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Pediatric Idiopathic Pulmonary Artery Hypertension

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Trans-Right-Ventricle and Transpulmonary MicroRNA Gradients in Human Pulmonary Arterial Hypertension.

Philippe Chouvarine, Jonas Geldner, Roberto Giagnorio, Ekaterina Legchenko, Harald Bertram, Georg Hansmann,

OBJECTIVES:We investigated whether concentrations of circulating microRNAs differ across the hypertensive right ventricle and pulmonary circulation, and correlate with hemodynamic/echocardiographic variables in patients with pulmonary arterial hypertension versus nonpulmonary arterial hypertension controls. DESIGN:Prospective blood collection during cardiac catheterization from the superior vena cava, pulmonary artery, and ascending aorta in 12 ... Read more >>

Pediatr Crit Care Med (Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies)
[2019, :]

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Pulmonary artery aneurysm: a review.

Mridul Gupta, Abhinav Agrawal, Annamaria Iakovou, Stuart Cohen, Rakesh Shah, Arunabh Talwar,

Pulmonary artery aneurysm is a rare but important entity in the spectrum of pulmonary vascular diseases. The etiologies can be varied and patients can present with non-specific symptoms with the diagnosis being incidental. There is limited consensus regarding the diagnostic criteria and follow-up imaging for patients diagnosed with this entity. ... Read more >>

Pulm Circ (Pulmonary Circulation)
[2020, 10(1):2045894020908780]

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A novel unidirectional-valved shunt approach for end-stage pulmonary arterial hypertension: Early experience in adolescents and adults.

Erika B Rosenzweig, Ashish Ankola, Usha Krishnan, William Middlesworth, Emile Bacha, Matthew Bacchetta,

OBJECTIVES:Despite advances in treatment of idiopathic pulmonary arterial hypertension (IPAH), there remains no medical cure, and patients can experience disease progression leading to right heart failure, progressive exercise intolerance, and death. The reversed Potts shunt (left pulmonary artery to descending aorta) was reintroduced for treatment of end-stage IPAH to permit ... Read more >>

J. Thorac. Cardiovasc. Surg. (The Journal of thoracic and cardiovascular surgery)
[2019, :]

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Perinatal Management of Bart's Hemoglobinopathy: Paradoxical Effects of Intrauterine, Transplacental, and Partial Exchange Transfusions.

Mark Curran, Michel Mikhael, Wang-Dar Sun, Jina Lim, Anna Leung, Gira Morchi, Ramen H Chmait,

We describe a fetus at 24 3/7 weeks' gestation that showed ultrasound evidence of anemia, hydrops, and severe growth restriction. Both parents were known to be cis heterozygous carriers for SEA α-thalassemia deletion (αα/-). Cordocentesis confirmed fetal anemia and homozygous α-thalassemia (-/-) in the fetus. Fetal intrauterine transfusions corrected the ... Read more >>

AJP Rep (AJP reports)
[2020, 10(1):e11-e14]

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Pediatric antiphospholipid syndrome.

Jacqueline A Madison, Yu Zuo, Jason S Knight,

Despite its potential to cause significant morbidity in children, pediatric antiphospholipid syndrome (APS) is an understudied condition. In this review, we will cover what is known about pediatric APS epidemiology and how the clinician might approach the diagnosis of pediatric APS. We will highlight similarities and differences with the adult ... Read more >>

Eur J Rheumatol (European journal of rheumatology)
[2019, :1-10]

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Cellular sources of IL-6 and associations with clinical phenotypes and outcomes in PAH.

Catherine E Simpson, Jenny Y Chen, Rachel L Damico, Paul M Hassoun, Lisa J Martin, Jun Yang, Melanie Nies, Megan Griffiths, R Dhananjay Vaidya, Stephanie Brandal, Michael W Pauciulo, Katie A Lutz, Anna W Coleman, Eric D Austin, Dunbar D Ivy, William C Nichols, Allen D Everett,

The pro-inflammatory cytokine interleukin-6 (IL-6) has been associated with outcomes in small pulmonary arterial hypertension (PAH) cohorts composed largely of patients with severe idiopathic PAH (IPAH). It is unclear whether IL-6 is a marker of critical illness or a mechanistic biomarker of pulmonary vascular remodelling. We hypothesised that IL-6 is ... Read more >>

Eur. Respir. J. (The European respiratory journal)
[2020, :]

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Effect of antenatal tetramethylpyrazine on lung development and YAP expression in rat model of experimental congenital diaphragmatic hernia.

Junzuo Liao, Wenying Liu, Libin Zhang, Qin Li, Fang Hou, Pingjin Zou,

The aim of this study was to investigate the therapeutic effects and underlying mechanism of tetramethylpyrazine (TMP) on lung development using a rat model of congenital diaphragmatic hernia (CDH). Nitrofen was used to induce CDH. Pregnant rats were divided into three groups: control, CDH, and CDH+TMP. In the CDH and ... Read more >>

Int J Clin Exp Pathol (International journal of clinical and experimental pathology)
[2020, 13(1):81-88]

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Hemodynamic and clinical effects of selexipag in children with pulmonary hypertension.

Abraham Rothman, Gabriel Cruz, William N Evans, Humberto Restrepo,

Selexipag is an oral prostacyclin receptor agonist; it was recently approved for use in adults with pulmonary arterial hypertension. The safety and efficacy of selexipag has not yet been determined in the pediatric population. We describe short-term hemodynamic and clinical data with selexipag therapy in four pediatric patients with pulmonary ... Read more >>

Pulm Circ (Pulmonary circulation)
[2020, 10(1):2045894019876545]

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Characterization of Patients with Pulmonary Arterial Hypertension: Data from the Polish Registry of Pulmonary Hypertension (BNP-PL).

Grzegorz Kopeć, Marcin Kurzyna, Ewa Mroczek, Łukasz Chrzanowski, Tatiana Mularek-Kubzdela, Ilona Skoczylas, Beata Kuśmierczyk, Piotr Pruszczyk, Piotr Błaszczak, Ewa Lewicka, Danuta Karasek, Katarzyna Mizia-Stec, Michał Tomaszewski, Wojciech Jacheć, Katarzyna Ptaszyńska-Kopczyńska, Małgorzata Peregud-Pogorzelska, Anna Doboszyńska, Agnieszka Pawlak, Zbigniew Gąsior, Wiesława Zabłocka, Robert Ryczek, Katarzyna Widejko-Pietkiewicz, Marcin Waligóra, Szymon Darocha, Michał Furdal, Michał Ciurzyński, Jarosław D Kasprzak, Marek Grabka, Karol Kamiński, Piotr Hoffman, Piotr Podolec, Adam Torbicki,

Current knowledge of pulmonary arterial hypertension (PAH) epidemiology is based mainly on data from Western populations, and therefore we aimed to characterize a large group of Caucasian PAH adults of Central-Eastern European origin. We analyzed data of incident and prevalent PAH adults enrolled in a prospective national registry involving all ... Read more >>

J Clin Med (Journal of clinical medicine)
[2020, 9(1):]

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Graded balloon atrial septostomy for palliation of congenital pulmonary hypertension in a dog: A case report.

Justin Allen, Nathan Peterson, Kirstie Barrett, Anthony Llamas,

CASE DESCRIPTION:A 6-month-old intact female Maltese dog was presented for acute onset of syncope. CLINICAL FINDINGS:The dog was presented for collapse upon excitement and exercise. It collapsed at discharge and suffered cardiopulmonary arrest. Echocardiography after resuscitation indicated severe pulmonary hypertension without evidence of intracardiac or extracardiac shunting. A presumptive diagnosis ... Read more >>

J. Vet. Intern. Med. (Journal of veterinary internal medicine)
[2020, 34(1):283-288]

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"Idiopathic" pulmonary arterial hypertension in early infancy: Excluding NFU1 deficiency.

Paquay Stéphanie, Barrea Catherine, Sluysmans Thierry, Vachiery Jean-Luc, Loeckx Isabelle, Seneca Sara, Vô Christophe, Nassogne Marie-Cécile,

NFU1 deficiency is a rare metabolic disorder affecting iron-sulfur cluster synthesis, an essential pathway for lipoic acid-dependent enzymatic activities and mitochondrial respiratory chain complexes. It is a little-known cause of pulmonary arterial hypertension (PAH), while PAH is a prominent feature of the disease. We herein report on a female infant ... Read more >>

Ann Pediatr Cardiol (Annals of pediatric cardiology)
[2019, 12(3):325-328]

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The Role of the Anti-Inflammatory Cytokine Interleukin-10 in Tissue Fibrosis.

Emily H Steen, Xinyi Wang, Swathi Balaji, Manish J Butte, Paul L Bollyky, Sundeep G Keswani,

Significance: Fibrosis is the endpoint of chronic disease in multiple organs, including the skin, heart, lungs, intestine, liver, and kidneys. Pathologic accumulation of fibrotic tissue results in a loss of structural integrity and function, with resultant increases in morbidity and mortality. Understanding the pathways governing fibrosis and identifying therapeutic targets ... Read more >>

Adv Wound Care (New Rochelle) (Advances in wound care)
[2020, 9(4):184-198]

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Life-Threatening Infectious Complications in Sickle Cell Disease: A Concise Narrative Review.

Dominik Ochocinski, Mansi Dalal, L Vandy Black, Silvana Carr, Judy Lew, Kevin Sullivan, Niranjan Kissoon,

Sickle cell disease (SCD) results in chronic hemolytic anemia, recurrent vascular occlusion, insidious vital organ deterioration, early mortality, and diminished quality of life. Life-threatening acute physiologic crises may occur on a background of progressive diminishing vital organ function. Sickle hemoglobin polymerizes in the deoxygenated state, resulting in erythrocyte membrane deformation, ... Read more >>

Front Pediatr (Frontiers in pediatrics)
[2020, 8:38]

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China lung transplantation developing: past, present and future.

Bo Wu, Chunxiao Hu, Wenhui Chen, Jianxing He, Gening Jiang, Ji Zhang, Dong Liu, Xiaoshan Li, Dong Wei, Guohui Jiao, Chen Wang, Jingyu Chen,

Lung transplantation in China has been developing for almost 40 years (1979-2019). The pioneers of this procedure experienced struggles and obstacles upon accomplishment of the initial 20 cases of lung transplantation. Like the expanding process of transplant programs elsewhere in western countries and other regions in Asia, transplant teams in ... Read more >>

Ann Transl Med (Annals of translational medicine)
[2020, 8(3):41]

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Pulmonary Hypertension Associated with Bronchopulmonary Dysplasia: A Review.

Nidhy Varghese, Danielle Rios,

Early pulmonary vascular disease (PVD) and established pulmonary hypertension (PH) are common associations of bronchopulmonary dysplasia (BPD). Diagnosis of PH is often made by echocardiography because of technical and logistic difficulties with the neonatal population. Optimization of respiratory support is the focus of treatment, however, medical therapies are being used ... Read more >>

Pediatr Allergy Immunol Pulmonol (Pediatric allergy, immunology, and pulmonology)
[2019, 32(4):140-148]

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Outcomes of 50 Cases of Pediatric Liver Transplantation for Intrapulmonary Shunt While Considering the Deteriorated Prognosis of Severe Intrapulmonary Shunt Patients.

Yoshihiro Hirata, Seisuke Sakamoto, Shohei Yoshimura, Masahiro Takeda, Hajime Uchida, Seiichi Shimizu, Masayuki Kitamura, Rie Irie, Osamu Miyazaki, Takako Yoshioka, Syunsuke Nosaka, Mureo Kasahara,

BACKGROUND:Intrapulmonary shunt (IPS) is recognized in 10% of chronic liver disease patients. Liver transplantation (LT) is associated with a high risk of morbidity and mortality in patients with IPS. PATIENTS AND METHODS:Of 519 pediatric LT cases between November 2005 and October 2018, 50 patients with IPS were enrolled in this ... Read more >>

Transplant. Proc. (Transplantation proceedings)
[2019, 51(8):2793-2797]

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Postinfectious Bronchiolitis Obliterans in Children: Diagnostic Workup and Therapeutic Options: A Workshop Report.

Silvija-Pera Jerkic, Folke Brinkmann, Alistair Calder, Alicia Casey, Megan Dishop, Matthias Griese, Geoffrey Kurland, Mandy Niemitz, Sylvia Nyilas, Dirk Schramm, Ralf Schubert, Michael Tamm, Stefan Zielen, Martin Rosewich,

Bronchiolitis obliterans (BO) is a rare, chronic form of obstructive lung disease, often initiated with injury of the bronchiolar epithelium followed by an inflammatory response and progressive fibrosis of small airways resulting in nonuniform luminal obliteration or narrowing. The term BO comprises a group of diseases with different underlying etiologies, ... Read more >>

Can. Respir. J. (Canadian respiratory journal)
[2020, 2020:5852827]

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Sex hormone exposure and reproductive factors in pulmonary arterial hypertension: a case-control study.

Jessica B Badlam, David Badesch, Evan Brittain, Shannon Cordell, Tan Ding, Kelly Fox, Anna Hemnes, James Loyd, Meredith Pugh, Ivan Robbins, Chang Yu, Eric D Austin,

Pulmonary arterial hypertension (PAH) is a sexually dimorphic disease that for unknown reasons affects women more than men. The role of estrogens, both endogenous and exogenous, and reproductive factors in this female susceptibility is still poorly understood. It has been strongly suggested that sex hormones may influence the development and ... Read more >>

Pulm Circ (Pulmonary circulation)
[2020, 10(1):2045894020908786]

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Risk factors of meconium-related ileus in very low birth weight infants: patients-control study.

Jeik Byun, Ji-Won Han, Joong Kee Youn, Hee-Beom Yang, Seung Han Shin, Ee-Kyung Kim, Hyun-Young Kim, Sung-Eun Jung,

Very low birth weight (VLBW) neonates experience various problems, including meconium-related ileus (MRI). This study investigated the risk factors of MRI and surgical MRI in VLBW infants. VLBW neonates admitted to the Neonatal Intensive Care Unit of Seoul National University Children's Hospital from October 2002 to September 2016 were included ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):4674]

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Report on the Epidemiological Features of Coronavirus Disease 2019 (COVID-19) Outbreak in the Republic of Korea from January 19 to March 2, 2020.

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Since the first case of coronavirus disease19 (COVID-19) was reported in Wuhan, China, as of March 2, 2020, the total number of confirmed cases of COVID-19 was 89,069 cases in 67 countries and regions. As of 0 am, March 2, 2020, the Republic of Korea had the second-largest number of ... Read more >>

J. Korean Med. Sci. (Journal of Korean medical science)
[2020, 35(10):e112]

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Successful Extracorporeal Membrane Oxygenation After Incidental Azygos Vein Cannulation in a Neonate With Right-Sided Congenital Diaphragmatic Hernia Interruption of the Inferior Vena Cava and Azygos Continuation.

Alessandra Mayer, Genny Raffaeli, Federico Schena, Valeria Parente, Gabriele Sorrentino, Francesco Macchini, Anna Maria Colli, Lucia Mauri, Simona Neri, Irene Borzani, Ernesto Leva, Fabio Mosca, Giacomo Cavallaro,

Incidental azygos vein cannulation has been reported in a few cases of neonatal extracorporeal membrane oxygenation (ECMO). This complication is described in the literature mainly in pathological conditions wherein increased central venous pressure dilates the superior vena cava (SVC), i.e., right congenital diaphragmatic hernia (CDH) or pulmonary hypertension. Azygos vein ... Read more >>

Front Pediatr (Frontiers in pediatrics)
[2019, 7:444]

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Treatment of pediatric pulmonary arterial hypertension: A focus on the NO-sGC-cGMP pathway.

Maurice Beghetti, Matthias Gorenflo, D Dunbar Ivy, Shahin Moledina, Damien Bonnet,

OBJECTIVE:While pulmonary arterial hypertension (PAH) is rare in infants and children, it results in substantial morbidity and mortality. In recent years, prognosis has improved, coinciding with the introduction of new PAH-targeted therapies, although much of their use in children is off-label. Evidence to guide the treatment of children with PAH ... Read more >>

Pediatr. Pulmonol. (Pediatric pulmonology)
[2019, 54(10):1516-1526]

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Contemporary approaches in the use of extracorporeal membrane oxygenation to support patients waiting for lung transplantation.

Steven P Keller,

The introduction of the lung allocation score in 2005 prioritized patients with decreased transplant-free survival as the recipients of donor organs and effectively increased the number of critically-ill patients with end-stage lung disease waiting for transplantation. This change presented transplant programs with the challenge of how to both extend the ... Read more >>

Ann Cardiothorac Surg (Annals of cardiothoracic surgery)
[2020, 9(1):29-41]

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Dynamic changes of soluble ST2 levels predicted fatality and were involved in coagulopathy in dengue fever in the elderly.

Chih-Cheng Hsieh, Ming-Yuan Hong, Tzong-Shiann Ho, Ching-Chuan Liu, Guey-Chuen Perng, Chia-Chang Chuang,

BACKGROUND:Severe dengue virus (DENV) infection involves plasma leakage and vascular collapse, and leads to significant morbidity and death. Serum soluble ST2 (sST2 [interleukin (IL)-1 receptor like-1 protein: IL-1-RL-1]) levels are high in pediatric cases of DENV infection, and the disease progresses. However, the correlation between serum sST2 levels and the ... Read more >>

PLoS Negl Trop Dis (PLoS neglected tropical diseases)
[2019, 13(12):e0007974]

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Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art.

Yuchen Wang, Selena Chen, Junbao Du,

Idiopathic pulmonary arterial hypertension (IPAH) is a complex disease associated with progressive deterioration. Targeted therapy for IPAH has improved in the last several decades. However, there remain many challenges to current treatment of children with IPAH, including poor prognosis and a median survival of 0.8 years. Endothelin-1 (ET-1) appears to ... Read more >>

Front Pediatr (Frontiers in Pediatrics)
[2019, 7:302]

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