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Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study

dc.contributor.authorOkongo, Lawrence Owinoen_ZA
dc.contributor.authorEsser, Monikaen_ZA
dc.contributor.authorWilmshurst, Joen_ZA
dc.contributor.authorScott, Christiaanen_ZA
dc.date.accessioned2017-01-25T10:32:41Z
dc.date.available2017-01-25T10:32:41Z
dc.date.issued2016-11-11
dc.identifier.citationOkongo, L. O., et al. 2016. Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. Pediatric Rheumatology, 14:60, doi:10.1186/s12969-016-0118-0
dc.identifier.issn1546-0096 (Online)
dc.identifier.otherdoi:10.1186/s12969-016-0118-0
dc.identifier.urihttp://hdl.handle.net/10019.1/100521
dc.descriptionCITATION: Okongo, L. O., et al. 2016. Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. Pediatric Rheumatology, 14:60, doi:10.1186/s12969-016-0118-0.
dc.descriptionThe original publication is available at http://ped-rheum.biomedcentral.com
dc.description.abstractBackground: Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory childhood myopathy of uncertain aetiology. The demographic and clinical presentation of JDM may differ by race and geographic regions. Few studies have described the characteristics of JDM patients from Africa. Methods: We conducted a retrospective observational study to determine clinical characteristics and outcomes of patients satisfying the Bohan and Peter criteria for probable JDM seen between 2004 and 2013 in three hospitals in Cape Town, South Africa. Results: Twenty five cases were identified: 16 female and 9 male; thirteen (52 %) were of indigenous African, eleven (44 %) mixed and one (4 %) European ancestry. The median ages at disease onset and diagnosis were 6.75 (range 2.0–9.7) and 7.9 (range 3.4–9.75) years respectively. Eleven patients had calcinosis while the mortality was 2/ 25 (8 %). Only 40 % of the patients had clinically inactive disease by PRINTO criteria (modified) at last review. There was no statistically significant difference in racial distribution (p-value = 1), age at disease onset (p-value = 0.87) and disease duration prior to treatment initiation (p-value = 0.75) between patients who had clinically active and inactive disease. Conclusion: The demographic characteristics of children with JDM were similar to that from most other regions of the world with female predominance and similar age at onset. Majority of the patients remained with clinically active disease, which put them at risk of further disease complications. Long term follow up and use of appropriate treatment guidelines may be indicated in management of JDM patients for optimum treatment outcomes.
dc.description.urihttp://ped-rheum.biomedcentral.com/articles/10.1186/s12969-016-0118-0
dc.format.extent8 pages
dc.language.isoen_ZAen_ZA
dc.publisherBioMed Central
dc.subjectJuvenile dermatomyositisen_ZA
dc.titleCharacteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional studyen_ZA
dc.typeArticle
dc.date.updated2016-12-09T12:11:08Z
dc.description.versionPublisher's version
dc.rights.holderAuthor retains copyright


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