Notes: Abstract  Dermatomyositis is an uncommon inflammatory myopathy with characteristic cutaneous manifestations which is frequently linked to several cancers. A 42-year-old man presented with left flank pain with typical symptoms of dermatomyositis. Computed tomography showed a solid mass in the pelvis of the left kidney and lymphadenopathy in the retroperitoneum. Since the general condition of the patient rapidly deteriorated because of marked muscle weakness of the respiratory muscles, the patient initially underwent medical treatment with prednisolone. After the improvement of respiratory function, the patient underwent radical nephroureterectomy and retroperitoneal lymphadenectomy. The pathological specimen showed moderately differentiated adenocarcinoma, stage pT3N2. After surgery, the patient showed a marked improvement of clinical symptoms related to dermatomyositis. Twenty-two months later, multiple organ metastases occurred and the patient died of cancer. We should point out the significance of surgical and medical treatment for these patients.

Notes: Abstract Objectives: The present study was carried out to evaluate the accuracy of helical computed tomography (CT) and intravenous digital subtraction angiography (IV-DSA) on anatomical assessment of renal vasculature for living renal donors.Methods: Forty-two healthy potential renal donors were prospectively evaluated and 35 subsequently underwent donor nephrectomy after helical CT and IV-DSA evaluation. The vascular and non-vascular findings were compared between the findings on helical CT, IV-DSA and surgery.Results: Ten prehilar branches and five accessory renal arteries were found at nephrectomy. Overall, operative findings agreed with the findings by IV-DSA in 89% and by helical CT in 83%. In delineating accessory arteries, IV-DSA had a sensitivity of 60% and specificity of 97%, whereas helical CT had a sensitivity of 40% and specificity of 100%. In delineating prehilar branches, IV-DSA had a sensitivity of 90% and specificity of 100%, whereas helical CT had a sensitivity of 70% and specificity of 100%. Accessory arteries and prehilar branches that were not detected by helical CT or IV-DSA, were less than 2 mm in diameter and did not require vascular reconstruction. Renal veins were delineated in 63% by IV-DSA, whereas they were clearly imaged by helical CT in all cases, including a case with a circumaortic renal vein. Non-vascular findings were obtained in 64% by helical CT, including two renal tumors. None of these findings were obtained by IV-DSA.Conclusion: Helical CT and IV-DSA provide comparably sufficient information on renal artery vasculature. However, helical CT provides significantly more information on venous and non-vascular findings as a single-imaging modality.

Notes: Background: Renal denervation and re-innervation may affect vascular responses to sympathomimetics, which may play a role in the maintainance of renal blood flow (RBF) following renal transplantation. The purpose of the present study was to elucidate pharmacological alteration of the renal sympathetic response following kidney transplantation. Using the rat kidney transplantation model, we focused on responses to various sympathomimetics in order to investigate the effects of sympathetic denervation and functional re-modulation mechanisms in the transplanted kidney.Methods: Male isogenic rats underwent unilateral kidney denervation or kidney transplantation accompanied by unilateral native nephrectomy. Renal blood flow was evaluated using a laser Doppler flow meter following bolus injection of 50 μg/kg dopamine and continuous infusion of 60 μg/min phenylephrine (PE) at 0, 30, 60, 90 and 240 days after transplantation as well as at 30 days after denervation.Results: Bolus injection of dopamine (50 μg/kg) induced an initial reduction in RBF (that of α-adrenoceptors) followed by a long-lasting increase in RBF (that of β-adrenoceptors) in the native kidney. In grafted or denervated kidneys, the dopamine-induced decrease in RBF mediated by α-adrenoceptors was markedly enhanced, while the increase in RBF mediated by an action of dopamine on β-adrenoceptors was blunted. The effects of the post-transplant period on vascular responses to dopamine were significant, but not completely synchronized with the native kidney, even at 240 days. Continuous infusion of PE (60 μg/min) produced a gradual increase in RBF in the native kidney, whereas a sudden reduction (i.e. breakthrough in autoregulation) was observed in the denervated and transplanted kidney.Conclusions: The responses to sympathomimetics in the grafted kidney were shifted towards flow reduction (i.e. enhanced vasoconstriction via stimulation of α-adrenoceptors and blunted vasodilatation via stimulation of β-adrenoceptors. This functional impairment was recovered in a time-dependent manner. In addition, the hemodynamic autoregulation system in the kidney deteriorated following transplantation or denervation.

Notes: Background To assess the feasibility of urodynamic study under general anesthesia (GA) we performed electromyography of the external urethral sphincter (EUS-EMG) on 73 children and cystometry (CM) alone on 10 children. Methods Subjects were divided into 3 groups. Those in groups I and II were suspected of having voiding dysfunction with (group I) or without (group II) overt neurospinal defects, while those in group III were thought to be functionally normal. EUS-EMG was performed under light anesthesia following cystourethroscopy to examine structural abnormalities. Atropine sulfate premedication was not used for the anesthetic procedure; muscle relaxants were used only for tracheal intubation. Results Voiding was observed in 83% of the patients. Among patients who voided, detrusor-external sphincter dyssynergia (DSD) was noted in 7 (38%) group I patients and 6 (19%) group II patients; in group III, voiding was synergic in all patients. In 10 cases, CM alone was carried out both under anesthesia and in the waking state; anesthesia suppressed detrusor hyperreflexia (DH) in all 9 patients but produced no change in bladder compliance. Conclusions In children with urinary disorders, urodynamic study under GA following cystourethroscopy is a feasible method for assessing EUS function and documenting DSD; DH is not evaluable, however. Stratifying urinary management on the basis of these examinations resulted in satisfactory clinical outcomes.

Notes: Abstract Downsizing and refinement of the pediatric endoscope in video-monitoring systems have facilitated genitourinary endoscopy even in small children without any traumatic instrumentation. Indications for endoscopy in children with hematuria or tractable urinary tract infection have been tailored for the rareness of genitourinary malignancy or secondary vesicoureteral reflux (VUR) as a result of infravesical obstruction. Most mechanical outlet obstructions can be relieved endoscopically irrespective of sex and age. Endoscopic decompression by puncture or incision of both intravesical and ectopic ureteroceles can be an initial treatment similar to open surgery for an affected upper moiety. Endoscopy is necessary following urodynamic study to exclude minor infravesical obstruction only in children with unexplained dysfunctional voiding. Genitourinary endoscopy is helpful for structural abnormalities before and at the time of repairing congenital urogenital anomalies. Endoscopic injection therapy of VUR has been established as a less invasive surgical treatment. Pediatric endoscopy will play a greater role in the armamentarium for most pediatric urological diseases through the analysis of visual data and discussion on the indications for endoscopy throughout the world.

Notes: Abstract Background: Renal transplantation is a definitive therapeutic modality in end-stage renal disease (ESRD). Most ESRD patients in Japan experience dialysis prior to renal transplantation. The present study was undertaken to examine the usefulness of pre-emptive renal transplantation (PET).Methods: Between 1987 and 1998, 255 renal transplantations were carried out by the authors. Among those consecutive cases, 10 were cases of PET. In nine pediatric cases, demographics, graft and patient survival, height growth and benefits from successful transplantation were studied and compared with age-matched dialyzed transplantation controls.Results: All transplantation was living-related. There was a disparity of causes of ESRD between the two groups. In PET, acquired renal deterioration due to a congenital lower urinary tract disorder was the major cause. Graft and patient prognosis was favorable in both groups. Growth retardation in PET patients under 15 years of age was significantly less apparent at the time of transplantation and after 3 years compared to the control. The benefits from transplantation were different in the two groups. Most PET patients felt an improvement of their physical condition; however, all of the control patients felt that the major boon was the freedom from the restriction of the daily diet and time for dialysis.Conclusion: In pediatric renal transplantation, short-term preceding dialysis does not have a detrimental effect, but PET could benefit ESRD patients by maintaining their quality of life. Moreover, PET minimizes the production of renal dwarfism in prepubertal children. Thus, PET should be taken into consideration in the choice of renal replacement therapy.

Notes: A patient with ectopic pheochromocytoma that developed in the spermatic cord about 5 years after successive resections of bilateral carotid body tumors and bilateral adrenal pheochromocytomas is reported. This is thought to be the first case of pheochromocytoma of the spermatic cord in a setting of multiplicity.

Notes: Background: Five renal recipients with neurovesical dysfunction (NVD) were retrospectively reviewed focusing on anatomical and urodynamic abnormalities of the lower urinary tract and their management prior to kidney transplantation.Methods: The underlying anomalies in these 5 patients were a posterior urethral valve (1 with an imperforate anus; n = 2), meningomyelocele (n = 2) and a congenital short urethra with an imperforate anus (n = 1). Their urinary tracts were evaluated prior to transplantation with voiding cystourethrography, urethrocystoscopy, cystometrography and electromyography of the external urethral sphincter to identify a possible focus of urinary tract infection, urine storage and voiding function.Results: All 5 patients had NVD proven by urodynamic studies or by documentation of urinary retention in the absence of mechanical outlet obstruction. Bilateral high grade vesicoureteral reflux was noted in all patients, requiring ureteroneocystostomy. Clean intermittent catheterization (CIC) was ultimately employed for bladder emptying in all patients. Two patients with poor bladder compliance underwent augmentation cystoplasty before transplantation. The Mitrofanoff procedure was used in 2 patients with structural urethral abnormalities to access the bladder for catheterization. After eradication of possible sources of infection and establishment of a low-pressure urine storage system with bladder emptying by CIC, kidney transplantation was performed. Following kidney transplantation, all of the recipients were asymptomatic for urinary tract infections using CIC. Although 1 patient lost his graft due to chronic rejection, the other 4 other patients have good renal function.Conclusion: Kidney transplantation in patients with NVD can be performed provided that their urinary tract problems are properly resolved.

Notes: From January 1989 to December 1995, 5 diabetic patients with end-stage renal disease (1 woman, 4 men) underwent kidney-alone transplantation. The mean age of the recipients at the time of transplantation was 37.4 years (range, 32 to 43). Graft function and glucose tolerance was evaluated for 5 to 72 months after surgery. Postoperative complications were seen in 2 patients; nonspecific subcutaneous infections and an asymptomatic partial allograft infarction. All renal allografts were functioning 1 year after transplantation, with a mean serum creatinine level of 1.10mg/dL (range, 0.8 to 1.8mg/dL), and a mean urinary protein level of 1 7.8mg/dL (range, 5 to 27mg/dL). The postoperative daily dose of insulin was higher than the preoperative dose, while the level of glycated hemoglobin (HbA1c) increased after surgery and peaked 6 months after transplantation; 1 year after transplantation it had reverted to the preoperative level. As long as the diabetic complications of the renal allograft recipients are not severe, the short-term survival and the renal function of diabetic patients with end-stage renal disease improves after kidney-alone transplantation, which is still the standard method of treatment in Japan.

Notes: Patients with aggressive angiomyxoma may experience local recurrences. We report a case of recurrent aggressive angiomyxoma medically treated successfully with a gonadotropin-releasing hormone agonist. A 34-year-old woman with a huge perineal tumor underwent an extensive resection of the abdominoperineal tumor combined with total pelvic exenteration. Histology showed aggressive angiomyxoma and the tumor cells were immunoreactive for estrogen and progesterone receptors. Although the patient had experienced no local recurrence for 12 months under adjuvant therapy with a gonadotropin-releasing hormone agonist, a recurrence occurred 3 months after the completion of adjuvant therapy. The patient underwent medical treatment with a gonadotropin-releasing hormone agonist and had a complete resolution of the recurrent tumor again. Hormonal treatment with a gonadotropin-releasing hormone agonist can be applied for small primary aggressive angiomyxomas in addition to adjuvant therapy for residual tumors.