[Long-term effects of combined therapy in patients with beta-thalassemia major].

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  • Additional Information
    • Transliterated Title:
      Effetti a distanza della terapia combinata in soggetti affetti da beta-thalassemia major.
    • Source:
      Publisher: Pediatria Medica E Chirurgica Country of Publication: Italy NLM ID: 8100625 Publication Model: Print Cited Medium: Print ISSN: 0391-5387 (Print) Linking ISSN: 03915387 NLM ISO Abbreviation: Pediatr Med Chir Subsets: MEDLINE
    • Publication Information:
      Publication: Vicenza : Pediatria Medica E Chirurgica
      Original Publication: Vicenza, Italia : [s.n.]
    • Subject Terms:
      beta-Thalassemia/*therapy; Bone Marrow Transplantation ; Child ; Child, Preschool ; Dose-Response Relationship, Drug ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Hypogonadism/etiology ; Infant ; Iron Chelating Agents/administration & dosage ; Male ; Splenectomy ; beta-Thalassemia/diagnosis
    • Abstract:
      We evaluated therapy complications in 19 beta-thalassemia major patients (mean age from 3 years/5 months and 1 years/6 months) who were followed at II Pediatric Department-University of Bari. 3 out of 19 patients underwent allogenic BMT from matched related donor; 2 out of 19 underwent splenectomy. All of them were receiving hypertransfusion therapy and continuous chelation with DFO. In all patients we performed physical examination, laboratory assays, cardiac and endocrinologic function tests, serum HBV-HCV-HIV antibodies, otoscopy and audiometric test, fundus oculi, skeletal x-ray. 1 out of 19 patients, who was under 15, had a slight dilatation of left ventricle and arythmia. All patients were HBsAb positive. 4/19 patients were HCV Ab positive (ELISA test) with an increase in ALT-AST serum levels since at least 6 months. In 3 of them we assessed RIBA test, always positive. 3 of them underwent liver biopsy (1 iron overload 2 chronic active hepatitis). All patients were HIV Ab negative. 4/15 patients revealed low GH levels after Arginina test. 13 pre-pubescent patients had normal results with GNRH test but lower results after FSH test. 1 pubescent patient had gonadotropic hypophyseal deficit. 4 patients had subclinic hypothiroidism. We couldn't find any sequelas in bone-eyes-ears. Hypertransfusion therapy, chelation, profilaxis of infections improved length and quality of life in thalassemic patients. Hypogonadotropic hypogonadism remains a serious sequela and we think it needs to be treated.
    • Accession Number:
      0 (Iron Chelating Agents)
    • Publication Date:
      Date Created: 19980711 Date Completed: 19980812 Latest Revision: 20061115
    • Publication Date:
      20221213
    • Accession Number:
      9658419