Treatment of Cholestasis in Infants and Young Children.
Curr Gastroenterol Rep
; 25(11): 344-354, 2023 Nov.
Article
en En
| MEDLINE
| ID: mdl-37651067
ABSTRACT
PURPOSE OF REVIEW Cholestasis is characterized by a conjugated hyperbilirubinemia secondary to impaired bile synthesis, transport, or excretion from the liver. It is always pathologic and can be indicative of an underlying hepatobiliary, genetic, or metabolic disorder, several of which require timely diagnosis to ensure proper management and optimal outcomes. This review provides an overview of the evaluation of cholestasis with a focus on current and emerging treatment strategies. RECENT FINDINGS:
Increased accessibility of next generation sequencing (NGS) allows for utilization of genetic testing early in the diagnostic process. This may alter the clinical algorithm for diagnosis of cholestatic disorders. An enhanced understanding of the underlying pathophysiology may help guide future development of targeted therapies, such as ileal bile acid transporter (IBAT) inhibitors. These were recently approved for treatment of cholestatic pruritus in patients with Alagille syndrome and Progressive Familial Intrahepatic Cholestasis. Current management of cholestasis is aimed at the biochemical consequences of impaired bile flow, including malnutrition, pruritus, and progressive fibrosis. NGS has led to an enhanced understanding of biliary pathology and may guide development of future treatment modalities based on specific gene mutations. Rapid discernment of the underlying etiology is essential as new treatment modalities emerge.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Colestasis
/
Colestasis Intrahepática
/
Síndrome de Alagille
Límite:
Child
/
Child, preschool
/
Humans
/
Infant
Idioma:
En
Revista:
Curr Gastroenterol Rep
Asunto de la revista:
GASTROENTEROLOGIA
Año:
2023
Tipo del documento:
Article
País de afiliación:
Estados Unidos