Long range plan of the National Kidney and Urologic Diseases Advisory Board.
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Biomedical subjects
Publications and source records attributed to V Braren.
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During a ten-year period, 35 children presenting with vesicoureteral reflux, ureteropelvic junction obstruction, or a "small kidney" were found to be hypertensive. Of these, 15 subsequently underwent surgical procedures for relief of hypertension. Seven were "cured," six were "improved," and two were "unchanged." The severity of hypertension could not be correlated with the degree of reflux nor with the degree of obstructive uropathy. However, all children with reflux in our study who were hypertensive had some degree of calicectasis noted preoperatively on intravenous pyelogram. Also it was noted that hypertension may occur several years after successful anti-reflux surgery. Children with vesicoureteral reflux, ureteropelvic junction obstruction, or a small kidney need to have blood pressure determinations at regular intervals, even if all previous readings had been in the normotensive range and whether or not they were followed up medically or post surgically. We suggest that blood pressure determinations be made every three months for the first year after diagnosis of reflux or ureteropelvic junction obstruction, and at least once a year thereafter.
We believe that the confusion regarding abnormal renal development could be reduced by more precise terminology. Therefore, we suggest precise definitions for dysgenesis, hypoplasia, dysplasia, hypodysplasia, aplasia and agenesis of the kidney. We suggest the term reflux nephropathy be a generic label for any instance of abnormal renal morphology (gross or microscopic) associated with vesicoureteral reflux. Hypoplasia and hypodysplasia can be subclassified on the basis of associated urological criteria. There have been many previous attempts to classify cystic disease of the kidney but none has been accepted collectively by pathologists, urologists, nephrologists and radiologists. On the basis of known patterns of inheritance, a classification is outlined in which renal cystic disease is divided into 2 major groups: genetic and nongenetic. Each entity is discussed.
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Our group has undertaken the determination of serum levels of beta-2-microglobulin in 250 patients. We have compared this diagnostic entity to radioisotopic glomerular filtration rate, serum creatinine, and blood urea nitrogen. We have also analyzed the cost effectiveness of each of these diagnostic entities and combinations thereof. We conclude that beta-2-microglobulin is a simple test to perform; it is specific, sensitive, and accurate. Its cost effectiveness is excellent. We advocate its wide clinical usage.
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The results of the application of clean intermittent catheterization to children are discussed. Of 34 patients only 7 maintained persistently sterile urine and 52 per cent had more than 1 positive culture in the followup period. Complications of the application of this technique are discussed. The major complication rate was 15 per cent and 6 per cent were considered failures of therapy. Only 1 of the 34 patients demonstrated progression of upper tract disease by excretory urography during the followup period. Intermittent catheterization was found to be effective in preserving renal function as well as helping to improve patient social acceptability.
Sacral agenesis is an uncommon congenital anomaly that usually is accompanied by other orthopedic anomalies but often occurs singularly. We report our experience in the treatment and long-term followup of 4 cases of pure sacral agenesis. We strongly stress the need for early diagnosis and long-term careful followup. In our group the diagnosis was often overlooked and followup was poor. Studies must be undertaken to determine the type and degree of neurogenic vesicopathy in each child since this may vary from one patient to the next. We urge the early and long-term use of intermittent catheterization.
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Children with myelodysplasia are best managed in medical centers by a team consisting of a urologist, an orthopedist, a neurosurgeon, a pediatrician, a social worker, an orthotic technician and physical therapists. A co-operative approach aids in the selection of a urinary diversion stoma site which will not interfere with the multiple orthopedic procedures these children must undergo. The urologic and orthopedic complications and benefits of 24 patients with umbilical stoma are compared with 24 patients with matched right lower quadrant stoma. The incidence of urologic complications is equal, while the orthopedic advantages manifest by the first group vastly outweigh the problems encountered in the second group. We advocate the use of a midline stoma site for urinary diversion in children with myelodysplasia.
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We report 3 cases of a series of 372 (0.8 per cent) renal transplant recipients in whom urinary tract calculi developed as a complication of the procedure. In each patient symptoms were minimal and not classic of calculous disease. We disagree with the contention that all such patients have either hypercalcemia, infection, or tubular acidosis, although thorough evaluation is indicated. We believe this entity should be considered in problematic renal transplantation patients. Conservative therapy is advocated when the situation permits.
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Since June 1, 1971 patients undergoing evaluation for hypertension have been evaluated with rapid sequence excretary urography, abdominal aortography and selective renal arteriography. Renal venous assays have been done in selected patients. Through July 31, 1974, 812 arteriographic studies in new hypertensive patients have been done. In 6 of these patients renal tumors were discovered by arteriography only and were not suggested by symptoms, urinalysis nor diagnosed on hypertensive excretory urography. This high incidence surprised us and we hope others will review their arteriographic series of hypertensive patients.
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