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姆塞莱尼的髋关节疾病

Hip disease of Mseleni.

作者信息

Du Toit G T

出版信息

Clin Orthop Relat Res. 1979 Jun(141):223-36.

PMID:477111
Abstract

Mseleni in Northern Zululand is a remote geographically isolated improverished district in which about 2,000 persons suffer from a crippling joint disease similar to dysplasia epiphysealis multiplex and spondyloepiphyseal dysplasia. Eleven per cent of the men and 39% of the women in young adult life are seriously disabled and, with concurrent osteoarthritis, 90% of the population are grossly incapacitated in old age. Affected and unaffected control areas have been compared. Analysis of the climate, dietary factors, social taboos and soil and water does not implicate these as causes. Genetic studies, despite considerable field work, sociological surveys and laboratory investigations have not provided the answer. Despite a high incidence in the Mseleni area no disturbance of blood group percentages could be determined. The disease had dissimilarities from Kashin Beck disease in which fusarium fungus allegedly causes small joint polyarthritis. Fusarium sporotrichiella has not been grown in subtropical Zululand and thus could not be a causative agent. Mseleni disease also differs from Handigodu disease in Karnataka India, in which the hips are also destroyed in 80% of cases in the acute form of this disease which is brought on by environmental factors related to subtropical climate, low animal protein intake and intake of a variety of possibly toxic products of vegetable origin. Investigation of stored groundnuts in the affected districts has not revealed a fungus toxin in food which can as yet be implicated. A multifactorial genetic and environmental cause appears probable.

摘要

祖鲁兰北部的姆塞莱尼是一个地理位置偏远、与世隔绝的贫困地区,约有2000人患有一种致残性关节疾病,类似于多发性骨骺发育异常和脊椎骨骺发育不良。在青壮年时期,11%的男性和39%的女性严重残疾,加上并发骨关节炎,90%的人口在老年时严重丧失劳动能力。已对患病地区和未患病的对照地区进行了比较。对气候、饮食因素、社会禁忌以及土壤和水的分析并未表明这些是病因。尽管进行了大量的实地调查、社会学调查和实验室研究,基因研究仍未给出答案。尽管姆塞莱尼地区发病率很高,但血型百分比并未出现异常。该疾病与 Kashin Beck 病不同,据称 Kashin Beck 病是由镰刀菌引起的小关节多关节炎。在亚热带的祖鲁兰地区尚未培养出镰刀菌,因此它不可能是致病因素。姆塞莱尼病也与印度卡纳塔克邦的汉迪戈杜病不同,在汉迪戈杜病中,80%的急性病例的髋关节会受到破坏,这种疾病是由与亚热带气候、低动物蛋白摄入以及多种可能有毒的植物源性产品摄入相关的环境因素引起的。对受灾地区储存的花生进行调查,尚未发现可能与之相关的食物中的真菌毒素。病因可能是多因素的,涉及遗传和环境因素。

相似文献

1
Hip disease of Mseleni.姆塞莱尼的髋关节疾病
Clin Orthop Relat Res. 1979 Jun(141):223-36.
2
Brachydactylous dwarfs of Mseleni.姆塞莱尼的短指侏儒症患者。
Am J Med Genet. 1993 Jul 1;46(6):636-40. doi: 10.1002/ajmg.1320460607.
3
Mseleni joint disease: a radiological study of two affected families.
S Afr Med J. 1973 Dec 15;47(49):2366-76.
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Distinct types of hip disorder in Mseleni joint disease.姆塞莱尼关节病中不同类型的髋关节疾病。
S Afr Med J. 1986 Jan 4;69(1):15-7.
5
[The radiological picture of gout].
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6
Mycotoxicological investigations on maize and groundnuts from the endemic area of Mseleni joint disease in Kwazulu.夸祖鲁省姆塞莱尼关节病流行地区玉米和花生的霉菌毒素毒理学调查。
S Afr Med J. 1986 Mar 15;69(6):369-74.
7
Mseleni joint disease: the pilot clinical survey.姆塞莱尼关节病:初步临床调查
S Afr Med J. 1973 Dec 1;47(47):2283-93.
8
Hemochromatosis arthropathy.血色素沉着症性关节病
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9
Roentgen findings in the XXXXY chromosome anomaly.XXXXY染色体异常的X线检查结果。
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Mseleni joint disease: a potential model of epigenetic chondrodysplasia.梅斯利尼关节病:一种潜在的表观遗传学性软骨发育不良模型。
Joint Bone Spine. 2010 Oct;77(5):399-404. doi: 10.1016/j.jbspin.2010.01.013. Epub 2010 May 14.

引用本文的文献

1
The fate of the hip in spondylo-epi-metaphyseal dysplasia: clinical and radiological evaluation of adults with SEMD Handigodu type.脊柱干骺端-骨骺发育不良性髋部病变的转归:HANDIGODU 型 SEMD 成人的临床和影像学评估。
Skeletal Radiol. 2012 Aug;41(8):939-45. doi: 10.1007/s00256-011-1327-8. Epub 2011 Nov 25.