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Videos de Conceptos Relacionados

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Chronic Pancreatitis II: Pathophysiology01:21

Chronic Pancreatitis II: Pathophysiology

Chronic pancreatitis is a progressive and irreversible inflammation of the pancreas, most often caused by long-term alcohol abuse, but it can also be related to ductal obstruction, smoking, or genetic factors.Chronic pancreatitis occurs when the pancreas is repeatedly exposed to harmful agents like alcohol, smoking, ductal obstruction, or genetic predisposition. These factors lead to the release of toxic metabolites and inflammatory cytokines, sustaining chronic inflammation in the pancreatic...
Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features01:24

Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features

Chronic bronchitis is a key phenotype of chronic obstructive pulmonary disease (COPD), characterized by airway-centered inflammation and mucus overproduction. It develops from long-term exposure to harmful particles or gases, most commonly cigarette smoke, which triggers a persistent inflammatory response.Cellular and Structural ChangesInflammation initially affects the large bronchi and later the smaller airways, with infiltration by immune cells, including neutrophils, macrophages, and...
Chronic Pancreatitis I: Introduction01:25

Chronic Pancreatitis I: Introduction

Chronic pancreatitis is a long-standing, relapsing inflammation of the pancreas, characterized by irreversible damage to the gland. It results in progressive destruction of the pancreatic parenchyma, fibrosis, and eventual loss of both exocrine and endocrine function. The disease may evolve gradually after multiple episodes of acute pancreatitis or develop independently.EtiologyChronic pancreatitis can arise from a variety of causes:Alcohol use is the leading cause, accounting for 70–80% of...
Chronic Pancreatitis I: Introduction01:24

Chronic Pancreatitis I: Introduction

The pancreas, an elongated and flat gland situated behind the stomach, serves a vital function in digesting food and managing blood sugar levels.
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...

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Corrigendum to "Results of a clinical trial of ANG003, a non-porcine pancreatic enzyme replacement therapy, in people with cystic fibrosis" [Journal of Cystic Fibrosis Original Article Articles in Press July 31, 2025].

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Updated: Jun 23, 2026

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
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Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Published on: August 29, 2025

La fibrosis quística también se conoce como fibrosis quística.

Brian P O'Sullivan1, Steven D Freedman

  • 1Department of Pediatrics, University of Massachusetts Medical School, Worcester, MA 01655, USA. osullivb@ummhc.org

Lancet (London, England)
|May 1, 2009
PubMed
Resumen
Este resumen es generado por máquina.

La atención de la fibrosis quística (FQ) ha mejorado debido al diagnóstico precoz y las terapias avanzadas dirigidas al defecto básico. Nuevos tratamientos están surgiendo, ofreciendo esperanza para los pacientes con esta enfermedad genética letal común.

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Published on: April 11, 2018

Área de la Ciencia:

  • Genética La genética.
  • Pulmonología Pulmonología.
  • Biología Molecular Biología Molecular

Sus antecedentes:

  • La fibrosis quística es un trastorno genético letal prevalente en las poblaciones caucásicas.
  • Las mejoras significativas en los resultados de los pacientes se atribuyen al diagnóstico temprano, los tratamientos intensivos y los centros de atención especializada.
  • Los avances en la comprensión de la base molecular de la FQ están allanando el camino para nuevas estrategias terapéuticas.

Objetivo del estudio:

  • Revisar las normas actuales de atención clínica para la fibrosis quística.
  • Para discutir los avances recientes en la comprensión de la patogénesis de la FQ.
  • Para resaltar los avances en el cribado neonatal y el desarrollo de terapias dirigidas al defecto molecular subyacente.

Principales métodos:

  • Revisión de la literatura de las prácticas de atención clínica.
  • Análisis de investigaciones recientes sobre la patogénesis de la fibrosis quística.
  • Examen de los protocolos emergentes de cribado neonatal.
  • Evaluación de nuevos enfoques terapéuticos, incluida la solución salina hipertónica y los tratamientos para el defecto básico.

Principales resultados:

  • Las perspectivas de los pacientes con fibrosis quística han mostrado una mejora constante con el tiempo.
  • Una comprensión más profunda del defecto molecular que impulsa la FQ está facilitando nuevas vías de tratamiento.
  • La solución salina hipertónica es un tratamiento establecido, con otras terapias novedosas en desarrollo avanzado.

Conclusiones:

  • El progreso continuo en la comprensión de la patogénesis de la fibrosis quística es crucial.
  • La mejora de la detección neonatal y las terapias dirigidas están transformando el manejo de la FQ.
  • El desarrollo de tratamientos que aborden el defecto molecular fundamental es una promesa significativa para mejorar la vida de las personas con fibrosis quística.