ESPE Abstracts

Background: Insulin Autoimmune Syndrome (IAS) is a rare cause of hyperinsulinemic hypoglycaemia with only few descriptions in children in the literature. Drugs containing the sulfhydryl group, such as methimazol, are known to be a causative factor of this syndrome. Diazoxide and octreotide are usually ineffective in such patients.

Objective: We aim to describe a rare case of IAS in a child, with a good response to a short course of glucocorticoid therapy.

Results: A previously healthy 3.5 year old Caucasian girl presented with hypoglycemic seizures. It is known that she had two courses of Piritinol treatment before the onset of the disease- for 1 month (6 months before) and for 2 weeks (10 days before). On admission blood glucose monitoring showed recurrent episodes of fasting hypoglycemia (1.7–2.8 mmol/l) and postprandial hyperglycemia (11–16 mmol/l), fasting tolerance was no longer than 1.5–2 h. Fasting test revealed non-ketotic hyperinsulinemic hypoglycemia: blood glucose 2.9 mmol/l, insulin >1000 μUn/ml, C-peptide 16.8 ng/ml. OGTT showed hyperglycemia (14.7 mmol/l) at 90 minute, but normal glucose levels at 120 min. (6.9 mmol/l). Based on extremely high levels of serum insulin in the absence of sulfonylurea intake, IAS was suspected. High levels of AIAb (>100 U/ml) and typical HLA alleles (DRB1* 04; DQA1* 0301) confirmed the diagnosis. To suppress autoimmunity, the girl was started on Prednisone 1.4 mg/kg per day. Normoglycemia was achieved in 3 days, but insulin and antibody levels remained elevated. Dose of prednisone was gradually reduced; treatment was stopped in 6 weeks. Follow up 2 months later showed clinical remission with negative fasting test, normalization of insulin secretion, but still mildly elevated AIAb (24.4 U/ml).

Conclusion: To our knowledge this is a first description of IAS in children in Russian Federation. We suspect that the likely trigger factor of the disease in this case was treatment of Piritinol, which has a disulfide bond, although we have not found information about the same cases in the literature. A short course of glucocorticoid treatment was effective in our case and might be recommended as an immunosuppressive therapy to achieve the remission rapidly.