Psychological distress in adults with and without cystic fibrosis during the COVID-19 lockdown.

J Cyst Fibros. 2020 Dec 23;:

Authors: Ciprandi R, Bonati M, Campi R, Pescini R, Castellani C

Abstract
BACKGROUND: Hundreds of papers have been published on the COVID-19 pandemic, and several of them on psychological themes connected with it, but very little is so far known on how adult patients with Cystic Fibrosis (pwCFs) are coping with this dramatic event.
METHODS: An online questionnaire was developed according to the Italian validated COVID-19 Peritraumatic Distress Index (CPDI) and addressed to the general population (GP). A similar questionnaire, augmented with CF specific questions, targeted pwCFs. The two web-based surveys were accessible for some weeks during the lockdown mandated by the Italian government.
RESULTS: The CF questionnaire was completed by 712 adult pwCFs (422 females), matched for sex and age with a 1/5 ratio to GP questionnaire respondents. Mild or medium distress affected 40.2% of pwCFs and 43.9% of GP controls, severe distress 5.3% of pwCFs and 6.2% of GP controls. The level of psychological distress was not correlated with the degree of pulmonary function impairment. When symptoms of anxiety and depression, and physical manifestations were independently analyzed, the control group featured a 55% higher level of mild-moderate anxiety symptoms. Signs of psychological distress, symptoms of anxiety and depression, and physical manifestations were significantly more frequent in female pwCFs compared to males, similarly to GP.
CONCLUSION: Adult pwCFs seem to have equal, and in some domains, lower levels of psychological distress than GP controls. This might be sustained by lifelong experiences in coping with the demands of their chronic disease. These results may orient future psychological interventions.

PMID: 33384222 [PubMed - as supplied by publisher]

Building a better biofilm - Formation of in vivo-like biofilm structures by Pseudomonas aeruginosa in a porcine model of cystic fibrosis lung infection.

Biofilm. 2020 Dec;2:100024

Authors: Harrington NE, Sweeney E, Harrison F

Abstract
Pseudomonas aeruginosa biofilm infections in the cystic fibrosis (CF) lung are highly resistant to current antimicrobial treatments and are associated with increased mortality rates. The existing models for such infections are not able to reliably mimic the clinical biofilms observed. We aimed to further optimise an ex vivo pig lung (EVPL) model for P. aeruginosa CF lung infection that can be used to increase understanding of chronic CF biofilm infection. The EVPL model will facilitate discovery of novel infection prevention methods and treatments, and enhanced exploration of biofilm architecture. We investigated purine metabolism and biofilm formation in the model using transposon insertion mutants in P. aeruginosa PA14 for key genes: purD, gacA and pelA. Our results demonstrate that EVPL recapitulates a key aspect of in vivo P. aeruginosa infection metabolism, and that the pathogen forms a biofilm with a clinically realistic structure not seen in other in vitro studies. Two pathways known to be required for in vivo biofilm infection - the Gac regulatory pathway and production of the Pel exopolysaccharide - are essential to the formation of this mature, structured biofilm on EVPL tissue. We propose the high-throughput EVPL model as a validated biofilm platform to bridge the gap between in vitro work and CF lung infection.

PMID: 33381751 [PubMed]

A bird eye view on cystic fibrosis: An underestimated multifaceted chronic disorder.

Life Sci. 2020 Dec 28;:118959

Authors: Kotnala S, Dhasmana A, Kashyap VK, Chauhan SC, Yallapu MM, Jaggi M

Abstract
Cystic fibrosis (CF) is an autosomal recessive disease which involves the mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CF involves the inflammatory processes and is considered as a multisystem disorder that is not confined to lungs, but it also affects other vital organs that leads to numerous co-morbidities. The respiratory disorder in the CF results in mortality and morbidity which is characterized by series of serious events involving mucus hypersecretion, microbial infections, airways obstruction, inflammation, destruction of epithelium, tissue remodeling and terminal lung diseases. Mucins are the high molecular weight glycoproteins important for the viscoelastic properties of the mucus, play a significant role in the disease mechanisms. Determining the functional association between the CFTR and mucins might help to identify the putative target for specific therapeutic approach. In fact, furin enzyme which helps in the entry of novel COVID-19 virus into the cell, is upregulated in CF and this can also serve as a potential target for CF treatment. Moreover, the use of nano-formulations for CF treatment is an area of research being widely studied as they have also demonstrated promising outcomes. The in-depth knowledge of non-coding RNAs like miRNAs and lncRNAs and their functional association with CFTR gene expression and mutation can provide a different range of opportunity to identify the promising therapeutic approaches for CF.

PMID: 33383045 [PubMed - as supplied by publisher]

Advanced Lung Disease in Patients with Cystic Fibrosis Is Associated with Low Diffusion capacity.

Isr Med Assoc J. 2020 Dec;22(12):770-774

Authors: Vilozni D, Dagan A, Sarouk I, Bar-Aluma BE, Ashkenazi M, Bezalel Y, Efrati O

Abstract
BACKGROUND: The single-breath diffusing capacity of the lungs (DLCOSB) test measures the extent to which carbon monoxide (CO) passes from the lung air sacs into the blood. The accessible alveolar volume (VASB) is measured by inert gas during a 10-second period. The single-breath transfer coefficient of the lung for carbon monoxide (KCOSB) is the DLCOSB divided by VASB. Cystic fibrosis (CF) disease comprises progressive airway obstruction with bronchiectasis and parenchyma fibrosis. Yet, the KCOSB appears insignificant in the assessment of pulmonary function in CF.
OBJECTIVES: To challenge the precision of normal KCOSB in CF.
METHODS: The authors collected pulmonary function tests (PFT) data from 74 confirmed CF patients (mean age 26 ± 10 years) with various levels of pulmonary disease severity. Tests included spirometry, DLCOBP, and body plethysmography (BP). Anatomical dead space was calculated by deducting anatomical dead space from total lung capacity TLC(BP) to establish alveolar volume (VABP) and to determine KCOBP. We also included individual data of arterial pCO2 blood-gas level.
RESULTS: KCOSB values were normal or higher in most patients, regardless of patient FEV1 value (R2 = 0.2204; P < 0.02). In contrast, the measurements of KCOBP were low corresponding with low FEV1 values, and negatively correlated with the elevation of trapped air and pCO2 levels (R2 = 0.1383; P = 0.0133, P > 0.05, respectively).
CONCLUSIONS: The 10- second perfusion time of the inert gas during DLCOSB represent the communicative alveolar volume in CF patients with advanced pulmonary disease. The findings justify the use of DLCOSB with the deterioration of FEV1 and elevation of pCO2 levels.

PMID: 33381950 [PubMed - in process]

Men's health in the modern era of cystic fibrosis.

J Cyst Fibros. 2020 Dec 26;:

Authors: Khan FN, Tangpricha V, Hughan KS, Jain R, Ladores SL, Taylor-Cousar JL, West NE, Montemayor K, Sawicki GS, Uluer A, Aitken ML, Shnorhavorian M, Bray LA, Kvam C, Stalvey M, Kazmerski TM

PMID: 33376059 [PubMed - as supplied by publisher]

Andrological findings in infertile men with two (biallelic) CFTR mutations: results of a multicentre study in Germany and Austria comprising 71 patients.

Hum Reprod. 2020 Dec 29;:

Authors: Rudnik-Schöneborn S, Messner M, Vockel M, Wirleitner B, Pinggera GM, Witsch-Baumgartner M, Murtinger M, Kliesch S, Swoboda M, Sänger N, Zschocke J, Tüttelmann F

Abstract
STUDY QUESTION: When should cystic fibrosis transmembrane conductance regulator (CFTR) mutation analysis be recommended in infertile men based on andrological findings?
SUMMARY ANSWER: CFTR mutation analysis is recommended in all men with unexplained azoospermia in the presence of normal gonadotropin levels.
WHAT IS KNOWN ALREADY: While 80-97% of men with congenital bilateral absence of the vas deferens (CBAVD) are thought to carry CFTR mutations, there is uncertainty about the spectrum of clinical and andrological abnormalities in infertile men with bilallelic CFTR mutations. This information is relevant for evidence-based recommendations to couples requesting assisted reproduction.
STUDY DESIGN, SIZE, DURATION: We studied the andrological findings of patients with two CFTR mutations who were examined in one of the cooperating fertility centres in Germany and Austria. In the period of January till July 2019, the completed and anonymized data sheets of 78 adult male patients were returned to and analysed by the project leader at the Institute of Human Genetics in Innsbruck, Austria.
PARTICIPANTS/MATERIALS, SETTING, METHODS: Minimum study entry criteria were the presence of two (biallelic) CFTR mutations and results of at least one semen analysis. Andrological assessments were undertaken by standardized data sheets and compared with normal reference values. Seventy-one patients were eligible for the study (n = 30, 42% from Germany, n = 26, 37% from Austria, n = 15, 21% other nations).
MAIN RESULTS AND THE ROLE OF CHANCE: Gonadotropin levels (FSH, LH) were normal, 22% of patients had reduced testosterone values. Mean right testis volume was 23.38 ml (SD 8.77), mean left testis volume was 22.59 ml (SD 8.68) and thereby statistically increased compared to normal (P < 0.01). although the means remained in the reference range of 12-25 ml. Semen analysis revealed azoospermia in 70 of 71 (99%) patients and severe oligozoospermia <0.1 × 106/ml in one patient. Four semen parameters, i.e. ejaculate volume, pH, α-glucosidase and fructose values, were significantly reduced (P < 0.01). Only 18% of patients had a palpatory and sonographically diagnosed CBAVD, while in 31% the diagnosis of CBAVD was uncertain, in 12% patients, the vas deferens was present but hypoplastic, and in 39% the vas deferens was normally present bilaterally. Seminal vesicles were not detectable in 37% and only unilaterally present in 37% of patients. Apart from total testes volume, clinical findings were similar in patients with two confirmed pathogenic CFTR mutations (Group I) compared with patients who carried one pathogenic mutation and one CFTR variant of unknown significance (Group II).
LIMITATIONS, REASONS FOR CAUTION: We could not formally confirm the in trans position of genetic variants in most patients as no family members were available for segregation studies. Nonetheless, considering that most mutations in our study have been previously described without other rare variants in cis, and in view of the compatible andrological phenotype, it is reasonable to assume that the biallelic genotypes are correct.
WIDER IMPLICATIONS OF THE FINDINGS: Our study reveals that CFTR mutation analysis has a broader indication than just the absence of the vas deferens. We recommend to completely sequence the CFTR gene if there is a suspicion of obstructive azoospermia, and to extend this analysis to all patients with unexplained azoospermia in the presence of normal gonadotropin levels.
STUDY FUNDING/COMPETING INTEREST(S): German Research Foundation Clinical Research Unit 'Male Germ Cells: from Genes to Function' (DFG CRU326, grants to F.T.). There are no conflicts of interest to declare.
TRIAL REGISTRATION NUMBER: N/A.

PMID: 33374015 [PubMed - as supplied by publisher]

SARS-CoV-2 related pneumonia in an adult with cystic fibrosis: natural favourable clinical course or effective therapy?

Monaldi Arch Chest Dis. 2020 Dec 23;90(4):

Authors: Giglia M, Beci G, Rosselli Del Turco E, Guardigni V, Amedeo A, Cucchetto G, Verucchi G, Cipolli M, Calza L, Viale P

Abstract
We report the case of a man affected by cystic fibrosis who developed a severe SARS-CoV-2 related pneumonia in March 2020. In addition to lopinavir/ritonavir and hydroxychloroquine, he was treated with two doses of tocilizumab, displaying a significant clinical improvement. This is the first case described in the literature of an adult patient affected by cystic fibrosis who received tocilizumab for COVID-19, with documented total recovery, also assessed by a spirometry.

PMID: 33372741 [PubMed - in process]

Carbamazepine-induced sperm disorders can be associated with the altered expressions of testicular KCNJ11/miR-let-7a and spermatozoal CFTR/miR-27a.

Andrologia. 2020 Dec 28;:e13954

Authors: Tektemur A, Etem Önalan E, Kaya Tektemur N, Dayan Cinkara S, Kılınçlı Çetin A, Tekedereli İ, Kuloğlu T, Türk G

Abstract
Male infertility is a global health problem, and the underlying molecular mechanisms are not clearly known. Ion channels and microRNAs (miRNAs), known to function in many vital functions in cells, have been shown to play a significant role in male infertility through changes in their expressions. The study aimed to evaluate the alterations of testicular and/or spermatozoal potassium voltage-gated channel subfamily J member 11 (KCNJ11), Cystic fibrosis transmembrane conductance regulator (CFTR), miR-let-7a and miR-27a expressions in carbamazepine-related male infertility. Here, we showed that carbamazepine reduced sperm motility, increased abnormal sperm morphology, and impaired hormonal balance as well as increased relative testis weight and decreased relative seminal vesicle weight. On the other hand, downregulated KCNJ11 and upregulated miR-let-7a expressions were determined in testis (p < .05). Also, downregulated KCNJ11 and upregulated CFTR and miR-27a expressions were found in spermatozoa (p < .05). Interestingly, altered testicular KCNJ11 and miR-let-7a expressions were correlated with decreased sperm motility and elevated sperm tail defect. Besides, spermatozoal CFTR and miR-27a expressions positively correlated with sperm tail defects. The results indicated a significant relationship between ion channel and/or miRNA expression alterations and impaired sperm parameters due to carbamazepine usage.

PMID: 33372325 [PubMed - as supplied by publisher]

Physiometric Response to High-Flow Nasal Cannula Support in Acute Bronchiolitis.

Hosp Pediatr. 2020 Dec 28;:

Authors: Sochet AA, Nunez M, Maamari M, McKinley S, Morrison JM, Nakagawa TA

Abstract
OBJECTIVES: To describe the rate of high-flow nasal cannula (HFNC) nonresponse and paired physiometric responses (changes [∆] in heart rate [HR] and respiratory rate [RR]) before and after HFNC initiation in hospitalized children with bronchiolitis.
METHODS: We performed a single-center, prospective descriptive study in a PICU within a quaternary referral center, assessing children aged ≤2 years admitted for bronchiolitis on HFNC from November 2017 to March 2020. We excluded for cystic fibrosis, airway anomalies, pulmonary hypertension, tracheostomy, neuromuscular disease, congenital heart disease, or preadmission intubation. Primary outcomes were paired ∆ and %∆ in HR and RR before and after HFNC initiation. Secondary outcomes were HFNC nonresponse rate (ie, intubation or transition to noninvasive positive pressure ventilation). Analyses included χ2, Student's t, Wilcoxon rank, and paired testing.
RESULTS: Of the 172 children studied, 56 (32.6%) experienced HFNC nonresponse at a median of 14.4 (interquartile range: 4.8-36) hours and 11 (6.4%) were intubated. Nonresponders had a greater frequency of bacterial pneumonia, but otherwise no major differences in demographics, comorbidities, or viral pathogens were noted. Responders experienced reductions in both %ΔRR (-17.1% ± 15.8% vs +5.3% ± 22.3%) and %ΔHR (-6.5% ± 10.5% vs 0% ± 10.9%) compared with nonresponders.
CONCLUSIONS: In this prospective, observational cohort study, we provide baseline data describing expected physiologic changes after initiation of HFNC for children admitted to the PICU for bronchiolitis. In our descriptive analysis, patients with comorbid bacterial pneumonia appear to be at additional risk for subsequent HFNC nonresponse.

PMID: 33372047 [PubMed - as supplied by publisher]

Update on Respiratory Fungal Infections in Cystic Fibrosis Lung Disease and after Lung Transplantation.

J Fungi (Basel). 2020 Dec 21;6(4):

Authors: Renner S, Nachbaur E, Jaksch P, Dehlink E

Abstract
Cystic fibrosis is the most common autosomal-recessive metabolic disease in the Western world. Impaired trans-membrane chloride transport via the cystic fibrosis transmembrane conductance regulator (CFTR) protein causes thickened body fluids. In the respiratory system, this leads to chronic suppurative cough and recurrent pulmonary infective exacerbations, resulting in progressive lung damage and respiratory failure. Whilst the impact of bacterial infections on CF lung disease has long been recognized, our understanding of pulmonary mycosis is less clear. The range and detection rates of fungal taxa isolated from CF airway samples are expanding, however, in the absence of consensus criteria and univocal treatment protocols for most respiratory fungal conditions, interpretation of laboratory reports and the decision to treat remain challenging. In this review, we give an overview on fungal airway infections in CF and CF-lung transplant recipients and focus on the most common fungal taxa detected in CF, Aspergillus fumigatus, Candida spp., Scedosporium apiospermum complex, Lomentospora species, and Exophiala dermatitidis, their clinical presentations, common treatments and prophylactic strategies, and clinical challenges from a physician's point of view.

PMID: 33371198 [PubMed]

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Influence of pre-diabetic and pancreatic exocrine states on pulmonary and nutritional status in adults with Cystic Fibrosis.

J Cyst Fibros. 2020 Dec 19;:

Authors: Potter KJ, Boudreau V, Shohoudi A, Mailhot M, Tremblay F, Lavoie A, Carricart M, Senior PA, Rabasa-Lhoret R

Abstract
BACKGROUND: In 1992, a landmark study demonstrated clinical deterioration in respiratory function and nutritional status prior to the onset of cystic fibrosis-related diabetes (CFRD). We re-evaluated this outcome.
METHODS: The Montreal Cystic Fibrosis Cohort is a prospective CFRD screening study. We performed a 6-year retrospective analysis of nutritional parameters and FEV1 (%) in subjects who developed incident CFRD and in controls who maintained normoglycemia (NG). In the former group, data was collected over 6 years prior to diabetes onset.
RESULTS: Subjects (n = 86) had a mean age of 31.7 ± 8.1 years, BMI of 23.0 ± 4.0 kg/m2, and FEV1% of 70.1 ± 24.2%. Eighty-one percent had pancreatic insufficiency (PI). Patients were grouped as follows: NG+PS (pancreatic sufficient) (n = 16), NG+PI (pancreatic insufficient) (n = 21), CFRD+PS (n = 3) and CFRD+PI (n = 46). At their most recent screen NG+PS subjects had significantly greater BMI, as compared to NG+PI and CFRD+PI groups (26.2 ± 3.6 kg/m2 vs 22.6 ± 4.2 kg/m2 vs 22.1 ± 3.5 kg/m2, p = 0.0016). FEV1 was significantly greater in the NG+PS group (91.5 ± 16.8% vs 67.8 ± 25.3% vs 63.5 ± 22.2%, p = 0.0002). The rates of change in weight, BMI, fat mass (%), and FEV1 prior to the most recent visit (NG+PS, NG+PI groups) or to the diagnosis of de novo CFRD were similar between groups.
CONCLUSION: In a contemporary context, CFRD onset is not preceded by deterioration in BMI, fat mass, or pulmonary function. Low BMI and FEV1 are more closely associated with PI than a pre-diabetic state.

PMID: 33353861 [PubMed - as supplied by publisher]

Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: A two center case series.

J Cyst Fibros. 2020 Dec 19;:

Authors: O'Connor KE, Goodwin DL, NeSmith A, Garcia B, Mingora C, Ladores SL, Rowe SM, Krick S, Solomon GM

Abstract
Infertility and subfertility are commonly faced by females with cystic fibrosis (FwCF) and resulting in decreased contraceptive use and increased utilization of reproductive technologies. Elexacaftor-tezacaftor-ivacaftor (ETI) is a CFTR modulator that affects common causes of subfertility. Two CF centers conducted a retrospective chart review on females with CF who were receiving ETI and became pregnant. We analyzed obstetrical-gynecological history, genotype, and clinical response to ETI therapy. Fourteen FwCF on ETI became pregnant. Half (7) of the FwCFs were previously attempting to conceive, but only three were using contraceptives. Four FwCF had a history of infertility; two were reconsidering use of reproductive technologies (IUI). Patients achieved conception at mean 8 weeks after initiating ETI. ETI may lessen CF-associated factors that affect fertility; however, its exact mechanism is unknown. This warrants counseling on contraceptive use and family planning prior to initiation of therapy and at routine intervals while utilizing ETI.

PMID: 33353860 [PubMed - as supplied by publisher]

Appraisal of Clinical Practice Guideline: Physiotherapy for cystic fibrosis in Australia and New Zealand.

J Physiother. 2020 Dec 19;:

Authors: Johnston LM

PMID: 33353829 [PubMed - as supplied by publisher]

Skin Metabolomics.

Trends Endocrinol Metab. 2020 Dec 19;:

Authors: Elpa DP, Chiu HY, Wu SP, Urban PL

Abstract
Skin retains numerous low-molecular-weight compounds (metabolites). Some of these compounds fulfill specific physiological roles, while others are by-products of metabolism. The skin surface can be sampled to detect and quantify skin metabolites related to diseases. Miniature probes have been developed to detect selected high-abundance metabolites secreted with sweat. To characterize a broad spectrum of skin metabolites, specimens are collected with one of several available methods, and the processed specimens are analyzed by chromatography, mass spectrometry (MS), or other techniques. Diseases for which skin-related biomarkers have been found include cystic fibrosis (CF), psoriasis, Parkinson's disease (PD), and lung cancer. To increase the clinical significance of skin metabolomics, it is desirable to verify correlations between metabolite levels in skin and other biological tissues/matrices.

PMID: 33353809 [PubMed - as supplied by publisher]

Congenital absence of the vas deferens: Cystic fibrosis transmembrane regulatory gene mutations.

Best Pract Res Clin Endocrinol Metab. 2020 Dec 01;:101476

Authors: Wong R, Gu K, Ko Y, Patel P

Abstract
Congenital absence of the vas deferens (CAVD) is a rare genetic condition first discovered in the mid-18th century related to mutations in the cystic fibrosis transmembrane regulatory genes. The condition is typically found during work-up of male infertility, and the majority of cases can be diagnosed with complete history and physical examination and pertinent investigations. The condition can be separated into three subcategories, and genetic advances have led to a much better understanding behind the disease, its pathogenesis, and options for treatment. In this review, we discuss the genetics, pathogenesis, embryology, and diagnosis of treatment of CAVD. Future work in this area likely will aim to better understand the epigenetic factors that influence the development of the condition in order to identify potential upstream therapeutic targets.

PMID: 33353780 [PubMed - as supplied by publisher]

Clinical Biofilm Ring Test® Reveals the Potential Role of β-Lactams in the Induction of Biofilm Formation by P. aeruginosa in Cystic Fibrosis Patients.

Pathogens. 2020 Dec 19;9(12):

Authors: Olivares E, Tasse J, Badel-Berchoux S, Provot C, Prévost G, Bernardi T

Abstract
Biofilms are characterized by high tolerance to antimicrobials. However, conventional antibiograms are performed on planktonic microorganisms. Through the clinical Biofilm Ring Test® (cBRT), initially aimed to measure the adhesion propensity of bacteria, we discerned a variable distribution of biofilm-producer strains among P. aeruginosa samples isolated from expectorations of cystic fibrosis (CF) patients. Despite a majority of spontaneous adherent isolates, few strains remained planktonic after 5 h of incubation. Their analysis by an adapted protocol of the cBRT revealed an induction of the biofilm early formation by sub-inhibitory doses of β-lactams. Microscopic observations of bacterial cultures stained with Syto 9/Propidium Iodide (PI) confirmed the ability of antimicrobials to increase either the bacterial biomass or the biovolume occupied by induced sessile cells. Finally, the cBRT and its derivatives enabled to highlight in a few hours the potential inducer property of antibiotics on bacterial adhesion. This phenomenon should be considered carefully in the context of CF since patients are constantly under fluctuating antimicrobial treatments. To conclude, assays derived from the Biofilm Ring Test® (BRT) device, not only define efficient doses preventing biofilm formation, but could be useful for the antimicrobial selection in CF, to avoid inducer molecules of the early biofilm initiation.

PMID: 33352641 [PubMed]

Care of Cystic Fibrosis Children in COVID-19 Pandemic.

Turk Thorac J. 2020 Nov;21(6):461-462

Authors: Kumar P, Goyal JP

PMID: 33352105 [PubMed - as supplied by publisher]

Results of Tobramycin Inhalation Therapy in Patients with Noncystic Fibrosis Bronchiectasis with Pseudomonas aeruginosa Colonization: Real Life Management.

J Aerosol Med Pulm Drug Deliv. 2020 Dec 22;:

Authors: Tanriverdi E, Yildirim BZ, Gul S, Ugur Chousein EG, Turan D, Çınarka H, Özgül MA, Cetinkaya E

Abstract
Background: Inhaled antibiotics for treating bronchiectasis have been investigated in the cystic fibrosis population since 1981 and long-term clinical benefits have been reported. However, studies on noncystic fibrosis bronchiectasis (NCFB) have only been performed more recently. Owing to limited evidence, inhaled antibiotics are not currently approved for treating NCFB by the U.S. Food and Drug Administration and the European Medicines Agency. The aim of this study was to evaluate the efficacy and safety of tobramycin inhalation therapy in patients with bronchiectasis with Pseudomonas aeruginosa (PA) colonization. Methods: In this retrospective cross-sectional study, NCFB patients who were Pseudomonas positive on three consecutive cultures 1 month apart and receiving tobramycin inhalation therapy were evaluated. Evaluation of the following parameters was done in this study: age, gender, smoking history, symptoms, pulmonary function test results, sputum culture results, tobramycin treatment duration, side effects of tobramycin and response evaluation, and hospital admissions before and after treatment. Treatment with 300 mg tobramycin through nebulizer twice daily for 28 days on-off cycles for a total of 6 months was considered to be one treatment period. The approvals for the study were received by the local ethics committee and institutional review board. Results: Of the 27 patients, 21 patients completed the first period, 7 patients completed the second period, 4 patients completed the third period, and 1 patient completed the fourth period. Sputum culture was negative in 10 (47.6%) of the 21 patients who completed the first period. Decreased sputum purulence and quantity, dyspnea, and cough were observed during treatment. The frequency of hospitalizations before treatment was 1.24 ± 1.36, whereas after treatment, it decreased to 0.52 ± 0.91, this difference was statistically significant (p = 0.019). The most common side effect was increased dyspnea after nebulization in five patients. Conclusion: Tobramycin inhalation appears to be a well-tolerated treatment in patients with PA colonization with bronchiectasis. This treatment may decrease the hospitalization rates and improve the symptoms.

PMID: 33351705 [PubMed - as supplied by publisher]

Caring for Adolescents with Cystic Fibrosis, in Portugal: The Nurse's Role.

Compr Child Adolesc Nurs. 2020 Dec 22;:1-9

Authors: Reisinho MDC, Gomes BP, Carvalho F, Borges E

Abstract
This study aimed to describe the nurse's role in the transition processes of adolescents with cystic fibrosis and their parents. Cystic fibrosis is a multisystem, life-shortening genetic disease, caused by malfunction of the protein-encoding gene Cystic fibrosis transmembrane conductance regulator, characterized by a disturbance of external secretion glands. Moreover, the potential presence of respiratory, gastrointestinal, pancreatic, and reproductive-related symptomatology in these patients can add a substantial burden to the disease. In Portugal, the prevalence rate is 1:7,963 of newborns and the life expectancy is around 40 years. Because of the disease multiple-related symptoms, sometimes disabling, health teams include different professionals, with nurses being the main responsible for caring for adolescents with Cystic Fibrosis and providing guidance to their parents. A qualitative paradigm of phenomenological type was designed. A total of 20 semi-structured interviews were conducted with participants selected through the snowball technique. Content analysis was performed as proposed by Bardin. The average age of participants ranged between 28 and 53 years, and the average time of professional exercise ranged between 5 and 27 years. From the analysis of the interviews, the category Nurse's Role emerged, followed by the subcategories, identify needs, caring, communicate, train, continuity of care, teamwork, care partnership, promoting standardization. Nurses identified the nature of the nursing role as a strong determinant to facilitate monitoring and intervention processes for adolescents with cystic fibrosis and their parents. Nursing therapeutics aim to promote, prevent, and effectively contribute to the development process and recovery of stability and well-being of adolescents with cystic fibrosis and their parents.

PMID: 33351649 [PubMed - as supplied by publisher]