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

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, […]

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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 »

An Unusual Etiology of Severe Right Heart Failure Deserving of Wider Recognition

Woosun Kang, Yu Zong, Dhruvil Ashishkumar Patel, Tushar Mishra, Luis AfonsoWayne State University. University of Chicago.United States Journal of the American College of Cardiology Case ReportsJACC Case Rep 2024; 29:DOI: 10.1016/j.jaccas.2024.102222 AbstractA previously healthy 31-year-old man presented with worsening shortness of breath and a petechial rash. Echocardiography showed severe right-sided heart failure with midsystolic notching of

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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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Variants in the SARS2 gene cause HUPRA syndrome with atypical features: two case reports and review of the literature

Elias Edward Lahham, JuhinaJamal Hasassneh, Dua Osamah Adawi, Mohamad Khaled IsmailAugusta Victoria Hospital. Beit-Jala Governmental Hospital.Palestine Oxford Medical Case ReportsOxford Med Case Rep 2023; 11: 407–409DOI: 10.1093/omcr/omad119 AbstractHyperuricemia, pulmonary hypertension, renal failure in infancy, and alkalosis (HUPRA syndrome) is a rare autosomal recessive mitochondrial disease with a prevalence of <1:1 000 000, due to variations in

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Pulmonary Artery Hypertension in Transfusion-Dependent Thalassemia

Vineeta Gupta, Vishnu Vijayakumar, Priyanka Aggarwal Ishan Kumar, Vikas AgrawalBanaras Hindu University. India Indian PediatricsIndian Pediatr 2024; 61: 49-52DOI: Not Available AbstractObjective: Patients with transfusion-dependent thalassemia (TDT) are at risk of developing pulmonary artery hypertension (PAH) due to chronic hemolysis, iron overload, hypercoagulability and splenectomy. The objective of the study was to assess the prevalence and

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