Li X, et al. – The effectiveness and safety of sulfonylurea therapy were examined in Chinese neonatal diabetes mellitus (NDM) patients during infancy before genetic testing results were available. Although, in all patients with NDM molecular genetic diagnosis was recommended, but if genetic testing results were delayed, sulfonylurea therapy should be considered before such results are received. This therapy was useful even in infants with newly diagnosed NDM.

Methods

• In this study, the medical records of NDM patients with their follow–up details were reviewed.
• Molecular genetic analysis was performed.
• Sulfonylurea transfer regimens were applied in patients diagnosed after May 2010.
• In each patient, glycemic status and side effects were assessed.

Results

• There were 23 NDM patients from 22 unrelated families, 10 had KCNJ11mutations, 3 harbored ABCC8 mutations, 1 had INS mutations, 4 had chromosome 6q24 abnormalities, 1 had a deletion at chromosome 1p36.23p36.12, and 4 had no genetic abnormality identified.
• 16 NDM infants were treated with glyburide at an average age of 49 days (range 14–120 days) before genetic confirmation.
• Out of 16, 11 patients (69%) were able to successfully switch to glyburide with a more stable glucose profile.
• As per the outcomes, the responsive glyburide dose was 0.51 ± 0.16 mg/kg/d (0.3–0.8 mg/kg/d), while the maintenance dose was 0.30 ± 0.07 mg/kg/d (0.2–0.4 mg/kg/d).
• No serious adverse events were observed.