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https://www.readbyqxmd.com/read/28331629/congenital-diaphragmatic-hernia-a-review
#1
REVIEW
Praveen Kumar Chandrasekharan, Munmun Rawat, Rajeshwari Madappa, David H Rothstein, Satyan Lakshminrusimha
Congenital Diaphragmatic hernia (CDH) is a condition characterized by a defect in the diaphragm leading to protrusion of abdominal contents into the thoracic cavity interfering with normal development of the lungs. The defect may range from a small aperture in the posterior muscle rim to complete absence of diaphragm. The pathophysiology of CDH is a combination of lung hypoplasia and immaturity associated with persistent pulmonary hypertension of newborn (PPHN) and cardiac dysfunction. Prenatal assessment of lung to head ratio (LHR) and position of the liver by ultrasound are used to diagnose and predict outcomes...
2017: Maternal Health, Neonatology and Perinatology
https://www.readbyqxmd.com/read/28324716/postnatal-mri-for-cdh-a-pictorial-review-of-late-presenting-and-recurrent-diaphragmatic-defects
#2
REVIEW
Wendy Kim, Jesse Courtier, Cara Morin, Narendra Shet, Eric Strauch, Jane S Kim
Late-presenting or recurrent diaphragmatic defects can pose a diagnostic challenge due to varying clinical presentations. Current diagnostic approaches include plain film radiograph for initial assessment, with other imaging modalities such as fluoroscopy, ultrasound, CT and MRI mainly utilized for troubleshooting. As a radiation-free modality, MRI can provide a more definitive diagnosis in particular cases due to its ability to visualize discontinuity of the diaphragm, distinguishing it from eventration. MRI can also accurately characterize hernia contents, defect location and size...
March 8, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28321802/sepsis-risk-factors-in-infants-with-congenital-diaphragmatic-hernia
#3
Michaël Levy, Nolwenn Le Sache, Mostafa Mokhtari, Guy Fagherazzi, Gaelle Cuzon, Benjamin Bueno, Virginie Fouquet, Alexandra Benachi, Sergio Eleni Dit Trolli, Pierre Tissieres
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly and remains among the most challenging ICU-managed disease. Beside severe pulmonary hypertension, lung hypoplasia and major abdominal surgery, infective complications remain major determinants of outcome. However, the specific incidence of sepsis as well as associated risk factors is unknown. METHODS: This prospective, 4-year observational study took place in the pediatric intensive care and neonatal medicine department of the Paris South University Hospitals (Le Kremlin-Bicêtre, France), CDH national referral center and involved 62 neonates with CDH...
December 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/28319942/right-congenital-diaphragmatic-hernias-is-there-a-correlation-between-prenatal-lung-volume-and-postnatal-survival-as-in-isolated-left-diaphragmatic-hernias
#4
Teresa Victoria, Enrico Danzer, Edward R Oliver, J Christopher Edgar, Suzanne Iyoob, Emily A Partridge, Ann M Johnson, William H Peranteau, Beverly G Coleman, Alan W Flake, Mark P Johnson, Holly H Hedrick, N Scott Adzick
OBJECTIVES: Whereas left-sided congenital diaphragmatic hernias (L-CDH) have been extensively studied and their prognostic parameters delineated, right-sided hernias (R-CDH) have not. Published results remain inconclusive. The aim of this study is to evaluate if proven prognostic indicators of postnatal survival in the fetus with L-CDH apply to the fetus with R-CDH. METHODS: Retrospective single-center study of R-CDH fetuses with available prenatal studies assessed for fetal lung volume by means of ultrasound-measured observed versus expected (O/E) lung area to head circumference (LHR) and magnetic resonance-calculated O/E total lung volume (TLV) in a 12-year time period...
March 21, 2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28303347/genome-wide-enrichment-of-damaging-de-novo-variants-in-patients-with-isolated-and-complex-congenital-diaphragmatic-hernia
#5
Mauro Longoni, Frances A High, Hongjian Qi, Maliackal P Joy, Regis Hila, Caroline M Coletti, Julia Wynn, Maria Loscertales, Linshan Shan, Carol J Bult, Jay M Wilson, Yufeng Shen, Wendy K Chung, Patricia K Donahoe
Congenital Diaphragmatic Hernia (CDH) is a common and often lethal birth defect characterized by diaphragmatic structural defects and pulmonary hypoplasia. CDH is isolated in 60% of newborns, but may also be part of a complex phenotype with additional anomalies. We performed whole exome sequencing (WES) on 87 individuals with isolated or complex CDH and on their unaffected parents, to assess the contribution of de novo mutations in the etiology of diaphragmatic and pulmonary defects and to identify new candidate genes...
March 16, 2017: Human Genetics
https://www.readbyqxmd.com/read/28302163/matrix-metalloproteinase-activity-stimulates-n-cadherin-shedding-and-the-soluble-n-cadherin-ectodomain-promotes-classical-microglial-activation
#6
Katherine Conant, Stefano Daniele, P Lorenzo Bozzelli, Tsion Abdi, Amanda Edwards, Arek Szklarczyk, India Olchefske, David Ottenheimer, Kathleen Maguire-Zeiss
BACKGROUND: Matrix metalloproteinases (MMPs) are a family of enzymes that are typically released from intracellular stores to act on specific extracellular substrates. MMP expression and activity can be increased in a neuronal activity-dependent manner, and further increased in response to tissue injury. MMP substrates include cell adhesion molecules (CAMs) that are abundantly expressed in the brain and well positioned for membrane proximal cleavage. Importantly, CAM integrity is important to synaptic structure and axon-myelin interactions, and shed ectodomains may themselves influence cellular function...
March 17, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28301893/congenital-diaphragmatic-hernia-the-side-of-diaphragmatic-defect-and-associated-nondiaphragmatic-malformations
#7
Ruža Grizelj, Katarina Bojanić, Jurica Vuković, Toby N Weingarten, Darrell R Schroeder, Juraj Sprung
Background Congenital diaphragmatic hernia (CDH) has different clinical presentations depending on whether it is right sided (R-CDH) or left sided (L-CDH). Some have suggested that L-CDH and R-CDH may represent different syndromic phenotypes. This theory would be indirectly supported if different nondiaphragmatic anomalies were associated with laterality. We assessed whether CDH laterality is associated with specific types of nondiaphragmatic anomalies. Methods Cases of CDH were retrospectively identified from five centers, and associated congenital anomalies, prenatal diagnosis, demographics, birth characteristics, and side of the CDH were analyzed...
March 16, 2017: American Journal of Perinatology
https://www.readbyqxmd.com/read/28295742/prognosis-of-isolated-congenital-diaphragmatic-hernia-using-lung-to-head-circumference-ratio-variability-across-centers-in-a-national-perinatal-network
#8
Marie-Victoire Senat, Hanane Bouchghoul, Julien Stirnemann, Pascal Vaast, Julia Boubnova, Laetitia Begue, Elizabeth Carricaburu, Agnes Sartor, Jacques Jani, Alexandra Benachi, Jean Bouyer
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly. The observed to expected lung area-to-head circumference ratio (o/e-LHR) has been shown to provide a useful prediction of subsequent survival in fetuses with CDH in reference centers with expertise and a large caseload. However, the accuracy of o/e-LHR measurements in unselected fetal medicine units or in centers with less expertise is not well known. OBJECTIVE: To evaluate o/e-LHR measurement in prognostic evaluation of postnatal survival when the measurement is performed in fetal medicine units with different levels of expertise...
March 13, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28277913/observed-to-expected-mri-fetal-lung-volume-can-predict-long-term-lung-morbidity-in-infants-with-congenital-diaphragmatic-hernia
#9
Hiroyuki Tsuda, Tomomi Kotani, Mayo Miura, Yumiko Ito, Shima Hirako, Tomoko Nakano, Kenji Imai, Fumitaka Kikkawa
OBJECTIVE: Congenital diaphragmatic hernia (CDH) causes pulmonary hypoplasia and pulmonary hypertension, which are associated with long-term respiratory problems in infants. The aim of this study was to establish a marker for predicting lung function at 1 year of age in infants with CDH. MATERIAL AND METHODS: Infants with isolated CDH who were delivered after 35 weeks of gestation from April 2008 to June 2016 at Nagoya University Hospital were registered. Regarding alive infants with CDH, only those who underwent follow-up for at least 1 year were registered...
February 24, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28276295/complete-bilateral-agenesis-of-the-diaphragm
#10
Mudher Al-Adnani, Andreas Marnerides
Bilateral agenesis of the diaphragm is a very rare congenital diaphragmatic defect. Bilateral congenital diaphragmatic hernia (CDH) is much more frequently associated with other anomalies compared with unilateral CDH (70% vs 30%-40%). These include cardiovascular, respiratory (other than lung hypoplasia), gastrointestinal, renal, and genital malformations. We report a case of complete bilateral agenesis of the diaphragm associated with a horseshoe kidney and an imperforate anus. These additional malformations have not previously been reported in association with complete bilateral agenesis of the diaphragm...
January 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28270239/-late-presentation-of-congenital-diaphragmatic-hernia
#11
N S Planting, A K E Hoffman-Haringsma
Congenital diaphragmatic hernia (CDH) is a rare congenital defect. Most cases are currently diagnosed either prenatally by ultrasound or shortly after birth. Late presentation of CDH is uncommon, and symptoms vary greatly. Here we describe two cases. The first concerns a 9-year-old boy with abdominal pain. The symptoms were interpreted as constipation and he was admitted for a high enema. After a few hours he developed severe respiratory distress; chest X-ray revealed a tension gastrothorax, and thoracostomy resulted in immediate respiratory improvement...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28267692/diabetes-increases-morbidities-of-colonic-diverticular-disease-and-colonic-diverticular-hemorrhage-a-systematic-review-and-meta-analysis
#12
REVIEW
Xiaoti Lin, Jingjing Li, Mingang Ying, Fengqin Wei, Xiaoming Xie
Colonic diverticular disease (CDD) and colonic diverticular hemorrhage (CDH) are the most common disorders in hospital admissions and outpatient health clinic visits. However, risk factors of CDD and CDH are complicated and need to be discussed. Diabetes mellitus (DM) has been related with CDD and CDH, but the associations remain ambiguous. Therefore, we performed a literature search for studies involving the associations among DM, morbidity of CDD, and incidence of CDH. Relative risks or odds ratios with their corresponding 95% confidence intervals (CIs) were combined and weighted to produce summary effect size...
March 2017: American Journal of Therapeutics
https://www.readbyqxmd.com/read/28267443/balloon-removal-after-fetoscopic-endoluminal-tracheal-occlusion-for-congenital-diaphragmatic-hernia
#13
Julio A Jiménez, Elisenda Eixarch, Philip Dekoninck, João R Bennini, Roland Devlieger, Cleisson F Peralta, Eduard Gratacos, Jan Deprest
BACKGROUND: Isolated congenital diaphragmatic hernia (CDH) defect allows viscera to herniate into the chest, competing for space with the developing lungs. At birth, pulmonary hypoplasia leads to respiratory insufficiency and persistent pulmonary hypertension that is lethal in up to 30% of patients. Antenatal measurement of lung size and liver herniation can predict survival after birth. Prenatal intervention aims at stimulating lung development, clinically achieved by percutaneous fetal endoscopic tracheal occlusion (FETO) under local anesthesia...
March 3, 2017: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28245812/molecular-and-catalytic-properties-of-fungal-extracellular-cellobiose-dehydrogenase-produced-in-prokaryotic-and-eukaryotic-expression-systems
#14
Su Ma, Marita Preims, François Piumi, Lisa Kappel, Bernhard Seiboth, Eric Record, Daniel Kracher, Roland Ludwig
BACKGROUND: Cellobiose dehydrogenase (CDH) is an extracellular enzyme produced by lignocellulolytic fungi. cdh gene expression is high in cellulose containing media, but relatively low CDH concentrations are found in the supernatant of fungal cultures due to strong binding to cellulose. Therefore, heterologous expression of CDH in Pichia pastoris was employed in the last 15 years, but the obtained enzymes were over glycosylated and had a reduced specific activity. RESULTS: We compare the well-established CDH expression host P...
February 28, 2017: Microbial Cell Factories
https://www.readbyqxmd.com/read/28242965/role-of-ultrasound-based-prenatal-prediction-of-pulmonary-function-in-congenital-diaphragmatic-hernia-does-it-have-prognostic-significance-postnatally
#15
Nupur Shah, Sujit Chowdhary, Anita Kaul
BACKGROUND AND OBJECTIVES: The incidence of congenital diaphragmatic hernia (CDH) in India is 1 in 1000. About 60 % of these are isolated, and the survival prognosis in them depends upon the quantum of contralateral functional lung. Out of the various pulmonary and extrapulmonary sonological predictors, observed to expected lung-head ratio (O/E LHR) is an efficient gestation-independent predictor of pulmonary function. This study was carried out to see the correlation of this prenatal predictor with the postnatal outcome depending on the pulmonary function...
February 2017: Journal of Obstetrics and Gynaecology of India
https://www.readbyqxmd.com/read/28230834/risk-stratification-for-congenital-diaphragmatic-hernia-by-factors-within-24%C3%A2-h-after-birth
#16
K Terui, K Nagata, Y Kanamori, S Takahashi, M Hayakawa, H Okuyama, N Inamura, H Yoshida, T Taguchi, N Usui
OBJECTIVE: To establish a simple risk stratification system for patients with congenital diaphragmatic hernia (CDH) based on postnatal information within 24 h after birth. STUDY DESIGN: A multi-institutional retrospective cohort study was conducted including 348 neonates who had isolated CDH born between 2006 and 2010. Based on the two most powerful variables for 90-day survival selected by multivariate analyses, a risk stratification system was established. RESULTS: Multiple logistic regression analysis identified two adverse prognostic factors: an Apgar score at 1 min (Ap1) of 0-4 (odds ratio (OR) 3...
February 23, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28211131/congenital-diaphragmatic-hernia-outcomes-of-neonates-treated-at-mayo-clinic-with-and-without-extracorporeal-membrane-oxygenation
#17
Katarina Bojanić, Jason M Woodbury, Alexandre N Cavalcante, Ruža Grizelj, Garth F Asay, Christopher E Colby, William A Carey, Gregory J Schears, Toby N Weingarten, Darrell R Schroeder, Juraj Sprung
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a rare anomaly with high mortality and long-term comorbid conditions. AIMS: Our aim was to describe the presenting characteristics, treatment, and outcomes of consecutive patients with CDH treated at our institution. METHODS: We performed a retrospective cohort study and identified consecutive neonates treated for CDH from 2001 to 2015 at our institution. For all patients identified, we reviewed hospital and postdischarge data for neonatal, disease, and treatment characteristics...
March 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28203431/pulmonary-sequestration-mimicking-a-pancreas-herniation-in-a-case-of-recurrent-bochdalek-hernia
#18
Gaëtan-Romain Joliat, Jean Yannis Perentes, Hans-Beat Ris, Nermin Halkic
In the reported scenario, the patient known for a history of operated Bochdalek hernia or congenital diaphragmatic hernia (CDH) presented with new abdominal pain. The CT-scan suspected the presence of pancreas herniation through a recurrent CDH. Intraoperatively, the patient was found to have a recurrent CDH containing greater omentum concomitantly with a pulmonary sequestration (PS). This case report highlights the fact that intraoperative findings can be different from preoperative radiological diagnosis...
January 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28193541/a-nano-scaled-and-multi-layered-recombinant-fibronectin-cadherin-chimera-composite-selectively-concentrates-osteogenesis-related-cells-and-factors-to-aid-bone-repair
#19
Junchao Xing, Tieniu Mei, Keyu Luo, Zhiqiang Li, Aijun Yang, Zhilin Li, Zhao Xie, Zehua Zhang, Shiwu Dong, Tianyong Hou, Jianzhong Xu, Fei Luo
Easily accessible and effective bone grafts are in urgent need in clinic. The selective cell retention (SCR) strategy, by which osteogenesis-related cells and factors are enriched from bone marrow into bio-scaffolds, holds great promise. However, the retention efficacy is limited by the relatively low densities of osteogenesis-related cells and factors in marrow; in addition, a lack of satisfactory surface modifiers for scaffolds further exacerbates the dilemma. To address this issue, a multi-layered construct consisting of a recombinant fibronectin/cadherin chimera was established via a layer-by-layer self-assembly technique (LBL-rFN/CDH) and used to modify demineralised bone matrix (DBM) scaffolds...
February 11, 2017: Acta Biomaterialia
https://www.readbyqxmd.com/read/28190559/use-of-renal-near-infrared-spectroscopy-measurements-in-congenital-diaphragmatic-hernia-patients-on-ecmo
#20
Patricio E Lau, Stephanie Cruz, Joseph Garcia-Prats, Milenka Cuevas, Christopher Rhee, Darrell L Cass, Sarah E Horne, Timothy C Lee, Stephen E Welty, Oluyinka O Olutoye
INTRODUCTION: This study tests the hypothesis that renal tissue oxygen saturation as measured by Near Infrared Spectroscopy (NIRS) would correlate with urine output in neonates with congenital diaphragmatic hernia (CDH) on extracorporeal membrane oxygenation (ECMO). METHODS: Between 2012 and 2015, neonates with CDH were enrolled as part of a comprehensive study that provided renal/cerebral/abdominal NIRS monitoring for the duration of ECMO support. Continuous NIRS measurements, mean arterial pressure, and urine output were recorded...
January 27, 2017: Journal of Pediatric Surgery
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