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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

REFERENCES

References: 

1. Crupi G, Macartney FJ, Anderson RH: Persistent truncus
arteriosus. A study of 66 autopsy cases with special
reference to definition and morphogenesis. Am J Cardiol
1977; 40: 569-578
2. Spear GS. The glomerulus in cyanotic congenital heart
disease and primary pulmonary hypertension. Nephron
1964; 29: 238-248
3. Fujimoto Y, Matsushima M, Tsuzuki K, Okada M, Shibata
M, Yanase Y, Usui K, Nagashima M: Nephropathy of
cyanotic congenital heart disease: clinical characteristics
and effectiveness of an angiotensin-converting enzyme
inhibitor. Clin Nephrol 2002; 58: 95-102
ÇALIŞKAN Y et al: A Case of Focal Segmental
Glomerulosclerosis Accompanied By Truncus Arteriosus
Cilt/Vol. 18, No, 3, 2009 Sayfa/Page 123-126
Türk Nefroloji Diyaliz ve Transplantasyon Dergisi
Turkish Nephrology, Dialysis and Transplantation Journal
4. Watanabe Y, Fukuzawa Y, Inaguma D: Angiotensin
converting enzyme inhibitor improves nephrotic syndrome
associated with cyanotic congenital heart disease. Clin
Nephrol 1996; 45: 362-363
5. Hida K, Wada J, Yamasaki H, Nagake Y, Zhang H,
Sugiyama H, Shikata K, Makino H: Cyanotic congenital
heart disease associated with glomerulomegaly and focal
segmental glomerulosclerosis: Remission of nephrotic
syndrome with angiotensin converting enzyme inhibitor.
Nephrol Dial Transplant 2002; 17: 144-147
6. Hagley MT, Murphy DP, Mullins D, Zarconi J: Decline
in creatinine clearance in a patient with glomerulomegaly
associated with a congenital cyanotic heart disease. Am J
Kidney Dis 1992; 20: 177-179
7. Perloff JK, Latta H, Barsotti P: Pathogenesis of the
glomerular abnormality in cyanotic congenital heart
disease. Am J Cardiol 2000; 86: 1198-1204
8. Hayabuchi Y, Matsuoka S, Akita H, Kuroda: Hyperuricaemia
in cyanotic congenital heart disease. Eur J Pediatr 1993;
152: 873-876
9. Hamada T, Hisatome I, Kinugasa Y, Matsubara K, Shimizu
H, Tanaka H, Furuse M, Sonoyama K, Yamamoto Y,
Ohtahara A, Igawa O, Shigemasa C, Yamamoto T: Effect
of the angiotensin II receptor antagonist losartan on uric
acid and oxypurine metabolism in healty subjects. Intern
Med 2002; 41: 793-797
10. de Jong PE, Weening JJ, Donker AJ, van der Hem GK: The
effect of phlebotomy on renal function and proteinuria in
a patient with congenital cyanotic heart disease. Nephron
1983; 33: 225-226
11. Dittrich S, Haas NA, Bührer C, Müller C, Dähnert I, Lange
PE: Renal impairment in patients with long-standing
cyanotic congenital heart disease. Acta Paediatr 1998; 87:
949-954
12. Harrison BD, Stokes TC: Secondary polycythaemia: Its
causes, effects and treatment. Br J Dis Chest 1982; 76: 313-
340
13. Erslev AJ, Caro J: Pathophysiology and classification of
polycythaemia. Scand J Haematol 1983; 31: 287-292
14. Cattran DC, Appel GB, Hebert LA, Hunsicker LG, Pohl
MA, Hoy WE, Maxwell DR, Kunis CL: A randomized
trial of cyclosporine in patients with steroid-resistant focal
segmental glomerulosclerosis. North America Nephrotic
Syndrome Study Group. Kidney Int 1999; 56: 2220-2226
15. Ambavalanan S, Fauvel JP, Sibley RK, Myers BD:
Mechanism of the antiproteinuric effect of cyclosporine in
membranous nephropathy. J Am Soc Nephrol 1996; 7: 290-
298
16. Zietse R, Wenting GJ, Kramer P, Schalekamp MA, Weimar
W: Effects of cyclosporin A on glomerular barrier function
in the nephrotic syndrome. Clin Science 1992; 82: 641-
650
17. Myers BD, Sibley R, Newton L, Tomlanovich SJ, Boshkos
C, Stinson E, Luetscher JA, Whitney DJ, Krasny D, Coplon
NS: The long-term course of cyclosporine-associated
chronic nephropathy. Kidney Int 1988; 33: 590-600

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