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This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Aessopos, A. Articles by Loukopoulos, D. Search for Related Content PubMed PubMed Citation Articles by Aessopos, A. Articles by Loukopoulos, D. Related Collections Red Cells Review Articles Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 1 January 2002, Vol. 99, No. 1, pp. 30-35 REVIEW ARTICLE Elastic tissue abnormalities resembling pseudoxanthoma elasticum in  thalassemia and the sickling syndromes Athanasios Aessopos, Dimitrios Farmakis, and Dimitris Loukopoulos From the First Department of Internal Medicine, University of Athens, School of Medicine, "Laiko" General Hospital, Athens, Greece. The development of clinical and histopathologic manifestations of a diffuse elastic tissue defect, resembling inherited pseudoxanthoma elasticum (PXE), has been encountered with a notable frequency in patients with  thalassemia, sickle cell disease, and sickle thalassemia. The PXE-like clinical syndrome, consisting of skin, ocular, and vascular manifestations, has a variable severity in these hemoglobinopathies and it is age-dependent, with a generally late onset, after the second decade of life. The defect is believed to be acquired rather than inherited and related to the consequences of the primary disease. The high prevalence of the findings implicates the elastic tissue injury as one of the main comorbid abnormalities encountered in  thalassemia and the sickling syndromes. In these patients a number of complications, sometimes serious, has been recognized to be related to ocular and vascular elastic tissue defects. Because several organ systems are involved, each medical specialty should be aware of the phenomenon. This coexistence, on the other hand, introduces a novel pathogenetic aspect of PXE and an important research challenge. © 2002 by The American Society of Hematology.   CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: K Orhan, C Delilbasi, and C. Paksoy Magnetic resonance imaging evaluation of mandibular condyle bone marrow and temporomandibular joint disc signal intensity in anaemia patients Dentomaxillofac. Radiol., July 1, 2009; 38(5): 247 - 254. [Abstract] [Full Text] [PDF] L. Martin, F. Maitre, P. Bonicel, P. Daudon, C. Verny, D. Bonneau, O. Le Saux, and N. Chassaing Heterozygosity for a Single Mutation in the ABCC6 Gene May Closely Mimic PXE: Consequences of This Phenotype Overlap for the Definition of PXE Arch Dermatol, March 1, 2008; 144(3): 301 - 306. [Abstract] [Full Text] [PDF] A. Aessopos, M. Kati, and D. Farmakis Heart disease in thalassemia intermedia: a review of the underlying pathophysiology Haematologica, May 1, 2007; 92(5): 658 - 665. [Abstract] [Full Text] [PDF] D. Farmakis, A. Polonifi, S. Deftereos, M. Tsironi, I. Papaioannou, and A. Aessopos Aortic Valve Replacement in a Patient With Thalassemia Intermedia Ann. Thorac. Surg., February 1, 2006; 81(2): 737 - 739. [Abstract] [Full Text] [PDF] N Chassaing, L Martin, P Calvas, M Le Bert, and A Hovnanian Pseudoxanthoma elasticum: a clinical, pathophysiological and genetic update including 11 novel ABCC6 mutations J. Med. Genet., December 1, 2005; 42(12): 881 - 892. [Abstract] [Full Text] [PDF] S Laube and C Moss Pseudoxanthoma elasticum Arch. Dis. Child., July 1, 2005; 90(7): 754 - 756. [Abstract] [Full Text] [PDF] T. Aranyi, M. Ratajewski, V. Bardoczy, L. Pulaski, A. Bors, A. Tordai, and A. Varadi Identification of a DNA Methylation-dependent Activator Sequence in the Pseudoxanthoma Elasticum Gene, ABCC6 J. Biol. Chem., May 13, 2005; 280(19): 18643 - 18650. [Abstract] [Full Text] [PDF] A. Aessopos, D. Farmakis, S. Deftereos, M. Tsironi, S. Tassiopoulos, I. Moyssakis, and M. Karagiorga Thalassemia Heart Disease: A Comparative Evaluation of Thalassemia Major and Thalassemia Intermedia Chest, May 1, 2005; 127(5): 1523 - 1530. [Abstract] [Full Text] [PDF] S. Bunda, N. Kaviani, and A. Hinek Fluctuations of Intracellular Iron Modulate Elastin Production J. Biol. Chem., January 21, 2005; 280(3): 2341 - 2351. [Abstract] [Full Text] [PDF] M. D. Trip, Y. M. Smulders, J. J. Wegman, X. Hu, J. M.A. Boer, J. B. ten Brink, A. H. Zwinderman, J. J.P. Kastelein, E. J.M. Feskens, and A. A.B. Bergen Frequent Mutation in the ABCC6 Gene (R1141X) Is Associated With a Strong Increase in the Prevalence of Coronary Artery Disease Circulation, August 13, 2002; 106(7): 773 - 775. [Abstract] [Full Text] [PDF]

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