Test #3 (surgical, metabolic, NEC, Neuro, GU)

Question 1

What defect is when the umbilical cord in uninvolved and to the right of the umbilicus?

Answer 1

gastroscheisis

Question 2

What does gastroschisis appear in embryonic life?

Answer 2

4 week

Question 3

What abd midline defect is associated with heart defects?

Answer 3

omphalocele

Question 4

Where does an omphalocele defect herniate?

Answer 4

through the umbilical ring

Question 5

When does omphalocele efect fail to return to abd cavity?

Answer 5

11 weeks

Question 6

What test should you have done before you do surgery on omphalocele?

Answer 6

ECHO

Question 7

What should you start IV fluids at 150ml/kg/day on day 1 with abd wall defects?

Answer 7

Bc of continuous sx on og output

Question 8

What is the % of Cardiac anomalies with TEF/eso atresia?

Answer 8

30%

Question 9

What defect has an x-ray with a gasless abd?

Answer 9

Eso atresia ONLY

Question 10

What is another cue on an x-ray with EA?

Answer 10

dilation esophageal pouch

Question 11

What is an ER surgery for EA/TEF?

Answer 11

gastrostomy for decompression

Question 12

Long term probs with EA/TEF: stricture/GE reflux, anastamotic leak, esophageal dysmotility all cause ____.

Answer 12

risk of aspiration

Question 13

What defect shows a double bubble?

Answer 13

Duodenal atresia

Question 14

Where is the level of obstruction in duodenal atresia?

Answer 14

distal to ampulla of Vater

Question 15

What is the sign seen on 1st DOL with malroatation/volvulus?

Answer 15

Bilious vomiting

Question 16

What disease is seen with congenital aganglionic megacolon?

Answer 16

Hirschprung’s disease

Question 17

What should you think when an infant has a meconium ileus?

Answer 17

Cystic fibrosis (90%)

Question 18

What abd defect should be repaired ASAP?

Answer 18

gastroschisis

Question 19

NEC correlates with degree of ____

Answer 19

Prematurity

Question 20

What is a critical finding in NEC that is uncorrectable?

Answer 20

Dead gut

Question 21

Free air seen in the abdominal x-ray is ____ with NEC

Answer 21

Pneumoperitoneum

Question 22

Persistent acidosis suggests what in NEC?

Answer 22

Necrotic bowel

Question 23

What is removed with surgery with NEC?

Answer 23

Frankly necrotic bowel

Question 24

What is a normal finding on NBS r/t prematurity?

Answer 24

decrease T4

Question 25

If left untreated what leads to irreversible brain damage and death?

Answer 25

Hypothyriodism

Question 26

What is an inborn error of with smelly urine?

Answer 26

Maple syrup urine diesase

Question 27

An X-ray with a double bubble, what else would you evaluate if the baby had trisomy 21?

Answer 27

Cardiac anomalies

Question 28

If a baby has galactosemia what formula should you feed?

Answer 28

Isomil

Question 29

Why in the fetus unaffected with inborn errors of metabolism?

Answer 29

the placenta removes the toxins

Question 30

What are some clues in family hx with Inborn errors of metabolism?

Answer 30

MR, SIDS, unexplained neonatal death

Question 31

What are some common s/s inborn errors of metabolism?

Answer 31

vomiting, poor fdg, no wt gain, diarrhea, jaundiace, heptomegaly

Question 32

When the radiologist says the cecum is riding high think!

Answer 32

Its not in the right place and probably malrotation

Question 33

When there is an increase in lactate with metabolic acidosis you should think?

Answer 33

Pyruvate metabolism defects

Question 34

What are the first dx studies for inborn errors of metabolism?

Answer 34

CBC Urinalysis CBG Electrolytes, glucose, ammonia level

Question 35

With dx studies for inborn errors of metabolism, a urinalysis should be tested for?

Answer 35

reducing substances

Question 36

What is seen in inborn errors of metabolism with hypoglycemia?

Answer 36

- urine non-glucose reducing substances | - galaxtosemia, tyrosimemia type 1

Question 37

In 21-OH deficiency what should be given to suppress SCTH and androgen overproduction?

Answer 37

Cortisol replacement - hydrocortisone

Question 38

what is the medical mgnt for Congential Adrenal hyperplasia?

Answer 38

glucocorticoid therapy mineralocorticoid therapy Na supplementation

Question 39

What is the typical age of onset for NEC? | for late preterm?

Answer 39

2-3 weeks | ~7days

Question 40

What are two major associations with NEC?

Answer 40

preinatal hypoxia | polycythemia

Question 41

What NEC is diminished bowel sounds, dilation, and pneumatosis.

Answer 41

Stage 2

Question 42

What NEC shows portal vein gas, metabolic acidosis, and throbocytopenia

Answer 42

Stage 2B

Question 43

What NEC stage is Neutropenia, abdominal wall erythema, hyoptension

Answer 43

Stage 3A

Question 44

What NEC stage has perforated bowel, shock, and pneumoperitoneum?

Answer 44

Stage 3B

Question 45

What is a tale tell sign with NEC?

Answer 45

Hyponatremia and hypothermia

Question 46

What is when a piece of bowel does not move and can indicate dead bowel?

Answer 46

Sentinel loop

Question 47

What is the tx for intestinal perf in ELBW along with explo lap?

Answer 47

PPD (peritoneal drainage) pin rose drain

Question 48

What defines short bowel syndrome?

Question 49

What intestinal disease that is not assoc. with pneumoatosis, unrelated to feeds?

Answer 49

Spon intestinal perforation

Question 50

What are 3 signs consistent with acute peripartum event?

Answer 50

APGAR 12

Question 51

What are some treatment options to prevent severe CND deficits?

Answer 51

Ventilate, prevent hypoxia, treat seizures, stable Vitals

Question 52

What are the eligibility criteria for therapeutic hypothermia?

Answer 52

Fetal cord gas with pH 16 acute perinatal event, APGAR

Question 53

What is the most common type of seizure and most often missed?

Answer 53

Subtle seizure

Question 54

Seizure or Jitters: | when activity easily elicited with stimulation

Answer 54

Jitterness

Question 55

What drug and dosage used for seizures?

Answer 55

Phenobarbital: Load with 20mg/kg, sz cont: 20mg/kg | theraputic level 15-40mg

Question 56

What are the outcomes for lower birth wt infants?

Answer 56

The outcome is worse with more severe neurological disease.

Question 57

Describe a grade 2 and 3 IVH?

Answer 57

II - IVH with normal ventricles | III - IVH with dilated ventricles

Question 58

PVL can occure separate from what?

Answer 58

P/IVH

Question 59

What will you see with a LP in Subarachnoid hemorrhage?

Answer 59

Blood in the LP

Question 60

what are some disease that cause prerenal failure

Answer 60

asphyxia, CHF, dehydration, hypotension, RDS, CHD

Question 61

What are some examples of Postrenal failure?

Answer 61

prune belly, renal calculi, blood clots, neurologenic dysfunction, ureterocele

Question 62

What is a good indicator of renal failure?

Answer 62

Urine SG prerenal >1.015, posrenal

Question 63

What is a good indicator of renal intrinsic dz?

Answer 63

granulae casts and spitheleal cells in urine

Question 64

What is the most common abdominal mass?

Answer 64

hydronephrosis

Question 65

What test can be preformed if there is a vesicourethral reflux?

Answer 65

VCUG

Question 66

What is the abnormal flow or urine from the bladder back into the ureters?

Answer 66

vesicourethral reflux?

Question 67

What are three clinical manifestations of ureterocele?

Answer 67

hematuria, abd mass, UTI

Question 68

What is the most common cause of bladder outlet obstruction in male infants?

Answer 68

Posterior urethreal valve

Question 69

What is a common sign of Posterior urethreal valves?

Answer 69

palpable distended bladder

Question 70

What is an autosomal recessive disease that is rare with poor prognosis?

Answer 70

Polycystic kidney disease

Question 71

What are some preinatal causes of renal vein thrombosis?

Answer 71

toxemia, maternal diabetes, placental insufficiency

Question 72

What are the most common S/S of renal vein thrombosis?

Answer 72

Mass, gross hematuria, and HTN!!!

Question 73

When there is a sudden severe blockage of the renal artery?

Answer 73

Renal artery thrombosis - lines

Question 74

What is a good indication of renal ARTeral thrombosis?

Answer 74

systemic HTN