Document Detail


Hydroxyurea therapy for diverse pediatric populations with sickle cell disease.
MedLine Citation:
PMID:  9317200     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Hydroxyurea (HU) is the first widely used treatment to have an impact on the severity of disease in adult patients with sickle cell anemia, but limited data are available for younger patients or those with variant genotypes. We reviewed 324 months of experience with HU in 16 patients from 5.3 to 18.4 years of age treated for 6 to 50 months. The major toxicity was reversible neutropenia. Linear growth continued unchanged, and all patients gained weight. Hematologic results were similar to those reported in adults with increases in mean corpuscular volume (MCV) and total and fetal hemoglobin (HbF). We noted that the maximal hematologic effects occurred at less than the maximum dose. Clinically, patients experienced an 80% reduction in episodes of acute chest syndrome and a reduced need for blood transfusion, as well as a 30% decrease in the number of hospitalizations for painful events during HU therapy compared with an equivalent number of months before HU. These highly statistically significant results confirmed the value of HU in ameliorating the severe clinical course of pediatric patients. Similar effects were observed in three patients with sickle beta degrees-thalassemia, sickle beta+-thalassemia, and S-O Arab. Recurrent acute splenic sequestration and progressive symptomatic osteonecrosis were observed during HU. Thus, HU may not prevent the development of complications once organ damage is present. The challenge remains to determine when and to which pediatric patients with sickle cell disease HU should be offered.
Authors:
Z R Rogers
Publication Detail:
Type:  Clinical Trial; Journal Article    
Journal Detail:
Title:  Seminars in hematology     Volume:  34     ISSN:  0037-1963     ISO Abbreviation:  Semin. Hematol.     Publication Date:  1997 Jul 
Date Detail:
Created Date:  1997-10-31     Completed Date:  1997-10-31     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0404514     Medline TA:  Semin Hematol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  42-7     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, The University of Texas Southwestern Medical Center, Children's Medical Center of Dallas, 75235-9063, USA.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Adolescent
Anemia, Sickle Cell / blood,  drug therapy*,  genetics
Blood Transfusion
Child
Child, Preschool
Erythrocyte Indices
Female
Fetal Hemoglobin / metabolism
Genotype
Globins / genetics
Humans
Hydroxyurea / adverse effects,  therapeutic use*
Male
Neutropenia / chemically induced
Weight Gain
Chemical
Reg. No./Substance:
127-07-1/Hydroxyurea; 9004-22-2/Globins; 9034-63-3/Fetal Hemoglobin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Use of hydroxyurea in children with sickle cell disease: what comes next?
Next Document:  ACQUISITION OF MAGNETIC DIRECTIONAL PREFERENCE IN HATCHLING LOGGERHEAD SEA TURTLES