Acute exacerbation of idiopathic pulmonary fibrosis after inhalation of a water repellent

The natural course of idiopathic pulmonary fibrosis (IPF) is unpredictable at the time of diagnosis. Some patients may experience episodes of acute respiratory worsening that have been termed acute exacerbations. A 58-year-old male was admitted to our Emergency Department due to progressive and intense dyspnea and dry cough after accidental inhalation of waterproof's vapor containing siloxanes. Chest high resolution computed tomography (HRCT) scan showed diffuse and bilateral ground glass attenuation, basal predominant reticular abnormalities and subpleural honeycombing. The patient didn't know that he suffered from IPF and siloxanes' inhalation triggered an acute exacerbation of his disease. Clinical course after the inhalation was aggressive and, despite steroids and cyclophosphamide therapy, the patient died 3 months after due to a respiratory failure. Inhalation of water repellents has been associated with an acute onset of respiratory symptoms and acute lung injury; usually, however, the prognosis is commonly good with a complete recovery. Our case is an example of an extremely negative reaction probably because of pre-existing and misdiagnosed IPF. Currently, no literature concerning water repellent inhalation as a trigger of acute exacerbation of IPF is available

The Role of Insulin Resistance in Fueling NAFLD Pathogenesis: From Molecular Mechanisms to Clinical Implications

Non-alcoholic fatty liver disease (NAFLD) represents a predominant hepatopathy that is rapidly becoming the most common cause of hepatocellular carcinoma worldwide. The close association with metabolic syndrome's extrahepatic components has suggested the nature of the systemic metabolic-related disorder based on the interplay between genetic, nutritional, and environmental factors, creating a complex network of yet-unclarified pathogenetic mechanisms in which the role of insulin resistance (IR) could be crucial. This review detailed the clinical and pathogenetic evidence involved in the NAFLD-IR relationship, presenting both the classic and more innovative models. In particular, we focused on the reciprocal effects of IR, oxidative stress, and systemic inflammation on insulin-sensitivity disruption in critical regions such as the hepatic and the adipose tissue, while considering the impact of genetics/epigenetics on the regulation of IR mechanisms as well as nutrients on specific insulin-related gene expression (nutrigenetics and nutrigenomics). In addition, we discussed the emerging capability of the gut microbiota to interfere with physiological signaling of the hormonal pathways responsible for maintaining metabolic homeostasis and by inducing an abnormal activation of the immune system. The translation of these novel findings into clinical practice could promote the expansion of accurate diagnostic/prognostic stratification tools and tailored pharmacological approaches

Acute exacerbation of idiopathic pulmonary fibrosis after inhalation of a water repellent

The natural course of idiopathic pulmonary fibrosis (IPF) is unpredictable at the time of diagnosis. Some patients may experience episodes of acute respiratory worsening that have been termed acute exacerbations. A 58-year-old male was admitted to our Emergency Department due to progressive and intense dyspnea and dry cough after accidental inhalation of waterproof’s vapor containing siloxanes. Chest high resolution computed tomography (HRCT) scan showed diffuse and bilateral ground glass attenuation, basal predominant reticular abnormalities and subpleural honeycombing. The patient didn’t know that he suffered from IPF and siloxanes’ inhalation triggered an acute exacerbation of his disease. Clinical course after the inhalation was aggressive and, despite steroids and cyclophosphamide therapy, the patient died 3 months after due to a respiratory failure. Inhalation of water repellents has been associated with an acute onset of respiratory symptoms and acute lung injury; usually, however, the prognosis is commonly good with a complete recovery. Our case is an example of an extremely negative reaction probably because of pre-existing and misdiagnosed IPF. Currently, no literature concerning water repellent inhalation as a trigger of acute exacerbation of IPF is available

Incidental and Underreported Pleural Plaques at Chest CT: Do Not Miss Them - Asbestos Exposure Still Exists

Pleural plaques (PPs) may be a risk factor for mortality from lung cancer in asbestos-exposed workers and are considered to be a marker of exposure. Diagnosing PPs is also important because asbestos-exposed patients should be offered a health surveillance that is mandatory in many countries. On the other hand PPs are useful for compensation purposes. In this study we aimed to evaluate the prevalence, as incidental findings, and the underreporting rate of PPs in chest CT scans (CTs) performed in a cohort of patients (1512) who underwent chest CT with a slice thickness no more than 1.25 mm. PPs were found in 76 out of 1482 patients (5.1%); in 13 out of 76 (17,1%) CTs were performed because of clinical suspicion of asbestos exposure and 5 of them (38%) were underreported by radiologist. In the remaining 63 cases (82.9%) there was no clinical suspicion of asbestos exposure at the time of CTs (incidental findings) and in 38 of these 63 patients (60.3%) PPs were underreported. Reaching a correct diagnosis of PPs requires a good knowledge of normal locoregional anatomy and rigorous technical approach in chest CT execution. However the job history of the patient should always be kept in mind

A: Deferasirox, deferiprone and desferrioxamine treatment in thalassemia major patients: cardiac iron and function comparison determined by quantitative magnetic resonance imaging. Haematologica 2011; 96

Background Oral deferiprone was suggested to be more effective than subcutaneous desferrioxamine for removing heart iron. Oral once-daily chelator deferasirox has recently been made commercially available but its long-term efficacy on cardiac iron and function has not yet been established. Our study aimed to compare the effectiveness of deferasirox, deferiprone and desferrioxamine on myocardial and liver iron concentrations and bi-ventricular function in thalassemia major patients by means of quantitative magnetic resonance imaging. Design and Methods From the first 550 thalassemia subjects enrolled in the Myocardial Iron Overload in Thalassemia network, we retrospectively selected thalassemia major patients who had been receiving one chelator alone for longer than one year. We identified three groups of patients: 24 treated with deferasirox, 42 treated with deferiprone and 89 treated with desferrioxamine. Myocardial iron concentrations were measured by T2* multislice multiecho technique. Biventricular function parameters were quantitatively evaluated by cine images. Liver iron concentrations were measured by T2* multiecho technique. Results The global heart T2* value was significantly higher in the deferiprone (34±11ms) than in the deferasirox (21±12 ms) and the desferrioxamine groups (27±11 ms) (P=0.0001). We found higher left ventricular ejection fractions in the deferiprone and the desferrioxamine versus the deferasirox group (P=0.010). Liver iron concentration, measured as T2* signal, was significantly lower in the desferrioxamine versus the deferiprone and the deferasirox group (P=0.004). Conclusions The cohort of patients treated with oral deferiprone showed less myocardial iron burden and better global systolic ventricular function compared to the patients treated with oral deferasirox or subcutaneous desferrioxamine. Key words: thalassemia, iron chelation therapy, cardiac magnetic resonance imaging. Citation: Pepe A, Meloni A, Capra M, Cianciulli P, Prossomariti L, Malaventura C, Putti MC, Lippi A, Romeo MA, Bisconte MG, Filosa A, Caruso V, Quarta A, Pitrolo L, Missere M, Midiri M, Rossi G, Positano V, Lombardi M, and Maggio A. Deferasirox, deferipron

Acute Delta Hepatitis in Italy spanning three decades (1991–2019): Evidence for the effectiveness of the hepatitis B vaccination campaign

Updated incidence data of acute Delta virus hepatitis (HDV) are lacking worldwide. Our aim was to evaluate incidence of and risk factors for acute HDV in Italy after the introduction of the compulsory vaccination against hepatitis B virus (HBV) in 1991. Data were obtained from the National Surveillance System of acute viral hepatitis (SEIEVA). Independent predictors of HDV were assessed by logistic-regression analysis. The incidence of acute HDV per 1-million population declined from 3.2 cases in 1987 to 0.04 in 2019, parallel to that of acute HBV per 100,000 from 10.0 to 0.39 cases during the same period. The median age of cases increased from 27 years in the decade 1991-1999 to 44 years in the decade 2010-2019 (p < .001). Over the same period, the male/female ratio decreased from 3.8 to 2.1, the proportion of coinfections increased from 55% to 75% (p = .003) and that of HBsAg positive acute hepatitis tested for by IgM anti-HDV linearly decreased from 50.1% to 34.1% (p < .001). People born abroad accounted for 24.6% of cases in 2004-2010 and 32.1% in 2011-2019. In the period 2010-2019, risky sexual behaviour (O.R. 4.2; 95%CI: 1.4-12.8) was the sole independent predictor of acute HDV; conversely intravenous drug use was no longer associated (O.R. 1.25; 95%CI: 0.15-10.22) with this. In conclusion, HBV vaccination was an effective measure to control acute HDV. Intravenous drug use is no longer an efficient mode of HDV spread. Testing for IgM-anti HDV is a grey area requiring alert. Acute HDV in foreigners should be monitored in the years to come

Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. The experience of the Italian SIR-SPRING registry and review of the world literature

Introduction: Systemic sclerosis (SSc) is characterized by a complex etiopathogenesis encompassing both host genetic and environmental -infectious/toxic- factors responsible for altered fibrogenesis and diffuse microangiopathy. A wide spectrum of clinical phenotypes may be observed in patients' populations from different geographical areas. We investigated the prevalence of specific clinical and serological phenotypes in patients with definite SSc enrolled at tertiary referral centres in different Italian geographical macro-areas. The observed findings were compared with those reported in the world literature.Materials and methods: The clinical features of 1538 patients (161 M, 10.5%; mean age 59.8 +/- 26.9 yrs.; mean disease duration 8.9 +/- 7.7 yrs) with definite SSc recruited in 38 tertiary referral centres of the SPRING (Systemic sclerosis Progression INvestiGation Group) registry promoted by Italian Society of Rheumatology (SIR) were obtained and clustered according to Italian geographical macroareas.Results: Patients living in Southern Italy were characterized by more severe clinical and/or serological SSc phenotypes compared to those in Northern and Central Italy; namely, they show increased percentages of diffuse cutaneous SSc, digital ulcers, sicca syndrome, muscle involvement, arthritis, cardiopulmonary symptoms, interstitial lung involvement at HRCT, as well increased prevalence of serum anti-Scl70 autoantibodies. In the same SSc population immunusppressive drugs were frequently employed. The review of the literature underlined the geographical heterogeneity of SSc phenotypes, even if the observed findings are scarcely comparable due to the variability of methodological approaches.Conclusion: The phenotypical differences among SSc patients' subgroups from Italian macro-areas might be correlated to genetic/environmental co-factors, and possibly to a not equally distributed national network of information and healthcare facilities

Silicosis in Workers Exposed to Artificial Quartz Conglomerates: Does It Differ From Chronic Simple Silicosis?

6reservedRecently, a number of reports have been published on silicosis in workers exposed to artificial quartz conglomerates containing high levels of crystalline silica particles (70-90%) used in the construction of kitchen and bathroom surfaces. Three cases of silicosis in workers exposed to artificial quartz conglomerates are reported. The diagnosis was derived from both the International Labour Office and the International Classification of HRCT for Occupational and Environmental Respiratory Diseases (ICOERD) classifications and cytological analysis of bronchoalveolar lavage fluid. In 2 cases, levels of respirable silica greatly in excess of recommended standards were measured in the workplace, and cytological analysis of bronchoalveolar lavage fluid highlighted a prevalence of lymphocytes, meeting criteria for the diagnosis of accelerated silicosis. The prevention of pneumoconiosis caused by the use of innovative materials, such as artificial conglomerates with high crystalline silica content must be addressed.mixedPaolucci, V; Romeo, R; Sisinni, Ag; Bartoli, D; Mazzei, Ma; Sartorelli, PPaolucci, Valentina; Romeo, Riccardo; Sisinni, ANTONIETTA GERARDINA; Bartoli, D; Mazzei, MARIA ANTONIETTA; Sartorelli, Pietr