Clinical events in a large prospective cohort of children with sickle cell disease in Nagpur, India: evidence against a milder clinical phenotype in India
Clinical events in a large prospective cohort of children with sickle cell disease in Nagpur, India: evidence against a milder clinical phenotype in India
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DOI：
10.1002/pbc.26085
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发表时间：
2016-10-01
影响因子：
3.2
通讯作者：
Krishnamurti, Lakshmanan
中科院分区：
文献类型：
--
作者：
Jain, Dipty;Arjunan, Aishwarya;Krishnamurti, Lakshmanan
研究背景印度的镰状细胞病（SCD）的临床表型比美国轻。该大型单中心研究的目的是检查并发症的发生率，以确定印度SCD的表型。MethodsThe rate of complications per 100 person-year in 833 pediatric SCD patients for 1954 person-year in纳格布尔，印度，包括在新生儿筛查（NBS）中诊断的患者和在儿童期后期出现的患者（非NBS）与镰状细胞病合作研究（CSSCD）中报道的结果进行了比较。事件发生率也进行了比较患者属于预定种姓（SC），预定部落（ST），和其他落后类（OBC）。结果比较CSSCD与纳格布尔NBS与纳格布尔非NBS的疼痛率（32.4 vs. 85.2 vs. 62.4），重度贫血（7.1 vs. 27 vs. 6.6），中风（0.7 vs. 0.8 vs. 1.4），脾隔离（3.4 vs. 6.7 vs. 1.6）、急性胸部综合征（24.5 vs. 23.6 vs. 1.0）和脑膜炎（0.8 vs. 0 vs. 0.1）显示，与CSSCD相比，纳格布尔的并发症发生率更高。ST、SC和OBC的疼痛发生率比较（84.6 vs. 71.9 vs. 63.5），急性胸部综合征（3.6 vs. 2.8 vs. 2.2），重度贫血（5.4 vs. 9.5 vs. 11.4），中风（1.2 vs. 0.4 vs. 0.3），脾隔离（0.6 vs. 2.4 vs. 1.9），和脑膜炎（0.8比0比0.1）显示ST. ConclusionsSCD相关的并发症更频繁的并发症在印度儿童比CSSCD观察。进一步的研究表明，以确定SCD表型在印度。
BackgroundThe 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.MethodsThe 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).ResultsComparison 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.ConclusionsSCD-related complications are more frequent in Indian children than that observed in CSSCD. Further study is indicated to define SCD phenotype in India.