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!

DOB