Table 1.
Summary of the survey results for congenital hyperinsulinism
| Transient CHI | Persistent CHI | Unknown | Total | |
|---|---|---|---|---|
| No. patients (%) | ||||
| Total | 197 | 225 | 25 | 447 |
| Male | 125 (63.5) | 120 (53.3) | 14 (56.0) | 259 (57.9) |
| Female | 72 (36.5) | 105 (46.7) | 11 (44.0) | 188 (42.1) |
| Age at onset | ||||
| Median | 0 day | 0 day | 0 day | 0 day |
| Range | 0 day–1 month | 0 day–2 years 4 months | 0 day–2 months | 0 day–2 years 4 months |
| Treatment (%) | ||||
| Nutritional treatment | 80 (40.6) | 124 (55.1) | 10 (40.0) | 214 (47.9) |
| Diazoxide | 99 (50.3) | 213 (94.7) | 14 (56.0) | 326 (72.9) |
| Somatostatin analogs | 1 (0.5) | 58 (25.8) | 0 (0) | 59 (13.2) |
| Glucagon | 9 (4.6) | 29 (12.9) | 3 (12.0) | 41 (9.2) |
| Glucocorticoids | 20 (10.2) | 31 (13.8) | 3 (12.0) | 54 (12.1) |
| Alpha‐glucosidase inhibitors | 0 (0) | 3 (1.3) | 0 (0) | 3 (0.7) |
| Calcium channel blockers | 0 (0) | 2 (0.9) | 0 (0) | 2 (0.4) |
| mTOR inhibitors | 0 (0) | 0 (0) | 0 (0) | 0 (0) |
| Pancreatectomy | 0 (0) | 25 (11.1) | 0 (0) | 25 (5.6) |
| Posttreatment complications (%) | ||||
| Residual hypoglycaemia | 1 (0.5) | 80 (35.6) | 2 (8.0) | 83 (18.6) |
| Diabetes mellitus | 2 (1.0) | 14 (6.2) | 0 (0) | 16 (3.6) |
| Developmental delay | 23 (11.7) | 63 (28.0) | 4 (16.0) | 90 (20.1) |
| Epilepsy | 4 (2.0) | 32 (14.2) | 1 (4.0) | 37 (8.3) |
Abbreviations: CHI, congenital hyperinsulinism; mTOR, mammalian target of rapamycin.