Management of neurogenic bladder in patients with spinal cord injuries/disorders and end stage renal disease: a case series.

INTRODUCTION: Patients with spinal cord injuries/disorders (SCI/D) often suffer from bladder dysfunction, commonly referred to as neurogenic bladder or neurogenic lower urinary tract dysfunction (NLUTD). Standard urologic evaluation and management help to minimize complications such as vesicoureteral reflux, urinary tract infection, and nephrolithiasis. However, we have further encountered patients with more complex issues, such as chronic kidney disease (CKD), end-stage renal disease (ESRD), bilateral nephrectomies, and urinary diversion/augmentation surgeries. Of particular interest, there is a lack of standardized guidance for bladder management in SCI/D patients with ESRD. These patients are at high risk for urological complications and would benefit from codified bladder management strategies. CASE PRESENTATION: In this article, we present eleven unique cases of NLUTD with associated ESRD and discuss recommendations utilizing simple and commonly available clinical interventions. DISCUSSION: The inherently small population size of SCI/D patients with NLUTD and ESRD makes detailing a large sample size case series difficult. Future studies must aim to include a larger sample size as able, however, to better determine standardized protocols for chronic bladder management in SCI/D patients with NLUTD and ESRD. Experiences from this small case series are offered for consideration.

Long-term complications and outcomes of augmentation cystoplasty in children with neurogenic bladder.

Augmentation cystoplasty (AC) is an effective surgical procedure for patients with neurogenic bladder whenever conservative treatments have failed. The present study aimed to determine the risks of metabolic complications, malignancy, long-term outcomes and histopathologic changes of native bladder and the augmented intestine after AC in children with neurogenic bladder. Pediatric patients < 18 years who underwent AC between 2000 and 2020 were enrolled. Early postoperative complications, long-term outcomes and histopathologic changes in mucosal biopsies of native bladder and the augmented intestine after AC were reviewed. Twenty-two patients with a mean age of 7.6 ± 4.4 years were included. The ileum was used in 19 patients and the sigmoid colon in 3 patients. The length of hospital stay was 14.8 ± 6.8 days. Post-operatively, the urinary continence rate improved from 22.7 to 81.8% (p < 0.001). Hydronephrosis resolved in 17 of 19 patients. Vesicoureteral reflux resolved in 16 (64.0%) of the refluxing ureter units and was downgraded in 7 (28.0%). Grades of hydronephrosis and reflux significantly improved following AC (p < 0.001). The estimated glomerular filtration rate also significantly increased (p = 0.012). Formation of urinary tract stones was the most frequent late complication (in 8 patients, 36.4%). Life-threatening spontaneous bladder perforation occurred in 1 patient. After a mean follow-up of 13.4 ± 5.9 years, there were no cases of mortality, new-onset symptomatic metabolic acidosis, or changes in serum electrolytes. Of the 17 patients who were followed for > 10 years, no cases of malignancy or metaplastic changes were identified in the native bladder or augmented bowel epithelium. AC is a safe and effective procedure with low surgical and metabolic complication rates. In addition, AC provides a satisfactory continence rate and long-term protection of renal function, increases functional capacity, and regresses reflux and hydronephrosis. Individualized surveillance is recommended for the early identification of urolithiasis and metabolic disturbances.

Bladder management is the top health concern among adults with a spinal cord injury.

INTRODUCTION AND OBJECTIVE: Individuals with spinal cord injury (SCI) commonly experience secondary complications though it is not known how they prioritize these different health domains. Using the Neurogenic Bladder Research Group (NBRG) SCI registry, our objective was to identify the top health concerns of individuals with SCI and identify factors that may be associated with these choices with particular focus on urologic issues that participants face. METHODS: Participants in the NBRG registry were asked: "What are the top 3 problems that affect you on a daily basis?" Urinary symptoms and QoL were assessed with the Neurogenic Bladder Symptom Score (NBSS). Multivariate regression was used to identify factors related to selecting a top ranked health issue. RESULTS: Among our 1461 participants, 882 (60.4%) were men and the median age was 45.1 years (IQR 25.3-64.9). Bladder management was the most commonly top ranked primary issue (39%) followed by pain (16.4%) and bowel management (11.6%). Factors associated with ranking bladder management as the primary concern included years since injury (OR 1.01 [1.00-1.02], p = 0.042), higher (worse) total NBSS (OR 1.05 [1.03-1.06], p < 0.001), and higher (worse) NBSS QoL (OR 1.25 [1.12-1.41], p < 0.001). Reporting chronic pain on a daily basis was associated with ranking pain as the primary health concern (OR 41.7 [15.7-170], p < 0.001). CONCLUSIONS: In this cohort, bladder management was ranked as the top health issue and increasing time from injury was associated with increased concern over bladder management. More bladder symptoms were also associated with ranking bladder management as a primary concern while bladder management method and urinary tract infections rate were not.

Bladder Drainage Options For People With Spinal Cord Injury: A Mini-Review.

Neurogenic bladder is one of the negative consequences following a spinal cord injury (SCI). SCI patients who have neurogenic bladder depend on alternative methods to drain urine from their bladder. These include indwelling catheters, reflex voiding , suprapubic tapping and intermittent catheterisation. This review summarizes evidence from the literature of five selected complications (renal failure, urinary tract infections, calculi, urethral stricture, and bladder cancer) that could result from use of the different bladder drainage methods. There is inconsistent evidence to support the superiority of intermittent over indwelling catheterisation on risk of renal impairment, urethral stricture, and renal calculi. Indwelling catheterisations are associated with higher risk of bladder calculi and cancer. Caution needs to be taken when interpreting this review, as many of its findings are from retrospective studies, and more than a decade old. Clinicians need to communicate the evidence to their patients when making the decision on method of bladder drainage.

Importance of Regular Examination and Follow-up in Pediatric Patients with Neurogenic Bladder: 24-Month Follow-up Study Using a Japanese Health Insurance Database.

INTRODUCTION: Data on the long-term management of neurogenic bladder (NGB) are scarce. We evaluated the current status of NGB management in Japanese children over 24-month follow-up using the JMDC database. METHODS: In this descriptive, observational, retrospective cohort study, patients (≤ 17 years) with NGB were included. Patient characteristics and their management status were investigated. A multivariate analysis evaluating the potential risk factors for the development of urinary tract infection (UTI) was performed. The diagnosis of spina bifida, demographics, baseline comorbidities, and early use of clean intermittent catheterization (CIC) and/or overactive bladder (OAB) drugs were used as independent variables. RESULTS: Of 883 eligible children, 39.3% had spina bifida. Over 12/24-month post-index periods, renal urinary tract ultrasound and urinalysis were performed at least once in > 35%/> 45% patients, respectively, while specific tests (urodynamics, cystourethrography, scintigraphy) were performed in substantially fewer (< 11%/< 13%) patients. Over 24 months, 21.5% patients used OAB medications (mostly anticholinergics) and 10.8% underwent CIC, alone or with medications; 1.2% patients underwent surgery. Lower UTI (23.3%), urinary incontinence (9.7%), and hydronephrosis (7.0%) were the most common incident complications. Multivariate analysis evaluating risk factors for UTI showed significantly higher odds ratios with point estimates of ≥ 2 for CIC (5.70), presence of spina bifida (2.86), and constipation (2.07). Overall, urodynamic assessments were inadequately performed. CONCLUSION: Patients with use of CIC and/or having spina bifida and constipation had a higher risk of UTI, suggesting the need for careful follow-up. More guideline-compliant and diligent patient management is necessary in Japanese children with NGB.

Urologic manifestations of hypotonia, ataxia, and delayed development syndrome (HADDS), a rare neurodevelopmental disorder.

INTRODUCTION: Early B-cell factor 3 (EBF3) is a transcription factor involved in neuronal differentiation and maturation. Pathogenic variants are associated with hypotonia, ataxia, and delayed development syndrome (HADDS) (MIM#617330). Urologic manifestations are common and may have implications regarding long term renal function. OBJECTIVE: To review all known patients with pathogenic variants of the EBF3 gene resulting in HADDS with urologic manifestations. We hypothesize a high rate of bladder dysfunction secondary to the EBF3 variant's impact on relaxation of the urinary sphincter leading to detrusor sphincter dyssynergia (DSD). METHODS: The PubMed database was queried for publications of the EBF3 mutation between January 2017 and January 2023. Search terms were "EBF3 mutation OR HADDS AND urology OR phenotype". Retrospective analysis of HADDS patients cared for in our institution was performed. Demographic and clinical information was collected. RESULTS: We identified 52 patients (33F:19M) through literature (28F:18M) and retrospective review (5F:1M). There was a high prevalence of genitourinary physical exam abnormalities, history of urinary tract infection, vesicoureteral reflux (VUR), and diagnosis of neurogenic bladder. Within the literature review cohort, 67% had a urologic diagnosis. Females were disproportionately affected with urologic manifestations. In our cohort, four of six children were diagnosed with VUR and severe voiding dysfunction consistent with neurogenic bladder (67%). These children were managed with a vesicostomy. Five children had bowel dysfunction requiring therapy. Urodynamics suggested a high prevalence of external sphincter dyssynergia. Less severe forms of DSD were felt to be implicated in the abnormal voiding parameters in children who presented later in life based on non-invasive flow studies. DISCUSSION: There is significant variability in the phenotypic presentation of patients with HADDS. While EBF3 plays a clear role in neurodevelopment, it also impacts muscle development and may impact muscle relaxation. The location of the genetic variant may impact the degree of DSD, with more severe forms leading to earlier presentations. Initial work-up should include a renal ultrasound (RUS) and post void residual (PVR). Consideration can be given to obtaining a VCUG, DMSA scan or urodynamic studies. Yearly screening should be pursued with an RUS and PVR in those with an initial unremarkable work-up given the variable timing and severity of presentation. CONCLUSION: Urologic manifestations of HADDS include high rates of bladder dysfunction secondary to DSD, vesicoureteral reflux, urinary tract infection, and cryptorchidism. These patients are at risk of renal deterioration if urinary abnormalities are not properly diagnosed and managed.

Follow-up for the upper urinary tract in patients with high-risk neurogenic lower urinary tract dysfunction.

PURPOSE: Patients with neurogenic lower urinary tract dysfunction (nLUTD) can be at risk of preventable damage to the upper urinary tract (UUT), a risk that varies with the underlying diagnosis. Existing literature fails to document precisely which domains of UUT must be followed. This review focusses on surveillance of UUT with special emphasis on high-risk nLUTD. METHODS: Narrative review of available evidence and current global guidelines to identify patients of nLUTD at higher risk of UUT damage and to define each domain that needs to be followed. RESULTS: Patients with open spina bifida, spinal cord injury, and anorectal malformation, as well as those with unsafe features on clinical evaluation or urodynamics should be considered at high risk. Structured program should include evaluation of (1) glomerular filtration rate (GFR) which can be estimated ± measured, (2) renal growth (in pre-pubertal children) by ultrasonography, (3) renal scarring by baseline nuclear renogram, (4) hydronephrosis and stones by ultrasonography, (5) vesicoureteral reflux by baseline video-urodynamics, (6) non-GFR measures of renal function (somatic growth, hypertension, proteinuria, anemia and metabolic acidemia). Serum creatinine-based estimations of GFR are more likely to be confounded in select patients with high-risk nLUTD than cystatin-based estimations and measured-GFR. Urological guidelines do not always describe details of UUT surveillance and appear to lack cognizance of nephrology guidelines for evaluation of kidneys. CONCLUSION: A structured surveillance protocol that includes clear documentation of each domain of the UUT is important in ensuring optimum care for patients with high-risk nLUTD.

The temporal burden of preparing catheters for re-use in adults with spinal cord injury: a cross-sectional study.

STUDY DESIGN: Cross-sectional OBJECTIVES: Neurogenic lower urinary tract dysfunction is common among people with spinal cord injury (SCI). Although single-use clean intermittent catheterization is recommended to facilitate routine bladder emptying, catheter re-use is common. Barriers associated with the preparation (i.e., cleaning) of catheters for re-use are unknown. This study examined barriers to catheter re-use in adult individuals with SCI by assessing (1) the time needed to clean a catheter, and (2) the perceived difficulty of the catheter cleaning routine. SETTING: Laboratory METHODS: Twenty individuals with chronic SCI ( ≥ 1 year since injury; Group 1 = 10 with tetraplegia; Group 2 = 10 with paraplegia) completed the study. Using a standardized cleaning procedure (i.e., Milton method), catheter cleaning was timed for each participant. Perceived difficulty was assessed using a 5-point Likert scale. Functional impairment was assessed with the Upper Extremity Motor Score (UEMS). RESULTS: Significant between-group differences were observed for total cleaning time (Group 1 = 1584.1 ± 179.8 s; Group 2 = 1321.0 ± 93.8 s, p = 0.004) and perceived difficulty [Group 1 = 2.6 (2, 3); Group 2 = 2 (1.7, 2.3), p = 0.028]. Total cleaning time was significantly correlated with UEMS (ρ = -0.709, p ≤ 0.001) and perceived difficulty (ρ = 0.468, p = 0.037). UEMS emerged as an independent predictor of total cleaning time (R2 = 0.745, ß = -0.833, p ≤ 0.001). CONCLUSIONS: Preparing catheters for re-use is time-intensive and difficult for people with higher SCI level, severity and more pronounced upper limb motor impairment, which was independently associated with total cleaning time. Performing this routine on a consistent basis would require a substantial time commitment and would have a profoundly negative impact on overall quality of life.

Evaluating the utility of routine urine culture and antibiotic treatment in children with neurogenic bladder undergoing intradetrusor OnabotulinumtoxinA injection.

INTRODUCTION: OnabotulinumtoxinA is used as treatment for refractory idiopathic and neurogenic detrusor overactivity in children. Many patients perform intermittent self-catheterization and therefore have higher rates of asymptomatic bacteriuria, which may increase their risk of symptomatic urinary tract infection (UTI) following treatment. Multiple injections are often needed due to the short-term efficacy of onabotulinumtoxinA treatment, which may also increase the risk of UTI. OBJECTIVE: We aim to evaluate whether a sterile urinary tract is necessary to decrease the risk of postoperative UTI in pediatric patients treated with onabotulinumtoxinA. STUDY DESIGN: A retrospective review of patients undergoing intradetrusor onabotulinumtoxinA injection from 2014 to 2021 was performed. Demographic data, clinical characteristics, antibiotic treatment and culture results were collected. A positive urine culture was defined as ≥ 103 CFU/ml of uropathogenic bacteria. Our primary outcome was symptomatic UTI within 14 days of the procedure. RESULTS: 103 patients underwent 158 treatments with onabotulinumtoxinA. The incidence of postoperative UTI was 3.2%. The incidence of symptomatic postoperative UTI in patients with asymptomatic bacteriuria compared to those with sterile urine was not significantly different (3.8% vs 0%, p = 0.57). Obtaining a preoperative urinalysis or urine culture did not affect the incidence of postoperative UTI (p = 0.54). The number needed to treat with antibiotics to prevent one postoperative UTI was 27. The incidence of postoperative UTI was highest in patients with low-risk bladders (p = 0.043). Prior history of multi-drug resistant UTI was a risk factor for postoperative UTI (p = 0.048). DISCUSSION: For children undergoing onabotulinumtoxinA injection, there are no evidence-based recommendations regarding antibiotic prophylaxis and the need to screen for and treat asymptomatic bacteruria prior to treatment. Our study addresses this important clinical question, and shows no difference in the rate of postoperative UTI between patients with asymptomatic bacteriuria and those with sterile urine. Patients with a history of multi-drug resistant UTI are at increased risk of symptomatic postoperative UTI and may benefit from preoperative urine testing and treatment. Limitations of our retrospective study include its small sample size in the face of such a low incidence of our primary outcome. CONCLUSIONS: The risk of UTI following onabotulinumtoxinA injection in children is low. The presence of sterile urine at the time of surgery does not significantly decrease the risk of postoperative UTI. Routine treatment of asymptomatic bacteriuria prior to surgery results in a large number of patients receiving unnecessary antibiotics. As a result, we recommend against preoperative urine testing for most asymptomatic patients.

Treatment of neurogenic detrusor overactivity and overactive bladder with Botox (onabotulinumtoxinA): Development, insights, and impact.

Neurogenic detrusor overactivity (NDO) is a complication of multiple sclerosis, spinal cord injury (SCI), stroke, head injury, and other conditions characterized by damage to the upper motor neuronal system. NDO often leads to high bladder pressure that may cause upper urinary tract damage and urinary incontinence (UI). Prior to the use of onabotulinumtoxinA, oral anticholinergics and surgical augmentation cystoplasty were the treatment options. Overactive bladder (OAB) is non-neurogenic and affects a much larger population than NDO. Both NDO and OAB negatively impact patients' quality of life (QOL) and confer high health care utilization burdens. Early positive results from pioneering investigators who injected onabotulinumtoxinA into the detrusor of patients with SCI caught the interest of Allergan, which then initiated collaborative clinical trials that resulted in FDA approval of onabotulinumtoxinA 200U in 2011 for NDO and 100U in 2013 for patients with OAB who inadequately respond to or are intolerant of an anticholinergic. These randomized, double-blind, placebo-controlled trials for NDO showed significant improvements in UI episodes, urodynamic parameters, and QOL; the most frequent adverse events were urinary tract infection (UTI) and urinary retention. Similarly, randomized, double-blind, placebo-controlled trials of onabotulinumtoxinA 100U for OAB found significant improvements in UI episodes, treatment benefit, and QOL; UTI and dysuria were the most common adverse events. Long-term studies in NDO and OAB showed sustained effectiveness and safety with repeat injections of onabotulinumtoxinA, the use of which has profoundly improved the QOL of patients failing anticholinergic therapy and has expanded the utilization of onabotulinumtoxinA into smooth muscle.

A grading system for evaluation of bladder trabeculation.

PURPOSE: To establish a parameter-based grading system for evaluating bladder trabeculation (BT). MATERIALS AND METHODS: A retrospective analysis was conducted on children diagnosed with posterior urethral valve (PUV) or neurogenic bladder (NB) who underwent voiding cystourethrogram (VCUG), urodynamic testing, and urological ultrasonography between January 2016 and October 2022. Cases involving urologic surgery, secondary bladder pathology, and an interval of more than 12 months between examinations were excluded. A parameter named Bladder Dispersion (BD) was calculated through fluoroscopic images, and the grading system was developed as follows: BD < 40 (Grade 0), 40 ≤ BD < 60 (Grade 1), 60 ≤ BD < 90 (Grade 2), BD ≥ 90 (Grade 3). Grades 0-1 were classified as low-risk group, while grades 2-3 were classified as high-risk group. Analysis of variance, Kruskal-Wallis test, and Chi-square test were performed to compare urodynamic results and complications across different grades and groups. RESULTS: A total of 74 patients were eligible to participate, which included 46 boys (62.2%) and 28 girls (37.8%), the mean age was 75.18 ± 48.39 months. Among them, 11 (14.9%) were PUV, 50 (67.6%) were NB, and 13 (17.5%) were PUV and NB. Significant differences were observed in maximum detrusor pressure, post-void residual urine ratio, and compliance among grades 0-3. Severe hydronephrosis and histories of urinary tract infection were more prevalent in the high-risk group. CONCLUSION: A reliable grading system with objective standards was proposed which could aid in the assessment of BT severity.

Risk factors associated with recurrent febrile urinary tract infection in children with neurogenic bladder who perform clean intermittent catheterization.

OBJECTIVE: To identify the clinical and urodynamic risk factors associated with the development of recurrent febrile urinary tract infections (FUTIs) in children with neurogenic bladder (NB) who perform clean intermittent catheterization (CIC). METHODS: Children with NB receiving CIC were prospectively enrolled from January to December 2019 and followed up prospectively for 2 years. All data were compared between occasional (0-1 FUTI) and recurrent FUTIs (≥2 FUTI) groups. In addition, the risk factors for recurrent FUTIs in children were evaluated. RESULTS: Complete data from 321 children were analyzed. Occasional FUTIs occurred in 223 patients, and 98 patients experienced recurrent FUTIs. Univariate and multivariate analyses, showed late-initiation and low-frequency CIC, vesicoureteral reflux (VUR), small bladder capacity and low compliance, and detrusor overactivity were associated with an increased risk of recurrent FUTIs. Children with high-grade VUR (grades IV-V) had a higher risk of recurrent FUTIs than those with low-grade VUR (grades I-III) (odds ratio [OR]: 26.95 vs. OR: 4.78, p < 0.001). CONCLUSIONS: Our study suggests that late-initiation and low-frequency CIC, VUR, small bladder capacity and low compliance, and detrusor overactivity were associated with recurrent FUTIs in patients with NB. In addition, high-grade VUR is a crucial risk factor for recurrent FUTIs.

Recurrent Urinary Tract Infection in Adult Patients, Risk Factors, and Efficacy of Low Dose Prophylactic Antibiotics Therapy.

BACKGROUND: Recurrent urinary tract infection (UTI) occurs in sizable percentages of patients after a single episode and is a frequent cause of primary healthcare visits and hospital admissions, accounting for up to one quarter of emergency department visits. We aim to describe the pattern of continuous antibiotic prophylaxis prescription for recurrent urinary tract infections, in what group of adult patients they are prescribed and their efficacy. METHODS: A retrospective chart review of all adult patients diagnosed with single and recurrent symptomatic urinary tract infection in the period of January 2016 to December 2018. RESULTS: A total of 250 patients with a single UTI episode and 227 patients with recurrent UTI episodes were included. Risk factors for recurrent UTI included diabetes mellitus, chronic renal disease, and use of immunosuppressive drugs, renal transplant, any form of urinary tract catheterization, immobilization and neurogenic bladder. E. coli infections were the most prevalent organism in patients with UTI episodes. Prophylactic antibiotics were given to 55% of patients with UTIs, Nitrofurantoin, Bactrim or amoxicillin clavulanic acid. Post renal transplant is the most frequent reason to prophylaxis antibiotics (44%). Bactrim was more prescribed in younger patients (P < 0.001), in post-renal transplantation (P < 0.001) and after urological procedures (P < 0.001), while Nitrofurantoin was more prescribed in immobilized patients (P = 0.002) and in patients with neurogenic bladder (P < 0.001). Patients who received continuous prophylactic antibiotics experienced significantly less episodes of urinary tract infections (P < 0.001), emergency room visits and hospital admissions due to urinary tract infections (P < 0.001). CONCLUSION: Despite being effective in reducing recurrent urinary tract infection rate, emergency room visits and hospital admissions due to UTI, continuous antibiotic prophylaxis was only used in 55% of patients with recurrent infections. Trimethoprim/sulfamethoxazole was the most frequently used prophylactic antibiotic. Urology and gynecological referral were infrequently requested as part of the evaluation process for patients with recurrent UTI. There was a lack of use of other interventions such as topical estrogen in postmenopausal women and documentation of education on non-pharmacological methods to decrease urinary tract infections.

Management of sphincter insufficiency in patients with neurogenic bladder and bladder augmentation.

PURPOSE: To investigate the need and efficacy of treatment of bladder neck procedures in patients with neurogenic bladder and augmentation. METHODS: The hospital database was reviewed for patients undergoing enterocystoplasty because of neurogenic bladder during 1990-2019. Diagnoses of patients as well as frequency, type, and efficacy of treatment of sphincter insufficiency were evaluated. RESULTS: Thirty-seven of 87 patients (43%) underwent surgery because of sphincter insufficiency. The median age at bladder augmentation was 11.9 years (IQR 8.5-14.8), and at the last control, 21.8 years (IQR 18.9-31.1). Bladder neck injections (BNI) were performed for 28 patients, fascial sling operation for 14 patients, and bladder neck closure (BNC) was done for five females. Full continence was achieved in 10/28 (36%) patients with one or repeat BNIs and 9/14 (64%) with sling operation. The outcome of BNIs and sling operations was similar in both sexes. All five female patients with BNC became continent. At the end of follow-up, 64 (74%) patients were dry, 19 (22%) had occasional incontinence episodes, and 4 (5%) had daily incontinence episodes necessitating pads. CONCLUSIONS: Treatment of sphincter insufficiency is challenging in patients with bladder augmentation and neurogenic disease. Only 74% of our patients became fully continent despite treatments for sphincter insufficiency.

Neurogenic Lower Urinary Tract Dysfunction.

Neurogenic lower urinary tract dysfunction (NLUTD) remains a formidable challenge to pediatric urologists to achieve the goals of renal preservation and the reduction of urinary tract infections as well as the attainment of continence and independence as children grow toward adulthood. Tremendous progress has occurred over the past 50 years which have witnessed an evolution in focus from mere survival to optimal quality of life. This review presents four separate guidelines for the medical and surgical care of pediatric NLUTD, most commonly related to spina bifida, to highlight the change in approach from expectant to more proactive management.

Psychological and mental impact of long-standing incontinence on children and adolescents with complex urologic disorders.

PURPOSE: Incontinence has a negative impact on the quality of life and is associated with psychiatric disturbances in humans. This study evaluates the impact of long-standing incontinence on psychological and mental development. METHODS: This is a cohort study carried out in a tertiary care urologic facility. After obtaining an IRB approval, we enrolled 49 children with severe incontinence (incontinence for at least 1 year duration and one surgical intervention) and interviewed between October 2019 and March 2020. Child Behavior Checklist (CBCL) and intelligence quotient (IQ) testing was performed for each using Stanford-Binet-4th edition. An age-matched control group was recruited for comparison. A total of 51 children in the control group were later recruited from March 2020 to October 2020 from the Psychiatry Department. RESULTS: A total of 49 children fulfilled the inclusion criteria. Mean age was 9.93 years, 31 males and 18 females. The etiology of incontinence was neuropathic bladder in 30, exstrophy in 8, incontinent epispadius in 4, valve bladder in 4; common urogenital sinus in 2, and refractory OAB in 1. Median number of procedures was 2 (Range 0-9). Median pads were 5 per day and median hospital stay was 32 days. Median total score of CBCL was 26.5 compared to 7 for the control group (p = 0.00023). Mean IQ of the study group was 88.3 as compared to that of the control (94.65), the difference is significant (p = 0.00023). CONCLUSION: Children with severe degree of incontinence suffered from significant psychiatric disorders and negative affection of their intelligence. A multidisciplinary approach is advised for management of those children.

Urinary tract infection in patients with lower urinary tract dysfunction.

Lower urinary tract dysfunction (LUTD) is becoming more common in aging societies worldwide. In patients with LUTD, the risk of urinary tract infection (UTI) rises due to several distinct mechanisms, including easy bacterial access to the urinary tract, impaired bacterial washout, and an impaired innate defense system. The pathophysiology of LUTD varies depending on whether it is neurogenic or non-neurogenic, as well as by gender; therefore, the etiology and characteristics of UTI differ according to the type of LUTD. Patients with neurogenic LUTD, especially those with spinal cord injury, have a high risk of febrile UTI, and strict bladder management is required to prevent UTI. Clean intermittent catheterization with or without appropriate pharmacological therapy is also strongly recommended for patients with neurogenic LUTD at risk of febrile UTI, unable to void, or with high post-void residual volume. In contrast, both male and female patients with non-neurogenic LUTD have a lower risk of symptomatic UTI. There is insufficient evidence for non-neurogenic LUTD regarding the association between symptomatic UTI but not asymptomatic bacteriuria and LUTD severity, including the presence of post-void residual volume, or whether therapeutic intervention for LUTS reduces the incidence of UTI, particularly in male patients. In this narrative review, we aimed to highlight the pathogenesis, epidemiology, and management of UTI in patients with LUTD.

Profiling the Urobiota in a Pediatric Population with Neurogenic Bladder Secondary to Spinal Dysraphism.

The human bladder has been long thought to be sterile until that, only in the last decade, advances in molecular biology have shown that the human urinary tract is populated with microorganisms. The relationship between the urobiota and the development of urinary tract disorders is now of great interest. Patients with spina bifida (SB) can be born with (or develop over time) neurological deficits due to damaged nerves that originate in the lower part of the spinal cord, including the neurogenic bladder. This condition represents a predisposing factor for urinary tract infections so that the most frequently used approach to treat patients with neurogenic bladder is based on clean intermittent catheterization (CIC). In this study, we analyzed the urobiota composition in a pediatric cohort of patients with SB compared to healthy controls, as well as the urobiota characteristics based on whether patients received CIC or not.

[Drugs for Neurogenic Bladder Dysfunction].

Urinary dysfunction includes an overactive bladder (OAB), post-void residual (PVR)/retention, or both entities. Brain diseases cause OAB, peripheral neuropathies are associated with significant PVR/retention, and multisystem atrophy/spinal cord diseases result in a combination of OAB and PVR/retention. Selective beta 3 adrenergic receptor agonists or anticholinergic agents are the first-choice treatment for OAB and clean intermittent self-catheterization, alpha-blocker and cholinergic stimulant therapy for significant PVR/retention. These therapies may be useful to maximize patients' quality of life and prevent serious complications, such as urosepsis or kidney dysfunction.

De novo truncating variants of TRIM8 and atypical neuro-renal syndrome: a case report and literature review.

BACKGROUND: The TRIM8 gene encodes a protein that participates in various biological processes. TRIM8 variants can lead to early termination of protein translation, which can cause a rare disease called neuro-renal syndrome. This syndrome is characterized by epilepsy, psychomotor retardation, and focal segmental glomerulosclerosis. However, we found that some patients may not present the above typical triad, and the reason may be related to their variant sites. CASE PRESENTATION: We report a case of a 6-year-old boy with nephrotic-range proteinuria as the first prominent manifestation of TRIM8 variant. He had stage 3 chronic kidney disease at the time of presentation, specific facial features, and a neurogenic bladder. He had not experienced seizures previously. There were no apparent abnormalities in his growth, intelligence, or motor development. The results of whole exome sequencing showed a TRIM8 variant. Renal biopsy revealed focal segmental glomerulosclerosis and renal tubular cystic dilatation. He did not respond to hormone and angiotensin-converting enzyme inhibitor treatment; however, the symptoms of neurogenic bladder were relieved after treatment with Solifenacin. CONCLUSION: In this case, renal disease was the prominent manifestation; the patient had no other obvious neurological symptoms except a neurogenic bladder. Notably, the variant site is the closest to the C-terminal to date. Based on the analysis of previously reported cases, we found that as the TRIM8 variant became closer to the C-terminal, the renal lesions became more prominent, and there were fewer neurologic lesions. Our findings provide a new understanding of neuro-renal syndrome caused by TRIM8 variant. Patients may only have kidney disease as a prominent manifestation. At the same time, we found that we should also pay attention to the eye lesions of these patients. Therefore, gene analysis is helpful in identifying the etiology and guiding the prognosis of patients with hormone-resistant proteinuria. We suggest that TRIM8 should be included in gene panels designed for the genetic evaluation of hormone-resistant proteinuria.