tailieunhanh - Pharmacotherapy of Sickle Cell Disease

eradicate extreme poverty and hunger (Mdg 1). Poverty contributes to unintended pregnancies and pregnancy-related mortality and morbidity in adolescent girls and women, and under-nutrition and other nutrition-related factors contribute to 35% of deaths of children under five each year, while also affecting women’s health. Charging people less for health services reduces poverty and makes women and children more willing to seek care. Further efforts at the community level must make nutritional interventions (such as exclusive breastfeeding for six months, use of micronutrient supplements and deworming) a routine part of care. . | 18th Expert Committee on the Selection and Use of Essential Medicines 21 to 25 March 2011 NEW SECTION Adults and Children Pharmacotherapy of Sickle Cell Disease Kathleen A. Neville . and Julie A. Panepinto . 1 Associate Professor of Pediatrics University of Missouri - Kansas City Director Experimental Therapeutics in Pediatric Cancer Program Divisions of Pediatric Clinical Pharmacology and Medical Toxicology and Hematology Oncology Children s Mercy Hospitals and Clinics 2 Associate Professor of Pediatrics Hematology Department of Pediatrics Section of Hematology Oncology Bone Marrow Transplantation Medical College of Wisconsin The Children s Research Institute of the Children s Hospital of Wisconsin Milwaukee WI Summary Sickle cell disease SCD is a potentially devastating condition that is caused by an autosomal recessive inherited hemoglobinopathy which results in the vaso-occlusive phenomena and hemolysis. The severity of the complications that occur with this disorder are widely variable but overall mortality is increased and life expectancy decreased when compared to the general population. Care of patients with sickle cell disease is largely supportive with hydroxyurea representing the only widely used drug which modifies disease pathogenesis. Painful vaso-occlusive events are the most common complication experienced by both children and adults with sickle cell disease and there are few treatment options to prevent the development of these events. Most are managed with traditional supportive care measures . aggressive hydration antiinflammatory and narcotic analgesics that have not changed in decades and which are adequately met by the current World Health Organization WHO Essential Medicines List Table 1 . Table 1. Pharmacotherapeutic agents utilized in the treatment of sickle cell disease which are currently on the Essential Medicines List and the Clinical Use of Blood Handbook. Disease Modifying Agents hydroxycarbamide hydroxyurea .