Sufficient increment of sulfonylurea without reintroduction of insulin ameliorates pubertal deterioration of glycaemic control in KCNJ11 neonatal diabetes treated with long-term sulfonylurea

Excerpt

To the Editor: Heterozygous activating mutations in KCNJ11 are the major cause of neonatal diabetes mellitus (NDM). Although patients with KCNJ11 NDM usually develop hyperglycaemia in an insulin-dependent state, most of them can transfer from insulin treatment to high-dose sulfonylurea [1]. The administered dose per body weight of sulfonylurea might be reduced over time because most patients show better glycaemic control [2]. However, some individuals show failure of long-term sulfonylurea treatment, which necessitates the reintroduction of insulin during adolescence or puberty [3]. The dose per body weight of sulfonylurea for these patients with worsened glycaemic control at the time of reintroduction of insulin was lower than the initial dose per body weight at the time of transferring from insulin, and it has not been verified whether further increment in sulfonylurea dose can control glucose levels in individuals with worsened condition during puberty. Here, we report a case of KCNJ11 NDM in which an increase in glibenclamide to the initial dose per body weight improved the pubertal deterioration of glycaemic control. …