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Fig. 1 | Orphanet Journal of Rare Diseases

Fig. 1

From: Cholic acid for primary bile acid synthesis defects: a life-saving therapy allowing a favorable outcome in adulthood

Fig. 1

Effect of long-term oral CA therapy on liver fibrosis in 15 children with 3β-hydroxy-Δ5-C27-steroid oxidoreductase deficiency (n = 13) or Δ4–3-oxosteroid 5β-reductase deficiency (n = 2). Fibrosis scores were assessed according to Metavir in liver biopsy specimens or as described in the patients and methods section in cases of assessment using non-invasive methods. See Table 2 for the details of fibrosis scores. Data are presented as mean +/− SD values. When in a patient 2 values of fibrosis score were recorded during the same time period, only the most recent score was considered to calculate the mean +/− SD values

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