anaerobic respiration. A measured lactate of >2.0 mmol is a ‘red flag’ for sepsis.
White blood-cell count of >12 × 10 9 /l
Although this would form part of a detailed review of a patient with suspected sepsis, the red flags refer specifically to physiological parameters. If abnormal, these should then trigger an in-depth review including investigations such as full blood count, blood cultures, radiological imaging as required.
You are sitting in the endocrine surgery clinic and a patient sees you with moon facies, central obesity and peripheral muscle wasting. You suspect Cushing syndrome. Which one of the following options is true of Cushing syndrome?
1) The commonest cause of Cushing syndrome is iatrogenic
2) Cushing syndrome is due to an excess of aldosterone
3) True Cushing syndrome is due to an adrenal adenoma
4) Removal of a pituitary adenoma carries a high risk of Nelson syndrome
5) Cushing syndrome is seen more in men than in women
The correct option is:
1) The commonest cause of Cushing syndrome is iatrogenic
Explanation:
• Iatrogenic Cushing syndrome is the most common cause of Cushing syndrome, resulting from the prolonged use of corticosteroid medications. These medications are often prescribed for conditions such as asthma, rheumatoid arthritis, and lupus. • Cushing syndrome is due to an excess of aldosterone: This statement is incorrect. Cushing syndrome is caused by an excess of cortisol, not aldosterone. Aldosterone excess is related to conditions such as Conn’s syndrome (primary hyperaldosteronism). • True Cushing syndrome is due to an adrenal adenoma: This statement is partially correct but not comprehensive. True Cushing syndrome can result from various sources, including pituitary adenomas (Cushing’s disease), ectopic ACTH production, or adrenal tumors (adenomas or carcinomas). However, adrenal adenomas are not the most common cause. • Removal of a pituitary adenoma carries a high risk of Nelson syndrome: This statement is incorrect. Nelson syndrome is a potential complication after bilateral adrenalectomy for Cushing’s disease, not after removal of a pituitary adenoma. • Cushing syndrome is seen more in men than in women: This statement is incorrect. Cushing syndrome is more commonly seen in women than in men, particularly in cases of Cushing’s disease (pituitary adenoma).
Thus, the most accurate statement regarding Cushing syndrome is that the commonest cause is iatrogenic, primarily due to the use of exogenous corticosteroids.
A 49-year-old man presented in the orthopaedic outpatient clinic with an X-ray that showed a compressed fracture at T10. He complained of increasing weakness over the past few months. Clinical examination revealed a blood pressure of 165/110 mmHg. Laboratory findings included a serum glucose of 8.6 mmol/l. He was mildly obese and had purplish striae on his thighs and abdomen. Which one of the following pathological lesions is most likely to explain these findings?
1. Adrenal cortical carcinoma 2. Anaplastic thyroid carcinoma 3. Multinodular goiter 4. Parathyroid adenoma 5. Phaeochromocytoma
The patient’s presentation is consistent with Cushing’s syndrome, which is characterized by a combination of features including central obesity, hypertension, glucose intolerance (as indicated by elevated serum glucose), and purplish striae. The clinical scenario provided points towards an underlying pathological lesion that can cause Cushing’s syndrome.
Among the options listed, the most likely cause is:
1. Adrenal cortical carcinoma
Explanation:
Adrenal cortical carcinoma can cause Cushing’s syndrome by producing excess cortisol. The symptoms described, such as obesity, hypertension, purplish striae, and glucose intolerance, align with Cushing’s syndrome. Other options, such as anaplastic thyroid carcinoma, multinodular goiter, parathyroid adenoma, and phaeochromocytoma, do not typically present with this specific combination of symptoms.
Thus, the correct answer is:
1. Adrenal cortical carcinoma
Adrenal cortical carcinoma is a rare cancer carrying a poor prognosis, with most cancers metastatic at diagnosis. The female to male ratio is 2–3:1, and there is a bimodal distribution, with peak incidence in the first decade of life and a second peak in the 4th–5th decade. Metastasis is most commonly to periadrenal tissue, regional lymph nodes, bone, liver, and lungs.
These are functioning tumors producing excess amounts of cortisol that are not under the control of pituitary adrenocorticotropic hormone (ACTH). Patients present with red flag symptoms such as fever, weight loss, back pain, and abdominal fullness, but also with symptoms relating to Cushing’s syndrome, as demonstrated in this scenario, and virilization syndromes.
Treatment is with adrenalectomy if the tumor is confined to the adrenal gland, or chemotherapy (mostly mitotane) and/or radiotherapy if there is extensive disease. If the tumor is confined to the adrenal gland and is treated, 5-year survival is 65%. This drops to 44% for tumors with local invasion to surrounding tissues and 7% for tumors with distant metastases at diagnosis.
Cushing’s syndrome involves chronic glucocorticoid excess, of which 90% are adrenocorticotropic hormone (ACTH)-dependent and 10% ACTH-independent. Causes of Cushing’s syndrome include Cushing’s disease and iatrogenic causes such as steroids. Signs of Cushing’s syndrome include:
Head and neck:
• Frontal balding • Moon face • Acne • Gynaecomastia in males • Plethoric complexion
Trunk:
• Kyphosis • Buffalo hump • Central obesity (due to altered fat distribution) • Purple striae on abdomen, breasts, and thighs • Thin, fragile skin • Predisposition to infection
Limbs:
• Proximal myopathy • Pathological fractures • Ankle edema (salt and water retention due to excess cortisol) • Hirsutism • Hair growth on the forearms in particular
Anaplastic carcinoma of the thyroid is a rare entity, accounting for 1–2% of all thyroid cancers, a very aggressive cancer with poor prognosis. It is not known to cause symptoms relating to Cushing’s syndrome.
A multinodular goiter is associated with hyperthyroidism but not Cushing’s syndrome. A parathyroid adenoma causes excessive secretion of parathyroid hormone (PTH) and results in symptoms relating to hypercalcemia and hypophosphatemia but not Cushing’s syndrome.
Phaeochromocytoma is a rare tumor arising most commonly in the chromaffin cells of the adrenal medulla and resulting in increased secretion of catecholamines. Patients tend to present with severe hypertension (often resistant to multiple therapeutic agents), excessive sweating, anxiety/panic attacks, headaches, and pallor.
You were asked to do the endocrine surgery in the outpatient clinic by your consultant. On that particular day you only saw patients with endocrine malignancies. Based on your knowledge about the natural history of various endocrine malignancies, which one of the following neoplasms has the best prognosis?
1) Adrenal cortical carcinoma
2) Anaplastic carcinoma of the thyroid
3) Follicular carcinoma of the thyroid
4) Papillary carcinoma of the thyroid
5) Parathyroid carcinoma
Explanation
Papillary carcinoma of the thyroid
Papillary carcinoma of the thyroid is the most common form of thyroid cancer accounting for 80% of cases, and of the examples in these answers, is the most common.
It is a well differentiated tumour, most commonly presenting in wom en (female to male ratio of 3:1), aged 35–40. It is slow growing and arises from T4 and thyroglobulin- producing follicular cells. It can metastasise via lymphatics and can be multifocal at diagnosis. It can metastasise to the lungs. It most commonly presents as a solitary thyroid nodule.
Histologically it shows stalks of epithelial cells (papillae) and in 50% of cases psammomabodies, representing calcific collections, are present. Additionally, cells show a characteristic pattern of nuclear inclusions called ‘orphan Annie eye’.
Radiologically it appears as a single mass with irregular borders, found in the subscapular region. Treatment is with thyroidectomy and radioactive iodine administration for positive lymph nodes.
If the tumour is confined to the thyroid gland on diagnosis, it has an excellent prognosis and a 5 year survival rate of 95%.
Adrenal cortical carcinoma
Adrenal cortical carcinoma is a rare cancer and prognosis depends on the extent of tumour spread at diagnosis. If the tumour is confined to the adrenal gland and is treated 5-year survival is 65%. This number drops to 44% for tumours with local invasion to surrounding tissues and 7% for tumour with distant metastases at diagnosis.
Anaplastic carcinoma of the thyroid
Anaplastic carcinoma of the thyroid is a rare entity, accounting for 1–2% of all thyroid cancers. It is however the most aggressive tumour and carries the worst prognosis. It
most commonly affects women and patients tend to present at an older age than with other thyroid cancers, ie in the 6th–7th decade of life. Patients present with a rapidly enlarging, firm, fixed thyroid mass and symptoms of local invasion such as hoarse voice and dyspnoea or stridor. Median patient survival is 8.1 months, as anaplastic carcinoma is poorly responsive to treatment.
Follicular carcinoma of the thyroid
Follicular carcinoma of the thyroid is the second most common malignancy of the thyroid accounting for about 10% of all thyroid cancers. It is a well differentiated cancer, typically presenting in women aged 40–60. These tumours arise from the follicular cells of the thyroid. 5-year survival rate is over 80% for non-invasive disease.
Parathyroid carcinoma
Parathyroid carcinoma is a very rare malignancy, occurring equally in men and women, with men having a worse prognosis. It usually occurs after the age of 30. Disease confined to the parathyroid glands at diagnosis has a 5 -year survival rate of 88.5%.
A 25-year-old woman was seen in the Emergency Department complaining of vague abdominal pain and some pain in her extremities. On further enquiry she revealed that she had been feeling depressed for several months. Physical examination revealed no major findings. A chest X-ray was normal. Serum biochemistry revealed the following:
Calcium: 3.47 mmol/l (2.20–2.60 mmol/l)
Serum albumin: 38 g/l (35–55 g/l)
Phosphate: 0.64 mmol/l (0.70–1.40 mmol/l)
What is the most likely diagnosis in this patient?
1) Chronic renal failure
2) Metastatic carcinoma
3) Parathyroid adenoma
4) Pituitary adenoma
5) Thyroid carcinoma
Explanation
Parathyroid adenoma
The patient has hypercalcaemia and hypophosphataemia, pointing towards the diagnosis of primary parathyroid disease. This patient most likely has primary hyperparathyroidism, the most common cause of which is a single parathyroid adenoma (85%). Other causes include: parathyroid gland hyperplasia affecting all four parathyroid glands (10–15%), multiple parathyroid adenomas (3–5%) or a
parathyroid carcinoma (1%).
The adenoma produces an excess of parathyroid hormone leading to: excessive calcium resorption from bone, increased renal calcium reabsorption, increased renal phosphate excretion and increased production of 1,25-dihydroxyvitamin D3 .
Therefore, blood tests reveal: a raised PTH, hypercalcaemia, hypophosphataemia and a raised vitamin D3 .
Treatment of a parathyroid adenoma is by surgical removal of the parathyroid glands.
For patients not fit/suitable for parathyroidectomy options for treatment include:
-preventing vitamin D deficiency, bisphosphonates to protect the bones, calcimimetics to control hypercalcaemia if required.
Chronic renal failure
Chronic kidney failure leads to secondary hyperparathyroidism. The diseased kidney fails to convert an adequate level of vitamin D to its active form, which reduces the amount of calcium absorbed from the intestines, and fails to excrete phosphate leading to formation of calcium phosphate compounds in the circulation and a reduction in free circulating calcium. Therefore, in secondary hyperparathyroidism there is a low serum concentration of calcium and vitamin D, a high serum concentration of parathyroid hormone and a high serum phosphate.
Metastatic carcinoma
The two most common causes of hypercalcaemia are primary hy perparathyroidism and hypercalcaemia of malignancy. The two are generally quite difficult to differentiate. In both calcium is raised (with hypercalcaemia of malignancy causing a more dramatic hypercalcaemia) and phosphate is low. parathyroid hormone (PTH) in primary
hyperparathyroidism is raised whereas in hypercalcaemia of malignancy it can be low/normal/raised depending on the mechanism involved.
Given the patient’s age and the fact that the symptoms have been ongoing for several months make parathyroid adenoma a far more likely diagnosis than metastatic carcinoma. The most common cancers associated with hypercalcaemia are: breast, lung and myeloma.
Pituitary adenoma
A pituitary adenoma causes visual symptoms (bitemporal hemianopia), headaches and symptoms relating to the cellular origin of the adenoma. It does not commonly cause
hypercalcaemia.
Thyroid carcinoma
Thyroid carcinoma can lead to hypercalcaemia, but this is usually seen with advanced disease.
A 48-year-old man smoker presented to his GP complaining of a chronic dry cough and backache. The GP noticed truncal obesity, muscular weakness, hypertension, purplish abdominal striae and tenderness in the region of the lower thoracic spine. The patient was taking no medications.
Which one of the following diseases is most likely to be the cause for these findings?
1) 21-Hydroxylase enzyme deficiency
2) Extra-adrenal paraganglioma
3) Multiple endocrine neoplasia, type I
4) Small-cell anaplastic (oat cell) carcinoma
5) Tuberculosis
Explanation
Small-cell anaplastic (oat cell) carcinoma
The presenting signs in this scenario point to Cushing’s syndrome, secondary to excess circulating cortisol. Given that the patient is a smoker presenting with a dry cough and
lower back pain, it is most likely that the patient has small-cell lung cancer (also known as oat cell lung cancer) that has metastasised to the bones, and also secretes ectopic ACTH causing Cushing’s syndrome, as part of a paraneoplastic syndrome. This is very common with bronchogenic carcinoma.
Small-cell lung cancer is strongly associated with smoking and is a neuroendocrine tumour arising in the bronchial mucosa. It is fast growing, highly metastatic, rarely operable at diagnosis and associated with a 5-year survival of less than 20%.
CUSHing’s is where Cortisol is GUSHing.
Cushing’s syndrome: chronic glucocorticoid excess of which 90% are ACTH-dependent and 10% ACTH-independent. Causes of Cushing’s syndrome include Cushing’s disease
and iatrogenic causes such as steroids.
Signs of Cushing’s:
Head and neck
Trunk
Limbs
Frontal balding
Kyphosis
Proximal myopathy
Moon face
Buffalo hump
Pathological fractures
Acne
Gynaecomastia in males
Ankle oedema (salt and water rententiondue to excess cortisol) Plethoric complexion
Central obesity (due to altered fat distribution)
Hirsutism
Purple striae on abdomen, breasts and thigs
Thin, fragile skin
Hair growth of the foreaems in particular
Predisposition to infection
21-Hydroxylase enzyme deficiency
21-Hydroxylase enzyme deficiency, also known as congenital adrenal hyperplasia, is a condition commonly presenting at birth or early childhood with ambiguous genitalia.
Extra-adrenal paraganglioma
An extra-adrenal paraganglioma, also known as extra-adrenal phaeochromocytoma, are tumours producing catecholamines, such as adrenaline. The signs the patient presents with in this scenario relate to extra circulating cortisol and not catecholamines.
Multiple endocrine neoplasia, type I
Multiple endocrine neoplasia type I is a combination of parathyroid neoplasia, a pancreatic islet tumour and pituitary tumours. Presentation is most commonly with
symptoms associated with hypercalcaemia.
Other symptoms include: diarrhoea and abdominal pain in gastrinoma, hypoglycaemia
in insulinomas. Depending on the place of the pituitary tumour it can present with
Cushing’s disease due to over secretion of ACTH stimulating cortisol secretion.
MEN 1 is however an autosomal-dominant familial disorder, therefore there must be
some family history, presents usually in teenagers or patients in the 4th decade, but
does not tend to present with a cough.
MEN type I = Primary = pathology beginning with P
Parathyroid neoplasia
Pancreatic islet cell tumour (Zollinger–Ellison syndrome (50%), insulinoma (20%))
Pituitary tumours
Tuberculosis Tuberculosis can present with a cough and back pain but it is not known to cause a
Cushing’s syndrome.
A 37-year-old woman presents to your Endocrine Clinic with symptoms of anxiety, panic attacks and frequent headaches. Subsequent investigations have revealed her to have a
phaeochromocytoma. Regarding this condition, what is the
first-line diagnostic test?
1) Clonidine suppression test
2) Metaiodobenzylguanidine (MIBG) scintigraphy
3) Magnetic resonance imaging (MRI) scan
4) Plasma noradrenaline
5) 24-hour urinary vanillylmandelic acid (VMA)/homovanillic (HVA) secretion
Explanation
24-hour urinary vanillylmandelic acid (VMA)/homovanillic (HVA) secretion
Phaeochromocytomas are rare neuroendocrine tumours arising in the chromaffin cells
most commonly found in the adrenal medulla. They are associated with increased
catecholamine production and tend to present with severe hypertension (often
resistant to multiple therapeutic agents), excessive sweating, anxiety/panic
attacks, headaches andpallor. They are usually curable if detected and treated early. If,
however, they remain undiagnosed, they can lead to severe complications including
death.
First-line investigation for phaeochromocytoma includes a 24 h urine collection and
quantification of urinary vanillylmandelic and homovanillic acids. These are byproducts
of catecholamine production and are present at a low concentration in the urine of
unaffected individuals. In phaeochromocytoma, however, the concentration of these
molecules in the urine is significantly raised, indicating an increased production of
catecholamines.
Phaeochromocytomas are associated with conditions such as: multiple endocrine
neoplasias 2A and 2B, von Hippel–Lindau syndrome, neurofibromatosis.
Clonidine suppression test
Clonidine suppression test forms the second line test in the diagnosis of
phaeochromocytoma in cases where there is a high clinical suspicion but there was
borderline elevation in the catecholamines, not enough to qualify for a definitive
diagnosis.
The test relies on the fact that clonidine is a suppressor of physiological catecholamine
secretion, but has no effect over autonomous catecholamine
secretion, as in phaeochromocytoma. There should be collection of urine from 21:00 on
day 1 to 7:00 on day 2 occurs and catecholamines quantified. Then, on day 2, clonidine
is administered and collection of urine follows from 21:00 to 7:00 of day 3.
Catecholamines are quantified. In a normal individual the urine catecholamines from
the second collection, ie following clonidine administration, should be greatly reduced
when compared with the original collection. In patients with a
phaeochromocytoma, however, clonidine is unable to suppress catecholamine release,
and these remain in high concentrations in the urine. Metaiodobenzylguanidine (MIBG) scintigraphy
MIBG scintigraphy is a very specific test for detecting phaeochromocytoma.
Radioactive iodine-131-metaiodobenzylguanidine is administered to the patient. In a
normal patient, the adrenal medulla appears to uniformly uptake the radioactive
solution and its intensity is less than that of the liver uptake. For a patient with a
phaeochromocytoma there is a focal area in the ad renal medulla where there is very
strong and prolonged uptake of the solution. MIBG scintigraphy is not considered the
first-line investigation to detect phaeochromocytoma. MIBG is useful in detecting extra -
adrenal tumours or disease recurrence.
Magnetic resonance imaging (MRI) scan
Computed tomography (CT) and MRI scans are both sensitive in detecting
phaeochromocytomas, with MRI being superior to CT scans, but are not considered the
first-line investigation. These imaging modalities are used to localise t he tumour once
elevated urine catecholamines are detected.
Plasma noradrenaline
Elevated plasma noradrenaline is a sensitive test in detecting phaeochromocytoma. This
test has a high sensitivity of 96%, but a relatively low specificity of 85%, as plasma
noradrenaline can be elevated in other cases such as in patients
taking amitriptyline and some antipsychotic medication.
A 19-year-old woman presents to her GP with anxiety, sweating, heat intolerance,
weight loss and a tremor. Blood tests reveal Graves’ disease.
Which one of the following options concerning Graves’ disease is correct?
1) Carbimazole is drug of choice for treatment throughout pregnancy
2) Has equal sex incidence
3) In children has a high relapse rate when treated with antithyroid drugs
4) Is due to immunoglobulin M (IgM) thyroid-stimulating antibodies
5) Is a form of toxic multinodular goitre
Explanation
In children has a high relapse rate when treated with antithyroid drugs
About half of the children with Graves’ disease treated with antithyroid drug therapy
have a complete remission, but up to 1 in 3 children suffer a relapse. Features that
increase the risk of relapse include a large goitre, a high radioactive iodine uptake and a
high thyroid-stimulating immunoglobulin titre.
Graves’ disease: also known as Graves’ ophthalmopathy, thyroid-associated
ophthalmopathy, dysthyroid eye disease and infiltrative eye disease. In Graves’
disease the thyroid-stimulating auto-immunoglobulins are of the IgG class.
Carbimazole is drug of choice for treatment throughout pregnancy
Hyperthyroidism in pregnancy affects about 0.2% of the pregnant population (0.1 –
0.4%). The vast majority of these cases is caused by Grave’s disease. Carbimazole is the
medication used as first line in the treatment of Graves’ disease. It has however, been
reported to cause severe congenital defects if used during pregnancy. Therefore,
propylthiouracil is the medication of choice to treat Graves’ disease during the first
trimester of pregnancy. Following the first trimester, carbimazole use can resume for
the remainder of the pregnancy, as propylthiouracil is associated with hepatotoxicity.
Propylthiouracil inhibits the production of thyroid hormones by inhibiting the enzyme
thyroperoxidase.
Has equal sex incidence
Hyperthyroidism caused by Graves’ disease is more common in women, with an female
to male ratio of 7–8:1.
Is due to immunoglobulin M (IgM) thyroid-stimulating antibodies
Graves’ disease is the most common cause of hyperthyroidism and is an autoimmune
condition mediated by IgG thyroid-stimulating antibodies, which bind the thyroid-
stimulating hormone receptor, mimic the function of thyroid-stimulating hormone
leading to thyroid hyperplasia and an overproduction of thyroid hormones.
Is a form of toxic multinodular goitre In Graves’ disease the antibodies stimulating the thyroid -stimulating hormone receptor
lead to hyperplasia of the thyroid follicular cells of the thyroid gland leading to a toxic
diffuse goitre.
A 63-year-old woman is seen with an enlarging mass in her thyroid and difficulty
swallowing. She is later diagnosed with follicular carcinoma of the thyroid.
Which one of the statements is correct regarding follicular carcinoma of the
thyroid?
1) It can be diagnosed by fine-needle aspiration cytology (FNAC)
2) It can be monitored for recurrence by calcitonin levels
3) The most common presentation is a hoarse voice
4) It has a higher incidence in iodine-deficient regions of the world
5) It is most common at puberty
Explanation
It has a higher incidence in iodine-deficient regions of the world
Follicular carcinoma occurs more commonly in the Caucasian population, with an
incidence higher in areas of work where there is a higher incidenc e of iodine deficiency.
It can be diagnosed by fine-needle aspiration cytology (FNAC)
FNAC cannot differentiate between follicular carcinoma and follicular adenoma.
Therefore, histological diagnosis follows a thyroid lobectomy. If follicular carcinoma is
confirmed, a completion thyroidectomy should be performed.
It can be monitored for recurrence by calcitonin levels
In follicular carcinoma, up to 30% of patients may suffer a recurrence, which can occur
decades after the primary disease. Thyroglobulin seru m levels are tested every 6–12
months to detect recurrence. A rise in thyroglobulin levels indicates recurrence.
Calcitonin levels are used to monitor recurrence of medullary carcinoma.
The most common presentation is a hoarse voice
The most common presentation of a follicular carcinoma is a single, unilateral nodule or
a thyroid mass felt on palpation of the neck. Other patients present with symptoms of
difficulty swallowing, a hoarse voice or dyspnoea.
It is most common at puberty
Follicular carcinoma is common in all age groups, with cases reported from age 15 to
- It is, however, more common in adults aged 40 –60.
A 25-year-old woman presents to her general practitioner with a history of episodes of severe headaches and palpitations. On examination, it is noted she has a blood pressure of 180/100 mmHg, and further questioning reveals a family history of multiple endocrine neoplasia (MEN) 2a.
Which of the following conditions is found in MEN 2a?
1) Pancreatic tumour
2) Parathyroid hyperplasia
3) Pituitary tumour
4) Renal cell carcinoma
5) Small cell lung carcinoma
Explanation
Parathyroid hyperplasia
MEN is a group of conditions affecting the endocrine organs and presenting with tumours in multiple endocrine organs simultaneously. MEN 2 affects 1 in 35 000 people and, of the two subtypes, the most common is MEN 2a. It is inherited in an autosomal dominant pattern, and therefore, patients have family members who are affected.
MEN 2a is the association of medullary thyroid carcinoma, phaeochromocytoma and parathyroid hyperplasia. MEN 2b is the association of medullary thyroid carcinoma, mucosal neuroma and phaeochromocytoma.
Pancreatic tumour
Pancreatic islet tumours are seen in patients with MEN type 1. Pituitary tumour
A pituitary tumour forms part of MEN syndrome type 1.
Renal cell carcinoma
Renal cell carcinomas are not part of the tumours seen in patients with MEN syndromes.
MEN syndromes are characterised by the formation of tumours in multiple endocrine organs. Though non-endocrine tumours can coexist, they are not part of the description of the syndrome.
Small cell lung carcinoma
Small cell lung carcinoma is not part of the tumours seen in patients with MEN syndromes. MEN syndromes are characterised by the formation of tumours in multiple endocrine organs. Though tumours in non-endocrine organs can coexist, they are not part of the syndrome.
A 54-year-old woman presents with severe epigastric pain and vomiting. Blood tests show an elevated amylase level, and prior blood tests showed she has had hypercalcaemia which is thought may have precipitated acute pancreatitis.
Investigation of the hypercalcaemia shows she has primary hyperparathyroidism caused by a parathyroid adenoma.
What proportion of cases of primary hyperparathyroidism are caused by adenomas?
1) 1%
2) 10%
3) 30%
4) 5%
5) 85%
Explanation
85%
A single parathyroid adenoma is the cause of primary hyperparathyroidism in about 85% of cases.
Less common causes of primary hyperparathyroidism include: parathyroid gland hyperplasia involving all four glands or parathyroid carcinoma.
Excess parathyroid hormone causes hypercalcaemia.
1%
A parathyroid carcinoma can be the cause of primary hyperparathyroidism in about 1% of cases.
10%
In 10–15% of cases of primary hyperparathyroidism, the abnormalities are caused by parathyroid hyperplasia, involving all four parathyroid glands.
30%
Only 30% of patients with primary hyperparathyroidism are symptomatic. The remaining majority are usually asymptomatic, and the abnormalities associated with hyperparathyroidism are detected incidentally.
5%
Three to 5% of cases of primary hyperparathyroidism are due to a double adenoma.
A farmer presents with his wife as he has noted a lump in his lower neck which has increased in size substantially over the last few months. On further questioning, he admits to some difficulty swallowing and his wife thinks his voice has become hoarser. Investigations reveal anaplastic thyroid carcinoma.
In which of the following age groups is this most common?
1) 10-20
2) 30-40
3) 40-50
4) 60-70
5) 80-90
Explanation
60-70
Anaplastic thyroid carcinoma is rare (1–2% of thyroid malignancies) and the most aggressive form of primary thyroid cancer. Peak incidence is seen in adults aged 60–70
and carries a very bad prognosis, with only about 5% survival at 5 years. It is more common in women and is associated with iodine deficiency and a history of a multinodular goitre.
It can metastasise to the bone, brain and lung. Patients present with a rapidly growing neck mass, usually with symptoms of compression such as dyspnoea and dysphagia.
Treatment is usually palliative with doxorubicin and cisplatin but does not affect survival.
10-20
Thyroid cancer is rare in children. The rate of presentation peaks around the ages of
35–39. However, a significant amount of cases have been reported in the literature where a diagnosis of thyroid cancer is made in early adulthood.
30-40
Papillary thyroid carcinoma, which is the most common form of thyroid carcinoma, accounting for about 70% of cases, presents usually in patients aged 35 –40, with a female gender predilection.
40-50
Thyroid lymphomas most commonly affect women aged over 50 and there is an association of these cancers with Hashimoto’s thyroiditis.
80-90
Hurtle cell carcinoma, a rare form of thyroid carcinoma, has been reported in patients from the age of 20–85 years.
A 37-year-old woman presents with a 2-month history of progressively worsening hypothyroid symptoms. On examination, there is diffuse enlargement of her thyroid
gland, and after testing, she is diagnosed with Hashimoto’s thyroiditis.
In addition to anti-thyroglobulin antibodies, which other autoantibodies would be commonly found in Hashimoto’s thyroiditis?
1) Anti-centromere antibodies
2) Anti-microsomal antibodies
3) Anti-mitochondrial antibodiesc
4) ANCA (cytoplasmic Anti-Neutophil Cystoplasmic antibody)
5) pANCA (perinuclear ANCA)
Explanation
Anti-microsomal antibodies
Hashimoto’s thyroiditis is an autoimmune thyroid condition where the presence of
autoantibodies leads to cell destruction in the thyroid, reduced hormone output
and hypothyroidism.
It is the most common cause of hypothyroidism in areas where there is adequate iodine
intake. It is ten times more common in women and tends to present at ages of 30 –50;
however, it can present at any point during a patient’s lifetime.
The most common form of autoantibodies detected in Hashimoto’s thyroiditis is
antibodies against thyroid peroxidase. This is a microsomal component, an essential
enzyme involved in the production of thyroid hormones, and therefore these antibodies
are also referred to as anti-microsomal antibodies. Another group of commonly
detected autoantibodies is anti-thyroglobulin antibodies.
Hashimoto’s thyroiditis is associated with other autoimmune conditions such as
pernicious anaemia and coeliac disease. It also increases the risk for developing
lymphoma of the thyroid at a later stage in life.
Patients tend to present with symptoms (see mnemonic) of insidious onset, gradually
worsening, and with the presence of a diffuse goitre.
Treatment is usually with thyroid hormo ne supplementation. Surgery is not usually
required, unless there are compression symptoms from the goitre. Administration of
steroids can assist in shrinking of the goitre.
Anti-centromere antibodies
Anti-centromere antibodies are found in limited sclerod erma disease (CREST
syndrome).
Anti-mitochondrial antibodies
Anti-mitochondrial antibodies are associated with primary biliary sclerosis, an
autoimmune condition causing liver scarring.
cANCA (cytoplasmic Anti-Neutophil Cystoplasmic antibody)
c-ANCA is an autoantibody directed against proteinase 3 and is found in Wegener’s
granulomatosis. pANCA (perinuclear ANCA)
p-ANCA is an autoantibody directed against myeloperoxidase and is associated with
Churg–Strauss syndrome.
A 33-year-old woman has been listed for a laparoscopic adrenalectomy after being diagnosed with phaeochromocytoma due to episodic palpitations, hyperhidrosis and hypertension. During preoperative investigations, it is discovered she has multiple endocrine neoplasia 2a, and further investigation shows a medullary thyroid carcinoma.
What proportion of medullary thyroid carcinoma is familial ?
1) 1%
2) 5%
3) 20%
4) 50%
5) 75%
Explanation
20%
Medullary thyroid carcinoma is a malignancy of parafollicular C cells, of neural ectodermal origin, accounting for about 5% of total primary thyroid malignancies.
Medullary thyroid carcinoma is sporadic in 80% of the cases and familial in 20%. The familial form usually presents as part of the multiple endocrine neoplasia 2 syndrome (both a and b). The tumour is usually found in the upper two -thirds of the thyroid gland and can produce a variety of hormones such as calcitonin, prostaglandins and adrenocorticotrophic hormone (ACTH). Treatment is with thyroidectomy, with lymph node clearance as required.
The patient in this scenario has medullary carcinoma of the thyroid, presenting as part of multiple endocrine neoplasia type 2a.
1%
Medullary thyroid carcinoma can present with a thyroid nodule and local lymphadenopathy. It is important to note that metastatic medullary thyroid carcinoma presents with diarrhoea and flushes.
5%
Medullary thyroid carcinoma, a malignancy arising from parafollicular C cells , accounts for 5–8% of total primary thyroid malignancy cases.
50%
If a patient presents with metastatic medullary thyroid carcinoma that has spread to other parts of the body, the 10-year survival rate is < 50%, more specifically 20–40%.
75%
Up to 75% of medullary thyroid carcinoma are sporadic and therefore occur in patients with no family history.
A 54-year-old woman has undergone some blood tests, as part of an employment health screen. She reports she is in good health and, being very health-conscious, takes regular
vitamin and mineral supplements. She is taking bendroflumethiazide 2.5 mg for hypertension, and her blood pressure is 132/82 mmHg. The only abnormality is a serum calcium concentration of 2.94 mmol/l (normal 2.20–2.60 mmol/l).
Which of the following is the most likely cause?
1) Diuretic treatment
2) High dietary calcium intake
3) High dietary vitamin D intake
4) Occult malignancy
5) Primary hyperparathyroidism
Explanation
Primary hyperparathyroidism
Given the fact that the patient is asymptomatic, the most likely diagnosis is hypercalcaemia secondary to primary hyperparathyroidism. Primary hyperparathyroidism is, in the majority of cases (85%), the result of a single parathyroid adenoma. Other causes include: parathyroid gland hyperplasia affecting all four parathyroid glands (10–15%), multiple parathyroid adenomas (3–5%) or a parathyroid carcinoma (1%).
The adenoma produces an excess of parathyroid hormone, leading to: excessive calcium resorption from bone, increased renal calcium reabsorption, increased renal phosphate excretion and increased production of 1,25-dihydroxyvitamin D3 .
Therefore, blood tests reveal: a raised parathyroid hormone level, hypercalcaemia, hypophosphataemia and a raised vitamin D 3 level.
Treatment of a parathyroid adenoma is by surgical removal of the parathyroid glands.
For patients not fit/suitable for parathyroidectomy, options for treatment include: preventing vitamin D deficiency, bisphosphonates to protect the bones and calcimimetics to control hypercalcaemia if required.
Diuretic treatment
Thiazide diuretics, such as bendroflumethiazide mentioned in the above scenario, block the thiazide-sensitive Na + /Cl – cotransporter found on the apical membrane of the distal
convoluted tubules and therefore inhibit sodium and chloride reabsorption.
Common side-effects of thiazide diuretics include: hyponatraemia, hypokalaemia, hypercalcaemia, hyperglycaemia, hyperuricaemia, gout, postural hypotension and hypochloraemic alkalosis.
Hypercalcaemia caused by thiazide diuretics, however, is usually mild, and therefore unlikely to be the cause of the raised calcium concentration in this scenario.
High dietary calcium intake
Calcium absorption in the bowel is tightly regulated, and an increased dietary intake of calcium is not thought to be a cause of hypercalcaemia. High dietary vitamin D intake
Hypervitaminosis D, ie excess vitamin D intake, can lead to hypercalcaemia. It is usually the result of taking a large amount of vitamin D supplements. In this case , the patient is
only taking bendroflumethiazide and standard vitamin supplementation.
Occult malignancy
The two most common causes of hypercalcaemia are primary hyperparathyroidism and malignancy. Given the patient in this scenario is asymptomatic, it is more likely that the hypercalcaemia is the result of primary hyperparathyroidism.
A 42-year-old man with a past history of parathyroid adenoma presents with a mass in the neck and enlarged cervical lymph nodes. Fine-needle aspiration cytology confirms a diagnosis of medullary carcinoma of the thyroid.
Which one of the following forms of familial endocrine disease is this most likely to represent?
1) Cowden syndrome
2) Familial medullary carcinoma of the thyroid
3) Multiple endocrine neoplasia type 1
4) Multiple endocrine neoplasia type 2a
5) Multiple endocrine neoplasia type 2b
Explanation
Multiple endocrine neoplasia type 2a
This patient with a history of parathyroid adenoma presents with a medullary carcinoma of the thyroid. This represents an example of multiple endocrine neoplasia type 2a. This is described as the combination of: medullary thyroid carcinoma, phaeochromocytoma and hyperparathyroidism.
Cowden syndrome
Cowden syndrome is a very rare autosomal dominant condition, also referred to as multiple hamartoma syndrome, associated with the presence of multiple non -cancerous
growths called hamartomas. It increases the susceptibility of individuals affected to develop some forms of cancers such as thyroid, endometrial, colorectal and renal cancers and melanoma.
Familial medullary carcinoma of the thyroid
Familial medullary carcinoma of the thyroid is a subtype of multiple endocrine neoplasia type 2 where patients dev elop medullary carcinoma of the thyroid but have a lower probability of having a parathyroid adenoma or a phaeochromocytoma. This accounts for 10–20% of all multiple endocrine neoplasia type 2 cases.
Multiple endocrine neoplasia type 1
Multiple endocrine neoplasia type I describes the combination of parathyroid hyperplasia, pancreatic islet cell tumours and pituitary tumours.
Multiple endocrine neoplasia type 2b
Multiple endocrine neoplasia type 2b describes the combination of a medullary thyroid carcinoma, mucosal neuromas, phaeochromocytoma and marfanoid body habitus.
A woman patient presents with a moderately enlarged thyroid enlargement is symmetrical with a rubbery texture. She is euthyroid middle-aged woman gland. The Anti-thyroglobulin and anti-thyroid peroxidase/anti-microsomal antibodies are raised.
Which one of the following is the most likely diagnosis?
1) Acute suppurative thyroiditis
2) Autoimmune thyroiditis (Hashimoto’s thyroiditis)
3) Graves’ disease
4) Riedel’s thyroiditis
5) Subacute thyroiditis (De Quervain’s thyroiditis)
Explanation
Autoimmune thyroiditis (Hashimoto’s thyroiditis)
Hashimoto’s thyroiditis is an autoimmune thyroid condition where the presence of autoantibodies leads to cell destruction in the thyroid, reduced hormone output and hypothyroidism. It is the most common cause of hypothyroidism in areas where there is adequate iodine intake. It is ten times more common in women and tends to
present at ages 30–50; however, it can present at any point during a patient’s lifetime.
The most common form of autoantibodies detected in Hashimoto’s thyroiditis is antibodies against thyroid peroxidase. This is a microsomal component, an essential
enzyme involved in the production of thyroid hormones, and therefore these antibodies are also referred to as anti-microsomal antibodies. Another group of commonly detected autoantibodies is anti-thyroglobulin antibodies. Patients tend to present with symptoms of insidious onset, gradually worsening, and with the presence of a goitre. It
is a lymphocytic infiltration of the gland, with subsequent fibrosis, leading to diffuse enlargement. The goitre associated with Hashimoto’s thyroiditis is symmetrical,
rubbery in texture and painless. Hashimoto’s thyroiditis is associated with other autoimmune conditions such as pernicious anaemia and coeliac disease. It also increases the risk for developing lymphoma of the thyroid at a later stage in life.
Treatment is usually with thyroid hormone supplementation. Surgery is not usually required, unless there are compression symptoms from the goitre. Administration of steroids can assist in shrinking of the goitre.
Acute suppurative thyroiditis
Acute suppurative thyroiditis is a rare condition caused by bacterial infection of the thyroid gland that tends to affect patients with anatomical defects or concurrent thyroid
disease.
Graves’ disease
Graves’ disease presents with a toxic, diffuse goitre and is associated with thyroid - stimulating antibodies that bind to the thyroid -stimulating hormone receptors.
Riedel’s thyroiditis Riedel’s thyroiditis is a rare condition. It describes the chronic fibrosis of the thyroid gland, resulting in a rapidly growing, painful, hard and fixed thyroid mass. Fibrosis can extend outside the thyroid gland to surrounding structures.
Subacute thyroiditis (De Quervain’s thyroiditis)
De Quervain’s thyroiditis (or subacute thyroiditis) is usually viral in origin, and patients report a preceding viral illness such as mumps, measles, influenza, etc.
A 56-year-old woman presents with abdominal pain and constipation. She has no past medical history. Her blood tests show the following.
Result
Hb 15.3 g/dl. 11.5–15.5 g/dl
MCV. 95 fl. 76–98 fl
WCC. 10.3 x 10 9 /l. 4–11 x 10 9 /l
Serum corrected calcium. 2.95 mmol/l. 2.20–2.60 mmol/l
Serum phosphate. 0.7 mmol/l. 0.7–1.4 mmol/l
Serum alkaline phosphatase. 150 iu/l. 30–130 iu/l
What is the underlying diagnosis?
1) Primary hyperparathyroidism
2) Secondary hyperparathyroidism
3) Tertiary hyperparathyroidism
4) Osteoporosis
5) Paget’s disease
Explanation
Primary hyperparathyroidism
The most common causes of hypercalcaemia are primary hyperparathyroidism and malignancy. In this case, given the patient has mild hypercalcaemia, it is more likely that this is due to primary hyperparathyroidism.
Most primary hyperparathyroidism (85%) is caused by the presence of a single parathyroid adenoma. The adenoma produces an excess of parathyroid hormone (PTH), leading to: excessive calcium resorption from bone, increased renal calcium reabsorption, increased renal phosphate excretion and increased production of 1,25-dihydroxyvitamin D3 .
Therefore, blood tests reveal: a raised PTH level, hypercalcaemia, hypophosphataemia and a raised vitamin D 3 level. A mildly raised alkaline phosphatase level is also seen in primary hyperparathyroidism.
Treatment of a parathyroid adenoma is by surgical removal of the parathyroid glands. For patients not fit/suitable for parathyroidectomy, options for treatment include:
preventing vitamin D deficiency, bisphosphonates to protect the bones and calcimimetics to control hypercalcaemia if required.
Secondary hyperparathyroidism
In chronic renal disease, there is a reduction in the secretion of phosphate, with reduced estimated glomerular filtration rate (eGFR). The resulting hyperphosphataemia
stimulates PTH secretion and causes formation of calcium and phosphate crystals, leading to hypocalcaemia.
Therefore, in secondary hyperparathyroidism, biochemical findings include: raised PTH, low calcium, raised phosphate and raised alkaline phosphatase levels.
Tertiary hyperparathyroidism
Following a long-standing period of secondary hyperparathyroidism or following a renal transplant, tertiary hyperparathyroidism can arise. Following a period of
parathyroid gland stimulation, the parathyroid glands continue to secrete PTH autonomously and result in: raised PTH levels, hypercalcaemia and hyperphosphataemia.
Osteoporosis
Osteoporosis leaves calcium, phosphate and PTH levels unaffected. Alkaline phosphatase is normal.
Paget’s disease
Paget’s disease of the bone is associated with excessive bone breakdown and formation, with subsequent disorganised bone remodelling. This results in weakened bones
associated with pain and fractures.
In Paget’s disease, calcium, phosphate and PTH levels remain unaffected. Alkaline phosphatase level is markedly raised in Paget’s disease.
You are in the General Surgery Clinic and the general practitioner has referred a man with a breast lump. You examine the man and find he has gynaecomastia, with no
palpable mass of any concern.
Which of the following is a cause for this?
1) Liver cirrhosis
2) Low-dose, occasional cimetidine
3) Oral corticosteroids
4) Parathyroid gland tumours
5) Regular bromocriptine therapy
Explanation
Liver cirrhosis
Gynaecomastia, defined as ‘benign proliferation of male breast tissue’, is caused by a disruption in the oestrogen:testosterone ratio, in favour of oestrogen. It can be secondary to some medication therapy, most commonly spironolactone.
In liver disease, there is an increased activity of aromatase enzyme, leading to conversion of androgens to oestrogens. Additionally, the resulting high oestrogenic state increases the production of sex hormone-binding globulin (SHBG) in the liver, leading to a further decrease in serum free testosterone levels.
This tip in the balance in favour of oestrogen leads to gynaecomastia, as the oestrogens induce ductal hyperplasia and proliferation of breast tissue. Patients with alcoholic liver disease are at a higher risk for gynaecomastia because
phyto-oestrogens found in alcohol are direct inhibitors of testosterone.
Low-dose, occasional cimetidine
Cimetidine is a histamine receptor blocker used in the treatment of gastro-oesophageal reflux disease and in the prevention of gastric ulcers. Common side-effects of cimetidine include dizziness, drowsiness, diarrhoea and headache. It can rarely cause gynaecomastia or a breast lump, as it affects the cytochrome P450system in the liver and leads to raised serum oestrogens. This can lead to
gynaecomastia but is dose-dependent and seen in high doses and prolonged treatment.
Oral corticosteroids
Oral corticosteroids can lead to fat redistribution but are not known to cause gynaecomastia.
Parathyroid gland tumours
Parathyroid gland tumours present with signs relating to hypercalcaemia such as polyuria, polydipsia, renal stones, constipation and mental disturbances (depression).
Regular bromocriptine therapy Bromocriptine is an ergot derivative and a potent dopamine receptor agonist. It is used
in the treatment of Parkinson’s disease and also to suppress lactation in the treatment of galactorrhoea or in women following an intrauterine fetal death, by inhibiting secretion of prolactin. Known side-effects include: nausea, vomiting, constipation, hypotension, confusion and lethargy. It is not known to cause gynaecomastia.
A 42-year-old woman is referred to the Endocrine Clinic, as she was noted by the general practitioner to have a persistently raised blood pressure which has not responded to medication. She complains of heat intolerance, sweating and palpitations. She also reports a family history of phaeochromocytoma.
Which of the following options would be a good first-line investigation for phaeochromocytoma?
1) Clonidine suppression test
2) MIBG scintigraphy
3) Magnetic resonance imaging (MRI)
4) Plasma noradrenaline
5) 24-hour urinary vanillylmandelic acid (VMA)/homovanillic acid (HVA) secretion
Explanation
24-hour urinary vanillylmandelic acid (VMA)/homovanillic acid (HVA) secretion
Phaeochromocytomas are rare neuroendocrine tumours arising in the chromaffin cells,
most commonly found in the adrenal medulla. They are associated with increased
catecholamine production and tend to present with severe hypertension (often
resistant to multiple therapeutic agents), excessive sweating, anxiety/panic attacks,
headaches and pallor. They are usually curable if detected and treated appropriately. If,
however, they remain undiagnosed, they can lead to severe complications, including
death.
The first-line investigation for phaeochromocytomas includes a 24 -hour urine collection
and quantification of urinary VMA and HVA. These are by-products of catecholamine
production and are present in low concentrations in the urine of unaffected individuals.
In phaeochromocytoma, however, the concentration of these molecules in the urine is
raised, indicating an increased production of catecholam ines.
Phaeochromocytomas are associated with conditions such as multiple endocrine
neoplasia types 2a and 2b, von Hippel–Lindau syndrome and neurofibromatosis.
Clonidine suppression test
The clonidine suppression test forms the second -line test in the diagnosis of
phaeochromocytoma in cases where there is a high clinical suspicion but there is
borderline elevation in the catecholamines, not enough to qualify for a definitive
diagnosis.
The test relies on the fact that clonidine is a suppressor of physiologi cal catecholamine
secretion but has no effect over autonomous catecholamine secretion, like in the case of
phaeochromocytoma.
Collection of urine from 2100 h on day 1 to 0700 h on day 2 occurs and catecholamines
quantified. Then, on day 2, clonidine is administered and collection of urine follows
from 2100 h to 0700 h on day 3. Catecholamines are quantified. In a normal individual,
the urine catecholamines from the second collection, ie following clonidine
administration, should be greatly reduced when comp ared to the original collection. In patients with phaeochromocytoma, however, clonidine is unable to suppress
catecholamine release, and these remain in high concentrations in the urine.
MIBG scintigraphy
MIBG scintigraphy is a very specific test for detecting phaeochromocytoma. Radioactive
iodine-131-metaiodobenzylguanidine is administered to the patient. In a normal
patient, the adrenal medulla appears to uniformly uptake the radioactive solution and
its intensity is less than that of liver uptake. In the case of a patient with
phaeochromocytoma, there is a focal area in the adrenal medulla where there is very
strong and prolonged uptake of the solution. MIBG scintigraphy is not considered the
first-line investigation to detect phaeochromocytoma. MIBG is u seful in detecting extra-
adrenal tumours or disease recurrence.
Magnetic resonance imaging (MRI)
Computerised tomography (CT) and MRI scans are both sensitive in detecting
phaeochromocytomas, with MRI being superior to CT, but are not considered the first -
line investigation. These imaging modalities are used to localise the tumour once
elevated urine catecholamines are detected.
Plasma noradrenaline
Elevated plasma noradrenaline is a sensitive test in detecting phaeochromocytoma. This
test has a high sensitivity of 96%, but a relatively low specificity of 85%, as plasma
noradrenaline can be elevated in other cases such as in patients taking amitriptyline
and some antipsychotic medication.
A 60-year-old man is 2 weeks post-renal transplant, complaining of polyuria and polydypsia. His blood tests show the following:
Result
Hb. 10.1 g/dl. (13-18 g/dl)
MCV. 80 fl. (76–98 fl)
Ca. 2.95 mmol/l. (2.20–2.60 mmol/l)
Phosphate. 1.8 mmol/l. (0.7–1.4 mmol/l)
Alkaline phosphatase (ALP). 150 iu/l. (30–130 iu/l)
What is the underlying problem?
1) Primary hyperparathyroidism
2) Secondary hyperparathyroidism
3) Tertiary hyperparathyroidism
4) Osteoporosis
5) Paget’s disease
Explanation
Tertiary hyperparathyroidism
The patient presents with symptoms relating to hypercalcaemia that is caused by
tertiary hyperparathyroidism. Following a long -standing period of secondary
hyperparathyroidism, such as that occurring during renal failure, tertiary
hyperparathyroidism can arise. This is commonly seen following renal transplantation
to treat renal failure. Due to the prolonged stimulation that has occurred in the
parathyroid glands, in response to the relative serum hypocalcaemia caused by renal
failure, the glands continue to secrete PTH autonomously and inappropriately, even
after the correction of hypocalcaemia. As in the above scenario, this results in: raised
PTH levels, hypercalcaemia and hyperphosphataemia. Alkaline phosphatase level is
mildly raised in all forms of hyperparathyroidism but can also be normal.
Primary hyperparathyroidism
Primary hyperparathyroidism is most commonly due to a parathyroid adenoma and
results in raised parathyroid hormone (PTH) levels, hypercalcaemia (usually mild to
moderate), hypophosphataemia and raised vitamin D 3 levels. A mildly raised alkaline
phosphatase level is also seen in primary hyperparathyroidism. Secondary hyperparathyroidism
In chronic renal disease, there is a reduction in the secretion of phosphate, with reduced
estimated glomerular filtration rate (eGFR). The resulting hyperphosphataemia
stimulates PTH secretion and causes the formation of calcium and phosphate crystals,
leading to hypocalcaemia.
Therefore, in secondary hyperparathyroidism, biochemical findings include: raised PTH,
low calcium, raised phosphate and raised alkaline phosphatase levels.
Osteoporosis
Osteoporosis leaves calcium, phosphate and PTH levels unaffected. Alkaline
phosphatase level is normal.
Paget’s disease
Paget’s disease of the bone is associated with excessive bone breakdown and formation,
with subsequent disorganised bone remodelling. This results in weakened bones
associated with pain and fractures.
In Paget’s disease, calcium, phosphate and PTH levels remain unaffected. Alkaline
phosphatase level is markedly raised in Paget’s disease.
A patient is admitted to A&E with deranged biochemistry. Which of the following malignancies could be responsible for hypercalcaemia and low serum phosphate levels?
1. Osteoclastoma 2. Squamous cell carcinoma of the lung 3. Prostate cancer 4. Transitional cell carcinoma of the bladder 5. Basal cell carcinoma
Squamous cell carcinoma of the lung is responsible for hypercalcaemia and low serum phosphate levels. Ten percent of all lung cancers produce hormones or peptides involved in paraneoplastic syndromes.
One of the most commonly encountered paraneoplastic syndromes is humoral hypercalcaemia of malignancy, seen with squamous cell carcinoma of the lung. This cancer produces parathyroid hormone-related peptide, which mimics the action of parathyroid hormone.
Consequently, it causes hypercalcaemia and hypophosphataemia, as parathyroid hormone stimulates calcium resorption from bone, renal calcium reabsorption, renal phosphate excretion, and increased production of 1,25-dihydroxyvitamin D3.
Osteoclastoma is normally a benign primary bone tumor and does not typically cause hypercalcaemia and hypophosphataemia.
Prostate cancer can metastasize to the bones and lead to the release of calcium and phosphate into the bloodstream, resulting in hypercalcaemia and hyperphosphataemia.
Transitional cell carcinoma of the bladder may metastasize to the bones. Metastatic cancerous bone lesions can result in the release of mineralized calcium and phosphate into the bloodstream, leading to hypercalcaemia and hyperphosphataemia.
Basal cell carcinoma rarely associates with hypercalcaemia. Hypercalcaemia is more commonly associated with squamous cell carcinoma of the skin and melanoma, but not with basal cell carcinoma.
A 70-year-old man complains of constantly feeling cold and lethargic. He presents to the outpatient clinic referred by the GP as possible malignancy. What is the most likely hormonal deficiency to account for this?
1. Somatostatin 2. Cholecystokinin 3. Testosterone 4. Thyroxine 5. Insulin
Thyroxine deficiency, or hypothyroidism, is most likely to be affecting the patient in this scenario. It can be either primary (due to the thyroid gland not producing enough thyroxine) or secondary (where the thyroid gland is normal but does not receive enough TSH for stimulation of hormone production, suggesting a pituitary problem). Thyroxine is involved in the stimulation of cell metabolism and thermoregulation. Deficiency presents with a variety of symptoms relating to metabolic and mental slowness. If hypothyroidism is confirmed, it is treated with hormone replacement with a synthetic T4 molecule and monitored by regular TSH serum levels.
Somatostatin is a peptide hormone produced in the delta cells of the islets of Langerhans in the pancreas and the ventromedial nucleus of the hypothalamus. It inhibits the production of glucagon and insulin in the pancreas and the secretion of gastric acid, gastrin, and secretin. In the pituitary, it inhibits the secretion of multiple hormones such as growth hormone and thyroid-stimulating hormone (TSH). Somatostatin overproduction can result in diabetes, gallstones, fat intolerance in the diet, steatorrhea, and diarrhea. Somatostatin deficiency is very rare; only a few cases have been reported in the literature. However, decreased cerebrospinal fluid levels of somatostatin have been documented in patients with Alzheimer’s disease.
Cholecystokinin is a hormone produced by the I cells of the duodenum, which has multiple functions. It is released in response to the presence of fat in the duodenum and inhibits gastric emptying, promotes pancreatic hormone secretion, and promotes contraction of the gall bladder and relaxation of the sphincter of Oddi. Deficiency of cholecystokinin is extremely rare and usually manifests as part of a polyglandular syndrome, resulting in malabsorption.
Testosterone deficiency presents with loss of libido, erectile dysfunction, low semen volume, fatigue, hair loss, decrease in bone and muscle mass, with a corresponding increase in body fat, as well as mood changes.
Insulin deficiency presents with features of diabetes mellitus such as polyuria, polydipsia, increased thirst, and loss of weight.
Remember, hypothyroidism is ten times more common in women than men and occurs mainly in middle life.
A 41-year-old woman presents with renal colic. Biochemistry results reveal a plasma calcium level of 3.3 mmol/l (2.20–2.60 mmol/l), with an albumin level of 45 g/l (35 –55 g/l). Her medical history includes dyspepsia and depression.
What is the most likely underlying diagnosis?
1) Actinomycosis infection
2) Ectopic parathyroid hormone
3) Hyperparathyroidism
4) Hypoparathyroidism
5) Vitamin D deficiency
Explanation
Hyperparathyroidism
The patient presents symptomatic of hypercalcaemia with a renal stone, dyspepsia and depression. Given the patient is young and fit, the most likely cause of the hypercalcaemia seen in this scenario is primary hyperparathyroidism. However, as hypercalcaemia is commonly due to malignancy, this needs to be excluded.
Primary hyperparathyroidism is, in the majority of cases (85%), the result of a single parathyroid adenoma. Other causes include: parathyroid gland hyperplasia affecting all
four parathyroid glands (10–15%), multiple parathyroid adenomas (3–5%) or a parathyroid carcinoma (1%).
The adenoma produces an excess of parathyroid hormone, leading to excessive calcium resorption from bone, increased renal calcium reabsorption, increased renal phosphate
excretion and increased production of 1,25-dihydroxyvitamin D3 .
Therefore, blood tests reveal: a raised parathyroid hormone level, hypercalcaemia, hypophosphataemia and a raised vitamin D 3 level.
Treatment of a parathyroid adenoma is by surgical removal of the parathyroid glands (parathyroidectomy). For patients not fit/suitable for parathyroidectomy, options for treatment include: preventing vitamin D deficiency, bisphosphonates to protect the bones and calcimimetics to control hypercalcaemia if required.
Actinomycosis infection
Actinomycosis infection is a bacterial infection characterised by a suppurative granulomatous inflammation and formation of multiple abscesses, discharging sulfur - containing granules. It commonly affects the thorax, abdomen or face and neck (cervicofacial). Abdominal actinomycosis presents with sensation of a mass, fever,
weight loss, fatigue, nausea and vomiting. Blood biochemistry is usually normal, with the exception of a raised alkaline phosphatase level in the case of hepatic actinomycosis.
Ectopic parathyroid hormone
Ectopic parathyroid hormone secretion is usually secondary to malignancy as part of a paraneoplastic syndrome. Even though hypercalcaemia of malign ancy is a common cause of hypercalcaemia and needs to be excluded in this case, given the patient is young and fit, the most likely cause of the biochemical picture seen is primary hyperparathyroidism. Hypoparathyroidism
Hypoparathyroidism results in parathyroid hormone deficiency and subsequently in hypocalcaemia.
Vitamin D deficiency
Vitamin D deficiency would result in hypocalcaemia, as vitamin D is essential for the absorption of dietary calcium in the gastrointestinal system.
A patient is seen in the clinic with exopthalmous.
Which of the following thyroid function tests (TFTs) is suggestive of Graves’
disease?
1) Raised thyroid-stimulating hormone (TSH), raised free T4, raised free T3
2) Normal TSH, raised free T4, decreased T3
3) Decreased TSH, raised free T4, raised free T3
4) Decreased TSH, decreased free T4, decreased free T3
5) Raised TSH, normal free T4, normal free T3
Explanation
Decreased TSH, raised free T4, raised free T3
Graves’ disease is an autoimmune condition leading to a diffuse enlargement of the
thyroid, hyperstimulation and increased production of thyroid hormones. In Graves’
disease, there are thyrotropin receptor antibodies (immunoglobulin G (IgG) antibodies)
circulating in the blood, stimulating the TSH receptor and promoting the production of
thyroid hormones.
There is therefore increased free T3 and T4; this acts as a negative feedback on the
pituitary, suppressing the production of endogenous TSH, which is why, in Graves’
disease, TSH is low.
Raised thyroid-stimulating hormone (TSH), raised free T4, raised free T3
This can be seen in the presence of a TSH-secreting pituitary tumour, amiodarone
therapy or acute psychiatric illness.
Normal TSH, raised free T4, decreased T3
Beta blocker therapy interferes with the conversion of T4 to T3 and can therefore cause
the above picture.
Decreased TSH, decreased free T4, decreased free T3
This clinical scenario describes patients who have recently been treated for
hyperthyroidism or patients with congenital TSH or thyrotropin-releasing hormone
deficiency.
Raised TSH, normal free T4, normal free T3
This is the presentation of subclinical hypothyroidism.
A 49-year-old woman presents to the Outpatient Clinic with a corrected calcium level of 1.9 mmol/l. Clinical examination is unremarkable.
What is the most likely cause for her clinical problem?
1) Acute renal failure
2) Multiple endocrine neoplasia type 1 (MEN 1)
3) Parathyroid adenoma
4) Pregnancy
5) Vitamin D deficiency
Explanation
Vitamin D deficiency
Vitamin D is essential for the absorption of calcium in the gastrointestinal tract.
Therefore, a deficiency in vitamin D can cause reduced absorbed calcium from the diet and lead to hypocalcaemia.
Secondary hyperparathyroidism is a condition that results in stimulation of PTH secretion due to a reduction in serum calcium concentration. It is most commonly seen in chronic renal failure where there is reduced vitamin D activation and reduced phosphate excretion. The result is a reduction in calcium absorption from the gastrointestinal tract, but also binding of the free calcium to phosphate, causing the
formation of calcium phosphate compounds. These both lead to hypocalcaemia and stimulation of the parathyroid glands to produce PTH, in an effort to increase calcium
concentration in the serum.
Acute renal failure
Hypocalcaemia is often seen with chronic renal failure, as part of secondary hyperparathyroidism.
Multiple endocrine neoplasia type 1 (MEN 1)
MEN 1 is associated with a parathyroid adenoma, leading to hypercalcaemia through the mechanism of primary hyperparathyroidism, a pancreatic islet cell tumour and a
pituitary tumour.
Parathyroid adenoma
A parathyroid adenoma leads to primary hyperparathyroidism, through excess
secretion of parathyroid hormone (PTH) from the adenoma. Biochemical features of
primary hyperparathyroidism include: hypercalcaemia, hypophosphataemia, raised
PTH level and raised vitamin D 3 level. There is sometimes raised alkaline phosphatase
level as well.
Pregnancy
Pregnancy is a cause of primary hyperparathyroidism, resulting from hyperplasia of the
parathyroid glands.
A 23-year-old woman is commenced on
long-term
prednisolone for adrenal
insufficiency. She is currently otherwise fairly fit and well
On which of the side-effects should you counsel her?
1) Avascular necrosis of bone
2) Hepatotoxicity
3) Hyperkalaemia
4) Bone marrow suppression
5) Hypotension
Explanation
Avascular necrosis of bone
Avascular necrosis of bone and osteoporosis are well -recognised side-effects of long-
term corticosteroid therapy, irrespective of its mode of administration.
This is a multifactorial process, characterised by interruption of the vascular supply to
the bone, leading to marrow, medullary bone and cortex necrosis. Most commonly,
affected bones are those with a terminal blood supply, and necrosis occurs in the
epiphysis. These include the femur, humerus, carpals and talus.
Factors that increase the risk include: female gender, high daily dose and prolonged
therapy. It is thought that concurrent autoimmune disease also increases the risk of
developing avascular necrosis with corticosteroid therapy.
Hepatotoxicity
Glucocorticoid therapy is not known to have toxic effects on the liver.
Hyperkalaemia
Glucocorticoids exert several effects on the kidneys. They increase water diuresis and
glomerular filtration rate, while causing hypernatraemia and hypokalaemia, due to an
increase in sodium retention and potassium secretion.
Bone marrow suppression
Initiating corticosteroid therapy can cause a refl ex leukocytosis, predominantly
affecting polymorphonuclear leukocytes, ie neutrophils.
Prolonged therapy can lead to suppression of the immune system and increase the risk
for infection. Steroid therapy is not, however, a common cause of bone marrow
suppression, with common causes being cytotoxic chemotherapy or
immunosuppressants such as azathioprine.
Hypotension
Glucocorticoid therapy is associated with hypertension, and not hypotension. Steroids
stimulate the mineralocorticoid receptor in the kidney, resulting in sodium and water
resorption, increase in intravascular volume and therefore increase in blood pressure.
A 44-year-old woman is seen in the pre-assessment clinic before inguinal hernia repair. Her chest radiograph is normal and her blood results are as follows:
Ca. 3.0 mmol/l. 2.20–2.60 mmol/l
Na. 136 mmol/l. 135–145 mmol/l
K. 3.7 mmol/l. 3.5–5.0 mmol/l
Urea. 5.5 mmol/l. 2.5–6.5 mmol/l
Cr. 50 μmol/l. 50–120 μmol/l
What is the most likely cause of the hypercalcaemia?
1) Addison’s disease
2) Chronic renal failure
3) Diabetes mellitus
4) Sarcoidosis
5) Thyrotoxicosis
Explanation
Thyrotoxicosis
Thyrotoxicosis is associated with hypercalcaemia. Thyroid hormones directly stimulate bone resorption leading to decalcification and demineralisation leading to an increase in
serum calcium. This mechanism is not associated with hyperparathyroidism. However, there is a significant proportion of patients with hyperthyroidism that have concomitant primary hyperparathyroidism, leading to hypercalcaemia.
It is estimated that hypercalcaemia is seen in 25% of patients with proven hyperthyroidism. The patient will need other tests such as: thyroid-stimulating hormone (TSH), parathyroid hormone (PTH) as well as free T4 / T 3 to confirm the diagnosis. Toxic goitres tend to most commonly present around the 4 th -5 th decade of life, fitting with the age of the patient in this scenario.
Addison’s disease
Addison’s disease is a known cause of hypercalcaemia. It is due to adrenocortical insufficiency leading to glucocorticoid and mineralocorticoid deficiency. As a result of the mineralocorticoid (aldosterone) deficiency there is sodium loss in the kidneys and potassium retention.
Therefore Addison’s disease is associated with hyponatraemia an hyperkalaemia. With a normal sodium and potassium in this scenario the cause of the hypercalcaemia is not Addison’s disease.
Chronic renal failure
In this scenario the patient’s renal function is completely normal, with a normal urea and creatinine, ruling out chronic renal failure. Chronic renal failure is a common cause of secondary hyperparathyroidism presenting with a raised parathyro id hormone, hypocalcaemia and hyperphosphataemia. This is because in chronic renal disease there is a reduction in the excretion of phosphate and a low glomerular filtration rate. The retained phosphate creates phosphate–calcium compounds in the blood leading to a low free serum calcium.
Diabetes mellitus
Patients with diabetes mellitus are more likely to develop hypocalcaemia, as a result of diabetic renal disease, rather than hypercalcaemia. However, in some cases there is co -
existence of hyperparathyroidism and diabetes mellitus.
Sarcoidosis
Even though sarcoidosis is a cause of hypercalcaemia, it is unlikely in this scenario given the mention of a normal chest radiograph. Sarcoidosis presents with hilar and mediastinal node enlargement as well as parench ymal lung disease, both evident on chest radiography.
A 53-year-old woman undergoes
total thyroidectomy
for a benign multinodular goitre.
Her post-operative calcium is
1.8 mmol/l
and she complains of
tingling
in her fingers.
What is the most likely diagnosis?
1) Hypercalcaemia and overall reduced osteoclast activity
2) Hypocalcaemia and overall increased bone resorption and reduced parathyroid
hormone secretion
3) Hypocalcaemia and reduced conversion of vitamin D to 1,25-
dihydroxycholecalciferol
4) Hypocalcaemia and reduced renal reabsorption of calcium through the action of
1,25-dihdroxycholecalciferol
5) Hypocalcaemia and increased bone resorption
Explanation
Hypocalcaemia and reduced conversion of vitamin D to 1,25-dihydroxycholecalciferol
Post-operative hypocalcaemia is the most common complication encountered following
a thyroidectomy. This is caused by acute parathyroid insufficiency from the reduction in
the parathyroid parenchymal tissue, as often some or all of the parathyroid glands are
removed during a total thyroidectomy. This results in a reduced level of synthesis of
parathyroid hormone and leads to a reduction in bone resorption, reduced conversion
of vitamin D to 1,25-dihydroxycholecalciferol in the kidney are a
resulting hypocalcaemia due to a reduced absorption of dietary calcium in the
gastrointestinal tract. The resulting hypocalcaemia presents with paraesthesia in the
extremities and peri-orally (circumoral), abdominal cramps, tetany and posi tive
Chvostek’s and Trousseau’s signs.
Hypercalcaemia and overall reduced osteoclast activity
The patient presents with hypocalcaemia.
Hypocalcaemia and overall increased bone resorption and reduced parathyroid
hormone secretion
Increased bone resorption is the result of increased action of the parathyroid hormone
(PTH) and leads to hypercalcaemia. In hypoparathyroidism, there is a reduced level of
parathyroid hormone, with an overall decrease in bone resorption and hypocalcaemia.
Hypocalcaemia and reduced renal reabsorption of calcium through the action of 1,25 -
dihdroxycholecalciferol
Absorption of calcium through the action of 1,25 -dihydroxycholecalciferol occurs in the
gastrointestinal tract and not in the renal tract.
Hypocalcaemia and increased bone resorption Increased bone resorption is the result of increased action of parathyroid hormone
(PTH) and leads to hypercalcaemia and not hypocalcaemia.
You review a 75-year-old woman in fracture clinic who has a left distal radial fracture,
following a low energy injury. She has previously had a fractured neck of femur and two
vertebral wedge fractures. You discuss osteoporosis with the patient. They ask you what
can cause poor bone calcification.
Which one of the following is a cause of poor bone calcification?
1) Hypothyroidism
2) Bisphosphonates
3) Calcitonin
4) Hormone replacement therapy (HRT)
5) 1,25-Dihydroxyvitamin D deficiency
Explanation
1,25-Dihydroxyvitamin D deficiency
This patient presents with fragility fractures secondary to osteoporosis. Osteoporosis, is
a disease characterised by reduced bone strength leading to increased risk of fractures
and it most commonly occurs in post-menopausal women due to a variety of causes that
favour bone catabolism rather than bone mass increases and build up. Osteoporosis
presents with unusual fractures at low impact eg vertebral, neck of femur and Colles’
fractures. Vitamin D deficiency is one of the common causes leading to osteoporosis and
fragility fractures. Inadequate 1,25-dihydroxyvitamin D (1,25-dihydroxycholecalciferol)
can result either from inadequate exposure to sunlight, inadequate intake of vitamin D.
Vitamin D deficiency in children results in rickets, and in adults in osteomalacia, both of
which are characterised by a reduced mineral density of the bones making bones brittle
and fragile. Additionally, in the absence of vitamin D causes a reduced absorption of
calcium from the gastrointestinal system, leading to serum hypocalcaemia which is
detected by the parathyroid glands that stimulate production of parathyroid hormone.
The latter leads to an increase in bone resorption. Bones therefore lose their calcium
and become demineralised and brittle. Management of osteoporosis is multifaceted. It
includes lifestyle changes such as adequate calcium and vitamin D intake, smoking
cessation, introduction of exercise in daily activities, reduction in alcohol consumption
and falls prevention strategies. Pharmacological treatments include calcium and vitamin
D supplementation, bisphosphonates and calcitonin.
Hypothyroidism
In hyperthyroidism, the excess circulating thyroxine, increases the rate of bone
metabolism and resorption. Hypothyroidism however does not have a similar effect.
Bisphosphonates
Bisphosphonates are used in the treatment of osteoporosis as they act by promoting
osteoclast apoptosis and slowing bone resorption. They reduce the risk of fragility
fractures in post-menopausal women with osteoporosis. The most commonly used
bisphosphonate is alendronic acid.
Calcitonin Calcitonin, a hormone produced by the C-cells of the thyroid gland, in response to
hypercalcaemia reduces the serum calcium level and opposes the effect of parathyroid
hormone. It inhibits the resorption of bone by inhibiting osteoclast activity. Therefore, it
helps in maintaining adequate bone calcification and is being used as a treatment for
patients with osteoporosis.
Hormone replacement therapy (HRT)
Hormone replacement therapy, in the form of oestrogen only or oestrogen –
progesterone, slows bone metabolism and increases bone mineral density and reduces
the risk of a bone fragility fracture. However, once HRT is discontinued, there is
accelerated bone metabolism with a rapid decrease in bone mineral density and the
protective function of HRT that has preceded is lost. Osteoporosis is more common in
post-menopausal women due to the loss of the oestrogenic protective factors.
You are treating a patient pre-operatively who is going to undergo resection of a phaeochromocytoma. The patient is exhibiting features of high levels of circulating adrenaline.
What signs and symptoms would you expect to see?
1) Decreased sweat production, paroxysmal hypertension and decreased metabolic rate
2) Sweating, paroxysmal hypertension and decreased metabolic rate
3) Sweating, paroxysmal hypertension and increased metabolic rate
4) Sweating, paroxysmal hypotension and decreased metabolic rate
5) Sweating, paroxysmal hypotension and increased metabolic rate
Explanation
Sweating, paroxysmal hypertension and increased metabolic rate
Phaeochromocytomas are rare tumours, most commonly arising in the chromaffin cells of the adrenal medulla and resulting in increased secretion of catecholamines (adrenaline, noradrenaline, dopamine, occasionally). Adrenaline is a potent stimulator of the sympathetic system and increases the metabolic rate causing sweating. It binds to a- and b-adrenoceptors. Binding to a-adrenergic receptors brings about effects such as vasoconstriction, elevation in blood pressure, glycogenolysis and gluconeogenesis.
Binding to b-adrenergic receptors brings about tachycardia and increased contractility of the heart. Phaeochromocytoma is treated by adrenalectomy, however, adequate a- and b-adrenoceptor blockage is necessary before surgery. Alpha-receptor blockage controlsblood pressure and prevents a hypertensive crisis, whereas b -receptor blockage controls the heart rate. Adequate a-adrenoceptor blockage is necessary first, as unopposed alpha adrenergic activity that can occur during b-blockade may precipitate a hypertensive crisis.
Decreased sweat production, paroxysmal hypertension and decreased metabolic rate
Phaeochromocytoma is associated with increased sweat production, paroxysmal hypertension and increased metabolic rate.
Sweating, paroxysmal hypertension and decreased metabolic rate
Although phaeochromocytomas can occur in any age their peak incidence has been reported in patients between the age of 30–50.
Sweating, paroxysmal hypotension and decreased metabolic rate
Occasionally patients with phaeochromocytoma can have postural hypotension during an episode due to the high volume of fluid lost from excessive sweating.
Sweating, paroxysmal hypotension and increased metabolic rate Phaeochromocytomas typically present with a tetrad of headaches, palpitations, severe hypertension and excessive sweating. It is important to note that the symptoms are not
constant and are episodic, varying in frequency and duration over the course of the day.
As the tumour size increases, episodes become more frequent and prolonged.
An elderly man with a history of prostatism presents with acute retention of urine. His serum creatinine concentration is 520 μmol/l (50–120 μmol/l).
Which one of the following additional abnormal serum biochemistry test results is most suggestive of a chronic component to his renal failure?
1) Hyperkalaemia
2) Hyperuricaemia
3) Hypocalcaemia
4) Hyponatraemia
5) Low serum bicarbonate concentration
Explanation
Hypocalcaemia
Hypocalcaemia suggests that there is at least in part a longstanding element to the renal failure seen in this patient. This is due to two mechanisms: firstly there is reduced renal
synthesis of 1,25-dihydrocholecalciferol, the active form of vitamin D, necessary in the absorption of dietary calcium in the gastrointestinal system. Secondly, as the glomerular
filtration rate (GFR) drops, and the filtering ability of the kidney deteriorates, less phosphate is excreted in the urine. The circulating phosphate forms calcium-phosphate
compounds, reducing the amount of free circulating calcium. These two mechanisms lead to hypocalcaemia.
Hyperkalaemia
Hyperkalaemia can be the result of both acute and chronic renal failure and occurs due to reduced excretion of potassium and accumulation in the body.
Hyperuricaemia
Hyperuricaemia is the result of both acute and chronic renal failure and occurs secondary to the reduced ability of the kidney to filter and excrete waste products from the blood, leading to accumulation of these in the blood.
Hyponatraemia
Hyponatraemia can be a feature of both acute and chronic renal failure and occurs mainly due to a continued fluid intake while there has been a reduced ability to excrete
this fluid.
Low serum bicarbonate concentration
Low serum bicarbonate is a feature of metabolic acidosis that can precipitate both acute and chronic renal failure.
A patient is seen in the surgical endocrine clinic. They have developed systemic signs
and symptoms.
Which one of the following hormones is secreted by the
anterior
pituitary?
1) Testosterone
2) Oxytocin
3) TSH
4) CRH
5) ADH
Explanation
TSH
Thyroid-stimulating hormone (TSH) is produced by the thyrotrope cells in the anterior
pituitary. The anterior pituitary gland produces six hormones.
Testosterone
Testosterone is produced in the Leydig cells of the testicles, under the influence of
luteinising hormone (LH) and follicle-stimulating hormone (FSH) produced from the
anterior pituitary gland.
Oxytocin
Oxytocin is produced by the posterior pituitary gland and causes uterine contraction,
milk ejection and labour.
CRH
Corticotrophin releasing hormone (CRH) is produced by cells in the paraventricular
nucleus of the hypothalamus and stimulates corticotropes in the anterior pituitary to
produce ACTH.
ADH
Antidiuretic hormone (ADH), also known as vasopressin, is secreted form the posterior
pituitary gland and increases water permeability of the renal collecting ducts and distal
convoluted tubules leading to increased water reabsorption in the kidneys.
A patient presents with failure to tolerate cold and lethargy. Following thyroid function tests a diagnosis of hypothyroidism of the thyroid gland is made. Hypothyroidism is associated with increased plasma level of which one of the following?
1) Cholesterol
2) Albumin
3) rT3
4) Iodide
5) Thyroid stimulating immunoglobin
Explanation
Cholesterol
Hypothyroidism is the second most common cause of hypercholesterolaemia after diet. Thyroid hormones are inducers of HMG –CoA reductase enzyme, the first step in cholesterol synthesis, but also T 3 upregulates low-density lipoprotein (LDL) receptors in
the liver, where LDL-cholesterol binds and catabolism occurs.
Therefore, in hypothyroidism, even though there is a reduction in cholesterol synthesis there is also a downregulation of LDL receptors and catabolism of cholesterol in the liver, leading to an overall increase in the serum level of cholesterol. Hypothyroid patients are at an increased risk of developing cardiovascular disease and their lipid profile should be carefully monitored.
Albumin
Thyroid hormones promote albumin catabolism, while some of the circulating thyroid molecules travel in the blood bound to albumin. Therefore in hypothyroidism, there is decreased albumin catabolism, leading to more albumin available for glycosylation. It is the glycated albumin level that is raised in hypothyroid patients. Similarly in
hyperthyroidism, the glycated albumin levels are reduced as albumin catabolism is induced.
rT3
rT3 , is reverse tri-iodothyronine and is an inactive molecule formed by the deiodination of thyroxine. It differs from tri-iodothyronine in the positions that the iodine atoms are attached to the aromatic rings. The concentration of rT 3 in the serum tends to follow that of T4 . It is therefore reduced in hypothyroidism and raised in hyperthyroidism.
Iodide
Iodide is reduced in hypothyroid disease.
Thyroid stimulating immunoglobin
Thyroid-stimulating immunoglobulin is an antibody that binds the thyrotropin (TSH) receptor on the thyroid gland, stimulating thyroid hormone production. These are diagnostic of Grave’s disease, a form of hyperthyroidism.
A patient presents to the A&E with pneumonia. Incidentally, significant biochemical
abnormalities are found.
A parathyroid adenoma will be most likely to cause which one of the following?
1) Decreased osteoclastic activity
2) Decreased urinary phosphate excretion
3) Hypocalcaemia
4) Increased osteoblastic activity
5) Increased osteoclastic activity
Explanation
Increased osteoclastic activity
A parathyroid adenoma leads to increased production of parathyroid hormone (PTH)
the actions of which aim to increase serum calcium. This is done by acting on the bone,
gastrointestinal system and the kidney. In the bone PTH stimulates the activity of
osteoclasts in the bone leading to increased bone resorption. In the kidney it promotes
the conversion of 25-hydroxycholecalciferol to 1,25-dihydroxycholecalciferol, the active
form of vitamin D, necessary to absorb dietary calcium from the gut. In the kidney PTH
also promotes excretion of phosphate in the urine at the renal proximal convoluted
tubule and calcium reabsorption in the distal convoluted tubule.
Decreased osteoclastic activity
A parathyroid adenoma produces parathyroid hormone (PTH) that stimulates
osteoclastic activity.
Decreased urinary phosphate excretion
A parathyroid adenoma producing parathyroid hormone (PTH) causes increased
urinary phosphate excretion in the proximal convoluted tubule.
Hypocalcaemia
A parathyroid adenoma produces parathyroid hormone (PTH) that causes
hypercalcaemia, hyperchloraemia and hypophosphataemia.
Increased osteoblastic activity
A parathyroid adenoma produces parathyroid hormone (PTH) that promotes the action
of osteoclasts, leading to bone resorption.
A 38-year-old woman presents with a plasma thyroid -stimulating hormone (TSH)
of
12.5 μ/L
(normal 0.17–3.2 μ/l) and a T 3 resin uptake of
19% (normal 25–35%).
Which one of the following clinical symptoms and signs would you expect in this
patient?
1) Anxiety
2) Increased body temperature
3) Palpitations
4) Periorbital swelling and lethargy
5) Tachycardia
Explanation
Periorbital swelling and lethargy
A raised TSH combined with a low T 3 resin uptake, and a low T4 , are characteristics of
hypothyroidism. Hypothyroidism is associated with a dull facial expression, periorbital
swelling secondary to mucopolysaccharide infiltration, decreased adrenergic drive –
causing bradycardia/cold intolerance/lethargy, as well as reduced basal metabolic rate
leading to decreased appetite and weight gain. Patients also experience mental
slowness, depression and forgetfulness. Periorbital swelling in hypothyroidism needs to
be distinguished to exophthalmos in Grave’s disease which is proptosis due to increased
retro-orbital tissue.
Anxiety
Anxiety is seen in hyperthyroid patients. The results of the thyroid function tests above
indicate that the patient is in a hypothyroid state.
Increased body temperature
Increased body temperature is a sign seen in hyperthyroid patients. The results of the
thyroid function tests above indicate that the patient is in a hypothyroid state.
Palpitations
Palpitations are experienced by hyperthyroid patients. The results of the thyroid
function tests above indicate that the patient is in a hypothyroid state.
Tachycardia
Tachycardia is a sign associated with hyperthyroidism. The results of
the thyroid function tests above indicate that the patient is in a hypothyroid state.
A 13-year-old girl develops
exophthalmos
, nervousness, diarrhoea and weight loss
following the death of her mother. Her blood pressure is
170/90 mmHg
(normal value
<120/80 mmHg).
What is the most likely diagnosis?
1) Multiple endocrine neoplasia 2 (MEN type 2)
2) Phaeochromocytoma
3) Graves’ disease
4) Retro-orbital pseudotumour
5) Acute appendicitis
Explanation
Graves’ disease
Graves’ disease is the most common cause of hyperthyroidism and is an autoimmune
condition mediated by thyroid-stimulating hormone antibodies. These bind the thyroid-
stimulating hormone receptor and mimic the action of endogenous TSH, leading to
hyperplasia of the follicular cells of the thyroid and excess hormone production. It can
present with a toxic diffuse goitre. It is m ore common in women, with a female-to-male
ratio of 7–8:1. The typical age range of presentation is 20 –40, but there have been
reports in teenage patients. The patient in this scenario presents symptomatic of
hyperthyroidism: nervousness, hypertension, diarrhoea and weight loss. Other
symptoms include: sweating, heat intolerance, tachycardia, an increased appetite and
emotional lability.
This patient also exhibits signs of Graves’ ophthalmopathy with exophthalmos and
proptosis from increased retro-orbital tissue. It is also associated with upper eyelid
retraction and lid lag. Treatment of Graves’ disease involves antithyroid drugs such as
carbimazole and propylthiouracil. Given the patient’s age propylthiouracil should be
avoided due to the high risk of hepatotoxicity especially in the paediatric population.
Other treatments involve radioactive iodine and thyroidectomy.
Multiple endocrine neoplasia 2 (MEN type 2)
Multiple endocrine neoplasia type 2 is associated with tumours of the endocrine system
and typically involves phaeochromocytoma and medullary thyroid cancer. Type 2A also
involves hyperparathyroidism, while those with type 2B may demonstrate a Marfanoid
body habitus. Even though the patient exhibits some signs associated with
phaeochromocytoma, it is very unlikely in this age group. Additionally, exophthalmos is
mostly associated with Graves’ disease, making hyperthyroidism a more likely
diagnosis.
Phaeochromocytoma
Even though the patient exhibits some signs associated with phaeochromocytoma
(hypertension, weight loss, diarrhoea, anxiety), it is an unlikely diagnosis in this age group. Additionally, exophthalmos is mostly associated with Graves’ disease, making
hyperthyroidism a more likely diagnosis.
Retro-orbital pseudotumour
A retro-orbital pseudotumour is an inflammatory condition involving the extraocular
muscles that presents with painful unilateral exophthalmos and diplopia. It is not
associated with diarrhoea or weight loss.
Acute appendicitis
Even though acute appendicitis can present with diarrhoea as well as hypertension and
anxiety relating to pain, it is not associated with exophthalmos or weight loss as in the
scenario above.
A patient is referred with refractory hypertension. Despite basic medical
treatment their blood pressure remains 180/110mmHg.
Which one of the following is correct regarding adrenals and surgical
hypertension?
1) Renovascular disease is a rare cause of secondary hypertension
2) A phaeochromocytoma can be shown by metaiodobenzylguanidine (MIBG)
scintigraphy
3) Most adrenal incidentalomas are hormone secreting
4) A raised midnight cortisol is diagnostic of Cushing’s syndrome
5) 30% of phaeochromocytomas are extra-adrenal
Explanation
A phaeochromocytoma can be shown by metaiodobenzylguanidine (MIBG) scintigraphy
MIBG scintigraphy is a very specific test for detecting phaeochromocytoma. Radioactive
iodine-131-metaiodobenzylguanidine is administered to the patient and localises to
adrenergic tissue. In the case of a patient with a phaeochromocytoma there is a focal
area in the adrenal medulla where there is very strong and prolonged uptake of the
solution. MIBG scintigraphy is not considered the first line investigation to detect
phaeochromocytoma. MIBG is useful in detecting extra-adrenal tumours or disease
recurrence.
Renovascular disease is a rare cause of secondary hypertension
Secondary hypertension accounts for 5–10% of hypertensive patients. Renovascular
disease is the most common cause of secondary hypertension, occu rring due to
narrowing of the renal artery (renal artery stenosis). It typically presents in young
women (<30 years) and older men (>50 years). Other causes of secondary hypertension
include: Conn syndrome, Cushing syndrome, phaeochromocytoma, hyperthyroid ism,
congenital adrenal hyperplasia, use of oral contraceptives, and pregnancy.
Most adrenal incidentalomas are hormone secreting
Adrenal incidentalomas are described as adrenal masses found incidentally. Of these,
85% are non-functional, ie do not secrete any hormones and the remaining 15% are
either hormone-secreting or malignant.
A raised midnight cortisol is diagnostic of Cushing’s syndrome
Cortisol levels are secreted in a diurnal rhythm. They peak in the morning between
0700–0900 h and then gradually reduce throughout the day, reaching a nadir at around
midnight. In Cushing syndrome there is loss of the diurnal rhythm and a raised midnight
cortisol is thought to be the earliest sign of Cushing syndrome with a very high
sensitivity. However, other factors can cause a midnight cortisol surge such as stress
resulting from physical illness, being awake at midnight to perform the test and depression. As a result, for the diagnosis of Cushing syndrome the dexamethasone
suppression test is used.
30% of phaeochromocytomas are extra-adrenal
About 10% of phaechromocytomas are extra-adrenal. It is important to remember that
phaeochromocytoma follows the rule of 10s: 10% are malignant, 10% are bilateral, 10%
are found in children, 10% of patients have multiple tumours, 10% are extra -adrenal
(found in the thorax, neck, bladder, kidney, scrotum) and 10% are familial (von Hippel –
Lindau syndrome).
You are treating a patient with hyperthyroidism. They want to know what the options
for ongoing management are.
Which one of the following statements is correct?
1) With antithyroid drugs patients have a high recurrence rate when therapy is
discontinued
2) Surgery is the most rapid method of permanent control and results in
euthyroidism in about 50% of cases at 5 years
3) Surgery has no permanent complications
4) Radio-iodine therapy gives permanent control and h as a low rate of
hypothyroidism
5) All other treatments, except surgery, are contraindicated in pregnancy
Explanation
With antithyroid drugs patients have a high recurrence rate when therapy is
discontinued
Antithyroid drugs do not offer permanent control as seen with surgery or radio-iodine.
If the patients discontinue their antithyroid medication, hyperthyroidism recurs in the
vast majority of cases. Antithyroid medication such as propylthiouracil and carbimazole
work as hormone antagonists therefore following cessation the disease can recur.
Combining antithyroid medication and radio -iodine therapy increases the rate of
achieving permanent control.
Surgery is the most rapid method of permanent control and results in euthyroidism in
about 50% of cases at 5 years
Thyroid surgery can lead to hypothyroidism, and occurs in 90% of patients post -
operatively at 5 years and this rises to almost 100% in 20 years.
Surgery has no permanent complications
Thyroid surgery is not without complications. These are grouped in to immediate, early
and late complications.
Radio-iodine therapy gives permanent control and has a low rate of hypothyroidism
Radio-iodine therapy yields good results and generally has a lower rate of recurrence of
hyperthyroidism when compared to medical therapy only. Return to a euthyroid state
takes about 2 months following treatment. Hypothyroidism is however a common
complication following radio-iodine therapy, affecting patients at a rate of up to 90% in
the first year.
All other treatments, except surgery, are contraindicated in pregnancy
Hyperthyroidism in pregnancy affects about 0.2% of pregnancies with Graves’ disease
being the most common cause, followed by gestational hyperthyroidism. During
pregnancy, the treatment options for hyperthyroidism ar e limited to antithyroid
medication and surgery. Radioactive iodine is contraindicated during pregnancy. During early pregnancy, propylthiouracil should be used, as carbimazole has a risk of
teratogenic effects. After the first trimester, the use of carbim azole can resume for the
remainder of the pregnancy, as propylthiouracil is associated with hepatotoxicity.
Surgery in pregnancy is reserved for cases of malignant disease where there is
uncontrolled hyperthyroidism or airway compromise. Otherwise, it is g enerally advised
for the operation to be postponed until after delivery due to the risk of general
anaesthesia on the fetus.
A 34-year-old woman has her blood pressure checked by her GP and it is found to be
180/100 mmHg (normal value <120/80 mmHg). She is diagnosed with a
phaeochromocytoma causing secondary hypertension.
Which one of the following is most likely regarding this patient’s
phaeochromocytoma?
1) It is usually bilateral
2) It is usually extra-adrenal
3) It is usually malignant
4) It may be screened for by urinary catecholamines
5) It is best treated medically
Explanation
It may be screened for by urinary catecholamines
Phaeochromocytoma is a rare neuroendocrine tumour, arising in the adre nals in 90% of
the cases, producing an excess amount of catecholamines. In patients where a
phaeochromocytoma is suspected, quantifying urinary total catecholamines
and metanephrines in a 24-hour urine sample collection, has the highest sensitivity and
specificity. Assessing the level of vanillylmandelic acid in a 24 -hour urine sample is not
as accurate.
It is usually bilateral
Phaeochromocytoma follows the rule of 10s. Only 10% of cases are bilateral.
It is usually extra-adrenal
Phaeochromocytoma follows the rule of 10s. Only 10% of cases are extra-adrenal and
have been described in the thorax, neck, bladder, kidney and scrotum.
It is usually malignant
Phaeochromocytoma follows the rule of 10s. Only 10% of cases are malignant.
It is best treated medically
There is no effective medical treatment for phaeochromocytoma. The patient is
adequately prepared pre-operatively with a-blockers and then b-blockers before
surgical removal of the phaeochromocytoma.
A 45-year-old man is being screened for secondary hypertension. He complains of muscle weakness and his blood results show hypernatraemia, hypokalaemia and
metabolic
alkalosis.
What is the most likely diagnosis?
1) Addison’s disease
2) Coarctation of the aorta
3) Conn syndrome
4) Incidental findings in patient with essential hypertension
5) Phaeochromocytoma
Explanation
Conn syndrome
The patient shows signs of Conn syndrome, with secondary hypertension,
hypernatraemia, hypokalaemia and a metabolic alkalosis. Conn syndrome is
primary hyperaldosteronism, ie excess secretion of aldosterone. Aldosterone is
produced in the zona glomerulosa of the cortex of the adrenals. It is a mineralocorticoid
hormone that acts on the mineralocorticoid receptors, found mainly in the distal
convoluted tubule and collecting ducts of the nephron, activating the basolateral
Na + /K + ion channels to reabsorb sodium and water and excrete potassium. It increases
the permeability of the collecting duct to sodium and stimulates the secretion of H + ions
into the urine. Through conservation of sodium and water, aldosterone increases blood
pressure. In hyperaldosteronism, there is excessive release of aldosterone, typically
from either adrenal hyperplasia or an adenoma. Conn syndrome most commonly affects
women, with a female to male ratio of 2:1, aged 30–50. Presenting features of Conn
syndrome include: hypertension, abdominal distension, weakness, ileus or features
related to complications of hypertension such as headaches, proteinuria. Treatment
incudes spironolactone (an aldosterone antagonist) or surgery with adrenalectomy.
Addison’s disease
Addison’s disease is the result of adrenocortical insufficiency and may present with
hypotension. It is associated with biochemical disturbances which include
hyponatraemia, hyperkalaemia and hypercalcaemia.
Coarctation of the aorta
Coarctation of the aorta is a known cause of secondary hypertension but does not lead
to the biochemical disturbances evident in the above scenario.
Incidental findings in patient with essential hypertension
Essential hypertension does not have a known cause and should not be associated with
biochemical abnormalities of hypernatraemia, hypokalaemia and metabolic alkalosis.
Phaeochromocytoma Phaeochromocytoma is a known cause of secondary hypertension, but does not lead to
the biochemical abnormalities evident in the scenario.
A 68-year-old man presents to his GP with weight gain, bruising and generalised weakness and is diagnosed with Cushing syndrome. He is found to be hyperglycaemic and serum adrenocorticotropic hormone (ACTH) levels are very high Overnight high-dose dexamethasone suppression test shows a raised morning cortisol level.
Which one of the following is most likely to be found?
1) Adrenocortical adenoma
2) Adrenocortical carcinoma
3) Basophil pituitary adenoma
4) Small-cell bronchial carcinoma
5) Bronchial carcinoid
Explanation
Small-cell bronchial carcinoma
The patient presents with signs relating to Cushing syndrome, characterised by the excess production of cortisol in the body. The raised ACTH excludes an adrenal cause. The high-dose overnight dexamethasone suppression test is used to differentiate ACTH -dependent causes of Cushing syndrome. If the cause is a pituitar y adenoma then high-dose dexamethasone suppresses the production of ACTH from the adenoma cells and therefore causes a low morning cortisol.
As the cortisol level remained unaffected, the source of ACTH is not the pituitary and is therefore an ectopic sour ce. From the options available, small-cell bronchial carcinoma, accounting for 15% of all primary lung tumours, is the most common cause of ectopic ACTH production. This is a paraneoplastic syndrome that can occur with the following tumours: small -cell lung tumours, bronchial carcinoid tumours, islet-cell tumours of the pancreas, medullary carcinoma of the thyroid and thymic tumours. Signs of Cushing syndrome are summarised in the table below:
Head and neck
Trunk
Limbs
Frontal balding
Kyphosis
Proximal myopathy
Moon face
Buffalo hump
Pathological fractures
Acne
Gynaecomastia in men
Ankle oedema (salt and water retention due to excess cortisol)
Plethoric complexion
Central obesity (due to altered fat distribution) Hirsutism
Purple striae on abdomen, breasts, thighs
Pigmentation in ACTH-dependent cases, in areas exposed to sunlight
Thin skin
Hair growth of forearms in particular
Tissue wasting
Predisposition to infection
Bad wound healing
Adrenocortical adenoma
When Cushing syndrome is the result of an adrenal cause, such as an adrenocortical adenoma or an adrenocortical carcinoma, the excess circulating cortisol suppresses the hypothalamus–pituitary axis and leads to a low corticotrophin releasing hormone (CRH) and ACTH. In this scenario ACTH is high as is morning cortisol.
Adrenocortical carcinoma
Where Cushing syndrome is the result of an adrenal cause, such as an adrenocortical adenoma or an adrenocortical carcinoma, the excess circulating cortisol suppresses the hypothalamus–pituitary axis and leads to a low CRH and ACTH. In this scenario ACTH is high as is morning cortisol.
Basophil pituitary adenoma
Cushing’s disease is caused by a pituitary adenoma, arising in either the basophil or chromophobe cells. However, in this scenario the cause of the patient’s presentation is not a pituitary adenoma. In the case of a pituitary adenoma, cortisol level should be suppressed, following a high-dose overnight dexamethasone suppression test.
Bronchial carcinoid
Both bronchial carcinoid and small-cell bronchial carcinoma can produce a Cushing syndrome by ectopic secretion of ACTH. Small -cell bronchial carcinoma is more common that bronchial carcinoid. Bronchial carcinoid is a rare entity, accounting for 1–6% of primary lung tumours and tends to present between the 4th and 5th decade of life.
A 43-year-old woman has a history of medullary thyroid carcinoma and parathyroid hyperplasia. She is concerned about symptoms to be aware of in her children. Which other condition would be associated with multiple endocrine neoplasia type 2A (MEN2A)?
1) Pituitary adenoma
2) Gastrinoma
3) Marfanoid habitus
4) Neurofibromatosis
5) Phaeochromocytoma
Explanation
Phaeochromocytoma
Phaeochromocytoma is found in patients with both MEN2A and MEN2B syndromes. Patients with MEN2A have the following combination of conditions: medullary thyroid carcinoma, phaeochromocytoma and hyperparathyroidism (parathyroid hyperplasia).
MEN2 is an autosomal-dominant condition associated with the RET proto-oncogene. There is also a third variation of MEN2 called familial MEN2, presenting with a medullary thyroid carcinoma and no other manifestations. MEN2A (Sipple syndrome):
type 2 = secondary = Sipple.
Pituitary adenoma
Pituitary adenomas are associated with multiple endocrine neoplasia type 1. This condition is described as the combination of: parathyroid hyperplasia, pancreatic islet - cell tumours and a pituitary adenoma.
Gastrinoma
A gastrinoma is a gastrin-secreting tumour most commonly found in the duodenum or pancreas and can lead to Zollinger–Ellison syndrome – peptic ulceration because of excess. Up to 50% of patients with Zollinger–Ellison syndrome are found to have MEN1.
Marfanoid habitus
Marfanoid habitus is seen in patients with multiple endocrine neoplasia type 2B. This condition describes the combination of medullary thyroid carcinoma, phaeochromocytoma, mucosal neuromas and Marfanoid body habitus.
Neurofibromatosis
Neurofibromatosis is not part of multiple endocrine neoplasia.
A 26-year-old woman presents to the pre-assessment clinic with elevated serum calcium levels. She tells you she has familial hypocalciuric hypercalcaemia. Which one of the following statements is correct regarding familial hypocalciuric hypercalcaemia?
1) Hypermagnesaemia may be seen
2) It is autosomal-recessive
3) It is associated with increased urinary calcium excretion – more than 200 mg per 24 h
4) It is associated with low parathyroid hormone (PTH) levels
5) The condition responds to parathyroidectomy
Explanation
Hypermagnesaemia may be seen
Familial hypocalciuric hypercalcaemia (FHH) is a rare autosomal -dominant condition with high penetrance, associated with loss-of-function mutations in the calcium sensing receptor (CaSR), a G-coupled protein receptor which is found in the kidney and parathyroid tissue. In the parathyroid gland its function is to sense serum calcium levels and regulate parathyroid hormone (PTH) release, whereas in the kidney it inhibits reabsorption of calcium. In FHH, there is loss of the negative feedback system on PTH release when serum calcium is elevated due to a reduction in the receptor’s sensitivity, leading to sustained mild hypercalcaemia. In the kidney there is reduced excretion leading to hypocalciuria. In most cases there is also hypermagn esaemia. Patients are usually asymptomatic. FHH is confirmed with genetic testing.
It is autosomal-recessive
Familial hypocalciuric hypercalcaemia is inherited in an autosomal -dominant fashion with a high penetrance.
It is associated with increased urinary calcium excretion – more than 200 mg per 24 h Familial hypocalciuric hypercalcaemia is characterised by raised serum calcium levels, low urinary calcium levels, normal or slightly elevated parathyroid hormone level and commonly associated with hypermagnesaemia.
It is associated with low parathyroid hormone (PTH) levels Familial hypocalciuric hypercalcaemia is characterised by normal or slightly raised levels of parathyroid hormone.
The condition responds to parathyroidectomy
In patients that are asymptomatic, treatment is not necessary. Familial hypocalciuric hypercalcaemia should not be treated with parathyroidectomy and does not respond to diuretic or bisphosphonate treatment. Calcimimetics have been used for symptomatic cases as well as pamidronate. Parathyroidectomy is reserved for patients with recurrent pancreatitis.
A 52-year-old woman with longstanding rheumatoid arthritis is seen in a pre-assessment clinic before a planned left total knee replacement. On examination, she is noted to be
overweight and struggles to rise from a chair. She is noted to be on long-term steroids
for rheumatoid arthritis and is found to have a high blood pressure ,raised fasting plasma glucose and striae seen in her skin.
Which one of the following conditions is she most likely to have developed secondary to her medication?
1) Addison’s disease
2) Congenital adrenal hyperplasia
3) Conn syndrome
4) Cushing syndrome
5) Phaeochromocytoma
Explanation
Cushing syndrome
Cushing syndrome is a condition related to excess cortisol. The commonest cause is
iatrogenic, resulting from exogenous administration of glucocorticoid medication for
chronic conditions such as asthma, chronic obstructive pulmonary disease (COPD),
arthritis etc. Endogenous causes of Cushing inclu de: a pituitary adenoma producing an
excess of adrenocorticotropic hormone (ACTH), adrenocortical adenoma or carcinoma
and a functional tumour producing ectopic adrenocorticotropic hormone (ACTH) such
as an oat-cell lung carcinoma. Cushing syndrome relates to a collection of signs and
symptoms that occur as the result of excess cortisol. These are summarised in the table
below:
Head and neck
Trunk
Limbs
Frontal balding
Kyphosis
Proximal myopathy
Moon face
Buffalo hump
Pathological features
Acne
Gynaecomastia in men
Ankle oedema (salt and water retention due to excess cortisol)
Plethoric complexion
Central obesity (due to altered fat distribution)
Hirsutism
Purple striae on abdomen, breasts, thighs
Pigmentation in ACTH-dependent cases, in areas exposed to sunlight
Thin skin
Hair growth of forearms in particular
Tissue wasting
Predisposition to infection
Bad wound healing
Addison’s disease
Addison’s disease is the result of adrenocortical insufficiency and can present with hypotension. It is associated with biochemical disturbances as well: hyponatraemia,
hyperkalaemia and hypercalcaemia.
Congenital adrenal hyperplasia
Congenital adrenal hyperplasia is an autosomal-recessive condition associated with
deficiency in the enzyme 21α-hydroxylase, involved in the synthesis of cortisol and
aldosterone. It tends to present at birth or early infancy.
Conn syndrome
Conn syndrome is a condition associated with hyperaldosteronism. It is mineralocorticoid and not glucocorticoid excess. Even though patients with Conn syndrome present with hypertension, the remaining signs described in the scenario above relate to glucocorticoid excess. Patients with Conn syndrome present with hypernatraemia and hypokalaemia.
Phaeochromocytoma
Phaeochromocytoma is a rare neuroendocrine tumour, arising in the chromaffin cells of
the adrenals in 90% of the cases, producing an excess amount of catecholamines. Even
though it is associated with hypertension, the remaining signs described in the scenario
relate to corticosteroid excess. Patients with phaeochromocytoma present with severe
hypertension associated with headaches, palpitations and diaphoresis.
A 35-year-old woman is found at her preoperative assessment to be
tachycardic
, with a
heart rate of 120 beats/min, extremely
anxious
and to have a smoothly
enlarged
goitre
in her neck. A diagnosis of
Graves’ disease
is confirmed.
What are her blood tests likely to show?
1) Low thyroxine; which regulates carbohydrate metabolism
2) Raised thyroxine; secreted by the colloid cells of the thyroid gland
3) Raised thyroxine level; mostly bound to albumin in the blood
4) Raised thyroxine-stimulating hormone (TSH) from the posterior pituitary
5) Thyroid-stimulating hormone receptor immunoglobulins
Explanation
Thyroid-stimulating hormone receptor immunoglobulins
Graves’ disease is a form of hyperthyroidism of autoimmune origin. It is associated with
the presence of circulating TSH receptor immunoglobulins (antibodies), that bind to the
TSH receptor in the thyroid. These antibodies mimic the function of TSH, leading to
stimulation of the thyroid gland to produce more triiodothyronine (T3) and thyroxine
(T4). The elevated serum-circulating hormones activate the negative feedback
mechanism and suppress release of TSH from the anterior pituitary gland.
Low thyroxine; which regulates carbohydrate metabolism
Thyroxine (T4) is the hormone produced from the thyroid gland. Graves’ disease is a
state of hyperthyroidism. Therefore, serum thyroxine level s would be
raised. Thyroxine stimulates lipid and carbohydrate metabolism.
Raised thyroxine; secreted by the colloid cells of the thyroid gland
Raised thyroxine is found in patients with Graves’ disease as this is a form of
hyperthyroidism, however thyroxine is secreted by the follicular and not the colloid
cells of the thyroid gland.
Raised thyroxine level; mostly bound to albumin in the blood
In overt hyperthyroidism there is a raised free (unbound) thyroxine level (free T4).
Raised thyroxine-stimulating hormone (TSH) from the posterior pituitary
TSH is secreted from the anterior pituitary. However, the cause of Graves’ disease is not
raised TSH but the presence of TSH receptor immunoglobulins, that mimic the function
of TSH by binding to the TSH receptor and stimulating the thyroid gland to release
hormone molecules. TSH is suppressed via a negative feedback mechanism and is
therefore low in patients with Graves’ disease.
A 45-year-old woman presents with generalised weakness and fatigue. She has a history of renal colic. Her calcium is found to be raised at 3.1. An inferior parathyroid adenoma
is suspected.
Which one of the following statements is most likely to be true?
1) This parathyroid gland would have developed fro m the second branchial pouch
2) This would be an unusual case, as the majority of primary hyperparathyroidism
is due to diffuse hypertrophy of the parathyroids
3) The adenoma could be localised by a combination of ultrasound and sestamibi
radionucleotide scan
4) This woman’s weakness and fatigue are incidental to the hypercalcaemia
5) Parathyroid tissue cannot be implanted elsewhere in the body after total
parathyroid excision
Explanation
The adenoma could be localised by a combination of ultrasound and sestamibi
radionucleotide scan
Pre-operative parathyroid adenoma localisation can be done by either ultrasound,
sestamibi (methoxyisobutylisonitrile (MIBI), uses technetium) radionucleotide scan or
technetium/thallium scan. The inferior parathyroids are much more variable in their
position.
This parathyroid gland would have developed from the second branchial pouch
The superior and inferior parathyroids develop from the fourth and third branchial
pouches, respectively.
This would be an unusual case, as the majority o f primary hyperparathyroidism is due
to diffuse hypertrophy of the parathyroids
Primary hyperparathyroidism is usually due to a solitary secreting parathyroid
adenoma. Hyperparathyroidism presents as:
‘bones’ (arthralgia and osteoporosis)
‘stones’ (renal)
abdominal ‘groans’ (pancreatitis, peptic ulcers and constipation)
‘moans’ (depression, fatigue and weakness).
This woman’s weakness and fatigue are incidental to the hypercalcaemia
This woman’s weakness and fatigue are almost definitely as a result of her
hypercalcaemia. Parathyroid tissue cannot be implanted elsewhere in the body after total parathyroid
excision
Tertiary hyperparathyroidism occurs in renal failure, in which the parathyroids
function autonomously. In such cases, subcutaneous reimplantation of half a
parathyroid in the forearm allows easy access if further resection is required.
A 55-year-old woman with
poorly-controlled
polymyalgia rheumatica (PMR) complains
of weight gain, muscle weakness and easy bruising. She is found to have increased
abdominal girth, striae, bruising and raised serum glucose levels. A diagnosis of
Cushing’s syndrome is made.
What is most likely to be the cause of her Cushing syndrome?
1) Iatrogenic steroids
2) Medullary carcinoma of the thyroid
3) Pituitary tumour
4) Lung tumour secreting adrenocorticotropic hormone (ACTH)
5) Polymyalgia rheumatica
Explanation
Iatrogenic steroids
This woman has Cushing syndrome and the commonest cause of this is iatrogenic
through the administration of steroids. In this case, this woman has poorly controlled
polymyalgia rheumatica (PMR) and is likely to have been on prolonged steroid
treatment. Endogenous cases are caused by primar y pituitary disease ie pituitary
adenoma (70%, referred to as Cushing’s disease), primary adrenal tumours (15%) and
ectopic adrenocorticotropic hormone (ACTH)-secreting tumours (15%).
The diagnosis of Cushing syndrome depends on the loss of normal circadia n rhythm as
well as persistent elevation of cortisol. It is necessary to take at least three serum
samples to demonstrate the loss of rhythm. The mean 24 -h cortisol levels can be
estimated by collecting and measuring the 24-h urinary free cortisol, which gives an
integrated measure of cortisol production. As a screening test it has 95% specificity.
Medullary carcinoma of the thyroid
Medullary carcinomas of the thyroid can rarely produce ectopic adrenocorticotropic
hormone (ACTH), however iatrogenic administration of steroids is more likely in this
case given the history of PMR.
Pituitary tumour
Pituitary tumours can produce adrenocorticotropic hormone (ACTH), however, the
history of polymyalgia rheumatica (PMR) means iatrogenic steroid administration is
more likely.
Lung tumour secreting adrenocorticotropic hormone (ACTH)
ACTH secreting tumours, of which bronchial carcinoma is the most common, can cause
Cushing syndrome, however there is no history indicative of lung cancer.
Polymyalgia rheumatica
Administration of steroids to treat polymyalgia rheumatica (PMR), not PMR per se,
causes Cushing syndrome.
A 65-year old man is diagnosed with a parathyroid
adenoma
. He attends the
Outpatient’s Clinic for advice on management options.
What is the best option for treatment?
1) Only surgery is curative
2) Bisphosphonates and calcitonin result in good long -term control
3) Octreotide results in good control of parathyroid hormone (PTH) levels
4) Surgery is reserved for those not improving on bisphosphonate treatment
5) Reducing dietary calcium intake can be curative
Explanation
Only surgery is curative
Parathyroidectomy is the only curative treatment for primary hyperparathyroidism
caused by a parathyroid adenoma.
Bisphosphonates and calcitonin result in good long-term control
Bisphosphonates, calcitonin and octreotide do not have a role in treatment.
Octreotide results in good control of parathyroid hormone (PTH) levels
Octreotide is used in nuclear imaging. Parathyroid tumours expressing somato statin
may show octreotide uptake, which may indicate a possibility for the use of
somatostatin analogues for medical treatment when surgery is not possible. Octreotide
does not have a role in the treatment of primary hyperparathyroidism.
Surgery is reserved for those not improving on bisphosphonate treatment
Surgery is the only curative treatment of primary hyperparathyroidism secondary to a
parathyroid adenoma. Bisphosphonates do not have a role in treatment of primary
hyperparathyroidism.
Reducing dietary calcium intake can be curative
Low calcium intake stimulates the parathyroids and high intake accentuates
hypercalcaemia, so the recommendation is moderate intake followed up with curative
surgery by removal of the parathyroid glands.
A 48-year old woman is found to have incidental deranged biochemistry. She is
subsequently diagnosed with
primary hyperparathyroidism
.
Which one of the following is likely to be correct?
1) She will also have diabetes mellitus
2) She will probably be asymptomatic
3) She will have reduced parathyroid hormone (PTH) levels
4) She will have a raised phosphate
5) She will have MEN2b syndrome
Explanation
She will probably be asymptomatic
Asymptomatic primary hyperparathyroidism is found in greater than 50 % of patients.
In 85% of cases, it is caused by a single parathyroid adenoma, but can be due to multiple
gland adenomas/hyperplasia and rarely even parathyroid carcinoma.
She will also have diabetes mellitus
Patients with primary hyperparathyroidism are very slightly more likely to also have
diabetes and vice versa. It is not certain therefore that this patient would have diabetes.
She will have reduced parathyroid hormone (PTH) levels
Patients will have a raised serum calcium and parathyroid hormone and a reduced
phosphate (secondary to renal excretion, causing phosphaturia).
She will have a raised phosphate
As mentioned, the patient will have a low phosphate due to renal excretion of phosphate
in response to raised serum calcium and parathyroid hormone.
She will have MEN2b syndrome
Hyperparathyroidism is found in MEN 1 and MEN 2a syndromes, but not in MEN 2b.
