Liver diseases due to porphyria

ICD-11 code : 5C58.11;

Preferred Label : Liver diseases due to porphyria;

ICD-11 definition : The porphyrias are a group of rare inherited or acquired disorders of certain enzymes that normally participate in the production of porphyrins and heme. Pathogenesis is storage of porphyrins, porphyrinogens, and their precursors. They manifest with either neurological complications or skin problems, or occasionally both. Porphyrias are classified as hepatic or erythropoietic based on the sites of accumulation of heme precursors, either in the liver or bone marrow and red blood cells.;

Details

Origin ID

554074681;

UMLS CUI

C0162533;

CISMeF manual mappings
- porphyrias, hepatic [MeSH Descriptor]
Currated CISMeF NLP mapping
- Hepatic porphyria (disorder) [SNOMED CT concept]
- hepatic porphyria, nos [SNOMED Notion]
- hepatic porphyria, nos [TUV Term]
- hepatic porphyria, nos [TUV Concept]
- liver porphyria [Disease (Nov.)]
- porphyrias, hepatic [MeSH concept]
ICD-10 Mapping
- Disorders of porphyrin and bilirubin metabolism [ICD-10 category]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
UMLS correspondences (same concept)
- Hepatic porphyria (disorder) [SNOMED CT concept]
- Porphyria hepatic [MedDRA LLT]
- acute porphyria [DO Concept]
- hepatic porphyria, nos [SNOMED Notion]
- hepatic porphyria, nos [TUV Concept]
- hepatic porphyria, nos [TUV Term]
- liver porphyria [Disease (Nov.)]
- porphyrias, hepatic [MeSH Descriptor]
- porphyrias, hepatic [MeSH concept]
Validated automatic mappings to NTBT
- Disorder of porphyrin metabolism (disorder) [SNOMED CT concept]
- Disorders of porphyrin and bilirubin metabolism [ICD-10 category]

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