Sections

Pediatric Primary Sclerosing Cholangitis

Sections Pediatric Primary Sclerosing Cholangitis
Overview
Presentation
DDx
Workup
Treatment
Medication
Media Gallery
References

Treatment

Approach Considerations

No pharmacologic therapy has been proven effective for primary sclerosing cholangitis (PSC). The goals of treatment of PSC are to slow and potentially reverse the disease process, but treatment may ultimately involve managing disease progression and the associated complications. Drug therapy is aimed at treating symptoms and managing complications. Orthotopic liver transplantation (OLT) has been proven successful in treating children with primary sclerosing cholangitis. Liver transplantation is the only therapy that can alter the eventual outcome.

Surgical drainage procedures (eg, portoenterostomy, choledochoenterostomy) are insignificant in the management of PSC. These procedures may provide palliation but do not alter the natural history of the disease because of the consistent involvement of the intrahepatic biliary tree. Surgical drainage procedures are associated with an increased risk of cholangitis postoperatively, and subsequent liver transplantation may become technically more difficult.

Dominant strictures of the extrahepatic biliary tree, most often at the bifurcation of the hepatic ducts, are major problems for patients with PSC. Transhepatic or endoscopic balloon dilatation of strictures has been shown to be useful in children with PSC. Short-term stenting of strictures has also demonstrated clinical improvement of symptomatic strictures.

Surgical, endoscopic, and interventional radiologic procedures to relieve symptomatic dominant strictures have been demonstrated to prolong survival time of the native liver in patients with PSC, but none of these interventions have altered the ultimate rate of progression of PSC to end-stage liver disease.

Bacterial cholangitis, which can occur spontaneously, is more common after endoscopic or surgical manipulation of the biliary tree. Episodes of cholangitis require prompt antibiotic therapy.

Pharmacologic Therapy

Pharmacologic treatment of patients with primary sclerosing cholangitis (PSC) should be directed at managing the following:^[44]

Cirrhosis and portal hypertension
Chronic cholestasis with pruritus and malabsorption
Ductular complications, such as dominant strictures, cholelithiasis, and ascending bacterial cholangitis
Other associated diseases, such as inflammatory bowel disease (IBD) or other autoimmune diseases

Choleretic therapy with ursodeoxycholic acid (UDCA) reportedly improves symptoms and biochemical abnormalities in adult patients with PSC. Some observational and controlled trial data also suggest a reduction in colonic dysplasia rates with UDCA.^[45]Children with PSC experience significant improvements in their liver biochemical indices when treated with UDCA; however, the long-term effect of UDCA on the clinical outcome of PSC has not been determined.^{[46, 47, 48]}

The efficacy of immunosuppressive agents such as glucocorticoids, methotrexate, cyclosporine, and tacrolimus has been studied in adults but has not demonstrated long-term benefit, specifically with regards to histology.^{[49, 50, 51, 52, 53, 54]}The potential therapeutic effect of antibiotics has also been studied in children with primary sclerosing cholangitis. Observational studies using oral vancomycin demonstrated improved symptoms and liver function tests but no consistent improvement in liver histology.^[55]Likewise, adult studies with oral vancomycin or metronidazole have been shown to decrease select liver biochemistries,^[56]but long-term follow-up studies are needed to assess the efficacy of antibiotic therapy.

Liver Transplantation

Orthotopic liver transplantation (OLT) has been proven successful in treating children with primary sclerosing cholangitis (PSC). Data from numerous liver transplantation centers demonstrate excellent long-term patient and graft survival for patients with end-stage primary sclerosing cholangitis.^{[30, 31, 32, 57, 58, 59, 60]}

Actual patient survival rates after OLT for primary sclerosing cholangitis have been shown to be greater than 90% at 1 year and approximately 90% at 5 years. In one study, although children with primary sclerosing cholangitis had patient-survival rates and graft-survival rates equaling those of age-matched children who underwent transplantation for other indications, patients with coexisting IBD demonstrated poorer outcomes. Overall, posttransplant recurrence was noted in 9.8%, and compared with other graft recipients, patients with primary sclerosing cholangitis had higher mean liver enzyme levels 5 years posttransplant.^[29]

Diet

As with other cholestatic disorders, provide fat-soluble vitamin supplementation (vitamins A, D, E, K) and nutritional support to ensure adequate growth. If the patient has steatorrhea, medium-chain triglycerides should be added to the diet. If pancreatic enzyme deficiency is present, oral supplementation is necessary, and calcium supplementation for bone disease may also be needed.

Consultations

Despite progress in early recognition, optimal treatment of patients with primary sclerosing cholangitis remains a challenge and requires a multidisciplinary approach between hepatologists, endoscopists, surgeons, and interventional radiologists. Consultation with a gastroenterologist is necessary, and an endocrinologist may be consulted for management of bone disease.

Medication

Roberts EA. Primary sclerosing cholangitis in children. J Gastroenterol Hepatol. 1999 Jun. 14(6):588-93. [Medline].
Ludwig J. Small-duct primary sclerosing cholangitis. Semin Liver Dis. 1991. 11:11-17. [Medline].
Rodriguez HJ Bss NM. Primary Sclerosing Cholangitis. Semin Gastrointest Dis. 2003. 14:189-98. [Medline].
MacFaul GR, Chapman RW. Sclerosing cholangitis. Curr opin gastroenterol. 2004. 20:275-990. [Medline].
Hyams J., Markowitz J., Treem W., et al. Characterization of hepatic abnormalities in children with inflammatory bowel disease. Inflamm Bowel Dis. 1995. 1:27-33. [Medline].
Aron JH, Bowlus CL. The immunobiology of primary sclerosing cholangitis. Semin Immunopathol. 2009 May 26. [Medline].
Aoki CA, Bowlus CL, Gershwin ME. The immunobiology of primary sclerosing cholangitis. Autoimmun Rev. 2005 Mar. 4(3):137-43. [Medline].
Narayanan Menon KV, Wiesner RH. Etiology and natural history of primary sclerosing cholangitis. J Hepatobiliary Pancreat Surg. 1999. 6(4):343-51. [Medline].
Girard M, Franchi-Abella S, Lacaille F, Debray D. Specificities of sclerosing cholangitis in childhood. Clinics and Research in Hepatology and Gastroenteorlogy. 2012. 36:530-35. [Medline].
Worthington J, Cullen S, Chapman R. Immunopathogenesis of primary sclerosing cholangitis. Clin Rev Allergy Immunol. 2005. 28:93-103. [Medline].
Pall H, Zielenski J, Jonas MM, DaSilva DA, Potvin KM, Yuan XW, et al. Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function. J Pediatr. 2007 Sep. 151(3):255-9. [Medline].
Feld JJ, Heathcote EJ. Epidemiology of autoimmune liver disease. J Gastroenterol Hepatol. 2003 Oct. 18(10):1118-28. [Medline].
Feldstein AE, Perrault J, El-Youssif M, et al. Primary sclerosing cholangitis in children: a long-term follow-up study. Hepatology. 2003 Jul. 38(1):210-7. [Medline].
Sisto A, Feldman P, Garel L, et al. Primary sclerosing cholangitis in children: study of five cases and review of the literature. Pediatrics. 1987. 80:918–23. [Medline].
el-Shabrawi M, Wilkinson ML, Portmann B, et al. Primary sclerosing cholangitis in childhood. Gastroenterology. 1987. 92:1226-35. [Medline].
Debray D, Pariente D, Urvoas E, et al. Sclerosing cholangitis in children. J Pediatr. 1994. 124:49-56. [Medline].
Wilschanski M, Chait P, Wade JA, et al. Primary sclerosing cholangitis in 32 children: clinical, laboratory, and radiographic features, with survival analysis. Hepatology. 1995. 22:1415-22. [Medline].
Batres LA, Russo P, Mathews M, et al. Primary sclerosing cholangitis in children: a histologic follow-up study. Pediatr Dev Pathol. 2005. 8:568-76. [Medline].
Mendes FD, Lindor KD. Primary sclerosing cholangitis. Clin Liver Dis. 2004. 8:195-211. [Medline].
Ludwig J, Barham SS, LaRusso NF, Elveback LR, Wiesner RH, McCall JT. Morphologic features of chronic hepatitis associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hepatology. 1981 Nov-Dec. 1(6):632-40. [Medline].
Kim WR, Therneau TM, Wiesner RH, et al. A revised natural history model for primary sclerosing cholangitis. Mayo Clin Proc. 2000 Jul. 75(7):688-94. [Medline].
Shetty K, Rybicki L, Carey WD. The Child-Pugh classification as a prognostic indicator for survival in primary sclerosing cholangitis. Hepatology. 1997 May. 25(5):1049-53. [Medline].
The Revised Natural History Model for Primary Sclerosing Cholangitis. Mayo Foundation for Medical Education and Research. Available at http://www.mayoclinic.org/gi-rst/mayomodel3.html. Accessed: March 30, 2012.
Kim WR, Poterucha JJ, Wiesner RH, et al. The relative role of the Child-Pugh classification and the Mayo natural history model in the assessment of survival in patients with primary sclerosing cholangitis. Hepatology. 1999 Jun. 29(6):1643-8. [Medline].
Bjornsson E, Angulo P. Cholangiocarcinoma in young indivuas with and without primary sclerosing cholangitis. Am J Gastroenterol. 2007. 102:1677-1682. [Medline].
Deneau M, Adler DG, Schwartz JJ, et al. Cholangiocarcinoma in a 17-year-old boy with primary sclerosing cholangitis and inflammatory bowel disease. J Pediatr Gastroenteorl Nutr. 2011. 52:617-620. [Medline].
Liu R, Cox K, Guthery SL, Book L, Witt B, Chadwick B, et al. Cholangiocarcinoma and High-Grade Dysplasia in Young Patients with Primary Sclerosing Cholangitis. Dig Dis Sci. 2014 Apr 19. [Epub ahead of print]:[Medline].
Miloh T, Arnon R, Shneider B, suchy F, Kerkar N. A retrospective Single-Center Review of Primary Sclerosing Cholangitis in Children. Clinical Gatroenterology and Hepatolog. 2009. 7:239-45. [Medline].
Miloh T, Anand R, Yin W, Vos M, Kerkar N, Alonso E. Pediatric liver transplantation for primary sclerosing cholangitis. Liver Transpl. 2011 Aug. 17(8):925-933. [Medline]. [Full Text].
Venkat VL, Ranganathan S, Mazariegos GV, Sun Q, Sindhi R. Recurrence of Primary Sclerosing Cholangitis in Pediatric Liver Transplant Recipients. Liver Transplantation. 2014 Jun. 20(6):679-86. [Medline].
Abu-Elmagd KM, Balan V. Recurrent primary sclerosing cholangitis: from an academic illusion to a clinical reality. Liver Transpl. 2005. 11:1326-8. [Medline].
Graziadei IW. Recurrence of primary sclerosing cholangitis after liver transplantation. Liver Transpl. 2002. 8:575-81. [Medline].
Shneider BL. Diagnostic and therapeutic challenges in pediatric primary sclerosing cholangitis. Liver Transpl. 2012 Mar. 18(3):277-81. [Medline].
Bangarulingam SY, Gossard AA, Petersen BT, Ott BJ, Lindor KD. Complications of endoscopic retrograde cholangiopancreatography in primary sclerosing cholangitis. Am J Gastroenterol. 2009 Apr. 104(4):855-60. [Medline].
Angulo P, Pearce DH, Johnson CD, et al. Magnetic resonance cholangiography in patients with biliary disease: its role in primary sclerosing cholangitis. J Hepatol. 2000 Oct. 33(4):520-7. [Medline].
Revelon G, Rashid A, Kawamoto S, Bluemke DA. Primary sclerosing cholangitis: MR imaging findings with pathologic correlation. AJR Am J Roentgenol. 1999 Oct. 173(4):1037-42. [Medline].
Fulcher AS, Turner MA, Franklin KJ, et al. Primary sclerosing cholangitis: evaluation with MR cholangiography—a case–control study. Radiology. 2000. 215:71-80. [Medline].
Ferrara C, Valeri G, Salvolini L, Giovagnoni A. Magnetic resonance cholangiopancreatography in primary sclerosing cholangitis in children. Pediatr Radiol. 2002. 32:413-17. [Medline].
Rustemovic N, Cukovic-Cavka S, Opacic M, Petrovecki M, Hrstic I, Radic D, et al. Endoscopic ultrasound elastography as a method for screening the patients with suspected primary sclerosing cholangitis. Eur J Gastroenterol Hepatol. 2009 Jun 2. [Medline].
Craig DA, MacCarty RL, Wiesner RH, et al. Primary sclerosing cholangitis: value of cholangiography in determining the prognosis. AJR Am J Roentgenol. 1991. 157:959-64. [Medline].
Olsson RG, Asztely MS. Prognostic value of cholangiography in primary sclerosing cholangitis. Eur J Gastroenterol Hepatol. 1995. 7:251-4. [Medline].
Ponsioen CY, Lam K, van Milligen de Wit AW, et al. Four years experience with short term stenting in primary sclerosing cholangitis. Am J Gastroenterol. 1999 Sep. 94(9):2403-7. [Medline].
Erickson NI, Balistereri WF. Sclerosing Cholangitis. S.R. Suchy FJ, Balistreri WF. Liver disease in children. 3. Cambridge University Press; 2007. 459-77.
Björnsson E. Management of primary sclerosing cholangitis. Minerva Gastroenterol Dietol. 2009 Jun. 55(2):163-72. [Medline].
Shi J, Li Z, Zeng X, Lin Y, Xie WF. Ursodeoxycholic acid in primary sclerosing cholangitis: meta-analysis of randomized controlled trials. Hepatol Res. 2009 May 7. [Medline].
Gilger MA, Gann ME, Opekun AR, Gleason WA Jr. Efficacy of ursodeoxycholic acid in the treatment of primary sclerosing cholangitis in children. J Pediatr Gastroenterol Nutr. 2000 Aug. 31(2):136-41. [Medline].
Pardi DS, Loftus EV, Kremers WK, et al. Ursodeoxycholic acid as a chemopreventive agent in patients with ulcerativecolitis and primary sclerosing cholangitis. Gastroenterology. 2003 Apr. 124(4):889-93. [Medline].
Tung BY, Emond MJ, Haggitt RC, et al. Ursodiol use is associated with lower prevalence of colonic neoplasia in patients with ulcerative colitis and primary sclerosing cholangitis. Ann Intern Med. 2001 Jan 16. 134(2):89-95. [Medline]. [Full Text].
Kochhar R, Goenka MK, Das K, Nagi B, Bhasin DK, Chawla YK, et al. Primary sclerosing cholangitis: an experience from India. J Gastroenterol Hepatol. 1996. 11(5):429. [Medline].
Angulo P, Batts KP, Jorgensen RA, LaRusso NA, Lindor KD. Oral budesonide in the treatment of primary sclerosing cholangitis. Am J Gastroenterol. 2000. 95(9):2333. [Medline].
Kyokane K, Ichihara T, Horisawa M, Suzuki N, Ichihara S, Suga S, et al. Successful treatment of primary sclerosing cholangitis with cyclosporine and corticosteroid. Hepatogastroenterology. 1994. 41(5):449. [Medline].
Van Thiel DH, Carroll P, Abu-Elmagd K, Rodriguez-Rilo H, Irish W, McMichael J, et al. Tacrolimus (FK 506), a treatment for primary sclerosing cholangitis: results of an open-label preliminary trial. Am J Gastroenterol. 1995. 90(3):455. [Medline].
Knox TA, Kaplan MM. Treatment of primary sclerosing cholangitis with oral methotrexate. Am J Gastroenterol. 1991. 86(5):546. [Medline].
Knox TA, Kaplan MM. A double-blind controlled trial of oral-pulse methotrexate therapy in the treatment of primary sclerosing cholangitis. Gastroenterology. 1994. 106(2):494. [Medline].
Davies YK, Cox KM, Abdullah BA, Safta A, Terry AB, Cox KL. Long-term treatment of primary sclerosing cholangitis in children with oral vancomycin: an immunomodulating antibiotic. J Pediatr Gastroenterol Nutr. 2008. 47(1):61. [Medline].
Tabibian JH, Weeding E, Jorgensen RA, Petz JL, Keach JC, Talwalkar JA, et al. Randomised clinical trial: vancomycin or metronidazole in patients with primary sclerosing cholangitis - a pilot study. Aliment Pharmacol Ther. March 2013. 37(6):604-12. [Medline].
Gow PJ, Chapman RW. Liver transplantation for primary sclerosing cholangitis. Liver. 2000 Apr. 20(2):97-103. [Medline].
Graziadei IW, Wiesner RH, Marotta PJ, et al. Long-term results of patients undergoing liver transplantation for primary sclerosing cholangitis. Hepatology. 1999 Nov. 30(5):1121-7. [Medline].
Liden H, Norrby J, Friman S, Olausson M. Liver transplantation for primary sclerosing cholangitis--a single- center experience. Transpl Int. 2000. 13 Suppl 1:S162-4. [Medline].
Sekido H, Takeda K, Morioka D, et al. Liver transplantation for primary sclerosing cholangitis. J Hepatobiliary Pancreat Surg. 1999. 6(4):373-6. [Medline].
Shimizu M, Iwasaki H, Mase S, Yachie A. Successful treatment of primary sclerosing cholangitis with a steroid and a probiotic. Case Rep Gastroenterol. 2012 May. 6(2):249-53. [Medline]. [Full Text].
Abdo AA, Bain VG, Kichian K, Lee SS. Evolution of autoimmune hepatitis to primary sclerosing cholangitis: A sequentialsyndrome. Hepatology. 2002 Dec. 36(6):1393-9. [Medline].
Angulo P, Larson DR, Therneau TM, et al. Time course of histological progression in primary sclerosing cholangitis. Am J Gastroenterol. 1999 Nov. 94(11):3310-3. [Medline].
Bambha K, Kim WR, Talwalkar J, et al. Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitisin a United States community. Gastroenterology. 2003 Nov. 125(5):1364-9. [Medline].
Bergquist A, Glaumann H, Persson B, Broome U. Risk factors and clinical presentation of hepatobiliary carcinoma in patients with primary sclerosing cholangitis: a case-control study. Hepatology. 1998 Feb. 27(2):311-6. [Medline].
Bharucha AE, Jorgensen R, Lichtman SN, et al. A pilot study of pentoxifylline for the treatment of primary sclerosing cholangitis. Am J Gastroenterol. 2000 Sep. 95(9):2338-42. [Medline].
Boberg KM, Lundin KE, Schrumpf E. Etiology and pathogenesis in primary sclerosing cholangitis. Scand J Gastroenterol Suppl. 1994. 204:47-58. [Medline].
Chalasani N, Baluyut A, Ismail A, et al. Cholangiocarcinoma in patients with primary sclerosing cholangitis: a multicenter case-control study. Hepatology. 2000 Jan. 31(1):7-11. [Medline].
Floreani A, Rizzotto ER, Ferrara F, Carderi I, Caroli D, Blasone L. Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. Am J Gastroenterol. 2005 Jul. 100(7):1516-22. [Medline].
Harrison PM. Diagnosis of primary sclerosing cholangitis. J Hepatobiliary Pancreat Surg. 1999. 6(4):356-60. [Medline].
Kita R, Kita-Sasai Y, Hanaoka I, et al. Beneficial effect of bezafibrate on primary sclerosing cholangitis (three casereports). Am J Gastroenterol. 2002 Jul. 97(7):1849-51. [Medline].
Kugelmas M, Spiegelman P, Osgood MJ, et al. Different immunosuppressive regimens and recurrence of primary sclerosing cholangitisafter liver transplantation. Liver Transpl. 2003 Jul. 9(7):727-32. [Medline].
Lee YM, Kaplan MM. Medical treatment of primary sclerosing cholangitis. J Hepatobiliary Pancreat Surg. 1999. 6(4):361-5. [Medline].
[Guideline] Mahadevan U, Kane S. American gastroenterological association institute medical position statement on the use of gastrointestinal medications in pregnancy. Gastroenterology. 2006 Jul. 131(1):278-82. [Medline].
[Guideline] Murray KF, Carithers RL Jr. AASLD practice guidelines: Evaluation of the patient for liver transplantation. Hepatology. 2005 Jun. 41(6):1407-32. [Medline].
Nakanuma Y, Harada K, Katayanagi K, et al. Definition and pathology of primary sclerosing cholangitis. J Hepatobiliary Pancreat Surg. 1999. 6(4):333-42. [Medline].
Olsson R, Boberg KM, de Muckadell OS, et al. High-dose ursodeoxycholic acid in primary sclerosing cholangitis: a 5-year multicenter, randomized, controlled study. Gastroenterology. 2005. 129:1464-72. [Medline].
Raj V, Lichtenstein DR. Hepatobiliary manifestations of inflammatory bowel disease. Gastroenterol Clin North Am. 1999 Jun. 28(2):491-513. [Medline].
Roozendaal C, de Jong MA, van den Berg AP, et al. Clinical significance of anti-neutrophil cytoplasmic antibodies (ANCA) in autoimmune liver diseases. J Hepatol. 2000 May. 32(5):734-41. [Medline].
Saarinen S, Olerup O, Broome U. Increased frequency of autoimmune diseases in patients with primary sclerosing cholangitis. Am J Gastroenterol. 2000 Nov. 95(11):3195-9. [Medline].
Shetty K, Rybicki L, Brzezinski A, et al. The risk for cancer or dysplasia in ulcerative colitis patients with primary sclerosing cholangitis. Am J Gastroenterol. 1999 Jun. 94(6):1643-9. [Medline].
Talwalkar JA, Lindor KD. Primary sclerosing cholangitis. Inflamm Bowel Dis. 2005 Jan. 11(1):62-72. [Medline].

Media Gallery

Fibro-obliterative cholangiopathy. Image courtesy of Dr. Kay Washington.
Endoscopic retrograde cholangiopancreatography performed in a patient with abnormal liver function test results shows multiple intrahepatic bile duct strictures and beading.
Double-contrast barium enema (same patient as in the previous image) shows filiform polyps and an ahaustral colon resulting from ulcerative colitis.
Percutaneous transhepatic cholangiogram shows dilatation, stricturing, and beading of the intrahepatic bile ducts. Note the surgical clips from a previous cholecystectomy.
T-tube cholangiogram shows irregularity of the common bile duct, stricturing, beading, and dilatation of the intrahepatic bile ducts. Note a calculus in the termination of the left hepatic duct (arrow).
Magnetic resonance cholangiopancreatography shows a normal-sized common bile duct, but strictures of both the left and right ducts are noted as well as a dilated proximal left hepatic duct.
Technetium-99m iminodiacetic acid scan shows retention of the radionuclide proximal to strictures in the distribution of the left hepatic duct. Note the lack of filling of the gallbladder because of a previous cholecystectomy. Isotope has entered the small bowel.

of 7

Author

Henry C Lin, MD Assistant Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Division of Gastroenterology, Hepatology, and Nutrition, Children’s Hospital of Philadelphia

Henry C Lin, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Study of Liver Diseases, American Medical Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Pennsylvania Medical Society, Philadelphia County Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Hisham Nazer, MBBCh, FRCP, DTM&H Professor of Pediatrics, Consultant in Pediatric Gastroenterology, Hepatology and Clinical Nutrition, University of Jordan Faculty of Medicine, Jordan

Hisham Nazer, MBBCh, FRCP, DTM&H is a member of the following medical societies: American Association for Physician Leadership, Royal College of Paediatrics and Child Health, Royal College of Surgeons in Ireland, Royal Society of Tropical Medicine and Hygiene, Royal College of Physicians and Surgeons of the United Kingdom

Disclosure: Nothing to disclose.

David A Piccoli, MD Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine

David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching. for: Abbott Nutritional, Abbvie, speakers' bureau.

Acknowledgements

Robert Baldassano, MD Director, Center for Pediatric Inflammatory Bowel Disease, Children's Hospital of Philadelphia; Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, University of Pennsylvania School of Medicine

Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Sections Pediatric Primary Sclerosing Cholangitis
Overview
Presentation
DDx
Workup
Treatment
Medication
Media Gallery
References

Pediatric Primary Sclerosing Cholangitis Treatment & Management

Approach Considerations

Pharmacologic Therapy

Liver Transplantation

Diet

Consultations

About

Membership

Apps

WebMD Network

Editions

Pediatric Primary Sclerosing Cholangitis Treatment & Management

Approach Considerations

Pharmacologic Therapy

Liver Transplantation

Diet

Consultations

References

Tables

Contributor Information and Disclosures

What would you like to print?

Find Us On

About

Membership

Apps

WebMD Network

Editions