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加拿大南亚移民中囊性纤维化的患病率及临床特征。

The prevalence and clinical characteristics of cystic fibrosis in South Asian Canadian immigrants.

作者信息

Mei-Zahav M, Durie P, Zielenski J, Solomon M, Tullis E, Tsui L-C, Corey M

机构信息

Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.

出版信息

Arch Dis Child. 2005 Jul;90(7):675-9. doi: 10.1136/adc.2003.042614.

Abstract

BACKGROUND

Cystic fibrosis (CF) is considered to be rare among individuals from the Indian subcontinent. Furthermore, affected individuals are reported to experience a more severe clinical course.

AIMS

It was hypothesised that CF is under diagnosed in people of South Asian origin and therefore the prevalence may be higher than previously estimated.

METHODS

The prevalence of CF in the South Asian and in the general population living in the same geographic region (Metropolitan Toronto) were compared between 1996 and 2001. Population data were obtained from the Canadian census survey. CF phenotype and genotype data were obtained from the Toronto CF database.

RESULTS

Among 381 patients with CF, 15 were of South Asian descent. The age related prevalence of CF among the South Asian and general populations was: 0-14 years, 1:9200 versus 1:6600; 15-24 years, 1:13,200 versus 1:7600; older than 25 years, 1:56,600 versus 1:12,400. Age at diagnosis, duration and severity of symptoms at diagnosis, current nutritional status, and FEV(1) were similar in the two groups. While not significant, FEV1 tended to be lower (48% versus 57% predicted) among adult South Asians, compared to the general CF population. Also, the percentage with pancreatic sufficiency was higher (27% versus 16%) and the frequency of DeltaF508 allele was lower (50% versus 65.1%).

CONCLUSIONS

These data suggest that the prevalence and natural history of CF in South Asians is similar to that among individuals of European origin. The relatively lower prevalence among older South Asians may reflect an improving recognition of CF in this ethnic subgroup.

摘要

背景

囊性纤维化(CF)在印度次大陆人群中被认为较为罕见。此外,据报道,患病个体的临床病程更为严重。

目的

据推测,南亚裔人群中CF的诊断率较低,因此其患病率可能高于先前的估计。

方法

比较了1996年至2001年间居住在同一地理区域(大多伦多)的南亚人群和普通人群中CF的患病率。人口数据来自加拿大人口普查。CF的表型和基因型数据来自多伦多CF数据库。

结果

在381例CF患者中,15例为南亚裔。南亚人群和普通人群中CF的年龄相关患病率为:0至14岁,1:9200对1:6600;15至24岁,1:13200对1:7600;25岁以上,1:56600对1:12400。两组的诊断年龄、诊断时症状的持续时间和严重程度、当前营养状况以及第一秒用力呼气容积(FEV₁)相似。虽然差异不显著,但与普通CF人群相比,成年南亚人的FEV₁往往较低(预计值分别为48%和57%)。此外,胰腺功能充足者的比例较高(27%对16%),而ΔF508等位基因的频率较低(50%对65.1%)。

结论

这些数据表明,南亚人群中CF的患病率和自然病史与欧洲裔个体相似。老年南亚人患病率相对较低可能反映出该种族亚组对CF的认识有所提高。

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