目的　探索Alport综合征的发病规律 ,以争取早诊断 ,指导生育 ,阻止患儿出生。方法　采用统计典型家系的方法逐一分析其发病率、肾脏症状及肾外症状发生率 ,疾病严重程序及与性别的关系。结果　部分病例有较明显的遗传规律 ,如显示X 联锁显性遗传 ,病情严重程序男大于女 ,女性最小发病年龄 8个月。其余家系未能明确其遗传规律。结论　对不典型家族史者 ,凡出现肾功能损害及耳聋者 ,应及时筛查 ,可行皮肤组织免疫荧光检查 ,指导诊断 Objective To explore the incidence of Alport syndrome in order to fight for early diagnosis, guide fertility and prevent the birth of children. Methods A typical pedigree method was used to analyze the morbidity, the incidence of renal symptoms and extra-renal symptoms, the serious disease process and the relationship with gender. Results Some cases have obvious genetic law, such as X-linked display dominant inheritance, serious illness male than female, female minimum age of onset of 8 months. The rest of the family failed to clarify its genetic law. Conclusion Atypical family history, those with renal dysfunction and deafness should be promptly screened, viable skin tissue immunofluorescence, to guide the diagnosis