Etiological diagnosis of post-diarrheal hemolytic uremic syndrome (HUS): humoral response contribution

• Published in: Pediatric nephrology (Berlin, West) West), 2023-03, Vol.38 (3), p.739-748
• Main Authors: Fiorentino, Gabriela A., Miliwebsky, Elizabeth, Ramos, María Victoria, Zolezzi, Gisela, Chinen, Isabel, Guzmán, Glenda, Nocera, Rubén, Fernández-Brando, Romina, Santiago, Adriana, Exeni, Ramón, Palermo, Marina S.
• Format: Article
• Language: English
• Subjects: Analysis; Antibodies, Bacterial; Atypical Hemolytic Uremic Syndrome; Care and treatment; Child; Diagnosis; Diagnosis, Differential; Diarrhea; Diarrhea - diagnosis; Diarrhea - epidemiology; Diarrhea - etiology; Differential diagnosis; Distribution; Escherichia coli Infections - complications; Escherichia coli Infections - diagnosis; Escherichia coli Infections - epidemiology; Etiology; Genotype; Genotyping; Hemolytic uremic syndrome; Humans; Immune response (humoral); Lipopolysaccharides; Medical diagnosis; Medicine; Medicine & Public Health; Methods; Nephrology; Original Article; Patients; Pediatrics; Serogroup; Serology; Shiga toxin; Shiga-Toxigenic Escherichia coli; Thrombocytopenia; Thrombosis; Urology
• ISSN: 0931-041X; 1432-198X; 1432-198X
• DOI: 10.1007/s00467-022-05671-6

Background Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolysis, thrombocytopenia, and thrombus formation leading to tissue injury. HUS is classified according to its etiology as post-diarrheal or atypical HUS. Differential diagnosis of both entities continues to be a challenge for pediatric physicians. Methods The aim was to improve the rapid etiological diagnosis of post-diarrheal HUS cases based on the detection of Shiga toxin (Stx)-producing Escherichia coli (STEC) infection by screening of stx 1/ stx 2 and rfb O157 in cultured stools by multiplex PCR, and the additional detection of anti-lipopolysaccharide (anti-LPS) O157, O145, and O121 antibodies by Glyco-iELISA test. In addition, we studied patients’ relatives to detect circulating pathogenic strains that could contribute to HUS diagnosis and/or lead to the implementation of measures to prevent dissemination of familial outbreaks. This study describes the diagnosis of 31 HUS patients admitted to Hospital Municipal de Niños Prof Dr Ramón Exeni during the 2017–2020 period. Results Stool PCR confirmed the diagnosis of STEC associated with HUS in 38.7% of patients (12/31), while anti-LPS serology did in 88.9% (24/27). In those patients in which both methods were carried out ( n  = 27), a strong association between the results obtained was found. We found that 30.4% of HUS patients had at least one relative positive for STEC. Conclusions We could identify 96.3% (26/27) of HUS cases as secondary to STEC infections when both methods (genotyping and serology) were used. The results demonstrated a high circulation of STEC in HUS families and the prevalence of the STEC O157 serotype (83%) in our pediatric cohort. Graphical abstract A higher-resolution version of the Graphical abstract is available as Supplementary information .