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MOCK OSCE 2012
    PUNE
• The OSCE Questions & Answers are prepared by
  the faculty for the benefit of Post graduate
  students and some are contributed from faulty
• These Questions and answers are made for the
  students to understand the pattern of OSCE and
  not meant to completely cover the subject of
  Pediatrics.
• Answer are checked from standard textbook in
  case of doubt plz email

•               ashwinborade@yahoo.com
    11/7/2012             CME,PUNE             2
Station 1

• A 10 year old boy brought to ER having consumed 5
  tablets of Tricyclic Antidepressants tablets 2 hrs back.
  On examination he is drowsy, but arousable. HR
  138/min. temp 100 F. RR 10/ min. BP 80/60 mmHg.

• What are the clinical manifestations of tricyclic
  antidepressant poisoning?
• What are the cardinal cardiovascular indicators of
  toxicity?
• What is the poor prognostic factor?
• What are the criteria for ECG diagnosis of poisoning ?
• What is specific management and principle behind it?
Answer station 1
• Anticholinergic toxidrome-delirium, mydriasis, dry mucus membranes, tachycardia,
  hyperthermia, mild hypertension, urinary retention, slow GI motility. CNS toxicity-
  lethargy, coma, myoclonic jerks, seizures. Sinus tachycardia-most imp; widening of
  QRS complex, PVC, ventricular arrhythmias .Refractory hypotension.

• Sinus tachycardia; refractory hypotension; QRS duration of > 100 ms indicates risk
  for seizures & cardiac arrhythmias; R wave in lead aVR of > 3 mm is also an
  indicator of toxicity.

• Refractory hypotension

• QRS duration of > 100 ms indicates risk for seizures & cardiac arrhythmias; R wave
  in lead aVR of > 3 mm is also an indicator of toxicity

• Alkalinisation with soda bicarb therapy (ANTIDOTE). Goal of bicarb therapy is to
  increase sodium concentration and arterial pH. Dose is 1-2 meq/kg until pH> 7.45.
  150 meq/L of sodium bicarb diluted with 5% dextrose to maintain pH. Treat
  hypotension with normal saline; Vasopressors for hypotension; Airway
  management.
Station 2

  A 9 month old infant who was playing with marbles is
  suddenly found to the struggling to breathe. What would
  be your steps of intervention.

• 1. Till the baby is conscious.(1)
• 2. If the baby becomes unconscious. (1)
Station 2B
• Identify the ECG.         [1]
• Write three common causes of this problem.
  [1)
• Write treatment of this problem [1 ]
Answer Station 2 A

1 (a) 5 back blows between the scapula.
  (b) 5 chest thrusts on the lower 3rd of sternum.

2 (a) Give –
1. 2 rescue breaths
2. 5 back blows
3. 5 chest thrusts
4. Look into the mouth
5. If FB seen - remove it.
6. If not - give 2 breaths and continue the sequence.
Answ 2 B
• Atrial fibrillation
•
• Rheumatic mitral valve disease ,left AV valve
  insufficiency, thyrotoxicosis, pulmonary
  emboli, and pericarditis
•
• The best initial treatment is digitalization,
  which restores the ventricular rate to normal,
  although the atrial fibrillation usually persists.
Station 3 :
A 13 year old boy under investigation in OPD

1. What is the abnormality? (1)
2. What is the diagnosis? (1)
3. What clinical features are likely to be present in the child? (1)
4. . Name 2 condition with X linked Recessive inheritance. (1+1)
Answer station3
1. Defect in the long arm of X chromosome(Fragile sites)
   (Fragile sites are regions of chromosomes that show a tendency for separation,
    breakage, or attenuation under particular growth conditions. The abnormality
    is caused by FMR1 gene expansion at Xp27.3 which is a fragile site)

2. Fragile X Syndrome

3. Long thin face
   Prominent jaw
   Macro orchidism
   Large ears
   Hyperkinetic behavior
   Mental retardation
   Large head
   Normal height

4. X linked Recessive Hemophilia
    Duchenne Muscular dystrophy
    Becker Muscular dystrophy
Station4
    A six week old infant is referred for poor weight gain and
    seizure. On examination, he looks cachexic and is dysmorphic
    with low set ears and a very small lower jaw. He also has severe
    thrush affecting the oral cavity and perineum. Cardiac apex is
    displaced into the left sixth intercostal space in anterior axillary
    line and there is a palpable thrill. A loud pan systolic murmur
    and an apical mid diastolic murmur can be heard. He is
    tachypneic but the lung fields are clear on auscultation. The
    liver is 3 cms below the costal margin in the mid clavicular line.
    Investigations: Na 136, K 4.9, Ca 1.7,Albumin 4.2, Urea 7.8, Hb
    10.2, WBC 3.8, Platelet 2.45 L, Echo Large VSD

•   What is the diagnosis?(1)
•   What is commonest presentation during newborn period. (1)
•   Most likely explanation for the low plasma calcium ?(1)
•   What is the causes of immunodeficiency? (1)
•   The etiopathogensis of all these features is ? (1)
Answer 4

1. DieGeorge syndrome
2. Hypocalcemic seizures
3. Primary hypoparathyroidism
4. Thymus gland aplasia leading to abnormal cell mediated
   immunity
5. An embryological defect of the third and fourth pharyngeal
   pouches
Station5
15 months old child admitted with anemia with hepatosplenomegaly for
evaluation, PS shows.
1.Identify and describe PBS findings. (1)
2.What is the diagnosis? (1)
3.What is definitive treatment? (1)
4.What are clinical features seen in classical case ? (1)
5.What is the basic etiology for this disorder ? (1)
Answer 5



1. PBS showing microcytic, hypochromic anemia with
   nucleated RBC, tear drops cells
2. Thalassemia major
3. BMT
4.Thalasemmic facies, Hs megaly, bone fractures
5. Ineffective erythropoisis
Station 6A
Identify the slide.(1)
What is treatment?(1)
Station 6 B

State True or False-
1. The following are useful in assessing the gestational age of an infant-        (0.2 x5)
      a) Posture                T/F      b) Palmar creases       T/F
      c) Elbow angle            T/F       d) Square window test T/F
      e) Nipple formation      T/F
2. Blood flow in the fetus-                                                      (0.2 x5)
     a) Blood flows from right to left through foramen ovale- T/F
     b) Blood in the ascending aorta has higher oxygen content than the blood in
    descending aorta-                     T/F
    c) The ductus arteriosus is closed T/F
    d) Pulmonary pressure equals systemic pressure T/F
    e) Hemoglobin may be 20 g/dl T/F
3. Genetic anticipation occurs in the following conditions-                        (0.2 x5)
          a) Cystic fibrosis              T/F
          b) Myotonic dystrophy           T/F
          c) Fragile X Syndrome           T/F
          d) Huntington’s chorea           T/F
          e) Marfan’s Syndrome             T/F
Answer :
6A :
a) Egg of Ascaris Lumbricoides
b) Albendazole 400 mg or Mebendazole 100 mg BD x 3 days

6B:
1. The following are useful in assessing the gestational age of an infant-
    a) True b)False c) False d) True e) True

2. Blood flow in the fetus-
    a) True b) True c) False d) False e) True

3. Genetic anticipation occurs in the following conditions-
    a) False b) True c) True d) True e) False
Station 7
A 15 yrs old child with no significant past history comes in OPD with
   slow myoclonic seizures since 2 days. Also parents noticed he had
   subtle personality changes and poor school performance .His EEG
   done

What are the EEG changes? (1)
What is the diagnosis?(1)
What is diagnostic criteria (1)
What is etiology for same ?(1)
What are CNS complications?(1)
Answer 7


•   High voltage burst slow waves with normal background
•   SSPE
•   Measles ab in CSF/EEG/histological finding on biopsy
•   Persistent measles viral infection in CNS
•   Hypertonia, bulbar palsy,choreatheosis,decerebrate
    posturing
• Station 8 A

A. What test is performed ?(1/2)
B. What are indications? (1/2)
C. What 2 important parameters
   it measures? (1/2)
D. Based on these result how
   can you differentiate disorders ? (1)


• Station 8 B

A. What is the elemental content of 1 ml ?(2)
B. Which solution is incompatible if mixed with this drug?(1)
C. Give 2 indications for its use (2)
Answer 8
A
A   Spirometry
B   To differentiate obstructive n restrictive respiratory diseases.
C   FVC and FeV1
D   FVC,FEV1 both low- restrictive
    FVC-N FEV1 Low - obstructive
    FVC low FEV1 very low- mixed diseases .
B
A. Ca Gluconate 10% (1 ml = 9 mg elemental Ca)
B. Do not mix with HCO3
C. Bradycardia, Asystole and Sclerosis
Station 9
Write the specific odour of urine in following condition (10x0.5=5 )

1.Glutaric acedemia                     mousy
2. Hawkinswria                          tomcat urine
3. 3-hydroxy 3 methyglutaric acidemia   maple syrup


4. Isovaluric acedemia                  cat urine
5. Maple syrup urine diseses            sweaty feet


6. Hypermethioninemia                   rancid butter /boiled cabbage
7. Multiple carboxylase defiency ,      boiled cabbage


8. Phenyketnuria                        swimming pool


9. Trimethylamuria                      rotting fish
10. Tyrosiemia                          sweaty feet
Answer Station 9
    The specific odour of urine in following condition
•   Glutaric acedemia –sweaty feet
•   Hawkinswria,-swimming pool
•   3-hydroxy 3 methyglutaric acidemia,-cat urine
•   isovaluric acedemia,-sweaty feet
•   maple syrup urine diseases –maple syrup
•   hypermethioninemia,-boiled cabbage
•   multiple carboxylase defiency ,-tomcat urine
•   Phenyketnuria-mousy
•   Trimethylamuria,-rotting fish
•   Tyrosiemia-rancid butter /boiled cabbage
Station 10 A

2 yrs old child admitted
with anemia , hepatosplenomegaly
and fever. PBS shows :




• What is the diagnosis?(1)
• What are complications?(1)
• What is late treatment failure in this case.(1)
Station 10 B
1. What is the radiological diagnosis? ( 1)
2. Name 2 risk factors for the development of this condition (1/2 +1/2)
Answer 10 A

• Falciparum malaria
• Severe anemia, cerebral malaria, respiratory distress,
  hypoglycemia
• Late treatment failure: Patient will exhibit late treatment
  failure (LTF) under following situations:
• (i) Development of danger sign or severe malaria on any
  day between day 4 and day 14 in presence of parasitemia.
• (ii) Axillary temperature >37.5ºC in presence of parasitemia
  on any day from day 4 to day 14.
Answer 10 B
1. Right & Left pneumothorax with left upper zone haziness

2.Risk Factors-
• Assisted ventilation (including CPAP)
• MAS,RDS
• Other Air Leak Syndromes (e.g. PIE)
• Pulmonary hypoplasia
• Idiopathic or spontaneous
Station 11
• A:Write content of the following in Human milk fortifier
  (1/4X 8=2)
             1. Protein
             2. Fat
             3. Sodium
             4. Calcium
             5. Vit D
             6. Thiamine
             7. Vit B12
             8. Zinc
• B:Write biological value, net protein utilization and protein
  efficacy ratio of –
             1.Meat
             2. Egg
             3. Bengal gram
             4. Wheat                              (12 X 0.25=3 )
Answer 11A
PROTEIN         0.2g
Fat             0.1g
NA              1.5
calcium         50
Vit D           250
Thiamine        12
B12             0.05
Zinc            0.18
Answer11B
              BIOLOGICAL   NPU   PER
              VALUE
Meat          74           76    3.2
EGG           96           96    3.8
Bengal gram   74           61    1.3
WHEAT         66           61    1.3
Station 12
• Identify diagram .(1)
• What are the uses? (1)
• Name each components A-F along with interpretation of
  each (1/4 x 6=3)
Answer 12
• Scatter diagram and correlations.

• A scatter diagram is a tool for analyzing relationships between two
  variables. One variable is plotted on the horizontal axis and the other is
  plotted on the vertical axis. The pattern of their intersecting points can
  graphically show relationship patterns.

• A;No Correlation There is no demonstrated connection between the two
  variables
• B;Weak Positive Correlation The value of Y increases slightly as the value of
  X increases
• C;Strong Positive Correlation The value of Y clearly increases as the value of
  X increases.
• D;Weak Negative Correlation The value of Y decreases slightly as the value
  of X increases
• E;Strong Negative Correlation The value of Y clearly decreases as the value
  of X increases.
• .F; Perfect Correlation The value of Y seems to be related to the value of X.
Station 13
• 2 yrs old girl brought with pubic hair and clitoromegaly, increased
  irritability gradually increasing since the past 3 months. No history
  of drug ingestion or hyperpigmentation.
• On examination she has BMI above the 95 th centile. BP:112/80
  mm of Hg. HR:92/min & RR:24/min. She has hirsutism and few
  acneform eruptions on the face. Her cheeks are chubby & few pink
  striae present over the face and abdomen. Genital examination
  shows pubic hair stage 2 & clitorus measuring 1.8 cms. She is
  extremely irritable. Systemic examination is normal.

• What is the diagnosis? (1)
• What is most likely etiology at this age? (1)
• Enlist 2 important screening tests used for the above condition?
  (1+1)
• Name 2 drugs used for the medical management of this condition?
  (1/2+ 1/2)
Answer 13
• Cushings syndrome
• Etiology:

   Below age of 7 yrs: ACTH independent autonomous adrenal lesion like Adrenal
    adenoma or carcinoma

• Screening tests:
      Assessment of diurnal cortisol rhythm
      Overnight dexamethasone supression test
      24 hour urinary free cortisol
      Salivary cortisol

• Drugs that can be used:
               Ketoconazole
               Aminoglutethimide
               Cyproheptidine
               Metyrapone
               Mitotane
Station 14
6 yr old boy brought with the deformity shown in the figure. His sister, 8 yrs also has a
   similar deformity gradually increasing since 3 yrs of age. On examination his height
   is below the third percentile & US/LS ratio is 1.6:1. There are multiple dental
   abscesses. Mother also has a similar deformity but very mild in nature. S. calcium :
   8.8 mg/dl, Phosphorus : 1.8 mg/dl Alk phosphatase: 380 IU PTH levels: 18 pg/ml
   (9-55 pg/ml) 25-OH Cholecalciferol: 26 ng/ml(10-50 ng/ml)
1,25(OH)2Cholecalciferol: 18 pg/ml (20-60 pg/ml)
TMPO4/GFR: reduced. X-ray of both
lower limb shows florid richetic changes

•   What is the Diagnosis? (1)
•   What is the inheritance ?(1)
•   It is due to mutation of which gene?( 1)
•   What is the Treatment?(1)
•   Write 4 endocrinal causes of short stature. (1)
Ans 14

• Hypophosphatemic Rickets

• X-linked dominant

• Mutation in PHEX gene

• Treatment: Phosphorus supplement orally- 1-4 g/day QDS Alfacalcidol- 25-50
  ng/kg/day or calcitriol 0.25-2 mcg/day.

• 4 causes-
1.Pan hypopitutarisum
2.Isolated GHD
3.Hypothyrodisum
4.Cushing syndrome
5.DM
Station 15
Our patient is a 17 yr old female with history of recurrent seizures. Admitted in PICU
  with stroke. She was evaluated for same.
• Identify and describe the investigation? (1/2+1/2)
• What is the diagnosis? (1)
• What other disorder are associated with this disease? (1)
• What is the treatment of choice? (1)
• What are syndromes associated with contralateral hemiplgia in children? (1)
Answer 15
1. a)MRI T1 weighted images show extensive collateralization
   of vessels in region of circle of willis.
   b) MRA reveals reduced luminal calibre of left internal
   carotid artery in petrous, cavernous and supraclinoid
   segments.
2. Moya moya disease.
3. Down syndrome, neurofibromatosis, or sickle cell disease
4. Direct superficial temporal artery (STA) to middle cerebral
   artery (MCA) bypass is considered the treatment of choice.
5. Weber syndrome, Benedict syndrome ,millard gublar
   syndrome.
Station 16 A



• Write RNTCP cat IV regimen (1)
Station 16 B :

What is the graph?                               (1)
What are its uses?                               (1)
How do you use it?                               (1)
What are the other charts used for this purpose? (1)
Answer 16 A

6(9) Kmofx(Lvx) Eto Cs Z E/ 18 ofx (Lvx) Eto Cs E


                           Answer 16 B

• Trivandrum development screening chart
• Development assessment upto 24 months
• SAT hospital, TVM
• A vertical line is drawn or pencil is kept vertically at level
  age at child being tested ,if child fails to achieve any item
  that falls short on left side of vertical line child had
  developmental delay
• DDST,DST,BSID
Station 17 ( 5 marks)
         Write the laboratory values for following disorders
                             ( either N,
              calcium   phosphorus   PTH      25(OH)2D3   1,25(OH)2
                                                          D3

Vit D def.

VDDR type 2

CRF

ADHR

Fanconi
syndrome
Answer 17

             calcium   phosphorus   PTH      25(OH)2D3   1,25(OH)2
                                                         D3


Vit D def.   N / dec   dec          incre    dec         Dec/N/Inc

VDDR type 2 N/dec      dec          incres   N           incre

CRF          N/dec     incre        incre    N           dec

ADHR         N         dec          N        N           Rd

Fanconi      N         dec          N        N           Rd or incre
syndrome
Station 18
   2 yrs old child with MR admitted for evaluation , On examination he had anemia
   with organomegaly. Lab evaluation showed anemia with thrombocytopenia .BM
   done .
1.What is the diagnosis?
2.What are the marrow findings?
3.What is the etiology for such findings?
4.What are the typical radiological findings?
5.What is the definitive treatment?
(1x5=5)
Answer 18

•   Gaucher disease .
•   Wrinkled paper cells in BM
•   Intracytoplsmic substrate inclusion
•   Erlenmeyer flask deformity in distal femur
•   Enzyme replacement with b glucosidase
Station 19 (1X5=5)

1.How will you immunize a child with bleeding disorder? (1)

2.Name the vaccines which can be placed in the freezer
  compartment of the refrigerator                      (1)

3.Write down the time limits for using the following vaccines after
  reconstitution-
• Varicella                                               (1)
• Measles/MMR                                             (1)

4. Write the schedule of rabies vaccine for a person, who has been
   bitten by a dog but has received 5 doses of rabies vaccine
   earlier.                                                (1)
Answer 19

1.Subcutaneous route should be used (Unless contraindicated)
  For aluminium adjuvanted vaccines that can only be given intramuscularly,
   vaccination should be scheduled after factor replacement therapy
  Needles <23G should be used for injection and the parents should be asked to apply
   firm and sustained pressure, without rubbing, for at least 5 minutes.

2.Freezer compartment: BCG, OPV, Measles, and MMR.

3. Varicella = 30 min (and protect from light)
   Measles/MMR = 4 to 6 hours

4. Two doses are given on days 0 and 3.
   (For re exposure at any point of time after completed (and documented) pre or post
    exposure prophylaxis)
Station 20

Interpret the following ABG reports:

1. pH-7.6/ PaCo2-25/ Pao2-160/ HCo3-24 (Fio2-50%)

         Calculate AaDO2 and write the formula (1+ 1/2)


2. pH-7.38/ PaCo2-65/ Pao2-48/ HCo3-34 (Fio2-60%)

         (a) what is the ABG diagnosis (1)
         (b) What is normal PaO2 level expected if a child is breathing at room air
     with normal lungs? (1)


3.    pH-7.45/ PaCo2-40/ Pao2-120/ HCo3-28.5/ Spo2 99%/ Hg 8 gm%

       Calculate Oxygen content in given blood gas (1+1/2)
Answer 20

1. 165.25
(Formula)
AaDO2 = (713 x FiO2) – (pCO2 / 0.8) – (paO2)

2. a)Respiratory acidosis with metabolic compensation
   b) 80-100 mmHg

3. 11 ml O2 /dl
   Arterial Oxygen content = (Hb x 1.36 x SpO2 ) + ( 0.0031
  x PaO2)
Station 21 :



Which materials are used to sterilize the given items- (1X5=5)

•   Floor
•   Ambu bag
•   Chittle forceps
•   Probe of pulse oximeter and Weighing scale
•   Laryngoscope
Answer 21

• FLOOR : By Phenol
• Ambu bag : after dismantling clean with soapimmearse
  in Cidex for 4-6 hrs rinse with water dry,wrap and put
  a date
• Cheattle forceps : autoclave
• Probe of Pulse oxymeter : by spirit
• Weighing machine : 2% Bacillocid
• Laryngoscope : by spirit. If used for infected patient than
  wash with soap & water -- after removing bulb,put blade
  in 2% cidex  wash, dry and wrap.
Station 22
                    Match the following (5)
                                                           Characterstics
Level of evidence




        I                                                    Case series




       II                           Lesser quality RCT ( eg less than 30% follow up; no blinding;
                                    improper randomization) /systematic review of level II RCT




       III                                                 Expert opinion




       IV                          High quality RCT with statistically significant difference or no
                                     statistically significant difference but narrow confidence
                                              interval/systematic review of level I RCT




       V                        Case control study, observational, retrospective comparative study/
                                                 systematic review of level III RCT
Station 22 answer (5)

                                                          Characterstics
Level of evidence




        I                         High quality RCT with statistically significant difference or no
                                    statistically significant difference but narrow confidence
                                             interval/systematic review of level I RCT

       II                          Lesser quality RCT ( eg less than 30% follow up; no blinding;
                                   improper randomization) /systematic review of level II RCT




       III                     Case control study, observational, retrospective comparative study/
                                                systematic review of level III RCT


       IV                                                   Case series




       V                                                  Expert opinion

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OSCE - Pune mock OSCE 2012

  • 2. • The OSCE Questions & Answers are prepared by the faculty for the benefit of Post graduate students and some are contributed from faulty • These Questions and answers are made for the students to understand the pattern of OSCE and not meant to completely cover the subject of Pediatrics. • Answer are checked from standard textbook in case of doubt plz email • ashwinborade@yahoo.com 11/7/2012 CME,PUNE 2
  • 3. Station 1 • A 10 year old boy brought to ER having consumed 5 tablets of Tricyclic Antidepressants tablets 2 hrs back. On examination he is drowsy, but arousable. HR 138/min. temp 100 F. RR 10/ min. BP 80/60 mmHg. • What are the clinical manifestations of tricyclic antidepressant poisoning? • What are the cardinal cardiovascular indicators of toxicity? • What is the poor prognostic factor? • What are the criteria for ECG diagnosis of poisoning ? • What is specific management and principle behind it?
  • 4. Answer station 1 • Anticholinergic toxidrome-delirium, mydriasis, dry mucus membranes, tachycardia, hyperthermia, mild hypertension, urinary retention, slow GI motility. CNS toxicity- lethargy, coma, myoclonic jerks, seizures. Sinus tachycardia-most imp; widening of QRS complex, PVC, ventricular arrhythmias .Refractory hypotension. • Sinus tachycardia; refractory hypotension; QRS duration of > 100 ms indicates risk for seizures & cardiac arrhythmias; R wave in lead aVR of > 3 mm is also an indicator of toxicity. • Refractory hypotension • QRS duration of > 100 ms indicates risk for seizures & cardiac arrhythmias; R wave in lead aVR of > 3 mm is also an indicator of toxicity • Alkalinisation with soda bicarb therapy (ANTIDOTE). Goal of bicarb therapy is to increase sodium concentration and arterial pH. Dose is 1-2 meq/kg until pH> 7.45. 150 meq/L of sodium bicarb diluted with 5% dextrose to maintain pH. Treat hypotension with normal saline; Vasopressors for hypotension; Airway management.
  • 5. Station 2 A 9 month old infant who was playing with marbles is suddenly found to the struggling to breathe. What would be your steps of intervention. • 1. Till the baby is conscious.(1) • 2. If the baby becomes unconscious. (1)
  • 6. Station 2B • Identify the ECG. [1] • Write three common causes of this problem. [1) • Write treatment of this problem [1 ]
  • 7. Answer Station 2 A 1 (a) 5 back blows between the scapula. (b) 5 chest thrusts on the lower 3rd of sternum. 2 (a) Give – 1. 2 rescue breaths 2. 5 back blows 3. 5 chest thrusts 4. Look into the mouth 5. If FB seen - remove it. 6. If not - give 2 breaths and continue the sequence.
  • 8. Answ 2 B • Atrial fibrillation • • Rheumatic mitral valve disease ,left AV valve insufficiency, thyrotoxicosis, pulmonary emboli, and pericarditis • • The best initial treatment is digitalization, which restores the ventricular rate to normal, although the atrial fibrillation usually persists.
  • 9. Station 3 : A 13 year old boy under investigation in OPD 1. What is the abnormality? (1) 2. What is the diagnosis? (1) 3. What clinical features are likely to be present in the child? (1) 4. . Name 2 condition with X linked Recessive inheritance. (1+1)
  • 10. Answer station3 1. Defect in the long arm of X chromosome(Fragile sites) (Fragile sites are regions of chromosomes that show a tendency for separation, breakage, or attenuation under particular growth conditions. The abnormality is caused by FMR1 gene expansion at Xp27.3 which is a fragile site) 2. Fragile X Syndrome 3. Long thin face Prominent jaw Macro orchidism Large ears Hyperkinetic behavior Mental retardation Large head Normal height 4. X linked Recessive Hemophilia Duchenne Muscular dystrophy Becker Muscular dystrophy
  • 11. Station4 A six week old infant is referred for poor weight gain and seizure. On examination, he looks cachexic and is dysmorphic with low set ears and a very small lower jaw. He also has severe thrush affecting the oral cavity and perineum. Cardiac apex is displaced into the left sixth intercostal space in anterior axillary line and there is a palpable thrill. A loud pan systolic murmur and an apical mid diastolic murmur can be heard. He is tachypneic but the lung fields are clear on auscultation. The liver is 3 cms below the costal margin in the mid clavicular line. Investigations: Na 136, K 4.9, Ca 1.7,Albumin 4.2, Urea 7.8, Hb 10.2, WBC 3.8, Platelet 2.45 L, Echo Large VSD • What is the diagnosis?(1) • What is commonest presentation during newborn period. (1) • Most likely explanation for the low plasma calcium ?(1) • What is the causes of immunodeficiency? (1) • The etiopathogensis of all these features is ? (1)
  • 12. Answer 4 1. DieGeorge syndrome 2. Hypocalcemic seizures 3. Primary hypoparathyroidism 4. Thymus gland aplasia leading to abnormal cell mediated immunity 5. An embryological defect of the third and fourth pharyngeal pouches
  • 13. Station5 15 months old child admitted with anemia with hepatosplenomegaly for evaluation, PS shows. 1.Identify and describe PBS findings. (1) 2.What is the diagnosis? (1) 3.What is definitive treatment? (1) 4.What are clinical features seen in classical case ? (1) 5.What is the basic etiology for this disorder ? (1)
  • 14. Answer 5 1. PBS showing microcytic, hypochromic anemia with nucleated RBC, tear drops cells 2. Thalassemia major 3. BMT 4.Thalasemmic facies, Hs megaly, bone fractures 5. Ineffective erythropoisis
  • 15. Station 6A Identify the slide.(1) What is treatment?(1)
  • 16. Station 6 B State True or False- 1. The following are useful in assessing the gestational age of an infant- (0.2 x5) a) Posture T/F b) Palmar creases T/F c) Elbow angle T/F d) Square window test T/F e) Nipple formation T/F 2. Blood flow in the fetus- (0.2 x5) a) Blood flows from right to left through foramen ovale- T/F b) Blood in the ascending aorta has higher oxygen content than the blood in descending aorta- T/F c) The ductus arteriosus is closed T/F d) Pulmonary pressure equals systemic pressure T/F e) Hemoglobin may be 20 g/dl T/F 3. Genetic anticipation occurs in the following conditions- (0.2 x5) a) Cystic fibrosis T/F b) Myotonic dystrophy T/F c) Fragile X Syndrome T/F d) Huntington’s chorea T/F e) Marfan’s Syndrome T/F
  • 17. Answer : 6A : a) Egg of Ascaris Lumbricoides b) Albendazole 400 mg or Mebendazole 100 mg BD x 3 days 6B: 1. The following are useful in assessing the gestational age of an infant- a) True b)False c) False d) True e) True 2. Blood flow in the fetus- a) True b) True c) False d) False e) True 3. Genetic anticipation occurs in the following conditions- a) False b) True c) True d) True e) False
  • 18. Station 7 A 15 yrs old child with no significant past history comes in OPD with slow myoclonic seizures since 2 days. Also parents noticed he had subtle personality changes and poor school performance .His EEG done What are the EEG changes? (1) What is the diagnosis?(1) What is diagnostic criteria (1) What is etiology for same ?(1) What are CNS complications?(1)
  • 19. Answer 7 • High voltage burst slow waves with normal background • SSPE • Measles ab in CSF/EEG/histological finding on biopsy • Persistent measles viral infection in CNS • Hypertonia, bulbar palsy,choreatheosis,decerebrate posturing
  • 20. • Station 8 A A. What test is performed ?(1/2) B. What are indications? (1/2) C. What 2 important parameters it measures? (1/2) D. Based on these result how can you differentiate disorders ? (1) • Station 8 B A. What is the elemental content of 1 ml ?(2) B. Which solution is incompatible if mixed with this drug?(1) C. Give 2 indications for its use (2)
  • 21. Answer 8 A A Spirometry B To differentiate obstructive n restrictive respiratory diseases. C FVC and FeV1 D FVC,FEV1 both low- restrictive FVC-N FEV1 Low - obstructive FVC low FEV1 very low- mixed diseases . B A. Ca Gluconate 10% (1 ml = 9 mg elemental Ca) B. Do not mix with HCO3 C. Bradycardia, Asystole and Sclerosis
  • 22. Station 9 Write the specific odour of urine in following condition (10x0.5=5 ) 1.Glutaric acedemia mousy 2. Hawkinswria tomcat urine 3. 3-hydroxy 3 methyglutaric acidemia maple syrup 4. Isovaluric acedemia cat urine 5. Maple syrup urine diseses sweaty feet 6. Hypermethioninemia rancid butter /boiled cabbage 7. Multiple carboxylase defiency , boiled cabbage 8. Phenyketnuria swimming pool 9. Trimethylamuria rotting fish 10. Tyrosiemia sweaty feet
  • 23. Answer Station 9 The specific odour of urine in following condition • Glutaric acedemia –sweaty feet • Hawkinswria,-swimming pool • 3-hydroxy 3 methyglutaric acidemia,-cat urine • isovaluric acedemia,-sweaty feet • maple syrup urine diseases –maple syrup • hypermethioninemia,-boiled cabbage • multiple carboxylase defiency ,-tomcat urine • Phenyketnuria-mousy • Trimethylamuria,-rotting fish • Tyrosiemia-rancid butter /boiled cabbage
  • 24. Station 10 A 2 yrs old child admitted with anemia , hepatosplenomegaly and fever. PBS shows : • What is the diagnosis?(1) • What are complications?(1) • What is late treatment failure in this case.(1)
  • 25. Station 10 B 1. What is the radiological diagnosis? ( 1) 2. Name 2 risk factors for the development of this condition (1/2 +1/2)
  • 26. Answer 10 A • Falciparum malaria • Severe anemia, cerebral malaria, respiratory distress, hypoglycemia • Late treatment failure: Patient will exhibit late treatment failure (LTF) under following situations: • (i) Development of danger sign or severe malaria on any day between day 4 and day 14 in presence of parasitemia. • (ii) Axillary temperature >37.5ºC in presence of parasitemia on any day from day 4 to day 14.
  • 27. Answer 10 B 1. Right & Left pneumothorax with left upper zone haziness 2.Risk Factors- • Assisted ventilation (including CPAP) • MAS,RDS • Other Air Leak Syndromes (e.g. PIE) • Pulmonary hypoplasia • Idiopathic or spontaneous
  • 28. Station 11 • A:Write content of the following in Human milk fortifier (1/4X 8=2) 1. Protein 2. Fat 3. Sodium 4. Calcium 5. Vit D 6. Thiamine 7. Vit B12 8. Zinc • B:Write biological value, net protein utilization and protein efficacy ratio of – 1.Meat 2. Egg 3. Bengal gram 4. Wheat (12 X 0.25=3 )
  • 29. Answer 11A PROTEIN 0.2g Fat 0.1g NA 1.5 calcium 50 Vit D 250 Thiamine 12 B12 0.05 Zinc 0.18
  • 30. Answer11B BIOLOGICAL NPU PER VALUE Meat 74 76 3.2 EGG 96 96 3.8 Bengal gram 74 61 1.3 WHEAT 66 61 1.3
  • 31. Station 12 • Identify diagram .(1) • What are the uses? (1) • Name each components A-F along with interpretation of each (1/4 x 6=3)
  • 32. Answer 12 • Scatter diagram and correlations. • A scatter diagram is a tool for analyzing relationships between two variables. One variable is plotted on the horizontal axis and the other is plotted on the vertical axis. The pattern of their intersecting points can graphically show relationship patterns. • A;No Correlation There is no demonstrated connection between the two variables • B;Weak Positive Correlation The value of Y increases slightly as the value of X increases • C;Strong Positive Correlation The value of Y clearly increases as the value of X increases. • D;Weak Negative Correlation The value of Y decreases slightly as the value of X increases • E;Strong Negative Correlation The value of Y clearly decreases as the value of X increases. • .F; Perfect Correlation The value of Y seems to be related to the value of X.
  • 33. Station 13 • 2 yrs old girl brought with pubic hair and clitoromegaly, increased irritability gradually increasing since the past 3 months. No history of drug ingestion or hyperpigmentation. • On examination she has BMI above the 95 th centile. BP:112/80 mm of Hg. HR:92/min & RR:24/min. She has hirsutism and few acneform eruptions on the face. Her cheeks are chubby & few pink striae present over the face and abdomen. Genital examination shows pubic hair stage 2 & clitorus measuring 1.8 cms. She is extremely irritable. Systemic examination is normal. • What is the diagnosis? (1) • What is most likely etiology at this age? (1) • Enlist 2 important screening tests used for the above condition? (1+1) • Name 2 drugs used for the medical management of this condition? (1/2+ 1/2)
  • 34. Answer 13 • Cushings syndrome • Etiology: Below age of 7 yrs: ACTH independent autonomous adrenal lesion like Adrenal adenoma or carcinoma • Screening tests: Assessment of diurnal cortisol rhythm Overnight dexamethasone supression test 24 hour urinary free cortisol Salivary cortisol • Drugs that can be used: Ketoconazole Aminoglutethimide Cyproheptidine Metyrapone Mitotane
  • 35. Station 14 6 yr old boy brought with the deformity shown in the figure. His sister, 8 yrs also has a similar deformity gradually increasing since 3 yrs of age. On examination his height is below the third percentile & US/LS ratio is 1.6:1. There are multiple dental abscesses. Mother also has a similar deformity but very mild in nature. S. calcium : 8.8 mg/dl, Phosphorus : 1.8 mg/dl Alk phosphatase: 380 IU PTH levels: 18 pg/ml (9-55 pg/ml) 25-OH Cholecalciferol: 26 ng/ml(10-50 ng/ml) 1,25(OH)2Cholecalciferol: 18 pg/ml (20-60 pg/ml) TMPO4/GFR: reduced. X-ray of both lower limb shows florid richetic changes • What is the Diagnosis? (1) • What is the inheritance ?(1) • It is due to mutation of which gene?( 1) • What is the Treatment?(1) • Write 4 endocrinal causes of short stature. (1)
  • 36. Ans 14 • Hypophosphatemic Rickets • X-linked dominant • Mutation in PHEX gene • Treatment: Phosphorus supplement orally- 1-4 g/day QDS Alfacalcidol- 25-50 ng/kg/day or calcitriol 0.25-2 mcg/day. • 4 causes- 1.Pan hypopitutarisum 2.Isolated GHD 3.Hypothyrodisum 4.Cushing syndrome 5.DM
  • 37. Station 15 Our patient is a 17 yr old female with history of recurrent seizures. Admitted in PICU with stroke. She was evaluated for same. • Identify and describe the investigation? (1/2+1/2) • What is the diagnosis? (1) • What other disorder are associated with this disease? (1) • What is the treatment of choice? (1) • What are syndromes associated with contralateral hemiplgia in children? (1)
  • 38. Answer 15 1. a)MRI T1 weighted images show extensive collateralization of vessels in region of circle of willis. b) MRA reveals reduced luminal calibre of left internal carotid artery in petrous, cavernous and supraclinoid segments. 2. Moya moya disease. 3. Down syndrome, neurofibromatosis, or sickle cell disease 4. Direct superficial temporal artery (STA) to middle cerebral artery (MCA) bypass is considered the treatment of choice. 5. Weber syndrome, Benedict syndrome ,millard gublar syndrome.
  • 39. Station 16 A • Write RNTCP cat IV regimen (1)
  • 40. Station 16 B : What is the graph? (1) What are its uses? (1) How do you use it? (1) What are the other charts used for this purpose? (1)
  • 41. Answer 16 A 6(9) Kmofx(Lvx) Eto Cs Z E/ 18 ofx (Lvx) Eto Cs E Answer 16 B • Trivandrum development screening chart • Development assessment upto 24 months • SAT hospital, TVM • A vertical line is drawn or pencil is kept vertically at level age at child being tested ,if child fails to achieve any item that falls short on left side of vertical line child had developmental delay • DDST,DST,BSID
  • 42. Station 17 ( 5 marks) Write the laboratory values for following disorders ( either N, calcium phosphorus PTH 25(OH)2D3 1,25(OH)2 D3 Vit D def. VDDR type 2 CRF ADHR Fanconi syndrome
  • 43. Answer 17 calcium phosphorus PTH 25(OH)2D3 1,25(OH)2 D3 Vit D def. N / dec dec incre dec Dec/N/Inc VDDR type 2 N/dec dec incres N incre CRF N/dec incre incre N dec ADHR N dec N N Rd Fanconi N dec N N Rd or incre syndrome
  • 44. Station 18 2 yrs old child with MR admitted for evaluation , On examination he had anemia with organomegaly. Lab evaluation showed anemia with thrombocytopenia .BM done . 1.What is the diagnosis? 2.What are the marrow findings? 3.What is the etiology for such findings? 4.What are the typical radiological findings? 5.What is the definitive treatment? (1x5=5)
  • 45. Answer 18 • Gaucher disease . • Wrinkled paper cells in BM • Intracytoplsmic substrate inclusion • Erlenmeyer flask deformity in distal femur • Enzyme replacement with b glucosidase
  • 46. Station 19 (1X5=5) 1.How will you immunize a child with bleeding disorder? (1) 2.Name the vaccines which can be placed in the freezer compartment of the refrigerator (1) 3.Write down the time limits for using the following vaccines after reconstitution- • Varicella (1) • Measles/MMR (1) 4. Write the schedule of rabies vaccine for a person, who has been bitten by a dog but has received 5 doses of rabies vaccine earlier. (1)
  • 47. Answer 19 1.Subcutaneous route should be used (Unless contraindicated) For aluminium adjuvanted vaccines that can only be given intramuscularly, vaccination should be scheduled after factor replacement therapy Needles <23G should be used for injection and the parents should be asked to apply firm and sustained pressure, without rubbing, for at least 5 minutes. 2.Freezer compartment: BCG, OPV, Measles, and MMR. 3. Varicella = 30 min (and protect from light) Measles/MMR = 4 to 6 hours 4. Two doses are given on days 0 and 3. (For re exposure at any point of time after completed (and documented) pre or post exposure prophylaxis)
  • 48. Station 20 Interpret the following ABG reports: 1. pH-7.6/ PaCo2-25/ Pao2-160/ HCo3-24 (Fio2-50%) Calculate AaDO2 and write the formula (1+ 1/2) 2. pH-7.38/ PaCo2-65/ Pao2-48/ HCo3-34 (Fio2-60%) (a) what is the ABG diagnosis (1) (b) What is normal PaO2 level expected if a child is breathing at room air with normal lungs? (1) 3. pH-7.45/ PaCo2-40/ Pao2-120/ HCo3-28.5/ Spo2 99%/ Hg 8 gm% Calculate Oxygen content in given blood gas (1+1/2)
  • 49. Answer 20 1. 165.25 (Formula) AaDO2 = (713 x FiO2) – (pCO2 / 0.8) – (paO2) 2. a)Respiratory acidosis with metabolic compensation b) 80-100 mmHg 3. 11 ml O2 /dl Arterial Oxygen content = (Hb x 1.36 x SpO2 ) + ( 0.0031 x PaO2)
  • 50. Station 21 : Which materials are used to sterilize the given items- (1X5=5) • Floor • Ambu bag • Chittle forceps • Probe of pulse oximeter and Weighing scale • Laryngoscope
  • 51. Answer 21 • FLOOR : By Phenol • Ambu bag : after dismantling clean with soapimmearse in Cidex for 4-6 hrs rinse with water dry,wrap and put a date • Cheattle forceps : autoclave • Probe of Pulse oxymeter : by spirit • Weighing machine : 2% Bacillocid • Laryngoscope : by spirit. If used for infected patient than wash with soap & water -- after removing bulb,put blade in 2% cidex  wash, dry and wrap.
  • 52. Station 22 Match the following (5) Characterstics Level of evidence I Case series II Lesser quality RCT ( eg less than 30% follow up; no blinding; improper randomization) /systematic review of level II RCT III Expert opinion IV High quality RCT with statistically significant difference or no statistically significant difference but narrow confidence interval/systematic review of level I RCT V Case control study, observational, retrospective comparative study/ systematic review of level III RCT
  • 53. Station 22 answer (5) Characterstics Level of evidence I High quality RCT with statistically significant difference or no statistically significant difference but narrow confidence interval/systematic review of level I RCT II Lesser quality RCT ( eg less than 30% follow up; no blinding; improper randomization) /systematic review of level II RCT III Case control study, observational, retrospective comparative study/ systematic review of level III RCT IV Case series V Expert opinion