SEWA Rural, Jhagadia, Gujarat. Correspondence to: Dr Kapilkumar Dave, Research Associate, SEWA Rural, Jhagadia, Gujarat.
SEWA Rural, Jhagadia, Gujarat.
Indian Pediatr. 2022 Mar 15;59(3):230-233. Epub 2022 Jan 9.
To present the result of newborn sickle cell disease (SCD) screening and clinical profile of SCD newborns in a tribal area of Gujarat.
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.
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.
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.
展示古吉拉特邦一个部落地区新生儿镰状细胞病（SCD）筛查的结果和 SCD 新生儿的临床特征。
我们在 2014 年至 2019 年期间，在古吉拉特邦一个部落地区的二级保健医院，使用高效液相色谱法（HPLC）在新生儿出生后两天内对所有镰状细胞特征（SCT）和 SCD 母亲的新生儿进行 SCD 筛查。患有 SCD 的新生儿在基于信息技术的医院综合护理平台上进行登记。对新生儿进行前瞻性随访，每 3 个月一次。如果他们错过诊所就诊，医疗顾问会上门家访收集所需信息。
在筛查的 2492 名新生儿中，有 87 名（3.5%）被诊断为 SCD。在对 67 名筛查α-地中海贫血缺失的新生儿中，有 64 名（95.4%）婴儿有α-地中海贫血缺失。在此期间，共进行了 554 次门诊就诊，随访期为 221.5 人年。急性发热性疾病、疼痛危象、住院和严重贫血的发生率分别为每 100 人年 42.9、14.9、14.9 和 4.5。记录了 2 例死亡，5 例婴儿（5.7%）患有严重 SCD。
我们发现古吉拉特邦部落地区新生儿 SCD 队列中α 地中海贫血缺失的发生率很高，70%的婴儿在随访中有至少一种临床并发症。
