Peds 3 Flashcards
MOA of Phenobarbital in treating jaundice
Phenobarbital induces UGT, thus increasing conjugation of bili
Most common cause of baby born with poor feeding, tachypnea, hypothermia, and poor perfusion
Group B Strep pneumonia
How do you prevent GBS infection in a newborn
Screen mothers at 35 weeks gestation, and give IV Penicillin during labor to those with high risk or positive GBS
Most common organisms of early-onset neonatal sepsis (within 6 days of life)
GBS (#1) , E. Coli, H. flu, Listeria monocytogenes
Tx of early onset neonatal sepsis
Abx directed at most common pathogens - usually combo of IV aminoglycosides and Penicillin
Tx of late onset neonatal sepsis (7-90 days of life)
Beta-lactamase resistant abx (such as vancomycin) and often 3rd generation cephalosporin
What is greatest risk factor for neonatal sepsis
Prematurity (#1)
Additional risk factors include low birth weight, rupture of membranes >18 hrs before deliver, young maternal age, initial apgar <5
Describe congenital adrenal hyperplasia
Disorder of adrenal steroid production with an enzymatic deficiency (most common 21-hydroxylase)
Causes inadequate production of cortisol (causing increase in ACTH), and excessive production of androgens (due to increased ACTH, leading to virilization)
Describe difference in presentation of 21 a-hydroxylase deficiency and 11 b-hydroxylase deficiency
Both have decreased cortisol, causing increased ACTH, and increased androgens
21 = has decreased mineralocorticoids (decreased BP and hyperkalemia)
11 = has increased intermediated mineralocorticoid (increased BP)
o If there is a 1 in the first digit of deficient enzyme (11 or 17) – will be HTN
o If there is a 1 in the second digit of deficient enzyme (11 or 21) – will be masculinization
Definition of hermaphroditism
Discrepancy between gonad morphology and external genitalia
Describe 5 alpha-reductase deficiency
Phenotypically normal female with functioning testicular tissue and short, blind-end vagina
Due to inability of 5 a-reductase to convert testosterone to DHT, which is needed for development of male external structures
Describe true hermaphroditism vs. pseudohermaphroditism (female and male)
True = both male and female internal genitalia (testes and ovaries)
Pseudo female = 46, XX with internal female and external male (usually caused by CAH)
Pseudo male = 46, XY with internal male and external female (e.g. 5 a-reductase deficiency)
What serum level should you check for in a baby with suspected CAH
Increased 17-hydroxyprogesterone
What is the classic disease associated with female with primary amenorrhea
5 alpha-reductase deficiency (46, XY with female phenotype)
Decreased androgen binding or androgen insensitivity
Describe difference in obstruction in meconium ileus vs. Hirschsprung disease
Meconium ileus = Seen in CF. Leads to obstruction of the ileum and a narrow, underdeveloped colon (microcolon)
Hirschsprung = Level of obstruction usually at the rectosigmoid junction, with proximal dilated colon
What are the 5 T’s that cause R-to-L shunt (early cyanosis
o Truncus arteriosus (1 vessel) o Transposition (2 switched vessels) o Tricuspid atresia (3 = tri) o Tetralogy of Fallot (4 = tetra) o TAPVR (5 letters in the name)
Describe transposition of the great vessels
- LV enters into the pulmonary trunk
- RV enters into the aorta
- Not compatible with life unless a shunt is present to allow mixing of blood (e.g. VSD, PDA, or PFO)
Describe Tetralogy of Fallot
- (a) Pulmonary valve stenosis
- (b) RV hypertrophy
- (c) VSD
- (d) Overriding aorta (sitting over VSD)
Describe tricuspid atresia
• Absence of tricuspid valve and hypoplastic RV
• Requires 2 shunts:
o ASD to get blood from RA to LA
o VSD to get blood from LV to RV so it can go to the lungs
Describe Truncus arteriosus
• Failure of truncus arteriosus to divide into pulmonary trunk and aorta
Describe total anamalous pulmonary venous return with obstruction
- Pulmonary veins drain into R heart circulation (e.g. SVC, coronary sinus, RA)
- Associated with ASD and sometimes PDA to allow for R-to-L shunting
What disorders often present with hemarthrosis (bleeding into joint spaces)
Clotting defect (e.g. Hemophilia)
Common presentation of platelet disorders
- Easy or prolonged mucosal bleeding
- Ecchymosis
- Petechiae
Lab findings in Hemophilia
Increased PTT
Describe presentation of Kartagener syndrome
Due to ciliary dyskinesia
Triad = situs inversus, recurrent sinusitis, bronchiectasis
Next step in management of infant with asymmetrical leg creases
Hip ultrasound
What are the TORCH infections
T = Toxoplasmosis O = Other (Varicella, Parvo) R = Rubella C = CMV H = HSV, HIV S = Syphilis
Presentation of congenital Rubella
Cataracts, sensory-neural deafness, PDA, jaundice, blueberry muffin rash, mental retardation, microcephaly
Presentation of congenital HSV
Skin vesicles, chorioretinitis (serpiginous corneal ulcers), microcephaly
Presentation of congenital CMV
Blueberry muffin rash, hepatosplenomegaly, jaundice, sensorineural hearing loss, ventriculomegaly, calcifications on ventricles, mental retardation, seizures
Presentation of congenital varicella
Limb hypoplasia, cutaneous scarring in dermatomal pattern, blindness
Presentation of congenital parvovirus
Hydrops fetalis
Presentation of congenital Toxoplasmosis (triad of sx)
(1) Intracranial calcifications, (2) hydrocephalus and seizures, (3) chorioretinitis, deafness
What is the standard diagnosis of HSV in neonates
HSV PCR of the CSF
How do you prevent transmission of HSV in woman with know genital HSV
C-section if lesions present at time of delivery
Daily antiviral therapy has not been shown to prevent neonatal infection. Surveillance cultures are not recommended
Tx of neonate with suspected herpes infection
Hospitalize, obtain HSV surface and vesicle fluid cultures and CSF for HSV culture and PCR
Presentation of esophageal atresia
• Most common type is esophageal atresia with distal tracheoesophageal fistula
o Aka blind upper esophagus with lower esophagus joined to trachea
- Excessive oral secretions, choking and coughing
- Polyhydramnios
- Air in stomach (due to tracheoesophageal fistula)
Describe “H-type” tracheoesophageal fistula
- Esophagus connects to stomach, but there is also a fistula between esophagus and trachea
- Often presents later in life with recurrent aspiration pneumonia or feeding difficulty
What is transient tachypnea of the newborn
o Slow absorption of fetal lung fluid with resultant tachypnea
o Commonly associated with C-section deliveries
Tx of transient tachypnea of the newborn
o supportive care including supplemental O2 if necessary
Describe neonatal respiratory distress syndrome
o Condition seen in newborn infants resulting from surfactant deficiency o Imaging (CT) reveals characteristic reticulonodular “ground glass” pattern
Tx of meconium aspiration syndrome
♣ No tx necessary in a vigorous infant with good apgar scores
♣ Endotracheal intubation with direct suctioning
♣ Bag-mask ventilation or endotracheal intubation without suction may increase volume of meconium aspirated
What is strabismus
- Misalignment of the visual axes
* Can result in the loss of vision
What is amblyopia
• Decrease or loss of vision caused by underuse of one eye (deprivation amblyopia) or lack of clear image projecting on the retina (strabismic amblyopia)
Diagnose: excessive tearing and photophobia, exam finding of corneal enlargement
Congenital glaucoma - requires immediate need for ophtho eval
Tx likely surgical
