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 2017/01/14

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.

8 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 2017/01/13

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.

Genomic information on multidrug-resistant livestock-associated methicillin-resistant Staphylococcus aureus ST398 isolated from a Brazilian patient with cystic fibrosis.

Mem Inst Oswaldo Cruz. 2017 Jan 01;112(1):79-80

Authors: Lima DF, Cohen RW, Rocha GA, Albano RM, Marques EA, Leão RS

Abstract
Alarmingly, the isolation of methicillin-resistant Staphylococcus aureus (MRSA) has been increasing among patients with cystic fibrosis (CF). During a previous molecular characterisation of MRSA isolates obtained from patients with CF from Rio de Janeiro, Brazil, one isolate was identified as the ST398 clone, a livestock-associated (LA) MRSA. In this study, we report the draft genome sequence of an LA-MRSA ST398 clone isolated from a patient with CF.

PMID: 28076471 [PubMed - in process]

Hospitalization rates for intussusception in children aged 0-59 months from 2009 to 2014 in Italy.

Hum Vaccin Immunother. 2017 Jan 11;:1-5

Authors: Restivo V, Costantino C, Tramuto F, Vitale F

Abstract
The real cause of intussusception is not fully understood and a variety of conditions have been associated with it (Meckel diverticulum, polyps, duplication cysts, parasites, Henoch-Schönlein purpura, cystic fibrosis, hemolytic-uremic syndrome and infectious gastroenteritis). Furthermore few European countries, following WHO recommendation to monitor baseline incidence of intussusception before implementation of immunization program for rotavirus, used intussusception rate as a baseline value to compare the same figures in the period before and after introduction of vaccination. In this study, data of intussusception hospitalizations occurred among Italian children aged 0 through 59 months from 2009 to 2014 were analyzed. A total amount of 3,088 children were included, accounting for a hospitalization rate of 20.2 per 100,000. Overall, the hospitalization rate for intussusception had a slight increase in trend from 2009 to 2014 (18%). In particular children 0-11 months had a hospitalization rate higher than 12-59 months with an aggregate value of 36 Vs. 16 per 100,000 respectively. Among all children hospitalized for intussusception a total of 239 (7.7%) had also a previous or concomitant hospitalization for gastroenteritis. This study demonstrates that Italian hospitalizations for intussusception are increasing by time and the role played by different risk factors, including acute gastroenteritis, have to be investigated in the future. These data could be useful to monitor intussusception hospitalization in the perspective of anti-rotavirus vaccination introduction in Italy.

PMID: 28075671 [PubMed - as supplied by publisher]

Role of IRE1α/XBP-1 in Cystic Fibrosis Airway Inflammation.

Int J Mol Sci. 2017 Jan 09;18(1):

Authors: Ribeiro CM, Lubamba BA

Abstract
Cystic fibrosis (CF) pulmonary disease is characterized by chronic airway infection and inflammation. The infectious and inflamed CF airway environment impacts on the innate defense of airway epithelia and airway macrophages. The CF airway milieu induces an adaptation in these cells characterized by increased basal inflammation and a robust inflammatory response to inflammatory mediators. Recent studies have indicated that these responses depend on activation of the unfolded protein response (UPR). This review discusses the contribution of airway epithelia and airway macrophages to CF airway inflammatory responses and specifically highlights the functional importance of the UPR pathway mediated by IRE1/XBP-1 in these processes. These findings suggest that targeting the IRE1/XBP-1 UPR pathway may be a therapeutic strategy for CF airway disease.

PMID: 28075361 [PubMed - in process]

Azoospermic patient's treatment: An experience of a PMA hospital unit and role of ultrasonography.

Arch Ital Urol Androl. 2016 Dec 30;88(4):314-316

Authors: Panella P, Pepe P, Borzì P, Vento ME, Pennisi M, Scollo P

Abstract
INTRODUCTION: Azoospermia causes about 10% of male infertility and the best therapeutic option is the retrieval of sperm from testis or epididymis.
MATERIAL AND METHODS: From Juanary 2008 to June 2016, 92 men (median 36 years; range: 25-54 years) were submitted in 47 cases to TESE (testicular sperm extraction) and in 45 cases to PESA (percutaneous epididymal sperm aspiration) for secretory and obstructive azoospermia, respectively; moreover, all the patients previously underwent color Doppler ultrasound of the testis and transrectal ultrasound of the prostate.
RESULTS: Serum FSH values were 9.4 ml/UI and 36.4 ml/UI (median 18.2 ml/UI) with an estimated volume of the testis equal to 5 ml; 40 men had the mutation for cystic fibrosis with bilateral agenesis of the deferentia vasa, 4 men had a cyst of the prostatic utricle, 1 man had retrograde ejaculation, 7 had an epididymis cyst and 2 had anejaculation secondary to traumatic neurologic spinal cord injury. The retrieval of sperm was performed in 39 (83%) and 36 (80%) of the patients submitted to TESE and PESA, respectively. The pregnancy rate was equal to 28% and 33% in men with secretory and obstructive azoospermia, respectively.
DISCUSSION: Assisted reproduction technology with a multidisciplinary team is provided of a pregnancy rate equal about 30% in men with azoospermia; ultrasound allows to evaluate abnormalities of the testis and prostate improving the percentage of pregnancy.

PMID: 28073201 [PubMed - in process]

7 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 2017/01/11

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.

9 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 2017/01/10

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.

Structural Insight into the Gene Expression Profiling of the hcn Operon in Pseudomonas aeruginosa.

Appl Biochem Biotechnol. 2017 Jan 07;:

Authors: Chowdhury N, Bagchi A

Abstract
Pseudomonas aeruginosa is a common opportunistic human pathogen. It generally attacks immunosuppressed patients like AIDS, cancer, cystic fibrosis, etc. The virulence of P. aeruginosa is mediated by various virulence factors. One of such potential virulence factors is HCN synthesized by HCN synthase enzyme, which is encoded by the hcnABC operon. The expressions of the genes of this operon are regulated by three transcriptional regulators, viz., LasR, ANR, and RhlR. In our previous work, we analyzed the molecular details of the functionalities of LasR. In this work, we focused on ANR. ANR is a regulatory protein which belongs to the FNR family and works in anaerobic condition. ANR binds to the promoter DNA, named ANR box, as a dimer. The dimerization of this ANR protein is regulated by Fe4S4, an iron-sulfur cluster. This dimer of ANR (ANR-Fe4S4/ANR-Fe4S4) recognizes and binds the promoter DNA sequence and regulates the transcription of this hcnABC operon. Till date, the biomolecular details of the interactions of ANR dimer with the promoter DNA are not fully understood. Thus, we built the molecular model of ANR-Fe4S4/ANR-Fe4S4. We docked the complex with the corresponding promoter DNA region. We analyzed the mode of interactions with the promoter DNA under different conditions. Thus, we tried to analyze the functionality of the ANR protein during the expressions of the genes of the hcnABC operon. So far, this is the first report to detail the molecular mechanism of the gene expression in P. aeruginosa.

PMID: 28064429 [PubMed - as supplied by publisher]

The sialic acid to urea ratio as a measure of airway surface hydration.

Am J Physiol Lung Cell Mol Physiol. 2017 Jan 06;:ajplung.00398.2016

Authors: Esther CR, Hill DB, Button B, Shi S, Jania CM, Duncan EA, Doerschuk CM, Chen G, Ranganathan S, Stick SM, Boucher RC

Abstract
INTRODUCTION: Although airway mucus dehydration is key to pathophysiology of cystic fibrosis (CF) and other airways diseases, measuring mucus hydration is challenging. We explored a robust method to estimate mucus hydration using sialic acid as a marker for mucin content.
METHODS: Terminal sialic acid residues from mucins were cleaved by acid hydrolysis from airway samples, and concentrations of sialic acid, urea, and other biomarkers analyzed using mass spectrometry.
RESULTS: In mucins purified from human airway epithelial (HAE), sialic acid concentrations after acid hydrolysis correlated with mucin concentrations (r2=0.92). Sialic acid/urea ratios measured from filters applied to the apical surface of cultured HAE correlated to % solids and were elevated in samples from CF HAEs relative to controls (2.2±1.1 vs. 0.93±1.8, p<0.01). Sialic acid/urea ratios were elevated in bronchoalveolar lavage fluid (BALF) from βENaC transgenic mice, known to have reduced mucus hydration, and mice sensitized to house dust mite allergen. In a translational application, elevated sialic acid/urea ratios were measured in BALF from young children with CF who had airway infection relative to those who did not (5.5±3.7 vs.1.9±1.4, p<0.02), and could be assessed simultaneously with established biomarkers of inflammation.
CONCLUSIONS: The sialic acid/urea ratio performed similarly to % solids, the gold standard measure of mucus hydration. The method proved robust and has potential to serve as flexible techniques to assess mucin hydration, particularly in samples like BALF in which established methods such as % solids cannot be utilized.

PMID: 28062483 [PubMed - as supplied by publisher]

Successful survival of an extreme premature infant with cystic fibrosis.

BMJ Case Rep. 2017 Jan 06;2017:

Authors: Goddard T, Andersen C, Tai A

Abstract
Extreme premature infants with cystic fibrosis typically do not survive the neonatal phase. This case report describes the youngest survivor of a premature infant with cystic fibrosis and highlights the importance of advanced neonatal care with cystic fibrosis therapy.

PMID: 28062439 [PubMed - in process]

Exposure of airway epithelial cells to Pseudomonas aeruginosa biofilm-derived quorum sensing molecules decrease the activity of the anti-oxidant response element bound by NRF2.

Biochem Biophys Res Commun. 2017 Jan 03;:

Authors: Roussel L, Rousseau S

Abstract
Chronic bacterial infections in cystic fibrosis lung disease are often characterized by Pseudomonas aeruginosa biofilms that are regulated by bacterial intercellular signals termed quorum sensing (QS), such as N-(3-oxododecanoyl)-l-homoserine lactone (3OC12-HSL). This study reports that biofilm-derived exoproducts decrease the transcriptional activity of the anti-oxidant response element in bronchial epithelial cells. In a live co-culture assay of BEAS-2B cells and P. aeruginosa biofilm, the QS molecule 3OC12-HSL was an important but not sole contributor to the inhibition of basal NRF2 luciferase reporter activity. Moreover, biofilm-derived exoproducts and 3OC12-HSL decrease the expression of endogenous antioxidant response element-regulated genes hemeoxygenase-1 (HO-1) and NAD(P)H Quinone Dehydrogenase-1 (NQO-1) while they increase IL-8 expression. As previously reported, IL-8 expression is partially dependent on p38 MAPK activity, but the inhibitory effect of biofilm QS molecules on HO-1 and NQO-1 expression occurs independently of this protein kinase. Finally, the transfection of CFTRdelF508 but not its wild type counterpart decreases basal, planktonic PsaDM and sulforaphane-driven NRF2 luciferase reporter activity in BEAS-2B cells. Therefore, the presence of quorum sensing molecules derived from bacterial biofilms lowers the transcriptional activity of the anti-oxidant response element, which may contribute to the establishment of chronic bacterial infections, especially in the presence of mutated CFTR. Increasing NRF2 activity may thus be a promising strategy to potentiate anti-biofilm activity in cystic fibrosis lung disease.

PMID: 28062182 [PubMed - as supplied by publisher]

10 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 2017/01/07

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.

CFTR structure: lassoing cystic fibrosis.

Nat Struct Mol Biol. 2017 Jan 05;24(1):13-14

Authors: Ford B

PMID: 28054564 [PubMed - in process]

Two cases of non-cystic fibrosis (CF) bronchiectasis with allergic bronchopulmonary aspergillosis.

Respir Med Case Rep. 2017;20:68-71

Authors: De H, Azad SM, Giri PP, Pal P, Ghosh A, Maitra A

Abstract
Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity reaction in patients with asthma or cystic fibrosis (CF), which is associated with bronchi colonized by the fungus Aspergillus species, most often Aspergillus fumigatus. ABPA is an important consideration for asthmatics that do not respond to asthma management or with recurrent chest infections and deteriorating lung function in children with cystic fibrosis. We present two cases of non CF bronchiectasis associated with ABPA who presented to our hospital with recurrent hospitalisations of undiagnosed aetiology.

PMID: 28053855 [PubMed - in process]

The international primary ciliary dyskinesia cohort (iPCD Cohort): methods and first results.

Eur Respir J. 2017 Jan;49(1):

Authors: Goutaki M, Maurer E, Halbeisen FS, Amirav I, Barbato A, Behan L, Boon M, Casaulta C, Clement A, Crowley S, Haarman E, Hogg C, Karadag B, Koerner-Rettberg C, Leigh MW, Loebinger MR, Mazurek H, Morgan L, Nielsen KG, Omran H, Schwerk N, Scigliano S, Werner C, Yiallouros P, Zivkovic Z, Lucas JS, Kuehni CE, PCD Italian Consortium, Swiss PCD Group, French Reference Centre for Rare Lung Diseases, Genetic Disorders of Mucociliary Clearance Consortium

Abstract
Data on primary ciliary dyskinesia (PCD) epidemiology is scarce and published studies are characterised by low numbers. In the framework of the European Union project BESTCILIA we aimed to combine all available datasets in a retrospective international PCD cohort (iPCD Cohort).We identified eligible datasets by performing a systematic review of published studies containing clinical information on PCD, and by contacting members of past and current European Respiratory Society Task Forces on PCD. We compared the contents of the datasets, clarified definitions and pooled them in a standardised format.As of April 2016 the iPCD Cohort includes data on 3013 patients from 18 countries. It includes data on diagnostic evaluations, symptoms, lung function, growth and treatments. Longitudinal data are currently available for 542 patients. The extent of clinical details per patient varies between centres. More than 50% of patients have a definite PCD diagnosis based on recent guidelines. Children aged 10-19 years are the largest age group, followed by younger children (≤9 years) and young adults (20-29 years).This is the largest observational PCD dataset available to date. It will allow us to answer pertinent questions on clinical phenotype, disease severity, prognosis and effect of treatments, and to investigate genotype-phenotype correlations.

PMID: 28052956 [PubMed - in process]

Hypoxia and sterile inflammation in cystic fibrosis airways: mechanisms and potential therapies.

Eur Respir J. 2017 Jan;49(1):

Authors: Montgomery ST, Mall MA, Kicic A, Stick SM, AREST CF

Abstract
Cystic fibrosis is one of the most common autosomal recessive genetic diseases in Caucasian populations. Diagnosis via newborn screening and targeted nutritional and antibiotic therapy have improved outcomes, however respiratory failure remains the key cause of morbidity and mortality. Progressive respiratory disease in cystic fibrosis is characterised by chronic neutrophilic airway inflammation associated with structural airway damage leading to bronchiectasis and decreased lung function. Mucus obstruction is a characteristic early abnormality in the cystic fibrosis airway, associated with neutrophilic inflammation often in the absence of detectable infection. Recent studies have suggested a link between hypoxic cell death and sterile neutrophilic inflammation in cystic fibrosis and other diseases via the IL-1 signalling pathway. In this review, we consider recent evidence regarding the cellular responses to respiratory hypoxia as a potential driver of sterile neutrophilic inflammation in the lung, current knowledge on hypoxia as a pathogenic mechanism in cystic fibrosis and the potential for current and future therapies to alleviate hypoxia-driven sterile inflammation.

PMID: 28052955 [PubMed - in process]

Lung Allocation Score: A Single-Center Simulation.

Transplant Proc. 2016 Mar;48(2):391-4

Authors: Rosso L, Palleschi A, Tosi D, Mendogni P, Righi I, Carrinola R, Montoli M, Damarco F, Rossetti V, Morlacchi LC, Nosotti M

Abstract
BACKGROUND: The lung allocation score (LAS) was introduced in the United States in May 2005 with the main goal of reducing the waiting list mortality of patients with end-stage lung diseases, but also to enhance the lung transplant benefit and improve the management of urgent candidates. Several papers have reported that LAS resulted in a reduction of the waiting list mortality but no significant survival benefit was noted.
METHODS: We evaluate the usefulness of LAS as a predictor for lung transplantation outcome in 123 patients listed for lung transplantation in an Italian center. Primary endpoints were waiting list mortality and posttransplant mortality at 1 year; secondary endpoints included perioperative circulatory support, cardiopulmonary bypass, primary graft dysfunction, and long-term survival after transplantation.
RESULTS: We observed the absence of correlation between LAS and waiting list mortality. The LAS did not affect the long-term survival in our population.
CONCLUSIONS: High LAS was predictive of primary graft dysfunction of grade 3 in the first 72 hours after transplantation.

PMID: 27109963 [PubMed - indexed for MEDLINE]

A Case of Surgical Site Infection Caused by Mycobacterium fortuitum, following Herniorrhaphy.

J Clin Diagn Res. 2016 Nov;10(11):DD01-DD02

Authors: Madhusudhan NS, Malini A, Sangma MM

Abstract
Rapidly Growing Mycobacteria (RGM) are opportunistic pathogens found in the environment. Mycobacterium fortuitum, M. chelonae and M.abscessus are the important human pathogens of this group. They cause wound infections, disseminated cutaneous disease, pulmonary infection in patients with cystic fibrosis or bronchiectasis, bone and joint infections and keratitis. Infections due to these Non-Tuberculous Mycobacteria (NTM) are increasingly reported. Post laparoscopic wound infections, mesh site infections and other surgical site infections due to M. fortuitum and M. chelonae have been reported. Usually wound infections due to atypical mycobacteria have delayed onset and do not respond to conventional antibiotics. Identification of RGM can be done by a set of cumbersome biochemical tests, High Performance Liquid Chromatography (HPLC), molecular methods using DNA probes or by Polymerase Chain Reaction (PCR). We here report a case of post-herniorrhaphy wound infection due to M. fortuitum which was identified by molecular method (HAIN mycobacterial species system). This case report underscores the importance of examining Ziehl-Neelsen (ZN) stain of all exudates with sterile culture on day one for non fastidious bacteria. Timely identification can lead to prompt therapy of patients preventing further complications.

PMID: 28050369 [PubMed]

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]