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Descriptor English: Porphyrias, Hepatic
Descriptor Spanish: Porfirias Hepáticas
Descriptor porfirias hepáticas
Entry term(s) porfiria hepática
Scope note: Grupo de enfermedades metabólicas debidas a deficiencia de ciertas enzimas hepáticas que intervienen en la biosíntesis del HEMO. Se caracterizan por acumulación e incremento de la excreción de PORFIRINAS o sus precursores. Las características clínicas comprenden síntomas neurológicos (PORFIRIA INTERMITENTE AGUDA), lesiones cutáneas por fotosensibilidad (PORFIRIA CUTÁNEA TARDA) o ambas manifestaciones (COPROPORFIRIA HEREDITARIA). Las porfirias hepáticas pueden ser congénitas o adquiridas por toxicidad hepática.
Descriptor Portuguese: Porfirias Hepáticas
Descriptor French: Porphyries hépatiques
Entry term(s): Porphyrie hépatique
Tree number(s): C06.552.830
C16.320.850.742
C17.800.827.742
C18.452.811.400
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D017094
Scope note: A group of metabolic diseases due to deficiency of one of a number of LIVER enzymes in the biosynthetic pathway of HEME. They are characterized by the accumulation and increased excretion of PORPHYRINS or its precursors. Clinical features include neurological symptoms (PORPHYRIA, ACUTE INTERMITTENT), cutaneous lesions due to photosensitivity (PORPHYRIA CUTANEA TARDA), or both (HEREDITARY COPROPORPHYRIA). Hepatic porphyrias can be hereditary or acquired as a result of toxicity to the hepatic tissues.
Annotation: do not confuse with PORPHYRIA, ERYTHROHEPATIC
Allowable Qualifiers: BL sang
CF liquide cérébrospinal
CI induit chimiquement
CL classification
CN congénital
CO complications
DG imagerie diagnostique
DH diétothérapie
DI diagnostic
DT traitement médicamenteux
EC économie
EH ethnologie
EM embryologie
EN enzymologie
EP épidémiologie
ET étiologie
GE génétique
HI histoire
IM immunologie
ME métabolisme
MI microbiologie
MO mortalité
NU soins infirmiers
PA anatomopathologie
PC prévention et contrôle
PP physiopathologie
PS parasitologie
PX psychologie
RH rééducation et réadaptation
RT radiothérapie
SU chirurgie
TH thérapie
UR urine
VE médecine vétérinaire
VI virologie
DeCS ID: 30605
Unique ID: D017094
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1993/01/01
Date of Entry: 1992/05/22
Revision Date: 2015/06/05
Porphyries hépatiques - Preferred
Concept UI M0025973
Preferred term Porphyries hépatiques
Entry term(s) Porphyrie hépatique



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