Porphyria cutanea tarda

ICD-11 code : 5C58.10;

Preferred Label : Porphyria cutanea tarda;

ICD-11 definition : Porphyria cutanea tarda (PCT) is due to an accumulation of uroporphyrins in plasma from blockage of the normal haem synthetic pathway in the liver at the level of uroporphyrinogen decarboxylase (URO-D). The majority of cases are sporadic and frequently associated with iron overload. PCT manifests as skin fragility and blistering in light-exposed skin, particularly on the dorsa of the hands, together with hypertrichosis.;

ICD-11 synonym : UROD deficiency; UROD - [uroporphyrinogen decarboxylase] deficiency; Porphyria, hepatocutaneous type; Heterozygous uroporphyrinogen decarboxylase deficiency; PCT - [porphyria cutanea tarda];

ICD-11 acronym : PCT;

Détails

Origin ID

370983230;

UMLS CUI

C0162566;

Currated CISMeF NLP mapping
- Porphyria Cutanea Tarda [NCIt concept]
- Porphyria cutanea tarda [PASCAL descriptor]
- Porphyria cutanea tarda [MedDRA LLT]
- Porphyria cutanea tarda [OMIM Phenotype]
- Porphyria cutanea tarda [ORDO Disease]
- Porphyria cutanea tarda [ICD-10 Sub-category]
- Porphyria cutanea tarda [ICD-10 Sub-category (who)]
- Porphyria cutanea tarda (disorder) [SNOMED CT concept]
- porphyria cutanea tarda [MeSH concept]
- porphyria cutanea tarda [DO Concept]
- porphyria cutanea tarda [MeSH Descriptor]
- porphyria cutanea tarda [Disease (Nov.)]
- porphyria cutanea tarda, nos [SNOMED Notion]
False automatic mappings
- Percent (property) (qualifier value) [SNOMED CT concept]
- Prevention, Clinical, and Therapeutic Subcommittee [NCIt concept]
- Procalcitonin [MeSH concept]
- Procalcitonin Measurement [NCIt concept]
- prothrombin consumption time, nos [SNOMED Notion]
ICD-10 Mapping
- Porphyria cutanea tarda [ICD-10 Sub-category]
See also inter- (CISMeF)
- CALCA wt Allele [NCIt concept]
- UROD wt Allele [NCIt concept]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]

Position du mot-clé dans l'(les) arborescence(s) :

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