Table 4 Procedures in Patients with RDEB
Variable | Data | Design, Country |
|---|---|---|
Esophageal dilation (ED) | ||
Proportion undergoing ED, % (n/N) | 74 (23/31) | NR, Chile [42] |
56 (157/283) | Registry analysis, US [23] | |
43 (34/79) | Registry analysis, UK [17] | |
38 (NR/25e) | Single-center, US [66] | |
33 (134/411) | Registry analysis, US [30] | |
29 (45/157) | Single-center, Germany [55] | |
Average EDs performed per patient, mean/median (N) | Mean, 7 [14] | Single-center, UK [65] |
Median, 6 (77) | Single-center, UK [41] | |
Median, 5 (17, inversa subtype) | Registry analysis, US [30] | |
Median, 3 (136, severe subtype) | ||
Median, 2 (258, intermediate subtype) | ||
Maximum number of EDs performed per patient, no (N) | 14 (14) | Single-center, UK [65] |
41 (77) | Single-center, UK [41] | |
50 (411) | Registry analysis, US [30] | |
Age at first ED, years (N) | 5.5 (77) | Single-center, UK [41] |
Gastrostomy tube | ||
Proportion undergoing GT, % (n/N) | 58 (33/57) | Single-center, UK [31] |
37 (104/283) | Registry analysis, US [23] | |
33 (27/83) | Survey, International [54] | |
32 (25/79) | Registry analysis, UK [17] | |
24 (97/412) | Registry analysis, US [30] | |
14 (22/157) | Single-center, Germany [55] | |
8 (2/25e) | Single-center, US [66] | |
Average GTs performed per patient, median (N) | Median, 1 (412) | Registry analysis, US [30] |
Maximum GTs performed per patient, no (N) | 10 (412) | Registry analysis, US [30] |
Age at first GT, years (N) | 6 (6a) | Single-center, UK24 |
8 (44a) | Single-center UK36` | |