An Economic Evaluation of a Web-Based Management Support System for Children With Urinary Incontinence: The eADVICE Trial.

PURPOSE: Children who require specialist outpatient care typically wait substantial periods during which their condition may progress, making treatment more difficult and costly. Timely and effective therapy during this period may reduce the need for lengthy specialist care. This study evaluated the cost-effectiveness of an individualized, evidence-informed, web-based program for children with urinary incontinence awaiting a specialist appointment (Electronic Advice and Diagnosis Via the Internet following Computerized Evaluation [eADVICE]) compared to usual care. eADVICE was supervised by a primary physician and delivered by an embodied conversational agent. MATERIALS AND METHODS: A trial-based cost-effectiveness analysis was performed from the perspective of the health care funder as a substudy of eADVICE, a multicenter, waitlist-controlled, randomized trial. Outcomes measures were incremental cost per incremental change in continence status and quality of life on an intention-to-treat basis. Uncertainty was examined using cost-effectiveness planes, scenarios, and 1-way sensitivity analyses. Costs were valued in 2021 Australian dollars. RESULTS: The use of eADVICE was found to be cost saving and beneficial (dominant) over usual care, with a higher proportion of children dry over 14 days at 6 months (risk difference 0.13; 95%CI 0.02-0.23, P = .03) and mean health care costs reduced by $188 (95%CI $61-$315) per participant. CONCLUSIONS: An individualized, evidence-informed, web-based program delivered by an embodied conversational agent is likely cost saving for children with urinary incontinence awaiting a specialist appointment. The potential economic impact of such a program is favorable and substantial, and may be transferable to outpatient clinic settings for other chronic health conditions.

A Randomized Controlled Trial of a Web-Based Management Support System for Children With Urinary Incontinence: The eADVICE Trial.

PURPOSE: Children referred to specialist outpatient clinics by primary care providers often have long waiting times before being seen. We assessed whether an individualized, web-based, evidence-informed management support for children with urinary incontinence while waiting reduced requests for specialist appointments. MATERIALS AND METHODS: A multicenter, waitlisted randomized controlled trial was conducted for children (5-18 years) with urinary incontinence referred to tertiary pediatric continence clinics. Participants were randomized to the web-based eHealth program electronic Advice and Diagnosis Via the Internet following Computerized Evaluation (eADVICE), which used an embodied conversational agent to engage with the child at the time of referral (intervention) or 6 months later (control). The primary outcome was the proportion of participants requesting a clinic appointment at 6 months. Secondary outcomes included persistent incontinence, and the Paediatric incontinence Questionnaire (PinQ) score. RESULTS: From 2018 to 2020, 239 children enrolled, with 120 randomized to eADVICE and 119 to the control arm. At baseline, participants' mean age was 8.8 years (SD 2.2), 62% were males, mean PinQ score was 5.3 (SD 2.2), 36% had daytime incontinence, and 97% had nocturnal enuresis. At 6 months, 78% of eADVICE participants vs 84% of controls requested a clinic visit (relative risk 0.92, 95% CI 0.79, 1.06, P = .3), and 23% eADVICE participants vs 10% controls were completely dry (relative risk 2.23, 95% CI 1.10, 4.50, P = .03). The adjusted mean PinQ score was 3.5 for eADVICE and 3.9 for controls (MD -0.37, 95% CI -0.71, -0.03, P = .03). CONCLUSIONS: The eADVICE eHealth program for children awaiting specialist appointments doubled the proportion who were dry at 6 months and improved quality of life but did not reduce clinic appointment requests.

The rationale for bladder washouts in children with neurogenic bladder.

Children with a neurogenic bladder are at risk of developing recurrent urinary tract infections and long-term kidney failure. Due to an altered lower urinary tract, children may be overtreated for simple bacteriuria or undertreated for a potentially severe urinary tract infection. This group of patients represent high users of healthcare, and are at risk of colonization and development of antibiotic resistance. Bladder washouts with non-antibiotic electrochemically activated solutions are a potential new prophylactic option for patients with bladder dysfunction when clean intermittent catheterization has resulted in chronic bacteriuria.

Laparoscopic versus open pyeloplasty in paediatric pelvi-ureteric junction obstruction.

AIM: Compared to open pyeloplasty (OP), we hypothesised that laparoscopic pyeloplasty (LP) is associated with early recovery, a shorter length of stay (LOS) and less analgesia requirement. METHODS: Between 2011 and 2016, 146 dismembered pyeloplasty cases were reviewed, of which 113 were in the OP group and 33 were in the LP group. We evaluated both groups regarding operative time, LOS, success rate, complications rate and analgesia requirement. Subgroup analysis was done for patients above the age of 5 years, and within the OP group (dorsal lumbotomy (DL) vs. loin incision (LI)). RESULTS: The success rate was 96% in the open group and 97% in the laparoscopic group. The median operative time was significantly shorter in the open group for the entire cohort (127 vs. 200 min; P < 0.05), and in children older than 5 years (n = 41, 134 vs. 225 min; P < 0.05). Other parameters were similar in both groups. The median LOS was significantly shorter (2 vs. 4 days; P < 0.05), and the median analgesia requirement was less (0.44 vs. 0.64 mg/kg morphine; P < 0.05) in the DL (n = 60) compared to LI (n = 53). CONCLUSION: Both OP and LP dismembered approaches are equally effective in treating pelvi-ureteric junction obstruction. Overall, the LOS, complications rate and analgesia requirement were not significantly different; however, the operative time was significantly longer in LP.

Optimising the management of children with concomitant bladder dysfunction and behavioural disorders.

Bladder dysfunction and behavioural disorders in children are commonly concomitant; hence, it is difficult to treat each in isolation. Pharmacotherapy is common treatment for behavioural disorders, and these medications may have intended or unintended positive or negative bladder sequelae. This review identifies the literature regarding the effects of behavioural pharmacotherapy on bladder functioning and possible bladder management strategies in children with concomitant behaviour and bladder disorders to enable clinicians to better manage both conditions. A PROSPERO registered PRISMA-guided review of three major databases was performed. After an initial scoping study revealed significant heterogeneity, a narrative approach was undertaken to discuss the results of all relevant cases relating to children being treated with pharmacotherapy for behaviour disorders and outcomes related to bladder function. Studies were screened to identify those that described effects of commonly prescribed medications in children with behavioural disorders such as stimulants, alpha 2 agonists, tricyclic antidepressants (TCA), serotonin and noradrenergic reuptake inhibitors (SNRI), selective serotonin reuptake inhibitors (SSRI) and antipsychotics, and the findings and implications were summarised. The review identified 46 studies relevant to behavioural pharmacotherapy and bladder function (stimulants (n = 9), alpha 2 agonists (n = 2), TCAs (n = 7), SNRIs (n = 8), SSRIs (n = 8) and antipsychotics (n = 6). Six studies focused specifically on bladder management in children with behavioural disorders with concurrent behavioural pharmacotherapy. This review identifies useful factors that may assist clinicians with predicting unintended bladder effects following initiation of behavioural pharmacotherapy to facilitate the best approach to the treatment of bladder dysfunction in children with behavioural disorders. With this evidence, we have provided a useful decision-making algorithm to aide clinicians in the management of these dual pathologies.

Emerging therapeutic potential of group 2 innate lymphoid cells in acute kidney injury.

Acute kidney injury (AKI) remains a global challenge and, despite the availability of dialysis and transplantation, can be fatal. Those that survive an AKI are at increased risk of developing chronic kidney disease and end stage renal failure. Understanding the fundamental mechanisms underpinning the pathophysiology of AKI is critical for developing novel strategies for diagnosis and treatment. A growing body of evidence indicates that amplifying type 2 immunity may have therapeutic potential in kidney injury and disease. Of particular interest are the recently described subset of innate immune cells, termed group 2 innate lymphoid cells (ILCs). Group 2 ILCs are crucial tissue-resident immune cells that maintain homeostasis and regulate tissue repair at multiple organ sites, including the kidney. They are critical mediators of type 2 immune responses following infection and injury. The existing literature suggests that activation of group 2 ILCs and production of a local type 2 immune milieu is protective against renal injury and associated pathology. In this review, we describe the emerging role for group 2 ILCs in renal homeostasis and repair. We provide an in-depth discussion of the most recent literature that use preclinical models of AKI and assess the therapeutic effect of modulating group 2 ILC function. We debate the potential for targeting these cells as novel cellular therapies in AKI and discuss the implications for future studies and translation. Copyright © 2019 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Parental decision regret in childhood hypospadias surgery: A systematic review.

We conducted a systematic review of the literature to establish the prevalence of and predictive factors for parental decision regret in hypospadias surgery. A search strategy without language restrictions was developed with expert help, and two reviewers undertook independent study selection. Five studies were included in this review (four for quantitative analysis) with a total of 783 participants. The mean overall prevalence of parental decision regret was 65.2% (moderate to severe - 20.3%). Although significant predictors of regret were identified (post-operative complications, small size glans, meatal location, decision conflict between parents, parental educational level and others), they had unexplained discordance between studies. Parental decision regret after proximal hypospadias surgery and refusing surgery was inadequately reported. In conclusion, even though the prevalence of parental decision regret after consenting for the hypospadias repair appears to be high, risk factors associated with it were discordant suggesting imprecision in estimates due to unknown confounders.

Fatal Exsanguination Following Rupture of an Iliac Artery Aneurysm in an Infant With Menkes Disease.

Menkes disease (MD) usually presents in infancy with respiratory and neurological complications. Severe isolated vasculo-connective tissue involvement in infancy is rare, and hence the precise and timely diagnosis is difficult. We report a case of an 8-week-old male infant who succumbed to acute, severe exsanguination, and hemorrhagic shock secondary to a large retroperitoneal hematoma due to rupture of a right iliac artery aneurysm. Perimortem musculoskeletal findings raised suspicion of nonaccidental injury. However, postmortem review of facial traits raised the suspicion of MD. MD was subsequently confirmed on genetic testing. Child health clinicians must remain aware of MD as a rare cause of infant vasculopathy or atypical skeletal abnormalities.

Current strategies to predict and manage sequelae of posterior urethral valves in children.

Posterior urethral valves (PUV) constitute a significant urological cause of chronic kidney disease (CKD) in children. The condition is characterised by the unique pathophysiology of the evolution of bladder dysfunction after relief of obstruction, which contributes to CKD. Improvements in prenatal diagnosis followed by selective foetal intervention have not yet produced improvement in long-term renal outcomes, although better patient selection may alter this in the future. Proactive management with surveillance, pharmacotherapy, timed voiding, double voiding, and/or assisted bladder-emptying, is being increasingly offered to those with severe bladder dysfunction and has the potential of reducing the burden of renal disease. Clinicians are currently able to counsel regarding the prognosis using serum creatinine and other emerging markers. However, much of this work remains to be validated. Satisfactory graft survival rates are now reported with aggressive management of bladder dysfunction in children who are candidates for renal transplantation. Knowledge gaps exist in identifying early markers of renal injury, risk stratification, and in understanding patient and carer perspectives in PUV.

Laparoscopic repair of intra-abdominal bladder perforation in preschool children.

Intraperitoneal bladder rupture is uncommon in very young children, but its incidence may increase with increasing use of seat and lap belts. To the best of our knowledge, there are no prior reports of laparoscopic repair of this injury in children. We describe two recent cases and discuss useful technical points that facilitate a successful laparoscopic repair. Both our patients were preschool age girls who sustained seat and lap belt injuries. Contrast computed tomography scan suggested a large amount of free peritoneal fluid and cystogram confirmed intraperitoneal bladder perforation (isolated injury). The injury was repaired using delayed absorbable sutures and intracorporeal suturing (continuous in 1, interrupted in 1) using a 3 port laparoscopic technique. Meticulous peritoneal lavage was carried out to minimise urinary peritonitis and the bladder as well as the peritoneal cavity were drained. Check cystograms (day 7) revealed no leaks. Young girls appear to be at risk of intraperitoneal bladder injuries following lap belt injuries. After exclusion of life-threatening injuries and concurrent abdominal injuries which need rapid control or preclude pneumoperitoneum, a laparoscopic repair can be safely performed.

Ventilation inhomogeneities in children with congenital thoracic malformations.

BACKGROUND: Congenital thoracic malformations (CTM) are rare lung lesions that are managed with surgical resection or active surveillance. The objective of this study was to comprehensively assess large and small airway function in children with CTM who underwent lobectomy in early life. We hypothesise that sensitive measures of lung function will demonstrate residual impairments in CTM compared to healthy children. METHODS: Nitrogen lung clearance index (LCI), reactance and resistance (X5Hz and R5Hz), forced expiratory volume in 1 s and forced vital capacity (FEV1 and FVC) were prospectively measured in 10 children with CTM (mean age/SD: 7.6/1.3) who had undergone surgical resection in early life and in 17 healthy children (mean age/SD: 4.8/0.4). Total lung capacity (TLC) was also conducted in children older than 7 years of age with CTM (n = 8). RESULTS: Mean LCI was 8.0 (95% CI 7.5 to 8.5) in the CTM group and 7.3 (95% CI 7.0 to 7.6) in healthy children (p = 0.016). Mean X5Hz was -0.44kPa/l/s (95% CI -0.58 to -0.31) in the CTM group and -0.31kPa/l/s (95% CI -0.35 to -0.27) in healthy children (p = 0.02). Mean Z score for X5Hz was -2.11 (95% CI -3.59 to -0.63) in the CTM group and -0.11 (95% CI -0.55 to 0.33) in healthy children (p = 0.0008). Mean FEV1 was 1.21 L (95% CI 0.97 to 1.45) in the CTM group and 1.02 L (95% CI 0.90 to 1.15) in healthy children (p = 0.22). Mean % predicted FEV1 was 83% (95% CI 74 to 92) in the CTM group and 97% (95% CI 87 to 107) in healthy children (p < 0.05). Mean % predicted TLC in CTM children was 121.3% (95% CI 88.45 to 154.1). Mean LCI was inversely correlated with height z-scores in the CTM group (rs = -0.88, p = 0.002) but not in healthy children (rs = 0.22, p = 0.4). CONCLUSIONS: Children with CTM have impaired lung function as demonstrated by the significant differences in LCI, reactance and FEV1 but not FVC, resistance and TLC. These findings may be of clinical relevance as ventilation inhomogeneities are closely correlated with somatic growth in this study.

Treatment modalities for paediatric functional daytime lower urinary tract disorders: an updated review.

Paediatric functional bladder disorders especially those causing daytime symptoms are a common cause of significant psychosocial and/or physical morbidity and impaired quality of life. Despite the availability of many therapeutic modalities, a significant number of children appear to be refractory to treatment and continue to have symptoms. In this review, we aim to evaluate the current evidence in the use of existing and novel therapeutic options for the management of daytime lower urinary tract disorders in children. We also aim to highlight the controversies around the terminology and diagnosis of paediatric lower urinary tract dysfunction (LUTD) and specific conditions. The article will then provide a reasonable critique of the existing and emerging treatment modalities in functional daytime LUTD in children including their mode of action, efficacy, indications, and recent advances. These include standard urotherapy, specific urotherapy comprised of biofeedback, alarm therapy and electrical neural stimulation and pharmacotherapy involving selective and non-selective anticholinergics, ß3 adrenergic agonists, alpha blockers and botulinum toxin. A better understanding of this common clinical problem may help clinicians achieve better profiling of these children's diagnoses to further enable specific, targeted treatment.

A review article about new treatment options for otherwise healthy children with long-term urinary symptoms occurring during the daytime Management of paediatric functional daytime LUT disorders is complex and may benefit from a combination of treatment modalities. Urotherapy and anticholinergics appear to be effective in the majority however, non-responders warrant careful re-evaluation to characterize the specific type of LUTD to target appropriate treatment. Various novel therapies and adjuncts have been shown effective and range from smartphone apps, bladder alarms, neuromodulation systems and more effective drug delivery systems. Despite being effective, non-selective antimuscarinics are less favoured for long-term use in children due to the side-effect profile. Therefore, more selective anticholinergics, ß3 agonists and combination treatment options are being evaluated to improve compliance while maintaining/enhancing treatment efficacy. Use of alpha blockers and intravesical injection of botulinum toxin have shown promising results especially in refractory cases.

Lack of consensus in atypical congenital obstructive urethral lesions in children: Snapshot of the web-based ObsCUre (obstruction to the child urethra) study.

Background: Atypical Congenital Obstructive Urethral Lesions (ACOUL) are uncommon causes of urethral obstruction in children. They include Cobb's collar or Moorman's ring, Type III posterior urethral valve (PUV), congenital urethral narrowing and anterior urethral valves. This study is aimed to evaluate the knowledge and current practice amongst clinicians attending to ACOUL. An international online case based questionnaire was performed. Materials and methods: A survey was administered to members of international urological societies. It included 22 clinical questions on cases with ACOUL (14 questions suitable for statistical analysis) using cases of Type I PUV as controls. Two sets of paired questions evaluated change in opinion(s) after additional information was provided. Results: One hundred twenty-one participants responded with 71% reporting exposure of less than 5 cases per annum. In questions regarding diagnosis between 11.6% (14/121) and 21.5% (26/121) of participants identified the ACOUL as PUV. Among them, 66% of respondents agreed on ACOUL's causative role in urethral obstruction. Gini coefficient was consistently lower for ACOUL compared to PUV: diagnosis (mean 0.33 vs. 0.44) and prognosis (0.23 vs. 0.43). High intra-rater concordance (kappa 0.420.57) was observed for paired questions-a mean of 5.79% (7.44% and 4.13% for questions 10 and 12, 16 and 17, respectively) of participants changed their answers from an alternate diagnosis to the correct diagnosis of ACOUL after viewing endoscopic images. High variation in management of ACOUL was noted (Gini 0.51). Conclusions: This global snapshot survey identified substantial inconsistency among clinicians dealing with ACOUL. Although rarely encountered in clinical practice, better overall education of ACOUL is warranted.

Reduction of Urinary Tract Infection in Pediatric Surgical Patients Using NSQIP-P and Quality Improvement Methodology.

BACKGROUND: Hospital-acquired urinary tract infections (UTIs) have a detrimental effect on patients, families, and hospital resources. The Sydney Children's Hospital Network (SCHN) participates in the NSQIP-Pediatric (NSQIP-P) to monitor postoperative complications. NSQIP-P data revealed that the median UTI rate at SCHN was 1.75% in 2019, 3.5 times higher than the NSQIP-P target rate of 0.5%. Over three quarters of the NSQIP-P identified patients with UTI also had a urinary catheterization performed intraoperatively. A quality improvement project was conducted between mid-2018 and 2021 to minimize catheter-associated UTIs (CAUTIs) at SCHN. STUDY DESIGN: NSQIP-P samples include pediatric (younger than 18 years) surgical patients from an 8-day cycle operative log. NSQIP-P data are statistically analyzed by the American College of Surgeons and provide biannual internationally benchmarked reports. The project used clinical redesign methodology with a 6-phase process for quality improvement projects. RESULTS: The objectives of the project were to reduce urinary catheter duration of use, educate parents or carers, and improve catheter care and insertion technique by health staff. The duration of a urinary catheter in situ reduced from a median of 4.5 to 3 days from 2017 to 2021. The median NSQIP-P UTI rate at SCHN was reduced by 47.4% from 1.75% in 2019 to 0.9% in 2022. CONCLUSIONS: A multifactorial approach in quality improvement has been shown to be an effective strategy to reduce UTI rates at SCHN, and patient outcomes were improved within a 3-year timeframe. Although this project has reduced UTI rates at SCHN, there remain opportunities for further improvement.

Adherence to ICCS nomenclature guidelines in subsequent literature: a bibliometric study.

AIMS: Since the publication of the 2006 International Children's Continence Society (ICCS) guidelines on terminologies for lower urinary tract dysfunction in children, little is known of their impact. In this study, we aim to quantify the adherence to the guidelines in the published literature, and to examine whether Medical Subject Headings (MeSH) in MEDLINE reflect the recommended "new" ICCS terminology. METHODS: Seven pairs of pre-specified paired terms (obsolete and recommended by the ICCS) were searched, limited to paediatric literature published between 2002 and 2010. Their use in the literature was compared between the pre-guideline (2002-2005) and post-guideline (2007-2010) period and across geographical regions. MeSH in MEDLINE were examined for the use of ICCS preferred terminology. RESULTS: Publications in paediatric urinary incontinence have shown a 49% increase from 2002-2005 to 2007-2010 (55-82 per year). There was about a fourfold increase in the likelihood of usage of ICCS recommended terminologies post ICCS guideline publication (OR: 4.19, 95% CI: 3.04-5.78, P < 0.001). Approximately 25% of the studies published between 2007 and 2010 used obsolete terminologies. Analysis indicated satisfactory uptake for most terms, with the exception of "urotherapy." There was no significant geographical variation in uptake. More than half of the ICCS-recommended terms (4/7) did not appear in the current MeSH indexing tree and scope notes. CONCLUSIONS: Overall uptake of recommended terms following release of ICCS terminology guidelines was encouraging although it remains suboptimal for certain terms. Efforts need to be made to improve the current MEDLINE indexing so that MeSH terms reflect terminology recommended by the ICCS.

Pharmacological interventions for the management of cystinuria: a systematic review.

BACKGROUND: Cystinuria is a rare genetic kidney stone disease, with no cure. Current treatments involve lowering urinary cystine levels and increasing cystine solubility. This systematic review evaluates the available literature regarding non-surgical interventions for cystinuria. METHODS: Key electronic databases were searched for studies that described the clinical management of cystinuria with high diuresis, alkalinizing agents and thiol-based drugs that were published between 2000 and 2022. Observational studies were included if they contained clinical investigation with at least one previous or current episode of cystine stones, urine cystine levels > 250 mg/L and patients being managed with urinary dilution, alkalinizing agents or other pharmacological agents. All included studies were assessed for study design, patient characteristics and outcomes. A qualitative and critical analysis was performed whereby study quality was assessed using Methodological Index for Non-Randomized Studies (MINORS). Two authors performed the quality assessment and excluded the studies with a low MINORS score. RESULTS: Fourteen studies met the review inclusion and quality criteria. Of the fourteen studies, two reported treatment using alkalinizing agents, six reported treatment using thiol-based drugs, and six reported combination treatment using alkalinizing agents and thiol-based drugs. These studies indicated that first-line therapies, including high fluid intake and urinary alkalinization, increased urine volume to > 3 L/day and urinary pH > 7.0, and were associated with reduced urinary cystine levels and cystine stone formation. Second-line therapy with cystine-binding thiol drugs, such as tiopronin and D-penicillamine, reduced urinary cystine levels, cystine crystal volume and increased cystine solubility, resulting in decreased cystine stone formation and stone recurrence rate. Further, combined intervention with alkalinizing agents and thiol-based drugs synergistically reduced stone recurrence. CONCLUSION: Cystinuria treatment may require a combined approach of high diuresis, alkalinization and pharmacological interventions with regular monitoring of urinary pH, cystine levels, cystine crystal volume and solubility. However, poor adherence to treatment is relatively frequent, hence the pressing urgency for improved therapies and treatments.

Ambulatory urodynamic studies (UDS) in children using a Bluetooth-enabled device.

OBJECTIVES: • To report the early observations of using ambulatory urodynamic studies (UDS) using a Bluetooth-enabled device in children • To evaluate the incremental value of ambulatory over conventional UDS. PATIENTS AND METHODS: • Ambulatory UDS were performed in selected children with voiding dysfunction between August 2009 and October 2010. • Conventional UDS were concurrently performed wherever possible. • The test results and treatment consequences of the two tests were compared. RESULTS: • In all, 12 ambulatory and seven conventional UDS were performed on 10 children (five boys, median [range] age 7 [4-16] years). • Six of the seven children had a normal conventional UDS. Ambulatory UDS detected phasic detrusor overactivity (DO) in five children and generalised DO in one. • Direct correlation of symptoms to DO was possible in two children during ambulatory UDS. Pressure rise during filling, seen in two children on conventional UDS, was not seen during ambulatory UDS. • Five children showed clinical improvement when therapy was guided by ambulatory UDS results. • Ambulatory UDS was generally well tolerated in eight children, with two complaining of discomfort. Inadequate information was obtained in two children who underwent ambulatory UDS due to technical problems in one and distress induced by the UDS in the other. CONCLUSIONS: • Ambulatory UDS provides useful additional information over conventional UDS and can be used to guide further therapy in selected children with voiding dysfunction. • It is safe and well tolerated in children. • There is a need for explicit guidance for the technical delivery and interpretation of ambulatory UDS in children.