The challenges of meeting nutritional requirements in children and adults with epidermolysis bullosa: proceedings of a multidisciplinary team study day

Hubbard, Lynne D.; Haynes, Lesley; Sklar, Michael C.; Martinéz, Anna E.; Mellerio, J.

doi:10.1111/j.1365-2230.2011.04091.x

Cited by 36 publications

(45 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Our study findings are consistent with several published smaller reviews and case-series, which all indicate anemia is prevalent in EB patients Fridge and Vichinsky, 1998, Hubbard et al, 2011, Kuo et al, 2006, Van den Akker et al, 2009. 54% of JEB-GS patients in a small longitudinal retrospective study ( n = 22) performed in Netherlands were reported to have anemia as one complication, without going into details as to severity [20].…”

Section: Discussionsupporting

confidence: 92%

See 1 more Smart Citation

Prevalence of anemia in patients with epidermolysis bullosa registered in Australia

Hwang

Daniel

Fergie

et al. 2015

International Journal of Women's Dermatology

View full text Add to dashboard Cite

BackgroundAnemia is a common complication of epidermolysis bullosa (EB). To date, no extensive data on the prevalence of anemia in EB patients have been well characterized worldwide.ObjectiveTo determine and to characterize the prevalence of anemia in the Australian EB population by conducting a retrospective cross-sectional study.MethodsAll (n = 368) EB patients registered in the Australasian Epidermolysis Bullosa Registry (AEBR) from 2006 to 2012 were reviewed for pathological evidence of anemia. Patients with EB without anemia and those without hematological parameters were excluded from the study. Patients’ particulars were separated into pediatric (< 18 years old) and adult (≥ 18 years old) male and female subgroups.ResultsOne-hundred sixty-nine out of 368 EB patients had eligible blood results to be analyzed, as milder forms of EB did not routinely have laboratory testing; 27.8% (n = 47/169) of EB patients were anemic at any time point in their lifetime. All generalized severe junctional EB (JEB-GS) cases (100%, n = 4/4); 68.0% (n = 17/25) of recessive dystrophic EB (RDEB); and 37.5% (n = 6/16) of generalized intermediate JEB (JEB-I) patients were anemic.LimitationsAs EB is an orphan disease, the limited sample size may have affected the significance of the study result.ConclusionThe high prevalence of anemia seen in RDEB and JEB generalized severe (JEB-GS) patients in our cohort is similar to those reported in case series.

show abstract

Section: Discussionsupporting

confidence: 92%

“…Hubbard et al (Hubbard et al, 2011) found that 96% ( n = 52/54) of pediatric RDEB patients were anemic in a cross-sectional study conducted in a multidisciplinary team study day in London. This study was limited to more severe patients already seeing a dietician and multidisciplinary team.…”

Section: Discussionmentioning

confidence: 99%

Prevalence of anemia in patients with epidermolysis bullosa registered in Australia

Hwang

Daniel

Fergie

et al. 2015

International Journal of Women's Dermatology

View full text Add to dashboard Cite

show abstract

“…13,14 Malnutrition leads to failure to thrive, delayed puberty, and anemia, a cascade of clinical and biological events, further affecting wound healing and increasing skin breakdown. 15-17 Low protein intake or relative deficiency can prevent the production of granulation tissue contributing to “stalled” healing. 18 Albumin levels, a gross indicator of long-term nutritional deficit, less than 2.0 to 3.0 g/dL (normal: 3.0-5.4 g/dL) 19 are associated with impaired healing.…”

Section: Resultsmentioning

confidence: 99%

A consensus approach to wound care in epidermolysis bullosa

Pope

Lara‐Corrales

Mellerio

et al. 2012

Journal of the American Academy of Dermatology

160

175

View full text Add to dashboard Cite

Background Wound care is the cornerstone of treatment for patients with epidermolysis bullosa (EB); however, there are currently no guidelines to help practitioners care for these patients. Objectives The objective of this study was to generate a list of recommendations that will enable practitioners to better care for patients with EB. Methods An expert panel generated a list of recommendations based on the best evidence available. The recommendations were translated into a survey, and sent to other EB experts to generate consensus using an online-based modified Delphi method. The list was refined and grouped into themes and specific recommendations. Results There were15 respondents (45% response rate), with significant experience in the EB field (>10 years [67%]). Respondents included physicians (67%), nurses (17%), and allied health professionals (7%). There was more than 85% agreement for all the proposed items. These were further refined and grouped into 5 main themes (assessment and management of factors that impair healing, patient-centered concerns, local wound care, development of an individualized care plan, and organizational support) and 17 specific recommendations. Limitations There is a paucity of scientific evidence with most recommendations based on expert opinion. Conclusions These recommendations will provide practitioners with a framework for caring for these patients. Additional scientific research including effectiveness studies for everyday practice and expert consensus, may further refine these recommendations.

show abstract

“…In addition, an insufficient fluid and fibre intake frequently causes constipation, which can induce painful defecation (anal fissures). Overall, nutritional compromise is more important in generalized forms of RDEB and JEB [80-82]. …”

Section: Care Of the Eb Newborn And Infantmentioning

confidence: 99%

Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa

Hachem

Zambruno

Bourdon-Lanoy

et al. 2014

Orphanet J Rare Dis

149

136

View full text Add to dashboard Cite

BackgroundInherited epidermolysis bullosa (EB) comprises a highly heterogeneous group of rare diseases characterized by fragility and blistering of skin and mucous membranes. Clinical features combined with immunofluorescence antigen mapping and/or electron microscopy examination of a skin biopsy allow to define the EB type and subtype. Molecular diagnosis is nowadays feasible in all EB subtypes and required for prenatal diagnosis. The extent of skin and mucosal lesions varies greatly depending on EB subtype and patient age. In the more severe EB subtypes lifelong generalized blistering, chronic ulcerations and scarring sequelae lead to multiorgan involvement, major morbidity and life-threatening complications. In the absence of a cure, patient management remains based on preventive measures, together with symptomatic treatment of cutaneous and extracutaneous manifestations and complications. The rarity and complexity of EB challenge its appropriate care. Thus, the aim of the present study has been to generate multicentre, multidisciplinary recommendations on global skin care addressed to physicians, nurses and other health professionals dealing with EB, both in centres of expertise and primary care setting.MethodsAlmost no controlled trials for EB treatment have been performed to date. For this reason, recommendations were prepared by a multidisciplinary team of experts from different European EB centres based on available literature and expert opinion. They have been subsequently revised by a panel of external experts, using an online-modified Delphi method to generate consensus.ResultsRecommendations are reported according to the age of the patients. The major topics treated comprise the multidisciplinary approach to EB patients, global skin care including wound care, management of itching and pain, and early diagnosis of squamous cell carcinoma. Aspects of therapeutic patient education, care of disease burden and continuity of care are also developed.ConclusionThe recommendations are expected to be useful for daily global care of EB patients, in particular in the community setting. An optimal management of patients is also a prerequisite to allow them to benefit from the specific molecular and cell-based treatments currently under development.

show abstract

The challenges of meeting nutritional requirements in children and adults with epidermolysis bullosa: proceedings of a multidisciplinary team study day

Cited by 36 publications

References 5 publications

Prevalence of anemia in patients with epidermolysis bullosa registered in Australia

Prevalence of anemia in patients with epidermolysis bullosa registered in Australia

A consensus approach to wound care in epidermolysis bullosa

Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa

Contact Info

Product

Resources

About