A 16-year-old female with acro-renal-ocular syndrome complicated by ventricular septal defect is described. Renal biopsy was performed for the first time in this syndrome, and the results suggested that proteinuria and renal dysfunction were caused by chronic pyelonephritis secondary to malrotation of the kidney and anomalous pelves. Chronic renal failure and hypoplasia of the optic papillae were also observed in the patient’s mother, suggesting a participation of heredity in the pathogenesis of the syndrome.
The effect of acute denervation of the kidney on renal sodium and water excretion, and hemodynamics including intrarenal blood flow, was studied in anesthetized dogs. The intrarenal blood flow was measured by the radioactive microsphere method. In all experiments denervation natriuresis and diuresis were observed without significant change in glomerular filtration rate, renal blood flow and distribution of intrarenal blood flow. There was, however, an associated increase in potassium excretion. We suggest that denervation natriuresis and diuresis may be caused by the elimination of a direct nervous control of sodium and water reabsorption.
The effects of intrarenal arterial (6.5 µg/kg/min) and intravenous (33 µg/kg/min) administration of propranolol on sodium excretion and renal hemodynamics were studied in anesthetized dogs. Heart rate, plasma renin activity and renal plasma flow were reduced by propranolol administered by either route. Blood pressure was reduced following intravenous infusion. Urinary sodium excretion was reduced by propranolol. This reduction was inversely correlated with the basal state of sodium excretion, and was also correlated with the reduction of glomerular filtration rate, effective renal plasma flow and blood pressure. We suggest that the antinatriuretic effect of propranolol is due to the reduction of glomerular filtration rate and of hydrostatic pressure in the proximal tubule, although additional factor(s) are not completely ruled out.
The effects of acute denervation of the kidney on renal tubular sodium and water excretion were studied in anesthetized, hypophysectomized, and cortisone-treated mongrel dogs during stable water diuresis produced by the infusion of 2.5% dextrose. In all experiments, denervation natriuresis, and diuresis were observed without significant change in glomerular filtration rate (GRF) and renal plasma flow (RPF). Fractional sodium delivery to the distal nephron (CNa + CH2O/100 ml GFR) and fractional free water clearance (CH23/100 ml GFR) was significantly greater in the denervated kidney compared with the innervated kidney (9.6+/-1.2 vs. 6.7+/-0.9% and 8.8+/-1.2 vs. 6.5+/-0.8%, respectively). Distal tubular sodium reabsorption (CH2O/(CNa + CH2O)) was not significantly different. We conclude that renal denervation primarily affects the proximal tubule as manifested by a decrease in the reabsorption of sodium and water. A small effect of denervation on the distal nephron is not completely ruled out.
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