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diaphragmatic hernia

Katarina Bojanić, Ruža Grizelj, Jurica Vuković, Lana Omerza, Marina Grubić, Tomislav Ćaleta, Toby N Weingarten, Darrell R Schroeder, Juraj Sprung
BACKGROUND: Patients with congenital diaphragmatic hernia (CDH) have a high residual morbidity rate. We compared self-reported health-related quality of life (HRQoL) between patients with CDH and healthy children. METHODS: Forty-five patients with CDH who were born from January 1, 1990, through February 15, 2015, were matched to healthy, age-matched control participants at a 1:2 ratio. The health records of the study participants were reviewed to determine comorbid conditions, and HRQoL was assessed by both the participants and their parents with the Pediatric Quality of Life Inventory (PedsQL)...
March 14, 2018: Health and Quality of Life Outcomes
Joanna L Kaplan, Catherine T Gunther-Harrington, Jessie S Sutton, Joshua A Stern
BACKGROUND: The teratogenic effects of immunomodulatory and certain antimicrobial therapies are described in small rodents and humans. While the described teratogenic effects in small rodents have been extrapolated to make conclusions about its use in the pregnant dam, teratogenic effects of prednisone and doxycycline have not yet been reported in the dog. Here we report and describe midline defects observed in a litter of golden retriever puppies exposed to mid-gestational immunosuppressive and antimicrobial therapy...
March 12, 2018: BMC Veterinary Research
Solina Tith, Garinder Bining, Laurent Bollag
Background : Opioid use during pregnancy is a growing concern in the United States. Buprenorphine has been recommended by "The American College of Obstetrics and Gynecology" as an alternative to methadone to decrease risks associated with the use of illicit opioids during pregnancy. The partial μ-opioid agonists' unique pharmacology, including its long half time and high affinity to the μ-opioid receptor, complicates patient management in a highly kinetic, and often urgent field like obstetric anesthesia...
2018: F1000Research
Gabriel Altit, Shazia Bhombal, Krisa Van Meurs, Theresa A Tacy
INTRODUCTION: Newborns with congenital diaphragmatic hernia (CDH) have varying degrees of pulmonary hypoplasia and pulmonary hypertension (PH), and there is limited evidence that cardiac dysfunction is present. We sought to study early neonatal biventricular function and performance in these patients by reviewing early post-natal echocardiography (ECHO) measurements and comparing them to normal term newborns. METHODS: Retrospective case-control study reviewing clinical and ECHO data on term newborns with CDH and normal controls born between 2009 and 2016...
March 9, 2018: Pediatric Cardiology
Karina Miura Costa, Amulya Kumar Saxena
BACKGROUND: Postoperative chylothorax occurs due to trauma to lymphatic vessels and can occur after any thoracic procedure. This study reviewed recent literature to evaluate the management and outcomes of surgical chylothorax in neonates. METHODS: PubMed database was searched for articles in English, Portuguese and Spanish from 2000 to 2016. Data were collected for surgery, chylothorax management, complications, mortality and length of hospital stay (LOS). RESULTS: Twenty studies offered 107 neonates: congenital diaphragmatic hernia (CDH) (n = 76, 71%), cardiac malformations (n = 25, 23...
March 5, 2018: World Journal of Pediatrics: WJP
Siyu Liu, Mugen Dai, Bin Ye, Zhigang Zhao, Yang Shi, Lirong Peng
RATIONALE: Diaphragmatic Hernia is rare as complication of Colonoscopy. Diaphragmatic hernia as a complication of colonoscopy has been reported only few cases. Additionally, it is often misdiagnosed as other disease by clinicians due to their lack of related knowledge, which delays diagnosis of Diaphragmatic hernia and thus exacerbates the prognosis. PATIENT CONCERNS: We report the case of a 66-year-old man with fecal occult blood. In the case, sudden epigastric pain after colonoscopy owing to diaphragmatic hernia in a left hemidiaphragm...
January 2018: Medicine (Baltimore)
Matthew Wong, Janette Reyes, Eveline Lapidus-Krol, Monping Chiang, Tilman Humpl, Malikah Al-Faraj, Greg Ryan, Priscilla P L Chiu
Prenatal observed/expected lung-to-head ratio (O/E LHR) by ultrasound correlates with postnatal mortality for congenital diaphragmatic hernia (CDH) patients. The aim of this study is to determine if O/E LHR correlates with pulmonary hypertension (PH) outcomes for CDH patients. METHODS: A single center retrospective chart review was performed for CDH neonates from January 1, 2006, to December 31, 2015, (REB #1000053124) to include prenatal O/E LHR, liver position, first arterial blood gas, repair type, echocardiogram (ECHO), and lung perfusion scan (LPS) results up to 5years of age...
February 7, 2018: Journal of Pediatric Surgery
Q Jiang, W Y Liu, W Feng, H H Jia, F Hou
Objective: To investigate the effect of prenatal administration of tetrandrine on the expression of Kruppel-like factors 5 (KLF5) and Survivin in the lung of congenital diaphragmatic hernia (CDH) rat model. Methods: This experiment took simple random group method, 11 pregnant SD rats were randomly divided into three groups: normal control group (group NC, 3), model control group (group MC, 4), model+ tetrandrine group (group MT, 4). The rats in group MC and MT were given gavage administration with 125 mg nitrofen to induce CDH and the rats in group MT were administered with 30 mg/kg tetrandrine for three days (gestational age of 18...
February 13, 2018: Zhonghua Yi Xue za Zhi [Chinese medical journal]
M O Ongele, E Benrashid, B F Gilmore, J Schroder, M Hartwig, S Zani
Use of ventricular assist devices (VADs) is increasingly common, as is the need for surgeons to be familiar with the management of common complications in this population. Nonetheless, repair of diaphragmatic hernias which commonly develop following VAD implantation remains technically challenging due to intra-abdominal adhesions and the proximity of vital structures. Despite the potential benefits of improved dexterity and visualization, robotic approaches have thus far not been used to address this. We present the first two described cases of robot-assisted repair of diaphragmatic hernias in the setting of prior or current VAD placement...
February 2018: Journal of Surgical Case Reports
Ivo Soldo, Ivo Baća, Marko Sever, Ivan Zoričić, Martin Grbavac, Rosana Troskot Perić, Zoran Čala, Maja Karaman Ilić, Anamaria Soldo, Branko Bakula
We report on three cases of diaphragmatic (Morgagni) hernia with different clinical presentation. It is important to consider the possibility of this rare but potentially very dangerous condition in patients with respiratory problems and pain in the upper abdomen. Before laparoscopy, two different approaches were used in diaphragmatic hernia operations (abdominal and thoracic approach). Laparoscopy has brought significant changes in the treatment of diaphragmatic hernia. It is important to stress that laparoscopic diaphragmatic surgical therapy uses stronger mesh than the mesh used to repair an inguinal hernia...
June 2017: Acta Clinica Croatica
Bashar Aldeiri, Urmas Roostalu, Alessandra Albertini, Julia Behnsen, Jason Wong, Antonino Morabito, Giulio Cossu
Pentalogy of Cantrell (PC) is a rare multi-organ congenital anomaly that impedes ventral body wall closure and results in diaphragmatic hernia, intra- and pericardial defects. The underlying cellular and molecular changes that lead to these severe developmental defects have remained unknown largely due to the lack of representative animal models. Here we provide in depth characterization of a mouse model with conditional ablation of TGFβRII in Transgelin (Tagln) expressing cells. We show that Tagln is transiently expressed in a variety of cells that participate in the embryonic development and patterning of ventral structures...
February 26, 2018: Scientific Reports
Lennart van der Veeken, Francesca M Russo, Johannes van der Merwe, David Basurto, Dyuti Sharma, Tram Nguyen, Marie P Eastwood, Namesh Khoshgoo, Jaan Toelen, Karel Allegaert, Philip Dekoninck, Stuart B Hooper, Richard Keijzer, Paolo DE Coppi, Jan Deprest
Congenital diaphragmatic hernia is rare birth defect, which can be easily corrected after birth. The main problem is that herniation of viscera during fetal development impairs lung development, leading to a 30 % mortality and significant morbidity. In isolated cases the outcome can be accurately predicted prenatally by medical imaging. Cases with a poor prognosis can be treated before birth; clinically this is by fetoscopic endoluminal tracheal occlusion. Obstruction of the airways triggers lung growth. This procedure and is currently being evaluated in a global clinical trial for left sided cases; right sided cases with poor prognosis are offered the procedure clinically...
February 23, 2018: Minerva Pediatrica
Anu Bashamboo, Caroline Eozenou, Anne Jorgensen, Joelle Bignon-Topalovic, Jean-Pierre Siffroi, Capucine Hyon, Attila Tar, Péter Nagy, Janos Sólyom, Zita Halász, Annnabel Paye-Jaouen, Sophie Lambert, David Rodriguez-Buritica, Rita Bertalan, Laetitia Martinerie, Ewa Rajpert-De Meyts, John C Achermann, Ken McElreavey
Emerging evidence from murine studies suggests that mammalian sex determination is the outcome of an imbalance between mutually antagonistic male and female regulatory networks that canalize development down one pathway while actively repressing the other. However, in contrast to testis formation, the gene regulatory pathways governing mammalian ovary development have remained elusive. We performed exome or Sanger sequencing on 79 46,XX SRY-negative individuals with either unexplained virilization or with testicular/ovotesticular disorders/differences of sex development (TDSD/OTDSD)...
February 16, 2018: American Journal of Human Genetics
José Ricardo N DE Souza Neto, Érika Branco, Elane G Giese, Ana Rita DE Lima
The wall of the diaphragm can be affected by congenital or acquired alterations which allow the passage of viscera between the abdominal and chest cavities, allowing the formation of a diaphragmatic hernia. We characterized morphology and performed biometrics of the diaphragm in the common squirrel monkey Saimiri sciureus. After fixation, muscle fragments were collected and processed for optical microscopy. In this species the diaphragm muscle is attached to the lung by phrenopericardial ligament. It is also connected to the liver via the coronary and falciform ligaments...
February 19, 2018: Anais da Academia Brasileira de Ciências
Catarina Barroso, Jorge Correia-Pinto
Although improvements have been made, the management of congenital diaphragmatic hernia (CDH) remains a major challenge for perinatologists and neonatal surgeons. Many aspects of the disease remain unknown and, being a rare entity, evidence-based data are hard to find. Surgical morbidity is considerable and affects long-term quality of life. Perioperative complications have been reviewed focusing on thoracoscopic repair. Intraoperative acidosis was more severe during thoracoscopy when compared with open surgery (OS), though it is possible that later neurodevelopment was not affected...
February 19, 2018: European Journal of Pediatric Surgery
Elin Öst, Björn Frenckner, Margret Nisell, Carmen Mesas Burgos, Maria Öjmyr-Joelsson
PURPOSE: The aim of this study was to examine health-related quality of life (HRQoL) in children born with congenital diaphragmatic hernia (CDH). METHODS: Between 1993 and 2003, a total of 102 children born with CDH were treated at Astrid Lindgren Children's hospital in Stockholm. In 2012, long-term survivors (n = 77) were asked to participate in the present study, which resulted in a 46% (n = 35) response rate. The KIDSCREEN-52 questionnaire was used for measuring HRQoL and a detailed review of medical records was performed...
February 16, 2018: Pediatric Surgery International
Daisuke Ieda, Ikumi Hori, Yuji Nakamura, Hironori Ohshita, Yutaka Negishi, Tsutomu Shinohara, Ayako Hattori, Takenori Kato, Sachiko Inukai, Katsumasa Kitamura, Tomoki Kawai, Osamu Ohara, Shinji Kunishima, Shinji Saitoh
INTRODUCTION: Filamin A (FLNA) is located in Xq28, and encodes the actin binding protein, filamin A. A mutation in FLNA is the most common cause of periventricular nodular heterotopia (PVNH), but a clear phenotype-genotype correlation has not been established. Indeed, some patients with a FLNA mutation have recently been shown to additionally have Ehlers-Danlos-like collagenopathy or macrothrombocytopenia. In an attempt to establish a clearer correlation between clinical symptoms and genotype, we have investigated a phenotype that involves thrombocytopenia in a patient with a truncation of the FLNA gene...
February 12, 2018: Brain & Development
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
Hailey Pinz, Louise C Pyle, Dong Li, Kosuke Izumi, Cara Skraban, Jennifer Tarpinian, Stephen R Braddock, Aida Telegrafi, Kristin G Monaghan, Elaine Zackai, Elizabeth J Bhoj
Myelin Regulatory Factor (MYRF) is a transcription factor that has previously been associated with the control of the expression of myelin-related genes. However, it is highly expressed in human tissues and mouse embryonic tissues outside the nervous system such as the stomach, lung, and small intestine. It has not previously been reported as a cause of any Mendelian disease. We report here two males with Scimitar syndrome [MIM 106700], and other features including penoscrotal hypospadias, cryptorchidism, pulmonary hypoplasia, tracheal anomalies, congenital diaphragmatic hernia, cleft spleen, thymic involution, and thyroid fibrosis...
February 15, 2018: American Journal of Medical Genetics. Part A
J V Arcos-Machancoses, C Ruiz Hernández, J Martin de Carpi, S Pinillos Pisón
Congenital diaphragmatic hernia survivors are a well-known group at risk for developing gastroesophageal reflux disease that may be particularly long-term severe. The aim of this study is to provide a systematic review of the prevalence of gastroesophageal reflux in infant and children survivors treated for congenital diaphragmatic hernia.Electronic and manual searches were performed with keywords related to congenital diaphragmatic hernia, gastroesophageal reflux disease, and epidemiology terms. Summary estimates of the prevalence were calculated...
February 9, 2018: Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus
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