منابع مشابه
Emerging 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 کاملBiochemical markers in early diagnosis and management of systemic amyloidoses.
Systemic amyloid diseases are characterized by widespread protein deposition as amyloid fibrils. Precise diagnostic framing is the prerequisite for a correct management of patients. This complex process is achieved through a series of steps, which include detection of the tissue amyloid deposits, identification of the amyloid type, demonstration of the amyloidogenic precursor, and evaluation of...
متن کاملThe 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 کاملPatient experience with hereditary and senile systemic amyloidoses: a survey from the Amyloidosis Research Consortium
Background Amyloidosis is caused by the accumulation of misfolded proteins, resulting in dysfunction of vital organs (eg, heart, kidneys, nervous system). Diagnosis and access to appropriate therapy pose significant challenges, and there is a paucity of literature depicting the patient (pt) experience. We conducted a survey to identify the challenges in establishing a diagnosis of amyloidosis a...
متن کاملSystemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types.
BACKGROUND Most studies of amyloidotic cardiomyopathy consider as a single entity the 3 main systemic cardiac amyloidoses: acquired monoclonal immunoglobulin light-chain (AL); hereditary, mutated transthyretin-related (ATTRm); and wild-type transthyretin-related (ATTRwt). In this study, we compared the diagnostic/clinical profiles of these 3 types of systemic cardiac amyloidosis. METHODS AND ...
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ژورنال
عنوان ژورنال: Annual Review of Biochemistry
سال: 2013
ISSN: 0066-4154,1545-4509
DOI: 10.1146/annurev-biochem-072611-130030