A5032116456- Hemophilia Treatment and Research
- Platelet Disorders and Treatments
- Sepsis Diagnosis and Treatment
- Blood Coagulation and Thrombosis Mechanisms
- Respiratory Support and Mechanisms
- Intensive Care Unit Cognitive Disorders
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Electrolyte and hormonal disorders
- COVID-19 Clinical Research Studies
- Pneumonia and Respiratory Infections
- Cancer-related gene regulation
- Trauma, Hemostasis, Coagulopathy, Resuscitation
- Emergency and Acute Care Studies
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Genetics and Neurodevelopmental Disorders
- Peptidase Inhibition and Analysis
- Venous Thromboembolism Diagnosis and Management
- Antiplatelet Therapy and Cardiovascular Diseases
- Respiratory viral infections research
- Orthopaedic implants and arthroplasty
- Neurogenetic and Muscular Disorders Research
- Thermal Regulation in Medicine
- Pancreatitis Pathology and Treatment
- Helicobacter pylori-related gastroenterology studies
- Liver Disease and Transplantation
Erasmus MC
2011-2025
Erasmus University Rotterdam
2015-2020
Summary Pregnant women with bleeding disorders require specialised peripartum care to prevent postpartum haemorrhage ( PPH ). If third trimester coagulation factor levels are <0.50 IU mL −1 , prophylactic treatment is indicated and administered according international guidelines. However, optimal dose duration unknown may still occur. The aim of this study was investigate the outcome in von Willebrand disease VWD ) or haemophilia carriership treated current practice From period 2002–2011,...
Summary Introduction: Desmopressin is frequently used in patients with bleeding disorders because of its prohaemostatic effects. In recent years desmopressin use increased due to reported high incidence inhibitors mild haemophilia after clotting factor infusion and the rising costs concentrates. The safety frequency side effects have hardly been assessed well‐designed studies. Aim: We therefore prospectively evaluated a large unselected cohort disorder patients, who received test dose....
Patients with coronavirus disease 2019 (COVID-19)-related acute respiratory distress syndrome (ARDS) who lack clinical improvement are frequently treated high-dose corticosteroids (HDS). Since HDS is used to reduce hyperinflammation in these patients, levels of (pro-)inflammatory biomarkers after commencing treatment could be useful predicting mortality. This study aims evaluate biomarker over time, along their capacity predict retrospective cohort included patients COVID-19 ARDS the...
Abstract Background & Aims Cirrhosis frequently affects multiple components of hemostasis. Reversal the coagulopathy these patients is required in case bleeding episodes, or as prophylaxis before invasive procedures. Although 1‐deamino‐8‐D‐arginine vasopressin ( DDAVP ) widely used a pro‐hemostatic agent with cirrhosis, it unclear whether truly enhances hemostasis patients. Here we investigated hemostatic effects single bolus cirrhosis. Methods Ten cirrhosis (child B C) and ten mild...
Summary Desmopressin causes two- to six-fold increase of factor VIII (FVIII) in mild or moderate haemophilia A patients. However, responses are variable and little is known whether this associated with F8 gene mutation. The study objective was assess the relationship between mutation desmopressin response (absolute relative) determined 97 hemophilia Four amino acid changes (Arg2169His, Pro149Arg, Asn637Ser, Arg612Cys) a number other mutations leading an aberrant FVIII protein deficiency were...
Background Nonsevere haemophilia A (HA) patients can be treated with desmopressin. Response of factor VIII activity (FVIII:C) differs between and is difficult to predict. Objectives Our aims were describe FVIII:C response after desmopressin its reproducibility by population pharmacokinetic (PK) modelling. Patients Methods Retrospective data 128 nonsevere HA (age 7–75 years) receiving an intravenous or intranasal dose used. PK modelling was performed nonlinear mixed effect Reproducibility...
In hospitalized patients with COVID-19, the dosing and timing of corticosteroids vary widely. Low-dose dexamethasone therapy reduces mortality in requiring respiratory support, but it remains unclear how to treat when this fails. critically ill patients, high-dose are often administered as salvage late disease course, whereas earlier administration may be more beneficial preventing progression. Previous research has revealed that increased levels various biomarkers associated mortality,...
Introduction Due to interindividual variation in desmopressin response, non‐severe haemophilia A patients require testing prior therapeutic treatment. However, adequate response or frequency of blood sampling is not standardised international guidelines. Consequently, various definitions and protocols are currently applied. Interestingly, sustainability incorporated into these definitions. Aim To study rates a cohort using accepted This, order formulate standardised, uniform which includes...
Abstract Background Hemophilia B is an X‐linked recessive disorder caused by mutations in the F9 on Xq27.1. Mainly males are affected but about 20% of female carriers have clotting factor IX activity below 0.40 IU /ml and bleeding problems. Fragile‐X syndrome ( FMR 1 ) FRAXE AFF 2 well‐known causes intellectual disability. Simultaneous deletion both results severe In females phenotype more variable. We report a 19‐year‐old with disability long‐standing history. Methods A SNP array analysis...
Abstract Introduction Diagnosis, treatment monitoring and assessment of desmopressin effect in haemophilia A patients are performed by measurement factor VIII activity (FVIII). The two assays commonly applied the one‐stage assay chromogenic assay. Especially non‐severe A, discrepancies between these common. It is still unestablished which corresponds best with bleeding phenotype effect. Aim To correlate FVIII levels measured and, additionally, to compare before after administration. Method...
Abstract Background Corticosteroids could improve outcomes in patients with community-acquired pneumonia (CAP). However, we hypothesize that corticosteroid effectiveness varies among individual patients, resulting inconsistent and unclear clinical indication. Therefore, developed validated a predictive, causal model based on baseline characteristics to predict individualized treatment effects (ITEs) of corticosteroids mortality CAP. Methods We obtained patient data from six randomized...
<sec> <title>BACKGROUND</title> In hospitalized patients with COVID-19, the dosing and timing of corticosteroids vary widely. Low-dose dexamethasone therapy reduces mortality in requiring respiratory support, but it remains unclear how to treat when this fails. critically ill patients, high-dose are often administered as salvage late disease course, whereas earlier administration may be more beneficial preventing progression. Previous research has revealed that increased levels various...
The prediction of disease outcomes in COVID-19 patients the ICU is critical importance, and examination host gene expressions a promising tool. 29-host mRNA Inflam-matix-Severity-3b (IMX-SEV-3b) classifier has been reported to predict mortality emergency department surgical patients. accuracy IMX-SEV-3b predicting admitted yet unknown. Our aim was investigate In addition, we assessed predictive performance routinely measured biomarkers Sequential Organ Failure Assessment (SOFA) score as...