[The megacystis-microcolon-intestinal hypoperistalsis syndrome: apropos of a case with prolonged survival].
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a severe congenital disease with autosomal recessive inheritance, characterized by vesical distension and intestinal hypoperistalsis what causes intestinal obstruction in newborn, with other abnormalities associated. It presents a low incidence, about a hundred cases are reported in the bibliography. Life expectancy doesn't reach a year because of the sepsis failure generally. In our study the survival is higher than the majority of the cases reported, with good cuality of life and acceptable ponderal development. Home parenteral nutrition with the following and multidisciplinary collaboration in a strict way, establish the success' key in this pathology.
Magaña Pintiado
M I
MI
Servicio de Farmacia, Hospital Universitario Central de Asturias, Oviedo. mmganyapintiado@yahoo.es
Al-Kassam Martínez
M
M
Bousoño García
C
C
Ramos Polo
E
E
Gómez Alvarez
M E
ME
D000015:Abnormalities, Multiple / Q000628:therapy; D000367:Age Factors; D002675:Child, Preschool; D003106:Colon / Q000002:abnormalities*; D006801:Humans; D008297:Male; D016331:Parenteral Nutrition, Home; D010528:Peristalsis; D011788:Quality of Life; D013534:Survival; D013577:Syndrome; D016896:Treatment Outcome; D001743:Urinary Bladder / Q000002:abnormalities*