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Congenital hernia

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https://www.readbyqxmd.com/read/28650950/neurobiologic-correlates-of-attention-and-memory-deficits-following-critical-illness-in-early-life
#1
Raisa M Schiller, Hanneke IJsselstijn, Marlous J Madderom, André B Rietman, Marion Smits, Arno F J van Heijst, Dick Tibboel, Tonya White, Ryan L Muetzel
OBJECTIVES: Survivors of critical illness in early life are at risk of long-term-memory and attention impairments. However, their neurobiologic substrates remain largely unknown. DESIGN: A prospective follow-up study. SETTING: Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands. PATIENTS: Thirty-eight school-age (8-12 yr) survivors of neonatal extracorporeal membrane oxygenation and/or congenital diaphragmatic hernia with an intelligence quotient greater than or equal to 80 and a below average score (z score ≤ -1...
June 23, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28641757/long-term-follow-up-of-congenital-diaphragmatic-hernia
#2
Laura E Hollinger, Matthew T Harting, Kevin P Lally
Increased survival of patients with congenital diaphragmatic hernia has created a unique cohort of children, adolescent, and adult survivors with complex medical and surgical needs. Disease-specific morbidities offer the opportunity for multiple disciplines to unite together to provide long-term comprehensive follow-up, as well as an opportunity for research regarding late outcomes. These children can exhibit impaired pulmonary function, altered neurodevelopmental outcomes, nutritional insufficiency, musculoskeletal changes, and specialized surgical needs that benefit from regular monitoring and intervention, particularly in patients with increased disease severity...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641756/regenerative-medicine-solutions-in-congenital-diaphragmatic-hernia
#3
Paolo De Coppi, Jan Deprest
Congenital diaphragmatic hernia (CDH) remains a major challenge and associated mortality is still significant. Patients have benefited from current therapeutic options, but most severe cases are still associated to poor outcome. Regenerative medicine is emerging as a valid option in many diseases and clinical trials are currently happening for various conditions in children and adults. We report here the advancement in the field which will help both in the understanding of further CDH development and in offering new treatment options for the difficult situations such as repair of large diaphragmatic defects and lung hypoplasia...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641755/ecmo-in-cdh-is-there-a-role
#4
David W Kays
Despite wide use and decades of experience, survival of congenital diaphragmatic hernia (CDH) patients treated with extra-corporeal membrane oxygenation (ECMO), as reported by the extra-corporeal life support organization (ELSO), remains unchanged at 50%. High-survival rates both with and without utilizing ECMO have been reported, fueling questions about the utility of ECMO support in this difficult population. This review looks at data from the Congenital Diaphragmatic Hernia Study Group and individual center reports, to evaluate the role of ECMO in CDH, focusing on defining the patients most likely to benefit, and discussing how those benefits can best be achieved...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641754/ventilation-modalities-in-infants-with-congenital-diaphragmatic-hernia
#5
Francesco Morini, Irma Capolupo, Willem van Weteringen, Irwin Reiss
Neonates with congenital diaphragmatic hernia are among the more complex patients to support with mechanical ventilation. They have particular features that add to the difficulties already present in the neonatal patient. A ventilation strategy tailored to the patient's underlying physiology rather than mode of ventilation is a crucial issue for clinicians treating these delicate patients.
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641753/cardiac-dysfunction-in-congenital-diaphragmatic-hernia-pathophysiology-clinical-assessment-and-management
#6
Neil Patel, Florian Kipfmueller
Cardiac dysfunction is an important consequence of pulmonary hypertension in congenital diaphragmatic hernia and a determinant of disease severity. Increased afterload leads to right ventricular dilatation and diastolic dysfunction. Septal displacement and dysfunction impair left ventricular function, which may also be compromised by fetal hypoplasia. Biventricular failure contributes to systemic hypotension and hypoperfusion. Early and regular echocardiographic assessment of cardiac function and pulmonary artery pressure can guide therapeutic decision-making, including choice and timing of pulmonary vasodilators, cardiotropes, ECMO, and surgery...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641752/congenital-diaphragmatic-hernia-associated-pulmonary-hypertension
#7
Matthew T Harting
Congenital diaphragmatic hernia (CDH) is a complex entity wherein a diaphragmatic defect allows intrathoracic herniation of intra-abdominal contents and both pulmonary parenchymal and vascular development are stifled. Pulmonary pathology and pathophysiology, including pulmonary hypoplasia and pulmonary hypertension, are hallmarks of CDH and are associated with disease severity. Pulmonary hypertension (PH) is sustained, supranormal pulmonary arterial pressure, and among patients with CDH (CDH-PH), is driven by hypoplastic pulmonary vasculature, including alterations at the molecular, cellular, and tissue levels, along with pathophysiologic pulmonary vasoreactivity...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641751/fetal-imaging-and-therapy-for-cdh-current-status
#8
Titilayo Oluyomi-Obi, Tim Van Mieghem, Greg Ryan
In congenital diaphragmatic hernia (CDH), herniation of the abdominal organs into the fetal chest causes pulmonary hypoplasia and pulmonary hypertension, the main causes of neonatal mortality. As antenatal ultrasound screening improves, the risk of postnatal death can now be better predicted, allowing for the identification of fetuses that might most benefit from a prenatal intervention. Fetoscopic tracheal occlusion is being evaluated in a large international randomized controlled trial. We present the antenatal imaging approaches that can help identify fetuses that might benefit from antenatal therapy, and review the evolution of fetal surgery for CDH to date...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641750/predicting-outcomes-in-congenital-diaphragmatic-hernia
#9
Oluwatomilayo Daodu, Mary E Brindle
Identification of CDH infant populations at high risk for mortality postnatally may help to develop targeted care strategies, guide discussions surrounding palliation and contribute to standardizing reporting and benchmarking, so that care strategies at different centers can be compared. Clinical prediction rules are evidence-based tools that combine multiple predictors to estimate the probability that a particular outcome in an individual patient will occur. In CDH, a suitable clinical prediction rule can stratify high- and low-risk populations and provide the ability to tailor management strategies based on severity...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641749/congenital-diaphragmatic-hernia-the-role-of-multi-institutional-collaboration-and-patient-registries-in-supporting-best-practice
#10
Pamela A Lally, Erik D Skarsgard
Among congenital malformations, congenital diaphragmatic hernia (CDH) is distinguished by its relatively low occurrence rate, need for resource intensive, integrated multidisciplinary care, and widespread variation in practice and outcome. Although randomized controlled trials (RCTs) are considered the gold standard for generating evidence, they are poorly suited to the study of a condition like CDH due to challenges in illness severity adjustment, unpredictability in clinical course and the impact limitations of studying a single intervention at a time...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641748/abnormal-lung-development-in-congenital-diaphragmatic-hernia
#11
Dustin Ameis, Naghmeh Khoshgoo, Richard Keijzer
The outcomes of patients diagnosed with congenital diaphragmatic hernia (CDH) have recently improved. However, mortality and morbidity remain high, and this is primarily caused by the abnormal lung development resulting in pulmonary hypoplasia and persistent pulmonary hypertension. The pathogenesis of CDH is poorly understood, despite the identification of certain candidate genes disrupting normal diaphragm and lung morphogenesis in animal models of CDH. Defects within the lung mesenchyme and interstitium contribute to disturbed distal lung development...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28631351/intraoperative-acidosis-and-hypercapnia-during-thoracoscopic-repair-of-congenital-diaphragmatic-hernia-and-esophageal-atresia-tracheoesophageal-fistula
#12
Augusto Zani, Ruben Lamas-Pinheiro, Irene Paraboschi, Sebastian K King, Justyna Wolinska, Elke Zani-Ruttenstock, Simon Eaton, Agostino Pierro
BACKGROUND: Intraoperative hypercapnia and acidosis have been associated with thoracoscopic repair of both congenital diaphragmatic hernia and esophageal atresia/tracheoesophageal fistula. AIM: The aim of the present study was to investigate whether thoracoscopic repair of congenital diaphragmatic hernia or esophageal atresia/tracheoesophageal fistula was associated with acidosis and hypercapnia in a large group of neonates, and to analyze the effects of acidosis and hypercapnia on early postoperative outcomes...
June 20, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28629240/foetoscopic-endotracheal-occlusion-feto-for-severe-isolated-left-sided-congenital-diaphragmatic-hernia-single-centre-polish-experience
#13
Przemyslaw Kosinski, Miroslaw Wielgos
OBJECTIVE: To present early experience with foetoscopic endotracheal occlusion (FETO) for congenital diaphragmatic hernia (CDH) in a new centre in Poland. METHODS: This was a prospective study in singleton pregnancies with CDH treated by FETO between 2014 and 2016 in the Medical University of Warsaw, Poland. FETO was carried out at 25.6-30.1 (median 27.7) weeks' gestation in 28 consecutive cases of isolated left-sided CDH with observed over expected lung area to head circumference ratio (o/e LHR) of 20...
June 20, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28622971/utilization-patterns-of-extracorporeal-membrane-oxygenation-in-neonates-in-the-united-states-1997-2012
#14
Ashley Y Song, Hsuan-Hsiu Annie Chen, Rachel Chapman, Ameish Govindarajan, Jeffrey S Upperman, Rita V Burke, James Stein, Philippe S Friedlich, Ashwini Lakshmanan
BACKGROUND: Extracorporeal membrane oxygenation (ECMO) remains one of the most intensive therapies for newborns in the United States. However, there is limited information on resource utilization for neonates receiving ECMO. METHODS: We conducted a retrospective data analysis of the Kids' Inpatient Database from 1997 to 2012. Bivariate and multivariate analysis was completed to identify predictors of LOS, hospital costs and mortality. Cardiac and non-cardiac diagnoses of neonates receiving ECMO were also included in the bivariate and multivariable analysis...
June 1, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28620733/management-of-congenital-diaphragmatic-hernia-in-newborn-paradigm-shift-and-ethical-issues
#15
Sushmita Nitin Bhatnagar, Yogesh Kumar Sarin
Management of congenital diaphragmatic hernia (CDH) begins soon after it is detected, whether antenatally or postnatally. Assessment of the severity of the condition, associated congenital anomalies, maternal health and related issues, weight of the fetus/baby, mode of delivery, timing of delivery, immediate appropriate management of the baby with CDH at birth, appropriate utilization of available treatment modalities as well as infrastructure of the treating institute have an impact on the outcome of the neonate...
June 16, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28611552/a-novel-mutation-in-pitx2-in-a-patient-with-axenfeld-rieger-syndrome
#16
Susan J Hassed, Shibo Li, Weihong Xu, Ashley C Taylor
Axenfeld-Rieger syndrome is a rare autosomal dominant condition. Anomalies include anterior segment dysgenesis of the eye, dental anomalies, maxillary hypoplasia, periumbilical anomalies, and congenital heart defects. We report a patient with Peters anomaly, dysmorphic features, congenital heart defect, umbilical hernia, short stature, and developmental delay. Diagnostic sequencing of 23 genes known to be causally related to the condition was performed on the patient, parents, and maternal grandparents. A variant of uncertain significance in PITX2 was identified...
March 2017: Molecular Syndromology
https://www.readbyqxmd.com/read/28609176/mothers-of-infants-with-congenital-diaphragmatic-hernia-describe-breastfeeding-in-the-neonatal-intensive-care-unit-as-long-as-it-s-my-milk-i-m-happy
#17
Elizabeth B Froh, Janet A Deatrick, Martha A Q Curley, Diane L Spatz
BACKGROUND: Very little is known about the breastfeeding experience of mothers of infants born with congenital anomalies and cared for in the neonatal intensive care unit (NICU). Often, studies related to breastfeeding and lactation in the NICU setting are focused on the mothers of late preterm, preterm, low-birth-weight, and very-low-birth-weight infants. Congenital diaphragmatic hernia (CDH) is an anatomic malformation of the diaphragm and affects 1 in every 2,000 to 4,000 live births...
June 1, 2017: Journal of Human Lactation: Official Journal of International Lactation Consultant Association
https://www.readbyqxmd.com/read/28607625/hepatic-portal-venous-gas-in-children-younger-than-2-years-old-radiological-and-clinical-characteristics-in-diseases-other-than-necrotizing-enterocolitis
#18
Marzena Barczuk-Falęcka, Przemysław Bombiński, Zofia Majkowska, Michał Brzewski, Stanisław Warchoł
BACKGROUND: Hepatic portal venous gas (HPVG) is a rare imaging finding in children. It can be an important manifestation of severe diseases such as necrotizing enterocolitis (NEC) in neonates or bowel wall rupture in older children. However, there are many other diseases presenting with HPVG that do not necessarily require a surgical intervention. CASE REPORT: In the period between 2011-2015, there were 12 cases of HPVG in children aged up to 24 months in our hospital...
2017: Polish Journal of Radiology
https://www.readbyqxmd.com/read/28607284/a-comparison-of-clinical-outcomes-between-endoscopic-and-open-surgery-to-repair-neonatal-diaphragmatic-hernia
#19
Ma Lishuang, Wei Yandong, Liu Shuli, Feng Cuiru, Zhang Yue, Wang Ying, Zhang Yanxia, Sun Bin, Li Jingna, Li Long
OBJECTIVE: The objective of this study is to evaluate the clinical efficacies of open versus endoscopic surgery in the treatment of congenital diaphragmatic hernia (CDH) and investigate the feasibility and safety of endoscopic surgery as an alternative to open surgery in these cases. PATIENTS AND METHODS: A retrospective analysis was performed from June 2002 to February 2014. A total of 59 cases were attempted. The neonates were divided into either an endoscopic or open surgery group...
July 2017: Journal of Minimal Access Surgery
https://www.readbyqxmd.com/read/28602525/factors-related-to-long-term-surgical-morbidity-in-congenital-diaphragmatic-hernia-survivors
#20
Stan Janssen, Kim Heiwegen, Iris Alm van Rooij, Horst Scharbatke, Jolt Roukema, Ivo de Blaauw, Sanne Mbi Botden
BACKGROUND: Patients born with a congenital diaphragmatic hernia (CDH) have a high mortality and morbidity. After discharge, complications and long-term morbidity are still encountered. This study describes the factors related to the surgical long-term outcomes in CDH survivors. METHODS: A cohort of CDH patients born between 2000 and 2014, with a minimum of two years follow up, were included in this retrospective study. Demographics, CDH specific characteristics, treatment, and long-term surgical outcome were evaluated using multivariate logistic regression analyses...
June 3, 2017: Journal of Pediatric Surgery
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