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Epidemiology, clinical presentation, and pathophysiology of atypical and recurrent hemolytic uremic syndrome

Zimmerhackl, L Bernd ; Besbas, Nesir ; Jungraithmayr, Therese ; van de Kar, Nicole ; Karch, Helge ; Karpman, Diana LU orcid ; Landau, Daniel ; Loirat, Chantal ; Proesmans, Willem and Prufer, Friederike , et al. (2006) In Seminars in Thrombosis and Hemostasis 32(2). p.113-120
Abstract
Hemolytic uremic syndrome (HUS) includes a heterogeneous group of hemolytic disorders. Among the identified causes of HUS are infections, particularly infections with Shiga toxin-producing ESCHERICHIA COLI (STEC), complement disorders, and disorders interfering with the degradation of von Willebrand factor (VWF). Other causes for atypical HUS include the cobalamin metabolism; pregnancy/hemolysis, elevated liver enzymes, and low platelets syndrome (HELLP); drugs; and other disorders (e.g., systemic diseases appearing as HUS, such as systemic lupus erythematosus and rejection after transplantation). The group not related to STEC is often also called atypical HUS. Most of the occurrences of infectious HUS have only one episode. Recurrent... (More)
Hemolytic uremic syndrome (HUS) includes a heterogeneous group of hemolytic disorders. Among the identified causes of HUS are infections, particularly infections with Shiga toxin-producing ESCHERICHIA COLI (STEC), complement disorders, and disorders interfering with the degradation of von Willebrand factor (VWF). Other causes for atypical HUS include the cobalamin metabolism; pregnancy/hemolysis, elevated liver enzymes, and low platelets syndrome (HELLP); drugs; and other disorders (e.g., systemic diseases appearing as HUS, such as systemic lupus erythematosus and rejection after transplantation). The group not related to STEC is often also called atypical HUS. Most of the occurrences of infectious HUS have only one episode. Recurrent episodes (recurrent HUS) have strong relationships to diseases of the complement system. In these two subgroups the prognosis is poor, with severe renal insufficiency, together with the need for renal replacement therapy. Severe arterial hypertension is common. Treatment options are limited. To better define this group of patients, the European Society for Pediatric Nephrology supported an initiative to develop a European HUS registry. In this registry, 167 patients were acquired; 73 were female (43.8%). The year of onset of the disease ranged from 1974 to 2005. The prevalence of atypical HUS/recurrent HUS can be calculated as 3.3 per million child population (< 18 years). Underlying disorders included factor H, factor I, MCP-1, pneumococci, and von Willebrand factor disturbances. In 33 patients at least one renal transplantation was performed (total, 55 kidneys); 18% were successful and 73% demonstrated recurrence or thrombosis. Treatment options were plasma substitution or plasmapheresis. Despite continued efforts, transplantation is not recommended at present for these patients. Living-related transplantation should be abandoned. New therapeutic strategies are urgently needed. (Less)
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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Enterohemorrhagic Escherichia coli - complement system - von Willebrand factor - transplantation - plasma treatment - recurrence - hemolytic uremic syndrome
in
Seminars in Thrombosis and Hemostasis
volume
32
issue
2
pages
113 - 120
publisher
Georg Thieme Verlag KG
external identifiers
  • pmid:16575686
  • scopus:33645557561
ISSN
1098-9064
DOI
10.1055/s-2006-939767
language
English
LU publication?
yes
id
36abe477-a864-4bba-a0e7-a625cc15a33d (old id 1135236)
date added to LUP
2016-04-01 17:03:48
date last changed
2020-11-03 04:29:20
@article{36abe477-a864-4bba-a0e7-a625cc15a33d,
  abstract     = {Hemolytic uremic syndrome (HUS) includes a heterogeneous group of hemolytic disorders. Among the identified causes of HUS are infections, particularly infections with Shiga toxin-producing ESCHERICHIA COLI (STEC), complement disorders, and disorders interfering with the degradation of von Willebrand factor (VWF). Other causes for atypical HUS include the cobalamin metabolism; pregnancy/hemolysis, elevated liver enzymes, and low platelets syndrome (HELLP); drugs; and other disorders (e.g., systemic diseases appearing as HUS, such as systemic lupus erythematosus and rejection after transplantation). The group not related to STEC is often also called atypical HUS. Most of the occurrences of infectious HUS have only one episode. Recurrent episodes (recurrent HUS) have strong relationships to diseases of the complement system. In these two subgroups the prognosis is poor, with severe renal insufficiency, together with the need for renal replacement therapy. Severe arterial hypertension is common. Treatment options are limited. To better define this group of patients, the European Society for Pediatric Nephrology supported an initiative to develop a European HUS registry. In this registry, 167 patients were acquired; 73 were female (43.8%). The year of onset of the disease ranged from 1974 to 2005. The prevalence of atypical HUS/recurrent HUS can be calculated as 3.3 per million child population (&lt; 18 years). Underlying disorders included factor H, factor I, MCP-1, pneumococci, and von Willebrand factor disturbances. In 33 patients at least one renal transplantation was performed (total, 55 kidneys); 18% were successful and 73% demonstrated recurrence or thrombosis. Treatment options were plasma substitution or plasmapheresis. Despite continued efforts, transplantation is not recommended at present for these patients. Living-related transplantation should be abandoned. New therapeutic strategies are urgently needed.},
  author       = {Zimmerhackl, L Bernd and Besbas, Nesir and Jungraithmayr, Therese and van de Kar, Nicole and Karch, Helge and Karpman, Diana and Landau, Daniel and Loirat, Chantal and Proesmans, Willem and Prufer, Friederike and Rizzoni, Gianfranco and Taylor, Mark C},
  issn         = {1098-9064},
  language     = {eng},
  number       = {2},
  pages        = {113--120},
  publisher    = {Georg Thieme Verlag KG},
  series       = {Seminars in Thrombosis and Hemostasis},
  title        = {Epidemiology, clinical presentation, and pathophysiology of atypical and recurrent hemolytic uremic syndrome},
  url          = {http://dx.doi.org/10.1055/s-2006-939767},
  doi          = {10.1055/s-2006-939767},
  volume       = {32},
  year         = {2006},
}