Glomerulocystic kidney disease (GCKD) and tubulointerstitial fibrosis in a patient with recurrent acute kidney injure
Journal Title: Postępy Nauk Medycznych - Year 2011, Vol 24, Issue 4
Abstract
We present a case of glomerular cystic kidney disease, most probably sporadic, revealed on renal biopsy performed to diagnose the deteriorating renal function in a 23 years old female. Surprisingly, the GCKD was accompanied by mild tubolointerstitial fibrosis, which is unusual and has been reported sporadically. Any farmacological intervention inevitably led to acute kidney injury (AKIN I-II). We hypothesize, that this could have happened also prior to diagnosis and the previous recurrent AKI episodes resulted in interstitial fibrosis. The inappropriately increased plasma uric acid concentrations could indicate the disturbance in uromodulin synthesis/secretion, which is typical to other ciliopathies. The unusual findings on renal biopsy and clinical course of GCKD in patient suggests direct link between ciliopathies and susceptibility to acute kidney injury, which can result in interstitial fibrosis and possibly, accelerated kidney failure progression.
Authors and Affiliations
Ewa Wolska, Ryszard Gellert, Danuta Kobus, Dorota Daniewska, Andrzej Mróz, Krzysztof Bardadin, Agnieszka Perkowska-Ptasińska
Keywords
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