Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
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Renal amyloidosis was confirmed in 6 related male and female Beagles, ranging in age from 5 to 11 years. The most commonly reported signs of illness included lethargy, anorexia, vomiting, and weight loss. Common clinicopathologic abnormalities were normocytic, normochromic anemia; hypoalbuminemia; azotemia; hypercholesterolemia; proteinuria; and urine specific gravity values below the normal range. Histologic examination of renal tissue from the 6 Beagles revealed moderate to severe glomerular amyloidosis with inconsistently observed mild medullary interstitial amyloidosis. Congo red-stained kidney sections from 4 of 4 affected dogs were potassium permanganate-sensitive, suggestive of reactive amyloidosis. Hereditary predisposition for renal amyloidosis was suspected in these Beagles.
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PMID:Renal amyloidosis in a family of beagles. 151 31

Renal amyloidosis was diagnosed in 14 young Chinese Shar Pei dogs, all of which were related. Clinical signs were those of renal failure and included vomiting, anorexia, lethargy, polydipsia, polyuria, weight loss, and dehydration. Some dogs had a history of intermittent fever and joint swelling. Laboratory findings also were compatible with renal failure and included azotemia, hyperphosphatemia, low total CO2 content in serum, isosthenuria, proteinuria, and hypercholesterolemia. All dogs had medullary deposition of amyloid, and 9 of 14 (64%) had glomerular involvement. The remaining renal lesions were typical of end-stage renal disease. In some dogs, amyloid deposits were found in other tissues (eg, liver, spleen, stomach, small intestine, myocardium, lymph node, prostate gland, thyroid gland, and pancreas). Amyloid deposits were sensitive to potassium permanganate oxidation, suggesting the presence of amyloid protein AA.
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PMID:Familial renal amyloidosis in Chinese Shar Pei dogs. 221 Dec 93

Renal amyloidosis was diagnosed in 8 related Abyssinian cats. The kidneys were characterized pathologically by medullary interstitial and glomerular amyloid deposition, interstitial fibrosis, and papillary necrosis. Amyloid deposits were birefringent under polarized light after Congo red staining, were thioflavine-T positive, and lost Congo red staining after permanganate oxidation. Four of the cats were evaluated clinically. Two of these cats were terminally uremic, with nonregenerative anemia, azotemia, hyperphosphatemia, metabolic acidosis, mild hyperglycemia, isosthenuria, proteinuria, cylindruria, and mild hematuria. The remaining 2 cats were only moderately azotemic. Three of the cats had severe gingivitis and all 4 cats had hyperproteinemia due to hyperglobulinemia.
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PMID:Renal amyloidosis in related Abyssinian cats. 711 93

Renal amyloidosis is a rare condition in cattle and this report is the first in Iran. A 5-year old Holstein cow was referred because of profuse diarrhoea, ventral oedema and decreased appetite. Physical examination revealed a well audible ping area on the upper half of the right paralumbar fossa. At rectal examination, a large firm and circular shaped kidney was palpated. Laboratory examination revealed hypoproteinaemia and severe proteinuria. At necropsy, the kidneys were enlarged, yellow and waxy. Amyloid deposits were found in glomeruli by microscopic examination of the kidneys, and with Congo red staining renal amyloidosis was confirmed.
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PMID:Renal amyloidosis in cattle: a case report in Iran. 946 6

Renal amyloidosis has been considered rare and late in the evolution of the transthyretin (TTR) familial amyloid polyneuropathy (FAP) of the Portuguese type (type I). Renal biopsy has been performed systematically in 14 patients with FAP type I before liver transplantation. In all patients, TTR Met30 mutation was shown. Seven had proteinuria or abnormal microalbuminuria, whereas seven others had no urinary abnormalities. All had renal amyloid deposition predominantly in the medulla. Glomerular and vascular involvement was more prominent in patients with urinary abnormalities. Patients with the most extensive renal lesions represented a subgroup with a low score of polyneuropathy disability, a high prevalence of nephropathy in the proband generation, or a late onset for relatives with nephropathy. Immunohistochemistry studies showed that the amyloid substance corresponded to transthyretin. We have shown that renal TTR-derived amyloid deposition is common in patients with FAP type I, even in the absence of urinary abnormalities. The clinical presentation of nephropathy is not a late occurrence in the disease.
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PMID:Familial amyloid polyneuropathy type I (Portuguese): distribution and characterization of renal amyloid deposits. 963 37

An 84-year-old man presented with ankle edema, significant proteinuria, and mild impairment of renal function soon after treatment was started for idiopathic myelofibrosis. Renal amyloidosis was found on biopsy. The amyloid deposit was resistant to potassium permanganate treatment and showed no immunoreactivity to immunoglobulin light chains, beta-amyloid protein, or amyloid A component. A review of the literature showed that the occurrence of amyloidosis in idiopathic myelofibrosis is very rare, and the chemical nature of the amyloid involved remains unclear.
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PMID:Amyloidosis complicating idiopathic myelofibrosis. 1058 33

The purpose of this study was to investigate children followed as having both Hodgkin's disease (HD) and nephropathy and discuss the factors which might play roles in the pathogenesis of this association by reviewing the pertinent literature. Our experience among 661 children with HD revealed ten cases (1.5%) with nephropathy; eight of these were biopsy proven. Tissue diagnoses were amyloidosis (AA type) in four cases, and membranoproliferative glomerulonephritis and minimal change glomerulopathy in two cases each. Sex distribution was equal. There was a predominance of the mixed cellular (MC) histologic type in our patients with HD. Nephropathy was shown to antedate the diagnosis of HD in two cases and to herald a relapse in one. In brief, the development of a nephropathy in a patient with HD can be considered as a paraneoplastic phenomenon. Renal amyloidosis may already be present at the time of diagnosis of HD and must be kept in mind as a cause of proteinuria due to preexisting nephropathy. Developing renal paraneoplastic syndrome, even in early-staged HD, in children, may be a poor prognostic factor.
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PMID:Hodgkin's disease and renal paraneoplastic syndromes in childhood. 1093 75

We report a 25-year-old Korean woman with Adult onset Still's disease (AOSD) presented with renal amyloidosis, which had developed four years after disease onset. We successfully treated her with prednisolone, colchicine and cyclophosphamide. A review of the literature uncovered about 10 cases, most of which were treated by various regimens that resulted in poor outcomes. Renal amyloidosis should be suspected in patients with AOSD who have unexplained proteinuria. Although the mechanism of renal amyloid deposition is not well known, earlier histopathologic diagnosis and choice of regimen may affect prognosis.
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PMID:Secondary renal amyloidosis in adult onset Still's disease: case report and review of the literature. 1099 26

A 65-year-old-woman presented with edema, ascites, proteinuria and abnormal liver function tests. A small amount of mixed cryoglobulin was detected in her serum. Liver biopsy revealed mild chronic active hepatitis, but tests for hepatotropic viral infection were negative. Electron microscopy of the renal biopsy revealed glomerular electron-dense deposits that contained numerous tubular structures. Renal amyloidosis and light chain deposition disease were ruled out by appropriate histological techniques. The ultrastructural findings of renal biopsy suggested either cryoglobulinemic glomerulonephritis or immunotactoid glomerulopathy. Although the exact interrelationship among the peculiar glomerulopathy, cryoglobulinemia and chronic active hepatitis in the present case remains undetermined, this report enlarges the spectrum of glomerulopathy characterized by extracellular deposition of microtubules.
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PMID:Glomerulonephritis with microtubular deposits associated with cryoglobulinemia and chronic active hepatitis. 1216 8

A retrospective study of 316 patients, who underwent renal biopsy, during the period of 1995-1999 in five nephrological centers of Lithuania. All renal biopsy materials were investigated in the State Center of Pathology. Male:female ratio was 204:112, the mean age 41.4 (SD 16.7) yrs., range 15-80. The main indications for renal biopsy were nephrotic syndrome (29.1%), hematuria and nonnephrotic proteinuria (27.8%). The leading type of kidney damage was primary glomerulonephritis--194 (69.3%), which was 2.4 times more frequent in males than in females. The dominant types of primary glomerulonephritis were IgA nephropathy--30.4%, membranoproliferative glomerulonephritis--26.8%, membranous nephropathy--10.3% and focal segmental glomerulosclerosis--9.8%. Renal amyloidosis was found even in 8.6% of all renal biopsies.
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PMID:[The indications of renal biopsies and spectrum of renal diseases in five nephrological centers of Lithuania (a five-year study)]. 1276 13


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