title: Bladder exstrophy-epispadias complex.
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.
authors.affiliation: Department of Clinical Chemistry and Pharmacology, University of Bonn, Sigmund-Freud-Str. 25, Bonn D-53105, Germany. mludwig@uni-bonn.de
authors.forename: Michael
authors.initials: M
authors.lastname: Ludwig
authors.forename: Bonnie
authors.initials: B
authors.lastname: Ching
authors.forename: Heiko
authors.initials: H
authors.lastname: Reutter
authors.forename: Simeon A
authors.initials: SA
authors.lastname: Boyadjiev
mesh_terms: D000818:Animals; D001746:Bladder Exstrophy / Q000145:classification / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D004195:Disease Models, Animal; D004842:Epispadias / Q000145:classification / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male