Background: Sildenafil is used for the treatment of pediatric pulmonary hypertension. A dried blood spot (DBS)-based LC-MS method for sildenafil quantitation was developed and applied to a group of patients.
Results: DBS showed high portability and stability of samples, and the method was selective and linear for quantitation of sildenafil (5-3,000 ng/ml) and N-desmethyl-sildenafil (3-1,500 ng/ml). After a single oral dose of sildenafil (1 mg/kg), method evidenced poor metabolism in these patients.
Conclusion: The method was successfully applied in peripheral blood and can be used for both pharmacokinetics and therapeutic drug monitoring. DBS proved to have advantages during sample translation and preservation of analytes. Data suggest that hazardous blood sildenafil levels may be reached in this population.
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http://dx.doi.org/10.4155/bio.14.126 | DOI Listing |
PLoS One
December 2024
School of Pharmacy, Faculty of Medical and Health Sciences, The University of Auckland, Auckland, New Zealand.
The value of 'data-enabled', digital healthcare is evolving rapidly, as demonstrated in the COVID-19 pandemic, and its successful implementation remains complex and challenging. Harmonisation (within/between healthcare systems) of infrastructure and implementation strategies has the potential to promote safe, equitable and accessible digital healthcare, but guidance for implementation is lacking. Using respiratory technologies as an example, our scoping review process will capture and review the published research between 12th December 2013 to 12th December 2023.
View Article and Find Full Text PDFNeurol Int
December 2024
Department of Neurology, Oregon Health and Sciences University, Portland, OR 97239, USA.
Background/objectives: Transverse myelitis (TM) is a rare, acute inflammatory disorder affecting the spinal cord, with severe potential consequences, particularly in pediatric patients. Therapeutic plasma exchange (TPE) has emerged as a possible intervention for children unresponsive to high-dose corticosteroids. This study explores the efficacy of early TPE in pediatric TM through a case report and scoping review aiming to clarify the therapeutic benefits of TPE when used in conjunction with corticosteroids in children.
View Article and Find Full Text PDFJ Cardiovasc Dev Dis
December 2024
Department of Anesthesiology, University Children's Hospital, 30-663 Krakow, Poland.
Background: Major aortopulmonary collateral arteries (MAPCAs) are rare remnants of pulmonary circulation embryological development usually associated with complex congenital anomalies of the right ventricular outflow tract and pulmonary arteries. Effective management requires surgical unifocalization of MAPCAs and native pulmonary arteries (NPAs). Traditional imaging may lack the spatial clarity needed for precise surgical planning.
View Article and Find Full Text PDFAdv Respir Med
December 2024
Department of Pediatrics and Adolescent Medicine, Ulm University Medical Center, Ulm University, 89075 Ulm, Germany.
Elexacaftor/Tezacaftor/Ivacaftor (ETI) is a CFTR modulator therapy approved for people with cystic fibrosis (pwCF) who have at least one phe508del mutation. However, its approval in the European Union (EU) for pwCF with non-phe508del mutations is lacking, because data on treatment response in this subgroup are scarce. This retrospective observational study evaluated six pwCF (ages 6 to 66) with responsive CFTR mutations (M1101K, R347P, 2789+5G>A, G551D) undergoing off-label ETI therapy.
View Article and Find Full Text PDFCirc Cardiovasc Interv
December 2024
Department of Pediatrics, Pediatric Cardiology, Stanford University, Palo Alto, CA. (J.K.Y., L.W., A.C.T., H.C., A.W.R., L.F.P., S.R.C., A.M.D., D.B.M.).
Background: Varying rates of nonsustained ventricular tachycardia (NSVT) have been reported early after transcatheter pulmonary valve replacement (TPVR) with the Harmony valve, but data regarding rhythm outcomes beyond hospital discharge are limited. This study aims to characterize ventricular arrhythmias after Harmony TPVR from implant through mid-term follow-up.
Methods: Ventricular arrhythmia data from postimplant telemetry and follow-up extended rhythm monitoring (ERM) were analyzed after Harmony TPVR.
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