Table 1

Baseline characteristics in individual KCNJ11 case subjects and INS control subjects and summary of group characteristics

CaseMutationInheritanceSexAge (years)Age at diabetes diagnosis (weeks)Age at genetic diagnosis (years)Age at transfer to SU (years)Treatment (total daily dose)HbA1c DCCT (%)HbA1c IFCC (mmol/mol)
KCNJ11 1G53SAutosomal dominantM3222323Glyburide 30 mg, metformin 1 g9.378
KCNJ11 2R201HPresumed de novoF2241515 (attempted)Insulin (restarted after trial of glyburide)8.165
KCNJ11 3R201HDe novoM36102929Gliclazide 120 mg8.165
KCNJ11 4R201CPresumed de novoF3652734Glyburide 40 mg7.053
KCNJ11 5R201CDe novoM1961313Glyburide 27.5 mg5.436
KCNJ11 6V252GDe novoM2882121Glyburide 85 mg10.895
KCNJ11 7V59MDe novoF25151717Glyburide 7.5 mg8.165
KCNJ11 8V59MDe novoM1751011Glyburide 55 mg5.941
INS 1C43FAutosomal dominantF357831N/AInsulinNKNK
INS 2F48CPresumed de novoF50542N/AInsulinNKNK
INS 3G75CDe novoM28826N/AInsulin7.963
INS 4H29DDe novoF202612N/AInsulin (pump)8.266
KCNJ11 groupN/ADe novo = 7 (87.5%), autosomal dominant = 1 (12.5%)5 (63%) M, 3 (37%) F26.5 (17–36)5.5 (2–15)19 (10–29)21 (11–34)1 of 8 insulin treated (12.5%)8.1 (5.4–10.8)65 (36–95)
INS groupN/ADe novo = 3 (75%), autosomal dominant = 1 (25%)1 (25%) M, 3 (75%) F31.5 (20–50)17 (5–78)28.5 (12–42)N/A4 of 4 insulin treated (100%)8.1 (7.9–8.2)65 (63–66)
P valueN/A1.00.550.440.150.23N/A0.010.790.79
  • Group summary data are presented as median (range) for continuous numerical data and n (%) for categorical data. Individual values are also presented for each participant (continuous and discrete numerical data and categorical data). Mutations were presumed to have arisen de novo if there was no parental history of diabetes but the mutation status of the parents had not been confirmed with a genetic test. HbA1c values are the results available closest to the time of the neurobehavioral assessment. DCCT, Diabetes Control and Complications Trial; IFCC, International Federation of Clinical Chemistry; N/A, not applicable; NK, not known; SU, sulfonylureas.