| Histopathology of an iliac aneurysm in a case of Menkes disease. | |
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MedLine Citation:
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PMID: 19522551 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Luciano de Figueiredo Borges; Helene Martelli; Monique Fabre; Ziad Touat; Guillaume Jondeau; Jean-Baptiste Michel |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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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:
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Created Date: 2010-07-27 Completed Date: 2010-09-02 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9809673 Medline TA: Pediatr Dev Pathol Country: United States |
Other Details:
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Languages: eng Pagination: 247-51 Citation Subset: IM |
Affiliation:
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INSERM U698, CHU X. Bichat, Paris, France. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Ceruloplasmin
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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:
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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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