Development of Endometrial Carcinoma in a Patient with Leprechaunism (Donohue Syndrome)

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 cour...

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Detalles Bibliográficos
Autores principales: Jo, Wakako, Sudo, Satoko, Nakamura, Akie, Endo, Daisuke, Konno, Yosuke, Ishizu, Katsura, Tajima, Toshihiro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society for Pediatric Endocrinology 2013
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3756185/
https://www.ncbi.nlm.nih.gov/pubmed/23990696
http://dx.doi.org/10.1297/cpe.22.33
Descripción
Sumario: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.

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