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Hypoventilation

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https://www.readbyqxmd.com/read/28716137/progressive-hypoventilation-due-to-mixed-cd8-and-cd4-lymphocytic-polymyositis-following-tremelimumab-durvalumab-treatment
#1
Sooraj John, Scott J Antonia, Trevor A Rose, Robert P Seifert, Barbara A Centeno, Aaron S Wagner, Ben C Creelan
BACKGROUND: The combination of CTLA-4 and PD-L1 inhibitors has a manageable adverse effect profile, although rare immune-related adverse events (irAE) can occur. CASE PRESENTATION: We describe an autoimmune polymyositis following a partial response to combination tremelimumab and durvalumab for the treatment of recurrent lung adenocarcinoma. Radiography revealed significant reduction in all metastases; however, the patient developed progressive neuromuscular hypoventilation due to lymphocytic destruction of the diaphragmatic musculature...
July 18, 2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28708033/neuropsychological-profile-and-social-cognition-in-congenital-central-hypoventilation-syndrome-cchs-correlation-with-neuroimaging-in-a-clinical-case
#2
Borja Esteso Orduña, Raquel Seijas Gómez, Elena García Esparza, Emily M Briceño, Javier Melero Llorente, María de la Concepción Fournier Del Castillo
INTRODUCTION: Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder due to paired-like homeobox gene (PHOX2B) mutations. CCHS patients suffer from dysregulation of the autonomic nervous system characterized by the absence of or extremely reduced response to hypercapnia and hypoxia, with neuropsychological deficits. The aim of this exploratory study is to describe the longitudinal neuropsychological profile and its correlations with magnetic resonance imaging (MRI) of a child with CCHS with a PHOX2B mutation...
July 14, 2017: Journal of Clinical and Experimental Neuropsychology
https://www.readbyqxmd.com/read/28702830/sleep-disordered-breathing-in-patients-with-neuromuscular-disease
#3
REVIEW
Mohamad Ammar Albdewi, Giuseppe Liistro, Riëm El Tahry
Sleep-disordered breathing (SDB) is relatively common in general population as well as in patients with neuromuscular disease. SDB comprises a wide spectrum of disorders varying from simple snoring to complete closure of the upper airway as seen in obstructive sleep apnoea (OSA). It includes also other disorders like prolonged hypoxemia, hypoventilation, and central sleep apnoea (CSA). Neuromuscular diseases (NMD) form a group of disorders that can cause significant reduction in the quality and span of life...
July 12, 2017: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/28701502/wrist-sensor-reveals-sympathetic-hyperactivity-and-hypoventilation-before-probable-sudep
#4
Rosalind W Picard, Matteo Migliorini, Chiara Caborni, Francesco Onorati, Giulia Regalia, Daniel Friedman, Orrin Devinsky
No abstract text is available yet for this article.
July 12, 2017: Neurology
https://www.readbyqxmd.com/read/28694199/initiation-of-non-invasive-ventilation-for-sleep-related-hypoventilation-disorders-advanced-modes-and-devices
#5
REVIEW
Bernardo J Selim, Lisa Wolfe, John M Coleman, Naresh Dewan
Although non-invasive ventilation (NIV) has been used since the 1960's in the polio epidemic, the development of modern bilevel PAP devices did not become a reality until the 1990's. Over the last 25 years bilevel PAP technology options have exponentially increased. The number of patients receiving this treatment both in the acute setting as well as at home is steadily growing. However, a knowledge gap exists in the way these device settings are adjusted to achieve synchrony and match patient's unique respiratory failure physiology...
July 7, 2017: Chest
https://www.readbyqxmd.com/read/28690533/dysphagia-in-perry-syndrome-pharyngeal-pressure-in-two-cases
#6
George Umemoto, Yoshio Tsuboi, Hirokazu Furuya, Takayasu Mishima, Shinsuke Fujioka, Naoki Fujii, Hajime Arahata, Miwa Sugahara, Mitsuaki Sakai
BACKGROUND: To investigate the impact of dysphagia in Perry syndrome (PS), an autosomal dominant parkinsonism caused by mutation of DCTN1, which is associated with hypoventilation, depression, and weight loss. CASE PRESENTATION: We used tongue pressure measurements and manofluorography to investigate swallowing function in 2 patients with PS. Case 1, a 60-year-old male showing parkinsonism, and case 2, a 49-year-old male admitted with pneumonia, were diagnosed as having PS based on the DCTN1 gene analysis...
May 2017: Case Reports in Neurology
https://www.readbyqxmd.com/read/28690393/effect-on-lung-function-of-mounthpiece-ventilation-in-steinert-disease-a-case-report
#7
Anna Annunziata, Giuseppe Fiorentino, Antonio Esquinas
In patients with muscular dystrophies both muscle length tension relationship changes and muscle elasticity and plasticity are decreased, resulting in impaired inspiratory muscle function and decreased vital capacity. Furthermore, the loss of deep breathing further increases the risk of alveolar collapse, hypoventilation and atelectasias. In this case report, a stable improvement of vital capacity after treatment with mounthpiece ventilation (MPV), was observed, suggesting that not invasive ventilation (NIV) might help to maintai lung and chest wall compliance, prevent hypoventilation and atelectasias which in turn may slow down the development of the restrictive respiratory pattern...
March 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/28682836/a-technique-of-awake-bronchoscopic-endotracheal-intubation-for-respiratory-failure-in-patients-with-right-heart-failure-and-pulmonary-hypertension
#8
Jimmy Johannes, David A Berlin, Parimal Patel, Edward J Schenck, Frances Mae West, Rajan Saggar, Igor Z Barjaktarevic
OBJECTIVE: Patients with pulmonary hypertension and right heart failure have a high risk of clinical deterioration and death during or soon after endotracheal intubation. The effects of sedation, hypoxia, hypoventilation, and changes in intrathoracic pressure can lead to severe hemodynamic instability. In search for safer approach to endotracheal intubation in this cohort of patients, we evaluate the safety and feasibility of an alternative intubation technique. DATA SOURCES: Retrospective data analysis...
July 4, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28673876/abnormal-respiration-under-hyperoxia-in-task-1-3-potassium-channel-double-knockout-mice
#9
Philipp K Buehler, Doris Bleiler, Ines Tegtmeier, Dirk Heitzmann, Christian Both, Michael Georgieff, Florian Lesage, Richard Warth, Jörg Thomas
Despite intensive research, the exact function of TASK potassium channels in central and peripheral chemoreception is still under debate. In this study, we investigated the respiration of unrestrained TASK-3 (TASK-3(-/-)) and TASK-1/TASK-3 double knockout (TASK-1/3(-/-)) adult male mice in vivo using a plethysmographic device. Ventilation parameters of TASK-3(-/-) mice were normal under control condition (21% O2) and upon hypoxia and hypercapnia they displayed the physiological increase of ventilation. TASK-1/3(-/-) mice showed increased ventilation under control conditions...
July 1, 2017: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/28665356/sleep-apnea-and-hypoventilation-in-patients-with-down-syndrome-analysis-of-144-polysomnogram-studies
#10
Zheng Fan, Mihye Ahn, Heidi L Roth, Leping Li, Bradley V Vaughn
Patients with Down syndrome (DS) are at risk for both obstructive sleep apnea (OSA) and central sleep apnea (CSA); however, it is unclear how these components evolve as patients age and whether patients are also at risk for hypoventilation. A retrospective review of 144 diagnostic polysomnograms (PSG) in a tertiary care facility over 10 years was conducted. Descriptive data and exploratory correlation analyses were performed. Sleep disordered breathing was common (seen in 78% of patients) with an average apnea-hypopnea index (AHI) = 10...
June 30, 2017: Children
https://www.readbyqxmd.com/read/28664015/central-hypoventilation-syndrome-complicated-with-lateral-medullary-infarction-after-endovascular-treatment-of-the-vertebral-artery-dissecting-aneurysm-a-case-report
#11
Katsuhiro Tanaka, Hideki Kanamaru, Atsunori Morikawa, Kenji Kawaguchi
Lateral medullary infarction rarely leads to central hypoventilation syndrome (CHS). CHS is a life-threatening disorder characterized by hypoventilation during sleep. We report the first case of CHS as a complication of lateral medullary infarction after endovascular treatment. A 65-year-old man presented twice with severe headache. Computed tomography revealed subarachnoid hemorrhage and cerebral angiography showed a right vertebral dissecting aneurysm involving the posterior inferior cerebellar artery. After emergent endovascular patent artery occlusion, he developed Wallenberg syndrome and experienced apnea and a conscious disturbance episode due to CHS on postoperative days 6 and 16...
October 2016: NMC Case Rep J
https://www.readbyqxmd.com/read/28651750/reduced-orexin-immunoreactivity-in-perry-syndrome-and-multiple-system-atrophy
#12
Takayasu Mishima, Koji Kasanuki, Shunsuke Koga, Monica Castanedes-Casey, Zbigniew K Wszolek, Yoshio Tsuboi, Dennis W Dickson
INTRODUCTION: Orexin is a neuropeptide that plays a key role in maintaining a state of arousal, and possibly associates with sleep apnea syndrome (SAS). Reduced orexin immunoreactivity has been reported in various neurologic conditions such as narcolepsy, Alzheimer's disease, Lewy body disease and multiple system atrophy (MSA); however, there has been no report investigating orexin in Perry syndrome, a rare hereditary neurodegenerative disease characterized by four clinical cardinal signs (parkinsonism, depression/apathy, weight loss, and central hypoventilation)...
June 12, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28644040/hypoxic-pulmonary-vasoconstriction-does-not-explain-all-regional-perfusion-redistribution-in-asthma
#13
Vanessa J Kelly, Kathryn A Hibbert, Puja Kohli, Mamary Kone, Elliot E Greenblatt, Jose G Venegas, Tilo Winkler, R Scott Harris
RATIONALE: Regional hypoventilation in bronchoconstricted asthmatics is spatially associated with reduced perfusion, which is proposed to result from Hypoxic Pulmonary Vasoconstriction (HPV). Our study aimed to determine the role of HPV in the regional perfusion redistribution in bronchoconstricted asthmatics. METHODS: Eight asthmatics completed PET-CT lung imaging at baseline and after bronchoconstriction, breathing either Room Air or 80% Oxygen (80%O2, separate days)...
June 23, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28633714/three-generation-family-with-congenital-central-hypoventilation-syndrome-and-novel-phox2b-gene-non-polyalanine-repeat-mutation
#14
Ajay S Kasi, Taryn J Jurgensen, Stephanie Yen, Sheila S Kun, Thomas G Keens, Iris A Perez
PHOX2B non-polyalanine repeat mutation (NPARM) in patients with congenital central hypoventilation syndrome (CCHS) is generally considered to be associated with full-time ventilator dependence and severe autonomic nervous system dysfunction. We report a three-generation family with four individuals possessing a novel PHOX2B NPARM (c.245C > T) with variable phenotypes. This mutation was inherited in an autosomal dominant pattern with variable penetrance. The affected family members with CCHS have a milder phenotype than is typically expected with a NPARM...
July 15, 2017: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://www.readbyqxmd.com/read/28632754/permissive-hypercapnia-for-severe-acute-respiratory-distress-syndrome-in-immunocompromised-children-a-single-center-experience
#15
Hans Fuchs, Nicola Rossmann, Manuel B Schmid, Manfred Hoenig, Ulrich Thome, Benjamin Mayer, Daniel Klotz, Helmut D Hummler
BACKGROUND: Controlled hypoventilation while accepting hypercapnia has been advocated to reduce ventilator-induced lung injury. The aim of the study was to analyze outcomes of a cohort of immunocompromised children with acute respiratory distress syndrome (ARDS) ventilated with a strategy of stepwise increasing PCO2 targets up to 140 mm Hg. METHODS: Retrospective analysis of outcomes of a cohort of children with oncologic disease or after stem cell transplantation and severe respiratory failure in comparison with a historical control cohort...
2017: PloS One
https://www.readbyqxmd.com/read/28625595/dctn1-related-neurodegeneration-perry-syndrome-and-beyond
#16
REVIEW
Takuya Konno, Owen A Ross, Hélio A G Teive, Jarosław Sławek, Dennis W Dickson, Zbigniew K Wszolek
Perry syndrome (PS) is a rare hereditary neurodegenerative disease characterized by autosomal dominant parkinsonism, psychiatric symptoms, weight loss, central hypoventilation, and distinct TDP-43 pathology. The mutated causative gene for PS is DCTN1, which encodes the dynactin subunit p150(Glued). Dynactin is a motor protein involved in axonal transport; the p150(Glued) subunit has a critical role in the overall function. Since the discovery of DCTN1 in PS, it has been increasingly recognized that DCTN1 mutations can exhibit more diverse phenotypes than previously thought...
June 12, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28622152/use-of-transnasal-humidified-rapid-insufflation-ventilatory-exchange-for-emergent-surgical-tracheostomy-a-case-report
#17
Neel Desai, Anna Fowler
Transnasal humidified rapid-insufflation ventilatory exchange (THRIVE) is a novel airway technique that utilizes high-flow humidified nasal oxygen. It can extend apnea time and maintain oxygen saturation. Here we report the use of THRIVE in a 35-year-old man who required emergent surgical tracheostomy for a clinically relevant compromised airway secondary to acute supraglottic and glottic pathology. Intravenous sedation resulted in hypoventilation close to apnea. THRIVE maintained oxygen saturation for 40 minutes until transient desaturation developed after complete airway obstruction...
June 15, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28613919/hypercapnia-accelerates-adipogenesis-a-novel-role-of-high-co2-in-exacerbating-obesity
#18
Ryota Kikuchi, Takao Tsuji, Osamu Watanabe, Kazuhiro Yamaguchi, Kinya Furukawa, Hiroyuki Nakamura, Kazutetsu Aoshiba
Obesity is a major risk factor for the development of obstructive sleep apnea (OSA) and obesity hypoventilation syndrome (OHS), which manifest as intermittent hypercapnia and sustained plus intermittent hypercapnia, respectively. In this study, we investigated whether CO2 affects adipocyte differentiation (adipogenesis) and maturation (hypertrophy). Human visceral or subcutaneous preadipocytes were grown to confluence and then induced to differentiate to adipocytes under hypocapnia, normocapnia, and hypercapnia with or without hypoxia...
June 14, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/28599619/a-devastating-case-of-diarrhea-associated-hemolytic-uremic-syndrome-associated-with-extensive-cerebral-infarction-why-we-need-to-do-better
#19
Werner Keenswijk, Evelyn Dhont, Ann Raes, An Bael, Johan Vande Walle
A 4-year-old girl with diarrhea-associated hemolytic uremic syndrome (D+HUS) was transferred to the PICU of our center due to deteriorating renal function and neurological involvement. On admission, a comatous child was seen with hypoventilation and she was placed on mechanical ventilation. Hemodialysis was commenced but plasma exchange was discontinued due to repeated hypersensitivity reactions. A trial of eculizumab was given in light of the worsening of her neurologic condition with development of a pyramidal syndrome and deepening of the coma...
June 9, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28574856/camera-based-measurement-of-respiratory-rates-is-reliable
#20
Christoph Becker, Stefan Achermann, Mukul Rocque, Ihor Kirenko, Andreas Schlack, Thomas Dreher-Hummel, Thomas Zumbrunn, Roland Bingisser, Christian H Nickel
OBJECTIVES: Respiratory rate (RR) is one of the most important vital signs used to detect whether a patient is in critical condition. It is part of many risk scores and its measurement is essential for triage of patients in emergency departments. It is often not recorded as measurement is cumbersome and time-consuming. We intended to evaluate the accuracy of camera-based measurements as an alternative measurement to the current practice of manual counting. MATERIALS AND METHODS: We monitored the RR of healthy male volunteers with a camera-based prototype application and simultaneously by manual counting and by capnography, which was considered the gold standard...
June 1, 2017: European Journal of Emergency Medicine: Official Journal of the European Society for Emergency Medicine
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