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Determinants of Renal Volume in Autosomal-dominant Polycystic Kidney Disease

Overview
Journal Kidney Int
Publisher Elsevier
Specialty Nephrology
Date 2007 Oct 26
PMID 17960141
Citations 53
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Abstract

The Consortium of Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) recently showed that renal enlargement in autosomal-dominant polycystic kidney disease mimicked exponential growth. We determined the effects of cyst initiation rate, total number, and growth rate on the time-dependent change of total cyst volume (TCV). Mathematical models with equations integrating cyst surface area, volume, and an invariant growth rate constant were used to compute the time-dependent change in volume of solitary and multiple cysts. Multiple expanding cysts increased TCV in an exponential-like pattern even when individual cysts formed at different rates or exhibited different but constant growth rates. TCV depended on the rate of cyst initiation and on the total number of cysts; however, the compounding effect of exponential-like growth was the most powerful determinant of long-term cyst expansion. Extrapolation of TCV data plots for individual subjects back to an age of 18 predicted TCV values within an established range. We conclude that cysts started early in life were the main contributor to eventual TCV while their growth rate primarily determined renal size; although the rate of formation and the ultimate number of cysts also contributed. The good fit between the exponential models and the extrapolated CRISP data indicates that the TCV growth rate is a defining trait for individual patients and may be used as a prognostic marker.

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References
1.
Bae K, Commean P, Lee J . Volumetric measurement of renal cysts and parenchyma using MRI: phantoms and patients with polycystic kidney disease. J Comput Assist Tomogr. 2000; 24(4):614-9. DOI: 10.1097/00004728-200007000-00019. View

2.
Magistroni R, He N, Wang K, Andrew R, Johnson A, Gabow P . Genotype-renal function correlation in type 2 autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2003; 14(5):1164-74. DOI: 10.1097/01.asn.0000061774.90975.25. View

3.
Grantham J . Lillian Jean Kaplan International Prize for advancement in the understanding of polycystic kidney disease. Understanding polycystic kidney disease: a systems biology approach. Kidney Int. 2003; 64(4):1157-62. DOI: 10.1046/j.1523-1755.2003.00242.x. View

4.
Brasier J, Henske E . Loss of the polycystic kidney disease (PKD1) region of chromosome 16p13 in renal cyst cells supports a loss-of-function model for cyst pathogenesis. J Clin Invest. 1997; 99(2):194-9. PMC: 507786. DOI: 10.1172/JCI119147. View

5.
Macdermot K, Saggar-Malik A, Economides D, Jeffery S . Prenatal diagnosis of autosomal dominant polycystic kidney disease (PKD1) presenting in utero and prognosis for very early onset disease. J Med Genet. 1998; 35(1):13-6. PMC: 1051180. DOI: 10.1136/jmg.35.1.13. View