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Truncus Arteriosus ile Birlikte Görülen Fokal Segmental Glomerüloskleroz Olgusu: Anjiyotensin Reseptör Antagonisti ve Siklosporin A’nın Etkinliği

A Case of Focal Segmental Glomerulosclerosis Accompanied By Truncus Arteriosus: Effectiveness of Angiotensin Receptor Antagonist and Cyclosporine A

Journal Name:

  • Türk Nefroloji, Diyaliz ve Transplantasyon Dergisi

Publication Year:

  • 2009

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Although glomerular injury has been recognized as a prominent complication of cyanotic congenital heart disease (CCHD), the nephrotic syndrome is rarely observed. A 23- year-old male with CCHD presented with edema in the eyelids and ankles since three months. He was cyanotic at birth and was diagnosed as having persistent truncus arteriosus and atrial septal defect. On admission, blood pressure was 130/90 mmHg and heart rate 76/minute. Laboratory findings revealed 5 g/day proteinuria, hemoglobin 17.1 g/dL, hematocrit 55.5%, serum creatinine 1.8 mg/dL, total protein 4.1 g/dL, serum albumin 2.2 g/dL and hypoxemia. Renal biopsy was performed and showed global sclerosis and focal segmental glomerulosclerosis (FSGS). Phlebotomy was performed and prednisolone 1 mg/kg/day and losartan 100 mg/day were started. After three months, cyclosporine A (CsA) (5 mg/kg/ day) was added to the treatment with a proteinuria level of 5.9 g/day. On the eight month of treatment proteinuria was reduced to 1.9 g/day with a serum creatinine level of 1.5 mg/dL, and albumin 3 g/dL. In this rare case, partial remission which was achieved by CsA and angiotensin receptor antagonist showed the important role of glomerular hyperfiltration in the development of CCHD-associated FSGS. When the secondary FSGS causes were ruled out, CCHD should be kept in mind and echocardiographic assessment should be performed.
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Abstract (Original Language): 
Glomerül hasarı, siyanotik konjenital kalp hastalığının (SKKH) önde gelen komplikasyonlarından olmasına rağmen, bu hastalarda nefrotik sendrom nadir olarak gözlenmektedir. SKKH olan 23 yaşında erkek hasta 3 aydır devam eden göz kapaklarında ve ayak bileklerinde ödem yakınması ile başvurdu. Doğumunda siyanotik olan hastaya, o dönemde yapılan incelemeler sonucunda persistan trunkus arteriosus ve atrial septal defekt tanıları konulmuş. Başvuru sırsında, kan basıncı 130/90 mmHg ve kalp hızı 76/dakika idi. Laboratuvar incelemelerinde; 5 gr/gün proteinüri, hemoglobin 17,1 g/dL, hematokrit %55,5, serum kreatinin 1,8 mg/dL, total protein 4,1 g/dL, albumin 2,2 g/dL ve hipoksemi saptandı. Böbrek biyopsisi yapıldı ve sonucunda glomerüllerde global skleroz ve fokal segmental glomerüloskleroz (FSGS) görüldü. Tedavi olarak flebotomi, prednizolon 1 mg/kg/gün, losartan 100 mg/ gün başlandı. Tedavinin 3. ayında proteinüri değeri 5,9 g/gün saptanınca siklosporin A (CsA) (5 mg/kg/gün) tedaviye eklendi. Tedavinin 8. ayında proteinüri 1,9 g/gün, serum kreatinin 1,5 mg/dL ve serum albumin 3 g/dL bulundu. Nadir görülen bu olguda, CsA ve anjiyotensin reseptör antagonisti ile elde edilen kısmi remisyon, SKKH ile ilişkili FSGS gelişiminde glomerüler hiperfiltrasyonun önemli bir rol oynadığını göstermektedir. Ayrıca FSGS’li olgularda sekonder FSGS nedenleri gözden geçirilirken, hastalar SKKH açısından sorgulanmalı ve ekokardiyografik değerlendirme yapılmalıdır.
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  • 3
123-126
  • Turkish

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Türk Nefroloji Diyaliz ve Transplantasyon Dergisi
Turkish Nephrology, Dialysis and Transplantation Journal
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