title: Diastolic dysfunction and pulmonary hypertension in sickle cell anemia: is there a role for L-carnitine treatment?
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 hypertension 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.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt. amalelbeshlawy@yahoo.com
authors.forename: A
authors.initials: A
authors.lastname: El-Beshlawy
authors.forename: E
authors.initials: E
authors.lastname: Abd El Raouf
authors.forename: F
authors.initials: F
authors.lastname: Mostafa
authors.forename: M
authors.initials: M
authors.lastname: Talaat
authors.forename: H
authors.initials: H
authors.lastname: Isma'eel
authors.forename: E
authors.initials: E
authors.lastname: Aoun
authors.forename: A V
authors.initials: AV
authors.lastname: Hoffbrand
authors.forename: A
authors.initials: A
authors.lastname: Taher
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D001794:Blood Pressure; D001803:Blood Transfusion; D002331:Carnitine / Q000008:administration & dosage*; D002648:Child; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D016032:Randomized Controlled Trials as Topic; D018754:Ventricular Dysfunction / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D014803:Vitamin B Complex / Q000008:administration & dosage*
chemical_list: D014803:Vitamin B Complex; D002331:Carnitine