Mucopolisacaridosis Flashcards

1
Q

Defina Mucopolisacaridosis (MPS):

A

Errores Inhatos del Metabolismo, producen acumulo de Glucosaminoglicanos

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2
Q

Clasificacion de las Mucopolisacaridosis

A

Se divide en 9 (I-IX) Pero son 7

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3
Q

Sindrome Mas comun de Mucopolisacaridosis (MPS)

A

TIpo III (Enfermedad de San Filippo)

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4
Q

Sindrome de MPS con mas afecciones Ortopedicas:

A

Tipo IV (sindrome de Morquio)

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5
Q

Deficiencias Enzimaticas en el Sindrome De Morquio

A

Tipo A/ Galactasa-6-Sulfatasa

Tipo B/ Galactidasa

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6
Q

Afecciones Ortopedicas Mas comunes en Sindrome de Morquio:

A

Pie Zambo, DDC, Giba Toracolumbar, Rigidez Articular, inestabilidad AtlantoAxoidea, Hipoplasia del Odontoides

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7
Q

Unico Sindrome de la MPS ligado al Sexo

A

Tipo II Sindrome de HUNTER (deficiencia de la IDS2)

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