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Pulmonary histologic changes in marfan syndrome: a case series and literature review.
MedLine Citation:
PMID:  22095370     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Marfan syndrome is one of the most common connective tissue diseases and may manifest with a range of symptoms and pathologic changes. We present a retrospective series of 5 cases of patients with Marfan syndrome and pulmonary pathology. Patients were young to middle-aged adults with absent or minimal smoking histories and absent to severe clinical pulmonary symptoms. Tissue specimens were obtained from the surgical pathology and autopsy services. Histologic examination revealed a consistent pattern of distal acinar emphysema in all patients. Comparisons are made with other cystic-type diseases of the lung that may histologically mimic this pattern. This is the largest contemporary series of histologic pulmonary involvement of Marfan syndrome and the first to describe this pattern of pulmonary changes in this patient population.
Authors:
Kathryn Dyhdalo; Carol Farver
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  American journal of clinical pathology     Volume:  136     ISSN:  1943-7722     ISO Abbreviation:  Am. J. Clin. Pathol.     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2011-11-18     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370470     Medline TA:  Am J Clin Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  857-63     Citation Subset:  AIM; IM    
Affiliation:
Dept of Anatomic Pathology L25, Pathology and Laboratory Institute, Cleveland Clinic, 9500 Euclid Ave, Cleveland, OH 44195.
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