Rümeyza KAZANCIOĞLU, Taner ALİOĞLU, Mehmet KENDİR, Şennur BUDAK, Birol BAYSAL, Zeynep KARAALİ, Şule KÜÇÜK, Meral TAŞAN, Fevzi BALKAN

Horseshoe kidney and nephrotic syndrome due to aa amyloidosis

Atnalı böbrek en sık görülen renal füzyon anomalisidir. Bu böbrekler normal parankimden ya da fibrotik dokudan oluşan istmus ile genellikle alt polde birleşir. Renal pelvis ön taraftadır ve üreter de önden çıkıp istmusun üzerinden geçer. Üretral duplikasyon, kriptorflizm, hipospadias gibi diğer ürogenital sistem anomalileri yanı sıra üriner sistem enfeksiyonları, hidronefroz, nefrolitiazis ve renal tümörler atnalı böbrek ile birlikte bulunabilir. Burada atnalı böbrek ve nefrotik sendromu olan bir vaka sunulmaktadır. Elli yaşında kadın hasta, polikliniğimize vücudunda yaygın şişlik nedeniyle başvurdu. Fizik muayenede anazarka tarzında ödem dışında bir özellik saptanmadı.Yirmidört saatlik idrarda protein atılımı 7,9 g/gün idi. Antinükleer antikor, romatoid faktör, HBs Ag, Anti HBs ve Anti HCV negatifti. Çekilen batın ultrasonu ve bilgisayarlı tomografide atnalı böbrek tespit edildi. Klinik ve labarotuar bulgularıyla nefrotik sendrom düşünülerek etyolojik tanı amaçlı yapılan rektal biopside damar çevresinde AAkarakteristi ğinde amiloid birikimi saptandı. Hastaya 1,5 mg/gün Kolflisin ve proteinüri için destek tedavisi verildi. Atnalı böbrek ve nefrotik sendrom birlikteliği literatürde ancak birkaç vakada tespit edilmiştir. Bizim literatür taramamıza göre, amiloidoz ile birlikte nefrotik sendrom ve atnalı böbrek henüz bildirilmemiştir. Biz bunun bir tesadüf olduğunu düşünüyoruz; ancak, atnalı böbrek ve nefrotik sendrom olan hastalarda, reflü nefropatiden başka renal bozukluklar ihtimali de göz önüne alınmalıdır

At nalı böbreği olan bir hastada aa amiloidoza bağlı nefrotik sendrom

Horseshoe kidney is the most common type of renal fusion anomaly. It consists of functioning kidneys on each side of the midline, connected at the lower poles. Urinary tract infection, hydronephrosis, calculi, and tumour of the renal pelvis are known as complications and multiple congenital anomalies can occur together with horseshoe kidney. Here, we report a patient with horseshoe kidney and nephrotic syndrome. A fifty-year old woman was admitted to our emergency department because of generalized edema. Physical examination was not remarkable, except for edema. Daily urinary protein excretion was 7900 mg/day. Antinuclear antibody, rheumatoid factor, hepatitis B surface antigen, anti-HBs, and hepatitis C virus antibody were negative. On abdominal ultrasound and computerized tomography, horseshoe kidney was revealed. Rectal biopsy was performed revealing amyloid deposition in the vessels. Amyloid was demonstrated in the vessels with Congo Red and Cyrstal Violet. Colchicine was initiated 1.5 mg per day and supportive therapy was given. Horseshoe kidney with nephrotic syndrome is extremely rare. According to our literature search, this is the first case of horseshoe kidney with amyloidosis. We believe this presentation is a coincidence; however, one should keep in mind the possibility of renal disorders other than reflux nephropathy in patients with horseshoe kidney and nephrotic syndrome.

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