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- W4385187904 abstract "Background and Aim: Amyloidosis is a systemic infiltrative disorder characterized by deposition of amyloid fibrils in extracellular spaces. Liver involvement can be seen in both primary and secondly amyloidosis which can be subclinical or symptomatic. However, primary hepatic amyloidosis is a very rare variant in which deposition of amyloid protein primarily occurs in liver only. We are presenting an interesting case of sub-acute liver failure due to primary hepatic amyloidosis who underwent living donor liver transplantation (LDLT). Case report: This was a 62-year-old female presented with history of slowly progressive painless jaundice and pedal edema for 4 months and abdominal distention for 2 weeks. There was no past history of any liver disease or other chronic systemic illness. On history, physical examination and initial laboratory evaluation, etiology of liver disease could not be found out. She underwent trans-Juglar liver biopsy which was suggestive of hepatic amyloidosis. She was initially given steroids, cyclophosphamide and bortezomib followed by daratumumab and lenalidomide due to non-response to initial therapy. However, she did not respond to medical treatment. Due to progressively rising bilirubin associated with decompensation in form of ascites and hepatic encephalopathy, she underwent LDLT after thorough evaluation. Her post-operative course was smooth and uneventful. Serum bilirubin and other liver biochemistry parameters gradually improved. Patient was discharged on 18th postoperative day. This is a rare case of primary hepatic amyloidosis presented as sub-acute liver failure who successfully underwent LDLT. Primary hepatic amyloidosis is extremely rare variant with very poor prognosis. Median survival of less than nine months has been reported in patients with presence of jaundice and portal hypertension. Conclusion: This case report describes a very rare case of primary hepatic amyloidosis presenting as liver failure who underwent successful LDLT." @default.
- W4385187904 created "2023-07-25" @default.
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- W4385187904 date "2023-01-01" @default.
- W4385187904 modified "2023-09-28" @default.
- W4385187904 title "Primary Hepatic Amyloidosis Related Liver Failure Rescued with Living Donor Liver Transplant: A Case Report" @default.
- W4385187904 doi "https://doi.org/10.1016/j.jceh.2023.07.113" @default.
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