Table 1

Characteristics of the 18 study patients

Mutations n of patients; patient no.
KCNJ11
 G228An = 1; 13
 E227Kn = 1; 14
 E292Gn = 1; 10
 H186Dn = 1; 5
 I182Tn = 1; 9
 Q51Gn = 1; 8
 R201Cn = 2; 16, 18
 R201Hn = 7; 1, 2, 3, 7, 11, 15, 17
 V59Mn = 1; 12
ABCC8
 R1183W n = 1; 4
 R1380Hn = 1; 6
CharacteristicsBefore SU therapyDuring SU therapy, month 12 or 18§
Age at SU initiation, years, median (range)5.3 (0.1–18.5)
Males, n (%)13 (72)
HbA1c, %, median (range)7.75 (5.5–12.8)6.4 (5.4–10)
Basal C-peptide, ng/mL, median (range)*0.07 (0.02–0.51)0.28 (0.12–0.82)**
Stimulated C-peptide, ng/mL, median (range)*0.1 (0.05–1.44)0.74 (0.2–1.99)**
Glibenclamide dosage, mg/kg/day, median (range)0.2 (0–1.43)
  • *C-peptide measured using a glucagon stimulation test;

  • **P < 0.01;

  • §month 12 for basal and stimulated C-peptide and month 18 for HbA1c and glibenclamide dosage.