Jaundice of intrahepatic cholestatic origin
Ictericia de origen colestásico intrahepatico
Abstract
Jaundice is a clinical sign that in all age groups, indicates the presence of a pathology either in the hepatobiliary sphere or not. It is observed when serum bilirubin levels increase, in conjugated or unconjugated form. The medical history allows a fairly accurate evaluation of jaundice and includes the performance of laboratory tests in which total and fractionated bilirubin, hematology, alanine transaminase - ALT, aspartate transaminase - AST, alkaline phosphatase, γ-glutamyltransferase, time of prothrombin and / or international. normalized ratio, serum proteogram (1-3). Imaging studies can identify pathologies in the liver parenchyma and / or the bile ducts. The unconjugated or indirect hyperbilirubinemia raises suspicion in hemolytic processes, in Gilbert's syndrome and conjugated or direct hyperbilirubinemia, in hepatocellular damage, such as viral hepatitis, due to Alcohol Use Disorder (AUD), cholestatic disorders, such as choledocholithiasis and neoplasms in the confluent biliopancreatic. Cholestasis is an acute or chronic hepatobiliary disorder, in which the synthesis, secretion or flow of bile is altered. Cholestasis can affect up to 10-20% of the general population.1 When the etiology is benign, which represents the highest percentage of cases, it is not associated with high morbidity and mortality, when it is prolonged and severe, it can progress Hepatic fibrosis, cirrhosis, hepatocellular carcinoma or cholangiocarcinoma and death can occur, all depending on the etiology as will be explained later.
Downloads
References
United Network for Organ Sharing - UNOS [Internet]. 2018. Available from: https://optn.transplant.hrsa.gov/data/view-data-reports/national-data.
Vuppalanchi R, Liangpunsakul S, Chalasani N. Etiology of new-onset jaundice: how often is it caused by idiosyncratic drug-induced liver injury in the United States? Am J Gastroenterol. 2007;102(3):558-562.
Lopez Velazquez, Jorge Alan. Bilirrubina, una vieja amiga con una nueva historia. Rev Invest Med Sur Mex. 2012; 19 (4): 228-234
Levitt DG, Levitt MD. Quantitative assessment of the multiple processes responsible for bilirubin homeostasis in health and disease. Clin Exp Gastroenterol. 2014; 7:307-328.
Cui Y, Konig J, Leier I, Buchholz U, Keppler D. Hepatic uptake of bilirubin and its conjugates by the human organic anion transporter SLC21A6. J Biol Chem 2001; 276: 9626-30.
Erlinger S, Arias IM, Dhumeaux D. Inherited disorders of bilirubin transport and conjugation: new insights into molecular mechanisms and consequences. Gastroenterology. 2014;146(7):1625-1638.
Roy Chowdhury N, Arias IM, Wolkoff AW, Roy Chowdhury J. Disorders of bilirubin metabolism. In: Arias IM, Jakoby WB, Schachter D, Shafritz DA. The liver: biology and pathobiology. 3rd Ed. New York: Raven Press; 2001.
Fevery J, Blanckaert N, Heirwegh KPM, Preaux AM, Berhelot P. Unconjugated bilirubin and an increased proportion of bilirubin monoconjugates in the bile patients with Gilbert‘s syndrome and Crigler Najjar disease. J Clin Invest 1977; 60: 970-9.
Gallagher PG. Abnormalities of the erythrocyte membrane. Pediatr Clin North Am. 2013;60(6):1349-1362.
Vuppalanchi R, Liangpunsakul S, Chalasani N. Etiology of new-onset jaundice: how often is it caused by idiosyncratic drug-induced liver injury in the United States? Am J Gastroenterol. 2007;102(3):558-562.
Roche SP, Kobos R. Jaundice in the adult patient. Am Fam Physician. 2004;69(2):299-304.
Winger J, Michelfelder A. Diagnostic approach to the patient with jaundice. Prim Care. 2011;38(3):469-482
Hirschfield GM. Genetic determinants of cholestasis. Clin Liver Dis 2013; 17:147-159
Korolnek T, Hamza I. Macrophages and iron trafficking at the birth and death of red cells. Blood. 2015;125(19):2893-2897.
Jalan-Sakrikar N, De Assuncao TM, Lu J, Almada LL, Lomberk G, Fernandez-Zapico ME, Urrutia R, Huebert RC. Hedgehog. Signaling Overcomes an EZH2-Dependent Epigenetic Barrier to Promote Cholangiocyte Expansion. PLoS One 2016; 11: e0168266 [PMID: 27936185 DOI: 10.1371/journal.pone.0168266]
Khungar V, Goldberg DS. Liver Transplantation for Cholestatic Liver Diseases in Adults. Clin Liver Dis 2016; 20: 191-203 [PMID: 26593299 DOI: 10.1016/j.cld.2015.08.011]
Onofrio, Fernanda Q. and Hirschfield, Gideon M. The Pathophysiology of Cholestasis and Its Relevance to Clinical Practice. Clinical Liver Disease. 2020; 15 (3): 110-114
American College of Radiology. ACR Appropriateness Criteria. Jaundice. 2012. https://acsearch.acr.org/list. Accessed Novemer 11, 2016.
European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Management of cholestatic liver diseases. Journal of Hepatology 51 (2009): 237-267
Heathcote, E. Jenny. Diagnosis and Management of Cholestatic Liver Disease. Clinical Gastroenterology and Hepatology 2007; 5:776 –782
Morales Laura, Vélez Natalia, Munoz M. Octavio Germán. Hepatotoxicidad: patrón colestásico inducido por fármacos. Rev Col Gastroenterol. 2016; 31 (1): 36-47
Yokoda, Raquel T. y Rodriguez Eduardo A. Pathogenesis of cholestatic liver diseases. World J Hepatol. 2020; August 27; 12(8): 423-435
Lopez Panqueva, Rocio del Pilar. Approaches to Pathological Diagnosis of Cholestatic Diseases. Rev Col Gastroenterol. 2014; 29 (2): 183-192
Li MK, Crawford JM. The pathology of cholestasis. Semin Liver Dis. 2004;24(1):21-42.
Lazaridis KN, LaRusso NF. The Cholangiopathies. Mayo Clin Proc 2015; 90: 791-800 [PMID: 25957621 DOI: 10.1016/j.mayocp.2015.03.017]
Hung OL, Kwon NS, Cole AE, et al. Evaluation of the physician’s ability to recognize the presence or absence of anemia, fever, and jaundice. Acad Emerg Med. 2000;7(2):146-156.
Nguyen KD, Sundaram V, Ayoub WS. Atypical causes of cholestasis. World J Gastroenterol 2014; 20:9418-9426.
Hirschfield GM, Heathcote J, Gershwin ME. Pathogenesis of cholestatic liver disease and therapeutic approaches. Gastroenterology 2010; 139:1481-1496.
European Association for the Study of the Liver. EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. J Hepatol 2017; 67:145-172.
Beuers U, Trauner M, Jansen P, et al. New paradigms in the treatment of hepatic cholestasis: from UDCA to FXR, PXR and beyond. J Hepatol 2015;62(suppl 1):S25-S37.
Jansen PLM. New therapies target the toxic consequences of cholestatic liver disease. Expert Rev Gastroenterol Hepatol 2018; 12:277-285.
Vinod S Hegade, A Stuart FW KendrickB and David EJ Jones. Drug treatment of pruritus in liver diseases. Clinical Medicine 2015; 15 (4): 351–7
Kremer AE, Oude Elferink RP, Beuers U. Pathophysiology and current management of pruritus in liver disease. Clin Res Hepatol Gastroenterol 2011; 35:89–97.
Jones DE. Pathogenesis of cholestatic itch: old questions, new answers, and future opportunities. Hepatology 2012; 56:1194–6