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Title: Pathogenesis of the prune-belly syndrome: a functional urethral obstruction caused by prostatic hypoplasia
Authors: Moerman, Philippe ×
Fryns, Jean-Pierre
Goddeeris, P
Lauweryns, J M #
Issue Date: Apr-1984
Series Title: Pediatrics vol:73 issue:4 pages:470-5
Abstract: Abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism are the three major features of the prune-belly syndrome, also referred to as triad syndrome or Eagle-Barrett syndrome. The etiology is unclear and the pathogenesis a subject of continuing debate. Clinical and pathologic experience with seven cases of prune-belly syndrome is reviewed. Findings indicate that the urogenital anomalies can be attributed to a functional urethral obstruction which in turn is the result of prostatic hypoplasia. The histology of the abdominal wall is that of atrophy-ie, the degeneration of already formed muscle--and not of primitive muscle. This observation supports the theory that the abdominal muscle hypoplasia is a nonspecific lesion, resulting from fetal abdominal distension of various causes. Transient fetal ascites may be an important feature of the prune-belly syndrome.
ISSN: 0031-4005
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Clinical Genetics Section (-)
Faculty of Medicine - miscellaneous
Translational Cell & Tissue Research
Department of Human Genetics - miscellaneous
× corresponding author
# (joint) last author

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