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Query: UMLS:C0010200 (cough)
 23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Myotonia is defined as a persistent contraction of skeletal muscles after their stimulation. This contracture is not prevented or relieved by regional anaesthesia or muscle relaxants. The sensitivity to non-depolarizing muscle relaxants is usually normal. Suxamethonium, neostigmine, hypothermia, a rise in kalaemia should be avoided. There have been case reports of malignant hyperthermia in patients with myotonia congenita. Dystrophia myotonica is the second most frequent of the inherited muscle diseases, after Duchenne's dystrophy. The severity of the disease is due more to the muscular atrophy and the multiple organ involvement than to the abnormal contraction. Atrioventricular heart block and dysrhythmias are more common than heart failure. Prolonged apnoea and pneumonia are the main risks of anaesthesia. In severe cases, exists a restrictive respiratory insufficiency which is preceded by a fall in the maximum expiratory pressure. Dysphagias and inefficient coughing may occur early. An increased susceptibility to hypnotic drugs and opiates is a common feature. Spontaneous sleep apnoeas should be sought before anaesthesia, especially by using pulse oximetry. The anaesthetic implications are reemphasized.
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PMID:[Anesthesia in myotonia]. 253 24

Ten individuals with spinal muscular atrophy (SMA) and chronic ventilatory insufficiency were trained in the use of assisted coughing techniques and received intermittent positive pressure ventilation (IPPV) via oral and/or nasal interfaces for a mean of 5.3 (range = 1 to 17) years. During this time they had significantly fewer respiratory complications than before introduction of noninvasive respiratory muscle aids despite the fact that 6 of the 10 went on to require more than 20 hours per day of ventilator use with less than 2 hours of ventilator-free breathing time (VFBT). All except two noninvasive IPPV users had vital capacities (VCs) less than 13% of predicted normal. They could, however, communicate verbally and take nutrition by mouth. All of the patients remained in the community. Five patients were gainfully employed and four were in school. We conclude that noninvasive respiratory muscle aids including noninvasive IPPV and assisted coughing techniques are effective and practical alternatives to tracheostomy for SMA patients with ventilatory failure but functional bulbar musculature.
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PMID:Noninvasive long-term ventilatory support for individuals with spinal muscular atrophy and functional bulbar musculature. 771 10

Twelve children with spinal muscular atrophy (SMA) type 2 and 13 children without physical disability underwent kinematic analysis of thoracoabdominal volume changes when breathing spontaneously and when breathing deeply. A very accurate optical method of kinematic analysis was used. Volumes were partitioned into upper thoracic, lower thoracic, and abdominal compartments. Abdominal volume increases accounted for 96% of the normal tidal volumes and 87% of the deep breathing volumes for the patients, but only 74 and 41% of the volumes, respectively, for the controls. For the patients the upper thoracic contribution to breathing volumes was --1.7% for normal tidal volumes and 0.3% for deep breathing volumes. Patients with less upper thoracic kinematic reserve were also found to be more likely to have chronic nocturnal hypoventilation. We conclude that kinematic analysis can be helpful in determining differences in regional lung mobility and risk for nocturnal ventilatory dysfunction for children with SMA. Therapeutic interventions need to be addressed to maintain thoracic kinematic reserve and lung compliance and, thereby, to facilitate more normal lung growth and the ability to cough.
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PMID:Spinal muscular atrophy: kinematic breathing analysis. 887 99

Our objectives were to describe survival, hospitalization, speech, and outcomes related to respirator needs for spinal muscular atrophy type 1 (SMA1) patients, using noninvasive or tracheostomy ventilation. From 65 SMA patients referred to our clinic since 1996, we chose 56 SMA1 patients who developed respiratory failure before age 2 years. Patients either had tracheostomy tubes (group A), or used noninvasive ventilation and assisted coughing; a previously reported extubation protocol (group B) was used as needed. Sixteen patients underwent tracheostomy at 10.8 +/- 5.0 months of age, 33 were in group B, and 7 others died without life-support interventions. Compared to group B, group A patients had fewer hospitalizations until age 3 years, but more after age 5, and 15 of 16 lost all spontaneous breathing tolerance posttracheostomy and could not speak. One group A patient died at 16 months of age, and the others were 73.8 +/- 57 months of age (the oldest was 19 years old). Two group B patients died at 6 and 13 months, respectively, whereas the other 31 were 41.8 +/- 26.0 months (and up to 8.3 years) old. Three of 31 in group B required high-span positive inspiratory pressure plus positive end-expiratory pressure (PIP + PEEP) continuously with minimal tolerance for breathing on their own, and 4 could not communicate verbally.In conclusion, SMA type 1 children can survive beyond 2 years of age when offered tracheostomy or noninvasive respiratory support. The latter is associated with fewer hospitalizations after age 5 years, freedom from daytime ventilator use, and the ability to speak.
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PMID:Spinal muscular atrophy type 1: management and outcomes. 1211 92

Chronic obstructive pulmonary disease (COPD) is the internationally preferred term for chronic, progressive lung disorders which are characterised by airflow limitation that is not fully reversible. The symptoms of COPD - including breathlessness, cough, excessive sputum production and reduced muscle tone and muscle wasting - reflect the complex pathophysiology of the disease. In order to address these symptoms, treatment regimens should take into account the multiple components that contribute to COPD. Clinical evidence has emerged indicating that, especially in patients with severe COPD, long-acting beta(2)-agonists (LABAs) and inhaled corticosteroids (ICS) result in improvements in symptoms, reduce the frequency and severity of exacerbations, and improve health-related quality of life. This review evaluates the clinical evidence for the potential of LABA/ICS treatment to address the symptoms of COPD and whether combination therapy of this nature adds significant benefit to patients.
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PMID:Effective management of COPD in primary care - the role of long-acting beta agonist/inhaled corticosteroid combination therapy. 1675 94

Progressive respiratory muscle weakness with bulbar involvement is the main cause of morbidity and mortality in type I and severe type II spinal muscular atrophy. Noninvasive positive pressure ventilation techniques coupled with laparoscopic gastrointestinal procedures may allow for improved morbidity and mortality. The authors present a series of 7 spinal muscular atrophy patients (6 type I and 1 severe type II) who successfully underwent laparoscopic gastrostomy tube insertion coupled with Nissen fundoplication and early postoperative extubation using noninvasive positive pressure ventilation techniques. The authors measured the length of survival and the frequencies of pneumonia and hospitalization before and after surgery as outcomes of these new surgical and medical interventions. All 7 patients had respiratory symptoms (unmanageable oropharyngeal secretions, cough, pneumonia), difficulty feeding, and weight loss. Six patients had documented reflux via diagnostic testing preoperatively. Five patients were on noninvasive positive pressure ventilation and other supportive respiratory therapies prior to surgery. All 7 patients survived the procedures. By August 2006, 5 patients with type I and 1 with severe type II spinal muscular atrophy were alive and medically stable at home 1.5 months to 41 months post-op. One patient with type I expired approximately 5 months post-op due to obstructive apnea. This case series demonstrates that laparoscopic gastrostomy tube placement coupled with Nissen fundoplication and noninvasive positive pressure ventilation can be successfully used as a treatment option to allow for early postoperative extubation and to optimize quality of life in type I and severe type II spinal muscular atrophy patients.
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PMID:Laparoscopic Nissen fundoplication during gastrostomy tube placement and noninvasive ventilation may improve survival in type I and severe type II spinal muscular atrophy. 1764 Dec 58

A child with spinal muscular atrophy type 2 was referred for evaluation of eating and swallowing problems. The dysphagia evaluation demonstrated coughing during eating and drinking and occasionally stertorous when eating solid food. The videofluoroscopic swallow study showed a late upper esophageal sphincter opening with hypopharyngeal residue, more with solid food than with thin liquid. His lumbar lordosis associated with anterior tilted pelvis and his problems with head balance due to weak neck musculature caused compensatory behavior like a retracted neck and mandible. This position negatively influences the opening of the upper esophageal sphincter. This case supported the idea that dysphagia in spinal muscular atrophy type 2 is caused by both a bulbar component as well as a treatable posture component.
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PMID:Is head balance a major determinant for swallowing problems in patients with spinal muscular atrophy type 2? 1840 79

Hereditary neuropathies are classified into HMSN/Charcot-Marie-Tooth disease (CMT), familial amyloid polyneuropathy (FAP), hereditary motor neuropathies (HMN) and hereditary sensory (and autonomic) neuropathies (HSAN). The clinical features of HMSN are generally characterized as distal dominant motor and sensory involvements. However, we have reported a novel HMSN with proximal dominancy (HMSN-P) originated in Okinawa and Shiga prefectures, Japan. The gene locus is located in the centromere region of chromosome 3. In 2008, a new family with the HMSN-P was reported from Brazilians of Japanese ancestry. This Brazilian family was initially diagnosed as having "a familial ALS". The HMSN-P linked to ch.3 is not limited in Japan, but may be present in the worldwide. The overseas scientific research for the elucidation of the mechanism of HMSN-P supported by JSPS KAKENHI (21406026) is planning. Recently several other types of HMSN-P have been reported; HMSN-P with urinary disturbance and paroxysmal dry cough, a patient with both CMT 1A and mild spinal muscular atrophy and CMT1A with severe paresis of the proximal lower limb muscles. Therefore the clinical concept of HMSN is not limited as the disease with distal dominant motor sensory involvement. HMSN has the wider spectrum from distal to proximal and motor/sensory to autonomic neuropathies.
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PMID:[Wide spectrum of hereditary motor sensory neuropathy (HMSN)]. 2003 Feb 57

The purpose of this article was to describe the use of noninvasive inspiratory and expiratory muscle aids to prevent chest wall deformities including pectus excavatum, to prevent respiratory complications of vertebral surgery, to prevent acute and long-term ventilatory insufficiency and failure in children with paralytic disorders who develop these deformities, and to permit the extubation and tracheostomy tube decanulation of "unweanable" patients. Noninvasive airway pressure aids can provide up to continuous ventilator support for patients with little or no vital capacity and can provide for effective cough flows for patients with severely dysfunctional expiratory muscles. An April 2010 consensus of clinicians from 20 centers in 14 countries reported over 1500 spinal muscular atrophy type 1 (SMA1), Duchenne muscular dystrophy (DMD), and amyotrophic lateral sclerosis (ALS) patients who survived using continuous ventilatory support without tracheostomy tubes. Four of the centers routinely extubated unweanable DMD patients so that none of their over 250 such patients has undergone tracheotomy.
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PMID:Physical medicine respiratory muscle aids to avert respiratory complications of pediatric chest wall and vertebral deformity and muscle dysfunction. 2122 90

Respiratory insufficiency is the primary cause of morbidity and mortality among patients with spinal muscular atrophy type 2. The primary complications include ineffective cough with decreased airway clearance, nocturnal hypoventilation, diminished lung and chest wall development, and increased risk for pulmonary infection. Respiratory devices including mechanical insufflator-exsufflator and bilevel positive airway pressure are the primary devices of respiratory maintenance and treatment and are associated with decreased morbidity and fewer hospital admissions. This article discusses the primary respiratory complications of spinal muscular atrophy type 2 and the role of respiratory interventions to promote growth and development, improve cough efficacy, reverse nocturnal hypoventilation, and prevent and treat pulmonary infection.
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PMID:Respiratory management of spinal muscular atrophy type 2. 2536 58


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