Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS): Report of a case with prolonged survival and literature review
| Autor: | Amilcar Polanco-Ortiz, Leovigildo Mateos-Sánchez, Eunice López-Muñoz, Judith Villa-Morales, Adelina Hernández-Zarco |
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| Rok vydání: | 2013 |
| Předmět: |
Adult
Male Pathology medicine.medical_specialty Colon Urology Longevity Urinary Bladder Pregnancy Colon surgery medicine Humans Abnormalities Multiple business.industry Intestinal Pseudo-Obstruction Megacystis Microcolon Prognosis medicine.disease Pedigree Parenteral nutrition Child Preschool Pediatrics Perinatology and Child Health Female Berdon syndrome Urinary tract obstruction business Hypoperistalsis |
| Zdroj: | Journal of Pediatric Urology. 9:e12-e18 |
| ISSN: | 1477-5131 |
| DOI: | 10.1016/j.jpurol.2012.05.017 |
| Popis: | Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) was first described in 1976. A rare congenital autosomal recessive alteration that predominantly affects females (4:1 ratio), it is characterized by the presence of distended bladder (without distal urinary tract obstruction), microcolon, and decreased or absent intestinal peristalsis. Inconsistent and non-specific histological changes affecting the bladder and intestinal smooth muscle, and intrinsic innervations, have been reported most frequently. MMIHS usually has a fatal prognosis in the first year of life; nevertheless there are some case reports of longer survival. Here is presented the case report of a boy with a diagnosis of MMIHS who has achieved prolonged survival, followed by a review of the literature. |
| Databáze: | OpenAIRE |
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