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Titolo:
Exciting new treatment approaches for pathophysiologic mechanisms of sickle cell disease
Autore:
Mankad, VN;
Indirizzi:
Univ Kentucky, Coll Med, Dept Pediat, Lexington, KY 40536 USA Univ Kentucky Lexington KY USA 40536 Dept Pediat, Lexington, KY 40536 USA
Titolo Testata:
PEDIATRIC PATHOLOGY & MOLECULAR MEDICINE
fascicolo: 1, volume: 20, anno: 2001,
pagine: 1 - 13
SICI:
1522-7952(200101)20:1<1:ENTAFP>2.0.ZU;2-5
Fonte:
ISI
Lingua:
ENG
Soggetto:
FETAL HEMOGLOBIN PRODUCTION; HYDROXYUREA THERAPY; IN-VIVO; ERYTHROCYTE DEHYDRATION; OXYGEN-AFFINITY; ANEMIA; CHILDREN; TRANSPORT; TRIAL; STIMULATION;
Keywords:
bone marrow transplantation; butyrate; clotrimazole; gene therapy; hydroxyurea; nitric oxide; poloxamer 188; sickle cell anemia disease;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
50
Recensione:
Indirizzi per estratti:
Indirizzo: Mankad, VN Univ Kentucky, Coll Med, Dept Pediat, Lexington, KY 40536 USA Univ Kentucky Lexington KY USA 40536 , Lexington, KY 40536 USA
Citazione:
V.N. Mankad, "Exciting new treatment approaches for pathophysiologic mechanisms of sickle cell disease", PEDIAT PATH, 20(1), 2001, pp. 1-13

Abstract

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion During associated with sick le cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vasoocclusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sick le cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for young er children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects ofmutant genes.

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Documento generato il 04/04/20 alle ore 08:39:12