MCQ pediatrics

JABER AHMED ALENAZI

APRIL 17 ,2006

1-All are essential amino acid except :

A-tryptophan

B-theronine

C-taurin

D-tyrosin

E-all of the above

1-All are essential amino acid except :

A-tryptophan

B-theronine

C-taurin

D-tyrosin

E-all of the above

A.A= 24 (9 essentials +15)For preterm +3

ArgininCystinTaurin

TMHL PTLIV CAT T tryptophan M methionin H histidin L lycin P phenylalan T threonin L leucin I isoleucin V valin C cystin A argnine T taurin

2-In which of the following forms of rickets, PTH hormone normal before initiation of treatment :

A -Ca deficiency B-vit. D def. C-25-hydroxylase def. D-1α hydroxylase def. E-X-linked hypophosphatemia

2-In which of the following forms of rickets, PTH hormone normal before initiation of treatment :

A -Ca deficiency B-vit. D def. C-25-hydroxylase def. D-1α hydroxylase def. E-X-linked hypophosphatemia

3-Non-ketotic hypoglycemia is usually a feature in except:

A-focal adenomatosis of the pancreas

B-KATP –channal hyperinsulinism

C-fatty acid oxidation disorder

D-GSD –glucose 6 phosphate defi.

E-exogenous insulin inj.

3-Non-ketotic hypoglycemia is usually a feature in except:

A-focal adenomatosis of the pancreas

B-KATP –channal hyperinsulinism

C-fatty acid oxidation disorder

D-GSD –glucose 6 phosphate defi.

E-exogenous insulin inj.

4- 2 y old boy presented with recurrent necrotizing skin ulcers ,remain febrile with poor wound healing ,CBC showed marked leukocytosis, his clinical feature suggest diagnosis:

A-shwachman-Diamond syndrome B-CGD C-LAD D-kostmann disease E-agammaglobulinemia

4- 2 y old boy presented with recurrent necrotizing skin ulcers ,remain febrile with poor wound healing ,CBC showed marked leukocytosis, his clinical feature suggest diagnosis:

A-shwachman-Diamond syndrome B-CGD C-LAD D-kostmann disease E-agammaglobulinemia

shwachman-Diamond syndrome congenital neutropenia with dwarfism and FTT,AR inheritance

kostmann disease maturation arrest of leukocyte at promylocyte level ,AD.

5-Ambiguous genitialia infant with hypoglycemia,hyponatremia and hyperkalemia with keryotype (46YX) most likely diagnosis:

A-21-hydroxlyase deficiency

B-11-hydroxlyase deficiency

C-3β-hydrosteroid dehydrogenas deficiency

D-5α-reductase deficiency

E-17-hydroxlylase deficiency

5-Ambiguous genitialia infant with hypoglycemia,hyponatremia and hyperkalemia with keryotype (46YX) most likely diagnosis:

A-21-hydroxlyase deficiency

B-11-hydroxlyase deficiency

C-3β-hydrosteroid dehydrogenas deficiency

D-5α-reductase deficiency

E-17-hydroxlylase deficiency

6-the following are modalities in turner syndrome except

A-GH B-Estrogen C-Anabolic steroid D-Gonadectomy E-All of the above

6-the following are modalities in turner syndrome except

A-GH B-Estrogen C-Anabolic steroid D-Gonadectomy E-All of the above

7-normal or elevated 25-hydroxly D3 with normal or depressed 1,25 VD3 in:

A-Nutritinal rickets

B-V-D dependent rickets- I

C-V-D dependent rickets- II

D-X hypophosphatemic rickets

E- B+D

7-normal or elevated 25-hydroxly D3 with normal or depressed 1,25 VD3 in:

A-Nutritinal rickets

B-V-D dependent rickets- I

C-V-D dependent rickets- II

D-X hypophosphatemic rickets

E- B+D

8-Following infants are at increased risk of neonatal hypocalcemia except:

A-IDM

B-osteogenesis imperfeca

C-neonate with septicemia

D-asphyxiated babies

E-infant of mother with hyperPTH

8-Following infants are at increased risk of neonatal hypocalcemia except:

A-IDM

B-osteogenesis imperfeca

C-neonate with septicemia

D-asphyxiated babies

E-infant of mother with hyperPTH

9-In addition to irritability, sweating and difficulty breathing with feeding the symptom that mostly indicative of CHF in a 3 wk old infant is:

A-ascitis

B-raised JVP

C-cynosis

D-diminished feeding volume

E-peritibial odema

9-In addition to irritability, sweating and difficulty breathing with feeding the symptom that mostly indicative of CHF in a 3 wk old infant is:

A-ascitis

B-raised JVP

C-cynosis

D-diminished feeding volume

E-peritibial odema

10-Asymptomatic 2 wk old baby is known to have III ESM with mild cynosis, at 3 mo of age suddenly develops irritability ,resp. difficulty. O/E marked cyanosis, tachypnea hyperapnea & barely audible short ESM I most likely diagnosis

A-sever aortic stenosis

B-sever CoA

C-sever valvular PS

D-TOF

E-truncus arteriosus

10-Asymptomatic 2 wk old baby is known to have III ESM with mild cyanosis, at 3 mo of age suddenly develops irritability ,resp. difficulty. O/E marked cyanosis, tachypnea ,hyperapnea & barely audible short ESM I most likely diagnosis

A-sever aortic stenosis

B-sever CoA

C-sever valvular PS

D-TOF

E-truncus arteriosus

11-Faint M. in this pt indicate:

A-non-restrictive VSD

B-minimal flow across RVOT flow

C-poor ventricular function

D-air trapping in lung

E-hypoplastic pulmonary arteries

11-Faint M. in this pt indicate:

A-non-restrictive VSD

B-minimal flow across RVOT flow

C-poor ventricular function

D-air trapping in lung

E-hypoplastic pulmonary arteries

12-In managing this infant ,least beneficial intervention is :

A-IV hydration

B-sedation

C-intropic support

D-surgery

E-correcting acidosis

12-In managing this infant ,least beneficial intervention is :

A-IV hydration

B-sedation

C-intropic support

D-surgery

E-correcting acidosis

13-most common cyanotic CHD in neonatal period :

A-TOF

B-TGA

C-TAPVR

D-Truncus arteriosus

E-TA

13-most common cyanotic CHD in neonatal period :

A-TOF

B-TGA

C-TAPVR

D-Truncus arteriosus

E-TA

14-after being healthy 2 wk infant present with few days of poor feeding ,followed by hypotension, poor central & peripheral pulses and sever metabolic acidosis of the following the BEST intervention to produce improvement will be:

A-100% O2

B-Dopamine infusion

C-gamma globulin infusion

D-phenylephrine infusion

E-PGE1

14-after being healthy 2 wk infant present with few days of poor feeding ,followed by hypotension, poor central & peripheral pulses and sever metabolic acidosis of the following the BEST intervention to produce improvement will be:

A-100% O2

B-Dopamine infusion

C-gamma globulin infusion

D-phenylephrine infusion

E-PGE1

15-Collapsing pulse (water hammer) sign of the following except:

A-tricuspid regurgitation

B-large PDA

C-anemia

D-hyperthyroidism

E-Aortic regurgitation

15-Collapsing pulse (water hammer) sign of the following except:

A-tricuspid regurgitation

B-large PDA

C-anemia

D-hyperthyroidism

E-Aortic regurgitation

16-idiosyncratic fulminant liver failure is rare but potentially fatal complication of the following AED s :

A-phenytoin B-Phenobarbital C- carbamazepine D-valoproic acid E-Ethosuximide

16-idiosyncratic fulminant liver failure is rare but potentially fatal complication of the following AED s :

A-phenytoin B-Phenobarbital C- carbamazepine D-valoproic acid E-Ethosuximide

17-most common causes of blood in stool of newborn infant is:

A-Vit-k deficiency

B-Ingested maternal blood

C-Infectious diarrhea

D-NEC

E-Hischsprung enterocolitis

17-most common causes of blood in stool of newborn infant is:

A-Vit-k deficiency

B-Ingested maternal blood

C-Infectious diarrhea

D-NEC

E-Hischsprung enterocolitis

18-most usefull test in diagnosis GER in 3y old boy with mod-sever asthma :

A-upper GI study

B-Esophageal manometry

C-Esophageal pH monitoring

D-Endoscopy

E- Gastric scintography

18-most usefull test in diagnosis GER in 3y old boy with mod-sever asthma :

A-upper GI study

B-Esophageal manometry

C-Esophageal pH monitoring

D-Endoscopy

E- Gastric scintography

19- Most definitive test in diagnosis of Wilson disease is:

A- serum ceruloplasmin

B-24 h- urine for copper

C-Brain MRI

D- Hepatic copper

E- Liver histology

19- Most definitive test in diagnosis of Wilson disease is:

A- serum ceruloplasmin

B-24 h- urine for copper

C-Brain MRI

D- Hepatic copper

E- Liver histology

20-commonest cause of chronic diarrhea of 6mo -36 mo in Saudi Arabia :

A-toddler diarrhea

B-Disacchariase deficiency

C-Giardiasis

D-congenital chlorid diarrhea

E-All of the above

20-commonest cause of chronic diarrhea of 6mo -36 mo in Saudi Arabia :

A-toddler diarrhea

B-Disacchariase deficiency

C-Giardiasis

D-congenital chlorid diarrhea

E-All of the above

21-One of the following signs / symptoms consistence with functional abdominal pain:

A-vomiting B-dysuria C-headache D-pallor E-weight loss

21-One of the following signs / symptoms consistence with functional abdominal pain:

A-vomiting B-dysuria C-headache D-pallor E-weight loss

22- one of the following infecion cause increased amylase level :

A-bacterial meningitis

B-viral meningitis

C-Giardiasis

D-mumps

E-measles

22- one of the following infections cause increased amylase level :

A-bacterial meningitis

B-viral meningitis

C-Giardiasis

D-mumps

E-measles

23-neoborn developed cyanosis at age of 6 days. ECG showed LAD most likely diagnosis:

A-TGA B-TOF C-TAPVR D-TA E-HLHS

23-neoborn developed cyanosis at age of 6 days. ECG showed LAD most likely diagnosis:

A-TGA B-TOF C-TAPVR D-TA E-HLHS

LADASD primum AVSD superior axisTA

24-5y old boy presents with his third episoid of painful Cx lymphadenitis need I & D which grew staph. Aureus. at age of 2y required aspiration of a liver abscess moat important lab:

A-PCR for ADA deficiency B-nitroblue tetrazolium C-MAC-1 assay D-neutrophil count E-bone marrow aspiration

24-5y old boy presents with his third episode of painful Cx lymphadenitis need I & D which grew staph. Aureus. at age of 2y required aspiration of a liver abscess moat important lab:

A-PCR for ADA deficiency B-nitroblue tetrazolium C-MAC-1 assay D-neutrophil count E-bone marrow aspiration

Catalase +ve organism Staph / streptPsaudomonusKlebsilla sppSerrchia sppAspergila candida

25-7mo old boy with diffuse seborrhic –like lesion since birth with FTT and chronic diarrhea ,no organomegaly nor TCP in CBC most likely diagnosis:

A-Wiskott –Aldrich syndrome

B-Histocytosis X

C-Omenn syndrome

D- Leiner disease

E-non of the above

25-7mo old boy with diffuse seborrhic –like lesion since birth with FTT and chronic diarrhea ,no organomegaly nor TCP in CBC most likely diagnosis:

A-Wiskott –Aldrich syndrome

B-Histocytosis X

C-Omenn syndrome

D- Leiner disease

E-non of the above

Leiner disease seborric-like dermatitis chronic diarrheaa, FTT.

Heiner syndrome primary pulmonary heamosidrosis

Meinear disease acute virtigo ,deafness ana headache in adult mainly vestibulo-coccoluar system

26-most importtant indication for splenectomy in thalassemic pt nowadys is

A-massive enlargement of spleen which cause mechanical discomfort

B-TCP,leukopenia and neutropenia together in one pt

C-Tx requirement of more than 100 ml/kg/y D-splenic sequestration E-progressive increase in Tx requirement

26-most importtant indication for splenectomy in thalassemic pt nowadys is

A-massive enlargement of spleen which cause mechanical discomfort

B-TCP,leukopenia and neutropenia together in one pt

C-Tx requirement of more than 100 ml/kg/y D-splenic sequestration E-progressive increase in Tx requirement

27-in DIC earliest abnormality appear:

A-prolonged PT

B-prolonged PTT

C-increased FDP

D-decreased platelet count

E-fragmentation of RBCs

27-in DIC earliest abnormality appear:

A-prolonged PT

B-prolonged PTT

C-increased FDP

D-decreased platelet count

E-fragmentation of RBCs

Low plat.

PT& aPTT prolongedDDx

DIC, hypoxiaLab

Thrombin timeFibrin split products

Microangiopathic on smearLow fibrin

Positive D-dimer

PT&aPTT nlDDx of TCP

Normal plat

aPTT prolonged aPTT normal

PT normal PT normalPT prolonged PT prolonged

FXIII deficiency Plat dysf.

Von willebrad’s diseaseA2antiplasmin deficiency

Plasminogen activator inhibitor def.

Plt dysf.Lab test:

fXIII assay Bleeding timeA2-antiplsminPAI 1 inhibitor

FII def.FVII def

Early vitD def.Lab test:FII assay

FVII assay

FVIII def.FIX def.FXI defFXII def.VWDHeparin Rx.lab testFVIII,IX,XI,XII assayBT

Give vitD/repeat PT &aPTT in 4H

PT &aPTTNormalDDx vit D def

Bleeding doesn't stopFV def.FX def.Liver diseaseFibrinogen def.Lab test:FV,X,fibrino-gen assay

28-radio opaque renal stone :

A-uric acid

B-xanthine

C-calcium oxylate

D-cystine

E-All of the above

28-radio opaque renal stone :

A-uric acid

B-xanthine

C-calcium oxylate

D-cystine

E-All of the above

29-most common glumerular histopathology in SCD:

A-FSGN B-MPGN C-MCNS D-membranous nephropathy E-diffuse proleferative GN

29-most common glumerular histopathology in SCD:

A-FSGN B-MPGN C-MCNS D-membranous nephropathy E-diffuse proleferative GN

30-regaarding neurodegenerative disease affecting white matter disease commonly present with these Sx except:

A-loss of motor skills B-seizure C-spasticity D-ataxia E-blindness

30-regaarding neurodegenerative disease affecting white matter disease commonly present with these Sx except:

A-loss of motor skills B-seizure C-spasticity D-ataxia E-blindness