Gene/Protein Disease Symptom Drug Enzyme Compound
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 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Amyloidosis is an uncommon plasma-cell dyscrasia with an incidence of eight patients per million per year. It is often difficult to recognize because of the myriad symptoms and vague nature of the clinical presentation. Symptoms include fatigue, dyspnea, edema, paresthesias, and weight loss. Clinical syndromes at presentation include nephrotic-range proteinuria with or without renal insufficiency, cardiomyopathy, hepatomegaly, symptomatic peripheral neuropathy, and autonomic failure. Recent advances have occurred in evaluation of patients by using the free light chain assay and new prognostic assessments with cardiac biomarkers. Newly developed therapeutic strategies, involving high-dose and intermediate-dose chemotherapy, have evolved in the last 3 years. This paper reviews a diagnostic pathway clinicians can use to diagnose the disorder, assess a patient's prognosis, and logically plan a therapeutic strategy.
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PMID:Amyloidosis. 1602 46

Amyloidosis is a rare plasma cell proliferative disorder. The annual incidence in Olmsted County, Minnesota, is 8 in 1,000,000 patients. This is a difficult disorder to diagnose, because the symptoms at presentation are vague and include dyspnea, paresthesias, edema, weight loss, and fatigue. The clinical syndromes at the time of presentation include nephrotic-range proteinuria with or without renal failure, cardiomyopathy, "atypical multiple myeloma," hepatomegaly, and autonomic or peripheral neuropathy. The serum immunoglobulin free light chain assay has been an important step forward in classifying systemic amyloidosis as an immunoglobulin light chain form and in monitoring therapy. Recently, the importance of serum cardiac biomarkers in assessing outcome has been recognized. New therapies developed over the past 5 years include high-dose chemotherapy with stem cell reconstitution, combinations of alkylating agents with dexamethasone, and, most recently, thalidomide.
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PMID:Amyloidosis: diagnosis and management. 1635 26

A descriptive study of one hundred and one pediatric patients with systemic lupus erythematosus treated between July 1985 and March 2003 in Department of Pediatrics, Faculty of Medicine Siriraj Hospital was conducted. According to existing database, there were a total of 181 patients, 101 of them (55.8%) had available data for review. The female to male ratio was 6.2:1. The mean and median ages of onset were 9.7 +/- 2.8 and 10 +/- 2.2 years, respectively (range 4-14 years). The clinical presentations were renal involvement in 87 patients (86.2%), skin and mucocutaneous involvement 77 patients (76.3%), hematological abnormalities 74 patients (73.4%), musculoskeletal involvement 32 patients (31.7%), prolongedfever 24 patients (23.8%), neuropsychiatric symptoms 21 patients (20.8%), gastrointestinal involvement 20 patients (19.8%), cardiac involvement 14 patients (13.9%), lymphadenopathy 13 patients (12.9%), and pulmonary involvement 7 patients (6.9%). The most common renal, skin and mucocutaneous, and hematological manifestations were proteinuria, malar rash, and anemia, respectively. Lupus nephritis with WHO class IV was the most common histopathological finding of the initial renal biopsies. The most common neuropsychiatric, gastrointestinal, cardiac, and pulmonary involvements were seizure, hepatomegaly, pericarditis, and pleuritis, respectively. Ninety-two percent of patients reported as having significant ANA positive results using rat liver tissue as a substrate. Sixty-six out of 94 patients (70.2%) had positive test result of Anti-dsDNA. In conclusion, the age at onset, clinical manifestations and laboratory investigation results of SLE in children at Siriraj Hospital are comparable to other studies in the Country and also to other Asian and Western studies.
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PMID:Pediatric systemic lupus erythematosus in Siriraj Hospital. 1685 52

A 47-year-old man presented with fever of unknown origin, generalized weakness, edema, and renal failure. He had left-sided pleural effusion, generalized lymphadenopathy, multiple nontender cutaneous nodules, hepatomegaly, renal failure, and hypergammaglobulinemia. Axillary lymph node biopsy showed findings consistent with Castleman disease of the hyaline vascular type associated with interfollicular plasmacytosis. A renal biopsy performed in view of proteinuria and acute renal failure showed hypercellular glomeruli with capillary loop thickening and double contours consistent with membranoproliferative glomerulonephritis. Skin nodule biopsy showed a glomeruloid hemangioma characterized by dermal proliferation of capillary loops in a nodular manner resembling a glomerulus. He experienced clinical and biochemical remission with steroids. Discontinuation of steroid therapy was associated with recurrence of renal failure, reappearance of nodules, lymphadenopathy, and appearance of bony lytic lesions. Biopsy of bony lytic lesions showed plasmacytoma. The patient achieved complete remission on treatment with steroids and cyclophosphamide and is free of symptoms at the end of 40 months of follow-up. To our knowledge, this is the first case report of the occurrence of membranoproliferative glomerulonephritis, glomeruloid hemangioma of the skin, and plasmacytoma in a patient with multicentric Castleman disease without human immunodeficiency virus infection.
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PMID:Renal involvement in multicentric Castleman disease with glomeruloid hemangioma of skin and plasmacytoma. 1686 Jan 82

An unusual case having IgM monoclonal gammopathy with clinical and pathologic features of multiple myeloma (MM) in association with neutrophilia and nephrotic syndrome is reported. The patient showed lytic bone lesions, decreased IgG and IgA levels, Bence-Jones proteinuria, nephrotic proteinuria with edema, and histological plasma cell infiltration typical of MM. Moreover, mature neutrophilic leukocytosis, hepatomegaly, high leukocyte alkaline phosphatase score (LAP), absence of Philadelphia (Ph) chromosome and bcr gene rearrangement were also evidenced, all these features representing findings typical of the recently described plasma cell dyscrasia-associated neutrophilia. After the diagnosis, the patient was treated with melphalan and prednisone, with an excellent response to the treatment. Different from the 30 cases so far reported, this is the first case of plasma-cell dyscrasia with associated neutrophilia due to IgM-producing monoclonal gammopathy. At the same time, this is the first reported case of nephrotic syndrome secondary to IgM myeloma.
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PMID:Nephrotic syndrome in a patient with IgM myeloma with associated neutrophilia. 1759 40

Light-chain (AL) amyloidosis is the most common form of systemic amyloidosis and is associated with an underlying plasma cell dyscrasia. The disease often is difficult to recognize because of its broad range of manifestations and what often are vague symptoms. The clinical syndromes at presentation include nephrotic-range proteinuria with or without renal dysfunction, hepatomegaly, congestive heart failure, and autonomic or sensory neuropathy. Recent diagnostic and prognostic advances include the serum free light-chain assay, cardiac magnetic resonance imaging, and serologic cardiac biomarkers. Treatment strategies that have evolved during the past decade are prolonging survival and preserving organ function in patients with this disease. This review outlines approaches to diagnosis, assessment of disease severity, and treatment of AL amyloidosis.
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PMID:Light-chain (AL) amyloidosis: diagnosis and treatment. 1769 66

Amyloidosis is characterized by extracellular deposition of abnormal protein. There are six types: primary, secondary, hemodialysis-related, hereditary, senile, and localized. Primary (AL) amyloidosis is associated with monoclonal light chains in serum and/or urine with 15% of patients having multiple myeloma. Secondary (AA) amyloidosis is associated with inflammatory, infectious, and neoplastic diseases. The presentation is protean, including macroglossia, a dilated and atonic esophagus, gastric polyps or enlarged folds, and luminal narrowing or ulceration of the colon. Amyloid deposition in the gastrointestinal (GI) tract is greatest in the small intestine. The symptoms include diarrhea, steatorrhea, or constipation. Pseudo-obstruction carries a particularly grave prognosis, often not responding to pro-motility agents. Hepatic involvement is common, but the clinical manifestations are usually mild with hepatomegaly and an elevated alkaline phosphatase level. Biopsies to diagnose amyloidosis can be taken from the fat, kidney, intestine, or bone marrow. The safety of liver biopsies is controversial. With Congo Red stain, amyloid appears red in normal light and apple-green in polarized light. Treatment for AL amyloidosis is chemotherapy and stem cell transplantation; treatment for AA amyloidosis is control of the underlying disease. Amyloidosis should be considered in patients with proteinuria, cardiomyopathy, hepatomegaly (with mildly abnormal liver tests), peripheral and autonomic neuropathy, weight loss, and GI symptoms.
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PMID:Gastrointestinal manifestations of amyloidosis. 1972 11

AL amyloidosis is a systemic disease characterized by extracellular amyloid deposition in tissues, causing progressive dysfunction of affected organs. The main clinical syndroms include nephrotic-range proteinuria with or without renal dysfunction, congestive heart failure, hepatomegaly and peripheral or autonomic neuropathy. The aim of therapy is the reduction of monoclonal light chains, by suppressing the underlying plasma cell dyscrasia. Recent therapeutic strategies involve intermediate-dose chemotherapy or high-dose melphalan supported by peripheral blood stem cell transplantation. Alternative therapeutic approaches include thalidomide, lenalidomide, iododoxorubicin, etanercept and rituximab. This paper reviews the pathogenesis, diagnosis and therapy of the AL amyloidosis, focusing on clinico-morphological symptoms of renal involvement, monitoring of treatment response and supportive therapy.
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PMID:[Advances in diagnosis and treatment of AL amyloidosis]. 1863 70

A 12-year-old, neutered male, mixed-breed dog was presented to The Ohio State University Veterinary Teaching Hospital with a history of weight loss and weakness. Laboratory abnormalities reported by the referring veterinarian during the past year included increased alkaline phosphatase (ALP) activity, hyperalbuminemia, and nonregenerative anemia. On referral, the dog appeared hydrated and had moderate muscle wasting and hepatomegaly. A large lobular hepatic mass was observed ultrasonographically. Laboratory results included mild to moderate nonregenerative anemia, urine-specific gravity of 1.035, 3+ proteinuria, increased serum activities of alanine aminotransferase (229 U/L, reference interval 10-55 U/L), ALP (813 U/L, reference interval 15-120 U/L), and the steroid-induced isoform of ALP (676 U/L, reference interval 0-6 U/L), marked hyperalbuminemia (5.3 g/dL, reference interval 2.9-4.2 g/dL), and an increased A/G ratio (1.7). Hyperalbuminemia was confirmed by reanalysis on 2 different analyzers and by agarose gel electrophoresis, and colloid osmotic pressure (COP) was markedly increased (42.5 mmHg, reference interval 20-25 mmHg). Cytologic examination of a fine-needle aspirate of the hepatic mass indicated hepatocellular proliferation; histologic examination of an excisional biopsy confirmed hepatocellular carcinoma. Three weeks after surgery, the albumin concentration, A/G ratio, COP, and ALT activity had normalized, but ALP activities remained high. We hypothesized that hyperalbuminemia developed secondary to hepatocellular carcinoma due to increased synthesis of albumin by malignant hepatocytes or due to decreased negative feedback from impaired hepatocellular osmoreceptivity. Hepatocellular carcinoma has been associated with paraneoplastic secretion of other proteins, but hyperalbuminemia has been reported only once in a human patient and has not previously in dogs.
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PMID:Hyperalbuminemia associated with hepatocellular carcinoma in a dog. 1947 32

The liver is a common site of amyloid deposition in primary systemic amyloidosis. We report the case of a 52-year-old white woman complaining of hepatomegaly, high levels of alkaline phosphatase and serum gamma-glutamyl transferase. Other laboratory tests showed proteinuria with light-chain type lambda. Color Doppler ultrasonography showed an enlarged bright liver with hepatopetal portal blood flow. Fine-needle aspiration biopsy of abdominal fat, with Congo red stain, was positive for amyloid. No liver biopsy was performed, but transient elastography showed high liver stiffness values (75 kPa), suggestive of amyloid infiltration, as other causes of elevation had been ruled out by clinical, laboratory and radiological findings. Bone marrow morphology and immunoistochemistry confirmed low-grade plasmacytoma with amyloidosis.
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PMID:Liver AL amyloidosis as a possible cause of high liver stiffness values. 2104 65


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