COMMAND HOSPITAL PUNE25TH July 2008

INTRODUCTION to Objective structured clinical examination

Observation Stations & Clinical Examination

5 min each

REST STATIONOBSERVED STATION

OBSERVED STATION

OBSERVED STATION

OBSERVED STATION

OBSERVED STATION

REST STATION

REST STATION

STATION 1

Blank OSCE SheetsClinical PhotoX RayClinical ScenarioECGABGKaryotypeBiostatistics

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 STATION 26. 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  

X-Rays/CT/MRI

If patient presents with fever and toxaemia, give three differential diagnosis

Give three modalities of management

1. Lung Abcess2. Infected Bronchogenic cyst 3. Infected Hydatid Cyst

1. Antibiotics (anaerobic +aerobic) 2. Chest physiotherapy 3. Percutaneous CT guided aspiration

0.5 each

Total 3 marks

What is the lesion ?Give two management modalities.What complication can occur during

management & how can it be prevented?

NCC 1NCC:Anticonvulsants 1Albendazole

Increase in Cerebral Edema 1Use steroid cover

Total 3 marks

Describe the lesion.What are the management modalities of such

lesions especially if they are leading to complications ?

Haemangioma scalp 1

Conservative 2Oral steroidsLocal steroidsPDL: pulse dyed laserIFN alfa interferon (in case of haemangiomas

causing pressure effects or erosion leading to significant morbidity)

Total 3 marks

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 ?

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

What is the diagnosis ?What is the likely organism ?

Pnematoceles 1

Likely organism Staphylococcus1

Total 2 mark

Give five radiological findings ?What is the diagnosis ?What is the management ?

1 Ground glass appearance of bone2

2 Thinned cortex3 Periosteal calcification4 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

What was the lesion ?What was the procedure carried out ?What are the complications ?

VSD 1Device implant 1

Device displacement 2Emboli formationHaemolytic Anaemia

Total 3 marks

Name the Investigation ?What is the diagnosis ?What are the further investigations ?

MCU

Posterior urethral Valves

DTPA (diethylene tetra amine pentoic acid) Function

DMSA (dimercapto succinic acid) -scarring

Total 3 marks

What is the abnormality? What is the diagnosis ?How is it suspected clinically ?What is the management ?

1. .Bowel loops in lt hemithorax, mediastinal shift to right 1

2. Congenital diaphragmatic hernia 1

3. Respiratory distress Mediastinal shift Bowel sounds in the thorax Scaphoid abdomen

2

4. Avoid B&M vent prop up and decompress stomach Ventilate Treat PPHN Surgical correction Antenatal tracheal ligation

3

What is the abnormality?

What is the likely diagnosis?

Delineate management.

Mention 3 complications

Multiple fluid levels. gasless lower abdomen 1Small bowel obstruction 1Surgical correction 1Dyselectrolytemia 1 Perforation Exaggerated hyperbilirubinemia Dysmotility syndrome

Martin: Fanaroff and Martin's Neonatal-Perinatal Medicine, 8th ed.,

Describe the X –rayDiagnosisGive three recent advances in management

Hyper inflated lung fields with areas of atelectasis interspersed with areas of overinflation

1MAS 1Lung lavage surfactant instillation HFOV NO liquid ventilation 2

What is the abnormalityThree high risk situations when this condition

is imminentManagement

Pneumothorax 1CDH Ventilation Post surfactant ventilation 2Chest tube drainage 1

What is the abnormalityWhat is it a complication of

Pneumomediastinum 1Forceful ventilation 1

Identify the abnormalityClinical presentationTreatment

Pneumopericardium 1Shock with weak pulses 1Drainage 1

What is the diagnosis ?

1 mark

What are the MRI findings ?What is the DDWhat further inv will you do on the CSF to

confirm the diagnosis ?

3 marks

Acute disseminated encephalomyelitis

Oligoclonal bands are also found in: Multiple sclerosis Devic's disease Systemic lupus erythematosus Neurosarcoidosis SSPE Subarachnoid haemorrhage Syphilis CNS Lymphoma

What are the MRI findings ?What could be the etiology ?

Multifocal cerebral & cerebellar encephalomalacia

Sequel of ischaemic injury in the perinatal period.

2 marks

What is the likely diagnosis ?

What is the IP?Which is the commonest

site?

TB Spine 1

2 YRS 1Thoraclumbar area of max stress valveless venous

drainage 2

4 mark

What are the MRI findings ?

Likely etiologyLikely CSF picture

3 mark

Increased proteins Increased cells likely lymphocytes Low sugar

TBM

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?

Lobar PneumoniaPnemococous/StaphylococcusSynpneumonic effusion, empyema, Penicillin for susceptible org and cefotaxime /

vancomycin for penicillin resistant org10-14 d

5 marks

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?

AnswersEsophagus

When foreign bodies lodge in the esophagus, the flat surface of the object is seen in the AP view *

T 4Anatomic areas of esophageal narrowing

CricoidTracheal bifurcationGastro-esophageal junction

Endoscopy

*

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Question1. What is the diagnosis?2. Fill in the blanks

In an exudative pleural fluida. Proteins >b. Pleural Fluid LDH > ………….c. Fluid to serum LDH ratio > ………..d. Cell count ………

3. What does VATS stand for?

Answers1. Pleural Effusion (Right)2. Fill in the blanks

In an exudative pleural fluida. Proteins > 3.0 g/dLb. Pleural Fluid LDH > 200 IU/Lc. Fluid to serum LDH ratio > 0.6d. Cell count > 1000

3. Video Assisted Thoracoscopic Surgery

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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 Heartb. Snowman silhouette c. Rib notching

Answers1. 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.

1.5

Pediatric cardiologyMyung K Park 5th ed

Answers2. Knee Chest Position

Morphine3. Hyperoxia Test4. X-ray appearances

a. Egg Shaped Heart Transposition of great arteries

b. Snowman silhouette Total anomalous pulmonary venous return

(supracardiac) c. Rib notching Co-arctation of aorta (long

standing)

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Park: Pediatric Cardiology for Practitioners, 5th ed.

3 day neonate withLethargyFeed refusalAbdominal

distension

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?

Answers1. NEC Stage II2. Pneumoperitoneum3. Any two of following

Hirschsprung's disease, Pseudomembranous enterocolitis, Neonatal ulcerative colitis,Ischemic bowel disease

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Martin: Fanaroff and Martin's Neonatal-Perinatal Medicine, 8th ed.,

PART 5 Neonatal Necrotizing Enterocolitis

6 weeks infantCase of Cholestatic

jaundice (Extra-Hepatic Biliary Atresia)

c/o swelling left thigh

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?

Answers1. Metabolic Bone disease (secondary to

Vitamin D deficiency due to malabsorption of fat soluble vitamins)

2. Replace 5,000-8,000 U /d of D2,or3 -5 µg/kq/d of 25-hydroxycholecalciferol

3. Ursodeoxycholic acid 15-20 mg/kg/day4. Alagille syndrome

(Arteriohepatic dysplasia)

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Previously healthy 7 years girl c/o

Sudden onset weakness right upper and lower limb

Facial palsy right (UMN)Normal sensoriumNo fever/ trauma/

seizures

L

cc

tt

Questions1. What is the level of lesion on MRI?2. What are the structures marked

ct

3. Which hemoglobinopathy can be associated with this kind of presentation?

4. A dilated and unreactive pupil indicates the compression of what structure?

Answers1. Infarct in the left basal ganglia, the

posterior limb of internal capsule, and the head of the caudate

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Answers2. C Caudate

nucleusT ThalamusP PutamenG Globus pallidus

White arrows indicate the ant and post limbs of internal capsule

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Answers3. Sickle cell anemia4. Compression of 3rd cranial nerve

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Questions?1. Diagnosis?2. What is the treatment (mention complete

schedule)?

Answers1. Miliary tuberculosis2. 2HRZE + 7HR

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1IAP Group 4

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?

Answers1. Congenital Diagphragmatic Hernia2. The posterolateral portion of the diaphragm has

remained 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 viscera b) Pulmonary hypoplasia from compression of the developing lungs in utero c) Pulmonary hypertension

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QuestionsDescribe the lesion?Give two D/DWhat is the triad of tumor lysis syndrome?

AnswersOsteolytic lesion of skullHistiocytosis

Ewing`s SarcomaLymphomaBone cyst

Hyperuricemia, hyperkalemia, and hyperphosphatemia

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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?

Answers1. Sunray appearance2. Medullary widening3. Chronic hemolytic anaemia4. Hereditary spherocytosis5. Corrected retic count

= reticulocyte % × (patient Hct/normal Hct)

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Below is a midline sagittal cut of a MRI scan of the brain. View the midline anatomic diagram of the brain and identify the following structures

AnswerS - Suprasellar cisternP0 - PonsP - Midbrain (cerebral peduncles)M - MedullaC - Quadrigeminal plate (superior and

inferior colliculi)Q - Quadrigeminal cisternV - Fourth ventricle

1 mark each

Question1. Diagnosis?2. What is the emergency management of the

condition?3. What is subsequent management after the

emergency management is over?

Answers1. Pneumothorax,

with mediastinal shift2. Put in a needle in second intercostal space3. Intercostal drain

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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?

Answers1. Rickets2. a) Cupping

b) Widening of distal end of metaphysisc) Fraying

3. Calcium, Phosphorus, Alkaline phosphatase4. Injection Vitamin D 6 lac unit IM stat

PO Calcium

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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?

Answers1. Pneumopericardium2. Hammans sign3. Invasive ventilation with high pressures

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X-ray neck lateral view in a child with respiratory distress

Questions1. What is the diagnosis?2. Which is the commonest organism

implicated in this disease?3. What antibiotics are useful in this

condition?

Answers1. Epiglottitis

12. Hemophilus influenzae type B

13. Cephalosporins/ Ampicillin/ sulbactam

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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?

Answers1. Occipital Encephalocele2. 26 days post conception3. Folic acid

Dose: 0.4mg/day Periconceptional period

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Questions1. Diagnosis2. What is the clinical

picture?3. What is the

requirement of echo before surgery?

Answers1. Tracheo-esophageal fistula2. Excessive drooling

Respiratory distress3. To rule out associated

Congenital heart diseasesRight sided aorta

PART 3 Selected Thoracic Gastrointestinal Anomalies

Martin: Fanaroff and Martin's Neonatal-Perinatal Medicine, 8th ed.,

Pictorials

What is the diagnosis ?Name three complications.What is the period of infectivity.

• 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

1. Give the diagnosis.

2. Give four important components.

3. State the most important prognostic factors.

1. Prune- Belly Syndrome 0.52. Important components

a. Abnormal abdominal musculature 0.5b. Abdominal cryptorchidism 0.5c. Renal and ureteric anomalies with VUR

0.5d. Pulmonary hypoplasia 0.5

3. Long term complicationa. ESRD 0.5

4. Prognostic factorsa. Degree of renal dysplasia 0.5b. Pulmonary hypoplasia` 0.5

(Total marks 4.0)

1. Give the diagnosis.

2. State mode of inheritance.

3. Name five important components.

4. Name most frequent immunological defect.

1. Ataxia – Telengiectasia 0.5

2. Autosomal recessive 0.5

3. Important componentsa. Cerebellar ataxia 0.5b. Oculo-cutaneous telengiectasia 0.5c. Immuno-deficiency 0.5d. Sino-pulmonary infections 0.5e. Lympho - reticular malignancies 0.5

4. Selective absence of Ig A0.5

(Total marks 4.0)(NO MARKS IF FIRST ANSWER IS WRONG)

1. Give the diagnosis

2. Give four important components

1. Seckel Syndrome2.0

2. Important features2.0

a. Microcephalyb. Beak like nose “Bird face”c. Hypertelorismd. GU anomaliese. Growth retardation

(Totalmarks 4.0) (NO MARKS IF FIRST ANSWER IS WRONG)

1. Give the diagnosis.

2. Give three important complications.

3. List three treatment modalities

1. Giant haemangioma 0.5

2. DIC 1.5HaemorrhageUlcerationInfection

3. Watchful waiting 1.5SteroidsInterferonsExision

(Total marks 4)(no marks if first answer is wrong)

1. State the diagnosis2. Name the abnormal karyotypes3. List three prenatal diagnostic

techniques4. Indication for parental screening

1. Down Syndrome 0.52. Trisomy 21 1.5

Translocation Mosaicism

3. Triple screen (b-HCG, a fetopr, estradiol)1.5UltrasonographyFetal DNA analysis

(maternal age >35 yrs)4. Translocation 0.5

(Total marks 4)

(No marks if first answer is wrong)

What is the condition? 1Describe the lesions. 1What is the mode of inheritance? 1What is CNS association? 1

Tuberous sclerosisAsh leaf macule , shagreen patch,

angiofibromaAutosomal dominantCharacteristic brain lesions are tubers

located in convolutions of cerebral hemisphere typically in the subependymal region.

What is the condition?

How will you treat it?

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

What is this condition?Name a metabolic complication

arising from this condition 2 marks

Collodion baby (Ichthyosis)

Hypernatremic dehydration

2 marks

What is the abnormality seen?

What is the diagnosis?

What other investigation would you advise in this case?

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

QUESTIONS

What is the condition? What is the root value of nerves involved? What is the life threatening complication

associated2 marks

Erb- Duchenne palsy

C5,C6Diaphragmatic paralysis

3 marksChapter 27 – Birth Injuries

Martin: Fanaroff and Martin's Neonatal-Perinatal Medicine, 8th ed

Name the condition.

What is the natural course of these lesions?

Strawberry angiomas

Lesions increase over the first few months of life and then regress

2 marks

Describe the abnormality 1

What is the likely diagnosis1

Webbed neck

In a female Turner syndrome

In a male Noonan’s Syndrome

2 marks

Questions

Diagnosis Commonest differential Management

AnswersEpulis: granular cell tumor of gumHemangioma Surgical excision Carbondioxide laser excision

4 marks

Martin: Fanaroff and Martin's Neonatal-Perinatal Medicine, 8th ed

Chapter 26 – Physical Examination of the Newborn

Questions

Abnormalities Syndrome Inheritance

Answers Proptosis ,high forehead,mid facial

hypoplasia.Crouzon’s syndrome.Autosomal Dominant

3 marks

What is the Diagnosis ?What is the Differential Diagnosis?What are the assoc anomalies?What is the Management ?

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 1

Martin: Fanaroff and Martin's Neonatal-Perinatal Medicine, 8th ed

PART 4 Selected Abdominal Gastrointestinal Anomalies

1. Give the diagnosis

2. Give five important components

3. Name an important metabolic abnormality and its management

1. Hemihypertrophy Syndrome0.5

2. Important featuresa. Macrosomia / (Hemi hypertrophy) 0.5

b. Macroglossia 0.5c. Hepato-splenomegaly

0.5d. Omphalocele 0.5e. Embryonal tumours 0.5

3. Metabolic abnormality & management a. Hyperinsulism Hypoglycaemia 0.5b. Diazoxide / pancreactectomy

0.5(Total

marks 4.0) Chapter 28 – Congenital Anomalies

Martin: Fanaroff and Martin's Neonatal-Perinatal Medicine, 8th ed

1. Give the diagnosis.2. Name the most important endocrinal feature3. Name the most important cardiovascular problem4. Name the mainstay of management

1. Congenital Lipodystrophy1.0

2. Insulin resistant DM 1.03. Hypertrophic cardiomyopathy 1.04. Dietary fat regulation 1.0

(Totalmarks 4)(No marks if first answer is wrong)

1. Give the diagnosis2. List clinical staging.3. What is the most important sequel and it’s

prediction?4. Outline the treatment.

1. Kawasaki Disease 0.5

2. Clinical Staginga. Acute febrile phase 0.5b. Sub-acute phase 0.5c. Convalescent phase 0.5

3. Complication and predictiona. Coronary artery aneurism 0.5b. Onset of coronary artery changes 0.5

within 2 months of onset of illness

4. Treatmenta. IVIG 0.5b. High dose aspirin 0.5

(Total marks 4.0)(NO MARKS IF FIRST ANSWER IS WRONG)

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?

Answers1. Bilateral Cleft Lip and Palate 12. Cleft lip

- Hypoplasia of mesenchymal layer 1 resulting in failure of medial nasal and maxillary process to joinCleft palate- Failure of palatal shelves to fuse 1

3. Cleft lip By 3 Months 1 Cleft palate By one year 1

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.

Answers1. Achondroplasia 12. Autosomal dominant

13. True/ false

a. True 1

b. True 1

c. True 1

A 3 year girl presents with Severe pruritus espDuring night and afterTaking bath with warmWater. Pruritus is most Significant in web spacesOf hands and toes

Questions1. Diagnosis2. What is causative

organism?3. What is Topical Rx?4. What is oral Rx?

Answers1. Scabies2. Sarcoptes scabie3. Permethrin 5%4. Ivermectin

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An infant presents with periorificial and acral dermatitis, diarrhea, alopecia and nail dystrophy.

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.

Answers1. Acrodermatitis enteropathica2. Low plasma zinc concentration3. PO Zinc (zinc sulfate, acetate,or gluconate) 50-

150mg/day4. Any three of following

Maple syrup urine disease, Organic aciduria, Methylmalonic acidemia, Biotinidase deficiency Essential fatty acid deficiency

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Questions1. Name the neurocutaneous marker seen

2. In NF-1 what is the diagnostic criteria with respect to this marker?

3. Name a X-linked dominant neurocutaneous syndrome

4. What are the three stages of syndrome in question 3?

Answers1. Café-au lait macule2. 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

3. Incontinentia pigmenti 4. Stage 1-Vesicular stage

Stage 2-Verrucous stage Stage 3-Pigmented stage

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Questions1. What is exhibited in

the photographs a and b?

2. Diagnosis?3. What is the mode of

inheritance?4. What is the major

ocular criteria for diagnosis?

a

b

Answers1. a: Hypermobility of finger joints.

b: Positive thumb (Steinberg) sign2. Marfan syndrome 3. Autosomal dominant4. Ectopia lentis

(superior and temporal )

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1. Diagnosis2. What is the pattern of inheritance?3. What is the characteristic radiographic image called?4. What is the opthalmologic complication?

Answers1. Sturge weber syndrome2. Sporadic3. Tram track appearance4. Bupthalmos

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Questions1. What is the

diagnosis?2. What are the three

species associated with it?

3. What are useful methods for diagnosing tinea infections?

Answers1. Tinea infection2. Trichophyton

MicrosporumEpidermophyton

3. Potassium hydroxide (KOH) preparations Fungal culture of skin scrapings inoculated on DTM (dermatophyte test media)

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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?

Answers1. Leucocoria2. Retinoblastoma 3. RB1 gene Chr 13q 144. Osteosarcoma

Soft tissue sarcomas Malignant melanoma

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Examination

Lax skin folds

Diagnosis?

Cutis laxa

LAB & OTHER INV

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

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

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

Answers: Lab – 2 (Blood)All three cell lines are depressedBone marrow depressionDiagnosis- Aplastic anemiaCauses

CMVHepatitis BHIV

Total 2 marks

Lab – 3 (Urine)• Age – 9yrs• Appearance - Turbid• Sp Gr – 1019• Proteins – 4 +• RBCs – 6 to 10 per HPF, occasional RBC casts

seenQuestions

– Diagnosis?– Causes?

Answers: Lab – 3 (Urine)Sp Gr – normalNephrotic range proteinuriaMicroscopic hematuria with RBC casts –

glomerular pathologyNephrotic syndrome with hematuria –

Nephritic onset Nephrotic syndromeCommon causes – Idiopathic, SLE, Malaria,

HSP, Hepatitis B

Total 3 marks

Lab – 4 (CSF)Age – 3 days term neonateCSF –

Appearance – clear Proteins – 10 mg/dl Glucose – 45 mg/dl ( Blood sugar – 80 mg/dl) Cells – WBC – 6/cmm pred neutros

QuestionsWhat is your interpretation

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/dlGlucose 46+/-10 mg/dl

Interpretation – normal CSF report

Total 3 marks

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?

Answer : Lab - 5 Peritoneal fluidNormal value

Transudate ExudateSp Gr <1.016 >1.106Protein <3gm/dl >3gm/dlWBCs <1000/cmm >1000/cmmRBCs <10,000/cmm VariableGlucose = serum <serumpH 7.4 to 7.5 <7.4

DiagnosisTuberculosis

Treatment- ATT as per IAP consensus Group 42 HRZE / 7 HR

Total 2 marks

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?

Answers1. Sickle cell disease2. On the beta chain, valine is substituted for

glutamic acid at position 6 3. Aplastic crisis

Vaso-occlusive crisis Acute splenic sequestration

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Nathan and Oski's Hematology of Infancy and Childhood, 6th ed. 2003, pp 802-811

Questions1. Describe the cells seen.2. Name one condition each

from following category in which these cells are seen

a. Neurologicalb. Metabolic c. Hepaticd. Endocrinal

Answers1. Acanthocytosis2. Conditions

a. Neurological Neuroacantocytosisb. Metabolic Abetalipoproteinemiac. Hepatic Severe liver dysfunction d. Endocrinal Hypothyroidism

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What is this modality of investigation?Name three common indications .

Answer: Spots: 1 Radionuclide bone scanIndication

Metastasis OsteomyelitisStress fracture

Total 2 marks

What is this modality of investigation?Name one common indication .

Answer : Spots: 2Hepatobiliary scintigraphyIndication

Biliary atresia

Total 2 marks

What is the diagnosis ?What are the cardiac conditions associated ?What are the endocrinal conditions associated ?

Turner’s Syndrome

1.Bicuspid aortic valve2.Co of aorta3.AS4.MVP5.TAPVR

HypogonadismHypothyroidismType-II DM

Total 4 marks

Questions1. What is the

diagnosis?2. Give two sexual

characters in this condition

3. Give one cardiac condition that may be associated with this condition

4. What would be the plasma levels of

a. FSHb. LHc. Testosterone

Answers1. Klinefelter Syndrome (47 XXY)2. Any two

Lack secondary sexual characteristics Infertility, azoospermia Gynecomastia Testicular dysgenesis

3. Any oneMitral valve prolapse (55%)Varicose veins (20-40%)

4. Plasma levels ofa. FSH Increasedb. LH Increasedc. Testosterone Decreased

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Questions

a. Diagnosisb. Commonest differential how do you

distinguish between two?c. Give 3 Steps of management

Answers PSVTSinus tachycardia - there is beat to beat

variability.Vagal manoeuvres:

Carotid MassageIce packs over faceValsalva manouver

Adenosine drug of choice.

4 marks4 marks

ECG

Questions Abnormalities Management Which drug eliminates the causative agent

Answers ECG changes of hyperkalemia Calcium gluconate, sodabicarb, asthalin

nebulisation, insulinKayexelate resin.

4 marks

What disease does this PBS picture suggest ?

Spherocytosis 1

CASE SCENARIOS

pH 7.51 (7.35-7.45)PO2 12 KPa (95 mmHg)PCO2 4.7 KPa (35 mmHg)Blood Urea 11 mmol/lSodium 131 mmol/lPotassium 3 mmol/lChloride 83 mmol/l

This is the ABG of a 6 week neonate admitted with projectile vomiting

Case

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?

Answers1. Metabolic alkalosis2. Congenital hypertrophic pyloric stenosis3. Ultrasonographic diagnostic criteria

a) Pyloric thickness>4mm orb) Pyloric length > 14mm

4. Pyloromyotomy (Ramstedt)

This is blood glucose profile of a 10 year boy with Diabetes. His Hb A1C is 12%. At that time his treatment included:Insulin Morning EveningRegular 6U 3UNPH 8U 5U

Blood glucose profile

0

50

100

150

200

250

300

350

400

6:00

AM

1200

1700

2200

2 M

idni

ght

6:00

AM

1200

1700

2200

6:00

AM

TIME

Blo

od

Glu

cose

(M

g/d

l)

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?

Answers1. Increase morning regular insulin dose by 2

Units2. 3 months3. After 5 years in prepubertal children

after 2 years in pubertal children4. Fundal photography

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D/D of polyuria and polydipsia

Screening guidelines

1. What is the diagnosis?2. What is the preferred treatment and dose?3. Give three alternative drugs for treatment.

Answers1. Ventricular tachycardia2. Cardioversion 0.5 to 1 joule/kg3. Amiodarone

ProcainamideLidocaine

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Park: Pediatric Cardiology for Practitioners, 5th ed

How does SVT in children differ from physiologic

sinus 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

CaseA 5 year girl is brought after accidental

ingestion of unquantified insecticide with salivation, lacrimation and miosis

Questions1. Name three muscarinic actions of OP

poisoning2. What is the specific antidote?

Answers1. Increased oral and tracheal secretions,

miosis, salivation, lacrimation, urination, vomiting, cramping, defecation, and bradycardia

2. Atropine

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OP poisoning effectsMuscarinic 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

CaseA 8 year old boy is admitted after consumtion

of > 60 tablets of ferrous sulfate kept at home

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?

Answers1. 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 not adsorb iron. Many adult-strength iron-containing

pills are very large and are often too large for orogastric lavage 3. Deferoxamine

Up to 15 mg/kg per hour via continuous IV infusion

4. MercuryAcrodynia

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Iron Toxicity StagesStage 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.

a b

c d

DiseasePresent Absent

Test

Positive

Negative

•Sensitivity• T(+)/ D(+) = a/a+c

•Specificity• T(-)/ D (-) = d/b+d

•Positive predictive value•D+/T+ = a/a+b

•Negative predictive value• D-/ T- = d/ c+d

Case Analyse the table below

Test Has disease

No disease

Positive 90 10

Negative 10 90

1. Calculate sensitivity2. Calculate specificity

Answer1. Sensitivity 90%2. Specificity 90%

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Case On auditing

urine growth, following were the result

Urinemicroscopy

Pure growth

Multiple growth

>50 WBC 95 15

<50 WBC 10 200

1. Calcute positive predictive value of urine microscopy

2. Calculate negative predictive value urine microscopy

Answer 1. PPV 95/110

2. NPV 200/210

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Case 8A 13-year old boy develops right upper-

quadrant pain and fever with chills and rigors.

An abdominal ultrasound reveals Hyperechoic liver parenchymaDilatation of several intrahepatic bile ductsFew bilateral renal cysts.

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?

Answers1. Caroli’s syndrome2. Cholangitis3. Intra-hepatic ductal ectasia

AR Polycystic kidney Disease4. PKHD1 gene

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Case9A 3 year old boy a case of steroid sensitive

nephrotic syndrome in remission has been brought with Periorbital puffinessProteinuria on dipstick evaluation at home

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?

Answers1. Nephrotic Syndrome

Heavy proteinuria Hypoalbuminemia Edema Hypercholesterolemia

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Answers2. Urine protein 3+/4+ (on dipstick)3. Urine albumin

3+ or 4+ (or proteinuria >40 mg/m2/h) for 3 consecutive early morning specimens having been in remission previously

4. Prednisolone 2 mg/kg/day (single or divided doses) until urine

protein is trace or nil for 3 consecutive days Then 1.5 mg/kg on alternate days for 4 weeks Then discontinued

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Case10A 12 year old girl a case of chronic kidney

disease due to FSGS is evaluated in OPD for hypertension, poor growth and dyslipidemia.

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.

Answers1. GFR(ml/min/1.73m2)

= 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

1

Answers2. Stage 2 CKD: GFR 60-89 ml/kg/1.73m2

3. True/ Falsea. True

Children with CKD who remain <2 SD for height despite optimal medical support may benefir from rHuGH (0.05mg/kg/d)

b. FalseAnemia is primarily the result of inadequate erythropoietin production

c. TrueThe most common finding in renal osteodystrophy is osteitis fibrosa cystica (high turnover bone disease secondary to hyperparathyroidism)

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Answers2. Stage 2 CKD: GFR 60-89 ml/kg/1.73m2

3. True/ Falsea. True

Children with CKD who remain <2 SD for height despite optimal medical support may benefir from rHuGH (0.05mg/kg/d)

b. FalseAnemia is primarily the result of inadequate erythropoietin production

c. TrueThe most common finding in renal osteodystrophy is osteitis fibrosa cystica (high turnover bone disease secondary to hyperparathyroidism)

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What are the main causes of chronic renal disease in children that result in renal

transplantation?

Obstructive uropathy Aplastic/hypoplastic/dysplastic kidneys Focal segmental glomerulosclerosis

CaseThe 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.

EEG

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?

Answers1. Hypsarrhythmia

Large amplitude slow wave activity mixed with multifocal spikes and sharp waves

1

Answers2. Infantile spasms- West Syndrome3. Tuberous Sclerosis4. Treatment

Unsatisfactory on most occasions AED`s Vigabatrin

Benzodiazepines(Clonazepam/ Nitrazepam)

Steroids/ ACTH

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What is the classic triad of infantile spasms? Spasms, hypsarhythmia, and

developmental regression

Infantile spasms are known as West's syndrome, and the condition is named for the physician who first described the condition in his own son in 1841.

After what period can AEDs be safely discontinued? When the child is free of seizures for 2 years

Smith R, Ball R: Discontinuing anticonvulsant medication in children. Arch Dis Child 87:259-260, 2002

Case 15The 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.

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?

Answers1. Occult spinal dysraphism 2. Risk of recurrence

One affected child 3-4%Two affected Children 10%

3. Intervention Folic acid 400 mcg/dStarted Periconceptionally

(started before pregnancy) Continued Till 12 wks of preg

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Answers4. Bladder incontinence management

Clean intermittent catheterization Artificial urinary sphincter Surgical urinary diversion Augmentation cystoplasty

2

What is the likelihood that a patient with myelomeningocele will have hydrocephalus?

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.

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.

Questions?1. Which of the following conditions might explain this

child’s condition?a. Phenylketonuriab. Homocystinuriac. Cystathioninuriad. Maple syrup urine diseasee. Histidinemia

2. Which one other systemic examination would you like to do?

3. What investigation would confirm the etiology?4. What treatment would you institute as a long term

measure?

Answers1. Homocystinuria (b)2. Eye (Ectopia lentis)3. Elevated plasma levels for

homocystine4. Vitamin B6 (200-1,000 mg/24 hr)

Folic acid 1-5mg/24 hr

Vit B 12 (1-2 g/24hrs)

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Match the following

Match the following sign/symptom with the expected location of the lesion in the brain:

a. Deafferented pupil

b. Cerebellum

c. Ipsilateral cortex

d. Midbrain tectum

e Pons

f. Cribriform plate of

ethmoid

Nystagmus.

Tonic deviation of the eye(s).

Marcus Gunn pupil.

Horner’s syndrome.

Midposition pupils

Pinpoint pupils

Leakage of CSF from nose.

Answera. Deafferented pupil Marcus Gunn pupil

b. Cerebellum Nystagmus

c. Ipsilateral cortex Tonic deviation of the eye(s)

d. Midbrain tectum Midposition pupils

e. Pons Pinpoint pupils

f. Cribriform plate Leakage of CSF from nose

3 marks

Match the following

a. Rete mirabile of Moyamoya.

b. Right-to-left cardiac shunt.

c. Arteriovenous malformations.

Thrombotic stroke.

Embolic stroke.

Intracerebral hemorrhage.

Answera. Rete mirabile of Thrombotic stroke

Moyamoya.b. Right-to-left cardiac Embolic stroke

shunt.c. Arteriovenous Intracerebral

h`age malformations.

Case19A 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

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?

Answers1. Decremental response to repetitive

stimulation2. Enlarged thymus3. Edrophonium test4. Aminoglycosides5. Neostigmine 0.4mg/kg PO 4-6 hrly

0.04mg/kg IM 4-6 hrly

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Fleisher GR, Ludwig S (eds): Textbook of Pediatric Emergency Medicine, 3rd ed.

Case 20Examination 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/dLGlucose 25 mg/dL

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?

Answers1. Tubercular/ Fungal meningitis2. Mannitol, glycerol3. Sixth cranial Nerve (Abducens)

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Case 21At 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.

Question1. The laboratory finding likely to explain this

child’s problem isa. Reduced serum hexosaminidase A activityb. Deficient activity of acid beta-galactosidasec. A defective gene on the X chromosomed. Complete lack of acid alpha-galactosidase activitye. Deficient activity of galactosyl-3-sulfate-ceramide

sulfatase

2. What is the diagnosis?

Answers1. Deficient activity of acid beta-galactosidase (b)2. Type 1 GM1 gangliosidosis

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22For each description select the most likely

diagnosis:

1. Eye blinking or throat-clearing noises in an otherwise healthy 8-year old boy

2. A 6-year-old boy with eye twitching and ecolalia

3. A 2-year-old infant who was born prematurely and is unable to walk or talk

4. A 14-year-old girl with a history of precocious puberty who now develops a large goiter

5. An infant with infantile spasms, a hypsarrhythmic EEG pattern, and ash-leaf depigmentation on her back

Diagnosis

a. Transient tic disorder of

childhood

b. Tourette syndrome

c. Cerebral palsy

d. Tuberous sclerosis

e. McCune-Albright

syndrome

Clinical scenario

Answer1. Transient tic disorder of childhood2. Tourette syndrome3. Cerebral palsy4. McCune-Albright syndrome5. Tuberous sclerosis

Total 5 marks

Case scenario 2316 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%

Questions1. Diagnosis2. Which of the above measurement is best for

monitoring

Answer1. Asthma

2. FEV1/FVC

List the possible acute side effects of salbutamol General: Hypoxemia, tachyphylaxis Renal: Hypokalemia Cardiovascular: Tachycardia, palpitations,

premature ventricular contractions, atrial fibrillation

Neurologic: Headache, irritability, insomnia, tremor, weakness

Gastrointestinal: Nausea, heartburn, vomiting

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.

Question 24Below is diagram of lung volumes,what do

the letters A and B represent

Case No 28

7 years male c/o increasing weakness of all limbs x 3 days & URTI one week back

ExaminationCNSBilateral facial nerve palsies

Power Grade 0 to 1 both legs; grade 3 armsLoss of DTJ,No sensory deficit/ papilloedema

AbdomenPalpable midline mass arising from pelvis

Other systems: Normal

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?

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

Answers2. 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)

Case No 30A 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.

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?

Answers1. Neurosurgical Opinion/ CT skull

a. Deteriorating level of consciousnessb. Focal neurological signsc. Depressed Skull fracture d. Basal Skull fracturee. Seizuresf. CSF leakg. Signs of Raised ICT (2)

Answers2. Temporary manoeuvres to reduce raised

ICT Nursing in a 30 degrees head up position Diuretics (Mannitol) Hypertonic saline Artificial hyperventilation

(2)

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)

CASE SCENARIO7 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 Rtknee and easy fatigability. Has leucocytosis, raised ESR, normal chest skiagram and negative Mx.

1. Give most probable diagnosis.

2. List other expected systemic clinical findings

3. Give value of serology in diagnosis

4. Outline steps in management

5. List prognostic factors

1. JRA – Systemic 0.52. Systemic exam findings

a. Hepato – splenomegaly 0.5b. Lymphadenopathy 0.5c. Serous cavity effusions 0.5

3. Serologya. ANA - Negative 0.5b. RF - Negative 0.5

4. Managementa. “Pyramid” approach – NSAIDS, chloroquine,

1.0 methotrexate,azothiaprine/cyclophosphamide.

b. Steroids 0.5c. TNF-alpha receptor antagonists

0.55. Number and severity of joint involvement

1.0(Total Marks 6.0)

This patient becomes ill with thrombocytopenia, profound anemia, and markedly elevated transaminases

probably has what complication? Macrophage activation syndrome

CASE Scenario12 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 achievedb. Dose of factor VIII

3. The patient has to undergo major surgerya. Level of factor VIII to be achievedb. Dose of factor VIII

4. What are the other precautions?5. What is the role of DDAVP in haemophilia A ?

1. <1% 0.5

2. Factor VIII in treatmenta. 35-40%

1.0b. 20 units/kg, repeat daily if required

1.0till joint normalises

3. Dose in major surgerya. 100%

1.0b. 50 units/kg, infuse 2-3 units/kg/hr to 1.0

maintain level at 100%for 24 hrsand then >50% for 7 days

4. Precautionsa. Avoid Anti-inflammatory drugs with anti-platelet

0.5action

b. Screen for Transfusion transmitted diseases if 0.5plasma products are used

5. Desmopressin Acetate: Release of factor VIII in mild cases 0.5

(Total marks 6.0)

CASE ScenarioA 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 diagnosis2. State most probable group of aetiology

based on answer to question ‘1’3. List three most important tests for

diagnosis with values4. Outline the management of this child

1. Kayser-Fleischer rings 0.5

2. Wilson disease 0.5

3. 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 0.5

1. Management a. Restrict copper intake

0.5b. Chelation with penicillamine 0.5c. B6 supplementation 0.5d. Liver transplant 0.5

(Total marks 6.0)

Drugs & Devices

Identify the instrument.Which illness is it used in ?What are the three settings ?

Peak Flow MeterBronchial Asthma

1.Green zone >80% Peak Exp Flow2.Yellow Zone 50-80 % PEF3.Red zone < 50% PEF

Total 3 marks

Which class does this antibiotic belong to ?

What is the spectrum?

What is the dosage?What is the imp

clinical use? 4 marks

OxazolidinonesGram-positive organisms including MRSAPenicillin-resistant pneumococciGram-negative anaerobesMycobacteria 10mg/kg/dose 8 hrlyMultiresistant gram-positive organisms,

including MRSA who have failed to respond to vancomycin.

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

Parental nutrition Dose 0.5 -3.5 g/kg/dayWhen enteral nutrition is insufficient or

contra-indicated.Day 1 Dyselectronemia, cholestasis, bacterial or

fungal colonisation and sepsis, thrombosis, azotemia, thrombocytopenia

Identify the product?

What are the advantages of its use?

Enumerate five complications?

PICC 1Preserves veins, allows concentrated infusates, permits long

term IV alimentation1

6 complications- 3Catheter fracture and embolisation, Leakage Thrombosis of catheterAccidental displacement / Tip MigrationDeep Venous ThrombosisInfection

What is the equipment ?

Identify the parts.Enumerate indications for

its use. What are the contra-

indications? How do you sterilise it?

5 marks

Self inflating 250 ml ambu bag 1Oxygen , 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.

1MSAF 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

Identify the product? What is the dosage? What are the indications for use?

Enumerate three precautions ?

Surfactant 0.54ml/kg 0.5Surfactant 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

What is the equipment? What are the indications of

its use?

What are the advantages?

Double lumen endotracheal tube 1

For surfactant instillation 1 To aid lung lavage

Permits efficient drug instillation without interrupting ventilation 1

Identify the equipmentIndications for usageComplications (mention any three)

Bubble CPAP apparatus 1Indications - 2

If FiO2 requirement is > 0.3If FiO2 requirement is > 0.3 SA Score SA Score >> 4 4 ABG Score ABG Score >> 3 on oxygen therapy 3 on oxygen therapy Frequent apnea with documented hypoxemia Frequent apnea with documented hypoxemia Clinically significant chest retractions after Clinically significant chest retractions after

extubation from mechanical ventilationextubation from mechanical ventilation ComplicationsComplications 11

Air leakAir leak Reduced venous returnReduced venous return IVHIVH

Which class does this antibiotic belong to ?

What is the spectrum?What is the dosage?What is the two imp side

effects?

CarbapenemsWidest spectrum of antimicrobial activity

among the β-lactam antibiotics against both gm-ve and gm + organisms except MRSA.

20 - 40mg/kg/dose 12hrlyTransient agranulocytosis, thrombocytopenia.Seizures

2 marks

ABG1pH 7.28

PaCO254mmHg

PaO2 45mmHg

HCO3 29mEq/L

Base excess +7

What are the-

Abnormalities? 1

Diagnosis? 1

Likely causes? 1

Low pH, high PaCO2, low PaO2, high HCO3

Uncompensated respiratory acidosis with hypoxemia

RESPIRATORY FAILURE (pneumonia, RDS)

ABG 2pH 7.57PaCO2 22mmHgPaO2 156mmHgHCO3 18mEq/LBase excess -8

What are the-

Abnormalities? 1

Diagnosis? 1

Likely cause? 1

High pH, low PaCO2, high PaO2, low HCO3

UNCOMPENSATED RESPIRATORY ALKALOSIS WITH HYPEROXIA

Hyperventilation with high FiO2

ABG 3

pH 7.32

PaCO2 30mmHg

PaO2 70mmHg

HCO3 12mEq/L

Base excess -8

QUESTIONS ABNORMALITIES 1 DIAGNOSIS 1

LIKELY CAUSES 1

• Low pH, low PaCO2, normal PaO2, low HCO3• UNCOMPENSATED METABOLIC ACIDOSIS• SHOCK, RENAL FAILURE

ABG 4

pH 7.60

PaCO2 21mmHg

PaO2 65mmHg

HCO3 24mEq/L

Base excess +2

QUESTIONS ABNORMALITIES 1

DIAGNOSIS 1

LIKELY CAUSES 1

High pH, low PaCO2, normal PaO2, normal HCO3

UNCOMPENSATED RESPIRATORY ALKALOSIS

HYPERVENTILATION (BRONCHIAL ASTHMA)

ABG 5

pH 7.36

PaCO2 70mmHg

PaO2 75mmHg

HCO3 35mEq/L

Base excess +14

QUESTIONS ABNORMALITIES 1

DIAGNOSIS 1

LIKELY CAUSES 1

Low pH, high PaCO2, normal PaO2, high HCO3

COMPENSATED RESPIRATORY ACIDOSIS

VENTILATED INFANT WITH TUBE BLOCK

ABG 6

pH 7.52

PaCO2 47mmHg

PaO2 80mmHg

HCO3 30mEq/L

Base excess +3

QUESTIONS ABNORMALITIES 1

DIAGNOSIS 1

LIKELY CAUSES 1

High pH, high PaCO2, normal PaO2, high HCO3

UNCOMPENSATED METABOLIC ALKALOSIS

VOMITING, PYLORIC STENOSIS

ABG 7

pH 7.14

PaCO2 54mmHg

PaO2 55mmHg

HCO3 14 mEq/L

Base excess -7

QUESTIONS ABNORMALITIES 1 DIAGNOSIS 1 LIKELY CAUSES 1

Low pH, high PaCO2, normal PaO2, low HCO3

UNCOMPENSATED RESPIRATORY AND METABOLIC ACIDOSIS

INFANT ON VENTILATOR WITH TUBE BLOCK & SHOCK

Describe the lesion.

What is the DD ?

Brain Stem cell glioma,

1 mark

Observed Station 1DRUG COUNSELLING

4 yr old boy weighing 15 Kg , diagnosed as Nephrotic Syndrome to be started on steroids.

1. Introduces self/ puts the child and attendant at ease. 0.5

2. Explains the disease in simple words 0.5

3. 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 0.5

6. Asks for queries if any. 0.5

7. Advises to report back if any problems. 0.5

8. Advises about pain abdomen 0.5(Total marks 6.0)

Observed Station 2

DRUG 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.

1. Introduces self & puts child and 0.5attendant 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 2.0with meals, sites, route and rotation of sites

5. Explains the calculation of dose in ml. 1.06. Explains the sequence of loading (regular first). 0.57. Briefs about hypoglycemia incl mgt.

0.58. 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)

Observed Station 3COUNSELING

24 yr old lady diagnosed as HIV + at 36 weeks of gestation. Counsel regarding perinatal transmission and follow up.

1. Ensures the presence of husband0.5

2. 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 0.5Intranatal 0.5Breast feeding 0.5

5. Explains modalities of reducing rate of transmissionART to mother and child 1.0LSCS Vs Vaginal delivery 1.0Breast feeding Vs top feeding 1.0

6. Explains effect of measures – reduction by 50%1.0

7. 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)

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.

Observed Station 4Drug Counseling

1. Introduces self & puts child and 0.5attendant at ease.

2. Explains the problem in brief. 0.53. Familiarizes the attendant with the drug 0.5

and dispenser4. Explains dose(75 mg-3.5 ml), in 3 divided 0.5

doses5. Advises not to miss the dose 0.56. Increase to (150 mg – 7.5 ml) 0.57. Explains side effects 1.08. Calls for blood counts weekly 0.59. Calls for serum levels of the drug 1.010. Advises to report in case of doubt 0.5

(Total marks 6.0)

Observed Station 5

DRUG COUNSELING

2 yr old child weighing 10 Kg is being discharged after admission for febrile seizure. Counsel the mother regarding domiciliary management of seizure with rectal diazepam.

1. Introduces self & puts child and 0.5attendant at ease.

2. Explains the problem in brief. 0.53. Familiarizes the attendant with the drug, 0.5

the syringe and the catheter.4. Explains dose(3mg), and loading of the syringe 0.55. Explains the position of the child (lateral) 0.56. Explains introduction of the catheter (lubrication, 1.0

and length of introduction)7. Explains pushing the drug

0.58. Advises pinching of the buttocks together after 1.09. Withdrawing the catheter)10. Advises one repetition after 15 mts

0.511. Explains side effects 0.5

(Total marks 6.0)

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