NeoPrep Potpourri Flashcards
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?
Parechovirus
Presents w hirschprungs disease associated enterocolitis. Shows up in meningitis/encephalitis (ME) panel
Classic triad of renal vein thrombosis
Gross hematuria
Flank mass
Thrombocytopenia
Risk factors: asphyxia, dehydration, shock, sepsis, congenital hypercoagulable state
Dx RUS w doppler
About __% of meconium ileus patients have CF?
Approximately __% of CF patients have meconium ileus?
90%
Meconium ileus is often first manifestation of disease
20% of CF pts have meconium ileus
Congenital lumbar hernia is a fluctuant mass lateral to the spine, depressible and may contain intestines. Which prenatal lab can be abnormal in mother?
High HbA1C or glycated Hgb
Congenital associated with maternal diabetes, which often presents with elevated glycated hemoglobin.
PIV infiltration can be treated with which 2 meds?
SubQ or intradermal hyaluronidase
If vasopressors infiltrate subq phentolamine
Which effect of epinephrine plays the MOST significant role in return of spontaneous circulation during NRP?
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.
Which biomarker has been studied to help detect AKI in neonates?
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.
What does first order elimination for a drug mean?
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.
What is the difference in bacterial colonization. between a preterm and term breastfed infants?
Preterm infants have higher concentrations of ɣ-proteobacteria with decreased Bacteroides and a substantial delay in colonization with Bifidobacterium compared with term infants.
Drug elimination zero order vs first order kinetics
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.
Which electrolyte abnormality is common with amphotericin B?
Hypokalemia, hypomagnesemia and hyponatremia 2/2 nephrotoxicity
Can also cause hepatotoxicity
Which is the most teratogenic antiepileptic drug?
Valproate- major congenital anomalies seen in ~10%
Increases risk of cognitive delay and autism
Contraindicated in pregnancy
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?
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
Testing at which age predicts need for HepC treatment?
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
Macrocephaly
Adducted thumbs
Hydrocephalus
+ Bedside diaphanoscopy (transilluminator)
MRI - with thin SYMMETRIC rim of cortical tissue, falx cerebri and unfused thalami
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.
25 weeker with anemia
S/P PRBC DOL 3, 8, 19
6 weeks of life with fever, hepatomegaly, rash, pancytopenia
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
Microcephaly Hypertonicity Facial features (synophrys, ling eyelashes, long philtrum, micrognathia, high arched palate) Low hairline GERD *
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
Diffuse hypotonia Actuve eye & brow movmt Bulbar Muscle weakness Poor suck Weak cry Tongue fasiculations Paradixical breathing
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
Fetal lung fluid is normally decreased before a normal vaginal birth by approximately______%.
65%
Prior to labor
35% ENac channel activation
Prior to delivery
30% squeeze
Severe hypoxic respiratory failure in the newborn period
Alveolar capillary dysplasia
Most common long term respiratory complication of infants woth BPD
Hospital readmissions during the first 2 years of life
Also Decreased FEV1
1/3 of term infnats eith symptomatic IVH have underlying
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
