Cystic-fibrosis-related diabetes: time for oral drugs?

Lancet Diabetes Endocrinol. 2017 Nov 30;:

Authors: Moran A

PMID: 29199114 [PubMed - as supplied by publisher]

Generation of Induced Progenitor-like Cells from Mature Epithelial Cells Using Interrupted Reprogramming.

Stem Cell Reports. 2017 Nov 27;:

Authors: Guo L, Karoubi G, Duchesneau P, Shutova MV, Sung HK, Tonge P, Bear C, Rogers I, Nagy A, Waddell TK

Abstract
A suitable source of progenitor cells is required to attenuate disease or affect cure. We present an "interrupted reprogramming" strategy to generate "induced progenitor-like (iPL) cells" using carefully timed expression of induced pluripotent stem cell reprogramming factors (Oct4, Sox2, Klf4, and c-Myc; OSKM) from non-proliferative Club cells. Interrupted reprogramming allowed controlled expansion yet preservation of lineage commitment. Under clonogenic conditions, iPL cells expanded and functioned as a bronchiolar progenitor-like population to generate mature Club cells, mucin-producing goblet cells, and cystic fibrosis transmembrane conductance regulator (CFTR)-expressing ciliated epithelium. In vivo, iPL cells can repopulate CFTR-deficient epithelium. This interrupted reprogramming process could be metronomically applied to achieve controlled progenitor-like proliferation. By carefully controlling the duration of expression of OSKM, iPL cells do not become pluripotent, and they maintain their memory of origin and retain their ability to efficiently return to their original phenotype. A generic technique to produce highly specified populations may have significant implications for regenerative medicine.

PMID: 29198829 [PubMed - as supplied by publisher]

Can Tangier disease cause male infertility? A case report and an overview on genetic causes of male infertility and hormonal axis involved.

Mol Genet Metab. 2017 Nov 26;:

Authors: Stocchi L, Giardina E, Varriale L, Sechi A, Vagnini A, Parri G, Valentini M, Capalbo M

Abstract
Tangier disease is an autosomal recessive disorder caused by mutations in the ABCA1 gene and characterized by the accumulation of cholesteryl ester in various tissues and a near absence of high-density lipoprotein. The subject in this investigation was a 36-year-old Italian man with Tangier disease. He and his wife had come to the In Vitro Fertilization Unit, Pesaro Hospital (Azienda Ospedaliera Ospedali Riuniti Marche Nord) seeking help regarding fertility issues. The man was diagnosed with severe oligoasthenoteratozoospermia. Testosterone is the sex hormone necessary for spermatogenesis and cholesterol is its precursor; hence, we hypothesized that the characteristic cholesterol deficiency in Tangier disease patients could compromise their fertility. The aim of the study was to therefore to determine if there is an association between Tangier disease and male infertility. After excluding viral, infectious, genetic and anatomical causes of the subject's oligoasthenoteratozoospermia, we performed a hormonal analysis to verify our hypothesis. The patient was found to be negative for frequent bacteria and viruses. The subject showed a normal male karyotype and tested negative for Yq microdeletions and Cystic Fibrosis Transmembrane Conductance Regulator gene mutations. A complete urological examination was performed, and primary hypogonadism was also excluded. Conversely, hormonal analyses showed that the subject had a high level of follicle stimulating hormone and luteinizing hormone, low total testosterone and a significant decline in inhibin B. We believe that the abnormally low cholesterol levels typically found in subjects with Tangier disease may result in a reduced testosterone production which in turn could affect the hormonal axis responsible for spermatogenesis leading to a defective maturation of spermatozoa.

PMID: 29198592 [PubMed - as supplied by publisher]

Comorbidities and the risk of mortality in patients with bronchiectasis: an international multicentre cohort study.

Lancet Respir Med. 2016 Dec;4(12):969-979

Authors: McDonnell MJ, Aliberti S, Goeminne PC, Restrepo MI, Finch S, Pesci A, Dupont LJ, Fardon TC, Wilson R, Loebinger MR, Skrbic D, Obradovic D, De Soyza A, Ward C, Laffey JG, Rutherford RM, Chalmers JD

Abstract
BACKGROUND: Patients with bronchiectasis often have concurrent comorbidities, but the nature, prevalence, and impact of these comorbidities on disease severity and outcome are poorly understood. We aimed to investigate comorbidities in patients with bronchiectasis and establish their prognostic value on disease severity and mortality rate.
METHODS: An international multicentre cohort analysis of outpatients with bronchiectasis from four European centres followed up for 5 years was done for score derivation. Eligible patients were those with bronchiectasis confirmed by high-resolution CT and a compatible clinical history. Comorbidity diagnoses were based on standardised definitions and were obtained from full review of paper and electronic medical records, prescriptions, and investigator definitions. Weibull parametric survival analysis was used to model the prediction of the 5 year mortality rate to construct the Bronchiectasis Aetiology Comorbidity Index (BACI). We tested the BACI as a predictor of outcomes and explored whether the BACI added further prognostic information when used alongside the Bronchiectasis Severity Index (BSI). The BACI was validated in two independent international cohorts from the UK and Serbia.
FINDINGS: Between June 1, 2006, and Nov 22, 2013, 1340 patients with bronchiectasis were screened and 986 patients were analysed. Patients had a median of four comorbidities (IQR 2-6; range 0-20). 13 comorbidities independently predicting mortality rate were integrated into the BACI. The overall hazard ratio for death conferred by a one-point increase in the BACI was 1·18 (95% CI 1·14-1·23; p<0·0001). The BACI predicted 5 year mortality rate, hospital admissions, exacerbations, and health-related quality of life across all BSI risk strata (p<0·0001 for mortality and hospital admissions, p=0·03 for exacerbations, p=0·0008 for quality of life). When used in conjunction with the BSI, the combined model was superior to either model alone (p=0·01 for combined vs BACI; p=0·008 for combined vs BSI).
INTERPRETATION: Multimorbidity is frequent in bronchiectasis and can negatively affect survival. The BACI complements the BSI in the assessment and prediction of mortality and disease outcomes in patients with bronchiectasis.
FUNDING: European Bronchiectasis Network (EMBARC).

PMID: 27864036 [PubMed - indexed for MEDLINE]

Painful and swollen hands 3 months after lungs graft: Suracute voriconazole-induced periostitis and exostosis.

Joint Bone Spine. 2017 Jan;84(1):97-98

Authors: Metayer B, Bode-Milin C, Ansquer C, Haloun A, Maugars Y, Berthelot JM

PMID: 27117297 [PubMed - indexed for MEDLINE]

Imaging viscosity of intragranular mucin matrix in cystic fibrosis cells.

Sci Rep. 2017 Dec 01;7(1):16761

Authors: Requena S, Ponomarchuk O, Castillo M, Rebik J, Brochiero E, Borejdo J, Gryczynski I, Dzyuba SV, Gryczynski Z, Grygorczyk R, Fudala R

Abstract
Abnormalities of mucus viscosity play a critical role in the pathogenesis of several respiratory diseases, including cystic fibrosis. Currently, there are no approaches to assess the rheological properties of mucin granule matrices in live cells. This is the first example of the use of a molecular rotor, a BODIPY dye, to quantitatively visualize the viscosity of intragranular mucin matrices in a large population of individual granules in differentiated primary bronchial epithelial cells using fluorescence lifetime imaging microscopy.

PMID: 29196739 [PubMed - in process]

Cystic fibrosis: a call for papers for ECFS 2018.

Lancet Respir Med. 2017 Nov 28;:

Authors: Grainger E

PMID: 29196045 [PubMed - as supplied by publisher]

Evaluation of respiratory dynamics by volumetric capnography during submaximal exercise protocol of six minutes on treadmill in cystic fibrosis patients.

J Pediatr (Rio J). 2017 Nov 28;:

Authors: Parazzi PLF, Marson FAL, Ribeiro MAGO, Schivinski CIS, Ribeiro JD

Abstract
OBJECTIVES: Volumetric capnography provides the standard CO2 elimination by the volume expired per respiratory cycle and is a measure to assess pulmonary involvement. Thus, the objective of this study was to evaluate the respiratory dynamics of healthy control subjects and those with cystic fibrosis in a submaximal exercise protocol for six minutes on the treadmill, using volumetric capnography parameters (slope 3 [Slp3], Slp3/tidal volume [Slp3/TV], and slope 2 [Slp2]).
METHODS: This was a cross-sectional study with 128 subjects (cystic fibrosis, 64 subjects; controls, 64 subjects]. Participants underwent volumetric capnography before, during, and after six minutes on the treadmill. Statistical analysis was performed using the Friedman, Mann-Whitney, and Kruskal-Wallis tests, considering age and sex. An alpha=0.05 was considered.
RESULTS: Six minutes on the treadmill evaluation: in cystic fibrosis, volumetric capnography parameters were different before, during, and after six minutes on the treadmill; the same was observed for the controls, except for Slp2. Regarding age, an Slp3 difference was observed in cystic fibrosis patients regardless of age, at all moments, and in controls for age≥12 years; a difference in Slp3/TV was observed in cystic fibrosis and controls, regardless of age; and an Slp2 difference in the cystic fibrosis, regardless of age. Regarding sex, Slp3 and Slp3/TV differences were observed in cystic fibrosis regardless of sex, and in controls in male participants; an Slp2 difference was in the cystic fibrosis and female participants. The analysis between groups (cystic fibrosis controls) indicated that.
CONCLUSIONS: Cystic fibrosis showed greater values of the parameters before, during, and after exercise, even when stratified by age and sex, which may indicate ventilation inhomogeneity in the peripheral pathways in the cystic fibrosis.

PMID: 29195083 [PubMed - as supplied by publisher]

Parent knowledge of disease management in cystic fibrosis: Assessing behavioral treatment management.

Pediatr Pulmonol. 2017 Nov 29;:

Authors: Nicolais CJ, Bernstein R, Riekert KA, Quittner AL

Abstract
BACKGROUND: Cystic fibrosis (CF) is a life-shortening, burdensome disease requiring complex knowledge to manage the disease. Significant gaps in knowledge have been documented for parents, which may lead to unintentionally poor adherence and insufficient transfer of treatment responsibility from parents to adolescents. There are no current, validated measures of parent knowledge for this population and there are no measures that assess the knowledge required for day-to-day behavioral management of CF. We assessed the psychometric properties of the parent version of the Knowledge of Disease Management-Cystic Fibrosis measure (KDM-CF-P) using data from iCARE (I Change Adherence and Raise Expectations), a randomized control adherence intervention trial.
METHODS: A total of 196 parents in the iCARE standard care/control arm completed 35 items assessing their knowledge of disease management at their 12-month study visit, prior to beginning the intervention. Items were eliminated from the measure if they met the threshold for ceiling effects, were deemed clinically irrelevant, or did not correlate well with their intended scale. Item-to-total correlations, confirmatory factor analysis, discriminant function, reliability, and convergent validity were calculated.
RESULTS: The KDM-CF-P (19 items) demonstrated internal consistency of KR20 = 0.60 on each scale and a two-scale structure. Convergent validity for knowledge scores was found with maternal education, family income, and type of medical insurance. Parents correctly answered approximately 85% of items on the KDM-CF-P.
CONCLUSIONS: The KDM-CF-P psychometrics support a two-scale measure with clinical utility. It is useful for assessing gaps in knowledge that can be remediated through individualized, tailored interventions.

PMID: 29193881 [PubMed - as supplied by publisher]

Continuous vancomycin in a pediatric cystic fibrosis patient.

Pediatr Pulmonol. 2017 Nov 29;:

Authors: McKinzie CJ, Esther CR, Vece TJ

Abstract
Continuous vancomycin has been previously reported to maximize antimicrobial activity while avoiding toxicities associated with dose escalation, but the efficacy of this dosing strategy has not been reported. This case report describes the successful use of continuous vancomycin, including improvement in lung function and avoidance of nephrotoxicity, demonstrated in a pediatric cystic fibrosis (CF) patient with MRSA.

PMID: 29193836 [PubMed - as supplied by publisher]

Correlations between cystic fibrosis genotype and sinus disease severity in chronic rhinosinusitis.

Laryngoscope. 2017 Nov 29;:

Authors: Abuzeid WM, Song C, Fastenberg JH, Fang CH, Ayoub N, Jerschow E, Mohabir PK, Hwang PH

Abstract
OBJECTIVE: Cystic fibrosis (CF) patients commonly develop chronic rhinosinusitis (CRS). The impact of the most common cystic fibrosis transmembrane conductance regulator (CFTR) mutation, F508del, on the severity of sinonasal disease remains inconclusive. The objective of this study is to evaluate the impact of CFTR genotype functional classification on sinonasal disease severity in patients with CRS.
METHODS: Retrospective chart review of patients with CF who underwent endoscopic sinus surgery for chronic rhinosinusitis from 1998 to 2015. Patients were divided into high- or low-risk genotypes based on standardized CFTR gene functional classification. The primary outcome was the 22-item Sino-Nasal Outcome Test (SNOT-22) score. Secondary outcomes included endoscopic scores, extent of surgery performed, presence of polyposis, number of revision surgeries, and Lund-MacKay computed tomography scores.
RESULTS: Thirty-eight patients harbored a high-risk CFTR genotype, and 11 had a low-risk genotype. On bivariate analysis, there was no association between CFTR genotype risk stratification and measures of preoperative disease severity or postoperative outcomes. There were no associations between genotype risk stratification and outcome variables on multivariate linear regression, adjusted for age and gender. There were significant improvements in several SNOT-22 subdomains before and after endoscopic sinus surgery (P < 0.05), but the magnitude of improvement was not significantly different on the basis of CFTR genotype risk stratification.
CONCLUSION: High-risk CFTR genotypes are not associated with worse sinonasal disease severity or postoperative symptom control than low-risk CFTR genotypes after adjusting for confounding factors.
LEVEL OF EVIDENCE: Level IV. Laryngoscope, 2017.

PMID: 29193105 [PubMed - as supplied by publisher]

Let's talk about sex: Behaviors, experience and health care utilization in young women with CF.

J Cyst Fibros. 2017 Nov 27;:

Authors: Heltshe SL, Taylor-Cousar JL

PMID: 29191659 [PubMed - as supplied by publisher]

Reduced bone length, growth plate thickness, bone content, and IGF-I as a model for poor growth in the CFTR-deficient rat.

PLoS One. 2017;12(11):e0188497

Authors: Stalvey MS, Havasi V, Tuggle KL, Wang D, Birket S, Rowe SM, Sorscher EJ

Abstract
BACKGROUND: Reduced growth and osteopenia are common in individuals with cystic fibrosis (CF). Additionally, improved weight and height are associated with better lung function and overall health in the disease. Mechanisms for this reduction in growth are not understood. We utilized a new CFTR knockout rat to evaluate growth in young CF animals, via femur length, microarchitecture of bone and growth plate, as well as serum IGF-I concentrations.
METHODS: Femur length was measured in wild-type (WT) and SD-CFTRtm1sage (Cftr-/-) rats, as a surrogate marker for growth. Quantitative bone parameters in Cftr-/- and WT rats were measured by micro computed tomography (micro-CT). Bone histomorphometry and cartilaginous growth plates were analyzed. Serum IGF-I concentrations were also compared.
RESULTS: Femur length was reduced in both Cftr-/- male and female rats compared to WT. Multiple parameters of bone microarchitecture (of both trabecular and cortical bone) were adversely affected in Cftr-/- rats. There was a reduction in overall growth plate thichkness in both male and female Cftr-/- rats, as well as hypertrophic zone thickness and mean hypertrophic cell volume in male rats, indicating abnormal growth characteristics at the plate. Serum IGF-I concentrations were severely reduced in Cftr-/- rats compared to WT littermates.
CONCLUSIONS: Despite absence of overt lung or pancreatic disease, reduced growth and bone content were readily detected in young Cftr-/- rats. Reduced size of the growth plate and decreased IGF-I concentrations suggest the mechanistic basis for this phenotype. These findings appear to be intrinsic to the CFTR deficient state and independent of significant clinical confounders, providing substantive evidence for the importance of CFTR on maintinaing normal bone growth.

PMID: 29190650 [PubMed - in process]

Neurological and Sleep Disturbances in Bronchiectasis.

J Clin Med. 2017 Nov 30;6(12):

Authors: Phua CS, Wijeratne T, Wong C, Jayaram L

Abstract
Bronchiectasis unrelated to cystic fibrosis is a chronic lung disease that is increasingly recognised worldwide. While other common chronic lung conditions such as chronic obstructive lung disease have been associated with cardiovascular disease, there is a paucity of data on the relationship between bronchiectasis and cardiovascular risks such as stroke and sleep disturbance. Furthermore, it is unclear whether other neuropsychological aspects are affected, such as cognition, cerebral infection, anxiety and depression. In this review, we aim to highlight neurological and sleep issues in relation to bronchiectasis and their importance to patient care.

PMID: 29189747 [PubMed]

Prevalence and characteristics of chronic kidney disease among Danish adults with cystic fibrosis.

J Cyst Fibros. 2017 Nov 27;:

Authors: Berg KH, Ryom L, Faurholt-Jepsen D, Pressler T, Katzenstein TL

Abstract
BACKGROUND: With improved prognosis of CF, comorbidities including chronic kidney disease (CKD) are becoming increasingly important. Identification of those at highest CKD risk is hence a priority.
METHODS: In this cross-sectional study, adults with CF attending the Copenhagen CF Centre at Rigshospitalet with ≥2 measurements of serum creatinine from 2013 to 2015 were included. Data was obtained from an electronic CF database, which contains anonymised clinical and laboratory data on all individuals attending the clinic. CKD was defined as a confirmed (≥3months apart) estimated glomerular filtration rate≤60mL/min/1.73m2.
RESULTS: Of 181 individuals, the CKD prevalence was 2.7% and increased to 11% after inclusion of lung transplanted patients. Individuals with CKD were generally older (median 39 (IQR, 36-45) vs. 31 (IQR, 24-39) years; p<0.001), diabetic (86% vs. 41%, p<0.001), with longer median duration of chronic pulmonary infection (28.3 (20.0-35.8) vs. 20.0 (9.9-34.7) years; p=0.008) and with longer intravenous aminoglycosides use (606 (IQR, 455-917) vs. 273 (IQR, 91-826) days, p=0.005).
CONCLUSIONS: The CKD prevalence is high and related to age, diabetes, chronic infection, transplantation and aminoglycosides use. These observations call for longitudinal studies investigating CKD predictors in adults with CF.

PMID: 29187303 [PubMed - as supplied by publisher]

Antibiotic duration and changes in FEV1 are not associated with time until next exacerbation in adult cystic fibrosis: a single center study.

BMC Pulm Med. 2017 Nov 29;17(1):160

Authors: Espel JC, Palac HL, Cullina JF, Clarke AP, McColley SA, Prickett MH, Jain M

Abstract
BACKGROUND: Pulmonary exacerbations (PEx) are a major driver of morbidity and mortality in cystic fibrosis and reducing their frequency by extending the time between them is an important therapeutic goal. Although treatment decisions for exacerbations are often made based on dynamic changes in lung function, it is not clear if these changes truly impact future exacerbation risk. We analyzed adults with chronic Pseudomonas aeruginosa infection to determine whether changes in FEV1 or duration of intravenous antibiotic therapy were associated with time to the next pulmonary exacerbation.
METHODS: Medical records and Cystic Fibrosis Foundation Patient Registry data were examined retrospectively to assess whether various patient-specific demographic factors and exacerbation-specific characteristics were associated with time until next exacerbation using the Andersen-Gill model in order to control for previous exacerbation frequency history.
RESULTS: We examined 59 patients with 221 CF pulmonary exacerbations over a 3-year study period. Mean age was 28.2 years and mean baseline FEV1 was 62% predicted. In our univariable model, fall in FEV1 at onset of exacerbation (median absolute -3% predicted change), recovery of FEV1 with treatment (median absolute +3% predicted change) and duration of IV antibiotics (median 16 days) were not associated with time to next exacerbation (median 93.5 days). Paradoxically each one-year increase in age was associated with a reduction in hazard of PEx by 3% (HR 0.97, P = 0.03, 95% CI 0.95-1.00).
CONCLUSIONS: FEV1 drop and recovery associated with onset and treatment of a CF pulmonary exacerbation or duration of intravenous antibiotics were not predictive of time until next exacerbation. Our finding that older age may be associated with decreased hazard of exacerbation is likely due to a healthy survivor effect and should be controlled for in clinical trials of pulmonary exacerbations.

PMID: 29187171 [PubMed - in process]

Non-Coding RNAs in Pediatric Airway Diseases.

Genes (Basel). 2017 Nov 27;8(12):

Authors: Narożna B, Langwiński W, Szczepankiewicz A

Abstract
Non-coding RNAs (ncRNAs) are involved in the regulation of numerous biological processes and pathways and therefore have been extensively studied in human diseases. Previous reports have shown that non-coding RNAs play a crucial role in the pathogenesis and aberrant regulation of respiratory diseases. The altered expression of microRNAs (miRNAs) and long noncoding RNAs in blood and also locally in sputum or exhaled breath condensate influences lung function, immune response, and disease phenotype and may be used for the development of biomarkers specific for airway disease. In this review, we provide an overview of the recent works studying the non-coding RNAs in airway diseases, with a particular focus on chronic respiratory diseases of childhood. We have chosen the most common chronic respiratory condition-asthma- and the most severe, chronic disease of the airways-cystic fibrosis. Study of the altered expression of non-coding RNAs in these diseases may be key to better understanding their pathogenesis and improving diagnosis, while also holding promise for the development of therapeutic strategies using the regulatory potential of non-coding RNAs.

PMID: 29186897 [PubMed]

Molecular Epidemiology of Methicillin-Resistant Staphylococcus aureus in Cystic Fibrosis Patients from Argentina.

Microb Drug Resist. 2017 Nov 29;:

Authors: Pena Amaya P, Haim MS, Fernández S, Di Gregorio S, Teper A, Vázquez M, Lubovich S, Galanternik L, Mollerach M

Abstract
Methicillin-resistant Staphylococcus aureus (MRSA) colonization in cystic fibrosis (CF) patients is an increasing problem in many countries. In our Respiratory Center at the Hospital de Niños "Dr. Ricardo Gutiérrez", Buenos Aires, Argentina, the prevalence has climbed from 23% in 1995 up to 32% in 2011. Our objective was to analyze the diversity of MRSA isolates recovered from respiratory samples of CF patients attending our center, characterizing their phenotypes and clonal distribution. Therefore, a prospective study was conducted on all CF patients attending the pediatric Respiratory Center between June 2012 and May 2013 to collect MRSA isolates. Antibiotic susceptibility testing, multilocus sequence typing, pulsed-field gel electrophoresis, spa typing, and agr genotyping were performed on collected isolates. The prevalence of MRSA during this period was 34.2%, and 71.9% of the patients were infected with isolates that carried SCCmec IV. High resistance rates were detected for gentamicin, erythromycin, clindamycin, ciprofloxacin, and rifampicin. Strains related to the community-associated MRSA clones, ST5-IV and ST30-IV, were the most frequently recovered. Remarkably, even though most of the isolates were related to these clones, the rate of multi-resistance shown in CF patients was higher than that reported for the same lineages recovered from other infections in our country.

PMID: 29185854 [PubMed - as supplied by publisher]

isGPT: An optimized model to identify sub-Golgi protein types using SVM and Random Forest based feature selection.

Artif Intell Med. 2017 Nov 25;:

Authors: Rahman MS, Rahman MK, Kaykobad M, Rahman MS

Abstract
The Golgi Apparatus (GA) is a key organelle for protein synthesis within the eukaryotic cell. The main task of GA is to modify and sort proteins for transport throughout the cell. Proteins permeate through the GA on the ER (Endoplasmic Reticulum) facing side (cis side) and depart on the other side (trans side). Based on this phenomenon, we get two types of GA proteins, namely, cis-Golgi protein and trans-Golgi protein. Any dysfunction of GA proteins can result in congenital glycosylation disorders and some other forms of difficulties that may lead to neurodegenerative and inherited diseases like diabetes, cancer and cystic fibrosis. So, the exact classification of GA proteins may contribute to drug development which will further help in medication. In this paper, we focus on building a new computational model that not only introduces easy ways to extract features from protein sequences but also optimizes classification of trans-Golgi and cis-Golgi proteins. After feature extraction, we have employed Random Forest (RF) model to rank the features based on the importance score obtained from it. After selecting the top ranked features, we have applied Support Vector Machine (SVM) to classify the sub-Golgi proteins. We have trained regression model as well as classification model and found the former to be superior. The model shows improved performance over all previous methods. As the benchmark dataset is significantly imbalanced, we have applied Synthetic Minority Over-sampling Technique (SMOTE) to the dataset to make it balanced and have conducted experiments on both versions. Our method, namely, identification of sub-Golgi Protein Types (isGPT), achieves accuracy values of 95.4%, 95.9% and 95.3% for 10-fold cross-validation test, jackknife test and independent test respectively. According to different performance metrics, isGPT performs better than state-of-the-art techniques. The source code of isGPT, along with relevant dataset and detailed experimental results, can be found at https://github.com/srautonu/isGPT.

PMID: 29183738 [PubMed - as supplied by publisher]

Daily spirometry in an acute exacerbation of adult cystic fibrosis patients.

Chron Respir Dis. 2017 Jan 01;:1479972317743756

Authors: Stephen MJ, Long A, Bonsall C, Hoag JB, Shah S, Bisberg D, Holsclaw D, Varlotta L, Fiel S, Du D, Zanni R, Hadjiliadis D

Abstract
To help answer the question of length of intravenous antibiotics during an acute exacerbation of cystic fibrosis (CF), we had subjects to follow daily home spirometry while on intravenous antibiotics. CF patients, 18 and older, with an acute exacerbation requiring intravenous antibiotics had a daily FEV1. The average time to a 10% increase over their initial sick FEV1 was calculated, as well as the time to a new baseline. A total of 25 subjects completed the study. Ten of the 25 subjects did not have a sustainable 10% increase in FEV1. Of the 15 subjects with a sustainable 10% increase in FEV1, it took 5.2 days (±4.5) after day 1, while a new baseline was achieved on average at 6.6 days (±4.8) after day 1. Given the wide range of time to a 10% improvement and new baseline, it is recommended there should be flexibility in length of intravenous antibiotics in CF, not by a preset number.

PMID: 29183160 [PubMed - as supplied by publisher]