Document Detail


A 7-year-old with pulmonary hypertension.
MedLine Citation:
PMID:  21897452     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
The authors discuss the case of a 7-year-old female who presented with exertional cyanosis and was found to have pulmonary arterial hypertension. Despite normal left-sided heart function, the patient developed pulmonary oedema in response to pulmonary vasodilator therapy, increasing suspicion for pathology in the pulmonary capillaries and veins. Lung biopsy confirmed a diagnosis of pulmonary capillary haemangiomatosis (PCH), a rare cause of pulmonary hypertension in both children and adults. The diagnosis requires lung biopsy and is often made postmortem. She was treated with interferon α-2a and doxycycline for their antiangiogenic properties and reports of disease regression. Although she initially demonstrated improvement in her pulmonary hypertension in response to these medications, she succumbed to the disease within the time frame previously reported for PCH.
Authors:
James Olin; Steven Abman; R Grady; D Ivy; Mark Lovell; David Partrick; Vivek Balasubramaniam
Publication Detail:
Type:  Journal Article     Date:  2011-04-20
Journal Detail:
Title:  BMJ case reports     Volume:  2011     ISSN:  1757-790X     ISO Abbreviation:  BMJ Case Rep     Publication Date:  2011  
Date Detail:
Created Date:  2011-09-07     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101526291     Medline TA:  BMJ Case Rep     Country:  England    
Other Details:
Languages:  eng     Pagination:  bcr0220113843     Citation Subset:  -    
Affiliation:
Department of Pediatrics, University of Colorado, Aurora, Colorado, USA.
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