Gaines J J
Department of Pathology, Medical College of Georgia Hospitals & Clinics, Augusta.
Hum Pathol. 1989 Jan;20(1):40-6. doi: 10.1016/0046-8177(89)90200-1.
The gross and microscopic pathology of alkaptonuric ochronosis is presented from a study of pathologic specimens from six cases in our files and from a review of the literature. Emphasis is placed on the most clinically relevant organ systems involved by ochronosis: musculoskeletal, cardiovascular, genitourinary, eye, and skin. Recent electron microscopic discoveries from several affected organs, including the synovium, articular cartilage, cardiovascular system, eye, and skin, are included in this report. In addition, the molecular pathology of alkaptonuria is briefly discussed. The pathologic literature regarding alkaptonurin ochronosis is fragmented, as most cases of this rare entity are reported individually or as small series of cases. A comprehensive review of alkaptonuria has not appeared since the clinicopathologic review of the world literature by O'Brien et al in 1963. The purpose of this report is to present an updated and unified pathologic study of alkaptonuric ochronosis.
通过对我们档案中6例病例的病理标本研究以及文献回顾，介绍了黑尿病性褐黄病的大体和微观病理学。重点关注褐黄病累及的最具临床相关性的器官系统：肌肉骨骼系统、心血管系统、泌尿生殖系统、眼睛和皮肤。本报告纳入了来自几个受累器官（包括滑膜、关节软骨、心血管系统、眼睛和皮肤）的最新电子显微镜发现。此外，还简要讨论了黑尿病的分子病理学。关于黑尿病性褐黄病的病理文献较为零散，因为这种罕见疾病的大多数病例都是单独报告或作为小系列病例报告的。自1963年奥布赖恩等人对世界文献进行临床病理回顾以来，尚未出现对黑尿病的全面综述。本报告的目的是提供一份关于黑尿病性褐黄病的最新统一病理研究。
