Abstract
Von Willebrand disease (VWD) is an inherited bleeding disorder that is caused by deficiency or dysfunction of Von Willebrand factor, a plasma protein that mediates the initial adhesion of platelets at sites of vascular injury and also binds and stabilizes blood clotting factor VIII in the circulation. VWD prevalence has been estimated in several countries on the basis of the number of symptomatic patients seen at haemostasis centers, and the values range from roughly 23 to 110 per million population. VWD is characterized by three key features: a personal history of excessive mucocutaneous bleeding, abnormal VWF laboratory studies, and evidence of a family history of the condition. Although bruising and epistaxis are common among children who have VWD, these symptoms also are reported in normal children. The accurate assessment of hemorrhagic symptoms is a key component in diagnosing VWD but this can present a significant challenge, particularly in the pediatric population. The challenges involved in making the diagnosis of Von Willebrand disease - focusing on personal bleeding history and the utility of bleeding questionnaires, along with family bleeding history and laboratory diagnosis in the pediatric population - will be reviewed.
Current Pediatric Reviews
Title: Diagnosis of Von Willebrand Disease in Children
Volume: 5 Issue: 3
Author(s): Nupur Mittal, Paula James and Leonard A. Valentino
Affiliation:
Abstract: Von Willebrand disease (VWD) is an inherited bleeding disorder that is caused by deficiency or dysfunction of Von Willebrand factor, a plasma protein that mediates the initial adhesion of platelets at sites of vascular injury and also binds and stabilizes blood clotting factor VIII in the circulation. VWD prevalence has been estimated in several countries on the basis of the number of symptomatic patients seen at haemostasis centers, and the values range from roughly 23 to 110 per million population. VWD is characterized by three key features: a personal history of excessive mucocutaneous bleeding, abnormal VWF laboratory studies, and evidence of a family history of the condition. Although bruising and epistaxis are common among children who have VWD, these symptoms also are reported in normal children. The accurate assessment of hemorrhagic symptoms is a key component in diagnosing VWD but this can present a significant challenge, particularly in the pediatric population. The challenges involved in making the diagnosis of Von Willebrand disease - focusing on personal bleeding history and the utility of bleeding questionnaires, along with family bleeding history and laboratory diagnosis in the pediatric population - will be reviewed.
Export Options
About this article
Cite this article as:
Mittal Nupur, James Paula and Valentino A. Leonard, Diagnosis of Von Willebrand Disease in Children, Current Pediatric Reviews 2009; 5 (3) . https://dx.doi.org/10.2174/157339609789007213
DOI https://dx.doi.org/10.2174/157339609789007213 |
Print ISSN 1573-3963 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-6336 |
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
Related Articles
-
MicroRNAs in Organogenesis and Disease
Current Molecular Medicine Glycoconjugates of Quinolines: Application in Medicinal Chemistry
Mini-Reviews in Medicinal Chemistry The Role of Soluble 5'-Nucleotidases in the Conversion of Nucleotide Analogs: Metabolic and Therapeutic Aspects
Current Medicinal Chemistry Proteasome Inhibitors: Recent Advances and New Perspectives In Medicinal Chemistry
Current Topics in Medicinal Chemistry Clinical Trial Update and Novel Therapeutic Approaches for Metastatic Prostate Cancer
Current Medicinal Chemistry Therapeutic Use of MicroRNAs in Cancer
Anti-Cancer Agents in Medicinal Chemistry Alternative Splice Variants of Survivin as Potential Targets in Cancer
Current Drug Discovery Technologies Immunotherapy for Myeloproliferative Neoplasms (MPN)
Current Cancer Drug Targets Neurokinin Receptors as Potential Targets in Breast Cancer Treatment
Current Drug Discovery Technologies Peptide-based Radiopharmaceuticals for Targeted Tumor Therapy
Current Medicinal Chemistry The Parathyroid Hormone Receptorsome and the Potential for Therapeutic Intervention
Current Drug Targets Somatic Mutations, Viral Integration and Epigenetic Modification in the Evolution of Hepatitis B Virus-Induced Hepatocellular Carcinoma
Current Genomics Red Cell Glycolytic Enzyme Disorders Caused by Mutations: An Update
Cardiovascular & Hematological Disorders-Drug Targets The LPS-Pretreated MSCs Supply a Positive Microenvironment for Tumor Cell Proliferation and Clone Formation
Current Protein & Peptide Science Physiology of Folic Acid in Health and Disease
Current Drug Metabolism Optimizing Tumor-Reactive γδT Cells for Antibody-Based Cancer Immunotherapy
Current Molecular Medicine Novel and Emerging Drugs for Chronic Lymphocytic Leukemia
Current Cancer Drug Targets Cytokine Therapy for Cancer
Current Pharmaceutical Design Efficacy and Safety of Combined Radiotherapy with EGFR Inhibitors and Chemotherapy for Laryngeal Organ Preservation in Patients with Locally Advanced Hypopharyngeal Carcinomas
Current Cancer Drug Targets Low Dose Decitabine Combined with Taxol and Platinum Chemotherapy to Treat Refractory/Recurrent Ovarian Cancer: An Open-Label, Single-Arm, Phase I/II Study
Current Protein & Peptide Science