Pathophysiologic and therapeutic insights into neonatal intrahepatic cholestasis caused by Citrin deficiency:fo-cusing on the canalicular transporters / 中华实用儿科临床杂志
Chinese Journal of Applied Clinical Pediatrics
; (24): 1447-1450, 2018.
Article
en Zh
| WPRIM
| ID: wpr-696616
Biblioteca responsable:
WPRO
ABSTRACT
The elevated NADH/ NAD + ratio in the plasma of the hepatocyte is a key pathophysiologic altera-tion for the development of neonatal intrahepatic cholestasis caused by Citrin deficiency (NICCD). The elevated ratio results in energy shortage in hepatocytes and impairs the function of the ATP - dependent canalicular transporters,in-cluding BSEP,MDR3,FIC1,Sterolin 1 / 2 and MRP2,leading to intrahepatic cholestasis in NICCD patients. On the other hand,the increased NADH/ NAD + ratio inhibits galactose metabolism and thus gives rise to secondary galactosemia, damaging the hepatocyte as well as extrahepatic organs including the lens of the eyes. The lactose - free and medium -chain triglyceride - enriched formulas can rapidly correct the energy shortage of the hepatocyte and alleviate secondary galactosemia,hence improving the clinical presentations effectively in NICCD patients.
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WPRIM
Idioma:
Zh
Revista:
Chinese Journal of Applied Clinical Pediatrics
Año:
2018
Tipo del documento:
Article