家族性鼻无纤毛综合征非常罕见，鼻粘膜纤毛机能障碍可因纤毛缺失或其超微结构异常所致。该文记述一个家系中4名儿童因纤毛缺如所引起的呼吸系统发病。患儿足月顺产，出生后48小时出现呼吸窘迫，胸片报告右上叶肺不张，氧饱和度84％，氢张力6-65kPa，二氧化碳张力6．00kPa，给 Familial nasal non-cilia syndrome is very rare, nasal mucosal ciliary dysfunction due to ciliary defects or its ultrastructural abnormalities caused. This article describes the respiratory illness in four children in a pedigree due to cilia deficiency. Children with full-term follow-up, 48 hours after birth, respiratory distress, chest radiograph report of right upper lobe atelectasis, oxygen saturation 84%, hydrogen tension 6-65kPa, carbon dioxide tension 6.00kPa, to