Cholestatic Liver Failure and Hypoglycemia in a Newborn: A Mitochondrial Pathology due to Citrin Deficiency

ABSTRACT In an infant with cholestasis and recurrent hypoglycemia, the combination of hypercitrullinemia, hypermethioninemia, and hyperthreoninemia should prompt testing for citrin deficiency, because early metabolic and genetic diagnosis allows targeted nutritional treatment and rapid clinical improvement.

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Publication Details

Journal
Clinical Case Reports
Published
2026-09-24
DOI
https://doi.org/10.1002/ccr3.73358
Primary Topic
Metabolism and Genetic Disorders
Type
article
Field-Weighted Citation Impact
0.00
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article

Cholestatic Liver Failure and Hypoglycemia in a Newborn: A Mitochondrial Pathology due to Citrin Deficiency

Julien Neveu, Mathilde Butori, Anne Spraul, Charlotte Hinault-Boyer et al.
Clinical Case Reports
Metabolism and Genetic Disorders
article

Cholestatic Liver Failure and Hypoglycemia in a Newborn: A Mitochondrial Pathology due to Citrin Deficiency

Julien Neveu, Mathilde Butori, Anne Spraul, Charlotte Hinault-Boyer, Mourad Naïmi
article en

Abstract

ABSTRACT In an infant with cholestasis and recurrent hypoglycemia, the combination of hypercitrullinemia, hypermethioninemia, and hyperthreoninemia should prompt testing for citrin deficiency, because early metabolic and genetic diagnosis allows targeted nutritional treatment and rapid clinical improvement.

Clinical Case ReportsVol. 14(10)
Fondation Lenval (FR), Hôpital Pasteur (FR), Bicêtre Hospital (FR)
Zero hunger
Openalex Percentile: Top 15%
Metabolism and Genetic Disorders
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