Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease of unknown cause that occurs sporadically, but it can also occur in families and so named as Familial Pulmonary Fibrosis (FPF). Some forms of FPF overlaps IPF features, namely the radiological and histological pattern of usual interstitial pneumonia (UIP). Genetic and environmental factors commonly play an important role in the pathogenesis of FPF and the most commonly identified mutations involve the telomerase complex. Here, we report a rare case of FPF in a male at the age of 44, in whom genetic testing showed heterozygous variants for the telomerase reverse transcriptase gene (TERT). Our report highlights the importance of compiling a thorough family history in younger patients identified with UIP serving as a resource for identifying the current and future genetic links to disease. Families with UIP hold a great promise in defining UIP pathogenesis, potentially suggesting targets for the development of future therapies.
Introduction: The classification of the severity of obstructive sleep apnea (OSA) based on the Apnea/Hypopnea Index (AHI) does not reflect the heterogeneity and prognosis of the disease. The Baveno classification proposes a new assessment system that includes symptoms and comor-bidities. The aim of our study was to evaluate the application of the Baveno classification in clinical practice and to explore its association with sleep indices, adherence to therapy and symptoms over a 6-months period. Material and methods: Prospective study including patients diagnosed with OSA between January and July 2021 was conducted. Patients were divided into 4 groups (A–D) according to the Baveno classification. The adherence to PAP treatment and Epworth Sleepiness Scale (ESS) values were obtained 6 months after initiation of therapy. Results: A total of 91 patients (84% male, 58 ± 13 years) were included in the study. The median ESS score was 10 (6–15), mean AHI was 28.4 ± 22.2 events/hour and the time with SpO2 < 90% (T90) was 9.7 ± 14.9%. At diagnosis, patients were classified into Baveno groups: A: 30%; B: 35%; C: 17%, D: 19%. There were no statisti-cal differences in AHI between the different groups. On the other hand, T90 had higher values in patients with comorbidities (C, D). Regarding the treatment, the prescription of PAP was higher in patients with comorbidities (C, D), and adherence to this treatment at 6 months was higher in group D. Among patients under PAP therapy, there was a statistically significant decrease in daytime sleepiness at 6 months in groups B and D. Conclusions: The Baveno classification distributes pa-tients with OSA evenly across the different phenotypes, regardless of the AHI value. The treatment decision was linked to the comorbidities (C, D) were the ones who had the greatest adherence to treatment at 6 months were in group D. ESS improved with greater emphasis in the most sympto-matic (B, D), while the AHI is essential for the diagnosis of OSA, the Baveno classification may guide physicians better in their treatment decision.
OBJECTIVE:The NELSON study demonstrated a positive association between computed tomography scanning and reduced mortality associated with lung cancer. The COPD-LUCSS-DLCO is a tool designed to improve screening selection criteria of lung cancer for chronic obstructive pulmonary disease patients. The aim of this study was to examine and compare the discriminating value of both scores in a community-based cohort of chronic obstructive pulmonary disease patients. METHODS: A retrospective study of chronic obstructive pulmonary disease patients followed in pulmonology consultation for a period of 10 years (2009-2019) was conducted. The NELSON criteria and COPD-LUCSS-DLCO score were calculated for each patient at the time of the study inclusion. The lung cancer incidence was calculated for each of the subgroups during the follow-up period. RESULTS: A total of 103 patients were included in the study (mean age 64.7±9.2 years, 88.3% male). Applying the COPD-LUCSS-DLCO score, high-risk patients have a 5.9-fold greater risk of developing lung cancer versus the low risk. In contrast, there was no significant association between NELSON selection criteria and lung cancer incidence. The area under the curve was 0.69 for COPD-LUCSS-DLCO and 0.59 for NELSON criteria. Comparing test results showed no differences. CONCLUSIONS: The use of the COPD-LUCSS-DLCO score in clinical practice can help to detect chronic obstructive pulmonary disease patients in greater risk of developing lung cancer with better performance than NELSON criteria. Therefore, models that include a risk biomarker strategy can improve selection criteria and consequently can enhance a better lung cancer prediction.
Introduction: Several biological agents for the treatment of severe asthma have been approved for self-administration on an outpatient basis in the last years. However, data on the impact of home administration in outcomes such as asthma control and quality of life in real-life settings are sparse. Being this knowledge crucial for clinical practice, this study aimed to assess asthma control and quality of life in patients who transitioned from day hospital administration of biological therapy to home administration. Methods: A single-center prospective analysis of 33 patients treated with biologics for severe asthma, who switched from hospital to home treatment was performed. Asthma Control Test (ACT), Control of Allergic Rhinitis and Asthma Test (CARAT), Asthma Life Quality (ALQ) and the number of exacerbations were assessed 3 months before and 3 and 6 months after of homeuse.Results: ACT and CARAT did not show statistical differences comparing to the baseline values (21.8 ± 2.7 and 23.8 ± 5.5) within 3 months (22.1 ± 2.4, p=0.609; 23.2 ± 5.3, p=0.572) or 6 months (23.4 ± 0.9, p=0.553; 23.7 ± 6.2, p=0.149) of home administration. Also, ALQ score did not show meaningful variations between baseline (9.5 ± 3.2) and after 3 months (11.2 ± 4.4, p=0.275) and 6 months (10.3 ± 3.8, p=0.209) of home-use. Regarding asthma exacerbations, we did not record a significant difference comparing to the baseline values of 3 months/patient exacerbations (0.2 ± 0.4) and after 3 months (0.2 ± 0.5, p=0.786) or 6 months (0.2 ± 0.4, p=1.000) of change in modality treatment. There was no cases of anaphylaxis or other serious adverse effects in those patients treated at home. Conclusions: Transition of day hospital administration of biologic treatment for severe asthma to home administration did not lead to any deterioration of asthma control or quality of life. Our results emphasized the efficacy and safety of home administration of biologic treatment and provide support on changing the paradigm of the administration of biological treatment in severe asthma.
Methods: Analysis of a data base analyzing the impact of a pain management protocol and opioid consumption after robotic lobectomy as part of ERAS pathway. Our protocol begins with admnistration of acetaminophen (1 g po), celecoxib (400 mg po) and gabapentin (600 mg po) 2 hours before anesthesia. Intraoperatively, we utilize cryoanalgesia (T4-T9) and intercostal blockade with bupivacaine (01.25%). At the end of the operation, we give ketorolac (15-30 mg IV) and acetaminophen (1 g IV). Postoperatively patients receive a combination of acetaminophen, ibuprofen, gabapentin and tramadol. Other opioids are prescribed for brakethrough pain as prn medications. Our database and the electronic health record were analyzed looking at the consumption of IV or oral opioids until the time of discharge. The Morphine Milligram Equivalent (MME) was calculated using a standard opioid conversion calculator. Pain scores and length of stay were additionally analyzed. Results: 27 patients (M = 14, F = 13) underwent robotic lobectomy for lung cancer. Mean age was 63 (55-81). Mean length of stay was 1.7 days (1-4). Pain score mean was 1.6 (0-5). Twelve patients did not receive any doses of hydromorphone (IV) or oxycodone ( po). Five patients received oxycodone but not hydromorphone. Four patients received hydromorphone but not oxycodone. The remaining six patients received both hydromorphone and oxycodone. Cryoanalgesia was utilized in all patients. The calculated morphine milligram equivalents (MME) were 2 for hydromorphone and 11.75 for oxycodone. Conclusions: A multimodality protocol for pain management in thoracic surgery patients that includes a variety of medications and techniques (including cryoanalgesia and intercostal blockade), results in a significant reduction or elimination of opioid consumption, while providing excellent pain relief and a short length of stay.
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