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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): Hepatic Porphyria
Hepatic Porphyrias
Porphyria, Hepatic
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 blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CN congenital
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Previous Indexing: Porphyria (1966-1992)
Public MeSH Note: 2005; see PORPHYRIA, HEPATIC 1993-2004
History Note: 2005 (1993)
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
Porphyrias, Hepatic - Preferred
Concept UI M0025973
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.
Preferred term Porphyrias, Hepatic
Entry term(s) Hepatic Porphyria
Hepatic Porphyrias
Porphyria, Hepatic



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