منابع مشابه
The hereditary amyloidoses.
Hereditary amyloidosis is, in general, a systemic condition related to multiple organ system involvement by beta-structured protein deposits. As such, it often mimics the more common forms of systemic amyloidosis: immunoglobulin light chain (AL, primary) and reactive (AA, secondary). The challenge diagnostically is to recognize hereditary amyloidosis as a distinct entity and then to determine t...
متن کاملEmerging treatment approaches for the systemic amyloidoses.
A 57-year-old man was diagnosed with AL amyloidosis after presenting with nephrotic syndrome. He had been well until 6 months earlier, when he noticed intermittent ankle swelling. He sought medical attention approximately 2 months later because of increased swelling of his legs and progressive fatigue. At that time, his urinary protein excretion was 7.4 g/day. Serum creatinine was 0.9 mg/dL and...
متن کاملDiagnosis and management of the cardiac amyloidoses.
Cardiac amyloidosis is a manifestation of one of several systemic diseases known as the amyloidoses.1,2 This uncommon disease is probably underdiagnosed, and even when a diagnosis of amyloidosis of the heart is made, the fact that there are several types of amyloid, each with its unique features and treatment, is often unrecognized. This can lead to errors in management and in the information c...
متن کاملTransthyretin cardiac amyloidoses in older North Americans.
The amyloidoses are a group of hereditary or acquired disorders caused by the extracellular deposition of insoluble protein fibrils that impair tissue structure and function. All amyloidoses result from protein misfolding, a common mechanism for disorders in older persons, including Alzheimer's disease and Parkinson's disease. Abnormalities in the protein transthyretin (TTR), a serum transporte...
متن کاملAn amyloidogenic determinant in N-terminal pro-brain natriuretic peptide (NT-proBNP): Implications for cardiac amyloidoses.
Deposition of amyloid in the atria (isolated atrial/cardiac amyloid) is fairly common in the aging heart. It consists of amyloid fibrils, formed both by atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) and the precursor molecule of ANP, proANP. This study examines whether amyloidogenic determinants (short peptides/amyloid forming favoring regions) exist in the sequence of NT...
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ژورنال
عنوان ژورنال: Nephrology Dialysis Transplantation
سال: 1998
ISSN: 1460-2385
DOI: 10.1093/ndt/13.suppl_7.33