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Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome

Ariceta, Gema; Besbas, Nesrin; Johnson, Sally; Karpman, Diana LU ; Landau, Daniel; Licht, Christoph; Loirat, Chantal; Pecoraro, Carmine; Taylor, C. Mark and Van de Kar, Nicole, et al. (2009) In Pediatric Nephrology 24(4). p.687-696
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... (More)
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. (Less)
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keywords
purpura, Hemolytic uremic syndrome, Complement factor I, Complement factor B, Thrombotic thrombocytopenic, Atypical HUS, member 13 (ADAMTS13), Plasmapheresis, Complement C3, A disintegrin and metalloproteinase with, co-factor protein MCP (CD46), Membrane, Enterohemorrhagic Escherichia coli, Complement factor H, a thrombospondin type 1 motif
in
Pediatric Nephrology
volume
24
issue
4
pages
687 - 696
publisher
Springer
external identifiers
  • wos:000263674300001
  • scopus:61549117207
ISSN
1432-198X
DOI
10.1007/s00467-008-0964-1
language
English
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yes
id
898f9612-5b49-4dd3-8afc-4e88488549f7 (old id 1370946)
date added to LUP
2009-05-04 11:05:27
date last changed
2017-12-10 04:00:42
@misc{898f9612-5b49-4dd3-8afc-4e88488549f7,
  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.},
  author       = {Ariceta, Gema and Besbas, Nesrin and Johnson, Sally and Karpman, Diana and Landau, Daniel and Licht, Christoph and Loirat, Chantal and Pecoraro, Carmine and Taylor, C. Mark and Van de Kar, Nicole and VandeWalle, Johan and Zimmerhackl, Lothar B.},
  issn         = {1432-198X},
  keyword      = {purpura,Hemolytic uremic syndrome,Complement factor I,Complement factor B,Thrombotic thrombocytopenic,Atypical HUS,member 13 (ADAMTS13),Plasmapheresis,Complement C3,A disintegrin and metalloproteinase with,co-factor protein MCP (CD46),Membrane,Enterohemorrhagic Escherichia coli,Complement factor H,a thrombospondin type 1 motif},
  language     = {eng},
  number       = {4},
  pages        = {687--696},
  publisher    = {Springer},
  series       = {Pediatric Nephrology},
  title        = {Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome},
  url          = {http://dx.doi.org/10.1007/s00467-008-0964-1},
  volume       = {24},
  year         = {2009},
}