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Biomedical subjects

P J Vignos

Publications and source records attributed to P J Vignos.

At least 19 recordsLinked to original sources
PubMed1996

Evaluation of a program for long-term treatment of Duchenne muscular dystrophy. Experience at the University Hospitals of Cleveland.

One hundred and forty-four boys who had Duchenne muscular dystrophy were managed at a single center between 1953 and 1994 and were followed for a mean of 8.9 years. The long duration of follow-up provided an opportunity to examine the effects of physical therapy and orthopaedic treatment on contractures of the lower extremities and on the duration of the ability to walk. Contractures of the lower extremities were controlled best when patients were managed with a combination of daily passive stretching exercises, prescribed periods of standing and walking, tenotomy of the Achilles tendon, posterior tibial-tendon transfer, and application of knee-ankle-foot orthoses. Approximately two years after bracing, the severity of the contracture of the heel cords was similar in the patients who had had an operation and those who had not. By the fourth year after bracing, however, the patients who had had an operation had less severe contractures than those who had had bracing alone. Five to seven years after the operation and bracing, control of contractures was still good, especially for the patients who had had posterior tibial-tendon transfer. Contracture of the knee was well controlled five to seven years after bracing in all patients who had had bracing, with or without an operation. The program enabled the patients who had been managed with bracing to walk until a mean age of 13.6 years. After loss of the ability to walk with bracing, the ability to stand continued for an additional two years with use of orthoses. The findings of the present study demonstrate the value of traditional methods of operative treatment and bracing for controlling contractures of the lower extremities in patients who have Duchenne muscular dystrophy and for prolonging their ability to walk.

Adolescent↗
PubMed1993

Assessment of hand function in Duchenne muscular dystrophy.

Eighteen boys with Duchenne muscular dystrophy (DMD) were assessed for their ability to perform tasks involving wrist and hand function. Each subject was assessed using the Jebsen Test of Hand Function, range of motion measurements, and muscle strength tests. Writing and simulated page turning were performed successfully by boys in all age groups. Boys over age 15 had difficulty completing simulated feeding and picking up large and small objects. The muscle strength of the wrist extensors and the radial deviation range of motion at the wrist were found to be strongly correlated with six of the seven tasks assessed. These two clinical assessments appear to be good indicators of overall wrist and hand function. Life expectancy with DMD is increasing with advances in respiratory care making preservation of wrist and hand function, the major activity remaining with advanced disease, increasingly important.

Adolescent↗
PubMed1989

Duchenne muscular dystrophy: a study of wrist and hand function.

The wrist and hands of 18 Duchenne muscular dystrophy (DMD) patients were assessed for abnormalities. The subjects were divided into three groups by age. Flexion and ulnar deviation contractures of the wrist began in the youngest age group, 8-14 years. Other abnormalities, present in all age groups, included extrinsic and intrinsic digital muscle shortness, boutonniere and swan neck deformities, and hyperextension of the digital interphalangeal joints. Pain found with passive proximal interphalangeal joint flexion has not been reported previously. This study supports the importance of early assessment of the wrist and hand in DMD and suggests intervention techniques to possibly retard the deforming process.

Adolescent↗
PubMed1986

Evaluation of pulmonary hypertrophic osteoarthropathy in cystic fibrosis. A comprehensive study.

A questionnaire survey of 375 patients with cystic fibrosis was performed to seek evidence of pulmonary hypertrophic osteoarthropathy (PHOA). Three hundred responses were obtained. Forty-five patients (15%) who described long-bone or joint pain met clinical criteria for the diagnosis of PHOA, 25 of whom had roentgenographic evidence of periostitis. Compared with an age- and sex-matched control group (group 3), the patients with cystic fibrosis and PHOA as well as roentgenographic evidence of periostitis (group 1) had worse Shwachman scores and pulmonary function and a significantly increased mortality rate (36%). The patients with PHOA but no roentgenographic evidence of periostitis (group 2) had Shwachman scores intermediate between groups 1 and 3. We believe PHOA is more common than previously suspected. Its incidence appears related to severity of disease.

Adolescent↗
PubMed1986

Serial isokinetic evaluations used for a patient with scapuloperoneal muscular dystrophy. A case report.

This article reports the reversal of an atypical rapid decline in muscle strength experienced by a patient with scapuloperoneal muscular dystrophy by modulating his excessive daily physical activity. This process was aided by our monitoring the strength of the quadriceps femoris and hamstring muscles using an isokinetic dynamometer. Serial torque values for muscle strength were compared as the total daily physical activity was decreased systematically. The torque values for thigh muscle strength increased as the subject's total work load was decreased. A complete management program for neuromuscular patients should include specified amounts of daily physical activity, rest, and therapeutic exercise.

Adult↗
PubMed1983

Physical models of rehabilitation in neuromuscular disease.

Physical methods of treatment for neuromuscular diseases constitute the mainstay of current management. The overall goals of management are the maintenance of independent ambulation and the optimal functional state consistent with the disease process. Maintenance of muscle strength requires regular daily physical activity. An active exercise program can give limited increases of strength in muscular dystrophy dependent on the severity of the disease. Active exercise programs do not result in overwork weakness when properly supervised. Endurance exercise training does not appear to be suitable for Duchenne muscular dystrophy but may have value in the less rapidly progressive neuromuscular diseases. contracture development can be retarded by passive stretching and splinting initiated early in the disease course. Weight control is important both for ambulatory and wheelchair-bound patients. Reliable assessment of the results of physical therapy programs has been improved by the introduction of newer, more objective methods for measuring muscle strength.

Body Weight↗
PubMed1983

Predicting the success of reambulation in patients with Duchenne muscular dystrophy.

We used biochemical and clinical variables to develop a method to predict the expected duration of independent walking following surgery and bracing in patients with Duchenne muscular dystrophy. Data from the records of fifty patients were analyzed by linear and multiple regression. The most useful factors, applied in combination, in predicting the duration of walking ability after bracing were: percentage of residual muscle strength, vital capacity, creatinine coefficient, motivation of the patient at the time of bracing, and decrease in creatinine coefficient in the two years prior to bracing. This system uses readily available variables to predict the response to bracing in patients with Duchenne muscular dystrophy. Improvement in the criteria for the selection of patients for surgery and bracing is important in view of the economic cost as well as the demands on the time and energy of these children and their parents.

Braces↗
PubMed1982

Glucocorticoid-induced alterations in the rabbit heart.

Daily intramuscular injection of the synthetic glucocorticoid, betamethasone, into rabbits for 2 weeks resulted in both gross and microscopic alteration of cardiac muscle. A 30 per cent increase in heart weight was based on increased muscle fiber size occasioned by the deposition of fibrillo-granular material (not glycogen in nature) in the cytosol. At the same time, some myocytes underwent profound myolysis, predominantly at the nuclear poles, occasionally pervading the entire cell. Conventional lysosomes were not increased in number or size, but certain cells possessed prominent cytoplasmic vacuoles that sometimes contained mitochondria. The biochemical basis for these cardiac changes is undetermined, but the obvious ultrastructural damage produced by betamethasone, particularly dissolution of myofibrils, may prove to have clinical significance.

Animals↗
PubMed1977

Urinary sodium, potassium and aldosterone in Duchenne muscular dystrophy.

Four adolescent boys with Duchenne (progressive) muscular dystrophy (DMD) of 10-11 years duration and six normal boys of similar age were studied on a metabolism ward for 22 days. Sodium and potassium intake was as follows: Period I, Na 60 mEq, K 60 mEq; Period II, Na 10, K 60; Period III, Na 10, K 95-150; Period IV, Na 60, K 60. The differences between the DMD group and the group of normal boys for sodium and potassium in serum and urine and for urinary aldosterone were not significant. These findings show that the pathologically elevated sodium-potassium ratio in skeletal muscle of patients with DMD is not due to increased aldosterone or other causes of renal wastage of potassium.

Adolescent↗
PubMed1976

Steroid myopathy in connective tissue disease.

In eight women with polymyositis (three patients), systemic lupus erythematosus (SLE) (three patients), rheumatoid arthritis (one patient) and shoulder-hand syndrome (one patient), weakness developed during high dose prednisone therapy. These women were studied using serial functional and manual muscle tests, determination of serum glutamic oxaloacetic transminase (SGOT), creatine phosphokinase (CPK) and serum aldolase levels, and urinary excretion of creatine. Insidious onset of weakness was characteristic. Myalgias were seen in five patients and unusual sudden weakness in two. Weakness was always most severe in the pelvic girdle muscles; there was a lesser involvement of shoulder girdle and distal muscles. Serum muscle enzyme levels were normal in all cases, but urinary creatine excretion was invariably increased and proved to be the most sensitive laboratory indicator for clinical diagnosis and for monitoring patient improvement. Serial urinary creatine excretion and serum enzyme studies were of value in differenting steroid myopathy from a flare of myositis in patients with connective tissue disease. Diagnosis and effective management were achieved by the use of readily available laboratory and clinical procedures without resorting to muscle biopsy.

Adolescent↗
PubMed1976

Evaluation of a clinic education program for patients with rheumatoid arthritis.

A group education program was developed for clinic patients with rheumatoid arthritis. Teaching methods used included the Arthritis Foundation's handbook, Rheumatoid Arthritis, and a lecture by a rheumatologist. The 20 patients in the study were given a multiple choice test before and after the teaching program to determine their knowledge of arthritis. Results showed group education to be an effective teaching device. It was demonstrated that patients learned from reading the handbook alone, but the combination of reading and lecture was found to be a more effective method. Correlation studies showed that prior to group education, patients had a significant knowledge of their disease which was related to native intelligence, formal education, and socio-economic status, but not to duration of disease or length of clinic attendance. This suggests that future patient education experiments should include a pre-instruction test to document prior knowledge.

Arthritis, Rheumatoid↗