Dataset Information

Beyond Loss of Kidney Function: Patient Care in Autosomal Dominant Polycystic Kidney Disease.

ABSTRACT: Patients with autosomal dominant polycystic kidney disease benefit from specialized care over their lifetimes, starting with diagnosis of the condition with ongoing discussion of both the renal course and extra-renal issues. Both renal and extra-renal issues may continue to cause major morbidity even after successful kidney transplant or initiation of RRT, and extra-renal disease aspects should always be considered as part of routine management. In this review, we will focus on updates in pain/depression screening, cardiac manifestations, liver and pancreatic cysts, kidney stone management, and genetic counseling. In some instances, we have shared our current clinical practice rather than an evidence-based guideline. We anticipate more standardization of care after the release of the Kidney Disease Improving Global Outcomes guidelines for management in autosomal dominant polycystic kidney disease later this year.

SUBMITTER: Hogan MC

PROVIDER: S-EPMC10758524 | biostudies-literature | 2023 Dec

REPOSITORIES: biostudies-literature

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Beyond Loss of Kidney Function: Patient Care in Autosomal Dominant Polycystic Kidney Disease.

Hogan Marie C MC Simmons Kathryn K Ullman Lawrence L Gondal Maryam M Dahl Neera K NK

Kidney360 20231127 12

Patients with autosomal dominant polycystic kidney disease benefit from specialized care over their lifetimes, starting with diagnosis of the condition with ongoing discussion of both the renal course and extra-renal issues. Both renal and extra-renal issues may continue to cause major morbidity even after successful kidney transplant or initiation of RRT, and extra-renal disease aspects should always be considered as part of routine management. In this review, we will focus on updates in pain/d ...[more]

PMID: 38010035

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Project description:Rationale & objectiveUsing OVERTURE (NCT01430494) study data on patient-perceived health, health care utilization, and productivity in autosomal dominant polycystic kidney disease (ADPKD), this research was conducted to characterize the burden of illness in patients with ADPKD and assess whether patient-reported outcome (PRO) assessment scores predict clinical and health-economic outcomes.Study designData were analyzed from a prospective, observational study.Setting & participantsThe study cohort comprised 3,409 individuals with ADPKD in 20 countries who were aged 12-78 years and were in chronic kidney disease (CKD) stages G1-G5 and Mayo risk subclasses 1A-1E.PredictorsScores on PRO instruments, including disease-specific assessments [ADPKD-Impact Scale (ADPKD-IS), and ADPKD-Urinary Impact Scale (ADPKD-UIS)] and generic measures were assessed.OutcomesClinical variables [eg, height-adjusted total kidney volume (htTKV), estimated glomerular filtration rate (eGFR), and abdominal girth] and health-economic outcomes were assessed.Analytical approachAssociations among variables were evaluated using Spearman correlations, logistic regression, and generalized linear mixed effects repeated measures models.ResultsBaseline CKD stage and Mayo risk classification showed little correlation with baseline PRO scores; however, scores on disease-specific instruments and measures of physical functioning were worse at more severe CKD stages. PRO scores predicted hospitalizations and sick days at 6-18 months, with strongest associations noted for the ADPKD-IS. PRO scores were not associated with htTKV and eGFR, but worse PRO scores were associated with greater abdominal girth. Poor baseline ADPKD-IS scores were positively associated with occurrence of ADPKD-related symptoms up to 18 months, including kidney pain (OR, 5.30; 95% CI, 2.75-10.24), hematuria (OR, 4.58; 95% CI, 1.99-10.53), and urinary tract infection (OR, 4.41; 95% CI, 1.93-10.11; P < 0.001 for all).LimitationsA limitation of the study was the maximum 18 months of follow-up available to assess outcomes.ConclusionsPRO scores predicted clinical and health-economic outcomes, such as hospitalization and absence from work, underscoring the importance of quality of life assessment of individuals with ADPKD.Plain-language summaryPatient-reported outcomes (PROs) are increasingly recognized as important parameters for assessing the clinical and humanistic burden of autosomal dominant polycystic kidney disease (ADPKD). We analyzed data from the observational OVERTURE study to better characterize disease impact on quality of life and determine whether patient-perceived burden might predict outcomes. Scores on PRO assessment instruments predicted hospitalizations and sick days at 6-18 months, with associations strongest for the disease-specific ADPKD-Impact Scale. Compared to patients who rated their health-related quality of life as good, those with poor baseline scores were significantly more likely to report ADPKD-related signs and symptoms up to 18 months of follow-up. These findings support using disease-specific PRO assessment instruments to assess and predict the impact of ADPKD.

Project description:Background and objectivesIt is assumed that in autosomal dominant polycystic kidney disease (ADPKD), kidney function remains in the normal range for several decades because of hyperfiltration of remnant nephrons. In this study, we investigate the extent to which patients with ADPKD hyperfilter.Design, setting, participants, & measurementsIn this cross-sectional study, we measured GFR as urinary clearance using continuous infusion of 125I-iothalamate. Kidney function reserve capacity was determined as increase in measured GFR after adding a dopamine infusion of 4.4-6 mg/h. Potential kidney donors were used as healthy controls and matched by age and sex to patients with ADPKD for comparisons across age groups and CKD stages. Hyperfiltration was defined by a loss of kidney function reserve capacity compared with healthy controls.ResultsA total of 300 participants were studied. In the youngest age group (18-29 years), measured GFR was not different between patients with ADPKD and healthy controls (103±21 versus 111±9 ml/min per 1.73 m2; P=0.14). In this age group kidney function reserve capacity was higher compared with healthy controls (11.1%±8.3% versus 5.3%±6.5%; P=0.04). Moreover, kidney function reserve capacity was similar to healthy controls in patients with ADPKD with early-stage disease (eGFR≥60 ml/min per 1.73 m2), either overall or when divided into fast or slow progressors according to their Mayo height-adjusted total kidney volume class. However, in patients with ADPKD, lower measured GFR was associated with lower kidney function reserve capacity (β=1.0 [95% confidence interval, 0.5 to 1.5] % per 10 ml/min per 1.73 m2; P<0.001). Kidney function reserve capacity was therefore lower compared with healthy controls at older age and later CKD stages.ConclusionsPatients with early-stage ADPKD, either classified as having rapidly or slowly progressive disease, are able to increase their GFR in response to dopamine. Hyperfiltration, defined by a loss of kidney function reserve capacity, may therefore not be an early phenomenon in ADPKD.

Dataset Information

Beyond Loss of Kidney Function: Patient Care in Autosomal Dominant Polycystic Kidney Disease.

Publications

Beyond Loss of Kidney Function: Patient Care in Autosomal Dominant Polycystic Kidney Disease.

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