Articles: amyloidosis.
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More than 35 amyloid precursor proteins have been identified and many have tropism for the kidney. Renal amyloidosis is most commonly seen in AL and AA amyloidosis and the main clinical manifestations are proteinuria and progressive renal dysfunction. ⋯ Management of renal amyloidosis typically combines therapy targeting the underlying amyloid process and supportive management. Patients with renal amyloidosis who progress to end-stage renal disease can be treated with dialysis, and in selected patients, with renal transplantation.
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Cardiac amyloidosis (CA) results in symptoms of heart failure, atrial and ventricular arrhythmias, conduction disturbances, and profound autonomic dysfunction. These symptoms present unique management challenges as compared with patients without CA, and can be very debilitating. Thus, management of the cardiovascular symptoms of these patients plays a central role in their care. This review presents an overview of the typical cardiovascular manifestations encountered in patients with CA and highlights the key management considerations for each.
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Review
Update on the Contemporary Treatment of Light Chain Amyloidosis Including Stem Cell Transplantation.
The management of immunoglobulin light chain (AL) amyloidosis is complex. Emerging data have shown promising results for several novel agents. We review the management of AL amyloidosis, including factors that determine transplant eligibility, treatment options for transplant-ineligible patients, and treatment options for relapsed/refractory AL amyloidosis. ⋯ The latter option is based on a landmark trial that led to the first US Food and Drug Administration-approved therapy for AL amyloidosis. For relapsed/refractory disease, novel therapeutics including proteosome inhibitors, immunomodulatory agents, and monoclonal antibodies have shown promising results. In this review, we summarize data for various therapeutics in different clinical scenarios of AL amyloidosis.
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Diagnoses of amyloidosis, particularly transthyretin amyloid cardiomyopathy (ATTR-CM), are steadily increasing throughout the world, but the condition remains underdiagnosed. Patients with amyloidosis may present to a range of medical and surgical specialties, often with multisystemic disease, and a high index of clinical suspicion is required for diagnosis. ⋯ Histological diagnosis of amyloid has been enhanced by laser capture microdissection and tandem mass spectrometry. Early diagnosis and treatment prior to the development of end-organ damage remains essential to improving morbidity and mortality for patients with amyloidosis.
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Amyloidosis is a heterogeneous disease that can cause a wide array of nonspecific symptoms when the gastrointestinal (GI) tract is involved, including weight loss, early satiety, change in bowel habits with diarrhea, constipation, or alternating bowel pattern. Endoscopy with biopsy for Congo red staining establishes the diagnosis and fibril subtyping helps to guide targeted treatment options. ⋯ Management of the symptoms of GI tract involvement with amyloidosis relate to addressing the underlying symptom complex that is produced and generally abstracted from the management of severe forms of functional GI disorders. Attention to improving symptom management and nutrition status can improve quality of life in these patients.