title: Recurrent familial hypobetalipoproteinemia-induced nonalcoholic fatty liver disease after living donor liver transplantation.
abstract: Familial hypobetalipoproteinemia (FHBL) is one of the causes of nonalcoholic steatohepatitis (NASH) and a codominant disorder. Patients heterozygous for FHBL may be asymptomatic, although they demonstrate low plasma levels of low-density lipoprotein (LDL) cholesterol and apolipoprotein B. Here we report a nonobese 54-year-old man with decompensated liver cirrhosis who underwent living donor liver transplantation with his son as the donor. Low albuminemia and refractory ascites persisted after transplantation. A biopsy specimen obtained 11 months after liver transplantation revealed severe steatosis and fibrosis, and recurrent NASH was diagnosed on the basis of pathological findings. Both the patient's and donor's laboratory tests demonstrated low LDL cholesterol and apolipoprotein levels. Because mutations in messenger RNAs of microsomal triglyceride transfer protein and apolipoprotein B genes were excluded neither in the recipient nor in the donor, both were clinically diagnosed as being heterozygous for FHBL. We successfully treated the recipient with heterozygous FHBL-induced recurrent NASH after liver transplantation using our diet and exercise programs.
authors: Department of Surgery and Medical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. nharada@surg2.med.kyushu-u.ac.jp
authors: Noboru
authors: N
authors: Harada
authors: Yuji
authors: Y
authors: Soejima
authors: Akinobu
authors: A
authors: Taketomi
authors: Tomoharu
authors: T
authors: Yoshizumi
authors: Hideaki
authors: H
authors: Uchiyama
authors: Toru
authors: T
authors: Ikegami
authors: Toshiharu
authors: T
authors: Saibara
authors: Takashi
authors: T
authors: Nishizaki
authors: Yoshihiko
authors: Y
authors: Maehara
mesh_terms: D000328:Adult; D001053:Apolipoproteins / Q000378:metabolism; D001706:Biopsy; D005192:Family Health; D005234:Fatty Liver / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology*; D006801:Humans; D006995:Hypobetalipoproteinemias / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D008099:Liver / Q000473:pathology; D016031:Liver Transplantation / Q000009:adverse effects* / Q000379:methods; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D009154:Mutation; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D001053:Apolipoproteins