Identification of Structure-Based Antisickling Peptides that Inhibit HBS Polymeri

Children'S Hospital Of Philadelphia, Philadelphia PA

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Abstract

Sickle cell disease (SCD) is the most common serious genetic disease in Africa. In Ghana, 2%[unreadable] of newborns have been found to have homozygous SCD (SCD-SS) or sickle cell disease-SC disease[unreadable] (SCD-SC). In the U.S. and elsewhere outside Africa, bacterial infections, particularly pneumococcal[unreadable] sepsis and meningitis, are the leading cause of death in children with SCD. Childhood mortality[unreadable] from pneumococcal sepsis has been reduced to low levels in the U.S. through early diagnosis in the[unreadable] newborn followed I_ypenicillin prophylaxis, anti-pneumococcal vaccination, and education of[unreadable] parents and health workers about this problem. In Africa, infections are also reported to be the[unreadable] leading cause of death in children with SCD. However, the causes of infection and their relative[unreadable] importance are not precisely determined. Pneumococcal infection is highly prevalent in the general[unreadable] childhood population in Africa, but there are conflicting data on its significance in children with[unreadable] SCD. Penicillin prophylaxis and pneumococcal vaccination are not widely used in young children[unreadable] with SCD in Africa. Host reports list malaria as the leading cause of death in children with SCD in[unreadable] Africa. However, the incidence of acute malaria infection and the risk factors associated with[unreadable] malaria in children with SCD have not been described. Anti-malarial prophylaxis is not practiced[unreadable] uniformly for all children with SCD in Africa.[unreadable] In this study, a cohort of young children with SCD diagnosed from birth will be monitored[unreadable] closely at steady state and during acute illness in order to collect prospective clinical and laboratory[unreadable] data and determine the causes of fever and infection. Parents will be trained to measure[unreadable] temperature and recognize other signs of infection and to bring ill children for evaluation and[unreadable] management. Home visits will be employed to monitor children at home and to reinforce education[unreadable] and preventive care. Blood cultures, malarial smears and hematologic studies will be obtained ,[unreadable] during acute illness. Protocols will be implemented to ensure that prompt management is provided[unreadable] all subjects with potential bacterial or acute malarial infection. Incidence and prevalence of[unreadable] bacterial and malarial infections and their associated risk factors will be determined.