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

Pulmonary Histopathologic Findings in Pediatric Patients After Hematopoietic Stem Cell Transplantation: An Autopsy Study

Nahir Cortes-Santiago, Kalyani R. Patel, Hao Wu, Sara E. Sartain, Saleh Bhar, Manuel Silva-Carmona, Jennifer PogorilerTexas Children’s Hospital and Baylor College of Medicine. Yale School of Medicine and Yale New Haven Hospital. Children’s Hospital of Philadelphia.United States Pediatric and Developmental PathologyPediatr Dev Pathol 2023; DOI: 10.1177/10935266231170101 AbstractBackground: Pathologic characterization of pulmonary complications following hematopoietic stem cell transplantation […]

Pulmonary Histopathologic Findings in Pediatric Patients After Hematopoietic Stem Cell Transplantation: An Autopsy Study 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

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 »

RASA3 is a candidate gene in sickle cell disease-associated pulmonary hypertension and pulmonary arterial hypertension

Clare C. Prohaska, Xu Zhang, Tae‐Hwi L. Schwantes‐An, Robert S. Stearman, Stanley Hooker, Rick A. Kittles, Micheala A. Aldred, Katie A. Lutz, Michael W. Pauciulo, William C. Nichols, Ankit A. Desai, Victor R. Gordeuk, Roberto F. MachadoIndiana University. University of Illinois at Chicago. City of Hope. Morehouse School of Medicine. Cincinnati Children’s Hospital Medical Center

RASA3 is a candidate gene in sickle cell disease-associated pulmonary hypertension and pulmonary arterial hypertension Read More »

Endothelin-1 gene polymorphism (G8002A) and endothelial monocyte-activating polypeptide II: Role in vascular dysfunction in pediatric patients with β-thalassemia major

Azza Abdel Gawad Tantawy, Mourad Alfy Ramzy Tadros, Amira Abdel Moneam Adly, Eman Abdel Rahman Ismail, Fatma A. Ibrahim, Nanis Mohammed Salah Eldin, Mahitab Morsy Hussein, Mervat Abdalhameed Alfeky, Sarah Mohammed Ibrahim, Marwa Adel Hashem, Fatma Soliman Elsayed EbeidAin Shams University. Military Medical Services and Military Medical Academy.Egypt CytokineCytokine 2023; 161: DOI: 10.1016/j.cyto.2022.156048 AbstractBackground: Endothelin-1 (ET-1), a potent endogenous vasoconstrictor, stimulates production of reactive oxygen species. Endothelial monocyte-activating polypeptide-II (EMAP-II) is a multifunctional polypeptide.Aim: To assess ET-1 gene polymorphism

Endothelin-1 gene polymorphism (G8002A) and endothelial monocyte-activating polypeptide II: Role in vascular dysfunction in pediatric patients with β-thalassemia major Read More »

The long-term efficacy in blood transfusions, hematologic parameter changes, and complications after splenectomy in patients with transfusion-dependent thalassemia

Nichanan Osataphan, Somying Dumnil, Adisak Tantiworawit, Teerachat Punnachet, Nonthakorn Hantrakun, Pokpong Piriyakhuntorn, Thanawat Rattanathammethee, Sasinee Hantrakool, Chatree Chai-Adisaksopha, Ekarat Rattarittamrong, Lalita Norasetthada, Kanda Fanhchaksai, Pimlak CharoenkwanChiang Mai University. Thailand Transfusion and Apheresis ScienceTransfus Apher Sci 2022; DOI: 10.1016/j.transci.2022.103620 AbstractBackground: A splenectomy can reduce transfusion requirements in patients with thalassemia. However, the role of a splenectomy remains controversial

The long-term efficacy in blood transfusions, hematologic parameter changes, and complications after splenectomy in patients with transfusion-dependent thalassemia Read More »

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

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

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 »

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 »

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