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PORPHYRIAS, HEPATIC
Descriptors Found:
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DeCS
Descriptor
English
:
Porphyrias, Hepatic
Descriptor
Spanish
:
Porfirias Hepáticas
Descriptor
Portuguese
:
Porfirias Hepáticas
Synonyms
English
:
Hepatic Porphyria
Tree Number:
C06.552.830
C16.320.565.708.400
C16.320.850.742
C17.800.827.742
C17.800.849.617.400
C18.452.648.708.400
C18.452.811.400
C18.452.880.617.400
Definition
English
:
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.
Indexing Annotation
English
:
do not confuse with PORPHYRIA, ERYTHROHEPATIC
History Note
English
:
2005 (1993)
Allowable Qualifiers
English
:
blood
cerebrospinal fluid
chemically induced
classification
congenital
complications
diet therapy
diagnosis
drug therapy
economics
ethnology
embryology
enzymology
epidemiology
etiology
genetics
history
immunology
metabolism
microbiology
mortality
nursing
pathology
prevention & control
physiopathology
parasitology
psychology
radiography
rehabilitation
radionuclide imaging
radiotherapy
surgery
therapy
urine
ultrasonography
veterinary
virology
Record Number:
30605
Unique Identifier:
D017094
Occurrence in VHL
:
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