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Histological findings in the livers of patients with neonatal intrahepatic cholestasis caused by citrin deficiency
Author(s) -
Kimura Akihiko,
Kage Masayoshi,
Nagata Ikuo,
Mushiake Sotaro,
Ohura Toshihiro,
Tazawa Yusaku,
Maisawa Shunichi,
Tomomasa Takeshi,
Abukawa Daiki,
Okano Yoshiyuki,
Sumazaki Ryo,
Takayanagi Masaki,
Tamamori Akiko,
Yorifuji Tohru,
Yamato Yasuhiko,
Maeda Kohji,
Matsushita Masami,
Matsuishi Toyojiro,
Tanikawa Ken,
Kobayashi Keiko,
Saheki Takeyori
Publication year - 2010
Publication title -
hepatology research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.123
H-Index - 75
eISSN - 1872-034X
pISSN - 1386-6346
DOI - 10.1111/j.1872-034x.2009.00594.x
Subject(s) - cholestasis , citrullinemia , fatty liver , pathology , liver biopsy , fibrosis , medicine , gastroenterology , staining , steatohepatitis , h&e stain , biopsy , chemistry , disease , urea cycle , biochemistry , amino acid , arginine
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.