 There are many ways to categorize the differential diagnosis of neonatal cholestasis. The table at the right presents diagnoses by frequency of occurrence. The most common individual causes are biliary atresia and neonatal hepatitis. The diagnosis of "idiopathic neonatal hepatitis" was and remains a catch-all term for otherwise undiagnosed neonatal cholestasis. Over the past 20 years, as more genetic, metabolic and specific infectious etiologies have been defined, the size of this group has declined by more than half.
|
Frequency of Causes
of Neonatal Cholestasis |
|
Biliary atresia |
25-30% |
|
Intrahepatic cholestasis |
20% |
|
(Alagille, PFIC, etc) |
|
|
Idiopathic neonatal hepatitis |
15% |
|
Alpha-1-antitrypsin deficiency |
5-15% |
|
Infectious/bacterial |
5-10% |
|
(sepsis, TORCH) |
|
|
Inborn errors of bile acid synthesis |
2-5% |
|
Endocrine/metabolic |
2-5% |
|
