Child nephrology and urology最新文献

Specific diagnostic and therapeutic considerations regarding urologic problems in the very young have encouraged the awareness that the treatment of congenital malformations requires special qualifications. In this paper 3 cases are presented to illustrate and stress our opinion that the treatment of congenital urologic anomalies in young children should be left to urologists who have been trained and work in the field of paediatric urology.

In the Milan hypertensive rats (MHS) glomerulosclerosis is less evident than in the normotensive strain (MNS). To clarify whether this pattern is due to a 'protective effect' of increased afferent arteriolar tone or to a different mechanism, we studied 12 first-generation hybrids (F1), 4 parental MHS and 4 parental MNS rats. Four-micrometer sections were stained with hematoxylin, Mallory's trichrome stain and periodic acid-Schiff reaction. The blood pressure of the F1 rats was only slightly higher than that of the MNS so that very probably renal vascular resistances were similar. The F1 rats had low proteinuria (23.3 +/- 2.7 mg/24 h) like the MHS (25.3 +/- 4.8), and few damaged glomeruli per section (18.5 +/- 1.2), again like the MHS (18.7 +/- 1.1). MNS had higher proteinuria (363.8 +/- 111.6; p less than 0.01 vs. MHS and F1) with a greater number of damaged glomeruli (51.4 +/- 4.5; p less than 0.01 vs. MHS and F1). The difference in afferent arteriolar resistance is not implicated in glomerulosclerosis.

The 7-year-old girl presenting with cushingoid appearance and gross hematuria was found to have a right renal tumor. Preoperative hormonal assay revealed an extremely high plasma ACTH level and elevated plasma cortisol levels. Right radical nephrectomy was performed, and pathology proved the tumor to be a clear cell sarcoma, a sarcomatous variant of Wilms' tumor, of the kidney. Postoperative plasma ACTH level declined dramatically and cortisol levels returned to normal, too. She received chemotherapy and radiotherapy then and gradually lost her cushingoid appearance. This is the first reported case of clear cell sarcoma of the kidney with ectopic ACTH syndrome.

The autopsy finding of congenital mesoblastic nephroma in a stillborn hydropic fetus is reported. We discuss the pathogenetic relation of both entities and summarize the optimal therapeutic attitude to follow in order to prevent this misfortunate outcome.

Inorganic sulfate reabsorption was studied during sulfate infusion in guinea pigs stabilized on high-sulfate, normal-sulfate or low-sulfate diets. Guinea pigs stabilized on a low-sulfate diet exhibited 86-91% fractional sulfate reabsorption at normal plasma sulfate concentrations. Fractional renal sulfate reabsorption in animals stabilized on a high-sulfate diet was 55-70%. Clearance techniques were used to determine the glomerular filtration rate and the fractional reabsorption of sulfate during sulfate infusion. Animals fed a high-sulfate diet manifested marked sulfaturia in response to sulfate infusion. The increase in fractional reabsorption associated with decreases in dietary sulfate intake suggests a tubular adaptive mechanism, similar to that demonstrated for phosphate, to increase sulfate reabsorption and maintain constant plasma sulfate concentration. Dietary sulfate-dependent alterations in renal sulfate reabsorption may play a significant role in establishing the rate of sulfate excretion and thus regulating sulfate balance.

Water and salt metabolism was studied in feti and pregnant rats with experimental venous congestion by measuring water and electrolyte content in the kidney, liver, cerebrum, skin hypodermic tissue, muscle, heart. In the kidney, venous occlusion resulted in increased potassium content. The role of the placenta is discussed.

The records of 178 children with a neural tube defect were reviewed to determine the pattern of associated congenital urinary malformation. Ten (6%) of the children had a significant congenital urinary malformation. Although unilateral renal agenesis was the most common single congenital urinary abnormality, as a group, abnormalities in fusion or migration were equally common in this study and more common in several previously reported studies. Lower congenital urinary tract abnormalities were also identified but were less common. No correlation was found between the level of the neural tube defect and the specific congenital urinary abnormality.

The available data on renal reserve and on the fraction of filtration capacity utilized at rest in childhood are analyzed both in healthy state and in renal disease. The methodological problems which might have biased available information are also discussed.

Eleven pediatric patients with nephrosis and focal segmental glomerulosclerosis were treated with long-term (8-38 months) ciclosporin A in combination with steroids. All had abnormal height-velocity curves and multiple hospitalizations for complications of nephrosis. Eight patients attained remission with a dramatic improvement in growth and decrease in necessity for hospitalization for therapy of nephrosis complications, while maintaining adequate renal function. Three nonresponders developed end-stage renal disease. Long-term ciclosporin A therapy may be of benefit in steroid-resistant nephrosis in childhood.