Causes of neonatal cholestasis
- Stephanie H Abrams, MD, MS
Stephanie H Abrams, MD, MS
- Assistant Professor of Pediatrics
- Baylor College of Medicine
- Robert J Shulman, MD
Robert J Shulman, MD
- Professor of Pediatrics
- Baylor College of Medicine
- Section Editors
- Steven A Abrams, MD
Steven A Abrams, MD
- Section Editor — Neonatology
- Professor, Department of Pediatrics
- Dell Medical School at the University of Texas at Austin
- Elizabeth B Rand, MD
Elizabeth B Rand, MD
- Section Editor — Pediatric Hepatology
- Associate Professor of Pediatrics
- University of Pennsylvania School of Medicine
Neonatal cholestasis is defined serologically as the accumulation of components of bile in the bloodstream, most frequently manifested as conjugated hyperbilirubinemia in the newborn period. Cholestasis results from diminished bile flow and/or excretion, which can be the result of a number of different causes.
Causes of neonatal cholestasis can be divided into the following categories (table 1):
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- BILIARY ATRESIA
- OTHER OBSTRUCTIVE CAUSES
- Biliary cysts
- ALAGILLE SYNDROME
- Clinical features
- Nonsyndromic paucity of interlobular bile ducts
- METABOLIC/GENETIC DISORDERS
- Progressive familial intrahepatic cholestasis (PFIC)
- Disorders of lipid metabolism
- Disorders of bile acid metabolism
- Mitochondrial disorders
- Citrin deficiency
- Alpha-1 antitrypsin deficiency
- GESTATIONAL ALLOIMMUNE LIVER DISEASE (NEONATAL HEMOCHROMATOSIS)
- Gestational therapy
- CYSTIC FIBROSIS
- INTESTINAL FAILURE-ASSOCIATED LIVER DISEASE
- ARC SYNDROME
- IDIOPATHIC NEONATAL HEPATITIS