Fibrosis quistica Flashcards

1
Q

Cromosoma afectado en la fibrosis quistica

A

brazo largo del cromosoma 7

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2
Q

mutacion mas frecuente de la fibrosis quistica

A

delecion de fenilalanina en la posicion delta 508

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3
Q

que tipo de deshidratacion presentan los px en la fibrosis quistica?

A

hipocloremica hiponatremica

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4
Q

que otro nombre recibe la fibrosis quisitica?

A

mucoviscidosis

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5
Q

Perdida total de fibrosis quistica

A

forma I, II y III

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6
Q

Perdida parcial de fibrosis quistica

A

IV y V

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7
Q

Se espesan todas las secresiones en la FQ, EXCEPTO

A

Cel. sudoriparas

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8
Q

proteina que interviene en la incapacidad para transportar el Cl de las cels epiteliales en la FQ?

A

Proteina Reguladora de la Conductancia Transmembrana de FQ (CFTR)

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9
Q

En la FQ, Cuando se suplementa con sal? y cuanto?

A

Cuando haya calor o diarrea (1-4 gr)

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10
Q

1º causa de muerte en los px con FQ?

A

Neumonias

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11
Q

Patogeno mas frecuente en las neumonias en px con FQ?

A

Hongos: CANDIDA

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12
Q

Alteracion mas frecuente en la FQ?

A

mala absorcion intestinal

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13
Q

Clinica en la FQ?

A

ileo meconial, mala absorcion intestinal, prolapso rectal (secundario al ileo meconial), deshidratacion, infertilidad por H: azoospermia M: deshidratacion de moco cervical

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14
Q

Como se hace el dx en la FQ?

A

-Tamiz neonatal– Tripsina inmunoreactiva

Prueba de cloruros en sudor (test del sudor): DX: >60 mEq

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15
Q

Seguimiento del px con FQ:

A
  • Rx torax anual

- Estudio de esputo c/3 meses

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16
Q

Tx en la FQ

A
  • Antibiotico: tobra, amika y genta
  • Mucolitico: acetilcisteina, alfadormasa
  • Inhaloterapia y broncodilatador
  • Dieta: ADEK (vitaminas)