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HORSESHOE KIDNEYAND NEPHROTIC SYNDROME DUE TO AA AMYLOIDOSIS

AT NALI BÖBREĞİ OLAN BİR HASTADA AA AMİLOİDOZA BAĞLI NEFROTİK SENDROM

Journal Name:

  • İstanbul Tıp Fakültesi Dergisi

Publication Year:

  • 2006

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
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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Abstract (Original Language): 
Atnal› böbrek en s›k görülen renal füzyon anomalisidir. Bu böbrekler normal parankimden ya da fibrotik dokudan oluflan istmus ile genellikle alt polde birleflir. Renal pelvis ön taraftad›r ve üreter de önden ç›k›p istmusun üzerinden geçer. Üretral duplikasyon, kriptorflizm, hipospadias gibi di¤er ürogenital sistem anomalileri yan› s›ra üriner sistem enfeksiyonlar›, hidronefroz, nefrolitiazis ve renal tümörler atnal› böbrek ile birlikte bulunabilir. Burada atnal› böbrek ve nefrotik sendromu olan bir vaka sunulmaktad›r. Elli yafl›nda kad›n hasta, poliklini¤imize vücudunda yayg› n flifllik nedeniyle baflvurdu. Fizik muayenede anazarka tarz›nda ödem d›fl›nda bir özellik saptanmad›.Yirmidört saatlik idrarda protein at›l›m› 7,9 g/gün idi. Antinükleer antikor, romatoid faktör, HBs Ag, Anti HBs ve Anti HCV negatifti. Çekilen bat›n ultrasonu ve bilgisayarl› tomografide atnal› böbrek tespit edildi. Klinik ve labarotuar bulgular› yla nefrotik sendrom düflünülerek etyolojik tan› amaçl› yap›lan rektal biopside damar çevresinde AAkarakteristi¤ inde amiloid birikimi saptand›. Hastaya 1,5 mg/gün Kolflisin ve proteinüri için destek tedavisi verildi. Atnal› böbrek ve nefrotik sendrom birlikteli¤i literatürde ancak birkaç vakada tespit edilmifltir. Bizim literatür taramam›za göre, amiloidoz ile birlikte nefrotik sendrom ve atnal› böbrek henüz bildirilmemifltir. Biz bunun bir tesadüf oldu¤unu düflünüyoruz; ancak, atnal› böbrek ve nefrotik sendrom olan hastalarda, reflü nefropatiden baflka renal bozukluklar ihtimali de göz önüne al›nmal›d›r.
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  • 1
23-25
  • English

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