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Histological findings in the livers of patients with neonatal intrahepatic cholestasis caused by citrin deficiency.
MedLine Citation:
PMID:  20070398     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Aim: To characterize the histological features of the livers of patients with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), we studied specimens from 30 patients diagnosed with NICCD by genetically analyzing the SLC25A13 gene. Methods: Liver biopsy specimens were subjected to hematoxylin-eosin, Azan, and Berlin-blue staining. Results: Most specimens showed varying degrees of fibrosis. The degree of inflammation varied among the specimens, with half showing moderate or severe inflammatory changes. Fat deposition in hepatocytes was observed in almost all of the specimens, and severe fatty liver was noted in 20 (67%) of them. There was a mixture of two types of hepatocytes with macrovesicular or microvesicular fat droplets, and cholestasis was observed at a rate of 77%. Hemosiderin deposition, mostly mild and localized in periportal hepatocytes and macrophages in portal areas, was observed in 57% of the specimens. Conclusion: A combination of mixed macrovesicular and microvesicular fatty hepatocytes and the above-described findings, such as fatty liver, cholestasis, necroinflammatory reaction and iron deposition, are almost never observed in other liver diseases in infants and adults. We believe that NICCD is a disease with characteristic hepatopathological features.
Authors:
Akihiko Kimura; Masayoshi Kage; Ikuo Nagata; Sotaro Mushiake; Toshihiro Ohura; Yusaku Tazawa; Shunichi Maisawa; Takeshi Tomomasa; Daiki Abukawa; Yoshiyuki Okano; Ryo Sumazaki; Masaki Takayanagi; Akiko Tamamori; Tohru Yorifuji; Yasuhiko Yamato; Kohji Maeda; Masami Matsushita; Toyojiro Matsuishi; Ken Tanikawa; Keiko Kobayashi; Takeyori Saheki
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Publication Detail:
Type:  Journal Article     Date:  2010-01-11
Journal Detail:
Title:  Hepatology research : the official journal of the Japan Society of Hepatology     Volume:  40     ISSN:  1386-6346     ISO Abbreviation:  Hepatol. Res.     Publication Date:  2010 Apr 
Date Detail:
Created Date:  2010-04-16     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9711801     Medline TA:  Hepatol Res     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  295-303     Citation Subset:  -    
Affiliation:
Department of Pediatrics and Child Health, Kurume University School of Medicine, 67 Asahi-machi, Kurume 830-0011, Japan.
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