Dataset Information

Predictors of Incident ESRD among Patients with Primary Hyperoxaluria Presenting Prior to Kidney Failure.

ABSTRACT:

Background and objectives

Overproduction of oxalate in patients with primary hyperoxaluria (PH) leads to calcium oxalate deposition in the kidney and ESRD in a substantial number of cases. However, the key determinants for renal outcome remain unclear. Thus, we performed a retrospective analysis to identify predictors for renal outcome among patients with PH participating in the Rare Kidney Stone Consortium (RKSC) PH Registry.

Design, setting, participants, & measurements

We characterized clinical and laboratory features of patients enrolled in the RKSC PH Registry. We assessed correlation between urinary measures and eGFR at diagnosis by Spearman rank correlation and estimated renal survival using the Kaplan-Meier method. We determined factors associated with renal survival by Cox proportional hazard models.

Results

Of 409 patients enrolled in the RKSC Registry as of March 2014, we excluded 112 patients who had ESRD at PH diagnosis from analysis. Among the remaining 297 patients, 65% had PH type 1, 12% had type 2, 13% had type 3, and 11% had unclassified PH. Median (25th, 75th percentile) age at PH diagnosis was 8.1 (4.0, 18.2) years with an eGFR of 73.0 (56.4, 97.5) ml/min per 1.73 m(2) and urinary oxalate excretion rate of 1.64 (1.11, 2.44) mmol/1.73 m(2) per 24 hours. During a median follow-up of 3.9 (1.0, 12.8) years, 59 (20%) patients developed ESRD. Urinary oxalate excretion at diagnosis stratified by quartile was strongly associated with incident ESRD (hazard ratio [HR], 3.4; 95% confidence interval [95% CI], 1.4 to 7.9). During follow-up there was a significant association between urinary oxalate quartile (Q) and incident ESRD (Q4 versus Q1: HR, 3.3; 95% CI, 1.2 to 9.3). This association remained even when adjusted for sex, age, and baseline eGFR (HR, 4.2; 95% CI, 1.6 to 10.8).

Conclusions

Among patients with PH, higher urinary oxalate excretion is predictive of poor renal outcome.

SUBMITTER: Zhao F

PROVIDER: S-EPMC4702224 | biostudies-literature | 2016 Jan

REPOSITORIES: biostudies-literature

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Publications

Predictors of Incident ESRD among Patients with Primary Hyperoxaluria Presenting Prior to Kidney Failure.

Zhao Fang F Bergstralh Eric J EJ Mehta Ramila A RA Vaughan Lisa E LE Olson Julie B JB Seide Barbara M BM Meek Alicia M AM Cogal Andrea G AG Lieske John C JC Milliner Dawn S DS

Clinical journal of the American Society of Nephrology : CJASN 20151210 1

<h4>Background and objectives</h4>Overproduction of oxalate in patients with primary hyperoxaluria (PH) leads to calcium oxalate deposition in the kidney and ESRD in a substantial number of cases. However, the key determinants for renal outcome remain unclear. Thus, we performed a retrospective analysis to identify predictors for renal outcome among patients with PH participating in the Rare Kidney Stone Consortium (RKSC) PH Registry.<h4>Design, setting, participants, & measurements</h4>We chara ...[more]

PMID: 26656319

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Project description:RationalePrimary hyperoxaluria (PH) is a rare autosomal recessive disorder more commonly diagnosed in children or adolescents. Owing to its rarity and heterogeneous phenotype, it is often underrecognized, resulting in delayed diagnosis, including diagnosis after end-stage kidney disease (ESKD) has occurred or recurrence after kidney-only transplantation.Case presentationA 40-year-old Caucasian Canadian woman with a history of recurrent nephrolithiasis since age 19 presented with ESKD and cutaneous symptoms. She had no known prior kidney disease and no family history of kidney disease or nephrolithiasis.DiagnosisA diagnosis of primary hyperoxaluria type 1 (PH1) due to homozygous splice donor mutation (AGXT c.680+1G>A) was made with kidney and cutaneous pathology demonstrating calcium oxalate deposition and ultrasound suggestive of nephrocalcinosis.InterventionsShe was initiated on frequent, high-efficiency, high-flux conventional hemodialysis and oral pyridoxine. Lumasiran was added 11 months later, after she developed bilateral swan-neck deformities.OutcomesAfter 14 months of high-intensity dialysis and 3 months of lumasiran, there have been no signs of renal recovery, and extra-renal involvement has increased with progressive swan-neck deformities, reduced cardiac systolic function, and pulmonary hypertension. The patient has been waitlisted for kidney-liver transplantation.Teaching pointsThis case report describes an adult presentation of PH1. The case highlights the importance of timely workup of metabolic causes of recurrent nephrolithiasis or nephrocalcinosis in adults which can be a presenting sign of PH and genetic testing for PH to facilitate early diagnosis and treatment especially in the era of novel therapeutics that may alter disease course and outcomes. The case also demonstrates the value of testing for PH in adults presenting with unexplained ESKD and a history of recurrent nephrolithiasis or nephrocalcinosis due to implications for organ transplantation strategy and presymptomatic family screening.

Project description:RATIONALE & OBJECTIVE:Atrial fibrillation (AF) is common in patients with kidney failure treated by maintenance dialysis. Whether the incidence of AF differs between patients receiving hemodialysis and peritoneal dialysis is uncertain. STUDY DESIGN:Retrospective cohort study. SETTING & PARTICIPANTS:Using the US Renal Data System, we identified older patients (?67 years) with Medicare Parts A and B who initiated dialysis therapy (1996-2011) without a diagnosis of AF during the prior 2 years. EXPOSURE:Dialysis modality at incident end-stage renal disease (ESRD) and maintained for at least 90 days. OUTCOME:Patients were followed up for 36 months or less for a new diagnosis of AF. ANALYTICAL APPROACH:Time-to-event analysis using multivariable Cox proportional hazards regression to estimate cause-specific HRs while censoring at modality switch, kidney transplantation, or death. RESULTS:Overall, 271,722 older patients were eligible; 17,487 (6.9%) were treated with peritoneal dialysis, and 254,235 (93.1%), with hemodialysis, at the onset of ESRD. During 406,225 person-years of follow-up, 69,705 patients had AF newly diagnosed. Because the proportionality assumption was violated, we introduced an interaction term between time (first 90 days vs thereafter) and modality. The AF incidence during the first 90 days was 187/1,000 person-years on peritoneal dialysis therapy and 372/1,000 person-years on hemodialysis therapy. Patients on peritoneal dialysis therapy had an adjusted 39% (95% CI, 34%-43%) lower incidence of AF than those on hemodialysis therapy. From day 91 onward, AF incidence was ?140/1,000 person-years with no major difference between modalities. LIMITATIONS:Residual confounding from unobserved differences between exposure groups; ascertainment of AF from billing claims; study of first modality may not generalize to patients switching modalities; uncertain generalizability to younger patients. CONCLUSIONS:Although patients initiating dialysis therapy using peritoneal dialysis had a lower AF incidence during the first 90 days of ESRD, there was no major difference in AF incidence thereafter. The value of interventions to reduce the early excess AF risk in patients receiving hemodialysis may warrant further study.

Dataset Information

Predictors of Incident ESRD among Patients with Primary Hyperoxaluria Presenting Prior to Kidney Failure.

Background and objectives

Design, setting, participants, & measurements

Results

Conclusions

Publications

Predictors of Incident ESRD among Patients with Primary Hyperoxaluria Presenting Prior to Kidney Failure.

Similar Datasets

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