Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The aetiology, clinical course and affected organs were studied in 124 patients with acquired systemic amyloidosis and seven patients with organ-limited amyloid deposits. Seventy-five patients had reactive systemic AA amyloidosis, which was associated with rheumatic disease in 55 and with chronic infection in 13 cases. Forty-nine patients had systemic AL amyloidosis. Thirteen of these cases were associated with myelomatosis and 11 with non-malignant immunocyte dyscrasias. In 25 patients with systemic AL disease no immunocyte dyscrasia was identified. Renal involvement dominated the clinical course of both forms of systemic amyloidosis, and renal failure was the most common cause of death. Gastrointestinal disturbance and hepatosplenomegaly were found in both AA and AL disease, although differences were noted in the distribution of amyloid protein within rectal biopsies. Amyloid cardiomyopathy, neuropathy and macroglossia were present in patients with AL amyloidosis only. These clinical patterns were reflected by tissue distribution at necropsy in 67 patients.
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PMID:Ten years' experience of an amyloid clinic--a clinicopathological survey. 399 77

Clinical and pathological findings are reported in a Japanese girl who died of secondary amyloidosis associated with juvenile rheumatoid arthritis two years after the onset of symptoms. The patient had intermittent high fever, rheumatoid rash, polyarthralgia, and hepatosplenomegaly. The joints showed the typical histologic changes of juvenile rheumatoid arthritis. Amyloid deposition was found in various tissues; however, remarkable deposition of amyloid was observed in the gastrointestinal tract, especially in the ileum. The amyloid protein in this patient was identified as protein AA using the methods of potassium permanganate treatment and the peroxidase-antiperoxidase unlabeled antibody technique.
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PMID:Amyloidosis associated with juvenile rheumatoid arthritis. 707 94