The Congenital Malformations Registry, Bureau of Environmental and Occupational Epidemiology, Center for Environmental Health, New York State Department of Health, Albany, New York, USA.
Genet Med. 2013 Mar;15(3):222-8. doi: 10.1038/gim.2012.128. Epub 2012 Sep 27.
Sickle cell disease is estimated to occur in 1:300-400 African-American births, with higher rates among immigrants from Africa and the Caribbean, and is less common among Hispanic births. This study determined sickle cell disease incidence among New York State newborns stratified by maternal race/ethnicity and nativity.
Newborns with confirmed sickle cell disease born to New York State residents were identified by the New York State newborn screening program for the years 2000-2008 and matched to birth records to obtain birth and maternal information. Annual incidence rates were computed and bivariate analyses were conducted to examine associations with maternal race/ethnicity and nativity.
From 2000 to 2008, 1,911 New York State newborns were diagnosed with sickle cell disease and matched to the birth certificate files. One in every 1,146 live births was diagnosed with sickle cell disease. Newborns of non-Hispanic black mothers accounted for 86% of sickle cell disease cases whereas newborns of Hispanic mothers accounted for 12% of cases. The estimated incidence was 1:230 live births for non-Hispanic black mothers, 1:2,320 births for Hispanic mothers, and 1:41,647 births for non-Hispanic white mothers. Newborns of foreign-born non-Hispanic black mothers had a twofold higher incidence of sickle cell disease than those born to US-born non-Hispanic black mothers (P < 0.001).
This study provides the first US estimates of sickle cell disease incidence by maternal nativity. Women born outside the United States account for the majority of children with sickle cell disease born in New York State. Such findings identify at-risk populations and inform outreach activities that promote ongoing, high-quality medical management to affected children.
镰状细胞病估计每 300-400 例非裔美国人的出生中就有 1 例，非洲和加勒比地区移民中的发病率更高，而西班牙裔出生中的发病率较低。本研究通过按产妇种族/民族和出生地对纽约州新生儿进行分层，确定了镰状细胞病的发病率。
通过纽约州新生儿筛查计划，确定了 2000 年至 2008 年期间出生于纽约州的镰状细胞病确诊新生儿，并与出生记录相匹配以获取出生和产妇信息。计算了每年的发病率，并进行了双变量分析以检查与产妇种族/民族和出生地的关联。
2000 年至 2008 年期间，纽约州有 1911 名新生儿被诊断患有镰状细胞病，并与出生证明文件相匹配。每 1146 例活产中就有 1 例被诊断患有镰状细胞病。非西班牙裔黑人母亲的新生儿占镰状细胞病病例的 86％，而西班牙裔母亲的新生儿占 12％。非西班牙裔黑人母亲的估计发病率为每 230 例活产 1 例，西班牙裔母亲为每 2320 例活产 1 例，非西班牙裔白人母亲为每 41647 例活产 1 例。外国出生的非西班牙裔黑人母亲的新生儿镰状细胞病发病率是非美国出生的非西班牙裔黑人母亲的两倍（P <0.001）。
本研究提供了美国首次按产妇出生地估计镰状细胞病发病率的结果。出生于美国境外的妇女占纽约州出生的镰状细胞病儿童的大多数。这些发现确定了高危人群，并为促进对受影响儿童进行持续、高质量医疗管理的宣传活动提供了信息。
