【摘要】 对3284例肾活检中20例(0 6%)Alport综合征(AS)进行回顾性总结。20例AS中男性12例(60%),女性8例(40%);成人15例(75%),儿童5例(25%)。AS电镜下以肾小球毛细血管基底膜(GBM)病变为主,8例(40%)表现为GBM增厚,致密层纵行撕裂形成板层状或编篮状,12例(60%)表现为GBM弥漫变薄和厚薄不均。9例(45%)肾间质中有泡沫细胞浸润。结论:并非所有AS具有典型的GBM增厚,分层或编篮状改变;部分病例GBM分层并不明显;肾间质中泡沫细胞浸润对AS有重要提示作用。本文结合文献对AS的诊断及鉴别诊断进行了探讨。更多还原

【Abstract】 20 cases of Alport syndrome (AS) in 3284 cases renal biopsies were studied retrospectively. In this group of patients, 12 cases were male (60%) and 8 cases were female(40%); 15 cases were aldult (75%) and 5 cases were children (25%). The Ultrastructural characteristic of AS was the changes of glomerular basement membrane (GBM). 8 cases (40%) showed typical changes of the GBM thickening due to reduplication of the lamina densa. The extensive thinning and irregular thickening and thinning of GBM were the major abnormality under electron microscope in 12 cases (60%). 9 cases (45%) showed infiltration of foamy cells in the interstitial. Conclusion:Not all biopsies from AS showed those typical ultrastructural changes. Reduplication of the lamina densa was not obvious in the early stage. The infiltration of foamy cells in the interstitial offered an important suggestion in the diagnosis of AS .The differential diagnosis of AS was also discussed.更多还原