Document Detail


Histopathology of an iliac aneurysm in a case of Menkes disease.
MedLine Citation:
PMID:  19522551     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
In Menkes disease, arterial tortuosity is frequent, whereas true aneurysms are rare. Here, we report aneurysmal pathology occurring in an infant with Menkes disease. An iliac aneurysm was diagnosed in a 2-month-old boy and attributed to Menkes syndrome on the basis of plasma copper deficiency. Samples of the aneurysmal wall were taken during surgery for histopathological analysis. As in other forms of aneurysm, the arterial wall was characterized by smooth muscle cell (SMC) disappearance, linked to SMC apoptosis and oxidative stress, areas of mucoid degeneration, and extracellular matrix breakdown, including disappearance of elastic fibers and presence of abnormal collagen.
Authors:
Luciano de Figueiredo Borges; Helene Martelli; Monique Fabre; Ziad Touat; Guillaume Jondeau; Jean-Baptiste Michel
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society     Volume:  13     ISSN:  1093-5266     ISO Abbreviation:  Pediatr. Dev. Pathol.     Publication Date:    2010 May-Jun
Date Detail:
Created Date:  2010-07-27     Completed Date:  2010-09-02     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9809673     Medline TA:  Pediatr Dev Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  247-51     Citation Subset:  IM    
Affiliation:
INSERM U698, CHU X. Bichat, Paris, France.
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MeSH Terms
Descriptor/Qualifier:
Ceruloplasmin / analysis
Copper / blood
Endoscopy
Humans
Iliac Aneurysm / blood,  complications,  pathology*,  surgery
Infant
Male
Menkes Kinky Hair Syndrome / blood,  complications,  pathology*
Treatment Outcome
Vascular Surgical Procedures
Chemical
Reg. No./Substance:
7440-50-8/Copper; EC 1.16.3.1/Ceruloplasmin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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