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...

Background Gastrointestinal (GI) complications such as constipation, diarrhea and gastroparesis are common in hereditary transthyretin (ATTR) amyloidosis. The mechanisms behind these disturbances have not been fully elucidated and the patients’ small bowel function remains largely unexplored. The aim of the present study was to compare the small bowel motility in patients with hereditary ATTR a...

Introduction AA amyloidosis may develop as a consequence of chronic inflammatory conditions including inherited periodic fever syndromes. Mevalonate-kinase (MVK) deficiency (MKD) appears to be the least frequent underlying condition after FMF, TRAPS and CAPS. Moreover, amyloidosis rarely manifests during childhood. We report a case of a small child in whom renal biopsy performed because of the ...

The larynx is a rare site of involvement of amyloidosis. We report two cases of laryngeal amyloidosis. Both patients were middle aged females with history of persistent hoarseness. Fibreoptic laryngoscopy used for diagnosis in both and debulking of the tumour was performed. Histopathological examination confirmed the diagnosis of amyloidosis. Appropriate follow up is an important part of the lo...

Here, we report a case of insulin-derived amyloidosis in the lower abdomen. The mass continued to develop even after the patient ceased injecting insulin into the mass. Histological examination led to a diagnosis of insulin-derived amyloidosis. Excision is preferable in cases of insulin-derived amyloidosis if patient's condition permits.

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...

The types of disease associated with secondary amyloidosis have undergone considerable change in recent years. Largely because of modern antibiotic treatment, amyloidosis associated with tuberculosis, osteomyelitis, chronic lung disease, and syphilis is now rarely seen. For this reason rheumatoid arthritis is now one of the commonest causes of secondary amyloidosis. Although amyloidosis may be ...