Cryptogenic fulminant hepatic failure in infancy: report of 2 cases with unique vascular obstructive changes in native livers.
Although the causes of fulminant hepatic failure (FHF) remain cryptogenic in many cases, a few reports have reviewed the pathological findings of native livers to evaluate the etiology. We report 2 cases of infantile cryptogenic FHF with unique vascular obstructive changes in the native livers. Clinically, it was notable that these 2 patients developed FHF very early in life, at 2 months and 5 months of age, respectively. One patient died from chronic rejection associated with hepatic veno-occlusive disease 4 months after transplantation. Histologically, hepatocytes in the native livers were completely destroyed in both patients, and vascular findings revealed obstruction of central veins in 1 patient and obstruction of portal veins in the other patient. Although the pathogenesis of vascular obstructions is not yet understood, this study suggests that an obstructive vascular event may be a contributing etiologic factor of FHF in infancy.
Shimojima
Naoki
N
Department of Surgery, Keio University School of Medicine, Tokyo, Japan.
Shibata
Rie
R
Hoshino
Ken
K
Kawachi
Shigeyuki
S
Tanabe
Minoru
M
Wakabayashi
Go
G
Shimazu
Motohide
M
Sakamoto
Michiie
M
Morikawa
Yasuhide
Y
Kitajima
Masaki
M
D001808:Blood Vessels / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007223:Infant; D008102:Liver Circulation / Q000502:physiology*; D017114:Liver Failure, Acute / Q000401:mortality / Q000473:pathology / Q000503:physiopathology*; D016031:Liver Transplantation; D008297:Male