A simple prognostic index for Shigatoxin-related hemolytic uremic syndrome at onset: data from the ItalKid-HUS network

on behalf of the ItalKid-HUS Network

Research output: Contribution to journalArticle

Abstract

Shigatoxin Escherichia coli-related hemolytic uremic syndrome (eHUS) is a severe thrombotic microangiopathy (TMA) burdened by life-threatening complications and long-term sequelae. Since hemoconcentration is associated with worse outcome, we tried to develop a reliable and easy-to-calculate index for predicting complications and sequelae based on hemoglobin (Hb) at presentation. The first laboratory examinations with signs of TMA in eHUS patients were analyzed in relation to the outcomes with the receiver operating characteristic curves and their areas under the curve (AUC) for Hb and creatinine (sCr). A total of 197 eHUS patients were identified of whom 24% did not have anemia at presentation. Hb level was the best predictor of a poor outcome (AUC 0.67) but the combination of Hb with sCr, in the formula [(Hb in g/dL + (sCr in mg/dL × 2)], showed an even better AUC of 0.75. The described scoring system was also strongly associated and predictive of all complications and health care needs (8% of patients with scoring > 13 died or entered a permanent vegetative state compared with 0% of those with ≤ 13). Conclusion: The presented score is a simple and early predictor of both short- and long-term outcomes and identifies patients who should undergo rapid volume expansion to counteract hemoconcentration, the spreading of microvascular thrombosis, and the consequent increased organ damage.What is Known:• In eHUS, hemoconcentration is associated with worse short- and long-term outcome.• A prognostic index to identify patients at higher risk for complications at presentation is not available.What is New:• We developed a simple and early prognostic index for eHUS outcome with the combination of Hb and sCr at onset, in the following formula [(Hb in g/dL + (sCr in mg/dL × 2)].• The proposed HUS Severity Score can promptly identify patients with good outcome and those with high risk of worse short- and long-term outcome.

Original languageEnglish
Pages (from-to)1667-1674
JournalEuropean Journal of Pediatrics
Volume177
Issue number11
DOIs
Publication statusPublished - 2018

Fingerprint

Hemolytic-Uremic Syndrome
Hemoglobins
Escherichia coli
Thrombotic Microangiopathies
Area Under Curve
Persistent Vegetative State
ROC Curve
Anemia
Creatinine
Thrombosis
Delivery of Health Care

Keywords

  • Hemolytic uremic syndrome
  • Prognostic index
  • Shigatoxin Escherichia coli infection
  • Thrombotic microangiopathy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

A simple prognostic index for Shigatoxin-related hemolytic uremic syndrome at onset : data from the ItalKid-HUS network. / on behalf of the ItalKid-HUS Network.

In: European Journal of Pediatrics, Vol. 177, No. 11, 2018, p. 1667-1674.

Research output: Contribution to journalArticle

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abstract = "Shigatoxin Escherichia coli-related hemolytic uremic syndrome (eHUS) is a severe thrombotic microangiopathy (TMA) burdened by life-threatening complications and long-term sequelae. Since hemoconcentration is associated with worse outcome, we tried to develop a reliable and easy-to-calculate index for predicting complications and sequelae based on hemoglobin (Hb) at presentation. The first laboratory examinations with signs of TMA in eHUS patients were analyzed in relation to the outcomes with the receiver operating characteristic curves and their areas under the curve (AUC) for Hb and creatinine (sCr). A total of 197 eHUS patients were identified of whom 24{\%} did not have anemia at presentation. Hb level was the best predictor of a poor outcome (AUC 0.67) but the combination of Hb with sCr, in the formula [(Hb in g/dL + (sCr in mg/dL × 2)], showed an even better AUC of 0.75. The described scoring system was also strongly associated and predictive of all complications and health care needs (8{\%} of patients with scoring > 13 died or entered a permanent vegetative state compared with 0{\%} of those with ≤ 13). Conclusion: The presented score is a simple and early predictor of both short- and long-term outcomes and identifies patients who should undergo rapid volume expansion to counteract hemoconcentration, the spreading of microvascular thrombosis, and the consequent increased organ damage.What is Known:• In eHUS, hemoconcentration is associated with worse short- and long-term outcome.• A prognostic index to identify patients at higher risk for complications at presentation is not available.What is New:• We developed a simple and early prognostic index for eHUS outcome with the combination of Hb and sCr at onset, in the following formula [(Hb in g/dL + (sCr in mg/dL × 2)].• The proposed HUS Severity Score can promptly identify patients with good outcome and those with high risk of worse short- and long-term outcome.",
keywords = "Hemolytic uremic syndrome, Prognostic index, Shigatoxin Escherichia coli infection, Thrombotic microangiopathy",
author = "{on behalf of the ItalKid-HUS Network} and Gianluigi Ardissino and Francesca Tel and Sara Testa and Fabio Paglialonga and Selena Longhi and Laura Martelli and Silvia Consolo and Damiano Picicco and Antonella Dodaro and Laura Daprai and Rosaria Colombo and Milena Arghittu and Michela Perrone and Giovanna Chidini and Catenacci, {Stefano Scalia} and Isabella Cropanese and Dario Consonni",
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AU - Tel, Francesca

AU - Testa, Sara

AU - Paglialonga, Fabio

AU - Longhi, Selena

AU - Martelli, Laura

AU - Consolo, Silvia

AU - Picicco, Damiano

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AU - Daprai, Laura

AU - Colombo, Rosaria

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