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At least 433 records · Page 24Linked to original sources
PubMed1999

Lung function abnormalities and respiratory muscle weakness in children with juvenile chronic arthritis.

In contrast to adult rheumatoid arthritis (RA) little is known about the prevalence, nature and cause of lung function abnormalities in children with juvenile chronic arthritis (JCA). The aim of this study was to determine whether children with polyarticular and systemic onset JCA have lung function abnormalities and if so, whether they are related to pulmonary disease, thoracic and/or muscular involvement. We determined lung function and disability in 31 children with polyarticular and systemic JCA. Respiratory muscle function, thorax expansion and spine mobility were determined in the same patients, as well as in 32 matched healthy children. Peak expiratory flow (PEF) and forced vital capacity (FVC) were significantly reduced in JCA patients, when compared to reference values. Thorax expansion and spine mobility were normal, compared to paired controls. Maximum inspiratory (PI,max) and expiratory (PE,max) pressures were significantly reduced in patients compared to paired control subjects. A positive correlation was found between PE,max and FVC and PEF, an inverse correlation between expiratory pressure and disability. In conclusion, children with polyarticular and systemic juvenile chronic arthritis show a pronounced impairment in respiratory muscle strength, severe enough to cause mild lung function abnormalities and an increase in disability-scores.

Adolescent↗
PubMed1994

Contribution of direct actions of the oxime HI-6 in reversing soman-induced muscle weakness in the rat diaphragm.

The actions of the bispyridinium oxime HI-6 ([[[(4-aminocarbonyl)pyridino]-methoxy]methyl]-2- [(hydroxyimino)methyl]-pyridinium dichloride) were investigated in vitro on rat phrenic nerve-hemidiaphragm preparations. Isometric twitch and tetanic tensions were elicited at 37 degrees C with supramaximal nerve stimulation at frequencies of 20 and 50 Hz. To approximate normal respiration patterns, trials consisting of 30 successive 0.55 s trains were alternated with 1.25 s rest periods. Under control conditions, the above stimulation pattern generated tensions that were well maintained at both frequencies. In contrast, a marked depression of muscle tension was observed in diaphragms removed from rats administered 339 micrograms/kg soman (3 LD50) and tested in vitro. Addition of HI-6, 4 h after soman exposure, led to a nearly complete recovery of muscle tension at 20 Hz. At 50 Hz, muscle tensions still declined especially when trains were elicited at 1.25 and 3 s intervals. The recovery by HI-6 observed in this study appears to be mediated by mechanisms unrelated to acetylcholinesterase reactivation since no increase of enzymatic activity was detected and the effect was reversed by a brief washout in oxime-free physiological solution. The results suggest that the direct action of HI-6 may play a role in restoring soman-induced diaphragmatic failure but this effect would be significant primarily under low use conditions.

Acetylcholinesterase↗
PubMed1999

Polymyositis with respiratory muscle weakness requiring mechanical ventilation in a patient with metastatic thymoma treated with octreotide.

Although most patients with thymoma present with a mediastinal mass amenable to surgical resection, some patients develop metastatic disease requiring systemic therapy. The majority of thymomas express somatostatin receptors as demonstrated by octreotide scanning, an observation which has prompted the clinical use of octreotide in patients with this disease. Many patients with thymoma exhibit autoimmune paraneoplastic syndromes, most frequently myesthenia gravis. We report here the case of a patient with metastatic thymoma who developed a profound autoimmune polymyositis and lupus-like syndrome that flared following treatment with octreotide and was associated with a clinical response to this agent. No evidence for myesthenia gravis was discovered. The severity of the myopathy necessitated mechanical ventilation for 12 weeks. The natural history of thymoma, treatment options including recent combination chemotherapy regimens, and potential mechanisms for flaring of autoimmune paraneoplastic syndromes triggered by therapy of thymoma are discussed.

Acute Disease↗
PubMed1986

Intermittent positive pressure breathing in patients with respiratory muscle weakness. Alterations in total respiratory system compliance.

Intermittent positive pressure ventilation (IPPB) is reported to improve lung compliance and decrease the work of breathing in subjects with kyphoscoliosis. These results suggest that IPPB may improve chest wall and lung compliance in patients with neuromuscular disease. We studied the short-term effects of IPPB on total respiratory system compliance in 14 subjects with neuromuscular disease. Seven were quadriplegics, and seven had muscular dystrophy. Vital capacity was reduced to 38 +/- 14 percent of the predicted normal values. Baseline measurements of total respiratory system compliance were 57 +/- 18 percent when compared to normal control values. After a 20 minute treatment of IPPB delivered with inspiratory pressures of 20 to 25 cm H2O that more than tripled resting tidal volume, there was no significant change in total respiratory system compliance in either group of patients. These findings indicate that patients with quadriplegia or muscular dystrophy do not derive immediate improvement in ventilatory mechanics from IPPB treatments.

Adolescent↗
PubMed1980

Hemodialysis encephalopathy with osteomalacic fractures and muscle weakness.

The hemodialysis unit at Columbia, South Carolina, opened in April, 1974. By June of 1977, 7 patients had died from dialysis encephalopathy, and 16 of the 51 surviving patients showed speech disorders, fits, and myoclonic jerks. Pathologic fractures were seen in 22 patients. Bone histomorphometry showed severe osteomalacia with minimal, if any, osteitis fibrosa, and serum alkaline phosphatase activity was normal. The mean serum aluminum concentration in 33 random patients was elevated at 83.5 microgram/liter (control group, 13.9 microgram/liter, P less than 0.001). The mean bone aluminum concentration in 4 patients who died from this syndrome was 307 ppm of bone ash (normal, less than 10 ppm). Dialysis fluid aluminum was high at 140 microgram/liter. Purification of the dialysis fluid with a water softener, reverse osmosis and a deionizer and abandoning extra-strength Basaljel resulted in a notable clinical and EEG improvement. None of 81 new patients who started hemodialysis between July of 1977 and July of 1979 after the change in treatment have developed any such symptoms. A syndrome of hemodialysis encephalopathy accompanied by pathologic osteomalacic fractures is described. Recovery is possible. The syndrome was eradicated after purification of the dialysis fluid.

Aged↗