1/193. Primary perireticulin amyloidosis in a 14-year-old girl.A primary perireticulin amyloidosis is reported in a 14-year-old girl, which showed the symptoms of a steroid-resistant nephrotic syndrome. The diagnosis was established by biopsies of kidney and rectum. Occurrence of amyloidosis in childhood and the clinical picture are discussed and references to clinical trials carried out are given.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
2/193. Tc-99m HDP uptake in cardiac amyloidosis.amyloidosis is characterized by the soft-tissue deposition of amyloid protein. It may occur as a primary disorder but more often is seen as a manifestation of chronic illness. Scattered reports of the affinity of amyloid for bone scanning agents have appeared over many years. Isolated cardiac uptake of Tc-99m HDP is described in a patient with biopsy-proved cardiac amyloidosis on a background of tuberculosis, prostate cancer, and coronary artery disease.- - - - - - - - - - ranking = 0.031407003270596keywords = cancer (Clic here for more details about this article) |
3/193. Striking cholestatic liver disease: a distinct manifestation of advanced primary amyloidosis.In patients with systemic amyloidosis, amyloid fibrils are typically deposited in numerous organs, including the kidneys, heart, and liver. Although amyloid deposition in the liver is common in patients with systemic amyloidosis, clinical liver disease is relatively rare. The patient described here had cholestatic liver disease as the primary manifestation of primary systemic amyloidosis. review of the literature suggests that prominent liver disease with cholestasis is unusual but probably underreported in patients with amyloidosis and appears to be restricted to patients with the primary form of amyloidosis. Nonetheless, cholestatic hepatic amyloidosis is characterized by distinct clinical, laboratory, and pathologic features; recognition of this process is critical because it identifies patients with widespread organ involvement and portends a poor prognosis.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
4/193. Progression of cardiomyopathy and neuropathy after liver transplantation in a patient with familial amyloidotic polyneuropathy caused by tyrosine-77 transthyretin variant.Familial amyloidotic polyneuropathy is an inherited form of amyloidosis associated with a mutant form of a protein called transthyretin. The methionine-30 variant is the most frequent mutation observed. This disorder is caused by deposition of this protein as amyloid in several organs, such as the heart, kidneys, and peripheral nervous system. The disease is always progressive and fatal, and patients die 7 to 10 years after the onset of symptoms. liver transplantation is at present the only choice for these patients because it provides improvement of symptoms and/or stops progression of the disease in most patients. We report the case of a patient who showed clear progression of cardiomyopathy and neuropathy after liver transplantation.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
5/193. Amyloid deposition in renal angiomyolipoma.An irregular tumor shadow, seen on the left kidney in a 48-year-old woman by computed tomography, was pathologically diagnosed as 'angiomyolipoma'. HMB-45 immunoreactivity distinguished angiomyolipoma from renal cell carcinoma of sarcomatoid type. The amyloid deposition was limited to the tumor. M-protein and Bence-Jones protein were negative. For amyloid protein characterization, immunohistochemical studies were performed with antiamyloid A, anti-kappa, anti-lambda, anti-prealbumin and anti-beta-2 microglobulin, but none reacted with the amyloid. This is the first documented case of amyloid deposition in angiomyolipoma and may represent a novel precursor protein of amyloid.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
6/193. Benign monoclonal gammopathy turning to AL amyloidosis after kidney transplantation.The fate of preexisting benign monoclonal gammopathy after organ transplantation is largely unknown. We report the case of a 47-year-old male kidney graft recipient with a pretransplantation IgG kappa monoclonal gammopathy who developed, 10 years after transplantation, de novo augloid light chain (AL) amyloidosis involving skin and kidney graft. The potential role of heavy immunosuppressive treatment in the development of this complication is discussed. The possible occurrence of AL amyloidosis should be kept in mind when a patient with benign monoclonal gammopathy is evaluated for organ transplantation, as well as when a transplanted patient with pre-existing monoclonal gammopathy develops new onset of proteinuria.- - - - - - - - - - ranking = 6keywords = kidney (Clic here for more details about this article) |
7/193. A case of amyloid goiter secondary to Crohn's disease.We herewith report a case of amyloid goiter secondary to Crohn's disease. The patient had been diagnosed as having Crohn's disease at the age of 15, and underwent right hemicolectomy at age 20. When he was 26 years old he complained of swelling of the anterior neck. Both TSH and thyroid hormones were within the normal range, and anti-thyroglobulin and anti-microsomal antibodies were negative. Only thyroglobulin was noticeably above the normal range. During the next year his goiter enlarged further and because he had a feeling of pressure he underwent total thyroidectomy. The presence of amyloid A protein in his surgical specimen led to the diagnosis of amyloid goiter. Although most cases of secondary amyloidosis are known to develop in neoplasms or chronic inflammatory diseases, our patient had no illness other than Crohn's disease. Perusal of literature revealed that Crohn's disease is rarely a cause of amyloid goiter.- - - - - - - - - - ranking = 0.00587673872242keywords = neoplasm (Clic here for more details about this article) |
8/193. A patient with multiple myeloma, amyloidosis and light-chain deposition disease in kidneys with a long survival.We report a case of a 39-year-old man with lambda-light-chain multiple myeloma, nephrotic syndrome due to lambda-light-chain deposition disease in kidneys and amyloidosis in other tissues. The patient was treated with melphalan and prednisone for 1 year. After that, he was administered interferon-alpha2b (IFN-alpha2b; 3 MU, 3 times a week) as maintenance therapy for 2 years. At present, 5 years and 6 months after the initial diagnosis, the patient receives IFN-alpha2b (3 MU, twice a week) and remains in complete haematological remission.- - - - - - - - - - ranking = 5keywords = kidney (Clic here for more details about this article) |
9/193. Acute leukemia following prolonged cytotoxic agent therapy.1. Nine patients in whom acute non-lymphoblastic leukemia (ANLL) developed following prolonged alkylating agent therapy are described. Five of the patients received no radiotherapy. The conditions treated were: Hodgkin's disease (four patients), primary amyloidosis, primary macroglobulinemia, malignant lymphoma, multiple myeloma, and carcinoma of the tonsil. 2. Prior to the advent of chemotherapy, this complication was not observed in large series of patients with lymphoproliferative disorders and multiple myeloma. However, the medical literature now contains at least 125 other detailed reports of ANLL developing after prolonged cytotoxic agent therapy. 3. multiple myeloma and Hodgkin's disease, both of which commonly have good responses to chemotherapy, predominate as the underlying diseases. However, 35% of the case reports involve patients with other illnesses, including 12 patients who did not have neoplasms. 4. More than half of the patients developing ANLL have received chemotherapy alone without radiotherapy. 5. At least half of the patients developing ANLL experienced long periods of significant cytopenia during therapy, often with documentation of bone marrow dysplasia. 6. The wide variety of drugs associated with this complication suggests that any cytotoxic agent may be leukemogenic. However, alkylating agents overwhelmingly predominate as the class of compounds which are most often associated with terminal ANLL. 7. The vast majority of patients reported in the literature with ANLL complicating underlying malignancies have received cytotoxic drugs for prolonged periods (median 3 1/2 years) and leukemia developed most commonly 3 to 5 years after the diagnosis of the underlying disease. Most of these patients benefited from therapy and survived longer (median 5 years) than historical control of untreated patients. 8. The leukemogenic potential in man of prolonged cytotoxic agents therapy, especially with alkylating agents, seems to be well established. This evidence admonishes against the prolonged use of these drugs in non-fatal disorders. 9. More accurate assessment of risk: benefit ratios awaits the results of prospective controlled studies. The results of these studies could also lead to significant modifications in recommendations for long-term maintenance therapy with cytotoxic agents.- - - - - - - - - - ranking = 0.00587673872242keywords = neoplasm (Clic here for more details about this article) |
10/193. Localized AL amyloidosis of the colon and clinical features of intestinal obstruction. A case report.The term amyloidosis refers to a group of disorders characterized by the extracellular accumulation of insoluble, fibrillar proteins (i.e. amyloid) in various organs and apparatuses. Local deposition of amyloid without systemic involvement is a rather uncommon form that gives rise to an amyloid pseudo-tumor or "amyloidoma". This paper describes the clinical features of an intestinal subocclusion observed in an elderly patient with localized primary amyloidosis of the transverse colon. The endoscopic picture indicated a stenosing neoplasm. Segmentary colectomy, however, followed by histological examination (characteristic green color in polarized light after staining with congo red) and immunohistochemical analysis of the resected tissue, revealed massive deposits of amyloid composed of lambda light chains in the interstitial connective, and perivascular tissue and muscular tunic, and resulted in a diagnosis of AL amyloidosis. This was successfully treated with a combination of melphalan and prednisone.- - - - - - - - - - ranking = 0.00587673872242keywords = neoplasm (Clic here for more details about this article) |
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