Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020440 (hypercapnia)
 7,939 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 38-year-old patient with effort dyspnea, somnolence, cianosis and cor pulmonale is presented. Chest roentgenograms and lung function studies suggested the diagnosis of pulmonary fibrosis. The patient showed also severe hypercapnia with normal resting ventilation and ventilatory response to exercise lower than usual for this condition. Autopsy confirmed the clinical diagnosis. This subject may belong to the growing group of patients where CO2 retention is not explained by their pulmonary pathology.
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PMID:Idiopathic interstitial pulmonary fibrosis with hypercapnia. 117 39

The Medical Research Council and the Nocturnal Oxygen Therapy Trial studies clearly demonstrated that long-term oxygen therapy (LTOT) for more than 15 h/day improved mortality and morbidity in a well-defined group of patients with chronic obstructive pulmonary disease. There are no similar randomised control studies in patients with other hypoxaemic lung diseases such as pulmonary fibrosis and pneumoconiosis. The prescription of oxygen for other restrictive lung disorders is complicated by hypoventilation requiring mechanical support as well as oxygen and should be restricted to special centres. The clearest indications for LTOT are for patients with cor pulmonale, hypoxic chronic bronchitis and emphysema, and in terminally ill patients who require palliation. Before LTOT is considered, the patient must be clinically stable and on appropriate optimum therapy such as antibiotics, bronchodilators, physiotherapy and having stopped smoking tobacco. Many patients first present for LTOT with profound hypoxaemia and hypercapnia during an infective, often oedematous exacerbation of their lung disease. Assessments should occur during convalescence when the patient is clinically stable. They should be shown to have a PaO2 less than 7.3 kPa and/or a PaCO2 greater than 6 kPa on two occasions at least 3 weeks apart. FEV1 should be less than 1.5 litres, and there should be a less than 15% improvement in FEV1 after bronchodilators. All patients should be assessed by an experienced chest physician. Patients with a PaO2 between 7.3 and 8 kPa who have polycythaemia, right heart failure or pulmonary hypertension may gain benefit from LTOT but this is still to be clearly proven.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Indications for long-term oxygen therapy. 151 74

A progressive pulmonary disease resulting in severe respiratory failure and death in an average of 3 weeks was diagnosed in 11 young Dalmatian dogs. The dogs were from 4 litters, all genetically related by a common ancestor. The initial clinical signs were tachypnea and noisy respiration. Respiratory distress developed shortly before death and was characterized by strenuous and rapid respirations, along with cyanosis and vomiting. On blood gas analysis, there were severe arterial hypoxemia, hypercapnia, and marked alveolar-arterial oxygen difference. Radiographically, a diffuse pattern of alveolar, interstitial, and peribronchial densities was observed in the lungs. Most dogs developed pneumomediastinum and gastroesophageal intussusception in the terminal phase of the disease. There was no response to treatment with antibiotics, corticosteroids, diuretics, or oxygen. At necropsy, the lungs were wet, heavy, and relatively airless. Absence of 1 kidney in 2 dogs and severe internal hydrocephalus in 2 dogs were additional necropsy findings. Pulmonary histopathology included metaplasia and atypia of the alveolar and bronchiolar epithelium, a nonpurulent inflammatory reaction characterized mainly by mononuclear cells and macrophages, eosinophilic hyaline membrane formation, and focal pulmonary fibrosis. The histological manifestations were typical of acute lung injury. Clinically, the findings were consistent with adult respiratory distress syndrome (ARDS), except for the relatively long course. No known risk factors for ARDS, such as trauma, toxin exposure, infection, or endotoxemia could be identified. The relationship of the other abnormalities (ie, renal aplasia, hydrocephalus) to the pulmonary disease also remains obscure. An inherited defect is suspected, because segregation analysis of the 4 litters suggests autosomal recessive inheritance.
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PMID:Lung injury leading to respiratory distress syndrome in young Dalmatian dogs. 767 17

Acute respiratory distress syndrome (ARDS) is an acute form of severe alveolar-capillary injury that evolves after a direct or indirect lung insult. It begins as noncardiogenic pulmonary edema and develops into a neutrophilic alveolitis, and, later, pulmonary fibrosis. Mortality remains high among children with ARDS, particularly when serious underlying conditions co-exist, sepsis occurs, and when there is multi-organ failure. Lung function improves with time among survivors, but pulmonary fibrosis may persist. Advances in the care of children with ARDS include the use of lung-protective ventilator strategies, permissive hypercapnia, inhaled nitric oxide, high-frequency ventilation, and extra-corporeal life support. These approaches reduce ventilator-associated lung injury and may improve survival when used in combination with one another. Interventions that reduce alveolar inflammation, enhance alveolar fluid removal, and reduce pulmonary fibrosis will further improve survival and recovery from ARDS in the future.
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PMID:Current concepts in adult respiratory distress syndrome in children. 1138 62

Chronic lung disease (CLD) or bronchopulmonary dysplasia is a recognized sequel of preterm birth. With improving survival of infants at lower gestational ages, the incidence is on the rise. Pathological features of CLD include alveolar maldevelopment, with or without areas of pulmonary fibrosis. Assisted ventilation, infection/inflammation, oxygen administration, and fluid overload are the major risk factors in the evolution of CLD.Interventions, including the treatment of maternal infection, administration of prenatal glucocorticoids, and postnatal surfactant replacement therapy, improve the survival of preterm infants; however, their effect on CLD is difficult to determine. Strategies that have been effective in reducing CLD are the administration of retinol (vitamin A), high frequency oscillatory ventilation, and administration of glucocorticoids. Previous concerns regarding neurological problems associated with high frequency ventilation have not been substantiated in recent studies. Current recommendations do not advise the routine use of glucocorticoids due to concerns regarding long-term neurodevelopment. Therapies that were found to be ineffective in reducing the incidence of CLD include prenatal thyrotropin, cromolyn sodium (sodium cromoglycate), alpha-1 antitrypsin, superoxide dismutase, tocopherol (vitamin E), ascorbic acid (vitamin C), allopurinol, ambroxol, inositol, inhaled bronchodilators, and fluid restriction. Strategies that may be effective in reducing lung injury and subsequent CLD include avoiding assisted ventilation, lung protective ventilatory maneuvers, permissive hypercapnia, prevention of infection, early aggressive nutrition, and the treatment of a patent ductus arteriosus. The use of inhaled glucocorticoids improves pulmonary dynamics but long-term effects are unknown. The management of infants with established CLD has not been studied adequately, and the role of various ventilatory strategies for infants with established CLD is not clear. Adequate oxygenation should be maintained to prevent hypoxic episodes. Diuretics are helpful during acute decompensation; however, their long-term impact has not been well studied. Provision of adequate nutrition, immunization (routine and against respiratory syncytial virus), follow-up, and monitoring are the key elements in the long-term management of infants with CLD. Future research priorities should be to identify strategies to prevent/treat inflammation and promote the healing processes in the injured lung. The long-term effects of lung-protective ventilation strategies need to be studied.
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PMID:Current perspectives on the prevention and management of chronic lung disease in preterm infants. 1283 19

The objective of the present study was to examine the impact of early stages of lung injury on ventilatory control by hypoxia and hypercapnia. Lung injury was induced with intratracheal instillation of bleomycin (BM; 1 unit) in adult, male Sprague-Dawley rats. Control animals underwent sham surgery with saline instillation. Five days after the injections, lung injury was present in BM-treated animals as evidenced by increased neutrophils and protein levels in bronchoalveolar lavage fluid, as well as by changes in lung histology and computed tomography images. There was no evidence of pulmonary fibrosis, as indicated by lung collagen content. Basal core body temperature, arterial Po(2), and arterial Pco(2) were comparable between both groups of animals. Ventilatory responses to hypoxia (12% O(2)) and hypercapnia (7% CO(2)) were measured by whole body plethysmography in unanesthetized animals. Baseline respiratory rate and the hypoxic ventilatory response were significantly higher in BM-injected compared with control animals (P = 0.003), whereas hypercapnic ventilatory response was not statistically different. In anesthetized, spontaneously breathing animals, response to brief hyperoxia (Dejours' test, an index of peripheral chemoreceptor sensitivity) and neural hypoxic ventilatory response were augmented in BM-exposed relative to control animals, as measured by diaphragmatic electromyelograms. The enhanced hypoxic sensitivity persisted following bilateral vagotomy, but was abolished by bilateral carotid sinus nerve transection. These data demonstrate that afferent sensory input from the carotid body contributes to a selective enhancement of hypoxic ventilatory drive in early lung injury in the absence of pulmonary fibrosis and arterial hypoxemia.
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PMID:Acute lung injury augments hypoxic ventilatory response in the absence of systemic hypoxemia. 1688 52

Breathing occurs in single breaths and in patterns which are altered by the onset, progression and resolution of respiratory diseases. Through modulations of rate, depth, and patterning of breathing, the ventilatory control system maintains numerous critical variables within their homeostatic ranges. A dynamic respiratory control system is critical to successful adaptation in the face of progressive pulmonary pathology. The objective of this review, is to illustrate functional changes and compensatory mechanisms which occur with the onset and progression of acute and chronic lung disease. Chronic obstructive pulmonary disease (COPD) will be considered as a model of a slowly progressive pulmonary process, where destruction of lung parenchyma and airway obstruction leads to hypoxemia and hypercapnia. Over time, adaptations of the respiratory control system to this disease include changes in the intrinsic properties of respiratory muscles, chemoreceptor signaling, and central respiratory drive which increase motor output to the respiratory muscles. In contrast, acute respiratory distress syndrome (ARDS) is an exemplar of an acute pulmonary process. The result of severe lung injury, ARDS is characterized by lung infiltrates, rapidly progressive hypoxemic respiratory failure, and possible progression to pulmonary fibrosis. Changes in breathing patterns result from these functional changes, as well as altered processing of afferent feedback by the central controller, possibly influenced by brainstem inflammation. Taken together, these disease models highlight the plasticity of the respiratory control system in response to the development and progression of lung disease.
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PMID:Control of ventilation in COPD and lung injury. 2385 86

Kenny Caffey syndrome (KCS) is a rare syndrome reported almost exclusively in Middle Eastern populations. It is characterized by severe growth retardation-short stature, dysmorphic features, episodic hypocalcaemia, hypoparathyroidism, seizures, and medullary stenosis of long bones with thickened cortices. We report a 10-year-old boy with KCS with an unusually severe respiratory and gastrointestinal system involvement-features not previously described in the literature. He had severe psychomotor retardation and regressed developmentally from walking unaided to sitting with support. MRI brain showed bilateral hippocampal sclerosis, marked supra-tentorial volume loss and numerous calcifications. A 12 bp deletion of exon 2 of tubulin-specific chaperone E (TBCE) gene was identified and the diagnosis of KCS was confirmed. Hypercarbia following a sleep study warranted nocturnal continuous positive airway pressure (CPAP) when aged 6. When boy aged 8, persistent hypercarbia with increasing oxygen requirement and increased frequency and severity of lower respiratory tract infections led to progressive respiratory failure. He became fully dependent on non-invasive ventilation and by 9 years he had a tracheotomy and was established on long-term ventilation. He developed retching, vomiting and diarrhea. Chest CT showed changes consistent with chronic aspiration, but no interstitial pulmonary fibrosis. He died aged 10 from respiratory complications.
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PMID:Kenny Caffey syndrome with severe respiratory and gastrointestinal involvement: expanding the clinical phenotype. 2602 52