Prevalence of levator ani muscle injuries in primiparous women after delivery and their influence on pelvic floor disorders-systematic review.

BACKGROUND: Studies show a significant association between the first vaginal delivery and injuries of the levator ani muscle (LAM), which can cause pelvic floor disorders (PFDs). OBJECTIVES: This study aims to identify the prevalence of short and long-term LAM injuries after vaginal delivery in primiparous women and its influence on PFDs. METHOD: A systematic review was conducted according to the PRISMA methodology. The databases used were Pubmed, Cochrane, and PEDro. The quality assessment of the evidence was carried out using the Critical Appraisal Skills Programme (CASP). Both the selection of studies and their evaluation were done by two researchers and a third reviewer in cases of disagreement. RESULTS: From the search, 57 articles were gathered, and 19 were included to match the eligibility criteria. The prevalence of avulsion of the LAM was found in association with vaginal delivery between 13% and 28% ≤ 1 year after delivery and between 16% and 29% > 1 year after delivery. Ballooning was detected between 20% and 37% ≤ 1 year, and 33% of women > 1 year after delivery, appearing to be more common when compared to avulsion. Pelvic organ prolapse (POP) was considered the most common disorder associated with injuries of the LAM, and there seems to be some connection with sexual dysfunction. CONCLUSION: Avulsion of the LAM and ballooning of the hiatal area have a high prevalence in primiparous women after vaginal delivery and have a strong direct relation to the development of POP.

The impact of nintedanib and pirfenidone on lung function and survival in patients with idiopathic pulmonary fibrosis in real-life setting.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing interstitial pneumonia of unknown cause that is associated with radiological and/or histological features of usual interstitial pneumonia (UIP). A mean survival of 2-5 years was reported previously to the advent of antifibrotics. According to clinical trials, nintedanib and pirfenidone induce a significant delay in functional decline, with a favorable impact on survival. METHODS: A real-life retrospective and longitudinal study was conducted to assess the efficacy and tolerability of antifibrotics in IPF patients, between January 2014 and December 2020. Two groups (under nintedanib or pirfenidone) were analyzed at diagnosis through their clinical features and radiological patterns. Lung function was assessed at diagnosis (time 0) and after 6, 12 and 24 months of treatment. We also compared this antifibrotic cohort with an older naïve antifibrotic cohort, mainly treated with immunosuppressive drugs and/or N- acetylcysteine. Survival was analyzed and prognostic features were also studied. Statistical analysis was performed with IBM® SPSS®. RESULTS: A cohort of 108 patients under antifibrotics (nintedanib n = 54; pirfenidone n = 54) was assessed. Lung function analysis showed an overall stabilization in FVC and DLCO mean predicted percentages at 6, 12 and 24 months of treatment. The mean decline in FVC and DLCO, at 12 months, was -40.95 ± 438.26 mL and -0.626 ± 1.31 mL/min/mmHg, respectively. However, during this period, 34.2% of the patients died mostly due to acute exacerbation associated with a poorer lung function at diagnosis. Mean survival in the naïve antifibrotic cohort was significantly lower than in the antifibrotic cohort (39.9 months versus 58.2 months; p < 0.005). Regarding lung function evolution and survival, we found no differences between definitive or probable UIP radiological patterns, both on patients under nintedanib and pirfenidone (p = 0.656). CONCLUSIONS: In this real-life observational study, the positive impact of antifibrotic therapy on the IPF clinical course and on survival was corroborated. Regarding efficacy, there was no difference between patients taking nintedanib or pirfenidone. The need for an early treatment was also demonstrated, since a worse outcome is clearly associated with lower lung volumes and lower diffusing capacity at diagnosis.

Pelvic floor muscle function after grade II tears-Surface electromyography test-retest and differences between nulliparous and primiparous.

BACKGROUND: Vaginal birth is a risk factor for weakening of the pelvic floor muscles (PFM) and development of pelvic floor dysfunction (PFD). Perineal tears may decrease PFM function. PFM tone can be assessed with surface EMG (sEMG), but reliability studies of sEMG in women with perineal tears are lacking. The aims of this study were to evaluate test-retest and intrarater reliability of sEMG and compare PFM activation between nulliparous and primiparous. METHODS: A sEMG test-retest was performed in 21 women (12 nulliparous and 9 primiparous with grade II tears) to assess intra-rater reliability during rest and maximal voluntary contraction (MVC) of the PFM. Intraclass Correlation Coefficient (ICC), standard error of measurement (SEM) and minimal detectable change (MDC) were tested. A comparison between nulliparous' and primiparous' PFM activation during rest and MVC was performed. RESULTS: sEMG demonstrated fair reliability in nulliparous (ICC: 0.239; SEM: 5.2; MDC: 14.5) and moderate reliability in primiparous (ICC: 0.409; SEM: 1.5; MDC: 4.2) during rest. For peak MVC very good intrarater reliability was found in nulliparous (ICC: 0.92; SEM: 8.0; MDC: 22.2) and in primiparous (ICC: 0.823; SEM: 8.0; MDC: 22.2). Statistically significant lower PFM activation was found in primiparous women with perineal tear grade II than in nulliparous at rest (mean difference 9.1 µV, 95% confidence interval [CI] 3.0-19.0, p = 0.001), and during MVCpeak (mean difference 50.0 µV, 95% CI 10.0-120.0 p = 0.021). CONCLUSIONS: sEMG is reliable when measuring PFM activation in primiparous women with perineal tears grade II. Women with perineal tears grade II have lower PFM activation both during rest and MVC.

A scoping review exploring stigma associated with postpartum urinary incontinence.

INTRODUCTION AND HYPOTHESIS: Postpartum urinary incontinence (UI) is prevalent, yet health-seeking behaviours for prevention and treatment are markedly low. Health-related stigma refers to conditions that may be socially devalued and considered deviating from "expected norms" and is a barrier to equitable health care. It may be plausible that stigma is associated with postpartum UI and leads to avoiding health-seeking behaviours, which this scoping review sought to examine and summarize. METHODS: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for scoping reviews was followed. The following keywords were used to develop a search strategy: Postpartum, Urinary Incontinence and Stigma. The search was carried out on PubMed, PsycInfo, Scopus, CINAHL, Web of Science and ProQuest Dissertation and Theses Global. All study designs (clinical trials, observational studies, qualitative studies) were eligible for inclusion. Data were extracted and mapped to identify causal factors of postpartum UI stigma and implications for outcomes and behaviours. RESULTS: Twelve studies were included. Most studies utilized questionnaires assessing constructs related to quality of life that also captured potential stigma, or interviews. Sources of postpartum UI stigma included community values surrounding UI and self-stigma, whereby participants directed stereotypes associated with urinary leakage towards themselves. Implications of postpartum UI stigma included negative mental emotions such as shame and embarrassment, which led to avoiding situations where they needed to disclose symptoms, including in health care environments. CONCLUSIONS: Future research requires a purposeful assessment of postpartum UI stigma to learn from lived experience how to mitigate stigma and improve quality of care.

The Role of Ultrasonography in the Diagnosis and Decision Algorithm for the Management of Pneumothorax after Transbronchial Lung Cryobiopsy.

BACKGROUND: Pneumothorax is one of the main complications of transbronchial lung cryobiopsy (TBLC). Chest ultrasound (CUS) is a radiation-free alternative method for pneumothorax detection. OBJECTIVE: We tested CUS diagnostic accuracy for pneumothorax and assessed its role in the decision algorithm for pneumothorax management. Secondary objectives were to evaluate the post-procedure pneumothorax occurrence and risk factors. METHODS: Eligible patients underwent TBLC, followed by chest X-ray (CXR) evaluation 2 h after the procedure, as our standard protocol. Bedside CUS was performed within 30 min and 2 h after TBLC. Pneumothorax by CUS was defined by the absence of lung sliding and comet-tail artefacts and confirmed with the stratosphere sign on M-mode. Pneumothorax size was determined through lung point projection on CUS and interpleural distance on CXR and properly managed according to clinical status. RESULTS: Sixty-seven patients were included. Nineteen pneumothoraces were detected at 2 h after the procedure, of which 8 (42.1%) were already present at the first CUS evaluation. All CXR-detected pneumothoraces had a positive CUS detection. There were 3 discordant cases (κ = 0.88, 95% CI: 0.76-1.00, p < 0.001), which were detected by CUS but not by inspiration CXR. We calculated a specificity of 97.5% (95% CI: 86.8-99.9) and a sensitivity of 100% (95% CI: 87.2-100) for CUS. Pneumothorax rate was higher when biopsies were taken in 2 lobes and if histology had pleural representation. Final diagnosis was achieved in 79.1% of patients, with the most frequent diagnosis being hypersensitivity pneumonitis. Regarding patients with large-volume pneumothorax needing drainage, the rate of detection was similar between CUS and CRX. CONCLUSION: CUS can replace CXR in detecting the presence of pneumothorax after TBLC, and the lung point site can reliably indicate its size. This useful method optimizes time spent at the bronchology unit and allows immediate response in symptomatic patients, helping to choose optimal treatment strategies, while preventing ionizing radiation exposure.

Enlightening clinical and therapeutic heterogeneity in thoracic actinomycosis: A review in bronchiectasis and non-bronchiectasis patients.

Actinomycosis is a rare chronic infection triggered by species of Actinomyces. Although thoracic involvement represents about 15% of human actinomycosis, its true incidence may be underestimated, not only because of its challenging diagnosis, but also because it can be treated unintentionally with antibiotics for other diseases. In this sense, this work aims at providing an up-to-date literature review on thoracic actinomycoses, with particular emphasis on presentation, diagnostic and therapeutic approaches, also paving upcoming clinical interventions from findings obtained of a presentation of a case series. Data discussed here clearly denote the rarity, non-specificity and heterogeneity of clinical presentations of the disease, reinforcing the need for individualized therapeutic approaches.

Impact of Azathioprine use in chronic hypersensitivity pneumonitis patients.

INTRODUCTION: Systemic corticosteroids are widely used in chronic hypersensitivity pneumonitis (CHP); however, there is not much evidence to support their use, besides being associated with significant side effects. Azathioprine (AZA) use is common in CHP, although not prospectively tested in randomized controlled trials. Our objective was to evaluate the lung function trajectory of CHP patients after AZA initiation, as well as to assess the safety profile of this drug. METHODS: Retrospective analysis of patients initiated on AZA following a multidisciplinary team diagnosis of CHP. The longitudinal trajectory of lung function in the first 2 years of treatment was assessed. RESULTS: Thirty-five out of 62 patients (56.5%) remained on treatment after 2 years. AZA treatment was associated with a significant improvement in forced vital capacity (FVC) at 12 and 24 months (p = 0.015 and p < 0.001, respectively). A slight increase in total lung capacity (TLC) and 6-min walking test (6MWT) were also reported, although it did not reach statistical differences at the end of 2 years. No changes in diffusion capacity for carbon monoxide (DLCO) were observed. CONCLUSIONS: This is the first study identifying an improvement in lung function (FVC) of CHP patients on AZA treatment for 2 years. Prospective studies are needed to confirm these results and to more adequately select CHP patients who may benefit from AZA.

Cortical grey matter sodium accumulation is associated with disability and secondary progressive disease course in relapse-onset multiple sclerosis.

OBJECTIVE: Sodium (23Na)-MRI is an emerging imaging technique to investigate in vivo changes in tissue viability, reflecting neuroaxonal integrity and metabolism. Using an optimised 23Na-MRI protocol with smaller voxel sizes and improved tissue contrast, we wanted to investigate whether brain total sodium concentration (TSC) is a biomarker for long-term disease outcomes in a cohort of patients with relapse-onset multiple sclerosis (MS), followed from disease onset. METHODS: We performed a cross-sectional study in 96 patients followed up ~ 15 years after a clinically isolated syndrome (CIS) and 34 healthy controls. Disease course was classified as CIS, relapsing-remitting MS or secondary progressive MS (SPMS). We acquired 1H-MRI and 23Na-MRI and calculated the TSC in cortical grey matter (CGM), deep grey matter, normal-appearing white matter (WM) and WM lesions. Multivariable linear regression was used to identify independent associations of tissue-specific TSC with physical disability and cognition, with adjustment for tissue volumes. RESULTS: TSC in all tissues was higher in patients with MS compared with healthy controls and patients who remained CIS, with differences driven by patients with SPMS. Higher CGM TSC was independently associated with Expanded Disability Status Scale (R2=0.26), timed 25-foot walk test (R2=0.23), 9-hole peg test (R2=0.23), Paced Auditory Serial Addition Test (R2=0.29), Symbol Digit Modalities Test (R2=0.31) and executive function (R2=0.36) test scores, independent of grey matter atrophy. CONCLUSIONS: Sodium accumulation in CGM reflects underlying neuroaxonal metabolic abnormalities relevant to disease course heterogeneity and disability in relapse-onset MS. TSC and should be considered as an outcome measure in future neuroprotection trials.

NK and NKT cells in the diagnosis of diffuse lung diseases presenting with a lymphocytic alveolitis.

BACKGROUND: Diffuse lung diseases (DLD) are characterized by different immunophenotypes in the bronchoalveolar lavage fluid (BALF). We aimed to evaluate the diagnostic value of BALF NK and NKT cell counts of patients with DLD and lymphocytic alveolitis. METHODS: We assessed 202 patients with DLD, who underwent BALF immunophenotyping. Samples were routinely processed by flow cytometry and lymphocyte subsets were compared between patients with sarcoidosis (n = 106), hypersensitivity pneumonitis (HP; n = 53), and other DLDs (n = 43). We compared absolute counts and percentages of NK and NKT cells between patients with HP versus the remaining DLD patients. To assess the accuracy of BALF lymphocyte subsets in the diagnosis of HP, we calculated the respective areas under the receiver operating characteristic curves (AUC-ROC). RESULTS: Patients with HP had significantly higher numbers of BALF NK cells, and its percentage was significantly associated with a higher odds of HP, even after adjustment for the NKT and CD8+ cells. For the absolute number of BALF NK cells, we found an AUC-ROC of 0.76 (95%CI = 0.68-0.84) when comparing patients with HP versus the remaining DLD. The cut-offs of 2000 NK cells/mL and of 2.4% NK cells in the BALF had a specificity and a negative predictive value over 80% for the diagnosis of HP. BALF NK cells absolute counts were significantly higher in HP patients with a restrictive pattern. No such differences were observed for NKT cells. CONCLUSIONS: BALF NK immunophenotyping may be a helpful adjunct to the diagnostic work-up of DLD, particularly in the differential diagnosis of HP.

Diagnostic Yield of Computed Tomography-Guided Transthoracic Lung Biopsy in Diffuse Lung Diseases.

BACKGROUND: Accurate diagnosis is essential for successful management of diffuse lung disease (DLD). Histopathology may sometimes be necessary. Surgical lung biopsy, the gold standard, carries a risk of morbidity and mortality. Computed tomography (CT) guided transthoracic lung biopsy (CT-TLB) is a minimally invasive method for obtaining lung tissue. However, its diagnostic yield is unknown in DLD. OBJECTIVE: To assess the diagnostic yield of CT-TLB in DLD according to the predominant high-resolution CT (HRCT) patterns. METHODS: Between January 2009 and December 2016, we enrolled all consecutive adult patients with suspicion of DLD who underwent CT-guided transthoracic lung biopsy during the diagnostic work-up. All biopsies were performed by a senior interventional radiologist using CT fluoroscopy. RESULTS: The study included 169 patients (50.3% men) with a mean (±SD) age of 58.3 ± 14 years. Consolidation was the predominant HRCT pattern. A definitive or probable diagnosis was made in 66.3%. The most frequent diagnosis was organizing pneumonia (36.2%). Diagnostic yield was higher when the predominant HRCT pattern was consolidation or nodular. The most common complication was pneumothorax (17.8%); other complications included mild hemoptysis (7.7%), hemothorax (1.2%), and death (0.59%). No acute exacerbation of the underlying condition was observed. CONCLUSIONS: CT-TLB proved to be accurate and safe for the diagnosis of DLD. The overall diagnostic yield of the procedure was 66.3%. Given its low complication rates, CT-TLB can be an option in patients whose respiratory function is seriously impaired and in those with substantial comorbidities, where more invasive procedures cannot be performed for reasons of safety.

Obstructive sleep apnoea in patients with fibrotic diffuse parenchymal lung disease-characterization and treatment compliance assessment.

INTRODUCTION: Recent studies have reported a high prevalence of obstructive sleep apnoea (OSA) among patients with diffuse parenchymal lung disease (DPLD), mainly idiopathic pulmonary fibrosis (IPF). Effective OSA treatment appears to have a positive impact on morbidity and mortality in these patients. However, poor compliance to positive airway pressure (PAP) treatment in fibrotic DPLD patients has been reported. The aims of our study were to characterize patients with fibrotic DPLD and OSA and to assess their compliance to PAP treatment. METHODS: Forty-nine patients with fibrotic DPLD underwent level III polysomnography. Auto-adjusting PAP (APAP) treatment was proposed for those patients with moderate-to-severe OSA and those with mild OSA with daytime sleepiness and/or cardiovascular disease. The APAP treatment compliance was assessed after 1 month of therapy. RESULTS: The distribution of the 49 fibrotic DPLD patients included was as follows: 21 with chronic hypersensitivity pneumonitis, 12 with IPF, 10 with connective-tissue associated DPLD, 4 with stage IV sarcoidosis, 1 with idiopathic pleuropulmonary fibroelastosis, and 1 with DPLD-associated vasculitis. Thirty-four (69.4%) of the patients presented with OSA; 22 had mild OSA, and 12 had moderate-to-severe OSA. APAP treatment was prescribed in 17 of the patients. After 1 month of therapy, all patients used APAP more than 70% of the nights for more than 4 h per night. CONCLUSION: We found a high prevalence of OSA among all of the patients with fibrotic DPLD (not only IPF). Despite certain difficulties, it was possible to achieve good APAP compliance in these patients.

Integrin α E ß 7 (CD103) expression in bronchoalveolar lymphocytes of patients with hypersensitivity pneumonitis.

PURPOSE: CD4+/CD8+ ratio in bronchoalveolar lavage fluid (BALF) often retrieves contradictory findings when used for diagnosis of sarcoidosis and hypersensitivity pneumonitis (HP), so CD103+ has been investigated as a possible differential marker. We aimed to compare CD103+ expression in BALF T-lymphocytes between patients with HP, sarcoidosis and other interstitial lung diseases (ILD). METHODS: An observational study carried out over a 2-year period included consecutive patients with suspected ILD who underwent BALF as part of their initial diagnostic work-up; CD103+ expression on BALF T-lymphocytes was evaluated. After a final diagnosis established according to international criteria, three patient subgroups-HP, ILD (which included idiopathic interstitial pneumonia and connective tissue disease-associated lung disorders) and sarcoidosis-were considered for further analysis. RESULTS: A total of 77 subjects were enrolled, 20 with HP, 16 with other ILD and 41 with sarcoidosis. A significantly higher number of CD4+ CD103+ and CD8+ CD103+ lymphocytes were found in HP patients. Among patients with sarcoidosis, 12 (29.3 %) presented a BALF CD4+/CD8+ <3.5, all of them with histological confirmation. Compared to these patients, also statistically significant higher CD4+ CD103+ counts in HP patients were observed (p = 0.007). Among HP patients, although bird fanciers (n = 14) presented higher percentages of both CD4+ CD103+ and CD8+ CD103+ T-lymphocytes than those with work-related HP (n = 5), the differences did not reach statistical significance. CONCLUSIONS: Patients with HP present significantly higher counts of CD103+ T-lymphocytes in BALF, both in the CD4+ and CD8+ subsets, when compared to sarcoidosis, even with sarcoidosis subgroup presenting a BALF CD4+/CD8+ <3.5. The expression of CD103 may help in the interpretation of BALF data in these diffuse granulomatous lung disorders.

Technical aspects of inter-recti distance measurement with ultrasonographic imaging for physiotherapy purposes: the scoping review.

BACKGROUND: Inter-recti distance (IRD) measurement using musculoskeletal USI has been used in physiotherapy research, in particular, to investigate pregnancy-related diastasis recti abdominis (DRA) and to seek its effective treatment methods. Severe and untreated diastasis may result in the formation of umbilical or epigastric hernias. OBJECTIVE: This study aimed to systematically map physiotherapy-related research articles that included descriptions of IRD measurement procedures using USI to present their similarities and differences, and formulate recommendations on the procedure. DESIGN: A scoping review was conducted according to PRISMA-ScR guidelines, including 49 of 511 publications from three major databases. Publications were selected and screened by two independent reviewers whose decisions were consulted with a third reviewer. The main synthesized data items were: the examinees' body position, breathing phase, measurement sites, and DRA screening methods. The final conclusions and recommendations were the result of a consensus between seven reviewers from four research centers. RESULTS: Studies used 1-5 measurement sites that were differently determined. IRD was measured at the umbilicus (n = 3), at its superior (n = 16) and/or inferior border (n = 9), and at different levels: between 2 and 12 cm above the umbilicus, or a third of the distance and halfway between the umbilicus and xiphoid (n = 37); between 2 and 4.5 cm below the umbilicus or halfway between the umbilicus and pubis (n = 27). Different approaches were used to screen subjects for DRA. CONCLUSIONS: The discrepancies between the measurement procedures prevent between-study comparisons. The DRA screening method should be standardized. IRD measurement protocol standardization has been proposed. CRITICAL RELEVANCE STATEMENT: This scoping review indicates that the inter-recti distance measurement procedures using ultrasound imaging differ between studies, preventing between-study comparisons. Based on the results synthesis, the measurement protocol standardization has been proposed. KEY POINTS: The inter-recti distance measurement procedures using USI differ between studies. Proposed standardization concerns body position, breathing phase, measurements number per location. Determination of measurement locations considering individual linea alba length is suggested. Recommended locations: umbilical top, ½ of umbilical top-xiphoid, » of umbilical top-xiphoid/pubis distances. Diastasis recti abdominis diagnostic criteria are needed for proposed measurement locations.

"Sound" Decisions: The Combined Role of Ambient Noise and Cognitive Regulation on the Neurophysiology of Food Cravings.

Our ability to evaluate long-term goals over immediate rewards is manifested in the brain's decision circuit. Simplistically, it can be divided into a fast, impulsive, reward "system 1" and a slow, deliberate, control "system 2." In a noisy eating environment, our cognitive resources may get depleted, potentially leading to cognitive overload, emotional arousal, and consequently more rash decisions, such as unhealthy food choices. Here, we investigated the combined impact of cognitive regulation and ambient noise on food cravings through neurophysiological activity. Thirty-seven participants were recruited for an adapted version of the Regulation of Craving (ROC) task. All participants underwent two sessions of the ROC task; once with soft ambient restaurant noise (∼50 dB) and once with loud ambient restaurant noise (∼70 dB), while data from electroencephalography (EEG), electrodermal activity (EDA), and self-reported craving were collected for all palatable food images presented in the task. The results indicated that thinking about future ("later") consequences vs. immediate ("now") sensations associated with the food decreased cravings, which were mediated by frontal EEG alpha power. Likewise, "later" trials also increased frontal alpha asymmetry (FAA) -an index for emotional motivation. Furthermore, loud (vs. soft) noise increased alpha, beta, and theta activity, but for theta activity, this was solely occurring during "later" trials. Similarly, EDA signal peak probability was also higher during loud noise. Collectively, our findings suggest that the presence of loud ambient noise in conjunction with prospective thinking can lead to the highest emotional arousal and cognitive load as measured by EDA and EEG, respectively, both of which are important in regulating cravings and decisions. Thus, exploring the combined effects of interoceptive regulation and exteroceptive cues on food-related decision-making could be methodologically advantageous in consumer neuroscience and entail theoretical, commercial, and managerial implications.

Mortality risk prediction with ILD-GAP index in a fibrotic hypersensitivity pneumonitis cohort.

BACKGROUND: Fibrotic hypersensitivity pneumonitis (fHP) is associated with significant morbidity and mortality. Interstitial lung disease-gender-age-physiology (ILD-GAP) performance in fHP outside the initial cohort was never performed. AIM: To assess the ILD-GAP index's ability to predict mortality in a Portuguese cohort of patients with fHP and analyse whether other clinical variables add value. METHODS: Retrospective analysis of fHP cohort in two Portuguese ILD centres. The baseline ILD-GAP index was calculated. Survival was analysed in months; mortality was the primary outcome. Univariate and multivariate analyses to identify mortality risk factors were performed. RESULTS: A total of 141 patients were included. Fifty-three patients (37.6%) died during the follow-up. The usual interstitial pneumonia (UIP) pattern was found in 49.6%, and their survival was inferior to non-UIP [32 months (interquartile range, IQR = 19, 60) versus 52 months (IQR = 28, 98), p = 0.048]. Patients with an ILD-GAP index higher than three double their risk of mortality [hazard ratio (HR) = 6.48, 95% confidence interval (CI) = (3.03-13.96)] when compared with the patients with an index between 2 and 3 [HR = 3.04, 95% CI = (1.62-5.71)] adjusting for acute exacerbation history. Even though UIP patients had worse survival, it did not reach statistical significance when UIP pattern was added to this model. Acute exacerbation history was an independent risk factor for mortality; however, ILD-GAP still predicted mortality after adjusting for this factor. PaO2 and 6-minute walk test desaturation were not significant risk factors. CONCLUSION: ILD-GAP index is a good predictor for mortality in fHP, even after adjusting for other mortality risk factors.

Pleuroparenchymal fibroelastosis as a complication of chemotherapy: A case report.

Pleuroparenchymal fibroelastosis (PPFE) is a rare and recently described distinct pattern of lung apical fibrosis involving the upper lobe parenchyma and pleural dome. PPFE has definable and reproducible clinical, radiological and histopathological criteria, which allowed its classification as an independent interstitial lung disease. Several factors have been associated with PPFE, such as chemotherapy, especially with alkylating agents. The authors present a case of a 34-year-old female with previous history of Hodgkin lymphoma treated with first line chemotherapy (doxorubicin, bleomycin, vinblastine and dacarbazine). The patient had no other known comorbidities or relevant exposure to lung irritants. A total of 2 years after completing cancer treatment, the patient developed clinical and radiological features of PPFE. Given their previous history of malignancy, a biopsy of the lesion was obtained, which confirmed the diagnosis of PPFE. The authors present this case to raise awareness of this disease and to demonstrate that PPFE can develop months to years following chemotherapy treatment. Moreover, to date, none of these chemotherapy agents have been associated with the development of PPFE.

Predictive value of common genetic variants in idiopathic pulmonary fibrosis survival.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial pneumonia of unknown etiology. The role of genetic risk factors has been the focus of numerous studies probing for associations of genetic variants with IPF. We aimed to determine whether single-nucleotide polymorphisms (SNPs) of four candidate genes are associated with IPF susceptibility and survival in a Portuguese population. A retrospective case-control study was performed with 64 IPF patients and 74 healthy controls. Ten single-nucleotide variants residing in the MUC5B, TOLLIP, SERPINB1, and PLAU genes were analyzed. Single- and multi-locus analyses were performed to investigate the predictive potential of specific variants in IPF susceptibility and survival. Multifactor dimensionality reduction (MDR) was employed to uncover predictive multi-locus interactions underlying IPF susceptibility. The MUC5B rs35705950 SNP was significantly associated with IPF: T allele carriers were significantly more frequent among IPF patients (75.0% vs 20.3%, P < 1.0 × 10-6). Genotypic and allelic distributions of TOLLIP, PLAU, and SERPINB1 SNPs did not differ significantly between groups. However, the MUC5B-TOLLIP T-C-T-C haplotype, defined by the rs35705950-rs111521887-rs5743894-rs5743854 block, emerged as an independent protective factor in IPF survival (HR = 0.37, 95% CI 0.17-0.78, P = 0.009, after adjustment for FVC). No significant multi-locus interactions correlating with disease susceptibility were detected. MUC5B rs35705950 was linked to an increased risk for IPF, as reported for other populations, but not to disease survival. A haplotype incorporating SNPs of the MUC5B-TOLLIP locus at 11p15.5 seems to predict better survival and could prove useful for prognostic purposes and IPF patient stratification. KEY MESSAGES : The MUC5B rs35705950 minor allele is associated with IPF risk in the Portuguese. No predictive multi-locus interactions of IPF susceptibility were identified by MDR. A haplotype defined by MUC5B and TOLLIP SNPs is a protective factor in IPF survival. The haplotype may be used as a prognostic tool for IPF patient stratification.

Lung cancer: atypical brain metastases mimicking neurocysticercosis.

The authors describe a case of a 47-year-old male smoker with a 3-month history of hearing loss, tinnitus and dizziness. Physical examination revealed neurosensory hearing loss. Small rounded hypodensities without mass effect were evident in a computed tomography scan of the head, confirmed by brain magnetic resonance imaging as multiple cystic lesions in both cerebral and cerebellar hemispheres, without perilesional edema or gadolinium enhancement, suggestive of neurocysticercosis. Extraparenchymal involvement was also noted. Albendazole and dexamethasone were started. As a chest radiograph showed a bilateral reticulonodular pattern, a bronchoscopy was performed showing normal results. However, transbronchial biopsy revealed lung adenocarcinoma. Thoracoabdominopelvic computed tomography scan showed secondary lung and bone lesions. Since brain lesions were not suggestive of secondary tumor lesions, a brain biopsy was performed confirming metastatic disease. This case illustrates some peculiar imagiological features of brain metastases in lung cancer, indicating that sometimes invasive procedures are required to establish a definitive diagnosis.

APAP impact on metabolic syndrome in obstructive sleep apnea patients.

PURPOSE: Prevalence of metabolic syndrome (MS) in obstructive sleep apnea (OSA) patients is high. The effect of autoadjusting positive airway pressure (APAP) on MS remains unclear. This study aimed to determine the prevalence of MS in OSA patients before and 6 months after APAP, and to identify potential determinants of metabolic status change. METHODS: Seventy-four male patients with moderate to severe OSA were enrolled. MS diagnosis was established according to the National Cholesterol Education Program/Adult Treatment Panel III. APAP was prescribed to all patients. RESULTS: In the studied population, mean age was 55.9 years (SD 10.7 years), median body mass index (BMI), Epworth sleepiness scale (ESS), and respiratory disturbance index (RDI) were 33.4 kg/m(2) (interquartile range (IQR) 8.4 kg/m(2)), 12.0 (IQR 8.0), and 46.9/h (IQR 33.6/h), respectively. Prevalence of MS before and 6 months after APAP was 63.5% and 47.3%, respectively, and this difference was statistically significant (p = 0.004). In the subgroup of patients with MS at baseline (n = 47), 14 did not present MS after APAP. In these patients, a significant negative association with RDI (p = 0.016) and a positive association with percent of total days of usage (p = 0.014) were found. Blood pressure (p = 0.018) and serum triglycerides (p = 0.001) had a statistically significant reduction during this period. In patients that still had MS, 22.2% presented a reduction of the number of MS criteria. CONCLUSIONS: After 6 months, APAP reduced the prevalence of MS, mainly in patients with less severe OSA and with a better therapeutic compliance. Blood pressure and serum triglycerides reduction contributed to this metabolic status change.