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1

what are the rule of 2's in Meckel's diverticulum?

2 feet from ileocecal valve
2 in long
2 times more common in males
2% of population
2% develop complication over course of their lives

2

what conditions mimic acute appendicities

Yersinia enterocolitis
mesenteric adenitis
constipation
crohn's
meckel's
ectopic pregnancy
UTI
ovarian torsion

3

what murmur is associated with marfan ?

aortic root dilatation leading to diastolic decrescendo murmur (aortic insufficiency)

can also have mitral valve prolapse

can lead to aortic dissection

4

what is DiGeorge syndrome

facial features
-low-set ears
-eyes wide apart
-palate anomalies
-widened area below nasal bridge
-short philtrum
-micrognathia

cardiac lesion
-abnormal heart sounds
-murmur
-right sided aortic arch
-conotruncal anomalies

No thymic shadow seen on CXR

Hypocalcemia- parathyroid hypoplasia - (tetancy, seizures)


results from failure of the third and fourth pharyngeal pouches

**lots of viral and fungal infections b/c of lack of thymus

5

what are the common conotruncal anomalies associated with DiGeorge

transposition of great vessels, truncus arteriosus, right sided aortic arch

6

by age 12 months, infants should weigh how much compared to birth weight

3 times

7

what is the point of substituting medium chain triglycerides for long chain triglycerides in a babies diet?

if a baby has malabsorption and steatorrhea this will help because MCTs do not require bile acids for absorption.

preterm infants have steatorrhea b/c of poor absorption of fat due to small bile acid pool

8

what is a mongolian spot

hyperpigmentation found in 66% of all infants of hispanic, asian and native american ethnicity

sometimes mistaken for bruise

9

vomiting in a 10 month old, with lethargy, dehydration, full anterior fontanelle , no diarrhea, posturing

administer dexamethasone to decrease the intracranial pressure

most likely a brain tumor

10

resp tract infection
neonatal jaundice
recurrent bronchiolitis
nasal polyps
chronic diarrhea
features of malabsorption
meconium ileus
recurrent sinus infection

cystic fibrosis


obstructive airway disease
clubbing

11

what antibiotics do you use for CF treatment

tobramycin plus antipseudomonal (ticarcillin/piperacillin)

tobramycin pluse third gen cephalosporin (cefepime, ceftazidime)

tobramycin plus carbapenem (imipenem/cilastatin, meropenem)

12

what antibiotic is used to cover pasturella multocida

penicillin is the treatment of choice, but rare isolates with B-lactamase activity have been reported.

amoxicillin-clavulanate helps with resistance

if penicilin allergy use azithromycin

dog/cat bites
symptoms occur within a 24 hour period

13

if you suspect a lactase deficiency in a kid, what test should you do

lactose breath test

Its prevalence
in subjects of Asian (Far-Eastern) extraction is at least 85%

sucrase-isomaltase deficiency is the most common congenital disaccharidase deficiency

14

what is the lactulose breath test used for

useful in evaluation of suspected bacterial overgrowth, particularly pt's who have undergone prior bowel surgery and present with symptoms that suggest a blind or stagnant intestinal loop

15

what are watery, acidic stools indicative of

carbohydrate malabsorption

16

“thumb sign”)

epiglottitis

lateral radiograph

Affected patients typically
present between the ages of 2 and 8 years with the rapid onset of fever,
sore throat, and the “four Ds” (drooling, dysphagia, dysphonia, and
dyspnea). Patients often assume a position of comfort by sitting upright,
leaning forward, and bracing themselves with their arms, known as the
tripod position.

17

steeple sign

AP view of croup

18

what is the treatment for acute rheumatic fever in a peds patient

Penicillin G or 10 days of oral penicillin even if throat culture is negative

19

what are the major criteria for rhf

polyarthritis
carditis
subcutaneous nodules
sydenham chorea
erythema marginatum

20

what are the minor manifestations for RHF

fever
arthralgia
prolonged PR interval on ECG
Elevated acute phase reactants (ESR, CRP)

21

hyponatremia
postural hypotension
hyperkalemia
lack of response to cosyntropin stimulation test

plus

neurologic deficits -weakness, spasticity, dementia, blindness, quadraparesis

Adrenoleukodystrophy

x-linked disease where there is a mutation in the transportation of very long-chain fatty acids (VLCFA) into peroxisomes, thereby preventing beta-oxidation and breakdown of VLCFA
accumulation of VLCFA in neurons and adrenal cortex causes symptoms

adrenal stuff is usually detected before neuro stuff

22

hypotonia
upslanting palpebral fissures
epicanthal folds
excess nuchal skin
enlarged tongue
clinodactyly of the fifth fingers
single transverse palmar crease

trisomy 21

23

small palpebral fissures
low set ears
low birth weight
microcephaly
rocker bottom feet
cleft lip
hypotonia
clenched hands

Edward syndrome (Trisomy 18)

24

what are the cyanotic diseases of the newborn

truncus arteriosus
tricuspid atresia
tetrology of fallot
transposition of the great arteries
total anomalous pulmonary return and pulmonary stenosis

25

what is rx for scabies

premethrin cream

26

what anomalies are associated with diabetes during pregnancy

small left colon
anencephaly
meningomyelocele
cardiac- assymetric septal hypertrophy, transposition of great vessels, VSD
-sacral agenesis
renal vein thrombosis
renal agenesis

27

what heart defect is associated with turner

coarc of aorta

28

rash that spreads from the trunk to the extremities and is particularly associated with high fevers ....

roseola infantum caused by human herpes virus 6

29

Patient presents with short stature, webbed neck, shield like chest and widely spaced nippled, no secondary sex characteristics.... what will the levels of FSH, LH and Estradiol be? What will a buccal smear show?

High FSH
High LH
Low estradiol
Buccal smear shows normal appearing epithelial cells with no barr bodies

Rx: Estrogen Therapy

Turner = XO

30

X-linked
causes low or absent numbers of B cells leading to panhypogammagloblulinemia

cellular immunity is intact

pt develops infections after approximately 6 months of age when maternal ab's have decreased to low levels

recurrent infections

Bruton Agammaglobulinemia

31

eczema
thrombocytopenia
recurrent infection

Wiskott -Aldrich Syndrome

32

positive whiff test

gardnerella or bacterial vaginitis

Rx. Metronidazole

33

most common cause of neonatal meningitis

strep agalactiae - group B strep

other common bugs are e coli and listeria monocytogenes

34

consumptive thrombocytopenia

microangiopathic hemolytic anemia

fever

renal dysfunction

fluctuating neuro deficitis

thrombotic thrombocytopenic purpura

35

anemia, thrombocytopenia, leukopenia and/or bone pain, hepatosplenomegaly, lymphadenopathy

ALL

36

what eye findings are seen in neurofibromatosis

lisch nodules (iris hamartomas)

37

what skin findings are seen in tuberous sclerosis

ash-leaf macules
facial angiofibromas (adenoma sebaceum) and shagreen patches (connective tissue nevi)

seizures are the most common presenting symptom

38

Cerebral calcification
facial nevus
glaucoma in the ipsilateral eye
seizures contralateral to the side of the nevus

sturge weber

39

freckling in the axillary or inguinal region

NF-1

40

history of neonatal jaundice
recurrent bronchiolitis
nasal polyps
chronic diarrhea
malabsorption
meconium ileus

cystic fibrosis

41

antidote for iron poisoning

deferoxamine

42

what is the antidote for lead poisoning

EDTA + either DMSA or BAL (Dimercaprol )

with encephalopathy?
EDTA + BAL

43

most common pathogen in young children with CF

staph aureus

Rx. assume there is methicillin resistance and start vancomycin

44

what drugs can be used to treat pseudomonas in CF pt's

amikacin
ceftazidime
ciprofloxacin