Membranoproliferative Hypocomplementemic Glomerulonephritis

Abstract

The diagnosis of membranoproliferative glomerulonephritis is made by its distinctive glomerular morphology. Early there may be an acute glomerulonephritis and late, a lobular glomerulonephritis. Hypocomplementemia often accompanies the disease in children. Onset is usually in childhood as a nephrotic or acute nephritic syndrome or a chance proteinuria. Origin is unknown and onset may be insidious. The disease is typically long standing with, terminally, rapid deterioration. Children may have a long interval of ‘silent’ nephritis. Activation of C3 by an alternate pathway involving a nephritic factor and the C3 proactivator produces the hypocomplementemia but has not been demonstrated to produce the nephritis. No treatment is consistently successful. The disease can recur in a renal transplant.

Long-Term Prognosis of Membranoproliferative Glomerulonephritis Type I

“…We are not able to provide an explanation for this discrepancy with the studies of other authors who have found the nephrotic syndrome to be associated with a less favorable prognosis. In previous investigations no corre lation could be seen between the frequency and the severity of hypocomplementemia on one hand and the progression/remission of MPGN on the other [1,[4][5][6]10,12,13,16]. In a study of 40 patients Swainson et al [2], however, found a lowersurvival rate in hypocomplementemicas opposed to normocomplementemic MPGN.…”

“…The prognosis is generally considered unfavorable [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16], but there is still uncer tainty about the factors causing the progression to endstage renal failure or even death of the patient. In earlier investigations [14,[17][18][19] we noticed certain correlations between the severity of tubulointerstitial changes at the time of biopsy and renal function in MPGN.…”

The 2010 Nephrology Quiz and Questionnaire

“…MPGN type I can be idiopathic or secondary to a variety of diseases (4) Traditionally, MPGN has been associated with the presence of chronic infections including hepatitis C (5,6), shunt nephritis (7), and abscesses and endocarditis (8)(9)(10)(11). Of these, hepatitis C virus (HCV) is by far the commonest with kidney involvement due to type II cryoglobulinemia in 50% to 60% of cases (6,(12)(13)(14)(15).…”

Membranoproliferative Glomerulonephritis: Correlations between Immunological and Histological Findings

“…It has been reported that hypocomplementemic MPGN do not usually have overt recurrent infections and a frank immunity deficiency syndrome [21 ].…”

“…In the serum of ten hypocomplcmcntemic patients the presence of circulating factor, which is able to break dow n the normal It has been clearly demonstrated in various studies [2,5,11] that menibranoproliferative glomerulonephritis (MPGN) or mesangiocapillary glom erulonephritis is one identifiable form of progressive glomerulonephritis with distinct histological and clinical features. This chronic nephritis occurs with greater frequency in children than in adults and is often accompanied by hypocomplementemia [2,13,21], The complement profile in the 'classical MPGN' and in 'lobular glomerulonephritis', which probably represents the same clinicopathological entity [5], often gives evidence of an alternate path way mechanism, activated by a circulating factor designated Cs nephritic factor (C;. NeF) [20,17], Many previous studies concern pediatric patients and only a few publi cations describe investigations in adults [11,8], The present study was under-…”