Alternative Indexes to Estimate the Functional Capacity From the 6-Minute Walk Test in Children and Adolescents With Cystic Fibrosis.

Respir Care. 2017 Jan 03;:

Authors: Okuro RT, de Oliveira Ribeiro MA, Ribeiro JD, Minsky RC, Schivinski CI

Abstract
BACKGROUND: Cystic fibrosis is a multi-systemic disease related to reduced functional capacity. The distance covered in the 6-min walk test (6MWT) has been known to assess functional capacity, but little is known about other indexes that can be derived. We sought to compare the performance during the 6MWT and the estimated indexes of functional capacity from the 6MWT between subjects with cystic fibrosis (CF) and healthy individuals as well as to assess the relationship among these indexes and disease severity, pulmonary function, and nutritional status in CF.
METHODS: This cross-sectional study was carried out at a university referral center for CF. It included a group of 55 non-oxygen-dependent CF subjects (CF group) with no acute pulmonary exacerbations and a group of 185 healthy controls (control group). All subjects were submitted to 6MWT and anthropometrics measurements.
RESULTS: Regarding performance during the 6MWT, the mean values of work, physiological cost index, average velocity, and 6-min walk distance (6MWD) were significantly lower in the CF group than in the control group (work: 21,690.58 ± 10,427.77 vs 26,057.51 ± 11,228.49 m × kg [P = .007]; physiological cost index: 0.31 ± 0.19 vs 0.37 ± 0.17; average velocity: 94.71 ± 12.89 vs 104.55 ± 9.13 m/min [P < .001]; and 6MWD: 568.02 ± 76.31 m versus 627.54 ± 54.81 m [P < .001]). Subjects with less severe CF had higher 6MWD, work, and average velocity during the 6MWT, compared with subjects with more severe CF (P = .008, P = .012, and P = .007, respectively). There was a correlation between 6MWD, work, average velocity, and disease severity and pulmonary function.
CONCLUSIONS: Considering the importance of standard measure (6MWD) the in 6MWT, alternative indexes can be useful as complementary outcomes and to provide a better understanding of limiting factors of exercise response in children and adolescents with CF.

PMID: 28049743 [PubMed - as supplied by publisher]

Cystic Fibrosis Mutation Detection with SPR Biosensor in Real Samples via Multiple Surfaces Binding Method.

Comb Chem High Throughput Screen. 2017 Jan 03;

Authors: Kayran YU, Ozkan-Ariksoysal D, Topkaya SN, Kaymaz BT, Can BK

Abstract
Surface Plasmon Resonance (SPR) based biosensor system was developed for the detection of Delta F508 (DF508del) Cystic Fibrosis (CF) mutation in both synthetic and real samples. In order to provide an effective hybridization between probe and the Polymerase Chain Reaction (PCR) amplicons (target), streptavidin was bound to the surface and biotin-tag probe was sent to the streptavidin-coated surface. For the target preparation, blood samples were collected from the patients who suffer from CF. Following the DNA isolation; samples were amplified with PCR with biotin-tag. Before sending the biotin-tag PCR amplicons onto the modified surface, amplicons were also interacted with the helper oligonucleotides to prevent re-annealing of the denatured DNA strands. This kind of 'multiple surface binding' method helps increasing of the sensitivity of the detection. The limit of detection(S/N= 3) was calculated as 12.24 pico-mole/ml for PCR-like synthetic long target sequence and 13x105 molecules for real samples in less than half hour. Using the both biotin-tag probe and the helper oligonucleotides together, hybridization was obtained much more efficiently than traditional denaturation protocols for real samples and biotin-free hybridization detection. To the best of our knowledge, the procedure described in this study is one of the simplest, rapid and sensitive methods for CF mutation detection with SPR based biosensor system in real samples.

PMID: 28049404 [PubMed - as supplied by publisher]

Genetic discrimination lawsuit raises broader concerns about testing, privacy: Case involves middle school student impacted by results of genetic screening test as newborn.

Am J Med Genet A. 2016 May;170A(5):1111-2

Authors:

PMID: 27075500 [PubMed - indexed for MEDLINE]

Inhibition of airway surface fluid absorption by cholinergic stimulation.

Sci Rep. 2016 Feb 05;6:20735

Authors: Joo NS, Krouse ME, Choi JY, Cho HJ, Wine JJ

Abstract
In upper airways airway surface liquid (ASL) depth and clearance rates are both increased by fluid secretion. Secretion is opposed by fluid absorption, mainly via the epithelial sodium channel, ENaC. In static systems, increased fluid depth activates ENaC and decreased depth inhibits it, suggesting that secretion indirectly activates ENaC to reduce ASL depth. We propose an alternate mechanism in which cholinergic input, which causes copious airway gland secretion, also inhibits ENaC-mediated absorption. The conjoint action accelerates clearance, and the increased transport of mucus out of the airways restores ASL depth while cleansing the airways. We were intrigued by early reports of cholinergic inhibition of absorption by airways in some species. To reinvestigate this phenomenon, we studied inward short-circuit currents (Isc) in tracheal mucosa from human, sheep, pig, ferret, and rabbit and in two types of cultured cells. Basal Isc was inhibited 20-70% by the ENaC inhibitor, benzamil. Long-lasting inhibition of ENaC-dependent Isc was also produced by basolateral carbachol in all preparations except rabbit and the H441 cell line. Atropine inhibition produced a slow recovery or prevented inhibition if added before carbachol. The mechanism for inhibition was not determined and is most likely multi-factorial. However, its physiological significance is expected to be increased mucus clearance rates in cholinergically stimulated airways.

PMID: 26846701 [PubMed - indexed for MEDLINE]

11 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2017/01/04

PubMed comprises more than 24 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

Beta-lactamase-producing Pseudomonas aeruginosa: Phenotypic characteristics and molecular identification of virulence genes.

J Chin Med Assoc. 2016 Dec 27;:

Authors: Ullah W, Qasim M, Rahman H, Jie Y, Muhammad N

Abstract
BACKGROUND: Pseudomonas aeruginosa causes common infections in immunocompromised and cystic fibrosis patients. However, drug resistance capability and release of virulence factors play a key role in bacterial pathogenicity.
METHODS: Beta-lactamase-producing clinical isolates of P. aeruginosa were screened for biofilm formation and pigment production. Subsequently, all the isolates were subjected to the detection of six virulence genes (OprI, OprL, LasB, PlcH, ExoS, and ToxA).
RESULTS: Among beta-lactamase-producing isolates (n=54), about 85.18% (n=46) were biofilm producers. Pigment production was observed in 92.59% (n=50) isolates. Clinical samples were subsequently screened for detection of virulence factors. Among them, 40.74% (n=22) isolates were found to be OprI positive, while 29.62% (n=16) were OprL producers. In the case of LasB and PlcH, 24% (n=13) and 18.5% (n=10) isolates produced these virulence genes, respectively. Among the isolates, 37.03% (n=20) and 33.33% (n=18) expressed virulence factors ExoS and ToxA, respectively. Furthermore, 42.59% (n=23) isolates coproduced more than one type of virulence factors.
CONCLUSION: In the current study, pigment display, biofilm formation, and virulence genes were detected in P. aeruginosa clinical isolates. Such factors could be crucial in the development of drug resistance.

PMID: 28038909 [PubMed - as supplied by publisher]

6 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2016/12/31

PubMed comprises more than 24 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

Perception of first respiratory infection with Pseudomonas aeruginosa by people with cystic fibrosis and those close to them: an online qualitative study.

BMJ Open. 2016 Dec 28;6(12):e012303

Authors: Palser SC, Rayner OC, Leighton PA, Smyth AR

Abstract
BACKGROUND: People with cystic fibrosis (CF) are susceptible to respiratory infection with Pseudomonas aeruginosa (PA), which may become chronic if initial eradication fails. Environmental acquisition and person-to-person transmission can occur. Respiratory PA infection is associated with increased mortality and more hospitalisations. This may cause patients and families anxiety and lead them to adopt preventive measures which may be ineffectual and intrusive. It is not possible to hold a conventional focus group to explore these issues because people with CF cannot meet together due to the risk of cross-infection.
OBJECTIVE: To explore the perceptions of first respiratory infection with PA in people with CF and those close to them.
DESIGN: We designed an online survey, to maximise accessibility and avoid the risk of cross-infection. This established the respondent's relationship with CF, asked 3 open questions about perceptions of PA and a final question about the prioritisation of research. Responses were analysed using a structured, iterative process. We identified keywords, analysed these incontext and derived key themes.
SETTING: Promotion through social media allowed respondents from any country to participate.
PARTICIPANTS: People with CF and those close to them.
RESULTS: Responses were received from 393 people, including 266 parents and 97 people with CF. The key themes were the emotional burden of PA (fear in particular); the burden of treatment PA entails and the need for accurate knowledge about PA.
CONCLUSIONS: Lack of knowledge and the health beliefs of individuals may promote fear of infection and inappropriate avoidance measures. Uncertainty about the implications of PA infection and the treatment required may cause anxiety. Healthcare professionals should provide clear information about how PA might be acquired and the treatment necessary, making clear the limitations of current understanding and acknowledging health beliefs.

PMID: 28031208 [PubMed - in process]

Thromboelastogram as a Tool to Predict Hypercoagulability in Children With Cystic Fibrosis.

Clin Appl Thromb Hemost. 2016 Jan 01;:1076029616683045

Authors: Anıl H, Kılıç Yıldırım G, Harmancı K, Bozkurt Turhan A, Akay OM, Bör Ö, Aydoğdu S, Kocak A

Abstract
Increased thrombophilic tendency in patients with cystic fibrosis (CF) has recently been reported. The determinants of thrombosis in children with CF remain largely unknown. Our aim in this study was to evaluate the thromboelastography (TEG) profile of children with CF through ROTEM (whole blood rotation thromboelastometry). Nineteen patients with CF and 20 controls were included in the study. Whole blood count, prothrombin time, activated prothrombin time, fibrinogen, d-dimer levels, and ROTEM assays (INTEM, EXTEM) were performed. Clotting time, clot formation time (CFT), and maximum clot firmness (MCF) were determined by INTEM and EXTEM analysis. In INTEM assay, MCF ( P = .001) value was significantly increased and CFT ( P = .031) value was decreased in patients with CF compared with those of the control group. In the EXTEM assay, there was a similar significant increase in MCF ( P = .023) value in patients with CF compared with that of the control group. There was a significant positive correlation between fibrinogen levels and MCF in EXTEM ( r = .72) and INTEM ( r = .76) assays, whereas there was a negative correlation with CFT in EXTEM ( r = -.61) and INTEM ( r = -.67). The results of our study indicated that TEG profiles in patients with CF were more hypercoagulable compared with those of the control group.

PMID: 28030968 [PubMed - as supplied by publisher]

HMGB1 levels in children with atopic eczema/dermatitis syndrome (AEDS).

Pediatr Allergy Immunol. 2016 Feb;27(1):99-102

Authors: Cuppari C, Manti S, Salpietro A, Valenti S, Capizzi A, Arrigo T, Salpietro C, Leonardi S

PMID: 26388323 [PubMed - indexed for MEDLINE]

A High Level of Soluble CD40L Is Associated with P. aeruginosa Infection in Patients with Cystic Fibrosis.

PLoS One. 2016;11(12):e0168819

Authors: Bustamante AE, Jaime-Pérez JC, Cordero-Pérez P, Galindo-Rodríguez G, Muñoz-Espinosa LE, Villarreal-Villarreal CD, Mercado-Longoria R

Abstract
OBJECTIVE: The aim of this study was to evaluate whether the concentration of sCD40L, a product of platelet activation, correlates with the presence of Pseudomonas aeruginosa in the airway of patients with cystic fibrosis (CF) and to determine its possible clinical association.
METHODS: Sixty patients with CF, ranging in age from 2 months to 36 years, were studied. The demographics, cystic fibrosis transmembrane conductance regulator (CFTR) genotype, spirometry measurements, radiographic and tomographic scans, platelet count in peripheral blood, sCD40L, IL-6, TNF-α and ICAM1 data were collected. Infection-colonization of the airway was evaluated using sputum and throat swab cultures; the levels of anti-Pseudomonas aeruginosa antibodies (Anti-PaAb) were evaluated.
RESULTS: Patients with CF and chronic colonization had anti-PaAb values of 11.6 ± 2.1 ELISA units (EU) and sCD40L in plasma of 1530.9 ±1162.3 pg/mL; those with intermittent infection had 5.7 ± 2.7 EU and 2243.6 ± 1475.9 pg/mL; and those who were never infected had 3.46 ± 1.8 EU (p≤0.001) and 1008.1 ± 746.8 pg/mL (p≤0.01), respectively. The cutoff value of sCD40L of 1255 pg/mL was associated with an area under the ROC (receiver operating characteristic curve) of 0.84 (95% CI, 0.71 to 0.97), reflecting P. aeruginosa infection with a sensitivity of 73% and a specificity of 89%. Lung damage was determined using the Brasfield Score, the Bhalla Score, and spirometry (FVC%, FEV1%) and found to be significantly different among the groups (p≤0.001).
CONCLUSION: Circulating sCD40L levels are increased in patients with cystic fibrosis and P. aeruginosa infection. Soluble CD40L appears to reflect infection and provides a tool for monitoring the evolution of lung deterioration.

PMID: 28030642 [PubMed - in process]

Effects of Propidium Monoazide (PMA) Treatment on Mycobiome and Bacteriome Analysis of Cystic Fibrosis Airways during Exacerbation.

PLoS One. 2016;11(12):e0168860

Authors: Nguyen LD, Deschaght P, Merlin S, Loywick A, Audebert C, Van Daele S, Viscogliosi E, Vaneechoutte M, Delhaes L

Abstract
INTRODUCTION AND PURPOSE: Propidium monoazide (PMA)-pretreatment has increasingly been applied to remove the bias from dead or damaged cell artefacts, which could impact the microbiota analysis by high-throughput sequencing. Our study aimed to determine whether a PMA-pretreatment coupled with high-throughput sequencing analysis provides a different picture of the airway mycobiome and bacteriome.
RESULTS AND DISCUSSION: We compared deep-sequencing data of mycobiota and microbiota of 15 sputum samples from 5 cystic fibrosis (CF) patients with and without prior PMA-treatment of the DNA-extracts. PMA-pretreatment had no significant effect on the entire and abundant bacterial community (genera expressed as operational taxonomic units (OTUs) with a relative abundance greater than or equal to 1%), but caused a significant difference in the intermediate community (less than 1%) when analyzing the alpha biodiversity Simpson index (p = 0.03). Regarding PMA impact on the airway mycobiota evaluated for the first time here; no significant differences in alpha diversity indexes between PMA-treated and untreated samples were observed. Regarding beta diversity analysis, the intermediate communities also differed more dramatically than the total and abundant ones when studying both mycobiome and bacteriome. Our results showed that only the intermediate (or low abundance) population diversity is impacted by PMA-treatment, and therefore that abundant taxa are mostly viable during acute exacerbation in CF. Given such a cumbersome protocol (PMA-pretreatment coupled with high-throughput sequencing), we discuss its potential interest within the follow-up of CF patients. Further studies using PMA-pretreatment are warranted to improve our "omic" knowledge of the CF airways.

PMID: 28030619 [PubMed - in process]

[Improved lung function in cystic fibrosis using mechanical insufflation-exsufflation].

An Pediatr (Barc). 2016 Oct 28;:

Authors: Fuentes LA, Caro P, Garcia-Ruiz AJ, Muñoz Gómez G, Martín-Montañez E

PMID: 28029527 [PubMed - as supplied by publisher]

Nutrition and Growth in Chronic Disease.

World Rev Nutr Diet. 2016;114:84-102

Authors: Hartman C, Shamir R

PMID: 26906408 [PubMed - indexed for MEDLINE]

Impact of azithromycin on the clinical and antimicrobial effectiveness of tobramycin in the treatment of cystic fibrosis.

J Cyst Fibros. 2016 Dec 23;:

Authors: Nichols DP, Happoldt CL, Bratcher PE, Caceres SM, Chmiel JF, Malcolm KC, Saavedra MT, Saiman L, Taylor-Cousar JL, Nick JA

Abstract
BACKGROUND: Concomitant use of oral azithromycin and inhaled tobramycin occurs in approximately half of US cystic fibrosis (CF) patients. Recent data suggest that this combination may be antagonistic.
METHODS: Test the hypothesis that azithromycin reduces the clinical benefits of tobramycin by analyses of clinical trial data, in vitro modeling of P. aeruginosa antibiotic killing, and regulation of the MexXY efflux pump.
RESULTS: Ongoing administration of azithromycin associates with reduced ability of inhaled tobramycin, as compared with aztreonam, to improve lung function and quality of life in a completed clinical trial. In users of azithromycin FEV1 (L) increased 0.8% during a 4-week period of inhaled tobramycin and an additional 6.4% during a subsequent 4-week period of inhaled aztreonam (P<0.005). CFQ-R respiratory symptom score decreased 1.8 points during inhaled tobramycin and increased 8.3 points during subsequent inhaled aztreonam (P<0.001). A smaller number of trial participants not using azithromycin had similar improvement in lung function and quality of life scores during inhaled tobramycin and inhaled aztreonam. In vitro, azithromycin selectively reduced the bactericidal effects tobramycin in cultures of clinical strains of P. aeruginosa, while up regulating antibiotic resistance through MexXY efflux.
CONCLUSIONS: Azithromycin appears capable of reducing the antimicrobial benefits of tobramycin by inducing adaptive bacterial stress responses in P. aeruginosa, suggesting that these medications together may not be optimal chronic therapy for at least some patients.

PMID: 28025037 [PubMed - as supplied by publisher]

6 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2016/12/27

PubMed comprises more than 24 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

Severe disease in Cystic Fibrosis and fecal calprotectin levels.

Immunobiology. 2016 Nov 13;:

Authors: Parisi GF, Papale M, Rotolo N, Aloisio D, Tardino L, Scuderi MG, Di Benedetto V, Nenna R, Midulla F, Leonardi S

Abstract
Fecal calprotectin (FC) is used to asses the presence of intestinal inflammation also in patients with Cystic Fibrosis (CF) and recent studies showed a correlation between bowel and lung disease in these patients. The aim of this study was to analyze the levels of FC in CF and correlate them with different phenotypes of disease. We enrolled a cohort of 54 CF patients and 50 healthy controls. In these patients, calprotectin has been assayed on a stools sample using an ELISA kit. In all patients we analyzed, FC levels were elevated above the cut-off value and significantly higher than in healthy controls. Among CF patients, FC was significantly higher in patients older than 18 years, with pancreatic insufficiency, underweight status, Pseudomonas Aeruginosa airways colonization, CF-related diabetes mellitus, reduced lung function, or high number of pulmonary exacerbations. These results suggest that in patients with CF, FC levels are not only influenced by the CF enteropathy but also by the severity of the genetic disease. Since we found higher FC levels in patients with a severe phenotype (P. Aeruginosa airways colonization, FEV1<50% of predicted, pancreatic insufficiency, underweight status,) we suggest that this marker could be useful to monitor longitudinally a clinical worsening.

PMID: 28012584 [PubMed - as supplied by publisher]

Changes in the inner ear structures in cystic fibrosis patients.

Int J Pediatr Otorhinolaryngol. 2017 Jan;92:108-114

Authors: Pauna HF, Monsanto RC, Kurata N, Paparella MM, Cureoglu S

Abstract
OBJECTIVE: Although prolonged use of antibiotics is very common in cystic fibrosis (CF) patients, no studies have assessed the changes in both cochlear and peripheral vestibular systems in this population.
METHODS: We used human temporal bones to analyze the density of vestibular dark, transitional, and hair cells in specimens from CF patients who were exposed to several types of antibiotics, as compared with specimens from an age-matched control group with no history of ear disease or antibiotic use. Additionally, we analyzed the changes in the elements of the cochlea (hair cells, spiral ganglion neurons, and the area of the stria vascularis). Data was gathered using differential interference contrast microscopy and light microscopy.
RESULTS: In the CF group, 83% of patients were exposed to some ototoxic drugs, such as aminoglycosides. As compared with the control group, the density of both type I and type II vestibular hair cells was significantly lower in all structures analyzed; the number of dark cells was significantly lower in the lateral and posterior semicircular canals. We noted a trend toward a lower number of both inner and outer cochlear hair cells at all turns of the cochlea. The number of spiral ganglion neurons in Rosenthal's canal at the apical turn of the cochlea was significantly lower; furthermore, the area of the stria vascularis at the apical turn of the cochlea was significantly smaller.
CONCLUSIONS: Deterioration of cochlear and vestibular structures in CF patients might be related to their exposure to ototoxic antibiotics. Well-designed case-control studies are necessary to rule out the effect of CF itself.

PMID: 28012509 [PubMed - in process]

Functional defect of variants in the adenosine triphosphate-binding sites of ABCB4 and their rescue by the cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor (VX-770).

Hepatology. 2016 Nov 05;:

Authors: Delaunay JL, Bruneau A, Hoffmann B, Durand-Schneider AM, Barbu V, Jacquemin E, Maurice M, Housset C, Callebaut I, Aït-Slimane T

Abstract
ABCB4 (MDR3) is an adenosine triphosphate (ATP)-binding cassette (ABC) transporter expressed at the canalicular membrane of hepatocytes, where it mediates phosphatidylcholine (PC) secretion. Variations in the ABCB4 gene are responsible for several biliary diseases, including progressive familial intrahepatic cholestasis type 3 (PFIC3), a rare disease that can be lethal in the absence of liver transplantation. In this study, we investigated the effect and potential rescue of ABCB4 missense variations that reside in the highly conserved motifs of ABC transporters, involved in ATP binding. Five disease-causing variations in these motifs have been identified in ABCB4 (G535D, G536R, S1076C, S1176L, and G1178S), three of which are homologous to the gating mutations of cystic fibrosis transmembrane conductance regulator (CFTR or ABCC7; i.e., G551D, S1251N, and G1349D), that were previously shown to be function defective and corrected by ivacaftor (VX-770; Kalydeco), a clinically approved CFTR potentiator. Three-dimensional structural modeling predicted that all five ABCB4 variants would disrupt critical interactions in the binding of ATP and thereby impair ATP-induced nucleotide-binding domain dimerization and ABCB4 function. This prediction was confirmed by expression in cell models, which showed that the ABCB4 mutants were normally processed and targeted to the plasma membrane, whereas their PC secretion activity was dramatically decreased. As also hypothesized on the basis of molecular modeling, PC secretion activity of the mutants was rescued by the CFTR potentiator, ivacaftor (VX-770).
CONCLUSION: Disease-causing variations in the ATP-binding sites of ABCB4 cause defects in PC secretion, which can be rescued by ivacaftor. These results provide the first experimental evidence that ivacaftor is a potential therapy for selected patients who harbor mutations in the ATP-binding sites of ABCB4. (Hepatology 2016).

PMID: 28012258 [PubMed - as supplied by publisher]

Transplant center volume and outcomes in lung transplantation for cystic fibrosis.

Transpl Int. 2016 Dec 24;:

Authors: Hayes D, Sweet SC, Benden C, Kopp BT, Goldfarb SB, Visner GA, Mallory GB, Tobias JD, Tumin D

Abstract
INTRODUCTION: Transplant volume represents lung transplant (LTx) expertise and predicts outcomes, so we sought to determine outcomes related to center volumes in CF.
METHODS: United Network for Organ Sharing data were queried for CF patients receiving bilateral LTx from 2005-2015. Multivariable Cox regression was used to model survival to 1 year and long-term (>1 year) survival, conditional on surviving at least 1 year.
RESULTS: 2,025 patients and 67 centers were included in the analysis. The median annual LTx volumes were 3 in CF (interquartile range [IQR]: 2, 6), and 17 in non-CF (IQR: 8, 33). Multivariable Cox regression in cases with complete data and surviving at least 1 year (n=1,510) demonstrated that greater annual CF LTx volume (HR per 10 LTx=0.66; 95% CI: 0.49, 0.89; p=0.006) but not greater non-CF LTx volume (HR=1.00; 95% CI: 0.96, 1.05; p=0.844) was associated with improved long-term survival in LTx recipients with CF. A Wald interaction test confirmed that CF LTx volume was more strongly associated with long-term outcomes than non-CF LTx volume (p=0.012). Center volume was not associated with 1-year survival.
CONCLUSIONS: CF-specific expertise predicted improved long-term outcomes of LTx for CF, whereas general LTx expertise was unassociated with CF patients' survival. This article is protected by copyright. All rights reserved.

PMID: 28012223 [PubMed - as supplied by publisher]