VPS13D-based disease: Expansion of the clinical phenotype in two brothers and mutation diversity in the Turkish population.

VPS13D is a recently described gene. Worldwide, only 15 families with 23 affected individuals have been reported with a VPS13D-based disease. Mutated VPS13D causes a complex phenotype with a hyperkinetic movement disorder and ataxia, especially in childhood onset disease. The clinical phenotype of the rare adult-onset cases consists of cerebellar ataxia and/or spastic paraplegia. Here, we report the extensive clinical, laboratory and genetic findings of two offspring from consanguineous parents, with ages of disease onset at 57 and 49 with VPS13D-based ataxia. Although conventional magnetic resonance imaging showed mild cerebellar and cerebral atrophy, diffusion tensor imaging, applied for the first time for VPS13D patients, revealed prominent atrophy in U fibers and cerebellopontine tracts. Whole exome sequencing analysis revealed a biallelic Ala4210Val mutation in the VPS13D, reported only once in the literature. Complementary screening of our in-house database consisting of 295 ataxia and hereditary spastic paraplegia patients revealed two further ataxia patients with novel VPS13D variants. Screening the control cohort for VPS13D variants revealed one asymptomatic individual carrying a novel VPS13D variant. In this study, the phenotypic spectrum of VPS13D-based disease is expanded with the description of pre-senile onset predominant ataxia. Further, with the additional novel mutations described, the report is expected to contribute to the understanding of the yet elusive phenotype-genotype correlations in the rare VPS13D-based movement disorder.

Percutaneous Nephrolithotomy in Children: 17 Years of Experience.

PURPOSE: We analyzed factors that might affect outcome in terms of success and incidence of complications in children up to 17 years after undergoing percutaneous nephrolithotomy. MATERIALS AND METHODS: The data of 346 renal units (294 patients) were analyzed in terms of postoperative outcome. Factors investigated that might affect outcome were patient gender, age, stone laterality, largest stone size, stone burden, number of stones, location of a single stone, previous intervention and instrument size. RESULTS: Mean ± SD patient age was 8.51 ± 4.91 years, and male-to-female ratio was 209:137. Mean ± SD stone burden was 3.49 ± 3.3 cm(2). Stone-free rates after a single procedure were 84.4% and 73.1% in patients with and without clinically insignificant residual stones, respectively. On univariate and multivariate analyses stone burden and number of stones affected the stone-free rate. Complications consisted of bleeding in 41 patients (11.8%), postoperative urinary tract infection in 21 (6%), urosepsis in 4 (0.1%) and hydrothorax in 4 (0.1%). One patient died of multiple organ failure. Through the years blood transfusion and complication rates decreased, and the use of smaller instruments increased significantly. Although bleeding occurred less often in cases where a 14Fr sheath was used rather than a larger sheath (5% vs 12%, p = 0.142), the difference was not significant. No significant factor affecting complication rates was detected. CONCLUSIONS: As in adults, percutaneous nephrolithotomy can be used in children with acceptable complication rates and good success rates for surgical treatment of complex renal stones. Number of stones and stone burden are predictive of postoperative stone-free rate.

A new simple scoring system for prediction of success and complication rates in pediatric percutaneous nephrolithotomy: stone-kidney size score.

INTRODUCTION: Nomograms and scoring systems designed for predicting the success of percutaneous nephrolithotomy (PCNL) in adults are currently available. However, no scoring system currently exists primarily for predicting PCNL success in children. OBJECTIVES: The objective of this study was to develop a scoring system to predict the stone-free and complication rates by using pre-operative parameters. STUDY DESIGN: A retrospective analysis was conducted on data from 434 renal units belonging to patients with kidney stones who underwent PCNL between 1997 and 2017. Renal stone index was calculated by dividing the length of the stone along its longest axis by the length of the kidney along its longest axis. Guy's Stone scores, S.T.O.N.E scores, and CROES scores for adults were calculated by filling in the variables for each patient. Factors that predicted success and complications were examined by univariate and multivariate analyses. RESULTS: Mean age was 8.3 (1-16) years, and male to female ratio was 236:165. When stone-free patients were compared with other patients, there was a statistically significant difference in the average stone/kidney index (SKI) value (stone size/kidney size on longitudinal axis) (0.266 vs 0.339, P < 0.001). In multivariate analysis, factors that predicted success were the SKI and number of stones. A newly developed scoring scale, the stone-kidney score (SKS), combined scores for both the SKI and the number of stones into one value. A minimum total SKS score was 2, and a maximum total SKS score was 4. Success rates for SKS scores of 2, 3, and 4 were 86.4%, 73%, and 62.9% (P < 0.001), respectively. Complication rates for SKS scores of 2, 3, and 4 were 13%, 22.1%, and 23.8%, respectively. DISCUSSION: The new scoring system has only two variables (number of stones and SKI) and three risk groups. An SKS score is easier to use and calculate. An SKI value can be simply calculated on any imaging modality by dividing the length of the stone along its longest axis by the length of the kidney along its longest axis. As in the new scoring system, the SKI value combined with the number of stones is useful in predicting stone-free rates after PCNL. CONCLUSION: When evaluated together, the SKI and presence of multiple stones may predict stone-free rates pre-operatively. The SKS is an individual-specific method that can be easily used in pediatric clinical practice. Further studies are required to develop and standardize this method.

Surgical outcome of patients with vesicoureteral reflux from a single institution in reference to the ESPU guidelines: a retrospective analysis.

INTRODUCTION: Vesicoureteral reflux (VUR) is an anatomic or functional disorder, and it is a condition associated with renal scarring, hypertension, and end-stage renal disease. Renal damage can be prevented by appropriate medical and surgical intervention for selected patients. OBJECTIVES: The objective of this study was to retrospectively analyze the surgically treated patient group of this study in reference to the risk analysis criteria used in European Association of Urology (EAU), European Society for Paediatric Urology (ESPU) guidelines to see the outcome of the study management protocol within the last 15 years in respect to this risk analysis. STUDY DESIGN: A total of 686 patients who were operated upon in a single institution for VUR between 1997 and 2016 were retrospectively analyzed. According to the criteria in EAU/ESPU guidelines, the patients were classified into three groups: low, medium, and high risk. Risk factors were compared between the groups. RESULTS: The patient numbers for low, medium, and high risk were 92 (13.4%), 485 (70.7%), and 109 (15.9%), respectively. In the high-risk group, surgeons tended to do more ureteroneocystostomy (UNC) (82.6%), whereas in the low-risk group, surgeons tended to do more subureteric injection (STING) (76.1%). The success rates for STING and UNC were found to be 75% and 93%, respectively. Although there was a difference in success rates among patients treated with STING or UNC, this difference was not statistically significant in success rates regarding risk groups for patients treated with STING or UNC. DISCUSSION: The most recent guideline was that which was published by the EAU/ESPU organization in 2012. This guideline is established based on the risk analysis. The analysis revealed that patients in the low-risk group tended to undergo endoscopic surgery treatment method, whereas patients in the high-risk group tended to undergo open surgery. Therefore, the study management over the last 10 years has been mainly in line with the current recommendations. CONCLUSION: The analysis shows that when the patients are classified according to the EAU/ESPU risk classification, surgeons tended to perform more endoscopic and more open surgery for the low- and high-risk groups, respectively. Although each surgical modality had similar success rates in each group, open surgical results were overall much higher than those of endoscopic surgery in each group. This was a specifically important finding in high-risk group where the endoscopically treated group of patients was small in number, and the need for a definitive correction is essential in this group because of increased risk of renal injury.

Renal transplantation in children with lower urinary tract dysfunction of different origin: a single-center experience.

INTRODUCTION: Renal transplantation in patients with lower urinary tract dysfunction (LUTD) of various origins is a challenging issue in the field of pediatric transplantation. We report our single-center experience to evaluate patient and graft survivals as well as the risks of the surgery and immunosuppressive therapy. PATIENTS AND METHODS: Among 70 pediatric transplant patients, 11 displayed severe LUTD. Videourodynamic tests were performed on all patients preoperatively as well as postoperatively if required. The cause of urologic disorders were neurogenic bladder (n = 5) and urethral valves (n = 6). Clean intermittent catheterization (CIC) was needed in six patients to empty the bladder. To achieve a low-pressure reservoir with adequate capacity pretransplantation augmentation ileocystoplasty was created in four patients and gastrocystoplasty in one patient. Three of the patients received kidneys from cadaveric and eight from living donors. All patients were treated with calcineurin-based immunosuppressive therapy. RESULTS: The mean age at transplantation was 15 +/- 4.7 years. The median follow-up after transplantation was 36 months (6 to 62 months). At their last visit the median creatinine level was 0.95 mg/dL (0.8 to 2.4 mg/dL). Three patients had recurrent symptomatic urinary tract infections who had augmented bladder on CIC. One patient with ileocystoplasty who developed urinary leak and ureteral stricture in the early postoperative period was treated by an antegrade J stent. CONCLUSION: Severe LUTD carried high risks for the grafted kidney. However, our data suggested that renal transplantation is a safe and effective treatment modality, if the underlying urologic diseases properly managed during the transplantation course. Since surgery and follow-up is more complicated, patient compliance and experience of transplantation team have significant impacts on outcomes.

How does the presence of urologic problems change the outcome of kidney transplantation in the pediatric age group.

PURPOSE: We retrospectively reviewed the impact of functional and anatomic urologic disorders on kidney transplantation outcomes in terms of the surgical and long-term results of pediatric renal transplantation. MATERIALS AND METHODS: Of the 55 kidney transplantations in the pediatric age group, end-stage renal disease (ESRD) was secondary to genitourinary disorders in 23 patients (42%). The urologic abnormalities were vesicoureteral reflux in 13 patients (59%), neurogenic bladder in 4 patients (18%), posterior urethral valves in 3 patients (14%), renal stone disease in 4 patients (18%), bilateral ureterovesical junction obstruction in 3 patients (14%), and unilateral renal agenesis with concomitant contralateral ureteropelvic junction obstruction in 1 patient (4%). RESULTS: Of the 23 patients with urologic problems, 19 (83%) had functioning grafts with a mean follow-up of 49 months (range, 7-120 months). In the other 32 patients, 26 (81%) had functioning grafts with a mean follow-up of 43 months (range, 1-144 months). The graft survival, mean serum creatinine, and urinary tract infection rates of the patients did not differ between the two groups. CONCLUSIONS: The presence of functional urologic disorders as the cause of ESRD did not seem to change the outcome of renal transplantation in terms of graft survival when compared with patients without any urologic disorders. Urinary tract infections seem to be a little more common and yet clinically not significant in those patients. Reflux does not always need to be corrected before transplantation, unless it is causing symptoms or infection.

Renal transplantation in children with augmentation enterocystoplasty.

INTRODUCTION: We report our experience with renal transplantation in patients with severe bladder dysfunction who underwent prior augmentation cystoplasty. PATIENTS AND METHODS: Among 58 pediatric patients, three underwent bladder augmentation prior to renal transplantation. The patients' ages at transplantation were 10, 13, and 17. The etiologies of bladder dysfunction were posterior urethral valves in two patients and contracted bladder in one patient. Vesicoureteral reflux was concomitantly present in three patients. Pretransplant ileocystoplasty was created in two patients and gastrocystoplasty in one patient. All patients received kidneys from cadaveric donors and were treated with calcineurin-based immunosuppressive therapy. RESULTS: The patients had normal renal function without hydronephrosis of the transplanted kidney at 13, 22, 49 months follow-up. No patients had morbidity due to technical complications. All the patients were continent. Two of three patients required clean intermittent catheterization from a Mitrofanoff conduit, while one patient spontaneously voids without significant residual urine. Urinary tract infections observed in two patients were successfully treated without any permanent deterioration in graft kidney function. CONCLUSIONS: Our data suggest that augmentation cystoplasty is a safe and effective option to treat patients with end-stage renal disease undergoing kidney transplantation. Experience of the transplantation team with a qualified pediatric urologist is essential due to the potentially high risk of surgical complications during the long term management of these patients.

Postvoidal residual urine is the most significant non-invasive diagnostic test to predict the treatment outcome in children with non-neurogenic lower urinary tract dysfunction.

INTRODUCTION: Uroflowmetry (UF) alone is often inadequate or unreliable to diagnose lower urinary tract dysfunction (LUTD). Therefore, other non-invasive tests, such as ultrasound (US), post-voiding residual volume (PVR) assessment and symptom scales, are used as well for objective definition of the problem. OBJECTIVE: The aim of this study was to investigate the possible predictive function of the non-invasive diagnostic tests for the response to treatment. STUDY DESIGN: The prospective registry data of 240 patients with LUTD, from November 2006 to September 2013, were retrospectively analyzed. All patients were aged 5-14 years old. Patients with a previous diagnosis of vesicoureteral reflux (VUR), neurogenic bladder, monosymptomatic nocturnal enuresis (NE) were excluded from the study. Uroflowmetry, US, PVR and the Dysfunctional Voiding and Incontinence Symptom Scale (DVISS) were performed on every patient at their first visit and follow-ups. A DVISS <9 was considered as the DVISS response; parental opinion was based on International Continence Society criteria of clinical response. Time passed until clinical response was the last outcome parameter. RESULTS: Mean age was 8.2 years. Median follow-up was 60.5 months. A total of 62% of patients had complete response, 28.1% had partial response, and 9.7% had no response. Demographic variables were not associated with clinical outcome. Co-existing enuresis nocturna, multiple pharmacotherapy, and increased DVISS were associated with longer time until clinical response. Post-voiding residual volume assessment was the only test to have a prognostic value. DISCUSSION: Resolution rates of LUTD ranged from 40 to 90%. High resolution rate could be attributed to the long follow-up period, and the chance of spontaneous resolution. Treatment modalities and co-existing NE were associated with longer time until clinical response. Only PVR was associated with prognosis. This was the first study in literature to report such findings. It was seen that the normalization of pathologic patterns was a good sign for treatment success. The DVISS results showed significantly higher rates of incontinence compared to initial symptoms defined by the patients and/or their parents. This showed the importance of using scoring systems to better define the severity of symptoms. It was hard to establish a standardized cut-off value for bladder wall thickness on US. However, US was a good test for diagnosing additional pathologies. CONCLUSION: Increased PVR was the single tool that was associated with prognosis and, therefore, should always be performed after UF. In addition, DVISS can help parents be counseled about their treatment expectations.

Does lower urinary tract status affect renal transplantation outcomes in children?

BACKGROUND: Lower urinary tract dysfunction (LUTD), an important cause of end stage renal disease (ESRD) in children, can adversely affect renal graft survival. We compared renal transplant patients with LUTD as primary renal disease to those without LUTD. METHODS: The data of 60 children who underwent renal transplantation (RTx) between 2000 and 2012 were retrospectively reviewed. All patients with LUTD were evaluated with urodynamic tests preoperatively; 15 patients required clean intermittent catheterization and 9 patients underwent augmentation cystoplasty before RTx. RESULTS: There were 25 children with LUTD. The mean follow-up for LUTD (+) and LUTD (-) groups were 63 (22-155) and 101 months (14-124), and graft survival were 76% for LUTD (+) and 80% for LUTD (-), respectively (P = .711). On the other hand, creatinine levels at last follow-up were significantly higher in the LUTD (+) group (1.3 ± 0.3 mg/dL vs 0.96 ± 0.57 mg/dL, P < .001). Infectious complications and postoperative urinary tract infection incidences were also higher in the LUTD (+) group (68% vs 25.7%, P = .002 and 60% vs 11.4%, P < .01). CONCLUSION: UTI is significantly higher after kidney transplantation in patients with LUTD. Despite the higher risk of UTI, renal transplantation can be performed safely in those patients with careful patient selection, preoperative management, and close postoperative follow-up. Restoration of good bladder function is the key factor in the success of kidney transplantation in those patients.

Clinical significance of serum ferritin in patients with renal cell carcinoma.

OBJECTIVES: We examined the serum ferritin levels in 158 patients with renal cell carcinoma and 101 healthy control subjects between 1987 and 1994 to investigate the value of this intracellular protein as a tumor marker. METHODS: Preoperative and postoperative serum ferritin values were analyzed and the patients were stratified to three groups accordingly: group 1, patients with normal values (N-N); group 2, those with preoperative high and postoperative normal (H-N); and group 3, those with preoperative normal or high with postoperative high ferritin levels (H-H). RESULTS: The mean serum ferritin level in 101 healthy control subjects was 85.7 +/- 63.6 ng/mL (range, 3.7 to 265.2). The upper limit of normal, which was calculated by adding 2 standard deviations to the mean was 219.9 ng/mL. Mean serum ferritin in patients with renal cell carcinoma was 274.2 +/- 276.3 ng/mL, which was significantly higher than that of control values (P < 0.01). The sensitivity, specificity, and overall accuracy rate for ferritin increase was 94%, 50%, and 61%, respectively. Multivariate analysis showed that the aforementioned grouping and stage of the disease were the two independent prognostic parameters. Preoperative ferritin levels lost its significance on multivariate analysis. CONCLUSIONS: Our study shows that although serum ferritin was a useful tool in diagnosing and staging patients, it was not ideal in early stages. However serum ferritin seems to be more valuable for follow-up; postoperative values, indeed, predict the prognosis.

Postchemotherapeutic surgery for metastatic testicular germ cell tumors: results of extended primary chemotherapy and limited surgery.

OBJECTIVE: Postchemotherapy surgery has become an increasingly important treatment for residual masses in germ cell tumors of the testis. However, it is still a challenge to find the optimal combination of chemotherapy and surgery for better survival and cure rates with lowest morbidity. This study evaluated the effectiveness of extended chemotherapy followed by surgery resecting only the residual masses. METHODS: After an extended course (one or two additional courses after there is no decrease in tumor size and/or after the normalization of tumor markers) of combination chemotherapies with cisplatin-based regimens, 32 patients underwent surgery for metastatic germ cell tumors of the testis. Complete excision of radiologically determined residual masses and macroscopically suspicious neighboring nodes was performed rather than a conventional retroperitoneal lymph node dissection. RESULTS: Histopathologic examination of the resected specimens revealed teratoma in 17 (55%), fibrosis and/or necrosis in 9 (26.5%), and active residual tumor in 8 (23.5%) of the patients. The patients with residual tumor have been treated with additional chemotherapy. In the follow-up (mean, 28.5 months) 4 patients have relapsed, and 1 died. None of the patients with residual teratomas have shown relapse. Only 1 of the 32 patients has had retrograde ejaculation. CONCLUSIONS: A more conservative approach, such as excision of the residual masses after an extended course of chemotherapy, has given excellent results both in the outcome of the patients in the follow-up and in the rate of retrograde ejaculation. We therefore suggest that this approach would be a good alternative to nerve-sparing surgery following chemotherapy.

Cellular congenital mesoblastic nephroma with contralateral medullary nephrocalcinosis.

Congenital mesoblastic nephroma is the most common renal mass in the newborn period and can present with atypical findings. Certain associated conditions such as hypercalcaemia, hypertension and reninism have been described. We report a cellular variant of congenital mesoblastic nephroma with hypercalcaemia and contralateral medullary nephrocalcinosis.

Results of extracorporeal shock wave lithotripsy in children, using the Dornier MPL 9000 lithotriptor.

PURPOSE: The objective of the report is to review extracorporeal shock wave litotripsy (ESWL) results in the pediatric age group treated with Dornier MPL 9000. METHODS: Records of 59 pediatric patients undergoing ESWL using Dornier MPL 9000 lithotriptor for upper urinary tract stones between October 1991 and September 1995 were reviewed. RESULTS: Seventy-one percent (42 of 59) of patients were completely stone free at 3 month follow-up, and 15% of the patients had partial stone clearance. Of 59, 13 had undergone ESWL for residual stone after open surgery. The mean stone size for all the patients was 14.5 mm (5 to 35 mm). CONCLUSIONS: ESWL is an effective method for the treatment of upper urinary tract stones in the pediatric age group. It can be used as the first line treatment in patients without previous surgery and patients with residual stones after open surgery. ESWL is an effective treatment for the upper urinary stones (up to 3.5 cm) in pediatric patients with a complete stone-free rate of 71% and partial clearance of stones in an additional 15%. We suggest that it should be used as the first line treatment in the pediatric age group.

Ureterocystoplasty: an alternative reconstructive procedure to enterocystoplasty in suitable cases.

PURPOSE: The objective of the report is to present the results of ureterocystoplasty in 6 children with megaureters and low-capacity, high-pressure bladders. METHODS: Of the 6 patients, 2 had valve bladders, 1 had Hinmann's syndrome, 1 had neuropathic bladder, and the remaining 2 with ureterocutaneostomy were mainly diverted because of refluxing megaureters. Nephrectomy was performed in both of the boys with posterior urethral valve because of vesicoureteral reflux dysplasia (VURD) syndrome, and the ipsilateral ureter was used for the augmentation. In 2 patients with ureterocutaneostomy and in 1 with Hinmann's syndrome, a transureteroureterostomy was carried out, and the distal part of the ureter was used to perform augmentation. The patient with neuropathic bladder had a nonfunctioning crossed ectopic left kidney with an associated ipsilateral, refluxing megaureter, and the ureter was used for augmentation after the nephrectomy. RESULTS: All of the patients are continent, and 4 patients who are neurologically normal void spontaneously without requiring clean intermittent catheterization (CIC). The average increase in bladder capacity is 263% (range, 190% to 340%). CONCLUSIONS: Ureterocystoplasty is the bladder augmentation of choice for patients with a nonfunctioning kidney with an associated ipsilateral, refluxing megaureter and for patients with kidneys both in good function and megaureters suitable for a transureteroureterostomy.

Percutaneous nephrolithotomy in older children.

PURPOSE: The aim of this study was to evaluate the safety and efficacy of percutaneous nephrolithotomy (PCNL) in pediatric patients, older than 8 years, without any technical and instrumental modifications. METHODS: The authors retrospectively evaluated the data of 16 percutaneously treated kidneys of 14 patients with a mean age of 11 years (range, 8 to 17). The mean stone burden was calculated as 301 mm2 (range, 120-750). Percutaneous accesses were done under fluoroscopic control, and 24F to 30F Amplatz dilators were used depending on patient size. Ultrasonic and laser lithotripsy were performed to fragment calculi. RESULTS: Mean operating time was 111 minutes (range, 60 to 210 min) and no intraoperative or postoperative major complication was observed. Mean hemoglobin drop after the procedure was 1.16 g/dL (range, 0.3 to 2.8). Blood transfusion was required in only 1 patient, and mean postoperative hospitalization was 4.6 days (range, 3 to 10). Stone-free rate was calculated as 69% (11 of 16), but with residual fragments smaller than 4 mm, the success rate becomes 100% at patient's discharge. CONCLUSIONS: PCNL is an effective and safe form of therapy in pediatric stone disease. Especially in older children the use of the same instruments and technique as in adults may achieve equal results without any increased risk of possible morbidity and need of blood transfusion.

Surveillance-only policy in clinical stage-I non-seminomatous germ-cell tumors of the testis.

With the advanced imaging techniques, the sensitive assays for tumor markers and the curability of small-volume metastatic disease with cis-platinum-based chemotherapy, surveillance has gained popularity in clinical stage-I non-seminomatous germ-cell tumors. This study reports our experience on 58 patients who have been included in a surveillance protocol. Patients with normal tumor-marker levels following surgery, no evidence of metastases on the CT scans of the abdomen and the chest and no residual tumor at the surgical margin were followed. Relapsing patients were treated with cis-platinum-based combination chemotherapy. Of the 58 patients, 17 relapsed (29.3%) in a period of 2-18 months (median 5 months). None of the relapsing patients later presented with evidence of disease in the follow-up period 14-79 months (median 39 months) after chemotherapy. Prognostic factors were evaluated by univariate analysis and the data for risk factors, such as the presence of embryonal carcinoma, absence of yolk-sac elements and scrotal violation, were evaluated by multivariate analysis. Statistical analysis showed that none of the risk factors were significantly different in predicting the relapse. Of the 6 relapsing patients with preorchiectomy elevated tumor-marker levels, 4 had shown a slower decline in tumor-marker levels than expected and they all relapsed with elevated levels of the same tumor markers. A surveillance-only policy actually seems to be a safe and logical approach if the patients are properly selected and cooperate fully.

Long-term treatment of nocturnal enuresis with desmopressin intranasal spray.

The purpose of this study was to determine the efficacy and safety of long-term treatment of nocturnal enuresis with desmopressin intranasal spray. Sixty-five children with primary nocturnal enuresis with a mean age of 11.3 years (range 7-17) underwent a 2-week observation period followed by dose titration period of 1 week. Those children completely dry with desmopressin entered a randomized, placebo-controlled, double-blind phase lasting 2 weeks, followed by a 6-month open treatment. The enuretic status of the children was documented for 2 weeks after the treatment was stopped. Eleven children had no change from baseline wetting with desmopressin. Thirty-two children receiving 20 mg and 9 children with 40 mg desmopressin were completely dry. Thirteen children were wet 1-2 nights per week, which was better than in the pretreatment period. During the 6-month open-treatment period, the effect of desmopressin was found to be stable. No side effects or adverse reactions were encountered. Two weeks after the treatment was stopped, 25 children were still completely dry (38% of the initial study population, 50% of the responders). The cure rate appeared to continue beyond 18 months after discontinuation of the treatment.

Sequential bilateral germ cell tumours of the testis.

Bilateral germ cell tumours of the testis are rare but a rise in their incidence is expected since with the new therapeutic possibilities a significant improvement in prognosis has been achieved even in patients with advanced metastatic spread. Of the 210 patients treated for malignant germ cell tumours at our Department, six (2.9%) developed a contralateral testicular tumour. All patients had metachronous tumours and the second tumours occurred after an interval ranging between 1 and 22 years. The epidemiology, histology, diagnosis, therapy and prognosis are discussed, and the significance of regular self-examination of the remaining testis in patients with testicular tumour is emphasized.

How significant are serial bone scans in monitoring advanced prostatic cancer?

We report a total of 169 serial bone scan studies conducted in 21 patients with histologically proven metastatic cancer of the prostate. Aim of the study was to investigate the concordance of findings on bone scans with serum acid phosphate (AP) levels and the clinical performance status (CPS) of the patients, and to see how important bone scan is by itself in determining the metastatic progression in the follow-up. Eighty-seven and 86% of scans demonstrated changes concordant with AP and CPS levels subsequently. It was also found that 100% of the progressions on bone scans along with elevated levels of AP had been confirmed as metastatic progression, whereas only 41% of progressions on bone scans solely had been shown to be metastases in the follow-up investigations. Findings on bone scans not in correlation with clinical findings and serum AP levels are mostly misleading. Use of bone scans in conjunction with serum AP levels and most probably with prostate-specific antigen and CPS is the most reliable and therefore treatment modality changes should not be based on bone scans only.