TABLE 1.
Overview of hospital admissions during metabolic decompensation in 128 patients with an IEM associated with fasting intolerance
| IEM | Total of patients, n | Total admissions, n | Unique patients with admission, n (%) a | Number of patients with ≥1 admission × number of admissions | Median age b , years [range] |
|---|---|---|---|---|---|
| GSDIa | 23 | 25 | 8 (35%) |
1 × 10 1 × 5 1 × 3 2 × 2 3 × 1 |
18 [1‐39] |
| GSDIb | 7 | 10 | 4 (57%) |
2 × 4 2 × 1 |
13 [4‐19] |
| GSDIIIa | 8 | 7 | 3 (38%) |
1 × 3 2 × 2 |
8 [6–11] |
| GSDIIIb | 3 | 0 | — | — | — |
| GSDVI | 1 | 0 | — | — | — |
| GSDIX | 15 | 16 | 7 (47%) |
1 × 4 3 × 3 3 × 1 |
3 [0–6] |
| GSDXI | 2 | 1 | 1 (50%) | 1 × 1 | 6 [NA] |
| MCADD | 63 | 50 | 26 (41%) |
4 × 4 3 × 3 6 × 2 13 × 1 |
3 [0‐13] |
| MADD | 3 | 14 | 2 (67%) | 2 × 7 | 4 [0‐21] |
| LCHADD | 2 | 3 | 2 (100%) |
1 × 2 1 × 1 |
4 [0–5] |
| VLCADD | 1 | 1 | 1 (100%) | 1 × 1 | 4 [NA] |
| Total population | 128 | 127 | 54 (42%) | 54 × 127 | 8 [0‐39] |
a
% is the number of unique patients with admission divided by total number of patients with a specific IEM.
b
Age at hospital admission.