Es un trastorno hereditario que provoca daños graves en los pulmones, el aparato digestivo y otros órganos del cuerpo.

Alofecta las células que producen mucosidad, sudor y jugos digestivos. Debido a una mutacion genetica las secreciones son más pegajosas y espesas, en lugar de actuar como lubricantes, se acumulan en los tubos, conductos y pasajes, en particular en los pulmones y el páncreas.

Esta enfermedad requiere cuidados diarios, sin embargo aquellos que la padecen generalmente pueden asistir a la escuela y el trabajo.


Las mejoras en los exámenes de detección y los tratamientos permitieron que las personas con fibrosis quística hoy puedan vivir, en promedio, hasta una edad de entre 35 y 39, y algunas, incluso, hasta los 40 y 50 años.

Síntomas

Los signos y síntomas de la fibrosis quística varían según la gravedad de la enfermedad. Incluso en la misma persona, los síntomas pueden empeorar o mejorar con el paso del tiempo. En algunos casos, los síntomas pueden no manifestarse hasta la adolescencia o la adultez.

Las personas que padecen fibrosis quística tienen más cantidad de sal en el sudor. A menudo, los padres pueden notar la sal cuando besan a sus hijos. La mayoría de los demás signos y síntomas de la fibrosis quística afectan los aparatos respiratorio y digestivo. Sin embargo, los adultos a los que se les diagnostica esta enfermedad son más propensos a presentar síntomas atípicos, como episodios recurrentes de inflamación del páncreas (pancreatitis), infertilidad y neumonía recurrente.

Signos y síntomas respiratorios
La mucosidad espesa y pegajosa característica de la fibrosis quística obstruye las vías respiratorias. Esto puede provocar los siguientes signos y síntomas:
-Tos persistente con mucosidad espesa (esputo)
-Silbido al respirar
-Falta de aliento
-Intolerancia al ejercicio
-Infecciones pulmonares recurrentes
-Fosas nasales inflamadas o congestión nasal

-Silbido al respirar
-Falta de aliento
-Intolerancia al ejercicio
-Infecciones pulmonares recurrentes
-Fosas nasales inflamadas o congestión nasal.

Signos y síntomas digestivos

La mucosidad espesa también puede obstruir las vías que transportan las enzimas digestivas desde el páncreas hasta el intestino delgado. A menudo, el resultado es el siguiente:

-Heces malolientes y grasosas
-Problemas para aumentar de peso y crecer
-Obstrucción intestinal, particularmente en los recién nacidos (íleo meconial)
-Estreñimiento grave
-Problemas para aumentar de peso y crecer
-Obstrucción intestinal, particularmente en los recién nacidos (íleo meconial)
-Estreñimiento grave


Debido al esfuerzo realizado para defecar, puede darse un prolapso rectal . Cuando esto ocurre en los niños, puede ser signo de fibrosis quística. A veces, el prolapso rectal en niños requiere cirugía.

¿Existe una cura?

Fibrosis Quística es una enfermedad compleja para la cual no existe hoy en día una curación definitiva, sin embargo cada año mejoran o aparecen nuevas formas de tratamiento, que permiten al paciente un desarrollo adecuado y la posibilidad de llegar a una vida adulta plena y productiva, tan normal como la de cualquier individuo.

Fuente

MayoClinic – Fibrosis Quistica ( https://www.mayoclinic.org/es-es/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700)
Clinica San Pablo – Que es la Fibrosis Quistica ( http://www.sanpablo.com.pe/que-es-la-fibrosis-quistica/)

Categorías: IFMSA-La RiojaSCOPH

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