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Nephrocalcinosis is a risk factor for kidney failure in primary hyperoxaluria.

ABSTRACT: Stone formation and nephrocalcinosis are both very common features of primary hyperoxaluria, yet the extent of each disease varies markedly between patients. Here we studied whether kidney damage from nephrocalcinosis and/or stone related events contributed to end-stage kidney disease (ESKD). Clinical information was analyzed from 348 patients enrolled in the Rare Kidney Stone Consortium Primary Hyperoxaluria registry and included demographic, laboratory and imaging features. Among all patients there were 277 with type 1, 37 with type 2, and 34 with type 3 primary hyperoxaluria. Overall, 58% passed a stone (mean 0.3/year) and one or more urologic procedures were required by 70% of patients (mean 0.15/year). Nephrocalcinosis was found in 34% of patients, including 41% with type 1 primary hyperoxaluria. High urine oxalate was associated with increased risk for both nephrocalcinosis and stone number, while low urine citrate was a risk factor for stone events and stone number. After adjustment for the type of primary hyperoxaluria, diagnosis by family screening and age at first image, the overall adjusted hazard ratio for ESKD among those with a history of nephrocalcinosis was 1.7 [95% CI 1.0-3.0], while the risk was 4.0 [1.9-8.5] for new onset nephrocalcinosis during follow-up. In contrast, the number of stones and stone events were not significantly associated with ESKD risk. Thus, nephrolithiasis and nephrocalcinosis appear to be pathophysiologically distinct entities. The presence of nephrocalcinosis implies increased risk for ESKD.

SUBMITTER: Tang X

PROVIDER: S-EPMC4344931 | biostudies-literature | 2015 Mar

REPOSITORIES: biostudies-literature

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Nephrocalcinosis is a risk factor for kidney failure in primary hyperoxaluria.

Tang Xiaojing X Bergstralh Eric J EJ Mehta Ramila A RA Vrtiska Terri J TJ Milliner Dawn S DS Lieske John C JC

Kidney international 20140917 3

Stone formation and nephrocalcinosis are both very common features of primary hyperoxaluria, yet the extent of each disease varies markedly between patients. Here we studied whether kidney damage from nephrocalcinosis and/or stone related events contributed to end-stage kidney disease (ESKD). Clinical information was analyzed from 348 patients enrolled in the Rare Kidney Stone Consortium Primary Hyperoxaluria registry and included demographic, laboratory and imaging features. Among all patients ...[more]

PMID: 25229337

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Project description:BackgroundPrimary hyperoxaluria type 1 (PH1) is associated with nephrocalcinosis (NC) and calcium oxalate (CaOx) kidney stones (KS). Populations of urinary extracellular vesicles (EVs) can reflect kidney pathology. The aim of this study was to determine whether urinary EVs carrying specific biomarkers and proteins differ among PH1 patients with NC, KS or with neither disease process.MethodsMayo Clinic Rare Kidney Stone Consortium bio-banked cell-free urine from male and female PH1 patients without (n?=?10) and with NC (n?=?6) or KS (n?=?9) and an eGFR?>?40 mL/min/1.73 m2 were studied. Urinary EVs were quantified by digital flow cytometer and results expressed as EVs/ mg creatinine. Expressions of urinary proteins were measured by customized antibody array and results expressed as relative intensity. Data were analyzed by ANCOVA adjusting for sex, and biomarkers differences were considered statistically significant among groups at a false discovery rate threshold of Q?<?0.20.ResultsTotal EVs and EVs from different types of glomerular and renal tubular cells (11/13 markers) were significantly (Q?<?0.20) altered among PH1 patients without NC and KS, patients with NC or patients with KS alone. Three cellular adhesion/inflammatory (ICAM-1, MCP-1, and tissue factor) markers carrying EVs were statistically (Q?<?0.20) different between PH1 patients groups. Three renal injury (?2-microglobulin, laminin ?5, and NGAL) marker-positive urinary EVs out of 5 marker assayed were statistically (Q?<?0.20) different among PH1 patients without and with NC or KS. The number of immune/inflammatory cell-derived (8 different cell markers positive) EVs were statistically (Q?<?0.20) different between PH1 patients groups. EV generation markers (ANO4 and HIP1) and renal calcium/phosphate regulation or calcifying matrixvesicles markers (klotho, PiT1/2) were also statistically (Q?<?0.20) different between PH1 patients groups. Only 13 (CD14, CD40, CFVII, CRP, E-cadherin, EGFR, endoglin, fetuin A, MCP-1, neprilysin, OPN, OPGN, and PDGFR?) out of 40 proteins were significantly (Q?<?0.20) different between PH1 patients without and with NC or KS.ConclusionsThese results imply activation of distinct renal tubular and interstitial cell populations and processes associated with KS and NC, and suggest specific populations of urinary EVs and proteins are potential biomarkers to assess the pathogenic mechanisms between KS versus NC among PH1 patients.

Dataset Information

Nephrocalcinosis is a risk factor for kidney failure in primary hyperoxaluria.

Publications

Nephrocalcinosis is a risk factor for kidney failure in primary hyperoxaluria.

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