Continuous subcutaneous IGF-1 therapy via insulin pump in a patient with Donohue syndrome

David R. Weber, Diana E. Stanescu, Robert Semple, Cheryl Holland, Sheela N. Magge

Research output: Contribution to journalArticlepeer-review

15 Scopus citations

Abstract

Donohue syndrome (DS) is a severe form of congenital insulin resistance due to mutation(s) in the insulin receptor (INSR) gene. Given the similarities between insulin and insulin-like growth factor 1 (IGF-1) receptors, recombinant human IGF-1 (rhIGF-1) has been used to treat severe insulin resistance due to INSR mutation(s). Traditional subcutaneous therapy may be limited by the shortened IGF-1 half-life in these patients. We report the case of a female with molecularly confirmed DS treated with continuous rhIGF-1 therapy via an insulin pump. With treatment, the patient's hemoglobin A1c decreased from 9.8% to 8.8%, and her weight increased by 0.8 kg. Development of an ovarian tumor complicated her course, but it was unclear whether this was related to rhIGF-1 therapy. Limited treatment options exist for patients with DS. The use of continuous rhIGF-1 via an insulin pump may be a viable option, although further experience is needed to establish safety and efficacy.

Original languageEnglish (US)
Pages (from-to)1237-1241
Number of pages5
JournalJournal of Pediatric Endocrinology and Metabolism
Volume27
Issue number11-12
DOIs
StatePublished - Nov 1 2014
Externally publishedYes

Keywords

  • Donohue syndrome
  • insulin infusion systems
  • insulin resistance
  • insulin-like growth factor

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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