Maternal phenylketonuria

Orpha code: 2209OMIM code: 261600

Definition

A rare disorder of phenylalanine (Phe) metabolism, an inborn error of amino acid metabolism, characterized by the development of microcephaly, growth retardation, congenital heart disease, facial dysmorphism and intellectual disability in non-phenylketonuric offspring of mothers with excess blood Phe concentrations.

Disease data
Classification

Malformation syndrome

Synonyms
Hyperphenylalaninemic embryopathy
Embriopatia fenyloketonowa
Embriopatia hiperfenyloalaninowa
Hiperfenyloalaninemia matczyna
PKU matczyna
Maternal PKU
Maternal hyperphenylalaninemia
Phenylketonuric embryopathy
ORPHA code
2209
OMIM code
261600
ICD10 code
E70.1
ICD11 code
5C50.02

No additional description.

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