Definition of sinonasal and otological exacerbation in patients with primary ciliary dyskinesia: an expert consensus.

Background: Recurrent infections of the nose, sinuses and ears are common problems for people with primary ciliary dyskinesia. While pulmonary exacerbations in primary ciliary dyskinesia are defined, there is no definition for ear-nose-throat exacerbations, a potential outcome for research and clinical trials.

Methods: We set up an expert panel of 24 ear-nose-throat specialists, respiratory physicians, other healthcare professionals and patients to develop consensus definitions of sinonasal and otological exacerbations in children and adults with primary ciliary dyskinesia for research settings.

[Evaluation of local anesthesia with buffered Xylocaine in pleural procedures: The DOULAPLUX study].

Introduction: Pleural procedures are painful interventions. While there exist recommendations aimed at preventing pain induced by local anesthesia, they have never been evaluated with regard to the thoracic wall. The objective of this study was to evaluate the effectiveness of buffered lidocaine local anesthesia in pleural procedures.

Clearance of pathogenic erythrocytes is maintained despite spleen dysfunction in children with sickle cell disease.

In children with sickle cell disease (SCD), splenectomy is immediately beneficial for acute sequestration crises and hypersplenism (ASSC/HyS) but portends a long-term risk of asplenia-related complications. We retrieved peripheral and splenic red blood cells (RBCs) from 17 SCD children/teenagers undergoing partial splenectomy for ASSC/HyS, 12 adult subjects without RBC-related disease undergoing splenectomy (controls), five human spleens perfused ex vivo with Hb- and Hb-RBC, and quantified abnormal RBC by microscopy, spleen-mimetic RBC filtration, and adhesion assays. Spleens were analyzed by immunohistochemistry and transmission electron microscopy (TEM).

Infertility and pregnancy outcomes among adults with primary ciliary dyskinesia.

Study Question: What is the prevalence of infertility and ectopic pregnancies among individuals with primary ciliary dyskinesia (PCD)?

Summary Answer: We found that 39 of 50 men (78%) and 72 of 118 women (61%) with PCD were infertile and that women with PCD had an increased risk of ectopic pregnancies (7.6 per 100 pregnancies, 95% CI 4.7-12.

Validity and reliability of the chronic composite XA, an upper limb motor assessment using Active Range of Motion in patients with chronic stroke.

Background: Upper limb (UL) spastic paresis has been classically evaluated with assessments of passive movements with limited functional validity. The aim of this study was to assess whether a composite measure of active range of motion (AROM, or XA) is valid and reliable in chronic post-stroke spastic paresis.

Aim: The primary objective was to investigate the validity and reliability of a composite score, comprising multiple XA measurements, to assess UL spastic paresis in patients in chronic stages post-stroke.

A BEAT-PCD consensus statement: a core outcome set for pulmonary disease interventions in primary ciliary dyskinesia.

Background: Consistent use of reliable and clinically appropriate outcome measures is a priority for clinical trials, with clear definitions to allow comparability. We aimed to develop a core outcome set (COS) for pulmonary disease interventions in primary ciliary dyskinesia (PCD).

Methods: A multidisciplinary international PCD expert panel was set up.

COPD after "Tabouna" Exposure: A Distinct Phenotype in Tunisian Women?

Background: COPD due to exposure to combustible biomass is an increasingly recognized phenotype, particularly among women who use traditional ovens, known as 'Tabouna', for baking bread. This paper aims to investigate the clinical and functional characteristics of COPD in Tunisian female patients attributed to the use of 'Tabouna'.

Methods: A retrospective single-center cohort study was conducted on patients recruited from the Department of Respiratory Disease at A.

Fertility care among people with primary ciliary dyskinesia.

Introduction: Fertility care is important for people living with primary ciliary dyskinesia (PCD) who are at increased risk of fertility problems. We investigated fertility care in an international participatory study.

Methods: Participants of the COVID-PCD study completed an online questionnaire addressing fertility issues.

SPLF/SMFU/SRLF/SFAR/SFCTCV Guidelines for the management of patients with primary spontaneous pneumothorax.

Introduction: Primary spontaneous pneumothorax (PSP) is the presence of air in the pleural space, occurring in the absence of trauma and known lung disease. Standardized expert guidelines on PSP are needed due to the variety of diagnostic methods, therapeutic strategies and medical and surgical disciplines involved in its management.

Methods: Literature review, analysis of the literature according to the GRADE (Grading of Recommendation, Assessment, Development and Evaluation) methodology; proposals for guidelines rated by experts, patients and organizers to reach a consensus.

International survey among hepatologists and pulmonologists on the hepatic hydrothorax: plea for recommendations.

Background: The Hepatic hydrothorax is a pleural effusion related to portal hypertension; its diagnosis and therapeutic management may be difficult. The aims of this article are which follows: To gather the practices of hepatogastroenterologists or pulmonologists practitioners regarding the diagnosis and management of the hepatic hydrothorax.

Methods: Practitioners from 13 French- speaking countries were invited to answer an online questionnaire on the hepatic hydrothorax diagnosis and its management.

International use of objective structured clinical examinations in respiratory training: a European Respiratory Society early career member survey.

https://bit.ly/3Zee6zP.

SPLF/SMFU/SRLF/SFAR/SFCTCV Guidelines for the management of patients with primary spontaneous pneumothorax: Endorsed by the French Speaking Society of Respiratory Diseases (SPLF), the French Society of Emergency Medicine (SFMU), the French Intensive Care Society (SRLF), the French Society of Anesthesia & Intensive Care Medicine (SFAR) and the French Society of Thoracic and Cardiovascular Surgery (SFCTCV).

Introduction: Primary spontaneous pneumothorax (PSP) is the presence of air in the pleural space, occurring in the absence of trauma and known lung disease. Standardized expert guidelines on PSP are needed due to the variety of diagnostic methods, therapeutic strategies and medical and surgical disciplines involved in its management.

Methods: Literature review, analysis of literature according to the GRADE (Grading of Recommendation Assessment, Development and Evaluation) methodology; proposals for guidelines rated by experts, patients, and organizers to reach a consensus.

Discriminating young platelets on human leukocyte antigen-I expression highlights their extremely high reactivity potential.

Background: The platelet population is heterogeneous, with different subsets that differ on the basis of their function and reactivity. An intrinsic factor participating in this difference of reactivity could be the platelet age. The lack of relevant tools allowing a formal identification of young platelets prevents so far to draw solid conclusions regarding platelet reactivity.

Anti-HLA Class I alloantibodies in platelet transfusion refractoriness: From mechanisms and determinants to therapeutic prospects.

Patients with hematological disorders and severe thrombocytopenia require extensive and iterative platelet transfusion support. In these patients, platelet transfusion refractoriness represents a serious adverse transfusion event with major outcomes for patient care. Recipient alloantibodies against the donor HLA Class I antigens expressed at the cell surface of platelets result in a rapid removal of transfused platelets from the circulation and thus, therapeutic and prophylactic transfusion failure leading to a major bleeding risk.

Genomic regions underlying positive selection in local, Alpine cattle breeds.

We used genome-wide SNP data from 18 local cattle breeds from six countries of the Alpine region to characterize population structure and identify genomic regions underlying positive selection. The geographically close breeds Evolèner, Eringer, Valdostana Pezzata Nera, and Valdostana Castana were found to differ from all other Alpine breeds. In addition, three breeds, Simmental, and Original Braunvieh from Switzerland and Pinzgauer from Austria built three separate clusters.

Respiratory recovery trajectories after severe-to-critical COVID-19: a 1-year prospective multicentre study.

Background: Survivors of severe-to-critical coronavirus disease 2019 (COVID-19) may have functional impairment, radiological sequelae and persistent symptoms requiring prolonged follow-up. This pragmatic study aimed to describe their clinical follow-up and determine their respiratory recovery trajectories, and the factors that could influence them and their health-related quality of life.

Methods: Adults hospitalised for severe-to-critical COVID-19 were evaluated at 3 months and up to 12 months post-hospital discharge in this prospective, multicentre, cohort study.

Marginal zone B cells are responsible for the production of alloantibodies following platelet transfusion in mice.

Alloimmunization against platelets remains a potentially serious adverse transfusion event. Alloantibodies produced by the recipient, mainly directed against human leukocyte antigen class I donor antigens, can compromise the therapeutic efficacy of subsequent transfusions, and may lead to refractoriness. Because the mechanism of anti-HLA alloantibody formation is poorly understood, this study aimed to identify the cells involved in the platelet immune response by focusing on the spleen, the main organ that orchestrates this alloimmune response.

Impact of the COVID-19 pandemic and associated lockdown measures on the management, health, and behavior of the cystic fibrosis population in France during 2020 (MUCONFIN).

Background: Most of the studies on cystic fibrosis (CF) focused on SARS-CoV-2 prevalence and suggested a low incidence of infection in this population. We aimed to assess the impact of the pandemic and related lockdown measures implemented in May 2020 in response to the first wave of SARS-CoV-2 infection on healthcare access, health, and behavior in CF patients.

Methods: A national questionnaire opened online from May 15th, 2020 to June 11, 2020 was completed by 751 CF-patients, aged 14 years and over.

Perinatal mortality in Ireland: inequalities by socio-economic group and country of birth.

Background: While perinatal mortality rates have decreased in Ireland in recent years, it is not known if this reduction was shared equally among all groups. The aim of this study is to examine inequalities in perinatal mortality by country of birth and socio-economic group in Ireland between 2004 and 2019.

Methods: Data for the analysis was obtained from the National Perinatal Reporting System dataset, which includes all births (including stillbirths) in Ireland.