Updates in Pediatric Cholestasis

Harpavat S, et al. Diagnostic yield of newborn screening for biliary atresia using direct or conjugated bilirubin measurements. JAMA. 2020;323(12):1141–50.

Gotze T, et al. Neonatal cholestasis - differential diagnoses, current diagnostic procedures, and treatment. Front Pediatr. 2015;3:43.

Hoofnagle JH. Chapter 40 - LiverTox: A website on drug-induced liver injury. In: Kaplowitz N, DeLeve LD, editors. Drug-induced liver disease (third edition). Boston: Academic Press; 2013. p. 725–32.

Ushigome Y, et al. Short- and long-term outcomes of 34 patients with drug-induced hypersensitivity syndrome in a single institution. J Am Acad Dermatol. 2013;68(5):721–8.

Kardaun SH, et al. Drug reaction with eosinophilia and systemic symptoms (DRESS): an original multisystem adverse drug reaction. Results from the prospective RegiSCAR study. Br J Dermatol. 2013;169(5):1071–80.

Gura KM, et al. Fish oil emulsion reduces liver injury and liver transplantation in children with intestinal failure-associated liver disease: a multicenter integrated study. J Pediatr. 2021;230:46-54 e2.

Heubi JE, Bove KE, Setchell KDR. Oral cholic acid is efficacious and well tolerated in patients with bile acid synthesis and Zellweger spectrum disorders. J Pediatr Gastroenterol Nutr. 2017;65(3):321–6.

Chinsky JM, Singh R, Ficicioglu C, van Karnebeek CDM, Grompe M, Mitchell G, Waisbren SE, Gucsavas-Calikoglu M, Wasserstein MP, Coakley K, Scott CR. Diagnosis and treatment of tyrosinemia type I: a US and Canadian consensus group review and recommendations. Genet Med. 2017;19(12):1380–95. https://doi.org/10.1038/gim.2017.101.

Welling L, et al. International clinical guideline for the management of classical galactosemia: diagnosis, treatment, and follow-up. J Inherit Metab Dis. 2017;40(2):171–6.

Taylor SA, et al. The effects of gestational alloimmune liver disease on fetal and infant morbidity and mortality. J Pediatr. 2018;196:123-128 e1.

Kriegermeier A, Green R. Pediatric cholestatic liver disease: review of bile acid metabolism and discussion of current and emerging therapies. Front Med (Lausanne). 2020;7:149.

Paumgartner G, Beuers U. Ursodeoxycholic acid in cholestatic liver disease: mechanisms of action and therapeutic use revisited. Hepatology. 2002;36(3):525–31.

Gregorio GV, et al. Effect of rifampicin in the treatment of pruritus in hepatic cholestasis. Arch Dis Child. 1993;69(1):141–3.

Jones EA, Bergasa NV. The pruritus of cholestasis and the opioid system. JAMA. 1992;268(23):3359–62.

Thornton JR, Losowsky MS. Plasma leucine enkephalin is increased in liver disease. Gut. 1989;30(10):1392–5.

Thebaut A, et al. Sertraline as an additional treatment for cholestatic pruritus in children. J Pediatr Gastroenterol Nutr. 2017;64(3):431–5.

Mayo MJ, et al. Sertraline as a first-line treatment for cholestatic pruritus. Hepatology. 2007;45(3):666–74.

Baumann U, et al. Effects of odevixibat on pruritus and bile acids in children with cholestatic liver disease: Phase 2 study. Clin Res Hepatol Gastroenterol. 2021;45(5): 101751.

Gonzales E, et al. Efficacy and safety of maralixibat treatment in patients with Alagille syndrome and cholestatic pruritus (ICONIC): a randomised phase 2 study. Lancet. 2021;398(10311):1581–92.

Sundaram SS, et al. Health related quality of life in patients with biliary atresia surviving with their native liver. J Pediatr. 2013;163(4):1052-7 e2.

Squires JE, et al. Neurodevelopmental outcomes in preschool and school aged children with biliary atresia and their native liver. J Pediatr Gastroenterol Nutr. 2020;70(1):79–86.