title: Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome.
abstract: This guideline for the investigation and initial treatment of atypical hemolytic uremic syndrome (HUS) is intended to offer an approach based on opinion, as evidence is lacking. It builds on the current ability to identify the etiology of specific diagnostic sub-groups of HUS. HUS in children is mostly due to infection, enterohemorrhagic Escherichia coli (EHEC), Shigella dysenteriae type 1 in some geographic regions, and invasive Streptococcus pneumoniae. These sub-groups are relatively straightforward to diagnose. Their management, which is outside the remit of this guideline, is related to control of infection where that is necessary and supportive measures for the anemia and acute renal failure. A thorough investigation of the remainder of childhood HUS cases, commonly referred to as "atypical" HUS, will reveal a risk factor for the syndrome in approximately 60% of cases. Disorders of complement regulation are, numerically, the most important. The outcome for children with atypical HUS is poor, and, because of the rarity of these disorders, clinical experience is scanty. Some cases of complement dysfunction appear to respond to plasma therapy. The therapeutic part of this guideline is the consensus of the contributing authors and is based on limited information from uncontrolled studies. The guideline proposes urgent and empirical plasmapheresis replacement with whole plasma fraction for the first month after diagnosis. This should only be undertaken in specialized pediatric nephrology centers where appropriate medical and nursing skills are available. The guideline includes defined terminology and audit points so that the early clinical effectiveness of the strategy can be evaluated.
authors.forename: Gema
authors.initials: G
authors.lastname: Ariceta
authors.forename: Nesrin
authors.initials: N
authors.lastname: Besbas
authors.forename: Sally
authors.initials: S
authors.lastname: Johnson
authors.forename: Diana
authors.initials: D
authors.lastname: Karpman
authors.forename: Daniel
authors.initials: D
authors.lastname: Landau
authors.forename: Christoph
authors.initials: C
authors.lastname: Licht
authors.forename: Chantal
authors.initials: C
authors.lastname: Loirat
authors.forename: Carmine
authors.initials: C
authors.lastname: Pecoraro
authors.forename: C Mark
authors.initials: CM
authors.lastname: Taylor
authors.forename: Nicole
authors.initials: N
authors.lastname: Van de Kar
authors.forename: Johan
authors.initials: J
authors.lastname: Vandewalle
authors.forename: Lothar B
authors.initials: LB
authors.lastname: Zimmerhackl
mesh_terms: D002648:Child; D002675:Child, Preschool; D003967:Diarrhea / Q000175:diagnosis* / Q000382:microbiology; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis* / Q000382:microbiology / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D009780:Occult Blood; D010956:Plasmapheresis
references.citation: J Pediatr. 2001 Mar;138(3):412-7
references.pmid: 11241053
references.citation: Semin Thromb Hemost. 2005 Dec;31(6):709-16
references.pmid: 16388422
references.citation: Pediatr Nephrol. 2007 Jun;22(6):874-80
references.pmid: 17295030
references.citation: Lancet. 1984 Dec 8;2(8415):1337
references.pmid: 6150343
references.citation: Am J Kidney Dis. 2006 Feb;47(2):e27-30
references.pmid: 16431247
references.citation: Arch Dis Child. 2007 Apr;92(4):298-303
references.pmid: 16905566
references.citation: Br J Haematol. 2004 Jul;126(1):11-28
references.pmid: 15198728
references.citation: Przegl Lek. 2006;63 Suppl 3:223-5
references.pmid: 16898537
references.citation: J Clin Microbiol. 2000 Mar;38(3):1023-31
references.pmid: 10698990
references.citation: J Infect Dis. 2002 Aug 15;186(4):493-500
references.pmid: 12195376
references.citation: Pediatr Nephrol. 2006 Nov;21(11):1769-71
references.pmid: 16909242
references.citation: Kidney Int. 2006 Aug;70(3):423-31
references.pmid: 16775594
references.citation: Mol Immunol. 2007 Sep;44(16):3889-900
references.pmid: 17768107
references.citation: Pediatr Nephrol. 2003 Sep;18(9):952-5
references.pmid: 12836093
references.citation: Am J Kidney Dis. 2001 Oct;38(4):E22
references.pmid: 11576909
references.citation: Transfusion. 2007 Oct;47(10):1837-42
references.pmid: 17880609
references.citation: Br J Haematol. 2003 Feb;120(4):556-73
references.pmid: 12588343
references.citation: Pediatr Nephrol. 2001 Jul;16(7):554-6
references.pmid: 11465803
references.citation: J Pediatr. 1993 Apr;122(4):532-7
references.pmid: 8463896
references.citation: Proc Natl Acad Sci U S A. 2007 Jan 2;104(1):240-5
references.pmid: 17182750
references.citation: Am J Kidney Dis. 2005 Feb;45(2):415-21
references.pmid: 15685522
references.citation: Nephrol Dial Transplant. 2002 Apr;17(4):684-5
references.pmid: 11917071
references.citation: Blood. 2006 Aug 15;108(4):1267-79
references.pmid: 16621965
references.citation: Pediatr Pathol. 1983 Oct-Dec;1(4):409-13
references.pmid: 6687290
references.citation: Arch Dis Child. 1990 Jul;65(7):716-21
references.pmid: 2201261
references.citation: Hematol Oncol Clin North Am. 1996 Apr;10(2):485-97
references.pmid: 8707765
references.citation: J Am Soc Nephrol. 2007 Aug;18(8):2392-400
references.pmid: 17599974
references.citation: Pediatr Nephrol. 2004 Aug;19(8):908-11
references.pmid: 15206027
references.citation: Hum Mutat. 2006 Jan;27(1):21-30
references.pmid: 16281287
references.citation: PLoS One. 2007 Oct 10;2(10):e1024
references.pmid: 17925872
references.citation: J Am Soc Nephrol. 2005 Feb;16(2):555-63
references.pmid: 15590760
references.citation: Pediatrics. 1996 Oct;98(4 Pt 1):649-58
references.pmid: 8885941
references.citation: Transpl Int. 2000;13(6):443-7
references.pmid: 11140243