Klinik und Poliklinik für Kinder- und Jugendmedizin
 Universitätsmedizin Leipzig

Clinical immunology

Projects

1. Plasminogen deficiency
Inherited severe hypoplasminogenaemia is a multisystemic disorder leading to deficient extravascular fibrinolysis. As a clinical consequence wound healing capacity of mucous membranes is markedly impaired leading to ligneous conjunctivitis and several other manifestations. during the last 15 years our group identified in >70% of patients various mutations in the plasminogen gene. The most common genetic alteration was a K19E mutation found in 34% of patients. Transient in vitro expression of PLG mutants R134K, delK212, R216H, P285T, P285A, T319_N320insN, and R776H in transfected COS-7 cells revealed significantly impaired secretion and increased degradation of PLG. These results demonstrate impaired secretion of mutant PLG proteins as a common molecular pathomechanism in type I PLG deficiency.

Plasminogenmangel

Publications

Kobelt L, Klammt J, Tefs K, Schuster V. Estrogen modulates plasminogen promoter activity. Biochem Biophys Res Commun. 2013 Aug 16;438(1):110-5

Klammt J, Kobelt L, et al. Identification of three novel plasminogen (PLG) gene mutations in a series of 23 patients with low PLG activity. Thromb Haemost. 2011;105:454-460

Further publications: see pubmed


2. Rotavirus studies
Viral gastroenteritis is one of the leading causes of morbidity in young children worldwide.
Since licensing in 2006, two oral attenuated live vaccines for rotavirus (RV) were introduced in many countries worldwide. Those are the pentavalent RV5 (Rotateq®, Merck & Co.) and the monovalent RV1 (Rotarix®, GlaxoSmithKline, London, UK). In 2009 the WHO recommended to include universal RV vaccination into all national immunization programs. Since 2007 we participated in several rotavirus vaccination studies (efficacy, immunogenicity, postmarketing). Our data suggest that rotavirus vaccination is effective in preventing RV hospitalization in Germany. There is evidence that both vaccines are similarly effective in preventing rotavirus associated morbidity requiring ambulatory medical care. We could not detect substantial side effects.

Publications

Uhlig U, Kostev K, Schuster V, Koletzko S, Uhlig HH. Impact of Rotavirus Vaccination in Germany: Rotavirus Surveillance, Hospitalization, Side Effects and Comparison of Vaccines. Pediatr Infect Dis J. 2014 Jun 6. [Epub ahead of print]

Huppertz HI*, Borte M*, Schuster V* (*contributed equally), Giaquinto C, Vesikari T. Report of the Third European Expert Meeting on Rotavirus Vaccination: Progress in rotavirus universal mass vaccination in Europe. Vaccine, 2014 [Epub ahead of print]

Vesikari T, Prymula R, Schuster V, Tejedor JC, Cohen R, Bouckenooghe A, Damaso S, Han HH. Efficacy and immunogenicity of a live-attenuated human rotavirus vaccine in breast-fed and formula-fed European infants. Pediatr Infect Dis J. 2012 May;31(5):509-13

Further publications: see pubmed


Scientific conferences

Third European Expert Meeting on Rotavirus Vaccination. 23-24 April 2013, Leipzig, Germany; chairs: M.Borte, HI Huppertz, V.Schuster
http://www.rotavirus-leipzig.de/

33rd Meeting of the European Society for Paediatric Infectious Diseases (ESPID), May 12 - 15, 2015, Leipzig, chairs: M.Borte, V.Schuster
http://espid2015.kenes.com/meeting-information/welcome-message


3. X-linked lymphoproliferative syndromes and related autosomal recessive disorders
During the last 15 years several EBV-associated lymphoproliferative syndrome have been identified and further characterized: XLP-1 (SH2D1A deficiency), XLP-2 (XIAP deficiency), ITK deficiency, CD27 deficiency and others. The prognosis is serious. Reduced Intensity Conditioning HSCT may b a therapeutic option.

X-chromosomale lymphoproliferative Erkrankungen

Publications

Schuster V, Latour S (2014) X-linked lymphoproliferative diseases, Chapter 44; in: Ochs HD, Smith CIE, Puck J (eds) Primary Immunodeficiency Diseases, a Molecular and Genetic Approach, 3rd edition, Oxford University Press, ISBN 978-0-19538983-8 (hardback), pp 557-579

Horn PC, Belohradsky BH, Urban C, Weber-Mzell D, Meindl A, Schuster V. Two new families with X-linked inhibitor of apoptosis deficiency and a review of all 26 published cases. J Allergy Clin Immunol. 2011 Feb;127(2):544-6

Further publications: see pubmed

Group

Kobelt L, Klammt J, Ziegler M, Tefs K (former), Schuster V

Contact

Prof. Dr. Volker Schuster
Hospital for Children and Adolescents
University of Leipzig
Liebigstrasse 20a, D-4103 Leipzig, Germany
Phone: +49 341 97 26242
Fax: +49 341 97 26229
E-mail: Volker.Schuster@medizin.uni-leipzig.de


 
Letzte Änderung: 22.10.2014, 12:24 Uhr
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