Table 2.
Hyperinsulinism clinical features and management
Patient # | Diazoxide responsiveness |
HI management | GIR | Surgery | Postsurgery management |
Age off treatment (months) |
Current age (months) |
---|---|---|---|---|---|---|---|
Surgery | |||||||
1 | Unknown | Deceased | 15 | ND | NA | NA | NA |
2 | − | Surgery | 25 | 98% | Oct+continuous feeds | NA | Deceased |
3 | − | Surgery | 44 | 2% | None | 1 | 22 |
4 | − | Surgery | 22 | 90% | Diazoxide | 7 | 15 |
5 | − | Surgery | 8 | 75% | none | 9 | 78 |
6 | − | Surgery | 13 | 60% | Continuous D20 | 5 | 23 |
7 | − | Deceased | 50 | ND | NA | NA | NA |
8 | − | Surgery | 5 | biopsies | Continuous D20 | 21 | 52 |
9 | − | Surgery | 32 | 99% | None | − | 2.25 |
10 | − | Surgery | 14.5 | 91% | None | 5 | 68 |
11 | − | Surgery | 22 | 95% | Continuous feeds | 28 | 44 |
12 | − | Surgery | 18 | 85% | None | 18 | 27 |
13 | Unknown | Deceased | 32 | ND | NA | NA | NA |
14 | − | Deceased | 24 | ND | NA | NA | NA |
Octreotide | |||||||
15 | − | Oct | 11 | − | NA | 12 | 62 |
16 | − | Oct | 5.5 | − | NA | 7 | 17 |
17 | − | Oct | 7.7 | − | NA | 24 | 60 |
18 | − | Oct | NK | − | NA | 48 | 107 |
19 | − | Oct | 6.1 | − | NA | 17 | 45 |
20 | − | Oct | 13 | − | NA | NA | Deceased |
Diazoxide | |||||||
21 | + | Diaz | NK | − | NA | 24 | 177 |
22 | + | Diaz | 11.5 | − | NA | 24 | 53 |
23 | + | Diaz | NK | − | NA | 1 | 75 |
24 | + | Diaz | 21 | − | NA | − | 16 |
25 | + | Diaz | 10 | − | NA | 12 | 63 |
26 | + | Diaz | 10 | − | NA | NK | 36 |
IC2 | |||||||
27 | + | Low-dose diaz | 12 | − | NA | − | 15 |
28 | NT | Frequent feeds | NK | − | NA | 13 | 22 |
D20, intravenous dextrose; Diaz, diazoxide; GIR, glucose infusion rate in mg/kg/min-; NA, not applicable; ND, patient died prior to surgery; NK, not known; NT, diazoxide not trialled; Oct, octreotide.