Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0037315 (sleep apnea)
 8,000 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Some patients with obesity show chronic hypercapnia while awake. Such patients are referred to as obesity hypoventilation syndrome(OHS). Particularly, patients with profound obesity who have clinical features of sleep disordered breathing, hypersomnolence, cor pulmonale and so on represent the Pickwickian syndrome. The mechanisms of hypoventilation in OHS are multifactorial. The level of the blunted chemosensitivity, mechanical impairments of the respiratory system, the severity of the sleep-disordered breathing, and chronic hypoxemia may be important determinants of chronic hypoventilation. In this paper, the characteristics of pulmonary functions in obesity and the possible mechanisms of hypoventilation in patients with OHS were reviewed. Furthermore, the definition of OHS and descriptions of thr severity of OHS as recommended by Respiratory Failure Research Committee of Japanese Ministry of Health and Welfare are introduced.
 ...
PMID:[Obesity and obesity hypoventilation syndrome]. 1094 42

A 6-year-old boy with Hurler's syndrome presented with right heart failure and pulmonary hypertension secondary to severe obstructive sleep apnoea. Both his sleep apnoea and cor pulmonale were effectively controlled with continuous positive airway pressure therapy.
 ...
PMID:Hurler's syndrome with cor pulmonale secondary to obstructive sleep apnoea treated by continuous positive airway pressure. 1296 15

Consequences of obstructive sleep apnea syndrome in children include reduced performance during day, behaviour problems, diurmal hypersomnia, psychomotor development delay, severe forms of cor pulmonale, systemic hypertension, growing delay and death. This paper describes the clinical case of a 3-year-old girl with perennial symptoms of nasal obstruction characterized by nocturnal snoring, oral breathing, nasal voice, sleep apnea, nasal pruritus and rhinorrhea. Her treatment is also described.
 ...
PMID:[Non-surgical treatment in case of obstructive sleep apnea syndrome in children. Report of a case]. 1496 87

Humans encounter hypoxia throughout their lives. This occurs by destiny in utero, through disease, and by desire, in our quest for altitude. Hypoxic pulmonary vasoconstriction (HPV) is a widely conserved, homeostatic, vasomotor response of resistance pulmonary arteries to alveolar hypoxia. HPV mediates ventilation-perfusion matching and, by reducing shunt fraction, optimizes systemic Po(2). HPV is intrinsic to the lung, and, although modulated by the endothelium, the core mechanism is in the smooth muscle cell (SMC). The Redox Theory for the mechanism of HPV proposes the coordinated action of a redox sensor (the proximal mitochondrial electron transport chain) that generates a diffusible mediator [a reactive O(2) species (ROS)] that regulates an effector protein [voltage-gated potassium (K(v)) and calcium channels]. A similar mechanism for regulating O(2) uptake/distribution is partially recapitulated in simpler organisms and in the other specialized mammalian O(2)-sensitive tissues, including the carotid body and ductus arteriosus. Inhibition of O(2)-sensitive K(v) channels, particularly K(v)1.5 and K(v)2.1, depolarizes pulmonary artery SMCs, activating voltage-gated Ca(2+) channels and causing Ca(2+) influx and vasoconstriction. Downstream of this pathway, there is important regulation of the contractile apparatus' sensitivity to calcium by rho kinase. Controversy remains as to whether hypoxia decreases or increases ROS and which electron transport chain complex generates the ROS (I and/or III). Possible roles for cyclic adenosine diphosphate ribose and an unidentified endothelial constricting factor are also proposed by some groups. Modulation of HPV has therapeutic relevance to cor pulmonale, high-altitude pulmonary edema, and sleep apnea. HPV is clinically exploited in single-lung anesthesia, and its mechanisms intersect with those of pulmonary arterial hypertension.
 ...
PMID:Hypoxic pulmonary vasoconstriction. 1559 9

Hepatic hydrothorax is defined as pleural effusion with liver cirrhosis but no primary cardiopulmonary disease. Hepatic hydrothorax is often resistant to various therapeutic interventions. The most likely cause is the transfer of ascites fluid from the abdomen to the pleural space via the diaphragm because of a negative intrathoracic pressure gradient. A 62-year-old man was diagnosed with hepatoma and cirrhosis. After a partial hepatectomy, he suffered with hepatic hydrothorax. He had snoring without obvious sleep apnea. The patient's hepatic hydrothorax markedly improved following nasal continuous positive airway pressure (nCPAP) treatment during sleep. The mechanism for the improvement may have been the intrathoracic positive pressure during sleep induced by the nCPAP treatment during sleep. nCPAP treatment may provide a new therapy for resistant hepatic hydrothorax.
 ...
PMID:Resistant hepatic hydrothorax: a successful case with treatment by nCPAP. 1573 99

Always take a history of snoring and sleep disturbance when reviewing children in primary care, as there is evidence that episodes of hypoxia and arousal during sleep may result in deficits in memory, attention and behaviour, in addition to the well known sequelae of growth failure, developmental delay and cor pulmonale. Check for changes in behaviour affecting school progress. To investigate for possible obstructive sleep apnoea syndrome (OSAS), clinical examination, lateral neck x-ray (adenoidal hypertrophy) and overnight oximetry (desaturation episodes) are useful screening tests, but oximetry is best used in conjunction with polysomnography. A negative oximetry test does not exclude OSAS. Polysomnography is the best method for detecting and assessing the severity of OSAS in children, and is especially helpful for prioritising treatment and evaluating the risk of perioperative complications of adenotonsillectomy. Adenotonsillectomy is thought to "cure" (ie, symptoms disappear and overnight respiratory parameters are corrected) in about 80% of children with OSAS. The remaining 20% need ongoing evaluation and treatment. Further research is needed to determine the "true" prevalence of OSAS; what degrees of severity of upper-airway obstruction lead to morbidity requiring treatment; and whether the deficits in neurocognitive function associated with sleep-disordered breathing are fully correctable.
 ...
PMID:8. Investigation and treatment of upper-airway obstruction: childhood sleep disorders I. 1585 Apr 41

Chronic cor pulmonale involves the enlargement of the right ventricle as a result of pulmonary hypertension due to pulmonary disorders involving the lung parenchyma, bellows function, or ventilatory drive. The right ventricular hypertrophy that occurs in chronic cor pulmonale is a direct result of chronic hypoxic pulmonary vasoconstriction and subsequent pulmonary artery hypertension, leading to increased right ventricular work and stress. We discuss methods by which hypoxic vasoconstriction and reduction in the pulmonary vascular bed lead to the development of pulmonary artery hypertension. This article reviews the interaction of the pulmonary vasculature and right ventricle in the non-diseased state as well as during disease exacerbations. Ventricular dependence and its contribution to the pathophysiology of right ventricular failure are also reviewed. In addition, we provide an overview of specific disease states that can result in the development of chronic cor pulmonale including chronic obstructive pulmonary disease (COPD), interstitial lung disease, sleep apnea, alveolar hypoventilation disorders, and primary pulmonary hypertension. We also review the current diagnostic studies used to evaluate and study cor pulmonale.
 ...
PMID:Cor pulmonale: an overview. 1608 45

Cor pulmonale frequently develops in patients with restrictive lung disease and neuromuscular disorders. Sleep disordered breathing, including nocturnal hypoventilation and obstructive apnea, has been associated with the development of cor pulmonale and may affect morbidity. The mechanisms responsible for sleep disordered breathing include defects in the control of breathing, respiratory muscle dysfunction, and abnormalities in chest wall and lung compliance. Symptoms of disturbed sleep may allow patients with sleep disordered breathing to be appropriately diagnosed and treated, often with nocturnal ventilation, before the development of right-sided heart failure.
 ...
PMID:Cor pulmonale and sleep-disordered breathing in patients with restrictive lung disease and neuromuscular disorders. 1608 50

Secondary pulmonary hypertension (SPHtn) is generally attributable to abnormalities in structure or function of the heart or lung parenchyma. While often defined as a physiologic parameter, pulmonary hypertension (PHtn) can be a major contributor to death and disability in cardiopulmonary diseases. Both detection and management are a challenge. We will review the pathophysiology, diagnostic tools, and treatment strategies in SPHtn with an emphasis on cor pulmonale associated with chronic obstructive pulmonary disease (COPD), pulmonary vasculopathies, and pulmonary embolus. The pathophysiology and common etiologies of SPHtn can be divided into three major categories: (1) elevated pulmonary venous pressure (LV failure and mitral valve disease), (2) pulmonary vascular occlusive disease with or without pulmonary parenchymal disease (pulmonary emboli, COPD, connective tissue diseases), and (3) hypoxemia (sleep apnea). The echo-Doppler is a simple cost-effective tool for detecting PHtn, evaluating right ventricular function, and distinguishing common etiologies such as abnormal systolic and diastolic left ventricular function and mitral valve disease. The ventilation-perfusion radionuclide scan can be used to exclude thromboembolic PHtn, but a helical computer tomography with contrast or pulmonary angiography are necessary to distinguish patients that may benefit from a pulmonary thromboendarterectomy. The six minute walk oxygen saturation test is useful as a quantitative measure of functional capacity, prognosis, response to therapy, and oxygen requirement. Treatment strategies in cor pulmonale are tailored to the specific diagnosis, but generally include proper nutrition, exercise, oxygen supplementation, medications such as digoxin, diuretics, anti-coagulation, and pulmonary vasodilator therapy in selected patients.
 ...
PMID:Secondary pulmonary hypertension--diagnosis and management. 1647 37

Recombinant human alpha-L-iduronidase (Aldurazyme, laronidase) was approved as an enzyme replacement therapy for patients with the lysosomal storage disorder, mucopolysaccharidosis I (MPS I). In order to assess the long-term safety and efficacy of laronidase therapy, 5 of 10 patients in the original laronidase Phase 1/2 clinical trial were re-evaluated after 6 years of treatment. Lysosomal storage was further improved at 6 years (urinary glycosaminoglycans (GAG) excretion decreased 76%; mean liver size at 1.84% of body weight). Shoulder maximum range of motion was maintained or further increased and reached a mean 33.2 (R) and 25.0 (L) degrees gained in flexion and 34.0 (R) and 27.3 (L) degrees gained in extension. Sleep apnea was decreased in four of five patients and the airway size index improved. Cardiac disease evaluations showed no progression to heart failure or cor pulmonale but pre-existing significant valve disease did progress in some patients. Substantial growth was observed for the pre-pubertal patients, with a gain of 33 cm (27%) in height and a gain of 31 kg in weight (105%). In general, the evaluated patients reported an improved ability to perform normal activities of daily living. Overall these data represent the first evidence that laronidase can stabilize or reverse many aspects of MPS I disease during long-term therapy and that early treatment prior to the development of substantial cardiac and skeletal disease may lead to better outcomes.
 ...
PMID:A follow-up study of MPS I patients treated with laronidase enzyme replacement therapy for 6 years. 1701 Dec 23


<< Previous 1 2 3 4 5 Next >>