Table 6.
Summary of the survey results for non‐insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata’s disease)
| Non‐insulinoma pancreatogenous insulin autoimmune syndrome | Insulin autoimmune syndrome (Hirata’s disease) | ||||
|---|---|---|---|---|---|
| Post‐gastric surgery HH | No surgery | ||||
| Postprandial HH | Adult nesidioblastosis | Unknown | |||
| No. patients (%) | |||||
| Total | 33 | 57 | 10 | 11 | 22 |
| Male | 23 (69.7) | 21 (36.8) | 5 (50.0) | 4 (36.4) | 10 (45.5) |
| Female | 10 (30.3) | 36 (63.2) | 5 (50.0) | 7 (63.6) | 12 (54.4) |
| Age at onset | |||||
| Median (years) | 69 | 41 | 49 | 43 | 63 |
| Range | 1–89 | 0.7–91 | 19–79 | 12–82 | 3–88 |
| Treatment (%) | |||||
| Nutritional Treatment | 18 (54.5) | 26 (45.6) | 1 (10.0) | 4 (36.4) | 8 (36.4) |
| Diazoxide | 2 (6.1) | 0 (0) | 7 (70.0) | 3 (27.3) | 0 (0) |
| Somatostatin analogs | 0 (0) | 0 (0) | 2 (20.0) | 0 (0) | 0 (0) |
| Glucagon | 0 (0) | 0 (0) | 0 (0) | 0 (0) | 1 (4.5) |
| Glucocorticoids | 2 (6.1) | 4 (7.0) | 1 (10.0) | 1 (9.1) | 3 (13.6) |
| Alpha‐glucosidase inhibitors | 18 (54.5) | 19 (33.3) | 3 (30.0) | 1 (9.1) | 11 (50.0) |
| Calcium channel blockers | 0 (0) | 1 (1.8) | 0 (0) | 0 (0) | 1 (4.5) |
| mTOR inhibitors | 0 (0) | 0 (0) | 0 (0) | 0 (0) | 0 (0) |
| Pancreatectomy | 1 (3.0) | 0 (0) | 6 (60.0) | 0 (0) | 0 (0) |
| Posttreatment complications (%) | |||||
| Residual hypoglycemia | 13 (39.4) | 17 (29.8) | 4 (40.0) | 5 (45.5) | 4 (18.2) |
| Diabetes mellitus | 4 (12.1) | 6 (10.5) | 1 (10.0) | 1 (9.1) | 8 (36.4) |
| Developmental delay | 1 (3.0) | 7 (12.3) | 2 (20.0) | 1 (9.1) | 0 (0) |
| Epilepsy | 2 (6.1) | 4 (7.0) | 1 (10.0) | 0 (0) | 0 (0) |
Abbreviations: HH, hyperinsulinemic hypoglycaemia; mTOR, mammalian target of rapamycin.