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ORPHA:567983Particular clinical situation in a disease or syndrome
Also called PNAC
What it is
A rare hepatic disease characterized by intrahepatic cholestasis and deterioration of liver function in patients receiving parenteral nutrition for extended periods of time (signs may appear as early as within the first two weeks of initiation of parenteral nutrition). The condition commonly occurs in neonates and usually resolves with transition to enteral feeding, although severe cases may progress to liver fibrosis, cirrhosis, and portal hypertension.
Key facts
- Age of onset
- All ages
- Inheritance
- Not applicable
- Classified as
- Particular clinical situation in a disease or syndrome
Signs and symptoms
Common30–79%
16- Abdominal pain
- Abnormal circulating fatty-acid concentration
- Abnormality of cytokine secretion
- Abnormal metabolism
- Biliary hyperplasia
- Elevated circulating alkaline phosphatase concentration
- Elevated circulating hepatic transaminase concentration
- Elevated gamma-glutamyltransferase level
- Hepatic failure
- Hepatomegaly
- Hyperlipidemia
- Jaundice
- Premature birth
- Small for gestational age
- Splenomegaly
- Villous atrophy
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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