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[Clinico-biological and immunohaematological profile of patients with β-thalassemia in Tunisia: about 26 cases]
- Source :
- Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguine. 21(6)
- Publication Year :
- 2014
-
Abstract
- To study the clinical and biological profile of β-thalassemic patients in our region, reflecting the quality of their care.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 level10g/dL by regular transfusions every 3-4 weeks. Iron chelation therapy, in order to maintain serum ferritin1500ng/mL, was introduced when serum ferritin exceeded 800-1000ng/mL.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.Improving the therapeutic care of β-thalassemic children requires better monitoring of transfusion recovery and improved adherence to chelation therapy.
- Subjects :
- Male
Erythrocytes
Tunisia
Adolescent
Hospital Departments
Autoimmunity
Pediatrics
Hospitals, University
Hemoglobins
Humans
Blood Transfusion
Child
Growth Disorders
Quality of Health Care
Retrospective Studies
beta-Thalassemia
Infant
Transfusion Reaction
Chelation Therapy
Failure to Thrive
Child, Preschool
Face
Ferritins
Splenomegaly
Patient Compliance
Female
Subjects
Details
- Language :
- French
- ISSN :
- 19538022 and 20102011
- Volume :
- 21
- Issue :
- 6
- Database :
- OpenAIRE
- Journal :
- Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguine
- Accession number :
- edsair.pmid..........20c9486082ac24ddc0d7ff8ec941227f