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Background: Respiratory failure is a severe complication in coronavirus disease 2019 (COVID-19) pneumonia that, in addition to oxygen therapy, may require continuous positive airway pressure (CPAP) support. It has been postulated that COVID-19 lung injury may share some features with those observed in hyperoxic acute lung injury. Thus, a correct target arterial oxygen tension ( P aO 2 ) during oxygen supplementation may be crucial to protect the lung from further tissue damage. The aims of this study were: 1) to evaluate the effects of conservative oxygen supplementation during helmet CPAP therapy on mortality and intensive care unit (ICU) admission in patients with COVID-19 and respiratory failure, and 2) to evaluate the effect of conservative oxygen supplementation on new-onset organ failure and secondary pulmonary infections., Methods: This was a single-centre, historically controlled study of patients with severe respiratory failure due to COVID-19 pneumonia, receiving either conservative or nonconservative oxygen supplementation during helmet CPAP. A cohort receiving conservative oxygen supplementation was studied prospectively in which oxygen supplementation was administered with a target P aO 2 <100 mmHg. Results of this cohort were compared with those of a cohort who had received liberal oxygen supplementation., Results: 71 patients were included in the conservative cohort and 75 in the nonconservative cohort. Mortality rate was lower in the conservative cohort (22.5% versus 62.7%; p<0.001). Rates of ICU admission and new-onset organ failure were lower in the conservative cohort (14.1% versus 37.3%; p=0.001 and 9.9% versus 45.3%; p<0.001, respectively)., Conclusions: In patients with COVID-19 and severe respiratory failure, conservative oxygen supplementation during helmet CPAP was associated with improved survival, lower ICU admission rate and less new-onset organ failure., Competing Interests: Conflict of interest: None declared., (Copyright ©The authors 2023.)

Natural Killer (NK) cells are key innate effectors of antiviral immune response, and their activity changes in ageing and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Here, we investigated the age-related changes of NK cell phenotype and function during SARS-CoV-2 infection, by comparing adult and elderly patients both requiring mechanical ventilation. Adult patients had a reduced number of total NK cells, while elderly showed a peculiar skewing of NK cell subsets towards the CD56 low CD16 high and CD56 neg phenotypes, expressing activation markers and check-point inhibitory receptors. Although NK cell degranulation ability is significantly compromised in both cohorts, IFN-γ production is impaired only in adult patients in a TGF-β-dependent manner. This inhibitory effect was associated with a shorter hospitalization time of adult patients suggesting a role for TGF-β in preventing an excessive NK cell activation and systemic inflammation. Our data highlight an age-dependent role of NK cells in shaping SARS-CoV-2 infection toward a pathophysiological evolution., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Fionda, Ruggeri, Sciumè, Laffranchi, Quinti, Milito, Palange, Menichini, Sozzani, Frati, Gismondi, Santoni and Stabile.)

False-negative cases of #COVID19 are being increasingly reported. Laboratory diagnosis through RT-PCR testing alone lacks adequate sensitivity to be recommended as the only valid criterion to confirm COVID-19 diagnosis. https://bit.ly/2BLFnEe., Competing Interests: Conflict of interest: M. Di Paolo has nothing to disclose. Conflict of interest: A. Iacovelli has nothing to disclose. Conflict of interest: F. Olmati has nothing to disclose. Conflict of interest: I. Menichini has nothing to disclose. Conflict of interest: A. Oliva has nothing to disclose. Conflict of interest: M. Carnevalini has nothing to disclose. Conflict of interest: E. Graziani has nothing to disclose. Conflict of interest: C.M. Mastroianni has nothing to disclose. Conflict of interest: P. Palange has nothing to disclose., (Copyright ©ERS 2020.)

Minimal clinically important difference (MCID) can be defined as the smallest change or difference in an outcome measure that is perceived as beneficial and would lead to a change in the patient's medical management.The aim of the current expert consensus report is to provide a "state-of-the-art" review of the currently available literature evidence about MCID for end-points to monitor asthma control, in order to facilitate optimal disease management and identify unmet needs in the field to guide future research.A series of MCID cut-offs are currently available in literature and validated among populations of asthmatic patients, with most of the evidence focusing on outcomes as patient reported outcomes, lung function and exercise tolerance. On the contrary, only scant and partial data are available for inflammatory biomarkers. These clearly represent the most interesting target for future development in diagnosis and clinical management of asthma, particularly in view of the several biologic drugs in the pipeline, for which regulatory agencies will soon require personalised proof of efficacy and treatment response predictors., Competing Interests: Conflict of interest: M. Bonini has nothing to disclose. Conflict of interest: M. Di Paolo has nothing to disclose. Conflict of interest: D. Bagnasco has nothing to disclose. Conflict of interest: I. Baiardini has nothing to disclose. Conflict of interest: F. Braido has nothing to disclose. Conflict of interest: M. Caminati has nothing to disclose. Conflict of interest: E. Carpagnano has nothing to disclose. Conflict of interest: M. Contoli reports grants from Chiesi, personal fees from Chiesi, AstraZeneca, Boehringer Ingelheim, Novartis Menarini, Mundipharma, Almirall and Zambon, and grants from University of Ferrara (Italy), outside the submitted work. Conflict of interest: A. Corsico has nothing to disclose. Conflict of interest: S. Del Giacco has nothing to disclose. Conflict of interest: E. Heffler has nothing to disclose. Conflict of interest: C. Lombardi has nothing to disclose. Conflict of interest: I. Menichini has nothing to disclose. Conflict of interest: M. Milanese has nothing to disclose. Conflict of interest: N. Scichilone has nothing to disclose. Conflict of interest: G. Senna has nothing to disclose. Conflict of interest: G.W. Canonica has nothing to disclose., (Copyright ©ERS 2020.)

Background: In Italy, basic health needs of patients with inherited bleeding disorders are met by a network of 50 haemophilia centres belonging to the Italian Association of Haemophilia Centres. Further emerging needs, due to the increased life expectancy of this patient group, require a multi-professional clinical management of the disease and provide a challenge to the organisation of centres.In order to achieve harmonised quality standards of haemophilia care across Italian Regions, an institutional accreditation model for haemophilia centres has been developed., Material and Methods: To develop an accreditation scheme for haemophilia centres, a panel of experts representing medical and patient bodies, the Ministry of Health and Regional Health Authorities has been appointed by the National Blood Centre. Following a public consultation, a technical proposal in the form of recommendations for Regional Health Authorities has been formally submitted to the Ministry of Health and has formed the basis for a proposal of Agreement between the Government and the Regions., Results: The institutional accreditation model for Haemophilia Centres was approved as an Agreement between the Government and the Regions in March 2013. It identified 23 organisational requirements for haemophilia centres covering different areas and activities., Discussion: The Italian institutional accreditation model aims to achieve harmonised quality standards across Regions and to implement continuous improvement efforts, certified by regional inspection systems. The identified requirements are considered as necessary and appropriate in order to provide haemophilia services as "basic healthcare levels" under the umbrella of the National Health Service. This model provides Regions with a flexible institutional accreditation scheme that can be potentially extended to other rare diseases.

Background: The Italian Association of Haemophilia Centres has developed a voluntary programme of professional accreditation of Haemophilia Centres, run by its members. Participation in the programme, which aims to foster staff involvement in clinical governance, includes both medical personnel and nurses., Materials and Methods: Accreditation is awarded provided the candidate Haemophilia Centre is able to adhere to a pre-established set of quality standards and meet a number of clinical and organisational requirements, previously defined on the basis of evidence-based medicine. Self-evaluation is the first step in the programme, followed by a site visit by a team of peer professionals experienced in quality auditing., Results: The programme has so far involved 21 Italian Haemophilia Centres. The comparison between self- and peer-evaluation revealed less discrepancies for disease-related than for organisational requirements, the latter being met to a lesser degree by most Haemophilia Centres., Discussion: This programme of professional accreditation developed by the Italian Association of Haemophilia Centres has the potential to describe, monitor and improve clinical and organisational performances in the management of patients with haemophilia and allied inherited coagulation disorders. It should also be seen as a contribution to the implementation of the strategy for improving professional governance in Haemophilia Centres.

Introduction: Due to the increase in life expectancy, patients with haemophilia and other inherited bleeding disorders are experiencing age-related comorbidities that present new challenges. In order to meet current and emerging needs, a model for healthcare pathways was developed through a project funded by the Italian Ministry of Health. The project aimed to prevent or reduce the social-health burden of the disease and its complications., Material and Methods: The National Blood Centre appointed a panel of experts comprising clinicians, patients, National and Regional Health Authority representatives. Following an analysis of the scientific and regulatory references, the panel drafted a technical proposal containing recommendations for Regional Health Authorities, which has been formally submitted to the Ministry of Health. Finally, a set of indicators to monitor haemophilia care provision has been defined., Results: In the technical document, the panel of experts proposed the adoption of health policy recommendations summarised in areas, such as: multidisciplinary integrated approach for optimal healthcare provision; networking and protocols for emergency care; home therapy; registries/databases; replacement therapy supply and distribution; recruitment and training of experts in bleeding disorders. The recommendations became the content of proposal of agreement between the Government and the Regions. Monitoring and evaluation of haemophilia care through the set of established indicators was partially performed due to limited available data., Conclusions: The project provided recommendations for the clinical and organisational management of patient with haemophilia. A particular concern was given to those areas that play a critical role in the comorbidities and complications prevention. Recommendations are expected to harmonise healthcare care delivery across regional networks and building the foundation for the national haemophilia network.

Introduction: Work Package 4 Development of the standardisation criteria of the European Haemophilia Network project has the main objective of implementing a common and shared European strategy for a certification system for two levels of Haemophilia Centres: European Haemophilia Treatment Centres and European Haemophilia Comprehensive Care Centres in the Member States of the European Union., Materials and Methods: An inclusive and participatory process for developing shared standards and criteria for the management of patients with inherited bleeding disorders has been carried out. The process has been implemented through four different consultation events involving the entire European community of stakeholders that significantly contributed in the drafting of the European Guidelines for the certification of Haemophilia Centres., Results: The Guidelines set the standards for the designation of centres that provide specialised and multidisciplinary care (Haemophilia Comprehensive Care Centres) as well as local routine care (Haemophilia Treatment Centres). Standards cover several issues such as: general requirements; patient care; advisory services; laboratory; networking of clinical and specialised services., Conclusions: The drafting of the European Guidelines for the certification of Haemophilia Centres was performed adopting a rigorous methodological approach. In order to build the widest possible consensus to the quality standards, the main institutional and scientific stakeholders have been involved. The resulting document will significantly contribute in promoting standardisation in the quality of diagnosis and treatment in European Haemophilia Centres.

Introduction: The European haemophilia community of professionals and patients has agreed on the principles of haemophilia care to address comprehensive optimal delivery of care which is nowadays scattered throughout Europe. Many of the health facilities call themselves Haemophilia Centres despite their variation in size, expertise and services provided. Only a small number of countries have Haemophilia Centre accreditation systems in place., Methods: In the framework of the European Haemophilia Network project, following an inclusive process of stakeholder involvement, the European Guidelines for the certification of haemophilia centres have been developed in order to set quality standards for European Haemophilia Centres and criteria for their certification., Results: The Guidelines define the standards and criteria for the designation of two levels of care delivery: European Haemophilia Treatment Centres, providing local routine care, and European Haemophilia Comprehensive Care Centres, providing specialised and multi-disciplinary care and functioning as tertiary referral centres. Additionally, they define standards about general requirements, patient care, provision of an advisory service and establishment of network of clinical and specialised services., Conclusions: The implementation of the European Guidelines for the certification of Haemophilia Centres will contribute to the reduction of health inequalities through the standardisation of quality of care in European Union Member States and could represent a model to be taken into consideration for other rare disease groups.