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Since its discovery in 1955, the incidence and geographical spread of reported Oropouche virus (OROV) infections have increased. Oropouche fever has been suggested to be one of the most important vector-borne diseases in Latin America. However, both literature on OROV and genomic sequence availability are scarce, with few contributing laboratories worldwide. Three reassortant OROV glycoprotein gene variants termed Iquitos, Madre de Dios, and Perdões virus have been described from humans and non-human primates. OROV predominantly causes acute febrile illness, but severe neurological disease such as meningoencephalitis can occur. Due to unspecific symptoms, laboratory diagnostics are crucial. Several laboratory tests have been developed but robust commercial tests are hardly available. Although OROV is mainly transmitted by biting midges, it has also been detected in several mosquito species and a wide range of vertebrate hosts, which likely facilitates its widespread emergence. However, potential non-human vertebrate reservoirs have not been systematically studied. Robust animal models to investigate pathogenesis and immune responses are not available. Epidemiology, pathogenesis, transmission cycle, cross-protection from infections with OROV reassortants, and the natural history of infection remain unclear. This Review identifies Oropouche fever as a neglected disease and offers recommendations to address existing knowledge gaps, enable risk assessments, and ensure effective public health responses. [ABSTRACT FROM AUTHOR]

Despite significant and known challenges to urinary and fecal incontinence (UI and FI, respectively) among children with spina bifida (SB) and their families, few studies have identified patient-centered measures and approaches to assessing them. This study represents the first stage of a larger study to develop a patient-centered goal-setting tool to guide incontinence management (Figure). Our aim was to understand patient experiences of UI and FI, and what goals should be included in the tool. We used a qualitative research approach integrated with human-centered design methods. We recruited, in clinic and online, children with SB (8–17 years old) and parents (>=18 years old) of children with SB (8–17 years old). Online activities were analyzed by four experienced design researchers using affinity diagramming, group analysis and modeling activities (mind maps, challenge maps, experience maps). Recruitment and thematic qualitative analysis continued until saturation was reached. Seventeen children with SB participated (9 female, 12 shunted, 13 using bladder catheterizations, 6 using Malone antegrade continence enema, median age: 15 years old). Fifteen parents participated (13 mothers/2 fathers, median age: 42 years old), including six mother-child dyads. Five major themes each were identified for UI and FI experiences: (1) negative emotional impact of incontinence, (2) unpredictability of incontinence, (3) challenging/unpleasant incontinence management, (4) inconvenient/unreliable incontinence management, (5) UI management having unpleasant complications and FI putting much responsibility on parents. We identified six UI goal domains and five FI goal domains. Four overlapping domains included: accidents, independence, interruptions at school and social/friends. Unique domains were: urinary tract infections (UI), catheterizations (UI) and enemas (FI). Findings of our study improve our understanding of children's and parents' experiences associated with incontinence in SB and potential continence goals. Children with SB experience incontinence as distressing and unpredictable. Incontinence management can be challenging and unreliable. Patient-centered continence goals cluster in domains, indicating how incontinence intermeshes with other life areas and offering potential approaches to structure continence goals in a goals-selection tool. [Display omitted] [ABSTRACT FROM AUTHOR]

To describe experiences of clitoromegaly in women with congenital adrenal hyperplasia (CAH). CAH females (46XX, ≥16 years old) from the United States and Canada were eligible for a cross-sectional online survey (2019–2020) if reporting clitoromegaly (life-long: "growing up with a larger than average clitoris," secondary: "clitoris grew over weeks or months"). A multidisciplinary team and women with CAH drafted questions assessing net effects of clitoromegaly on 10 activities and 10 life domains. Fisher's exact test was used to compare net effect (positive-negative) vs. no effect (Bonferroni p = 0.05/10 = 0.005). Of 97 women with CAH enrolled, 53 women (55%, median age: 36 years, advocacy group recruitment: 81%) reported recognizing clitoromegaly at median 11–13 years old, with 21% identifying it in adulthood. There was no difference in self-reported timing or clitoral shape between life-long or secondary clitoromegaly (p ≥ 0.06). There were no net positive effects of clitoromegaly. Rather, clitoromegaly had net negative effects on 7/10 activities (p ≤ 0.003) and no net effect (neutral) on 3 (Table). Women were less likely to wear tight clothing, change clothes in public locker rooms and play group sports. Women reported net negative effects for most romantic activities (dating, any sexual activity, pain-free sexual activity, having a partner see their genitalia, p=<0.003), but did not report a net effect on pleasurable sexual activity (p = 0.12). Clitoromegaly had net negative effects in 9/10 life domains (p < 0.001) and neutral on job self-perception (p = 0.25). Few women reported any positive impact (2–6%). However, 49–59% of women experienced poor self-esteem, anxiety, gender self-perception and body image, while 36% felt "down or depressed." Also, 21–23% experienced negative self-perception as friends and parents, 42–47% reported negative effects on plans for romantic and sexual relationships. Responses did not differ with advocacy group membership (p ≥ 0.02). Our findings support qualitative and case series evidence that clitoromegaly has a negative psychological outcome on women with CAH. Clitoromegaly may add to the burden of living with a chronic endocrine disease. Women with positive and negative experiences had the same opportunity to participate. Since we could not assess objective clitoral size, baseline virilization and exact nature of any childhood clitoral procedures, these data cannot be used to estimate the impact of specific clitoral size or effectiveness of early clitoral treatments. Clitoromegaly appears to be common among women with CAH. While experiences of clitoromegaly vary between women, the overall experience is negative in multiple social, romantic, and emotional activities and domains. [ABSTRACT FROM AUTHOR]

The Set Brave Goals app is the first digital health app (DHA) aimed at helping children with spina bifida (SB) aged 8–17 years old to select and track their urinary and fecal continence goals. Developed by children, parents, providers and researchers, its usability, or appropriateness to a purpose ("user-friendliness"), remains unknown. In an alpha testing stage, our aim was to determine the usability of the app prior to clinical use. We recruited children with SB and their parents in clinic and via social media. A recruitment goal of 10 children exceeded industry standard of 5 participants (10 participants are expected to identify approximately 96% of usability problems). Participants downloaded and used the app for a week. They completed a questionnaire, including the System Usability Scale (SUS) and closed and open-ended questions. The SUS is a 10-item validated usability questionnaire most widely used in DHA usability testing (scores range from 0 to 100, 100 representing greatest usability). Median/mean SUS scores for DHAs are 68. Mann-Whitney-U and t-tests were used. Ten children with SB participated (median age: 14 years old, 6 female, 8 shunted, 8 using bladder catheterizations). Twenty parents participated (17 mothers, median age: 42 years old). Median SUS score was 77.5 for children and 73.8 for parents, corresponding to "good" to "excellent" usability (Summary Figure). There were no significant differences between child/parent scores (p = 0.69) or those for other DHAs (p = 0.11). It took a median 5–10 min to go through the app (2 parents felt it took too much time). All participants felt the app was easy to understand and use (100%). Most participants would recommend it to children and parents (children: 89%, parents: 80%), and believed it would be valuable to children (90%, 75%) and urologists (80%, 80%). Free text responses related to more varied color schemes, keeping free-text notes within the app and more flexible goal/alarm setting. These changes were incorporated into the app. Usability testing prior to clinical launch identified areas for app improvement. Although this study sample met industry standards, findings are limited by a small group of participants. The app will undergo further refinement during prospective beta testing. The Set Brave Goals app for children with SB has acceptable usability parameters. This justifies proceeding with wider use to formalize children's continence goal setting/tracking and testing its value to children with SB and their healthcare providers. [Display omitted] [ABSTRACT FROM AUTHOR]

Parents are at risk of decision regret (DR) for decisions affecting their children. The Decision Regret Scale (DRS) measures medical DR but lacks context outside of healthcare. To compare parental DR 1) between common pediatric urologic surgeries and everyday decisions and 2) with preference to make a different choice. We conducted a cross-sectional online survey of randomly selected parents >1year (y) after their children underwent: orchiopexy (males ≤10y), open ureteral reimplant (OUR, females 2-6y), open pyeloplasty (OP, ≤2y), or robotic pyeloplasty (RP, 5-17y) (2017–2021). Higher DRS scores indicate increased DR (none: 0, mild: 1–25, moderate: 30–50, strong: 55–75, very strong: 80–100). Parents completed DRS on four decisions: their child's surgery, most recent/current romantic relationship, most recent leased/purchased car, and most recent purchased meal. Parents reported if they would make the same choice (yes/no). Nonparametric statistics were used. We surveyed 191 parents (orchiopexy n = 52, OUR n = 50, OP n = 51, RP n = 38). The median parent age was 36y (mothers: 86%). Some DR was reported for all decisions, but with significant differences in DR severity. The lowest median DRS score was seen with surgery (orchiopexy 0 [IQR 0–10], OUR 0 [IQR 0–5], OP 0 [IQR 0-0], RP 0 [IQR 0-0]), with no difference between surgery groups (p = 0.78). This was followed by relationship (0, IQR 0–20), car (15, IQR 0–25), and meal (20, IQR 0–30, p < 0.001). Most parents did not report any DR regarding surgery (orchiopexy 69%, OUR 74%, OP 76%, RP 76%, with no difference between surgery groups p = 0.85, Summary Figure). Comparatively, 59% of parents did not have any regret about their relationship, 37% their car, and 28% their meal (p < 0.001). All surgical DR was mild or moderate. No parent (0%) would have chosen differently for their child's surgery versus 4–12% for non-surgical decisions (p < 0.001). Overall, increasing DR corresponded to increasing desire to have made a different choice (DRS≤10: 0%, DRS 45–50: 32%, DRS 55–60: 66%, DRS≥75: 100%, p < 0.001). Parental DR varied between urological surgical and non-surgical decisions. It was lowest after surgery. Some regret was reported after every decision, but the subset of parents with regret was smallest after surgical decisions. Positive DRS scores do not necessarily correspond to parents wishing they made a different choice. [Display omitted] [ABSTRACT FROM AUTHOR]

The accuracy of self-reported urinary incontinence (UI) and fecal incontinence (FI) among adults with spina bifida (SB) is unknown. We aimed to quantify the accuracy of self-reported recall incontinence in the last 4 weeks using prospective diary data. Adults with SB were enrolled via patient advocacy groups in a larger 30-day smartphone-based ecological momentary assessment study of daily well-being and incontinence. We examined agreement between yes/no questions on exit questionnaires ("In the last 4 weeks, did you leak any urine and get your underwear, pads, pull-ups or disposable underwear wet?") and 30-day diaries. Collected data included a non-validated 4-item UI Negativity scale (UIN: 0–100, 0 = no impact). Inter-rater reliability was assessed with Cohen's kappa (>0.60 = substantial). FI was analyzed similarly. Sub-groups were too small for statistical analysis. Median age of 88 adults was 35 years old (70% female, 53% shunted, 71% community ambulators). Among 81 adults reporting UI in the last month, 79 (98%) had diary-documented UI (UI agreement), 2 (2%) did not (Summary Table). Among 7 adults reporting no UI in the last month, 5 (71%) did not record UI in their diaries (agreement in no UI), 2 (29%) did. Both adults reporting no UI on exit questionnaires, despite contrary diary data, experienced single UI episodes (negativity: 0 and 6). In contrast, the UI agreement group recorded UI on median 18 days (median negativity: 22). Sensitivity of the recall UI question was 0.98 (specificity 0.71, kappa 0.69). Among 66 adults reporting FI in the last month, 65 (98%) had diary-documented FI (FI agreement), 1 (2%) did not. Among 22 adults reporting no FI in the last month, 17 (77%) did not record FI in their diaries (agreement in no FI), 5 (23%) did. Five adults reporting no FI on exit questionnaires, despite contrary diary data, experienced a median single episode (median negativity: 8). In contrast, the FI agreement group recorded FI on median 5 days (median negativity: 31). Sensitivity of the recall FI question was 0.93 (specificity 0.94, kappa 0.81). Results were unchanged when analyzing the first and last 28 days of data. Sensitivity/specificity of single incontinence questions approach those reported for women with UI but without SB. Diaries may best serve SB adults with bothersome incontinence. Self-reported incontinence accurately and reliably captures UI and FI among adults with SB. It may minimize less bothersome incontinence, supporting its use in screening for clinical practice and research. [ABSTRACT FROM AUTHOR]

Estimated glomerular filtration rate (eGFR) in the general population is stable in children after 2 years of age until adulthood. In the first three decades after age 18, eGFR decreases by 0.3–0.8 ml/min/1.73 m2/year. Little data exists regarding eGFR changes in the spina bifida (SB) population given variability in muscle mass. In the absence of a validated SB-specific eGFR formula, the performance of different eGFR formulas may vary. We performed a cross-sectional study (1) to determine trends in eGFR with increasing age in children and adults with SB and (2) to compare eGFRs calculated using different formulas. We retrospectively reviewed records of patients 2–50 years old with SB followed at our institution (2014–2019). We determined eGFR using four pediatric formulas (2–17 years: CKiD SCr , CKiD Cys , CKiD SCr-Cys , Zappitelli SCr-Cys) and four adult formulas (18 + years: MDRD SCr , CKD-EPI SCr , CKD-EPI Cys , CKD-EPI SCr-Cys). One eGFR per patient was included (most recent eGFR for those with serial measurements). Patients were categorized as chronic kidney disease (CKD) stage 2 (eGFR 60-89) and Stage 3+ (<60). Non-parametric tests, linear regression, and Spearman's correlation were used for analysis. Among 209 children with SB (median age 10.3 years), depending on the formula used, eGFR decreased by −0.7 to −1.8 ml/min/1.73 m2/year (CKiD Cys , CKiD Scr , p ≤ 0.001), remained stable (CKiD SCr-Cys , p = 0.41), or increased by +2.7/year (Zappitelli SCr-Cys , p < 0.001) (Figure). The proportion of children with CKD 2 or higher varied between formulas (11.5–58.9%, p < 0.001). Correlations between pediatric formulas were negligible to moderate. Comparing any two formulas, 12.0–65.6% of children were assigned a different CKD stage. Among 164 adults (median age 26.3), eGFR decreased for each formula (range: −1.3 to −2.2/year, p ≤ 0.01) (Figure). The proportion of adults with CKD 2 or higher varied between formulas (9.2–30.5%, p < 0.001). Correlations between adult formulas were moderate to very high. Comparing any two formulas, 8.5–26.8% of adults were assigned a different CKD stage. We cannot reliably determine whether eGFR changed during childhood. Among adults, eGFR decreased with age for every formula evaluated at greater than twice the general population rate. Without a validated SB-specific eGFR formula, we are left with formulas providing different results. Estimated GFR among adults with SB appears to deteriorate at a higher rate than in the general population. Due to lack of precision of accepted eGFR formulas in the SB population, this may be a real phenomenon, an artifact of inaccurate eGFR formulas, or both. A validated SB-specific eGFR formula is needed. Summary Figure Estimated glomerular filtration rate among 209 children and 164 adults with spina bifida calculated using different published formulas. Formulas for children included: CKiD SCr (blue), CKiD Cys (green), CKiD CSCr-ys (black), Zappitelli SCr-Cys (red), adults: MDRD SCr (black), CKD-EPI SCr (red), CKD-EPI Cys (green), CKD-EPI SCr-Cys (blue). Curves were drawn using locally estimated scatterplot smoothing (LOESS function). Summary Figure [ABSTRACT FROM AUTHOR]

The impact of having a child with atypical genitalia due to a life-threating chronic medical condition like congenital adrenal hyperplasia (CAH) is poorly understood. The aim of the study was to determine parental stress and impact of CAH on parental decisions, including decisions regarding female genital restoration surgery (FGRS). The authors surveyed consecutive parents of girls with CAH ≤3 years presenting at a tertiary referral center for FGRS consultation (2016–2019). The survey was developed by three families of daughters with CAH and six clinicians. Nine potentially stressful past experiences were rated on a 6-point Likert scale ('not at all' to 'extremely' stressful). Overall parental stress and strain (broader negative consequences) were reported using validated instruments (Perceived Stress Scale and Caregiver Strain Questionnaire Short Form, respectively). Impact of CAH on past decisions about childcare, social interactions, and who changes diapers were also assessed. Non-parametric tests were used for analysis. Twenty-nine parents (median age: 32years) of 22 consecutive children participated (Prader 3/4/5: 59.1%/36.4%/4.5%). After the study, 20 girls (90.9%) underwent FGRS at a median age of 8 months. The most stressful experiences were having an adrenal crisis ('very much' stressful), waiting for the CAH diagnosis, and making sense of the diagnosis (both 'quite a bit') (Figure 1). Remaining issues were 'somewhat' stressful. Deciding whether to proceed with FGRS was ranked as the least stressful issue. Overall parental stress was similar to overall stress previously reported by spousal caregivers of stroke or heart failure survivors (P ≥ 0.15). Overall parental strain was similar to parents of adolescents receiving mental health counseling (P = 0.77). Congenital adrenal hyperplasia impacted decisions about babysitting, daycare, who changed diapers, and choosing a pediatrician (P ≤ 0.02), but did not impact parental social interactions (P ≥ 0.11). Diapers were typically changed by parents (100.0%) and grandmothers (50.0%). Parents anticipated that some individuals currently not allowed to change diapers would be allowed after FGRS: grandfathers (+18.2%), aunts/uncles (+27.3–32.8%), cousins (+18.2%), and family friends (+45.5%). The authors present the first assessment of parental stress with respect to different aspects of care of a daughter with CAH. Larger studies are required to determine if the parental stress associated with these experiences varies over time and how these stressors rank relative to each other through the child's development. Parents experience multiple stressors after having a daughter with CAH. Parental stress surrounding a decision about FGRS appears less severe than events pertaining to the diagnosis and medical management of CAH. Congenital adrenal hyperplasia impacts multiple parental decisions. [ABSTRACT FROM AUTHOR]

Objective: To validate a parental assessment of children's external genitalia scale for females (PACE-F) for girls with congenital adrenal hyperplasia (CAH) by adapting the validated adult female genital self-image scale.Methods: PACE-F was administered to parents of girls (Tanner 1, 2 months-12 years) with and without CAH. Final questions were determined by clinical relevance and psychometric properties (scores: 0-100). A reference range was established using 95% confidence interval among controls. Age-matched controls were compared to girls with CAH (1) <4 years old before and after female genital reconstruction surgery (FGRS), and (2) 4-12-year olds after FGRS. Nonparametric statistics were used.Results: Participants included 56 parents of 41 girls with CAH (median 3.9 years old, 97.6% FGRS) and 139 parents of 130 girls without CAH. Face and content validity was established by families, experts, and factor analysis. Internal consistency was high (Cronbach's alpha: 0.83). Population reference score range was 66.7-100. Ten consecutive girls had pre- and post-FGRS PACE-F scores. All scores improved at 4 months after surgery and all preoperative scores were below reference range and lower than controls (P = .0001). All postoperative scores were within reference range, no different from controls (P = .18). Scores for girls with CAH after FGRS aged 4-12 years were no different from controls (100.0 vs 88.9, P = .77) and 90.0% were in reference range, as expected (P = .99).Conclusion: We present a validated instrument for parental assessment of genital appearance in girls with CAH. We demonstrate improved parent-reported appearance after FGRS, with scores similar to age-matched controls. [ABSTRACT FROM AUTHOR]

While the Malone antegrade continence enema (MACE) facilitates bowel movements in patients with spina bifida (SB) and neuropathic bowel, little is known about its long-term use. We aimed to assess long-term MACE use and potential risk factors for disuse. All patients with SB who underwent MACE procedures at our institution were retrospectively reviewed. Main outcome was MACE disuse (no longer catheterizing the MACE for antegrade enemas) based on self-report on a clinic questionnaire, or medical record for patients last seen before introducing the questionnaire 5 years ago. Survival analysis used two timeframes: time after surgery (Analysis 1) and chronological age: accounting for older children reaching adulthood earlier (Analysis 2). Overall, 411 patients (54% female, 78% shunted, 65% augmented) underwent a MACE procedure at median 7.9 years old (median follow-up: 8.4 years). Thirty-three (8%) patients no longer used their MACE. Most common reasons for doing so were channel/stomal stenosis (61%) and excision at colostomy or other abdominal surgery (12%). Bowel management afterwards included oral agents ± enemas (55%), Chait tube (30%), colostomy (12%). After correcting for differential follow-up, 90% of participants used their MACE at 10 years and 87% at 15 years after surgery. Based on chronological age, 97% used their MACE at 15 years old, 92% at 20 and 81% at 30 (Summary Figure). On multivariate analysis, umbilical MACEs were 2.4 times more likely to be disused than right lower quadrant MACEs (p = 0.04). Without correcting for chronological age (Analysis 1), patients undergoing MACE surgery at older ages were more likely to stop MACE use (p = 0.03). However, after accounting for chronological age (Analysis 2), patients undergoing a MACE procedure at older ages were no more likely to stop its use (p = 0.47, Figure). Gender, SB type, shunt status, mobility status, bladder augmentation or a urinary catheterizable channel were not associated with stopping MACE use (p ≥ 0.10). Participants were regularly followed in multi-disciplinary SB clinics. We did not assess continence, satisfaction or long-term urinary channel use, making it premature to recommend optimal stomal locations. Most patients with SB followed by a multi-disciplinary team continue using their MACE; 1% stopped MACE use annually, particularly after adolescence. This strongly suggests it is an effective bowel management method and transitioning to self-care plays a role in maintaining long-term MACE use. Umbilical MACEs may be at high risk of disuse, but all people with a MACE can benefit from support as they transition to adult care. [Display omitted] [ABSTRACT FROM AUTHOR]

No tools exist to help children with spina bifida (SB) describe their incontinence concerns and to help their providers account for them when evaluating management success. Our aim was to understand patients', parents', and clinicians' preferences about how a SB continence goal-selection tool (MyGoal-C) should look and function, and to create a tool prototype. We used a qualitative research approach integrated with human-centered design methods. We recruited children with SB (8–17 years old), parents (≥ 18 years old) of children with SB (8–17 years old) in clinic and online, and urology healthcare providers at our institution. We surveyed children and parents, and conducted parent and provider Zoom-based prototyping sessions to iteratively design the app. Design researchers analyzed online activities using affinity diagramming, group analysis and modeling activities. Provider sessions were analyzed with qualitative thematic analysis based on grounded theory. Recruitment continued until saturation was reached. Thirteen children with SB participated (median age: 10 years old, 4 female, 9 shunted, 10 using bladder catheterizations). Thirty-seven parents participated (33 mothers, median age: 43 years old). Children and parents unanimously recommended an app and their comments generated 6 major theme domains: goal-setting process, in-app content, working toward goals, urology provider check-in, app customization, and using big data to improve future functionality. Twenty-one of the parents participated in 3 prototyping sessions with 6 breakout groups. The remaining 16 parents and 13 children then completed the Prototype Testing Survey, leading to a refined prototype and a visual flow map of the app experience (Figure). Feedback from 11 urology healthcare providers (7 female, 6 advanced practice providers) generated 8 themes: patient engagement/autonomy, clearly displaying results, integration into clinic workflow, not increasing clinical staff burden, potential clinician bias, parental involvement, limitations of the app, and future app adaptation. These cumulative data allowed for a construction of a final app prototype. Findings of our study lay the foundation for creating a goal-selection app that meets preferences and needs of children, parents, and providers. Next steps involve building the app, testing its usability and assessing it prospectively in a clinical setting. Children with SB and parents preferred an app to help them set and track continence goals. All stakeholders, including urology providers, offered complementary and mutually reinforcing feedback to guide the creation of an app prototype that would ultimately be integrated into a clinic visit. [Display omitted] [ABSTRACT FROM AUTHOR]

Data on sexual function of men with spina bifida (SB) is limited. We aimed to assess sexual activity and erectile dysfunction (ED) in a large international sample of men with SB. Men with SB (≥18yo) were recruited in an international online survey via clinics and social media. We collected data on demographics, ambulation (Hoffer classification), penile rigidity (Erection Hardness Score), sexual activity and ED (International Index of Erectile Function). Non-parametric tests were used. A total of 162 men (median age 35, 62% shunted, 38% community ambulators) reported sexual desire similar to the general population (p = 0.82), but 55% were dissatisfied with their sex life (Summary Table). Overall, 36% reported full penile rigidity with erections, more commonly with better ambulation (p = 0.01), 69% had ever experienced orgasm and 84% ejaculated. In 44 men (27%) attempting sexual intercourse in the last 4 weeks, 59% had ED (11% severe, 7% moderate, 14% mild-moderate, 27% mild). In this group, 91% of men reporting less than full penile rigidity had ED, compared to 30% with full penile rigidity (p = 0.001). Overall, partnered non-genital contact in the last 3 months was reported by 56%, solo masturbation: 62%, partnered intercourse: 48% (31% vaginal). Of 54 men who used phosphodiesterase type 5 inhibitors (PDE5I), 80% reported improved erections, 56% improved intercourse. Overall, 40% reported non-genital erogenous zones as most pleasurable, especially with poorer ambulation (p = 0.002, chest/nipples: 73%). Strengths of this study include anonymous, voluntary, online participation maximizing participation of a heterogenous, international population. Whenever available, we compared findings to published values for the general population. Since romantic and sexual activity is a complex intersection of interest, opportunity and ability, a more comprehensive assessment was beyond the study's scope. Future work will focus on the interplay with issues like incontinence. ED was frequent among men with SB, especially in men with poorer ambulation. PDE5 inhibitors may be beneficial. Partnered sexual activity was reported by half of the men, although it may not involve penetrative intercourse. Summary Table Sexual activity and function. Summary Table Outcome Nonambulators (n = 81) Household ambulators (n = 19) Community ambulators (n = 33) Normal ambulators (n = 29) p-value Overall (n = 162) Sexual desire (median, IRQ) (IIEF items 11 and 12) 7 (6–9) 8 (5–9) 7 (6–9) 7 (6–8) 0.97 7 (6–9) Overall sex life satisfaction, regardless of sexual activity in the last 4 weeks Very satisfied 4 (4.9%) 2 (10.5%) 4 (12.1%) 5 (17.2%) 0.06 15 (9.3%) Somewhat satisfied 9 (11.1%) 1 (5.3%) 9 (27.3%) 3 (10.5%) 22 (13.6%) Equally satisfied and dissatisfied 14 (17.3%) 2 (10.5%) 3 (9.1%) 5 (17.2%) 24 (14.8%) Somewhat dissatisfied 14 (17.3%) 7 (36.8%) 5 (15.2%) 8 (27.6%) 34 (21.0%) Very dissatisfied 35 (43.2%) 5 (26.3%) 9 (27.3%) 6 (20.7%) 55 (34.0%) Missing 5 (6.2%) 2 (10.5) 3 (9.1%) 2 (6.9%) 12 (7.4%) Erection hardness Does not get bigger 13 (16.1%) 1 (5.3%) 0 (0.0%) 2 (6.9%) 0.003 16 (9.9%) Bigger but not hard 3 (3.7%) 1 (5.3%) 0 (0.0%) 0 (0.0%) 4 (2.5%) Hard but not hard enough for penetration 19 (23.5%) 1 (5.3%) 4 (12.1%) 3 (10.3%) 27 (16.7%) Hard enough for penetration but not completely hard 22 (27.2%) 7 (36.8%) 10 (30.5%) 8 (27.6%) 47 (29.0%) Completely hard and fully rigid 17 (21.0%) 9 (47.4%) 17 (51.5%) 15 (51.7%) 58 (35.8%) Missing 7 (8.6%) 0 (0.0%) 2 (6.1%) 1 (3.5%) 10 (6.2%) Romantic and sexual activity in the past 3 months Any non-genital partnered contact 36 (44.4%) 10 (52.5) 25 (75.8%) 20 (69.0%) 0.01 91 (56.2%) Cuddled 30 (37.0%) 9 (47.4%) 24 (72.7%) 16 (55.2%) 0.01 79 (48.8%) Kissed 33 (40.7%) 8 (42.1%) 24 (72.7%) 16 (55.2%) 0.02 81 (50.0%) Held hands 35 (43.2%) 10 (52.6%) 25 (75.8%) 19 (65.5%) 0.01 89 (54.9%) Masturbated alone 50 (61.7%) 14 (73.7%) 18 (54.6%) 18 (62.1%) 0.62 100 (61.7%) Used a sex toy 7 (8.6%) 5 (26.3%) 5 (15.2%) 9 (31.0%) 0.02 26 (16.1%) Tested or treated for sexually transmitted infections 2 (2.5%) 1 (5.3%) 1 (3.0%) 3 (10.3%) 0.25 7 (4.3%) Any partnered intercourse 29 (35.8%) 6 (31.6%) 24 (72.7%) 18 (62.1%) <0.001 77 (47.5%) Touched partner's genitals 26 (32.1%) 6 (31.6%) 24 (72.7%) 17 (58.6%) <0.001 73 (45.1%) Partner touched my genitals 21 (25.9%) 6 (31.6%) 22 (66.7%) 16 (55.2%) <0.001 65 (40.1%) Gave oral sex 16 (19.8%) 4 (21.1%) 17 (51.5%) 16 (55.2%) <0.001 53 (32.7%) Received oral sex 16 (19.8%) 5 (26.3%) 13 (39.4%) 15 (51.7%) 0.01 49 (30.3%) Vaginal sex 12 (14.8%) 4 (21.1%) 19 (57.6%) 15 (51.7%) <0.001 50 (30.9%) Anal sex 5 (6.2%) 1 (5.3%) 2 (6.1%) 6 (20.7%) 0.12 14 (8.6%) Median age at first sexual contact (IQR) 18 (16–25) 18.5 (16–26) 19 (18–25) 20 (17–25.5) 0.63 19 (16–25) Full Table available in the Online Appendix. [ABSTRACT FROM AUTHOR]

We aimed to quantify end-stage kidney disease (ESKD) risk after infancy in individuals with myelomeningocele (MMC) followed by urology in the modern medical era and to assess if ESKD risk was higher after surgery related to a hostile bladder. We retrospectively reviewed patients with MMC followed by urology at our institution born ≥ 1972 (when clean intermittent catheterization was introduced) past 1 year of age (when mortality is highest, sometimes before establishing urology care). ESKD was defined as requiring permanent peritoneal/hemodialysis or renal transplantation. Early surgery related to hostile bladder included incontinent vesicostomy, bladder augmentation, detrusor Botulinum A toxin injection, ureteral reimplantation, or nephrectomy for recurrent urinary tract infections. Survival analysis and proportional hazards regression were used. Sensitivity analyses included: risk factor analysis with only vesicostomy, timing of surgery, including the entire population without minimal follow-up (n = 1054) and only patients with ≥ 5 years of follow-up (n = 925). Overall, 1029 patients with MMC were followed for a median of 17.0 years (49% female, 76% shunted). Seven patients (0.7%) developed ESKD at a median 24.3 years old (5 hemodialysis, 1 peritoneal dialysis, 1 transplantation). On survival analysis, the ESKD risk was 0.3% at 20 years old and 2.1% at 30 years old (Figure). This was ∼100 times higher than the general population (0.003% by 21 years old, p < 0.001). Patients who underwent early surgery for hostile bladder had higher ESKD risk (HR 8.3, p = 0.001, 6% vs. 1.5% at 30 years). On exploratory analyses, gender, birth year, shunt status and wheelchair use were not associated with ESKD risk (p ≥ 0.16). Thirty-year ESKD risk was 10% after early vesicostomy vs. 1.4% among children without one (p = 0.001). Children undergoing bladder surgery between 1.5 and 5 years old had a higher risk of ESKD. No other statistically/clinically significant differences were noted. Patients with MMC remain at risk of progressive renal damage throughout life. We relied on the final binary ESKD outcome to quantify this risk, rather than imprecise glomerular filtration rate formulas. Analysis was limited by few people developing ESKD, inconsistent documentation of early urodynamic findings and indications for bladder-related surgery. While ESKD is relatively uncommon in the MMC population receiving routine urological care, affecting 2.1% of individuals in the first 3 decades, it is significantly higher than the general population. Children with poor bladder function are likely at high risk, underlining the need for routine urological care, particularly in adulthood. [Display omitted] [ABSTRACT FROM AUTHOR]

We sought to evaluate long-term surgical urinary and bowel management in cloacal exstrophy (CE) in a multi-institutional study. We performed a cross-sectional study of people with CE and covered variants managed at five participating institutions. Those with <1 year follow-up or born with variants without hindgut involvement were excluded. Primary outcomes were methods of urinary and bowel management. Urinary management included: voiding via urethra, clean intermittent catheterizations (CIC), incontinent diversion and incontinent in diaper. Bowel management included: intestinal diversion (colostomy/ileostomy) and pull-through (with/without MACE). We evaluated three age groups: children (<10 years), older children (10 to <18) and adults (≥18). We assessed if management varied by age, institution or time (born≤2000 vs. >2000). A total of 160 patients were included (40% male). Median follow-up was 15.2 years (36% children, 22% older children, 43% adults). While 42% of children were incontinent in diapers, 73% of older children and adults managed their bladder with CIC, followed by incontinent urinary diversion (21%) (p < 0.001, Table). CIC typically occurred after augmentation (88%) via a catheterizable channel (89%). Among older children and adults, 86% did not evacuate urine per urethra and 28% of adults had an incontinent urinary diversion. No child or adult voided per urethra. Age-adjusted odds of undergoing incontinent diversion was no different between institutions (p = 0.31) or based on birthyear (p = 0.08). Most patients (79%) had an intestinal diversion, irrespective of age (p = 0.99). Remaining patients had a pull-through, half with a MACE. The probability of undergoing bowel diversion varied significantly between institutions (range: 55–91%, p = 0.001), but not birth year (p = 0.85). We believe this large long-term data presents a sobering but realistic view of outcomes in CE. A limitation is our data does not assess comorbidities or patient-reported outcomes. Rarity of volitional urethral voiding in CE forces the question of whether is a potentially unachievable goal. We advocate thoughtful surgical decision making and thorough counseling about appropriate expectations, distinguishing between volitional voiding and urinary and fecal dryness. In this long-term, multi-institutional study of patients with CE, 94% of older children and adults manage their bladder with incontinent diversion or CIC. Nearly 80% of patients, regardless of age, have an intestinal diversion. Given that no patients were dry and voided via urethra and 86% of older patients do not evacuate urine per urethra, these data bring into question what functional goals are achievable when performing reconstructive surgery for these patients. Summary Table Urinary and bowel management stratified by age. Summary Table Younger children (0–9.9 years old, n = 57) Older children (10.0–17.9 years old, n = 35) Adults (≥18 years old, n = 68) p-value Overall (n = 160) Urinary management Clear intermittent catheterizations (CIC) 18 (32%) 29 (83%) 46 (68%) <0.001 93 (58%) Per channel 16 (89%) 28 (97%) 39 (85%) 83 (89%) Per urethra 2 (11%) 1 (3%) 7 (15%) 10 (11%) Augmentation 12 (67%) 25 (86%) 45 (98%) 82 (88%) Incontinent diversion 15 (26%) 3 (9%) 19 (28%) 37 (23%) Incontinent into diaper 24 (42%) 3 (9%) 2 (3%) 29 (18%) Empties per urethra with Valsalva maneuver 0 (0.0%) 0 (0%) 1 (1%) 1 (0.6%) Overall: empties urine via urethra (CIC, incontinent, Valsalva) 26 (46%) 4 (11%) 10 (15%) <0.001 40 (25%) Bowel management Intestinal diversion 45 (79%) 27 (77%) 54 (79%) 0.99 126 (79%) Pull-through without a MACE 7 (12%) 4 (11%) 7 (10%) 18 (11%) Pull-through with a MACE 5 (9%) 4 (11%) 7 (10%) 16 (10%) [ABSTRACT FROM AUTHOR]

Objective: To develop and validate a self-reported health-related QUAlity of Life Assessment in Spina bifida for Children (QUALAS-C).Methods: We drafted a 27-question pilot instrument using a patient-centered comprehensive item generation and refinement process. It was administered to a sample of children 8-12 years old with spina bifida (SB) recruited online via social media and in person at an outpatient SB clinic (January 2013-September 2014). Healthy controls were recruited at routine pediatrician visits. Validation and final questions were determined based on clinical relevance, high loadings on factor analysis, and domain psychometrics. Children with SB also completed the validated generic Kidscreen-27 instrument.Results: Median age of 150 participants was 9.6 years (60.7% male, 72.7% Caucasian), similar to 46 controls (P ≥ .10). There were 97 online and 53 clinic participants (89.0% and 84.2% of eligible, respectively). Face and content validities of the 2-domain, 10-question QUALAS-C were established by patients, parents, and experts. Internal consistency and test-retest reliability was high for the Esteem & Independence and Bladder & Bowel domains (Cronbach's alpha: 0.72-0.76, ICC: 0.74-0.77). Correlations between QUALAS-C domains were low (r = 0.51), indicating that QUALAS-C can differentiate between two distinct health-related quality of life components. Correlations between QUALAS-C and Kidscreen-27 were also low (r ≤ 0.44). QUALAS-C scores were significantly lower in children with SB than without (P < .0001).Conclusion: QUALAS-C is a short, valid health-related quality of life tool for children with SB. It will be useful in clinical and research settings. [ABSTRACT FROM AUTHOR]

Highlights: [•] We modeled the odds of life threatening head, neck and spine injuries based on vertical roof crush in rollover motor vehicle crashes. [•] We used a generalized estimating equation to account for the correlated nature of two occupants in the same vehicle. [•] We used a matched case–control design to compare injured occupants to uninjured occupants based on roof crush. [•] Increasing roof crush measurements were statistically associated with higher odds of injury to the head, neck and spine. [ABSTRACT FROM AUTHOR]

Abstract: Aim of the study: In this study we examined the effects of Taiji on perceived stress and general self-efficacy (GSE), and investigated the mediating role of a Taiji-induced GSE increase on Taiji-related reduction of perceived stress. Materials and methods: 70 healthy participants were randomly allocated either to the Taiji intervention group or the waiting list control group. The intervention lasted for 12 weeks comprising two Taiji classes per week. Before, shortly after, and two months after the intervention, we assessed the degree of perceived stress and GSE in all participants by employing the Perceived Stress Scale (PSS) and the GSE-Scale. Results: Compared to controls, participants of the Taiji group showed a significantly stronger decrease of perceived stress and a higher increase in GSE from pre- to post-intervention assessment (PSS: p =0.009; GSE: p =0.006), as well as from pre-intervention to follow-up assessment (PSS: p =0.018; GSE: p =0.033). A mediator analysis based on a multiple regression approach revealed that a Taiji-related increase in GSE statistically mediated the reduction in perceived stress after Taiji as compared to baseline. Post hoc testing showed that the mediating effect of GSE was significant (p =0.043). Conclusions: Our findings confirm previously reported Taiji-related stress reducing and GSE enhancing effects with GSE increase mediating Taiji related reduction of perceived stress. [Copyright &y& Elsevier]

Abstract: Purpose: No method exists for predicting which child with vesicoureteral reflux (VUR) will have surgery. Our goals were to analyze practice patterns at a major pediatric center and to identify factors predisposing children to surgery for specific indications. Materials and methods: We analyzed a cohort of 3738 children presenting with primary VUR (1996–2005). Surgical indications included: 1) breakthrough urinary tract infection (UTI), 2) non-resolution over 3 years, 3) renal scan abnormality and 4) parent/surgeon preference. Logistic regression was applied to a random 60% subset of children. Validation in the remaining 40% was done using receiver operating characteristic curve analysis and the Hosmer and Lemeshow goodness-of-fit test. Results: Independent predictors of surgery included higher age at presentation, antenatal hydronephrosis (ANH), bilateral VUR and VUR grade. Predictors of surgery for a breakthrough UTI included female gender, increasing age, and bilateral and high-grade VUR. Girls were less likely than boys to be operated for renal scan abnormality or parent/surgeon preference. ANH was a predictor of surgery for decreased function and parent/surgeon preference. The model had fair discrimination (c-statistic = 0.68–0.76) and high calibration (p ≥ 0.24). Probabilities of surgery were calculated. Conclusions: Higher age at presentation, being followed for ANH, and bilateral and high-grade VUR are independent predictors of VUR-corrective surgery. Predictors of surgery vary with indication. Our methods allow comparison of urological practice patterns and outcomes between institutions by taking into account indications for surgery. [Copyright &y& Elsevier]

Objectives: Widespread implementation of health-related quality-of-life (HRQOL) measurement in prostate cancer practice and research requires concise instruments. With 50 questions, the full-length Expanded Prostate Cancer Index Composite (EPIC) is cumbersome to administer outside of studies focusing exclusively on HRQOL. To facilitate HRQOL measurement in a broad range of prostate cancer research and practice settings, we developed and validated an abbreviated version of the EPIC. Methods: The 50 questions that constitute the full-length EPIC-50 were evaluated to identify the items suitable for elimination while retaining the ability to measure the 5 prostate cancer-specific HRQOL domains of the EPIC-50. The resulting abbreviated version (EPIC-26) was validated using question responses from 252 subjects who had undergone brachytherapy, external beam radiotherapy, or prostatectomy for prostate cancer. The EPIC-26 internal consistency was measured by Cronbach''s α coefficient and reliability using test–retest correlation. Results: Using the high item–scale correlations, clinically relevant content, and preservation of domain psychometrics, 26 items were retained in the EPIC-26 from the 50 questions in the full-length EPIC-50. A high correlation was observed between the EPIC-50 and EPIC-26 versions for the urinary incontinence, urinary irritation/obstruction, bowel, sexual, and vitality/hormonal domain scores (all r ≥0.96). The correlations between the different domains were low, confirming that EPIC-26 retained the ability to discern the 5 distinct HRQOL domains. The internal consistency and test–retest reliability for EPIC-26 (Cronbach''s α ≥0.70 and r ≥0.69, respectively for all 5 HRQOL domains) supported its validity. Conclusions: EPIC-26 is a brief, valid, and reliable subjective measure of health quality among patients with prostate cancer and is suitable for measuring the HRQOL among patients undergoing treatment of early-stage prostate cancer. [Copyright &y& Elsevier]

Abstract: Recent studies suggest that the formyl-peptide-receptor-like-1 (FPRL1) plays an essential role in the inflammatory responses of host defense mechanisms and neurodegenerative disorders such as Alzheimer''s disease (AD). We therefore analyzed whether amyloid β1-42 (Aβ1-42) increased the activity of phospholipase D (PLD) via FPRL1, which is an enzyme involved in the secretion, endocytosis and receptor signaling. PLD activity was determined using a transphosphatidylation assay. The internalization of Aβ1-42 via FPRL1 was visualized using fluorescence microscopy and quantified by ELISA (Enzyme Linked Immunosorbent Assay). Determining receptor activity by extracellular-signal regulated kinases 1/2 (ERK1/2) phosphorylation and cAMP level measurement verified the Aβ1-42-induced activation of FPRL1. We were able to show that Aβ1-42 is rapidly internalized via FPRL1 in astrocytes and microglia. PLD was additionally activated by Aβ1-42 and via FPRL1 in rat glial cells. Furthermore, the ERK1/2 phosphorylation by FPRL1 agonists was dependent on the PLD product phosphatidic acid (PA). Together, these data suggest that PLD plays an important role in the regulation of Aβ1-42-induced endocytosis and FPRL1 receptor signaling. [Copyright &y& Elsevier]

Filum section (FS) has been used to treat tethered cord syndrome (on MRI or occult) in pediatric patients with refractory dysfunctional voiding (DV). While controversial, some groups have previously reported significant improvement in patients' symptoms after FS, even in the presence of a normal preoperative spinal MRI. Until recently, it was our practice to refer patients with DV to pediatric neurosurgery for evaluation, MRI, and possible FS. We report our experience with sacral nerve stimulator (SNS) placement for pediatric patients with refractory DV after failed FS with the primary outcome being complications and explantations. The secondary outcome being change in quality of life. We retrospectively reviewed all consecutive patients <18 years old who underwent second stage SNS placement after FS at our institution between November 2012 and December 2019. We abstracted rate of 2nd stage implantation, complication, and explantation. We also collected age-appropriate preoperative and postoperative 15-question quality of life questionnaires (PedsQL™). The PedsQL scores ranged 0–100, higher numbers correlating with higher quality of life. A paired t-test was used for statistical analysis. Overall, 23 children proceeded to the second stage SNS due to persistent symptoms following FS. Median age at SNS implantation was 10.3 years (IQR 7.5–11.8 years), and 13 were female (56.5%). There were no intraoperative complications. Median follow-up was 2.8 years (IQR 1.1–3.7 years). One patient had the SNS removed due to a need for MRI, one for resolution of symptoms, and 4 patients underwent lead revision for lead fracture and return of symptoms (17.4%). All 15 patients who completed both preoperative and postoperative PedsQL reported significantly improved overall scores after SNS (Figure). Median scores improved from 61.7 to 86.7 (p < 0.0001). Symptom relief in the child with refractory DV using SNS is not new. However, we sought to review our results in those with previous FS for tethered cord syndrome. While our study is limited by its small size, we assessed both surgical and patient-reported outcomes. Our patients' marked improvement in quality of life is similar to that of other reported pediatric SNS cohorts. SNS placement after FS is feasible, safe, and can result in significant improvement in overall quality of life. Given these findings, we have changed our practice to offer SNS in patients with refractory DV with a normal lumbosacral spinal MRI. For those with MRI abnormalities, we seek neurosurgical evaluation for FS (if deemed necessary) prior to considering SNS. [Display omitted] [ABSTRACT FROM AUTHOR]

To assess long-term APV and split-appendix MACE durability and to compare split and intact appendix APVs in a large patient cohort. This retrospective cohort study included consecutive patients ≤21 years old undergoing an APV at our institution (1990–2019). Main outcomes were stomal and subfascial revisions. Kaplan Meier survival and Cox proportional hazards analysis were used. A total of 339 patients underwent APV creation at a median 7.4 years old (41% female vs. 59% male; 37% umbilical stoma vs. 63% other). In total, 36 patients underwent a stomal revision and 19 a subfascial revision (median channel follow-up 6.3 years). On survival analysis, the risk of stomal revision of the APV was 9.1% at 5 years, 12.6% at 10 years and 16.5% at 15 years. Risk of subfascial revision of the APV was 5.1% at 5 years, 7.0% at 10 years and 8.2% at 15 years. A split-appendix APV was performed in 118 (34.8%) of 339 patients. They had a shorter follow-up compared to those with an intact APV (5.1 vs. 7.0 years, p = 0.03). After correcting for differential follow-up time, there was no significant difference between groups for stomal revisions (HR 1.11, p = 0.76) or subfascial revisions (HR 0.80, p = 0.67, Figure). Risk of APV stomal revision was independent of stomal location and age at surgery (p ≥ 0.37). Similarly, risk of subfascial APV revision was independent of stomal location and age at surgery (p ≥ 0.18). Risk of stomal revision for split-appendix MACE channels was 16.2% at 5, 10 and 15 years (similar to split-appendix APV and all APVs, p ≥ 0.26). Risk of MACE subfascial revision was 5.5% at 5 years, 5.5% at 10 years and 14.7% at 15 years (similar to split-appendix APV and all APVs, p ≥ 0.36). We focused on surgical complications, as these entail the highest morbidity, however, we did not assess non-surgical, percutaneous or endoscopic management which also impact long-term outcome and patient quality of life. We did not compare the outcomes of the split-appendix MACE to an intact-appendix MACE cohort, as this patient population was not captured in this review. The split-appendix technique has durable long-term results for both the APV and MACE channels, which are comparable to the technique utilizing the intact appendix. Channel complications occur over the channel's lifetime, as 1 in 8 APVs in the entire cohort underwent a stomal revision and 1 in 14 APVs underwent a subfascial revision at 10 years after surgery. [Display omitted] [ABSTRACT FROM AUTHOR]

Risks of nephrolithiasis after bladder augmentation in people with spina bifida (SB) remain unclear. Annual incidence of nephrolithiasis in the general population is 0.01% for 10–14 years old, 0.07% for 15–19 years old and 0.2% for 20–24 years old. Our aim was to assess the incidence and risk factors of nephrolithiasis in SB patients after augmentation. Patients with SB and augmentation followed at our institution were retrospectively reviewed (born ≥1972, surgery 1979–2019). Patients were screened annually with renal bladder ultrasound and abdominal radiograph. Main outcome was nephrolithiasis treatment. Kaplan–Meier survival and Cox proportional hazards analysis were used. Possible predictors were assessed using stepwise forward selection (variables with p < 0.1 on univariate analysis included in multivariate analysis). 427 patients with SB and augmentation were included (51.8% female, 74.9% shunted). Median age at augmentation was 8.5 years (median follow-up: 12.4 years, ileum segment: 81.0%, bladder neck procedure: 60.7%, urinary channel: 74.2%) and 28.8% developed bladder stones. Overall, 47 (11.0%) patients were treated for nephrolithiasis. After correction for differential follow-up, nephrolithiasis was treated in 7.3% at 10 years, 13.2% at 15 years, and 18.0% at 20 years (Figure). Patients presented with either a urinary tract infection (46.8%), on screening (44.7%), or pain (8.5%). Stones were treated percutaneously, endoscopically or by ESWL (63.8%/34.0%/10.7%, respectively). Most were calcium stones (58.3%). On multivariate analysis, compared to younger patients, patients augmented at ≥10 years of age had 1.84 times the risk of nephrolithiasis (p = 0.01). Nephrolithiasis was more common in those who developed bladder stones (HR = 3.00, p < 0.0001). Among those with both renal and bladder stones, bladder stones typically preceded nephrolithiasis (55.2%), were treated concurrently (31.0%) and 13.8% occurred after nephrolithiasis. Gender, wheelchair use, bowel segment used, MACE and skeletal fractures were not associated with higher nephrolithiasis risk (p ≥ 0.11). This study of a large cohort of SB patients with long-term follow-up highlights that the risk of nephrolithiasis is cumulative and related to bladder stone formation, age at augmentation and time since augmentation. An association with bladder stones suggests potential shared metabolic causes. The study's retrospective design likely led to underestimating the risk of nephrolithiasis by not capturing spontaneously passed stones. Approximately 1% of patients with SB develop nephrolithiasis annually after augmentation. Close long-term surveillance after augmentation is strongly indicated, as nephrolithiasis incidence in augmented patient with SB is at least 10 times higher than general population. Patients with bladder stones are especially at risk. [Display omitted] [ABSTRACT FROM AUTHOR]

While most paediatric urologists consider patients' quality of life (QOL) important, few actually measure this outcome. Our goal was to assess instruments used in the pediatric urology QOL literature, specifically looking at whether they captured QOL. We searched MEDLINE and EMBASE for articles with a self-described primary outcome of measuring QOL. All validated QOL instruments in the papers were analyzed by QOL instrument content experts. Instruments were classified as focusing on: Functioning or QOL (Table). The term Functioning focuses on performing activities. QOL captures person's perceptions about their position in life, informed by circumstances, functioning and conditions. QOL instruments were further subdivided into generic QOL, health-related QOL (HRQOL) and disease-specific HRQOL. Only direct patient self-reported QOL instruments were then assessed, since they are the most clinically useful, reliably assessing patients' own perception of their QOL. Forty-three publications met inclusion criteria (published 1999–2019). Most common conditions included urinary incontinence (16, 37.2%) and kidney transplantation (12, 27.9%). Overall, 22 unique instruments purporting to measure QOL were identified. Looking at the concepts measured by each instrument, nine instruments (40.9%) assessed Functioning. Nine instruments (40.9%) measured a combination of Functioning and QOL. Only the remaining 4 instruments (18.2%) assessed strictly QOL. The 13 instruments assessing any QOL focused on generic QOL (n = 4), HRQOL (n = 3) and disease-specific HRQOL (n = 6). Of the subset of four instruments assessing strictly QOL, and not Functioning, all had patient self-reported versions available: two generic QOL instruments (KINDL, KIDSCREEN), one generic HRQOL (DISABKIDS), and one disease-specific HRQOL (QUALAS). Thirteen of 43 studies (30.2%) employed more than one instrument. Thirty-eight studies (88.4%) used an instrument measuring Functioning, with 19 (44.1%) measuring only Functioning, not QOL at all. Twenty-four studies (55.8%) used an instrument measuring actual QOL, although 17 (39.5%) used a combined Functioning/QOL instrument. Only nine (20.9%) used a strictly QOL instrument (strictly HRQOL instruments: 4.7%). We present encouraging evidence of sustained interest in QOL research in pediatric urology and identify areas needing improvement. Selecting appropriate QOL tools requires a working knowledge of their various underlying meanings and purposes. Whether it adequately assess QOL must be considered. We discuss strengths and weaknesses of instruments and a practical approach to QOL instrument selection. Much of pediatric urology is grounded in improving QOL. Unfortunately, most studies published to date focus on Functioning, rather than young people's perception-based QOL. Future QOL studies should ideally employ validated instruments capturing patient-reported QOL. [Display omitted] [ABSTRACT FROM AUTHOR]

Antegrade continence enemas have transformed treatment and improved the quality of life in children with neuropathic bowel, refractory constipation and fecal incontinence. However, it can often be difficult to manage problems that arise with ACE flushes. We report the use of an online tool designed for nurses to help troubleshoot calls for problems associated with antegrade continence enema (ACE) flushes as well as update our algorithm for managing refractory constipation/fecal incontinence in a large single institution experience. We developed an online tool based on our management protocol for managing refractory constipation/fecal incontinence (Summary Figure). Patient frequency and bother was assessed prior to the intervention and at one month after the intervention using 5- and 4-point Likert scales respectively. Patient demographics, MACE/Chait information, type of difficulty, volume of flush, and use of additives were recorded. Nurses were also interviewed prior to using the tool and 14 months after its development with regards to taking these phone calls and the helpfulness of the tool. Over 14 months, the nurses received 22 patients calls via the nursing triage line regarding ACE flush problems and prospectively collected data. Half reported multiple episodes of fecal incontinence. Other complaints included no response to flush (8, 36.4%), occasional episodes of liquid fecal incontinence (2, 9.1%) and time of flush exceeding 60 min (1, 4.5%). While patients did not report decreased frequency of problems as a result of nurse troubleshooting using the ACE algorithm (2.5 vs. 2, p = 0.55), patients did report a significant improvement in their bother scores (4 vs. 2, p = 0.02). All but one patient reported that the recommendation was "some" or "a lot" helpful on follow up interview. The nurses all indicated that the tool helped "some" or "a lot." The antegrade continence enema is valuable in managing neurogenic bowel, refractory constipation, and fecal incontinence, however, some patients experience problems with flushes that can often be difficult to manage. Patients reported less bother with their bowel issues after using our algorithm for managing refractory constipation/fecal incontinence and nurses reported that the tool was helpful. [Display omitted] [ABSTRACT FROM AUTHOR]

The aim of this study was to investigate the relationship between COVID-19 diagnosis and the risk of developing a first-ever vascular event (VE) compared with the same risk in those with respiratory tract infection (RTI). This was a retrospective cohort study. This study using data from Disease Analyzer Database (IQVIA) included patients aged ≥18 years with at least one visit to a German practice during the index period. VEs were defined as cardiovascular or cerebrovascular events. Two cohorts were created: patients with a diagnosis of COVID-19 and those diagnosed with RTI. These were matched using propensity scores. Kaplan–Meier curves were created for the purposes of time to event analysis. A Poisson model was used to calculate incidence rates and derive incidence rate ratios (IRRs). A total of 58,904 patients were matched. There was no significant association between COVID-19 diagnosis and increased incidence of VE events among females (IRR [95% confidence interval (CI)]: 0.96 [0.82–1.11] and 1.30 [0.88–1.81]) or males (IRR, 95% CI: 0.91 [0.78–1.05] and 1.13 [0.80–1.62]). Overall, no significant association between COVID-19 diagnosis and incidence of VE was observed across age categories except for cardiovascular vascular events in the age category ≥70 years (IRR [95% CI]: 0.78 [0.67–0.94]). Overall, our study suggests that COVID-19 diagnosis was not associated with an increased risk of developing VE compared with RTI diagnosis. However, further research in a variety of healthcare settings and regions is needed to confirm these preliminary findings from our cohort, which is a good reflection of routine clinical practice in Germany. [ABSTRACT FROM AUTHOR]

To assess opinions of females with CAH, and parents of females with CAH, about designating this population "intersex," particularly in legislation about genital surgery during childhood. We conducted a mixed-methods (quantitative and qualitative) anonymous cross-sectional online survey of females with CAH (46XX, 16+years old) and independently recruited parents of girls with CAH (2019–2020) diagnosed in first year of life from the United States. A multidisciplinary CAH team drafted the survey in collaboration with women with CAH and parents. Fisher's exact test was used to compare female and parent responses. A qualitative thematic approach was used to analyze open-ended answers for emergent categories of reasons why CAH females should or should not be considered as intersex. Of 57 females with CAH participating (median age: 39 years, 75.5% of ≥25year olds had post-secondary degree), all had classical CAH and 93.0% underwent genital surgery at median 1–2 years old. While 89.5% did not endorse the intersex designation for CAH, the remaining 5.3% did (5.3% provided no answer, Summary Figure). Most CAH females (63.2%) believed CAH females should be considered separately in "any laws banning or allowing surgery of children's genitals" (19.3% disagreed, 17.5% neutral, 0.0% no answer). Most common themes identified by females with CAH not endorsing an intersex designation were: normal female internal organs, sex chromosomes, personal identity, genital appearance, issues with language, hormones, and those endorsing it: genital appearance, community/group experiences, topic complexity. Overall, 132 parents of females with CAH participated (parent/child median ages: 40/11 years, 81.7% of ≥25year olds had post-secondary degree). All children had classical CAH and 78.8% underwent surgery at median <1 year old. While 95.5% of parents did not endorse the intersex designation for CAH, 2.3% did (2.3% no answer), similar to females (p = 0.29). Most parents (81.1%) believed CAH females should be considered separately in legislation (9.1% disagreed, 6.1% neutral, 3.8% no answer), a slightly higher percentage than females (p = 0.01). Echoing previously published disagreement with clinically designating CAH females as intersex, majority of CAH females and parents oppose a legal intersex designation. Differing opinions among females and parents strengthen concern about a one-size-fits-all approach to legislation about childhood genital surgery. Differences in opinions between female and parent responses, while statistically significant, were relatively small. Majority of females with CAH and parents believe CAH should be excluded from the intersex designation, and should be considered separately in legislation pertaining to childhood genital surgery. [Display omitted] [ABSTRACT FROM AUTHOR]

Pediatric extrarenal malignant rhabdoid tumors (MRTs) are rare and aggressive (20% 5-year survival). Only 2 cases of bladder MRTs have been published. We report on a 9-year-old boy presenting with gross hematuria, palpable pelvic mass, and an obstructed, nonfunctional kidney. Evaluation was consistent with a 9.7 cm extrarenal MRT invading the bladder and prostate. He underwent a cystoprostatectomy, Indiana pouch continent urinary reservoir creation, and a left nephroureterectomy. A discrete 2.5 cm focus of renal MRT was found. To our knowledge, this is the first case of simultaneous perivesical and renal MRT. We review the current management of pediatric extrarenal MRTs. [Copyright &y& Elsevier]

To evaluate real-world energy consumption and tail-pipe emissions of current plug-in hybrid electric vehicles (PHEVs), we tested three PHEVs in different driving conditions. We considered measurements of emissions at different ambient temperatures and for various operating conditions regarding hybridization. The results show that the initial battery state-of-charge (SoC) determines the absolute CO 2 emissions as CO 2 emissions decrease with a higher proportion of electric driving. Therefore, the use of different hybrid driving strategies can have a strong influence on the overall fuel-saving potential. This does not apply to other pollutants such as NO x and particles, as the emission of these pollutants depends on the operating conditions of the internal combustion engine. RDE tests with a high proportion of electric driving show, in some cases, higher pollutant emissions than in tests with a low electric share. In general, the emission measurements done under real driving conditions show a higher variance compared to the test bench tests. In the case of PHEVs, this effect is further increased due to the different choice of driving modes and engine cold starts during electric driving. The variance of these parameters makes it difficult to estimate the emission benefits of PHEVs. • Emissions of three Plug-in hybrid electric vehicles (PHEVs) are measured on-road. • The choice of PHEV driving mode determines the energy and emission balance per trip. • NO x and particles do not correlate with the share of electric driving. [ABSTRACT FROM AUTHOR]

As robotic-assisted surgery becomes increasingly utilized for pediatric ureteropelvic junction (UPJ) obstruction, open surgeons have countered by using muscle-splitting, miniature (≤2 cm) incisions. To prepare for this type of incision during pyeloplasty, it is necessary to define the exact location of the UPJ. The use of retrograde pyelogram (RPG) at the time of pyeloplasty helps the surgeon to identify the exact location of UPJ, and thus be able to use a muscle-splitting, miniature incision for open pyeloplasty. We hypothesize that when performing a muscle-splitting, miniature incision open approach; preoperative RPG frequently changes the traditional pyeloplasty flank incision at the tip of the 11th or 12th rib. A retrospective review of open pyeloplasties performed by a single surgeon at our institution from 7/1/2010 to 12/31/2018 was performed to determine rate of use of RPG, open pyeloplasty incision location and to determine what factors are predictive of incisional site. 114 of 122 (93.4%) patients with 115 renal units had pyeloplasties with preoperative RPG performed. Of the 8 procedures without RPG, two had a pelvic kidney diagnosed prior to surgery, two had narrow ureteric orifices that were difficult to cannulate, and four had associated reflux. In 31/115 (27%) pyeloplasties the incision was changed from a standard incision position at the 11th or 12th rib to an alternative incision (i.e. extended muscle-transecting incision at the tip of the 11th or 12th rib, or to an alternate incision site including Gibson, McBurney's incision, or low anterior abdominal incision). 84/115 (73.0%) had a miniature (<2 cm) incision at the tip of the 11th or 12th rib. Grade IV hydronephrosis was a significant predictor for changing the traditional incision site (p = 0.02). Preoperative nephrostomy tube insertion was also associated with an increased likelihood of having an alternate incision (p = 0.04). Incision site was not significantly affected by age of the patient at surgery, patient sex, size of the affected kidney, T1/2 times of <30 min, split function of <30%, kidney length differential, or laterality. The consistent use of RPG prior to pyeloplasty helps surgeons to plan for a small muscle-splitting, miniature open incisions. In our experience, 27% of pyeloplasties required alternative incision sites based on the results of pre-operative RPG. Summary Fig. Intraoperative Retrograde Pyelogram: A low Gibson incision with a transverse skin line incision was made anteromedial to the iliac crest, in a muscle-splitting fashion. Figure [ABSTRACT FROM AUTHOR]

Core outcome sets (COS) represent a minimum set of outcomes to measure in a given condition to enable outcome comparison across studies. This cross-sectional study aimed to map the PROMs recommended in existing COS, as well as to document the outcome domains covered and overlaps across disease areas. The majority of the instruments (86.4%) were recommended in just one COS, 23 were recommended in 2 COS, 6 in 3 COS, 2 in 4 COS, and 3 in more than 4 COS (i.e. HAQ-DI, EQ-5D, and SF-36). [Extracted from the article]

Patients with bladder augmentation (BA) are routinely counseled to irrigate their bladders daily. However, reports of adherence with this regimen are lacking. To evaluate adherence to a bladder irrigation protocol and identify risk factors associated with adherence among adults with spina bifida (SB) and BA. Adults with SB after BA followed in a multidisciplinary clinic were identified (2017–2019). All patients or caregivers were taught the importance of and the technique for the bladder irrigation protocol prior to and after BA. Patient demographics (age, gender, ambulatory status, and presence of a caregiver in clinics) and surgical details (type of BA, age at surgery, length of follow-up, presence of a catheterizable channel, position of stoma, bladder neck surgery, presence of Malone antegrade colonic enema or ventriculo-peritoneal shunt, and number of stone surgeries) were obtained from the medical record. Patients reported other variables in a standardized clinic questionnaire. Answers were confirmed by health care providers. The variables included who performs clean intermittent catheterization (CIC), size of catheter, frequency of CIC, use of overnight catheterization, difficulties with CIC, number of UTIs, and continence per urethra and per catheterizable channel. Adherence to bladder irrigation was also assessed in the questionnaire. ‛Strict adherence' was defined as bladder irrigation performed ≥6 times/week with ≥120 mL of saline. For statistical analysis, a more lenient definition of ‛higher adherence' was used: bladder irrigation ≥2 times/week with at least 60 mL. ‛Lower adherence' was defined as ≤1 time/week or with less than 60 mL. Factors associated with ‛higher adherence' were assessed with non-parametric tests (Bonferroni-corrected p -value: 0.002). Adherence was assessed in 87 eligible patients (60.9% females; mean age of 28.8 ± 8.2 years). No patient (0.0%) reported ‛strict adherence', and 62 and 25 patients (71.3% and 28.7%) reported ‛higher' and ‛lower' adherence' to bladder irrigation, respectively. Nine patients (10.3%) in the ‛lower adherence' group did not irrigate at all. No variables were statistically significant on univariate analysis, including previous bladder stone surgery or having a channel (p ≥ 0.01). On exploratory analysis, higher adherence was only associated with self-catheterizations versus those performed by caregivers (76.7% vs 33.3%, p = 0.01). Adherence to a bladder irrigation protocol in adults with SB and BA is poor. A history of bladder stones requiring surgery and the presence of a catheterizable channel do not appear to affect adherence. It remains unclear why some patients are more likely than others to irrigate their bladders. Future work will focus on methods to improve adherence. Summary Table Adherence prevalence. Summary N = 87 Strict adherence (≥6/wk. + ≥120 mL) 0 (0.0%) Some adherence (≥2–5/wk. and ≥60 mL) 62 (71.3%) Poor adherence (≤1/wk. or ≤60 mL) 16 (18.4%) No adherence (no irrigation at all) 9 (10.3%) N = 87 Higher adherence (2 or more times/wk. with 60 mL or more each time) 62 (71.3%) Lower adherence (1 time/wk. or less with 60 mL or less each time) 25 (28.7%) mL, milliliter; wk., week. [ABSTRACT FROM AUTHOR]

Patients with spina bifida (SB) often have cognitive impairments making the difficult transition process to adult care even more challenging. The objective of this study was to identify what patient factors impact transition readiness in this population. It is hypothesized that young adults with SB with more healthcare exposure were more likely to report readiness to transition. Consecutive patients ≥ 18 years old in the adult SB clinic were evaluated using the validated Transition Readiness Assessment Questionnaire (TRAQ, 8/17-5/18). Five TRAQ domains assess 20 skills necessary to transition. Responses are 1 No, I do not know how"; 2 No, but I want to learn"; 3 No, but I am learning how to do this"; 4 Yes, I have started doing this"; 5 Yes, I always do this when I need to" (considered "fully transitioned"). Demographics, ambulatory status, shunt status, shunt revisions, number of medications, and prior bladder augmentation were assessed. Domain and total TRAQ scores were analyzed using non-parametric statistics and linear regression. Ninety-six patients (60.4% females, 70.8% shunted) participated at median age 25.5 years. Overall median TRAQ score was 4.0/5.0, indicating that patients were starting to transition. On univariate analysis, age >25 years was associated with higher TRAQ scores across every domain and overall (P ≤ 0.01). Female gender was also associated with higher TRAQ scores for "Appointment Keeping," "Tracking Health Issues" and overall (P ≤ 0.03). Race, ambulatory status, shunt status, number of shunt revisions, number of medications, and prior bladder augmentation were not associated with TRAQ scores (P ≥ 0.12). After adjusting for gender on bivariate analysis, age >25 years was associated with higher TRAQ scores for "Appointment Keeping," "Tracking Health Issues," "Talking with Providers," and "Managing Daily Activities" domains and overall (P ≤ 0.03), but not the "Managing Medications" domain (P = 0.07). Female gender was not independently associated with higher domain or overall TRAQ scores (P ≥0.10). The transition readiness of young adults with SB compared based on demographic factors and factors increasing healthcare exposure is described. Limitations include the small sample size, potentially limiting generalizability, as well as cross-sectional nature. Transitioning adults with SB had TRAQ scores indicating that they were not yet fully transitioned in terms of their healthcare-related behavior. Older age was the only factor associated with transition readiness, which was not impacted by other demographics. Increased attention to transition readiness and consideration of a longer transition process in this population is necessary. [ABSTRACT FROM AUTHOR]

To evaluate the prevalence of and risk factors for urinary fecal incontinence (UI, FI) during sexual activity (UIS, FIS) among adults with spina bifida (SB). An international online survey of adults with SB was administered through SB clinics and SB organizations via social media. Adults with a history of masturbation or partnered sexual activity were included. The primary outcome was ever experiencing UIS/FIS. Nonparametric tests and logistic regression were used for analysis. A total of 341 adults met inclusion criteria (median age: 36 years, 59% female, 52% shunted, 48% community ambulators). Baseline UI in the last 4 weeks was reported by 50% and FI by 41%. Nineteen (5%) had a urostomy. Eight (2%) had a colostomy. Overall, 93% had a history of partnered genital contact. Among adults without a diversion, UIS was more common than FIS (70% vs 45%, P <.001). Among adults without a urostomy, UIS was more common among women (76% vs 62%, P =.01) and those with baseline UI (84% vs 50%, P <.001). UIS was not associated with age, shunt, ambulatory, or catheterization status (P >=.32). On bivariate analysis, female sex and baseline UI were independent predictors of UIS (P <=.001). Among adults without a colostomy, FIS was associated with female sex (50% vs 39%, P =.046), baseline FI (59% vs 32%, P <.001), community ambulation (52% vs 40%, P =.04), but not age, shunt, or MACE status (P >=.27). On multivariate analysis, baseline FI was independently associated with FIS (P <.001). Among adults with UIS/FIS, 29% experienced UIS "almost always" to "always," compared to 5% for FIS (P <.001). Virtually all adults found UIS/FIS bothersome (>=96% for each), even when incontinence occurred "almost never." UIS/FIS mostly occurred before and/or during orgasm than afterward (P <.001). UIS was reported by 53% of adults with a urostomy (100% bothersome). FIS was reported by 38% of adults with a colostomy (100% bothersome). Incontinence during sexual activity is a common problem for men and women with SB. Baseline incontinence is an independent, but not absolute, predictor of both. While FIS is less frequent than UIS, both are virtually always bothersome. [ABSTRACT FROM AUTHOR]

Summary Purpose Despite devoting many resources to managing urinary and fecal incontinence (UI and FI) in children with spina bifida (SB), it remains unclear whether either is associated with lower health-related quality of life (HRQOL). We sought to determine the impact of UI and FI on HRQOL in this population. Materials and methods Children with SB (8–17 years) living in the United States were surveyed online and in SB clinics (2013–15). We evaluated incontinence over the previous 4 weeks using a UI dry interval (<4 h, ≥4 h), FI clean interval (<1 week, ≥ 1 week), and self-reported amount of UI and FI (for each: a lot, medium, a little, none). HRQOL was assessed with QUALAS, a validated SB-specific instrument. Linear regression was used. Results The mean age of 298 children was 12.5 years (52.0% male). Overall, 73.1% had UI, 52.3% had FI, and 43.6% had both. Adjusting for concurrent UI and FI, any UI was associated with lower HRQOL in 14–17 year olds ( p < 0.0001) and 10–13 year olds ( p = 0.048), but not 8–9 year olds ( p = 0.98) ( Figure ). All age groups reported lower HRQOL with FI ( p ≤ 0.0001). On multivariate analysis, UI and FI incontinence intervals were not associated with lower HRQOL for any age group ( p ≥ 0.58 and p ≥ 0.10, respectively). Higher quantities of UI were associated with ever-lower HRQOL, particularly in 14–17 year olds ( p ≤ 0.02). Any quantity of FI was associated with lower HRQOL in all ages. The findings did not change significantly on exploratory analysis correcting for demographic and clinical variables. Discussion We report the first evidence that incontinence matters to children and adolescents with SB. Being a cross-sectional study, we were unable to track HRQOL over time. A prospective study is required to assess if HRQOL impact of UI indeed changes as a child grows up and if improving incontinence with treatments improves HRQOL. Findings are similar to those reported in adults with SB and suggest that the concept of “social continence” based on time interval has no HRQOL relevance in children, adolescents or adults with SB. Conclusions UI is negatively associated with HRQOL in children with SB in an age-dependent fashion: starting in 10 year olds and increasing until 14 years. FI correlates with lower HRQOL regardless of age. Similar to findings in adults with SB, HRQOL is lower with increasing amounts of UI and not the length of a dry interval. FI impacts HRQOL uniformly, regardless of frequency or amount. Figure Independent effects of urinary and fecal incontinence on health-related quality of life (HRQOL) in children, adolescents and adults with spina bifida. HRQOL was assessed by QUALAS Bowel and Bladder domain. Negative score differences signify lower HRQOL. Those without incontinence served as controls within each age group. Results for adults were obtained from 383 adults with spina bifid living in the United States enrolled in a separate, previously published international HRQOL study (Szymanski [13] ). [ABSTRACT FROM AUTHOR]

Summary Purpose The role of female genital restoration surgery (FGRS) in girls with congenital adrenal hyperplasia (CAH) is controversial, with no long-term parent-reported outcomes available. Decisional regret (DR) affects most parents after their children's treatment of pediatric conditions, including hypospadias. We aimed to assess parental DR after FGRS in infancy or toddlerhood and explore optimal timing for surgery. Materials and methods One-hundred and six parents of females with CAH undergoing FGRS before 3 years old and followed at our institution (1999–2017) were invited to enroll online. Higher Decision Regret Scale (DRS) scores indicated greater DR (range 0–100). Participants also reported preferred FGRS timing relative to their surgery (earlier, same, later/delayed). Non-parametric statistical tests were used. Results Thirty-nine parents (median 4.4 years after FGRS) participated (36.8% response rate). Median age at FGRS was 9 months. Median DRS score was 0 (mean: 5.0). Overall, 20.5% of parents reported some regret (all mild-moderate) (Figure). Fewer parents reported DR after FGRS compared with published DR after hypospadias repair (50–92%, p ≤ 0.001) or adenotonsillectomy (41–45%, p ≤ 0.03). No parent preferred delayed FGRS. Seven parents (18.1%) preferred earlier surgery, especially when performed after birthday (80.0% vs. 8.8%, p = 0.004). Discussion We present the first report of validated long-term parent-reported outcomes after FGRS in infant and toddler girls with CAH. One limitation is that this is largely a single surgeon series. Reasons for the observed low levels of DR are likely multifactorial. Far from a definitive study, we aimed to provide parents willing to share about their experience an opportunity to do so. For that reason, selection bias may exist in our study. While parents with higher DR were potentially less likely to participate because of mistrust of the medical establishment, those with a negative experience may in fact be more likely to voice their opinions. A low participation rate was likely a result of the sensitive nature of FGRS, a desire for privacy, and inability to locate parents. A larger study will be required to assess how DR is affected by sexual function, genital appearance and complications, and DR among women with CAH. Conclusions Parents of females with CAH report low levels of DR after FGRS in infancy and toddlerhood. This appears to be lower than after other genital and non-genital pediatric procedures. When present, parental DR is usually mild. No parents preferred delayed surgery, even among those with DR. Some preferred earlier surgery. [ABSTRACT FROM AUTHOR]

Summary Purpose The need for mechanical inpatient bowel preparation (IBP) in reconstructive pediatric urology has come under scrutiny, secondary to literature demonstrating little benefit regarding outcomes. Starting in 2013, a majority of patients undergoing reconstructive procedures at our institution no longer underwent IBP. We hypothesized that outpatient bowel preparation (OBP) would reduce length of stay (LOS) without increasing postoperative complications after appendicovesicostomy surgery. Materials and methods An institutional database of patients undergoing lower urinary tract reconstruction between May 2010 and December 2014 was reviewed. Starting in 2013, a departmental decision was made to replace IBP with OBP. Patients undergoing an augmentation cystoplasty or continent ileovesicostomy were excluded because of insufficient numbers undergoing OBP. Patients undergoing IBP were admitted 1 day prior to surgery and received polyethylene glycol/electrolyte solution. A personalized preoperative OBP was introduced in 2013. Cost data were obtained from the Pediatric Health Information System. Results Sixty-seven patients met the inclusion criteria, with 30 (44.8%) undergoing IBP. There were no differences with respect to gender, age, presence of ventriculoperitoneal shunt, body mass index, glomerular filtration rate, preoperative diagnosis, operative time, and prior or simultaneous associated surgeries ( p ≥ 0.07). Patients undergoing an IBP had a longer median LOS (7 vs. 5 days, p = 0.0002) and a higher median cost (US$4,288, p = 0.01). Postoperative complications in both groups were uncommon and were classified as Clavien–Dindo grade 1–2, with no statistical difference (IBP 20.0% vs. OBP 5.4%, p = 0.13). No serious postoperative complication occurred, such as a dehiscence, bowel obstruction, or shunt infection. Discussion This is the first analysis of hospitalization costs and IBP, showing a higher median cost of US$4288 compared with OBP. The LOS was shorter with an OBP (figure), similar to a previous report. Similar complication rates between the groups add to the growing body of literature that avoidance of IBP is safe in pediatric lower urinary tract reconstruction. Being a retrospective review of a practice change, differences in care that influenced cost and LOS may be missing. Also, as the surgeons do not know if a usable appendix is initially present, our data may not extrapolate to all patients. Despite these potential limitations, our data support the safety of utilizing OBP in patients with a high likelihood of a usable appendix, including those undergoing a synchronous Malone antegrade continence enema via a split-appendix technique. Conclusion In patients undergoing an appendicovesicostomy, preoperative IBP led to longer LOS and higher costs of hospitalization. OBP was not associated with increased risks of postoperative complications. Summary Figure. Length of hospital admission for children and adolescents undergoing appendicovesicostomy. Summary Fig [ABSTRACT FROM AUTHOR]