Search

Renal pelvis dilatation (RPD) is diagnosed in utero on prenatal ultrasonography (US) and can resolve spontaneously. However, isolated RPD can also reflect ureteropelvic junction obstruction (UPJO), which requires surgical treatment to prevent progressive renal deterioration. The diagnosis of UPJO can only be confirmed after birth with repeat US and renal isotope studies. 1 H Nuclear Magnetic Resonance spectroscopy (NMR) was performed on urine of newborns with prenatally diagnosed unilateral RPD and healthy controls to identify specific urinary biomarkers for UPJO. The original combination of EigenMS normalization and sparse partial-least-squares discriminant analysis improved selectivity and sensitivity. In total, 140 urine samples from newborns were processed and 100 metabolites were identified. Correlation network identified discriminant metabolites in lower concentrations in UPJO patients. Two main metabolic pathways appeared to be impaired in patients with UPJO i.e. amino acid and betaine metabolism. In this prospective study, metabolic profiling of urine samples by NMR clearly distinguishes patients who required surgery for UPJO from patients with transient dilatations and controls. This study will pave the way for the use of metabolomics for the diagnosis of prenatal hydronephrosis in clinical routine., (© 2022. The Author(s).)

Perirenal urine extravasation, also known as urinoma, occurs usually after renal trauma. Spontaneous urinoma is a very rare pathology in daily urology practice. The patients with spontaneous urinoma usually present with flank pain, and radiologic imagings confirm the diagnosis. Ultrasonography, intravenous urography, and computed tomography are the radiologic modalites for the diagnosis. We report a case of spontaneous urinoma in a patient with renal pelvic tumor.

Objective: To explore the prognosis and risk factors of pyelectasis in high-risk infants. Methods: This was a retrospective study. Totally 960 high-risk infants, who accepted type B ultrasonic examination for fetus at 28th week of gestation and for newborns in 48 hours after birth, were included in the study in departments of obstetrics and eonatology, Shunyi Maternal and Children's Hospital of Beijing Children's Hospital during May 2012 to April 2013. The degree of pyelectasis was classified using Grignon grade and the paients were followed up for 3 years. The factors of epidemiology, high risk pregnant women, fetus and high-risk newborns that relate to pyelectasis were summarized. High-risk factors were analyzed by using logistic multivariate regression analysis. Results: Of 960 high-risk infants, 103 had abnormal urinary ultrasound results, 87 (9.1% of high-risk infants) were diagnosed with pyelectasis, 16 (1.7% of high-risk infants) were diagnosed with congenital anomalies of the kidney and urinary tract. According to the degree of pyelectasis, 68 infants were Grignon grade Ⅰ, male:female ratio=5.8∶1, left side:right side ratio=1.91∶1; 19 infants were Grignon grade Ⅱ, male:female ratio=5.33∶1, left side:right side ratio=2.12∶1. Postnatal follow-up results showed that pyelectasis disappeared in 48 cases (55% of pyelectasis cae), 40 infants were Grignon grade Ⅰ (59% of all Grignon grade Ⅰ patients), 8 infants were Grignon grade Ⅱ (42% of all Grignon grade Ⅱ patients); The result of risk factors analysis showed that the risk of pyelectasis in males was 4.368 times that of females (95% CI: 2.33-8.189, P< 0.05); the risk of pyelectasis in low birth weight infants was 22.434 times that of non low birth weight infants (95% CI : 5.883-85.547, P< 0.05). Conclusion: The incidence of pyelectasis in high-risk infants was 9.1%. The mitigation rate of pyelectasis in Grignon grade Ⅰ to Ⅱ in fetal or newborn period is high. Patients in Grignon grade Ⅲ and above in fetal or new born period had high risk of congenital anomalies of the kidney and urinary tract. The risk of pyelectasis of male was higher than that of female; the risk of pyelectasis of low birth weight infant was higher than appropriate for gestational age infants.

Background: IgG4-related disease is a recently recognized systemic disease characterized by storiform fibrosis with infiltration of IgG4-positive plasma cells. In rare incidences, IgG4-related renal disease can present as a solitary mass lesion at renal pelvis and can pose a diagnostic challenge since these lesions mimic malignancy. Herein, we present a rare case of IgG4-related disease presenting as inflammatory pseudotumor lesion, involving the renal pelvis and also neighboring renal parenchyma., Case Presentation: A 75-year-old man with no history of IgG4-related disease underwent computed tomography (CT) scan for evaluation of prostatic cancer. The CT scan incidentally revealed a mass lesion located at the right renal pelvis. Radiologic findings were highly suggestive of malignancy. Therefore, the patient underwent right nephroureterectomy. Microscopically, the mass lesion showed storiform fibrosis with diffuse and intense inflammatory cell infiltration. Infiltrating cells were mainly histiocytes and plasma cells. Tubulointerstitium adjacent to the lesion also showed fibrosis with abundant plasmacytic infiltration. Immunohistochemical staining revealed the presence of IgG4-positive plasma cells in both the mass lesion and tubulointerstitium (mean of 94/HPF per field)., Conclusion: Considering these findings, we diagnosed the mass lesion as IgG4-related inflammatory pseudotumor of the renal pelvis. In patients with renal pelvic masses, IgG4-related inflammatory pesudotumor should be considered in the differential diagnosis to avoid unnecessary surgical intervention.

Objective: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare syndrome characterized by mullerian and renal anomalies. Renal agenesis is classically considered to be part of the definition of OHVIRA syndrome. There are increasing reports that the lack of an ipsilateral kidney on imaging studies is not from true renal agenesis but renal dysplasia and atrophy. We have observed cases where this ipsilateral dysplastic and atrophic kidney leads to clinical symptoms. The objective of this study is to report cases of OHVIRA syndrome where an ipsilateral dysplastic and atrophic kidney led to clinical symptoms, and to raise awareness in the pediatric urology community of this variant of OHVIRA syndrome., Material and Methods: All cases of OHVIRA syndrome with an ipsilateral dysplastic and atrophic kidney were identified from January 2010 to June 2013. The patient's presentation, clinical course, surgical management, radiologic findings, and pathologic finding were reviewed., Results: There were three cases of OHVIRA syndrome with a symptomatic ipsilateral dysplastic and atrophic kidney identified. All three of these dysplastic and atrophic kidneys had an ectopic ureter to the obstructed hemivagina and led to persistent vaginal drainage after resection of the vaginal septum. These dysplastic and atrophic kidneys were not visualized on any imaging studies. Laparoscopic removal of the dysplastic and atrophic kidney led to cessation of vaginal drainage in all cases., Conclusion: This study reports additional cases to the literature that do not fit the classic definition of OHVIRA syndrome. In the cases in this study, there was no ipsilateral renal agenesis, but an ipsilateral dysplastic and atrophic kidney with an ectopic ureter to the obstructed hemivagina. In addition, these cases demonstrate that the dysplastic and atrophic kidney may cause symptoms such as persistent vaginal drainage after resection of vaginal septum. The dysplastic and atrophic kidneys may not be detected by any imaging modality including MRI and DMSA scan and may be ectopic in location. All three of our cases had persistent vaginal drainage after resection of the vaginal septum which was cured by removal of the dysplastic and atrophic kidney ipsilateral to the obstructed hemivagina. The limitations of this study include the small number of patients and retrospective nature. While the classic definition of OHVIRA syndrome includes ipsilateral renal agenesis, there is increasing evidence that the absence of a kidney on imaging is due to renal dysplasia and atrophy and not true renal agenesis. In addition, we have observed that this dysplastic and atrophic kidney may cause clinically significant issues such as persistent vaginal drainage through an ectopic ureter to the ipsilateral hemivagina after resection of vaginal septum. This small dysplastic and atrophic kidney may not be visualized on imaging studies and laparoscopy can be diagnostic and therapeutic if indicated. Ipsilateral renal anomalies, not ipsilateral renal agenesis, should be considered part of the definition of OHVIRA syndrome. Pediatric urologists need to be aware of the spectrum of renal anomalies in OHVIRA syndrome., (Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

We present an impressive case of a pregnant woman presenting with severe right flank pains. A rare rupture of the right renal pelvis turned out to be the cause of her problems. Renal pelvis rupture in pregnancy is difficult to diagnose. It is easily confused with other more prevalent diagnoses, like hydronephrosis, ureteral distension, renal calculus and pyelonephritis. Ultrasonography is not always conclusive. Pyelography seems to be a good diagnostic tool, this case shows that MRI is another option. Renal pelvis rupture can be effectively treated with the insertion of a JJ-stent., (2015 BMJ Publishing Group Ltd.)

A 52-year-old Japanese woman was admitted to our hospital with the renal pelvic mass lesion detected on a health screening examination. The surgical specimen contained a mass exhibiting the histological features of immunoglobulin (Ig)G4-related disease, including lymphoplasmacytic infiltration and sclerosis with numerous IgG4-producing plasma cells. Postoperatively, an elevation of the serum IgG4 level was confirmed at 403 mg/dL; however, there was no evidence of tubulointerstitial nephritis or glomerulopathy, including membranous nephropathy, and the urothelium of the renal pelvis was intact without inflammation. We herein report this case in which IgG4-related disease of the renal pelvic region presented with a mass lesion in the intrarenal sinus near the renal pelvis, not 'pyelitis' (as described by Stone).

Pure adenocarcinomas of the urothelium are very rare and their location in the pelvis is uncommon. Although their pathogenesis is not well defined, adenocarcinomas are likely to originate from neoplastic transformation of the glandular cells of the urothelial intestinal metaplasia usually arising in response to chronic irritating stimuli, such as long-duration inflammation, urolithiasis, and hydronephrosis. We report a case of an 81-year-old woman who underwent right nephrectomy for relapsing renal abscess due to a staghorn calculus. Histological examination disclosed an infiltrating adenocarcinoma arising from a tubulovillous adenoma with the surrounding pelvic mucosa showing a sequence of intestinal metaplasia, low- and high-grade villous adenoma, and invasive adenocarcinoma, supporting the hypothesis of cancer progression due to chronic inflammation from the urothelium through the metaplasia step.

The extrarenal calyx (ERC) is a rare congenital anomaly, associated with various other abnormalities of the urogenital system. We report a unique case of ERC in a solitary functioning kidney with a massively dilated pelvis that developed spontaneous rupture. A dismembered pyeloplasty was performed at the time of rupture. The patient did well post-operatively with a stable creatinine and stable SFU grade 2 hydronephrosis., (Copyright © 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: To analyze the factors indicating renal injury in patients with bilateral ureteropelvic-junction obstruction (UPJO)., Methods: We investigated 26 patients, 35 kidney units (KUs), who were diagnosed with bilateral UPJO and underwent unilateral or bilateral pyeloplasty between January 2000 and December 2011. During pyeloplasty, ipsilateral kidney biopsy was performed. Kidney biopsy results were divided into 3 categories. Hydronephrosis was analyzed using both the Society of Fetal Urology (SFU) and Onen's grading system. We investigated factors related with the renal histopathologic results of 29 KUs. We additionally analyzed whether there were factors predicting contralateral pyeloplasty after initial unilateral pyeloplasty., Results: Ten KUs (34.5%) showed grade 1 histopathologic change, whereas 10 KUs (34.5%) and 9 KUs (31.0%) showed grade 2 and 3 change, respectively. Preoperative differential renal function (DRF) and serum creatinine levels were not different among groups (P = .697 and .516, respectively). Hydronephrosis grade was not different when graded by SFU system (P = .100) while significantly different by Onen's system (P <.001). Among 23 patients with initial unilateral pyeloplasty, additional contralateral pyeloplasty was required in 6 patients (26.1%). There were no significant preoperative factors predicting the need for additional operations., Conclusion: In bilateral UPJO, DRF, and SFU grade of hydronephrosis did not correctly reflect renal injury. Onen's hydronephrosis grade showed a significant relationship with renal histopathologic grade and could be an indicator for renal injury in bilateral UPJO., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Crossed fused ectopia of kidneys is a rare congenital anomaly. It is usually associated with congenital anomalies of the gut. Development of malignancy in crossed fused ectopic kidney is uncommon. We present two cases, both females aged 47 and 60 years with crossed fused renal ectopia detected due to the development of malignancy in them. This report highlights the rare occurrence of the entity and role of US, CT scan and MRI in such patients.

Laparoscopic dismembered pyeloplasty for ureteropelvic junction (UPJ) obstruction is considered to be a routine procedure in many pediatric surgical centers. UPJ obstruction is known to be associated with horseshoe kidney and several reports on successful laparoscopic repair in such cases exist. The case of a 9-month-old girl with Turner syndrome is reported. A horseshoe kidney with grade 4 hydronephrosis on the left side was diagnosed by ultrasound during the neonatal period. MAG3 diuretic renography and dynamic magnetic resonance imaging nephrography revealed a differential renal function of 31% and 69% on the left and right side, respectively. No drainage from the left renal pelvis could be demonstrated. Laparoscopy showed a combined UPJ obstruction and a calyceal diverticulum with a narrow infundibulum of the upper pole calices on the left side of the horseshoe kidney. Laparoscopic dismembered pyeloplasty and an additional infundibulopelvic anastomosis was performed. No intraoperative complications occurred. The immediate postoperative course was uneventful. Unobstructed drainage and stable differential renal function on the left side could be demonstrated on MAG3 diuretic renography 6 weeks postoperatively. In conclusion, laparoscopic repair of complex malformations of the upper urinary tract is feasible and leads to good functional outcome in selected cases., (Copyright © 2010 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Following the introduction of routine prenatal scanning, and more recently detailed anomaly scans, antenatal ultrasound (US) diagnosis of fetal renal tract abnormalities has become well established. Prenatal detection has resulted in a group of asymptomatic infants becoming patients in the last two decades. These infants are referred for paediatric (general, urological and nephrological) consultations, radiological imaging and monitoring. The urgency with which to undertake imaging depends on the suspected antenatal diagnosis and clinical scenario, with bilateral hydronephrosis, posterior urethral valves and complicated duplex systems taking a high priority. Treatment is mainly preventive and relies on close follow-up and timely intervention. US remains the main imaging modality of choice postnatally; together with nuclear medicine, it gives a powerful combination of both anatomy and function. However, magnetic resonance urography may have increasing importance in becoming the investigation of choice of the future for more complex cases.

Radiofrequency ablation (RFA) has emerged as a minimally invasive nephron-sparing treatment for small (<4-cm) renal tumors. Post-RFA complications have been reported. We describe a patient who developed complete renal-pelvic obstruction after RFA. To our knowledge, this is the first such case to be reported and the second reported renal-unit loss as the result of collecting-system obstruction after RFA.

Kidney diseases lead to the failure of urinary excretion of metabolism products. In the Munich ethylnitrosourea (ENU) mouse mutagenesis project, which is done on a C3H inbred genetic background, blood samples of more than 15,000 G1 offspring and 500 G3 pedigrees were screened for alterations in clinical-chemical parameters. We identified 44 animals consistently exhibiting increased plasma urea concentrations. Transmission analysis of the altered phenotype of 23 mice to subsequent generations led to the establishment of five mutant lines. Both sexes were affected in these lines. Urinary urea levels were decreased in the mutants. In addition, most mutants showed increased plasma and decreased urinary creatinine levels. Pathological investigation of kidneys from the five mutant lines revealed a broad spectrum of alterations, ranging from no macroscopic and light microscopic kidney alterations to decreased kidney weight-to-body weight ratio, dilation of the renal pelvis, and severe glomerular lesions. Thus screening for elevated plasma urea levels in a large-scale ENU mouse mutagenesis project resulted in the successful establishment of mouse strains which are valuable tools for molecular studies of mechanisms involved in urea excretion or which represent interesting models for kidney diseases.

Congenital urinary tract obstruction is the most important cause of renal insufficiency in infants and children, and angiotensin-converting enzyme (ACE) inhibitors attenuate the progression of renal disease in adults. ACE inhibitors are increasingly utilized in children with progressive renal disease. Because angiotensin is necessary for normal renal development, we examined the effects of ACE inhibition both during and immediately following the period of postnatal nephrogenesis in the neonatal rat subjected to sham operation or partial unilateral ureteral obstruction (UUO) under general anesthesia within the first 48 h of life. Rats in group I received enalapril 30 mg/kg body wt (or vehicle) daily for the first 10 days, while in group II, the 10 days of treatment began 10 days after surgery. Kidneys were harvested at day 21 and analyzed for apoptosis (TUNEL), interstitial macrophages (ED-1 immunohistochemistry), myofibroblasts (alpha-smooth muscle actin), and collagen (Sirius red). Partial UUO delayed glomerular maturation and increased ipsilateral renal macrophage infiltration, alpha-smooth muscle actin and Sirius red staining. In group I, enalapril increased myofibroblast accumulation in sham-operated kidneys, but not in obstructed kidneys. In contrast, in group II, enalapril further increased macrophage, myofibroblast, and collagen accumulation following partial UUO. The relative abundance of components of the kallikrein-kinin system, measured by Western blot, was not altered by partial UUO in the 14- and 28-day-old rat. Thus, in contrast to its salutary effects at later ages, ACE inhibition can worsen injury to the partially obstructed kidney during renal maturation even after the completion of nephrogenesis.

Aim: The purpose of this study was twofold: first to provide data for more accurate counselling of parents with regard to prognosis, and second, to ensure that by following a policy of selective micturating cystourethography (MCUG), significant pathology is not missed, in particular vesicoureteric reflux (VUR). (MCUG is only undertaken if the renal pelvic diameter (RPD) is >/= 10 mm or if there is calyceal or ureteric dilatation.), Material and Methods: Data were collected prospectively over a 6-year period. Pre and postnatal imaging findings were collected for all infants in whom a RPD of >/= 5 mm was identified at any gestational age. The imaging records of all patients were reviewed in 2005 for evidence of pathology detected after re-presentation with symptoms. The age range at review varied from 2-8 years., Results: Complete data were available in 527 infants. The risk of significant pathology was related to the degree of antenatal renal pelvic dilatation varying from 6% for a RPD of 5 mm at 20 weeks gestation to 38% at 10 mm. At 28-33 weeks gestation the risk varied from 5% at 5mm to 15% at 10 mm. Subsequent imaging record review revealed only one patient with grade II VUR in the study population not picked up by our selective MCUG policy., Conclusion: The present study provides prognostic information that can be given to parents both antenatally and postnatally, and reassurance that a selective MCUG policy is appropriate.

Objective: This study describes the frequency of sonographically detected fetal renal disorders, the correlation of fetal renal pelvis dilatation (RPD) with the need for postnatal surgery, and proposed management of RPD., Methods: The study population consisted of 342 fetuses with prospectively identified isolated renal abnormalities and known follow-up. Fetuses with RPD were considered separately with respect to underlying renal disease, postnatal testing, and the need for surgical correction. Obstructive RPD was defined as the need for surgical treatment. Nonobstructive RPD included those cases that required no therapy. The correlation between prenatal RPD and the need for postnatal evaluation was examined., Results: Renal pelvis dilatation was the primary postnatal sonographic finding in 66.4% of cases. The remainder were distributed between multicystic dysplastic kidney, duplication malformations, and reflux, with a smaller number of other diagnoses. Renal pelvis dilatation in the obstructive group was significantly greater than in the nonobstructive group. However, 10% of fetuses with maximum RPD of 10 mm or less had an obstructive process, whereas 58% of fetuses with RPD of greater than 10 mm did not have obstruction. There were no cases in which preterm delivery was necessitated by RPD., Conclusions: Renal pelvis dilatation is the most common fetal renal abnormality. The greater the RPD, the more likely it is due to obstruction. However, the overlap between obstruction and no obstruction dictates postnatal evaluation. In that RPD, regardless of degree, did not change the timing of delivery, a single follow-up sonographic examination either late in pregnancy or after delivery is considered adequate for follow-up of RPD detected earlier in pregnancy.

Purpose: We reviewed our experience with open dismembered pyeloplasty, with specific focus on the presentation and management of failed pyeloplasty in the pediatric population., Materials and Methods: We performed a retrospective review of patients who had undergone open dismembered pyeloplasty between 1998 and 2003. All patients with less than 6 months of followup were excluded from analysis. The patients were followed postoperatively with serial ultrasounds, with renograms reserved for those patients with prolonged, persistent or worsening hydronephrosis, or recurrent symptoms during followup., Results: A total of 105 pyeloplasties were performed in 103 patients (71 males and 32 females) 1 to 204 months old (mean 60), with an overall success rate of 93.3%. Followup ranged from 6 to 69 months (mean 23). The 7 patients with treatment failure were males 1 to 204 months old (mean 125), who presented most commonly with pain within 3 to 38 months (mean 13.1) after initial surgery. Subsequent ultrasound revealed worsening hydronephrosis, and renography in these patients showed a pattern consistent with obstruction. Five patients underwent initial balloon dilation, in which 1 was successful. In addition, 1 of these patients underwent an unsuccessful antegrade laser endopyelotomy. Six patients (86%) underwent open surgery, consisting of ureterocalicostomy in 3 and reoperative dismembered pyeloplasty in 3. Dense scarring was seen in all patients, 2 patients had a redundant pelvis causing kinking and 2 patients had unrecognized crossing vessels. Overall salvage rate was 100% with continued followup of 3 to 50 months (mean 18)., Conclusions: Dismembered pyeloplasty was successful in the vast majority of patients. In our series failures occurred as late as 3 years postoperatively, although most presented within 12 months of followup. Excluding routine postoperative nuclear renography did not seem to affect our ability to identify these cases. Although anatomical features such as renal pelvic size may have a role, failure is most likely secondary to technical issues, including missed crossing vessels and dependency of the anastomosis. In this series failed pyeloplasties did not respond well to balloon dilation, likely due to scar formation. Our current practice is to manage failures by open surgery, although endoscopic management by an incision may be an option.

Moderate antenatal renal pelvic dilation (5-15 mm) may suggest vesicoureteric reflux, but it is not known to predict renal scarring. Dimercaptosuccinic acid scans on such children aged over 4 years showed a scarring rate (0/133 boys, 1/56 girls) similar to our local population. Investigation and treatment of moderate dilation may not be required.

Cystic hamartoma of the renal pelvis is a rare benign tumor in the same category as mixed epithelial and stromal tumors. We present a 33-year-old woman with a solid and cystic intrarenal tumor extending into the renal pelvis. She underwent radical nephrectomy and ureterectomy under the diagnosis of renal tumor or renal pelvic tumor. Histopathologically, the tumor was composed of a biphasic proliferation of epithelial and mesenchymal elements. We believe the present case is best classified as a cystic hamartoma of the renal pelvis in the category of mixed epithelial and stromal tumors because of the coexistence of hamartomatous lesions, such as the proliferation of adipose cells and well to poorly differentiated fibromuscular lesions.

Objective: To report a rare case of plasma cell granuloma of the renal pelvis. To our knowledge, only five cases have been reported in the literature., Methods: Clinicopathological description of a case of plasma cell granuloma of the renal pelvis with light chain immunohistochemical study., Results: A young male patient with a radiologically benign mass in the right renal pelvis extending into the upper calices that caused hematuria is described. The diagnosis was suggested during the intraoperative study of the mass that was suggested by pyelotomy (conservative surgery). Immunohistochemical study confirmed the initial diagnosis., Conclusions: Although this condition is rare, plasma cell granuloma, an inflammatory pseudotumor, can occur in the urinary tract and should be considered in the differential diagnosis of mass lesions. Histopathological diagnosis requires a complete study of the entire tumor and the use of immunohistochemical techniques to discard plasmacytic non-Hodgkin lymphoma or neoplastic plasma cell proliferation.

Background: To study the usefulness and safety of ureteropyeloscopy in the diagnosis of upper tract hematuria of unknown etiology by standard diagnostic methods., Methods: Fifteen patients with upper tract hematuria of unknown etiology were the subjects of the present study. Prior to ureteropyeloscopy, they underwent standard diagnostic methods, including cystourethroscopy, excretory urography and computed tomography scan. The upper tract (ureter, renal pelvis and calyces) was inspected systematically with a flexible ureteropyeloscope under epidural anesthesia. A biopsy specimen was obtained when neoplasm of a suspicious lesion was seen. Bleeding and hemangiomatous lesions were fulgurated at the time of ureteropyeloscopy., Results: Unilateral gross hematuria was seen in 12 patients. Imaging studies revealed a filling defect in four patients, ureteral stenosis in one patient and nutcracker phenomenon in one patient. Urine cytology was positive in three patients and suspicious in four patients. Results of ureteropyeloscopy were papillary tumor in three patients, whitish encrustation in one patient, redness of the renal pelvis in one patient, bleeding from the renal calyx in two patients, hemangiomatous lesion in one patient, ureteral stenosis in two patients and no abnormalities in five patients. Biopsies were performed in five patients. The pathology results were transitional cell carcinoma in four patients and no abnormality in one patient. Although a ureteral stent catheter was placed in one patient, no serious complications were encountered during or after the procedures., Conclusions: Ureteropyeloscopy was useful and relatively safe. This endoscopic examination can differentiate insignificant lesions from significant lesions by visual inspection of the lesions, in addition, pathological diagnosis by biopsy specimen can also be performed if deemed necessary. Ureteropyeloscopy is recommended in the diagnosis of upper tract hematuria of unknown etiology.

Background: Inhibition of angiotensin action, pharmacologically or genetically, during the neonatal period leads to renal anomalies involving hypoplastic papilla and dilated calyx. Recently, we documented that angiotensinogen (Agt -/-) or angiotensin type 1 receptor nullizygotes (Agtr1 -/-) do not develop renal pelvis nor ureteral peristaltic movement, both of which are essential for isolating the kidney from the high downstream ureteral pressure. We therefore examined whether these renal anomalies could be characterized as "obstructive" nephropathy., Methods: Agtr1 -/- neonatal mice were compared with wild-type neonates, the latter subjected to surgical complete unilateral ureteral ligation (UUO), by analyzing morphometrical, immunohistochemical, and molecular indices. Agtr1 -/- mice were also subjected to a complete UUO and were compared with wild-type UUO mice by quantitative analysis. To assess the function of the urinary tract, baseline pelvic and ureteral pressures were measured., Results: The structural anomalies were qualitatively indistinguishable between the Agtr1 -/- without surgical obstruction versus the wild type with complete UUO. Thus, in both kidneys, the calyx was enlarged, whereas the papilla was atrophic; tubulointerstitial cells underwent proliferation and also apoptosis. Both were also characterized by interstitial macrophage infiltration and fibrosis, and within the local lesion, transforming growth factor-beta 1, platelet-derived growth factor-A and insulin-like growth factor-1 were up-regulated, whereas epidermal growth factor was down-regulated. Moreover, quantitative differences that exist between mutant kidneys without surgical obstruction and wild-type kidneys with surgical UUO were abolished when both underwent the same complete surgical UUO. The hydraulic baseline pressure was always lower in the pelvis than that in the ureter in the wild type, whereas this pressure gradient was reversed in the mutant., Conclusion: The abnormal kidney structure that develops in neonates during angiotensin inhibition is attributed largely to "functional obstruction" of the urinary tract caused by the defective development of peristaltic machinery.

Background: Renal sarcoidosis exceptionally presents as a unilateral pyelic pseudotumor as in this case where it was associated with granulomatous nephropathy., Case Report: A 33-year-old man had a two-year history of systemic sarcoidosis with no renal involvement. He developed renal failure related to interstitial granulomatous nephropathy associated with a pyelic localization leading to unilateral hydronephrosis. Urine drainage associated with corticosteroid therapy provided a favorable course., Discussion: Renal involvement in sarcoidosis is usually the consequence of hypercalcemia and hypercalciuria related to ectopic secretion of calcitriol by the sarcoidosic granulomas, with urinary lithiasis and nephrocalcinosis leading to renal failure and also granluomatous interstitial nephropathy. Glomerulopathy or obstructive nephropathy are rarely reported. Intraluminal localizations such as the pyelic lesion in our case are exceptional but must be detected early since they respond to corticosteroid therapy.

We report a case of a rare cystic renal tumor previously termed cystic hamartoma of the renal pelvis. A 53-year-old woman presented to her gynecologist with menometorrhagia. She subsequently had a computed tomographic scan that demonstrated an incidental cystic mass in the lower pole of the left kidney. Histologically, the tumor was composed of a mixture of benign mesenchymal and epithelial components. The stroma consisted of spindle cells with monomorphic nuclei and abundant eosinophilic cytoplasm that resembled smooth muscle and that reacted positively with antibodies to alpha-smooth muscle actin, desmin, and vimentin. The epithelial component was composed mostly of cysts lined by cuboidal-to-columnar epithelium. Focal dilated cysts were lined by epithelium with oncocytic features. We think that this entity is distinct from other renal tumors, including mesoblastic nephroma, cystic nephroma, or a cystic, partially differentiated nephroblastoma, and that it is best classified as a cystic hamartoma of the renal pelvis.

We report the changes in imaging findings over a 5 year period of a patient with unilateral malakoplakia involving renal parenchyma and pelvis. The imaging findings of malakoplakia will be reviewed.

Chyluria is due to the passage of chyle into the urine giving it a typical milky appearance. Although rarely seen in our countries it is well-known in areas where infections with Wuchereria bancrofti are endemic. Chyluria can also have non-parasitic causes of which only a few cases have been described. We briefly discuss etiologies and pathogenesis of chyluria and present a case of non-parasitic chyluria successfully treated by surgery. A short and critical review of the different treatment modalities is made.

Abdominal ultrasound examinations of 20 dogs with confirmed leptospirosis were reviewed retrospectively for renal abnormalities. Three dogs had a normal ultrasound examination. The remaining 17 dogs had sonographic abnormalities of the kidneys. These abnormalities, seen either alone or in combination, included renalmegaly (n=10), pyelectasia (n=9), increased cortical echogenicity (n=15), perinephric effusion (n=5), and a medullary band of increased echogenicity (n=6). At our institution, the medullary band of increased echogenicity has only been seen in dogs with leptospirosis and may therefore be a specific sonographic sign for this disease.

The authors report a case of malakoplakia of the left renal parenchyma, an uncommon site for an inflammatory disease first described in the bladder by Michaelis and Guttmann in 1902 and Von Hansmann in 1903. This case was observed in a 24-year-old girl with no urological history, presenting with bilateral vulval hypertrophy and reflex sympathetic dystrophy of the upper limbs, in whom malakoplakia of the left kidney was discovered incidentally. Intravenous urography showed two large kidneys with a stretched appearance of the renal pelvis. Pathological examination of the partial nephrectomy specimen revealed the diagnosis of malakoplakia, an inflammatory diseases of the renal parenchyma. The pathogenesis has been only partially elucidated, but probably involves a disorder of macrophage function.

We evaluated postnatal management after the prenatal diagnosis of moderate dilatation of the renal pelvis (antero-posterior diameter superior to 1 mm per month of pregnancy and inferior to 15 mm at full term). Eighty-nine neonates who had a moderate dilatation in utero were evaluated by ultrasound and voiding cysto-urethrography. Ultrasound was performed between day 2 and day 7 in 83 cases, after day 7 in 6 cases. Voiding cysto-urethrography was performed during first month in 80 cases, after this period in 9 cases. Sixty-five neonates were shown to have a malformation of the urinary tract: reflux (n = 27), megaureter (n = 20), uretero-pelvic junction obstruction (n = 19), renal duplication (n = 7), posterior urethral valves (n = 2), horseshoe kidney (n = 1), ureteric cyst (n = 1). Reflux which is frequent in neonates with urinary tract infection, is the most frequent cause of moderate dilatation of the fetal renal pelvis. Combination of ultrasound and voiding cysto-urethrography in neonates allows a quick, full diagnosis. It helps rationalize the use of prophylactic antibiotic treatment.

A 48-year-old male experienced sudden pain in the right flank and macrohematuria with subsequent repeated episodes of painless macrohematuria. In the radiological work-up, CT was interpreted as inflammatory enlargement of the right kidney and MRI diagnosed renal vein thrombosis. There was some suspicion that there might be a malignancy. Five months later the patient had to be admitted to the hospital, because of increasing flank pain, desiccation and general signs of inflammatory disease. Radiological changes compatible with pulmonary metastases were found. Biopsy of enlarged mediastinal lymph nodes revealed undifferentiated carcinoma. Because of the expression of Cytokeratin primary urothelial carcinoma was suspected. The patient then deteriorated rapidly. No further specific therapy could be carried out. Death was chiefly caused by respiratory insufficiency 5 months after the first episode of pain and 1 month after admission. Autopsy revealed a poorly differentiated urothelial carcinoma of the right renal pelvis, with extensive carcinomatous angiomatosis and metastases to the left kidney, lungs, and regional and mediastinal lymph nodes. Independently of the carcinoma, both kidneys also showed interstitial nephritis, papillary necrosis and capillarosclerosis, compatible with analgesic-user kidney ("phenacetin kidney") in the absence of a corresponding history.

The authors report the various therapeutic modalities of uropathies associated with horseshoe kidney, based on a series of 20 pathologic horseshoe kidneys, associated with 12 cases of renal stones, 5 ureteropelvic junction obstructions, 3 kidney tumors, 2 cases of pyonephrosis and finally 1 traumatic horseshoe kidney. The specific anatomic and surgical features of this uncommon malformation are emphasized and the therapeutic features of each uropathy associated with horseshoe kidney are discussed.

Calcification in the wall of the renal pelvis is rare. We report on a 65-year-old man with hydronephrosis secondary to ureteropelvic junction obstruction with renal pelvic calcification. Calcium deposit was found in the wall of the severely dilated renal pelvis. Pathological examination revealed a damaged and hyalinized fibrous renal pelvic wall and serum calcium level was normal. Thus, we speculated that this calcification was dystrophic. Chronic extensive dilatation with intermittent hemorrhage of the renal pelvic wall may have caused this dystrophic renal pelvic calcification.

The authors report a case of Waldenström's disease in which the initial staging assessment reveals an isolated tumour of the left upper urinary tract. Combination chemotherapy (6 courses of protocol M2) induced a marked reduction in the monoclonal IgM peak and in the tumour mass. In the light of this atypical case, which emphasises the diversity of the potential sites of Waldenström's macroglobulinaemia, the authors review its potential for progression and the various therapeutic modalities available.

To assess the epidemiology of habitual use of analgesics in South-West Germany (i) urine specimens of employees of a factory, of patients seen by a general practitioner (GP) and of patients attending a renal clinic were examined for paracetamol; (ii) mucosa of the left renal pelvis was examined for the presence of capillary sclerosis as an index of habitual use of phenacetin or paracetamol in 258 consecutive autopsies; (iii) in a regional renal clinic, the frequency of analgesic nephropathy was determined amongst outpatients and amongst patients on hemodialysis. Paracetamol was found in the urine of 4.1% of factory employees, in 3.5% of patients seen by a GP and in 2% of patients seen in the renal clinic. Capillary sclerosis was found in only one of 258 autopsies, in a patient dialysed for analgesic nephropathy. During the past two years, analgesic nephropathy was diagnosed in 3% of all patients seen for nephrological examination. Analgesic nephropathy was the cause of terminal renal failure in 15 of 166 patients (9.4%) on dialysis. The present study documents that habitual use of paracetamol-type analgesics continues in the general population despite the ban of phenacetin.

The association between analgesic nephropathy and urothelial cancer, usually in the renal pelvis, is well established. We report two such patients in whom careful morphologic study of nephrectomy specimens demonstrated severe and extensive urothelial dysplasia, with focal carcinoma in situ and grossly invisible invasive tumors. In one of these patients, no gross lesion was apparent in the urothelium. These findings support the development of invasive carcinoma through a phase of carcinoma in situ caused by the action of carcinogenic analgesic metabolites in the urine. Further, they indicate the need for caution in the assessment of urinary cytologic atypia and for care in the examination of excised urothelium in those patients.

A 62-year-old woman complaining of asymptomatic hematuria was hospitalized. Although a cytologic examination of the urine was negative, abnormal findings in the right renal pelvis by DIP, RP and CT led us to suspect a pelvic tumor, and a right nephroureterotomy was performed. Light microscopy revealed deposits of amorphous, eosinophilic material in the pelvis and proximal portion of upper ureter, however tumor cells were not found. Electron microscopy revealed that these materials were aggregates of linear, non-branching fibrils, measuring about 8 nm in diameter. Histochemically, these were seen to be consistent with amyloid, producing an apple-orange birefringence with Congo red staining under a polarized light. Alkali Congo red staining after pretreatment of KMnO4 demonstrate that this amyloid protein was composed largely of AA protein in addition to lesser amounts of non-AA protein, and a primary localized amyloidosis (amyloid tumor) was diagnosed. A clinicopathologic study that was performed on 12 cases of primary localized amyloidosis of the pelvis, including our case, also is reported.

One rare case of spontaneous rupture of pyelocaliceal diverticulum is reported in a 31-year-old Japanese man presented with a severe left flank pain. Intravenous pyelography and computerized tomography demonstrated extravasation of contrast medium from his diverticulum which contained numerous calculi. As a possible cause of spontaneous rupture of pyelocaliceal diverticulum in this case, we suggest that the paper-thin parenchyma covering the diverticulum was not strong enough to tolerate the minimum elevation of intrapelvic pressure due to the obstruction of delivered stones.