Literature DB >> 6231520

Pathogenesis of the prune-belly syndrome: a functional urethral obstruction caused by prostatic hypoplasia.

P Moerman, J P Fryns, P Goddeeris, J M Lauweryns.   

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.

Entities:  

Mesh:

Year:  1984        PMID: 6231520

Source DB:  PubMed          Journal:  Pediatrics        ISSN: 0031-4005            Impact factor:   7.124


  6 in total

1.  A hypothesis for the higher incidence of vesico-ureteral reflux and primary megaureters in male babies.

Authors:  E F Avni; E Gallety; F Rypens; M Hall; S Dedeire; C C Schulman
Journal:  Pediatr Radiol       Date:  1992

2.  Rare copy number variants identified in prune belly syndrome.

Authors:  Nansi S Boghossian; Robert J Sicko; Andreas Giannakou; Aggeliki Dimopoulos; Michele Caggana; Michael Y Tsai; Edwina H Yeung; Nathan Pankratz; Benjamin R Cole; Paul A Romitti; Marilyn L Browne; Ruzong Fan; Aiyi Liu; Denise M Kay; James L Mills
Journal:  Eur J Med Genet       Date:  2017-11-23       Impact factor: 2.708

3.  Good outcome in prune-belly syndrome despite associated severe anomalies.

Authors:  I Zelikovic; S Dabbagh; A L Friedman; D T Uehling; R W Chesney
Journal:  Pediatr Nephrol       Date:  1988-10       Impact factor: 3.714

Review 4.  Investigation of the dilated urinary tract.

Authors:  H F Parkhouse; T M Barratt
Journal:  Pediatr Nephrol       Date:  1988-01       Impact factor: 3.714

5.  McKusick-Kaufman syndrome: the diagnostic challenge of abdominal distension in the neonatal period.

Authors:  C Schaap; C E de Die-Smulders; R H Kuijten; J P Fryns
Journal:  Eur J Pediatr       Date:  1992-08       Impact factor: 3.183

6.  The absence of the verumontanum at voiding cystourethrography as a sign of prostate maldevelopment.

Authors:  A L Valentini; P Ferrara; C Manzoni; A P Mancini; S Pulitanò; L Bonomo
Journal:  Adv Urol       Date:  2010-11-30
  6 in total

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