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

  • Gianluigi Ardissino
  • Francesca Tel
  • Sara Testa
  • Fabio Paglialonga
  • Selena Longhi
  • Laura Martelli
  • Silvia Consolo
  • Damiano Picicco
  • Antonella Dodaro
  • Laura Daprai
  • Rosaria Colombo
  • Milena Arghittu
  • Michela Perrone
  • Giovanna Chidini
  • Stefano Scalia Catenacci
  • Isabella Cropanese
  • Dario Consonni
  • on behalf of the ItalKid-HUS Network
Original Article

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 

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

Notes

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.

Authors’ 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.

Funding

No support/funding is present in this paper.

Compliance with ethical standards

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.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Gianluigi Ardissino
    • 1
  • Francesca Tel
    • 1
  • Sara Testa
    • 1
  • Fabio Paglialonga
    • 1
  • Selena Longhi
    • 1
  • Laura Martelli
    • 1
  • Silvia Consolo
    • 1
  • Damiano Picicco
    • 2
  • Antonella Dodaro
    • 2
  • Laura Daprai
    • 2
  • Rosaria Colombo
    • 2
  • Milena Arghittu
    • 2
  • Michela Perrone
    • 3
  • Giovanna Chidini
    • 4
  • Stefano Scalia Catenacci
    • 4
  • Isabella Cropanese
    • 5
  • Dario Consonni
    • 6
  • on behalf of the ItalKid-HUS Network
  1. 1.Center for HUS Prevention Control and Management, Pediatric Nephrology, Dialysis and Transplantation UnitIRCCS Foundation Ca’ GrandaMilanItaly
  2. 2.Center for HUS Prevention Control and Management, Laboratory of MicrobiologyIRCCS Foundation Ca’ GrandaMilanItaly
  3. 3.Center for HUS Prevention Control and Management, Neonatal Intensive Care UnitIRCCS Foundation Ca’ GrandaMilanItaly
  4. 4.Center for HUS Prevention Control and Management, Maternal-Child Anesthesia and Intensive Care DepartementIRCCS Foundation Ca’ GrandaMilanItaly
  5. 5.Center for HUS Prevention Control and Management, Child and Adolescent Neuropsychiatric UnitIRCCS Foundation Ca’ GrandaMilanoItaly
  6. 6.Center for HUS Prevention Control and Management at the Epidemiology UnitFondazione IRCCS Ca’ GrandaMilanoItaly

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