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        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">El s&#237;ndrome de megavejiga-microcolon-hipoperistaltismo intestinal es una rara alteraci&#243;n gen&#233;tica caracterizada por distensi&#243;n vesical e hipoperistaltismo intestinal que afecta a todo el tracto gastrointestinal&#46; Presentamos un nuevo caso que presenta los hallazgos cl&#237;nicos y radiol&#243;gicos t&#237;picos de este s&#237;ndrome&#46; El diagn&#243;stico debe ser sospechado en un reci&#233;n nacido que presenta cl&#237;nica de obstrucci&#243;n intestinal y retenci&#243;n urinaria&#59; siendo confirmado mediante estudios de imagen&#44; fundamentalmente radiograf&#237;a simple de abdomen y ecograf&#237;a urinaria&#46; Los hallazgos histol&#243;gicos no son espec&#237;ficos&#59; encontramos un adelgazamiento de la capa muscular intestinal y proliferaci&#243;n de tejido conectivo&#46; El pron&#243;stico es generalmente grave&#46; Nuestro paciente falleci&#243; por sepsis a los 25 d&#237;as de vida&#46;</p>"
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Vol. 53. Núm. 3.
Páginas 253-256 (septiembre 2000)
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Vol. 53. Núm. 3.
Páginas 253-256 (septiembre 2000)
Acceso a texto completo
Síndrome de megavejiga-microcolon-hipoperistaltismo intestinal
Megacystis-microcolon-intestinal hypoperistalsis syndrome
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S. Rite Graciaa, B. Fernández Álvarez de Sotomayora, V. Rebage Moisésa, A. Marco Telloa, J.A. Esteban Ibarzb, M. Romeo Uleciab, M.C. Yus Gotorc, S. Rite Montañésa,
Autor para correspondencia
segundorite@jazzfree.com

Manuel Lasala, 44, 4.o C. 50006 Zaragoza
a Unidad de Neonatología. Servicio de Pediatría. Hospital Infantil Universitario Miguel Servet. Zaragoza
b Servicio de Cirugía Pediátrica. Hospital Infantil Universitario Miguel Servet. Zaragoza
c Servicio de Anatomía Patológica. Hospital Infantil Universitario Miguel Servet. Zaragoza
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Estadísticas

El síndrome de megavejiga-microcolon-hipoperistaltismo intestinal es una rara alteración genética caracterizada por distensión vesical e hipoperistaltismo intestinal que afecta a todo el tracto gastrointestinal. Presentamos un nuevo caso que presenta los hallazgos clínicos y radiológicos típicos de este síndrome. El diagnóstico debe ser sospechado en un recién nacido que presenta clínica de obstrucción intestinal y retención urinaria; siendo confirmado mediante estudios de imagen, fundamentalmente radiografía simple de abdomen y ecografía urinaria. Los hallazgos histológicos no son específicos; encontramos un adelgazamiento de la capa muscular intestinal y proliferación de tejido conectivo. El pronóstico es generalmente grave. Nuestro paciente falleció por sepsis a los 25 días de vida.

Palabras clave:
Síndrome de megavejiga-microcolon-hipoperistaltismointestinal
Recién nacido
Obstrucción intestinal

Megacystis-microcolon-intestinal hypoperistalsis is a rare congenital disorder characterized by urinary bladder distention and hypoperistalsis throughout the entire gastrointestinal tract. We present a new case with the typical clinical and radiological findings of the syndrome. This diagnosis should be suspected in patients who present with intestinal obstruction and urine retention and should be confirmed with imaging techniques, including abdominal plain films and urinary ultrasonography. Histological findings are non-specific; we found thinning of the intestinal muscle layer and connective tissue proliferation. The prognosis is generally very poor. Our patient died from sepsis at the age of 25 days.

Key words:
Megacystis-microcolon-intestinal hypoperistalsis syndrome
Newborn
Intestinal obstruction
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Bibliografía
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Copyright © 2000. Asociación Española de Pediatría
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