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XERODERMA PIGMENTOSUM
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DeCS
Descriptor
English
:
Xeroderma Pigmentosum
Descriptor
Spanish
:
Xerodermia Pigmentosa
Descriptor
Portuguese
:
Xeroderma Pigmentoso
Synonyms
English
:
Kaposi Disease
Tree Number:
C04.834.867
C16.131.831.936
C16.320.850.970
C17.800.600.925
C17.800.621.936
C17.800.804.936
C17.800.827.970
C18.452.284.975
Definition
English
:
A rare, pigmentary, and atrophic autosomal recessive
disease
. It is manifested as an extreme photosensitivity to
ULTRAVIOLET RAYS
as the result of a deficiency in the enzyme that permits excisional repair of ultraviolet-damaged
DNA.
Indexing Annotation
English
:
if non-neoplastic, coordinate IM with precoordinated organ/disease term (IM); if neoplastic, coordinate IM with organ/neoplasm term (IM); do not confuse KAPOSI
DISEASE
with
SARCOMA
, KAPOSI
History Note
English
:
63; KAPOSI DISEASE was KAPOSI'S DISEASE 1963-97
Allowable Qualifiers
English
:
blood
blood supply
cerebrospinal fluid
chemistry
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
secondary
secretion
surgery
therapy
ultrastructure
urine
ultrasonography
veterinary
virology
Record Number:
15382
Unique Identifier:
D014983
Occurrence in VHL
:
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