Abstract
The present study was carried out to determine clinical and immunopathological features of IgA nephropathy in children.
Of 224 children who underwent renal biopsy between 1975 & 1979 and on whom the specimens were studied by immunofiuorescence, 35 cases (male 22, female 13, ages 4 to 17 years) of IgA nephropathy were detected. Their clinical, immunological and histological characteristics were compared to that of 71 cases of IgA nephropathy found in 311 biopsied specimens of adult patients. All the specimens were analysed by a routine light microscopy and immunofluorescent study for immunoglobulins, β1C, β1E, properdin and fibrinogen. In order to estimate mesangial cell proliferation, matricial increase, mesangial deposition, and tubulo-interstitial changes quantitatively, each specimen was analysed on our scoring system. Results are summerized as follows.
1) Clinical manifestations: 23 (65.7%) were presented by chance proteinuria and/or hematuria, and 7 (20%) by macroscopic hematuria. During the course of illness, 5 (14.3%) developed nephrotic syndrome and 15 (42.5%) macroscopic hematuria. They were followed over a mean period of 52.1 months: 9 improved, 28 unchanged, and 1 deteriorated. It may suggest better prognosis in pediatric patients despite a higher incidence of heavy proteinuria and nephrotic syndrome.
2) Serum IgA levels: Serum IgA levels were elevated in 7 of 26 children above 10 years of age but remained within normal range in all of 9 children below 10 years.
3) Immunopathological study: In comparison with adult cases, mesangial cell proliferation was more prominent in majority of the 35 cases, but increase in mesangial matrix and deposits, and tubulo-interstitial changes were milder. Mesangial immunofluorescence was unequivocally demonstrated in all of the 35 children, being composed of IgA s̄ β1C in 4, IgA c̄ β1C in 12, and complex immunogloblins c̄ β1C in 19. In the adult patients, however, β1C was stainable in the mesangium in all but one of the 71 cases.
The present data suggests that IgA nephropathy in children differs distinctively from that of adults in clinical, immunological, and pathological manifestations.
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Nakahara, C., Aosai, F., Hasegawa, O. et al. IgA NEPHROPATHY IN CHILDREN: A MODIFIED VIEW OF CLINICO-PATHOLOGICAL CHARACTERISTICS. Pediatr Res 14, 995 (1980). https://doi.org/10.1203/00006450-198008000-00133
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DOI: https://doi.org/10.1203/00006450-198008000-00133