title: Congenital intrahepatic portocaval shunt associated with trimethylaminuria.
abstract: A case of congenital portal-systemic shunting due to an intrahepatic connection diagnosed by ultrasound scanning and color Doppler in an 8-month-old girl is reported. She began to manifest trimethylaminuria 3 years later. At 7 years of age, she is asymptomatic without therapeutic measures except for diet. This is the seventh reported case and the third in a child to our knowledge.
authors.affiliation: Departamento de Cirugía Pediátrica, Hospital Infantil "La Fe" de Valencia, Av. Campanar N degrees 21, 46009 Valencia, Spain.
authors.forename: M S
authors.initials: MS
authors.lastname: Fernández
authors.forename: C
authors.initials: C
authors.lastname: Gutiérrez
authors.forename: J J
authors.initials: JJ
authors.lastname: Vila
authors.forename: A
authors.initials: A
authors.lastname: López
authors.forename: V
authors.initials: V
authors.lastname: Ibáñez
authors.forename: C
authors.initials: C
authors.lastname: Sangüesa
authors.forename: J
authors.initials: J
authors.lastname: Lluna
authors.forename: J E
authors.initials: JE
authors.lastname: Barrios
mesh_terms: D001165:Arteriovenous Malformations / Q000000981:diagnostic imaging / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008099:Liver / Q000098:blood supply*; D008661:Metabolism, Inborn Errors / Q000150:complications; D008744:Methylamines / Q000652:urine*; D010089:Oxidoreductases, N-Demethylating / Q000172:deficiency; D010166:Palliative Care; D011169:Portal Vein / Q000002:abnormalities* / Q000000981:diagnostic imaging; D018615:Ultrasonography, Doppler, Color; D014682:Vena Cava, Inferior / Q000002:abnormalities* / Q000000981:diagnostic imaging
chemical_list: D008744:Methylamines; D010089:Oxidoreductases, N-Demethylating; C023186:trimethylamine dehydrogenase; C023336:trimethylamine