Document Detail


Pseudoxanthoma-elasticum-like syndrome and thalassemia: an update.
MedLine Citation:
PMID:  19903435     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Pseudoxanthoma Elasticum (PXE) is an autosomal recessive, multisystem disorder affecting connective tissues. We describe three cases of the acquired PXE-like syndrome that often occurs in association with hemolytic anemias, in particular the hemoglobinopathies, and review the literature on the subject. The pathogenesis of the acquired PXE-like lesions is not yet completely understood. None of the mutations observed in the inherited form has been detected in the syndrome accompanying thalassemia. The cardiovascular complications could be life-threatening. Therefore, an close surveillance of these patients is mandatory.
Authors:
Elena Fabbri; Gian Luca Forni; Giulia Guerrini; Caterina Borgna-Pignatti
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Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2009-07-15
Journal Detail:
Title:  Dermatology online journal     Volume:  15     ISSN:  1087-2108     ISO Abbreviation:  Dermatol. Online J.     Publication Date:  2009  
Date Detail:
Created Date:  2009-11-11     Completed Date:  2010-02-18     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9610776     Medline TA:  Dermatol Online J     Country:  United States    
Other Details:
Languages:  eng     Pagination:  7     Citation Subset:  IM    
Affiliation:
Pediatrics, University of Ferrara.
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MeSH Terms
Descriptor/Qualifier:
Adult
Anemia, Sickle Cell / complications*,  therapy
Arterial Occlusive Diseases / etiology,  pathology
Blood Transfusion / adverse effects
Blood Vessels / pathology
Calcinosis / etiology,  metabolism
Dermis / pathology
Elastic Tissue / ultrastructure
Female
Humans
Iron Overload / etiology
Male
Middle Aged
Pseudoxanthoma Elasticum / etiology*,  metabolism,  pathology
Retina / pathology
Retinal Diseases / etiology,  pathology
Syndrome
Thalassemia / complications*,  therapy

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