100 Cases Questions II

Question 1

Which triad of blood test abnormalities are most commonly associated with multiple myeloma? (3)

Answer 1

• Anaemia
• Hypercalcaemia
• Renal failure

Question 2

Outline the pathophysiology of renal failure secondary to multiple myeloma?

Answer 2

Monoclonal proteins produced by overproliferation of plasma cells accumulate at filtration barrier in the kidney interfering with normal ultrafiltration

Question 3

Outline the components of a myeloma screen? (4)

Answer 3

• Serum electrophoresis; to detect monoclonal M-bands
• 24hr urine collection; to measure urine Bence-Jones proteins
• Serum β₂microglobulin; raised in cells undergoing excess proliferation
• Full skeletal survey; whole body radiography for lytic lesions

Question 4

Outline the complications associated with multiple myeloma? (6)

Answer 4

• Anaemia; impaired erythropoiesis
• Recurrent infections; leukopaenia (neutropaenia)
• Clotting abnormalities; thrombocytopaenia
• Renal failure
• Bone disease; lytic lesions and fractures
• Amyloidosis; myeloma, hypertension, enlarged tongue, peripheral neuropathy

Question 5

Outline the main medical emergency associated with multiple myeloma?

Answer 5

Cord compression secondary to vertebral crush fractures as a result of lytic bone lesions in the spine

Question 6

Why must vitamin B12 deficiency be corrected first in patients who have a combined vitamin B12 and folate deficiency?

Answer 6

Because correction of folate first will stimulate the erythropoeisis in the bone marrow thus further depleting B12 stores and increasing the risk of subacute degeneration of the spinal cord

Question 7

Which group of conditions are vitally important to rule out in patients presenting with lethargy and pancytopaenia?

Answer 7

Acute Leukaemias<br></br>- Acute lymphoblastic leukaemia (ALL)<br></br>- Acute myeloid leukaemia (AML)

Question 8

Outline the causes of macrocytic pancytopaenia? (6)

Answer 8

• Acute leukaemias; due to increased blast cell presence
• Severe nutrient deficiencies; B12 and folate
• Cytotoxic medications
• Viral infections; HIV and EBV
• Endocrine abnormalities; myxoedema (severe hypothyroidism)
• Autoimmune disease; systemic lupus erythematosus (SLE)

Question 9

Which single test can be used to exclude acute leukaemias as a cause of pancytopaenia with macrocytosis?

Answer 9

Blood film; to look for presence of blast cells

Question 10

What is the most common cause of macrocytic anaemia?

Answer 10

Vitamin B12/folate deficiecies

Question 11

Outline the most common cause of a retroperitoneal mass?

Answer 11

Enlarged retroperitoneal lymph nodes; secondary to infection or malignancy

Question 12

What is the most common cause of retroperitoneal lymph node enlargement, how does this differ in young and older patients? (2)

Answer 12

Malignancy

• Young patients; metastases from solid organs draining to para-aortic nodes
• Older patients; haematological malignancies most likely high-grade lymphoma

Question 13

What is the most common infection associated with retroperitoneal lymph node enlargement?

Answer 13

Tuberculosis

Question 14

Which single blood test can be used as marker of high cell turnover in malignancy?

Answer 14

Serum lactate dehydrogenase (LDH)

Question 15

What is the most common type of Hodgkin lymphoma (HL)?

Answer 15

Nodular sclerosis; accounts for around 70% of casess

Question 16

What is the most common type of non-Hodgkin lymphoma (NHL)?

Answer 16

Diffuse large B cell lymphoma (DLBCL); accounts for around 20% of cases

Question 17

Outline the standard chemotherapy regimes used in the treatment of Hodgkin and non-Hodgkin lymphoma? (2)

Answer 17

• Hodgkin Lymphoma (HL); ABVD (Doxorubicin, Bleomycin, Vinblastine, Dacarbazine)<br></br>- Non-Hodgkin Lymphoma (NHL); R-CHOP (Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Vincristine, Prednisolone)

Question 18

Outline the most common chromosomal abnormality associated with Burkitt’s (Non-Hogdkin) lymphoma?

Answer 18

t(8;14)(q24;q32); resutling in c-myc expression under control of IGH promoter

Question 19

Outline the most pathogenic event associated with diffuse large B cell lymphoma (DLBCL)?

Answer 19

Dyregulation of BCL-6 transcriptional repressor which leads to stimulation of B-cell differentiation in germinal centres

Question 20

What type of hypersensitivity reaction is a haemolytic transfusion reaction?

Answer 20

Type II Antibody-mediated (IgM or IgG) cytotoxic

Question 21

Which immunoglobulin isotype are those generated against A and B blood groups and those against Rhesus D antigens? (2)

Answer 21

• A/B antibodies; IgM<br></br>- Rhesus D antibodies; IgG

Question 22

What is the difference in the haemolysis triggered by ABO antibodies and that triggered by Rhesus D antibodies? (2)

Answer 22

• ABO antibodies; intravascular red cell lysis<br></br>- Rhesus D antibodies; extravascular red cell lysis in reticuloendothelial system

Question 23

Outline the features of an acute haemolytic transfusion reaction? (4)

Answer 23

• Fever
• Abdominal pain
• Hypotension
• Haemoglobuinuria

Question 24

How can haemoglobinuria be differentiated from haematuria?

Answer 24

Centrifugation of the urine

• Haematuria; RBCs will settle at the bottom of the sample which will return to normal urine colouration
• Haemoglobinuria; urine will remain red and no red cells will collected at the bottom of the sample

Question 25

Outline the two key differentials for a patient who remains pyrexial despite appropriate antimicrobial therapy? (2)

Answer 25

- Antimicrobial Resistence 
- Abscess/Collection

Question 26

Outline the secondary causes of hyperlipidaemia? (6)

Answer 26

- Nephrotic syndrome 
- Diabetes
- Hypothyroidism
- Alcohol 
- Cholestasis
- Antipsychotics

Question 27

Briefly outline the three main cholesterol transport pathways? (3)

Answer 27

- Exogenous Pathway; absorption of dietary lipids and cholesterol and transport from the gut to the liver
- Endogenous Pathway; transport of lipids from the liver to the peripheral tissues 
- Reverse Cholesterol Transport Pathway; transport of lipids from the peripheral tissues to the liver

Question 28

Which criteria can be used to aid in the diagnosis of familial hypercholesterolaemia? (2)

Answer 28

- Simon Broome Diagnostic Criteria (SBDC) | - Dutch Lipid Clinic Network Score (DLCNS)

Question 29

Outline the triad of features associated with haemolytic uraemic syndrome? (3)

Answer 29

- Haemolytic anaemia; normocytic anaemia with raised urea 
- Renal failure; reduced urine output 
- Thrombocytopaenia

Question 30

What is an education, health and care plan (EHCP) and when are these required? (2)

Answer 30

An education, health and care plan (EHCP) is a statement produced by a local authority that documents a child's special educational needs (SEN) and how they can be supported. These are produced following assessments by the child’s school, an educational psychologist, a doctor and other professionals.

Question 31

What contributing factors to a child's learning difficulties must be considered and rectified/optimised if possible? (7)

Answer 31

- Hearing impairments
- Visual impairments
- Chronic medical condition(s)
- Epilepsy 
- Autism spectrum disorder (ASD)
- Dyspraxia
- Negelct 

Question 32

Which assessment can be carried out to help support children with special educational needs/learning difficulties? 

Answer 32

Education, health and care plan (EHCP)

Question 33

Which diagnoses are particularly important to consider in young childrent presenting with chronic headaches?

Answer 33

Organic causes; hence threshold for scanning decreases, the younger the patient

Question 34

Which of the typical features of migraine is more common in children than adults?

Answer 34

Nausea and vomiting

Question 35

Which two organisms are most commonly associated with the development of a Bell's palsy? (2)

Answer 35

- Herpex simplex virses (HSV) | - Varicella zoster virus (VZV)

Question 36

What differentiates the facial weakness associated with a Bell's palsy compared to a stroke? (2)

Answer 36

- Bell's palsy; lower motor neurone (LMN) lesion due to oedema compressing the facial nerve in the stylomastoid foramen causing facial and forehead weakness - Stroke; upper motor neurone (UMN) lesion affecting the facial area of the primary motor cortex that only affects the lower face due to additional innervation of the forehead and eyes from the ipsilateral cerebral hemisphere

Question 37

Outline the management of Bell's palsy? (2)

Answer 37

- Prednisolone 1 mg kg^-1 OD (PO) up to 60 mg for 7 days
- Artificial tears such as hypromellose eye drops

Question 38

Outline the different aetiologies of back pain in children? (5)

Answer 38

- Developmental abnormalities; spondylolysis, spondylolisthesis, scoliosis
- Traumatic; vertebral stress fractures, muscle spasm due to overuse, prolapsed intervertebral disc
- Neoplastic; primary benign or malignant vertebral or spinal cord tumours, leukaemias or lymphomas, metastases (neuroblastoma)
- Infection; discitis (common before 6 years), vertebral osteomyelitis
- Rheumatological; oligoarticular juvenile idiopathic arthritis, ankylosing spondylitis, psoriatic arthritis

Question 39

Outline the red flags to be aware of in children with back pain? (5)

Answer 39

- Persistent or worsening pain - Systemic features; fever, malaise or weight loss - Neurological symptoms or signs - Sphincter dysfunction - Young age; especially < 4 years (tumours are most likely)

Question 40

Outline the classification of spinal tumours? (3)

Answer 40

- Intramedullary; within the cord
- Extramedullary intradural; usually benign
- Extramedullary extradural; usually metastases

Question 41

What is the most likely cause of an abdominal mass associated with back pain in a child?

Answer 41

Bladder enlargement; due to loss of sphincter control as a result of spinal cord compression from a malignant process

Question 42

Outline the triad of features associated with West syndrome? (3)

Answer 42

- Infantile spasms; seizure type causing contraction of axial muscles
- Development regression; loss of previously obtained milestones
- Hypsarrhythmia; a chaotic pattern of activity seen on electroencephalogram (EEG)

Question 43

Outline the specific aetiologies associated with West Syndrome? (4)

Answer 43

- Congenital structural brain abnormalities - Acquired brain injury; hypoxia, meningitis - Neurometabolic disease; mitochondrial disorders - Neurocutaneous syndromes; neurofibromatosis, tuberous sclerosis

Question 44

What is the most common cause of developmental regression associated with infantile spasms?

Answer 44

Tuberous sclerosis

Question 45

What is the most common cause of hearing impairment in children?

Answer 45

Conductive hearing loss secondary to glue ear (OME)

Question 46

What is meant by glue ear?

Answer 46

Glue ear; chronic otitis media with effusion (OME)

Question 47

Which group of paediatric patients are particularly susceptible to glue ear (chronic otitis media with effusion)?

Answer 47

Children with Down syndrome; because the have large adenoids a small nasopharynx and narrow Eustachian tubes

Question 48

Why must children with Down syndrome have regular screening for hearing impairment?

Answer 48

Becuse the are particularly vulnerable to 'glue ear' or otitis media with effusion (OME) because they have large adenoids a small nasopharynx and narrow Eustachian tubes which make aeration of the middle ear less efficient

Question 49

Whick kind of hearing loss is associated with chronic otitis media with effusion (OME) or 'glue ear'?

Answer 49

Conductive hearing loss

Question 50

Outline the two main types of squint? (2)

Answer 50

- Convergent squint; affected eye is deviated toward the nose when attempting to look directly at a target
- Divergent squint; affected eye is deviated away from the nose when attempting to look directly at a target

Question 51

Outline the two main aetiologies of a squint? (2)

Answer 51

- Paralytic squint; due to dysfunction of the cranial nerves controlling eye movements (III, IV and VI) or the muscles of the eye themselves
- Concomitant squint; due to extraocular muscle imbalance in infants or refractive errors after infancy

Question 52

What is the major risk factor associated with an uncorrected squint?

Answer 52

Amblyopia; in the developing brain, the image from the squinting eye is suppressed to avoid diplopia. This leads to irreversible suppression of the visual pathways and subsequent visual impairment in that eye and possible blindness.

Question 53

Outline the potential management options of concomitant squints? (3)

Answer 53

- Glasses; to correct any refractive errors
- Patching of good eye; to force use of the other eye and correctextaocular imbalances 
- Surgery; on the extraocular muscles themselves

Question 54

Which test can be performed to determine the nature of a squint?

Answer 54

Cover-uncover test 

Question 55

Outline the absolute indications for surgical management of an ectopic pregnancy as a first-line treatment? (7)

Answer 55

- Serum β-hCG > 5000 IU L^-1
- > 35 mm adnexal mass
- Fetal heart beat present 
- Haemodynamic instability 
- Significant pain 
- Significant haemoperitoneum
- Patient choice

Question 56

What is the likelhood of recurrent ectopic pregnancy following salpingectomy/salpingotomy?

Answer 56

10 - 15% chance of subsequent ectopic pregnancies

Question 57

Which contraceptives should be avoided in patients who have had an ectopic pregnancy? (3)

Answer 57

- Progesterone only pill (POP)
- Intrauterine device (Cu-IUD)
- Intrauterine system (LNG-IUS)

Question 58

What percentage of women who experience an ectopic pregnancy will go on to have a live birth within the next 3 years?

Answer 58

60%

Question 59

When should transvaginal ultrasound scanning (TVUS) begin in women with a previous ectopic pregnancy?

Answer 59

Early TVUS indicated at 5 weeks gestation in women with a history of ectopic pregnancy

Question 60

Outline the differentials diagnoses for abdominal pain in early pregnancy? (8)

Answer 60

- Corpus luteal cyst - Ectopic pregnancy - Miscarriage - Ovarian cysts - Urinary tract infection  - Renal tract calculus - Constipation - Appendicitis 

Question 61

What is the definition of a pregnancy of unknown location (PUL)?

Answer 61

Pregnancy of unknown location (PUL); woman with a positive pregnancy test but with no ultrasound signs of either intra or extrauterine pregnancy or retained products of conception

Question 62

Outline the differential diagnoses of a pregnancy of unknown location? (3)

Answer 62

- Early intrauterine pregnancy; too early to be visualised
- Failed pregnancy; complete miscarriage
- Ectopic pregnancy; either in adnexa but too small to be visualised or elsewhere in the pelvis

Question 63

Outline the rough serum β-hCG concentration at which an intrauterine pregnancy should be able to be visualised on transvaginal ultrasound (TVUS)?

Answer 63

From a serum β-hCG concentration around 1000 - 1500 IU L^-1 and above 

Question 64

Outline the trend in serum β-hCG concentrations in an early intrauterine pregnancy?

Answer 64

Serum β-hCG concentrations should increase by 66% every 48 hours 

Question 65

Outline the management of pregnancies of unknown location (PUL)? (4)

Answer 65

- Follow-up serum β-hCG concentrations
- Serum progesterone concentrations
- Repeat transvaginal ultrasound scan within a few days 
- Laparoscopy if no pregnancy visualised on second scan

Question 66

Which clotting factor is deficient in haemophilia A?

Answer 66

Clotting factor VIII

Question 67

Which clotting factor is deficient in haemophilia B?

Answer 67

Clotting factor IX

Question 68

Outline the definition of an extended spectrum β lactamase (ESBL) positive organism?

Answer 68

Resistance to multiple different classes of β-lactam antibiotics including penicillins, cephalosporins and monobactam antibiotics 

Question 69

Resistance to which other class of antibiotics is commonly associated with extended spectrum β-lactam (ESBL) resistance?

Answer 69

Aminoglycosides such as gentamicin

Question 70

How can neonatal respiratory distress sydrome (NRDS) be prevented aside from administering maternal corticosteroids?

Answer 70

Endotracheal administration of surfactant

Question 71

What is the most common cause of respiratory distress in newborn term neonates? 

Answer 71

Transient tachypnoea of the newborn (TTNB)

Question 72

Why is it vital to intubate neonates with congenital diaphragmatic hernias?

Answer 72

To prevent swallowing of air which may distend the herniated bowel and worsen respiratory compromise

Question 73

Outline the management of a congenital diaphragmatic hernia? (3)

Answer 73

- Intubation; prevent swallowing of air
- NG tube; aspirate gastric fluid and air 
- Surgery; to return the herniated bowel

Question 74

What is the most common complication of a congenital diaphragmatic hernia?

Answer 74

Pulmonary hypoplasia due to compression of the lungs by the bowel within the thorax 

Question 75

Outline the definition of an apnoeic episode?

Answer 75

Episode of cessation of breathing lasting for longer than 20 seconds or less, if associated with bradycardia/colour change

Question 76

Outline the main risk factors associated with neonatal sepsis? (6)

Answer 76

- Preterm premature rupture of membranes (PPROM) - Prolonged rupture of membranes (PROM) - Current maternal group B Streptococcus colonisation - Group B Streptococcus infection in previous neonate - Preterm birth following spontaneous labour - Maternal intrapartum fever/chorioamnionitis 

Question 77

Outline the main radiological features of osteomyelitis? (2)

Answer 77

- Chronic inflammatory destruction of the bone resulting from infection 
- Formation of sequestrae; dead pieces of bone contained within living bone 

Question 78

Outline the process of sequestrae formation in osteomyelitis?

Answer 78

Pus collection in the bone raises the intraosseous pressure which results in impaired perfusion and subsequent ischaemic necrosis of surrounding bone 

Question 79

Which organism is most classically associated with osteomyelitis?

Answer 79

Staphylococcus aureus

Question 80

Outline the two main mechanisms by which osteomyelitis may develop? (2)

Answer 80

- Contiguous spread; from adjacent soft tissue or joints via trauma or direct inoculation, more common in adults
- Haematogenous spread; from distant sites via the blood stream, more common in children

Question 81

Outline the criteria for a diagnosis of osteomyelitis? (4)

Answer 81

≥ 2 of the following 
- Pus aspirated from affected bone
- Positive bone or blood culture 
- Positive imaging findings
- Classical localised signs; bone pain swelling, redness

Question 82

Which cohort of patients are particularly at risk of developing osteomyelitis?

Answer 82

Diabetics; due to the increased risk of developing foot ulcers and/or peripheral neuropathy

Question 83

Which family of bacteria are most commonly associated with infective endocarditis?

Answer 83

Gram positive cocci 
- Staphylococci
- Streptococci
- Enterococci

Question 84

Under which circumstances are urinary tract infections (UTIs) in adults referred to as complicated? (5)

Answer 84

- Occuring in men
- Occuring in the elderly
- Occuring in pregnant women 
- Occuring where the is an in-dwelling catheter
- Occuring where there is an anatomical or functional abnormality in the urinary tract

Question 85

Outline the most common symptoms associated with Lyme disease? (2) 

Answer 85

- Flu-like symptoms 
- Erythema migrans 'target-lesions'

Question 86

What is the causative organism in Lyme disease?

Answer 86

Borrelia burgdorferi

Question 87

Outline the main risk factors associated with Clostridium difficile infection? (8)

Answer 87

CARE-BLIP

- Care home resident 
- Antibiotics

- Recent hospital admission

- Elderly
- Bowel surgery

- Laxatives
- Immunosuppression 

- Proton pump inhibitors (PPIs)

Question 88

Which atypical pneumonia-causing organism is associated with hyponatraemia secondary to syndrome of inappropriate ADH release (SIADH)?

Answer 88

Legionella pneumophilia

Question 89

Which antibiotics are used to treat Legionella pneumophilia atypical pneumonia?

Answer 89

Macrolides; usually clarithromycin

Question 90

Which organism is most commonly identified as causative in septic arthritis?

Answer 90

Staphylococcus aureus

Question 91

What is the most common route of spread in septic arthritis?

Answer 91

Haematogenous

Question 92

Which organism is the most commonly identified cause of acute otitis externa?

Answer 92

Pseudomonas aeruginosa 

Question 93

Outline the choice of antibiotics in the management of acute otitis externa? (2)

Answer 93

- Mild-to-moderate; topical neomycin plus dexamethasone (otomize)
- Moderate-to-severe; oral flucloxicillin (erythromycin if penicillin allergic)

Question 94

Which manoeuvres can be used to exacerbate the pain associated with acute otitis externa?

Answer 94

Pulling on the pinna or pushing on the tragus

Question 95

Which type of organisms are the most common cause of conjunctivitis?

Answer 95

Viruses; adenoviruses, coxsackieviruses and herpesviruses

Question 96

What is the most common cause of inflammation of the eye?

Answer 96

Viral conjunctivitis

Question 97

How can viral and bacterial causes of conjuncitivitis be differentiated symptomatically? (2)

Answer 97

- Viral conjunctivitis; watery discharge, pre-auricular lymphadenopathy
- Bacterial conjunctivitis; muco-purulent discharge, eyelid matting

Question 98

Which organisms are most commonly associated with neonatal bacterial conjunctivitis? (2)

Answer 98

- Neisseria gonorrhoea
- Chlamydia trachomatis

Question 99

How are blanching and non-blanching rashes different in terms of their underlying cause?

Answer 99

- Blanching rashes; caused by inflammation of the skin 
- Non-blanching rashes; caused by bleeding beneath the skin

Question 100

What is the difference between macules and papules?

Answer 100

- Macules are flat lesions < 0.5 mm in diameter
- Papules are raised lesions < 0.5 mm in diameter

Question 101

What is the difference between petechiae and purpura?

Answer 101

- Petechiae; non-blanching lesions < 2 mm in diameter
- Purpura; non-blanching lesions > 2 mm in diameter 

Question 102

How can one differentiate petechiae and purpura from macules and papules?

Answer 102

- Petechiae and purpura are flattened and not raised unlike papules - Similarly petechiae and purpura do not blanch whereas macules and papules will

Question 103

Why can parvovirus B19 infection (erythema infectiosum/fifth disease) precipitate an aplastic crisis in patients with sickle cell disease?

Answer 103

Parvovirus B19 infects red cell progenitor cells in the bone marrow which results in a blockade or erythropoeisis

Question 104

Outline the complications associated with parvovirus B19 infection (erythema infectiosum)? (3)

Answer 104

- Fetal hydrops; due to fetal anaemia and cardiac failure - Aplastic crisis; in patients with underlying haemaglobinopathies  - Chronic infection and anaemia; due to failure to produce IgM

Question 105

Which patients are at increased risk of complications following parvovirus B19 infections (erythema infectiosum)? (3)

Answer 105

- Pregnant women; risk of fetal hydrops
- Immunocompromised; risk of chronic infection
- Haemoglobinopathies; risk of transient aplastic crises

Question 106

What is the most common cause of a sudden deterioration in a ventilated pre-term neonate?

Answer 106

Pneumothorax

Question 107

How can pneumothoraces be diagnosed in neonates? (2)

Answer 107

- Cold-light transillumination of the chest
- Chest X-ray 

Question 108

Which group of patients are particularly at risk of duodenal atresia?

Answer 108

Patients with Down syndrome; around 20% of patients with duodenal atresia have Down syndrome

Question 109

Which sign seen on abdominal X-ray imaging is classically associated with duodenal atresia?

Answer 109

Double-bubble sign; due to dilatation of the stomach and the proximal duodenum

Question 110

Broadly speaking outline the aetiologies of polyhydramnios and oligohydramnios? (2)

Answer 110

- Polyhdramnios; occurs due to an inability of the infant to swallow amniotic fluid - Oligohydramnios; occurs due to an inability to excrete amniotic fluid in the urine due to renal agenesis or dysgenesis

Question 111

What is the major complication of untreated jaundice in a neonate?

Answer 111

Unconjugated bilirubin can cross the blood-brain barrier and very high levels can lead to kernicterus which in-turn can cause deafness and choreoathetoid cerebral palsy.

Question 112

Outline the definitive management of extrahepatic biliary atresia?

Answer 112

Kasai procudure (hepatoportoenterostomy); roux-en-Y loop of small intestine is anastomosed directly to the hilum of the liver after excision of the remnant biliary system to restore normal bile flow.

Question 113

Which long-term complication may occur as a result of surgical resection and/or radiotherapy aimed at treating a craniopharyngioma?

Answer 113

Hypopituitarism; e.g. secondary adrenal insufficiency due to damage to the pituitary gland and impaired ACTH release

Question 114

Which genetic conditions are associated with an increased risk of developing phaeochromocytomas? (3)

Answer 114

- Multiple endocrine neoplasm type 2 (MEN2) - Von Hippel-Lindau (VHL) Syndrome - Neurofibromatosis type 1 (NF1)

Question 115

Outline the main features of a phaeochromocytoma? (5)

Answer 115

- Hypertension - Headaches - Palpitations - Sweating - Tremor

Question 116

Why is it that the features of phaeochromocytoma are often transient/episodic?

Answer 116

- Because the tumours intermittently release both noradrenaline and adrenaline. - Noradrenaline is an α-selective adrenoceptor agonist that will cause arterial vasoconstriction and thus will increase blood pressure. - Adrenaline is a non-selective adrenoceptor agonist that will stimulate both the α-adrenoceptors, triggering an increase in blood pressure, and the β-adrenoceptors, causing a tachycardia. 

Question 117

Outline the two key investigations to be carried out in suspected cases of phaeochromocytoma? (2)

Answer 117

- 24hr urine collection and urine metanephrine levels | - Urinary vanillylmandelic acid (VMA) levels 

Question 118

Which organism is the most common cause early-onset neonatal sepsis in the UK?

Answer 118

Group B Streptococci (GBS); usually Streptococcus agalactiae which accounts for ~ 75% of cases

Question 119

Which organism is most commonly associated with haemolytic uraemic syndrome (HUS)?

Answer 119

Escherichia coli O157:H7

Question 120

Which organisms are classically associated with pelvic inflammatory disease (PID)? (2)

Answer 120

- Chlamydia trachomatis
- Neisseria gonorrhoea

Question 121

What is the most common cause of pelvic inflammatory disease (PID)?

Answer 121

Sexually transmitted infection (STI)

Question 122

Outline the complications associated with pelvic inflammatory disease (PID)? (3)

Answer 122

- Congenital eye/lung infections in neonates 
- Infertility and ectopic pregnancy 
- Fitz-Hugh-Curtis syndrome; perihepatitis due to adhesions within the peritoneum

Question 123

Outline the classical characteristics of actinomycosis? (4)

Answer 123

DAFT - Draining sinuses - Abscesses - Fistulae - Tissue fibrosis 

Question 124

Which region of the body is most commonly affected by actinomycosis?

Answer 124

Jaw; accounts for 50-70% of cases

Question 125

Outline the typical presentation of cervicofacial actinomycosis?

Answer 125

Chronic and slowly progressive painful swelling of the jaw with purulent exudate

Question 126

Which organism is classically associated with actinomycosis?

Answer 126

Actinomyces israelii

Question 127

Outline the typical appearance of actinomyces on gram staining?

Answer 127

Branching gram positive beaded rods (bacilli) that may be contained within 'sulfur' granules

Question 128

Which special stain can be used to identify actinomycosis?

Answer 128

Grocott-Gomori Methenamine-Silver

Question 129

Outline the types of abnormal red blood cells that can indicate hyposplenism? (3)

Answer 129

- Howell-Jolly bodies (HJ); abnormal RBCs with basophilic staining due to presence of nuclear remnants - Acanthocytes (AC); abnormal RBCs with a dense, shrunken appearance and spike-like projections of cell membrane - Target cells (TC); abnormal RBCs with a central haemoglobinised region surrounded by a ring of pallor indicative of iron-deficiency anaemia

Question 130

Outline the differential diagnoses of neonatal jaundice presenting within 24hrs of birth? (6)

Answer 130

- Rhesus haemolytic disease of the newborn (HDNB)
- ABO incompatibility 
- Neonatal sepsis (GBS)
- Congenital viral infection (CMV, Rubella)
- Glucose-6-phosphate dehydrogenase (G6PD) deficiency 
- Hereditary spherocytosis

Question 131

Outline the differential diagnoses of neonatal jaundice presenting between 24hrs and 14 days of birth? (4)

Answer 131

- Physiological jaundice
- Bruising 
- Polycythaemia
- Infection 

Question 132

Outline the differential diagnoses of neonatal jaundice presenting after 14 days from birth? (6)

Answer 132

- Persistent pathological jaundice  - Breast milk jaundice - Neonatal hepatitis - Biliary atresia - Hypothyroidism - Galactosaemia

Question 133

Outline the pathophysiology of dermatitis herpetiformis as a complication of coeliac disease?

Answer 133

The anti-TTG IgA antibodies bind to epidermal transglutaminase present in dermal papillae causing chronic, intensely itchy, blistering skin lesions

Question 134

Which triad of histological features are commonly seen on duodenal biopsies of patients with coeliac disease? (3)

Answer 134

- Crypt hyperplasia - Villous atrophy  - Lymphocytic infiltration

Question 135

What is the most common causative organism in patients with traveller's diarrhoea?

Answer 135

Enterotoxigenic Escherichia coli (ETEC)

Question 136

Outline the clinical features associated with giardia-positive traveller's diarrhoea? (4)

Answer 136

- Chronic watery diarrhoea
- Steatorrhoea
- Abdominal cramps
- Weight loss 

Question 137

Outline the most common cause of parasitic traveller's diarrhoea?

Answer 137

Giardia lamblia; a protozoan parasite acquired by ingestion of cysts in contaminated water or food

Question 138

Outline the two essential investigations in all patients presenting with traveller's diarrhoea? (2)

Answer 138

- Stool microscopy, culture and sensitivity (MC&S)
- Stool ova, cysts and parasites (OCP)

Question 139

What may be seen on stool ova, cysts and parasite microscopy in patients with giardiasis?

Answer 139

Smooth-walled oval cystic structures 

Question 140

Which stain can be used to identify parasitic cysts during microscopy of fecal material?

Answer 140

Lugol's iodine stain; cytst and trophozoite stages seen in 90% of cases of giardiasis

Question 141

Outline the main complications associated with malaria in pregnancy? (6)

Answer 141

- Miscarriage
- Stillbirth 
- Intrauterine growth restriction (IUGR)
- Maternal anaemia
- Placental infection 
- Hypoglycaemia

Question 142

Which drug is used first-line to treat malaria in pregnant women?

Answer 142

Quinine

Question 143

Which Plasmodium spp. are known to cause disease in humans? (5)

Answer 143

- Plasmodium falciparum
- Plasmodium vivax
- Plasmodium ovale
- Plasmodium malariae
- Plasmodium knowlesi 

Question 144

How long after a bite from an infected-mosquito will symptoms of malaria first appear?

Answer 144

Usually 10 - 15 days after infection

Question 145

Outline the risk factors associated with cervical cancer? (6)

Answer 145

- High-risk human papillomavirus (hrHPV) infection; HPV-16, HPV-18 - Smoking  - Combined oral contraceptive (COCP) usage - Family history of cervical cancer  - Immunosuppression  - High multiparity; > 5 full term live births

Question 146

Outline the risk factors associated with endometrial hyperplasia/endometrial cancer? (8)

Answer 146

- Unopposed oestrogen therapy; oestrogen-only HRT, tamoxifen - Increasing age - Early menarche - Late menopause - Nulliparity - Polycystic ovarian syndrome (PCOS); due to chronic anovulation - Obesity - Hereditary predisposition; Lynch syndrome 

Question 147

Outline the risk factors associated with ovarian cancer? (10)

Answer 147

- Increasing age - Smoking - Obesity - Endometriosis - Asbestos -  Nulliparity - Early menarche - Late menopause - Unopposed oestrogen therapy; oestrogen-only HRT, tamoxifen, IVF - Genetic predisposition; BRCA1/BRCA2, Lynch syndrome

Question 148

Outline the risk factors associated with developing pre-eclampsia? (6)

Answer 148

- First pregnancy  - Multiple pregnancy  - Family history of pre-eclampsia - BMI ≥ 35 - > 10 years since last pregnancy 

Question 149

Outline the risk factors associated with ectopic pregnancy? (8)

Answer 149

- Previous ectopic pregnancy - Fallopian tube damage - Pelvic inflammatory disease (PID)/sexually transmitted infection (STI) - Endometriosis - Smoking - Intrauterine contraceptives (IUD or IUS) - Progesterone only contraceptive pill (POCP) - In vitro fertilisation (IVF)

Question 150

Outline the risk factors associated with miscarriage? (9)

Answer 150

- Embryological abnormalities - Advancing maternal age - Previous miscarriage - Thrombophilias; factor V leiden (FVL) - Antiphospholipid syndrome (APLS) - Anatomical/structural abnormalities - Endocrinological disorders - Obesity - Smoking

Question 151

Outline the risk factors associated with preterm premature rupture of membranes (P-PROM)? (7)

Answer 151

- Previous preterm birth and/or P-PROM - Smoking - Polyhydramnios - Anatomical/structural abnormalities of the cervix/uterus/placenta - Sexually transmitted infection (STI) - Low BMI - Low socioeconomic status (SES)

Question 152

Outline the methods of confirming amniotic fluid leakage? (4)

Answer 152

- AmnioQuick; amniotic fluid lateral flow/rapid antigen test; looking for IGFBP-1 presence  - Speculum examination; fluid expelled when patient asked to cough and/or fluid seen pooling in the posterior vaginal fornix - Fern test; examine fluid using microscopy looking for arborisation pattern classical of amniotic fluid - Transvaginal ultrasound scan; to look for oligohydramnios as a result of amniotic fluid loss

Question 153

Which class of viruses are the most common cause of viral meningitis? (2)

Answer 153

Enteroviruses
- Coxsackieviruses
- Echovirus

Question 154

Give examples of the key clinically relevant bacteria that cause disease via intracellular pathogenicity? (6)

Answer 154

- Neisseria meningitidis  - Mycobacterium tuberculosis  - Listeria monocytogenes  - Legionellae pneumophilia - Coxiella burnetti - Chlamydia trachomatis

Question 155

Outline the typical incubation period before symptomatic onset of meningococcal disease (N. meningitidis bacterial meningitis)?

Answer 155

2 - 10 days

Question 156

Outline the most important pathogenic serotypes of Neisseria meningitidis? (6)

Answer 156

- A
- B
- C
- W135
- X
- Y

Question 157

Outline the most clinically important congenital infections? (9)

Answer 157

- Toxoplasmosis (Toxoplasma gondii)
- Rubella (Rubella virus)
- Cytomegalovirus (CMV)
- Herpes simplex virus (HSV)
- Coxsackievirus 
- Parvovirus B19
- Syphalis (Treponema pallidum)
- Varicella-zoster virus (VZV)
- Human immunodeficiency virus (HIV) 

Question 158

Which pathogens associated with congenital infections are most commonly associated with placental thickening, microcephaly and intrauterine growth restriction (IUGR)? (4)

Answer 158

- Toxoplasma gondii
- Cytomegalovirus (CMV)
- Herpes simplex virus (HSV)
- Varicella-zoster virus (VZV)

Question 159

Outline the risk of CMV infection of the fetus during the pregnancy of a CMV positive mother?

Answer 159

About 33% 

Question 160

Which test can be used to confirm intrauterine infection with CMV and other congenital infection-causing viruses?

Answer 160

Amniocentesis

Question 161

Which feature seen on ultrasound is heavily indicative of CMV infection of a developing fetus?

Answer 161

Periventricular cerebral calcifications

Question 162

Why may neonates born with congenital infections be positive for IgG antibodies?

Answer 162

IgG is capable of crossing the placenta hence these are likely maternal antibodies

Question 163

Outline the triad of symptoms consistent with a presentation of pulmonary tuberculosis (PTB)? (3)

Answer 163

- Chronic cough - Night sweats - Weight loss

Question 164

Outline the radiological features seen on a PA X-ray film consistent with pulmonary tuberculsis? (3)

Answer 164

- Enlarged hilar regions
- Upper lobe consolidation 
- Cavitation

Question 165

How can central nervous system and peripheral neuromuscular causes of a floppy infant be differentiated? (2)

Answer 165

- Central nervous system floppiness; deep tendon reflexes present, no significant weakness
- Peripheral neuromuscular floppiness; deep tendon reflexes absent, significant weakness 

Question 166

How can the causes of a floppy infant be stratified?

Answer 166

Based on whether the aetiology is central (nervous) or peripheral (neuromuscular)

Question 167

Outline the central nervous system aetiologies associated with a floppy (hypotonic) infant? (5)

Answer 167

- Chromosomal disorders; Prader-Willi syndrome, Down syndrome 
- Brain injury; hypoxic ischaemic encephalopathy, intraventricular haemorrhage
- Brain infection; meningitis, encephalitis, sepsis
- Metabolic disturbances; hypoglycaemia 
- Drug exposure; pethidine

Question 168

Outline the peripheral neuromuscular causes of a floppy infant? (5)

Answer 168

- Infantile spinal muscular atrophy (SMA) - Congenital myasthenia gravis (CMG) - Congenital myotonic dystrophy (DM1) - Congenital myopathy (CM) - Congenital musclar dystrophy (CMD)

Question 169

What is the most common aetiology of hypotonia in infants?

Answer 169

Central causes

Question 170

Why may the first few inflation breaths during neonatal resuscitation not cause observable chest movement?

Answer 170

The first few inflation breaths may not produce chest movement because they act to displace lung fluid.

Question 171

How can the affectiveness of the first few inflation breaths given during neonatal resuscitation be measured?

Answer 171

Checking for an increase in the heart rate associated with the inflation breaths

Question 172

What percentage of live births are affected by congenital malformations?

Answer 172

Around 3% 

Question 173

How can we stratify the causes of congenital malformations? (3)

Answer 173

- Genetic causes - Environmental causes - Sporadic causes

Question 174

Outline the main genetic causes of congenital malformations? (2)

Answer 174

- Chromosomal abnormalities; DiGeoge syndrome (22q11 deletion)
- Gene mutations; CHARGE syndrome caused by CHD7 mutation

Question 175

Outline the features of CHARGE syndrome? (6)

Answer 175

- Coloboma; a hole or defect of the iris of the eye
- Heart defects; most commonly a VSD
- Atresia choanae; blockade of the back of the nasal passage
- Retardation; affecting growth and/or development
- Genital abnormalities; hypogonadism, undescended testicles, hypospadias
- Ear abnormalities; deafness, abnormally bowl-shaped and concave ears

Question 176

Outline the classic presentation of metabolic disease?

Answer 176

Sudden deterioration in the neonatal period

Question 177

Outline the common metabolic disorders associated with severe metabolic acidosis? (3)

Answer 177

- Propionic acidaemia  - Methylmalonic acidaemia - Isovaleric acidaemia

Question 178

What kind of acid-base disorder is most commonly associated with congenital metabolic disease?

Answer 178

Severe metabolic acidosis with increased anion gap and reduced base excess

Question 179

Outline the normal ratio of luteinising hormone (LH) to follicle stimulating hormone (FSH) in healthy women of reproductive age?

Answer 179

1:1

Question 180

What is the most common cause of a raised luteinising hormone (LH) to follicle stimulating hormone (FSH) ratio in women?

Answer 180

Polycystic ovarian syndrome (PCOS)

Question 181

Outline the pathophysiology of polycystic ovarian syndrome (PCOS)? (4)

Answer 181

- Loss of pulsatility and/or increased LH release from the anterior pituitary results in the excessive stimulation of the ovaries - This results in the excess production of androgens alongside the development of multiple immature follicles - Excess androgen secretion results in hirsuitism and acne whilst development of the follicles causes the classic polycystic appearance of the ovaries - In addition to this a state of insulin resistance develops which causes increased insulin release that further stimulates the ovaries to produce androgens

Question 182

Outline the most common complications associated with polycystic ovarian syndrome (PCOS)? (6)

Answer 182

- Endometrial hyperplasia +/- progression to endometrial carcinoma
- Infertility due to anovulation 
- Anxiety and depression 
- Metabolic syndrome; diabetes, atherosclerosis
- Gestational diabetes (GDM) 
- Cerebrovascular accidents (CVA)

Question 183

Outline the features of the triple assessment of a breast lump? (3)

Answer 183

- Clinical assessment; history and examination
- Imaging; either ultrasound or mammography
- Histology; either fine-needle aspiration or core biopsy

Question 184

Outline the benign causes of a breast lump? (10)

Answer 184

- Fibroadenoma
- Fibrocytstic breast changes
- Breast cysts 
- Fat necrosis
- Lipoma 
- Galactocoele 
- Pyllodes tumour
- Sclerosing adenosis
- Epithelial hyperplasia
- Duct papilloma 

Question 185

Outline the main histological subtypes of in situ breast carcinoma? (2)

Answer 185

- Ductal carcinoma 
- Lobular carcinoma

Question 186

Outline the main histological subtypes of invasive (infiltrating) breast carcinoma? (5)

Answer 186

- Ductal carcinoma 
- Lobular carcinoma
- Mucinous carcinoma
- Medullary carcinoma
- Tubular carcinoma

Question 187

Outline the first-line investigations carried out in patients presenting with a breast lump? (3)

Answer 187

Triple Assessment
- Clinical assessment; history and examination
- Imaging; either ultrasound or mammography
- Histology; either fine-needle aspiration or core biopsy

Question 188

Outline the set investigations carried out in patients following a diagnosis of breast cancer? (5)

Answer 188

- Histopathology; histological subtyping and tumour grading (TNM)  - Disease staging scans; MRI breast, CT-CAP, CT brain, CXR, liver USS and bone scan  - Receptor expression; oestrogen (ER), progesterone (PR) and EGF-R (ERBB2/HER2) - Assessment of axillae; USS +/- FNA/sentinel node biopsy - Genetic testing; DNA microarray of tumour and genetic testing of patient

Question 189

What is Paget's disease of the breast/nipple? (3)

Answer 189

- Paget's disease is an eczematoid change of the nipple associated with straw-coloured or bloody discharge and a burning sensation around nipple
- Often represents an underlying breast malignancy and can be found alongside a breast mass 
- It is thought to be due to malignant ductal cells migrating into the lactiferous sinuses and the nipple skin

Question 190

Outline the investigations required in patients presenting with Paget's disease of the nipple? (2)

Answer 190

- Imaging; mammography/breast ultrasound  | - Punch biopsy; histological and cytological analysis

Question 191

Outline the indications for a coroners referral following a patients' death? (9)

Answer 191

- Unknown cause of death

- Sudden, unexpected death; all deaths < 24 hours of hospital admission

- Deceased had not seen a doctor within 2 weeks

- Death due to accident, self-neglect or neglect

- Death due to occupation

- Death due to abortion

- Death occurred during an operation or prior to anesthetic recovery

- Potential suicide

- Death occurred whilst in police custody

Question 192

Outline the subtypes of non-germ cell testicular tumours? (2)

Answer 192

- Sex cord-stromal; sertoli cell, leydig cell | - Others; lymphoma, metastases

Question 193

What features of a patients transaminitis can suggest a chronic inflammatory process over an acute picture?

Answer 193

Mildly raised transaminases point towards a chronic inflammatory process as opposed to an acute picture, particularly in the context of hepatitis

Question 194

Which hepatitis virus is most commonly associated with chronic infection and cirrhosis?

Answer 194

Hepatitis C; chronic and cirrhotic

Question 195

Outline the routes of transmission of hepatitis B? (3)

Answer 195

- Parenterally 
- Sexually 
- Vertically 

Question 196

What is the approximate incubation period of hepatitis B?

Answer 196

~ 3 months

Question 197

What percentage of patients develop chronic hepatitis B infections?

Answer 197

5 - 10%

Question 198

Which organisms are known to cause enteric (typhoid) fever? (2)

Answer 198

- Salmonella enterica typhi
- Salmonella enterica paratyphi

Question 199

Outline the gram staining results of a blood culture from a patient with typhoid fever?

Answer 199

Gram negative rods (bacilli)

Question 200

Which features specifically raise suspicion of typhoid fever in a patient with fever and abdominal pain? (2)

Answer 200

- Relative bradycardia  | - Relatively low or normal WBC and platelet count

Question 201

Which organisms are most commonly associated with infection of the skin and soft tissues? (2)

Answer 201

- Staphylococcus aureus
- Streptococcus pyogenes 

Question 202

Which specific strains of Staphylococcus aureus are commonly associated with necrotising pneumonia and necrotising fasciitis?

Answer 202

Panton-Valentine Leukocidin (PVL) toxin-producing strains; this is a β-pore forming cytotoxin and virulence factor capable of lysing white blood cells

Question 203

Outline the mainstay treatment used in infected abscesses?

Answer 203

Incision and drainage

Question 204

What is the definition of methicillin-resistant staphylococcus aureus (MRSA)?

Answer 204

Staphylococcus aureus strains resistant to multiple groups of the β-lactam class of antibiotics (penicillins, cephalosporins, carbapenems) and sometimes aminoglycosides, macrolides and quinolones also

Question 205

Outline the pathogenesis of Streptococcal toxic-shock syndrome (TST)? (2)

Answer 205

- Some Streptococcus pyogenes strains produce streptococcal pyrogenic exotoxins (SPEs)
- SPE-A, SPE-B and SPE-C act as superantigens directly stimulating T-cells thus resulting in cytokine storm and sepsis 

Question 206

Outline the pathogenesis of Staphylococcal toxic-shock syndrome (TST)? (2)

Answer 206

- Some Staphylococcus aureus strains produce the exotoxin toxic-shock syndrome toxin-1 (TSST-1)
- TSST-1 acts as a superantigen directly stimulating T-cells thus resulting in cytokine storm and sepsis 

Question 207

Outline the two main categories of urethral discharge? (2)

Answer 207

- Gonococcal urethritis (GU); caused by Neisseria gonorrhoeae - Non-gonococcal urethritis (NGU); caused by non-gonococcal organisms

Question 208

Which organisms are associated with non-gonococcal urethrtitis (NGU)? (4)

Answer 208

CUM-T
- Chlamydia trachomatis
- Ureaplasma urealyticum 
- Mycoplasma genitalium
- Trichomonas vaginalis 

Question 209

What features may allow differentiation of gonococcal and non-gonoccocal urethrtitis? (3)

Answer 209

- Gonoccocal urethritis (GU); acute onset, prominent dysuria, thick-yellow discharge - Non-gonoccocal urethritis (NGU); indolent onset, less prominent dysuria, thin, watery discharge 

Question 210

Which organism is most commonly associated with non-gonoccocal urethritis (NGU)?

Answer 210

Chlamydia trachomatis

Question 211

What is considered the standard test for patients suspected of having gonococcal urethritis (GU)?

Answer 211

Nucleic acid amplification test (NAAT)

Question 212

How do the symptoms of gonococcal urethritis differ in men and women? (2)

Answer 212

- Men; mucopurulent yellow urethral discharge
- Women; endocervical infection

Question 213

Outline the dysmorphic features associated with Down syndrome? (6)

Answer 213

- Flattened face/nasal bridge - Upward slanting palpebral fissures - Epicanthic folds - White speckles in the iris (Brushfield spots) - Short broad hands - Single palmar and plantar fisssures (Simian crease)

Question 214

Outline the features commonly associated with Down syndrome? (4)

Answer 214

- Low intelligence quotient (IQ) - Congenital heart disease - Duodenal atresia - Short stature

Question 215

Which investigation should all children with confirmed Down syndrome have?

Answer 215

Cardiac echocardiogram; even if no murmur is detected as prevelance of cardiac abnormalities is high (~30%)

Question 216

Outline the three different types of down syndrome? (3)

Answer 216

- Trisomy 21; ~96% of cases, due to a complete duplication of chromosome 21
- Robertsonian translocation; ~3% of cases, caused by translocation 14q21q 
- Mosaic down syndrome; ~1% of cases, mixture of trisomy 21 and non-trisomy 21 cells

Question 217

Outline the significance of the Robertsonian translocation in Down syndrome? (2)

Answer 217

- This is often an inherited translocation that can be passed on from an asymptomatic carrier parent to a child who will manifest the disease - As such this is associated with a 5-15% risk of any children born to the carrier parent being affected by Down syndrome

Question 218

Outline the causes of hypocalcaemia in infancy? (7)

Answer 218

- Prematurity - Hypoxic ischaemic encephalopathy (HIE) - Hypoparathyroidism; transient or permanent  - Hypomagnesaemia; magnesium facilitates PTH release  - Maternal hypercalcaemia; suppression of fetal PTH release - Maternal vitamin D deficiency - Familial mutations; calcium-sensing receptor 

Question 219

Why do children who experience neonatal siezures need surveillance and follow-up?

Answer 219

Neonatal seizures are associated with adverse developmental outcomes

Question 220

Outline the investigations that can be used to inform clinicians whether a women is likely to go into premature labour? (3)

Answer 220

- Fetal fibronectin (fFN) test; elevated levels associated with premature labour 
- Transvaginal ultrasound (TVUS); measurement of cervical length, > 15 mm suggests premature labour unlikely 
- PartoSure; rapid antigen test for placental α microglobulin 1 (PAMG-1) 

Question 221

Which organism is most commonly associated with puerperal sepsis?

Answer 221

Group A Streptococci (Streptococcus pyogenes)

Question 222

Outline the pathophysiology of how infection can lead to preterm premature rupture of membranes (P-PROM)?

Answer 222

Bacterial infections trigger a cascade of signalling that results in the release of prostaglandins and matrix metalloproteinases (MMPs) that can break down the fetal membranes and initiate the labour process

Question 223

Outline the risk factors associated with periventricular leukomalacia (PVL)? (3)

Answer 223

- Prematurity - Fetal distress at birth - Chorioamnionitis

Question 224

Outline the pathophysiology of periventricular leukomalacia (PVL) secondary to infection?

Answer 224

Bacterial antigens enter the fetal brain and trigger the localised production of cytokines which results in oligodendrocyte apoptosis and myelin degeneration

Question 225

Outline the most common primary tumours associated with boney metastases in men and women? (2)

Answer 225

- Men; prostate cancer
- Women; breast cancer

Question 226

Which types of tumours do mutations in MLH1/MSH2 particularly predispose patients to? (2)

Answer 226

- Colorectal carcinoma | - Endometrial carcinoma

Question 227

Outline the features associated with an innocent murmur? (6)

Answer 227

The 6 S's
- Sensitive to position
- Short duration
- Sternal edge (left)
- Soft and blowing
- Systolic
- No symptoms

Question 228

Which tumours are most commonly associated with 'small round blue cells'? (3)

Answer 228

- Ewing sarcoma 
- Neuroblastoma
- Small cell lung cancer (SCLC)

Question 229

What is the most common cause of gastroenteritis in children?

Answer 229

Rotavirus

Question 230

Outline the most common benign and malignant brain tumours in children? (2)

Answer 230

- Benign; pilocytic astrocytoma
- Malignant; medulloblastoma

Question 231

Oultine the main differences between Ewing sarcoma and osteosarcoma? (3)

Answer 231

- Osteosarcoma; osteoid-producing, affects metaphysis, mesenchymal
- Ewing sarcoma; osteo-lytic, affects diaphysis, neuroectoderm (CD99) 

Question 232

Outline the risk factors associated with developmental dysplasia of the hip (DDH)? (6)

Answer 232

The 6 F's;
- Feet-first (breech)
- Female,
- Fluid low (oligohydramnios)
- Fat (maternal type 2 diabetes mellitus)
- First-born
- Family history

Question 233

Outline the components of the combined screening test in pregnancy? (4)

Answer 233

- Free beta human chorionic gonadotropin (β-hCG)
- Pregnancy-associated plasma protein-A (PAPP-A)

- Nuchal translucency (NT)
- Crown-rump length (CRL)

Question 234

Outline the components of the quadruple screening test in pregnancy? (4)

Answer 234

- Free beta human chorionic gonadotropin (β-hCG)
- Alpha-fetoprotein (AFP)
- Unconjugated estriol (uE₃)
- Inhibin A

Question 235

Outline the circumstances in which the quadruple test used instead of the combined test as screening during pregnancy? (4)

Answer 235

- After 13⁺⁶ weeks and/or - Nuchal translucency (NT) cannot be measured - Crown-rump length (CRL) > 84 mm  - Head circumference (HC) 101 - 172 mm

Question 236

Outline the components of the standard booking bloods offered to all women? (6)

Answer 236

- Full blood count (FBC); anaemia - Blood grouping; red cell antigens (ABO) and rhesus-D antigen status - Serum antibodies; RhD antibody status  - Viral serology; HBV, HIV and RV - Syphilis serology; anti-treponemal antibodies - Haemoglobin electrophoresis; screening for haemoglobinopathies in at-risk women

Question 237

Outline the post-partum management of neonates born to mothers with confirmed hepatitis B infection during pregnancy? (3)

Answer 237

- Accelerated HBV vaccination; 12 hrs, 1-2 months and 6 months
- Hepaitits B virus immunoglobulin (HBIG); within 12 hrs of birth 
- Consider antivirals; lamivudine if high viral load in mother

Question 238

Outline the classical features of allergic rhinitis? (4)

Answer 238

- Rhinorrhoea
- Sneezing
- Nasal congestion 
- Itching eyes

Question 239

Which atopic disease classically presents first in childhood?

Answer 239

Eczema; this usually manifests in infancy

Question 240

Outline the classic triad of atopic diseases? (3)

Answer 240

- Asthma - Eczema - Allergic rhinits

Question 241

Outline the two main tests that can be used to investigate potential allergies? (2)

Answer 241

- Radioallergosorbent testing (RAST); IgE-specific allergen test
- Skin-prick testing; more sensitive and specific 

Question 242

Which types of fracture are particularly suspicious for non-accidental injury (NAI)? (3)

Answer 242

- Metaphyseal fractures; occuring at the widest point of the bone  - Posterior rib fractures; highly unusual especially in immoble infants - Spiral fractures; sharp angular fractures that imply a twisting injury

Question 243

Outline the investigations required in cases of suspected non-accidental injury (NAI)? (3)

Answer 243

- Full skeletal survey; investigate for potential fractures - Brain CT; investigate subdural haemorrhage - Clotting screen; investigate any bruising 

Question 244

Outline the common risk factors associated with non-accidental injury (NAI)? (5)

Answer 244

- First-time parents - Young parents - No local support - Low socioeconomic status - Difficulties with feeding/parenting

Question 245

What is the most common cause of malodorous discharge in pre-pubertal females?

Answer 245

Vulvovaginitis

Question 246

What is the most common cause of an abnormal head shape in an infant?

Answer 246

Positional (deformational) plagiocephaly; caused by asymmetrical pressure on the developing occiput or skull base

Question 247

Outline the differential diagnoses for an infant with an abnormal asymmetrical head? (2)

Answer 247

- Positional (deformational) plagiocephaly; caused by asymmetrical sleeping position, torticollis or cervical spine abnormalities.
- Craniosynostosis; caused by premature fusion of one or more cranial sutures

Question 248

What is the most common cause of an englarged head in an infant?

Answer 248

Famililal macrocephaly 

Question 249

Outline the differential diagnoses of an enlarged head in an infant? (5)

Answer 249

- Familial macrocephaly - Hydrocephalus - Fragile X syndrome - Subdural haematoma - Space occupying lesion

Question 250

What is the most common cause of accidental poisoning in childhood?

Answer 250

Iron poisoning; because iron-containing preparations are widely available and ofren resemble sweets

Question 251

Outline the features of childhood iron poisoning? (5)

Answer 251

- Acute liver dysfunction - Tissue hypoxia - Encephalopathy  - Gastrointestinal bleeding - Diarrhoea/vomiting

Question 252

Outline the most common differential diagnoses associated with intermenstrual bleeding? (6)

Answer 252

- Endometrial hyperplasia/malignancy
- Cervcal malignancy 
- Cervical ectropion 
- Endocervical polyp
- Atrophic vaginitis 
- Pregnancy 
- Irregular bleeding due to POCP

Question 253

Outline the definitive management of an endometrial polyp?

Answer 253

Hysteroscopy and resection

Question 254

Outline the main aetiologies that can cause hyperprolactinaemia? (5)

Answer 254

- Physiological; breast feeding, pregnancy, stress
- Malignancy associated; prolactinoma
- Drugs; antipsychotics, metoclopramide, methyldopa
- Idiopathic 
- Others; hypothyroidism, chronic kidney disease

Question 255

Which feature of the history is vitally imporant in women presenting with amenorrhoea/infertility?

Answer 255

Medication history; as a number of drugs can cause hyperprolactinaemia

Question 256

Outline the threshold value of the day 21 serum progesterone that is highly suggestive of ovulation?

Answer 256

Serum progesterone > 30 nmol L^-1 is suggestive of ovulation

Question 257

What percentage of women with hyperprolactinaemia will present with galactorrhoea?

Answer 257

< 50%; it is not a common symptom

Question 258

What is the most common presenting complaint seen in women with hyperprolactinaemia?

Answer 258

Infertility and/or amenorrhoea

Question 259

Outline the risk factors associated with preterm labour? (8)

Answer 259

- Extremes of maternal age
- Low SES 
- Infection 
- Previous preterm birth 
- Multiple pregnancy 
- Hypertension in pregnancy 
- Polyhydramnios 
- Structural abnormalities of the cervix and/or uterus 

Question 260

Outline the interventions that may be used to prevent preterm delivery in women with risk factors? (2)

Answer 260

- Rescue cervical cerclage; rescue suture
- Progesterone supplementation; decreases myometrial activity and prevents cervical remodelling

Question 261

Outline the management of preterm labour with intact membranes? (6)

Answer 261

- Engange fetal monitoring (CTG) - Tocolysis; nifedipine or atosiban if before 34 weeks - Maternal corticosteroids; to promote fetal lung maturation - IV magnesium sulfate; neuroprotective of fetal brain - Intrapartum antibiotics; benzylpenicillin (penicillin G) 3 g IV then 1.5 g IV every 4 hours until delivery - Delayed cord clamping; increase circulating volume and haemoglobin

Question 262

Outline the management of preterm premature rupture of membranes (P-PROM)? (4)

Answer 262

- Erythromycin 250 mg (PO) QDS for 10 days 
- Maternal corticosteroids; to promote fetal lung maturation
- IV magnesium sulfate; neuroprotective of fetal brain 
- Induction of labour if > 34 weels

Question 263

When is co-amoxiclav contraindicated in pregnancy and why?

Answer 263

Co-amoxiclav is contraindicated in preterm premature rupture of membranes (P-PROM) due to the risk of the neonate developing necrotising enterocolitis (NEC)

Question 264

Outline the difference between small for gestational age (SGA) fetuses and intrauterine growth restriction (IUGR)? (2)

Answer 264

- Small for gestational age (SGA); consistenly below 10th centile in all indices 
- Intrauterine growth restriction (IUGR); dropping centiles in 1 or more indices

Question 265

Which metric measured as part of the routine monitoring on ultrasound during pregnancy is affected first by intrauterine growth restriction (IUGR)?

Answer 265

Fetal abdominal circumference

Question 266

Outline the common metrics measured during a growth scan? (5)

Answer 266

- Fetal abdominal circumference (FAC)
- Estimated fetal weight (EFW)
- Biparietal diameter (BPD)
- Uterine arterial pulsitility index (UA-PI)
- Amniotic fluid volume (AFV)

Question 267

How can we stratify the risk factors associated with intrauterine growth restriction (IUGR)? (3)

Answer 267

- Fetal; congenital infections, chromosomal abnormalities - Maternal; drugs, smoking, extremes of age, HTN, DM, TORCH - Placental; PET, multiple pregnancy, placental insufficiency

Question 268

Outline the investigations that should be carried out following a diagnosis of intrauterine growth restriction (IUGR)? (4)

Answer 268

- Maternal doppler ultrasound scan; investigate flow through uterine artery 
- Repeat anomaly scan; investigate any undiagnosed fetal anomalies
- TORCH screen; checking for new maternal infections
- Cardiotocography (CTG); assess fetal wellbeing 

Question 269

Which investigations should be carried out in all teenagers presenting drunk? (2)

Answer 269

- Blood glucose levels; to rule out hypoglycaemia
- Cranial CT; if GCS is reduced 

Question 270

At what age should pica (repeated/chronic ingestion of non-nutritive substances) be investigated?

Answer 270

After the age of 2-years as it may indicate underlying pathology

Question 271

Outline the abnormal red blood cell features that can indicate lead poisoning? (2)

Answer 271

- Microcytosis; due to iron-deficiency anaemia | - Basophilic stippling of RBCs; indicative of disturbed erythropoiesis

Question 272

Which mineral deficiencies are associated with lead poisioning and why? 

Answer 272

Calcium and iron deficiency; because lead competes with these metals for uptake from the intestines

Question 273

Outline the key risk factors associated with pica in children?

Answer 273

Emotional and social neglect

Question 274

What volume of 1/1000 adrenaline should be given to patients < 6 years of age with signs of anaphylactic shock?

Answer 274

0.15 mL of 1/1000 adrenaline for a total dose of 0.15 mg

Question 275

What volume of 1/1000 adrenaline should be given to patients 6 - 12 years years of age with signs of anaphylactic shock?

Answer 275

0.30 mL of 1/1000 adrenaline for a total dose of 0.30 mg

Question 276

What volume of 1/1000 adrenaline should be given to patients > 12 years years of age with signs of anaphylactic shock?

Answer 276

0.50 mL of 1/1000 adrenaline for a total dose of 0.50 mg

Question 277

How does premature ovarian failure often present?

Answer 277

Signs of early menopause; hot flushes, vaginal dryness, amenorrhoea

Question 278

Outline the management of premature ovarian failure? (2)

Answer 278

- Continuous oestrogen replacement; for bone and cardiovascular protection
- Progesterone supplementation; either continuous or cyclical to minimise risks of unopposed oestrogens 

Question 279

Which type of lung cancer is most commonly associated with hypercalcaemia?

Answer 279

Squamous cell carcinoma (SCC); due to ectopic parathyroid hormone (PTH) secretion 

Question 280

Outline the differentials diagnoses associated with an ovarian mass? (4)

Answer 280

- Follicular cyst - Corpus luteal cyst  - Endometrioma  - Ovarian tumour

Question 281

Outline the main classes of ovarian tumour? (4)

Answer 281

- Epithelial tumours - Sex cord-stromal tumour - Germ cell tumours - Metastases

Question 282

Outline the specific ovarian tumour markers and the specific type of tumour they are associated with? (6)

Answer 282

- LDH; dysgerminomas
- CA-125; epithelial ovarian tumours
- CEA; mucinous ovarian tumours
- AFP; yolk-sac tumours
- β-hCG; choriocarcinomas
- Inhibin-A/B; granulosa cell tumours 

Question 283

Which genes are most commonly tested for in patients with breast cancer? (3)

Answer 283

- BRCA1 - BRCA2 - PALB2

Question 284

Outline the most common type of invasive breast carcinoma?

Answer 284

Carcinoma of no special type (NST)

Question 285

Outline the triad of features associated with Meig's syndrome? (3)

Answer 285

- Benign ovarian tumour; usually a fibroma
- Pleural effusion 
- Ascites 

Question 286

Outline how the Bishop score can be used to guide decisions about induction of labour? (2)

Answer 286

- Score < 5; labour unlikely to start without induction - Score ≥ 8; cervix is ripe/favourable and there is a high chance of spontaneous labour, or response to interventions made to induce labour

Question 287

Outline the threshold gestation of pregnancy from which gestational hypertension can be diagnosed?

Answer 287

- From 20 weeks onwards as blood pressure tends to fall in the first trimester - Hypertension before this point is therefore most likely chronic (essential) hypertension

Question 288

Which vaccinations are routinely offered to pregnant women in the UK?

Answer 288

- Influenza | - Pertussis

Question 289

Outline the risk factors associated with shoulder dystocia? (4)

Answer 289

- Gestational diabetes - Macrosomia - Maternal obesity - Previous shoulder dystocia

Question 290

Outline the management options that can be used in cases of shoulder dystocia? (4)

Answer 290

- Suprapubic pressure - McRoberts manoeuvre - Wood's screw manoeuvre - Symphysiotomy

Question 291

Outline the threshold values of protein quantification that can be used to distinguish nephrotic syndrome from nephritic syndrome? (3)

Answer 291

Nephrotic Range - 24 hr urinary protein > 3.5 g - Albumin creatinine ratio > 220 - Protein creatinine ratio > 300

Question 292

Which glomerulonephritides commonly present with nephritic syndrome? (5)

Answer 292

- Post-streptococcal glomerulonephritis (PSGN) - Lupus nephritis (LN) - IgA nephropathy (IGAN) - Anti-glomerular basemement membrane (anti-GBM) disease; Goodpasture's syndrome - ANCA positive vasculitides; microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA)

Question 293

Which glomerulonephritides commonly present with nephrotic syndrome? (4)

Answer 293

- Minimal change disease (MCD) - Focal segmental glomerulosclerosis (FSGS) - Membranous nephropathy - Renal amyloidosis

Question 294

Why is an abducens nerve (CN VI) palsy often referred to as a 'false localising sign' in the context of raised intracranial pressure?

Answer 294

As the abducens nerve has the longest intracranial course, it is often the first to be affected by raised intracranial pressure. This results in an abducens nerve palsy due to stretching of the nerve as a result of posterior displacement of the brainstem from where it emerges.

Question 295

What is the most common cause of a subarachnoid haemorrhage (SAH)?

Answer 295

Ruptured saccular (berry) aneurysm

Question 296

Outline the most common pathogenic mechanisms resulting in brain abscesses? (3)

Answer 296

- Recent neurosurgical procedures; burr-hole, external ventricular drain - Parameningeal infection; contiguous spread from sinusitis, otitis, mastoiditis, dental abscess - Distant infection; haematogenous spread from lung abscesses and empyema

Question 297

What is meant by the term leptomeninges?

Answer 297

Leptomeninges refers to the innermost layers of the meninges; the pia mater and the arachnoid mater. They are referred to as such as the subarachnoid space is essentially a potential space only in the healthy state

Question 298

Which atypical pneumonia-causing organism is most commonly associated with a concurrent transaminitis alongside pneumonia symptoms?

Answer 298

Coxiella burnetti (Q fever)

Question 299

Outline the differential diagnoses associated with failure to pass meconium within the first 48hrs of life? (6)

Answer 299

- Hirschsprungs disease (congenital aganglionic megacolon) - Meconium ileus/plugging - Intestinal atresia - Anorectal malformations - Hypoganglioma - Ganglioneuromatosis

Question 300

Which test is used to diagnose Hirschsprung's disease?

Answer 300

Suction rectal biopsy

Question 301

Outline the pathophysiology of Hirschsprung's disease?

Answer 301

Failure of migration of neural crest cells to the colon resulting in the absence of enteric ganglia and thus the absence of peristaltic movement in the bowel

Question 302

Outline the definition of hydrops fetalis (fetal hydrops)?

Answer 302

``` - Accumulation of fluid in ≥ 2 body sites • Skin • Pleural cavity • Pericardial cavity • Peritoneal cavity ```

Question 303

Outline the features associated with oesophageal atresia? (3)

Answer 303

- Excessive salivation - Post-feed vomiting - Polyhydramnios during pregnancy

Question 304

What is the most likely cause of a sudden deterioration in symptoms of a patient with known chronic lymphocytic leukaemia (CLL)?

Answer 304

Richter's transformation; a sudden transformation of chronic lymphocytic leukaemia (CLL) into the high-grade non-Hodgkin lympoma (NHL) diffuse large B cell lymphoma (DLBCL)

Question 305

What is different about tuberculous meningitis compared with other bacterial causes of meningitis? (2)

Answer 305

- Tuberculous meningitis tends to predominantly affect the brain stem resulting in cranial nerve palsies - Lymphocytes are the predominant white blood cells detected on CSF analysis

Question 306

What threshold value of serum FSH is considered to be associated with a poor prognosis for conception?

Answer 306

FSH > 10 IU L^-1

Question 307

Which additional test can be used to indicate ovarian reserve in women with infertility who have raised serum FSH?

Answer 307

Anti-Mullerian hormone (AMH) levels

Question 308

Outline the rough staging of ovarian cancer? (4)

Answer 308

- Stage 1; confined to the ovaries - Stage 2; pelvic spread to uterus or fallopian tubes - Stage 3; peritoneal metastases and/or regional lymph nodes - Stage 4; distant metastases to liver parenchyma and beyond

Question 309

Outline the typical presenting features of a leiomyoma (uterine fibroid)? (4)

Answer 309

PAI(M) - Pressure effect from pressure on the bladder, stomach or bowel - Abdominal mass - Infertility - Menorrhagia

Question 310

Which group of patients are at increased risk of developing uterine fibroids (leiomyomas)?

Answer 310

Afro-Caribbean women

Question 311

Outline the management options in patients with uterine fibroids (leiomyomas)? (5)

Answer 311

- Gonadotrophin-releasing hormone analogues - Gonadotrophin-releasing hormone agonists - Uterine artery embolisation - Hysteroscopic transcervical resection of the fibroid (TCRF) - Hysterectomy or myomectomy

Question 312

Outline the mechanism of action of ulipristal acetate?

Answer 312

Ulipristal acetate is a selective progesterone receptor modulator (SPRM) with both progesterone receptor agonist and antagonist properties

Question 313

What is the difference between dyskaryosis and dysplasia in the context of cervical screening? (2)

Answer 313

- Dyskaryosis; refers to the abnormal appearance of cervical epithelial cells observed on a cervical smear - Dysplasia; refers to the dedifferentiation of cells as viewed on histological analysis of cervical biopsies

Question 314

What is meant by the term dysfunctional uterine bleeding (DUB)?

Answer 314

Excessive heavy, prolonged or frequent bleeding that is not due to pregnancy or any recognisable pelvic or systemic disease

Question 315

Outline the management options used in dysfunctional uterine bleeding (DUB)? (5)

Answer 315

- Tranexamic acid (TxA); during menstruation - Levonorgestrel-releasing intrauterine system (LNG-IUS) - Combined oral contraceptive pill (COCP); if < 35 years of age - Endometrial ablation; if not wanting to preserve fertility - Hysterectomy; as a last resort only

Question 316

Which blood tests are vital in women with amennorhoea? (2)

Answer 316

- Serum prolactin | - Thyroid function tests

Question 317

What is the threshold value of prolactin associated with a pituitary adenoma (prolactinoma)?

Answer 317

Prolactin levels > 1000 u L^-1 ; below this but raised can be a result of stress, venepuncture, breast examination or associated with polycystic ovarian syndrome (PCOS)

Question 318

Which additional investigation should be carried out in women suspected of having a prolactinoma?

Answer 318

Visual field testing; prolactinomas can cause a bitemporal superior quadrantanopia

Question 319

Which feature seen on transvaginal ultrasound scan (TVUS) can help to point towards a diagnosis of adenomyosis? (2)

Answer 319

- An indistinct myometrial-endometrial border | - Venetian blind sign; bands of linear shadows along lines of sight, often diverging

Question 320

Outline the features seen on X-rays of patients with osteoarthritis? (4)

Answer 320

LOSS; - Loss of joint space - Osteophytes - Subchondral sclerosis; increased density of the bone along the joint line - Subchondral cysts; fluid-filled holes in the bone, aka geodes

Question 321

Which long-term complication is associated with haemophilia?

Answer 321

Haemophilic arthropathy; osteoarthritis-like disorder with boney joint erosions

Question 322

Outline the abnormal findings that may be seen on blood film analysis of patients with multiple myeloma? (3)

Answer 322

- Rouleaux formation; stacks of RBCs due to monoclonal immunoglobulin protein coating - Plasma cells; in end-stage disease, usually confined to the bone marrow - Anisocytosis; varying sizes of RBCs consisted with mixed anaemia

Question 323

Outline the causes of a normocytic anaemia? (6)

Answer 323

4 A's, 2 H's; - Anaemia of chronic disease - Acute blood loss - Anisocytosis; mixed anaemia picture due to macrocytic and microcytic RBCs - Aplastic anaemia - Haemolytic anaemia - Hypothyroidism

Question 324

Outline the pathophysiology behind thrombocytopaenia in haemolytic uraemic syndrome (HUS)?

Answer 324

Shiga-like toxin produced by Escherichia coli O157:H7 damages the endothelial cell wall which in-turn leads to the activation of platelets and thus their deposition and consumption

Question 325

Outline the pathophysiology behind renal impairment in haemolytic uraemic syndrome (HUS)? (2)

Answer 325

- Shiga-like toxin produced by Escherichia coli O157:H7 damages the endothelial cell wall which in-turn leads to the activation and deposition of platelets particularly in the kidney - This can often be visualised upon histopathological analysis whereby platelet-fibrin thrombi can be seen in the glomeruli

Question 326

Which kind of anaemia is associated with haemolytic uraemic syndrome (HUS)?

Answer 326

Microangiopathic haemolytic anaemia (MAHA); microcytic anaemia with raised LDH, thrombocytopaenia and schistocyte presence on blood film analysis

Question 327

Why is the direct antiglobulin/direct Coomb's test (DAT/DCT) negative in haemolytic uraemic syndrome (HUS)? (2)

Answer 327

- The the direct antiglobulin/direct Coomb's test (DAT/DCT) is only useful in diagnosing autoimmune haemolytic anaemia where the RBCs are bound by antibodies. - In the case of haemolytic uraemic syndrome (HUS) the cause of the haemolysis is shiga-like toxin and therefore the test will be negative.

Question 328

Outline the management of haemolytic uraemic syndrome (HUS)? (3)

Answer 328

- Supportive management; no need for antibiotics - Inform Public Health England/Infection Control - Isolate patient with barrier nursing

Question 329

What key features allow you to differentiate haemolytic uraemic syndrome (HUS) from thrombotic thrombocytopaenic purpura (TTP)?

Answer 329

Thrombotic thrombocytopaenic purpura (TTP) will present with the additional features of neurological deficits and fever.

Question 330

Outline the final common pathophysiological mechanisms that contribute to the development of hydrops fetalis (fetal hydrops)? (3)

Answer 330

- Raised central venous pressure - Low plasma oncotic pressure - Reduced lymphatic fluid flow

Question 331

Outline the management of women exposed to varicella zoster virus (chickenpox) during pregnancy who are confirmed to be non-immune? (2)

Answer 331

- ≤ 20 weeks gestation; give varicella-zoster immunoglobulin (VZIG) immediately if within 10 days of exposure - > 20 weeks gestation; EITHER • Antivirals (aciclovir) 7-14 days post-exposure • Varicella-zoster immunoglobulin (VZIG) if within 10 days of exposure

Question 332

What is the most common cause of hydrops fetalis (fetal hydrops) in the third trimester of pregnancy?

Answer 332

Immune causes; rhesus disease, ABO incompatibility, anti-Kell, anti-Duffy

Question 333

Which test can be used to calculate the dose of anti-RhD to give a mother following a sensitising event?

Answer 333

Kleihauer; acid elution test which breaks down adult haemoglobin which can be visualised under the microscope, whilst fetal haemoglobin remains resistant

Question 334

What additional investigation is required in all patients diagnosed with a Staphylococcus aureus bacteraemia?

Answer 334

Transthoracic or transoesophageal echocardiogram; due to the risk of Staphylococcus aureus endocarditis

Question 335

Which groups of heart valves are most commonly affected by infective endocarditis? (2)

Answer 335

- Most patients; aortic and mitral valves | - IVDU, CHDs, ICD; pulmonary and tricuspid

Question 336

Outline the organisms most commonly associated with culture negative endocarditis? (5)

Answer 336

HACEK - Haemophilus spp. - Aggregatibacter spp. - Cardiobacterium spp. - Eikinelia spp. - Kingella spp.

Question 337

Which specific organism has been shown to be associated with infective endocarditis in elderly patients with colorectal cancer?

Answer 337

Streptococcus bovis (group D β-haemolytic streptococcus)

Question 338

Which organisms are most commonly associated with infective endocarditis and rheumatic heart disease? (2)

Answer 338

- Infective endocarditis; caused by Streptococcus viridans (α-haemolytic Streptococcus) - Rheumatic heart disease; caused by Streptococcus pyogenes (group A β-haemolytic Streptococcus)

Question 339

Outline the components of the sepsis six used in the management approach to the septic patient? (6)

Answer 339

- 3 in; oxygen, broad-spectrum antibiotics, IV fluids | - 3 out; urine output, blood cultures, lactate

Question 340

Outline the acute management of the septic patient? (3)

Answer 340

- A-E assessment - Alert senior - Sepsis six

Question 341

Which electrolyte abnormality is most commonly associated with an acute kidney injury (AKI)?

Answer 341

Hyperkalaemia

Question 342

Outline the swab components of a sexually transmitted infection (STI) screen? (3)

Answer 342

- Endocervical swab for gonorrhoea - Endocervical swab for chlamydia - High vaginal swab for trichomonas and candida

Question 343

Outline the triad of features associated with a renal infarct? (3)

Answer 343

- Flank pain; with acute onset that the patient can pinpoint - Hypertension - Haematuria

Question 344

Outline the most common complication associated with infective endocarditis?

Answer 344

Septic embolism - Left-sided valves; septic brain emboli - Right-sided valves; septic pulmonary emboli

Question 345

Outline the difference between red infarcts and white infarcts? (2)

Answer 345

- Red infarcts are caused by venous occlusion in tissue with collateral or dual circulation - White infarcts are caused by arterial occlusion in tissue without collateral or dual circulation

Question 346

Which organs are most commonly associated with red infarcts? (4)

Answer 346

- Liver - Lung - Bowel - Brain

Question 347

Which organs are most commonly associated with white infarcts? (4)

Answer 347

- Heart - Kidney - Spleen - Brain

Question 348

Outline the three main types of necrosis and the situations in which you might see them? (3)

Answer 348

- Coagulative necrosis; most organs - Liquefactive necrosis; brain - Caseous necrosis; tuberculosis

Question 349

Outline the two most common risk factors associated with infective endocarditis? (2)

Answer 349

- Previous rheumatic fever/rheumatic heart disease | - Non-native cardiac valves

Question 350

Outline the pathophysiology of glomerulonephritis secondary to infective endocarditis?

Answer 350

Antigen-antibody complexes embolise from the site of infection and become deposited in the glomerular basement membrane where they trigger a type III immune complex-mediated hypersensitivity reaction that induces mast cell degranulation via F_cγRIII activation. This results in inflammation and impaired renal function.

Question 351

Which empirical antibiotic treatment regimen is used in patients with native valve endocarditis?

Answer 351

Amoxicillin plus gentamicin

Question 352

Outline the management of cystic fibrosis? (5)

Answer 352

- High calorie diet; including pancreatic enzymes and fat soluble vitamins (ADEK) - Chest physiotherapy; to release secretions and help lung function - Mucolytic agents; human recombinant DNase (dornase alpha) plus hypertonic saline nebuliser - Prophylactic antibiotics; usually flucloxacillin for Staphylococcus aureus prophylaxis - Lumacaftor/Ivacaftor (Orkambi); if homozyogus for δF508 mutation

Question 353

Outline the triad of features associated with shaken-baby syndrome? (3)

Answer 353

- Retinal haemorrhages - Subdural haemorrhage - Encephalopathy

Question 354

Gives examples of particularly concerning sites/features of lesions that raise suspicion of non-accidental injury (NAI)? (8)

Answer 354

Sites; - Genitalia - Ears - Neck - Trunk Features; - ≤ 4 years of age - Non-mobile infant - Patterned bruising - Bilateral

Question 355

Which conditions make up the seronegative spondyloarthropathies? (4)

Answer 355

PEAR - Psoriatic arthritis - Enteropathic arthritis - Ankylosing spondylitis - Reactive arthritis

Question 356

Which scoring system can be used to stratify a woman's risk of having ovarian cancer?

Answer 356

Risk of malignancy index (RMI)

Question 357

Outline the components of the risk of malignancy index (RMI) scoring system that is used to aid investigation suspected of ovarian cancer? (3)

Answer 357

- Ultrasound findings - Menopausal status - CA-125 levels

Question 358

Outline how the risk of malignancy index (RMI) score is calculated?

Answer 358

RMI = U x M x CA125

Question 359

Outline the key complication associated with transcervical resection of fibroids (TCRF)?

Answer 359

Fluid overload and consequent hyponatraemia

Question 360

Outline the management of Turner's syndrome (X0)? (5)

Answer 360

- Psychological management and counselling; if required - Human growth hormone; to correct the short stature - Oestrogen therapy; to stimulate secondary sex characteristics and protect against osteoporosis - Cyclical progesterone; added later to induce withdrawal bleeds - Fertility input; ovum donation and hormonal support

Question 361

Outline the first-line investigations that should be carried out in women presenting with symptoms of premenstrual syndrome (PMS)?

Answer 361

Symptom diary; a 3-month record of symptoms alongside the days on which menstruation occurs

Question 362

How can premenstrual syndrome (PMS) be differentiated from depressive episodes?

Answer 362

Premenstrual syndrome (PMS) can be diagnosed by the presence of symptoms during the luteal phase of the cycle (day 14-0 in a 28-day cycle) and resolving within a 1-2 days of menstruation starting.

Question 363

Outline the physical symptoms that can be associated with premenstrual syndrome (PMS)? (2)

Answer 363

- Breast tenderness | - Feeling bloated

Question 364

Outline the the most frequently used management options in premenstrual syndrome (PMS)? (2)

Answer 364

- Selective serotonin reuptake inhibitors (SSRIs); either taken continuously or during the luteal phase - New-generation combined oral contraceptive pill (COCP); drospirenone-containing formulations that interrupt ovulation

Question 365

Outline the management options used in the different stages of cervical cancer? (4)

Answer 365

- CIN I - III; large loop excision of the transformation zone (LLETZ) or cervical cone biopsy - Stage 1B - 2A; trachelectomy or radical hysterectomy - Stage 2B - 4A: chemotherapy and radiotherapy - Stage 4B: combination of surgery, radiotherapy, chemotherapy and palliative care

Question 366

Outline the immediate management of intrauterine death? (2)

Answer 366

Induction of labour (IOL) - Mifepristone; antiprogesterone - Misoprostol; 48 hours after to induce contractions

Question 367

Why is it vital that induction of labour (IOL) should be offered as soon as possible in pregnancies where there is an intrauterine death?

Answer 367

Due to the increased risk of the development of sepsis or disseminated intravascular coagulopathy (DIC)

Question 368

Outline the investigations that should be carried out after an intrauterine death? (6)

Answer 368

Maternal Investigations - FBC and clotting screen; to exclude DIC - Kleihauer test; to assess fetomaternal haemorrhage - Anticardiolipin and lupus anticoagulant; to assess for antiphospholipid syndrome (APLS) Fetal Investigations - Karyotype; to assess for chromosomal abnormalities - Fetal and placental swabs; for MC&S to rule out infection - Post-mortem; if agreed to by parents

Question 369

Outline the four main methods of distinguishing pre-renal, renal and post-renal causes of acute kidney injury? (4)

Answer 369

- Urea:creatinine ratio - Urine [Na⁺] - Urine osmolality - Fractional Na⁺ excretion (FE_Na)

Question 370

What is the most common cause of nephrotic syndrome in adults?

Answer 370

Membranous nephropathy

Question 371

Outline the triad of paraneoplastic symptoms associated with renal cell carcinoma (RCC)? (3)

Answer 371

- Hypertension - Hypercalcaemia - Polycythaemia

Question 372

Which genetic conditions are associated with an inherited predisposition to renal cell carcinoma (RCC)? (4)

Answer 372

- Von Hippel-Lindau (VHL) syndrome - Tuberous sclerosis (TS) - Birt-Hogg-Dubé (BHD) syndrome - Hereditary papillary renal carcinoma (HPRC)

Question 373

Which two forms of amyloidosis are particularly associated with renal amyloidosis and nephrotic syndrome? (2)

Answer 373

- Immunoglobulin light chain amyloidosis (AL); associated with multiple myeloma and hypergammaglobulinaemia - Serum amyloid A amyloidosis (AA); associated with deposition of the acute-phase protein SAA due to chronic inflammation

Question 374

What is the term used to describe the situation in which an individual possesses two different disease-causing alleles of the same gene?

Answer 374

Compound heterozygosity

Question 375

Which conditions are classically associated with a microangiopathic haemolytic anaemia (MAHA)? (3)

Answer 375

- Disseminated intravascular coagulation (DIC) - Thrombotic thrombocytopaenic purpura (TTP) - Haemolytic uraemic syndrome (HUS)

Question 376

What is the main complication associated with artificial rupture of membranes (ARM)?

Answer 376

Umbilical cord prolapse

Question 377

Which group of immunohistochemical markers can be used to determine cancers of epithelial origin?

Answer 377

Cytokeratins (CKs)

Question 378

Mutations in which genes are commonly found in lung adenocarcinoma tumours? (2)

Answer 378

- Anaplastic lymphoma kinase (ALK) | - Epidermal growth factor receptor (EGFR)

Question 379

At what value of the crown-rump length (CRL) should the fetal heart beat be visible?

Answer 379

At a crown-rump length (CRL) > 7 mm the fetal heart beat should be visible

Question 380

Which conditions are classically associated with a complete hydatidiform mole (gestational trophoblastic disease)? (3)

Answer 380

- Hyperemesis - Thyrotoxicosis - Pre-eclampsia

Question 381

Outline the management of a complete hydatidiform mole (gestational trophoblastic disease)? (3)

Answer 381

- Surgical evacuation of the uterus - Follow-up serial β-hCG levels - Chemotherapy if trophoblast persists

Question 382

Outline the complication associated with persistent trophoblast in patients with a complete hydatidiform mole (gestational trophoblastic disease)?

Answer 382

Increased risk of developing choriocarcinoma

Question 383

Outline the main caveat to using intramuscular methotrexate in the medical management of ectopic pregnancy?

Answer 383

Untrauterine pregnancy must be ruled out; by repeat ultrasound and serial β-hCG measurements

Question 384

From what gestation in pregnancy is anti-RhD required in rhesus negative women who miscarry?

Answer 384

From 12 weeks onwards women who miscarry should be given anti-RhD

Question 385

What is the most common gynaecological cause of pain in early pregnancy?

Answer 385

Corpus luteal cysts

Question 386

Outline the clinical definition of hyperemesis gravidarum?

Answer 386

Severe or protracted vomiting appearing for the first time before the 20th week of pregnancy that is not associated with other coincidental conditions

Question 387

Outline the management of hyperemesis gravidarum? (5)

Answer 387

- Fluids; 3-4L of normal (0.9%) saline per day - Potassium; to correct hypokalaemia as a result of vomiting - Antiemetics; cyclizine, metoclopramide or prochlorpeazine - Thiamine and folic acid; to prevent Wernicke's encephalopathy and Korsakoff's syndrome - Thromboembolic stockings (TEDS) and heparin; women with hyperemesis are at risk of thrombosis

Question 388

What percentage of cases of vaginal bleeding during pregnancy are associated with miscarriage?

Answer 388

50% of women who experience vaginal bleeding during pregnancy will go on to miscarry

Question 389

Outline the management options in stress incontinence? (3)

Answer 389

- Conservative; lifestyle changes and pelvic floor exercises - Surgical; tension-free vaginal tape or transoburator tape - Medical; duloxetine (SNRI) that reduces incontinent episodes

Question 390

Which endocrinological disorder can be associated with dermoid ovarian cysts (benign teratomas)?

Answer 390

Hyperthyroidism; sometimes benign teratomas may contain thyroid tissue within them that causes excess thyroid hormone secretion; these are known as struma ovarii cysts

Question 391

Outline the pathological causes of precocious puberty in women? (5)

Answer 391

- Hypothyroidism - CNS lesion; hydrocephaly, neurofibromatosis - Ovarian tumours - Adrenal tumours - Exogenous oestrogens

Question 392

Which type of renal tumour is most commonly diagnosed during the neonatal period?

Answer 392

Mesoblastic nephroma

Question 393

Which electrolyte abnormality is associated with mesoblastic nephroma?

Answer 393

Hypercalcaemia

Question 394

Outline the trend in serum alpha-fetoprotein (AFP) levels following birth?

Answer 394

- At birth the serum AFP will be at its highest, sometimes several thousand-fold higher than the adult amount - Following birth the serum AFP gradually declines until around 8 months

Question 395

What are the threshold values associated with bronchodilator reversibility that indicate a diagnosis of asthma as opposed to chronic obstructive pulmonary disease (COPD)? (2)

Answer 395

- ≥ 12% improvement in FEV1 post-bronchodilator AND - ≥ 200 mL improvement in FEV1 compared to pre-bronchodilator

Question 396

What is the most common cause of a macrocytosis without anaemia?

Answer 396

Alcohol intake

Question 397

Which gene is mutated in α1 antitrypsin deficiency (AATD)?

Answer 397

SERPINA1 found on chromosome 14

Question 398

What is the mode of inheritance in α1 antitrypsin deficiency (AATD)?

Answer 398

Autosomal recessive; homozygotes with the so-called 'PiZZ' genotype will have the condition

Question 399

Which human leukocyte antigen (HLA) alleles are associated with coeliac disease? (2)

Answer 399

- HLA-DQ2 | - HLA-DQ8

Question 400

Outline the complication that is commonly associated with poorly-controlled coeliac disease?

Answer 400

Enteropathy Associated T-cell Lymphoma (EATL)

Question 401

Outline the current hypothesis surrounding the aetiology of endemic Burkitt's lymphoma in sub-saharan Africa? (2)

Answer 401

- Endemic Burkitt lymphoma in sub-saharan Africa is related to EBV and Plasmodium falciparum co-infection - It is thought to arise due to Plasmodium falciparum-induced polyclonal B cell activation or impairment of EBV-specific T-cells

Question 402

When during pregnancy is anti-RhD given routinely to rhesus negative women? (3)

Answer 402

- 28 weeks - 34 weeks - < 72 hours of delivery; if not already sensitised

Question 403

Outline the pathophysiology of fibroid degeneration? (4)

Answer 403

- Uterine fibroids initially grow in pregnancy due to the increase in circulating oestrogens - However there comes a point where the growth of the fibroid actually outgrows its blood supply - At this point the fibroid undergoes 'red degeneration' characterised by necrosis within the fibroid - This causes localised uterine tenderness and may cause some pregnant women to present to hospital

Question 404

Which additional tests should be carried out in women who are IV drug users who are found to be HBsAg positive in pregnancy? (4)

Answer 404

- HBeAg; to determine risk of transmission to the fetus - Liver function tests (LFTs); to investigate degree of hepatitis - HCV IgG and IgM antibodies; as high risk for hepatitis C - Urine toxicology; investigate additional drug-related risks to the fetus

Question 405

What is the threshold value considered to be associated with a high risk of Down syndrome based on the results of the combined screening tests?

Answer 405

A risk of 1 in 150 or less is considered high risk (1 in 2 to 1 in 150)

Question 406

How can trisomy 21 (Down syndrome) be differentiated from trisomy 13 (Patau syndrome) and trisomy 18 (Edward syndrome) based on the results of the combined screening test? (2)

Answer 406

- All three have decreased PAPP-A and increased β-hCG and nuchal translucency - However down syndrome usually has a higher β-hCG than Edward and Patau syndrome

Question 407

What are the implications of a previous post-partum haemorrhage (PPH) on the management of subsequent labours?

Answer 407

Women who have had a previous post-partum haemorrhage (PPH) must have active management of the third stage

Question 408

Outline the management of iron deficiency anaemia during pregnancy? (3)

Answer 408

- Dietary advice; iron-rich diet (meat, lentils, spinach) - Oral ferrous sulphate 200 mg (BD); increasing to three times if tolerated - Oral iron suspension or parenteral (IM) iron injections; if tablets not tolerated

Question 409

Outline the timing for delivery in women with pre-eclampsia? (2)

Answer 409

- 34-36⁺⁶ weeks; continue surveillance unless there are indications for planned early birth - ≥ 37 weeks; initiate birth within 24-48 hours either by induction of labour or caesarean section

Question 410

Outline the absolute indications for low-dose aspirin prophylaxis of pre-eclampsia? (5)

Answer 410

HDAC; - Hypertension; chronic or previous gestational hypertension - Diabetes mellitus; type 1 or type 2 - Autoimmune disease; SLE, anti-phospholipid syndrome - Chronic kidney disease (CKD)

Question 411

Outline the risk factors associated with gestational diabetes mellitus (GDM)? (6)

Answer 411

- BMI >30 - Previous macrosomic baby >4.5kg - Previous gestational diabetes - First-degree relative with diabetes - Ethnic origin; South Asia, Black Caribbean, Middle Eastern - Polycystic ovarian syndrome (PCOS)

Question 412

Outline the features associated with McCune-Albright syndrome? (5)

Answer 412

- Fibrous dsyplasia - Café-au-lait spots - Bone cysts - Oestrogen-producing ovarian cysts - Precocious puberty

Question 413

Outline the major complication associated with a pituitary adenoma?

Answer 413

Pituitary apoplexy; haemorrhage into the pituitary fossa often due to rupture of the blood vessels as the tumour grows, this can result in a global hypopituitarism that renders the patient reliant on exogenous pituitary hormones

Question 414

Outline the standard structure of a death certificate? (4)

Answer 414

Part I: Causal Sequence - IA: disease or condition that directly lead to death - IB: factor(s) that ore-disposed/caused the disease or condition that lead to death - IC: the underlying cause of death Part II: Contributing Factors - II: factors which contributed to, but not caused death

Question 415

Which neurodevelopmental disorder is commonly associated with Hirschsprung's disease?

Answer 415

Down syndrome

Question 416

Outline the pathognomonic feature of retained products of conception as a cause of secondary post-partum haemorrhage?

Answer 416

Open cervical os visualised on speculum examination

Question 417

Outline the features classically associated with uterine rupture? (5)

Answer 417

- Cessation of contractions - Abdominal pain - Vaginal bleeding in labour - Maternal tachycardia - Haematuria

Question 418

Outline the main risk factors associated with uterine rupture during labour? (2)

Answer 418

- Previous Caesarean section(s) | - Induction or augmentation of labour

Question 419

Outline the rule of 3's used during the management of fetal bradycardia? (3)

Answer 419

- Deceleration (bradycardia < 100 bpm) lasting 3 minutes; call for help - Deceleration (bradycardia < 100 bpm) lasting 6 minutes; transfer to theatre and prepare for immediate delivery. - Deceleration (bradycardia < 100 bpm) lasting 9 minutes; deliver immediately by category one (‘crash’) caesarean section

Question 420

Which investigations should be carried out in women with suspected chorioamnionitis? (2)

Answer 420

- Blood cultures | - High vaginal swab

Question 421

Outline the immediate management in HELLP syndrome?

Answer 421

Urgent delivery of the the fetus

Question 422

What is the definition of prolonged pregnancy?

Answer 422

Any pregnancy continuing beyond 42 weeks gestation

Question 423

What is the threshold value of the Bishops score needed for artificial rupture of membranes to be carried out?

Answer 423

Bishop score ≥ 6

Question 424

What is the main risk associated with a prolonged pregnancy?

Answer 424

Increased risk of stillbirth

Question 425

When is induction of labour recommended in women who have carried to term?

Answer 425

Between 41 and 42 weeks gestation

Question 426

Outline the potential complications associated with shoulder dystocia? (5)

Answer 426

- Perinatal mortality - Hypoxic ischaemic encephalopathy - Brachial plexus injury (Erb's palsy) - Post-partum haemorrhage (PPH) - Perineal tear

Question 427

Which sign can be seen on ultrasound scans during the first trimester than indicate dichorionic diamniotic (DCDA) twins?

Answer 427

Lambda sign

Question 428

Outline the additional monitoring required in cases of twin pregnancy? (4)

Answer 428

- Regular full blood count (FBC) - Close blood pressure and urinalysis monitoring - Fetal growth surveillance from 28-weeks - Screening for gestational diabetes mellitus (GDM)

Question 429

Outline the main features associated with fetal varicella syndrome? (3)

Answer 429

- Skin scarring - Limb hypoplasia - Neurological abnormalities

Question 430

Outline the surgical sieve framework for stratifying differential diagnoses?

Answer 430

VITAMIN CDEF; - Vascular - Infective/inflammatory - Traumatic - Autoimmune - Metabolic - Iatrogenic - Neoplastic - Congenital - Degenerative - Endocrine - Functional