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Diaphragmatic hernia neonate

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https://www.readbyqxmd.com/read/29448010/improved-outcomes-in-management-of-hypoplastic-left-heart-syndrome-associated-with-congenital-diaphragmatic-hernia-an-algorithmic-approach
#1
Kaitlin Balduf, T K Susheel Kumar, Umar Boston, Shyam Sathanandam, Marc V Lee, Tim Jancelewicz, Christopher J Knott-Craig
BACKGROUND: Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival of neonates with CDH and HLHS is only 1-5%. We review our experience with CDH and HLHS and compare our outcomes to published literature. METHODS: Retrospective review of all neonates with CDH and HLHS at our institution over a 10 year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies and outcomes were reviewed and an algorithmic approach is proposed...
February 12, 2018: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29420402/twenty-week-abortion-bans-on-pregnancies-with-a-congenital-diaphragmatic-hernia-a-cost-effectiveness-analysis
#2
Kimberley A Bullard, Brian L Shaffer, Karen S Greiner, Ashley E Skeith, Maria I Rodriguez, Aaron B Caughey
OBJECTIVE: To estimate the effect of 20-week abortion bans on maternal and consequent neonatal health outcomes and costs in the setting of fetal congenital diaphragmatic hernia. METHODS: A decision-analytic model was built using TreeAge software to evaluate the effect of a 20-week ban on abortion in a theoretical cohort of 921 women diagnosed with fetal congenital diaphragmatic hernia. Probabilities, utilities, and costs were derived from the literature. The cohort size was based on the annual rate of prenatal diagnoses of congenital diaphragmatic hernia and live births among the 20 states with bans...
February 5, 2018: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/29390430/congenital-diaphragmatic-hernia-in-association-with-congenital-short-esophagus-a-case-report
#3
Bo Xia, Chun Hong, Jing Tang, Cuifen Liu, Gang Yu
RATIONALE: Congenital diaphragmatic hernia (CDH) associated congenital anomalies are present in about 25%. Congenital short esophagus (CSE) is a relatively rare condition. Both CDH and congenital intrathoracic stomach caused by CSE can be diagnosed in utero. However, CSE can be easily misdiagnosed in utero. PATIENT CONCERNS: We present a case of left CDH with CSE in a female neonate who was diagnosed at 24 weeks gestational age by antenatal ultrasound. DIAGNOSES: The neonate with CDH and congenital intrathoracic stomach due to CSE was confirmed by operation...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29338874/extracorporeal-membrane-oxygenation-in-infants-with-congenital-diaphragmatic-hernia
#4
REVIEW
Theresa R Grover, Natalie E Rintoul, Holly L Hedrick
Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly which impairs normal pulmonary development leading to acute and chronic respiratory failure, pulmonary hypoplasia, pulmonary hypertension, and mortality. CDH is the most common non-cardiac indication for neonatal ECMO. Prenatal and postnatal predictors of CDH severity aid in patient selection. Centers vary in preferred mode of ECMO and timing of CDH repair. Survivors of severe CDH with ECMO are at risk for long-term sequelae including neurodevelopmental delays...
January 12, 2018: Seminars in Perinatology
https://www.readbyqxmd.com/read/29330547/recessive-loss-of-function-pign-alleles-including-an-intragenic-deletion-with-founder-effect-in-la-r%C3%A3-union-island-in-patients-with-fryns-syndrome
#5
Jean-Luc Alessandri, Christopher T Gordon, Marie-Line Jacquemont, Nicolas Gruchy, Norbert F Ajeawung, Guillaume Benoist, Myriam Oufadem, Asma Chebil, Yannis Duffourd, Coralie Dumont, Marion Gérard, Paul Kuentz, Thibaud Jouan, Francesca Filippini, Thi Tuyet Mai Nguyen, Olivier Alibeu, Christine Bole-Feysot, Patrick Nitschké, Asma Omarjee, Duksha Ramful, Hanitra Randrianaivo, Bérénice Doray, Laurence Faivre, Jeanne Amiel, Philippe M Campeau, Julien Thevenon
Fryns syndrome (FS) is a multiple malformations syndrome with major features of congenital diaphragmatic hernia, pulmonary hypoplasia, craniofacial dysmorphic features, distal digit hypoplasia, and a range of other lower frequency malformations. FS is typically lethal in the fetal or neonatal period. Inheritance is presumed autosomal recessive. Although no major genetic cause has been identified for FS, biallelic truncating variants in PIGN, encoding a component of the glycosylphosphatidylinositol (GPI)-anchor biosynthesis pathway, have been identified in a limited number of cases with a phenotype compatible with FS...
January 12, 2018: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/29323665/arterial-tortuosity-syndrome-40-new-families-and-literature-review
#6
Aude Beyens, Juliette Albuisson, Annekatrien Boel, Mazen Al-Essa, Waheed Al-Manea, Damien Bonnet, Ozlem Bostan, Odile Boute, Tiffany Busa, Nathalie Canham, Ergun Cil, Paul J Coucke, Margot A Cousin, Majed Dasouki, Julie De Backer, Anne De Paepe, Sofie De Schepper, Deepthi De Silva, Koenraad Devriendt, Inge De Wandele, David R Deyle, Harry Dietz, Sophie Dupuis-Girod, Eudice Fontenot, Björn Fischer-Zirnsak, Alper Gezdirici, Jamal Ghoumid, Fabienne Giuliano, Neus Baena Diéz, Mohammed Z Haider, Joshua S Hardin, Xavier Jeunemaitre, Eric W Klee, Uwe Kornak, Manuel F Landecho, Anne Legrand, Bart Loeys, Stanislas Lyonnet, Helen Michael, Pamela Moceri, Shehla Mohammed, Laura Muiño-Mosquera, Sheela Nampoothiri, Karin Pichler, Katrina Prescott, Anna Rajeb, Maria Ramos-Arroyo, Massimiliano Rossi, Mustafa Salih, Mohammed Z Seidahmed, Elise Schaefer, Elisabeth Steichen-Gersdorf, Sehime Temel, Fahrettin Uysal, Marine Vanhomwegen, Lut Van Laer, Lionel Van Maldergem, David Warner, Andy Willaert, Tom R Collins, Andrea Taylor, Elaine C Davis, Yuri Zarate, Bert Callewaert
PurposeWe delineate the clinical spectrum and describe the histology in arterial tortuosity syndrome (ATS), a rare connective tissue disorder characterized by tortuosity of the large and medium-sized arteries, caused by mutations in SLC2A10.MethodsWe retrospectively characterized 40 novel ATS families (50 patients) and reviewed the 52 previously reported patients. We performed histology and electron microscopy (EM) on skin and vascular biopsies and evaluated TGF-β signaling with immunohistochemistry for pSMAD2 and CTGF...
January 11, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/29319634/hyperoxia-and-hypocapnia-during-pediatric-extracorporeal-membrane-oxygenation-associations-with-complications-mortality-and-functional-status-among-survivors
#7
Katherine Cashen, Ron Reeder, Heidi J Dalton, Robert A Berg, Thomas P Shanley, Christopher J L Newth, Murray M Pollack, David Wessel, Joseph Carcillo, Rick Harrison, J Michael Dean, Robert Tamburro, Kathleen L Meert
OBJECTIVES: To determine the frequency of hyperoxia and hypocapnia during pediatric extracorporeal membrane oxygenation and their relationships to complications, mortality, and functional status among survivors. DESIGN: Secondary analysis of data collected prospectively by the Collaborative Pediatric Critical Care Research Network. SETTING: Eight Collaborative Pediatric Critical Care Research Network-affiliated hospitals. PATIENTS: Age less than 19 years and treated with extracorporeal membrane oxygenation...
January 9, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29316358/continuous-intravenous-sildenafil-as-an-early-treatment-in-neonates-with-congenital-diaphragmatic-hernia
#8
Florian Kipfmueller, Lukas Schroeder, Christoph Berg, Katrin Heindel, Peter Bartmann, Andreas Mueller
BACKGROUND: Pulmonary hypertension (PH) is an important contributor of morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). Treatment options are limited, but sildenafil might improve oxygenation and PH in neonates with CDH. OBJECTIVE: Aim of this study is to assess effects of intravenous sildenafil on oxygenation and PH in neonates with CDH. METHODS: A retrospective chart review was performed in all neonates with CDH born in our institution between September 2012 and December 2014...
January 5, 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29304545/prenatal-diagnosis-of-congenital-diaphragmatic-hernia-does-laterality-predict-perinatal-outcomes
#9
Jeffrey D Sperling, Teresa N Sparks, Victoria K Berger, Jody A Farrell, Kristen Gosnell, Roberta L Keller, Mary E Norton, Juan M Gonzalez
OBJECTIVE:  The objective of this study was to examine laterality as a predictor of outcomes among fetuses with prenatally diagnosed congenital diaphragmatic hernia (CDH). METHODS:  This is a retrospective cohort study of pregnancies with CDH evaluated at our center from 2008 to 2016 compared cases with right-sided CDH (RCDH) versus left-sided CDH (LCDH). The primary outcome was survival to discharge. Secondary outcomes included ultrasound predictors of poor prognosis (liver herniation, stomach herniation, lung area-to-head circumference ratio [LHR]), concurrent anomalies, hydrops, stillbirth, preterm birth, mode of delivery, small for gestational age, use of extracorporeal membrane oxygenation, and length of stay...
January 5, 2018: American Journal of Perinatology
https://www.readbyqxmd.com/read/29302380/a-case-of-fatal-pulmonary-hypoplasia-with-congenital-diaphragmatic-hernia-thoracic-myelomeningocele-and-thoracic-dysplasia
#10
Ai Ito, Hideshi Fujinaga, Sachiko Matsui, Kumiko Tago, Yuka Iwasaki, Shuhei Fujino, Junko Nagasawa, Shoichiro Amari, Masao Kaneshige, Yuka Wada, Shigehiro Takahashi, Keiko Tsukamoto, Osamu Miyazaki, Takako Yoshioka, Akira Ishiguro, Yushi Ito
Background  Congenital diaphragmatic hernia (CDH) is fatal in severe cases of pulmonary hypoplasia. We experienced a fatal case of pulmonary hypoplasia due to CDH, thoracic myelomeningocele (MMC), and thoracic dysplasia. This constellation of anomalies has not been previously reported. Case Report  A male infant with a prenatal diagnosis of thoracic MMC with severe hydrocephalus and scoliosis was born at 36 weeks of gestation. CDH was found after birth and the patient died of respiratory failure due to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn at 30 hours of age despite neonatal intensive care...
October 2017: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/29251627/pbx-transcription-factors-drive-pulmonary-vascular-adaptation-to-birth
#11
David J McCulley, Mark D Wienhold, Elizabeth A Hines, Timothy A Hacker, Allison Rogers, Ryan J Pewowaruk, Rediet Zewdu, Naomi C Chesler, Licia Selleri, Xin Sun
A critical event in the adaptation to extrauterine life is relaxation of the pulmonary vasculature at birth, allowing for a rapid increase in pulmonary blood flow that is essential for efficient gas exchange. Failure of this transition leads to pulmonary hypertension (PH), a major cause of newborn mortality associated with preterm birth, infection, hypoxia, and malformations including congenital diaphragmatic hernia (CDH). While individual vasoconstrictor and dilator genes have been identified, the coordination of their expression is not well understood...
December 18, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29250478/an-unusual-complication-of-congenital-diaphragmatic-hernia
#12
E Tian Tan, Keren Sloan, Kokila Lakhoo
A term newborn was referred to our unit with a postnatal diagnosis of a right-sided congenital diaphragmatic hernia (CDH). She was managed with high-frequency oscillatory ventilation, inotropic support, and nitric oxide, with planned surgical repair when she was medically optimized. On day 6 of life, there was an acute deterioration causing difficulty maintaining adequate ventilation and the infant requiring increasing analgesia and paralysis, especially during abdominal examination. A repeat X-ray showed distended bowel loops in the right hemithorax when compared with previous films raising suspicion of bowel obstruction...
January 2017: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/29209510/milrinone-in-congenital-diaphragmatic-hernia-a-randomized-pilot-trial-study-protocol-review-of-literature-and-survey-of-current-practices
#13
REVIEW
Satyan Lakshminrusimha, Martin Keszler, Haresh Kirpalani, Krisa Van Meurs, Patricia Chess, Namasivayam Ambalavanan, Bradley Yoder, Maria V Fraga, Holly Hedrick, Kevin P Lally, Leif Nelin, Michael Cotten, Jonathan Klein, Stephanie Guilford, Ashley Williams, Aasma Chaudhary, Marie Gantz, Jenna Gabrio, Dhuly Chowdhury, Kristin Zaterka-Baxter, Abhik Das, Rosemary D Higgins
Background: Congenital diaphragmatic hernia (CDH) is commonly associated with pulmonary hypoplasia and pulmonary hypertension (PH). PH associated with CDH (CDH-PH) is frequently resistant to conventional pulmonary vasodilator therapy including inhaled nitric oxide (iNO) possibly due to right and left ventricular dysfunction. Milrinone is an intravenous inotrope and lusitrope with pulmonary vasodilator properties and has been shown anecdotally to improve oxygenation in PH. We developed this pilot study to determine if milrinone infusion would improve oxygenation in neonates ≥36 weeks postmenstrual age (PMA) with CDH...
2017: Maternal Health, Neonatology and Perinatology
https://www.readbyqxmd.com/read/29203958/role-of-catalytic-iron-and-oxidative-stress-in-nitrofen-induced-congenital-diaphragmatic-hernia-and-its-amelioration-by-saireito-tj-114
#14
Shima Hirako, Hiroyuki Tsuda, Fumiya Ito, Yasumasa Okazaki, Tasuku Hirayama, Hideko Nagasawa, Tomoko Nakano, Kenji Imai, Tomomi Kotani, Fumitaka Kikkawa, Shinya Toyokuni
Congenital diaphragmatic hernia (CDH) is a life-threatening neonatal disease that leads to lung hypoplasia and pulmonary hypertension. We recently found that maternal prenatal administration of Saireito (TJ-114) ameliorates fetal CDH in a nitrofen-induced rat model. Here, we studied the role of iron and oxidative stress in neonates of this model and in lung fibroblasts IMR90-SV in association with nitrofen and Saireito. We observed increased immunostaining of 8-hydroxy-2'-deoxyguanosine in the lungs of neonates with CDH, which was ameliorated by maternal Saireito intake...
November 2017: Journal of Clinical Biochemistry and Nutrition
https://www.readbyqxmd.com/read/29194146/risk-factors-of-impaired-neuropsychologic-outcome-in-school-aged-survivors-of-neonatal-critical-illness
#15
Lisette Leeuwen, Raisa M Schiller, André B Rietman, Joost van Rosmalen, Enno D Wildschut, Robert Jan M Houmes, Dick Tibboel, Hanneke IJsselstijn
OBJECTIVE: Until now, long-term outcome studies have focused on general cognitive functioning and its risk factors following neonatal extracorporeal membrane oxygenation and/or congenital diaphragmatic hernia. However, it is currently unknown which neuropsychological domains are most affected in these patients and which clinical variables can be used to predict specific neuropsychological problems. This study aimed to identify affected neuropsychological domains and its clinical determinants in survivors of neonatal extracorporeal membrane oxygenation and/or congenital diaphragmatic hernia...
November 30, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/29193315/review-shows-that-implementing-a-nationwide-protocol-for-congenital-diaphragmatic-hernia-was-a-key-factor-in-reducing-mortality-and-morbidity
#16
REVIEW
L Storme, J Boubnova, S Mur, L Pognon, D Sharma, E Aubry, R Sfeir, P Vaast, T Rakza, A Benachi
The French Rare Disease Reference Center for Congenital Diaphragmatic Hernia (CDH) was created in 2008, to implement a national protocol for fetuses and children with this serious condition. Neonatal mortality from CDH is 30-40%, mainly due to pulmonary hypoplasia and persistent pulmonary hypertension, and half of those who live have high respiratory, nutritional and digestive morbidity. CDH management requires long-term and specialised multidisciplinary care. It has been well established that a standardised management protocol improves the prognosis of children with CDH...
November 28, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/29189614/acquired-infection-and-antimicrobial-utilization-during-initial-nicu-hospitalization-in-infants-with-congenital-diaphragmatic-hernia
#17
Sarah Keene, Karna Murthy, Eugenia Pallotto, Beverly Brozanski, Jason Gien, Isabella Zaniletti, Cheryl Hulbert, Ruth Seabrook, Natalie Rintoul, Louis Chicoine, Nicolas Porta, Theresa R Grover
BACKGROUND: In addition to substantial medical and surgical intervention, neonates with congenital diaphragmatic hernia (CDH) often have concurrent concerns for acquired infection. However, few studies focus on infection and corresponding antimicrobial utilization in this population. METHODS: The Children's Hospital Neonatal Database was queried for CDH infants hospitalized from Jan 2010- Feb 2016. Patient charts were linked to the Pediatric Health Information Systems (PHIS) database...
November 16, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/29189422/extracorporeal-membrane-oxygenation-in-premature-infants-with-congenital-diaphragmatic-hernia
#18
Milenka Cuevas Guamán, Adesola C Akinkuotu, Stephanie M Cruz, Pamela A Griffiths, Stephen E Welty, Timothy C Lee, Oluyinka O Olutoye
Prematurity and low birth weight have been exclusion criteria for extracorporeal membrane oxygenation (ECMO); however, these criteria are not evidence based. With advances in anticoagulation, improved technology, and surgical expertise, it is difficult to deny a potential therapy based on these criteria alone. We report the outcome of three neonates who were ineligible based on traditional criteria but were offered ECMO as a life-saving measure. We highlight the interdisciplinary nature of modern decision-making...
November 14, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29181040/is-there-a-determining-factor-that-predicts-mortality-in-patients-with-congenital-diaphragmatic-hernia
#19
Tansel Gunendi, Basak Erginel, Ercan Bastu, Ibrahim Kalelioglu, Recep Has, Feryal Gun Soysal, Erbug Keskin, Aladdin Celik, Tansu Salman
Aim: This study was designed to investigate the factors affecting the prognosis in neonates with congenital diaphragmatic hernia (CDH) who were treated in our clinic. These factors included prenatal lung-head ratio (LHR), prenatal stomach and liver presence in the thorax, blood gases in the first 24 h and the modified ventilation index (MVI). Material and methods: The study was carried out retrospectively in 30 neonates with prenatally diagnosed left CDH who were treated in our clinic between January 2007 and 2013...
September 2017: Kardiochirurgia i Torakochirurgia Polska, Polish Journal of Cardio-Thoracic Surgery
https://www.readbyqxmd.com/read/29165681/diaphragmatic-function-in-infants-and-children-with-congenital-diaphragmatic-hernia-a-cross-sectional-study
#20
Sonia Khirani, Alessandro Amaddeo, Naziha Khen-Dunlop, Jorge Olmo Arroyo, Alexandre Lapillonne, Odile Becquet, Elsa Kermorvant-Duchemin, Sylvie Beaudoin, Véronique Rousseau, Christophe Delacourt, Brigitte Fauroux
OBJECTIVES: Few studies have evaluated long-term diaphragmatic function in congenital diaphragmatic hernia (CDH). The aim of our cross-sectional study was to assess diaphragmatic function in infants and young children with CDH after surgical repair. METHODS: All the patients with CDH repair followed in our centre between February 2014 and January 2016 were enrolled. Patients with a postnatal diagnosis after 1 month of life were excluded. Breathing pattern and diaphragmatic function were assessed using esophageal and gastric (Pgas) pressure recording after surgery, or at 1 or 5 years of age...
November 20, 2017: European Journal of Cardio-thoracic Surgery
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