Article

Bile acid synthesis disorders in Japan: long-term outcome and chenodeoxycholic acid treatment

2020-01-28

Abstract excerpt

<title>Abstract</title> <p>Background: We encountered 7 Japanese patients with bile acid synthesis disorders (BASD) over 21 years between 1996 and 2017. Diagnoses were made by bile acid and genetic analyses. We gave low-dose, long-term chenodeoxycholic acid (CDCA) treatment to 5 of the patients, who had 3β-hydroxy-Δ 5 -C 27 -steroid dehydrogenase/isomerase (3β-HSD) deficiency (n=3) or Δ 4 -3-oxosteroid 5β-reducta...

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Literature Corpus work: d3f5d073-ed14-5ea6-92c0-7ee4589831b0
DOI: 10.21203/rs.2.22018/v1

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