keyword
MENU ▼
Read by QxMD icon Read
search

Congenital diaphragmatic hernia

keyword
https://www.readbyqxmd.com/read/28410786/the-use-of-ecmo-for-gastroschisis-and-omphalocele-two-decades-of-experience
#1
Joanne E Baerg, Arul Thirumoorthi, Andrew O Hopper, Edward P Tagge
PURPOSE: The aim was to review the respiratory failure causes and outcomes of infants with omphalocele or gastroschisis receiving ECMO and reported to the Extracorporeal Life Support Organization (ELSO). METHODS: Gastroschisis and omphalocele infants supported with ECMO and reported to the ELSO Registry between 1992 and 2015 were retrospectively reviewed. Clinical variables, diagnosis of respiratory failure (pulmonary hypertension (PHN), congenital heart defects (CHD), congenital diaphragmatic hernia (CDH), and sepsis), and outcomes were recorded...
March 16, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28399114/lung-hypoplasia-in-newborn-rabbits-with-a-diaphragmatic-hernia-affects-pulmonary-ventilation-but-not-perfusion
#2
Andreas W Flemmer, Marta Thio, Megan J Wallace, Katie Lee, K Marcus J Kitchen, Lauren Kerr, Charles C Roehr, Andreas Fouras, Richard Carnibella, Jaccques J Jani, Philip DeKoninck, Arjan B tePas, James T Pearson, Stuart B Hooper
BACKGROUND: A congenital diaphragmatic hernia can result in severe lung hypoplasia that increases the risk of morbidity and mortality after birth, but little is known about the cardiorespiratory transition at birth. METHODS: Using phase contrast X-ray imaging and angiography, we examined the cardiorespiratory transition at birth in rabbit kittens with diaphragmatic hernias (DH). Surgery was performed on pregnant New Zealand white rabbits (n=18) at 25 days gestation to induce a left-sided DH...
April 11, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28398654/survival-disparities-associated-with-congenital-diaphragmatic-hernia
#3
Cynthia F Hinton, Csaba Siffel, Adolfo Correa, Stuart K Shapira
BACKGROUND: We assessed sociodemographic and clinical factors that are associated with survival among infants with congenital diaphragmatic hernia (CDH). METHODS: Using data from the Metropolitan Atlanta Congenital Defects Program, we ascertained 150 infants born with CDH between 1979 and 2003 and followed via linkage with state vital records and the National Death Index. Kaplan-Meier survival probabilities and adjusted hazard ratios (HRs) were calculated for socioeconomic and clinical characteristics...
April 10, 2017: Birth Defects Res
https://www.readbyqxmd.com/read/28389079/safety-and-tolerability-of-subcutaneous-treprostinil-in-newborns-with-congenital-diaphragmatic-hernia-and-life-threatening-pulmonary-hypertension
#4
E Carpentier, S Mur, E Aubry, L Pognon, T Rakza, F Flamein, D Sharma, P Tourneux, L Storme
BACKGROUND: Prolonged pulmonary hypertension (PH) is highly predictive for pulmonary morbidity and death in infants with congenital diaphragmatic hernia (CDH). OBJECTIVES: To report the effects and tolerability of subcutaneous treprostinil in newborns with severe CDH and late life-threatening PH. METHODS: We recorded clinical and echocardiography data before and after starting subcutaneous treprostinil, on patients with severe CDH and late PH, refractory to inhaled nitric oxide and oral sildenafil...
March 28, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28385427/percent-predicted-lung-volume-changes-on-fetal-magnetic-resonance-imaging-throughout-gestation-in-congenital-diaphragmatic-hernia
#5
Hester F Shieh, Carol E Barnewolt, Jay M Wilson, David Zurakowski, Susan A Connolly, Judy A Estroff, Jill Zalieckas, C Jason Smithers, Terry L Buchmiller
PURPOSE: Percent predicted lung volume (PPLV)<15% on fetal MRI predicts high-risk CDH. Potential changes in PPLV throughout gestation and impact on risk stratification are unknown. We reviewed CDH patients with serial fetal MRIs to follow PPLV and determine correlation with postnatal outcomes. METHODS: CDH patients with serial fetal MRIs from 2005 to 2015 were included. We recorded prenatal MRI gestational age (GA) and PPLV, postnatal ECMO use, and survival. Data were analyzed by logistic regression and Fisher's exact test...
March 16, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28370263/fetal-left-sided-cardiac-structural-dimensions-in-left-sided-congenital-diaphragmatic-hernia-association-with-severity-and-impact-on-postnatal-outcomes
#6
Joshua Kailin, Gurpreet Dhillon, Shiraz Maskatia, Darrell Cass, Alireza Shamshirsaz, Amy Mehollin-Ray, Christopher Cassady, Nancy Ayres, Yunfei Wang, Michael Belfort, Oluyinka Olutoye, Rodrigo Ruano
OBJECTIVES: Fetuses with congenital diaphragmatic hernia (CDH) demonstrate varying degrees of left heart hypoplasia. Our study assesses the relationship between fetal left-sided cardiac structural dimensions, lung size, percentage liver herniation, lung-to-head ratio, postnatal left-sided cardiac structural dimensions, and postnatal outcomes. METHODS: We performed a retrospective cohort study of fetuses with left-sided CDH who had prenatal echocardiographic, ultrasound and MRI examinations at our institution between January 2007 and March 2015...
March 31, 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28363469/down-regulation-of-n-deacetylase-n-sulfotransferase-1-signaling-in-the-developing-diaphragmatic-vasculature-of-nitrofen-induced-congenital-diaphragmatic-hernia
#7
Toshiaki Takahashi, Florian Friedmacher, Julia Zimmer, Prem Puri
BACKGROUND: Congenital diaphragmatic hernia (CDH) has been attributed to various developmental abnormalities of the underlying tissue components. N-deacetylase-N-sulfotransferase-1 (Ndst1) is a strongly expressed biosynthetic enzyme in endothelial cells, which has recently been identified as an important factor during diaphragmatic vascularization. Loss of endothelial Ndst1 has been demonstrated to cause angiogenic defects in the developing diaphragm and disrupt normal diaphragmatic development...
March 18, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28359590/factors-associated-with-early-recurrence-after-congenital-diaphragmatic-hernia-repair
#8
Luke R Putnam, Vikas Gupta, Kuojen Tsao, Carl F Davis, Pamela A Lally, Kevin P Lally, Matthew T Harting
BACKGROUND: The purpose of this study was to identify patient and treatment characteristics associated with early (in hospital) hernia recurrence after congenital diaphragmatic hernia (CDH) repair. METHODS: Data from the Congenital Diaphragmatic Hernia Study Group registry were queried from 2007 to 2015. Recurrence of the diaphragmatic hernia after initial repair and prior to death or discharge was determined at the time of reoperation. Minimally invasive surgery (MIS) approaches included laparoscopy or thoracoscopy, and open approaches consisted of laparotomy or thoracotomy...
March 16, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28358826/a-growing-animal-model-for-neonatal-repair-of-large-diaphragmatic-defects-to-evaluate-patch-function-and-outcome
#9
Mary Patrice Eastwood, Luc Joyeux, Savitree Pranpanus, Johannes Van der Merwe, Eric Verbeken, Stephanie De Vleeschauwer, Ghislaine Gayan-Ramirez, Jan Deprest
OBJECTIVES: We aimed to develop a more representative model for neonatal congenital diaphragmatic hernia repair in a large animal model, by creating a large defect in a fast-growing pup, using functional pulmonary and diaphragmatic read outs. BACKGROUND: Grafts are increasingly used to repair congenital diaphragmatic hernia with the risk of local complications. Growing animal models have been used to test novel materials. METHODS: 6-week-old rabbits underwent fiberoptic intubation, left subcostal laparotomy and hemi-diaphragmatic excision (either nearly complete (n = 13) or 3*3cm (n = 9)) and primary closure (Gore-Tex patch)...
2017: PloS One
https://www.readbyqxmd.com/read/28352483/ultrasound-echocardiography-mri-and-genetic-analysis-of-a-fetus-with-congenital-diaphragmatic-hernia-and-partial-11q-trisomy
#10
Yolanda Fernández-Perea, Lutgardo García-Díaz, Javier Sánchez, Guillermo Antiñolo, Salud Borrego
Congenital diaphragmatic hernia (CDH) is a serious birth defect with a significant mortality and morbidity. The current and constant progress in ultrasound techniques has led to the improvement of the prenatal diagnosis of this malformation. CDH is a developmental defect whose etiology is heterogeneous and takes place when the pleuroperitoneal folds and septum transversum fail to converge and fuse. Survival depends on the extent of pulmonary hypoplasia and the disease may be potentially worsened by the presence of added congenital defects...
2017: Case Reports in Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28336004/-postnatal-diagnosis-of-gastric-volvulus-revealing-congenital-diaphragmatic-hernia
#11
A Aprahamian, V Nouyrigat, D Grévent, E Hervieux, G Chéron
Postnatally diagnosed congenital diaphragmatic hernias (CDH) are rare and have a better prognosis than those diagnosed prenatally. Postnatal symptoms can be respiratory, digestive, or mixed. Gastric volvulus can reveal CDH. Symptoms are pain, abdominal distension, and/or vomiting. Upper gastrointestinal barium X-ray radiography provides the diagnosis. Prognosis is related to early surgical management in complicated forms with intestinal occlusion or sub-occlusion. We report on an infant who presented with vomiting, which revealed gastric volvulus associated with a CDH...
May 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28331629/congenital-diaphragmatic-hernia-a-review
#12
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/28325581/prenatal-prediction-of-pulmonary-hypoplasia
#13
REVIEW
Jourdan E Triebwasser, Marjorie C Treadwell
Pulmonary hypoplasia, although rare, is associated with significant neonatal morbidity and mortality. Conditions associated with pulmonary hypoplasia include those which limit normal thoracic capacity or movement, including skeletal dysplasias and abdominal wall defects; those with mass effect, including congenital diaphragmatic hernia and pleural effusions; and those with decreased amniotic fluid, including preterm, premature rupture of membranes, and genitourinary anomalies. The ability to predict severe pulmonary hypoplasia prenatally aids in family counseling, as well as obstetric and neonatal management...
March 15, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28321802/sepsis-risk-factors-in-infants-with-congenital-diaphragmatic-hernia
#14
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
#15
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
#16
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/28301893/congenital-diaphragmatic-hernia-the-side-of-diaphragmatic-defect-and-associated-nondiaphragmatic-malformations
#17
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
#18
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/28289880/pediatric-morgagni-diaphragmatic-hernia-a-descriptive-study
#19
Jamie Golden, Wesley E Barry, Gene Jang, Nam Nguyen, David Bliss
INTRODUCTION: The Morgagni hernia (MH) accounts for 3-4% of congenital diaphragmatic hernias. There is a paucity of data regarding this rare defect. The purpose of this study is to describe the characteristics of children with MH, surgical approaches for repair, and patient outcomes. METHODS: Pediatric patients (ages 0-18) with a MH from 2002 to 2014 at a single, freestanding pediatric hospital were retrospectively reviewed. Patient presentation, demographics, operative methods and findings, and outcomes were evaluated...
March 13, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28286461/psychomotor-retardation-with-a-1q42-11-q42-12-deletion
#20
Jialing He, Yingjun Xie, Shu Kong, Wenjun Qiu, Xiaoman Wang, Ding Wang, Xiaofang Sun, Deming Sun
A 1q42 deletion is a rare structure variation that commonly harbours various deletion breakpoints along with diversified phenotypes. In our study, we found a de novo 1q42 deletion in a boy who did not have a cleft palate or a congenital diaphragmatic hernia but presented with psychomotor retardation. A 1.9 Mb deletion located within 1q42.11-q42.12 was validated at the molecular cytogenetic level. This is the first report of a 1q42.11-q42.12 deletion in a patient with onlypsychomotor retardation. The precise break points could facilitate the discovery of potential causative genes, such as LBR, EPHX1, etc...
2017: Hereditas
keyword
keyword
1256
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"