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The role of antibodies directed against double-stranded DNA in the manifestations of systemic lupus erythematosus in childhood.

The specificity of antibodies directed against dsDNA for SLE in a childhood population was tested by analyzing sera from 62 children with lupus and 283 children with other known or suspected autoimmune diseases. The role of these antibodies in the manifestations of SLE was then examined by correlating dsDNA Ab titer with clinical manifestations in 311 sera from 20 children followed for a mean of 51 months. Antibodies to dsDNA were found to be highly specific for SLE. The presence of antibodies in titers of 1:80 or greater correlated with the presence of active disease, arthritis, and rash, but not with azotemia, proteinuria, or increasing proteinuria; this indicated that their role in the induction of lupus nephritis was different from that in the induction of rash and arthritis. This may be due to a requirement for small immune complex formation during times of antigen excess in the initiation of lupus nephritis.

Adult↗

Renal failure in children with hepatic failure undergoing liver transplantation.

Over a 3 1/2 year period, 133 children with hepatic failure underwent orthotopic liver transplantation (OLT) at our center. Renal failure (creatinine clearance less than 20 ml/min/1.73 m2) was present in 19 (14.3%) of these children. In seven of the 19 children, renal failure was present before OLT, and in the other 12 after OLT. The causes of renal failure included hepatorenal syndrome in seven, postischemic acute tubular necrosis in five, severe prerenal azotemia in five, and cyclosporine nephrotoxicity in two. Eight other patients died of renal failure while awaiting emergency transplantation. Of the total of 31 deaths among 133 children who underwent OLT, nine occurred in the 19 patients with renal failure. Thus patients with OLT and renal failure had a significantly higher mortality than other patients with transplants (P less than 0.025). Dialysis was not associated with improved survival. The majority of deaths in patients with renal failure were related to severe hemorrhage, thromboembolic events, and systemic fungal infections. Our experience suggests that renal failure is common in children with hepatic failure and is associated with reduced patient survival after OLT.

Acute Kidney Injury↗

Acquired immunodeficiency syndrome-associated renal disease in children.

Five children with acquired immunodeficiency syndrome (AIDS) and clinically significant renal disease had detailed pathologic examination of renal tissue (biopsy specimens, autopsy specimens, or both). All patients had proteinuria, hypoalbuminemia, and edema; one patient had persistent azotemia. In two cases, renal disease was the first manifestation of human immunodeficiency virus (HIV) infection. All patients had progressive renal disease, and four of the five died. Pathologic studies revealed focal glomerulosclerosis and mesangial proliferative glomerulonephritis with deposits of immunoglobulins and complement demonstrated by immunofluorescence and electron microscopy. Characteristic tubuloreticular structures were also demonstrated in the glomerular endothelial or epithelial cells in two cases. Renal disease is part of the multisystem involvement in children with AIDS. The pathogenesis of renal disease is not known, but circulating immune complexes are known to occur in children with HIV infection and may be involved.

Acquired Immunodeficiency Syndrome↗

Reconstructive surgery for the management of genitourinary tuberculosis: a single center experience.

PURPOSE: We evaluated the role of surgery for genitourinary tuberculosis with special emphasis on reconstructive procedures. MATERIALS AND METHODS: Case records of 241 patients with genitourinary tuberculosis who underwent surgery at our center during a period of 17 years were reviewed. Clinical features, organ involvement, investigations, treatment and outcome of therapy were studied. RESULTS: There were 129 males and 112 females with a mean age of 34.6 years. The most common presentation was irritative voiding symptoms. Azotemia was seen in 54 (22.4%) cases. The most commonly involved organ was the kidney in 130 (53.94%) cases. Preoperative bacteriologic diagnosis was confirmed in 70 (29%) cases. All patients received antitubercular drug therapy for 9 months. A total of 248 procedures, including 33 endoscopic, 87 ablative and 128 reconstructive, were performed with some patients requiring more than 1 procedure. Early complications, which mainly involved the bowel, were seen in 19 (7.88%) cases. Bacteriologic cure was achieved in all culture positive cases. Renal functional parameters stabilized or improved in 44 of 54 patients (81.5%) in whom they were deranged at presentation. CONCLUSIONS: Genitourinary tuberculosis is common in developing countries. Diagnosis is often delayed because of late presentation and many patients present with cicatrization sequelae. A combination of antitubercular drug therapy and judicious surgery achieves satisfactory results in the majority of cases. With improved antitubercular drug therapy and experience with the use of bowel segments in the urinary tract, more reconstructive procedures are being performed with satisfactory outcomes. In patients who undergo reconstructive procedures, a rigorous and prolonged followup is necessary.

Adolescent↗

Outcome after temporary vascular occlusion for the management of renal trauma.

To determine the impact of temporary vascular occlusion on patient outcome after surgery for renal trauma, we reviewed the records of 30 patients managed since 1977 whose injuries represented 17% of a total of 181 injuries in 175 patients. Reconstruction was judged to be adequate in 25 patients, while the remaining 5 underwent immediate nephrectomy. Compared with patients whose renal injuries did not require temporary occlusion, these 30 were more likely to have renovascular trauma, shock at presentation and higher transfusion requirements. Postoperatively, of 20 patients renal imaging (9) and radionuclide scanning (11) demonstrated preservation of significant renal parenchyma or function in 18 (90%). Although complications were more common in patients whose renal injuries required temporary vascular occlusion, only 2 were related to the renal injury or its method of repair (urinary extravasation in 1 patient and azotemia in 1 with bilateral injury). Temporary vascular occlusion can be performed expeditiously and safely, and may have an important role in preserving renal function. Our results support the routine use of early vascular control and the selective use of temporary vascular occlusion in renal injuries requiring exploration.

Adolescent↗

Management of renal cell carcinoma with coexistent renal artery disease.

Coexistence of renal cell carcinoma and renal artery disease is an unusual and challenging problem. From 1969 to 1991, 34 patients presented with localized renal cell carcinoma and renal artery disease affecting all of the functioning renal parenchyma. These patients represented 4 categories: 1) a solitary kidney with renal cell carcinoma and renal artery disease (5), 2) bilateral renal cell carcinoma and coexistent renal artery disease (5), 3) unilateral renal cell carcinoma and contralateral renal artery disease (13), and 4) unilateral renal cell carcinoma and bilateral renal artery disease (11). Atherosclerosis was the most common cause of renal artery disease (30), followed by medial fibroplasia (2), renal artery aneurysm (1) and arteriovenous malformation (1). A total of 23 patients (68%) presented with azotemia (serum creatinine 1.5 mg./dl. or more) and 11 (32%) presented with hypertension. All patients underwent complete surgical excision of renal cell carcinoma. A nephron sparing operation was performed preferentially (30 patients) and bilateral renal cancer operations were staged. Eight patients underwent simultaneous partial (6) or radical (2) nephrectomy and surgical renal revascularization. There were no operative deaths. Postoperatively, preservation of renal function was achieved in 33 patients and 1 required chronic dialysis. At mean followup of 47 months 23 patients (68%) were alive with no evidence of malignancy and 2 were alive with recurrent renal cell carcinoma. Three patients died of metastatic renal cell carcinoma, while 6 died of unrelated causes. All of the latter 6 patients were free of renal cell carcinoma at death. Nephron sparing surgery combined occasionally with renal arterial reconstruction can yield gratifying results in this complex patient population.

Adult↗

Laparoscopically assisted percutaneous renal biopsy.

We performed laparoscopically assisted percutaneous renal biopsy on 4 patients with azotemia or renal dysfunction who were believed to be unsuitable candidates for percutaneous renal biopsy. Tissue adequate for diagnosis was obtained in all 4 cases. Complications included subcutaneous emphysema in 1 patient and a small splenic capsular tear in 1, which was managed laparoscopically and did not require transfusion. Bleeding from the renal biopsy occurred in 1 patient and was easily managed laparoscopically. We recommend laparoscopically assisted percutaneous renal biopsy as an alternative method of renal biopsy in patients who can tolerate general anesthesia and who are not candidates for percutaneous renal biopsy.

Adult↗

Long-term indwelling bilateral ureteral stents for bilateral hydronephrosis of unknown etiology.

We describe a rare case of long-term indwelling bilateral ureteral stents that were replaced yearly for 7 years because of azotemia associated with bilateral hydronephrosis of undetermined etiology on radiological examinations. During year 5 the patient had an uneventful gestation with delivery of a normal neonate by cesarean section. Hydronephrosis remained improved for 2 years after withdrawal of the stents.

Adult↗

Urease and the acidosis of urinary intestinal diversion.

Previous investigators have suggested that urinary tract infections with urea-splitting organisms may be a primary etiologic factor in the acidosis which is seen after urinary diversion. This study employs a model in which small intestinal segments are perfused with an artificial urine solution over a three hour period. Urease is then added in order to determine its effect on acid-base balance and net intestinal electrolyte transport. Urease created no significant increase in acid load (delta HCO3- = -7.5 +/- 2.2 for controls vs. -8.7 +/- 2.9 for urease group), but did increase the osmolality of the intestinal contents and resulted in a 24% increase in free water loss (p = .037). Analysis of sodium and chloride movement following the addition of urease to the perfusate suggests that both ammonium and bicarbonate are absorbed by the intestinal segment. Thus any acidosis resulting from increased ammonium absorption following the addition of urease appears to be offset by concomitant bicarbonate absorption. The azotemia of urinary diversion appears to be primarily the result of urea absorption, partially the result of ammonium absorption, and is not significantly increased by urease.

Acid-Base Equilibrium↗

Treatment of idiopathic retroperitoneal fibrosis by immunosuppression.

Idiopathic retroperitoneal fibrosis is exceedingly uncommon in childhood and its etiology is uncertain. Support for an immunological basis for the disease is given by a report of a 14-year-old girl with severe retroperitoneal fibrosis causing progressive azotemia in whom azathioprine and prednisolone were used successfully. This case supports the efficacy of immunotherapy in the treatment of idiopathic retroperitoneal fibrosis.

Adolescent↗

Systemic bacillus-Calmette Guerin infection after transurethral administration for superficial bladder carcinoma.

It is well known that intravesical bacillus Calmette-Guerin therapy is a safe and effective treatment for superficial bladder carcinoma with few serious complications. However, we report a case of pneumonitis, hepatitis, azotemia, fever and pancytopenia in a patient after transurethral administration of bacillus Calmette-Guerin. Even though severe systemic complications are rare, patients who have high fevers of signs of other systemic manifestations require documentation of the extent of the infection. Also, we address the use of prophylactic antituberculous drugs, not only to prevent these symptoms but also to raise the questions of diminishing the immunological effect of the treatment.

Administration, Intravesical↗

Ureteral replacement using ileum in compromised renal function.

Ureteral replacement by ileum is an accepted technique in a highly selective patient population. Two major contraindications in using an ileal ureter are compromised renal function (serum creatinine greater than 2) and a functionally abnormal bladder. We used ileum to bridge a ureteral defect in animals with half of a solitary kidney and low grade azotemia to see if the antirefluxing mechanism of the lower ureter prevented further deterioration in renal function. Twelve female mongrel dogs underwent a right nephrectomy, followed by a left partial nephrectomy six weeks later. Group I (six dogs) had a six cm. segment of ileum interposed between the upper and lower ureteral segments (nonrefluxing). Group II (five dogs) had a ten cm. segment of ileum placed from the upper third of the ureter to the bladder (refluxing). Cystograms, intravenous pyelograms, serum electrolytes, BUN and creatinine were obtained preoperatively, six weeks after the right nephrectomy, one month after left partial nephrectomy and six months after ileal replacement prior to sacrifice. The BUN and creatinine deteriorated in Group II compared to Group I, p = .02 and p = 0.4 respectively (Mann-Whitney test). The BUN and creatinine also deteriorated between one month after left partial nephrectomy and six months after ileal replacement within Group II, p = .07 and p = .14, respectively (Wilcoxon matched-pairs test) but not in Group I. These data suggest that the antirefluxing mechanism of the lower ureter might prevent further deterioration in renal function. We feel that ileum can be used with caution, as an interposition in compromised renal function.

Animals↗

Early vascular control for renal trauma: a critical review.

To determine the incidence of, indications for and sequelae of temporary vascular occlusion in the management of renal trauma the records of 90 patients with 92 renal injuries were reviewed. Patients who required temporary vascular occlusion were compared to patients with similar injuries who also required renal surgery but not vascular occlusion. Only 11 of 92 renal injuries (12 per cent) required temporary vascular occlusion for reconstruction and control of renal bleeding. Neither the type nor the extent of renal and associated injury was a significant predictor of the need for occlusion. Renal injuries associated with large or expanding retroperitoneal hematomas were more likely to require temporary vascular occlusion than injuries without such associated hematomas. Temporary vascular occlusion was not associated with an increase in postoperative azotemia or mortality. The procedure is safe and effective, and allows for confident inspection and reconstruction of the kidney in patients who otherwise would be at high risk for nephrectomy.

Constriction↗

Congenital progressive hydronephrosis in mice: a new recessive mutation.

We describe a new autosomal recessive mutation, congenital progressive hydronephrosis (cph), that arose in the C57BL/6J inbred mouse strain. The clinical, histopathological, biochemical and radiographic characteristics, and the genetic linkage of this new mutation are discussed. Our studies indicate that the homozygous mutant mice have progressive bilateral upper urinary tract obstruction leading to azotemia and death of renal failure. The anatomical site of obstruction appears to be at the level of the ureteropelvic junction. Genetic mapping studies have localized the cph gene to the distal half of chromosome 15. The cph mouse strain provides a reproducible model for analysis of the onset and development of obstructive uropathic conditions in the neonatal period.

Animals↗

A comparison of estramustine phosphate versus cis-platinum alone versus estramustine phosphate plus cis-platinum in patients with advanced hormone refractory prostate cancer who had had extensive irradiation to the pelvis or lumbosacral area.

Single and combination chemotherapy was compared in a clinical trial for men with advanced, metastatic prostate cancer who had received prior pelvic irradiation and had had progression of disease despite hormonal therapy. The 149 patients were randomized to receive estramustine phosphate or cis-platinum alone or in combination. Of the 149 patients 25 (17 per cent) were excluded from the study but 124 were evaluated for response and survival. Entry variables were distributed similarly among patients in each treatment arm. There were no complete or partial responders but there were nearly twice as many patients whose disease was stabilized (33 per cent) on the combination regimen compared to estramustine phosphate (18 per cent) and about a third more than for cis-platinum (21 per cent). Analysis of survival revealed some advantage for patients on combination therapy. Major toxicities for all treatments were nausea and vomiting (62 to 88 per cent) and accompanying anorexia (72 to 95 per cent). Azotemia developed in 45 per cent of the patients receiving combination therapy. In addition an elevation in serum creatinine occurred in 22 per cent of the patients receiving combination therapy and in 17 per cent of those receiving cis-platinum alone. Myelosuppression occurred infrequently.

Aged↗

Renal revascularization in patients with total occlusion of the renal artery.

A total of 15 patients with renovascular hypertension underwent revascularization of totally occluded renal arteries. Preoperative clinical assessments suggesting renal salvageability include urographic visualization of the involved kidney, renal length greater than 9 cm., filling of the distal renal arterial tree on angiography and elevated renal vein renin levels. Postoperatively, hypertension was cured or improved in 14 patients and there was 1 failure. Over-all renal function improved in 9 of 10 patients with preoperative azotemia and stabilized in the remaining 5 patients. Revascularization can be done successfully in properly selected patients with complete renal artery occlusion, with control of hypertension and reversal of renal failure.

Adult↗

Cecocystoplasty for bladder augmentation.

Augmentation of the bladder by cecocystoplasty has been studied in 37 patients during a 15-year period. The indications for an operation were a small contracted bladder secondary to tuberculosis in 31 cases, interstitial cystitis in 5 and carcinoma of the bladder in 1. Results were studied by the assessment of symptomatic improvement, the effect on renal function, urinary tract infection and the postoperative complications. Urodynamic studies also were done on 23 patients with tuberculosis of the renal tract and the results are discussed. Azotemia was not a contraindication to an operation but patients with enuresis or those who have evidence of psychiatric disturbance should be assessed carefully.

Adolescent↗

Atherosclerotic renal artery occlusion extending into branches: successful revascularization in situ with a branched saphenous vein graft.

In some patients successful renal revascularization can be done after complete renal artery occlusion. We report on a patient with atherosclerotic occlusion of the renal artery and its branches in whom an aortorenal bypass with a branched saphenous vein graft was performed in situ, with cure of hypertension and reversal of azotemia. This is a useful and versatile technique for replacing the renal artery and its major branches.

Arteriosclerosis↗