FIBRotic Interstitial Lung Disease and Nocturnal OXygen
Idiopathic Pulmonary FibrosisObstructive Sleep Apnea2 moreHome sleep studies - which allow the measurement of breathing while the person sleeps - will be performed on patients with fibrotic interstitial lung disease attending two of the UK's largest respiratory medicine services.The study will investigate at how symptoms, and breathing and exercise tests differ between these two groups after 12 months of study.
Obesity Hypoventilation Syndrome Prevalence Study
Obesity Hypoventilation SyndromeThis is an observational study to characterize the prevalence of Obesity Hypoventilation Syndrome in patients referred to the sleep lab.
Assessment of Therapeutic Education About Non Invasive Ventilation in Patients With Chronic Respiratory...
Alveolar HypoventilationThe purpose of the study is to assess the therapeutic education of patients with chronic respiratory failure who start to be treated with non invasive ventilation and during the first six months of ventilation.
Prevalence of Nocturnal Hypoventilation in Neuromuscular Diseases According to Different Definitions...
Neuromuscular DiseaseDifferent definitions of nocturnal hypoventilation are used in the recent literature. The investigators aimed to compare the prevalence of nocturnal hypoventilation in a neuromuscular disease's population according to different definitions issued from the literature.
Monitoring of Non-invasive Ventilation
HypoventilationThe purpose of this study is to determine whether clinical examination, report from ventilator software, nocturnal Sp02 and daytime arterial blood gas are sufficient as screening tools to reveal clinical relevant problems with patients receiving long term treatment with non invasive ventilation.
Late-onset Congenital Central Hypoventilation Syndrome and the Mutation of Phox2B Gene
Congenital Central Hypoventilation SyndromeCongenital central hypoventilation syndrome (CCHS) is a rare disorder of respiratory control characterized by ventilatory impairment that results in arterial hypoxemia. Although patients typically present this disease as newborns and rarely in later infancy, there have been reports of patients presenting with CCHS in adulthood. The present study reports a unique familial case in which the father (proband) presented late-onset CCHS with an expansion mutation of the Phox2B gene that was confirmed by genetic analysis. Surprisingly, the proband did not report any manifestation of the disease during childhood, and the disease progressed following an insidious course until adulthood. At the time of diagnosis, he did not present signs of pulmonary hypertension and right-side heart failure. The patient responded well to nocturnal invasive ventilation. In contrast, his son presented CCHS as a newborn with the full complement of symptoms while his daughter did not. The present report shows that CCHS cases characterized by a mutated Phox2 gene can progress without many symptoms and that the treatment approach used here was efficient for controlling the course of the disease. Furthermore, this case indicates that incomplete penetrance can occur. Genetic screening of family members is mandatory to evaluate the reproductive risk of the disease, especially because asymptomatic mutation carriers may be at high risk to develop the disease and transmit it to the next generation.
Transcutaneous Measurement of Carbon Dioxide Versus Current Sedation Practices in Sedated Patients...
ColonoscopyDoes transcutaneous carbon dioxide partial pressure (TcCO2) monitoring detect hypoventilation prior to current sedation monitoring practices in order to prevent hypoxemia in sedated patients undergoing colonoscopies?
Factors Associated With Chronic Respiratory Failure in Obesity
ObesityObesity Hypoventilation SyndromeSome overweight individuals develop problems with their breathing such that they gradually breathe less and less. This leads to a lack of oxygen and a buildup of carbon dioxide in the blood, called ventilatory failure. As a consequence, if such a person develops a chest infection, they are more likely to become seriously ill and need intensive care. In addition they are much more likely to develop severe complications during and following operations. This problem can be treated with a machine at home used overnight to help breathing. It is interesting that ventilatory failure only happens in some overweight individuals, and the investigators do not understand what factors make this complication develop. There are a number of theories: for example the distribution of the fat, additional lung disease (such as asthma), the addition of obstructive sleep apnoea, a condition when there are periods of cessation of breathing overnight (which is more common in obese individuals), weak muscles of breathing (perhaps due to fatty infiltration of muscles or vitamin D deficiency), and other hormonal changes. The investigators intend to measure many potential factors in a range of overweight individuals, some who have ventilatory failure, and some who do not, to try and work out which are the important factors that cause this problem. If the investigators can identify such factors, then this will help predict in advance who is at risk from chest infections and during operations; thus allowing for earlier provision of an overnight breathing machine. This should reduce complications and potentially deaths in such individuals.
Usefulness of Transcutaneous Capnography to Assess Residual Hypoventilation in Obesity-Hypoventilation...
Obesity Hypoventilation SyndromeTranscutaneous capnography (PtcCo2) recently showed good agreement with blood gases to assess hypercapnia. There is no study that has evaluated the benefit to realise systematically a nocturnal PtcCo2 in the follow-up of home-ventilated patient with obesity hypoventilation syndrome (OHS). The investigators will evaluate the intake bring by nocturnal transcutaneous capnography, compared to nocturnal oximetry and blood gases, to diagnose nocturnal alveolar hypoventilation in a population of patient presenting an OHS, treated by non invasive ventilation (NIV) at home .
Respiratory Muscle Endurance in Obesity Hypoventilation Syndrome
Obesity Hypoventilation Syndrome (OHS)Obesity hypoventilation syndrome (OHS) is defined as a combination of obesity [body mass index (BMI) ≥30 kg/m2], chronic daytime hypercapnia (PaCO2 >45 mm Hg), and sleep-apnea in the absence of other known causes of hypercapnia. Respiratory system compliance decreases and resistance increases in OHS. This causes increase in work of breathing and oxygen cost of breathing, which may result in respiratory muscle fatigue. Increase in respiratory workload and increase in resistance to respiration is expected to decrease in respiratory muscle endurance (RME) in subjects with OHS.