Prediction of cough effectiveness in amyotrophic lateral sclerosis patients assessed by ultrasuond of the diaphragm during the cough expiration phase.

Assessing cough effectiveness, using Cough Peak Flow, is crucial for patients with Neuromuscular Diseases, such as Amyotrophic Lateral Sclerosis. Impaired cough function can contribute to respiratory decline and failure. The goal of the study is to determine the correlation between diaphragmatic excursion and cough expiratory phase, potentially utilizing ultrasonographic indices to estimate Cough Peak Flow in these patients. Twenty-two patients were enrolled in this study. The upward displacement of the diaphragm was measured with ultrasonography during voluntary cough expiration and Cough Peak Flow was simultaneously measured. A multivariable linear regression model was built to quantify the association between Cough Peak Flow and diaphragm expiratory excursion. There is significative relationship between Cough Peak Flow and diaphragm excursion with a Pearson's r coefficient of 0.86 observed in the patients group. Multiple linear regression analysis for Cough Peak Flow (Adjusted R2 = 0.86) revealed significant associations between Cough Peak Flow and expiratory excursion (adjusted ß-coefficient: 64.78, 95 %, CI: 51.50-78.07, p<0.001) and sex (adjusted ß-coefficient: -69.06; 95 % CI: -109.98 to -28.15, p=0.001). Our results predict the cough effectiveness by using M-mode diaphragmatic sonography with a potentially significant impact on therapeutic choices.

Effective treatment with oral Salbutamol on late onset respiratory impairment in a DOK7 Congenital Myasthenia Syndrome: a case report.

INTRODUCTION: DOK7 gene deficiency is a neuromuscular disease with an alteration in post-synaptic neuromuscular junction, leading to progressive respiratory impairment. Although, the therapy is not standardized, adrenergic agonists are suggested as first-line treatment.  Case presentation: Our patient had an ambiguous late childhood-onset and had a generalized muscle weakness free of respiratory symptoms during the early phase of the disease. Subsequently, when the respiratory muscle and the diaphragm involvement was impaired, a substantial loss of respiratory function with hypopneas and severe desaturation was detected. It was noteworthy the striking respiratory beneficial impact of oral salbutamol in the resolution of symptoms and functional impairments, leading to a remarkable respiratory improvement and a better quality of life.  Conclusion: Oral salbutamol treatment combined to a timely clinical recognition led to an outstanding respiratory improvement.

Surfactant Protein B Plasma Levels: Reliability as a Biomarker in COPD Patients.

Background: The diagnosis of COPD is based on both clinical signs and functional tests. Although there are different functional tests used to assess COPD, no reliable biomarkers able to provide information on pathogenesis and severity are available. The aim of the present study is to explore the relationship between surfactant protein B (Sp-B) serum levels and clinical, radiological, and functional pulmonary parameters in COPD patients. Methods: Forty COPD patients and twenty smokers without airflow limitations or respiratory symptoms were enrolled. Each patient was given questionnaires (CAT and mMRC) and 6MWT, spirometry, DLCO, and computer tomography (CT) were performed. All participants underwent a venous blood sample drawing, and quantitative detection of their Sp-B plasma levels was performed by an enzyme-linked immunosorbent assay. The spirometry and Sp-B plasma levels were assessed after 12 months. Results: A statistically significant difference was found in the plasma Sp-B levels between COPD patients compared to the other group (4.72 + 3.2 ng/mL vs. 1.78 + 1.5 ng/mL; p < 0.001). The change in FEV1 after 12 months (Delta FEV1) showed a significantly negative correlation with respect to the change in Sp-B levels (Delta SpB) (r = −0.4; p < 0.05). This correlation indicates that increasing the plasma dosage of SpB is a foretoken of functional decline. Conclusions: SpB may be considered as a useful marker in COPD assessment and provides prognostic information on lung functional decline. Despite its usefulness, further studies are needed to define its reliability as a biomarker.

Assessing Static Lung Hyperinflation by Whole-Body Plethysmography, Helium Dilution, and Impulse Oscillometry System (IOS) in Patients with COPD.

Purpose: Lung hyperinflation is a feature of chronic obstructive pulmonary disease (COPD) and can determine pivotal consequence on symptoms, exercise tolerance and quality of life. Despite the relevance of assessing lung hyperinflation, there is still no single consensus as to what volume should be taken into account. We investigate which spirometric measurement is more reliable in assessing static lung hyperinflation and which is more related with impulse oscillometry system (IOS) measurements in COPD. Patients and Methods: Fifty-five COPD patients were enrolled. TLC, RV and RV:TLC ratio were obtained both with helium and plethysmography techniques. IOS measurements (X5, Fres and R5-R20) were performed. Pearson and Spearman correlation determined the relationships between the functional parameters that evaluate static hyperinflation (RV: TLC, TLC, RV) and IOS measurements. Results: As expected, we reported a statistically significant difference between these two techniques in terms of mean percentage values of TLC (7.57 ± 3.26 L; p= 0.02) and RV (15.24 ± 7.51 L; p=0.04), while RV:TLC measured with the two methods was similar (5.21 ± 4.69%; p=0.27). The correlation analysis showed that IOS parameters, such as difference in resistance between 5 Hz and 20 Hz (R(5-20)) and resonant frequency (Fres), were positively correlated with RV:TLC ratio, while reactance at 5 Hz (X(5)) was negatively correlated with it. In particular, we pointed out a weak correlation between RV:TLC (%) (Pleth) and R(5-20) (r=0.3, p=0.04), Fres (r=0.3; p=0.03), while X5 had a mild correlation with RV:TLC (%) (r=-0.5;p<0.0001). Moreover, we noticed a strong relationship between RV:TLC (%)(He) and X5 (r=-0.7; p=0.0001) and a mild correlation between RV:TLC (%) (He) and Fres (r=0.4; p=0.003). Between R5-R20 and RV:TLC, there was a weak correlation (r=0.3; p=0.001). No correlation between TLC, RV (L,%) (both helium and Pleth derived) and IOS parameters (R(5-20), X5, Fres) was found. Conclusion: RV:TLC can represent the most reliable parameter in the assessment of hyperinflation, considering the absence of significant difference in its measurement between the two techniques. IOS provides supplementary information in the assessment of static hyperinflation.

Diaphragm paralysis: a case report.

INTRODUCTION: Bilateral paralysis of the diaphragm may be an idiopathic clinical condition or associated with several diseases such as trauma, surgery, viral infections, neurologic disorders. The diaphragm is the main respiratory muscle. It is a cupoliform muscle-tendon structure, innervated bilaterally by phrenic nerve, which originates from C3-C5 nerve roots. Diaphragmatic paralysis is a clinical disorder that generates hypoventilation and basal pulmonary atelectasis, predisposing to hypercapnic respiratory failure. The clinic manifestations mimic cardio-respiratory pathologies, therefore often misdiagnosticated. CASE PRESENTATION: A 55-year-old man with a previous C6-7 traumatic fracture, referred multiple accesses to the emergency room for acute nocturnal dyspnoea, treated with antibiotic therapy, diuretic therapy and long-term oxygen therapy, without beneficial effects. He referred to our pulmonary clinic for evaluation of persistent and worsening orthopnoea due to unknown cause for about 2 years. Clinical examination, respiratory functional tests and diaphragm ultrasound revealed a strong suspicion of diaphragmatic deficit, confirmed by electromyography. CONCLUSIONS: The patient accesses to the emergency room numerous times and the clinical frame have been always oriented towards a cardio-respiratory origin. From the onset of the symptom to the respiratory evaluation, about 2.5 years have passed. The manifestation of clear orthopnoea has addressed the functional respiratory study towards a more thorough diaphragmatic evaluation assessed by ultrasound.

Protective role of brain derived neurotrophic factor (BDNF) in obstructive sleep apnea syndrome (OSAS) patients.

Obstructive sleep apnea syndrome (OSAS) is a common disorder characterized by repeated episodes of upper airways collapse during the sleep. The following intermittent hypoxia triggers a state of chronic inflammation, which also interests the nervous system leading to neuronal damage and increased risk of cognitive impairment. Brain derived neurotrophic factor (BDNF) is a growth factor often associated with neuroplasticity and neuroprotection whose levels increase in several condition associated with neuronal damage. However, whether patients affected by OSAS have altered BDNF levels and whether such alteration may be reflective of their cognitive impairment is still controversial. Here we show that, when compared to healthy control volunteers, OSAS patients have increased serum levels of BDNF. Moreover, OSAS patients with the higher levels of BDNF also have reduced neurocognitive impairment as measured by The Montreal Cognitive Assessment (MoCA) questionnaire. Treatment with standard non-invasive mechanical ventilation (CPAP) also was able to ameliorate the level of cognitive impairment. Altogether our results indicate that BDNF levels represent a neuroprotective response to intermittent hypoxia in OSAS patients.

Percutaneous transcatheter aortic valve replacement induces femoral artery shrinkage: angiographic evidence and predictors for a new side effect.

OBJECTIVES: We sought to investigate the possible impact of transaortic valve replacement (TAVR) on common femoral artery (CFA) integrity as assessed by angiography. BACKGROUND: CFA represents the most adopted access for TAVR but various degrees of vascular damage may be induced by the procedure. METHODS: Patients underwent percutaneous transfemoral TAVR who had both pre- and post-TAVR access-site angiography were retrospectively selected. Clinical and procedural data (including technique and complications) were prospectively recorded into a structured TAVR database. Pre-TAVR and post-TAVR angiograms were analyzed using a quantitative angiographic analysis software to assess reference diameters, minimum luminal diameter (MLD), and percentage of diameter stenosis (DS). RESULTS: A total of 124 patients entered the study (mean age: 85 years, mean Euroscore II: 10%). ProStar (13.5%) and double ProGlide (82.2%) preclosure were the main hemostatic techniques. CFA exhibited a significant shrinkage with TAVR as assessed by significant MLD reduction (5.6 mm after TAVR vs. 6.8 mm before, P < .001) and DS increase (30.3% after vs. 17.0%, P < .001). Such differences remained statistically significant after exclusion of 18 patients (14.2%) who had (minor or major) vascular complications. At multivariable analysis, pre-TAVR DS (P = .03) and history of peripheral arterial disease (P = .01), were significantly associated with vascular complications. CONCLUSIONS: Percutaneous TAVR induces an angiographically detectable CFA lumen reduction. Such findings call for further studies assessing clinical impact of this phenomenon and open the door for further refinements of the TAVR access management aimed at preserving vessel integrity.