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MVD是以外分泌器官及组织为主的全身性遗传性疾病。由于呼吸道粘液电解质成分变化,糖蛋白增多,粘膜腺体病变,致使分泌液的流变学指标有改变,纤毛周液层脱水,分泌物变粘稠并积聚,粘液纤毛传输功能低下,腺体分泌功能紊乱。基于以上病理生理改变,故患者中有耳鼻喉疾病者明显高于一般人群。最常见病变是鼻窦炎,可高达92%,次为鼻息肉,占6～15%,有人对450例回顾性分析中,11%患有鼻窦炎,10%有鼻息肉,8%有急或慢性中耳炎;Fritze等发现1/3患者听力有改变,与
MVD is a systemic disease characterized by exocrine organs and tissues. As the respiratory mucus electrolyte composition changes, increased glycoprotein, mucosal gland lesions, resulting in changes in fluid rheology indicators, cilia, liquid layer of dehydration, thickening and accumulation of secretions, mucus ciliary transfer dysfunction, secretion of glands Dysfunction. Based on the above pathophysiological changes, so patients with ENT diseases were significantly higher than the general population. The most common disease is sinusitis, up to 92%, followed by nasal polyps, accounting for 6 to 15%, some 450 cases of retrospective analysis, 11% suffering from sinusitis, 10% of nasal polyps, 8% of urgent or Chronic otitis media; Fritze found that 1/3 of patients with hearing changes, and