Pediatric emergency medicine trisk 619

Conjugated bilirubin is reported as direct and is elevated because of obstructed
excretion.
Goals of Treatment. The goals of ED evaluation of the icteric infant include
rapid diagnosis of the acutely treatable causes of icterus (sepsis, obstruction,
metabolic disease), prevention of kernicterus and brain injury, and reassurance
when the jaundice is physiologic.
Clinical Considerations
Clinical Recognition. In a child with true jaundice, the sclera will be yellow.
Jaundice in the first 24 hours of life of a term newborn is pathologic. Full-term,
well-appearing neonates over 1 day of age often have physiologic or breast-milk
jaundice. Physiologic jaundice is benign. Bilirubin levels spike close to day 3 of
life and then decrease. Breast-feeding jaundice may present in the first few days,
before sufficient milk production, or may present later, for unknown reasons.
Breast-fed infants may have prolonged unconjugated hyperbilirubinemia lasting
up to several weeks, thought to be related to compounds in breast-milk.
Clinical Assessment. Infants presenting with jaundice require a careful history,
looking for timing of jaundice onset, stool and urine color, breast or bottle
feeding, maternal and infant blood type, traumatic delivery, and maternal history
of diabetes, hepatitis, and medications. A careful examination should assess for
cephalohematoma and signs of infection. Acholic stools generally indicate biliary
obstruction or severe hepatic failure.
CBC, total bilirubin level, and direct bilirubin level should be obtained. A
direct Coombs test should be obtained if maternal blood type in unknown,
hemoglobin is low, or total bilirubin is at levels requiring intervention (see
below). Consider blood typing mother and neonate for ABO and Rh factors if
unknown. Rh incompatibility is uncommon in mothers who have received
prenatal care.
Infants presenting with elevated indirect bilirubin, direct bilirubin less than
15% of the total, and normal stool color have unconjugated hyperbilirubinemia.
Causes of unconjugated hyperbilirubinemia include physiologic jaundice, breastmilk jaundice, breast-feeding jaundice, hemolytic disease, blood group
incompatibility, infection, dehydration, polycythemia, abnormalities in the

conjugating enzyme (UDP-glucuronosyltransferase or UGT) as with Crigler–
Najjar syndrome or Lucey–Driscoll syndrome, Gilbert syndrome, or
hypothyroidism.


Infants with elevated direct bilirubin have conjugated hyperbilirubinemia,
which is always pathologic. Elevated direct bilirubin indicates obstructed
excretion, or cholestasis, usually due to biliary obstruction, hepatocellular
pathology, or metabolic disorder. Hepatitis, and in particular, idiopathic neonatal
hepatitis, is a common cause of conjugated hyperbilirubinemia. This must be
distinguished from infectious hepatitis, including hepatitis B, rubella,
cytomegalovirus, toxoplasmosis, coxsackie virus, echovirus, HSV, syphilis,
Listeria, and tuberculosis. In addition, systemic infectious disease can also result
in a toxic hepatitis, particularly systemic infections due to Escherichia coli,
Pneumococcus, Proteus, and Salmonella. Prolonged parenteral alimentation is
another common cause of conjugated hyperbilirubinemia that can persist several
months beyond the cessation of parenteral nutrition. Metabolic disorders of the
liver that can also result in conjugated hyperbilirubinemia include alpha 1antitrypsin deficiency, galactosemia, tyrosinemia, fructosemia, glycogen storage
diseases, lipid storage diseases, cerebrohepatorenal syndrome, trisomy 18, cystic
fibrosis, hemochromatosis, and idiopathic hypopituitarism.
Obtain CBC, blood culture, albumin, LFT’s PT/PTT, hepatitis serologies,
urinalysis, urine culture, and urine-reducing substances. Biliary atresia and
neonatal hepatitis are the most common etiologies and patients with biliary atresia
have better outcomes the earlier they are diagnosed. Neonates presenting with
elevated direct bilirubin with microcephaly, seizures, organomegaly, or petechiae
should be evaluated for congenital TORCH (toxoplasmosis, other infections,
rubella, CMV, herpes simplex) infections.
Management. Determine if the infant has indirect or direct hyperbilirubinemia.
Infants with direct (conjugated) hyperbilirubinemia or acholic stools require
admission for further diagnostic evaluation. Treatment of neonatal hepatitis is

supportive, with supplementation of the fat-soluble vitamins. Prevention of
hepatitis B infection requires early administration of HBIG and HBV vaccines to
prevent chronic infection in exposed newborns. Septic infants should be treated
with broad-spectrum antibiotics, typically with rapid normalization of
hyperbilirubinemia. While some of the metabolic disorders (galactosemia,
tyrosinemia, fructosemia, and the storage diseases) may be palliated with dietary
restrictions, most progress to chronic cirrhosis and liver failure.
Indirect Hyperbilirubinemia. Infants with physiologic jaundice can be
discharged if they do not meet criteria for phototherapy or exchange transfusion.
The Subcommittee on Hyperbilirubinemia of the AAP publishes guidelines for
management of hyperbilirubinemia in healthy term infants according to total


bilirubin levels and infants age (see Fig. 40.1 ). Infants with breast-milk jaundice
usually require increased breast-feedings, and close follow-up with pediatrician
until adequate milk supply is insured. Infants of diabetic mothers, infants with
congenital hypothyroidism, or with resorption from large cephalohematomas may
have higher levels of icterus than expected physiologically and may require serial
bilirubin level checks.
Pallor
CLINICAL PEARLS AND PITFALLS
Anemia in the neonate is recognized as pallor most commonly when
the hemoglobin falls below 10 g/dL.
Current Evidence. True pallor in the neonate can be due to significant anemia,
sepsis, shock, or severe chemical/electrolyte imbalance.
Clinical Recognition. Anemia in the neonate is recognized as pallor most
commonly when the hemoglobin falls below 10 g/dL.
Clinical Assessment. ED evaluation of pallor should include a careful history and
physical examination to distinguish between anemia, septic/cardiogenic shock,
electrolyte imbalance, and genetically determined fair skin coloring. Questions

should be asked regarding perinatal history, the baby’s feeding, level of alertness,
occurrence of vomiting or diarrhea, fever, and infant’s responsiveness. Careful
physical examination should include attention to vital signs, hydration status, and
lethargy, and search for cephalohematoma and hepatosplenomegaly. Consider
obtaining a CBC.

Dermatologic Findings
CLINICAL PEARLS AND PITFALLS
As few as one to two vesicles on an erythematous base, most
commonly on the scalp and face, may be the only presenting signs of
HSV infection, which should be considered even in the absence of
maternal signs or history of infection.
A tuft of coarse dark hair located in midline lumbosacral region may be
associated with spina bifida occulta.


Benign Rashes
Various papular rashes may be observed in the healthy newborn. Characteristic
body distribution patterns and age at appearance help differentiate these rashes
from more worrisome conditions. Diagnosis can be made by physical
examination alone and do not require further evaluation or specific treatment.
Parents should be reassured these are not worrisome conditions.
Milia are small 1- to 2-mm ivory or yellow papules located primarily on the
forehead, nose, and cheeks of newborns. Milia are keratin retention cysts. They
will spontaneously rupture and disappear during the first 3 to 4 weeks of life (
Fig. 96.4 ). Miliaria, or neonatal prickly heat, is caused by sweat retention and is
characterized by easily ruptured, 1- to 2-mm vesicles located primarily on the
face chest and back. Erythema toxicum is a more generalized eruption of small
papules or pustules on an erythematous base that may occur anywhere on the
body. Usually presenting during the first 3 to 4 days of life, these lesions may be

noted as late as 2 weeks of age. If the diagnosis is in question, a smear of the
papular contents will show a predominance of eosinophils with no organisms and
relative absence of neutrophils ( Fig. 96.5 ). Neonatal acne is characterized by
erythematous papules or pustules confined primarily to cheeks, chin, and
forehead. Lesions are caused by circulating maternal hormones, usually appear at
3 to 4 weeks of age and disappear within a few weeks ( Fig. 96.6 ). Diaper rash is
located on the skin covered by the diaper. Irritants and contact dermatitis are often
the initial precursor to the rash. Candida infections are common and diagnosed if
the rash is present in the intertriginous folds. Candida infections are beefy red
with well-demarcated borders and satellite papules and pustules. Mostly a clinical
diagnosis, candida can be confirmed if necessary by microscopic examination
looking for budding yeasts and pseudohyphae.