Rapidly Progressive Glomerulonephritis in Children: A Report of Thirteen Cases and a Review of the Literature

Abstract

Summary MATERIALS AND METHODSThe clinical course and outcome of rapidly progressive glomerulonephritis (RPGN) of variable etiology are not well defined in children. The present investigation reports on the clinical characteristics, the course and outcome, as well as the results of treatment of 13 children with apparent postinfectious RPGN. Three of 7 patients with documented streptococcal RPGN and 3 of 6 with RPGN of nonstrept&occal etiology progressed to chronic renal failure. In some patients, anticoagulant and anti--platelet therapy appear to have improved survival. The severity of crescent formation, not the presumable etiology, appears to be a reliable prognosticator. SpeculationA number of disease processes can result in renal damage of sufficient severity to cause crescent formation. The induction, resolution, or progression of crescents to sclerosis may be influenced by anticoagulant therapy.A retrospective analysis was performed on all pediatric patients seen at The University of Texas Medical Branch at Galveston since 1963 who had a rapid deterioration of renal function in association with an apparent acute onset of glomerulonephritis. Specifically, patients were included in the study population only if they met all of the following requirements: 1) documented glomerulonephritis with red blood cells casts present in the urine or the presence of red blood cells in the urine in association with proteinuria (by Labstix); 2) kidneys of normal or near-normal size, as determined by iv pyelography or tomography; 3) evidence of increasing azotemia (blood urea nitrogen, creatinine) over a maximum period of four months; 4) a renal biopsy specimen in which a substantial percentage of glomeruli were involved. This involvement included variable proliferation of cells in Bowman's space, crescents circumscribing in arc of 180' or more or glomerular obsolescence.Laboratory data, obtained by standard hospital procedures when the patient was first evaluated, were used in the tabulation of data. Oliguria was defined as a urine output of less than 200 cc/m2/day. Renal biopsies were performed 2 io 16 weeks after the progressive glomerulone~hritis (RPGN), recognized onset of symptoms by a technique which has been described Ellis (13) and further defined by Heptinstall (21), was first de-previously scribed as an unusual form of acute glomerulonephritis (AGN) Sections for light microscopy were fixed in 2% paraformaidewhich progressed to renal failure, in contrast to the typical course hyde and 2-pm sections were cut and stained with 2 or more of of rapid resolution. A clinical feature which seemed to separate the following: periodic acid-~chiff, methenamhe silver, hematox. RPGN from AGN was a period of prolonged oliguria. ylin and eosin, and Gomori's trichrome. For irnmunofluorescence, It is now known that the renal histology in RPGN is character-tissues were snap-frozen in -700 isopentane; then 4-pm sections ized by crescent formation with or without endocapillary prolif-were cut and with monospec~fic antisera to human imera...

Crescentic Glomerulonephritis in Children: A Review of 43 Cases

“…however, noted that in all of them, crescents were generally cellular and there was little evidence of chronic histologic changes. The findings of an earlier study [3] and our observations do not support such a view and suggest that the severity of the crescentic changes is the chief prognostic determinant. In adults, a postinfectious etiol ogy of rapidly progressive GN is considered to indicate a better prognosis [4,5,7], In our series, besides the 11 patients with poststreptococcal GN, 8 others had endocapillary proliferation in the glomeruli, and immune reac tants were demonstrated in 3 of 4 cases.…”

“…The outcome in crescentic GN appears to be related to the severity and extent of crescent formation and disrup tion of Bowman's capsule [2,3,7,10,11,19], although findings to the contrary have also been occasionally reported [20,21], A number of clinical features including gross hematuria, hypertension and heavy proteinuria did not have any correlation with the severity of crescent for mation or with the outcome in our cases. However, the number of patients who did not have these features was very small.…”

“…Crescentic GN usually manifests with an acute ne phritic syndrome [1][2][3][4], In a small number of adult patients, however, an insidious onset with nephrotic syn drome [ 10,17], and rarely with nonspecific symptoms [9], with a gradual deterioration of renal function has been observed. A similar evolution of the disease was found in 28% of our cases.…”

“…This morphology is usually associated with an acute nephritic syndrome and rapidly increasing renal crescentic GN [5,10,11 ]. A wide variety of disorders may be associated with such a histology [1], The prognosis is generally poor [ 1,3,4,7,12], although the possibility of recovery is considered to be higher in poststreptococcal and postinfectious cases [2,5,7,13]. The role of immuno suppressive and other specific therapy is not clearly estab lished, and the long-term outcome in patients who re cover is uncertain [8,9].…”

Clinical spectrum and outcome of crescentic glomerulonephritis in children in developing countries

“…Over the years, there have been significant advancements in supportive, as well as specific, therapy. There is a paucity of reports from developing countries [2][3][4][5][6][7][8][9] and there are hardly any series from other parts of the world also. This study was conducted to evaluate the aetiology, clinical spectrum, treatment and outcome of crescentic glomerulonephritis in children.…”