| Primary sclerosing cholangitis complicated by amyloid A amyloidosis: complete regression of the nephrotic syndrome by liver transplantation. | |
| | |
MedLine Citation:
|
PMID: 20729743 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Secondary amyloidosis may complicate chronic inflammatory conditions and mostly presents as a renal disease with nephrotic syndrome or renal insufficiency. Its prognosis is largely affected by control of the underlying disease. We report a patient with primary sclerosing cholangitis, who developed cirrhosis over a 4-year period. Therapy with steroids and azathioprine was necessary for symptom control. Despite this treatment, she developed secondary amyloidosis with nephrotic syndrome 4 years after the initial presentation. The inflammatory process in the bile ducts was considered the cause of amyloid A amyloidosis. To control the nephrotic syndrome, liver transplantation was performed with the removal of the diseased liver and bile duct system. Liver transplantation was followed by a progressive and complete disappearance of the nephrotic syndrome. This is the first report describing the occurrence of amyloid A amyloidosis in primary sclerosing cholangitis, and the reversal of a secondary amyloidosis-induced nephrotic syndrome as a result of liver transplantation. |
| | |
Authors:
|
Werner Van Steenbergen; Lieve Braeye; Rudy Harlet; Frederik Nevens; Johan Fevery; Valeer Desmet; Tania Roskams; Jacques Pirenne |
Related Documents
:
|
490283 - Neuroblastoma presenting with the nephrotic syndrome. 7925893 - Small cell lung cancer with paraneoplastic nephrotic syndrome. 2619613 - Non-epileptic myoclonus and mitochondrial encephalomyopathy. |
Publication Detail:
|
Type: Case Reports; Journal Article |
Journal Detail:
|
Title: European journal of gastroenterology & hepatology Volume: 22 ISSN: 1473-5687 ISO Abbreviation: Eur J Gastroenterol Hepatol Publication Date: 2010 Oct |
Date Detail:
|
Created Date: 2010-09-09 Completed Date: 2010-12-28 Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 9000874 Medline TA: Eur J Gastroenterol Hepatol Country: England |
Other Details:
|
Languages: eng Pagination: 1265-70 Citation Subset: IM |
Affiliation:
|
Department of Internal Medicine, Liver Unit, University Hospital Gasthuisberg, Catholic University of Leuven, Turnhout, Belgium. Werner.vansteenbergen@uzleuven.be |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adult Amyloidosis / complications*, metabolism, surgery* Cholangitis, Sclerosing / complications, surgery Female Humans Liver Transplantation* Nephrotic Syndrome / etiology* Remission Induction Serum Amyloid A Protein / metabolism |
| Chemical | |
Reg. No./Substance:
|
0/Serum Amyloid A Protein |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Serum lipids in European chronic HCV genotype 1 patients during and after treatment with pegylated i...
Next Document: Disparities in rates and trends of HIV/AIDS during pregnancy across the decade, 1998-2007.