From a physician in Al Ain, United Arab Emirates:
I am taking care of a family with nine children. Five of them have a rare mutation in insulin receptor (Il119 for Met) causing extreme insulin resistance and huge problems with controlling diabetes. Do you have experience with IGF-1 or insulin sensitizers? I would be very grateful for your kind advice and opinion.
We have not had to look after a family such as you describe and have had only very little experience with the management. In one case of type A resistance, the use of glitazones was disappointing. Looking in PubMed under 'Ile Met insulin receptor mutations' and 'Treatment of insulin receptor defects', I found a number of references to the successful use of IGF-1 given subcutaneously before meals in a total daily dose of 1.6 mg/kg. These seemed to be mostly from Japan and in cases of leprechaunism. If you have access to a medical library, the two references that first struck me as helpful were J.Hone et al in the J.Med.Gen 31:715,1994 and M.Kato et.al in Hokkaido Igako Zesshi73:613,1998. I hope this contributes something and it would be very nice if you could find time to write this family up!
Original posting 22 Nov 2000
Posted to Daily Care
Last Updated: Tuesday April 06, 2010 15:09:15
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