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Congenital diaphragmatic hernia

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https://www.readbyqxmd.com/read/28211131/congenital-diaphragmatic-hernia-outcomes-of-neonates-treated-at-mayo-clinic-with-and-without-extracorporeal-membrane-oxygenation
#1
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...
February 17, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28203431/pulmonary-sequestration-mimicking-a-pancreas-herniation-in-a-case-of-recurrent-bochdalek-hernia
#2
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/28196822/hepatopulmonary-fusion-a-rare-and-potentially-lethal-association-with-right-congenital-diaphragmatic-hernia
#3
Vishesh Jain, Devendra Kumar Yadav, Devasenathipathy Kandasamy, Devendra Kumar Gupta
Hepatopulmonary fusion is an extremely rare accompaniment of right congenital diaphragmatic hernia. It is associated with abnormal systemic arterial supply and venous drainage of the right lung along with congenital heart disease. Children with this condition have a comparatively poor prognosis. We report a case of right congenital diaphragmatic hernia with hepatopulmonary fusion along with review of the literature with stress on diagnosis and management.
February 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28190559/use-of-renal-near-infrared-spectroscopy-measurements-in-congenital-diaphragmatic-hernia-patients-on-ecmo
#4
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
https://www.readbyqxmd.com/read/28188034/follistatin-like-1-expression-is-decreased-in-the-alveolar-epithelium-of-hypoplastic-rat-lungs-with-nitrofen-induced-congenital-diaphragmatic-hernia
#5
Toshiaki Takahashi, Julia Zimmer, Florian Friedmacher, Prem Puri
BACKGROUND/PURPOSE: Pulmonary hypoplasia (PH), characterized by incomplete alveolar development, remains a major therapeutic challenge associated with congenital diaphragmatic hernia (CDH). Follistatin-like 1 (Fstl1) is a crucial regulator of alveolar formation and maturation, which is strongly expressed in distal airway epithelium. Fstl1-deficient mice exhibit reduced airspaces, impaired alveolar epithelial cell differentiation, and insufficient production of surfactant proteins similar to PH in human CDH...
January 28, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28178582/short-term-weight-gain-velocity-in-infants-with-congenital-diaphragmatic-hernia-cdh
#6
Jason Gien, Karna Murthy, Eugenia K Pallotto, Beverly Brozanski, Louis Chicoine, Isabella Zaniletti, Ruth Seabrook, Sarah Keene, Deepthi Alapati, Nicolas Porta, Natalie Rintoul, Theresa R Grover
BACKGROUND: Appropriate post-natal growth remains a mainstay of therapeutic goals for infants with CDH, with the hypothesis that optimizing linear growth will improve survival through functional improvements in pulmonary hypoplasia. However, descriptions of growth and the effect on survival are limited in affected infants. OBJECTIVE: Describe in-hospital weight gain related to survival among infants with CDH. DESIGN/METHODS: Children's Hospitals Neonatal Database (CHND) identified infants with CDH born ≥34weeks' gestation (2010-14)...
February 5, 2017: Early Human Development
https://www.readbyqxmd.com/read/28174644/interstitial-deletion-of-chromosome-1-1p21-1p12-in-an-infant-with-congenital-diaphragmatic-hernia-hydrops-fetalis-and-interrupted-aortic-arch
#7
Masitah Ibrahim, Matthew Hunter, Lucy Gugasyan, Yuen Chan, Atul Malhotra, Arvind Sehgal, Kenneth Tan
We report a case of an infant with congenital diaphragmatic hernia (CDH) and hydrops fetalis who died from hypoxic respiratory failure. Autopsy revealed type B interrupted aortic arch (IAA). Microarray revealed a female karyotype with deletion of chromosome 1p21.1p12. There may be an association between 1p microdeletion, CDH, and IAA.
February 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28162765/mutations-in-bmpr2-are-not-present-in-patients-with-pulmonary-hypertension-associated-with-congenital-diaphragmatic-hernia
#8
Joanne S Chiu, Lijiang Ma, Julia Wynn, Usha Krishnan, Erika B Rosenzweig, Gudrun Aspelund, Marc Arkovitz, Brad W Warner, Foong-Yen Lim, George B Mychaliska, Kenneth Azarow, Robert A Cusick, Dai H Chung, Wendy K Chung
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a prevalent major congenital anomaly with significant morbidity and mortality. Thirty to 40% mortality in CDH is largely attributed to pulmonary hypoplasia and pulmonary hypertension (PH). We hypothesized that the underlying genetic risk factors for hereditary PH are shared with CDH associated PH. METHODS: Participants were recruited as part of the Diaphragmatic Hernia Research & Exploration; Advancing Molecular Science (DHREAMS) study, a prospective cohort of neonates with a diaphragmatic defect enrolled from 2005 to 2012...
January 26, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28157247/congenital-diaphragmatic-hernia-pathogenesis-prenatal-diagnosis-and-management-literature-review
#9
Przemysław Kosiński, Mirosław Wielgoś
Congenital diaphragmatic hernia (CDH) is a developmental discontinuity of the diaphragm. It allows abdominal viscera to herniate into the chest and leads to lung hypoplasia. Congenital diaphragmatic hernia is one of the most severe birth defects, with extremely high neonatal mortality. This paper presents a review of the available literature on prenatal diagnosis, management and treatment options for CDH. In selected cases, a prenatal procedure to improve neonatal survival is possible. The authors of this manuscript believe their work might contribute to a better understanding of congenital diaphragmatic hernia and patient selection for the FETO (fetal endoscopic tracheal occlusion) surgery or expectant management...
2017: Ginekologia Polska
https://www.readbyqxmd.com/read/28152192/effects-of-tracheal-occlusion-with-retinoic-acid-administration-on-normal-lung-development
#10
Delabaere Amélie, Marceau Geoffroy, Coste Karen, Blanchon Loïc, Déchelotte Pierre Jean, Blanc Pierre, Sapin Vincent, Gallot Denis
INTRODUCTION: Tracheal occlusion (TO) is an investigational therapy for severe congenital diaphragmatic hernia (CDH) that decreases pulmonary hypoplasia, but sustained TO also induces deficient surfactant synthesis. Intramuscular maternal administration of retinoic acid (RA) in a surgical rabbit model of CDH showed a beneficial effect on lung maturation. We evaluated the potential of RA delivery into the trachea and studied the combined effects of TO and RA on normal lung development...
February 2, 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28150314/the-upturned-superior-mesenteric-artery-sign-for-first-trimester-detection-of-congenital-diaphragmatic-hernia-and-omphalocele
#11
Ravi Selvaraj Lakshmy, Joy Agnees, Nity Rose
OBJECTIVES: The aim of this study was to follow the course of the superior mesenteric artery (SMA) in first-trimester fetuses to predict the location of the small bowel. Its abnormal course aids in early detection of congenital diaphragmatic hernia (CDH) and assessment of the contents of omphalocele. METHODS: The SMA can be easily identified in a sagittal section of the fetus by using color Doppler sonography at the 11- to 14-week scan, and normally, it has a downward course caudally to supply the intestines...
February 2, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28147253/minimally-invasive-vs-open-congenital-diaphragmatic-hernia-repair-is-there-a-superior-approach
#12
Luke R Putnam, Kuojen Tsao, Kevin P Lally, Martin L Blakely, Tim Jancelewicz, Pamela A Lally, Matthew T Harting
BACKGROUND: The minimally-invasive (MIS) approach for congenital diaphragmatic hernia (CDH) repair remains controversial. Our objective was to compare outcomes and complications of the MIS and open approaches, with risk-stratification of patients based on defect size and key patient characteristics. STUDY DESIGN: The multi-national CDH Study Group (CDHSG) registry was queried for the period from 2007-2015. Patient demographics and operative details including the CDHSG Staging System defect size (A-D) were reviewed...
January 29, 2017: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/28134682/aggressive-surgical-management-of-congenital-diaphragmatic-hernia-worth-the-effort-a-multicenter-prospective-cohort-study
#13
Matthew T Harting, Laura Hollinger, Kuojen Tsao, Luke R Putnam, Jay M Wilson, Ronald B Hirschl, Erik D Skarsgard, Dick Tibboel, Mary E Brindle, Pamela A Lally, Charles C Miller, Kevin P Lally
OBJECTIVE: The objectives of this study were (i) to evaluate infants with congenital diaphragmatic hernia (CDH) that do not undergo repair, (ii) to identify nonrepair rate by institution, and (iii) to compare institutional outcomes based on nonrepair rate. BACKGROUND: Approximately 20% of infants with CDH go unrepaired and the threshold to offer surgical repair is variable. METHODS: Data were abstracted from a multicenter, prospectively collected database...
January 27, 2017: Annals of Surgery
https://www.readbyqxmd.com/read/28133553/congenital-diaphragmatic-hernia-presenting-in-a-7-day-old-infant
#14
Christopher Rouse, Luke Schmidt, Lee Brock, Angela Fagiana
A 7-day-old male infant presented to the emergency room after respiratory distress was noted at an outpatient well child check. On exam, he was observed to have tachypnea, increased work of breathing, and decreased breath sounds on the left side of the chest. On chest X-ray, he was found to have a left-sided congenital diaphragmatic hernia. The infant was transported to a tertiary care facility where the defect was repaired without complication. Interestingly, the mother had a history of a normal antenatal ultrasound, completed at 19 + 2 weeks of gestational age...
2017: Case Reports in Emergency Medicine
https://www.readbyqxmd.com/read/28130958/a-definition-of-gentle-ventilation-in-congenital-diaphragmatic-hernia-a-survey-of-neonatologists-and-pediatric-surgeons
#15
Christiana Farkouh-Karoleski, Tasnim Najaf, Julia Wynn, Gudrun Aspelund, Wendy K Chung, Charles J Stolar, George B Mychaliska, Brad W Warner, Amy J Wagner, Robert A Cusick, Foong-Yen Lim, David T Schindel, Douglas Potoka, Kenneth Azarow, C Michael Cotten, Anthony Hesketh, Samuel Soffer, Timothy Crombleholme, Howard Needelman
Ventilation practices have changed significantly since the initial reports in the mid 1980 of successful use of permissive hypercapnia and spontaneous ventilation [often called gentle ventilation (GV)] in infants with congenital diaphragmatic hernia (CDH). However, there has been little standardization of these practices or of the physiologic limits that define GV. We sought to ascertain among Diaphragmatic Hernia Research and Exploration; Advancing Molecular Science (DHREAMS) centers' GV practices in the neonatal management of CDH...
January 28, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28108023/the-intersection-of-fetal-palliative-care-and-fetal-surgery-addressing-mortality-and-quality-of-life
#16
David Munson
Over the last few decades, the fields of fetal surgery and maternal-fetal medicine have developed interventions aimed at modifying severe diseases in utero. Innovations in fetal approaches to congenital diaphragmatic hernia and myelomeningocele have shown considerable promise in modifying the clinical course with fetal intervention. Patients who present to fetal centers to be evaluated for these interventions face challenging decisions that directly relate to questions of mortality and quality of life. This article explores how clinicians might apply the tools and principles of fetal palliative care to supporting a woman and her family who are considering fetal surgery...
January 17, 2017: Seminars in Perinatology
https://www.readbyqxmd.com/read/28100120/hybrid-stage-i-procedure-as-initial-palliation-for-neonate-with-hypoplastic-left-heart-syndrome-and-right-congenital-diaphragmatic-hernia
#17
Draginja Cvetkovic, Joseph Giamelli, Michael Lyew, Markus Erb, Suvro Sett, Youmna DiStefano
During the past decade, a hybrid procedure has emerged and dramatically evolved as an alternative stage I palliation to the conventional Norwood procedure in neonates with hypoplastic left heart syndrome (HLHS). The hybrid approach avoids the need for cardiopulmonary bypass (CPB) utilizing stenting of the arterial duct and bilateral pulmonary artery banding. Cerebral and coronary perfusion pressure is maintained, and the pulmonary vasculature is protected from higher systemic pressure. Elimination of risks associated with CPB gains vital time to stabilize the patient and correct coexisting noncardiac anomalies and allows growth in preparation for the later stages of the Fontan pathway...
January 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28099974/temporary-vacuum-assisted-closure-of-the-open-abdomen-in-neonates
#18
Kengo Hattori, Alp Numanoglu, Sharon Cox
Introduction The need for open abdomen in the treatment of severely ill neonates will increase in time as more complex abdominal procedures are undertaken. However, the experience of temporary closure of an open abdomen using vacuum-assisted closure (VAC) system is still relatively limited in premature and term neonates. The aim of this study is to describe and review our experience in the use of temporary VAC of the open abdomen for neonates with varying pathological processes. Materials and Methods A retrospective folder review of all neonates treated with VAC for open abdomen over the study period of 2010 to 2014 at our institution was performed...
January 18, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28095996/antenatal-predictors-of-outcome-in-prenatally-diagnosed-congenital-diaphragmatic-hernia-cdh
#19
REVIEW
Titilayo Oluyomi-Obi, Verena Kuret, Pramod Puligandla, Abhay Lodha, Helen Lee-Robertson, Kovid Lee, David Somerset, Joann Johnson, Greg Ryan
BACKGROUND: Pulmonary hypoplasia is the main cause of mortality in isolated congenital diaphragmatic hernia (CDH) and its prediction is paramount when counseling parents. We sought to identify antenatal parameters that predicted neonatal mortality in CDH. METHOD: Search was conducted in MEDLINE, EMBASE, Cochrane Database of Systematic reviews, PubMed, Scopus, and Web of Science on the ability of lung-to-head ratio (LHR), observed-to-expected LHR (o/e LHR), total fetal lung volume (TFLV), o/e TFLV, percentage predicted lung volume (PPLV) and degree of liver herniation to predict neonatal morbidity and mortality in fetuses with CDH...
December 21, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28087138/corrigendum-to-delayed-presentation-of-congenital-diaphragmatic-hernia-manifesting-as-combined-type-acute-gastric-volvulus-a-case-report-and-review-of-the-literature-j-pediatr-surg-43-3-2008-e35-9-doi-10-1016-j-jpedsurg-2007-11-015
#20
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