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Pinch Purpura: A Clinical Clue to Primary Systemic Amyloidosis.
Indian Dermatol Online J
September 2024
Department of Dermatology, IMS and SUM Hospital, Bhubaneswar, Odisha, India.
POEMS Syndrome.
Presse Med
January 2025
Department of Hematology and Cellular Therapy, National Reference Center "AL Amyloidosis and Other Monoclonal Immunoglobulin Deposit Diseases, University Hospital of Limoges, Limoges, France.
POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, Skin changes) is a syndrome that involves a monoclonal B-cell proliferation, most often plasmacytic, and a variable number of manifestations listed or not in the acronym. These manifestations include sclerotic bone lesions, plasmacytic Castleman disease, papillary edema, peripheral edema, ascites, thrombocytosis and/or polycythemia, venous and/or arterial thrombosis, and renal, pulmonary, and cardiac impairments . Diagnosis is often delayed due to the rarity of this entity and its clinical polymorphism, which can mimic other neurological disorders.
View Article and Find Full Text PDFThe critical role of the variable domain in driving proteotoxicity and aggregation in full-length light chains.
J Mol Biol
January 2025
Department of Biosciences, University of Milan, Italy; Institute of Molecular and Translational Cardiology, IRCCS, Policlinico San Donato, Milan, Italy. Electronic address:
Light chain (AL) amyloidosis is the most common systemic amyloid disease characterized by abnormal accumulation of amyloid fibrils derived from immunoglobulin light chains (LCs). Both full-length (FL) LCs and their isolated variable (VL) and constant (CL) domains contribute to amyloid deposits in multiple organs, with the VL domain predominantly forming the fibril core. However, the role and interplay of these domains in amyloid aggregation and toxicity are poorly understood.
View Article and Find Full Text PDFMultifaceted pulmonary manifestations of amyloidosis: state-of-the-art update.
Expert Rev Respir Med
January 2025
Division of Pulmonary & Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
Introduction: Amyloidosis, a polymeric deposition disease classified according to protein subtype, may have varied pulmonary manifestations. Its anatomic-radiologic phenotypes include nodular, cystic, alveolar-septal, and tracheobronchial forms. Clinical presentation may range from asymptomatic parenchymal nodules to respiratory failure from diffuse parenchymal infiltration or diaphragmatic deposition.
View Article and Find Full Text PDFCarpal Tunnel, Trigger Finger, and Spinal Stenosis: The Rest of the Story.
S D Med
November 2024
Sanford Orthopedics and Sports Medicine, Sioux Falls, South Dakota.
Amyloidosis is a deadly systemic disease in which misfolded proteins accumulate in human tissue eventually leading to morbid dysfunction in multiple organ systems. The prognosis of untreated amyloidosis is poor. Orthopedic manifestations of amyloidosis include carpal tunnel syndrome (CTS), trigger digit, distal biceps tendon rupture, rotator cuff disease, and lumbar spinal stenosis.
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