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diaphragmatic hernia

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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
#1
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
#2
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
#3
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/28092257/-chilaiditi-s-sign-and-syndrome-case-report
#4
César Raúl Aguilar-García, Jesús García-Acosta
The interposition of a portion of the colon between the liver and the diaphragm is called Chilaiditi sign and discovered incidentally during radiological study for other reasons and usually asymptomatic presentation. When the discovery is accompanied by clinical symptoms such as abdominal pain, nausea, vomiting, bloating, constipation called Chilaiditi Syndrome. The Chilaiditi sign is a very rare condition, and the Chilaiditi syndrome is even more, especially if associated with other acute diseases. We report the case of a man of 41 years was admitted with right upper quadrant abdominal pain, nausea, vomiting, difficulty was diagnosed with radiographic and tomographic images making the differential diagnosis with lung abscess and diaphragmatic hernia...
January 2017: Revista Médica del Instituto Mexicano del Seguro Social
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
#5
https://www.readbyqxmd.com/read/28081579/predicting-full-enteral-feeding-in-the-postoperative-period-in-infants-with-congenital-diaphragmatic-hernia
#6
Carlos Zozaya, Miryam Triana, Rosario Madero, Steven Abrams, Leopoldo Martinez, Maria Virginia Amesty, Miguel Sáenz de Pipaón
Introduction The objective of the study is to examine the factors associated with time to achieve full enteral feeding after repair of congenital diaphragmatic hernia. Materials and Methods Demographic, clinical, and therapeutic data were retrospectively assessed, and uni- and multivariate Cox regression were performed to examine factors predictive of achieving full enteral feeding that was defined as time to achieve120 mL/kg/d after surgical repair. Results Of 78 infants, 66 underwent intervention before hospital discharge...
January 12, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28079865/differences-in-mortality-characteristics-in-neonates-with-down-s-syndrome
#7
C L Cua, U Haque, S Santoro, L Nicholson, C H Backes
OBJECTIVE: Neonates with Down's syndrome (nDS) may have multiple medical issues that place them at increased risk for mortality during the newborn period. Goal of this study was to determine if there are differences in baseline characteristics, medical complications or procedures performed during hospitalization between nDS who survived versus those who died during initial hospitalization. STUDY DESIGN: Data from 2000 to 2014 were reviewed using the Pediatric Health Information Systems (PHIS) database on all DS patients admitted to the hospital <30 days postnatal life...
January 12, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28078737/3d-assessment-of-the-umbilical-vein-deviation-angle-uvda-for-the-prediction-of-liver-herniation-in-left-congenital-diaphragmatic-hernia
#8
Nicola Volpe, Eleonora Mazzone, Brunella Muto, Alice Suprani, Tiziana Fanelli, Christine Tita Kaihura, Andrea Dall'Asta, Giuseppe Pedrazzi, Carmine Del Rossi, Enrico Maria Silini, Cinzia Magnani, Paolo Volpe, Tullio Ghi, Tiziana Frusca
OBJECTIVES: To introduce a new sonographic marker of intrathoracic liver herniation in fetuses with left congenital diaphragmatic hernia (CDH) METHODS: In a consecutive series of fetuses with isolated CDH an ultrasound volume of the fetal abdomen was acquired. On this the offline calculation of the angle formed by the midline of the abdomen (joining the center of the vertebral body to the abdominal insertion of the umbilical cord) and a second line joining the center of the vertebral body to the intra-abdominal convexity of the umbilical vein was carried out (Umbilical Vein Deviation Angle or UVDA)...
January 12, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28070244/assessment-of-fetus-during-second-trimester-ultrasonography-using-hdlive-software-what-is-its-real-application-in-the-obstetrics-clinical-practice
#9
Gabriele Tonni, Gianpaolo Grisolia, Eduardo Félix Santana, Edward Araujo Júnior
AIM: To show imaging results from application of four-dimensional (4D) ultrasound lightening technique (HDlive™) in clinical obstetrics practice. METHODS: Normal and abnormal fetuses at second and third trimester of pregnancy undergoing routine scan with 4D HDlive™ (5DUS) in the rendering mode are described. Realistic features of fetal structures were provided by 5DUS in the rendering mode. Normal anatomy as well as pathology like cleft lip, hypoplastic face, micrognathia, low-set ears, corpus callosum, arthrogryposis, aortic arch, left congenital diaphragmatic hernia are highlighted in this study...
December 28, 2016: World Journal of Radiology
https://www.readbyqxmd.com/read/28061863/prognostic-indicators-for-perioperative-survival-after-diaphragmatic-herniorrhaphy-in-cats-and-dogs-96-cases-2001-2013
#10
Claire Legallet, Kelley Thieman Mankin, Laura E Selmic
BACKGROUND: To determine associations between perioperative mortality after surgery for traumatic diaphragmatic hernia, medical records of 17 cats and 79 dogs that underwent diaphragmatic herniorrhaphy were reviewed. RESULTS: The combined perioperative survival rate was 81.3% (88.2% in cats and 79.8% in dogs). Data from acute and chronic cases was assessed separately. Of the acute cases (12 cats and 48 dogs), 10 cats (83.3%) and 38 dogs (79.2%) survived to discharge...
January 7, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28056166/nitric-oxide-for-respiratory-failure-in-infants-born-at-or-near-term
#11
REVIEW
Keith J Barrington, Neil Finer, Thomas Pennaforte, Gabriel Altit
BACKGROUND: Nitric oxide (NO) is a major endogenous regulator of vascular tone. Inhaled nitric oxide (iNO) gas has been investigated as treatment for persistent pulmonary hypertension of the newborn. OBJECTIVES: To determine whether treatment of hypoxaemic term and near-term newborn infants with iNO improves oxygenation and reduces rate of death and use of extracorporeal membrane oxygenation (ECMO), or affects long-term neurodevelopmental outcomes. SEARCH METHODS: We used the standard search strategy of the Cochrane Neonatal Review Group to search the Cochrane Central Register of Controlled Trials (CENTRAL; 2016, Issue 1), MEDLINE via PubMed (1966 to January 2016), Embase (1980 to January 2016) and the Cumulative Index to Nursing and Allied Health Literature (CINAHL; 1982 to January 2016)...
January 5, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28055024/prenatally-diagnosed-congenital-diaphragmatic-hernia-optimal-mode-of-delivery
#12
C M Burgos, B Frenckner, M Luco, M T Harting, P A Lally, K P Lally
OBJECTIVE: To evaluate if the delivery mode of infants with prenatally diagnosed congenital diaphragmatic hernia (CDH) affects the outcome. STUDY DESIGN: Data from the CDH Study Group database of infants with prenatal diagnosis between 2001-2015 were divided into four delivery mode groups: vaginal spontaneous, vaginal induced, elective caesarean section, and emergent caesarean section. Outcomes were analyzed in relation to the time of day of delivery and the gestational age at birth...
January 5, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28051921/tube-thoracostomy-at-the-time-of-congenital-diaphragmatic-hernia-repair-reassessing-the-risks-and-benefits
#13
Avraham Schlager, Kelly Arps, Ragavan Siddharthan, Matthew S Clifton
PURPOSE: Postoperative pneumothorax and effusion remain a concern following congenital diaphragmatic hernia (CDH) repair. Despite a recent trend away from intraoperative thoracostomy, few studies have actually compared outcomes with and without a chest tube. Rationale commonly cited for the more minimalistic approach include the presumed low likelihood of postoperative complications, potential risk of patch infection, and prolonged intubation. We evaluate these theories, as well as the implications of intraoperative chest tube (IOCT) placement...
January 4, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/28050602/exome-sequencing-identifies-de-novo-pathogenic-variants-in-fbn1-and-trps1-in-a-patient-with-a-complex-connective-tissue-phenotype
#14
Diane B Zastrow, Patricia A Zornio, Annika Dries, Jennefer Kohler, Liliana Fernandez, Daryl Waggott, Magdalena Walkiewicz, Christine M Eng, Melanie A Manning, Ellyn Farrelly, Paul G Fisher, Euan A Ashley, Jonathan A Bernstein, Matthew T Wheeler
Here we describe a patient who presented with a history of congenital diaphragmatic hernia, inguinal hernia, and recurrent umbilical hernia. She also has joint laxity, hypotonia, and dysmorphic features. A unifying diagnosis was not identified based on her clinical phenotype. As part of her evaluation through the Undiagnosed Diseases Network, trio whole-exome sequencing was performed. Pathogenic variants in FBN1 and TRPS1 were identified as causing two distinct autosomal dominant conditions, each with de novo inheritance...
January 2017: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/28050431/asymptomatic-traumatic-hepatothorax-symptomatic-gall-stone-disease-a-rare-coincidence
#15
Somak Das, Dinesh Zirpe, Chandrasekharn Valiathan Gopakumar, Sudeepta Kumar Swain, Rajagopal Surendran
Traumatic diaphragmatic hernia rarely affects right side due to protective effect of liver. In adult it is mainly caused by blunt abdominal trauma. Acute presentations are often life threatening and usually clinch the diagnosis early. It may remain asymptomatic for many years unless being detected incidentally during investigations for some unrelated reason or getting complicated by some pathology of herniated viscera. High degree of suspicion is required to detect this delayed presentation particularly in a post-trauma patient as this condition may require modifications in management...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28049534/case-report-maternal-mosaicism-resulting-in-inheritance-of-a-novel-gata6-mutation-causing-pancreatic-agenesis-and-neonatal-diabetes-mellitus
#16
Daphne Yau, Elisa De Franco, Sarah E Flanagan, Sian Ellard, Miriam Blumenkrantz, John J Mitchell
BACKGROUND: Haploinsufficiency of the GATA6 transcription factor gene was recently found to be the most common cause of pancreatic agenesis, a rare cause of neonatal diabetes mellitus. Although most cases are de novo, we describe three siblings with inherited GATA6 haploinsufficiency and the rare finding of parental mosaicism. CASE PRESENTATION: The proband was born at term with severe intrauterine growth restriction, the first child of non-consanguineous parents...
January 3, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28043315/right-sided-congenital-diaphragmatic-hernia-and-myelomeningocele-a-rare-association
#17
Syed Rehan Ali, Shakeel Ahmed
Congenital diaphragmatic hernia (CDH) is a rare birth defect with a prevalence of < 0.5 per 1,000 live births. Majority of these defects are left-sided as most studies suggest that frequency of right-sided CDH was 10% of the total. The association of CDH with myelomeningocele (MMC) is extremely rare; as in Sweed's study of 116 consecutive cases of CDH, the incidence of associated MMC with CDH was stated as 4.3%. There has been one previous case report of leftsided CDH, MMC and hydrocephalus prenatally diagnosed; but to the best of authors' knowledge, this is the first reported case of the above constellation with a right-sided CDH diagnosed prenatally...
December 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28040456/exhaled-breath-condensate-analysis-from-intubated-newborns-by-nano-hplc-coupled-to-high-resolution-ms
#18
A S Kononikhin, N L Starodubtseva, V V Chagovets, A Y Ryndin, A A Burov, I A Popov, A E Bugrova, R A Dautov, A O Tokareva, Y L Podurovskaya, O V Ionov, V E Frankevich, E N Nikolaev, G T Sukhikh
Invasiveness of examination and therapy methods is a serious problem for intensive care and nursing of premature infants. Exhaled breath condensate (EBC) is the most attractive biofluid for non-invasive methods development in neonatology for monitoring the status of intubated infants. The aim of the study was to propose an approach for EBC sampling and analysis from mechanically ventilated neonates. EBC collection system with good reproducibility of sampling was demonstrated. Discovery-based proteomic and metabolomic studies were performed using nano-HPLC coupled to high resolution MS...
December 23, 2016: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/28040139/prenatal-diagnosis-of-paternal-duplication-of-11p15-5%C3%A2-14-3-its-implication-of-beckwith-wiedemann-syndrome
#19
Kuan Ju Chen, Yu Mei Liu, Chien Hong Li, Yao Lung Chang, Shuenn Dyh Chang
OBJECTIVE: To characterize a prenatally detected chromosomal aberration with molecular cytogenetic approaches and explore its relationship with Beckwith-Wiedemann syndrome (BWS). CASE REPORT: A 33-year-old woman, gravida 2, para 0, was referred to our prenatal clinic at 20+ weeks due to an abnormal amniocentesis karyotyping finding, which showed 46,XY,add(11)(q24.2)dn. The mother conceived through in vitro fertilization-intracytoplasmic sperm injection (IVF-ICSI), then embryo transfer...
December 2016: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28040135/pallister-killian-syndrome-cytogenetics-and-molecular-investigations-of-mosaic-tetrasomy-12p-in-prenatal-chorionic-villus-and-in-amniocytes-strategy-of-prenatal-diagnosis
#20
Francesco Libotte, Domenico Bizzoco, Ivan Gabrielli, Alvaro Mesoraca, Pietro Cignini, Salvatore Giovanni Vitale, Ilaria Marilli, Ferdinando Antonio Gulino, Agnese Maria Chiara Rapisarda, Claudio Giorlandino
OBJECTIVE: Pallister-Killian syndrome (PKS) is a rare, sporadic genetic disorder caused by mosaic tetrasomy of the short arm of chromosome 12 (12p). Clinically, PKS is characterized by several systemic abnormalities, such as intellectual impairment, hearing loss, epilepsy, hypotonia, craniofacial dysmorphism, pigmentary skin anomalies, epilepsy, and a variety of congenital malformations. Prenatally, PKS can be suspected in the presence of ultrasound anomalies: diaphragmatic hernia, rhizomelic micromelia, hydrops fetalis, fetal overweight, ventriculomegaly in the central nervous system, congenital heart defects, or absent visualization of the stomach...
December 2016: Taiwanese Journal of Obstetrics & Gynecology
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