Newborn Screening and Clinical Profile of Children With Sickle Cell Disease in a Tribal Area of Gujarat
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Date
2022-03
Journal Title
Journal ISSN
Volume Title
Publisher
Indian Academy of Pediatrics
Abstract
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.
Description
Keywords
?-Thalassemia deletion, Follow-up, Mortality, Outcome.
Citation
Dave Kapilkumar, Desai Shrey, Italia Yazdi, Mukherjee Malay B, Mehta Pallavi, Desai Gayatri. Newborn Screening and Clinical Profile of Children With Sickle Cell Disease in a Tribal Area of Gujarat. Indian Pediatrics. 2022 Mar; 59(3): 230-233