10

Question 1

bilious emesis due to intestinal obstruction, abdominal distension, blood per rectum, and lethargy in infant, think

Answer 1

malrotation with volvulus

vs intussusception: colicky abdominal pain and currant jelly stools

Question 2

COMMON ETIOLOGIES OF ACUTE ABDOMINAL PAIN IN INFANTS AND YOUNG CHILDREN

Answer 2

http://casefiles.mhmedical.com.mwu.idm.oclc.org/ViewLarge.aspx?figid=140632540&gbosContainerID=75&gbosid=219808

Question 3

if unable to get a peripheral IV in a decompensating pt, next step?

Answer 3

interosseous IV, quicker than central line

Question 4

classic triad of situs inverses, recurrent sinusitis, and bronchiectasis

Answer 4

Kartagener syndrome

Question 5

galactosemia caused by deficiency of ?

Answer 5

galactose-1-phosphate uridyle transferase

-cataracts, lethargy, hepsplenmeg, hypoglycemia, convulsions, poor weight gain, jaundice

Question 6

iron def. vs thalassemia

Answer 6

anemia in iron def will have an increased RDW

thalassemia will have normal RDW and blood count, both will have low MCV

Question 7

levels in Turners
what are these girls at risk for?
the swelling in these girls is due to?

Answer 7

low estrogen and high FSH due to lack of negative feedback

• osteoporosis due to estrogen deficiency
• lymphedema

Question 8

Tourettes meds

Answer 8

haloperidol, pimozide, risperidone

Question 9

angular cheilosis (lips), stomatitis, glossitis, normocytic anemia, seborrheic dermatitis

Answer 9

vitamin B2 (riboflavin) deficiency 
(similar to B6 pyridoxine def. but that should have mental changes)

Question 10

polycythemia risk factors

symptoms?

Answer 10

delayed cord clamping, maternal HTN, DM

resp. distress, hypoglycemia, neuro manifestations

Question 11

lactic vs renal tubular acidosis

Answer 11

RTA will have a normal anion gap, lactic acidosis will have an elevated anion gap

Question 12

metabolic/e-lyte abnormalities in IDM

Answer 12

hypoglycemia, hypocalcemia, hypomagnesemia

Question 13

vaccines to give at 2 months

Answer 13

HepB, Rotavirus, DTaP, Hib, IPV, PCV (pneumococcal)

Question 14

pts with ataxia-telangiectasia will have what changes to immunglobulins

Answer 14

low IgA, IgG, IgE, elevated IgM

Question 15

Abdominal radiographs may be normal or have nonspecific findings in cases of volvulus; thus, an ? is the test of choice

Answer 15

upper gastrointestinal contrast series

Question 16

characteristic finding in cases of volvulus on upper GI series

Answer 16

a “corkscrew” pattern of the duodenum or “bird’s beak” of the second or third portions of the duodenum

Question 17

management of an unstable pt with malrotation before emergent surgical intervention

Answer 17

evaluate fluid status
place a nasogastric tube to aid GI decompression
initiate IV abx
Exploratory laparotomy to assess bowel viability
Areas of necrotic bowel are removed and Ladd procedure of disengaging bowel with anomalous fixation and appendectomy is performed

Question 18

distension, vomiting, bloody stools, and systemic signs such as temperature instability, thrombocytopenia, poor feeding, apnea, and respiratory failure.

Answer 18

necrotizing enterocolitis (NEC)

Question 19

how to diagnose posterior urethral valves (PUV)

Answer 19

renal US or VCUG

can also see on prenatal US

Question 20

? are the most common cause of severe urinary tract obstruction in boys

Answer 20

Posterior urethral valves

25% to 30% ultimately have end-stage renal disease or chronic renal insufficiency

Question 21

Neonates with PUVs may present with

Answer 21

respiratory distress secondary to lung hypoplasia from oligohydramnios, distended bladders, poor or dribbling urinary streams, palpable kidneys, reduced renal function, or UTI

Question 22

Immediate relief of PUV obstruction includes ?

labs?

Answer 22

• bladder catheterization through the urethra with a small feeding tube
• start abx if thinking UTI
• Serum electrolytes, BUN and creatinine levels are measured with correction as needed
• Hemodynamic status is monitored because sepsis or renal failure can lead to cardiovascular collapse

Question 23

PUV tx once stabilized

Answer 23

endoscopic transurethral valve ablation

Question 24

If the serum creatinine remains elevated, the urethral lumen is too narrow, or the UTI does not respond to antibiotics, ? may be necessary for PUV instead of endoscopic transurethral valve ablation

Answer 24

emergent vesicostomy

Question 25

what kids should undergo evaluation for vesicoureteral reflux in addition to being treated with antimicrobials

Answer 25

2 to 24 months, children with recurrent UTIs, febrile UTIs, or pyelonephritis
renal US, then VCUG, then cystoscopy

Question 26

how to tx nursemaid’s elbow

Answer 26

supinating the child’s forearm with the elbow in flexed position while applying pressure over the radial head

Question 27

HUS

Answer 27

A syndrome of nephropathy, thrombocytopenia, and microangiopathic hemolytic anemia. It is associated with E.coli 0157:H7, Shigella, and Salmonella. A prodrome of bloody diarrhea is common

Question 28

TTP

Answer 28

Pentad of fever, microangiopathic hemolytic anemia, thrombocytopenia, abnormal renal function, and CNS changes
-more common in adolescents than younger kids

Question 29

ITP

Answer 29

A condition of increased platelet destruction by circulating antiplatelet antibodies, most frequently antiglycoprotein IIb/IIIa.

Question 30

Laboratory findings of ITP

Answer 30

thrombocytopenia, which can be severe (<20,000/mm3), but the platelet size is normal or increased.
WBC count and hemoglobin level are normal (unless excessive bleeding has occurred)
PT and aPTT are normal

Question 31

when to do BM eval in ITP

Answer 31

typically unnecessary, do if peripheral blood smear is concerning, the WBC count is abnormal, or adenopathy or organomegaly is present
-will show ncreased number of megakaryocytes in ITP

Question 32

most serious ITP complication

Answer 32

intracranial hemorrhage

Question 33

ITP tx

Answer 33

most are self-limiting

ITP with significant bleeding: IVIG for 1-2 ds, IV anti-D therapy, or a 2- to 3-week course of systemic corticosteroids

Question 34

meds that cause immune-mediated thrombocytopenia

Answer 34

PCN, TMP-SMX, igoxin, quinine, quinidine, cimetidine, benzodiazepine, and heparin
MMR vaccine

Question 35

Approximately ? of children with ITP have spontaneous resolution within 6 months

Answer 35

70% to 80%

Question 36

peripheral blood smear of HUS

Answer 36

helmet cells, burr cells, and fragmented RBCs

Question 37

A fracture of the long bones that is considered pathognomonic for inflicted injury

Answer 37

classic metaphyseal lesion
“corner fracture,” the “bucket handle fracture,” and the “metaphyseal fragmentation fracture.”
-occurs at the primary spongiosa region of the ends of the long bones and has the appearance of a bone fragment.

Question 38

Bacterial diseases that share similar features of fever and exanthem to Kawasaki

Answer 38

group A streptococcal disease (scarlet fever, toxic-shock syndrome), Staphylococcus aureus toxic shock syndrome, Rocky Mountain Spotted fever, and leptospirosis

Question 39

Noninfectious causes of similar symptoms of Kawasaki

Answer 39

drug hypersensitivity reactions or systemic-onset juvenile idiopathic arthritis (JIA)

Question 40

lab findings in Kawasaki

Answer 40

elevated ESR and CRP normocytic anemia, leukocytosis, thrombocytosis, hypoalbuminemia, elevated ALT, and sterile pyuria. CSF pleocytosis and mildly elevated hepatic transaminase levels

Question 41

associated Kawasaki symptoms

Answer 41

painful and frequent urination, meningismus, vomiting, or right upper quadrant pain

Question 42

Klinefelter syndrome

Answer 42

syndrome comprised of behavioral problems (immaturity, insecurity), developmental delay (speech, language, lower IQ), and physical abnormalities (gynecomastia, hypogonadism, long limbs) caused by an extra X chromosome in males
-caused by nondisjunction

Question 43

etiologies of ID (intellectual disability)

Answer 43

preconception and early embryonic disruptions, fetal brain insults, perinatal difficulties, postnatal brain inures, postnatal family difficulties

Question 44

Physical findings to be considered in patients with suspected ID

Answer 44

the size of the occiput, unusual hair color or distribution, eye shape and placement, malformed ears or nose, and abnormalities in jaw size, mouth shape, or palate height
short metacarpals or metatarsals, overlapping or supernumerary digits, abnormal palmar creases, or nail changes
afé au lait spots or depigmented nevi, and the genitalia may be abnormally sized or ambiguous
tall and thin with long extremities
small testes/penis, gynecomastia, sparse facial hair, and azoospermia

Question 45

chromosomal analysis of Klinfelter syndrome

Answer 45

one extra X chromosome (47,XXY) but may show additional X chromosomes (48,XXXY) or mosaicism (46,XY/47,XXY)

Question 46

lab and imaging for intellectual disability

Answer 46

urine and serum amino and organic acids, serum levels of ammonia, lead, zinc, and copper, and serum titers for congenital infections
cranial CT, MRI, EEG

Question 47

XYY males presentation (NOT XXY: Klinefelter)

Answer 47

explosive (often antisocial) behavior, weakness with poor fine motor control, accelerated growth in mid-childhood, large teeth, prominent glabella and asymmetrical ears, and severe acne at puberty

Question 48

Girls with Turner syndrome (45,XO) present how

Answer 48

short stature, amenorrhea, excessive nuchal skin, low posterior hairline, broad chests with widely spaced nipples, edema of the hands and feet in the newborn period, cubitus valgus, coarctation of the aorta, hypothyroidism, and decreased hearing. Hypertension is common, possibly due to renal abnormalities (horseshoe kidney)
Intellectual development is typically normal

Question 49

Fragile X syndrome

Answer 49

the most common form of inherited intellectual disability, is seen primarily in boys and can be diagnosed in patients with intellectual disability who have macrocephaly, long face, high arched palate, large ears, and macroorchidism after puberty.