PERSISTENT HEPATOCELLULAR SECRETORY FAILURE: A RARITY IN HEPATOLOGY
DOI:
https://doi.org/10.55519/JAMC-S4-13240Keywords:
Obstructive Jaundice, Hyperbilirubinemia, ERCPAbstract
Persistent hepatocellular secretory failure (PHSF) is a rare condition that presents as elevated bilirubin despite treatment and elimination of all possible causes, including intrahepatic and extrahepatic biliary obstruction. Very limited literature is available regarding this condition, highlighting its rarity. A 65 years old male who was diagnosed with symptoms of cholestasis. He was diagnosed with pancreatic adenocarcinoma leading to obstructive jaundice. He underwent endoscopic retrograde cholangiopancreaticography and self-expandable metallic stent (SEMS) placement in the common bile duct. Despite SEMS placement, he complained of persistent jaundice, pale stools and itching. His bilirubin was persistently elevated and imaging showed patent stent. After a thorough work-up and discussion in a multidisciplinary team meeting, he was diagnosed as a case of PHSF. The patient is being managed conservatively with ursodeoxycholic acid and cholestyramine. His liver function tests improved after addition of rifampin.
References
1. van Dijk R, Kremer AE, Smit W, van den Elzen B, van Gulik T, Gouma D, et al. Characterization and treatment of persistent hepatocellular secretory failure. Liver Int 2015;35(4):1478–88.
2. Shi M, Sheng L, Lian M, Miao Q, Wang Q, Xiao X, et al. Efficacy and safety of rifampicin in patients with persistent hepatocellular secretory failure. J Gastroenterol Hepatol 2021;36(11):3233–8.
3. Zollner G, Trauner M. Nuclear receptors as therapeutic targets in cholestatic liver diseases. Br J Pharmacol 2009;156(1):7–27.
4. Bachs L, Parés A, Elena M, Piera C, Rodés J. Comparison of rifampicin with phenobarbitone for treatment of pruritus in biliary cirrhosis. Lancet 1989;1(8638):574–6.
5. Cançado ELR, Cubero Leitão RM, Carrilho FJ, Laudanna AA. Unexpected Clinical Remission of Cholestasis After Rifampicin Therapy in Patients With Normal or Slightly Increased Levels of Γ-Glutamyl Transpeptidase. Am Coll Gastroenterol 1998;93(9):1510–7.
6. Yerushalmi B, Sokol RJ, Narkewicz MR, Smith D, Karrer FM. Use of Rifampin for Severe Pruritus in Children with Chronic Cholestasis. J Pediatr Gastroenterol Nutr 1999;29(4):442–7.
7. Stiehl A, Thaler MM, Admirand WH. The effects of phenobarbital on bile salts and bilirubin in patients with intrahepatic and extrahepatic cholestasis. N Engl J Med 1972;286(16):858–61.
8. Bloomer JR, Boyer JL. Phenobarbital effects in cholestatic liver diseases. Ann Intern Med 1975;82(3):310–7.
9. Ozturk K, Demirci H, Sakin YS, Uygun A, Bagci S. Efficiency of rifampicin in emergency treatment of severe hyperbilirubinemia : report of two cases and review of literature. Acta Gastroenterol Belg 2015;78(2):256–8.
10. Ellis E, Wagner M, Lammert F, Nemeth A, Gumhold J, Strassburg CP, et al. Successful treatment of severe unconjugated hyperbilirubinemia via induction of UGT1A1 by rifampicin. J Hepatol 2006;44(1):243–5.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2024 Faisal Ali, Muhammad Abdurrahman Butt, Ibrahim Ahmed, Bassam Rao, Mohammad Salih
This work is licensed under a Creative Commons Attribution-NoDerivatives 4.0 International License.
Journal of Ayub Medical College, Abbottabad is an OPEN ACCESS JOURNAL which means that all content is FREELY available without charge to all users whether registered with the journal or not. The work published by J Ayub Med Coll Abbottabad is licensed and distributed under the creative commons License CC BY ND Attribution-NoDerivs. Material printed in this journal is OPEN to access, and are FREE for use in academic and research work with proper citation. J Ayub Med Coll Abbottabad accepts only original material for publication with the understanding that except for abstracts, no part of the data has been published or will be submitted for publication elsewhere before appearing in J Ayub Med Coll Abbottabad. The Editorial Board of J Ayub Med Coll Abbottabad makes every effort to ensure the accuracy and authenticity of material printed in J Ayub Med Coll Abbottabad. However, conclusions and statements expressed are views of the authors and do not reflect the opinion/policy of J Ayub Med Coll Abbottabad or the Editorial Board.
USERS are allowed to read, download, copy, distribute, print, search, or link to the full texts of the articles, or use them for any other lawful purpose, without asking prior permission from the publisher or the author. This is in accordance with the BOAI definition of open access.
AUTHORS retain the rights of free downloading/unlimited e-print of full text and sharing/disseminating the article without any restriction, by any means including twitter, scholarly collaboration networks such as ResearchGate, Academia.eu, and social media sites such as Twitter, LinkedIn, Google Scholar and any other professional or academic networking site.
