Expanding etiology of progressive familial intrahepatic cholestasis

doi:10.4254/wjh.v11.i5.450

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World Journal of Hepatology

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1948-5182

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Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA

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World J Hepatol. May 27, 2019; 11(5): 450-463
Published online May 27, 2019. doi: 10.4254/wjh.v11.i5.450

Table 2 Adult manifestations of progressive familial intrahepatic cholestasis gene mutations

Etiology	Genetic defect	Manifestations
FIC1 deficiency	ATP8B1	BRIC1
		ICP1 and contraceptive-induced cholestasis
		Cryptogenic cirrhosis
BSEP deficiency	ABCB11	BRIC2
		ICP2 and contraceptive-induced cholestasis
		DILI
		Cryptogenic cirrhosis
MDR3 deficiency	ABCB4	ICP3 and contraceptive-induced cholestasis
		Drug induced cholestasis
		Low phospholipid-associated cholestasis
		Cholesterol gallstone disease
		Biliary fibrosis or liver cirrhosis without cholestasis
		Cryptogenic cirrhosis
TJP2 deficiency	TJP2	Cryptogenic cirrhosis
FXR	NR1H4	ICP
FXR	NR1H4	Drug induced cholestasis associated with propylthiouracil

BRIC: Benign recurrent intrahepatic cholestasis; BSEP: Bile salt export pump; ICP: Intrahepatic cholestasis of pregnancy; DILI: Drug induce liver injury.

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Citation: Henkel SA, Squires JH, Ayers M, Ganoza A, Mckiernan P, Squires JE. Expanding etiology of progressive familial intrahepatic cholestasis. World J Hepatol 2019; 11(5): 450-463
URL: https://www.wjgnet.com/1948-5182/full/v11/i5/450.htm
DOI: https://dx.doi.org/10.4254/wjh.v11.i5.450

Henkel SA, Squires JH, Ayers M, Ganoza A, Mckiernan P, Squires JE. Expanding etiology of progressive familial intrahepatic cholestasis. World J Hepatol 2019; 11(5): 450-463 [PMID: 31183005 DOI: 10.4254/wjh.v11.i5.450]

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