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Congenital diaphragmatic hernia

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https://www.readbyqxmd.com/read/28631351/intraoperative-acidosis-and-hypercapnia-during-thoracoscopic-repair-of-congenital-diaphragmatic-hernia-and-esophageal-atresia-tracheoesophageal-fistula
#1
Augusto Zani, Ruben Lamas-Pinheiro, Irene Paraboschi, Sebastian K King, Justyna Wolinska, Elke Zani-Ruttenstock, Simon Eaton, Agostino Pierro
BACKGROUND: Intraoperative hypercapnia and acidosis have been associated with thoracoscopic repair of both congenital diaphragmatic hernia and esophageal atresia/tracheoesophageal fistula. AIM: The aim of the present study was to investigate whether thoracoscopic repair of congenital diaphragmatic hernia or esophageal atresia/tracheoesophageal fistula was associated with acidosis and hypercapnia in a large group of neonates, and to analyze the effects of acidosis and hypercapnia on early postoperative outcomes...
June 20, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28629240/foetoscopic-endotracheal-occlusion-feto-for-severe-isolated-left-sided-congenital-diaphragmatic-hernia-single-centre-polish-experience
#2
Przemyslaw Kosinski, Miroslaw Wielgos
OBJECTIVE: To present early experience with foetoscopic endotracheal occlusion (FETO) for congenital diaphragmatic hernia (CDH) in a new centre in Poland. METHODS: This was a prospective study in singleton pregnancies with CDH treated by FETO between 2014 and 2016 in the Medical University of Warsaw, Poland. FETO was carried out at 25.6-30.1 (median 27.7) weeks' gestation in 28 consecutive cases of isolated left-sided CDH with observed over expected lung area to head circumference ratio (o/e LHR) of 20...
June 20, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28622971/utilization-patterns-of-extracorporeal-membrane-oxygenation-in-neonates-in-the-united-states-1997-2012
#3
Ashley Y Song, Hsuan-Hsiu Annie Chen, Rachel Chapman, Ameish Govindarajan, Jeffrey S Upperman, Rita V Burke, James Stein, Philippe S Friedlich, Ashwini Lakshmanan
BACKGROUND: Extracorporeal membrane oxygenation (ECMO) remains one of the most intensive therapies for newborns in the United States. However, there is limited information on resource utilization for neonates receiving ECMO. METHODS: We conducted a retrospective data analysis of the Kids' Inpatient Database from 1997 to 2012. Bivariate and multivariate analysis was completed to identify predictors of LOS, hospital costs and mortality. Cardiac and non-cardiac diagnoses of neonates receiving ECMO were also included in the bivariate and multivariable analysis...
June 1, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28620733/management-of-congenital-diaphragmatic-hernia-in-newborn-paradigm-shift-and-ethical-issues
#4
Sushmita Nitin Bhatnagar, Yogesh Kumar Sarin
Management of congenital diaphragmatic hernia (CDH) begins soon after it is detected, whether antenatally or postnatally. Assessment of the severity of the condition, associated congenital anomalies, maternal health and related issues, weight of the fetus/baby, mode of delivery, timing of delivery, immediate appropriate management of the baby with CDH at birth, appropriate utilization of available treatment modalities as well as infrastructure of the treating institute have an impact on the outcome of the neonate...
June 16, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28609176/mothers-of-infants-with-congenital-diaphragmatic-hernia-describe-breastfeeding-in-the-neonatal-intensive-care-unit-as-long-as-it-s-my-milk-i-m-happy
#5
Elizabeth B Froh, Janet A Deatrick, Martha A Q Curley, Diane L Spatz
BACKGROUND: Very little is known about the breastfeeding experience of mothers of infants born with congenital anomalies and cared for in the neonatal intensive care unit (NICU). Often, studies related to breastfeeding and lactation in the NICU setting are focused on the mothers of late preterm, preterm, low-birth-weight, and very-low-birth-weight infants. Congenital diaphragmatic hernia (CDH) is an anatomic malformation of the diaphragm and affects 1 in every 2,000 to 4,000 live births...
June 1, 2017: Journal of Human Lactation: Official Journal of International Lactation Consultant Association
https://www.readbyqxmd.com/read/28607625/hepatic-portal-venous-gas-in-children-younger-than-2-years-old-radiological-and-clinical-characteristics-in-diseases-other-than-necrotizing-enterocolitis
#6
Marzena Barczuk-Falęcka, Przemysław Bombiński, Zofia Majkowska, Michał Brzewski, Stanisław Warchoł
BACKGROUND: Hepatic portal venous gas (HPVG) is a rare imaging finding in children. It can be an important manifestation of severe diseases such as necrotizing enterocolitis (NEC) in neonates or bowel wall rupture in older children. However, there are many other diseases presenting with HPVG that do not necessarily require a surgical intervention. CASE REPORT: In the period between 2011-2015, there were 12 cases of HPVG in children aged up to 24 months in our hospital...
2017: Polish Journal of Radiology
https://www.readbyqxmd.com/read/28607284/a-comparison-of-clinical-outcomes-between-endoscopic-and-open-surgery-to-repair-neonatal-diaphragmatic-hernia
#7
Ma Lishuang, Wei Yandong, Liu Shuli, Feng Cuiru, Zhang Yue, Wang Ying, Zhang Yanxia, Sun Bin, Li Jingna, Li Long
OBJECTIVE: The objective of this study is to evaluate the clinical efficacies of open versus endoscopic surgery in the treatment of congenital diaphragmatic hernia (CDH) and investigate the feasibility and safety of endoscopic surgery as an alternative to open surgery in these cases. PATIENTS AND METHODS: A retrospective analysis was performed from June 2002 to February 2014. A total of 59 cases were attempted. The neonates were divided into either an endoscopic or open surgery group...
July 2017: Journal of Minimal Access Surgery
https://www.readbyqxmd.com/read/28602525/factors-related-to-long-term-surgical-morbidity-in-congenital-diaphragmatic-hernia-survivors
#8
Stan Janssen, Kim Heiwegen, Iris Alm van Rooij, Horst Scharbatke, Jolt Roukema, Ivo de Blaauw, Sanne Mbi Botden
BACKGROUND: Patients born with a congenital diaphragmatic hernia (CDH) have a high mortality and morbidity. After discharge, complications and long-term morbidity are still encountered. This study describes the factors related to the surgical long-term outcomes in CDH survivors. METHODS: A cohort of CDH patients born between 2000 and 2014, with a minimum of two years follow up, were included in this retrospective study. Demographics, CDH specific characteristics, treatment, and long-term surgical outcome were evaluated using multivariate logistic regression analyses...
June 3, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28600484/perinatal-case-of-fatal-simpson-golabi-behmel-syndrome-with-hyperplasia-of-seminiferous-tubules
#9
Nives Zimmermann, Jerzy Stanek
BACKGROUND Simpson-Golabi-Behmel syndrome (SGBS) is a rare X-linked recessive syndrome characterized by fetal overgrowth. CASE REPORT We present a case of a male infant with SGBS. Abnormal prenatal ultrasound (including congenital diaphragmatic hernia) prompted microarray testing of amniotic fluid cells, which showed deletion on chromosome Xq26.2 affecting the glypican-3 gene consistent with SGBS type I. The infant died six hours after birth and at autopsy showed features of SGBS, including macrosomia, organomegaly, diaphragmatic hernia with consequent pulmonary hypoplasia, cleft palate, large tongue with a midline groove, a supernumerary nipple, Meckel's diverticulum, and abnormal phalanges...
June 10, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28592524/loss-of-function-in-robo1-is-associated-with-tetralogy-of-fallot-and-septal-defects
#10
Paul Kruszka, Pranoot Tanpaiboon, Katherine Neas, Kathleen Crosby, Seth I Berger, Ariel F Martinez, Yonit A Addissie, Yupada Pongprot, Rekwan Sittiwangkul, Suchaya Silvilairat, Krit Makonkawkeyoon, Lan Yu, Julia Wynn, James T Bennett, Heather C Mefford, William T Reynolds, Xiaoqin Liu, Mathilda T M Mommersteeg, Wendy K Chung, Cecilia W Lo, Maximilian Muenke
BACKGROUND: Congenital heart disease (CHD) is a common birth defect affecting approximately 1% of newborns. Great progress has been made in elucidating the genetic aetiology of CHD with advances in genomic technology, which we leveraged in recovering a new pathway affecting heart development in humans previously known to affect heart development in an animal model. METHODS: Four hundred and sixteen individuals from Thailand and the USA diagnosed with CHD and/or congenital diaphragmatic hernia were evaluated with chromosomal microarray and whole exome sequencing...
June 7, 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/28549685/advanced-minimal-access-surgery-in-infants-weighing-less-than-3kg-a-single-center-experience
#11
James K Wall, Tiffany J Sinclair, William Kethman, Christina Williams, Craig Albanese, Karl G Sylvester, Matias Bruzoni
BACKGROUND: Minimal access surgery (MAS) has gained popularity in infants less than 5kg, however, significant challenges still arise in very low weight infants. STUDY DESIGN: A retrospective chart review was performed to identify all infants weighing less than 3kg who underwent an advanced MAS or equivalent open procedure from 2009 to 2016. Advanced case types included Nissen fundoplication, duodenal atresia repair, Ladd procedure, congenital diaphragmatic hernia repair, esophageal atresia/tracheoesophageal fistula repair, diaphragmatic plication, and pyloric atresia repair...
May 11, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28529538/emergency-surgery-due-to-diaphragmatic-hernia-case-series-and-review
#12
REVIEW
Mario Testini, Antonia Girardi, Roberta Maria Isernia, Angela De Palma, Giovanni Catalano, Angela Pezzolla, Angela Gurrado
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a congenital abnormality, rare in adults with a frequency of 0.17-6%. Diaphragmatic rupture is an infrequent consequence of trauma, occurring in about 5% of severe closed thoraco-abdominal injuries. Clinical presentation ranges from asymptomatic cases to serious respiratory or gastrointestinal symptoms. Diagnosis depends on anamnesis, clinical signs and radiological investigations. METHODS: From May 2013 to June 2016, six cases (four females, two males; mean age 58 years) of diaphragmatic hernia were admitted to our Academic Department of General Surgery with respiratory and abdominal symptoms...
2017: World Journal of Emergency Surgery: WJES
https://www.readbyqxmd.com/read/28527575/coup-tf-genes-human-diseases-and-the-development-of-the-central-nervous-system-in-murine-models
#13
Xiong Yang, Su Feng, Ke Tang
COUP-TFI and -TFII are members of the steroid/thyroid nuclear receptor superfamily. Recent clinical studies reveal that COUP-TFI gene mutations are associated with Bosch-Boonstra-Schaaf optic atrophy syndrome displaying symptoms of optic atrophy, intellectual disability, hypotonia, seizure, autism spectrum disorders, oromotor dysfunction, thin corpus callosum, or hearing defects, and COUP-TFII gene mutations lead to congenital heart defects and/or congenital diaphragmatic hernia with developmental delay and mental defects...
2017: Current Topics in Developmental Biology
https://www.readbyqxmd.com/read/28527042/parenting-stress-among-parents-of-children-with-congenital-diaphragmatic-hernia
#14
Elin Öst, Margret Nisell, Björn Frenckner, Carmen Mesas Burgos, Maria Öjmyr-Joelsson
PURPOSE: The aim of this study was to examine parental stress among parents of children with congenital diaphragmatic hernia (CDH). METHODS: Between 2005 and 2009, a total of 51 children with CDH were treated at Astrid Lindgren Children's Hospital. The survival rate at discharge was 86% and long-term survival rate 80%. One parent each of the long-term survivors (41 children) was included in the present study, and 34 parents (83%) agreed to participate. Participants received the Swedish Parenthood Stress Questionnaire (SPSQ)...
May 19, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28512408/cytoreductive-surgery-followed-by-hyperthermic-intraperitoneal-chemotherapy-for-recurrent-ovarian-cancer-with-incidental-bochdalek-hernia-and-postoperative-bilateral-thalamic-infarct-a-case-report
#15
Ilker Kahramanoglu, Hasan Turan, Ece Yamak Altinpulluk, Zahid Mammadov, Tugan Bese, Macit Arvas, Fuat Demirkiran
Congenital Bochdalek hernia is a defect of the diaphragm and very rare in adults. Only around 100 cases have been reported in the literature. Herein, we present a case with a recurrent ovarian cancer who underwent secondary cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. An oval defect with dimensions of 3 × 4 cm was seen in the left posterolateral site of the diaphragm during surgical exploration. In addition, a 6 × 3 cm iatrogenic right-sided diaphragmatic defect was found and repaired...
January 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28500579/robotic-repair-of-a-right-sided-bochdalek-hernia-a-case-report-and-literature-review
#16
Amani Jambhekar, Shawn Robinson, Brian Housman, James Nguyen, Kevin Gu, Vadim Nakhamiyayev
BACKGROUND: Bochdalek hernias (BHs) are usually diagnosed in the neonatal period, occurring in 1/2200-1/12,500 live births. There are few reported cases of BHs in adults. Robotic repair has not been described in current literature as opposed to the laparoscopic approach. Here we present a case of an adult with clinical signs of bowel obstruction secondary to a BH which was repaired using a robotic approach. CASE REPORT: A 74-year-old gentleman with past medical history of benign prostatic hyperplasia presented to the emergency department with a 1-week history of nausea, vomiting, diarrhea, and decline in appetite...
May 12, 2017: Journal of Robotic Surgery
https://www.readbyqxmd.com/read/28497001/klinefelter-syndrome-in-association-with-tetralogy-of-fallot-and-congenital-diaphragmatic-hernia
#17
Sethuraman Swaminathan, Sudheer R Gorla, Deborah S Barbouth
Klinefelter syndrome (KS) is the most common sex chromosomal aneuploidy in males. Major cardiovascular and diaphragmatic anomalies are uncommon in this syndrome. Here we report an infant with KS who had tetralogy of Fallot and congenital diaphragmatic hernia, all of which were identified prenatally and managed successfully after birth. Microarray analysis did not reveal any deletions or duplications other than the additional X-chromosome, to account for the additional abnormalities in this infant. To the authors' knowledge, this is the first such report of major cardiac and diaphragm anomaly occurring together, in an infant with KS...
June 2017: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/28496996/anterior-abdominal-wall-defects-diaphragmatic-hernia-and-other-major-congenital-malformations-of-the-musculoskeletal-system-in-barbados-1993-2012
#18
Keerti Singh, Alok Kumar
This study describes the prevalence and patterns of major congenital malformations of the musculoskeletal system and the resulting morbidity and mortality. It is a retrospective population-based study over the period 1993 to 2012. The overall prevalence of major congenital malformations of the musculoskeletal system was 9.02/10,000 live births. The prevalences of omphalocele, gastroschisis, and diaphragmatic hernia were 2.53, 2.22, and 1.42 per 10,000 live births, respectively. The case fatality ratio for the omphalocele, gastroschisis, and diaphragmatic hernia was 12...
June 2017: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/28492159/iloprost-instillation-in-two-neonates-with-pulmonary-hypertension
#19
Sezin Unal, Selma Aktas, Meltem Aksu, Ibrahim M Hirfanoglu, Yildiz Atalay, Canan Turkyilmaz
Pulmonary hypertension may coexist with certain diseases in neonates. Iloprost inhalation is one of the treatments which cause selective pulmonary vasodilatation. Inhalation is not an easy way of drug administration in mechanically ventilated infants; as some exhibit desaturations during inhalation. Moreover, inhalation of drug requires cessation of mechanical ventilation, if patient is on high frequency oscillatory ventilation. We presented two patients with pulmonary hypertension; term baby with congenital diaphragmatic hernia and preterm baby with respiratory distress syndrome; who had iloprost instillation during mechanical ventilation treatment...
April 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28489671/closer-look-at-the-nutritional-outcomes-of-patients-after-primary-repair-of-congenital-diaphragmatic-hernia
#20
Lan T Vu, Carrie McFarland, Barbara Bratton, Hanmin Lee
OBJECTIVES: We hypothesize that the patients after primary repair of congenital diaphragmatic hernia (CDH) can have poor nutritional outcomes and plan to identify risk factors to further stratify these patients. METHODS: Retrospective cohort of patients who had primary repair of CDH between 2000 and 2014 and had follow-up at our institution. Z-scores (weight for age and weight for length) were calculated using the WHO and CDC growth standards. RESULTS: For the 67 patients in the cohort, the median age at the time of repair was 3 days (IQR 2-5) and at the time of discharge was 20...
May 9, 2017: Journal of Pediatric Gastroenterology and Nutrition
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