Abstract：
:The predominant cause of hereditary renal amyloidosis is a mutation of the fibrinogen Aalpha chain (AFib), the most common being the E526V mutation. The evolution towards terminal renal insufficiency is constant and raises the question of renal transplantation and the risk of recurrence. We describe the case of a Portuguese woman with the E526V mutation without any renal or hepatic history in her family which developed a nephrotic syndrome at the age of 35, followed by stage 5 renal insufficiency. Because of the risk of recurrence of amyloidosis on its transplant, we carried out a combined transplantation liver-kidney despite the absence of clinical or biological hepatic abnormalities. Four years later, the result is excellent and there is no sign of the disease on the new organs. This successful experience as well as the five other published cases of combined liver-kidney transplantation in Aalpha fibrinogen chain amyloidosis, demonstrates the feasibility and efficacy of this treatment in AFib amyloidosis.
journal_name
Nephrol Therjournal_title
Nephrologie & therapeutiqueauthors
Delabre JP,Pageaux GP,Le Quellec A,Raynaud P,Grateau G,Mourad Gdoi
10.1016/j.nephro.2008.08.015subject
Has Abstractpub_date
2009-04-01 00:00:00pages
139-43issue
2eissn
1769-7255issn
1872-9177pii
S1769-7255(08)00214-9journal_volume
5pub_type
杂志文章,评审abstract：:Focal segmental glomerulosclerosis is a common glomerular histological lesion, which is usually characterised by non-nephrotic range proteinuria or nephrotic syndrome. It may be idiopathic or occurs secondarily to drugs, diabetes, obesity or HIV nephropathy and other infections. Dasatinib, a tyrosine kinase inhibitor ...
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journal_title：Nephrologie & therapeutique
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,多中心研究
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,多中心研究
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journal_title：Nephrologie & therapeutique
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
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更新日期：2008-07-01 00:00:00
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
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journal_title：Nephrologie & therapeutique
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
doi：10.1016/j.nephro.2009.10.005
更新日期：2010-04-01 00:00:00
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章,评审
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
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journal_title：Nephrologie & therapeutique
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
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journal_title：Nephrologie & therapeutique
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更新日期：2006-11-01 00:00:00
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journal_title：Nephrologie & therapeutique
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journal_title：Nephrologie & therapeutique
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journal_title：Nephrologie & therapeutique
pub_type： 杂志文章
doi：10.1016/j.nephro.2017.12.001
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abstract：:Dialysis-related constraints encourage questioning about discontinuation of treatment. In France, the 04/22/2005 law, related to patients' rights and end-of-life issues, defines bounds to treatment withdrawal, authorizing it in specific conditions, to avoid foolish obstinacy. Shortly before the publication of this law...
journal_title：Nephrologie & therapeutique
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