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Diaphragmatic hernia neonate

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https://www.readbyqxmd.com/read/29624536/working-memory-training-following-neonatal-critical-illness-a-randomized-controlled-trial
#1
Raisa M Schiller, Marlous J Madderom, Joost van Rosmalen, Arno F J van Heijst, Ivo de Blaauw, Elisabeth Utens, André B Rietman, Frank Verhulst, Dick Tibboel, Tonya White, Hanneke IJsselstijn
OBJECTIVES: To test the immediate and long-term effectiveness of Cogmed Working Memory Training following extracorporeal membrane oxygenation and/or congenital diaphragmatic hernia. DESIGN: A nationwide randomized controlled trial assessing neuropsychologic outcome immediately and 1 year post Cogmed Working Memory Training, conducted between October 2014 and June 2017. Researchers involved in the follow-up assessments were blinded to group allocation. SETTING: Erasmus MC-Sophia Children's Hospital, Rotterdam, and Radboud University Medical Center, Nijmegen, the Netherlands...
April 5, 2018: Critical Care Medicine
https://www.readbyqxmd.com/read/29610961/surgical-perspectives-regarding-application-of-biomaterials-for-the-management-of-large-congenital-diaphragmatic-hernia-defects
#2
REVIEW
Amulya K Saxena
This review focuses on the surgical viewpoints on patch repairs in neonates with large congenital diaphragmatic hernia defects. The main focus  is on the various biomaterials that have been employed to date with regard to their source of origins, degradation properties as well as tissue integration characteristics. Further focus  is on the present knowledge on patch integration when biomaterials are placed in the diaphragmatic defect. The review will also look at the present evidence on the biomechanical characteristics of the most commonly used biomaterials and compares these materials to diaphragmatic tissue to offer more  insight on the present practice of patch repairs in large defects...
April 2, 2018: Pediatric Surgery International
https://www.readbyqxmd.com/read/29605270/pumpless-arteriovenous-extracorporeal-membrane-oxygenation-a-novel-mode-of-respiratory-support-in-a-lamb-model-of-congenital-diaphragmatic-hernia
#3
Emily A Partridge, Marcus G Davey, Matthew Hornick, Kevin C Dysart, Aliza Olive, Robert Caskey, James T Connelly, Holly L Hedrick, William H Peranteau, Alan W Flake
BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is commonly required in neonates with congenital diaphragmatic hernia (CDH) complicated by pulmonary hypertension (PH). ECMO carries significant risk, and is contraindicated in the setting of extreme prematurity or intracranial hemorrhage. Pumpless arteriovenous ECMO (P-ECMO) may represent an alternative for respiratory support. The present study summarizes our initial experience with P-ECMO in a lamb model of CDH. STUDY DESIGN: Surgical creation of CDH was performed at 65-75days' gestation...
March 9, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29582948/-advances-in-fetal-therapy-in-complicated-monochorionic-pregnancies-and-in-severe-congenital-diaphragmatic-hernia-five-years-experience
#4
Yuval Gielchinsky, Dan V Valsky, Nili Yanai, Firas J Abdeljawad, Mazen A Muhaisen, Dan Arbell, Yehuda Ginosar, Orna Ben-Yosef, Orite Winograd, Benjamin Bar-Oz, Simcha Yagel, Smadar Eventov-Friedman
INTRODUCTION: : Minimally invasive fetal therapeutic procedures reduce the morbidity and mortality in monochorionic (MC) twins and in fetuses with congenital diaphragmatic hernia (CDH). MC pregnancies share their blood systems due to communicating vessels over their single placenta and may develop specific complications: Twin-to-Twin transfusion syndrome (TTTS), Selective intrauterine growth restriction (sIUGR), Twin Anemia-Polycythemia Sequence (TAPS), Twin Reverse Arterial Perfusion Syndrome (TRAP) or anomalies in one...
March 2018: Harefuah
https://www.readbyqxmd.com/read/29577002/use-of-nonabsorbable-spiral-tacks-for-mesh-reinforcement-in-thoracoscopic-repair-of-congenital-diaphragmatic-hernia
#5
Anna Poupalou, Celine Vrancken, Erwin Vanderveken, Henri Steyaert
Thoracoscopic prosthetic repair of congenital diaphragmatic hernia (CDH) is a well-established and safe technique in experienced hands but the patching procedure is technically demanding and time consuming. To address the challenges associated with this process (confined working space and restricted time), the aim of this article is to assess the potential improvements in feasibility, efficacy, and safety of patch fixation by using nonabsorbable helicoidal tacks in neonates and infants for the repair of large CDH by thoracoscopy...
January 2018: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/29552525/pediatric-thoracoscopic-repair-of-congenital-diaphragmatic-hernias
#6
Anne Schneider, François Becmeur
Congenital diaphragmatic hernia (CDH) is a rare congenital disease requiring neonatal surgical treatment. The traditional surgical management of CDH consists of diaphragmatic repair by laparotomy. Thoracoscopic repair techniques have been well described for CDH with late presentation. Nevertheless, its feasibility for CDH treatment in neonates emerged only the past few years because the use of thoracoscopy with carbon dioxide insufflation remains controversial in these patients more vulnerable to hypothermia and acidosis...
2018: Journal of Visualized Surgery
https://www.readbyqxmd.com/read/29550580/regeneration-of-diaphragm-with-bio-3d-cellular-patch
#7
Xiu-Ying Zhang, Yusuke Yanagi, Zijing Sheng, Kouji Nagata, Koichi Nakayama, Tomoaki Taguchi
Neonates with congenital diaphragmatic hernia often require surgical defect closure with a patch. Alternatives to native diaphragmatic tissue are critically needed for this paediatric surgery. The clinical efficacy of mesh patches is limited by complications associated with residual foreign material and by hernia recurrence. In this study, we used a novel bio-3D printer method to generate large scaffold-free tissue patches composed of human cells. The resulting large tissue constructs had high elasticity and strength...
March 9, 2018: Biomaterials
https://www.readbyqxmd.com/read/29549119/the-case-for-early-use-of-rapid-whole-genome-sequencing-in-management-of-critically-ill-infants-late-diagnosis-of-coffin-siris-syndrome-in-an-infant-with-left-congenital-diaphragmatic-hernia-congenital-heart-disease-and-recurrent-infections
#8
Nathaly M Sweeney, Shareef A Nahas, Shimul Chowdhury, Miguel Del Campo, Marilyn C Jones, David P Dimmock, Stephen F Kingsmore, Rcigm Investigators
Congenital diaphragmatic hernia (CDH) results from incomplete formation of the diaphragm leading to herniation of abdominal organs into the thoracic cavity. CDH is associated with pulmonary hypoplasia, congenital heart disease and pulmonary hypertension. Genetically, it is associated with aneuploidies, chromosomal copy number variants, and single gene mutations. CDH is the most expensive non-cardiac congenital defect: Management frequently requires implementation of Extracorporeal Membrane Oxygenation (ECMO), which increases management expenditures 2...
March 16, 2018: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/29527296/management-of-eight-labor-and-delivery-patients-dependent-on-buprenorphine-subutex%C3%A2-a-retrospective-chart-review
#9
Solina Tith, Garinder Bining, Laurent Bollag
Background : Opioid use during pregnancy is a growing concern in the United States. Buprenorphine has been recommended by "The American College of Obstetrics and Gynecology" as an alternative to methadone to decrease risks associated with the use of illicit opioids during pregnancy. The partial μ-opioid agonists' unique pharmacology, including its long half time and high affinity to the μ-opioid receptor, complicates patient management in a highly kinetic, and often urgent field like obstetric anesthesia...
2018: F1000Research
https://www.readbyqxmd.com/read/29523920/diminished-cardiac-performance-and-left-ventricular-dimensions-in-neonates-with-congenital-diaphragmatic-hernia
#10
Gabriel Altit, Shazia Bhombal, Krisa Van Meurs, Theresa A Tacy
INTRODUCTION: Newborns with congenital diaphragmatic hernia (CDH) have varying degrees of pulmonary hypoplasia and pulmonary hypertension (PH), and there is limited evidence that cardiac dysfunction is present. We sought to study early neonatal biventricular function and performance in these patients by reviewing early post-natal echocardiography (ECHO) measurements and comparing them to normal term newborns. METHODS: Retrospective case-control study reviewing clinical and ECHO data on term newborns with CDH and normal controls born between 2009 and 2016...
March 9, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29508361/surgical-chylothorax-in-neonates-management-and-outcomes
#11
Karina Miura Costa, Amulya Kumar Saxena
BACKGROUND: Postoperative chylothorax occurs due to trauma to lymphatic vessels and can occur after any thoracic procedure. This study reviewed recent literature to evaluate the management and outcomes of surgical chylothorax in neonates. METHODS: PubMed database was searched for articles in English, Portuguese and Spanish from 2000 to 2016. Data were collected for surgery, chylothorax management, complications, mortality and length of hospital stay (LOS). RESULTS: Twenty studies offered 107 neonates: congenital diaphragmatic hernia (CDH) (n = 76, 71%), cardiac malformations (n = 25, 23...
March 5, 2018: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/29502796/pulmonary-hypertension-in-congenital-diaphragmatic-hernia-patients-prognostic-markers-and-long-term-outcomes
#12
Matthew Wong, Janette Reyes, Eveline Lapidus-Krol, Monping Chiang, Tilman Humpl, Malikah Al-Faraj, Greg Ryan, Priscilla P L Chiu
Prenatal observed/expected lung-to-head ratio (O/E LHR) by ultrasound correlates with postnatal mortality for congenital diaphragmatic hernia (CDH) patients. The aim of this study is to determine if O/E LHR correlates with pulmonary hypertension (PH) outcomes for CDH patients. METHODS: A single center retrospective chart review was performed for CDH neonates from January 1, 2006, to December 31, 2015, (REB #1000053124) to include prenatal O/E LHR, liver position, first arterial blood gas, repair type, echocardiogram (ECHO), and lung perfusion scan (LPS) results up to 5years of age...
February 7, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29458230/perioperative-complications-of-congenital-diaphragmatic-hernia-repair
#13
Catarina Barroso, Jorge Correia-Pinto
Although improvements have been made, the management of congenital diaphragmatic hernia (CDH) remains a major challenge for perinatologists and neonatal surgeons. Many aspects of the disease remain unknown and, being a rare entity, evidence-based data are hard to find. Surgical morbidity is considerable and affects long-term quality of life. Perioperative complications have been reviewed focusing on thoracoscopic repair. Intraoperative acidosis was more severe during thoracoscopy when compared with open surgery (OS), though it is possible that later neurodevelopment was not affected...
February 19, 2018: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29448010/improved-outcomes-in-management-of-hypoplastic-left-heart-syndrome-associated-with-congenital-diaphragmatic-hernia-an-algorithmic-approach
#14
Kaitlin Balduf, T K Susheel Kumar, Umar Boston, Shyam Sathanandam, Marc V Lee, Tim Jancelewicz, Christopher J Knott-Craig
Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival rate of neonates with CDH and HLHS is only 1%-5%. We review our experience with CDH and HLHS and compare our outcomes with published literature. Retrospective review of all neonates with CDH and HLHS at our institution over a 10-year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies, and outcomes were reviewed, and an algorithmic approach was proposed...
February 12, 2018: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29420402/twenty-week-abortion-bans-on-pregnancies-with-a-congenital-diaphragmatic-hernia-a-cost-effectiveness-analysis
#15
Kimberley A Bullard, Brian L Shaffer, Karen S Greiner, Ashley E Skeith, Maria I Rodriguez, Aaron B Caughey
OBJECTIVE: To estimate the effect of 20-week abortion bans on maternal and consequent neonatal health outcomes and costs in the setting of fetal congenital diaphragmatic hernia. METHODS: A decision-analytic model was built using TreeAge software to evaluate the effect of a 20-week ban on abortion in a theoretical cohort of 921 women diagnosed with fetal congenital diaphragmatic hernia. Probabilities, utilities, and costs were derived from the literature. The cohort size was based on the annual rate of prenatal diagnoses of congenital diaphragmatic hernia and live births among the 20 states with bans...
March 2018: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/29390430/congenital-diaphragmatic-hernia-in-association-with-congenital-short-esophagus-a-case-report
#16
Bo Xia, Chun Hong, Jing Tang, Cuifen Liu, Gang Yu
RATIONALE: Congenital diaphragmatic hernia (CDH) associated congenital anomalies are present in about 25%. Congenital short esophagus (CSE) is a relatively rare condition. Both CDH and congenital intrathoracic stomach caused by CSE can be diagnosed in utero. However, CSE can be easily misdiagnosed in utero. PATIENT CONCERNS: We present a case of left CDH with CSE in a female neonate who was diagnosed at 24 weeks gestational age by antenatal ultrasound. DIAGNOSES: The neonate with CDH and congenital intrathoracic stomach due to CSE was confirmed by operation...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29338874/extracorporeal-membrane-oxygenation-in-infants-with-congenital-diaphragmatic-hernia
#17
REVIEW
Theresa R Grover, Natalie E Rintoul, Holly L Hedrick
Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly which impairs normal pulmonary development leading to acute and chronic respiratory failure, pulmonary hypoplasia, pulmonary hypertension, and mortality. CDH is the most common non-cardiac indication for neonatal ECMO. Prenatal and postnatal predictors of CDH severity aid in patient selection. Centers vary in preferred mode of ECMO and timing of CDH repair. Survivors of severe CDH with ECMO are at risk for long-term sequelae including neurodevelopmental delays...
March 2018: Seminars in Perinatology
https://www.readbyqxmd.com/read/29330547/recessive-loss-of-function-pign-alleles-including-an-intragenic-deletion-with-founder-effect-in-la-r%C3%A3-union-island-in-patients-with-fryns-syndrome
#18
Jean-Luc Alessandri, Christopher T Gordon, Marie-Line Jacquemont, Nicolas Gruchy, Norbert F Ajeawung, Guillaume Benoist, Myriam Oufadem, Asma Chebil, Yannis Duffourd, Coralie Dumont, Marion Gérard, Paul Kuentz, Thibaud Jouan, Francesca Filippini, Thi Tuyet Mai Nguyen, Olivier Alibeu, Christine Bole-Feysot, Patrick Nitschké, Asma Omarjee, Duksha Ramful, Hanitra Randrianaivo, Bérénice Doray, Laurence Faivre, Jeanne Amiel, Philippe M Campeau, Julien Thevenon
Fryns syndrome (FS) is a multiple malformations syndrome with major features of congenital diaphragmatic hernia, pulmonary hypoplasia, craniofacial dysmorphic features, distal digit hypoplasia, and a range of other lower frequency malformations. FS is typically lethal in the fetal or neonatal period. Inheritance is presumed autosomal recessive. Although no major genetic cause has been identified for FS, biallelic truncating variants in PIGN, encoding a component of the glycosylphosphatidylinositol (GPI)-anchor biosynthesis pathway, have been identified in a limited number of cases with a phenotype compatible with FS...
March 2018: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/29323665/arterial-tortuosity-syndrome-40-new-families-and-literature-review
#19
Aude Beyens, Juliette Albuisson, Annekatrien Boel, Mazen Al-Essa, Waheed Al-Manea, Damien Bonnet, Ozlem Bostan, Odile Boute, Tiffany Busa, Nathalie Canham, Ergun Cil, Paul J Coucke, Margot A Cousin, Majed Dasouki, Julie De Backer, Anne De Paepe, Sofie De Schepper, Deepthi De Silva, Koenraad Devriendt, Inge De Wandele, David R Deyle, Harry Dietz, Sophie Dupuis-Girod, Eudice Fontenot, Björn Fischer-Zirnsak, Alper Gezdirici, Jamal Ghoumid, Fabienne Giuliano, Neus Baena Diéz, Mohammed Z Haider, Joshua S Hardin, Xavier Jeunemaitre, Eric W Klee, Uwe Kornak, Manuel F Landecho, Anne Legrand, Bart Loeys, Stanislas Lyonnet, Helen Michael, Pamela Moceri, Shehla Mohammed, Laura Muiño-Mosquera, Sheela Nampoothiri, Karin Pichler, Katrina Prescott, Anna Rajeb, Maria Ramos-Arroyo, Massimiliano Rossi, Mustafa Salih, Mohammed Z Seidahmed, Elise Schaefer, Elisabeth Steichen-Gersdorf, Sehime Temel, Fahrettin Uysal, Marine Vanhomwegen, Lut Van Laer, Lionel Van Maldergem, David Warner, Andy Willaert, Tom R Collins, Andrea Taylor, Elaine C Davis, Yuri Zarate, Bert Callewaert
PurposeWe delineate the clinical spectrum and describe the histology in arterial tortuosity syndrome (ATS), a rare connective tissue disorder characterized by tortuosity of the large and medium-sized arteries, caused by mutations in SLC2A10.MethodsWe retrospectively characterized 40 novel ATS families (50 patients) and reviewed the 52 previously reported patients. We performed histology and electron microscopy (EM) on skin and vascular biopsies and evaluated TGF-β signaling with immunohistochemistry for pSMAD2 and CTGF...
January 11, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/29319634/hyperoxia-and-hypocapnia-during-pediatric-extracorporeal-membrane-oxygenation-associations-with-complications-mortality-and-functional-status-among-survivors
#20
Katherine Cashen, Ron Reeder, Heidi J Dalton, Robert A Berg, Thomas P Shanley, Christopher J L Newth, Murray M Pollack, David Wessel, Joseph Carcillo, Rick Harrison, J Michael Dean, Robert Tamburro, Kathleen L Meert
OBJECTIVES: To determine the frequency of hyperoxia and hypocapnia during pediatric extracorporeal membrane oxygenation and their relationships to complications, mortality, and functional status among survivors. DESIGN: Secondary analysis of data collected prospectively by the Collaborative Pediatric Critical Care Research Network. SETTING: Eight Collaborative Pediatric Critical Care Research Network-affiliated hospitals. PATIENTS: Age less than 19 years and treated with extracorporeal membrane oxygenation...
March 2018: Pediatric Critical Care Medicine
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