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Aims To investigate the effect of losartan on preventing bladder fibrosis and protecting renal function in rats with neurogenic paralysis bladder (NPB). Materials and methods Rats were assigned to the transecting spinal nerves group (TSNG), transecting spinal nerves + losartan group (LSTG), and control group (CG). On Day 32 postsurgery, bladder capacity (BC), bladder compliance (ΔC), bladder leakage pressure (Pves.leak) of TSNG and LSTG while BC, ΔC, and bladder threshold pressure (Pves.thre) of CG were measured by cystometry in each cohort. Renal function and the expression quantity of Angiotensin Ⅱ (Ang II) in blood were detected, in addition Ang II, Ang II Type 1 receptor (AT1), transformation growth factor β1 (TGFβ1), Collagen Ⅲ, and collagen fibrin in the bladder tissue were detected too. Results ΔC in TSNG and LSTG decreased significantly compared to the CG. Pves.leak in TSNG and LSTG were significantly higher than Pves.thre in CG. Renal function of both TSNG and LSTG decreased significantly compared with the CG, but renal function in LSTG was better than in TSNG. Ang Ⅱ in blood and bladder tissue in TSNG and LSTG increased significantly compared with CG. AT1 was expressed in the bladder tissue of all rats. The TGFβ1, Collagen Ⅲ, and collagen fibrin expression level increased significantly in TSNG compared with LSTG and CG, while these levels were not significantly different between CG and LSTG. Conclusion Losartan might prevent NPB fibrosis by stopping the upregulated signaling of Ang II/AT1/TGFβ1 and consequently may reduce kidney damage from occurring.

International audience; Congenital anomalies of the kidney and urinary tract (CAKUT) constitute one of the most frequent birth defects and represent the most common cause of chronic kidney disease in the first three decades of life. Despite the discovery of dozens of monogenic causes of CA-KUT, most pathogenic pathways remain elusive. We performed whole-exome sequencing (WES) in 551 individuals with CAKUT and identified a heterozygous de novo stop-gain variant in ZMYM2 in two different families with CAKUT. Through collaboration, we identified in total 14 different heterozygous loss-of-function mutations in ZMYM2 in 15 unrelated families. Most mutations occurred de novo, indicating possible interference with reproductive function. Human disease features are replicated in X. tropicalis larvae with morpho-lino knockdowns, in which expression of truncated ZMYM2 proteins, based on individual mutations, failed to rescue renal and cranio-facial defects. Moreover, heterozygous Zmym2-deficient mice recapitulated features of CAKUT with high penetrance. The ZMYM2 protein is a component of a transcriptional corepressor complex recently linked to the silencing of developmentally regulated endoge-nous retrovirus elements. Using protein-protein interaction assays, we show that ZMYM2 interacts with additional epigenetic silencing complexes, as well as confirming that it binds to FOXP1, a transcription factor that has also been linked to CAKUT. In summary, our findings establish that loss-of-function mutations of ZMYM2, and potentially that of other proteins in its interactome, as causes of human CAKUT, offering new routes for studying the pathogenesis of the disorder.

Congenital anomalies of the kidney and urinary tract (CAKUT) are the most common cause of chronic kidney disease in the first three decades of life, and in utero obstruction to urine flow is a frequent cause of secondary upper urinary tract malformations. Here, using whole-exome sequencing, we identified three different biallelic mutations in CHRNA3, which encodes the α3 subunit of the nicotinic acetylcholine receptor, in five affected individuals from three unrelated families with functional lower urinary tract obstruction and secondary CAKUT. Four individuals from two families have additional dysautonomic features, including impaired pupillary light reflexes. Functional studies in vitro demonstrated that the mutant nicotinic acetylcholine receptors were unable to generate current following stimulation with acetylcholine. Moreover, the truncating mutations p.Thr337Asnfs(∗)81 and p.Ser340(∗) led to impaired plasma membrane localization of CHRNA3. Although the importance of acetylcholine signaling in normal bladder function has been recognized, we demonstrate for the first time that mutations in CHRNA3 can cause bladder dysfunction, urinary tract malformations, and dysautonomia. These data point to a pathophysiologic sequence by which monogenic mutations in genes that regulate bladder innervation may secondarily cause CAKUT.

In this study, a new species of Pseudogilbia Møller, SchwarzhansNielsen 2004 is described based on two male specimens (40-44 mm L

Human studies have shown loss of diversity of the gut microbiome following hematopoietic stem cell transplantation (HSCT) in association with significant gut injury caused by the preparative regimen. Prolonged antibiotic use worsens loss of microbiome diversity and increases risk of complications such as graft-versus-host disease (GVHD). Our data support the hypothesis that loss of intestinal commensals that produce short-chain fatty acids (SCFAs) may increase dysbiosis. Here, we report an extensive longitudinal examination of changes in the luminal SCFAs in children undergoing allogeneic HSCT, and the relationship of those changes to the microbiota and antibiotic exposure. We found significant and progressive alterations in butyrate, and in additional SCFAs in stool in the first 14 days after transplant, a finding not observed in published mouse studies. SCFA levels were lower in children receiving antibiotics with activity against anaerobic organisms. Moreover, day 14 post-HSCT butyrate and propionate levels are lower in children who went on to develop GVHD, although our disease population was small. These data provide insight into the mechanism of prior observations that loss of diversity and increased antibiotic use are associated with GVHD following HSCT. Our findings offer potential modifiable targets to reduce risk of GVHD and improve survival after HSCT.

Aims: To introduce the standard procedure of cystometry and interpretation of the results in children. Methods: The literature on cystometry in children in PubMed for the last 20 years was reviewed. The updated knowledge regarding indication, preparation, technique, and interpretation of cystometry in children were summarized. Results: Filling cystometry is the core content of a paediatric urodynamic study. In this section, the technique for performing cystometry is introduced in details. Emphasis is placed on correctly setting up the equipment according to ICS and ICCS guidelines, using appropriate terminology, providing indications for its performance with specific considerations for children, and proper interpretation of results. Conclusions: Cystometry can be used in children including newborn to evaluate lower urinary tract dysfunction.

To investigate the correlation between urethral instability (URI) and overactive bladder (OAB) in children. We retrospectively investigated 126 children with OAB and 36 children without OAB using synchro-cystourethrometry. The prevalence of detrusor overactivity (DO) and URI, and the diagnostic sensitivity of DO alone and DO combined with URI, was compared. The OAB children with URI voluntarily received transcutaneous electrical pudendal nerve stimulation with anisodamine (stimulation group, SG) or anisodamine alone (non-stimulation group, NSG). The effectiveness of treatment was evaluated. Average voided volume (AVV), maximum voided volume (MVV), and number of voids per day (NV) were collected and analyzed. In OAB children, the prevalence of DO and URI was 51.6 and 32.5%, respectively. The prevalence of URI was 5.6% in controls. The prevalence of URI was significantly higher in OAB children. The diagnostic sensitivity and Youden index of DO combined with URI were higher than DO alone. In SG, 45.7% of children were cured, with a ≥ 50% improvement rate of 82.9%, while no child was cured, with a ≥ 50% improvement rate of 36.8% in NSG. A significant increase in AVV and MVV together, with a decrease in NV, was seen in SG. There was a significant difference in visual analogue scale values between SG and NSG (P

Patients with a history of intravenous drug abuse included in an official opioid substitution program represent an important subgroup of patients with chronic hepatitis C. The objective of this study was to assess the efficacy of and adherence to treatment with peginterferon and ribavirin in Austrian patients on stable opioid substitution therapy (OST). This prospective, multicenter, observational, non-interventional trial (clinicaltrials.gov identifier, NCT01416610) included treatment-naive patients with chronic hepatitis C on OST. Treatment consisted of peginterferon alpha-2a (PEGASYS®, 180 µg/week) plus ribavirin (COPEGUS®, 1000/1200 mg/day in genotypes (GT) 1/4 and 800 mg/day in GT 2/3) for 24–72 weeks, according to GT and viral response. The intention-to-treat (ITT) population comprised 88 patients. Mean duration of therapy was 6.0 ± 2.8 months. Treatment was discontinued earlier than planned in 34 out of 88 patients (39%), mainly because of poor adherence or side effects of treatment. At the end of treatment 65/88 patients (74%) were PCR negative. During follow-up, 5 patients relapsed. Only 44/88 patients (50%) could be evaluated 24 weeks after the end of treatment. Sustained virologic response 24 weeks after end of therapy (SVR24) was documented in 39/88 patients (44%). If only patients were considered who finished treatment as planned and for whom results at follow-up week 24 were available, the SVR24 rate was 89% (32/36). Despite favorable prognostic factors, such as young age and a high proportion of GT3, SVR rates were low in this cohort of patients receiving OST, the main reason being poor adherence; however, in those patients completing treatment, the SVR rate was high.

BACKGROUND: Independent associations between chronic liver disease, MELD, and postoperative outcomes among patients selected for liver resection have not been completely established. We hypothesized independent associations between MELD, cirrhosis, and postoperative mortality. METHODS: Patient-level data from the targeted hepatectomy module and ACS NSQIP PUF during 2014–2015 were merged. Multivariable regression models with interaction effect between MELD and liver texture (normal vs congested/fatty vs cirrhotic) tested the independent effects of covariates on mortality and morbidity. RESULTS: 3,530 patients were included, of whom 668 patients (19%) had cirrhosis. ACS NSQIP defined mortality (3.9%vs1.1%) and morbidity (23.5%vs15.8%) were higher in patients with cirrhosis (both p

Pictures are often used in cognitive research to represent objects and many species have demonstrated the ability to recognize two-dimensional pictures as representations of their three-dimensional counterparts. However, for ursids picture recognition has been reported in only one study of a single 11-year-old female American black bear (Johnson-Ulrich et al. 2016). We tested the picture recognition abilities of an additional species, the sloth bear. After a food preference test by which the bears' food options were ranked and categorized as high-, mid-, and low-preference items, we tested a sub-adult male and an adult female sloth bear by presenting two pictures of food in each testing trial-a high-preference food and a low-preference food. Both bears met the criterion by choosing the pictures of their preferred foods in at least 80% of the trials in three consecutive testing sessions. We then presented never-before-used pictures of high-preference versus low-preference food items and they again met our criterion.

Aims: A pilot survey shows that primary nocturnal enuresis (PNE) prevalence has increased significantly during the past decade in Mainland China. Whether it is related to the delay of elimination communication (EC) is unclear. This study retrospectively investigated the influence of delayed EC on the PNE prevalence in children and adolescents in mainland China. Methods: A cross-sectional study of PNE prevalence was performed by distributing 19 500 anonymous self-administered questionnaires to parents in five provinces of mainland China from July 2017 to October 2017. The questionnaires included sociodemographic data, family caregivers’ information, and details about the disposable diapers (DD) usage, EC commencement date, psychological disorders, lower urinary tract symptoms, and family history of PNE in children and adolescents. The 2017 PNE prevalence was compared with that of 2006 in Mainland China. Results: The total response rate was 97.04% (18 631 of 19 500) and 92.39% (18 016 of 19 500) qualified for statistical analysis. The PNE prevalence in 2017 has increased significantly compared to that of 2006 (7.30% vs 4.07%, P < 0.001). The PNE prevalence in children with EC starting before 6 months of age was significantly lower than those who start after 12 months of age. The longer DD were used and the later the beginning of EC, the higher the PNE prevalence was found. Conclusions: The PNE prevalence in Mainland China has increased significantly during the past 10 years. A longer use of DD and later onset of EC may be risk factors for PNE.

To examine whether the daily consumption of normal-protein (NP) vs higher-protein (HP) breakfasts improve free-living glycemic control in overweight/obese, 'breakfast skipping' adolescents. Twenty-eight healthy, but overweight, teens (age: 19±1 year; BMI: 29.9±0.8 kg m(-2)) completed a 12-week randomized parallel-arm study in which the adolescents consumed either a 350 kcal NP breakfast (13 g protein) or HP breakfast (35 g protein). Pre- and post-study 24-h blood glucose measures were assessed using continuous glucose monitoring. Although no main effects of time or group were detected, time by group interactions were observed. Post hoc pairwise comparisons assessing the post-pre changes revealed that the daily consumption of the HP breakfasts tended to reduce the 24-h glucose variability (s.d.) vs NP (-0.17±0.09 vs +0.09±0.10 s.d.; P=0.06) and tended to reduce the time spent above the high glucose limit (-292±118 vs -24±80 min; P=0.09). The consumption of the HP breakfasts also reduced the 24-h maximal (peak) glucose response (-0.94±0.36 vs +0.30±0.18 mmol l(-1); P0.01) and reduced postprandial glucose fluctuations (-0.88±0.44 vs +0.49±0.34 mmol l(-1); P0.03) vs NP. These data suggest that the daily addition of a HP breakfast, containing 35 g of high-quality protein, has better efficacy at improving free-living glycemic control compared with a NP breakfast in overweight/obese, but otherwise healthy, 'breakfast skipping' adolescents.

We evaluated the relationship between testicular volume differential, total testis volume and total motile sperm count in adolescents with varicocele. Prior reports have been inconclusive regarding the association of testis volume with semen analysis parameters.We retrospectively reviewed a single institution database of Tanner 5 adolescents with nonoperated left varicocele with at least 1 semen analysis available. Patients were included in analysis if they had clinical left varicocele, ultrasound measurement of testis volume and no prior inguinal or scrotal surgery.We analyzed the records of 100 patients. Mean ± SD age at presentation was 15.2 ± 3.5 years. The mean testicular volume differential was 8% ± 23% and 31 patients (31%) had a greater than 20% testicular volume differential. Mean total testis volume was 30.4 ± 13.3 cc. Varicocele was grade 3 in 39 patients, grade 2 in 50, grade 1 in 8 and ungraded in 3. Median total motile sperm count in the cohort was 42.0 (IQR 9.2, 105.8). ROC analysis with the Youden index indicated an optimal total testis volume cutoff of 30 cc to predict the total motile sperm count. In a multivariable logistic model accounting for age and varicocele grade a testicular volume differential of greater than 20% was associated with twice the odds of a total motile sperm count of less than 20 million per cc (OR 2.1, 95% CI 1.02-4.12, p = 0.04). Total testis volume less than 30 cc more than quadrupled the odds of a total motile sperm count of less than 20 million per cc (OR 4.2, 95% CI 1.8-9.7, p0.001).Total testis volume and the testicular volume differential are associated with semen analysis outcomes in adolescents with varicocele. A testicular volume differential of greater than 20% doubles the odds of a low total motile sperm count. A total testis volume of less than 30 cc quadruples the odds of a low total motile sperm count.

Congenital anomalies of the kidney and urinary tract (CAKUT) are the most common cause (40-50%) of chronic kidney disease (CKD) in children. About 40 monogenic causes of CAKUT have so far been discovered. To date less than 20% of CAKUT cases can be explained by mutations in these 40 genes. To identify additional monogenic causes of CAKUT, we performed whole exome sequencing (WES) and homozygosity mapping (HM) in a patient with CAKUT from Indian origin and consanguineous descent. We identified a homozygous missense mutation (c.1336C>T, p.Arg446Cys) in the gene Von Willebrand factor A domain containing 2 (VWA2). With immunohistochemistry studies on kidneys of newborn (P1) mice, we show that Vwa2 and Fraser extracellular matrix complex subunit 1 (Fras1) co-localize in the nephrogenic zone of the renal cortex. We identified a pronounced expression of Vwa2 in the basement membrane of the ureteric bud (UB) and derivatives of the metanephric mesenchyme (MM). By applying in vitro assays, we demonstrate that the Arg446Cys mutation decreases translocation of monomeric VWA2 protein and increases translocation of aggregated VWA2 protein into the extracellular space. This is potentially due to the additional, unpaired cysteine residue in the mutated protein that is used for intermolecular disulfide bond formation. VWA2 is a known, direct interactor of FRAS1 of the Fraser-Complex (FC). FC-encoding genes and interacting proteins have previously been implicated in the pathogenesis of syndromic and/or isolated CAKUT phenotypes in humans. VWA2 therefore constitutes a very strong candidate in the search for novel CAKUT-causing genes. Our results from in vitro experiments indicate a dose-dependent neomorphic effect of the Arg446Cys homozygous mutation in VWA2.

Summary Introduction Urethral meatotomy as treatment for meatal stenosis is a common pediatric urology procedure; however, little is known about the patient experience following this procedure. Objective We aim to evaluate clinical factors associated with patient-reported symptom improvement after urethral meatotomy. Study design The families of boys undergoing urethral meatotomy between 2/2013 and 8/2016 received a survey by mail 6 weeks after surgery. Families were queried on changes in symptoms using a Likert-type scale (5 = much improved, 4 = somewhat improved, 3 = no change, 2 = somewhat worse, and 1 = much worse). Patient and procedure characteristics of the respondents were obtained via chart review. These included surgical indication(s) (abnormal stream, dysuria, or storage symptoms), postoperative complications, reoperation, and unplanned postoperative communications. Patients who had procedures other than simple urethral meatotomy were excluded. Descriptive statistics were compiled, and generalized estimating equations used to determine the associations of patient and procedure characteristics with symptom improvement. Results We sent 629 surveys and received 194 responses (30.4%). Twelve respondents were excluded for complex procedures or miscoding. The majority of respondents were privately insured (74%) and were between 5 and 12 years old (45%) or 1 and 4 years old (42%). The most frequent surgical indication was abnormal stream (72%) followed by pain (21%) and storage symptoms (15.5%). Nine respondents had minor complications (4.9%). Four patients had restenosis requiring repeat urethral meatotomy. After surgery, a majority (79%) were “much improved,” 16% were “somewhat improved,” 3% had “no change,” and 1% were “somewhat worse.” No family reported “much worse.” Those patients who had “abnormal stream” as a surgical indication were significantly more likely to report “much improved” (OR 1.83, p = 0.014) than those without. Patient-reported improvement was not associated with suture use, patient age, insurance, surgeon, or location of the procedure (Table). Discussion Little has been written about patient-reported outcomes following urethral meatotomy. Our study affirms that the majority of boys improve following this procedure. However, improvement is significantly more likely if the child has a preoperative indication of an abnormal stream, such as deflection or spraying. Boys with symptoms of dysuria, frequency, or incontinence may be experiencing sequelae of meatal stenosis that simply take longer to improve. Alternatively, the meatal stenosis may be incidental to the primary symptoms. Conclusions A majority of families report substantial symptomatic improvement after urethral meatotomy. However, boys undergoing urethral meatotomy for reasons other than a urinary stream abnormality are less likely to experience improvement. Table . Patient characteristics and key outcomes stratified by patient-reported improvement. Not “much improved” “Much improved” p-value (n = 38) (n = 144) Patient characteristics Indication (n; %) Stream abnormality 26/141 (18.4%) 115/141 (81.6%) 0.014 ∗ Pain 11/41 (26.8%) 30/41 (73.2%) 0.40 Storage symptoms 6/30 (20.0%) 24/30 (80.0%) 0.90 Suture used (n; %) 25/131 (19.1%) 106/131 (80.9%) 0.21 Outcomes Complications 5/9 (55.6%) 4/9 (44.4%) 0.02 ∗ Reoperation 3/4 (75.0%) 1/4 (25.0%) 0.02 ∗ ∗ A p-value

Aims As awareness and frequency of tethered spinal cord (TSC) related to occult spinal dysraphism (OSD) has increased with magnetic resonance imaging (MRI), variability exists in its evaluation and management. Due to no published level I data, we summarize the current International Children's Continence Society (ICCS) recommendations for diagnosis and treatment of OSD. Methods Guidelines were formulated based on analysis of pertinent literature and consensus among authors. This document was vetted by the multidisciplinary members of the ICCS via its website before submission for peer review publication. Results The more frequent diagnosis of OSD is associated with increased operative intervention. Spinal cord untethering (SCU) has a highly variable risk profile, largely dependent on the specific form of OSD. Progressive neurological deterioration attributed to “tethered cord” may occur, with or without surgery, in selected forms of OSD whereas other cohorts do well. Conclusion Infants with classic cutaneous markers of OSD, with progressive neurologic, skeletal, and/or urologic findings, present no diagnostic or therapeutic dilemma: they routinely undergo MRI and SCU. Conversely, in asymptomatic patients or those with fixed, minor abnormalities, the risk profile of these OSD cohorts should be carefully considered before SCU is performed. Irrespective of whether or not SCU is performed, patients at risk for progression should be followed carefully throughout childhood and adolescence by a multidisciplinary team.

Concern in patients with bladder exstrophy after reconstruction regarding potential injury to pelvic neurourological anatomy and a resultant functional deficit prompted combined (simultaneous) cystometrography and electromyography after complete primary repair of bladder exstrophy. We determined whether complete primary repair of bladder exstrophy would adversely affect the innervation controlling bladder and external urethral sphincter function.Complete primary repair of bladder exstrophy was performed via a modified Mitchell technique in newborns without osteotomy. Postoperative evaluation included combined cystometrography and needle electrode electromyography via the perineum, approximating the external urethral sphincter muscle complex. Electromyography was done to evaluate the external urethral sphincter response to sacral reflex stimulation and during voiding.Nine boys and 4 girls underwent combined cystometrography/electromyography after complete primary repair of bladder exstrophy. Age at study and time after complete primary repair of bladder exstrophy was 3 months to 10 years (median 11.5 months). Cystometrography revealed absent detrusor overactivity and the presence of a sustained detrusor voiding contraction in all cases. Electromyography showed universally normal individual motor unit action potentials of biphasic pattern, amplitude and duration. The external urethral sphincter sacral reflex response was intact with a normal caliber with respect to Valsalva, Credé, bulbocavernosus and anocutaneous (bilateral) stimulation. Synergy was documented by abrupt silencing of external urethral sphincter electromyography activity during voiding.After complete primary repair of bladder exstrophy combined cystometrography/electromyography in patients with bladder exstrophy showed normal neurourological findings, including sacral reflex responses, sustained detrusor voiding contraction and synergic voiding, in all patients postoperatively. These findings confirm the safety of complete primary repair of bladder exstrophy. Based on our results we have discontinued routine electromyography in these patients.

PURPOSE: We present a consensus view of members of the International Children's Continence Society (ICCS) together with pediatric gastroenterologists, experts in the field of functional gastrointestinal disorders, on the management of functional constipation in children with lower urinary tract symptoms.MATERIALS AND METHODS: Discussions were held by the board of the ICCS and a multidisciplinary core group of authors was appointed. The draft document review process was open to all ICCS members via the website. Feedback was considered by the core authors and, by agreement, amendments were made as necessary.RESULTS: Guidelines on the assessment, and pharmacological and nonpharmacological management of functional constipation in children with lower urinary tract symptoms are outlined.CONCLUSIONS: The final document is not a systematic literature review. It includes relevant research when available, as well as expert opinion on the current understanding of functional constipation in children with lower urinary tract symptoms. The document is intended to be clinically useful in primary, secondary and tertiary care settings.

Florida manatees inhabit the coastal and inland waters of the peninsular state. They have little difficulty navigating the turbid waterways, which often contain obstacles that they must circumnavigate. Anatomical and behavioral research suggests that the vibrissae and associated follicle-sinus complexes that manatees possess over their entire body form a sensory array system for detecting hydrodynamic stimuli analogous to the lateral line system of fish. This is consistent with data highlighting that manatees are tactile specialists, evidenced by their specialized facial morphology and use of their vibrissae during feeding and active investigation/manipulation of objects. Two Florida manatees were tested in a go/no-go procedure using a staircase method to assess their ability to detect low-frequency water movement. Hydrodynamic vibrations were created by a sinusoidally oscillating sphere that generated a dipole field at frequencies from 5 to 150 Hz, which are below the apparent functional hearing limit of the manatee. The manatees detected particle displacement of less than 1 μm for frequencies of 15-150 Hz and of less than a nanometer at 150 Hz. Restricting the facial vibrissae with various size mesh openings indicated that the specialized sensory hairs played an important role in the manatee's exquisite tactile sensitivity.

The first record of pughead deformity in a Neotropical freshwater fish not raised in captivity is provided, based on an adult male of Cichla kelberi introduced into an estuary of Rio de Janeiro State, south-eastern Brazil. A general biological characterization of the specimen is provided and aspects of its aberrant anatomy are described. Environmental and biological factors that may cause pughead deformity in fishes are discussed.

Background Children with urinary incontinence (UI) have associated functional constipation (FC) and fecal incontinence (FI). The physiology between lower urinary tract (LUT) and anorectum in children has not been elucidated. Aims: Observe the effect of rectal distention (RD) on LUT function, and bladder filling and voiding on anorectal function. Methods Children with voiding dysfunction referred to Boston Children's Hospital were prospectively enrolled in combined urodynamic (UDS) and anorectal manometry (ARM). Anorectal and urodynamic parameters were simultaneously measured. Patients underwent two micturition cycles, first with rectal balloon deflated and second with it inflated (RD). Lower urinary tract and anorectal parameters were compared between cycles. Key Results Ten children (seven UI, four recurrent UTIs, nine FC ± FI) were enrolled. Postvoid residual (PVR) increased (p = 0.02) with RD. No differences were observed in percent of bladder filling to expected bladder capacity, sensation, and bladder compliance with and without RD. Bladder and abdominal pressures increased at voiding with RD (p < 0.05). Intra-anal pressures decreased at voiding (p < 0.05), at 25% (p = 0.03) and 50% (p = 0.06) of total volume of bladder filling. Conclusions & Inferences The PVR volume increased with RD. Stool in the rectum does not alter filling cystometric capacity but decreases the bladder's ability to empty predisposing patients with fecal retention to UI and UTIs. Bladder and abdominal pressures increased during voiding, demonstrating a physiological correlate of voiding dysfunction. Intra-anal pressures decreased during bladder filling and voiding. This is the first time intra-anal relaxation during bladder filling and voiding has been described.

Recent data comparing prenatal to postnatal closure of myelomeningocele showed a decreased need for ventriculoperitoneal shunting and improved lower extremity motor outcomes in patients who underwent closure prenatally. A total of 11 children whose spinal defect was closed in utero were followed at our spina bifida center. We hypothesized that in utero repair of myelomeningocele improves lower urinary tract function compared to postnatal repair.Eleven patients who underwent in utero repair were matched to 22 control patients who underwent postnatal repair according to age, gender and level of spinal defect. Urological outcomes were retrospectively reviewed including urodynamic study data, need for clean intermittent catheterization, use of anticholinergic agents and prophylactic antibiotics, and surgical history. The need for ventriculoperitoneal shunting or spinal cord untethering surgery was also reviewed.Mean followup was 7.2 years for patients who underwent in utero repair and 7.3 years for those who underwent postnatal repair. Mean patient age at compared urodynamic studies was 5.9 years for in utero repair and 6.0 years for postnatal repair. The in utero repair group was comprised of 5 lumbar and 6 sacral level defects with equal matching (1:2) in the postnatal repair cohort. There were no differences between the groups in terms of need for clean intermittent catheterization, incontinence between catheterizations or anticholinergic/antibiotic use. Urodynamic parameters including bladder capacity, detrusor pressure at capacity, detrusor overactivity and the presence of detrusor sphincter dyssynergia were not significantly different between the groups. There was no difference in the rate of ventriculoperitoneal shunting (p = 0.14) or untethering surgery (p = 0.99).While in utero closure of myelomeningocele has been shown to decrease rates of ventriculoperitoneal shunting and improve motor function, it is not associated with any significant improvement in lower urinary tract function compared to repair after birth.

We investigated associated urodynamic abnormalities in toilet trained children with vesicoureteral reflux.A total of 298 toilet trained children with primary vesicoureteral reflux underwent urodynamic evaluation. Urodynamic parameters were reviewed and correlated with age, gender, presence of lower urinary tract symptoms and reflux severity.Symptomatic lower urinary tract symptoms were present in 111 children (37.2%, group 1). Children with lower urinary tract symptoms had significantly decreased severity of vesicoureteral reflux compared to children without these symptoms (187 patients, group 2). The majority of the patients had normal early bladder compliance regardless of presence of lower urinary tract symptoms or reflux grade. On the other hand, decreased late bladder compliance was more common in group 1 vs group 2. Ratio of cystometric bladder capacity to expected bladder capacity was higher in group 2. Detrusor overactivity was observed in 28.5% of the children, and the incidence was significantly higher in group 1 vs group 2, and in mild vs moderate or severe reflux. Dysfunctional voiding from bladder sphincter dyscoordination was seen in 32% of children 2.5 to 4 years old with vesicoureteral reflux and lower urinary tract symptoms, compared to 8% in children 5 to 16 years old.The presence of lower urinary tract symptoms in children with vesicoureteral reflux correlated well with some urodynamic findings suggestive of overactive bladder and negatively correlated with reflux severity. In contrast, dysfunctional voiding was more common in younger children with reflux and lower urinary tract symptoms. These findings suggest that treatment of voiding dysfunction should be directed toward the specific type of abnormality in children with vesicoureteral reflux.

We prospectively evaluated the precision of ultrasound and computerized tomography to diagnose urinary stones in children and determined whether these differences in radiological findings have any impact on clinical management.A total of 50 consecutive patients with suspected urolithiasis underwent computerized tomography and ultrasound. Two radiologists reviewed each study independently in blinded fashion. When a difference in findings was detected, 8 pediatric urologists reviewed the case. Clinical management was based on the results of each radiological test independently. Statistical analysis was performed using Fisher's exact test.Compared to computerized tomography ultrasound had 76% sensitivity and 100% specificity. In 8 patients stone(s) seen on computerized tomography was not seen on ultrasound. The average size of missed stones was 2.3 mm. In 7 patients computerized tomography showed stones bilaterally but stone was seen on only 1 side on ultrasound. When evaluating the clinical impact, the ultrasound/computerized tomography discrepancy did not result in any significant change in clinical management except in 4 cases. In these cases ultrasound findings suggested that additional imaging was required and, thus, stone(s) in the distal ureter would have been identified on subsequent imaging.Although computerized tomography is more sensitive for detecting urolithiasis than ultrasound, the difference in usefulness between the 2 radiological tests may not be clinically significant. Given concerns for the potentially harmful cumulative long-term effect of radiation, ultrasound should be considered the first imaging test in children with suspected urolithiasis.

OBJECTIVES To review our experience at a children's hospital over a 10-year period with the Palomo, Ivanissevich, subinguinal and laparoscopic techniques for varicocele, assessing the success and complication rates according to specific procedure, and the added effect that the modifications of microsurgery and artery-sparing has had on these rates. A second objective was to assess the rate of testicular compensatory growth after surgery for testicular hypotrophy. PATIENTS AND METHODS Ninety-two patients with >1 year of follow-up between 1996 and 2006 were assessed retrospectively. The median (range) age at surgery was 15 (8-21) years. Patients were stratified based on the surgical technique used by eight different urology faculty members. Microsurgery and attempted artery-sparing were applied to some Palomo, Ivannisevich, and subinguinal cases but not to laparoscopic procedures. RESULTS The laparoscopic (100%) and Palomo (93%) techniques had significantly higher success rates than the Ivanissevich approach (69%). The success rate with the subinguinal technique (88%) was intermediate between the more successful supra-inguinal and less successful inguinal approaches. There was a higher hydrocele rate (32%) in the laparoscopic approach. Artery sparing significantly lowered hydrocele rates but had no effect on success rates. Incorporating microsurgery also had no effect on success rates but resulted in no hydrocele formation. One case of testicular atrophy occurred in a patient undergoing microsurgical artery-sparing subinguinal spermatic vein ligation. There was compensatory growth in 68% of patients operated on for testicular hypotrophy. CONCLUSIONS During our 10-year experience the laparoscopic and Palomo approaches were the most successful. The subinguinal approach (usually incorporating microsurgery and artery sparing) had an intermediate success rate. The Ivanissevich approach was least successful. Hydroceles did not occur when microsurgery was used, and were significantly less common with artery sparing. The only case of testicular atrophy was with a microsurgical artery-sparing subinguinal approach. When the spermatic vein was ligated for testicular hypotrophy there was compensatory growth in two-thirds of testes.

The natural history of caliceal diverticula in children is unknown. We review our series of children with caliceal diverticula to examine the presentation, natural history and management.We retrospectively reviewed the records of 22 children presenting with caliceal diverticula between 1983 and 2006. All pertinent clinical data were recorded, including demographics, imaging studies, treatment and clinical outcome.A total of 22 children had 23 caliceal diverticula. There were 14 girls and 8 boys who presented at a mean age of 5.4 +/- 3.1 years (range 0.2 to 12). At clinical presentation 10 patients had febrile urinary tract infection, 2 had hematuria, 2 had abdominal pain, 1 had flank pain and 8 were asymptomatic. Mean diameter of all caliceal diverticula was 2.2 +/- 1.7 cm. All patients underwent voiding cystourethrogram. Two patients (9%) had concomitant ipsilateral caliceal diverticula and vesicoureteral reflux. A total of 10 patients with caliceal diverticula (43%) were treated at a mean of 3.0 +/- 2.3 years after initial presentation due to symptomatic enlargement in 5, symptomatic calculus in 3, complicated abscess in 1 and urosepsis in 1. Treatment modalities included percutaneous ablation, open marsupialization/ablation, partial nephrectomy and laparoscopic marsupialization/ablation. There were no recurrences during a mean followup of 3.1 +/- 2.9 years (range 0.1 to 10.1) in these 10 patients. In the 13 caliceal diverticula (57%) that were observed mean followup was 6 +/- 3.7 years (range 1.2 to 10.5). These caliceal diverticula were stable in size and remained asymptomatic.Caliceal diverticula in children are rare. Most caliceal diverticula remain stable and asymptomatic but approximately 20% may have symptomatic enlargement that may warrant surgical management. Given the morbidity associated with caliceal diverticula and concomitant vesicoureteral reflux, evaluation for ipsilateral reflux is mandatory. Overall approximately 43% of children with caliceal diverticula require surgical intervention, and various treatment options are available.

The purpose of this prospective study was to assess whether outcome of speech and language in children 5-10 years after corrective surgery for tetralogy of Fallot (TOF) or ventricular septal defect (VSD) in infancy was influenced by the preoperative condition of hypoxemia or cardiac insufficiency and whether it was associated with perioperative risk factors and neurodevelopmental outcome. A total of 35 unselected children, 19 with TOF and hypoxemia and 16 with VSD and cardiac insufficiency, operated with combined deep hypothermic circulatory arrest and low-flow cardiopulmonary bypass at mean age 0.7+/-0.3 (mean+/-standard deviation) years, underwent, at mean age 7.4+/-1.6 years, standardized evaluation of speech and language functions. Results were compared between subgroups and related to perioperative factors, sociodemographic and neurodevelopmental status. Age at testing, socioeconomic status and history of speech and language development were not different between the subgroups. In contrast, total scores on oral and speech motor control functions (TFS) as well as on oral and speech apraxia (Mayo Test) were significantly reduced (p

Surgical indications for correcting adolescent varicocele include high varicocele grade and testicular hypotrophy. To our knowledge these findings have not been correlated with semen parameters to date. We examined the relationship between unilateral left varicocele grade or the testicular volume differential and semen parameters in adolescents.Semen analyses were done in 57 Tanner stage V adolescent males at ages 14 to 20 years (mean+/-SD 18 +/- 1.6). Varicocele grade was determined by the attending urologist. Testicular volumes were determined by scrotal ultrasound performed by an attending sonologist. Data analysis was performed using nonparametric statistical methods.Boys with testicular volume differentials greater than 10% vs those with differentials less than 10% had significantly lower sperm concentration and total motile sperm counts. For differentials greater than 20% these decreases were more dramatic. Percent motile sperm was significantly lower for boys with volume differentials greater than 20% vs those in the less than 10% and 10% to 20% categories. No significant differences were detected in percent normal morphology among the volume differentials. No significant differences were detected for any semen analysis parameter as a function of varicocele grade.Sonographically derived volume differentials greater than 10% between normal and affected testes correlate with a significantly decreased sperm concentration and total motile sperm count. This finding may serve as a marker to identify adolescents with unilateral left varicocele who are at greatest risk for future infertility. At least semen analysis and close annual followup of these adolescents seem warranted.

Summary Background The pathogenesis of the chronic inflammatory skin disease hidradenitis suppurativa (HS, also known as acne inversa) involves epidermal alterations such as psoriasiform epidermal hyperplasia and keratin plugging. Keratinocytes are an important source of proinflammatory molecules in inflammatory skin diseases and can be stimulated by interleukin (IL)-17+ cells. Objectives To explore the possible role of the epithelium in the pathogenesis of HS. Methods We performed immunohistochemical stainings and Western blot experiments to investigate the localization and expression of inflammation-associated molecules, including the cytokine IL-17, components of the inflammasome including caspase-1, and the endogenous danger-associated molecular pattern molecules S100A8 and S100A9 (calprotectin). To examine a possible effect of upregulated proinflammatory cytokines on the inflammatory infiltrate, differences in the cellular composition of perifollicular and deep dermal infiltrates were analysed. Results The number of IL-17+ cells is increased in lesional and perilesional HS skin. The epidermis produces proinflammatory molecules and shows an upregulated expression of components of the NLRP3 inflammasome, activated caspase-1 and expression of S100A8/S100A9. Additionally, the course of the inflammatory process in HS involves influx of innate immune cells, particularly IL-17-expressing neutrophils. Conclusions IL-17-producing cells are present in lesional and perilesional HS skin and may contribute to the initiation of inflammatory processes. Furthermore, the epidermis is a source of proinflammatory cytokines, shows inflammasome activation and expresses S100A8/S100A9, thereby possibly contributing to the propagation of inflammation. A massive influx of IL-17-expressing neutrophils is observed in the deep infiltrate.

Summary Background Sacral agenesis (SA) is a rare congenital condition that refers to the absence of part or all of two or more lower sacral vertebral bodies. It can be associated with neurogenic bladder dysfunction that does not necessarily correlate with the level of spinal or skeletal defect. Patients with SA should undergo urodynamic studies (UDS) to guide lower urinary tract (LUT) management. Objective This review aimed to update the present institutional experience since 1981 of this rare patient population with detailed, long-term follow-up of bladder and kidney function. Study design A single institution, retrospective, IRB-approved review was performed on patients born after January 1, 1981 with an isolated diagnosis of sacral agenesis without spina bifida, and followed with urologic involvement at Boston Children's Hospital. Records were reviewed for demographics, radiologic imaging, UDS including cystometrogram (CMG) and electromyography (EMG), surgery, and blood chemistries. Comparisons were made between groups of patients based on age at diagnosis, with specific focus on renal function and stability of neurogenic bladder lesion. Results Forty-three patients were identified: 23 female and 20 male. Thirty-seven children (86%) had a known age of diagnosis. Nineteen were diagnosed before 2 months old, including five who were diagnosed prenatally, 11 were diagnosed between 2 and 18 months, and seven were diagnosed after 18 months. All 43 had UDS, with 24 (55.8%) studied at the time of diagnosis ( Summary Table ). Twenty had serial full UDS, with 30% demonstrating neurourologic instability. None developed end-stage renal disease (ESRD) or required spinal cord detethering. Discussion Many children with SA appeared to be diagnosed prenatally or early in life; SA was mostly identified during evaluation of associated anomalies. Though UDS aid in urologic management, testing was not routinely utilized at the time of diagnosis. Conclusions This review of long-term follow-up in SA patients showed stable LUT and renal function, with minimal risk of progression to ESRD. Summary Table . Group 1 Group 2 Group 3 Group 4 Adjusted P-value Age at diagnosis 2–18 months >18 months unknown n 19 11 7 6 Most recent method of bladder emptying Voiding 7 (36.8%) 3 (27.3%) 2 (28.6%) 1.00 CIC 13 (68.4%)* 8 (72.7%) 5 (71.4%) 1.00 No hydronephrosis on recent ultrasound 13 (68.4%) 5 (45.5%) 3 (42.8%) 0.90 Recent serum creatinine (ng/dl) Median 0.4 0.4 0.55 0.91 IQR 0.3–0.5 0.4–0.7 0.4–0.8 0.91 *One patient utilized both CIC and voiding to empty.

Introduction Existing survey instruments for bowel dysfunction in the pediatric population are either parent-reported or focus on non-neurogenic bowel dysfunction. Objective The purpose of this study was to develop and validate an adolescent-reported survey to assess the severity of bowel dysfunction in spina bifida patients and examine its impact on quality of life (QOL). Study design We performed a cross-sectional study of patients in our Myelodysplasia Program, aged 11–17 years, with a history of constipation and/or fecal incontinence (FI) from November 2010 to June 2013. Control patients, aged 11–17 years, were recruited from the stone clinic. Exclusion criteria were lack of English fluency, insufficient reading skills, or an incontinent fecal diversion. A 29-item version of the Adolescent Fecal Incontinence and Constipation Symptom Index (A-FICSI) was developed with five domains (Figure). Test re-test reliability and correlation with the total global health-related QOL score from the Parkin survey were measured using the Pearson correlation coefficient. A factor analysis model with four-fold correlations was tested. Results Of the 65 study-eligible individuals approached, 25 (11 boys) completed the A-FICSI (median age 12.6 years, IQR 11.8–14.7 years) and 17 completed another survey on urinary incontinence (64.6% response rate). Twenty-one control patients with nephrolithiasis completed the A-FICSI. Nine of the 25 completed a second administration of the survey. The mean correlation between repeated administrations of the survey was r = 0.43. There was a significant negative correlation between severity of constipation (r = −0.299, p 0.30) in the predicted direction. Unstandardized residuals were 8.7% (95% CI 6.4–10.9%). Item reduction was performed on the 29-item instrument based on results of the factor analysis. The finalized instrument contained 21 items. Discussion This is the first adolescent-reported bowel dysfunction instrument to undergo formal psychometric assessment in the spina bifida population. The instrument demonstrated adequate reliability and the five-factor structure fit the data well. This study highlights the negative impact of bowel dysfunction on the QOL of spina bifida patients. It is limited by the moderate sample size that is a common issue in relatively rare diseases. Conclusion The A-FICSI possesses desirable psychometric properties for the measurement of bowel dysfunction in the spina bifida population. Figure. Conceptual model of the Adolescent Fecal Incontinence and Constipation Symptom Index. Figure options Download full-size image Download high-quality image (276 K) Download as PowerPoint slide

We compared urodynamic findings in patients who underwent prenatal closure of myelomeningocele with those of patients who underwent postnatal closure, and equivalent lower urinary tract evaluations.Urodynamic studies of 5 patients (2 boys, 3 girls) who underwent prenatal closure of myelomeningocele were compared to those of 88 patients with similar level lesions who underwent repair postnatally between 1979 and 2002.All 5 patients in the prenatally treated cohort had lower lumbosacral lesions on neurological examination. These patients displayed no evidence of electromyographic activity, indicating complete denervation of the external sphincter. In comparison 34 of the 88 patients in the postnatal cohort (39%) lacked sphincter activity at newborn examination, with similar findings noted at 1-year evaluation. In terms of bladder function all 5 patients in the prenatal cohort exhibited detrusor overactivity, compared to 33 of the 88 patients (38%) in the postnatal cohort at the newborn examination, with similar findings at 1-year evaluation.Fetal closure of myelomeningocele is associated with a higher incidence of complete denervation of the external urethral sphincter and detrusor overactivity compared to postnatal closure. Patients who undergo this novel procedure should undergo urodynamic studies in the immediate newborn period and should be under close postnatal surveillance to document possible tethering of the spinal cord, urinary incontinence and increased detrusor pressures.

Summary Background Patients with end-stage bladder disease can be treated with cystoplasty using gastrointestinal segments. The presence of such segments in the urinary tract has been associated with many complications. We explored an alternative approach using autologous engineered bladder tissues for reconstruction. Methods Seven patients with myelomeningocele, aged 4–19 years, with high-pressure or poorly compliant bladders, were identified as candidates for cystoplasty. A bladder biopsy was obtained from each patient. Urothelial and muscle cells were grown in culture, and seeded on a biodegradable bladder-shaped scaffold made of collagen, or a composite of collagen and polyglycolic acid. About 7 weeks after the biopsy, the autologous engineered bladder constructs were used for reconstruction and implanted either with or without an omental wrap. Serial urodynamics, cystograms, ultrasounds, bladder biopsies, and serum analyses were done. Results Follow-up range was 22–61 months (mean 46 months). Post-operatively, the mean bladder leak point pressure decrease at capacity, and the volume and compliance increase was greatest in the composite engineered bladders with an omental wrap (56%, 1·58-fold, and 2·79-fold, respectively). Bowel function returned promptly after surgery. No metabolic consequences were noted, urinary calculi did not form, mucus production was normal, and renal function was preserved. The engineered bladder biopsies showed an adequate structural architecture and phenotype. Conclusions Engineered bladder tissues, created with autologous cells seeded on collagen-polyglycolic acid scaffolds, and wrapped in omentum after implantation, can be used in patients who need cystoplasty.

The renal histologic changes associated with congenital ureteropelvic junction obstruction (UPJO) and the relationship to clinical imaging have not been well studied. In order to better understand the histologic alterations of congenital UPJO and their relationship with clinical imaging and outcomes, we examined renal biopsies from 61 patients undergoing pyeloplasty for congenital UPJO. Glomeruli were analyzed for various injury patterns and the tubulointerstitium was examined for tubular atrophy/simplification and fibrosis. Two methods were used to evaluate tubular mass: glomerular density and morphometric measurement of tubular size and density. Control specimens were obtained from age-matched autopsy specimens without renal pathology. Glomerular changes were identified in 73% of all biopsies and were present in a range from 1.7 to 91% of glomeruli in each patient. Overt tubulointerstitial changes were present in 26% of all biopsies. Fibrosis was noted to occur with tubulointerstitial changes in a significantly greater fraction of children over the age of 1 year (P=0.026). Increased glomerular density was associated with severe hydronephrosis (P45%), postoperative functional decline was predicted only by increased glomerular density. 20 biopsies without overt tubulointerstitial changes were analyzed morphometrically and showed a significant reduction in proximal tubular (PT) size, but unchanged density. Distal tubular (DT) size was unchanged in UPJO, but density was increased. The PT/DT ratio was therefore markedly decreased in UPJO (P0.5 mm and this was associated with greater degrees of tubulointerstitial changes and glomerular alterations. Congenital UPJO produces a variety of renal parenchymal changes, which may in part reflect abnormal development. Some of these alternations are seen in clinical imaging and may help predict outcomes, but there is significant discordance between conventional imaging and histological findings.

SummaryInterleukin (IL)-16 is involved in the regulation of the expression of several proinflammatory cytokines, i.e. tumour necrosis factor (TNF)α and interleukin (IL)-1β. The present study aimed to determine the prevalence of the −295 promoter polymorphism of the interleukin (IL)-16 gene in periodontal disease. A total of 123 patients with periodontal disease and 122 healthy controls were genotyped for the −295 IL-16 promoter polymorphism. Genotyping has been performed by PCR and restriction fragment length polymorphism (RFLP) analysis. The frequencies of alleles and genotypes as well of haplotypes within both study groups were compared using the Pearson χ2 test at a level of significance of 5% (P

Auditory evoked potential (AEP) measurements of two Florida manatees (Trichechus manatus latirostris) were measured in response to amplitude modulated tones. The AEP measurements showed weak responses to test stimuli from 4 kHz to 40 kHz. The manatee modulation rate transfer function (MRTF) is maximally sensitive to 150 and 600 Hz amplitude modulation (AM) rates. The 600 Hz AM rate is midway between the AM sensitivities of terrestrial mammals (chinchillas, gerbils, and humans) (80-150 Hz) and dolphins (1,000-1,200 Hz). Audiograms estimated from the input-output functions of the EPs greatly underestimate behavioral hearing thresholds measured in two other manatees. This underestimation is probably due to the electrodes being located several centimeters from the brain.

A 3-year-old Wirehaired Fox Terrier was presented to the University Veterinary Hospital, University College Dublin, for evaluation of chronic cough of 8-months duration. Bronchoscopy showed a severely dilated collapsed left principal bronchus filled with highly viscous white mucus. Cytologically, globular lipid-like material and round concentrically laminated crystalline structures were evident within the proteinaceous mucus. These findings resembled the calcospherites and granular caseous debris often observed in human tuberculous patients. A Ziehl-Neelsen-stained cytocentrifuged preparation of material obtained by bronchoalveolar lavage revealed a few acid-fast rods within macrophages, suggestive of tuberculosis. At necropsy, granulomas with caseous necrosis were present in the lung parenchyma, bronchial and mediastinal lymph nodes, liver, pancreas, and mesentery. Granulomas were adherent to both kidney capsules and to the diaphragm. Histologically, there was evidence of mild calcification within caseous granulomas, which was confirmed by von Kossa's stain. Using Ziehl-Neelsen stain, acid-fast rods were identified within granulomas; bacterial culture was positive for Mycobacterium bovis. The cytologic findings in this case have not been reported previously in dogs and demonstrate a possible correlation between tuberculosis and calcospherite-like bodies with caseous, globular material in bronchial mucus, similar to that described in human patients.

The experience from Boston in anterior urethral valves and diverticula over a 40-year period is described. Other articles describe experience with both ignoring and treating VUR, and ballooning of the foreskin and physiological phimosis. OBJECTIVE To review a series of children with anterior urethral valves and diverticula, to elucidate the pathophysiology and optimal management of this entity. PATIENTS AND METHODS Nine cases (all boys; 1963 to 2003) were reviewed retrospectively. RESULTS Seven of nine boys had bulbar diverticula. Continuity between Cowper's duct and the diverticulum was noted endoscopically in two and confirmed radiographically in one. Initially, open surgery was curative but more recently endoscopic management has been the procedure of choice. CONCLUSION This series indicates that the distal lip of a ruptured syringocele may function as a flap-valve, leading to anterior urethral obstruction. Advances in imaging and endoscopic instruments have altered the mode of presentation and management of this entity.

We examined the mechanisms responsible for technical failure of the endoscopic technique for correcting vesicoureteral reflux based on endoscopic anatomy.The study included 25 patients, 2 to 15 years old (mean age 8) who received 57 ureteral injections on 1 or more occasions which failed to correct reflux. Each endoscopic procedure for every patient was videotaped and endoscopic photographs were taken to document pretreatment and posttreatment anatomy. Based on the comparative endoscopic anatomical findings, failures were categorized as secondary to volume loss, mound displacement, mound extrusion or indeterminate etiology.Of the 57 ureters the cause of failure was believed to be due to mound displacement in 35%, volume loss in 23%, displacement and volume loss in 29%, mound extrusion in 2% and indeterminate in 11%. The direction of mound displacement was toward the bladder neck (medial, distal) in 76% of those cases.Endoscopic anatomical findings can account for failure of the injection technique to correct reflux in the majority of failed cases (90%). Mound displacement and volume loss account comparably for treatment failure. One incidence of mound extrusion, previously hypothesized as a mechanism for treatment failure, was documented in our study.

The overactive bladder (OAB) in children is defined as both involuntary detrusor contractions and urethral instability. The development of urinary control plays a key role in its incidence and in our understanding of its pathogenesis. It is seen in a number of conditions but by far is most common among patients with dysfunctional voiding. Urinary infection can be both a cause and an effect of OAB. In some instances, vesicoureteral reflux may result from detrusor overactivity because its successful resolution has been shown to depend on abolition of the hyperactivity. Early diagnosis and appropriate treatment can affect upper urinary tract function and drainage and ultimate bladder function. Recognition is noted via a thorough history and careful physical examination. Urodynamic assessment is indicated in neurologically normal children >5 years old and is combined with a voiding cystourethrogram in boys and a radionuclide cystogram in girls who have a history of recurrent urinary infection. Treatment consists of prophylactic use of anticholinergic agents in patients with neurologic dysfunction, as an early adjunctive measure in boys after ablation of posterior urethral valves, and in children with vesicoureteral reflux. The dose must be carefully titrated in children with cerebral palsy to prevent the appearance of elevated residual urine. Behavioral therapy and biofeedback techniques are effective alternatives to anticholinergic agents for children with dysfunctional voiding.

While intermittent ureteropelvic junction obstruction is well recognized clinically, intermittent obstruction at the ureterovesical junction has not been previously described. We herein present a case report of intermittent ureterovesical junction obstruction, characterized by intermittent hydroureteronephrosis on ultrasonography and symptoms of flank/abdominal pain, nausea and vomiting.

Summary Objective This pilot study evaluated the safety, feasibility, and usefulness of the Self-Cathing Experience Journal (SC-EJ), an online resource for patients and families to address issues and stigma surrounding clean intermittent catheterization (CIC). Modeled after previous assessments of the Cardiac and Depression Experience Journals (EJs), this project uniquely included patients and caregivers. We explored whether patients and caregivers would find the SC-EJ helpful in increasing their understanding of CIC, accepting the medical benefits of self-catheterization, improving hopefulness, and diminishing social isolation. Methods Patients seen in a tertiary urology clinic were asked to view the SC-EJ for 30 min and rate its safety and efficacy. The cross-sectional sample included 25 families: 17 surveys were completed by the patient and their caregiver, five by the patient only, and three by the caregiver only. Mean patient age was 15.7 ± 5.8 years (range 7–29 years). The patients were 64% female, and 72% used CIC due to neurological diagnoses. Results Mean overall patient satisfaction with the SC-EJ was moderately high (mean = 5, out of a 7-point Likert scale from 1 = not at all to 7 = extremely). Mean overall caregiver satisfaction was high (mean = 5.55) and was similar to caregiver satisfaction scores recorded in caregivers with children with congenital heart disease and depression (mean = 5.7 and mean = 5.75, respectively). No significant differences were noted in satisfaction between CIC patients and CIC caregivers or among caregivers of the three populations surveyed (CIC, Cardiac, and Depression). CIC patients and caregivers reported that SC-EJ viewing gave them a strong sense that others are facing similar issues (patient mean = 6.15, caregiver mean = 6.21) and that it was helpful to read about other families' CIC experiences (patient mean = 6, caregiver mean = 5.89). Discussion The SC-EJ appears to be safe, feasible, and useful to patients and families using CIC. Ratings from caregivers of CIC patients were similar to other cohorts of caregivers facing chronic childhood conditions. Despite beliefs that the EJ format best targets adults, high satisfaction ratings among patients suggest that the SC-EJ is acceptable and beneficial to children and adolescents. This web-based intervention can be a helpful clinical supplement in promoting healthy coping skills and a decreased sense of isolation among patients and families facing CIC. The unique integration of real patient and family experiences with accurate and vetted medical knowledge has the potential to enhance resiliency among viewers who use CIC. Table . Mean ratings and SDs for satisfaction and safety of the Self-Cathing Experience Journal by patients and caregivers and comparison of caregiver ratings from the cardiac and depression experience journals. a Patients using CIC ( n = 22) Caregivers of patients using CIC ( n = 20) Mothers of patients with cardiac disease ( n = 40) Caregivers of patients with depression ( n = 35) Comparison of CIC patients and caregivers ( p ) Comparison of caregivers (CIC, cardiac, depression) ( p ) Overall, how satisfied are you with the Self-Cathing Experience Journal? 5 ± 1.17 5.55 ± 1.15 5.7 ± 1.8 5.75 ± 1.66 0.1329 0.9070 Did you feel that the Self-Cathing Experience Journal was hurtful? 1.15 ± 0.37 1.35 ± 1.35 1.2 ± 0.7 1.43 ± 1.14 0.5076 0.6219 Did the Self-Cathing Experience Journal give you any sense that there are others facing similar issues? 6.15 ± 1.18 6.21 ± 1.08 6.7 ± 0.7 6.44 ± 0.91 0.8648 0.1103 Did you find it helpful to read about and see other families' experiences? 6 ± 1.20 5.89 ± 1.24 5.7 ± 1.5 6.06 ± 1.39 0.7718 0.5449 Did the Self-Cathing Experience Journal increase your sense of hope? 4.95 ± 1.70 5.11 ± 1.76 5.1 ± 1.9 5.44 ± 1.42 0.7661 0.6518 CIC = clean intermittent catheterization, used in self-catheterization. a Based on 1–7 Likert scale (1 = not at all, 4 = moderate, 7 = extremely).

The objective of this document created by the ICCS standardization subcommittee is to provide a uniform guideline on measurement, quality control and documentation of urodynamic studies in children.This guideline was created using expert opinion and critical review of the published literature on urodynamic studies in children. Currently no standardized guideline or level 1 data exists on the proper technique for this subject matter.The document provides a throughout explanation on how to approach a child who presents with lower urinary tract dysfunction, whether it be of neurogenic, anatomic or functional origin. Formation of an urodynamic question after a comprehensive history and physical examination is paramount in selecting the urodynamic study(ies) that will be most appropriate for each child. Appropriate application of each test with careful consideration of the needs of the child and family will provide the most accurate and reproducible results. Recommendations on how to execute each of the components of an urodynamic study as well as interpretation are included in the document.Urodynamic studies have become a major tool in evaluating lower urinary tract dysfunction in children. There are many subtleties in performing these studies in children in juxtaposition to adults; therefore, adaptations specific to children must be made to achieve accurate and reproducible results. Uniformity in how the studies are conducted from center to center will allow for healthier transparency and enhanced comparison of results in both clinical and research situations.

More sophisticated endoscopic instruments, combined with a better understanding of bladder and urethral pathology, have significantly improved the therapeutic approaches for both posterior urethral valves and ureteroceles. New generation lithotripters have allowed for a safe and efficient method of treating urinary calculi in children, which was once thought too injurious a process with first-generation machines. The rapidly advancing field of laparoscopy, aided by the development of more optically refined and diminutive instruments, has allowed for its application in a wide variety of surgical interventions in pediatric urology. The tubularized incised plate urethroplasty has challenged more traditional approaches to hypospadias repair and is now considered by many pediatric urologists to be the best approach for midshaft and distal hypospadias. The one-stage approach to exstrophy repair may hold the answer to improved continence without a formal bladder neck reconstruction. Finally, the field of tissue engineering leads the way to new advances in autologous biological substitutes in the surgically-challenged patient where there is a shortage of local tissues at the surgeon's disposal.

We evaluated the impact of tubularized incised plate urethroplasty on primary and repeat hypospadias repair.We retrospectively reviewed the medical records of all boys who underwent hypospadias repair at our institution during a recent 3-year period. The level of the hypospadias defect, technique of repair, primary repair versus reoperation, age at surgery and complications were recorded.A total of 520 hypospadias repairs were done from May 1996 through June 1999. We began to perform tubularized incised plate urethroplasty in November 1996. During the ensuing consecutive 32 months 181 primary and 25 repeat hypospadias repairs were done using this technique. Mean patient age at surgery was 22 months (range 3 months to 30 years). During the 6 months immediately before we began to use this method the Mathieu flip-flap procedure was the most commonly performed technique, accounting for 38% of all hypospadias repairs. In contrast, during the last 6 months reviewed tubularized incised plate urethroplasty accounted for 63% of all repairs, including 41 of 65 primary operations (63%) and 4 of 6 reoperations (67%), while no Mathieu procedures were performed. Postoperative followup was 6 to 38 months for tubularized incised plate repair. Overall meatal stenosis and a urethrocutaneous fistula developed in 1 and 14 boys, respectively (7% complication rate).Tubularized incised plate urethroplasty has become the preferred technique of primary and repeat hypospadias repair at our institution. The technique has few complications as well as proved success and versatility that continues to expand its applicability and popularity.

Testicular volume measurements obtained with the Prader and Rochester orchidometers were compared to those obtained using scrotal ultrasound. The ability of each orchidometer versus ultrasound in detecting volume differential between 2 testes and the accuracy of orchidometer measurement by a less experienced examiner to that of a urologist were compared.A total of 65 males were examined by the attending urologist and urology nurse using the Prader and Rochester orchidometers, and scrotal ultrasound was subsequently performed by an attending radiologist. Statistical analysis of the results was performed to determine the correlation of orchidometer measurements between examiners, as well as with ultrasound, and sensitivity and specificity of orchidometer and ultrasound in detecting defined volume differentials between testes of 10%, 15%, 20% and 25%.There was a strong linear relationship between testicular volume measurements using either orchidometer and ultrasound. To detect a defined volume differential as determined by ultrasound orchidometer sensitivity was weak, whereas orchidometer specificity was better. There was a strong correlation between orchidometer measurements of the urology nurse and attending urologist.Although the orchidometer remains valuable in assessing size of the individual testis, it is too insensitive to volume differentials relative to ultrasound to be used routinely to determine growth impairment. For this reason observation of an adolescent with varicocele should include an annual ultrasound assessment of testicular volume.

The split spinal cord malformation (SSCM) is an occult spinal dysraphism which causes tethering of the spinal cord. We performed a retrospective analysis of 15 patients who had split cord malformations (without associated open neural tube defect) who underwent both pre- and postoperative urodynamic studies (UDS) in order to determine if a significant percentage of these patients, even in the absence of overt urologic symptoms, had evidence of urologic dysfunction. Eleven patients presenting in early childhood and 4 patients presenting later in life are reviewed. Despite the lack of preoperative urologic symptoms in almost all patients, 73% of patients had voiding abnormalities on formal testing. UDS on these patients before and after surgery was a useful adjunct to perioperative management and decision making, helped define the success of surgery, and gave objective information for cases in which retethering was suspected.

High pressure dyssynergic voiding may result in irreversible damage to the urinary tract. Prophylactic therapy in the form of clean intermittent catheterization and anticholinergic medication may significantly decrease the incidence of upper urinary tract deterioration. Whether prophylactic therapy in the high risk bladder may also lead to improved long-term bladder dynamics prompted us to study the effect of early versus late treatment of bladder hypertonicity and detrusor-sphincter dyssynergia on the ultimate need for bladder augmentation.We retrospectively reviewed urological outcomes in patients with myelodysplasia who were at risk for urological deterioration within year 1 of life based on bladder sphincter dyssynergia and/or high filling or voiding pressure. We recorded the dates when high risk voiding dynamics were initially observed, and when intermittent catheterization and anticholinergic therapy were initiated. Patients in whom treatment began at the time a high risk profile was noted (prophylactic group seen between 1985 and 1990) were compared to controls with the same high risk voiding parameters who did not receive early therapy (observation group seen between 1978 and 1984 with therapy instituted 1 year or longer after high risk was noted). The number of augmentations performed in each group was indexed to the total number of years of followup in the 2 populations, respectively. Patients with less than 2 years of followup were excluded from further analysis.Of the 45 patients at risk clean intermittent catheterization and anticholinergic medication were immediately initiated in 18, while 27 were treated expectantly. Patients in the observation group were followed an average of 4.1 years (range 1.1 to 14) before clean intermittent catheterization and anticholinergic medication were started. Of the 27 children treated expectantly 11 (41%) required augmentation, whereas only 3 of the 18 (17%) treated prophylactically required enterocystoplasty. When the number of augmentations was indexed to total years of followup in each of the 2 groups (296 versus 156 years) patients in the expectant group were nearly twice as likely to require augmentation.Identification and early proactive treatment of the high pressure, dyssynergic lower urinary tract significantly decreases the need for bladder augmentation as children with neurogenic bladder secondary to myelomeningocele mature.

In order to investigate to what extent various determinants in the decision-making process for the treatment of severely ill incompetent patients are influenced by cultural and sociopolitical factors, 540 physicians in Austria, Germany (East and West), and Sweden, countries representing different healthcare systems, were surveyed using a self-administered questionnaire. It provided three case vignettes with different levels of information about the patient's treatment wishes in case of incompetence in a life-threatening situation. We found a general trend to a lower level of treatment in line with the patient's wishes when the information provided was more detailed. Remarkably, a substantial number of doctors did not comply with the patient's wishes. Ethical concerns and patient's wishes appeared as the most important factors whereas religious beliefs of the physician and hospital costs scored lowest. Because of the variability of treatment decisions and the importance of various factors determining the decision-making, an advance directive may be a feasible way of reducing the number of conflicts in critical situations. We recommend that ethical issues of clinical practice should be emphasized in the medical curriculum and in the training of physicians.