Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome.

Citation data:

European journal of pediatric surgery reports, ISSN: 2194-7619, Vol: 3, Issue: 1, Page: 33-9

Publication Year:
2015
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PMID:
26171313
DOI:
10.1055/s-0034-1395986
PMCID:
PMC4487116
Author(s):
Muensterer, Oliver, Abellar, Rosanna, Otterburn, David, Mathew, Rajamma
Publisher(s):
Thieme Publishing Group, Georg Thieme Verlag KG
article description
Pulmonary agenesis is a rare congenital disorder with large variability in presentation and prognosis. We describe a full-term infant born with right-sided pulmonary agenesis who underwent thoracoscopic placement of a tissue expander. He ultimately died of pulmonary hypertension. Immunohistology showed intimal hyperplasia without the loss of endothelial caveolin-1 expression. A literature review revealed that while some of these patients have favorable outcome, many succumb despite therapy.

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