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NDT Advance Access published online on September 17, 2009
Nephrology Dialysis Transplantation, doi:10.1093/ndt/gfp499
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© The Author 2009. Published by Oxford University Press [on behalf of ERA-EDTA]. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org


Might there be an association between polycystic kidney desease and noncompaction of the ventricular myocardium?

Riccardo Lubrano1, Paolo Versacci1, Giuliana Guido1, Elena Belleli1, Giammarco Andreoli1 and Marco Elli2

1 Dipartimento di Pediatria, ‘Sapienza’ Università di Roma, Roma 2 Dipartimento di Scienze Cliniche ‘Osp. Luigi Sacco’, Università degli Studi di Milano, Milano, Italy

Correspondence and offprint requests to: Riccardo Lubrano; E-mail: riccardo.lubrano{at}uniroma1.it



  Abstract

We report on a paediatric case of autosomal dominant polycystic kidney disease, where myocardial hypertrophy proved a consequence of noncompaction of the ventricular myocardium. Deletion of PKD1 and PKD2, the genes responsible for polycystic renal disease, has been linked also to disorganized myocardial arrangement in experimental animals. Two adults with polycystic kidney disease and myocardial hypertrophy in whom a careful diagnostic workup led to a diagnosis of non-compaction of the ventricular myocardium have been reported in the literature. Nephrologists must be aware of the possible association between the two diseases because early recognition of the disease may help in preventing the onset of complications.

Keywords: autosomal dominant polycystic kidney disease; child; noncompaction of the ventricular myocardium

Received for publication: 1. 6.09
Accepted in revised form: 27. 8.09


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