16 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 2019/11/13

PubMed comprises more than millions of 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.

Bilateral lung transplantation in cystic fibrosis with hepatitis C infection - a study of two cases.

Kardiochir Torakochirurgia Pol. 2019 Oct;16(3):133-135

Authors: Ruszel N, Kubisa B, Lisowski P, Piotrowska M, Kubisa MJ, Brykczyński M, Wojtyś M, Pieróg J, Czarnecka M, Wójcik J, Wójcik N, Sielicki P, Bielewicz M, Grodzki T

PMID: 31708987 [PubMed]

Pancreatic Malnutrition in Children.

Pediatr Ann. 2019 Nov 01;48(11):e441-e447

Authors: Normatov I, Sentongo T

Abstract
Exocrine pancreatic insufficiency in children can lead to lifelong complications related to malnutrition and poor growth. The clinical presentation can be subtle in the early stages of insufficiency as the large functional capacity of the pancreas is gradually lost. The pediatrician plays a crucial role in the early identification of these children to ensure a timely referral so that a diagnosis can be made and therapy initiated. Early nutritional therapy allows for prevention and correction of deficiencies, which leads to improved outcomes and survival. When insufficiency is suspected, the workup should start with an indirect test of exocrine pancreatic function, such as fecal elastase, to establish the diagnosis. Once a diagnosis is established, further testing to delineate the etiology should be pursued, with cystic fibrosis being high on the differential list and assessed for with a sweat test. Assessment of anthropometry at every visit is key, as is monitoring of laboratory parameters and physical examination findings that are suggestive of malabsorption and malnutrition. The mainstay of management is administration of exogenous pancreatic enzymes to facilitate digestion and absorption. [Pediatr Ann. 2019;48(11):e441-e447.].

PMID: 31710363 [PubMed - in process]

Cystic fibrosis in Turkey: First data from the national registry.

Pediatr Pulmonol. 2019 Nov 11;:

Authors: Dogru D, Çakır E, Şişmanlar T, Çobanoğlu N, Pekcan S, Cinel G, Yalçın E, Kiper N, Şen V, S Şen H, Ercan Ö, Keskin Ö, B Eltan S, Al Shadfan LM, Yazan H, Altıntaş DU, Şaşihüseyinoğlu Ş, Sapan N, Çekiç Ş, Çokuğraş H, A Kılınç A, R Gürsoy T, Aslan AT, Bingöl A, Başaran AE, Özdemir A, Köse M, Hangül M, Emiralioğlu N, Tuğcu G, Yüksel H, Yılmaz Ö, Orhan F, Gayretli Aydın ZG, Topal E, Tamay Z, Süleyman A, Can D, Bal CM, Çaltepe G, Özçelik U

Abstract
BACKGROUND: Cystic fibrosis (CF) care has been implemented in Turkey for a long time; however, there had been no patient registry. For this purpose, the Turkish National CF Registry was established. We present the first results of registry using data collected in 2017.
METHODS: The data were collected using a data-entry software system, which was accessed from the internet. Demographic and annually recorded data consisted of 15 and 79 variables, respectively.
RESULTS: There were 1170 patients registered from 23 centers; the estimated coverage rate was 30%. The median age at diagnosis was 1.7 years (median current age: 7.3 years); 51 (4.6%) patients were aged over 18 years. Among 293 patients who were under 3 years of age, 240 patients (81.9%) were diagnosed through newborn screening. Meconium ileus was detected in 65 (5.5%) patients. Genotyping was performed in 978 (87.4%) patients and 246 (25.2%) patients' mutations were unidentified. The most common mutation was deltaF508 with an allelic frequency of 28%, followed by N1303K (4.9%). The median FEV1% predicted was 86. Chronic colonization with Pseudomonas aeruginosa was seen in 245 patients. The most common complication was pseudo-Bartter syndrome in 120 patients. The median age of death was 13.5 years in a total of 15 patients.
CONCLUSIONS: Low coverage rate, lack of genotyping, unidentified mutations, and missing data of lung functions are some of our greatest challenges. Including data of all centers and reducing missing data will provide more accurate data and help to improve the CF care in Turkey in the future.

PMID: 31710166 [PubMed - as supplied by publisher]

[Investigation of role of anaerobic bacteria in cystic fibrosis patients].

Tuberk Toraks. 2019 Sep;67(3):151-161

Authors: Doğan Ö, Tunçkanat F, Cinel G, Şener B, Özçelik HU, Yalçın EE, Doğru Ersöz D, Kiper EN

Abstract
Introduction: Repetitive pulmonary infections are the main cause of morbidity and mortality in cystic fibrosis (CF) patients. In recent years, non-culture dependent metagenomic studies showed complex dynamics of the pulmonary environment of CF patients and pointed out the importance of anaerobic bacteria. Molecular-based studies indicate that anaerobic bacteria can be found more than aerobic or facultative anaerobic bacteria in CF lung environment. However, limited number of studies are far away to clarify the importance of anaerobic bacteria in CF pulmonary disease.
Materials and Methods: The aim of this study was to evaluate the role of anaerobic bacteria in CF patients admitted to Hacettepe University, Pediatric Respiratory Diseases Department, by using quantitative culture method for both aerobic and anaerobic bacteria. Anaerobic bacteria were identified by conventional and semi-automated methods. Antibiotic susceptibilities were performed by agar dilution method.
Result: Seventy-seven anaerobic bacteria were isolated from 35 (81.4%) of 43 patients. The total count of anaerobes and facultative bacteria (mean 16 x 106), was higher than aerobes and facultative bacteria (mean 14.1 x 106). If anaerobe culture were not performed merely 63.65% of all species could be obtained. In patients whose samples yielded intermediate or high numbers of PMNLs, significantly more obligate anaerobic bacteria were isolated (p= 0.046). Patients older than 18 years were colonized with higher number of anaerobic bacteria. Susceptibilities of 72 isolates out of 77, against ampicillin, sulbactam-ampicillin, piperacillin, piperacillin-tazobactam, moxifloxacin, metronidazole, imipenem, and clindamycin were also evaluated. Clindamycin was found to be the least effective antibiotic among all. None of the isolates was resistant to imipenem.
Conclusions: This is the first study to show the role and importance of anaerobic bacteria in CF patients in our country. The resistance rates in anaerobic bacteria isolated from CF patients is concerning. Therefore, intermittent anaerobic culture and follow-up of resistance rates will be helpful in the follow-up of these patients.

PMID: 31709946 [PubMed - in process]

A new role for heat shock factor 27 in the pathophysiology of Clostridium difficile toxin B.

Am J Physiol Gastrointest Liver Physiol. 2019 Nov 11;:

Authors: Yanda MK, Guggino WB, Cebotaru L

Abstract
Clostridium difficile (CD) is a common pathogen that causes severe gastrointestinal inflammatory diarrhea in patients undergoing antibiotic therapy. Its virulence derives from two toxins, toxin CD, A & B (TcdA and TcdB) (10). Among the prime candidates for CD colonization are patients with cystic fibrosis (CF), who are routinely treated with antibiotics and frequently hospitalized. Indeed, ~50% of CF patients are colonized with virulent forms of CD but do not exhibit diarrhea (7, 9, 61). We found that TcdB has global effects on colonic cells, including reducing the steady-state levels of sodium proton exchange regulatory factors (NHERF), reducing the levels of heat shock protein Hsp27, and increasing the fraction of total Hsp27 bound to the cystic fibro-sis transmembrane regulator (CFTR). Also, since some mutations in CFTR seem to be protective, we asked whether CFTR is a target of TcdB. We show here that TcdB increases the maturation of CFTR and transiently increases its function. These combined effects promote increased surface expression of CFTR, resulting in a transient increase in Cl- secretion. This increase is followed by a precipitous decline in both CFTR-dependent Cl- secretion and transepithelial resistance (TER), suggesting a breakdown in the epithelial cells' tight junctions. We also found that overexpressing Hsp27 reverses some of the deleterious effects of TcdB, in particular preserving TER, and therefore likely the maintenance of barrier function. Thus, our data suggest that Hsp27 plays a role in the diarrhea generated by CD infection and is a potential therapeutic target for treating this diarrhea.

PMID: 31709831 [PubMed - as supplied by publisher]

The environmental occurrence of Pseudomonas aeruginosa.

APMIS. 2019 Nov 10;:

Authors: Crone S, Vives-Flórez M, Kvich L, Saunders AM, Malone M, Nicolaisen MH, Martínez-García E, Rojas-Acosta C, Catalina Gomez-Puerto M, Calum H, Whiteley M, Kolter R, Bjarnsholt T

Abstract
Pseudomonas aeruginosa is generally described as ubiquitous in natural settings, such as soil and water. However, because anecdotal observations and published reports have questioned whether or not this description is true, we undertook a rigorous study using three methods to investigate the occurrence of P. aeruginosa: we investigated environmental samples, analyzed 16S rRNA data, and undertook a systematic review and meta-analysis of published data. The environmental sample screening identified P. aeruginosa as significantly associated with hydrocarbon and pesticide contaminated environments and feces, as compared to uncontaminated environments in which its prevalence was relatively low. The 16S rRNA data analysis showed that P. aeruginosa sequences were present in all habitats but were most abundant in samples from human and animals. Similarly, the meta-analysis revealed that samples obtained from environments with intense human contact had a higher prevalence of P. aeruginosa compared to those with less human contact. Thus, we found a clear tendency of P. aeruginosa to be present in places closely linked with human activity. Although P. aeruginosa may be ubiquitous in nature, it is usually scarce in pristine environments. Thus, we suggest that P. aeruginosa should be described as a bacterium largely found in locations associated with human activity.

PMID: 31709616 [PubMed - as supplied by publisher]

A rare frameshift variant in trans with the IVS9-5T allele of CFTR in a Chinese pedigree with congenital aplasia of vas deferens.

J Assist Reprod Genet. 2019 Nov 10;:

Authors: Ge B, Zhang M, Wang R, Wang D, Li T, Li H, Wang B

Abstract
PURPOSE: Congenital aplasia of vas deferens (CAVD) is an atypical form of cystic fibrosis (CF) and causes obstructive azoospermia and male infertility. Compound heterozygous variants of CFTR are the main cause of CAVD. However, most evidence comes from genetic screening of sporadic cases and little is from pedigree analysis. In this study, we performed analysis in a Chinese pedigree with two CAVD patients in order to determine the genetic cause of this familial disorder.
METHODS: In the present study, we performed whole-exome sequencing and co-segregation analysis in a Chinese pedigree involving two patients diagnosed with CAVD.
RESULTS: We identified a rare frameshift variant (NM_000492.3: c.50dupT;p.S18Qfs*27) and a frequent CBAVD-causing variant (IVS9-TG13-5T) in both patients. The frameshift variant introduced a premature termination codon and was not found in any public databases or reported in the literature. Co-segregation analysis confirmed these two variants were in compound heterozygous state. The other male members, who harbored the frameshift variant and benign IVS9-7T allele, did not have any typical clinical manifestations of CF or CAVD.
CONCLUSION: Our findings may broaden the mutation spectrum of CFTR in CAVD patients and provide more familial evidence that the combination of a mild variant and a severe variant in trans of CFTR can cause vas deferens malformation.

PMID: 31709488 [PubMed - as supplied by publisher]

Intranasal Peptide-Based FpvA-KLH Conjugate Vaccine Protects Mice From Pseudomonas aeruginosa Acute Murine Pneumonia.

Front Immunol. 2019;10:2497

Authors: Sen-Kilic E, Blackwood CB, Boehm DT, Witt WT, Malkowski AC, Bevere JR, Wong TY, Hall JM, Bradford SD, Varney ME, Damron FH, Barbier M

Abstract
Pseudomonas aeruginosa is an opportunistic pathogen causing acute and chronic respiratory infections associated with morbidity and mortality, especially in patients with cystic fibrosis. Vaccination against P. aeruginosa before colonization may be a solution against these infections and improve the quality of life of at-risk patients. To develop a vaccine against P. aeruginosa, we formulated a novel peptide-based P. aeruginosa subunit vaccine based on the extracellular regions of one of its major siderophore receptors, FpvA. We evaluated the effectiveness and immunogenicity of the FpvA peptides conjugated to keyhole limpet hemocyanin (KLH) with the adjuvant curdlan in a murine vaccination and challenge model. Immunization with the FpvA-KLH vaccine decreased the bacterial burden and lung edema after P. aeruginosa challenge. Vaccination with FpvA-KLH lead to antigen-specific IgG and IgM antibodies in sera, and IgA antibodies in lung supernatant. FpvA-KLH immunized mice had an increase in recruitment of CD11b+ dendritic cells as well as resident memory CD4+ T cells in the lungs compared to non-vaccinated challenged mice. Splenocytes isolated from vaccinated animals showed that the FpvA-KLH vaccine with the adjuvant curdlan induces antigen-specific IL-17 production and leads to a Th17 type of immune response. These results indicate that the intranasal FpvA-KLH conjugate vaccine can elicit both mucosal and systemic immune responses. These observations suggest that the intranasal peptide-based FpvA-KLH conjugate vaccine with curdlan is a potential vaccine candidate against P. aeruginosa pneumonia.

PMID: 31708925 [PubMed - in process]

Serum amyloid A: A potential biomarker of lung disorders.

Respir Investig. 2019 Nov 07;:

Authors: Vietri L, Fui A, Bergantini L, d'Alessandro M, Cameli P, Sestini P, Rottoli P, Bargagli E

Abstract
Serum amyloid A is an acute-phase protein with multiple immunological functions. Serum amyloid A is involved in lipid metabolism, inflammatory reactions, granuloma formation, and cancerogenesis. Additionally, serum amyloid A is involved in the pathogenesis of different autoimmune lung diseases. The levels of serum amyloid A has been evaluated in biological fluids of patients with different lung diseases, including autoimmune disorders, chronic obstructive pulmonary diseases, obstructive sleep apnea syndrome, sarcoidosis, asthma, lung cancer, and other lung disorders, such as idiopathic pulmonary fibrosis, tuberculosis, radiation pneumonitis, and cystic fibrosis. This review focuses on the cellular and molecular interactions of serum amyloid A in different lung diseases and suggests this acute-phase protein as a prognostic marker.

PMID: 31708467 [PubMed - as supplied by publisher]

The Phagocytosis of Blood Leukocytes from Cystic Fibrosis Patients is not Impaired in General.

Lung. 2019 Nov 09;:

Authors: Leuer L, Krill A, Wilkens H, Wagenpfeil G, Bischoff M, Meier C, Bals R, Tschernig T

Abstract
Impaired phagocytosis of Pseudomonas aeruginosa was found in isolated monocytes of peripheral blood of cystic fibrosis patients, but not in their neutrophils, as reported some years ago. In the present study, we analysed the phagocytic capacity of peripheral blood neutrophils and monocytes of cystic fibrosis patients and of healthy controls. Phagocytosis was determined using a commercial phagocytosis "in whole blood" assay on the basis of fluorescence-labelled opsonized Escherichia coli bacteria and flow cytometry. Venous blood of cystic fibrosis patients and of healthy controls was collected and the phagocytosis assay was performed. No differences in the percentage of phagocytic cells or in the overall phagocytic capacity were found between samples of cystic fibrosis patients and healthy controls either in monocytes or in neutrophils. Thus, our results did not support the hypothesis of a generally reduced phagocytic ability in the peripheral blood immune cells of cystic fibrosis patients.

PMID: 31707460 [PubMed - as supplied by publisher]

Regional differences in infection and structural lung disease in infants and young children with cystic fibrosis.

J Cyst Fibros. 2019 Nov 06;:

Authors: Carzino R, Frayman KB, King L, Vidmar S, Ranganathan S, AREST CF

Abstract
BACKGROUND: Both infection and inflammation are critical to the progression of cystic fibrosis (CF) lung disease. Potential anatomical differences in lower airway infection, inflammation and bronchiectasis in young children with CF raise questions regarding the pathogenesis of early structural lung disease.
METHODS: A longitudinal multi-centre birth cohort study of infants newly diagnosed with CF was conducted. Paired bronchoalveolar lavage (BAL) samples were obtained from the right middle lobe (RML) and lingula bronchi. Chest computed tomography (CT) was performed biennially and analysed using the modified CF-CT scoring system.
RESULTS: One hundred and twenty-four children (0.11 - 7.0 years) contributed 527 BAL samples and underwent 388 CT chest scans. Pro-inflammatory microbes were detected in 279 BAL samples (53%), either in both lingula and RML samples (69%), in the lingula alone (24%), or in the RML alone in only 7% of samples. Overall, the prevalence of structural lung disease was greater in the setting of pro-inflammatory microbes. Although infection was less commonly isolated in the right lung, bronchiectasis was more commonly detected in the right lung compared with the left. No anatomical differences in the presence of air trapping were detected.
CONCLUSION: Overall, the detection of pro-inflammatory microbes in the lower airways was associated with increased risk of both air trapping and bronchiectasis. However, the apparent discordance between commonest sites of isolation of pro-inflammatory microbes and the anatomical site of early bronchiectasis warrants further exploration.

PMID: 31706731 [PubMed - as supplied by publisher]

Male gender and unemployment are associated with lower levels of perceived social support in adults with cystic fibrosis.

J Psychosom Res. 2019 Nov 02;127:109858

Authors: Flewelling KD, Sellers DE, Sawicki GS, Robinson WM, Dill EJ

Abstract
OBJECTIVE: Adults with cystic fibrosis (CF) face unique challenges with regard to the attainment and maintenance of social support. Although social support has been shown to improve treatment outcomes in other patient-populations, research on social support in adults with CF is limited. In fact, no studies have examined factors associated with less perceived social support in this population. The current study aimed to fill this gap, thus providing CF care teams with empirical evidence about who may be most likely to lack support and inform future intervention.
METHODS: Participants in this cross-sectional study included 233 adults with CF who were part of a larger, longitudinal study. Participants completed the Interpersonal Support Evaluation List, a measure of social support, and attended routine clinical visits where measures of disease severity were obtained.
RESULTS: Being female and employed were associated with greater perceptions of social support in this sample. Age, income, education, marital status, and disease-severity were not related to perceptions of social support.
CONCLUSION: The present study revealed that individuals with CF who are unemployed and those who are male perceived having lower social support, perhaps identifying subgroups for whom targeted interventions may be appropriate.

PMID: 31706070 [PubMed - as supplied by publisher]

Ibuprofen Safety at the Golden Anniversary: Are all NSAIDs the Same? A Narrative Review.

Adv Ther. 2019 Nov 08;:

Authors: Varrassi G, Pergolizzi JV, Dowling P, Paladini A

Abstract
Ibuprofen first came to market about 50 years ago and rapidly moved to over-the-counter (OTC) sales. In April 2019, the National Agency for the Safety of Medicines and Health Products (ANSM) of France issued a warning for NSAID uses by patients with infectious diseases based on an analysis of 20 years of real-world safety data on ibuprofen and ketoprofen. Nevertheless, ibuprofen remains a mainstay in the analgesic armamentarium and with numerous randomized clinical trials, head-to-head studies, and decades of clinical experience. The authors offer a review of the safety of ibuprofen and how it may differ from other NSAIDs. Ibuprofen is associated with certain well-known gastrointestinal adverse effects that are related to dose and patient population. Among nonsteroidal anti-inflammatory drugs (NSAIDs), ibuprofen has a comparatively low risk of cardiovascular adverse effects. It has been associated with renal and hepatic adverse effects, which appear to depend on dose, concomitant medications, and patient population. The association of ibuprofen with infections is more complex in that it confers risk in some situations but benefits in others, the latter in cystic fibrosis. Emerging interest in the literature is providing evidence of the role of ibuprofen as a possible endocrine disrupter as well as its potential antiproliferative effects for cancer cells. Taken altogether, ibuprofen has a favorable safety profile and is an effective analgesic for many acute and chronic pain conditions, although it-like other NSAIDs-is not without risk. After 50 years, evidence is still emerging about ibuprofen and its unique safety profile among NSAIDs. FUNDING: The Rapid Service Fee was funded by Abbott Established Pharmaceuticals Division (EPD).

PMID: 31705437 [PubMed - as supplied by publisher]

Ethnically Diverse Normative Data for Diffusing Capacity and Lung Volumes: Another Research Priority.

Ann Am Thorac Soc. 2019 Nov 08;:

Authors: Weiner DJ, Graham B, Stanojevic S

PMID: 31702941 [PubMed - as supplied by publisher]

Prevalence of Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis Patients using Two Different Diagnostic Criteria.

Eur Ann Allergy Clin Immunol. 2019 Nov 08;:

Authors: Maleki M, Hassanzad V, Mahdaviani SA, Poorabdollah M, Mehrian P, Behnampour N, Mirenayat MS, Abastabar M, Tavakoli M, Hedayati MT

Abstract
Summary: Objective. There are different diagnostic criteria for the diagnosis of Allergic bronchopulmonary aspergillosis (ABPA) in CF patients. In this present study we evaluated the prevalence of ABPA in Iranian CF patients by two more usual diagnostic criteria as ISHAM working criteria (A) and CF Foundation Consensus Conference criteria (B). Methods. Eighty six CF patients were included in the study. All CF patients underwent for Aspergillus skin prick test (AST), Aspergillus-specific IgE (sIgEAf) and Aspergillus-specific IgG (sIgGAf), total IgE. The ABPA prevalence was estimated by two diagnostic criteria, (A) and (B) and compared. Results. The frequency of positive AST, total IgE, sIgEAf and sIgGAf were 47 (54.6%), 9 (10.5%), 42 (48.8%) and 67 (77.9%), respectively. The obtained rate of ABPA prevalence (10.5%) was identical in two diagnostic criteria A and B (kappa value of 1.000). Conclusions. The applied diagnostic criteria had no significant effect on the reported rate of ABPA prevalence.

PMID: 31702121 [PubMed - as supplied by publisher]

Metabolomic studies of Pseudomonas aeruginosa.

World J Microbiol Biotechnol. 2019 Nov 07;35(11):178

Authors: Mielko KA, Jabłoński SJ, Milczewska J, Sands D, Łukaszewicz M, Młynarz P

Abstract
Pseudomonas aeruginosa is a common, Gram-negative environmental organism. It can be a significant pathogenic factor of severe infections in humans, especially in cystic fibrosis patients. Due to its natural resistance to antibiotics and the ability to form biofilms, infection with this pathogen can cause severe therapeutic problems. In recent years, metabolomic studies of P. aeruginosa have been performed. Therefore, in this review, we discussed recent achievements in the use of metabolomics methods in bacterial identification, differentiation, the interconnection between genome and metabolome, the influence of external factors on the bacterial metabolome and identification of new metabolites produced by P. aeruginosa. All of these studies may provide valuable information about metabolic pathways leading to an understanding of the adaptations of bacterial strains to a host environment, which can lead to new drug development and/or elaboration of new treatment and diagnostics strategies for Pseudomonas.

PMID: 31701321 [PubMed - in process]

Non-invasive carbon dioxide monitoring in patients with cystic fibrosis during general anesthesia: end-tidal versus transcutaneous techniques.

J Anesth. 2019 Nov 07;:

Authors: May A, Humston C, Rice J, Nemastil CJ, Salvator A, Tobias J

Abstract
INTRODUCTION: The gold standard for measuring the partial pressure of carbon dioxide remains arterial blood gas (ABG) analysis. For patients with cystic fibrosis undergoing general anesthesia or polysomnography studies, continuous non-invasive carbon dioxide monitoring may be required. The current study compares end-tidal (ETCO2), transcutaneous (TCCO2), and capillary blood gas carbon dioxide (Cap-CO2) monitoring with the partial pressure of carbon dioxide (PaCO2) from an ABG in patients with cystic fibrosis.
METHODS: Intraoperatively, a single CO2 value was simultaneously obtained using ABG (PaCO2), capillary (Cap-CO2), TCCO2, and ETCO2 techniques. Tests for correlation (Pearson's coefficient) and agreement (Bland-Altman analysis) were performed. Data were further stratified into two subgroups based on body mass index (BMI) and percent predicted forced expiratory volume in 1 s (FEV1%). Additionally, the absolute difference in the TCCO2, ETCO2, and Cap-CO2 values versus PaCO2 was calculated. The mean ± SD differences were compared using a paired t test while the number of times the values were ≤ 3 mmHg and ≤ 5 mmHg from the PaCO2 were compared using a Fishers' exact test.
RESULTS: The study cohort included 47 patients (22 males, 47%) with a mean age of 13.4 ± 7.8 years, median (IQR) BMI of 18.7 kg/m2 (16.7, 21.4), and mean FEV1% of 87.3 ± 18.3%. Bias (SD) was 4.8 (5.7) mmHg with Cap-CO2 monitoring, 7.3 (9.7) mmHg with TCCO2 monitoring, and 9.7 (7.7) mmHg with ETCO2 monitoring. Although there was no difference between the degree of bias in the population as a whole, when divided based on FEV1% and BMI, there was greater bias with ETCO2 in patients with a lower FEV1% and a higher BMI. The Cap-CO2 vs. PaCO2 difference was 5.2 ± 5.3 mmHg (SD), with 16 (48%) ≤ 3 mmHg and 20 (61%) ≤ 5 mmHg from the ABG value. The TCCO2-PaCO2 difference was 9.1 ± 7.2 mmHg (SD), with 11 (27%) ≤ 3 mmHg and 15 (37%) ≤ 5 mmHg from the ABG value. The ETCO2-PaCO2 mean difference was 11.2 ± 7.9 mmHg (SD), with 5 (12%) ≤ 3 mmHg and 11 (26%) ≤ 5 mmHg from the ABG value.
CONCLUSIONS: While Cap-CO2 most accurately reflects PaCO2 as measured on ABG, of the non-invasive continuous monitors, TCCO2 was a more accurate and reliable measure of PaCO2 than ETCO2, especially in patients with worsening pulmonary function (FEV1% ≤ 81%) and/or a higher BMI (≥ 18.7 kg/m2).

PMID: 31701307 [PubMed - as supplied by publisher]

MiR-146a is over-expressed and controls IL-6 production in cystic fibrosis macrophages.

Sci Rep. 2019 Nov 07;9(1):16259

Authors: Luly FR, Lévêque M, Licursi V, Cimino G, Martin-Chouly C, Théret N, Negri R, Cavinato L, Ascenzioni F, Del Porto P

Abstract
Cystic fibrosis (CF) is an inherited disease that is characterised by susceptibility to bacterial infections and chronic lung inflammation. Recently, it was suggested that macrophages contribute to impaired host defence and excessive inflammatory responses in CF. Indeed, dysfunction attributed to CF macrophages includes decreased bacterial killing and exaggerated inflammatory responses. However, the mechanisms behind such defects have only been partially defined. MicroRNAs (miRNAs) have emerged as key regulators of several macrophage functions, including their activation, differentiation and polarisation. The goal of this study was to investigate whether miRNA dysregulation underlies the functional abnormalities of CF macrophages. MiRNA profiling of macrophages was performed, with 22 miRNAs identified as differentially expressed between CF and non-CF individuals. Among these, miR-146a was associated with significant enrichment of validated target genes involved in responses to microorganisms and inflammation. As miR-146a dysregulation has been reported in several human inflammatory diseases, we analysed the impact of increased miR-146a expression on inflammatory responses of CF macrophages. These data show that inhibition of miR-146a in lipopolysaccharide-stimulated CF macrophages results in increased interleukin-6 production, which suggests that miR-146a overexpression in CF is functional, to restrict inflammatory responses.

PMID: 31700158 [PubMed - in process]

ERS/TSANZ Task Force Statement on the Management of Reproduction and Pregnancy in Women with Airways Diseases.

Eur Respir J. 2019 Nov 07;:

Authors: Middleton PG, Gade EJ, Aguilera C, MacKillop L, Button BM, Coleman C, Johnson B, Albrechtsen C, Edenborough F, Rigau D, Gibson PG, Backer V

Abstract
This Statement outlines a review of the literature and expert opinion concerning management of reproduction and pregnancy in women with airways diseases. The Statement represents the first collaboration of the European Respiratory Society (ERS) and the Thoracic Society of Australia and New Zealand (TSANZ) to develop such documents. This Statement covers airways diseases - namely Asthma, Cystic Fibrosis (CF) and non-CF Bronchiectasis. Many women with these diseases are now living into reproductive age with some developing moderate to severe impairment of lung function in early adulthood. The Statement covers aspects of fertility, management during pregnancy, effects of drugs and issues during delivery. The Statement summarises current knowledge but does not make recommendations. This Statement will not discuss the physiological changes occurring in pregnancy, such as the changes in airway physiology, resting ventilation and sleep nor other forms of lung disease such as pulmonary fibrosis, pulmonary hypertension, gastro-oesophageal reflux-related lung disease or issues with nicotine consumption.

PMID: 31699837 [PubMed - as supplied by publisher]