2 patients developed the nephrotic syndrome several years after diagnoses of chronic lymphocytic leukemia. In both cases light microscopy showed membranoproliferative glomerulonephritis. Electron microscopy and immunofluorescent staining revealed electron-dense deposits and deposition of immunoglobulins and C3. Both patients had single-component IgG cryoglobulinemia. The eluted glomerular-bound protein contained IgG only. IgG in patients’ sera, cryoglobulins, and kidney eluate had kappa light chains only. Immune complexes were detected in the sera and in the cryoglobulins by the C1q binding test. Immunoadsorption studies revealed anti-IgG antibodies in the patients’ sera, cryoglobulin, and kidney eluate. Direct immunofluorescent studies using the patients’ sera, cryoglobulins, and kidney eluate on frozen sections of patients’ kidneys were positive, providing additional evidence for the immune complex nature of the glomerulonephritis. The immunohistochemical studies of our patients are suggestive of the presence of circulating IgG-anti-IgG immune complexes and their possible involvement in the pathogenesis of the glomerulonephritis and the nephrotic syndrome in these 2 cases.
AV malformations of the kidneys are rare and AV malformations replacing the normal vasculature of both kldneys, so far as we are aware, has not been previously docrlbed. The purpose of this abstract Is to descrlbe such an abnormality In a glrl aged 3 years and 10 months. She presented wlth hypertenslon (190/140mnHg). a hemolytic anemla wlth red cell fragmentation (Hb 6.1 G/dl, Retlculocytes 6.2'Z,haptoglobin 0 mg/dl), thrombocytopen la (platelets 72000/cmn) and decreased renal function (S.Creatlnlne 1.4 mg/dl. BUN 32 mg/dl, GFR 35ml/mln/1.73m ).There was no evidence of intravascular coaqulatlon. Blood renln was normal. She also had hypertrophy of the left leg. wlth prominent velns, probably due to an associated AV malformation of the leg. Arterlography showed that the vasculature throughout both kidneys. was replaced by multlple AV malformatlons. Drainage was through larqe, tortuous velns a n a s t m s l n g wlth perlureteral and pelvic velns. The Inferior vena cava was vlsuallzed early. She has been treated wlth chlorothlazlde, propranolol, hydrallazlne. methyldopa, asplrln and dlpyrldamole for 9 months. The hypertenslon Is controlled. and the platelet count has returned to normal. The Hb has risen to 10.5 G/dl but Red cell fr qmsntatlon persists. The GFR has rlsen to only 57 ml/mln/l .73mq; Dsapite two controlled investigations of childhood nephrosis totalling 373 patients, there remains a paucity of detailed clinical and pathologic information of msangial proliferative glo.mrulonephritis with the nephrotic syndrone in childhood. We had the opportunity to study 8 patients wlth the nephrotic syndrone, the m a n age of onset was 7.2 years with a range of 3.5 to 12.2 years; there were 5 males and 3 females. All patients had renal biopsies which were studied by light, electron and imnunofluorescent microscopy. The pathologic findings with hematoxylineosin showed predominant mesangial proliferation in all patients.
MESANGIAL
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