Case report reveals clinical recovery in an 11-year-old girl with benign recurrent intrahepatic cholestasis, highlighting diagnostic challenges.
Cholestasis beyond infancy is uncommon and presents a diagnostic challenge. We report an 11‐year‐old girl presenting with severe pruritus followed by jaundice and a cholestatic biochemical profile with normal gamma‐glutamyl transferase levels. Extensive evaluation excluded infectious, autoimmune, metabolic, and obstructive causes. Liver biopsy demonstrated bland cholestasis with preserved hepatic architecture. The patient showed complete clinical and biochemical recovery following supportive therapy with ursodeoxycholic acid. Although this represents a first documented episode, the findings are most consistent with a benign recurrent intrahepatic cholestasis–like phenotype. This case highlights the importance of considering inherited cholestatic disorders in older children with unexplained GGT‐normal cholestasis.
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Hosiian et al. (2026) studied this question.
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