Presentation and Outcome of Patients with Systemic Amyloidosis Undergoing Dialysis

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Presentation and outcome of patients with systemic amyloidosis undergoing dialysis.

BACKGROUND AND OBJECTIVES Light chain (AL) and secondary (AA) amyloidosis usually present as a systemic disease frequently involving the kidney and leading to ESRD. Data regarding patients with AA or AL amyloidosis undergoing dialysis remain scarce. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS We retrospectively studied patients with AA or AL amyloidosis who started dialysis in five French c...

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Senile Systemic Amyloidosis: Clinical Features at Presentation and Outcome

BACKGROUND Cardiac amyloidosis is a fatal disease whose prognosis and treatment rely on identification of the amyloid type. In our aging population transthyretin amyloidosis (ATTRwt) is common and must be differentiated from other amyloid types. We report the clinical presentation, natural history, and prognostic features of ATTRwt compared with cardiac-isolated AL amyloidosis and calculate the...

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Unusual initial presentation of primary systemic (AL) amyloidosis with severe cardiomyopathy and fatal outcome.

Primary (AL) amyloidosis is the most common form of systemic amyloidosis seen in current clinical practice. The symptoms of the disease are usually vague, special features are seen in fewer than one fifth of patients, and the combination of organs and systems involved provides a clue for the diagnosis. We describe a patient in whom asymptomatic hepatomegaly, cardiomegaly, hyperlipidaemia and el...

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Dialysis-Related Amyloidosis: Pathogenesis and Clinical Features in Patients Undergoing Dialysis Treatment

To date, there are 27 types of amyloidosis known extracellular fibril proteins in human, and each amyloidosis is characterized amyloid protein precursor, systemic (S) or localized organ (L), and syndrome or involved tissues [1]. In the nomenclature, dialysis-related amyloidosis (DRA) is defined as β2-microglobulin-related (Aβ2M) amyloid which precursor protein is β2microglobulin (β2-m). It is a...

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A case of primary systemic amyloidosis with nail dystrophy

We hereby report a 79-year-old Iranian man presenting with nail dystrophy and subsequent development of purpuric and ecchymotic plaques, hemorrhagic bullae, and infiltrated papules on the head, neck and trunk. Histological examination of the gingiva, bone marrow aspiration, and biopsy confirmed the diagnosis of primary systemic amyloidosis. In this case, nail dystrophy was the presenting sign o...

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ژورنال

عنوان ژورنال: Clinical Journal of the American Society of Nephrology

سال: 2008

ISSN: 1555-9041,1555-905X

DOI: 10.2215/cjn.02470607