domingo, 15 de agosto de 2010

How to prevent mortality in sickle cell anemia kids

Brazillian scientists have found high mortality in children with sickle cell anemia, a frequent cause of inherited anemia in Brazil.

Abstract:

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 to 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 Sβ+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 Sβ+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.

J Pediatr (Rio J). 2010;86(4):279-284:
Anemia, sickle cell, mortality, socioeconomic factors, health services.

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