A5103198302- Complement system in diseases
- Blood groups and transfusion
- Liver Disease and Transplantation
- Platelet Disorders and Treatments
- Adenosine and Purinergic Signaling
- Organ Transplantation Techniques and Outcomes
- Erythrocyte Function and Pathophysiology
- Renal Diseases and Glomerulopathies
- Pneumonia and Respiratory Infections
- Streptococcal Infections and Treatments
- Neonatal and Maternal Infections
Mario Negri Institute for Pharmacological Research
2017
Istituti di Ricovero e Cura a Carattere Scientifico
2017
Kantonsspital Winterthur
2017
University Children's Hospital Zurich
2013-2014
Universitätskinderklinik
2011
Abstract von Willebrand factor (VWF), a multimeric protein with central role in hemostasis, has been shown to interact complement components. However, results are contrasting and inconclusive. By studying 20 patients congenital thrombotic thrombocytopenic purpura (cTTP) who cannot cleave VWF multimers because of genetic ADAMTS13 deficiency, we investigated the mechanism through which modulates its pathophysiological implications for human diseases. Using assays ex vivo serum-induced C3 C5b-9...
Pneumococcal hemolytic uremic syndrome (HUS) in children is caused by infections with Streptococcus pneumoniae. Because endothelial cell damage a hallmark of HUS, we studied how HUS-inducing pneumococci derived from infant HUS patients during the acute phase disrupt layer. efficiently bound human plasminogen. These clinical isolates plasminogen via bacterial surface proteins Tuf and PspC. When activated to plasmin at surface, active protease degraded fibrinogen cleaved C3b. Here, show that...
Streptococcus pneumoniae serotype 3 strains are highly resistant to opsonophagocytosis due recruitment of the complement inhibitor Factor H via Hic, a member pneumococcal surface protein C (PspC) family. In this study, we demonstrated that Hic also interacts with vitronectin, fluid-phase regulator involved in haemostasis, angiogenesis, and terminal cascade as well component extracellular matrix. Blocking by specific antiserum or genetic deletion significantly reduced binding soluble...
Hintergrund: Das Rezidivrisiko bei Kindern mit aHUS nach Nierentransplantation ist sehr hoch. Guidelines für ein mutation-assoziiertes Management zur Vermeidung von Rezidiven eines sind noch nicht etabliert. Die isolierte führt den meisten Formen zu konsekutivem Transplantatverlust. Patient: Ein 8 ½ jähriger Knabe basierend auf einer MCP und CFI Mutation (für beide heterozygot, Eltern gesunde Träger je Mutation) wurde 4 Jahre peritonealdialysiert im Alter 5 Jahren nierentransplantiert...