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Paediatric urology is a subspeciality of urology, with close links to paediatric surgery. This review concludes that a holistic life-long approach to management in highly specialised treatment centres is essential for many of the rare congenital conditions - in Denmark, paediatric urology is centralised to two institutions: Rigshospitalet in Copenhagen and Aarhus University Hospital in Aarhus. Other than performing basic urology in paediatric patients, both centres specialise in complex and rare urological conditions and thus have been accredited by the European Reference Network on rare diseases through the eUrogen collaboration. Patient populations have covered span from prenatal to childhood, transition and for some anomalies, even into adulthood.

Background: Hypospadias surgery undertaken in early life often continues to impose challenges as patients age. Little is known about the natural history of uncorrected hypospadias persisting into adulthood., Objective: To describe presenting symptoms and management strategies in men with uncorrected hypospadias referred to our national tertiary transitional clinic for congenital urological conditions., Materials and Methods: Patients with uncorrected hypospadias older than 16 years at the time of referral were identified by searching the electronic patient record system for ICD-10 hypospadias codes. Data were extracted over a 10-year period according to a predefined protocol., Results: Among 201 referrals, 65 men with hypospadias (glanular n = 12, coronal n = 26, subcoronal n = 9, corporal n = 4, penoscrotal n = 2 and MIP n = 12) had never previously had reconstructive surgery undertaken. Obstructive symptoms predominated (n = 30) and the risk of symptoms increased with advancing age (Figure). Presenting complaints varied across the age span; cosmetic issues (n = 11) and coital pain (n = 5) were primarily seen in youth as opposed to urinary obstructive symptoms that were increasingly more frequent with age (p = 0.002) (Figure). Management included reconstructive surgery (n = 24), minor procedures (preputioplasty, circumcision, meatoplasty, dilatation/urethrotomy, total n = 28) as well as counselling (n = 12). The management strategies were independent of age and hypospadias type., Discussion: The current cohort delineates the dynamic nature of hypospadias in itself. We speculate that the distinction in the primary complaint leading to referral between the extremes of age may relate to the vanity and insecurity of youth while older patients first come forward when other symptoms arise. Dissatisfaction with genital appearance is uncommon in previous smaller studies on men with uncorrected hypospadias unlike in our study, where 11 patients were assessed mainly for cosmetic concerns. Obstruction is the main symptom encountered in adult hypospadias patients operated in early life, and a similar picture was observed in our cohort of unoperated cases. Urethral dilatation and internal urethrotomy are temporizing procedures but were successful in immediate alleviation of obstructive symptoms in patients not willing to consign themselves to formal surgery. The study is limited by its retrospective design, and our symptomatic cohort may also represent the extreme end of the hypospadias spectrum., Conclusion: Medical issues vary across the age span in men with unrepaired hypospadias. Minor surgical procedures as well as counselling play an equally important role as reconstructive hypospadias surgery in the management of unrepaired hypospadias in adulthood., Competing Interests: Conflicts of interest The third author is a statistical advisor for Journal of Pediatric Urology. All other authors have no conflicts of interest., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

To test the hypothesis that use of oral contraceptives (OC) changes diurnal variation in fluid balance mechanisms including blood pressure, secretion of vasopressin and oxytocin, and renal water and electrolyte excretion. Fifteen naturally cycling (NC) women in mid-follicular phase and 11 long-term OC users were included in a 24-h standardized inpatient study for measurements of vasopressin, oxytocin, sodium, and osmolality in plasma as well as urinary excretion of electrolytes, aquaporin-2, and prostaglandin E2. Blood pressure and heart rate were monitored noninvasively. Plasma vasopressin showed circadian rhythm ( P  = 0.02) and were similar in both groups ( P  = 0.18) including nighttime increases ( P  < 0.001). There was no circadian rhythm in plasma oxytocin within ( P  = 0.84) or between groups ( P  = 0.22). OC users had significantly lower plasma osmolality (Δosm: 3.05 ± 0.29 mosm/kg, P  = 0.04) and lower plasma sodium (ΔNa +: 0.91 ± 0.09 mmol/l, P  = 0.05). The two groups showed similar nighttime decreases in diuresis (1.08 ± 0.04 mL/(kg·h), P  < 0.001) and increases in urine osmolality (109 ± 9 mosm/kg, P  = 0.02), but similar rates of excretion of Aquaporin-2, prostaglandin E2 and sodium. Nighttime decreases in mean arterial pressure of approximately 13% were significant in both groups ( P  < 0.001), but 24-h average mean arterial pressure was significantly higher in OC users than in controls (+4.7 ± 0.4 mmHg, P  = 0.02). Packed cell volumes were similar between groups ( P  = 0.54). OC does not change the diurnal patterns of renal fluid excretion, but resets the osmoreceptors for vasopressin release and leads to a significant increase in arterial blood pressure., (© 2017 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of The Physiological Society and the American Physiological Society.)

Aim: We investigated the influence of gender on the diurnal regulation of urine production with special focus on vasopressin, oxytocin and prostaglandin E2., Methods: Fifteen young women in mid-follicular phase and 22 young men (20-33 years) were included. All participants underwent a 24-h circadian inpatient study under standardized conditions for measurements of plasma vasopressin, oxytocin, sodium and osmolality. Urine was fractionally collected for measurements of electrolytes, aquaporin-2 and prostaglandin E2., Results: Plasma vasopressin expressed a diurnal rhythm with a night-time increase in both genders (P < 0.001). The ratio between mean daytime and mean night-time was 1.57 [95% CI: 1.33-1.84] P < 0.001 in men and 1.35 [95% CI: 1.11-1.64] P = 0.002 in women. P-vasopressin was higher in males during the night (P < 0.05). There was no difference in diuresis (P = 0.43), urine osmolality (P = 0.12) or aquaporin-2 excretion (P = 0.80) between genders. We found a trend towards a higher reabsorption of free water in males (P = 0.07). The excretion of prostaglandin E2 was higher in males (P < 0.001). There was no diurnal rhythm in p-oxytocin (P = 0.37) and no correlation to diuresis, urine osmolality or aquaporin-2 excretions., Conclusion: Similar urinary flows and osmolalities are associated with levels of plasma vasopressin and renal PGE2, which are higher in males than in females. Oxytocin does not seem to play a role in the diurnal urine formation, whereas prostaglandin E2 could represent a mediator of the gender difference, not only as a mediator of the vasopressin response, but also as an independent factor. These findings need further elucidation., (© 2014 Scandinavian Physiological Society. Published by John Wiley & Sons Ltd.)

Purpose: We evaluated the long-term outcome of bladder autoaugmentation in children with neurogenic bladder dysfunction., Materials and Methods: Data were compiled from the records of 25 children with a median age of 9.3 years (range 0.9 to 14.2) who underwent detrusor myotomy between 1992 and 2008. All patients were diagnosed with small bladder capacity, low compliance and high end filling pressures, and were unresponsive to clean intermittent catheterization and anticholinergics., Results: Median followup was 6.8 years (range 0.1 to 15.6). Median postoperative bladder capacity was unchanged or decreased to 95 ml (range 25 to 274) during the first 3 months compared to a median preoperative capacity of 103 ml (14 to 250). At 5 months postoperatively median bladder capacity increased significantly to 176 ml (range 70 to 420, p<0.01). This increase remained significant during the rest of followup. Median bladder compliance doubled after 1 year to 10 ml/cm H2O (range 1 to 31, p<0.05) compared to the preoperative level, and further increased to 17 ml/cm H2O (5 to 55) at 5 years (p<0.05). Median maximal detrusor pressure was 43 cm H2O (range 8 to 140) preoperatively. This value decreased significantly postoperatively (p<0.01) and at final followup it was 26 cm H2O (range 6 to 97). Kidney function developed normally in all patients except 1 with persistent uremia. Reflux was alleviated in 7 of 9 cases. Of the patients 18 became continent on clean intermittent catheterization., Conclusions: Bladder autoaugmentation in children with neurogenic bladder dysfunction offers, after a transient decrease in bladder capacity, a long lasting increase in capacity and compliance, while the end filling pressure decreases., (Copyright © 2013 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate two-stage laparoscopic Fowler-Stephens (FS) orchidopexy for intra-abdominal testes., Materials and Methods: A retrospective analysis was performed of the clinical findings, interventions and outcomes in 111 boys undergoing laparoscopy for 132 impalpable testes., Results: The median age was 5.7 years (1.1-14.6 years). Twenty-seven testes were absent. Eighty testes were intra-abdominal, of which 10 were removed laparoscopically due to anatomical anomalies (4), short spermatic cord (5) or atresia (1). Twenty-five testes were located in the groin. Laparoscopic FS procedure was performed for 65 intra-abdominal testes: 60 two-stage operations and five where the testes were removed during FS second stage due to short vas or testicular atrophy. Outcome was successful in 80%. One-stage laparoscopic orchidopexy without vessel division was performed in five intra-abdominal testes with satisfactory results in three. In general, the success rate is higher in boys with bilateral intra-abdominal testes, probably due to younger age at operation., Conclusions: At our centre two-stage laparoscopic Fowler-Stephens orchidopexy for intra-abdominal testes showed results comparable to most other studies, but less satisfactory than a couple of recent studies. The median age at referral was higher than recommended. Operation at an earlier age may further improve the results.

Background: Sex hormones have a pronounced effect on arginine vasopressin (AVP), and therefore on the diurnal water homeostasis. Low and high levels of plasma-estradiol as seen in the follicular phase of the menstrual cycle may therefore alter the diurnal regulation of urine production. Furthermore the structural resemblance of oxytocin to vasopressin has led to speculations about the possible antidiuretic properties of oxytocin under normal physiological conditions. To elucidate the influence of high and low p-estradiol on the regulation of the diurnal urine production, 15 normal menstruating women (21-33 y) underwent two circadian in-patient investigations, both situated in follicular phase., Methods: Admitting the participants solely in the follicular phase resulted in high and low plasma-estradiol whereas plasma-progesterone was similar. Urine and blood samples were taken at predetermined time points to determine plasma AVP, plasma oxytocin, plasma aldosterone, plasma natriuretic peptide (ANP), urinary solute excretions, and urinary excretions of prostaglandin E2 (PGE-2) and aquaporin-2 (AQP-2). Blood pressure was measured every hour., Results: Plasma AVP, plasma aldosterone and plasma ANP were unaffected by the different levels of estradiol. All had marked circadian variations whereas oxytocin did not display any circadian rhythm. High estradiol resulted in lower p-osmolality and p-sodium reflecting the downward resetting of the osmoreceptors. Oxytocin did not correlate with either diuresis or urine osmolality. The diurnal urine production was similar in the two groups as were urine osmolality, excretion of PGE-2 and AQP-2. AQP-2 does not have a circadian rhythm and is not significantly correlated to either AVP or oxytocin under normal physiological conditions., Conclusion: High and low level of estradiol has no influence on the circadian rhythm of AVP or the subsequent urine production. High p-estradiol resets the osmoreceptors for AVP release. Furthermore it appears that oxytocin under normal physiological conditions do not contribute to the overall antidiuretic effect.

Purpose: Monosymptomatic polyuric nocturia is a consequence of aging. We investigated physiological differences between nonpolyuric and polyuric nocturia in the elderly population in relation to urine production regulation in young volunteers with special reference to gender., Materials and Methods: We performed a study in 37 elderly healthy volunteers 65 years or older and 30 young healthy volunteers 20 to 40 years old who were hospitalized for 48 hours. Before admittance and during hospitalization fluid intake was standardized. The first 24 hours were at baseline conditions. On night 2 participants were given a single oral dose of desmopressin (0.4 mg). During 48 hours urine and blood samples were taken at predetermined time points to measure urine output and plasma arginine vasopressin levels., Results: Elderly individuals with nocturnal polyuria had an inverted rhythm in urine output, which was restored after a single dose of desmopressin. There was an age related change in the circadian rhythm of arginine vasopressin secretion, which was associated with the presence or absence of nocturnal polyuria. A novel and unexpected finding was a decreased circadian rhythm of arginine vasopressin secretion in young women, similar to the pattern observed in elderly women but with a preserved decrease in nighttime urine production. Compensatory diuresis following the induction of temporary antidiuresis was markedly postponed in elderly participants., Conclusions: Age and gender related decreased arginine vasopressin secretion at night underscores the fact that other factors modulate urine production. The pharmacodynamics of desmopressin as an antidiuretic in the elderly population are different from those in young individuals.

Objective: To investigate the pharmacokinetic profile of oral desmopressin in elderly patients with nocturia, and to analyse any possible correlation between the absorption and clinical effect., Patients and Methods: In all, 32 patients were screened to determine the baseline number of nocturnal voids and the nocturia index; of these, 24 fulfilled the inclusion criteria and were enrolled for a pharmacokinetic evaluation of oral desmopressin 400 microg. A double-blind, randomized, placebo-controlled, crossover-effect evaluation period was then used to test the association between the absorption of desmopressin and pharmacodynamic effect. Serial plasma samples were collected for 8 h for a pharmacokinetic analysis of desmopressin. The pharmacodynamics after an equivalent oral dose before bedtime were assessed by measuring changes in the number of nocturnal voids, time to first nocturnal void and nocturnal diuresis, from placebo to active treatment., Results: There was a linear relationship between plasma desmopressin at 2 h after dosing and the area under the plasma concentration curve from 0 to infinity (Pearson's rho 0.923, P < 0.001). Women had a significantly higher plasma desmopressin concentration than men (P = 0.0012) and more adverse events. There was no correlation between plasma desmopressin at 2 h after dosing and the within-patient response in any of the effect variables. Generally, the number of nocturnal voids and nocturnal diuresis were half that with placebo. The time to the first nocturnal void was almost doubled compared with placebo., Conclusions: There seems to be a relationship between gender, plasma level of desmopressin and the incidence of adverse events. Plasma desmopressin at 2 h after dosing cannot be used to predict the pharmacodynamic response, although desmopressin lowers the nocturnal diuresis and the number of nocturnal voids.

Purpose: We studied the characteristics of a group of monosymptomatic nocturnal enuretics successfully treated with the alarm system, with special reference to changes in functional bladder capacity., Materials and Methods: The diaries of 7 girls and 19 boys 7 to 13 years old with severe nocturnal enuresis, small daytime bladder capacity (70% or less of expected capacity for age) and poor or absent response to desmopressin were analyzed. Patients were treated with an alarm until complete dryness was achieved for 21 consecutive nights before ending therapy. Immediately after the treatment they recorded a 1-week followup diary of voiding and fluid intake., Results: Mean duration of the alarm treatment was 82 days, and there was no change in nocturnal or 24-hour diuresis from baseline to followup. Nocturia developed during the alarm treatment in 48% of the children. The nocturnal diuresis on nocturia nights was significantly higher than on nights without nocturia. Daytime functional bladder capacity increased significantly in children with and without nocturia., Conclusions: Treatment with an alarm system increases daytime functional bladder capacity significantly in children with and without nocturia. A higher nocturnal urine production on nocturia nights explains why some children have nocturia and others do not.

Objective: To collect data on voiding patterns at baseline (no treatment) and during short-term desmopressin treatment, with special reference to the functional and the mean bladder capacity., Patients and Methods: The study included 120 children (aged 6-16 years) with monosymptomatic nocturnal enuresis. While at home they recorded their fluid intake and diuresis in two separate periods, i.e. 2 weeks as a baseline registration and another 2 weeks during desmopressin titration. On four study days the children recorded the time and volume of all voids and of fluid intake. From the diaries their functional and mean bladder capacities, 24-h diuresis and day/night ratio of diuresis were determined., Results: The mean 24-h diuresis was significantly lower during short-term desmopressin treatment. In most of the enuretics the mean day/night ratio increased on desmopressin treatment. The mean functional and mean bladder capacities were unaffected by desmopressin. Those not responding had bladder capacities of approximately 100 mL less than full responders. Regardless of response, practically all the enuretics in the study had a smaller functional bladder capacity than expected for their age. Among responders the morning void was significantly larger than the following voids during the day, and among non-responders the fourth void was significantly larger than the previous voids in the day. Desmopressin treatment did not influence these volumes significantly., Conclusions: Short-term desmopressin treatment does not affect functional and mean bladder capacity; 24-h urine production was reduced significantly (P<0.01) during desmopressin treatment.

Many elderly experience nocturia to such a degree that it influences their quality of life. Different studies use equivocal definitions when trying to analyze data on nocturia. The literature on etiology of female nocturia is sparse, whereas male nocturia has been studied rather extensively because of the association with lower urinary tract symptoms (LUTS). At present the few studies on female nocturia questions the fact that male and female nocturia have identical etiologies. This review will go through the present reports on prevalence and etiology of nocturia among women with reference to the male situation.

Objective: To study and compare the demography of enuretic children 7-16 years old attending a tertiary referral centre for childhood urinary incontinence, with that of normal children and what is generally known about enuretics from population-based studies., Methods and Materials: This was a retrospective analysis of data compiled from 298 enuretic patients referred to The Centre of Child Incontinence, Aarhus University Hospital, Skejby, Denmark, and 53 healthy controls. Data was obtained by conducting standardised questionnaire interviews. Patients were stratified according to accompanying symptoms and severity of enuresis. Statistical analysis of the results was then performed., Results: Two hundred and ninety-three patients had sufficient data registered to allow reliable analysis. Male-female ratio was 2.2:1. Primary and secondary enuresis was found in 87.4% and 11.6% of the patients respectively. Enuresis was associated with a reported history of bronchial asthma (p < 0.05), verified allergy (p < 0.05) and a positive family history of the same disorder (p < 0.01). No association with psycho-developmental factors was evident. Approximately 98% of the patients had undergone some form of therapy at presentation. 74.1% of the patients had pure monosymptomatic enuresis, 16.4% had day and night time incontinence, while the remaining 9.6% had nocturnal incontinence combined with other lower urinary tract symptoms other than daytime wetting., Conclusions: The demography of our patient population was, with a few exceptions, within the confines of what has previously been reported. Our results also reaffirmed the heterogeneity of enuretic patients.

Unilateral ureteral obstruction (UUO) is associated with reductions in ipsilateral renal blood flow (RBF) and glomerular filtration rate (GFR) caused by an active preglomerular vasoconstriction, where angiotensin II (ANGII) may be an important mediator. Calcium-channel blockers preferentially dilate preglomerular vessels and abolish the vasoconstrictor actions of ANGII in preglomerular arterioles of the hydronephrotic rat kidney. In this study, we, therefore, examined the effects of the calcium-channel blocker verapamil (3.65 microg/kg per minute i.v.) on RBF, GFR and renal vascular resistance (RVR) in our pig model with UUO, where ultrasonic flow probes are mounted on each renal artery and catheters placed in the abdominal aorta and both renal veins. Verapamil treatment was associated with a 34% reduction in ipsilateral RBF (from 182.6 +/- 20.5 ml/min to 120.6 +/- 12.2 ml/min, P < 0.001), which was similar to the 27% reduction in ipsilateral RBF in controls (from 194.6 +/- 13.1 ml/min to 140.6 +/- 15.2 ml/min, P < 0.001). Ipsilateral GFR was reduced by 70% in the verapamil-treated pigs (from 29.0 +/- 2.6 to 8.5 +/- 0.9 ml/min, P < 0.001) and by 73% in control animals (from 29.2 +/- 3.1 to 7.6 +/- 2.1 ml/min, p < 0.001). However, the increase in RVR was significantly attenuated in the verapamil-treated pigs. Ipsilateral RVR increased by 19% in the verapamil-treated pigs (from 0.585 +/- 0.076 to 0.726 +/- 0.081 mmHg/min/ml, P < 0.05) compared with a 34% increase in control pigs (from 0.560 +/- 0.056 to 0.854 +/- 0.091 mmHg/min per milliliter, P<0.001), suggesting that an intact calcium-channel may be important for the increase in renal vascular resistance during unilateral ureter obstruction. In conclusion, the present study shows that verapamil is able to modulate the increase in renal vascular resistance in response to increased pelvic pressure.