Clinical and molecular study of the three children with early-onset diabetes and information about their sulfonylureas treatment
| Case . | 1 . | 2 . | 3 . |
|---|---|---|---|
| Molecular study | R201L in KCNJ11 | R201H in KCNJ11 | Q211K in ABCC8 |
| Age diabetes was diagnosed (months) | 4 | 6 | 4 |
| Ketoacidosis at onset | + | + | + |
| Insulin dose before sulfonylurea treatment (units · kg−1 · day−1) | 0.6 | 0.7 | 0.3 |
| Type of insulin used | Glargine/lispro | Isophane/lispro | Glargine/lispro |
| A1C before sulfonylurea treatment (%) | 7.3 | 8.9 | 6.7 |
| Sulfonylureas treatment that allowed stopping insulin | |||
| Age sulfonylurea treatment began (years) | 1.4 | 3.1 | 3 |
| Drug used for successful transfer | Glibenclamide | Glibenclamide | Glibenclamide |
| Dose (mg · kg−1 · day−1) | 0.8 | 0.6 | 0.3 |
| Doses/day (n) | 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 |
| Quantity/day (mg · kg−1 · day−1) | 0.3 | 0.6 | 1.4 |
| Doses/day (n) | 3 | 3 | 1 |
| Quantity/dose (mg/dose) | 0.75, 1.25, 2.25 | 2.25, 2.75, 3.75 | 15.6 |
| A1C 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 | Sick day management Unexplained occasional hyperglycemia | Hypoglycemia with low doses of glibenclamide Transitory diarrhea with glibenclamide |
| Sick day management |
| Case . | 1 . | 2 . | 3 . |
|---|---|---|---|
| Molecular study | R201L in KCNJ11 | R201H in KCNJ11 | Q211K in ABCC8 |
| Age diabetes was diagnosed (months) | 4 | 6 | 4 |
| Ketoacidosis at onset | + | + | + |
| Insulin dose before sulfonylurea treatment (units · kg−1 · day−1) | 0.6 | 0.7 | 0.3 |
| Type of insulin used | Glargine/lispro | Isophane/lispro | Glargine/lispro |
| A1C before sulfonylurea treatment (%) | 7.3 | 8.9 | 6.7 |
| Sulfonylureas treatment that allowed stopping insulin | |||
| Age sulfonylurea treatment began (years) | 1.4 | 3.1 | 3 |
| Drug used for successful transfer | Glibenclamide | Glibenclamide | Glibenclamide |
| Dose (mg · kg−1 · day−1) | 0.8 | 0.6 | 0.3 |
| Doses/day (n) | 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 |
| Quantity/day (mg · kg−1 · day−1) | 0.3 | 0.6 | 1.4 |
| Doses/day (n) | 3 | 3 | 1 |
| Quantity/dose (mg/dose) | 0.75, 1.25, 2.25 | 2.25, 2.75, 3.75 | 15.6 |
| A1C 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 | Sick day management Unexplained occasional hyperglycemia | Hypoglycemia with low doses of glibenclamide Transitory diarrhea with glibenclamide |
| Sick day management |