The pullback pressure from the left renal vein (LRV) to the inferior vena cava was studied in 16 patients with left renal bleeding of unknown origin (group A), 15 patients with hematuria of miscellaneous laterality (group B), and nine control subjects (group C). The mean pressure gradients were 5.0 +/- 2.0 mm Hg (mean +/- SD) in group A, 1.9 +/- 1.0 mm Hg in group B, and 1.1 +/- 0.9 mm Hg in group C. The mean pressure gradient of group A was significantly higher than that of groups B and C (P less than .001). From the results of control studies, we regarded pressure gradients greater than or equal to 3.0 mm Hg as indicative of LRV hypertension. With this criterion, 88% (14 of 16) of the patients in group A had LRV hypertension, which was considered a cause of hematuria. Analysis of the results of renal angiography in the 31 patients revealed that opacification of collateral pathways of the LRV was a significant angiographic finding in LRV hypertension.
A sister and brother with Vici syndrome are described. They both had oculocutaneous albinism, agenesis of the corpus callosum, cataracts, and cardiomyopathy. They were born to healthy unrelated parents, and had postnatal growth retardation, profound developmental delay, hypotonia, and cataracts. The sister had recurrent infections, and died of progressive heart failure at age 19 months. The brother is alive at age six months with mild cardiomyopathy, and had a single episode of acute bronchitis at age three months. Review of the clinical manifestations of the sibs we described and six children reported in the literature indicates that Vici syndrome is a distinct clinical entity. Its main clinical manifestations include growth retardation, profound developmental delay, hypotonia, albinism, agenesis of the corpus callosum, cataracts, cardiomyopathy, and recurrent infections. The occurrence of the syndrome in three pairs of sibs of both sexes born to unaffected parents supports autosomal recessive inheritance.
We examined pp60c-src protein kinase activity in human gastric carcinoma cell lines and gastric carcinoma tissues as well as normal mucosa. pp60c-src kinase activity was detected in all 5 carcinoma cell lines at various levels. Of 16 gastric carcinoma tissues, 8 showed higher pp60c-src kinase activity in tumor tissues than in corresponding normal mucosa. However, the levels of expression of pp60c-src detected by Western blotting were not always consistent with the activities of pp60c-src protein kinase. These findings suggest that the increase in pp60c-src protein kinase activity might be brought about by post-translational changes.
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