Table 1. Clinical and molecular study of the three children with early onset diabetes mellitus, as well as information about their sulfonylureas treatment.
| Case | 1 | 2 | 3 |
|---|---|---|---|
| Molecular study | R201L in KCNJ11 | R201H in KCNJ11 | Q211K in ABCC8 |
| Age DM was diagnosed (months) | 4 | 6 | 4 |
| Ketoacidosis at onset | + | + | + |
| Insulin dose before sulfonylurea treatment (U/Kg/day) | 0.6 | 0.7 | 0.3 |
| Type of insulin used | Glargine/lispro | NPH/lispro | Glargine/lispro |
| HbA1c before sulfonylurea treatment | 7.3 | 8.9 | 6.7 |
| Sulfonylureas treatment that allowed stopping insulin | |||
| Age sulfonylurea treatment was begun (yr) | 1.4 | 3.1 | 3 |
| Drug used for successful transfer | Glibenclamide | Glibenclamide | Glibenclamide |
| Dose (mg/kg/day) | 0.8 | 0.6 | 0.3 |
| Number of doses/day | 2 | 3 | 2 |
| Dose (mg/dose) | 3.75 - 3.75 | 2.5 - 3.0 -3.5 | 1.5 - 1.5 |
| Sulfonylureas treatment at the last medical visit | |||
| Drug used at the last medical visit | Glibenclamide | Glibenclamide | Tolbutamide |
| Duration of sulfonylureas treatment (months) | 26.0 | 5.0 | 4.5 |
| Dose (mg/kg/day) | 0.3 | 0.6 | 1.4 |
| Number of doses/day | 3 | 3 | 1 |
| Dose (mg/dose) | 0.75 -1.25 - 2.25 | 2.25 - 2.75 - 3.75 | 15.6 |
| HbA1c during sulfonylureas treatment | 5.0-6.5 | 5.8 | 6.8 |
| Medical problems observed during sulfonylureas treatment | |||
| Initial diarrhea Unexplained hyperglycemia Hyperglycemia associated with missed dose Hypoglycemia Sick day management |
Sick day management Unexplained occasional Hyperglycemia |
Hypoglycemia with low doses of glibenclamide Transitory diarrhea with glibenclamide |