von hippel lindau semio
TRANSCRIPT
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SindromesSindromes NeurocutaneosNeurocutaneosFacomatosis
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Grupo de enfermedades congenitas,habitualmente hereditarias que afectana tejidos derivados del Neuroectodermo(Piel y al sistema Nervioso)asociada a tumores y comprosmisomultisistemico.
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E SCLERO SIS TUBERO SAT rastorno de ladiferenciacin yproliferacin celular,que puede afectar elcerebro, la piel, elcorazn, el ojo y elrin y otros rganos.
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M ANIFE STACIO NE S
NE
URO
LGICO
S
R etardo
mental
Adenoma
Sebaceo
Epilepsia
intratable
Autismo
Sindrome deHipertension
Endocraneana Angiofibroma enla region malar de la cara
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M cula hipopigmentada
M ANIFE STACIO NE S
DER M
ATO
LGICAS
parche lijoso
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Angiofibromasfaciales
Fibromas ungueales
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Astrocitomasretinianos
Hamartomas retinianos
. reas acrmicas en laretina.
M ANIFE STACIO NE SO FTALM O LGICAS
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AFE CTACINRE NAL
Angiomiolipomas
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AFE CTACIN CAR DACA
R abdomioma Cardaco
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AFE CTACIN PULM O NAR
linfoangio leiomiomatosis
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Sndrome hereditario caracterizado por laexistencia de ndulos a modo de tumoresbenignos en los ojos, la piel y el cerebro.
Etiopatogenia desconocida, aunquepuede por un error que afectaespecficamente a una zona de la crestaneural.
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nevo cutaneofacia l
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h emangiomas intrabuca les
sus caractersticas se inc luyen:
ca lcificaciones intracranea les
h iperp lasia gingiva l
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SNTO M AS
NEVUSFACIAL (EN VINO
DE PORT O
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PR OBLEM ASVISUALES (GLAUCOM A /
HEM IANOPSIA)
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CONVULSIONES
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DIFICULTADPAR A EL
APR ENDIZAJE
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hiperplasias vascularesThe image part with relationship ID rId3 was not found in the file.
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hemorragia gingivalThe image part with relationship ID rId5 was not found in the file.
M ANIFE STACIO NE S BUCALE S
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V on Hippe l Lindau
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V O N HIPPE L LINDAUEnfermedad autosmica Dominante(mutacion de un gen)
Predisposicion a tumores en diferentesorganos.
Incidencia: 1/36.000
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Entre ellos:Hemangioblastomas del SNCAngiomas retinianosCancer renal y quistesFeocromocitomasQuistes y tumores pancreaticos
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M ANIFE STACIO NE S CLINICAS1. M ANIFE STACIO NE S
O CULARE S
Angiomas R etinianos
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M anifestaciones:
Disminucin de laagudeza visual oalteraciones del campovisualHemorragias
Desprendimientos de laretina o pliegues en lamacula
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SISTE M A NER V IO SO CE NTR AL
Hemangiob lastomas
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Sintomato logaCefaleaNauseaVomitoM areoAtaxiaSensacin de
InestabilidadT umores medulares:Dolor el mas referido
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CANCER RE NAL YQUISTE S
C ncer R enal
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FEO CRO M O CITO M A
SINTO M ATO LO GIA:Cefa leaSudoracinPa lpitaciones con o sintaquicardiaNerviosismoPerdida de pesoDolor abdomina lNauseas con o sin vmitos
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PANCRE ASPueden ser de 2tipos:Quistes pancreaticosy cistoadenomasserososT umores de losislotes pancreaticos
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