Prune Belly Syndrome: Report of Twelve Autopsy Cases and Possible Pathogenesis
Autor: | Takayoshi Ikeda, Kenji Kawamoto, Yukio Satow, Hiroshi Maeda, Juing-Yi Lee, Takeshi Matsuo, Okamoto N, Naotaka Akimoto |
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Rok vydání: | 1985 |
Předmět: |
Embryology
Pathology medicine.medical_specialty Autopsy General Medicine Anatomy Biology medicine.disease Urethral atresia Abdominal wall Urethra medicine.anatomical_structure Prune belly syndrome Pediatrics Perinatology and Child Health medicine Stage (cooking) Genital tubercle Imperforate anus Developmental Biology |
Zdroj: | Congenital Anomalies. 25:1-15 |
ISSN: | 1741-4520 0914-3505 |
DOI: | 10.1111/j.1741-4520.1985.tb00629.x |
Popis: | Twelve autopsy cases (9 males, 3 females) of the prune belly syndrome are presented. Principal anomalies of this syndrome are a prune-like abdominal feature and a giant bladder. Urethral atresia was observed in most but two cases. Imperforate anus and rectovesical fistula were observed in 8 and 7 cases respectively. Associated anomalies were those which may or may not be embryologically related to the principal anomalies. As regards the pathogenesis, the authors propose that a primary defect may occur during any developmental stage of the somitic mesoderm, genital tubercle and urethra. In conclusion, the prune belly syndrome may be of spectrum anomalies depending on the stage specificity mainly in the abdominal wall and genito-urinary organs. |
Databáze: | OpenAIRE |
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