Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0010200 (cough)
 23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A program of respiratory muscle sparing in 9 patients with late stage Duchenne muscular dystrophy is presented. Results indicate that regular efficient night-time support by body respirator provides a constant significant improvement in daytime gas exchange for periods averaging up to 2 years following the occurrence of moderately severe respiratory failure. Until now, the longest survival is more than 4 years at the age of 28. the average PaCO2 and PaO2 before night ventilation were 60.8mmHg and 59.3mmHg, respectively. The levels after the program was begun were 45.5 (PaCO2) and 74.6 (PaO2). These arterial blood gas levels are acceptable for good cardiopulmonary homeostasis. There have been no deaths or episodes of cardiac failure since the program began. The ease of integrating the program into the patient's lifestyle and home are important advantages. Cost of equipment is discussed. The view that these patients are terminal must be qualified. A case is also made for early aggressive conservative measures including body respirators, postural drainage, chest physiotherapy with abdominal assisted coughing, intratracheal suctioning and bronchoscopy to combat respiratory infection, and decreasing the need of tracheostomy and volume ventilators which can be so disabling to the chronic restrictive lung disease (CRLD) patient. Research and development into better design of body respirators is vitally needed. Acute care hospitals must prepare themselves for such patients who now attain more independent living and longer lifespan. For this reason there is a growing need for these facilities to acquire tank ventilators for handling such patients who may need hospitalization for acute medical complications.
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PMID:Night ventilation by body respirators for patients in chronic respiratory failure due to late stage Duchenne muscular dystrophy. 678 53

A 36-year-old Japanese man was hospitalized with coughing and exertional dyspnea (NYHA class I). He was diagnosed as having congestive heart failure, and was treated with diuretics and a beta-adrenergic blocking agent. He responded well to the treatment and his symptoms completely disappeared within a few days. Based on his clinical, laboratory, and molecular genetic findings, he was diagnosed as having X-linked dilated cardiomyopathy (XLDCM). He was found to have a large deletion in the dystrophin gene, involving exons 45-55. This is the first report on a Japanese XLDCM patient with a mutation in the central hot-spot region of this gene.
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PMID:X-linked dilated cardiomyopathy with a large hot-spot deletion in the dystrophin gene. 1181 47

Spirometry is used to monitor respiratory progress in children with Duchenne muscular dystrophy (DMD). Mucociliary clearance depends on cough strength, which can be measured by peak cough flow (PCF). It is not routinely measured in most centers. When the PCF falls below 270 l/min, mucociliary clearance is likely to be impaired during viral illnesses, and techniques to assist mucociliary clearance should be taught. There is no known association between spirometry and PCF. Our aim was to assess if PCF relates to spirometry measures, and if spirometry can be used to predict when the PCF <270 l/min. Children with DMD aged 6-19 years were recruited. Spirometry was performed with a Jaeger Masterscope with version 4.60 software. PCF was performed with a Wright peak flow meter. Data were collected into an Access '97 database, and statistics were performed with Stata 7.0. The association between PCF and spirometry was defined with linear regression. Logistic regression was used to predict the probability that the PCF would be <270 l/min for any given forced vital capacity (FVC) or forced expired volume in 1 sec (FEV1). The risk ratios for PCF <270 l/min were calculated for the spirometry parameters. PCF is associated with FVC (R2, 0.72) and FEV1 (R2, 0.69). The likelihood of PCF <270 l/min rises when FVC <2.l and FEV1 <2.l/sec. The risk ratio for PCF <270 l/min when FVC <2.1 l is 4.80 (1.72-13.40) and when FEV1 <2.1 l/sec is 3.94 (1.43-10.85). In children with DMD, PCF should be measured when FVC <2.1 l or FEV1 <2.1 l/sec, so that techniques to assist with mucociliary clearance can be effectively used.
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PMID:Relationship between peak cough flow and spirometry in Duchenne muscular dystrophy. 1576 40

The aim of this study was to investigate the factors affecting cough ability, and to compare the assisted cough methods in patients with Duchenne muscular dystrophy (DMD). A total seventy-one male patients with DMD were included in the study. The vital capacity (VC) and maximum insufflation capacity (MIC) were measured. The unassisted peak cough flow (UPCF) and three different techniques of assisted peak cough flow were evaluated. UPCF measurements were possible for all 71 subjects. But when performing the three different assisted cough techniques, peak cough flows (PCFs) could be obtained from only 51 subjects. The mean value of MICs (1801 +/- 780 cc) was higher than that of VCs (1502 +/- 765 cc) (p< 0.01). All three assisted cough methods showed a significantly higher value than the unassisted method (F=80.92, p< 0.01). The manual assisted PCF under MIC (MPCFmic) significantly exceeded those produced by manual assisted PCF (MPCF) or PCF under MIC (PCFmic). The positive correlation between the MIC, VC difference (MIC-VC), and the difference between PCFmic and UPCF (PCFmic-UPCF) was seen (r=0.572, p< 0.01). The preservation of pulmonary compliance is important for the development of an effective cough as well as assisting the compression and expulsive phases. Thus, the clinical importance of the inspiratory phase and pulmonary compliance in assisting a cough should be emphasized.
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PMID:Assisted cough and pulmonary compliance in patients with Duchenne muscular dystrophy. 1586 96

We report the case of a Duchenne muscular dystrophy patient with good bulbar function but severely decreased forced vital capacity (9%) and spontaneous peak cough flow (PCF) (2.35 L/s). The patient needed continuous noninvasive ventilation (NIV) consisting of a volumetric ventilator with a nighttime nasal mask and a daytime mouthpiece. He also required application of manually assisted coughing techniques by insufflation with a resuscitation bag and chest thrust (manually assisted PCF after maximum insufflation capacity of 4.33 L/s). An episode of serious food aspiration was resolved by his main caregiver through NIV and manually assisted coughing. Bronchoscopy under sedation using NIV with a lip seal connection to his volumetric ventilator later revealed that no material remained. This case exemplifies the potential role of skilled respiratory management in some neuromuscular diseases.
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PMID:[Respiratory muscle aids during an episode of aspiration in a patient with Duchenne muscular dystrophy]. 1619 18

The function of inspiratory muscles is crucial for effective cough as well as expiratory muscles in patients with Duchenne muscular dystrophy (DMD). However, there is no report on the correlation between cough and inspiratory muscle strength. To investigate the relationships of voluntary cough capacity, assisted cough techniques, and inspiratory muscle strength as well as expiratory muscle strength in patients with DMD (n= 32). The vital capacity (VC), maximum insufflation capacity (MIC), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP) were measured. Unassisted peak cough flow (UPCF) and three different techniques of assisted PCF were evaluated. The mean value of MICs (1918 +/- 586 mL) was higher than that of VCs (1474 +/- 632 mL) (p < 0.001). All three assisted cough methods showed significantly higher value than unassisted method (212 +/- 52 L/min) (F = 66.13, p < 0.001). Combined assisted cough technique (both manual and volume assisted PCF; 286 +/- 41 L/min) significantly exceeded manual assisted PCF (MPCF; 246 +/- 49 L/ min) and volume assisted PCF (VPCF; 252 +/- 45 L/min) (F = 66.13, p < 0.001). MIP (34 +/- 13 cmH2O) correlated significantly with both UPCF and all three assisted PCFs as well as MEP (27 +/- 10 cmH2O) (p < 0.001). Both MEP and MIP, which are the markers of respiratory muscle weakness, should be taken into account in the study of cough effectiveness.
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PMID:Respiratory muscle strength and cough capacity in patients with Duchenne muscular dystrophy. 1664 46

Patients with Duchenne muscular dystrophy (DMD) are living longer into adulthood due to a variety of improvements in health care practices. This growing patient population presents new therapeutic challenges. In this article, we review the literature on current treatment of adult DMD as well as our own experience as a multidisciplinary team actively caring for 23 men ages 19-38 years of age. Approximately one quarter of our adult DMD patients have remained on moderate dose corticosteroids. Daily stretching exercises are recommended, particularly of the distal upper extremities. Cardiomyopathy is anticipated, detected, and treated early with afterload reduction. Oxygen saturation monitoring, noninvasive positive pressure ventilation and cough assist devices are routinely used. Other medical issues such as osteoporosis, gastrointestinal and urinary symptoms are addressed. Current and future therapies directed at prolonging the lifespan of those with DMD will result in further increases in this adult population with special needs and concerns. These needs are best addressed in a multidisciplinary clinic.
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PMID:Current treatment of adult Duchenne muscular dystrophy. 1688 41

Duchenne muscular dystrophy (DMD) is an X-linked, rapidly progressive myopathy affecting the limb muscles, the respiratory muscles, the heart, the intestines, and the brain. Since about 90% of DMD patients die from muscular respiratory failure or cardiomyopathy, early and adequate therapy is essential. Ventilatory failure from muscle weakness requires mechanical support for ventilation and coughing as soon as there is symptomatic nocturnal hypoventilation. Today noninvasive positive-pressure ventilation (NIPPV) is the method of choice for supportive long-term mechanical ventilation in DMD. For assisted coughing, various methods are available, among which the mechanical in-exsufflator is the most widely used device. There is large nonrandomized clinical trial evidence that NIPPV improves quality of life and prolongs the lives of DMD patients if medical, social, economic, and ethical issues, raised by the availability of long-term NIPPV, are adequately addressed. Cardiac involvement in DMD manifests as impulse generation or impulse conduction abnormalities or cardiomyopathy. Cardiac abnormalities in DMD respond well to adequate therapy. Though DMD is ultimately a fatal disease, quality of life and life expectancy can be markedly improved if cardiopulmonary manifestations are adequately treated.
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PMID:Cardiopulmonary support in duchenne muscular dystrophy. 1700 47

The impact of ventilatory support on the natural history of neuromuscular disease (NMD) has become clearer over the last 2 decades as techniques have been more widely applied. Noninvasive ventilation (NIV) allows some patients with nonprogressive pathology to live to nearly normal life expectancy, extends survival by many years in patients with other conditions (eg, Duchenne muscular dystrophy), and in those patients with rapidly deteriorating disease (eg, amyotrophic lateral sclerosis) survival may be increased, but symptoms can be palliated even if mortality is not reduced. A growing number of children with NMD are surviving to adulthood with the aid of ventilatory support. The combination of NIV with cough-assist techniques decreases pulmonary morbidity and hospital admissions. Trials have confirmed that NIV works in part by enhancing chemosensitivity, and in patients with many different neuromuscular conditions the most effective time to introduce NIV is when symptomatic sleep-disordered breathing develops.
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PMID:Recent advances in respiratory care for neuromuscular disease. 1716 12

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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