Newborn sickle cell screening in a region of Western New York State.
J Pediatr
; 154(1): 121-5, 2009 Jan.
Article
em En
| MEDLINE
| ID: mdl-18804215
ABSTRACT
OBJECTIVES:
To assess local trends in the incidence of sickle cell disease (SCD) and hemoglobin (Hb) S trait. Hemoglobinopathy clinic follow-up and cohort mortality rates were also evaluated. STUDYDESIGN:
A longstanding newborn hemoglobinopathy screening program was reviewed. Incidence rates were computed with information from a confidential database, specialty clinic/hospital data, and local birth statistics.RESULTS:
Over 27 years, the incidence of Hb SS in live black births was 0.163% or 1 in 615. Over 18 years, the incidence of Hb AS was 8.5% or 1 in 11.8. No significant differences in the incidence of Hb SS, Hb AS, and the S allele were found over time. Specialty clinic follow-up improved. Death before age 18 years was documented for 6 SCD cases (2.8%; mortality rate of 0.23 per 100 patient years).CONCLUSIONS:
Local screening activities may have had an impact on participation in specialized SCD care and the disease-associated mortality rate. The incidence of Hb SS has remained unchanged over 27 years, and that of Hb S trait and the S allele has been unaffected in the last 18 years. Trait notification goals and approaches should be reevaluated.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Tipo de estudo:
Diagnostic_studies
/
Incidence_studies
/
Prognostic_studies
/
Screening_studies
Limite:
Humans
/
Newborn
País/Região como assunto:
America do norte
Idioma:
En
Ano de publicação:
2009
Tipo de documento:
Article