Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1762617 (weakness)
 37,932 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients with clinically mild congenital myopathies presented with chronic respiratory failure. Muscle weakness alone could not account for the respiratory insufficiency since static respiratory pressures were not markedly impaired, ventilation during exercise was normal, and daytime ventilation was normal if ventilatory assistance was provided at night. The ventilatory responses to inhaled carbon dioxide were very low, suggesting that impairment of the central nervous respiratory chemoreceptor contributed to hypoventilation. These patients and others described in the literature suggest that central depression of ventilation may occur more frequently than previously recognized in patients with muscular disorders. Patients with chronic respiratory failure due to central depression of respiratory drive can be effectively managed by assisted ventilation at night.
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PMID:Blunted respiratory drive in congenital myopathy. 1 71

Acid-base terminology including the sue of SI units is reviewed. The historical reasons why nomograms have been particularly used in acid-base work are discussed. The theoretical basis of the Henderson-Hasselbalch equation is considered. It is emphasized that the solubility of CO2 in plasma and the apparent first dissociation constant of carbonic acid are not chemical constants when applied to media of uncertain and varying composition such as blood plasma. The use of the Henderson-Hasselbalch equation in making hypothermia corrections for PCO2 is discussed. The Astrup system for the in vitro determination of blood gases and derived parameters is described and the theoretical weakness of the base excess concept stressed. A more clinically-oriented approach to the assessment of acid-base problems is presented. Measurement of blood [H+] and PCO2 are considered to be primary data which should be recorded on a chart with in vivo CO2-titration lines (see below). Clinical information and results of other laboratory investigations such as plasma bicarbonate, PO2,P50 are then to be considered together with the primary data. In order to interpret this combined information it is essential to take into account the known ventilatory response to metabolic acidosis and alkalosis, and the renal response to respiratory acidosis and alkalosis. The use is recommended of a chart showing the whole-body CO2-titration points obtained when patients with different initial levels of non-respiratory [H+] are ventilated. A number of examples are given of the use of this [H+] and PCO2 in vivo chart in the interpretation of acid-base data. The aetiology, prognosis and treatment of metabolic alkalosis is briefly reviewed. Treatment with intravenous acid is recommended for established cases. Attention is drawn to the possibility of iatrogenic production of metabolic alkalosis. Caution is expressed over the use of intravenous alkali in all but the severest cases of metabolic acidosis. The role of 2,3-diphosphoglycerate on tissue oxygenation is stressed and use of intravenous sodium phosphate as an alternative to intravenous bicarbonate is mentioned.
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PMID:The physiological assessment of acid-base balance. 23 27

Lung involvement was assessed in 30 consecutive patients with systemic lupus erythematosus (SLE), not selected by respiratory symptoms. Pulmonary function tests revealed a higher rate of abnormality than either clinical history or radiography. The single breath carbon monoxide diffusing capacity was below 80 per cent of the predicted value in 24 patients (80 per cent), and a reduced total lung capacity was present in 13 (43 per cent). There was a weak correlation between the severity of the functional defect and disease activity, assessed antinuclear factor and DNA binding. No correlation was found with serum complement of Clq precipitation. Since pulmonary fibrosis in SLE is uncommon it cannot account for the high frequency of abnormal findings, and the pathogenesis of the functional changes is probably multifactorial. In seven of the patients with the smallest lung volumes, measurements of static pressure volume curves and of maximum respiratory pressures indicated extrapulmonary volume restriction. In five of these patients, diaphragm function was specifically assessed and found to be grossly abnormal in four. The inability of the diaphragm to generate normal pressure may be due to either severe weakness or immobility following extensive pleural adhesions. The well recognized syndrome of "shrinking lungs" and high "sluggish" diaphragms with clear lung fields on radiography is probably due to dysfunction of the diaphragm rather than to primary intrapulmonary pathology.
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PMID:Diaphragm function and lung involvement in systemic lupus erythematosus. 60 14

Two cases of the Marfan syndrome presented with spontaneous pneumothorax. Both had chest radiographs showing bilateral bullae in the upper lung zones and pulmonary function tests consistent with mild emphysema. There were dereases in forced expiratory flow rates at low lung volumes, carbon monoxide transfer factor, and lung elastic recoil. It is suggested that pneumothorax and bullous emphysema in this syndrome are caused by a weakness in the pulmonary connective tissue framework.
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PMID:Fragile lung in the Marfan syndrome. 101 48

A syndrome of alveolar hypoventilation has been identified in a group of patients with bilateral diaphragm paralysis. Eight patients were studied in whom diaphragm weakness had been suggested by paradoxical (inward) movement of the abdominal wall on inspiration, of whom seven had evidence of a generalized neuromuscular disorder. Diaphragm function was assessed quantitatively by measurement of the change in transdiaphragmatic pressure during a maximum inspiration (deltaPDi). In five patients, deltaPDi was zero and in the others ranged from 2-6 cm H2O (normal greater than 25 cm H2O) indicating paralysis and severe weakness in the respective groups. Fluoroscopy of the diaphragm was found to give misleading results, and the resons for this are considered. Vital capacity ranged from 65-30 per cent of the predicted normal in the upright posture, typically falling by about a half in the supine posture. Alveolar hypoventilation was present in five patients when supine and in six when asleep, the deterioration in blood gases associated with sleep generally being much greater in these patients than in normal subjects. Respiratory rate was significantly greater than age-matched controls. The ventilatory response to CO2 was impaired. The PCO2 could be brought to normal levels by voluntary hyperventilation, and the unreliability of voluntary respiratory manoeuvres of this kind as indices of ventilatory reserve is emphasized. Alveolar hypoventilation was associated with disturbed sleep, morning headache and day-time fatigue. Symptomatic benefit was achieved by the use of a cuirass respirator at night.
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PMID:Diaphragm function and alveolar hypoventilation. 106 15

Cardiopulmonary function was examined in 18 dogs with serious chronic heartworm disease showing ascites, subcutaneous edema, prostration, weakness, jaundice and so on. After surgical heartworm removal from the pulmonary arteries, 10 dogs recovered (surviving group), and 8 dogs died or were euthanatized because of poor prognosis (nonsurviving group). The number of live heartworms residing in the pulmonary arteries of the surviving group tended to be larger than that in the nonsurviving group. At necropsy, severe pulmonary arterial lesions such as thromboembolism including dead heartworms, proliferative and villous lesions and intimal hyperplasia were noticed in all dogs examined, and tended to be severer in the nonsurviving group. Heartworm-coiling around the tricuspid valve chord was found in 1 dog of the surviving group and 4 dogs of the nonsurviving group. Before heartworm removal, there was no significant difference in the mean pulmonary arterial pressure (MPAP) between the surviving and nonsurviving group. Right atrial pressure (v-wave) was higher, and the cardiac index (CI) was lower in the nonsurviving group. Arterial oxygen tension was lower in the surviving group than in the heartworm-free group, and it was lower in the nonsurviving group than in the surviving group. Carbon dioxide tension was lower in the surviving group than in the heartworm-free group. Bicarbonate concentration (HCO3-) was lower both in the surviving and nonsurviving groups than in the heartworm-free group. One week after heartworm removal, MPAP decreased (P less than 0.05), and CI and HCO3- tended to increase in the surviving group.
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PMID:Cardiopulmonary function in dogs with serious chronic heartworm disease. 139 Nov 87

In 20 patients with chronic renal failure on a hemodialysis with acetate-containing dialysing fluid gasometric, ventilation and breathing patterns disturbances were determined. The loss of CO2 in the dialysate is attributed the major cause of hypoxemia due to alveolar hypoventilation. Hemodialysis with bicarbonate-containing dialysate can be performed in the absence of any change in ventilation and PaO2 despite a systemic alkalosis. Hyperventilation during HD with high concentration of bicarbonate indicate that changes in CO2 tension in the pulmonary circulation can lead to a change in minute ventilation due to the presence of slowly adapting pulmonary chemoreceptors. In patients with low respiratory response, respiratory muscle weakness intensified additionally by hypercapnia may explain this phenomenon.
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PMID:[Effect of carbon dioxide (CO2) pressure on minute ventilation, parameters of gas exchange and blood gases during hemodialysis using fluid containing acetate and bicarbonate buffers]. 143 19

A 20-year-old woman in status asthmaticus who failed to respond to conventional therapy and ventilation of the lungs with 0.5-2.0% halothane, was cooled to 30 degrees C for almost 5 days as the arterial carbon dioxide tension rose above 15 kPa. Halothane was not of immediate value, contrary to other reports. A reduction in carbon dioxide production by controlled hypothermia permitted more suitable ventilation parameters, but extensive muscle weakness caused by a steroid-induced myopathy complicated weaning from respiratory support. Prospective measurement of serum creatinine phosphokinase concentration in patients given high dose corticosteroids may herald the onset of a myopathy.
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PMID:Treatment of acute severe asthma assisted by hypothermia. 156 91

A previous optimal chemical-mechanical model (C.-S. Poon. J. Appl. Physiol. 62: 2447-2459, 1987) suggested that the normal ventilatory responses to CO2 and exercise inputs and mechanical loading can be predicted by the minimization of a controller objective function consisting of the total chemical and mechanical costs of breathing. In this study the model was generalized to include a description of the inspiratory neuromuscular drive as the control output. With a mechanical work rate index for both inspiration and expiration, the general optimization model accurately reproduced the observed responses in the waveshape of inspiratory drive, breathing pattern, and total ventilation under differing conditions of CO2 inhalation, exercise, and inspiratory/expiratory mechanical loads. The simulation results are in general agreement with a wide range of respiratory phenomena, including exercise hyperpnea, CO2 chemoreflex, and post-inspiratory (postinflow) inspiratory activity, as well as respiratory neural compensations for mechanical loading, respiratory muscle fatigue, and muscle weakness.
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PMID:Optimization character of inspiratory neural drive. 160 12

In 17 patients with myotonic dystrophy, spirometric, flow-volume, and maximal mouth occlusion variables were obtained and compared with 8 normal subjects. Ventilatory CO2 response was measured by the estimation of the steady-state effect of a sufficiently large serial dead space. Variability of resting breathing pattern was expressed by the variation coefficients of respiratory cycle time and tidal volume. The group means of the total lung capacity (TLC), vital capacity (VC), forced expiratory volume in 1 sec (FEV)1 and forced inspiratory volume in 1 sec (FIV)1 showed a restrictive pattern. Only maximal static mouth pressure (Pi,max), measured at residual volume (RV) level, showed a significant positive correlation with both VC (p = 0.03) and FIV1 (p = 0.02), suggesting inspiratory muscle weakness as a determinant of the restriction. Although the differences were just not significant, both variation coefficients of the respiratory cycle time and tidal volume were larger in the group with a CO2 sensitivity below the lower limit of normal compared to those with a normal ventilatory response to CO2. In 3 patients, fluctuations in FRC were also present. We hypothesize that, in addition to the already documented FRC fluctuations by uncoordinated spontaneous intercostal muscle action, a defect of integration of afferent neural input and chemical drive in the medullary region may also be present in these patients.
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PMID:Pulmonary function and resting breathing pattern in myotonic dystrophy. 161 21


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