Desai M, Weller P H, Spencer D A
Department of Cystic Fibrosis and Respiratory Medicine, Birmingham Childen's Hospital, United Kingdom.
Pediatr Pulmonol. 1995 Nov;20(5):307-8. doi: 10.1002/ppul.1950200509.
Nebulized recombinant human DNase (rhDNase) reduces sputum viscosity, improves pulmonary function, and results in a small reduction in acute respiratory exacerbations requiring intravenous antibiotics in many patients with cystic fibrosis (CF). rhDNase is now recommended for use in CF patients with moderately severe suppurative lung disease. A 14-year-old girl with suppurative lung disease [forced expiratory volume in 1 second (FEV1) 69% and forced vital capacity (FVC) 81% predicted] secondary to Kartagener's syndrome and severe gastroesophageal reflux had worsening spirometry together with intractable gastrointestinal symptoms over the previous 18 months despite conventional treatment. She was, therefore, started on 2.5 mg rhDNase once daily. Her cough lessened and the volume of sputum decreased within 72 hours of commencement of treatment; this improvement was strongly associated with a dramatic reduction in gastrointestinal symptoms. Spirometry after 4 weeks of treatment demonstrated a 20% improvement in FEV1 and a 13% improvement in FVC. These improvements have been maintained after 4 months of rhDNase therapy. The use of rhDNase should be considered in patients with Kartagener's syndrome and a multicenter trial may be justified.
雾化重组人脱氧核糖核酸酶（rhDNase）可降低痰液黏稠度，改善肺功能，并使许多囊性纤维化（CF）患者因急性呼吸道加重而需要静脉使用抗生素的情况略有减少。目前推荐rhDNase用于患有中度严重化脓性肺病的CF患者。一名14岁患有化脓性肺病的女孩[一秒用力呼气量（FEV1）为预测值的69%，用力肺活量（FVC）为预测值的81%]，继发于卡塔格内综合征和严重胃食管反流，尽管接受了常规治疗，但在过去18个月中肺活量测定结果恶化，同时伴有难以治疗的胃肠道症状。因此，她开始每天一次使用2.5毫克rhDNase。治疗开始后72小时内，她的咳嗽减轻，痰液量减少；这种改善与胃肠道症状的显著减轻密切相关。治疗4周后的肺活量测定结果显示，FEV1提高了20%，FVC提高了13%。rhDNase治疗4个月后，这些改善情况得以维持。对于患有卡塔格内综合征的患者，应考虑使用rhDNase，且开展一项多中心试验可能是合理的。
