Hemolytic-uremic syndrome: from theory to practice. Clinical case

Hemolytic uremic syndrome (HUS) is a member of the group of thrombotic microangiopathies (TMA) and includes a triad of symptoms: microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Clinical manifestations of TMA are determined by the damage to those organs and systems where damage to the microcirculatory vessels by microthrombi is formed. The main diseases associated with the development of TMA are the above-mentioned HUS and thrombotic thrombocytopenic purpura (TTP). TMA can be both primary and secondary. Moreover, secondary TMA occurs in a wide range of pathologies – autoimmune and lymphoproliferative diseases, infections, oncology, and the use of certain medications. Although rare, HUS is one of the most common causes of acute kidney injury (AKI) in previously healthy children and is also one of the causes of end-stage chronic kidney disease in young adults. Patients are hospitalized in a specialized hospital depending on the prevailing clinical manifestations, therefore, it is important in the diagnosis of the above pathology to conduct a timely diagnostic search for the etiology of this disease with the involvement of related specialists and subsequent adequate treatment. A clinical case of identified hemolytic-uremic syndrome in a 19-year-old young man during military service is presented. As a result of an integrated approach to treating the disease using glucocorticosteroid and renal replacement therapy, as well as the involvement of specialized doctors, an improvement in the patient’s condition was achieved.

1. Atypical Hemolytic-Uremic Syndrome: Genetic Basis, Clinical Manifestations, and a Multidisciplinary Approach to Management / К. Yerigeri [et al.] // J. Multidiscip. Healthc. – 2023. – Vol. 16. – P. 2233–2249. doi.org/10. 2147/JMDH.S245620.

2. Kozlovskaja, N. L., Galstjan G. M., Stepanjuk V. N. Slozhnye voprosy diagnostiki atipichnogo gemolitikouremicheskogo sindroma v otdelenii reanimacii i intensivnoj terapii // Vestnik anesteziologii i reanimatologii. – 2019. – № 16(4). – S. 65–76. doi: org/10.21292/2078-5658-2019-16-4-65-76.

3. Pathogenesis of Atypical Hemolytic Uremic Syndrome / Y. Yoshida [et al.] // J. Atheroscler. Thromb. – 2019. – Vol. 26(2). – P. 99–110. doi: 10.5551/jat.RV17026.

4. Fadi, Fakhouri, Nora Schwotzer, Véronique FrémeauxBacchi. How I diagnose and treat atypical hemolytic uremic syndrome // Blood. – 2023. – Vol. 141(9). – P. 984–995. doi: org/10.1182/blood.2022017860.

5. Outbreak of haemolytic uraemic syndrome in Germany [Electronic resource] // WHO. – Available at: https://www. who.int/emergencies/disease-outbreak-news/item/2011_ 05_27-en. – Accessed: 27 May 2011.

6. Consensus opinion on diagnosis and management of thrombotic microangiopathy in Australia and New Zealand / L. C Fox [et al.] // Nephrology. – 2018. – Vol. 23. – P. 507–517. doi: org/10.1111/nep.13234.

7. Use of Highly Individualized Complement Blockade Has Revolutionized Clinical Outcomes after Kidney Transplantation and Renal Epidemiology of Atypical Hemolytic Uremic Syndrome. / J. Zuber [et al.] // J. Am. Soc. Nephrol. – 2019. – Vol. 30(12). – P. 2449–2463. doi: org/10.1681/ASN.2019040331.