Difficulties in diagnosing and treating recurrent aHUS in a modern hospital setting


DOI: https://dx.doi.org/10.18565/nephrology.2023.4.28-33

Popov S.V., Yesayan A.M., Guseynov R.G., Perepelitsa V.V., Vorobyova O.A., Sadovnikova A.V.

1) St. Petersburg St. Luke Clinical Hospital, St. Petersburg, Russia; 2) S.M. Kirov Military Medical Academy, St. Petersburg, Russia; 3) St. Petersburg State University, St. Petersburg, Russia; 4) Pavlov University, St. Petersburg, Russia; 5) OOO National Center for Clinical Morphological Diagnostics, St. Petersburg, Russia
Background. Atypical hemolytic uremic syndrome (aHUS) is a chronic systemic disease of genetic origin, which is based on uncontrolled activation of the alternative complement pathway, leading to generalized thrombus formation in the microvasculature (complement-mediated thrombotic microangiopathy - TMA). A typical triad of aHUS symptoms includes consumptive thrombocytopenia, nonimmune hemolytic anemia, and acute kidney injury. The predominant damage to the kidneys is probably associated with the increased sensitivity of the fenestrated glomerular endothelium to such damage. At the moment, the diagnosis of aHUS is one of the diagnoses of exceptions and does not have specific pathognomonic signs. It is established on the basis of the clinical picture of the disease after excluding other forms of TMA, both primary and secondary.
Objective. Demonstration of the difficulties of diagnosing and treating aHUS using a clinical example.
Description of the clinical case. In patient I., 27 years old, since 2016, proteinuria up to 1 g/day with “empty” urinary sediment and episodes of increased blood pressure were recorded, which was interpreted as chronic glomerulonephritis. Symptoms characteristic of aHUS: anemia, decreased platelet levels, and acute kidney injury of the graft developed in the patient in the early postoperative period. However, nonspecific symptoms were regarded as consequences of significant blood loss during surgery and delayed graft function. Discussion of the diagnosis of TMA became possible primarily due to the results of a morphological study of the graft tissue and was difficult due to the development of postoperative complications and the current septic condition.
Conclusion. It is extremely important for doctors to be aware of the disease and the possibilities of its diagnosis, because a timely confirmed diagnosis of aHUS and the initiation of targeted therapy can significantly improve or completely restore the function of the affected organs.
Keywords: hemolytic anemia, Eculizumab, atypical hemolytic-uremic syndrome, thrombocytopenia, kidney transplantation, thrombotic microangiopathy
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About the Autors

Sergey V. Popov – Dr.Sci. (Med.), Professor, Chief Physician of St. Petersburg St. Luke Clinical Hospital, Head of the Center for Endoscopic Urology and New Technologies, Professor at the Department of Urology, S.M. Kirov Military Medical Academy, Head of the Department of Urology, St. Petersburg Medical and Social Institute. Address: 46 Chugunnaya st., St. Petersburg, Russia, 194044. Tel.: +7 (812) 576-11-08. E-mail: doc.popov@gmail.com. ORCID ID 0000-0003-2767-7153
Ashot M. Yesayan – Dr.Sci. (Med.), Professor, Head of the Department of Nephrology and Dialysis, Faculty of Postgraduate Education, Pavlov University, Chief External Expert in Nephrology of the Northwestern Federal District of the Russian Federation. Address: 17 Lev Tolstoy st., St. Petersburg, Russia, 197022. Tel.: +7 (812) 338-69-20. E-mail: essaian.ashot@gmail.com. ORCID: 0000-0002-7202-3151
Ruslan G.Guseynov – Cand.Sci. (Med.), Deputy Chief Physician for Scientific Activities, St. Petersburg St. Luke State Clinical Hospital, Teaching Assistant at the Department of Hospital Surgery, St. Petersburg State University. Address: 46 Chugunnaya st., St. Petersburg, Russia, 194044. Tel.: +7 (812) 576-11-08. E-mail: rusfa@yandex.ru. ORCID ID 0000-0001-9935-0243
Vitaly V.Perepelitsa – Cand. Sci. (Med.), Urologist, St. Petersburg St. Luke Clinical Hospital. Address: 46 Chugunnaya st., St. Petersburg, Russia, 194044. Tel.: +7 (812) 576-11-08. E-mail: perepelitsa_vit@mail.ru. ORCID iD 0000-0002-7656-4473
Olga A. Vorobyova – Cand.Sci. (Med.), Pathologist, Head of the Department of Pathology of Native and Transplanted Kidneys, National Center for Clinical Morphological Diagnostics. Address: 32 Slavy Ave St. Petersburg, Russia 192071. Tel.: +7 (812) 679-42-50. E-mail: olvorob70@gmail.com. ORCID iD 0000-0007-5070-926Х
Anastasia V. Sadovnikova– Nephrologist, St. Petersburg St. Luke Clinical Hospital. Address: 46 Chugunnaya st., St. Petersburg, Russia, 194044 Tel.: +7 (812) 576-11-08. E-mail: stenia1407@mail.ru. ORCID ID 0009-0004-9544-7944
Roman O. Grushevsky – Nephrologist, St. Petersburg St. Luke Clinical Hospital. Address: 46 Chugunnaya st., St. Petersburg, Russia, 194044.. E-mail: lucaclinic.ru


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