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Vol. 115, No. 1-2, 2006
Issue release date: January 2006
Acta Haematol 2006;115:91–96
(DOI:10.1159/000089472)

Diastolic Dysfunction and Pulmonary Hypertension in Sickle Cell Anemia: Is There a Role for L-Carnitine Treatment?

El-Beshlawy A. · Abd El Raouf E. · Mostafa F. · Talaat M. · Isma’eel H. · Aoun E. · Hoffbrand A.V. · Taher A.
aDepartment of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt; bDepartment of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon; cDepartment of Haematology, Royal Free Hospital, London, UK

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Abstract

Clinical manifestations of cardiovascular abnormalities in patients with sickle cell (SC) anemia are well documented. Many variables were assessed in our study before and after administration of L-carnitine to randomly selected 37 SC disease (SCD) children for a period of 6 months. Variables such as weight, height, serum ferritin levels, units of blood transfused and the number of veno-occlusive crises all showed significant improvement after the 6 months of therapy with L-carnitine. Our study also showed that cardiac diastolic function and pulmonary hypertention are common in pediatric SCD patients. These two disorders showed some improvement after L-carnitine administration. Therefore, L-carnitine deserves a rigorous large-scale randomized clinical trial to evaluate its potential benefits as treatment for SCD patients with cardiac complications.



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