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During the coronavirus disease 2019 (COVID-19) pandemic, noninvasive respiratory support has played a central role in managing patients affected by moderate-to-severe acute hypoxemic respiratory failure, despite inadequate scientific evidence to support its usage. High-flow nasal cannula (HFNC) treatment has gained tremendous popularity because of its effectiveness in delivering a high fraction of humidified oxygen, which improves ventilatory efficiency and the respiratory pattern, as well as its reported high tolerability, ease of use, and application outside of ICUs. Nevertheless, the risk of infection transmission to health-care workers has raised some concerns about its use in the first wave of the pandemic outbreak, with controversial recommendations provided by different scientific societies. This narrative review provides an overview of the recent evidence on the physiologic rationale, risks, and benefits of using HFNC instead of conventional oxygen therapy and other types of noninvasive respiratory support devices, such as continuous positive airway pressure and noninvasive ventilation in patients affected by COVID-19 pneumonia with associated acute hypoxemic respiratory failure. It also summarizes the available evidence with regard to the clinical use of HFNC during the current pandemic and its reported outcomes, and highlights the risks of bioaerosol dispersion associated with HFNC use.

The COVID‐19 pandemic has had a strong impact on healthcare workers (HCWs), affecting their physical and mental health. In Italy, HCWs have been among the first exposed to unprecedented pressure, dealing with large numbers of infections during the first pandemic wave. However, the severe psychological consequences on HCWs find little evidence in the literature, especially in terms of comparison to the status quo ante pandemic. The aim of this study was to provide an assessment of the mental health burden in a cohort of Italian HCWs during the COVID‐19 pandemic, comparing their condition with that before the emergency, to direct the promotion of mental well‐being among HCWs worldwide. In this retrospective study, we included physicians, physical therapists, and nurses working in the Respiratory Intensive Care Unit, Neurology Unit, and Rehabilitation Unit from a Southern Italy University Hospital. All study participants underwent a battery of psychological tests, aimed at verifying their state of mental health during the COVID‐19 emergency and before it. Depressive, anxiety, and burnout symptoms were assessed using the following questionnaires: Maslach Burnout Inventory, Patient Health Questionnaire‐9 (PHQ‐9), and General Anxiety Disorder‐7. Depressive, anxiety, and burnout clinical relevance symptoms were present in HCWs during the COVID‐19 pandemic more than those before the emergency. Fifty percent of the HCWs obtained a score clinically significant during the emergency. Moreover, a depersonalization factor showed a statistically significant increase in average scores (p, Highlights Depressive, anxiety, and burnout were present in Italian healthcare workers during COVID‐19 pandemic.A depersonalization factor showed to be significant increased.The 47.1% of the operators reported a depressive state.The female gender conferred greater risks for depression.

Over the past three decades, the use of noninvasive ventilation or “NIV” to assuage symptoms of hypoventilation for patients with early onset or mild ventilatory pump failure has been extended to up to the use of continuous noninvasive ventilatory support (CNVS) at full ventilatory support settings as a definitive alternative to tracheostomy mechanical ventilation. NVS, along with mechanical insufflation-exsufflation, now provides a noninvasive option for the management of both chronic and acute respiratory failure for these patients. The most common diagnoses for which these methods are useful include chest wall deformities, neuromuscular diseases, morbid obesity, high level spinal cord injury and idiopathic, primary or secondary disorders of the ventilatory control. Thus, NVS is being used in diverse settings: critical care units, medical wards, at home, and in extended care. The aim of this review is to examine the techniques used for daytime support.

Background: Noninvasive mechanical ventilation (NIV) is an effective treatment in patients with neuromuscular diseases (NMD) to improve symptoms, quality of life, and survival. Summary: NIV should be used early in the course of respiratory muscle involvement in NMD patients and its requirements may increase over time. Therefore, training on technical equipment at home and advice on problem solving are warranted. Remote monitoring of ventilator parameters using built-in ventilator software is recommended. Telemedicine may be helpful in reducing hospital admissions. Anticipatory planning and palliative care should be carried out to lessen the burden of care, to maintain or withdraw from NIV, and to guarantee the most respectful management in the last days of NMD patients’ life. Key Message: Long-term NIV is effective but challenging in NMD patients. Efforts should be made by health care providers in arranging a planned transition to home and end-of-life discussions for ventilator-assisted individuals and their families.

Noninvasive ventilation has become the initial treatment for symptomatic ventilatory pump failure but, when used at ventilatory support settings, can be an alternative to tracheostomy mechanical ventilation. The intermittent abdominal pressure ventilator, a corset with an internal air sack inflated by a ventilator, allows to increase tidal volumes through the raising of the diaphragm and its consequent passive descent. It has been used for daytime support for more than 70 yrs, but its knowledge among clinicians is scarce. A narrative review was performed by searching PubMed, Medline, and the Cochrane Database of Systematic Reviews using the terms "IAPV" or "pneumobelt." One hundred forty patients were cited using the intermittent abdominal pressure ventilator from 1946 until it went off the market in the 1970s, although many continued to use it. There was only one publication on its use from 2003 to 2017, but three publications from 2017 through 2021. It has been used for full diurnal ventilatory support by some patients for more than 50 yrs and has even been used throughout the labor of a mother with no ventilator-free breathing ability. The intermittent abdominal pressure ventilator is a ventilatory support alternative for patients with ventilatory pump failure. It can be effective and well tolerated and maintain quality of life without facial interfaces. More widespread application is warranted.

Background and aim Little is known about the current use of long-term home noninvasive ventilation (LTHNIV) in restrictive thoracic diseases, including chest wall disorders and neuromuscular disorders (NMD). This study aimed to capture the pattern of LTHNIV in patients with restrictive thoracic diseases via a web-based international survey. Methods The survey involved European Respiratory Society (ERS) Assembly 2.02 (NIV-dedicated group), from October to December 2019. Results 166 (22.2%) out of 748 members from 41 countries responded; 80% were physicians, of whom 43% worked in a respiratory intermediate intensive care unit. The ratio of NMD to chest wall disorders was 5:1, with amyotrophic lateral sclerosis the most frequent indication within NMD (78%). The main reason to initiate LTHNIV was diurnal hypercapnia (71%). Quality of life/sleep was the most important goal to achieve. In 25% of cases, clinicians based their choice of the ventilator on patients’ feedback. Among NIV modes, spontaneous-timed pressure support ventilation (ST-PSV) was the most frequently prescribed for day- and night-time. Mouthpieces were the preferred daytime NIV interface, whereas oro-nasal masks the first choice overnight. Heated humidification was frequently added to LTHNIV (72%). Single-limb circuits with intentional leaks (79%) were the most frequently prescribed. Follow-up was most often provided in an outpatient setting. Conclusions This ERS survey illustrates physicians’ practices of LTHNIV in patients with restrictive thoracic diseases. NMD and, specifically, amyotrophic lateral sclerosis were the main indications for LTHNIV. NIV was started mostly because of diurnal hypoventilation with a primary goal of patient-centred benefits. Bi-level ST-PSV and oro-nasal masks were more likely to be chosen for providing NIV. LTHNIV efficacy was assessed mainly in an outpatient setting., For the first time since EUROVENT, the REINVENT survey of @EuroRespSoc members expert in noninvasive (NIV) ventilatory support illustrates physicians’ practices of long-term home NIV in restrictive thoracic disorder patients https://bit.ly/2MbC3YI

The management of respiratory diseases requires various levels of care: multidisciplinary teams, educational and behavioural interventions, self-management and home-based technical support are vital to ensure adequate care management. However, it is often difficult to access these networks due to fragmentation of patient care and treatment burden. Care coordination aims to ensure patients have a central role and that there is continuity of care among various levels and professionals involved. Moreover, the coronavirus disease pandemic has caused strain on the global healthcare system, with care coordination becoming increasingly important in increasing the resilience of health systems, supporting healthcare professionals and ensuring the right treatment and adequate level of care for these patients., Care coordination is fundamental in treating respiratory disease. Although it involves considerable effort it ensures professionals to work together to reduce the treatment burden for respiratory patients. https://bit.ly/2Qrrpz0

The article recently published by Hjembaek-Brandt et al. focused on humidification during the collection of Exhaled Breath Condensate (EBC) in invasively mechanically ventilated (IMV) patients [1]. Indeed, in the current guidelines it is still unclear the standard humidification setting and technique that should be used [2, 3]. In some studies the EBC has been performed with active humidification (AH) on [4] while others removed it before collection [5]. Hjembaek-brandt demonstrated that diverse settings of AH may remarkably vary the amount of sample collected. The authors concluded that the EBC collection should be performed in no humidification (H-), turning off the humidifier 10 min before starting the exam [1].

Background Patients hospitalized for COVID-19-related pneumonia often need several degrees of ventilatory support, which are performed between Respiratory Intermediate Care Units (RICUs) and Intensive Care Units (ICUs), and which depend on the severity of acute respiratory distress syndrome. There is no firm consensus on transfer predictors from the RICU to the ICU. Methods In this retrospective observational single center study, we evaluated 96 COVID-19 patients referred to the RICU for acute respiratory failure (ARF) according to their transferal to the ICU or their stay at the RICU. We compared demographic data, baseline laboratory profile, and final clinical outcomes to identify early risk factors for transfer. Results The best predictors for transfer to the ICU were elevated C-reactive protein and lymphopenia. The mortality rate was lower in the RICU than in the ICU, where transferred patients who died were mostly younger men and with less comorbidities than those in the RICU. Conclusions Few inflammatory markers can predict the need for transfer from the RICU to the ICU. Due to the ongoing COVID-19 pandemic, we urge better clinical stratification by early and meaningful profiles in patients admitted to the RICU who are at risk of transferal to the ICU.

The recent Coronavirus disease 19 (COVID-19) pandemic, first in China and then also in Italy, brought to the attention the problem of the saturation of Intensive Care Units (ICUs). Almost all previous reports showed that in ICU less than half of patients were treated with invasive mechanical ventilation (IMV) and the rest of them with non-invasive respiratory support. This highlighted the role of respiratory intensive care units (RICUs), where patients with moderate to severe respiratory failure can be treated with non-invasive respiratory support, avoiding ICU admission. In this report, we describe baseline characteristics and clinical outcomes of 97 patients with moderate to severe respiratory failure due to COVID-19 admitted to the RICU of the Policlinico of Bari from March 11th to May 31st 2020. In our population, most of the subjects were male (72%), non-smokers (76%), with a mean age of 69.65±14 years. Ninety-one percent of patients presented at least one comorbidity and 60% had more than two comorbidities. At admission, 40% of patients showed PaO2/FiO2 ratio between 100 and 200 and 17% showed Pa02/FiO2 ratio

Background: Little is known about the current use of long term home NIV (LTHNIV) in restrictive diseases (RD) including Chest wall and Neuromuscular disorders (CWD, NMD). Aims: This survey aimed to assess LTHNIV use in RD within the ERS Respiratory Intensive care Assembly and to compare its findings with Eurovent Methods: A survey was performed over a 3-month-period. Information on prescribers, diseases involved, NIV-modes, circuits, interfaces, humidification and follow-up were collected. Results: Response rate: 165 responders (20,5%) from 41 countries; 80% were physicians, and they were working in 63% in respiratory ward/intensive care unit (RICU). Ratio of CWD vs NMD on LTHNIV was 1:5. Amyotrophic lateral sclerosis (ALS) was the n°1 indication for LTHNIV (76% of RD), n°2 Muscular dystrophy (11%). Diurnal hypercapnia was the first reason to start LTHNIV (71%). Quality of life/sleep was the best achieved benefit. Among NIV-modes PSV S/T,PSV+VT, PCV were the 3 most used. Daytime-NIV was mainly provided via 1.mouthpiece; 2.nasal pillows; 3.nasal mask. Night-time NIV interfaces were: 1.oro-nasal; 2.full-face; 3.nasal. Heated humidification was n°1 prescribed humidification (72%). Tubings/valves used were: single with intentional leaks (79%), true expiration valve (13%), double (8%). Follow up was most often provided in an outpatient setting on average every three-month period. Conclusions: This survey highlighted specifics of LTHNIV in RD, showing that patients with NMD affected by ALS are the biggest group. In comparison to Eurovent, LTHNIV has increased in NMD while it has decreased in CWD. Bi-level PSV-ST,PSV+VT, PCV are the most used modes and oro-nasal mask is the preferred interface.

Background: Impulse Oscillometry (IOS) is a non-invasive test that evaluates the respiratory system resistance, reactance of large and small airways. No available data are available in patients with Amyotrophic Lateral Sclerosis (Als). Aims: The study aimed to assess the reliability of IOS test, and to compare its values with anthropometric parameters and respiratory test (spirometry and sniff inspiratory nasal pressure, SNIP). Methods: Thirty-eight consecutive ALS patients (M/F=18/20) underwent routine respiratory function test, including spirometry, SNIP, and IOS. Snip values were divided into 2 groups above and below 60cmH20. Lung reactance of small airways was defined by X5 values. T student analysis for unpaired samples and ANOVA analysis were performed. Interclass correlations (ICCs) were calculated to evaluate the reliability of IOS compared to spirometry. Results: Individual and average ICC for X5 (0.89; 0.97) showed good reliability compared with individual and average ICC for FVC (0.91; 0.97). Als patients with lower X5 values had lower BMI mean values (p 60cmH20 (p Conclusions: In Als patients, lower X5 values are related to lower BMI, and SNIP values

IntroductionThere are no clear guidelines as yet for the selection of patients affected by COVID-19 who can be treated in intermediate RICU, neither shared criteria for their intubation and transfer in ICU.In the present study we described the clinical course and risk factors for transfer to ICU and mortality of SARS-Cov-2 positive patients affected by acute respiratory failure, hospitalized in a Respiratory Intermediate Care Unit in the south of Italy.MethodsIn this retrospective, observational single centre study we evaluated 96 laboratory confirmed COVID-19 patients affected by acute respiratory failure (ARF). We compared demographic data, laboratory data and clinical outcomes between deceased and survived patients, aiming to identify risk factors for transfer to ICU and mortality, and possible gender-related differences.ResultsOf 96 patients, 51 (53.1%) survived and 45 (46.9 %) died. Among those who died, 23 (51.1%) deceased in RICU. Twenty-nine (30.2%) were transferred to ICU, of whom 22 (75.9%) died in ICU. Patients affected by COPD have a higher mortality compared to patients without this comorbidity (p = 0.002). Lower baseline P/F ratio (p = 0,014) and neurologic comorbidities (p = 0,008) emerged as risk factors for death.Male were younger than female patients (66 vs 80 y.o.; p = 0.042). In female patients, lower peripheral blood lymphocyte count (p = 0.007) is a risk factor for death, characteristic gender-related in our sample.Female sex was a protective parameter against transfer to ICU (p = 0,036) and P/F ratio wasn’t a significant predictor of transfer to ICU (p = 0,227).Only higher baseline CRP (p = 0,034) has shown a predictive role for transfer to ICU in our sample. Patients deceased after a transfer to ICU had younger age (p = 0,000), lower median comorbidity number (p = 0,000), lower D-dimer (p = 0,029) and lower prevalence of female sex (p = 0,029).DiscussionMortality in our study was similar to that found in other studies involving patients in non-invasive ventilation. In our study older age and comorbidities play as predictors of death in COVID-19 patients. COPD, despite presenting low prevalence, is a risk factor for death, both in men and women. In female patients chronic ischemic heart disease and congestive heart failure are death predictors. High CRP and lymphopenia, linked to inflammatory status, are predictors of transfer to ICU. Patients transferred to ICU higher mortality than the others, and patients who die in ICU are mostly men, younger and have less comorbidities. Baseline P/F ratio is not a good predictor of transfer to ICU, while in our sample is a sensible predictor of death. More studies need to be performed on COVID-19 patients, in the urgency of COVID-19 pandemic persistence.

Objective: Sniff nasal inspiratory pressure (SNIP), a useful tool for the assessment of diaphragm function in patients with Amyotrophic Lateral Sclerosis (ALS), is usually performed together with l...

We describe the clinical deterioration of a 26-yr-old man with Duchenne muscular dystrophy on oral daily high-dose deflazacort. Although this daily regimen was targeted to benefit ambulation and respiration, it resulted in premature death with lethal sequelae from liver failure, decubiti, diabetes mellitus, and morbid obesity. This case illustrates the need for further research weighing risk versus benefit of daily glucocorticoid therapy, specifically deflazacort, in Duchenne muscular dystrophy patients. Thus, curtailment of daily dosing to eliminate dire sequelae in patients living longer into adulthood than ever before is recommended.

The use of continuous positive airway pressure (CPAP) in asthma has been a point of debate over the past several years. Various studies, including those on animals and humans have attempted to understand the role and pathophysiology of CPAP in patients with either well controlled or poorly controlled asthma. The aim of this manuscript is to review the currently available literature on the physiologic and clinical effects of CPAP in animal models of asthma and on humans with stable asthma.

Background: Advances in management have improved mortality of individuals with chronic respiratory failure (CRF), leading to an increase in need for long-term oxygen therapy and/or ventilatory support. These individuals require frequent visits and monitoring of their physiological parameters as well as of the functioning of their devices, such as ventilators or oxygen concentrators. Telemedicine is a clinical application of Information Communication Technology connecting patients to specialised care consultants. This narrative review aims to explore the current available telemonitoring options for individuals with CRF and reported or potential results. Methods: The research focused on EMBASE, CINALH, PubMed, and Scopus databases. Papers published between 2003 and 2021 in English were considered. Results: Different sensors, transmission devices and systems, and interventions are used with promising but not conclusive clinical results. However, legal problems are still unsolved, and economic advantages for health care systems, although potentially high, are still under debate. Conclusions: Telemonitoring systems for individuals with CRF are increasingly used; with promising results still to be clarified, legal, economical and organisational issues must be defined.

Background While lung ultrasonography (LUS) has utility for the evaluation of the acute phase of COVID-19 related lung disease, its role in long-term follow-up of this condition has not been well described. The objective of this study is to compare LUS and chest computed tomography (CT) results in COVID-19 survivors with the intent of defining the utility of LUS for long-term follow-up of COVID-19 respiratory disease. Methods Prospective observational study that enrolled consecutive survivors of COVID-19 with acute hypoxemic respiratory failure (HARF) admitted to the Respiratory Intensive Care Unit. Three months following hospital discharge, patients underwent LUS, chest CT, body plethysmography and laboratory testing, the comparison of which forms the basis of this report. Results 38 patients were enrolled, with a total of 190 lobes analysed: men 27/38 (71.1%), mean age 60.6 y (SD 10.4). LUS findings and pulmonary function tests outcomes were compared between patients with and without ILD, showing a statistically significant difference in terms of LUS score (p: 0.0002), FEV1 (p: 0.0039) and FVC (p: 0.012). ROC curve both in lobe by lobe and in patient's overall analysis revealed an outstanding ILD discrimination ability of LUS (AUC: 0.94 and 0.95 respectively) with a substantial Cohen's coefficient (K: 0.74 and 0.69). Conclusions LUS has an outstanding discrimination ability compared to CT in identifying an ILD of at least mild grade in the post COVID-19 follow-up. LUS should be considered as the first-line tool in follow-up programs, while chest CT could be performed based on LUS findings.

Discussion about patients' end-of-life (E-o-L) preferences should be part of the routine practice. Using a semi-structured interview with a scenario-based decision, we performed a prospective multicentre study to elicit the patients' E-o-L preferences in very severe chronic obstructive pulmonary disease (COPD). We also checked their ability to retain this information and the respect of their decisions when they die. Forty-three out of ninety-one of the eligible patients completed the study. The choice of E-o-L practice was equally distributed among the three proposed options: endotracheal intubation (ETI), 'ceiling' non-invasive ventilation (NIV), and palliation of symptoms with oxygen and morphine. NIV and ETI were more frequently chosen by patients who already experienced them. ETI preference was also associated with the use of anti-depressant drugs and a low educational level, while a higher educational level and a previous discussion with a pneumologist significantly correlated with the preference for oxygen and morphine. Less than 50% of the patients retained a full comprehension of the options at 24 hours. About half of the patients who died in the follow-up period were not treated according to their wishes. In conclusion, in end-stage COPD more efforts are needed to improve communication, patients' knowledge of the disease and E-o-L practice.

predictive of cardiovascular death and heart failure hospitalization in heart failure patients. J Am Coll Cardiol 2015;65:657-65. 4. Bayes-Genis A, Barallat J, Pascual-Figal D, et al. Prognostic value and kinetics of soluble neprilysin in acute heart failure. A pilot study. JACC Heart Fail 2015;3:341-4. 5. Yandle TG, Richards M, Smith M, et al. Assay of endopeptidase-24 11 activity in plasma applied to in vivo studies of endopeptidase inhibitors. Clin Chem 1992;38:1785-91. 6. Vodovar N, Seronde MF, Laribi S, et al. Elevated plasma B-type natriuretic peptide concentrations directly inhibit circulating neprilysin activity in heart failure. JACC Heart Fail 2015;3:629-36.

The purpose of this study was to estimate vascular endothelial growth factor (VEGF) and transforming growth factor (TGF)-beta1 serum levels in children with hereditary hemorrhagic telangiectasia (HHT) type 1 and type 2 and to correlate them to the presence of arteriovenous malformations (AVMs). High VEGF levels were initially found in an infant who had been hospitalized with intestinal bleeding and suspected HHT. This case led to the evaluation of VEGF and TGF-beta1 by standard enzyme-linked immunosorbent assay in 13 children with HHT and familiarity. Patients were divided into 2 groups on the basis of the presence/absence of pulmonary AVMs. No significant difference was found for VEGF and TGF-beta1 levels in HHT patients versus controls. Among HHT patients, serum levels of VEGF in those without AVM were significantly lower than those with AVM and normal controls. No difference for TGF-beta1 levels was found in these patient subgroups. Low VEGF levels may represent a protection factor against the onset of pulmonary AVMs in HHT children. However, neither VEGF nor TGF-beta1 can be used as biochemical markers for an early diagnosis in HHT. The diagnosis of HHT still requires clinical criteria, which permitted to confirm the presence of the disease in the infant with intestinal bleeding.

Background Patients with diffuse pulmonary arteriovenous malformations (PAVM), a small but important subset of the PAVM population, have significant morbidity and mortality rates. Methods Thirty-six patients (21 female and 15 male) with diffuse PAVM from a cohort of 821 consecutive patients with PAVM were evaluated. Diffuse PAVM were categorized angiographically: involvement of one or more segmental pulmonary arteries in one or both lungs. Hereditary hemorrhagic telangiectasia (HHT) status, gender, presence or absence of large (≥ 3-mm diameter artery) focal PAVM, oxygen saturations, complications including hemoptysis, years of follow-up, and survival were tabulated. Results HHT was present in 29 of 36 patients (81%), and diffuse PAVM were more commonly bilateral (26 of 36 patients, 72%) than unilateral (10 of 36 patients, 28%) [p = 0.02]. Female gender was associated with bilateral diffuse PAVM (19 of 26 patients, 73%) [p = 0.01]. Focal PAVM were present in both groups but more commonly in patients with bilateral involvement (16 of 26 patients, 62%) [p = 0.02]. Initial oxygen saturations (pulse oximetry, standing) of patients with unilateral and bilateral diffuse PAVM were 87 ± 7% and 79 ± 8% (mean ± SD), respectively (p = 0.02). The last or current values for patients with unilateral and bilateral involvement are 95 ± 3% and 85 ± 7%, respectively (p Conclusions Patients with diffuse PAVM are a high-risk group, and yearly follow-up is recommended.

During the past 20 years, lung transplantation (LTX) has evolved and it is now accepted as a mainstream modality for care of patients with severe life-threatening respiratory diseases that are refractory to maximal conventional therapies. Improvements in surgical techniques and in antirejection medications have resulted in prolonged survival in these patients. Several studies have explored quality of life after LTX and its improvement has been noted especially in the early period between 3 and 6 months. This article discusses the salient features of the physiology of breathing and sleep disturbances before and after LTX and its alterations during sleep.

Background The difficulty in establishing a timely correct diagnosis is a relevant matter of concern for several rare diseases. Many rare-disease-affected patients suffer from considerable diagnostic delay, mainly due to their poor knowledge among healthcare professionals, insufficient disease awareness among patients’ families, and lack of promptly available diagnostic tools. Hereditary Haemorrhagic Telangiectasia (HHT) is an autosomal-dominantly inherited vascular dysplasia, affecting 1:5,000-10,000 patients. HHT is characterized by high variability of clinical manifestations, which show remarkable overlapping with several common diseases. Aim To perform a detailed analysis concerning the diagnostic time lag occurring in patients with HHT, defined as the time period spanning from the first clinical manifestation to the attainment of a definite, correct diagnosis. Methods A questionnaire was administered to the HHT patients previously recruited from 2000 and 2009. Clinical onset, first referral to a physician for disease manifestations, and first correct diagnosis of definite HHT were collected. Eventual misdiagnosis at first referral and serious complications occurring throughout the time elapsing between disease onset and definite diagnosis were also addressed. Results In the 233 respondents, the clinical onset of disease occurred at an age of 14.1 yrs, while the age of first referral and the age of first definite diagnosis of HHT were 29.2 yrs and 40.1 yrs, respectively. Only 88/233 patients received a correct diagnosis at first counseling. Thus, the diagnostic time lag, represented by the time elapsing from disease onset and first definite diagnosis of HHT, proved to be 25.7 yrs. Twenty-two patients suffered from severe complications during this time interval. The diagnostic delay was significantly longer (p Conclusions Our data report for the first time a systematic inquiry of diagnostic delay in HHT showing that patients receive a definite diagnosis only after nearly three decades from disease onset. Concerted efforts are still to be made to increase awareness of this disease among both families and physicians.

The use of medications could be responsible of both side effects and adverse drug reaction (ADR). Identifying risk factors could improve the possibility of avoiding severe reactions in old people. We investigated the prevalence of unpredictable drug adverse reactions among patients admitted to the emergency departments (EDs) of three large Italian hospitals in the period 2005-2008. Clinical characteristics and demographics were carefully recorded in a dedicated database. The assessment of the drug reactions was carried out by an allergist after the first emergency evaluation. Over the considered period, 56,031 patients were admitted at the ED, 2644 (21.2%) of which for ADR. Out of those patients, 55 (2.1%) were identified as unpredictable ADRs. In 96% of the cases the clinical presentation was cutaneous and antibiotics were the most frequently responsible drugs. Patients over 65 years accounted for 37% of the reactions. In those patients the multiple drug regimens were significantly more frequent, as well as the presence of comorbidities. Smoking habit, alcohol abuse and personal and familiar history of atopy did not differ between the younger and the elderly. In the older group, antibiotics were more frequently involved, whereas in the65 years, nonsteroidal antinflammatory drugs accounted for most reactions. Our date outline that in the elderly the comorbidities and correlated multiple regime therapy cause an increased incidence of ADRs, thus suggesting a careful management of therapeutics regimens by means of educational campaigns for patients and guidelines for doctors finalized to avoid excessive drug prescription.

Patients with diffuse pulmonary arteriovenous malformations (PAVMs) are subject to frequent complications and need to be followed closely. As part of this follow-up, we have employed exercise stress testing (EST) as an aid to assess their status. Twenty patients from a cohort of 35 with diffuse PAVMs have undergone EST using a standard cycle ergometer test. All patients had previously undergone pulmonary angiography, noncontrast chest computed tomography (CT), and repair of large focal PAVMs, prior to EST. Mean room air oxygen saturation at baseline and at maximum exercise (85% of maximum heart rate) were tabulated. Serial studies in six children and young adults were plotted by year and compared using the patient as their own control. Fourteen females and six males ranging in age from 4 to 50 years (mean 22 years) were studied. Baseline mean oxygen saturation was 84% and fell to 73% at maximum exercise. There was no significant difference between those with unilateral and bilateral involvement (P = 0.09). In four of the six patients with serial EST, the baseline and exercise oxygen saturations were quite stable. In the two patients who became symptomatic, with age, growth, and more activity, complete embolization of one or more segments of the lung improved their EST and functionality. Based on our previous work in patients with diffuse PAVMs, EST appears to offer a relatively safe and noninvasive method for assessing these patients. Our limited experience with serial EST suggests a good correlation with decreased functionality in these patients.

Background Endothelin-1 (ET-1) and Nitric Oxide (NO) are crucial mediators for establishing pulmonary artery hypertension (PAH). We tested the hypothesis that their imbalance might also occur in COPD patients with PAH. Methods The aims of the study were to measure exhaled breath condensate (EBC) and circulating levels of ET-1, as well as exhaled NO (FENO) levels by, respectively, a specific enzyme immunoassay kit, and by chemiluminescence analysis in 3 groups of subjects: COPD with PAH (12), COPD only (36), and healthy individuals (15). In order to evaluate pulmonary-artery systolic pressure (PaPs), all COPD patients underwent Echo-Doppler assessment. Results Significantly increased exhaled and circulating levels of ET-1 were found in COPD with PAH compared to both COPD (p < 0.0001) only, and healthy controls (p < 0.0001). In COPD with PAH, linear regression analysis showed good correlation between ET-1 in EBC and PaPs (r = 0.621; p = 0.031), and between arterial levels of ET-1 and PaPs (r = 0.648; p = 0.022), while arterial levels of ET-1 inversely correlated with FEV1%, (r = -0.59, p = 0.043), and PaPs negatively correlated to PaO2 (r = -0.618; p = 0.032). Significantly reduced levels of FENO were found in COPD associated with PAH, compared to COPD only (22.92 ± 11.38 vs.35.07 ± 17.53 ppb; p = 0.03). Thus, we observed an imbalanced output in the breath between ET-1 and NO, as expression of pulmonary endothelium and epithelium impairment, in COPD with PAH compared to COPD only. Whether this imbalance is an early cause or result of PAH due to COPD is still unknown and deserves further investigations.

Vascular dementia patients show a component of their impairment as emotional. In the two studies that we present it was possible to illustrate the characteristics of such emotional component. a. The discrepancy between immediate recall and delayed recall appears to be very sensitive to anxiety interference when immediate recall is lower than delayed. b. The presence of emotional problems contributing to memory complaints in VaD patients is not equally distributed along the severity of deterioration dimension but can be identified as a specific component different from the cognitive one especially in that subgroup of patients who present an intermediate level of deterioration.