Introduction Renal biopsy series from North America suggest that leucocyte chemotactic factor 2 (ALECT2) amyloid is the third most common type of renal amyloid. We report the first case series from a European Centre of prevalence, clinical presentation and diagnostic findings in ALECT2 amyloidosis and report long-term patient and renal outcomes for the first time. Methods We studied the clini...

Amyloidosis of urinary bladder is a rare condition and may be primary or secondary in nature. A case of primary localized vesical amyloidosis (VA) in a 40-yr-old man is described confused with neoplasm by cystoscopic, urographic. Surgical specimens obtained by transurethral resection (TUR) were diagnostic and histologically revealed amyloid deposits in sub-epithelial stroma with chronic inflamm...

Systemic amyloidosis is recognized as a serious complication of rheumatoid arthritis or inflammatory bowel disease, but also skin disease. However, the detailed molecular mechanism associated with cutaneous inflammation remains unclear, and therapeutic approaches are limited. Here, we investigated pathophysiology secondary to effects Janus kinase (JAK) inhibitors by examining mouse model sponta...

The liver has become an increasingly interesting target for oligonucleotide therapy. Mutations of the gene encoding transthyretin (TTR), expressed in vast amounts by the liver, result in a complex degenerative disease, termed familial amyloid polyneuropathy (FAP). Misfolded variants of TTR are linked to the establishment of extracellular protein deposition in various tissues, including the hear...

Background Amyloidosis is characterized by accumulation of insoluble fibrils composed different monomers in extracellular spaces organs, and demonstration deposits non-invasive methods important especially for organs difficult to sample. Transient elastography (Fibroscan) a diagnostic method measuring liver stiffness (LS) being used chronic diseases. Objectives We herein aimed search potential ...

An adult swan goose (Anser cygnoides) kept in a zoological garden had gross hepatic enlargement with extensive ill-defined white foci. Microscopically, the hepatic lesions were composed of a mixture of adipocytes and myeloid cells. The goose was also affected with systemic amyloidosis and there were areas of osseous metaplasia associated with deposition of amyloid within the liver.

Two cases of pure congenital dilatation of the intrahepatic bile ducts are presented. One patient developed amyloidosis secondary to suppuration, and had isolated cysts in the renal medulla. The position of congenital dilatation of the intrahepatic bile ducts compared with cysts in the liver and its association with ;cystic' diseases of the kidneys is discussed.

Transudative chylothorax is a rare type of pleural effusion. It has been described to be present in the setting of liver cirrhosis, heart failure, amyloidosis, nephrotic syndrome, superior vena cava thrombosis, sclerosing mesenteritis and heart failure. We present the case of an 86-year-old woman with a right-side transudative chylothorax associated with heart failure and lymphangioleiomyomatosis.

To the Editor: Systemic amyloidosis is characterized by the extracellular deposition of misfolded proteins as insoluble amyloid fibrils in various tissues. The familial forms of amyloidosis (AF) comprise a group of autosomal dominant diseases associated with mutations in a number of genes encoding amyloid precursor proteins. These diseases collectively exhibit various phenotypes, including ages...

PURPOSE Assessment of ocular involvement in transthyretin-related familial amyloidosis with polyneuropathy (FAP) in a large cohort of Portuguese patients. METHODS We reviewed the medical records of 513 Portuguese FAP mutation carriers, at the Ophthalmology Service, Centro Hospitalar do Porto, between 1 January 2008 and 31 January 2013. Abnormal conjunctiva vessels (ACV), Schirmer test, tear b...