E70.1
BillableOther hyperphenylalaninemias
Other hyperphenylalaninemias
Coding Notes
Excludes 1
Codes that cannot be used together with this code (mutual exclusion)
Related Codes(7)
ICD-11 Equivalents(1)
ICD-11 Equivalents
View full mappingCorresponding ICD-11 codes from the WHO crosswalk mapping
Also Known As / Clinical Terms(139)
SNOMED CT
- Essential hyperphenylalaninaemia440009
- Essential hyperphenylalaninemia440009
- Hyperphenylalaninaemia, type II440009
- Hyperphenylalaninemia, type II440009
- Persistent hyperphenylalaninaemia440009
- Persistent hyperphenylalaninemia440009
- Classical phenylketonuria7573000
- Hyperphenylalaninaemia, type I7573000
- Hyperphenylalaninemia, type I7573000
- Imbecilitus phenylpyruvica7573000
- Severe phenylalanine hydroxylase deficiency7573000
- GTP - Guanosine-5-triphosphate cyclohydrolase deficiency23447005
- GTP cyclohydrolase I deficiency23447005
- Guanosine triphosphate (GTP) cyclohydrolase I deficiency23447005
- Guanosine triphosphate cyclohydrolase I deficiency23447005
- Guanosine-5-triphosphate cyclohydrolase deficiency23447005
- Hyperphenylalaninaemia with neopterin deficiency23447005
- Hyperphenylalaninemia with neopterin deficiency23447005
- Hyperphenylalaninaemia, type III28575006
- Hyperphenylalaninemia, type III28575006
- Neonatal hyperphenylalaninaemia28575006
- Neonatal hyperphenylalaninemia28575006
- Transient hyperphenylalaninaemia28575006
- Transient hyperphenylalaninemia28575006
- Transient mild hyperphenylalaninaemia28575006
- Transient mild hyperphenylalaninemia28575006
- Hyperphenylalaninaemia, type VI40751003
- Hyperphenylalaninemia, type VI40751003
- Persistent hyperphenylalaninaemia AND tyrosinaemia40751003
- Persistent hyperphenylalaninemia AND tyrosinemia40751003
- 7,8-Dihydrobiopterin synthetase deficiency45116002
- Sepiapterin reductase deficiency45116002
- Atypical PKU58256000
- Atypical phenylketonuria58256000
- DHPR - Dihydropteridine reductase deficiency58256000
- DHPR deficiency58256000
- Dihydropteridine reductase deficiency58256000
- Hyperphenylalaninaemia, type IV58256000
- Hyperphenylalaninemia, type IV58256000
- Phenylketonuria II58256000
- Hyperphenylalaninaemia68528007
- Hyperphenylalaninemia68528007
- Deficiency of tetrahydrobiopterin68724006
- Hyperphenylalanineaemia due to BH4 (tetrahydrobiopterin) deficiency68724006
- Hyperphenylalanineaemia due to tetrahydrobiopterin deficiency68724006
- Hyperphenylalaninemia due to BH4 (tetrahydrobiopterin) deficiency68724006
- Hyperphenylalaninemia due to tetrahydrobiopterin deficiency68724006
- Tetrahydrobiopterin synthesis defect68724006
- Folling disease190687004
- Oligophrenia phenylpyruvica190687004
- PAH (phenylalanine hydroxylase) deficiency190687004
- PAH-gene related phenylketonuria190687004
- PKU - phenylketonuria190687004
- Phenylalanine hydroxylase deficiency190687004
- Phenylketonuria190687004
- Disorder of tetrahydrobiopterin metabolism237913008
- 6-Pyruvoyl-tetrahydrobiopterin synthase deficiency237914002
- 6-Pyruvoyl-tetrahydropterin synthase deficiency237914002
- 6PTS - 6-Pyruvoyl-tetrahydrobiopterin synthase deficiency237914002
- Pterin-4-carbinolamine dehydratase deficiency276261007
- Maternal PKU (phenylketonuria)297225000
- Maternal phenylketonuria297225000
- PAH-gene related maternal phenylketonuria297225000
- Hyperphenylalanineaemia due to DNAJC12 deficiency1177177000
- Hyperphenylalanineaemia due to DnaJ heat shock protein family (Hsp40) member C12 deficiency1177177000
- Hyperphenylalaninemia due to DNAJC12 deficiency1177177000
- Hyperphenylalaninemia due to DnaJ heat shock protein family (Hsp40) member C12 deficiency1177177000
- Non-phenylketonuric non-BH4-deficiency hyperphenylalanineaemia1177177000
- Non-phenylketonuric non-BH4-deficiency hyperphenylalaninemia1177177000
Clinical Terms
- Hyperphenylalanineaemia due to tetrahydrobiopterin deficiency
- Atypical PKU
- PKU - phenylketonuria
- Tetrahydrobiopterin synthesis defect
- Essential hyperphenylalaninemia
- Folling disease
- Atypical phenylketonuria
- Hyperphenylalaninaemia, type IV
- Phenylketonuria
- Hyperphenylalaninemia due to DnaJ heat shock protein family (Hsp40) member C12 deficiency
- Hyperphenylalaninemia, type II
- Hyperphenylalaninemia, type IV
- GTP - Guanosine-5-triphosphate cyclohydrolase deficiency
- Hyperphenylalaninaemia, type I
- PAH (phenylalanine hydroxylase) deficiency
- Hyperphenylalaninemia due to DNAJC12 deficiency
- Hyperphenylalaninemia with neopterin deficiency
- Phenylketonuria II
- Disorder of tetrahydrobiopterin metabolism
- 6-Pyruvoyl-tetrahydrobiopterin synthase deficiency
- Hyperphenylalaninaemia, type III
- 7,8-Dihydrobiopterin synthetase deficiency
- DHPR deficiency
- Guanosine triphosphate cyclohydrolase I deficiency
- Hyperphenylalaninemia due to tetrahydrobiopterin deficiency
- Phenylalanine hydroxylase deficiency
- Hyperphenylalaninemia, type I
- Guanosine-5-triphosphate cyclohydrolase deficiency
- Persistent hyperphenylalaninaemia AND tyrosinaemia
- Non-phenylketonuric non-BH4-deficiency hyperphenylalaninemia
- Pterin-4-carbinolamine dehydratase deficiency
- Imbecilitus phenylpyruvica
- Persistent hyperphenylalaninemia
- Hyperphenylalaninaemia, type II
- Dihydropteridine reductase deficiency
- Guanosine triphosphate (GTP) cyclohydrolase I deficiency
- Non-phenylketonuric non-BH4-deficiency hyperphenylalanineaemia
- Hyperphenylalaninemia, type III
- Severe phenylalanine hydroxylase deficiency
- Transient mild hyperphenylalaninaemia
- Hyperphenylalanineaemia due to BH4 (tetrahydrobiopterin) deficiency
- Maternal phenylketonuria
- Hyperphenylalanineaemia due to DnaJ heat shock protein family (Hsp40) member C12 deficiency
- Sepiapterin reductase deficiency
- Deficiency of tetrahydrobiopterin
- Maternal PKU (phenylketonuria)
- Hyperphenylalaninaemia with neopterin deficiency
- 6PTS - 6-Pyruvoyl-tetrahydrobiopterin synthase deficiency
- Transient hyperphenylalaninemia
- Essential hyperphenylalaninaemia
- PAH-gene related maternal phenylketonuria
- Hyperphenylalaninemia, type VI
- Neonatal hyperphenylalaninemia
- Persistent hyperphenylalaninemia AND tyrosinemia
- PAH-gene related phenylketonuria
- Hyperphenylalanineaemia due to DNAJC12 deficiency
- Neonatal hyperphenylalaninaemia
- Persistent hyperphenylalaninaemia
- Transient mild hyperphenylalaninemia
- Hyperphenylalaninaemia
- GTP cyclohydrolase I deficiency
- Hyperphenylalaninemia
- 6-Pyruvoyl-tetrahydropterin synthase deficiency
- Oligophrenia phenylpyruvica
- Transient hyperphenylalaninaemia
- Classical phenylketonuria
- DHPR - Dihydropteridine reductase deficiency
- Hyperphenylalaninemia due to BH4 (tetrahydrobiopterin) deficiency
- Hyperphenylalaninaemia, type VI
Frequently Asked Questions
What is the ICD-10 code for other hyperphenylalaninemias?
The ICD-10-CM code for other hyperphenylalaninemias is E70.1. The full clinical description is "Other hyperphenylalaninemias". E70.1 is a billable/specific code that can be used on insurance claims and medical billing.
What does ICD-10 code E70.1 mean?
ICD-10-CM code E70.1 represents “Other hyperphenylalaninemias”. It is classified under Chapter 4: Endocrine, Nutritional and Metabolic Diseases and is a billable/specific code that can be used on a claim.
Is E70.1 a billable code?
Yes, E70.1 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.
What chapter is E70.1 in?
E70.1 is in Chapter 4: Endocrine, Nutritional and Metabolic Diseases (codes E00-E89).
What codes cannot be used with E70.1?
E70.1 has Excludes1 notes indicating codes that cannot be used together with it, including: transitory endocrine and metabolic disorders specific to newborn (P70-P74); androgen insensitivity syndrome (E34.5-); congenital adrenal hyperplasia (E25.0); and 3 more.
What SNOMED CT codes does E70.1 map to?
E70.1 maps to 15 SNOMED CT concepts: 237914002, 45116002, 58256000, 7573000, 68724006, and 10 more. SNOMED CT is a clinical terminology used in electronic health records.
What are the UMLS CUIs for E70.1?
E70.1 is linked to 1 UMLS Concept Unique Identifier: C0348482. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.
How does E70.1 relate to ICF functioning codes?
ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like other hyperphenylalaninemias affect a person's functioning: body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.
What is the ICD-11 equivalent of E70.1?
E70.1 maps to the ICD-11 code: 5C50.Z (Inborn errors of amino acid or other organic acid metabolism, unspecified).
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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.