University of Rochester Medical Center, Rochester, New York.
Shirati KMT Hospital, Shirati, Tanzania.
Pediatr Blood Cancer. 2020 Nov;67(11):e28620. doi: 10.1002/pbc.28620. Epub 2020 Aug 16.
BACKGROUND/OBJECTIVES: Sickle cell disease (SCD) is an important, hidden cause of childhood mortality worldwide. It is most prevalent in sub-Saharan Africa where national newborn screening programs remain unavailable and most children in rural areas are never diagnosed. We conducted a study at a rural district hospital in northern Tanzania to determine the birth prevalence and community awareness of SCD and to determine the feasibility of using point-of-care testing to enroll newborns in a new SCD clinic for ongoing treatment.
DESIGN/METHODS: We screened infants at Shirati KMT hospital for SCD using HemoTypeSC, an inexpensive point-of-care test. Infants who screened positive were enrolled in the SCD clinic and instructed to return at 6-12 weeks for confirmatory testing, counseling, and preventive care.
A total of 999 newborns were screened from February to September 2019. Among these, 31.6% (315/999) had sickle cell trait and 3.9% (39/999) had SCD. No hemoglobin C was detected. Very few parents knew their own sickle cell status (0.3%). At 5 months after completion, 12 infants from the screening study and 30 additional children had been seen at the SCD clinic for ongoing counseling and care.
Birth prevalence of SCD in rural Tanzania is extremely high and community awareness is low. Newborn point-of-care testing enhances case finding and enables early enrollment in preventive care for SCD, even in rural sub-Saharan Africa with minimal laboratory capacity. SCD-specific clinical services implemented at the district hospital level could expand access to many children and significantly reduce early SCD morbidity and mortality.
背景/目的:镰状细胞病(SCD)是全球儿童死亡的一个重要但被忽视的原因。该病在撒哈拉以南非洲最为普遍,但那里的国家新生儿筛查计划仍然无法实施,大多数农村地区的儿童从未被诊断出患有该病。我们在坦桑尼亚北部的一家农村地区医院进行了一项研究,以确定 SCD 的出生流行率和社区意识,并确定使用即时检测在新的 SCD 诊所招募新生儿进行持续治疗的可行性。
方法/设计:我们使用廉价的即时检测方法 HemoTypeSC 在 Shirati KMT 医院对婴儿进行 SCD 筛查。筛查阳性的婴儿被纳入 SCD 诊所,并被指示在 6-12 周时返回进行确认性检测、咨询和预防保健。
2019 年 2 月至 9 月,共对 999 名新生儿进行了筛查。其中,31.6%(315/999)为镰状细胞特征,3.9%(39/999)为 SCD。未检测到血红蛋白 C。很少有父母知道自己的镰状细胞状况(0.3%)。在完成筛查研究后的 5 个月内,有 12 名来自筛查研究的婴儿和另外 30 名儿童在 SCD 诊所接受了持续的咨询和护理。
坦桑尼亚农村地区 SCD 的出生流行率极高,社区意识很低。新生儿即时检测可增强病例发现,并使 SCD 患者能够及早接受预防保健,即使在实验室能力有限的撒哈拉以南非洲农村地区也是如此。在地区医院层面实施的 SCD 特异性临床服务可以扩大许多儿童的获得途径,并显著降低早期 SCD 的发病率和死亡率。