Amyloidosis is a disorder of protein folding in which normally soluble proteins are deposited as abnormal, insoluble fibrils that disrupt tissue structure and cause disease 1 . Some 20 different unrelated proteins can form amyloid in vivo , and amyloidosis is classified clinically according to the ...
IgM Amyloidosis: Clinical Features in Therapeutic Outcomes It is not widely recognized that patients with amyloidosis may be associated with an IgM monoclonal protein. We reviewed the characteristics of 22 IgM amyl... MA Gertz,FK Buadi,SR Hayman - 《Clinical Lymphoma Myeloma & Leukemia》 被引量...
Currently, multiple drugs are in various stages of clinical trials, and these new treatments may improve the prognosis of patients with a confirmed diagnosis of ATTR amyloidosis (Table 1).ATTR淀粉样变病曾经被认为是不可治疗的,...
To clarify the clinicopathological features of cardiac amyloidosis in transthyretin (TTR) familial amyloid polyneuropathy (FAP), 169 patients were divided into three groups. Group I consisted of 113 patients with ATTR Val30Met who originated from an endemic focus, II consisted of 36 patients with ...
In those with suspected amyloidosis : Screening for monoclonal light chains If monoclonal light chains are negative → bone scintigraphy (to confirm the presence of transthyretin amyloid) Genetic testing is performed in those found with transthyretin amyloid to differentiate hereditary variant from wild...
The disorder, characterized by amyloid deposits composed of P2microglobulin (&M) fibrils as the major constituent protein, primarily affects joints and periarticular bone. The principal clinical and radiologic features of this type of amyloidosis are chronic arthrafgias, carpal tunnel syndrome, erosive...
Amyloid goiter: Report of the clinical, histological and biochemical features of five casesAMYLOID GOITERAA AMYLOIDOSISThe clinical course and pathological findings of five cases of amyloid goiter in patients with AA amyloidosis are presented. The underlying diseases which caused the amyloidosis were: ...
[84,85,86], although the earliest stages of amyloidosis (Thal stages 1–2 [87]) might not be detected [88,89]. Unlike most neuroimaging modalities, the regional distribution of amyloid-PET signal is usually non-informative—PET signal is typically seen throughout the neocortex, with little ...
Dry eyes can be caused by amyloidosis, inflammation (chronic blepharitis or conjunctivitis, pemphigoid, or Stevens-Johnson syndrome), SS, neurologic conditions that impair eyelid or lacrimal gland function, sarcoidosis, toxicity (burns or drugs), and a variety of other conditions (corneal anesthesia,...
Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis. We sought to better define the clinical, echocardiographic, and electrocardiographic features of cardiac involvement in SSA and to compare them with the ... B Ng - 《Archives of Internal Me...