Postdiarrhoeal haemolytic uraemic syndrome without thrombocytopenia

Balestracci, Alejandro; Toledo, Ismael; Battaglia, Luciana Meni; Lillo, Leonardo De; More, Natalia; Cao, Gabriel; Alvarado, Caupolicán

doi:10.1016/j.nefroe.2017.09.001

Cited by 4 publications

(4 citation statements)

References 20 publications

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“…If this definition is applied, a small number of outliers will occur, with MAHA and no thrombocytopenia, or thrombocytopenia without anemia, whom are at a much lesser risk of AKI. Similar outliers have been reported in other TMA disorders, such as hemolytic uremic syndrome 30,31 . We propose that TMA in snakebite is a spectrum disorder.…”

Section: Discussionsupporting

confidence: 86%

Schistocyte quantitation, thrombotic microangiopathy and acute kidney injury in Australian snakebite coagulopathy [ASP28]

Noutsos

Currie

Brown

2021

Int J Lab Hematology

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Introduction The major systemic manifestation of hemotoxicity in human snakebite envenoming is venom‐induced consumption coagulopathy (VICC). A subset of patients with VICC develop thrombotic microangiopathy (TMA), in which acute kidney injury (AKI) occurs. We aimed to investigate the association between schistocytosis in snakebite patients with VICC and AKI, compared to non‐envenomed patients. Methods Serial blood films collected from a prospective cohort of snakebite patients (Australian Snakebite Project) were examined. Cases were classified a priori as non‐envenomed snakebites (normal controls), envenomed without VICC, partial VICC without AKI, complete VICC without AKI, and VICC with AKI based on defined clinical and laboratory criteria. The percentage of schistocytes between groups was compared and correlated by Kendall's tau b test. Results Seven hundred and eighty blood films from 234 snakebite cases were analyzed. There was a statistically significant correlation (τ = .69, SE .03, P < .001) for schistocytosis between the ordered groups of non‐envenomed snakebites, envenomed without VICC, partial VICC without AKI, complete VICC without AKI, and VICC with AKI groups. Patients with VICC and AKI had a platelet nadir median of 42 × 109/L (interquartile range [IQR] :25‐130 × 109/L), hemoglobin nadir of median 107 g/L (IQR 66‐122 g/L), and maximum LDH median of 1128 U/L (IQR 474‐3255 U/L). A 1.0% threshold for schistocytosis yielded 90% sensitivity (95% CI: 67%‐98%) and 71% specificity (95% CI: 62%‐79%) for predicting AKI in patients with VICC. Conclusion Schistocyte quantitation has good diagnostic utility in snakebite patients with VICC. A definition of snakebite TMA as MAHA with ≥1.0% schistocytes and thrombocytopenia, would appear to be appropriate.

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Section: Discussionsupporting

confidence: 86%

Schistocyte quantitation, thrombotic microangiopathy and acute kidney injury in Australian snakebite coagulopathy [ASP28]

Noutsos

Currie

Brown

2021

Int J Lab Hematology

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“…31 In addition, the absence of this criterion may have little clinical impact because it is usually observed in patients with a mild presentation. 32,33 Some authors also propose an increase in immature platelet fraction (> 3 %) as a marker of platelet consumption; 12 this parameter was not studied in the patients included in this series. Last but not least, from an epidemiological perspective, it is also important to have a highly sensitive definition that will allow to implement timely isolation measures aimed at limiting bacterial dissemination.…”

Section: Discussionmentioning

confidence: 99%

Diagnostic sensitivity of extended renal and hematologic criteria to define hemolytic uremic syndrome

et al. 2021

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Introduction. The usual definition of Shiga toxin-producing Escherichia coli hemolytic uremic syndrome (STEC-HUS) is based on the presence of anemia, thrombocytopenia, and elevated serum creatinine levels, with or without proteinuria and/or hematuria. The strict definition only considers elevated serum creatinine levels as a renal criterion. The extended definition maintains flexible renal criteria, although it replaces anemia with hemolysis and considers a sharp drop in platelet count as an indicator of platelet consumption. The objective of this study was to estimate and compare the diagnostic sensitivity of these definitions in patients with STEC-HUS as hospital discharge diagnosis. Population and methods. Retrospective review of medical records of HUS patients. Sensitivity and positive predictive value, with their corresponding 95 % confidence intervals (CIs), were estimated for the 3 definitions based on a discharge diagnosis of STEC-HUS (reference diagnosis). The McNemar test was used. Results. Out of 208 patients, 107 (51.4 %), 133 (63.9 %), and 199 (95.6 %) were identified with the strict, usual, and extended definition, respectively. Sensitivity was lower for the strict definition (51.4 %; 95 % CI: 44.8-58.3), intermediate for the usual definition (63.9 %; 95 % CI: 56.9-70.4), and higher for the extended one (95.6 %; 95 % CI: 91.6-97.8); (p < 0.001). Conclusion.The different STEC-HUS definitions showed significant differences in diagnostic sensitivity. The extended definition reached a sensitivity above 95 %, so its generalized use may help to reduce diagnostic delays.

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“…Table 2 shows the synopsis of classic and “partial” HUS. Balestracci et al reported a 5.6% incidence of absence of thrombocytopenia in patients with post-diarrheal HUS [ 15 ]. Sellier-Leclerc et al reported that 15% of atypical HUS patients with complement mutation had normal platelet counts [ 16 ].…”

Section: Discussionmentioning

confidence: 99%

Efficacy and Safety of Eculizumab in Enteroaggregative E. coli Associated Hemolytic Uremic Syndrome

Acharya,

Clapp,

Upadhyay

2024

Pediatric Reports

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Background. Hemolytic uremic syndrome (HUS) may present atypically without the full triad of classical HUS. Eculizumab has been shown to be efficacious in complement-mediated atypical HUS and some cases of Shiga-toxin (ST) associated HUS. We report the utility of eculizumab in enteroaggregative E. coli (EAEC) associated HUS. Case summary. A female toddler presented with hemolytic anemia, oliguric acute kidney injury (AKI) without thrombocytopenia, and peripheral schistocytes. The stool examination for ST was negative but positive for EAEC. She required several hemodialysis sessions and received one dosage of eculizumab with rapid reversal of AKI and hemolytic markers. A kidney biopsy revealed acute tubular injury and segmental glomerular basement membrane splitting. Genetic testing was negative for complement mutations or deficiencies. A follow-up six months later showed persistently normal renal function and hematological markers. Conclusion. The clinical and histological manifestations of non-ST-associated diarrheal HUS and the role of eculizumab in this condition warrant future larger studies.

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Postdiarrhoeal haemolytic uraemic syndrome without thrombocytopenia

Cited by 4 publications

References 20 publications

Schistocyte quantitation, thrombotic microangiopathy and acute kidney injury in Australian snakebite coagulopathy [ASP28]

Schistocyte quantitation, thrombotic microangiopathy and acute kidney injury in Australian snakebite coagulopathy [ASP28]

Diagnostic sensitivity of extended renal and hematologic criteria to define hemolytic uremic syndrome

Efficacy and Safety of Eculizumab in Enteroaggregative E. coli Associated Hemolytic Uremic Syndrome

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