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 of Pediatrics
- Baylor College of Medicine
- 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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- Chen ST, Su YN, Ni YH, et al. Diagnosis of neonatal intrahepatic cholestasis caused by citrin deficiency using high-resolution melting analysis and a clinical scoring system. J Pediatr 2012; 161:626.
- Yeh JN, Jeng YM, Chen HL, et al. Hepatic steatosis and neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) in Taiwanese infants. J Pediatr 2006; 148:642.
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- Rodrigues F, Kallas M, Nash R, et al. Neonatal hemochromatosis--medical treatment vs. transplantation: the king's experience. Liver Transpl 2005; 11:1417.
- Leonis MA, Balistreri WF. Neonatal hemochromatosis: it's OK to say "NO" to antioxidant-chelator therapy. Liver Transpl 2005; 11:1323.
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- CRAIG JM, LANDING BH. Form of hepatitis in neonatal period simulating biliary atresia. AMA Arch Pathol 1952; 54:321.
- BILIARY ATRESIA
- OTHER OBSTRUCTIVE CAUSES
- Biliary cysts
- ALAGILLE SYNDROME
- Clinical features
- METABOLIC DISORDERS
- 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
- PARENTERAL NUTRITION-ASSOCIATED LIVER DISEASE
- ARC SYNDROME
- IDIOPATHIC NEONATAL HEPATITIS