Hypocomplementemic and normocomplementemic persistent (chronic) glomerulonephritis; clinical and pathologic characteristics

West, Clark D.; McAdams, A. James; McConville, Janice M.; Davis, Neil C.; Holland, Nancy H.

doi:10.1016/s0022-3476(65)80213-x

Cited by 206 publications

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“…The presence of C3NeF in detectable amounts in both patients suggest that C3LyNeF, formed by the reaction of C3NeF and cofactor, was responsible for the low Dr. Vallota is a Fellow of the National Kidney Foundation. INTRODUCTION Membrano-proliferative glomerulonephritis, also designated progressive or hypocomplementemic persistent glomerulonephritis, is characterized by distinctive changes in glomerular morphology and, usually in children, by a marked and prolonged hypocomplementemia (1)(2)(3). Labeled antibody studies show that glomerular-bound C3 is consistently present and at times Clq and IgG can be demonstrated (4).…”

mentioning

confidence: 99%

Continuing C3 breakdown after bilateral nephrectomy in patients with membrano-proliferative glomerulonephritis

Vallota¹,

Forristal²,

Spitzer³

et al. 1971

J. Clin. Invest.

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A B S T R A C T Serum levels of complement components and of C3 nephritic factor (C3NeF) were measured serially in two patients with membrano-proliferative glomerulonephritis who were subjected to bilateral nephrectomy and maintained by peritoneal dialysis for 2 wk before renal transplantation. In both patients, low levels of C3 and high levels of preformed alpha 2D, a C3 breakdown product, were present before nephrectomy and remained essentially unchanged during the anephric period. With transplantation, C3 levels rose towards normal and alpha 2D disappeared from the serum. The serum of both patients contained detectable amounts of C3NeF, a factor which has been shown to react with a cofactor found in normal serum to form an enzyme, designated C3 lytic nephritic factor (C3LyNeF), which will cleave C3 to form the breakdown products, plA and alpha 2D. The level of C3NeF was high in one patient before nephrectomy, increased somewhat during the anephric period, and fell after transplantation. In the other patient, the C3NeF level was initially lower, remained relatively constant during the anephric period, and was not significantly affected by transplantation. In both patients, levels of C4 and C5 were either normal or elevated over the period of the study and bore no relationship to the C3 level. The following conclusions can be drawn from the data. The high levels of alpha 2D during the anephric period and the disappearance of this protein as C3 levels approach normal at the time of transplantation indicate that the low C3 levels were largely the result of C3 breakdown rather than diminished synthesis. The presence of C3NeF in detectable amounts in both patients suggest that C3LyNeF, formed by the reaction of C3NeF and cofactor, was responsible for the low Dr. Vallota is a Fellow of the National Kidney Foundation.

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confidence: 99%

Continuing C3 breakdown after bilateral nephrectomy in patients with membrano-proliferative glomerulonephritis

Vallota¹,

Forristal²,

Spitzer³

et al. 1971

J. Clin. Invest.

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“…Second, primary MPGN usually shows low serum C3 associated with predominant C3 deposition. Hypocomplementemia is considered to be present in about 50% of cases at the initial onset time and 80-95% during the course (West et al 1965;Habib et al 1973;Iitaka et al 1995), whereas these patients showed no hypocomplementemia during the entire period. In the present patient, the lack of hypocomplementemia, intensive IgA as well as IgG deposition, and favorable prognosis do not positively support the diagnosis of MPGN.…”

Section: Discussionmentioning

confidence: 99%

Infantile Immunoglobulin A Nephropathy Showing Features of Membranoproliferative Glomerulonephritis

Kurosu

Oka

Hamaguchi

et al. 2012

Tohoku J. Exp. Med.

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Immunoglobulin A nephropathy (IgAN) showing predominant IgA and complement 3 (C3) deposition on the mesangium is an immune complex-mediated glomerulonephritis. This renal disease is the most common primary glomerular disease worldwide. However, infantile onset of IgAN is rare. In the present patient, urinary protein and occult blood were detected in a girl aged 1 year and 8 months on urinalysis at a nursery school. Despite being young, a kidney biopsy was performed for diagnosis and the correct choice of therapy. Glomerular mesangial cell proliferation and a double contour of the glomerular basement membrane (GBM) resembling a railroad track were noted on light microscopy. Therefore, the patient was diagnosed morphologically with membranoproliferative glomerulonephritis (MPGN), because mesangial hypercellularity and thickening of the GBM were identified. However, on immunofluorescent staining, the deposition of immune complexes mainly consisting of IgA, IgG, and C3 was noted in the mesangial region and glomerular capillary loops. On electron microscopy, electron-dense deposits were recognized in the subendothelial and paramesangial regions associated with mesangial cell interposition into the subendothelial space. Autoimmune diseases and infection-associated secondary glomerulonephritis were clinically excluded, because there were no relevant signs or symptoms. Steroid treatment was initiated and findings of urinalysis were normalized within 8 months. This patient was finally diagnosed with IgA nephropathy showing the features of MPGN. The present patient was the youngest among reported cases of IgA nephropathy, suggesting that early onset of IgAN is associated with an MPGN-like lesion. The present report provides information for pathogenesis of IgA nephropathy.

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“…This network linked investigators in the United States and Europe and conducted remarkably informative studies of the value of renal biopsy as a tool and the correlation of biopsy findings with treatment and prognosis (18,29,30). The use of light microscopy, immunofluorescence, and electron microscopy techniques by the ISKDC and other groups permitted the clinicopathologic identification of several important renal disorders including membranoproliferative glomerulonephritis (31,32), IgA nephropathy (Berger disease) (33), and membranous nephropathy (34).…”

Section: Emergence Of Pediatric Nephrology As a Distinct Disciplimentioning

confidence: 99%

“…Later, Boston Children's Hospital became another important training site. These centers, respectively, focused on animal models of glomerular disease, extremely wellconducted clinicopathologic studies in children, and a fundamental immunologic approach to understanding glomerulonephritis including the role of complement pathway consumption and T cell and B cell function (17,32,35). The physiologic approach, first espoused by Homer Smith, was the mode of operation at Albert Einstein, Buffalo, Georgetown, University of California -San Francisco, and University of TexasGalveston.…”

Section: Development Of Training Programs: 1970 -1980mentioning

confidence: 99%

The Development of Pediatric Nephrology

CHESNEY

2002

Pediatric Research

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The following is the first article in this series. It Pediatric nephrology, as a discipline, arose from descriptive studies of childhood glomerulonephritis in Europe and the field of pediatric metabolism in the United States. While pediatric scientists before 1950 were concerned with fluid and electrolyte metabolism, regulation of intracellular and extracellular fluid, acid-base homeostasis, and parenteral fluid therapy, the defined field of nephrology developed after the Second World War around six major advances: ACTH and glucocorticoid therapy for nephrotic syndrome; renal biopsy to diagnose glomerular disease; the role of immunologic factors in glomerular injury; the use of dialysis as renal replacement therapy; renal transplantation as the optimal form of therapy in children with end stage renal failure; and recognition of renal disease in the etiology of 80% of cases of childhood hypertension. These discoveries led to focused research, the definition of specific training in nephrology, establishment of an American, European, and an International Society of Pediatric Nephrology, as well as an American SubBoard of Pediatric Nephrology, and the inception of a journal, Pediatric Nephrology, now in its 15th year. Major research themes have included developmental nephrology, transplantation immunology, and concerns about growth in children with renal disease. Many clinical entities have been described in detail, some of which are almost confined to children. The scientific basis of pediatric nephrology, ongoing patient care needs, and its technical aspects -renal biopsy, dialysis and transplantationassure its continuing future as a major pediatric discipline on all continents. (Pediatr Res 52: 770-778, 2002) I. THE ORIGINS OF INTEREST IN CHILDHOOD RENAL DISEASES: 1820 -1950The development of the discipline of pediatric nephrology arose from clinical research conducted in the 130-year period from 1820 to 1950. It was during this period that pediatric scientists became interested in the definition of glomerular disorders and in fluid and electrolyte metabolism, the maintenance of normal volume and tonicity and acid-base status, as well as the pathophysiology of such disorders as rickets and diarrhea (1-5) ( Table 1).Pediatric kidney disease was initially considered from a descriptive rather than quantitative perspective. Early treatises on kidney diseases in children were remarkably accurate in terms of their clinical descriptions of various renal disorders, particularly glomerular conditions. In the 11th edition of Eduard Henoch's classic text Kinderkrankheiten (6), he describes

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Hypocomplementemic and normocomplementemic persistent (chronic) glomerulonephritis; clinical and pathologic characteristics

Cited by 206 publications

References 0 publications

Continuing C3 breakdown after bilateral nephrectomy in patients with membrano-proliferative glomerulonephritis

Continuing C3 breakdown after bilateral nephrectomy in patients with membrano-proliferative glomerulonephritis

Infantile Immunoglobulin A Nephropathy Showing Features of Membranoproliferative Glomerulonephritis

The Development of Pediatric Nephrology

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