For pediatric patients with hepatocyte nuclear factor-1A (HNF1A)-maturity-onset diabetes of the young (
MODY 3), treatment with sulfonylureas is recommended. In adults with HNF1A-MODY,
meglitinide analogues achieve lower postprandial
glucose levels and pose a lower risk of delayed
hypoglycemia compared with sulfonylureas. This
therapy has not yet been reviewed in pediatric patients. We report on
meglitinide analogue treatment in 3 adolescents with HNF1A-MODY. Case 1 (14-year-old girl) was diagnosed asymptomatically but had an
hemoglobin A1c (HbA1c) level of 7.4%; her father had been recently diagnosed with HNF1A-MODY. With
repaglinide, her HbA1c level decreased to 5.5%, with no
hypoglycemic episodes. Case 2 (14-year-old boy) was diagnosed incidentally with glucosuria (HbA1c level: 7.0%) and was treated with
insulin. After the HNF1A-MODY diagnosis, he was switched to
glibenclamide. Due to several
hypoglycemic episodes, treatment was changed to
nateglinide and his HbA1c level decreased to 6.2% with no further
hypoglycemic episodes. Case 3 (11-year-old girl) presented with
polyuria and
polydipsia (HbA1c level: 10.1%) and was initially treated with
insulin. After the HNF1A-MODY diagnosis, treatment was changed to
repaglinide. She was obese (BMI: 28.8 kg/m(2); z-score: +2.2), and
glucose control with
repaglinide alone was insufficient. Therefore,
neutral protamine Hagedorn insulin (0.27 U/kg per day) was added. With this combination
therapy, her HbA1c level decreased to 8.2%. The use of meglitinides in these 3 adolescent patients was well tolerated and effective. Furthermore,
hypoglycemic episodes were rare compared with treatment with
insulin or sulfonylureas. We therefore suggest considering meglitinides as the primary oral treatment option for adolescents suffering from HNF1A-MODY.