Skip to main content
SpringerLink
Log in

Hemoconcentration: a major risk factor for neurological involvement in hemolytic uremic syndrome

  • Original Article
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript

Abstract

Background

Shigatoxin-associated hemolytic uremic syndrome (STEC-HUS) is a common thrombotic microangiopathy (TMA) in which central nervous system (CNS) involvement is responsible for the majority of deaths and for severe long-term sequelae. We have analyzed the role of hemoconcentration in disease severity.

Methods

This was a retrospective review of the records and laboratory data at presentation of all patients with STEC-HUS cases (n = 61) over a 10-year period. The patients were grouped into three severity classes: group A, comprising patients who did not require dialysis; group B, patients who were dialyzed without CNS involvement; group C, patients with CNS involvement.

Results

Patients with CNS involvement (group C) had a higher mean hemoglobin level (11.2 ± 2.3 g/dL) than those of group A or B ( 9.4 ± 2.1 and 7.5 ± 1.9 g/dL, respectively; p < 0.0001). We also observed that the higher the initial hemoglobin level, the more severe the long-term renal damage (p < 0.007).

Conclusions

In patients with STEC-HUS, hemoconcentration and hypovolemia may be responsible for more severe ischemic organ damage (both short and long term) at disease onset, and these signs should be regarded as risk factors for CNS damage and for more severe TMA. Therefore, we recommend that hydration status should be actively monitored in HUS patients and that dehydration, when diagnosed, should be promptly corrected.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Flynn JT (1998) Causes, management approaches, and outcome of acute renal failure in children. Curr Opin Pediatr 10(2):184–189

    Article  CAS  PubMed  Google Scholar 

  2. Ruggenenti P, Noris M, Remuzzi G (2001) Thrombotic microangiopathy, haemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Kidney Int 60:831–846

    Article  CAS  PubMed  Google Scholar 

  3. Gianviti A, Rosmini F, Caprioli A, Corona R, Matteucci MC, Principato F, Luzzi I, Rizzoni G (1994) Haemolytic uraemic syndrome in childhood: surveillance and case-control studies in Italy. Italia HUS Study Group. Pediatr Nephrol 8:705–709

    Article  CAS  PubMed  Google Scholar 

  4. Kaplan BS, Meyers KE, Shulman SI (1998) The pathogenesis and treatment of haemolytic uremic syndrome. J Am Soc Nephrol 9:1126–1133

    CAS  PubMed  Google Scholar 

  5. Schieppati A, Ruggenenti P, Cornejo RP, Errario F, Gregorini G, Zucchelli P, Rossi E, Remuzzi G (1992) Renal function at hospital admission as a prognostic factor in adult haemolytic uremic syndrome. The Italian Registry for Haemolytic Uremic Syndrome. J Am Soc Nephrol 2:1640–1644

    CAS  PubMed  Google Scholar 

  6. Taylor CM, Chua C, Howie AJ, Ridson RA (2004) Clinic-pathological findings in diarrhoea-negative haemolytic uremic syndrome. Pediatr Nephrol 19:419–425

    Article  CAS  PubMed  Google Scholar 

  7. Wong CS, Mooney JC, Brandt JR, Staples AO, Jelacic S, Boster DR, Watkins SL, Tarr PI (2012) Risk factors for the hemolytic uremic syndrome in children infected with Escherichia coli O157:H7: a multivariable analysis. Clin Infect Dis 55(1):33–41

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  8. Scheiring J, Andreoli SP, Zimmerhackl LB (2008) Treatment and outcome of Shiga-toxin-associated hemolytic uremic syndrome (HUS). Pediatr Nephrol 23:1749–1760

    Article  PubMed  Google Scholar 

  9. Noris M, Remuzzi G (2005) Hemolytic uremic syndrome. J Am Soc Nephrol 6:1035–1050

    Article  Google Scholar 

  10. Eriksson K, Boyd S, Tasker R (2001) Acute neurology and neurophysiology of haemolytic uraemic syndrome. Arch Dis Child 84:434–435

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  11. Oakes R, Siegler R, Mc Reynolds M, Pysher T, Pavia A (2006) Predictors of fatality in postdiarreheal haemolytic uremic syndrome. Pediatrics 117:1656–166

    Article  PubMed  Google Scholar 

  12. 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

    PubMed  Google Scholar 

  13. Rooney JC, Anderson RM, Hopkins IJ (1971) Clinical and pathological aspects of central nervous system involvement in the hemolytic-uremic syndrome. Aust Paediatr J 7:28–33

    CAS  PubMed  Google Scholar 

  14. Gianantonio CA, Vitacco M, Mendilaharzu F, Gallo GE, Sojo ET (1973) The hemolytic-uremic syndrome. Nephrol 11:172–192

    Google Scholar 

  15. Bale JF, Brasher C, Sigler RL (1980) CNS manifestations of the hemolytic-uremic syndrome: relationship to metabolic alterations and prognosis. Am J Dis Child 134:869–872

    Article  PubMed  Google Scholar 

  16. Upadhyaya K, Barwick K, Fishaut M, Kashgarian M, Siegel NJ (1980) The importance of nonrenal involvement in haemolytic uremic syndrome. Pediatrics 65:115–120

    CAS  PubMed  Google Scholar 

  17. Nathanson S, Kwon T, Elmaleh M, Charbit M, Launay EA, Harambat J, Brun M, Ranchin B, Bandin F, Cloarec S, Bourdat-Michel G, Piètrement C, Champion G, Ulinski T, Deschênes G (2010) Acute neurological involvement in diarrhea-associated hemolytic uremic syndrome. Clin J Am Soc Nephrol 5:1218–1228

  18. Sheth K, Herbert S, Haworth N (1986) Neurogical involvement in hemolytic uremic syndrome. Ann Neurol 19:90–93

    Article  CAS  PubMed  Google Scholar 

  19. Dhuna A, Pascual LA, Talwar D, Torres F (1992) EEG and seizures in children with haemolytic uremic syndrome. Epilepsia 33(3):482–486

    Article  CAS  PubMed  Google Scholar 

  20. Jeong YK, Kim IO, Kin WS, Hwamg YS, Choi Y, Yeon KM (1994) Haemolytic uremic syndrome: MR findings of CNS complications. Pediatr Radiol 24:584–586

    Article  Google Scholar 

  21. Theobald I, Kuwertz-Broking E, Schiborr M, Heindel W (2001) Central nervous system involvement in haemolytic uremic syndrome (HUS)—a retrospective analysis of cerebral CT and MRI studies. Clin Nephrol 56(1):S3–S8

    CAS  PubMed  Google Scholar 

  22. Hager A, Staudt M, von Klare B, Einsiedel Grafin H, Krageloh-Mann I (1999) Hemolytic-uremic syndrome with involvement of basal ganglia and cerebellum. Neuropediatrics 30:210–213

    Article  CAS  PubMed  Google Scholar 

  23. Ake J, Jelacic S, Ciol M, Watkins S, Murray K, Christie D, Klein E, Tarr P (2005) Relative nephroprotection during Escherichia Coli 0157:H7 infections: association with intravenous volume expansion. Pediatrics 115:673–680

    Article  Google Scholar 

  24. Hickey CA, Beattie TJ, Cowieson J, Miyashita Y, Strife CF, Frem JC, Peterson JM, Butani L, Jones DP, Havens PL, Patel HP, Wong CS, Andreoli SP, Rothbaum RJ, Beck AM, Tarr PI (2011) Early volume expansion during diarrhea and relative nephroprotection during subsequent hemolytic uremic syndrome. Arch Pediatr Adolesc Med 165:884–889

    Article  PubMed Central  PubMed  Google Scholar 

  25. 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(8):1407–1410

    Article  PubMed  Google Scholar 

  26. Kidney Disease: Improving Global Outcomes (KDIGO) CKD Work Group (2013) KDIGO 2012 Clinical Practice Guideline for the evaluation and management of chronic kidney disease. Kidney Int Suppl 3:1–150

  27. Schwartz GJ, Brion LP, Spitzer A (1987) The use of plasma creatinine concentration for estimating glomerular filtration rate in infants, children, and adolescents. Pediatr Clin North Am 34:571–590

    CAS  PubMed  Google Scholar 

  28. Gianviti A, Tozzi AE, De Petris L, Caprioli A, Ravà L, Edefonti A, Ardissino G, Montini G, Zacchello G, Ferretti A, Pecoraro C, De Palo T, Caringella A, Gaido M, Coppo R, Perfumo F, Miglietti N, Ratsche I, Penza R, Capasso G, Maringhini S, Li Volti S, Setzu C, Pennesi M, Bettinelli A, Peratoner L, Pela I, Salvaggio E, Lama G, Maffei S, Rizzoni G (2003) Risk factors for poor renal prognosis in children with haemolytic uremic syndrome. Pediatr Nephrol 18:1229–1235

    Article  PubMed  Google Scholar 

  29. Kelles A, Van Dyck M, Proesmans W (1994) Childhood haemolytic uraemic syndrome: long term outcome and prognostic features. Eur J Pediatr 153:38–42

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  31. Gallo GE, Gianantonio CA (1995) Extrarenal involvement in post-diarrhoea-associated haemolytic uraemia syndrome. Pediatr Nephrol 9:117–119

    Article  CAS  PubMed  Google Scholar 

  32. Stricklett PK, Hughes AK, Ergonul Z, Kohan DE (2002) Molecular basis for up-regulation by inflammatory cytokines of Shiga toxin 1 cytotoxicity and globotriaosylceramide expression. J Infect Dis 186(7):976–982

    Article  CAS  PubMed  Google Scholar 

  33. Holtz LR, Neill MA, Tarr PI (2009) Acute bloody diarrhea: a medical emergency for patients of all ages. Gastroenterology 136:1887–1898

    Article  PubMed  Google Scholar 

  34. Coad NA, Marshall T, Rowe B, Taylor CM (1991) Changes in the postenteropathic form of the hemolytic uremic syndrome in children. Clin Nephrol 35(1):10–16

    CAS  PubMed  Google Scholar 

Download references

Acknowledgments

Gianluigi Ardissino, Center for HUS Control, Prevention and Management, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy, had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Funding source

All aspects of this study were funded by a research grant from the "Progetto Alice ONLUS. Associazione per la lotta alla SEU"

Financial disclosure

None.

Conflict of interest

None.

Author information

Authors and Affiliations

  1. Center for HUS Control, Prevention and Management, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via Commenda 9, 20122, Milan, Italy

    Gianluigi Ardissino, Valeria Daccò, Sara Testa, Cristina Felice Civitillo, Francesca Tel, Ilaria Possenti, Mirco Belingheri, Pierangela Castorina, Nicolò Bolsa-Ghiringhelli, Silvana Tedeschi, Fabio Paglialonga, Stefania Salardi, Elena Zoia, Patrizia Salice & Giovanna Chidini

  2. Unit of Epidemiology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

    Dario Consonni

Authors
  1. Gianluigi Ardissino
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Valeria Daccò
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Sara Testa
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Cristina Felice Civitillo
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Francesca Tel
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Ilaria Possenti
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Mirco Belingheri
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Pierangela Castorina
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Nicolò Bolsa-Ghiringhelli
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Silvana Tedeschi
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Fabio Paglialonga
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Stefania Salardi
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Dario Consonni
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Elena Zoia
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Patrizia Salice
    View author publications

    You can also search for this author in PubMed Google Scholar

  16. Giovanna Chidini
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Gianluigi Ardissino.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Ardissino, G., Daccò, V., Testa, S. et al. Hemoconcentration: a major risk factor for neurological involvement in hemolytic uremic syndrome. Pediatr Nephrol 30, 345–352 (2015). https://doi.org/10.1007/s00467-014-2918-0

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00467-014-2918-0

Keywords

Search

Navigation