60% of newborns are jaundiced b/c spleen removes excess RBCs carrying HbF -> hemolysis -> high blood Hb -> high bilirubin
Transient hyperbilirubinemia of the newborn
when is jaundice in the newborn considered pathologic
Any of the following:
1) if appears on 1st day of life or after 1st 2 wks or persists after 2 wks
2) total bilirubin >5 mg/dL/day or >0.5 mg/dL/hr
3) total bilirubin >15 mg/dL
4) direct bilirubin >10% of TB
5) Persists beyond 1 week in term and 2 weeks in preterm
whats the most serious complication of pathologic jaundice in the newborn
kernicterus
how to treat pathologic jaundice in the newborn
- phototherapy w/ blue-green light (12-20 mg/dL; 270 nm breaks down bilirubin to prevent kernicterus, toxic to retina)
- exchange transfusion: if bilirubin >20-25 mg/dL
Icteric sclera, bilirubin level
> 2 mg/dL
AKA retrolental fibroplasia; ↓ vascularity of retina
ROP
Absence of UGDP causing an increase in indirect bilirubin. These infants present in the first 24 hrs and die within the 1st yr of life.
Crigler Najjar
Milder form of Crigler Najjar
Gilbert
___–>↑ UGDP enzyme–>↑ conjugation of bilirubin–>↓ unconjugated bilirubin; can help ↓ neonatal jaundice
Phenobarbital
Peak B1 at 12 mg/dL at 3rd DOL or 15 mg/dL at 5th DOL for premature infants
Physiologic jaundice
Criteria for physiologic jaundice
1) after 48 hrs of life 2) TB not increasing > 5 mg/dL/day 3) DB less than 10% of TB 4) Resolves by 1 week in term and 2 weeks in preterm
Jaundice is ALWAYS pathologic if
Bilirubin >15mg/mL; at first day of life
What are the two inborn disorders of metabolism that lead to an UNCOJUGATED hyperbilirubinemia?
Gilbert’s and Crigler-Najjar
Physiologic hyperbilirubinemia is seen ___, peaks at ___, and resolves over ___
After the first 24 hours of life, 3 days, 2 weeks
What are the Danger Signs in Jaundiced Infants?
(1) Family history of significant hemolytic disease, (2) Vomiting, (3) Lethargy, (4) Poor feeding, (5) Fever, (6) Onset of jaundice after the third day, (7) High-pitched cry
MCC of jaundice?
Unconjugated hyperbilirubinemia (indirect): physiologic and breastfeeding associated
Management for breast milk jaundice
stop breast feeding for 2-3 days, using formula instead; then resume nursing
Type of bilirubin increased in breast milk jaundice
Unconjugated
Onset of breastfeeding jaundice
3-4 DOL
Onset of breastmilk jaundice
7th DOL
Increases risk of physiologic jaundice
Preterm, diabetic mother, asian
Bilirubin levels drop rapidly when breastfeeding stops
Breastmilk jaundice
Causes of pathologic jaundice
1) Extrahepatic cholestasis (biliary atresia, choledochal) 2) Intrahepatic cholestasis (neonatal hepatitis, inborn errors of metab, TPN cholestasis) 3) Dubin-Johnson 4) Rotor 5) TORCH
To reduce incidence of breastfeeding jaundice
Frequent breastfeeding (>10/24h), discourage 5% dextrose or water
Bilirubin level if breastfeeding is continued in breast milk jaundice
Gradually decreases but may persist up to 10 weeks at lower levels
Type of bilirubin increased in breastfeeding jaundice
Unconjugated
Metabolic causes of direct hyperbilirubinemia
DiRect: Dubin Johnson and Rotor
Enzyme responsible for conjugation in liver
UDP glucuronyl transferase
Inherited form of non-hemolytic jaundice
Crigler-Najjar syndrome
More severe type of Crigler-Najjar syndrome
Type I
Crigler-Najjar syndrome, arias syndrome
Type II
Autosomal recessive disorder that causes increase of conjugated bilirubin without elevation of liver enzymes
Dubin-Johnson
MC hereditary cause of increased bilirubin
Gilbert
Non-itching jaundice
Rotor
Mechanism of phototherapy for jaundice
Geometric photoisomerization of unconjugated bilirubin (lumirubin)
Inhibits heme oxygenate hence may be used in treatment of hyperbilirubinemia
Metalloporphyrins
In exchange transfusion ___% of circulating RBCs is replaced when an equivalent of 2 neonatal blood volumes is used
85%
T/F Hyperbilirubinemia in neonates is benign in most cases
T
Absence or reduction in number of bile ducts; results from progressive destruction of the ducts
Alagille syndrome
Jaundice, approximate levels for involvement: 4 to 8 mg/dL
Head and neck
Jaundice, approximate levels for involvement: 5-12 mg/dL
Upper trunk
Jaundice, approximate levels for involvement: 8-16 mg/dL
Lower trunk and thighs
Jaundice, approximate levels for involvement: 11-18 mg/dL
Arms and lower legs
Jaundice, approximate levels for involvement: 15 mg/dL
Palms and soles
Unconjugated hyperbilirubinemia (indirect): When an infant’s indirect (unconjugated )serum bilirubin level is > \_\_\_
10 mg/dL
Serious sequelae of NEC
Intestinal strictures, malabsorption, fistulae, and short bowel syndrome (in case of surgery)
Caused by proliferation of immature retinal vessels due to excessive use of oxygen; Can lead to retinal detachment and blindness in severe cases
ROP
Male vs female infants, more susceptible to neonatal infections
Male
Sepsis that may be seen from birth to 7 days, transmitted vertically or from the mother’s genital tract
Early-onset
Sepsis that may be seen from 8-28 days, transmitted vertically or from the postnatal environment
Late-onset
Sepsis with a fulminant course and associated with multi system pneumonia
Early-onset
Sepsis with a more insidious course and associated with focal infection (meningitis common)
Late-onset
Common risk factor for early and late onset sepsis
Prematurity
Sepsis that occurs most commonly among low birth weight infants
Nosocomial sepsis
Transplacentally transmitted infections
CMV, rubella, listeria, T. pallidum
Route of vertical transmission
Ascending or from passage through birth canal
Predominant pathogens in late-onset sepsis
S. aureus, coagulase negative staph, fungal infection, enterococcus, G- enteric bacilli
what are the TORCH organisms in congenital infections
- T: toxoplasmosis
- O: others; like syphilis, HepB, varicella
- R: rubella
- C: cytomegalovirus
- H: herpes simplex
presentation of congenital toxoplasmosis
TRIAD:
- chorioretinitis
- hydrocephalus
- multiple ring-enhancing lesions on CT (calcification)
how to dx congenital toxoplasmosis
- IgM to toxoplasma(initial)
- PCR(accurate)
how to treat neonatal sepsis
-ampicillin + gentamicin
What is the main cause of Gram (-) sepsis and meningitis in the newborn?
E. coli
how to treat congenital toxoplasmosis
-pyrimethamine & sulfadiazine (with leucovorin for 1 year)
___ is given to patients treating with toxoplasmosis to counteract bone marrow depression in patients taking pyrimethamine and sulfadiazine
folinic acid
during what trimester is toxoplasma causes severe disease
1st trimester
test for toxoplasmosis
sabin felman dye test
Only in ___ can T. gondii complete its life cycle and produce oocysts
Cats
Transmission of T. gondii
Transplacentally during first trimester (17%, more severe); 3rd trimester (65%, transplacental or vaginal delivery, mild or inapparent)
Asymptomatic patients with toxoplasmosis are still at high risk of developing abnormalities especially ___
Chorioretinitis
T/F Treatment with TMP-SMX in toxoplasmosis will eradicate encysted parasite
F
- rash on palms & soles
- snuffles
- frontal bossing(prominent forehead)
- hutchinson triad: 8th nerve deafness + interstitial keratitis(corneal scarring) + hutchinson’s teeth
- saddle nose
Congenital syphilis
how to dx congenital syphilis
- VDRL/RPR (initial)
- FTA-ABS/dark field microscopy (accurate)
Olympian brow
-congenital syphilis: Frontal bossing
Higoumenaki’s sign
-congenital syphilis: Unilateral or bilateral thickening of the sternoclavicular portion of the clavicle
Mulberry molars
-congenital syphilis: Abnormal 1st lower molar
Rhagades
Congenital syphilis - linear scars on mouth, anus, genitals
Clutton joint
Congenital syphilis - painless knee joint swelling with sterile synovial fluid
Pseudoparalysis of Parrot
Congenital syphilis - Refusal to move involved extremity
Earliest manifestation of congenital syphilis
Snuffles
Transplacental transmission of syphilis can occur as early as as
6 weeks AOG
Phase of maternal syphilis wherein infection can most likely be transmitted
Primary or secondary, rather than latent disease
T/F Majority of affected live-born infants who have congenital syphilis are asymptomatic at birth
T
Early signs of congenital syphilis appear during
First 2 years of life
Early signs of syphilis is analogous to ___ phase of acquired syphilis
Secondary
Late signs of syphilis appear during
First 2 decades of life
Early vs late sign of syphilis: Periostitis of long bones
Early
Early vs late sign of syphilis: Renal involvement
Early
Early vs late sign of syphilis: CNS involvement
Early
Early vs late sign of syphilis: Mucous patches and condylomatous lesions
Early
Late signs of syphilis
Olympian brow, Saber shin, Hutchinson teeth, mulberry molars, saddle nose, rhagades, juvenile paresis, juvenile tabes, 8th nerve deafness, Clutton joint
Teeth manifestations of syphilis erupt when
6 y/o
Asymptomatic infants considered at risk for congenital syphilis should be evaluated if
1) Maternal treatment was inadequate, unknown, or undocumented 2) Maternal treatment was less than or equal to 30 days before delivery 3) Mother was treated with erythromycin or neopenicillin regimen 4) Maternal treponemal tigers did not decrease sufficiently to demonstrate a cure four-fold or greater
T/F Varicella infection is a contraindication to breatfeeding
T
Treatment for immunocompromised child/newborn exposed to varicella
VZIG
if the mother has varicella __ days prior to delivery she may pass the virus to the child but it is attenuated since there is Ab of the mother passed also.
more than 5 days
maternal varicela IgG can pass through the placenta at what aog
by 30 wks
neonate with cicatrical skin scarring with limb hypoplasia and neurologic manifestation
congenital varicella syndrome
varicella vaccine is given when
total of 2 dose on 12-15 months and 4-6 year old
pag catch up na less than 12 – 2 dose 3 months apart and pag more than 12, 2 dose na 1 months apart
new borns whose mother has varicella 5 days before delivery or 2 days after delivery should be given
1 vial of Varicella Ig
Mode of admin of varicella vaccine
SC
Content of varicella vaccine
Cell-free, live attenuated varicella virus
Vaccine contraindicated in patients with anaphylactic reaction to vaccine, neomycin, or gelatine and those with altered immunity
Varicella vaccine
Congenital Varicella Syndrome is caused by maternal varicella infection in ___ weeks of pregnancy
first 20 (VZV embryopathy) or last 3
T/F Maternal varicella may cause premature delivery and miscarriage
T
Congenital varicella causes ___ during 6-12 weeks AOG
Interruption of limb development
Congenital varicella causes ___ during 16-20 weeks AOG
Eye and brain involvement
T/F Varicella can congenitally cause ophthalmologic problems including Horner syndrome
T
Treatment for infants with severe varicella
Acyclovir
T/F, VZIG is not beneficial after clinical disease has developed
T
Neonate
- microcephaly
- cataracts
- sensorineural hearing loss
- hepatosplenomegaly
- thrombocytopenia
- blueberry muffin rash
- hyperbilirubinemia
Congenital rubella
Congenital heart disease associated with rubella
PDA (other: pulmonary artery stenosis
how to treat congenital rubella
supportive
Dx test Rubella
Maternal IgM in neonatal serum
in congenital rubella infection, what trimester results in severe defects (congenital rubella SYNDROME)
1st 8 wks of pregnancy
Majority of infants acquire maternal rubella infection before ___ weeks
11
MC clinical manifestation of rubella
Sensorineural hearing loss (then cataracts and glaucoma)
Neonate
- intracranial (periventricular) calcifications
- microcephaly
- chorioretinitis
- hearing loss
- petechiae
CMV (cytomegalic inclusion disease)
how to dx congenital CMV
- urine & saliva viral titers (initial)
- urine/saliva PCR (BEST)
how to treat congenital CMV
-ganciclovir: if signs of end organ damage (not for asymptomatic infants due to side effects)
Occurs when a mother has a primary CMV infection in the 1st trimester of pregnancy–>death of baby; greater risk of feral infection
Symptomatic congenital disease
Occurs in the event of reactivation of CMV infection during pregnancy, maternal IgG passes transplacentally, protecting the infant from serious infection; less risk of feral infection
Asymptomatic congenital disease
MCC of congenital infection
CMV
MC manifestation of CMV
Chorioretinitis
T/F Majority of infants with congenital CMV are asymptomatic at birth
T
T/F Majority of congenital CMV develop long term complications (learning and hearing deficits)
T
___ antibody test is of little diagnostic value in congenital CMV because it also reflects maternal Abs
IgG
Negative ___ antibody test excludes diagnosis of CMV
IgG
___ antibody test lacks sensitivity and specificity and unreliable for diagnosis of congenital CMV
IgM
Neonate
- skin eye mouth infections
- CNS/systemic infection
HSV
Treatment for congenital HSV
-acyclovir + supportive (14 days if limited to skin, eye, and mouth; 21 days if disseminated or localised in the CNS)
CMV transmitted intrapartum, through infected blood or through breast milk, is not associated with ___ deficits
neurologic
how to prevent conjunctivitis in a newborn (ophthalmia neonatorum) & who takes this precaution
- erythromycin/tetracycline ointment + silver nitrate solution
- given in delivery room to ALL newborns
what are the 2 most common causes of ophthalmia neonatorum
- n gonorrhoeae
- chlamydia trachomatis
how to dx congenital herpes
- tzanck smear(initial)
- PCR(accurate)
presentation of congenital herpes by week
- week 1: shock & DIC
- week 2: vesicular skin lesions
- week 3: encephalitis
Conjunctivitis, Most Likely Cause if symptoms start at: Day 1- Day2-7- >7 Days- >3 weeks-
Day 1- chemical irritation
Day 2-7- Neisseria gonorrhea
>7 Days- Chlamydia trachomatis
>3 weeks- herpes
HSV is primarily transmitted through
Maternal genital tract from passage through birth canal
Plan of delivery if primary herpes is detected in the 3rd trimester
Planned CS offered
Plan of delivery if (+) herpes but no active lesions
Vaginal
Hallmark of neonatal herpes
Vesicular rash present at birth or shortly thereafter
Gold standard for diagnosis of HSV encephalitis
PCR
T/F Serology is useful in neonatal HSV
F
____ are very effective in reducing head injury by 85%
Helmets
Pedestrian injury occurs during the day and peaks on
The afterschool period
Major street should not be crossed until the child is ___ yrs old
10
Begins at birth and includes the 1st mo of life
Newborn/neonatal period
The average length and head circumference in a newborn
50 cm (20inch) and 35cm (14inch)
low birth weight infants is defined as infants weighing less than ___
2.5 kg
very low birth weight infants weighs ___
less than 1.5kg
when fetal growth stops and over time decline to less than the 5th percentile of growth for gestational age or when growth proceeds slowly but absolute size remains less than 5th percentile
IUGR
the first audible heart tones by fetoscope are detected at
18-20 weeks
___ a rare cause of vomitting in infants that is demonstrated as obstruction at the cardiac end of the esophagus without organic stenosis
infantile achalasia ( cardiospasm)
absence of rectal gas at 24 hours is abnormal. True or False?
TRUE
a common early GI sign of Hirschsprung disease
vomiting with obstipation (severe or complete constipation)
if there is increased indirect bilirubin with (+) Coombs test what is the possible diagnosis
Rh, ABO, other blood group
jaundice in breastfed infants seen after the 7th day of life with maximal concentrations as high as 10-30mg/dl
breastfeeding jaundice
breastmilk jaundice is attributed to the presence of ___ in some breast milk causing an increase in intestinal absorption of bilirubin
glucoronidase (pregnanediol: milk glucuronidase)
kernicterus results from the deposition of un conjugated bilirubin in the ____
basal ganglia and basal nuclei
what are the common initial signs of kernicterus
lethargy, poor feeding and loss of Moro
dark greyish brown skin discoloration in infants undergoing phototherapy
bronze baby syndrome
90% of Rh incompatibility is __ type
D type
in Rh incompatibility the mother is Rh __ and the infant is Rh ___
mother is Rh (-) and child is Rh (+)
injection of ____ into the mother immediately after the delivery of Rh + infant is successful in reducing Rh hemolytic disease
anti-D gamma globulin
__ is performed to determine fetal hemoglobin levels and to transfuse packed RBC in serious fetal anemia
percutaneous umbilical blood sampling
what is the treatment of choice for fetal anemia
intravascular (umbilical vein) transfusion packed RBC
what are the indications for umbilical vein transfusion
hydrops
Blue with feed
Choanal atresia
Blue with cry
Tetrology of Fallot
d/t estrogen withdrawal from mom
Newborn vaginal bleeding
Sacral hair
Spina bifida occulta
Acrochordon
Skin tag
Midline cyst
Thyroglossal cyst (thyroid comes down from tongue)
Lateral cyst
Branchial cleft cyst
Extra nipples are always on ___ line
vertical
Umbilical stump bleeding
Factor 13 deficiency
Delayed umbilical cord separation (6 wk)
Leukocyte adhesion deficiency
Congenital hip dislocation, spread both hips Out, feel for clunk
Ortolani [OUTolani]
Congenital hip dislocation, bend knee & hip, feel for clunk w/ middle finger → do US
Barlow maneuver
discrepancy btw chronological age & mental age
MR
Touch cheek → they turn toward it
Rooting
spread arms symmetrically when startled
Moro
Used to support self on a surface, “fencing reflex
Tonic-Labyrinthine reflex
what is the indication for intubation & ABG for a newborn
-if RDS or not breathing
what are the normal newborn vitals(RR, HR)
- RR: 40-60
- HR: 120-160
what is the purpose of apgar score & when is it done?
- quantifiable measure whether or not theres need for resuscitation
- 1 min after birth(evaluates labor/delivery) & 5 min after birth(evaluates resuscitation response)
is a low apgar score assoc w/ risk of cerebral palsy
no
what is the apgar criteria
- appearance(skin color): 0 = body is blue; 1 = normal except extremities; 2 = normal
- pulse: 0 = 100
- grimace(reflex irritability): 0 = no response; 1 = feeble cry; 2 = sneeze/cough
- activity(tone): 0 = none; 1 = minor flexion; 2 = active movement
- respiration: 0 = absent; 1 = weak/irregular; 2 = strong
what is the most common complication that occurs to newborns who didn’t receive routine newborn care
-vit K deficiency: example = bright red blood per rectum/urine/umbilicus or intracranial bleeding(lethargy)
does breast milk have vit K?
not enough
what in the colon flora is required to produce sufficient vit K & what clotting factors need vit K
- e coli
- 2, 7, 9, 10, C, S
what prevention routine is taken in newborns to prevent vit k def bleeding
-single intramuscular vit K
during hearing test in newborn, what is does this test exclude?
-congenital sensory-neural hearing loss: necessary for early detection to maintain speech & assess need for cochlear implants
how to dx cystic fibrosis in newborn
- sweat chloride test(initial): elevated sweat chloride
- genetic analysis of CFTR gene(accurate)
what normal finding in the newborn is indicative of transient polycythemia of the newborn
-splenomegaly
-hypoxia during delivery -> stimulates erythropoeitin -> highered RBC’s -> first breath -> highered O2 -> lowered erythropoeitin -> Hb normalization
transient polycythemia of the newborn
if transient tachypnea of the newborn lasts >4 hrs, what is the new dx? and what must you do to evaluate it?
- sepsis until proven otherwise
- blood & urine cultures
if transient tachypnea of the newborn lasts >4 hrs, & pt also has presents w/ neuro signs, what tests must you order
-besides blood & urine cultures for sepsis eval: LP(CSF & culture)
-benign minute hemorrhages caused by rapid intrathoracic pressure as chest compressed thru birth canal
subconjunctival hemorrhage of the newborn
MC skull fracture in newborn
Linear
Most fatal skull fracture in newborn
Basilar
Skull fracture in newborn that needs surgery
Cortical
swelling of soft tissues of scalp that crosses suture lines
caput succedaneum
subperiosteal hemorrhage that does not cross suture lines
cephalohematoma
the incidence of brachial palsy of the newborn is most common in
macrosomic infants of DM mothers
how to treat any type of brachial palsy of the newborn
immobilization
what is the most common newborn frx due to shoulder dystocia
clavicular
what is the most common cause of facial nerve palsy of the newborn
-forcep trauma of the facial nerve during delivery
how to treat facial nerve palsy of the newborn
-no treatment: recovers in time
no abdominal muscles -> no urination
prune belly
what is the most common cause of elevated AFP
-incorrect dating
what congenital disease is omphalocele highly assoc w/
-edwards syndrome(trisomy 18)
what congenital disease is umbilical hernia highly assoc w/
-congenital hypothyroidism
when is surgical intervention indicated for umbilical hernia & why
- after age 4: most spontaneously close at age 3
- prevent bowel strangulation & necrosis
Most common causative agent of neonatal conjunctivitis
Chlamydial conjunctivitis
When to not breastfeed
HIV infection, active pulmonary TB, malaria, typhoid fever, septicemia, women undergoing cancer tx, lithium, most Ab (except tetra); Very few contraindications. Everything else, BREASTFEED.
Crying makes respiration better (improvement of cyanosis) b/c they use their mouths
choanal atresia
Newborns lose weight right after birth, when do they regain their birth weight?
by 2 wks of age.
PALMAR GRASP, when does it disappear?
by 2-3 months
MORO reflex, when does it disappear?
4 MONTHS
schwarzeneggar pose (passively turning the child’s head to one side causes the ipsilateral arm to extend and the contralateral arm to flex at the elbow )
Asymmetric Tonic Neck Reflex
Asymmetric Tonic Neck Reflex, when does it disappear?
6 months
must disappear before walking possible
plantar grasp reflex
BABINSKI, when does it disappear?
persists up to 1-2 years
most sensitive test for detecting developmental dysplasia of the hip
UTZ
What age do most children start sleeping through the night?
4-6 months
By what ages do children double and triple their birthweight?
double at 5mo, triple at 12mo.
When do most children reach double their birth length?
4 years
What are some possibilities when you cannot elicit the red reflex on eye exam?
ataracts, glaucoma, retinoblastoma and chorioretinitis
postdates, grunting respirations, meconium staining, signs of air trapping, RR>100
meconium aspiration
Most common cause of abdominal mass in a newborn?
enlarged kidney
Infants above how many Kg are considered Macrosomic?
4kg
What is considered Small for Gestational Age (SGA)?
birth weight
How many blood vessels are in a normal umbilical cord? What are they?
2 arteries, 1 vein
What are brushfield spots?
“salt and pepper” speckling (white spots) on the iris seen in downs
What might a large fontanelle imply?
hypothyroidism, Osteogenesis imperfecta, chromosomal anomalies
Maternal LUPUS (SLE) might lead to what in the neonate?
FIRST degree AV block
When should stool switch from meconium and to what do they switch?
By the 3rd day of life, they should begin to appear yellow.
How often/day and for how long do babies typically nurse
Babies usually nurse 8-12 times in 24 hours, and the feedings may initially range from 20 to 60 minutes (although consistently lengthy feeds may indicate a problem)
Primitive reflexes
Palmar, rooting, moro, parachute
Important risk factor for congenital hip dislocation
Female sex