2,841 results match your criteria Hypoventilation Syndromes


Respiratory Control by Phox2b-expressing Neurons in a Locus Coeruleus-preBötzinger Complex Circuit.

Neurosci Bull 2020 May 28. Epub 2020 May 28.

Department of Physiology, Hebei Medical University, Shijiazhuang, 050017, China.

The locus coeruleus (LC) has been implicated in the control of breathing. Congenital central hypoventilation syndrome results from mutation of the paired-like homeobox 2b (Phox2b) gene that is expressed in LC neurons. The present study was designed to address whether stimulation of Phox2b-expressing LC (Phox2b) neurons affects breathing and to reveal the putative circuit mechanism. Read More

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http://dx.doi.org/10.1007/s12264-020-00519-1DOI Listing

Sleep-related breathing disorders associated with the characteristics of underlying congenital rare diseases of Moebius syndrome and Poland syndrome.

Respirol Case Rep 2020 Jul 18;8(5):e00579. Epub 2020 May 18.

Division of Respiratory Medicine Juntendo University Faculty of Medicine & Graduate School of Medicine Tokyo Japan.

A 24-year-old woman was referred to us with daytime sleepiness. She has two congenital intractable and rare diseases, namely, Moebius syndrome and Poland syndrome. Physiological examinations and a detailed usage analysis under a ventilation device helped to conclude that hypoglossal nerve paralysis and thoracic deformity from her two underlying diseases were associated closely with her final diagnosis of obstructive sleep apnoea and sleep-related hypoventilation due to medical disorders. Read More

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http://dx.doi.org/10.1002/rcr2.579DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7235444PMC

DCTN1-related Parkinson-plus disorder (Perry syndrome).

Pract Neurol 2020 May 20. Epub 2020 May 20.

Department of Neurology, St George's Hospital, London, UK

Dynactin-1 (DCTN1)-related Parkinson-plus disorder (Perry syndrome) is an autosomal dominant neurodegenerative disorder characterised by levodopa-resistant parkinsonism, weight loss, mood change and central hypoventilation. Ventilatory insufficiency is the predominant cause of death. It has been previously described in 87 people from 20 families with a worldwide distribution. Read More

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http://dx.doi.org/10.1136/practneurol-2020-002505DOI Listing

Effect of ventilation therapy on mortality rate among obesity hypoventilation syndrome and obstructive sleep apnoea patients.

ERJ Open Res 2020 Apr 11;6(2). Epub 2020 May 11.

Sleep Unit, Dept of Respiratory Medicine, Heart and Lung Center, Helsinki University Hospital, University of Helsinki, Helsinki, Finland.

Introduction: The prevalence of obesity is continually increasing worldwide, which increases the incidence of obesity hypoventilation syndrome (OHS) and its consequent mortality.

Methods: We reviewed the therapy mode, comorbidity and mortality of all OHS patients treated at our hospital between 2005 and 2016. The control group consisted of randomly selected patients with obstructive sleep apnoea (OSA) treated during the same period. Read More

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http://dx.doi.org/10.1183/23120541.00101-2019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7211948PMC

ROHHAD(NET) Syndrome: Systematic review of the clinical timeline and recommendations for diagnosis and prognosis.

J Clin Endocrinol Metab 2020 May 14. Epub 2020 May 14.

Department of Human Genetics, CHU Liège, Belgium.

Context: Rapid onset Obesity with Hypothalamic dysfunction, Hypoventilation, Autonomic Dysregulation and Neural Tumor Syndrome (ROHHHAD(NET)) is a rare and potentially fatal disease. No specific diagnostic biomarker is currently available, making prompt diagnosis challenging. Since its first definition in 2007, a complete clinical analysis leading to specific diagnosis and follow-up recommendations is still missing. Read More

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http://dx.doi.org/10.1210/clinem/dgaa247DOI Listing

Neurocognitive functioning in individuals with congenital central hypoventilation syndrome.

BMC Pediatr 2020 May 6;20(1):194. Epub 2020 May 6.

Department of Pediatrics, 2Department of Pediatrics, McGovern Medical School at the University of Texas Health Science Center, Houston, TX, USA.

Background: Congenital central hypoventilation syndrome (CCHS) is a rare disorder characterized by respiratory system abnormalities, including alveolar hypoventilation and autonomic nervous system dysregulation. CCHS is associated with compromised brain development and neurocognitive functioning. Studies that evaluate cognitive skills in CCHS are limited, and no study has considered cognitive abilities in conjunction with psychosocial and adaptive functioning. Read More

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http://dx.doi.org/10.1186/s12887-020-2006-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7203975PMC

Long-term Noninvasive Ventilation in Obesity Hypoventilation Syndrome Without Severe OSA: The Pickwick Randomized Controlled Trial.

Chest 2020 Apr 25. Epub 2020 Apr 25.

Department of Medicine/Pulmonary and Critical Care, University of Chicago, IL.

Background: Noninvasive ventilation (NIV) is an effective form of treatment in obesity hypoventilation syndrome (OHS) with severe OSA. However, there is paucity of evidence in patients with OHS without severe OSA phenotype.

Research Question: Is NIV effective in OHS without severe OSA phenotype?

Study Design And Methods: In this multicenter, open-label parallel group clinical trial performed at 16 sites in Spain, we randomly assigned 98 stable ambulatory patients with untreated OHS and apnea-hypopnea index < 30 events/h (ie, no severe OSA) to NIV or lifestyle modification (control group) using simple randomization through an electronic database. Read More

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http://dx.doi.org/10.1016/j.chest.2020.03.068DOI Listing

Familial Sleep Disorders in Unknown Genetic Syndrome.

J Pediatr Genet 2020 Jun 21;9(2):132-136. Epub 2019 Oct 21.

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Sleep-disordered breathing (SDB) is common in children, especially in those with congenital or genetic diseases. The factors involved include obstructive sleep apnea, disrupted rapid eye movement sleep, and central hypoventilation. Diagnosing and treating SDB in these children have a positive impact on the quality of life of them and their families, reducing the risk of both further impairment of cognitive abilities and cardiopulmonary complications. Read More

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http://dx.doi.org/10.1055/s-0039-1698808DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7183398PMC

Adult-onset congenital central hypoventilation syndrome due to PHOX2B mutation.

Acta Neurol Belg 2020 Apr 25. Epub 2020 Apr 25.

Department of Neurology, Ghent University Hospital, Ghent, Belgium.

Central hypoventilation in adult patients is a rare life-threatening condition characterised by the loss of automatic breathing, more pronounced during sleep. In most cases, it is secondary to a brainstem lesion or to a primary pulmonary, cardiac or neuromuscular disease. More rarely, it can be a manifestation of congenital central hypoventilation syndrome (CCHS). Read More

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http://dx.doi.org/10.1007/s13760-020-01363-wDOI Listing

Letter to the Editor: Obesity hypoventilation syndrome and severe COVID-19.

Metabolism 2020 Apr 22;108:154249. Epub 2020 Apr 22.

NAFLD Research Center, Department of Hepatology, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China; Institute of Hepatology, Wenzhou Medical University, Wenzhou, China; Key Laboratory of Diagnosis and Treatment for The Development of Chronic Liver Disease in Zhejiang Province, Wenzhou, China. Electronic address:

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http://dx.doi.org/10.1016/j.metabol.2020.154249DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7195293PMC

Perry syndrome: a case of atypical parkinsonism with confirmed DCTN1 mutation.

N Z Med J 2020 Apr 24;133(1513):116-118. Epub 2020 Apr 24.

Neurologist, Waikato General Hospital, Hamilton, Waikato.

Perry syndrome is a rare neurological condition characterised clinically by depression, sleep disturbance, central hypoventilation and parkinsonism. Perry syndrome is a TAR DNA-binding protein 43 (TDP-43) proteinopathy associated with mutated dynactin-1 protein, inherited in an autosomal dominant manner. Several pathogenic mutations in exon 2 in the dynactin 1 gene have been identified; p. Read More

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Is Adipose Tissue a Reservoir for Viral Spread, Immune Activation, and Cytokine Amplification in Coronavirus Disease 2019?

Obesity (Silver Spring) 2020 Apr 21. Epub 2020 Apr 21.

Centre for Research in Vascular Biology, APC Microbiome Ireland, University College Cork, Cork University Hospital, Cork, Ireland.

Coronavirus disease 2019 (COVID-19), the worst pandemic in more than a century, has claimed >125,000 lives worldwide to date. Emerging predictors for poor outcomes include advanced age, male sex, preexisting cardiovascular disease, and risk factors including hypertension, diabetes, and, more recently, obesity. This article posits new obesity-driven predictors of poor COVID-19 outcomes, over and above the more obvious extant risks associated with obesity, including cardiometabolic disease and hypoventilation syndrome in intensive care patients. Read More

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http://dx.doi.org/10.1002/oby.22843DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7264526PMC
April 2020
3.734 Impact Factor

Effects of Wait Times on Treatment Adherence and Clinical Outcomes in Patients With Severe Sleep-Disordered Breathing: A Secondary Analysis of a Noninferiority Randomized Clinical Trial.

JAMA Netw Open 2020 Apr 1;3(4):e203088. Epub 2020 Apr 1.

Department of Medicine, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.

Importance: Sleep-disordered breathing (SDB) is common and associated with substantial adverse health consequences. Long wait times for SDB care are commonly reported; however, it is unclear whether wait times for care are associated with clinical outcomes.

Objective: To evaluate the association of wait times for care with clinical outcomes for patients with severe SDB. Read More

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http://dx.doi.org/10.1001/jamanetworkopen.2020.3088DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7171552PMC

Bi-allelic Loss-of-Function Variants in NUP188 Cause a Recognizable Syndrome Characterized by Neurologic, Ocular, and Cardiac Abnormalities.

Am J Hum Genet 2020 May 9;106(5):623-631. Epub 2020 Apr 9.

Department of Pediatrics, Division of Genetic Medicine, University of Washington, Seattle, WA 98195, USA; Seattle Children's Hospital, Seattle, WA 98105, USA; Brotman Baty Institute for Precision Medicine, Seattle, WA 98195, USA. Electronic address:

Nucleoporins (NUPs) are an essential component of the nuclear-pore complex, which regulates nucleocytoplasmic transport of macromolecules. Pathogenic variants in NUP genes have been linked to several inherited human diseases, including a number with progressive neurological degeneration. We present six affected individuals with bi-allelic truncating variants in NUP188 and strikingly similar phenotypes and clinical courses, representing a recognizable genetic syndrome; the individuals are from four unrelated families. Read More

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http://dx.doi.org/10.1016/j.ajhg.2020.03.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7212259PMC

Screening Children with a Family History of Central Congenital Hypoventilation Syndrome.

Case Rep Pediatr 2020 26;2020:2713606. Epub 2020 Mar 26.

Department of Pediatrics, The University of Texas Health Science Center at Houston, McGovern Medical School, Houston, TX, USA.

Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder of an autonomic nervous disorder that affects breathing. It is characterized by respiratory insufficiency secondary to insensitivity to hypoxemia and hypercarbia, particularly during sleep leading to persistent apnea. We report four individuals across two generations harboring heterozygous 25 polyalanine repeats mutations (PARMs) in PHOX2B with a varying degree of phenotypic clinical manifestations. Read More

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http://dx.doi.org/10.1155/2020/2713606DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7136801PMC

Primary tracheocutaneous fistula closure with immediate transition to nocturnal noninvasive positive pressure ventilation in two children with Congenital Central Hypoventilation Syndrome.

Int J Pediatr Otorhinolaryngol 2020 Mar 24;134:110019. Epub 2020 Mar 24.

Children's Hospital of Wisconsin, Department of Otolaryngology/Medical College of Wisconsin, Milwaukee, WI, USA. Electronic address:

Transitioning children with Congenital Central Hypoventilation Syndrome (CCHS) from nocturnal invasive ventilation via tracheostomy to noninvasive positive pressure ventilation (NIPPV) is challenging due to the leak caused by the tracheocutaneous fistula (TCF), resulting in insufficient ventilation. Decannulation and primary closure of the TCF with immediate transition to nocturnal NIPPV was performed in two children with CCHS at a tertiary care children's hospital. Neither child developed significant adverse effects such as pneumomediastinum or pneumothorax. Read More

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http://dx.doi.org/10.1016/j.ijporl.2020.110019DOI Listing

Long-Term Noninvasive Ventilation in the Geneva Lake Area: Indications, Prevalence, and Modalities.

Chest 2020 Mar 31. Epub 2020 Mar 31.

Division of Pulmonary Diseases, Geneva University Hospitals (HUG), Geneva, Switzerland; Faculty of Medicine, University of Geneva, Geneva, Switzerland. Electronic address:

Background: Noninvasive ventilation (NIV) is standard of care for chronic hypercapnic respiratory failure, but indications, devices, and ventilatory modes are in constant evolution.

Research Question: To describe changes in prevalence and indications for NIV over a 15-year period; to provide a comprehensive report of characteristics of the population treated (age, comorbidities, and anthropometric data), mode of implementation and follow-up, devices, modes and settings used, physiological data, compliance, and data from ventilator software.

Study Design And Methods: Cross-sectional observational study designed to include all subjects under NIV followed by all structures involved in NIV in the Cantons of Geneva and Vaud (1,288,378 inhabitants). Read More

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http://dx.doi.org/10.1016/j.chest.2020.02.064DOI Listing

Positive airway pressure in obesity hypoventilation syndrome: is it worth it?

Thorax 2020 Jun 26;75(6):439-440. Epub 2020 Mar 26.

Lane Fox Respiratory Service, Guy's & St Thomas' NHS Foundation Trust, London, UK.

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http://dx.doi.org/10.1136/thoraxjnl-2019-214403DOI Listing

Cost-effectiveness of positive airway pressure modalities in obesity hypoventilation syndrome with severe obstructive sleep apnoea.

Thorax 2020 Jun 26;75(6):459-467. Epub 2020 Mar 26.

Respiratory Department, San Pedro de Alcantara Hospital, Caceres, Spain.

Background: Obesity hypoventilation syndrome (OHS) is treated with either non-invasive ventilation (NIV) or CPAP, but there are no long-term cost-effectiveness studies comparing the two treatment modalities.

Objectives: We performed a large, multicentre, randomised, open-label controlled study to determine the comparative long-term cost and effectiveness of NIV versus CPAP in patients with OHS with severe obstructive sleep apnoea (OSA) using hospitalisation days as the primary outcome measure.

Methods: Hospital resource utilisation and within trial costs were evaluated against the difference in effectiveness based on the primary outcome (hospitalisation days/year, transformed and non-transformed in monetary term). Read More

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http://dx.doi.org/10.1136/thoraxjnl-2019-213622DOI Listing

ISCCM Guidelines for the Use of Non-invasive Ventilation in Acute Respiratory Failure in Adult ICUs.

Indian J Crit Care Med 2020 Jan;24(Suppl 1):S61-S81

Department of Division of Critical Care Medicine, Department of Anaesthesia, Critical Care and Pain, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India, , e-mail:

A. ACUTE HYPERCAPNIC RESPIRATORY FAILURE A1. Acute Exacerbation of COPD: Recommendations: NIV should be used in management of acute exacerbation of COPD in patients with acute or acute-on-chronic respiratory acidosis (pH = 7. Read More

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http://dx.doi.org/10.5005/jp-journals-10071-G23186DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7085817PMC
January 2020

Nocturnal hypoventilation in Down syndrome children with or without sleep apnea.

Pediatr Pulmonol 2020 May 28;55(5):1246-1253. Epub 2020 Feb 28.

Department of Pediatric Pulmonology, Armand Trousseau Hospital, AP-HP, Paris, France.

Background: There is a high prevalence of obstructive sleep apnea (OSA) in children with Down syndrome (DS), sometimes associated with alveolar hypoventilation.

Objective: To compare transcutaneous partial pressure of carbon dioxide (PtcCO ) and pulse oximetry (SpO ) in children with DS and in control children with OSA.

Patients And Methods: This retrospective case-control study involved children followed in Trousseau Hospital (Paris) Sleep Center. Read More

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http://dx.doi.org/10.1002/ppul.24703DOI Listing

Sodium glucose cotransporter2 inhibitor-possible treatment for patients with diabetes, pulmonary disease and CO retention.

Med Hypotheses 2020 Jun 14;139:109631. Epub 2020 Feb 14.

Department of Internal Medicine E, HaEmek Medical Center, Rabin Blvd, 18101 Afula, Israel; The Ruth and Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.

Chronic pulmonary diseases such as chronic obstructive pulmonary disease, obstructive sleep apnea and obesity hypoventilation syndrome are common conditions which share decreased pulmonary ventilation and CO retention. CO is an end-product of metabolism of all body cells. When CO accumulates, it is recommended to consider measures to reduce its endogenous production. Read More

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http://dx.doi.org/10.1016/j.mehy.2020.109631DOI Listing

Use of ultra-rapid whole-exome sequencing to diagnose congenital central hypoventilation syndrome.

Pediatr Pulmonol 2020 Apr 14;55(4):855-857. Epub 2020 Feb 14.

Respiratory and Sleep Medicine, Royal Children's Hospital, Melbourne, Australia.

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http://dx.doi.org/10.1002/ppul.24686DOI Listing

Time-to-death in chronic respiratory failure on home mechanical ventilation: A cohort study.

Respir Med 2020 02 13;162:105877. Epub 2020 Jan 13.

Lane Fox Respiratory Unit and Lane Fox Clinical Respiratory Physiology Research Centre, Guy's and St Thomas' Hospital NHS Foundation Trust, SE1 7EH, London, UK; Faculty of Life Sciences and Medicine, King's College London, WC2R 2LS, London, UK.

Background And Objective: Home mechanical ventilation (HMV) is used in heterogeneous conditions underlying chronic hypercapnic respiratory failure, but there are sparse data on long-term clinical outcomes. The aim was to systematically analyse the time and the circumstances of death on HMV.

Methods: All-cause mortality data of HMV patients were prospectively collected between 2008 and 2018 in a large tertiary centre. Read More

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http://dx.doi.org/10.1016/j.rmed.2020.105877DOI Listing
February 2020

AVAPS-AE versus ST mode: A randomized controlled trial in patients with obesity hypoventilation syndrome.

Respirology 2020 Feb 13. Epub 2020 Feb 13.

Service de Pneumologie, oncologie thoracique et Soins Intensifs Respiratoires, Rouen University Hospital, Rouen, France.

Background And Objective: Average volume-assured pressure support-automated expiratory positive airway pressure (AVAPS-AE) combines an automated positive expiratory pressure to maintain upper airway patency to an automated pressure support with a targeted tidal volume. The aim of this study was to compare the effects of 2-month AVAPS-AE ventilation versus pressure support (ST) ventilation on objective sleep quality in stable patients with OHS. Secondary outcomes included arterial blood gases, health-related quality of life, daytime sleepiness, subjective sleep quality and compliance to NIV. Read More

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http://dx.doi.org/10.1111/resp.13784DOI Listing
February 2020

Correction to: Life-threatening cardiac arrhythmias in congenital central hypoventilation syndrome.

Eur J Pediatr 2020 Feb 11. Epub 2020 Feb 11.

Children's Hospital Los Angeles, Los Angeles, CA, USA.

The publisher regrets that in the original published version of this article, one of the author's name was incorrectly presented as "Yaniv Bar Cohen". The correct presentation should have been "Yaniv Bar-Cohen" and is now presented correctly in this article. Read More

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http://dx.doi.org/10.1007/s00431-020-03604-9DOI Listing
February 2020

The Effect of Hospital Discharge with Empiric Noninvasive Ventilation on Mortality in Hospitalized Patients with Obesity Hypoventilation Syndrome. An Individual Patient Data Meta-Analysis.

Ann Am Thorac Soc 2020 May;17(5):627-637

Department of Medicine, Boston University School of Medicine, Boston, Massachusetts; and.

Hospitalized patients with acute-on-chronic hypercapnic respiratory failure due to obesity hypoventilation syndrome (OHS) have increased short-term mortality. It is unknown whether prescribing empiric positive airway pressure (PAP) at the time of hospital discharge reduces mortality compared with waiting for an outpatient evaluation (i.e. Read More

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http://dx.doi.org/10.1513/AnnalsATS.201912-887OCDOI Listing

Ondine's curse: the origin of the myth.

Arq Neuropsiquiatr 2020 Apr;78(4):238-240

Universidad del Rosario, School of Medicine and Health Sciences, Neuroscience Research Group NEUROS, Bogotá, Colombia.

Central alveolar hypoventilation syndrome has been known for decades as Ondine's curse. It was named as such after a German myth. Although most of the stories resemble one another, word of mouth has led to misinterpretation of this tale among the medical community. Read More

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http://dx.doi.org/10.1590/0004-282X20190162DOI Listing

Positional impairment of gas exchange during diaphragm pacing alleviated by increasing amplitude settings in congenital central hypoventilation syndrome.

J Clin Sleep Med 2020 Mar 14;16(3):459-462. Epub 2020 Jan 14.

Division of Pediatric Pulmonology and Sleep Medicine, Emory University, Atlanta, Georgia.

None: Diaphragm pacing (DP) by phrenic nerve stimulation is a modality of chronic ventilatory support in individuals with congenital central hypoventilation syndrome (CCHS). We report a 9-year-old girl with CCHS who uses DP without tracheostomy during sleep. Her parents report hypoxemia and hypercapnia related to positional changes of the body during sleep requiring frequent adjustment of pacer settings. Read More

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http://dx.doi.org/10.5664/jcsm.8232DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7075083PMC

Weight Loss Interventions as Treatment of Obesity Hypoventilation Syndrome. A Systematic Review.

Ann Am Thorac Soc 2020 Apr;17(4):492-502

Department of Medicine, University of Chicago, Chicago, Illinois.

Obesity hypoventilation syndrome (OHS) is an undesirable consequence of obesity. Weight loss is an important component of management based on clinical rationale, but the evidence supporting weight loss has not been summarized and the optimal approach has not been determined. This systematic review informed an international, multidisciplinary panel of experts who had converged to develop a clinical practice guideline on OHS for the American Thoracic Society. Read More

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http://dx.doi.org/10.1513/AnnalsATS.201907-554OCDOI Listing

Congenital Central Hypoventilation Syndrome Presenting with Seizures.

Cureus 2020 Jan 16;12(1):e6680. Epub 2020 Jan 16.

Urology, King Faisal Specialist Hospital and Research Center, Riyadh, SAU.

Congenital central hypoventilation syndrome (CCHS) is a critical and rare autosomal dominant disorder that was first described by Robert Mellins in 1970. CCHS is defined to be an autonomic nervous system (ANS) dysfunction that usually presents in the neonatal period with hypoventilation and dysregulated autonomic homeostasis on a multi-system level. Classically, CCHS presents with normal ventilation while awake, and hypoventilation with normal respiratory rate during sleep. Read More

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http://dx.doi.org/10.7759/cureus.6680DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6968830PMC
January 2020

Is Obesity Hypoventilation Syndrome A Postmenopausal Disorder?

Open Respir Med J 2019 15;13:51-54. Epub 2019 Nov 15.

Prince Naif Health Research Center, King Saud University, Riyadh, Saudi Arabia.

Previous studies have assessed the role of gender and menopause in Obstructive Sleep Apnea (OSA). It is well known that menopause is a major risk factor for OSA. However, analogous studies on obesity Hypoventilation Syndrome (OHS) are limited. Read More

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http://dx.doi.org/10.2174/1874306401913010051DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6952852PMC
November 2019

Life-threatening cardiac arrhythmias in congenital central hypoventilation syndrome.

Eur J Pediatr 2020 May 16;179(5):821-825. Epub 2020 Jan 16.

Children's Hospital Los Angeles, Los Angeles, CA, USA.

Congenital central hypoventilation syndrome (CCHS) patients are at risk for life-threatening cardiac arrhythmias, and presentation is dependent on their PHOX2B gene mutation. We describe the presentation of life-threatening arrhythmias in our cohort of CCHS patients. We reviewed the records of 72 CCHS patients seen at CHLA from 2004 to 2018. Read More

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http://dx.doi.org/10.1007/s00431-019-03568-5DOI Listing

Associated syndromes in patients with Pierre Robin Sequence.

Int J Pediatr Otorhinolaryngol 2020 Apr 30;131:109842. Epub 2019 Dec 30.

Pediatric ENT and Facial Plastic Surgery, Children's of Minnesota, University of Minnesota Department of Otolaryngology-Head and Neck Surgery, Minneapolis, MN, USA. Electronic address:

Objectives: Classically, Pierre Robin Sequence (PRS) is a triad of micrognathia, glossoptosis, and airway obstruction, although frequently associated with cleft palate. Current literature reports that Stickler syndrome is the most common syndrome associated with PRS, and 22q11 deletion syndrome (22q11 DS) as the second most common. This study identifies associations between PRS and genetic syndromes. Read More

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http://dx.doi.org/10.1016/j.ijporl.2019.109842DOI Listing

Adaptation of Respiratory-Related Brain Regions to Long-Term Hypercapnia: Focus on Neuropeptides in the RTN.

Front Neurosci 2019 13;13:1343. Epub 2019 Dec 13.

Department of Pharmacology, School of Medical Sciences, University of New South Wales, Sydney, NSW, Australia.

Long-term hypercapnia is associated with respiratory conditions including obstructive sleep apnea, chronic obstructive pulmonary disease and obesity hypoventilation syndrome. Animal studies have demonstrated an initial (within hours) increase in ventilatory drive followed by a decrease in this response over the long-term (days-weeks) in response hypercapnia. Little is known about whether changes in the central respiratory chemoreflex are involved. Read More

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http://dx.doi.org/10.3389/fnins.2019.01343DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6923677PMC
December 2019

Neurally Adjusted Ventilatory Assist: An Early Clue to Diagnosis of Congenital Central Hypoventilation Syndrome.

Indian J Crit Care Med 2019 Nov;23(11):536-537

Department of Pediatrics (Neurology), Sir Ganga Ram Hospital, New Delhi, India.

Congenital central hypoventilation syndrome (CCHS) is characterized by shallow breathing during sleep due to negligible ventilatory sensitivity to hypercarbia and hypoxemia. It is diagnosed using a genetic test for PHOX2B mutation, which is not easily available. Neurally adjusted ventilatory assist (NAVA) is a spontaneous ventilatory mode that was designed basically for better adapting the ventilator to the patient by using electrical activity of diaphragm (EAdi) signals. Read More

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http://dx.doi.org/10.5005/jp-journals-10071-23286DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6900881PMC
November 2019

Perioperative care of the obese patient.

Br J Surg 2020 Jan;107(2):e39-e55

Department of Surgical, Oncological and Gastroenterological Sciences, Section of Surgery, University of Padua, Padua, Italy.

Background: Obesity has become an increasing problem worldwide during the past few decades. Hence, surgeons and anaesthetists will care for an increasing number of obese patients in the foreseeable future, and should be prepared to provide optimal management for these individuals. This review provides an update of recent evidence regarding perioperative strategies for obese patients. Read More

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http://dx.doi.org/10.1002/bjs.11447DOI Listing
January 2020

Obesity-Hypoventilation Syndrome: Baseline Hemodynamic Status and Impact of non-Invasive Ventilation.

Arch Bronconeumol 2019 Dec 24. Epub 2019 Dec 24.

Servicio de Neumología, Área de Pulmón, Hospital Universitario Central de Asturias, Oviedo, Asturias, España.

Introduction: Obesity-hypoventilation syndrome (OHS) is a risk factor for heart failure (HF). Some studies associate the use of non-invasive ventilation (NIV) with changes in hemodynamic parameters. Our objective was to describe the hemodynamic status of a group of patients with OHS and to study the effect of NIV. Read More

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http://dx.doi.org/10.1016/j.arbres.2019.08.022DOI Listing
December 2019

Diagnosing obstructive sleep apnea patients with isolated nocturnal hypoventilation and defining obesity hypoventilation syndrome using new European Respiratory Society classification criteria: an Indian perspective.

Sleep Med 2020 Feb 29;66:85-91. Epub 2019 Aug 29.

Department of Biochemistry, AIIMS Bhopal, India.

Background: Recently, new classification criteria for obesity hypoventilation syndrome (OHS) have been described. OHS prevalence according to new criteria has not been reported in obstructive sleep apnea (OSA). Prevalence of OHS has not been previously reported from India. Read More

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http://dx.doi.org/10.1016/j.sleep.2019.08.009DOI Listing
February 2020
3.154 Impact Factor

Sleep disorders in aging polio survivors: A systematic review.

Ann Phys Rehabil Med 2019 Nov 30. Epub 2019 Nov 30.

Département de physiologie, explorations fonctionnelles, unité des pathologies du sommeil, AP-HP, hôpital Raymond-Poincaré, 104, boulevard Raymond-Poincaré, 92380 Garches, France.

Background: Sleep disturbances, especially sleep disordered breathing and sleep movement disorders, seem to be highly prevalent among aging polio survivors. They could contribute to late functional deterioration, fatigue, poor quality of life and negative health outcomes, thereby increasing cardiovascular risk.

Objectives: This review focused on current knowledge of the prevalence of sleep disorders in polio survivors, their features, predictive factors and management. Read More

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http://dx.doi.org/10.1016/j.rehab.2019.10.007DOI Listing
November 2019

Pediatric polysomnography-A review of indications, technical aspects, and interpretation.

Paediatr Respir Rev 2020 Apr 30;34:9-17. Epub 2019 Oct 30.

Children's Hospital of Philadelphia Sleep Center, United States; Division of Pulmonary and Sleep Medicine, Children's Hospital of Philadelphia, United States. Electronic address:

Polysomnography is an elaborate diagnostic test composed of numerous data-collecting sensors working concomitantly to aid in the evaluation of varied sleep disorders in all age groups. Polysomnography is the study of choice for the assessment of pediatric sleep-disordered breathing, including obstructive sleep apnea syndrome, central apnea, and hypoventilation disorders, and is used to help determine treatment efficacy. Beyond the purview of snoring and breathing pauses, polysomnography can elucidate the etiology of hypersomnolence, when associated with a multiple sleep latency test, and abnormal movements or events, whether nocturnal seizure or complex parasomnia, when a thorough patient history cannot provide clear answers. Read More

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http://dx.doi.org/10.1016/j.prrv.2019.09.009DOI Listing

Positive Airway Pressure in Obesity Hypoventilation: Getting to the Heart of the Matter.

Am J Respir Crit Care Med 2020 03;201(5):509-511

Department of Respiratory & Sleep MedicineRoyal Prince Alfred HospitalCamperdown, Australia.

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http://dx.doi.org/10.1164/rccm.201911-2162EDDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7047462PMC

[Obesity hypoventilation syndrome : 2019's management].

Rev Med Suisse 2019 Nov;15(671):2100-2103

Service de pneumologie, CHUV, 1011 Lausanne.

Obesity hypoventilation syndrome is an underdiagnosed pathology, whose prevalence is increasing due to the progressively higher prevalence of obesity in the general population. Early detection allows early management and lowers the risk of acute exacerbation, hospitalization and mortality. The diagnosis is done by excluding other, pulmonary or extra pulmonary, reasons of hypercapnia; a nocturnal polygraphy is mandatory to diagnose an associated obstructive sleep apnea syndrome. Read More

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November 2019

The Role of Positive Airway Pressure Therapy in Adults with Obesity Hypoventilation Syndrome. A Systematic Review and Meta-Analysis.

Ann Am Thorac Soc 2020 Mar;17(3):344-360

Department of Medicine, University of Chicago, Chicago, Illinois.

Obesity hypoventilation syndrome (OHS) is an undesirable complication of severe obesity. Although weight loss is an accepted component of management, it is difficult to achieve and sustain the degree of weight loss necessary to reverse OHS. As such, positive airway pressure (PAP) during sleep has become the cornerstone therapy for most patients with OHS. Read More

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http://dx.doi.org/10.1513/AnnalsATS.201907-528OCDOI Listing

Is there a threat of an increase in the rates of obesity hypoventilation syndrome?

Expert Rev Respir Med 2020 Feb 14;14(2):117-119. Epub 2019 Nov 14.

Cardio-Thoracic Department, Respiratory and Sleep disorders Unit, Policlinic University hospital, UNIBA, Bari, Italy.

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http://dx.doi.org/10.1080/17476348.2020.1690992DOI Listing
February 2020

Obesity hypoventilation syndrome: Stretching the health dollar.

Authors:
Bhajan Singh

Respirology 2020 Apr 10;25(4):356-357. Epub 2019 Nov 10.

Pulmonary Physiology and Sleep Medicine, Sir Charles Gairdner Hospital, Perth, WA, Australia.

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http://dx.doi.org/10.1111/resp.13729DOI Listing

ST2 Predicts Mortality In Patients With Acute Hypercapnic Respiratory Failure Treated With Noninvasive Positive Pressure Ventilation.

Int J Chron Obstruct Pulmon Dis 2019 23;14:2385-2393. Epub 2019 Oct 23.

Department of Clinical Sciences Malmo, Faculty of Medicine, Lund University, Lund, Sweden.

Background: Patients with Acute Hypercapnic Respiratory Failure (AHRF) are often treated with Noninvasive Positive Pressure Ventilation (NPPV). In this heterogeneous patient group, there is a lack of clinical tools for predicting mortality and outcome.

Aims: In order to facilitate the choice of treatment in patients with AHRF we evaluated the protein ST2, an established biomarker for cardiac stress, and its role in predicting mortality in patients with AHRF treated with NPPV. Read More

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http://dx.doi.org/10.2147/COPD.S211448DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6815753PMC
April 2020
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