Document Detail


Pleuropulmonary pathology of vascular Ehlers-Danlos syndrome: spontaneous laceration, haematoma and fibrous nodules.
MedLine Citation:
PMID:  20500229     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
AIMS: The aim was to clarify the pleuropulmonary pathological findings of vascular Ehlers-Danlos syndrome (vEDS). METHODS AND RESULTS: Nine patients with confirmed vEDS by means of cell culture and/or molecular biological studies who had undergone surgical lung biopsy (SLB), lobectomy or autopsy were studied. Six patients were male and three were female with a mean age of 23.2 years. Histological features were as follows: (i) the main pulmonary lesions related to fragility and spontaneous laceration, these being haematomas in seven, acute haemorrhage in nine, fibrous nodule in eight, with ossification or bone marrow formation in six; vascular disruption in five; intraluminal haemosiderosis in nine; interstitial haemosiderosis in seven, with iron deposition in the alveolar wall and/or vessel wall in five and foreign body reaction in two; emphysematous changes in eight; and bleb formation in two; (ii) secondary iatrogenic pleuropulmonary injuries during SLB or lobectomy comprised pleural laceration in seven of 10 and lung laceration in eight of 10 specimens. CONCLUSIONS: Spontaneous laceration of lung tissue is an essential feature and is followed by haematoma and possible fibrous nodule formation.
Authors:
Yoshinori Kawabata; Akira Watanabe; Shozaburo Yamaguchi; Masahiro Aoshima; Akira Shiraki; Atushi Hatamochi; Tetsuji Kawamura; Takashi Uchiyama; Atushi Watanabe; Yuh Fukuda
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Publication Detail:
Type:  Journal Article     Date:  2010-05-24
Journal Detail:
Title:  Histopathology     Volume:  56     ISSN:  1365-2559     ISO Abbreviation:  Histopathology     Publication Date:  2010 Jun 
Date Detail:
Created Date:  2010-07-19     Completed Date:  2010-10-25     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7704136     Medline TA:  Histopathology     Country:  England    
Other Details:
Languages:  eng     Pagination:  944-50     Citation Subset:  IM    
Affiliation:
Division of Pathology, Saitama Cardiovascular and Respiratory Centre, Saitama, Japan. k369900q@pref.saitama.lg.jp
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Ehlers-Danlos Syndrome / pathology*
Female
Hematoma / pathology*
Humans
Lacerations / pathology*
Lung / pathology*
Male

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