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Table 2 Overview of evidence per outcome

From: Occupational therapy for epidermolysis bullosa: clinical practice guidelines

Outcome Number of allocated papers Total participants with EB * Methodologies SIGN rate ref Average quality rate % (range) Benefits and limitations
Activities of Daily Living Relating to Self Care 9 3496*
EBS 1856+
JEB 297+
DEB 486+
RDEB 505+
KS
2 NEBR
2 qualitative study
3 expert opinion
1CG
1 consensus
2-[8]
3[9]
3[10]
3[14]
4[11]
4[12]
4[13]
4[7]
4[15]
48
(19-75)
Six articles did not specify EB subtype numbers. Two studies are based on NEBR database of 3280 subjects. This affected the total number of participants. Three articles are expert opinion based on limited literature review. One article states numbers of children 140 to adults 234 and another Male 11 to Female 13.
Instrumental Activities of Daily Living 5 115*
EBS 62+
JEB 8+
DEB 25+
RDEB 16+
1 qualitative study
1 quantitative study
1 systematic review
2 expert opinion
3[10]
3[16]
1-[17]α
4[18]
4[19]
55
(28-75)
Three articles did not specify EB subtype numbers. One was not specific to persons with EB. One states numbers of male 11 to female 13 participants. Two are expert opinion based on limited literature review.
Maximization of Hand Function 8 3351*
EBS 1700+
JEB 247+
DEB 437+
RDEB 457+
1 NEBR
5 expert opinion
1 consensus
1 cross sectional
3[9]
4[11]
4[13]
4[20]
4[21]
4[15]
3[22]
4[23]
41
(19-71)
Six articles did not specify EB subtype numbers. One study used a database of 3280 subjects. One qualitative questionnaire study was carries out in children age 2-18 years old and had 39 girls to 32 boys’ participants. Four articles are expert opinion based on limited literature review.
Fine Motor Development and Fine Motor Retention 5 3403*
EBS 1710+
JEB 247+
DEB 442+
RDEB 473+
1 NEBR
2 expert opinion
1 cross sectional
1 pilot study
3[9]
4[11]
4[21]
3[22]
3[24, 25]β
54
(19-71)
Five articles did not specify EB subtype numbers. One study used the NEBR database (n=3280). One was a pilot study that has been recently validated, [25]β but published post appraisal stage. This study was carried out in children (16) and adults (15); age 1-50 years old and had 14 females to 17 male participants. Two are expert opinion based on limited literature review.
Oral Feeding Skills 5 No values 5 Expert opinion 4[13]
4[21]
4[19]
4[26]
4[27]
42
(28-53)
No articles specify EB subtype numbers. All articles were expert opinion, based on limited literature review.
  1. Key: *total number of persons with EB in all papers combined; Ref references, % percentage, EB epidermolysis bullosa, RDEB recessive dystrophic epidermolysis bullosa, JEB Junctional epidermolysis bullosa, DDEB dominant dystrophic epidermolysis bullosa, EBS Epidermolysis bullosa simplex, NEBR National EB Registry, KS Kindler syndrome, +: value can be more as some papers did not state values, : reported present but no value given, CG consensus guideline, n: number of, α: article not on EB population, β: Article 25 was not appraised due to being published past the appraisal period