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肯尼亚裂谷地区口面部裂隙的分布及一种罕见裂隙变体的频繁出现。

Distribution of orofacial clefts and frequent occurrence of an unusual cleft variant in the Rift Valley of Kenya.

作者信息

Spritz Richard A, Arnold Thomas D, Buonocore Samuel, Carter David, Fingerlin Tasha, Odero Wilson W, Wambani John O, Tenge Robert K, Weatherley-White R Christopher

机构信息

Human Medical Genetics Program, University of Colorado at Denver and Health Sciences Center, Aurora 80045, USA.

出版信息

Cleft Palate Craniofac J. 2007 Jul;44(4):374-7. doi: 10.1597/06-136.1.

Abstract

OBJECTIVE

To investigate the pattern and distribution of nonsyndromic orofacial clefts among patients in the Rift Valley region of northwestern Kenya.

METHODS

Subjects were categorized anatomically for occurrence of an atypical cleft lip variant (ACL), typical cleft lip (CL), cleft lip and palate (CLP), or cleft palate (CP), and family history of orofacial clefts. Tribal ethnicity data were obtained from both cleft and noncleft clinic attendees.

RESULTS

There were 194 patients with CL (52.7%), 153 with CLP (41.6%), and 21 with CP (5.7%). CL constitutes a greater fraction of orofacial clefts in the Rift Valley region than reported elsewhere in Africa, principally due to frequent occurrence of ACL (52.5% of all CL). Among noncleft clinic attendees there was a lower fraction of Bantu and larger fraction of Nilotic-Paranilotic tribal ethnicity than in Kenya overall. In contrast, among patients with orofacial clefts there was significant underrepresentation of Bantu and overrepresentation of Nilotic-Paranilotic tribes, particularly Kalenjin. Patients of Kalenjin origin had a much higher rate of positive family history of orofacial clefts than Bantu patients.

CONCLUSIONS

There is an unusual anatomic distribution of orofacial clefts in the Kenya Rift Valley, with frequent occurrence of an atypical CL variant. Our findings indicate that Bantu tribes have lower risk of orofacial clefts than Nilotic-Paranilotic tribes, possibly due to inherited genetic differences, perhaps accounting for the relatively low prevalence of orofacial clefts through much of Africa.

摘要

目的

调查肯尼亚西北部裂谷地区患者非综合征性口面部裂隙的类型和分布情况。

方法

根据非典型唇裂变异型(ACL)、典型唇裂(CL)、唇腭裂(CLP)或腭裂(CP)的发生情况以及口面部裂隙的家族史,对研究对象进行解剖学分类。从腭裂和非腭裂门诊患者处获取部落种族数据。

结果

有194例唇裂患者(52.7%),153例唇腭裂患者(41.6%),21例腭裂患者(5.7%)。与非洲其他地区报道的情况相比,裂谷地区唇裂在口面部裂隙中所占比例更大,这主要是由于ACL的频繁发生(占所有唇裂的52.5%)。在非腭裂门诊患者中,班图族的比例低于肯尼亚总体水平,尼罗-半尼罗族部落的比例高于肯尼亚总体水平。相比之下,在口面部裂隙患者中,班图族的比例明显偏低,尼罗-半尼罗族部落,尤其是卡伦金族的比例偏高。卡伦金族血统的患者口面部裂隙家族史阳性率远高于班图族患者。

结论

肯尼亚裂谷地区口面部裂隙的解剖分布异常,非典型唇裂变异型频繁出现。我们的研究结果表明,班图族部落患口面部裂隙的风险低于尼罗-半尼罗族部落,这可能是由于遗传差异所致,或许可以解释非洲大部分地区口面部裂隙患病率相对较低的原因。

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