succimer and Bladder-Exstrophy

Historically, repair of bladder exstrophy (BE) is associated with compromise to the upper tracts; the single stage repair of BE was considered to exacerbate risks of kidney impairment.. We aim to evaluate the risk of upper urinary tract deterioration or chronic kidney disease after the complete primary repair of exstrophy (CPRE).. As part of the U.S.-India Multi-institutional Bladder Exstrophy Collaboration, we prospectively performed data collection on all patients managed at the Civil Hospital, Ahmedabad from 2010 to 2020. All patients who underwent primary or redo BE or primary penopubic epispadias (PE) repair using CPRE were included. Data on annual VCUG and DMSA, serum creatinine and cystatin-C, urinary albumin, and creatinine were aggregated.. 72/104 patients who underwent CPRE at a median age of 1.7 years (IQR: 1.1-4.6) were included: 43(60%) patients with primary BE, 17(24%) with redo BE, and 12(17%) with primary PE. At a median follow-up of 4 years (IQR: 3-6), the overall median eGFR was 105 for BE, and 128 ml/min for PE. 14(19%) patients had eGFR<90, and 22(31%) had microalbuminuria. 21(30%) patients had kidney scarring in DMSA and 31(44%) had VUR. Multivariate analysis showed that neither kidney scarring nor VUR could predict the presence of eGFR<90 or microalbuminuria. Of 72 patients, 2 (3%) patients had dry intervals >3 h, 9 (13%) patients have dry intervals of 1-3 h and 44 (61%) patients had dry intervals <1 h during follow-up. We found that kidney function outcomes (i.e., eGFR and microalbuminuria) were not associated with continence status (p = 0.3).. In this series, we report a 5% incidence of CKD stage 2 or above that was not impacted by continence status. Furthermore, a 40% incidence of VUR and a 30% incidence of kidney scarring during follow-up was observed within this cohort, neither of which had a significant impact on renal function deterioration (i.e, decline in eGFR), but underscores the need for close kidney surveillance in children that have undergone bladder exstrophy repair.. Modern CPRE technique for the repair of BE may increase the risk of kidney scarring in the intermediate-term follow-up, however, this finding does not correlate with low eGFR and presence of albuminuria inpatients. Therefore, close follow-up with serial kidney function measurements is warranted and necessary after CPRE.

Topics: Bladder Exstrophy; Child; Child, Preschool; Epispadias; Glomerulonephritis; Humans; Infant; Kidney; Succimer

Exstrophy-epispadias complex (EEC) is a complex malformation of the lower abdominal wall, bladder, and pelvic floor, which necessitates multiple successive reconstruction procedures. Surgical and infectious complications are frequent. Our aim was to evaluate kidney function in these patients.. This cross-sectional study included patients with EEC, followed since birth in a pediatric urology clinic, who underwent nephrological evaluation (blood pressure (BP) measurement and blood and urine chemistries) and imaging studies (urinary tract ultrasound and DMSA kidney scan) during 2017-2020.. The high rate of signs of kidney injury in pediatric patients with EEC dictates early-onset long-term kidney function monitoring by joint pediatric urological and nephrological teams. A higher resolution version of the Graphical abstract is available as Supplementary information.

Topics: Bladder Exstrophy; Child; Cross-Sectional Studies; Epispadias; Humans; Hypertension; Kidney; Male; Succimer

Twenty-eight children with bladder exstrophy, prior to surgery for continence, underwent a urodynamic evaluation, cystogram, ultrasound and dimercaptosuccinic acid scan, to define parameters of lower urinary tract function that are a risk for upper tract damage and/or impair development of bladder capacity. The bladders in 7 of 28 demonstrated no storage function [leak pressure)LP) = 0]; but storage parameters could be measured in the other 21. Their LP was 10-35 cm H2O; 17 of 21 revealed involuntary contractions and 7 of 21 had an end-fill pressure greater than 10 cm H2O. Bladder capacity was better in those with a higher LP and those with no involuntary contractions. The 4 patients with bilateral hydronephrosis (3 of whom also had bilateral multiple scars) were among the 6 with LP greater than 30 cm H2O. Of the 7 with an end-fill pressure greater than 10 cm H2O, 5 had upper tract damage. Involuntary contractions may impair development of capacity. A LP of greater than 30 cm H2O is a risk factor for upper tract damage and an end-fill pressure greater than 10 cm H2O may also be a factor.

Topics: Bladder Exstrophy; Child; Child, Preschool; Female; Humans; Infant; Male; Succimer; Ultrasonography; Urinary Incontinence; Urinary Tract; Urinary Tract Physiological Phenomena; Urodynamics