Pavia, Italy
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Ambrosino N.,U.O. Pneumologia e Terapia Intensiva Respiratoria | Ambrosino N.,Volterra | Confalonieri M.,Science Pneumologia | Crescimanno G.,Cervello | And 2 more authors.
Respiratory Medicine | Year: 2013

Respiratory failure is an unavoidable event in the natural history of some neuromuscular diseases, while appearing very infrequently in others. In some cases, such as Pompe disease, respiratory failure progresses more rapidly than motor impairment, sometimes being the onset event. Home mechanical ventilation improves survival and quality of life of these patients, with a reduction in healthcare costs. Therefore, pulmonologists must improve their skills in order to play a more relevant role in the care of these patients. The aim of this statement is to provide pulmonologists with some simple information in order for them to fulfil their role of primary caregiver, enabling appropriate and rapid diagnosis and treatment. © 2013.


Bisconti M.,Pneumologia | Barbaro M.P.F.,University of Foggia | Serafni A.,Science Pneumologia | Martucci P.,U.O.C. Endoscopia Bronchiale | And 2 more authors.
Rassegna di Patologia dell'Apparato Respiratorio | Year: 2015

The main substances responsible for the diseases of the human respiratory system are marijuana, cocaine, ecstasy and some improperly used medicinal products. Drug epidemic begun in the '50s favoured by the wrong feeling that inhaled drugs were less harmful than injected ones. However they lead as well to sometimes lethal pathologic processes. While D.I.R.D. - Drug Induced Respiratory Diseases - have been thoroughly investigated in Northern Europe and USA they are barely known in Italy. Drug-related respiratory diseases include: asthma, pulmonary oedema, eosinophilic lung disease, COPD, BOOP, pneumothorax, Crack lung syndrome, empyema, granulomatosis, interstitial pulmonary fibrosis, Churg-Strauss Syndrome, ABPA, invasive aspergillosis, RADS, CAP, endocarditis, atelectasis, emphysema, pulmonary arterial hypertension, infections, bronchiectasis, haemoptysis and others.


PubMed | Royal Brompton Hospital, Witten/Herdecke University, Science Pneumologia, St Vincents University Hospital and 11 more.
Type: Journal Article | Journal: Breathe (Sheffield, England) | Year: 2015

The background and purpose of the HERMES (Harmonising Education in Respiratory Medicine for European Specialists) initiative has been discussed at length in previous articles [1-3]. This article aims to provide more detailed and specific insight into the process and methodology of the Sleep HERMES Task Force in developing a core curriculum in respiratory sleep medicine.


Luisetti M.,Centro Per La Diagnosi Del Deficit Ereditario Of Alfa 1 Antitripsina | Luisetti M.,University of Pavia | Ferrarotti I.,Centro Per La Diagnosi Del Deficit Ereditario Of Alfa 1 Antitripsina | Ferrarotti I.,University of Pavia | And 15 more authors.
COPD: Journal of Chronic Obstructive Pulmonary Disease | Year: 2015

Alpha1-antitrypsin Deficiency (AATD) is a rare hereditary disorder with an estimated prevalence of about 1/5000 individuals in Italy. Deficient patients are at a higher risk of developing lung emphysema and chronic liver disease. The low estimated prevalence of AATD prompted the establishment of a registry with the aim of learning more about the natural history and the quality of care of these patients. The Italian registry for AATD was established in 1996. In this study, genetic and clinical findings of Italian AATD patients are presented. Moreover, we also evaluated the changes in health-related quality of life (HRQoL) in patients with COPD and AAT deficiency over a three-year period, in relation to augmentation therapy. In a period spanning 18 years (1996-2014) a total of 422 adult subjects with severe AATD were enrolled, namely 258 PI∗ZZ, 74 PI∗SZ, 4 PI∗SS and 86 patients with at least one rare deficient allele. The 21.3% frequency for AATD patients with at least one deficient rare variant is the highest so far recorded in national registries of AATD. The registry data allow a detailed characterization of the natural course of the disease and the level of patient care, as well as confirm the usefulness of early AATD detection. © Informa Healthcare USA, Inc.


Campo I.,Science Pneumologia | Zorzetto M.,Science Pneumologia | Bonella F.,University of Duisburg - Essen
Expert Review of Respiratory Medicine | Year: 2015

Interstitial lung diseases (ILDs) are a heterogeneous group of >100 pulmonary disorders. ILDs are characterized by an irreversible architectural distortion and impaired gas exchange; however, there is great variability in the clinical course. ILD diagnosis requires a combination of clinical data, radiological imaging and histological findings (when a lung biopsy is required). At the same time, successful management of ILD patients strictly depends on an accurate and confident diagnosis. In this context, the detection of reliable biomarkers able to identify ILD subtypes, avoiding lung biopsy, as well as the capacity to stratify patients and predict over time the disease course, has become a primary aim for all research studies in this field. © Informa UK, Ltd.


Baldi S.,Science Pneumologia | Coni F.,Science Pneumologia | Ruffini E.,Cattedra di Chirurgia Toracica | Oliaro A.,Cattedra di Chirurgia Toracica
Rassegna di Patologia dell'Apparato Respiratorio | Year: 2011

Bronchial fistula represents a complication after pulmonary resection, frequently leading to pleural empyema. Their closure is therefore indispensable for the patient recovering, and the bronchoscopist often plays a central role in this treatment. We report 2 cases of patients undergoing pneumonectomy complicated by bronchial stump fistula, very different from each other, who have been dealt with a new device called amplatzer, used by cardiologists to close the inter-septal defects. To our knowledge only 3 cases have been reported in literature. If the first case, characterized by a longstanding fistula, was successful, not so for the second one, which was characterized by recent onset fistula after pneumonectomy in a patient undergoing lung re-transplantation.


Baraniuk J.N.,Georgetown University | Casado B.,Georgetown University | Pannell L.K.,University of South Alabama | McGarvey P.B.,Georgetown University | And 3 more authors.
International Journal of COPD | Year: 2015

Rationale: Subtypes of cigarette smoke-induced disease affect different lung structures and may have distinct pathophysiological mechanisms. Objective: To determine if proteomic classification of the cellular and vascular origins of sputum proteins can characterize these mechanisms and phenotypes. Subjects and methods: Individual sputum specimens from lifelong nonsmokers (n=7) and smokers with normal lung function (n=13), mucous hypersecretion with normal lung function (n=11), obstructed airflow without emphysema (n=15), and obstruction plus emphysema (n=10) were assessed with mass spectrometry. Data reduction, logarithmic transformation of spectral counts, and Cytoscape network-interaction analysis were performed. The original 203 proteins were reduced to the most informative 50. Sources were secretory dimeric IgA, submucosal gland serous and mucous cells, goblet and other epithelial cells, and vascular permeability. Results: Epithelial proteins discriminated nonsmokers from smokers. Mucin 5AC was elevated in healthy smokers and chronic bronchitis, suggesting a continuum with the severity of hypersecretion determined by mechanisms of goblet-cell hyperplasia. Obstructed airflow was correlated with glandular proteins and lower levels of Ig joining chain compared to other groups. Emphysema subjects’ sputum was unique, with high plasma proteins and components of neutrophil extracellular traps, such as histones and defensins. In contrast, defensins were correlated with epithelial proteins in all other groups. Protein-network interactions were unique to each group. Conclusion: The proteomes were interpreted as complex “biosignatures” that suggest distinct pathophysiological mechanisms for mucin 5AC hypersecretion, airflow obstruction, and inflammatory emphysema phenotypes. Proteomic phenotyping may improve genotyping studies by selecting more homogeneous study groups. Each phenotype may require its own mechanistically based diagnostic, risk-assessment, drug- and other treatment algorithms. © 2015 Baraniuk et al.


Canessa P.A.,Science Pneumologia | Ferro P.,Anatomia patologica | Manta C.,Science Pneumologia | Sivori M.,Science Pneumologia | And 3 more authors.
Medical Oncology | Year: 2013

The aim of this study based on the third phase of the architecture of diagnostic research was to assess the sensitivity and specificity of soluble mesothelin-related peptide (SMRP) in pleural exudative effusions (PE) compared to the histology obtained by medical thoracoscopy as the diagnostic gold standard examination. We assessed 104 consecutive thoracoscopies. SMRP concentrations were obtained using an ELISA test. We had 34 mesotheliomas (25 epithelioid and 9 sarcomatoid), 35 pleural metastases, and 35 benign diseases. PE-SMRP were significantly higher in patients with epitheliomorphic mesothelioma (mean ± SD 46.55 ± 44.29 nM) than in patients with sarcomatoid mesothelioma (16.11 ± 25.02 nM; p = 0.061), pleural metastasis (7.52 ± 10.77 nM; p < 0.0001), or benign diseases (5.82 ± 8.86 nM; p < 0.0001). Using ROC curve analysis, PE-SMRP offered an AUC of 0.767 in its ability to differentiate between patients with mesothelioma and all other diagnoses. The diagnostic sensitivity and specificity of PE-SMRP for distinguishing mesothelioma from all other causes of pleural effusion, at a cut-off value of 19.6 nM, were 58.8 and 97.1 %, respectively. PE-SMRP higher than the assumed cut-off of 19.6 nM were observed in 18/25 (72.0 %) epitheliomorphic mesotheliomas, 2/9 (22.2 %) sarcomatoid mesotheliomas, 5/35 (14.3 %) pleural metastases, and 1/35 (2.9 %) benign diseases. We conclude that PE-SMRP adds some clinical information in the work-up of patients with a PE of unknown origin: (1) thoracoscopy should always be done in patients with a positive mesothelin; (2) a negative mesothelin does not exclude a malignant disease. © Springer Science+Business Media New York 2013.


Canessa P.A.,Science Pneumologia | Manta C.,Science Pneumologia | Sivori M.,Science Pneumologia | Intersimone D.,Anatomia Patologica | Fedeli F.,Anatomia Patologica
Rassegna di Patologia dell'Apparato Respiratorio | Year: 2015

Background. Pleural effusions are frequently the presenting symptom of neoplastic disease. The cytological examination of pleural effusion is recognized as being the most commonly used investigation in the diagnosis of malignancy. Objectives. The aim of this study was to assess the sensitivity and specificity of cytology in pleural exudate effusions compared to the histology obtained by medical thoracoscopy as the diagnostic gold standard examination. Methods. We assessed 256 consecutive thoracoscopies performed between 2006 and 2010 in the Pneumology ward of the Sarzana Hospital (Italy). Pleural disease was diagnosed based on histological criteria. Results. We had 80 mesotheliomas, 52 pleural metastasis and 124 non malignant pleural diseases. Cytologic examination permitted the diagnosis of 35 mesotheliomas (28 epithelioid, 2 sarcomatoid, 2 desmoplastic, 3 biphasic), 37 pleural metastasis (21 lung, 8 breast, 1 lymphoma, 3 ovary, 1 stomach, 1 liver, 2 uterus). Cytology remained negative in 45 mesotheliomas (28 epithelioid, 11 sarcomatoid, 2 desmoplastic, 4 biphasic) and in 15 pleural metastasis (6 lung, 4 breast, 3 lymphoma, 1 sarcoma, 1 thyme). The sensitivity of cytology was 53.8% and the specificity was 97.6%; the sensitivity of pleural metastasis was greater than the sensitivity of malignant mesothelioma (71.2% vs 43.7%). Conclusions. We conclude that sensitivity of first cytology samples in pleural effusion remains about 50% in the immunocytochemical era and that it is too low to avoid a diagnostic thoracoscopy. A negative cytologic examination of pleural effusion does not exclude a diagnostic thoracoscopy; a positive cytology for metastasis could exclude diagnostic thoracoscopy, even though thoracoscopy might be performed for talc poudrage. A positive cytology for mesothelioma requires confirmation by histology obtained by thoracoscopy.


PubMed | Science Pneumologia
Type: Journal Article | Journal: Therapeutic advances in respiratory disease | Year: 2010

Pulmonary alveolar proteinosis is a rare condition characterized by the accumulation of lipoproteinaceous material within the airspaces, resulting in impaired gas transfer, and clinical manifestations ranging from asymptomatic to severe respiratory failure. To the best of the authors knowledge, there are only a few conditions whose natural history has been so dramatically changed by the influence of advances in basic science, clinical medicine, and translational research in therapeutic approaches. Whole-lung lavage is the current standard of care and it plays a critical role as a modifier factor of the natural history of proteinosis. That notwithstanding, the identification of autoantibodies neutralizing granulocyte-macrophage colony-stimulating factor in serum and lung of patients affected by the form of proteinosis previously referred to as idiopathic, has opened the way to novel therapeutic options, such as supplementation of exogenous granulocyte-macrophage colony-stimulating factor, or strategies aimed at reducing the levels of the autoantibodies. The aim of this paper is to provide an updated review of the current therapeutic approach to proteinosis.

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