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Table 4 Procedures in Patients with RDEB

From: A systematic literature review of the disease burden in patients with recessive dystrophic epidermolysis bullosa

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`
  1. ED, esophageal dilation; GT, gastrostomy tube; No, number; NR, not reported; RDEB, recessive dystrophic epidermolysis bullosa; UK, United Kingdom; US, United States
  2. aChildren with RDEB