TABLE 1

Main clinical characteristics of the patients with MODY5 and of those with no molecular alteration of TCF2

Molecular alteration of TCF2P
YesNo
n2812
Age at latest follow-up (years)42.8 ± 13.2 (13–73)55.3 ± 15.4 (21–71)0.01
Age at first manifestation (years)24.2 ± 14.8 (1–58)42.1 ± 18.2 (1–60)0.004
Diabetes
    Age at diagnosis (years)28.1 ± 13.1 (10–58)46.3 ± 14.3 (17–61)0.002
    Symptoms at diagnosis (yes/no)11/172/10NS
    BMI at diagnosis (kg/m2)23.8 ± 3.8 (15–32)27.4 ± 3.1 (21.6–32.5)0.004
    HbA1c at last examination (%)7.2 ± 1.2 (5.5–9.5)7.2 ± 1.5 (5.3–10.8)NS
    Insulin therapy (yes/no)20/82/100.002
Kidney disease
    Age at diagnosis (years)28.9 ± 16.9 (1–63)47.8 ± 17.1 (1–66)0.002
    Presence of kidney cysts (yes/no)21/710/2NS
    Other kidney morphological abnormalities (yes/no)*14/141/110.01
    Creatinine clearance at latest examination (ml/min per 1.73 m2)47.0 ± 26 (5.5–108)52.7 ± 27.2 (23.9–96)NS
    Systolic blood pressure (mmHg)127 ± 17 (104–170)139 ± 12 (117–160)0.01
    Diastolic blood pressure (mmHg)75 ± 7 (60–90)77 ± 9 (64–95)NS
Liver disease
    Liver tests abnormalities (yes/no)23/44/60.01
First-degree family history
    Diabetes (yes/no)12/1610/20.03
    Renal disease (yes/no)11/175/7NS
  • Data are means ± SD (range). Comparisons between groups were performed using Mann-Whitney test or Fisher’s exact test where appropriate. HbA1c normal values, 4–6%.

  • * Renal agenesis, renal atrophy, and pelvicaliceal abnormalities.