Abstract
Hemolytic-uremic syndrome (HUS) is a leading cause of acute renal failure in childhood. In its typical presentation, it is preceded by an episode of diarrhea mostly due to Shiga-toxin-producing Escherichia coli. There is important geographical variation of many aspects of this syndrome. Nationwide data on childhood HUS in Switzerland have not been available so far. In a prospective national study through the Swiss Pediatric Surveillance Unit 114 cases (median age 21 months, 50% boys) were reported between April 1997 and March 2003 by 38 pediatric units (annual incidence 1.42 per 105 children ≤16 years). Shiga-toxin-producing E. coli were isolated in 32 (60%) of tested stool samples, serotype O157:H7 in eight. Sixteen children presented with only minimal renal involvement, including three with underlying urinary tract infection. Six patients presented with atypical hemolytic-uremic syndrome, and six with HUS due to invasive Streptococcus pneumoniae infection. Mortality was 5.3%, including two out of six children with S. pneumoniae infection. The severity of thrombocytopenia and the presence of central nervous system involvement significantly correlated with mortality. In conclusion, childhood HUS is not rare in Switzerland. Contrasting other countries, E. coli O157:H7 play only a minor role in the etiology. Incomplete manifestation is not uncommon.
Similar content being viewed by others
Abbreviations
- HUS:
-
hemolytic-uremic syndrome
- D+ HUS:
-
typical HUS (enteropathic or non-enteropathic)
- D− HUS:
-
atypical HUS
- cHUS:
-
complete HUS
- iHUS:
-
incomplete HUS
- Stx:
-
Shiga toxin
- EHEC:
-
enterohemorrhagic Escherichia coli
- NENT:
-
Swiss National Centre for Enteropathogenic Bacteria
- SPSU:
-
Swiss Pediatric Surveillance Unit
- STEC:
-
Shiga-toxin-producing Escherichia coli
- UTI:
-
urinary tract infection
- CI:
-
confidence interval
References
Ake JA, Jelacic S, Ciol MA, Watkins SL, Murray KF, Christie DL, Klein EJ, Tarr PI (2005) Relative nephroprotection during Escherichia coli O157:H7 infections: association with intravenous volume expansion. Pediatrics 115(6):e673–e680
Banatvala N, Griffin PM, Greene KD, Barrett TJ, Bibb WF, Green JH, Wells JG (2001) The United States National Prospective Hemolytic Uremic Syndrome Study: microbiologic, serologic, clinical, and epidemiologic findings. J Infect Dis 183(7):1063–1070
Bell BP, Goldoft M, Griffin PM, Davis MA, Gordon DC, Tarr PI, Bartleson CA, Lewis JH, Barrett TJ, Wells JG et al (1994) A multistate outbreak of Escherichia coli O157:H7-associated bloody diarrhea and hemolytic uremic syndrome from hamburgers. The Washington experience. JAMA 272(17):1349–1353
Bell BP, Griffin PM, Lozano P, Christie DL, Kobayashi JM, Tarr PI (1997) Predictors of hemolytic uremic syndrome in children during a large outbreak of Escherichia coli O157:H7 infections. Pediatrics 100(1):E12
Besbas N, Karpman D, Landau D, Loirat C, Proesmans W, Remuzzi G, Rizzoni G, Taylor CM, Van de Kar N, Zimmerhackl LB, European Paediatric Research Group of HUS (2006) A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders. Kidney Int 70(3):423–431
Boyce TG, Swerdlow DL, Griffin PM (1995) Escherichia coli O157:H7 and the hemolytic-uremic syndrome. N Engl J Med 333(6):364–368
Caprioli A, Tozzi AE, Rizzoni G, Karch H (1997) Non-O157 Shiga toxin-producing Escherichia coli infections in Europe. Emerg Infect Dis 3(4):578–579
Cimolai N, Carter JE, Morrison BJ, Anderson JD (1990) Risk factors for the progression of Escherichia coli O157:H7 enteritis to hemolytic-uremic syndrome. J Pediatr 116(4):589–592
Copelovitch L, Kaplan BS (2008) The thrombotic microangiopathies. Pediatr Nephrol 23(10):1761–1767
Copelovitch L, Kaplan BS (2008) Streptococcus pneumoniae-associated hemolytic uremic syndrome. Pediatr Nephrol 23(11):1951–1956
Elliott EJ, Robins-Browne RM, O’Loughlin EV, Bennett-Wood V, Bourke J, Henning P, Hogg GG, Knight J, Powell H, Redmond D, Contributors to the Australian Paediatric Surveillance Unit (2001) Nationwide study of haemolytic uraemic syndrome: clinical, microbiological, and epidemiological features. Arch Dis Child 85(2):125–131
Essers B, Burnens AP, Lanfranchini FM, Somaruga SG, von Vigier RO, Schaad UB, Aebi C, Bianchetti MG (2000) Acute community-acquired diarrhea requiring hospital admission in Swiss children. Clin Infect Dis. 31(1):192–196
Garg AX, Suri RS, Baroowman N, Rehman F, Matsell D, Rosas-Arellano MP, Salvadori M, Haynes RB, Clark WF (2003) Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: a systematic review, meta-analysis, and meta-regression. JAMA 290(10):1360–1370
Gasser C, Gautier E, Steck A, Siebenmann RE, Oechslin R (1955) Hemolytic-uremic syndrome: bilateral necrosis of the renal cortex in acute acquired hemolytic anemia. Schweiz Med Wochenschr 85(38–39):905–909
Gerber A, Karch H, Allerberger F, Verweyen HM, ZImmerhackl LB (2002) Clinical course and the role of shiga toxin-producing Escherichia coli infection in the hemolytic-uremic syndrome in pediatric patients, 1997–2000, in Germany and Austria: a prospective study. J Infect Dis 186(4):493–500
Hassink RI, Zeerleder SS, Truttmann AC, Bianchetti MG (1997) Hemolytic-uremic syndrome after Escherichia coli urinary tract infection. Pediatr Infect Dis J 16(8):828
Ikeda K, Ida O, Kimoto K, Takatorige T, Nakanishi N, Tatara K (2000) Predictors for the development of haemolytic uraemic syndrome with Escherichia coli O157:H7 infections: with focus on the day of illness. Epidemiol Infect 124(3):343–349
Imoberdorf G, Bianchetti MG, Rossi E, Gugler E, Oetliker OH (1993) Hemolytic-uremic syndrome in children: a 19-year retrospective study. Schweiz Med Wochenschr 123(29):1439–1444
Johnson KE, Thorpe CM, Sears CL (2006) The erging clinical importance of non-O157 Shiga toxin-producing Escherichia coli. Clin Infect Dis 43(12):1587–1595
Kernland KH, Laux-End R, Truttmann AC, Reymond D, Bianchetti MG (1997) How is hemolytic-uremic syndrome in childhood acquired in Switzerland? Schweiz Med Wochenschr 127(29–30):1229–1233
Loirat C, Noris M, Fremeaux-Bacchi V (2008) Complement and the atypical hemolytic uremic syndrome in children. Pediatr Nephrol 23(11):1957–1972
López EL, Contrini MM, Devoto S, de Rosa MF, Graña MG, Aversa L, Gómez HF, Genero MH, Cleary TG (1995) Incomplete hemolytic-uremic syndrome in Argentinean children with bloody diarrhea. J Pediatr 127(3):364–367
Lopez EL, Diaz M, Grinstein S, Devoto S, Mendilaharzu F, Murray BE, Ashkenazi S, Rubeglio E, Woloj M, Vasquez M et al (1989) Hemolytic uremic syndrome and diarrhea in Argentine children: the role of Shiga-like toxins. J Infect Dis 160(3):469–475
Noris M, Brioschi S, Caprioli J, Todeschini M, Bresin E, Porrati F, Gamba S, Remuzzi G, International Registry of recurrent and familial HUS/TTP (2003) Familial haemolytic uraemic syndrome and an MCP mutation. Lancet 362(9395):1542–1547
Scheiring J, Andreoli SP, Zimmerhackl LB (2008) Treatment and outcome of Shiga-toxin-associated hemolytic uremic syndrome (HUS). Pediatr Nephrol 23(10):1749–1760
Siegler RL, Pavia AT, Christofferson RD, Milligan MK (1994) A 20-year population-based study of postdiarrheal hemolytic uremic syndrome in Utah. Pediatrics 94(1):35–40
Siegler RL (1994) Spectrum of extrarenal involvement in postdiarrheal hemolytic-uremic syndrome. J Pediatr 125(4):511–518
Simonetti GD, Dumont-Dos Santos K, Pachlopnik JM, Ramelli G, Bianchetti MG (2003) Hemolytic uremic syndrome linked to infectious mononucleosis. Pediatr Nephrol 18(11):1193–1194
Starr M, Bennet-Wood V, Bigham AK, de Koning-Ward TF, Bordun AM, Lightfoot D, Bettelheim KA, Jones CL, Robins-Browne RM (1998) Hemolytic-uremic syndrome following urinary tract infection with enterohemorrhagic Escherichia coli: case report and review. Clin Infect Dis 27(2):310–315
Tarr PI, Beill MA, Clausen CR, Watkins SL, Christie DL, Hickman RO (1990) Escherichia coli O157:H7 and the hemolytic uremic syndrome: importance of early cultures in establishing the etiology. J Infect Dis 162(2):553–556
Trachtman H (2008) Introduction: education teaching article series on hemolytic uremic syndrome. Pediatr Nephrol 23(9):1423–1424
Verweyen HM, Karch H, Allerberger F, Zimmerhackl LB (1999) Enterohemorrhagic Escherichia coli (EHEC) in pediatric hemolytic-uremic syndrome: a prospective study in Germany and Austria. Infection 27(6):341–347
von Vigier RO, Offermann N, Beretta-Piccoli BC, Bianchetti MG (2000) Paucisymptomatic hemolytic uremic syndrome. Pediatr Nephrol 14(5):436–437
Waters AM, Kerecuk L, Luk D, Hag MR, Fitzpatrick MM, Gilbert RD, Inward C, Jones C, Pichon B, Reid C, Slack MP, Van't Hoff W, Dillon MJ, Taylor CM, Tullus K (2007) Hemolytic uremic syndrome associated with invasive pneumococcal disease: the United Kingdom experience. J Pediatr 151(2):140–144
Wolf G (2004) Not known from ADAM(TS-13)—novel insights into the pathophysiology of thrombotic microangiopathies. Nephrol Dial Transplant 19(7):1687–1693
Wong CS, Jelacic S, Habeeb RL, Watkins SL, Tarr PI (2000) The risk of the hemolytic-uremic syndrome after antibiotic treatment of Escherichia coli O157:H7 infections. N Engl J Med 342(26):1930–1936
Acknowledgment
National Centre for Enteropathogenic Bacteria (NENT), Lucerne: A. Burnens, P. Boerlin, and H. Hächler
Conflicts of interest
The authors declare that they have no conflicts of interest
Author information
Authors and Affiliations
Consortia
Corresponding author
Additional information
Swiss Pediatric Surveillance Unit Committee: Ch. Aebi, Berne (President); V. Bernet-Büttiker, Zurich; P. Hüppi, Geneva; B. Laubscher, Neuchâtel; Ch. Rudin, Basel; H. Zimmermann, Berne; D. Beeli, Berne.
Rights and permissions
About this article
Cite this article
Schifferli, A., von Vigier, R.O., Fontana, M. et al. Hemolytic-uremic syndrome in Switzerland: a nationwide surveillance 1997–2003. Eur J Pediatr 169, 591–598 (2010). https://doi.org/10.1007/s00431-009-1079-9
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00431-009-1079-9