title: Retrospective epidemiological study of Latin American patients with transfusional hemosiderosis: the first Latin American epidemiological study in iron overload--the RELATH study.
abstract: The retrospective epidemiological study of Latin Americans with transfusional hemosiderosis is the first regional patient registry to gather data regarding the burden of transfusional hemosiderosis and patterns of care in these patients. Retrospective and cross-sectional data were collected on patients ≥2 years with selected chronic anemias and minimum 20 transfusions. In the 960 patients analyzed, sickle-cell disease (48·3%) and thalassemias (24·0%) were the most frequent underlying diagnoses. The registry enrolled 355 pediatric patients (187 with sickle-cell disease/94 with thalassemia). Serum ferritin was the most frequent method used to detect iron overload. Complications from transfusional hemosiderosis were reported in ~80% of patients; hepatic (65·3%), endocrine (27·5%), and cardiac (18·2%) being the most frequent. These data indicate that hemoglobinopathies and complications due to transfusional hemosiderosis are a significant clinical problem in the Latin American population with iron overload. Chelation therapy is used insufficiently and has a high rate of discontinuation.
authors: Instituo Estadual de Hematologia Arthur de Siqueira Cavalcanti--HEMORIO, Rua Frei Caneca 8, Rio de Janeiro, Brazil. diretorio@hemorio.rj.gov.br
authors: Clarisse
authors: C
authors: Lobo
authors: Ivan L
authors: IL
authors: Angulo
authors: Lidia R
authors: LR
authors: Aparicio
authors: Guillermo I
authors: GI
authors: Drelichman
authors: Maria A
authors: MA
authors: Zanichelli
authors: Rodolfo
authors: R
authors: Cancado
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015913:Chelation Therapy; D002648:Child; D002675:Child, Preschool; D004700:Endocrine System Diseases / Q000150:complications; D005260:Female; D005293:Ferritins / Q000097:blood; D006331:Heart Diseases / Q000150:complications; D006454:Hemoglobins / Q000378:metabolism; D006486:Hemosiderosis / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology* / Q000209:etiology*; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use; D007843:Latin America / Q000453:epidemiology / Q000208:ethnology; D008107:Liver Diseases / Q000150:complications; D008297:Male; D008875:Middle Aged; D012042:Registries; D012189:Retrospective Studies; D012880:Skin Pigmentation; D065227:Transfusion Reaction; D055815:Young Adult
chemical_list: D006454:Hemoglobins; D007502:Iron Chelating Agents; D005293:Ferritins