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Sickle cell disease in central India
Archana B Patel1, Ambarish M Athavale
1Department of Pediatrics & Clinical Epidemiology Unit, Indira Gandhi Medical College, Nagpur, India. archana@giasbm01.vsnl.net.in
Insights
In India, malnutrition and non-compliance with penicillin prophylaxis significantly increase the risk of sickle cell disease (SCD) complications and infections in children. These factors heighten susceptibility to pain crises and other severe health issues.
Area of Science:
- Pediatrics
- Hematology
- Infectious Diseases
Background:
- Sickle Cell Disease (SCD) management in India lacks comprehensive data on disease incidence and risk factors, particularly concerning vaccination and prophylaxis.
- Understanding these factors is crucial for improving patient outcomes and developing targeted interventions.
Purpose of the Study:
- To determine the incidence and risk factors for sickle cell crises (SCC) and infections in Indian children with SCD.
- To evaluate the impact of pneumococcal vaccination and penicillin prophylaxis on disease outcomes.
Main Methods:
- A prospective hospital-based study followed 325 children with SCD on penicillin prophylaxis for over 146 person-years.
- 161 children received pneumococcal vaccination; data on crises, infections, and risk factors were collected.
Main Results:
- The incidence of SCC was 1.25 per patient-year, and infections occurred at 1.38 per person-year.
- Key risk factors for both SCC and infections included protein energy malnutrition (PEM), Mahar caste, and non-compliance with medication.
Conclusions:
- Malnutrition and non-compliance with medication are significant risk factors that increase susceptibility to SCC and infections in children with SCD.
- These findings highlight the need for improved nutritional support and adherence monitoring in SCD management in India.
Objective:
The incidence and the risk factors of sickle cell disease (SCD), vaccinated with Pneumococcal vaccine and on penicillin prophylaxis has not been previously reported in India.
Methods:
This prospective hospital based study followed 325 children on penicillin prophylaxis, of which 161 were vaccinated for pneumococci, over 146.84 person years to determine the incidence and determinants of crisis (SCC) and infections. The average age at presentation was 7.05 +/- 3.26 years with male preponderance below 2 years.
Results:
The main causes for hospitalizations were for blood transfusion, SCC and infections. The incidence of SCC was 1.25 per patient per year and that of infection was 1.38 per person per year. The risk factors for SCC were Mahar caste (p = 0.007) non-compliance (p = 0.000) and protein energy malnutrition (PEM) (p = 0.0015) and for infection were also PEM (p = 0.023), Mahar caste (p = 0.021) and noncompliance (p = 0.001).
Conclusion:
Malnutrition and non-compliance with medication increased the patient's susceptibility to SCC and infections.
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