Glomerulocystic Disease



Glomerulocystic Disease


Aleksandr Vasilyev, MD, PhD

Anthony Chang, MD










Bisected kidney with GCKD from 20-week-old fetus is shown. There are numerous small cysts throughout the cortical renal parenchyma.






Glomerulocystic kidney has markedly dilated Bowman capsules containing glomerular tufts separated by small tubules. No connection to the proximal tubules is evident. Little or no inflammation is present.


TERMINOLOGY


Abbreviations



  • Glomerulocystic kidney disease (GCKD)


  • Glomerulocystic kidney (GCK) refers to pattern of injury irrespective of cause


Synonyms



  • Autosomal dominant glomerulocystic kidney disease (ADGCKD)


Definitions



  • Cystic dilatation of Bowman space to 2-3x normal, > 5% of glomeruli in absence of other kidney diseases


  • Glomerulocystic kidneys are divided into 5 clinicopathologic categories



    • 1: Familial


    • 2: Associated with other hereditary diseases


    • 3: Syndromic nonhereditary


    • 4: Sporadic


    • 5: Acquired


  • GCKD reserved for diseases caused by mutations in uromodulin or HNF1β genes

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Glomerulocystic Disease
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