Difficulties in diagnosis and treatment of atypical hemolytic-uremic syndrome: clinical case
- Authors: Bakhareva Y.S.1, Chapaeva N.N.2
-
Affiliations:
- Research Institute of Therapy and Preventive Medicine – Branch Campus of the Federal Research Center Institute of Cytology and Genetics of the Siberian Branch of the Russian Academy of Sciences
- Novosibirsk State Medical University of the Ministry of Health of the Russian Federation
- Issue: Vol 16, No 2 (2024)
- Pages: 58-60
- Section: Clinical case
- URL: https://journal-vniispk.ru/2075-3594/article/view/261030
- DOI: https://doi.org/10.18565/nephrology.2024.2.58-60
- ID: 261030
Cite item
Open Access
Access granted
Subscription Access
Abstract
Background. Atypical hemolytic uremic syndrome (aHUS) is a disease with uncontrolled activation of the alternative complement pathway leading to the development of complement-mediated thrombotic microangiopathy (TMA). A typical triad of aHUS includes acute kidney injury (AKI), nonimmune hemolytic anemia, and consumptive thrombocytopenia.
Objective. Demonstration of the difficulties of diagnosing and treating aHUS.
Clinical case. Patient D., 19 years old, AKI sharply developed after a viral infection. Symptoms characteristic of aHUS included anemia, thrombocytopenia and AKI. Discussion of the diagnosis of TMA within the framework of aHUS became possible due to the results of the patient’s examination for ADAMTS-13, complete restoration of renal function during plasma therapy.
Conclusion. It is extremely important for doctors to be aware of the possibilities of timely diagnosis of aHUS; adequate pathogenetic therapy provides complete restoration of the function of the affected organs.
Full Text
##article.viewOnOriginalSite##About the authors
Yulia S. Bakhareva
Research Institute of Therapy and Preventive Medicine – Branch Campus of the Federal Research Center Institute of Cytology and Genetics of the Siberian Branch of the Russian Academy of Sciences
Author for correspondence.
Email: 8578511@inbox.ru
ORCID iD: 0000-0003-0480-1510
Junior Researcher, Laboratory of Clinical, Population and Preventive Research of Therapeutic and Endocrine Diseases
Russian Federation, NovosibirskNatalya N. Chapaeva
Novosibirsk State Medical University of the Ministry of Health of the Russian Federation
Email: n.chapaeva@mail.ru
ORCID iD: 0000-0003-0907-5044
Dr.Sci. (Med.), Professor at the Department of Hospital Therapy
Russian Federation, NovosibirskReferences
- Talarico V., Aloe M., Monzani A., et al. Hemolytic uremic syndrome in children. Minerva Pediatr. 2016;68(6): 441–55.
- Crawford B., Strebeck P., Saccente S. Constipation and hemolytic uremic syndrome. Pediatr. Nephrol. 2024;39(2):603–07. doi: 10.1007/s00467-023-06093-8.
- Viteri B., Saland J.M. Hemolytic Uremic Syndrome. Pediatr. Rev. 2020;41(4):213–15. doi: 10.1542/pir.2018-0346.
- Yerigeri K., Kadatane S., Mongan K., et al. Atypical Hemolytic-Uremic Syndrome: Genetic Basis, Clinical Manifestations, and a Multidisciplinary Approach to Management. J. Multidiscip. Healthc. 2023;16:2233–49. doi: 10.2147/JMDH.S245620.
- Leon J., LeStang M.B., Sberro-Soussan R., et al. Complement-driven hemolytic uremic syndrome. Am. J. Hematol. 2023;98(4): 44–56. doi: 10.1002/ajh.26854.
- Spasiano A., Palazzetti D., Dimartino L., et al. Underlying Genetics of aHUS: Which Connection with Outcome and Treatment Discontinuation? Int. J. Mol. Sci. 2023;24(19):14496. doi: 10.3390/ijms241914496.
- Fakhouri F., Schwotzer N., Frémeaux-Bacchi V. How I diagnose and treat atypical hemolytic uremic syndrome. Blood. 2023;141(9): 984–95. doi: 10.1182/blood.2022017860.
Supplementary files
