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Benign recurrent cholestasis with normal gamma-glutamyl-transpeptidase activity
A Lachaux1, I Loras-Duclaux, R Bouvier
1Département de Pédiatrie, Hôpital Edouard Herriot, Lyon, France.
The Journal of Pediatrics
|July 1, 1992
Summary
Two infant cases of benign recurrent cholestasis presented with normal gamma-glutamyl-transpeptidase levels. This finding suggests benign recurrent cholestasis is a pediatric intrahepatic cholestasis subtype with normal enzyme levels.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Biochemistry
Background:
- Intrahepatic cholestasis in infants often involves elevated gamma-glutamyl-transpeptidase (GGT).
- Diagnostic challenges exist for cholestatic liver diseases in early childhood.
- Byler disease is a known pediatric intrahepatic cholestasis with normal GGT.
Observation:
- Two infants presented with clinical signs of intrahepatic cholestasis.
- Serum GGT levels were within the normal range for both infants.
- These cases met the diagnostic criteria for benign recurrent cholestasis.
Findings:
- Benign recurrent cholestasis can manifest with normal GGT levels in infants.
- This contrasts with typical presentations of other cholestatic conditions.
- The enzyme profile differentiates it from other forms of pediatric liver disease.
Implications:
- Normal GGT levels do not exclude intrahepatic cholestasis in infants.
- Benign recurrent cholestasis should be considered in the differential diagnosis of pediatric cholestasis.
- Further research is needed to understand the GGT-normal variants of pediatric cholestasis.