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1-NEUROFIBROMATOSIS
2-TUBER SCLEROSIS
3-FRAGILE X SYNDROME
4-DOWN SYNDROME
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
1-musty
2-cabbag الكرنب
3-sweety
4-sweaty
5-cat
6-galric ((ثوم
7-wintergreen
a-Tyrosinemia 2
b-glutaric aciduria ii 4
c-maple syrup 3
d-multiple carboxylase def 5
e- PKU 1
f-organophosphate 6
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
1-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
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
38-all cause pulmonary hypoplasia except none تعديل السؤال بدل تيرنر-
1-hydrops fetalis
2-potter syndrome
3-werdnig-hoffmann syndrome
4-diaphragmatic hernia
5-none
1-b thalasemia
2-diamond blackfan syndrome
3-osteopetrosis
4-g6pd def
47-thrtombocytopnea+eczema+immune def :
1-scwaman-dimoind syndrome
2-wiskot Aldrich syn
3-kartenger
4-folic acid def
1-scwaman-dimoind syndrome
2-wiskot Aldrich syn
3-kartenger
4-digeorge syndrome
1-kostman syn
2- scwaman-dimoind syndrome
3-LAD 1
4-LAD 2
1-JRA b
2-SLE a
3-JAS d
4-poststrep arthritis c
5-allagile syn e
a-epescleritis
b-anterior uveitis
c-posterior uveitis
d-iritis
e-empryotoxon
53-match
1-polyarticular JRA
2-pauciarticular JRA
3-still dz
1-90%
2-70%
3-50%
4-30%
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 2
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
1-spherocytosis
2-wilson dz
3-hemolytic uremic syndrome
4-allagile syndrome
69-match
1-cardiac rabdomyoma g
2-hypertrophic cardiomyopathy f
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 j
10-dextracardia a
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
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
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
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. 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
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. Gowers 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 5
b) Lyme disease 2
c) Juvenile rheumatoid arthritis 7
d) Endocarditis 10
e) Pyloric stenosis 9
f) Downs syndrome 8
g) Muscular dystrophy 3
h) Tuberous sclerosis 1
i) Neurofibromatosis 4
j) Wilsons disease 6
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
a-acanthocyte 3
b-schistocyte 1
c-burr cell 2
d-basophilic stippling 6
e-heinz bodies 4
f-target cells 5
116-when you have a child with absent radii you expect that he had:
1-thrompocytosis
2-thrombocytopnea
3-lymphopnea
4-non
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.
142-female +sezure+hemolysis+thromboctiopnea+uremia
1-HUS
2-TTP
3-ITP
4-antiphopholipid syn
149-portal HTN+systemicHTN:
1-nephrotic
2-AR polycystic kidney kidney cysts+hepatic fibrosis
3-AD polycystic kidney
4-niphritic
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. Ebsteins anomaly
D. Ventricular septal defect
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
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
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
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
good luck