Treatment with Cytochrome c and Evaluation of Insulin Secretion in a Patient with Kearns-Sayre Syndrome

抄録

A 23-year-old male with Kearns-Sayre syndrome (KSS) and diabetes mellitus (DM) was treated with cytochrome c (Cardiocrome^®). We evaluated the insulin secretion by glucagon loading test, as well as changes in DM control after the treatment. When the patient was diagnosed with DM, he had already presented with very low insulin secretion which further deteriorated with time. Symptoms found in KSS patients such as muscle weakness and sensory defects make control of DM more difficult. Cardiocrome did not ameliorate the insulin secretion, probably because almost all the β cells had already been irreversibly damaged. It was the improvement in the patient’s activity in daily life that was responsible for the decreased incidence of extreme hypo- or hyperglycemic episodes. Cardiocrome treatment for KSS may be useful in controlling DM at least through the improvement of the general life condition. For the direct effect on β cells, further investigation with more patients is necessary.