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Query: UMLS:C0020440 (hypercapnia)
 7,939 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nocturnal nasal intermittent positive pressure ventilation (nIPPV) has been used successfully in the management of patients with respiratory failure due to chest wall deformity and neuromuscular disease. In order to determine if nIPPV is useful in patients with cystic fibrosis (CF) complicated by respiratory failure, we treated four hypercapnic patients for up to 18 months. All patients had failed to respond to intensive conventional therapy, including nocturnal nasal CPAP in three of the patients. Within a few days of commencing nIPPV, all reported improved length and quality of sleep. There was lessening of the degree of hypercapnia and an increase in respiratory muscle strength. After stabilization in the hospital, all patients were able to be discharged home receiving nocturnal assisted ventilation. The improvements seen in these patients have been maintained for up to 18 months. We believe nIPPV offers an effective therapeutic approach for patients with end-stage CF in hypercapnic respiratory failure and may be particularly advantageous for those awaiting heart-lung transplant.
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PMID:Nocturnal nasal IPPV stabilizes patients with cystic fibrosis and hypercapnic respiratory failure. 151 13

As more women with cystic fibrosis (CF) live to childbearing age, more become pregnant and deliver healthy infants. A 1980 review shows 129 pregnancies in 100 CF women. 80% were full term. The perinatal mortality rate was 8.5% (almost all deaths were premature infants). 18% of the mothers dies within 2 years of delivery, but none died during pregnancy. This mortality rate matched the expected rate for nonpregnant CF women at the same age. If CF women are in sound health and want to have children, physicians should encourage them to do so. Despite rumors to the contrary and theoretical problems with dehydrated cervical mucus, women with mild CF have little difficulty conceiving. Overall contraception issues are the same for both CF women and non-CF women. A few differences do exist, however. CF women should prevent unwanted pregnancy because an abortion poses special risks for them and the child adds more demands on a woman who often needs hospital care. Unpredictable absorption in the intestines makes oral contraceptives unreliable for CF women. A CF woman must consider timing, family support, and genetics of the father when planning a pregnancy. Pregnancy may not affect lung function greatly because lung volume in CF women depends on the condition of the airways rather than the size of the thoracic cage. In pregnant women with severe CF, minute ventilation cannot rise enough so hypercapnia occurs, and blood volume and cardiac output may increase 50% in the 3rd trimester. All these changes could trigger cor pulmonale in these women. Pregnancy is contraindicated for CF women with raised PaCO2, and SaO2 of 90%, and cor pulmonale. Physicians should manage pregnant CF women the same as they would other women, but increase emphasis on controlling pulmonary infection and adequate nutrition. They should also avoid teratogenic drugs and drugs with no proven record.
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PMID:Cystic fibrosis and pregnancy. 159 39

The case histories of six cystic fibrosis patients awaiting heart-lung transplantation are reviewed. They all deteriorated with severe hypoxia and hypercapnia before donor organs became available. Nasal intermittent positive pressure ventilation was used in preference to conventional ventilation with excellent results in four patients. There were no episodes of hypotension or toxaemia and the patients were in a stable condition at the time of surgery and made an excellent post-operative recovery. The patients who were transplanted and the patient who died, for whom suitable donor organs did not become available, probably had a more comfortable time than they would have done if treated with conventional ventilation. This method of ventilation appears to be a useful bridge to transplantation when a patient suddenly deteriorates. It gives them a chance of survival for a few more days or even weeks during which time an urgent search for donor organs can be made. This is also a very cost effective method of ventilation and does not encroach on conventional Intensive Care Unit (ICU) facilities.
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PMID:Non-invasive mechanical ventilation for cystic fibrosis patients--a potential bridge to transplantation. 193 22

Exercise training has been considered suitable only in cystic fibrosis (CF) patients with mild to moderate pulmonary dysfunction without progressive hypoxaemia during exercise. We trained 16 CF patients, all with advanced lung disease (mean standardized forced expiratory volume in 1 s (FEV1), 30% pred.), with a ventilatory limitation to exercise and a progressive hypoxaemia and hypercapnia at low maximal exercise capacity, Wmax (mean Wmax, 50% pred). Exercise training was performed on a cycle ergometer twice a day for 20 min at approximately 75% of the maximal predicted heart rate for at least 3 weeks. Supplemental oxygen was administered to reach a haemoglobin oxygen saturation of 90% during training. Patients considered malnourished because of a Quetelet Index of less than 20 kg m-2 received hyperalimentation orally or by duodenal tube (total 3500-4000 kcal day-1). Evaluation directly after the training period showed a statistically significant improvement in Wmax, maximal oxygen consumption, maximal minute ventilation, pulse, PaCO2 at rest, FEV1 and body weight. None of the pretraining variables was able to predict the outcome of the training programme in the individual patient. We detected no adverse effects of the programme. This study shows that oxygen-assisted exercise training in combination with correction of the nutritional status is safe and beneficial in CF patients with severe lung disease.
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PMID:Oxygen-assisted exercise training in adult cystic fibrosis patients with pulmonary limitation to exercise. 193 21

Forty patients with cystic fibrosis (CF) (mean age, 13 +/- 2.5 years) were studied with transcutaneous (tc) blood gas monitoring (TCM) during sleep and exercise. By comparing arterial blood samples and TCM in 24 of them (27 samples), a mean bias of tcO2 -15.91 mmHg with a precision of 8.4 mmHg was found. The mean bias of tcCo2 was 7.21 mmHg with a precision of 3.9 mmHg. A standardized submaximal exercise test (1.7 W/kg) was performed in all 40 CF patients and in 14 healthy control subjects (mean age, 13 +/- 0.5 years). The typical tc trend during exercise for CF and healthy subjects was a slight increase of tcO2 levels and tcCO2 stability. A minor decrease of tcO2 values occurred in four CF patients (no greater than 7 mmHg). In 28 patients (mean age, 13 +/- 3 years), tcO2 and tcCO2 were recorded during sleep. In 13 of them, apparent hypoxemic episodes were noted, without relation to the degree of airway obstruction. There were simultaneous episodes of hypercapnia in ten patients. Some difficulties were encountered in analyzing long-term recordings. TcO2 drifts upward and tcCO2 decreases during the recordings over several hours. The change in electrode position after 4 h of sleep modified tcO2 and tcCO2 values. Such difficulties limit the usefulness of long-term but not short-term recordings.
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PMID:Transcutaneous blood gas analysis during sleep and exercise in cystic fibrosis. 234 8

The compensated chronic respiratory acidosis in a girl with cystic fibrosis changed into a mixed respiratory acidosis and metabolic alkalosis under the influence of therapeutic measures. As a consequence respiratory insufficiency worsened. Conservative management of the alkalosis alone both improved hypoxemia and hypercapnia without needing artificial ventilation.
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PMID:[Respiratory insufficiency in mucoviscidosis. Pathophysiologic aspects of conservative drug therapy]. 292 95

The main cause of secondary pulmonary hypertension in the view of a pulmonologist is alveolar hypoventilation - eventually potentiated by acidosis and hypercapnia - which leads to reflectory hypoxemic vasoconstriction of the small pulmonary arteries. Anatomic changes in the pulmonary vessels may be absent or may be limited to medial hypertrophy of the arterioles. If the underlying cause of the hypoxia can be corrected, this reflectory pulmonary hypertension is reversible. In diffuse progressive lung disease, interstitial fibrosis with destruction of the alveolar wall and capillaries may occur, leading to restriction of the pulmonary vascular bed. In such cases pulmonary hypertension may not be completely reversible. The most frequent causes of pulmonary hypertension in childhood are obstructive (e.g. Cystic Fibrosis) or restrictive lung diseases (e.g. interstitial fibrosis). Rare but important in the differential diagnosis are upper airway obstruction, thoracic cage deformity, neuromuscular disorders, high altitude and respiratory center dysfunction. The therapy is elimination of the underlying disease or optimal treatment. In addition prophylactic or therapeutic longterm application of oxygen is more efficient than treatment with pulmonary vasodilators or modern substances like Almitrine. Right heart decompensation should be treated by diuretics. The longterm prognosis is dependent of the underlying disease and is poor in a chronic progressive lung disease like cystic fibrosis and certain types of lung fibrosis.
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PMID:[Pulmonary hypertension from the viewpoint of the pediatric pulmonologist]. 310 Dec 91

The diseases which are commonly complicated by hypercapnic respiratory failure also compromise the respiratory muscles in several ways. Increased work of breathing, mechanical disadvantage, neuromuscular disease, impaired nutritional status, shock, hypoxemia, acidosis, and deficiency of potassium, magnesium, and inorganic phosphorus are the major non-neurologic factors which contribute to respiratory muscle fatigue and failure. Respiratory muscle fatigue has two components. High frequency fatigue occurs rapidly with intense contractile efforts but is usually not severe. It also recovers rapidly with rest. Low frequency fatigue develops more slowly but is severe and requires hours for recovery. Since the spontaneous rate of neural stimulation is predominantly in the low frequency range, this component of fatigue is of particular clinical importance. Fatigue of the inspiratory muscles leads to acute respiratory acidosis, but before carbon dioxide retention occurs, it can be recognized from characteristic symptoms and signs. These include dyspnea which responds to mechanical ventilation, rapid shallow breathing, and asynchronous movements of the chest and abdomen. Inspiratory muscle fatigue must be treated by putting these muscles to rest, by mechanically supporting ventilation. In addition, underlying metabolic nutritional and circulatory abnormalities must be corrected and infection treated. Aminophylline and isoproterenol can restore inspiratory muscle contractility, but controlled clinical trials remain to be done regarding their application in acute and chronic respiratory failure. Inspiratory muscle training improves strength and endurance in patients with obstructive lung disease, cystic fibrosis, and spinal cord injury, but does not always improve physical exercise performance. Again, more work is needed to develop the indications for inspiratory muscle training and to determine the optimum type and duration of the training regimen.
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PMID:Respiratory muscle failure. 634 27

We determined the effect of nocturnal low-flow oxygen (NLFO) on arterial oxygen saturation (SaO2), transcutaneous PCO2 (TcPCO2), and sleep quality in 10 patients with cystic fibrosis (CF) and severe stable chronic obstructive pulmonary disease (COPD). The patients were studied on 2 nights, 1 with oxygen and 1 with air at 2 L/min. The NLFO had no effect upon sleep quality in our patients. The minimal SaO2 occurred during REM sleep and averaged 79.4%. With NLFO, this improved to 92.7%. The average maximal rise in TcPCO2 was 5.6 mmHg on falling asleep while breathing air; this increased a further 5.1 mmHg with NLFO. Two patients also had obstructive sleep apnea. Their SaO2 improved dramatically with NLFO, with no deterioration of ventilation. In 4 patients, ventilation was measured quantitatively. The only consistent changes during air were an increase in abdominal contribution to tidal volume and a drop in minute ventilation from Stage 3-4 to REM sleep of 26%, almost entirely caused by a drop in breathing frequency. The same changes occurred with NLFO. We conclude that NLFO is effective in alleviating the nocturnal hypoxemia of patients with CF with stable COPD and does not cause clinically important hypercapnia.
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PMID:The effect of oxygen on sleep, blood gases, and ventilation in cystic fibrosis. 642 55

Hypoxemia, hypercarbia, and cor pulmonale ultimately occur in most patients with chronic lung disease. Although oxygen therapy may reduce or delay the development of pulmonary hypertension and myocardial failure in these patients, its use is thought to lead to CO2 narcosis and apnea. The effect of O2 administration during sleep has been examined in 12 patients (seven with cystic fibrosis, three with bronchopulmonary dysplasia, one with bronchiolitis obliterans, and one with severe hypersensitivity pneumonitis) using skin surface O2 (Roche) and CO2 (Radiometer) electrodes. Both electrodes were calibrated over wet gas and applied at 44 C. Ten patients had chronic hypercarbia (PaCO2 62 +/- 19 torr; range 46 to 103 torr) when awake. Humidified oxygen was administered by nasal cannula, Venturi mask, or head hood. Oxygen flow was increased every 20 minutes from 80 minutes or until the patient awoke. In eight of ten patients with hypercarbia and in the two normocarbic patients, skin surface carbon dioxide tension (PsCO2) increased by 10% or less as the skin surface oxygen tension (PsO2) was increased. In the remaining two patients with hypercarbia (both had cystic fibrosis) PsCO2 increased 18% and 24% as PsO2 was increased. These last two patients with depressed responsiveness to CO2 could not be separated from the other patients by clinical or laboratory criteria. It is concluded that the skin surface blood gas tensions are a simple and reproducible method for adjusting oxygen therapy in patients with chronic lung disease, and although the response to oxygen varies from patient to patient, most patients with chronic hypercarbia retain their central responsiveness to CO2 during sleep and for them O2 therapy is probably safe.
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PMID:Effect of oxygen administration during sleep on skin surface oxygen and carbon dioxide tensions in patients with chronic lung disease. 678 98


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