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https://www.readbyqxmd.com/read/27926976/are-cytokines-useful-biomarkers-to-determine-disease-severity-in-neonates-with-congenital-diaphragmatic-hernia
#1
Thomas Schaible, Janine Reineke, Ludwig Gortner, Dominik Monz, Regine Schaffelder, Erol Tutdibi
Background This study investigates plasma cytokine levels in neonates with the more common left-sided congenital diaphragmatic hernia (CDH) and correlates them with severity of disease indicated by position of the liver. An intrathoracic part of liver is associated with higher need for extracorporeal membrane oxygenation (ECMO) and higher risk for chronic lung disease (CLD). Methods A total of 28 newborns with CDH were subdivided by their liver position in partially intrathoracic (n = 16) and only abdominal (n = 12) position...
December 7, 2016: American Journal of Perinatology
https://www.readbyqxmd.com/read/27920046/resuscitation-of-infants-with-congenital-diaphragmatic-hernia
#2
Anthony O'Rourke-Potocki, Kamal Ali, Vadivelam Murthy, Anthony Milner, Anne Greenough
OBJECTIVE: To determine whether the respiratory response to resuscitation in infants with congenital diaphragmatic hernia (CDH) as measured by a respiratory function monitor differed between those who did and did not survive. DESIGN: Observational study. SETTING: Tertiary perinatal centre. PATIENTS: Thirty-eight infants born at 34 weeks of gestation or greater and diagnosed antenatally with a CDH. INTERVENTIONS: Expiratory tidal volume (VTe), peak inflation pressure (PIP) and end-tidal carbon dioxide level (ETCO2) were simultaneously recorded during resuscitation using a respiratory function monitor...
December 5, 2016: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/27912974/pulmonary-function-and-nutritional-morbidity-in-children-and-adolescents-with-congenital-diaphragmatic-hernia
#3
Beth Haliburton, Marialena Mouzaki, Monping Chiang, Vikki Scaini, Margaret Marcon, Wenming Duan, David Wilson, Priscilla Pl Chiu, Theo J Moraes
BACKGROUND: Malnutrition is common among congenital diaphragmatic hernia (CDH) survivors and may result from elevated respiratory effort. We evaluated body mass index (BMI), measured resting energy expenditure (mREE) and pulmonary function test (PFT) results in children and adolescents with CDH to determine if there is a correlation. METHODS: With ethics approval (REB# 1000035323), anthropometrics, indirect calorimetry (IC) results and PFTs were collected from patients 5-17years of age during CDH clinic visits between 2000 and 2016...
November 14, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27902503/bedside-placement-of-the-postpyloric-tube-in-infants
#4
Patricia Clifford, Elizabeth Ely, Lauren Heimall
BACKGROUND: Patients in the neonatal intensive care unit are a vulnerable population with specific nutritional requirements, which include increased protein and caloric needs for adequate growth. Some infants cannot tolerate gastric feeds and need to have postpyloric feeds to grow. Placement of a postpyloric tube can be done by gastric insufflation. Gastric insufflation is a technique where air is inserted into the stomach as a nasogastric tube is advanced through the pylorus to the duodenum...
November 29, 2016: Advances in Neonatal Care: Official Journal of the National Association of Neonatal Nurses
https://www.readbyqxmd.com/read/27894762/bilateral-congenital-diaphragmatic-hernia-prognostic-evaluation-of-a-large-international-cohort
#5
Sanne Mbi Botden, Kim Heiwegen, Iris Alm van Rooij, Horst Scharbatke, Pamela A Lally, Arno van Heijst, Ivo de Blaauw
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a lethal birth defect, which occurs in 1:2000-3000 live births. Bilateral CDH is a rare form (1%), with a high mortality. This study presents the outcomes of the largest cohort of bilateral CDH patients. METHODS: The records of patients with bilateral CDH from the Congenital Diaphragmatic Hernia Registry born between 1995 and 2015 were retrospectively analyzed to identify parameters associated with mortality. RESULTS: Eighty patients with a bilateral CDH were identified...
November 17, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27882737/neonatal-bowel-strangulation-rare-presentation-of-congenital-diaphragmatic-hernia
#6
Salvatore Arena, Gianfranco Scalfari, Nunzio Turiaco, Lucia Marseglia, Patrizia Perrone, Eloisa Gitto, Carmelo Romeo
We report a case of congenital diaphragmatic hernia (CDH) with perinatal bowel strangulation requiring intestinal resection. Ten hours after birth, the newborn started to be lethargic and developed bilious emesis. X-ray documented distended loops of bowel with air fluid levels in the abdomen and a gasless, non-homogeneous opacity of the left hemithorax, a right mediastinal shift and loss of the sharp left hemidiaphram line. On gastrographin enema the left colon was above the adjacent left diaphragm. Emergency surgery was performed at 16 h of age...
November 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27880037/suppressed-erythropoietin-expression-in-a-nitrofen-induced-congenital-diaphragmatic-hernia
#7
Hajime Takayasu, Koki Hagiwara, Kouji Masumoto
BACKGROUND: Erythropoietin (EPO), an essential stimulator of erythropoiesis produced by the fetal liver, is important both in vascular remodeling and modulation of the endothelial response in the pulmonary vasculature. In addition, EPO guides alveolar development, along with retinoic acid (RA). EPO is a direct target of RA, and the retinoid pathway is altered in the nitrofen-induced congenital diaphragmatic hernia (CDH) model. In the present study, we tested the hypothesis that the synthesis of EPO is suppressed in a rat model of CDH...
November 23, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27872786/congenital-diaphragmatic-hernia-with-delayed-presentation
#8
Alireza Malekzadegan, Alireza Sargazi
Congenital diaphragmatic hernia (CDH) is caused due to abnormal formation of the muscular parts of diaphragm. The incidence of CDH in common births ranges from 1/25000 to 1/30000. Pulmonary hypoplasia and pulmonary hypertension are factors that associate with the increase of mortality and morbidity due to CDH. We presented a 68-year-old Iranian woman with abdominal pain and tenderness in right upper quadrant who was diagnosed as having CDH. The disease was detected using chest X-ray and chest and abdomen sonography and confirmed with chest and abdomen CT scan with and without oral contrast...
2016: Case Reports in Surgery
https://www.readbyqxmd.com/read/27869490/congenital-diaphragmatic-hernia-causing-cardiac-arrest-in-a-30-year-old-woman
#9
H J Manson, Y M Goh, P Goldsmith, P Scott, P Turner
Congenital diaphragmatic hernia (CDH) usually presents in infancy with respiratory failure requiring urgent surgical correction. Mortality in this group of patients remains poor and persistent pulmonary hypertension is a significant contributor. It is therefore rare for patients to reach adulthood undiagnosed. CDH is often identified incidentally in adults but when symptoms arise, they relate to the organ involved, and include gastrointestinal symptoms of dyspepsia and obstruction, as well as respiratory complaints such as dyspnoea...
November 21, 2016: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/27864598/impact-of-fetal-counseling-on-outcome-of-antenatal-congenital-surgical-anomalies
#10
Shilpa Sharma, Ranjana Bhanot, Dipika Deka, Minu Bajpai, Devendra K Gupta
AIM: To analyze the impact of counseling on antenatal congenital surgical anomalies (ACSA). METHODS: Cases presenting with ACSA for fetal counseling and those presenting in post-natal period following diagnosis of ACSA (PACSA) for surgical opinion were analyzed for spectrum, presentation and outcome. RESULTS: 117 cases including ACSA(68);PACSA(49) were analyzed. Gestational age at diagnosis of ACSA;PACSA was 17-37;17-39 weeks (median 24;32 weeks)...
November 18, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27851982/interdomain-electron-transfer-in-cellobiose-dehydrogenase-is-governed-by-surface-electrostatics
#11
Alan Kadek, Daniel Kavan, Julien Marcoux, Johann Stojko, Alfons K G Felice, Sarah Cianférani, Roland Ludwig, Petr Halada, Petr Man
BACKGROUND: Cellobiose dehydrogenase (CDH) is a fungal extracellular oxidoreductase which fuels lytic polysaccharide monooxygenase with electrons during cellulose degradation. Interdomain electron transfer between the flavin and cytochrome domain in CDH, preceding the electron flow to lytic polysaccharide monooxygenase, is known to be pH dependent, but the exact mechanism of this regulation has not been experimentally proven so far. METHODS: To investigate the structural aspects underlying the domain interaction in CDH, hydrogen/deuterium exchange (HDX-MS) with improved proteolytic setup (combination of nepenthesin-1 with rhizopuspepsin), native mass spectrometry with ion mobility and electrostatics calculations were used...
November 13, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27837262/outcomes-of-congenital-diaphragmatic-hernia-repair-on-extracorporeal-life-support
#12
Jamie Golden, Nicole Jones, Jessica Zagory, Shannon Castle, David Bliss
PURPOSE: Extracorporeal life support (ECLS) is applied to refractory pulmonary hypertension in congenital diaphragmatic hernia (CDH). We evaluate the single-center outcomes of infants with CDH to determine the utility of late repair on ECLS versus repair post-decannulation. METHODS: Records of infants with CDH (2004-2014) were retrospectively reviewed. RESULTS: CDH was diagnosed in 177 infants. Sixty six (37%) underwent ECLS, of which, 11 died prior to repair, 33 were repaired post-decannulation, and 22 were repaired on ECLS...
November 11, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27836848/investigation-of-the-role-played-by-cellobiose-dehydrogenases-from-podospora-anserina-during-lignocellulose-degradation
#13
Narumon Tangthirasunun, David Navarro, Sona Garajova, Didier Chevret, Laetitia Chan Ho Tong, Valérie Gautier, Kevin D Hyde, Philippe Silar, Jean-Guy Berrin
: Conversion of biomass into high-value products including biofuels is of great interest to develop sustainable biorefineries. Fungi are an inexhaustible source of enzymes to degrade plant biomass. Cellobiose dehydrogenases (CDHs) play an important role in the breakdown through synergistic action with fungal lytic polysaccharide monooxygenases (LPMOs). The three CDH genes of the model fungus Podospora anserina were inactivated resulting in singly and multiple CDH mutants. We detected almost no difference in the growth and fertility of the mutants on various lignocellulose sources, except on crystalline cellulose, where a two-fold decrease in fertility of the mutants lacking PaCDH1 and PaCDH2 was observed...
November 11, 2016: Applied and Environmental Microbiology
https://www.readbyqxmd.com/read/27836357/does-the-ex-utero-intrapartum-treatment-to-extracorporeal-membrane-oxygenation-procedure-change-morbidity-outcomes-for-high-risk-congenital-diaphragmatic-hernia-survivors
#14
Hester F Shieh, Jay M Wilson, Catherine A Sheils, C Jason Smithers, Virginia S Kharasch, Ronald E Becker, Mollie Studley, Donna Morash, Terry L Buchmiller
PURPOSE: In high-risk congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before extracorporeal membrane oxygenation (ECMO) can be initiated. We previously examined ex utero intrapartum treatment (EXIT)-to-ECMO in our most severe CDH patients, but demonstrated no survival advantage. We now report morbidity outcomes in survivors of this high-risk cohort to determine whether EXIT-to-ECMO conferred any benefit. METHODS: All CDH survivors with <15% predicted lung volume (PPLV) from September 1999 to December 2010 were included...
October 25, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27833996/expression-of-t-box-transcription-factors-2-4-and-5-is-decreased-in-the-branching-airway-mesenchyme-of-nitrofen-induced-hypoplastic-lungs
#15
Toshiaki Takahashi, Florian Friedmacher, Julia Zimmer, Prem Puri
PURPOSE: Pulmonary hypoplasia (PH), characterized by smaller lung size and reduced airway branching, remains a major therapeutic challenge in newborns with congenital diaphragmatic hernia (CDH). T-box transcription factors (Tbx) have been identified as key components of the gene network that regulates fetal lung development. Tbx2, Tbx4 and Tbx5 are expressed throughout the mesenchyme of the developing lung, regulating the process of lung branching morphogenesis. Furthermore, lungs of Tbx2-, Tbx4- and Tbx5-deficient mice are hypoplastic and exhibit decreased lung branching, similar to PH in human CDH...
November 11, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27822781/decreased-endoglin-expression-in-the-pulmonary-vasculature-of-nitrofen-induced-congenital-diaphragmatic-hernia-rat-model
#16
Julia Zimmer, Toshiaki Takahashi, Alejandro D Hofmann, Prem Puri
AIM OF THE STUDY: Pulmonary hypertension (PH) remains a therapeutical challenge in neonates born with congenital diaphragmatic hernia (CDH). Endoglin (Eng), an auxiliary receptor component of the transforming growth factor β (TGFβ) signalling pathway, is expressed mainly by endothelial cells and has been found to be involved in angiogenesis and vascular remodelling. Genetic studies have linked TGFβ and Eng mutations to human arterial PH and other cardiovascular syndromes. Eng interacts with the TGFβ receptors 1 and 2 (Tgfβr1, Tgfβr2)...
November 7, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27822779/appropriate-timing-of-surgery-for-neonates-with-congenital-diaphragmatic-hernia-early-or-delayed-repair
#17
Hiroomi Okuyama, Noriaki Usui, Masahiro Hayakawa, Tomoaki Taguchi
PURPOSE: This study was aimed to evaluate the influence of timing of surgery on patient outcomes, and to clarify appropriate timing of surgery in neonates with congenital diaphragmatic hernia (CDH). METHODS: A total of 477 neonates with isolated CDH were included. Patients were classified into two groups by timing of surgery: early repair (ER) (≤48 h) and delayed repair (DR) (>48 h). The primary outcome was 90-day survival, with treatment duration (ventilation, oxygen, and hospitalization) being a secondary outcome...
November 7, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27821174/eosinophilic-esophagitis-after-congenital-diaphragmatic-hernia
#18
Amelia Licari, Riccardo Castagnoli, Gian Luigi Marseglia
BACKGROUND: Eosinophilic esophagitis (EoE) is an increasingly diagnosed disease, especially in the western world. Although its pathogenesis remains poorly understood, there is strong evidence that the eosinophilic inflammation in EoE is primarily triggered by immune dysregulation secondary to allergic sensitization to dietary or aero-allergens. Recent studies have reported a higher prevalence of EoE in children with congenital gastrointestinal malformations, i.e. esophageal atresia and/or tracheoesophageal fistula...
November 8, 2016: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/27796650/the-value-of-short-term-pain-relief-in-predicting-the-1-month-outcome-of-indirect-cervical-epidural-steroid-injections
#19
Holger Joswig, Armin Neff, Christina Ruppert, Gerhard Hildebrandt, Martin Nikolaus Stienen
BACKGROUND: Clinical management after epidural steroid injections (ESI) of patients with radiculopathy secondary to a cervical disc herniation (CDH) is uncertain. This study aims to determine whether short-term arm pain alleviation following computed tomography-guided 'indirect' cervical ESI can predict the 1-month outcome. METHODS: We conducted a prospective observation of 45 consecutive patients at a tertiary radiological department. Study components were visual analog scale arm and neck pain at baseline, 15, 30, and 45 min, 1, 2, and 4 h, on days 1-14, 1 month, and at 1 year...
October 28, 2016: Acta Neurochirurgica
https://www.readbyqxmd.com/read/27780277/hernia-sac-presence-portends-better-survivability-of-isolated-congenital-diaphragmatic-hernia-with-liver-up
#20
Ruža Grizelj, Katarina Bojanić, Jurica Vuković, Milivoj Novak, Toby N Weingarten, Darrell R Schroeder, Juraj Sprung
Objective The objective of this study was to investigate the prognostic value of a hernia sac in isolated congenital diaphragmatic hernia (CDH) with intrathoracic liver herniation ("liver-up"). Study Design A retrospective study from the single tertiary center. Isolated "liver-up" CDH neonates referred to our institution between 2000 and 2015 were reviewed for the presence or absence of a hernia sac. Association between the presence of a hernia sac and survival was assessed. Results Over the study period, there were 29 isolated CDH patients with "liver-up" who were treated, 7 (24%) had a sac, and 22 (76%) did not...
October 25, 2016: American Journal of Perinatology
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