DNB Pediatrics OSCE CME (Command Hospital, Pune)


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DNB Pediatrics OSCE CME - Command Hospital, Pune

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  • Gradrng of vesicoureteral reflux. Grade I: reflux into a nondilated ureter. Grade II: reflux into the upper collecting system withour dilatation. Grade III: reflur into dilated ureter and/or bluntinu of calvceal fornices. Grade IV: reflux into a grossly dilated ureter. Grade V Massive reflux. With significant ureteral dilatation and tortuosity and loss of the papillary impression
  • Acrodynia The symptom complex included swelling and redness of the hands and feet, skin rashes, diaphoresis, tachycardia, hypertension, photophobia, and an intense irritability with anorexia and insomnia. Infants were often very limp, laying in a frog-like position, with impressive weakness of the hip and shoulder girdle muscles. Similar symptoms have been described in children exposed to other forms of mercury, including broken fluorescent light bulbs or diapers rinsed in mercuric chloride.
  • DNB Pediatrics OSCE CME (Command Hospital, Pune)

    1. 1. COMMAND HOSPITAL PUNE 25TH July 2008
    2. 2. INTRODUCTION toObjective structured clinical examination Observation Stations & Clinical Examination 5 min each
    4. 4. G cen AB ario G Kar yBio otype Blank OSCE Sheets sta tisti cs
    5. 5. 1. STATIONS:2. OBSERVED STATION: Foreign Body Apiration: demonstrate action on a manikin3. Statistics4. Neonatal Chest X ray with MAS and umbilical venous cannulation. Qs on complications and heparin infusion5. Rest Station6. Spirometry7. OBSERVED STATION: History Taking: examiner is observing your procedures.8. History taking of a 4 year old child with febrile seizures—have all the points to diagnose, prognosticate and manage the child covered.9. Congenital Anomaly: Qs on diagnosis, prognosis and causes of death.10. Photomicrograph of a PBS of a haemolytic anaemia. Qs on the abnormalities of the RBCs, the diagnosis, two inv, two treatment stratigies.11. X- Ray Chest of a CHD. Qs on radiological findings, diagnosis, antenatal history, DD, definitive manangement of the condition.12. Cavernous sinus thrombosis13. OBSERVED STATION: drug counselling to the mother of a child with bronchial asthma: from the drugs to devices.14. Photomicrograph of a PBS of different types of mononuclear WBCs. Qs on identification of the cells.15. Photograph of a dermatological condition. Qs on diagnosis, etiology, management.16. Vaccine: Qs on a particular vaccine, its storage, route, indications.17. Drug: Qs on the mechanism of action, group, metaboism, SEs.18. OBSERVED STATION: Examination of a system.19. Photograph of a Karyotype. Qs on the abnormality, diagnosis, 3 CFs, type of inheritance.20. Micronutrients and their deficiency disorders.21. Biostatics: calculation of relative risk, Odd’s ratio and Attributable risk.22. Photograph of an ectoparasite. Qs on identification, disease caused, drug therapy.23. ECG: abnormality, axis, diagnosis, complications, management.24. OBSERVED STATION: examiner asses the skills. Newborn gestational age assessment.25. REST STATION26. Neonatal: HMD:Qs four risk factors, ventilation modalitiws, ABG: a/A ratio, A-aDO2, OI for the ABG & ventilator settings.27. Lab Test: Haemolytic anaemia, antenatal diagnosis, management.28. Short Stature scenario with Ht, Wt, SMR, Bone age given. Qs on endocrine inv.29. Drugs: doses, routes, antidotes.30. Clinical Situation: GBS31. Photograph of oxygen delivery devices: Qs on Fio2 rates delivered.32. REST STATION
    6. 6. X-Rays/CT/MRI
    7. 7. If patient presents with fever and toxaemia, give three differential diagnosis Give three modalities of management
    8. 8. 1. Lung Abcess2. Infected Bronchogenic cyst3. Infected Hydatid Cyst1. Antibiotics (anaerobic +aerobic)2. Chest physiotherapy3. Percutaneous CT guided aspiration 0.5 each Total 3 marks
    9. 9. What is the lesion ?Give two management modalities.What complication can occur during management & how can it be prevented?
    10. 10. NCC 1NCC:Anticonvulsants 1AlbendazoleIncrease in Cerebral Edema 1Use steroid cover Total 3 marks
    11. 11. Describe the lesion.What are the management modalities of such lesions especially if they are leading to complications ?
    12. 12. Haemangioma scalp 1Conservative 2Oral steroidsLocal steroidsPDL: pulse dyed laserIFN alfa interferon (in case of haemangiomas causing pressure effects or erosion leading to significant morbidity) Total 3 marks
    13. 13. Worrisome Hemangiomas Multiple cutaneous hemangiomas: Associated with visceral hemangiomas (e.g., liver) Large hemangiomas: May cause significant disfigurement of underlying structures and may be associated with congestive heart failure "Beard" hemangiomas: May be a marker for underlying laryngeal or subglottic hemangioma that may impair respiratory function Midline spinal hemangiomas: May be a marker for underlying spinal cord abnormality Head and neck hemangiomas: May be associated with other congenital anomalies, including central nervous system, cardiac, ocular, and sternal defects (e.g., Posterior fossa malformation, Hemangioma, Arterial abnormalities, Coarctation, Eye abnormalities, Sternal defects [PHACES] syndrome). Vulnerable anatomic locations: Impair vital functions, cause disfigurement (e.g. periocular, neck, lip, nasal tip) Ulcerated hemangiomas: Increased risk of superinfection, cause pain and lead to scarring
    14. 14. What are two imp radiological abnormalities?What is the diagnosis ?What are the three imp investigations ?What are the metabolic abnormalities expected?What is the management ?
    15. 15.  Distension of stomach No gas in the intestines Pyloric Stenosis USS abdomen: pyloric thickness >4mm/ pyloric length >14 mm S electrolytes Ba studies Hypochloremic, hypokalemic, metabolic alkalosis Management of the fluid & electrolytes Ramstedt’s pyloromyotomy 0.5 each Total 5 marks
    16. 16. What is the diagnosis ?What is the likely organism ?
    17. 17. Pnematoceles 1Likely organism Staphylococcus 1 Total 2 mark
    18. 18. Give five radiological findings ?What is the diagnosis ?What is the management ?
    19. 19. 1 Ground glass appearance of bone 22 Thinned cortex3 Periosteal calcification4 White line of Fraenkel (well calcified cartilage)5 Wimberger’s sign (white ring)Scurvy 11 Vit C 100-200 mg/ day12 Dietary Therapy Total 4 marks
    20. 20. What was the lesion ?What was the procedure carried out ?What are the complications ?
    21. 21. VSD 1Device implant 1Device displacement 2Emboli formationHaemolytic Anaemia Total 3 marks
    22. 22. Name the Investigation ?What is the diagnosis ?What are the further investigations ?
    23. 23. MCUPosterior urethral ValvesDTPA (diethylene tetra amine pentoic acid) FunctionDMSA (dimercapto succinic acid) -scarring Total 3 marks
    24. 24. What is the abnormality?What is the diagnosis ?How is it suspected clinically ?What is the management ?
    25. 25. 1. .Bowelloops in lt hemithorax, mediastinal shift to right 12. Congenital diaphragmatic hernia 13. Respiratory distress Mediastinal shift Bowel sounds in the thorax Scaphoid abdomen 24. Avoid B&M vent prop up and decompress stomach Ventilate Treat PPHN Surgical correction Antenatal tracheal ligation 3
    26. 26. CDH Pleuroperitoneal membrane defectFetal lung development Pseudoglandular stage 5 to 16 weeks Canalicular 16 to 24 weeks Saccular stage Alveolar stage continues till 8 years of life
    27. 27. What is the abnormality?What is the likely diagnosis? Delineate management. Mention 3 complications
    28. 28. Multiple fluid levels. gasless lower abdomen 1 Small bowel obstruction 1 Surgical correction 1 Dyselectrolytemia 1 Perforation Exaggerated hyperbilirubinemia Dysmotility syndromeMartin: Fanaroff and Martins Neonatal-Perinatal Medicine, 8th ed.,
    29. 29. Describe the X –rayDiagnosisGive three recent advances in management
    30. 30. Hyper inflated lung fields with areas of atelectasis interspersed with areas of overinflation 1MAS 1Lung lavage surfactant instillation HFOV NO liquid ventilation 2
    31. 31. What is the abnormalityThree high risk situations when this condition is imminentManagement
    32. 32. Pneumothorax 1CDH Ventilation Post surfactant ventilation 2Chest tube drainage 1
    33. 33. What is the abnormalityWhat is it a complication of
    34. 34. Pneumomediastinum 1Forceful ventilation 1
    35. 35. Identify the abnormalityClinical presentationTreatment
    36. 36. Pneumopericardium 1Shock with weak pulses 1Drainage 1
    37. 37. What is the diagnosis ?
    38. 38. 1 mark
    39. 39. What are the MRI findings ?What is the DDWhat further inv will you do on the CSF to confirm the diagnosis ?
    40. 40. Acute disseminated encephalomyelitis 3 marks
    41. 41. Oligoclonal bands are also found in: Multiple sclerosis Devics disease Systemic lupus erythematosus Neurosarcoidosis SSPE Subarachnoid haemorrhage Syphilis CNS Lymphoma
    42. 42. What are the MRI findings ?What could be the etiology ?
    43. 43. Multifocal cerebral & cerebellar encephalomalaciaSequel of ischaemic injury in the perinatal period. 2 marks
    44. 44. What is the likely diagnosis ?What is the IP?Which is the commonest site?
    45. 45. TB Spine 12 YRS 1Thoraclumbar area of max stress valveless venous drainage 2 4 mark
    46. 46. What are the MRI findings ?Likely etiologyLikely CSF picture
    47. 47. TBMIncreased proteins 3 mark Increased cells likely lymphocytes Low sugar
    48. 48. What is the X ray suggestive of ?What is the likely organism?What are the complications?What is the drug of choice?What is the duration of therapy?
    49. 49. Lobar PneumoniaPnemococous/StaphylococcusSynpneumonic effusion, empyema,Penicillin for susceptible org and cefotaxime / vancomycin for penicillin resistant org10-14 d 5 marks
    50. 50. Questions1. What is the anatomical structure in which coin is lodged?2. What is the location of carina with respect to thoracic vertebrae?3. What are the anatomical areas of esophageal narrowing?4. How can this foreign body be removed?
    51. 51. Answers 1Esophagus When foreign bodies lodge in the esophagus, the flat surface of the object is seen in the AP view *T 4 1Anatomic areas of esophageal narrowing 3 Cricoid Tracheal bifurcation Gastro-esophageal junctionEndoscopy 1 *
    52. 52. L
    53. 53. Question1. What is the diagnosis?2. Fill in the blanks In an exudative pleural fluid a. Proteins > b. Pleural Fluid LDH > …………. c. Fluid to serum LDH ratio > ……….. d. Cell count ………3. What does VATS stand for?
    54. 54. Answers 21. Pleural Effusion (Right)2. Fill in the blanks In an exudative pleural fluid 0.5 a. Proteins > 3.0 g/dL 0.5 b. Pleural Fluid LDH > 200 IU/L c. Fluid to serum LDH ratio > 0.6 0.5 d. Cell count > 1000 0.53. Video Assisted Thoracoscopic Surgery 1
    55. 55. Questions1. Diagnosis?2. What are the first two steps in treatment of hypoxic spell?3. In a cyanotic newborn, how can you distinguish pulmonary disease from cyanotic congenital heart disease?4. Which cardiac conditions are associated with following a. Egg Shaped Heart b. Snowman silhouette c. Rib notching
    56. 56. Answers 1.51. Cyanotic Congenital Heart Disese Probably TOF  The heart size is normal  Pulmonary vascular markings are decreased  A hypoplastic main pulmonary artery segment contributes to the formation of the “boot-shaped” heart. Pediatric cardiology Myung K Park 5th ed
    57. 57. Answers 12. Knee Chest Position 1 Morphine 13. Hyperoxia Test4. X-ray appearances a. Egg Shaped Heart Transposition of great arteries 0.5 b. Snowman silhouette Total anomalous pulmonary venous return (supracardiac) 0.5 c. Rib notching Co-arctation of aorta (long standing) 0.5 Park: Pediatric Cardiology for Practitioners, 5th ed.
    58. 58. 3 day neonate withLethargyFeed refusalAbdominal distension
    59. 59. Questions1. What stage of NEC is depicted in the X-ray?2. What is the radiological feature of Bell stage III NEC?3. Name two more conditions associated with pneumatosis intestinalis?
    60. 60. Answers 1 1. NEC Stage II 1 2. Pneumoperitoneum 2 3. Any two of following Hirschsprungs disease, Pseudomembranous enterocolitis, Neonatal ulcerative colitis, Ischemic bowel diseasePART 5 Neonatal Necrotizing EnterocolitisMartin: Fanaroff and Martins Neonatal-Perinatal Medicine, 8th ed.,
    61. 61. 6 weeks infantCase of Cholestatic jaundice (Extra-Hepatic Biliary Atresia)c/o swelling left thigh
    62. 62. Questions1. What is the likely cause of fracture femur in this case?2. How can this complication be prevented?3. How do you manage pruritus in these patients?4. An infant with cholestasis, triangular facies, and a pulmonic stenosis murmur is likely to have what syndrome?
    63. 63. Answers 11. Metabolic Bone disease (secondary to Vitamin D deficiency due to malabsorption of fat soluble vitamins) 12. Replace 5,000-8,000 U /d of D2,or 3 -5 µg/kq/d of 25-hydroxycholecalciferol3. Ursodeoxycholic acid 15-20 mg/kg/day 14. Alagille syndrome 1 (Arteriohepatic dysplasia)
    64. 64. L Previously healthy 7 years girl c/oc Sudden onset weakness right upper and lower limbt Facial palsy right (UMN) Normal sensorium No fever/ trauma/ seizures
    65. 65. Questions1. What is the level of lesion on MRI?2. What are the structures marked c t3. Which hemoglobinopathy can be associated with this kind of presentation?4. A dilated and unreactive pupil indicates the compression of what structure?
    66. 66. Answers 21. Infarct in the left basal ganglia, the posterior limb of internal capsule, and the head of the caudate 1
    67. 67. Answers 2. C Caudate 0.5 nucleus T Thalamus 0.5 P Putamen G Globus pallidus White arrows indicate the ant and post limbs of internal capsule 1
    68. 68. Answers 1 13. Sickle cell anemia 14. Compression of 3rd cranial nerve 1
    69. 69. Questions?1. Diagnosis?2. What is the treatment (mention complete schedule)?
    70. 70. Answers 11. Miliary tuberculosis2. 2HRZE + 7HR 1 IAP Group 4
    71. 71. 1. Diagnosis2. What are the embryologic events that lead to this development?3. What are three causes of respiratory distress in a baby born with this condition?
    72. 72. Answers 11. Congenital Diagphragmatic Hernia2. The posterolateral portion of the diaphragm has remained 1 open between the ninth and tenth weeks of gestation as a result the viscera will pass into the chest, and a CDH will result.3. a) Mechanical compression of the lungs from the herniated 1 viscera b) Pulmonary hypoplasia from compression of the developing lungs in utero 1 c) Pulmonary hypertension 1
    73. 73. QuestionsDescribe the lesion?Give two D/DWhat is the triad of tumor lysis syndrome?
    74. 74. Answers 1Osteolytic lesion of skull 2Histiocytosis Ewing`s Sarcoma Lymphoma 3 Bone cystHyperuricemia, hyperkalemia, and hyperphosphatemia
    75. 75. Questions1. Describe the X-ray appearance2. Pathogenesis of the appearance3. Possible Diagnosis4. Which disorder is most commonly associated with an elevated MCHC?5. How is the corrected reticulocyte count calculated?
    76. 76. Answers1. Sunray appearance 12. Medullary widening 13. Chronic hemolytic anaemia 14. Hereditary spherocytosis 15. Corrected retic count = reticulocyte % × (patient Hct/normal Hct) 1
    77. 77. Below is a midline sagittal cut of a MRI scan of thebrain. View the midline anatomic diagram of the brainand identify the following structures
    78. 78. AnswerS - Suprasellar cisternP0 - PonsP - Midbrain (cerebral peduncles)M - MedullaC - Quadrigeminal plate (superior and inferior colliculi)Q - Quadrigeminal cisternV - Fourth ventricle 1 mark each
    79. 79. Question1. Diagnosis?2. What is the emergency management of the condition?3. What is subsequent management after the emergency management is over?
    80. 80. Answers 11. Pneumothorax, 1 with mediastinal shift 12. Put in a needle in second intercostal space3. Intercostal drain 1
    81. 81. Questions1. What is the diagnosis?2. Describe three features seen on the X-ray of the disease?3. What biochemical test would help clinch the diagnosis?4. What is the treatment of the condition?
    82. 82. Answers 11. Rickets 0.52. a) Cupping 0.5 b) Widening of distal end of metaphysis 0.5 c) Fraying3. Calcium, Phosphorus, Alkaline phosphatase 1.54. Injection Vitamin D 6 lac unit IM stat PO Calcium 1 1
    83. 83. Questions1. What is the diagnosis?2. What is the clinical sign for the diagnosis called as?3. Name one intervention which can lead to this?
    84. 84. Answers 11. Pneumopericardium 12. Hammans sign 13. Invasive ventilation with high pressures
    85. 85. X-ray neck lateral view in a child with respiratory distress
    86. 86. Questions1. What is the diagnosis?2. Which is the commonest organism implicated in this disease?3. What antibiotics are useful in this condition?
    87. 87. Answers1. Epiglottitis 12. Hemophilus influenzae type B 13. Cephalosporins/ Ampicillin/ sulbactam 1
    88. 88. Questions1. Diagnosis2. By what gestational age would this defect occur?3. This can be prevented in subsequent pregnancies by intake of Folic acid. Folic acid should be taken in what dose and started when?
    89. 89. Answers 11. Occipital Encephalocele 12. 26 days post conception3. Folic acid Dose: 0.4mg/day 1 Periconceptional period 1
    90. 90. Questions1. Diagnosis2. What is the clinical picture?3. What is the requirement of echo before surgery?
    91. 91. Answers 1. Tracheo-esophageal fistula 2. Excessive drooling Respiratory distress 3. To rule out associated Congenital heart diseases Right sided aortaPART 3 Selected Thoracic Gastrointestinal AnomaliesMartin: Fanaroff and Martins Neonatal-Perinatal Medicine, 8th ed.,
    92. 92. Pictorials
    93. 93. What is the diagnosis ?Name three complications.What is the period of infectivity.
    94. 94. • Chicken pox 11. Pneumonia 12. Encephalitis3. Ataxia4. ADEM5. Progressive varicella: organ invol, h’ge• Airborne or direct contact : contagious 2 days before & till all lesions are dry 1 Total 3 marks
    95. 95. 1. Give the diagnosis.2. Give four important components.3. State the most important prognostic factors.
    96. 96. 1. Prune- Belly Syndrome 0.52. Important components a. Abnormal abdominal musculature 0.5 b. Abdominal cryptorchidism0.5 c. Renal and ureteric anomalies with VUR 0.5 d. Pulmonary hypoplasia 0.53. Long term complication a. ESRD 0.54. Prognostic factors a. Degree of renal dysplasia 0.5 b. Pulmonary hypoplasia` 0.5 (Total marks 4.0)
    97. 97. 1. Give the diagnosis.2. State mode of inheritance.3. Name five important components.4. Name most frequent immunological defect.
    98. 98. 1. Ataxia – Telengiectasia 0.52. Autosomal recessive 0.53. Important components a. Cerebellar ataxia 0.5 b. Oculo-cutaneous telengiectasia 0.5 c. Immuno-deficiency 0.5 d. Sino-pulmonary infections 0.5 e. Lympho - reticular malignancies 0.54. Selective absence of Ig A 0.5 (Total marks 4.0) (NO MARKS IF FIRST ANSWER IS WRONG)
    99. 99. 1.Give the diagnosis2
    100. 100. 1. Seckel Syndrome 2.02. Important features 2.0 a. Microcephaly b. Beak like nose “Bird face” c. Hypertelorism d. GU anomalies e. Growth retardation (Total marks 4.0) (NO MARKS IF FIRST ANSWER IS WRONG)
    101. 101. 1. Give the diagnosis.2. Give three important complications.3. List three treatment modalities
    102. 102. 1.Giant haemangioma 0.52.DIC 1.5
    103. 103. 1. State the diagnosis2. Name the abnormal karyotypes3. List three prenatal diagnostic techniques4. Indication for parental screening
    104. 104. 1. Down Syndrome 0.52. Trisomy 21 1.5 Translocation Mosaicism3. Triple screen (b-HCG, a fetopr, estradiol)1.5 Ultrasonography Fetal DNA analysis (maternal age >35 yrs)4. Translocation0.5 (Total marks 4) (No marks if first answer is wrong)
    105. 105. What is the condition? 1Describe the lesions. 1What is the mode of inheritance? 1What is CNS association? 1
    106. 106. Tuberous sclerosisAsh leaf macule , shagreen patch, angiofibromaAutosomal dominantCharacteristic brain lesions are tubers located in convolutions of cerebral hemisphere typically in the subependymal region.
    107. 107. What is the condition?How will you treat it?
    108. 108. Bitot’s spotsVit A 6 mo to 1 year 1 lac U oral rpt 48 hrs 1 yr to 6 years 2 2 marks
    109. 109. What is this condition?Name a metabolic complication arising from this condition2 marks
    110. 110. Collodion baby (Ichthyosis)Hypernatremic dehydration 2 marks
    111. 111. What is the abnormality seen?What is the diagnosis?What other investigation would you advise in this case?
    112. 112. Absence of depression of the right angle of mouth while cryingDAOM (deficiency of depressor anguli oris muscle)2D echocardiography as the condition is associated with cardiac anomalies 3 marks
    113. 113. QUESTIONSWhat is the condition?What is the root value of nerves involved?What is the life threatening complication associated 2 marks
    114. 114. Erb- Duchenne palsy  C5,C6 Diaphragmatic paralysis 3 marksChapter 27 – Birth InjuriesMartin: Fanaroff and Martins Neonatal-Perinatal Medicine, 8th ed
    115. 115. Name the condition.What is the natural course of these lesions?
    116. 116. Strawberry angiomasLesions increase over the first few months of life and then regress 2 marks
    117. 117. Describe the abnormality 1What is the likely diagnosis1
    118. 118. Webbed neck In a female  Turner syndrome In a male  Noonan’s Syndrome 2 marks
    119. 119. QuestionsDiagnosisCommonest differentialManagement
    120. 120. Answers Epulis: granular cell tumor of gum Hemangioma Surgical excision  Carbondioxide laser excision 4 marksChapter 26 – Physical Examination of the NewbornMartin: Fanaroff and Martins Neonatal-Perinatal Medicine, 8th ed
    121. 121. QuestionsAbnormalitiesSyndromeInheritance
    122. 122. AnswersProptosis ,high forehead,mid facial hypoplasia.Crouzon’s syndrome.Autosomal Dominant 3 marks
    123. 123. What is the Diagnosis ?What is the Differential Diagnosis?What are the assoc anomalies?What is the Management ?
    124. 124. 1. GASTROCHISIS 1 2. OMPHALOCOELE 1 3. Beckwith Wiedman Trisomies Congenital cardiac anomalies 1 4. Cover defect with sterile draping fluid replacement Early surgical correction 1PART 4 Selected Abdominal Gastrointestinal AnomaliesMartin: Fanaroff and Martins Neonatal-Perinatal Medicine, 8th ed
    125. 125. 1. Give the diagnosis2. Give five important components3. Name an important metabolic abnormality and its management
    126. 126. 1. Hemihypertrophy Syndrome 0.5 2. Important features a. Macrosomia / (Hemi hypertrophy) 0.5 b. Macroglossia 0.5 c. Hepato-splenomegaly 0.5 d. Omphalocele 0.5 e. Embryonal tumours 0.5 3. Metabolic abnormality & management a. Hyperinsulism Hypoglycaemia 0.5 b. Diazoxide / pancreactectomy 0.5 (Total marks 4.0)Chapter 28 – Congenital AnomaliesMartin: Fanaroff and Martins Neonatal-Perinatal Medicine, 8th ed
    127. 127. 1. Give the diagnosis.2. Name the most important endocrinal feature3. Name the most important cardiovascular problem4. Name the mainstay of management
    128. 128. 1. Congenital Lipodystrophy 1.02. Insulin resistant DM 1.03. Hypertrophic cardiomyopathy 1.04. Dietary fat regulation 1.0 (Totalmarks 4) (No marks if first answer is wrong)
    129. 129. 1. Give the diagnosis2. List clinical staging.3. What is the most important sequel and it’s prediction?4. Outline the treatment.
    130. 130. 1. Kawasaki Disease 0.52. Clinical Staging a. Acute febrile phase 0.5 b. Sub-acute phase 0.5 c. Convalescent phase 0.53. Complication and prediction a. Coronary artery aneurism 0.5 b. Onset of coronary artery changes 0.5 within 2 months of onset of illness1. Treatment a. IVIG 0.5 b. High dose aspirin 0.5 (Total marks 4.0) (NO MARKS IF FIRST ANSWER IS WRONG)
    131. 131. 1. What is the diagnosis?2. What is the embryologic basis of these defects?3. In an otherwise healthy child, when are these defects repaired?
    132. 132. Answers1. Bilateral Cleft Lip and Palate 12. Cleft lip - Hypoplasia of mesenchymal layer 1 resulting in failure of medial nasal and maxillary process to join Cleft palate - Failure of palatal shelves to fuse 13. Cleft lip By 3 Months 1 Cleft palate By one year 1
    133. 133. Questions1. What is the likely diagnosis?2. What is the mode of inheritance?3. State True or False a. Limb shortening is greatest in proximal segments. b. Fingers often have a trident configuration. c. Lumbar canal stenosis generally does not develop till early adulthood.
    134. 134. Answers1. Achondroplasia 12. Autosomal dominant 13. True/ false a. True 1 b. True 1 c. True 1
    135. 135. A 3 year girl presents withSevere pruritus espDuring night and afterTaking bath with warmWater. Pruritus is mostSignificant in web spacesOf hands and toesQuestions1. Diagnosis2. What is causative organism?3. What is Topical Rx?4. What is oral Rx?
    136. 136. Answers 11. Scabies 12. Sarcoptes scabie 13. Permethrin 5% 14. Ivermectin
    137. 137.  An infant presents with periorificial and acral dermatitis, diarrhea, alopecia and nail dystrophy.
    138. 138. Questions1. Diagnosis?2. What laboratory test helps to clinch the diagnosis?3. What is the treatment?4. Name any three IEM which can have similar finding.
    139. 139. Answers 11. Acrodermatitis enteropathica 12. Low plasma zinc concentration3. PO Zinc (zinc sulfate, acetate,or gluconate) 50-150mg/day 14. Any three of following Maple syrup urine disease, 3 Organic aciduria, Methylmalonic acidemia, Biotinidase deficiency Essential fatty acid deficiency
    140. 140. Questions1. Name the neurocutaneous marker seen2. In NF-1 what is the diagnostic criteria with respect to this marker?3. Name a X-linked dominant neurocutaneous syndrome4. What are the three stages of syndrome in question 3?
    141. 141. Answers1. Café-au lait macule 12. Six or more CAL macules larger than 5 mm in greatest diameter in prepubertal And larger than 15 mm in greatest diameter in postpubertal individuals 13. Incontinentia pigmenti4. Stage 1-Vesicular stage Stage 2-Verrucous stage Stage 3-Pigmented stage 1 1 1
    142. 142. Questions 1. What is exhibited in the photographs a and b? 2. Diagnosis?a 3. What is the mode of inheritance? 4. What is the major ocular criteria for diagnosis? b
    143. 143. Answers 11. a: Hypermobility of finger joints. 1 b: Positive thumb (Steinberg) sign 12. Marfan syndrome3. Autosomal dominant 14. Ectopia lentis 1 (superior and temporal )
    144. 144. 1. Diagnosis2. What is the pattern of inheritance?3. What is the characteristic radiographic image called?4. What is the opthalmologic complication?
    145. 145. Answers 11. Sturge weber syndrome 12. Sporadic 13. Tram track appearance 14. Bupthalmos
    146. 146. Questions1. What is the diagnosis?2. What are the three species associated with it?3. What are useful methods for diagnosing tinea infections?
    147. 147. Answers 11. Tinea infection 12. Trichophyton 1 Microsporum 1 Epidermophyton3. Potassium hydroxide (KOH) preparations 1 Fungal culture of skin scrapings inoculated on DTM (dermatophyte test media) 1
    148. 148. Questions1. What is the clinical condition called?2. Which is the commonest malignancy associated with this?3. Which chromosome is implicated in malignancy mentioned in question 2?4. What other malignant disorders are associated with the malignancy being discussed?
    149. 149. Answers 11. Leucocoria 12. Retinoblastoma 13. RB1 gene Chr 13q 14 14. Osteosarcoma Soft tissue sarcomas 1 Malignant melanoma 1
    150. 150. Examination Lax skin folds
    151. 151. Diagnosis?
    152. 152. Cutis laxa
    153. 153. LAB & OTHER INV
    154. 154. Lab – 1(Blood) • Age – 4 yrs • Hb – 7 g% • RBCs – Polychromatophilic reticulocytosis, many spherocytes, central pallor less conspicuous • WBC and platelet series- normal • Bone Marrow – Erythroid Hyperplasia • Questions – Differential diagnosis – Confirmatory test
    155. 155. Answers: Lab – 1(Blood)• Anemia• Increased retic count – Hemolysis• PBS – spherocytes, other series normal• Bone marrow – hyperplastic – hemolysis• D/D of hemolytic anemia with spherocytes – Hereditary spherocytosis – osmotic fragility – Autoimmune hemolytic anemia – coombs test – Wilsons disease – serum ceruloplasmin – Burns - History Total 2 marks
    156. 156. Lab – 2 (Blood)• Age – 3yrs• Hb – 6 gm%• TLC – 1500/cmm• Platelets – 20,000/cmm• Bone marrow – Cellularity reduced• Questions – Diagnosis? – Name 2 infectious causes
    157. 157. Answers: Lab – 2 (Blood)All three cell lines are depressedBone marrow depressionDiagnosis- Aplastic anemiaCauses CMV Hepatitis B HIV Total 2 marks
    158. 158. Lab – 3 (Urine) •Age – 9yrs •Appearance - Turbid •Sp Gr – 1019 •Proteins – 4 + •RBCs – 6 to 10 per HPF, occasional RBC casts seen Questions – Diagnosis? – Causes?
    159. 159. Answers: Lab – 3 (Urine)Sp Gr – normalNephrotic range proteinuriaMicroscopic hematuria with RBC casts – glomerular pathologyNephrotic syndrome with hematuria – Nephritic onset Nephrotic syndromeCommon causes – Idiopathic, SLE, Malaria, HSP, Hepatitis B Total 3 marks
    160. 160. Lab – 4 (CSF)Age – 3 days term neonateCSF –  Appearance – clear  Proteins – 10 mg/dl  Glucose – 45 mg/dl ( Blood sugar – 80 mg/dl)  Cells – WBC – 6/cmm pred neutrosQuestions What is your interpretation
    161. 161. Answers: Lab – 4 (CSF)Normal CSF in a neonate WBC 11+/-10 (90th centile-22) polys 2.2+/-3.8 % (90th centile- 6)Protein 84+/-45 mg/dlGlucose 46+/-10 mg/dlInterpretation – normal CSF report Total 3 marks
    162. 162. Lab - 5 Peritoneal fluid • AGE 10 yrs • Peritoneal fluid – Protein 4.5g/dl – LDH 750 IU (90u/L) – WBC 7500/cmm – GLUCOSE 56mg/dl (Blood sugar 80mg/dl) Question – Diagnosis? – Treatment?
    163. 163. Answer : Lab - 5 Peritoneal fluid  Normal value Transudate Exudate Sp Gr <1.016 >1.106 Protein <3gm/dl >3gm/dl WBCs <1000/cmm >1000/cmm RBCs <10,000/cmm Variable Glucose = serum <serum pH 7.4 to 7.5 <7.4  Diagnosis Tuberculosis  Treatment- ATT as per IAP consensus Group 4 2 HRZE / 7 HR Total 2 marks
    164. 164. 1. What is the diagnosis?2. What is the mutation that results in this disease?3. What are the three main categories of crises in patients with sickle cell disease?
    165. 165. Answers 11. Sickle cell disease2. On the beta chain, valine is substituted for 1 glutamic acid at position 63. Aplastic crisis 1 Vaso-occlusive crisis 1 Acute splenic sequestration 1 Nathan and Oskis Hematology of Infancy and Childhood, 6th ed. 2003, pp 802-811
    166. 166. Questions1. Describe the cells seen.2. Name one condition each from following category in which these cells are seen a. Neurological b. Metabolic c. Hepatic d. Endocrinal
    167. 167. Answers 11. Acanthocytosis2. Conditions 1 a. Neurological Neuroacantocytosis 1 b. Metabolic Abetalipoproteinemia c. Hepatic Severe liver dysfunction 1 d. Endocrinal Hypothyroidism 1
    168. 168. What is this modality of investigation?Name three common indications .
    169. 169. Answer: Spots: 1Radionuclide bone scanIndication Metastasis Osteomyelitis Stress fracture Total 2 marks
    170. 170. What is this modality of investigation?Name one common indication .
    171. 171. Answer : Spots: 2Hepatobiliary scintigraphyIndication Biliary atresia Total 2 marks
    172. 172. What is the diagnosis ?What are the cardiac conditions associated ?What are the endocrinal conditions associated ?
    173. 173. Turner’s Syndrome1. Bicuspid aortic valve2. Co of aorta3. AS4. MVP5. TAPVRHypogonadismHypothyroidismType-II DM Total 4 marks
    174. 174. Questions1. What is the diagnosis?2. Give two sexual characters in this condition3. Give one cardiac condition that may be associated with this condition4. What would be the plasma levels of a. FSH b. LH c. Testosterone
    175. 175. Answers 11. Klinefelter Syndrome (47 XXY)2. Any two 2 Lack secondary sexual characteristics Infertility, azoospermia Gynecomastia Testicular dysgenesis3. Any one Mitral valve prolapse (55%) 0.5 Varicose veins (20-40%)4. Plasma levels of a. FSH Increased 0.5 b. LH Increased 0.5 c. Testosterone Decreased 0.5
    176. 176. Questionsa. Diagnosisb. Commonest differential how do you distinguish between two?c. Give 3 Steps of management
    177. 177. AnswersPSVTSinus tachycardia - there is beat to beat variability.Vagal manoeuvres: Carotid Massage Ice packs over face Valsalva manouverAdenosine drug of choice. 4 marks 4 marks
    178. 178. ECG
    179. 179. QuestionsAbnormalitiesManagementWhich drug eliminates the causative agent
    180. 180. AnswersECG changes of hyperkalemiaCalcium gluconate, sodabicarb, asthalin nebulisation, insulinKayexelate resin. 4 marks
    181. 181. What disease does this PBS picture suggest ?
    182. 182. Spherocytosis 1
    183. 183. CASE SCENARIOS
    184. 184. CasepH 7.51 (7.35-7.45) This is the ABG of a 6 weekPO2 12 KPa (95 mmHg) neonate admitted withPCO2 4.7 KPa (35 mmHg) projectile vomitingBlood Urea 11 mmol/lSodium 131 mmol/lPotassium 3 mmol/lChloride 83 mmol/l
    185. 185. Questions1. What type of alkalosis the patient has: metabolic/ respiratory?2. What is the most likely diagnosis?3. What are the ultrasonographic criteria to diagnose the condition?4. What is the treatment of choice?
    186. 186. Answers1. Metabolic alkalosis2. Congenital hypertrophic pyloric stenosis3. Ultrasonographic diagnostic criteria a) Pyloric thickness>4mm or b) Pyloric length > 14mm4. Pyloromyotomy (Ramstedt)
    187. 187. This is blood glucose profile of a 10 year boywith Diabetes. His Hb A1C is 12%. At that timehis treatment included:Insulin Morning EveningRegular 6U 3UNPH 8U 5U 400 Blood glucose profile Blood Glucose (Mg/dl) 350 300 250 200 150 100 50 0 t AM AM AM 00 00 00 gh 00 00 00 12 17 22 12 17 22 ni 00 00 00 id M 6: 6: 6: TIME 2
    188. 188. Questions1. What changes would you institute in insulin dosage?2. Considering his Hb A1c over what period his glycaemic control is likely to be unsatisfactory?3. When do you start screening for diabetic retinopathy ?4. What is the preferred method for screening for diabetic retinopathy?
    189. 189. Answers 11. Increase morning regular insulin dose by 2 Units 12. 3 months3. After 5 years in 1 prepubertal children after 2 years in 1 pubertal children 14. Fundal photography
    190. 190. D/D of polyuria and polydipsia
    191. 191. Screening guidelines
    192. 192. 1. What is the diagnosis?2. What is the preferred treatment and dose?3. Give three alternative drugs for treatment.
    193. 193. Answers 1 1. Ventricular tachycardia 1 2. Cardioversion 0.5 to 1 joule/kg 3. Amiodarone 1 Procainamide 1 Lidocaine 1Park: Pediatric Cardiology for Practitioners, 5th ed
    194. 194. How does SVT in children differ from physiologicsinus tachycardia?SVT typically has the following features: Sudden onset and termination rather than a gradual change in rate Persistent ventricular rate of >180 bpm Fixed or almost fixed RR interval on ECG Abnormal P-wave shape or axis or absent P waves Little change in heart rate with activity, crying, or breath holding
    195. 195. CaseA 5 year girl is brought after accidental ingestion of unquantified insecticide with salivation, lacrimation and miosis
    196. 196. Questions1. Name three muscarinic actions of OP poisoning2. What is the specific antidote?
    197. 197. Answers 31. Increased oral and tracheal secretions, miosis, salivation, lacrimation, urination, vomiting, cramping, defecation, and bradycardia2. Atropine 1
    198. 198. OP poisoning effectsMuscarinic effects: Increased oral and tracheal secretions, miosis, salivation, lacrimation, urination, vomiting, cramping, defecation, and bradycardia; may progress to frank pulmonary edemaCentral nervous system effects: Agitation, delirium, seizures, and/or comaNicotinic effects: Sweating, muscle fasciculation, and, ultimately, paralysis
    199. 199. CaseA 8 year old boy is admitted after consumtion of > 60 tablets of ferrous sulfate kept at home
    200. 200. Questions1. Which stage of iron toxicity is free of any symptoms?2. What is the preferred method of gastrointestinal decontamination in patients with iron overdose?3. What is the antidote with dose?4. What metal intoxication can mimic Kawasaki disease?
    201. 201. Answers 11. Stage 2 (6-24 hours): Iron silently accumulates in the mitochondria during this period, which is relatively free of symptoms.2. Whole-bowel irrigation -Syrup of ipecac is no longer recommended, and activated charcoal will 1 not adsorb iron. Many adult-strength iron-containing pills are very large and are often too large for orogastric lavage3. Deferoxamine 1 Up to 15 mg/kg per hour via continuous IV infusion 14. Mercury 1 Acrodynia
    202. 202. Iron Toxicity StagesStage 1 (0.5-6 hours): During this stage, iron exhibits a direct corrosive effect on the small bowel. Symptoms include nausea, vomiting, abdominal pain, and/or gastrointestinal hemorrhage.Stage 2 (6-24 hours): Iron silently accumulates in the mitochondria during this period, which is relatively free of symptoms.Stage 3 (4-40 hours): This phase is characterized by systemic toxicity with shock, metabolic acidosis, depressed cardiac function, and hepatic necrosis.Stage 4 (2-8 weeks): During this phase, pyloric stenosis and obstruction can develop as a result of earlier local bowel irritation.
    203. 203. Disease Present Absent a b PositiveTest c d Negative •Sensitivity •Positive predictive value • T(+)/ D(+) = a/a+c •D+/T+ = a/a+b •Specificity •Negative predictive value • T(-)/ D (-) = d/b+d • D-/ T- = d/ c+d
    204. 204. CaseAnalyse the table below Test Has disease No disease Positive 90 10 Negative 10 901. Calculate sensitivity2. Calculate specificity
    205. 205. Answer 11. Sensitivity 90% 12. Specificity 90%
    206. 206. Case Urine Pure growth Multiple growth microscopyOn auditing urine growth, following >50 WBC 95 15 were the result <50 WBC 10 2001. Calcute positive predictive value of urine microscopy2. Calculate negative predictive value urine microscopy
    207. 207. Answer 11. PPV 95/110 12. NPV 200/210
    208. 208. Case 8A 13-year old boy develops right upper-quadrant pain and fever with chills and rigors.An abdominal ultrasound reveals Hyperechoic liver parenchyma Dilatation of several intrahepatic bile ducts Few bilateral renal cysts.
    209. 209. Questions1. What is the Diagnosis?2. What is the cause of fever with chills in this case?3. What are the components of this disease/syndrome?4. Which gene has been implicated for the above?
    210. 210. Answers 11. Caroli’s syndrome 12. Cholangitis 13. Intra-hepatic ductal ectasia AR Polycystic kidney Disease 14. PKHD1 gene 1
    211. 211. Case9A 3 year old boy a case of steroid sensitive nephrotic syndrome in remission has been brought with Periorbital puffiness Proteinuria on dipstick evaluation at home
    212. 212. Questions1. What are the components of nephrotic syndrome?2. How do you define proteinuria of nephrotic range on dipstick?3. How do you define relapse?4. What is the treatment of relapse?
    213. 213. Answers1. Nephrotic Syndrome 0.5  Heavy proteinuria 0.5  Hypoalbuminemia 0.5  Edema  Hypercholesterolemia 0.5
    214. 214. Answers 12. Urine protein 3+/4+ (on dipstick)3. Urine albumin 0.5  3+ or 4+ (or proteinuria >40 mg/m2/h) 0.5  for 3 consecutive early morning specimens  having been in remission previously 0.5 0.52. Prednisolone  2 mg/kg/day (single or divided doses) until urine protein is 0.5 trace or nil for 3 consecutive days  Then 1.5 mg/kg on alternate days for 4 weeks  Then discontinued 0.5 0.5
    215. 215. Case10A 12 year old girl a case of chronic kidney disease due to FSGS is evaluated in OPD for hypertension, poor growth and dyslipidemia.
    216. 216. Questions1. How do you determine GFR using serum creatinine with Schwartz formula?2. What is Stage II Chronic Kidney disease (CKD) in terms of GFR?3. State True/ False a. Growth hormone is a mode of treatment for short stature due to CKD. b. Anemia in CKD is caused primarily due to loss of ferritin in urine. c. The most common form of renal osteodystrophy seen with CKD is high turnover bone disease.
    217. 217. Answers1. GFR(ml/min/1.73m2) 1 = k x Height (cm) serum creatinine (mg/dL) where k is  0.33 for low-birthweight infants younger than 1 yr,  0.45 for term AGA infants younger than 1 yr,  0.55 for children and adolescent females  and 0.70 for adolescent males
    218. 218. Answers 12. Stage 2 CKD: GFR 60-89 ml/kg/1.73m23. True/ False 1 a. True Children with CKD who remain <2 SD for height despite optimal medical support may benefir from rHuGH (0.05mg/kg/d) b. False 1 Anemia is primarily the result of inadequate erythropoietin production c. True The most common finding in renal osteodystrophy is osteitis fibrosa cystica 1 (high turnover bone disease secondary to hyperparathyroidism)
    219. 219. Answers2. Stage 2 CKD: GFR 60-89 ml/kg/1.73m2 13. True/ False a. True 1 Children with CKD who remain <2 SD for height despite optimal medical support may benefir from rHuGH (0.05mg/kg/d) b. False Anemia is primarily the result of inadequate erythropoietin production 1 c. True The most common finding in renal osteodystrophy is osteitis fibrosa cystica (high turnover bone disease secondary to hyperparathyroidism) 1
    220. 220. What are the main causes of chronic renaldisease in children that result in renaltransplantation?Obstructive uropathyAplastic/hypoplastic/dysplastic kidneysFocal segmental glomerulosclerosis
    221. 221. Questions1. Define Time constant2. Two conditions with increased time constant3. Two conditions with decreased time constant
    222. 222. Answers 11.Time constant is the product of compliance and resistance(amount of time required for proximal airway pressure to equilibrate with alveolar pressure)2.Asthma and bronchiolitis3.Pneumonia and pulmonary edema 1 1 Nelson18th edition pg 1722
    223. 223. Questions1. Mechanism of grunting2. Significance of grunting3. Helpful in which conditions (name two)
    224. 224. Answers 11. Produced by expiration against a partially closed glottis expiration 12. Attempt to maintain positive airway pressure during expiration for as long as possible.3. Alveolar diseases(pneumonia/pulmonary edema/HMD) and small airway 1 obstruction(bronchiolitis) Nelson18th edition pg 1724
    225. 225. Questions1. Significance of clubbing2. Two thoracic causes of clubbing3. Two extrathoracic causes of clubbing4. Sign associated with clubbing
    226. 226. Answers 11. Chronic hypoxia 12. Small lung cancer and lung abscess 13. Cyanotic heart diseases and IBD4. Schamroth’s sign 1
    227. 227. CaseThe EEG record is from a 5 month infant who episodically raises his arms and then flexes his neck, trunk and hips. The episodes last a few seconds and end with a brief cry and return to normal posture.The episodes occur in quick succession with several hours passing between each cluster of attacks.
    228. 228. EEG
    229. 229. Questions1. What does the EEG show?2. What syndrome is suggested by the history and EEG?3. Give one genodermatoses condition associated with this syndrome.4. What is the long term prognosis?
    230. 230. Answers 11. Hypsarrhythmia  Large amplitude slow wave activity mixed with multifocal spikes and sharp waves
    231. 231. Answers 12. Infantile spasms- West Syndrome 13. Tuberous Sclerosis4. Treatment 1  Unsatisfactory on most occasions  AED`s Vigabatrin Benzodiazepines (Clonazepam/ Nitrazepam)  Steroids/ ACTH
    232. 232. What is the classic triad ofinfantile spasms?Spasms, hypsarhythmia, and developmental regressionInfantile spasms are known as Wests syndrome, and the condition is named for the physician who first described the condition in his own son in 1841.
    233. 233. After what period can AEDs be safely discontinued? When the child is free of seizures for 2 yearsSmith R, Ball R: Discontinuing anticonvulsant medication in children.Arch Dis Child 87:259-260, 2002
    234. 234. Case 15The examination of a newborn reveals a lump of soft tissue of size of 50 paise coin overlying the lower spine. There is no neurological deficit.
    235. 235. Questions?1. What is the likely diagnosis?2. What is likely risk of recurrence of disorder in future pregnancies?3. What intervention started at what time and for how long, can reduce the risk of intervention?4. What are the options for managing bladder incontinence in this condition?
    236. 236. Answers dysraphism1. Occult spinal 12. Risk of recurrence 1 One affected child 3-4% Two affected Children 10%3. Intervention Folic acid 400 mcg/d 1 Started Periconceptionally 0.5 (started before pregnancy) Continued Till 12 wks of preg 0.5
    237. 237. Answers 24. Bladder incontinence management  Clean intermittent catheterization  Artificial urinary sphincter  Surgical urinary diversion  Augmentation cystoplasty
    238. 238. What is the likelihood that a patient withmyelomeningocele will havehydrocephalus?Hydrocephalus is seen in 95% of children with thoracic or high lumbar myelomeningocele.The incidence decreases progressively with more caudal spinal defects to a minimum of 60% if the myelomeningocele is located in the sacrum.
    239. 239. Case 16A 6-month-old child was noted to be normal at birth, but over the ensuing months you have been somewhat concerned about his slowish weight gain and his mild delay in achieving developmental milestones.The family calls you urgently at 7:00 A.M. noting that their child seems unable to move the right side of his body.
    240. 240. Questions?1. Which of the following conditions might explain this child’s condition? a. Phenylketonuria b. Homocystinuria c. Cystathioninuria d. Maple syrup urine disease e. Histidinemia1. Which one other systemic examination would you like to do?2. What investigation would confirm the etiology?3. What treatment would you institute as a long term measure?
    241. 241. Answers 11. Homocystinuria (b) 12. Eye (Ectopia lentis)3. Elevated plasma levels for homocystine 14. Vitamin B6 (200-1,000 mg/24 hr) 1 Folic acid 1-5mg/24 hr Vit B 12 (1-2 g/24hrs)
    242. 242. Match the followingMatch the following sign/symptom with the expected location of the lesion in the brain:
    243. 243. a. Deafferented pupil  Nystagmus.b. Cerebellum  Tonic deviation of the eye(s).c. Ipsilateral cortex  Marcus Gunn pupil.d. Midbrain tectum  Horner’s syndrome.e Pons  Midposition pupilsf. Cribriform plate of ethmoid  Pinpoint pupils  Leakage of CSF from nose.
    244. 244. Answera. Deafferented pupil Marcus Gunn pupilb. Cerebellum Nystagmusc. Ipsilateral cortex Tonic deviation of the eye(s)d. Midbrain tectum Midposition pupilse. Pons Pinpoint pupilsf. Cribriform plate Leakage of CSF from nose 3 marks
    245. 245. Match the following Thrombotic stroke.a. Rete mirabile of Moyamoya.b. Right-to-left cardiac shunt. Embolic stroke.c. Arteriovenous malformations. Intracerebral hemorrhage.
    246. 246. Answera. Rete mirabile of Thrombotic stroke Moyamoya.b. Right-to-left cardiac Embolic stroke shunt.c. Arteriovenous Intracerebral h`age malformations.
    247. 247. Case19A 4-year-old child is observed to hold his eyelids open with his fingers and has drooping of eyes, especially in the evening. He has some trouble swallowing his food. He can throw a ball, and he runs well.He undergoes EMG and is diagnosed asMYASTHENIA GRAVIS
    248. 248. Questions?1. What is the characteristic EMG in Myasthenia?2. What is the chest X-ray finding in this condition?3. What is the clinical test for diagnosing myasthenia?4. Which antibiotics can worsen the condition?5. What drugs are used for treatment?
    249. 249. Answers 11. Decremental response to repetitive stimulation2. Enlarged thymus 13. Edrophonium test 14. Aminoglycosides5. Neostigmine 0.4mg/kg PO 4-6 hrly 1 0.04mg/kg IM 4-6 hrly 0.5 0.5
    250. 250. Fleisher GR, Ludwig S (eds): Textbook of Pediatric Emergency Medicine, 3rd ed.
    251. 251. Case 20Examination of the cerebrospinal fluid of an 8-year- old, mildly febrile child with nuchal rigidity and intermittent stupor for 3 weeks shows the following: White blood cells 100/μL (all lymphocytes) Negative Gram stain Protein 750 mg/dL Glucose 25 mg/dL
    252. 252. Questions?1. What are the likely differential diagnosis?2. What drugs can be used to reduce intracranial tension?3. Which cranial nerve is involved in false localizing sign?
    253. 253. Answers 11. Tubercular/ Fungal meningitis 12. Mannitol, glycerol 13. Sixth cranial Nerve (Abducens)
    254. 254. Case 21At birth, an infant is noted to have an abnormal neurologic examination.Over the next 1-2 weeks he develops severe progressive central nervous system degeneration, an enlarged liver and spleen, macroglossia, coarse facial features, and a cherry-red spot in the eye.
    255. 255. Question1. The laboratory finding likely to explain this child’s problem is a. Reduced serum hexosaminidase A activity b. Deficient activity of acid beta-galactosidase c. A defective gene on the X chromosome d. Complete lack of acid alpha-galactosidase activity e. Deficient activity of galactosyl-3-sulfate-ceramide sulfatase1. What is the diagnosis?
    256. 256. Answers 11. Deficient activity of acid beta-galactosidase (b) 12. Type 1 GM1 gangliosidosis
    257. 257. 22For each description select the most likely diagnosis:
    258. 258. Clinical scenario1. Eye blinking or throat-clearing noises in an otherwise healthy 8-year old Diagnosis boy2. A 6-year-old boy with eye twitching a. Transient tic disorder of and ecolalia childhood3. A 2-year-old infant who was born prematurely and is unable to walk or b. Tourette syndrome talk c. Cerebral palsy4. A 14-year-old girl with a history of precocious puberty who now d. Tuberous sclerosis develops a large goiter e. McCune-Albright syndrome5. An infant with infantile spasms, a hypsarrhythmic EEG pattern, and ash-leaf depigmentation on her back
    259. 259. Answer1. Transient tic disorder of childhood2. Tourette syndrome3. Cerebral palsy4. McCune-Albright syndrome5. Tuberous sclerosis Total 5 marks
    260. 260. Case scenario 2316 year boy followed up in your clinic for several years. His latest lung function tests areFVC 85%FEV1 57%PEF 53%FEV1/FVC 67%
    261. 261. Questions1. Diagnosis2. Which of the above measurement is best for monitoring
    262. 262. Answer1. Asthma2. FEV1/FVC
    263. 263. List the possible acute side effectsof salbutamolGeneral: Hypoxemia, tachyphylaxisRenal: HypokalemiaCardiovascular: Tachycardia, palpitations, premature ventricular contractions, atrial fibrillationNeurologic: Headache, irritability, insomnia, tremor, weaknessGastrointestinal: Nausea, heartburn, vomiting
    264. 264. What proportion of childhood asthmatics "outgrow" their symptoms? 30-50%Sears MR, Greene JM, Willan AR, et al: A longitudinal, population-based,cohort study of childhood asthma followed to adulthood.N Engl J Med 349:1414-1422, 2003.
    265. 265. Question 24Below is diagram of lung volumes,what do the letters A and B represent
    266. 266. AnswerA inspiratory reserve volumeB residual lung volume
    267. 267. Case No 28 7 years male c/o increasing weakness of all limbs x 3 days & URTI one week backExamination CNS Bilateral facial nerve palsies Power Grade 0 to 1 both legs; grade 3 arms Loss of DTJ,No sensory deficit/ papilloedema Abdomen Palpable midline mass arising from pelvis Other systems: Normal
    268. 268. Questions1. What is the likely diagnosis?2. Mention 3 investigations to clinch diagnosis.3. What is the most sensitive measure of respiratory muscle involvement?4. Mention 3 modalities of treatment.5. What is the midline mass, explain its significance and how should it be managed?
    269. 269. Answers1. Guillain-Barre Syndrome (1)  The history points to a symmetrical motor neuropathy without sensory nerve involvement.  Muscle disorders should have normal DTJ  Polio will cause asymmetrical weakness  Spinal cord abnormalities will not cause facial muscle weakness
    270. 270. Answers 2. CSF-protein cellular dissociation NCV EMG (1.5) 3. The most sensitive measure of respiratory muscle involvement is (1) VITAL CAPACITY In children, the normal VC may be calculated as VC = 200 mL × age in years. If the VC falls below 25% of normal, endotracheal intubation is performed. 4. IVIG,Plasmapheresis,steroids (1.5) 5. Bladder distension due to ANS involvement (1)
    271. 271. Case No 30A 2 years boy was unconscious for 1 min following head injury.Although he is fully alert now there is bruising on the left side of the head over the parietal bone.Skull x-rays are performed and he is admitted for neurological observations.
    272. 272. Questions1. What clinical features would suggest requirement of CT skull and/ or neurosurgical opinion?2. What non-surgical temporary measures can be used to reduce raised intracranial pressure?3. What advice needs to be given to parents if after 24 h the child has remained well and is ready for discharge?
    273. 273. Answers1. Neurosurgical Opinion/ CT skull a. Deteriorating level of consciousness b. Focal neurological signs c. Depressed Skull fracture d. Basal Skull fracture e. Seizures f. CSF leak g. Signs of Raised ICT (2)
    274. 274. Answers2. Temporary manoeuvres to reduce raised ICT  Nursing in a 30 degrees head up position  Diuretics (Mannitol)  Hypertonic saline  Artificial hyperventilation (2)
    275. 275. Answers3. At discharge it should be stressed that child should be brought back if he develops any of following:  Vomiting  Drowsy/ altered sensorium  Blurred/ double vision  Seizures (2)
    276. 276. CASE SCENARIO 7 year old girl presents with high irregular fever for 3 weeks, each episode associated with erythematous rashes over the trunk and proximal extremities, pain and swelling of Rt knee and easy fatigability. Has leucocytosis, raised ESR, normal chest skiagram and negative Mx.1. Give most probable diagnosis.2. List other expected systemic clinical findings3. Give value of serology in diagnosis4. Outline steps in management5. List prognostic factors
    277. 277. 1. JRA – Systemic 0.52. Systemic exam findings a. Hepato – splenomegaly 0.5 b. Lymphadenopathy 0.5 c. Serous cavity effusions 0.53. Serology a. ANA - Negative 0.5 b. RF - Negative 0.54. Management a. “Pyramid” approach – NSAIDS, chloroquine, 1.0 methotrexate,azothiaprine/cyclophosphamide. b. Steroids 0.5 c. TNF-alpha receptor antagonists 0.55. Number and severity of joint involvement 1.0 (Total Marks 6.0)
    278. 278. This patient becomes ill with thrombocytopenia,profound anemia, and markedly elevated transaminasesprobably has what complication?Macrophage activation syndrome
    279. 279. CASE Scenario 12 yr old boy, case of Haemophilia A, is admitted with spontaneous haemarthrosis. 1. What is the level of factor VIII ? 2. Factor VIII in treatment. a. Level of factor VIII to be achieved b. Dose of factor VIII 3. The patient has to undergo major surgery a. Level of factor VIII to be achieved b. Dose of factor VIII 4. What are the other precautions? 5. What is the role of DDAVP in haemophilia A ?
    280. 280. 1. <1% 0.52. Factor VIII in treatment a. 35-40% 1.0 b. 20 units/kg, repeat daily if required 1.0 till joint normalises3. Dose in major surgery a. 100% 1.0 b. 50 units/kg, infuse 2-3 units/kg/hr to 1.0 maintain level at 100%for 24 hrs and then >50% for 7 days4. Precautions a. Avoid Anti-inflammatory drugs with anti-platelet 0.5 action b. Screen for Transfusion transmitted diseases if 0.5 plasma products are used4. Desmopressin Acetate: Release of factor VIII in mild cases 0.5 (Total marks 6.0)
    281. 281. CASE Scenario A 7 yr old girl with strong family history of chronic fatal liver disease presents with features of haemolysis. 1. List one clinical examination which may give the diagnosis 2.State most probable group of aetiology based on answer to question ‘1’ 3.List three most important tests for diagnosis with values 4.Outline the management of this child
    282. 282. 1. Kayser-Fleischer rings 0.52. Wilson disease 0.53. Diagnostic tests a.Serum ceruloplasmin 0.5 < 200 mg/lit 0.5 b. Urinary copper 0.5 >100 microgram/day 0.5 c. Hepatic copper 0.5 >250 microgram/gm of dry weight 0.51. Management a. Restrict copper intake 0.5 b. Chelation with penicillamine 0.5 c. B6 supplementation 0.5 d. Liver transplant 0.5 (Total marks 6.0)
    283. 283. Drugs & Devices
    284. 284. Identify the instrument.Which illness is it used in ?What are the three settings ?
    285. 285. Peak Flow MeterBronchial Asthma1. Green zone >80% Peak Exp Flow2. Yellow Zone 50-80 % PEF3. Red zone < 50% PEF Total 3 marks
    286. 286. Which class does this antibiotic belong to ?What is the spectrum?What is the dosage?What is the imp clinical use?4 marks
    287. 287. OxazolidinonesGram-positive organisms including MRSAPenicillin-resistant pneumococciGram-negative anaerobesMycobacteria10mg/kg/dose 8 hrlyMultiresistant gram-positive organisms, including MRSA who have failed to respond to vancomycin.
    288. 288. Indications for use of this compound?what is the dose?What are the indicationsWhen should it be started?What are the side effects of parenteral nutrition?4 marks
    289. 289. Parental nutritionDose 0.5 -3.5 g/kg/dayWhen enteral nutrition is insufficient or contra- indicated.Day 1Dyselectronemia, cholestasis, bacterial or fungal colonisation and sepsis, thrombosis, azotemia, thrombocytopenia
    290. 290. Identify the product?What are the advantages of its use?Enumerate five complications?
    291. 291. PICC 1Preserves veins, allows concentrated infusates, permits long term IV alimentation 16 complications- 3Catheter fracture and embolisation,Leakage Thrombosis of catheterAccidental displacement / Tip MigrationDeep Venous ThrombosisInfection
    292. 292. What is the equipment ?Identify the parts.Enumerate indications for its use.What are the contra- indications?How do you sterilise it?5 marks
    293. 293. Self inflating 250 ml ambu bag 1Oxygen , air inlet , pt outlet, valve assembly, oxygen reservoir and pressure release valve. 1Gasping/apneic infant, cyanosis inspite of free flow oxygen and HR<100/m 30secs after initial steps. 1MSAF and suspected cong diaph hernia 1 Wash with detergent and hot water. Dry thoroughly. If visibly soiled with blood/body fluids, clean and send to Central Sterilization Dept 1
    294. 294. Identify the product?What is the dosage?What are the indications for use?Enumerate three precautions ?
    295. 295.  Surfactant 0.5 4ml/kg 0.5 Surfactant replacement therapy for prevention and treatment of RDS in preterms, 0.5 Survanta should not be removed from the refrigerator for more than 24 hours and it should not be warmed and returned to the refrigerator more than once. Do not suction the infant for 1 hour after dosing unless signs of significant airway obstruction occur. Marked improvements in oxygenation and decrease in lung compliance may occur within minutes of administration of SURVANTA. Therefore, frequent and careful clinical observation and monitoring. Donot shake the vial 1.5
    296. 296. What is the equipment?What are the indications of its use?What are the advantages?
    297. 297. Double lumen endotracheal tube 1For surfactant instillation 1 To aid lung lavagePermits efficient drug instillation without interrupting ventilation 1
    298. 298. Identify the equipmentIndications for usageComplications (mention any three)
    299. 299.  Bubble CPAP apparatus 1 Indications - 2 • If FiO2 requirement is > 0.3 • SA Score > 4 • ABG Score > 3 on oxygen therapy • Frequent apnea with documented hypoxemia • Clinically significant chest retractions after extubation from mechanical ventilation• Complications 1 • Air leak • Reduced venous return • IVH
    300. 300. Which class does this antibiotic belong to ?What is the spectrum?What is the dosage?What is the two imp side effects?
    301. 301. CarbapenemsWidest spectrum of antimicrobial activity among the β-lactam antibiotics against both gm-ve and gm + organisms except MRSA.20 - 40mg/kg/dose 12hrlyTransient agranulocytosis, thrombocytopenia.Seizures 2 marks
    302. 302. ABG1pH 7.28PaCO254mmHgPaO2 45mmHgHCO3 29mEq/LBase excess +7
    303. 303. What are the-Abnormalities? 1Diagnosis? 1Likely causes? 1
    304. 304. Low pH, high PaCO2, low PaO2, high HCO3Uncompensated respiratory acidosis with hypoxemiaRESPIRATORY FAILURE (pneumonia, RDS)
    305. 305. ABG 2pH 7.57PaCO2 22mmHgPaO2 156mmHgHCO3 18mEq/LBase excess -8
    306. 306. What are the-Abnormalities? 1Diagnosis? 1Likely cause? 1
    307. 307. High pH, low PaCO2, high PaO2, low HCO3UNCOMPENSATED RESPIRATORY ALKALOSIS WITH HYPEROXIAHyperventilation with high FiO2
    308. 308. ABG 3 pH 7.32 PaCO2 30mmHg PaO2 70mmHg HCO3 12mEq/L Base excess -8
    311. 311. ABG 4 pH 7.60 PaCO2 21mmHg PaO2 65mmHg HCO3 24mEq/L Base excess +2
    314. 314. ABG 5 pH 7.36 PaCO2 70mmHg PaO2 75mmHg HCO3 35mEq/L Base excess +14
    317. 317. ABG 6 pH 7.52 PaCO2 47mmHg PaO2 80mmHg HCO3 30mEq/L Base excess +3
    318. 318. QUESTIONS
    320. 320. ABG 7 pH 7.14 PaCO2 54mmHg PaO2 55mmHg HCO3 14 mEq/L Base excess -7
    323. 323. Describe the lesion.What is the DD ?
    324. 324. Brain Stem cell glioma, 1 mark
    325. 325. Observed Station 1DRUG COUNSELLING4 yr old boy weighing 15 Kg , diagnosed as Nephrotic Syndrome to be started on steroids.
    326. 326. 1. Introduces self/ puts the child and attendant at ease. 0.52. Explains the disease in simple words 0.53. Explains the medication  Dose 0.5  Frequency 0.5  Relationship with meals 0.54. Explains side effects  GIT 0.5  Steroid effect 0.55. Explains monitoring of response  Urinary output 0.5  Body weight 0.56. Asks for queries if any. 0.57. Advises to report back if any problems. 0.58. Advises about pain abdomen 0.5 (Total marks 6.0)
    327. 327. Observed Station 2DRUG COUNSELINGA 8yr old boy, weighing 30 kg, diagnosed as DM Type I requires 30 units insulin/day, in two divided doses ,combining short and intermediate acting insulins. Counsel regarding administration.
    328. 328. 1.Introduces self & puts child and 0.5 attendant at ease. 2. Explains the problem in brief. 0.5 3. Familiarizes the attendant with the injectables 0.5 and the syringes. 4. Explains dose, time of injection and relationship 2.0 with meals, sites, route and rotation of sites 5. Explains the calculation of dose in ml. 1.0 6. Explains the sequence of loading (regular first). 0.5 7. Briefs about hypoglycemia incl mgt. 0.5 8. Inquires about any doubts and advises to report 0.5 back in case of any problems. (Dose – 2/3rd before breakfast,1/3rd before dinner) (Ratio intermediate /regular 2:1/3:1) (Total marks 6.0)
    329. 329. Observed Station 3COUNSELING24 yr old lady diagnosed as HIV + at 36 weeks of gestation. Counsel regarding perinatal transmission and follow up.
    330. 330. 1. Ensures the presence of husband 0.52. Introduces self/ puts the couple at ease. 0.53. Explains the disease in simple words 0.54. Explains the incidence and modes of perinatal transmission 20-30% 0.5 Prenatal 0.5 Intranatal 0.5 Breast feeding 0.55. Explains modalities of reducing rate of transmission ART to mother and child 1.0 LSCS Vs Vaginal delivery 1.0 Breast feeding Vs top feeding 1.06. Explains effect of measures – reduction by 50% 1.07. Explains screening of the infant 0.58. Explains safety of cuddling, petting and kissing 1.09. Asks for queries if any. 0.510. Advises to report back if any problems. 0.5 (Total marks 10.0)
    331. 331. Observed Station 4Drug Counseling4 yr old child (15Kg) ,a case of gen tonic-clonic seizures has been advised syr carbamezapine (100 mg/5ml).Counsel the mother regarding administration.