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)

In recent years more than 150 cases of glomerulonephritis characterized by deposits of irregularly arranged fibrils have been documented. In the majority of these cases immunoglobulins and complement are the prime constituents of these deposits. We recently made a diagnosis of fibrillary glomerulonephritis without immunoglobulin deposition in two members of a family, a father and a son. In the father, proteinuria was first discovered 18 years ago. In 1985 he was referred to our outpatient clinic because of hypertension and increasing proteinuria. From that time onward he was regularly seen for blood pressure control. Nephrotic-range proteinuria persisted, without hardly any evidence of deterioration of renal function. Renal biopsies were performed in 1985 and 1993. His son underwent a renal biopsy in 1993 because of moderate proteinuria. The biopsies of both patients disclosed a distinct form of fibrillary glomerulonephritis that was characterized by massive deposits of a homogeneous, eosinophilic material in the mesangial and subendothelial areas. Staining for amyloid was negative. Immunofluorescence revealed that the biopsy specimens only stained faintly for immunoglobulins, complement factors C1q and C3, the extracellular matrix proteins, collagen IV, and laminin. However, they strongly stained for fibronectin. Using monoclonal antibodies specific for cell-derived fibronectin (IST-9) and plasma- and cell-derived fibronectin (IST-4), in the biopsy of the son we demonstrated that the fibronectin deposited in the glomeruli was mainly derived from the plasma, and to a lesser extent from resident glomerular cells. In addition, a moderate staining for amyloid P and vitronectin also was present. No or minor enhanced staining for collagen I, III, or V, heparan sulfate proteoglycan or its glycosaminoglycan side chains, tenascin, or thrombospondin could be observed. By electron microscopy the deposits in the mesangium and the subendothelial spaces appeared focally to be composed of irregularly arranged fibrils or microtubules 10 to 12 nm in diameter. Fibrillary glomerulonephritis with massive deposits of fibronectin represents a rare form of familial glomerulonephritis. In our patients the glomerulonephritis has an indolent course with hardly any deterioration of renal function.
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PMID:Familial glomerulonephritis characterized by massive deposits of fibronectin. 774 33

We report a 63 years old female patient presenting with progressive edema, alopecia and pallor. Laboratory showed a proteinuria of 1.9 g/24 h, microhematuria, a serum creatinine of 3.1 mg/dl, erythrocyte sedimentation rate of 133 mm/h and antinuclear antibodies of 1/40 with a homogeneous pattern. No extrarenal disease was demonstrated and a kidney biopsy, performed 18 months later, showed a fibrillary glomerulonephritis, nodular sclerosis variety, with 20 nm phi fibrillae, immune and kappa and lambda light chain deposits and negative Congo red stain. The patient died 30 months later due to a respiratory infection. Fibrillary glomerulonephritis is an infrequent form of mesangiocapillary glomerulonephritis not associated to plasma cell dyscrasia. It leads to terminal renal failure and it recurs in transplanted kidneys.
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PMID:[Fibrillary glomerulonephritis of the nodular glomerulosclerosis variety]. 808 85

Fibrillary glomerulonephritis (FGN) is a rare cause of nephrotic syndrome. In three patients (65-year-old male; 57- and 38-year-old females) FGN was diagnosed by percutaneous renal biopsy. Clinically, the disease manifested itself with long term proteinuria and nephrotic syndrome without any associated systemic disorder. Histologically, glomeruli showed deposition of PAS+, fuchsinophilic and Orange G+ material in the mesangium and basal membranes. Strong granular immunofluorescence IgG, C3 and weak kappa and lambda light chains mesangial and peripheral deposition were observed in two patients. Electronmicroscopically, deposition of fibrillary material into mesangium and basal membranes was found. Randomly distributed nonbranching fibrils were 18-28 nm thick. After 10 to 13 months of follow-up, the therapy with corticosteroids and immunosuppressives was without effect, and the disease progressed into chronic renal failure in two patients; the third patient did well.
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PMID:[Fibrillary glomerulonephritis]. 1242 85

Fibrillary glomerulonephritis (FGN) is a rarely diagnosed disease with clinical manifestations such as proteinuria, microscopic hematuria, nephrotic syndrome or decreased kidney function. Around one half of patients develop chronic renal failure in the course of several years. The diagnosis of fibrillary glomerulonephritis is to be established only basing on the results of renal biopsy. Pathognomonic is the electron-microscopic examination, evidencing fibrillar deposits in mesangium and in basal membranes of glomeruli. Fibrils are similar to those seen at amyloidosis, however, with larger diameter, non-linear deposition and do not stain with Congo red or thioflavin T. Immunofluorescency test usually shows the presence of IgG, namely the subclasses IgG4, C3 and kappa and lambda of light immunoglobulin chains. The presented case report describes clinical and laboratory findings at a patient suffering from nephrotic syndrome. Results of renal biopsy and detailed histological examinations concluded the diagnosis as fibrillary glomerulonephritis. The patient was treated with a combination of prednisone (1 mg/kg/24 hrs) with cyclophosphamide (2 mg/kg/24 hrs) for six months. This led to a decrease of proteinuria from the initial value of 5.38 g/24 hours to 1.88 g/24 hours, as well as to a partial remission of nephritic syndrome. Glomerular filtration, evaluated using endogenous creatinine clearance, remained within limits of normal values throughout the follow-up, with the value of 2.6 ml/s after the treatment.
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PMID:[Fibrillary glomerulonephritis--a rare cause of nephrotic syndrome]. 1552 Nov 99

We report a 62-year-old man with documented type 2 diabetes mellitus and hypertension, who presented with a rapid deterioration in renal function. The sudden decrease in renal function in this well-controlled diabetic patient prompted us to consider a nondiabetic and nonhypertensive cause. The urinary sediment showed a glomerular haematuria suggestive of glomerulonephritis. A diagnosis of fibrillary glomerulonephritis was made on renal biopsy. Fibrillary glomerulonephritis is a rarely diagnosed disease with clinical manifestations such as proteinuria, microscopic haematuria, nephrotic syndrome and impairment of renal function. A diagnosis of fibrillary glomerulonephritis can only be made by electronmicroscopy of the renal tissue. In this case report the spectrum of this disease is reviewed.
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PMID:Fibrillary glomerulonephritis in a patient with type 2 diabetes mellitus. 1660 59

Fibrillary glomerulonephritis belongs to a group of disorders characterized by pathogenic deposition of fibrils in glomeruli. This glomerulopathy tends to progress to end-stage kidney disease, and there currently are no treatments of proven benefit, including corticosteroids and cytotoxic agents. Because the glomerular deposits contain an immunoglobulin component, it was postulated that anti-B-cell therapy with rituximab, an anti-CD20 monoclonal antibody, may be effective in the treatment of patients with fibrillary glomerulonephritis. We describe 3 patients with fibrillary glomerulonephritis who were treated with rituximab for nephrotic-range proteinuria. Each patient also received standard antiproteinuria therapy, including blockade of the renin-angiotensin system and strict blood pressure control. All patients showed a decrease in proteinuria to less than 1.5 g/d of protein by 27 months, and kidney function was preserved throughout the duration of therapy and follow-up. No adverse effects were seen with rituximab. These outcomes suggest that treatment with rituximab may be a promising approach to the management of fibrillary glomerulonephritis, an entity previously considered refractory to therapy.
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PMID:Rituximab treatment of fibrillary glomerulonephritis. 1882 85

Fibrillary glomerulonephritis (FibGN) is a rare cause of progressive renal dysfunction, often leading to dialysis within a few years. A 60-year-old woman presented with a 2 month history of right-sided retro-orbital pain and recent diplopia. Laboratory testing revealed an altered renal function with increased serum creatinine and mild proteinuria. MRI of the brain revealed the presence of a soft tissue mass on the right cavernous sinus compatible with the diagnosis of Tolosa-Hunt syndrome (THS). Renal biopsy showed a pattern compatible with fibrillary glomerulonephritis. For this reason steroid therapy was initiated at a dose of 1 mg/kg/day and adjusted according to the clinical course. Neurological symptoms regressed shortly after the beginning of therapy and renal function and proteinuria remained stable for the 3 years following the withdrawal of steroid therapy. Percutaneous renal biopsy was again performed and confirmed the previous diagnosis of FibGN in association with other glomerular-lesion-like mesangial widening, thickening of capillary walls and severe arterio-arteriolosclerosis. This case report describes what is believed to first report of the association of FibGN and THS, which both responded to steroid therapy.
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PMID:A simultaneous occurrence of Tolosa-Hunt syndrome and fibrillary glomerulonephritis: a case report. 1918 38

In routine diagnosis for renal biopsy, glomerular diseases are encountered that, viewed by electron microscopy, reveal deposits with a fibrillary structure in the extracellular matrix of the glomeruli. Amyloidosis is the most common glomerulopathy that shows deposits with an ultrastructural fibrillary structure. However, other glomerulopathies have deposits with an ultrastructural fibrillary structure and which are negative for Congo-red stain, but positive for immunoglobulins. Fibrillary glomerulonephritis is a well-characterized disease, and clearly identified in approximately 0.5% to 1% of biopsies of native kidneys. These cases usually manifest themselves as severe renal insufficiencies with nephrotic-range proteinuria. No treatment regimes have been clearly established and the prognosis is poor. Herein, the clinical and histological characteristics are described for the first case of idiopathic fibrillary glomerulonephritis reported in Colombia.
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PMID:[Glomerular deposition disease, regarding a case of fibrillary glomerulonephritis]. 2044 Apr 52

Fibrillary glomerulonephritis (FGN) is characterized by deposition of non-amyloidotic fibrillary material in glomeruli, and most patients with the disease show heavy proteinuria and hematuria, and progress into end-stage renal failure. We report a 62-year-old woman with FGN who showed mild proteinuria without hematuria and developed rapidly progressive renal failure requiring hemodialysis. Renal biopsy showed severe tubulointerstitial injury associated with non-amyloidotic fibrillary deposits in the tubular basement membrane, interstitium and vessel walls, in addition to glomeruli. The patient died from liver abscess 1 year after the introduction of hemodialysis. Postmortem examination showed the presence of non-amyloidotic fibrillary deposits arranged in tightly packed electron-dense and bundle-shaped structures in many organs. These findings suggest systemic non-amyloidotic fibril deposition in FGN.
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PMID:Systemic non-amyloidotic fibril deposition disease: a probable variant form of fibrillary glomerulonephritis. 2117 54

Fibrillary glomerulonephritis and immunotactoid glomerulopathy belong to the rare renal disorders characterized by formation of the organized glomerular deposits. Pathogenesis of these disorders is still not fully clarified but they could appear as a primary condition or be regarded as a part of the various systemic mainly lymphoproliferative disorders. Clinical presentation includes proteinuria, hematuria, arterial hypertension and progressive renal insufficiency during several years. In this work we presented a male patient with fibrillary glomerulonephritis and a female patient with immunotactoid glomerulopathy as a part of a non-Hodgkin lymphoma. The aim of this presentation is to show the features of the fibrillary glomerulonephritis and immunotactoid glomerulopathy as well as emphasize the significance of the electron microscopy in the identification of these uncommon entities.
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PMID:[Fibrillary glomerulonephritis and immunotactoid glomerulopathy: case reports]. 2216 79


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