Interplay between speech and gait variables in Parkinson's disease patients treated with subthalamic nucleus deep brain stimulation: A long-term instrumental assessment.

OBJECTIVE: To evaluate correlations between speech and gait parameters in the long term and under different medication and subthalamic nucleus deep brain stimulation (STN-DBS) conditions in a cohort of advanced Parkinson's disease (PD) patients. METHODS: This observational study included consecutive PD patients treated with bilateral STN-DBS. Axial symptoms were evaluated using a standardized clinical-instrumental approach. Speech and gait were assessed by perceptual and acoustic analyses and by the instrumented Timed Up and Go (iTUG) test, respectively. Disease motor severity was evaluated with the total score and subscores of the Unified Parkinson's Disease Rating Scale (UPDRS) Part III. Different stimulation and drug treatment conditions were assessed: on-stimulation/off-medication, off-stimulation/off-medication, and on-stimulation/on-medication. RESULTS: Twenty-five PD patients with a 5-year median follow-up after surgery (range 3-7 years) were included (18 males; disease duration at surgery: 10.44 [SD 4.62] years; age at surgery: 58.40 [SD 5.73] years). In the off-stimulation/off-medication and on-stimulation/on-medication conditions, patients who spoke louder had also the greater acceleration of the trunk during gait; whereas in the on-stimulation/on-medication condition only, patients with the poorer voice quality were also the worst to perform the sit to stand and gait phases of the iTUG. Conversely, patients with the higher speech rate performed well in the turning and walking phases of the iTUG. CONCLUSIONS: This study underlines the presence of different correlations between treatment effects of speech and gait parameters in PD patients treated with bilateral STN-DBS. This may allow us to better understand the common pathophysiological basis of these alterations and to develop a more specific and tailored rehabilitation approach for axial signs after surgery.

Monitoring Involuntary Muscle Activity in Acute Patients with Upper Motor Neuron Lesion by Wearable Sensors: A Feasibility Study.

Sustained involuntary muscle activity (IMA) is a highly disabling and not completely understood phenomenon that occurs after a central nervous system lesion. We tested the feasibility of in-field IMA measuring at an acute rehabilitation ward. We used wearable probes for single differential surface EMG (sEMG), inclusive of a 3D accelerometer, onboard memory and remote control. We collected 429 h of data from the biceps brachii of 10 patients with arm plegia. Data quality was first verified in the time and frequency domains. Next, IMA was automatically identified based on the steady presence of motor unit action potential (MUAP) trains at rest. Feasibility was excellent in terms of prep time and burden to the clinical staff. A total of 350.5 h of data (81.7%) were reliable. IMA was found in 85.9 h (25%). This was often present in the form of exceedingly long-lasting trains of one or a few MUAPs, with differences among patients and variability, both within and between days in terms of IMA duration, root mean square (RMS) and peak-to-peak amplitude. Our results proved the feasibility of using wearable probes for single differential sEMG to identify and quantify IMA in plegic muscles of bedridden acute neurological patients. Our results also suggest the need for long-lasting acquisitions to properly characterize IMA. The possibility of easily assessing IMA in acute inpatients can have a huge impact on the management of their postures, physiotherapy and treatments.

Feasibility and predictive performance of the Hendrich Fall Risk Model II in a rehabilitation department: a prospective study.

BACKGROUND: Falls are a common adverse event in both elderly inpatients and patients admitted to rehabilitation units. The Hendrich Fall Risk Model II (HIIFRM) has been already tested in all hospital wards with high fall rates, with the exception of the rehabilitation setting. This study's aim is to address the feasibility and predictive performances of HIIFRM in a hospital rehabilitation department. METHODS: A 6 months prospective study in a Italian rehabilitation department with patients from orthopaedic, pulmonary, and neurological rehabilitation wards. All admitted patients were enrolled and assessed within 24 h of admission by means of the HIIFRM. The occurrence of falls was checked and recorded daily. HIIFRM feasibility was assessed as the percentage of successful administrations at admission. HIIFRM predictive performance was determined in terms of area under the Receiver Operating Characteristic (ROC) curve (AUC), best cutoff, sensitivity, specificity, positive and negative predictive values, along with their asymptotic 95% confidence intervals (95% CI). RESULTS: One hundred ninety-one patents were admitted. HIIFRM was feasible in 147 cases (77%), 11 of which suffered a fall (7.5%). Failures in administration were mainly due to bedridden patients (e.g. minimally conscious state, vegetative state). AUC was 0.779(0.685-0.873). The original HIIFRM cutoff of 5 led to a sensitivity of 100% with a mere specificity of 49%(40-57%), thus suggesting using higher cutoffs. Moreover, the median score for non-fallers at rehabilitation units was higher than that reported in literature for geriatric non fallers. The best trade-off between sensitivity and specificity was obtained by using a cutoff of 8. This lead to sensitivity = 73%(46-99%), specificity = 72%(65-80%), positive predictive value = 17% and negative predictive value = 97%. These results support the use of the HIIFRM as a predictive tool. CONCLUSIONS: The HIIFRM showed satisfactory feasibility and predictive performances in rehabilitation wards. Based on both available literature and these results, the prediction of falls among all hospital wards, with high risk of falling, could be achieved by means of a unique tool and two different cutoffs: a standard cutoff of 5 in geriatric wards and an adjusted higher cutoff in rehabilitation units, with predictive performances similar to those of the best-preforming pathology specific tools for fall-risk assessment.

Endobronchial ultrasound-guided transbronchial needle aspiration under conscious sedation with meperidine and midazolam.

Endobronchial Ultrasound guided transbronchial needle aspiration (EBUS-TBNA) is usually performed under general anesthesia or deep sedation with drugs such as Propofol that, at least in Italy, can be administered only by an anesthesiologist. Aim of the study was to assess conscious sedation feasibility, safety and tolerability using Meperidine and Midazolam as administered by Pulmonologist and relevant impact on the efficiency of the sampling procedures.All patients undergoing EBUS-TBNA from February 2013 to July 2014 were examined retrospectively. Efficiency using Meperidine and Midazolam during EBUS-TBNA has been assessed: completion of lymph-nodal sampling, sampling adequacy, diagnostic yield, cough during endoscopic procedure complications and need for procedure repetition with Anesthesiology assistance. Patient satisfaction and cost/effectiveness were also evaluated. One hundred and thirty-four consecutive patients were considered; 97.7% completed the procedure. In 96.9% of cases the prefixed program of lymph-nodal sampling was accomplished. Sampling adequacy was 92,4%. Diagnostic yield was 55%. In 94.7% of cases cough was absent or did not interfere with EBUS-TBNA. The need to repeat the endoscopic procedure occurred in 6 cases but only in 2 the presence of an Anesthesiologist was required. Patient satisfaction was very high, with 95.9% of subjects reporting they would "definitely return". A 27% cost reduction was calculated. EBUS-TBNA under conscious sedation using Meperidine and Midazolam prescribed and administered by pulmonologist without the Anesthesiologist assistance, revealed to be a safe, well tolerated and cost saving procedure. The efficiency of sampling was good, apart from a relatively low diagnostic yield due to different expertise of operators.

Self-managed physical activity in breast cancer survivors: A scoping review.

OBJECTIVE: Breast cancer survivors (BCS) experience many issues of rehabilitative concern due to the treatments they have undergone. Given the chronicity of these outcomes, the increasing number of survivors, and the positive results obtained by supervised exercise, professionals should consider offering self-managed physical activity (PA) programs to this population. Our aim was to map the currently available evidence about self-care rehabilitation for BCS. METHODS: Medline, CINAHL, and Cochrane databases were searched for primary literature. Scoping review methodological frameworks were used to tackle the heterogeneity of the topic. Studies investigating self-managed PA interventions prescribed to adult BCS were included. RESULTS: One hundred-eight studies were included, with sample sizes ranging from 6 to 692 patients. Information was systematically collected in tables displaying study design, type of PA, duration and recommended frequency, professional leading the study, type of supervision, initial training, strategies used to help patients integrate self-care into their daily lives, and self-managed PA efficacy. Tables were produced for every oncological side effect that BCS might experience: lymphedema, arthralgia, cancer-related fatigue, a decline in physical parameters, treatment-related cardiotoxicity, peripheral neurotoxicity, and a possible decline in the quality of life. CONCLUSIONS: Self-managed PA has the potential to improve BCS oncological issues. Professionals can adopt many strategies to support patients and empower them with long-lasting self-care competencies. This scoping review provided a comprehensive and easy-to-consult overview of self-managed PA interventions for BCS. We also provided recommendations for future primary studies and secondary synthesis.

Outcome measures for assessing the effectiveness of physiotherapy interventions on equinus foot deformity in post-stroke patients with triceps surae spasticity: A scoping review.

OBJECTIVE: Equinus foot deformity (EFD) is the most common deviation after stroke. Several physiotherapy interventions have been suggested to treat it. However, studies evaluating the efficacy of these treatments vary widely in terms of assessment modalities, type of data analysis, and nomenclature. This scoping review aimed to map current available evidence on outcome measures and the modalities employed to assess the effectiveness of physiotherapy programs for the reduction of triceps surae (TS) spasticity and EFD in patients with stroke. METHODS: Scoping review methodological frameworks have been used. Three databases were investigated. Primary literature addressing TS spasticity in adult patients with stroke using physiotherapy interventions was included. Findings were systematically summarized in tables according to the intervention used, intervention dosage, control group, clinical, and instrumental outcome measures. RESULTS: Of the 642 retrieved studies, 53 papers were included. TS spasticity was assessed by manual maneuvers performed by clinicians (mainly using the Ashworth Scale), functional tests, mechanical evaluation through robotic devices, or instrumental analysis and imaging (such as the torque-angle ratio, the H-reflex, and ultrasound images). A thorough critical appraisal of the construct validity of the scales and of the statistics employed was provided, particularly focusing on the choice of parametric and non-parametric approaches when using ordinal scales. Finally, the complexity surrounding the concept of "spasticity" and the possibility of assessing the several underlying active and passive causes of EFD, with a consequent bespoke treatment for each of them, was discussed. CONCLUSION: This scoping review provides a comprehensive description of all outcome measures and assessment modalities used in literature to assess the effectiveness of physiotherapy treatments, when used for the reduction of TS spasticity and EFD in patients with stroke. Clinicians and researchers can find an easy-to-consult summary that can support both their clinical and research activities.

Long-term effects of bilateral subthalamic nucleus deep brain stimulation on gait disorders in Parkinson's disease: a clinical-instrumental study.

OBJECTIVE: To assess the long-term effects of bilateral subthalamic nucleus deep brain stimulation (STN-DBS) on gait in a cohort of advanced Parkinson's Disease (PD) patients. METHODS: This observational study included consecutive PD patients treated with bilateral STN-DBS. Different stimulation and drug treatment conditions were assessed: on-stimulation/off-medication, off-stimulation/off-medication, and on-stimulation/on-medication. Each patient performed the instrumented Timed Up and Go test (iTUG). The instrumental evaluation of walking ability was carried out with a wearable inertial sensor containing a three-dimensional (3D) accelerometer, gyroscope, and magnetometer. This device could provide 3D linear acceleration, angular velocity, and magnetic field vector. Disease motor severity was evaluated with the total score and subscores of the Unified Parkinson Disease Rating Scale part III. RESULTS: Twenty-five PD patients with a 5-years median follow-up after surgery (range 3-7) were included (18 men; mean disease duration at surgery 10.44 ± 4.62 years; mean age at surgery 58.40 ± 5.73 years). Both stimulation and medication reduced the total duration of the iTUG and most of its different phases, suggesting a long-term beneficial effect on gait after surgery. However, comparing the two treatments, dopaminergic therapy had a more marked effect in all test phases. STN-DBS alone reduced total iTUG duration, sit-to-stand, and second turn phases duration, while it had a lower effect on stand-to-sit, first turn, forward walking, and walking backward phases duration. CONCLUSIONS: This study highlighted that in the long-term after surgery, STN-DBS may contribute to gait and postural control improvement when used together with dopamine replacement therapy, which still shows a substantial beneficial effect.

Clinical audit on diagnostic accuracy and management of respiratory failure in COPD.

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.

Self-managed physical activity in cancer survivors for the management of cancer-related fatigue: A scoping review.

OBJECTIVE: Cancer-related fatigue (CRF) is a disabling chronic condition that cancer survivors could experience during and after recovery and that might benefit from self-managed physical activity (PA) programs. This scoping review aimed to map self-managed PA interventions found in literature for the management of CRF. METHODS: Given the heterogeneity of the topic, scoping review methodological frameworks were used. Pubmed, Cinahl and Cochrane databases were searched for primary literature. Inclusion criteria: self-managed PA meant as any exercise program prescribed by a professional either with or without initial supervision and training which then continued independently for a given time frame; patient-reported fatigue assessment included in the outcome measures. Articles dealing with entirely supervised interventions, dietary or psychological-only therapies, and with palliative care were excluded. RESULTS: Of the 543 experimental or observational studies screened, 63 were included. Of these forty-three studies were randomized controlled trials. Data were summarized in tables describing self-managed interventions according to: type of self-managed activity, frequency and duration, strategies to promote adherence, professionals supervising the treatment, outcome measures, and efficacy. A narrative synthesis was also added to further explain findings. CONCLUSIONS: We collected the available evidence on PA when this was self-managed by patients after prescription by a healthcare provider. Clinicians and researchers should consider incorporating self-care programs in CRF patients' recovery journey gradually, identifying the best strategies to integrate them into daily life. Researchers should specify the characteristics of PA programs when designing new studies. This review highlighted the areas to be investigated for future studies pertaining to self-managed PA.

Physical therapy interventions for the correction of equinus foot deformity in post-stroke patients with triceps spasticity: A scoping review.

Objective: Equinus foot deformity (EFD) is the most common deformity following a stroke. Several approaches have been suggested for its correction, including pharmacological, surgical, and physical therapy (PT) interventions. This scoping review aims to map and synthesize the available evidence focusing on physical therapy treatments for EFD caused by triceps surae (TS) spasticity. Methods: Scoping review methodological frameworks have been used. Pubmed, Cinahl, and Cochrane databases were searched for primary literature. Studies focusing on the treatment of EFD in adult stroke patients were included only when the intervention involved PT treatments and presented at least one outcome measure for the functional and/or structural condition of the TS. Data were systematically collected and reported in tables inclusive of type of intervention, sample characteristics, dosage, comparators, outcomes, follow-up timeline, and treatment efficacy. A narrative synthesis was also added. Results: Of the 642 experimental or observational screened studies, 53 were included, focusing on stretching exercises, shock waves, electrical stimulation, dry needling, TENS, vibration therapy, ultrasounds, cryotherapy, and active physiotherapy. Patients with EFD benefited from specific physical therapy treatments. These usually resulted in Modified Ashworth Scale reduction, typically by 1 point, and an increase in ROM. Interventions consisting of shock waves, dry needling, and electrostimulation showed the best results in reducing EFD. Heterogeneous dosage and delivery mode generally limited conclusions. Conclusions: This scoping review summarized available primary literature based on PT treatments for the correction of EFD. By highlighting the remaining gaps in knowledge, it provides a reference for future studies on this pathology. Further investigations are necessary to pinpoint the best dosage and delivery methods. Future studies should investigate whether early rehabilitation programs started during the acute phase might help prevent or limit the development of secondary deformities.

Freezing of Gait in Parkinson's Disease Patients Treated with Bilateral Subthalamic Nucleus Deep Brain Stimulation: A Long-Term Overview.

Bilateral subthalamic nucleus deep brain stimulation (STN-DBS) is an effective treatment in advanced Parkinson's Disease (PD). However, the effects of STN-DBS on freezing of gait (FOG) are still debated, particularly in the long-term follow-up (≥5-years). The main aim of the current study is to evaluate the long-term effects of STN-DBS on FOG. Twenty STN-DBS treated PD patients were included. Each patient was assessed before surgery through a detailed neurological evaluation, including FOG score, and revaluated in the long-term (median follow-up: 5-years) in different stimulation and drug conditions. In the long term follow-up, FOG score significantly worsened in the off-stimulation/off-medication condition compared with the pre-operative off-medication assessment (z = -1.930; p = 0.05) but not in the on-stimulation/off-medication (z = -0.357; p = 0.721). There was also a significant improvement of FOG at long-term assessment by comparing on-stimulation/off-medication and off-stimulation/off-medication conditions (z = -2.944; p = 0.003). These results highlight the possible beneficial long-term effects of STN-DBS on FOG.

Performance of the Hendrich Fall Risk Model II in Patients Discharged from Rehabilitation Wards. A Preliminary Study of Predictive Ability.

(1) Background: Falls are a dangerous adverse event in patients discharged from rehabilitation units, with the risk of falling being higher in the first weeks after discharge. In this study, we assessed the predictive performance of the Hendrich Fall Risk Model II tool (HIIFRM) when administered before discharging patients to their home from rehabilitative units in orthopedic (OR), neurologic (NR) and pulmonary (PR) rehabilitation wards. (2) Methods: Over a 6-month period, all adult patients who returned home after discharge were assessed by HIIFRM. At six months from discharge the occurrence of falls was obtained by performing a structured survey. The HIIFRM predictive performance was determined by the area under the ROC curve (AUC), sensitivity (Se) and specificity (Sp) for the whole sample and split by ward. (3) Results: 85 of 141 discharged patients were living at home and agreed to take part in the survey. Of these, 19 subjects fell, 6 suffered fractures or head traumas and 5 were hospitalized. The AUC was 0.809 (95% CI: 0.656-0.963), Se was 0.67 (0.30-0.93) and Sp was 0.79 (0.63-0.90) for OR patients. (4) Conclusions: Our preliminary results support the use of HIIFRM as a tool to be administered to OR patients at discharge and provides data for the design of a large study of predictive ability.

Rehabilitation and respiratory management in the acute and early post-acute phase. "Instant paper from the field" on rehabilitation answers to the COVID-19 emergency.

COVID-19 is a respiratory infectious disease that can cause respiratory, physical and psychological long-term dysfunctions in patients. First recommendations on respiratory management were published, but they were not based on the specific needs due to COVID-19. In this paper we share the early experiences from the clinical field in Northern Italy, where the epidemic started in February. This paper summarizes the second webinar on COVID-19 (230 live attendees, 11,600 viewers of the recorded version) organized by the Italian Society of Physical and Rehabilitation Medicine about rehabilitation and in particular respiratory management in the acute (Intensive Care Unit [ICU]) and immediate post-acute phases. There is the need to prepare for the post-acute phase. ICU length of stay is relatively long, with immobilization in prone position. Some specific problems are described, including severe muscle weakness and fatigue, joint stiffness, dysphagia, (neuro)psychological problems, impaired functioning concerning mobility, activities of daily life and work. A lot is yet unknown and patients can experience long-term consequences as we know from the literature on the postintensive care syndrome, but COVID-19 has unique features to be investigated and understood. As a colleague stated during the Covinar: this is a marathon, not a sprint….

The effectiveness of a bronchial drainage technique (ELTGOL) in COPD exacerbations.

BACKGROUND AND OBJECTIVE: Exacerbations of COPD are often characterized by increased mucus production that is difficult to treat and worsens patients' outcome. This study evaluated the efficacy of a chest physiotherapy technique (expiration with the glottis open in the lateral posture, ELTGOL) during acute exacerbations of COPD using as outcome measures sputum volume, length of hospitalization, reduction in dyspnoea (Borg score), improvement in quality of life (assessed by the St George Respiratory Questionnaire) and incidence of COPD exacerbations during follow up. METHODS: The study recruited 59 patients hospitalized for the treatment of acute exacerbation of COPD, who were randomly assigned to a control group and an intervention group. The control group was treated with standard medical therapy while the intervention group was treated with ELTGOL plus medical therapy. A subgroup of patients was followed for 6 months to verify the effects on COPD exacerbations and need for hospitalizations. RESULTS: At the time of hospital discharge there was no significant difference between the two groups in the outcome measures, with the exception of the Borg score, which was significantly improved in the ELTGOL group (4.3 +/- 1.5 in the control group vs 3.0 +/- 1.8 in the ELTGOL group, P = 0.004). After 6 months there was no significant difference in the other measured parameters between a subset of the groups available for follow up. During follow up, the ELTGOL group had numerically fewer exacerbations and less need for hospitalization though differences were not statistically significant. CONCLUSIONS: Chest physiotherapy using the ELTGOL technique has a limited role in patients with mild exacerbation of moderate to severe COPD with a tendency towards fewer exacerbations and hospitalizations.

Development of the Decannulation Prediction Tool in Patients With Dysphagia After Acquired Brain Injury.

OBJECTIVES: Patients with acquired brain injuries (ABIs) often need tracheostomy because of dysphagia. However, many of them may recover over time and be eventually decannulated during post-acute rehabilitation. We developed the Decannulation Prediction Tool (DecaPreT) to identify, early in the post-acute course, patients with ABIs who can be safely decannulated. DESIGN: Nonconcurrent cohort study. SETTING AND PARTICIPANTS: Patients with ABI, as well as with dysphagia and tracheostomy, were retrospectively selected from the database of a neurorehabilitation unit in Correggio, Reggio Emilia, Italy. MEASURES: Potential bivariate predictors of decannulation were screened from variables collected on admission during clinical examination, conducted by an expert speech therapist. Multivariable prediction was then obtained in 2 separate random subsamples to develop and validate the logistic regression model of the DecaPreT. RESULTS: Of 463 patients with ABI (mean age 52.2 years) selected, 73.0% could be safely decannulated before discharge. After bivariate screening, multivariable predictors of decannulation were identified in the development subsample and confirmed in the validation subsample, each with its odds ratio and 95% confidence interval as follows: age tertile (1.77, 1.08-2.89; P = .024), no saliva aspiration (3.89, 1.73-8.64; P = .001), pathogenesis of ABI (trauma vs other causes vs stroke vs anoxia: 2.23, 1.41-3.54; P = .001), no vegetative status (8.47; 2.91-24.63; P < .001), and coughing score (voluntary and reflex vs voluntary vs reflex vs neither voluntary nor reflex cough: 2.62, 1.70-4.05; P < .001). In the validation subsample, the predicting equation obtained an area under the receiver operating characteristics curve of 0.836. IMPLICATIONS: The DecaPreT predicts safe decannulation in patients with dysphagia and tracheostomy, using simple clinical variables detected early in the post-acute phase of ABI. The tool can help clinicians choose timing and intensity of rehabilitation interventions and plan discharge.

Heat-induced necrosis after bronchial thermoplasty: a new concern?

BACKGROUND: Bronchial thermoplasty (BT) is an endoscopic procedure for the treatment of severe refractory asthma, based on the local airways delivery of radio-frequency at 65 °C. Several controlled clinical studies demonstrated the effectiveness of BT on clinical outcomes, particularly the reduction of asthma exacerbations. During procedure or shortly after, significant but transient respiratory adverse events have been reported. CASE REPORT: We describe the case of a male, caucasian, 56-year-old, non-smoker patient with non-allergic severe asthma. A few days after the second BT session performed in the left lower lobe, persistent haemoptysis appeared requiring patient hospitalization. A chest CT scan showed mild varicoid bronchiectasis and distal parenchymal infiltrate in the basal anterior segment of the left lower lobe. At fibreoptic bronchoscopy two small nodular neoformations were observed in sub-segmental areas of the same lobe. Histological examination showed mild non-specific inflammation of bronchial mucosa, and some large fragments of peribronchial pulmonary parenchyma with an area of haemorrhagic necrosis. The patient was treated empirically with co-amoxiclav, azithromycin and prednisone. A new chest CT showed a complete resolution of the parenchymal opacity. Finally, the patient underwent the third session of BT, without recurrence of haemoptysis or radiological changes. DISCUSSION: Bronchial thermoplasty is a generally safe procedure. To our knowledge this is the first report of necrosis of the treated bronchus and haemoptysis complicating BT after the second session. The pulmonary damage was most likely determined by a thermal shock induced by BT. One hypothesis could be a structural fragility of the treated bronchus, possibly related to bronchiectasis. A technical malfunction of the BT controller or the catheter, causing an excessive energy delivery could not be excluded. Adverse events following BT deserve particular attention but should not discourage clinicians from the application of this promising procedure.

Acute respiratory failure as presentation of late-onset Pompe disease complicating the diagnostic process as a labyrinth: a case report.

BACKGROUND: Acute respiratory failure can be triggered by several causes, either of pulmonary or extra-pulmonary origin. Pompe disease, or type II glycogen storage disease, is a serious and often fatal disorder, due to a pathological accumulation of glycogen caused by a defective activiy of acid α-glucosidase (acid maltase), a lysosomal enzyme involved in glycogen degradation. The prevalence of the disease is estimated between 1 in 40,000 to 1 in 300,000 subjects. CASE PRESENTATION: This case report describes a difficult diagnosis of late-onset Pompe disease (LOPD) in a 52 year old Caucasian woman with acute respiratory failure requiring orotracheal intubation and subsequent tracheostomy for long-term mechanical ventilation 24 h/day. Despite a complex diagnostic process including several blood tests, bronchoscopy with BAL, chest CT, brain NMR, electromyographies, only a muscle biopsy allowed to reach the correct diagnosis. DISCUSSION: The most frequent presentation of myopathies, including LOPD, is proximal limb muscle weakness. Respiratory related symptoms (dyspnea on effort, reduced physical capacity, recurrent infections, etc.) and respiratory failure are often evident in the later stages of the diseases, but they have been rarely described as the onset symptoms in LOPD. In our case, a third stage LOPD, the cooperation between pulmonologists and neurologists was crucial in reaching a correct diagnosis despite a very complex clinical scenario due to different confounding co-morbidities as potential causes of respiratory failure and an atypical presentation. In this patient, enzyme replacement therapy with infusion of alglucosidase alfa was associated with progressive reduction of ventilatory support to night hours, and recovery of autonomous walking.

Bronchial thermoplasty and the role of airway smooth muscle: are we on the right direction?

Asthma is characterized by inflammation of the airways that includes eosinophils, basal membrane thickening, epithelial sloughing, vascular changes, smooth muscle hypertrophy and hyperplasia, and mucous gland hyperplasia. Recently, there have been studies on the role of hypersensitivity and inflammation in asthma, but the role of bronchial smooth muscle remains unclear. Bronchial thermoplasty is an endoscopic procedure that is approved by the US Food and Drug Administration (FDA) for the treatment of severe refractory asthma, based on the local delivery of radio frequency at 65°C to the airways, with the aim of controlling bronchospasm through a reduction of airway smooth muscle (ASM). Several recent studies have shown significant improvement in clinical outcomes of bronchial thermoplasty for asthma, including symptom control, reduction in exacerbation and hospitalization rates, improved quality of life, and reduction in number of working days or school days lost due to asthma. Data from these recent studies have shown reduction in ASM following bronchial thermoplasty and changes in inflammation patterns. It has also been argued that bronchial thermoplasty may have modulating effects on neuroendocrine epithelial cells, bronchial nerve endings, TRPV1 nerve receptors, and type-C unmyelinated fibers in the bronchial mucosa. This may involve interrupting the central and local reflexes responsible for the activation of bronchospasm in the presence of bronchial hyperreactivity. Several questions remain regarding the use of bronchial thermoplasty, mechanism of action, selection of appropriate patients, and long-term effects. In this review, the role of ASM in the pathogenesis of asthma and the key aspects of bronchial thermoplasty are discussed, with a focus on the potential clinical effects of this promising procedure, beyond the reduction in ASM.

Near-fatal asthma responsive to mepolizumab after failure of omalizumab and bronchial thermoplasty.

Severe asthma affects between 5% and 10% of patients with asthma worldwide and requires best standard therapies at maximal doses, but there is a subgroup of patients refractory to all treatments. We share a case report of a 53-year-old woman with a history of severe allergic asthma that progressively worsened over the years despite the best therapy. She had been hospitalized 35 times, including nine admissions to the respiratory intensive care unit due to severe exacerbations. To rule out other possible diagnoses, several investigations were performed, such as computed tomography scan of the chest and neck, fiberoptic laryngoscopy, antineutrophil cytoplasmic antibodies, and complete blood cell count. The patient was first treated with omalizumab, which was completely ineffective, and then with bronchial thermoplasty (BT), again without clinical benefit. The situation remained critical for about 3 months during the last hospitalization, but in February 2017, the Italian Medicines Agency approved the treatment of severe refractory eosinophilic asthma with mepolizumab (Nucala®). Given a blood eosinophil count of 300 cells/µL, our patient was started on 100 mg mepolizumab treatment. After the second administration, symptoms improved progressively, with a reduction in the number and severity of exacerbations, so the patient could finally be discharged from hospital. At follow-up, it was possible to reduce and then suspend oral corticosteroids by continuing only with inhaled corticosteroids/long-acting beta-agonists and montelukast. No further asthmatic exacerbations occurred; symptom control and quality of life improved significantly. To our knowledge, this is the first case of a patient unresponsive to omalizumab and BT but with excellent clinical response to mepolizumab. She is also the first patient to be treated with an anti-IL5 agent in Italy in a real-life clinical setting. The availability of new effective biological agents will allow many patients to resume as normal a life as possible, with a positive outcome also from a social and economic point of view.

A randomized controlled trial on office spirometry in asthma and COPD in standard general practice: data from spirometry in Asthma and COPD: a comparative evaluation Italian study.

STUDY OBJECTIVES: To evaluate whether office spirometry by general practitioners (GPs) is feasible and may improve the diagnosis of asthma and COPD. METHODS: A prospective, randomized, comparative trial was planned involving 57 Italian pulmonology centers and 570 GPs who had to enroll consecutive subjects aged 18 to 65 years with symptoms of asthma or COPD without a previous diagnosis. Patients were randomized 1:1 into two groups with an interactive voice responding system: conventional evaluation alone vs conventional evaluation and spirometry. Office spirometry was performed by GPs who were trained by reference specialists using a portable electronic spirometer (Spirobank Office; MIR; Rome, Italy). Diagnosis was confirmed by the reference specialist center in blind fashion. RESULTS: Seventy-four GPs complied to the trial. Of 333 patients enrolled, 136 nonrandom violators completed the protocol. Per-protocol analysis showed a concordant diagnosis between GPs and specialists in 78.6% of cases in the conventional evaluation-plus-spirometry group vs 69.2% in the conventional evaluation group (p = 0.35). In the intention-to-treat analysis, the respective percentages of concordant diagnosis were 57.9 and 56.7 (p = 0.87). CONCLUSIONS: Office spirometry by GPs is feasible, but frequent protocol violation and inadequate sample size did not allow us to prove a significant advantage of office spirometry in improving the diagnosis of asthma and COPD in standard general practice as organized at present in Italy, thus reinforcing the need for close cooperation between GPs and specialists in respiratory medicine.