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Congenital hernia

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https://www.readbyqxmd.com/read/28325581/prenatal-prediction-of-pulmonary-hypoplasia
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
https://www.readbyqxmd.com/read/28285557/trisomy-13-with-prenatally-diagnosed-congenital-cystic-adenomatoid-malformation-and-hernia-of-the-umbilical-cord-a-case-report
#9
Kohei Nakamura, Showa Aoki, Tomoko Ishihara, Kentaro Nakayama, Haruhiko Kanasaki, Satoru Kyo
No abstract text is available yet for this article.
March 13, 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28278439/a-case-of-single-incision-laparoscopic-surgery-for-a-bleeding-meckel-s-diverticulum-diagnosed-pre-operatively-by-double-balloon-endoscopy
#10
Toshio Shiraishi, Tetsuro Tominaga, Takashi Nonaka, Kouki Wakata, Masaki Kunizaki, Shuichi Tobinaga, Yorihisa Sumida, Shigekazu Hidaka, Naoe Kinoshita, Terumitsu Sawai, Takeshi Nagayasu
INTRODUCTION:  Meckel's diverticulum (MD) is a congenital true diverticulum that is residual yolk duct tissue, and some cases with complications require surgery. It has been reported that laparoscopic surgery is effective for patients with an MD. PRESENTATION OF CASE: A 79-year-old man with melena visited our hospital. Upper gastrointestinal series and colonoscopy showed no bleeding lesion. Double-balloon endoscopy was then performed to examine the small intestine...
February 22, 2017: International Journal of Surgery Case Reports
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
#11
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
#12
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/28273609/a-rare-case-of-a-strangulated-littre-s-hernia-with-meckel-s-diverticulum-duplication-case-report-and-literature-review
#13
C R López-Lizárraga, M P Sánchez-Muñoz, G E Juárez-López, L Pelayo-Orozco, L F De la Cerda-Trujillo, C F Ploneda-Valencia
INTRODUCTION: The Meckel's diverticulum (MD) is the most common congenital anomaly of the gastrointestinal tract present in approximately 1-4% of the population; the MD duplication is exceedingly rare with only a few reports of it. Here we present the firs case of a strangulated Littre's hernia with MD duplication. PRESENTATION OF CASE: A 30-year-old male presented to the emergency room with clinical signs of small bowel obstruction, at physical examination, a right incarcerated inguinal hernia with erythema was found...
February 21, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28270239/-late-presentation-of-congenital-diaphragmatic-hernia
#14
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/28267443/balloon-removal-after-fetoscopic-endoluminal-tracheal-occlusion-for-congenital-diaphragmatic-hernia
#15
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/28260541/aortic-arch-advancement-for-type-a-interrupted-aortic-arch-with-persistent-fifth-aortic-arch-type-b
#16
Ziyad M Binsalamah, Peter Chen, Emmett D McKenzie
Persistence of the fifth aortic arch is a very rare anomaly, but is clinically relevant when it is associated with coarctation. We report a case of a neonate with type A interrupted aortic arch and severe coarctation of a persistent fifth aortic arch, which was discovered after repair of a left congenital diaphragmatic hernia. The combination of anomalies was discovered intra-operatively following left thoracotomy, and was treated with aortic arch advancement. The postoperative course was uneventful.
March 6, 2017: Cardiology in the Young
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
#17
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
#18
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/28223011/extracorporeal-membrane-oxygenation-support-in-neonates-a-single-medical-center-experience-in-taiwan
#19
Chi-Man Kuok, Po-Nien Tsao, Chien-Yi Chen, Hung-Chieh Chou, Wu-Shiun Hsieh, Shu-Chien Huang, Yih-Sharng Chen, En-Ting Wu
BACKGROUND: Extracorporeal membrane oxygenation (ECMO) was used in neonates with severe cardiopulmonary failure who failed to respond to conventional therapy. We started to apply neck venoarterial ECMO (VA-ECMO) in neonatal patients from 2000. In this study, we have focused on neonates who received ECMO support and described the current status of ECMO in neonates for both cardiac and pulmonary support and the risk factors associated with their outcomes. METHODS: Data were retrieved from our ECMO database for the neonates (age < 28 days) who received neck VA-ECMO support from January 2005 to June 2015...
January 17, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28214762/paracaecal-hernia-a-case-report-on-the-evolving-role-of-laparoscopy
#20
Ammar Tayaran, Haider Abdulrasool, Hai T Bui
A paracaecal hernia, a type of pericaecal hernias, is a rare cause of small intestinal obstruction. Failure of early recognition and reduction of this type of internal hernia may lead to strangulation of the herniated intestine. There has been a number of case reports in the literature about the different types of pericaecal hernias, however the anatomy of these hernias is still poorly understood and the management is still evolving. We are presenting a 75year old woman, who presented clinically and radiologically with distal small intestinal obstruction...
January 17, 2017: International Journal of Surgery Case Reports
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