[Berger's disease in childhood].
Berger's disease or IgA nephropathy (NIgA) is the most common form of glomerulonephritis in the world. In children macroscopic haematuria is the first sign in about 80% of the patients. Renal failure appears in 20% of cases after twenty years of follow-up. The most important prognosis indicators are a nephrotic syndrome at the onset, a proteinuria > 1 g/24 hours, diffuse tubulo-interstitial lesions and extracapillary proliferation with crescents in more than 50% of the glomeruli. The pathogenic mechanisms are just emerging and involve a disrupted process of the systemic tolerance to mucosal antigen with abnormal mucosal gamma delta T cell repertoire, abnormally glycosylated IgA1 molecules and a down-regulation of Fc alpha receptors on blood cells. After IgA deposition, the mechanisms of mesangial cell damage and activation involve vascular factors as endothelin/nitric oxide system, cytokines and growth factors such as interleukine-6, platelet derived growth factor and transforming growth factor beta. There is no curative treatment but steroids are useful in diffuse proliferative extracapillary forms, when histological activity score is high with a short delay between diagnosis and treatment, or for moderately severe NIgA with normal renal function.
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Service de néphrologie pédiatrique, hôpital des Enfants, 330, avenue de Grande-Bretagne, BP 3119, 31026 Toulouse, France.
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D002648:Child; D016207:Cytokines / Q000276:immunology; D016232:Endothelins / Q000276:immunology; D005922:Glomerulonephritis, IGA / Q000209:etiology / Q000473:pathology / Q000628:therapy; D006801:Humans; D007107:Immune System; D007070:Immunoglobulin A / Q000276:immunology / Q000378:metabolism; D015850:Interleukin-6 / Q000276:immunology; D011379:Prognosis
D000305:Adrenal Cortex Hormones; D016207:Cytokines; D016232:Endothelins; D007070:Immunoglobulin A; D015850:Interleukin-6
La maladie de Berger chez l'enfant.