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Pulmonary outcome of alport syndrome with familial diffuse esophageal leiomymatosis.
MedLine Citation:
PMID:  21284097     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
X-linked Alport syndrome is associated in some families with diffuse leiomyomatosis. We describe herein, the pulmonary complications and outcome of three family members (mother, daughter and son). The three underwent esophagectomy at different age (22 years, three years and 15 months respectively). Their current forced expiratory volume in the first second (FEV1) range from 33% in the mother to 60% in the daughter and 97% in the son. It is suggested that earlier intervention may lead to improved pulmonary function tests. Pediatr. Pulmonol. © 2011 Wiley-Liss, Inc.
Authors:
Galit Livnat; Lael Anson Best; Ludmila Guralnik; Lea Bentur
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-1-31
Journal Detail:
Title:  Pediatric pulmonology     Volume:  -     ISSN:  1099-0496     ISO Abbreviation:  -     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-2-1     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8510590     Medline TA:  Pediatr Pulmonol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 Wiley-Liss, Inc.
Affiliation:
Pediatric Pulmonary Unit, Meyer Children's Hospital, Haifa, Israel; Rambam Health Care Campus, The Bruce Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel.
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