Respiratory Center Research Articles

Constitutive Expression of Hif2α Confers Acute O2 Sensitivity to Carotid Body Glomus Cells.

Acute oxygen (O2) sensing and adaptation to hypoxia are essential for physiological homeostasis. The prototypical acute O2 sensing organ is the carotid body, which contains chemosensory glomus cells expressing O2-sensitive K+ channels. Inhibition of these channels during hypoxia leads to cell depolarization, transmitter release, and activation of afferent sensory fibers terminating in the brain stem respiratory and autonomic centers. Focusing on recent data, here we discuss the special sensitivity of glomus cell mitochondria to changes in O2 tension due to Hif2α-dependent expression of several atypical mitochondrial electron transport chain subunits and enzymes. These are responsible for an accelerated oxidative metabolism and the strict dependence of mitochondrial complex IV activity on O2 availability. We report that ablation of Epas1 (the gene coding Hif2α) causes a selective downregulation of the atypical mitochondrial genes and a strong inhibition of glomus cell acute responsiveness to hypoxia. Our observations indicate that Hif2α expression is required for the characteristic metabolic profile of glomus cells and provide a mechanistic explanation for the acute O2 regulation of breathing.

Экономическая эффективность длительной кислородотерапии у больных бронхолегочными заболеваниями с тяжелой хронической артериальной гипоксемией

Background: long-term oxygen therapy (LTOT) for chronic lung diseases associated with chronic hy-poxemia is effective in terms of increasing life expectancy. However, by now, the costs of LTOT are not reimbursed by the healthcare system of the Russian Federation. Aim: to evaluate the economic efficiency of respiratory therapy centers in providing pa-tients with chronic lung diseases and severe chronic hypoxemia with LTOT at home. Patients and Methods: direct medical and nonmedical costs associated with the introduc-tion of LTOT into clinical practice were identified and calculated. Costs of LTOT, outpatient management of patients without exacerbations, treatment for exacerbations, and disability-related payments were considered. Clinical and economic modeling was performed. A cost-effectiveness analysis was performed and the budget impact was assessed. Published data and the experience of St. Petersburg Regional Respiratory Support Center were used to evaluate LTOT efficacy. Results: additional costs for LTOT per patient for 5 years are 229,759 RUB. LTOT signif-icantly (by 74%) reduce the costs of treating patients with exacerbations of chronic obstructive pulmonary disease, mainly due to a reduction in exacerbation rate, including those requiring ad- mission to hospital. Analysis of budget impact over 5 years demonstrates a decrease in direct medical costs per 100 oxygen concentrators by 32% (from 181,666,728.90 RUB to 123,247,630.82 RUB). Given mortality, the estimated number of patients opting for LTOT using 100 oxygen concentrators over 5 years is 150. Conclusions: reimbursement for LTOT costs in Russian healthcare is economically reason-able. KEYWORDS: long-term oxygen therapy, economic efficiency, respiratory therapy center, cost-effectiveness analysis, chronic hypoxemia. FOR CITATION: Gomon Yu.M., Titova O.N., Kolbin A.S., Kuzubova N.A., Balykina Yu.E. Economic efficiency of long-term oxygen therapy in patients with chronic lung diseases and severe chronic hypoxemia. Russian Medical Inquiry. 2023;7(8):475–481 (in Russ.). DOI: 10.32364/2587-6821-2023-7-8-1.

Prevalence of tuberculosis/COVID-19 co-infection and factors associated with SARS-CoV-2 infection in pulmonary tuberculosis patients at a respiratory diseases center: a cross-sectional study.

currently, tuberculosis (TB) is the second cause of infectious disease-related deaths before COVID-19. These two infections have several similarities but little data is available on TB/COVID-19 co-infection, hence, we sought to investigate the prevalence of this co-infection and the factors associated with Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection in tuberculosis patients in a tuberculosis-endemic area. we conducted a prospective cross-sectional study from January to June 2022 at Respiratory Diseases Center in Douala, Cameroon by enrolling all consenting pulmonary tuberculosis patients. The presence of SARS-CoV-2 ribonucleic acid (RNA) and gamma-interferon levels were laboratory analyzed using the Reverse Transcriptase-Polymerase Chain Reaction and the enzyme-linked immunosorbent assay (ELISA) technique, respectively. The factors associated with COVID-19 carriage in pulmonary tuberculosis patients were analyzed by logistic regressions. overall, we enrolled 185 patients; 57.8% were males (sex ratio of 1.36) and their mean age was 43.70 ± 17.89 years. The prevalence of SARS-CoV-2 RNA in pulmonary TB patients was 24.3%. Asthma and sore throat were the factors favoring carriage (OR=3.74; 95% CI=1.271-11.017; p=0.017 and OR=4.05; 95%CI=1.204-13.600; p=0.024) and cough was a protective factor (OR=0.15; 95% CI = 0.034-0.690; p=0.015). the prevalence of SARS-CoV-2 carriage in tuberculosis patients is high and greater than the national prevalence. Asthma and sore throat would be associated factors.

Conceptual Study of Trimarma W. S. R. to Sirah Marma

The science of Marma (vital point), i.e., Marma Vijyaniyam is an extraordinary and dynamic part mentioned in classical texts of Ayurveda that has tremendous value while performing surgery. Marma is such an imperative and unique principle of Ayurveda. Trimarma is the basic organs of the body representing the three different systems – nervous, cardiovascular, and urinary system. Trimarma is gaining importance as “tripod of life” because they are more Sadyopranahara Marma than other Marma. Sirah Marma is considered first because trauma to the head causes disturbance in respiratory, circulatory, and temperature-regulating center in the brain area and may lead to unconsciousness and death. Charak stated that Prana is situated in Siras which is also called Uttamangam due to its control over all the Indriya (Sense). In this article, an effort is made to compile and analyze the fundamental concept of Trimarma.

Respiratory arrest after posterior fossa decompression in patients with Chiari malformations: An overview.

Chiari malformation type 1 (CM1) is a structural abnormality in the skull and cerebellum, causing cerebellar tonsils to shift downward. Decompression of the posterior fossa is a common surgical method to relieve symptoms and prevent neurological deterioration. After posterior fossa decompression (PFD), individuals with CM1 were more likely to have respiratory arrest. Here, we present, for the first time, a comprehensive overview of the potential risk factors and causes of respiratory arrest following PFD. A review of the literature highlighting the risk factors for postoperative respiratory arrest in CM1 patients was conducted in the databases of PubMed, Medline, and Google Scholar. Patients with syringomyelia and CM1 are at increased risk for respiratory arrest due to a number of factors, including impaired respiratory mechanics, central respiratory center dysfunction from edema or ischemia, intraoperative brain stem ischemia, and delayed gastric emptying from autonomic dysfunction. Occipitalization of the first cervical vertebra, basilar impression, and fusion of C2-C3 are all risk factors for respiratory arrest. Implications for CM1 patient care and prospects for further investigation of postoperative respiratory arrest's causes and risk factors were discussed.

DLCO and Spirometry in non-small cell lung cancer patients receiving EGFR-TKI in Indonesia

Abstract Introduction EGFR-TKI is the treatment of choice in non-small cell lung cancer (NSCLC) with EGFR mutation in exon 19 or 21. The influence of EGFR-TKI therapy on lung function is still unrecognizable in NSCLC patients. Objectives This prospective study aims to examine the influence of EGFR-TKI therapy on lung function of lung adenocarcinoma patients with a single mutation in EGFR exon 19 or 21 at Persahabatan National Respiratory Center Hospital Jakarta, Indonesia. The pulmonary function test (PFT), including spirometry and diffusing capacity (DLCO), was performed before and after three months of EGFR-TKI therapy. After three months, the patients were divided into groups based on the Response Evaluation Criteria in Solid Tumors (RECIST) criteria and the change of PFT was compared before and after therapy. Results Among twenty lung cancer patients enrolled, we found increase of predicted mean FVC value from 60.6% to 68.25% (p=0.03), mean predicted FEV1 value from 59.7% to 67.05% (p=0.036), mean DLCO from 11.55 ml/minute/mmHg to 13.72 ml/min/mmHg (p=0.004), and predicted DLCO from 53.4% to 63.85% (p=0.03). The increase of mean predicted DLCO was greatest in the partial response group, which was 16.43% (p=0.056). Conclusion This study found that the majority of NSCLC patients with single EGFR exon 19 or 21 mutation had significantly improved lung function after EGFR-TKI therapy. Lung function test might become a beneficial tool to evaluate the effectiveness of EGFR-TKI in NSCLC patients, especially in clinical setting where computerized tomography (CT) scan is not available.

N-acetylcysteine as adjuvant therapy for hospitalized Covid-19 patients: A single-center prospective cohort study.

Whilst over two years have passed since the COVID-19 pandemic's emergence, the proper management of the disease remains challenging. N-acetylcysteine (NAC) as a potentially effective therapeutic option has been suggested by studies, while the exact clinical role of this agent is yet to be evaluated. This prospective case-control study was conducted in a major referral respiratory center in Tehran, Iran. We enrolled 217 patients treated with an intravenous daily dose of 1500 mg NAC as a case group; and 245 control patients who did not receive NAC. Two groups were matched based on other treatments, socio-demographics, medical history, and comorbidities. After ten days of adjuvant therapy with NAC, patients in the NAC group and control group had median room-air SpO2 of 91% and 88%, respectively (P=0.02). Also, the SpO2 to FiO2 ratio had a median of 463 and 421 in the case and control groups, respectively (P=0.01). Furthermore, the case group's hospitalization period was three days shorter (P=0.002). Further, cough, dyspnea, and decreased appetite were reported to have a significantly lower incidence in the case group (P=0.03, 0.001, 0.008). We showed that a daily intravenous dose of NAC in hospitalized COVID-19 patients could shorten the hospital stay and improve some clinical symptoms; however, it does not remarkably improve the risk of ICU admission and the 28 days in-hospital mortality rate.

ЭВОЛЮЦИЯ ВЗГЛЯДОВ НА ФИЗИОЛОГИЮ ДЫХАНИЯ В НЕВЕСОМОСТИ

The author summarizes the results of own long-term investigations of the human respiratory system onboard space objects. These results were analyzed and compared to the data of other authors who also had studied the breathing system in microgravity. The paper describes the logic of space experiments arrangement, sequence, progressive accumulation of knowledge about breathing physiology and microgravity. Microgravity alters the structure of pulmonary volumes and respiratory rate and volume; it disturbs biomechanics of the respiratory muscles contraction that may expedite muscular fatigue and weaken the proprioceptrive inputs to the respiratory center. The thoracic-abdominal breathing ratio changes for abdominal. Microgravity affects the cosmonaut's ability to control respiratory movements intentionally and reduces sensitivity of the central respiratory mechanism to endogenous hypoxia and hypercapnia. Magnitude of these deviations as compared to the ground measurements is not critical for cosmonaut's health and performance. However, in complex they may aggravate the effect of hypoxemia in microgravity, as has been noted by several authors, and be the reason for degradation of physical performance in space mission and, particularly, after landing.

Telephone-Based Smoking Cessation Counseling Service: Satisfaction and Outcomes in Vietnamese Smokers

As a method to acknowledge the devastating health and economic impacts of tobacco usage worldwide, telephone-based tobacco cessation counseling services have emerged as a potential tool to aid people in their quitting process. This study explores the satisfaction of smokers who use the QUITLINE service and factors associated with their quit attempts and cessation. A cross-sectional survey of 110 participants was conducted from June to July 2016 at the Respiratory Center at Bach Mai Hospital, Hanoi, Vietnam. Multivariate logistic regression was used, and it was found that the percentage of people quitting smoking increased after using the service. In total, 65.5% of participants were completely satisfied with the counseling service. The mean score of staff/s capacity/responsiveness, motivation, and service convenience were 4.37 ± 0.78, 4.30 ± 0.81, and 4.27 ± 0.66, respectively. The smoking relapse rate was relatively high at 58.3%, which mainly resulted from cravings and busy work (26.2% and 14.3%, respectively). A higher satisfaction score in "Staffs' capacity and responsiveness" was negatively associated with "ever tried to quit smoking in consecutive 24 h" and actually quit smoking after receiving counseling. Meanwhile, a higher score in the "Motivation" domain was positively associated with both quit attempt indicators as well as actually quitting smoking after receiving counseling (OR = 9.48; 95%CI = 2.27; 39.57). These results suggest that it is crucial for decision makers to place more focus on countermeasures for smoking relapse and to strengthen the capacity of staff, especially in motivating clients. Interventions should also be maintained throughout a long period of time to prevent relapse.

Autologous Blood Patch Pleurodesis for Secondary Spontaneous Pneumothorax: A Narrative Review, a Retrospective Case Series and State of Play in the UK.

Treatment of prolonged air leak due to secondary spontaneous pneumothorax is challenging. Autologous blood patch pleurodesis (ABPP) is a treatment option. Previous evidence is reliant on single-centre series and underpowered trials and is mostly described in air leaks post cardiothoracic intervention. There are no United Kingdom (UK) wide data. Members of the UK Pleural Society were surveyed for their practice and for patients who underwent blood patch. There were 16 respondents from 333 members. Twelve had performed the procedure, and six had kept records and could submit data. Basic demographics, intervention and clinical details of patients were then collected. The study was sponsored by the Audit Department of Northumbria Healthcare NHS Foundation Trust (reference 8124), and Caldicott Clearance for data sharing was provided by the Trust's Information Goverance Board (reference C4221). There was no requirement for informed consent. Data for 12 patients that received ABPP between 2014 and 2022 in six respiratory centres were assessed. The aetiology of the secondary pneumothoraces was mostly due to chronic obstructive pulmonary disease and end-stage interstitial lung disease. The patients had a median age of 75years. The median air leak time before ABPP was 17days. A total of 50-100 ml of blood was used for ABPP. Five patients had two attempts at ABPP. Air leak resolved in six patients (50%). Four patients had pleural apposition prior to ABPP. Four patients were diagnosed with hospital-acquired pneumonia following ABPP. This is the only UK-wide retrospective case series of ABPP of 'medical' patients with secondary pneumothorax. There is widespread variation in care. No formal conclusions can be drawn, and much larger robust datasets are required. An application has been made to the European Respiratory Society to incorporate ABPP within the International Collaborative Effusion database.

Application of a Standardized Terminology and Nomenclature for Respiratory Cytology: Experience from a Large Tertiary Respiratory Cancer Centre

Introduction: In 2020, the World Health Organization-International Agency for Research on Cancer/International Academy of Cytology (WHO-IARC IAC) joint project was commenced to develop standardized nomenclature and diagnostic criteria in cytopathology internationally. Our institution has been coding all respiratory cytological specimens in a similar fashion for over 10 years. Our aim was to analyse the effectiveness of our respiratory cytology coding system by calculating the estimated risk of malignancy (ROM) and rates of each diagnostic category. Methods: Over a 2 year period, all endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA), bronchial brushing, bronchial washing, bronchial lavage, and sputum specimens reported at our institution were analysed. For each specimen, the diagnostic code, the relevant indication for each diagnostic procedure, the diagnosis, and the presence or absence of a positive corresponding biopsy were recorded. Results: In total, 1,432 respiratory cytological specimens from 945 patients over a 2-year period were analysed. 467 specimens were confirmed to be associated with a malignant process. The overall ROM for respiratory cytology specimens was 37.7% for nondiagnostic, 18.1% for benign, 46.7% for atypical, 85.7% for suspicious for malignancy, and 91.9% for malignant. For each diagnostic procedure, the ROM increased from the benign to malignant categories. Discussion/Conclusion: Our ROM rates for overall respiratory cytology specimens and for EBUS-TBNA, bronchial brushing, and bronchial washing specimens separately are concordant with other major international studies. With the WHO-IARC IAC joint project in progress and an international respiratory cytology coding system being developed, our study has the potential to add value by providing indicative ROM rates, which can be used to inform the development of this new classification system. Our rates of diagnostic accuracy are in keeping with international standards, which support the accuracy of our data.

The gut-to-brain axis for toxin-induced defensive responses

After ingestion of toxin-contaminated food, the brain initiates a series of defensive responses (e.g., nausea, retching, and vomiting). How the brain detects ingested toxin and coordinates diverse defensive responses remains poorly understood. Here, we developed a mouse-based paradigm to study defensive responses induced by bacterial toxins. Using this paradigm, we identified a set of molecularly defined gut-to-brain and brain circuits that jointly mediate toxin-induced defensive responses. The gut-to-brain circuit consists of a subset of Htr3a+ vagal sensory neurons that transmit toxin-related signals from intestinal enterochromaffin cells to Tac1+ neurons in the dorsal vagal complex (DVC). Tac1+ DVC neurons drive retching-like behavior and conditioned flavor avoidance via divergent projections to the rostral ventral respiratory group and lateral parabrachial nucleus, respectively. Manipulating these circuits also interferes with defensive responses induced by the chemotherapeutic drug doxorubicin. These results suggest that food poisoning and chemotherapy recruit similar circuit modules to initiate defensive responses.

Validation Criteria for PETCO2 Kinetics during the Hyperventilation Provocation Test in the Diagnosis of Idiopathic Hyperventilation Syndrome.

Background: The hyperventilation provocation test (HPTest) is a diagnostic tool for idiopathic hyperventilation syndrome (HVS), encountered in some long-COVID patients. However, interpretation of the HPTest remains unclear regarding the relevant PETCO2 values to focus on and whether subjective symptoms should be considered. This study aimed to re-evaluate objective HPTest results for diagnosing HVS by determining accurate PETCO2 kinetics in two groups of patients previously screened via the Nijmegen questionnaire (NQ). Methods: The kinetics of PETCO2 during the HPTest were mathematically modeled and compared between 37 HVS patients (NQ ≥23/64) and 37 healthy controls (NQ <23/64) matched for gender, age, and body dimensions. AUC values with sensitivity and specificity were calculated, and analysis was monitored in a validation cohort of 152 routine HPTests. Results: A threshold value of a less than 12.8 mmHg increment of PETCO2 at the 5th minute of the recovery phase of the HPTest diagnosed HVS patients with excellent sensitivity (0.92) and specificity (0.84). These results were confirmed in the validation cohort, highlighting the presence of 24% false positives/negatives when diagnosing on the basis of complaints in the NQ. Conclusions: For HVS diagnosis, we suggest considering the HPTest, which can more reliably reflect the mechanisms of CO2 homeostasis and the response of the respiratory center to a stimulus, regardless of the subjective onset of symptoms.

Electrocution- post-mortem presentations, problems and pitfalls.

Deaths due to electrocution occur when there has been passage of an electric current through the body. Lethal mechanisms may involve the heart with ventricular fibrillation, or the respiratory muscles or brainstem respiratory centres with respiratory paralysis/cessation. The size of the current that flows through the body is directly dependent on the potential difference of the circuit and the resistance of the tissues. The majority of deaths are accidental, with suicides being less common and homicides being very rare. Problems arising in the evaluation of cases include situations where electrocution has clearly occurred from the scene findings, but no injuries are detectable at autopsy, or when electrical burns are found at autopsy with no defective equipment/circuitry identified at the death scene.

Clinicodemographic profile and outcome of tuberculosis treatment in TB-HIV co-infected patients receiving daily ATT under a single window TB/HIV services delivery initiative.

The risk of death in HIV-TB coinfected individuals is far greater than in HIV-only patients. It is critical to provide timely and appropriate therapy in HIV-TB coinfected patients in order to reduce morbidity and mortality. The purpose of this study was to evaluate the clinical presentation and outcome of TB treatment in HIV-TB co-infected patients receiving daily anti-tubercular therapy (ATT) and concurrent antiretroviral therapy (ART) at a tertiary respiratory care centre in New Delhi, India. The research was cross-sectional, observational, and hospital-based A. From September 2018 to August 2019, a total of 53 patients with HIV-TB coinfection were enrolled at the Institute's ART centre. Patients were evaluated with a structured proforma. Data were evaluated using SPSS version 23.0 and p-value of less than 0.05 was considered statistically significant. Among the patients enrolled, the mean age was 35.98 years. Among the patients enrolled, 56.6% patients had EPTB, 32% had PTB and 11.3% had both PTB and EPTB. The majority of the enrolled patients (n=46, 86.7%) had favourable TB treatment outcomes, while 13.3% (n=7) had unfavourable outcome [including death (n=5) and loss to follow up (n=2)]. During the study and follow-up period, no patients transferred out or relapsed. In univariate analysis, low SES, bedridden functional status, low BMI, anaemia, hypoalbuminemia, and a low CD-4 cell count (<100 cells/mm3 were significantly associated with an unfavourable outcome. Bedridden functional status (p=0.002), anaemia (p=0.040), and low BMI (p<0.001) were independently associated with a poor outcome. Adequate disease knowledge and health education can be very beneficial in reducing morbidity and mortality. Early ART in combination with ATT can reduce mortality in TB-HIV co-infected patients.

Effect of pharmacological inhibition of the pontine respiratory group on swallowing interneurons in the dorsal medulla oblongata

ObjectivesTo examine the role of neurons of the pontine respiratory group (PRG) overlapping with the Kölliker-Fuse nucleus in the regulation of swallowing, we compared the activity of swallowing motor activities and interneuron discharge in the dorsal swallowing group in the medulla before and after pharmacological inhibition of the PRG. MethodsIn 23 in situ perfused brainstem preparation of rats, we recorded the activities of the vagus (VNA), hypoglossal (HNA), and phrenic nerves (PNA), and swallowing interneurons of the dorsal medulla during fictive swallowing elicited by electrical stimulation of the superior laryngeal nerve or oral water injection. Subsequently, respiratory- and swallow-related motor activities and single unit cell discharge were assessed before and after local microinjection of the GABA-receptor agonist muscimol into the area of PRG ipsilateral to the recording sites of swallowing interneurons. ResultsAfter muscimol injection, the amplitude and duration of swallow-related VNA bursts decreased to 71.3 ± 2.84 and 68.1 ± 2.76 % during electrically induced swallowing and VNA interburst intervals during repetitive swallowing decreased. Similar effects were observed for swallowing-related HNA. The swallowing motor activity was similarly qualitatively altered during physiologically induced swallowing. All 23 neurons were changed in either discharge duration or frequency after PRG inhibition, however, the general discharge patterns in relation to the motor output remained unchanged. ConclusionDescending synaptic inputs from PRG provide control of the primary laryngeal sensory gate and synaptic activity of the PRG partially determine medullary cell and cranial motor nerve activities that govern the pharyngeal stage of swallowing.

FMRI studies evaluating central respiratory control in humans.

A plethora of neural centers in the central nervous system control the fundamental respiratory pattern. This control is ensured by neurons that act as pacemakers, modulating activity through chemical control driven by changes in the O2/CO2 balance. Most of the respiratory neural centers are located in the brainstem, but difficult to localize on magnetic resonance imaging (MRI) due to their small size, lack of visually-detectable borders with neighboring areas, and significant physiological noise hampering detection of its activity with functional MRI (fMRI). Yet, several approaches make it possible to study the normal response to different abnormal stimuli or conditions such as CO2 inhalation, induced hypercapnia, volitional apnea, induced hypoxia etc. This review provides a comprehensive overview of the majority of available studies on central respiratory control in humans.

Progress of Muscle Chain Theory in Shoulder Pain Rehabilitation: Potential Ideas for Pulmonary Rehabilitation.

Pulmonary dysfunction is very common in stroke patients. A study has shown that acute stroke patients often cause a series of pulmonary dysfunction due to primary damage to the respiratory center, which is an important reason for hindering disease treatment and recovery. American Thoracic Society (ATS) and the European Respiratory Society (ERS) pointed out that pulmonary rehabilitation (PR) can be applied to the rehabilitation of stroke patients to improve their lung function. PR can improve the respiratory muscle strength of stroke patients, which is beneficial to improving the respiratory function of patients. At the same time, it can also significantly increase the maximum oxygen intake of patients, effectively improve the cardiopulmonary function of stroke patients, and reduce respiratory complications such as aspiration pneumonia. However, the common dysfunction of joints and muscles such as shoulder pain after stroke will affect the process of pulmonary rehabilitation. This is mainly because the changes in the position of the shoulder girdle, the decrease in the range of motion of the cervical and thoracic spine, and the changes in the cervical spondylolisthesis position caused by the elevation of the upper limbs will directly affect the breathing movement during the pulmonary rehabilitation process. The instability of the spine will weaken the deep abdominal muscles and reduce the function of the diaphragm; moreover, changes in the alignment and stability of the cervical and thoracic spine will also lead to wrong breathing methods. Therefore, it is of practical clinical significance to evaluate the functional rehabilitation of shoulder joint muscles and evaluate the efficacy of stroke patients to improve their respiratory function. This article through an extensive review of domestic and foreign literature in recent years, combined with clinical practice experience, summarizes the practical application of chain structure theory in the fields of rehabilitation training, postural adjustment, pain relief, etc., and further studies the functional exercise method based on muscle chain theory. The research on the muscle chain of shoulder pain rehabilitation as a model illustrates the positive effect of reconstructing neuroarticular muscle function on the respiratory system, hoping to provide new ideas for the treatment of respiratory diseases in stroke patients.

Case Report: Considerations of nocturnal ventilator support in ROHHAD syndrome in chronic care of childhood central hypoventilation with hypothalamus dysfunction

Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) is a rare life-threatening disorder that can occur during childhood. All children with ROHHAD develop alveolar hypoventilation during wakefulness and sleep. The key treatment for these patients is the optimization of oxygenation and ventilation. Here, we report the case of a 5-year-old girl with suspected ROHHAD, with rapid weight gain, breathing cessation, decreased height, hypoventilation, central hypothyroidism, hyperprolactinemia, and absolute deficiency of growth hormone, and negative PHOX2B sequencing results. The presentation met the diagnostic criteria for ROHHAD syndrome. During the 5-year follow-up, she presented with progressive deterioration of the function of the hypothalamus and respiratory center, hypoxemia (PO2 < 60 mmHg), and hypercapnia [transcutaneous carbon dioxide (TcPCO2) > 70 mmHg] during the first two cycles of N3 sleep with a poor response to ventilatory support. Early diagnosis and application of non-invasive positive pressure ventilation during sleep can improve the quality of life and outcomes of patients with ROHHAD, and polysomnography and TcPCO2 should be repeated every 3–6 months to follow the progress and regulate ventilator support. Multidisciplinary care is crucial for the successful management of these patients.

Sleep and Breathing Disturbances in Children With Leigh Syndrome: A Comparative Study

BackgroundLeigh syndrome (LS) is a progressive neurodegenerative mitochondrial disease characterized by necrotizing lesions affecting different parts of the central nervous system, especially in the brainstem and basal ganglia. Lesions in this area may involve respiratory and sleep centers, resulting in the clinically significant disturbances seen—but poorly characterized—in LS. The purpose of the present study is to characterize and compare the physiologic responses to respiratory disturbances quantified by polysomnography metrics of children with LS with age-sex- and apnea-hypopnea index (AHI)-matched patients with obstructive sleep apnea (OSA), a common clinical population with similar burden of sleep-disordered breathing. MethodsRetrospective comparative study of polysomnographic data from six patients with LS were reviewed and compared with 18 age-sex-AHI-matched patients with OSA, with particular attention to cardiorespiratory and sleep architecture metrics. ResultsSleep architecture and stage duration were conserved in LS and OSA groups, but increased wake after sleep onset was seen among the first group. The LS group exhibited both obstructive and central sleep apnea. The group also had significantly greater values of heart rate, ≥3% oxygen desaturation index, and lower values of sleep efficiency, respiratory arousal index, and total sleep time when compared with the OSA group. ConclusionsPatients with LS exhibited significantly more sleep-related cardiorespiratory disturbances and sleep fragmentation when compared with neurotypical children with OSA. Given that these findings are plausibly detrimental to health and development, sleep evaluation in patients with similar conditions should be encouraged for early management.