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We assessed the probability of bladder augmentation/diversion and clean intermittent catheterization in classic bladder exstrophy in a multi-institutional cohort.We included children born from 1980 to 2016 with bladder exstrophy and treated across 5 centers (exclusion criteria less than 1 year followup after birth, isolated epispadias, bladder exstrophy variants etc). Outcomes were probability of bladder augmentation/diversion after bladder closure and proportion of patients performing clean intermittent catheterization at last followup. Survival analysis was used.Of 216 patients 63.4% were male (median followup 14.4 years). Overall 4 patients (1.9%) underwent primary diversion and 212 underwent primary closure (72.6% in first week of life). After primary closure 50.9% underwent augmentation, 4.7% diversion and 44.8% neither. By age 18 years 88.5% underwent a bladder neck procedure (synchronous augmentation 27.3%). On survival analysis the probability of bladder augmentation/diversion was 14.9% by age 5 years, 50.7% by 10 years and 70.1% by 18 years. Probability of bladder augmentation/diversion varied significantly between centers (p=0.01). Probability of bladder augmentation/diversion was 60.7% 10 years after bladder neck procedure. At last followup of the entire cohort 67.4% performed clean intermittent catheterization. Among 95 patients with intact native bladders 30.5% performed clean intermittent catheterization (channel 72.4%). Among 76 adults without a diversion 85.5% performed clean intermittent catheterization (augmented bladder 100.0% clean intermittent catheterization, native bladder 31.3%). Fifteen patients underwent diversion (continent 8, ureterosigmoidostomy 5, incontinent 2).On long-term followup probability of bladder augmentation/diversion increased with age, with 1 in 2 patients by age 10 years and the majority in adulthood. Probability of bladder augmentation/diversion differed among institutions. Almost a third of patients, including adults, with a closed native bladder performed clean intermittent catheterization. Considering all adults only 14% did not perform clean intermittent catheterization.

Summary Background Patients with bladder augmentation (BA) are routinely counseled to irrigate their bladders daily. However, reports of adherence with this regimen are lacking. Objective To evaluate adherence to a bladder irrigation protocol and identify risk factors associated with adherence among adults with spina bifida (SB) and BA. Study design 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). Results 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). Conclusions 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. 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.

Subglottic stenosis (SGS) is a challenging disease to diagnose in neonates. Long-range optical coherence tomography (OCT) is an optical imaging modality that has been described to image the subglottis in intubated neonates. A major challenge associated with OCT imaging is the lack of an automated method for image analysis and micrometry of large volumes of data that are acquired with each airway scan (1 to 2Gb). We developed a tissue segmentation algorithm that identifies, measures, and conducts image analysis on tissue layers within the mucosa and submucosa and compared these automated tissue measurements with manual tracings. We noted small but statistically significant differences in thickness measurements of the mucosa and submucosa layers in the larynx (p

Summary Purpose 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. Methods 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: CKiDSCr, CKiDCys, CKiDSCr-Cys, ZappitelliSCr-Cys) and four adult formulas (18 + years: MDRDSCr, CKD-EPISCr, CKD-EPICys, CKD-EPISCr-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+ ( Results 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 (CKiDCys, CKiDScr, p ≤ 0.001), remained stable (CKiDSCr-Cys, p = 0.41), or increased by +2.7/year (ZappitelliSCr-Cys, p 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 Comment 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. Conclusions 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. Download : Download high-res image (188KB) Download : Download full-size image Summary Figure . Estimated glomerular filtration rate among 209 children and 164 adults with spina bifida calculated using different published formulas. Formulas for children included: CKiDSCr (blue), CKiDCys (green), CKiDCSCr-ys (black), ZappitelliSCr-Cys (red), adults: MDRDSCr (black), CKD-EPISCr (red), CKD-EPICys (green), CKD-EPISCr-Cys (blue). Curves were drawn using locally estimated scatterplot smoothing (LOESS function).

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.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.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).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.

BACKGROUND: Primary distal renal tubular acidosis (dRTA) is a rare disorder, and we aimed to gather data on treatment and long-term outcome. METHODS: We contacted paediatric and adult nephrologists through European professional organizations. Responding clinicians entered demographic, biochemical, genetic and clinical data in an online form. RESULTS: Adequate data were collected on 340 patients (29 countries, female 52%). Mutation testing had been performed on 206 patients (61%); pathogenic mutations were identified in 170 patients (83%). The median (range) presentation age was 0.5 (0-54) years and age at last follow-up was 11.0 (0-70.0) years. Adult height was slightly below average with a mean (SD score) of -0.57 (±1.16). There was an increased prevalence of chronic kidney disease (CKD) Stage ≥2 in children (35%) and adults (82%). Nephrocalcinosis was reported in 88%. Nephrolithiasis was more common with SLC4A1 mutations (42% versus 21%). Thirty-six percent had hearing loss, particularly in ATP6V1B1 (88%). The median (interquartile range) prescribed dose of alkali (mEq/kg/day) was 1.9 (1.2-3.3). Adequate metabolic control (normal plasma bicarbonate and normocalciuria) was achieved in 158 patients (51%), more commonly in countries with higher gross domestic product (67% versus 23%), and was associated with higher height and estimated glomerular filtration rate. CONCLUSION: Long-term follow-up from this large dRTA cohort shows an overall favourable outcome with normal adult height for most and no patient with CKD Stage 5. However, 82% of adult patients have CKD Stages 2-4. Importance of adequate metabolic control was highlighted by better growth and renal function but was achieved in only half of patients.

Waveform feature is one of the requirements for the FRIB LLRF controllers. It is desired that the LLRF con-trollers store the internal data (e.g. the amplitude and phase information of forward/reverse/cavity signals) for at least one second of sampled data at the RF feedback control loop rate (around 1.25 MHz). One use case is to freeze the data buffer when an interlock event happens and read out the fast data to diagnose the problem. An-other use case is to monitor a set of signals at a decimated rate (user settable) while the data buffer is still running, like using an oscilloscope. The detailed implementation will be discussed in the paper, including writing data into the DDR memory through the native interface, reading out the data through the bus interface, etc., Comment: Poster presented at LLRF Workshop 2019 (LLRF2019, 1909.06754)

Utbyggingen av vannkraftverk fikk en solid fremdrift etter andre verdenskrig. Spesielt i 60-årene startet flere nye kraftverker opp og flere av de er bygd i fjell. Teknikkene for driving av tunneler har endret seg de siste 70 årene. Høyere presisjon, mer effektiv drift og bedre sprengstoffer er noen av stikkordene for forskjeller ved tunneldriving nå mot før. Bruken av bly i sprengstoff er kanskje en av de viktigste forskjellene man bør merke seg. Eldre vannkrafttunneler ble ofte bygd for å opprette enn vannhastighet på 2-3m/s noe som ikke er optimalt etter dagens standard på 1 m/s. Det ligger store potensialer i å forbedre eksisterende tverrsnitt eller å anlegge en ny parallell tunnel ved denne type kraftverker. Tunnsjødal kraftverk som startet opp i 1963 faller under denne beskrivelsen. Vannhastigheten i tilløpstunnelen er opp mot 2 m/s, dette medfører ekstra store friksjonstap i tunneltverrsnittet. De eksisterende tunnelene er underdimensjonert med 1,9 ganger middelvannføringen, normalt for nye tunneler er 0,8-0,9 ganger middelvannføring. For en råsprengt tunnel med ujevn overflate opererer man med Manningstall rundt 30, og råsprengt normal for 35. Manningstallet ved Tunnsjødal er målt til 21 i tilløpstunnel og 17 i avløpstunnel. Dette ligger langt under akseptabelt nivå. Det lave Manningstallet er et resultat av underdimensjonert tverrsnitt og friksjonstap som følge av ruheten i tunneltverrsnittet og singulærtap fra store rasmasser og løsrevet dekke som blokkerer uniformiteten til vannstrømmen. Alternativer for reduksjon av dette friksjonstapet kan være lønnsomt på sikt. Hver cm av falltap i et kraftverk er et direkte tap av potensiell inntekt. Dette må igjen veies opp mot kostnadene en eventuell utbedring vil påløpe seg. I denne oppgaven er det gått nærmere inn på ny tunnel, strossing og rensk. Strossing av eksisterende tunnel til dobbel størrelse gir, ved Manningstall på 35, et falltap på 2,48 m. Det må påberegnes nedetid og tapt produksjon eller en fordeling av arbeidet over flere år for å unngå produksjonstap. Ved å danne en parallelltunnel vil det totale falltapet utgjøre 9,27 m for tunnelen. Dette er noe høyere enn ved utstrossing da vannmengden må fordeles over to tverrsnitt. Fordeler med parallelltunnel er kort nedetid av anlegg, redundans ved vedlikehold, reparasjoner og annet uforutsett. Falltapet kan reduseres med enda noen meter dersom rensk av eksisterende tunnel får høyere ruhet enn benyttet i beregningen. The construction of hydropower plants got a solid boost after WW2. In the 1960’s the building of new powerplants started and several of them where built inside of mountains. The techniques for tunneling have changed over the last 70 years. Better precision, higher efficiency and better explosives is some of the keywords when it comes to the differences in tunneling then and now. The use of lead in explosives is one of the most important differences one should note. Older hydropower plant tunnels were often built to maintain the water velocity at 2-3 m/s, which is suboptimal after todays standard of 1 m/s. There is a great potential in improving existing cross section or building a new parallel tunnel at these powerplants. Tunnsjoedal powerplant, which were put online in 1963, falls under this description. The velocity in the headrace tunnel is up to 2 m/s, this causes a large head loss when the water flows through the tunnel. The existing tunnels is under dimensioned with a target of 1,9 times the median water level, normal for new tunnels is 0,8-0,9 times the median water level. For a rough blasted tunnel with an uneven surface it is expected get a manning’s number of approximately 30, a rough blasted tunnel with a more even surface gives a manning’s number of approximately 35. The manning’s number in Tunnsjoedal is measured to 21 in the headrace tunnel and 17 in the tailrace tunnel. This is far from an acceptable number. The low manning’s number is a result of under dimensioned cross section and friction influences in the form of uneven surfaces and singular losses caused by large masses of rocks. In addition, there is also large pieces of old concrete and asphalt slabs that’s been ripped up from the flooring, laying around in the tunnel. Alternatives for reduction of head loss may prove profitable in the long run. Every inch of head loss in a powerplant is a direct loss of potential profits. This must be compared to the expenses of a possible improvement. In this paper the possibilities of a new parallel tunnel, expanding the existing tunnel and improving the existing tunnel is explored. Back ripping of the existing headrace tunnel up to double its original size results in a total head loss of 2,48 m including an increase of the manning’s number up to 35. Back ripping does involve downtime time and loss of production, unless the construction time is distributed over several years. By constructing a parallel headrace tunnel the total head loss will be 9,27 m. The manning’s number is set at 35 for the new and 30 for the old tunnel. The total head loss is higher than with back ripping because the water flows through two separate tunnels. Advantages such as reducing downtime for the powerplant during construction and being able to production during maintenance and revisions in one of the tunnels should be considered. Some additional meters of head loss can be cut if the scaling manages to increase the manning’s number up to 35 or higher.

OBJECTIVES: Posterior urethral valves predispose children to renal replacement therapy (RRT) and bladder dysfunction. Researchers of single-institutional series were unable to refine risk stratification because of rarity of the disease. We aimed to identify clinical variables associated with the risk of RRT and clean intermittent catheterization (CIC) in a large multicenter cohort study. METHODS: Children with posterior urethral valves born between 1995 and 2005 who were treated before 90 days of life at 5 children’s hospitals were retrospectively reviewed. Outcomes included RRT and recommendation for CIC. Predictors and outcomes were assessed by using survival analysis. RESULTS: A total of 274 patients were managed for a median of 6.3 years, and 42 progressed to RRT. On survival analysis, 16% progressed to RRT by 10 years of age. RRT varied by the serum nadir creatinine level in the first year of life (SNC1) (log-rank P < .001). After stratifying by the SNC1, the estimated risk of progressing to RRT by 10 years of age was 0%, 2%, 27%, and 100% for an SNC1 of CONCLUSIONS: Risk of RRT and CIC recommendation increased with age. The SNC1 strongly predicted need for RRT. These results allow for both improved family counseling and the potential for more appropriate screening and intervention strategies for those identified in higher-risk groups.

Spina bifida (SB) patients are at increased risk for hydronephrosis, bladder storage and emptying problems, and renal failure that may require multiple bladder surgeries.We retrospectively reviewed patients born with SB 2005-2009, presenting to our institution within 1 year of birth. Outcomes at 8-11 years old included final renal/bladder ultrasound (RBUS) results, clean intermittent catheterization (CIC) use, anticholinergic use, surgical interventions, and final renal function. We excluded those without follow-up past age 8 and/or no RBUS or fluoroscopic urodynamic images (FUI) within the first year of life. Imaging was independently reviewed by four pediatric urologists blinded to radiologists' interpretation and initial findings compared with final outcomes.Of 98 children, 62 met inclusion criteria (48% male, 76% shunted). Median age at last follow-up was 9.6 years. Upon initial imaging, 74% had hydronephrosis (≥ SFU grade 1), decreasing to 5% at 10 years (p 0.0001). Initially, 9% had ≥ SFU grade 3 hydronephrosis, decreasing to 2% (p = 0.13). CIC and anticholinergic use increased from 61% and 37% to 87% and 86%, respectively (p = 0.001 and p 0.0001, respectively). With follow-up, 55% had surgical intervention and 23% had an augmentation. Of children with a serum creatinine/cystatin-C at 8-11 years old, one had confirmed chronic kidney disease (stage 2).Despite initial high incidence of hydronephrosis, this was low grade and resolved in the first decade of life. Additionally, the 8-11-year incidence of kidney disease and upper tract changes was low due to aggressive medical management.

The effects of urinary incontinence on health related quality of life in adults with spina bifida is poorly understood. We determined which quantification method best captures bother with urinary incontinence. We also quantified the impact of urinary incontinence on health related quality of life.We surveyed an international sample of adults with spina bifida online from January 2013 through September 2014. We evaluated dry intervals (4 hours or greater considered social continence), quantity of urinary incontinence (a lot, medium, little, none) and number of undergarments worn daily (pads, pull-ups or disposable underwear). A 5-point Likert bother scale was used. We applied validated instruments, including QUALAS-A (Quality of Life Assessment in Spina Bifida for Adults) and the generic WHOQOL-BREF (WHO Quality of Life). We analyzed data using linear regression with a range of 0 to 100 for all outcomes.Mean age of the 461 participants was 32 years and 31.0% were male. Overall 26.5% and 51.8% of participants were dry for less than 4 hours and 4 hours or greater, respectively, while 21.7% were always dry. On multivariate analysis worse bother was predominantly determined by the quantity of urinary incontinence (a lot 32.9 and medium 16.2 vs little, p0.0001) rather than dry intervals less than 4 hours (7.21, p = 0.08) or number of undergarments (-2.2 to 4.2, p ≥0.43). Bladder and bowel health related quality of life was lower with higher quantities of urinary incontinence (a lot -31.2, medium -23.0 and little -17.2 vs none, p0.0001) but not for dry intervals less than 4 hours (-4.1, p = 0.14). Use of undergarments regardless of number was associated with lower health related quality of life (-10.2 to -15.4, p ≤0.001). Quantity of urinary incontinence was the main predictor of lower WHOQOL-BREF scores.We report that adults with spina bifida and urinary incontinence have lower health related quality of life than those who are dry. Self-reported quantity of urinary incontinence was the best predictor of bother and health related quality of life.

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 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.

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 of30 min, split function of30%, 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 Introduction Patients with spina bifida (SB) often have cognitive impairments making the difficult transition process to adult care even more challenging. Objective 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. Methods 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. Results 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). Discussion 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. Conclusion 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.

About 12.7 % of the German Federal State Mecklenburg-Vorpommern is covered by wetlands. They are predominantly drained by systems of open ditches. Only a small proportion about 2.8 % remained near-natural. If the water level of the receiving water is lower than the water level of the wetland, its discharge is realized gravitationally by an outlet construction like a weir or sill, either uncontrolled or regulated. In the opposite case, the water has to be elevated into the receiving water body by a pumping station. The interaction between ditches and groundwater depends significantly on saturated hydraulic conductivity of the soil substrate or aquifer. This key factor is also determining in re-wetting lowlands. In case of high hydraulic conductivity, a ditch impounding is sufficient to re-wet the area by subsurface flow processes. However, soils with a low conductivity require a water logging or even a flooding to be re-wetted. This is often the case in, for example, degraded wetlands and especially during summer conditions with high potential evapotranspiration., Около 12,7% федерального штата Мекленбург-Ворпоммерн, Германия покрыты водно-болотными угодьями. Они преимущественно дренируются системами открытых канав. Только небольшая часть этих территорий (2,8 %) сохранена в естественном состоянии. В основном болота осушены системами открытых траншей, сообщающимися с естественными водными объектами. В случае, когда уровень воды в водоёме-приёмнике ниже уровня воды в траншее, происходит сброс через регулируемый или нерегулируемый водослив. В противном случае, уровень подтопления регулируется при помощи насосов. Взаимодействие между траншеями и подземными водами существенно зависит от гидропроводности почвенного субстрата или водоносного горизонта. Более того гидропроводность является решающим фактором при выборе мероприятий для восстановления природного болотного ландшафта. В случае высокой гидропроводности запруднение траншей достаточно для восстановления заболоченной территории. Если же гидропроводность низкая, как в случае осушенных болот, необходимо заболачивание или даже затопление восстанавливаемой местности, особенно в летний период при высоком потенциальном суммарном испарении.

Significant numbers of young adults with chronic health conditions fail to transition.The aim of the study was to evaluate how ready transitioned urologic patients were for that process. Owing to the cognitive impairments frequently seen with spina bifida (SB), it is hypothesized that these individuals will be less prepared to transition their medical care to adult providers compared with their healthy counterparts.Participants included consecutive patients in the transitional SB clinic at the study institution and controls (college students without obvious physical disability or interest in healthcare-related fields aged 18-25 years). Both groups were administered the Transition Readiness Assessment Questionnaire (TRAQ) over a nine-month period. Five TRAQ domains assess 20 skills necessary to transition. Likert scale responses range from 1 "no, I do not know how" to 5 "yes, I always do this when I need to" (which the authors considered appropriate for transitioned patients). Demographics and the number of daily medications taken were collected. Patients and healthy controls were compared using (1) total and domain TRAQ scores, (2) the proportion of non-transitioned skills ("1"), and (3) fully transitioned skills ("5"). Non-parametric statistics were used.Forty-three unique SB patients (30.8% shunted, 46.5% female) and 100 controls were enrolled. Patients with SB were older than controls (21 vs 20 years, p 0.001). There was no gender difference between groups (p = 0.33). Transitioned patients and college students were fully transitioned only in the "Talking with Providers" domain (Figure). College students performed significantly better than patients in the domains of "Appointment Keeping" (p = 0.04) and "Tracking Health Issues" (p = 0.02). Transitioned patients were less likely to be interested in learning how to perform skills in the domains of "Appointment Keeping" and "Tracking Health Issues" (p 0.001 for both domains).The transition readiness of young adults with SB compared to healthy controls and other youths with chronic health conditions is described. The limitations include the small sample size, potentially limiting generalizability, and cross-sectional nature."Transitioned" patients with SB had lower TRAQ scores in some domains compared to healthy college students, who themselves had scores indicating that they were not fully ready for transition. Increased attention to transition readiness in people with SB is necessary, as even healthy young adults struggle with these tasks and are poorly prepared for transition.

Precise data for quasi-free photoproduction of η-mesons off the deuteron have been measured at the Bonn ELSA accelerator with the combined Crystal Barrel/TAPS detector for incident photon energies up to 2.5GeV. The η-mesons have been detected in coincidence with recoil protons and neutrons. Possible nuclear effects like Fermi motion and re-scattering can be studied via a comparison of the quasi-free reaction off the bound proton to η-production off the free proton. No significant effects beyond the folding of the free cross-section with the momentum distribution of the bound protons have been found. These Fermi motion effects can be removed by an analysis using the invariant mass of the η-nucleon pairs reconstructed from the final-state four-momenta of the particles. The total cross-section for quasi-free η-photoproduction off the neutron reveals even without correction for Fermi motion a pronounced bump-like structure around 1GeV of incident photon energy, which is not observed for the proton. This structure is even narrower in the invariant-mass spectrum of the η-neutron pairs. Position and width of the peak in the invariant-mass spectrum are W ≈ 1665 MeV and FWHM Γ ≈ 25 MeV. The data are compared to the results of different models

Summary Introduction Postobstructive diuresis (POD) after unilateral pyeloplasty or percutaneous nephrostomy (PCN) tube insertion for ureteropelvic junction obstruction (UPJO) in patients with a normal contralateral kidney is not well described. Objective The objective of this study was to determine the incidence and characteristics of POD after relief of unilateral UPJO in patients with a normal contralateral kidney. Study design Children who underwent a unilateral pyeloplasty or PCN for UPJO from 2010 to 2017 with a normal contralateral kidney were retrospectively reviewed. Postobstructive diuresis was defined as urine output (UO) of >300% of expected UO. Patients with a solitary kidney or those who underwent bilateral pyeloplasty or bilateral PCN tube placement were excluded. Results Out of 396 children meeting inclusion criteria, seven (1.8%) developed POD (4 after pyeloplasty and 3 after PCN tube placement). Median age at intervention was 1.7 years (range 11 days–18 years); median weight was 11.4 kg (range 3.7–54.2 kg). Postobstructive diuresis was more likely to occur in patients with grade 4 hydronephrosis (3.0%) and larger kidneys and if a PCN tube was placed before pyeloplasty. There was no significant difference in age, gender, kidney laterality, or function between those who developed POD and those who did not. Postobstructive diuresis was managed with additional intravenous fluids and electrolyte monitoring. Median initial postprocedure UO was 5.9 mg/kg/hr (range 3.2–10.0 mg/kg/hr). In five children who underwent PCN in whom UO could be differentiated between kidneys, median initial postprocedure UO was 6.1 mg/kg/hr (range 2.5–9.1 mg/kg/hr) from the affected side and 0.8 mg/kg/hr (range 0.4–0.9 mg/kg/hr) from the unaffected side. The median length of time to resolution of POD was 3 days (range 2–4 days). One patient developed significant acidosis and lethargy that improved with intravenous fluid management. Mild hyponatremia developed in two, hypokalemia in one, hypophosphatemia in one, acidosis in one, and hypoglycemia in 1 patient. Discussion A low but clinically significant risk of POD occurring after relief of unilateral UPJO in children with a normal contralateral kidney is described. Limitations include retrospective analysis and small sample size due to the rarity of the condition. Conclusion Postobstructive diuresis after decompression of UPJO in patients with a normal contralateral kidney is a rare event (1.8%). However, POD does occur, and patients should be carefully monitored after these procedures given the potential for significant dehydration and electrolyte disturbances. Table . Predictors of postobstructive diuresis. Factors affecting postobstructive diuresis No POD (N = 389) POD (N = 7) P-value Grade of hydronephrosis of the affected kidney before intervention, # (%) 3 or lower 166 (42.7%) 0 (0%) 0.04 4 223 (57.3%) 7 (100.0%) Median size differential of the affected compared to the unaffected kidney, cm (IQR) +1.4 (+2.4 to −0.8) +4.4 (+5.6 to −2.5) 0.02 Median size ratio of the affected to the unaffected kidney (IQR) 1.2 (1.1–1.4) 1.8 (1.5–2.1) 0.01 Need for nephrostomy tube before pyeloplasty, # (%) 23 (5.9%) 3 (42.9%) IQR, interquartile range; POD, postobstructive diuresis.

Subglottic stenosis is a severe and challenging disease to manage in neonates. Previous reports describe the usage of long-range optical coherence tomography (LR-OCT) to image the subglottis through an endotracheal tube and potentially identify subepithelial changes in subglottic mucosa which are correlated with edema or scar tissue. A major challenge associated with OCT imaging is that large volumes of data (1-2 GB) are acquired with each airway scan, with no existing automated method for image analysis and tissue measurement. We have developed an innovative MATLAB based auto-segmentation program which identifies and measures tissue layers within the mucosa. LR-OCT data sets of 21 neonates were analyzed for mucosal thickness of the proximal trachea, subglottis and larynx. The auto-segmentation measurements were compared with measurements from manual tracings by a single operator. We found statistically significant associations between the thickness of the mucosa (p

Hypospadias is a common birth defect. It is present in about 34.2 in 10,000 live births in North America. However, few studies have evaluated the immediate reoperation and readmission rates following elective repair.This study aimed to define the rates of readmission and reoperation following elective hypospadias repair, to improve pre-operative counseling on risks of the procedure.The Pediatric Health Information System (PHIS) was interrogated from 2004 to 2015 for all elective hypospadias repairs (ICD-9-CM code 58.45) performed in the ambulatory surgical setting. The following were then determined: age at initial operation, insurance status, race, presence or absence of readmission or reoperation within 30 days, presence of repeat hypospadias repair (same ICD-9 code), and presence or absence of another urethral operation (ICD-9 CM code 58.XX, excluding 58.45). Mixed effects logistic regression were then performed with dependent variables of 30-day repeat encounter, 30-day emergency department (ED) visit, 30-day readmission, or 30-day reoperation; and independent variables of age, race, ethnicity, and insurance status.The study identified 45,264 hypospadias repairs during 2004-2015 performed in 43 hospitals. Within 30 days of the procedures, 2826 (6.2%) had additional encounters in the ED at the same facilities, and 546 (1.2%) had readmissions. A total of 105 (0.2%) underwent second anesthetic within the first 30 days. With regards to a 30-day repeat encounter, odds of repeat encounter were significantly increased in patients aged5 years, Black and Asian patients, and those with Medicaid. Of the 4882 repeat encounters, 954 (19.5%) had discharge ICD-9 codes related to the penis, or to postoperative complications in general.This study described the epidemiology of clinical events occurring at the same tertiary children's hospital within the first 30 days following more than 45,000 hypospadias repairs. Limitations included a cohort generated from a single set of ICD-9 codes.Elective hypospadias repair had a low rate of readmission (1.2%) and reoperation (0.2%) within the first 30 days. Patients aged5 years, of non-white race, Hispanic ethnicity, and on Medicaid had significantly higher odds of 30-day repeat encounters.

Background Innovations in fish nutrition act as drivers for the sustainable development of the rapidly expanding aquaculture sector. Probiotic dietary supplements are able to improve health and nutrition of livestock, but respective bacteria have mainly been isolated from terrestrial, warm-blooded hosts, limiting an efficient application in fish. Native probiotics adapted to the gastrointestinal tract of the respective fish species will establish within the original host more efficiently. Results Here, 248 autochthonous isolates were cultured from the digestive system of three temperate flatfish species. Upon 16S rRNA gene sequencing of 195 isolates, 89.7% (n = 175) Gram-negatives belonging to the Alpha- (1.0%), Beta- (4.1%) and Gammaproteobacteria (84.6%) were identified. Candidate probiotics were further characterized using in vitro assays addressing 1) inhibition of pathogens, 2) degradation of plant derived anti-nutrient (saponin) and 3) the content of essential fatty acids (FA) and their precursors. Twelve isolates revealed an inhibition towards the common fish pathogen Tenacibaculum maritimum, seven were able to metabolize saponin as sole carbon and energy source and two isolates 012 Psychrobacter sp. and 047 Paracoccus sp. revealed remarkably high contents of docosahexaenoic acid (DHA) and eicosapentaenoic acid (EPA). Furthermore, a rapid and cost-effective method to coat feed pellets revealed high viability of the supplemented probiotics over 54 d of storage at 4°C. Conclusions Here, a strategy for the isolation and characterization of native probiotic candidates is presented that can easily be adapted to other farmed fish species. The simple coating procedure assures viability of probiotics and can thus be applied for the evaluation of probiotic candidates in the future.

Prior to the advent of maternal ultrasonography in the 1980s, children with significant congenital hydronephrosis requiring surgery presented symptomatically with abdominal pain, urinary infection, hypertension, hematuria, or failure to thrive. Antenatal hydronephrosis (AHN) became one of the most commonly detected ultrasound (US) findings, affecting 1–5% of pregnancies.1 The majority of AHN in the third trimester is discovered due to US for maternal indications. The benefits of this early detection of urinary tract dilation include a reduction in the renal damage due to obstruction and infection.2,3 On the other hand, many of these antenatally detected cases spontaneously resolve with observation and consequently can be submitted to unnecessary investigations and continued medical followup.4 The challenge to this day remains to predict which of these prenatally detected infants will require corrective surgery, prior to the development of symptoms or potentially irreversible renal damage, thus permitting a more tailored screening.

Tajikistan is often affected by atmospheric mineral dust. The direct and indirect radiative effects of dust play a sensitive role in the climate system in Central Asia. The Central Asian Dust Experiment (CADEX) provides first lidar measurements in Tajikistan. The autonomous multiwavelength polarization Raman lidar PollyXT was operated for 1.5 years (2015/16) in Dushanbe. In spring, lofted layers of long-range transported dust and in summer/ autumn, lower laying dust from local or regional sources with large optical thicknesses occurred.

Intronic GGGGCC repeat expansions in C9orf72 are the most common known cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS), which are characterised by degeneration of cortical and motor neurons, respectively. Repeat expansions have been proposed to cause disease by both the repeat RNA forming foci that sequester RNA-binding proteins and through toxic dipeptide repeat proteins generated by repeat-associated non-ATG translation. GGGGCC repeat RNA folds into a G-quadruplex secondary structure, and we investigated whether targeting this structure is a potential therapeutic strategy. We performed a screen that identified three structurally related small molecules that specifically stabilise GGGGCC repeat G-quadruplex RNA We investigated their effect in C9orf72 patient iPSC-derived motor and cortical neurons and show that they significantly reduce RNA foci burden and the levels of dipeptide repeat proteins. Furthermore, they also reduce dipeptide repeat proteins and improve survival in vivo, in GGGGCC repeat-expressing Drosophila Therefore, small molecules that target GGGGCC repeat G-quadruplexes can ameliorate the two key pathologies associated with C9orf72 FTD/ALS These data provide proof of principle that targeting GGGGCC repeat G-quadruplexes has therapeutic potential.