Manifestações clínicas e diagnóstico laboratorial da doença falciforme : uma revisão sistemática.

Abstract

Sickle cell anemia is a chronic hemolytic disease , the most common hereditary character in Brazil. The objective of this study was to accomplish systematic revision on the clinical complications and types of diagnosis of the bearers of that disease , as well as showing the gr eat importance of neonatal screening . Scientific publications were selected on Medline and Lilacs and SciELO through doPubmed available between the years 2000 and 2014, were included in the analysis sickle cell anemia , clinical aspects related to complica tions , laboratory diagnosis and neonatal screening . The results showed that, of the 29 selected articles , 03 ( 10.3%) were published between 2002 and 2004 , 6 ( 20.7%) between 2005 and 2007 , 13 ( 44.8%) between 2008 and 2010 and 7 ( 24.1) between 2011 and 2013. Regarding the concept and definition of sickle cell anemia , all 29 articles approached a little about the subject ; 6 ( 18.2%) detached the epidemiology and its worldwide distribution ; 7 ( 24.1%) covered the clinical manifestations of the disease ; 20 ( 68.9%) include laboratory diagnostics and neonatal screening . The m ost studies happened i n Brazil , the Northeast Region , and the capitals of Fortaleza and Salvador presented the highest prevalence of sickle cell anemia. The most common clinical manifestations we re infections and splenic sequestration . The best method for detection of sickle cell anemia was highly sensitive and reproducible H igh Performance Liquid Chromatography ( HPLC), highly sensitive and reproducible. Sickle cell disease is still associated wit h high morbidity and mortality , and this scenary neonatal screening is an effective tool for early precocious detection and administration of this disease .