全部 标题 作者
关键词 摘要

OALib Journal期刊
ISSN: 2333-9721
费用:99美元

查看量下载量

相关文章

更多...

Successful Treatment of Recurrent Primary Sclerosing Cholangitis after Orthotopic Liver Transplantation with Oral Vancomycin

DOI: 10.1155/2013/314292

Full-Text   Cite this paper   Add to My Lib

Abstract:

Primary sclerosing cholangitis (PSC) is a progressive, cholestatic disease of the liver that is marked by inflammation of the bile ducts and damage to the hepatic biliary tree. Approximately 60–70% of patients also have inflammatory bowel disease and progression of PSC can lead to ulcerative colitis and cirrhosis of the liver. Due to limited understanding of the etiology and mechanism of PSC, the only existing treatment option is orthotopic liver transplantation (OLT); however, recurrence of PSC, after OLT is estimated to be between 5% and 35%. We discuss the successful treatment of a pediatric patient, with recurrent PSC, after OLT with oral Vancomycin. 1. Introduction Primary sclerosing cholangitis (PSC) is a progressive inflammatory disease, of unknown etiology and with significant morbidity and mortality, which damages the intra- and/or extrahepatic biliary tree leading to portal hypertension and cirrhosis of the liver. The clinical course is variable including hepatobiliary carcinoma, especially cholangiocarcinoma in 6–20% of patients [1–6]. Additionally, it is estimated that 60–70% of people with PSC have inflammatory bowel disease [7, 8]. Orthotopic liver transplantation (OLT) is the only treatment option for patients with end-stage liver disease due to the progressive damage caused from PSC [9, 10]. The recurrence of PSC in the new graft is estimated to be between 5–35% [11–13]. We report the successful treatment of a patient after OLT, who had shown recurrence of disease, with oral Vancomycin. 2. Case Report A 12-year-old girl presented with a three-week history of jaundice and lethargy, with periumbilical pain every other day. There had been no recent travel or sick contacts. Screening tests revealed platelet level was low (89?K/uL; normal range 150 to 400). Antinuclear Antibody (ANCA) was positive with a homogeneous pattern, and Antinuclear Antibody Titer was elevated (640; normal range <40). Serum copper was normal at 1065?ug/L and ceruloplasmin was normal at 24?mg/dL. γ-Glutamyl transpeptidase (GGT) level was elevated (139?U/L; reference range from 5 to 36). C-Reactive Protein (CRP) was elevated at 2.80?mg/dL; reference range was from 0.0 to 0.5. PTT was elevated at 48.6?sec; normal range was from 23.3 to 33.8. Prothrombin Time was elevated at 21.0?sec; normal range was from 11.8 to 14.2. INR was increased to 1.9?sec; normal range was from 0.9 to 1.1. Erythrocyte sedimentation rate (ESR) was elevated at 107?mm/hr; bilirubin was elevated at 3.5?mg/dL, and ammonia was elevated at 55?umol/L. Patient underwent an open liver biopsy, which

References

[1]  A. Wee, J. Ludwig, and R. J. Coffey, “Hepatobiliary carcinoma associated with primary sclerosing cholangitis and chronic ulcerative colitis,” Human Pathology, vol. 16, no. 7, pp. 719–726, 1985.
[2]  C. B. Rosen, D. M. Nagorney, R. H. Wiesner, R. J. Coffey, and N. F. LaRusso, “Cholangiocarcinoma complicating primary sclerosing cholangitis,” Annals of Surgery, vol. 213, no. 1, pp. 21–25, 2001.
[3]  J. M. Farrant, K. M. Hayllar, M. L. Wilkinson et al., “Natural history and prognostic variables in primary sclerosing cholangitis,” Gastroenterology, vol. 100, no. 6, pp. 1710–1717, 1991.
[4]  U. Broomé, R. Olsson, L. L??f et al., “Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis,” Gut, vol. 38, no. 4, pp. 610–615, 1996.
[5]  C. Y. Ponsioen, S. M. E. Vrouenraets, W. Prawirodirdjo et al., “Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population,” Gut, vol. 51, no. 4, pp. 562–566, 2002.
[6]  J. J. W. Tischendorf, P. N. Meier, C. Stabburg, et al., “Characterization and clinical course of hepatobiliary carcinoma in patients with primary sclerosing cholangitis,” Scandinavian Journal of Gastroenterology, vol. 41, pp. 1227–1234, 2000.
[7]  J. J. W. Tischendorf, H. Hecker, M. Kruger, M. P. Manns, and P. N. Meier, “Characterization, outcome and prognosis in 273 patients with primary sclerosing cholangitis,” The New England Journal of Medicine, vol. 310, pp. 899–903, 1984.
[8]  R. H. Wiesner, P. M. Grambsch, E. R. Dickson et al., “Primary sclerosing cholangitis: natural history, prognostic factors and survival analysis,” Hepatology, vol. 10, no. 4, pp. 430–436, 1989.
[9]  R. Olsson, A. Danielsson, G. Jarnerot et al., “Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis,” Gastroenterology, vol. 100, no. 5, pp. 1319–1323, 1991.
[10]  K. M. Abu-Elmagd, M. Malinchoc, E. R. Dickson et al., “Efficacy of hepatic transplantation in patients with primary sclerosing cholangitis,” Surgery Gynecology and Obstetrics, vol. 177, no. 4, pp. 335–344, 1993.
[11]  K. M. Abu-Elmagd, R. Selby, S. Iwatsuki et al., “Cholangiocarcinoma and sclerosing cholangitis: clinical characteristics and effect on survival after liver transplantation,” Transplantation Proceedings, vol. 25, no. 1, pp. 1124–1125, 1993.
[12]  B. Nashan, H. J. Schlitt, G. Tusch et al., “Biliary malignancies in primary sclerosing cholangitis: timing for liver transplantation,” Hepatology, vol. 23, no. 5, pp. 1105–1111, 1996.
[13]  B. Brandsaeter, H. Isoneimi, U. Broome, et al., “Liver transplantation for primary sclerosing cholangitis, predictors and consequences of hepatobiliary malignancy,” Journal of Hepatology, vol. 40, pp. 815–822, 2004.
[14]  R. Chapman, J. Fevery, A. Kalloo et al., “Diagnosis and management of primary sclerosing cholangitis,” Hepatology, vol. 51, no. 2, pp. 660–678, 2010.
[15]  N. L. Nikolaidis, O. I. Giouleme, K. A. Tziomalos et al., “Small-duct primary sclerosing cholangitis: a single-center seven-year experience,” Digestive Diseases and Sciences, vol. 50, no. 2, pp. 324–326, 2005.
[16]  A. E. Feldstein, J. Perrault, M. El-Youssif, K. D. Lindor, D. K. Freese, and P. Angulo, “Primary sclerosing cholangitis in children: a long-term follow-up study,” Hepatology, vol. 38, no. 1, pp. 210–217, 2003.
[17]  G. Mieli-Vergani and D. Vergani, “Unique features of primary sclerosing cholangitis in children,” Current Opinion in Gastroenterology, vol. 26, no. 3, pp. 265–268, 2010.
[18]  K. L. Cox and K. M. Cox, “Oral vancomycin: treatment of primary sclerosing cholangitis in children with inflammatory bowel disease,” Journal of Pediatric Gastroenterology and Nutrition, vol. 27, no. 5, pp. 580–583, 1998.
[19]  Y. K. Davies, K. M. Cox, B. A. Abdullah, A. Safta, A. B. Terry, and K. L. Cox, “Long-term treatment of primary sclerosing cholangitis in children with oral vancomycin: an immunomodulating antibiotic,” Journal of Pediatric Gastroenterology and Nutrition, vol. 47, no. 1, pp. 61–67, 2008.
[20]  M. Siedlar, A. Szczepanik, J. Wi?ckiewicz, A. Pituch-Noworolska, and M. Zembala, “Vancomycin down-regulates lipopolysaccharide-induced tumour necrosis factor alpha (TNFα) production and TNFα-mRNA accumulation in human blood monocytes,” Immunopharmacology, vol. 35, no. 3, pp. 265–271, 1997.
[21]  G. Di Febo, C. Calabrese, and F. Matassoni, “New trends in non-absorbable antibiotics in gastrointestinal disease,” Italian Journal of Gastroenterology, vol. 24, no. 9, pp. 10–13, 1992.

Full-Text

comments powered by Disqus

Contact Us

service@oalib.com

QQ:3279437679

WhatsApp +8615387084133