Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
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Hereditary nephritis is a disease presenting with hematuria, proteinuria, and systemic findings including ocular lesions and deafness. Renal failure frequently occurs in males who have the disease at a young age. We report the case of a female with hereditary nephritis who presented with the classic clinical and pathologic features of crescentic glomerulonephritis post-pregnancy. A sibling also had a comparable course. It is proposed that crescentic glomerulonephritis may be a heretofore unreported presentation of hereditary nephritis with terminal renal failure.
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PMID:Alport's syndrome representing as crescentic glomerulonephritis: a report of two siblings. 72 17

In dealing with asymptomatic hematuria or proteinuria in the pediatric population, the physician should begin with an organized, logical sequence of diagnostic steps, including careful review of the history, physical findings, and laboratory data. If serious glomerular disease is suspected (eg, presentation atypical of acute poststreptococcal glomerulonephritis, nephrotic syndrome beyond the usual age for idiopathic nephrotic syndrome of childhood, renal insufficiency, severe hypertension), biopsy is probably inevitable, and early referral to a nephrologist is urged.
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PMID:Asymptomatic hematuria and proteinuria in children: differentiating the causes. 74 May 71

In 1968 an outbreak of 348 cases of acute poststreptococcal glomerulonephritis (AGN) was observed in Maracaibo, Venezuela. During the year, the epidemic had three peaks of incidence. Districts with better sanitation showed a lower incidence of disease than those with less adequate facilities. Endemic cases occur every year. Lowering of serum complement activity was observed in 96% and hypertension in 90% of the patients. The mortality rate in the acute phase during the epidemic was 1.31%. A history of antecedent infection was found in 39% of the cases. 70% of these infections were in the upper respiratory tract. During 1973-74, 120 of the patients (19 adults and 101 children) were reexamined. All but one had been completely asymptomatic. Sixteen patients (13.3%) had one or more of the following abnormalities (group A): low CCr, microscopic hematuria, proteinuria of 1 g/day or more and hypertension. One hundred and four patients (86.7%) were normal by all clinical and laboratory parameters tested (group B). The incidence of persisting disease, as judged by biochemical findings, was significantly higher (P less than .01) in adults (36.7%) than in children (8.9%). Percutaneous renal biopsies from 7 patients of group A, and 8 patients from group B, were studied by light microscopy and immune histology. All biopsies from group A patients had evidence of advanced glomerular disease by light microscopy and by immune histology. Seven biopsies from group B patients were also abnormal showing mild changes with corresponding immunologic findings. Only in one patient was the biopsy completely normal. Our studies indicate that significant numbers of patients progress towards chronicity after epidemic, poststreptococcal AGN. Such progression is more common in adults than in children.
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PMID:Epidemic glomerulonephritis in Maracaibo. Evidence for progression to chronicity. 78 25

Six patients with mesangiocapillary glomerulonephritis and intramembranous dense "deposits" developed terminal renal failure and were transplanted, three from living and three from cadaver donors. Eight renal biopsy specimens were obtained from five of the grafts, from 1 to 26 months following transplantation. All six biopsy specimens taken later than seven months following the graft showed recurrence of dense intramembranous "deposits" in the basement membranes of glomeruli, and of Bowman's capsule and tubular basement in five. Recurrence of "deposits" was associated with deposition of C3 on immunofluorescent study in all but one specimen; in addition, IgM was found in two specimens, but IgG and early complement components were absent. Only two patients, however, showed glomerular proliferation associated with profuse proteinuria. In the other subjects the recurrence of the "deposit" was not associated with clinical findings. Graft loss, which occurred in two patients, was predominantly from rejection.
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PMID:Transplantation in mesangiocapillary glomerulonephritis with intramembranous dense "deposits": recurrence of disease. 78 88

Association of membranous glomerulonephropathy with crescentic glomerulonephritis is apparently extremely rare. We report three patients who had this combination. One patient had biopsy-proven membranous glomerulonephropathy thirteen months prior to sudden and rapid decline in renal function necessitating hemodialysis. A repeat renal biopsy showed a superimposed crescentic nephritis and antiglomerular (GBM) antibodies were demonstrable in the serum. A second patient had proteinuria of unknown duration and then developed renal failure. Renal biopsy showed crescentic nephritis with a fine granular glomerular immunofluorescence for IgG typical of membranous glomerulonephropathy. Anti-GBM antibodies were present in this patient's serum. The third patient presented with acute renal failure of moderate severity. A renal biopsy revealed crescentic nephritis, granular deposits of immunoglobulins, and epimembranous electron-dense deposits typical of membranous glomerulonephropathy. Although his creatinine clearance improved spontaneously, nephrotic syndrome has persisted and a repeat renal biopsy showed a progression of the membranous glomerulonephropathy with the disappearance of the crescentic lesions. The reason for this peculiar association of membranous glomerulonephropathy and crescentic glomerulonephritis is unclear. It is possible that deposition of immune-complexes along glomerular basement membrane may render the glomerulus more susceptible to additional injury from a variety of other agents. Alternatively, depostis formed in one disease could initiate release of normal or altered basement membrane material and lead to formation of anti-GBM antibodies and subsequent development.
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PMID:Association of crescentic glomerulonephritis with membranous glomerulonephropathy: a report of three cases. 78 51

Percutaneous renal biopsies from 56 patients with systemic lupus erythematosus were studied to determine the relationship between renal function and the light microscopic, electron microscopic and immunofluorescent antibody findings. The glomerular lesions were classified into 5 major groups: diffuse membranoproliferative glomerulonephritis (34%), diffuse proliferative glomerulonephritis (26.8%), membranous nephropathy (12.5%), minimal lesion ('nil') and minimal lesion with increased mesangial matrix and/or cells (21.4%) and focal and segmental glomerulonephritis (5.3%). Minimal lesions and focal and segmental glomerulonephritis were invariably associated with normal renal function. Patients with moderate to severe renal involvement and the nephrotic syndrome had predominantly diffuse membranoproliferative and diffuse proliferative glomerulonephritis. Membranous nephropathy was associated with moderate renal involvement and the nephrotic syndrome in 50% of cases. Patients with pure mesangial electron dense deposits had normal renal function or mild renal involvement when the deposits were heavy. Moderate and heavy subepithelial, and intramembranous/subepithelial deposits were associated with moderate to severe renal involvement and the nephrotic syndrome. Renal involvement was most severe with heavy subendothelial deposits. Cytoplasmic tubuloreticular structures measuring approximately 18 to 20 nm in diameter and 80 to 100 nm in length were found in 93% of all biopsies, but bore no relationship to the renal function of the patients. Anti-Hu-IgG fluorescent deposits were found in all the renal biopsies; in 81.3% these were associated with less heavily stained deposits of immunoglobulin IgA, IgD and IgM. Early complement components Clq and C4 were utilized in the complement pathway of activation. Pure mesangial fluorescent deposits were associated with normal renal function or mild proteinuria. Diffuse granular and lumpy deposits along the capillary loops were usually associated with moderate to severe renal involvement and the nephrotic syndrome. In the present series of cases, there was a good correlation between renal function of patients with systemic lupus erythematosus and the glomerular lesions as determined by light, electron microscopic and immunofluorescent microscopic findings.
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PMID:Lupus nephritis: correlation between light, electron microscopic and immunofluorescent findings and renal function. 78 52

The third component of complement (C3) was measured in the urine of 98 patients with a variety of renal diseases. Renal biopsy was performed on 83 of the patients and examined by light, electron, and immunofluorescence microscopy. Urinary C3 was detected in cases of membranous glomerulonephritis, mesangiocapillary glomerulonephritis, rapidly progressive glomerulonephritis, and renal amuloidosis. It was not detected in minimal lesion glomerulonephritis; in cases of proliferative glomerulonephritis it was detected only in those showing histological evidence of a progressive lesion. Concentrations were low or undetectable in cases of non-immunological renal diseases. There was a good correlation between urinary C3 concentrations and the deposition of C3 in glomerular capillary walls, as seen by immunofluorescence microscopy, and there was no correlation with the degree or selectivity of proteinuria. Urinary C3 excretion appears to be an accurate indicator of continuing activity of disease. It is suggested that the presence of C3 in urine is due to complement fixation by immune complexes in glomerular capillary walls, and that urinary C3 estimations have potential applications in the study of glomerulonephritis.
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PMID:Significance of urinary C3 excretion in glomerulonephritis. 78 6

Mice were infected with 1X 107 Plasmodium berghei Yoelii parasites intraperitoneally. Circulating parasite, malaria antibody and C3 concentrations were measures: parasitaemia and hypocomplementaemia were transient, but the antibody response was persistent. Animals were sacrificed at intervals and their kidneys examined: a glomerulonephritis associates with predominantly mesangial deposits of C3, IgG1, IgM and some IgA always developed after 7 days and persisted for up to 6 mth. Malaria antigen and antibody were demonstrated within the glomeruli. Microscopic haematuria occurred with proteinuria but without marked deterioration in renal function. Strains producing high and low affinity antibody were equally susceptible to the disease. Treatment with glucocorticoid, immunosuppressive, platelet function inhibiting and/or anticoagulant drugs, or indomethacin from the 1st day of infection failed to prevent development of the disease or to lead to its early cure. Eradication of the infection within its first 3 days prevented glomerular deposition of antibody and complement, and infection with a smaller antigen load followed by later treatment also produced subsequent cure.
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PMID:Mouse malaria nephropathy. 79 19

A clinicopathological study was done on 85 Japanese patients with IgA glomerulonephritis. Of patients with glomerulonephritis whose clinical course is of slow progression, the presence of IgA glomerulonephritis was demonstrated immunohistologically. The majority of such patients presented with a mild degree of proteinuria and/or persistent microscopic hematuria. In 15% of the patients, recurrent gross hematuria was noted. These findings suggest that immunohistological study is important in dealing with adult patients with so-called idiopathic renal hematuria. Light-microscopic findings of IgA glomerulonephritis were divided into 4 groups. In 56% of the patients, diffuse stalk thickening of the mesangium was noted and in one third of them, small crescent formations were present. In 22% of the patients, focal segmental proliferation was noted. Diffuse mesangial deposition of IgA noted by immunofluorescence was considered to correspond to the characteristic nodular deposition detected by light microscopy and to the relatively large globular hemispheric deposits detected by electron microscopy.
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PMID:IgA glomerulonephritis in Japan. 79 23

The myeloma kidney is characterized by casts in the distal and collecting tubules. The glomeruli are hardly affected unless amyloidosis is present. When the glomeruli are involved, the proteinuria is nonselective and, in some cases, the whole paraprotein is excreted in the urine. Nephrocalcinosis may be present and focal myeloma cell infiltration in the interstitium is a characteristic, but inconstant, finding. The nephrotic syndrome is extremely rare; if it exists, amyloidosis should be suspected. In contrast to multiple myeloma, the glomeruli are frequently involved in macroglobulinemia of Waldenstrom. Hyaline intracapillary deposits consisting of pure IgM are a characteristic finding as is infiltration of the kidney with lymphoid cells. No characteristic lesion of the kidney has been described in the heavy-chain diseases. Mixed cryoglobulinemia associated with an IgM paraprotein can produce glomerulonephritis that is due to the deposition in the glomeruli of an immune complex consisting of IgG, IgM, and complement.
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PMID:Renal pathologic findings associated with monoclonal gammopathies. 80 64


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