Pediatric Nephrology

, Volume 32, Issue 5, pp 869–878 | Cite as

Long-term health-related quality of life and psychological adjustment in children after haemolytic-uraemic syndrome

  • Helene Werner
  • Kathrin Buder
  • Markus A. Landolt
  • Thomas J. Neuhaus
  • Guido F. Laube
  • Giuseppina Spartà
Original Article



In children after haemolytic-uraemic syndrome (HUS), little is known about long-term health-related quality of life (HRQoL) and psychological adjustment as defined by behavioural problems, depressive symptoms and post-traumatic stress symptoms.


Sixty-two paediatric patients with a history of HUS were included in this study. Medical data of the acute HUS episode were retrieved retrospectively from hospital records. Data on the clinical course at study investigation were assessed by clinical examination and laboratory evaluation. HRQoL and psychological adjustment data were measured by standardised, parent- and self-reported questionnaires.


Haemolytic-uraemic syndrome was diagnosed at a mean of 6.5 years before the initiation of the study (standard deviation 2.9, range 0.1–15.7) years. Among the preschool children, parents reported that their child was less lively and energetic (HRQoL emotional dimension), while no increased behavioural problems were reported. In the school-age children, self- and proxy-reported HRQoL was well within or even above the norms, while increased total behavioural problems were found. The school-age children reported no increased depression scores. Also none of the children met the criteria for full or partial HUS-associated posttraumatic stress disorder.


Healthcare providers should be particularly alert to behavioural problems in school-age children with a history of HUS and to lower HRQoL in preschool children.


Kidney disease Outcome Behavioural problems Depression Post-traumatic stress disorders Paediatric patients 


Compliance with ethical standard

Funding sources

The study was supported by the “Erika Bär-Spycher” Foundation and by the “Kinder für Kinder” Foundation.

Conflict of interest

The authors declare that there is no conflict 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.


  1. 1.
    Andreoli SP (2009) Acute kidney injury in children. Pediatr Nephrol 24:253–263CrossRefPubMedGoogle Scholar
  2. 2.
    Schifferli A, von Vigier RO, Fontana M, Spartà G, Schmid H, Bianchetti MG, Rudin C, Unit SPS (2010) Hemolytic-uremic syndrome in Switzerland: a nationwide surveillance 1997-2003. Eur J Pediatr 169:591–598CrossRefPubMedGoogle Scholar
  3. 3.
    Spinale JM, Ruebner RL, Copelovitch L, Kaplan BS (2013) Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatr Nephrol 28:2097–2105CrossRefPubMedGoogle Scholar
  4. 4.
    Rosales A, Hofer J, Zimmerhackl L-B, Jungraithmayr TC, Riedl M, Giner T, Strasak A, Orth-Höller D, Würzner R, Karch H, German-Austrian HUS Study Group (2012) Need for long-term follow-up in enterohemorrhagic Escherichia coli-associated hemolytic uremic syndrome due to late-emerging sequelae. Clin Infect Dis 54:1413–1421CrossRefPubMedGoogle Scholar
  5. 5.
    Spinale JM, Ruebner RL, Kaplan BS, Copelovitch L (2013) Update on Streptococcus pneumoniae associated hemolytic uremic syndrome. Curr Opin Pediatr 25:203–208CrossRefPubMedGoogle Scholar
  6. 6.
    Fremeaux-Bacchi V, Fakhouri F, Garnier A, Bienaime F, Dragon-Durey M-A, Ngo S, Moulin B, Servais A, Provot F, Rostaing L, Burtey S, Niaudet P, Deschenes G, Lebranchu Y, Zuber J, Loirat C (2013) Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol 8:554–562CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Matza LS, Swensen AR, Flood EM, Secnik K, Leidy NK (2004) Assessment of health-related quality of life in children: A review of conceptual, methodological, and regulatory issues. Value Health 7:79–92CrossRefPubMedGoogle Scholar
  8. 8.
    de Ridder D, Geenen R, Kuijer R, van Middendorp H (2008) Psychological adjustment to chronic disease. Lancet 372:246–255CrossRefPubMedGoogle Scholar
  9. 9.
    Schlieper A, Orrbine E, Wells GA, Clulow M, McLaine PN, Rowe PC (1999) Neuropsychological sequelae of haemolytic uraemic syndrome. Investigators of the HUS Cognitive Study. Arch Dis Child 80:214–220CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Qamar IU, Ohali M, MacGregor DL, Wasson C, Krekewich K, Marcovitch S, Arbus GS (1996) Long-term neurological sequelae of hemolytic-uremic syndrome: a preliminary report. Pediatr Nephrol 10:504–506CrossRefPubMedGoogle Scholar
  11. 11.
    Schlieper A, Rowe PC, Orrbine E, Zoubek M, Clark W, Wolfish N, McLaine PN (1992) Sequelae of haemolytic uraemic syndrome. Arch Dis Child 67:930–934CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Lopes M, Ferraro A, Koch VH (2014) Health-related quality of life of children and adolescents with CKD stages 4-5 and their caregivers. Pediatr Nephrol 29:1239–1247CrossRefPubMedGoogle Scholar
  13. 13.
    Kul M, Cengel Kultur E, Senses Dinc G, Bilginer Y, Uluc S, Baykan H (2013) Quality of life in children and adolescents with chronic kidney disease: a comparative study between different disease stages and treatment modalities. Turk J Pediatr 55:493–499PubMedGoogle Scholar
  14. 14.
    Kilis-Pstrusinska K, Medynska A, Chmielewska IB, Grenda R, Kluska-Jozwiak A, Leszczynska B, Niedomagala J, Olszak-Szot I, Miklaszewska M, Szczepanska M, Tkaczyk M, Urzykowska A, Wasilewska A, Zachwieja K, Zajaczkowska M, Ziolkowska H, Zagozdzon I, Zwolinska D (2013) Perception of health-related quality of life in children with chronic kidney disease by the patients and their caregivers: Multicentre national study results. Qual Life Res 22:2889–2897CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Marciano RC, Soares CM, Diniz JS, Lima EM, Silva JM, Canhestro MR, Gazzinelli A, Melo CC, Dias CS, Simoes e Silva AC, Correa H, Oliveira EA (2011) Behavioral disorders and low quality of life in children and adolescents with chronic kidney disease. Pediatr Nephrol 26:281–290CrossRefPubMedGoogle Scholar
  16. 16.
    Buyan N, Turkmen MA, Bilge I, Baskin E, Haberal M, Bilginer Y, Mir S, Emre S, Akman S, Ozkaya O, Fidan K, Alpay H, Kavukcu S, Sever L, Ozcakar ZB, Dogrucan N (2010) Quality of life in children with chronic kidney disease (with child and parent assessments). Pediatr Nephrol 25:1487–1496CrossRefPubMedGoogle Scholar
  17. 17.
    Gerson AC, Wentz A, Abraham AG, Mendley SR, Hooper SR, Butler RW, Gipson DS, Lande MB, Shinnar S, Moxey-Mims MM, Warady BA, Furth SL (2010) Health-related quality of life of children with mild to moderate chronic kidney disease. Pediatrics 125:e349–357CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    McKenna AM, Keating LE, Vigneux A, Stevens S, Williams A, Geary DF (2006) Quality of life in children with chronic kidney disease - patient and caregiver assessments. Nephrol Dial Transpl 21:1899–1905CrossRefGoogle Scholar
  19. 19.
    Goldstein SL, Graham N, Burwinkle T, Warady B, Farrah R, Varni JW (2006) Health-related quality of life in pediatric patients with ESRD. Pediatr Nephrol 21:846–850CrossRefPubMedGoogle Scholar
  20. 20.
    Kogon AJ, Vander Stoep A, Weiss NS, Smith J, Flynn JT, McCauley E (2013) Depression and its associated factors in pediatric chronic kidney disease. Pediatr Nephrol 28:1855–1861CrossRefPubMedPubMedCentralGoogle Scholar
  21. 21.
    Hernandez E, Loza R, Vargas H, Jara M (2011) Depressive symptomatology in children and adolescents with chronic renal insufficiency undergoing chronic dialysis. Int J Nephrol 2011:798692. PubMedPubMedCentralGoogle Scholar
  22. 22.
    Amr M, Bakr A, El Gilany AH, Hammad A, El-Refaey A, El-Mougy A (2009) Multi-method assessment of behavior adjustment in children with chronic kidney disease. Pediatr Nephrol 24:341–347CrossRefPubMedGoogle Scholar
  23. 23.
    Hooper SR, Duquette PJ, Icard P, Wetherington CE, Harrell W, Gipson DS (2009) Social-behavioural functioning in paediatric chronic kidney disease. Child Care Health Dev 35:832–840CrossRefPubMedGoogle Scholar
  24. 24.
    Bakr A, Amr M, Sarhan A, Hammad A, Ragab M, El-Refaey A, El-Mougy A (2007) Psychiatric disorders in children with chronic renal failure. Pediatr Nephrol 22:128–131CrossRefPubMedGoogle Scholar
  25. 25.
    American Psychiatric Association (APA) (1994) The diagnostic and statistical manual of mental disorders. APA, Washington DCGoogle Scholar
  26. 26.
    Buder K, Latal B, Nef S, Neuhaus TJ, Laube GF, Spartà G (2015) Neurodevelopmental long-term outcome in children after hemolytic uremic syndrome. Pediatr Nephrol 30:503–513CrossRefPubMedGoogle Scholar
  27. 27.
    Buder K, Werner H, Landolt MA, Neuhaus TJ, Laube GF, Spartà G (2016) Health-related quality of life and mental health in parents of children with hemolytic uremic syndrome. Pediatr Nephrol 31:923–932CrossRefPubMedGoogle Scholar
  28. 28.
    Zipfel PF, Wolf G, John U, Kentouche K, Skerka C (2011) Novel developments in thrombotic microangiopathies: is there a common link between hemolytic uremic syndrome and thrombotic thrombocytic purpura? Pediatr Nephrol 26:1947–1956CrossRefPubMedGoogle Scholar
  29. 29.
    Hogg RJ, Furth S, Lemley KV, Portman R, Schwartz GJ, Coresh J, Balk E, Lau J, Levin A, Kausz AT, Eknoyan G, Levey AS (2003) National Kidney Foundation's Kidney Disease Outcomes Quality Initiative clinical practice guidelines for chronic kidney disease in children and adolescents: evaluation, classification, and stratification. Pediatrics 111:1416–1421CrossRefPubMedGoogle Scholar
  30. 30.
    Schwartz GJ, Munoz A, Schneider MF, Mak RH, Kaskel F, Warady BA, Furth SL (2009) New equations to estimate GFR in children with CKD. J Am Soc Nephrol 20:629–637CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Fekkes M, Bruil J, Vogels T (2004) TAPQOL-manual. Leiden Center for Child Health and Pediatrics LUMC-TNO, LeidenGoogle Scholar
  32. 32.
    Bisegger C, Cloetta B, Group TEK (2005) Kidscreen: Fragebogen zur Erfassung der gesundheitsbezogenen Lebensqualität von Kindern und Jugendlichen. Manual der deutschsprachigen Versionen für die Schweiz. Abteiung für Sozial- und Präventivmedzin der Universität, BernGoogle Scholar
  33. 33.
    Ravens-Sieberer U, Auquier P, Erhart M, Gosch A, Rajmil L, Bruil J, Power M, Duer W, Cloetta B, Czemy L, Mazur J, Czimbalmos A, Tountas Y, Hagquist C, Kilroe J (2007) The KIDSCREEN-27 quality of life measure for children and adolescents: psychometric results from a cross-cultural survey in 13 European countries. Qual Life Res 16:1347–1356CrossRefPubMedGoogle Scholar
  34. 34.
    Achenbach T (1991) Manual for the child behavior checklist/4-18. Department of Psychiatry, University of Vermont BurlingtonGoogle Scholar
  35. 35.
    Achenbach T, Rescorla L (2000) Manual for the ASEBA preschool forms and profiles. Research Center for Children, Youth & Families, University of Vermont, BurlingtonGoogle Scholar
  36. 36.
    Arbeitsgruppe Deutsche Child Behavior Checklist (2002) Elternfragebogen für Klein- und Vorschulkinder (CBCL/1.5-5). Arbeitsgruppe Kinder-, Jugend- und Familiendiagnostik (KJFD), Cologne, GermanyGoogle Scholar
  37. 37.
    Steinhausen H, Winkler Metzke C, Kannenberg R (1996) Handbuch: Elternfragebogen über das Verhalten von Kindern und Jugendlichen. Die Zürcher Ergebnisse zur deutschen Fassung der Child Behavior Checklist (CBCL). Psychiatrische Universitäts-Poliklinik für Kinder und Jugendliche, ZurichGoogle Scholar
  38. 38.
    Kovacs M (1985) The Children's Depression, Inventory (CDI). Psychopharmacol Bull 21:995–998PubMedGoogle Scholar
  39. 39.
    Stiensmeier-Pelster J, Schürmann M, Duda K (2000) Handanweisung. Depressions-Inventar für Kinder und Jugendliche. Hogrefe, GöttingenGoogle Scholar
  40. 40.
    Landolt M, Schnyder U, Maier T, Schoenbucher V, Mohler-Kuo M (2013) Trauma exposure and posttraumatic stress disorder in adolescents: a national survey in Switzerland. J Trauma Stress 26:209–216CrossRefPubMedGoogle Scholar
  41. 41.
    Steinberg AM, Brymer MJ, Kim S, Briggs EC, Ippen CG, Ostrowski SA, Gully KJ, Pynoos RS (2013) Psychometric properties of the UCLA PTSD reaction index: part I. J Trauma Stress 26:1–9CrossRefPubMedGoogle Scholar
  42. 42.
    Largo RH, Pfister D, Molinari L, Kundu S, Lipp A, Duc G (1989) Significance of prenatal, perinatal and postnatal factors in the development of AGA preterm infants at five to seven years. Dev Med Child Neurol 31:440–456CrossRefPubMedGoogle Scholar
  43. 43.
    Cohen AH (1988) Statistical power analysis for the bevahioral sciences. Lawrence Erlbaum Associates, HillsdaleGoogle Scholar
  44. 44.
    Askenazi DJ, Feig DI, Graham NM, Hui-Stickle S, Goldstein SL (2006) 3-5 Year longitudinal follow-up of pediatric patients after acute renal failure. Kidney Int 69:184–189CrossRefPubMedGoogle Scholar
  45. 45.
    Faul F, Erdfelder E, Lang AG, Buchner A (2007) G*Power 3: A flexible statistical power analysis program for the social, behavioral, and biomedical sciences. Behav Res Methods 39:175–191CrossRefPubMedGoogle Scholar
  46. 46.
    Etling P (2003) Überprüfung der psychometrischen Parameter von CBCL11/2-5 und C-TRF an einer deutschen Stichprobe. Johann Wolfgang Goethe-Universitätsklinik, Frankfurt am MainGoogle Scholar

Copyright information

© IPNA 2016

Authors and Affiliations

  • Helene Werner
    • 1
    • 2
  • Kathrin Buder
    • 3
  • Markus A. Landolt
    • 1
    • 2
  • Thomas J. Neuhaus
    • 4
  • Guido F. Laube
    • 3
  • Giuseppina Spartà
    • 3
  1. 1.Department of Psychosomatics and PsychiatryUniversity Children’s Hospital ZurichZurichSwitzerland
  2. 2.Division of Child and Adolescent Health Psychology, Department of PsychologyUniversity of ZurichZurichSwitzerland
  3. 3.Paediatric Nephrology UnitUniversity Children’s Hospital ZurichZurichSwitzerland
  4. 4.Children’s Hospital of Lucerne, Cantonal Hospital of LucerneLucerne 16Switzerland

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