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犬的性别发育障碍——新命名法的采用及已报道病例的重新分类。

Disorders of sex development in the dog-Adoption of a new nomenclature and reclassification of reported cases.

机构信息

Institute of Veterinary Pathology, Ludwig-Maximilians-University, Munich, Germany.

出版信息

Anim Reprod Sci. 2010 Sep;121(3-4):197-207. doi: 10.1016/j.anireprosci.2010.04.011. Epub 2010 Apr 24.

Abstract

Intersexuality is a rare congenital abnormality in domestic animals. It is reported in numerous species including the swine, goat, horse, cat, and dog. The present work provides an overview of the variety of intersexual conditions known in different dog breeds. Each case was reclassified based on the described gonadal constitution, reproductive tract abnormalities and karyogram, and categorised according to the stages normal sex development is undergoing resulting in three main categories: (1) sex chromosome disorders, (2) disorders of gonadal sex development, and (3) disorders of phenotypic sex development. Reclassification disclosed that the current classification scheme and terminology are inconsistently used in literature masking the real occurrence and frequency of various intersex conditions in dogs. For establishment of an individual, precise and definite diagnosis, introduction of a new nomenclature is proposed as recently recommended for humans. The new terminology is based on the gonosomal constellation and gonadal constitution, contributes to a systematic classification of canine intersex cases, and replaces the common but confusing diagnoses "true hermaphrodite" and "pseudohermaphrodite". The literature survey was supplemented by adding the results from own investigations in a German Pinscher and Berger Picard dog with bilateral ovotestes and ambiguous external genitalia. The diagnostic approach and clinical, pathomorphological and cytogenetic findings were described in detail.

摘要

雌雄间性是一种罕见的家畜先天性异常。它在许多物种中都有报道,包括猪、山羊、马、猫和狗。本工作概述了不同犬种中已知的各种雌雄间性情况。每个病例都是根据描述的性腺组成、生殖道异常和核型进行重新分类的,并根据正常性别发育所经历的阶段进行分类,导致三种主要类别:(1)性染色体异常,(2)性腺性别发育障碍,和(3)表型性别发育障碍。重新分类表明,目前的分类方案和术语在文献中使用不一致,掩盖了狗中各种雌雄间性情况的实际发生和频率。为了进行个体化、精确和明确的诊断,建议引入新的命名法,正如最近为人类推荐的那样。新术语基于性染色体组合和性腺组成,有助于对犬雌雄间性病例进行系统分类,并取代常见但令人困惑的诊断“真性两性畸形”和“假性两性畸形”。文献调查通过添加德国牧羊犬和伯杰皮卡犬双侧卵睾和外生殖器模糊的调查结果进行了补充。详细描述了诊断方法以及临床、病理形态学和细胞遗传学发现。

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