共查询到10条相似文献，搜索用时 15 毫秒
1.
Quitterie Reynaud Muriel Rabilloud Sylvain Roche Stéphanie Poupon-Bourdy Jean Iwaz Raphaële Nove-Josserand Emilie Blond Martine Laville Cathy Llerena Sébastien Quetant Philippe Reix Sandrine Touzet Isabelle Durieu 《Journal of cystic fibrosis》2018,17(3):400-406
Background
The prevalence of cystic fibrosis-related diabetes is increasing. This condition is potentially responsible for respiratory decline.Methods
At inclusion, then yearly (over three years), 111 children and 117 adults with cystic fibrosis had oral glucose tolerance and insulin tests at one (G1) and 2 h (G2). KmL analysis identified homogeneous G1 and G2 glucose trajectories. A linear mixed model quantified the relationships between trajectories and FEV1 changes.Results
In children, there were three G1 and four G2 trajectories and FEV1 decrease was not significantly different between G1 or G2 trajectories. In adults, two G1 and four G2 trajectories were identified and FEV1 change was estimated at ? 0.85/year (95% CI: [? 1.54; ? 0.17], p = 0.01) whatever the G1 trajectory and found significantly faster in the high and increasing G2 trajectory (? 2.1/year, [? 3.9; ? 0.2], p = 0.03).Conclusions
In case of persistent G2 abnormality, physicians should be alert for clinical deterioration and intensify patient surveillance. 相似文献2.
Jordana E. Hoppe Brandie D. Wagner Frank J. Accurso Edith T. Zemanick Scott D. Sagel 《Journal of cystic fibrosis》2018,17(6):760-768
Background
Pulmonary exacerbations (PEx) in children with cystic fibrosis (CF) are frequently treated in the outpatient setting with oral antibiotics. However, little is known about the characteristics of PEx managed on an outpatient basis and the effectiveness of oral antibiotic therapy. We sought to prospectively evaluate clinical and laboratory changes associated with oral antibiotic treatment for PEx.Methods
Children with CF between 8 and 18?years of age prescribed two weeks of oral antibiotics for a PEx were eligible to enroll. The study consisted of a visit within 48?h of starting antibiotics and a second visit within one week of antibiotic completion. Twenty-eight participants were evaluated by exacerbation score, quality of life measurements, lung function, sputum microbiology and inflammation.Results
Oral antibiotic treatment was associated with a significant improvement in exacerbation score and quality of life measured by the CF Questionnaire-Revised (CFQ-R) respiratory domain. Following treatment, forced expiratory volume in 1?s (FEV1) % predicted increased [median (range)] 9% (?8%, 31%), and 22 (81%) subjects returned to 90% or higher of baseline FEV1. Bacterial density of the primary organism identified on sputum culture decreased significantly with a median (range) decrease of 0.8 log10 cfu/mL (?8 log10, 2 log10, p?=?0.03). Sputum neutrophil elastase [?37?μg/mL (?464, 272), p?=?0.02] and IL-1β [?2.8?×?103μg/mL (?6.9?×?104, 3.3?×?104), p?=?0.03] decreased significantly following treatment in this cohort.Conclusions
Treatment of PEx with oral antibiotics was associated with measurable improvements in patient reported outcomes, lung function, bacterial density and sputum inflammatory markers. 相似文献3.
Patrick A. Flume Claire E. Wainwright D. Elizabeth Tullis Sally Rodriguez Minoo Niknian Mark Higgins Jane C. Davies Jeffrey S. Wagener 《Journal of cystic fibrosis》2018,17(1):83-88
Background
Pulmonary exacerbations (PEx) are associated with acute loss of lung function that is often not recovered after treatment. We investigated lung function recovery following PEx for ivacaftor- and placebo-treated subjects.Methods
Short- and long-term pulmonary function recovery data after PEx were summarized from a placebo-controlled trial in 161 cystic fibrosis patients ≥ 12 years old with the G551D-CFTR mutation (NCT00909532). Short-term recovery was measured 2 to 8 weeks after treatment, and long-term recovery was determined at the end-of-study, both compared with baseline measured just prior to the PEx.Results
Fewer patients receiving ivacaftor experienced a PEx than patients receiving placebo (33.7% vs. 56.4%; P = 0.004) and had a lower adjusted incidence rate of PEx (0.589 vs. 1.382; P < 0.001). The proportion of PEx followed by full short-term recovery of percent predicted forced expiratory volume in 1 s was similar (ivacaftor vs. placebo, 57.1% vs. 53.7), as was the proportion of patients having long-term recovery (46.4% vs. 47.7%).Conclusions
Ivacaftor treatment reduces the frequency of PEx but does not improve on the rate of complete lung function recovery after PEx when compared with placebo. 相似文献4.
Munck A Gérardin M Alberti C Ajzenman C Lebourgeois M Aigrain Y Navarro J 《Journal of pediatric surgery》2006,41(9):1556-1560
Objective
This matched case-control study compared the nutritional and the pulmonary long-term outcomes of cystic fibrosis (CF) patients presenting a history of meconium ileus (MI) with early-diagnosed symptomatic CF without MI (non-MI).Material and Method
Twenty-six patients with CF treated for MI between 1980 and 1997 have been matched for sex, birth date, and earliest CF symptomatic diagnosis for the children with non-MI CF. Clinical characteristics, genotype and complications were evaluated as well as the progression of the CF disease from infancy to 15 years old by nutritional status (z score weight, z score height), pulmonary function tests (PFTs) (FVC and FEV1), and Pseudomonas aeruginosa acquisition.Results
Median duration of the follow-up was 12.5 years (range, 10-17 years). Genotype identification showed no significant difference. Further on, the rate of complications and the occurrence of chronic P aeruginosa colonization did not differ. At age of 15 years (n = 13), nutritional status and PFTs did not demonstrate any significant difference.Conclusion
These results suggest that adequate initial nutritional and medical management of MI allows further similar nutritional status and PFTs compared with other early-diagnosed symptomatic CF patients. In this study, MI did not represent an additional risk factor for the patient's life. 相似文献5.
Koji Komori Seiichi Hirobe Kenji Okumura Shogo Kasai Mayumi Suenaga 《Journal of pediatric surgery》2009,44(11):2096-2100
Purpose
We evaluated the long-term pulmonary function after lobectomy for congenital cystic lung disease, in both infants and children, using radionuclide imaging (RI).Methods
We performed a retrospective review of 93 patients who underwent resection of cystic lung lesions between 1974 and 2001. The results of postoperative lung volume/perfusion scintigraphy at 1 (n = 64), 5 (n = 32), and 10 years (n = 18) after surgery (V1, 5, 10/Q1, 5, 10) and mean transit time (MTT—a marker for air-trapping) at 1, 5, and 10 years after surgery (MTT1, 5, 10) were compared with respect to age at operation, preoperative infection, underlying disease, and type of surgery.Results
Patients who were younger than 1 year at the time of surgery showed a significantly lower MTT5 (1.09 ± 0.08) and MTT10 (1.15 ± 0.11) than patients who were older than 1 year at the time of surgery (MTT5, 1.49 ± 0.67; MTT10, 1.54 ± 0.33). The noninfected group had significantly higher Q10 and lower MTT10 values (P < .05) compared to the infected group. No significant differences were observed between patients with single lobe vs multiple lobe resection.Conclusions
The optimal age for surgery in patients with congenital cystic lung disease appears to be less than 1 year. 相似文献6.
目的比较分析胸腔镜解剖性肺段切除及肺楔形切除术对肺小气道功能的影响。 方法收集2021年1~6月期间,在民航总医院行胸腔镜肺段切除及肺楔形切除124例患者资料,其中行解剖性肺段切除64例、肺楔形切除60例。通过比较两组患者术前、术后1周,以及术后1、3、6个月的MEF25%、MEF50%、MEF75%/25%变化,明确肺段切除与肺楔形切除对肺小气道功能的影响。 结果MEF50%、MEF75%/25%、MEF25%两组指标术后1周均降低,两者之间差异无统计学意义;术后1个月较术后1周升高,且肺楔形切除组较肺段切除组结果升高;术后3个月两组指标均较前升高,且肺楔形切除组仍较肺段切除组结果高;MEF50%、MEF75%/25%术后6个月恢复至术前水平,MEF25%肺楔形切除组恢复至术前水平,较肺段切除组指标高,肺段切除组尚未恢复至术前水平。 结论小气道肺功能指标更能反映出机体肺功能情况;对于<1 cm且实性成分<5 mm的肺小结节,肺楔形切除术后肺功能指标比肺段切除恢复较快;提示切除肺部组织越小,保留的肺功能越多,越具有长期的功能优势。 相似文献
7.
Don B. Sanders George M. Solomon Valeria V. Beckett Natalie E. West Cori L. Daines Sonya L. Heltshe Donald R. VanDevanter Jonathan E. Spahr Ronald L. Gibson Jerry A. Nick Bruce C. Marshall Patrick A. Flume Christopher H. Goss 《Journal of cystic fibrosis》2017,16(5):592-599
Background
The Standardized Treatment of Pulmonary Exacerbations (STOP) program has the intent of defining best practices in the treatment of pulmonary exacerbations (PEx) in patients with cystic fibrosis (CF). The objective of this analysis was to describe the clinical presentations of patients admitted for intravenous (IV) antibiotics and enrolled in a prospective observational PEx study as well as to understand physician treatment goals at the start of the intervention.Methods
We enrolled adolescents and adults admitted to the hospital for a PEx treated with IV antibiotics. We recorded patient and PEx characteristics at the time of enrollment. We surveyed treating physicians on treatment goals as well as their willingness to enroll patients in various study designs. Additional demographic and clinical data were obtained from the CF Foundation Patient Registry.Results
Of 220 patients enrolled, 56% were female, 19% were adolescents, and 71% were infected with P. aeruginosa. The mean (SD) FEV1 at enrollment was 51.1 (21.6)% predicted. Most patients (85%) experienced symptoms for ≥ 7 days before admission, 43% had received IV antibiotics within the previous 6 months, and 48% received oral and/or inhaled antibiotics prior to IV antibiotic initiation. Forty percent had ≥ 10% FEV1 decrease from their best value recorded in the previous 6 months, but for 20% of patients, their enrollment FEV1 was their best FEV1 recorded within the previous 6 months. Physicians reported that their primary treatment objectives were lung function recovery (53%) and improvement of symptoms (47%) of PEx. Most physicians stated they would enroll patients in studies involving 10-day (72%) or 14-day (87%), but not 7-day (29%), treatment regimens.Conclusions
Based on the results of this study, prospective studies are feasible and physician willingness for interventional studies of PEx exists. Results of this observational study will help design future PEx trials. 相似文献8.
Natalie E. West Valeria V. Beckett Raksha Jain Don B. Sanders Jerry A. Nick Sonya L. Heltshe Elliott C. Dasenbrook Donald R. VanDevanter George M. Solomon Christopher H. Goss Patrick A. Flume 《Journal of cystic fibrosis》2017,16(5):600-606
Background
Pulmonary Exacerbations (PEx) are associated with increased morbidity and mortality in individuals with CF. PEx management practices vary widely, and optimization through interventional trials could potentially improve outcomes. The object of this analysis was to evaluate current physician treatment practices and patient outcomes for PEx.Methods
The Standardized Treatment of Pulmonary Exacerbations (STOP) observational study enrolled 220 participants ≥ 12 years old admitted to the hospital for PEx at 11 U.S. CF centers. Spirometry and daily symptom scores were collected during the study. Physicians were surveyed on treatment goals and their management practices were observed. Treatment outcomes were compared to stated goals.Results
The mean (SD) duration of IV antibiotic treatment was 15.9 (6.0) days. Those individuals with more severe lung disease (< 50% FEV1) were treated nearly two days longer than those with > 50% FEV1. Physician-reported FEV1 improvement goals were 10% (95% CI: 5%, 14%) lower for patients with 6-month baseline FEV1 ≤ 50% predicted compared with those with 6-month baseline FEV1 > 50% predicted. There were clinically and statistically significant improvements in symptoms from the start of IV antibiotic treatment to the end of IV antibiotic treatment and 28 days after the start of treatment. The mean absolute increase in FEV1 from admission was 9% predicted at end of IV antibiotic treatment, and 7% predicted at day 28. Only 39% fully recovered lost lung function, and only 65% recovered at least 90% of lost lung function. Treatment was deemed successful by 84% of clinicians, although 6-month baseline FEV1 was only recovered in 39% of PEx.Conclusions
In this prospective observational study of PEx, treatment regimens and durations showed substantial variation. A significant proportion of patients did not reach physician's treatment goals, yet treatment was deemed successful. 相似文献9.
F.H. Pals H.J. Verkade V.A.M. Gulmans B.A.E. De Koning B.G.P. Koot T.G.J. De Meij D.M. Hendriks N. Gierenz A.C.E. Vreugdenhil R.H.J. Houwen F.A.J.A. Bodewes 《Journal of cystic fibrosis》2019,18(3):385-389
BackgroundUp to 10% of patients with Cystic Fibrosis develop cirrhotic CF-related liver disease with portal hypertension: CF cirrhosis (CFC). In a nationwide study, we aimed to determine the role of CFC on survival in the Netherlands between 1 and 1-2009 and1-1-2015.MethodsWe identified all CFC patients in the Netherlands, based on ultrasonographic liver nodularity and portal hypertension. A non-cirrhotic control group was obtained from the national Dutch CF patient registry. We compared groups with regards to baseline lung function and nutritional status and survival and age at death over a 6-year period. In case of death of CFC patients, the clinical reported cause was recorded.ResultsAt baseline, we found no significant difference in lung function and nutritional status between the CFC patients (N = 95) and controls (N = 980). Both the 6-year survival rate (77 vs. 93%; P < .01) and the median age at death (27 vs. 37 years; P = .02) was significantly lower in CFC compared to controls. In the deceased CFC patients, the reported primary cause of death was pulmonary in 68% of cases, and liver failure related in 18% of cases.ConclusionsIn the Netherlands, the presence of CFC is associated with a higher risk for early mortality and an approximately 10-year lower median age at death. This substantial poorer outcome of CFC patients was not reflected in a lower baseline lung function or a diminished nutritional status. However, in the case of mortality, the reported primary cause of death in CFC patients is predominantly pulmonary failure and not end-stage liver disease. 相似文献
10.