What are the optimal followup and management of infants identified with hemoglobinopathies (disease and carriers)?
Although the technology to screen infants in the newborn period has been available for the past 15 to 20 years, screening has not received widespread acceptance largely because of the perception that, without effective treatment, early diagnosis would not decrease morbidity and mortality. There is now indisputable evidence that rates of morbidity and mortality can be significantly reduced by programs that screen newborns for sickle cell disease, if they are linked to comprehensive clinical management systems that include parental education. A recent multicenter randomized trial of oral penicillin prophylaxis in children with sickle cell disease showed an impressive 85 percent reduction in the incidence of infection in the group treated with oral penicillin as compared with the group given placebo. The 13 septic episodes among the 110 patients in the placebo group resulted in 3 deaths compared with no deaths and only 2 septic episodes among the 105 patients treated with prophylactic pen
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