Congenital intrahepatic portocaval shunt associated with trimethylaminuria. 1997

M S Fernández, and C Gutiérrez, and J J Vila, and A López, and V Ibáñez, and C Sangüesa, and J Lluna, and J E Barrios
Departamento de Cirugía Pediátrica, Hospital Infantil "La Fe" de Valencia, Av. Campanar N degrees 21, 46009 Valencia, Spain.

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.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008099 Liver A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances. Livers
D008661 Metabolism, Inborn Errors Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero. Inborn Errors of Metabolism,Metabolism Errors, Inborn,Error, Inborn Metabolism,Errors Metabolism, Inborn,Errors Metabolisms, Inborn,Errors, Inborn Metabolism,Inborn Errors Metabolism,Inborn Errors Metabolisms,Inborn Metabolism Error,Inborn Metabolism Errors,Metabolism Error, Inborn,Metabolism Inborn Error,Metabolism Inborn Errors,Metabolisms, Inborn Errors
D008744 Methylamines Derivatives of methylamine (the structural formula CH3NH2).
D010089 Oxidoreductases, N-Demethylating N-Demethylase,N-Demethylases,Oxidoreductases, N Demethylating,Demethylating Oxidoreductases, N,N Demethylase,N Demethylases,N Demethylating Oxidoreductases,N-Demethylating Oxidoreductases
D010166 Palliative Care Care alleviating symptoms without curing the underlying disease. (Stedman, 25th ed) Palliative Treatment,Palliative Supportive Care,Palliative Surgery,Palliative Therapy,Surgery, Palliative,Therapy, Palliative,Care, Palliative,Palliative Treatments,Supportive Care, Palliative,Treatment, Palliative,Treatments, Palliative
D011169 Portal Vein A short thick vein formed by union of the superior mesenteric vein and the splenic vein. Portal Veins,Vein, Portal,Veins, Portal
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females

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