Top

12-03-2025 | Cholangitis | Original Article

A comprehensive clinical and microbiological study on the diagnosis and management of cholangitis in patients with biliary atresia undergoing kasai portoenterostomy

Authors: Merna Adel Aziz, Hala Mohsen Abdullatif, May S. Soliman, Sawsan Okasha, Nevian Nabil, Mariam Mahmoud Balah, Hanaa El-Karaksy

Published in: Indian Journal of Gastroenterology

Abstract

Background and Objectives

Kasai-portoenterostomy (KPE) is the initial attempt to restore the bile flow and salvage the native liver in biliary atresia (BA) patients. Cholangitis is a frequent complication after KPE and adequate treatment impacts the long-term outcome. The aim of our study is to assess the severity of cholangitis episodes in a cohort of BA patients post KPE, identify the causative agents, using several diagnostic methods, as well as to assess the tolerability and efficacy of our antimicrobial protocol.

Methods

This analytical retrospective observational study, conducted at Pediatric Hepatology Unit, Cairo University Pediatric Hospital, included infants and children with cholangitis post-KPE enrolled over 30 months. Clinical data collection, basic laboratory investigations inflammatory markers, B-D glucan, blood culture, 16SrDNA, 18SrDNA were performed in all enrolled patients. Cholangitis episodes were treated with intravenous antibiotics according to our antimicrobial protocol that has been implemented in conjunction with the antimicrobial stewardship committee.

Results

This study included 30 post-KPE patients, who experienced 47 episodes of cholangitis. Twenty-five episodes of cholangitis were culture positive cholangitis (positive blood culture and/or PCR results and/or liver biopsy). Klebsiella Variicola and Klebsiella pneumoniae were the most prevalent pathogens in 13 and seven cultures, respectively. Meropenem was the most successful antibiotic in the eradication of infection in 11(23.4%) episodes. Culture positive cholangitis showed increased incidence of sepsis and worse outcome in comparison to culture negative cholangitis. The severity of cholangitis was classified into 16 patients (34%) with infection, 28 (60%) sepsis, one (2%) severe sepsis and two (4%) septic shock.

Conclusion

Almost half of cholangitis episodes were culture-positive; the commonest pathogen was Klebsiella, showing more severe sepsis and worse outcome.

Graphical abstract

Fawaz R, Baumann U, Ekong U, et al. Guideline for the evaluation of cholestatic jaundice in infants: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2017;64:154–68. https://doi.org/10.1097/MPG.0000000000001334.CrossRefPubMed

Davenport M, Madadi-Sanjani O, Chardot C, Verkade HJ, Karpen SJ, Petersen C. Surgical and medical aspects of the initial treatment of biliary atresia: Position Paper. J Clin Med. 2022;11:6601. https://doi.org/10.3390/jcm11216601. CrossRefPubMedPubMedCentral

Gunadi, Gunawan TA, Widiyanto G, Yuanita A, Mulyani NS, Makhmudi A. Liver transplant score for prediction of biliary atresia patients’ survival following Kasai procedure. BMC Res Notes. 2018;11:381. https://doi.org/10.1186/s13104-018-3498-z.CrossRefPubMedPubMedCentral

Calinescu AM, Madadi-Sanjani O, Mack C, et al. Cholangitis: definition and treatment after Kasai hepatoportoenterostomy for biliary atresia: A delphi process and international expert panel. J Clin Med. 2022;11:494. https://doi.org/10.3390/jcm11030494.CrossRefPubMedPubMedCentral

Koga H, Wada M, Nakamura H, et al. Factors influencing jaundice-free survival with the native liver in post-portoenterostomy biliary atresia patients: results from a single institution. J Pediatr Surg. 2013;48:2368–72. https://doi.org/10.1016/j.jpedsurg.2013.08.007.CrossRefPubMed

Liu J, Dong R, Chen G, Dong K, Zheng S. Risk factors and prognostic effects of cholangitis after Kasai procedure in biliary atresia patients: A retrospective clinical study. J Pediatr Surg. 2019;54:2559–64. https://doi.org/10.1016/j.jpedsurg.2019.08.026.CrossRefPubMed

Meng L, Liu J, Wang J, et al. Characteristics of the gut microbiome and IL-13/TGF-β1 mediated fibrosis in post-kasai cholangitis of biliary atresia. Front Pediatr. 2021;8:751204. https://doi.org/10.3389/fped.2021.751204.CrossRef

Kumar R, Lal BB, Sood V, et al. Predictors of successful Kasai portoenterostomy and survival with native liver at 2 years in infants with biliary atresia. J Clin Exp Hepatol. 2019;9:453–9. https://doi.org/10.1016/j.jceh.2018.09.008.CrossRefPubMed

Chen SY, Lin CC, Tsan YT, et al. Number of cholangitis episodes as a prognostic marker to predict timing of liver transplantation in biliary atresia patients after Kasai portoenterostomy. BMC Pediatr. 2018;18:119. https://doi.org/10.1186/s12887-018-1074-2.CrossRefPubMedPubMedCentral

10.

Goldstein B, Giroir B, Randolph A. International Consensus Conference on Pediatric Sepsis International pediatric sepsis consensus conference definitions for sepsis and organ dysfunction in pediatrics. Pediatr Crit Care Med. 2005;6:2–8. https://doi.org/10.1097/01.PCC.0000149131.72248.E6.CrossRefPubMed

11.

Meisner M. Procalcitonin: biochemistry and clinical diagnosis. Dresden (Germany): UNI-MED-Verlag; 2010.

12.

White SK, Schmidt RL, Walker BS, Hanson KE. (1→ 3)‐β‐D‐glucan testing for the detection of invasive fungal infections in immunocompromised or critically ill people. Cochrane Database Syst Rev. 2020;7:CD009833 https://doi.org/10.1002/14651858.CD009833.pub2

13.

Murray PR. Manual of Clinical Microbiology. 12th ed. Washington, DC: American Society for Microbiology; 2019.

14.

Goldenberger D, Schmidheini T, Altwegg M. Detection of Bartonella henselae and Bartonella quintana by a simple and rapid procedure using broad-range PCR amplification and direct single-strand sequencing of part of the 16S rRNA gene. Clin Microbiol Infect. 1997;3:240–5. https://doi.org/10.1111/j.1469-0691.1997.tb00604.x.CrossRefPubMed

15.

White, T.J. Amplification and direct sequencing of fungal ribosomal RNA genes for phylogenetics. In: PCR Protocols, a Guide to Methods and Applications. 1990:315–22. https://doi.org/10.1016/B978-0-12-372180-8.50042-1

16.

Mogahed EA, Mansy YA, Al Hawi Y, El-Sayed R, El-Raziky MS, El-Karaksy H. Blind percutaneous liver biopsy in infants and children: Comparison of safety and efficacy of percussion technique and ultrasound assisted technique. Arab J Gastroenterol. 2016;17:168.CrossRefPubMed

17.

Hegarty JE, Williams R. Liver biopsy: techniques, clinical applications, and complications. Br Med J (Clin Res Ed). 1984;288:1254–6.CrossRefPubMed

18.

Chung PH, Tam PK, Wong KK. Does the identity of the bacteria matter in post-Kasai cholangitis? A comparison between simple and intractable cholangitis. J Pediatr Surg. 2018;53:2409–11. https://doi.org/10.1016/j.jpedsurg.2018.08.028.CrossRefPubMed

19.

Luo Q, Hao F, Zhang M, Guo C. Serum bacterial DNA detection in patients with cholangitis after Kasai procedure. Pediatr Int. 2015;57:954–60. https://doi.org/10.1111/ped.12737.CrossRefPubMed

20.

Baek SH, Kang JM, Ihn K, Han SJ, Koh H, Ahn JG. The epidemiology and etiology of cholangitis after Kasai portoenterostomy in patients with biliary atresia. J Pediatr Gastroenterol Nutr. 2020;70:171–7. https://doi.org/10.1097/MPG.0000000000002555.CrossRefPubMed

21.

Gad EH, Kamel Y, Salem TA, Ali MA, Sallam AN. Short- and long-term outcomes after Kasai operation for type III biliary atresia: Twenty years of experience in a single tertiary Egyptian center-A retrospective cohort study. Ann Med Surg (Lond). 2021;62:302–14. https://doi.org/10.1016/j.amsu.2021.01.052.CrossRef

22.

Ortega M, Marco F, Soriano A, et al. Epidemiology and prognostic determinants of bacteraemic biliary tract infection. J Antimicrob Chemother. 2012;67:1508–13. https://doi.org/10.1093/jac/dks062.CrossRef

23.

Gomi H, Takada T, Hwang TL, et al. Updated comprehensive epidemiology, microbiology, and outcomes among patients with acute cholangitis. J Hepatobiliary Pancreat Sci. 2017;24:310–8. https://doi.org/10.1002/jhbp.452.CrossRefPubMed

24.

Clancy CJ, Nguyen MH. Finding the “missing 50%” of invasive candidiasis: how non-culture diagnostics will improve understanding of disease spectrum and transform patient care. Clin Infect Dis. 2013;56:1284–92. https://doi.org/10.1093/cid/cit006 CrossRefPubMed

25.

Zweitzig DR, Riccardello NM, Morrison J, et al. Measurement of microbial DNA polymerase activity enables detection and growth monitoring of microbes from clinical blood cultures. PLoS One. 2013;8:e78488.https://doi.org/10.1016/j.jpedsurg.2004.08.027.CrossRef

26.

Frances R, Munoz C, Zapater P, et al. Bacterial DNA activates cell mediated immune response and nitric oxide overproduction in peritoneal macrophages from patients with cirrhosis and ascites. Gut. 2004;53:860–4. https://doi.org/10.1136/gut.2003.027425.CrossRefPubMedPubMedCentral

27.

Sandy NS, Hessel G, Bellomo-Brandao MA. Major complications of pediatric percutaneous liver biopsy do not differ among physicians with different degrees of training. Am J Gastroenterol. 2020;115:786–9. https://doi.org/10.14309/ajg.0000000000000459.CrossRefPubMed

28.

Luo Y, Zheng S. Current concept about postoperative cholangitis in biliary atresia. World J Pediatr. 2008;4:14–9. https://doi.org/10.1007/s12519-008-0003-0.CrossRefPubMed

29.

Decharun K, Leys CM, West KW, Finnell SM. Prophylactic antibiotics for prevention of cholangitis in patients with biliary atresia status post-Kasai portoenterostomy: a systematic review. Clin Pediatr. 2016;55:66–72. https://doi.org/10.1177/0009922815594760.CrossRef

30.

Chung PH, Wong KK, Tam PK. Predictors for failure after Kasai operation. J Pediatr Surg. 2015;50:293–6. https://doi.org/10.1016/j.jpedsurg.2014.11.015. CrossRefPubMed

31.

Salomão R, Ferreira BL, Salomão MC, Santos SS, Azevedo LCP, Brunialti MKC. Sepsis: evolving concepts and challenges. BrazJ Med Biol. 2019;52:e8595. https://doi.org/10.1590/1414-431X20198595.CrossRef

32.

Wong KK, Fan AH, Lan LC, Lin SC, Tam PK. Effective antibiotic regime for postoperative acute cholangitis in biliary atresia—an evolving scene. J Pediatr Surg. 2004;39:1800–2. https://doi.org/10.1016/j.jpedsurg.2004.08.027 .CrossRefPubMed

Title: A comprehensive clinical and microbiological study on the diagnosis and management of cholangitis in patients with biliary atresia undergoing kasai portoenterostomy
Authors: Merna Adel Aziz
Hala Mohsen Abdullatif
May S. Soliman
Sawsan Okasha
Nevian Nabil
Mariam Mahmoud Balah
Hanaa El-Karaksy
Publication date: 12-03-2025
Publisher: Springer India
Keywords: Cholangitis
Antibiotic
Septicemia
Septicemia
Meropenem
Published in: Indian Journal of Gastroenterology
Print ISSN: 0254-8860
Electronic ISSN: 0975-0711
DOI: https://doi.org/10.1007/s12664-024-01721-z

Keynote webinar | Spotlight on advances in lupus

Live
Webinar | 27-05-2025 | 18:00 (CEST)

Systemic lupus erythematosus is a severe autoimmune disease that can cause damage to almost every system of the body. Join this session to learn more about novel biomarkers for diagnosis and monitoring and familiarise yourself with current and emerging targeted therapies.

Join us live: Tuesday 27th May, 18:00-19:15 (CEST)

Prof. Edward Vital

Prof. Ronald F. van Vollenhoven

Developed by: Springer Medicine

Elevate your expertise in aplastic anemia (Link opens in a new window)

Aplastic Anemia
Independent Medical Education

Transform the way you care for your patients with aplastic anemia with our 3-module series using real-world case studies and expert insights. Discover why early diagnosis matters, explore the benefits and risks of current treatments, and develop tailored approaches for complex cases.

Supported by:

Pfizer

Developed by: Springer Healthcare IME

Learn more

Keynote webinar | Spotlight on advances in lupus

Springer Medicine

Abstract

Background and Objectives

Methods

Results

Conclusion

Graphical abstract

Please log in to get access to this content

Keynote webinar | Spotlight on advances in lupus

Elevate your expertise in aplastic anemia (Link opens in a new window)