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DUBIN-JOHNSON SYNDROME
Descriptors Found:
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DeCS
Descriptor
English
:
Jaundice, Chronic Idiopathic
Descriptor
Spanish
:
Ictericia Idiopática Crónica
Descriptor
Portuguese
:
Icterícia Idiopática Crônica
Synonyms
English
:
Dubin-Johnson Syndrome
Tree Number:
C16.320.565.300.764
C16.614.451.500.250
C18.452.648.300.764
Definition
English
:
A benign, autosomally recessive inherited
hyperbilirubinemia
characterized by the presence of a dark pigment in the centrilobular region of the
liver
cells
. There is a functional defect in biliary excretion of
bilirubin
, cholephilic dyes, and
porphyrins
. Affected
persons
may be asymptomatic or have vague constitutional or gastrointestinal
symptoms
. The
liver
may be slightly enlarged, and oral and intravenous
cholangiography
fails to visualize the
biliary tract.
Allowable Qualifiers
English
:
blood
cerebrospinal fluid
chemically induced
classification
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:
7735
Unique Identifier:
D007566
Occurrence in VHL
:
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