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• W2103648284 abstract "Pediatrics InternationalVolume 42, Issue 6 p. 685-688 3β-Hydroxy-Δ5-C27-steroid dehydrogenase/isomerase deficiency in a 23-year-old woman Masakazu Kobayashi, Masakazu Kobayashi Departments of Pediatrics andSearch for more papers by this authorMichio Koike, Michio Koike Departments of Pediatrics andSearch for more papers by this authorMichiyo Sakiyama, Michiyo Sakiyama Departments of Pediatrics andSearch for more papers by this authorShuuji Okuda, Shuuji Okuda Departments of Pediatrics andSearch for more papers by this authorMasumi Okuda, Masumi Okuda Departments of Pediatrics andSearch for more papers by this authorTomoyuki Tanaka, Tomoyuki Tanaka Microbiology, Wakayama Medical College, Wakayama City,Search for more papers by this authorAtushi Unno, Atushi Unno Department of Pediatrics, Juntendo University School of Medicine, Tokyo,Search for more papers by this authorHiroshi Nittono, Hiroshi Nittono Junshin Clinic, Yokohama City,Search for more papers by this authorHajime Takei, Hajime Takei Tokyo Health Service Association, Tokyo andSearch for more papers by this authorTsuyoshi Murai, Tsuyoshi Murai Faculty of Pharmaceutical Sciences, Health Sciences University of Hokkaido, Hokkaido, JapanSearch for more papers by this authorTeruki Yoshimura, Teruki Yoshimura Faculty of Pharmaceutical Sciences, Health Sciences University of Hokkaido, Hokkaido, JapanSearch for more papers by this authorTakao Kurosawa, Takao Kurosawa Faculty of Pharmaceutical Sciences, Health Sciences University of Hokkaido, Hokkaido, JapanSearch for more papers by this author Masakazu Kobayashi, Masakazu Kobayashi Departments of Pediatrics andSearch for more papers by this authorMichio Koike, Michio Koike Departments of Pediatrics andSearch for more papers by this authorMichiyo Sakiyama, Michiyo Sakiyama Departments of Pediatrics andSearch for more papers by this authorShuuji Okuda, Shuuji Okuda Departments of Pediatrics andSearch for more papers by this authorMasumi Okuda, Masumi Okuda Departments of Pediatrics andSearch for more papers by this authorTomoyuki Tanaka, Tomoyuki Tanaka Microbiology, Wakayama Medical College, Wakayama City,Search for more papers by this authorAtushi Unno, Atushi Unno Department of Pediatrics, Juntendo University School of Medicine, Tokyo,Search for more papers by this authorHiroshi Nittono, Hiroshi Nittono Junshin Clinic, Yokohama City,Search for more papers by this authorHajime Takei, Hajime Takei Tokyo Health Service Association, Tokyo andSearch for more papers by this authorTsuyoshi Murai, Tsuyoshi Murai Faculty of Pharmaceutical Sciences, Health Sciences University of Hokkaido, Hokkaido, JapanSearch for more papers by this authorTeruki Yoshimura, Teruki Yoshimura Faculty of Pharmaceutical Sciences, Health Sciences University of Hokkaido, Hokkaido, JapanSearch for more papers by this authorTakao Kurosawa, Takao Kurosawa Faculty of Pharmaceutical Sciences, Health Sciences University of Hokkaido, Hokkaido, JapanSearch for more papers by this author First published: 25 December 2001 https://doi.org/10.1046/j.1442-200x.2000.01292.xCitations: 18 Masakazu Kobayashi, Department of Pediatrics Wakayama Medical College, 811-1 Kimiidera, Wakayama City 641–0012, Japan. Email: [email protected] Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat References 1 Clayton PT, Leonard JV, Lawson AM et al. Familial giant cell hepatitis associated with synthesis of 3β,7α-dihydroxy- and 3β,7α,12α-trihydroxy-5-cholenoic acids. J. Clin. Invest. 1987; 79: 1031 8. 2 Buchmann MS, Kvittingen EA, Nazer H et al. Lack of 3β-hydroxy-Δ5-C27-steroid dehydrogenase/isomeraze in fibroblasts from a child with urinary excretion of 3β-hydroxy-Δ5-bile acids. J. Clin. Invest. 1990; 86: 2034 7. 3 Ichimiya H, Nazer H, Gunasekaran T, Clayton P, Sjouvall J. Treatment of chronic liver disease caused by 3β-hydroxy-Δ5-C27-steroid dehydrogenase deficiency with chenodeoxycholic acid. Arch. Dis. Child. 1990; 65: 1121 4. 4 Ichimiya H, Egestard B, Nazer H, Baginski ES, Clayton PT, Sjouvall J. Bile acid and bile alcohol in a child with hepatic 3β-hydroxy-Δ5-C27-steroid dehydrogenase deficiency: Efects of chenodeoxycholic acid treatment. J. Lipid Res. 1991; 32: 829 41. 5 Jaccquemin E, Stetchell KDR, O’Connell NC et al. A new cause of progressive intrahepatic cholestasis: 3β-Hydroxy-Δ5-C27-steroid dehydrogenase/isomerase deficiency. J. Pediatr. 1994; 125: 379 84. 6 Kobayashi M, Koike M, Sakiyama M et al. The first case of 3β-hydroxy-Δ5-C27-steroid dehydrogenase/isomerase deficiency in Japan. Jpn. J. Pediatr. Gastroenterol. Nutr. 1998; 2: 161 7. Citing Literature Volume42, Issue6December 2000Pages 685-688 ReferencesRelatedInformation" @default.
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