Case
177 Diagnosis |
 |
|||
Versión
en Español |
|||
Go back to clinical information and images
Diagnosis: Hemolytic Uremic Syndrome
Hemolytic uremic syndrome (HUS) is a rare disease with high morbidity and mortality. It is characterized by the presentation of nonimmune microangiopathic hemolytic anemia, thrombocytopenia, and multi-system failure with mainly renal involvement. Two clinical entities are currently described: hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli (STEC-HUS), also known as typical HUS, and atypical HUS (aHUS), which can be associated with immunological, infectious, neoplastic, toxic, hemodynamic, gestational causes, etc. (secondary aHUS), or due to an alteration in the regulation of the alternative complement pathway (primary aHUS). In STEC-HUS, the Shiga toxin causes direct endothelial damage, with increased production of pro-inflammatory cytokines, increasing the risk of thrombosis with damage to different organs, mainly the kidneys. Additionally, this toxin activates the alternative pathway of the complement system, amplifying the inflammatory response. STEC-HUS is initially manifested by abdominal pain, vomiting, and diarrhea, and within a period of 5 to 10 days after the onset of symptoms, renal function disorder, non-immune hemolytic anemia, and thrombocytopenia occur. The degree of renal involvement varies from the presence of hematuria and proteinuria to the development of severe acute renal injury, which requires renal replacement therapy in up to 50% of patients; neurological compromise may also present in about 25% of cases, which is associated with high mortality (Nieto-Rios JF, et al. Hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli in a renal transplant recipient case report. J Bras Nefrol. 2020 Nov. Epub ahead of print. [PubMed link]).
In our case, the Shiga toxin in stool testing resulted positive and, then, STEC-HUS was finally diagnosed.
See the chapter: Vascular Diseases of our Tutorial.
Go back to clinical information and images
References
- Nieto-Rios JF, Zuluaga-Quintero M, Valencia-Maturana JC, Bello-Marquez DC, Aristizabal-Alzate A, Zuluaga-Valencia GA, Serna-Higuita LM, Arias LF. Hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli in a renal transplant recipient case report. J Bras Nefrol. 2020 Nov Epub ahead of print. [PubMed link]
- Khandelwal P, Bagga A. Guidelines on Hemolytic Uremic Syndrome by Indian Society of Pediatric Nephrology: Key Messages. Indian Pediatr. 2020 Aug 15;57(8):744-747. [PubMed link]
- Yüksel S, Işık Gönül İ, Canpolat N, Gökçe İ, Özlü SG, Özçakar ZB, Ozaltin F, Söylemezoğlu O. Renal Biopsy Prognostic Findings in Children With Atypical Hemolytic Uremic Syndrome. Pediatr Dev Pathol. 2020 Sep-Oct;23(5):362-371. [PubMed link]
- Bhandari J, Sedhai YR. Hemolytic Uremic Syndrome. 2020 May 4. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan–. PMID: 32310498. [PubMed link]
- Ylinen E, Salmenlinna S, Halkilahti J, Jahnukainen T, Korhonen L, Virkkala T, Rimhanen-Finne R, Nuutinen M, Kataja J, Arikoski P, Linkosalo L, Bai X, Matussek A, Jalanko H, Saxén H. Hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli in children: incidence, risk factors, and clinical outcome. Pediatr Nephrol. 2020 Sep;35(9):1749-1759. [PubMed link]