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Bladder exstrophy-epispadias complex

✍ Scribed by Michael Ludwig; Bonnie Ching; Heiko Reutter; Simeon A. Boyadjiev

Book ID
101708520
Publisher
John Wiley and Sons
Year
2009
Tongue
English
Weight
181 KB
Volume
85
Category
Article
ISSN
1542-0752

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✦ Synopsis

Abstract

The bladder exstrophy‐epispadias complex (BEEC) represents an anterior midline defect with variable expression comprising a spectrum of anomalies involving the abdominal wall, pelvis, urinary tract, genitalia, and occasionally the spine and anus. The vast majority of BEEC cases are classified as non‐syndromic and the etiology of this malformation is still unknown. This review presents the current state of knowledge on this multifactorial disorder, including historical retrospect, phenotypic and anatomical characterization, epidemiology, proposed developmental mechanisms, existing animal models, and implicated genetic and environmental components. These published lines of evidence argue strongly that BEEC occurs as a result of strong genetic predisposition that is yet to be deciphered. Birth Defects Research (Part A), 2009. © 2009 Wiley‐Liss, Inc.

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## Abstract ## BACKGROUND: The bladder exstrophy‐epispadias complex represents a spectrum of urogenital anomalies in which part or all of the distal urinary tract fail to close and are exposed on the outer abdominal wall. Previous studies are suggestive of an underlying multifactorial mode of inhe