Jaundice in Infants – Direct

Jaundice in Infants – Direct: Excerpt from In A Page: Pediatric Signs and Symptoms

Jaundice refers to the presence of a yellow hue of the skin, sclerae, or mucous membranes. Direct, or more specifically, conjugated hyperbilirubinemia represents a pathologic condition of cholestasis. It is defined as a direct bilirubin fraction greater than 2 mg/dL, or greater than 15% of the total serum bilirubin. Because the outcome of treatment varies greatly with the rapiditiy of diagnosis, prompt recognition and evaluation are critical in the neonatal period.

Differential Diagnosis

• Bile duct obstruction
  –Biliary atresia: Represents the most frequent cause for liver transplantation in the pediatric patient; prompt diagnosis is crucial, as patient outcome is better if intervention comes before 60 days of life
  –Choledochal cyst
  –Common bile duct gallstone
  –Choledochocele
  –Bile duct stricture
  –Alagille syndrome
  –Caroli disease
  –Congenital hepatic fibrosis

• Neonatal hepatitis
  –Idiopathic hepatitis: Diagnosis of exclusion that should be made only when other causes are excluded; accounts for 60% of patients with neonatal cholestasis
  –Infections: TORCH, hepatitis B, HIV, E. coli, adenovirus, enterovirus, parvovirus B16, tuberculosis, listeriosis, malaria

• Metabolic disorders
  –α-1 antitrypsin deficiency
  –Cystic fibrosis
  –Hypothyroidism
  –Neonatal iron storage disease
  –Amino acids: tyrosinemia
  –Carbohydrates: Galactosemia, fructosemia
  –Lipids: Niemann-Pick, Gaucher, Wolman, cholesterol ester storage disease
  –Mitochondropathies
  –Bile acid synthetic disorders
  –Peroxisomal: Zellweger syndrome
  –Urea cycle defects
• Toxins
  –Total parenteral nutrition
  –Drugs: Trimethaprim-sulfamethoxazole, anticonvulsants
• Miscellaneous
  –Sepsis/hypoperfusion
  –Erythrophagocytic lymphohistiocytosis
  –Extracorporeal membrane oxygenation
  –Trisomy 17, 18, 21
  –Neonatal lupus erythematosus
  –Donohue syndrome
  –Rotor syndrome
  –Dubin-Johnson syndrome
  –Byler disease (PFIC type 1)
  –Cholestasis of North-American Indians
  –Nielsen syndrome

Workup and Diagnosis

• History
  –Prenatal/perinatal history: Infections, gestational age, birth weight, miscarriages, newborn screen
  –Age of onset, activity level, oral intake, urine output, stool color, emesis, hematemesis, hematochezia, melena, bruising, bleeding, fever, developmental milestones, medications, formula type
• Physical exam: Weight/length, icteris, dysmorphic features, cardiac murmur, ascites, abdominal distension, hepatosplenomegaly, edema, bruising, tone, reflexes
• Labs (initial): Fractionated bilirubin (total, indirect, direct), AST, ALT, GGT, alkaline phosphatase, total protein, albumin, CBC, electrolytes including glucose, PT, PTT, blood/urine culture, U/A
• Labs (directed): Thyroid function tests, serum α-1 antitrypsin level, urine for reducing sugars, serum and urine amino acids, urine organic acids including succinylacetone, infectious serologies, serum iron levels, sweat test, consider Alagille genetic testing
• Ultrasound provides best initial radiographic study for obstruction, hepatosplenomegaly
  
• Hepatobiliary scintigraphy (HIDA scan)
• Percutaneous liver biopsy
  –Histology, virology, electron microscopy for bile duct expansion/paucity, storage disorders
• Exploratory laparotomy and intraoperative cholangiogram to rule out biliary atresia
• Consider X-ray for butterfly vertebrae (for Alagille)

Treatment

• Varies by specific disorder

• General medication principles of cholestasis include
  –Promoting bile flow with ursodeoxycholic acid
  –Consider phenobarbital (increases bile excretion)
  –Fat-soluble vitamins including K, D, E
  –Vitamin A is a relative contraindication given hepatotoxicity at high levels

Book Source Details

• Book Title: In A Page: Pediatric Signs and Symptoms
• Author(s): Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan
• Year of Publication: 2007
• Copyright Details: In A Page: Pediatric Signs and Symptoms, Copyright © 2007 Lippincott Williams & Wilkins.

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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: In A Page: Pediatric Signs and Symptoms Authors: Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan Publisher: Lippincott Williams & Wilkins Copyright: 2007 ISBN: 1-4051-0427-9

 » Next page: Jaundice in Infants – Indirect (In A Page: Pediatric Signs and Symptoms)

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