Hemolitik Üremik Sendrom; 10 Yıllık Tek Merkez Deneyimi

Yıl 2022, Cilt: 6 Sayı: 1, 32 - 39, 22.04.2022

Ayşe Seda Pınarbaşı , Sibel Yel , Neslihan Günay , Hakan Poyrazoğlu , Aynur Gencer Balaban , İsmail Dursun , Zübeyde Gündüz Ruhan Düşünsel

https://doi.org/10.46332/aemj.874209

Cited By: 1

Öz

Amaç: Hemolitik üremik sendrom (HÜS) mikroanjiopatik hemolitik anemi, trombositopeni ve akut böbrek hasarı ile karakterize bir tablodur. Şiga toksini üreten Escherchia coli ilişkili HÜS (STEC-HÜS) çocuklarda en sık nedendir. Farklı dönemlerde salgınlar şeklinde karşımıza çıkan HÜS vakalarında son yıllarda merkezimizde de artış görülmüştür ve bu yazımızda on yıllık HÜS ile takipli hastaların klinik özellikleri değerlendirilmiştir.
Araçlar ve Yöntem: 2008-2018 yılları arasında HÜS tanısı alan hastalar çalışmaya alındı. Hastaların demografik özellikleri, başvuru klinik bulguları, başvuru anında ve hastalığın akut fazında hematolojik ve biyokimyasal bulguları, kompleman değerleri, HÜS’e bağlı organ tutulumları, uygulanan tedaviler ve böbrek yerine koyma tedavileri değerlendirildi.
Bulgular: Kırk üç hasta çalışmaya alındı. Hastaların %86’sında ishal öyküsü vardı ve bu hastaların yarısında ishal kanlı idi. Nörolojik tutulum hastaların %30’unda vardı. On sekiz hastaya ekulizumab tedavisi verildi. Ekulizumab tedavisi alan hastaların proteinüri, hipertansiyon gibi böbrek hastalığının progresyonunu gösteren parametreleri daha belirgin idi. İki hastada takipte son dönem böbrek yetmezliği gelişti.
Sonuç: Akut böbrek hasarının önemli bir nedeni olan HÜS çocuk hastalarda en sık ishal ilişkili olarak karşımıza çıkmaktadır. Nörolojik tutulum en sık böbrek dışı tutulum şeklidir. Hastaların çoğunda (%70) böbrek yerine koyma tedavisine gereksinim duyulmakta ve ekulizumab tedavisini progresyonu kötü olan olgularda daha sık kullanılmaktadır. 

Anahtar Kelimeler

Hemolitik üremik sendrom, Ekulizumab, Pediatri

Destekleyen Kurum

yok

Hemolytic Uremic Syndrome; 10 Year Single Center Experience

Öz

Purpose: Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Shiga toxin producing Escherchia coli-associated HUS (STEC-HUS) is the most common cause in children. In recent years, an increase has been observed in our center in the cases of HUS, which appeared as outbreaks in different periods, and in this study, the clinical characteristics of patients with HUS were evaluated.
Materials and Methods: Patients diagnosed with HUS between 2008-2018 were included in the study. The demographic characteristics of the patients, clinical findings at admission, hematological and biochemical findings at the time of admission and in the most active period of the disease, complement values, organ involvement due to HUS, applied treatments and kidney replacement treatments were evaluated.
Results: Forty-three patients were included in the study. 86% of the patients had a history of diarrhea and half of these patients had diarrhea with blood. Neurological involvement was present in 30% of the patients. Eighteen patients were given eculizumab treatment. Parameters showing the progression of kidney disease such as proteinuria and hypertension were more prominent in patients who received eculizumab treatment. End-stage kidney disease developed in two patients during follow-up.
Conclusion: HUS, which is an important cause of acute kidney damage, is most frequently associated with diarrhea in pediatric patients. Neurological involvement is the most common extra-renal manifestation. Kidney replacement therapy is required in most patients (70%) and eculizumab therapy is used more frequently in severe cases.

Anahtar Kelimeler

Hemolytic uremic syndrome, Eculizumab, Pediatrics

Kaynakça

• 1. Bitzan M. Glomeruler Diseases. Phadke K, Goodyer P, Bitzan M, Ed. Manuel of Pediatr Nephrol. 1. ed. Newyork: Springer; 2014:215–225.
• 2. Nester CM, Andreoli S. Renal involvement in Children with HUS. Avner ED, Harmon WE, Niaudet P, Yoshikava N, Emm a F, Goldstein SL, editörler. Pediatr Nephrol. 7. ed. New York: Springer Reference; 2016:1490–1513.
• 3. Vivarelli M, Thurman JM. Complement-mediated glomerulonephritis and thrombotic microangiopathy. Bomback AS, Perazella MA, Tonelli M, editörler. Primer on Kidney Diseases. 7. ed. Philadelphia: Elsevier; 2018:208–219.
• 4. Gasser C, Gautier E, Steck A, Siebenmann R, Oechslin R. Hemolytic-uremic syndrome: bilateral necrosis of the renal cortex in acute acquired hemolytic anemia. Schweiz Med Wochenschr. 1955;85(38-39):905-909.
• 5. Corrigan JJ, Boineau FG. Hemolytic-uremic syn-drome. Pediatr Rev. 2001;22(11):365-369.
• 6. Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Eng J Med. 2009;361(17):1676-1687.
• 7. Cody EM, Dixon BP. Hemolytic uremic syndrome. Pediatr Clin North Am. 2019;66(1):235-246.
• 8. Fakhouri F, Zuber J, Frémeaux-Bacchi V, Loirat C. Haemolytic uraemic syndrome. Lancet. 2017;390 (10095):681-696.
• 9. Frank C, Werber D, Cramer JP, et al. Epidemic profile of shiga-toxin–producing. N Eng J Med. 2011;365 (19):1771-1780.
• 10. Yıldırım ZY, Yılmaz A, Yavaş Aksu B ve ark. Diyare öyküsü olan hemolitik üremik sendrom tanılı hastaların klinik özellikleri. İst Tıp Fak Derg. 2015;78(2):46-50.
• 11. Goodyer P, K P. Evaluation of renal disease. Phadke K, Goodyer P, Bitzan M, editörler. Manuel of Pediatric Nephrology. 1st ed. New York: Springer; 2014:1-64.
• 12. KDIGO 2012 Clinical Practice Guideline for the evaluation and management of chronic kidney disease. Kidney Int. 2013;3(1):1-163.
• 13. IBM Corp. IBM SPSS Statistics for Windows, Version 22.0. Armonk, New York: IBM Corp. Released 2013.
• 14. Watanabe Y, Ozasa K, Mermin JH et al. Factory outbreak of Escherichia coli O157:H7 infection in Japan. Emerg Infect Dis. 1999;5(3):424-428.
• 15. Ekinci Z, Candan C, Alpay H et al. Hemolytic uremic syndrome outbreak in Turkey in 2011. Turk J Pediatr. 2013;55(3):246-252.
• 16. Yun YS, Kim NO, Chun JH, Hwang KJ, Hong S. The prevalence and characteristics of Shiga toxin producing Escherichia coli isolated by enteric pathogens active surveillance network (Enter-Net) in the republic of Korea, 200-9-2018. Microb Pathog. 2021;158: 105005.
• 17. Canpolat N. Hemolytic uremic syndrome. Turk Arch Pediatr. 2015;50(2):73-82.
• 18. Keenswijk W, Raes A, De Clerck M, Vande Walle J. Is plasma exchange efficacious in shiga toxin-associated hemolytic uremic syndrome? A narrative review of current evidence. Ther Apher Dial. 2019;23(2):118-125.
• 19. Kielstein JT, Beutel G, Fleig S et al. Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: An analysis of the German STEC-HUS registry. Nephrol Dial Transpl. 2012;27(10):3807-3815.
• 20. Walsh PR, Johnson S. Eculizumab in the treatment of Shiga toxin haemolytic uraemic syndrome. Pediatr Nephrol. 2019;34(9):1485-1492.
• 21. Buchholz U, Bernard H, Werber D et al. German outbreak of Escherichia coli O104:H4 associated with sprouts. N Eng J Med. 2011;365(19):1763-1770.
• 22. German Society of Nephrology. "Advice of the German Society of Nephrology on the use of Eculizumab during the 2011 EHEC HUS outbreak" http://www.dgfn.eu/aktuell/ehec-informationen/fuer-das-fachpublikum/advice-on-the-useof-ecilizumab.ht ml. Erişim tarihi: 10 Haziran, 2011.
• 23. Siegler RL, Pavia AT, Christofferson RD, Milligan MK. A 20-year population-based study of post-diarrheal hemolytic uremic syndrome in Utah. Pediatrics. 1994;94(1):35-40.
• 24. Mody RK, Gu W, Griffin PM, et al. Postdiarrheal hemolytic uremic syndrome in United States children: Clinical spectrum and predictors of in-hospital death. J Pediatr. 2015;166(4):1022-1029.