Flores SHS, Cruz AD, Frías MM

Language: Spanish
References: 7
Page: 36-39
PDF size: 258.96 Kb.

ABSTRACT

The atypical Hemolytic Uremic Syndrome (aHUS) is a rare variant of a TMA caused by an abnormality in alternative complement pathway, resulting in an endothelial dysfunction producing thrombus formation at the level of the microvasculature. It is considered as an atypical HUS since it is not caused by any of the common etiologies of classic HUS (shyga toxin produced by E. coli O157:H7 or S. dysenteriae). The genetic alterations or acquired dysregulations of the alternative complement pathway result in 40%-60% of aHUS causes, suggesting a high genetic predisposition.

REFERENCES

1. Raina R, Krishnappa V, et al. Atypical Hemolytic-UremicSyndrome: An Update on Pathophysiology,Diagnosis, and Treatment. Ther Apher Dial. 2019Feb; 23(1): 4-21.

2. Kato H, Nangaku M, Hataya H, et al. Joint Committeefor the Revision of Clinical Guides of AtypicalHemolytic Uremic Syndrome in Japan. Clinical guidesfor atypical hemolytic uremic syndrome in Japan.Clin Exp Nephrol. 2016 Aug; 20(4): 536-543.

3. Campistol JM. Actualización en síndrome hemolíticourémico atípico: diagnóstico y tratamiento. Documentode consenso. SEN. 2015; 35(5): 421-447.

4. Fremeaux-Bacchi V, Fakhouri F, Bienaimé F, Dragon-Durey MA, Ngo S, Moulin B, et al. Genetics andoutcome of atypical hemolytic uremic syndrome: anationwide French series comparing children andadults. Clin J Am Soc Nephrol. 2013 Apr; 8(4): 554-62.

5. Noris M, Bresin E, Mele C, Remuzzi G. GeneticAtypical Hemolytic-Uremic Syndrome. 2007 Nov16 [updated 2021 Sep 23]. In: Adam MP, EvermanDB, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH,Gripp KW, Amemiya A, editors. GeneReviews® [Internet].Seattle (WA): University of Washington, Seattle;1993-2023. PMID: 20301541.

6. Greenbaum LA, Fila M, Ardissino G, Al-Akash S,Evans J, Henning P, et al. Eculizumab is a safe andeffective treatment in pediatric patients with atypicalhemolytic uremic syndrome. Kidney Int. 2016Mar; 89(3): 701-11.

7. Buelna CC, Gálvez AC, Ornelas VA. Síndrome urémicohemolítico atípico. Med Int Mex. 2014; 30: 622-28.