Abstract
OBJECTIVE: In order to provide an overview of current knowledge, the literature was systematically examined for clinical studies, which evaluate the safety and effectiveness of bosentan in pediatric pulmonary arterial hypertension (PAH). SOURCES: 3 databases (MEDLINE, EMBASE and BIOSIS) were searched for the period January 2000 – October 2007 using the key words “pulmonary arterial hypertension”, “bosentan”, and “pediatric patients/children”. RESULTS: Of 165 identified publications, 21 clinical studies were selected: 1 interventional prospective, 6 observational prospective, 5 observational retrospective, and 9 case reports/case series. In the absence of controlled trials, these 21 studies represent the current evidence on the effectiveness and safety of bosentan in the treatment of pediatric PAH. Bosentan appears to improve long-term functional status and hemodynamics in children with PAH but improvement in exercise capacity is not consistently demonstrated. Promising results are reported for the combination of bosentan with other PAHspecific treatments although guidelines for instituting combination therapy have not been defined. Overall, no safety concern is raised by these studies; adverse events, including liver enzyme elevations, appear to be less frequent than reported in the adult PAH clinical trials. CONCLUSION: Recent experience, although uncontrolled, suggests that bosentan is a well-tolerated and effective therapy for pediatric PAH.
Keywords: Bosentan, endothelin, endothelin receptors, pulmonary arterial hypertension, pediatrics
Current Vascular Pharmacology
Title: Bosentan in Pediatric Patients with Pulmonary Arterial Hypertension
Volume: 7 Issue: 2
Author(s): Maurice Beghetti
Affiliation:
Keywords: Bosentan, endothelin, endothelin receptors, pulmonary arterial hypertension, pediatrics
Abstract: OBJECTIVE: In order to provide an overview of current knowledge, the literature was systematically examined for clinical studies, which evaluate the safety and effectiveness of bosentan in pediatric pulmonary arterial hypertension (PAH). SOURCES: 3 databases (MEDLINE, EMBASE and BIOSIS) were searched for the period January 2000 – October 2007 using the key words “pulmonary arterial hypertension”, “bosentan”, and “pediatric patients/children”. RESULTS: Of 165 identified publications, 21 clinical studies were selected: 1 interventional prospective, 6 observational prospective, 5 observational retrospective, and 9 case reports/case series. In the absence of controlled trials, these 21 studies represent the current evidence on the effectiveness and safety of bosentan in the treatment of pediatric PAH. Bosentan appears to improve long-term functional status and hemodynamics in children with PAH but improvement in exercise capacity is not consistently demonstrated. Promising results are reported for the combination of bosentan with other PAHspecific treatments although guidelines for instituting combination therapy have not been defined. Overall, no safety concern is raised by these studies; adverse events, including liver enzyme elevations, appear to be less frequent than reported in the adult PAH clinical trials. CONCLUSION: Recent experience, although uncontrolled, suggests that bosentan is a well-tolerated and effective therapy for pediatric PAH.
Export Options
About this article
Cite this article as:
Beghetti Maurice, Bosentan in Pediatric Patients with Pulmonary Arterial Hypertension, Current Vascular Pharmacology 2009; 7 (2) . https://dx.doi.org/10.2174/157016109787455653
DOI https://dx.doi.org/10.2174/157016109787455653 |
Print ISSN 1570-1611 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-6212 |
Call for Papers in Thematic Issues
Advancements in Arterial Stiffness: Novel Therapeutic Frontiers
Arterial stiffness, a hallmark of cardiovascular disease, poses significant challenges in contemporary healthcare. This thematic issue delves into the multifaceted landscape of arterial stiffness and explores cutting-edge therapeutic interventions aimed at mitigating its adverse effects. Within these pages, readers will find a comprehensive overview of the mechanisms underlying arterial stiffness, ...read more
TREATMENT OF CARDIOVASCULAR DISEASE IN CHRONIC AND END STAGE KIDNEY DISEASE
Cardiovascular disease still remains the leading cause of death in Chronic and End Stage Kidney Disease, accounting for more than half of all deaths in dialysis patients. During the past decade, research has been focused on novel therapeutic agents that might delay or even reverse cardiovascular disease and vascular calcification, ...read more
- 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
- Announcements
Related Articles
-
A Clinician’s Guide to Monoamine Oxidase Inhibitors
Current Psychiatry Reviews Cortisol Regulation in the Metabolic Syndrome. A Novel Therapeutic Approach
Immunology, Endocrine & Metabolic Agents in Medicinal Chemistry (Discontinued) Prognostic Role of miRNAs in Coronary Artery Disease
Current Topics in Medicinal Chemistry Irritable Bowel Syndrome and Drospirenone-Containing Oral Contraceptives; A Comparative-Safety Study
Current Drug Safety Wasp Venom Toxins as a Potential Therapeutic Agent
Protein & Peptide Letters Ginkgo Biloba for Mild Cognitive Impairment and Alzheimer’s Disease: A Systematic Review and Meta-Analysis of Randomized Controlled Trials
Current Topics in Medicinal Chemistry Therapy of Chronic Hepatitis C: A Critical Review
Current Drug Targets - Infectious Disorders Lipid Profile of Children Suffering from Pediatric Rheumatic Diseases (pRDS)
Current Rheumatology Reviews Natural Polyphenols in the Treatment of Alzheimer’s Disease
Current Drug Targets Hypertension in Hemodialysis Patients
Current Hypertension Reviews Myocardial Perfusion and Coronary Vasomotor Function: Emerging Role of PET Imaging
Vascular Disease Prevention (Discontinued) ENaC in the Brain - Future Perspectives and Pharmacological Implications
Current Molecular Pharmacology Role of NADPH Oxidase in Hypertension and Diabetic Nephropathy
Current Hypertension Reviews Optimization of Important Early ADME(T) Parameters of NADPH Oxidase-4 Inhibitor Molecules
Medicinal Chemistry Vascular Consequences of Aldosterone Excess and Mineralocorticoid Receptor Antagonism
Current Hypertension Reviews Systemic Therapies for Pancreatic Cancer - The Role of Pharmacogenetics
Current Drug Targets Anti-Angiogenesis in the Treatment of Genito-Urinary Cancers: Last Updates
Current Angiogenesis (Discontinued) Cu-Based Solid Catalysts: Applications in Organic Transformations for NHeterocyclic Compounds
Current Organic Chemistry Beta-blockers in the Perioperative Period: Are there Indications other than Prevention of Cardiac Ischemia?
Current Drug Targets New Drugs, Therapeutic Strategies, and Future Direction for the Treatment of Pulmonary Arterial Hypertension
Current Medicinal Chemistry