Abdominal Pain/etiology* ; Amyloidosis/diagnosis* ; Humans ; Immunoglobulin Light-chain Amyloidosis/complications* ; Immunoglobulin lambda-Chains

Renal amyloidosis secondary to anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare. Here, we reported a 77-year-old woman with ANCA-associated vasculitis. Renal biopsy with Masson trichrome staining showed pauci-immune crescentic glomerulonephritis, and electron microscopy showed amyloid deposition in the mesangial area. Immunofluorescence revealed kappa light chain and lambda light chain negative. Bone marrow biopsy revealed no clonal plasma cell. Finally, she was diagnosed as ANCA-associated vasculitis with secondary renal amyloid A amyloidosis.
Female ; Humans ; Aged ; Glomerulonephritis/diagnosis* ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/pathology* ; Antibodies, Antineutrophil Cytoplasmic ; Kidney/pathology* ; Amyloidosis/complications*

No abstract available.
Aged, 80 and over ; Amyloidosis/*complications/diagnosis ; Biopsy ; Dacryocystorhinostomy/methods ; Humans ; Lacrimal Apparatus/*diagnostic imaging/surgery ; Lacrimal Apparatus Diseases/*diagnosis/etiology/surgery ; Male ; Tomography, X-Ray Computed

A 53-year-old woman was admitted with epigastric discomfort and weakness. Laboratory examination at admission showed mild anemia and proteinuria. Esophagogastroduodenoscopy revealed marked mucosal atrophy, diffuse nodularity and granular appearance with mucosal friability. Biopsy was performed on the antrum and body of the stomach. On the next day, the patient began to complain of severe dyspnea, and hypoxia was present on pulse oximetry. Therefore, emergency echocardiography was conducted and it showed restrictive cardiomyopathy along with thrombus in the left atrium. With time, heart failure was aggravated despite intensive management. The result of gastric biopsy revealed amyloid deposits which stained positively with Congo red. On immunohistochemistry study, kappa and lambda chain were present. In addition, kappa chain was significantly elevated in urine and serum on electrophoresis. Although the patient was finally diagnosed as having primary gastric amyloidosis with restrictive cardiomyopathy, her general condition rapidly deteriorated and died at 12th hospital day. When obscure gastric lesion is encountered, performing gastric biopsy is strongly recommended since it be primary gastric amyloidosis. Herein, we present an unusual case of primary gastric amyloidosis.
Amyloidosis/complications/*diagnosis/pathology ; Endoscopy, Digestive System ; Female ; Heart Atria/diagnostic imaging ; Heart Failure/complications/*diagnosis ; Humans ; Immunoglobulin kappa-Chains/blood/urine ; Immunoglobulin lambda-Chains/blood/urine ; Immunohistochemistry ; Magnetic Resonance Imaging ; Middle Aged ; Stomach Diseases/complications/*diagnosis/pathology ; Thrombosis/diagnosis/diagnostic imaging ; Tomography, X-Ray Computed ; Ultrasonography

No abstract available.
Amyloidosis/complications/*diagnosis/pathology ; Colitis, Ulcerative/diagnosis ; Colonoscopy ; Gastrointestinal Hemorrhage/*etiology ; Heart Ventricles/radiography ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged

We report herein a case of intestinal amyloidosis with grave prognosis that caused intractable diarrhea and intestinal pseudo-obstruction, alternately in spite of intensive conservative treatment. A 44-year-old woman was admitted for fever, diarrhea, and crampy abdominal pain which had been continuned during 6 months. Abdomen CT scan showed edematous wall thickening of the small bowel and right colon, and colonoscopic biopsy revealed amyloid deposition in the mucosa. Monoclonal light chains in serum and/or urine were not detected and highly elevated serum amyloid A was shown. In spite of intensive treatment including oral prednisolone and colchicine, diarrhea and intestinal pseudo-obstruction developed alternately, general status rapidly got worsened and died after two months.
Administration, Oral ; Adult ; Amyloidosis/complications/*diagnosis/drug therapy ; Anti-Inflammatory Agents/therapeutic use ; Colchicine/therapeutic use ; Colonoscopy ; Diarrhea/*etiology ; Female ; Humans ; Intestinal Mucosa/pathology ; Intestinal Pseudo-Obstruction/*diagnosis/etiology ; Prednisolone/therapeutic use ; Serum Amyloid A Protein/metabolism ; Tomography, X-Ray Computed ; Tubulin Modulators/therapeutic use

Primary systemic amyloidosis is a relatively rare disease, caused when abnormal extracellular deposition of fibrillary protein builds up in a variety of target organs, such as heart, kidneys, lungs liver, and so forth. The symptoms of the disease are usually vague, while many kinds of auxiliary or laboratory examinations especially pathologic biopsy can provide a clue for the diagnosis. Here we described a case who had purpura-like lesions in the initial stage, followed by progressive malfunctions in the kidneys, the heart, the lungs, as well as the liver. The final diagnosis was primary systemic amyloidosis determined by skin pathologic biopsy. And the disease led to a fatal outcome within three months after the diagnosis.
Amyloidosis ; complications ; diagnosis ; diagnostic imaging ; Female ; Humans ; Middle Aged ; Purpura ; diagnostic imaging ; etiology ; pathology ; Ultrasonography

Aneurysms of the major thoracic veins are rare. They are usually asymptomatic and thus treated conservatively. We report an extremely rare case of rapidly progressing superior vena cava (SVC) aneurysm complicated by thrombosis and acute pulmonary thromboembolism (PTE) with right ventricular dysfunction. Thrombolytic therapy for hemodynamically significant acute PTE was harmful to the patient in the present case, because it induced further thrombosis and mobilization of the thrombi within the aneurysm, subsequently causing de novo PTE. Surgical aneurysmectomy combined with pulmonary artery embolectomy would be a treatment of choice in patients with SVC aneurysm complicated by acute PTE.
Abdominal Pain ; Amyloidosis/complications/*pathology ; Biopsy ; Diagnosis, Differential ; Endoscopy ; Humans ; Inflammatory Bowel Diseases/diagnosis ; Intestine, Small/*pathology ; Lymphoma, B-Cell, Marginal Zone/complications/*pathology ; Male ; Middle Aged

A 62-yr-old man presented with a 5-yr history of intermittent abdominal distention and pain. These symptoms persisted for several months and subsided without treatment. A diagnosis of suspected small bowel lymphoma was made based on plain radiograph and computerized tomogram findings, and he was referred to our institution for further evaluation. Segmental resection of the small intestine was performed and the diagnosis of marginal zone B-cell lymphoma associated with amyloidosis was made. This is the first case of marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) in the small intestine associated with amyloidosis in Korea.
Abdominal Pain ; Amyloidosis/complications/*pathology ; Biopsy ; Diagnosis, Differential ; Endoscopy ; Humans ; Inflammatory Bowel Diseases/diagnosis ; Intestine, Small/*pathology ; Lymphoma, B-Cell, Marginal Zone/complications/*pathology ; Male ; Middle Aged

This study was designed to identify the causes of the development of carpal tunnel syndrome (CTS) associated with end stage kidney disease (ESKD). A total of 112 patients with ESKD, 64 on hemodialysis (HD) and 48 on peritoneal dialysis (PD), were enrolled. The duration of ESKD and dialysis, the site of the arteriovenous (A-V) fistula for HD, laboratory data such as blood urea nitrogen, creatinine, and beta-2-microglobulin were determined. Clinical evaluation of CTS and electrophysiological studies for the diagnosis of CTS and peripheral neuropathy were performed. The electrophysiological studies showed that the frequency of CTS was not different in the HD and PD groups (P = 0.823) and the frequency of CTS was not different in the limb with the A-V fistula compared to the contralateral limb (P = 0.816). The frequency of HD and PD were not related to beta-2-microglobulin levels, an indicator of amyloidosis. The frequency of CTS did not increase as the severity of the peripheral neuropathy and the duration of ESKD and dialysis increased (P = 0.307). The results of this study do not support that microglobulin induced amyloidosis or placement of an A-V fistula are associated with an increase in CTS.
Adult ; Aged ; Amyloidosis/complications ; Arteriovenous Fistula/complications ; Blood Urea Nitrogen ; Carpal Tunnel Syndrome/*complications/*diagnosis ; Creatinine/blood ; Electrophysiological Phenomena ; Female ; Humans ; Kidney Failure, Chronic/*complications/*therapy ; Male ; Middle Aged ; Peritoneal Dialysis/adverse effects ; Polyneuropathies/*complications/*diagnosis ; Renal Dialysis/adverse effects ; beta 2-Microglobulin/blood