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Acute liver failure due to primary amyloidosis in a nephrotic syndrome: a swiftly
progressive course
#MMPMID26965175
Cardoso BA
; Leal R
; Sá H
; Campos M
BMJ Case Rep
2016[Mar]; 2016
(?): ? PMID26965175
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AL amyloidosis is a clonal plasma cell proliferative disorder characterised by
extracellular tissue deposits of insoluble fibrils derived from ? or ?
immunoglobulin light chains. The most common organs affected by AL amyloidosis
are the kidney, presenting with nephrotic syndrome and/or progressive renal
dysfunction, and the heart, with restrictive cardiomyopathy. Hepatic deposition
of fibrils occurs in half the cases but the liver is rarely the predominantly
affected organ. The most common presentation of hepatic amyloidosis is
hepatomegaly with elevated alkaline phosphatase. Acute liver failure with
cholestasis and jaundice is a rare complication, with a prevalence of
approximately 5%, and is usually associated with a worse prognosis. We report a
case of a 39-year-old man admitted to our nephrology department with an unusual
presentation of primary amyloidosis with nephrotic syndrome and acute liver
failure, complicated by obstructive cholestasis resulting in death 2?months after
diagnosis.
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