Sebahat TÜLPAR, Funda BAŞTUĞ, M. Hakan POYRAZOĞLU, Zübeyde GÜNDÜZ, Hülya AKGÜN, Ali YIKILMAZ

Kresentik glomerülonefritle başvuran bir Wegener granülomatozu olgusu

Wegener granülomatozu çocukluk çağında nadir görülen, tedavi edilmediğinde morbidite ve mortalite ile seyredebilen bir vaskülittir. Başvurusunda pulmoner renal sendrom tablosunda olan 10 yaşındaki bir kız hasta sunuldu. Yapılan tetkikler sonucunda Wegener Granülomatozu tanısı konup immunosupresif tedavi başlandı. Böbrek biyopsisi kresentik glomerülonefrit ile uyumluydu. Hasta şu anda evre 4 kronik böbrek yetmezliği tanısı ile takip edilmektedir.

Wegener granulomatosis presented with crescentic glomerulonephritis: Case report

Wegener granulomatosis is a rare vasculitis in childhood and is associated with morbidity and mortality in untreated cases. We reported a 10 year-old female who presented with pulmonary renal syndrome. Laboratory investigations revealed the diagnosis of Wegener granulomatosis, and immunosuppressive therapy was started. Renal biopsy was consistent with crescentic glomerulonephritis. The patient has been still followed-up with stage 4 chronic kidney disease.

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1. Dedeoglu F, Sundel R.P. Vasculitis in Children. Pediatr Clin N Am 2005; 52: 547-75.
2. Ozen S, Bakkaloglu A, Dusunsel R, et al. Childhood vasculitides in Turkey: a nationwide survey. Clin Rheumatol 2007; 26:196-200.
3. Valentini RP, Smoyer WE. Renal Vasculitis. In: Avner ED, Harmon WE, Niaudet P, eds. Pediatric Nephrology, 5th edn. USA, Lippincott Williams&Wilkins, 2004: 835-849.
4. Papiris SA, Manali ED, Kalomenidis I, et al. Bench-to-bedside review: Pulmonary–renal syndromes –an update for the intensivist. Crit Care 2007, 11: 213.
5. Von Vigier RO, Trummler SA, Laux-End R, et al. Pulmonary renal syndrome in childhood: a report of twenty-one cases and a review of the literature. Pediatr Pulmonol 2000; 29:382–38.
6. Vehaskari VM, Aviles DH. Acute glomerulonephritis and rapidly progressive glomerulonephritis. In: Kher KK, Schnaper HW, Makker SP (eds), Clinical Pediatric Nephrology. Second edition. London: Informa UK Ltd; 2007. p: 145-154.
7. Arslan Ş. Hemolitik Üremik Sendromlar. Türkiye Klinikleri Pediatri Özel Dergisi 2004, 2: 104-111.
8. Rees L, Webb NJA, Brogan PA. Vasculitis. In: Rees L, Webb NJA, Brogan PA, eds. Paediatric Nephrology, 1th edn. New York, Oxford University Pres, 2007: 297-316.
9. Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006; 65:936-41.