Sildenafil therapy in thalassemia patients with doppler-defined risk for pulmonary hypertension

نویسندگان

  • Claudia R. Morris
  • Hae-Young Kim
  • John C. Wood
  • John B. Porter
  • Elizabeth S. Klings
  • Felicia L. Trachtenberg
  • Nancy Sweeters
  • Nancy F. Olivieri
  • Janet L. Kwiatkowski
  • Lisa Virzi
  • Sylvia T. Singer
  • Ali Taher
  • Ellis J. Neufeld
  • Alexis A. Thompson
  • Vandana Sachdev
  • Sandra Larkin
  • Jung H. Suh
  • Frans A. Kuypers
  • Elliott P. Vichinsky
چکیده

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منابع مشابه

Thalassemia Associated Pulmonary Hypertension

Cardiac disease is the main cause of death in both forms of thalassemia; thalassemia major (TM) and thalassemia intermedia (TI). Pulmonary hypertension (PH) is one of the cardiopulmonary morbidities with high mortality that, if not treated, may trigger right-sided heart failure and premature death. PH is defined as a mean pulmonary artery pressure of ≥25 mmHg at rest or ≥30 mmHg during exercise...

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Sildenafil therapy in thalassemia patients with Doppler-defined risk of pulmonary hypertension.

Pulmonary hypertension is a common but often overlooked complication associated with thalassemia syndromes. There are limited data on the safety and efficacy of selective pulmonary vasodilators in this at-risk population. We, therefore, designed a 12-week, open-label, phase 1/2, pilot-scale, proof-of-principle trial of sildenafil therapy in 10 patients with β-thalassemia and at increased risk o...

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Prevalence and Risk Factors of Pulmonary Arterial Hypertension in Thalassemia Major Patients of Ilam, 2014

Pulmonary arterial hypertension (PAH) is a progressive disease with high morbidity and mortality rates. Research has shown that PAH has a prevalence rate of 10-79% in thalassemia major patients. This cross-sectional study was carried out in 2014 to determine the prevalence and risk factors of PAH in all thalassemia major patients of over 18 years of age in Ilam, Iran. A cardiologist measured sy...

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Efficacy and safety of sildenafil for the treatment of severe pulmonary hypertension in patients with hemoglobinopathies: results from a long-term follow up.

We read with interest the article by Morris et al. evaluating the role of sildenafil therapy for thalassemia patients with Doppler-defined risk for pulmonary hypertension (PH). Here we reflect on the Authors’ findings and highlight our experience with sildenafil therapy in a similar patient group, although on long-term therapy. PH of varying severity is commonly observed in patients with hemogl...

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تعیین شاخص های فارماکوکینتیکی شربت سیلدنافیل در هیپرتانسیون ریوی کودکان

Background and purpose: Pulmonary arterial hypertension (PAH) is a fatal disease affecting patients in all ages that could result in acute and chronic heart failure with high mortality rate. Oral sildenafil is approved to treat PAH in adults and recently in children. There are limited studies on the pharmacokinetics parameters of sildenafil, therefore, this study was designed to determine the p...

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تاریخ انتشار 2013