Newborn Screening and Clinical Profile of Children With Sickle Cell Disease in a Tribal Area of Gujarat
Newborn Screening and Clinical Profile of Children With Sickle Cell Disease in a Tribal Area of Gujarat
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DOI：
10.1007/s13312-022-2476-7
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发表时间：
2022-03-01
影响因子：
2.3
通讯作者：
Desai, Gayatri
中科院分区：
文献类型：
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作者：
Dave, Kapilkumar;Desai, Shrey;Desai, Gayatri
目的：介绍古吉拉特邦部落地区新生儿镰状细胞病(SCD)筛查结果及新生儿SCD的临床特征。方法：2014-2019年，我们在古吉拉特邦部落地区的一家二级护理医院对所有镰状细胞性状(SCT)新生儿和SCD母亲在出生后两天内进行了SCD筛查。在基于信息技术的医院综合护理平台下登记患有SCD的新生儿。每3个月对新生儿进行前瞻性随访。如果他们错过了诊所的访问，一名医疗顾问会在家中拜访他们，以收集所需的信息。结果：在2492名新生儿中，87名(3.5%)被诊断为SCD。在筛查出的67例α-地中海贫血新生儿中，(95.4%)的新生儿有α-地中海贫血缺失。在221.5人年期间，我们记录了总共554次诊所就诊。急性发热病、疼痛危象、住院率和重度贫血率分别为42.9、14.9、14.9和4.5/百人年。有2人死亡，5名婴儿(5.7%)患有严重的SCD。结论：我们在古吉拉特邦部落地区的新生儿SCD队列中发现了很高的α地中海贫血缺失率，70%的婴儿在随访中至少有一种临床并发症。
Objectives: To present the result of newborn sickle cell disease (SCD) screening and clinical profile of SCD newborns in a tribal area of Gujarat. Methods: We screened all newborns of sickle cell trait (SCT) and SCD mothers for SCD using high-performance liquid chromatography (HPLC) within two days of birth at a secondary care hospital in a tribal area in Gujarat from 2014 to 2019. Newborns with SCD were registered under an information technology based platform for hospital-based comprehensive care. Neonates were followed prospectively every 3 months. If they missed the clinic visit, a medical counsellor visited them at home to collect the required information. Results: Out of 2492 newborns screened, 87 (3.5%) were diagnosed with SCD. Among the 67 newborns screened for alpha-thalassemia deletion, 64 (95.4%) of babies had alpha-thalassemia deletion. We recorded total 554 clinic visits over the period of 221.5 person-years. The rates of acute febrile illness, painful crisis, hospitalization and severe anemia were 42.9, 14.9, 14.9 and 4.5 per 100 person-year, respectively. Two deaths were recorded, and 5 babies (5.7%) had severe SCD. Conclusion: We found a high prevalence of alpha thalassemia deletion among newborn SCD cohort in tribal area of Gujarat, and 70% babies had atleast one clinical complication on follow-up.