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1.
Juliana I. Sesma Bryant Wu Timothy J. Stuhlmiller David W. Scott 《Journal of cystic fibrosis》2019,18(2):244-250
Background
In healthy lungs, epithelial sodium channel (ENaC) is regulated by short, palate, lung, and nasal clone 1 (SPLUNC1). In cystic fibrosis (CF), ENaC is hyperactivated in part due to a loss of SPLUNC1 function. We have developed SPX-101 to replace the lost function of SPLUNC1 in the CF lung.Methods
Expression of SPLUNC1 was determined in sputum from healthy and CF donors. Stability of SPLUNC1, S18 (the ENaC regulatory domain of SPLUNC1), and SPX-101 was determined in sputum from CF donors and towards neutrophil elastase. Activity of SPX-101 after exposure to CF sputum was determined in airway epithelial cells from CF donors and in the βENaC transgenic mouse model.Results
SPLUNC1 protein expression is significantly reduced in CF as compared to healthy sputum. SPLUNC1 is rapidly degraded in CF sputum as well as by a number of individual proteases known to be found in the sputum. SPX-101, but not S18, is stable in CF sputum. Finally, SPX-101 retains its ability to internalize ENaC, regulate airway surface liquid height, and increase survival of βENaC mice after exposure to CF sputum.Conclusions
Our results demonstrate that SPX-101, but not SPLUNC1 or S18, is stable in CF sputum. These results support the therapeutic development of SPX-101 for the treatment of cystic fibrosis. 相似文献2.
Binyu Lu Li Li Molly Schneider Craig A. Hodges Calvin U. Cotton James D. Burgess Thomas J. Kelley 《Journal of cystic fibrosis》2019,18(2):175-181
Background
Previous studies have demonstrated that CF epithelial cells exhibit increased cholesterol content at the plasma membrane compared to wild type controls as measured by electrochemical methods. Microtubule dysregulation that impacts intracellular transport has also been identified in CF cells and is reversible with histone deacetylase 6 (HDAC6) inhibition, a regulator of tubulin acetylation. The hypothesis of this study is that increased membrane cholesterol content in CF cells is dependent on HDAC6 regulation.Methods
Electrochemical measurement of membrane cholesterol in mouse trachea and in primary human CF bronchial epithelial cells is used to monitor CFTR correction and manipulation of cholesterol processing by HDAC6 inhibition.Results
Data demonstrate that induction of Cftr expression in an inducible CF mouse model restores tubulin acetylation levels and normalizes membrane cholesterol content. To test the relationship between tubulin acetylation, membrane cholesterol levels were measured in a CF mouse model depleted of Hdac6 expression (CF/HDA). CF/HDA mouse trachea have WT membrane cholesterol levels while CF mice have approximately two-fold increase in membrane cholesterol compared to WT consistent with previous studies. Pharmacological inhibition of HDAC6 in primary human CF bronchial epithelial cells also reduces membrane cholesterol levels.Conclusions
This study demonstrates that elevated membrane cholesterol in CF epithelium is regulated by HDAC6 function and that the electrochemical measure of membrane cholesterol correlates with both genetic and pharmacological CFTR correction. 相似文献3.
4.
Angel Li Tim Vigers Laura Pyle Edith Zemanick Kristen Nadeau Scott D. Sagel Christine L. Chan 《Journal of cystic fibrosis》2019,18(1):144-149
Background
The effects of lumacaftor-ivacaftor therapy on glycemia have not been thoroughly investigated. Continuous glucose monitoring (CGM) provides detailed information about glycemic patterns and detects glucose abnormalities earlier than traditional screening tools for diabetes.Methods
CGM measures, HbA1c, and oral glucose tolerance test (OGTT) results were collected and within-subject results compared in F508del homozygous youth with CF before and after initiation of lumacaftor-ivacaftor using the Wilcoxon signed-rank test.Results
Nine youth with CF (6 males, median age 12.7?years) were enrolled. CGM was performed in all participants before (median 26?weeks) and after lumacaftor-ivacaftor (median 29?weeks). HbA1c and fasting plasma glucose increased (p?=?.02) after lumacaftor-ivacaftor initiation. No changes in OGTT 1?h or 2?h glucose nor CGM measures were observed overall. When analyzed by sex, males showed lower glycemic variability, as reflected by the mean amplitude of glycemic excursions, on the post-treatment CGM.Conclusions
Glycemic abnormalities persisted in CF patients treated with lumacaftor-ivacaftor, although sex-dependent differences in glycemic response to treatment may exist. 相似文献5.
Béla Nagy Zsolt Bene Zsolt Fejes Sonya L. Heltshe David Reid Nicola J. Ronan Yvonne McCarthy Daniel Smith Attila Nagy Elizabeth Joseloff György Balla János Kappelmayer Milan Macek Scott C. Bell Barry J. Plant Margarida D. Amaral István Balogh 《Journal of cystic fibrosis》2019,18(2):271-277
Background
We have recently shown that human epididymis protein 4 (HE4) levels correlate with the severity of cystic fibrosis (CF) lung disease. However, there are no data on how HE4 levels alter in patients receiving CFTR modulating therapy.Methods
In this retrospective clinical study, 3 independent CF patient cohorts (US-American: 29, Australian: 12 and Irish: 19 cases) were enrolled carrying at least one Class III CFTR CF-causing mutation (p.Gly551Asp) and being treated with CFTR potentiator ivacaftor. Plasma HE4 was measured by immunoassay before treatment (baseline) and 1–6?months after commencement of ivacaftor, and were correlated with FEV1 (% predicted), sweat chloride, C-reactive protein (CRP) and body mass index (BMI).Results
After 1?month of therapy, HE4 levels were significantly lower than at baseline and remained decreased up to 6?months. A significant inverse correlation between absolute and delta values of HE4 and FEV1 (r?=??0.5376; P?<?.001 and r?=??0.3285; P?<?.001), was retrospectively observed in pooled groups, including an independent association of HE4 with FEV1 by multiple regression analysis (β?=??0.57, P?=?.019). Substantial area under the receiver operating characteristic curve (ROC-AUC) value was determined for HE4 when 7% mean change of FEV1 (0.722 [95% CI 0.581–0.863]; P?=?.029) were used as classifier, especially in the first 2?months of treatment (0.806 [95% CI 0.665–0.947]; P?<?.001).Conclusions
This study shows that plasma HE4 levels inversely correlate with lung function improvement in CF patients receiving ivacaftor. Overall, this potential biomarker may be of value for routine clinical and laboratory follow-up of CFTR modulating therapy. 相似文献6.
Insa Korten Elisabeth Kieninger Sophie Yammine Giulia Cangiano Sylvia Nyilas Pinelopi Anagnostopoulou Florian Singer Claudia E. Kuehni Nicolas Regamey Urs Frey Carmen Casaulta Ben D. Spycher Philipp Latzin 《Journal of cystic fibrosis》2019,18(1):118-126
Background
Lung impairment in cystic fibrosis (CF) starts in infancy. However, tools to monitor early lung disease are limited. Respiratory rate (RR) as a key vital sign is easy to assess during sleep and is elevated during acute respiratory disease. Thus, elevated RR could indicate early lung impairment and potentially serve as a diagnostic tool in disease monitoring.Methods
In a prospective cohort of infants with CF diagnosed by newborn screening and healthy controls RR was measured and respiratory symptoms reported weekly throughout infancy. Infants performed a lung function measurement within the first weeks of life.Results
The analyses included 5656 measurements from 153 infants (43 with CF). RR declined from 43.2 (40.5)/min at 6?weeks of age to 28.3 (24.6)/min at 50?weeks in infants with CF (healthy controls). Infants with CF had consistently higher RR than controls (mean difference: 4.15/min; (95% CI 2.86–5.44); p?<?.001). In both study groups, RR was increased throughout the study period in infants with higher lung clearance indices (LCI) and during episodes of respiratory infections.Conclusions
Infants with CF have a higher RR compared to healthy controls during the first year of life. The association with early LCI measurements, the current gold standard to assess physiology of peripheral airways persisted throughout the study period. This may indicate tracking of lung function by RR. It might thus be an early subtle sign of functional respiratory deficit. Further studies will show if RR can be used as a sensitive and promising marker to monitor early CF lung disease. 相似文献7.
Katherine Keenan Annie Dupuis Katherine Griffin Carlo Castellani Elizabeth Tullis Tanja Gonska 《Journal of cystic fibrosis》2019,18(2):265-270
Background
The “mild” gene variant, p.Arg117His in cystic fibrosis (CF) results in highly variable phenotypes ranging from male infertility to severe lung disease. Due to current interest to include this group in CFTR-targeted therapies, this study aims to describe the disease spectrum.Methods
Retrospective study of Toronto CF and CFTR-related p.Arg117His patients. Longitudinally captured clinical data were compared between patients with 5T/7T-variants and those with a CF or CFTR-related diagnosis. Comparison was made between p.Arg117His adults and infants identified through CF newborn screening (NBS).Results
Twenty of fifty patients carried the 5T variant, all with a diagnosis of CF (p.Arg117His-5TCF), and 30/50 carried 7T, 7 diagnosed with CF (p.Arg117His-7TCF) and 23 with a CFTR-related disorder (p.Arg117His-7TCFTR). For those with chest HRCT results available, 75% p.Arg117His-5TCF, 33% p.Arg117His-7TCF and 27% p.Arg117His-7TCFTR patients had bronchiectasis. Further, 79% p.Arg117His-5T, 29% p.Arg117His-7TCF and 13% p.Arg117His-7TCFTR had abnormal lung function. Of those, 80% grew CF-related pathogens on respiratory culture. Interestingly, the mean maximum sweat chloride and the percentage of patients growing CF-related bacterial pathogens were identical in p.Arg117His-7?TCFTR adults and p.Arg117His infants.Conclusions
Generally, p.Arg117His-5T patients had more severe CF disease. However, a subset of p.Arg117His-7?T patients demonstrated equally severe disease, thus warranting clinical monitoring of all p.Arg117His patients including p.Arg117His infants identified via NBS. 相似文献8.
Kathrin Krause Benjamin T. Kopp Mia F. Tazi Kyle Caution Kaitlin Hamilton Asmaa Badr Chandra Shrestha Dmitry Tumin Don Hayes Frank Robledo-Avila Luanne Hall-Stoodley Brett G. Klamer Xiaoli Zhang Santiago Partida-Sanchez Narasimham L. Parinandi Stephen E. Kirkby Duaa Dakhlallah Karen S. McCoy Amal O. Amer 《Journal of cystic fibrosis》2018,17(4):454-461
Introduction
Cystic fibrosis (CF) is a multi-organ disorder characterized by chronic sino-pulmonary infections and inflammation. Many patients with CF suffer from repeated pulmonary exacerbations that are predictors of worsened long-term morbidity and mortality. There are no reliable markers that associate with the onset or progression of an exacerbation or pulmonary deterioration. Previously, we found that the Mirc1/Mir17–92a cluster which is comprised of 6 microRNAs (Mirs) is highly expressed in CF mice and negatively regulates autophagy which in turn improves CF transmembrane conductance regulator (CFTR) function. Therefore, here we sought to examine the expression of individual Mirs within the Mirc1/Mir17–92 cluster in human cells and biological fluids and determine their role as biomarkers of pulmonary exacerbations and response to treatment.Methods
Mirc1/Mir17–92 cluster expression was measured in human CF and non-CF plasma, blood-derived neutrophils, and sputum samples. Values were correlated with pulmonary function, exacerbations and use of CFTR modulators.Results
Mirc1/Mir17–92 cluster expression was not significantly elevated in CF neutrophils nor plasma when compared to the non-CF cohort. Cluster expression in CF sputum was significantly higher than its expression in plasma. Elevated CF sputum Mirc1/Mir17–92 cluster expression positively correlated with pulmonary exacerbations and negatively correlated with lung function. Patients with CF undergoing treatment with the CFTR modulator Ivacaftor/Lumacaftor did not demonstrate significant change in the expression Mirc1/Mir17–92 cluster after six months of treatment.Conclusions
Mirc1/Mir17–92 cluster expression is a promising biomarker of respiratory status in patients with CF including pulmonary exacerbation. 相似文献9.
Rebecca J. Darrah Frank J. Jacono Neha Joshi Anna L. Mitchell Abdus Sattar Cara K. Campanaro Paul Litman Jennifer Frey David E. Nethery Eric S. Barbato Craig A. Hodges Harriet Corvol Garry R. Cutting Michael R. Knowles Lisa J. Strug Mitchell L. Drumm 《Journal of cystic fibrosis》2019,18(1):127-134
Background
Pulmonary disease remains the primary cause of morbidity and mortality for individuals with cystic fibrosis (CF). Variants at a locus on the X-chromosome containing the type 2 angiotensin II receptor gene (AGTR2) were identified by a large GWAS as significantly associating with lung function in CF patients. We hypothesized that manipulating the angiotensin-signaling pathway may yield clinical benefit in CF.Methods
Genetic subset analysis was conducted on a local CF cohort to extend the GWAS findings. Next, we evaluated pulmonary function in CF mice with a deleted AGTR2 gene, and in those who were given subcutaneous injections of PD123,319, a selective AGTR2 antagonist for 12?weeks beginning at weaning.Results
The genetic subset analysis replicated the initial GWAS identified association, and confirmed the association of this locus with additional lung function parameters. Studies in genetically modified mice established that absence of the AGTR2 gene normalized pulmonary function indices in two independent CF mouse models. Further, we determined that pharmacologic antagonism of AGTR2 improved overall pulmonary function in CF mice to near wild-type levels.Conclusions
These results identify that reduced AGTR2 signaling is beneficial to CF lung function, and suggest the potential of manipulating the angiotensin-signaling pathway for treatment and/or prevention of CF pulmonary disease. Importantly, the beneficial effects were not CF gene mutation dependent, and were able to be reproduced with pharmacologic antagonism. As there are clinically approved drugs available to target the renin-angiotensin signaling system, these findings may be quickly translated to human clinical trials. 相似文献10.
Background
Cystic fibrosis (CF, mucoviscidosis) is caused by mutations in the gene encoding CF transmembrane conductance regulator (CFTR), which is a chloride and bicarbonate channel necessary for fluid secretion and extracellular alkalization. For a long time, research concentrated on abnormal Cl- and Na+ transport, but neglected bicarbonate as a crucial factor in CF.Methods
The present short review reports early findings as well as recent insights into the role of CFTR for bicarbonate transport and its defects in CF.Results
The available data indicate impaired bicarbonate transport not only in pancreas, intestine, airways, and reproductive organs, but also in salivary glands, sweat duct and renal tubular epithelial cells. Defective bicarbonate transport is closely related to the impaired mucus properties and mucus blocking in secretory organs of CF patients, causing the life threatening lung disease.Conclusions
Apart from the devastating lung disease, abrogated bicarbonate transport also leads to many other organ dysfunctions, which are outlined in the present review. 相似文献11.
S.J. Newsome R.M. Daniel S.B. Carr D. Bilton R.H. Keogh 《Journal of cystic fibrosis》2019,18(1):110-117
Background
Dornase alfa (DNase) is one of the commonest cystic fibrosis (CF) treatments and is often used for many years. However, studies have not evaluated the effectiveness of its long-term use. We aimed to use UK CF Registry data to investigate the effects of one-, two-, three-, four- and five-years of DNase use on lung function to see if the benefits of short-term treatment use are sustained long term.Methods
We analysed data from 4,198 people in the UK CF Registry from 2007 to 2015 using g-estimation. By controlling for time-dependent confounding we estimated the effects of long-term DNase use on percent predicted FEV1 (ppFEV1) and investigated whether the effect differed by ppFEV1 at treatment initiation or by age.Results
Considering the population as a whole, there was no significant effect of one-year's use of DNase; change in ppFEV1 over one year was ?0.1% in the treated compared to the untreated (p?=?0.51) and this did not change with long-term use. However, treatment was estimated to be more beneficial in people with lower lung function (p?<?0.001); those with ppFEV1?<?70% at treatment initiation, showed an increase in lung function over one year that was sustained out to five years. The estimated effect of DNase did not depend on age (p?=?0.35).Conclusions
DNase improved lung function in individuals with reduced lung function, bringing a step-change in lung function, but no change in the slope of decline. There was no evidence for a benefit in lung function in those initiating treatment with ppFEV1?>?70%. 相似文献12.
13.
Alison DaCosta Cameron L. Jordan Olivia Giddings Feng-Chang Lin Peter Gilligan Charles R. Esther 《Journal of cystic fibrosis》2017,16(4):483-487
Background
Mycobacterium abscessus infection is associated with declining lung function in cystic fibrosis (CF), but there is little evidence on clinical efficacy to guide treatment.Methods
Retrospective review of 37 CF patients treated for M. abscessus respiratory infection at a single center from 2006 to 2014. Outcomes included change in FEV1 at 30, 60, 90, 180, and 365 days after treatment and clearance of M. abscessus from sputum cultures.Results
Lung function was significantly improved after 30 and 60 days of treatment, but not at later time points. Gains were inversely related to starting lung function. Antibiotic choices did not influence outcomes except for greater clearance with clarithromycin.Conclusions
Treatment of M. abscessus resulted in short term improvement in lung function that is inversely related to pre-treatment FEV1. 相似文献14.
Samuel T. Montgomery A. Susanne Dittrich Luke W. Garratt Lidija Turkovic Dario L. Frey Stephen M. Stick Marcus A. Mall Anthony Kicic 《Journal of cystic fibrosis》2018,17(6):715-722
Background
Little is known about the role of interleukin (IL)-1 in the pathogenesis of cystic fibrosis (CF) lung disease. This study investigated the relationship between IL-1 signalling, neutrophilic inflammation and structural lung changes in children with CF.Methods
Bronchoalveolar lavage fluid (BALf) from 102 children with CF were used to determine IL-1α, IL-1β, IL-8 levels and neutrophil elastase (NE) activity, which were then correlated to structural lung changes observed on chest computed tomography (CT) scans.Results
IL-1α and IL-1β were detectable in BAL in absence of infection, increased in the presence of bacterial infection and correlated with IL-8 (p?<?0.0001), neutrophils (p?<?0.0001) and NE activity (p?<?0.01 and p?<?0.001). IL-1α had the strongest association with structural lung disease (p <?0.01) in the absence of infection (uninfected: p?<?0.01 vs. infected: p?=?0.122).Conclusion
Our data associates IL-1α with early structural lung damage in CF and suggests this pathway as a novel anti-inflammatory target. 相似文献15.
Kathryn A. Ramsey Caroline McGirr Stephen M. Stick Graham L. Hall Shannon J. Simpson 《Journal of cystic fibrosis》2017,16(6):713-718
Background
We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease.Methods
Multiple breath washout (MBW) was performed in seated and supine postures in 25 healthy children and 21 children with CF. Children with CF also underwent a chest CT scan. Functional residual capacity (FRC), lung clearance index (LCI) and moment ratios were calculated from the MBW test. CT scans were evaluated for CF-related structural lung disease.Results
FRC was lower in the supine than in the seated posture, whereas LCI was higher in the supine than in the seated posture. In children with CF, associations between LCI and the extent of structural lung disease were stronger when performed in the supine posture.Conclusions
Body posture influences lung volumes and ventilation distribution in both healthy children and children with CF. MBW testing in the supine posture strengthened associations with structural lung damage. 相似文献16.
Julia Seyfarth Sutharsan Sivagurunathan Sarah Ricken Gerhard Weinreich Laura Olbrich Christian Taube Ertan Mayatepek Dirk Schramm Marc Jacobsen 《Journal of cystic fibrosis》2019,18(1):71-77
Background
Patients with cystic fibrosis (CF) are highly susceptible to infection and colonization of pulmonary epithelia. Repeated and chronic infections may affect disease course and efficacy of host immune protection. Higher Interleukin (IL)-7 serum levels, indicating impaired T-cell response to IL-7, have been described for chronic viral and mycobacterial infections.Methods
Time course measures of IL-7 serum concentrations in patients with CF (n?=?164; n?=?78 for the second time point) and healthy controls (n?=?60) were done. CF patients were characterized for disease severity parameters as well as infection status and association with IL-7 serum levels was determined.Results
CF patients had significantly higher IL-7 serum concentrations as compared to healthy controls (9.79?pg/ml, IQR 6.76–13.6 versus 4.55?pg/ml, IQR 2.76–9.51, p?<?.001). IL-7 serum levels were negatively correlated with individual CF patient's BMI (r?=??0.19, p?=?.021) and a tendency of increased IL-7 levels in Staphylococcus aureus infected CF patients was found. Linear regression of multiple parameters revealed significant negative correlation of FEV1%pred with IL-7 serum concentrations in patients with CF (ß-coefficient: ?0.04, 95% confidence interval [?0.08; ?0.003], p?=?.034). Time course analyses after 1?year +/? 6?months showed increased IL-7 serum levels (time point 1:9.26?pg/ml, IQR 6.94–13.12 time point 2:10.86?pg/ml, IQR 9.14–14.76, p?=?.016) that correlated negatively with decreased FEV1%pred during CF disease course.Conclusions
High IL-7 serum levels were found in CF patients and correlated with impaired lung function during CF disease course. As a candidate biomarker of T-cell dysfunction, higher IL-7 serum level may also indicate worsened immune competence of patients with CF. 相似文献17.
Jeff R. Crosby Chenguang Zhao Chong Jiang Dong Bai Melanie Katz Sarah Greenlee Hiroshi Kawabe Michael McCaleb Daniela Rotin Shuling Guo Brett P. Monia 《Journal of cystic fibrosis》2017,16(6):671-680
Background
Epithelial sodium channel (ENaC, Scnn1) hyperactivity in the lung leads to airway surface dehydration and mucus accumulation in cystic fibrosis (CF) patients and in mice with CF-like lung disease.Methods
We identified several potent ENaC specific antisense oligonucleotides (ASOs) and tested them by inhalation in mouse models of CF-like lung disease.Results
The inhaled ASOs distributed into lung airway epithelial cells and decreased ENaC expression by inducing RNase H1-dependent degradation of the targeted Scnn1a mRNA. Aerosol delivered ENaC ASO down-regulated mucus marker expression and ameliorated goblet cell metaplasia, inflammation, and airway hyper-responsiveness. Lack of systemic activity of ASOs delivered via the aerosol route ensures the safety of this approach.Conclusions
Our results demonstrate that antisense inhibition of ENaC in airway epithelial cells could be an effective and safe approach for the prevention and reversal of lung symptoms in CF and potentially other inflammatory diseases of the lung. 相似文献18.
Adegboyega Timothy Adewale Steven M. Rowe George M. Solomon 《Journal of cystic fibrosis》2019,18(2):e11-e13
Purpose
To raise awareness of colocolonic intussusception as a gastrointestinal complication of CF mimicking distal intestinal obstruction syndrome (DIOS) and discuss risk of recurrence.Case summary
A 33-year-old Caucasian male with cystic fibrosis presented with an acute abdomen diagnosed via imaging as colocolonic intussusception. He was managed with fluid replacement therapy and polyethylene glycol. He was re-admitted due to recurrence likely secondary to recurrent constipation and development of a fecalith. Surgery was contraindicated due to absence of tissue ischemia or necrosis.Discussion
Several possible etiological factors have been described, especially some that tend to occur within the context of CF disease, such as DIOS and PERT, and symptoms of colocolonic intussusception are similar to those of other causes of an acute abdomen but distinguishable by advanced imaging modalities. Due to risk of recurrence, an etiology of intussusception should be sought.Conclusion
Colo-colonic intussusception is a rare cause of an acute abdomen in the adult Cystic Fibrosis (CF) patient and may be associated with underlying constipation or presence of a fecalith. 相似文献19.
Ana C. Blanchard Ashley M. Rooney Yvonne Yau Yu Zhang Patrick J. Stapleton Eric Horton Michelle Klingel Sanja Stanojevic Felix Ratjen Bryan Coburn Valerie Waters 《Journal of cystic fibrosis》2018,17(6):723-728
Background
Infection with Pseudomonas aeruginosa (Pa) with a chronic phenotype is associated with antibiotic eradication therapy (AET) failure. Our objective was to determine whether higher levels of Pa (detected using qPCR) prior to culture positivity were associated with AET failure in pediatric CF patients.Methods
Pa-specific qPCR was performed on stored sputa prior to culture positivity in pediatric CF patients with new-onset culture-positive Pa infections undergoing AET with a 28-day course of tobramycin-inhaled solution (TIS). DNA concentrations were compared in patients in whom AET was successful (Eradicated) to those with persistently positive sputum cultures (Persistent).Results
Forty-seven patients were included. AET was successful in 32 cases (68%), but failed in 15 cases (32%). Median sputum Pa-specific DNA concentration preceding the positive sputum culture was 2.2?×?10?6?μg/mL in Eradicated cases compared to 3?×?10?5?μg/mL in Persistent cases (p?=?0.14). There was no significant difference in DNA concentration in the last sputum sample prior to culture positivity, nor in maximal DNA values. There was also no difference in sputum Pa DNA concentrations in patients who had a mucoid (compared to non-mucoid) Pa infection.Conclusions
Pediatric CF patients with new-onset Pa infections have detectable Pa-specific DNA in the year preceding a positive culture, however, there is no significant difference in Pa DNA concentrations between patients in whom AET is successful compared to those in whom it fails. Therefore, early molecular detection of Pa may not lead to improved eradication success rates. 相似文献20.
Tobias G.P. Engel Lydie Slabbers Carmen de Jong Willem J.G. Melchers Ferry Hagen Paul E. Verweij Peter Merkus Jacques F. Meis 《Journal of cystic fibrosis》2019,18(2):221-226