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Table 1—

Clinical and molecular study of the three children with early-onset diabetes and information about their sulfonylureas treatment

Case123
Molecular study R201L in KCNJ11 R201H in KCNJ11 Q211K in ABCC8 
Age diabetes was diagnosed (months) 
Ketoacidosis at onset 
Insulin dose before sulfonylurea treatment (units · kg−1 · day−10.6 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 
    Drug used for successful transfer Glibenclamide Glibenclamide Glibenclamide 
    Dose (mg · kg−1 · day−10.8 0.6 0.3 
    Doses/day (n
    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−10.3 0.6 1.4 
    Doses/day (n
    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   
Case123
Molecular study R201L in KCNJ11 R201H in KCNJ11 Q211K in ABCC8 
Age diabetes was diagnosed (months) 
Ketoacidosis at onset 
Insulin dose before sulfonylurea treatment (units · kg−1 · day−10.6 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 
    Drug used for successful transfer Glibenclamide Glibenclamide Glibenclamide 
    Dose (mg · kg−1 · day−10.8 0.6 0.3 
    Doses/day (n
    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−10.3 0.6 1.4 
    Doses/day (n
    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   
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