Hemolitik üremik sendrom tanısıyla izlenen çocuk hastaların geriye dönük değerlendirilmesi

Serra Sürmeli Döven; Esra Danacı; Ali Delibaş

doi:10.26559/mersinsbd.878072

Research Article

Hemolitik üremik sendrom tanısıyla izlenen çocuk hastaların geriye dönük değerlendirilmesi

Year 2021, Volume: 14 Issue: 3, 444 - 452, 15.12.2021

Serra Sürmeli Döven Esra Danacı Ali Delibaş

https://doi.org/10.26559/mersinsbd.878072

Cited By: 1

Abstract

Amaç: Hemolitik Üremik Sendrom (HÜS), mikroanjiyopatik hemolitik anemi, trombositopeni ve akut böbrek hasarı ile karakterizedir. Bu çalışmada, merkezimizde, Çocuk Nefroloji Polikliniği’nde HÜS tanısıyla takip edilen hastaların etiyolojik faktörleri, takip ve tedavileri açısından değerlendirilmesi amaçlanmıştır. Yöntem: Çocuk Nefrolojisi ve Çocuk Acil Polikliniği’ne Aralık 2010 ve Aralık 2020 tarihleri arasında başvuran ve HÜS tanısı alan çocuk hastalar dahil edildi. Hasta grubunun demografik ve klinik özellikleri, aldıkları tedaviler ve HÜS’e bağlı gelişen böbrek dışı tutulumlar geriye dönük olarak kayıtlardan elde edildi. Bulgular: Hastaların ortalama tanı yaşı 28.8±33.6 aydı. Hastaların 10’una (%52.6) atipik HÜS tanısı konulurken, dokuzuna STEC-HÜS tanısı konuldu (%47,4). Hastaların 16’sında (%84.2) hipertansiyon gelişti. Taze donmuş plazma infüzyonu hastaların 18’ine (18/19, %94.7), ekulizumab ise atipik HÜS tanısıyla izlenen sekiz hastaya (8/10, %80) verilirken üç hastaya plazma değişimi (3/19, %15.8) uygulandı. Renal replasman tedavisi, STEC-HÜS’lü hastaların üçüne (3/9, %33.3), atipik HÜS’lü hastaların ise tamamına uygulandı. Böbrek dışı organ tutulumu, STEC-HÜS tanısı alan hastaların sadece birinde gelişirken (1/9, %11.1), aHÜS’lü hastaların tamamında gelişti. Atipik HÜS tanısıyla takip edilen ve ekulizumab tedavisi alan hastalardan biri gastrointestinal kanama sebebiyle, diğeri ise kalp yetmezliği sebebiyle eksitus oldu. Atipik HÜS tanısıyla izlenen üç hastada (3/19, %15.8) kronik böbrek hastalığı gelişti. Sonuç: Hemolitik Üremik Sendrom, çocuklarda akut böbrek hasarının önemli bir sebebi olup HÜS’e hipertansiyon sıklıkla eşlik etmektedir. Özellikle atipik HÜS’te böbrek dışı tutulum, renal replasman tedavisi ihtiyacı ve mortalite oranı yüksektir.

Keywords

Akut böbrek hasarı, atipik hemolitik üremik sendrom, trombositopeni

References

1. Schifferli A, von Vigier RO, Fontana M, et al. Hemolytic-uremic syndrome in Switzerland: a nationwide surveillance 1997–2003. Eur J Pediatr. 2010; 169: 591–8.
2. Lynn RM, O’Brien SJ, Taylor CM, et al. Childhood hemolytic uremic syndrome, United Kingdom and Ireland. Emerg Infect Dis. 2005; 11: 590–6.
3. Rangel JM, Sparling PH, Crowe C, et al. Epidemiology of Escherichia coli O157:H7 outbreaks, United States, 1982–2002. Emerg Infect Dis. 2005; 11: 603–9.
4. Tarr PI, Neill MA, Clausen CR, et al. Escherichia coli O157:H7 and the hemolytic uremic syndrome: Importance of early cultures in establishing the etiology. J Infect Dis. 1990; 162: 553–6.).
5. Walsh PR, Johnson S. Treatment and management of children with haemolytic uraemic syndrome. Arch Dis Child. 2018; 103(3): 285-291. doi: 10.1136/archdischild-2016-311377. Epub 2017 Sep 12. PMID: 28899876.
6. Loirat C, Fakhouri F, Ariceta G et al. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2016; 31(1): 15-39. doi: 10.1007/s00467-015-3076-8. Epub 2015 Apr 11. PMID: 25859752.
7. Kavanagh D, Goodship TH, Richards A. Atypical hemolytic uremic syndrome. Semin Nephrol. 2013; 33: 508–30.
8. Waters AM, Licht C. aHUS caused by complement dysregulation: new therapies on the horizon. Pediatr Nephrol. 2011; 26: 41–57.
9. Gruppo RA, Rother RP. Eculizumab for congenital atypical hemolytic-uremic syndrome. N Engl J Med. 2009; 360: 544–6.
10. Nürnberger J, Philipp T, Witzke O, et al. Eculizumab for atypical hemolytic-uremic syndrome. N Engl J Med. 2009; 360: 542–4.
11. Licht C, Greenbaum LA, Muus P, et al. Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies. Kidney Int. 2015; 87: 1061–73.
12. Ståhl AL, Vaziri-Sani F, Heinen S, et al. Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation. Blood 2008; 111: 5307–5315.
13. Cavero T, Arjona E, Soto K et al. Severe and malignant hypertension are common in primary atypical hemolytic uremic syndrome. Kidney Int. 2019; 96(4): 995-1004. doi: 10.1016/j.kint.2019.05.014. Epub 2019 May 31. PMID: 31420192.
14. Karoui KE, Boudhabhay I, Petitprez F et al. Impact of hypertensive emergency and rare complement variants on the presentation and outcome of atypical hemolytic uremic syndrome. Haematologica. 2019; 104(12): 2501–2511.
15. Ariceta G, Besbas N, Johnson S et al. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol. 2009;2 4(4): 687-96. doi: 10.1007/s00467-008-0964-1. Epub 2008 Sep 18. PMID: 18800230.
16. Johnson S, Stojanovic J, Ariceta G, et al. An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome. Pediatr Nephrol 2014; 29:1967–1978
17. Legendre C, Greenbaum L, Sheerin N et al. Eculizumab efficacy in aHUS patients with progressing TMA, with or without prior renal transplant [Abstract]. Am J Transplant. 2013; 13:278–279
18. Olson SR, Lu E, Sulpizio E et al. When to Stop Eculizumab in Complement-Mediated Thrombotic Microangiopathies. Am J Nephrol. 2018; 48(2): 96-107. doi: 10.1159/000492033. Epub 2018 Aug 15. PMID: 30110670.
19. Wijnsma KL, Duineveld C, Wetzels JFM, van de Kar NCAJ. Eculizumab in atypical hemolytic uremic syndrome: strategies toward restrictive use. Pediatr Nephrol. 2019; 34(11): 2261-2277. doi: 10.1007/s00467-018-4091-3. Epub 2018 Nov 6. Erratum in: Pediatr Nephrol. 2019 Jan 15;: PMID: 30402748; PMCID: PMC6794245.
20. Conkar S, Mir S, Berdeli A. Genetics and consequences of atypic hemolytic uremic syndrome in Turkish children. IJKD. 2019; 13(5): 316-321.
21. Hofer J, Rosales A, Fischer C, Giner T. Extra renal manifestations of complement mediated thrombotic microangiopathies. Front Pediatr. 2014; 2:97.
22. Fremeaux-Bacchi V, Fakhouri F, Garnier A et al.Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol. 8 :554–562
23. Fidan K, Göknar N, Gülhan B et al. Extra-Renal manifestations of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2018; 33(8): 1395-1403. doi: 10.1007/s00467-018-3933-3. Epub 2018 Apr 2. PMID: 29610995.

Evaluatıon of the pedıatrıc patıents wıth hemolytıc uremıc syndrome retrospectıvely

Year 2021, Volume: 14 Issue: 3, 444 - 452, 15.12.2021

Serra Sürmeli Döven Esra Danacı Ali Delibaş

https://doi.org/10.26559/mersinsbd.878072

Cited By: 1

Abstract

Aim: Hemolytic Uremic Syndrome (HUS) is characterized as microangiopathic hemolytic anemia, trombocytopenia and acute kidney injury. This study aimed to evaluate etiological factors, follow-up and treatment results of the patients with HUS who were followed-up at Pediatric Nephrology Department of our center. Method: Nineteen patients who were admitted to Department of Pediatric Emergency and Pediatric Nephrology Units between December 2010 and December 2020 with HUS diagnosis were included in the study. Demographic and clinical features, treatments and extra-renal involvement of the patients were collected retrospectively from the records. Results: Mean age of the patients was 28.8±33.6 months. Atypic and STEC-HUS was diagnosed in 10 (52.6%) and nine (47.4%) of the patients, respectively. Hypertension developed in 16 (84.2%) of the patients. Fresh frozen plasma infusions were given to 18 (94.7%) patients. Eculizumab were given to three patients with atypic HUS (8/10, 80%). Plasma exchange were applied to three patients (3/19, 15.8%). Renal replecament therapy was applied to three of the patients with STEC-HUS (3/9, 33.3%) and to all of the patients with atypic HUS. Extra-renal involvement was observed in one patient with STEC-HUS (1/19, 11.1%) and in all patients with atypic HUS. Two patients with atypic HUS died due to gastrointestinal bleeding and heart failure, respectively. Chronic kidney disease was developed in three patients with atypic HUS. Conclusion: Hemolytic uremic syndrome is an important etiology for acute kidney injury in children. Hypertension is commonly accompanied with HUS. The rate of extra-renal involvement, requirement of renal replacement therapy and mortality were high in patients with atypic HUS.

Keywords

Acute kidney disease, atypic hemolytic uremic syndrome, thrombocytopenia

References

1. Schifferli A, von Vigier RO, Fontana M, et al. Hemolytic-uremic syndrome in Switzerland: a nationwide surveillance 1997–2003. Eur J Pediatr. 2010; 169: 591–8.
2. Lynn RM, O’Brien SJ, Taylor CM, et al. Childhood hemolytic uremic syndrome, United Kingdom and Ireland. Emerg Infect Dis. 2005; 11: 590–6.
3. Rangel JM, Sparling PH, Crowe C, et al. Epidemiology of Escherichia coli O157:H7 outbreaks, United States, 1982–2002. Emerg Infect Dis. 2005; 11: 603–9.
4. Tarr PI, Neill MA, Clausen CR, et al. Escherichia coli O157:H7 and the hemolytic uremic syndrome: Importance of early cultures in establishing the etiology. J Infect Dis. 1990; 162: 553–6.).
5. Walsh PR, Johnson S. Treatment and management of children with haemolytic uraemic syndrome. Arch Dis Child. 2018; 103(3): 285-291. doi: 10.1136/archdischild-2016-311377. Epub 2017 Sep 12. PMID: 28899876.
6. Loirat C, Fakhouri F, Ariceta G et al. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2016; 31(1): 15-39. doi: 10.1007/s00467-015-3076-8. Epub 2015 Apr 11. PMID: 25859752.
7. Kavanagh D, Goodship TH, Richards A. Atypical hemolytic uremic syndrome. Semin Nephrol. 2013; 33: 508–30.
8. Waters AM, Licht C. aHUS caused by complement dysregulation: new therapies on the horizon. Pediatr Nephrol. 2011; 26: 41–57.
9. Gruppo RA, Rother RP. Eculizumab for congenital atypical hemolytic-uremic syndrome. N Engl J Med. 2009; 360: 544–6.
10. Nürnberger J, Philipp T, Witzke O, et al. Eculizumab for atypical hemolytic-uremic syndrome. N Engl J Med. 2009; 360: 542–4.
11. Licht C, Greenbaum LA, Muus P, et al. Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies. Kidney Int. 2015; 87: 1061–73.
12. Ståhl AL, Vaziri-Sani F, Heinen S, et al. Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation. Blood 2008; 111: 5307–5315.
13. Cavero T, Arjona E, Soto K et al. Severe and malignant hypertension are common in primary atypical hemolytic uremic syndrome. Kidney Int. 2019; 96(4): 995-1004. doi: 10.1016/j.kint.2019.05.014. Epub 2019 May 31. PMID: 31420192.
14. Karoui KE, Boudhabhay I, Petitprez F et al. Impact of hypertensive emergency and rare complement variants on the presentation and outcome of atypical hemolytic uremic syndrome. Haematologica. 2019; 104(12): 2501–2511.
15. Ariceta G, Besbas N, Johnson S et al. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol. 2009;2 4(4): 687-96. doi: 10.1007/s00467-008-0964-1. Epub 2008 Sep 18. PMID: 18800230.
16. Johnson S, Stojanovic J, Ariceta G, et al. An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome. Pediatr Nephrol 2014; 29:1967–1978
17. Legendre C, Greenbaum L, Sheerin N et al. Eculizumab efficacy in aHUS patients with progressing TMA, with or without prior renal transplant [Abstract]. Am J Transplant. 2013; 13:278–279
18. Olson SR, Lu E, Sulpizio E et al. When to Stop Eculizumab in Complement-Mediated Thrombotic Microangiopathies. Am J Nephrol. 2018; 48(2): 96-107. doi: 10.1159/000492033. Epub 2018 Aug 15. PMID: 30110670.
19. Wijnsma KL, Duineveld C, Wetzels JFM, van de Kar NCAJ. Eculizumab in atypical hemolytic uremic syndrome: strategies toward restrictive use. Pediatr Nephrol. 2019; 34(11): 2261-2277. doi: 10.1007/s00467-018-4091-3. Epub 2018 Nov 6. Erratum in: Pediatr Nephrol. 2019 Jan 15;: PMID: 30402748; PMCID: PMC6794245.
20. Conkar S, Mir S, Berdeli A. Genetics and consequences of atypic hemolytic uremic syndrome in Turkish children. IJKD. 2019; 13(5): 316-321.
21. Hofer J, Rosales A, Fischer C, Giner T. Extra renal manifestations of complement mediated thrombotic microangiopathies. Front Pediatr. 2014; 2:97.
22. Fremeaux-Bacchi V, Fakhouri F, Garnier A et al.Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol. 8 :554–562
23. Fidan K, Göknar N, Gülhan B et al. Extra-Renal manifestations of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2018; 33(8): 1395-1403. doi: 10.1007/s00467-018-3933-3. Epub 2018 Apr 2. PMID: 29610995.

There are 23 citations in total.

Details

Primary Language	Turkish
Subjects	Health Care Administration
Journal Section	Articles
Authors	Serra Sürmeli Döven 0000-0001-9109-859X Esra Danacı This is me 0000-0001-9468-1131 Ali Delibaş 0000-0002-1469-9276
Publication Date	December 15, 2021
Submission Date	February 10, 2021
Acceptance Date	May 27, 2021
Published in Issue	Year 2021 Volume: 14 Issue: 3

Cite

APA	Sürmeli Döven, S., Danacı, E., & Delibaş, A. (2021). Hemolitik üremik sendrom tanısıyla izlenen çocuk hastaların geriye dönük değerlendirilmesi. Mersin Üniversitesi Sağlık Bilimleri Dergisi, 14(3), 444-452. https://doi.org/10.26559/mersinsbd.878072
AMA	Sürmeli Döven S, Danacı E, Delibaş A. Hemolitik üremik sendrom tanısıyla izlenen çocuk hastaların geriye dönük değerlendirilmesi. Mersin Univ Saglık Bilim derg. December 2021;14(3):444-452. doi:10.26559/mersinsbd.878072
Chicago	Sürmeli Döven, Serra, Esra Danacı, and Ali Delibaş. “Hemolitik üremik Sendrom tanısıyla Izlenen çocuk hastaların Geriye dönük değerlendirilmesi”. Mersin Üniversitesi Sağlık Bilimleri Dergisi 14, no. 3 (December 2021): 444-52. https://doi.org/10.26559/mersinsbd.878072.
EndNote	Sürmeli Döven S, Danacı E, Delibaş A (December 1, 2021) Hemolitik üremik sendrom tanısıyla izlenen çocuk hastaların geriye dönük değerlendirilmesi. Mersin Üniversitesi Sağlık Bilimleri Dergisi 14 3 444–452.
IEEE	S. Sürmeli Döven, E. Danacı, and A. Delibaş, “Hemolitik üremik sendrom tanısıyla izlenen çocuk hastaların geriye dönük değerlendirilmesi”, Mersin Univ Saglık Bilim derg, vol. 14, no. 3, pp. 444–452, 2021, doi: 10.26559/mersinsbd.878072.
ISNAD	Sürmeli Döven, Serra et al. “Hemolitik üremik Sendrom tanısıyla Izlenen çocuk hastaların Geriye dönük değerlendirilmesi”. Mersin Üniversitesi Sağlık Bilimleri Dergisi 14/3 (December 2021), 444-452. https://doi.org/10.26559/mersinsbd.878072.
JAMA	Sürmeli Döven S, Danacı E, Delibaş A. Hemolitik üremik sendrom tanısıyla izlenen çocuk hastaların geriye dönük değerlendirilmesi. Mersin Univ Saglık Bilim derg. 2021;14:444–452.
MLA	Sürmeli Döven, Serra et al. “Hemolitik üremik Sendrom tanısıyla Izlenen çocuk hastaların Geriye dönük değerlendirilmesi”. Mersin Üniversitesi Sağlık Bilimleri Dergisi, vol. 14, no. 3, 2021, pp. 444-52, doi:10.26559/mersinsbd.878072.
Vancouver	Sürmeli Döven S, Danacı E, Delibaş A. Hemolitik üremik sendrom tanısıyla izlenen çocuk hastaların geriye dönük değerlendirilmesi. Mersin Univ Saglık Bilim derg. 2021;14(3):444-52.

Cited By

Çocuk Yoğun Bakımda Hemolitik Üremik Sendrom Nedeniyle İzlenen Hastaların Değerlendirilmesi

Journal of Contemporary Medicine

https://doi.org/10.16899/jcm.1178547

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