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Tipik Hemolitik Üremik Sendromlu Çocuklarda Klinik Özelliklerin ve Renal Replasman Tedavisinin Değerlendirilmesi

Year 2022, Volume: 16 Issue: 2, 127 - 133, 16.03.2022
https://doi.org/10.12956/tchd.1018164

Abstract

Amaç: Tipik hemolitik üremik sendrom (HÜS) hastalarının bazılarında, agresif tıbbi müdahaleye rağmen renal replasman tedavisi (RRT) gerekebilir. Bu çalışmanın amacı, tipik HÜS tanısı alan hastaların klinik ve laboratuvar bulgularının değerlendirilmesi, HÜS tanılı hastalarda periton diyalizi (PD) ile hemodiyalizin (HD) etkinlik ve güvenilirliğinin karşılaştırılmasıdır.

Gereç ve Yöntemler: Tipik HÜS tanısı olan hastalar retrospektif olarak belirlendi ve RRT modalitelerinin renal sağ kalım üzerindeki etkisi değerlendirildi.

Bulgular: Çalışmaya tipik HÜS tanılı 49 hasta dahil edildi ve ortalama yaşları 2.99 ± 1.88 yıldı. Nörolojik tutulum 15 (%30.6) hastada saptandı. Nötrofil/lenfosit oranı nörolojik tutulum olan grupta anlamlı olarak yüksek bulundu [olasılık oranı (OO) 15.42, %95 güven aralığı (GA) (3.39-70.1), p = 0.005]. Toplamda 43 (%87.7) hastaya RRT uygulandı ve ilk kaydedilen semptomdan RRT’ye kadar geçen ortanca süre 6 gündü (aralık 3-11). RRT boyunca hastaların %10.2’sinde mekanik ventilasyon desteği gerekti. Yirmi altı (%53.1) hastada PD, 17 (%34.6) hastada HD yapılmıştı. Takipte tüm hastaların %10.2’si kronik böbrek hastalığına (KBH) ilerledi, %6.1’inde proteinüri ve %4.08’inde hipertansiyon saptandı. PD yapılan hastalarda 3 aylık dönemde KBH riski HD yapılan hastalara göre anlamlı olarak daha düşüktü [ OO 7.69, %95 GA (0.77-76.07), p = 0.04].

Sonuç: Böbrek iyileşmesi açısından PD’in HD kadar etkili hatta daha güvenli olabileceği sonucuna vardık. Tipik HÜS vakalarında klinik özellikler ve inflamatuar belirteçler sayesinde nörolojik tutulum tahmin edilebilir.

References

  • Viteri B, Saland JM. Hemolytic Uremic Syndrome. Pediatr Rev 2020;41:213-5.
  • Besbas N, Karpman D, Landau D, Loirat C, Proesmans W, Remuzzi G, et al. European Paediatric Research Group for HUS: A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders. Kidney Int 2006;70:423–31.
  • Fakhouri F, Zuber J, Fre ́meaux-Bacchi V, Loirat C. Haemolytic uraemic syndrome. Lancet 2017;390:681–96.
  • Salvadori M, Bertoni E. Update on hemolytic uremic syndrome: diagnostic and therapeutic recommendations. World J Nephrol 2013;2:56–76.
  • Walsh PR, Johnson S. Treatment and management of children with haemolytic uraemic syndrome. Arch Dis Child 2018;103:285-91.
  • Talarico V, Aloe M, Monzani A, Miniero R, Bona G. Hemolytic uremic syndrome in children. Minerva Pediatr 2016;68:441-55.
  • Grisaru S. Management of hemolytic-uremic syndrome in children. Int J Nephrol Renovasc Dis 2014;7:231-9.
  • Bitzan M. Treatment options for HUS secondary to Escherichia coli O157:H7. Kidney Int Suppl 2009;(112):S62–6.
  • Tarr PI, Gordon CA, Chandler WL. Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet 2005;365:1073–86.
  • Trachtman H, Cnaan A, Christen E, Gibbs K, Zhao S, Acheson DWK, et al. Investigators of the HUS-SYNSORB Pk Multicenter Clinical Trial. Effect of an oral Shiga toxin-binding agent on diarrhea-associated hemolytic uremic syndrome in children: a randomized controlled trial. JAMA 2003;290:1337–44.
  • Rosales A, Hofer J, Zimmerhackl LB, Jungraithmayr TC, Riedl M, Giner T, et al. Need for long-term follow-up in enterohemorrhagic Escherichia coli-associated hemolytic uremic syndrome due to late-emerging sequelae. Clin Infect Dis 2012;54:1413–21.
  • Basu B, Mahapatra TK, Roy B, Schaefer F. Efficacy and outcomes of continuous peritoneal dialysis versus daily intermittent hemodialysis in pediatric acute kidney injury. Pediatr Nephrol 2016;31:1681-9.
  • Bunchman TE. Treatment of acute kidney injury in children: from conservative management to renal replacement therapy. Nat Clin Pract Nephrol 2008;4:510–4.
  • Palevsky PM, Liu KD, Brophy PD, Chawla LS, Parikh CR, Thakar CV, et al. KDOQI US commentary on the 2012 KDIGO clinical practice guideline for acute kidney injury. Am J Kidney Dis 2013;61:649-72.
  • Schwartz GJ, Muñoz A, Schneider MF, Mak RH, Kaskel F, Warady BA, et al. New equations to estimate GFR in children with CKD. J Am Soc Nephrol 2009;20:629-37.
  • Ariceta G. Clinical practice: proteinuria. Eur J Pediatr 2011;170:15-20.
  • Lurbe E, Agabiti-Rosei E, Cruickshank JK, Dominiczak A, Erdine S, Hirth A, et al. 2016 European Society of Hypertension guidelines for the management of high blood pressure in children and adolescents. J Hypertens 2016;34:1887-920.
  • Levey AS, Coresh J, Balk E, Kausz AT, Levin A, Steffes MW, et al. National Kidney Foundation practice guidelines for chronic kidney disease: evaluation, classification, and stratification. Ann Intern Med 2003;139:137-47.
  • Micheletti M V, LavorattiG, Materassi M, Pela I. Hemolytic Uremic Syndrome: Epidemiological and Clinical Features of a Pediatric Population in Tuscany. Kidney Blood Press Res 2010;33:399-404.
  • Balgradean M, Croitoru A, Leibovitz E. An outbreak of hemolytic uremic syndrome in southern Romania during 2015-2016: Epidemiologic, clinical, laboratory, microbiologic, therapeutic and outcome characteristics. Pediatr Neonatol 2019;60:87-94.
  • Jenssen GR, Vold L, Hovland E, Bangstad HJ, Nyard K, Bjerre A. Clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study from 1999–2008. BMC Infect Dis 2016;16:285.
  • Zhao SA, Ning BT, Mao JH. Clinical characteristics of children with hemolytic uremic syndrome in Hangzhou, China. World J Pediatr 2017;13:183-5.
  • Scheiring J, Andreoli SP, Zimmerhackl LB. Treatment and outcome of Shiga-toxin associated hemolytic uremic syndrome (HUS). Pediatr Nephrol 2008;23:1749-60.
  • McKee RS, Schnadower D, Tarr PI, Xie J, Finkelstein Y, Desai N, et al. Predicting hemolytic uremic syndrome and renal replacement therapy in Shiga toxin-producing Escherichia coli-infected children. Clin Infect Dis 2020;70:1643-51.
  • Costigan C, Raftery T, Carroll AG, Wildes D, Reynolds C, Cunney R, et al. Neurological involvement in children with hemolytic uremic syndrome. Eur J Pediatr 2022;181:501-12.
  • Nathanson S, Kwon T, Elmaleh M, Charbit M, Launay EA, Harambat J, et al. Acute neurological involvement in diarrhea associated hemolytic uremic syndrome. Clin J Am Soc Nephrol 2010;5:1218-28.

Evaluation of Clinical Features and Renal Replacement Therapy in Children with Typical Hemolytic Uremic Syndrome

Year 2022, Volume: 16 Issue: 2, 127 - 133, 16.03.2022
https://doi.org/10.12956/tchd.1018164

Abstract

Objective: A subset of typical Hemolytic-uremic syndrome (HUS) patients requires initiation of renal replacement therapy (RRT) despite aggressive medical intervention. This study aims to evaluate the clinical and laboratory findings of patients diagnosed with typical HUS and efficacy and safety of peritoneal dialysis (PD) and hemodialysis (HD) were compared in managing these cases.

Material and Methods: Patients having a diagnosis of typical HUS were retrospectively determined, and assessment the effects of RRT modalities on renal survival.

Results: The present study included 49 children with typical HUS, and the mean age was 2.99 ± 1.88 years. Neurological involvement was detected in 15 (30.6%) patients. The neutrophil/lymphocyte ratio (NLR) was significantly higher in the neurological involvement group [odds ratio (OR) 15.42, 95% CI (3.39-70.1), p = 0.005]. In total, 43 (87.7%) patients received RRT and the median time from the first recorded symptom to RRT was six days (range 3-11). While throughout RRT, 10.2% of patients required mechanical ventilation. In this study, 26 (53.1%) patients were managed with PD, and 17 (34.6%) patients were managed with HD. During follow-up, 10.2% of all patients progressed to chronic kidney disease (CKD), proteinuria was detected in 6.1% and hypertension in 4.08%. The risk of CKD was significantly lower in the 3-month period in PD patients than in HD patients (odds ratio (OR) 7.69, 95% CI (0.77-76.07), p = 0.04).


Conclusion:
We concluded that PD might be as effective and safer as HD concerning kidney recovery. Clinical features and inflammatory markers can predict neurological involvement in typical HUS cases.

References

  • Viteri B, Saland JM. Hemolytic Uremic Syndrome. Pediatr Rev 2020;41:213-5.
  • Besbas N, Karpman D, Landau D, Loirat C, Proesmans W, Remuzzi G, et al. European Paediatric Research Group for HUS: A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders. Kidney Int 2006;70:423–31.
  • Fakhouri F, Zuber J, Fre ́meaux-Bacchi V, Loirat C. Haemolytic uraemic syndrome. Lancet 2017;390:681–96.
  • Salvadori M, Bertoni E. Update on hemolytic uremic syndrome: diagnostic and therapeutic recommendations. World J Nephrol 2013;2:56–76.
  • Walsh PR, Johnson S. Treatment and management of children with haemolytic uraemic syndrome. Arch Dis Child 2018;103:285-91.
  • Talarico V, Aloe M, Monzani A, Miniero R, Bona G. Hemolytic uremic syndrome in children. Minerva Pediatr 2016;68:441-55.
  • Grisaru S. Management of hemolytic-uremic syndrome in children. Int J Nephrol Renovasc Dis 2014;7:231-9.
  • Bitzan M. Treatment options for HUS secondary to Escherichia coli O157:H7. Kidney Int Suppl 2009;(112):S62–6.
  • Tarr PI, Gordon CA, Chandler WL. Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet 2005;365:1073–86.
  • Trachtman H, Cnaan A, Christen E, Gibbs K, Zhao S, Acheson DWK, et al. Investigators of the HUS-SYNSORB Pk Multicenter Clinical Trial. Effect of an oral Shiga toxin-binding agent on diarrhea-associated hemolytic uremic syndrome in children: a randomized controlled trial. JAMA 2003;290:1337–44.
  • Rosales A, Hofer J, Zimmerhackl LB, Jungraithmayr TC, Riedl M, Giner T, et al. Need for long-term follow-up in enterohemorrhagic Escherichia coli-associated hemolytic uremic syndrome due to late-emerging sequelae. Clin Infect Dis 2012;54:1413–21.
  • Basu B, Mahapatra TK, Roy B, Schaefer F. Efficacy and outcomes of continuous peritoneal dialysis versus daily intermittent hemodialysis in pediatric acute kidney injury. Pediatr Nephrol 2016;31:1681-9.
  • Bunchman TE. Treatment of acute kidney injury in children: from conservative management to renal replacement therapy. Nat Clin Pract Nephrol 2008;4:510–4.
  • Palevsky PM, Liu KD, Brophy PD, Chawla LS, Parikh CR, Thakar CV, et al. KDOQI US commentary on the 2012 KDIGO clinical practice guideline for acute kidney injury. Am J Kidney Dis 2013;61:649-72.
  • Schwartz GJ, Muñoz A, Schneider MF, Mak RH, Kaskel F, Warady BA, et al. New equations to estimate GFR in children with CKD. J Am Soc Nephrol 2009;20:629-37.
  • Ariceta G. Clinical practice: proteinuria. Eur J Pediatr 2011;170:15-20.
  • Lurbe E, Agabiti-Rosei E, Cruickshank JK, Dominiczak A, Erdine S, Hirth A, et al. 2016 European Society of Hypertension guidelines for the management of high blood pressure in children and adolescents. J Hypertens 2016;34:1887-920.
  • Levey AS, Coresh J, Balk E, Kausz AT, Levin A, Steffes MW, et al. National Kidney Foundation practice guidelines for chronic kidney disease: evaluation, classification, and stratification. Ann Intern Med 2003;139:137-47.
  • Micheletti M V, LavorattiG, Materassi M, Pela I. Hemolytic Uremic Syndrome: Epidemiological and Clinical Features of a Pediatric Population in Tuscany. Kidney Blood Press Res 2010;33:399-404.
  • Balgradean M, Croitoru A, Leibovitz E. An outbreak of hemolytic uremic syndrome in southern Romania during 2015-2016: Epidemiologic, clinical, laboratory, microbiologic, therapeutic and outcome characteristics. Pediatr Neonatol 2019;60:87-94.
  • Jenssen GR, Vold L, Hovland E, Bangstad HJ, Nyard K, Bjerre A. Clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study from 1999–2008. BMC Infect Dis 2016;16:285.
  • Zhao SA, Ning BT, Mao JH. Clinical characteristics of children with hemolytic uremic syndrome in Hangzhou, China. World J Pediatr 2017;13:183-5.
  • Scheiring J, Andreoli SP, Zimmerhackl LB. Treatment and outcome of Shiga-toxin associated hemolytic uremic syndrome (HUS). Pediatr Nephrol 2008;23:1749-60.
  • McKee RS, Schnadower D, Tarr PI, Xie J, Finkelstein Y, Desai N, et al. Predicting hemolytic uremic syndrome and renal replacement therapy in Shiga toxin-producing Escherichia coli-infected children. Clin Infect Dis 2020;70:1643-51.
  • Costigan C, Raftery T, Carroll AG, Wildes D, Reynolds C, Cunney R, et al. Neurological involvement in children with hemolytic uremic syndrome. Eur J Pediatr 2022;181:501-12.
  • Nathanson S, Kwon T, Elmaleh M, Charbit M, Launay EA, Harambat J, et al. Acute neurological involvement in diarrhea associated hemolytic uremic syndrome. Clin J Am Soc Nephrol 2010;5:1218-28.
There are 26 citations in total.

Details

Primary Language English
Subjects ​Internal Diseases
Journal Section ORIGINAL ARTICLES
Authors

Tülin Güngör 0000-0002-5881-1565

Deniz Karakaya 0000-0001-7720-4923

Evrim Kargın Çakıcı 0000-0002-1697-6206

Fatma Yazılıtaş 0000-0001-6483-8978

Mutlu Uysal Yazıcı 0000-0001-7377-4718

Evra Çelikkaya 0000-0003-2695-2045

Ebru Azapağası 0000-0002-0684-8219

Mehmet Bülbül 0000-0001-9007-9653

Publication Date March 16, 2022
Submission Date November 2, 2021
Published in Issue Year 2022 Volume: 16 Issue: 2

Cite

Vancouver Güngör T, Karakaya D, Kargın Çakıcı E, Yazılıtaş F, Uysal Yazıcı M, Çelikkaya E, Azapağası E, Bülbül M. Evaluation of Clinical Features and Renal Replacement Therapy in Children with Typical Hemolytic Uremic Syndrome. Türkiye Çocuk Hast Derg. 2022;16(2):127-33.


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