Congenital intrahepatic portocaval shunt associated with trimethylaminuria.
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.
Fernández
M S
MS
Departamento de Cirugía Pediátrica, Hospital Infantil "La Fe" de Valencia, Av. Campanar N degrees 21, 46009 Valencia, Spain.
Gutiérrez
C
C
Vila
J J
JJ
López
A
A
Ibáñez
V
V
Sangüesa
C
C
Lluna
J
J
Barrios
J E
JE
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
D008744:Methylamines; D010089:Oxidoreductases, N-Demethylating; C023186:trimethylamine dehydrogenase; C023336:trimethylamine