MRCP 1 Flashcards

Question

Digoxin

Answer 1

Reversibly binds to Na/K/ATPase Shortens QT Toxicity (aggravated by low K) - dizziness, vomiting, blurred vision

Answer 2

Blocks sodium channels Increases AP duration Quinidine, procainamide, disopyramide

Answer 3

Blocks sodium channels Decreases AP duration Lidocaine, tocainide, mexiletine

Answer 4

Block sodium channels Flecainide, encainide, propafenone

Answer 5

Betablockers Propranolol, bisoprolol, atenolol

Answer 6

Blocks potassium channels Amiodarone, sotalol,

Answer 7

Calcium channel blockers Verapamil, diltiazem

Answer 8

Lymphoplasmacytoid malignancy characterised by the secretion of a monoclonal IgM paraprotein Seen in older men Weight loss, lethargy, hyperviscosity, lymphadenopathy, cryoglobulinaemia IgM paraproteinaemia Bone marrow diagnostic Rituximab chemo

Answer 9

Deficiency of cystathionine beta synthase Autosomal recessive Marfanoid, learning difficulties, kyphosis, seizures, DVTs Increased serum homocysteine Treated with B6 (pyridoxine)

Answer 10

Primary immunodeficiency disorder caused by T cell deficiency and dysfunction, micro deletion of chromosome 22 CATCH 22

Answer 11

47 XXY Tall, lack of secondary characteristics, small firm testes, infertile, gynaecomastia, high gonadotropin hormone and low testosterone, increased LH and FSH

Answer 12

Bind to ATP dependent potassium channel on beta pancreatic cells, e.g. gliclazide

Answer 13

Inhibits ADP binding to platelet receptors

Answer 14

Aromatase inhibitors that reduce peripheral oestrogen receptors

Answer 15

pANCA small-medium vessel vasculitis Asthma, mononeuritis, eosinophilia, paranasal sinusitis

Answer 16

BCG, yellow fever, MMR, polio, typhoid

Answer 17

Burkitts lymphoma

Answer 18

Mantle cell lymphoma

Answer 19

Ewing sarcoma

Answer 20

Acute promyelocytic syndrome

Answer 21

Follicular lymphoma

Answer 22

Autosomal dominant, FBN1 gene mutation (fibrillin-1=glycoprotein) cardiovascular, ocular, and skeletal abnormalities (tall, scoliosis, flat feet, pectus excavatum, mitral/tricuspid regurg, aortic aneurysm)

Answer 23

Anti-glomerular basement membrane disease, pulmonary haemorrhage and rapidly progressive glomerulonephritis, HLA DR2, renal biopsy: linear IgG deposits along the basement membrane, raised transfer factor secondary to pulmonary haemorrhages, management = plasma exchange (plasmapheresis), steroids, cyclophosphamide

Answer 24

Thrombosis of hepatic veins, usually in context of underlying haematological disease e.g. polycythaemia rubra vera, thrombophilia: activated protein C resistance, antithrombin III deficiency, protein C & S deficiencies, pregnancy, combined oral contraceptive pill: accounts for around 20% of cases, classic triad of ascites, abdominal pain, and hepatomegaly. USS abdo w/ doppler

Answer 25

Precipitated by thyroid/non-thyroid surgery, trauma infection, iodine load e.g. contrast, sx include fever > 38.5ºC, tachycardia, confusion and agitation, nausea and vomiting, hypertension, heart failure, abnormal liver function test - jaundice may be seen clinically. Management = dexamethasone, luogol's iodine, propylthiouracil/methimazole and beta blockers

Answer 26

Fragile X (CGG), Huntington's (CAG), myotonic dystrophy (CTG), Friedreich's ataxia* (GAA), spinocerebellar ataxia, spinobulbar muscular atrophy, dentatorubral pallidoluysian atrophy

Answer 27

Axonal - normal conduction velocity, reduced amplitude Demyelinating - reduced conduction velocity, normal amplitude

Answer 28

mainly in children, usually idiopathic, T-cell and cytokine-mediated damage to the glomerular basement membrane → polyanion loss the resultant reduction of electrostatic charge → increased glomerular permeability to serum albumin, causes: nephrotic syndrome, normotension - hypertension is rare, highly selective proteinuria, only intermediate-sized proteins such as albumin and transferrin leak through the glomerulus renal biopsy, normal glomeruli on light microscopy, electron microscopy shows fusion of podocytes and effacement of foot processes Management: oral corticosteroids: majority of cases (80%) are steroid-responsive, cyclophosphamide is the next step for steroid-resistant cases

Answer 29

Enhance phagocytosis of bacteria and viruses Fixes complement and passes to the fetal circulation Most abundant isotype in blood serum

Answer 30

IgA is the predominant immunoglobulin found in breast milk. It is also found in the secretions of digestive, respiratory and urogenital tracts and systems Provides localized protection on mucous membranes Most commonly produced immunoglobulin in the body (but blood serum concentrations lower than IgG.) Transported across the interior of the cell via transcytosis

Answer 31

First immunoglobulins to be secreted in response to an infection Fixes complement but does not pass to the fetal circulation Anti-A, B blood antibodies (cannot pass to the fetal circulation, which could result in haemolysis) Pentamer when secreted

Answer 32

Role in immune system largely unknown Involved in activation of B cells

Answer 33

Mediates type 1 hypersensitivity reactions Synthesised by plasma cells Binds to Fc receptors found on the surface of mast cells and basophils Provides immunity to parasites such as helminths Least abundant isotype in blood serum

Answer 34

Primary adrenal insufficiency, characterised by a deficiency in the hormones cortisol and aldosterone produced by the adrenal glands, hypoglycaemia, hyponatraemia, hyperkalaemia, metabolic acidosis. short synacthen test, adrenal autoantibodies

Answer 35

Associated with antipsychotics and withdrawal of L dopa. Theory is dopamine blockade induced by antipsychotics triggers massive glutamate release and subsequent neurotoxicity and muscle damage. Occurs in hours to days of starting antipsychotics. pyrexia, muscle rigidity, autonomic lability: typical features include hypertension, tachycardia and tachypnoea, agitated delirium with confusion, management = dantrolene/bromocriptine, stop antipsychotic, IVT.

Answer 36

Minimal change disease (children, Hokin's, nsaids) Membranous glomerulonephritis (proteinuria, nephrotic syndrome, CKD, infection, rheumatoid drugs, malignancy) Focal segmental glomerulonephritis (idiopathic, HIV, heroin, proteinuria, nephrotic syndrome, chronic kidney disease)

Answer 37

Haematuria, hypertension Rapidly progressive glomerulonephritis - aka crescentic glomerulonephritis rapid onset, often presenting as acute kidney injury, causes include Goodpasture's, ANCA positive vasculitis IgA nephropathy - aka Berger's disease, mesangioproliferative GN typically young adult with haematuria following an URTI, there is considerable pathological overlap with Henoch-Schonlein purpura (HSP)

Answer 38

Diffuse proliferative glomerulonephritis classical post-streptococcal glomerulonephritis in child presents as nephritic syndrome / acute kidney injury most common form of renal disease in SLE Membranoproliferative glomerulonephritis (mesangiocapillary) type 1: cryoglobulinaemia, hepatitis C type 2: partial lipodystrophy

Answer 39

CREST syndrome Aka limited scleroderma Calcinosis, Raynaud’s, oesophageal dysfunction, sclerodactyl and telangiectasia

Answer 40

Drug-induced lupus

Answer 41

Livedo reticularis describes an purplish, non-blanching, reticulated rash caused by obstruction of the capillaries resulting in swollen venules. Causes idiopathic (most common) polyarteritis nodosa systemic lupus erythematosus cryoglobulinaemia antiphospholipid syndrome Ehlers-Danlos Syndrome homocystinuria

Answer 42

Hypogonadotrophic hypogonadism, normal or above average height with anosmia, low testosterone, LH and FSH are low or inappropriately normal. Causes of lack of secondary sexual characteristics

Answer 43

Accounts for 25% of drug induced kidney injury. Acute inflammation causing marked interstitial oedema and interstitial infiltrates in the connective tissue between renal tubules. Triad of rash, joint pain and eosinophilia, also mild renal impairment and hypertension sterile pyuria (wcc in urine but no infection) and white cell casts Associated drugs: penicillin, rifampicin, NSAIDs, allopurinol, furosemide Mx: stop causative agent, sometimes steroids

Answer 44

aka distal failure to excrete H+ Causes: hereditary (most common, diagnosed in infants and children), autoimmune (e.g. Sjogrens, SLE, thyroiditis), nephrocalcinosis (e.g. primary hyperparathyroidism, vitamin D intoxification), nephrotoxins (e.g. amphotericin B, toluene inhalation), obstructive nephropathy hyperchloraemic acidosis, alkaline urine, and renal stone formation, normal anion gap Mx: sodium bicarb

Answer 45

aka proximal failure to reabsorb bicarb in proximal tubule metabolic acidosis with an inappropriately high urinary pH and hyperchloraemia (Cl- replaces HCO3 in circulation), low K, high urine HCO3- Causes: hereditary (most common, diagnosed in infants and children), part of Fanconi syndrome (proximal tubular defects with impaired reabsorption of glucose, phosphate and amino acids as well as HCO3), vitamin D deficiency, cystinosis, lead nephropathy, amyloidosis, medullary cystic disease

Answer 46

Mixed type 1 and 2 proximal bicarbonate leak in addition to a distal acidification defect (mixed proximal and distal RTA)

Answer 47

Tubular hyperkalaemia Caused by aldosterone deficiency or failure of receptors in collecting duct to respond to aldosterone causing reduced H+ and K+ excretion. associated with renal failure caused by disorders affecting the renal interstitium and tubules GFR >20mL/min (unlike uraemic acidosis) always associated with hyperkalaemia (unlike others) defect in cation-exchange in the distal tubule with reduced secretion of both H+ and K+ physiological reduction in proximal tubular ammonium excretion (impaired ammoniagenesis) due to to hypoaldosteronism, results in a decrease in urine buffering capacity associated with: Addison’s disease or post bilateral adrenalectomy acidosis not common unless there is associated renal damage affect the distal tubule the H+ pump in the tubule is not abnormal so that patients with this disorder are able to decrease their urinary pH to < 5.5 in response to the acidosis mild metabolic acidosis, HCO3 >15, hyperkalaemia

Answer 48

A wave = atrial contraction (large if increased pressure e.g. tricuspid/pulmonary stenosis, pulmonary hypertension, absent if AF) Cannon a waves = atrial contraction against closed tricuspid valve, seen in complete heart block, VT, nodal rhythm, single chamber pacing C wave = closure of tricuspid valve V wave = passive filling of blood into atrium against closed tricuspid valve, giant v waves in tricuspid regurg X descent = fall in atrial pressure during ventricular systole Y descent = opening of tricuspid valve

Answer 49

Anaphylactic - IgE bound to mast cells, associated with atopy

Answer 50

Cell bound - IgM or IgG binds to antigen on cell surface. E.g. goodpastures, pernicious anaemia, autoimmune haemolytic anaemia, haemolytic transfusion reaction, ITP, bullous pemphigoid

Answer 51

Free antigen and antibody combine, IgG or IgA. E.g. SLE, post strep glomerulonephritis, extrinsic allergic alveolitis

Answer 52

T cell mediated. E.g. TB, graft vs host disease, contact dermatitis, scabies, MS, GBS

Answer 53

Antibodies recognise and bind to cell surface receptors either stimulating or blocking ligand binding. E.g. Graves, myasthenia gravis

Answer 54

Mixed connective tissue disease

Answer 55

Greater than normal fall in systolic BP during inspiration = faint or absent pulse in inspiration = severe asthma/cardiac tamponade

Answer 56

Aortic stenosis

Answer 57

Aortic regurgitation, patent ductus arteriosus, hyperkinetic states e.g. anaemia, thyrotoxic, fever, exercise, pregnancy

Answer 58

Regular alternation of force of arterial pulse - severe LVSD

Answer 59

Double pulse - 2 systolic peaks, mixed aortic valve disease

Answer 60

HOCM (also sometimes bisferiens)

Answer 61

Polymyositis (more sensitive) Dermatomyositis

Answer 62

Autoimmune hepatitis

Answer 63

Scleroderma

Answer 64

Alport's syndrome is usually inherited in an X-linked dominant pattern. It is due to a defect in the gene which codes for type IV collagen resulting in an abnormal glomerular-basement membrane (GBM). The disease is more severe in males with females rarely developing it. Alport's syndrome usually presents in childhood. The following features may be seen: microscopic haematuria progressive renal failure bilateral sensorineural deafness lenticonus: protrusion of the lens surface into the anterior chamber retinitis pigmentosa Diagnosis: molecular genetic testing renal biopsy electron microscopy: characteristic finding is of the longitudinal splitting of the lamina densa of the glomerular basement membrane, resulting in a 'basket-weave' appearance

Answer 65

lower O2 delivery low acidity, low temp, HbF, carboxy/methaemoglobinaemia

Answer 66

higher O2 delivery, high acidity, high temp

Answer 67

GTN, aspirin and statin Add in bisoprolol or long-acting dihydropyridine CCB e.g. amlodipine/ MR nifedipine/ MR felodipine Can be used in combination but do not give verapamil/diltiazem with beta blocker (= complete heart block/HF) If still symptomatic, add in long acting nitrate e.g. nicorandil, , ivabradine, ISMN or ranolazine. Cannot give ivabradine with verapamil/diltiazem = bradycardia. Cannot use sildenafil etc with nitrates.

Answer 68

Sx - inhibit anticholinesterase causing accumlation of acetylcholine, cause bradycardia, miosis, diarrhoea, confusion, vomiting, headache, weakness and increased urination. Managed with IV atropine.

Answer 69

Autosomal recessive disorder of copper metabolism causing accumulation. Causes hepatic, neurological and psychiatric symptoms. Caeruloplasmin is low. Hepatic, serum and urine levels of copper are high. Caused by absence of ATP7B protein.

Answer 70

Mainly in children although accounts for 28% nephrotic syndrome in adults Main presentation is nephrotic syndrome with normal renal function and normal histology except foot process fusion on electron microscopy Treated with pred and cyclophosphamide for recurrent relapses. Can go on to develop FGFS.

Answer 71

Mainly in children although accounts for 28% nephrotic syndrome in adults Main presentation is nephrotic syndrome with normal renal function and normal histology except foot process fusion on electron microscopy Treated with pred and cyclophosphamide for recurrent relapses. Can go on to develop FGFS.