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Congenital bile acid synthesis defect 4

MONDO:0008967

An anomaly of bile acid synthesis characterized by mild cholestatic liver disease, fat malabsorption and/or neurological disease.

Also known as: AMACR deficiency, 2-methylacyl-CoA racemase deficiency, Alpha-methyl-acyl-CoA racemase deficiency, BAS defect type 4, BASD4, CBAS4, bile acid synthesis defect, congenital, type 4, congenital bile acid synthesis defect 4

1 clinical trial for this condition and its sub-types.

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Broader categories

Disease (680) Metabolic disease (233) Inherited lipid metabolism disorder (189) Hereditary disease (176) Inborn errors of metabolism (45) Human disease (14) Alpha-methylacyl-CoA racemase deficiency (2) Disease of genetic or genomic mechanism (2) Peroxisomal disease (2) Congenital bile acid synthesis defect (1)
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  • New diagnostic strategy aims to end diagnostic odyssey for rare diseases

    Diagnosis Completed

    This study tested a new approach to diagnose peroxisomal disorders, a group of rare genetic diseases. The strategy uses advanced metabolic and genetic tests to find the cause faster in people with suspicious symptoms or lab results. Researchers included 8 participants from four h…

    Sponsor: University Hospital, Lille • Aim: Diagnosis

    Last updated Jun 27, 2026 08:02 UTC

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