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[Observations on the Prune-Belly syndrome].

P Welling, R A Pfeiffer, W Kosenow

    Zeitschrift Fur Kinderheilkunde
    |January 1, 1975
    PubMed
    Summary

    Prune-Belly syndrome, a condition affecting males with urogenital and abdominal muscle defects, was studied in five children. Two presented with hearing loss, suggesting a potential link or co-occurrence.

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    American journal of medical genetics·2001

    Area of Science:

    • Pediatric Medicine
    • Medical Genetics
    • Developmental Biology

    Context:

    • The Prune-Belly syndrome is characterized by congenital malformations of the urogenital tract and aplasia of abdominal muscles.
    • While predominantly affecting males, the syndrome has been documented in females.
    • The exact cause and pathogenesis remain unclear.

    Purpose:

    • To describe clinical features of Prune-Belly syndrome in five male children.
    • To investigate potential associated conditions, such as hearing impairment.
    • To identify potential novel hereditary syndromes.

    Summary:

    • Five male children with typical Prune-Belly syndrome features were analyzed.
    • Two of these children also exhibited deafness.
    • A separate familial occurrence of abdominal muscle defects and joint contractures, without renal anomalies, suggests a distinct hereditary syndrome.

    Impact:

    • Highlights the variability and potential co-occurring conditions in Prune-Belly syndrome.
    • Suggests the possibility of a new hereditary syndrome presenting with abdominal muscle defects and contractures.
    • Underscores the need for further research into the etiology and pathogenesis of these conditions.

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