Is endoscopic decompression of the neonatal extravesical upper pole ureterocele necessary for prevention of urinary tract infections or bladder neck obstruction?

PURPOSE: It has been hypothesized that endoscopic decompression of the duplex extravesical ureterocele is necessary to prevent the complications of urinary tract infections and progressive hydronephrosis. This study was performed to test this premise. MATERIALS AND METHODS: Infants younger than 2 weeks with an extravesical ureterocele associated with a duplex upper pole moiety were assigned to immediate endoscopic puncture of the ureterocele followed by antibiotic prophylaxis or antibiotic prophylaxis with plans for delayed surgical intervention. Radiographic studies and catheterized urine cultures were obtained at ages 3 and 6 months and for fever greater than 38.5C. All patients included in this study were followed through 6 months of life. RESULTS: Of the patients 32 underwent endoscopic puncture of the ureterocele. Median patient age at endoscopy was 5 days (range 3 to 13). During the first 6 months of life complications developed in 4 (12%), including febrile urinary tract infections in 3 (9%) and with progressive hydronephrosis due to incomplete puncture of the ureterocele in 1 (3%). The remaining 40 patients were treated with antibiotic prophylaxis and delayed open surgery. Median time to open surgery was 3 months (range 2 to 6). During the first 6 months of life complications developed in 5 (13%), including 3 (8%) febrile urinary tract infections and progressive hydronephrosis in 2 (5%). No statistical difference was noted between the 2 treatment groups. CONCLUSIONS: In patients with extravesical duplex ureteroceles neonatal complications of urinary tract infection and progressive hydronephrosis are not significantly different between those treated with immediate endoscopic decompression versus delayed open surgical intervention.

Felbamate urolithiasis.

PURPOSE: To report a case of felbamate (FBM) urolithiasis. METHODS: Urographic imaging [sonography, abdominal computed tomography (CT), intravenous pyelogram, voiding cystourethrogram] and urologic procedures (cystoscopy with lithotripsy, ureteral stent) to define and capture the stones. Stone identification was by infrared spectroscopy and gas chromatography/mass spectrometry. RESULTS: A 15-year-old boy had painful hematuria, bilateral ureteral obstruction, and urinary retention. Kidney, bladder, and ureteral stones were found, and ureteral stent placement was required to relieve obstruction. The stone material was identified as FBM by chemical analysis. Stone formation ceased with discontinuation of FBM. CONCLUSIONS: FBM urolithiasis can occur, and possible contributory factors include high felbamate dosage, drug polypharmacy, and risk factors for forming stones of other types. FBM urolithiasis may be heralded by crystalluria.

The catalytic activities of the bifunctional Azotobacter vinelandii mannuronan C-5-epimerase and alginate lyase AlgE7 probably originate from the same active site in the enzyme.

The Azotobacter vinelandii genome encodes a family of seven secreted Ca(2+)-dependent epimerases (AlgE1--7) catalyzing the polymer level epimerization of beta-D-mannuronic acid (M) to alpha-L-guluronic acid (G) in the commercially important polysaccharide alginate. AlgE1--7 are composed of two types of protein modules, A and R, and the A-modules have previously been found to be sufficient for epimerization. AlgE7 is both an epimerase and an alginase, and here we show that the lyase activity is Ca(2+)-dependent and also responds similarly to the epimerases in the presence of other divalent cations. The AlgE7 lyase degraded M-rich alginates and a relatively G-rich alginate from the brown algae Macrocystis pyrifera most effectively, producing oligomers of 4 (mannuronan) to 7 units. The sequences cleaved were mainly G/MM and/or G/GM. Since G-moieties dominated at the reducing ends even when mannuronan was used as substrate, the AlgE7 epimerase probably stimulates the lyase pathway, indicating a complex interplay between the two activities. A truncated form of AlgE1 (AlgE1-1) was converted to a combined epimerase and lyase by replacing the 5'-798 base pairs in the algE1-1 gene with the corresponding A-module-encoding DNA sequence from algE7. Furthermore, substitution of an aspartic acid residue at position 152 with glycine in AlgE7A eliminated almost all of both the lyase and epimerase activities. Epimerization and lyase activity are believed to be mechanistically related, and the results reported here strongly support this hypothesis by suggesting that the same enzymatic site can catalyze both reactions.

A multi-institutional analysis of laparoscopic orchidopexy.

OBJECTIVE: To combine and analyse the results from centres with a large experience of laparoscopy for the impalpable testis with small series, to determine the expected success rate for laparoscopic orchidopexy. METHODS: A questionnaire was distributed to participating paediatric urologists; each contributor retrospectively reviewed the clinical charts for their cases of therapeutic laparoscopy for an impalpable testis, detailing 36 variables for each patient. The data were collated centrally into a computerized database. For inclusion, the testis was intra-abdominal (including 'peeping' at the internal ring) at laparoscopic examination, was not managed through an open approach and did not undergo orchidectomy. Three surgical groups were assessed, with success defined as lack of atrophy and intrascrotal position: group 1, primary laparoscopic orchidopexy; group 2, a one-stage Fowler-Stephens (F-S) orchidopexy; and group 3, a two-stage F-S orchidopexy. RESULTS: Data were gathered from 10 centres in the USA, covering the period 1990-1999; 252 patients representing 310 testes were included and overall, 15.2% were lost to follow-up. There was no significant difference between success rates in the larger and smaller series. Atrophy occurred in 2.2% of 178 testes, 22.2% of 27 testes and 10.3% of 58 testes in groups 1-3, respectively. Testes were not in a satisfactory scrotal position in 0.6%, 7.4% and 1.7% of groups 1-3, respectively. The mean follow-up for each group was 7.7, 8.6 and 20.0 months, respectively. The overall success for all groups was 92.8% (97.2% group 1; 74.1% group 2; 87.9% group 3), with an atrophy rate of 6.1%. CONCLUSION: Laparoscopic orchidopexy for the intra-abdominal testis, in both large and small series, can be expected to have a success rate higher than that historically ascribed to open orchidopexy. Within this series, single-stage F-S laparoscopic orchidopexy resulted in a significantly higher atrophy rate than the two-stage repair. However, when considering both F-S approaches, the laparoscopic approach gave greater success than previously reported for the same open approaches. Despite the weaknesses inherent in a retrospective unrandomized study, we conclude that laparoscopic orchidopexy is, if not the procedure of choice, an acceptable and successful approach to the impalpable undescended testicle.

Acute renal failure due to obstruction in Burkitt lymphoma.

Acute renal failure in Burkitt lymphoma is commonly the result of tumor lysis syndrome. We present a 15-year-old boy who developed hypertension, seizures, and acute renal failure due to extrinsic compression of the bladder and ureters by a large retrovesical Burkitt lymphoma. The causes of acute renal failure in Burkitt lymphoma and the incidence of acute urinary obstruction in this disease are reviewed.

Laparoscopic creation of a catheterizable cutaneous ureterovesicostomy.

Nephrectomy and creation of a cutaneous ureterovesicostomy for intermittent catheterization of the bladder traditionally requires two surgical procedures performed through separate incisions. Herein we report completion of these procedures using a transperitoneal laparoscopic approach, with the ureterovesicostomy stoma created at one of the laparoscopic working ports. The clinical course was remarkable for a shortened postoperative hospitalization (48 hours) with minimal incisional pain, and an excellent long-term result with complete bladder emptying and resolution of urinary infections. Laparoscopic application of the Mitrofanoff principle for creation of a catheterizable cutaneous ureterovesicostomy combines the advantages of both, allowing optimal preservation of ureteral vascularity, minimal morbidity, and efficient bladder evacuation.

Preservation of continence after posterior sagittal surgery.

PURPOSE: An animal study was performed to evaluate the effect of posterior sagittal pararectal mobilization on anorectal sphincter function. MATERIALS AND METHODS: We initially divided 11 juvenile pigs into 3 groups: group 1-anesthesia alone (3), group 2-posterior sagittal incision alone (4) and group 3-posterior sagittal incision with unilateral pararectal dissection (4). Two animals in group 1 subsequently underwent posterior sagittal incision with circumferential pararectal dissection (group 4). The anal canal was preserved intact in all animals. Anorectal sphincter manometry was performed preoperatively, and 2, 4, 8 and 12 weeks postoperatively. Electromyography was performed 12 weeks postoperatively. Anorectal sphincter muscle complexes were harvested for histological examination. RESULTS: All animals had postoperative bowel continence. Postoperatively manometry revealed no difference from preoperative measurements in all study groups (p = 0.90). Electromyography and histological examination of the anorectal sphincters were normal in all but 2 animals. Denervation injury and histological atrophy were detected after repair of inadvertent enterotomy in 1 animal following unilateral pararectal dissection, and polyphasic motor unit potentials implying reinnervation were detected in another after circumferential pararectal mobilization. CONCLUSIONS: These results indicate that posterior sagittal incision and unilateral pararectal mobilization cause no permanent injury to the anorectal sphincter. However circumferential pararectal dissection or repair of a rectal injury may cause measurable changes in sphincter function.

Extrarenal Wilms tumor occurring in the inguinal canal.

We report an unusual case of extrarenal Wilms tumor discovered incidentally during routine inguinal orchiopexy. The world literature and embryological implications of Wilms tumor in the inguinal canal are reviewed.

Role of superoxide dismutase in the pathogenesis of pyelonephritis: immunological localization of superoxide dismutase in human renal tissues.

The enzyme superoxide dismutase affords a protective effect from renal scarring secondary to acute pyelonephritis in primates. To investigate the relationship between renal superoxide dismutase content and age we selected formalin-fixed normal human renal tissue from subjects of varying age, ranging from premature infant to adult, for immunostaining with human anti-superoxide dismutase antibody using the peroxidase-antiperoxidase technique. Sections that demonstrated acute pyelonephritis were immunostained for comparison. Immunostaining for superoxide dismutase was detected consistently in the proximal tubular cell cytoplasm in all specimens regardless of subject age. Superoxide dismutase was not detected in other segments of the nephron. In kidneys that demonstrated acute pyelonephritis we detected enhanced immunostaining in the proximal tubules, as well as increased background staining related to the inflammatory cells present. These results in conjunction with recent demonstrations of proximal tubular cell endocytosis of bacteria suggest that superoxide dismutase has an important role in mediating the initial events of pyelonephritis within the proximal tubular cell.

Autosomal dominant polycystic kidney disease in infants: asymmetric disease mimicking a unilateral renal mass.

Unilateral radiographic presentation of autosomal dominant polycystic kidney disease is detected only rarely in infants and may suggest the presence of a complex cystic mass. We report such a case and review the literature concerning unilateral presentation of autosomal dominant polycystic kidney disease in infants and children.

Evaluation of the stable fly (Stomoxys calcitrans) as a vector of enzootic bovine leukosis.

Experiments reported here were directed at 2 questions: (1) Can the stable fly (Stomoxys calcitrans) transmit enzootic bovine leukosis? (2) Could early viremia augment the probability of transmission by this insect? In one vector experiment, calves and bovine leukemia virus (BLV)-infected cows were housed with and without stable flies. The calves were monitored serologically during a 3-month postexposure period, using the agar gel immunodiffusion test. All fly-infested and fly-free calves remained BLV-seronegative. For a second vector experiment, donor calves, newly injected with blood from BLV-infected cows with high virus expression, were tethered alternately between uninoculated, weaned BLV-seronegative calves. These groups were housed with or without flies in 2 replicate trials. The inoculated calves from the first replicate seroconverted at 16 and 23 days after inoculation; the inoculated calves from the second replicate seroconverted at 11, 16, 16, and 37 days after inoculation. All uninoculated calves remained BLV-seronegative. In a manual transmission experiment, 50 unfed stable flies were allowed to complete a meal on each of 3 BLV-seronegative calves after feeding on a BLV-seropositive cow with high (42%) virus expression. One control calf was injected with blood from the cow. Seroconversion occurred in the control calf and 1 calf on which flies were given access. A scanning electron microscopic study was made of the everted and closed mouth parts of the stable fly. Given the lymphocyte count in blood from the cow used in the manual vector transmission experiment, it was calculated that 3,950 mouth part volumes would be necessary to transmit BLV.(ABSTRACT TRUNCATED AT 250 WORDS)

Nephrogenic adenoma occurring in an ileal conduit.

We report a case in which papillary lesions developed in an ileal conduit that had been constructed for management of nonmalignant disease. Pathological diagnosis was nephrogenic adenoma, an entity previously thought to occur only in the urothelium. The pathogenesis of this unusual tumor is discussed.

Chromosome studies in 17 patients with the Sézary syndrome.

Chromosome studies were done on phytohemagglutinin-stimulated peripheral blood from 17 patients with Sézary syndrome. A chromosomally abnormal clone was found in five patients: each patient had an abnormal chromosome 6 and four had an abnormal chromosome 1. Six patients without abnormal clones had more than 20% metaphases with random heteroploidy and sporadic structural anomalies. Only normal metaphases were seen in four patients, and no metaphases were found in two. Four of the five patients with an abnormal clone died, and their median survival from chromosome analysis was 6 months; only one of these patients died of lymphoma. The six patients with increased heteroploidy had long survivals and no apparent malignant process. Two of the four patients with normal metaphases died of malignant disease: one had lymphoma and the other squamous cell carcinoma. A third patient with normal chromosomes died of extensive visceral cutaneous T-cell lymphoma.

Anaplastic plasma cell myeloma and immunoblastic lymphoma. Clinical, pathologic, and immunologic comparison.

To test whether highly anaplastic myeloma and immunoblastic lymphoma are truly identical disease processes, simultaneous series were compared in respect to cytomorphologic features, immunoglobulin content or secretion, clinical and laboratory findings, and patient survival. Although the series partially overlapped in each studied feature, different trends served to distinguish them. Of the 14 patients with myeloma, all were dead at two years, whereas six of the 22 patients with lymphoma were disease-free at 35 to 78 months. Only 50 percent of patients with myeloma received intensive chemotherapy, whereas all 19 patients with stage III or IV lymphoma received such therapy. Myelomas secreted predominantly IgA heavy chain rather than IgG and lambda light chain rather than kappa. Lymphomas contained predominantly IgM rather than IgG and kappa rather than lambda. There were no IgM myelomas and no IgA lymphomas. The shorter survival of patients with the extremely anaplastic form of myeloma, as compared with patients who had immunoblastic lymphoma, may relate, in part, to prior therapy for previous lower grade myeloma; however, intrinsic differences in the nature of these two disease processes are reflected in their disparate immunologic characteristics.