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)

Forty-one out of 408 cases (or 10%) of primary glomerular disease had diffuse fine granular to arc-like short linear mesangial deposits of IgM by direct immunofluorescence. The IgM deposition was accompanied by C1q and/or C4 in the same locality in 29 cases, by C3 in 10, and by trace amounts of IgA in 6. Properdin-factor B was not detected. Fine granular electron dense deposits of low density were detected in the mesangium in all 41 cases by electron microscopy, usually as a discrete granular or arc-like pattern beneath the mesangial glomerular basement membrane and correlated well with the immunofluorescence findings. An immune complex disease with complement activation via the classical pathway is suggested. The ages of the patients varied from 2 to 58 years (average 23.8 years). A male predominance of 2.2:1 was identified. Serum IgM level was elevated in 46.7% of the cases. The majority (87.8%) of the cases manifested a nephrotic syndrome or relapse at time of biopsy, and the remaining cases experienced persistent or intermittent proteinuria. Among the 36 nephrotic patients, 22 cases (61.1%) demonstrated complete remission with steroid therapy, 9 cases (25%) were resistant, and 5 cases (13.9%) had partial remission. Complete and partial remissions were later achieved with cytotoxic drugs or methylprednisolone pulse therapy in 3 and 4 cases respectively in the steroid resistant patients. Frequent relapses occurred during the course in 22 out of 32 cases (68.8%) who had experienced complete or partial remission. Follow-up study after biopsy demonstrated that sustained complete remission was achieved with prednisolone with or without cytotoxic drugs and pulse therapy in only 14 (42.4%) of the 33 nephrotic cases who had been followed up for longer than 6 months, and six of them had had previous relapses. Pathologically, 56.1% of the patients showed mild to moderate increase in mesangial matrix and cellularity. Focal and segmental sclerosis was demonstrated in four cases (9.8%). However, minimal glomerular change was also common (34.1%). The patients with minimal change seemed to have a higher complete remission rate than patients with more evident glomerular alterations, although the difference was not statistically significant. This clinical and immunopathological study suggests that mesangial IgM nephropathy is an important disease in Taiwan, with a variable response to treatment and frequent relapses.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Clinical and immunopathologic study of mesangial IgM nephropathy: report of 41 cases. 637 23

A case of mesangial IgM nephropathy associated with chronic renal failure is described. The patient did not reveal nephrotic syndrome during the clinical course. Renal biopsy specimens revealed typical features of mesangial IgM nephropathy when studied by light microscopy, electron microscopy and immunofluorescence staining. Mesangial IgM nephropathy is considered to be a heterogenous disorder, because this disorder is clinically characterized by nephrotic syndrome, mild proteinuria and/or hematuria or renal failure.
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PMID:A case of mesangial IgM nephropathy with decreased renal function. 640 Jan 49

The renal biopsies of three cases of nephrotic syndrome were examined by light, electron and immunofluorescent microscopy. The histological appearances were characterized by mild mesangial proliferation and extensive diffuse and global deposition of IgM in the mesangial areas, with some in the capillary walls. C3 and C1q were also identified in two of the cases. Electron microscopy revealed scanty electron dense deposits in the mesangial areas and subendothelial spaces. All three patients had a nephrotic proteinuria but no hematuria. None had hypertension or renal dysfunction throughout their clinical courses. They received steroid therapy and achieved complete remission within one month after the initial treatment. Two of them, however, have had frequent relapses whenever the dosage of steroid was gradually reduced. One case continues to be free of proteinuria. These cases indicate that a distinct disease may exist, because of the uniform immunofluorescent findings. We wish to designate it as IgM nephropathy.
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PMID:Nephrotic syndrome with diffuse mesangial IgM deposits. 704 43

Primary chronic glomerulonephritis may emerge clinically as acute nephritis, the nephrotic syndrome as well as asymptomatic hematuria and proteinuria. Therapeutic consequences still depend on the morphological diagnosis. In cases of minor proteinuria [< 3,5 g/24 h] immunosuppressive therapy is not superior to symptomatic therapy. In patients with nephrotic syndrome immunosuppressive therapy depends on the morphological diagnosis. Glucocorticoids are the therapy of choice in minimal changes glomerulopathy and should be tried in focal segmental sclerosing glomerulonephritis. Steroids may be tried in pure mesangial as well as in IgA and IgM nephropathy. Especially Ponticelli claims significant therapeutic success in patients with membranous glomerulonephritis treated with Prednisolone and Chlorambucil. Failures and relapses may be treated with Cyclophosphamide, Chlorambucil or Cyclosporin A. Anticoagulants may be advantageous in the therapy of membranoproliferative glomerulonephritis.
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PMID:[Therapy of primary chronic glomerulonephritis]. 778 93

The lipid and lipoprotein profiles including apolipoprotein A1 and B100 are measured in 50 idiopathic nephrotic patients (males 26, females 24) with mean age of 32 + 13.6 yrs, serum creatinine 1.32 +/- 0.43 mg/dl compared with 50 age matched normal controls. The renal histology consist of IgM nephropathy 70 per cent, membranous 12 per cent, and IgA 2 per cent. The serum cholesterol, triglycerides, LDL- cholesterol, VLDL-cholesterol, apolipoprotein B (521.6 +/- 201.6, 291.4 +/- 156.2, 438.8 +/- 207.4, 58.3 +/- 31.2, 265.1 +/- 119.8) are statistically significantly higher than controls (p < 0.001). The HDL-cholesterol (30.2 +/- 16.1) is also significantly lower than controls (p < 0.001) but apolipoprotein A is not different from normal subjects. The most common hyperlipoprotein type is type IIb (66%), less common are type IIa (22%), IV (6%) and III (4%) respectively. There is no correlation between serum lipids, lipoproteins and urinary protein, serum albumin, and histological diagnosis. The ratio of cholesterol: HDL, LDL: HDL and Apo A1: B are all significantly higher than normal control (p < 0.001) and correlate with urinary protein levels. This study shows that the nephrotic patients who have persistent heavy proteinuria have dyslipidemia which is highly atherogenic and probably increases the incidence of coronary heart disease.
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PMID:Lipoprotein abnormalities in adult nephrotic syndrome. 796 58

The distinctiveness of IgM nephropathy (IgMN) as a clinicopathologic entity is controversial. Twenty-seven children (16 males, 11 females) with IgMN as defined immunohistochemically by diffuse mesangial staining of glomeruli for IgM were compared to a group of 63 children (40 males, 23 females) with minimal change disease (MCD). While mesangial expansion was significantly greater in IgMN than in MCD (p = 0.0014), there were no significant differences between the two groups with respect to the other biopsy factors. IgMN showed a significantly higher incidence of hypertension at presentation. More than 90% of patients in both groups presented with the nephrotic syndrome which in most initially responded to prednisone. Frequently relapsing/steroid-dependent nephrotic syndrome was the most common indication for biopsy in both groups. Approximately 60% of patients from both groups received cytotoxic therapy. Eight percent of IgMN and 7% of MCD patients failed to respond to therapy. Relapse rates and mean dose of prednisone at relapse were very similar in both groups prior to biopsy. Relapse rates diminished significantly after treatment in the postbiopsy interval, but mean dose of prednisone at relapse did not change appreciably over time. None of the patients developed renal failure or hypertension in the follow-up period. At last visit 23% of IgMN and 27% of MCD had proteinuria. The results indicate that IgMN and MCD are indistinguishable clinically in children who are biopsied for the nephrotic syndrome.
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PMID:Childhood IgM nephropathy: comparison with minimal change disease. 890 59

Uncontrolled or refractory nephrotic syndrome (NS), seen in a variety of glomerular disorders, leads to end-stage renal disease (ESRD). This study describes the use and efficacy of cyclosporine (CSA) for the treatment of refractory NS in 83 children seen over a 10-year period. The histological diagnosis leading to the NS was focal segmental glomerulosclerosis (FSGS) in 51% (n = 42), IgM nephropathy in 20% (n = 17), membranoproliferative glomerulonephritis in 10% (n = 8), lupus nephritis in 6% (n = 5), human immunodeficiency virus (HIV) nephropathy in 5% (n = 4), minimal change disease in 7% (n = 6), and membranous nephropathy in 1% (n = 1) of patients. During CSA therapy the mean proteinuria of the study population decreased from 5.14 g/24 h (4.80 g/m2 per 24 h) to 1.23 g/24 h (0.92 g/m2 per 24 h) (P < 0.001), the mean serum albumin increased from 2.13 g/dl to 3.53 g/dl (P < 0.001), the mean serum cholesterol decreased from 364 mg/dl to 223 mg/dl (P < 0.001), and the mean serum creatinine increased from 0.77 mg/dl to 1.2 mg/dl (P < 0.01). When analyzed by histological diagnosis, similar significant trends of reduction in proteinuria were seen in all but the lupus group. There was a rise in serum creatinine following the use of CSA in patients with FSGS, lupus nephritis, and HIV nephropathy; however the elevated serum creatinine was only significant in patients with FSGS. At the end of the study period, 20 patients had reached ESRD, of which 11 had FSGS, 5 had lupus nephritis, and 4 were patients with HIV nephropathy. Fifty-four patients were in remission at the end of the study period (48 with proteinuria < 100 mg/24 h and 6 with proteinuria < 500 mg/24 h). In conclusion, among children with refractory NS, CSA induced a remission in a large proportion. However toxicity, as noted by the rise in serum creatinine, was observed in several patients. Since this toxicity may be drug induced or a natural progression of the disease, careful monitoring and close follow-up are essential.
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PMID:One-center experience with cyclosporine in refractory nephrotic syndrome in children. 1010 Feb 85

We describe a pair of 17-year-old identical twin brothers with asymptomatic proteinuria, one of whom showed focal segmental glomerulosclerosis (FSGS) while the other showed immunoglobulin M (IgM) nephropathy. For each twin, audiological examination was normal. There was no family history of renal failure, deafness, or hematuria. HLA typing revealed an identical phenotype consisting of A25, A33, B44, B54, Cw1, Cw7, DR7 and DRB1. There is still controversy about whether minimal change disease, IgM nephropathy, and FSGS are discrete entities or different aspects of the same disease. The coexistence of IgM nephropathy and FSGS in identical twins suggests that the same genetic factors may be involved in the development of both diseases. However, although the brothers are identical twins, they had different eating habits and body weight. The twin who preferred to eat a protein-rich diet and who was heavier developed early proteinuria and manifested FSGS on renal biopsy. The discordant evolution of asymptomatic proteinuria in identical twins may provide a clue for the existence of environmental factors on the progression from IgM nephropathy to FSGS. Therefore, this report provides indirect support for the hypothesis that IgM nephropathy and FSGS represent different aspects in the spectrum of a single disease.
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PMID:Discordant evolution of asymptomatic proteinuria in identical twins. 1036 10

Renal biopsy specimens from patients with systemic lupus erythematosus (SLE) rarely show changes that are pathogenetically and morphologically unrelated to SLE. The morphology and behavior of these nonlupus nephritides are not well known. Two hundred fifty-two renal biopsies performed on 224 patients with SLE collected from 3,036 native kidney biopsies performed between 1975 and 1998 were reviewed, and those that showed nonlupus nephritides (index biopsies) were selected for studies. Thirteen biopsy specimens with nonlupus nephritides were identified in 13 patients, who belonged to 3 clinically distinct groups. Group I included 6 patients in whom SLE was diagnosed at the time of index biopsies. The index biopsies in these patients showed focal segmental glomerusclerosis (FSGS; 3 cases), Immunoglobulin (Ig) M nephropathy (1 case), and thin basement membrane disease (1 case). The diagnostic features for FSGS included segmental sclerosis involving at least 1 glomerulus, absence of lupus nephritis or other conditions that may cause nonspecific segmental sclerosis of glomeruli such as ischemia or nephrosclerosis, and nephrotic-range proteinuria. There was uniform, global, diffuse and marked thinning of the glomerular basement membrane in the case of thin basement membrane disease. Group II included 3 patients in whom SLE was diagnosed 2 to 9 years before the time of index biopsies and SLE was active at the time of biopsy. The index biopsies in these patients showed FSGS (2 cases) and hypertensive nephrosclerosis (1 case). Group III included 4 patients in whom SLE was diagnosed 5 to 36 years before the time of index biopsies and SLE was inactive at the time of biopsy. The index biopsies in these patients showed 1 case each of amyloidosis, FSGS, hypertensive nephrosclerosis, and allergic acute tubulointerstitial nephritis. Previous renal biopsies, performed in 5 patients, showed IgM nephropathy (1 case), diffuse proliferative lupus GN (1 case), focal proliferative lupus GN (1 case), and mesangial proliferative lupus GN (2 cases). Follow-up biopsies, performed in 3 patients, confirmed the diagnosis of FSGS (2 cases) and hypertensive nephrosclerosis (1 case) noted in the index biopsies. Nonlupus nephritides may occasionally be encountered in SLE patients, regardless of clinical or serologic disease activity. These renal lesions display a broad morphologic spectrum in which FSGS seems most frequent. Renal biopsy plays a crucial role in identifying these lesions, which may have prognostic and therapeutic implications distinct from those of lupus nephritis.
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PMID:Nonlupus nephritides in patients with systemic lupus erythematosus: a comprehensive clinicopathologic study and review of the literature. 1167 48

A case of glomerulonephritis in a 35-year-old man with Crohn's disease is described here. The patient presented with severe diarrhea, nephrotic range proteinuria, hematuria, microangiopathic hemolytic anemia, thrombocytopenia, hypocomplementemia, acute renal failure requiring hemodialysis, cryoglobulinemia, and extensive thrombotic gangrene of the distal upper and lower limbs. The patient did not respond to plasmapheresis and steroid therapy and died of upper gastrointestinal bleeding. Renal tissue obtained at autopsy showed IgA-mediated antiglomerular basement membrane crescentic glomerulonephritis. Linear staining of the glomerular basement membrane by non-IgG antibodies is quite unusual with only 11 cases previously reported in the worldwide literature, 8 caused by IgA. Glomerulonephritis is a rarely reported extraintestinal manifestation of inflammatory bowel disease, and there are only 24 previously described cases that are reviewed and summarized in this report. Glomerulonephritis occurred in the setting of active bowel inflammation in all cases, circulating immune complexes were found in nearly half the cases, and serum complements usually were normal. Renal insufficiency and nephrotic range proteinuria were typically present at the time of diagnosis of glomerulonephritis and most often improved in parallel with treatment of the gastrointestinal disorder. The histologic findings were varied and included membranoproliferative glomerulonephritis, mesangioproliferative glomerulonephritis, membranous nephropathy, IgA nephropathy, and IgM nephropathy. Thus, the authors present the first case of glomerulonephritis caused by antiglomerular basement membrane disease in association with inflammatory bowel disease.
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PMID:IgA antiglomerular basement membrane nephritis associated with Crohn's disease: a case report and review of glomerulonephritis in inflammatory bowel disease. 1272 45


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