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Clin Pediatr Endocrinol. 2013 Apr;22(2):33-8. doi: 10.1292/cpe.22.33. Epub 2013 Apr 26.

Development of endometrial carcinoma in a patient with leprechaunism (donohue syndrome).

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  • 1Department of Pediatrics, Hokkaido University School of Medicine, Hokkaido, Japan ; Department of Pediatrics, Sapporo City Hospital, Hokkaido, Japan.

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.

KEYWORDS:

Leprechaunism; endometrioid adenocarcinoma; insulin receptor; rh-IGF-1

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