Pseudoxanthoma elasticum-like skin lesions and angioid streaks in beta-thalassemia
| Autor: | P. Savvides, P. Kaklamanis, Athanasios Aessopos, Markisia Karagiorga, G. Stamatelos, Thomas Tassiopoulos, Phaedon Fessas, Ioannis Rombos |
|---|---|
| Rok vydání: | 1992 |
| Předmět: |
Hemolytic anemia
Adult Male Pathology medicine.medical_specialty Heterozygote Adolescent Thalassemia Connective tissue Skin Diseases Lesion Pathogenesis medicine Prevalence Humans Pseudoxanthoma Elasticum business.industry Homozygote beta-Thalassemia Hematology Middle Aged Pseudoxanthoma elasticum medicine.disease Angioid streaks medicine.anatomical_structure Hemoglobinopathy Hematocrit Ferritins Angioid Streaks Female medicine.symptom business |
| Zdroj: | American journal of hematology. 41(3) |
| ISSN: | 0361-8609 |
| Popis: | One hundred patients with homozygous or doubly heterozygous β-thalassemia (62 with the major form and 38 with β-thalassemia intermedia) were examined for signs of Pseudoxanthoma elasticum (PXE). Diagnostic skin lesions were found in 16 patients with either form of the basic disease. Twenty percent of all patients had angioid streaks (AS); both PXE skin lesions and AS were found in 10% of the patients; in all, 26% had either one or both of these manifestations. A positive correlation was found between the presence of one or both types of lesion and age of the patients (P = 0.032); there were no differences as regards ferritin and hematocrit levels, number of transfused units, chelation therapy, and splenic status between patients with PXE/AS findings and those without. The pathogenesis of these connective tissue manifestations at such a high frequency in β-thalassemia is not clear; the possibilities of it's being acquired or inherited are discussed, the former being considered to be the more economical interpretation. © 1992 Wiley-Liss, Inc. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Plný text