Class 5. Pulmonary Hypertension Associated with Hematological, Systemic, Metabolic, Nutritional and Other Disorders

Sex as an Independent Risk Factor for Venous Thromboembolism in Sickle Cell Disease: A Cross-Sectional Study

Andrea H. Roe, Arden McAllister, Corinne Kete, Hilary Whitworth, Courtney A. Schreiber, Farzana A. SayaniUniversity of Pennsylvania Perelman School of Medicine. The Children’s Hospital of Philadelphia.United States Journal of Women’s HealthJ Womens Health 2022; 31: 1467-1471DOI: 10.1089/jwh.2022.0046 AbstractVenous thromboembolism (VTE) affects up to 25% of individuals with sickle cell disease (SCD), but risk factors are not […]

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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

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

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

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

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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Case report: Pulmonary hypertensive crisis leading to cardiac arrest during endoscopic evaluation in a 6-year-old boy with autism, severe malnutrition, and undiagnosed scurvy

Laura A. Quinn, Stephanie P. Gilley, Allison D. Ta, Benjamin S. Frank, Carolyn B. Foley, Jaime M. MooreUniversity of Colorado School of Medicine.United States Frontiers in PediatricsFront Pediatr 2022; DOI: 10.3389/fped.2022.1008507 AbstractPediatric gastroenterologists are often responsible for the evaluation of malnutrition in the setting of selective eating. Endoscopic evaluation for conditions including eosinophilic esophagitis and celiac

Case report: Pulmonary hypertensive crisis leading to cardiac arrest during endoscopic evaluation in a 6-year-old boy with autism, severe malnutrition, and undiagnosed scurvy Read More »

Pulmonary hypertension screening in children with sickle cell disease

Kok Hoe Chan, Syeda Hiba Rizvi, Wilfredo De Jesus-Rojas, James M. Stark, Ricardo A. Mosquera, Adriana Carolina Prada-Ruiz, Traci Gonzales, Deborah L. Brown, Neethu M. Menon, Trinh T. Nguyen, Cindy K. Jon, Aravind YadavMcGovern Medical School at the University of Texas Health Science Center at Houston. Ponce Health Science University and Ponce Research Institute. Baylor

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Randomized Clinical and Biochemical Study Comparing the Effect of L-arginine and Sildenafil in Beta Thalassemia Major Children With High Tricuspid Regurgitant Jet Velocity

Eman El-Khateeb, Sahar Mohamed El-Haggar, Osama El-Razaky, Mohamed Ramadan El-Shanshory, Tarek Mohamed Mostafa68904Tanta University.United Kingdom and Egypt Journal of Cardiovascular Pharmacology and TherapeuticsJ Cardiovasc Pharmacol Ther 2022; DOI: 10.1177/10742484221132671 AbstractBackground: Pulmonary hypertension (PHT) is common in β-thalassemia patients due to hemolysis, iron overload and diminished nitric oxide (NO) levels. Biochemical markers can help to understand the pathophysiology and to introduce new therapies for this condition.Aim: This study

Randomized Clinical and Biochemical Study Comparing the Effect of L-arginine and Sildenafil in Beta Thalassemia Major Children With High Tricuspid Regurgitant Jet Velocity Read More »

Sleep disordered breathing and its relation to stroke and pulmonary hypertension in children with sickle cell disease: a single-center cross-sectional study

Azza Tantawy, Nayera El‑Sherif, Sara Makkeyah, Nahed Salah Eldeen, Noura Bahaa El‑Din Farghal, Nanies Soliman, Fatma S. E. EbeidAin Shams University.Egypt Annals of HematologyAnn Hematol 2023; 102: 271-281DOI: 10.1007/s00277-023-05099-4 AbstractSleep disordered breathing (SDB) is a common underdiagnosed sequela of sickle cell disease (SCD) that has been linked to the frequency of vaso-occlusive crises. To determine the frequency of SDB in children with SCD and its association to SCD-related

Sleep disordered breathing and its relation to stroke and pulmonary hypertension in children with sickle cell disease: a single-center cross-sectional study Read More »

Pulmonary hypertension screening in children with sickle cell disease

Kok Hoe Chan, Syeda Hiba Rizvi, Wilfredo De Jesus-Rojas, James M. Stark, Ricardo A. Mosquera, Adrianna Carolina Prada-Ruiz, Traci Gonzalez, Deborah L. Brown, Neethu M. Menon, Trinh T. Nguyen, Cindy K. Jon, Aravind YadavUniversity of Texas Health Science Center at Houston. Ponce Health Science University and Ponce Research Institute. Baylor College of Medicine.United States Pediatric

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Pulmonary hypertension due to high cardiac output

Kanza N. Qaiser, Sandeep Sahay, Arriano R. TonelliCleveland Clinic. Houston Methodist Hospital.United States Respiratory MedicineRespir Med 2023; DOI: 10.1016/j.rmed.2022.107034 AbstractPulmonary hypertension (PH) is usually associated with a normal or decreased cardiac output (CO). Less commonly, PH can occur in the context of a hyperdynamic circulation, characterized by high CO (>8 L/min) and/or cardiac index ≥4 L/min/m2 in

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