Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort study.

BACKGROUND: The decline in pulmonary function is a predictor of disease progression in patients with cystic fibrosis (CF). This study aimed to determine the decline rate of percent predicted forced expiratory volume in 1 s (ppFEV1) based on the data of the CF Registry of Turkey. The secondary aim was to investigate the risk factors related to the decline in ppFEV1. METHODS: A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017-2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors. RESULTS: A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p < .001) and mucoid P. aeruginosa colonization (p < .001) were significantly higher in this group (p < .001). Overall mean annual ppFEV1 decline was -0.97% (95% confidence interval [CI] = -0.02 to -1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 ≥ 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40-69) two groups (p = .004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26-2.54; p = .01) and initial ppFEV1 ≥ 70 (OR = 2.98 95% CI = 1.06-8.36), p = .038) were associated with significant ppFEV1 decline in the whole cohort. CONCLUSIONS: This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression.

A Case Report of Lung Transplantation After Hematopoietic Stem Cell Transplantation and Literature Review.

Pulmonary complications may occur after hematopoietic stem cell transplantation for hematologic malignancies. Lung transplantation is the only treatment option for end-stage lung failure. We presented a case of acute myeloid leukemia who received a hematopoietic stem cell transplantation and underwent bilateral lung transplantation with end-stage usual interstitial pneumonia and chronic obstructive lung disease. This case showed that lung transplantation could be successfully applied in properly selected hematologic malignancy patients with long disease-free survival, like lung transplantations performed for other indications.

European Network for ICU-Related Respiratory Infections (ENIRRIs): a multinational, prospective, cohort study of nosocomial LRTI.

PURPOSE: Lower respiratory tract infections (LRTI) are the most frequent infectious complication in patients admitted to the intensive care unit (ICU). We aim to report the clinical characteristics of ICU-admitted patients due to nosocomial LRTI and to describe their microbiology and clinical outcomes. METHODS: A prospective observational study was conducted in 13 countries over two continents from 9th May 2016 until 16th August 2019. Characteristics and outcomes of ventilator-associated pneumonia (VAP), ventilator-associated tracheobronchitis (VAT), ICU hospital-acquired pneumonia (ICU-HAP), HAP that required invasive ventilation (VHAP), and HAP in patients transferred to the ICU without invasive mechanical ventilation were collected. The clinical diagnosis and treatments were per clinical practice and not per protocol. Descriptive statistics were used to compare the study groups. RESULTS: 1060 patients with LRTI (72.5% male sex, median age 64 [50-74] years) were included in the study; 160 (15.1%) developed VAT, 556 (52.5%) VAP, 98 (9.2%) ICU-HAP, 152 (14.3%) HAP, and 94 (8.9%) VHAP. Patients with VHAP had higher serum procalcitonin (PCT) and Sequential Organ Failure Assessment (SOFA) scores. Patients with VAP or VHAP developed acute kidney injury, acute respiratory distress syndrome, multiple organ failure, or septic shock more often. One thousand eight patients had microbiological samples, and 711 (70.5%) had etiological microbiology identified. The most common microorganisms were Pseudomonas aeruginosa (18.4%) and Klebsiella spp (14.4%). In 382 patients (36%), the causative pathogen shows some antimicrobial resistance pattern. ICU, hospital and 28-day mortality were 30.8%, 37.5% and 27.5%, respectively. Patients with VHAP had the highest ICU, in-hospital and 28-day mortality rates. CONCLUSION: VHAP patients presented the highest mortality among those admitted to the ICU. Multidrug-resistant pathogens frequently cause nosocomial LRTI in this multinational cohort study.

The success of the Cystic Fibrosis Registry of Turkey for improvement of patient care.

BACKGROUND: Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease. METHODS: Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data. RESULTS: There were 1170 people with CF in 2017 and 1637 in 2019 CF registry. Eight hundred and fourteen people were registered in both 2017 and 2019 of whom z-scores of heights and BMI were significantly higher in 2019 (p = 0.002, p =0.039, respectively). Inhaled hypertonic saline, bronchodilator, and azithromycin usages were significantly higher in 2019 (p =0.001, p = 0.001, p = 0.003, respectively). The percent predicted of forced expiratory volume in 1 sec and forced vital capacity were similar in 2017 and 2019 (88% and 89.5%, p = 0.248 and 84.5% and 87%, p =0.332, respectively). Liver diseases and osteoporosis were significantly higher, and pseudo-Bartter syndrome (PBS) was significantly lower in 2019 (p = 0.011, p = 0.001, p = 0.001, respectively). CONCLUSIONS: The z-scores of height and BMI were higher, the use of medications that protect and improve lung functions was higher and incidence of PBS was lower in 2019. It was predicted that registry system increased the care of people with CF regarding their follow-up. The widespread use of national CF registry system across the country may be beneficial for the follow-up of people with CF.

Communication: The Follow-Up of Biomarkers Better Predicts the Poor Outcome in COVID-19 Patients.

OBJECTIVE: To investigate the course of biomarkers on admission and follow-up in order to identify early predictors for poor outcome in COVID-19 patients. METHODS: In this study, 132 COVID-19 patients were classified as good outcome (n=62) and poor outcome (n=70) groups. Laboratory parameters were evaluated on admission and within 5-7 days after hospitalization. RESULTS: Baseline levels of neutrophil-lymphocyte ratio, CRP, procalcitonin, ferritin, D-dimer and LDH were higher (p<0.01); lymphocyte count was lower in the poor outcome patients. During follow-up there was a larger decrease in lymphocyte count and more prominent increases in other biomarkers (p<0.001). In ROC analysis, the AUCs strongly indicated the poor outcome on days 5-7 of the hospitalization. CONCLUSIONS: This study suggests that the follow-up measurements of the biomarkers better predict the poor outcome in COVID-19 pneumonia.

[Colistin use in ventilator-associated pneumonia due to panresistant Pseudomonas aeruginosa and Acinetobacter baumannii]. / Panrezistan Pseudomonas aeruginosa ve Acinetobacter baumannii ile gelisen ventilatörle iliskili pnömonilerde kolistin kullanimi.

Nosocomial infections by resistant gram-negative microorganisms are important causes of mortality in intensive care unit (ICU)'s. The treatment choices are limited in infections due to Acinetobacter baumannii and Pseudomonas aeruginosa, especially if they are panresistant. In these type of resistant infections, colistin--an old antibiotic--has become a current issue. The aim of this study was to evaluate the efficacy of colistin in 9 cases (6 males, mean age 75.8 +/- 9.4 years), with ventilator associated pneumonia (VAP) caused by panresistant A. baumannii and P. aeruginosa in respiratory ICU. All cases were referred to ICU from other hospitals or clinics. It was detected that 7 of 9 cases were treated with anti-pseudomonal antibiotics before the development of VAP. Panresistant A. baumannii was isolated in 5 cases and P. aeruginosa in 4 cases. VAP by these microorganisms was detected on the 26.6 +/- 12.4th days of invasive mechanical ventilation and the cases were followed up for 54.2 +/- 25.7 days in ICU. During colistin treatment, dermatitis (one case) and nephrotoxicity (one case) were observed as side effects. Microbiological response to colistin was obtained in 6 cases. Three cases died due to non-eradication of panresistant microorganisms and three cases died due to other infections during ICU follow-up. The data presented in this study demonstrates that colistin can be considered as a safe and effective antibiotic in the treatment of panresistant A. baumannii and P. aeruginosa infections.

Evaluation of Traffic Accident Risk in In-City Bus Drivers: The Use of Berlin Questionnaire.

OBJECTIVES: Traffic accidents associated with high mortality rate may produce serious problems especially in highways. Obstructive sleep apnea (OSA) has been associated with a high risk for traffic accidents due to excessive daytime sleepiness even in in-city drivers. In the present study, it was aimed to evaluate the rate of OSA symptoms and to identify risk factors associated with traffic accidents in in-city bus drivers. MATERIAL AND METHODS: A self-administered questionnaire including demographic and anthropometric features, sleep and work schedules, Berlin questionnaire, Epworth sleepiness score (ESS), and history of traffic accidents was used. RESULTS: The questionnaire was conducted for 1400 male bus drivers (mean age, 38.0±6.4 y, body mass index, 27.8±3.9 kg/m2). A total of 1058 (75.6%) drivers had one or more accidents while driving bus. According to the Berlin questionnaire, 176 (12.6%) drivers were found to have high OSA risk and the accident rate was 83.0% in high-risk group, whereas 74.5% of low-risk drivers had accidents (p=0.043). The drivers with a history of traffic accident were older (p=0.030), had higher ESS (p=0.019), and were more in the high-risk OSA group according to the Berlin questionnaire (p=0.015). In multivariate linear regression analysis, traffic accident was associated with only Berlin questionnaire (p=0.015). CONCLUSION: The present results support that city bus drivers with high OSA risk according to Berlin questionnaire have increased accident rates. Therefore, we suggest using Berlin questionnaire for screening sleep apnea not only in highway drivers but also in in-city bus drivers.

Expression of p63, TTF-1 and Maspin in Non-Small Cell Lung Carcinoma and Their Effect on the Prognosis and Differential Diagnosis.

OBJECTIVE: Lung cancer is still the leading cause of cancer mortality. Antiapoptotic genes and protease inhibitors play an important role in the development of lung cancer. MATERIAL AND METHOD: p63, TTF-1 and maspin expression and their role in the differential diagnosis, overall survival, progression-free survival and other clinicopathological characteristics of the patients were investigated in 80 surgically-resected non-small cell lung carcinomas. RESULTS: The maximal tumor diameter range was 1.5-11 cm (mean: 4.06±1.8 cm). Forty-five (56.3%) tumors were adenocarcinoma, 23 (28.8%) squamous cell carcinoma, four (5%) large cell carcinoma, six (7.5%) large cell neuroendocrine carcinoma, one (1.2%) sarcomatoid carcinoma while one was (1.2%) both adenocarcinoma and squamous cell carcinoma. The patients with advanced TNM stage and a tumor diameter more than 3 cm had markedly poor survival. Immunohistochemically, p63 staining was present in 87.5% of squamous cell carcinomas, 4.3% of adenocarcinomas, 25% of large cell carcinomas, and 16.7% of large cell neuroendocrine carcinomas. Similarly, maspin was positive in 66.7% of squamous cell carcinomas and 17.4% of adenocarcinomas. The TTF-1 staining rate was higher in adenocarcinomas (84.8%). There was no immunoreactivity in squamous cell carcinomas (p < 0.001). We found that p63 and TTF-1 had no significant effect on survival in either tumor group (p > 0.05) while maspin has a negative prognostic effect in adenocarcinoma (p=0.048). CONCLUSION: This study suggests that p63 and TTF-1 are reliable markers in non-small cell lung carcinoma and can be used in differential diagnosis. Maspin has been identified as a prognostic marker in adenocarcinoma. However, more studies are required to elucidate the significance of maspin.

An unusual presentation of anti-Jo-1 syndrome, mimicking lung metastases, with massive pleural and pericardial effusions.

We report a case of anti-Jo-1 syndrome (a rare autoimmune condition that may manifest with various forms of interstitial lung disease), which in our case presented unusually with multiple pulmonary nodules, mimicking carcinoma. She subsequently developed pleural and pericardial effusions (which are rare in this syndrome), myopathy, and "mechanic's hand," with similar lesions on the feet. "Mechanic's foot" noted in this patient has not been previously described. She initially responded well to immunosuppression but has subsequently progressed to pulmonary fibrosis.