Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A study of 26 wheelchair-dependent patients with Duchenne muscular dystrophy aged between 11 and 23 years (average 16.5 years) showed no correlation between handedness or prolonged use of one upper extremity and convexity of the spinal curve. Severe, untreated scoliosis results in loss of function. A straight, stable spine allows patients to use their upper extremities functionally rather than for supporting the trunk.
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PMID:Spinal curvature, hand dominance and prolonged upper-extremity use of wheelchair-dependent DMD patients. 378 Nov 5

Fifty-seven boys with Duchenne muscular dystrophy aged between 6 years 3 months and 13 years 6 months, who were at the point of losing the ability to walk or had recently done so, were fitted with lightweight knee-ankle-foot orthoses to re-establish walking. 47 walked well and independently in their orthoses, achieving good stability and confidence. 20 are still ambulant; the other 27 stopped walking at intervals ranging from eight to 48 months. Prolongation of walking prevented the development of scoliosis, joint contractures and deformities and also benefited the boys psychologically.
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PMID:Prolongation of walking in Duchenne muscular dystrophy with lightweight orthoses: review of 57 cases. 399 72

Ten patients suffering from Duchenne muscular dystrophy were followed up for more than 7 years. The diagnosis was determined by biopsy. Progress of the disease was severe and all patients died before the age of 18. The average angle of the scoliosis at the end of this development was 73 degrees. The maximum vital capacity was 1198 ml. The authors conclude that neither the use of a brace nor adaptation of a wheelchair are helpful. Surgical treatment is hazardous when the curve is greater than 23 degrees. The indications for such treatment are based on the importance of the degree of pulmonary restriction and on early signs of severe progress of the scoliosis.
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PMID:[Therapeutic indications in scoliosis of Duchenne muscular dystrophy. Apropos of 10 cases]. 624 91

Segmental instrumentation and fusion of spinal deformity in patients with Duchenne muscular dystrophy yields good correction and provides firm internal stabilization that allows rapid mobilization of patients following surgery. When surgery to stabilize spinal deformity is done in younger patients in whom pulmonary function is better and curves are milder, complication rate and length of hospital stay are diminished, correction and balance are improved, and patients rapidly return to their normal life-style. We no longer routinely attempt orthotic treatment of these curvatures, but advise stabilization of the collapsing spine surgically with segmental instrumentation and fusion when scoliosis reaches 30-40 degrees.
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PMID:Advantage of early spinal stabilization and fusion in patients with Duchenne muscular dystrophy. 649 Aug 69

Nine boys with Duchenne muscular dystrophy, chairbound but without scoliosis, were fitted with a recently developed brace of metal and moulded medium-density polyethylene, the modified Calot brace. Each was also fitted with a wrap-around, moulded leather jacket. Each brace held the relaxed lumbar spine in an angle of lordosis similar to that achieved by a brief active effort to sit erect without a brace. Over-all, they proved equally acceptable to patients and carers. The modified Calot brace caused substantially less restriction of pulmonary function and so may be preferable to the moulded leather jacket.
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PMID:A new spinal brace for use in Duchenne muscular dystrophy. 651 63

The problem of real distress from the discomfort of collapsing scoliosis is predictable in Duchenne muscular dystrophy (DMD). Once the lumbar curve has exceeded 35 degrees, further progression is inevitable. A vital capacity, then, of 35% or more permits consideration of spinal surgery. Using these indications, 24 patients with DMD had long Harrington instrumentations and spinal fusions from S1 up to the upper thoracic spine (T4, 5, or 6). After two weeks recumbent, they were mobilized wearing a light spinal support in their wheelchairs. The complications encountered are described in detail. One patient died two years after his operation from dystrophic cardiomyopathy. With a follow-up period of four months to 42 months, the rest of these patients are well and sitting with comfort. The authors think that this experience indicates that prophylactic spinal fusion deserves consideration in the care planned for these patients.
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PMID:Surgical stabilization of the spine in Duchenne muscular dystrophy. 666 79

This study investigates the effectiveness of spinal supports and seating systems in retarding the progress of spinal curvature in Duchenne muscular dystrophy. The approach was to take six monthly radiographs of children with this disease attending the Regency Park Centre sitting in their wheelchairs with their usual spinal supports. Scoliosis progressed from a mean value of approximately 10 degrees for children under 12 years to a value of the order of 75 degrees by the age of 16 to 18 years. The rate of progression peaked at an average of more than 1 degree per month between 14 and 18 years. The curvatures observed using modular seats, spinal jackets and custom-molded seats were not significantly different from those observed using unmodified wheelchair seats.
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PMID:Orthotic management of scoliosis in Duchenne muscular dystrophy. 669 8

There are not, as yet, clear indications for the surgical management of scoliosis in Duchenne muscular dystrophy (DMD), taking into account the varying severity of the clinical course. Monitoring the vital capacity can be most important for the indication and timing of surgery. In some cases, delaying surgical intervention with conservative management using spinal braces and wheelchair inserts can permit the restrictive lung syndrome to advance to the point that surgery will be contraindicated. Ten such patients conservatively treated for an average of 5 years exhibited perhaps a slower progression but ultimately an advanced deformity. From a second group of five carefully selected and surgically treated patients, indications for spinal surgery were reviewed. Surgical intervention should be prophylactically undertaken when there is high risk of a rapidly evolving curve with a severe restrictive lung syndrome.
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PMID:The treatment of scoliosis in Duchenne muscular dystrophy. 672 12

Electron microscopic X-ray microanalysis and X-ray fluorescence spectrometry were done on platelets from patients with Duchenne muscular dystrophy and idiopathic scoliosis (both these conditions are known to be associated with increased intramyofibre calcium). A significant increase in calcium and phosphorus concentrations was found in the dense granules and whole cells of both conditions. The findings suggest that idiopathic scoliosis like Duchenne muscular dystrophy is a multisystem disease with detectable changes in platelets.
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PMID:Microanalysis and X-ray fluorescence spectrometry of platelets in diseases with elevated muscle calcium. 678 Mar 62

Pulmonary function was correlated with patient age and degree of thoracic scoliosis in 25 patients with Duchenne muscular dystrophy in a retrospective, longitudinal study. The observed forced vital capacity (FVC) was found to peak at approximately the age when standing ceases, then to decline rapidly. Thoracolumbar curves were found to be insignificant in adversely affecting pulmonary function in patients with Duchenne muscular dystrophy. Percent FVC was found to be the parameter of pulmonary function that was most strongly correlated with age and scoliosis measurements. In addition, age and thoracic scoliosis together were better predictors of percent FVC than either one alone. Each 1 year of age had approximately the same negative influence on percent FVC that each 10 degrees of thoracic scoliosis had; both decreased percent FVC by approximately 4%. A regression equation for percent FVC is presented which predicts that the patient who has had scoliosis progression halted by spinal fusion would, subsequent to the surgery, show a slower rate of decline of percent FVC and that this rate is quantifiable, predictable, and dependent solely on the patient's advancing age. Therefore, early spinal instrumentation and fusion is advocated in the patient with Duchenne muscular dystrophy.
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PMID:Correlation of scoliosis and pulmonary function in Duchenne muscular dystrophy. 687 33


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