Nephrocalcinosis among children at king hussein medical center: Causes and outcome

Abstract

Nephrocalcinosis (NC) is defined as deposition of calcium crystals in the renal parenchyma and tubules. This is a retrospective review of all the data of 63 children presented to Pediatric Nephrology Clinic at King Hussein Medical Center (KHMC) over a 15-year period with bilateral NC. We determine their causes, clinical presentation and evaluate their growth and renal function during their follow-up. Thirty-five (55.5%) cases were males and 28 (44.5%) were females. The median (range) age at presentation was four (2-192) months. The most common leading cause to NC was hereditary tubulopathy in 48% followed by hyperoxaluria in 35%. The cause of NC remained unknown in 3% and Vitamin D excess accounts for 5% of the cases. The most presenting symptom was a failure to thrive (68%) and the second most common symptom was abdominal pain and recurrent urinary tract infection was found in 40%, polyuria and polydipsia were found in 32% of cases, and 16% of cases were diagnosed incidentally. At a median follow-up of 38 (14-200) months, estimated glomerular filtration rate had decreased from 84.0 (42-110) mL/min per 1.73 m2 body surface area to 68.2 (10-110) mL/min/1.73 m2 body surface (P = 0.001). This study illustrated the need for a national registry of rare renal diseases to help understand the causes of these conditions in our populations and provide support to affected patients and their families.

Nephrocalcinosis in a portuguese pediatric population

“…In our study, a high proportion of patients (34%, n=12) presented failure to thrive in the first year of life, considered a clinical sign that should be investigated, as it can encompass a wide spectrum of causes. In other studies 14,15 , 55% to 68% of patients also presented with failure to thrive in the first year. We believe that our finding may be in relation to the young age of our cohort (40% under two years old) and to prematurity (five of 12 patients).…”

Caracterización fenotípica del síndrome amelogénesis imperfecta–nefrocalcinosis: una revisión

“…Aunque la NC es responsable de una serie de problemas clínicos, generalmente es detectada gracias a la investigación radiológica simple, ultrasonido o tomografía computarizada. Desde el inicio la NC es asintomática, pero en ocasiones los cólicos renales pueden ser el primer síntoma referido por el paciente, probablemente debido a una nefrolitiasis asociada o por la extrusión de un nódulo calcificado 6,7 .…”

“…Sin embargo, el cólico renal es el síntoma más frecuente cuando la NC es sintomática, probablemente debido a una nefrolitiasis asociada o por la extrusión de un nódulo calcificado. La progresión de la NC hacia la enfermedad renal crónica, justifica una evaluación rigurosa por el nefrólogo que permita la preservación renal 6 . Wrong 7 clasificaron la NC en tres tipos diferentes; la primera es la NC molecular, en la que se registra un aumento del calcio intracelular a nivel renal pero sin formación de cristales; la segunda es la NC microscópica, en la que el fosfato de calcio pudiese identificarse a través de la microscopía óptica pero no radiológicamente; y la tercera es la NC macroscópica, en la que la calcificación producida por las sales de calcio es visible radiológicamente.…”

Clinical outcomes of nephrocalcinosis in preschool-age children: association between nephrocalcinosis improvement and long-term kidney function

“…A few retrospective studies on pediatric NC have been conducted in several centers over the years (2)(3)(4)(5)(6)(7). Their results recommended the early diagnosis of underlying diseases for improving kidney outcomes.…”