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 51-year-old woman had been suffering from blood-stained purulent sinusitis and antibiotic-resistant bouts of fever for 4 months. She had microhematuria and serological evidence of inflammation (erythrocyte sedimentation rate [ESR] 92/135 mm, C-reactive protein 5.0 mg/dl). When she was admitted to hospital suspected of having postinfectious glomerulonephritis she complained of spontaneous colic-like pains in the left flank. Within one day the haemoglobin concentration fell from 10 to 6.5 g/dl. Ultrasound and computed tomography demonstrated a large space-occupying lesion around the left kidney. At operation this was found to be a rupture of the kidney with perirenal bleeding which was treated without removing the kidney. No biopsy was taken, but serological tests showed antineutrophil cytoplasmatic antibodies (cANCA), indicating Wegener's granulomatosis as the cause of the compensated renal insufficiency and spontaneous renal rupture. Under immunosuppressive treatment the inflammatory signs (ESR 18/44 mm), fever, chronic maxillary sinusitis, raised serum creatinine concentration and the ANCA titre all regressed, while proteinuria of about 4 g/24 h persisted. There was no recurrence during a follow-up period of 15 months. Serological signs of marked inflammatory activity, urinary sediments of nephritis and spontaneous retroperitoneal bleeding should suggest that, in addition to lupus erythematodes and panarteritis nodosa, Wegener's granulomatosis be included in the differential diagnosis.
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PMID:[Spontaneous kidney rupture as an early complication of Wegener's granulomatosis]. 154 1

A 38-year old patient had been suffering, since the age of 17, from membranoproliferative glomerulonephritis associated with chronic atopic eczema and recurrent sinusitis. Bouts of eczema with severe itching occurred simultaneously with sinusitis and proteinuria. Permanently extreme serum IgE levels (greater than 10,000 IU/ml), defective neutrophil chemotaxis and monocyte phagocytic function (Buckley's syndrome) were present. Because cyclosporin reduces excessive IgE levels in Brown Norway rats with mercuric chloride nephritis, we gave the patient this drug in daily doses of 3-4 mg/kg. A dramatic improvement resulted within a few days: itching disappeared, the eczema progressively cleared, proteinuria decreased to less than 0.5 g/day and serum IgE levels to 4000 KIU/l. Reduction of dosage was followed by recurrence of all clinical and biological signs. In spite of the improvement obtained, serum creatinine levels, which were initially high (200-250 mumol/l) rose up to 300 mumol/l after one year of treatment.
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PMID:[Membranoproliferative glomerulonephritis associated with Buckley's syndrome treated with cyclosporin]. 295 74

Eleven patients with Wegener's granulomatosis were seen at this Institute over a period of 20 years. There were six men and five women. The average age of presentation was 38.3 years, and the mean duration of symptoms was 10.5 months. Constitutional symptoms (82%), cough (82%), ocular symptoms (64%), arthralgias (55%), rhinorrhoea (55%), haemoptysis (45%), nasal granuloma (45%), otorrhoea (36%), sinusitis (36%), skin lesions (27%), and renal failure (27%) were the clinical manifestations encountered. All patients had an elevated ESR, and 55% had leucocytosis. Proteinuria and haematuria were observed in 64% and 55% respectively. Chest radiographs were abnormal in 82%. In four patients the disease had a fulminant course and the patients died before adequate treatment was given. Two patients received corticosteroids alone and have since been lost to follow up. Five (45%) received adequate cytotoxic therapy and have done well for 8-46 months (mean, 24.8 months) after diagnosis. Wegener's granulomatosis in India is apparently similar to that seen elsewhere, but the high incidence of tuberculosis interferes with early diagnosis and treatment.
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PMID:Wegener's granulomatosis in north India. An analysis of eleven patients. 343 65

A 35-year old male patient who, since the age of 17, had membranoproliferative glomerulonephritis presented with recurrent atopic eczema and sinusitis. Outbursts of eczema, sinusitis and proteinuria occurred simultaneously. Signs of immune deficiency, with permanently extreme IgE levels (greater then 10.000 I.U/ml), defective neutrophil chemotaxis and monocyte phagocytotic function (Buckley's syndrome) were present. The role of these abnormalities in the pathogenesis of glomerulonephritis (chronic antigen production associated with focal infection and resulting in the formation of immune complexes, defective elimination of these circulating immune complexes) is discussed.
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PMID:[Hyperimmunoglobulinemia E syndrome with recurrent infections (Buckley's syndrome) associated with membranoproliferative glomerulonephritis. A case in an adult]. 622 Dec 61

The authors present a case report of a 62-year old woman, with hypertension for many years. She suffered from weakness, anorexia and weight loss in the last 6 months. On admission, anemia, elevated ESR, haematuria, proteinuria and renal failure were present. Renal biopsy was compatible with chronic glomerulonephritis. The clinical picture and positivity for P-ANCA suggested systemic vasculitis. Later evidence of maxillary sinusitis and nasal mucosae ulcers as well as pneumonitis, although biopsy did not reveal granulomas, suggested the diagnosis of Wegener Vasculitis. Medicated with Cyclophosphamide and Prednisolone, for a year, with improvement. The authors make a brief discussion of the clinical criteria for classification of ANCA-associated systemic vasculitis.
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PMID:[Vasculitis associated with ANCA]. 794 37

Congenital nephrotic syndrome of the Finnish type (CNF) is an autosomal recessively inherited disease manifesting as massive proteinuria, edema and ascites in the neonatal period. The disease is believed to be limited to the kidneys and recurrences after renal transplantation have not been reported. At our center 29 transplantations have been performed on 28 CNF patients. One to 33 months after transplantation, seven grafts (24%) of six patients have developed a steroid-resistant nephrotic syndrome. The clinical data and renal histology of these patients were analyzed in order to elucidate the cause of the proteinuria. At the onset of six of the seven episodes of nephrosis, the patient had evidence of a preceding CMV- or EBV-infection and the remaining patient had sinusitis. Upon light and electron microscopy examination, endothelial swelling of the glomerular capillaries resembling transplant glomerulopathy (TG) was seen, but unlike TG, the glomerular basement membranes were normal. The response of proteinuria to steroid or cyclophosphamide therapy was poor, with total remission in only two patients and partial remission in one patient, all treated with methylprednisolone and cyclophosphamide immediately after the diagnosis. Four grafts have been lost. Our data show that CNF patients have an increased tendency for post-transplantation nephrosis.
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PMID:Post-transplantation nephrosis in congenital nephrotic syndrome of the Finnish type. 825 63

Complement factor I deficiency is known to be associated with recurrent pyogenic infections. The patient described here had recurrent attacks of otitis, sinusitis, and bronchopneumonia since childhood. At the age of 24 years, he had an acute episode of systemic vasculitis with purpura, but no nephritis. A factor I deficiency was diagnosed when he was 36 years old. Because of the uncontrolled activation of the alternative pathway of complement, several other components were depleted, in particular C3, which explained the predisposition for pyogenic infections. A progressive loss of renal function accompanied by proteinuria and hematuria started after the age of 40 years. Renal biopsy showed a focal segmental glomerulonephritis (GN) with glomerular deposits of immunoglobulins and complement C3 and C4 fragments. The glomerular podocytes showed an almost complete loss of complement receptor 1 (CR1; CD35). The expression of CR1 was very low on erythrocytes, as well. Thus, CR1, the most efficient cell-bound cofactor for the inactivation of C4b/C3b by factor I, appears to be consumed when factor I is missing. Although this is the first report of factor I deficiency associated with GN, it is unlikely that the development of the nephritis was fortuitous because GN has been found in many other diseases characterized by uncontrolled activation of the alternative pathway.
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PMID:Glomerulonephritis in a patient with complement factor I deficiency. 1035 6

A 32 year-old man presented with sinusitis, proteinuria, mononeuritis multiplex, very increased acute phase proteins. Anti-PR3 ANCA were detected and Wegener's granulomatosis (WG) was diagnosed. As abdominal tomodensitometry detected a tumoral process of the left kidney, a paraneoplastic vasculitis associated with a renal cancer was suspected. Biopsy of the mass showed fibrosis, inflammatory infiltrates and necrotizing granulomas. No malignant cells were detected. The outcome was favourable after administration of methylprednisolone and cyclophosphamide. Characteristics of the nine previously reported renal inflammatory pseudotumors associated with WG are discussed.
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PMID:Wegener's granulomatosis masquerading as a renal cancer: a case report and review of the literature. 1718 31

Here we report a case of a 71-year-old Japanese woman with membranous nephropathy complicated with chronic sinusitis. The patient visited our hospital for treatment of edema and proteinuria, and was diagnosed as nephrotic syndrome with serum total protein 7.5 g/dL, albumin 2.2 g/dL and urine protein 3.7 g/day. Renal biopsy revealed membranous glomerulonephritis with tubulointerstitial nephritis. She had suffered from chronic sinusitis for several years. Laboratory tests showed that immunoglobulin G (IgG) and soluble interleukin-2 receptor (sIL-2R) were markedly increased (3,233 mg/dL and 5,110 U/mL, respectively) and Gallium scintigraphy showed abnormal accumulation to the paranasal sinus. After operation for chronic sinusitis, the levels of IgG and sIL-2R were decreased, and nephrotic syndrome was improved without any other specific treatments. It is known that various diseases, including membranous nephropathy and sinusitis, are caused by T-cell dysfunction. Thus, we suspect the pathogenetic link between membranous nephropathy and chronic sinusitis in this case, most probably through T-cell dysfunction.
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PMID:A case of membranous nephropathy associated with chronic sinusitis. 1938 48

Renal involvement in Churg-Strauss syndrome (CSS) is not uncommon, but nephrotic syndrome is rarely reported in patients with CSS. A 25-year-old woman with a long history of bronchial asthma presented to our hospital with a chief complaint of generalized edema. Laboratory studies revealed normocytic normochromic anemia, marked eosinophilia, positive anti-neutrophil cytoplasmic antibody, hypoalbuminemia, and hypercholesterolemia. Urinalysis showed heavy proteinuria (4+) without significant casts. The 24-hour urinary protein excretion was 6.5 g with a selective index of 0.35. Echocardiography and X-ray of the paranasal sinuses suggested restrictive cardiomyopathy and maxillary sinusitis, respectively. Diagnoses of CSS and nephrotic syndrome were made on the basis of clinical and laboratory findings. Renal biopsy was performed, and pathologic findings revealed focal segmental glomerulosclerosis with mesangiolysis. The patient's clinical symptoms and proteinuria improved markedly after combined treatment with corticosteroids and cyclophosphamide. Herein, we report a case of nephrotic syndrome in a patient with CSS.
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PMID:A case of nephrotic syndrome in a patient with Churg-Strauss syndrome. 1969 37


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