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View article: Advancements in High-Resolution Computed Tomography: Revolutionising Bone Health Micro-Research
Advancements in High-Resolution Computed Tomography: Revolutionising Bone Health Micro-Research Open
Bone disorders such as osteoporosis, osteopenia, and osteoarthritis affect millions worldwide, creating an urgent need for earlier and more accurate assessment of bone health. High-resolution imaging has transformed this field: micro-compu…
View article: “Diagnosis of Inherited Platelet Disorders”: Update of the Interdisciplinary S2k-Guideline [] of the Permanent Pediatric Commission of the Society of Thrombosis and Haemostasis Research (GTH e.V.)
“Diagnosis of Inherited Platelet Disorders”: Update of the Interdisciplinary S2k-Guideline [] of the Permanent Pediatric Commission of the Society of Thrombosis and Haemostasis Research (GTH e.V.) Open
Inherited platelet disorders (IPD) are a heterogeneous group of diseases causing bleeding, which are often challenging to diagnose. To improve the diagnostic process for these disorders, the ThromKidplus study group of the Permanent Pediat…
View article: Reduced Bone Quality of Sacrum and Lumbal Vertebrae Spongiosa in Toll-like Receptor 2- and Toll-like Receptor 4-Knockout Mice: A Blinded Micro-Computerized Analysis
Reduced Bone Quality of Sacrum and Lumbal Vertebrae Spongiosa in Toll-like Receptor 2- and Toll-like Receptor 4-Knockout Mice: A Blinded Micro-Computerized Analysis Open
Toll-like receptors (TLRs) are pivotal in modulating immune responses and have been implicated in bone remodeling. This in vivo study investigates the impact of TLR2 and TLR4 signaling on trabecular bone structure using micro-computed tomo…
View article: The Diagnostic Assessment of Platelet Function Defects - Part 2: Update on Platelet Disorders
The Diagnostic Assessment of Platelet Function Defects - Part 2: Update on Platelet Disorders Open
Congenital platelet disorders are rare and targeted treatment is usually not possible. Inherited platelet function disorders (iPFDs) can affect surface receptors and multiple platelet responses such as defects of platelet granules, signal …
View article: The Diagnostic Assessment of Inherited Platelet Function Defects - Part 1: An Overview of the Diagnostic Approach and Laboratory Methods
The Diagnostic Assessment of Inherited Platelet Function Defects - Part 1: An Overview of the Diagnostic Approach and Laboratory Methods Open
In this article, our goal is to offer an introduction and overview of the diagnostic approach to inherited platelet function defects (iPFDs) for clinicians and laboratory personnel who are beginning to engage in the field. We describe the …
View article: Leitlinie der Gesellschaft für Thrombose- und Hämostaseforschung (GTH) zur Struktur- und Prozessqualität von Hämophilie-Zentren
Leitlinie der Gesellschaft für Thrombose- und Hämostaseforschung (GTH) zur Struktur- und Prozessqualität von Hämophilie-Zentren Open
Since the 1970s, specialized hemophilia centers have been established to optimize the complex and costly treatment of patients with severe bleeding disorders. In 2019, the first GTH guidelines on the structural and process quality of hemop…
View article: Murine vs. Human Osteoblast Responses to Coagulation and Inflammatory Factors: Reconsidering the Use of Animal Models in Hemophilia A Research
Murine vs. Human Osteoblast Responses to Coagulation and Inflammatory Factors: Reconsidering the Use of Animal Models in Hemophilia A Research Open
Background/Objectives: Hemophilia A is associated with frequent bleeding episodes, joint damage, and reduced bone mineral density (BMD). The role of coagulation factors and inflammatory cytokines on bone metabolism, particularly on osteobl…
View article: Update on the Use of Thrombopoietin-Receptor Agonists in Pediatrics
Update on the Use of Thrombopoietin-Receptor Agonists in Pediatrics Open
This review summarizes the rationale and current data on the use of thrombopoietin receptor agonists (TPO-RAs) for treating severe thrombocytopenia in infants, children, and adolescents. It focuses on substances that have been approved by …
View article: Long-Term Comparison of Two- and Three-Dimensional Computed Tomography Analyses of Cranial Bone Defects in Severe Parietal Thinning
Long-Term Comparison of Two- and Three-Dimensional Computed Tomography Analyses of Cranial Bone Defects in Severe Parietal Thinning Open
Parietal thinning was detected in a 72-year-old with recurrent headaches. Quantification of bone loss was performed applying two- and three-dimensional methods using computerized tomographies. Two-dimensional methods provided accurate meas…
View article: Loss or Dilution—A New Diagnostic Method to Assess the Impact of Dilution on Standard Laboratory Parameters
Loss or Dilution—A New Diagnostic Method to Assess the Impact of Dilution on Standard Laboratory Parameters Open
Intraoperative fluid therapy is regularly used in patients undergoing cardiac surgery procedures with cardiopulmonary bypass (CPB). Although fluid administration has several advantages, it unavoidably leads to hemodilution. The hemodilutio…
View article: Progress in Hemostasis (Part 1): Improved Management of Inherited Platelet Disorders: Reality or Illusion?
Progress in Hemostasis (Part 1): Improved Management of Inherited Platelet Disorders: Reality or Illusion? Open
Platelets are key drivers of hemostasis. Low platelet counts, dysfunction in platelet adhesion, and aggregation lead to increased bleeding tendency. Inherited platelet disorders (IPDs) form a highly heterogeneous group of rare diseases wit…
View article: State-of-the-Art Targeted High-Throughput Sequencing for Detecting Inherited Platelet Disorders
State-of-the-Art Targeted High-Throughput Sequencing for Detecting Inherited Platelet Disorders Open
Inherited platelet disorders (IPDs) are a heterogeneous group of rare entities caused by molecular divergence in genes relevant for platelet formation and function. A rational diagnostic approach is necessary to counsel and treat patients …
View article: Treatment of Inherited Platelet Disorders: Current Status and Future Options
Treatment of Inherited Platelet Disorders: Current Status and Future Options Open
Inherited platelet disorders (IPDs) comprise a heterogeneous group of entities that manifest with variable bleeding tendencies. For successful treatment, the underlying platelet disorder, bleeding severity and location, age, and sex must b…
View article: Long-Term Comparison of Two- and Three-Dimensional Computed Tomography Analyses of Cranial Bone Defects in Severe Parietal Thinning
Long-Term Comparison of Two- and Three-Dimensional Computed Tomography Analyses of Cranial Bone Defects in Severe Parietal Thinning Open
Quantification of bone loss for follow-up comparisons has not been routinely performed in patients with parietal thinning so far. This study compares different methodological approaches including both 2- and 3-dimensional methods in comput…
View article: Clinical Trials in Chronic Arthritic Diseases with Underestimated Impact of Placebo Effects on Study Size Calculation
Clinical Trials in Chronic Arthritic Diseases with Underestimated Impact of Placebo Effects on Study Size Calculation Open
Whether and to which extent placebo treatment in double-blinded randomized controlled clinical trials is effective in chronic arthritic diseases has not been studied before. Therefore, a systematic literature search was undertaken to detec…
View article: Optimization of the Alizarin Red S Assay by Enhancing Mineralization of Osteoblasts
Optimization of the Alizarin Red S Assay by Enhancing Mineralization of Osteoblasts Open
The alizarin red S assay is considered the gold standard for quantification of osteoblast mineralization and is thus widely used among scientists. However, there are several restrictions to this method, e.g., moderate sensitivity makes it …
View article: Low Bone Mineral Density in Hemophiliacs
Low Bone Mineral Density in Hemophiliacs Open
Objective To review the current knowledge on bone health in patients with hemophilia A and the underlying pathogenetic mechanisms. Data Sources Original research articles, meta-analyses, and scientific reviews. Data Synthesis Already in ch…
View article: Administration of fibrinogen concentrate combined with prothrombin complex maintains hemostasis in children undergoing congenital heart repair (a long‐term propensity score‐matched study)
Administration of fibrinogen concentrate combined with prothrombin complex maintains hemostasis in children undergoing congenital heart repair (a long‐term propensity score‐matched study) Open
Background Bleeding is a common problem in children with congenital heart disease undergoing major cardiac surgery requiring cardiopulmonary bypass (CPB). Little is known about optimal management with blood products. Objective To investiga…
View article: Practical Guidance of the GTH Haemophilia Board on the Use of Emicizumab in Patients with Haemophilia A
Practical Guidance of the GTH Haemophilia Board on the Use of Emicizumab in Patients with Haemophilia A Open
Emicizumab has been approved for bleeding prophylaxis in patients with haemophilia A (PWHAs) with or without inhibitors. Because of substantial differences between factor VIII (FVIII) and Emicizumab, the ‘Ständige Kommission Hämophilie’ of…
View article: Paroxysmal Nocturnal Hemoglobinuria: An Underestimated Cause of Pediatric Thromboembolism
Paroxysmal Nocturnal Hemoglobinuria: An Underestimated Cause of Pediatric Thromboembolism Open
Paroxysmal nocturnal hemoglobinuria (PNH) is a chronic disease caused by complement-mediated hemolysis. Clinical symptoms include intravascular hemolysis, nocturnal hemoglobinuria, thromboses, cytopenia, fatigue, abdominal pain, and a stro…
View article: Child Abuse or Bleeding Disorder—An Interdisciplinary Approach
Child Abuse or Bleeding Disorder—An Interdisciplinary Approach Open
Children with an unexplained bleeding tendency are frequently referred to a haemostaseologist for further evaluation. Careful standardized history taking and clinical evaluation should allow for distinguishing bleeds after minor injury and…
View article: Use of Targeted High-Throughput Sequencing for Genetic Classification of Patients with Bleeding Diathesis and Suspected Platelet Disorder
Use of Targeted High-Throughput Sequencing for Genetic Classification of Patients with Bleeding Diathesis and Suspected Platelet Disorder Open
Inherited platelet disorders (IPD) form a rare and heterogeneous disease entity that is present in about 8% of patients with non-acquired bleeding diathesis. Identification of the defective cellular pathway is an important criterion for st…
View article: Differential clinical presentation of Adamantiades–Behçet's disease in non‐endemic and endemic areas: retrospective data from a Middle‐European cohort study
Differential clinical presentation of Adamantiades–Behçet's disease in non‐endemic and endemic areas: retrospective data from a Middle‐European cohort study Open
Objectives To assess demographical and clinical data in a Middle‐European cohort of patients with Adamantiades–Behçet's disease ( ABD ), together with the use of medication in adherence to international guidelines. Methods In a retrospecti…