Produção Nacional
The invisibility of sickle cell disease in Brazil: lessons from a study in Maranhão
2014; Elsevier BV; Volume: 37; Issue: 1 Linguagem: Inglês
10.1016/j.bjhh.2014.11.001
ISSN1806-0870
Autores Tópico(s)Prenatal Screening and Diagnostics
ResumoSickle cell disease (SCD) is a genetic disorder in which hemoglobin S (Hb S) predominates in red blood cells.][3] Sickle cell anemia (SCA, Hb SS) is the most common subtype of SCD in the world.Although its clinical course is variable, patients with SCA generally have the most severe phenotype.SCD also includes the heterozygous combination of Hb S with other hemoglobin variants (Hb SC, Hb SD-Punjab, and others).The combination of Hb S with  thalassemia (Hb S/ 0 and Hb S/ + thalassemia) leads to other subtypes of SCD with a variable relative incidence depending on the ethnic composition of the population. 4,5he relative death rate due to hemoglobin disorders in under five-year-old children all over the world is reported to be 3.4% of all deaths. 6Morbidity and mortality are especially high in developing countries. 7[10] There are only two newborn-screening cohort studies in Brazil, which have reported the death rate for children with SCD.In both studies, it was very high compared to figures reported in developed countries.In Minas Gerais, 3 the crude death rate for 1396 children (all subtypes) diagnosed in a seven-year period was 5.6%.The Kaplan-Meier estimated probability of death at five years of age for children with Hb SS or Hb S/ 0 thalassemia was 10.6% (standard error: 1.4).In Rio
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