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
Ischemic Cardiovascular Diseases: Mechanisms, Diagnosis and Therapy
Ischemic cardiovascular disease includes myocardial infarction, coronary atherosclerotic heart disease, angina pectoris, etc., constitute the leading cause of patient mortality by preventing tissues from getting sufficient oxygen and nutrients. Ischemic heart disease, as a clinical condition, is characterized by myocardial ischemia, causing an imbalance between myocardial blood supply and demand, ...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
-
Relaxin as a Cardiovascular Hormone: Physiology, Pathophysiology and Therapeutic Promises
Cardiovascular & Hematological Agents in Medicinal Chemistry The Antiplatelet and Antithrombotic Actions of Statins
Current Pharmaceutical Design Functional Genomics of Blood Pressure Determination: Dissecting and Assembling a Polygenic Trait by Experimental Genetics
Current Hypertension Reviews Role of Nitric Oxide and Mitochondrial Nitric Oxide Synthase in Energy Adaptive Responses
Current Cardiology Reviews Prevention and treatment of atherosclerosis with flaxseed -derived compound secoisolariciresinol diglucoside
Current Pharmaceutical Design Hypertension in the Neonatal Period: An Update
Current Hypertension Reviews The Control of Male Sexual Responses
Current Pharmaceutical Design Microvascular Function/Dysfunction Downstream a Coronary Stenosis
Current Pharmaceutical Design Alpha-2 Agonists: Can they Modify the Outcomes in the Postanesthesia Care Unit?
Current Drug Targets The Neuroprotective Effect of Ginkgo biloba Leaf Extract and its Possible Mechanism
Central Nervous System Agents in Medicinal Chemistry Metabolic Syndrome the Gynecologist’s Approach
Current Women`s Health Reviews Parental Hypertension and Cardiovascular Reactivity in Young Adults
Vascular Disease Prevention (Discontinued) Resistant Hypertension: Novel Insights
Current Hypertension Reviews Colloidal and Pharmacological Activity of Albumin in Clinical Fluid Management:Recent Developments
Current Drug Therapy Cardiovascular Disease in Masked Hypertension: Clinical Implications
Current Hypertension Reviews From French Paradox to Cancer Treatment: Anti-cancer Activities and Mechanisms of Resveratrol
Anti-Cancer Agents in Medicinal Chemistry Mass Spectrometric Analysis of F2-Isoprostanes: Markers and Mediators in Human Disease
Current Pharmaceutical Analysis Osteoprotegerin and Diabetes-Associated Pathologies
Current Molecular Medicine The Roles of Abnormal Renal Sodium Handling in Hypertension Associated with Metabolic Syndrome
Current Hypertension Reviews Membrane Transporters in Physiological Barriers of Pharmacological Importance
Current Pharmaceutical Design