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Biomedical subjects

J M Xipell

Publications and source records attributed to J M Xipell.

At least 19 recordsLinked to original sources

Osteofibrous dysplasia of the tibia and fibula.

Osteofibrous dysplasia (ossifying fibroma of long bone) is an uncommon disorder of bone affecting the tibia and, rarely, the fibula of children and adolescents. It has been delineated from monostotic fibrous dysplasia, from which it can be distinguished by its radiological and histological characteristics and by its clinical course. We report 11 patients with osteofibrous dysplasia whose ages ranged from 10 months to 22 years. Four presented with a pathological fracture and in 2 a pseudarthrosis developed. A circumscribed area of intracortical lucency and expansion in the diaphysis is the characteristic radiological finding. Histologically there is a similarity to fibrous dysplasia but the trabeculae are lamellar or have a lamellar surface and most show osteoblastic rimming. Treatment may be difficult but more recent experience strongly favours a conservative approach wherever possible.

Adolescent↗

Benign fibrous histiocytoma of bone.

The clinical, radiologic, and pathological features of eight cases of fibrohistiocytic bone lesions histologically identical to the nonossifying fibroma of childhood are presented. They differed from the childhood lesion in their clinical and radiological features. They occurred in adults, and were frequently associated with pain in the absence of complicating fracture. They were not confined to the metaphysis of long bones. When metaphyseal, the lesions also frequently showed a tendency to involve the epiphysis. Others occurred in the diaphysis of long bones, in the pelvis, and in a rib. Three recurred locally, but none has metastasized. Other fibrohistiocytic and fibroblastic tumours of bone, including malignant fibrous histiocytoma, giant cell tumour, fibrosarcoma, and desmoplastic fibroma can be differentiated on radiological and histological features, and hyperparathyroidism may need to be excluded by biochemical investigations.

Adult↗

Dialysis osteomalacia: a possible role for zirconium as well as aluminium.

Six patients with dialysis osteomalacia were studied before and after treatment with desferrioxamine. Before treatment, all six had severe osteomalacia with histochemical evidence of metals at the mineralization front, confirmed by energy dispersive X-ray microanalysis to include Al, Zr and Fe. Zr was not detected by histological staining in patients without dialysis osteomalacia. After treatment a decrease of Al and Zr was associated with improvement in clinical, biochemical, radiological and histological parameters. These observations suggest the possibility of a role for metals other than Al in the pathogenesis of dialysis osteomalacia.

Chronic Kidney Disease-Mineral and Bone Disorder↗

Parathyroidectomy in chronic renal failure.

Parathyroidectomy was carried out in 26 patients over a 14-year period. Excellent results were obtained in patients with severe hyperparathyroidism. Vascular calcification, hypercalcaemia and pruritus did not justify surgery unless associated with unequivocal hyperparathyroidism. 13 patients required intravenous calcium infusion for up to 2 weeks to control post-operative hypocalcaemia. Calcium requirements could be predicted from the pre-operative plasma alkaline phosphatase level. Following operation continued treatment with vitamin D was necessary to prevent hypocalcaemia. Hyperparathyroidism recurred in 1 patient after 8 years and 4 patients developed osteomalacia. Since parathyroid hormone may have toxic effects other than those on bone, maintenance of normal levels should be a long-term objective in the treatment of patients with chronic renal failure. Where large parathyroid glands are present, surgical reduction in gland mass is a logical prelude to long-term suppression of parathyroid hormone with vitamin D and phosphate-binding agents.

Adolescent↗

Gelatinous transformation of the bone marrow.

A case of gross macroscopic gelatinous transformation of bone marrow associated with prolonged malabsorption due to celiac disease is reported. Histochemical studies support the view that the extracellular myxoid tissue is composed predominantly of acid mucopolysaccharides, and electron microscopy shows extracellular fine fibrillar and granular material. The replacement of marrow in this condition is histochemically different from serous fat atrophy occurring in epicardial fat, and it is suggested that it results from an increase in normal ground substance. It must be differentiated from metastatic signet ring adenocarcinoma.

Adenocarcinoma, Papillary↗

Treatment of dialysis osteomalacia with desferrioxamine.

Two patients with severe dialysis osteomalacia, whose fractures and deformities had become progressively worse over 3 years, were treated with desferrioxamine (DFO). They improved rapidly and over 6 months were able to stop taking analgesics and return to normal activities. Pre-treatment bone biopsy samples contained extensive osteoid covering trabecular surfaces. Electron microscopy showed morphological abnormalities in the matrix. Treatment brought about a decrease in the extent and depth of osteoid, but matrix abnormalities remained. Energy-dispersive X-ray microanalysis showed the presence of several metals not seen in normal bone. The changes in trace metals after DFO treatment were not consistent. Atomic absorption analysis showed no significant change in the aluminium content of bone with treatment. DFO appears to be an effective treatment for severe dialysis osteomalacia.

Adult↗

Assessment of osteodystrophy in patients with chronic renal failure.

Nine patients with chronic renal failure were followed for more than one year by serial bone biopsies which were assessed by quantitative histological techniques. All patients had evidence of bone disease; this progressed during the interbiopsy period in eight. Patients who had the most advanced histologic disease at initial biopsy showed the most progression in resorption and demineralization, but with greater progression of hyperparathyroid bone disease than osteomalacia. The type of bone disease and its rate of progression could only be accurately assessed histologically. No predictive parameters of early bone disease were found from clinical history, biochemistry or radiology. Raised serum alkaline phosphatase occurred only in advanced hyperparathyroid bone disease. Minor radiological abnormalities in magnified views of the hands were indicative of histologically advanced asymptomatic hyperparathyroidism.

Adult↗

Malignant mesothelioma: a review of 35 cases with diagnosis and prognosis.

Twenty-five men and 10 women with malignant mesothelioma seen at the Austin Hospital between 1965 and 1978 were reviewed. The patients ranged in age from 34 to 79 yr. Prognosis was poor but was somewhat better for patients with epithelioid tumours than for patients with fibrous mesotheliomas and biphasic tumours. A history of occupational exposure to asbestos was obtained in the majority of patients, the latent period being from 18 to 52 yr. Ten patients had no history of asbestos exposure. The pathological diagnosis was established on tissue obtained at open biopsy in 22 cases and at post mortem in 11. Needle biopsy provided a diagnosis in only 2 cases. Cytology suggested a diagnosis of mesothelioma in 1 case. Gross pathology in general conformed with a diffuse confluent-nodular serosal tumor. A right sided preponderance was found in pleural tumours, and metastases were found rather more frequently than is usually reported. Histological features were distinctive in the majority of cases provided that sufficient material was examined. Recognition of coexisting patterns was often more helpful in making the diagnosis than any one single feature. Histochemical reactions are useful in distinguishing mesothelioma from mucin-secreting adenocarcinoma. In a majority of cases the histology and histochemistry were not characteristic and the diagnosis depended on the clinical features and the absence of another primary source of tumour.

Adult↗

Histology of normal bone--a computerized study in the iliac crest.

The application of an electronic digitizer and a computerized program for the quantitative assessment of bone morphology is reported. The method was used to measure bone surfaces and areas in undecalcified sections of iliac crest in 50 control subjects at necropsy.

Adult↗

Clinical, biochemical and histological observations on the effect of porcine calcitonin in Paget's disease of bone.

The response to porcine calcitonin has been assessed in 38 patients with Paget's disease, observed during 44 treatment periods of from three to 42 months. In 36 of the treatment courses significant relief of pain was achieved but the contribution of placebo effect could not be determined. Serum alkaline phosphatase and urinary hydroxyproline levels reached normal in a few patients, but the grouped data indicated a plateau effect above the range of normal. The acute hypocalcaemic response to calcitonin was lost only in those patients whose bone turnover was restored to normal. Quantitative histology on iliac crest bone biopsy samples showed no statisically significant lowering of osteoclast counts. No antibody-based clinical resistance occurred and the incidence of side effects was low. The results indicate that porcine calcitonin is a useful treatment of Paget's disease, and the experience of the study helps in arriving at patient selection and treatment schedules. Treatment is recommended for bone pain and for active disease in the relatively young, using intermittent therapy with course of at least six months duration. Resumption of therapy is based on clinical and biochemical indications.

Adult↗

Acute silicoproteinosis.

A case of alveolar lipoproteinosis associated with silicosis is reported. A 58-year-old man had been exposed to silica for seven years and died three years after the onset of symptoms. Light microscopy of biopsy and necropsy material showed small silicotic nodules, silica particles, and alveolar lipoproteinosis, and ultrastructural studies were performed to define changes in alveolar epithelium and macrophages. The case provides a further example of alveolar lipoproteinosis developing as a response of the lung to injury by an external agent.

Acute Disease↗

Ewing's sarcoma.

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Adolescent↗