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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 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


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