Disorders of branched-chain amino acid and fatty acid metabolism — ICD-10 code E71
ICD-10 code E71 — Disorders of branched-chain amino acid and fatty acid metabolism (Class E00-E90, Endocrine, nutritional and metabolic diseases). There are 9 guidelines from the Russian Ministry of Health associated with this code.
Subcategories of E71
- E71.0 — Maple syrup urine disease
- E71.1 — Other disorders of branched-chain amino acid metabolism
- E71.3 — Disorders of fatty acid metabolism
Guidelines
- X-linked adrenoleukodystrophy
- Maple syrup urine disease
- 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
- Other disorders of branched-chain amino acid metabolism (Methylmalonic acidemia/aciduria)
- Isovaleric acidemia/aciduria
- Disorders of mitochondrial fatty acid $\beta$-oxidation
- 3-Methylcrotonyl-CoA carboxylase deficiency (3-methylcrotonylglycinuria, methylcrotonyl-CoA carboxylase deficiency)
- Mitochondrial acetoacetyl-CoA thiolase deficiency ($\beta$-ketothiolase deficiency, T2 deficiency)
- Propionic acidemia/aciduria
Clinical calculators and scalesRisk calculation, dosing, and scoring using scales from clinical guidelines — online, no registration required.
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