Abstract
Patients with congenital kidney and urinary tract abnormalities (CAKUT) will often develop end-stage renal disease at some point and the need for renal replacement therapy is associated with high rates of morbidity and mortality. Hence, efforts to slow the progression of the disease are essential. Hypertension has been proven to be an independent risk factor for faster decline of glomerular filtration rate in renal patients, but studies involving only children with CAKUT are scarce. We performed a literature review to explore the association of hypertension with faster chronic kidney disease progression in children with CAKUT and also treatment options in this condition. A recent study reported an annual decline in GFR of 1.8 ml/min/1.73 m2 among hypertensive patients with non-glomerular CKD, compared with 0.8 ml/min/1.73 m2 in normotensive children. A multicenter prospective cohort in Brazil showed that a 1-unit increase in systolic blood pressure Z-score was associated with a 1.5-fold higher risk of disease progression. Since renin-angiotensin-aldosterone system activation is the most important mechanism of hypertension in these children, the first-line therapy involves the use of inhibitors of this axis, including angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers type I, which also promote an anti-fibrotic effect. Recent studies have shown a good safety profile for use in patients with chronic kidney disease and also in those with solitary kidneys. Hypertension is an independent risk factor for kidney disease progression and should be promptly managed for renal protection, especially among patients with CAKUT, the primary cause of chronic kidney disease in the pediatric population.
Highlights
Congenital anomalies of the kidney and urinary tract (CAKUT) are the primary cause of chronic kidney disease (CKD) in the pediatric population [1,2,3,4]
end-stage renal disease (ESRD) is associated with high morbidity and mortality rates and strategies to reduce the rate of CKD progression and delay renal replacement therapy can be crucial for improving life expectancy and quality of life of patients
In their 2017 guideline, the Academy of Pediatrics (AAP) advises that, irrespective of apparent control of BP based on office measurements, children and adolescents with CKD and history of hypertension should have BP assessed by ambulatory blood pressure monitoring (ABPM) at least yearly to screen for masked hypertension [25]
Summary
Congenital anomalies of the kidney and urinary tract (CAKUT) are the primary cause of chronic kidney disease (CKD) in the pediatric population [1,2,3,4]. Bilateral renal hypoplasia and dysplasia, with or without concomitant urinary tract malformation, are present in over 50% of children and adolescents requiring renal replacement therapy [2]. According to data published for the Chronic Kidney Disease in Children (CKiD) cohort in 2015, of the 689 children involved, 76% had a non-glomerular cause for CKD, of which 69% were CAKUT-associated: 25% obstructive uropathy; 21% aplasia, hypoplasia or renal dysplasia; 19% reflux nephropathy; and 4% other CAKUTs [3]. For previous registries reporting CKD etiology in infancy, the NAPRTCS found CAKUT in 48% of cases and the ItalKid in 58% [5, 6]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
