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Table 1 Demographic and baseline clinical characteristics

From: A prospective short-term study to evaluate methodologies for the assessment of disease extent, impact, and wound evolution in patients with dystrophic epidermolysis bullosa

  Mild
N = 9
Moderate
N = 10
Severe
N = 11
Overall
N = 30
Age (years)     
 Mean (SD) 18.3 (18.1) 29.1 (17.9) 19.1 (10.9) 22.2 (16.0)
 Median (range) 9.1 (3.9–58.7) 33.2 (3.8–50.7) 15.9 (4.1–37.9) 17.8 (3.8–58.7)
Age group, n (%)     
 ≤ 2 years 0 0 0 0
 > 2 to 10 years 5 3 2 10 (33.3)
 > 10 to 18 years 1 0 4 5 (16.7)
 > 18 years 3 7 5 15 (50.0)
Sex. n (%)     
 Male 7 3 5 15 (50.0)
 Female 2 7 6 15 (50.0)
Race, n (%)     
 White 9 7 10 26 (86.7)
 Asian 0 2 0 2 (6.7)
 Other 0 1 1 2 (6.7)
BMI category (patients < 18 years), n (%)     
 Underweight (< 5th percentile) 1 3 3 7 (46.7)
 Healthy weight (5th to 75th percentile) 5 0 3 8 (53)
 Overweight (> 75th percentile) 0 0 0 0
BMI category (patients ≥ 18 years), n (%)     
 Underweight (16 to < 18.5 kg/m2) 0 3 4 7 (46.7)
 Healthy weight (18.5 to < 25.0 kg/m2) 2 4 1 7 (46.7)
 Overweight (≥ 25 kg/m2) 1 0 0 1 (6.7)
Primary DEB diagnosis and subtype, n (%)     
 Recessive DEB 8 10 11 29 (96.7)
  Generalized severe 0 0 9 9 (30.0)
  Generalized intermediate 6 9 2 17 (56.7)
  Inversa 2 1 0 3 (10.0)
 Dominant DEB 1 0 0 1 (3.3)
  Other subtype 1 0 0 1 (3.3)
Medical history, n (%)     
 Dysphagia 5 7 11 23 (76.7)
 Esophageal stenosis 3 7 10 20 (66.7)
 Microstomia 3 3 7 13 (43.3)
 Squamous cell carcinomaa 0 2 1 3 (10.0)
Surgical/procedural history, n (%)     
 Esophageal dilation 0 4 5 9 (30.0)
 Hand repair operation 0 3 3 6 (20.0)
 Gastrostomy 0 2 2 4 (13.3)
 Finger repair operation 0 1 2 3 (10.0)
 Gastrointestinal tube insertion 0 0 3 3 (10.0)
  1. BMI body mass index, DDEB dominant dystrophic epidermolysis bullosa, DEB dystrophic epidermolysis bullosa
  2. aAll in patients aged > 37 years