Universität zu Köln

Holle, J., Lange-Sperandio, B., Mache, C., Oh, J., Pape, L., Schaefer, F., Vester, U., Weber, L. T. and Mueller, D. (2017). Hemolytic uremic syndrome in childhood and adolescence. Mon.schr. Kinderheilkd., 165 (11). S. 1005 - 1019. NEW YORK: SPRINGER. ISSN 1433-0474

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Abstract

Hemolytic uremic syndrome (HUS) is defined by the combination of microangiopathic hemolytic anemia, thrombocytopenia and acute functional impairment of the kidneys. The most common form in childhood is STEC-HUS caused by an infection with shiga toxin-producing bacteria leading to hemorrhagic enterocolitis. This results in HUS in approximately 10% of cases. Extrarenal symptoms might occur. The confirmation of the diagnosis is carried out by stool and serological diagnostics. There is no specific therapy available. Complement-mediated HUS gives rise to mutations, antibodies and trigger events, which lead to dysregulation of alternative complement pathways. Due to the specific treatment options with the C5 inhibitor eculizumab the prognosis has significantly improved. In rare cases HUS is caused by infections (e. g. Streptococcus pneumoniae) or other underlying conditions (e. g. cobalamin C deficiency). Due to the complexity of the diagnostics and therapy, treatment should be performed in a specialized pediatric nephrology center.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Holle, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Lange-Sperandio, B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Mache, C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Oh, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Pape, L. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schaefer, F. UNSPECIFIED UNSPECIFIED UNSPECIFIED Vester, U. UNSPECIFIED UNSPECIFIED UNSPECIFIED Weber, L. T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Mueller, D. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-212947
DOI:	10.1007/s00112-017-0331-z
Journal or Publication Title:	Mon.schr. Kinderheilkd.
Volume:	165
Number:	11
Page Range:	S. 1005 - 1019
Date:	2017
Publisher:	SPRINGER
Place of Publication:	NEW YORK
ISSN:	1433-0474
Language:	German
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language COMPLEMENT INHIBITOR ECULIZUMAB; ESCHERICHIA-COLI; THROMBOTIC MICROANGIOPATHIES Multiple languages Pediatrics Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/21294