Passmed Round 1

Question 1

In intravascular haemolysis, free haemoglobin is released which then binds to —— As ———– becomes saturated haemoglobin binds to albumin forming methaemalbumin (detected by ———–). Free haemoglobin is excreted in the urine as haemoglobinuria, haemosiderinuria

Answer 1

In intravascular haemolysis, free haemoglobin is released which then binds to HAPTOGLOBIN. As HAPTOGLOBIN becomes saturated haemoglobin binds to albumin forming methaemalbumin (detected by SCHUMM’S TEST). Free haemoglobin is excreted in the urine as haemoglobinuria, haemosiderinuria

Question 2

Intravascular haemolysis causes?

Answer 2

• Mismatched blood transfusion
• G6PD deficiency*
• Red cell fragmentation: heart valves, TTP, DIC, HUS
• Paroxysmal nocturnal haemoglobinuria
• Cold autoimmune haemolytic anaemia

*strictly speaking there is an element of extravascular haemolysis in G6PD as well, although it is usually classified as a intravascular cause

Question 3

Extravascular haemolysis causes?

Answer 3

• Haemoglobinopathies: sickle cell, thalassaemia
• Hereditary spherocytosis
• Haemolytic disease of newborn
• Warm autoimmune haemolytic anaemia

Question 4

Wilson’s disease is an —– genetic —– disorder characterised by excessive copper deposition in the tissues. Metabolic abnormalities include increased ———- and decreased ——–. Wilson’s disease is caused by a defect in ——- located on chromosome ——.

Answer 4

Wilson’s disease is an autosomal recessive disorder characterised by excessive copper deposition in the tissues. Metabolic abnormalities include increased copper absorption from the small intestine and decreased hepatic copper excretion. Wilson’s disease is caused by a defect in the ATP7B gene located on chromosome 13.

Question 5

Small vessel vasculitides are divided into two brackets.

What are they?
Which vasculitides are in each bracket?

Answer 5

ANCA-associated vasculitides

• granulomatosis with polyangiitis (Wegener’s granulomatosis)
• eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
• microscopic polyangiitis

immune complex small-vessel vasculitis

• Henoch-Schonlein purpura
• Goodpasture’s syndrome (anti-glomerular basement membrane disease)
• cryoglobulinaemic vasculitis
• hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)

Question 6

What antibodies are associated with SLE?

Answer 6

• 99% are ANA positive
• —-this high sensitivity makes it a useful rule out test, but —–it has low specificity
• 20% are rheumatoid factor positive
• anti-dsDNA: highly specific (> 99%), but less sensitive (70%)
• anti-Smith: highly specific (> 99%), sensitivity (30%)
• also: anti-U1 RNP, SS-A (anti-Ro) and SS-B (anti-La)

Question 7

What are the causes of upper lung fibrosis?

Answer 7

CHARTS
C - Coal worker's pneumoconiosis
H - Histiocytosis/ hypersensitivity pneumonitis
A - Ankylosing spondylitis
R - Radiation
T - Tuberculosis
S - Silicosis/sarcoidosis

Question 8

What are the clinical features of limited systemic sclerosis?

Answer 8

Raynaud’s may be first sign

scleroderma affects face and distal limbs predominately
associated with anti-centromere antibodies

a subtype of limited systemic sclerosis is CREST syndrome: Calcinosis, Raynaud’s phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia

Question 9

What are the adverse effects of hydroxychloroquine?

Answer 9

bull’s eye retinopathy - may result in severe and permanent visual loss

• —recent data suggest that retinopathy caused by hydroxychloroquine is more common than previously thought and the most recent RCOphth guidelines (March 2018) suggest colour retinal photography and spectral domain optical coherence tomography scanning of the macula
• —-baseline ophthalmological examination and annual screening is generally recommened

Question 10

What do you use to treat UTI in pregnancy?

Answer 10

if the pregnant woman is symptomatic:

-a urine culture should be sent in all cases
should be treated with an antibiotic for

-first-line: nitrofurantoin (should be avoided near term)
second-line: amoxicillin or cefalexin

-trimethoprim is teratogenic in the first trimester and should be avoided during pregnancy

Question 11

What are the absolute contraindications to lung transplantation in CF patients?

Answer 11

Burkholderia cepacia colonization
—–Specifically, Burkholderia cepacia genomavar III (cenocepacia) has been found to be associated with a survival rate that is unacceptably low to justify transplantation.

Other absolute contraindications include systemic sepsis and failure to identify an appropriate antibiotic regimen for treatment.

Question 12

What are the symptoms of the following paraneoplastic antibodies?

Anti -Ri
Anti GAD
Anti-Hu
Anti Yo

Answer 12

Anti-Ri (Rieally blurry vision)
Anti GAD GAAAAD he’s stiff (stiff man syndrome)
Anti-Hu who kicked my chair (pain) and then fell over (ataxia)
Anti yo- Yo lady give me back my danish (cerebellar syndrome, lady for breast + ovarian)

Question 13

What does a non-pulsatile JVP indicate?

Answer 13

SVC obstruction

Question 14

Describe the parts of the JVP

Answer 14

a wave - atrial contraction

• increased if atrial pressure (e.g. tricuspid stenosis/ pulm stenosis, pulm hypertension
• Absent in AF

C wave - closure of tricuspid (usually not seen)

x descent - fall in atrial pressure during ventricular systole

V wave - due to passive filling of blood into the atrium against a closed tricuspid valve
-Giant V waves in tricuspid regurg

y descent - opening of tricuspid valve

Question 15

What is the management of a primary pneumothorax?

Answer 15

if the rim of air is < 2cm and the patient is not short of breath then discharge should be considered

otherwise, aspiration should be attempted
if this fails (defined as > 2 cm or still short of breath) then a chest drain should be inserted

patients should be advised to avoid smoking to reduce the risk of further episodes - the lifetime risk of developing a pneumothorax in healthy smoking men is around 10% compared with around 0.1% in non-smoking men

Question 16

What is the management of a secondary pneumothorax?

Answer 16

if the patient is > 50 years old and the rim of air is > 2cm and/or the patient is short of breath then a chest drain should be inserted.

otherwise aspiration should be attempted if the rim of air is between 1-2cm. If aspiration fails (i.e. pneumothorax is still greater then 1cm) a chest drain should be inserted.

All patients should be admitted for at least 24 hours
if the pneumothorax is less the 1cm then the BTS guidelines suggest giving oxygen and admitting for 24 hours

regarding scuba diving, the BTS guidelines state: ‘Diving should be permanently avoided unless the patient has undergone bilateral surgical pleurectomy and has normal lung function and chest CT scan postoperatively.’

Question 17

What are the complications of measles?

Answer 17

otitis media: the most common complication
pneumonia: the most common cause of death
encephalitis: typically occurs 1-2 weeks following the onset of the illness)
subacute sclerosing panencephalitis: very rare, may present 5-10 years following the illness
febrile convulsions
keratoconjunctivitis, corneal ulceration
diarrhoea
increased incidence of appendicitis
myocarditis

Question 18

Left anterior fascicular block causes what on the ECG?

Answer 18

Left axis deviation

Question 19

Right anterior fascicular block causes what on the ECG?

Answer 19

Right axis deviation

Question 20

Right bundle branch block influences the cardiac axis in what way?

Answer 20

No change because left ventricle overrides

Question 21

What does bifascicular block on ECG show?

Answer 21

RBBB and left axis deviation

Question 22

What rate is the ventricular escape rhythm?

Answer 22

Around 30 bpm

Question 23

What rate does the heart have to be to diagnose ventricular tachycardia?

Answer 23

> 120 bpm

Question 24

What are northern, southern and western blots used for?

Answer 24

SNOW DROP

South - DNA
NOrth - RNA
West - Protein

Question 25

What are the good prognostic factors in ALL?

Answer 25

``` French-American-British (FAB) L1 type common ALL pre-B phenotype low initial WBC del(9p) Hyperploidy Trisomy 4, 10 and 17 t(12;21), t(1;19) ```

Question 26

What are the poor prognostic factors in ALL?

Answer 26

``` FAB L3 type T or B cell surface markers Philadelphia translocation, t(9;22) age < 2 years or > 10 years male sex CNS involvement high initial WBC (e.g. > 100 * 109/l) non-Caucasian Hypoploidy ```

Question 27

What are the glucocorticoid side effects of corticosteroids?

Answer 27

endocrine: impaired glucose regulation, increased appetite/weight gain, hirsutism, hyperlipidaemia Cushing's syndrome: moon face, buffalo hump, striae musculoskeletal: osteoporosis, proximal myopathy, avascular necrosis of the femoral head immunosuppression: increased susceptibility to severe infection, reactivation of tuberculosis psychiatric: insomnia, mania, depression, psychosis gastrointestinal: peptic ulceration, acute pancreatitis ophthalmic: glaucoma, cataracts suppression of growth in children intracranial hypertension neutrophilia

Question 28

Name the Primary Immunodeficiencies categorised as neutrophil disorders

Answer 28

Neutrophil Disorders - Chronic granulomatous disease - Chediak-Higashi syndrome - Leukocyte adhesion deficiency

Question 29

What drugs cause Steven Johnson Syndrome?

Answer 29

``` allopurinol carbamazepine lamotrigine nevirapine the "oxicam" class of anti-inflammatory drugs (including meloxicam and piroxicam) phenobarbital phenytoin sulfamethocazole and other sulfa antibiotics sertraline sulfasalazine ```

Question 30

What drugs cause pancreatitis?

Answer 30

Thiazides Azathioprine Corticosteroids Sodium valproate

Question 31

What conditions are HLA-DR3?

Answer 31

dermatitis herpetiformis Sjogren's syndrome primary biliary cirrhosis

Question 32

What are the signs of hyposplenism on blood film?

Answer 32

``` Target cells Howell-Jolly bodies Pappenheimer bodies Siderotic granules Acanthocytes ```

Question 33

Give examples of mitochondrial disease

Answer 33

Leber's optic atrophy MELAS syndrome: mitochondrial encephalomyopathy lactic acidosis and stroke-like episodes MERRF syndrome: myoclonus epilepsy with ragged-red fibres Kearns-Sayre syndrome: onset in patients < 20 years old, external ophthalmoplegia, retinitis pigmentosa. Ptosis may be seen sensorineural hearing loss

Question 34

What are the levels of evidence of medical studies?

Answer 34

Ia - evidence from meta-analysis of randomised controlled trials Ib - evidence from at least one randomised controlled trial IIa - evidence from at least one well designed controlled trial which is not randomised IIb - evidence from at least one well designed experimental trial III - evidence from case, correlation and comparative studies IV - evidence from a panel of experts

Question 35

What are the adverse effects of ciclosporin?

Answer 35

``` nephrotoxicity hepatotoxicity fluid retention hypertension hyperkalaemia hypertrichosis gingival hyperplasia tremor impaired glucose tolerance hyperlipidaemia increased susceptibility to severe infection ```

Question 36

What are the risk factors for endometrial cancer?

Answer 36

``` obesity nulliparity early menarche late menopause unopposed oestrogen. The addition of a progestogen to oestrogen reduces this risk (e.g. In HRT). The BNF states that the additional risk is eliminated if a progestogen is given continuously diabetes mellitus tamoxifen polycystic ovarian syndrome hereditary non-polyposis colorectal carcinoma ```

Question 37

What are the clinical features of Von Hippel Lindau syndrome?

Answer 37

cerebellar haemangiomas: these can cause subarachnoid haemorrhages retinal haemangiomas: vitreous haemorrhage renal cysts (premalignant) phaeochromocytoma extra-renal cysts: epididymal, pancreatic, hepatic endolymphatic sac tumours clear-cell renal cell carcinoma

Question 38

What are the clinical features of Turner Syndrome?

Answer 38

- Short stature - Shield chest, widely spaced nipples - Webbed neck - Bicuspid aortic valve (15%), coarctation of the aorta (5-10%) - Primary amenorrhoea - Cystic hygroma (often diagnosed prenatally) - High-arched palate - Short fourth metacarpal - Multiple pigmented naevi - Lymphoedema in neonates (especially feet) - Gonadotrophin levels will be elevated - Hypothyroidism is much more common in Turner's - Horseshoe kidney: the most common renal abnormality in Turner's syndrome

Question 39

Name the live attenuated vaccines

Answer 39

You Musn't Prescribe BCG Incase They RIP Stat= Yellow fever, MMR, Polio(oral), BCG, Influenza(intranasal), Typhoid, Rotavirus(oral), Shingles ``` Yellow fever MMR Polio (oral) BCG Influenza (intranasal) Typhoid Rotavirus (oral) Shingles ```

Question 40

Name the inactivated preparation vaccines

Answer 40

rabies hepatitis A influenza (intramuscular)

Question 41

Name the toxoid vaccines

Answer 41

tetanus diphtheria pertussis

Question 42

Name the subunit/ conjugate vaccines

Answer 42

``` pneumococcus (conjugate) haemophilus (conjugate) meningococcus (conjugate) hepatitis B human papillomavirus ```

Question 43

What are the causes of a prolonged PR interval?

Answer 43

``` idiopathic ischaemic heart disease digoxin toxicity hypokalaemia* rheumatic fever aortic root pathology e.g. abscess secondary to endocarditis Lyme disease sarcoidosis myotonic dystrophy ```

Question 44

What are the cyanotic and acyanotic congenital heart conditions?

Answer 44

Cyanotic - transposition of the great arteries - tricuspid atresia - tetralogy of Fallot Acyanotic - coarctation of the aorta - aortic valve stenosis - ventricular septal defect - atrial septal defect

Question 45

What drugs cause ankle swelling?

Answer 45

Amlodipine | Diltiazem

Question 46

What are the adverse effects of carbamazepine?

Answer 46

``` P450 enzyme inducer dizziness and ataxia drowsiness headache visual disturbances (especially diplopia) Steven-Johnson syndrome leucopenia and agranulocytosis hyponatraemia secondary to syndrome of inappropriate ADH secretion ```

Question 47

What drugs cause headache?

Answer 47

``` Amlodipine Nicorandil ISMN Carbamazepine Sulphasalazine Ivabradine ```

Question 48

What drugs cause hepatotoxicity?

Answer 48

Immunosuppression: Methotrexate, Ciclosporin TB: Rifampicin, Isoniazid, Pyrazinamide Antiepileptics: Phenytoin, Sodium Valproate Diabetic: Pioglitazone, Sulphonurea Amiodarone

Question 49

What drugs cause myelofibrosis/ agranulocytosis?

Answer 49

``` Hydroxurea Cyclophosphamide Flurouracil (5-FU) Irinotecan Methotrexate ```

Question 50

What drugs undergo first pass metabolism?

Answer 50

``` Aspirin Isosorbide dinitrate Glyceryl trinitrate Lignocaine Propranolol Verapamil Isoprenaline Testosterone Hydrocortisone ```

Question 51

What drugs exhibit zero order kinetics?

Answer 51

Phenytoin Salicylates Heparin Ethanol

Question 52

What drugs are affected by acetylator status?

Answer 52

``` Isoniazid Procainamide Hydralazine Dapsone Sulphasalazine ```

Question 53

What drugs cause peripheral neuropathy?

Answer 53

``` Amiodarone Phenytoin Metronidazole Nitrofurantoin Isoniazid ```

Question 54

What drugs cause ataxia

Answer 54

Phenytoin Carbamazepine Sodium valproate Amantadine

Question 55

What are the features of parietal lobe lesions?

Answer 55

Sensory inattention Apraxias Astereognosis (tactile agnostic) Inferior homonymous quadrantanopia Gerstmanns syndrome - lesion of the dominant parietal - alexia, acalculia, finger agnosia, left right disorientation

Question 56

What are the features of occipital lobe lesions?

Answer 56

Homonymous hemianopia (with macula sparing) Cortical blindness Visual agnosia

Question 57

What are the features of a temporal lobe lesion?

Answer 57

Wernickes aphasia Superior homonymous quadrantonopia Auditory agnosia Prosopagnosia (difficulty recognising faces)

Question 58

What are the features of a frontal lobe lesion?

Answer 58

``` Broca’s (expressive) aphasia Disinhibition Perseveration Anosmia Inability to generate a list ```

Question 59

What organisms cause meningitis in 0-3 months?

Answer 59

Group B streptococcus (most common) E. Coli Listeria monocytogenes

Question 60

What organisms cause meningitis at 3 months to 6 years?

Answer 60

Neisseria meningitidis Streptococcus pneumoniae Haemophilis influenzae

Question 61

What organisms cause meningitis between 6 and 60?

Answer 61

Neisseria meningitidis Streptococcus pneumoniae In over 60 also listeria monocytogenes

Question 62

What conditions cause renal tubular acidosis type 1?

Answer 62

``` Rheumatoid arthritis SLE sjogrens Amphoteracin b toxicity Analgesic nephropathy ```

Question 63

What are the causes of dilated cardiomyopathy?

Answer 63

- idiopathic: the most common cause - myocarditis: e.g. Coxsackie B, HIV, diphtheria, Chagas disease - ischaemic heart disease - peripartum - hypertension - iatrogenic: e.g. doxorubicin - substance abuse: e.g. alcohol, cocaine - inherited: either a familial genetic predisposition to DCM or a specific syndrome e.g. Duchenne muscular dystrophy around a third of patients with DCM are thought to have a genetic predisposition a large number of heterogeneous defects have been identified the majority of defects are inherited in an autosomal dominant fashion although other patterns of inheritance are seen infiltrative e.g. haemochromatosis, sarcoidosis + these causes may also lead to restrictive cardiomyopathy nutritional e.g. wet beriberi (thiamine deficiency)

Question 64

What are the following oncogenes associated with? ``` ABL c-MYC n-MYC BCL-2 RET RAS erb-B2 ```

Answer 64

ABL - Cytoplasmic tyrosine kinase - Chronic myeloid leukaemia c-MYC - Transcription factor - Burkitt's lymphoma n-MYC - Transcription factor - Neuroblastoma BCL-2 - Apoptosis regulator protein - Follicular lymphoma RET - Tyrosine kinase receptor - Multiple endocrine neoplasia (types II and III) RAS - G-protein - Many cancers especially pancreatic - Also neurofibromatosis erb-B2 (HER2/neu) - Tyrosine kinase receptor - Breast and ovarian cancer

Question 65

What are the following tumour suppressor genes associated with? ``` p53 APC BRCA1 BRCA2 NF1 Rb WT1 Multiple tumor suppressor 1 (MTS-1, p16) ```

Answer 65

p53 -Common to many cancers, Li-Fraumeni syndrome APC Colorectal cancer BRCA1 -Breast and ovarian cancer BRCA2 -Breast and ovarian cancer NF1 -Neurofibromatosis Rb -Retinoblastoma WT1 -Wilm's tumour Multiple tumor suppressor 1 (MTS-1, p16) -Melanoma

Question 66

What are the causes of cushing's syndrome and how are they divided?

Answer 66

ACTH dependent causes - Cushing's disease (80%): pituitary tumour secreting -ACTH producing adrenal hyperplasia - ectopic ACTH production (5-10%): e.g. small cell lung cancer is the most common causes ACTH independent causes - iatrogenic: steroids - adrenal adenoma (5-10%) - adrenal carcinoma (rare) - Carney complex: syndrome including cardiac myxoma - micronodular adrenal dysplasia (very rare) Pseudo-Cushing's - mimics Cushing's - often due to alcohol excess or severe depression - causes false positive dexamethasone suppression test or -24 hr urinary free cortisol - insulin stress test may be used to differentiate

Question 67

What are the adverse effects of isotretinoin?

Answer 67

teratogenicity -females should ideally be using two forms of contraception (e.g. Combined oral contraceptive pill and condoms) dry skin, eyes and lips/mouth -the most common side-effect of isotretinoin low mood -whilst this is a controversial topic, depression and other psychiatric problems are listed in the BNF raised triglycerides hair thinning nose bleeds (caused by dryness of the nasal mucosa) intracranial hypertension: isotretinoin treatment should not be combined with tetracyclines for this reason photosensitivity

Question 68

Name the Primary Immunodeficiencies categorised as B Cell disorders

Answer 68

B Cell Disorders - Common variable immunodeficiency - Bruton's (x-linked) congenital agammaglobulinaemia - Selective immunoglobulin A deficiency

Question 69

Name the Primary Immunodeficiencies categorised as T cell disorders

Answer 69

T Cell Disorders | -Di George Syndrome

Question 70

Name the primary immunodeficiencies categorised as Combined B- and T- cell disorders

Answer 70

Combined B- and T- Cell disorders - Severe combined immunodeficiency - Ataxic telangiectasia - Wiskott-Aldrich Syndrome - Hyper IgM syndromes

Question 71

What are the stages of clinical trial?

Answer 71

I - Determines pharmacokinetics and pharmacodynamics and side-effects prior to larger studies - Conducted on healthy volunteers II - Assess efficacy + dosage - Involves small number of patients affected by particular disease - May be subdivided into - -IIa - assesses optimal dosing - -IIb - assesses efficacy III - Assess effectiveness - Typically involves 100-1000's of people, often as part of a randomised controlled trial, comparing new treatment with established treatments IV - Postmarketing surveillance - Monitors for long-term effectiveness and side-effects

Question 72

What drugs induce impaired glucose tolerance?

Answer 72

``` thiazides, furosemide (less common) steroids tacrolimus, ciclosporin interferon-alpha nicotinic acid antipsychotics ``` Beta-blockers cause a slight impairment of glucose tolerance. They should also be used with caution in diabetics as they can interfere with the metabolic and autonomic responses to hypoglycaemia

Question 73

What drugs cause urinary retention?

Answer 73

``` tricyclic antidepressants e.g. amitriptyline anticholinergics opioids NSAIDs disopyramide ```

Question 74

What are the causes of massive splenomegaly?

Answer 74

``` myelofibrosis chronic myeloid leukaemia visceral leishmaniasis (kala-azar) malaria Gaucher's syndrome ```

Question 75

What drugs lengthen the QT interval?

Answer 75

amiodarone ciprofloxacin antipsychotics tricyclic antidepressants

Question 76

What are the causes of left axis deviation?

Answer 76

left anterior hemiblock left bundle branch block inferior myocardial infarction Wolff-Parkinson-White syndrome* - right-sided accessory pathway hyperkalaemia congenital: ostium primum ASD, tricuspid atresia minor LAD in obese people

Question 77

What are the causes of right axis deviation?

Answer 77

``` right ventricular hypertrophy left posterior hemiblock lateral myocardial infarction chronic lung disease → cor pulmonale pulmonary embolism ostium secundum ASD Wolff-Parkinson-White syndrome* - left-sided accessory pathway normal in infant < 1 years old minor RAD in tall people ```

Question 78

What condition is caused by Onchocerca volvulus?

Answer 78

Causes 'river blindness'. Spread by female blackflies Features include blindness, hyperpigmented skin and possible allergic reaction to microfilaria rIVERblidness = IVERmectin

Question 79

What are the causes of a loud S2?

Answer 79

Causes of a loud S2 - hypertension: systemic (loud A2) or pulmonary (loud P2) - hyperdynamic states - atrial septal defect without pulmonary hypertension

Question 80

What are the side effects of amiodarone?

Answer 80

``` Thyroid dysfunction: both hyper and hypothyroidism Corneal deposits Pulmonary fibrosis/ pneumonitis Liver fibrosis / hepatitis Peripheral neuropathy, myopathy Photosensitivity "slate grey" appearance Thrombophlebitis and injection site reactions Bradycardia lengths QT interval ``` SLATE TAN - Slate gray - Liver damage - Ataxia/ arrhythmias - Thyroid dysfunction - Eye/ corneal reversible microdeposits - Taste disturbance - Alveolitis - Neuropathy

Question 81

What infections occur at CD4 count 200-500?

Answer 81

Oral Thrush Hairy Leukoplakia Kaposi sarcoma Shingles

Question 82

What infections occur at CD4 count 100-200?

Answer 82

Cryptosporidiosis -(Whilst patients with a CD4 count of 200-500 may develop cryptosporidiosis the disease is usually self-limiting and similar to that in immunocompetent hosts) Cerebral toxoplasmosis Progressive multifocal leukoencephalopathy -(Secondary to the JC virus) Pneumocystis jirovecii pneumonia HIV dementia

Question 83

What infections occur at CD4 count 50-100?

Answer 83

Aspergillosis -(Secondary to Aspergillus fumigatus) Oesophageal candidiasis -(Secondary to Candida albicans) Cryptococcal meningitis Primary CNS lymphoma -(Secondary to EBV)

Question 84

What infections occur at CD4 count <50?

Answer 84

Cytomegalovirus retinitis -(Affects around 30-40% of patients with CD4 < 50 cells/mm³) Mycobacterium avium-intracellulare infection

Question 85

Name the common autosomal recessive conditions

Answer 85

``` Albinism Ataxic telangiectasia Congenital adrenal hyperplasia Cystic fibrosis Cystinuria Familial Mediterranean Fever Fanconi anaemia Friedreich's ataxia Gilbert's syndrome* Glycogen storage disease Haemochromatosis Homocystinuria Lipid storage disease: Tay-Sach's, Gaucher, Niemann-Pick Mucopolysaccharidoses: Hurler's PKU Sickle cell anaemia Thalassaemias Wilson's disease ```

Question 86

What is the equation for sensitivity?

Answer 86

Sensitivity TP / (TP + FN ) Proportion of patients with the condition who have a positive test result ``` TP = True Positive FN = False Negative ```

Question 87

What is the equation for specificity?

Answer 87

Specificity TN / (TN + FP) Proportion of patients without the condition who have a negative test result ``` TN = True Negative FP = False Positive ```

Question 88

What is the equation for positive predictive value?

Answer 88

Positive predictive value TP / (TP + FP) The chance that the patient has the condition if the diagnostic test is positive ``` TP = True Positive FP = False Positive ```

Question 89

What is the equation for negative predictive value?

Answer 89

Negative predictive value TN / (TN + FN) The chance that the patient does not have the condition if the diagnostic test is negative ``` TN = True Negative FN = False Negative ```

Question 90

What is the equation for likelihood ratio for a positive test result?

Answer 90

Likelihood ratio for a positive test result sensitivity / (1 - specificity) How much the odds of the disease increase when a test is positive

Question 91

What is the equation for likelihood ratio for a negative test result?

Answer 91

Likelihood ratio for a negative test result (1 - sensitivity) / specificity How much the odds of the disease decrease when a test is negative

Question 92

What are the clinical features of Alport Syndrome?

Answer 92

- Microscopic haematuria - Progressive renal failure - Bilateral sensorineural deafness - Lenticonus: protrusion of the lens surface into the anterior chamber - Retinitis pigmentosa - Renal biopsy: splitting of lamina densa seen on electron microscopy

Question 93

What are the stages of the cardiac action potential?

Answer 93

0 - Rapid depolarisation - Rapid sodium influx - These channels automatically deactivate after a few ms 1 - Early repolarisation - Efflux of potassium 2 - Plateau - Slow influx of calcium 3 - Final repolarisation - Efflux of potassium 4 - Restoration of ionic concentrations - Resting potential is restored by Na+/K+ ATPase - There is slow entry of Na+ into the cell decreasing the potential difference until the threshold potential is reached, triggering a new action potential

Question 94

What are the causes of Type 2 RTA?

Answer 94

``` idiopathic, as part of Fanconi syndrome, Wilson's disease, cystinosis, outdated tetracyclines, carbonic anhydrase inhibitors (acetazolamide, topiramate) ```

Question 95

What are the clinical features of tuberous sclerosis?

Answer 95

Cutaneous features - depigmented 'ash-leaf' spots which fluoresce under UV light - roughened patches of skin over lumbar spine (Shagreen patches) - adenoma sebaceum (angiofibromas): butterfly distribution over nose - fibromata beneath nails (subungual fibromata) - café-au-lait spots* may be seen Neurological features - developmental delay - epilepsy (infantile spasms or partial) - intellectual impairment Also - retinal hamartomas: dense white areas on retina (phakomata) - rhabdomyomas of the heart - gliomatous changes can occur in the brain lesions - polycystic kidneys, renal angiomyolipomata - lymphangioleiomyomatosis: multiple lung cysts

Question 96

What are the clinical features and treatment of organophosphate insecticide poisoning?

Answer 96

Features can be predicted by the accumulation of acetylcholine (mnemonic = SLUD) - Salivation - Lacrimation - Urination - Defecation/diarrhoea cardiovascular: hypotension, bradycardia also: small pupils, muscle fasciculation Management - atropine - the role of pralidoxime is still unclear - meta-analyses to date have failed to show any clear benefit

Question 97

What is the initial blind therapy of infective endocarditis?

Answer 97

Native valve amoxicillin, consider adding low-dose gentamicin If penicillin allergic, MRSA or severe sepsis vancomycin + low-dose gentamicin If prosthetic valve vancomycin + rifampicin + low-dose gentamicin

Question 98

What are the causes of raised prolactin?

Answer 98

``` Prolactinoma Pregnancy Oestrogens Physiological (Stress, exercise, sleep) Acromegaly (1/3 of patients) PCOS Primary hypothyroidism (due to TRH stimulating prolactin release ``` Drug causes: -Metoclopromide, domperidone Phenothiazines haloperidol

Question 99

What causes increased BNP levels?

Answer 99

``` left ventricular hypertrophy ischaemia tachycardia right ventricular overload hypoxaemia (including PE) GFR <60 Sepsis COPD Diabetes Age >70 Liver cirrhosis ```

Question 100

What causes decreased BNP levels?

Answer 100

``` Obesity Diuretics ACEi/ ARBs B blockers Aldosterone antagonists ```

Question 101

What are the causes of restrictive cardiomyopathy?

Answer 101

Amyloidosis (e.g. secondary to myeloma) - most common cause in UK Haemochromatosis Post radiation fibrosis Loffler's syndrome -endomyocardial fibrosis with a prominent eosinophillic infiltrate Endocardial fibroelastosis - Thick fibroelastic tissue forms in the endocardium - Most commonly seen in young children Sarcoidosis Scleroderma

Question 102

What is the management of ITP?

Answer 102

Platelet >30 - Observe Platelet <30 - ORAL prednisolone ``` Emergency treatment (life threatening or organ threatening bleeding -Platelet transfusion, IV methylprednisolone and IV immunogolulin ```

Question 103

What are the causes of cranial Diabetes insipidus?

Answer 103

``` idiopathic post head injury pituitary surgery craniopharyngiomas histiocytosis x DIDMOAD (Wolfram's syndrome) Haemochromatosis ```

Question 104

What is macrophage activation syndrome?

Answer 104

Uncontrolled hyper-inflammatory state associated with many systemic autoimmune diseases but in particular JIA. ACR/EULAR classification criteria for macrophage activation syndrome (MAS) state that in a patient with JIA who presents with a fever, a diagnosis of MAS can be made if the ferritin level is > 684 ng/ml and any two of the following are present (platelets < 181 * 109/L, AST > 48 U/L, triglycerides > 156 mg/dl, fibrinogen < 360 mg/dl). Refractory fever and hepatosplenomegaly are typical clinical features. Interferon-gamma is responsible for the activation of macrophages and is heavily implicated in the pathogenesis of this condition.

Question 105

What is the incidence of down's syndrome by age?

Answer 105

30 years 1/1000 35 years 1/300 40 years 1/100 45 years 1/30 Divide by 30 for each 5 years

Question 106

What are the different types of thyroid cancer?

Answer 106

Papillary carcinoma (70%) - Usually contain a mixture of papillary and colloidal filled follicles - Histologically tumour has papillary projections and pale empty nuclei - Seldom encapsulated - Lymph node metastasis predominate - Haematogenous metastasis rare Follicular adenoma (20% for all follicular) - Usually present as a solitary thyroid nodule - Malignancy can only be excluded on formal histological assessment - Follicular carcinoma - May appear macroscopically encapsulated, microscopically capsular invasion is seen. Without this finding the lesion is a follicular adenoma. - Vascular invasion predominates - Multifocal disease raree Medullary carcinoma - C cells derived from neural crest and not thyroid tissue - Serum calcitonin levels often raised - Familial genetic disease accounts for up to 20% cases - Both lymphatic and haematogenous metastasis are recognised, nodal disease is associated with a very poor prognosis. Anaplastic carcinoma (1%) - Most common in elderly females - Local invasion is a common feature - Treatment is by resection where possible, palliation may be achieved through isthmusectomy and radiotherapy. - Chemotherapy is ineffective. Lymphoma (rare) -Associated with hashimoto's thyroiditis

Question 107

What may precipitate lithium toxicity?

Answer 107

dehydration renal failure drugs: diuretics (especially thiazides), ACE inhibitors/angiotensin II receptor blockers, NSAIDs and metronidazole.

Question 108

What are the features of Wernicke's Encephalopathy?

Answer 108

A useful mnemonic to remember the features of Wernicke's encephalopathy is CAN OPEN ``` Confusion Ataxia Nystagmus Ophthamoplegia PEripheral Neuropathy ```

Question 109

When do you give dexamethasone for meningitis?

Answer 109

NICE guidelines recommend treating bacterial meningitis with dexamethasone if lumbar puncture reveals either: purulent CSF, white cell count > 1000 cells/µL, raised white cell count plus protein count > 1g/L, or bacteria on gram stain. Dexamethasone should be given within 4 hours of starting antibiotics and should be avoided if the duration from starting antibiotics is beyond 12 hours.

Question 110

What is cardiac syndrome X?

Answer 110

Cardiac syndrome X - also called microvascular angina. Patients have a normal ECG at rest and normal coronary arteries but develop ST depression on exercise stress testing.

Question 111

What are the diagnostic criteria for tumour lysis syndrome?

Answer 111

From 2004 TLS has been graded using the Cairo-Bishop scoring system - Laboratory tumor lysis syndrome: abnormality in two or more of the following, occurring within three days before or seven days after chemotherapy. - uric acid > 475umol/l or 25% increase - potassium > 6 mmol/l or 25% increase - phosphate > 1.125mmol/l or 25% increase - calcium < 1.75mmol/l or 25% decrease Clinical tumor lysis syndrome: laboratory tumour lysis syndrome plus one or more of the following: - increased serum creatinine (1.5 times upper limit of normal) - cardiac arrhythmia or sudden death - seizure

Question 112

What are the most common causes of endocarditis?

Answer 112

Staphylococcus aureus Staphylococcus epidermidis if < 2 months post valve surgery

Question 113

What are the features of MEN type 1?

Answer 113

Parathyroid (95%): hyperparathyroidism due to parathyroid hyperplasia Pituitary (70%) Pancreas (50%): e.g. insulinoma, gastrinoma (leading to recurrent peptic ulceration) Also: adrenal and thyroid

Question 114

What are the features of MEN type IIa?

Answer 114

Medullary thyroid cancer (70%) 2 P's Parathyroid (60%) Phaeochromocytoma

Question 115

What are the features of MEN type IIb

Answer 115

Medullary thyroid cancer 1 P Phaeochromocytoma Marfanoid body habitus Neuromas

Question 116

What is the management of trigeminal neuralgia?

Answer 116

carbamazepine is first-line failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology

Question 117

What is anakinra?

Answer 117

Anakinra is an interleukin-1 (IL-1) receptor antagonist. Interleukin-1 refers to a 'superfamily' of 11 cytokines that are responsible for acute inflammation and inducing fever. Blocking IL-1 with anakinra is beneficial in pro-inflammatory conditions such as rheumatoid arthritis. Il-1 is predominantly secreted by macrophages.

Question 118

What are human herpes virus (HHV): ``` HHV 2 HHV 3 HHV 4 HHV 5 HHV 6 and 7 HHV 8 ```

Answer 118

Human herpesvirus virus 2 (HHV-2) also commonly known as herpes simplex virus-2 (HSV-2) causes oral and/or genital herpes, therefore this is the incorrect answer in this case. Human herpesvirus virus 3 (HHV-3) also commonly known as varicella-zoster virus (VZV) causes chickenpox and shingles, therefore this is the incorrect answer in this case. Human herpesvirus virus 4 (HHV-4) also commonly known as Epstein–Barr virus (EBV) causes Epstein-Barr virus-associated lymphoproliferative diseases, therefore this is the incorrect answer in this case. Pityriasis rosea is associated with the reactivation of herpesviruses 6 and 7. Influenza viruses and vaccines have triggered pityriasis rosea in some cases. The herald patch is a single plaque that appears 1–20 days prior to the generalised rash of pityriasis rosea. Human herpesvirus virus 8 (HHV-8) also commonly known as Kaposi's sarcoma-associated herpesvirus causes Kaposi's sarcoma, therefore this is the incorrect answer in this case.

Question 119

t(9;22) is seen in which malignancy?

Answer 119

t(9;22) - Philadelphia chromosome present in > 95% of patients with CML this results in part of the Abelson proto-oncogene being moved to the BCR gene on chromosome 22 the resulting BCR-ABL gene codes for a fusion protein which has tyrosine kinase activity in excess of normal poor prognostic indicator in ALL

Question 120

t(15;17) is seen in which malignancy?

Answer 120

t(15;17) seen in acute promyelocytic leukaemia (M3) fusion of PML and RAR-alpha genes

Question 121

t(8;14) is seen in which malignancy?

Answer 121

t(8;14) seen in Burkitt's lymphoma MYC oncogene is translocated to an immunoglobulin gene

Question 122

t(11;14) is seen in which malignancy?

Answer 122

t(11;14) Mantle cell lymphoma deregulation of the cyclin D1 (BCL-1) gene

Question 123

t(14;18) is seen in which malignancy?

Answer 123

t(14;18) follicular lymphoma (seen in 90%) increased BCL-2 transcription

Question 124

What are the different strengths of steroid creams and give examples

Answer 124

Mild -Hydrocortisone 0.5-2.5% Moderate - Betamethasone valerate 0.025% (Betnovate RD) - Clobetasone butyrate 0.05% (Eumovate) Potent - Fluticasone propionate 0.05% (Cutivate) - Betamethasone valerate 0.1% (Betnovate) Very Potent -Clobetasol propionate 0.05% (Dermovate)

Question 125

What are the side effects of the TB drugs?

Answer 125

Rifampicin - mechanism of action: inhibits bacterial DNA dependent RNA polymerase preventing transcription of DNA into mRNA - potent liver enzyme inducer - hepatitis, orange secretions - flu-like symptoms Isoniazid - mechanism of action: inhibits mycolic acid synthesis - peripheral neuropathy: prevent with pyridoxine (Vitamin B6) - hepatitis, agranulocytosis - liver enzyme inhibitor Pyrazinamide - mechanism of action: converted by pyrazinamidase into pyrazinoic acid which in turn inhibits fatty acid synthase (FAS) I - hyperuricaemia causing gout - arthralgia, myalgia - hepatitis Ethambutol - mechanism of action: inhibits the enzyme arabinosyl transferase which polymerizes arabinose into arabinan - optic neuritis: check visual acuity before and during treatment - dose needs adjusting in patients with renal impairment

Question 126

Give 2 sulphonureas

Answer 126

glimepiride and glipizide

Question 127

What is the mechanism of action of Dipyridamole?

Answer 127

inhibits phosphodiesterase, elevating platelet cAMP levels which in turn reduce intracellular calcium levels other actions include reducing cellular uptake of adenosine and inhibition of thromboxane synthase

Question 128

What organisms are splenectomy patients vulnerable to?

Answer 128

Yes Some Nasty Killer Bacteria Have Some Capsule(2) Protection.... ``` Yesenia S.pneumonia N.meningitidis K.pneumonia Bacillus H.influenza (not covered by Pen V) S.typhi Clostridium and C.neoformans P.aeruginosa ```

Question 129

How do you treat chronic plaque psoriasis?

Answer 129

regular emollients may help to reduce scale loss and reduce pruritus first-line: NICE recommend: - a potent corticosteroid applied once daily plus vitamin D analogue applied once daily - should be applied separately, one in the morning and the other in the evening) - for up to 4 weeks as initial treatment second-line: if no improvement after 8 weeks then offer: -a vitamin D analogue twice daily third-line: if no improvement after 8-12 weeks then offer either: -a potent corticosteroid applied twice daily for up to 4 weeks, or -a coal tar preparation applied once or twice daily short-acting dithranol can also be used

Question 130

How can you divide hypokalaemia and blood pressure?

Answer 130

Hypokalaemia with hypertension - Cushing's syndrome - Conn's syndrome (primary hyperaldosteronism) - Liddle's syndrome - 11-beta hydroxylase deficiency* Carbenoxolone, an anti-ulcer drug, and liquorice excess can potentially cause hypokalaemia associated with hypertension Hypokalaemia without hypertension - diuretics - GI loss (e.g. Diarrhoea, vomiting) - renal tubular acidosis (type 1 and 2**) - Bartter's syndrome - Gitelman syndrome

Question 131

What is the treatment for acromegaly?

Answer 131

Trans-sphenoidal surgery is the first-line treatment for acromegaly in the majority of patients. If a pituitary tumour is inoperable or surgery unsuccessful then medication may be indicated: somatostatin analogue directly inhibits the release of growth hormone for example octreotide effective in 50-70% of patients pegvisomant GH receptor antagonist - prevents dimerization of the GH receptor once daily s/c administration very effective - decreases IGF-1 levels in 90% of patients to normal doesn't reduce tumour volume therefore surgery still needed if mass effect dopamine agonists for example bromocriptine the first effective medical treatment for acromegaly, however now superseded by somatostatin analogues effective only in a minority of patients External irradiation is sometimes used for older patients or following failed surgical/medical treatment

Question 132

What are the tumour in Von Hippel Lindau syndrome?

Answer 132

PEE BREAK ``` Pancreas (benign cystic or malignant neurendocrine) Epididymal cystadenomas Endolymphatic sac (inner ear) ``` Brain (CNS haemangioblastoma in 70%) Retinal haemangioblastoma (in 60%) Ears (enodlymphatic sac as above, just to make the mnemonic work lol) Adrenal (phaeochromocytoma, may be intra- or extra-adrenal) Kidney (RCC and cystic)

Question 133

What drugs must be stopped in pregnancy?

Answer 133

Antibiotics - tetracyclines - aminoglycosides - sulphonamides and trimethoprim - quinolones: the BNF advises to avoid due to arthropathy in some animal studies Other drugs - ACE inhibitors, angiotensin II receptor antagonists - statins - warfarin - sulfonylureas - retinoids (including topical) - cytotoxic agents - Bosentan

Question 134

What are the clinical features of polyarteritis nodosa?

Answer 134

- fever, malaise, arthralgia - weight loss - hypertension - mononeuritis multiplex, sensorimotor polyneuropathy - testicular pain - livedo reticularis - haematuria, renal failure - perinuclear-antineutrophil cytoplasmic antibodies (ANCA) -are found in around 20% of patients with 'classic' PAN - hepatitis B serology positive in 30% of patients

Question 135

What is the mechanism of action of baclofen?

Answer 135

Baclofen is an agonist of GABA receptors and is used as a muscle relaxant to treat spasticity conditions such as multiple sclerosis and cerebral palsy.

Question 136

What is the mechanism of action of flumazenil.

Answer 136

An example of a GABA antagonist is flumazenil. Baclofen is an agonist rather than antagonist at GABA receptors.

Question 137

What is the mechanism of action of buscopan?

Answer 137

Muscarinic M3 receptor antagonist is buscopan, used to treat pain associated with bowel wall spasm and respiratory secretions during end-of-life care

Question 138

What are the trinucleotide repeats for: - Huntington's disease - Friedrich Ataxia - Myotonic dystrophy - Fragile X syndrome

Answer 138

CAG: Huntington's disease GAA: Friedrich Ataxia CTG: Myotonic dystrophy CGG: Fragile X syndrome

Question 139

Give an example of entry inhibitors in HIV. When are they used?

Answer 139

Maraviroc (binds to CCR5, preventing an interaction with gp41), Enfuvirtide (binds to gp41, also known as a 'fusion inhibitor') Prevent HIV-1 from entering and infecting immune cells Tend to be used in patients with treatment-resistant HIV with persistent high viral load and/or low CD4 count.

Question 140

What area of the brain if affected in internuclear opthalmoplegia?

Answer 140

The medial longitudinal fasciculus is located in the paramedian area of the midbrain and pons

Question 141

What drugs exacerbate myasthenia gravis?

Answer 141

``` penicillamine quinidine, procainamide beta-blockers lithium phenytoin antibiotics: gentamicin, macrolides, quinolones, tetracyclines ```

Question 142

What is the highest to lowest proportions of immunoglobulins found in blood?

Answer 142

GAMDE

Question 143

Give an example of alkylating chemotherapy. What is its mechanism of action and side effect?

Answer 143

Cyclophosphamide Alkylating agent - causes cross-linking in DNA Haemorrhagic cystitis, myelosuppression, transitional cell carcinoma

Question 144

What is the mechanism of action of anthracyclines? | Give an example

Answer 144

Anthracyclines (e.g doxorubicin) Stabilizes DNA-topoisomerase II complex inhibits DNA & RNA synthesis Cardiomyopathy

Question 145

What is the mechanism of action of fluorouracil (5-FU)?

Answer 145

Pyrimidine analogue inducing cell cycle arrest and apoptosis by blocking thymidylate synthase (works during S phase)

Question 146

What is the mechanism of action of 6-mercaptopurine?

Answer 146

Purine analogue that is activated by HGPRTase, decreasing purine synthesis

Question 147

What is the mechanism of action of cytarabine?

Answer 147

Pyrimidine antagonist. Interferes with DNA synthesis specifically at the S-phase of the cell cycle and inhibits DNA polymerase

Question 148

What are the causes of membranous glomerulonephropathy?

Answer 148

Membranous nephropathy is split into two main causes - primary and secondary. Primary membranous nephropathy is most commonly associated with anti-PLA2R antibodies. Secondary membranous nephropathy means another process is linked with its development. The most common processes are: - Malignancy such as solid tumours (lung, colon, breast, kidney) - Infections: hepatitis B or C, HIV, malaria, syphilis, schistosomiasis - Autoimmune diseases: SLE, sarcoidosis, IBD - Drugs: NSAID's, captopril, gold, penicillamine, lithium, clopidogrel

Question 149

What is the genetics of Autosomal polycystic kidney disease?

Answer 149

ADPKD Type 1 - 85% of cases - Chromosome 16 - Presents with renal failure earlier ADPKD Type 2 - 15% of cases - Chromosome 4

Question 150

What is the treatment for Tetanus?

Answer 150

Metronidazole is now preferred to benzylpenicillin

Question 151

What is the mechanism of action or irinotecan?

Answer 151

Inhibition of topoisomerase I. By doing so, it prevents the relaxation of supercoiled DNA. Adverse effects include myelosuppression. It is usually used for colon cancer.

Question 152

What are the poor prognostic factors in Rheumatoid arthritis?

Answer 152

``` Rheumatoid factor positive Anti-CCP antibodies Poor functional status at presentation X-ray: early erosions (e.g. after <2 years) Extra articulate features e.g. nodes HLA DR4 Insidious onset ```

Question 153

What are the Kings’s College Hospital criteria for liver transplantation?

Answer 153

Arterial pH < 7.3, 24 hours after ingestion Or all of the following - Prothrombin time >100 seconds - Creatinine > 300 - Grade III or IV encephalopathy

Question 154

What foods are high in potassium? Avoid in renal failure

Answer 154

``` Bananas Oranges Kiwi fruit Avocado Spinach Tomatoes ```

Question 155

What is associated with HLA-A3?

Answer 155

Haemachromatosis

Question 156

What is associated with HLA-B51?

Answer 156

Behçet’s disease

Question 157

What is associated with HLA-B27?

Answer 157

Ankylosis spondylitis Reactive arthritis Acute anterior uveitis

Question 158

What is associated with HLA-DQ2/DQ8?

Answer 158

Coeliac disease

Question 159

What is associated with HLA-DR2?

Answer 159

Narcolepsy | Goodpasture’s

Question 160

What is associated with HLA-DR4

Answer 160

``` Type 1 DM (also associated with DR3 but more strongly DR4) Rheumatoid arthritis (in particular the DRB1 gene) ```

Question 161

What are the features of subacute combined degeneration of the cord? What are nervous system areas they relate to?

Answer 161

Damage to posterior columns - loss of proprioception, light touch and vibration sensation (sensory ataxia and positive Romberg’s test) Damage to lateral columns - spastic weakness and up going planters (UMN signs) Damage to peripheral nerves - absent ankle and knee jerks (LMN signs)

Question 162

What does C1 deficiency cause?

Answer 162

Hereditary angioedema C1-INH is a multi factorial serine protease inhibitor Probable mechanism is uncontrolled release of bradykinin resulting in oedema in tissues

Question 163

C1q, C1rs, C2, C4 deficiency (classical pathway components) cause what?

Answer 163

Immune complex disease | E.g. SLE, henoch-schonlein purpura

Question 164

C3 deficiency causes what?

Answer 164

Recurrent bacterial infections

Question 165

C5-9 deficiency causes what?

Answer 165

Encodes the membrane attack complex (MAC) Particularly prone to Neisseria meningitidis infection

Question 166

What is the mechanism of action of cyclosporin?

Answer 166

Ciclosporin is an immunosuppressant which decreases clonal proliferation of T cells by reducing IL-2 release. It acts by binding to cyclophilin forming a complex which inhibits calcineurin, a phosphatase that activates various transcription factors in T cells

Question 167

What are the indications for a ICD?

Answer 167

``` long QT syndrome hypertrophic obstructive cardiomyopathy previous cardiac arrest due to VT/VF previous myocardial infarction with non-sustained VT on 24 hr monitoring, inducible VT on electrophysiology testing and ejection fraction < 35% Brugada syndrome ```

Question 168

What blood investigations are raised in Paget's Disease of the bone?

Answer 168

Increased alk phos. Otherwise bone profile and vitamin D is normal. Other markers of bone turnover including hydroxyproline, which is raised in both the urine and serum, as well as procollagen type I N-terminal propeptide (PINP), C-telopeptide (CTx) and N-telopeptide (NTx).

Question 169

What is the mechanism of action of amantadine? What does it act against?

Answer 169

inhibits uncoating (M2 protein) of virus in cell. Also releases dopamine from nerve endings "Amanta doesn't want to take her coat off" Influenza, Parkinson's disease

Question 170

What are the types of T helper cell and what do they secrete?

Answer 170

Th1 involved in the cell-mediated response and delayed (type IV) hypersensitivity secrete IFN-gamma, IL-2, IL-3 Th2 involved in mediating humoral (antibody) immunity e.g. stimulating production of IgE in asthma secrete IL-4, IL-5, IL-6, IL-10, IL-13

Question 171

What factors increase the risk of warfarin induced skin necrosis?

Answer 171

Inherited deficiency of Protein C, Protein S or Factor V Leiden Mutations in methylene tetrahydrofolate reductase gene causing hyperhomocysteinaemia Antithrombin III deficiency Antiphospholipid antibodies.

Question 172

What is the classification of SLE renal disease?

Answer 172

``` WHO classification class I: normal kidney class II: mesangial glomerulonephritis class III: focal (and segmental) proliferative glomerulonephritis class IV: diffuse proliferative glomerulonephritis class V: diffuse membranous glomerulonephritis class VI: sclerosing glomerulonephritis ``` Class IV (diffuse proliferative glomerulonephritis) is the most common and severe form. Renal biopsy characteristically shows the following findings: glomeruli shows endothelial and mesangial proliferation, 'wire-loop' appearance if severe, the capillary wall may be thickened secondary to immune complex deposition electron microscopy shows subendothelial immune complex deposits granular appearance on immunofluorescence

Question 173

What are the features of severe malaria?

Answer 173

``` schizonts on a blood film parasitaemia > 2% hypoglycaemia acidosis temperature > 39 °C severe anaemia complications as below ``` Complications - cerebral malaria: seizures, coma - acute renal failure: blackwater fever, secondary to intravascular haemolysis, mechanism unknown - acute respiratory distress syndrome (ARDS) - hypoglycaemia - disseminated intravascular coagulation (DIC)

Question 174

What is the target INR for mechanical aortic and mitral valves?

Answer 174

``` Aortic = 3.0 Mitral = 3.5 ```

Question 175

What antibiotics inhibit cell wall synthesis?

Answer 175

penicillins: binds transpeptidase blocking cross-linking of peptidoglycan cell walls cephalosporins

Question 176

What antibiotics inhibit protein synthesis?

Answer 176

``` aminoglycosides (cause misreading of mRNA) chloramphenicol macrolides (e.g. erythromycin) tetracyclines fusidic acid ``` These antibiotics are bacteriostatic

Question 177

What antibiotics inhibit DNA synthesis?

Answer 177

quinolones (e.g. ciprofloxacin) metronidazole sulphonamides trimethoprim

Question 178

What do prostaglandin analogues do in primary open angle glaucoma?

Answer 178

e.g. latanoprost Increase uveoscleral outflow Once daily administration Adverse effects include brown pigmentation of the iris and increased eyelash length

Question 179

What is the mechanism of action of amantadine?

Answer 179

inhibits uncoating (M2 protein) of virus in cell. Also releases dopamine from nerve endings "Amanta doesn't want to take her coat off"

Question 180

What is the mechanism of action of aciclovir? What does it act against?

Answer 180

Guanosine analog, phosphorylated by thymidine kinase which in turn inhibits the viral DNA polymerase HSV, VZV

Question 181

What is the mechanism of action of ganciclovir? What does it act against?

Answer 181

Guanosine analog, phosphorylated by thymidine kinase which in turn inhibits the viral DNA polymerase CMV

Question 182

What is the mechanism of action of Ribavirin? What does it act against?

Answer 182

Guanosine analog which inhibits inosine monophosphate (IMP) dehydrogenase, interferes with the capping of viral mRNA Chronic hep C RSV

Question 183

What is the mechanism of action of Oseltamivir? What does it act against?

Answer 183

Inhibits neuraminidase Influenza

Question 184

What is the mechanism of action of docarnet? What does it act against?

Answer 184

Pyrophosphate analog which inhibits viiral DNA polymerase CMV HSV if not responding to aciclovir

Question 185

What is the mechanism of action of Interferon-a? What does it act against?

Answer 185

Human glycoproteins which inhibit synthesis of mRNA Chronic hepatitis B & C, hairy cell leukaemia

Question 186

What is the mechanism of action of cidofovir? What does it act against?

Answer 186

Acyclic nucleoside phosphonate, and is therefore independent of phosphorylation by viral enzymes (compare and contrast with aciclovir/ganciclovir) CMV retinitis in HIV

Question 187

What are the two types of mesangiocapillary glomerulonephritis (membranoproliferative) and what are they associated with?

Answer 187

Type 1: cryoglobulinaemia, Hepatitis C Type 2: Partial lipodystrophy

Question 188

What do prostaglandin analogues do?

Answer 188

e.g. latanoprost Increase uveoscleral outflow Once daily administration Adverse effects include brown pigmentation of the iris and increased eyelash length

Question 189

What is the mechanism of action of beta blockers in primary open angle glaucoma?

Answer 189

e.g. timolol, betaxolol Reduces aqueous production Should be avoided in patients with asthma and heart block

Question 190

What is the mechanism of action of sympathomimetics in primary open angle glaucoma?

Answer 190

(e.g. brimonidine, an alpha2-adrenoreceptor agonist) Reduces aqueous production and increases outflow Avoid if taking MAOI or TCAs Adverse effects include hyperaemia

Question 191

What is the mechanism of action of carbonic anhydraze inhibitors in primary open angle glaucoma?

Answer 191

(e.g. Dorzolamide) Reduces aqueous production Systemic absorption may cause sulphonamide like reactions

Question 192

What is the mechanism of miotics?

Answer 192

e.g. pilocarpine (a muscarinic receptor agonist) Increases uveoscleral outflow Adverse effects include a constricted pupil, headache and blurred vision

Question 193

What is the criteria for IVIG in varicella zoster exposure?

Answer 193

The following criteria should be met to determine who would benefit from active post-exposure prophylaxis: 1. significant exposure to chickenpox or herpes zoster e.g. exposure to limited, covered-up shingles may not warrant post-exposure prophylaxis 2. a clinical condition that increases the risk of severe varicella; this includes immunosuppressed patients, neonates and pregnant women e.g. long-term steroids, methotrexate and other common immunosuppressants 3. no antibodies to the varicella virus ideally all at-risk exposed patients should have a blood test for varicella antibodies this should not, however, delay post-exposure prophylaxis past 7 days after initial contact Patients who fulfill the above criteria should be given varicella-zoster immunoglobulin (VZIG).

Question 194

What causes a raised TLCO?

Answer 194

``` asthma pulmonary haemorrhage (Wegener's, Goodpasture's) left-to-right cardiac shunts polycythaemia hyperkinetic states male gender, exercise ```

Question 195

What causes a lower TLCO?

Answer 195

``` pulmonary fibrosis pneumonia pulmonary emboli pulmonary oedema emphysema anaemia low cardiac output ```

Question 196

What causes an increased KCO with normal or reduced TLCO?

Answer 196

KCO also tends to increase with age. Some conditions may cause an increased KCO with a normal or reduced TLCO pneumonectomy/lobectomy scoliosis/kyphosis neuromuscular weakness ankylosis of costovertebral joints e.g. ankylosing spondylitis

Question 197

What drugs cause cholestasis (+/- hepatitis)?

Answer 197

combined oral contraceptive pill antibiotics: flucloxacillin, co-amoxiclav, erythromycin* anabolic steroids, testosterones phenothiazines: chlorpromazine, prochlorperazine sulphonylureas fibrates rare reported causes: nifedipine

Question 198

What drugs cause a hepatocellular picture?

Answer 198

``` paracetamol sodium valproate, phenytoin MAOIs halothane anti-tuberculosis: isoniazid, rifampicin, pyrazinamide statins alcohol amiodarone methyldopa nitrofurantoin ```

Question 199

How do you manage chickenpox EXPOSURE in pregnancy?

Answer 199

if there is any doubt about the mother previously having chickenpox maternal blood should be urgently checked for varicella antibodies if the pregnant woman <= 20 weeks gestation is not immune to varicella she should be given varicella-zoster immunoglobulin (VZIG) as soon as possible RCOG and Greenbook guidelines suggest VZIG is effective up to 10 days post exposure if the pregnant woman > 20 weeks gestation is not immune to varicella then either VZIG or antivirals (aciclovir or valaciclovir) should be given days 7 to 14 after exposure the Public Health England (PHE) guidelines state that 'The decision on choice of PEP for women exposed from 20 weeks of pregnancy should take into account patient and health professional preference as well as the ability to offer and provide PEP in a timely manner' why wait until days 7-14? From the PHE guidelines: 'In a study evaluating the comparative effectiveness of 7 days course of aciclovir given either immediately after exposure or starting at day 7 after exposure to healthy children, the incidence and severity of varicella infection was significantly higher in those given aciclovir immediately (10/13 (77%) who received aciclovir immediately developed clinical varicella compared with 3/14 (21%) who started aciclovir at day 7)' it seems part of this guidance is related to a limited supply of VZIG within the NHS

Question 200

What are the poor prognostic factors in CLL? (median survival 3-5 years)

Answer 200

``` male sex age > 70 years lymphocyte count > 50 prolymphocytes comprising more than 10% of blood lymphocytes lymphocyte doubling time < 12 months raised LDH CD38 expression positive TP53 mutation ``` "A MALE who is OVER 70 with a LYMPHOCYTE COUNT > 50 whose LYMPHOCYTE DOUBLING TIME IS < 12 MONTHS. He has an ELEVATED LDH and POSITIVE CD38 CELLS."

Question 201

What antiepileptics are contraindicated in absence seizures?

Answer 201

Carbamazepine is contraindicated and can worsen absence seizures (along with phenytoin, vigabatrin and gabapentin).

Question 202

What fruit is included in fruit-latex syndrome?

Answer 202

banana, pineapple, avocado, chestnut, kiwi fruit, mango, passion fruit and strawberry.

Question 203

What are the poor prognostic factors for HOCM?

Answer 203

Syncope not chest pain Family history of sudden death Young age at presentation Non-sustained ventricular tachycardia on 24 or 48-hour Holter monitoring Abnormal (↓↓) blood pressure changes on exercise An increased septal wall thickness > 30mm ventricular wall thickness Specific genetic mutations (such as in myosin binding protein C and troponin T)

Question 204

Disorders associated with glomerulonephritis and low serum complement levels?

Answer 204

post-streptococcal glomerulonephritis subacute bacterial endocarditis systemic lupus erythematosus mesangiocapillary glomerulonephritis

Question 205

What conditions cause a demyelinating peripheral neuropathy? (reduced velocity on NCS)

Answer 205

Guillain-Barre syndrome chronic inflammatory demyelinating polyneuropathy (CIDP) amiodarone hereditary sensorimotor neuropathies (HSMN) type I paraprotein neuropathy

Question 206

What conditions cause an axonal peripheral neuropathy? (reduced amplitude on NCS)

Answer 206

``` alcohol diabetes mellitus* vasculitis vitamin B12 deficiency* hereditary sensorimotor neuropathies (HSMN) type II ``` *may also cause demyelinating neuropathy...

Question 207

What chromosome is alpha thalassaemia gene found on?

Answer 207

16

Question 208

What chromosome is beta thalassaemia gene found on?

Answer 208

11

Question 209

What nerve is formed from C8 and T1?

Answer 209

C8 and T1 supply to ulnar nerve. Ulnar nerve injury causes loss of sensation to the medial 1 and ½ fingers and impaired movement of the intrinsic hand muscles.

Question 210

What nerve is formed from C7 and C8?

Answer 210

C7 and C8 supply the radial nerve. The radial nerve may be injured in association with a humeral shaft spiral fracture. Symptoms include loss of sensation to the posterior aspect of the arm and impaired extension of the arm muscles.

Question 211

What nerve is formed from C6, C8 and T1?

Answer 211

C6, C8, and T1 supply the median nerve that provides sensation to the anterior forearm and powers flexion of the wrist, fingers, and pronation of the forearm.

Question 212

What nerve is formed from C3, C4 and C5?

Answer 212

C3, C4, and C5 supply the diaphragm through the phrenic nerve (i.e. C3,4,5 keeps the diaphragm alive). Injury to these nerve roots would cause respiratory depression.

Question 213

What are the risk factors for COPD?

Answer 213

``` Cigarettes Coal Cadmium Cotton Cement Cereals (grain) ```

Question 214

What are the different types of sickle cell trait/ disease genetically?

Answer 214

HbSC disease is the second most common type of sickle cell disease. It occurs when you inherit the Hb S gene from one parent and the Hb C gene from the other. Individuals with HbSC have similar but milder symptoms to individuals with HbSS. Normal adult haemoglobin, Haemoglobin A, is the most common haemoglobin found in adults and is composed of two α and two β-globin chains. If a person inherits a haemoglobin A gene from each parent (HbAA), that person will have normal haemoglobin. Haemoglobin C is an abnormal type of haemoglobin and is a type of haemoglobinopathy. The disease is caused by a problem in the β-globin gene and is one of the most common structural haemoglobin variants. People with haemoglobin C trait (HbAC) are phenotypically normal, with no clinically evident limitations or symptoms. People who inherit one sickle cell gene (HbS) and one normal gene (HbA) have sickle cell trait (HbAS). As sickle cell disease is inherited in an autosomal recessive pattern, people with sickle cell trait usually have no medical problems related to sickle cell trait. HbSS is the most common and most severe type of sickle cell disease. It occurs when you inherit the haemoglobin S gene mutation from both parents. In this type, the body only produces haemoglobin S.

Question 215

Name the Vaughan Williams class 1a

Answer 215

Quinidine Procainamide Disopyramide Block sodium channels Increases AP duration

Question 216

Name the Vaughan Williams class 1b

Answer 216

Lidocaine Mexiletine Tocainide Block sodium channels Decreases AP duration

Question 217

Name the Vaughan Williams class 1c

Answer 217

Flecainide Encainide Propafenone Block sodium channels No effect on AP duration

Question 218

Name the Vaughan Williams class II

Answer 218

Propranolol Atenolol Bisoprolol Metoprolol Beta-adrenoceptor antagonists

Question 219

Name the Vaughan Williams class III

Answer 219

Amiodarone Sotalol Ibutilide Bretylium Block potassium channels

Question 220

Name the Vaughan Williams class Iv

Answer 220

Verapamil Diltiazem Calcium channel blockers

Question 221

How can you differentiate Barter's and Gittleman's syndrome with electrolytes?

Answer 221

Gitelman syndrome always has hypomagnasaemia | Barter's its 30%

Question 222

What are the causes of Chorea?

Answer 222

``` Huntington's disease, Wilson's disease, ataxic telangiectasia SLE, anti-phospholipid syndrome rheumatic fever: Sydenham's chorea drugs: oral contraceptive pill, L-dopa, antipsychotics neuroacanthocytosis pregnancy: chorea gravidarum thyrotoxicosis polycythaemia rubra vera carbon monoxide poisoning cerebrovascular disease ```

Question 223

What is the pathophysiology of chorea?

Answer 223

Chorea is caused by damage to the basal ganglia, especially the caudate nucleus

Question 224

What are the clinical features of hypercalcaemia?

Answer 224

bones, stones, groans and psychic moans' corneal calcification shortened QT interval on ECG hypertension

Question 225

What are the classical features of TTP?

Answer 225

Pentad Microangiopathic haemolytic anaemia, Thrombocytopaenic purpura, Neurological dysfunction, Renal dysfunction and Fever

Question 226

Name the P450 Inducers

Answer 226

antiepileptics: phenytoin, carbamazepine barbiturates: phenobarbitone rifampicin St John's Wort chronic alcohol intake griseofulvin smoking (affects CYP1A2, reason why smokers require more aminophylline)

Question 227

Name the P450 inhibitors

Answer 227

``` antibiotics: ciprofloxacin, erythromycin isoniazid cimetidine,omeprazole amiodarone allopurinol imidazoles: ketoconazole, fluconazole SSRIs: fluoxetine, sertraline ritonavir sodium valproate acute alcohol intake quinupristin ```

Question 228

What are the precipitating factors in digoxin toxicity?

Answer 228

classically: hypokalaemia digoxin normally binds to the ATPase pump on the same site as potassium. Hypokalaemia → digoxin more easily bind to the ATPase pump → increased inhibitory effects increasing age renal failure myocardial ischaemia hypomagnesaemia, hypercalcaemia, hypernatraemia, acidosis hypoalbuminaemia hypothermia hypothyroidism drugs: amiodarone, quinidine, verapamil, diltiazem, spironolactone (competes for secretion in distal convoluted tubule therefore reduce excretion), ciclosporin. Also drugs which cause hypokalaemia e.g. thiazides and loop diuretics

Question 229

What are the causes of a false positive cardiolipin test?

Answer 229

``` pregnancy SLE, anti-phospholipid syndrome TB leprosy malaria HIV ```

Question 230

What tests can be used for syphilis?

Answer 230

Cardiolipin tests syphilis infection leads to the production of non-specific antibodies that react to cardiolipin examples include VDRL (Venereal Disease Research Laboratory) & RPR (rapid plasma reagin) insensitive in late syphilis becomes negative after treatment Treponemal specific antibody tests example: TPHA (Treponema pallidum HaemAgglutination test) remains positive after treatment Therefore, following treatment for syphilis: VDRL becomes negative TPHA remains positive

Question 231

What are the features of pseudoxanthoma elasticum

Answer 231

retinal angioid streaks 'plucked chicken skin' appearance - small yellow papules on the neck, antecubital fossa and axillae cardiac: mitral valve prolapse, increased risk of ischaemic heart disease gastrointestinal haemorrhage

Question 232

What is the treatment for pelvic inflammatory disease?

Answer 232

Treatment of pelvic inflammatory disease: oral ofloxacin + oral metronidazole or intramuscular ceftriaxone + oral doxycycline + oral metronidazole

Question 233

What are the causes of raynauds?

Answer 233

``` connective tissue disorders scleroderma (most common) rheumatoid arthritis systemic lupus erythematosus leukaemia type I cryoglobulinaemia, cold agglutinins use of vibrating tools drugs: oral contraceptive pill, ergot cervical rib ```

Question 234

In Raynauds what factors indicate the presence of an underlying secondary cause?

Answer 234

``` onset after 40 years unilateral symptoms rashes presence of autoantibodies features which may suggest rheumatoid arthritis or SLE, for example arthritis or recurrent miscarriages digital ulcers, calcinosis very rarely: chilblains ```

Question 235

What is the management of Raynauds?

Answer 235

all patients with suspected secondary Raynaud's phenomenon should be referred to secondary care first-line: calcium channel blockers e.g. nifedipine IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months

Question 236

Which complement deficiencies predispose to immune complex disease (e.g. henoch schonlein purpura, SLE)?

Answer 236

C1q, C1rs, C2, C4 deficiency (classical pathway components)

Question 237

What are the clinical features of a patent ductus arteriosus?

Answer 237

``` left subclavicular thrill continuous 'machinery' murmur large volume, bounding, collapsing pulse wide pulse pressure heaving apex beat ```

Question 238

How do SSRIs increase risk of bleeding?

Answer 238

SSRI potentially deplete platelet serotonin, resulting in a reduction in clot formation, therefore, increasing the risk of bleeding

Question 239

What are the acute adverse effects of phenytoin?

Answer 239

initially: dizziness, diplopia, nystagmus, slurred speech, ataxia later: confusion, seizures

Question 240

What are the chronic adverse effects of phenytoin?

Answer 240

-common: gingival hyperplasia (secondary to increased expression of platelet derived growth factor, PDGF), hirsutism, coarsening of facial features, drowsiness - megaloblastic anaemia (secondary to altered folate metabolism) - peripheral neuropathy - enhanced vitamin D metabolism causing osteomalacia - lymphadenopathy - dyskinesia

Question 241

How do you investigate suspected acromegaly?

Answer 241

First line - IGF-1 measurement Second - OGTT with serial GH measurements. (The Endocrine Society recommends in patients with clinically suspected acromegaly, and an elevated or equivocal serum IGF-1 level, the diagnosis should then be confirmed by finding a lack of GH suppression with an OGTT.) Small print -Pituitary MRI can be used to identify pituitary lesion after diagnosis -Serum GHRH level (Acromegaly secondary to GHRH secreting tumours are rare and should only be investigated for cases where a pituitary defect cannot be confirmed.)

Question 242

What are the causes of mydriasis (large pupil)?

Answer 242

``` third nerve palsy Holmes-Adie pupil traumatic iridoplegia phaeochromocytoma congenital ```

Question 243

What are the fundoscopy findings in retinitis pigmentosa?

Answer 243

black bone spicule-shaped pigmentation in the peripheral retina, mottling of the retinal pigment epithelium

Question 244

What are the gram positive cocci?

Answer 244

staphylococci + streptococci (including enterococci)

Question 245

What are the gram negative cocci?

Answer 245

Neisseria meningitidis + Neisseria gonorrhoeae, also Moraxella catarrhalis

Question 246

What are the gram negative bacilli?

Answer 246

ABCD L ``` Actinomyces Bacillus anthracis (anthrax) Clostridium Diphtheria: Corynebacterium diphtheriae Listeria monocytogenes ```

Question 247

What are the gram negative rods?

Answer 247

Remaining organisms are Gram-negative rods, e.g.: - Escherichia coli - Haemophilus influenzae - Pseudomonas aeruginosa - Salmonella sp. - Shigella sp. - Campylobacter jejuni

Question 248

What is associated with Type 1 cryoglobulinaemia?

Answer 248

multiple myeloma, Waldenstrom macroglobulinaemia Raynauds only seen in type 1

Question 249

What is associated with Type 2 cryoglobulinaemia?

Answer 249

hepatitis C, rheumatoid arthritis, Sjogren's, lymphoma

Question 250

What is associated with Type 3 cryoglobulinaemia?

Answer 250

rheumatoid arthritis, Sjogren's

Question 251

What are the features of leukaemoid reaction that can help differentiate from CML?

Answer 251

- high leucocyte alkaline phosphatase score - toxic granulation (Dohle bodies) in the white cells - 'left shift' of neutrophils i.e. three or fewer segments of the nucleus

Question 252

What are the features of an S1 nerve root compression?

Answer 252

Compression of the S1 nerve root results in a sensory loss of the posterolateral aspect of the leg and lateral aspect of the foot. There is a weakness of foot plantar flexion and ankle reflexes are normally reduced. Finally, pain can be elicited when the patient performs a straight leg raise; known as the sciatic nerve stretch test.

Question 253

What are the features of an L1-2 nerve root compression?

Answer 253

An L1-2 nerve root compression will result in discomfort over the groin and upper thigh region with associated weakness of the pelvic and psoas muscles. Although rare, patients suffering prolapse of the L1-2 discs often have difficulty lifting the affected leg and therefore struggle to walk upstairs etc.

Question 254

What are the features of an L3 nerve root compression?

Answer 254

The pain pattern and neurological deficit of an L3 nerve root compression is commonly a sensory loss over the anterior aspect of the thigh, weakness of the quadriceps with a reduction in the knee reflex on the affected side. Finally, pain can be elicited in the anterior thigh when performing the femoral stretch test, where the knee is passively flexed with the patient lying prone.

Question 255

What are the features of an L4 nerve root compression?

Answer 255

An L4 nerve root compression can present with a very similar pattern to an L3 compression with weakness of the quadriceps, a reduced knee reflex and a positive femoral stretch test. In L4 root compression, however, there will be a sensory loss over the anterior aspect of the knee, not the thigh.

Question 256

What are the features of an L5 nerve root compression?

Answer 256

L5 nerve root compression can be identified on examination by evidence of sensory loss on the dorsum aspect of the foot with weakness of the foot and big toe dorsiflexion. L5 nerve compressions will also result in a positive sciatic nerve stretch test but the reflexes are normally unaffected.

Question 257

What antibodies are seen in dermatomyositis?

Answer 257

ANA positive in 60% anti-Mi-2 antibodies are highly specific for dermatomyositis, but are only seen in around 25% of patients anti-Jo-1 antibodies are not commonly seen in dermatomyositis - they are more common in polymyositis where they are seen in a pattern of disease associated with lung involvement, Raynaud's and fever

Question 258

What is the treatment of discoid lupus erythematosis?

Answer 258

topical steroid cream oral antimalarials may be used second-line e.g. hydroxychloroquine avoid sun exposure

Question 259

What are the features of Autoimmune polyendocrinopathy syndrome type 1?

Answer 259

2 out of three must be present - chronic mucocutaneous candidiasis (typically first feature as young child) - Addison's disease - primary hypoparathyroidism

Question 260

What are the features of Autoimmune polyendocrinopathy syndrome type 2?

Answer 260

APS type 2 has a polygenic inheritance and is linked to HLA DR3/DR4. Patients have Addison's disease plus either: - type 1 diabetes mellitus - autoimmune thyroid disease

Question 261

What are the adverse effects of sodiium valproate?

Answer 261

teratogenic maternal use of sodium valproate is associated with a significant risk of neurodevelopmental delay in children guidance is now clear that sodium valproate should not be used during pregnancy and in women of childbearing age unless clearly necessary. Women of childbearing age should not start treatment without specialist neurological or psychiatric advice. ``` P450 inhibitor gastrointestinal: nausea increased appetite and weight gain alopecia: regrowth may be curly ataxia tremor hepatotoxicity pancreatitis thrombocytopaenia hyponatraemia hyperammonemic encephalopathy: L-carnitine may be used as treatment if this develops ```

Question 262

What drugs can be cleared by haemodialysis?

Answer 262

Drugs that can be cleared with haemodialysis - mnemonic: BLAST - Barbiturate - Lithium - Alcohol (inc methanol, ethylene glycol) - Salicylates - Theophyllines (charcoal haemoperfusion is preferable)

Question 263

What is the mechanism of action of dipyridamole?

Answer 263

Dipyridamole is a non-specific phosphodiesterase inhibitor and decreases cellular uptake of adenosine. It can be used in combination with aspirin for secondary prevention following transient ischaemic attack if clopidogrel is not tolerated.

Question 264

What drugs can cause thrombocytopenia?

Answer 264

quinine abciximab NSAIDs diuretics: furosemide antibiotics: penicillins, sulphonamides, rifampicin anticonvulsants: carbamazepine, valproate heparin

Question 265

What are the causes of a soft S2?

Answer 265

Causes of a soft S2 | -aortic stenosis

Question 266

What are the causes of a split S2?

Answer 266

Atrial septal defect

Question 267

What are the causes of a widely split S2?

Answer 267

Causes of a widely split S2 - deep inspiration - RBBB - pulmonary stenosis - severe mitral regurgitation

Question 268

What are the causes of a reversed (paradoxical) split S2? (P2 occurs before A2)

Answer 268

Causes of a reversed (paradoxical) split S2 (P2 occurs before A2) - LBBB - severe aortic stenosis - right ventricular pacing - WPW type B (causes early P2) - patent ductus arteriosus

Question 269

What are the "anchor" drugs in rheumatoid arthritis?

Answer 269

Sulfasalazine Hydroxychloroquine Methotrexate Leflunomide Must try at least 2 of these conventional therapies before progressing to newer agents like biologics.

Question 270

Which renal stones are radio-opaque?

Answer 270

All the ones that are OOOOOpaque contain an o (phosphate (incl stag horn), oxalate), all the ones that are radiolucent don't (urate and xanthine) - just have to remember that cystine are semi-opaque (the c looks like half an o)

Question 271

How do you diagnose paroxysmal nocturnal haemoglobinuria?

Answer 271

flow cytometry of blood to detect low levels of CD59 and CD55 has now replaced Ham's test as the gold standard investigation in PNH Ham's test: acid-induced haemolysis (normal red cells would not)

Question 272

What are the causes of cavitating lung lesions?

Answer 272

WAP RATS Wegners, Abscess, PE RA, Aspergillosis, TB, SCC

Question 273

In head injury what are the indications for a CT head within 8 hours?

Answer 273

- age 65 years or older - any history of bleeding or clotting disorders - dangerous mechanism of injury (a pedestrian or cyclist struck by a motor vehicle, an occupant ejected from a motor vehicle or a fall from a height of greater than 1 metre or 5 stairs) - more than 30 minutes' retrograde amnesia of events immediately before the head injury If a patient is on warfarin who have sustained a head injury with no other indications for a CT head scan, perform a CT head scan within 8 hours of the injury.

Question 274

What drug group is associated with a significantly increased mortality in dementia patients?

Answer 274

Antipsychotics

Question 275

What does damage to the lateral corticospinal tracts and dorsal columns in B12 deficiency cause?

Answer 275

lateral corticospinal tracts and dorsal columns resulting in upper motor neuron signs (extensor plantar) together with reduced proprioception and vibration sense.

Question 276

What does the anterior coritcospinal tract do?

Answer 276

It is responsible for the gross and postural movement of the trunk and proximal musculature

Question 277

What does the anterior spinocerebellar tract do?

Answer 277

carries proprioceptive and cutaneous information from the lower body

Question 278

What does the anterior spinothalamic pathway do?

Answer 278

anterior spinothalamic pathway, which is responsible for coarse touch and pressure.

Question 279

What does the lateral spinothalamic pathway do?

Answer 279

Pain and temperature.

Question 280

What are the signs of ebstein's anomaly?

Answer 280

cyanosis prominent 'a' wave in the distended jugular venous pulse, hepatomegaly tricuspid regurgitation pansystolic murmur, worse on inspiration right bundle branch block → widely split S1 and S2 Associated with WPW so may get slurred upstroke in QRS complexes.

Question 281

How does the Mantoux (Tuberculin skin test or quantiferon) work?

Answer 281

Example of type IV (delayed) hypersensitivity reactions. These are largely mediated by interferon-γ secreted by Th1 cells which in turn stimulates macrophage activity.

Question 282

What is the mechanism of action of aminoglycosides?

Answer 282

Binds to 30S subunit causing misreading of mRNA

Question 283

What is the mechanism of action of tetracyclines?

Answer 283

Binds to 30S subunit blocking binding of aminoacyl-tRNA

Question 284

What is the mechanism of action of chloramphenicol?

Answer 284

Binds to 50S subunit, inhibiting peptidyl transferase

Question 285

What is the mechanism of action of clindamycin?

Answer 285

Binds to 50S subunit, inhibiting translocation (movement of tRNA from acceptor site to peptidyl site)

Question 286

What is the mechanism of action of the macrolides?

Answer 286

Binds to 50S subunit, inhibiting translocation (movement of tRNA from acceptor site to peptidyl site)

Question 287

How is Lassa Fever contracted?

Answer 287

(Arenaviridae) Lassa fever is contracted by direct contact with the excreta of infected African rats or by person to person spread. It is predominantly seen in West Africa.

Question 288

What are the causes of a raised BNP apart from heart failure?

Answer 288

``` COPD pneumonia, sepsis, other cardiac causes such as AF and valve disease, liver disease, older age, chemotherapy. ```

Question 289

What are the causes of a falsely low BNP?

Answer 289

OBESITY flash pulmonary oedema pericardial constriction, use of ACE-is, ARBs and diuretics

Question 290

What are the features of pituitary apoplexy?

Answer 290

sudden onset headache similar to that seen in subarachnoid haemorrhage vomiting neck stiffness visual field defects: classically bitemporal superior quadrantic defect extraocular nerve palsies features of pituitary insufficiency e.g. hypotension/hyponatraemia secondary to hypoadrenalism

Question 291

What is the genetics of Prader Wili syndrome?

Answer 291

microdeletion of paternal 15q11-13 (70% of cases) | maternal uniparental disomy of chromosome 15

Question 292

What factors exacerbate plaque psoriasis?

Answer 292

trauma alcohol drugs: beta blockers, lithium, antimalarials (chloroquine and hydroxychloroquine), NSAIDs and ACE inhibitors, infliximab withdrawal of systemic steroids

Question 293

What drugs cause impaired glucose tolerance?

Answer 293

``` thiazides, furosemide (less common) steroids tacrolimus, ciclosporin interferon-alpha nicotinic acid antipsychotics ```

Question 294

What is the commonest cause of Filiriasis? | What is it treated with?

Answer 294

Wuchereria bancrofti ``` Parasitic filarial nematode Accounts for 90% of cases of filariasis Usually diagnosed by blood smears Usually transmitted by mosquitos Treatment is with diethylcarbamazine ```

Question 295

What is the mechanism of action of statins?

Answer 295

Statins inhibit HMG-CoA reductase, the rate-limiting enzyme in hepatic cholesterol synthesis

Question 296

How does smoking and asbestos exposure increase your risk of lung Ca?

Answer 296

Smoking increases risk by factor of 10 Asbestos by a factor of 5 These have a synergistic effect. So an asbestos exposed smoker has 50 times increased risk.

Question 297

What does C5 deficiency cause?

Answer 297

predisposes to Leiner disease | recurrent diarrhoea, wasting and seborrhoeic dermatitis

Question 298

What COPD features suggest responsiveness to steroids?

Answer 298

- previous diagnosis of asthma or atopy - a higher blood eosinophil count - substantial variation in FEV1 over time (at least 400 ml) - substantial diurnal variation in peak expiratory flow (at least 20%)

Question 299

What complement proteins are associated with SLE?

Answer 299

Low levels of C4a and C4b have been shown to be associated with an increased risk of developing systemic lupus erythematous Low levels of C3 and C4 indicate active disease

Question 300

Hashimoto's thyroiditis is associated with which type of cancer?

Answer 300

MALT lymphoma This is a rare form of thyroid lymphoma

Question 301

How does AST and ALT relate in alcoholic hepatitis?

Answer 301

the ratio of AST:ALT is normally > 2, a ratio of > 3 is strongly suggestive of acute alcoholic hepatitis

Question 302

How do you determine who gets steroids in alcoholic hepatitis?

Answer 302

Maddrey discriminant function (calculated from PT and bilirubin) NICE currently recommends initiation of corticosteroids in patients with a Maddrey discriminant function >32 and recommends consideration of liver biopsy where there is uncertainty around diagnosis

Question 303

What are the features of disseminated gonococcal infection (DGI)?

Answer 303

Key Features: - tenosynovitis - migratory polyarthritis - dermatitis (lesions can be maculopapular or vesicular) Later complications include septic arthritis, endocarditis and perihepatitis (Fitz-Hugh-Curtis syndrome)

Question 304

What is the management of gonnorhoea?

Answer 304

single dose of IM ceftriaxone 1g If sensitivities are known (and the organism is sensitive to ciprofloxacin) then a single dose of oral ciprofloxacin 500mg should be given if ceftriaxone is refused (e.g. needle-phobic) then oral cefixime 400mg (single dose) + oral azithromycin 2g (single dose) should be used

Question 305

What are the bacteriostatic antibiotics?

Answer 305

``` chloramphenicol macrolides tetracyclines sulphonamides trimethoprim ``` All others are bactericidal

Question 306

What electrographic features increase the likelihood of VT (vs SVT with aberrancy)?

Answer 306

- Absence of typical RBBB or LBBB morphology - Extreme axis deviation (“northwest axis”) — QRS is positive in aVR and negative in I + aVF. - Very broad complexes (>160ms) - AV dissociation (P and QRS complexes at different rates) - Capture beats — occur when the sinoatrial node transiently ‘captures’ the ventricles, in the midst of AV dissociation, to produce a QRS complex of normal duration. - Fusion beats — occur when a sinus and ventricular beat coincides to produce a hybrid complex. - Positive or negative concordance throughout the precordial (chest) leads, i.e. leads V1-6 show entirely positive (R) or entirely negative (QS) complexes, with no RS complexes seen. - Brugada sign – The distance from the onset of the QRS complex to the nadir of the S-wave is > 100ms -Josephson sign – Notching near the nadir of the S-wave RSR’ complexes with a taller left rabbit ear. This is the most specific finding in favour of VT. Note: This is in contrast to RBBB, where the right rabbit ear is taller.

Question 307

What are the risk factors for renal stones?

Answer 307

dehydration hypercalciuria, hyperparathyroidism, hypercalcaemia cystinuria high dietary oxalate renal tubular acidosis medullary sponge kidney, polycystic kidney disease beryllium or cadmium exposure

Question 308

What are the complications of plasma exchange (plasmapheresis)?

Answer 308

``` hypocalcaemia: due to the presence of citrate used as an anticoagulant for the extracorporeal system metabolic alkalosis removal of systemic medications coagulation factor depletion immunoglobulin depletion ```

Question 309

What is the treatment of acne rosacea?

Answer 309

topical metronidazole may be used for mild symptoms (i.e. Limited number of papules and pustules, no plaques) topical brimonidine gel may be considered for patients with predominant flushing but limited telangiectasia more severe disease is treated with systemic antibiotics e.g. Oxytetracycline recommend daily application of a high-factor sunscreen camouflage creams may help conceal redness laser therapy may be appropriate for patients with prominent telangiectasia patients with a rhinophyma should be referred to dermatology

Question 310

What causes Pellagra?

Answer 310

Deficiency in nicotinic acid = niacin = Vitamin B3

Question 311

What are the indications for starting steroid in sarcoidosis?

Answer 311

parenchymal lung disease, uveitis, hypercalcaemia and neurological or cardiac involvement

Question 312

What are the poor prognostic features in CML?

Answer 312

> 60 years > 20% blasts after first course of chemo cytogenetics: deletions of chromosome 5 or 7 "an OLDER PERSON who has had PREVIOUS MYELODYSPLASIA whose CYTOGENES SHOW CHROMOSOMES 5 OR 7 DELETION"

Question 313

What features support MGUS over multiple myeloma?

Answer 313

- normal immune function - normal beta-2 microglobulin levels - lower level of paraproteinaemia than myeloma (e.g. < 30g/l IgG, or < 20g/l IgA) - stable level of paraproteinaemia - no clinical features of myeloma (e.g. lytic lesions on x-rays or renal disease)

Question 314

When increasing the dose of opioids how much should you increase by?

Answer 314

30-50%

Question 315

How do you convert from codeine to morphine?

Answer 315

Divide by 10

Question 316

How do you convert tramadol to morphine?

Answer 316

Divide by 10 | previously 5 but currently BNF states 10

Question 317

How much oral morphine is equivalent to a 12 microgram fentanyl patch?

Answer 317

a transdermal fentanyl 12 microgram patch equates to approximately 30 mg oral morphine daily

Question 318

How much oral morphine is equivalent to a 10 microgram buprenorphine patch?

Answer 318

a transdermal buprenorphine 10 microgram patch equates to approximately 24 mg oral morphine daily.

Question 319

How do you convert from oral to subcut morphine?

Answer 319

Divide by 2

Question 320

How do you convert from oral morphine to subcut diamorphine?

Answer 320

Divide by 3

Question 321

What tumour markers are raised in testicular germ cell cancers?

Answer 321

seminomas: seminomas: hCG may be elevated in around 20% non-seminomas: AFP and/or beta-hCG are elevated in 80-85% LDH is elevated in around 40% of germ cell tumours

Question 322

What are the biochemical findings in osteomalacia?

Answer 322

low: calcium, phosphate raised: alkaline phosphatase

Question 323

The deposition of what causes dermatitis herpetiformis?

Answer 323

IgA

Question 324

What are the features of neurofibromatosis type 1?

Answer 324

``` Café-au-lait spots (>= 6, 15 mm in diameter) Axillary/groin freckles Peripheral neurofibromas Iris hamatomas (Lisch nodules) in > 90% Scoliosis Pheochromocytomas ```

Question 325

What are the most common brain metastases?

Answer 325

``` lung (most common) breast bowel skin (namely melanoma) kidney ```

Question 326

How to meningiomas appear on imaging?

Answer 326

Meningiomas are typically benign, extrinsic tumours of the central nervous system. They arise from the arachnoid cap cells of the meninges and are typically located next to the dura and cause symptoms by compression rather than invasion. • They typically are located at the falx cerebri, superior sagittal sinus, convexity or skull base. They contrast enhance on CT

Question 327

What is the histology of meningiomas?

Answer 327

Spindle cells in concentric whorls and calcified psammoma bodies

Question 328

How does glioblastoma visualise on imaging?

Answer 328

On imaging they are solid tumours with central necrosis and a rim that enhances with contrast. Disruption of the blood-brain barrier and therefore are associated with vasogenic oedema.

Question 329

What is the histology of glioblastoma?

Answer 329

Pleomorphic tumour cells border necrotic areas

Question 330

How does vestibular schwannoma appear histologically?

Answer 330

Antoni A or B patterns are seen. Verocay bodies (acellular areas surrounded by nuclear palisades)

Question 331

What is the histology of astrocytoma?

Answer 331

Rosenthal fibres (corkscrew eosinophilic bundle)

Question 332

What is the histology of medulloblastoma?

Answer 332

Small, blue cells. Rosette pattern of cells with many mitotic figures

Question 333

What is the histology of ependydoma?

Answer 333

perivascular pseudorosettes

Question 334

What is the histology of oligodendroma?

Answer 334

Calcifications with 'fried-egg' appearance

Question 335

What is the histology of haemangioblastoma?

Answer 335

foam cells and high vascularity

Question 336

What is the histology of craniopharyngioma?

Answer 336

Derived from remnants of Rathke pouch

Question 337

What are the causes of hypokalaemia with hypertension?

Answer 337

Hypokalaemia with HTN = Little Cushing Conned 11 people Liddle's syndrome Cushing's syndrome Conn's syndrome (primary hyperaldosteronism) 11-beta hydroxylase deficiency

Question 338

What are the features of homocystinuria?

Answer 338

often patients have fine, fair hair musculoskeletal Marfanoid body habitus: arachnodactyly etc osteoporosis kyphosis neurological: may have learning difficulties, seizures ocular downwards (inferonasal) dislocation of lens severe myopia increased risk of arterial and venous thromboembolism also malar flush, livedo reticularis

Question 339

What are the pathological steps in the formation of atherosclerosis?

Answer 339

1. Endothelial dysfunction triggered by smoking, hypertension or hyperglycaemia. 2. Pro-inflammatory, pro-oxidant, proliferative changes in the endothelium. 3. Fatty infiltration of the subendothelial space by low-density lipoprotein (LDL). 4. Macrophages phagocytose oxidised low-density lipoprotein. 5. Smooth muscle proliferation and migration from the tunica media into the intima.

Question 340

What are the common indications for lung transplantation in CF?

Answer 340

- life-threatening exacerbation requiring ICU admission, - pulmonary hypertension, - FEV1 less than 30% of predicted, - recurrent exacerbations requiring antibiotic therapy, - recurrent and/or refractory pneumothorax, and - recurrent haemoptysis not controlled by embolisation.

Question 341

What is the mechanism of action of hydralazine?

Answer 341

It elevates the levels of cyclic GMP leading to a relaxation of the smooth muscle to a greater extent in the arterioles than the veins

Question 342

What percentage of those contracting Hepatitis C become chronically infected?

Answer 342

55-85%

Question 343

How do you stage Hodgkin's lymphoma?

Answer 343

``` Ann-Arbor staging of Hodgkin's lymphoma I: single lymph node II: 2 or more lymph nodes/regions on same side of diaphragm III: nodes on both sides of diaphragm IV: spread beyond lymph nodes ``` Each stage may be subdivided into A or B A = no systemic symptoms other than pruritus B = weight loss > 10% in last 6 months, fever > 38c, night sweats (poor prognosis

Question 344

What is the mechanism of action of bicalutamide?

Answer 344

bicalutamide - non-steroidal anti-androgen - blocks the androgen receptor

Question 345

What is the mechanism of action of cyproterone acetate?

Answer 345

cyproterone acetate - steroidal anti-androgen - prevents DHT binding from intracytoplasmic protein complexes - used less commonly since introduction of non-steroidal anti-androgens

Question 346

What is the mechanism of action of abiraterone?

Answer 346

abiraterone - androgen synthesis inhibitor - option for the treatment of hormone-relapsed metastatic prostate cancer in patients who have no or mild - symptoms after androgen deprivation therapy has failed, and before chemotherapy is indicated

Question 347

What are the signs of an addisonian crisis?

Answer 347

Neurological - syncope - confusion - lethargy - convulsions Haemodynamic - hypotension - hypothermia Biochemical - hyponatraemia - hyperkalaemia - hypoglycaemia

Question 348

What are the causes of autonomic neuropathy?

Answer 348

diabetes Guillain-Barre syndrome multisystem atrophy (MSA), Shy-Drager syndrome Parkinson's infections: HIV, Chagas' disease, neurosyphilis drugs: antihypertensives, tricyclics craniopharyngioma

Question 349

What are the indications for chest drain insertion in pleural infection?

Answer 349

Patients with frankly purulent or turbid/cloudy pleural fluid on sampling should receive prompt pleural space chest tube drainage. The presence of organisms identified by Gram stain and/or culture from a non-purulent pleural fluid sample indicates that pleural infection is established and should lead to prompt chest tube drainage. Pleural fluid pH < 7.2 in patients with suspected pleural infection indicates a need for chest tube drainage.

Question 350

What drugs should be avoided in pregnancy?

Answer 350

Antibiotics - tetracyclines - aminoglycosides - sulphonamides and trimethoprim - quinolones: the BNF advises to avoid due to arthropathy in some animal studies Other drugs - ACE inhibitors, angiotensin II receptor antagonists - statins - warfarin - sulfonylureas - retinoids (including topical) - cytotoxic agents

Question 351

What is does inflixamab act against? | What is it used in?

Answer 351

infliximab (anti-TNF): used in rheumatoid arthritis and Crohn's

Question 352

What is does rituximab act against? | What is it used in?

Answer 352

rituximab (anti-CD20): used in non-Hodgkin's lymphoma and rheumatoid arthritis

Question 353

What is does cetuximab act against? | What is it used in?

Answer 353

cetuximab (epidermal growth factor receptor antagonist): used in metastatic colorectal cancer and head and neck cancer

Question 354

What is does trastuzumab act against? | What is it used in?

Answer 354

trastuzumab (HER2/neu receptor antagonist): used in metastatic breast cancer

Question 355

What is does alemtuzumab act against? | What is it used in?

Answer 355

alemtuzumab (anti-CD52): used in chronic lymphocytic leukaemia

Question 356

What is does abxizamab act against? | What is it used in?

Answer 356

abciximab (glycoprotein IIb/IIIa receptor antagonist): prevention of ischaemic events in patients undergoing percutaneous coronary interventions

Question 357

What does OKT3 act against? | What is it used in?

Answer 357

OKT3 (anti-CD3): used to prevent organ rejection

Question 358

What is the treatment for cutaneous anthrax?

Answer 358

Cirprofloxacin

Question 359

What is the mechanism of action of benzodiasepines?

Answer 359

Benzodiazepines enhance the effect of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA) by increasing the FREQUENCY of chloride channels

Question 360

What anaphylaxis patients qualify for a fast-track discharge (after 2 hours)?

Answer 360

fast-track discharge (after 2 hours of symptom resolution): - good response to a single dose of adrenaline - complete resolution of symptoms - has been given an adrenaline auto-injector and trained how to use it - adequate supervision following discharge

Question 361

What anaphylaxis patients qualify for a 6 hour discharge?

Answer 361

minimum 6 hours after symptom resolution - 2 doses of IM adrenaline needed, or - previous biphasic reaction

Question 362

What anaphylaxis patients qualify for a 12 hour discharge?

Answer 362

minimum 12 hours after symptom resolution - severe reaction requiring > 2 doses of IM adrenaline - patient has severe asthma - possibility of an ongoing reaction (e.g. slow-release medication) - patient presents late at night - patient in areas where access to emergency access care may be difficult - observation for at 12 hours following symptom resolution

Question 363

What are the features of zinc deficiency?

Answer 363

``` perioral dermatitis: red, crusted lesions acrodermatitis alopecia short stature hypogonadism hepatosplenomegaly geophagia (ingesting clay/soil) cognitive impairment ```

Question 364

Name the x-linked recessive conditions

Answer 364

``` Androgen insensitivity syndrome Becker muscular dystrophy Colour blindness Duchenne muscular dystrophy Fabry's disease G6PD deficiency Haemophilia A,B Hunter's disease Lesch-Nyhan syndrome Nephrogenic diabetes insipidus Ocular albinism Retinitis pigmentosa Wiskott-Aldrich syndrome ```

Question 365

What is the management of brain abscesses?

Answer 365

surgery - -a craniotomy is performed and the abscess cavity debrided - -the abscess may reform because the head is closed following abscess drainage. - IV antibiotics: IV 3rd-generation cephalosporin + metronidazole - intracranial pressure management: e.g. dexamethasone

Question 366

Carcinoid is associated with which murmur?

Answer 366

Pulmonary stenosis (Hedinger syndrome)

Question 367

Where can nicorandil cause ulcerations?

Answer 367

skin, mucosal and eye ulceration gastrointestinal ulcers including anal ulceration It is a potassium-channel activator with vasodilation is through activation of guanylyl cyclase which results in increase cGMP.

Question 368

What conditions are associated with mitral valve prolapse?

Answer 368

``` congenital heart disease: PDA, ASD cardiomyopathy Turner's syndrome Marfan's syndrome, Fragile X osteogenesis imperfecta pseudoxanthoma elasticum Wolff-Parkinson White syndrome long-QT syndrome Ehlers-Danlos Syndrome polycystic kidney disease ```

Question 369

What conditions are associated with aortic regurgitation?

Answer 369

Causes (due to valve disease) - rheumatic fever - infective endocarditis - connective tissue diseases e.g. RA/SLE - bicuspid aortic valve Causes (due to aortic root disease) - aortic dissection - spondylarthropathies (e.g. ankylosing spondylitis) - hypertension - syphilis - Marfan's, Ehler-Danlos syndrome

Question 370

When should statins be offered to Type 1 DM patients?

Answer 370

older than 40 years, or have had diabetes for more than 10 years or have established nephropathy or have other CVD risk factors

Question 371

What are the 5 key features of HIV associated nephropathy?

Answer 371

massive proteinuria resulting in nephrotic syndrome normal or large kidneys focal segmental glomerulosclerosis with focal or global capillary collapse on renal biopsy elevated urea and creatinine normotension

Question 372

What are the causes of pseudohyperkalaemia?

Answer 372

haemolysis during venepuncture (excessive vacuum of blood drawing or too fine a needle gauge) delay in the processing of the blood specimen abnormally high numbers of platelets, leukocytes, or erythrocytes (such as myeloproliferative disorders) familial causes

Question 373

What are the causes of anaemia in renal failure?

Answer 373

- reduced erythropoietin levels - the most significant factor - reduced erythropoiesis due to toxic effects of uraemia on bone marrow - reduced absorption of iron - anorexia/nausea due to uraemia - reduced red cell survival (especially in haemodialysis) - blood loss due to capillary fragility and poor platelet function - stress ulceration leading to chronic blood loss

Question 374

What are the features of Lofgren's syndrome?

Answer 374

LoFGREN'S - Lymphadenopathy, Fever,aRthraligia, Girls, Erythema Nodosum, Sarcoidosis

Question 375

What are the contraindications for surgery in non-small cell lung cancer?

Answer 375

assess general health stage IIIb or IV (i.e. metastases present) FEV1 < 1.5 litres is considered a general cut-off point* malignant pleural effusion tumour near hilum vocal cord paralysis SVC obstruction * However if FEV1 < 1.5 for lobectomy or < 2.0 for pneumonectomy then some authorities advocate further lung function tests as operations may still go ahead based on the results

Question 376

How long does it take for an arteriovenous fistula to develop?

Answer 376

6-8 weeks

Question 377

What factors affect the MDRD calculation of eGFR?

Answer 377

pregnancy muscle mass (e.g. amputees, body-builders) eating red meat 12 hours prior to the sample being taken

Question 378

What vaccines are contraindicated in HIV?

Answer 378

Cholera CVD103-HgR Influenza-intranasal Poliomyelitis-oral (OPV) Tuberculosis (BCG)

Question 379

What vaccines can be used in HIV as long as CD4 >200

Answer 379

Measles, Mumps, Rubella (MMR) Varicella Yellow Fever

Question 380

How can you localise horner's syndrome based on sweating?

Answer 380

Horner's syndrome - anhydrosis determines site of lesion: - head, arm, trunk = central lesion: stroke, syringomyelia - just face = pre-ganglionic lesion: Pancoast's, cervical rib - absent = post-ganglionic lesion: carotid artery

Question 381

What are the British Society of Haematology Guidelines for someone on warfarin undergoing emergency surgery?

Answer 381

If surgery can wait for 6-8 hours - give 5 mg vitamin K IV If surgery can't wait - 25-50 units/kg four-factor prothrombin complex

Question 382

Hyperacute graft rejection is mediated by which immunoglobulins?

Answer 382

Hyperacute graft rejection is due to pre-existent antibodies to HLA antigens and is therefore IgG mediated

Question 383

What is the relative importance of different HLA matching for renal transplants?

Answer 383

DR > B > A

Question 384

What are the X-linked dominant conditions?

Answer 384

- Alport's syndrome (in around 85% of cases - 10-15% of cases are inherited in an autosomal recessive fashion with rare autosomal dominant variants existing) - Rett syndrome - Vitamin D resistant rickets *pseudohypoparathyroidism was previously classified as an X-linked dominant condition but has now been shown to be inherited in an autosomal dominant fashion in the majority of cases

Question 385

How do you differentiate Zike, Chikungunya and Dengue?

Answer 385

Zika, Chikungunya and Dengue can produce similar symptoms. Zika is prevalent in South America. It tends to cause mild fever whereas dengue and chikungunya tend to cause abrupt onset of high fever. Chikungunya and dengue would cause more joint pain and conjunctivitis is less common with these conditions.

Question 386

What is a normal anion-gap?

Answer 386

8-14

Question 387

What is the antibody for mixed connective tissue disease? What are the features?

Answer 387

Anti-RNP R Raynoud N NO synovitis (but puffy/ swollen hands) P Pain in muscle and joints

Question 388

What drugs cause photosensitivity?

Answer 388

``` thiazides tetracyclines, sulphonamides, ciprofloxacin amiodarone NSAIDs e.g. piroxicam psoralens sulphonylureas ```

Question 389

Genital warts are caused by which HPV?

Answer 389

Types 6 and 11 are responsible for 90% of genital warts cases

Question 390

Cervical cancer is caused by which HPV?

Answer 390

16, 18 and 33 typically

Question 391

What are the features of dengue fever?

Answer 391

Retro-orbital headache, fever, facial flushing, rash, thrombocytopenia in returning traveller

Question 392

What is the maximum normal diameter of the bowel?

Answer 392

3/6/9 Maximum normal diameter 3cm = small bowel 6cm = large bowel 9cm = caecum

Question 393

What are the causes of nephrogenic diabetes insipidus?

Answer 393

genetic: the more common form affects the vasopression (ADH) receptor, the less common form results from a mutation in the gene that encodes the aquaporin 2 channel electrolytes: hypercalcaemia, hypokalaemia lithium lithium desensitizes the kidney's ability to respond to ADH in the collecting ducts demeclocycline tubulo-interstitial disease: obstruction, sickle-cell, pyelonephritis

Question 394

What are the different alleles for alpha-1 antitrypsin deficiency?

Answer 394

alleles classified by their electrophoretic mobility - M for normal, S for slow, and Z for very slow -normal: PiMM -heterozygous: PiMZ evidence base is conflicting re: risk of emphsema however, if non-smoker low risk of developing emphsema but may pass on A1AT gene to children - homozygous PiSS: 50% normal A1AT levels - homozygous PiZZ: 10% normal A1AT levels

Question 395

What interactions need to be considered in anticholinesterase inhibitors (donepezil, rivastigmine and galantamine)?

Answer 395

One of the important possible side effects of the acetylcholinesterase inhibitors (donepezil, rivastigmine and galantamine) is bradycardia (or SA block or AV block). Hence these medications might be contraindicated or should be started with caution in patients with conduction abnormalities or those already taking negatively chronotropic medications such as beta blockers, rate-limiting calcium channel blockers or digoxin.

Question 396

What are the clinical features of homocystinuria?

Answer 396

Tall, long fingered, downward lens dislocation, learning difficulties, DVT often patients have fine, fair hair musculoskeletal Marfanoid body habitus: arachnodactyly etc osteoporosis kyphosis neurological: may have learning difficulties, seizures ocular downwards (inferonasal) dislocation of lens severe myopia increased risk of arterial and venous thromboembolism also malar flush, livedo reticularis Caused by deficiency in cystathionine beta synthase

Question 397

What are the causes of jejunal villous atrophy?

Answer 397

``` coeliac disease tropical sprue hypogammaglobulinaemia gastrointestinal lymphoma Whipple's disease cow's milk intolerance ```

Question 398

What virus is associated with a low glucose level in CSF?

Answer 398

Mumps | also rarely herpes encephalitis

Question 399

What conditions are associated with Eissenmenger's syndrome?

Answer 399

ventricular septal defect atrial septal defect patent ductus arteriosus NOT Tetralogy of fallot "As the aorta overlies the VSD, it receives blood from the right ventricle and the left ventricle. This causes circulation of deoxygenated blood and central cyanosis (bluish discoloration). The high right ventricular pressure, caused by the outflow obstruction, facilitate this. Because less blood goes through the pulmonary circulation, cyanosis is independant of pulmonary hypertension (in contrast to Eisenmenger’s syndrome)."

Question 400

What are the effects of chronic hepatitis C infection?

Answer 400

rheumatological problems: arthralgia, arthritis eye problems: Sjogren's syndrome cirrhosis (5-20% of those with chronic disease) hepatocellular cancer cryoglobulinaemia: typically type II (mixed monoclonal and polyclonal) porphyria cutanea tarda (PCT): it is increasingly recognised that PCT may develop in patients with hepatitis C, especially if there are other factors such as alcohol abuse membranoproliferative glomerulonephritis

Question 401

What are the acute phase proteins?

Answer 401

``` CRP* procalcitonin ferritin fibrinogen alpha-1 antitrypsin caeruloplasmin serum amyloid A serum amyloid P component** haptoglobin complement ```

Question 402

What cancers are associated with CA-125?

Answer 402

CA-125: 1(uterus), 2(ovaries -> ovarian ca), 5 : P-ive(five)-> P-eritoneal ca.

Question 403

Give examples of Type 1 sensitivity reaction

Answer 403

Antigen reacts with IgE bound to mast cells * Anaphylaxis * Atopy (e.g. asthma, eczema and hayfever)

Question 404

Give examples of Type 2 sensitivity reaction

Answer 404

IgG or IgM binds to antigen on cell surface * Autoimmune haemolytic anaemia * ITP * Goodpasture's syndrome * Pernicious anaemia * Acute haemolytic transfusion reactions * Rheumatic fever * Pemphigus vulgaris / bullous pemphigoid

Question 405

Give examples of Type 3 sensitivity reaction

Answer 405

Free antigen and antibody (IgG, IgA) combine * Serum sickness * Systemic lupus erythematosus * Post-streptococcal glomerulonephritis * Extrinsic allergic alveolitis (especially acute phase)

Question 406

Give examples of Type 4 sensitivity reaction

Answer 406

T-cell mediated * Tuberculosis / tuberculin skin reaction * Graft versus host disease * Allergic contact dermatitis * Scabies * Extrinsic allergic alveolitis (especially chronic phase) * Multiple sclerosis * Guillain-Barre syndrome

Question 407

Give examples of Type 5 sensitivity reaction

Answer 407

Antibodies that recognise and bind to the cell surface receptors. This either stimulating them or blocking ligand binding * Graves' disease * Myasthenia gravis

Question 408

What is the most common cause of peritonitis secondary to peritoneal dialysis?

Answer 408

Coagulase-negative Staphylococcus is the most common cause of peritonitis secondary to peritoneal dialysis (e.g. Staph epidermidis)

Question 409

What is the pathology of Haemolytic Uraemic Syndrome?

Answer 409

Binding of Shiga-toxin to globotriaosylceramide

Question 410

What is the investigation of Whipple’s disease?

Answer 410

Jejunal biopsy shows deposition of macrophages containing Periodic acid-Schiff (PAS) granules

Question 411

What is the most common site of primary cardiac tumours in adults?

Answer 411

Left atrium Most common site of atrial myxoma is the fossa ovalis border in the left atrium

Question 412

At the glomerular level, how do NSAIDs and ACE-i contribute to the development of AKI?

Answer 412

NSAIDs inhibit afferent arteriole vasodilation by prostaglandins ACE-i inhibit angiotensin-II mediated vasocontriction of the Efferent arteriole

Question 413

What measurement shows satisfactory progression of treatment in DKA?

Answer 413

Capillary blood ketones falling by 0.5 mmol/L per hour

Question 414

Complete heart block in congenital SLE is associated with which antibody?

Answer 414

anti-Ro

Question 415

What is the treatment for acute angle closure glaucoma?

Answer 415

IV analgesia and antiemetics, lie patient supine. Topical beta blockers and steroids. IV acetazolamide. After initial treatment, pilocarpine will induce meiosis and open the angle. This is initially ineffective due to pressure induced ischaemic paralysis of the iris. Iridotomy 24-48 hours after intra-ocular pressure is controlled to prevent recurrence

Question 416

What is the treatment for cystinuric renal stones?

Answer 416

The cornerstones of cystinuric stone prevention are hydration and urinary alkalinisation. Sodium bicarbonate was traditionally the agent of choice, however, there is concern about the development of hypertension in patients on long-term treatment. As a result, potassium citrate has become the alkalinising agent of choice. This patient is already drinking 3 litres per day and his pH is already 7.5, so neither of these options is correct. If hydration and alkalinisation fail, the chelating agent D-penicillamine can be used. It combines with cysteine and renders it 50 times more soluble than cystine. Adverse reactions are common, and the newer agent alpha-mercaptopropionylglycine is better tolerated. Captopril is used in patients with concomitant hypertension.

Question 417

What are the side effects of efavirenc?

Answer 417

Disturbing dreams Psychosis

Question 418

How many people develop pouchitis following ideal pouch-anal anastomosis after total colectomy?

Answer 418

Up to 30 % of patients develop pouchitis following ileal pouch-anal anastomosis after total colectomy in ulcerative colitis. This presents with increased stool frequency, urgency, incontinence and nocturnal seepage. First line treatment is with antibiotics such as metronidazole or ciprofloxacin. Probiotics are used in some cases. In 5 % of cases, pouchitis can become chronic, ultimately leading to pouch failure and requiring pouch excision

Question 419

What are the diagnostic criteria for DKA?

Answer 419

Diagnostic criteria: all three of the following must be present capillary blood glucose above 11 mmol/L capillary ketones above 3 mmol/L or urine ketones ++ or more venous pH less than 7.3 and/or bicarbonate less than 15 mmol/L