[Arrhythmia in thalassemia major: evaluation of iron chelating therapy by dynamic ECG]. 1993

L Cavallaro, and A Meo, and G Busà, and A Coglitore, and G Sergi, and G Satullo, and A Donato, and M P Calabrò, and M Miceli
Servizio di Cardiologia con UTIC, Ospedale Papardo, Messina.

Arrhythmias and sudden death represent striking features in the natural history of thalassemia major. Antiarrhythmic treatment, however, does not appear to change the clinical course. During recent years the disease's therapeutics approach has undergone a substantial evolution, being more adequate the transfusional regimens as well as more effective the iron chelation therapy through subcutaneous infusion of deferoxamine. The aim of the present study was to determine possible influences exerted by the current treatment upon disease's arrhythmic disorders. Thirty patients of both sexes were enrolled in the study. The age ranged from 9 to 24 years. No congenital or acquired heart diseases were present. Each patient underwent concentrated red cell transfusions (in order to obtain pretransfusional hemoglobin levels of 10-11 g%), and iron-binding therapy through continuous subcutaneous microinjection of deferoxamine 40-50 mg/kg/day (6-8 hours/day, 6 days/week). Patients were divided in 2 groups: the first group (group A) comprising the 16 patients with good therapeutic compliance and regular pharmacological regimen; the second group (Group B) including the remaining poorly compliant 14 patients. The following parameters were analyzed: age, average hemoglobin levels during the last year, total amount of red cell transfusions, ferritin levels, starting age of iron-binding therapy. Moreover, each patient underwent 24-hour ECG Holter monitoring. Age (Group A: 18 +/- 4.6; Group B: 14 +/- 2.7; p < 0.02), total amount of transfusions (Group A: 272 +/- 73; Group B: 211 +/- 44; p < 0.03), and ferritin levels (Group A: 1697 +/- 860; Group B: 2908 +/- 730; p < 0.002) proved to be significantly different in the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)

UI MeSH Term Description Entries
D007502 Iron Chelating Agents Organic chemicals that form two or more coordination links with an iron ion. Once coordination has occurred, the complex formed is called a chelate. The iron-binding porphyrin group of hemoglobin is an example of a metal chelate found in biological systems. Iron Chelates,Agents, Iron Chelating,Chelates, Iron,Chelating Agents, Iron
D008297 Male Males
D001803 Blood Transfusion The introduction of whole blood or blood component directly into the blood stream. (Dorland, 27th ed) Blood Transfusions,Transfusion, Blood,Transfusions, Blood
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003645 Death, Sudden The abrupt cessation of all vital bodily functions, manifested by the permanent loss of total cerebral, respiratory, and cardiovascular functions. Sudden Death
D003676 Deferoxamine Natural product isolated from Streptomyces pilosus. It forms iron complexes and is used as a chelating agent, particularly in the mesylate form. Desferrioxamine,Deferoxamine B,Deferoxamine Mesilate,Deferoxamine Mesylate,Deferoxamine Methanesulfonate,Deferoximine,Deferrioxamine B,Desferal,Desferioximine,Desferrioxamine B,Desferrioxamine B Mesylate,Desferroxamine,Mesilate, Deferoxamine,Mesylate, Deferoxamine,Mesylate, Desferrioxamine B,Methanesulfonate, Deferoxamine
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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