Table 1. Clinical characteristics of the study groups.
Insulin gene (INS) mutations were investigated in 1044 patients with permanent diabetes diagnosed before 6 months (PNDM, 0-26 weeks), between 6-12 months (infancy, 27-52 weeks), 1-2 years (early childhood, 53-104 weeks), MODY diagnosed <25 years or YT2D diagnosed <45 years, and in 49 patients with hyperinsulinism. The data is shown as the median with the range in parentheses. Data sets for BMI were incomplete (indicated by an asterisk). NA= not available.
| Clinical characteristic |
PNDM | Infancy | Early childhood |
MODY | YT2D | Hyperinsulinism |
|---|---|---|---|---|---|---|
|
Diabetes (Age-at-
diagnosis) |
0-26 weeks | 27-52weeks | 53-104weeks | <25 years | <45 years | |
| n | 141 | 86 | 58 | 296 | 463 | 49 |
| Sex (% Male) | 50% | 58% | 41% | 36% | 48% | 67% |
| Current age (yrs) | 7 (0-69) | 10 (1-51) | 13 (2- 72) | 36 (5-77) | 38 (3-87) | 3 (0.4-21) |
| Age-at-diagnosis | 8 weeks (0-26) |
39 weeks (27-52) |
97 weeks (57-104) |
16 years (3-25) |
29 years (3-46) |
7 weeks (0-728) |
| BMI (kg/m2) | 17 (9-40)* | 19 (13-30)* | 22 (17-25)* | 24 (13-30)* | 23 (15-30) | NA |
|
Number of
patients with an affected first- degree relative |
24 (17%) | 9 (10%) | 11 (19%) | 296 (100%) | 208 (45%) | – |
|
Treatment: INS/
OHA+INS/ OHA/ Diet (% cases) |
100/0/0/0 | 100/0/0/0 | 100/0/0/0 | 49/4/26/21 | 45/2/32/21 | – |
|
KCNJ11/ ABCC8
mutations excluded |
141 / 129 | 86 / 21 | 58 / 2 | 0 / 0 | 0/0 | 49 / 49 |
|
HNF1A / HNF4A /
GCK mutations excluded |
– | – | – | 189/81/56 | 180/76/87 | – |