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).