objective(s):familial mediterranean fever (fmf), an inherited autosomal recessive disorder, is frequently present among individuals of mediterranean origin. differences in the clinical manifestations of fmf between different ethnic groups have been documented. the aim of the present study was to determine the most common characteristics of fmf and the relationship between clinical findings and ...

Amyloidosis is a disorder of protein metabolism which is characterized by deposition of eosinophilic material intercellularly which has characteristic ultra-structural morphology and biochemical features. Amyloidosis may be primary but more often it is secondary to various inflammatory, neoplastic and suppurative conditions. Inflammatory bowel disease is an unusual cause for secondary amyloidos...

Amyloidosis is a broad and complex class of diseases that comprises several etiologies, many manifestations, and a diversity of outcomes. We discuss a patient with primary systemic amyloidosis associated with multiple myeloma that illustrates many of the typical and atypical features of the disease process. Despite more in-depth assessment and accurate classification, survival for patients with...

We report herein the association of primary pulmonary amyloidosis and ankylosing spondylitis. To our knowledge, this rare association has never been reported. This case reemphasizes that not all pulmonary complications that appear in the course of ankylosing spondylitis are related to the seronegative spondyloarthropathy. Primary pulmonary amyloidosis should be considered in patients with inter...

Background: Waldenstrom macroglobulinemia is a lymphoplasmacytic lymphoma with elevated serum immunoglobulin M and multi-organ involvement. Primary systemic amyloidosis usually develops due to immunoglobulin light chains depositions in different organs due to an underlying gammopathy. Case presentation: Our patient was an 86-year-old man with macroglossia, ecchymotic patches and bullous lesion...

In immunoglobulin light-chain (L) amyloidosis the cause of death is most commonly due to cardiac involvement. Cardiac amyloidosis is frequently diagnosed by postmortem examination. There are many reports of diagnoses of cardiac amyloidosis established by autopsy of cardiac tissue. We report four patients whom we could diagnose as having cardiac amyloidosis by echocardiogram and electrocardiogra...

There are two major forms of amyloidosis, primary amyloidosis (AL) and secondary amyloidosis. AL amyloidosis results from deposition of immunoglobulin light chains or their fragments. One such example is AL amyloidosis associated with multiple myeloma, in which overproduced immunoglobulin light chains get deposited onto tissues, leading to tissue dysfunction. Amyloidosis in the intestines can p...

Amyloidosis comprises a group of diseases that occurs in five to nine cases per million patients per year worldwide irrespective of its classification. Although the hepatic involvement in primary amyloidosis is frequent, the clinical manifestations of liver amyloidosis are mild or even absent. The authors report the case of an aged man who complained of diffuse abdominal pain and marked weight ...

amyloidosis is characterized by an abnormal extracellular deposition of amyloid in different organs, where it usually causes some type of dysfunction. its cause is unknown. five different types of amyloidosis have been described according to the underlying disease; immunoglobulin amyloidosis, familial amyloidosis, senile systemic amyloidosis, secondary amyloidosis and hemodialysis-associated am...

Primary (AL) amyloidosis is the most common form of systemic amyloidosis seen in current clinical practice. The symptoms of the disease are usually vague, special features are seen in fewer than one fifth of patients, and the combination of organs and systems involved provides a clue for the diagnosis. We describe a patient in whom asymptomatic hepatomegaly, cardiomegaly, hyperlipidaemia and el...