Cholestasis Treatment & Management
- Author: Hisham Nazer, MB, BCh, FRCP, , DTM&H; Chief Editor: Carmen Cuffari, MD more...
Much medical care in patients with cholestasis is disease specific.
- Cholestasis often does not respond to medical therapy of any sort. Some reports indicate success in children with chronic cholestatic diseases with the use of ursodeoxycholic acid (20-30 mg/kg/d), which acts to increase bile formation and antagonizes the effect of hydrophobic bile acids on biological membranes. Phenobarbital (5 mg/kg/d) may also be useful in some children with chronic cholestasis.
- Treatment of fat malabsorption principally involves dietary substitution. In older patients, a diet that is rich in carbohydrates and proteins can be substituted for a diet containing long-chain triglycerides. In infants, that may not be possible, and substitution of a formula containing medium-chain triglycerides may improve fat absorption and nutrition. This, however, has not clearly been proven, and therapeutic formulas containing medium-chain triglycerides may not be worth their expense. Bile salt therapy to replace missing bile salts is not practical. Ursodeoxycholic acid, which is used to treat some cholestatic conditions, does not form mixed micelles and has no effect on fat absorption.
- In chronic cholestasis, careful attention must be paid to prevent fat-soluble vitamin deficiencies, which are common complications in pediatric patients with chronic cholestasis. This is accomplished by administering fat-soluble vitamins and monitoring the response to therapy. Oral absorbable, fat-soluble vitamin formulation A, D, E, and K supplementation is safe and potentially effective in pediatric patients with cholestasis.
See the list below:
- Surgical care is disease specific; therefore, refer to articles about disease states (see Causes).
See the list below:
- Referral to a specialist in gastroenterology or hepatology is indicated for any patient with cholestatic liver disease, particularly if it is severe or prolonged.
See the list below:
- See Medical Care.
Decock S, Roelandts R, Van Steenbergen W, et al. Cholestasis-Induced Pruritus Treated with Ultraviolet B Phototherapy: An Observational Case Series Study. J Hepatol. 2012 May 18. [Medline].
Strople J, Lovell G, Heubi J. Prevalence of Subclinical Vitamin K Deficiency in Cholestatic Liver Disease. J Pediatr Gastroenterol Nutr. 2009 Jun 3. [Medline].
Hutchin T, Preece MA, Hendriksz C, et al. Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) as a cause of liver disease in infants in the UK. J Inherit Metab Dis. 2009 Jun 11. [Medline].
Mathias A, Wax JR, Pinette MG, Cartin A, Blackstone J. Progressive familial intrahepatic cholestasis complicating pregnancy. J Matern Fetal Neonatal Med. 2009 Jun 1. 1-3. [Medline].
Shneider BL. Liver transplantation for progressive familial intrahepatic cholestasis: the evolving role of genotyping. Liver Transpl. 2009 Jun. 15(6):565-6. [Medline].
Zhang X, Yu L, Ding Y. Human leukocyte antigen G and miR-148a are associated with the pathogenesis of intrahepatic cholestasis of pregnancy. Exp Ther Med. 2014 Dec. 8(6):1701-1706. [Medline]. [Full Text].
Lo JO, Shaffer BL, Allen A, Little SE, Cheng YW, Caughey AB. Intrahepatic cholestasis of pregnancy and timing of delivery. J Matern Fetal Neonatal Med. 2014 Nov 5. 1-20. [Medline].
Arnon R, Suchy F. Neonatal Cholestasis. El Zouki A, Harfi H, Nazer H, et al. Textbook of Clinical Pediatrics. 2nd edition. Heildelberg Dordrecht, London, New York: Springer-Verlag Berlin, Heidelberg; 2012. Vol 3: Chapter 205, pages 1987-2001. [Full Text].
Balistreri WF. Neonatal cholestasis. J Pediatr. 1985 Feb. 106(2):171-84. [Medline].
Brough AJ, Bernstein J. Conjugated hyperbilirubinemia in early infancy. A reassessment of liver biopsy. Hum Pathol. 1974 Sep. 5(5):507-16. [Medline].
Bunchorntavakul C, Reddy KR. Pruritus in chronic cholestatic liver disease. Clin Liver Dis. 2012 May. 16(2):331-46. [Medline].
Davis AR, Rosenthal P, Newman TB. Nontransplant surgical interventions in progressive familial intrahepatic cholestasis. J Pediatr Surg. 2009 Apr. 44(4):821-7. [Medline].
Elferink RP, Groen AK. The mechanism of biliary lipid secretion and its defects. Gastroenterol Clin North Am. 1999 Mar. 28(1):59-74, vi. [Medline].
Festi D, Montagnani M, Azzaroli F, et al. Clinical efficacy and effectiveness of ursodeoxycholic acid in cholestatic liver diseases. Curr Clin Pharmacol. 2007 May. 2(2):155-77. [Medline].
Haber B, Ferreira CT, Aw M, et al. Cholestasis: current issues and plan for the future. J Pediatr Gastroenterol Nutr. 2008 Aug. 47(2):220-4. [Medline].
Jones EA, Bergasa NV. The pruritus of cholestasis and the opioid system. JAMA. 1992 Dec 16. 268(23):3359-62. [Medline].
Kaufman SS, Murray ND, Wood RP. Nutritional support for the infant with extrahepatic biliary atresia. J Pediatr. 1987 May. 110(5):679-86. [Medline].
Nightingale S, Ng VL. Optimizing nutritional management in children with chronic liver disease. Pediatr Clin North Am. 2009 Oct. 56(5):1161-83. [Medline].
Oude Elferink RP, Paulusma CC, Groen AK. Hepatocanalicular transport defects: pathophysiologic mechanisms of rare diseases. Gastroenterology. 2006 Mar. 130(3):908-25. [Medline].
Poddar U, Thapa BR, Das A,et al. Neonatal cholestasis: differentiation of biliary atresia from neonatal hepatitis in a developing country. Acta Paediatr. 2009 May 13. [Medline].
Sabesin SM. Cholestatic lipoproteins--their pathogenesis and significance. Gastroenterology. 1982 Sep. 83(3):704-9. [Medline].
Shen YM, Wu JF, Hsu HY, et al. A Pilot Study of an Oral Absorbable Fat-Soluble Vitamin Formulation in Pediatric Patients with Cholestasis. J Pediatr Gastroenterol Nutr. 2012 May 16. [Medline].
Suchy FJ. Neonatal cholestasis. Pediatr Rev. 2004 Nov. 25(11):388-96. [Medline].
Trauner M, Meier PJ, Boyer JL. Molecular pathogenesis of cholestasis. N Engl J Med. 1998 Oct 22. 339(17):1217-27. [Medline].
Tufano M, Nicastro E, Giliberti P, Vegnente A, Raimondi F, Iorio R. Cholestasis in neonatal intensive care unit: incidence, aetiology and management. Acta Paediatr. 2009 Nov. 98(11):1756-61. [Medline].
Vajro P, Ferrante L, Paolella G. Alagille syndrome: An overview. Clin Res Hepatol Gastroenterol. 2012 Jun. 36(3):275-7. [Medline].
Whitington PF. Chronic cholestasis of infancy. Pediatr Clin North Am. 1996 Feb. 43(1):1-26. [Medline].
Whitington PF, Whitington GL. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology. 1988 Jul. 95(1):130-6. [Medline].
Park HW, Lee NM, Kim JH, Kim KS, Kim SN. Parenteral fish oil-containing lipid emulsions may reverse parenteral nutrition-associated cholestasis in neonates: a systematic review and meta-analysis. J Nutr. 2015 Feb. 145 (2):277-83. [Medline].
Lauriti G, Zani A, Aufieri R, Cananzi M, Chiesa PL, Eaton S, et al. Incidence, prevention, and treatment of parenteral nutrition-associated cholestasis and intestinal failure-associated liver disease in infants and children: a systematic review. JPEN J Parenter Enteral Nutr. 2014 Jan. 38 (1):70-85. [Medline].
Gupta K, Wang H, Amin SB. Parenteral Nutrition-Associated Cholestasis in Premature Infants: Role of Macronutrients. JPEN J Parenter Enteral Nutr. 2014 Oct 13. [Medline].