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Background: The aim of the study was to evaluate the adequacy of diagnosis and management of respiratory failure (RF) in COPD., Methods: Retrospective analysis of the hospital discharge forms of COPD patients hospitalized for RF from January 2007 to June 2008. Using the clinical audit tool, the primary end point was the accuracy of RF diagnosis. The secondary end points were mortality, re-hospitalization rate, length of hospital stay, accuracy of long-term oxygen therapy (LTOT) prescription, and agreement of the treatments with the Global Initiative for Chronic Obstructive Lung Disease (GOLD) 2008 guidelines. Statistical analysis used Pearson and Spearman correlation test and the Cohen kappa for degree of agreement. Differences in demographics and clinical parameters were analyzed with the chi-square test, t test, or the Fisher test, as appropriate., Results: We studied 130 patients, 81 males (62%), mean ± SD age 76.6 ± 9.1 years. Arterial blood gas analysis (ABG) was performed in 118 patients (90.8%), and in 77 (81%) a P(aO(2)) < 60 mm Hg was found at admission. Of these, 42 cases (54.5%) had no diagnosis of RF, despite a P(aO(2)) < 60 mm Hg. In 18 (19%) P(aO(2)) was ≥ 60 mm Hg; of these, 6 cases (33.3%) received an incorrect RF diagnosis. At discharge 8.1% of patients did not receive a diagnosis of RF, despite a compatible ABG. The highest mortality was found in the medicine departments (14.7%). The re-hospitalization rate at 90 days was 19.5%. Adherence of the treatment to the GOLD guidelines during hospitalization was confirmed in 75.8% of patients. In 41.1% of cases LTOT was prescribed at discharge; in 24 out of 27 cases P(aO(2)) values were < 55 mm Hg., Conclusions: Agreement between diagnosis of RF and ABG values was found to be insufficient in about half the cases. Among secondary end points, adherence of the treatment to guidelines and LTOT prescription were, however, found to be good. Data showed significant inaccuracies in the management of RF at our institution.

Objectives: The aim of this study was to assess the stability of the effectiveness of omalizumab as add-on treatment in 11 patients with severe persistent allergic asthma followed for 4 years. Secondary outcomes were safety and economic impact, in terms of use of healthcare resources., Methods: This retrospective study was designed to analyse a series of patients with severe allergic asthma treated with omalizumab. Patients were initially enrolled as part of the CIGE025A2425 international multicentre clinical trial. At the end (week 32), 11 responsive patients went on to complete the study and continued omalizumab treatment until June 2010. The monitoring visits coincided with the timescales planned for administering the drug and for the follow up. To estimate the economic impact, the PRE-POST treatment comparison was obtained by comparing the annual pretreatment costs with an annual average of the 4-year posttreatment period costs, Results: After 4 years, 81.8% of patients showed a good/excellent Global Evaluation of Treatment Effectiveness scale score and 81.2% showed an excellent increase (>1.5) in the Asthma Quality of Life Questionnaire score. The average forced expiratory volume in one second (FEV(1)) at 4 years was 75.3% compared with the predicted normal value for each patient, with a net increase (p = 0.009) compared with baseline FEV(1) values (58.6%). The frequency of serious exacerbations dropped by 94.7% compared with the pretreatment period, while mild-moderate exacerbations fell by 41.8%. A reduction in costs was observed for hospital admissions (97.3%), visits to emergency department (ED) (97.5%) and mild-moderate exacerbations (84%). The average cost reduction of concomitant drugs remained at 36%., Conclusions: This study confirms the effectiveness and reliability of omalizumab over the long term, while providing an excellent safety profile. The additional cost due the use of omalizumab was offset by the medium- and long-term savings associated with the reduction in hospital admissions and access to ED.

Sarcoidosis is a multi-systemic inflammatory disease of unknown origin characterized by the presence of noncaseating epitheloid cell granulomas in multiple organs. Diagnosis is made on the basis of a compatible clinical-radiological scenario and the histological demonstration of the typical granulomas in the affected tissues. Interferons are immuno-modulators that have been used in a wide range of diseases, including hepatitis C virus infection, multiple sclerosis, and multiple myeloma and other types of tumours, including leukemia, lymphomas, Kaposi's sarcoma, and melanoma. Interferon-alpha-induced sarcoidosis has been reported repeatedly and there are two reports in the literature of cases of pulmonary sarcoidosis treated with interferon-1b therapy: one for advanced renal cell carcinoma and the other for multiple myeloma. A 35-year-old man on chronic immune-modulant Interferon-1b-based therapy for multiple sclerosis presented to the Neurology Unit with mild dyspnoea, dry cough, and transient pain to right upper abdomen. Lungs, spleen, liver, and almost all lymphnode stations of abdomen and mediastinum were clearly involved on ultrasound examination, chest X-ray, and computed tomography. A transbronchial biopsy showed non-caseating granuloma on histopathologic evaluation of the lungs. To the best of our knowledge, this is the first report of a chronic multisystemic sarcoidosis that was associated with interferon-beta treatment.

Introduction: Legionella pneumonia can appear with different levels of severity and it can often present with complications such as acute respiratory distress syndrome., Case Presentation: We report the case of a 44-year-old Caucasian man with Legionella pneumonia with successive development of severe acute respiratory distress syndrome. During his stay in intensive care the clinical and radiological situation of the previously observed acute respiratory distress syndrome unexpectedly worsened due to acute pulmonary eosinophilic infiltrate of iatrogenic origin., Conclusion: Levofloxacin treatment caused the occurrence of acute eosinophilic infiltrate. Diagnosis was possible following bronchoscopic examination using bronchoaspirate and transbronchial biopsy.

Unlabelled: Omalizumab is an anti-IgE monoclonal antibody available since 2006 for the treatment of GINA step 4 asthma. We studied a 41-year old male who has been suffering from severe steroid-resistant asthma with severe co-morbidity and treated with Omalizumab. He was found to be non-responder to the treatment until the 48th week, starting from which we began to see a distinct improvement in the symptoms and all the correlated parameters, in addition to remission of the co-existent allergy to milk., Conclusions: we wish to point out the late response to Omalizumab, which occurred way beyond the times envisaged in literature. It seems possible that some patients are late responders to the drug.

Asthma is thought to be a Th2 disease while sarcoidosis is considered a Th1 granulomatous disorder. Organising pneumonia is a histologic pattern of lung injury. When it has no recognisable cause it is defined as cryptogenic organising pneumonia. We herein report the case of a patient with recurrent and steroid sensitive organising pneumonia associated with chronic sarcoidosis in an atopic, moderate persistent asthmatic patient. Each disease has been documented with transbronchial biopsies and recurrence of organising pneumonia was suggested by clinical features and by follow up HRCT which shows distinctive signs even in associated disease. Steroids are the mainstay of therapy for these disorders and especially for the consolidated processes typical of organising pneumonia but prognostic indices for relapse and progression are lacking.

We describe the case of a 33-year-old woman who presented with a pattern of diffuse micronodular opacities with centrolobular distribution at high resolution chest tomography (HRCT) performed after exposure to the smoke of a home fire. An abdominal CT scan showed the presence of 3 rounded hypodense lesions in the spleen parenchyma. A bronchoalveolar lavage (BAL) was performed, showing the presence of lipid laden cells in the aspirated fluid. A video-assisted thoracoscopic biopsy confirmed the presence of the foamy cells filling the alveolar spaces and showed a preserved background pulmonary architecture; the bronchiolar epithelium presented a cytoplasm with a particularly clear colour. Foam cells were present in the bone marrow aspirate too, that contained also sea-blue histiocytes. These data induced us to consider the diagnosis of a metabolic storage disorder, in the first instance Niemann-Pick type B, that was confirmed by low acid lysosomal sphingomyelinase activity on cultured fibroblasts: 5.22 nmol/mg prot/h (n.v. 92 +/- 18.2). Lung involvement is an important complication of Niemann-Pick disease, presenting with the clinical and radiological features of a diffuse lung disease.

Background: Occupational exposure to asbestos may cause pleural and lung disorders and, less frequently, diseases of the peritoneum and pericardium. An exceedingly small number of cases of benign pericardial effusion have been described so far in the medical literature., Objectives: To report a rare case of haemorrhagic pericardial effusion caused by occupational asbestos exposure in a patient with pre-existent aortic regurgitation, bilateral pleural plaques and no signs of interstitial lung involvement due to asbestosis., Methods: A thorough clinical and instrumental evaluation (laboratory tests, tuberculin skin test, chest X-rays, transthoracic and transesophageal echocardiography, contrast coronary and aortic angiography, a histological examination of pericardial and pleural surgical specimens) was performed to examine all the known causes of pericardial effusion., Results: The tests performed did not demonstrate any specific cause of pericardial effusion. Surgical assessment three months later, during an aortic valve replacement, showed no signs of aortic dissection or intraparietal hematoma. A nine-year follow up did not reveal any reoccurrence of pericardial effusion., Conclusions: Asbestos related pericardial effusion is rarely described in the medical literature but must be considered in patients with previous occupational asbestos exposure. There are no specific clinical or pathological aspects indicative of this etiology and the diagnosis remains one of exclusion. A thorough occupational history should be obtained in patients with pericardial effusion of unknown etiology.

Objective: To report about 6 new patients with round atelectasis of the lung, 1 of them professionally exposed to asbestos and another to silicates., Results: The patients, 5 males and 1 female, presented with a peripheral, rounded pulmonary opacity, simulating a neoplasm. The examination of the surgical specimen revealed a pleuritis, with multiple pleural folding: the underlying lung parenchima was compressed, but otherwise unremarkable., Conclusions: Round atelectasis is relatively unusual for the pathologist. However, the correct diagnosis is potentially important, because the lesion can be the sign of a significant asbestos exposure.

We report on 50-year-old woman without tuberous sclerosis, presenting with recurrent spontaneous pneumothorax. A CT-scan of the chest showed multiple, bilateral, thin-walled cysts, consistent with pulmonary lymphangioleiomyomatosis. A videothoracoscopic lung biopsy confirmed the diagnosis of lymphangioleiomyomatosis, but revealed also a micrometastasis from an occult papillary carcinoma of the thyroid gland. The main histologic differential diagnosis and the possible correlation between lymphangioleiomyomatosis and thyroid diseases are briefly discussed.

OBJECTIVE: To report a syndrome that is uncommon in childhood and call pediatricianśattention to the tubular diseases - just like Bartters syndrome - in differential diagnosis of failure to thrive and other diseases that can be usually found in children.METHODS: Two patients are presented. The first, a 3 years and 2 months old boy who was submitted for investigation of a failure to thrive detected when he was 9 months old. The second patient, a 3 months old girl, was admitted to the Intensive Care Unit due to severe electrolyte disturbances. She was supposed to have a pyloric hypertrophic stenosis.RESULTS: Both patients had failure to thrive, hypocloremic alkalosis, hypokalemia, and hypercalciuria. The first had a positive obstetric history for polihydramnios that is frequently found in the neonatal form of this syndrome. Treatment was done by blood potassium correction, together with indometacin and spironolactone administration. These drugs where well tolerated by the patients who have improved their growth rhythm only a short time after electrolytic disturbances had been corrected.CONCLUSIONS: The Bartter's syndrome is a tubular disease that is unusual in childhood. It must be considered as a possible cause of failure to thrive. The neonatal form is rare and can produce severe hydro-electrolytic disturbances, increasing the difficulties for diagnosis. The treatment is well tolerated, even by small children, and must begin early to reduce the troubles to thrive.

In normal individuals the 5/9 monoclonal antibody (5/9 MAb) recognizes a T-cell fraction that includes all T lymphocytes with inducer activities. Here, circulating 5/9+ and 5/9- T lymphocytes were isolated from tuberculim skin-positive subjects and the proliferative response induced by PPD was investigated. The results show that the total PPD-induced lymphocyte DNA synthesis is confined to the 5/9+ T cell fraction. PPD was unable to induce a direct or indirect (through the 5/9+ cells) proliferation of T8+ cells. Whether 5/9 antigen, expressed on the PPD responsive T-cell subset, is involved in PPD-induced cell proliferation, was also analyzed. No significant effect was observed adding 5/9 MAb, whereas complete inhibition of antigen-induced blastogenesis was observed upon addition of a MAb (D1.12) directed to common determinants of Ia antigens. The supernatant fluids of 5/9+ and 5/9- cells stimulated with PPD were studied for the presence of IL-2 and IFN-gamma. Production of both these lymphokines was detected in the 5/9+ T-cell supernatants, whereas neither IL-2 or IFN-gamma were found in supernatants of 5/9- cells. In addition, preliminary experiments suggested that only the supernatants of PPD-stimulated 5/9+ T cells were able to activate a macrophage cell line, inducing a remarkable release of hydrogen peroxide. These results indicate that the lymphocyte responsiveness to PPD is confined in a small T cell fraction, expressing the 5/9 antigen; only these T cells are able to release soluble factors (i.e., IL-2 and IFN-gamma); those or other soluble factor(s) produced in PPD-stimulated cultures seem able to activate the macrophages.

Four cases of disseminated tuberculosis with prevailing spleen involvement and lack of tuberculin reactivity are described. The atypical clinical picture justified, at the beginning of the disease, the suspect of a lymphoreticular disorder (malignant lymphoma in 3 cases) or of a pulmonary hemosiderosis (in 1 case). The splenectomy and the following anti-tubercular chemotherapy were fully successful in all 4 patients and the skin reactivity was restored. The Authors discuss the pathogenesis of the observed features and the differential diagnosis of the cases of tuberculosis with only extrapulmonary involvement. These cases represent today about 1/6 of the patients with postprimary tuberculosis.