Mortality of children with sickle cell disease: a population study.
OBJECTIVE
To describe the deaths of children with sickle cell disease (SCD) in Minas Gerais, Brazil, and followed up at the Fundação Hemominas.


METHODS
Cohort of children diagnosed by the Neonatal Screening Program in Minas Gerais (March/1998 - February/2005). Deaths were identified by searching for children who did not attend scheduled consultations at hemocenters. Clinical and epidemiological data were abstracted from death certificates, the newborn screening database, individual medical records, and from interviews with families.


RESULTS
During the period, 1,833,030 newborns were screened; 1,396 had SCD (1:1,300). There were 78 deaths: 63 with SS genotype, 12 with SC genotype, and three with Sbeta+thalassemia genotype. Fifty-six children (71.8%) died before 2 years of age; 59 died in hospitals and 18 at home or during transportation. Causes of death according to certificates (n = 78): infections, 38.5%; acute splenic sequestration, 16.6%; other causes, 9%; did not receive medical care, 15.4%; and not identified on certificates, 20.5%. According to interviews (n = 52) acute splenic sequestration was responsible for one third of deaths, in contrast with 14% recorded on death certificates. Survival probabilities at 5y (SEM) for children with SS, SC, and Sbeta+thalassemia were 89.4 (1.4), 97.7 (0.7), and 94.7% (3.0), respectively (SS vs. SC, p < 0.0001).


CONCLUSIONS
Even with a carefully controlled newborn screening program, the probability of SS children dying was still found to be high. Causes not identified on death certificates may indicate difficulties recognizing SCD and its complications. Educational campaigns directed at health professionals and SCD patients' families should be boosted in order to decrease SCD mortality.
Fernandes
Ana Paula Pinheiro Chagas
AP
Núcleo de Ações e Pesquisa em Apoio Diagnóstico, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
Januário
José Nélio
JN
Cangussu
Camila Blanco
CB
Macedo
Daniela Lino de
DL
Viana
Marcos Borato
MB
D000755:Anemia, Sickle Cell / Q000401:mortality*; D001938:Brazil / Q000453:epidemiology; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D015997:Neonatal Screening / Q000706:statistics & numerical data; D015996:Survival Rate