Discussion
We report the case of severe insulin resistance due to a compound heterozygosity for two mutations in the INSR . Management of the hyperglycemia in patients with RMS is challenging, and the case here presented illustrates this difficulty: despite of high insulin dose associated with two classes of insulin sensitizers, desirable glycemic control was not achieved. Introduction of an SGLT2 inhibitor promoted a decrease of HbA1c from 10.5% to 7.7%, higher than the decrease observed when metreleptin or recombinant human IGF1were used in patients with SIR (3). Increase in blood ketone bodies was not detected during the treatment. Previous reports of treatment with SGLT2 inhibitors in patients with severe insulin-resistance syndromes were in cases of generalized lipodystrophy, SHORT syndrome and type A insulin resistance syndrome (4,5).