Orthopedic approaches for the treatment of lower extremity contractures in the Duchenne muscular dystrophy patient in the United States and Canada.
Explore the source record for details and available documents.
Canada↗
Biomedical subjects
J D Hsu
Publications and source records attributed to J D Hsu.
The development of current approaches to the management of spinal deformity for patients with neuromuscular disease.
Special attention needs to be paid to the spine of a patient with a neuromuscular disorder. Spinal muscular weakness that causes progressive spinal collapse with resulting scoliosis, kyphosis, or kyphoscoliosis can lead to pain and difficulty in standing, walking, sitting, or balancing. Function and independence may be further lost. Appropriate measures, including spinal fusion in many instances, need to be undertaken as part of the overall care of the individual with neuromuscular disease.
Humans↗
Haemotoxic effect of phenylurea herbicides in rats: role of haemoglobin-adduct formation in splenic toxicity.
Three substituted phenylurea herbicides were used for the study of the haemotoxic effects on rats of chronic exposure to these compounds. Female Sprague-Dawley rats were given monuron, diuron or fenuron (250-1000 mg/kg diet) for 14 months. The final body weights were similar to those of controls. No treatment-related effects on organ weights were observed at autopsy, except for a dose-related increase in spleen weights in rats treated with monuron or diuron, but not in those treated with fenuron. The proportion of haemoglobin in the form of methaemoglobin increased in the dosed group and resulted in a secondary anaemia with changes in the morphology of erythrocytes. Haemoglobin adducts of aromatic amines released from the herbicides were present at dose-related levels in rats treated with monuron or diuron. Compound-related lesions were observed histologically in treated rats, with increased pigmentation (haemosiderin) in the spleen, reflecting the response to the haemolytic anaemia and methaemoglobinaemia induced by the herbicides. Pigment deposition consisting of golden brown granules in the cytoplasm of the tubular epithelium in the kidney and in the Kupffer cells in the liver were observed only in rats treated with monuron. The haemotoxic effects that were observed may indicate that the formation of adducts between haemoglobin and the parent aromatic amines released metabolically from these herbicides has a role in the splenic toxicity of these compounds.
Animals↗
Gait and posture changes in the Duchenne muscular dystrophy child.
As the child with Duchenne muscular dystrophy (DMD) grows older, gait and posture changes occur. This is attributable to the natural progress of muscle weakness secondary to muscle tissue destruction. Bracing improves body alignment and maintains limb stability passively. Surgical correction of contractures allows for bracing and maintenance function and is recommended as an adjunct to the overall care of the ambulatory DMD patient.
Braces↗
Fractures caused by falling from a wheelchair in patients with neuromuscular disease.
This report discusses the occurrence of fractures caused by falling from a wheelchair. 13 distal femur, two proximal humerus and three distal tibia fractures were seen in 13 patients, of whom 11 had Duchenne muscular dystrophy, one had Becker muscular dystrophy and one had spinal muscular atrophy. All were non-ambulant and had been wheelchair-dependent for at least one year before the fall occurred. Six patients sustained fractures between 1976 and 1982, and five more between 1983 and 1985. Eight subsequent fractures from wheelchair falls have occurred since 1986. Although wheelchair design, controls and adaptations have continued to improve, wheelchair safety has not.
Accidental Falls↗
Triple arthrodesis in the treatment of fixed cavovarus deformity in adolescent patients with Charcot-Marie-Tooth disease.
Ten adolescent patients with Charcot-Marie-Tooth disease (12 feet) were evaluated an average of 7 years, 7 months following triple arthrodesis (TA) for the treatment of cavovarus deformity. The TA was the primary or index bone procedure. At follow-up, five feet were plantigrade, asymptomatic, and radiographically fused at all three joints. Three feet were plantigrade and asymptomatic, but two had talonavicular pseudarthrosis and one had calcaneocuboid pseudarthrosis. Three feet were radiographically fused at all three joints, but in nonplantigrade positions and symptomatic. One foot required a revision TA. In general, TA offers adolescent Charcot-Marie-Tooth patients a stable plantigrade foot in the face of a progressive disorder. The residual deformity, revision, and pseudarthrosis rates are similar to those for adults. Postoperative pain was related to a nonplantigrade foot and not to the presence of pseudarthrosis. It is not known whether a nonplantigrade foot was a result of incomplete surgical correction or recurrent deformity due to progressive muscle imbalance.
Adolescent↗
Suppression of aflatoxin B1-induced hepatotoxic lesions by crocetin (a natural carotenoid).
The suppressive effects of crocetin (a natural carotenoid) on the hepatotoxic lesions induced by aflatoxin B1 (AFB1) were investigated in male Wistar rats. Rats were divided into five groups: groups I and II served as normal and solvent control respectively. Group III was given AFB1 (25 micrograms/day/rat) alone; group IV was given crocetin (0.1 mg/day/rat) alone; and group V received both AFB1 and crocetin. Rats received AFB1 and crocetin for 9 and 10 weeks respectively, and were maintained on basal diet for 35 weeks. At the end of the experiment (week 45), the incidence of liver lesions in rats of group V was significantly reduced by approximately 40% compared with group III. There were no liver lesions in rats of groups I, II and IV. A significant protective effect of crocetin on AFB1 hepatotoxicity was shown, as manifested by reduced effects on the activities of serum aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and gamma-glutamyl transpeptidase (P less than 0.01-0.001). From our previous results and present data, we suggest that the suppression of crocetin on AFB1 hepatotoxicity in the rats might be due to the defense mechanisms of hepatic tissues that elevated the GSH S-transferase activity and decreased the formation of hepatic AFB1-DNA adducts.
Aflatoxin B1↗
Accumulation of CK-MM is impaired in innervated and contracting cultured muscle fibers of Duchenne muscular dystrophy patients.
No specific abnormalities have been reproducibly manifested in aneurally cultured muscle of Duchenne muscular dystrophy (DMD) patients. We now report that the accumulation of the muscle-"specific" isozyme of creatine kinase (CK-MM) was significantly and preferentially impaired in long-term innervated contracting muscle fibers cultured from 4 DMD patients (DMD-InnCMFs) compared to: i) their noninnervated sister-cultured muscle fibers, and ii) innervated contracting control cultured human muscle fibers (Control-InnCHMFs). Accumulation of other muscle-"specific" isozymes (MSIs), viz. glycogen phosphorylase, phosphoglycerate mutase, and lactic dehydrogenase, was not significantly impaired. We have not observed preferentially-impaired CK-MM accumulation in any Control-InnCHMFs from 22 patients (children and adults) with a variety of neuromuscular diseases. There was no apparent difference between DMD-InnCMFs and Control InnCHMFs regarding: acceptance of innervation; neuronally-driven, virtually continuous muscle-fiber contractions; characteristic myofiber organization by phase-contrast microscopy, and increased longevity of the innervated fibers.
Animals↗
Spinal curvature, hand dominance and prolonged upper-extremity use of wheelchair-dependent DMD patients.
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.
Adolescent↗
Morphometry and pathology of the paraspinous muscles in idiopathic scoliosis.
Muscle biopsies from the apex of both sides of the curve of 31 patients with idiopathic scoliosis showed abnormalities in fiber-type distribution in 68 per cent and in fiber size in 55 per cent. There was no preference for either side. Type 1 fiber predominance was as common as type 1 fiber deficiency. Atrophy occurred in 33 per cent and affected mainly type 1 fibers: atrophy of type 2 fibers was rare. Hypertrophy was limited to type 2 fibers, and occurred in 26 per cent. The strength factor for type 1 fibers exceeded that for type 2. Type 2A fibers were no larger than 2B fibers: there was a large type 2A predominance, more so on the convex side. Most of the muscle changes appear to be secondary and compensatory: none suggests a pathogenesis for the curve.
Adolescent↗
Mobile arm supports: criteria for successful use in muscle disease patients.
Twenty-nine subjects who had been fitted with mobile arm supports 6 months to 11 years prior to the initiation of this study were evaluated to determine criteria for successful use of such supports. All subjects were diagnosed as having Duchenne muscular dystrophy or spinal muscular atrophy. Three methods were used to collect data: 1) review of medical charts, 2) interview with patient and family member(s), and 3) observation of the patient in his wheelchair for sitting stability, adjustment and control of the mobile arm support, and speed and efficiency in self-feeding with and without the support. The physical criteria previously established for optimal use were met by the successful users in the sample except for presence of elbow flexion contractures. Among those who had curvature of the spine, spinal fusion increased the percentage of successful users of mobile arm supports. Successful use declined with muscle grades of below poor in elbow flexors. The incidence of success was greater when the tasks of self-feeding, driving a motorized wheelchair and performing leisure skills could be accomplished only with the support. Primary reasons for early rejection of the support were related to attitude toward the equipment, influence of the social support system, and patterns of activities performed with the equipment.
Activities of Daily Living↗
Histochemical and morphometric changes in muscles of stroke patients.
There is little information on the muscle fiber changes or the fiber type affected in supraspinal hemiplegia. Muscle biopsy specimens from 20 patients with stroke, obtained during orthopedic reconstruction, were examined by modern histochemistry. Atrophy was present in all of the muscles, affecting Type 1 fibers in 100% and Type 2 fibers in 95% of the patients. Type 2 atrophy was more severe than Type 1 atrophy. Group atrophy and fiber type grouping, present in 40%, seemed related to peripheral nerve or root damage. Hypertrophy of Type 1 fibers was present in 45%, associated with Type 2 hypertrophy in 15%. Although diffuse morphometric atrophic seemed not to correlate with the level of motor activity in this group of 20 patients, hypertrophy appeared related to activity. Hence, efforts to mobilize and rehabilitate stroke patients cannot prevent atrophy of some fibers, they seem to stimulate a hypertrophy not seen in inactive patients.
Adult↗
Fiber composition and morphometry of the quadriceps femoris muscle in athletes and non-athletic individuals after knee injury.
Explore the source record for details and available documents.
Adolescent↗
The natural history of spine curvature progression in the nonambulatory Duchenne muscular dystrophy patient.
The natural progression of the spinal curve of eight nonambulatory Duchenne Muscular Dystrophy (DMD) patients were followed from 38 to 91 months (average, five years and two months). Curve progression was seen in all patients. In patients whose curves exceeded 40 degrees, secondary complications, including loss of function diminished sitting tolerance, use of the arms and hands to prop the body up when seated, pain, and a diminished vital capacity (12-16%), were present.
Activities of Daily Living↗
Posterior tibial tendon transfer: a review of the literature and analysis of 74 procedures.
At Rancho Los Amigos Hospital on the Children's Orthopedic Service, 43 patients underwent anterior transfer of the posterior tibial tendon through the interosseous membrane between 1969 and 1979. This included 74 procedures for the following diagnoses: Duchenne muscular dystrophy, club-foot, cerebrospastic disease (cerebral palsy and head injury), Charcot-Marie-Tooth disease, scapuloperoneal dystrophy, and peroneal palsy. The purpose of this paper is to present the 54 of these transfers that have a minimum 2 year follow-up were performed with a uniform surgical technique, and had uniform postoperative management. This series is analyzed and compared with a review of the literature in order to set down guidelines for the use of the procedure. The analysis reveals that the patient with Duchenne muscular dystrophy who has decreasing gait function or brace fitting problems is the ideal candidate for the procedure with 26 of 28 satisfactory results. Gait electromyography was critical to the selection of the cerebrospastic patient for the transfer with those patients demonstrating swing-phase function of the posterior tibialis muscle yielding the best results. Finally, posterior tibial tendon transfer is generally not indicated in congenital clubfoot with six unsatisfactory results in 10 procedures, and the procedure should not be done in Charcot-Marie-Tooth disease as part of staged treatment that includes a possible future hindfoot stabilization.
Cerebral Palsy↗
Skeletal changes in children with neuromuscular disorders.
This report presents the skeletal findings in 45 Duchenne muscular dystrophy boys, ages 1-21 years, and 81 spinal muscular atrophy patients, ages 1-35 years. Long bones are characterized by thin cortices and osteoporosis. Joints are distorted depending on the contracted position. Fractures are seen in this bone and around joints when little force is applied to these extremities. Recognition of the problems, treatment by proper positioning of the weakened extremity and spine, and prevention of fracture by proper handling of such patients are recommended.
Adolescent↗
Fracture of the femur in the Duchenne muscular dystrophy patient.
This report summarizes the management of 20 femur fractures seen in 16 Duchenne muscular dystrophy patients. The most common cause of fracture was falling. In the ambulatory patient, reduction of the fracture was followed by casting so that ambulation could be maintained. This was the preferred treatment and was carried out in six of seven patients. Nine nonambulatory patients presented with 11 femoral fractures. Gross displacement was seldom seen. Short-term immobilization generally gave satisfactory pain relief. Prevention of further function loss during fracture treatment present a challenge to the physician caring for this group of severely disabled children.
Adolescent↗
Challenges in the care of the retarded child with Duchenne muscular dystrophy.
Recent psychological testing and neuropathologic studies support the occurrence of relative retardation, an in some cases severe retardation, in patients with Duchenne muscular dystrophy. Muscle deterioration and wasting are associated with the natural progression of the disease. Progressive physical weakness can be described in the following stages: early, walking, wheelchair, and late. The more emotionally mature the family, the more effective they are in coping at each stage of the disease. Nevertheless, a constant stress is present in all families. This stress can increase or plateau at the various stages and as new problems are encountered. When mental retardation is significant, the stress on the family become even more marked.
Ambulatory Care↗