05Endocrine, nutritional or metabolic diseases Metabolic disorders Inborn errors of metabolism 5C50Inborn errors of amino acid or other organic acid metabolism 5C50.0Phenylketonuria
5C50.00
category
Classical phenylketonuria
Definition Classical phenylketonuria is a severe form of phenylketonuria (PKU) an inborn error of amino acid metabolism characterised in untreated patients by severe intellectual deficit and neuropsychiatric complications.
Also indexed as
Classical phenylketonuria Classical phenylketonuria, phenylalanine hydroxylase partial deficiency Classical phenylketonuria, phenylalanine hydroxylase total deficiency Folling disease PAH - [phenylalanine hydroxylase] deficiency Typical PKU - [phenylketonuria] Typical phenylketonuria hyperphenylalaninaemia Type I imbecilitus phenylpyruvica oligophrenia phenylpyruvica phenylpyruvic aciduria phenylpyruvic oligophrenia
Nearby in Phenylketonuria
International Classification of Diseases, Eleventh Revision (ICD-11), World Health Organization (WHO) 2019/2021, https://icd.who.int/browse11. Licensed under Creative Commons Attribution-NoDerivatives 3.0 IGO (CC BY-ND 3.0 IGO).
Release 2026-01.
View this entity in the official WHO ICD-11 browser .
This page reproduces WHO classification content for reference. It is not
medical advice and must not be used to diagnose or treat. For coding
decisions, consult the official release and your national coding guidelines.
About · Data & methodology ·
Privacy · Contact