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Implications for the metabolic fate of oral glutamine supplementation within plasma and erythrocytes of patients with sickle cell disease: A pharmacokinetics study

Claudia R. Morris, Frans A. Kuypers, Robert Hagar, Sandra Larkin, Lisa Lavrisha, Augusta Saulys, Elliott P. Vichinsky, Jung H. SuhEmory University School of Medicine and Children’s Healthcare of Atlanta. UCSF-Benioff Children’s Hospital Oakland.United States Complementary Therapies in MedicineComplement Ther Med 2022; 64: DOI: 10.1016/j.ctim.2022.102803 AbstractObjectives: L-Glutamine is FDA-approved for sickle cell disease (SCD), yet the mechanism(s)-of-action are […]

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Heterozygous Tropomodulin 3 mice have improved lung vascularization after chronic hypoxia

Tsering Stobdan, Pritesh P. Jain, Mingmei Xiong, Vineet Bafna, Jason X.-J. Yuan, Gabriel G. HaddadUniversity of California San Diego. Rady Children’s Hospital.United States Human Molecular GeneticsHum Mol Genet 2022; 31: 1130-1140DOI: 10.1093/hmg/ddab291 AbstractThe molecular mechanisms leading to high-altitude pulmonary hypertension (HAPH) remains poorly understood. We previously analyzed the whole genome sequence of Kyrgyz highland population and

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Prevalent pharmacotherapy of US Fontan survivors: A study utilizing data from the MarketScan Commercial and Medicaid claims databases

Michael L. O’Byrne, Jennifer A. Faerber, Hannah Katcoff, Jing Huang, Jonathan B. Edelson, David M. Finkelstein, Bethan Lemley, Christopher M. Janson, Catherine M. Avitabile, Andrew C. Glatz, David J. GoldbergChildren’s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania. United States American Heart JournalAm Heart J 2022; 243: 158-166DOI: 10.1016/j.ahj.2021.09.012 AbstractBackground: Survivors of

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Multiparametric evaluation of right ventricular function in pulmonary arterial hypertension associated with congenital heart disease

Emmanuelle Fournier, Maëlle Selegny, Myriam Amsallem, Francois Haddad, Sarah Cohen, Estibaliz Valdeolmillos, Jérôme Le Pavec, Marc Humbert, Marc-Antoine Isorni, Arshid Azarine, Olivier Sitbon, Xavier Jais, Laurent Savale, David Montani, Elie Fadel, Joy Zoghbi, Emre Belli, Sebastien HascoëtMarie Lannelongue Hospital, Groupe Hospitalier Paris Saint Joseph and Paris-Saclay University. Stanford University School of Medicine. Marie Lannelongue Hospital.

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Mortality after Lung Transplantation for Children Bridged with Extracorporeal Membrane Oxygenation

Kyle Thompson, Steven J. Staffa, Viviane G. Nasr, Jill M. Zalieckas, Francis Fynn-Thompson, Debra Boyer, Ravi R. ThiagarajanBoston Children’s Hospital.United States Annals of the American Thoracic SocietyAnn Am Thorac Soc 2022; 19: 415-423DOI: 10.1513/AnnalsATS.202103-250OC AbstractRationale: Extracorporeal membrane oxygenation (ECMO) is increasingly used to bridge children who are wait-listed and failing conventional respiratory support for lung transplantation. Objectives: To compare

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Long-Term Observational Outcomes after Total Correction of Congenital Heart Disease in Korean Patients with Down Syndrome: A National Cohort Study

Ji Hee Kwak, Seung Won Lee, Hye Ryeong Cha, June Huh, I-Seok Kang, Tae-Gook Jun, Ji-Hyuk Yang, Man Yong Han, Jinyoung SongKangbuk Samsung Hospital and Sungkyunkwan University School of Medicine. Sejong University College of Software Convergence. CHA Bundang Medical Center and CHA University School of Medicine.Republic of Korea ChildrenChildren 2022; 9: DOI: 10.3390/children9091329 AbstractBackground: In the

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Post-operative course of pulmonary artery pressure after complete atrioventricular canal defect repair

Marie Emeline Pierre Louis, Adnan Bhutta, Adrian Holloway, Peter GaskinHouston Healthcare. University of Maryland School of Medicine. United States Cardiology in the YoungCardiol Young 2022; 32: 398-404DOI: 10.1017/S1047951121002201 AbstractComplete atrioventricular canal defect is a CHD associated with intra-cardiac shunting of blood, which can lead to irreversible changes in pulmonary vascular resistance and pulmonary artery hypertension if

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Thiamine-responsive, life-threatening, pulmonary hypertensive crisis with encephalopathy in young infants: A case series

C. Suryakanthi, P Keerthi Kundana, Nihaal Reddy, B. Satyanarayana Reddy, Preetham Poddutoor, Akheel Rizwan, Ramesh KoankiRainbow Children’s Hospital. India European Journal of Paediatric NeurologyEur J Paediatr Neurol 2022; 36: 93-98DOI: 10.1016/j.ejpn.2021.12.010 AbstractObjective: Thiamine (Vitamin B1) deficiency (TD), although reduced in incidence, is still seen in infants. We describe a rarely reported form of infantile TD with life-threatening

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Case Report: Vitamin C combined with multiple micronutrient deficiencies is associated with pulmonary arterial hypertension in children with autistic spectrum disorder

Wirada Sakamornchai, Oraporn Dumrongwongsiri, Sirinapa SiwaromRamathibodi Hospital and Mahidol University.Thailand Frontiers in NutritionFront Nutr 2022; 9: DOI: 10.3389/fnut.2022.928026 AbstractChildren with developmental and behavioral problems including autistic spectrum disorders (ASDs) may have inappropriate feeding behaviors, which leads to an increased risk of multiple nutrient deficiencies. Vitamin C deficiency is one of the common nutrient deficiencies reported in

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Comparison between BNP and NT-proBNP in pediatric populations

Kwaku D. Tawiah, Caroline E. Franks, Jessica Tang, Avihu Gazit, Dennis J. Dietzen, Christopher W. FarnsworthWashington University School of Medicine. National Institutes of Health.United States Clinical BiochemistryClin Biochem 2022; 109-110: 74-78DOI: 10.1016/j.clinbiochem.2022.08.003 AbstractBackground: B-type natriuretic peptide (BNP) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) are essential biomarkers for the evaluation of cardiac pathologies. However, pediatric reference intervals for

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