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OSCE

OSCE. DR PRASHANT R UTAGE. 4. A) Identify the abnormality on peripheral smear? B) Name 4 conditions where these cells can be seen. Acanthocytes are seen in. 4. • Hereditary abetalipoproteinemia • Hereditary acanthocytosis • End stage liver disease • Anorexia nervosa • Malnutrition

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  1. OSCE DR PRASHANT R UTAGE

  2. 4 A) Identify the abnormality on peripheral smear?B) Name 4 conditions where these cells can be seen.

  3. Acanthocytes are seen in 4 • Hereditary abetalipoproteinemia • Hereditary acanthocytosis • End stage liver disease • Anorexia nervosa • Malnutrition • Post splenectomy • Intravenous hyperalimentation particularly with intralipid infusion

  4. 10 year male referred for autoimmune hemolytic anemia, frequent diarrhea, normal tonsils , father is a diagnosed case having low IgA levels. His immunoglobulin levels were low • What is the immunodeficiency type will you suspect? • How will you treat the patient?

  5. 4 • COMMON VARIABLE IMMUNODEFICIENCY • IVIg therapy, Septran Prophylaxis

  6. 4yr old child was followed by a paediatrician for a period of 1 year. He presented with haemoptysis, 1-2 episodes of malena.O/E: B/L wheeze and coarse crepsGen. Exam: Severe pallor, clubbing, cyanosis.CBC showed Iron deficiency anaemiaA] What is the diagnosis?B] What is the diagnostic investigation with findings?C] Which heart condition can give rise to this condition?

  7. Answers:A] Pulmonary haemosiderosisB] Bronchoalveolar lavage Haemosiderin laden macrophagesC] Mitral stenosis. 4

  8. This 5 year old boy was brought with learning disability and attention deficit. His examination revealed skin lesions Identify the skin lesions ?Give at least 2 features required for making the diagnosisWhat will the ophthalmic examination show?What is the inheritance pattern?

  9. Café au lait spots. Any two of the following 6 or more café- au –lait macules more than 5mm in prepubertal and over 15mm in post-pubertal individuals. Axillary or inguinal freckling. 2 or more lisch nodules. 2 or more neurofibromas. Optic gliomas. Opthal examination for lisch nodules Autosomal dominant. 5

  10. A full term ,male child develops jaundice on day 3 of life, (S. bilirubin –34 mg%)and undergoes an exchange transfusion . • Describe the signs of immediate complication likely to occur? • Where is the anatomical abnormality? • What are the long term complications? • Name 1 investigation you would insist on after discharge?

  11. 6 1)Kernicterus Phase (1)- Decreased alertness, hypotonia, and poor feeding Phase 2 (variable onset and duration): Hypertonia of the extensor muscles is a typical sign. Patients present clinically with retrocollis (backward arching of the neck), opisthotonus (backward arching of the back), or both. • Phase 3 (infants aged >1 wk): Hypotonia is a typical sign 2)Globus pallidus, dentate nucleus, cerebellar vermis, cochlear nuclei 3)Choreoathetoid cerebral palsy, dystonic / dyskinetic CP, Sensorineural deafness, 4)Audiometry testing

  12. 18) Name the sign 3 DDs 3 investigations in sequence of importance Inheritance/ locus

  13. 6 • Gowers Sign (1) • Duchenne, SMA III, Limb girdle dystrophy, BMD, myopathy(1.5) • Dystrophin gene study, EMG /NCV, CPK levels(1.5) • X linked recessive/ Xp 21(2)

  14. Describe the X ray Diagnosis Anatomical abnormality

  15. 5 1) Dextroposition with curvilinear shadow parallel to right heart border 2)Scimitar syndrome 3) PAPVC with R Lung sequestration

  16. Describe the ECG 2 Conditions associated

  17. 6 • Lead II showing biatrial enlargement (1) • P wave more than 2.5 sec duration and 2.5 mm (2) • PR interval >0.3 sec(1) • Tricuspid atresia , ebstein anomaly , ECD.(2)

  18. A) Which 1 investigation would you advice?B) What is the diagnosis?C) Which type would he be categorized as?D) What is the mode of inheritance? 1.5yr old boy with minor injury develops this swelling?

  19. 6 6 A) PT/PTTK B) hemophillia A or B C) moderate D) X linked Recessive

  20. What is the dose and schedule of Indomethacin given for the closure of PDA in a newborn who is less than 48 hrs old? • Name another condition which can be prevented by administration of indomethacin in a preterm newborn. • What are the four main Contraindications to the use of indomethacin.

  21. 5 • 200 microgram stat foll by 100 microgram foll by 100 microgram at 12 to 24 hr interval.(2) • Intracranial Hemorrhage.(1) • Poor renal function, Thrombocytopenia, NEC, sepsis.(2)

  22. A 8 day old baby was brought to PHC with C/O difficulty in feeding , crying excessively,& seizure. Parents of baby were 2nd degree cousins & labourers by occupation. Mother had not received any ANC care. Baby was at home., had moderate cry , was well till D8 .His picture is shown HR-140/min RR-42/min AF-at level, sutures - normal Tone- Increased in all 4 limbs with neck retraction, intermittent seizures. , worsened with stimulus . (a) What is the diagnosis of this baby ? What is the treatment? What is the usual cause of death in this condition?. What are the various types?

  23. 5 • Neonatal tetanus (1) • Penicillin,tetglob,Musle relaxants- Diazepam, Baclofen.(1.5) (c) Recurrent seizure causing airway obstruction.(0.5) (d) Neonatal, Localized , Generalised, Cephalic (2)

  24. Describe the karyotype Clinical features

  25. 5 • Klinefelter's syndrome, XXY male(1) • Occurs due to nondisjunction of X chromosomes during prophase of meiosis I in females.(2) • sterile male who may have some female body characteristics, and increased learning hurdles (2)

  26. Barr body is a ----------------------(1) • What does RFLPs mean in genetics(2) • Mother is A+ and Child is B+ what should be blood group of the father , give explanation (2) A. B or O B. A, B, AB or O C. AB or B D. A or B E. A, B, or AB

  27. 5 A1)The inactive X-chromosome in female somatic cells, visualized as a densely staining body within the nucleus and attached to the nuclear envelope, is known as a "Barr body.“ A2)RFLP: "restriction fragment length polymorphisms“ A3) The child in question has blood type B. Since the mother is blood type A, the child must have received an O allele from the mother, and a B allele from the biological father. The mother's blood type is AO, the child is BO, and the father is either AB or B, which are the only two types that could be the source of a B allele. • If the father is type B, the genotype could be either BB (homozygous) or BO (heterozygous).

  28. 8 month old boy brought to the clinic with failure to thrive, recurrent vomiting and polyuria. On examination, the child weighed 5.5 kg for a birth weight of 3 kg. He was normotensive, had no evidence of rickets, and systemic examination was normal. Investigations revealed Hb -12.1g%, BUN -8mg%, s. creatinine -0.4 mg%, s. Na -141mEq/l, s. potassium -2.5mEq/l, s. Cl -90mEq/l, blood gas pH -7.52, HCO3 -30mEq/l. Blood levels of renin and aldosterone are markedly increased. Urinary chlorides 35mEq/l. Questions (A) Diagnosis? (B) Differential diagnosis? (C) What is the treatment?

  29. 7 • Bartter’s syndrome(1) • Gitelman’s syndrome presents at a later age and is milder in presentation. Hypomagnesemia and hypocalciuria are features of this syndrome(2) • Potassium supplementation of 3 to 4 mEq/kg/day.& Indomethacin 1 to 4 mg/kg/day.(7)

  30. A 13 month old infant known case of collecting bilateral VUR and posterior urethral valves lost to follow up after initial diagnosis, came with the history of high grade fever and irritability. Urine routine revealed 50 WBC/hpf. Patient was admitted in view of persistence of fever inspite of starting appropriate antibiotic therapy. He was severely dehydrated. After infusing Ringer Lactate, he became more tachypneic and tachycardiac and less responsive. He went into shock . In the ICU, cardiac monitor showed marked arrhythmia, predominantly ventricular tachycardia. The lab results showed, ABG= pH -6.853, pO2 – 39.3 mm/Hg, HCO3 – 8.1mEq/l, S. Na- 145 mEq/l, S. K – 8.3mEq/l, BUN – 82 mg/dl, S. Creat – 4.9 mg/dl. Questions (A) What is the electrolyte disturbance causing the worsening of the condition? (B) What are ECG changes seen in order of severity? ( C )What is the treatment ?

  31. 7 A) Severe hyperkalemia( no mark) B) The T-waves become tall and peaked with prolongation of PR interval.(2) The P wave becomes smaller and may eventually disappear. The QRS complex widens and becomes distorted and a “sine wave” pattern may appear. Ventricular fibrillation Cessation of electrical activity C) Cardiac and ventilatory support to be instituted (5) Specific measures for hyperkalemia- Infusion of NaHCO3 and Ca gluconate. Infusion of glucose and insulin. Use of sodium polystyrene sulfonate retention enemas.. Dialysis.

  32. 18 month old child with pallor and cutaneous markers at the back with an abnormal skeletal profile. P/S was consistent with macrocytic anemia and pancytopenia.

  33. What is the most probable diagnosis? • Name the cutaneous marker. • What malignancies are expected in this patient?

  34. 3 • Fanconi anemia • Café au lait spot • Acute leukemia

  35. 3 year old girl child, with a weight of 7 kg and height of 75 cm, is brought with c/o difficulty in seeing and pain in left eye with discharge. The child also has h/o recurrent episodes of loose stools and pneumonias. The examination findings are shown below:

  36. What is the diagnosis? • Enumerate the WHO staging and mention what stage this child belongs to? • What is the prognosis? • How can this be prevented?

  37. 8 A. Vitamin A deficiency(1) B. Stage X3B(4) WHO Staging: Primary signsX 1 A: Conjunctival xerosisX 1 B : Bitot's spots X2 : Corneal xerosis X3A : Corneal ulceration (<1/3rd) X3B : Keratomalacia Secondary signsXN : Night blindnessXF : Xerophthalmia fundus XS : Corneal scars C. Keratomalacia is usually irreversible and results in scar formation(1) D. Administer one dose of 1 lac units of vitamin A at 9 months followed by 4 more doses of 2 lac units eact at 18, 24, 30 and 36 months, Consumption of Vitamin A rich food should be encouraged (2)

  38. C A B Identify the approximate ages of these children E D

  39. 5 • 13 months • 9-10 months • 6 months • 4-5 months • 8 months

  40. What are WHO-MGRS charts? • What are the exclusion criteria used in these charts? • What are the advantages of these charts over the CDC/NCHS charts?

  41. 7 • . WHO- Multicentric Growth Reference Study charts, developed in 2006(2) • Exclusion criteria:(2) -Formula fed infants -Children of mothers who smoked during pregnancy or after. -Morbidities that affect growth (repeated bouts of infection diarrhea and Crohns dis C. Advantages(3) -New growth charts that would show how children should grow in all countries rather than merely describing how they flow at particular time and place. -MGRS is a standard rather than a reference. -Any deviation from suggested pattern are e/o abnormal growth. -MGRS provide a solid foundation for development of a standard b’coz they are based on healthy children living under condition likely to favour achievement of their full genetic potential

  42. 25% 50% 75% 90% • Identify in which test this bar is used • Suppose a mother comes to you for routine check up of a 8 month old infant. You do a developmental assessment of the child and you plot the child at the point marked above in all areas of development. What do you interpret from this? • Had the child been between 75th-90th centile in 2 areas, would you change your interpretation? What would be the implications?

  43. 5 • Denver Developmental Screening Tool(1) • The child is normal as per his age because his age line falls between 25th-75th centile(2) • Yes, they would have changed. If the child would have fallen between 75th-90th centile, he would be put as caution and would have required close follow up(2)

  44. This female neonate was born with a large mass in relation to the umbilical cord

  45. Identify the condition • Give three important aspects that you will take care of in the transport of such a neonate.

  46. Answers 4 • Exomphalos major/ omphalocele • Transport supine with the hernia suspended by a string • Cover the omphalocele with a waterproof covering • Provide additional fluids

  47. Observed Observed Station • Give intradermal BCG vaccine

  48. Material Required Station 1 • Insulin Syringes • Dummy • BCG Vial • Cutter • Spirit • Saline ampoules

  49. Methodology For the Examiner 10 • (Each step carries 2 points) • Amount of vaccine (0.1 ml = 0.1 mg of reconstituted vaccine) and Load in to syringe (Breakage of vial) • Selection of area (Left deltoid just above its insertion) • Don’t clean the area with spirit • Keep the beveled end of needle up and technique of insertion • Don’t rub the area

  50. A patient is admitted to the ICU with the following lab values: • BLOOD GASES under room air • pH: 7.199PCO2: 32.2HCO3: 12PO2: 86.6 • ELECTROLYTES, BUN & CREATININE • Na: 136 ,K: 4 ,Cl: 103 • Describe metabolic condition (1) • Describe compensation (calculate exact compensation) (1) • Calculate anion gap (1) • Name two conditions with similar anion gap as above (2)

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