NeoPrep Potpourri

Question 1

Term neonate with fever, signs of sepsis, lethargic with what appears to be acute abdomen, palm and sole erythema. WBC count 6.1K, no immatures, CSF neg gram stain. AXR dilated instestines, negative BE. What is the most common viral illness?

Answer 1

Parechovirus

Presents w hirschprungs disease associated enterocolitis. Shows up in meningitis/encephalitis (ME) panel

Question 2

Classic triad of renal vein thrombosis

Answer 2

Gross hematuria
Flank mass
Thrombocytopenia

Risk factors: asphyxia, dehydration, shock, sepsis, congenital hypercoagulable state
Dx RUS w doppler

Question 3

About __% of meconium ileus patients have CF?

Approximately __% of CF patients have meconium ileus?

Answer 3

90%
Meconium ileus is often first manifestation of disease

20% of CF pts have meconium ileus

Question 4

Congenital lumbar hernia is a fluctuant mass lateral to the spine, depressible and may contain intestines. Which prenatal lab can be abnormal in mother?

Answer 4

High HbA1C or glycated Hgb

Congenital associated with maternal diabetes, which often presents with elevated glycated hemoglobin.

Question 5

PIV infiltration can be treated with which 2 meds?

Answer 5

SubQ or intradermal hyaluronidase

If vasopressors infiltrate subq phentolamine

Question 6

Which effect of epinephrine plays the MOST significant role in return of spontaneous circulation during NRP?

Answer 6

Vasoconstriction

Systemic vasoconstriction (such as occurs with administration of epinephrine) from alpha-1 effect increases systemic vascular resistance and diastolic blood pressure, directly improving coronary perfusion pressure. Improvement in coronary blood flow and perfusion is critical to the return of spontaneous circulation.

Question 7

Which biomarker has been studied to help detect AKI in neonates?

Answer 7

NGAL

Neutrophil gelatinase-associated lipocalin (N-GAL) expression increases early in ischemic kidney injury and is a potential biomarker for detection of acute kidney injury in neonates before an increasing serum creatinine concentration.

Question 8

What does first order elimination for a drug mean?

Answer 8

For drugs following first order elimination, nearly 97% of the drug is eliminated from the body in 5 half-lives of the drug.

For drugs following first order elimination, the plasma concentration will be nearly 97% of the final steady state concentration in the blood after 5 half-lives of the drug.

Question 9

What is the difference in bacterial colonization. between a preterm and term breastfed infants?

Answer 9

Preterm infants have higher concentrations of ɣ-proteobacteria with decreased Bacteroides and a substantial delay in colonization with Bifidobacterium compared with term infants.

Question 10

Drug elimination zero order vs first order kinetics

Answer 10

First order kinetics (most drugs amp gent)-constant FRACTION of drug is eliminated per unit of time. Ie more drug eliminated earlier compared to later intervals of time. Half-life is independent of dose. In a logarithmic scale linear relationship vs in a linear scale it is curved.

Zero order (phenytoin ethanol) constant AMOUNT of drug is eliminated per unit time. Half life increases w higher doses.

Question 11

Which electrolyte abnormality is common with amphotericin B?

Answer 11

Hypokalemia, hypomagnesemia and hyponatremia 2/2 nephrotoxicity

Can also cause hepatotoxicity

Question 12

Which is the most teratogenic antiepileptic drug?

Answer 12

Valproate- major congenital anomalies seen in ~10%
Increases risk of cognitive delay and autism

Contraindicated in pregnancy

Question 13

Infant with infancy with diarrhea and dermatitis, diarrhea worsened when switched from breastmilk to formula. 1 sibling died from same issue. Which is another common abnormality?

Answer 13

Hyperglycemia for type 1 diabetes

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a systemic autoimmune disease caused by immune dysregulation because of a mutation of FOXP3 affecting control of T-regulatory cells.

Presents in infancy with the classic triad of enteropathy, dermatitis, and endocrinopathy (type 1 diabetes mellitus).

Bone marrow and allogeneic hematopoietic stem cell transplantation are the only potential cures

Question 14

Testing at which age predicts need for HepC treatment?

Answer 14

Treatment is based on hepatitis C virus (HCV)-RNA nucleic acid amplification testing at age 3 years as some have spontaneous clearance before 3 yrs and treatment fda approved for 3 years of age

For screening: Antibody testing is recommended for children of hepatitis C–positive mothers at 18 months of age

Question 15

Macrocephaly
Adducted thumbs
Hydrocephalus

+ Bedside diaphanoscopy (transilluminator)

MRI - with thin SYMMETRIC rim of cortical tissue, falx cerebri and unfused thalami

Answer 15

X-linked hydrocephalus

• caused by stenosis of aqueduct of sylvius
• mutation L1CAM

Hydranencephaly has ASYMMETRIC cortex loss due to early in utero vascular accident
Presence of falx cerebri

Cranial imaging demonstrates the presence of the falx cerebri and unfused thalami in both hydranencephaly
and hydrocephalus.

A thin symmetric rim of cortical tissue may be seen in hydrocephalus, whereas small
asymmetric remnants of cortical tissue are typically found in hydranencephaly.

Question 16

25 weeker with anemia
S/P PRBC DOL 3, 8, 19
6 weeks of life with fever, hepatomegaly, rash, pancytopenia

Answer 16

Transfusion associated Graft vs Host Disease

Tx- immunosuppressive tx (corticosteroid)

Median time to fever - 28 d

Prevention is key - use irradiated RBC

Leukoreduction decreases viral (CMV), HLA allo immunization, WBC in donated blood and therefore 60% of febrile non hemolytic rxn

Question 17

Microcephaly 
Hypertonicity
Facial features (synophrys, ling eyelashes, long philtrum, micrognathia, high arched palate)
Low hairline
GERD *

Answer 17

Cornelia de Lange

NIBL gene in 5p13
Single nucleotide mutation (de novo + sporadic) —-therefore microarray will be normal

AD

Assoc’d problems- GERD (90%), malrotation +/- volvulus (2->10%)
UGI recommended early if emesis present

Question 18

Diffuse hypotonia
Actuve eye & brow movmt
Bulbar Muscle weakness
Poor suck
Weak cry 
Tongue fasiculations 
Paradixical breathing

Answer 18

SMA1

AR
Frameshift mutation in survival motir neuron 2 (SMN2) gene on chr 5q13.
Presents before 6 mo

Diffuse hypotonia, muscle weakness, decreased to absent DTR, tongue fasciculations, paradoxical breathing

Early treatment with nusinersin leads to increase survival and improved motor function

Question 19

Fetal lung fluid is normally decreased before a normal vaginal birth by approximately______%.

Answer 19

65%

Prior to labor
35% ENac channel activation
Prior to delivery
30% squeeze

Question 20

Severe hypoxic respiratory failure in the newborn period

Answer 20

Alveolar capillary dysplasia

Question 21

Most common long term respiratory complication of infants woth BPD

Answer 21

Hospital readmissions during the first 2 years of life

Also Decreased FEV1

Question 22

1/3 of term infnats eith symptomatic IVH have underlying

Answer 22

Cerebral sinovenous thrombosis

Most colmon places for ivh is choriod plexus and thalamus

MC presenting sign is seizures

Risk factors mother w PreE, diabetes, ? Chorio, prothrombotic factors and HTN

Risk factor neonate with birth asphyxia, meconium, dehydration, sepsis, meningitis, cardiac defects, prothrombotic factors, ECMO

Tx: anticoagulation, if none f/u mrv 1 week to assess clot propagation