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

A 68-year-old man with severe dyspnea was admitted as an emergency case. He had no past history of any respiratory or neuromuscular diseases. Immediately after insufflation of oxygen, respiratory arrest occurred. The blood gas analysis showed hypoxemia and severe hypercapnia (PaO2; 32 mmHg, PaCO2; 127 mmHg). We diagnosed as CO2 narcosis, and he was treated with a respirator in the ICU. He showed nonflaccid bilateral diaphragmatic paralysis and muscle atrophy of the upper extremities. As the EMG showed giant spikes of neurogenic pattern, he was diagnosed as ALS. Weaning from the respirator failed because of his respiratory muscle fatigue. He was given rehabilitation during the day time and ventilatory support with the respirator during the night. We conclude that if we meet with an emergency patient with CO2 narcosis without any pulmonary disorder, we have to suspect neuromuscular diseases, e.q. ALS. In some of such cases, mechanical ventilation supports social rehabilitation.
 ...
PMID:[A case of emergency admission for CO2 narcosis in a patient with amyotrophic lateral sclerosis]. 852 59

Alveolar hypoventilation associated with neuromuscular disease can occur in acute and chronic forms. In the acute form, progressive weakness of respiratory muscles leads to rapid reduction in vital capacity followed by respiratory failure with hypoxemia and hypercarbia. Symptoms are those of acute respiratory failure, including dyspnea, tachypnea, and tachycardia. In the chronic form, impairment of the respiratory muscles affects mechanical properties of the lungs and chest wall, decreases the ability to clear secretions, and eventually may alter the function of the central respiratory centers. Symptoms include orthopnea, fatigue, disturbed sleep, and hypersomnolence. Treatment and outcome of the disease's chronic form are dependent on the underlying clinical cause of the alveolar hypoventilation. For chronic but stable diseases such as old polio, quadriplegia, or kyposcoliosis, mechanical support of minute ventilation can reverse symptoms. For chronic and progressive disease such as muscular dystrophy and amyotrophic lateral sclerosis, mechanical support of minute ventilation provides only symptomatic relief and is usually associated with deterioration to the point of complete ventilator dependency for survival. For the chronic progressive forms of alveolar hypoventilation, there is currently a need for quality randomized controlled clinical trials to define physiologic indicators and appropriate timing for mechanical support of minute ventilation.
 ...
PMID:Neuromuscular disease and hypoventilation. 1057 Jul 36

Intermittent non-invasive home ventilation is expected to improve the quality of life, but progression of underlying neuromuscular diseases may lead to a deterioration. We observed after 82-1085 days of such home mechanical ventilation (7 patients with muscular dystrophy (age [mean/std.) 33 +/- 15 years), 8 patients with amyotrophic lateral sclerosis (ALS. age 60 +/- 8 years) a nonsignificant decrease of vital capacity (1.6 +/- 0.4 rp. 1.2 +/- 0.4 l) and an improvement of hypercapnia (49.3 +/- 8.5 rp. 43 +/- 18.5 mmHg). Quality of life (SF-36, Medical Outcomes Trust, Boston, USA) increased significantly only for mental health in patients with ALS (55 +/- 13 rp. 64 +/- 17%). Despite progression of the underlying disease the quality of life remained stable under home mechanical ventilation and mental health improved.
 ...
PMID:[Intermittent assisted ventilation in neuromuscular diseases: course and quality of life]. 1061 50

Respiratory complications account for the majority of deaths occurring in patients suffering from amyotrophic lateral sclerosis (ALS). Patients normally succumb to their illness within an average of 3 to 5 years from the time of diagnosis from complications such as hypoventilation, hypoxemia, hypercarbia, aspiration, and other pneumonia and pulmonary emboli. Although invariably disabling, ALS need not be fatal if respiratory involvement is detected early, which will allow sufficient time to discuss and implement treatment options. The recently published American Academy of Neurology guidelines for the management of ALS recommends the following: Serial measures of pulmonary function to guide management and determine prognosis. Noninvasive ventilatory support--an effective initial therapy for symptomatic chronic hypoventilation and prolonged survival. Invasive ventilatory support when long-term survival is the goal and noninvasive support is no longer sufficient. Physicians respect the right of the patient to choose, refuse, or withdraw ventilatory support. Liberal use of opiates and anxiolytics to relieve dyspnea and anxiety when ventilatory support is refused or withdrawn.
 ...
PMID:Respiratory Failure or Impairment in Amyotrophic Lateral Sclerosis. 1118 Jul 50

Although ventilatory failure is the most common cause of death in amyotrophic lateral sclerosis (ALS) and measurement of respiratory muscle strength (RMS) has been shown to have prognostic value, no single test of strength can predict the presence of hypercapnia reliably. RMS was measured in 81 ALS patients to evaluate the relationship between tests of RMS and the presence of ventilatory failure, defined as a carbon dioxide tension > or = 6 kPa. We studied the predictive value of vital capacity (VC), static inspiratory and expiratory mouth pressures (MIP, MEP), maximal sniff oesophageal (sniff P(oes)), transdiaphragmatic (sniff P(di)) and nasal (SNP) pressure, cough gastric (cough P(gas)) pressure and transdiaphragmatic pressure after bilateral cervical magnetic phrenic nerve stimulation (CMS P(di)) to identify the risk of ventilatory failure in the whole group and in subgroups of patients with and without significant bulbar involvement. For patients without significant bulbar involvement, sniff P(di) had greatest predictive power [odds ratio (OR) 57] with specificity, sensitivity and positive and negative predictive values (PPV, NPV) of 87, 90, 74 and 95%, respectively Of the less invasive tests, per cent predicted SNP had greater overall predictive power (OR 25, specificity 85%, sensitivity 81%) than per cent predicted VC (9, 89%, 53%) and per cent predicted MIP (6, 83%, 55%). No test had significant predictive power for the presence of hypercapnia when used to measure RMS in a subgroup of patients with significant bulbar weakness. Thirty-five patients underwent polysomnography. CMS P(di), sniff P(di) and per cent predicted SNP were significantly correlated with the apnoea/hypopnoea index (AHI) (P = 0.035, 0.042 and 0.026, respectively). The correlations between AHI and per cent predicted MIP and VC were less strong (both non-significant). In ALS patients without significant bulbar involvement, novel tests of RMS have greater predictive power than conventional tests to predict hypercapnia. In particular, the non-invasive SNP is more sensitive than VC and MIP, suggesting that it could usefully be included in tests of respiratory muscle strength in ALS and will be helpful in assessing the risk of ventilatory failure. In patients with significant bulbar involvement, tests of respiratory muscle strength do not predict hypercapnia. Sleep-disordered breathing is correlated with RMS and the novel tests of RMS having the strongest relationship with the degree of sleep disturbance.
 ...
PMID:Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis. 1157 Dec 18

Home ventilation is a growth area. Rapid expansion during the 1990s was stimulated by the development of noninvasive ventilation (NIV) via a mask and the recognition that an increased number of patient groups can benefit. Although patients receiving NIV in the home outnumber those receiving invasive ventilation via tracheostomy, there is substantial variation in practice between European countries. Evidence that individuals who develop ventilatory failure as a consequence of chest wall disease or stable neuromuscular disease such as old poliomyelitis benefit from nocturnal NIV is overwhelming. Patients with progressive neuromuscular disease such as Duchenne muscular dystrophy and amyotrophic lateral sclerosis can also derive prolongation of life, palliation of symptoms and an improvement in quality of life. Home ventilation in chronic obstructive pulmonary disease (COPD) patients remains controversial. Multicentric randomised controlled trials of long-term oxygen therapy (LTOT) versus NIV plus LTOT in COPD have produced mixed results, although certain subgroups, e.g. those with recurrent infective exacerbations requiring short-term NIV, patients aged >65 yrs, and those with uncontrolled hypercapnia on LTOT or symptomatic nocturnal hypoventilation, may benefit. At the other end of the age spectrum, children as young as a few months can be successfully treated with noninvasive ventilation. Most work on paediatric home ventilation centres on children with congenital neuromuscular disease. Pressure preset bilevel ventilators are now the dominant form of ventilator in adults and children. Discharge planning is vital for the home ventilator patient and a sensible risk management strategy should be in place.
 ...
PMID:Home ventilation. 1462 Nov 16

We investigated neuro-respiratory function of a 60-year-old woman with a 7-year-history of amyotrophic lateral sclerosis. She was trachomized 4 years ago, however, spontaneous respiration had been observed. Plethysmography disclosed autonomous and abolished volitional control on breathing. Cough reflex and emotional laughing influenced on respiration pattern. Positive reaction of respiration to hypercapnia was observed. Then, it seemed to be preserved respiratory neurons in the medulla oblongata and bulbospinal tract, and remarkable impairement of the corticospinal tract.
 ...
PMID:[Selective volitional respiration control loss (inverse Ondiene's curse syndrome) in amyotrophic lateral sclerosis]. 1550 36

Amyotrophic lateral sclerosis (ALS) is a disease with progressive muscle weakness, also affecting respiratory muscles. In the terminal phase most patients experience a progression. Nutrition, speech and breathing capacity decrease. It is important to inform the patient and relatives in time and to give them a chance to decide. "Care Planning" and "Advance Directives" especially concerning ventilation reduces fear and helps the doctors and carers to decide, following the will of the patient. Nobody knows the speed of the progression. The patient in this case had few subjective symptoms at the time of the family conference. Progression till death lasted one month only. Treatment of his dyspnoe was not optimised, but during care all decisions were based on the actual will of the patient. Generally nocturnal hypoventilation, for instance non-invasive ventilation by BiPAP-mode, can relieve symptoms of dyspnoe in ALS patients. Low-dose morphine and/or benzodiazepine relieve respiratory discomfort and remove the negative spiral of dysnoe-fear-dyspnoe. Oxygen therapy is usually not needed (only in the very last stages of the disease) and is not recommended especially during the night. Hypercapnia can occur because of hypoventilation. This can cause growing unconsciousness and maybe death during sleep. Prolonging life is only possible by invasive long-term ventilation with all the problems of intensive care measures. The patient could have been given low dose morphine from the time of the family conference. Ventilation by CPAP-mode was insufficient for him.
 ...
PMID:[Amyotrophic lateral sclerosis--when planning is almost too late]. 1683 Feb 50

Respiratory muscle weakness represents the major cause of mortality in patients with amyotrophic lateral sclerosis (ALS). As a result, ventilatory assistance is an important part of disease management. Nowadays, noninvasive ventilation (NIV) has become the first choice modality for most patients and represents an alternative to tracheostomy intermittent positive-pressure ventilation. Although, some consensus guidelines have been proposed to initiate NIV in patients with restrictive chronic respiratory failure, these criteria are discussed regarding ALS. While the current consensus recommends that NIV may be used in symptomatic patients with hypercapnia or forced vital capacity<50p.cent of predicted value, early use of NIV is proposed in the literature and reported in this paper.
 ...
PMID:[Types of ventilatory support and their indications in amyotrophic lateral sclerosis]. 1712 21

Mechanical ventilation has become an important treatment option in chronic ventilatory failure. There are different diseases which lead to ventilatory failure and to home mechanical ventilation (HMV). A primary loss of in- and expiratory muscle strength is the reason for respiratory deterioration in neuromuscular disease. In most of these diseases ventilatory failure develops because of the progressive character of muscular damage. Initially, ventilatory failure can be found during night-time. In the case of hypercapnia at daytime, life expectancy is strongly reduced, especially in amyotrophic lateral sclerosis and Duchenne muscular dystrophy. HMV leads to a prolongation of life and to an increase in quality of life, if bulbar involvement is not severe. Impressive clinical improvements under HMV have been found in restrictive disorders of the rib cage like kyphoscoliosis or posttuberculosis sequelae, with an increase of quality of life, walking distance and a decrease in pulmonary hypertension. Only few data are published about long-term results of HMV in Obesity Hypoventilation. In terms of retrospective analyses of clinical data HMV seems to improve survival in this population. Some patients only need CPAP treatment, but most patients have to be treated with ventilatory support. The application of HMV in patients with chronic ventilatory failure due to chronic obstructive pulmonary disease (COPD) is growing, but there are controversial results in randomised clinical trials. Analysis of these data suggest better results of HMV in patients with severe hypercapnia, with the application of higher effective ventilatory pressure and a ventilator mode with a significant reduction in the work of breathing. Under such conditions HMV leads to a reduction of hypercapnia, an improvement in sleep quality, walking distance and quality of life, but until now there is no evidence in reduction of mortality in COPD.
 ...
PMID:[Mechanical ventilation in chronic ventilatory insufficiency]. 1762 Feb 31


1 2 Next >>