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)

A patient developed recurrent IgM proliferative glomerulonephritis and a nephrotic syndrome following HLA-identical living donor renal transplantation. Two intensive five-day courses of plasma exchange were followed by sustained reduction of proteinuria. Renal function has remained normal at all times. Immune complex sizing revealed a high titer of middle range complexes (mol. wt. 1 x 10(6) daltons app.); immune complex clearance following an antigen load was not improved by plasma exchange suggesting no alteration of reticulo-endothelial function. Possible mechanisms of benefit are discussed.
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PMID:Reduction of post-transplant proteinuria due to recurrent mesangial proliferative (IgM) glomerulonephritis following plasma exchange. 702 69

An unusual familial glomerular disease, characterized by the presence of diffuse round mesangial deposits of C3, is described in 2 siblings (1 male and 1 female) and their mother. The clinical picture in the 3 patients was a long-lasting proteinuria. An acute hemolytic uremic syndrome with malignant hypertension developed in the male at the age of 24 years, requiring bilateral nephrectomy. The glomerulonephritis recurred on a renal allograft. This disease is not HLA-linked and no characteristic abnormality of complement profile was seen in the 3 patients.
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PMID:A new form of familial glomerulonephritis. 704 14

In a series of 60 patients with idiopathic membranous nephropathy (IMN), 8 subjects, aged 16-65 years at presentation, suffered spontaneous relapse of proteinuria after remissions of 25 months to 30 years. Renal biopsy was performed at the time of relapse in 5 cases and revealed histopathology identical to that of the original lesion. Eight courses of immunosuppressive therapy given to 6 patients did not affect either the appearance or duration of remission or relapse. No patient had a familial tendency to renal disease. Immunogenetic markers, HLA A, B and DR, did not distinguish those who relapsed from other patients with IMN. At the end of the study, 3 patients were in a second remission, one had died of myocardial infarction during relapse, 3 remained nephrotic and one had mild renal insufficiency but no proteinuria. As compared with the rest of the series the overall prognosis was not influenced by the relapses.
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PMID:Relapsing idiopathic membranous nephropathy. 715 46

Three of five siblings developed a steroid-resistant nephrotic syndrome with focal segmental glomerulosclerosis within a four-month period. Two of the siblings with nephrotic syndrome (Patients 1 and 2) also have sickle cell anemia; the third (Patient 3) carries the thalassemia trait. The dizygotic twin brother of Patient 2 has sickle cell anemia, but does not have the nephrotic syndrome. The nephrotic syndrome of patient 1 was resistant to corticosteroid and cyclophosphamide therapy and she developed severe renal failure 14 months after onset. The nephrotic syndrome of Patients 2 and 3 was steroid resistant but was partially responsive to cyclophosphamide therapy. They have persistent proteinuria with mild elevation of serum creatinine concentration and hypertension 5 1/2 years after diagnosis. In this family, the nephrotic syndrome appeared unrelated to the specific hemoglobinopathy, HLA type or mixed lymphocyte culture responsiveness despite the similarity of the renal disease.
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PMID:Familial nephrotic syndrome and focal segmental glomerulosclerosis. 719 18

Twenty-five patients with rheumatoid arthritis treated with D-penicillamine were retrospectively reviewed for signs of drug intolerance. Nine patients (36%) developed adverse drug reactions, the most common of which was proteinuria in six patients (24%). Comparative analysis of patients with and without penicillamine-induced proteinuria revealed the only significant correlate to be a previous history of gold nephropathy. Five (83%) of six patients who developed penicillamine-induced proteinuria had had gold-induced proteinuria; in contrast, only three (20%) of 19 who tolerated penicillamine had prior gold-induced proteinuria (p less than .01). HLA typing performed in five of the six patients with penicillamine nephropathy revealed DRw4 at a prevalence less than that expected for a population with rheumatoid arthritis, with DRw3 and/or B8 present in four patients. Further studies are in progress to determine whether a genetic predisposition is present. These data suggest that cautious observation is warranted in rheumatoid patients receiving D-penicillamine who have a prior history of gold nephropathy.
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PMID:The relationship between D-penicillamine--induced proteinuria and prior gold nephropathy. 720 1

Glomerulonephritis with mesangial deposition of IgA was diagnosed in 31 patients. In Spain this disease is the second more frequent primary glomerulonephritis, representing 27% of them. One out of every four patients with IgA mesangial glomerulonephritis ends the clinical course in renal failure. Such bad evolution might be predicted by the existence of arterial hypertension, severe proteinuria, degree of glomerular sclerosis, presence of HLA Bw35, and increased polymeric IgA in serum. The present pathogenetic concepts are reviewed.
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PMID:[IgA mesangial glomerulonephritis (author's transl)]. 725 41

Fifty-five renal allografts (44 from living-related and 11 from cadaver donors) that have functioned for at least 20 years (mean 22.9 +/- 2.3, range 20.1 to 30.7 years) were evaluated in three groups based on renal function: group I (n = 26), with a GFR of > or = 60 ml/min/1.73 m2 or serum creatinine < or = 1.4 mg/dl and no proteinuria; group II (n = 9), with a GFR of > or = 60 ml/min/1.73 m2 or serum creatinine < or = 1.4 mg/dl but > 150 mg proteinuria/24 hr; and group III (n = 20), with a GFR < 60 ml/min/1.73 m2 and/or serum creatinine > 1.4 mg/dL with or without proteinuria. Allograft factors, including acute rejection (AR) in 62% (34/55) and delayed function (DF) in 55% (6/11) of the cadaver grafts, did not preclude 20-year success and the prospect of continued survival since they were not significantly more frequent in group I, II, or III. However, AR was confined to a limited period within the first three months posttransplant in 18/18 recipients in groups I and II but only in 7/16 of group III (P = 0.0002). In groups I and II AR was treated with IVMP in 14/18 cases and only 6/16 in group III (P = 0.035). Donor age < or = 50 years and recipient age < or = 40 years each occurred in 87% (48/55) of these transplants. One- or two-HLA haplotype matching was present in 98% (43/44) of living related transplants. Major risks to the recipient were coronary artery disease (11 cases and 3 deaths), malignancy (18 cases and 1 death), and severe infection and hepatitis (35 cases and 3 deaths, 2 of whom also had coronary artery disease). Hypertension occurred in 25 recipients and diabetes mellitus in 12. Potential open-end success was compromised by renal dysfunction in groups II and III, but appeared possible in 12 of the 26 patients in group I. There is no apparent "safe-haven" point of time for immunosuppressed renal allograft recipients, who remain at increased risk for eventual renal allograft dysfunction, as well as cardiovascular, neoplastic, infectious, and metabolic diseases. In order to clarify and standardize the words "long-term," a simple classification of long-term allograft survivals is proposed.
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PMID:The fate of renal allografts functioning for a minimum of 20 years (level 5A)--indefinite success or beginning of the end? A proposed classification of long-term allograft survivals. 748 35

The aetiology and pathogenesis of focal glomerulosclerosis is poorly understood and many conflicting reports suggest HLA locus associations in both familial and non-familial glomerulosclerosis. We report a family in which 4 of 5 sisters developed proteinuria, 2 with hypertension and 1 progressing to end-stage renal failure (index case). Three underwent renal biopsy which displayed characteristic features of focal glomerulosclerosis and all shared the HLA alleles HLA-A1, B8, DR3, DR7. The index case received two cadaveric renal transplants from HLA-A1, B8, DR3 donors and developed chronic rejection with no histological evidence of recurrent glomerulonephritis in either kidney. The frequency of this haplotype in the Australian dialysis and transplant population with focal glomerulosclerosis was compared to that seen in the general Australian Caucasian population and was not significantly different suggesting that the presence of the HLA alleles HLA-A1, B8, DR3, DR7 may increase the predisposition to familial glomerulosclerosis but additional factors are required for disease development and progression.
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PMID:Familial focal glomerulosclerosis: a genetic linkage to the HLA locus? 750 47

Allotransplantation of solid organs transfers passenger leucocytes which may give rise to a state of persistent microchimaerism. In this report we describe the case of a patient who developed a solitary plasmacytoma in a transplanted kidney more than 10 years after allografting. The diagnosis was established on the basis of the presence of a monoclonal IgG kappa peak in the serum, and light chain proteinuria, the plasmacytoid features of tumour cells including cell surface expression of IgG, kappa light chains, CD20, CD38 and CD56, the absence of lytic bone lesions and a normal bone marrow biopsy, and the disappearance of the monoclonal IgG peak after graft nephrectomy. A donor origin of the tumour was established by HLA DNA typing of tumour, tumour-free kidney tissue, and peripheral blood leucocytes, respectively.
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PMID:A solitary plasmacytoma of donor origin arising 14 years after kidney allotransplantation. 757 26

Hereditary interstitial nephritides are a heterogeneous group of disorders comprising medullary cystic disease, several varieties of Alport's syndrome and also one familial disorder with a distinct clinical syndrome and without characteristic ultrastructural glomerular basement membrane changes. Our family consisted of 11 members, 5 of which presented with renal dysfunction of varying degrees. Clinically, the affected siblings presented with long-standing hypertension, minimal proteinuria and no hematuria. All known causes of a secondary diffuse interstitial nephritis, Alport's syndrome and medullary cystic disease have been excluded. An HLA association is suggested between the affected and unaffected members of the family. Renal biopsy subsequently showed the typical features of a chronic interstitial nephritis without basement membrane changes.
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PMID:Hereditary interstitial nephritis without basement membrane changes. 777 6


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