E71.19
BillableOther disorders of branched-chain amino-acid metabolism
Other disorders of branched-chain amino-acid metabolism
Coding Notes
Inclusion Terms
Alternative clinical terms for this condition
- Hyperleucine-isoleucinemia
- Hypervalinemia
Excludes 1
Codes that cannot be used together with this code (mutual exclusion)
Also Known As / Clinical Terms(161)
SNOMED CT
- Hyperleucine-isoleucinaemia7046009
- Hyperleucine-isoleucinemia7046009
- 3-Methylcrotonyl-CoA carboxylase deficiency13144005
- BMCC deficiency13144005
- MCC deficiency13144005
- Methylcrotonyl-CoA carboxylase deficiency13144005
- Methylcrotonyl-coenzyme A carboxylase deficiency13144005
- beta-Methylcrotonylglycinuria, type 113144005
- Hyperleucinaemia24013007
- Hyperleucinemia24013007
- Leucine sensitivity24013007
- Leucinosis24013007
- Hyperaminoaciduria42930003
- Inborn error of amino acid metabolism42930003
- Autism, childhood onset43614003
- Autistic disorder of childhood onset43614003
- Childhood autism43614003
- Hypervalinaemia47719001
- Hypervalinemia47719001
- Valinaemia47719001
- Valine transaminase deficiency47719001
- Valinemia47719001
- Intermittent branched-chain ketonuria52270006
- Leucine-induced hypoglycaemia62151007
- Leucine-induced hypoglycemia62151007
- 3-Ketothiolase deficiency124258007
- 3-Methylhydroxybutyric acidaemia124258007
- 3-Methylhydroxybutyric acidemia124258007
- Acetoacetyl-CoA thiolase deficiency124258007
- Deficiency of acetoacetyl-CoA thiolase124258007
- Deficiency of acetyl-CoA acetyltransferase124258007
- Deficiency of acetyl-coenzyme A acetyltransferase124258007
- alpha-Methylacetoacetic aciduria124258007
- Deficiency of leucine aminotransferase124288003
- Deficiency of leucine-tRNA ligase124691008
- Deficiency of leucine-transfer ribonucleic acid ligase124691008
- Deficiency of leucyl-tRNA synthetase124691008
- Deficiency of isoleucine-tRNA ligase124692001
- Deficiency of isoleucine-transfer ribonucleic acid ligase124692001
- Deficiency of isoleucyl-tRNA synthetase124692001
- Deficiency of valine-tRNA ligase124695004
- Deficiency of valine-transfer ribonucleic acid ligase124695004
- Deficiency of valyl-tRNA synthetase124695004
- Isoleucinosis190701006
- Valinosis237944004
- Beta-ketothiolase deficiency237953006
- Mitochondrial 2-methylacetoacetyl-CoA thiolase deficiency - potassium stimulated237953006
- 3-Hydroxyisobutyric aciduria237957007
- Clinical manifestation of enzyme deficiency410053003
- 3-hydroxy-3-methylglutaric aciduria410059004
- 3-hydroxy-3-methylglutaryl-CoA lyase deficiency410059004
- HMG-CoA lyase deficiency410059004
- Hydroxymethylglutaric aciduria410059004
- Disorder of isoleucine metabolism444755001
- Disorder of valine metabolism444756000
- Isobutyrylglycinuria445571008
- CMAMMA - combined malonic and methylmalonic aciduria702365002
- Combined malonic and methylmalonic aciduria702365002
- Autism epilepsy syndrome due to branched chain ketoacid dehydrogenase kinase deficiency771448004
- Developmental delay due to ALDH6A1 (aldehyde dehydrogenase 6 family member A1) deficiency782828005
- Developmental delay due to MMSDH (methylmalonate semialdehyde dehydrogenase) deficiency782828005
- Developmental delay due to methylmalonate semialdehyde dehydrogenase deficiency782828005
- ACAD8-gene related deficiency of isobutyryl-coenzyme A dehydrogenase1306747001
- Isobutyric aciduria1306747001
- Isobutyryl-CoA dehydrogenase deficiency disease1306747001
- Isobutyryl-coenzyme A dehydrogenase deficiency disease1306747001
- 2-methylbutyric aciduria1306751004
- 2-methylbutyryl-coenzyme A dehydrogenase deficiency disease1306751004
- ACADSB-gene related deficiency of 2-methylbutyryl-coenzyme A dehydrogenase1306751004
- Short/branched-chain acyl-coA dehydrogenase deficiency disease1306751004
UMLS
- HYPERLEUCINE-ISOLEUCINEMIAC0268574
- Hyperleucine-IsoleucinemiaC0268574
- Hyperleucine-isoleucinaemiaC0268574
- Hyperleucine-isoleucinemiaC0268574
- Hyperleucine-isoleucinemia (disorder)C0268574
- HYPERVALINEMIAC0268573
- High blood valine concentrationC0268573
- HypervalinaemiaC0268573
- HypervalinemiaC0268573
- Hypervalinemia (disorder)C0268573
- VALINE TRANSAMINASE DEFICIENCYC0268573
- VALINEMIAC0268573
- ValinaemiaC0268573
- Valine transaminase deficiencyC0268573
- ValinemiaC0268573
- hypervalinemiaC0268573
- valinemiaC0268573
- Other disorders of branched-chain amino-acid metabolismC0348484
Clinical Terms
- Autism, childhood onset
- Deficiency of leucine-transfer ribonucleic acid ligase
- Deficiency of leucine aminotransferase
- ACAD8-gene related deficiency of isobutyryl-coenzyme A dehydrogenase
- 3-Methylhydroxybutyric acidemia
- HYPERVALINEMIA
- Beta-ketothiolase deficiency
- 2-methylbutyryl-coenzyme A dehydrogenase deficiency disease
- Developmental delay due to methylmalonate semialdehyde dehydrogenase deficiency
- Hyperleucine-isoleucinaemia
- VALINE TRANSAMINASE DEFICIENCY
- HYPERLEUCINE-ISOLEUCINEMIA
- 3-Ketothiolase deficiency
- 3-Hydroxyisobutyric aciduria
- Deficiency of acetyl-CoA acetyltransferase
- alpha-Methylacetoacetic aciduria
- Isobutyryl-coenzyme A dehydrogenase deficiency disease
- Combined malonic and methylmalonic aciduria
- Short/branched-chain acyl-coA dehydrogenase deficiency disease
- Deficiency of leucyl-tRNA synthetase
- Methylcrotonyl-CoA carboxylase deficiency
- Deficiency of acetyl-coenzyme A acetyltransferase
- VALINEMIA
- 3-hydroxy-3-methylglutaryl-CoA lyase deficiency
- beta-Methylcrotonylglycinuria, type 1
- MCC deficiency
- Methylcrotonyl-coenzyme A carboxylase deficiency
- Developmental delay due to ALDH6A1 (aldehyde dehydrogenase 6 family member A1) deficiency
- Hyperleucinemia
- CMAMMA - combined malonic and methylmalonic aciduria
- Developmental delay due to MMSDH (methylmalonate semialdehyde dehydrogenase) deficiency
- Inborn error of amino acid metabolism
- Autism epilepsy syndrome due to branched chain ketoacid dehydrogenase kinase deficiency
- Valinaemia
- BMCC deficiency
- Hyperleucinaemia
- Deficiency of leucine-tRNA ligase
- Deficiency of isoleucine-transfer ribonucleic acid ligase
- Leucine-induced hypoglycaemia
- Leucine sensitivity
- Leucine-induced hypoglycemia
- Mitochondrial 2-methylacetoacetyl-CoA thiolase deficiency - potassium stimulated
- Deficiency of valine-transfer ribonucleic acid ligase
- Deficiency of isoleucyl-tRNA synthetase
- Hypervalinaemia
- Isobutyric aciduria
- High blood valine concentration
- HMG-CoA lyase deficiency
- Childhood autism
- 3-Methylcrotonyl-CoA carboxylase deficiency
- 3-hydroxy-3-methylglutaric aciduria
- Autistic disorder of childhood onset
- Isobutyrylglycinuria
- ACADSB-gene related deficiency of 2-methylbutyryl-coenzyme A dehydrogenase
- Acetoacetyl-CoA thiolase deficiency
- Deficiency of acetoacetyl-CoA thiolase
- Deficiency of valyl-tRNA synthetase
- Hypervalinemia (disorder)
- Deficiency of valine-tRNA ligase
- Clinical manifestation of enzyme deficiency
- Hyperaminoaciduria
- Isoleucinosis
- Intermittent branched-chain ketonuria
- 3-Methylhydroxybutyric acidaemia
- Leucinosis
- 2-methylbutyric aciduria
- Hyperleucine-isoleucinemia (disorder)
- Disorder of valine metabolism
- Disorder of isoleucine metabolism
- Valinosis
- Deficiency of isoleucine-tRNA ligase
- Hydroxymethylglutaric aciduria
- Isobutyryl-CoA dehydrogenase deficiency disease
Frequently Asked Questions
What is the ICD-10 code for other disorders of branched-chain amino-acid metabolism?
The ICD-10-CM code for other disorders of branched-chain amino-acid metabolism is E71.19. The full clinical description is "Other disorders of branched-chain amino-acid metabolism". E71.19 is a billable/specific code that can be used on insurance claims and medical billing.
What does ICD-10 code E71.19 mean?
ICD-10-CM code E71.19 represents “Other disorders of branched-chain amino-acid metabolism”. 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 E71.19 a billable code?
Yes, E71.19 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.
What chapter is E71.19 in?
E71.19 is in Chapter 4: Endocrine, Nutritional and Metabolic Diseases (codes E00-E89).
What codes cannot be used with E71.19?
E71.19 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 E71.19 map to?
E71.19 maps to 27 SNOMED CT concepts: 1306751004, 237957007, 124258007, 13144005, 410059004, and 22 more. SNOMED CT is a clinical terminology used in electronic health records.
What are the UMLS CUIs for E71.19?
E71.19 is linked to 3 UMLS Concept Unique Identifiers: C0268574, C0268573, C0348484. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.
How does E71.19 relate to ICF functioning codes?
ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like other disorders of branched-chain amino-acid metabolism 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 E71.19?
There is no direct ICD-11 mapping available for E71.19 in the WHO crosswalk tables. This may mean the concept is classified differently in ICD-11. Use the ICD-10 to ICD-11 converter to search for related codes.
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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.