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

 

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dc.contributor.author Okong’o, Lawrence O
dc.contributor.author Esser, Monika
dc.contributor.author Wilmshurst, Jo
dc.contributor.author Scott, Christiaan
dc.date.accessioned 2017-03-16T12:34:13Z
dc.date.available 2017-03-16T12:34:13Z
dc.date.issued 2016-11-11
dc.identifier.citation Okong’o, L. O., Esser, M., Wilmshurst, J., & Scott, C. (2016). Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. Pediatric Rheumatology, 14(1), 60.
dc.identifier.uri http://dx.doi.org/10.1186/s12969-016-0118-0
dc.identifier.uri http://hdl.handle.net/11427/24050
dc.description.abstract Background: 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.publisher BioMed Central
dc.rights This article is distributed under the terms of the Creative Commons Attribution 4.0 International License
dc.rights.uri https://creativecommons.org/licenses/by/4.0/
dc.subject.other Juvenile dermatomyositis
dc.subject.other JDM
dc.subject.other Outcome
dc.subject.other Africa
dc.title Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study
dc.type Journal Article
dc.date.updated 2016-11-11T19:02:11Z
dc.language.rfc3066 en
dc.rights.holder The Author(s).
uct.type.publication Research en_ZA
uct.type.resource Article en_ZA
dc.publisher.institution University of Cape Town
dc.publisher.faculty Faculty of Health Sciences en_ZA
dc.publisher.department Department of Paediatrics and Child Health en_ZA
uct.type.filetype Text
uct.type.filetype Image
dc.identifier.apacitation , Esser, M., Wilmshurst, J., & Scott, C. (2016). Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. http://hdl.handle.net/11427/24050 en_ZA
dc.identifier.chicagocitation , Monika Esser, Jo Wilmshurst, and Christiaan Scott "Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study." (2016) http://hdl.handle.net/11427/24050 en_ZA
dc.identifier.vancouvercitation , Esser M, Wilmshurst J, Scott C. Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. 2016; http://hdl.handle.net/11427/24050. en_ZA
dc.identifier.ris TY - Journal Article AU - Okong’o, Lawrence O AU - Esser, Monika AU - Wilmshurst, Jo AU - Scott, Christiaan AB - Background: 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. DA - 2016-11-11 DB - OpenUCT DO - 10.1186/s12969-016-0118-0 DP - University of Cape Town LK - https://open.uct.ac.za PB - University of Cape Town PY - 2016 T1 - Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study TI - Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study UR - http://hdl.handle.net/11427/24050 ER - en_ZA


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