Fifteen-year experience of pediatric-onset mixed connective tissue disease
Identifieur interne : 001871 ( Main/Exploration ); précédent : 001870; suivant : 001872Fifteen-year experience of pediatric-onset mixed connective tissue disease
Auteurs : Yi-Ying Tsai [République populaire de Chine] ; Yao-Hsu Yang [République populaire de Chine] ; Hsin-Hui Yu [République populaire de Chine] ; Li-Chieh Wang [République populaire de Chine] ; Jyh-Hong Lee [République populaire de Chine] ; Bor-Luen Chiang [République populaire de Chine, Taïwan]Source :
- Clinical Rheumatology [ 0770-3198 ] ; 2010-01-01.
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Abstract
Abstract: The aim of this study was to investigate the initial clinical manifestations, laboratory data, complications, and outcomes of patients with pediatric-onset mixed connective tissue disease (MCTD) in Taiwan. We reviewed medical charts of patients younger than 18 years with a diagnosis of mixed connective tissue disease based on the criteria of Kasukawa (1) at the pediatric department of National Taiwan University Hospital from 1993 to 2008. A total of 12 patients were included. All of the patients were female. The mean age at disease onset was 10.7 years (range 6.5 to 14 years). The most common symptoms at disease onset were polyarthritis (7/12 patients) and Raynaud's phenomenon (7/12 patients). The clinical symptoms changed with time, and other symptoms encompassing the criteria for MCTD developed sequentially. Inflammatory manifestations (arthritis, fever, and skin rash) improved following treatment, whereas sclerodermatous features (sclerodactyly, esophageal disease, and vasculopathy) persisted and were often unresponsive to therapy. The organ involvement-free rates at 2 years, 5 years, and 10 years were 91.7%, 78.6%, and 52.4%, respectively. In this retrospective study, sclerodermatous changes of internal organs were a poor prognostic factor in our population, and we emphasize that long-term follow-up is necessary, and appropriate treatment should be applied to improve the outcomes.
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DOI: 10.1007/s10067-009-1276-y
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type="ISSN">0770-3198</idno><idno type="eISSN">1434-9949</idno><imprint><publisher>Springer-Verlag</publisher><pubPlace>London</pubPlace><date type="published" when="2010-01-01">2010-01-01</date><biblScope unit="volume">29</biblScope><biblScope unit="issue">1</biblScope><biblScope unit="page" from="53">53</biblScope><biblScope unit="page" to="58">58</biblScope></imprint><idno type="ISSN">0770-3198</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0770-3198</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Mixed connective tissue disease</term><term>Pediatric onset</term><term>Retrospective study</term><term>Sclerodermatous changes</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Abstract: The aim of this study was to investigate the initial clinical manifestations, laboratory data, complications, and outcomes of patients with pediatric-onset mixed connective tissue disease (MCTD) in Taiwan. We reviewed medical charts of patients younger than 18 years with a diagnosis of mixed connective tissue disease based on the criteria of Kasukawa (1) at the pediatric department of National Taiwan University Hospital from 1993 to 2008. A total of 12 patients were included. All of the patients were female. The mean age at disease onset was 10.7 years (range 6.5 to 14 years). The most common symptoms at disease onset were polyarthritis (7/12 patients) and Raynaud's phenomenon (7/12 patients). The clinical symptoms changed with time, and other symptoms encompassing the criteria for MCTD developed sequentially. Inflammatory manifestations (arthritis, fever, and skin rash) improved following treatment, whereas sclerodermatous features (sclerodactyly, esophageal disease, and vasculopathy) persisted and were often unresponsive to therapy. The organ involvement-free rates at 2 years, 5 years, and 10 years were 91.7%, 78.6%, and 52.4%, respectively. In this retrospective study, sclerodermatous changes of internal organs were a poor prognostic factor in our population, and we emphasize that long-term follow-up is necessary, and appropriate treatment should be applied to improve the outcomes.</div></front></TEI><affiliations><list><country><li>République populaire de Chine</li><li>Taïwan</li></country></list><tree><country name="République populaire de Chine"><noRegion><name sortKey="Tsai, Yi Ying" sort="Tsai, Yi Ying" uniqKey="Tsai Y" first="Yi-Ying" last="Tsai">Yi-Ying Tsai</name></noRegion><name sortKey="Chiang, Bor Luen" sort="Chiang, Bor Luen" uniqKey="Chiang B" first="Bor-Luen" last="Chiang">Bor-Luen Chiang</name><name sortKey="Lee, Jyh Hong" sort="Lee, Jyh Hong" uniqKey="Lee J" first="Jyh-Hong" last="Lee">Jyh-Hong Lee</name><name sortKey="Wang, Li Chieh" sort="Wang, Li Chieh" uniqKey="Wang L" first="Li-Chieh" last="Wang">Li-Chieh Wang</name><name sortKey="Yang, Yao Hsu" sort="Yang, Yao Hsu" uniqKey="Yang Y" first="Yao-Hsu" last="Yang">Yao-Hsu Yang</name><name sortKey="Yu, Hsin Hui" sort="Yu, Hsin Hui" uniqKey="Yu H" first="Hsin-Hui" last="Yu">Hsin-Hui Yu</name></country><country name="Taïwan"><noRegion><name sortKey="Chiang, Bor Luen" sort="Chiang, Bor Luen" uniqKey="Chiang B" first="Bor-Luen" last="Chiang">Bor-Luen Chiang</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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