Twenty five cases of idiopathic type I mesangiocapillary glomerulonephritis (MCGN) diagnosed by renal biopsy at the Department of Pediatric, Seoul National University Children's Hospital from jan. 1975 through June. 1992, were reviewed
retrospectively.
@ES The results were as follows:
@EN 1) Male to female ratio was 1.1:1 and the mean age at onset was 9.01±3.55 years. Most patients (76%) were above 8 years of age.
2) the mode of onset was quite variable. 6 children (24%) presented with nephrotic syndrome, 6(24%) with mixed nephrotic-nephritic syndrome, 6(24%) with acute nephritic syndrome, 4(16%) with recurrent hematuria. 2(8%) with asymptomatic
proteinuria
and
1(4%) with acute renal failure. Nephrotic syndrome was the initial manifestation in 12 cases, and appeared later in the course of disease in 7 cases.
3) As initial accompanying symptoms and signs, hypertension, hematuria, azotemia and glycosuria were detected in 48%, 92%, 28%, and 16% of the cases respectively. The history of preceding upper respiratory tract infection was obtained in 44% of
the
cases.
4) For the level of serum complements, it showed persistent reduction of both C3 and C4, C3 only. C4 only for 28%, 16%, and 0% of cases, respectively. But in 40% of cases te levels of C3 and C4 were normal.
5) In histopathologic examination lobular pattern of glomerulus, global sclerosis, crescents and segmental sclerosis were founded in 64%, 36%, 20% and 20% of the cases respectively. C3 deposits along the peripheral capillary walls were detected
in
all
cases with immunofluorescent microscopic studies. Subendothelinal electron dense deposits were noted in all cases and subepithelial deposits. Intramembranous deposits and mesangial deposits were noted in 35%, 15% and 30%, respectively .
6) Long-term alternate day prenisolone, methyprednisolone pulse, dipyridamole and cyclophosphamide were gi ven in uncontrolled manners and the response was poor in general. Eventually 48% showed persistent clinical pictures and only 12% achieved
complete remission.
7) The reanl survival rate was 75.7% at 3 years and 49.1% at 5 years after diagnosis of the disease.
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