Respiratory Impairment Research Articles

Respiratory impairments in patients suffering from Fabry disease - A cross-sectional study.

The inherited X-linked disorder, Fabry disease, is caused by deficient lysosomal enzyme α-galactosidase A, with progressive accumulation of globotriaosylceramide in multiple organs including the upper and lower airways. To assess pulmonary function at the time of the first pulmonary function test (PFT) performed among the National Danish Fabry cohort and define the prevalence of affected lung function variables. A cross-sectional retrospective cohort study of 86 adult patients enrolled in one or both international patient registry databases for Fabry disease, Fabry Registry or FollowME with at least one PFT. The Mainz Severity Score Index (MSSI) was calculated to determine the disease severity. Lung function variables were examined by multivariate regression adjusted for important variables for developing airway illness. Seventeen patients (20%) showed obstructive airflow limitation and 7 (8%) a restrictive lung deficiency. Smoking status (p = .016) and MSSI (p < .001) were associated with increasing obstructive airway limitation. The prevalence of affected lung function among the National Danish Fabry cohort was 28%. Patients with classic gene variants frequently developed a decrease in lung function regardless of their smoking status, with significant relationship with disease severity.

Long-term mechanical ventilation in a myasthenic patient with suspected obstructive sleep apnea

Myasthenic crisis is a medical emergency with a very poor prognosis if not treated early and appropriately. In the absence of mechanical ventilation, the respiratory impairment is the most serious aspect of the condition, leading the intensive care anaesthetist to resort to intubation. The association of this disease with sleep apnea syndrome is frequent but little-known, and can make it difficult to disconnect the patient from the ventilator. Making the diagnosis of sleep apnea syndrome is difficult because of polysomnography, which is laborious to set up in intensive care, but can be facilitated by the use of a simple questionnaire when there is a strong clinical suspicion. Weaning a patient suffering from neuromuscular disease, whether or not associated with sleep apnea syndrome from mechanical ventilation can be a complex task. When extubation proves impossible, tracheostomy remains the most desirable alternative. The introduction of long-term mechanical ventilation at home is an interesting solution for shortening the long hospital stay of patients in intensive care units, and significantly reduces the risk of infection, morbidity and mortality.

Assessment of pulmonary function and respiratory symptoms among INDIAN textile sizing mill workers.

Textile-sizing mill workers are exposed to various hazards in the sizing units during their working hours and are at risk of acquiring lung impairments due to the usage of sizing chemicals in the sizing process. The main aim of this study is to assess the influence of cotton dust and sizing agents on lung function and breathing difficulties among Indian textile sizing mill workers. This cross-sectional study was carried out at a textile-sizing mill from August 2022 to September 2022. A modified questionnaire based American Thoracic Society's standard was used to assess respiratory symptoms among sizing mill workers and the pulmonary function test was conducted Spirometry. The chi-square test was used to find the difference between respiratory symptoms and the t-test was used to find the difference between spirometric parameters. Textile sizing mill workers showed significant (P < 0.0001) decline in peak expiratory flow rate, forced vital capacity (FVC), ratio of FEV1 and forced vital capacity, and forced expiratory volume in 1 s (FEV1). There was an association between symptoms and duration of exposure to pulmonary abnormality. Sizing mill workers showed a significant decline in lung functions and an increase in pulmonary symptoms. As the service duration of exposure in terms of years increased, respiratory symptoms increased and spirometric abnormality also increased. This study confirms that sizing agents such as polyvinyl alcohol (PVA), emulsifier, wax, carboxymethyl cellulose (CMC), and starch used in sizing mills are also responsible for respiratory illness and lung impairment among textile workers.

Fut2 Deficiency Promotes Intestinal Stem Cell Aging by Damaging Mitochondrial Functions via Down-Regulating α1,2-Fucosylation of Asah2 and Npc1.

Fut2-mediated α1,2-fucosylation is important for gut homeostasis, including the intestinal stem cell (ISC). The stemness of ISC declines with age, and aging-associated ISC dysfunction is closely related to many age-related intestinal diseases. We previously found intestinal epithelial dysfunction in some aged Fut2 knockout mice. However, how Fut2-mediated α1,2-fucosylation affects ISC aging is still unknown. On this basis, the herein study aims to investigate the role of Fut2-mediated α1,2-fucosylation in ISC aging. Aging models in ISC-specific Fut2 knockout mice were established. ISCs were isolated for proteomics and N-glycoproteomics analysis. ISC functions and mitochondrial functions were examined in mice and organoids. Ulex europaeus agglutinin I chromatography and site-directed mutagenesis were used to validate the key target fucosylated proteins of Fut2. As a result, Fut2 knockout impaired ISC stemness and promoted aging marker expression in aged mice. Proteomics analysis indicated mitochondrial dysfunction in Fut2 knockout ISC. More injured mitochondria, elevated levels of reactive oxygen species, and decreased levels of adenosine 5'-triphosphate (ATP) in Fut2 knockout ISC were found. Moreover, respiratory chain complex impairment and mitophagy dysfunction in Fut2 knockout ISC were further noted. Finally, Fut2 was demonstrated to regulate mitochondrial functions mainly by regulating the α1,2-fucosylation of N-acyl sphingosine amidohydrolase 2 (Asah2) and Niemann-Pick type C intracellular cholesterol transporter 1 (Npc1). In conclusion, this study demonstrated the substantial role of Fut2 in regulating ISC functions during aging by affecting mitochondrial function. These findings provide novel insights into the molecular mechanisms of ISC aging and therapeutic strategies for age-related intestinal diseases.

Paraseptal Emphysema in Indium Lung: Tracing the Pathological Footprints of Chronic Exposure.

Indium lung is an occupational lung disease caused by exposure to indium-tin-oxide (ITO) dust. Compared to other occupational lung diseases, indium lung has a shorter latency period and the respiratory status continues to worsen even after exposure to the work environment improves. Paraseptal emphysema which affects mainly the subpleural area is seen on chest images obtained via computed tomography (CT), regardless of the smoking history. However, the pathogenesis of emphysema in indium lung is still unclear. Therefore, we re-evaluated the pathology of three previously reported cases of indium lung. Paraseptal emphysema was observed in both smokers and nonsmokers. Obstructive respiratory impairment worsened over time in the cases with paraseptal emphysema. Many alveolar walls were destroyed independent of the presence or absence of emphysetamous changes or fibrosis. Moreover, bronchiolitis was found to be less common in indium lung than in asbestosis (the most common occupational lung disease) or common cases of chronic obstructive pulmonary disease caused by smoking. It has been shown that ITO causes protease anti-protease imbalance, oxidant-antioxidant imbalance, and continuous, abnormal inflammation (the three major causes of emphysema). In addition, nano-sized ITO is less likely to be trapped in the upper airways and may easily reach the subpleural alveoli. Furthermore, ITO may continue to cause sustained tissue injury at the alveolar level potentially resulting in emphysema. Further studies are needed to elucidate the detailed pathogenesis of indium lung by comparing it with other occupational lung diseases.

Clinical assessment of the severity of bronchopulmonary dysplasia in children born prematurely

Bronchopulmonary dysplasia (BPD) – chronic lung disease, which evolves with broncho-obstructive syndrome, signs of respiratory failure, pulmonary fibrosis. Aim. Analytical case-control study included 41 children with BPD and 40 preterm children without BPD, who were clinically evaluated. Methods. Evaluation of clinical respiratory syndromes and degree of severity of bronchopulmonary dysplasia in premature infants. Results. The study demonstrated that the O2 saturation determined by pulse oximetry in premature children with BPD is reduced - 94.5±0.4%, compared to the O2 saturation of premature children without BPD (97.85±0.14%), which is significantly higher (F stat = 66.7, T stat = 8.17, p&lt;0.0001). Children with mild grade BPD present intercostal retractions in 22.2%: 95%CI 2.8-60, in moderate grade BPD – 52.6%: 95%CI 28.9-75.6 and in severe grade DBP all the children had chest retractions. Depending on the degrees of severity, children with mild BPD present intercostal retractions in 22.2%, in moderate BPD – 73.7% and in severe BPD all children had intercostal retractions. Costal retractions, which is an indicator of respiratory insufficiency, was found in all preterm infants with severe BPD, in 52.6%: 95%CI 28.9-75.6 preterm infants with moderate BPD. Premature children with mild BPD did not present costal retractions at the stage of diagnosis confirmation (χ2 = 15.3; p&lt;0.001). Conclusions. According to the study, bronchopulmonary dysplasia in children born prematurely is manifested by signs of respiratory insufficiency - accelerated breathing, intercostal and thoracic retractions, low values of O2 saturation. The severity of signs of respiratory impairment is dependent on the degree of bronchopulmonary dysplasia, having a clinical expression through different forms of thoracic retractions.

Methods of Infanticide: A Review Article

Infanticide refers to the act of killing of infant under the age of 12 months. It is mostly associated with mental disturbance during and immediately after pregnancy. Infanticide can result either from the act of commission or omission. Violence can be used by application of a blunt force or hard object on the infant leading to fractures or damage to vital organs. Strangulation is cutting off the flow of air by constriction of the air passages. This can be done manually or using a cord that wrap the neck. Drowning is the process of submersion or immersion in a liquid medium leading to respiratory impairment. Exposing the infant into the extremes of temperature is also one of the methods used in infanticide. It eventually results in organ dysfunction such as cardiac and respiratory failure, leading to death. Smothering is a form of asphyxia which is caused by deliberate mechanical blocking of the nose and mouth using hands, pillows, or any other objects. It is one of the favorable methods as the absence of resistance from the weak infant hardly leave any injuries. Stabbing is also one of the rare methods used in infanticide. Defenestration is the act of throwing someone or something out of a window leading to severe polytrauma and damage to vital brain centers. Negligence, which is the deliberate withholding of the infant's normal care, is one of the common methods in which death results from dehydration and starvation.

Alteraciones pulmonares persistentes tras 18 meses de neumonía por SARS-CoV-2

ObjectiveTo describe persistent pulmonary abnormalities detected on HRCT after 18months of SARS-CoV-2 pneumonia, and to determine their extension and correlation with pulmonary function. Patients and methodsA prospective cross-sectional study with an initial cohort of 90 patients in follow-up due to persisting lung abnormalities on imaging, functional respiratory impairment and/or respiratory symptoms. Of these, 31 (34%) were selected for analysis due to the persistence of their lung abnormalities on HRCT at 18 months after infection. A double reading was performed for each HRCT (62 observations). ResultsOf the 31 patients included: 20 (65%) were men; mean age was 67years; 17 (55%) were smokers/ex-smokers. The mean hospitalisation time was 38days. Eighteen (58%) patients were admitted to intensive care units. Five patients (16%) suffered an acute pulmonary thromboembolism and three (9.7%) had a pneumothorax. The mean time between the onset of pneumonia and the follow-up HRCT was 20.34 months. Extension of total pulmonary abnormalities, ground-glass opacities and reticulation was 19%, 12% and 4.5% respectively. The findings of the 62 readings were: ground-glass opacities (100%), reticulation (83%), subpleural curvilinear lines (62%), parenchymal bands (34%), traction bronchiectasis (69%), displacement of vessels/fissures (46%) and honeycombing (4.9%). Pulmonary function 18months after the acute episode revealed a mean FVC of 92% of predicted value, with an FVC <80% of predicted value in 11 patients (35.4%). Mean DLCO was 71% of predicted value, with a DLCO <80% in 22 patients (70%). We observed a statistically significant relationship between total extension of abnormalities on HRCT and FVC (P<.05), and a trend towards statistical significance with DLCO (P=.051); there was a statistically significant relationship between the presence of ground-glass opacities and FEV1/FVC (P<.01). The relationships between reticulation and FVC, FVC%, FEV1, FEV1% and DLCO% were also considered statistically significant (P<.05). ConclusionPersistent interstitial lung abnormalities are seen on HRCT for a subset of patients infected with SARS-CoV-2 pneumonia. Seventy percent of these patients suffered a slight decrease in DLCO.

Hypoxia enhances human myoblast differentiation: involvement of HIF1α and impact of DUX4, the FSHD causal gene

BackgroundHypoxia is known to modify skeletal muscle biological functions and muscle regeneration. However, the mechanisms underlying the effects of hypoxia on human myoblast differentiation remain unclear. The hypoxic response pathway is of particular interest in patients with hereditary muscular dystrophies since many present respiratory impairment and muscle regeneration defects. For example, an altered hypoxia response characterizes the muscles of patients with facioscapulohumeral dystrophy (FSHD).MethodsWe examined the impact of hypoxia on the differentiation of human immortalized myoblasts (LHCN-M2) cultured in normoxia (PO2: 21%) or hypoxia (PO2: 1%). Cells were grown in proliferation (myoblasts) or differentiation medium for 2 (myocytes) or 4 days (myotubes). We evaluated proliferation rate by EdU incorporation, used myogenin-positive nuclei as a differentiation marker for myocytes, and determined the fusion index and myosin heavy chain-positive area in myotubes. The contribution of HIF1α was studied by gain (CoCl2) and loss (siRNAs) of function experiments. We further examined hypoxia in LHCN-M2-iDUX4 myoblasts with inducible expression of DUX4, the transcription factor underlying FSHD pathology.ResultsWe found that the hypoxic response did not impact myoblast proliferation but activated precocious myogenic differentiation and that HIF1α was critical for this process. Hypoxia also enhanced the late differentiation of human myocytes, but in an HIF1α-independent manner. Interestingly, the impact of hypoxia on muscle cell proliferation was influenced by dexamethasone. In the FSHD pathological context, DUX4 suppressed HIF1α-mediated precocious muscle differentiation.ConclusionHypoxia stimulates myogenic differentiation in healthy myoblasts, with HIF1α-dependent early steps. In FSHD, DUX4-HIF1α interplay indicates a novel mechanism by which DUX4 could interfere with HIF1α function in the myogenic program and therefore with FSHD muscle performance and regeneration.

A predictive analytics model using machine learning algorithms to estimate the risk of shock development among dengue patients

Dengue is a common viral disease in tropical and subtropical countries. The clinical manifestation of dengue has a wide spectrum, from asymptomatic seroconversion to severe dengue infection. Severe dengue is defined as dengue with the presence of specific symptoms, including severe plasma leakage leading to shock or the accumulation of fluids with respiratory distress, severe bleeding, and severe organ impairment. Examining the progression of shock with the integration of patients’ physiological information and biochemical parameters would help in understanding the progression of the disease and early detection of shock. In this study, physiological patient data diagnosed with dengue are collected from a University Malaya Medical Centre’s electronic record. A prediction model learned from the measurement of a patient’s physiological data is the basis for effective treatment and prevention of shock development in critically ill patients. Hence, this study presents the predictive performance of machine learning algorithms to estimate the risk of shock development among dengue patients. Logistic regression, decision trees, support vector machines and neural networks are evaluated. Lastly, ensemble learnings of bagging and boosting are also applied to the weak learner to optimize performance. The experimental results show that the bagging algorithm outperforms other competing methods with a 14.5% improvement from the individual decision tree. The full blood count (FBC) specifically haemoglobin (Hb) on day 2 is found to be a strong predictor for severe dengue occurrence.

The Effect of MitoTEMPO on Rat Diaphragm Muscle Contraction Parameters in an Experimental Diabetes Model Induced with Streptozotocin

Objective: Diabetes Mellitus (DM) not only causes hyperglycemia but also leads to clinical challenges involving respiratory functional impairments. The contraction of the diaphragm reduces pleural pressure, thereby contributing significantly to the process of breathing. This study examines the functional impairments in diaphragm muscle isometric contraction parameters due to increased reactive oxygen species (ROS) associated with DM, as well as the effects of MitoTEMPO, a mitochondria-specific antioxidant, on these impairments. Methods: Wistar Albino male rats at 12-14 weeks of age were randomly divided into three groups: the control group (CON, n=6), the diabetes group (DM, n=6), and the diabetes + MitoTEMPO (MT, n=6) group. A single dose of 50 mg/kg streptozotocin (STZ) was administered to the rats in the DM and MT groups. When the rats in the MT group reached a blood glucose level of 300 mg/dl, they were administered MitoTEMPO at a dose of 0.7 mg/kg/day for 28 days. Isometric contraction recordings were obtained from diaphragm muscle preparations isolated from the experimental animals at the end of the 28-day period. Results: Although the effectiveness of mitochondria-specific antioxidants in reducing blood glucose levels in DM is debated in the literature, results for the MT group were interestingly indicative of a statistically significant decrease in blood glucose levels following MitoTEMPO administration at the end of the fourth week. Furthermore, MitoTEMPO exhibited therapeutic effects on diaphragm muscle contraction parameters impaired by DM. Conclusion: The findings suggest that in DM patients, MitoTEMPO could be utilized for blood glucose control and might also be effective in the treatment of DM-induced diaphragm muscle mechanical dysfunction.

Tuberculosis and Risk of Emphysema among US Adults in the NHANES I Epidemiologic Follow-Up Study Cohort, 1971-1992.

(1) Background: History of TB is a known risk factor for long-term respiratory impairment affecting lung functions in both restrictive and obstructive lung disease. (2) Methods: We analyzed data from the NHANES I Epidemiologic Follow-up Study (NHEFS), a longitudinal study conducted on a noninstitutionalized adult US population aged 25-74 years. Approximately 93 percent of the original NHANES I cohort was successfully traced by the end of the survey period and was available for analysis. The final adjusted model included age groups, gender, family income, lifetime smoking, body mass index (BMI), and frequency of alcohol consumption as potential confounders. (3) Results: The estimated hazards ratio of developing emphysema during follow-up for individuals with a past diagnosis of TB was 54% lower (95% CI = 0.35, 0.61) that that in individuals with no past TB, after controlling for potential confounders and using proportional hazards regression appropriate to the complex sample design. The association, however, was not statistically significant (HR = 0.86, p-value = 0.38) when only a self-reported history of TB was considered as the exposure in an unadjusted model. (4) Conclusions: Tuberculosis (self-reported or LTBI) was strongly (but inversely) associated with emphysema incidence. The association was not statistically significant with only a self-reported history of TB as exposure.

Palatal prolapse during nasal expiration in patients with myasthenia gravis.

We have encountered patients with myasthenia gravis (MG) who exhibited palatal prolapse (PP) during nasal expiration in the supine position while awake. This may be an overlooked cause of dyspnea in MG patients. This study aimed to examine and describe the characteristics of MG patients with PP. We reviewed the medical records of 183 consecutive patients who were diagnosed with MG in our hospital from 2012 to 2021. Thirty-two patients underwent laryngoscopy because of bulbar symptoms. Eight of these patients (25%) exhibited PP on laryngoscopy. Clinical features of these eight patients were retrospectively characterized. Median age of the eight patients with PP was 70 years. Six were men. Median body mass index was 21.6 kg/m2 . All patients exhibited PP in the supine position but not the sitting position. Although no patient had abnormal findings on spirometry nor chest computed tomography, six reported dyspnea or difficulty with nasal expiration only in the supine position. PP improved in all four patients who underwent edrophonium testing. All eight patients eventually improved after immunotherapy. PP during nasal expiration may be a cause of dyspnea in MG patients, along with respiratory muscle impairment, lung disease, and vocal cord paralysis. Laryngoscopy in the supine position is required to confirm.

Brazilian Thoracic Association recommendations for the management of post-tuberculosis lung disease.

Historically, all efforts against tuberculosis were focused on rapid diagnosis and effective treatment to break the chain of transmission of Mycobacterium tuberculosis. However, in the last few years, more and more evidence has been found on the dramatic consequences of the condition defined as post-tuberculosis lung disease (PTLD). Approximately one third of patients surviving pulmonary tuberculosis face considerable ongoing morbidities, including respiratory impairment, psychosocial challenges, and reduced health-related quality of life after treatment completion. Given the important global and local burden of tuberculosis, as well as the estimated burden of PTLD, the development of a consensus document by a Brazilian scientific society-Sociedade Brasileira de Pneumologia e Tisiologia (SBPT)-was considered urgent for the prevention and management of this condition in order to allocate resources to and within tuberculosis services appropriately and serve as a guide for health care professionals. A team of eleven pulmonologists and one methodologist was created by the SBPT to review the current evidence on PTLD and develop recommendations adapted to the Brazilian context. The expert panel selected the topics on the basis of current evidence and international guidelines. During the first phase, three panel members drafted the recommendations, which were divided into three sections: definition and prevalence of PTLD, assessment of PTLD, and management of PTLD. In the second phase, all panel members reviewed, discussed, and revised the recommendations until a consensus was reached. The document was formally approved by the SBPT in a special session organized during the 2023 SBPT Annual Conference.

Autophagy-mediated NKG2D internalization impairs NK cell function and exacerbates radiation pneumonitis.

Radiation pneumonitis is a critical complication that constrains the use of radiation therapy for thoracic malignancies, leading to substantial morbidity via respiratory distress and lung function impairment. The role of Natural killer (NK) cells in inflammatory diseases is well-documented; however, their involvement in radiation pneumonitis is not fully understood. To explore the involvement of NK cells in radiation pneumonitis, we analyzed tissue samples for NK cell presence and function. The study utilized immunofluorescence staining, western blotting, and immunoprecipitation to investigate CXCL10 and ROS levels, autophagy activity, and NKG2D receptor dynamics in NK cells derived from patients and animal models subjected to radiation. In this study, we observed an augmented infiltration of NK cells in tissues affected by radiation pneumonitis, although their function was markedly diminished. In animal models, enhancing NK cell activity appeared to decelerate the disease progression. Concomitant with the disease course, there was a notable upsurge in CXCL10 and ROS levels. CXCL10 was found to facilitate NK cell migration through CXCR3 receptor activation. Furthermore, evidence of excessive autophagy in patient NK cells was linked to ROS accumulation, as indicated by immunofluorescence and Western blot analyses. The association between the NKG2D receptor and its adaptor proteins (AP2 subunits AP2A1 and AP2M1), LC3, and lysosomes was intensified after radiation exposure, as demonstrated by immunoprecipitation. This interaction led to NKG2D receptor endocytosis and subsequent lysosomal degradation. Our findings delineate a mechanism by which radiation-induced lung injury may suppress NK cell function through an autophagy-dependent pathway. The dysregulation observed suggests potential therapeutic targets; hence, modulating autophagy and enhancing NK cell activity could represent novel strategies for mitigating radiation pneumonitis.

Respiratory function in LAMA2-related muscular dystrophy and SELENON-related congenital myopathy, a 1.5-year natural history study

IntroductionLAMA2-related muscular dystrophy (LAMA2-MD) and SELENON(SEPN1)-related congenital myopathy (SELENON-RM) are rare neuromuscular diseases with respiratory impairment from a young age. Prospective natural history studies are needed for prevalence estimations, respiratory characterization, optimizing clinical care and selecting outcome measures for trial readiness. MethodsOur prospective 1.5-year natural history study included spirometry (forced vital capacity (FVC); difference between upright and supine vital capacity (dVC)), respiratory muscle strength tests (sniff nasal inspiratory pressure (SNIP)) (age≥5 years), and diaphragm ultrasound (thickness; thickening; echogenicity; all ages). ResultsTwenty-six LAMA2-MD patients (M = 8, median 21 [9; 31] years) and 11 SELENON-RM patients (M = 8, 20 [10; 33] years) were included. At baseline, 17 (85 %) LAMA2-MD (FVC%: 59 % [33; 68]) and all SELENON-RM patients (FVC%: 34 % [31; 46]) had an impaired respiratory function (FVC%<80 %). Nine (35 %) LAMA2-MD and eight (73 %) SELENON-RM patients received mechanical ventilation at baseline, and two additional SELENON-RM patients started during follow-up. Contrarily to LAMA2-MD, SELENON-RM patients had severe diaphragm atrophy (diaphragm thickness z-score: 2.5 [-3.1; −2.1]) and dysfunction (diaphragm thickness ratio: 1.2 [1.0; 1.7]; dVC: 30 % [7.7; 41]). SNIP was low in both neuromuscular diseases and correlated with motor function. In SELENON-RM, respiratory function decreased during follow-up. ConclusionThe majority of LAMA2-MD and all SELENON-RM patients had respiratory impairment. SELENON-RM patients showed lower respiratory function which was progressive, more prevalent mechanical ventilation, and more severe diaphragm atrophy and dysfunction than LAMA2-MD patients. Spirometry (FVC%, dVC) and respiratory muscle strength tests (SNIP) are useful in clinical care and as outcome measure in clinical trials. Clinical trial numberNCT04478981.

Diagnostic Potential of Oscillometry: A Population-based Approach.

Rationale: Respiratory resistance (Rrs) and reactance (Xrs) as measured by oscillometry and their intrabreath changes have emerged as sensitive parameters for detecting early pathological impairments during tidal breathing. Objectives: This study evaluates the prevalence and association of abnormal oscillometry parameters with respiratory symptoms and respiratory diseases in a general adult population. Methods: A total of 7,560 subjects in the Austrian LEAD (Lung, hEart, sociAl, boDy) Study with oscillometry measurements (computed with the Resmon Pro FULL; Restech Srl) were included in this study. The presence of respiratory symptoms and doctor-diagnosed respiratory diseases was assessed using an interview-based questionnaire. Rrs and Xrs at 5 Hz, their inspiratory and expiratory components, the area above the Xrs curve, and the presence of tidal expiratory flow limitation were analyzed. Normality ranges for oscillometry parameters were defined. Measurements and Main Results: The overall prevalence of abnormal oscillometry parameters was 20%. The incidence of abnormal oscillometry increased in the presence of symptoms or diagnoses: 17% (16-18%) versus 27% (25-29%), P < 0.0001. All abnormal oscillometry parameters except Rrs at 5 Hz were significantly associated with respiratory symptoms/diseases. Significant associations were found, even in subjects with normal spirometry, with abnormal oscillometry incidence rates increasing by 6% (4-8%; P < 0.0001) in subjects with symptoms or diagnoses. Conclusions: Abnormal oscillometry parameters are present in one-fifth of this adult population and are significantly associated with respiratory symptoms and disease. Our findings underscore the potential of oscillometry as a tool for detecting and evaluating respiratory impairments, even in individuals with normal spirometry.

Impact of diabetes mellitus on the respiratory function of amyotrophic lateral sclerosis patients.

Respiratory insufficiency and its complications are the main cause of death in amyotrophic lateral sclerosis (ALS). The impact of diabetes mellitus (DM) on respiratory function of ALS patients is uncertain. A retrospective cohort study was carried out. From the 1710 patients with motor neuron disease followed in our unit, ALS and progressive muscular atrophy patients were included. We recorded demographic characteristics, functional ALS rating scale (Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised [ALSFRS-R]) and its subscores at first visit, respiratory function tests, arterial blood gases, phrenic nerve amplitude (PhrenAmpl), and mean nocturnal oxygen saturation (SpO2 mean). We excluded patients with other relevant diseases. Two subgroups were analysed: DIAB (patients with DM) and noDIAB (patients without DM). Independent t-test, χ2 , or Fisher exact test was applied. Binomial logistic regression analyses assessed DM effects. Kaplan-Meier analysis assessed survival. p < 0.05 was considered significant. We included 1639 patients (922 men, mean onset age = 62.5 ± 12.6 years, mean disease duration = 18.1 ± 22.0 months). Mean survival was 43.3 ± 40.7 months. More men had DM (p = 0.021). Disease duration was similar between groups (p = 0.063). Time to noninvasive ventilation (NIV) was shorter in DIAB (p = 0.004); total survival was similar. No differences were seen for ALSFRS-R or its decay rate. At entry, DIAB patients were older (p < 0.001), with lower forced vital capacity (p = 0.001), arterial oxygen pressure (p = 0.01), PhrenAmpl (p < 0.001), and SpO2 mean (p = 0.014). ALS patients with DM had increased risk of respiratory impairment and should be closely monitored. Early NIV allowed for similar survival rate between groups.

Carnation Italian Ringspot Virus p36 Expression Induces Mitochondrial Fission and Respiratory Chain Complex Impairment in Yeast.

Positive-strand RNA virus replication invariably occurs in association with host cell membranes, which are induced to proliferate and rearrange to form vesicular structures where the virus replication complex is assembled. In particular, carnation Italian ringspot virus (CIRV) replication takes place on the mitochondrial outer membrane in plant and yeast cells. In this work, the model host Saccharomyces cerevisiae was used to investigate the effects of CIRV p36 expression on the mitochondrial structure and function through the determination of mitochondrial morphology, mitochondrial respiratory parameters, and respiratory chain complex activities in p36-expressing cells. CIRV p36 ectopic expression was shown to induce alterations in the mitochondrial network associated with a decrease in mitochondrial respiration and the activities of NADH-cyt c, succinate-cyt c (C II-III), and cytochrome c oxidase (C IV) complexes. Our results suggest that the decrease in respiratory complex activity could be due, at least in part, to alterations in mitochondrial dynamics. This yeast-based model will be a valuable tool for identifying molecular targets to develop new anti-viral strategies.

Robin Sequence Facial Profile After Conservative Treatment: A Long-Term Follow-Up.

Robin sequence (RS) is a congenital clinical condition characterized by micrognathia, glossoptosis, and respiratory distress. Conservative methods could be responsible for releasing feeding and respiratory impairment but little information about mandibular growth is known in long-term follow-up. Assessing the longitudinal behavior of the facial profile of individuals with isolated RS who underwent conservative micrognathia treatment using photographs during the whole craniofacial growth. Photographs of the right facial profile of 100 patients were used (50 individuals with isolated RS and 50 individuals without craniofacial anomaly). The individuals with RS were evaluated at 3 different times (T1: infant, T2: mixed dentition, T3: permanent dentition) by measuring the facial convexity angle (FCA; G.Sn.Pog´). A comparison between T3 and control group (C), individuals without craniofacial anomalies and in permanent dentition, was also performed, checking the FCA, nasolabial angle (Ls.Sn.Cm), mentolabial fold (Li.Si.Pog´), facial inferior third (Sn.Gn´.C) angles and the ratio between middle anterior facial height and lower anterior facial height. The T3 group showed an increased angle of facial convexity and increased facial inferior third angle and middle anterior facial height/lower anterior facial height ratio compared with the control group. In the longitudinal evaluation of individuals with isolated RS, significant differences were identified between T1 and T2 groups and T1 and T3 groups showing that the increased facial convexity was higher in the infants and that did not change significantly between the phases of mixed and permanent dentition. RS showed increased facial convexity in all phases evaluated, but their convexity decreased with growth. When compared with individuals without craniofacial anomalies, the individuals continue to exhibit retrognathism in the permanent dentition. The lack of a mandible projection has led to a considerable number of orthognathic surgeries for the correction of discrepancies.