E70.1

Billable

Other hyperphenylalaninemias

Other hyperphenylalaninemias

Status

Billable / Specific

Block

E70-E88

Parent Code

E70

ICD-11 Mapping

1 equivalent

Coding Notes

Related Codes(7)
ICD-11 Equivalents(1)
Also Known As / Clinical Terms(139)

SNOMED CT

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).

Automate ICD-10 Coding With AI

Send clinical text to the AutoICD API and get back structured ICD-10 codes with confidence scores. Integrates into any EHR or billing system in minutes.

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.