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Published on: September 1, 2015
Early renal abnormalities in autosomal dominant polycystic kidney disease
Esther Meijer1, Mieneke Rook, Hilde Tent
1Division of Nephrology, Department of Medicine, University Medical Center Groningen, Groningen, The Netherlands.
Insights
Autosomal dominant polycystic kidney disease (ADPKD) causes early renal abnormalities, including decreased effective renal plasma flow (ERPF) and increased filtration fraction (FF) and urinary albumin excretion (UAE), even with normal GFR. These markers may better indicate disease severity in young adults.
Area of Science:
- Nephrology
- Genetics
- Internal Medicine
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by the development of kidney cysts.
- Therapeutic interventions for ADPKD are emerging, necessitating a better understanding of disease progression and optimal treatment timing.
- Monitoring renal abnormalities is crucial for managing ADPKD.
Purpose of the Study:
- To evaluate the prevalence and characteristics of renal abnormalities in ADPKD patients across different age groups.
- To identify potential early biomarkers for ADPKD progression.
Main Methods:
- A cross-sectional study included 103 prevalent ADPKD patients.
- Key parameters measured included mean arterial pressure, total renal volume (TRV), glomerular filtration rate (GFR), effective renal plasma flow (ERPF), renal vascular resistance (RVR), and filtration fraction (FF).
- Twenty-four-hour urine collections assessed urinary albumin excretion (UAE) and osmolarity. Patients were compared to age- and gender-matched healthy controls.
Main Results:
- Young adult ADPKD patients (first age quartile) exhibited near-normal GFR but significantly decreased ERPF and increased FF compared to controls.
- Elevated 24-hour urinary volumes, lower urinary osmolarity, and increased UAE were observed in young ADPKD patients.
- Despite these functional changes, total renal volume (TRV) was only modestly enlarged in this group.
Conclusions:
- ADPKD patients demonstrate significant renal abnormalities at a young adult age, preceding substantial GFR decline or TRV enlargement.
- Decreased ERPF, increased FF, and elevated UAE appear to be sensitive early indicators of ADPKD severity.
- These findings suggest that ERPF, FF, and UAE may serve as more effective biomarkers for monitoring ADPKD progression than GFR alone.
Background And Objectives:
Potential therapeutic interventions are being developed for autosomal dominant polycystic kidney disease (ADPKD). A pivotal question will be when to initiate such treatment, and monitoring disease progression will thus become more important. Therefore, the prevalence of renal abnormalities in ADPKD at different ages was evaluated.
Design, Setting, Participants, & Measurements:
Included were 103 prevalent ADPKD patients (Ravine criteria). Measured were mean arterial pressure (MAP), total renal volume (TRV), GFR, effective renal plasma flow (ERPF), renal vascular resistance (RVR), and filtration fraction (FF). Twenty-four-hour urine was collected. ADPKD patients were compared with age- and gender-matched healthy controls.
Results:
Patients and controls were subdivided into quartiles of age (median ages 28, 37, 42, and 52 years). Patients in the first quartile of age had almost the same GFR when compared with controls, but already a markedly decreased ERPF and an increased FF (GFR 117 +/- 32 versus 129 +/- 17 ml/min, ERPF 374 +/- 119 versus 527 +/- 83 ml/min, FF 32% +/- 4% versus 25% +/- 2%, and RVR 12 (10 to 16) versus 8 (7 to 8) dynes/cm(2), respectively). Young adult ADPKD patients also had higher 24-hour urinary volumes, lower 24-hour urinary osmolarity, and higher urinary albumin excretion (UAE) than healthy controls, although TRV in these young adult patients was modestly enlarged (median 1.0 L).
Conclusions:
Already at young adult age, ADPKD patients have marked renal abnormalities, including a decreased ERPF and increased FF and UAE, despite modestly enlarged TRV and near-normal GFR. ERPF, FF, and UAE may thus be better markers for disease severity than GFR.
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