Çocuklarda Henoch-Schönlein Purpura Nefriti

Henoch-Schönlein purpurası çocuklarda sık görülen ve küçük çaplı damarları tutan bir vaskülittir. Cilt, eklem,gastrointestinal sistem ve renal tutulum ile kendini gösterir. Genellikle böbrek tutulum dışında kendi kendineiyileşebilen bir hastalıktır. Henoch-Schönlein purpuralı çocukların %30-50’sinde böbrek tutulumu görülür. Renaltutulum için risk faktörleri arasında 7 yaş üstü olmak, şiddetli gastrointestinal bulguların olması ve purpuranın uzunsürmesi gözlenmiştir. Böbrek tutulumunda mikroskopik ve/veya makroskopik hematüri, proteinüri, daha seyrek olaraknefrotik sendrom, akut nefritik sendrom, hipertansiyon ve akut böbrek yetmezliğine kadar uzanan değişik klinik şekillergörülebilir. Henoch-Schönlein purpura nefriti ve IgA nefropatisi glomerüllerde glikozile IgA1’in depolanması ile ilgilihastalıklardır. Hastalık renal tutulum, tedaviye bağlı olarak sekelsiz iyileşebileceği gibi, ağır kresentik glomerülonefrit vekronik böbrek yetmezliği ile sonuçlanabilir. Bu nedenle renal tutulum hastalığın prognozunu belirleyen en önemlibulgudur. Bu derlemede Henoch-Schönlein purpurasının renal tutulumu ve tedavisi tartışılmıştır.

Henoch-Schonlein Purpura Nephritis in Children

Henoch-Schonlein purpura is a vasculitis commonly seen in children and holding small diameter vessels. It manifests with skin, joint, gastrointestinal system and renal involvement. It is usually a self-healing disease other than renal involvement. Kidney involvement is frequently seen in 30-50% children with Henoch-Schönlein purpura. Risk factors for renal involvement were over 7 years of age, severe gastrointestinal findings, and long duration of purpura. Renal involvement can be seen in various clinical forms ranging from microscopic and/or macroscopic hematuria, proteinuria, rarely nephrotic syndrome, acute nephritic syndrome, hypertension and acute renal failure. HenochSchönlein purpura nephritis and IgA nephropathy are diseases related to the storage of glycosylated IgAlin glomeruli. Disease renal involvement may improve without sequelae due to treatment, as well as severe crescentic glomerulonephritis and chronic renal failure. Therefore, renal involvement is the most important finding that determines the prognosis of the disease. In this review, renal involvement and treatment of Henoch-Schönlein purpura nefritis has been discussed.

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