OSCE 5th Mar

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RHEUMATOLOGY Q1. 5 year old male child presents to casualty with H/o fever- 3 days, rash over the forearm, legs and pain abdomen since 2 days . O/E the resident confirms the above findings and describes the rash to be non tender, reddish lesions with irregular surface. What is likely diagnosis b) What is the commonest type of abdominal pathology causing pain in above condition

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c) His urine shows proteinuria 2+ and haematuria. How long will you follow up this child d) Indications of using immunosuppressants in this child

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Ans1. a) HSP b) Ileoileal Intussception c) Glomerulonephritis- 3 mo(min), if abnormal a 3 mo- regular follow up d) Scrotal edema Intussception Nephritis Neurological manifestation

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Q2 An infant presents to ER with h/o excessive irritability and fever since 1 week. O/E the resident finds him to have a temperature of 102F, HR-150/mt RR-38/mt BP-100/60 mmHg. He has a fine rash over his upper chest and his throat and oral mucosa is congested. Mother has been giving Ciprofloxacin eye drops for the conjunctivitis which he developed 3 days ago as prescribed by his local physician. His liver is 3 cm and is tender to palpate. Likely diagnosis b) What drugs would you use for Rx. Mention doses

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c) What will be USG abdomen findings d) Name 4 common D/Ds

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Ans 2. a) Kawasaki Disease b) IVIG 2gm/kg slowly over 10-12 hours Aspirin (initial 100mg/kg/d then 3-5 mg/kg/day) C ) Hydrops Gall bladder d) Measles Scarlet fever JRA Drug rxn/ Toxic shock syndrome

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Q3 10 day old newborn referred to nursery by a local doctor as he found funny pattern of his cardiac activity. O/E child had fine rashes over the face. ECG done in ER showed a heart block Diagnosis b) Pathogenesis of this disease c) What is the Rx of this heart block d) What is the serious complication

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Ans 3 a) Neonatal Lupus b) Transfer of anti Ro ab b/w 12-16 wk of gestation c) Cardiac pacing d) Cardiomyopathy

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Q4 TRUE/ FALSE about SLE Oral ulcers are a very rare C/F in adolescent with SLE b) Complement levels are important to be monitored serially in cases of CNS lupus c) Mycofenolate Mofetil is better than Cyclophosphamide in treatment of severe lupus. d) LMW heparin is the anticoagulant of choice in APLA syndrome

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Ans 4 a) False: common b) False: Lupus nephritis c) false: Cyclophosphamide + Steroids in gold std d) True

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Q5 TRUE/ FALSE about JIA Infliximab is a monoclonal ab to TNF alpha b) DOC for long term treatment of polyarticular JIA is steroids c) Polyarticular JIA has 4 or more joints involved d) MAS in context of JIA refers to minimal access surgery

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Ans 5. a)True b) False: Methotrexate-DOC c)False: 5 or more d) false: macrophage activation syndrome

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Question 1 1.Describe 3 diagnostic characteristics of the cells in the smear 2.Diagnosis 3. Name 2 special stains which can aid in diagnosis

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Answer 1 1. Features Increased nuclear/cytoplasmic ratio Dense chromatin Pale cytoplasm 2. ALL L1 3. PAS, Sudan Black, NSE, MPO

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Question 2 The smear shows macrocytic RBC What is the normal MCV of a newborn child Formula for MCV according to age 3. 3 Non nutritional causes of macrocytic anemia

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Answer 2 Normal MCV: 95-120 ( Nelson, reference range) Formula for lower limit for normal MCV : 70+age in years Causes of macrocytic anemia: Orotic aciduria Hypothyroidism Liver disease

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Expand the term NESTROFT Tests for Prenatal diagnosis of thalassemia Question 3

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Answer 3 1. Naked Eye Single Tube Red Cell Osmotic Fragility Test 2.Methods CVS and Fetal DNA analysis Cordocentesis with globin chain synthesis in fetal blood samples ( beta/alpha ratio)

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Question 4 Identify the abnormality pointed Give 2 differential diagnosis Describe Porter Index

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Answer 4 1. Basophilic stippling 2. Thalassemia Lead poisoning 3. The desferal therapeutic index or Porter index is defined as mean daily dose of desferrioxamine in mg/kg, divided by serum ferritin This is calculated every 6 months in paitents receiving Desferroxamine Porter Index should not exceed 0.025 inorder to minimize sensorineural hearing loss.

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Question 5 Diagnosed case of Megaloblastic Anemia 1. Identify the cell 2. Describe the neurological findings that may be observed in this condition 3. Name the urinary investigation in this condition

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Answer 5 1. Hypersegmented neutrophil (>5 lobes) 2. Neurological findings: Paraesthesias Impaired position and vibration sense Ataxia Hyper or hypo reflexia Positive babinski a 3. Excessive excretion of methyl malonic acid in urine is a sensitive index of Vit B12 deficiency 3.

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Question 6 What is the classic pentad of TTP? What is the familial etiology of TTP? Management of TTP?

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Answer 6 1. Pentad Thrombocytopenia Microangiopathic hemolytic anemia Neurological abnormalities Renal dysfunction Fever 2. Congenital deficiency of metalloproteinase can result in familial TTP 3. Plasmapheresis

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Question 7 Seen here is the bone marrow aspirate of a 15 month old child with a history of hypertonicity, aspiration pneumonia and hepatosplenomegaly. Describe the findings Give 2 clinical differential diagnosis Management

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Answer 7 Two macrophages are shown which have a fibrillar, crumpled appearing cytoplasm and eccentric nuclei, consistent with Gaucher cells. The other hematopoietic elements present show normal maturation. Niemann Pick, MPS Enzyme replacement

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Question 8 Describe platelet satellitism Clinical significance Next step in evaluation

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Answer 8 1. Platelet satellitism : platelet clustering around neutrophils in the presence of EDTA 2. Pseudothrombocytopenia 3. Repeat platelet count in citrate sample

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Question 9 Identify this instrument Procedure for which this instrument is used What is the minimum platelet count to be kept prior to this procedure

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Answer 9 Jamshidi Bone Marrow biopsy needle Bone Marrow biopsy No cut off for platelets needed prior to Bone marrow study 1. Very easy to control bleeding as it is superficial 2. Release of thromboplastin: effective thrombosis

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NEPHROLOGY Q1) A 3 year old male child developed a viral upper respiratory tract infection. After a week of the above illness he became irritable and lethargic. Mother noticed decreased urine output and took the child to a local physician who found the child to be pale and referred him to a higher centre where the investigations revealed the child to have a BUN=74 mg% and S.Creatinine =4mg %

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What can be the possible findings on peripheral smear of this child. Would you use any special stains. Name the common organism this disease is usually associated with c) What diet would you advise this child to have d) 6 months after recovery this child has a creatinine of 2mg% and a phosphorus of 8mg/dl. Please comment

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Answer 1 I. Peripheral smear: Burr cell, helmet cell, fragmented RBC, leucocytes, thrombocytopenia Spl . Stain- for reticulocyte counting b) E coli 0157:H7 (80% of HUS) Shiga toxin c) Restrict K+ Moderate consumption of protein Restrict salt if increase in BP d) Probably CRF (10% of acute HUS- CRF)

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Q2 A child being evaluated for haematuria was found to have a normal coagulation profile,normal renal functions,complement level and no proteinuria with normal BP and Ht a)What imp initial investigation is missing in the above question. b) His USG KUB and Sickle preparation is normal. What would be the next step in evaluating this child

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c) After evaluation parents were advised to start the child on some drug after which his urine frequency increased. He was advised sodium and Ca restriction. What is likely diagnosis. What is likely drug d) If this child has a refractory error and some hearing loss , what would be the likely diagnosis . How would you screen for same

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Answer 2. a) Urine analysis:If urine is cola colour, prot +, RBC cast+ - Glomerular origin b) Urine Ca/Cr ratio(>0.2), If infant < 7mo can be as high as 0.8 or a 24 hr urine Ca(>4mg/kg/day) c) Diagnosis- Idiopathic hypercalciuia Drug: Hydrochlorothiazide d) Alports syndrome Screening test 1. Urinanalysis of relatives 2. Eye check up 3. Hearing evaluation

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Q3 11 year old male child was brought to hospital with c/o facial puffiness(early in morning) since past 3 months. Was told by his doctor to have some kidney problem and started on wysolone-50mg/day(divided doses) , 2 months back. His urine protein at beginning of Rx was 4+ and after 1 mo of Rx was 3+ and presently since past 1 wk 2+/3+. His BP is 140/90 mm hg, wt=28 kg, ht=125 cm, and has mild edema .

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Likely diagnosis How will you investigate Renal histology picture d) If you want to start him on cyclophosphamide/ cyclosporine, what precautions would you take

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Answer 3. a) Non MCD, NS, Steroid resistant b) C3, Renal biopsy c) Mesangial prolif/ FSGS d) Cyclophos-Requar CBC, haemorrhagic cystitis Cyclosporine- prior renal biopsy, serial Creat

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Q4 True/ false Losartan has antiproteinuric and antihypertensive effect b) RTA is associated with high A.G met.acidosis c) Urine pH< 5.5 and glycosuria/ proteinuria seen in type IV RTA d) K+ binding resin is given intravenous if S.K+ level is 7mEq/l

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Answer 4. True b) false: normal AG c) false: Type II RTA prox RTA, fanconi disease d) false: oral/ rectal

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Q5) YES/ NO Peak T waves is earliest ECG change in hyperkalemia b) ESRD is characterized by GFR < 25 ml/min /1.73m2 c) Known S/E of erythropoitien are iron deficiency and hypertension d) Approximate Cost of renal transplant in our country is 15 lakh/ transplant

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Answer 5. a) yes b) No (< 10) c) Yes d) No (3-4 lakhs)