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

Updates in Pediatric Sickle Cell Lung Disease

Michelle Gillespie, Olufunke Afolabi-Brown, Evans Machogu, Shaina Willen, Benjamin T. KoppOhio State University Wexner Medical Center and Nationwide Children’s Hospital.Children’ Hospital of Philadelphia. Riley Hospital for Children and Indiana University School of Medicine. University of California Davis Medical Center. Children’s Healthcare of Atlanta.United States Clinics in Chest MedicineClin Chest Med 2024; 45: 749-760DOI: 10.1016/j.ccm.2024.02.022 AbstractSickle cell […]

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Cancer-Associated Thrombotic Microangiopathy: Literature Review and Report of Five Cases

L. Posado-Domínguez, A. J. Chamorro, E. Del Barco-Morillo, M. Martín-Galache, D. Bueno-Sacristán, E. Fonseca-Sánchez, A. Olivares-HernándezUniversity Hospital of Salamanca and University of Salamanca. Spain LifeLife 2024; 14:DOI: 10.3390/life14070865 AbstractThrombotic microangiopathy (TMA) is an anatomopathological lesion mediated by endothelial dysfunction and characterized by the creation of microthrombi in small vessels. In patients with cancer, it may be

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Pulmonary hypertension in the intensive care unit after pediatric allogeneic hematopoietic stem cell transplant: incidence, risk factors, and outcomes

Michael A. Smith, Geoffrey Cheng, Rachel Phelan, Ruta Brazauskas, Joelle Strom, Kwang Woo Ahn, Betty Ky Hamilton, Andrew Peterson, Bipin Savani, Hélène Schoemans, Michelle L. Schoettler, Mohamed Sorror, Roberta L. Keller, Christine S. Higham, Christopher C. Dvorak, Jeffrey R. Fineman, Matt S. ZinterUniversity of California, San Francisco. Medical College of Wisconsin. Cleveland Clinic. Vanderbilt University

Pulmonary hypertension in the intensive care unit after pediatric allogeneic hematopoietic stem cell transplant: incidence, risk factors, and outcomes Read More »

Splenectomy is significantly associated with thrombosis but not with pulmonary hypertension in patients with transfusion-dependent thalassemia: a meta-analysis of observational studies

Tsampika-Vasileia Kalamara, Konstantino Dodos, Efthymia VlachakiHippokration General Hospital and Aristotle University of Thessaloniki.Greece Frontiers in MedicineFront Med 2023; 10: DOI: 10.3389/fmed.2023.1259785 AbstractIntroduction: Thromboembolism (TE) and pulmonary hypertension (PH) constitute frequently occurring complications in patients with transfusion-dependent thalassemia and have been associated with splenectomy in different studies. Nevertheless, the size of the possible association varies greatly in literature. Herein,

Splenectomy is significantly associated with thrombosis but not with pulmonary hypertension in patients with transfusion-dependent thalassemia: a meta-analysis of observational studies Read More »

Association of elevated tricuspid regurgitation velocity with cerebrovascular and kidney disease in children with sickle cell disease

Chibuzo Ilonze, Parul Rai, Najibah Galadanci, Rima Zahr, Victoria I. Okhomina, Guolian Kang, Dakshin Padmanabhan, Jeffrey Lebensburger, Ammar Saadoon AlishlashUniversity of Alabama at Birmingham. St. Jude Children’s Research Hospital. University of Tennessee Health Science Center.United States Pediatric Blood and CancerPediatr Blood Cancer 2024;DOI: 10.1002/pbc.31002 AbstractBackground: Tricuspid regurgitation velocity (TRV), measured by echocardiography, is a surrogate marker for

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Low global arginine bioavailability: a common phenomenon in pulmonary hypertension

Dunia Hatabah, Teresa De Marco, Dana P. McGlothlin, Mary Malloy, Loretta Z. Reyes, Rawan Korman, Gregory J. Kato, Claudia R. MorrisEmory University School of Medicine. University of California, San Francisco. Kaiser Permanente San Francisco. Children’s Healthcare of Atlanta. Blood Science Consulting.United States American Journal of Physiology Lung Cellular and Molecular PhysiologyAm J Physiol Lung Cell

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Thrombin activatable fibrinolysis inhibitor plasma levels and TAFI Thr325Ile genetic polymorphism in a cohort of Egyptian sickle cell disease patients and impact on disease severity

Mona Hamdy, Iman A. Shaheen, Mohamed Khallaf, Yasmeen M. M. SelimCairo University.Egypt Pediatric Blood and CancerPediatr Blood Cancer 2024; 71:DOI: 10.1002/pbc.30959 AbstractBackground: Thrombin is a critical protease modulating thrombosis as well as inflammation, which are one of the main pathophysiological mechanisms in sickle vasculopathy, and its levels were reported to be high in sickle cell disease (SCD).

Thrombin activatable fibrinolysis inhibitor plasma levels and TAFI Thr325Ile genetic polymorphism in a cohort of Egyptian sickle cell disease patients and impact on disease severity Read More »

Mortality in adults with sickle cell disease: Results from the sickle cell disease implementation consortium (SCDIC) registry

Franklin Njoku, Norma Pugh, Donald Brambilla, Barbara Kroner, Nirmish Shah, Marsha Treadwell, Robert Gibson, Lewis L. Hsu, Victor R. Gordeuk, Jeffrey Glassberg, Jane S. Hankins, Abdullah Kutlar, Allison A. King, Julie KanterUniversity of Illinois at Chicago. Research Triangle International. Duke University. University of California San Francisco. Augusta University. Icahn School of Medicine at Mount Sinai.

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Transient abnormal myelopoiesis requiring advanced neonatal intensive care treatment

Maria Chalia, Emilie Seager, Anupama Rao, Simon HannamGreat Ormond Street Hospital for Children.United Kingdom Acta PaediatricaActa Paediatr 2024; DOI: 10.1111/apa.17142 AbstractAim: Five to thirty percent of neonates with trisomy 21 develop transient abnormal myelopoiesis (TAM) with a high mortality rate. The aim of the study was to identify contributing factors that determine mortality and need for chemotherapy

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[Clinical and genetic analysis of a patient with HUPRA syndrome due to missense variants of SARS2 gene and literature review]

J. Huang, Q. Y. Li. W. Ji, X.F. Guo, X. H. HuFujian Children’s Hospital. Beijing Anzhen Hospital and Capital Medical University. Shanghai Jiaotong University School of Medicine and Shanghai Children’s Medical Center. Fujian Provincial Maternity and Children’s Hospital. Xinhua Hospital.China Zhonghua Xin Xue Guan Bing Za Zhi 2024; 52: 172-179DOI: 10.3760/cma.j.cn112148-20231009-00268 Abstract (English)Objective: To explore the clinical

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