AA amyloidosis is one of the principal causes of morbidity and mortality in captive cheetahs (Acinonyx jubatus), which are in danger of extinction, but little is known about the underlying mechanisms. Given the transmissible characteristics of AA amyloidosis, transmission between captive cheetahs may be a possible mechanism involved in the high incidence of AA amyloidosis. In this study of anim...

INTRODUCTION Hereditary transthyretin amyloidosis (ATTR) is a genetic disease caused by a point mutation in the TTR gene that causes the liver to produce an unstable TTR protein. The most effective treatment has been liver transplantation in order to replace the variant TTR producing liver with one that produces only wild-type TTR. ATTR amyloidosis patients' livers are reused for liver sick pat...

INTRODUCTION Amyloidosis is a group of diseases pathohistologically diagnosed by characteristic extracellular deposition of an abnormal fibrillary protein (i.e. amyloid) into organs, leading to organ dysfunction secondary to destruction of normal tissue architecture. METHODS Case-report of a 44 year-old female, presenting with massive abdominal distension clinically suspected of ascites. RE...

Amyloidosis, a heterogenous group of disorders, is characterized by the extracellular deposition autologous, insoluble, fibrillar misfolded proteins. These proteins deposit in tissues aggregated s-pleated sheets arranged an antiparallel fashion and cause distortion to tissue architecture function. In current literature, about 60 heterogeneous amyloidogenic have been identified, out which 27 ass...

Hereditary fibrinogen A -chain amyloidosis: Phenotypic characterization of a systemic disease and the role of liver transplantation. Blood 115: 2998–3007, 2010 Arie J. Stangou, Nicholas R. Banner, Bruce M. Hendry, Mohamed Rela, Bernard Portmann, Julia Wendon, Mark Monaghan, Philip MacCarthy, Muriel Buxton-Thomas, Christopher J. Mathias, Juris J. Liepnieks, John O’Grady, Nigel D. Heaton, and Mer...

Transthyretin is primarily synthesized in the liver and transports thyroxine vitamin A body. The transthyretin when dissociated into monomers can misfold ultimately form amyloid fibrils. There are two types of ATTR amyloidosis: hereditary (caused by mutations TTR gene) wild-type (also referred to as senile systemic amyloidosis). Amyloid cardiomyopathy develop patients with both amyloidosis, has...

Hepatic involvement in primary amyloidosis (AL type) is not rare but is often clinically silent. However, presentation with jaundice in AL-type amyloidosis is rare, with an incidence of less than 5% reported in the literature. It is considered to be a preterminal sign. We herein report on a case of primary hepatic amyloidosis presenting with severe intrahepatic cholestasis. Viral, drug, alcohol...

Hepatic venous catheterisation and transvenous liver biopsy were performed in five patients with hepatic amyloidosis. In three patients, hepatic venous pressures were normal and histological examination of the liver biopsy specimen showed discrete and sparse perisinusoidal amyloid deposits. In the other two, however, the gradient between wedged and free hepatic venous pressures was increased (1...

Cardiovascular (cardiac) amyloidosis (CA) is a clinical pathology, usually of geneticallymediated nature, initiated by the precipitation process insoluble fibrous protein with β-pleated sheet secondary structure. Such anomalous changes lead to formation amyloid fibrils, which may give rise various forms amyloidosis. Amyloid can be found in organs and systems, such as cardiovascular system, cent...

Methods Healthy volunteers (n=70; 35 male; 35 female; median age 46 years) and patients with systemic AL Amyloidosis (n=56; 36 male; 20 female; median age 62 years), with clinical indications for CMR scanning, additionally underwent multi-organ ECV measurement. Technical details were: gadoteric acid, 0.1mmol/Kg plus infusion, multibreathhold T1 measurement and equilibrium imaging of heart, live...