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Biomedical subjects

R B Gledhill

Publications and source records attributed to R B Gledhill.

9 recordsLinked to original sources

Myoblast transfer in Duchenne muscular dystrophy.

One biceps muscle of 8 patients with Duchenne muscular dystrophy was injected at 55 sites with a total of 55 million viable, purified, and contamination-free normal myoblasts (myoblast transfer). The other biceps of each patient was injected with a placebo to serve as a control. The procedure was blinded to the patients, parents, and investigators. Myoblasts derived from a biopsy specimen of the fathers were cultured and purified under strict conditions and carefully screened for microbial contamination. All patients received cyclophosphamide for immunosuppression for 6 or 12 months. No serious complications were observed after myoblast transfer, indicating that the procedure is safe. The overall therapeutic efficiency of myoblast transfer was poor as judged by the results in maximal voluntary force generation, dystrophin content of the muscle, magnetic resonance imaging of the muscle, and the lack of donor-derived DNA and dystrophin messenger RNA in the injected muscle. An improved efficiency of the take of myoblasts might be achieved by using younger cells and injecting the myoblasts with a myonecrotic agent (to increase the prevalence of regeneration) and a basal laminal fenestrating agent.

Analysis of Variance↗

Radiologic evaluation of osteosarcoma.

Twelve patients with osteosarcoma were evaluated by plain radiographs, radionuclide bone scans and computed tomography (CT). Plain films were the primary radiologic tool for the investigation and follow-up of the skeletal lesion and were particularly helpful for the demonstration of periosteal calcification and bone permeation. The main value of the radiophosphate scan was to detect metastatic or multifocal bone disease. CT was able to show new bone formation in the soft-tissue mass not seen on plain films, to determine proximal intramedullary extension and to assess the response of the bone lesion to preamputation chemotherapy. The lungs were followed at regular intervals with plain chest radiographs and CT scans.

Adolescent↗

Musculoskeletal deformities following treatment of Wilms' tumour.

Wilms' tumour is one of the most common neoplasms of infancy and childhood. Current treatment regimens result in a cure rate of about 80% for localized tumours but may also cause musculoskeletal deformities. Assessment of 21 patients previously treated for Wilms' tumour showed that all had flank atrophy on the treated side. Radiologic abnormalities included asymmetry of vertebral bodies, vertebral end-plate irregularities, scoliosis, kyphosis, platyspondyly and hypoplasia of the ilium. Although the vertebral changes following radiotherapy for Wilms' tumour are present from an early age and the potential is great for an increase in spinal deformity with growth, few spinal curves progress past 20 degree. Since one cannot predict which curves will progress, all such patients need careful orthopedic follow-up until skeletal maturity is achieved.

Child↗

Dominant inheritance of Scheuermann's juvenile kyphosis.

Familial occurrence of Scheuermann's juvenile kyphosis is well known, but no specific mode of inheritance has been recognized. We describe five families in which the disease seems to follow and autosomal dominant pattern of inheritance.

Adolescent↗

Simplified technique for scapulocostal fusion in facioscapulohumeral dystrophy.

Surgical stabilization of the scapula by a simplified technique is suggested. An average gain of nearly 30 degrees in both shoulder abduction and flexion with elimination of scapular winging resulted. In all cases, this allowed elevation of the arm above the head, with concomitant improvement in activities of daily living (ADL). There were no instances of pleuritic chest pain, hemothorax, or pneumothorax. There was no substantive change in pulmonary function. Scapulocostal fusion in patients with facioscapulohumeral (FSH) dystrophy is an effective operation to improve upper extremity function. Patients report a subjective increase in endurance and are uniformly pleased with the cosmetic result.

Activities of Daily Living↗

Predicting height from arm measurements.

Height measurement is required to standardize measures of physical capacity (e.g., pulmonary function) and to adjust drug dosage in the physically disabled. To estimate height in wheelchair-bound patients, we examined the relation of four different upper extremity measurements to height in 119 normal individuals aged 0.5-56 years. Regression analysis was performed for each of the following: (a) interacromioclavicular distance, (b) upper arm, (c) lower arm (cubit), (d) arm span; with height. Pearson correlation coefficients of 0.937, 0.967, 0.975, and 0.989, respectively, were obtained (accuracy +/- 5%, 90% confidence interval, for upper and lower arm measurements). We conclude that height can be predicted reliably from arm measurements.

Adolescent↗