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Clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study from 1999–2008
BACKGROUND: Hemolytic-uremic syndrome (HUS) is a clinical triad of microangiopathic hemolytic anemia, impaired renal function and thrombocytopenia, primarily affecting pre-school-aged children. HUS can be classified into diarrhea-associated HUS (D(+)HUS), usually caused by Shiga toxin-producing Esch...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4906913/ https://www.ncbi.nlm.nih.gov/pubmed/27297224 http://dx.doi.org/10.1186/s12879-016-1627-7 |
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author | Jenssen, Gaute Reier Vold, Line Hovland, Eirik Bangstad, Hans-Jacob Nygård, Karin Bjerre, Anna |
author_facet | Jenssen, Gaute Reier Vold, Line Hovland, Eirik Bangstad, Hans-Jacob Nygård, Karin Bjerre, Anna |
author_sort | Jenssen, Gaute Reier |
collection | PubMed |
description | BACKGROUND: Hemolytic-uremic syndrome (HUS) is a clinical triad of microangiopathic hemolytic anemia, impaired renal function and thrombocytopenia, primarily affecting pre-school-aged children. HUS can be classified into diarrhea-associated HUS (D(+)HUS), usually caused by Shiga toxin-producing Escherichia coli (STEC), and non-diarrhea-associated HUS (D(−)HUS), both with potentially serious acute and long-term complications. Few data exists on the clinical features and long-term outcome of HUS in Norway. The aim of this paper was to describe these aspects of HUS in children over a 10-year period. METHODS: We retrospectively collected data on clinical features, therapeutic interventions and long-term aspects directly from medical records of all identified HUS cases <16 years of age admitted to Norwegian pediatric departments from 1999 to 2008. Cases of D(+)HUS and D(−)HUS are described separately, but no comparative analyses were possible due to small numbers. Descriptive statistics are presented in proportions and median values with ranges, and/or summarized in text. RESULTS: Forty seven HUS cases were identified; 38 D(+)HUS and nine D(−)HUS. Renal complications were common; in the D(+)HUS and D(−)HUS group, 29/38 and 5/9 developed oligoanuria, 22/38 and 3/9 needed dialysis, with hemodialysis used most often in both groups, and plasma infusion(s) were utilized in 6/38 and 4/9 patients, respectively. Of extra-renal complications, neurological complications occurred in 9/38 and 2/9, serious gastrointestinal complications in 6/38 and 1/9, respiratory complications in 10/38 and 2/9, and sepsis in 11/38 and 3/9 cases, respectively. Cardiac complications were seen in two D(+)HUS cases. In patients where data on follow up ≥1 year after admittance were available, 8/21 and 4/7 had persistent proteinuria and 5/19 and 4/5 had persistent hypertension in the D(+)HUS and D(−)HUS group, respectively. Two D(+)HUS and one D(−)HUS patient were diagnosed with chronic kidney disease and one D(+)HUS patient required a renal transplantation. Two D(+)HUS patients died in the acute phase (death rate; 5 %). CONCLUSIONS: The HUS cases had a high rate of complications and sequelae, including renal, CNS-related, cardiac, respiratory, serious gastrointestinal complications and sepsis, consistent with other studies. This underlines the importance of attention to extra-renal manifestations in the acute phase and in renal long-term follow-up of HUS patients. |
format | Online Article Text |
id | pubmed-4906913 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-49069132016-06-15 Clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study from 1999–2008 Jenssen, Gaute Reier Vold, Line Hovland, Eirik Bangstad, Hans-Jacob Nygård, Karin Bjerre, Anna BMC Infect Dis Research Article BACKGROUND: Hemolytic-uremic syndrome (HUS) is a clinical triad of microangiopathic hemolytic anemia, impaired renal function and thrombocytopenia, primarily affecting pre-school-aged children. HUS can be classified into diarrhea-associated HUS (D(+)HUS), usually caused by Shiga toxin-producing Escherichia coli (STEC), and non-diarrhea-associated HUS (D(−)HUS), both with potentially serious acute and long-term complications. Few data exists on the clinical features and long-term outcome of HUS in Norway. The aim of this paper was to describe these aspects of HUS in children over a 10-year period. METHODS: We retrospectively collected data on clinical features, therapeutic interventions and long-term aspects directly from medical records of all identified HUS cases <16 years of age admitted to Norwegian pediatric departments from 1999 to 2008. Cases of D(+)HUS and D(−)HUS are described separately, but no comparative analyses were possible due to small numbers. Descriptive statistics are presented in proportions and median values with ranges, and/or summarized in text. RESULTS: Forty seven HUS cases were identified; 38 D(+)HUS and nine D(−)HUS. Renal complications were common; in the D(+)HUS and D(−)HUS group, 29/38 and 5/9 developed oligoanuria, 22/38 and 3/9 needed dialysis, with hemodialysis used most often in both groups, and plasma infusion(s) were utilized in 6/38 and 4/9 patients, respectively. Of extra-renal complications, neurological complications occurred in 9/38 and 2/9, serious gastrointestinal complications in 6/38 and 1/9, respiratory complications in 10/38 and 2/9, and sepsis in 11/38 and 3/9 cases, respectively. Cardiac complications were seen in two D(+)HUS cases. In patients where data on follow up ≥1 year after admittance were available, 8/21 and 4/7 had persistent proteinuria and 5/19 and 4/5 had persistent hypertension in the D(+)HUS and D(−)HUS group, respectively. Two D(+)HUS and one D(−)HUS patient were diagnosed with chronic kidney disease and one D(+)HUS patient required a renal transplantation. Two D(+)HUS patients died in the acute phase (death rate; 5 %). CONCLUSIONS: The HUS cases had a high rate of complications and sequelae, including renal, CNS-related, cardiac, respiratory, serious gastrointestinal complications and sepsis, consistent with other studies. This underlines the importance of attention to extra-renal manifestations in the acute phase and in renal long-term follow-up of HUS patients. BioMed Central 2016-06-13 /pmc/articles/PMC4906913/ /pubmed/27297224 http://dx.doi.org/10.1186/s12879-016-1627-7 Text en © The Author(s). 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Research Article Jenssen, Gaute Reier Vold, Line Hovland, Eirik Bangstad, Hans-Jacob Nygård, Karin Bjerre, Anna Clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study from 1999–2008 |
title | Clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study from 1999–2008 |
title_full | Clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study from 1999–2008 |
title_fullStr | Clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study from 1999–2008 |
title_full_unstemmed | Clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study from 1999–2008 |
title_short | Clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study from 1999–2008 |
title_sort | clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in norwegian children: a nationwide retrospective study from 1999–2008 |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4906913/ https://www.ncbi.nlm.nih.gov/pubmed/27297224 http://dx.doi.org/10.1186/s12879-016-1627-7 |
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