Pediatric Cholestatic Diseases: Common and Unique Pathogenic Mechanisms.
Annu Rev Pathol
; 19: 319-344, 2024 Jan 24.
Article
en En
| MEDLINE
| ID: mdl-38265882
ABSTRACT
Cholestasis is the predominate feature of many pediatric hepatobiliary diseases. The physiologic flow of bile requires multiple complex processes working in concert. Bile acid (BA) synthesis and excretion, the formation and flow of bile, and the enterohepatic reuptake of BAs all function to maintain the circulation of BAs, a key molecule in lipid digestion, metabolic and cellular signaling, and, as discussed in the review, a crucial mediator in the pathogenesis of cholestasis. Disruption of one or several of these steps can result in the accumulation of toxic BAs in bile ducts and hepatocytes leading to inflammation, fibrosis, and, over time, biliary and hepatic cirrhosis. Biliary atresia, progressive familial intrahepatic cholestasis, primary sclerosing cholangitis, and Alagille syndrome are four of the most common pediatric cholestatic conditions. Through understanding the commonalities and differences in these diseases, the important cellular mechanistic underpinnings of cholestasis can be greater appreciated.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Colestasis
/
Colestasis Intrahepática
Límite:
Child
/
Humans
Idioma:
En
Revista:
Annu Rev Pathol
Asunto de la revista:
PATOLOGIA
Año:
2024
Tipo del documento:
Article