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Neuropediatrics 2017; 48(05): 363-370
DOI: 10.1055/s-0037-1603643
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Neurological Involvement in Pediatric Hemolytic Uremic Syndrome: A Symptom-Oriented Analysis

Sevim Şahin
1   Division of Pediatric Neurology, Department of Pediatrics, School of Medicine, Karadeniz Technical University, Trabzon, Turkey
,
Elif Bahat Özdoğan
2   Division of Pediatric Nephrology, Department of Pediatrics, School of Medicine, Karadeniz Technical University, Trabzon, Turkey
,
Gülay Kaya
3   Pediatric Intensive Care Unit, Department of Pediatrics, School of Medicine, Karadeniz Technical University, Trabzon, Turkey
,
Nezir Özgün
4   Department of Pediatric Neurology, Diyarbakır Children's Hospital, Diyarbakır, Turkey
,
Ali Cansu
1   Division of Pediatric Neurology, Department of Pediatrics, School of Medicine, Karadeniz Technical University, Trabzon, Turkey
,
Mukaddes Kalyoncu
2   Division of Pediatric Nephrology, Department of Pediatrics, School of Medicine, Karadeniz Technical University, Trabzon, Turkey
,
Embiya Dilber
5   Department of Pediatrics, School of Medicine, Karadeniz Technical University, Trabzon, Turkey
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Publikationsverlauf

01. Februar 2017

24. April 2017

Publikationsdatum:
01. Juni 2017 (online)

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Abstract

Background Neurological involvement is common in hemolytic uremic syndrome (HUS), but each neurological symptom may be due to a variety of factors.

Objective We aimed to evaluate predisposing factors to the neurological symptoms in HUS.

Materials and Methods The 10-year follow-up data on HUS patients were retrospectively analyzed. Statistical comparisons were made across subgroups based on age and neurological symptoms.

Results The neurological involvement rate was 37.5%. The female-to-male ratio increased in patients with neurological involvement (3.8 vs. 1.37). Regarding all HUS patients, hemoglobin levels were higher in patients with paresis. The rate of paresis was twofold higher in patients with a hemoglobin level above 11 g/dL (p < 0.05). In diarrhea-associated HUS patients, D-dimer and C-reactive protein levels were higher in patients with paresis, and leukocyte counts were higher in patients with seizures. Patients with altered consciousness had higher creatinine levels. The survival rate was significantly lower in patients with a reticulocyte percentage of less than 2% (50 vs. 100%).

Conclusion Results of our study indicate that neurological involvement depends on overall findings in HUS. A symptom-oriented approach, which is different from that employed in previous studies, reveals some clues to the pathogenesis and management of these patients.

Keywords

hemolytic uremic syndrome - neurological involvement - child - seizure - paresis

Disclosures

The authors state that they have no interests that might be perceived as posing a conflict or bias. They received no financial support for the research, authorship, and/or publication of this article.


 

  • References

  • 1 Ardissino G, Daccò V, Testa S. , et al. Hemoconcentration: a major risk factor for neurological involvement in hemolytic uremic syndrome. Pediatr Nephrol 2015; 30 (02) 345-352
    CrossrefPubMedGoogle Scholar
  • 2 Ruggenenti P, Noris M, Remuzzi G. Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Kidney Int 2001; 60 (03) 831-846
    CrossrefPubMedGoogle Scholar
  • 3 Gitiaux C, Krug P, Grevent D. , et al. Brain magnetic resonance imaging pattern and outcome in children with haemolytic-uraemic syndrome and neurological impairment treated with eculizumab. Dev Med Child Neurol 2013; 55 (08) 758-765
    PubMedGoogle Scholar
  • 4 Trachtman H, Austin C, Lewinski M, Stahl RA. Renal and neurological involvement in typical Shiga toxin-associated HUS. Nat Rev Nephrol 2012; 8 (11) 658-669
    PubMedGoogle Scholar
  • 5 Braune SA, Wichmann D, von Heinz MC. , et al. Clinical features of critically ill patients with Shiga toxin-induced hemolytic uremic syndrome. Crit Care Med 2013; 41 (07) 1702-1710
    CrossrefPubMedGoogle Scholar
  • 6 Bauer A, Loos S, Wehrmann C. , et al. Neurological involvement in children with E. coli O104:H4-induced hemolytic uremic syndrome. Pediatr Nephrol 2014; 29 (09) 1607-1615
    CrossrefPubMedGoogle Scholar
  • 7 Sheth KJ, Swick HM, Haworth N. Neurological involvement in hemolytic-uremic syndrome. Ann Neurol 1986; 19 (01) 90-93
    CrossrefPubMedGoogle Scholar
  • 8 Ardissino G, Salardi S, Colombo E. , et al. Epidemiology of haemolytic uremic syndrome in children. Data from the North Italian HUS network. Eur J Pediatr 2016; 175 (04) 465-473
    CrossrefPubMedGoogle Scholar
  • 9 Cimolai N, Morrison BJ, Carter JE. Risk factors for the central nervous system manifestations of gastroenteritis-associated hemolytic-uremic syndrome. Pediatrics 1992; 90 (04) 616-621
    PubMedGoogle Scholar
  • 10 Nathanson S, Kwon T, Elmaleh M. , et al. Acute neurological involvement in diarrhea-associated hemolytic uremic syndrome. Clin J Am Soc Nephrol 2010; 5 (07) 1218-1228
    CrossrefPubMedGoogle Scholar
  • 11 Elfving P, Marjoniemi O, Niinisalo H. , et al. Estimating the incidence of connective tissue diseases and vasculitides in a defined population in Northern Savo area in 2010. Rheumatol Int 2016; 36 (07) 917-924
    CrossrefPubMedGoogle Scholar
  • 12 Soybilgic A. Neuropsychiatric systemic lupus erythematosus in children. Pediatr Ann 2015; 44 (06) e153-e158
    CrossrefPubMedGoogle Scholar
  • 13 Loos S, Ahlenstiel T, Kranz B. , et al. 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 2012; 55 (06) 753-759
    CrossrefPubMedGoogle Scholar
  • 14 Ganapathi MK, Rzewnicki D, Samols D, Jiang SL, Kushner I. Effect of combinations of cytokines and hormones on synthesis of serum amyloid A and C-reactive protein in Hep 3B cells. J Immunol 1991; 147 (04) 1261-1265
    PubMedGoogle Scholar
  • 15 Dierkes F, Andriopoulos N, Sucker C. , et al. Indicators of acute and persistent renal damage in adult thrombotic microangiopathy. PLoS One 2012; 7 (01) e30886
    CrossrefPubMedGoogle Scholar
  • 16 Arslan S, Ugurlu S, Bulut G, Akkurt I. The association between plasma D-dimer levels and community-acquired pneumonia. Clinics (Sao Paulo) 2010; 65 (06) 593-597
    CrossrefPubMedGoogle Scholar
  • 17 Rose PE, Enayat SM, Sunderland R, Short PE, Williams CE, Hill FG. Abnormalities of factor VIII related protein multimers in the haemolytic uraemic syndrome. Arch Dis Child 1984; 59 (12) 1135-1140
    CrossrefPubMedGoogle Scholar
  • 18 Forsyth KD, Simpson AC, Fitzpatrick MM, Barratt TM, Levinsky RJ. Neutrophil-mediated endothelial injury in haemolytic uraemic syndrome. Lancet 1989; 2 (8660): 411-414
    PubMedGoogle Scholar
  • 19 Landoni VI, Schierloh P, de Campos Nebel M. , et al. Shiga toxin 1 induces on lipopolysaccharide-treated astrocytes the release of tumor necrosis factor-alpha that alter brain-like endothelium integrity. PLoS Pathog 2012; 8 (03) e1002632
    CrossrefPubMedGoogle Scholar
  • 20 Liu Z, Wang S, Liu J, Wang F, Liu Y, Zhao Y. Leukocyte infiltration triggers seizure recurrence in a rat model of temporal lobe epilepsy. Inflammation 2016; 39 (03) 1090-1098
    PubMedGoogle Scholar
  • 21 Kim SY, Buckwalter M, Soreq H, Vezzani A, Kaufer D. Blood-brain barrier dysfunction-induced inflammatory signaling in brain pathology and epileptogenesis. Epilepsia 2012; 53 (06) (Suppl. 06) 37-44
    CrossrefPubMedGoogle Scholar
  • 22 Brouns R, De Deyn PP. Neurological complications in renal failure: a review. Clin Neurol Neurosurg 2004; 107 (01) 1-16
    CrossrefPubMedGoogle Scholar
  • 23 Hosoya K, Tachikawa M. Roles of organic anion/cation transporters at the blood-brain and blood-cerebrospinal fluid barriers involving uremic toxins. Clin Exp Nephrol 2011; 15 (04) 478-485
    CrossrefPubMedGoogle Scholar
  • 24 Gofshteyn JS, Stephenson DJ. Diagnosis and management of childhood headache. Curr Probl Pediatr Adolesc Health Care 2016; 46 (02) 36-51
    CrossrefPubMedGoogle Scholar
  • 25 Agarwal R, Davis C, Altinok D, Serajee FJ. Posterior reversible encephalopathy and cerebral vasoconstriction in a patient with hemolytic uremic syndrome. Pediatr Neurol 2014; 50 (05) 518-521
    CrossrefPubMedGoogle Scholar
  • 26 Gulleroglu K, Fidan K, Hançer VS, Bayrakci U, Baskin E, Soylemezoglu O. Neurologic involvement in atypical hemolytic uremic syndrome and successful treatment with eculizumab. Pediatr Nephrol 2013; 28 (05) 827-830
    CrossrefPubMedGoogle Scholar
  • 27 Koehl B, Boyer O, Biebuyck-Gougé N. , et al. Neurological involvement in a child with atypical hemolytic uremic syndrome. Pediatr Nephrol 2010; 25 (12) 2539-2542
    CrossrefPubMedGoogle Scholar
  • 28 Woodruff TM, Ager RR, Tenner AJ, Noakes PG, Taylor SM. The role of the complement system and the activation fragment C5a in the central nervous system. Neuromolecular Med 2010; 12 (02) 179-192
    CrossrefPubMedGoogle Scholar
  • 29 Chavarria L, Cordoba J. Magnetic resonance of the brain in chronic and acute liver failure. Metab Brain Dis 2014; 29 (04) 937-944
    CrossrefPubMedGoogle Scholar
  • 30 Janus J, Moerschel SK. Evaluation of anemia in children. Am Fam Physician 2010; 81 (12) 1462-1471
    PubMedGoogle Scholar
  • 31 Scheiring J, Andreoli SP, Zimmerhackl LB. Treatment and outcome of Shiga-toxin-associated hemolytic uremic syndrome (HUS). Pediatr Nephrol 2008; 23 (10) 1749-1760
    CrossrefPubMedGoogle Scholar