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Treatment of early-onset Gorham syndrome with 8-year follow-up.

Identifieur interne : 002246 ( PubMed/Corpus ); précédent : 002245; suivant : 002247

Treatment of early-onset Gorham syndrome with 8-year follow-up.

Auteurs : Annabel Maruani ; Sylvie Thimon ; Benoît De Courtivron ; Gonzague De Pinieux ; Françoise Baulieu ; Laurent Machet ; Gérard Lorette

Source :

RBID : pubmed:22380698

English descriptors

Abstract

A case of Gorham disease with several years of follow-up is reported. At birth he had a mass in the thigh which was had pathology demonstrating a lymphangioma. By age 3 years, he had lymphedema of the ipsilateral foot and discrepant leg lengths. Radiography revealed heterogenous dystrophy of the bones and osteolysis of the hallux. At age 6, a spontaneous fracture of the right tibia was treated with surgery. Histopathology of a bone sample demonstrated bone remodelling, fibrous tissue, and large vascular lacunas within bone tissue, bordered by cells expressing the lymphaticmarker D2-40. At 8 years old, lymphedema of the right inferior leg had increased, leg lengths still differed, but other clinical signs were absent.

DOI: 10.1111/j.1525-1470.2011.01712.x
PubMed: 22380698

Links to Exploration step

pubmed:22380698

Le document en format XML

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<title xml:lang="en">Treatment of early-onset Gorham syndrome with 8-year follow-up.</title>
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<name sortKey="Maruani, Annabel" sort="Maruani, Annabel" uniqKey="Maruani A" first="Annabel" last="Maruani">Annabel Maruani</name>
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<nlm:affiliation>Departments of Dermatology Pediatric Orthopaedic Surgery Pathology, and Nuclear Medicine, University François Rabelais Tours CHRU Tours Inserm U930, Tours, France.</nlm:affiliation>
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<name sortKey="Thimon, Sylvie" sort="Thimon, Sylvie" uniqKey="Thimon S" first="Sylvie" last="Thimon">Sylvie Thimon</name>
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<name sortKey="De Courtivron, Benoit" sort="De Courtivron, Benoit" uniqKey="De Courtivron B" first="Benoît" last="De Courtivron">Benoît De Courtivron</name>
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<name sortKey="De Pinieux, Gonzague" sort="De Pinieux, Gonzague" uniqKey="De Pinieux G" first="Gonzague" last="De Pinieux">Gonzague De Pinieux</name>
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<name sortKey="Baulieu, Francoise" sort="Baulieu, Francoise" uniqKey="Baulieu F" first="Françoise" last="Baulieu">Françoise Baulieu</name>
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<name sortKey="Machet, Laurent" sort="Machet, Laurent" uniqKey="Machet L" first="Laurent" last="Machet">Laurent Machet</name>
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<name sortKey="Lorette, Gerard" sort="Lorette, Gerard" uniqKey="Lorette G" first="Gérard" last="Lorette">Gérard Lorette</name>
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<term>Femoral Fractures (diagnostic imaging)</term>
<term>Femoral Fractures (pathology)</term>
<term>Femoral Fractures (surgery)</term>
<term>Follow-Up Studies</term>
<term>Hallux (diagnostic imaging)</term>
<term>Hallux (pathology)</term>
<term>Humans</term>
<term>Infant</term>
<term>Infant, Newborn</term>
<term>Lymphangioma (diagnostic imaging)</term>
<term>Lymphangioma (pathology)</term>
<term>Lymphedema (pathology)</term>
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<div type="abstract" xml:lang="en">A case of Gorham disease with several years of follow-up is reported. At birth he had a mass in the thigh which was had pathology demonstrating a lymphangioma. By age 3 years, he had lymphedema of the ipsilateral foot and discrepant leg lengths. Radiography revealed heterogenous dystrophy of the bones and osteolysis of the hallux. At age 6, a spontaneous fracture of the right tibia was treated with surgery. Histopathology of a bone sample demonstrated bone remodelling, fibrous tissue, and large vascular lacunas within bone tissue, bordered by cells expressing the lymphaticmarker D2-40. At 8 years old, lymphedema of the right inferior leg had increased, leg lengths still differed, but other clinical signs were absent.</div>
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<CopyrightInformation>© 2012 Wiley Periodicals, Inc.</CopyrightInformation>
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