Abstract
BACKGROUND AND OBJECTIVES
In autosomal dominant polycystic kidney disease, progressive renal enlargement secondary to expanding cysts is a hallmark.
The total cyst load and range of cyst diameters are unknown. The purpose of this study was to quantify the total number and
range of diameters of individual cysts in adults with preserved GFR.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS
A retrospective, morphometric analysis of renal cyst number and diameter using magnetic resonance images from eight adult
autosomal dominant polycystic kidney disease patients was performed at baseline and after 6.9 years. Cyst number and diameter
were measured in microscopic sections of nephrectomy specimens from five different adults.
RESULTS
The diameters of 1010 cysts ranged from 0.9 to 77.1 mm in baseline T2 magnetic resonance images, and the mean total number
of cysts increased from 682 to 1002 in 6.9 years. However, magnetic resonance imaging detects only cysts above the lower limit
of detection. In 405 cysts measured in nephrectomy specimens, 70% had diameters <0.9 mm. Cyst counts by magnetic resonance
in eight subjects compared with histology revealed approximately 62 times more cysts below the limit of magnetic resonance
imaging detection than above it.
CONCLUSIONS
This study presents quantitative data indicating that renal cysts develop in a minority of renal tubules. Increased numbers
detected by magnetic resonance imaging are caused primarily by cysts below detection at baseline enlarging to a detectable
diameter over time. The broad range of diameters, with a heavy concentration of microscopic cysts, may be most appropriately
explained by a formation process that operates continuously throughout life.
Links
Authors
Grantham JJ, Mulamalla S, Grantham CJ, Wallace DP, Cook LT, Wetzel LH, Fields TA, Bae KT
Institution
Department of Internal Medicine, Kidney Institute, University of Kansas Medical Center, Kansas City, KS 66160, USA. jgrantha@kumc.edu
Source
Clinical journal of the American Society of Nephrology : CJASN 7:7 2012 Jul pg 1087-93MeSH
AdolescentAdult
Female
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Polycystic Kidney, Autosomal Dominant
Retrospective Studies
Young Adult
Pub Type(s)
Journal ArticleResearch Support, N.I.H., Extramural
Language
eng
PubMed ID
22580785
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