Table 1. Demographic characteristics of pediatric genetically confirmed MODY cases in a Turkish cohort (n=224).
|
HNF4A n=8 |
GCK n=146 |
HNF1A n=43 |
PDX1 n=1 |
HNF1B n=7 |
NEUROD1 n=3 |
KLF-11 n=8 |
CEL n=1 |
INS n=3 |
ABCC8 n=3 |
KJNC11 n=1 |
Mean |
|
|
Age at diagnosis (years) |
10.3±4.6 |
8.8±3.9 |
10.6±4.3 |
3.9 |
12.4±3 |
9±7.8 |
11.1±2 |
6 |
9.1±2.3 |
10.5±1.8 |
8.5 |
9.4±4.1 (range 0.15-17) |
|
Sex F/M |
3/5 |
63/83 |
22/21 |
0/1 |
3/4 |
1/2 |
4/4 |
1/0 |
1/2 |
2/1 |
1 |
101/123 (45%/55%) |
|
Symptom (+) (-) |
6 2 |
23 123 |
5 18 |
1 0 |
5 2 |
0 3 |
7 1 |
0 1 |
0 3 |
1 2 |
1 0 |
30% (49/224) |
|
Presentation (n) IH DK DKA |
5 1 2 |
145 0 0 |
31 9 2 |
0 0 1 |
5 1 2 |
2 0 1 |
3 3 0 |
1 0 0 |
3 1 0 |
3 1 0 |
1 0 0 |
197 (87%) 16 (7%) 8 (3.5%) |
|
Negative family history (n) |
2 |
17 |
4 |
0 |
1 |
0 |
0 |
0 |
0 |
0 |
0 |
24/224 (11%) |
F/M: female/male, IH: incidental hyperglycemia, DK: diabetic ketosis, DKA: diabetic ketoacidosis, MODY: maturity onset diabetes of the young