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

We report two cases of severe, acute myopathy with selective degeneration of myosin filaments in asthmatics who developed respiratory failure with hypercapnia and acidosis requiring endotracheal intubation, administration of vecuronium and prolonged ventilatory support. Hypoxia was documented in one case and probably present in the other. Both patients received prolonged treatment with high doses of intravenous methylprednisolone. Flaccid quadriparesis was noted after discontinuation of vecuronium. Muscle biopsy showed nonspecific myopathic changes on light microscopy. Electron microscopy revealed selective loss of myosin filaments in many fibers. Recovery occurred within 2 months with supportive treatment. This entity is probably related to a combination of high doses of corticosteroids, vecuronium administration and metabolic abnormalities associated with respiratory failure.
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PMID:Acute myopathy with selective degeneration of myosin filaments following status asthmaticus treated with methylprednisolone and vecuronium. 135 45

A 21-year old women with rhizomelic muscular deficit and signs of hypercapnia developed acute respiratory failure. Laboratory tests revealed high creatine kinase activity, and electromyograms showed myogenic patterns with a few myotonic discharges. Biopsy of the quadriceps muscle elicited major vacuolar myopathy with glycogen overload. Acid maltase activity was undetectable in muscular tissue. After 7 months on high-protein diet (1540 calories, 37% proteins) there was no clinical or biochemical improvement. The other published cases of acid maltase deficiency treated with high-protein diet are discussed.
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PMID:[Myopathy in adults caused by acid maltase deficiency. A trial of treatment with high protein diet]. 141 Aug 90

We report the results of a retrospective study of a group of 27 patients with a myopathy who were ventilated at home using a nasal mask over a period of 5 years. Twelve patients were ventilated in a preventive fashion before any orthopaedic surgical intervention and 15 out of necessity because of respiratory failure and/or hypercapnia. There was a statistically significant improvement in the PaO2 while the PaCO2 remained stable. The vital capacity (CV) was unaltered. Side effects were relatively frequent but did not lead to this method of ventilation being stopped. One patient died from a very advanced cardio-myopathy after having stopped his own assisted ventilation. Another patient died at home of bronchial congestion. One patient had a tracheotomy after 3 years of ventilation. The treatment was judged overall as positive amongst the 19 patients who responded to a questionnaire anonymously. We are able to confirm the efficacy of this mode of ventilation by the nasal route as much therapeutically as prophylactically, which is against the recently reported results in a multi-centre study.
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PMID:[Prolonged mechanical nasal ventilation. Apropos of 27 case of myopathy]. 176 20

The presence of a peripheral myopathy in hypothyroidism has been well recognized. Involvement of the diaphragm has been suggested recently but the clinical spectrum never clearly defined. We studied three patients with hypothyroidism presenting with fatigue, dyspnea, exercise limitation, and in two, chronic alveolar hypoventilation (PaCO2 of 51 and 75 mm Hg) before and after thyroid hormone replacement. In all patients diaphragmatic strength as determined by the maximal transdiaphragmatic pressure was low (2, 13, and 64 cm H2O) and improved with therapy (86, 84, and 90 cm H2O). Similarly, all patients manifested a fatiguing breathing pattern, as determined by the diaphragmatic tension time index. These values (0.22, 0.55, and 0.36) decreased after hormone replacement (0.16, 0.20, and 0.15). These changes were associated with the correction of hypercarbia in the two patients with hypoventilation and an improvement in lung volumes and exercise endurance in all patients. This study confirms that in patients with hypothyroidism diaphragmatic dysfunction occurs more frequently than has been suspected and might be of varying severity. This dysfunction reverses with adequate hormone replacement.
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PMID:Hypothyroidism. A reversible cause of diaphragmatic dysfunction. 280 37

We report a study of an adult with a maltase acid deficiency myopathy. A restrictive respiratory syndrome due to respiratory muscle weakness is associated with paralysis of other muscular groups. In 1982 the patient presented with an alveolar hypoventilation, and mechanical ventilation was required after acute respiratory failure. The patient has received nocturnal mechanical ventilation by tracheostomy at home for 5 years. His clinical status gradually improved in parallel to amelioration of his respiratory condition. Functional respiratory tests improved: initial hypoxia-hypercapnia disappeared, vital capacity increased. The possible mechanisms underlying the improvement are discussed. Increase in pulmonary compliance is an argument to explain the functional improvement observed. Ventilatory response to carbon dioxide was abnormal whereas the ventilatory response to exercise and maxima minute ventilation test were normal. Results are consistent with a respiratory control impairment. The role of mechanical ventilation is difficult to assess in the improvement we observed.
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PMID:Adult maltase acid deficiency myopathy: treatment with long-term home mechanical ventilation. 305 36

Abnormal ventilatory function is common in patients with neuromuscular disorders. This report describes depressed ventilatory response to both hypoxia and hypercapnia, as well as arterial oxygen desaturation during sleep in a family with congenital myopathy. The index patient also had an abnormal ventilatory response to metabolic acid loading. There was clinical evidence of proximal muscle weakness, and a muscle biopsy specimen was consistent with myopathy. The reduction in ventilatory drive, however, could not be explained solely by ventilatory muscle weakness. This report describes a distinct familial syndrome of congenital myopathy and abnormal ventilatory response to hypercapnia and hypoxia. All affected family members had unique facial features, proximal muscle weakness, and impaired ventilatory responses. The combination of impaired ventilatory drive and reduced ventilatory muscle strength leaves patients particularly vulnerable, and heightened awareness of this association is important in the treatment of these patients.
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PMID:Abnormal ventilatory chemosensitivity and congenital myopathy. 366 6

We studied 53 patients with proximal myopathy to determine at what level of muscle weakness hypercapnic respiratory failure is likely, and which tests of pulmonary function or respiratory muscle strength would best suggest this development. Respiratory muscle strength was determined from maximal static efforts and in half the patients, both inspiratory and expiratory muscle strengths were less than 50% of normal. In the 37 patients without lung disease respiratory muscle weakness was accompanied by significant decreases in vital capacity, total lung capacity, and maximum voluntary ventilation; by significant increases in residual volume and arterial carbon dioxide tension (PaCO2); and greater likelihood of dependence on ventilators, atelectasis, and pneumonia. Hypercapnia was particularly likely when respiratory muscle strength was less than 30% of normal in uncomplicated myopathy, and when vital capacity was less than 55% of the predicted value in any patient.
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PMID:Respiratory muscle and pulmonary function in polymyositis and other proximal myopathies. 641 85

Despite advancing knowledge of the pathophysiology and treatment of asthma, asthma morbidity and mortality are on the rise. To help avert this trend, clinicians and patients must focus their attention on the early identification and treatment of asthma exacerbations. As in the words of Dr. Thomas Petty: " ... the best treatment of status asthmaticus is to treat it three days before it occurs." (7) Still, there will be asthmatics with life-threatening attacks that require careful assessment and aggressive management. Inhaled beta-agonists, systemic corticosteroids, and oxygen remain the drugs of choice in SA. Anticholinergics play a lesser role in the treatment of acute asthma, and debate continues regarding the efficacy of theophylline in this setting. Available data do not support the routine use of magnesium sulfate or antibiotics in patients with SA. Patients failing drug therapy should be considered early for intubation and mechanical ventilation. A strategy of mechanical ventilation that prolongs TE by limiting VE and decreasing inspiratory time, and that tolerates hypercapnia, avoids excessive lung hyperinflation and barotrauma and should improve the outcome of these most critically ill asthmatics. Intubated and mechanically ventilated patients should be aggressively sedated. Paralytic agents should be used only if adequate control of the cardiopulmonary status cannot be achieved by sedation alone. Minimizing the use of paralytic agents may decrease risk of myopathy and other adverse consequences of muscle paralysis. Finally, after successful treatment of a life-threatening episode of asthma, the treatment team should address prevention of future episodes of SA prior to discharge.
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PMID:The assessment and management of adults with status asthmaticus. 773 78

A 49-year-old woman was admitted to the hospital for hypercapnia. Pulmonary function testing showed small lung volumes without parenchymal lung disease. Muscle enzyme levels were normal and the EMG was nonspecific. Finally, muscle biopsy revealed abundant nemaline bodies characteristic of nemaline myopathy. Nasal intermittent pressure ventilation was started with a preset pressure ventilator during sleeping hours with a good response.
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PMID:Adult onset of nemaline myopathy presenting as respiratory insufficiency. 855 68

Four patients with nemaline myopathy, 1 with the severe infantile form and 3 with the benign congenital (classical) form, exhibited significant respiratory problems. In the patient with the severe infantile form, respiratory failure paralleled the generalized muscle weakness, whereas the 3 patients with the benign infantile form suddenly developed respiratory failure while still ambulant. Polysomnographic studies performed on 2 patients revealed that apnea or irregular thoracic movements occurred only during rapid eye movement sleep, when hypercapnia was also demonstrated on serial transcutaneous partial O2 and partial CO2 monitoring. A discrepancy between motor ability and respiratory involvement probably is not uncommon in patients with the moderate congenital form of nemaline myopathy. Clinicians must always be alert to respiratory failure when monitoring patients with nemaline myopathy.
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PMID:Respiratory failure in nemaline myopathy. 974 41


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