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119 Cards in this Set

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Syndrome of microcephaly, smooth philtrum, small palpebral fissures, short nose, and cleft lip/palate
FAS
syndrome of protruding tongue, brushfield's spots, redundant neck skin, brachycephaly, small ears, clinodactyly of 5th digit
Trisomy 21 (DS)
Syndrome of midface hypoplasia, low nasal bridge, ocular hypertelorism, accentuated cupids bow of upper lip
Dilantin exposure
Syndrome of X-linked hematuria, progressive nephritis
Alport's syndrome
infants of diabetic mothers have increased risk for what pathologies:
cong.heart dis, caudal regression syndrome, small left clon, LGA, hypoglycemia and hypocalcemia
Factor associated with ncreased neurological damage due to jaundice in newborn?
neonatal sepsis
Sulfisoxazole mechanism of decreasing jaundice in newby
compete with BR for binding sights on albumin; thus cause dislocation of bilirubin from tissues
factors reducing binding of BR in brain in jaundiced newby?
Met acidosis, Sulfisoxazole tx, phenobarbitol (INDUCES g transferase) hyperalbuminemia causing increased binding of unconj BR to album and decr free unconj BR.
newby has cyanosis +apnea, worse with feeding, better w/crying NS in M?
nasal catheter to check for choanal atresia
nwby has inspiratory stridor worse with feeding, next step?
flexible bscopy to check for laryngeo/tracheomalacia
M/C cause of stridor in infant < 2 mo?
congenital stridor = M/C = laryngomalacia
M/C cause of stridor in 3mo-3y/o
Croup = parainfl, infl, or RSV
treatment of non-severe stridor?
no tx; outside, cool air only (+/- mist)
tx of severe stridor in ER?
IM steroids, nebulized epinephrine. Do NOT depress tongue or check for gag reflex! may cause bspasm and child may die!
causes of cong stridor <2mo?
(laryngeomalacia >>>subglottic stenosis, vascular rings, v/c abnormality)
all causes of non-cong stridor ie in 3mo-3y/o?
infection w/Para, Infl, or RSV, FBA, retropharyngeal abscess, etc
2nd child to CMV mother at risk for congenital CMV?
No - by then mom has AB's that will cross placenta if she gets infection
m/c cause of congenital infection?
CMV
syndrome of HSM, splenomegaly, jaundice, petechiae, purpura, microcephaly in MOM
CMV
% of toddlers becoming seropositiv CMV in day/care /
up to 80%
low apgars, meconium stain, and resp distress suggest what has occurred?
asphyxia
C/I's to breastfeeding?
Lithium, active TB or HIV, ACUTE Hep B, neoplastic drugs, Tetx. NOT mastitis, engorgement, cracked/bleeding nipples, other AB's, or URTI in baby
When administer VZIG (immunoglobulin) to baby?
only if MOM had VZV inf w/in 5 d before deliv or w/in 2 d after deliv. If baby exposed during this time, also consider Acyclovir
Hematemesis or melena in newby: NSiM?
Apt test to check if blood is babys or mom's; if mom's no tx.
NSiM of clinically suspected NEC in preme?
Stop feeds, start IV fluids, order serial abd films, initiate systemic AB's
Preme's on breastmilk will develop what?
hypocalcemia! must supplement bmilk or form
Primiparous mom gives birth to infant w/BR 12 mg/dL at age 36hr, and hematocrit 55%?
ABO hemolytic disease (Mom O, baby A or B or AB) (not PCV b/c must have >65% for PCV)
SGA more likely to have?
congen malformations, future growth retard,neonatal asphyxia +meconium aspiration syndrome --> pthorax, pmediastinum, or pulm hemorrhage, hypoglycemia, increased hct. NOTE: LGA also may have meconium+asp due to stress of birth, as well as old placenta causing hypoxia--> [PCV with incr hematocrit and hypoglycemia due to low glycogen stores]
PREME'S more likely to have?
hyalin membrane dis (RDS),
Apnea def?
absence of respirations >20secs
apnea in preme while feeding m/c due to?
incompleteley developed respiratory center
other causes of apnea?
OSA, seizures, hypoglycemia, pulm disease (pneum, RSV, etc),
recurrent breathing pauses of 3-10sec in low-birth-wt babies?
Periodic breathing
untreated cong hypothyroidism will cause?
cretinism = retardation, poor growth, etc.
85% of cong hypothyroidism caused by?
sporadic thyroid dysgenesis
Who screened for cong hypothyroidism?
ALL babies!! tx must be given immediately if found.
1 week old infant with R clavicle mass, suspect?
clavicular fracture from birth
2 types of problems may occur due to injury at birth to brachial plexus?
Erb-Duchenne palsy (cant abduct or ext rotate arm = c5,c6), phrenic nerve paralysis = undescending diaphragm on xray and cause of resp problems); C7,8, T1 injuries causing paralysis of hand and possible Horner's... these often spont resolve;
3 Newby head fluid findings and differentiation?
1. Sbgaleal/subaponeurotic hemorrhage = squishy fluid filled, crossing midline or throughout entire scalp, fluid wave. Life threatening, may lose 1/3 bv and to it and cause hov shock.
2. Caput (edema) Succedaneum= firm, nonfluent, may cross midline.
3. Cephalohematoma = under periosteum of skull bones, so stops at suture lines; feels fluid-filled/fluctuant.
Mg sulfate given to preg mother for?
for constipation, calf cramps, preterm contraction without cervical effacement
Normal blood glucose in FT newby first day?
30 mg/dL
pregnancy HTN may cause what in fetus?
decreases uteroplacental blood flow -> fetal hypoxemia -> dec glycogen storage and increased EPO release in fetus0--> infants born w/ hypoglycemia and PCV
PCV def in neonate?
persistent venous Hct of >65%
Effects of PCV in neonate?
Hyperviscosity syndrome: jitters, sludging/thrombi in renal veins, NEC, tachypnea
Tx of PCV in infant?
partial exchange transfusion of saline / dilutional transfusion
Tx of lethargic infant born to mother given local narcotic (meperidine) anasthesia?
Naloxone; IM, IV, or intratracheal
Birth with pea-soup amnio fluid to 42 w mom, with apneic, pale, limp baby: immediate NSiM?
This is a post-term baby and may have chronic placental insufficiency (=LBW for gest age and wasted appearance); has higher risk for asphyxiation from meconium. Thus, must suction trachea (with n/g tube, or laryngoscope if baby is already needing intubation). Do not ventilate immediately as it may push meconium further into smaller airways.
Silver nitrate or erthromycin prophylaxis in newby may cause?
chemical conjunctivitis w/in 6-12 hrs of birth
Most serious bact eye infection of newbies?
Gon. conjunctivitis
Gonococcal conjunctivitis presentation/ sx?
2-5 days post birth, puffy tense eyelids, red conj., copious purulent discarge, chemosis (swelling of conj)---> may lead to blindness
Tx of Gon. conjunctivitis?
Prompt topical (e-mycin)AND IV AB's (cefotaxime)
Chlamydial conjunctivitis (trachoma) presentation/sx?
5-14 days post-birth. mucopurulent d/c, conj redness,--> +/- chlamydial pneumonia, corneal scarring (eyelids turn inwards and scratch cornea)
Ophthalmia neonatorium 3 m/c causes
Gon, Chlamydia trachomatis, chemical
Chlamydial conj tx?
Oral + topical e-mycin
Appropriate meds for delivery for HIV + mother?
1. Pre-delivery tx (weeks) w/antiretroviral;
2. Zidovudine for infant directly after birth
Does IV Ig to newby prevent HIV transmission from HIV + mom?
No
How to tell Turner syndrome at birth?
Low birth weight, edema over dorsum of hands and feet, and redundant skin folds at nape of neck
Other findings in later life of Turner's syndrome?
Sexual infantilism, streak gonads, Coarctation of aorta, hypertension, and bicuspid ao valve, horseshoe kidney, high palate
Findings in infant of coarse, tremulous movements, harlequin color change (dependent half red, other half white, craniotabes, palpable liver?
All these are found frequently in NORMAL infants. NO dx significance.
Sharply demarcated, hard red lesion on cheeks, buttocks, limbs?
Sub-cut fat necrosis (SCFN) = self-remitting, non-recurring
2 signs of diaphragmatic hernia?
1) scaphoid abdomen
2) passage of n/g tube goes down then up into thorax!
Type of TE fistula dx'd later by multiple recurrent pneumonias ?
H-shaped/type
M/c TE fistula?
Upper Esoph - Trachea fistula with blind end of lower esoph.
Failure to give Vit K may cause what in infant?
Hemorrhagic Dis of Newby: increased umbilical, GI, venipuncture, and circumcision bleeding ; incr PT time
Bleeding time in Hem Dis of Newborn?
Normal
% of Hep B pts getting cirrhosis? % of Hep B infants developing cirrhosis
10% ; 90%!
Preg mom takes heparin for dvt...does heparin cross placenta?
No
preg mom takes Phenytoin. does phenytoin cause birth problems?
Yes if taken 1st trimester
Preg mom takes penicillin for Rheumatic Disease...does penicillin cross placenta or hurt baby?
No
Preg mom takes Aluminium hydroxide for acid reflux...does it harm the baby?
no
Preg mom takes propanolol. Can it harm the baby?
YES - may cause IUGR, may cause low HR and inability to increase CO in asphyxiated baby
Bilious regurg at 6 hrs old?
duod atresia
Bilious vomit at 3-4 days old?
Midgut volvulus
Non-bilious P-rojectile vomiting in 3wk-few month old?
Pyloric stenosis
Goat's milk for infants has inadequate what?
Folate, iron
Unpasteurized goats milk for infants maycause?
brucellosis!!
Jaundice sets in at what BR levels in neonates? kids?
>5mg/dL in neonates, >2mg/dL
in kids
Conjugated hyperbilirubinemia is always path or non-path?
Pathological!
Why in preme-s is there greater risk for kernicterus at much lower levels of unconj BR?
Their BBB's are even less mature = increased crossing of unconj BR
Lethargy, irritability, hypotonia, opisthotonos (arched, hyperextension of full body, seizures, mental retardation, CP, deafness may all be due to what?
Kernicterus
Physiologic J of Newborn begins when, peaks when, and is caused by what?
Begins @ 24hrs, peaks @ 3rd day of life; caused by:
1) Incr. fetal RBC load + decr. fetal RBC survival time + increased enterohepatic circulation = INCREASED BREAKDOWN of RBC's by fetal macrophages in RE system
2) Immature liver = defective hepatic uptake and conj. of BR = DECREASED UPTAKE/CONJ
3) Immature liver = DECREASED BR excretion from liver
Definitions of pathological jaundice? (jaundice that must be evaluated) (4)
1) J at birth or in first 24hrs +
2) Increase in total BR by > .5mg/dL or >5mg/dL/day +
3) Total BR > 19.5
4) ANY conj hyperbilirubinemia ie direct BR > 2.0 mg/dL
5) prolonged jaundice >1wk FT or 2wks preme
What is breastmilk jaundice?
Incr. glucuronidases in breastmilk causing incr. b/d of conj. into unconjugated BR = INCREASED ABSORPTION=INCREASED ENTEROHEPATIC CIRC. ; is a pathological condition
Unconj pathological jaundice ddx?
1-ABO/Rh hemolytic dis of nb
2-hereditary sphero/elliptocyt.
3-drug rxn
4-Hbopathy (Sickle, a, b-thal,
5-DIC/Sepsis
6-PCV
7-cephalohematoma/bruising at birth
8- incr. e-hep circ ie intestinal obstruction, ilius
9- Bmilk jaundice
10-Gilbert';s, C-Najjar
11-Hothyroid, hypopit,
12-Enzyme def ie G6PD, PK def
Conjugated jaundice ddx?
1-Obstructive: Biliary atresia, chlithiasis, chdochal cyst,common duct stenosis, pcitis
2- persistent intrahepatic cholestasis
3- acquired intrahepatic cholestasis from hepatitis, VZV, EBV, CoxV, cirrhosis, drug induced, neoplasms
4- BR metabolism defects ie Dubin Johnson's (BR secretion defect), Rotor's (similar), galactosemia, disorders of AA, carb, lipid (Niemann-Pick's, Gaucher's), trisomy 18 and 21, metabolic (Wilson's, a-antitrypsin def
Bodily assessment of BR levels?
when reached umbilicus = 10mg/dL
when reached palms/soles > 15mg/dL
Initial eval of jaundice?
1) CBC (for Hct) + periph blood smear (for sph/elliptos) and reticulocyte ct,
2) determination of maternal and fetal blood types
3) Coomb's test (direct + indir)
4) BR levels unconj and conj.
What is breast-feeding jaundice?
Poor breastmilk production (ie after c-section) causes inadequate bowel movements = unable to remove BR from body; gets reabsorbed
Main clinical differentiation of path jaundice from physiological jaundice?
presence of intrauterine retardation, stigma of intrauterine infections (e.g. cataracts, microcephaly, hepatosplenomegaly etc), cephalhematoma, bruising, signs of intra ventricular hemorrhage etc.; or family history, maternal drugs
Cleft lip + palate often has what other ENT complications?
recurrent otitis media and hearing loss and speech difficulty
Cleft lip repaired when? cleft palate when?
3mo ; 5mo-5yrs old
large head, large fontanelles, + umbilical hernia and constipation at birth?
Congenital hypothyroidism
Transient tachypnea of Newborn caused by?
retained fetal lung fluid
TTPN clinical presentation, cxray findings, and tx?
grunting, tachypnea, retractions in FT BABY, cxr: fluid in fissures, prominent pulmonary vascular markings, and flattened diaphragms tx = supportive/ maintain O2sat
Normal birth baby that develops into m/p peeling rash on face, palms, and soles, snuffles (serous d/c from nose +/- blood), persistent rhinitis, and hepatosplenomegaly?
Congenital syphilis
More obvious signs of congenital syphilis?
Keratitis (acute inflam of cornea --> scarring --> blindness), hutchinson's teeth, mulberry molars, saddle nose (caused by persistent rhinitis!)
Polydactyly that requires further investigation/ chrom studies?
thumb (preaxial)- sided. Assoc w/ Trisomy 13 (patau's) and other genetic diseases
syndrome of mental & motor challenged, polydactyly (extra digits), microcephaly, low-set ears, holoprosencephaly (failure of the forebrain to divide properly).
heart defects
structural eye defects, including microphthalmia, Peters anomaly, cataract, iris and/or fundus (coloboma), retinal dysplasia or retinal detachment, sensory nystagmus, vision loss,
cleft palate, cleft lip, meningomyelocele, omphalocele, abnormal genitalia, abnormal palm pattern
overlapping of fingers over thumb. cutis aplasia (missing portion of the skin/hair), prominent heel, kidney defects, rocker-bottom feet
Patau's syndrome (Trisomy 13)
recommended folate / day for preggers?
400 ug /day
newby with jitters, tremors, vomiting, diarrhea, and irritability?
Born to narcotics user: w/drawing from heroin!
Idiopathic apnea common and expected in?
Preme's; is NOT common and should be reason for concern in full-terms
Apnea in FT newby ddx?
sepsis, gerd, CongHD, seizures, hypoglycemia, OSA (a/w obstruction)
In addition to birthweight decreasing in first few days / week of life, what else also decreases in newby in this time?
Hct also decreases as a response to increased O2. During this time the BR also increases.
polyhydramnios immediately suspect? (3)
1) TE fistula
2) duodenal atresia
3) trisomy 18
4) anencephaly

*T18=micrognathia, low set ears, microceph, coloboma, CHD, clenched hands, crossed legs, pectus carinatum
think: 18=Election age
Ears low set
Edwards
VS:
Patau's (T-13) = Pataus
Puberty age
Polydactyly
Rocker bottom feet
oligohydramnios suspect?
renal agenesis or GU obstruction
petechia and jaundice on 2nd day of life, think?
Congenital INFECTION +sepsis! ie syphilis, toxo, CMV, Rubella
Workup for petechia +jaundice in 2nd day of life
Bculture, Viral cultures, LP, Xrays of long bones (cong rubella has longitudinal striations in metaphyses...syphilis has osteochondritis or periostitis
PLUS ISOLATE INFANT since these syph, cmv, or rubella are all highly contagious
bilateral cateracts, microphthalmia, IUGR, hmorrhagic skin lesions and systolic murmur
Congenital RUBELLA !
week old infant with fever and focal seizure?
congenital HSV!
newby with **hydrocephalus, chorioretinitis, intracranial calcifications, and anemia
congenital TOXO!!
Newby with **microcephaly, intracranial calcifications, chorioretinitis, hepatomegaly, and marked hyperbilirubinemia and thrombocytopenia
congenital CMV!!
Only TORCH infection causing heart defects?
cong Rubella!
most common congenital infection?
cong CMV
preme's with persistent O2 requirements, respiratory difficulties, abnormal cxrays, and often requiring diuretics
BPD
lethargy, vomiting, diarrhea, hypotonia, jaundice, hepatomegaly, FTT and cataracts
galactosemia
tx of galactosemia
removal of lactose from diet (ie give soy milk)
PKU tx?
Special formulas w/ low phenylalanine producing agents
dermatitis, alopecia, ataxia, hypotonia, seizures, dev delay, immunodeficiency and met acidosis?
biotinidase deficiency (autosomal recessive trait)
aniridia, hemihypertrophy, and flank mass?
wilm's tumor