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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

So far there is no causal treatment for Duchenne muscular dystrophy up to now, it has been proven, however, that its course can be considerably improved by an early contracture-prophylactic operation of both lower limbs--mostly between age 4 and 6 years--as well as by a surgical stabilization of the spine before any progressive scoliosis appears, that is at the very beginning of the wheel-chair stage: Walking and standing ability can be prolonged for several years and a significant scoliosis can be avoided. A decisive prolongation of life can be achieved by treating the fatal respiratory insufficiency with timely started mechanical ventilation. Our first experiences have shown, that there is no proven justification for a reserved attitude against early lower limb surgery in view of modern anaesthesia. Malignant hyperthermia-reaction and hyperkalaemia are the known anaesthetic rise factors of operations in Duchenne muscular dystrophy. However, we could exclude virtually these rises by choosing the right anaesthesia and by a comprehensive monitoring routine. If need be, malignant hyperthermia can be treated effectively by using the obligatory antidote (Dantrolene). In view of an expected causal treatment in future, early detection of Duchenne muscular dystrophy by newborn screening ("CK-Test") as well as a comprehensive stage-depending treatment programme (early surgery/mechanical ventilation) are nowadays of outstanding importance.
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PMID:[Duchenne muscular dystrophy--contracture preventive operations of the lower extremities with special reference to anesthesiologic aspects]. 202 63

Respiratory failure is the major cause of death in patients with Duchenne muscular dystrophy (DMD). In this report we retrospectively examined the clinical courses, pulmonary function, progression of scoliosis and the time of assisted ventilation. Forty nine patients, aged 2 to 27 years were studied and thirty eight patients of them were examined twice in some intervals. The subjects of examinations included pulmonary function studies consisting of spirometry, measurement of the thoracic scoliosis measured according to the method of Cobb and room air arterial blood gas values. The results were the following; 1) The decline in percentage values of vital capacity (%VC) began at age 8 or 9 and decreased consistently with age. Finally %VC reached under 10 percent at mean age of 21.53 with respiratory failure. 2) Thoracic scoliosis measured according to the method of Cobb increased further with advancing age. The greater the angle of curvature, the more likely the development of respiratory failure. 3) Sixteen patients received negative pressure ventilator (chest respirator: CR) between 16.5 and 25.1 years of age. Twelve patients used part-time (mainly night-time) ventilator and four patients used it in full-time. The average PaCO2 and PaO2 before administration of ventilator were 67.49 mmHg and 71.46 mmHg, respectively. The levels after ventilation were 58.01 mmHg (PaCO2) and 82.09 mmHg (PaO2).
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PMID:[Respiratory failure and its care in Duchenne muscular dystrophy]. 206 Feb 38

This study evaluated the age-related and ambulation-related incidence of scoliosis among boys with Duchenne muscular dystrophy (DMD) and the relationship between wheelchair dependency and scoliosis. Charts of 88 DMD boys were reviewed retrospectively. The relationships between age and both wheelchair dependency and scoliosis were graphed as cumulative distributions with increasing patient age. The relationship between ambulation status and scoliosis was analyzed with a stepwise series of chi square analyses, assuming increasing time intervals between wheelchair dependency and scoliosis. Wheelchair dependency and scoliosis were both age-related phenomena. The relationship between ambulation status and scoliosis became significant only after 3.5 years of wheelchair dependency. It should be noted that 24% of boys with scoliosis developed their curves before cessation of ambulation. While development of scoliosis among DMD boys is clearly an age-related phenomenon, its previously assumed relationship to ambulation requires reevaluation.
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PMID:Scoliosis associated with Duchenne muscular dystrophy. 229 3

In the absence of any effective drug treatment, physical methods of management are still the mainstay of treatment. Our current practice in Duchenne muscular dystrophy is to provide lightweight knee-ankle-foot orthoses at the time of loss of ambulation. This prolongs independent walking for an average of two years, and has the effect of delaying the onset of scoliosis, particularly if the boy remains ambulant during the pubertal growth spurt. We are currently assessing the value of radical surgery, performed early in the course of the disease, which may stabilize and prolong independent walking. In non-ambulant patients instrumentation of the spine, using mainly the Luque technique, has revolutionised the treatment of progressive scoliosis. Ventilator support produces clinical improvement in late cases with symptomatic hypoventilation. Its place in the management of asymptomatic patients with nocturnal hypoventilation still needs evaluation, as does the role of early prophylactic respiratory support. We have reviewed the clinical drug trials over the past 10 years. There has been an overall improvement in their quality control.
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PMID:Management of children: pharmacological and physical. 268 29

We reviewed the clinical charts and spinal radiographs of fifty-one boys who had Duchenne muscular dystrophy, had not had surgical treatment of the spine, and had been followed until death. All had scoliosis. None of the following variables was useful in predicting which curves would become severe: age when the patient initially walked, age when he ceased walking, age at onset of spinal collapse, surgical release of the iliotibial bands, or age at the time of death. Radiographs were made within eighteen months before death for thirty-three patients; in thirty-one of them, the final curve exceeded 40 degrees and in seventeen, 90 degrees. For the remaining eighteen patients, final radiographs were made more than eighteen months before death; at that time, eight of them already had a curve of more than 90 degrees. Although there was a relationship between extension of the lumbar spine and severity of scoliosis at the time of final follow-up, early maintenance of the lumbar spine in extension rarely prevented the development of a severe curve. For most of the patients who had a severe curve, sitting was difficult and was accompanied by breakdown of the skin and pain. When a patient's curve exceeded 35 degrees, the vital capacity usually was less than 40 per cent of the predicted normal value. Therefore, when walking becomes impossible for boys who have Duchenne muscular dystrophy, routine spinal arthrodesis should be considered.
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PMID:Progression of scoliosis in Duchenne muscular dystrophy. 276 82

Two-hundred eighty-three boys with Duchenne dystrophy and 10 with Becker dystrophy have been followed for up to 10 years in a protocol that accurately measured their function, strength, contractures, and back curvature. Clinical heterogeneity is noted. Patients whose muscles were stronger were more likely to die from a cardiomyopathy. Weaker patients died from respiratory failure. A series of milestones is defined, which is of use in following the illness in an individual patient. This approach permits a scoring system that allows the severity of the disease to be defined in an individual boy. Evaluation of physical therapy and surgical intervention shows that night splints and scoliosis surgery are effective forms of treatment.
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PMID:Duchenne muscular dystrophy: patterns of clinical progression and effects of supportive therapy. 292 72

We reviewed the incidence and severity of scoliosis in 93 boys with Duchenne muscular dystrophy who had been rehabilitated in light-weight knee-ankle-foot orthoses at the point of loss of ambulation, between the ages of 6 and 12 years. There was an inverse relationship between the severity of the scoliosis and the age walking was lost in the orthoses. The scoliosis was less severe in the 20 boys (22%) who walked in their orthoses beyond 13 years of age than in those who stopped walking in their orthoses before 13 years. There was also a rapid deterioration in the scoliosis between the ages of 13 and 15 years in boys who had stopped walking in their orthoses before the age of 13 years, while in comparison, boys of the same age who were ambulant in their orthoses beyond 13 years showed a much slower rate of deterioration. These results strongly suggest that walking in orthoses beyond the age of 13 years prevented rapid progression of scoliosis between 13 and 15 years of age, ie, during the pubertal growth spurt.
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PMID:Prevention of rapidly progressive scoliosis in Duchenne muscular dystrophy by prolongation of walking with orthoses. 319 93

In order to determine the role of the spinal muscles in the etiology of the scoliosis associated with Duchenne muscular dystrophy, we carried out a study of 16 children using computerized tomography. Scans were taken at the level of the 9th dorsal and the 3rd lumbar vertebrae. Density readings were taken in the medial and lateral portions of the erector spinae. The results confirmed the loss of muscle and replacement by fat which was greater on the concave side. The density differences between the convex and concave sides correlated with the degree of curvature measured by Cobb's method. Computed tomography may offer a prognostic indicator in the development of scoliosis.
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PMID:Investigation of scoliosis in Duchenne dystrophy using computerized tomography. 340 44

The management of scoliosis in Duchenne muscular dystrophy is not standardized. Some centers offer no treatment, some provide customized body jackets or wheelchair inserts, and others recommend early surgical spinal fusion. Spinal orthoses are frequently prescribed at significant cost and inconvenience to the patient, with little evidence as to effectiveness in preventing scoliosis. This paper reports the preliminary findings of 22 patients involved in a study assessing the usefulness of a modified Jewett hyperextension brace. Seven brace wearers and 15 controls were entered into the study. The preliminary results indicate a mean curvature of 23.6 degrees (+/- 12.8 degrees) in the experimental group and 35.7 degrees (+/- 24.8 degrees) in the controls. Over an observation period ranging from two to seven years, the wearers have shown an average curve progression of 8.8 degrees/yr while the control group progressed at 11.2 degrees/yr. This difference is not considered statistically significant. We conclude that wearing the brace minimally slows scoliosis progression, but does not ultimately prevent a significant curve.
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PMID:Scoliosis management in Duchenne muscular dystrophy: prospective study of modified Jewett hyperextension brace. 357 38

The pathoanatomy and natural history of scoliosis associated with Duchenne muscular dystrophy were established by a review of 105 patients. Scoliosis developed in 95% of these patients after the loss of ambulation. The direction of the curve was determined by asymmetrical contracture of the iliotibial band. Functional classification using the radiographic sitting kyphotic index proved a reliable guide to the age at loss of ambulation and life expectancy, as well as the rate of progression and eventual severity of spinal deformities. Spinal orthoses failed to control curve progression in 94% of patients (30 of 32). Fourteen patients underwent spinal fusion with excellent long-term results.
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PMID:Scoliosis associated with Duchenne muscular dystrophy. 361 41


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