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1-the most common form of familial mental retardation: 1-neurofibromatosis 2-tuber sclerosis 3-fragile x syndrome 4-down syndrome 2-at what age child can drinks from cup 1-6mon 2-9mon 3-12mon 4-15mon 3-the most common cause of FTT : 1-ifectios diseases 2-metabolic disease 3-mental retardation 4-enviromental 4-the adequacy of milk intake in infant assessed by:1-times of feeding 2-weight gain 3-stooling pattern 4-color of urine 5-goat milk assosiated with def of 1-iron 2-ca 3-folic acid 4-essential fa 6-indications for nutramigen milk are except: 1-maldigestion 2-cows milk allergy 3-soy protein allergy 4-vegeterian diet 7-all are contraindication for breast milk except: 1-herpes12-galactosemia 3-hiv positive 4-HBVsag 8-hypervitaminosis A assosiated with 1-polyuria 2-increase intra cranial pressure 3-hepatotoxicity 4-renal failure 6 و5 ضيف خيار 5-2+3 6-1+2 9-hypervitaminosis C associated with: 1-oxalate stone 2-sensory neuropathy 3-motor neuropathy 3-iron def anemia 10- hyperaceusis associated with except: 1-tay sachs 2-krrabe dz 3-vit b6 def 4-causher ds 11-beri beri all tru except: 1-heart failure2-thiamine def 3- aphonic cry 4- high pitch crying 12-all cause hypokalemia except: 1-proximal RTA 2-cystic fibrosis 3-barrter syndrome 4-urinary tract obstruction 13-all cause hyperkalemia except : 1-tumor lysis syndrome 2-renovascular dz 3-thrombocytosis 4-adrenoleukodystrophy 14-match drugs with antidote 1-diazepam b 2-ca chanel bloker f 3-cynide h 4-methemoglubin d 5-opiate a 6-paracetamol e 7- cyclic antidepressant c A-naloxon B-flumazenil C-sodium bicar D-mythelene blu E-n acytl cystein F- glucagone G- o2 H-nitrates 15-all are features of neurofibromatosis 1 except: 1-axillary freckling 2-lisch nodule 3-café au lait spot 4-brushfeild nodule 16-spina bifida inherited as: 1-ar 2-ad 3-x link 4-multifactorial 17-parder willi syndrome all except: 1- obesity 2-hypotonia 3-hyper gonadotropic hypogonadism4-hypogonadotropic hypogonadism 18-all cause cataract except: 1-down 2-rubella 3-tay sachs 4-galactosemia 19-in down all true except 1-endocardial cusion 2-deudenal atresia 3- hypo tonia 4-coloboma 5-cataract 20-charge syndrome all true except 1-cardiac 2-chonal atresia 3-coloboma 4-anal atresia 5-retardation 21-coarctation of aorta all true except: 1-turner association 2- bicusbed aortic valve 3-intracranial aneurysm 4-murmur atrt intrascapular area 5-3 sign at x ray 22-foam cells at bone marrow is pathognomic for: 1-tay sachs 2-nimmin pick dz 3-hurler 4-caucher 23-all cause cherry red spot except: 1-causher 2-tay sacs 3-nimmin pick 4-siliadiosis 24-match odor with dz1-musty 2-cabbag 3-sweety 4-sweaty 5-cat 6-galric ((ثوم 7-wintergreen A-tyrosinemia 2 B-glutaric aciduria ii C-maple syrup 3 D-multiple carboxylase def E- pku 1 F-organophosphate 6 4 5 25-macrocytic anemia +pancreatic insufficiency 1-alpers syndrome 2-pearson syn 3-leigh dz 4-diamond blackfan 26-fetal pulmonary maturity assessed by: Sphingomylin/licithine ratio of 2/1 2-s/l ratio of 1/2 3-s/l 1/1 4-non 27-potter syndrome all true except: 1-oligohydromins 2-lung hypoplasia 3-polyhydrominos 4-renal agenesis 28-poland syndrome : 1-ambiguous genitalia 2-abcsense of chest muscle pectorilis major 3- absence of back muscle 4-absence spleen : 29-prune belly syndrome all true except: 1-absence of abdominal muscle 2-aspleenia 3-undesended testes 4-renal abnormality 30-all cause neonatal hypoca except1-william syn 2-digeorge synd 3-maternal diabetes 4-maternal hyperparathyroid 31-what antibodes cross placenta in maternal sle: 1-ana 2-anca 3-antiro anti la 4-anti smith 32-the teratogenic effect of lithium is : 1-spina bifida2-ebstein anomaly 3-aortic stenosis 4-deafness 33-match ds with the most common organism cause sepsis1-galactosemia a 2-iron toxicity c 3-ig m def f 4-nephrotic syn b 5-late hypocomplement d 6-chronic granulomatous dz e A-e coli B-s.pneumonia C-yersenia D-meningiococcus E-staph F-whipple bacteria 34-match ds with radio finding 1-hmd f 2-cystic fibrosis f meconium ilius 3-deudenal atresia k 4-pyloric stenosis g on barium 5-necrotising enterocolitis m 6-bronchopulmonary dysplasia i 7-transpositin of great vessels h 8-ttn e 9-tetralogy of fallot d 10-epiglositis j 11-croup b 12-staph a 13-rt side aortic arch c 14-aortic coarctation l 15-tuber sclerosis o 16struge weber synd n A-pneumatocele B-steeple sign C-vascular ring D-boot shepe heart E-fliud in lung fissure F-ground glass G-string sign H-narrow waist heart I-sponge appearance J-thumb sign K-double bubble L-3 sign M-pneumatosis intestinalis N-intercortical calsification O-periventricular calcification 35-match dz with skin lesion 1-fanconi anemia i 2-tuber sclerosis e 3-sruge weber syndrome f 4-lyme dz k 5-coxaci virus typea d 6-still dz a 7-mycoplasma pneumonia h 8-rheumatic fever g 9-seborrheic dermatitis m 10-herpes simplex j 11-celiac dz b 12-vit a def c 13-zinc def l A-salamon morbilliform rash B-dermatitis herptiform C-hyperkeratosis D-mouth hand foot rash E-ash leaf spot F-port wine stain G-erythema marginatum H-steven jonson syndrome I-café au lait spot J-erythema multiformma minor K-erythema migrans L-acrodermatitis enteropathica M-cradle cap 36-all are manifestation of diabetic mother on neonates except 1-polycythemia 2-direct hyper bilirubinemia 3-hypocalcemia 4-hpoglycemia 37-the most common type of apnea of prematurity: 1-obstructive 2-mixed 3-central 4-sleep apnea 38-all cause pulmonary hypoplasia except 1-hydrops fetalis 2-potter syndrome 3-werdnig-hoffmann syndrome 4-diaphragmatic hernia none -تعديل السؤال بدل تيرنر 5-none 39-all cause anemia in neoborn except: 1-b thalasemia 2-diamond blackfan syndrome 3-osteopetrosis 4-g6pd def 40-all are true for physiological jaundice except: 1-occurs after 24 h of life 2-the level is less than 13-15mg/dl 3-increase more than 5mg/day 4-pe is normal 41 -all are at risk for polycythemia in neoborn except: 1-diabetic mother 2-adrenogenital syndrome 3-neonatal hypothyroidism 4-beck- withweidman syndrome 5-down syndrome 42-the most common cause of neonatal thrombocytopnea is: 1-congenital 2-isoimmune 3-wiskott aldrich syndrome 4-itp 43-anemia and thrompcytopnea due to hemangioma is called: 1-men syndrome 2-soto syndrome 3-kasabach merritt syndrome 4-diamond blackfan 44-all are risk factors for nec except: 1-breast milk 2-prematurity 3-ischemia 4-formula 45-the most common congenital infection : 1-rubella 2-herpes simp 3-cmv 4-varceilla 46-hutchinson triad (keratitis,8th cn palsy, hutchinson teeth) in: 1-toxoplasmosis 2-syphlis 3-parvovirus 4-n gonnorhea 47-thrtombocytopnea+eczema+immune def : 1-scwaman-dimoind syndrome 2-wiskot aldrich syn 3-kartenger 4-folic acid def 48-hypocalcemia+immune def+interrupted aortic arch: -1-scwaman-dimoind syndrome 2-wiskot aldrich syn 3-kartenger 4-digeorge syndrome 49-pancreatic def +neutropnia+malabsorption: 1-scwaman-dimoind syndrome 2-wiskot aldrich syn 3-kartenger 4-digeorge syndrome 50-neutrophil dys +dely separation of umbilical cord: 1-kostman syn 2- scwaman-dimoind syndrome 3-lad 1 4-lad 2 51-which cause erosive arthritis: 1-leukemia 2-lyme dz 3-gonococemia 4-kawasaki 52-match dz with eye lesion 1-jra b 2-sle a 3-jas d 4-poststrep arthritis 5-allagile syn e c A-epescleritis B-anterior uveitis C-posterior uveitis D-iritis E-empryotoxon 53-match 1-polyarticular jra 2-pauciarticular jra 3-still dz A-most common type 2 B-not involve small joint 2 C-rf positive 1 D-ana negative 3 E-cause more uveitis 2 54-fever+severe thrombocytosis+strawberry tonge+irritability: 1-jra 2-kawasaki 3-scarlet fever 4-rosliosa infantum 55-the most serious complication of congenital sle: 1-renal failure 2-heart block 3-cns manifestation 4-joints manifestation -56-gi manifestation of hsp occurs in about: 1-90% 2-70% 3-50% 4-30% 57-which responsible for about 1/3 of febrile convulsion cases: 1-meningitis 2-encephalitis 3-herpes 6 4-herpes 7 58-match dz with incubation period:1-measeles b 2-hepatitis a c 3-varceilla a A-11-20 days B-10-12 days C-15-30 days 59-match organism with dz 1-pseudomonas 2-salmonella 3-staph 4-b.henselae 5-chlamydia trochomatis 6-parainflaunza 7-adenvirus 8-rsv 9-etec etec travellar 10-ehec ehec hus 11-giardia 12-proteus 13-treponoma pallidum 14-parvovirus 15-trypnosoma cruzi 16-strep pneumonia 17-rota virus A-cat scratch dz 4 B-pneumonia in less than 5yrs 8 C-bronchiolitis obliterance 7 D-hemolytic uremic syndrome 10 E-lactose intolerance 11 F-renal stones 12 G-otitis externa 1 H-osteomyelitis+hemoglobinopathy I-travellar diarrhea 9 J-impetigo 3 K-croup 6 L-hydrops fetalis 14 M-afebrile pneumonia 5 N-chagas dz 15 O-jarisch herxheimer reaction 13 2 60-the most common type of tracheoesophegeal fistula tef: 1-h type 2-distal tef with esophegial atresia 3-proximal tef with esophegial atresia 4-distal +proximal tef with esophegial atresia 61-all true in pyloric stenosis except: 1-projuctile vomiting 2-billios vomiting 3-jaundice 4-string sign on barium 62-all true in intussuption except:1-the most common type is illiocolic 2- the most common type is illiocecal 3-current jelly stool 4-paraxosym of crying and pallor 5-lethargy 63-all are coombs negative hemolysis except: 1-spherocytosis 2-wilson dz 3-hemolytic uremic syndrome 4-allagile syndrome 64-the most common cause of sudden infant death syndrome: 1-rsv 2-idiopathic 3-sepsis 4-hypoglysemia -65-the most common cause of stridor in infants: 1-croup 2-laryngiomalacia 3-epiglotitis 4-adenoid hypertrophy 66-the most common laryngeal tumor in child hood: 1-rhabdomyoma 2-melanoma 3-hemangioma 4-papilloma 5-squamos cell 67-fever +stridor+drooling+muffeled voice,: 1-croup 2-trachitis 3-epiglototis 4-diphtheria 68-the most common cause of tracheal compression is: 1-vascular ring 2-anomalous innominate artery 3-pulmonary sling 4-cysticf hygroma 69-match 1-cardiac rabdomyoma g 2-hypertrophic cardiomyopathy 3-pda e 4-libman sacks edocarditis c 5-coronary thrombosis i 6-endocardial cushion h 7-aortic coarctation d 8-interrupted aortic arch b 9-supravalvular aortic stenosis 10-dextracardia a f j A-asplenia syndrome B-digeorge syndrome C-sle D-turner E-rubella F-diabetic mother G-tuber sclerosis H-down I-homocysteinuria J-william syndrome 70-at what age the lenghth of child is double 1-1yr 2-2yr 3-3yr 4-4yr 71-21year-old g2p1 woman feels that she is large for dates. She has felt fetal movement for the past week. She has a screening ultrasound scan performed at 16 weeks gestation. This ultrasound reveals that there is markedly increased amniotic fluid (polyhydramnios). Which of the following fetal abnormalities is most likely to cause this finding? A- renal agenesis B -ventricular septal defect C- cytomegalovirus infection D -small intestinal atresia E anencephaly F gastroschisis 72-7-month-old infant has failure to thrive, with vomiting and diarrhea noted since 1 month of age. Developmental neurologic milestones are not being met. A physical examination reveals hepatomegaly. The eyes show cataract formation in the crystalline lenses. The baby dies of fulminant escherichia coli septicemia at 8 months of age. Which of the following congenital abnormalities is most likely to produce these findings? A -von gierke's disease B -marfan's syndrome C -galactosemia D -congenital rubella E -phenylketonuria 73-25-year-old woman has a workup for infertility. She has never experienced menarche. On physical examination she is short, with a broad chest and a webbed neck. She has normal intelligence. A blood sample is obtained for chromosome analysis. Which of the following karyotypes is she most likely to have? A -47, xxx B -45, x C -46, xx D -47, xxy E 46, xx / 47, xxy 74-31-year-old g2 p1 woman has an uncomplicated pregnancy and gives birth at term. Examination of the newborn reveals aniridia. The external genitalia are ambiguous. Chromosome analysis reveals a 46, xy karyotype. The infant does not meet developmental milestones and it is apparent by the age of two that mental retardation is present. At the age of 3 the child is found to have a left renal mass. Which of the following conditions is this child most likely to have? A -wagr syndrome B-edwards syndrome C -tay-sachs disease D -amnionic band syndrome E –galactosemia 75-25-year-old mother notes that her 18 month old child is not keeping up with the normal growth curve for the past 5 months. She has noted that her child has not been feeding well over that time. The baby's abdomen seems larger than it should be. On physical examination, there is a left upper quadrant mass. Which of the following is the most likely diagnosis in this child? A -recessive polycystic kidney disease B -cystic fibrosis C -galactosemia D -neuroblastoma E-cystic hygroma 76--20year-old g2 p1 woman has felt minimal fetal movement by 17 weeks gestation. On examination the uterine size appears small for dates. An ultrasound scan shows growth retardation. Amniocentesis is performed and chromosome analysis on fetal cells reveals a 47 xx, +13 karyotype. Which of the following ultrasound findings is most likely to be present in this fetus? A -oligohydramnios B -cystic hygroma of the neck C -polydactaly D -hepatomegaly E -neural tube defect 77-if dietary iron is not provided, infants begin to deplete their iron stores by: A) 1 month B) 2 months C) 4 months D) 6 months E) 12 months 78-patients deficient in late complement are susceptible to: A) viruses B) fungus C) encapsulated bacteria D) intracellular bacteria E) protozoa F) gonococcus and meningococcus 79-a patient presents with recurrent infections with s. Aureus and serratia marcescens in the form of Cutaneous abscesses, lymphadenitis, pneumonia complicated by empyema, and peri-rectal abscess. His Defect is in which enzyme: A) super-oxide dismutase (sod) B) nadph oxidase C) catalase D) g6pd E) phosphofructokinase (pfk 80-downs syndrome is not associated with: A. Transposition between chromosome 14 and 21 (t14:21) B. Epicanthal fold and single palmar crease C. Increased risk of leukemia and alzheimers disease D. Radial aplasia E. Trisomy 21 from maternal dysjunction 81-4 yo bm presents with numerous hypopigmented macules of skin, sebaceous adenomas, mental Retardation, and h/o recurrent seizures. What is the mode of inheritance of this disorder? A. Aut recessive B. Aut dominant C. X linked D. Aut co dominant 82-newborn female is noted to have edema of the hands and feet. Which of the following should be included In the differential? A. Turners syndrome B. Neuroblastoma C. Sepsis D. Developmental dysplasia of the hip 83-what is the most likely diagnosis of a 9 month old child who drinks excessive amounts of only whole cow's Milk with blue sclerae and no history of trauma? A. Osteogenesis imperfecta B. Wilson's disease C. Fe deficiency anemia D. Tay-sach's 84-which skin lesion is most likely not to spontaniously resolve? A. Hemangioma B. Lymphangioma C. Mongolian spot D. Milia 85-two year old black male is admitted to the hospital for his second episode of bacterial meningitis. His Family history is positive for two other male siblings dying in infancy from sepsis. He also has a significant Past medical history of recurrent ear infections and skin infections. What is the most likely diagnosis? A. Factitious disorder by proxy B. Severe combined immunodeficiency C. Digeorge syndrome D. Bruton's agammaglobulinemia 86-match 1. Ash-leaf spots, intracranial calcifications 2. Erythema chronicum migrans, chronic relapsing arthritis sequela 3. Gower’s sign 4. Café au lait spots, cutaneous tumors 5. Intermittent limp, knee pain, limited internal rotation/abduction at hip 6. Kayser-fleischer rings, jaundice 7. Fever, skin rash, tender joint swellings 8. Duodenal atresia, simian crease, epicanthal folds 9. Vomiting, palpable abdominal “olive” in neonate 10. Roth spots, janeway lesions, osler nodes A) legg-calve-perthes disease B) lyme disease 2 C) juvenile rheumatoid arthritis 7 D) endocarditis 10 E) pyloric stenosis 9 F) down’s syndrome 8 G) muscular dystrophy 3 H) tuberous sclerosis 1 I) neurofibromatosis 4 J) wilson’s disease 6 5 87-problems not associated with infants of diabetic mothers include A. Macrosomia B. Small for gestational age C. Hypoglycemia D. Hyperbilirubinemia E. None of the above 88-what is the most frequent cause of ger in infants? A)lactose intolerance B)overfeeding C)pyloric stenosis D)medications E)increased les tone 89-which of the following is not one of the criteria for diagnosis of kawasaki disease? A. Fever >5 days B. Bilateral conjunctivitis C. Polymorphous rash D. Hepatosplenomegaly E. Cervical lymphadenopath 90-all true in poisinig with botulism except: 1-constipation 2-multiple bulbar palsys 3-hypotonia 4-diarrhea 91-all occur in reye syndrome except: 1-follow upper res tract infection 2-prolonge pt 2-hyperammonomia 3-splenomegaly 4-positive pabinski sign 5-hepatomegaly 92-the most common type of cerebral palsy: 1-choreoathetosis 2-spastic 3-dyskinetic 4-ataxic 93-all are associated with asthma except_ 1-burrel chest 2-harison sulcus 3-milk allergy 4-pulsus alternus 5-acssesory muscle use 94-the most common functional murmur is: 1-veus hum 2-carotid bruit 3-still murmur 4-pulmonary flow murmur 95-all cause ejection systolic murmur except: 1-tetrallogy of fallot 2-aortic coarctation 3-still murmur 4-marfan syndrome 96-the most common symptomatic arrythmias_ 1-atrial fibrillation 2-complete heart block 3-supraventricular tachy 4-supraventricular brady 97-all occure in pda except: 1-rubella associated 2-acomplication of hmd 3-bounding pulse 4-pulsus paradoxus 98-which associated with internus versus: 1-cystic fibrosis 2-a1-antitrypsis def 3-kartenger syndrome 4-immun def 99-squatting position in: 1-transposition of great vessels 2-tetralogy of fal 3-asd 4-hypoplastic left heart 100-the most common congenital heart dz: 1-aortic coarctation 2-membranous vsd 3-muscular vsd 4-asd 101-the most common variable of asd: 1- septum secundum 2- septum primum 3- septum venosum 102-most common cause of non traumatic limp in children 1-jra 2-sle 3-toxic synovitis 4- lipped captial femoral epiphysis - 103-all cause heart failure in neoborn except: 1-transposition of great vessels 2-viral myocarditis 3-asd 4-hypoplastic left heart 5-complete heart block 104-all cause cardiomyopathy except: 1-doxorubicin 2-amyloid 3-selenium def 4-glycogen storage dz type 3 5-friedreich ataxia 105-match 1-dic 2-uremia 3-abetalipoproteinemia 4-g6pd def 5-b thalasemia 6-lead boisinig A-acanthocyte 3 B-schistocyte 1 C-burr cell 2 D-basophilic stippling E-heinz bodies 4 F-target cells 5 6 106-all cause macrocytic anemia except: 1-fanconi anemia 2-diamond black fan syn 3-tec 4-down 5-pearson syndrome 107-deafness+optic atrophy +anemia: 1- pearson syndrome 2- fanconi anemia 3-osteopetrosis 4- diamond black fan syn 108-anemia+cleft palate+tripharyngeal thumb+widespread nipple: 1-turner 2- diamond black fan syn 3- fanconi anemia 4- osteopetrosis 109-methylmalonic acid increase in 1-folate def 2-transcobolamin def vit b12 def 3-iron def 4-copper def 110-anemia+leukemia+absent thumb+absent kidney: 1-pearson syndrome 2- fanconi anemia 3-osteopetrosis 4- diamond black fan syn 111-thalasemia b minor is diagnosed by: 1-increase hb f 2- increase hb a2 3—increase hb gower 4- a2g2 112-all true in g6pd def anemia except: 1-extravascular hemolysis 2-intravascular hemolysis 3-x- link 4-neither 113-the most common type of vasoocclusive events in sickle: 1-acute chest syndrome 2-prapism 3-pain crisis 4-hematuria 114-thrombotic microangiopathy occur in except: 1-ttp 2-dic 3-sle 4-hus 115-all cause thrombocytopnea except: 1-fanconi 2-reumatoid arthritis 3-sle 4-tar syndrome 5-organic academia thrombocytopnea+neutropnea 116-when you have a child with absent radii you expect that he had: 1-thrompocytosis 2-thrombocytopnea 3-lymphopnea 4-non 117-the most common type of vwf dz is: 1-type 1 2-type 2 3-type3 4-type 4 118-all cause dic except: 1- acute promyelocytic leukemia 2-aml m3 3-aml m1 4-snake bite 119-retinoblastoma associated with wich tumor: 1-wilms 2-osteo sarcoma 3-leukemia 4-astrocytoma 120-which associated with secretory diarrhea: 1-wilms 2-retinoblastoma 3-neuroblastoma 4-nephroblastoma 121-auer rod cell is pathognomic for: 1-all 2-aml 3-cml 4-hodgkin 122-philadelphia chromosome pathognomic for: 1- all 2-aml 3-non hodgkin 4-hodgkin 123-enolase is atumor marker for : 1-wilms 2-retinoblastoma 3-neuroblastoma 4-astrocytoma 124-opsomyoclonus is a paraneopastic syndrome of: 1-wilms 2-astrocytoma 3-neuroblastoma 4-retinoblastoma 125-all cause secretory diarrhea except: 1-carcinoid 2-neuroblastoma 3-rotavirus 4-cryptospordium 126-all invade the colon except: 1-amebia 2-giardia 3-compylobacter 4-ulcerative colitis 127-ge associated with convulsion: 1-rota 2-salmmonela 3-shagilla 4-compylobacter 128-vaccine that cause intussusption: 1-pertusis 2-rabies 3-mmr 4-rota vaccine 129-antibiotic cause pyloric stenosis: 1-clarythromycin 2-erythromycin 3-vancomycine 4-sulfa 130-contraindication for antibiotics: 1-salmonella 2-ehec 3-etec 4-cryptospordium 131-the most common soft tissue sarcoma in child: 1-osteogenic 2-rhabdomyosarcoma 3-chondrosarcoma 4-chostochondroma 132-all associated with increase a feto protein in amniotic fluid except 1-chromosomal anomaly 2-congenital nephrotic syndrome 3-neural tube def 133-regarding infantile hypertrophic pyloric stenosis (a) usually presents between 6 and 12 months of age (b) the female : male ratio is 4:1 (c) has a strong familial predisposition (d) pathologically shows hypertrophy of the longitudinal muscle layer of the pylorus (e) presents with bile-stained projectile vomiting 134-regarding intussusception all true except (a) it is the commonest cause of intestinal obstruction between 6 and 18 months (b) usually iliocolic (c) the lead point may be a peyer's patch or meckel's diverticulum (d) usually presents with few clinical signs (e) has a characteristic 'doughnut' appearance on ultrasound 135-regarding exomphalos and gastroschisis (a) a gastroschisis has a sac (b) gastroschisis is associated with major congenital abnormalities (c) the postoperative mortality of surgery for gastroschisis approaches 50% (d) both conditions may be diagnosed prenatally with ultrasound (e) both conditions require delivery by caesarian section 136-congenital diaphragmatic hernia all true except (a) usually occur through the foramen of bochdalek (b) are more common on the right than the left (c) are associated with other congenital anomalies (d) present with respiratory distress in the neonatal period (e) neonates usually require sedation, ventilation and intestinal decompression prior to surgery 137-regarding hirschsprung's disease all true except (a) often presents with neonatal large bowel obstruction (b) results from absence of ganglion cells in both the meissner's and auberbach's plexus (c) a contrast-study will show dilatation of the aganglionic segment (d) the diagnosis can be confirmed by histological evdience of increase acetylcholinesterase in the aganglionic segment (e) early treatment may involve rectal irrigation or an emergency colostomy 138-hematuria after one day of urti: 1-posstrep glomerulonephritis 2-iga nephropathy 3-nephrotic 4-hypersensetivity 139-noonan syndrome, A) is a chromosomal syndrome. B) is an autosomal recessive disorder. C) is called “male turner”, therefore only affect males. D) aortic stenosis is the most common cardiac abnormally. E) mutation in ptpn11 gene is associated with 50% of the cases. 140-hypocomplementinemia occur in all except: 1-poststrep 2-sle 3-iga nephropathy 4-membranoproliferative 141-x-linked agammaglobulinemia (xla) A) there is no circulating b cells B) associated with enlarge tonsils and lymphadenopathy C) affected individual are unwell from birth D) affected individual can receive oral polio vaccine with no increase risk 142-female +sezure+hemolysis+thromboctiopnea+uremia 1-hus 2-ttp 3-itp 4-antiphopholipid syn 143-red man syndrome. a. Is commonly seen after second oral dose of penicillin b. Is a anaphylactic reaction c. Is cause by mast cells degranulation d. Also called “red bottom syndrome” in paediatrics, as the erythematous rash in children with red man syndrome is seen mainly around the gluteal region. e. Must be treated immediately with im adrenaline 144-the most common cause of arf in child: 1-acute tubular necrosis 2-acute cortical necrosis 3-tumor lysis syn 4-myoglubinuria 145-all in crf except: 1-anemia 2-hyperk 3-polyuria 4-secondary hypoparathyroid 146-the most common cause of htn in child 1-essential 2-renal 3-vascular 4-endocrine 147-renal agenasis occur in all except: 1-potter 2-infant of diabetic mother 3-poland syn 4-vactrl syn 5-non 148 -the most common cause of bladder outlet obstruction in male 1-urethral stenosis 2-iatrogenic stricture 3-post urethral valve 4-prostatic hypertrophy 149-portal htn+systemichtn: 1-nephrotic 2-ar polycystic kidney kidney cysts+hepatic fibrosis 3-ad polycystic kidney 4-niphritic 150-cerebral aneurysm +kidney enlargement: 1-wilms 2-ar polycystic kidney 3-ad polycystic kidney 4-diabetes 151-all cause renal stone except: 1-cystinuria 2-lesh nyhan syn 3-type 1 rta 4-vit c overdose 5-non 152- dka all true except: 1-ketonuria 2-ph less than 7.25 3-hco4 less than 18 4-increase urea level 153-all cause hypoglycemia except: 1-reye syn 2-gsd 3-metabolic syndrome x 4-beck withweidman syn 154-cystic hygroma most often occur in 1-noonan syn 2-down 3-soto syn 4-russel silver syn 155-anosmia+delayed puberty: 1-klinfilter 2-turner 3-keshan dz 4-kallman syn 156-plethoric lung fields are seen in all of the following conditions, except: a. Atrial septal defect (asd) b. Tapvc (total anomalous pulmonary venous connection) c. Ebstein’s anomaly d. Ventricular septal defect 157-cardiomyopathy may be seen in all of the following except: a. Duchenne muscular dystrophy b. Friedreich’s ataxia c. Type ii glycogen storage disease d. Alkaptonuria 158-hb-6.4 gm/dl; tlc-26,500/mm3 platelet-35,000/mm3; prathrombin time–20 sec with a control of 13 sec; partial thromboplastin time-50 sec; and fibrinogen 10 mg/dl. Peripheral smear was suggestive of acute myeloblastic leukernice. Which of the following is the most likely? A. Myeloblastic leukemia without maturation b. Myeloblastic leukemia with maturation c. Promyelocytic leukemia d. Myelomonscytic leukemia 159-the defective migration of neural crest cells results in: a. Congenital megacolon b. Albinism c. Adrenogenital hypoplasia d. Dentinogenesis imperfecta 160-all of the following drugs are used for managing status epilepticus except: a. Phenytoin b. Diazepam c. Thiopentone sodium d. Carbamazepine 161-the most common cause of congenital hypothyroid 1-digeorge 2-aplasia of thyroid 3-hashimoto 4-dyshormonogenesis 162-all cause adrenal insuffecinsy except: 1-meningiococcus 2-hemochromatosis 3-zelweger syn 4-ketoconazole 5-adrenoleukodystrophy 163-the most common form of adrenogenital syn: 1-11 hydroxylase def 2-15 hydroxylase def 3-21 hydroxylase def 4-20 hydroxylase def 164-which virus cause bloody csf: 1-echo 2-mumps 3-herps 4-rabies 165-the commonest cause of west syn 1-hpoglycemia 2-strug weber syn 3-tuber sclerosis 4-aicardi syn 166-all cause hypotonia except: 1-kinky hair dz 2-rta 3-marfan 4-wilson 5-lowe syn 167-cushing triad all except: 1-htn 2-bradycardia 3-hypothermia 4-respiratory variations 168-which inheridited by mitochondrial genetic: 1-soto syn 2-laber heridety retinopathy 3-hnpac 4-x fragile 169- all in spina bifida occulata except: 1-lipoma 2-hemangioma 3-hyperkeratosis 4-hypertrichosis 170-hydroceph+abcense corpus callosum+enlarge post fossa: 1-arnold chiari syn 2-aicardi syn 3-dandy walker syn 4-soto syn 171-all of the following are true concerning otitis media except: a. Chronic or recurrent ear infections lead to conductive hearing loss. b. Bottle feeding can predispose children to ear infections c. Treatment of choice is clindamycin d. One of the most common causes of bacterial otitis media is strep.pneumonia 172-select the true statement for hiv positive mothers (positive before and at the birth of child): a. 90-100% will infect the child during delivery b. Must not take zidovudine (azt) due to its teratogenicity c. Should not breast feed their children d. Must insist their hiv positive child be immunized with all vaccines s cheduled for a non-hiv positive child 173- what characteristically happens if you incorrectly treat a pt with infectious mono by giving him/her Ampicillin? A. Severe anaphylactic reaction B. Nothing C. A rash develops D. Excessive nausea/vomiting 174-a 15yr old comes to you for a football physical. Which finding below in this childs history would preclude him from a contact sport? A. Sickle cell disease B. Asthma C. A heart mumur D. Patient has one kidney 175-a 2 year old comes in for a well baby check-up. She should be able to do all of the following except: A. Turn pages one at a time B. Use 2-3 word phrases C. Follow two step commands D. Participate in group play. 176-. A child has recently started to transfer objects, babble and use the raking grasp. He should also be able to: A. Sit unsupported B. Walk with support C. Say "mama" and "dada D. Build a tower of two blocks 177-a 15 year old white female presents to clinic complaining of numbness in both legs which started yesterday. She has recently recovered from a uri which she had about 10 days ago. She also complains of mild weakness in her proximal muscles. Her condition: A. May require treatment with ivig and plasmaphoresis B. Involves degeneration of the anterior horn cells C. Consists of symptoms which wax and wane over time D. Is only viral in origin. She should be sent home and advised to rest and drink plenty of fluids 178-a ten month old recently had a mild uri. Over the next couple of days, he develops a "barky cough" with intermittent stridor which is worse at night. He has a low grade fever. Crying seems to exacerbate the stridor. This history is most consistent with: A. Epiglottitis B. Aspiration of a foreign body C. A "thumb print" sign on lateral xray of the neck. D. A "steeple sign" on ap neck film 179-4 year old child child presents with fever, vomiting, and shortness of breath on exertion. Physical exam shows muffled heart sounds, fever, a gallop rhythm, and tachycardia. The best diagnosis for this child is A. Pericarditis B. Costochondritis C. Myocarditis D. Mitral valve prolapse E. None of the above 180-a 4mo male is brought to you for a well child check. No apparent abnormalities were noted at birth(apgars 8 and 9), but you hear a ii/vi holosystolic murmur. The mother denies any episodes of cyanosis Or respiratory distress, but doesn't think hes growing very well (birth wt. 2.7kg, currently 5.6kg). Ecg shows evidence of left ventricular hypertrophy which is also supported by cxr. What is your diagnosis? A)small vsd B)pda C)aortic stenosis D)large vsd E)aortic coarctation 181- most infant formulas contain __ kcal/oz. And most infants need __ kcal/kg/day. A. 30, 150 B. 20, 100 C. 10, 90 D. 50, 200 In children, the most common cause of hearing loss is : 1-snake bite 2-usher syndrome 3-otitis media 4-cholestetoma 5-alport syndrome The most common cause of deafness with blindness: 1-alport syndrome 2-usher syndrome 3-acoustic neuroma 4-osteopetrosis The most common cause of hematuria in children: 1-tb 2-uti 3-iga nephropathy 4-poststrep glomeriolonephritis The most common cause of gm : 1-posstrep 2-sle 3-iga nephropathy 4-minimal change dz Cerebral palsy with jaundice: 1-spastic 2-ataxic 3-chorioathetosis 4-mixed The rx of choice for juvenile myoclonus epilepsy: 1-carbamezebine 2-valporate 3-phenobarb 5-phynitoin Posterior cervical +occipital lymphoadenopathy is pathognomic for: 1-measles 2-ebv 3-hodgkin 4-rubella 5-reseliosa infantum Vit a def associated with all except: 1-measeles 2-hyperkeratosis 3-cheliosis 4-immun def Which cause periosteal hemorrhage: 1-vit k def 2-vit c def 4-copper def 5-zink def Reed strenberg cell pathognomic for: 1-ebv 2-hodgkin 3-burkitt b 4-sle vasculitis Which is the poorest stage of all: 1-l0 2-l2 3-l3 4-l1 5 yrs child complaining of joint pain +meningeal signs,cbc show pancytopnea Chemical tests show hyperurecemia,hypercreatinine , the most likely dx: 1-gout due to lysh nyhan syndrome 2-meningeococcs 3-all 4-jra tumor lysis syndrome In pancytopnea the first sign is due to: 1-neutropnea –infection 2-anemia-pallor 3-thrombocytopnea- bleeding The commonest cause of fever and rash: 1-roseliosa infantum 2-kawasaki 3-meningeococcemia 4-measeles 5-ebv Which cause cardiomyopathy: 1-gsd4 2-carnitine def 3-gsd3 4-pearson syndrome Which type of gsd associated with immune def +thrombocytopnea: 1- 1 2-2 3-3 4-4 The most common type of hypercoagulapility: 1-protein c def 2-protein s def 3-antithrombin iii def 4-leiden factor def The commonest cause of death in hemophilia: 1-liver hemmorage 2-intracranial hemmorage 3-hiv 4-hcv Pnh all true except: 1-extravascular hemolysis 2-thrombosis 3-pancytopnea 4-complement activation hemolysis Hemolytic anemia +retinitis pigmentosoa+malabsorption: 1-causher 2-lowe syndrome 3-shwaman diamond syndrome 4-abetalipoproteinemia Hartnup dz is a defect in transport of: 1-cystein 2-tryptophan 3-mg 4-tyrosin All incease icp except: 1-vit a overdose 2-hydroceph 3-hyperaldosteronism 4-hypoparathyroid 5-dka The most common cause of cushing syndrome: 1-pituatry adenoma 2-adrenal adenoma 3-exogenous cortisol 4-paraneoplastic acth In 21 hydroxylase def all true except: 1-increase 17ohp 2- increase testesterone 3- increase cortisol 4-decrease aldosterone In pseudohypoparathyroid all true except: 1-increase pth 2-decrease pth 3-increase phosphate 4-deceased ca Hypomagnesemia may cause 1-hyperkalemia 2-hypopth 3-hyper ca 4-hypophosphatemia All cause stridor except: 1-laryngiomalacia 2-hypo ca 3-hyper ca 4-vocal cord paralysis All cause macroglossia except: 1-beck with weid man syn 2-fetal alchol syndrome 3-pomp dz 4-down 5-hypothyroid In congenital hypothyroid all true except: 1-carotinemia 2-umbilical hernia 3-hypotonia 4-direct jauindice good luck