Clinical Investigation

Kidney International (1982) 22, 643–652; doi:10.1038/ki.1982.224

A multicenter study of IgA nephropathy in children. A report of the Southwest Pediatric Nephrology Study Group1

Correspondence: Dr R J Hogg, Southwest Pediatric Nephrology Study Group Central Office, Room G3.254, University of Texas Health Science Center at Dallas, 5323 Harry Hines Boulevard, Dallas, Texas 75235, USA

1 Southwest Pediatric Nephrology Study Group (SPNSG, Central Office, University of Texas Health Science Center at Dallas, Dallas, Texas): Director, Ronald J. Hogg; Pathology co-ordinators, Fred Silva, Pat Walker, Arthur G. Weinberg; Clinical co-ordinators, Shane Roy, Luther Travis, Jim Wenzl; Statistician, Joan Reisch; SPNSG Centers and individuals participating in this study: Baylor College of Medicine, Houston, Phillip Berry, Edith Hawkins, L. Leighton Hill, Sami Sanjad; Tulane University Medical Center, New Orleans, Frank Boineau, John Lewy, Pat Walker; University of Arkansas, Little Rock, Watson Arnold, A. R. Kalderon; University of Colorado, Denver, Stephen Guggenheim, Gary Lum; University of Oklahoma Medical Center, Oklahoma City, Henry Krous, James Matson, Jim Wenzl; University of Tennessee, Memphis, William Murphy, Shane Roy, Bruder Stapleton; University of Texas Health Science Center at Dallas, Billy S. Arant, Jr., Edwin H. Eigenbrodt, Mark Houser, Ruben Meyer, H. Leslie Moore, Joan Reisch, Fred Silva, Arthur G. Weinberg; University of Texas Health Science Center at Houston, Eileen Brewer, Susan B. Conley, Regina Verani; University of Texas Medical Branch, Galveston, Ben Brouhard, Tito Cavallo, Alok Kalia, Srinivasan Rajaraman, Luther Travis; preparation of this study and manuscript, Ronald J. Hogg, Fred Silva.

Received 25 March 1982; Revised 14 June 1982.

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Abstract

A multicenter study of IgA nephropathy in children. A report of the Southwest Pediatric Nephrology Study Group. Clinical and pathologic features of IgA nephropathy were evaluated in 62 children (age range, 4 to 18 years; 46 boys, 16 girls) in a collaborative study carried out by members of the Southwest Pediatric Nephrology Study Group (SPNSG). Microscopic hematuria was present in all of the patients prior to renal biopsy and was associated with gross hematuria in 85%, proteinuria (greater than or equal to 2+) in 48%, hypertension in 6%, and depressed GFR in 25% of the patients. Renal biopsy specimens were reviewed and classified into three groups on the basis of light microscopy (LM): (1) normal glomeruli (16 patients), (2) mesangial hypercellularity (25 patients), and (3) focal and segmental proliferative and/or sclerosing glomerulonephritis (21 patients). Tubulo-interstitial changes, which were present in 56% of the biopsy specimens, showed no correlation with the duration of clinical disease. Peripheral glomerular capillary wall changes shown by electron microscopy (EM) were present in 40% of the biopsy specimens and were associated with more severe glomerular changes revealed by LM. Proteinuria and episodes of gross hematuria were associated with more severe histologic changes (group 3) and peripheral capillary wall changes demonstrated by EM. Mild patterns of glomerular damage (groups 1 and 2) were associated with female sex. The studies show that evidence of tubulo-interstitial damage and peripheral glomerular capillary wall changes are not uncommon in children with IgA nephropathy and suggest that these features may be harbingers of a more serious prognosis than previously thought.

Etude multicentrique de la néphropathie à IgA chez des enfants. Le rapport du Southwest Pediatric Nephrologic Study Group. Les caractéristiques cliniques et pathologiques de la néphropathie à IgA ont été étudiées chez 62 enfants (4 à 18 ans; 46 garçons, 16 filles) dans une étude coopérative faite par les membres du Southwest Pediatric Nephrologic Study Group (SPNSG). Une hématurie microscopique était présente chez tous les malades avant la biopsie rénale et était associée à une hématurie macroscopique chez 85%, à une protéinurie (greater than or equal to 2+) chez 48%, à une hypertension chez 6%, et à une diminution de GFR chez 25% des malades. Les biopsies rénales ont été revues et classées en trois groupes sur la base de la microscopie optique (LM): (1) glomérules normaux (16 malades), (2) hypercellularité mésangiale (25 malades), et (3) hypercellularité mésangiale avec glomérulonéphrite focale segmentaire proliférative et/ou sclérosante surajoutée (21 malades). Les modifications tubulo-interstitielles, qui étaient présentes dans 56% des biopsies, n'étaient pas corrélées avec la durée de la maladie clinique. Des modifications de paroi du capillaire glomérulaire périphérique en microscopie electronique (EM) étaient présentes dans 40% des biopsies et étaient associées avec des modifications glomérulaires plus sévères en LM. La protéinurie et les épisodes d'hématurie macroscopique étaient associés avec des modifications histologiques plus sévères (groupe 3) et des modifications de la paroi capillaire périphérique en EM. Des aspects modérés de lésion glomérulaire (groupes 1 et 2) étaient associés chez les filles. Ces études montrent que l'existence de lésions tubulointerstitielles et de modifications de la paroi capillaire glomérulaire périphérique ne sont pas rares chez les enfants atteints de néphropathie à IgA et suggèrent que ces caractéristiques pourraient être les prémices d'un pronostic plus sérieux qu'on le pensait auparavant.

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