Peds 3 Flashcards

1
Q

MOA of Phenobarbital in treating jaundice

A

Phenobarbital induces UGT, thus increasing conjugation of bili

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2
Q

Most common cause of baby born with poor feeding, tachypnea, hypothermia, and poor perfusion

A

Group B Strep pneumonia

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3
Q

How do you prevent GBS infection in a newborn

A

Screen mothers at 35 weeks gestation, and give IV Penicillin during labor to those with high risk or positive GBS

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4
Q

Most common organisms of early-onset neonatal sepsis (within 6 days of life)

A

GBS (#1) , E. Coli, H. flu, Listeria monocytogenes

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5
Q

Tx of early onset neonatal sepsis

A

Abx directed at most common pathogens - usually combo of IV aminoglycosides and Penicillin

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6
Q

Tx of late onset neonatal sepsis (7-90 days of life)

A

Beta-lactamase resistant abx (such as vancomycin) and often 3rd generation cephalosporin

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7
Q

What is greatest risk factor for neonatal sepsis

A

Prematurity (#1)

Additional risk factors include low birth weight, rupture of membranes >18 hrs before deliver, young maternal age, initial apgar <5

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8
Q

Describe congenital adrenal hyperplasia

A

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)

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9
Q

Describe difference in presentation of 21 a-hydroxylase deficiency and 11 b-hydroxylase deficiency

A

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

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10
Q

Definition of hermaphroditism

A

Discrepancy between gonad morphology and external genitalia

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11
Q

Describe 5 alpha-reductase deficiency

A

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

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12
Q

Describe true hermaphroditism vs. pseudohermaphroditism (female and male)

A

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)

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13
Q

What serum level should you check for in a baby with suspected CAH

A

Increased 17-hydroxyprogesterone

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14
Q

What is the classic disease associated with female with primary amenorrhea

A

5 alpha-reductase deficiency (46, XY with female phenotype)

Decreased androgen binding or androgen insensitivity

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15
Q

Describe difference in obstruction in meconium ileus vs. Hirschsprung disease

A

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

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16
Q

What are the 5 T’s that cause R-to-L shunt (early cyanosis

A
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)
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17
Q

Describe transposition of the great vessels

A
  • 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)
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18
Q

Describe Tetralogy of Fallot

A
  • (a) Pulmonary valve stenosis
  • (b) RV hypertrophy
  • (c) VSD
  • (d) Overriding aorta (sitting over VSD)
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19
Q

Describe tricuspid atresia

A

• 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

20
Q

Describe Truncus arteriosus

A

• Failure of truncus arteriosus to divide into pulmonary trunk and aorta

21
Q

Describe total anamalous pulmonary venous return with obstruction

A
  • 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
22
Q

What disorders often present with hemarthrosis (bleeding into joint spaces)

A

Clotting defect (e.g. Hemophilia)

23
Q

Common presentation of platelet disorders

A
  • Easy or prolonged mucosal bleeding
  • Ecchymosis
  • Petechiae
24
Q

Lab findings in Hemophilia

A

Increased PTT

25
Describe presentation of Kartagener syndrome
Due to ciliary dyskinesia Triad = situs inversus, recurrent sinusitis, bronchiectasis
26
Next step in management of infant with asymmetrical leg creases
Hip ultrasound
27
What are the TORCH infections
``` T = Toxoplasmosis O = Other (Varicella, Parvo) R = Rubella C = CMV H = HSV, HIV S = Syphilis ```
28
Presentation of congenital Rubella
Cataracts, sensory-neural deafness, PDA, jaundice, blueberry muffin rash, mental retardation, microcephaly
29
Presentation of congenital HSV
Skin vesicles, chorioretinitis (serpiginous corneal ulcers), microcephaly
30
Presentation of congenital CMV
Blueberry muffin rash, hepatosplenomegaly, jaundice, sensorineural hearing loss, ventriculomegaly, calcifications on ventricles, mental retardation, seizures
31
Presentation of congenital varicella
Limb hypoplasia, cutaneous scarring in dermatomal pattern, blindness
32
Presentation of congenital parvovirus
Hydrops fetalis
33
Presentation of congenital Toxoplasmosis (triad of sx)
(1) Intracranial calcifications, (2) hydrocephalus and seizures, (3) chorioretinitis, deafness
34
What is the standard diagnosis of HSV in neonates
HSV PCR of the CSF
35
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
36
Tx of neonate with suspected herpes infection
Hospitalize, obtain HSV surface and vesicle fluid cultures and CSF for HSV culture and PCR
37
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)
38
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
39
What is transient tachypnea of the newborn
o Slow absorption of fetal lung fluid with resultant tachypnea o Commonly associated with C-section deliveries
40
Tx of transient tachypnea of the newborn
o supportive care including supplemental O2 if necessary
41
Describe neonatal respiratory distress syndrome
``` o Condition seen in newborn infants resulting from surfactant deficiency o Imaging (CT) reveals characteristic reticulonodular “ground glass” pattern ```
42
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
43
What is strabismus
* Misalignment of the visual axes | * Can result in the loss of vision
44
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)
45
Diagnose: excessive tearing and photophobia, exam finding of corneal enlargement
Congenital glaucoma - requires immediate need for ophtho eval Tx likely surgical