Abstract
Since advent of renal biopsy, the definition of a type of persistent nephritis characterized by focal and segmental lesions has been possible. Heptinstall has recognized this lesion in adults in the absence of lupus, bacterial endocarditis, anaphylactoid purpura or periatritis. We have observed 11 cases of focal nephritis in children not attributable to any of the above entities. All the patients have been normocomplementemic and have had a course characterized by a mild-to-severe nephrotic syndrome, usually with signs of nephritis. The data suggest that signs and symptoms develop rapidly after onset, without a long, silent, subclinical stage. In 9 of the cases, onset was preceded by an upper respiratory infection, but ASO titers were usually not elevated. The entity is important to recognize because of the beneficial effects of steroid therapy. In 3 of our patients not treated with steroid initially, the nephriticnephrotic picture subsided, but all signs and symptoms recurred several months later, usually following a respiratory infection. After treatment in these 3 patients as well as in 7 of 8 treated shortly after onset, no recurrences have been noted in follow-ups ranging from 9 months to 4 years. The 11 th patient never responded to therapy and eventually succumbed with a severe nephrotic syndrome. In the remaining 10 patients, signs of the nephrotic syndrom have slowly disappeared with therapy, and several show no evidence of renal disease except for focal glomerular scarring. The similar pathological picture, clinical course and therapeutic results in the 10 responsive patients suggest that focal nephritis in children is a disease entity. Its diagnosis rests on typical histopathological changes by renal biopsy in a normocomplementemic patient. (APS)
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West, C., McAdams, A. & Northway, J. 42 Focal Nephritis, a Form of Normocomplementemic Persistent Glomerulonephritis Responsive to Steroid. Pediatr Res 1, 211 (1967). https://doi.org/10.1203/00006450-196705000-00049
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DOI: https://doi.org/10.1203/00006450-196705000-00049