Abstract

We describe two patients that had a history of recurrent renal stones and chronic renal insufficiency. The first case was a 51-year-old man with an adenine phophoribosyltransferase (APRT) deficiency who was diagnosed only after he had been referred for severe renal failure requiring hemodialysis. This led to a screening of the entire family, which identified six carriers and an additional affected relative (a 41-year-old man and the second case reported herein). Genetic analysis of the APRT gene revealed an atypical mutation previously described only once in a compound heterozygote.

Details

Title
Is adenine phophorybosiltransferase deficiency a still underdiagnosed cause of urolithiasis and chronic renal failure? A report of two cases in a family with an uncommon novel mutation
Author
Italia Perruzza 1 ; Valentina Di Pietro 2 ; Tavazzi, Barbara 2 ; Lazzarino, Giuseppe 3 ; Gamberini, Marco 1 ; Barsotti, Paola 4 ; Amorini, Angela Maria 3 ; Giardina, Bruno 2 ; Balducci, Alessandro 1 

 Department of Nephrology, Azienda Ospedaliera S. Giovanni-Addolorata, Rome 
 Institute of Biochemistry and Clinical Biochemistry, Catholic University of Rome, Rome 
 Department of Chemical Sciences, Laboratory of Biochemistry, University of Catania, Catania 
 Department of Experimental Medicine and Pathology, University of Rome “La Sapienza”, Rome, Italy 
Pages
292-295
Publication year
2008
Publication date
Oct 2008
Publisher
Oxford University Press
ISSN
17530784
e-ISSN
17530792
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
3170681729
Copyright
© The Author [2008]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: [email protected]. This work is published under http://creativecommons.org/licenses/by-nc/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.