Hereditary fibrinogen Aα-chain amyloidosis (Aα-chain amyloidosis) is a type of autosomal dominant systemic amyloidosis caused by mutations in fibrinogen Aα-chain gene (FGA). Patients with Aα-chain amyloidosis have been mainly reported in Western countries but have been rarely reported in Asia, with only five patients with Aα-chain amyloidosis being reported in Korea, China, and Japan. Clinicall...

A conception of amyloidosis as merely a complication of chronic syphilitic, tuberculous or septic disease of bones and joints or of chronic intrathoracic sepsis is no longer adequate. This older view has had to be modified as the association of amyloidosis with other diseases came to be recognized: the predisposing diseases are now known to include rheumatic and rheumatoid affections, myelomato...

It takes at least several months from the suspicion of amyloidosis to diagnosis. This delays start specific therapy. Diagnosis should consist not only in differential diagnosis and confirmation diagnosis, but also determining type amyloidosis. The case report is interesting that paraclinical diagnostics determined picture characteristic ATTR-amyloidosis with involvement peripheral nervous syste...

BACKGROUND AND OBJECTIVES Cardiac involvement is frequent in systemic amyloidosis and is the most important determinant of the clinical outcome. The aims of this study were to assess the incidence and prognosis of cardiac amyloidosis and discuss the diagnostic issues related to cardiac amyloidosis. SUBJECTS AND METHODS We retrospectively studied all patients diagnosed with systemic amyloidosi...

OBJECTIVE To investigate if serum apolipoprotein A-I and A-II (apoAI and apAII) concentrations change in subjects with systemic amyloidosis secondary to underlying disorders. METHODS Serum concentrations of apoAI and apoAII were measured in 21 multiple myeloma patients, including eight with amyloidosis; 95 rheumatoid arthritis patients, including 45 with amyloidosis; and 73 haemodialysis pati...

AMYLOIDOSIS is caused by the accumulation of amyloid fibrils in vital organs, leading to functional disturbances. Approximately 40% of patients with primary amyloidosis have had cardiac amyloidosis. In cardiac amyloidosis, right heart failure is the predominant sign, whereas left heart function is preserved until late in the disease. Because of the cardiac involvement, when patients with cardia...

Amyloidosis includes a spectrum of diseases that involve the deposition of misfolded proteins in various tissues throughout the body. There are many different proteins known to cause amyloidosis and the etiology of the amyloid subtype determines where the abnormal proteins deposit. AL, ATTRwt (formerly senile systemic amyloidosis), and ATTRm (formerly familial amyloidosis) are the most common a...

Abstract Background Bone scintigraphy is extremely valuable when assessing patients with suspected cardiac amyloidosis (CA), but the clinical significance and associated phenotype of different degrees myocardial tracer uptake across types yet to be defined. Purpose We sought define phenotypes varying on bone scintigraphy, multiple systemic using extensive characterisation comprising biomarkers,...

Amyloidosis cutis dyschromica is a rare type of primary cutaneous amyloidosis characterized by reticulate hyper-pigmentation with discrete hypopigmented macules. Up to date, about 50 cases of amyloidosis cutis dyschromica have been reported and the majority are familial cases of Asian ethnicity. Various diseases, particularly autoimmune diseases such as systemic sclerosis and systemic lupus ery...