title: [Clinico-biological and immunohaematological profile of patients with β-thalassemia in Tunisia: about 26 cases].
abstract: OBJECTIVE
  To study the clinical and biological profile of β-thalassemic patients in our region, reflecting the quality of their care.
  
  
  METHODS
  A retrospective study (2010-2011) on 26 β-thalassemic patients followed in the pediatrics service at CHU Farhat Hached Sousse, Tunisia. Epidemiological, clinical and biological data were collected from medical records and transfusion files of patients. The transfusion protocol adopted was to maintain a hemoglobin level>10g/dL by regular transfusions every 3-4 weeks. Iron chelation therapy, in order to maintain serum ferritin<1500ng/mL, was introduced when serum ferritin exceeded 800-1000ng/mL.
  
  
  RESULTS
  The mean age of patients at diagnosis was 15 months. The clinical impact of anemia had resulted in failure to thrive in 54% of patients and facial dysmorphism in 23%. The average transfusion requirement was estimated at 311.02mL/kg/year with 6 cases of hyperconsumption. The immunohaematological monitoring showed the appearance of anti-RBC alloimmunization in one patient and 4 cases of autoimmunization. Poor adherence of chelation therapy was 62% and causing 5 cases of cardiac complications, 4 cases of liver injury and 14 cases of endocrine complications.
  
  
  CONCLUSIONS
  Improving the therapeutic care of β-thalassemic children requires better monitoring of transfusion recovery and improved adherence to chelation therapy.
authors.affiliation: Centre régional de transfusion sanguine, hôpital Farhat-Hached, Sousse, Tunisie. Electronic address: houda.romdhane@gmail.com.
authors.forename: H
authors.initials: H
authors.lastname: Romdhane
authors.affiliation: Centre régional de transfusion sanguine, hôpital Farhat-Hached, Sousse, Tunisie.
authors.forename: H
authors.initials: H
authors.lastname: Amara
authors.affiliation: Centre régional de transfusion sanguine, hôpital Farhat-Hached, Sousse, Tunisie.
authors.forename: S
authors.initials: S
authors.lastname: Abdelkefi
authors.affiliation: Service de pédiatrie clinique, hôpital Farhat-Hached, Sousse, Tunisie.
authors.forename: N
authors.initials: N
authors.lastname: Souyeh
authors.affiliation: Centre régional de transfusion sanguine, hôpital Farhat-Hached, Sousse, Tunisie.
authors.forename: T
authors.initials: T
authors.lastname: Chakroun
authors.affiliation: Centre régional de transfusion sanguine, hôpital Farhat-Hached, Sousse, Tunisie.
authors.forename: I
authors.initials: I
authors.lastname: Jarrey
authors.affiliation: Centre régional de transfusion sanguine, hôpital Farhat-Hached, Sousse, Tunisie.
authors.forename: M
authors.initials: M
authors.lastname: Bouslama
authors.affiliation: Centre régional de transfusion sanguine, hôpital Farhat-Hached, Sousse, Tunisie.
authors.forename: S
authors.initials: S
authors.lastname: Belhedi
authors.affiliation: Centre régional de transfusion sanguine, hôpital Farhat-Hached, Sousse, Tunisie.
authors.forename: B
authors.initials: B
authors.lastname: Houissa
authors.affiliation: Service de pédiatrie clinique, hôpital Farhat-Hached, Sousse, Tunisie.
authors.forename: L
authors.initials: L
authors.lastname: Boughammoura
authors.affiliation: Centre régional de transfusion sanguine, hôpital Farhat-Hached, Sousse, Tunisie.
authors.forename: S
authors.initials: S
authors.lastname: Jemni Yacoub
mesh_terms: D000293:Adolescent; D015551:Autoimmunity; D001803:Blood Transfusion / Q000706:statistics & numerical data; D015913:Chelation Therapy; D002648:Child; D002675:Child, Preschool; D004912:Erythrocytes / Q000276:immunology; D005145:Face / Q000002:abnormalities; D005183:Failure to Thrive / Q000209:etiology; D005260:Female; D005293:Ferritins / Q000097:blood; D006130:Growth Disorders / Q000209:etiology; D006454:Hemoglobins / Q000032:analysis; D006748:Hospital Departments / Q000706:statistics & numerical data; D006785:Hospitals, University / Q000706:statistics & numerical data; D006801:Humans; D007223:Infant; D008297:Male; D010349:Patient Compliance; D010372:Pediatrics; D011787:Quality of Health Care; D012189:Retrospective Studies; D013163:Splenomegaly / Q000209:etiology; D065227:Transfusion Reaction; D014416:Tunisia / Q000453:epidemiology; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications / Q000453:epidemiology* / Q000276:immunology / Q000628:therapy
chemical_list: D006454:Hemoglobins; D005293:Ferritins
keywords: Auto-immunisation/allo-immunisation anti-érythrocytaire; Autoimmunization/alloimmunization of red blood cells; Coverage; Iron overload; Prise en charge; Surcharge en fer; Thalassemia; Transfusion; β-thalassémie