Severe Streptococcus pyogenes Infections, United Kingdom, 2003–2004

MMWR. 1998;47:169-172 1 table omitted SICKLE CELL disease (SCD) is an autosomal recessive disorder characterized by production of abnormal (sickle) hemoglobin, resulting inanemia, susceptibility to pneumococcal and other infections, pain, stroke, and multiple organ dysfunctions.ThemostcommontypesincludehemoglobinSS(homozygous)disease,sickle cell–hemoglobin C disease, and the sickle beta-thalassemiasyndromes.1 Arandomized controlled trial published in 1986 indicatedthatdailyoralpenicillinprophylaxis reduced the incidence of serious infection in young children with SCD and led to widespread adoption of newborn screening programs for SCD.2 To study the effectiveness and utilization of prevention programs among large populations of infants with SCD, several newborn screening programs in the United States are now attempting to determine rates of complications and actual use of early medical interventions (e.g., penicillin prophylaxis and pneumococcal vaccination). This report focuses on recent mortality in California, Illinois, and New York. In California and Illinois, mortality fromallcausesamongblackchildrenborn during 1990-1994 with SCD was slightly less than overall mortality for all black children born in the same time period. All newborns in California, Illinois, and New York are screened for hemoglobinopathies. Health departments implemented screening programs in New York in 1975, Illinois in 1989, and California in 1990. For this investigation, SCD was defined as any clinically significant sickle hemoglobinopathy in an infant born during 1990-1994. In California and Illinois, identifying variables from SCD databases were matched with computerized records of state-specific death certificates for 1990-1995. In New York, all SCD-related deaths among children aged ,3 years listed in state vital records for 1990-1994 were matched with the state SCD database. Additional follow-up extending through 1997 was available in California and Illinois: local physicians (i.e., through surveys) and public health nurses informed the respective state health department about the circumstances of SCD-related deaths; such information was not available in New York. Mortality rates per person-year were calculated assuming complete death ascertainment through December 31, 1994, in New York and through December 31, 1995, in California and Illinois. During 1990-1994, a total of 2487 children with presumed or confirmed SCD were identified by the three newborn screening programs. Excluding two deaths of children presumably born in other states, 27 deaths were reported among children with SCD; 20 death certificates provided causes that included SCD or other conditions related to SCD. The median age at death for the 20 infants who had SCD-related deaths was 22 months (range: 2-53 months). Mortality rates for each state were similar. In California and Illinois, where mortality for all causeswasascertained,bytheendof1995 the cumulative mortality rate was 1.5 per 100 black children with SCD born during 1990-1994. The equivalent cumulative mortality rate for all black children born during this period in California and Illinois was 2.0 per 100 black newborns, based on approximate age-coded data in national multiple-cause mortality files.3 Mortality data was available until the third birthday for the subgroup of 768 children with presumed or confirmed hemoglobin SS disease born during 19901991 in New York and during 1990-1992 inCaliforniaandIllinois.Ofthese768children,1.0%diedasaresultofSCD-related causes during the first 3 years of life (0.35 per 100 person-years, based on 2258 person-years[95%confidenceinterval=0.150.70 per 100 person-years]). The rate of compliance with penicillin prophylaxis wasunknown;aninvestigationofriskfactorsisbeingconductedtoanalyzethisand other factors in relation to death and other serious complications. Information about risk factors will be obtained through parental and physician surveys.