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

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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Reversible severe pulmonary hypertension related to scurvy in children

Marin Satawiriya, Apichai Khongphatthanayothin, Alisa LimsuwanFaculty of Medicine Ramathibodi Hospital and Mahidol University. Bangkok Heart Hospital. Chulalongkorn University. Thailand BioMed Central Cardiovascular DisordersBMC Cardiovasc Disord 2024; 24:DOI: 10.1186/s12872-023-03629-6 AbstractBackground: Severe pulmonary hypertension (PH) in childhood is rare and can manifest as a life-threatening episode. We present 2 children with restrictive dietary habits with severe pulmonary hypertension secondary

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Sickle Cell Disease

MA Bender, Katie CarlbergUniversity of Washington.United States GeneReviews (Internet)GeneReviews 2023;DOI: Not Available. Bookshelf ID: NBK1377 AbstractExcerptClinical characteristics: Sickle cell disease (SCD) is characterized by intermittent vaso-occlusive events and chronic hemolytic anemia. Vaso-occlusive events result in tissue ischemia leading to acute and chronic pain as well as organ damage that can affect any organ system, including the bones, spleen,

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Cardiovascular complications of sickle cell disease: A primer for the general clinician

Chibuzo Ilonze, Gift C. Echefu, Alexandria L. Broadnax, Adedoyin Johnson, Aniekeme Etuk, Onyedika J. IlonzeUniversity of Alabama at Birmingham. University of Tennessee Health Science Center. Indiana University School of Medicine. Infirmary Health Thomas Hospital.United States Journal of the National Medical AssociationJ Natl Med Assoc 2023;DOI: 10.1016/j.jnma.2023.11.010 AbstractSickle cell disease (SCD) is the most common hereditary hemoglobinopathy

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Thrombotic Microangiopathy After Hematopoietic Stem Cell and Solid Organ Transplantation: A Review for Intensive Care Physicians

Sanober Nusrat, Hugh Davis, Kira MacDougall, James N. George, Ryotaro Nakamura, Azra BorogovacUniversity of Oklahoma Health Sciences Center. City of Hope. United States Journal of Intensive Care MedicineJ Intensive Care Med 2023; DOI: 10.1177/08850666231200193 AbstractIntensive care physicians may assume the primary care of patients with transplant-associated thrombotic microangiopathy (TA-TMA), an uncommon but potentially critical complication of

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Is restrictive atrial septal defect a risk in partial anomalous pulmonary venous drainage repair?

Jiaquan Zhu, Yasuhiro Kotani, Devin Chetan, Lisa Zhao, John G. Coles, Christopher A. Caldarone, Glen S. Van Arsdell, Osami HonjoThe Hospital for Sick Children and University of Toronto.Canada Annals of Thoracic SurgeryAnn Thorac Surg 2014; 97: 1664-1670DOI: 10.1016/j.athoracsur.2014.01.051 AbstractBackground: The creation or enlargement of an atrial septal defect (ASD) in partial anomalous pulmonary venous drainage (PAPVD) repair

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Anomalous origin of the left pulmonary artery from the right pulmonary artery. Report of 2 cases and review of the literature

Kenneth L. Jue, Gunay Raghib, Kurt Amplatz, Paul Adams Jr, Jesse E. EdwardsCharles T. Miller Hospital and University of Minnesota.United States American Journal of Roentgenology Radium Therapy and Nuclear MedicineAm J Roentgenol Radium Ther Nucl Med 1965; 95: 598-610DOI: 10.2214/ajr.95.3.598 AbstractAbstract Not Available CategorySegmental Pulmonary Venous Disease. Without a Focus on Pulmonary HypertensionPulmonary Vascular Pathology Age

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