Table 1—

Clinical characteristics of patients with SUR1 mutations according to success of treatment with sulfonylureas

CharacteristicAll patientsPatients with successful sulfonylurea treatmentPatients with unsuccessful sulfonylurea treatmentP*
n27234
Mutation (number of patients)NAV86G, P45L/G1401R- (2), D209E (3), T229I/V1523L, Q211K, V86A (2), E1507G, V215I/V607M, E208K/Y263D, R1380L (2), D212I (3)§, T229I/T229I, R1183W§, L225P, R826W, and D209NF132L (2), F132V, and N72S (mosaic).NA
Classification: TNDM initial/TNDM relapse/PNDM/non-neonatal diabetes2/6/17/22/6/13/20/0/4/0NA
Neurological features6 (22)4 (17)2 (50)0.20
Male sex10 (37)8 (35)2 (50)0.61
Birth weight (g)2,675 (1,470–3,870)2,675 (1,470–3,500)2,670 (2,440–3,870)
Birth weight (SD score)−1.3 (−2.8 to 0.64)−1.3 (−2.8 to −0.1)−1.1 (−1.4 to 0.64)0.22
Age at diagnosis (weeks)6 (0–30)5 (0–30)17 (5–26)0.046
Age at start of sulfonylurea treatment (years)7.8 (0.2–46.5)7.1 (0.2–46.5)14.9 (0.4–28.5)0.52
A1C before sulfonylurea treatment (%)7.5 (5.0–21.0)7.1 (5.0–21.0)10.0 (7.5–12.0)0.11
Insulin dose (units · kg−1 · day−1)0.5 (0.2–1.2)0.4 (0.2–0.9)0.8 (0.4–1.2)0.097
Equivalent dose of glyburide (mg · kg−1 · day−1)0.28 (0.07–2.80)0.26 (0.07–2.80)1.00 (1.00–1.12)NA
  • Data are median (range), n (%), or n unless otherwise indicated. Percentages are rounded up to the nearest whole number.

  • *

    * P values are for comparison of the patients with a successful switch with patients with an unsuccessful switch and were calculated by the Mann-Whitney U test or Fisher's exact test for categorical data.

  • Reported in Ellard et al., 2007 (ref. 7);

  • reported in Patch et al., 2007 (ref. 8);

  • §

    § reported in Flanagan et al., 2007 (ref. 3). NA, not applicable.