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

doi:10.1007/s00431-013-2034-3


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

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Title:	Rapid progression to pulmonary arterial hypertension crisis associated with mixed connective tissue disease in an 11-year-old girl
Authors:	Okura, Yuka Browse this author
	Takezaki, Shunichiro Browse this author
	Yamazaki, Yasuhiro Browse this author
	Yamada, Masafumi Browse this author
	Kobayashi, Ichiro Browse this author
	Ariga, Tadashi Browse this author →KAKEN DB
Keywords:	Mixed connective tissue disease
	Pulmonary arterial hypertension
	Pulmonary arterial hypertension crisis
	Pulmonary function test
	B-type natriuretic peptide
Issue Date:	Sep-2013
Publisher:	Springer
Journal Title:	European journal of pediatrics
Volume:	172
Issue:	9
Start Page:	1263
End Page:	1265
Publisher DOI:	10.1007/s00431-013-2034-3
PMID:	23686514
Abstract:	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.
Rights:	The final publication is available at link.springer.com
Type:	article (author version)
URI:	http://hdl.handle.net/2115/56838
Appears in Collections:	医学院・医学研究院 (Graduate School of Medicine / Faculty of Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

Submitter: 有賀正

OAI-PMH ( junii2 , jpcoar_1.0 )

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