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Children pulmonary hypertension

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https://www.readbyqxmd.com/read/29327146/mid-term-outcome-of-100-consecutive-ross-procedures-excellent-survival-but-yet-to-be-a-cure
#1
Corina Zimmermann, Christine Attenhofer Jost, René Prêtre, Christoph Mueller, Matthias Greutmann, Burkhardt Seifert, Emanuela Valsangiacomo Büchel, Oliver Kretschmar, Hitendu Hasmukhlal Dave, Roland Weber
The Ross procedure offers excellent short-term outcome but the long-term durability is under debate. Reinterventions and follow-up of 100 consecutive patients undergoing Ross Procedure at our centre (1993-2011) were analysed. Follow-up was available for 96 patients (97%) with a median duration of 5.3 (0.1-17.1) years. Median age of the patient cohort was 15.2 (0.04-58.4) years with 76 males. 93% had underlying congenital aortic stenosis. Root replacement technique was applied in all. The most common valved conduits used for reconstruction of the right ventricular outflow tract were homografts (66 patients) and bovine jugular vein (ContegraR) graft (31 patients)...
January 12, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29322607/stenting-of-the-inter-atrial-septum-in-infants-and-small-children-indications-techniques-and-outcomes
#2
Gur Mainzer, Sebastian Goreczny, Gareth J Morgan, Shakeel Qureshi, Thomas Krasemann, Pawel Dryzek, Jadwiga A Moll, Tomasz Moszura, Eric Rosenthal
OBJECTIVES: To evaluate the procedural success and outcome of inter-atrial stenting. BACKGROUND: Inter-atrial stenting has been shown to be an effective way to maintain inter-atrial blood flow, however it is considered a high risk procedure, usually performed urgently in patients with significant hemodynamic compromise. METHODS: Between September 2004 and August 2016, inter-atrial stenting was attempted in 29 children. Procedural, clinical, and follow-up data were collected retrospectively...
January 11, 2018: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/29319632/extracorporeal-membrane-oxygenation-for-pertussis-predictors-of-outcome-including-pulmonary-hypertension-and-leukodepletion
#3
Michele Domico, Deborah Ridout, Graeme MacLaren, Ryan Barbaro, Gail Annich, Luregn J Schlapbach, Katherine L Brown
OBJECTIVE: The recent increase of pertussis cases worldwide has generated questions regarding the utility of extracorporeal membrane oxygenation for children with pertussis. We aimed to evaluate factors associated with extracorporeal membrane oxygenation outcome. DESIGN: The study was designed in two parts: a retrospective analysis of the Extracorporeal Life Support Organization Registry to identify factors independently linked to outcome, and an expanded dataset from individual institutions to examine the association of WBC count, pulmonary hypertension, and leukodepletion with survival...
January 9, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29313743/express-resource-utilization-at-the-time-of-prostacyclin-initiation-in-children-in-pulmonary-arterial-hypertension-a-multicenter-analysis
#4
Stephen Hart, Gaurav Arora, Brian Feingold
No abstract text is available yet for this article.
January 1, 2018: Pulmonary Circulation
https://www.readbyqxmd.com/read/29310361/severe-pertussis-infection-a-clinicopathological-study
#5
Fernando Palvo, Alexandre Todorovic Fabro, Maria Célia Cervi, Davi Casale Aragon, Fernando Silva Ramalho, Ana Paula de Carvalho Panzeri Carlotti
We aimed to investigate the clinicopathological features of pertussis in children admitted to a tertiary-care university hospital in Brazil.This was a retrospective cohort study of all pediatric hospital admissions with pertussis from January 1, 2008 to December 31, 2014. We also reported the autopsy findings in children who died.Fifty-five patients admitted to the hospital over the study period had laboratorial confirmation of Bordetella pertussis infection, 17 (30.9%) needed pediatric intensive care unit (PICU) admission and 6 (10...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29306249/biomarker-and-shear-stress-in-secondary-pediatric-pulmonary-hypertension
#6
Ayhan Pektaş, Rana Olguntürk, Serdar Kula, Erman Çilsal, Ayşe Deniz Oğuz, Fatma Sedef Tunaoğlu
Background/aim: Endothelial dysfunction, tissue damage, inflammation, and microthrombosis are involved in the pathogenesis of pulmonary hypertension (PH), which may be present as a complication of congenital heart diseases. This study aims to identify how indicators of endothelial dysfunction (shear stress), tissue damage (brain natriuretic peptide and troponin T), inflammation (C-reactive protein (CRP)), and microthrombosis (D-dimer and von Willebrand factor) are altered in children with congenital left-to-right shunting...
December 19, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29246465/resource-use-and-morbidities-in-pediatric-cardiac-surgery-patients-with-genetic-conditions
#7
Jamie Furlong-Dillard, David Bailly, Venugopal Amula, Jacob Wilkes, Susan Bratton
OBJECTIVE: To evaluate and describe resource use and perioperative morbidities among those patients with genetic conditions undergoing cardiac surgery. STUDY DESIGN: Using the Pediatric Health Information System database, we identified patients ≤18 years old with cardiac surgery classified by Risk Adjustment for Congenital Heart Surgery (RACHS) during 2003-2014. A total of 95 253 patients met study criteria and included no genetic conditions (84.6%), trisomy 21 (9...
December 12, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29243189/congenital-portosystemic-venous-shunt
#8
REVIEW
M Papamichail, M Pizanias, N Heaton
Congenital portosystemic venous shunts are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation and have been divided into extra- and intrahepatic shunts. They occur during liver and systemic venous vascular embryogenesis and are associated with other congenital abnormalities. They carry a higher risk of benign and malignant liver tumors and, if left untreated, can result in significant medical complications including systemic encephalopathy and pulmonary hypertension...
December 14, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29242651/perioperative-sildenafil-therapy-for-children-with-ventricular-septal-defects-and-associated-pulmonary-hypertension-undergoing-corrective-surgery-a-randomised-clinical-trial
#9
Sidharth Bhasin, Pooja Gogia, Rajeev Nair, Tapan Kumar Sahoo
Background and Aims: Sildenafil is known to reduce pulmonary artery pressure but its role in the perioperative period has not been well studied. We aimed to evaluate the efficacy of sildenafil in controlling post-operative pulmonary hypertension in children with pulmonary hypertension undergoing surgeries for correction of ventricular septal defect. Methods: The patients were divided randomly into two groups of thirty each. Group 1 (placebo) received pre-operative placebo and post-operative sildenafil (0...
October 2017: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/29233198/clinical-efficacy-and-safety-of-switch-from-bosentan-to-macitentan-in-children-and-young-adults-with-pulmonary-arterial-hypertension
#10
Ebru Aypar, Dursun Alehan, Tevfik Karagöz, Hayrettin Hakan Aykan, İlker Ertugrul
BACKGROUND: Macitentan is an orally active, potent, dual endothelin receptor antagonist and is the only registered treatment for pulmonary arterial hypertension that significantly reduced morbidity and mortality in a long-term event-driven study. Aim Few studies compared the clinical efficacy and safety of switch from bosentan to macitentan only in adult patients with pulmonary arterial hypertension. We aimed to evaluate the clinical efficacy and safety of switch from bosentan to macitentan in children and young adults...
December 13, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/29228916/physical-and-psychosomatic-health-outcomes-in-people-bereaved-by-suicide-compared-to-people-bereaved-by-other-modes-of-death-a-systematic-review
#11
Ailbhe Spillane, Celine Larkin, Paul Corcoran, Karen Matvienko-Sikar, Fiona Riordan, Ella Arensman
BACKGROUND: Little research has been conducted into the physical health implications of suicide bereavement compared to other causes of death. There is some evidence that suicide bereaved parents have higher morbidity, particularly in terms of chronic illness. This systematic review aims to examine the physical and psychosomatic morbidities of people bereaved by a family member's suicide and compare them with family members bereaved by other modes of death. METHODS: MEDLINE, EMBASE, CINAHL, and PsycINFO were searched from 1985 to February 2016...
December 12, 2017: BMC Public Health
https://www.readbyqxmd.com/read/29222289/what-is-the-role-of-screening-for-pulmonary-hypertension-in-adults-and-children-with-sickle-cell-disease
#12
REVIEW
Shaina M Willen, Mark T Gladwin
Patient case: An 18-year-old male patient with homozygous hemoglobin SS disease was evaluated for progressive dyspnea and elevated tricuspid regurgitant jet velocity (TRV) on echocardiography. The patient's case is described in detail in Lancet1 He had been treated with regular transfusions since childhood for stroke, had rare episodes of vaso-occlusive pain episodes, and did not take narcotic pain medications. He presented with progressive severe dyspnea on exertion and lower extremity edema. His laboratory tests were notable for a total hemoglobin level of 11...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29217599/ers-statement-on-obstructive-sleep-disordered-breathing-in-1-to-23-month-old-children
#13
Athanasios G Kaditis, Maria Luz Alonso Alvarez, An Boudewyns, Francois Abel, Emmanouel I Alexopoulos, Refika Ersu, Koen Joosten, Helena Larramona, Silvia Miano, Indra Narang, Hui-Leng Tan, Ha Trang, Marina Tsaoussoglou, Nele Vandenbussche, Maria Pia Villa, Dick Van Waardenburg, Silke Weber, Stijn Verhulst
The present statement was produced by a European Respiratory Society Task Force to summarise the evidence and current practice on the diagnosis and management of obstructive sleep disordered breathing (SDB) in children aged 1-23 months. A systematic literature search was completed and 159 articles were summarised to answer clinically relevant questions. SDB is suspected when symptoms or abnormalities related to upper airway obstruction are identified. Morbidity (pulmonary hypertension, growth delay, behavioural problems) and coexisting conditions (feeding difficulties, recurrent otitis media) may be present...
December 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29198536/infants-with-atypical-presentations-of-alveolar-capillary-dysplasia-with-misalignment-of-the-pulmonary-veins-who-underwent-bilateral-lung-transplantation
#14
Christopher T Towe, Frances V White, R Mark Grady, Stuart C Sweet, Pirooz Eghtesady, Daniel J Wegner, Partha Sen, Przemyslaw Szafranski, Pawel Stankiewicz, Aaron Hamvas, F Sessions Cole, Jennifer A Wambach
OBJECTIVE: To describe disease course, histopathology, and outcomes for infants with atypical presentations of alveolar capillary dysplasia with misalignment of the pulmonary veins (ACDMPV) who underwent bilateral lung transplantation. STUDY DESIGN: We reviewed clinical history, diagnostic studies, explant histology, genetic sequence results, and post-transplant course for 6 infants with atypical ACDMPV who underwent bilateral lung transplantation at St. Louis Children's Hospital...
November 30, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29195085/clinical-and-hematological-profile-in-a-newborn-cohort-with-hemoglobin-sc
#15
Paulo V Rezende, Millane V Santos, Gustavo F Campos, Laura L M Vieira, Maristela B Souza, André R Belisário, Celia M Silva, Marcos B Viana
OBJECTIVES: Hemoglobin SC is the second most common variant of sickle-cell disease worldwide, after hemoglobin SS. The objectives of the study were to describe the clinical and laboratory characteristics of hemoglobin SC disease in children from a newborn screening program and treated at a blood donor center. METHODOLOGY: This study assessed a cohort of 461 infants born between 01/01/1999 and 12/31/2012 and followed-up until 12/31/2014. Clinical events were expressed as rates for 100 patient-years, with 95% confidence intervals...
November 28, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/29193315/review-shows-that-implementing-a-nationwide-protocol-for-congenital-diaphragmatic-hernia-was-a-key-factor-in-reducing-mortality-and-morbidity
#16
REVIEW
L Storme, J Boubnova, S Mur, L Pognon, D Sharma, E Aubry, R Sfeir, P Vaast, T Rakza, A Benachi
The French Rare Disease Reference Center for Congenital Diaphragmatic Hernia (CDH) was created in 2008, to implement a national protocol for fetuses and children with this serious condition. Neonatal mortality from CDH is 30-40%, mainly due to pulmonary hypoplasia and persistent pulmonary hypertension, and half of those who live have high respiratory, nutritional and digestive morbidity. CDH management requires long-term and specialised multidisciplinary care. It has been well established that a standardised management protocol improves the prognosis of children with CDH...
November 28, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/29187656/-perioperative-safety-of-tibetan-children-with-congenital-heart-disease-undergoing-cardiac-surgery-and-anesthesia-in-low-altitude-area
#17
Yundan Pan, Jiaojiao Niu, Gang Qin, Lu Wang, Fan Zhang, E Wang
To observe the clinical parameters and short-term prognosis of Tibetan high-altitude area children with congenital heart disease undergoing surgery and anaesthesia in low-altitude area, and to investigate the perioperative safety of the treatment.
 Methods: From January, 2016 to December, 2016, 14 children with congenital heart disease who underwent surgery were assigned into 2 groups (n=7 each): the high-altitude area group (X group, children from Tibetan Autonomous Region) and the low-altitude area group (H group, children from Hunan Province)...
November 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/29169714/-idiopathic-nephrotic-syndrome
#18
O Boyer, V Baudouin, E Bérard, C Dossier, V Audard, V Guigonis, I Vrillon
Nephrotic syndrome (NS) is defined by massive proteinuria and hypoalbuminemia, with resulting hyperlipidemia and edema. The most common cause of NS in children is idiopathic nephrotic syndrome (INS), also called nephrosis. Its annual incidence has been estimated to 1-4 per 100,000 children and varies with age, race, and geography. Many agents or conditions have been reported to be associated with INS such as infectious diseases, drugs, allergy, vaccinations, and malignancies. The disease may occur during the 1st year of life, but it usually starts between the ages of 2 and 7 years...
December 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29129352/pediatric-cardiovascular-morbidity-of-the-early-term-newborn
#19
Gil Gutvirtz, Tamar Wainstock, Eyal Sheiner, Daniella Landau, Asnat Walfisch
OBJECTIVE: To determine whether early term delivery (at 37(0/7)-38(6/7) weeks of gestation) is associated with long-term pediatric cardiovascular morbidity of the offspring. STUDY DESIGN: A population-based cohort analysis was performed including all term deliveries occurring between 1991 and 2014 at a single tertiary medical center. Gestational age at delivery was subdivided into early term (37(0/7)-38(6/7)), full term (39(0/7)-40(6/7)), late term (41(0/7)-41(6/7)) and post term (≥42(0/7)) delivery...
November 9, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29125447/multiple-echocardiography-abnormalities-associated-with-endoscopic-third-ventriculostomy-failure
#20
Ashish H Shah, George M Ibrahim, Jun Sasaki, John Ragheb, Sanjiv Bhatia, Toba N Niazi
OBJECTIVE Although endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has gained increasing prominence in the management of hydrocephalus caused by intraventricular hemorrhage of prematurity, the rates of long-term shunt independence remain low. Furthermore, limited evidence is available to identify infants who might benefit from the procedure. The authors tested the hypothesis that elevated venous pressure that results from comorbid cardiac disease might predispose patients to ETV/CPC failure and shunt dependence...
November 10, 2017: Journal of Neurosurgery. Pediatrics
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