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Item does not contain fulltext, Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders affecting cortisol biosynthesis. Reduced activity of an enzyme required for cortisol production leads to chronic overstimulation of the adrenal cortex and accumulation of precursors proximal to the blocked enzymatic step. The most common form of CAH is caused by steroid 21- hydroxylase deficiency due to mutations in CYP21A2. Since the last publication summarizing CAH in Endocrine Reviews in 2000 there have been numerous new developments. These include more detailed understanding of steroidogenic pathways, refinements in neonatal screening, improved diagnostic measurements utilizing chromatography and mass spectrometry coupled with steroid profiling, and improved genotyping methods. Clinical trials of alternative medications and modes of delivery have been recently completed or are under way. Genetic and cell-based treatments are being explored. A large body of data concerning long-term outcomes in patients affected by CAH, including psychosexual well-being, has been enhanced by the establishment of disease registries. This review provides the reader with current insights in congenital adrenal hyperplasia with special attention to these new developments.

Contains fulltext : 253193.pdf (Publisher’s version ) (Open Access)

Contains fulltext : 282361.pdf (Publisher’s version ) (Open Access), Klinefelter syndrome (KS) is associated with an increased risk of neuropsychological morbidity, such as learning disabilities, which may have a significant impact on socioeconomic status (SES). The objective of this study was to investigate the SES in men with KS and to associate this outcome with social participation, age at diagnosis, testosterone therapy and physical and mental health status. Men with KS were recruited in 14 clinical study centers in six European countries which participated in the European dsd-LIFE study. Two hundred five men with KS were eligible for inclusion. Male normative data from the European Social Surveys (ESS) were used for comparison. Data related to education, occupation, satisfaction with income and householding were collected. Compared to the ESS reference population, fewer men with KS achieved a high level of education (13% vs 25%, P < 0.001). There was a significant difference in having a paid job (55% vs 66%, P < 0.001), and the percentage of absence by sickness or disability was higher among men with KS (10% vs 3%, P < 0.001). Furthermore, satisfaction with current household's income was lower (32% vs 42%, P < 0.01). Lower scores for subjective general health were associated with lower scores for these outcomes. Men with KS achieve on average lower levels of education, occupation and report less satisfaction with income compared to the ESS reference population. The presence of health problems and lower scores of subjective general health was related to lower levels of occupation and lower satisfaction with income in men with KS.

Contains fulltext : 240992.pdf (Publisher’s version ) (Closed access)

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Item does not contain fulltext, PURPOSE: To analyze agreement on postoperative complications after hypospadias surgery according to medical records and parents' reports. MATERIALS & METHODS: In this retrospective cohort study, data were collected from 409 children who received an initial one-stage hypospadias correction in the Radboudumc, The Netherlands. Postoperative complications according to medical records were compared with parent-reported complications in an online questionnaire. Main complications studied were wound-related complications, urinary tract infections, fistulas, stenosis, and prepuce-related complications. Agreement was determined by Cohen's kappa coefficient. RESULTS: Slightly less complications were mentioned in medical records (37%) compared to parents' reports (42%). Overall agreement was moderate (kappa=0.50, 95% confidence interval (CI):0.41-0.59), but poor for some specific complications. Agreement was higher for complications that needed reoperation compared to when no reoperation was performed (kappa=0.53, 95% CI: 0.43-0.62 and kappa=0.18, 95% CI: 0.06-0.31) and for patients with recent surgery (<5years before questionnaire completion) compared to less recent surgeries (kappa=0.69, 95% CI: 0.55-0.84 and kappa=0.43, 95% CI: 0.33-0.54). CONCLUSIONS: Agreement on complications according to medical records and parents' reports was poor to moderate, but better after reoperation and more recent surgery. Some complications mentioned in medical records were missing from parents' reports and the other way around. Better agreement will give physicians and parents a more reliable view on postoperative outcome after hypospadias surgery. TYPE OF STUDY: Diagnostic test. LEVEL OF EVIDENCE: Level III.

Contains fulltext : 193189.pdf (publisher's version ) (Closed access)

Contains fulltext : 193650.pdf (publisher's version ) (Open Access), Repair of long ureteral defects often requires long graft tissues and extensive surgery. This is associated with complications, including a lack of suitable tissue and graft site morbidity. Tissue engineering may provide an attractive alternative to the autologous graft tissues. In this study, ureteral repair using (preimplanted) tubular collagen-Vicryl templates was evaluated in a new goat model. Tubular templates were prepared from tubularized Vicryl meshes and 0.7% type-I collagen (length = 6 cm, inner diameter = 6 mm, wall thickness = 3 mm). In total, twelve goats were used and evaluated after 3 months. Eight goats were implanted with the collagen-Vicryl templates and in four goats the templates were first preimplanted in the subcutis and subsequently used as ureteral graft. Template implantation was successful in 92% of the goats(11/12). During follow-up, 82% of the animals (9/11) survived without signs of discomfort. Two animals were sacrificed prematurely due to kidney perforation by the stent and urine leakage. Two other animals presented with stenosis of the neoureter due to stent migration. After preimplantation, the templates were remodeled mostly to autologous tissue with similar mechanical characteristics as the native ureter. Goats grafted with preimplanted templates presented with predominantly healthy kidneys, whereas the goats grafted with the collagen-Vicryl templates presented with fibrotic and inflamed regions in the kidneys. The use of preimplanted tissue templates showed favorable results compared with direct functional implantation of the templates. Partial remodeling toward autologous tissue and similar mechanical characteristics likely improved the integration in the ureteral tissue. Preimplantation of tissue-engineered templates should therefore be considered when two-stage procedures using a nephrostomy catheter are indicated or when planning allows for additional time to treatment.

Contains fulltext : 195631.pdf (Publisher’s version ) (Open Access)

Contains fulltext : 178252.pdf (publisher's version ) (Open Access), It is common to test medical devices in large animal studies that are or could also be used in humans. In this short report we describe the use of a ureteral J-stent for the evaluation of biodegradable tubular constructs for tissue reconstruction, and the regeneration of ureters in Saanen goats. Similarly to a previous study in pigs, the ureteral J-stent was blindly inserted until some resistance was met. During evaluation of the goats after three months, perforation of the renal cortex by the stent was observed in four out of seven animals. These results indicated that blind stent placement was not possible in goats. In four new goats, clinical protocols were followed using X-ray and iodinated contrast fluids to visualize the kidney and stent during stent placement. With this adaptation the stents were successfully placed in the kidneys of these four new goats with minimal additional effort. It is likely that other groups in other fields ran into similar problems that could have been avoided by following clinical protocols. Therefore, we would like to stress the importance of following clinical protocols when using medical devices in animals to prevent unnecessary suffering and to reduce the number of animals needed.

Radboud Universiteit Nijmegen, 28 januari 2016, Promotor : Feitz, W.F.J. Co-promotores : Kortmann, B.B.M., Heesakkers, J.P.F.A., Contains fulltext : 150236.pdf (publisher's version ) (Open Access)

Item does not contain fulltext, PURPOSE: A readily available artificial urinary conduit might be substituted for autologous bowel in standard urinary diversions and minimize bowel associated complications. However, the use of large constructs remains challenging as host cellular ingrowth and/or vascularization is limited. We investigated large, reinforced, collagen based tubular constructs in a urinary diversion porcine model and compared subcutaneously pre-implanted constructs to cell seeded and basic constructs. MATERIALS AND METHODS: Reinforced tubular constructs were prepared from type I collagen and biodegradable Vicryl(R) meshes through standard freezing, lyophilization and cross-linking techniques. Artificial urinary conduits were created in 17 female Landrace pigs, including 7 with a basic untreated construct, 5 with a construct seeded with autologous urothelial and smooth muscle cells, and 5 with a free graft formed by subcutaneous pre-implantation of a basic construct. All pigs were evaluated after 1 month. RESULTS: The survival rate was 94%. At evaluation 1 basic and 1 cell seeded conduit were occluded. Urinary flow was maintained in all conduits created with pre-implanted constructs. Pre-implantation of the basic construct resulted in a vascularized tissue tube, which could be used as a free graft to create an artificial conduit. The outcome was favorable compared to that of the other conduits. Urinary drainage was better, hydroureteronephrosis was limited and tissue regeneration was improved. CONCLUSIONS: Subcutaneous pre-implantation of a basic reinforced tubular construct resulted in a vascularized autologous tube, which may potentially replace bowel in standard urinary diversions. To our knowledge we introduce a straightforward 2-step procedure to create artificial urinary conduits in a large animal model.

Item does not contain fulltext, OBJECTIVE: To compare the regenerative capacity of diseased bladder in a large animal model of bladder exstrophy with regeneration in healthy bladder using a highly porous collagen scaffold. MATERIALS AND METHODS: Highly porous bovine type I collagen scaffolds with a diameter of 32 mm were prepared. In 12 fetal sheep a bladder exstrophy was surgically created at 79 days' gestation. Lambs were born at full term (140 days' gestation). After 1 week the bladder lesion was reconstructed and augmented with a collagen scaffold (group 1). In nine normal newborn lambs the bladder was augmented with a collagen scaffold 1 week after birth (group 2). Functional (video-urodynamics) and histological evaluation was performed at 1 and 6 months after surgery. RESULTS: The survival rate was 58% in group 1 and 100% in group 2. Cystograms were normal in all lambs, besides low-grade reflux in both groups. Urodynamics showed comparable capacity between both groups and a trend to lower compliance in group 1. Histological evaluation at 1 month revealed a non-confluent urothelial layer, an immature submucosa, and initial ingrowth of smooth muscle cells. At 6 months both groups showed normal urothelial lining, standard extracellular matrix development, and smooth muscle cell ingrowth. CONCLUSIONS: Bladder tissue regeneration with a collagen scaffold in a diseased bladder model and in healthy bladder resulted in comparable functional and histological outcome, with a good quality of regenerated tissue involving all tissue layers. Improvements may still be needed for larger augmentations or more severely diseased bladders.

Item does not contain fulltext, PURPOSE: To evaluate treatment effectiveness for children with enuresis, according to the definitions of the International Children's Continence Society (ICCS, 2006). MATERIAL AND METHODS: Children >/=6 years of age followed a 4-month outpatient treatment consisted of a visit during which history regarding enuresis was taken, causes were explained and therapeutic tips & tricks were discussed. All children received a booklet about enuresis and were trained with an alarm and/or pharmacological therapy. At baseline, 4, 10 and 16 months, the number of wet nights during the previous 28 days and the use of medication were assessed. Success of treatment was determined using ICCS definitions of treatment outcome. RESULTS: 66 children with enuresis were included (48 boys/18 girls) in this retrospective study. Mean age: 11(+/-2.6) years. 91%(n = 60) of the children had non-monosymptomatic enuresis. Results at 4 months: 46% full, 15% good, 21% partial response (n = 66). At 10 months: 55% full, 4% good, 29% partial response (n = 49). At 16 months: 53% full, 6% good, 25% partial response (n = 34). Overall, use of pharmacological therapy showed a decline in time. CONCLUSION: According to the ICCS definitions, outpatient treatment for enuresis shows a good overall treatment response, and these results can be used to compare with other studies in the future.

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Item does not contain fulltext, OBJECTIVE: Transfers to adult care can be problematic, resulting in postponement due to the protective nature of pediatric care and patient dependency. It is unknown whether these findings apply specifically to urology patients. Our department is taking part in a national general transition project. In this light, our aim was to investigate the specific needs of adolescent urologic patients, regarding their independence and transition. PATIENTS AND METHODS: 80 patients, born in 1975-1998, with a chronic bladder condition received a questionnaire. They were divided into pre- and post-transfer groups. Parents (n = 7) of post-transfer patients formed a third group. Questionnaires were based on those used in the national transition study, supplemented with urological questions. Pre-transfer patients were asked about their level of independence, what subjects were discussed during consultations, and their expectations and wishes regarding transfer. Post-transfer patients and parents were asked for their opinions on the transfer process. RESULTS: 73% (n = 58) responded (55 pre-transfer and 3 post-transfer patients plus parents). It appeared that the confidence built-up with the pediatric urologist impeded the transfer. An adequate level of disease-related knowledge was reported. Relationships, sexuality and fertility were hardly talked about (respectively n = 17, 16 and 18). Parents played an important role, which patients appreciated, confirming their dependency. Despite the 49% (n = 27) who stated they can arrange their urological care themselves, 44% (n = 24) felt ill-prepared for transfer. CONCLUSION: Although overall self-perceived knowledge is sufficient, the trust in and personal relationship with the pediatric urologist formed the greatest obstruction to successful transition. These findings have been used to improve support during transition by creating a transition protocol.

1 augustus 2012, Item does not contain fulltext, PURPOSE: The ileal conduit has been considered the gold standard urinary diversion for patients with bladder cancer and pediatric patients. Complications are mainly related to the use of gastrointestinal tissue. Tissue engineering may be the technical platform on which to develop alternatives to gastrointestinal tissue. We developed a collagen-polymer conduit and evaluated its applicability for urinary diversion in pigs. MATERIALS AND METHODS: Tubular constructs 12 cm long and 15 mm in diameter were prepared from bovine type I collagen and Vypro(R) II synthetic polymer mesh. Characterized tubes were sterilized, seeded with and without primary porcine bladder urothelial cells, and implanted as an incontinent urostomy using the right ureter in 10 female Landrace pigs. At 1 month the newly formed tissue structure was functionally and microscopically evaluated by loopogram and immunohistochemistry, respectively. RESULTS: The survival rate was 80% with 1 related and 1 unrelated death. By 1 month the collagen was resorbed and a retroperitoneal tunnel had formed that withstood 40 cm H(2)O water pressure. In 5 cases the tunnel functioned as a urostomy. Histological analysis revealed a moderate immune response, neovascularization and urothelial cells in the construct lumen. The polymer mesh provoked fibroblast deposition and tissue contraction. No major differences were observed between cellular and acellular constructs. CONCLUSIONS: After implanting the tubular constructs a retroperitoneal tunnel was formed that functioned as a urinary conduit in most cases. Improved large tubular scaffolds may generate alternatives to gastrointestinal tissue for urinary diversion.

Contains fulltext : 97495.pdf (publisher's version ) (Closed access), INTRODUCTION: Urological anomalies are frequently seen in patients with anorectal malformations (ARM) and can result in upper urinary tract deterioration. Whether the current method of screening is valid, adequate and needed for all patients is not clear. We, therefore, evaluated the urological screening methods in our ARM patients for changes in urological treatment, outcome and follow-up. METHODS: The medical records of 331 children born with an ARM in the period 1983-2003 were retrospectively studied. Documentation of diagnosis, screening method, urological anomalies, treatment, complications, follow-up and outcome were measured. Results : The overall incidence of urological anomalies was 52%. The incidence of urological anomalies and urological follow-up time decreased with diminishing complexity of the ARM. Hydronephrosis, vesico-urethral reflux, lower urinary tract dysfunction and urinary incontinence were encountered most. Treatment invasiveness increased with the increase of complexity of an ARM. Lower urinary tract dysfunction needing urological care occurred in 43% in combination with lumbosacral or spinal cord anomalies and in 8% with no abnormalities in the lumbosacral-/spinal region. CONCLUSIONS: Urological anomalies in patients with complex ARM are more severe than in patients with less complex ARM. Ultrasonography of the urinary tract should be performed in all patients. Voiding cysto-urethrography can be reserved for patients with dilated upper urinary tracts, urinary tract infections or lumbosacral and spinal abnormalities. All patients with complex ARM need urodynamic investigations. When using these indications, the screening for urological anomalies in ARM patients can be optimized with long-term follow-up in selected patients.

Contains fulltext : 80574.pdf (publisher's version ) (Closed access)

Contains fulltext : 59316.pdf (publisher's version ) (Closed access), OBJECTIVES: To establish whether routine evaluation of light microscopic changes in bladder biopsies in men with graded bladder outlet obstruction correlates with urodynamic parameters and to evaluate the performance of two pathologists in bladder biopsy interpretation. METHODS: In 63 consecutive patients presenting with lower urinary tract symptoms suggestive of bladder outlet obstruction, the workup included bladder biopsies, which were semiquantitatively and independently evaluated by two pathologists for fibrosis, elastosis, muscle cell degeneration, inflammation, and additional remarkable findings. Furthermore, as determined by their general impression, the pathologists gave a diagnosis of normal or graded abnormality. The pathologists' performance was compared and the clinical and urodynamic data were correlated to the consensus histologic diagnoses. RESULTS: Fifty-four of 63 cases could be evaluated. The intraobserver and interobserver reproducibility were reasonable, with a kappa of 0.35 and 0.50, respectively. The consensus diagnosis of abnormal bladder biopsy correlated with impaired bladder compliance and capacity. However, no correlation was found between the presence and severity of histologic abnormalities and Schafer class, urethral resistance factor, instability, postvoid residual urine volume, symptom score, and prostate volume. CONCLUSIONS: Although abnormal bladder biopsies correlated significantly with impaired bladder capacity and compliance, routine evaluation of bladder biopsies is not indicated in the evaluation of bladder outlet obstruction.

Contains fulltext : 57159.pdf (publisher's version ) (Closed access), HYPOTHESIS: Surgeons' preferences for any of 3 methods of inguinal hernia repair are comparable in terms of operating time, incision length, perceived difficulty, and surgeon's satisfaction. DESIGN: Randomized patient-blinded study. SETTING: Teaching hospital. PATIENTS: A total of 334 patients randomized to receive 1 of the 3 repairs. INTERVENTIONS: Patients underwent hernia repair with the Prolene Hernia System, mesh plug repair, or Lichtenstein technique. MAIN OUTCOME MEASURES: Operating variables, surgeon's rating of satisfaction and difficulty, grade and experience of the operating team, and complications. RESULTS: The Lichtenstein technique took significantly the longest operating time (52 minutes vs 41 or 42 minutes; P<.001). The mesh plug repair scored the best results in difficulty and satisfaction. Overall, surgeons having performed more than 5 procedures rated the repairs less difficult and with significantly more satisfaction (P<.001 and P = .001, respectively). The complication rate did not differ between the treatment groups. None of the operative findings was correlated to the outcome, except for adverse correlation with the body mass index. CONCLUSION: From a surgeon's point of view, the mesh plug repair is superior to the Lichtenstein technique and the Prolene Hernia System in terms of operating time, incision length, perceived difficulty, and surgeon's satisfaction.

Item does not contain fulltext, PURPOSE: The efficacy of alpha-adrenoceptor blockers for the treatment of lower urinary tract symptoms has been proven in numerous studies. However, little is known about the efficacy of the longer term. We investigated the long-term risk of re-treatment in patients using alpha-adrenoceptor blockers for lower urinary tract symptoms and the parameters that influence this risk. MATERIALS AND METHODS: We reviewed the files of 316 patients with lower urinary tract symptoms treated at our department with the alpha-blockers terazosin, alfuzosin or tamsulosin. Using followup data up to 3 years, we calculated re-treatment percentages in each treatment group. Using extended followup of 5 years, we calculated the predictive value of various baseline characteristics for re-treatment. RESULTS: The re-treatment rates were 27% for tamsulosin, 37% for alfuzosin and 49% for terazosin. The re-treatment rates of patients with mild, moderate and severe lower urinary tract symptoms were 27%, 33% and 70%, respectively. Patients with a maximum urine flow of less or more than 10 ml. per second had a re-treatment rate of 58% and 47%, respectively. Patients with a prostate volume of less or more than 40 ml. had a re-treatment rate of 48% and 72%, respectively. Patients who were urodynamically unobstructed versus obstructed patients had a re-treatment rate of 44% and 59%, respectively. CONCLUSIONS: Patients given alpha-blockers for lower urinary tract symptoms have a high risk of re-treatment. Tamsulosin has a markedly lower re-treatment percentage than alfuzosin and terazosin. Severe symptoms, poor urine flow, an enlarged prostate and urodynamically proven bladder outlet obstruction increase the risk of treatment failure. Preselection of the most suitable candidates for alpha-blockade may reduce this risk.

Item does not contain fulltext, PURPOSE: We evaluate the durable effect of high-energy transurethral microwave thermotherapy and transurethral prostatic resection for treatment of patients with lower urinary tract symptoms suggestive of bladder outflow obstruction. MATERIALS AND METHODS: Between January 1996 and March 1997, 155 patients with lower urinary tract symptoms suggestive of bladder outflow obstruction were randomized to receive transurethral microwave thermotherapy (Prostatron*; device and commercial software) (82) or undergo transurethral prostatic resection (73). Initial patient evaluation was performed according to international standards. Patients were followed annually with the International Prostate Symptom Score (I-PSS) and uroflowmetry (maximum flow rate). The Kaplan-Meier survival analysis was used to calculate the cumulative risk of re-treatment, adjusted for loss to followup. RESULTS: A total of 78 patients received transurethral microwave thermotherapy and 66 underwent transurethral prostatic resection. Median followup was 33 months. In the thermotherapy group mean maximum urinary flow rate improved from 9.2 ml. per second at baseline to 15.1, 14.5 and 11.9 ml. per second at 1, 2 and 3 years, and mean I-PSS decreased from 20 to 8, 9, and 12, respectively. In the resection group the corresponding numbers for maximum urinary flow rate were 7.8, 24.5, 23.0 and 24.7 ml. per second at 1, 2 and 3 years, and for I-PSS were 20, 3, 4 and 3, respectively. At 36 months, 14 patients in the thermotherapy and 8 from the resection groups underwent re-treatment, and the cumulative risk was 19.8% (95% confidence interval 10.4% to 29.3%) and 12.9% (4.5% to 21.3%), respectively (p = 0.28). CONCLUSIONS: Transurethral microwave thermotherapy and transurethral prostatic resection achieve durable improvement in patients with lower urinary tract symptoms suggestive of bladder outflow obstruction, while the magnitude of improvement is higher with resection. The repeat thermotherapy is based on failur

Item does not contain fulltext, PURPOSE: We investigated the effect of the alpha-blockers alfuzosin, terazosin and tamsulosin on urodynamic parameters after 6 months of therapy. MATERIALS AND METHODS: Between February 1992 and June 1998, 163 patients with lower urinary tract symptoms suggestive of bladder outlet obstruction were treated with alfuzosin (60), terazosin (66) and tamsulosin (37). Patients were evaluated with urodynamic studies, including pressure flow analysis, before treatment and after 6 months of therapy. Initially, all patients were also assessed by the International Prostate Symptom Score questionnaire and measurement of urinary flow rate. RESULTS: The majority of patients had no clear improvement in obstructive parameters, regardless of the alpha-blocker used, as urethral resistance factor and detrusor pressure maximum flow rate decreased by only 4 cm. H2O. There was a clear subjective and statistically significant decrease in International Prostate Symptom Score and quality of life scores of 6 and 2 points, respectively. No relevant statistical difference was noted among the effects of the 3 alpha-blockers on relieving symptoms or improving urodynamic parameters of obstruction. CONCLUSIONS: The alpha-blockers are effective for treating symptoms suggestive of bladder outlet obstruction in patients presenting with lower urinary tract symptoms but not for treating the obstruction.

KUN, 08 juni 2001, Promotor : Debruyne, F.M.J., Contains fulltext : 18983.pdf (publisher's version ) (Open Access)