Specificity of iodobenzylguanidine scanning in neuroblastoma.
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Biomedical subjects
Publications and source records attributed to C Dicks-Mireaux.
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We present our findings in two unrelated patients with the characteristic clinical and radiological features of the Winchester syndrome. The histological findings in gum and skin biopsies taken from one of the subjects, indicated excessive collagen turnover (active phagocytosis, an active endoplasmic reticulum, and an abundance of fibrillogranular material of probable collagen origin). An abnormal oligosaccharide was detected in urine from both patients which was identified as a trisaccharide containing one fucose and two galactose residues. The finding of this oligosaccharide may prove a useful marker in other cases of this rare syndrome and may help elucidate the underlying biochemical defect.
The antigen to which the monoclonal antibody UJ13A binds is expressed by normal primate brain and most tumors of neuroectodermal origin. The antibody has been used in radioimmunologic studies in patients with neuroblastoma and glioma, though the uptake of antibody into tumors is low. Animal models have been sought to allow definition of the parameters that influence tumor uptake. Animals include nude mice, in which xenografted human tumors can be studied, and marmosets, in which the primate response to exposure to murine antibodies can be investigated. This presentation reports the preliminary pharmacokinetic data from studies of mice and marmosets and compares the data with those obtained in patients.
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Nine patients developed osteochondromata, a mean of 6 years after total body irradiation (TBI) given before bone marrow transplantation for childhood leukaemia. This represents 23% of patients receiving TBI during the period from 1981 to 1989 surviving > or =5 years after bone marrow transplantation. The patients were followed up for a mean of 12.5 years from diagnosis of leukaemia and a mean of 2.5 years from diagnosis of osteochondromata. No osteochondroma, including three lesions removed surgically, showed evidence of malignant change. Six patients received growth hormone for irradiation-induced growth hormone deficiency, but this did not appear to influence the natural history of the osteochondromata. Radiation-induced osteochondromata (RIO) are often multiple and are indistinguishable from the more common idiopathic type. The incidence of RIO after TBI was higher than that reported after local irradiation.