Universität zu Köln

Holle, Johannes ORCID: 0000-0001-8032-4096, Habbig, Sandra, Gratopp, Alexander ORCID: 0000-0001-5879-2918, Mauritsch, Anna, Mueller, Dominik and Thumfart, Julia (2021). Complement activation in children with Streptococcus pneumoniae associated hemolytic uremic syndrome. Pediatr. Nephrol., 36 (5). S. 1311 - 1316. NEW YORK: SPRINGER. ISSN 1432-198X

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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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Holle, Johannes UNSPECIFIED orcid.org/0000-0001-8032-4096 UNSPECIFIED Habbig, Sandra UNSPECIFIED UNSPECIFIED UNSPECIFIED Gratopp, Alexander UNSPECIFIED orcid.org/0000-0001-5879-2918 UNSPECIFIED Mauritsch, Anna UNSPECIFIED UNSPECIFIED UNSPECIFIED Mueller, Dominik UNSPECIFIED UNSPECIFIED UNSPECIFIED Thumfart, Julia UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-581070
DOI:	10.1007/s00467-021-04952-w
Journal or Publication Title:	Pediatr. Nephrol.
Volume:	36
Number:	5
Page Range:	S. 1311 - 1316
Date:	2021
Publisher:	SPRINGER
Place of Publication:	NEW YORK
ISSN:	1432-198X
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language Pediatrics; Urology & Nephrology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/58107