A case of generalized amyloidosis associated with cyclic neutropenia is presented. A 24-yr-old female with cyclic neutropenia died from intestinal obstruction caused by necrosis and perforation of the small intestine. Post-mortem examination revealed generalized amyloidosis involving almost all organs. Amyloid deposits were prominent, especially in the alimentary tract, kidneys, spleen, and sma...

Urethral amyloidosis is a rare condition in which eosinophilic amyloid proteins are deposited in the urethra. Only a small number of reports on urethral amyloidosis have been published. Increased interest has been associated with this disease due to its clinical similarities with urothelial carcinoma. A biopsy of the lesion and a histological examination are essential for the correct diagnosis....

There is no specific therapy for primary amyloidosis, and acquired generalised amyloidosis can be treated only if the underlying disease is eliminated. In this study we have investigated the role of colchicine therapy in primary amyloidosis, and dimethylsulphoxide (DMSO) in leprosy associated secondary amyloidosis. No effect on creatinine clearance or 24 h proteinuria could be observed in the p...

Systemic amyloid light chain (AL) amyloidosis (previously known as primary amyloidosis) is a multisystem disease characterized by the extracellular deposition of fibrils composed of immunoglobulin light chains within various organs. AL amyloidosis is associated with different types of monoclonal plasma cell dyscrasias, including multiple myeloma and other monoclonal gammopathies.1 Cardiac invol...

purpose: controversy has recently risen about the presence of compensatory ocular countertorsion (coct) after head tilt. this study was performed to define the functional range of this phenomenon. methods: cycloplegic autorefraction was performed on 80 eyes with regular astigmatism 2d. objective autorefraction was performed in normal position, right and left head tilt positions of 5º, 10º, 15º,...

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...

Primary localized cutaneous amyloidosis (PLCA) is caused by the extracellular deposition of amyloid material in the skin without other cutaneous or systemic organ involvement. PCLA is classified into lichen, macular,and nodular amyloidosis. Macular amyloidosis and lichen amyloidosis are named as biphasic amyloidosis when they are concurrently seen in a patient. The treatment of this disease is ...

Cardiac involvement occurs frequently in primary amyloidosis and is associated with heart failure hospitalizations and poor survival. The initial presentation of the disease may be misleading, resulting in under-diagnosis of cardiac amyloidosis and late initiation of treatment. We present a case of cardiac amyloidosis initially misdiagnosed as hypertrophic cardiomyopathy and we discuss the key ...

Background Systemic amyloidosis is a disorder characterized by extracellular deposition of different insoluble protein fibrils in various organs leading to organ dysfunction. Cardiac involvement is associated with limited survival. We aimed to use cardiac magnetic resonance imaging (CMR) to identify structural and functional alterations related to the different forms of amyloidosis, e. g. hered...

Globular amyloidosis is a very infrequent amyloidosis subtype, characterized by the deposition of rounded bodies of protein, occasionally perivascular instead of the usual linear deposits. The most frequently affected organ is the liver, although other organs can also been involved. To date, only eight cases (1-3) of globular amyloidosis of the gastrointestinal tract have been described. Clinic...