Abstract：Objective To compare the clinical features of children with different clinical forms of congenital hepatic fibrosis (CHF), and provides a description of the characteristics of childhood CHF. Methods Sixty children with CHF between January 2002 and June 2015 were enrolled, including 26 children with portal hypertensive CHF (PH CHF), 3 children with cholangitic CHF, 30 children with combined portal hypertensive and cholangitic CHF (mixed CHF), and 1 child with latent forms of CHF. The medical data of 26 children with PH CHF and 30 children with mixed CHF, including gender, age, clinical manifestations, physical signs, laboratory tests and imaging characteristics, were retrospectively studied. Results Fever, jaundice and hepatomegaly were more frequently noted in children with mixed CHF than in those with PH CHF (PPPConclusions PH and mixed CHF are common forms in childhood CHF. The children with the two forms of PH usually manifest portal hypertension such as cirrhosis and hepatosplenomegaly. The liver damage may be common in children with mixed CHF.
WU Xin,DU Xiao-Rang,DING Jin-Fang et al. Clinical features of different clinical forms of childhood congenital hepatic fibrosis[J]. CJCP, 2016, 18(4): 335-339.
Karpen SJ. Update on the etiologies and management of neonatal cholestasis[J]. Clin Perinatol, 2002, 29(1): 159-180.
[12]
Woolbright BL, Jaeschke H. Novel insight into mechanisms of cholestatic liver injury[J]. World J Gastroenterol, 2012, 18(36): 4985-4993.
[13]
Allen K, Jaeschke H, Copple BL. Bile acids induce inflammatory genes in hepatocytes: a novel mechanism of inflammation during obstructive cholestasis[J]. Am J Pathol, 2011, 178(1): 175-186.
[14]
Jaeschke H. Reactive oxygen and mechanisms of inflammatory liver injury: Present concepts[J]. J Gastroenterol Hepatol, 2011, 26 Suppl 1: 173-179.
[15]
Laschke MW, Dold S, Menger MD, et al. Platelet-dependent accumulation of leukocytes in sinusoids mediates hepatocellular damage in bile duct ligation-induced cholestasis[J]. Br J Pharmacol, 2008, 153(1): 148-156.
[16]
Sullivan BP, Wang R, Tawfik O, et al. Protective and damaging effects of platelets in acute cholestatic liver injury revealed by depletion and inhibition strategies[J]. Toxicol Sci, 2010, 115(1): 286-294.
[17]
Park Y, Schoene N, Harris W. Mean platelet volume as an indicator of platelet activation: methodological issues[J]. Platelets, 2002, 13(5-6): 301-306.
[18]
Vieira-de-Abreu A, Campbell RA, Weyrich AS, et al. Platelets: versatile effector cells in hemostasis, inflammation, and the immune continuum[J]. Semin Immunopathol, 2012, 34(1): 5-30.
[19]
Poupon R. Liver alkaline phosphatase: a missing link between choleresis and biliary inflammation[J]. Hepatology, 2015, 61(6): 2080-2090.
[20]
Kuntz E, Kuntz HD. Cholestasis[M]//Kuntz E, Kuntz HD. Hepatology principles and practice. 2nd. Wetzlar: Springer Berlin Heidelberg, 2006: 227-242.
[21]
Lazaridis K N, Strazzabosco M, Larusso N F. The cholangiopathies: disorders of biliary epithelia[J]. Gastroenterology, 2004, 127(5): 1565-1577.
