Cholestasis: Multimedia

References

1. Poddar U, Thapa BR, Das A, Bhattacharya A, Rao KN, Singh K. Neonatal cholestasis: differentiation of biliary atresia from neonatal hepatitis in a developing country. Acta Paediatr. May 13 2009;[Medline].

2. Strople J, Lovell G, Heubi J. Prevalence of Subclinical Vitamin K Deficiency in Cholestatic Liver Disease. J Pediatr Gastroenterol Nutr. Jun 3 2009;[Medline].

3. 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. Jun 11 2009;[Medline].

4. Mathias A, Wax JR, Pinette MG, Cartin A, Blackstone J. Progressive familial intrahepatic cholestasis complicating pregnancy. J Matern Fetal Neonatal Med. Jun 1 2009;1-3. [Medline].

5. Shneider BL. Liver transplantation for progressive familial intrahepatic cholestasis: the evolving role of genotyping. Liver Transpl. Jun 2009;15(6):565-6. [Medline].

6. Brough AJ, Bernstein J. Conjugated hyperbilirubinemia in early infancy. A reassessment of liver biopsy. Hum Pathol. Sep 1974;5(5):507-16. [Medline].

7. [Guideline] Moyer V, Freese DK, Whitington PF, et al. Guideline for the evaluation of cholestatic jaundice in infants: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr. Aug 2004;39(2):115-28. [Medline]. [Full Text].

8. [Guideline] Murray KF, Carithers RL Jr. AASLD practice guidelines: Evaluation of the patient for liver transplantation. Hepatology. Jun 2005;41(6):1407-32. [Medline]. [Full Text].

9. Balistreri WF. Neonatal cholestasis. J Pediatr. Feb 1985;106(2):171-84. [Medline].

10. Elferink RP, Groen AK. The mechanism of biliary lipid secretion and its defects. Gastroenterol Clin North Am. Mar 1999;28(1):59-74, vi. [Medline].

11. Jones EA, Bergasa NV. The pruritus of cholestasis and the opioid system. JAMA. Dec 16 1992;268(23):3359-62. [Medline].

12. Kaufman SS, Murray ND, Wood RP. Nutritional support for the infant with extrahepatic biliary atresia. J Pediatr. May 1987;110(5):679-86. [Medline].

13. Oude Elferink RP, Paulusma CC, Groen AK. Hepatocanalicular transport defects: pathophysiologic mechanisms of rare diseases. Gastroenterology. Mar 2006;130(3):908-25. [Medline].

14. Sabesin SM. Cholestatic lipoproteins--their pathogenesis and significance. Gastroenterology. Sep 1982;83(3):704-9. [Medline].

15. Suchy FJ. Approach to the infant with cholestasis. In: Liver Disease in Children. 1st ed. St. Louis, Mo:. Mosby;1994:349-355.

16. Trauner M, Meier PJ, Boyer JL. Molecular pathogenesis of cholestasis. N Engl J Med. Oct 22 1998;339(17):1217-27. [Medline].

17. Whitington PF. Chronic cholestasis of infancy. Pediatr Clin North Am. Feb 1996;43(1):1-26. [Medline].

18. Whitington PF, Whitington GL. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology. Jul 1988;95(1):130-6. [Medline].

Further Reading

• Relevant clinical guidelines include the following:
  • Guideline for the evaluation of cholestatic jaundice in infants: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition ⁷
  • American Association for the Study of Liver Diseases practice guidelines: evaluation of the patient for liver transplantation ⁸
• Relevant clinical trials include the following:
  • Ursodiol for Treating Parenteral Nutrition Associated Cholestasis in Neonates (URSONEONAT)
  • Evaluating the Genetic Causes and Progression of Cholestatic Liver Diseases
  • Efficacy of an Omega-3 Enriched Intravenous Fat Emulsion in the Prevention of Parenteral Nutrition Induced Injury in Infants
• Related eMedicine topics include the following:
  • Progressive Familial Intrahepatic Cholestasis
  • Primary Sclerosing Cholangitis (Gastroenterology)
  • Primary Sclerosing Cholangitis (Pediatrics: General Medicine)
  • Biliary Atresia
  • Biliary Obstruction

Keywords

cholestasis, reduced bile formation, reduced bile flow, bile duct obstruction, cholestatic disorders, Dubin-Johnson syndrome, Rotor syndrome, Alagille syndrome, hyperchloremia, gram-negative sepsis, heart failure, metabolic disease, pruritus, itching, scratching, hyperlipidemia, xanthomas, xanthoma, failure to thrive, cholestatic liver disease, vitamin E deficiency, vitamin D deficiency, osteomalacia, rickets, biliary atresia, steatorrhea, scleral icterus, cutaneous jaundice, choledochal cyst, choledochal cyst, cholelithiasis, primary sclerosing cholangitis, hepatitis A, hepatitis B, hepatitis C, progressive familial intrahepatic cholestasis, treatment, diagnosis

Contributor Information and Disclosures

Author

Karan M Emerick, MD, Consulting Staff, Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Connecticut Children's Medical Center
Karan M Emerick, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Study of Liver Diseases, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Medical Editor

Jayant Deodhar, MD, Associate Professor in Pediatrics, BJ Medical College, India; Honorary Consultant, Departments of Pediatrics and Neonatology, King Edward Memorial Hospital, India
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

CME Editor

Paul D Petry, DO, FACOP, FAAP, Consulting Staff, Freeman Pediatric Care, Freeman Health System
Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association
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, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.