Name: Meirielli Vieira Bruzeguini
Type: MSc dissertation
Publication date: 15/12/2016
Advisor:
Name |
Role |
|---|---|
| Cecília Maria Figueira Silva | Advisor * |
Examining board:
| Name |
Role |
|---|---|
| Cecília Maria Figueira Silva | Advisor * |
| Eliana Zandonade | Internal Alternate * |
| Lorena Barros Furieri | Internal Examiner * |
| Valmin Ramos da Silva | External Examiner * |
Summary: Objective: To analyse the epidemiological of sickle cell (SC) disease mortality in the state of Espirito Santo (ES).
Method: A descriptive epidemiological study of mortality of children diagnosed with SC by the National Neonatal Screening Program of the State of Espírito Santo (NNSP/ES) from October 2001 to December 2013. Clinical and epidemiological data were obtained from Mortality Information System (MIS), NNSP/ES database and Information System on Live Births (ISLB).
Results: 560,348 children have been screened, with 339 positive neonatal screening test for SC (1:1652). Of these, 201 (59,3%) presented genotype HbFS; and 99 (29,2%) HbFSC genotype. 31 deaths were identified resulting from SC and its aggravations, and 54,5% in male children and the predominant age range between 1 and 5 years. In the group of patients sorted there has been 18 deaths, 13 of them the International Classification of Diseases (ICD) of the SC and complications was present in Death Certificate (DC) and absent in 05 children with positive screening of SC. They were observed in patients not screened, 13 deaths containing the ICD for SC and its complications as the underlying cause in DC.
Conclusions: There is fragility of comprehensiveness of NNPS/ES and also difficulty on the part of medical professionals to recognize the SC
and its complications. Should be developed campaigns that reinforce the importance of neonatal screening, identification of patients in emergency care and educational orientation of the relatives to decrease mortality from complications of SC.
