Abstract
BackgroundAutosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disorder that leads to end stage renal disease (ESRD). Cyst expansion in ADPKD is strongly associated with the decline in renal function. However, the correlation between total kidney volume (TKV) and glomerular filtration rate (GFR) at an early stage has not been well demonstrated. There is growing evidence that utilization of estimated GFR (eGFR) may induce misleading information in a population with near normal renal function. Therefore, a more accurate method is essential.MethodsA prospective cohort of ADPKD patients was conducted with clinical data and laboratory collection. Measured GFR (mGFR) was assessed by iohexol plasma clearance method using ultra performance liquid chromatography. eGFR was calculated using the CKD-EPI equation. Kidney volumes were evaluated using MRI imaging protocol.ResultsThirty two patients completed the study. The mean age was 56 years old. The mean initial mGFR was 83.8 mL/min/1.73m2. The mean change in mGFR per year was –2.99 mL/min/1.73m2/year. The mean initial height-adjusted TKV (htTKV) was 681.0 mL/m. The mean percentage change in htTKV per year (%ΔhtTKV/y) was 4.77 %/year. mGFR had a better association with clinical parameters than eGFR. Initial mGFR was significantly and inversely correlated with initial htTKV and age. The percentage change in mGFR per year was significantly and inversely correlated with the %ΔhtTKV/y and 24-hr urine albumin. The %ΔhtTKV/y was significantly correlated with initial htTKV.ConclusionsOur studies demonstrated that mGFR using iohexol is a more reliable and accurate method than eGFR for evaluating GFR changes in the early stages of ADPKD patients. There is a strong inverse correlation between kidney volume and mGFR in an Asian ADPKD population. The initial htTKV is a good predictor of kidney volume progression. The %ΔhtTKV/y is a good early surrogate marker for the decline in renal function. 24-hr urine albumin is also a good indicator for renal progression.
Highlights
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disorder that leads to end stage renal disease (ESRD)
Other clinical data were prospectively collected including: age of enrollment, weigh, height, body mass index (BMI), mean systolic blood pressure (SBP), mean diastolic blood pressure (DBP), serum creatinine, serum uric, fasting blood sugar, and LDL-cholesterol. 24-h urine protein and 24-h urine albumin were collected after being enrolled in the study for 4 weeks
Evaluation of renal function is a crucial part in assessing kidney disease, especially in ADPKD patients whose kidney functions remain stable for years, followed by a sharp decline in glomerular filtration rate (GFR) once a critical renal size is reached [6, 7]
Summary
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disorder that leads to end stage renal disease (ESRD). The correlation between total kidney volume (TKV) and glomerular filtration rate (GFR) at an early stage has not been well demonstrated. Clinical presentation varies widely, including hypertension, hematuria, proteinuria and renal insufficiency [3]. It is characterized by gradual renal enlargement and cyst growth prior to loss of renal function [4]. The kidney function remains stable in ADPKD patients for years, followed by a sharp decline in glomerular filtration rate (GFR) once a critical renal size is reached [6, 7]. Clinical parameters associated with decline in kidney function in ADPKD patients have been gradually reported [8,9,10]. The lack of a sensitive measure of disease progression in the early stages of ADPKD has caused problems in the development of prevention and of therapeutic agents [11,12,13]
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