AL-amyloidosis of the kidney initially presenting as minimal change glomerulonephritis

Hetzel, Gerd R.; Uhlig, Katrin; Mondry, Adrian; Helmchen, Udo; Grabensee, B

doi:10.1053/ajkd.2000.16205

Cited by 18 publications

(8 citation statements)

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“…There is no significant correlation between the extent of amyloid deposition and amount of proteinuria. Even a small amount of amyloid deposition can lead to severe NS [ 18 ]. Liver involvement and renal dysfunction seems more common in patients of κ-type in our series, and this result is consistent with previous studies [ 19 ].…”

Section: Discussionmentioning

confidence: 99%

The clinical features and outcomes of systemic AL amyloidosis: a cohort of 231 Chinese patients

Huang

Wang

Jiang

et al. 2014

Clinical Kidney Journal

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BackgroundFew data are available on the clinical features and outcomes of Chinese patients with systemic immunoglobulin light-chain (AL) amyloidosis. The aim of this study is to reveal the clinical picture and risk factors of disease progression in a large cohort of Chinese patients with AL amyloidosis.MethodsPatients in the Jinling Hospital amyloidosis registry from 2003 to 2011 were studied. The clinical and laboratory information were collected from first presentation to death or until the last available clinical follow-up. The patients' survival and renal outcomes were analyzed, and the relationships between the clinical parameters and survival were also assessed.ResultsA total of 231 patients were enrolled in this study, all the patients studied had renal involvement. One hundred and fifty-three (66.2%) were male, and the median age at diagnosis was 56 years. A total of 198 (85.7%) cases had light-chain λ-type. One hundred and forty-seven (63.6%) cases presented as nephrotic syndrome (NS), and 25% of patients had renal insufficiency at diagnosis. Liver involvement and NS appeared to be more common in patients of κ-type amyloidosis, and renal impairment is more severe in κ-type amyloidosis. The median survival time of all patients was 36.3 months, and the 1-, 2-, 3- and 5-year cumulative survival rates were 67, 53, 48 and 35%, respectively. Multivariate COX analysis showed that age, hepatic involvement and heart involvement can significantly influence survival in these patients. The median time that patients remained dialysis free was 50 months. The percentage of patients that remained dialysis free at 1, 2, 3 and 5 years were 78, 69, 62 and 37%, respectively. Multivariate COX analysis showed that serum creatinine and hypotension were the important risk factors of renal failure.Conclusionλ-Type is the most dominant type of AL amyloidosis in Chinese patients. The survival of patients with AL amyloidosis is poor. The risk factors included heart and hepatic involvement, hypotension and impairment of renal function. The high serum creatinine level and hypotension at diagnosis are associated with poor renal outcome.

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Section: Discussionmentioning

confidence: 99%

The clinical features and outcomes of systemic AL amyloidosis: a cohort of 231 Chinese patients

Huang

Wang

Jiang

et al. 2014

Clinical Kidney Journal

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“…However, even when electron microscopy is performed, the diagnosis of amyloidosis can be missed if fibrils are scant. Such cases sometimes are misdiagnosed as minimal-change disease (9,10).…”

Section: Histologic Demonstration Of Amyloidmentioning

confidence: 99%

Amyloidosis-Associated Kidney Disease

Dember

2006

Journal of the American Society of Nephrology

349

284

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The amyloidoses are a group of disorders in which soluble proteins aggregate and deposit extracellularly in tissues as insoluble fibrils, causing progressive organ dysfunction. The kidney is one of the most frequent sites of amyloid deposition in AL, AA, and several of the hereditary amyloidoses. Amyloid fibril formation begins with the misfolding of an amyloidogenic precursor protein. The misfolded variants self-aggregate in a highly ordered manner, generating protofilaments that interact to form fibrils. The fibrils have a characteristic appearance by electron microscopy and generate birefringence under polarized light when stained with Congo red dye. Advances in elucidating the mechanisms of amyloid fibril formation, tissue deposition, and tissue injury have led to new and more aggressive treatment approaches for these disorders. This article reviews the pathogenesis, diagnosis, clinical manifestations, and treatment of the amyloidoses, focusing heavily on the renal aspects of each of these areas.

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“…Eine Koinzidenz mit einer glomerulären Amyloidose ist auch für andere Glomerulonephritisformen sowie für eine diabetische Glomerulosklerose beschrieben worden [5,16]. Eine Sonderstellung nehmen in diesem Zusammenhang möglicher-weise "minimal changes" ein, da sie durch Ablagerungen von noch nicht fibrillär umgewandelten, daher noch Kongorot-negativen, amyloidogenen Vorläuferproteinen verursacht sein könnten [17]. Ein schwerer Markbefall und eine Ummantelung der hier gelegenen Sammelrohre durch Amyloid (.…”

Section: Intrarenale Amyloidverteilungunclassified