ReviewArchives of disease in childhood2000
Dilated cardiomyopathy in dystrophic epidermolysis bullosa.
Review in Archives of disease in childhood, 2000. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 2 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
12 citing papers in PubMed, 2 syntheses or guidelines pooled it, 63 citations in OpenAlex.
- A systematic literature review of the disease burden in patients with recessive dystrophic epidermolysis bullosa.Orphanet journal of rare diseases · 2021Pooled it
- Clinical practice guidelines: Oral health care for children and adults living with epidermolysis bullosa.Special care in dentistry : official publication of the American Association of Hospital Dentists, the Academy of Dentistry for the Handicapped, and the American Society for Geriatric Dentistry · 2020Pooled it
- A Single Center Retrospective Chart Review of Monitoring Laboratory Tests in Recessive Dystrophic Epidermolysis Bullosa.Pediatric dermatology · 2026Article
- Rare variants in cardiomyopathy genes predispose to cardiac injury in severe COVID-19 patients of African or Hispanic ancestry.Journal of molecular medicine (Berlin, Germany) · 2025Article
- Heterozygous nonsense variants in laminin subunit 3α resulting in Ebstein's anomaly.HGG advances · 2023Article
- The analysis of echocardiographic results in patients suffering from epidermolysis bullosa.Postepy dermatologii i alergologii · 2020Article
- The Extracellular Matrix in Ischemic and Nonischemic Heart Failure.Circulation research · 2019Review
- Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa.Orphanet journal of rare diseases · 2014Review
- Clinical practice: heart failure in children. Part I: clinical evaluation, diagnostic testing, and initial medical management.European journal of pediatrics · 2010Review
- The risk of cardiomyopathy in inherited epidermolysis bullosa.The British journal of dermatology · 2008Article
- Dystrophic epidermolysis bullosa.Journal of perinatology : official journal of the California Perinatal Association · 2004Review
- Maintenance Hemodialysis Using Native Arteriovenous Fistula in a Patient with Severe Generalized Recessive Dystrophic Epidermolysis Bullosa.Case reports in nephrology and dialysisArticle
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundDystrophic epidermolysis bullosa (DEB) is an uncommon genetic disorder of the skin and mucosae. In 1996, we reported the occurrence of lethal dilated cardiomyopathy (DCM) in two affected children.
methodsIn the past seven years we have routinely screened patients with severe DEB who have been under the care of this hospital by yearly clinical review, echocardiography, and quantification of plasma selenium and carnitine concentrations, as deficiency of these micronutrients is known to be associated with the development of DCM.
resultsSix of 61 children have developed DCM over the seven year period of this study, four of whom have not been previously reported, and three of whom have since died. We compared the concentrations of selenium and free and total carnitine in the children who developed DCM to concentrations in those with severe DEB who did not. The concentrations of free and total carnitine when first measured were significantly lower in the children with DCM, but the selenium concentrations were not.
conclusionsWe now believe that DCM is a not infrequent complication of severe recessive DEB, and may be related in part to carnitine concentrations, though the exact mechanism remains unclear. We therefore recommend that patients with this condition should undergo regular cardiac review including echocardiography.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.