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Sickle cell anemia from central India: a retrospective analysis
Dipty Jain1, Khushnooma Italia, Vijaya Sarathi
1Department of Pediatrics, Government Medical College, Nagpur, India. dipty47@rediffmail.com
Insights
Sickle cell anemia in Indian children often presents severely, contrary to common belief. This study highlights significant healthcare needs, including frequent hospitalizations and transfusions, for these patients.
Area of Science:
- Hematology
- Pediatric Medicine
- Genetics
Background:
- Sickle cell anemia (SCA) in India is often perceived as having a mild clinical course.
- Limited data exists on the severity of SCA in central Indian pediatric populations.
Purpose of the Study:
- To retrospectively investigate the clinical presentation and healthcare utilization of children with SCA in central India.
- To identify the proportion of pediatric SCA patients experiencing severe disease and requiring specific therapies.
Main Methods:
- Retrospective analysis of 316 children diagnosed with SCA.
- Longitudinal follow-up over a mean period of 5.8 years.
- Data collection on blood transfusions, vaso-occlusive crises, hospitalizations, and treatment eligibility.
Main Results:
- A significant proportion (30%) of children presented with severe sickle cell anemia.
- High rates of medical intervention were observed: 55.4 blood transfusions and 43.3 hospitalizations for vaso-occlusive crises per 100 person-years.
- 74 patients met criteria for hydroxyurea therapy, indicating substantial disease burden.
Conclusions:
- Pediatric sickle cell anemia in central India frequently manifests with severe clinical symptoms.
- These findings underscore the need for regular medical monitoring and intervention for affected children in this region.
Abstract:
Although sickle cell anemia in India is believed to have a mild clinical presentation, few studies report severe disease in many patients from central India. Hence, we have retrospectively studied 316 children with SCA who were followed up for a period of 5.8±5.7 years. There were 55.4 blood transfusions, 43.3 episodes of vaso-occlusive crises requiring hospitalization, and 108.9 hospitalizations per 100 person years. Ninety six (30%) patients had severe disease whereas 74 patients also fulfilled the criteria for hydroxyurea therapy. Significant proportion of children with sickle cell anemia from central India present with severe clinical presentation and require regular medical attention.
