Membranoproliferative glomerulonephritis and persistent hypocomplementaemia.
The clinical, laboratory, and histological findings of 50 patients with membranoproliferative glomerulonephritis are described. Three-quarters of the patients, who were mostly older children and young adults, presented clinically with a mixture of "nephritic" and "nephrotic" symptoms; the remaining quarter had no symptoms and were diagnosed after the discovery of proteinuria and microscopic haematuria.Though this clinical picture may occur in other forms of glomerulonephritis, the patients described here were unified as a group by their glomerular morphological appearance-namely, a combination of mesangial proliferation and capillary wall thickening, mainly due to subendothelial accumulations of mesangial matrix.In 68% serum C3 (beta10-globulin) levels were reduced initially, while a further 16% subsequently showed a fall to abnormally low levels. All patients had substantial proteinuria, usually of moderately impaired selectivity, and all but one had haematuria in addition. Children frequently presented with an illness resembling acute nephritis, whereas adults usually had a nephrotic syndrome from the start.In 31 patients, followed for periods of one to eight and a half years, serial measurements of glomerular filtration rate were made. Sixteen have experienced no deterioration of renal function, though their proteinuria continues unchanged. Fifteen have shown progressive deterioration; six of them are still well, six are on regular dialysis treatment, and three have died. Treatment with corticosteroids, azathioprine, or cyclophosphamide, alone or in combination, did not seem to influence the course of the disease, and another two patients died from complications of steroid therapy. The disease usually runs a chronic course and appears to be progressive.
D000293:Adolescent; D000328:Adult; D001379:Azathioprine / Q000627:therapeutic use; D001609:Beta-Globulins / Q000032:analysis; D001796:Blood Protein Disorders / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D003165:Complement System Proteins / Q000032:analysis*; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005919:Glomerular Filtration Rate; D005921:Glomerulonephritis / Q000145:classification* / Q000150:complications / Q000473:pathology / Q000628:therapy; D006417:Hematuria / Q000150:complications; D006801:Humans; D007668:Kidney / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009393:Nephritis / Q000150:complications; D009401:Nephrosis / Q000150:complications; D009404:Nephrotic Syndrome / Q000150:complications; D011239:Prednisolone / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D011507:Proteinuria / Q000150:complications; D006435:Renal Dialysis
D001609:Beta-Globulins; D003520:Cyclophosphamide; D003165:Complement System Proteins; D011239:Prednisolone; D001379:Azathioprine; D011241:Prednisone