BACKGROUND AL amyloidosis is an acquired systemic disease in which a pathologic amorphous substance produced as a result of abnormal protein metabolism is deposited in the extracellular space of various tissues. OBJECTIVES The aim of the study was to investigate the relationship between the kappa and lambda serum free light chains (sFLCs) and the development of AL amyloidosis in patients suff...

position of insoluble fibrillar proteins in various organs [1]. In humans, more than 23 different and unrelated proteins are known to form amyloid fibrils [2]. Amyloidosis is divided into primary (i. e. idiopathic) and secondary amyloidosis (i. e. associated with chronic inflammatory conditions, and infectious and neoplastic disorders) [1]. Primary amyloidosis is extremely rare in the gastroint...

Amyloidosis of the gastrointestinal tract, with biopsy-proven disease, is rare. We reviewed a series of patients who presented with biopsy-proven gastrointestinal amyloidosis and report their clinical characteristics, treatments, and survival. This is a retrospective review of data prospectively collected from January 1998 to December 2011 in a tertiary referral center; 2,334 patients with all ...

Amyloid A (AA) amyloidosis, a fatal systemic amyloid disease, occurs secondary to chronic inflammatory conditions in humans. Although persistently elevated serum amyloid A (SAA) levels are required for its pathogenesis, not all individuals with chronic inflammation necessarily develop AA amyloidosis. Furthermore, many diseases in cats are associated with the elevated production of SAA, whereas ...

Cardiac amyloidosis is a prognostically critical condition, since it is a frequent cause of death in patients with systemic amyloidosis. Lee et al. reported, for the first time, describing systemic amyloidosis in Korea from the perspective of cardiologists. In this paper, the authors enrolled 129 systemic amyloidosis patients retrospectively from 1999 to 2011. They showed that cardiac involve w...

AMA Milewska J, Małachowska U, Czuwara Rudnicka L, Olszewska M. Lichen amyloidosis. Dermatology Review/Przegląd Dermatologiczny. 2023;110(1):37-42. doi:10.5114/dr.2023.127656. APA Milewska, J., Małachowska, U., Czuwara, Rudnicka, L., & Olszewska, (2023). Dermatologiczny, 110(1), 37-42. https://doi.org/10.5114/dr.2023.127656 Chicago Justyna, Urszula Joanna Lidia and Małgorzata Olszewska. 2023. "...

Hepatic involvement in primary amyloidosis is an infrequent challenge to the hepatologist. Although usually asymptomatic, amyloidosis may have unusual manifestations. Liver biopsy is an important diagnostic tool for this condition. Herein, we report three cases of portal hypertension related to primary hepatic amyloidosis, one of them in the form of acute liver failure.

Although the gastrointestinal tract is susceptible to the deposition of amyloid materials with systemic disease, localised amyloidosis of the gut is very rare.' Complications associated with amyloidosis of the gastrointestinal tract are uncommon and colonic perforation is exceptionally rare. We report a case ofperforation of the sigmoid colon secondary to localised amyloidosis and review other ...

UNLABELLED Serum amyloid P component (SAP) binds to amyloid. (123)I-SAP scintigraphy is used to evaluate the extent and distribution of amyloid in systemic amyloidosis and has great clinical value in the detection of systemic amyloidosis. The aim of the study was to assess during scintigraphy the diagnostic performance and prognostic value of a simple parameter describing extravascular (123)I-S...

Transthyretin amyloidosis (ATTR-amyloidosis) is a systemic disorder associated with extracellular deposition in the tissues and organs of amyloid fibrils, transthyretin-containing insoluble protein-polysaccharide complexes. The change transthyretin conformation, leading to its destabilization amyloidogenicity, can be acquired (wild type, ATTRwt) hereditary due mutations TTR gene (variant, ATTRv...