| Prune belly syndrome associated with cloacal anomaly, patent urachal remnant, and omphalocele in a female infant. | |
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MedLine Citation:
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PMID: 21034928 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Prune belly syndrome (PBS), megacystis-microcolon-intestinal hypoperistalsis (MMIH), and omphalocele-exstrophy of the bladder-imperforate anus-spine abnormalities complex (OEIS) are rare congenital malformations of the newborn that lead to incomplete formation of the gastrointestinal and genitourinary tract systems. To date, incomplete mesodermal development is identified as the cause for all these complex genetic syndromes even if the etiology is still unknown. We present an original case sharing characteristics common to PBS, MMIH, and OEIS complex, without a clear inclination toward any particular one. This case hints toward a common pathway in the creation of the 3 syndromes. We hypothesize that they are a spectrum of malformations based on the time frame when the mesoderm fails to create a normal interaction between infraumbilical mesoderm, urorectal septum, lumbosacral somites in the formation of the abdominal wall and the genitourinary and lower gastrointestinal tracts. |
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Authors:
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Stefano Giuliani; Christopher Vendryes; Ajay Malhotra; Donald B Shaul; Dean M Anselmo |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of pediatric surgery Volume: 45 ISSN: 1531-5037 ISO Abbreviation: J. Pediatr. Surg. Publication Date: 2010 Nov |
Date Detail:
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Created Date: 2010-11-01 Completed Date: 2010-11-30 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0052631 Medline TA: J Pediatr Surg Country: United States |
Other Details:
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Languages: eng Pagination: e39-42 Citation Subset: IM |
Copyright Information:
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Published by Elsevier Inc. |
Affiliation:
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Department of Pediatric Surgery, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA 90027, USA. stegiuliani@hotmail.com |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple
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diagnosis*,
surgery Cloaca / abnormalities* Diagnosis, Differential Digestive System Surgical Procedures / methods Female Follow-Up Studies Gynecologic Surgical Procedures / methods Hernia, Umbilical / diagnosis*, surgery Humans Infant, Newborn Magnetic Resonance Imaging Prune Belly Syndrome / diagnosis*, surgery Reconstructive Surgical Procedures / methods Urachus / abnormalities* Urography |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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