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Congenital diaphragmatic hernia

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https://www.readbyqxmd.com/read/28710247/congenital-diaphragmatic-hernia-and-growth-to-12-years
#1
Lisette Leeuwen, Daphne S Mous, Joost van Rosmalen, Joanne F Olieman, Laura Andriessen, Saskia J Gischler, Koen F M Joosten, Rene M H Wijnen, Dick Tibboel, Hanneke IJsselstijn, Marjolein Spoel
OBJECTIVES: Growth problems are reported in patients with congenital diaphragmatic hernia during the first years of life. However, it is unknown if poor growth persists during childhood. We therefore evaluated growth of patients longitudinally until 12 years of age. METHODS: This prospective study included 172 patients (43 treated with extracorporeal membrane oxygenation [ECMO]) born from 1999 to 2014. Z scores of height-for-age (HFA), weight-for-height, and distance-to-target height were calculated at 6 months of age and at 1, 2, 5, 8, and 12 years of age...
July 14, 2017: Pediatrics
https://www.readbyqxmd.com/read/28706241/abnormal-paraventricular-nucleus-of-hypothalamus-and-growth-retardation-associated-with-loss-of-nuclear-receptor-gene-coup-tfii
#2
Su Feng, Can Xing, Tingyu Shen, Yunbo Qiao, Ran Wang, Jun Chen, Jiaoyang Liao, Zhuo Lu, Xiong Yang, Saber Mohamed Abd-Allah, Jinsong Li, Naihe Jing, Ke Tang
The paraventricular nucleus of hypothalamus plays important roles in the regulation of energy balance and fetal growth. However, the molecular mechanisms underlying its formation and function have not been clearly elucidated. Various mutations in the human COUP-TFII gene, which encodes a nuclear receptor, result in growth retardation, congenital diaphragmatic hernia and congenital heart defects. Here, we show that COUP-TFII gene is expressed in the developing hypothalamus in mouse. The ventral forebrain-specific RXCre/+; COUP-TFII (F/F) mutant mice display growth retardation...
July 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28701846/rare-presentation-of-congenital-diaphragmatic-hernia-in-a-sexagenarian
#3
C Danny Darlington, G Fatima Shirly Anitha
Congenital diaphragmatic hernia (CDH) usually presents in the neonatal period, and about 10% of reported cases occur in adults. The most common type is Bochdalek's hernia, which occurs through a defect in the posterolateral portion of the diaphragm with an estimated prevalence of 1 in 2500 live births. CDH in adults presents with gastrointestinal or respiratory symptoms, which can be acute or intermittent. We report a case of CDH diagnosed in a 55-year-old man, who presented with acute onset of chest pain and dyspnea with insignificant past history...
June 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28701001/downregulated-elastin-microfibril-interfacer-1-expression-in-the-pulmonary-vasculature-of-experimental-congenital-diaphragmatic-hernia
#4
Julia Zimmer, Toshiaki Takahashi, Alejandro D Hofmann, Prem Puri
No abstract text is available yet for this article.
July 12, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28698228/perturbations-to-lysyl-oxidase-expression-broadly-influence-the-transcriptome-of-lung-fibroblasts
#5
Ivana Mižíková, Francesco Palumbo, Tamás Tábi, Susanne Herold, István Vadász, Konstantin Mayer, Werner Seeger, Rory E Morty
Lysyl oxidases are credited with pathogenic roles in lung diseases, including cancer, fibrosis, pulmonary hypertension, congenital diaphragmatic hernia, and bronchopulmonary dysplasia (BPD). Lysyl oxidases facilitate the covalent intra- and inter-molecular cross-linking of collagen and elastin fibers, thereby imparting tensile strength to the extracellular matrix (ECM). Alternative ECM-independent roles have recently been proposed for lysyl oxidases, including regulation of growth factor signaling, chromatin remodeling, and transcriptional regulation; all of which impact cell phenotype...
July 10, 2017: Physiological Genomics
https://www.readbyqxmd.com/read/28695234/pre-and-neonatal-imaging-of-gastrointestinal-complications-in-congenital-diaphragmatic-hernia
#6
REVIEW
Katharina Minkner, Leonor Alamo
The initial outcome in infants with congenital diaphragmatic hernia is mainly related to the associated lung hypoplasia. However, these patients frequently present with additional gastrointestinal pathology that also influences their quality of life and final prognosis. Congenital gastrointestinal anomalies are often observed and the displacement of the liver, the stomach and/or the intestines into the thorax may cause distortion of the vascular axis of these organs, increasing the risk of congestion and/or ischemia...
July 10, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28685301/steroid-use-for-refractory-hypotension-in-congenital-diaphragmatic-hernia
#7
Jason O Robertson, Cory N Criss, Lily B Hsieh, Niki Matsuko, Josh S Gish, Rodrigo A Mon, Kevin N Johnson, Samir K Gadepalli
PURPOSE: Guidelines for diagnosis and treatment of adrenal insufficiency (AI) in newborns with congenital diaphragmatic hernia (CDH) are poorly defined. METHODS: From 2002 to 2016, 155 infants were treated for CDH at our institution. Patients with shock refractory to vasopressors (clinically diagnosed AI) were treated with hydrocortisone (HC). When available, random cortisol levels <10 μg/dL were considered low. Outcomes were compared between groups. RESULTS: Hydrocortisone was used to treat AI in 34% (53/155) of patients...
July 6, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28669608/evaluation-of-neonatal-lung-volume-growth-by-pulmonary-magnetic-resonance-imaging-in-patients-with-congenital-diaphragmatic-hernia
#8
Melissa A Schopper, Laura L Walkup, Jean A Tkach, Nara S Higano, Foong Yen Lim, Beth Haberman, Jason C Woods, Paul S Kingma
OBJECTIVE: To evaluate postnatal lung volume in infants with congenital diaphragmatic hernia (CDH) and determine if a compensatory increase in lung volume occurs during the postnatal period. STUDY DESIGN: Using a novel pulmonary magnetic resonance imaging method for imaging neonatal lungs, the postnatal lung volumes in infants with CDH were determined and compared with prenatal lung volumes obtained via late gestation magnetic resonance imaging. RESULTS: Infants with left-sided CDH (2 mild, 9 moderate, and 1 severe) were evaluated...
June 29, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28666768/strategies-for-improving-early-nutritional-outcomes-in-children-with-oesophageal-atresia-and-congenital-diaphragmatic-hernia
#9
REVIEW
Dominic A Fitzgerald, Andrea Kench, Lucy Hatton, Jonathan Karpelowsky
Post-natal growth in surgical lung conditions, such as congenital diaphragmatic hernia and oesophageal atresia with tracheo-oesophageal fistula, is often sub-optimal in the early years of life when lung growth is occurring. Whilst constitutional, behavioural and mechanical factors may contribute to poor feeding and weight gain, there is a common path of management with greater caloric supplementation that may change growth trajectories and potentially lead to better respiratory, anthropometric and cognitive outcomes...
May 31, 2017: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28660829/incarcerated-bochdalek-hernia-causing-bowel-obstruction-in-an-adult-male-patient
#10
Emnp Ekanayake, S A Fernando, P L Durairajah, Jasb Jayasundara
Bochdalek hernias occur as a result of congenital fusion failure of the posterolateral muscular portion of the diaphragm derived from the embryonic pleuroperitoneal membrane. The vast majority of symptomatic Bochdalek hernias are diagnosed during the neonatal period. Congenital diaphragmatic hernias showing symptoms in adulthood are rare. We report the case of a 28-year-old man who presented with subacute bowel obstruction, later diagnosed to have a left-sided incarcerated Bochdalek hernia containing an ischaemic splenic flexure and the distal transverse colon...
July 2017: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/28652420/cerebral-perfusion-after-repair-of-congenital-diaphragmatic-hernia-with-common-carotid-artery-occlusion-after-ecmo-therapy
#11
Claudia Henzler, Frank G Zöllner, Meike Weis, Fabian Zimmer, Stefan O Schoenberg, Katrin Zahn, Thomas Schaible, K Wolfgang Neff
AIM: To prospectively evaluate cerebral perfusion after repair of congenital diaphragmatic hernia (CDH) and right-common-carotid-artery (rCCA) occlusion after extracorporeal membrane oxygenation (ECMO) therapy. PATIENTS AND METHODS: A total of 29 2-year-old-children with a history of CDH repair underwent cerebral magnetic resonance perfusion imaging. In 14 patients, the rCCA was occluded after ECMO therapy. Fifteen patients with CDH without ECMO served as controls...
July 2017: In Vivo
https://www.readbyqxmd.com/read/28650950/neurobiologic-correlates-of-attention-and-memory-deficits-following-critical-illness-in-early-life
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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