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"Cystic Fibrosis"

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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.

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 2019/06/27

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.

Exhaled nitric oxide in pediatric patients with respiratory disease.

J Breath Res. 2019 Jun 24;:

Authors: Rachel M, Biesiadecki M, Aebisher D, Galiniak S

Abstract
Measurement of nitric oxide (NO) levels in exhaled air from the upper and lower airways is currently used as a non-invasive marker of inflammation in respiratory diseases. Assessment of NO exhaled from the lower air respiratory tract is considered as a quick method for confirmation and control of asthma in patients as well as an estimation of treatment efficiency.
 The main aim of this study was to determine differences between levels of exhaled nitric oxide (fractional exhaled NO; FeNO) in patients with respiratory disease as measured by an electrochemical analyzer. Measurements were taken in 352 pediatric patients aged 4-17 with cystic fibrosis (n=43), asthma (n=69), allergic rhinitis (n=70), asthma and allergic rhinitis (n=128) and non-diseased children (n=42) recruited from the Allergology Outpatient Department, Provincial Hospital No 2, Rzeszów. The second objective of this study was to assess any correlations between FeNO and clinical parameters of patients.
 The level of FeNO in patients with CF was normal when compared with control subjects (10.8 ± 2.9 vs 11.4 ± 6 ppb). We found significantly higher FeNO in patients with asthma (26.6 ± 15.3 ppb, p<0.001), allergic rhinitis (18.4 ± 9.6 ppb, p<0.01) as well as in patients with both asthma and allergic rhinitis (43.3 ± 31.1 ppb, p<0.001) when compared to healthy children. Statistical analysis revealed a positive correlation between FeNO and age, height and weight of control subjects, and height in children with allergic rhinitis. FeNO was independent of sex, BMI, spirometry and blood results as well as the type of residence in control children and subjects with cystic fibrosis, asthma, allergic rhinitis and combined asthma and allergic rhinitis. 
 We found normal levels of FeNO in children with CF and elevated levels in patients with asthma, allergic rhinitis and combined asthma and allergic rhinitis as compared to control subjects. Due to conflicting data, there is still a need for additional research, especially related to regarding factors that affect FeNO levels in respiratory disease.

PMID: 31234165 [PubMed - as supplied by publisher]

Intravenous antibiotic use and exacerbation events in an adult cystic fibrosis centre: A prospective observational study.

Respir Med. 2019 Jun 18;154:109-115

Authors: Hoo ZH, Bramley NR, Curley R, Edenborough FP, Walters SJ, Campbell MJ, Wildman MJ

Abstract
INTRODUCTION: In CF, people with higher FEV1 are less aggressively treated with intravenous (IV) antibiotics, with resultant negative impact on their health outcomes. This could be entirely clinician-driven, but patient choice may also influence IV use. In this prospective observational study, we explored IV recommendations by clinicians and IV acceptance by adults with CF to understand how clinical presentations consistent with exacerbations resulted in IV use.
METHODS: Clinical presentations consistent with exacerbations, IV recommendation by clinicians and IV acceptance by patients were prospectively identified for every adult with CF in Sheffield throughout 2016, excluding those who had lung transplantation (n = 7) or on ivacaftor (n = 13). Relevant demographic data, e.g. %FEV1, were extracted from medical records. Multi-level mixed-effects logistic regression models were used to compare IV recommendations vs non-recommendations for all clinical encounters, and IV acceptance vs non-acceptance for all IV recommendations.
RESULTS: Among 186 adults (median age 27 years, median FEV1 78.5%), there were 434 exacerbation events and 318 IV use episodes following 1010 clinical encounters. Only 254 (58.5%) of exacerbations were IV treated. A diagnosis of exacerbation, higher number of symptoms and lower %FEV1 were independent predictors for IV recommendation by clinicians. Higher number of symptoms and lower %FEV1 were also independent predictors for IV acceptance by adults with CF.
CONCLUSIONS: Lower IV use among adults with higher %FEV1 was influenced by both clinicians' and patients' decisions. Using IV antibiotics as an exacerbation surrogate could under-estimate exacerbation rates and conceal differential treatment decisions according to varying clinical characteristics.

PMID: 31234038 [PubMed - as supplied by publisher]

What do we know about travel for children with special health care needs? A review of the literature.

Travel Med Infect Dis. 2019 Jun 21;:

Authors: Kohl SE, Barnett ED

Abstract
BACKGROUND: Children travel with their families, including children with chronic illness. We know that adults with chronic illness who travel are more likely than their healthy peers to become sick while traveling. A review of the literature was undertaken to identify what is known about traveling with children with special health care needs and to identify gaps in our knowledge.
METHODS: An Online search of the PubMed, CINAHL and Google databases of English language literature was conducted June 2016, October 2017, June 2018 and April 2019 using the terms children and travel, air travel, travel health, disabled child, children with special healthcare needs, parents of disabled children, vacations, recreation, international, wheelchairs, planning techniques, asthma, diabetes, altitude, cystic fibrosis, inflammatory bowel disease, sickle cell disease, depression, food allergies, Attention Deficit Hyperactivity Disorder (ADHD), and seizures. The search was limited to years 2000-2019. A secondary search of relevant articles was conducted using the reference sections of articles identified in the primary search.
RESULTS: 185 papers were examined for travel health related outcomes for children and adults with chronic diseases. Articles were excluded if they addressed the educational needs of students with disabilities traveling abroad, did not directly address travel health (e.g travel skills, travel itineraries), contained outdated policy statements, or were case reports of a single patient. The remaining 84 papers were organized and reviewed by organ systems. The articles were primarily descriptive and did not lend themselves to a systematic review.
CONCLUSION: Children traveling with chronic and complex health conditions are a heterogeneous group of vulnerable travelers. Closing the knowledge gap about how to best help these travelers requires a multipronged approach. Research is urgently needed to identify best practices for five of the most common chronic childhood diseases: asthma, depression, ADHD, food allergies and autism. For less common illnesses, ones typically cared for in specialty clinics, expert consensus opinion and multi-center studies are needed. Families and disease advocacy societies should be included in the research as they may have already identified the most pressing travel-related health concerns and solutions for these problems.

PMID: 31233860 [PubMed - as supplied by publisher]

Common content between quality of life questionnaires for children with cystic fibrosis and the International Classification of Functionality, Disability and Health.

J Rehabil Med. 2019 Jun 10;:

Authors: Gomes DC, Longo E, de Camargo OK, de Sousa Dantas D, Ferreira HN, Regalado ICR, Ribeiro LC, Pereira SA

Abstract
OBJECTIVE: To identify the most common quality of life instruments for children with cystic fibrosis and link the content with the International Classification of Functioning, Disability and Health (ICF).
METHODS: The study was conducted in 2 stages. The first stage involved a review of the literature to select quality of life questionnaires. In the second stage 2 independent reviewers identified questionnaire items and categories corresponding to the ICF, according to approved methodology. The degree of agreement was calculated using the kappa coefficient.
RESULTS: Two questionnaires were selected: the Cystic Fibrosis Questionnaire and DISABKIDS®. A total of 130 concepts were identified from the 112 items. Forty-seven different ICF categories were linked (k<0.62 for all questionnaires), 21 (44.7%) were related to the "body function" domain, 20 (42.6%) to "activity and participation" and 6 (12.8%) to "environmental factors". Thirteen items (10%) could not be linked because they represent personal factors or are not covered by the ICF.
CONCLUSION: Body functions were the category most linked to the ICF. Environmental factors were poorly described, and no items were related to body structures in any of the instruments.

PMID: 31233182 [PubMed - as supplied by publisher]

Mesenteric Lift for Constipation in Cystic Fibrosis.

J Am Osteopath Assoc. 2019 Jul 01;119(7):e36-e37

Authors: Sarti F, Wolf K, Talsma J, Pierce-Talsma S

PMID: 31233115 [PubMed - in process]

OMT for the Prevention and Management of Chronic Constipation and Distal Intestinal Obstructive Syndrome in Cystic Fibrosis: A Pilot Study.

J Am Osteopath Assoc. 2019 Jul 01;119(7):e31-e35

Authors: Modlin SE, Borofka K, Franzini D, Klene-Bowns AC, Nuño VA

Abstract
Context: Cystic fibrosis (CF) is an autosomal recessive genetic disorder primarily affecting the lungs and digestive system. Patients with CF often have multiorgan dysfunction, including chronic lung infections, pancreatic insufficiency, chronic constipation, and distal intestinal obstructive syndrome (DIOS).
Objective: To understand the impact of osteopathic manipulative treatment (OMT) on the prevention and management of gastrointestinal symptoms in patients with CF.
Methods: This study used OMT for physical manipulation of the viscera, spine, and other somatic components to improve bowel symptoms and prevent DIOS. These effects were achieved by releasing myofascial restrictions found in the abdomen and somatic structures with the intent to optimize the autonomic and lymphatic systems and improve range of motion.
Results: Four of 5 participants had a decrease in pain, and 3 participants had a reduced need for laxatives during treatment. Four participants had an overall increase in satisfaction with their bowel movements while being treated with OMT.
Conclusion: These findings support the use of OMT as a method for the management of chronic constipation and DIOS in the CF population. However, because of the small population size, more research with larger populations is needed.

PMID: 31233114 [PubMed - in process]

Network Biology Identifies Novel Regulators of CFTR Trafficking and Membrane Stability.

Front Pharmacol. 2019;10:619

Authors: Loureiro CA, Santos JD, Matos AM, Jordan P, Matos P, Farinha CM, Pinto FR

Abstract
In cystic fibrosis, the most common disease-causing mutation is F508del, which causes not only intracellular retention and degradation of CFTR, but also defective channel gating and decreased membrane stability of the small amount that reaches the plasma membrane (PM). Thus, pharmacological correction of mutant CFTR requires targeting of multiple cellular defects in order to achieve clinical benefit. Although small-molecule compounds have been identified and commercialized that can correct its folding or gating, an efficient retention of F508del CFTR at the PM has not yet been explored pharmacologically despite being recognized as a crucial factor for improving functional rescue of chloride transport. In ongoing efforts to determine the CFTR interactome at the PM, we used three complementary approaches: targeting proteins binding to tyrosine-phosphorylated CFTR, protein complexes involved in cAMP-mediated CFTR stabilization at the PM, and proteins selectively interacting at the PM with rescued F508del-CFTR but not wt-CFTR. Using co-immunoprecipitation or peptide-pull down strategies, we identified around 400 candidate proteins through sequencing of complex protein mixtures using the nano-LC Triple TOF MS technique. Key candidate proteins were validated for their robust interaction with CFTR-containing protein complexes and for their ability to modulate the amount of CFTR expressed at the cell surface of bronchial epithelial cells. Here, we describe how we explored the abovementioned experimental datasets to build a protein interaction network with the aim of identifying novel pharmacological targets to rescue CFTR function in cystic fibrosis (CF) patients. We identified and validated novel candidate proteins that were essential components of the network but not detected in previous proteomic analyses.

PMID: 31231217 [PubMed]

Challenging barriers to an option for improved provision of enteral nutrition.

J Cyst Fibros. 2019 Jul;18(4):447-449

Authors: Schwarzenberg SJ, Borowitz D, 20 gastroenterologists, 23 CF physicians, 17 CF dietitians and 1 PharmD

PMID: 31230797 [PubMed - in process]

The polysaccharide extracted from the biofilm of Burkholderia multivorans strain C1576 binds hydrophobic species and exhibits a compact 3D-structure.

Int J Biol Macromol. 2019 Jun 20;:

Authors: Bellich B, Distefano M, Syrgiannis Z, Bosi S, Guida F, Rizzo R, Brady JW, Cescutti P

Abstract
Microorganisms often grow in communities called biofilms where cells are imbedded in a complex self-produced biopolymeric matrix composed mainly of polysaccharides, proteins, and DNA. This matrix, together with cell proximity, confers many advantages to these microbial communities, but also constitutes a serious concern when biofilms develop in human tissues or on implanted prostheses. Although polysaccharides are considered the main constituents of the matrices, their specific role needs to be clarified. We have investigated the chemical and morphological properties of the polysaccharide extracted from biofilms produced by the C1576 reference strain of the opportunistic pathogen Burkholderia multivorans, which causes lung infections in cystic fibrosis patients. The aim of the present study is the definition of possible interactions of the polysaccharide and the three-dimensional conformation of its chain within the biofilm matrix. Surface plasmon resonance experiments confirmed the ability of the polysaccharide to bind hydrophobic molecules, due to the presence of rhamnose dimers in its primary structure. In addition, atomic force microscopy studies evidenced an extremely compact three-dimensional structure of the polysaccharide which may form aggregates, suggesting a novel view of its structural role into the biofilm matrix.

PMID: 31229548 [PubMed - as supplied by publisher]

Successful lung transplantation from a hepatitis C RNA-positive donor to a hepatitis C treatment-experienced recipient with cystic fibrosis.

J Thorac Cardiovasc Surg. 2019 May 24;:

Authors: Harano T, Haidar G, Schaheen L, Morrell MR, Pilewski JM, D'Cunha J

PMID: 31229293 [PubMed - as supplied by publisher]

Conversion of human and mouse fibroblasts into lung-like epithelial cells.

Sci Rep. 2019 Jun 21;9(1):9027

Authors: Wong AP, Shojaie S, Liang Q, Xia S, Di Paola M, Ahmadi S, Bilodeau C, Garner J, Post M, Duchesneau P, Waddell TK, Bear CE, Nagy A, Rossant J

Abstract
Cell lineage conversion of fibroblasts to specialized cell types through transdifferentiation may provide a fast and alternative cell source for regenerative medicine. Here we show that transient transduction of fibroblasts with the four reprogramming factors (Oct4, Sox2, Klf4, and c-Myc) in addition to the early lung transcription factor Nkx2-1 (also known as Ttf1), followed by directed differentiation of the cells, can convert mouse embryonic and human adult dermal fibroblasts into induced lung-like epithelial cells (iLEC). These iLEC differentiate into multiple lung cell types in air liquid interface cultures, repopulate decellularized rat lung scaffolds, and form lung epithelia composed of Ciliated, Goblet, Basal, and Club cells after transplantation into immune-compromised mice. As proof-of-concept, differentiated human iLEC harboring the Cystic Fibrosis mutation dF508 demonstrated pharmacological rescue of CFTR function using the combination of lumacaftor and ivacaftor. Overall, this is a promising alternative approach for generation of patient-specific lung-like progenitors to study lung function, disease and future regeneration strategies.

PMID: 31227724 [PubMed - in process]

Effect of Cystic Fibrosis Transmembrane Receptor Modulators on Anthropometric and Growth Parameters in Individuals with Cystic Fibrosis: A Systematic Review (OR36-08-19).

Curr Dev Nutr. 2019 Jun;3(Suppl 1):

Authors: Alvarez J, Bailey J, Bowser E, Farnham K, Sr MM, McDonald C, Padula L, Porco K, Rozga M

Abstract
Objectives: Individuals with cystic fibrosis (CF) are at high risk for malnutrition and poor growth. New drugs that modulate CF transmembrane receptor (CFTR) function are available, although their effects on anthropometric parameters are not clear. This systematic review aimed to determine if various CFTR modulator therapies affect weight and growth parameters in individuals with CF.
Methods: A literature search was conducted using MEDLINE, CINAHL and Embase databases for peer-reviewed, randomized controlled trials (RCT) published from January 2002 to May 2018 that examined the effect of CFTR modulation therapy on weight and growth parameters in children and adults with CF. Articles were screened for relevance, data was extracted and summarized, and risk of bias was assessed to determine the quality of evidence.
Results: A total of 612 original articles were identified, and 13 articles representing 10 RCTs were included in qualitative synthesis and three were included in quantitative synthesis. All but one RCT demonstrated low risk of bias. The targeted class of CFTR mutation varied by RCT. Studies indicated that, in children and adults with CF with class III mutations, ivacaftor treatment alone increased weight and BMI (4 RCTs), but it did not influence growth in individuals with class II (1 RCT) or class IV mutations (1 RCT). Treatment with ivacaftor + lumacaftor in adults homozygous for the F508del CFTR mutation (class II) increased BMI following 24 weeks of treatment (2 RCTs), but there was no effect in adults heterozygous for the mutation treated for 8 weeks (1 RCT). Treatment with lumacaftor + tezacaftor in individuals ages 12-20 years with class II mutations did not change growth parameters (1 RCT).
Conclusions: Effects of CFTR modulators on increasing weight and growth in individuals with CF are likely dependent on the therapy formulation (single vs combination therapy) and the targeted CFTR mutation. Whereas ivacaftor alone increases weight and growth parameters in individuals with class III mutations, ivacaftor + lumacaftor is more effective at increasing BMI in adults homozygous for the class II F508del CFTR mutation. Additional studies are needed to determine effects of other CFTR modulator drugs on growth parameters in individuals with varying CFTR mutations.
Funding Sources: Academy of Nutrition and Dietetics/Medical Nutrition DPG, National Institutes of Health.

PMID: 31225112 [PubMed]

Impacts of Genistein on the Gut Microbiota of a Mouse Model of Cystic Fibrosis (P06-070-19).

Curr Dev Nutr. 2019 Jun;3(Suppl 1):

Authors: Whisner C, Argo K, Sweazea K, Al-Nakkash L

Abstract
Objectives: Dysbiosis of the gut microbiota (GM) is observed in both murine models and humans with cystic fibrosis (CF). Laxative treatments are typically used to improve gastric motility and alleviate gastrointestinal symptoms associated with CF. Genistein, a phytochemical in soy, exerts therapeutic benefits by activating intestinal CFTR ion channels but whether genistein affects the GM in CF is unknown. This study aimed to establish whether dietary genistein alters the GM community structure in a murine model of the most common CF-associated mutation, DF508.
Methods: In this 3-arm parallel randomized intervention, 21-day old DF508 mice (n = 24) were fed standard chow (CHOW; n = 11), CHOW plus Colyte (laxative; n = 7), or CHOW plus 600 mg of dietary genistein (GEN) per kg of diet (n = 6) for 45 days. Fecal pellets from day 45 were frozen at -80˚C and microbial genomic DNA extracted using a DNeasy PowerSoil Kit. The 16S rRNA gene was sequenced via Illumina MiSeq. Sequences were quality checked and denoised using DADA2 and taxonomy determined using the GreenGenes 13.8 database. Treatment differences in the GM were compared using both alpha (within-sample) and beta (between-sample) diversity metrics (Kruskal-Wallis and PERMANOVA tests).
Results: Both observed operational taxonomic units (OTUs) and Shannon's diversity index (alpha diversity richness measures) were greater for CHOW (OTUs: p = < 0.003; Shannon's: p < 0.003) and Colyte (OTUs: p = 0.012; Shannon's: p = 0.006) compared to GEN animals. Colyte resulted in significantly lower species richness (OTUs: p < 0.005) compared to CHOW. Species evenness (Pielou's) did not differ suggesting the importance of species richness over evenness for group differences. Jaccard, Bray-Curtis, unweighted and weighted UniFrac metrics (beta diversity measures) revealed significant group differences (GEN vs. CHOW: p's < 0.004; GEN vs. Colyte: p's < 0.005; CHOW vs. Colyte: p's < 0.006) suggesting that differences in GM community structure were driven by presence/absence, abundance, and phylogeny of taxa.
Conclusions: Colyte and GEN resulted in lower within-sample diversity and significant differences in beta diversity compared to CHOW mice. Species abundance differences between Colyte and GEN groups and how disease prognosis is impacted require further investigation.
Funding Sources: Midwestern University intramural funds (LA) and Arizona State University internal funds (CMW).

PMID: 31224454 [PubMed]

Dietary Macronutrient Distribution and Nutrition-Related Outcomes in Cystic Fibrosis: A Systematic Review (P12-016-19).

Curr Dev Nutr. 2019 Jun;3(Suppl 1):

Authors: Rozga M, Alvarez J, Bowser E, Bailey J, Farnham K, McDonald C, Mangus M, Porco K, Padula L

Abstract
Objectives: Conduct a systematic review (SR) to determine the relationship between dietary macronutrient distribution and nutrition-related outcomes in pediatric and adult participants with cystic fibrosis (CF).
Methods: A literature search of Medline, CINAHL and Embase databases was conducted to identify peer-reviewed articles published from January 2002- May 2018 that examined human participants with CF and addressed the research objective. Articles were screened for relevance, data was extracted and summarized, and risk of bias was assessed by a multidisciplinary team of researchers, practitioners, patient advocates and SR methodologists.
Results: A total of 2409 articles were identified in the search and eight cross-sectional studies and one case-control study met inclusion criteria (N = 4 in pediatrics, N = 4 in adults, N = 1 combined). Evidence quality was low due to weak study designs, small samples size and inconsistent outcome reporting. Available studies did not show statistically significant relationships between dietary macronutrient distribution and lung function (FEV1%) (3 studies) when estimated protein intake ranged from 10-23% of energy, fat intake from 20-46% of energy and carbohydrate intake from 32-67% of energy. Macronutrient distribution was not significantly associated with anthropometric measurements/growth (3 studies), gastrointestinal symptoms (2 studies), glucose fluctuations (1 study) or lipid profile (1 study) in this SR. There were no studies identified examining the relationship between macronutrient distribution and mortality or Quality of Life. Meta-analysis was not possible due to the low quality of data reported.
Conclusions: Recent evidence describing the relationship between dietary macronutrient distribution and nutrition-related outcomes in participants with CF is sparse and low in quality. The evidence reviewed does not suggest that dietary macronutrient distribution is related to key clinical CF outcomes. Higher-quality trials and longitudinal studies are needed to confirm these findings. The need for updated dietary studies is particularly important in light of recent therapeutic advances that are changing the clinical course of individuals with CF.
Funding Sources: Academy of Nutrition and Dietetics and the Medical Nutrition Practice Group DPG.

PMID: 31224441 [PubMed]

Cadmium at Human Dietary Levels Disturbed Homeostasis of Nutritional Metals in Lung (P24-055-19).

Curr Dev Nutr. 2019 Jun;3(Suppl 1):

Authors: Go YM, Smith M, Fernandes J, Orr M, Hu X, Jones D

Abstract
Objectives: Multiple metals are present in the human diet, with some being required as essential inorganic nutrients and all causing toxicity in excess or deficiency. Cadmium (Cd), a toxic heavy metal accumulates in human organs, kidney, liver and lung via food consumption. Our recent mouse studies showed that Cd decreased lung selenium level, and caused metabolic and transcriptomic disruption. However, little is known about the effects of Cd on nutritional metal distribution and subsequent impact on human health. In this study, we examined effects of oral Cd on distribution and interaction of nutritional metals in mouse lung.
Methods: C57BL/6 J mice were treated with Cd at low concentrations (0, 1, 3.3, 10 mg CdCl2/L) by drinking water for 16 weeks. Lungs were collected from mice and lung samples and isolated lung mitochondria were analyzed for 25 inorganic nutritional and toxic environmental metals by inductively coupled plasma mass spectrometry (ICP-MS). In addition, human lung samples from the Emory Transplant Program (Emory IRB 0,0006248) and from International Institute for Advancement of Medicine were analyzed for metals by ICP-MS.
Results: Lungs (n = 50) from mice exposed to Cd in a dose-response study showed that cobalt (Co), vanadium (V), copper (Cu) and molybdenum (Mo) were positively correlated with Cd [Spearman correlation (r); 0.38, 0.31, 0.42, 0.41, P < 0.05], and correlation of V and Cu to Cd were increased in lung mitochondria (r; 0.73, 0.87). The data for human lungs (n = 24) showed that Cd was positively correlated with Co, V and manganese (Mn), r; 0.43, 0.73 and 0.39, P < 0.05) in chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, emphysema, cystic fibrosis, and negatively correlated with magnesium (r = -0.46).
Conclusions: The results show that Co and V each had positive correlations with Cd in human and mouse lung studies, and interactions of metals with Cd were increased in lung mitochondria. Additionally, the results suggest interactions of Cd with Cu, Mo and Mn, which could impact the function of these essential nutrients. Given the range of toxic effects of Cd, the results indicate that studies are needed to understand potential dysfunctions related to disturbed homeostasis of nutritionally essential metals.
Funding Sources: NIEHS R01 ES023485 and NIEHS R21 ES025632.

PMID: 31223986 [PubMed]

Evaluation of Quality of Life in Terms of Sinonasal Symptoms in Children with Cystic Fibrosis.

Biomol Concepts. 2019 Jun 17;10(1):91-98

Authors: Hassanzad M, Derakhshan KF, Ghaffaripour H, Naeini AS, Emami H, Velayati AA

Abstract
Objective Sinusitis is a common complaint in children with cystic fibrosis. However, the actual prevalence of chronic rhinosinusitis and its effect on the quality of life of children have not been well considered. Therefore, the objective of this study was to determine the effect of sinonasal quality of life in children with cystic fibrosis. Materials and methods This study was a diagnostic study performed on 80 children with cystic fibrosis ranging from 2 to 20 years old, who were referred to the cystic fibrosis clinic of Masih Daneshvari Hospital from 2017-2018. The questionnaires used in this study were chronic rhinosinusitis screening questionnaire based on the European task force and the evaluation of the sinonasal quality of life was based on the SN-5 survey. Results Of the 80 patients with fibrosis from 2 to 20 years old who were recruited in the study, 41 patients were female (51.3%) and 39 were male (48.8%). In 61 cases (76.3%), there was no chronic rhinosinusitis and 19 cases (23.8%) had chronic rhinosinusitis. The mean SN-5 score in 19 patients with chronic rhinosinusitis was 3.4105 and the mean score of patients without rhinosinusitis was 1.8426, with a P-value of 0.000. The mean SN-5 score was significant between the two groups. In patients with nasal congestion, there was a significant difference in quality of life factors such as sinus infection, nasal obstruction, and allergy symptoms (P<0.001). In patients with facial pain, there was a significant difference in quality of life factors such as sinus infection, nasal obstruction, allergic symptoms, and physical activity limitation (P <0.001). There was also a significant difference in the quality of life factors such as sinus infection, nasal obstruction, and allergy symptoms in patients with postnasal drip (P <0.001). Conclusion In children with cystic fibrosis, the quality of life of sinonasal has a significant relationship with absence of chronic rhinosinusitis. This study showed that children with chronic renosinusitis have significantly lower quality of sinonasal life than children with chronic rhinosinusitis. The results demonstrated that quality of life scores in sinus infections, nasal obstruction, and allergy symptoms were significantly higher in patients with chronic rhinosinusitis than in those without chronic rhinosinusitis. The findings of this study are important for improving children's health related quality of life, as it leads to promoting communication between the patient and the health care provider, identifying overlooked problems, monitoring the progress of the disease and the burden of treatment, and promoting interventions in the daily life of patients.

PMID: 31226098 [PubMed - in process]

Inhaled corticosteroids prescriptions increased in the ED for recurrent asthma exacerbations by automated electronic reminders in the ED.

J Asthma. 2019 Jun 21;:1-8

Authors: Farmer A, Mirea L, Carter J, Rank M, Bulloch B, Vaidya V, Drewek R

Abstract
Objective: The objective of this study was to evaluate the impact of an electronic alert on the prescription rate of inhaled corticosteroids (ICS) by ED providers for poorly controlled persistent asthmatic children. Methods: Study subjects included asthmatic patients age 4-18 presenting to the ED at Phoenix Children's Hospital between February 9, 2018 and December 4, 2018, with a history of at least 2 previous ED visits for acute exacerbation of asthma within 365 days, no active ICS prescription within 90 days, and free from developmental delay, bronchopulmonary dysplasia due to prematurity, cystic fibrosis, sickle cell disease, and/or interstitial ling disease. Patients meeting these criteria triggered an electronic alert prompting the medical provider to prescribe ICS or indicate reason for not prescribing. Instruction on the alert was provided to ED attending physicians and residents by email and through several educational sessions held prior to the implementation. Results: Among 62 patients without prior ICS who were discharged home from the ED, ICS was prescribed for 48 (77%). No statistically significant differences were detected in baseline characteristics between patients discharged home from the ED with and without ICS prescription. While ICS was prescribed by a larger proportion of physicians (56%) compared to residents (42%), statistical significance was not reached. For the 14 (33%) patients who were discharged home without ICS, no reason was provided to indicate why ICS were not prescribed. Conclusion: An electronic alert incorporated into the ED workflow to populate a discharge order set is effective to initiate asthma controller medication for poorly controlled pediatric patients. Additional data describing reasons for not prescribing ICS can further refine recommendations for ICS prescriptions, and provide a comprehensive strategy to support clinical decision for pediatric asthma control in acute care settings.

PMID: 31226000 [PubMed - as supplied by publisher]

A dedicated diribonucleotidase resolves a key bottleneck as the terminal step of RNA degradation.

Elife. 2019 Jun 21;8:

Authors: Kim SK, Lormand JD, Weiss CA, Eger KA, Turdiev H, Turdiev A, Winkler WC, Sondermann H, Lee VT

Abstract
Degradation of RNA polymers, an ubiquitous process in all cells, is catalyzed by specific subsets of endo- and exoribonucleases that together recycle RNA fragments into nucleotide monophosphate. In γ-proteobacteria, 3-'5' exoribonucleases comprise up to eight distinct enzymes. Among them, Oligoribonuclease (Orn) is unique as its activity is required for clearing short RNA fragments, which is important for cellular fitness. However, the molecular basis of Orn's unique cellular function remained unclear. Here we show that Orn exhibits exquisite substrate preference for diribonucleotides. Crystal structures of substrate-bound Orn reveal an active site optimized for diribonucleotides. While other cellular RNases process oligoribonucleotides down to diribonucleotide entities, Orn is the one and only diribonucleotidase that completes the terminal step of RNA degradation. Together, our studies indicate RNA degradation as a step-wise process with a dedicated enzyme for the clearance of a specific intermediate pool, diribonucleotides, that affects cellular physiology and viability.

PMID: 31225796 [PubMed - as supplied by publisher]