Stiff-man syndrome and emphysema.
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Biomedical subjects
Publications and source records attributed to J R Campbell.
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Severe renal osteodystrophy with metaphyseal fractures developed in two children with hypoplastic-dysplastic kidneys and chronic renal failure despite therapy with vitamin D, CaCO3, phosphate-binding agents, and protein restriction. Serum immunoreactive parathyroid hormone (iPTH) levels were elevated to 709 and 1,537 pg/mL (N = 255 +/- 92 pg/mL). Total parathyroidectomy and then autotransplantation of a small portion of parathyroids into the left brachioradialis muscle resulted in complete healing of renal osteodystrophy with the same dose of vitamin D. Serum iPTH and histological studies have demonstrated functioning parathyroid autotransplants, 19 and 20 months postoperatively in these two patients. Advantage of such a procedure over 3 3/4 parathyroidectomy is that this transplanted parathyroid tissue is easily accessible for partial removal in case of recurrence of uncontrollable hyperparathyroidism. We believe that total parathyroidectomy and autotransplantation can be successfully performed even in small children.
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Morphologically the canine cranial cruciate ligament can be divided into a cranio-medial and caudo-lateral component which perform reciprocal functions at all angles of flexion of the stifle joint. Histologically the main constituents of these two components are bundles of longitudinally orientated collagen fibre. The results of the study of the effect of partial and total sectioning of the cranial cruciate ligament on the "anterior-draw" movement implied that the relatively minor degree of movement, elicited following sectioning of either of the components of the ligament, would not be detected under clinical conditions. For joint instability to be clinically detectable most of the ligament must have ruptured, or the intact portion must have undergone degenerative or disruptive changes.
Sequential angiographic studies were done in six children to stage and assess the results of radiation and/or chemotherapy of solid abdominal malignancies: one bilateral Wilms' tumor, two neuroblastomas, two hepatoblastomas and one hepatocarcinoma. Angiography was of value in demonstrating the tumor, its location, extent and vascular characteristics, as well as its regression and recurrence. Wilms' tumor and neuroblastoma responded and well to radiation and chemotherapy with substantial decrease in tumor size and regression or disappearance of tumor neovasculature. Resceted tumors revealed this to be due to tumor necrosis, hemorrhage and/or cystic degeneration. Hepatoblastoma and hepatocarcinoma did not respond as well to chemotherapy, with only mild decrease in size and neovasculature of the tumor.
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A 13-year-old female with Hutchinson-Gilford progeria, who developed an osteosarcoma of the right chest wall, is reported. This is the first reported association of a malignant neoplasm with this syndrome.
Cyst wall vascularity not evident on abdominal aortography was demonstrated by selective magnification angiography in two children with complicated solitary, nonparasitic liver cysts. In one, its character and degree led to a false diagnosis of tumor. The observed mural vascularity probably reflected secondary changes of inflammation and necrosis in one case and prior surgery in the other case. Histologic findings in previously reported cases, however, indicate that, contrary to accepted angiographic beliefs, mural vascularity also occurs in many noncomplicated, simple liver cysts. Angiographers should be aware of this, and that the possibility exists of visualizing this mural vascularity by selective magnification angiography.
The experimental results are reported on a series of sheep in which unilateral hip excision arthroplasty was performed. These sheep were slaughtered at regular postoperative intervals. Radiography and histology were performed on the normal and excised hip joints of the experimental sheep to provide information of the formation of the pseudarthrosis that develops following excision arthroplasty. The efficiency of the false joint that develops is good, judged on a clinical basis. Continuous remodelling of the acetabulum and resected face was noted throughout the experimental period. A sequence of degenerative changes in the acetabular cartilage was observed followed by filling in of the acetabular favity with new bone. New bone also developed on the cemoral component of the pseudarthrosis in the form of osteophytes situated around the perimeter of the resected surface. Growth deformities of the contralateral, nonoperated limb were induced.
Although the femorotibial joint is subject to a number of common abnormalities which have been investigated in the last few years there is still no obvious and agreed optimal treatment in many instances of abnormality. The clinical derangements which occur in the joint are considered and an assessment is made of the state of aetiological knowledge. The most useful way of diagnosing and treating the conditions are also considered.
An analysis has been made of the long term results of excision of the canine femoral head and neck. The most frequent postoperative findings include shortening of the operated limb and restricted movement of the hip pseudarthrosis. Although few dogs show lameness or pain in the operated hip, muscle atrophy is a frequent finding even after eight years. Difficulty in jumping and in climbing stairs are more commonly found postoperatively in the larger breeds of dog. Despite these noted abnormalities almost all owners expressed their satisfaction with the outcome of excision arthroplasty.
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The present study demonstrates that a 2 hr period of sustained hypoxia results in a focally destructive process, a process which resolves by 5 days. That the changes described in earlier work are not overwhelming to the animal suggest that an early, focal, and reversible stage of the development of acute enterocolitis in the puppy can be described with the aid of electron microsocpy.
The hemolytic-uremic syndrome consists of hemolytic anemia, renal failure, and thrombocytopenia. Submucosal hemorrhage with "thumbprinting" on roentgenographic examination of the colon with barium was demonstrated in four patients, prolapse of the rectum in two patients, and pseudomembranous enterocolitis and toxic megacolon in one. These lesions are not generally associated with hemolytic-uremic syndrome. The presence of these lesions in a child with bloody diarrhea should suggest hemolytic-uremic syndrome as a possible diagnosis. Sigmoidoscopy and roentgenographic examination of the colon with barium should be done in selected patients with hemolytic-uremic syndrome to evaluate the degree of colonic involvement and the need for surgery.
The effect of systemic hypoxemia and hypovolemia on pulmonary fine structure was studied and compared in 12 Labrador puppies 6 days to 3 weeks old. Central venous and arterial pressures were monitored and arterial pH, PCO2 and PO2 recorded serially. In 4 puppies lung biopsies were taken after a two hour period of breathing 7% oxygen. Four other puppies were made hypovolemic by acute hemorrhage of 50% of the calculated blood volume and lung biopsies taken after one hour. Four control puppies were maintained under anesthesia for two hours, breathing an atmosphere of 20% oxygen before lung biopsies were taken. Both hypoxemia and hypovolemia resulted in similar pulmonary ultrastructural changes. These changes which occurred more frequently in areas where the air spaces and blood vessels were expanded, were characterized by aggregation of platelets and leucocytes in the vascular space, focal endothelial cell disruption, interstitial edema and type I epithelial cell discontinuities with exudation of plasma into the alveolar air spaces.