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https://www.readbyqxmd.com/read/29779603/diaphragm-pacing
#1
REVIEW
Anthony F DiMarco
Diaphragm pacing (DP) is a useful and cost-effective alternative to mechanical ventilation in patients with ventilator-dependent spinal cord injury and central hypoventilation syndrome. Patients with SCI should be carefully screened to assess the integrity of their phrenic nerves. In eligible patients, DP improves mobility, speech, olfaction, and quality of life. The stigma of being attached to a mechanical device and risk of ventilator disconnection are eliminated. There is also some evidence that DP results in a reduction in the rate of respiratory tract infections...
June 2018: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/29779600/obesity-hypoventilation-syndrome
#2
REVIEW
Imran H Iftikhar, Joshua Roland
Obesity hypoventilation syndrome has been noted for centuries, yet we still are trying to uncover the exact mechanisms behind the disease and best treatment modalities for patients afflicted by the condition. The syndrome, which results in symptoms based on a diverse spectrum of interactions between obesity, ventilatory drive, and sleep's impact on respiration, has been shown to worsen morbidity and mortality far beyond that of more typical sleep-disordered breathing. In this article, the authors discuss current knowledge and research about the epidemiology, pathophysiology, and clinical features, along with treatment considerations of obesity hypoventilation syndrome...
June 2018: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/29779591/sleep-disordered-breathing-in-neuromuscular-and-chest-wall-diseases
#3
REVIEW
Janet Hilbert
Neuromuscular and chest wall diseases include a diverse group of conditions that share common risk factors for sleep-disordered breathing, including respiratory muscle weakness and/or thoracic restriction. Sleep-disordered breathing results from both the effects of normal sleep on ventilation and the additional challenges imposed by the underlying disorders. Patterns of sleep- disordered breathing vary with the specific diagnosis and stage of disease. Sleep hypoventilation precedes diurnal respiratory failure and may be difficult to recognize clinically because symptoms are nonspecific...
June 2018: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/29779589/chest-wall-diseases-respiratory-pathophysiology
#4
REVIEW
George E Tzelepis
The chest wall consists of various structures that function in an integrated fashion to ventilate the lungs. Disorders affecting the bony structures or soft tissues of the chest wall may impose elastic loads by stiffening the chest wall and decreasing respiratory system compliance. These alterations increase the work of breathing and lead to hypoventilation and hypercapnia. Respiratory failure may occur acutely or after a variable period of time. This review focuses on the pathophysiology of respiratory function in specific diseases and disorders of the chest wall, and highlights pathogenic mechanisms of respiratory failure...
June 2018: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/29773292/health-related-quality-of-life-measurement-in-patients-with-chronic-respiratory-failure
#5
REVIEW
Toru Oga, Wolfram Windisch, Tomohiro Handa, Toyohiro Hirai, Kazuo Chin
The improvement of health-related quality of life (HRQL) is an important goal in managing patients with chronic respiratory failure (CRF) receiving long-term oxygen therapy (LTOT) and/or domiciliary noninvasive ventilation (NIV). Two condition-specific HRQL questionnaires have been developed to specifically assess these patients: the Maugeri Respiratory Failure Questionnaire (MRF) and the Severe Respiratory Insufficiency Questionnaire (SRI). The MRF is more advantageous in its ease of completion; conversely, the SRI measures diversified health impairments more multi-dimensionally and discriminatively with greater balance, especially in patients receiving NIV...
May 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29767787/sleep-disordered-breathing-in-c57bl-6j-mice-with-diet-induced-obesity
#6
Thomaz Fleury Curado, Huy Pho, Slava Berger, Candela Caballero-Eraso, Mi-Kyung Shin, Luiz Ubirajara Sennes, Luu Pham, Alan R Schwartz, Vsevolod Y Polotsky
Obesity leads to sleep disordered breathing (SDB), as manifested by recurrent upper airway obstructions during sleep termed obstructive sleep apnea and carbon dioxide retention due to obesity hypoventilation. The objective of this work was to characterize breathing during sleep in C57BL6/J mice with diet-induced obesity. Arterial blood gas was measured in nine obese and nine lean mice during wakefulness. Nine male mice with diet induced obesity and six lean male C57BL/6J mice were head mounted with EEG and EMG electrodes...
May 15, 2018: Sleep
https://www.readbyqxmd.com/read/29766929/sleep-disorders-in-amyotrophic-lateral-sclerosis-a-questionnaire-based-study-from-india
#7
Samhita Panda, Mandaville Gourie-Devi, Ankkita Sharma
Background: Amyotrophic lateral sclerosis (ALS) is a relatively rare neurological disorder affecting upper and lower motor neurons in the brain and spinal cord with survival for 3-5 years and rarely beyond 10 years. Sleep disturbances in ALS are underreported and undertreated and there is no related data from India. This study aimed to assess the frequency of sleep disorders in patients of ALS and their determinants. Methods: Patients with definite and probable ALS as per the El Escorial criteria were recruited from May 2014 to April 2016...
May 2018: Neurology India
https://www.readbyqxmd.com/read/29764583/-syndromic-hirschsprung-prime-s-disease-and-its-mode-of-inheritance
#8
Jing-Ru Zhang, Zhi-Bo Zhang
Hirschsprung′s disease (HSCR) is one of the major causes of chronic incomplete intestinal obstruction in children. HSCR is considered a type of neurocristopathy caused by no colonization of ganglion cells on some parts of the bowel wall due to abnormal termination of the migration of vagal neural cells during embryonic development. This disease can be classified into different types according to the length of the affected intestinal canal. Most HSCR patients present with single deformity, but some HSCR patients are affected by other deformities, which constitutes syndromic HSCR, such as congenital central hypoventilation syndrome, Fryns syndrome, and cartilage-hair hypoplasia syndrome...
May 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29756062/effect-of-preoperative-gabapentin-and-acetaminophen-on-opioid-consumption-in-video-assisted-thoracoscopic-surgery-a-retrospective-study
#9
Robert Qiu, Albert C Perrino, Holly Zurich, Nitin Sukumar, Feng Dai, Wanda Popescu
Background: Patients undergoing video-assisted thoracoscopic surgery (VATS) are particularly vulnerable to opioid-induced sedation and hypoventilation. Accordingly, reducing opioid consumption in these patients is a primary goal of multimodal analgesic regimens. Although administration of preoperative gabapentin and acetaminophen has been shown to decrease postoperative opioid consumption in other surgeries, this approach has not been studied in VATS lobectomy. Our objective was to examine the impact of the addition of preoperative gabapentin and acetaminophen to a VATS lobectomy multimodal analgesic plan on postoperative opioid consumption, nausea/vomiting, and sedation...
April 2018: Romanian Journal of Anaesthesia and Intensive Care
https://www.readbyqxmd.com/read/29755801/central-hypoventilation-a-rare-complication-of-wallenberg-syndrome
#10
Keithan Sivakumar, Manveer Garcha, Dev Mehta, Megan C Leary, Hussam A Yacoub
Central alveolar hypoventilation disorders denote conditions resulting from underlying neurologic disorders affecting the sensors, the central controller, or the integration of those signals leading to insufficient ventilation and reduction in partial pressures of oxygen. We report a patient who presented with a left lateral medullary ischemic stroke after aneurysm repair who subsequently developed a rare complication of CAH. Increased awareness of this condition's clinical manifestations is crucial to make an accurate diagnosis and understand its complications and prognosis...
2018: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/29743771/cervical-myelopathy-in-a-child-a-rare-cause-of-hypoventilation-syndrome-presenting-with-type-2-respiratory-failure
#11
Alolika Mondal, Prabhas Prasun Giri
Hypoventilation syndrome leading to Type 2 respiratory failure is not a rare cause of Pediatric Intensive Care Unit admission and mechanical ventilation. Common causes in pediatric population are Guillain-Barre syndrome and various central nervous system disorders such as encephalitis, traumatic brain injury, and drugs. Any injury or disease in the cervical cord can also produce respiratory paralysis causing respiratory failure. Here, we present two cases of mixed cerebral palsy with cervical myelopathy due to compression effect of fractured segments of first and second cervical vertebrae...
April 2018: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/29738021/unusual-case-of-central-alveolar-hypoventilation-lessons-learned-from-ventilatory-management-of-patients-with-alveolar-hypoventilation-in-critical-care-conditions
#12
https://www.readbyqxmd.com/read/29734990/exosomal-cargo-properties-endothelial-function-and-treatment-of-obesity-hypoventilation-syndrome-a-proof-of-concept-study
#13
Rakesh Bhattacharjee, Abdelnaby Khalyfa, Ahamed A Khalyfa, Babak Mokhlesi, Leila Kheirandish-Gozal, Isaac Almendros, Eduard Peris, Atul Malhotra, David Gozal
STUDY OBJECTIVES: Longitudinal studies support the usage of positive airway pressure (PAP) therapy in treating obstructive sleep apnea (OSA) to improve cardiovascular disease. However, the anticipated benefit is not ubiquitous. In this study, we elucidate whether PAP therapy leads to immediate improvements on endothelial function, a subclinical marker of cardiovascular status, by examining the effect of circulating exosomes, isolated from patients before and after PAP therapy, on naive endothelial cells...
April 30, 2018: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://www.readbyqxmd.com/read/29728260/anesthesia-in-a-pediatric-patient-with-rohadd-syndrome
#14
E Esparza Isasa, M A Palomero Rodríguez, I Acebedo Bambaren, C Medrano Viñas, D Gil Mayo, F Domínguez Pérez, D Pestaña Lagunas
Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome is a rare entity that is characterised by its onset in healthy children at 2-4 years of age. It is a complex syndrome that includes, among other symptoms, rapid weight gain with hyperphagia, hypothalamic dysfunction, central hypoventilation, and autonomic dysregulation. The case is presented of a 10-year-old boy with a diagnosis of ROHHAD syndrome undergoing insertion of a port-a-cath under general anaesthesia, who developed complications during the anaesthetic procedure related to his illness...
May 1, 2018: Revista Española de Anestesiología y Reanimación
https://www.readbyqxmd.com/read/29724743/efficacy-of-bilevel-positive-airway-pressure-and-continuous-positive-airway-pressure-therapy-in-patients-with-obesity-hypoventilation-syndrome-protocol-for-systematic-review-and-meta-analysis
#15
Lili Guan, Weiliang Wu, Yating Huo, Xiaoying Li, Bingpeng Guo, Yuqiong Yang, Jiawen Xu, Fangfang Jiang, Luqian Zhou, Rongchang Chen
INTRODUCTION: Obesity hypoventilation syndrome (OHS) is a major respiratory complication caused by severe obesity, being associated with significant morbidity, negative impacts on quality of life and reduced survival if not treated appropriately. Positive airway pressure therapy is the first-line treatment for OHS although the optimal modality remains unclear. The goal of this study is to identify the efficacy of home bilevel positive airway pressure therapy by comparison to continuous positive airway pressure therapy and determine the best strategy for patients with OHS...
May 3, 2018: BMJ Open
https://www.readbyqxmd.com/read/29704303/atypical-presentations-associated-with-non-polyalanine-repeat-phox2b-mutations
#16
Umakanth Katwa, Alissa M D'Gama, Anita E Qualls, Lucas M Donovan, Jody Heffernan, Jiahai Shi, Pankaj B Agrawal
Congenital central hypoventilation syndrome (CCHS) is a disorder of ventilatory control and autonomic dysregulation that can be caused by mutations in the paired-like homeobox 2B (PHOX2B) gene. The majority of CCHS cases are caused by polyalanine repeat mutations (PARMs) in PHOX2B; however, in rare cases, non-polyalanine repeat mutations (NPARMs) have been identified. Here, we report two patients with NPARMs in PHOX2B. Patient 1 has a mild CCHS phenotype seen only on polysomnogram, which was performed for desaturations and stridor following a bronchiolitis episode, and characterized by night-time hypoventilation and a history of ganglioneuroblastoma...
April 28, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29701198/-a-case-of-severe-obstructive-sleep-apnea-hypopnea-syndrome-with-urinary-and-anal-incontinence
#17
Li Zhou, Ruoyun Ouyang, Ping Chen, Hong Luo, Hanmei Liu, Guiqian Liu
A case of a young male patient, who came to the Second Xiangya Hospital, Central South University because of snoring for 10 years and nocturnal gatism for half month, was analyzed retrospectively. He was diagnosed as obstructive sleep apnea hypopnea syndrome (OSAHS) finally. The patient had been diagnosed and treated as stroke in the local hospital, while urinary and anal incontinence were not relieved. It was a dilemma for him to be properly diagnosed and treated. Polysomnography in our hospital revealed apnea hypopnea index (AHI) at 44...
March 28, 2018: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/29696799/expanding-the-phenotype-of-congenital-central-hypoventilation-syndrome-impacts-management-decisions
#18
Heather M Byers, Maida Chen, Andrew S Gelfand, Bruce Ong, Marisa Jendras, Ian A Glass
Congenital central hypoventilation syndrome (CCHS) is a neurocristopathy caused by pathogenic heterozygous variants in the gene paired-like homeobox 2b (PHOX2B). It is characterized by severe infantile alveolar hypoventilation. Individuals may also have diffuse autonomic nervous system dysfunction, Hirschsprung disease and neural crest tumors. We report three individuals with CCHS due to an 8-base pair duplication in PHOX2B; c.691_698dupGGCCCGGG (p.Gly234Alafs*78) with a predominant enteral and neural crest phenotype and a relatively mild respiratory phenotype...
April 25, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29696617/-clinical-and-neuropsychological-characteristics-in-congenital-central-hypoventilation-syndrome
#19
R Seijas-Gomez, B Esteso-Orduna, J Melero-Llorente, M C Fournier-Del Castillo
INTRODUCTION: Congenital central hypoventilation syndrome (CCHS) syndrome is a rare disease caused by mutations in the PHOX2B gene. Patients show a reduced response to hypercapnia and hypoxia accompanied by diffuse disturbances of the autonomic nervous system and occasionaly also disturbances in neuroimaging. A specific neuropsychological profile has not been described in children and adolescents with CCHS. CASE REPORTS: We describe three cases (aged between 4 and 19 years) with different profiles of affectation in cognitive and functionality...
May 1, 2018: Revista de Neurologia
https://www.readbyqxmd.com/read/29694990/respiratory-instability-and-hypoxemia-episodes-in-preterm-infants
#20
Eduardo Bancalari, Nelson Claure
Oxygenation instability is a very common problem in the premature infant that manifests as intermittent hypoxemia episodes (HEs). These are particularly frequent in premature infants who are on mechanical ventilation beyond the first weeks after birth. However, they can also occur in spontaneously breathing infants. Some of these episodes are due to central apnea, but in ventilated infants, they are frequently due to contractions of the abdominal musculature that can splint the respiratory pump, resulting in periods of decreased lung volume and hypoventilation...
May 2018: American Journal of Perinatology
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