Introduction: Progressive interstitial lung diseases (ILDs) are rare but severe diseases, with high mortality and morbidity, with no effective pharmacological treatment allowing for long-term remission, and therefore no clear therapeutic recommendations. Several ILDs present inflammatory components (ILDic), which may justify the use of anti-inflammatory and immunosuppressive drugs, as first-step therapy. Except for systemic sclerosis (SSc)-ILD and sarcoidosis, the evidence in favor of this approach is very weak.
View Article and Find Full Text PDFBackground: Interstitial lung disease (ILD) is rarer in children (chILD) than adults, but with increasing diagnostic awareness, more cases are being discovered. chILD prognosis is often poor, but increasing numbers are now surviving into adulthood.
Aim: To characterize chILD-survivors and identify their impact on adult-ILD centers.
Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease that may progress towards pulmonary fibrosis. Data about fibrosis prevalence and risk factors are lacking.
Methods: In this retrospective multicentre nationwide cohort, we included patients newly diagnosed with aPAP between 2008 and 2018 in France and Belgium.
A new approach in the finite difference framework is developed, which consists of three steps: choosing the dimension of the local approximation subspace, constructing a vector basis for this subspace, and determining the coefficients of the linear combination. New schemes were developed to form the basis of the local approximation subspace, which were derived by approximating only the k 2 u term of the Helmholtz equation. The construction of a basis of the local approximation subspace allows the new approach to be able to represent any finite difference scheme that belongs to this subspace.
View Article and Find Full Text PDFIntroduction: Cutaneous squamous cell carcinoma (cSCC) is the second most common skin cancer. Surgery is the standard of care with curative intent of localized disease; however, for large head and neck cSCCs, it is associated with high morbidity. Cemiplimab, an antibody anti programmed cell death-1 (PD-1) protein, is effective in locally advanced unresectable or metastatic cSCC.
View Article and Find Full Text PDFObjectives: to identify factors associated with preventable infant deaths, classified as neonatal and post-neonatal.
Methods: this is an epidemiological and population-based study relating to 2020. Data from the Mortality Information System (MIS) and the preventability classification proposed in the Brazilian Health System List of Causes of Deaths Preventable by Interventions were used.
Background: Epidemiology of spontaneous subarachnoid hemorrhage (SAH) and unruptured intracranial aneurysm (UIA) is valuable for determining neurosurgical and general health care effectiveness. There is an information gap regarding these conditions in middle- and low-income countries. Therefore, we aimed to investigate hospitalization and mortality rates for SAH and UIA in Brazil from 2011 to 2019.
View Article and Find Full Text PDFPremature telomere shortening or telomere instability is associated with a group of rare and heterogeneous diseases collectively known as telomere biology disorders (TBDs). Here we identified two unrelated individuals with clinical manifestations of TBDs and short telomeres associated with the identical monoallelic variant c.767A>G; Y256C in Although the replication protein A2 (RPA2) mutant did not affect ssDNA binding and G-quadruplex-unfolding properties of RPA, the mutation reduced the affinity of RPA2 with the ubiquitin ligase RFWD3 and reduced RPA ubiquitination.
View Article and Find Full Text PDFObjective: to investigate factors associated with digital health literacy, hand hygiene knowledge and behavior among Brazilian adolescents during the COVID-19 pandemic.
Method: cross-sectional study with 473 adolescents aged 15 to 19. Data were collected virtually between June and August 2021 through a questionnaire on sociodemographic characteristics, digital health literacy, knowledge and behavior regarding hand hygiene.
Background: Sarcoidosis is an idiopathic systemic granulomatosis whose evolution is self-limiting in most cases. However, it can progress to organ damage that menaces the vital or functional prognosis of patients. Sarcoidosis itself, but also its comorbidities, can pose a threat to the patient, require rapid initiation of treatment, and justify emergency hospitalization.
View Article and Find Full Text PDFDue to their potential positive outcomes, hamstring eccentric exercises are becoming increasingly popular in training regimens. Among the various exercises, the Nordic Hamstring Exercise (NHE) is the most common. Despite its popularity, there are still some doubts about its benefits and risks.
View Article and Find Full Text PDFSarcoidosis-associated pulmonary hypertension (SAPH) is a very severe complication of the disease, largely impacting its morbidity and being one of its strongest predictors of mortality. With the recent modifications of the hemodynamic definition of pulmonary hypertension (mean arterial pulmonary pressure >20 instead of <25 mmHg,) its prevalence is presently not precisely known, but it affects from 3 to 20% of sarcoid patients; mostly, although not exclusively, those with an advanced, fibrotic pulmonary disease. Its gold-standard diagnostic tool remains right heart catheterization (RHC).
View Article and Find Full Text PDFBackground: Several rare surfactant-related gene (SRG) variants associated with interstitial lung disease are suspected to be associated with lung cancer, but data are missing. We aimed to study the epidemiology and phenotype of lung cancer in an international cohort of SRG variant carriers.
Methods: We conducted a cross-sectional study of all adults with SRG variants in the OrphaLung network and compared lung cancer risk with telomere-related gene (TRG) variant carriers.
The utilisation of seaweeds as feed supplements has been investigated for their potential to mitigate enteric methane emissions from ruminants. Enteric methane emissions are the primary source of direct greenhouse gas emissions in livestock and significantly contribute to anthropogenic methane emissions worldwide. The aim of the present study is to evaluate the nutritional role and the in vitro effect on cumulative gas and methane production of (native species) and (invasive species), two species of red algae from the Azorean Sea, as well as the ability to reduce biogas production when incubated with single pasture ( and ) as substrate.
View Article and Find Full Text PDFObjective: To analyze the relationship between sociodemographic and clinical factors with health literacy in Brazilian adolescents.
Method: This is a cross-sectional study with 526 adolescents aged 14 to 19. Data were collected virtually between July and September 2021 using a sociodemographic characterization questionnaire, clinical profile and the Health Literacy Assessment Tool - Portuguese version.
Background And Objective: Variants in surfactant genes SFTPC or ABCA3 are responsible for interstitial lung disease (ILD) in children and adults, with few studies in adults.
Methods: We conducted a multicentre retrospective study of all consecutive adult patients diagnosed with ILD associated with variants in SFTPC or ABCA3 in the French rare pulmonary diseases network, OrphaLung. Variants and chest computed tomography (CT) features were centrally reviewed.