Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Amyloidosis is the extracellular deposition of normally soluble autologous protein in a characteristic abnormal fibrillar form. Systemic amyloidosis and some local forms are progressive, cause major morbidity, and are often fatal. No treatment specifically causes the resolution of amyloid deposits, but therapy that reduces the supply of amyloid fibril precursor proteins can improve survival and preserve organ function. Major regression of amyloid occurs in at least a proportion of such cases, suggesting that the clinical improvement reflects mobilization of amyloid. The clearest evidence for regression of amyloid has been obtained in juvenile rheumatoid arthritis patients with AA amyloidosis treated with chlorambucil. This drug suppresses the acute phase production of serum amyloid A protein, the precursor of AA amyloid fibrils, and is associated with remission of proteinuria and greatly improved survival. In many such patients, scintigraphy with serum amyloid P component shows major regression of amyloid over 12 to 36 months and frequently reveals a discrepancy between the local amyloid load and organ dysfunction. Measurement of target organ function is therefore not an adequate method for monitoring treatment aimed at promoting the resolution of amyloid. In monoclonal immunoglobulin light chain (AL) amyloidosis the aim of treatment is to suppress the underlying B-cell clone and, therefore, production of the amyloid fibril precursor protein. This can be difficult to achieve or sustain and, since the prognosis is so poor, many patients die before benefits of therapy are realized. A recent development has been the introduction of liver transplantation as treatment for familial amyloid polyneuropathy caused by transthyretin gene mutations. This leads to the disappearance of variant transthyretin from the plasma and halts progression of the neurologic disease. Features of autonomic neuropathy frequently ameliorate, and improvement in peripheral motor nerve function has been recently reported. Serum amyloid P component scans show regression of associated visceral amyloidosis. This surgical form of gene therapy holds much promise for patients with familial amyloid polyneuropathy and has been widely adopted. The only other form of amyloidosis in which the supply of the fibril precursor protein can be sharply reduced is beta 2M amyloidosis in long-term hemodialysis patients. Renal transplantation lowers the plasma concentration of beta 2M to normal levels and is associated with rapid improvement of the osteoarticular symptoms. Preliminary observations suggest that the beta 2M amyloid deposits also can regress in some patients.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Treatment of amyloidosis. 764 31

A rare case of secondary amyloidosis associated with Castleman's disease is reported. A 53-year-old woman was referred for investigation of proteinuria. Biopsy specimens from kidney and gastric mucosa revealed numerous amyloid deposits, defined as AA amyloidosis by immunohistological staining. Castleman's disease was found in the abdomen as the primary disease for the amyloidosis. Although the urinary protein was somewhat reduced and the inflammatory findings were improved after removal of the lymphoma, renal insufficiency progressed and hemodialysis was begun.
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PMID:Secondary amyloidosis associated with Castleman's disease. 772 77

Approach to the management of AA amyloidosis complicating RA. (A) In case of proteinuria or loss of renal function a rectal biopsy or a subcutaneous fat biopsy is a suitable screening method for the detection of amyloidosis. If in any doubt, try to ascertain the diagnosis by renal biopsy. Adequate staining with alkaline Congo red and preferably immunohistochemical staining with anti-AA antibodies should be performed. Beware of renal pathology other than amyloidosis even in the presence of a positive rectal biopsy. (B) A vigorous attempt to control disease activity of the RA should be made in order to eliminate the production of SAA, an acute phase protein. The response to treatment should be monitored by serial measurements of CRP and preferably SAA. (C) The function of some vital organs should be evaluated: (a) endogenous creatinine clearance and the extent of proteinuria; (b) electrocardiogram and optional echocardiography; (c) thyroid function and adrenocortical function; (d) intestinal absorption tests; (e) optional--SAP scintigraphy and turnover studies. (D) Attention should be given to adequate supportive treatment: (a) blood pressure control; (b) treatment of intercurrent infections; (c) corticosteroids during major surgical procedures; (d) pay attention to the possible effect of NSAID on proteinuria and renal function. (E) In case of total renal failure or uncontrollable proteinuria: (a) consider the possibility of primary renal transplantation; (b) otherwise regular haemodialysis is indicated.
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PMID:Clinical and therapeutic aspects of AA amyloidosis. 795 68

Secondary amyloidosis is a well recognised complication of rheumatic diseases, but the association with systemic lupus erythematosus is very unusual. We report the case of a patient with systemic lupus erythematosus who developed proteinuria due to renal amyloidosis. We also review the cases previously reported and the hypothesis on the pathogenic mechanisms.
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PMID:When does amyloidosis complicate systemic lupus erythematosus? 856 38

Secondary or reactive (AA) amyloidosis is a well-known complication of certain rheumatic diseases, particularly rheumatoid arthritis (RA). This case report describes a patient with RA complicated by amyloidosis and the nephrotic syndrome, which regressed after treatment with azathioprine. The AA amyloidosis was documented by renal and lymph node biopsies and by the presence of proteinuria. Evaluation for other etiologies of proteinuria was negative. After treatment with azathioprine, the proteinuria resolved and the serum albumin level increased from 1.9 mg/dl to normal. This is the first published report of azathioprine therapy resulting in a reversal of the nephrotic syndrome in a patient with RA and secondary amyloidosis.
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PMID:Regression of the nephrotic syndrome in rheumatoid arthritis and amyloidosis treated with azathioprine. A case report. 891 20

A 20 year old male patient with a four-years-history of moderately active ulcerative colitis was admitted due to extensive generalized edema. He suffered from a nephrotic syndrome, which was caused by secondary renal amyloidosis due to the colitis. Under continuous low dose therapy with colchicine in addition to the usual colitis therapy proteinuria decreased from values above 10 g/die to about 3 g/die and edema disappeared completely. Secondary amyloidosis following inflammatory bowel disease is a rare event, in case of ulcerative colitis a true curiosity. Cases published so far are discussed.
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PMID:[Secondary amyloidosis in ulcerative colitis--successful treatment with colchicine]. 904 37

Systemic AA amyloidosis is a serious and potentially fatal complication of Crohn's disease. Proteinuria is the most common presentation, but the diagnosis can only be confirmed by showing amyloid in the tissues and is often delayed. Recently, scintigraphy has been developed using 123I-serum amyloid P component (SAP) as a noninvasive and quantitative alternative to histology in which this nuclear medicine tracer specifically targets amyloid deposits in vivo. This study investigates 4 patients with Crohn's disease who, despite having only low-grade clinical activity, developed progressive AA amyloidosis and renal failure. One patient died while receiving hemodialysis, but in the other 3 cases, renal transplantation and standard antirejection therapy were associated with remission of inflammatory activity. Serial SAP scintigraphy showed regression of amyloid in 2 of these patients and absence of progression in the other. These case studies show the dynamic but variable behavior of AA deposits in different patients and show the use of radiolabeled SAP for diagnosis and monitoring of amyloidosis in Crohn's disease.
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PMID:Long-term survival in systemic amyloid A amyloidosis complicating Crohn's disease. 1057 78

Secondary amyloidosis without a known cause, diagnosed antemortem in a patient with idiopathic myelofibrosis, is reported here. This is the first such case, to our knowledge. Amyloid deposits were seen in the bone marrow, renal glomeruli and jejunum. Reasons for investigating for amyloidosis were hypogammaglobulinemia, proteinuria and recurrent diarrhea.
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PMID:Secondary amyloidosis in the course of idiopathic myelofibrosis. 939 8

Although systemic AA amyloidosis complicating Crohn's disease has been found in 0.5 to 6% in America and Europe, it is relatively rare in Japan. We report a case of systemic AA amyloidosis complicating Crohn's disease. In 1979, a 26-year-old Japanese man presented with diarrhea, melena and perianal abscesses, and was diagnosed as having Crohn's disease. He was treated with oral prednisolone, salazosulfapyridine and diet therapy. However, the gastrointestinal symptoms recurred and he was hospitalized several times. In 1991, his thyroid gland was found to be swollen, but with normal thyroid function, and his thyroid gland became larger subsequently. In October 1995, he showed renal dysfunction (blood urea nitrogen 33.2 mg/dl; serum creatinine 1.5 mg/dl) with proteinuria. His renal function had been deteriorating rapidly. On September 13, 1996, he was admitted to the Tsukuba University Hospital. At the time of admission, his renal function showed a blood urea nitrogen of 129.5 mg/dl with a creatinine of 5.4 mg/dl. The urine contained 0.8 g of protein per 24 hours. He presented with diarrhea for several days before admission and was treated with central venous hyperalimentation. Despite supportive care, he developed end-stage renal failure, then hemodialysis was initiated on October 7. His condition was complicated by a complete auriculoventricular block on October 18. He died of hemoperitoneum on October 25. On postmortem examination, extensive amyloid deposits were found in multiple organs including kidneys, intestine, heart, thyroid gland, lungs, liver, spleen, pancreas, gall bladder, adrenal glands, testis, prostate, bone marrow and parathyroid glands. Analysis of amyloid protein in the autopsy specimens showed type AA.
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PMID:[A case of systemic AA amyloidosis complicating Crohn's disease]. 965 12

A 55-year old female with rheumatoid arthritis (RA) had presented with proteinuria since July 1996. She was referred to us for persistent edema on her face and legs in November 1996. On admission, her 24-hour urinary protein excretion was 4.4 g/day, total serum protein level was 5.3 g/dl, and serum level of amyloid A protein (SAA) was elevated to 45.8 mg/ml. A percutaneous renal biopsy was performed, and light microscopy revealed varying degrees of amyloid deposits in the mesangial areas and arteriolar walls. The diagnosis of secondary amyloidosis (AA amyloidosis) was based on immunohistochemical staining for amyloid A protein using monoclonal antibody against SAA. Four weeks after treatment with salazosulfapyridine (SASP) and dipyridamole, proteinuria began to decrease and the edema had disappeared. Finally she recovered from nephrotic syndrome. AA amyloidosis has been thought to have a poor prognosis, with progression to renal failure. Since there is no specific effective therapy for the disease, it is very important to reduce the activity of the underlying cause. In our patient with renal amyloidosis following RA, SASP was evidently effective for arthritis and improvement of renal function. SASP might have a beneficial effect on AA amyloidosis by suppressing inflammatory cytokines.
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PMID:[Beneficial effect of salazosulfapyridine (SASP) in a patient with secondary renal amyloidosis]. 980 22


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