Table 6.
Overall neurodevelopmental outcome for children with congenital hyperinsulinism (CHI) by patient data.
| Normal development | Neurodevelopmental impairment | |
|---|---|---|
| Geography and center category | 40 (53%) | 35 (47%) |
| Scandinavians | 12 (80%) | 3 (20%) |
| Eastern Europeans | 12 (44%) | 15 (56%) |
| Russians | 16 (36%) | 17 (52%) |
| Onset | ||
| Early onset | 24 (48%) | 26 (54%) |
| Late onset | 16 (64%) | 9 (36%) |
| Medical delay from first symptom | ||
| ≤2 days | 19 (61%) | 12 (39%) |
| >2 days | 21 (48%) | 23 (52%) |
| Medical expert delay from first symptom | ||
| ≤5 days | 12 (80%) | 3 (20%) |
| >5 days | 28 (47%) | 32 (53%) |
| Lowest blood glucose | ||
| Blood glucose ≤1 mmol/L | 10 (33%) | 20 (67%) |
| Blood glucose >1 mmol/L | 30 (67%) | 15 (33%) |
| Severity of disease | ||
| Non-severe CHI | 15 (68%) | 7 (32%) |
| Severe CHI | 25 (47%) | 28 (53%) |
| Genetics | ||
| KATP-channel mutations | 17 (44%) | 22 (56%) |
| Other gene mutations | 6 (86%) | 1 (14%) |
| No mutations detected | 11 (52%) | 10 (48%) |
| 18F-DOPA PET/CT scan | ||
| Focal | 6 (40%) | 9 (60%) |
| Diffuse | 14 (56%) | 11 (44%) |
| N/A | 21 (60%) | 14 (40%) |
| Magnetic resonance imaging brain | ||
| Normal | 16 (76%) | 5 (24%) |
| Abnormal | 3 (16%) | 16 (84%) |
| N/A | 21 (60%) | 14 (40%) |
| Age at follow-up | ||
| Age ≤810 days | 18 (50%) | 18 (50%) |
| Age >810 days | 22 (56%) | 17 (44%) |
Normal development: WISC-IV score >70, Bayley-III or Movement ABC-2 >15 percentile, or DP-3 score >70. Neurodevelopmental impairment: WISC-IV score <50, Bayley-III or movement ABC-2 <5 percentile, Developmental Profile 3 score <50, epilepsy, cerebral palsy, visual impairment, or too retarded to complete testing.