{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,7,7]],"date-time":"2024-07-07T08:02:42Z","timestamp":1720339362381},"reference-count":10,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2016,2,4]],"date-time":"2016-02-04T00:00:00Z","timestamp":1454544000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Grant-in-Aid for Scientific Research (C)","award":["26460192"],"award-info":[{"award-number":["26460192"]}]},{"name":"Astellas Foundation for Research on Metabolic Disorders to H.H"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatrics International"],"published-print":{"date-parts":[[2016,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The bile salt export pump is expressed at the canalicular membrane of hepatocytes and mediates biliary excretion of bile salts. 4\u2010Phenylbutyrate (4\u2009PB), a drug used to treat ornithine transcarbamylase deficiency, has been found to increase the hepatocanalicular expression of bile salt export pump. The beneficial effects of 4\u2010phenylbutyrate therapy have been reported for patients with progressive familial intrahepatic cholestasis, an inherited autosomal recessive liver disease. This is the first study to show the therapeutic effect of 4\u2009PB in a preterm infant with cholestasis and liver fibrosis. The preterm infant had severe cholestasis with jaundice and failure to thrive refractory to ursodeoxycholic acid. Histology indicated giant cell hepatitis, cholestasis, and severe fibrosis. Bile salt export pump immunostaining showed lower expression than in a control. Oral 4\u2009PB was started at a daily dose of 200\u2009mg\/kg\/day. After the start of 4\u2009PB therapy, cholestasis improved.<\/jats:p>","DOI":"10.1111\/ped.12839","type":"journal-article","created":{"date-parts":[[2016,2,4]],"date-time":"2016-02-04T10:14:42Z","timestamp":1454580882000},"page":"506-509","source":"Crossref","is-referenced-by-count":5,"title":["Effects of 4\u2010phenylbutyrate therapy in a preterm infant with cholestasis and liver fibrosis"],"prefix":"10.1111","volume":"58","author":[{"given":"Shogo","family":"Ito","sequence":"first","affiliation":[{"name":"Department of Pediatrics and Neonatology Graduate School of Medical Sciences, Nagoya City University  Nagoya Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hisamitsu","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Pharmacokinetics Graduate School of Pharmaceutical Sciences, University of Tokyo  Tokyo 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