title: Development of endometrial carcinoma in a patient with leprechaunism (donohue syndrome).
abstract: Leprechaunism is a rare autosomal recessive disease that is characterized by severe insulin resistance. This disease is caused by a defective insulin receptor and features abnormal glucose metabolism and retarded intrauterine and postnatal growth. However, there are few reports on the long-term course of leprechaunism. We reported the long-term clinical course and rh-IGF-1 treatment in a patient with leprechaunism. During follow-up her diabetes gradually deteriorated despite of treatment of rh-IGF-1. Furthermore, she developed endometrioid adenocarcinoma at the age of 24 yr. The development of endometrial disease must be carefully followed up in this disease.
authors.affiliation: Department of Pediatrics, Hokkaido University School of Medicine, Hokkaido, Japan ; Department of Pediatrics, Sapporo City Hospital, Hokkaido, Japan.
authors.forename: Wakako
authors.initials: W
authors.lastname: Jo
authors.forename: Satoko
authors.initials: S
authors.lastname: Sudo
authors.forename: Akie
authors.initials: A
authors.lastname: Nakamura
authors.forename: Daisuke
authors.initials: D
authors.lastname: Endo
authors.forename: Yosuke
authors.initials: Y
authors.lastname: Konno
authors.forename: Katsura
authors.initials: K
authors.lastname: Ishizu
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Tajima
keywords: Leprechaunism; endometrioid adenocarcinoma; insulin receptor; rh-IGF-1