Tonelli Adriano R, Drane Walter E, Collins Dennis P, Nichols Wanda, Antony Veena B, Olson Eric L
Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Gainesville, Florida, United States.
J Cyst Fibros. 2009 May;8(3):193-7. doi: 10.1016/j.jcf.2009.02.001. Epub 2009 Mar 9.
Gastrointestinal manifestations are frequently encountered in cystic fibrosis patients. Gastroparesis evidenced by a variety of diagnostic methods has been described in patients with cystic fibrosis, predominantly in children and in individuals with advanced lung disease. The presence of gastroparesis in adult patients with different degrees of lung involvement and its response to the acute and chronic administration of macrolides have not been reported.
Using the University of Florida Cystic Fibrosis database we identified symptomatic patients who had gastroparesis confirmed by a prolonged half-time during gastric emptying scintigraphy.
Of 86 cystic fibrosis patients, periodically followed in our institution, we found five who had classical symptoms and prolonged gastric emptying half-time. Age 25.2+/-8 years, 80% females, BMI 22+/-9 kg/m(2), HbA1c 5.8+/-0.6 g/dl, FEV1 53.2+/-15% of predicted. Gastric emptying half-time was 191.4+/-91.4 min (range 100-300 min) and decreased to 12.2+/-6 min (range 5-20 min) after IV administration of erythromycin (p=0.043). Patients were followed up for 3+/-2.1 years. All patients but one, who was taking opiods, had good clinical response to PO macrolides.
Gastroparesis occurs in patients with cystic fibrosis, even in patients with relatively preserved lung function and in those without cystic-fibrosis related diabetes. Macrolides may be an effective therapy in cystic fibrosis patients with gastroparesis when administered acutely or chronically.
胃肠道表现常见于囊性纤维化患者。多种诊断方法证实的胃轻瘫已在囊性纤维化患者中被描述,主要见于儿童及晚期肺部疾病患者。不同程度肺部受累的成年囊性纤维化患者中胃轻瘫的存在情况及其对大环内酯类药物急性和慢性给药的反应尚未见报道。
利用佛罗里达大学囊性纤维化数据库,我们识别出经胃排空闪烁扫描时半衰期延长证实患有胃轻瘫的有症状患者。
在我们机构定期随访的86例囊性纤维化患者中,我们发现5例有典型症状且胃排空半衰期延长。年龄25.2±8岁,80%为女性,体重指数22±9kg/m²,糖化血红蛋白5.8±0.6g/dl,第1秒用力呼气容积占预计值的53.2±15%。胃排空半衰期为191.4±91.4分钟(范围100 - 300分钟),静脉注射红霉素后降至12.2±6分钟(范围5 - 20分钟)(p = 0.043)。患者随访3±2.1年。除1例服用阿片类药物的患者外,所有患者口服大环内酯类药物均有良好临床反应。
囊性纤维化患者中会发生胃轻瘫,即使在肺功能相对保留的患者以及无囊性纤维化相关糖尿病的患者中也会出现。大环内酯类药物在急性或慢性给药时,可能是囊性纤维化合并胃轻瘫患者的有效治疗方法。