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

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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.

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Abbreviations

AUC:

Area under the curve

CKD:

Chronic kidney disease

eGFR:

Estimated glomerular filtration rate

IQR:

Interquartile range

eHSS:

eHUS Severity Score

eHUS:

Shigatoxin Escherichia coli-related hemolytic uremic syndrome

Hb:

Hemoglobin

LDH:

Lactate dehydrogenase

ROC:

Receiver operating characteristic

RRT:

Renal replacement therapy

STEC:

Shiga Toxin E. Coli

sCr:

Serum creatinine

TMA:

Thrombotic microangiopathy

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Acknowledgements

The following members of the ItalKid-HUS Network, to whom we are particularly thankful, gave an essential contribution to the present study: P. Accorsi (Pieve di Coriano), N. Altamura (Sesto San Giovanni), S. Andreoni (Novara), C. Baldioli (Cittiglio), B. Balduzzi (Esine), A. Bonazza (Brescia), A. Bonomini (Melzo), G. Bossi (Pavia), E. Cama (Desenzano del Garda), P. Carlucci (Milano), M. Casciana (Mantova), D. Casnaghi (Rho), R. Celano (Milano), R. Ceruti (Mantova), M. Facchini (Milano), C. Giacomazzi (Aosta), V. Goj (Milano), S. Grossi (Brescia), S. Maiandi (Lodi), ML. Melzi (Monza), E. Milanesi (Cremona), A. Monzani (Novara), A. Negri (Varese), B. Osnaghi (Magenta), F. Pagani (Brescia), L. Parola (Magenta), P. Pedroni (Manerbio), A. Pellegatta (Busto Arsizio), I. Possenti (Alessandria), A. Reciputo (Cinisello Balsamo), B. Roman (Vimercate), A. Rosco (Garbagnate), F. Salvini (Milano), S. Sardini (Asola), C. Sciuto (Lecco), M. Silvestri (Verbania), P. Tommasi (Milano), and C. Zambetti (Lodi). We would like to thank PROGETTO ALICE–ASSOCIAZIONE PER LA LOTTA ALLA SEU for their support and continuous commitment to our research.

Funding

No support/funding is present in this paper.

Author information

Authors and Affiliations

  1. Center for HUS Prevention Control and Management, Pediatric Nephrology, Dialysis and Transplantation Unit, IRCCS Foundation Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy

    Gianluigi Ardissino, Francesca Tel, Sara Testa, Fabio Paglialonga, Selena Longhi, Laura Martelli & Silvia Consolo

  2. Center for HUS Prevention Control and Management, Laboratory of Microbiology, IRCCS Foundation Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy

    Damiano Picicco, Antonella Dodaro, Laura Daprai, Rosaria Colombo & Milena Arghittu

  3. Center for HUS Prevention Control and Management, Neonatal Intensive Care Unit, IRCCS Foundation Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy

    Michela Perrone

  4. Center for HUS Prevention Control and Management, Maternal-Child Anesthesia and Intensive Care Departement, IRCCS Foundation Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy

    Giovanna Chidini & Stefano Scalia Catenacci

  5. Center for HUS Prevention Control and Management, Child and Adolescent Neuropsychiatric Unit, IRCCS Foundation Ca’ Granda, Ospedale Maggiore Policlinico, Milano, Italy

    Isabella Cropanese

  6. Center for HUS Prevention Control and Management at the Epidemiology Unit, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milano, Italy

    Dario Consonni

Authors
  1. Gianluigi Ardissino
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  2. Francesca Tel
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  3. Sara Testa
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  4. Fabio Paglialonga
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  5. Selena Longhi
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  6. Laura Martelli
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  7. Silvia Consolo
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  8. Damiano Picicco
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  9. Antonella Dodaro
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  10. Laura Daprai
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  11. Rosaria Colombo
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  12. Milena Arghittu
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  13. Michela Perrone
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  14. Giovanna Chidini
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  15. Stefano Scalia Catenacci
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  16. Isabella Cropanese
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  17. Dario Consonni
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Consortia

on behalf of the ItalKid-HUS Network

Contributions

Dr. Ardissino performed data collection, analyzed the data, drafted the initial manuscript, and revised and approved the final version.

Dr. Tel, Testa, Paglialonga, Consolo, and Chidini designed the data collection instruments, performed data collection, revised the article, and approved the final version.

Dr. Longhi, Martelli, Picicco, Dodaro, and Daprai performed data collection, edited, revised, and approved the final version.

Dr. Colombo, Arghittu, Perrone, and Scalia Catenacci performed data collection, analyzed the data, revised the article, and approved the final version.

Dr. Consonni analyzed the data and performed the statistical analysis.

Corresponding author

Correspondence to Gianluigi Ardissino.

Ethics declarations

The Ethics Committee of our hospital was informed about the study, which was conducted in accordance with our institution’s ethical regulations.

Conflicts of interest

The authors declare that they have no conflicts of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study, formal consent is not required.

Additional information

Communicated by Mario Bianchetti

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Ardissino, G., Tel, F., Testa, S. et al. A simple prognostic index for Shigatoxin-related hemolytic uremic syndrome at onset: data from the ItalKid-HUS network. Eur J Pediatr 177, 1667–1674 (2018). https://doi.org/10.1007/s00431-018-3198-7

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