dc.contributor.author
Holle, Johannes
dc.contributor.author
Habbig, Sandra
dc.contributor.author
Gratopp, Alexander
dc.contributor.author
Mauritsch, Anna
dc.contributor.author
Müller, Dominik
dc.contributor.author
Thumfart, Julia
dc.date.accessioned
2023-07-18T12:22:34Z
dc.date.available
2023-07-18T12:22:34Z
dc.identifier.uri
https://refubium.fu-berlin.de/handle/fub188/40149
dc.identifier.uri
http://dx.doi.org/10.17169/refubium-39871
dc.description.abstract
Background: Hemolytic uremic syndrome caused by invasive pneumococcal disease (P-HUS) is rare in children and adolescents, but accompanied by high mortality in the acute phase and complicated by long-term renal sequelae. Abnormalities in the alternative complement pathway may additionally be contributing to the course of the disease but also to putative treatment options.
Methods: Retrospective study to assess clinical course and laboratory data of the acute phase and outcome of children with P-HUS.
Results: We report on seven children (median age 12 months, range 3-28 months) diagnosed with P-HUS. Primary organ manifestation was meningitis in four and pneumonia in three patients. All patients required dialysis which could be discontinued in five of them after a median of 25 days. In two patients, broad functional and genetic complement analysis was performed and revealed alternative pathway activation and risk haplotypes in both. Three patients were treated with the complement C5 inhibitor eculizumab. During a median follow-up time of 11.3 years, one patient died due to infectious complications after transplantation. Two patients showed no signs of renal sequelae.
Conclusions: Although pathophysiology in P-HUS remains as yet incompletely understood, disordered complement regulation seems to provide a clue to additional insights for pathology, diagnosis, and even targeted treatment.
en
dc.rights.uri
https://creativecommons.org/licenses/by/4.0/
dc.subject
Hemolytic uremic syndrome
en
dc.subject
Streptococcus pneumonia
en
dc.subject.ddc
600 Technik, Medizin, angewandte Wissenschaften::610 Medizin und Gesundheit::610 Medizin und Gesundheit
dc.title
Complement activation in children with Streptococcus pneumoniae associated hemolytic uremic syndrome
dc.type
Wissenschaftlicher Artikel
dcterms.bibliographicCitation.doi
10.1007/s00467-021-04952-w
dcterms.bibliographicCitation.journaltitle
Pediatric Nephrology
dcterms.bibliographicCitation.number
5
dcterms.bibliographicCitation.originalpublishername
Springer Nature
dcterms.bibliographicCitation.pagestart
1311
dcterms.bibliographicCitation.pageend
1315
dcterms.bibliographicCitation.volume
36
refubium.affiliation
Charité - Universitätsmedizin Berlin
refubium.funding
Springer Nature DEAL
refubium.resourceType.isindependentpub
no
dcterms.accessRights.openaire
open access
dcterms.bibliographicCitation.pmid
33538911
dcterms.isPartOf.issn
0931-041X
dcterms.isPartOf.eissn
1432-198X