POS-446 SPECIFIC FEATURES AND OUTCOME OF NEPHROCALCINOSIS IN CHILDREN: ABOUT A SERIES OF 30 CASES

Abstract

Nephrocalcinosis is a rare pediatric renal disease resulting in calcifications of the kidney tissue. It comes from various causes, especially genetic mutations. Management is based on symptomatic measures and treatment of the underlying condition. The aim of this study (from January 2010 to June 2020) is to underly the specific features of nephrocalcinosis in our pediatric population. For this, we gathered retrospective data of 30 children diagnosed with nephrocalcinosis. The mean age at diagnosis was of 3,5 years. One-third of our patients had first-degree consanguineous parents, while two-thirds of them were males. Similar cases in siblings and family members were absent. The majority of children presented with polyuria, polydipsia, and dehydration at diagnosis. Also, half of them had simultaneous urologic calculus. In five cases, nephrocalcinosis was discovered on routine abdominal ultrasound while the children were asymptomatic. The sonographic classification was in favor of stage III nephrocalcinosis in 23 cases. The underlying disease was represented mostly by tubulopathies in 40% of cases. Distal tubular acidosis was predominant. Nephrocalcinosis secondary to recurrent urinary tract infections was seen in 10% of our children. While, prematurity, primary hyperoxaluria, and familial hypercalciuria were rare (both seen in 10% of children). One child developed chronic kidney disease stage II after a follow up of seven years. Meanwhile, management was successful in stopping the evolution of the disease in all the remaining cases. It consisted on hyperhydration in all cases, alkalinization of urine in the case of acidosis, along with the specific treatment of the underlying disease. Early management of nephrocalcinosis in children can prevent the evolution of chronic kidney disease. Systematic screening should be implemented in high-risk children, especially those with tubulopathies. The high frequency of consanguineous marriages in our population suggests a genetic predisposition that needs to be proven by further studies.