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Autosomal dominant polycystic kidney disease (ADPKD) is one of the best-known genetic diseases. Almost half of the patients with ADPKD will develop end-stage renal disease, and the majority of patients are treated with renal transplantation. The current study presents a case that developed papillary renal cell carcinoma (PRCC) in the native right kidney 10 years after renal transplantation. PRCC is a not common malignant tumour entity (18.5% of all cases of renal cell carcinoma) compared with common clear cell renal carcinoma (65-70% of all cases of RCC)., Competing Interests: The authors declare that they have no competing interests., (Copyright: © Abbas et al.)

Background: Urethrocutaneous (UC) fistulae are common complications after hypospadias surgery and they have been a serious problem for surgeons since the repair was first attempted. We present the results of our multicentre retrospective study for repairing UC fistulae using the Patio ("preserve the tract and turn it inside out") repair described by Malone., Materials and Methods: A total of 16 boys (Eschweiler 2, Lingen 4, Reading 10) at the ages of 1-10 years were treated for UC fistulae. Instead of excising the fistula tract, it is preserved and turned inside out, this creates a flap valve inside the urethral lumen. After a circumferential incision around the skin and meticulous dissection of the fistula tract, a 2/0 nylon suture is passed down the tract and brought out through the external urinary meatus. As a result, the fistula tract is inserted into the urethral lumen. In order to keep the fistula tract inverted, it is sutured to the tip of the external urinary meatus, or fixed by an angler lead (modification from Lingen). Due to the narrow base, the excess tissue atrophies postoperatively and leads to an appealing cosmetic result., Results: A total of 9 fistula repairs were performed on an outpatient basis without using a transurethral catheter; 7 boys were treated on an inpatient basis with an average length of stay in the hospital for 1-2 days with/without catheterization. During a mean follow-up of up to 4.5 years, only one fistula recurrence occurred; no other complications were observed., Conclusion: The Patio repair for urethrocutaneous fistula is an outpatient, simply reproducible surgical technique without the necessity of transurethral catheterization. The short-term results are impressive; long-term results of a larger patient cohort will follow.

Until a few years ago, the treatment options for metastatic renal cell cancer (mRCC) were very limited. The growing understanding of the molecular pathomechanisms underlying RCC allowed the development of new treatment approaches. Meanwhile, several approved target-oriented substances from different drug classes are available for mRCC. The mechanism of action of vascular endothelial growth factor (VEGF) and VEGF receptor or mTOR inhibition is well documented by phase III trials and reflected in the current guidelines. However, no predictive biomarkers have been identified in mRCC so far to demonstrate a benefit by a specific compound in an individual patient. Meanwhile, the sequential use of 'targeted therapies' in mRCC has been established as standard treatment. The optimal sequence of available agents is still unclear. A German RCC expert panel discussed and developed an algorithm for the choices of first- and second-line treatment in mRCC based on established clinical criteria.

Background: The aim of this study was to evaluate the laparoscopic abdominal access modifications in children with prune belly syndrome undergoing a first stage Fowler-Stephens procedure., Study Design: Eleven consecutive boys underwent a transperitoneal laparoscopic bilateral first stage Fowler-Stephens procedure. Patient age ranged from 1.5 to 3 years (mean age 2.2 years). In these patients, the floppy abdominal wall required a modified approach with regard to access technique, insufflation pressures, and work port stabilization methods. Duration of the procedures and intraoperative technical challenges encountered were prospectively documented., Results: Mean operative time was 40 minutes (range 30 to 75 minutes), and all procedures were completed without any complications. Forceful insertion of ports was not possible, and all ports were introduced under complete open access. Larger volumes of carbon dioxide were used in the initial part of our series, when the ports were not sutured to the abdominal wall. An abdominal pressure of 8 mmHg was maintained in all patients and was considered optimal for the procedures. Short laparoscopy instruments (240 mm) were unsuitable for the procedures and had to be replaced by longer instruments (310 mm or 430 mm)., Conclusions: Technical modifications are required to the approach in laparoscopic abdominal access to overcome the challenges posed by the floppy abdominal wall in prune belly patients. Open access, suture fixation of the optic and work ports, use of threaded sleeve ports, and use of proper length of laparoscopy instruments are valuable modifications to overcome the technical hurdles posed by these patients.

Objectives: Combined immunochemotherapy with interleukin 2 (IL-2), interferon alpha (IFN-alpha), and 5-fluorouracil (5-FU) is an established first-line therapy for metastatic renal cell carcinoma (RCC). However, data on histologic parameters predictive of clinical benefit are rare. In this study, we evaluated the response to immunochemotherapy in the main histologic subtypes of renal cell carcinoma and performed a subgroup analysis of inoperable patients., Methods: From 164 patients treated with one or two cycles of combined immunochemotherapy, radical nephrectomy had revealed 22 cases of papillary RCC (pRCC; 13.4%) and 131 cases of clear cell RCC (ccRCC; 79.9%). In the remaining 11 (6.7%) their disease was inoperable. The overall response rates were evaluated according to World Health Organization criteria., Results: For ccRCC and inoperable disease, responses of 34.4% and 27.3% after one cycle and 28.8% and 16.7% after two cycles, respectively, were noted. In contrast, no patient with pRCC showed any response after two cycles of combined immunochemotherapy., Conclusions: No objective response was seen in patients with pRCC. Hence, the use of immunotherapeutic agents must be questioned in this histologic subtype.

Endothelin-1 and its receptors ETAR and ETBR, commonly referred to as the Endothelin-axis, are emerging to play a role in cancer. The Endothelin-axis has been shown to be involved in proliferation, angiogenesis and metastasis in various human tumours. To assess the role of the Endothelin-axis in renal cell carcinoma, we analysed its expression in archival tumour tissue of 183 patients. Representative tumour blocks were selected for constructing a tissue microarray. Paraffin sections were assessed immunohistochemically using monoclonal and polyclonal antibodies for Endothelin-1, ETAR and ETBR. Staining intensities were analysed semiquantitatively and the results were correlated with various histopathologic factors. Overexpression of Endothelin-1, ETAR and ETBR was identified in 12.8%, 84.1% and 93.3% of cases, respectively. No association with pathological tumour stage and histologic grading was found. Papillary renal cell carcinomas expressed highly significantly more Endothelin-1 than clear cell renal cell carcinomas (34.5% vs. 6.7%, p<0.001), while there was no difference between ETAR- and ETBR-expression in these histologic subtypes. However, ETAR tended to be overexpressed in the subgroup of G3-tumours (p=0.044). Studies are underway assessing the role of the Endothelin-axis and its potential use as a molecular target in renal cell carcinoma.

Renal angiomyolipomas are mesenchymal tumors that are composed of fat tissue, smooth muscle cells and vessels. These are benign tumors, but in rare cases they show a more aggressive growth pattern with invasion into the venous system but without revealing any signs of malignancy. We report a new case of bilateral renal angiomyolipomas with a caval thrombus in a 36 year old female patient with tuberous sclerosis, and give a brief review of the related literature.

"Obstructive uropathy" is a generic term which combines different diseases in infants and childhood. Both the upper and lower urinary tract may be affected. Diseases of the urinary tract can cause an intrinsic obstruction. Sometimes tumours may cause a compression and as secondary effect an obstruction (extrinsic). Ultrasound is the key diagnostic tool and shows dilatation of the obstructed urinary tract. But for the functional exploration of babies and toddlers, renal scanning and X-ray examinations are necessary. These examinations lead to an exposure to radiation which necessitates careful indication. Some of the congenital diseases (for example ureteropelvic junction obstruction, megaureter) show a maturation without any intervention. So one has to decide whether to wait and see or to operate. A percutaneous nephrostomy or a DJ-catheter is not often used in the treatment of obstruction in general. These forms of drainage are more often used in the treatment of stones or of extrinsic obstruction. A pyelocutaneostomy or ureterocutaneostomy is a special surgical procedure in pediatric urology for transient drainage of the upper urinary tract (megaureter). The operation of a seriously ill new-born should be done in a centre for pediatric urology and pediatric nephrology. When the upper urinary tract is dilated, patients may need an antibiotic prophylaxis, because the dilatation of the upper urinary tract increases the risk of urinary tract infections (UTI). The indication for antibiotic prophylaxis should by guided by the criteria of the APN-Consensus Paper. Long-term follow-up is necessary and should comprise ultrasound, physical examination, controlling the blood pressure, urine analysis and blood tests. The aims of diagnostics, treatment and long-term follow-up are the preservation of renal function and to protect the children from UTI. This goal must be reached under conditions that are appropriate for children and their parents.

Immunotherapies using interferons and/or interleukins are currently the treatment of choice for metastatic renal cell carcinoma (RCC). Bone metastases and non-resectable local recurrence are negative predictors for successful immunotherapy and signs of poor prognosis. The present study was designed to evaluate the effectiveness of combined immunochemotherapy (ICT) and radiation therapy (RT) for bone metastases or local recurrence from RCC in a prospective fashion. From September 1997 to September 1999, 20 patients with progressive RCC were treated with a combination of RT and ICT [s.c. interleukin-2a (IL-2), s.c. interferon alpha (IFN-alpha) and i.v. 5-fluorouracil]. RT started in week 2 of ICT. The radiation field was limited to the symptomatic bone metastases (15 patients) or the local recurrence (five patients). The total dosages of the RT ranged between 45 and 50 Gy, administered in fractions of from 1.8 to 2 Gy daily. In case of objective response or stable disease, the patients received up to two further ICT courses. All patients had good pain relief. Three out of 20 achieved complete remission, three had a partial remission, nine were stable and five patients had progressive disease under the combined treatment. Median survival was 21 months, mean survival 24 months (range: 5-59 months). The side effects of the combined treatment are in the same range as with ICT alone (World Health Organisation grade 2 and 3). Of 20 patients, 19 had their pain medication reduced after treatment. The combination of ICT and RT is feasible. There is remarkable pain relief. Our data suggest that the combination of immunochemotherapy and radiation therapy may induce a synergistic antitumor effect for the treatment of bone metastases or local recurrence from RCC compared to data from the literature for ICT or RT alone.

Regional anesthetic techniques are widely used in pediatric anesthesia since they represent excellent tools to prevent and treat postoperative pain. Their range of application has considerably expanded during the last decades, and virtually all aspects of surgical pain management can benefit from their appropriate use. Previously considered unreliable and potentially hazardous, regional anesthetic techniques have now been proven safe. Most children undergoing outpatient surgery can benefit from regional anesthetic techniques, either as the sole anesthetic regimen or, as usual in pediatric practice, in combination with light anesthesia. Central blocks as well as peripheral nerve blocks are well tolerated by infants and young children. They are easy to perform and show a high level of efficacy. Only the patient's history has to be available. No tests, such as invasive blood coagulation screening, are required. Subsequent to a regional anesthesia, non-opioid analgetics, e. g., paracetamol or ibuprofen, have to be used for further pain management.

An 11-mo-old child with primary hyperoxaluria was scheduled for a nephroureteromia procedure. Anesthesia was induced and maintained with sevoflurane. Two days after the operation, a hepatomegaly was diagnosed, and a considerable increase in liver enzymes was observed. These pathologic findings disappeared without treatment within 7 days. In a subsequent operation 2 wk later, general anesthesia was performed (sevoflurane was avoided). After the second operation, no pathologic findings could be detected. Nothing in this patient's disease or the conduct of the anesthesia suggested a cause for the injury other than an idiosyncratic response to sevoflurane.

Objectives: To report a retrospective study of unilateral multicystic dysplastic kidneys (MCDK) in children, assessing the contralateral kidneys and urinary tract, the functional consequences, and the urological and nephrological management and outcome, as unilateral MCDK is the most common cause of renal cystic disease in children, and malformations of the contralateral urinary tract and kidney (pelvi-ureteric obstruction, megaureter, reflux, renal dysplasia) have been reported., Patients and Methods: The study included 97 patients (60 boys, 37 girls) with MCDK seen between 1985 and 1998; 82 were diagnosed in utero by ultrasonography (US). After birth, the diagnosis was verified by US, renal scanning (in 93) or intravenous urography (in four), and 89 (92%) had voiding cysto-urethrography (VCUG). Of the 97 children, 87 (90% had a mean (range) follow-up of 44.3 (15-115) months., Results: The MCDK was removed in 17 children; the follow-up of 75 children (five lost to follow-up) showed total involution of the MCDK in 25%, shrinkage in 60% and a stable size in 15%. None had any sign of malignancy. The contralateral kidney showed anomalies in 19 of 97 children (20%); 12 had a dilated renal pelvis (two with megaureter), six had a high echogenicity of the contralateral kidney (one had reflux, and two also pelvic dilatation). In only four of the 89 children was reflux found by VCUG; 16 of the 19 anomalies were detected by US. Five children needed surgery on the contralateral urinary tract (three a pyeloplasty, and one each a pyeloplasty plus ureteroneocystostomy, and an antireflux procedure). Of the contralateral kidneys 43% showed compensatory hypertrophy. There was mild renal insufficiency in three children; renal function seemed to be slightly impaired in many. Five infants had hypertension (four with spontaneous resolution) caused by renal scarring after pyelonephritis or inborn dysplasia of the contralateral kidney. There were symptomatic urinary tract infections in seven children., Conclusion: US can be used safely to diagnose unilateral MCDKs and malformations of the contralateral urinary tract and kidney. In cases where US of the dysplastic kidney remains uncertain renal scintigraphy is necessary to detect the lack of renal function. The low rate of reflux makes routine VCUG unnecessary if the contralateral upper urinary tract and kidney appear to be normal on US. Nephrectomy of the dysplastic kidney in typical cases is also unnecessary. A long-term nephro-urological follow-up of children with MCDK is recommended.

Introduction: Congenital prepubic sinus (CPS) is a rare diagnosis. It is defined as a blind-ending tract originating from the midline of the genital region. There are three types of CPS classified according to the course of the tract and location of the skin opening. The etiology is thought to be an intussusception during fusion of the abdominal wall or, alternatively, incomplete urethral duplication., Case Report: We report on a two-year-old boy with a skin fistula on the dorsal side of the penis. A slight secretion occurred when the surrounding subcutaneous tissue was compressed. After total resection of the sinus, histological examination revealed that the tract was lined primarily with multilayered epithelium. Immunohistochemical studies showed that the sinus was lined with transitional and squamous epithelium. At the base the lining epithelium was transitional and shifted distally to noncornifying squamous epithelium. The epithelial layer therefore corresponded to the inner surface of the urethra, thus supporting the assumption that CPS results from incomplete urethral duplication., Conclusion: The immunohistochemical examination of the epithelium of Type II CPS proved, in this case, the existence of urothelium as the inner surface of the sinus. In view of this evidence it appears likely that the congenital prepubic sinus can be classified etiologically as an incomplete urethral duplication.

Within the last 10 years, immunotherapy has progressively become an established treatment for patients with metastatic renal cell carcinoma. The cytokines interleukin-2 (IL-2) and interferon-alpha (IFN-alpha) are the substances that have shown the greatest effects. Both have been approved for the treatment of patients with metastatic renal cell carcinoma in Germany. Subcutaneous application of these frequently combined cytokines is the schedule of immunotherapy used most often in Germany. Combined cytokine therapy (IL-2 and IFN-alpha) achieves response rates comparable to more aggressive immunotherapies. The retrospective analysis of treatment results from 66 patients with a follow-up of at least 5 years after the start of combined s.c. IL-2 and s.c. IFN-alpha +/- 5-fluorouracil (response classification: CR: 7, PR: 11, SD: 20, PD: 28) shows that the classification of the treatment results according to WHO criteria is the strongest predictor for survival compared with basic factors such as TNM status, grading, or number of metastatic sites. The combination of cytokine treatment with other treatment modalities (for example, surgical intervention) leads to a differentiated treatment according to the tumor status of the patient with metastatic renal cell carcinoma. Specific immunotherapies are still experimental. No approval has been granted for any of these treatments. Only standardization of these protocols can lead to a supplemental form of immunotherapy. Although several aspects of cytokine-based immunotherapy need further scientific evaluation, it is the treatment of choice for patients with metastatic renal cell carcinoma. However, for further progress in this field, prospective evaluation of immunotherapy for metastatic renal cell carcinoma is still needed. The German society for immunotherapy serves as a platform for this research.

Purpose: The assessment of plasma cytokine levels adds a useful tool to the diagnostic measures in severe inflammatory diseases. Proinflammatory cytokine levels in abdominal fluid after abdominal surgery have been shown to far exceed plasma cytokine levels. Thus, we investigated the local release of interleukin 1beta, interleukin 6, and tumor necrosis factor-alpha in patients after colorectal surgery during the early postoperative period to evaluate whether it may serve as an indicator of evolving peritonitis., Method: In a prospective, observational pilot study, the first 12 consecutive patients who did not develop any postoperative complications (Group I), and the first 12 patients with secondary peritonitis caused by an anastomotic leakage (Group II), were included in the study. Interleukin 6, interleukin 1beta, and tumor necrosis factor-alpha levels were determined in the abdominal exudate and compared between the groups within the first four days after colorectal surgery., Results: Abdominal fluid interleukin 6 levels in Group II patients were higher (162,500 +/- 105,800 pg/ml) as early as the first postoperative day compared with Group I (27,940 +/- 13,860 pg/ml; P < 0.0001); this lasted for the whole observation period. The same applies to tumor necrosis factor-alpha levels (461.4 +/- 167.8 pg/ml vs. 175.8 +/- 178.6 pg/ml on day 1; P = 0.0007). The difference in interleukin 1beta cytokine levels became statistically significant on the third postoperative day. Moreover, abdominal fluid cytokine levels rose in Group II, whereas they remained virtually unchanged or even tended to decrease over time in Group I., Conclusion: We suggest that the estimation of the peritoneal cytokine levels might be an additional diagnostic tool that can support the early recognition of peritonitic complications in colorectal surgery.

Ectopic kidneys are frequently associated with primary renal dysplasia or a disturbance of urine transport. Sacral agenesis is defined by the absence of two or more bodies of the lower vertebral and is often associated with a neurogenic bladder dysfunction. A 5-year-old boy with sacral agenesis and a right-sided cake kidney, presented with progredient renal failure caused by recurrent urinary tract infections, incomplete bladder emptying and vesicorenal reflux. After extensive diagnostics, the anatomical situation was explored by laparotomy to find a solution for this complex situation. Despite modern diagnostic tools, the preoperatively estimated renal function of the cake kidney was incorrect. After resecting of the right collecting system and refluxing megaureter out of the cake kidney, anti-refluxive implantation of the left ureter into the bladder was performed and the megacystis was treated by detrusor duplication. After three years, the now 8-year-old boy voids residual free without any signs of urinary tract infection. Renal function and proteinuria have improved. The only medication required is nifedipine (20 mg twice a day) for treatment of the renal hypertension.

Objectives: Extracorporeal shock wave lithotripsy (ESWL) is effective and safe for the treatment of upper urinary tract calculi in adults. Some speculations concerning possible damages from ESWL on the growing kidney have been raised., Methods: From January 1990 to December 1998, 64 children (30 girls and 34 boys; 8 months to 15 years old, mean 5.6 years) with a total of 83 stones of the upper urinary tract were treated by ESWL (Lithostar). Preoperative evaluation included history, physical examination, routine blood tests, urinalysis, urine culture, intravenous urography and optional renal scintigraphy. The impulse rate per treatment varied from 750 to 4,000 (mean 2,996). After acute treatment, routine follow-up included renal ultrasound, blood pressure controls, laboratory tests and eventually plain film X-ray., Results: Successful fragmentation of the stones was achieved in all patients. In 54% the patients were free of stones treated at the time of discharge. At 3 months after treatment radiographic studies showed no residual fragments in 80% of the treated children. 83% of the treated stones were cleared entirely. The remaining fragments were clinically insignificant. An average of 2.5 ESWL treatments per child in general anesthesia were required. Stone analysis showed 20 calcium oxalate, 38 calcium phosphate, 12 struvite, 2 uric acid and 9 cystine calculi. Ureteral stents were placed in 43%. No significant urinary infection was seen under antibiotic prophylaxis. Only 3 children showed a recurrence (1 x cystinuria with low compliance and 2 x struvite). There was no case of renal scarring. No change in renal function or blood pressure was found compared to the preoperative values. Hematuria and proteinuria disappeared in all children who were free of stones. Renal ultrasound revealed no growth difference between treated and untreated renal units., Conclusions: In childhood, ESWL is an efficacious and safe treatment of stones of the upper urinary tract. The long-term follow-up after ESWL with a second-generation lithotriptor did not show any signs of damage to the growing kidney. Sometimes repeated ESWL treatments are justified by the low rate of complications.

Pathogenic aspects of interstitial cystitis (IC) are reviewed on the basis of the current literature applying the criteria of evidence-based medicine (EBM). Three hundred forty-six peer-reviewed publications of the past 30 years were retrieved via MEDLINE employing the key words "interstitial cystitis and pathogenesis or etiology" and classified according to EBM criteria and subject categories. The papers were then reviewed and summarized and are discussed considering the theory of acute and chronic inflammatory responses. In the literature, nearly all steps of the normal inflammatory response are considered pathogenically important in development of the IC syndrome. Applying EBM criteria, all studies fail to meet the criteria for sound statistical evaluation and to demonstrate reproducibly a reliable pathogenic factor. Most publications report only small numbers of patients. Control groups are frequently lacking or too small. Clinically useful criteria for the diagnosis of IC are not properly defined. There is no commonly accepted animal model in which IC occurs naturally or can be artificially induced. Of the 683 IC papers studied, 346 (50.7%) deal with pathogenic or etiological aspects of IC. The inflationary use of the terms pathogenesis or etiology in one out of every two papers calls for more prudent application of terminology in the future. As yet, none of the published pathogenic factors was found to represent the main trigger of the IC syndrome. Some of them seem to be part of a vicious cycle of the inflammatory reaction present. Detailed knowledge of the process of chronic inflammation can lead to treatments that may interrupt an inflammatory response. This might contribute to solving the pathogenic issue of IC and could be helpful in designing further investigations. Advances in understanding the causes of IC require objective criteria to subclassify the heterogenous patient cohort presently referred to as IC syndrome. Such subclassifications are a predisposition for pathogenic investigations and determining future treatment strategies.

Purpose: Bone metastases or local recurrences are widely viewed as poor prognostic signs for successful immunotherapy for metastatic renal cell carcinoma (RCC), and even partial remission is a rarity. We assessed the efficacy of the combination of radio and chemo-immunotherapy for bone metastases or local recurrences form RCC., Materials and Methods: From February 1994 until September 1997 twelve patients with progressive renal cell carcinoma (9 with bone metastases and 3 with local recurrence) were treated with a combination of chemo-immunotherapy and radio therapy., Results: Four out of twelve patients achieved complete remission (CR), one patient had a partial remission (PR), three were stable and four had disease progression under radio therapy and chemo-immunotherapy. Yet three pts. died of the disease. The toxicity symptoms according to WHO ranged between grade 2 and grade 3., Conclusion: Our data suggest that the combination of radio therapy and chemo-immunotherapy may induce a synergistic antitumor effect for the treatment of bone metastases or local recurrences from renal cell carcinoma.

In principle, the fibrosing, preneoplastic and neoplastic morphological reactions to natural and man-made mineral fibres are relatively uniform, and are thus non-specific for the various different types of fibre. In order to establish a causal chain between the injurious "fibrous" agent and the morphologically and clinically manifest pathological lesion, close cooperation between epidemiologists, occupational physicians, pneumologists and pathologists is needed. On its own, the morphological contribution towards solving the problem is of only a limited nature. Light-microscopic, electron-microscopic and element-analytical studies, however, used in conjunction with the results obtained in animal experiments, in the individual case with consideration also being given to aspects of medical insurance, can lead to useful information for the clinical situation and for the generation of expertize.

The intraperitoneal test is a suitable experimental method for studying the different patterns of morphological reaction to foreign body substances of various kinds and concentrations as well as their transport within and elimination from the organism, Kevlar fibres are synthetic aromatic polyamid (aramid) fibres which, investigated by means of the intraperitoneal test in Wistar rats, show distinct pathogenetic reaction patterns: 1. In the early stage after application, the formation of multinucleated giant cells with phagocytosis of the amber-coloured Kevlar fibres, and an inflammatory reaction are foremost features. 2. The typical feature of the second stage is the development of granulomas with central necrosis indicating the cytotoxic nature of Kevlar fibres. 3. The third stage is dominated by the mesenchymal activation with capsular structures of collagenous fibres. Besides granulomatous foci, a slight submesothelial fibrosis is observed. 4. Fragments of Kevlar fibres are drained through lymphatic pathways and stored in lymph nodes where they lead to inflammatory reactions. 5. The reactive granulomatous changes in the greater omentum of rats are accompanied by proliferative mesothelial changes which, in one cases, even led to the development of a multilocular mesothelioma.