Extracorporeal life support in multisystem smooth muscle dysfunction syndrome

Prabhu, Sudesh, Fox, Scott, Mattke, Adrian, Armes, Jane E. and Alphonso, Nelson (2016) Extracorporeal life support in multisystem smooth muscle dysfunction syndrome. World Journal for Pediatric and Congenital Heart Surgery, 8 6: 750-753. doi:10.1177/2150135116658457


Author Prabhu, Sudesh
Fox, Scott
Mattke, Adrian
Armes, Jane E.
Alphonso, Nelson
Title Extracorporeal life support in multisystem smooth muscle dysfunction syndrome
Journal name World Journal for Pediatric and Congenital Heart Surgery   Check publisher's open access policy
ISSN 2150-136X
2150-1351
Publication date 2016-01-01
Year available 2016
Sub-type Article (original research)
DOI 10.1177/2150135116658457
Open Access Status Not yet assessed
Volume 8
Issue 6
Start page 750
End page 753
Total pages 4
Place of publication Thousand Oaks, CA United States
Publisher Sage Publications
Language eng
Subject 2700 Medicine
Abstract We describe an infant with congenital mydriasis, patent ductus arteriosus (PDA), pulmonary hypertension, and cystic lung disease. She had all the major components of multisystemic smooth muscle dysfunction syndrome. Due to progressive respiratory deterioration, she required surgical PDA interruption, extracorporeal life support, and subsequent prolonged respiratory support. Genetic testing revealed ACTA2 R179H mutation and cystic lung disease on biopsy.
Keyword Congenital mydriasis
Patent ductus arteriosus
Cystic lung disease
Mutation
Extracorporeal life support
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status UQ
Additional Notes PMCID:27549731

Document type: Journal Article
Sub-type: Article (original research)
Collections: Mater Research Institute-UQ (MRI-UQ)
HERDC Pre-Audit
Admin Only - School of Medicine
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Created: Thu, 01 Dec 2016, 20:57:52 EST by Julia McCabe on behalf of School of Medicine