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Paroksismal Nokturnal Hemoglobinüri ve Akut Böbrek Hasarı: Olgu Sunumu

Paroxysmal Nocturnal Hemoglobinuria and Acute Kidney Injury: A Case Report

Journal Name:

  • Türk Nefroloji, Diyaliz ve Transplantasyon Dergisi

Publication Year:

  • 2013
DOI: 
DOI 10.5262/tndt.2013.1001.22

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare cause of hemolytic anemia. The major outlines of clinical presentation comprises findings of hemolysis, venous thrombosis and findings of bone marrow failure. The presentation of the disease with acute kidney injury (AKI) is not frequent. Massive hemoglobinuria following a serious attack of hemolysis during the course of PNH may lead to AKI by way of causing acute tubular necrosis. Hemoglobin casts and accumulation of hemosiderin in the proximal tubule may be seen in renal biopsy. The treatment is not different from that of acute tubular necrosis. Herein, we present a case who was admitted to our clinic with AKI and diagnosed to have PNH during hospitalization.
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Abstract (Original Language): 
Paroksismal nokturnal hemoglobinüri (PNH) nadir görülen bir hemolitik anemi nedenidir. Kliniğinin ana hatlarını hemoliz bulguları, venöz trombozlar ve kemik iliği yetersizlik bulguları oluşturmaktadır. Hastalığın akut böbrek hasarı (ABH) ile prezantasyonu sık değildir. PNH seyrinde ciddi bir hemoliz atağını takiben gelişen masif hemoglobinüri akut tubuler nekroza yol açarak ABH’na neden olabilir. Böbrek biyopsisinde hemoglobin silendirleri ve proksimal tubulde hemosiderin birikimi izlenebilir. Tedavi akut tübüler nekroza yaklaşımdan farksızdır. Burada ilk olarak ABH ile kliniğimize başvuran ve yatışı sırasında PNH tanısı koyulan bir olgu sunuyoruz.
FULL TEXT (PDF): 
PDF icon arastrmx_172977_22_pp_126-128.pdf
  • 1
126-128
  • Turkish

REFERENCES

References: 

1. Brodsky RA: Narrative review: Paroxysmal nocturnal
hemoglobinuria: The physiology of complement-related hemolytic
anemia. Ann Intern Med 2008; 148: 587-595
2. Bessler M, Mason PJ, Hillmen P, Miyata T, Yamada N, Takeda J,
Luzzatto L, Kinoshito T: Paroxysmal nocturnal hemoglobinuria
(PNH) is caused by somatic mutations in the PIG-A gene. EMBO
Journal 1994; 13: 110-117
3. Hillmen P, Richards SJ: Implications of recent insights into the
pathophysiology of paroxysmal nocturnal haemoglobinuria. Br J
Haematol 2000; 108: 470-479
4. Rosse WF: Paroxysmal nocturnal hemoglobinuria as a molecular
disease. Medicine (Baltimore) 1997; 76: 63-93
5. Socié G, Mary JY, de Gramont A, Rio B, Leporrier M, Rose C,
Heudier P, Rochant H, Cahn JY, Gluckman E: Paroxysmal nocturnal
haemoglobinuria: Long-term follow-up and prognostic
6. De Latour RP, Mary JY, Salanoubat C, Terriou L, Etienne G, Mohty
M, Roth S, de Guibert S, Maury S, Cahn JY, Socié G: Paroxysmal
nocturnal hemoglobinuria: Natural history of disease subcategories.
Blood 2008; 112: 3099
7. Rubin H: Paroxysmal nocturnal hemoglobinuria with renal failure.
JAMA 1971; 215: 433-436
8. Chow KM, Lai FM, Wang AY, Chan YL, Tang NL, Li PK: Reversible
renal failure in paroxysmal nocturnal hemoglobinuria. Am J Kidney
Dis 2001; 37: E17
9. Grover DS, Atta MG, Eustace JA, Kickler TS, Fine DM: Lack of
clinical utility of urine myoglobin detection by microconcentrator
ultrafiltration in the diagnosis of rhabdomyolysis. Nephrol Dial
Transplant 2004; 19: 2634-2638
10. Parab RB: Paroxysmal nocturnal hemoglobinuria: A study of 17
cases. J Postgrad Med 1990; 36: 23-26
11. De Vriese AS, Robbrecht DL, Vanholder RC, Vogelaers DP, Lameire
NH: Rifampicin-associated acute renal failure: Pathophysiologic,
immunologic, and clinical features. Am J Kidney Dis 1998; 31:
108-115
12. Woolf DC, Jacobs P: Methyldopa, intravascular haemolysis and
renal disease: A case report. S Afr Med J 1990; 77: 213-214
13. Trang TT, Phu NH, Vinh H, Hien TT, Cuong BM, Chau TT, Mai
NT, Waller DJ, White NJ: Acute renal failure in patients with severe
falciparum malaria. Clin Infect Dis 1992; 15: 874-880
14. Onwubalili JK: Haemoglobinuric renal failure and typhoid fever.
Trans R Soc Trop Med Hyg 1988; 82: 482-484
15. Malhotra KK, Mirdehghan CM, Tandon HD: Acute renal failure
following scorpion sting. Am J Trop Med Hyg 1978; 27: 623-626
16. Zager RA: Rhabdomyolysis and myohemoglobinuric acute renal
failure. Kidney Int 1996; 49: 314-326
17. Heyman SN, Rosen S, Fuchs S, Epstein FH, Brezis M:
Myoglobinuric acute renal failure in the rat: A role for medullary
hypoperfusion, hypoxia, and tubular obstruction. J Am Soc Nephrol
1996; 7: 1066-1074
18. Holt S, Moore K: Pathogenesis of renal failure in rhabdomyolysis:
the role of myoglobin. Exp Nephrol 2000; 8: 72-76
19. Zager RA, Burkhart KM, Conrad DS, Gmur DJ: Iron, heme
oxygenase, and glutathione: Effects on myohemoglobinuric
proximal tubular injury. Kidney Int 1995; 48: 1624
20. Mooraki A, Boroumand B, Mohammad Zadeh F, Ahmed SH,
Bastani B: Acute reversible renal failure with paroxysmal nocturnal
hemoglobinuria. Clin Nephrol 1998, 50: 255-257
21. Clark DA, Butler SA, Braren V, Hartmann RC, Jenkins DE Jr: The
kidneys in paroxysmal nocturnal hemoglobinuria. Blood 1981; 57:
83-89
22. Braren V, Butler SA, Hartmann RC, Jenkins DE: Urologic
manifestations of paroxysmal nocturnal hemoglobinuria. J Urol
1975 ; 114 (3): 430-434

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