Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis

Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis
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DOI:
10.1001/jama.293.5.581
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发表时间:
2005-02-02
影响因子:
120.7
通讯作者:
Splaingard, ML
Splaingard, ML
中科院分区:
医学1区
文献类型:
--
作者:
Li, ZH;Kosorok, MR;Splaingard, ML

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背景虽然铜绿假单胞菌是囊性纤维化(CF)中最常见的毒性呼吸道病原体,但铜绿假单胞菌感染的纵向发展及其对CF儿童抗体应答和肺部疾病进展的影响仍不清楚。我们前瞻性评估了1985年4月15日至2004年4月15日期间在威斯康星州麦迪逊和密尔沃基的2个CF中心的56名CF患者,从出生到16岁,通过威斯康星州CF新生儿筛查项目进行诊断。主要结果测量通过首次阳性结果评估非粘液型铜绿假单胞菌和粘液型铜绿假单胞菌获得的时间。研究了从无铜绿假单胞菌到非粘液铜绿假单胞菌以及从非粘液铜绿假单胞菌到粘液铜绿假单胞菌的纵向发育。结果测量包括抗体滴度,呼吸道症状,定量胸片,肺功能tests.Results 16例(29%)收购nonmucoid Paerplasty在前6个月的生活。年龄特异性的粘液性银屑病患病率从4岁到16岁显著增加。非粘液型和粘液型铜绿假单胞菌的中位年龄分别为1.0岁和13.0岁。与从无铜绿假单胞菌到非粘液型铜绿假单胞菌的转变时间较短相比,从非粘液型铜绿假单胞菌到粘液型铜绿假单胞菌的转变时间相对较长(中位数,10.9年),并且可以通过短暂/低剂量抗铜绿假单胞菌抗生素治疗稍微延长。抗体滴度增加,这两个过渡,但在咳嗽评分,胸片评分,肺功能恶化相关最好的过渡,从非粘液型到粘液型铜绿假单胞菌。结论早期预防和检测非粘液型和粘液型铜绿假单胞菌是至关重要的,因为早期收购和流行。初始非粘液性铜绿假单胞菌存在抑制和可能根除(通过积极的抗铜绿假单胞菌治疗)的机会窗。粘液性肺水肿在CF肺疾病进展中的作用比非粘液性肺水肿大得多。抗体滴度、咳嗽评分和胸片是非粘液型铜绿假单胞菌尤其是粘液型铜绿假单胞菌分期的早期体征。
Context Although Pseudomonas aeruginosa is the most common virulent respiratory pathogen in cystic fibrosis (CF), the longitudinal development of P aeruginosa infection and its effect on antibody responses and lung disease progression in children with CF remain unclear.Objective To prospectively examine the epidemiology of Paeruginosa infection and its impact on CF pulmonary morbidity.Design, Setting, and Patients We prospectively evaluated 56 CF patients at 2 CF centers in Madison and Milwaukee, Wis, from birth up to age 16 years between April 15, 1985, and April 15, 2004, with diagnoses made through the Wisconsin CF Neonatal Screening Project.Main Outcome Measures Timing of nonmucoid P aeruginosa and mucoid P aeruginosa acquisition was assessed by first positive result. Longitudinal development from no P aeruginosa to nonmucoid P aeruginosa and from nonmucoid Paerugginosa to mucoid Paeruginosa was examined. Outcome measurements included antibody titers, respiratory symptoms, quantitative chest radiography, and pulmonary function tests.Results Sixteen patients (29%) acquired nonmucoid Paeruginosa in the first 6 months of life. The age-specific prevalence of mucoid Paeruginosa increased markedly from age 4 to 16 years. Nonmucoid and mucoid P aeruginosa were acquired at median ages of 1.0 and 13.0 years, respectively. In contrast with the short transition time from no Paeruginosa to nonmucoid P aeruginosa, the transition time from nonmucoid to mucoid P aeruginosa was relatively long (median, 10.9 years) and could be slightly extended by brief/low anti-Paeruginosa antibiotic treatment. Antibody titers increased with both transitions, but the deterioration in cough scores, chest radiograph scores, and pulmonary function correlated best with transition from nonmucoid to mucoid P aeruginosa.Conclusions Early prevention and detection of nonmucoid and mucoid P aeruginosa are critical because of early acquisition and prevalence. There is a Window of opportunity for suppression and possible eradication (by aggressive anti-P aeruginosa treatment) of initial nonmucoid Paeruginosa. Mucoid Paeruginosa plays a much greater role in CF lung disease progression than nonmucoid Paeruginosa. Antibody titers, cough scores, and chest radiographs are early signs of nonmucoid P aeruginosa and especially mucoid P aeruginosa stages.