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非洲的白化病:耻辱、屠杀和宣传活动。

Albinism in Africa: stigma, slaughter and awareness campaigns.

机构信息

Scripps Mercy Hospital, San Diego, CA 92103, USA.

出版信息

Dermatol Clin. 2011 Jan;29(1):79-87. doi: 10.1016/j.det.2010.08.015.

Abstract

Oculocutaneous albinism is an autosomal recessive disorder characterized by a lack of pigment in the hair, skin, and eyes. Albinism is caused by defective or absent tyrosinase, an enzyme necessary for melanogenesis. Although rare in the western world, albinism is quite common in sub-Saharan Africa, likely as a result of consanguinity. Albinism has long been associated with stigma and superstitions, such as the belief that a white man impregnated the mother or that the child is the ghost of a European colonist. Recently, a notion has emerged that albino body parts are good-luck charms or possess magical powers. These body parts may be sold for as much as $75,000 on the black market. As a result there have been over 100 albino murders in Tanzania, Burundi, and other parts of Africa in the past decade, which is now beginning to garner international attention and thus prompting novel legislation. To ameliorate the plight of individuals with albinism in Africa, a coordinated effort must be organized, involving medical professionals (dermatologists, ophthalmologists, oncologists), public health advocates and educators, social workers, human rights and antidiscrimination activists, law-enforcement agencies, and governmental support groups. The main issues that should be addressed include skin cancer prevention education, stigma and discrimination denouncement, and swift prosecution of albino hunters and their sponsors.

摘要

眼皮肤白化病是一种常染色体隐性遗传疾病,其特征是毛发、皮肤和眼睛中缺乏色素。白化病是由于酪氨酸酶的缺陷或缺失引起的,而酪氨酸酶是黑色素生成所必需的酶。虽然在西方世界很少见,但白化病在撒哈拉以南非洲地区相当常见,这可能是由于近亲繁殖的结果。白化病长期以来一直与耻辱和迷信联系在一起,例如认为一个白人男子使母亲怀孕,或者孩子是欧洲殖民者的鬼魂。最近,出现了一种观念,认为白化病人的身体部位是吉祥物或具有神奇的力量。这些身体部位在黑市上的售价可能高达 75000 美元。因此,在过去十年中,坦桑尼亚、布隆迪和非洲其他地区发生了超过 100 起白化病谋杀案,现在开始引起国际关注,并因此促使出台了新的立法。为了改善非洲白化病患者的困境,必须组织协调一致的努力,涉及医学专业人员(皮肤科医生、眼科医生、肿瘤学家)、公共卫生倡导者和教育工作者、社会工作者、人权和反歧视活动家、执法机构和政府支持团体。应该解决的主要问题包括皮肤癌预防教育、谴责耻辱和歧视,以及迅速起诉白化病猎人及其赞助者。

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