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01-11-2018 | Original Article

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

Authors: 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

Published in: European Journal of Pediatrics | Issue 11/2018

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.

Ardissino G, Possenti I, Tel F, Testa S, Paglialonga F (2014) Time to change the definition of hemolytic uremic syndrome. Eur J Intern Med 25:e29. https://doi.org/10.1016/j.ejim.2013.12.002 CrossRefPubMed

Ardissino G, Daccò V, Testa S, Civitillo CF, Tel F, Possenti I, Belingheri M, Castorina P, Bolsa-Ghiringhelli N, Tedeschi S, Paglialonga F, Salardi S, Consonni D, Zoia E, Salice P, Chidini G (2015) Hemoconcentration: a major risk factor for neurological involvement in hemolytic uremic syndrome. Pediatr Nephrol 30:345–352. https://doi.org/10.1007/s00467-014-2918-0 CrossRefPubMed

Ardissino G, Salardi S, Colombo E, Testa S, Borsa-Ghiringhelli N, Paglialonga F, Paracchini V, Tel F, Possenti I, Belingheri M, Civitillo CF, Sardini S, Ceruti R, Baldioli C, Tommasi P, Parola L, Russo F, Tedeschi S (2016) Epidemiology of haemolytic uremic syndrome in children. Data from the north Italian HUS network. Eur J Pediatr 175:465–473. https://doi.org/10.1007/s00431-015-2642-1 CrossRefPubMed

Ardissino G, Tel F, Possenti I, Testa S, Consonni D, Paglialonga F, Salardi S, Borsa-Ghiringhelli N, Salice P, Tedeschi S, Castorina P, Colombo RM, Arghittu M, Daprai L, Monzani A, Tozzoli R, Brigotti M, Torresani E (2016) Early volume expansion and outcomes of hemolytic uremic syndrome. Pediatrics 137:1–9. https://doi.org/10.1542/peds.2015-2153. CrossRef

Ardissino G, Tel F, Picicco D, Dodaro A, Daprai L, Consolo S, Testa S, Paglialonga F, Consonni D, ItalKid HUS Network (2018) Comment on “blood urea nitrogen to serum creatinine ratio is an accurate predictor of outcome in diarrhea-associated hemolytic uremic syndrome” by Keenswijk et al. Eur J Pediatr 2017;176(3): 355-360. Eur J Pediatr 177:269–270. https://doi.org/10.1007/s00431-017-2989-6 CrossRefPubMed

Balestracci A, Martin SM, Toledo I, Alvarado C, Wainsztein RE (2012) Dehydration at admission increased the need for dialysis in hemolytic uremic syndrome children. Pediatr Nephrol 27:1407–1410. https://doi.org/10.1007/s00467-012-2158-0 CrossRefPubMed

Garg AX, Suri RS, Barrowman N, Rehman F, Matsell D, Rosas-Arellano MP, Salvadori M, Haynes RB, Clarck WF (2003) Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: a systematic review, meta-analysis, and meta-regression. JAMA 290:1360–1370. https://doi.org/10.1001/jama.290.10.1360 CrossRefPubMed

Keenswijk W, Vanmassenhove J, Raes A, Dhont E, Vande Walle J (2017) Blood urea nitrogen to serum creatinine ratio is an accurate predictor of outcome in diarrhea-associated hemolytic uremic syndrome, a preliminary study. Eur J Pediatr 176:355–360. https://doi.org/10.1007/s00431-016-2846-z CrossRefPubMed

Loos S, Ahlenstiel T, Kranz B, Staude H, Pape L, Härtel C, Vester U, Buchtala L, Benz K, Hoppe B, Beringer O, Krause M, Müller D, Pohl M, Lemke J, Hillebrand G, Kreuzer M, König J, Wigger M, Konrad M, Haffner D, Oh J, Kemper MJ (2012) An outbreak of Shiga toxin–producing Escherichia coli O104:H4 hemolytic uremic syndrome in Germany: presentation and short-term outcome in children. Clin Infect Dis 55:753–759. https://doi.org/10.1093/cid/cis531 CrossRefPubMed

10.

Mody RK, Gu W, Griffin PM, Jones TF, Rounds J, Shiferaw B, Tobin-D’Angelo M, Smith G, Spina N, Hurd S, Lathrop S, Palmer A, Boothe E, Luna-Gierke RE, Hoekstra RM (2015) Postdiarrheal hemolytic uremic syndrome in United States children: clinical spectrum and predictors of in-hospital death. J Pediatr 166:1022102–1022109. https://doi.org/10.1016/j.jpeds.2014.12.064 CrossRef

11.

Oakes RS, Siegler RL, McReynolds MA, Pysher T, Pavia AT (2006) Predictors of fatality in postdiarrheal hemolytic uremic syndrome. Pediatrics 117:1656–1662. https://doi.org/10.1542/peds.2005-0785 CrossRefPubMed

12.

Oakes RS, Kirkham JK, Nelson RD, Siegler RL (2008) Duration of oliguria and anuria predictors of chronic renal-related sequelae in post-diarrheal hemolytic uremic syndrome. Pediatr Nephrol 23:1303–1308. https://doi.org/10.1007/s00467-008-0799-9 CrossRefPubMed

13.

Ojeda JM, Kohout I, Cuestas E (2013) Dehydration upon admission is a risk factor for incomplete recovery of renal function in children with haemolytic uremic syndrome. Nefrologia 33:372–376. https://doi.org/10.3265/Nefrologia.pre2012.Nov.11648. CrossRefPubMed

14.

Scheiring J, Andreoli SP, Zimmerhackl LB (2008) Treatment and outcome of Shiga-toxin-associated hemolytic uremic syndrome (HUS). Pediatr Nephrol 23:1749–1760. https://doi.org/10.1007/s00467-008-0935-6 CrossRefPubMed

15.

Siegler RL, Pavia AT, Christofferson RD, Milligan MK (1994) A 20-year population-based study of postdiarrheal hemolytic uremic syndrome in Utah. Pediatrics 94:35–40PubMed

16.

Spinale JM, Ruebner RL, Copelovitch L, Kaplan BS (2013) Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatr Nephrol 28:2097–2105. https://doi.org/10.1007/s00467-012-2383-6 CrossRefPubMed

Title: A simple prognostic index for Shigatoxin-related hemolytic uremic syndrome at onset: data from the ItalKid-HUS network
Authors: 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
Publication date: 01-11-2018
Publisher: Springer Berlin Heidelberg
Published in: European Journal of Pediatrics / Issue 11/2018
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-018-3198-7

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

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

Abstract

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

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