Claudio Tirelli's research while affiliated with University of Milan and other places
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Publications (24)
Chronic Obstructive Pulmonary Disease (COPD) is a pathological condition of the respiratory system characterized by chronic airflow obstruction, associated with changes in the lung parenchyma (pulmonary emphysema), bronchi (chronic bronchitis) and bronchioles (small airways disease). In the last years, the importance of phenotyping and endotyping C...
Niemann–Pick Disease (NPD) is a rare autosomal recessive disease belonging to lysosomal storage disorders. Three types of NPD have been described: NPD type A, B, and C. NPD type A and B are caused by mutations in the gene SMPD1 coding for sphingomyelin phosphodiesterase 1, with a consequent lack of acid sphingomyelinase activity. These diseases hav...
Introduction: Obstructive Sleep Apnea (OSA) in children is characterized by repeated episodes of partial or complete obstruction of the upper airways that impair normal ventilation and cause hypoxia and sleep disruption. These episodes activate innate and adaptive immunity resulting in the production of proinflammatory cytokines: IL-1β, IL-6, TNF-α...
Background
Bronchoscopy is a useful technique adopted in the management of patients with COVID-19. 10–40% of COVID-19 survivors experience persistent symptoms. A comprehensive description of the utility and safety of bronchoscopy in the management of patients with COVID-19 sequelae is lacking. The aim of the study was to evaluate the role of bronch...
Background:
Severe alpha1-antitrypsin (AAT) deficiency (AATD) is associated with a high risk of airflow obstruction and emphysema. The risk of lung disease in those with intermediate AAT deficiency is unclear. Our aims were to compare pulmonary function, time of onset of symptoms, and indicators of quality of life among patients with severe AATD (...
Simple Summary
Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is responsible for the pandemic coronavirus disease-2019 (COVID-19), which can lead to severe pneumonia and acute respiratory distress syndrome (ARDS). The immune system plays a key role in the defense against COVID-19. Nonetheless, if dysregulated and hyperactivated, the i...
Idiopathic pulmonary fibrosis (IPF) is a rare disease of the lung with a largely unknown etiology and a poor prognosis. Intriguingly, forms of familial pulmonary fibrosis (FPF) have long been known and linked to specific genetic mutations. There is little evidence of the possible role of genetics in the etiology of sporadic IPF. We carried out a no...
Introduction:
Positive microbiological fungal culture from bronchoalveolar-lavage-fluid (BAL) for Aspergillus or tissue biopsy and the detection of high levels of Aspergillus Galactomannan (GM) are commonly considered standard for diagnosing Invasive Pulmonary Aspergillosis (IPA). However, Aspergillus infection induces both cellular and humoral im...
Background:
Chronic cough is a common symptom, addressed in the clinical setting by empirical treatment together with some laboratory investigations. Purpose of the present study is to investigate the value of testing eosinophilic cationic protein (ECP) serum levels combined with other diagnostic procedures and empirical treatment in the diagnosti...
Despite low rates of bacterial co-infections, most COVID-19 patients receive antibiotic therapy. We hypothesized that patients with positive pneumococcal urinary antigens (PUAs) would benefit from antibiotic therapy in terms of clinical outcomes (death, ICU admission, and length of stay). The San Matteo COVID-19 Registry (SMACORE) prospectively enr...
Niemann-Pick Disease type B (NPDB) is a rare autosomal recessive disease belonging to the family of lysosomal storage disorders. NPDB is caused by mutations of sphingomyelin phosphodiesterase 1 gene (SMPD1) and is characterized by hepatosplenomegaly, interstitial lung disease, recurrent pulmonary infections, and neurologic disorders. Bronchiectasis...
Properly performed staging in non-small-cell lung cancer (NSCLC) is necessary to avoid wrong therapeutic decisions. Here we present a case which manifested as advanced NSCLC but ultimately was composed of two different and rare pathologies. The first is a TTF-1 positive axillary lymph node that could be defined either as an unusual isolated differe...
Pulmonary involvement in Sjogren syndrome (SS) could manifest as cystic lung disease (CLD). CLD in SS includes lymphocytic interstitial pneumonia (LIP) and pulmonary amyloidosis. Differential diagnosis usually requires surgical lung biopsy, whereas CT-guided percutaneous fine needle aspiration biopsy (CT-FNAB) has not yet explored. We describe the...
Background
The staging of interstitial lung disease (ILD) is important to monitor disease progression and for prognostication. A disease severity scale of Systemic Sclerosis (SSc)-related lung disease has long been proposed (i.e. Medsger’s severity scale). This scale was mostly developed by discussion and consensus and stage thresholds were not com...
Purpose of the review
Antisynthetase syndrome (ASSD) is a rare, heterogeneous, systemic disease characterized by the clinical triad of arthritis, myositis, and interstitial lung disease (ILD) together with other accompanying findings, which often has a progressive and life-threatening evolution. The rarity of this condition and the lack of classifi...
Interstitial lung disease (ILD) encompasses a wide range of parenchymal lung pathologies with different clinical, histological, radiological, and serological features. Follow-up, treatment, and prognosis are strongly influenced by the underlying pathogenesis. Considering that an ILD may complicate the course of any connective tissue disease (CTD) a...
Background
Double aortic arch is a rare congenital and complete vascular ring around trachea and esophagus. It is usually diagnosed during infancy. The symptoms are generally related to respiratory and gastroesophageal tracts.
Case presentation
A 20-year-old female patient was referred to our outpatient clinic for persistent dry cough. She had a h...
Background
Over the years it has been widely stated that approximately one-third of asthmatic women experience worsening of the disease during pregnancy. However, the literature has not been reviewed systematically and the meta-analytic reviews include old studies. This study aimed to examine whether the prevalence of worsening asthma during pregna...
Background: Double aortic arch is a rare congenital and complete vascular ring around trachea and esophagus. It is usually diagnosed during infancy. The symptoms are generally related to respiratory and gastroesophageal tracts. Case presentation: A 20-year-old female patient was referred to our outpatient clinic for persistent dry cough. She had a...
Citations
... Additionally, peri-bronchovascular interstitial thickening may be observed along with interlobular septal thickening. Centrilobular nodular opacities or a "crazy paving" pattern, with the typical presence of ground-glass opacities and thickening of interlobular septa, may also be observed and mimic lipoid pneumonia [1,12,27]. Other non-specific HRCT findings, such as segmental atelectasis and bronchiectasis, have been reported and have been specifically correlated with a particular SMPD1 gene mutation (p.Ala461⁄4) [2]. ...
... The disease presents a non-reversible obstruction in pulmonary function tests. Environmental factors, exposure to toxic inhalants, along with genetic and epigenetic alterations, can contribute to the pathogenesis of the disease [1,2]. Oxidative stress and changes in lipid and amino acid metabolism contribute to the development of the disease. ...
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... Inactivated vaccines are derived from the complete SARS-CoV-2 virus, which has been rendered inactive through exposure to physical or chemical inactivating agents like UV rays, formalin, or formaldehyde administration [93]. Like SARS-CoV-2 recombinant protein subunit-based vaccines, inactivated whole virus vaccines must be combined with adjuvants to be effective and able to boost their immunogenicity. ...
... Current therapies only delay progression (2) , leaving lung transplant as the sole remaining option (3,4) . Risk factors include older age, male sex, smoking history (1) , other inhalant exposures (5) and in familial cases, inherited mutations (6,7) primarily in genes linked to alveolar epithelial type II (AT2) cell biology (8)(9)(10)(11)(12) , implicating AT2 cells in IPF pathogenesis (13) . Furthermore, mutations affecting telomere maintenance have been implicated in both familial and sporadic IPF (14) . ...
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... BALF collected from COPD patients represents a biomatrix rich in metabolites that can be studied for metabolomic profiles [33,34]. ...
... A direct cytopathic effect of SARS-CoV-2 on lymphocytes might be excluded in the absence of ACE-2 receptors on these cells, while enhanced apoptosis driven by TNF-α, or SARS-CoV-2 alternative mechanisms of lymphocytes invasion driven by CD147, seem more plausible [46,47]. Notably, if persistent, lymphopenia may facilitate bacterial co-infection in COVID-19, with poor prognostic outcomes [10]. During the convalescent phase, T lymphocytes mainly show a memory phenotype. ...
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... Centrilobular nodular opacities or a "crazy paving" pattern, with the typical presence of ground-glass opacities and thickening of interlobular septa, may also be observed and mimic lipoid pneumonia [1,12,27]. Other non-specific HRCT findings, such as segmental atelectasis and bronchiectasis, have been reported and have been specifically correlated with a particular SMPD1 gene mutation (p.Ala461⁄4) [2]. In one case report, focal areas of low attenuation, resembling cysts, were identified alongside ground-glass opacities, primarily in the lower lobes of the lungs [1,12]. ...
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... Metabolomics thus seems to be a useful scientific tool to enhance our understanding of disease mechanisms and to complement what genetics can already explain about lung diseases. In the future, it may not only aid in obstructive diseases but also in restrictive diseases, such as Interstitial Lung Diseases (ILD) [5,6]. ...
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... In this sub-study of the CLASS project, we aimed to evaluate the real-world performance of anti-ARS detection by comparing the results obtained in local laboratories of participating centres to those obtained from central laboratories in Italy (University and IRCCS Policlinico San Matteo Foundation Hospital of Pavia and Humanitas Clinical and Research Center, Milan) and UK (University of Bath). With multiple publications, these centres are recognised as international reference hubs for autoantibody testing (1,16,17). We also evaluated whether the performance of local testing was affected by anti-ARS antibody specificity and the technique used for detection. ...
... Other immunomodulating agents such as IVIG, Methotrexate (MTX), cyclophosphamide and cyclosporine have also been mentioned as effective medications in various studies. IVIG is used to treat myositis in IIM, but is not effective in cases of ILD [15,20]. Recently, there has been a growing tendency to use of Rituximab in the treatment of ASS specifically for patients with refractory ILD and/or myositis [19]. ...
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