Рецепт. 2018; : 590-598
Доброкачественный рецидивирующий внутрипеченочный холестаз (случай из клинической практики)
Аннотация
статье представлено описание клинического случая выявления доброкачественного рецидивирующего холестаза и обзор современных литературных данных по проблеме. Несмотря на благоприятное течение данного синдрома без прогрессирования патологии печени и формирования цирроза, периоды рецидивов характеризуются наступлением кожного зуда и холестаза, затрудняющих трудоспособность пациентов (в описанном клиническом случае общая продолжительность временной нетрудоспособности пациента составила 68 дней). Рифампицин и плазмаферез облегчают симптомы у пациентов и снижают уровень билирубина. Наиболее оптимальным при неосложненной атаке доброкачественного рецидивирующего холестаза является назначение рифампицина в дозе 150–300 мг в сутки под контролем состояния пациента и печеночных показателей в биохимическом анализе крови.
Список литературы
1. Folvik G. (2012) Benign recurrent intrahepatic cholestasis: review and long-term follow-up of fi ve cases. Scand J Gastroenterol., vol. 47, pp. 482–488.
2. Van der Woerd W. (2010) Familial cholestasis: progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis and intrahepatic cholestasis of pregnancy. Best Pract Res Clin Gastroenterol., vol. 24, pp. 541–553.
3. Matthias C. (2017) Genetic determinants of cholangiopathies: molecular and systems genetics, BBA – Molecular Basis of Disease: accepted manuscript. Homburg, 36 р.
4. Luketic V.A. (1999) Benign recurrent intrahepatic cholestasis. Clin. Liver Dis., vol. 3, no 3, pp. 509–528.
5. Summerfi eld J.A. (1980) Benign recurrent intrahepatic cholestasis: studies of bilirubin kinetics, bile acids, and cholangiography. Gut, vol. 21, Issue 2, pp. 154–160.
6. Kumar P. (2016) Benign Recurrent Intrahepatic Cholestasis in a Young Adult. Journal of Clinical and Diagnostic Research, vol. 10, Issue 6, pp. 1–2.
7. Carey J.B. (1961) Relief of pruritus of jaundice with bile acid sequestering resin. JAMA, vol. 176, pp. 432–435.
8. Bloomer J.R. (1975) Phenobarbital eff ects in cholestatic liver disease. Ann Intern., vol. 82, pp. 310–317.
9. Summerfi eld J.A. (1980) Naloxone modulates the perception of itch in man. Br J Clin Pharmacol., vol. 10, no 2, pp. 180–183.
10. Shiff E.R. (ed.) (1999) Diseases of the Liver, 7 th edn. Philadelphia: Lippincott-Raven, pp. 611–631.
11. Alva J. (1965) Relief of pruritus of jaundice with methandrostenolone and speculation of the nature of pruritus in liver disease. Am J Med Sci., vol. 250, pp. 60–65.
12. Marshall H. (2005) Rifampicin and ursodeoxycholic acid in cholestatic liver disease. Gastroenterology, vol. 129, pp. 476–485.
13. Paumgartner G. (2008) Medical treatment of cholestatic liver disease. Clin Liver Dis., vol. 12, pp. 53–81.
14. Jankowska I. (2014) Ileal exclusion in children with progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr., vol. 58, pp. 92–95.
15. Duncan J. (1984) Treatment of pruritus due to chronic obstructive liver disease. Br Med J Res Ed., vol. 289, pp. 11–22.
16. Hegade V.S. (2016) The safety and effi cacy of nasobiliary drainage in the treatment of refractory cholestatic pruritus: a multicenter European study. Aliment Pharmacol Ther, vol. 43, pp. 294–302.
17. Van Dijk R. (2015) Characterization and treatment of persistent hepatocellular secretory failure. Liver Int., vol. 35, pp. 1478–1488.
18. Webb G.J. (2018) Low risk of hepatotoxicity from rifampicin when used for cholestatic pruritus: a cross-disease cohort study. Alimentary Pharmacology & Therapeutics, vol. 47, no 8, pp. 1213–1219.
19. Khurana S. (2006) Rifampin is safe for treatment of pruritus due to chronic cholestasis: a metaanalysis of prospective randomized-controlled trials. Liver International, vol. 26, pp. 943–948.
20. Bachs L. (1992) Eff ects of long-term rifampicin administration in primary biliary cirrhosis. Gastroenterology, vol. 102, no 6, p p. 2077–2088.
21. Sheuer P. (1974) Rifampicin hepatitis. Lancet, pp. 421–425.
Recipe. 2018; : 590-598
The case report: Benign recurrent intrahepatic cholestasis
Abstract
The article presents the case report of benign recurrent intrahepatic cholestasis and modern literature review of this problem. In spite of favorable current of this syndrome without progression of liver pathology and formation of cirrhosis, recurrence periods are characterized with itchy skin and cholestasis obstructing patients ability to work (in described clinical case total duration of patient temporary inability to work was 68 days). Rifampicin and plasmapheresis relieve symptoms and decrease bilirubin level. The most optimal measure in uncomplicated attack of benign recurrent intrahepatic cholestasis is the administration of rifampicin in 150–300 mg dose per day under the patient’s condition control and hepatic parameters control in biochemical blood analysis.
References
1. Folvik G. (2012) Benign recurrent intrahepatic cholestasis: review and long-term follow-up of fi ve cases. Scand J Gastroenterol., vol. 47, pp. 482–488.
2. Van der Woerd W. (2010) Familial cholestasis: progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis and intrahepatic cholestasis of pregnancy. Best Pract Res Clin Gastroenterol., vol. 24, pp. 541–553.
3. Matthias C. (2017) Genetic determinants of cholangiopathies: molecular and systems genetics, BBA – Molecular Basis of Disease: accepted manuscript. Homburg, 36 r.
4. Luketic V.A. (1999) Benign recurrent intrahepatic cholestasis. Clin. Liver Dis., vol. 3, no 3, pp. 509–528.
5. Summerfi eld J.A. (1980) Benign recurrent intrahepatic cholestasis: studies of bilirubin kinetics, bile acids, and cholangiography. Gut, vol. 21, Issue 2, pp. 154–160.
6. Kumar P. (2016) Benign Recurrent Intrahepatic Cholestasis in a Young Adult. Journal of Clinical and Diagnostic Research, vol. 10, Issue 6, pp. 1–2.
7. Carey J.B. (1961) Relief of pruritus of jaundice with bile acid sequestering resin. JAMA, vol. 176, pp. 432–435.
8. Bloomer J.R. (1975) Phenobarbital eff ects in cholestatic liver disease. Ann Intern., vol. 82, pp. 310–317.
9. Summerfi eld J.A. (1980) Naloxone modulates the perception of itch in man. Br J Clin Pharmacol., vol. 10, no 2, pp. 180–183.
10. Shiff E.R. (ed.) (1999) Diseases of the Liver, 7 th edn. Philadelphia: Lippincott-Raven, pp. 611–631.
11. Alva J. (1965) Relief of pruritus of jaundice with methandrostenolone and speculation of the nature of pruritus in liver disease. Am J Med Sci., vol. 250, pp. 60–65.
12. Marshall H. (2005) Rifampicin and ursodeoxycholic acid in cholestatic liver disease. Gastroenterology, vol. 129, pp. 476–485.
13. Paumgartner G. (2008) Medical treatment of cholestatic liver disease. Clin Liver Dis., vol. 12, pp. 53–81.
14. Jankowska I. (2014) Ileal exclusion in children with progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr., vol. 58, pp. 92–95.
15. Duncan J. (1984) Treatment of pruritus due to chronic obstructive liver disease. Br Med J Res Ed., vol. 289, pp. 11–22.
16. Hegade V.S. (2016) The safety and effi cacy of nasobiliary drainage in the treatment of refractory cholestatic pruritus: a multicenter European study. Aliment Pharmacol Ther, vol. 43, pp. 294–302.
17. Van Dijk R. (2015) Characterization and treatment of persistent hepatocellular secretory failure. Liver Int., vol. 35, pp. 1478–1488.
18. Webb G.J. (2018) Low risk of hepatotoxicity from rifampicin when used for cholestatic pruritus: a cross-disease cohort study. Alimentary Pharmacology & Therapeutics, vol. 47, no 8, pp. 1213–1219.
19. Khurana S. (2006) Rifampin is safe for treatment of pruritus due to chronic cholestasis: a metaanalysis of prospective randomized-controlled trials. Liver International, vol. 26, pp. 943–948.
20. Bachs L. (1992) Eff ects of long-term rifampicin administration in primary biliary cirrhosis. Gastroenterology, vol. 102, no 6, p p. 2077–2088.
21. Sheuer P. (1974) Rifampicin hepatitis. Lancet, pp. 421–425.
