Over the last few days I have been studying Sanjay Sharma's book and have summarised the topics which I found difficult. Hopefully it migh be of help to others during their revision of this book for MRCP Part 2 Exam.
Sanjay Sharma revision (question numbers in brackets)
1. ( RUQ pain, fever, jaundice --> ascending cholangitis. can occur even after cholecsytectomy. Inv. blood cultures (E.coli), U/S to exclude abscess. liver biosy should never be 1st test for jaundice and fever.
2. (10) Histiocytosis X: manifestations are due to histiocytomas 2-4 yrs, M>F. lung fibrosis and COPD, bone infiltration (esp skull), proptosis, deafness, hepatosplenomegally, lymphadenopathy, diabetes insipidus (pituitry infiltration). Dx by histology (small round cells). Rx steroids, occasionally vincritine. occasional spontaneous regression.
3. (12) flow volume loop: URT obstruction decreased inspiratory flow.
4. (12, 39) leucoerrythroblastic blood film: marrow infiltration, leaukaemoid reaction to infection (dohle bodies, toxic granulations). Differentiaite by presence of hepatosplenomegally or lymhadenopathy and absense of left shift).
5. (14) inc Ca, inc BP, palpitations, goitre --> MEN2 = thyroid C cell Carcinoma, parathyroid adenoma, phaeochromocytoma. Ret oncogene (chr 10). calcitonin elevated. Relatives need regular screening.
6. (15) echoes of mitral valve prolapse (posterior motion of mitral valve in systole) and atrial myxoma (obliteration of mitral valve orifice)
7. (16) sickle cell disease: hyposplenism means encapsulated bacterial infection, chronic low grade HEMOLYSIS --> pigment gallstones
8. (17) antiphosholipid syndrome – false positive VDRL, recurrent thrombosis/miscarriage, in vitro prolonged clotting --> RAISED APTT +/- PT, does not correct with addition of normal plasma due to LUPUS anticoagulant. Rx prophylactic anticoagulants. Assoc livedo reticularis (conn tiss disorders), pulm hypertension, cerebral problems, valvular heart disease, low plt.
9. (1 alkapatonuria aut rec – homogentesic oxidase deficiency. Urine darkens on standing, ochronosis (pigmented cartilage, conn tissue e.g. Ears, joints). Premature arthritis. Xray --> intervertabral disk calcification and aorta. Rx low protein diet, poorly affective.
10. (27) echordiography: mitral valve lesions
11. (2 porphyria cutanea tarda – liver disease, SKIN rash (hands, head). Uroporphyrin decarboxylase deficiency. XS uroporphyrin -> photosensitive blistering rash (PAS staining), cirrhosis. Precipitants are enzyme inducers: alcohol, anticonvulsants, oestrogens. There is often secondary haemaochromatosis, secondary polycythemia. Rx --> alcohol abstainence, avoid sun exposure, venesect if Hb >12. Chloroquin binds uroporphyrin. End product blocked is corporoporphyrin.
12. (30) purple = positive gram stain. (it went Positively Purple with pleasure). Pink = negative. GPCocci=Strep/Staph. Gndiplococci=Neisseria, GNrods=HAEMOPHILUS . TB=ZN red.
13. (31) precipitants of HONK – XS sugary drinks, infection, thiazides, steroids, MI. If hypernatraemic use 0.45% N Saline, half strength sliding scale, DVT prophylaxis. Decrease osmolality gradually. Treat underlying cause.
14. (32) genetic pedigrees. 1. look at transmission – no male to male = X linked, 2. female to male/female only then mitochondrial.
15. (32) alports syndrome: x linked dominant: renal failure in males (females haematuria), senorineural deafness, ocular abnormailities (myopia, cataract, retinitis pigmentosa – see pic). Post renal transplantation anti-GBM disease because original defect was type 4 collagen in GBM (goodpasture antigen).
16. (34) complications of nephrotic syndrome: portal vein thrombosis (abnormal lfts) diagnose by US, venography Rx. Thrombolysis
17. (40) Chronic Active hepatitis – risk: old blood transfusion. Usually asymptomatic, chronic progressive. Causes: Hep B (eAg present) and Hep C (anti-HCAb's) also SLE, LKM, methyldopa, isoniazid, antytipsin, Wilsons, UC). AST, BR elevated >>ALP. Piecemeal necrosis. Rx. Iv IFN, success 30%, often relapse.
18. (41) Cardiac catheter values – 1. look for unusual pressures and saturations, 2. look for gradients across pulmonary and aortic valves. mitral valve disease reflected in PCWP.
19. (44) Acute intermittent porphyria – aut dom, Chr 11 PBG deaminase deficiency. 5F>M. Increased dALA and PBG blood and urine--. gastro and neuro sequelae, (SKIN manifestations rare cf PCT). Precipitants: drugs (barbiturates, anticonvulsants), alcohol, fasting, sepsis, OCP. Presents as acute abdominal abdomen (can mimic surgical abdomen but AXR normal). Neuropathy/ANS excitation/motor paralysis including CN's, epilepsy, hyponatraemia (SIADH). Rx 1. withdraw precipitant, 2. high carbohydrate diet /iv dextrose (reduces haem precursors in blood by reducing ALA sythetase activity), 3. haematin iv, 4. opiate analgesia. Avoid metoclopramide (another precipitant), B blockers for tachy. Epilepsy: calories, fluid restriction (inc Na), chlormethiazole or diazepam (most other anticonvulsants are preciptants). Tests: erlichs dye --> red (also jaundice) PLUS chloroform --> purple sedimnt (porhyria).clear (bilirubin). (Watson Scwarz test). Levels elevated in attack but may be normal when well. Can assay red cell PBG deaminase (low), ALA dehydrogenase (high).
20. (51) Causes of erythema multiforme. Infection (mycoplasma, HSV, orf, TB), drugs (penicillin, barbs, sulphonamides), other (conn tiss dx, vasculitis, internal malignancy)
21. (52) Hereditory angioedema (c1 esterase inhibitor deficiency) aut dom. Attacks preceded by painful macular rash. precipitants: trauma. Laryngeal oedema, abdominal pain. Normall rx supportive responds in 72 hours. Can give FFP if severe/protracted. Long-term rx. Danazol (inhibits plasmin) Hydrocortisone/chlorpheniramine less effective cf. anaphylaxis. Diagnosis C1 esterase inhibitor (low), c1 esterase (high), c2 & c4 both low.
22. (57) Aortic dissection: proximal --> urgent Surgery . Seen clearly on 2D echo. Important to exclude before thrombolysing any MI. assoc. hypertensive blacks, Marfans, Ehrler Danlos, pregnancy, coarctation.
23. (5 Scurvy: elderly, normocytic anaemia, bruising (haematomas), prolonged bleeding time --> always think of vitC deficiency. Easy bruising, bleeding gums, periosteal haemmorhage, muscle haematomas. Classic: perifolicular haemorhage, corkscrew hairs. APTT/PT normal, BT increased. Dx by plt or leucocyte ascorbate concentration. Rx. Vit C.
24. (71) livedo reticularis: assoc vasculitis and conn tiss disease e.g. PAN
25. (71) polyarteritis nodosa – males 60-70 myalgia, night sweats, weight loss, livedo reticularis, testicular pain, assoc HbsAg. Multisystem (neuro, gi, renal, cvs), aneuysm --> thrombosis -->organ infarction. dx. ANCA suggestive, organ biopsy , microaneurysms on angiography. Rx. Steroids.
26. (72) Heinz bodies: low Hb, high retic count -> bleed or HEMOLYSIS . Differentiate by blood film --> Hz bodies (pptd Hb) seen in Hbopathies or metHbaemia (Fe3+) e.g. Sulphasalasine Rx esp if G6PD deficiency. Cf Howell Jolly bodies, cf. Malaria blood films (can be confused)
27. (74) Apical lung fubrosis: TB, EAA, ankylosing spondylitis, apergillosis, radiation, sarcoidosis, histoicytosis X.
28. (76) FAMILIAL benign hypocalciuruc hypercalcaemia – aut dom. Usually lifelong asymptomatic hypercalcaemia occasional gall stones/pancreatitis/joint deposition. Difficult to distinguish between 1ry PTHism so patients often have uneccesary parathyroidectomy and FH parathyroidectmy. Can distgiuish because urinary calcium is LOW (it is high in all other causes of hypercalcaemia). Conservative management.
29. (77) Eruptive xanthomas: think hypertriglyceridaemia (associated with pancreaitis). There is pseudohyponatreamia due to lipids binding Na – the free sodium only is normally measured. Can differentiate by looking at serum osmolality.
30. (84) Wolf Parkinson White syndrome: accessory fast conducting atrioventricular pathway (Bundle of Kent). Ventricles depolarise prematurely --> short PR, slurred (delta wave) upstroke of QRS. 2 arrythmias possible: AV re-entrant tachycardia (P burried AFTER QRS Rx. Vagal stim, adenosine) , AF (delta waves sometimes seen Rx adenosine, vagal stim usually fails). 2nd line --> cardioversion. Definitive management radiofrequesncy ablation. Prevention with class I/III
(sotalol, flecainide, amiodarone, disopyramide). Avoid digoxin and verapamil as they increase accessory pathway conduction predisposing to malignant ventricular arrythmias.
31. (87) Polycythemia rubra vera – Hb can be normal if coexisting Fe deficiency anaemia (low MCV), look at PCV and rbc count to make the diagnosis. All marrow cells increased (wcc, plt) and splenomegally.
Incidious onset assoc lethargy, depression, vertigo, tinnitus, amaurosis fugax. Plethora, hypertension, spenomegally. Bledding (usually upper GI) due to throbocythaemia, stroke (hyperviscocity), gout (increased cell tunover). Can progress to myelofibrosis or AML. Rx. Venesection (can cause fe def anaemia). Busuphan and hydroxyurea to control thrombocytosis.
32. (91) Hypokalaemic periodic paralysis – aut dom, episodic paralysis classically while patient asleep or with prolonged rest. Ppt by dextrose and insulin, alcohol, cho, anxiety, tension. Rx can give K supplements or longeterm K sparing duiretics. Associated with orientals with thyrotoxicosis. Hypokalaemic arrythmias. Rx of thyrotoxicosis prevents paralysis too, propranolol also works. Only affects males. Need to exclude GBS or myasthenia gravis.
33. (94) Schmidtz syndrome (polyendocrine deficiency type II) – 10% of addisons pients have other endocrine abnormailies too - autoimmune hypothyroidism and IDDM. Type I PED = parathyroid, adrenal, candida, alopecia, pernicous anaemia. Vitiligo and hypogonadism occur in both. Hypothyroidism can cuase a large heart .
34. (97) Leprospirosis (rat faeces) – liver failure common, renal failure can also occur. Brucellosis (unpasteurised cows milk) – liver failure rare, assoc hepatosplenomegally, high fever and leucopenia.
35. (9 Atrial enlargement on ECG. R atrium = tall P II, V1 >2.5 small squares tall. L atrium= bifid P in II >3 small squares wide, V1 – negative portion p wave >1 small square duration AND amplitude.
36. (9 Sokolow's criteria for ventricular hypertrophy look at V1 ad V5/6: Right ventricle >12.5mm. Left ventricle >40mm. LVH shows more toward V6, RVH in V1.
37. (101) HIV infections: oral hairy leucoplakia (H=EBC infection) - white plaques on the lateral border of tongue Rx. Acyclovir cf candida rx. Nystatin, flucanazole. Crypotosporidium diarrohea – ZN stain of stool --> red cysts. Rx. Symptomtic + spiramycin/paromomycin.
38. (103) SLE – arthropathy, fever, polyserositis. 10F:1M, blacks, usully 6, LRTI, polm oedema, accelerated hypertension, arrythmia, bulbar involvement, campylobacter infection.
41. (110) Homocytinuria – cystathionine deficiency, aut recessive, chr 21---> homocysteine accumulation--> oxidised to homocystine (elevated urine levels). Cystine NOT produced. Defects in collagen crosslinking. Marfanoid features (tall, arachnodactyly, high arched palate, lens dislocation), osteoperosis, epilepsy/mental hadicap, venous/arterial thrombosis (OCP contraindicated), folate deficiency (inc MCV), premature coronary art disease. Rx. Restrict methionine, add cystine. Give Pyridoxine as soon as diagnosis is made.
Pictures to know:
1.Heinz bodies
2.Livedo reticularis
3.Lymphoerythroblastic bone marrow
4.retinitis pigmentosa
5.eruptive xanthomas
I will probably add another 25 facts before the exam
Dr Sujit Vasanth
Note: originally posted at http://www.rxpgonline.com/postt37085.html
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