BACKGROUND Amyloid deposition in heart is a common occurrence in systemic amyloidosis. But localised valvular amyloid deposits are very uncommon. It was only in 1922 that the cases of valvular amyloidosis were reported. Then in 1980, Goffin et al reported another type of valvular amyloidosis, which he called the dystrophic valvular amyloidosis. We report a case of aortic valve amyloidosis which...

Primary systemic amyloidosis (AL) is an uncommon disease characterized by the extracellular deposition of a protein with a beta-fibrillar structure, consisting of monoclonal immunoglobulin light chains, lambda or kappa (ratio of lambda to kappa, 3:1). In systemic amyloidosis liver involvement is frequent but it rarely has clinical importance. The massive and localized liver deposition of amyloi...

Primary gastric lymphoma, an uncommon gastric tumor caused by infection with Helicobacter pylori, is rarely associated with gastric amyloidosis. Chronic bacterial infection is known to cause amyloidosis. We report a 53-year-old man who had an antral and duodenal mass with narrowing and ulceration on endoscopy and CT scan; endoscopic biopsy revealed gastric amyloidosis. Rapid urease test and ser...

INTRODUCTION The aim of this study was to develop an empirical approach to classifying patients with transthyretin amyloidosis (ATTR) based on clinical signs and symptoms. METHODS Data from 971 symptomatic subjects enrolled in the Transthyretin Amyloidosis Outcomes Survey were analyzed using a latent class analysis approach. Differences in health status measures for the latent classes were ex...

INTRODUCTION Primary localized laryngeal amyloidosis is an extremely rare condition. It usually presents with hoarseness, pain and/or difficulty in breathing. CASE PRESENTATION We present the case of a 23-year-old woman with primary localized laryngeal amyloidosis who presented with hoarseness and dysphagia. CONCLUSION A search of PubMed shows that dysphagia in patients with laryngeal amylo...

Cutaneous amyloidosis is a rare disease characterized by the deposition of amyloid in the dermis. It can be primary or secondary, depending on associated diseases. It has been linked to various autoimmune diseases, including primary biliary cirrhosis. We present the case of a patient with an autoimmune hepatitis-primary biliary cirrhosis overlap syndrome with concomitant cutaneous amyloidosis, ...

Primary pulmonary amyloidosis is a relatively rare condition, characterized by amyloid deposition in the lungs and other associated structures. We report a case of primary nodular parenchymal pulmonary amyloidosis in a 44-year-old male. The patient was referred to our hospital for further evaluation of multiple lobulated nodules in both lungs. As the multiple lung nodules were suspected to be m...

Amyloidosis is characterized by the extracellular deposition of highly-organized fibrillar aggregates showing a cross-beta super-secondary structure (1). Several proteins are amyloidogenic in humans, resulting in different clinical presentations, either systemic or localized. Transthyretin-related hereditary amyloidosis (ATTR) is a late-onset, dominantly inherited systemic amyloidosis. Heterozy...

Amyloid fibrils from two cases of cancer-associated, systemic amyloidosis with renal cell carcinoma and mesothelioma as the respective underlying disorders were studied. The immunochemical studies suggested strongly that amyloid A comprised a principal fibril component in both cases of cancer-associated amyloidosis. This was definitively proven by amino acid sequence analyses, which revealed st...

Amyloidosis is an uncommon disease that results from the extracellular deposition of abnormal fibrillary protein. This paper reports a case of primary amyloidosis with predominant involvement of the gastrointestinal tract and heart as a mimicker of Crohn's disease in a sixty-seven years old man admitted with repeated diarrhea and fatigue. This patient poorly responded to 5-aminosalicylic acid a...