Implementation of a standardized clinical pathway in a dedicated posterior urethral valves clinic: short-term outcomes.

BACKGROUND: To determine if the implementation of a posterior urethral valves (PUV) clinic and standardized management pathway improves the short-term kidney outcomes of infants with PUV. METHODS: From 2016-2022, 50 consecutive patients were divided into groups after the implementation of the clinic (APUV, n = 29) and before (BPUV, n = 21) during a comparable timeframe. Assessed data included age at initial visit, timing and type of surgery, frequency of follow-up visits, medications, nadir creatinine, and development of CKD/kidney failure. Data are shown as median with interquartile range (IQR) and odds ratios (OR) with 95% confidence interval (CI). RESULTS: APUV had higher rates of prenatal diagnoses (12/29 vs. 1/21; p = 0.0037), earlier initial surgical intervention (8 days; IQR 0, 105 vs. 33 days; IQR 4, 603; p < 0.0001), and higher rates of primary diversions (10/29 vs. 0/21; p = 0.0028). Standardized management led to earlier initiation of alpha blockers (326 days; IQR 6, 860 vs. 991; IQR 149, 1634; p = 0.0019) and anticholinergics (57 days; IQR 3, 860 vs. 1283 days; IQR 477, 1718; p < 0.0001). Nadir creatinine was reached at earlier ages in APUV (105 days; IQR 2, 303 vs. 164 days; IQR 21, 447; p = 0.0192 BPUV). One patient progressed to CKD5 in APUV compared to CKD3, CKD5 and one transplant in BPUV. CONCLUSION: Implementing the PUV clinic with standardized treatment expedited postnatal management and resulted in a higher number of cases detected prenatally, a shift in primary treatment, younger ages at initial treatment, reduced time to nadir creatinine, and timely initiation of supportive medications. A higher resolution version of the Graphical abstract is available as Supplementary information.

The impact of posterior urethral valves on pediatric quality of life and family function: Prospective analysis of serial patients managed in a dedicated multidisciplinary clinic.

INTRODUCTION: Posterior urethral valves (PUV) is a chronic condition that can adversely impact child and family health related quality of life (HRQOL). Surprisingly, to date, the impact of this condition on the QOL of children with PUV has not been studied. Thus, our aim is to evaluate this important aspect of care using validated questionnaires in our institutional PUV clinic. METHODS: From December 2022 to January 2024, the PedsQL Inventory and associated family impact module was distributed in our PUV clinic. Higher scores reflect better HRQoL and less impact on family. Children >8 years old completed the tool themselves, otherwise parents completed the tools on behalf of the child. We compared PUV scores against reported psychometric data from healthy children and other chronic illnesses. RESULTS: A total of 112 families completed the questionnaires. The total HRQoL score was 80 ± 13. Overall, we noted that the domains reflecting psychosocial impact were significantly lower than those of physical well-being (76 ± 16 vs. 89 ± 15 for psychosocial and physical impact, respectively; p < 0.01). There was a significant difference in the psychosocial emotional impact for children managed with clean intermittent catheterization (CIC) compared to those who were not (69 ± 21 vs. 81 ± 16; p = 0.005) (Table 2). In addition, we noted that children with PUV scored similar to healthy controls, but higher than children with other chronic illnesses. With respect to the impact on family, overall, parent, and family impacts were 76 ± 19, 78 ± 21, and 81 ± 21, respectively. DISCUSSION: Chronic illness has been associated with a negative impact on HRQoL in both adults and children. We noted similar findings the present study, particularly for young children and those who are maintained on CIC. To our knowledge, this is the first study to evaluate the impact of PUV on a child's HRQoL as well as the impact on the family, and to document a negative psychosocial impact of CIC in this population. However, there are some important limitations to be addressed including relying on families to complete questionnaires accurately during busy clinic visits and the lack of controls from the same environment. CONCLUSION: When compared to healthy controls, PUV patients score similarly in HRQoL assessment. In addition, children <3years of age report a negative impact on physical well being, and CIC negatively impacts the emotional well-being. These data can be used to advocate for better mental health supports and social work for these children.