Correlates of low bone mass in children with generalized forms of epidermolysis bullosa

Bruckner, Anna L.; Bedocs, Laleh A.; Keiser, Elizabeth; Tang, Jean Y.; Doernbrack, Catherine; Arbuckle, H. Alan; Berman, Stephen; Kent, Kyla; Bachrach, Laura K.

doi:10.1016/j.jaad.2010.08.028

Cited by 35 publications

(53 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…As such, the extent of skin blistering may be described as a marker of disease severity. They also confirmed that elevated CRP and ESR were associated with reduced BMD compared to age‐matched counterparts …”

Section: Methodsmentioning

confidence: 56%

“…However, its small sample size and convenience sampling method means that multivariate linear regression analysis could not be performed. Thus, the impact of different predictors related to BMD could not be established …”

Section: Methodsmentioning

confidence: 99%

“…Clinically, inflammatory markers such as C‐reactive protein (CRP), erythrocyte sedimentation rate (ESR), ferritin and platelet counts will be significantly raised . Elevated serum interleukin‐6 has also been shown to be predictive of decreased bone mineral density in other inflammatory diseases, such as ulcerative colitis …”

Section: Methodsmentioning

confidence: 99%

See 2 more Smart Citations

Prevalence and pathogenesis of osteopenia and osteoporosis in epidermolysis bullosa: An evidence‐based review

Chen

Yang

Murrell

2018

Experimental Dermatology

View full text Add to dashboard Cite

Background Osteopenia or osteoporosis is one of the many comorbidities in patients with Epidermolysis Bullosa (EB). Current literature on the prevalence of osteoporosis in EB is scarce. Objective This review will analyse the current literature in the field of patients with compromised bone health in EB and any articles on the prevalence of such diseases in EB groups. Methods A systematic search for articles related to bone health and epidermolysis bullosa (EB) (1946‐2017) was performed on seven databases: MEDLINE, EMBASE and EBM, PubMed, ProQuest, Scopus and Web of Science. Search terms: epidermolysis bullosa, osteop*, bone mass, bone mineral*, fracture, dual X‐ray absorptiometry, vitamin D, calcium, nutrition, exercise and physical activity. Abstracts from all search results were screened, and reference scanning of the search results was performed. Eighty‐three articles met the selection criteria and were considered for review. Letters to the editor and abstract‐only articles were excluded. Articles were favoured based on citation count, impact factor of their journal and study sample size. The search included all languages. Results The searches yielded a total of 1309 articles including 717 duplicates. The remaining 592 articles were screened by title and abstracts. Eighty‐three full‐text articles were analysed. Twenty‐one articles directly relating to bone health in EB were included. Three descriptive studies and one case‐control study were found, indicating a need for research of larger scale. Conclusion Further investigations into osteoporosis in EB, especially the milder forms of EB, are valuable in providing evidence to support guideline developments for EB bone health management.

show abstract

Section: Methodsmentioning

confidence: 56%

Section: Methodsmentioning

confidence: 99%

See 1 more Smart Citation

Prevalence and pathogenesis of osteopenia and osteoporosis in epidermolysis bullosa: An evidence‐based review

Chen

Yang

Murrell

2018

Experimental Dermatology

View full text Add to dashboard Cite

show abstract

“…Other contributing factors for the poor BMD for these patients include reduced weight-bearing activities, nutritional imbalance, pubertal delay, low vitamin D level from reduced UV exposure, and increased osteoclastic activity in the presence of chronic inflammation and recurrent infection (15). Bruckner et al (16) demonstrated that short stature, a greater extent of skin blistering, and anemia also correlate with lower BMD in RDEB. A subset of patients with RDEB also suffers from liver and/or renal complications, resulting in impaired activation of vitamin D.…”

Section: What Is Known About Osteoporosis?mentioning

confidence: 99%

“…A few cases have also been reported in patients with JEB. According to the National EB Registry (NEBR) data, the cumulative risks of developing dilated cardiomyopathy after the age of 20 in severe generalized RDEB (RDEB-SG), JEB and intermediate generalized RDEB (RDEB-O) were 4.5%, 1.1% and 0.4%, retrospectively (16). Despite a low incidence, dilated cardiomyopathy is a fatal complication with high mortality.…”

Section: What Is Known About Dilated Cardiomyopathy In Epidermolysis mentioning

confidence: 99%

Epidermolysis Bullosa - Why Does a Multidisciplinary Team Approach Matter?

Kim¹,

Yang²,

Murrell³

2016

tdd

View full text Add to dashboard Cite

Epidermolysis bullosa (EB) is a rare inherited group of mechanobullous genodermatoses. It is known to be one of the most devastating chronic conditions due to its frequent and detrimental cutaneous and extracutaneous complications. Current treatment is aimed at optimizing the quality-of-life and effectively managing the EB associated complications. In order to provide the best possible care, clinicians need to recognize and appreciate that different subtypes of EB carry different associated co-morbidities. The complexity of medical care in these patients warrants a regular review by a multidisciplinary team experienced in EB. Optimal collaboration and coordination between professionals in the delivery of integrated care is essential for the provision of high-quality care in patients with EB. Öz AbstractEpidermolysis bullosa (EB) mekano-büllöz genodermatozların nadir kalıtsal bir grubudur. Sık ve zarar verici kutanöz ve ekstra-kutanöz komplikasyonlarından dolayı en harap edici kronik durumlardan biri olarak bilinir. Mevcut tedavi yaşam kalitesini optimize etmeyi ve EB ile ilişkili komplikasyonları etkili bir şekilde yönetmeyi amaçlar. Olası en iyi tedavinin sağlanabilmesi için klinisyenlerin EB'nin farklı alt tiplerinin farklı komorbiditeler taşıyabileceğinin farkında olması ve takdir etmesi gerekmektedir. Bu hastalarda medikal tedavinin karmaşıklığı, EB alanında tecrübeli bir multidispliner bir ekip tarafından konunun düzenli olarak değerlendirilmesi gereksinimini haklı kılmaktadır. Entegre bakımda profesyoneller arasında optimal düzeyde bir işbirliği ve koordinasyon EB'li hastalarda yüksek kaliteli bakımın sağlanmasında oldukça gereklidir.

show abstract