Can genetic testing guide the therapy of cholestatic pruritus? A case of benign recurrent intrahepatic cholestasis type 2 with severe nasobiliary drainage-refractory itch

Holz R, Kremer A, Luetjohann D, Wasmuth HE, Lammert F, Krawczyk M (2018)

Publication Type: Journal article

Publication year: 2018

Journal

Hepatology Communications Wiley

Book Volume: 2

Pages Range: 152-154

Journal Issue: 2

DOI: 10.1002/hep4.1144

Abstract

Benign recurrent intrahepatic cholestasis (BRIC) is a peculiar familial disease caused by mutations of the genes encoding hepatocanalicular flippase for phosphatidylserine (ATP8B1; BRIC type 1) or the bile salt export pump (ABCB11; BRIC type 2). Here, we report on a patient with nasobiliary drainage-refractory BRIC type 2 who improved under plasma separation and anion absorption therapy. We also suggest that nasobiliary drainage might be an ineffective approach in carriers of severe loss-of-function mutations of the bile salt export pump ABCB11. (Hepatology Communications 2018;2:152-154).

Authors with CRIS profile

Andreas Kremer Department of Medicine 1 – Gastroenterology, Pneumology and Endocrinology

Involved external institutions

Universität des Saarlandes (UdS) DE Germany (DE) Rheinische Friedrich-Wilhelms-Universität Bonn DE Germany (DE) Luisenhospital Aachen DE Germany (DE)

How to cite

APA:

Holz, R., Kremer, A., Luetjohann, D., Wasmuth, H.E., Lammert, F., & Krawczyk, M. (2018). Can genetic testing guide the therapy of cholestatic pruritus? A case of benign recurrent intrahepatic cholestasis type 2 with severe nasobiliary drainage-refractory itch. Hepatology Communications, 2(2), 152-154. https://doi.org/10.1002/hep4.1144

MLA:

Holz, Robert, et al. "Can genetic testing guide the therapy of cholestatic pruritus? A case of benign recurrent intrahepatic cholestasis type 2 with severe nasobiliary drainage-refractory itch." Hepatology Communications 2.2 (2018): 152-154.

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