Enfermedad hepática en la fibrosis quística pediátrica. Una revisión de los conocimientos actuales / Cystic fibrosis liver disease in children A review of our current understanding
Arch. argent. pediatr
; 121(4): e202202905, ago. 2023. ilus
Article
in English, Spanish
| LILACS, BINACIS
| ID: biblio-1442708
Responsible library:
AR94.1
RESUMEN
La fibrosis quística, la segunda enfermedad genética más frecuente, es el resultado de una proteína de canal mutada, la CFTR, que secreta iones de cloro que fluidifican las secreciones. La esperanza de vida en los pacientes ha aumentado en años recientes gracias a mejoras en el tratamiento. No obstante, las complicaciones hepáticas son la tercera causa de muerte y la comprensión de su fisiopatología es aún deficiente. Se considera que la obstrucción biliar secundaria a la presencia de secreciones espesas conduce a la cirrosis. Sin embargo, el ácido ursodesoxicólico no ha modificado la historia natural. Además, la presencia de hipertensión portal en ausencia de cirrosis no puede ser explicada. Se ha propuesto el rol de la CFTR como modulador de tolerancia inmune, que explica la presencia de una inflamación portal persistente que culmina en fibrosis. El eje intestino-hígado tendría un rol importante en la presentación y la progresión de esta enfermedad
ABSTRACT
Cystic fibrosis is the second most common genetic disease in infancy. It is the result of a mutated channel protein, the CFTR, which secretes chloride ions, fluidifying secretions. Recent improvements in the treatment have increased life expectancy in these patients. Nevertheless, liver involvement remains the third cause of death. Unfortunately, our understating of the physiopathology is still deficient. Biliary obstruction secondary to the presence of thick secretions is considered to lead to cirrhosis. However, treatment with ursodeoxycolic acid has not changed the natural history. Furthermore, the presence of portal hypertension in the absence of cirrhosis cannot be explained. Recently, the role of CFTR as modulator of immune tolerance has been proposed, which could explain the presence of a persistent portal inflammation leading to fibrosis, and the gut-liver axis would also have a role in disease presentation and progression.
Full text:
Available
Collection:
International databases
Health context:
SDG3 - Health and Well-Being
/
SDG3 - Target 3.4 Reduce premature mortality due to noncommunicable diseases
/
SDG3 - Target 3.2 Reduce avoidable death in newborns and children under 5
Health problem:
Target 3.2: Reduce avoidable death in newborns and children under 5
/
Cirrhosis
/
Digestive System Diseases
/
Endocrine System Diseases
/
Other Respiratory Diseases
/
Environmental Health
/
Neonatal Healthcare
Database:
BINACIS
/
LILACS
Main subject:
Cystic Fibrosis
/
Liver Diseases
Limits:
Humans
Language:
English
/
Spanish
Journal:
Arch. argent. pediatr
Journal subject:
Pediatrics
Year:
2023
Document type:
Article
Affiliation country:
Canada
Institution/Affiliation country:
Centre Hospitalier Universitaire Mère-enfant Sainte-Justine de Montréal/CA
/
Centre Hospitalier Universitaire Mère-enfant SainteJustine de Montréal/CA