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Clinical events in a large prospective cohort of children with sickle cell disease in Nagpur, India: evidence against a milder clinical phenotype in India
Author(s) -
Jain Dipty,
Arjunan Aishwarya,
Sarathi Vijaya,
Jain Harshwardhan,
Bhandarwar Amol,
Vuga Marike,
Krishnamurti Lakshmanan
Publication year - 2016
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.26085
Subject(s) - medicine , acute chest syndrome , pediatrics , sickle cell anemia , anemia , disease , cohort , meningitis , stroke (engine) , clinical phenotype , gastroenterology , phenotype , gene , mechanical engineering , biochemistry , chemistry , engineering
Background The clinical phenotype of sickle cell disease (SCD) has been reported to be milder in India than in the United States. The objective of this large single‐center study was to examine the rate of complications to define the phenotype of SCD in India. Methods The rate of complications per 100 person‐years in 833 pediatric SCD patients for 1954 person‐years in Nagpur, India including those diagnosed on newborn screen (NBS) and those presenting later in childhood (non‐NBS) was compared to those reported in the cooperative study of sickle cell disease (CSSCD). Event rates were also compared between patients belonging to scheduled castes (SCs), scheduled tribes (STs), and other backward classes (OBC). Results Comparison of CSSCD versus Nagpur NBS versus Nagpur non‐NBS for rates of pain (32.4 vs. 85.2 vs. 62.4), severe anemia (7.1 vs. 27 vs. 6.6), stroke (0.7 vs. 0.8 vs. 1.4), splenic sequestration (3.4 vs. 6.7 vs. 1.6), acute chest syndrome (24.5 vs. 23.6 vs. 1.0), and meningitis (0.8 vs. 0 vs. 0.1) revealed more frequent complications in Nagpur compared to CSSCD. Comparison of ST, SC, and OBC for rates of pain (84.6 vs. 71.9 vs. 63.5), acute chest syndrome (3.6 vs. 2.8 vs. 2.2), severe anemia (5.4 vs. 9.5 vs. 11.4), stroke (1.2 vs. 0.4 vs. 0.3), splenic sequestration (0.6 vs. 2.4 vs. 1.9), and meningitis (0.8 vs. 0 vs. 0.1) revealed significantly more frequent complications among ST. Conclusions SCD‐related complications are more frequent in Indian children than that observed in CSSCD. Further study is indicated to define SCD phenotype in India.