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PORPHYRIAS, HEPATIC
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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
:
BL
blood
CF
cerebrospinal fluid
CI
chemically induced
CL
classification
CO
complications
CN
congenital
DI
diagnosis
DH
diet therapy
DT
drug therapy
EC
economics
EM
embryology
EN
enzymology
EP
epidemiology
EH
ethnology
ET
etiology
GE
genetics
HI
history
IM
immunology
ME
metabolism
MI
microbiology
MO
mortality
NU
nursing
PS
parasitology
PA
pathology
PP
physiopathology
PC
prevention & control
PX
psychology
RA
radiography
RI
radionuclide imaging
RT
radiotherapy
RH
rehabilitation
SU
surgery
TH
therapy
US
ultrasonography
UR
urine
VE
veterinary
VI
virology
Record Number:
30605
Unique Identifier:
D017094
Occurrence in VHL
:
Similar:
DeCS
CID-10
SciELO
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