Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome.
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.
Ariceta
Gema
G
Besbas
Nesrin
N
Johnson
Sally
S
Karpman
Diana
D
Landau
Daniel
D
Licht
Christoph
C
Loirat
Chantal
C
Pecoraro
Carmine
C
Taylor
C Mark
CM
Van de Kar
Nicole
N
Vandewalle
Johan
J
Zimmerhackl
Lothar B
LB
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
J Pediatr. 2001 Mar;138(3):412-7
11241053
Semin Thromb Hemost. 2005 Dec;31(6):709-16
16388422
Pediatr Nephrol. 2007 Jun;22(6):874-80
17295030
Lancet. 1984 Dec 8;2(8415):1337
6150343
Am J Kidney Dis. 2006 Feb;47(2):e27-30
16431247
Arch Dis Child. 2007 Apr;92(4):298-303
16905566
Br J Haematol. 2004 Jul;126(1):11-28
15198728
Przegl Lek. 2006;63 Suppl 3:223-5
16898537
J Clin Microbiol. 2000 Mar;38(3):1023-31
10698990
J Infect Dis. 2002 Aug 15;186(4):493-500
12195376
Pediatr Nephrol. 2006 Nov;21(11):1769-71
16909242
Kidney Int. 2006 Aug;70(3):423-31
16775594
Mol Immunol. 2007 Sep;44(16):3889-900
17768107
Pediatr Nephrol. 2003 Sep;18(9):952-5
12836093
Am J Kidney Dis. 2001 Oct;38(4):E22
11576909
Transfusion. 2007 Oct;47(10):1837-42
17880609
Br J Haematol. 2003 Feb;120(4):556-73
12588343
Pediatr Nephrol. 2001 Jul;16(7):554-6
11465803
J Pediatr. 1993 Apr;122(4):532-7
8463896
Proc Natl Acad Sci U S A. 2007 Jan 2;104(1):240-5
17182750
Am J Kidney Dis. 2005 Feb;45(2):415-21
15685522
Nephrol Dial Transplant. 2002 Apr;17(4):684-5
11917071
Blood. 2006 Aug 15;108(4):1267-79
16621965
Pediatr Pathol. 1983 Oct-Dec;1(4):409-13
6687290
Arch Dis Child. 1990 Jul;65(7):716-21
2201261
Hematol Oncol Clin North Am. 1996 Apr;10(2):485-97
8707765
J Am Soc Nephrol. 2007 Aug;18(8):2392-400
17599974
Pediatr Nephrol. 2004 Aug;19(8):908-11
15206027
Hum Mutat. 2006 Jan;27(1):21-30
16281287
PLoS One. 2007 Oct 10;2(10):e1024
17925872
J Am Soc Nephrol. 2005 Feb;16(2):555-63
15590760
Pediatrics. 1996 Oct;98(4 Pt 1):649-58
8885941
Transpl Int. 2000;13(6):443-7
11140243