Alpha-1 antitripsyn deficiency and augmentation therapy in pregnancy: two case reports.

Alpha-1 antitrypsin deficiency (AATD) is an inherited condition characterized by reduced plasma levels of alpha-1 antitrypsin (AAT), often leading to pulmonary diseases primarily emphysema and/or chronic obstructive pulmonary disease (COPD), but also bronchiectasis, bronchial asthma, or other less common disorders. Early diagnosis enables AAT augmentation therapy, which has proven to be effective in slowing down functional decline and improving survival rates. This article presents two cases of pregnant women with rare allelic variants of AATD who received AAT augmentation therapy, exploring the limited evidence on its safety during pregnancy and the potential role of decreased serum AAT levels in pregnancy-related complications.

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Aim of the study was to assess the use of pharmacological therapies provided in Nursing Homes and Protected Residences of the Umbria Region, with reference to central nervous system medications. In 2022, 200 ddd (defined daily doses) per 100 bed-days were administered in Umbria's long term care facilities, representing a 6.2% decrease compared to the previous year.

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Objectives: To assess the safety profile of dietary supplements with a focus on those purchased online or reported via the internet.

Results: Among the 2,367 reports of Adverse Reactions (ARs) to dietary supplements collected within the Italian Phitovigilance System, 49.4% were from citizens and 33.

Long-Term High-Flow Nasal Therapy in Patients with Bronchiectasis of Different Severity: A Retrospective Cohort Study.

: High-flow nasal therapy (HFNT) has been shown to reduce exacerbations of COPD and some evidence displays benefits in non-cystic fibrosis bronchiectasis (NCFB) patients. The present study aimed to compare the effectiveness of 12 months of home HFNT on the annual exacerbation rate between mild/moderate and severe NCFB patients, classified by the bronchiectasis severity index (BSI). Secondary outcomes were the evaluation of the dyspnea, pulmonary function, and sputum cultures in both groups.

Use of antidepressant and antipsychotic drugs in subjects with hemophilia of the Umbria Region in the period 2011-2022.

Introduction: Chronic diseases, such as hemophilia, can evoke psychological sequelae and be associated with a higher risk of mental health disorders. The utilization of antidepressant and antipsychotic drugs in subjects with hemophilia is not completely understood and few data are available.

Objectives: The aim of this analysis is to describe use of antidepressant and antipsychotic drugs in subjects with hemophilia of the Umbria Region in the period 2011-2022.

η-Arene Ru(II) Complexes as Modulators of Amyloid Aggregation.

Inorganic medicinal compounds represent a unique and versatile source of potential therapeutics in many diseases and, more recently, in neurodegeneration. Herein we investigated the effects of two η-arene Ru(II) complexes on the self-aggregation processes of several amyloidogenic peptides endowed with different kinetics and primary sequences. The Ru(II) complexes exhibit, around the metal ion, two chlorides, one NHC = -heterocyclic carbene, with a glucosyl and a methyl substituent and separately a hexamethylbenzene, which is named , and one benzene, named .

Indications and techniques of non-invasive ventilation (NIV) in neuromuscular diseases.

The neuromuscular patients may experience the need for respiratory support due to the onset of respiratory failure. Some skills are essential to achieve therapeutic success. In addition to technical knowledge, it is essential to have knowledge relating to individual neuromuscular diseases.

An unusual way to improve lung function in congenital myopathies: the power of singing.

Congenital myopathies (CMs) are a clinically and genetically heterogeneous group of disorders characterized by early onset weakness, hypotonia and characteristic structural abnormalities in muscle fibres. Hypotonia and weakness can be present at birth or appear in infancy, and a static or slowly progressive clinical course may present with muscle weakness, loss of spontaneous movement, involuntary muscle activity, and muscle atrophy. Often patients develop a restrictive syndrome and respiratory failure and require respiratory support In our case, we described lung improvement and respiratory muscle training due to singing in a young patient, affected by CMs with a poor adherence to non-invasive mechanical ventilation.

Transplant with MZ genotype liver: what is the clinical pulmonary picture after 30 years? a case report and review of the literature.

Background: Alpha-1 antitrypsin, also known as alpha1 proteinase inhibitor, is a protein 90% synthesized by hepatocytes. Alpha-1 antitrypsin deficiency should be suspected if patients have unexplained emphysema or liver disease in the absence of others recognized causes. The diagnosis is based on tests that measure the amount of the enzyme in the blood and confirm by molecular analysis.

Psychological Factors Influencing Adherence to NIV in Neuromuscular Patients Dependent on Non Invasive Mechanical Ventilation: Preliminary Results.

Background: Non-invasive ventilation (NIV) is associated with improvement of both morbility and mortality in patients affected by neuromuscular diseases with chronic respiratory failure. Several studies have also shown that long-term NIV positively impacts the patient's quality of life and perception of disease status. Its effectiveness is likely related to the adherence to NIV.

The role of dexmedetomidine in ARDS: an approach to non-intensive care sedation.

Introduction: Severe COVID-19 is a life-threatening condition characterized by complications such as interstitial pneumonia, hypoxic respiratory failure, and acute respiratory distress syndrome (ARDS). Non-pharmacological intervention with mechanical ventilation plays a key role in treating COVID-19-related ARDS but is influenced by a high risk of failure in more severe patients. Dexmedetomidine is a new generation highly selective α2-adrenergic receptor (α2-AR) agonist that provides sedative effects with preservation of respiratory function.

Diagnosis of Paediatric Obstructive Sleep-Disordered Breathing beyond Polysomnography.

Obstructive sleep-disordered breathing (SDB) has significant impacts on health, and therefore, a timely and accurate diagnosis is crucial for effective management and intervention. This narrative review provides an overview of the current approaches utilised in the diagnosis of SDB in children. Diagnostic methods for SDB in children involve a combination of clinical assessment, medical history evaluation, questionnaires, and objective measurements.

Intermittent abdominal pressure ventilation management in neuromuscular diseases: a Delphi panel Consensus.

Background: Intermittent abdominal pressure ventilator (IAPV) use started in the 1930s for ventilatory assistance with muscular dystrophy patients. Later, the device was perfected and expanded for other neuromuscular disorders (NMD). In recent years, the morbidity and mortality tracheotomies and trach tubes related renewed the interest around IAPV.

Effects on Right Ventricular Function One Year after COVID-19-Related Pulmonary Embolism.

The aim of this study was to investigate the presence of subclinical cardiac dysfunction in recovered coronavirus disease 2019 (COVID-19) patients, who were stratified according to a previous diagnosis of pulmonary embolism (PE) as a complication of COVID-19 pneumonia. Out of 68 patients with SARS-CoV-2 pneumonia followed up for one year, 44 patients (mean age 58.4 ± 13.

Ruthenium complexes bearing glucosyl ligands are able to inhibit the amyloid aggregation of short histidine-peptides.

Neurodegenerative diseases are often characterized by the formation of aggregates of amyloidogenic peptides and proteins, facilitating the formation of neurofibrillary plaques. In this study, we investigate a series of Ru-complexes sharing three-legged piano-stool structures based on the arene ring and glucosylated carbene ligands. The ability of these complexes to bind amyloid His-peptides was evaluated by ESI-MS, and their effects on the aggregation process were investigated through ThT and Tyr fluorescence emission.

Remdesivir treatment and clinical outcome in non-severe hospitalized COVID-19 patients: a propensity score matching multicenter Italian hospital experience.

Introduction: Remdesivir exerts positive effects on clinical improvement, even though it seems not to affect mortality among COVID-19 patients; moreover, it was associated with the occurence of marked bradycardia.

Methods: We retrospectively evaluated 989 consecutive patients with non-severe COVID-19 (SpO ≥ 94% on room air) admitted from October 2020 to July 2021 at five Italian hospitals. Propensity score matching allowed to obtain a comparable control group.