- Patterns of use, survival and prognostic factors in patients receiving home mechanical ventilation in Western Australia: A single centre historical cohort study. [Journal Article]
- CRChron Respir Dis 2018 Jan 01; :1479972318755723
- Home mechanical ventilation (HMV) is used in a wide range of disorders associated with chronic hypoventilation. We describe the patterns of use, survival and predictors of death in Western Australia....
Home mechanical ventilation (HMV) is used in a wide range of disorders associated with chronic hypoventilation. We describe the patterns of use, survival and predictors of death in Western Australia. We identified 240 consecutive patients (60% male; mean age 58 years and body mass index 31 kg m-2) referred for HMV between 2005 and 2010. The patients were grouped into four categories: motor neurone disorders (MND; 39%), pulmonary disease (PULM; 25%, mainly chronic obstructive pulmonary disease), non-MND neuromuscular and chest wall disorders (NMCW; 21%) and the obesity hypoventilation syndrome (OHS; 15%). On average, the patients had moderate ventilatory impairment (forced vital capacity: 51%predicted), sleep apnoea (apnoea-hypopnea index: 25 events h-1), sleep-related hypoventilation (transcutaneous carbon dioxide rise of 20 mmHg) and daytime hypercarbia (PCO2: 54 mmHg). Median durations of survival from HMV initiation were 1.0, 4.2, 9.9 and >11.5 years for MND, PULM, NMCW and OHS, respectively. Independent predictors of death varied between primary indications for HMV; the predictors included (a) age in all groups except for MND (hazard ratios (HRs) 1.03-1.10); (b) cardiovascular disease (HR: 2.35, 95% confidence interval (CI): 1.08-5.10) in MND;
- Severe peri-ictal respiratory dysfunction is common in Dravet syndrome. [Journal Article]
- JCIJ Clin Invest 2018 Feb 12
- Dravet syndrome (DS) is a severe childhood-onset epilepsy commonly due to mutations of the sodium channel gene SCN1A. Patients with DS have a high risk of sudden unexplained death in epilepsy (SUDEP)...
Dravet syndrome (DS) is a severe childhood-onset epilepsy commonly due to mutations of the sodium channel gene SCN1A. Patients with DS have a high risk of sudden unexplained death in epilepsy (SUDEP), widely believed to be due to cardiac mechanisms. Here we show that patients with DS commonly have peri-ictal respiratory dysfunction. One patient had severe and prolonged postictal hypoventilation during video EEG monitoring and died later of SUDEP. Mice with an Scn1aR1407X/+ loss-of-function mutation were monitored and died after spontaneous and heat-induced seizures due to central apnea followed by progressive bradycardia. Death could be prevented with mechanical ventilation after seizures were induced by hyperthermia or maximal electroshock. Muscarinic receptor antagonists did not prevent bradycardia or death when given at doses selective for peripheral parasympathetic blockade, whereas apnea, bradycardia, and death were prevented by the same drugs given at doses high enough to cross the blood-brain barrier. When given via intracerebroventricular infusion at a very low dose, a muscarinic receptor antagonist prevented apnea, bradycardia, and death. We conclude that SUDEP in patients with DS can result from primary central apnea, which can cause bradycardia, presumably via a direct effect of hypoxemia on cardiac muscle.
- [Non-alcoholic fatty liver disease, as a component of the metabolic syndrome, and its causal correlations with other extrahepatic diseases]. [Review]
- OHOrv Hetil 2017; 158(52):2051-2061
- Non-alcoholic fatty liver disease is the most common non-infectious chronic liver-disease in our age, and is a spectrum of all the diseases associated with increased fat accumulation in the hepatocyt...
Non-alcoholic fatty liver disease is the most common non-infectious chronic liver-disease in our age, and is a spectrum of all the diseases associated with increased fat accumulation in the hepatocytes. Its development is promoted by sedentary life-style, over-feeding, and certain genetic predisposition. Prevalence in the adult population, even in Hungary is ~30%. In a part of cases, this disease may pass into non-alcoholic steatohepatitis, later into fibrosis, rarely into primary hepatocellular cancer. Fatty liver is closely and bidirectionally related to the metabolic syndrome and type 2 diabetes, and nowadays there is a general consensus that fatty liver is the hepatic manifestation of the metabolic sycndrome. The importance of the fatty liver has been highly emphasized recently. In addition to the progression into steatohepatitis, its causal relationship with numerous extrahepatic disorders has been discovered. In our overview, we deal with the epidemiology, pathomechanism of the disease, discuss the possibilities of diagnosis, its relationship with the intestinal microbiota, its recently recognized correlations with bile acids and their receptors, and its supposed correlations with the circadian CLOCK system. Hereinafter, we overview those extrahepatic disorders, which have been shown to be causal link with the non-alcoholic fatty liver disease. Among these, we emphasize the metabolic syndrome/type 2 diabetes, cardiovascular disorders, chronic kidney disease, sleep apnea/hypoventilation syndrome, inflammatory bowel disease, Alzheimer's disease, osteoporosis, and psoriasis, as well. Based on the above, it can be stated, that high risk individuals with non-alcoholic fatty liver disease need systemic care, and require the detection of other components of this systemic pathological condition. While currently specific therapy for the disease is not yet known, life-style changes, adequate use of available medicines can prevent disease progression. Promising research is under way, including drugs, manipulation of the intestinal flora or the possibility of therapeutic use of bile acid receptors, and also bariatric surgery. Orv Hetil. 2017; 158(52): 2051-2061.
- StatPearls [BOOK]
- BOOKStatPearls Publishing: Treasure Island (FL)
- Acute respiratory failure is caused by a wide range of etiologies. Progression to cardiopulmonary arrest and ultimately death is likely in the absence of effective and timely airway management. Ther...
Acute respiratory failure is caused by a wide range of etiologies. Progression to cardiopulmonary arrest and ultimately death is likely in the absence of effective and timely airway management. Therefore, one of the primary goals of airway management is to provide adequate ventilation and oxygenation to avoid or halt the progression to cardiopulmonary arrest. Effective and timely airway management is also an essential component of successful cardiopulmonary resuscitation. Airway management is critical in the pediatric population as pediatric airway problems are commonly seen in pediatric and general emergency departments. Respiratory distress is the fourth most common chief complaint in children presenting to the emergency department. Initial steps in airway management include airway positioning maneuvers (for example, head-tilt-chin lift, jaw-thrust), suctioning, supplemental oxygen, and re-positioning of the airway if the previous steps are ineffective. Airway positioning maneuvers place the airway in a neutral position and help move the tongue and palatal tissues away from the posterior wall of the pharynx. When choosing an airway positioning maneuver, one must be cognizant of the possible presence or absence of a cervical spine injury. Suctioning assists with the removal of secretions that could be causing or contributing to airway obstruction. If these steps do not help in maintaining a patent airway or in providing adequate ventilation and oxygenation, then an airway adjunct should be utilized. Airway adjuncts are used to relieve or bypass an upper airway obstruction during airway management. However, upper airway obstruction may be present for several reasons, and airway adjuncts may not be able to relieve or bypass all types of obstruction. Upper airway obstruction may occur from anatomical causes such as choanal atresia, pathological causes such as a tonsillar abscess or adverse effects from patient management such as loss of airway patency during the administration of sedation and/or analgesia. There are also subsets of patients that are more prone to develop upper airway obstruction. Obese patients are at significant risk for upper airway obstruction due to altered upper airway anatomy. Pharyngeal tissues have increased fat deposition causing excess upper airway tissue and an increased likelihood of pharyngeal wall collapse resulting in airway obstruction. This can be exacerbated when obese patients are given drugs that depress the central nervous system or have other co-morbidities, such as obstructive sleep apnea (OSA) and/or obstructive hypoventilation syndrome (OHS). The presence of OSA and/or OHS can be associated with increased sensitivity to the respiratory depressant effects of sedatives and narcotics increasing the tendency to obstruct the airway. Pediatric patients, in particular infants and young children, are susceptible to upper airway obstruction. This predisposition is due to the differences between pediatric and adult airways. Infants and young children have a relatively large occiput that causes neck flexion when lying supine. This results in a natural tendency to obstruct the upper airway. They have a proportionally large tongue relative to the size of their oral cavity which also causes a natural obstruction of the airway. Additionally, a shortened thyromental distance in this patient population brings the tongue into proximity of the soft palate. Consequently, this leads to obstruction of the airway. Lastly, compared to adults, infants and young children have larger adenoidal tissue, as well as, more distensible and compliant larger airways which predisposes them to airway obstruction. In general, by the age of eight, the pediatric airway is very similar to that of an adult airway. There are two types of airway adjuncts. One is an oropharyngeal airway, and the other is a nasopharyngeal airway. This article will summarize the former.
- Congenital central hypoventilation syndrome: An overview of etiopathogenesis, associated pathologies, clinical presentation, and management. [Review]
- ANAuton Neurosci 2017 Nov 13
- Congenital central hypoventilation syndrome (CCHS), known colloquially as Ondine's curse, is a rare disorder characterized by impaired autonomic control of breathing during sleep from the loss of vag...
Congenital central hypoventilation syndrome (CCHS), known colloquially as Ondine's curse, is a rare disorder characterized by impaired autonomic control of breathing during sleep from the loss of vagal input and diminished sensitivity of CO2receptors in the medulla. CCHS correlates to the malformation of the neural crest located in the brainstem; this consequently affects the loss of sensitivity of CO2chemoreceptors, bringing about hypoventilation during sleep. The primary cause of CCHS is the mutation of the paired-like homeobox PHO2XB gene, found in 90% of the patients. This mutation not only affects breathing but also drives neurological abnormalities such as autonomic and neurocognitive dysfunction. Though typically congenital, there have been late-onset (i.e., acquired) cases reported. It is vital for physicians and clinicians to be able to diagnose CCHS due to its similar presentation to other syndromes and disorders, which may cause it to be misdiagnosed and may account for its deleterious effects. CCHS can lead to a constellation of symptoms, and consideration of diseases that present concomitantly with CCHS affords us a better understanding of the etiology of this illness. Although a rare syndrome, we aim to review the current literature to emphasize the pathogenesis, etiology, clinical presentation, symptoms, diagnosis, and current treatment methods of CCHS for clinicians to better identify and understand this condition.
- Invasive versus non-invasive ventilation for acute respiratory failure in neuromuscular disease and chest wall disorders. [Review]
- CDCochrane Database Syst Rev 2017 Dec 04; 12:CD008380
- CONCLUSIONS: Acute respiratory failure is a life-threatening complication of acute onset neuromuscular disease and of chronic neuromuscular disease and chest wall disorders. We found no randomised trials on which to elaborate evidence-based practice for the use of non-invasive versus invasive mechanical ventilation. For researchers, there is a need to design and conduct new randomised trials to compare NIV with invasive ventilation in acute neuromuscular respiratory failure. These trials should anticipate variations in treatment responses according to disease condition (acute onset versus acute exacerbation on chronic neuromuscular diseases) and according to the presence or absence of bulbar dysfunction.
- Pseudotumor cerebri syndrome in a patient with narcolepsy type 1. [Journal Article]
- EJEur J Paediatr Neurol 2018; 22(1):194-198
- Type 1 narcolepsy (NT1) is a chronic primary disorder of hypersomnolence characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, hypnagogic hallucinations and disrupted nocturnal s...
Type 1 narcolepsy (NT1) is a chronic primary disorder of hypersomnolence characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, hypnagogic hallucinations and disrupted nocturnal sleep. NT1 is linked to hypothalamic hypocretin deficiency, strongly associated with Human Leukocyte Antigen (HLA) marker DQB1*06:02 and of probable autoimmune origin. NT1 is usually associated with increased rates of overweight and obesity, and sometimes with increases in overnight blood pressure and increased rates of hypoventilation with raised CO2levels overnight. Many of these are predisposing factors for pseudotumor cerebri syndrome (PTCS). We present a case of a young girl with both NT1 and PTCS that responded well to treatment with acetazolamide after early identification, with improvement of headache and resolution of hypoventilation.
- Completed FDA feasibility trial of surgically placed temporary diaphragm pacing electrodes: A promising option to prevent and treat respiratory failure. [Journal Article]
- AJAm J Surg 2017 Nov 11
- CONCLUSIONS: This trial demonstrates ease of placement, removal, functionality and safety of temporary DP electrodes which therapeutically decreases diaphragm atrophy.
- Factors associated with change in health-related quality of life among individuals treated with long-term mechanical ventilation, a 6-year follow-up study. [Journal Article]
- JAJ Adv Nurs 2018; 74(3):651-665
- CONCLUSIONS: Long-term mechanical ventilation over 6 years improved health-related quality of life in most patients. Patient training, follow-up and reduction of side effects, largely delivered by trained nurses, contribute to achieve the main goal of the treatment-improved health-related quality of life.
New Search Next
- Supraglottic jet oxygenation and ventilation enhances oxygenation during upper gastrointestinal endoscopy in patients sedated with propofol: a randomized multicentre clinical trial. [Journal Article]
- BJBr J Anaesth 2017 Jul 01; 119(1):158-166
- CONCLUSIONS: The use of SJOV during upper gastrointestinal endoscopy for patients who are sedated with propofol reduces the incidence of hypoxia, with minor and tolerable adverse events. Supraglottic jet oxygenation and ventilation has a favourable risk-to-benefit ratio and may improve patient safety.