Atypical hemolytic-uremic syndrome in children
https://doi.org/10.35401/2541-9897-2022-25-4-62-67
Abstract
Background: Atypical hemolytic-uremic syndrome (aHUS) is a rare progressive form of systemic thrombotic microangiopathy (TMA), which develops due to uncontrolled activation of the alternative pathway of the complement system and is characterized by a predominant lesion of the vessels of the renal microcirculatory bed.
Clinical cases description: The article describes two clinical cases of aHUS, which demonstrate the difficulties of differential diagnosis of typical and atypical HUS at the initial stages of the disease.
Conclusion: Given the lack of clear laboratory criteria of aHUS, it is necessary to carry out a complex and consistent differential diagnosis of various TMA options, the exclusion of which makes it possible to make a correct diagnosis more accurately.
About the Authors
A. V. BurlutskayaRussian Federation
Alla V. Burlutskaya, Dr. Sci. (Med.), Head of the Pediatrics Department no. 2
4, M. Sedina str., Krasnodar, 350063
A. V. Statova
Russian Federation
Anastasia V. Statova, Cand. Sci. (Med.), Associate Professor, Pediatrics Department no. 2
4, M. Sedina str., Krasnodar, 350063
D. V. Ustyuzhaninа
Russian Federation
Diana V. Ustyuzhaninа, Resident, Pediatrics Department no. 2
4, M. Sedina str., Krasnodar, 350063
Yu. V. Pisotskaya
Russian Federation
Yulia V. Pisotskaya, Resident, Pediatrics Department no. 2
4, M. Sedina str., Krasnodar, 350063
References
1. Bayko SV. The first experience of using a biosimilar of the original drug eculizumab for prevention of atypical hemolytic-uremic syndrome recurrence after renal transplantation in a child. Pediatrics. Journal named after G.N. Speransky. 2021;100(1):293–296. (In Russ.). https://doi.org/10.24110/0031-403X-2021-100-1-293-296
2. Bayko SV. Atypical hemolytic-uremic syndrome in children: practical aspects of differential diagnosis and treatment. Pediatrics. Journal named after G.N. Speransky. 2021;100(4):64–73. (In Russ.). https://doi.org/10.24110/0031-403X-2021-100-4-64-73
3. Kozlovskaya NL, Emirova HM. Complement pathology in the practice of a nephrologist: atypical hemolytic-uremic syndrome. Collection of actual clinical observations. Moscow: Mediasphere; 2020:152. (In Russ.).
4. Kuznik BI, Sturov VG, Levshin NYu, et al. Hemorrhagic and thrombotic diseases and syndromes in children and adolescents: Pathogenesis, clinic, diagnosis, therapy and prevention. Novosibirsk: Nauka; 2018:524. (In Russ.).
5. Yakubov A.V., Sologub G.N. Komlichenko E.V., et al. Clinical case of treatment of patient with aHUS in obstetrical practice. Experience of anesthesiology and resuscitation department. Annals of critical care. 2018;2:87–94. (In Russ.). https://doi.org/10.21320/1818-474X-2018-2-87-94
6. Avila Bernabeu AI, Cavero Escribano T, Cao Vilarino M. Atypical hemolytic uremic syndrome: new challenges in the complement blockage era. Nephron. 2020;144(11):537–549. PMID: 32950988. https://doi.org/10.1159/000508920
7. Ariceta G. Hemolytic Uremic Syndrome. Curr Treat Options Peds. 2020;6:252–262. https://doi.org/10.1007/s40746-020-00216-1
8. Kaabak MM, Molchanova EA, Nesterenko IV, et al. Interdisciplinary Expert Council Resolution. Kidney transplantation in patients with atypical hemolytic-uremic syndrome: clinical and organizational-methodical aspects of patient management. Clinical nephrology. 2018;2:21–27. (In Russ.). https://doi.org/10.18565/nephrology.2018.3.8-14
9. Feitz WJC, van de Kar NCAJ, Orth-Höller D, et al. The genetics of atypical hemolytic uremic syndrome. Med Genet. 2018;30(4):400–409. PMID: 30930551. PMCID: PMC6404389. https://doi.org/10.1007/s11825-018-0216-0
10. Yeryushova TYu, Aminova AI, Lakhova SA, et al. Challenges in Diagnostics and Management of Atypical Hemolytic Uremic Syndrome Associated with Acute Intestinal Infection in Adolescent: Clinical Case. Current pediatrics. 2019;18(5):354–361. (In Russ.). https://doi.org/10.15690/vsp.v18i5.2059
11. Kozlovskaya N.L. Atypical hemolytic-uremic syndrome: current understanding of the pathogenesis, clinic, approaches to diagnosis and treatment. Thrombosis, hemostasis and rheology. 2019;80(4):13–20. (In Russ.). https://doi.org/10.25555/THR.2019.4.0895
Review
For citations:
Burlutskaya A.V., Statova A.V., Ustyuzhaninа D.V., Pisotskaya Yu.V. Atypical hemolytic-uremic syndrome in children. Innovative Medicine of Kuban. 2022;(4):62-67. (In Russ.) https://doi.org/10.35401/2541-9897-2022-25-4-62-67