title: Case of cholangiocellular carcinoma in a patient with glycogen storage disease type Ia.
abstract: Glycogen storage disease (GSD) type Ia is caused by a deficiency in glucose-6-phosphatase. Long-term complications, including renal disease, gout, osteoporosis and pulmonary hypertension, develop in patients with GSD type Ia. In the second or third decade, 22-75% of GSD type Ia patients develop hepatocellular adenoma (HCA). In some of these patients, the HCA evolves into hepatocellular carcinoma. However, little is known about GSD type Ia patients with HCA who develop cholangiocellular carcinoma (CCC). Here, we report for the first time, a patient with GSD type Ia with HCA, in whom intrahepatic CCC was developed.
authors.affiliation: Department of Internal Medicine, Aichi Medical University, Nagakute, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Kanamori
authors.forename: Yukiomi
authors.initials: Y
authors.lastname: Nakade
authors.forename: Takaya
authors.initials: T
authors.lastname: Yamamoto
authors.forename: Yuji
authors.initials: Y
authors.lastname: Kobayashi
authors.forename: Ken
authors.initials: K
authors.lastname: Sato
authors.forename: Kiyoaki
authors.initials: K
authors.lastname: Ito
authors.forename: Tomohiko
authors.initials: T
authors.lastname: Ohashi
authors.forename: Noiku
authors.initials: N
authors.lastname: Nakao
authors.forename: Norimitsu
authors.initials: N
authors.lastname: Ishii
authors.forename: Emiko
authors.initials: E
authors.lastname: Takahashi
authors.forename: Toyoharu
authors.initials: T
authors.lastname: Yokoi
authors.forename: Haruhisa
authors.initials: H
authors.lastname: Nakao
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Kurokawa
authors.forename: Chikara
authors.initials: C
authors.lastname: Yamaguchi
authors.forename: Masashi
authors.initials: M
authors.lastname: Yoneda
keywords: cholangiocellular carcinoma; glycogen storage disease; hepatocellular adenoma