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Published in: Pediatric Nephrology 2/2009

01-02-2009 | Original Article

Membranous glomerulonephritis: treatment response and outcome in children

Authors: Rudolph P. Valentini, Tej K. Mattoo, Gaurav Kapur, Abubakr Imam

Published in: Pediatric Nephrology | Issue 2/2009

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Abstract

The aim of this study was to characterize clinical features, treatment response, and outcome of idiopathic membranous glomerulonephritis (MGN) in a single-center cohort of children. A retrospective review of biopsy-proven idiopathic MGN in 12 children (mean age 11.9 years) was undertaken. Presentation was nephrotic syndrome (NS) (75%), hematuria/proteinuria (17%), and asymptomatic proteinuria (8%). Ten patients (83%) with NS and nephrotic range proteinuria (NRP) were treated with prednisone, and two patients with non-NRP were not treated with immunosuppressive medications. Steroid response in the treated patients was complete (10%), partial (40%), and absent (50%), respectively. Oral cyclophosphamide was used in seven patients of whom five were steroid resistant, one was steroid dependent, and one was partially responsive. At the mean follow up of 27 months, outcome parameters included an estimated glomerular filtration rate of 128 cc/min per 1.73 m2, albumin of 4.2 gm/dL, and a urine protein/creatinine ratio of 0.87 [median 0.16 (range 0.02–6.52)]. Remission was complete in 75% of the patients and partial in 17%. One patient (8%) with chronic kidney disease (stage 2) was unresponsive to therapy. Complete remission was significantly associated with the absence of chronic histological changes (p = 0.03). In conclusion, children with NS and/or NRP associated with MGN appear to have a good prognosis when treated with a combination of corticosteroids and cyclophosphamide.
Literature
1.
go back to reference Srivastava T, Simon SD, Alon US (1999) High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood. Pediatr Nephrol 13:13–18CrossRef Srivastava T, Simon SD, Alon US (1999) High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood. Pediatr Nephrol 13:13–18CrossRef
2.
go back to reference Churg J, Habib R, White RH (1970) Pathology of the nephrotic syndrome in children: a report for the International Study of Kidney Disease in Children. Lancet 760:1299–1302CrossRef Churg J, Habib R, White RH (1970) Pathology of the nephrotic syndrome in children: a report for the International Study of Kidney Disease in Children. Lancet 760:1299–1302CrossRef
3.
go back to reference Gulati S, Sengupta D, Sharma RK, Sharma A, Gupta RK, Singh U, Gupta A (2006) Steroid resistant nephrotic syndrome: role of histopathology. Indian Pediatr 43:55–60PubMed Gulati S, Sengupta D, Sharma RK, Sharma A, Gupta RK, Singh U, Gupta A (2006) Steroid resistant nephrotic syndrome: role of histopathology. Indian Pediatr 43:55–60PubMed
4.
go back to reference Ponticelli C, Zucchelli P, Passerini P, Cesana P, Locatelli F, Pasquali S, Sasdelli M, Redaelli B, Grassi C, Pozzi C, Bizzarri D, Banfi G (1995) A 10-year follow-up of a randomized study with methylprednisolone and chlorambucil in membranous nephropathy. Kidney Int 48:1600–1604CrossRef Ponticelli C, Zucchelli P, Passerini P, Cesana P, Locatelli F, Pasquali S, Sasdelli M, Redaelli B, Grassi C, Pozzi C, Bizzarri D, Banfi G (1995) A 10-year follow-up of a randomized study with methylprednisolone and chlorambucil in membranous nephropathy. Kidney Int 48:1600–1604CrossRef
5.
go back to reference Haas MMS, Karrison TG, Spargo BH (1997) Changing etiologies of unexplained adult nephrotic syndrome: a comparison of renal biopsy findings from 1976–1979 and 1995–1997. Am J Kidney Dis 30:621–631CrossRef Haas MMS, Karrison TG, Spargo BH (1997) Changing etiologies of unexplained adult nephrotic syndrome: a comparison of renal biopsy findings from 1976–1979 and 1995–1997. Am J Kidney Dis 30:621–631CrossRef
6.
go back to reference Schieppati A, Mosconi L, Perna A, Mecca G, Bertani T, Garattini S, Remuzzi G (1993) Prognosis of untreated patients with idiopathic membranous nephropathy. N Engl J Med 329:85–89CrossRef Schieppati A, Mosconi L, Perna A, Mecca G, Bertani T, Garattini S, Remuzzi G (1993) Prognosis of untreated patients with idiopathic membranous nephropathy. N Engl J Med 329:85–89CrossRef
7.
go back to reference Cattran DC, Appel GB, Hebert LA, Hunsicker LG, Pohl MA, Hoy WE, Maxwell DR, Kunis CL (2001) Cyclosporine in patients with steroid-resistant membranous nephropathy: a randomized trial. Kidney Int 59:1484–1490CrossRef Cattran DC, Appel GB, Hebert LA, Hunsicker LG, Pohl MA, Hoy WE, Maxwell DR, Kunis CL (2001) Cyclosporine in patients with steroid-resistant membranous nephropathy: a randomized trial. Kidney Int 59:1484–1490CrossRef
8.
go back to reference Passerini P, Ponticelli C (2003) Corticosteroids, cyclophosphamide, and chlorambucil therapy of membranous nephropathy. Semin Nephrol 23:355–361CrossRef Passerini P, Ponticelli C (2003) Corticosteroids, cyclophosphamide, and chlorambucil therapy of membranous nephropathy. Semin Nephrol 23:355–361CrossRef
9.
go back to reference Reichert LJ, Koene RA, Wetzels JF (1998) Prognostic factors in idiopathic membranous nephropathy. Am J Kidney Dis 31:1–11CrossRef Reichert LJ, Koene RA, Wetzels JF (1998) Prognostic factors in idiopathic membranous nephropathy. Am J Kidney Dis 31:1–11CrossRef
10.
go back to reference du Buf-Vereijken PW, Branten AJ, Wetzels JF (2005) Idiopathic membranous nephropathy: outline and rationale of a treatment strategy. Am J Kidney Dis 46:1012–1029CrossRef du Buf-Vereijken PW, Branten AJ, Wetzels JF (2005) Idiopathic membranous nephropathy: outline and rationale of a treatment strategy. Am J Kidney Dis 46:1012–1029CrossRef
11.
go back to reference Wu Q, Jinde K, Nishina M, Tanabe R, Endoh M, Okada Y, Sakai H, Kurokawa K (2001) Analysis of prognostic predictors in idiopathic membranous nephropathy. Am J Kidney Dis 37:380–387CrossRef Wu Q, Jinde K, Nishina M, Tanabe R, Endoh M, Okada Y, Sakai H, Kurokawa K (2001) Analysis of prognostic predictors in idiopathic membranous nephropathy. Am J Kidney Dis 37:380–387CrossRef
12.
go back to reference Perna A, Schieppati A, Zamora J, Giuliano GA, Braun N, Remuzzi G (2004) Immunosuppressive treatment for idiopathic membranous nephropathy: a systematic review. Am J Kidney Dis 44:385–401CrossRef Perna A, Schieppati A, Zamora J, Giuliano GA, Braun N, Remuzzi G (2004) Immunosuppressive treatment for idiopathic membranous nephropathy: a systematic review. Am J Kidney Dis 44:385–401CrossRef
13.
go back to reference Olbing H, Greifer I, Bennett BP, Bernstein J, Spitzer A (1973) Idiopathic membranous nephropathy in children. Kidney Int 3:381–390CrossRef Olbing H, Greifer I, Bennett BP, Bernstein J, Spitzer A (1973) Idiopathic membranous nephropathy in children. Kidney Int 3:381–390CrossRef
14.
go back to reference Trainin EB, Boichis H, Spitzer A, Greifer I (1976) Idiopathic membranous nephropathy. Clinical course in children. N Y State J Med 76:357–360PubMed Trainin EB, Boichis H, Spitzer A, Greifer I (1976) Idiopathic membranous nephropathy. Clinical course in children. N Y State J Med 76:357–360PubMed
15.
go back to reference Ramirez F, Brouhard BH, Travis LB, Ellis EN (1982) Idiopathic membranous nephropathy in children. J Pediatr 101:677–681CrossRef Ramirez F, Brouhard BH, Travis LB, Ellis EN (1982) Idiopathic membranous nephropathy in children. J Pediatr 101:677–681CrossRef
16.
go back to reference Lee BH, Cho HY, Kang HG, Ha IS, Cheong HI, Moon KC, Lim IS, Choi Y (2006) Idiopathic membranous nephropathy in children. Pediatr Nephrol 21:1707–1715CrossRef Lee BH, Cho HY, Kang HG, Ha IS, Cheong HI, Moon KC, Lim IS, Choi Y (2006) Idiopathic membranous nephropathy in children. Pediatr Nephrol 21:1707–1715CrossRef
17.
go back to reference Jennette JC, Iskandar SS, Dalldorf FG (1983) Pathologic differentiation between lupus and nonlupus membranous glomerulopathy. Kidney Int 24:377–385CrossRef Jennette JC, Iskandar SS, Dalldorf FG (1983) Pathologic differentiation between lupus and nonlupus membranous glomerulopathy. Kidney Int 24:377–385CrossRef
18.
go back to reference Atta MG, Choi MJ, Longenecker JC, Haymart M, Wu J, Nagajothi N, Racusen LC, Scheel PJ, Brancati FL, Fine DM (2005) Nephrotic range proteinuria and CD4 count as noninvasive indicators of HIV-associated nephropathy. Am J Med 118:1288CrossRef Atta MG, Choi MJ, Longenecker JC, Haymart M, Wu J, Nagajothi N, Racusen LC, Scheel PJ, Brancati FL, Fine DM (2005) Nephrotic range proteinuria and CD4 count as noninvasive indicators of HIV-associated nephropathy. Am J Med 118:1288CrossRef
19.
go back to reference National High Blood Pressure Education Program Work Group on Hypertension Control in Children and Adolescents (2004) The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 114:555–576CrossRef National High Blood Pressure Education Program Work Group on Hypertension Control in Children and Adolescents (2004) The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 114:555–576CrossRef
20.
go back to reference Schwartz GJ, Brion LP, Spitzer A (1987) The use of plasma creatinine concentration for estimating glomerular filtration rate in infants, children, and adolescents. Pediatr Clin North Am 34:571–590CrossRef Schwartz GJ, Brion LP, Spitzer A (1987) The use of plasma creatinine concentration for estimating glomerular filtration rate in infants, children, and adolescents. Pediatr Clin North Am 34:571–590CrossRef
21.
go back to reference White RH, Glasgow EF, Mills RJ (1970) Clinicopathological study of nephrotic syndrome in childhood. Lancet 1:1353–1359CrossRef White RH, Glasgow EF, Mills RJ (1970) Clinicopathological study of nephrotic syndrome in childhood. Lancet 1:1353–1359CrossRef
22.
go back to reference The Southwest Pediatric Nephrology Study Group (1986) Comparison of idiopathic and systemic lupus erythematosus-associated membranous glomerulonephropathy in children. Am J Kidney Dis 7:115–124CrossRef The Southwest Pediatric Nephrology Study Group (1986) Comparison of idiopathic and systemic lupus erythematosus-associated membranous glomerulonephropathy in children. Am J Kidney Dis 7:115–124CrossRef
23.
go back to reference Yoshikawa N, Ito H, Yamada Y, Hashimoto H, Katayama Y, Matsuyama S, Hasegawa O, Okada S, Hajikano H, Yoshizawa H, Mayumi M, Matsuo T (1985) Membranous glomerulonephritis associated with hepatitis B antigen in children: a comparison with idiopathic membranous glomerulonephritis. Clin Nephrol 23:28–34PubMed Yoshikawa N, Ito H, Yamada Y, Hashimoto H, Katayama Y, Matsuyama S, Hasegawa O, Okada S, Hajikano H, Yoshizawa H, Mayumi M, Matsuo T (1985) Membranous glomerulonephritis associated with hepatitis B antigen in children: a comparison with idiopathic membranous glomerulonephritis. Clin Nephrol 23:28–34PubMed
24.
go back to reference Cameron JS (1990) Membranous nephropathy in childhood and its treatment. Pediatr Nephrol 4:193–198CrossRef Cameron JS (1990) Membranous nephropathy in childhood and its treatment. Pediatr Nephrol 4:193–198CrossRef
25.
go back to reference Kallen RJ, Lee SK, Aronson AJ, Spargo BH (1977) Idiopathic membranous glomerulopathy preceding the emergence of systemic lupus erythematosus in two children. J Pediatr 90:72–76CrossRef Kallen RJ, Lee SK, Aronson AJ, Spargo BH (1977) Idiopathic membranous glomerulopathy preceding the emergence of systemic lupus erythematosus in two children. J Pediatr 90:72–76CrossRef
26.
go back to reference Wakai S, Magil AB (1992) Focal glomerulosclerosis in idiopathic membranous glomerulosclerosis. Kidney Int 41:428–434CrossRef Wakai S, Magil AB (1992) Focal glomerulosclerosis in idiopathic membranous glomerulosclerosis. Kidney Int 41:428–434CrossRef
27.
go back to reference Troyanov S, Wall CA, Miller JA, Scholey JW, Cattran DC (2004) Idiopathic membranous nephropathy: definition and relevance of a partial remission. Kidney Int 66:1199–1205CrossRef Troyanov S, Wall CA, Miller JA, Scholey JW, Cattran DC (2004) Idiopathic membranous nephropathy: definition and relevance of a partial remission. Kidney Int 66:1199–1205CrossRef
28.
go back to reference Filler G, Young E, Geier P, Carpenter B, Drukker A, Feber J (2003) Is there really an increase in non-minimal change nephrotic syndrome in children? Am J Kidney Dis 42:1107–1113CrossRef Filler G, Young E, Geier P, Carpenter B, Drukker A, Feber J (2003) Is there really an increase in non-minimal change nephrotic syndrome in children? Am J Kidney Dis 42:1107–1113CrossRef
29.
go back to reference Latham P, Poucell S, Koresaar A, Arbus G, Baumal R (1982) Idiopathic membranous glomerulopathy in Canadian children: a clinicopathologic study. J Pediatr 101:682–685CrossRef Latham P, Poucell S, Koresaar A, Arbus G, Baumal R (1982) Idiopathic membranous glomerulopathy in Canadian children: a clinicopathologic study. J Pediatr 101:682–685CrossRef
30.
go back to reference Ruggenenti P, Mosconi L, Vendramin G, Moriggi M, Remuzzi A, Sangalli F, Remuzzi G (2000) ACE inhibition improves glomerular size selectivity in patients with idiopathic membranous nephropathy and persistent nephrotic syndrome. Am J Kidney Dis 35:381–391CrossRef Ruggenenti P, Mosconi L, Vendramin G, Moriggi M, Remuzzi A, Sangalli F, Remuzzi G (2000) ACE inhibition improves glomerular size selectivity in patients with idiopathic membranous nephropathy and persistent nephrotic syndrome. Am J Kidney Dis 35:381–391CrossRef
31.
go back to reference The GISEN Group (Gruppo Italiano di Studi Epidemiologici in Nefrologia) (1997) Randomised placebo-controlled trial of effect of ramipril on decline in glomerular filtration rate and risk of terminal renal failure in proteinuric, non-diabetic nephropathy. Lancet 349:1857–1863CrossRef The GISEN Group (Gruppo Italiano di Studi Epidemiologici in Nefrologia) (1997) Randomised placebo-controlled trial of effect of ramipril on decline in glomerular filtration rate and risk of terminal renal failure in proteinuric, non-diabetic nephropathy. Lancet 349:1857–1863CrossRef
32.
go back to reference Tsukahara H, Takahashi Y, Yoshimoto M, Hayashi S, Fujisawa S, Suehiro F, Akaishi K, Nomura Y, Morikawa K, Sudo M (1993) Clinical course and outcome of idiopathic membranous nephropathy in Japanese children. Pediatr Nephrol 7:387–391CrossRef Tsukahara H, Takahashi Y, Yoshimoto M, Hayashi S, Fujisawa S, Suehiro F, Akaishi K, Nomura Y, Morikawa K, Sudo M (1993) Clinical course and outcome of idiopathic membranous nephropathy in Japanese children. Pediatr Nephrol 7:387–391CrossRef
33.
go back to reference Habib R, Kleinknecht C, Gubler MC (1973) Extramembranous glomerulonephritis in children: report of 50 cases. J Pediatr 82:754–766CrossRef Habib R, Kleinknecht C, Gubler MC (1973) Extramembranous glomerulonephritis in children: report of 50 cases. J Pediatr 82:754–766CrossRef
34.
go back to reference Halevy D, Radhakrishnan J, Appel GB (2001) Racial and socioeconomic factors in glomerular disease. Semin Nephrol 21:403–410CrossRef Halevy D, Radhakrishnan J, Appel GB (2001) Racial and socioeconomic factors in glomerular disease. Semin Nephrol 21:403–410CrossRef
35.
go back to reference Trompeter RS, Evans PR, Barratt TM (1981) Gonadal function in boys with steroid-responsive nephrotic syndrome treated with cyclophosphamide for short periods. Lancet 1:1177–1179CrossRef Trompeter RS, Evans PR, Barratt TM (1981) Gonadal function in boys with steroid-responsive nephrotic syndrome treated with cyclophosphamide for short periods. Lancet 1:1177–1179CrossRef
36.
go back to reference Alexopoulos E, Papagianni A, Tsamelashvili M, Leontsini M, Memmos D (2006) Induction and long-term treatment with cyclosporine in membranous nephropathy with the nephrotic syndrome. Nephrol Dial Transplant 21:3127–3132CrossRef Alexopoulos E, Papagianni A, Tsamelashvili M, Leontsini M, Memmos D (2006) Induction and long-term treatment with cyclosporine in membranous nephropathy with the nephrotic syndrome. Nephrol Dial Transplant 21:3127–3132CrossRef
37.
go back to reference Ponticelli C, Altieri P, Scolari F, Passerini P, Roccatello D, Cesana B, Melis P, Valzorio B, Sasdelli M, Pasquali S, Pozzi C, Piccoli G, Lupo A, Segagni S, Antonucci F, Dugo M, Minari M, Scalia A, Pedrini L, Pisano G, Grassi C, Farina M, Bellazzi R (1998) A randomized study comparing methylprednisolone plus chlorambucil versus methylprednisolone plus cyclophosphamide in idiopathic membranous nephropathy. J Am Soc Nephrol 9:444–450PubMed Ponticelli C, Altieri P, Scolari F, Passerini P, Roccatello D, Cesana B, Melis P, Valzorio B, Sasdelli M, Pasquali S, Pozzi C, Piccoli G, Lupo A, Segagni S, Antonucci F, Dugo M, Minari M, Scalia A, Pedrini L, Pisano G, Grassi C, Farina M, Bellazzi R (1998) A randomized study comparing methylprednisolone plus chlorambucil versus methylprednisolone plus cyclophosphamide in idiopathic membranous nephropathy. J Am Soc Nephrol 9:444–450PubMed
Metadata
Title
Membranous glomerulonephritis: treatment response and outcome in children
Authors
Rudolph P. Valentini
Tej K. Mattoo
Gaurav Kapur
Abubakr Imam
Publication date
01-02-2009
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 2/2009
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-008-1005-9

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