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Mild hyperphenylalaninemia

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Disease definition

A rare form of phenylketonuria, an inborn error of amino acid metabolism, characterized by blood phenylalanine (Phe) concentrations of 120-600 micromol/L with or without clinical manifestations of impaired cognitive function, and behavioral and developmental disorders.

ORPHA:79651

Classification level: Subtype of disorder

Synonym(s):
  • Mild HPA
  • Non-PKU HPA
  • mHPA

Prevalence: Unknown

Inheritance: Autosomal recessive

Age of onset: Infancy, Neonatal

ICD-10: E70.1

ICD-11: 5C50.0Y

OMIM: 261600

UMLS: C5680207

A summary on this disease is available in Français, Español, Deutsch, Italiano, Português, Nederlands Polski, Ελληνικά
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
Svenska (2020) - Socialstyrelsen
Guidelines
Emergency guidelines
Français (2014.pdf) - Orphanet Urgences
Español (2019.pdf) - Orphanet Urgences
Clinical practice guidelines
English (2025) - Mol Genet Metab
English (2021) - Orphanet J Rare Dis Logo ERN
English (2017) - Orphanet J Rare Dis
English (2025) - Genet Med
Disease review articles
Review article
English (2025) - Orphanet J Rare Dis
Clinical genetics review
English (2025) - GeneReviews
Genetic testing
Guidance for genetic testing
English (2011) - Eur J Hum Genet
Patient-Centered Outcome Measures (PCOMs)
Access questionnaires assessing quality of life in this disease (English)
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.