Budd-Chiari syndrome-acute-on-chronic liver failure with simultaneous thrombotic and non-thrombotic acute insults
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A 21-year-old man presented with acute onset of jaundice, abdominal pain, ascites, and hepatomegaly, along with a history of Budd-Chiari syndrome previously treated with vena cava angioplasty. Investigations revealed rapidly worsening jaundice, coagulopathy, elevated creatinine levels, reactive hepatitis B serology, and positive antiphospholipid antibodies, with scores indicating a poor prognosis for liver transplant-free survival. Abdominal computed tomography demonstrated a narrowed intrahepatic vena cava and new thrombosis in the right and middle hepatic veins. Renal biopsy, prompted by nephritic range proteinuria, indicated mesangioproliferative glomerulonephritis (MPGN) with immune complex deposition. The described case involves acute-on-chronic liver failure with acute insults from new onset hepatic vein thrombosis and hepatitis B reactivation, in a patient at a non-transplant center, who also had underlying antiphospholipid antibody syndrome, and MPGN. The patient was successfully treated with antiviral, anticoagulation, and antiplatelet agents, along with a sodium-glucose cotransporter 2 inhibitor and a direct intrahepatic portosystemic shunt, despite having a Model for End-Stage Liver Disease score of 35.
Abstract
A 21-year-old man presented with acute onset of jaundice, abdominal pain, ascites, and hepatomegaly, along with a history of Budd-Chiari syndrome previously treated with vena cava angioplasty. Investigations revealed rapidly worsening jaundice, coagulopathy, elevated creatinine levels, reactive hepatitis B serology, and positive antiphospholipid antibodies, with scores indicating a poor prognosis for liver transplant-free survival. Abdominal computed tomography demonstrated a narrowed intrahepatic vena cava and new thrombosis in the right and middle hepatic veins. Renal biopsy, prompted by nephritic range proteinuria, indicated mesangioproliferative glomerulonephritis (MPGN) with immune complex deposition. The described case involves acute-on-chronic liver failure with acute insults from new onset hepatic vein thrombosis and hepatitis B reactivation, in a patient at a non-transplant center, who also had underlying antiphospholipid antibody syndrome, and MPGN. The patient was successfully treated with antiviral, anticoagulation, and antiplatelet agents, along with a sodium-glucose cotransporter 2 inhibitor and a direct intrahepatic portosystemic shunt, despite having a Model for End-Stage Liver Disease score of 35.
참고문헌 (16)
- Shukla A, "Association of acute-on-chronic liver failure with vascular liver diseases", Hepatol Int, 13, pp.399~402 (2019)
- Langlet P, "Clini"copathological forms and prognostic index in Budd-Chiari syndrome", J Hepatol, 39, pp.496~501 (2003)
- European Association for the Study of the Liver, "EASL 2017 Clinical Prac"tice Guidelines on the management of hepatitis B virus infection", J Hepatol, 67, pp.370~398 (2017)
- Shukla A, "Budd-Chiari syndrome : consensus guidance of the Asian Pacific Association for the study of the liver(APASL)", Hepatol Int, 15, pp.531~567 (2021)
- Sarin SK, "Acute-on-chronic liver failure : consensus recommendations of the Asian Pacific association for the study of the liver(APASL) : an update", Hepatol Int, 13, pp.353~390 (2019)
- Shibata R, "Effect of dapagliflozin on the initial estimated glomerular filtration rate dip in chronic kidney disease patients without diabetes mellitus", Clin Exp Nephrol, 27, pp.44~53 (2023)
- Hadengue A, "The changing scene of hepatic vein thrombosis : recognition of asymptomatic cases", Gastroenterology, 106, pp.1042~1047 (1994)
- Menon KV, "The Budd-Chiari syndrome", N Engl J Med, 350, pp.578~585 (2004)
- Parekh J, "Budd-Chiari syndrome causing acute liver failure : a multicenter case series", Liver Transpl, 23, pp.135~142 (2017)
- Shalimar, "Acute on chron"ic liver failure because of acute hepatic insults : etiologies, course, extrahepatic or"gan failure and predictors of mortality", J Gastroenterol Hepatol, 31, pp.856~864 (2016)
- Shalimar, "Acute-on"chronic liver failure in India : The Indian National Association for Study of the Liver consortium experience", J Gastroenterol Hepatol, 31, pp.1742~1749 (2016)
- Shalimar, "Acute-on-chronic liver failure in Budd-Chiari syndrome : profile and predictors of outcome", Dig Dis Sci, 65, pp.2719~2729 (2020)
- Radha Krishna Y, "Clinical features and predictors of outcome in acute hepatitis A and hepatitis E virus hepatitis on cirrhosis", Liver Int, 29, pp.392~398 (2009)
- Guy J, "New model for end stage liver disease improves prognostic capability after transjugular intrahe"patic portosystemic shunt", Clin Gastroenterol Hepatol, 7, pp.1236~1240 (2009)
- Spengler EK, "Transjugular intrahepatic portosystemic shunt does not independently increase risk of death in high model for end stage liver disease patients", Hepatol Commun, 1, pp.460~468 (2017)
- de Franchis R, "Expanding consensus in portal hypertension : report of the Baveno VI Consensus Workshop : stratifying risk and individualizing care for portal hypertension", J Hepatol, 63, pp.743~752 (2015)