A5054034610- Blood Coagulation and Thrombosis Mechanisms
- Venous Thromboembolism Diagnosis and Management
- Platelet Disorders and Treatments
- Hemophilia Treatment and Research
- Acute Myocardial Infarction Research
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Blood groups and transfusion
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Vascular anomalies and interventions
- Adolescent and Pediatric Healthcare
- Cardiovascular Issues in Pregnancy
- Blood disorders and treatments
- Erythrocyte Function and Pathophysiology
- Lipoproteins and Cardiovascular Health
- Childhood Cancer Survivors' Quality of Life
- Heparin-Induced Thrombocytopenia and Thrombosis
- Abdominal Trauma and Injuries
- Nuclear Structure and Function
- Traumatic Brain Injury and Neurovascular Disturbances
- Cerebral Palsy and Movement Disorders
- Vitamin K Research Studies
- Atrial Fibrillation Management and Outcomes
- Neonatal and fetal brain pathology
- Trauma, Hemostasis, Coagulopathy, Resuscitation
- Sepsis Diagnosis and Treatment
University of Münster
2007-2016
University Hospital Münster
2007-2016
Palmetto Hematology Oncology
2016
Universitätskinderklinik
2009-2013
Boston Children's Hospital
2010
Klinik für Schlafmedizin
2009
Hospital for Sick Children
2008
University Children's Hospital Zurich
2008
In-Q-Tel
2008
Temple Street Children's University Hospital
2007
Abstract Objective: Limited data are available on health‐related quality of life (HR‐QoL) in pediatric stroke survivors. The aim the present study was to assess HR‐QoL by self‐assessment and parent/proxy‐assessment children adolescents who survived a first episode. Methods: We investigated survivors (71 preschool [G1] 62 school children/adolescents [G2]) 169 healthy controls. assessed patients parents/proxies with generic KINDL‐R questionnaire exploring overall well‐being 6 subdimensions...
In patients with von Willebrand disease (VWD) replacement therapy factor VIII/von (VWF) concentrates is increasingly applied as prophylactic regimen. Since 2000, 82 consecutively enrolled clinically relevant bleeding episodes (spontaneous, peri- or postoperative) were diagnosed VWD [type 1: 42/82; type 2: 24/82; 3: 13/82; acquired: 3/82]. all patients, decision for initiating prophylaxis was based on a score > 2 prior to diagnosis, concomitant recurrent bleeds associated anaemia in on-demand...
The present multicenter cohort study of 107 pediatric PUPs was performed to determine whether the concomitant inheritance factor (F) V G1691A or F II G20210A mutation influences clinical expression severe hemophilia A (HA). Carriers FV and FII mutations had a significantly lower annual bleeding frequency (ABF) than non-carriers (p=0.012). Joint damage (Pettersson score) less in patients with thrombophilia (p=0.022). protective effect thrombophilic risk factors shown for ABF (OR [CIs]:...
Venous thromboembolism [TE] is a multifactorial disease, and protein S deficiency [PSD] constitutes major risk factor. In the present study prevalence of PSD clinical presentation at TE onset in cohort children reported. 367 unselected paediatric patients with (age 0.1-18 years) recruited between July 1996 December 2013, comprehensive thrombophilia screening was performed along recording anamnestic data. Thirty (8.2 %) derived from 27 families had PSD. Mean age first 14.5 years (range 0.1 to...
Summary Deficiency of antithrombin ( AT ), protein C PC ) or S PS constitutes a major risk factor for venous thromboembolism VTE ). Individuals at high recurrence who benefit from screening need to be identified. The primary study objective was determine the individual among children with first non‐central‐venous‐catheter‐associated respect their thrombophilia status and evaluate if clinical presentation onset differs between , deficiency versus no thrombophilia. We calculated absolute...
alpha(2)-Macroglobulin (alpha2MG) is a broad-spectrum protease inhibitor that known to neutralize alpha-thrombin, plasmin, and activated protein C, which suggests it has anticoagulant as well procoagulant properties. The present study was conducted evaluate the role of alpha2MG in children with venous thromboembolism [VTE: paradoxical embolism causing ischemic stroke (IS) or deep-vein thrombosis (DVT)].alpha2MG levels measured after acute VTE onset white patients were compared data obtained...
Patients with severe haemophilia A (HA) can either be treated by regular FVIII infusions twice or three times per week (prophylaxis), only in case of bleeding episodes (on-demand). Whereas prophylaxis reduces the number and may therefore prevent development haemophilic arthropathy, there is still a lot controversy surrounding recommendations on age dose at start prophylactic regimens. The present database study was performed to investigate role primary versus secondary HA children. outcome...
Treatment with exogenous von Willebrand factor (VWF) is indicated in patients disease (VWD) whom treatment 1-deamino-8-d-arginine vasopressin/desmopressin contraindicated. Wilate a new generation plasma-derived concentrate of native VWF and coagulation VIII (FVIII) (in physiological 1:1 ratio) developed for the VWD. This first study to report safety, efficacy vivo recovery (IVR) data from 15 paediatric less than 6 years age who received either prophylaxis, on-demand or surgical procedures...
Objectives . To investigate self-reported health-related quality of life (HrQoL) in children and adolescents with chronic medical conditions compared siblings/peers. Methods Group 1 (6 treatment centers) consisted 74 children/adolescents aged 8–16 years hereditary bleeding disorders (HBD), 12 siblings, 34 peers. 2 (one center) 70 stroke/transient ischemic attack, 14 72 HrQoL was assessed the “revised KINDer Lebensqualitätsfragebogen” (KINDL-R) questionnaire. Multivariate analyses within...
Summary Venous thromboembolism (VTE) is a rare disease that being increasingly diagnosed and recognized in paediatrics the past decade, usually as secondary complication of primary severe underlying diseases. Apart from acquired thrombophilic risk factors, such lupus anticoagulants, inherited thrombophilias (IT) have been established factors for venous thromboembolic events adults. In children with idiopathic VTE paediatric populations which thromboses were associated medical diseases, IT...
Linking the genetic background of patients with bleeding diathesis and altered platelet function remains challenging. We aimed to assess how a multiparameter microspot-based measurement thrombus formation under flow can help identify disorder. For this purpose, we studied 16 presenting and/or albinism suspected dysfunction 15 relatives. Genotyping revealed novel biallelic pathogenic variant in RASGRP2 (splice site c.240-1G>A), abrogating CalDAG-GEFI expression, compound heterozygosity...
Request for further standardization issues future studies in paediatric haemophilia patients – Rebuttal to “Including the life-time cumulative number of joint bleeds definition primary prophylaxis” -