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

Arterialized blood gases were analyzed in 143 patients with Duchenne muscular dystrophy (DMD) to assess the relationship between forced vital capacity (FVC) and hypercapnia. The majority of patients studied had PaCO2 values in the low or normal range. Only six older patients had hypercapnia (PaCO2 greater than or equal to 45 mm Hg), and all these patients had FVC values less than or equal to 40% predicted. We conclude that hypercapnic respiratory failure occurs as a late preterminal event in DMD.
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PMID:Hypercapnia in relation to pulmonary function in Duchenne muscular dystrophy. 249 32

Respiratory disorders during sleep were studied in 42 patients with Duchenne muscular dystrophy (DMD) (mean age 18.4 years). Chest and abdominal movement, nasal airflow, snoring sounds, eye movement, and oxygen saturation were monitored during sleep. Three patterns of disorders were found: obstructive apnea, central apnea, and paradoxical respiration without upper airway obstruction (non-obstructive paradoxical respiration). Of these three patterns, obstructive apnea was the most common. Hypertrophy of the tongue and collapsibility of the upper airway seemed to be responsible for the obstructive apnea in these patients. The relationships between PaCO2 while breathing room air and the various indices of respiratory disorders were studied. The index of central apnea differed significantly between patients in whom PaCO2 was less than 50 Torr (early disease, n = 22) and those in whom PaCO2 was greater than or equal to 50 Torr (advanced disease, n = 20), but the indices of obstructive apnea and non-obstructive paradoxical respiration did not differ between those two groups. In conclusion, sleep disorders were common in patients with DMD, and the most common was obstructive apnea. In the patients with advanced DMD, blood gas analysis showed hypercapnia, and the index of central sleep apnea was high, probably because of respiratory muscle weakness or abnormalities in the respiratory center.
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PMID:[Respiratory disorders during sleep in Duchenne muscular dystrophy]. 747 61

Duchenne muscular dystrophy (DMD) is the most common muscular dystrophy in children. Paralysis of respiratory muscles causes a decrease in forced vital capacity (FVC) from age 12 years, and death occurs between 20 and 25 years old and is usually related to respiratory insufficiency. Uncontrolled studies suggest that early home use of nasal intermittent positive-pressure ventilation (NIPPV) in DMD patients free of respiratory failure could limit progression of the restrictive syndrome and therefore improve survival because efficacy of preventive NIPPV has not been demonstrated in a controlled trial, we undertook a randomised multicentre study in which 70 patients with DMD were included. Patients were free of daytime respiratory failure and FVC was between 20 and 50% of predicted values. At least 6 h of nocturnal NIPPV (n = 35) was compared with conventional treatment (n = 35). During a mean follow-up of 52 months, 10 patients died, 8 in the NIPPV group and 2 in the control group (p = 0.05, log-rank test). No differences were observed between the two groups for occurrence of hypercapnia, decrease of FVC below 20% of initial values, or use of necessary mechanical ventilation. Preventive NIPPV did not improve respiratory handicap and reduced survival of DMD patients. Use of NIPPV for preventive purposes should be avoided in patients with FVC between 20 and 50% of predicted values.
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PMID:Randomised trial of preventive nasal ventilation in Duchenne muscular dystrophy. French Multicentre Cooperative Group on Home Mechanical Ventilation Assistance in Duchenne de Boulogne Muscular Dystrophy. 798 Aug 25

Respiratory handicap due to neurological diseases is often underestimated. Given clinical signs are either mild or absent, systematic measurement of the vital capacity is the best mean to detect in practice the restrictive syndrome. The onset of home mechanical ventilatory support should be decided at steady state, apart from episodes of acute respiratory failure. Two types of indications should be distinguished. Necessary ventilation aims at supplying over day and night the respiratory insufficiency incurred by the paralysis of respiratory muscles. Although the criteria for the use of such a supply differ according to the neurological disease, a daytime hypercapnia above 45 mmHg is widely accepted in the literature. It is otherwise established to use first a non invasive technique, while tracheostomy is secondarily proposed in case of failure of these techniques. The application of this therapeutic strategy in Duchenne de Boulogne muscular dystrophy showed that, given that tracheostomy will become necessary in this evolutive disease, proposal of an early tracheostomy is not nonsensical. By contrast, preventive ventilation aims at preventing from the aggravation of the restrictive syndrome in those patients with no criterion for necessary ventilation. It has been proved ineffective in Duchenne muscular dystrophy through a controlled clinical trial.
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PMID:[Long-term ventilation at home in adults with neurological diseases]. 980 60

In many patients with neuromuscular diseases, respiratory failure is mainly caused by alveolar hypoventilation in their terminal stages. Malnutrition is one of the common and serious problems in patients with chronic respiratory failure. Energy consumption for breathing is remarkably high in respiratory compromised patients, causing subsequent increase of total energy expenditure. However, most patients have limited capacity of oral intake. Nutritional depletion is associated with wasting of respiratory muscles, impairment of respiratory drive, alteration of respiratory pattern, and pathological change of pulmonary parenchyma. These indicate that nutritional and ventilatory support is very important in these patients. However, overfeeding also may have detrimental influence on respiratory failure. We experienced a Duchenne muscular dystrophy (DMD) patient on noninvasive positive pressure ventilation (NIPPV) who developed hypercapnia after total parenteral nutrition (TPN). Analysis of clinical course of this patient revealed that there is a significant correlation between PaCO2 and caloric intake. Excess carbohydrate intake can precipitate fat synthesis which induces over-production of carbon dioxide (CO2). Since NIPPV doesn't have a closed circuit, there are some difficulties in respiratory management, such as air leakage to stomach and mouth, and airway obstruction. Failure to optimize NIPPV setting against increased CO2 load might cause hypercapnia in this patient. These suggest that evaluation of energy expenditure and design of nutritional program are essential to avoid hypercapnia due to nutritional support.
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PMID:[Excess caloric intake induced severe hypercapnia in a patient with Duchenne muscular dystrophy on noninvansive positive pressure ventilation]. 1007 34

A 29-year-old Japanese man with Duchenne muscular dystrophy was placed on a mechanical ventilator support at 23 years of age and admitted to our hospital at 25 years of age. He had severe neck contracture deviated to the left side which resulted in dysphagia and microaspiration. At 29 years of age, he developed left lobar pneumonia accompanied by slight fever, back pain and a foul odor from the patient's sputum. Although the patient received broad spectrum antibiotics, pneumonia disseminated to the right lung. A week later, chest computed tomography was conducted which revealed tracheopulmonary-subcutaneous fistula, and a massive subcutaneous abscess with free air production. Drainage from the subcutaneous abscess was done through a chest tube; however, respiratory hypercapnia was not corrected and the patient died. From the culture of drained fluid, anaerobic bacteria including peptostreptococcus sp. were detected. This tracheopulmonary-subcutaneous fistula was thought to be caused by chronic microaspiration of mouth anaerobes, mechanical injury of the trachea under long term ventilator support, and decreased deep back muscle bulk with substitution of adipose tissue around the chest.
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PMID:[Tracheopulmonary-subcutaneous fistula associated with anaerobic subcutaneous abscess on mechanical ventilator support in a patient with Duchenne muscular dystrophy]. 1100 35

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.
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PMID:Home ventilation. 1462 Nov 16

The present study aimed to assess the impact of diurnal mouthpiece intermittent positive pressure ventilation (MIPPV) as the extension of the nasal intermittent positive pressure ventilation (NIPPV) in Duchenne muscular dystrophy (DMD). In total, 42 DMD patients aged 15-33 yrs, normocapnic at night with NIPPV and receiving MIPPV since end-diurnal hypercapnia, were studied. Transcutaneous CO2 tension (Pt,CO2) was prospectively monitored at the end of the day, before and after MIPPV initiation. Vital capacity (VC), breathing pattern and maximal inspiratory strength were measured. Patients were asked to score the presence (1 point) or absence (0 point) of seven respiratory-linked symptoms before and after MIPPV establishment. Survival rates reached 88, 77, 58 and 51% after 1, 3, 5 and 7 yrs, respectively. The mean survival rate was 31 yrs. VC stabilised during 5 yrs with MIPPV. Symptom scores significantly decreased and Pt,CO2 normalised during the day (8.17 +/- 2.22 to 5.78 +/- 0.73 kPa). No accident and minor side-effects were observed in this 184 cumulated patient-yrs study. In conclusion, daytime mouthpiece ventilation is safe, prolongs survival and stabilises vital capacity in Duchenne muscular dystrophy patients. It is recommended on the condition that patients are equipped with a self-supporting harness.
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PMID:Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients. 1694 91

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.
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PMID:[Mechanical ventilation in chronic ventilatory insufficiency]. 1762 Feb 31

Chronic respiratory insufficiency is inevitable in the course of disease progression in patients with Duchenne muscular dystrophy (DMD). Without mechanical ventilation (MV), morbidity and mortality are highly likely towards the end of the second decade of life. The present review reports evidence and clinical implications regarding DMD patients treated with MV. There is no doubt that nocturnal hypercapnia precedes daytime hypercapnia. Historical comparisons have provided evidence that non-invasive intermittent positive pressure ventilation (NIPPV) at night is effective and improves quality of life and survival by 5-10 years. By contrast, the optimal criteria and timing for initiation of NIPPV are inconsistent. A recent randomized study however demonstrated the benefits of commencing NIPPV as soon as nocturnal hypoventilation is detected (Ward S, et al., Randomised controlled trial of non-invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia. Thorax 2005; 60: 1019-24). The respective role of the three hypotheses of the indirect action of nocturnal NIPPV on daytime blood gases may be complimentary; the main improvement may be due to improved ventilatory response to CO2. The ultimate time to offer full time ventilation with the most advantageous interface is lacking in evidence. Full time NIV is possible with a combination of a nasal mask during the night and a mouthpiece during the day, however tracheostomy may be provided when mechanical techniques of cough-assistance are useless to treat chronic cough insufficiency.
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PMID:Mechanical ventilation in Duchenne patients with chronic respiratory insufficiency: clinical implications of 20 years published experience. 1771 17


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