All issues > Volume 28(1); 1985

Original Article

J Korean Pediatr Soc. 1985;28(1):45-57. Published online January 31, 1985.

Idiopathic Membranoproliferative Glomerulonephritis in Children.

Hae Il Cheong¹, Hee Shang Youn¹, Whan Jong Lee¹, Yong Choi¹, Kwang Wook Ko¹, Yong Il Kim²

¹Department of Pediatrics, College of Medicine, Seoul National University
²Deparment of Pathology, College of Medicine, Seoul National University

Abstract

Among total 452 children with percutaneous kidney needle biopsy due to renal disease in the Department of Pediatric, Seoul National University Hospital, from Jan. 75 to Nov.84, 20(4.4%) were confirmed as embranoproliferative glomerulonephritis(MPGN) histopathologically. And 4 out of these 20 were excluded from this report because of association of systemic disease. A review of clinical and pathological findings of the remaining 16 cases with idiopathic MPGN was done and the results were as follows; 1)Male to female ratio was 1 : 1 and the mean age at the onset of disease was 8.98±3.63 years with a tendency of frequent occurrence after the age of 8 years. 2)The mode of onset was quite variable. Five children(31.3%) presented initially with nephrotic syndrome, 4(25.0%) with mixed nephrotic-nephritic syndrome, 3(18.8%) with acute nephritic syndrome, 1(6.3%) with acute renal failure, 1(6.3%) with recurrent gross hematuria, and 2(12.5%) with asymptomatic proteinuria. And in 13(81.3%) cases out of 16, nephrotic syndrome was the initial clinical manifestation (9 cases) or appeared later in the course of disease(4 cases), and they held 5.1 %(13/256) of total idiopathic nephrotic syndrome in children in our hospital. 3)As initial accompanying symptoms and signs, hypertension, hematuria, azotemia and glycosuria were detected in 43.8%, 93.5%, 40.0% and 25.0% of cases respectively, and the proteinuria was poorly selective in all cases examined with a mean selectivity index of 0.428±0.218. 4)High serum ASO titer was noted in 13.3% and reduction of serum complement C3, C4 and CH60 were found in 62.5%, 15.4% and 50.0% of cases, respectively. 5)Response to steroid therapy was poor in general. Only 1 out of 7 with initial daily prednisolone therapy responded partially and 3 out of 9 showed late partial response to a long-term alternate day prednisolone therapy. And pulsed steroid therapy with methylpre dnisolone resulted in 1 partial response out of 2 cases. 6)Histologic examination showed 14 cases of MPGN Type 1, 1 of MPGN Type K and 1 of MPGN Type H. And segmental sclerosis, global sclerosis and crescents were found additionally in 12.5%, 37.5% and 31.3% of cases, respectively. 7)C3 deposits along the peripheral capillary walls were detected in all cases with immunofluorescent microscopic studies, and IgG, IgM, IgA and fibrinogen deposits in 92.3%, 46.2%, 23.1% and 69.2%, respectively. 8)Subendothelial electron dense deposits were noted in all cases with electron microscopic examinations except one with MPGN Type H, and subepithelial deposits, intramembranous deposits and mesangial deposits were noted in 25.0%, 16.7% and 25.0%, respectively. 9)Among all clinical and pathological findings, some parameters including the mode of onset showed slight differences between in those with maintenance of normal renal function and in those with progressive renal functional deterioration during follow-up, but the differences of all parameters had no statistical significances. 10)The clinical course was a steadily progressive one with an actuarial survival of 75.0% after 3 years and 54.0% after 5 years from onset of disease.

Keywords :Idiopathic MPGN in Children;Clinical Findings;Pathological Findings;Mode of Onset;Steroid Responsiveness;Actuarial Survival