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Literature DB >> 1889693

A case of prominent hepatic cholestasis developing to hepatic failure in lambda-AL amyloidosis.

K Dohmen¹, M Nagano, R Iwakiri, Y Yamano, Y Kikuchi, M Mizoguchi, Y Iwata, Y Mori, H Ishibashi.

Abstract

We report a case of lambda-AL amyloidosis which manifested prominent hepatic cholestasis. The patient was a 71-year-old Japanese male who was admitted to our hospital because of abdominal distension and jaundice. Laboratory examination revealed a marked deterioration of liver function with cholestasis. Gastric biopsy revealed amyloid deposition. Under a diagnosis of primary amyloidosis he was treated with corticosteroid and dimethylsulfoxide. However, jaundice progressed, renal function deteriorated rapidly, and he died two weeks after admission. Autopsy revealed a profound deposition of lambda-AL amyloid not only in the liver but also in the kidneys, spleen, lungs, heart and intestine.

Entities: Chemical Disease Species

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Year: 1991 PMID： 1889693 DOI： 10.1007/bf02781928

Source DB: PubMed Journal: Gastroenterol Jpn ISSN： 0435-1339

18 in total

A case of prominent hepatic cholestasis developing to hepatic failure in lambda-AL amyloidosis.

1. Amyloid disease of the liver. Correlation of clinical, functional and morphologic features in forty-seven patients.

2. Cholestatic jaundice associated with primary amyloidosis.

3. Primary amyloidosis presenting as "sicca complex" and severe intrahepatic cholestasis.

4. Cholestasis and liver failure with lambda-AL amyloidosis.

5. Amyloidosis: review of 236 cases.

6. Intrahepatic cholestasis with predominant pericentral deposition in systemic amyloidosis.

7. Different morphologic aspects and clinical features in massive hepatic amyloidosis.

8. Primary systemic amyloidosis: resolution of the nephrotic syndrome with melphalan and prednisone.

9. Systemic amyloidosis involving two renal transplants.

10. Hepatic amyloidosis--two cases report.

1. A case of fatal intrahepatic cholestasis with primary AL amyloidosis: is early diagnosis possible?

Review 2. Hepatic failure in a case of multiple myeloma-associated amyloidosis (kappa-AL)

3. Progressive liver failure in a patient with adult Niemann-Pick disease associated with generalized AL amyloidosis.

4. Rare case of acute on chronic hepatic failure in a patient with multiple myeloma-associated amyloidosis.

5. Primary Hepatic Amyloidosis Presenting as Acute-on-Chronic Liver Failure.