Evaluation of the patient’s urine, specically focusing on the degree of protein­uria/albuminuria, can provide a baseline from which to compare subsequent studies and estimate the risk of later CKD.Similarly, attention should be paid to the serum electrolytes, especially calcium, magnesium, potassium, and phosphate, which can be markers of tubular injury. In the setting of kidney stones, urinary measurements of calcium, oxalate, uric acid, and creatinine can be informative along with a 24-hour urine collection for a stone risk prole. Increased hydration and a low-sodium diet can be helpful in the prevention and management of kidney stones prior to initiation of diuretic medications.

Hypertension in pediatric patients with urologic abnormalities is a known complica­tion and is common among children with CKD.In a follow-up study of 36 children with BE who underwent complete primary repair at a single institution, investiga­tors averaged clinic visits and blood pressure readings on the same day for patients and included readings from multiple visits over time. Reimplantation, lower kidney length on US, and higher eGFR were associated with a decreased hazard of systolic or diastolic blood pressure readings >90th percentile for age and sex. No associa­tions were found between the presence of hydronephrosis, urinary continence, pyelonephritis, and elevated systolic or diastolic blood pressures [17]. None of the patients (mean age of 8.9years) had a systolic or diastolic blood pressure >90th percentile for age and sex, were diagnosed with hypertension, or required antihy­pertensive therapy.

The progression of CKD to ESKD results in many of the health consequences of this disease; however, the natural history and the rate of progression are both highly variable and unpredictable. Pediatric data suggests that there is a slower progression of disease in those with congenital renal disorders when compared to those with glomerular etiologies of their CKD [20]. Studies have demonstrated that the pro­gression of CKD can be inuenced by multiple factors, many of which are not modiable (e.g., underlying renal pathology, genetics, race, age, and gender) [21]. However, some risk factors can be modied, such as obesity, hypertension, and proteinuria [22]. There is evidence in pediatric studies that hypertension and pro­teinuria are the most signicant risk factors for CKD progression; minimizing pro­teinuria and maximizing blood pressure control, targeting the 50th percentile based on age, sex, and height, can slow progression of CKD [23, 24]. Treatment with either angiotensin-converting enzyme inhibitors (ACE inhibitor), or angiotensin II receptor blockers (ARBs) has been shown to be more effective for both blood pres­sure control and reduction in proteinuria when compared to other agents currently available [25].

It is postulated that proteinuria contributes to CKD progression due to tubular damage resulting in interstitial inammation, brosis, and subsequent apoptosis of proximal tubular cells [26]. In a pediatric study of 1232 children combining the CKiD participants and children in the ESCAPE trial, investigators found that pro­teinuria (dened as urine protein to creatinine ratio (UPCR) on a spot sample in mg/ mg) contributes to CKD progression. The median age in this combined cohort was 12years (IQR, 8–15), and the median eGFR 47mL/min/1.73m2 (IQR, 33–62). The authors reported six ordered stages with varying combinations of eGFR categories (60–89, 45–59, 30–44, and 15–29mL/min/1.73m2) and UPCR categories (<0.5,

The goal and optimal treatment for ESKD is kidney transplant. Kidney transplant has been done successfully in children with urologic disorders, and graft survival rates at 1 and 5years are comparable to those without urologic disorders. However, in one study of 55 patients who underwent 56 kidney transplants (only 3 had pri­mary BE as the reason for ESKD), investigators found graft survival at 1year was 89% and at 5years 66%. They reported that those with a normal bladder fared better than those with an abnormal bladder at 5 years post-transplant (75% vs. 57%, respectively) and those with abnormal bladders had worse kidney function at 5years. They also found worse kidney function in those with reux and urinary tract infections post-transplant. High-pressure systems and residual urine volumes after voiding also contributed to the overall decline in kidney function [29]. A retrospec­tive study from three transplant centers in France that included 14 patients with BE who had a kidney transplant at a median age of 42.8years also found that graft survival was good, with 93% graft survival after a median follow-up of 8.2years; graft survival out to 20years was the same [30]. This study did not nd an associa­tion between pyelonephritis and graft dysfunction. Both studies emphasize the importance of proper work-up and evaluation of bladder function and bladder pres­sure, evaluation for reux, and urodynamic studies to measure post-void residual prior to kidney transplantation in this population of patients. And some advocate for prophylactic antibiotics in the rst 6months post-transplant to prevent urinary tract infections [29].

• Recipe for saline—To make saline, use 4 cups (1000mL) of distilled water or 4

solved. Label the container (bottle or jar) with the date and store it in the refrig-