Document Detail

Pulmonary aneurysms and intracardiac thrombi due to Behçet's disease in an African-American adolescent with oculocutaneous albinism.
MedLine Citation:
PMID:  17047893     Owner:  NLM     Status:  MEDLINE    
Behçet's disease (BD) is a systemic vasculitis that may involve a variety of organs. We describe a girl with oculocutaneous albinism (OCA) who, on initial evaluation for popliteal artery occlusion, was noted to have multiple cardiac thrombi. She later developed hemoptysis, which was the result of bilateral pulmonary artery aneurysms. Her clinical picture was consistent with BD. She was initially treated with prednisone and cyclophosphamide, followed by maintenance therapy with infliximab. This case describes a unique patient with OCA who developed BD with large pulmonary aneurysms and has remained stable for over 1 year while receiving infliximab.
Lois M Endo; Steven M Rowe; Robb L Romp; Mark A Buckmaster; T Prescott Atkinson
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2006-09-19
Journal Detail:
Title:  Clinical rheumatology     Volume:  26     ISSN:  0770-3198     ISO Abbreviation:  Clin. Rheumatol.     Publication Date:  2007 Sep 
Date Detail:
Created Date:  2007-07-30     Completed Date:  2007-10-03     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8211469     Medline TA:  Clin Rheumatol     Country:  Belgium    
Other Details:
Languages:  eng     Pagination:  1537-9     Citation Subset:  IM    
Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA.
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MeSH Terms
African Americans
Albinism, Oculocutaneous / complications*
Aneurysm / etiology*
Antibodies, Monoclonal / therapeutic use
Behcet Syndrome / complications*,  diagnosis,  drug therapy
Heart Diseases / etiology*
Pulmonary Artery / physiopathology
Thrombosis / etiology*
Tumor Necrosis Factor-alpha / antagonists & inhibitors
Vasculitis / drug therapy,  etiology
Reg. No./Substance:
0/Antibodies, Monoclonal; 0/Tumor Necrosis Factor-alpha; 0/infliximab

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