Nephrocalcinosis in a portuguese pediatric population

Introduction and Aims: Nephrocalcinosis is characterized by the deposition of calcium in the kidney parenchyma and tubules. The renal prognosis depends on the underlying cause, emphasizing the importance of its identification. We aim to review the data of children with nephrocalcinosis concerning etiology, clinical manifestations, growth and renal function at presentation and outcomes. Methods: Retrospective study of the records of children (<18 years) with nephrocalcinosis followed by a pediatric nephrology unit of level III hospital, between 2008-17. Clinical features, etiology, treatment and outcomes were evaluated. Results: We identified 35 cases: 24 isolated (69%) and 11 with nephrolithiasis (31%). The group was mostly constituted of girls (54%). Median age at presentation was 6 years (7 months - 17 years old); 40% of patients were under 2 years of age, 31% between 3 and 9 years and 29% older than 10 years. Mean follow-up was 4 years (1-9). The most common clinical manifestation was failure to thrive in the first year of life (34%) and flank or abdominal pain (20%); in 23% it was an incidental finding. Eleven percent of patients had a systemic syndromic disease. Renal function at diagnosis was normal in all children. The most frequent causes were metabolic abnormalities (23%), hereditary tubulopathies (23%), prematurity (20%) and pharmacologic (14%). Eleven percent were considered idiopathic. In a logistic regression analysis, sex, age of presentation and familiar history of nephrocalcinosis showed no correlation with nephrocalcinosis, nephrocalcinosis and nephrolithiasis or hereditary/metabolic etiologies. Discussion: Despite the small sample, in this study, the hereditary and/or metabolic disorders were the main cause of nephrocalcinosis. Associated symptoms and comorbidities, such as prematurity, growth retardation, intestinal malabsorption, or bone demineralization, should be evaluated for diagnostic purposes. No patient developed chronic kidney disease.

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Main Authors: Bota,Sofia, Andrade,Joana V, Francisco,Telma, Santos,Raquel, Neto,Gisela, Abranches,Margarida
Format: Digital revista
Language:English
Published: Sociedade Portuguesa de Nefrologia 2019
Online Access:http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000100004
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spelling oai:scielo:S0872-016920190001000042019-05-20Nephrocalcinosis in a portuguese pediatric populationBota,SofiaAndrade,Joana VFrancisco,TelmaSantos,RaquelNeto,GiselaAbranches,Margarida nephrocalcinosis nephrolithiasis children Portuguese Introduction and Aims: Nephrocalcinosis is characterized by the deposition of calcium in the kidney parenchyma and tubules. The renal prognosis depends on the underlying cause, emphasizing the importance of its identification. We aim to review the data of children with nephrocalcinosis concerning etiology, clinical manifestations, growth and renal function at presentation and outcomes. Methods: Retrospective study of the records of children (<18 years) with nephrocalcinosis followed by a pediatric nephrology unit of level III hospital, between 2008-17. Clinical features, etiology, treatment and outcomes were evaluated. Results: We identified 35 cases: 24 isolated (69%) and 11 with nephrolithiasis (31%). The group was mostly constituted of girls (54%). Median age at presentation was 6 years (7 months - 17 years old); 40% of patients were under 2 years of age, 31% between 3 and 9 years and 29% older than 10 years. Mean follow-up was 4 years (1-9). The most common clinical manifestation was failure to thrive in the first year of life (34%) and flank or abdominal pain (20%); in 23% it was an incidental finding. Eleven percent of patients had a systemic syndromic disease. Renal function at diagnosis was normal in all children. The most frequent causes were metabolic abnormalities (23%), hereditary tubulopathies (23%), prematurity (20%) and pharmacologic (14%). Eleven percent were considered idiopathic. In a logistic regression analysis, sex, age of presentation and familiar history of nephrocalcinosis showed no correlation with nephrocalcinosis, nephrocalcinosis and nephrolithiasis or hereditary/metabolic etiologies. Discussion: Despite the small sample, in this study, the hereditary and/or metabolic disorders were the main cause of nephrocalcinosis. Associated symptoms and comorbidities, such as prematurity, growth retardation, intestinal malabsorption, or bone demineralization, should be evaluated for diagnostic purposes. No patient developed chronic kidney disease.info:eu-repo/semantics/openAccessSociedade Portuguesa de NefrologiaPortuguese Journal of Nephrology &amp; Hypertension v.33 n.1 20192019-03-01info:eu-repo/semantics/articletext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000100004en10.32932/pjnh.2019.04.005
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country Portugal
countrycode PT
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databasecode rev-scielo-pt
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libraryname SciELO
language English
format Digital
author Bota,Sofia
Andrade,Joana V
Francisco,Telma
Santos,Raquel
Neto,Gisela
Abranches,Margarida
spellingShingle Bota,Sofia
Andrade,Joana V
Francisco,Telma
Santos,Raquel
Neto,Gisela
Abranches,Margarida
Nephrocalcinosis in a portuguese pediatric population
author_facet Bota,Sofia
Andrade,Joana V
Francisco,Telma
Santos,Raquel
Neto,Gisela
Abranches,Margarida
author_sort Bota,Sofia
title Nephrocalcinosis in a portuguese pediatric population
title_short Nephrocalcinosis in a portuguese pediatric population
title_full Nephrocalcinosis in a portuguese pediatric population
title_fullStr Nephrocalcinosis in a portuguese pediatric population
title_full_unstemmed Nephrocalcinosis in a portuguese pediatric population
title_sort nephrocalcinosis in a portuguese pediatric population
description Introduction and Aims: Nephrocalcinosis is characterized by the deposition of calcium in the kidney parenchyma and tubules. The renal prognosis depends on the underlying cause, emphasizing the importance of its identification. We aim to review the data of children with nephrocalcinosis concerning etiology, clinical manifestations, growth and renal function at presentation and outcomes. Methods: Retrospective study of the records of children (<18 years) with nephrocalcinosis followed by a pediatric nephrology unit of level III hospital, between 2008-17. Clinical features, etiology, treatment and outcomes were evaluated. Results: We identified 35 cases: 24 isolated (69%) and 11 with nephrolithiasis (31%). The group was mostly constituted of girls (54%). Median age at presentation was 6 years (7 months - 17 years old); 40% of patients were under 2 years of age, 31% between 3 and 9 years and 29% older than 10 years. Mean follow-up was 4 years (1-9). The most common clinical manifestation was failure to thrive in the first year of life (34%) and flank or abdominal pain (20%); in 23% it was an incidental finding. Eleven percent of patients had a systemic syndromic disease. Renal function at diagnosis was normal in all children. The most frequent causes were metabolic abnormalities (23%), hereditary tubulopathies (23%), prematurity (20%) and pharmacologic (14%). Eleven percent were considered idiopathic. In a logistic regression analysis, sex, age of presentation and familiar history of nephrocalcinosis showed no correlation with nephrocalcinosis, nephrocalcinosis and nephrolithiasis or hereditary/metabolic etiologies. Discussion: Despite the small sample, in this study, the hereditary and/or metabolic disorders were the main cause of nephrocalcinosis. Associated symptoms and comorbidities, such as prematurity, growth retardation, intestinal malabsorption, or bone demineralization, should be evaluated for diagnostic purposes. No patient developed chronic kidney disease.
publisher Sociedade Portuguesa de Nefrologia
publishDate 2019
url http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000100004
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