Wednesday [16/09/2021] Flashcards
A newborn child is assessed. They are found to be in the 25th centile for their weight along with a systolic murmur heard best over the back. When feeling the femoral pulses the doctor notices that there is a radio-femoral delay. Which of the following may be causing these examination findings?
Turner’s syndrome
Radio-femoral delay is associated with coarctation of the aorta
This question is asking about a neonate presenting with a systolic murmur, low birth weight and radio-femoral delay, these are all characteristic features of coarctation of the aorta. Of the above conditions, only Turner’s syndrome has a strong association with coarctation of the aorta.
What is CHARGE syndrome?
CHARGE syndrome is a genetic syndrome associated with some congenital heart defects. However, these are most commonly tetralogy of Fallot or ventricular septal defects.
What is Klinefelter’s syndrome?
Klinefelter’s syndrome is a condition caused by 47, XXY (an extra X chromosome) which characteristically presents in slim tall males with infertility and lack of secondary sexual characteristics.
Characteristic of patent ductus arteriosus?
A patent ductus arteriosus is a common congenital heart defect that will not cause radio-femoral delay. The characteristically associated murmur is a venous hum, which is a continuous murmur.
Features of coarctation of aorta?
infancy: heart failure
adult: hypertension
radio-femoral delay
mid systolic murmur, maximal over back
apical click from the aortic valve
notching of the inferior border of the ribs (due to collateral vessels) is not seen in young children
Associations of coarctation of aorta
Turner’s syndrome
bicuspid aortic valve
berry aneurysms
neurofibromatosis
A 28-year-old man of African descent presents to his general practitioner with complaints of fever, reduced appetite and shortness of breath on exertion for the last 3 months.
As part of the initial workup, blood samples are taken which are significant for the following:
Calcium 2.9 mmol/L (2.1-2.6)
A chest x-ray is performed which is reported as showing bilateral hilar lymphadenopathy.
What is the most likely diagnosis?
Hypercalcaemia + bilateral hilar lymphadenopathy → ?sarcoidosis
This patient presented with symptoms of fever, reduced appetite and shortness of breath on exertion, which are suggestive of a systemic disease affecting the respiratory system. primarily. The fact that he is a young, male patient of African descent points to the diagnosis of sarcoidosis, which can present with the aforementioned symptoms and with an insidious onset (3 months).
Sarcoidosis, the correct answer, is made even more likely due to the combination of findings of hypercalcaemia and bilateral hilar lymphadenopathy, which are typical of the condition.
What would make diagnosis more likely leprosy?
Leprosy is incorrect, as lack of skin and neurological manifestations such as paresthesias makes this condition less likely in this case
Features of sarcoidosis
acute: erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthralgia
insidious: dyspnoea, non-productive cough, malaise, weight loss
skin: lupus pernio
hypercalcaemia: macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)
Which populations sarcoidsosi more common in?
Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas. It is more common in young adults and in people of African descent
Syndromes associated with sarcoidosis?
Lofgren’s syndrome is an acute form of the disease characterised by bilateral hilar lymphadenopathy (BHL), erythema nodosum, fever and polyarthralgia. It usually carries an excellent prognosis
In Mikulicz syndrome* there is enlargement of the parotid and lacrimal glands due to sarcoidosis, tuberculosis or lymphoma
Heerfordt’s syndrome (uveoparotid fever) there is parotid enlargement, fever and uveitis secondary to sarcoidosis
A 38-year-old man was admitted to the surgical receiving unit by his GP with sudden onset epigastric pain. His is a known alcoholic and is also overweight, but has no other past medical history. He has severe nausea and vomiting, unable to tolerate any food or drink.
His blood results come back as below:
Hb 154 g/L Male: (135-180)
Female: (115-160)
Platelets 290 * 109/L (150 - 400)
WBC 27.8 * 109/L (4.0 - 11.0)
Na+ 140 mmol/L (135 - 145)
K+ 3.8 mmol/L (3.5 - 5.0)
Adj Ca2+ 1.8mmol/L (2.2 - 2.6)
Urea 9.1 mmol/L (2.0 - 7.0)
Creatinine 102 µmol/L (55 - 120)
CRP 13 mg/L (< 5)
ALP 74 U/L (30-130)
ALT 27 U/L (<41)
Bilirubin 12 µmol/L (<21)
Which differential is most likely to account for his abnormal results?
Acute pancreatitis may cause hypocalcemia
Acute pancreatitis is the most likely diagnosis as it is the most likely to result in a hypocalcaemia. It can also can a raised WBC and CRP due to the inflammatory process.
Alcoholic liver disease is unlikely as the LFTs are normal and this would not account for the hypocalcaemia. It would also be unlikely to present in such an acute manner.
Peptic ulcers can also cause epigastric pain however would not account for the hypocalcaemia or such raised inflammatory markers.
Ruptured abdominal aortic aneurysm may present with sudden onset epigastric pain radiating to the back and is important to consider. However it would be accompanied by a low haemoglobin and would not normally cause hypocalcaemia.
Spontaneous bacterial peritonitis occurs as a complication of liver cirrhosis and would cause raised white cells however it would be unlikely with normal LFTs and hypocalcaemia is not a typically presentation of this.
You are asked to review a 76-year-old woman with metastatic bowel cancer. She was admitted four days prior with abdominal pain and has not opened her bowels for the last six days.
She is receiving diamorphine via a syringe driver. However, she is still having intermittent severe abdominal pain.
Which of the following medications should be added to her syringe driver?
Syringe drivers: respiratory secretions & bowel colic may be treated by hyoscine hydrobromide, hyoscine butylbromide, or glycopyrronium bromide
This patient is experiencing colicky pain secondary to mechanical obstruction caused by bowel cancer.
Hyoscine butylbromide (also known as scopolamine butylbromide) is the correct answer in this case. It is an antimuscarinic drug that reduces smooth muscle contractions. It is therefore useful in the treatment of colicky pain.
Metoclopramide is incorrect as it is a prokinetic antiemetic. It will therefore worsen the pain by promoting bowel contraction against the obstruction.
Codeine phosphate is incorrect as the patient is already receiving diamorphine via her syringe driver. Further analgesia should be added by increasing the dose of diamorphine, not adding another opioid.
Midazolam is incorrect as it is a sedative which would not address the underlying cause of her symptoms.
Gabapentin is incorrect at it is not indicated for the treatment of pain due to gastrointestinal obstruction.
What type of drug is metoclopramide and what will it worsen?
Metoclopramide is incorrect as it is a prokinetic antiemetic. It will therefore worsen the pain by promoting bowel contraction against the obstruction.
When are syringe drivers considered?
A syringe driver should be considered in the palliative care setting when a patient is unable to take oral medication due to nausea, dysphagia, intestinal obstruction, weakness or coma. In the UK there are two main types of syringe driver: Graseby MS16A (blue): the delivery rate is given in mm per hour Graseby MS26 (green): the delivery rate is given in mm per 24 hours
Which syringe driver drugs should you give with sodium chloride rather than water?
The majority of drugs are compatible with water for injection although for the following drugs sodium chloride 0.9% is recommended:
granisetron
ketamine
ketorolac
octreotide
ondansetron
Commonly used drugs for N and V syringe driver?
nausea and vomiting: cyclizine, levomepromazine, haloperidol, metoclopramide
Commonly used drugs for respiratory secretions/bowel colic syringe driver?
hyoscine hydrobromide, hyoscine butylbromide, or glycopyrronium bromide.
Commonly used drugs for agitation/restlessness syringe driver?
midazolam, haloperidol, levomepromazine
Commonly used drugs for pain syringe driver?
diamorphine is the preferred opioid
Mixing and compatibility issues with syringe drivers drugs?
diamorphine is compatible with the majority of other drugs used including cyclizine*, dexamethasone, haloperidol, hyoscine butylbromide, hyoscine hydrobromide, levomepromazine, metoclopramide, midazolam
cyclizine is incompatible with a number of drugs including clonidine, dexamethasone, hyoscine butylbromide (occasional), ketamine, ketorolac, metoclopramide, midazolam, octreotide, sodium chloride 0.9%
*precipitation may be seen at higher doses
A 61-year-old woman attends general practice with her daughter, who believes her mum has been looking ‘yellow’ recently. On observation, the patient is visibly jaundiced and her abdomen is distended.
On questioning, the patient describes feeling increasingly bloated over the past month and has found ‘small red dots’ appearing on her upper chest, these disappear when pressed on, and subsequently, refill from the centre. She is uncertain if she has lost weight but she does describe her clothes seeming baggier over the past few months.
She has a background of type 2 diabetes, hypertension, and liver cirrhosis secondary to chronic hepatitis B. She admits to missing several follow up appointments with gastroenterology over the past couple of years.
What is the most likely cause of the patient’s deterioration?
Hepatocellular carcinoma
Deterioration in patient with hepatitis B - ? hepatocellular carcinoma
Hepatocellular carcinoma (HCC) is correct. The patient is presenting with decompensated liver disease. HCC is a known cause of this. Further, hepatitis B is known to be a risk factor for hepatocellular carcinoma, and the patient’s history of probable weight loss, anorexia, and missing follow up appointments fits this picture
How often should patients Dx with cirrhosis have surviellance?
Patients diagnosed with cirrhosis should have surveillance at six-monthly intervals for HCC consisting of abdominal ultrasound and measuring AFP levels
What is hepatitis B a risk factor for?
Further, hepatitis B is known to be a risk factor for hepatocellular carcinoma, and the patient’s history of probable weight loss, anorexia, and missing follow up appointments fits this picture
Mx options for patients Dx with liver cirrhosis
Management options include surgical resection in early stages, radiofrequency ablation, transarterial chemoembolization multikinase inhibitors such as sorafenib, and liver transplantation.
Difference between compensated and decompensated liver disease?
Compensated: When you don’t have any symptoms of the disease, you’re considered to have compensated cirrhosis.
Decompensated: When your cirrhosis has progressed to the point that the liver is having trouble functioning and you start having symptoms of the disease, you’re considered to have decompensated cirrhosis.
Signs of decompensated liver disease
fatigue
easy bruising and bleeding
itching
yellowing of the skin and eyes (jaundice)
fluid build-up in the abdomen (ascites)
fluid build-up in the ankles and legs
abdominal pain
nausea
fever
brownish or orange urine
loss of appetite or weight loss
confusion, memory loss, or insomnia (hepatic encephalopathy)
Signs of hepatitis D infectino
Hepatitis D infection is incorrect, as although it can cause a decompensated liver failure picture, there are no signs pointing to this in the clinical scenario. This would typically present with features of acute hepatitis such as fever, nausea and vomiting, abdominal pain, jaundice, dark urine and pale stools, but rarely progresses to chronic hepatitis.
What can hepatitis D in someone with heptaitis B cause?
Fulminant hepatitis is a rare syndrome of massive necrosis of the liver parenchyma. This can be due to infection with certain hepatitis viruses e.g. hepatitis D co-infection in someone with hepatitis B. Hepatitis A can also cause fulminant hepatitis, although this is more rare. Other causes include toxic agents or drug-induced injury e.g. with acetaminophen. This presents with rapid deterioration, including coagulopathy due to liver failure, disseminated intravascular coagulation, and hepatorenal syndrome. This may progress to coma and cerebral oedema over a period of several days to weeks.
What does pancreatic adenocarconma classical present as?
Pancreatic adenocarcinoma classically presents with painless jaundice. Although, it is common also to present in non-specific ways such as with anorexia, weight loss, epigastric pain, atypical back pain. There may also be loss of exocrine function e.g steatorrhoea and loss of endocrine function e.g diabetes mellitus. Some features of this fit with the clinical presentation, such as jaundice, weight loss and anorexia, however, the other signs of hepatic decompensation do not fit.
What is cholangiocarcinoma?
Bile duct cancer, also called cholangiocarcinoma, is a cancer that’s found anywhere in the bile ducts. The bile ducts are small tubes that connect different organs. They are part of the digestive system.
Cholangiocarnoma presentation?
Cholangiocarcinoma typically presents with persistent jaundice, biliary colic pain, Sister Mary Joseph nodes (periumbilical lymphadenopathy) and Courvoisier’s sign (a palpable mass in the right upper quadrant).
What is hepatitis B?
Hepatitis B is a double-stranded DNA hepadnavirus and is spread through exposure to infected blood or body fluids, including vertical transmission from mother to child.
Incubation period for hepatitis B
The incubation period is 6-20 weeks.
Features of hepatitis B
The features of hepatitis B include fever, jaundice and elevated liver transaminases.
Cx of hepatitis B infection
chronic hepatitis (5-10%). ‘Ground-glass’ hepatocytes may be seen on light microscopy
fulminant liver failure (1%)
hepatocellular carcinoma
glomerulonephritis
polyarteritis nodosa
cryoglobulinaemia
Who should be immunised against hepatitis B?
Immunisation against hepatitis B (please see the Greenbook link for more details)
children born in the UK are now vaccinated as part of the routine immunisation schedule. This is given at 2, 3 and 4 months of age
at risk groups who should be vaccinated include: healthcare workers, intravenous drug users, sex workers, close family contacts of an individual with hepatitis B, individuals receiving blood transfusions regularly, chronic kidney disease patients who may soon require renal replacement therapy, prisoners, chronic liver disease patients
contains HBsAg adsorbed onto aluminium hydroxide adjuvant and is prepared from yeast cells using recombinant DNA technology
around 10-15% of adults fail to respond or respond poorly to 3 doses of the vaccine. Risk factors include age over 40 years, obesity, smoking, alcohol excess and immunosuppression
testing for anti-HBs is only recommended for those at risk of occupational exposure (i.e. Healthcare workers) and patients with chronic kidney disease. In these patients anti-HBs levels should be checked 1-4 months after primary immunisation
the table below shows how to interpret anti-HBs levels:
How to interpret anti-HB levels?
Anti-HBs level (mIU/ml) Response
> 100 Indicates adequate response, no further testing required. Should still receive booster at 5 years
10 - 100 Suboptimal response - one additional vaccine dose should be given. If immunocompetent no further testing is required
< 10 Non-responder. Test for current or past infection. Give further vaccine course (i.e. 3 doses again) with testing following. If still fails to respond then HBIG would be required for protection if exposed to the virus
Mx of hepatitis B
pegylated interferon-alpha used to be the only treatment available. It reduces viral replication in up to 30% of chronic carriers. A better response is predicted by being female, < 50 years old, low HBV DNA levels, non-Asian, HIV negative, high degree of inflammation on liver biopsy
whilst NICE still advocate the use of pegylated interferon firstl-line other antiviral medications are increasingly used with an aim to suppress viral replication (not in a dissimilar way to treating HIV patients)
examples include tenofovir, entecavir and telbivudine (a synthetic thymidine nucleoside analogue)
On a ward round you see a 70-year-old female who’s recently had a resection of her bowel for colon cancer, and has been bed bound for several days. She complains of a sore, red calf, and a feeling of breathlessness. A pulmonary embolism is suspected, and a CT-pulmonary angiogram (CTPA) is ordered, however it comes back negative for a pulmonary embolism (PE).
What is the next most appropriate action to aid diagnosis?
Perform a proximal leg vein doppler ultrasound:
- Investigating suspected PE: if the CTPA is negative then consider a proximal leg vein ultrasound scan if DVT is suspected.
Perform a ventilation-perfusion (V/Q) scan is incorrect because it would not be completed before a leg vein ultrasound. In diagnosing a PE, a ventilation perfusion (V/Q) scan can be performed after a negative CTPA in certain clinical circumstances, though this is rare as CTPA is considered the gold standard. However a proximal leg vein doppler should be performed first (it is faster, cheaper, and exposes the patient to less radiation).
Proximal leg vein CT-venogram is incorrect, as it is not used for diagnosing DVT, and is mostly reserved for research due to the primary role of ultrasound as a faster, cheaper, radiation-free alternative.
Perform an emergency electrocardiogram (ECG) is incorrect, because while it is appropriate to do an ECG, this would not be diagnostic of a PE and would not diagnose the painful, erythematous calf. However, an ECG would invariably be done to rule out certain dyspnoea differentials, if all else came back negative.
Repeat CTPA after 24 hours is incorrect because CTPA is very unlikely to change after 24 hours, and would also re-expose the patient to significant amounts of radiation.
While an ECG will undoubtedly be performed in such a scenario, PE can not be diagnosed on ECG and must be further investigated, usually with doppler ultrasound and/or a CTPA.
How often is it that patients present iwhr textbook triad Sx?
We know from experience that few patients (around 10%) present with the textbook triad of pleuritic chest pain, dyspnoea and haemoptysis. Pulmonary embolism can be difficult to diagnose as it can present with virtually any cardiorespiratory symptom/sign depending on its location and size.
Studies suggest actually which features more likely for a PE?
The PIOPED study1 in 2007 looked at the frequency of different symptoms and signs in patients who were diagnosed with pulmonary embolism.
The relative frequency of common clinical signs is shown below:
Tachypnea (respiratory rate >20/min) - 96%
Crackles - 58%
Tachycardia (heart rate >100/min) - 44%
Fever (temperature >37.8°C) - 43%
It is interesting to note that the Well’s criteria for diagnosing a PE use tachycardia rather than tachypnoea.
All patients with symptoms or signs suggestive of a PE should have a history taken, examination performed and a chest x-ray to exclude other pathology.
PE rule out criteria? [PERC]
NICE updated their guidelines on the investigation and management of venous thromboembolism (VTE) in 2020. One of the key changes was the use of the pulmonary embolism rule-out criteria (the PERC rule)
a copy of criteria can be found in the image below
all the criteria must be absent to have negative PERC result, i.e. rule-out PE
this should be done when you think there is a low pre-test probability of PE, but want more reassurance that it isn’t the diagnosis
this low probability is defined as < 15%, although it is clearly difficult to quantify such judgements
a negative PERC reduces the probability of PE to < 2%
if your suspicion of PE is greater than this then you should move straight to the 2-level PE Wells score, without doing a PERC
What to do if PE is likely [mrep than 4 points]?
If a PE is ‘likely’ (more than 4 points)
arrange an immediate computed tomography pulmonary angiogram (CTPA)
If there is a delay in getting the CTPA then interim therapeutic anticoagulation should be given until the scan is performed.
interim therapeutic anticoagulation used to mean giving low-molecular-weight heparin
NICE updated their guidance in 2020. They now recommend using an anticoagulant that can be continued if the result is positive.
this means normally a direct oral anticoagulant (DOAC) such as apixaban or rivaroxaban
- if the CTPA is positive then a PE is diagnosed
- if the CTPA is negative then consider a proximal leg vein ultrasound scan if DVT is suspected
types of collagen
types of hepatitis
2-level PE Wells score
How to manage PE flowchart?
What do these ECGs show?
HOCM
