Rapid progression to pulmonary arterial hypertension crisis associated with mixed connective tissue disease in an 11-year-old girl

Okura, Yuka; Takezaki, Shunichiro; Yamazaki, Yasuhiro; Yamada, Masafumi; Kobayashi, Ichiro; Ariga, Tadashi

Rapid progression to pulmonary arterial hypertension crisis associated with mixed connective tissue disease in an 11-year-old girl

Okura, Yuka; Takezaki, Shunichiro; Yamazaki, Yasuhiro et al.

European journal of pediatrics, 2013, 172(9), 1263-1265

Permalink : https://hdl.handle.net/2115/56838

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アクセス権	open access
URI	https://hdl.handle.net/2115/56838
タイトル	Rapid progression to pulmonary arterial hypertension crisis associated with mixed connective tissue disease in an 11-year-old girl
著者	Okura, Yuka ; Takezaki, Shunichiro ; Yamazaki, Yasuhiro ; Yamada, Masafumi ; Kobayashi, Ichiro ; Ariga, Tadashi
著者名 Okura, Yuka
著者名 Takezaki, Shunichiro
著者名 Yamazaki, Yasuhiro
著者名 Yamada, Masafumi
著者名 Kobayashi, Ichiro
e-Rad_Researcher 60322806 著者名 Ariga, Tadashi
言語	英語
キーワード	Mixed connective tissue disease
	Pulmonary arterial hypertension
	Pulmonary arterial hypertension crisis
	Pulmonary function test
	B-type natriuretic peptide
発行日	2013-09
出版者	Springer
収録物名	European journal of pediatrics
巻(号)	172(9)
ページ	1263-1265
抄録	Mixed connective tissue disease (MCTD) is rare in pediatric rheumatic diseases. Pulmonary arterial hypertension (PAH) associated with MCTD usually progresses gradually and is difficult to note at the asymptomatic phase. We report a 11-year-old girl with MCTD complicated with rapidly progressive PAH. Although PAH was not detected by echocardiogram or chest CT scan at the initial examination, it became clear in 1 year and suddenly came to cardiac arrest during an invasive procedure. She was successfully treated with extracorporeal assist and both vasodilative and immunosuppressive medication. A combination of echocardiogram and plasma BNP levels could be a useful marker for the follow-up of such cases. PAH could develop early in the course of pediatric MCTD and needs attention to unexpected acute exacerbation, especially under emotional stress.
資源タイプ	学術雑誌論文
出版タイプ	Accepted Manuscript
権利情報	The final publication is available at link.springer.com
PISSN	0340-6199
NCID	AA00639676
関連情報	PMID 23686514
関連情報 (isVersionOf)	出版社版DOI https://doi.org/10.1007/s00431-013-2034-3