An adult case with Hunter's syndrome presenting prominent hepatic failure: light and electron microscopic features of the liver.
We describe a 40-year-old male patient with Hunter's syndrome. His main manifestations were ascites and esophageal varices due to cirrhotic liver. We obtained hepatic biopsy samples and examined them. Ultrastructurally, the features of the hepatocytes and Kupffer cells were the same as those reported in young patients. The passage of 40 years led to gradual progression to fibrosis, and ultimately liver cirrhosis. Namely, with a longer survival time, the complications of liver cirrhosis become more remarkable. Hepatic fibrosis in Hunter's syndrome is slowly progressive and patients who are expected to have a longer life span should be continuously monitored for hepatic complications.
Yoshimoto
Tsuyoshi
T
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka.
Nakamuta
Makoto
M
Kotoh
Kazuhiro
K
Kohjima
Motoyuki
M
Morizono
Shusuke
S
Miyagi
Yuzuru
Y
Sakai
Hironori
H
Enjoji
Munechika
M
D000328:Adult; D001022:Aortic Valve Insufficiency / Q000209:etiology; D001706:Biopsy; D018450:Disease Progression; D005500:Follow-Up Studies; D022781:Hepatocytes / Q000648:ultrastructure; D006801:Humans; D007728:Kupffer Cells / Q000648:ultrastructure; D008103:Liver Cirrhosis / Q000209:etiology / Q000473:pathology; D017093:Liver Failure / Q000209:etiology* / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron; D016532:Mucopolysaccharidosis II / Q000150:complications*