Table 2. Clinical characteristics of probands diagnosed in infancy and their family members with an INS gene mutation.
Characteristic | Age-at-diagnosis | All subjects | ||
---|---|---|---|---|
0-26 weeks | 27-52 weeks | 1 -45 years | ||
n | 39 | 5 | 2 | 46 |
Sex (% Male) | 19/39 (49%) | 2/5 (40%) | 2 (100%) | 23/46 (50%) |
Current age
(years) |
10 (0.5-42) | 16 (1-45) | 42 (35-48) | 14 (0.5-48) |
Current BMI
(kg/m2) |
17 (11-36) | 18 (16-30) | NA | 17 (11-36) |
Birth weight (kg) | 2.6 (1.7-3.8) | 3 (2.9-3.9) | 2.7 (2.4-2.9) | 2.7 (1.7-3.9) |
Gestational age
(weeks) |
40 (35-42) | 40 (37-42) | 40 | 40 (35-42) |
Corrected birth
weight (Centile) |
3 (<1st-87) | 44 (5-83) | 4.5 (<1-8) | 6 (<1st -87) |
Age-at-diagnosis | 9 weeks (0-26) | 35 weeks (31 -48) | 14.5 years (7-22) | 11 weeks (0-1144) |
Insulin treatment | 100% | 100% | 100% | 100% |
Insulin dose
(U/kg/day) |
0.73 (0.3-1.9) | 0.6 (0.2 -0.7) | 0.45 (0.3-0.6) | 0.7 (0.3-1.9) |
HbA1c | 7.9 (4.6-13.8) | 7.6 (6.5-9.5) | NA | 7.95 (4.6-13.8) |
Antibody status:
Neg/Pos/NA |
18/0/21 | 2/0/3 | 0/0/2 | 19/1/26 |