Clinical and ultrasonographical characterization of childhood cystic kidney diseases in Egypt.

Abstract:

BACKGROUND:Renal cystic disorders (RCD) constitute an important and leading cause of end-stage renal disease (ESRD) in children. It can be acquired or inherited; isolated or associated with extrarenal manifestations. The precise diagnosis represents a difficult clinical challenge. METHODS:The aim of this study was to define the pattern of clinical phenotypes of children with renal cystic diseases in Pediatric Nephrology Center, Cairo University. We have studied the clinical phenotypes of 105 children with RCD [45 (43%) of them had extrarenal manifestations]. RESULTS:The most common disorders were the presumably inherited renal cystic diseases (65.7%) mainly nephronophthisis and related ciliopathies (36.2%), as well as polycystic kidney diseases (29.5%). Moreover, multicystic dysplastic kidneys accounted for 18% of study cases. Interestingly, eight syndromic cases are described, yet unclassified as none had been previously reported in the literature. CONCLUSION:RCD in this study had an expanded and complex spectrum and were largely due to presumably inherited/genetic disorders (65.7%). Moreover, we propose a modified algorithm for clinical and diagnostic approach to patients with RCD.

journal_name

Ren Fail

journal_title

Renal failure

authors

Soliman NA,Nabhan MM,Bazaraa HM,Badr AM,Shaheen M

doi

10.3109/0886022X.2014.883996

subject

Has Abstract

pub_date

2014-06-01 00:00:00

pages

694-700

issue

5

eissn

0886-022X

issn

1525-6049

journal_volume

36

pub_type

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