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https://www.readbyqxmd.com/read/29053746/external-auditory-exostoses-and-hearing-loss-in-the-shanidar-1-neandertal
#1
Erik Trinkaus, Sébastien Villotte
The Late Pleistocene Shanidar 1 older adult male Neandertal is known for the crushing fracture of his left orbit with a probable reduction in vision, the loss of his right forearm and hand, and evidence of an abnormal gait, as well as probable diffuse idiopathic skeletal hyperostosis. He also exhibits advanced external auditory exostoses in his left auditory meatus and larger ones with complete bridging across the porus in the right meatus (both Grade 3). These growths indicate at least unilateral conductive hearing (CHL) loss, a serious sensory deprivation for a Pleistocene hunter-gatherer...
2017: PloS One
https://www.readbyqxmd.com/read/29050815/right-ventricular-outflow-tract-reintervention-after-primary-tetralogy-of-fallot-repair-in-neonates-and-young-infants
#2
Shyamasundar Balasubramanya, David Zurakowski, Michele Borisuk, Aditya K Kaza, Sitaram M Emani, Pedro J Del Nido, Christopher W Baird
OBJECTIVE: To assess the outcomes following primary tetralogy of Fallot (TOF) repair in neonates and young infants with pulmonary stenosis (PS) and pulmonary atresia and compare differences in reintervention on the right ventricular outflow tract (RVOT) among those undergoing valve sparing repair (VSR), transannular RVOT patch (TAP), and right ventricle-to-pulmonary artery (RV-PA) conduit surgeries. METHODS: Data were collected retrospectively in 101 patients who underwent TOF repair over a 10-year period between January 2005 and September 2015...
September 19, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29049818/dor-procedure-for-pulmonary-atresia-with-intact-ventricular-septum-in-an-infant
#3
Yoonjin Kang, Jae Gun Kwak, Eung Rae Kim, Woong-Han Kim
Pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation confers a high risk for myocardial infarction and complications of ischaemia, including left ventricle aneurysms. We describe an infant with pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation and a left ventricle apical aneurysm who successfully underwent the Dor procedure and a subsequent Fontan operation. Surgery for postinfarction left ventricle aneurysm can be considered in infants...
September 2, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29049701/effects-of-blood-flow-dynamics-on-autologous-pericardial-degeneration-in-reconstructed-pulmonary-arteries
#4
Nobuyasu Kato, Masaaki Yamagishi, Keiichi Itatani, Takako Miyazaki, Yoshinobu Maeda, Satoshi Asada, Yoshiro Matsui, Hitoshi Yaku
OBJECTIVES: To clarify the risk factors for abnormal degeneration of autologous pericardium, the mechanical stress on the endothelial tissue caused by turbulent blood flow in the pulmonary artery (PA) reconstructed with autologous pericardium and major aortopulmonary collateral arteries was assumed in pulmonary atresia and ventricular septal defect. METHODS: Patient-specific PA models were created for 6 patients based on their past computed tomography images taken after unifocalization of major aortopulmonary collateral arteries...
October 12, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29049668/one-stage-definitive-repair-of-complete-atrioventricular-septal-defect-and-pulmonary-atresia-with-major-aortopulmonary-collateral-arteries
#5
Yujiro Ide, Masaya Murata, Hiroki Ito, Kisaburo Sakamoto
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is considered the most severe end of the tetralogy of Fallot spectrum, whereas complete atrioventricular septal defect associated with tetralogy of Fallot is a rare congenital defect. However, the combination of pulmonary atresia, major aortopulmonary collateral artery and complete atrioventricular septal defect is extremely rare, and surgical repair has not been reported. We describe a successful, 1-stage correction in a 7-month-old boy with this condition, who had an additional complication of a single aortic outlet from the right ventricle and significant atrioventricular valve regurgitation...
October 11, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29049228/the-efficiency-of-sonography-in-diagnosing-volvulus-in-neonates-with-suspected-intestinal-malrotation
#6
Wenhua Zhang, Hongjun Sun, Fangqiong Luo
This study is to prospectively evaluate the efficiency of sonography for volvulus diagnosis in neonates with clinically suspected intestinal malrotation.A total of 83 patients with suspected intestinal malrotation who underwent detailed abdominal sonography and upper gastrointestinal contrast study were included. Malrotation was characterized by inversion of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV) in sonographic examination. The "whirlpool sign" of Color Doppler Sonography was recognized as a characteristic for malrotation with volvulus...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29048729/beyond-down-syndrome-phenotype-paternally-derived-isodicentric-chromosome-21-with-partial-monosomy-21q22-3
#7
Manesha Putra, Urvashi Surti, Jie Hu, Deana Steele, Michele Clemens, Devereux N Saller, Svetlana A Yatsenko, Aleksandar Rajkovic
Inverted isodicentric chromosome 21 is a rare form of chromosomal rearrangement that may result in trisomy 21; sometimes this rearrangement may also lead to segmental monosomy of the terminal long arm of chromosome 21. In this report, we describe the prenatal diagnosis and neonatal follow-up of a child with a paternally derived, de novo isodicentric chromosome 21 and a concurrent ∼1.2 Mb deletion of the 21q22.3 region [46,XX,idic(21)(q22.3)]. This child presented with unusual phenotype of Down syndrome and additional defects including esophageal atresia and tethered cord syndrome...
October 19, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29047321/perioperative-and-anesthetic-considerations-in-pulmonary-atresia-with-intact-ventricular-septum
#8
Stephen Gleich, Gregory J Latham, Denise Joffe, Faith J Ross
Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare right-heart obstructive lesion with a wide anatomic and physiologic spectrum of disease, ranging from simple membranous pulmonary valve atresia with a fully developed right ventricle (RV) to a severely hypoplastic RV and ventriculocoronary (RV-coronary) fistulas. Affected neonates are dependent on prostaglandin for adequate pulmonary blood flow. Depending on the severity of disease, treatment options range from transcatheter pulmonary valve perforation and ultimate biventricular repair to staged single-ventricle palliation...
October 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29046629/cerebellar-vermis-and-midbrain-hypoplasia-upon-conditional-deletion-of-chd7-from-the-embryonic-mid-hindbrain-region
#9
Alex P A Donovan, Tian Yu, Jacob Ellegood, Kimberley L H Riegman, Christa de Geus, Conny van Ravenswaaij-Arts, Cathy Fernandes, Jason P Lerch, M Albert Basson
Reduced fibroblast growth factor (FGF) signaling from the mid-hindbrain or isthmus organizer (IsO) during early embryonic development results in hypoplasia of the midbrain and cerebellar vermis. We previously reported evidence for reduced Fgf8 expression and FGF signaling in the mid-hindbrain region of embryos heterozygous for Chd7, the gene mutated in CHARGE (Coloboma, Heart defects, choanal Atresia, Retarded growth and development, Genitourinary anomalies and Ear defects) syndrome. However, Chd7(+/-) animals only exhibit mild cerebellar vermis anomalies...
2017: Frontiers in Neuroanatomy
https://www.readbyqxmd.com/read/29046057/bile-duct-ligature-in-young-rats-a-revisited-animal-model-for-biliary-atresia
#10
Matias Garrido, Camila Escobar, Constanza Zamora, Carolina Rejas, Juan Varas, Mario Párraga, Sebastian San Martin, Sandra Montedónico
Biliary atresia leads to cirrhosis in the vast majority of patients and constitutes the first cause of paediatric liver transplantation. Animal models allow us to understand the molecular basis and natural history of diseases. The aim of this study is to describe a surgically created animal model of biliary atresia with emphasis in long-term liver function. Forty-two 3-week-old Sprague-Dawley rats were randomly divided into two groups: bile duct ligature (BDL) and control. The animals were sacrificed on the 2nd, 4th, and 6th postoperative weeks...
September 13, 2017: European Journal of Histochemistry: EJH
https://www.readbyqxmd.com/read/29045944/prevalence-estimates-of-rare-congenital-anomalies-by-integrating-two-population-based-registries-in-tuscany-italy
#11
Alessio Coi, Michele Santoro, Anna Pierini, Sonia Marrucci, Federica Pieroni, Fabrizio Bianchi
BACKGROUND/AIMS: Population-based registries play a key role in the epidemiological surveillance of congenital anomalies (CAs). This study is aimed at improving the epidemiological surveillance and providing prevalence estimates of rare CAs using the Registry of Rare Diseases as an added data source to the Registry of Congenital Anomalies. METHODS: Cases of diagnosed rare CAs (2006-2013) were extracted from the Tuscany Registry of Rare Diseases and the Tuscany Registry of Congenital Anomalies in order to set up an integrated dataset...
September 19, 2017: Public Health Genomics
https://www.readbyqxmd.com/read/29043689/-mesenteric-edema-as-a-prenatal-ultrasound-sign-of-poor-prognosis-in-gastroschisis
#12
M Dore Reyes, P Triana Junco, J L Encinas Hernández, E Alvarado Antolín, J L Bartha Rasero, V Núñez Cerezo, M Romo Muñoz, M Gómez Cervantes, A Sánchez Galán, L Martínez Martínez, M López Santamaría
INTRODUCTION/AIM OF THE STUDY: Gastroschisis is a congenital malformation with an easy and early prenatal diagnosis, however, it has a variable post-natal outcome. Our aim was to determine if certain ultrasound markers or early delivery were related with a worse postnatal outcome. PATIENTS AND METHODS: Retrospective study of a cohort of patients with gastroschisis diagnosed between 2005-2014, with emphasis on prenatal ultrasounds, gestational age at delivery and post-natal outcome...
July 20, 2017: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/29039049/japanese-biliary-atresia-registry
#13
Masaki Nio
The Japanese Biliary Atresia Registry (JBAR) was launched in 1989 by the Japanese Biliary Atresia Society (JBAS). JBAR employed an initial questionnaire, a questionnaire for liver transplantation, and a follow-up questionnaire. The questionnaire has been sent to the pediatric surgeons of JBAS member institutions and the hospitals in which council members of the Japanese Society of Pediatric Surgeons are working. One hundred and twenty-three hospitals have been included in JBAR between 1989 and 2015. Each patient is to be followed up for 30 years...
October 16, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29039048/sclerosing-and-obstructive-cholangiopathy-in-biliary-atresia-mechanisms-and-association-with-biliary-innate-immunity
#14
REVIEW
Kenichi Harada
Biliary atresia (BA) is histologically characterized by a progressive, sclerosing cholangitis and the obstruction of extrahepatic bile ducts. In terms of the etiology and pathogenesis of BA, several viral infections consisting of dsRNA, including Reoviridae, have been implicated. Human biliary epithelial cells (BECs) possess an innate immune system consisting of Toll-like receptors (TLRs). BECs have negative regulatory mechanisms of TLR tolerance to avoid an excessive inflammatory response to lipopolysaccharide (LPS), a TLR4 ligand; however, they lack the tolerance to poly(I:C) (a synthetic analog of viral dsRNA), a TLR3 ligand...
October 16, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29036008/ethnic-disparity-in-the-incidence-and-outcome-of-biliary-atresia-in-new-zealand
#15
Helen M Evans, M Innes Asher, Sophia Cameron-Christie, Sonja Farthing, John McCall, Stephen P Robertson, Hayley Wong, Philip N Morreau
In order to determine incidence and outcome of biliary atresia (BA) between ethnic groups in New Zealand (NZ), a retrospective review was undertaken of children with BA born 2002-2014. Prioritised ethnicity was used to determine ethnicity and was compared to population data. Uni- and multivariate analyses were undertaken to determine demographic and biochemical factors associated with outcome. Overall incidence was 1 in 9,181 (Māori 1 in 5,285; European 1 in 16,228; p < 0.0001). Overall and transplant-free survival rates at 1, 2 and 5 years were 92%, 86%, 82% and 70%, 49%, 30% respectively with Māori having improved transplant-free survival (p < 0...
October 13, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29034890/derivation-of-a-disease-specific-human-induced-pluripotent-stem-cell-line-from-a-biliary-atresia-patient
#16
Lipeng Tian, Lindsey Eldridge, Pooja Chaudhari, Linyi Zhang, Robert A Anders, Kathleen B Schwarz, Zhaohui Ye, Yoon-Young Jang
Biliary atresia (BA) is a common cause of pediatric end-stage liver disease. While its etiology is not yet clear, evidence has suggested that BA results from interactions between genetic susceptibility and environmental factors. Disease relevant human cellular models of BA will facilitate identification of both genetic and environmental factors that are important for disease prevention and treatment. Here we report the generation of a human induced pluripotent stem cell line from a BA patient using episomal vectors...
October 2017: Stem Cell Research
https://www.readbyqxmd.com/read/29034656/guidelines-of-the-italian-society-of-videosurgery-in-infancy-for-the-minimally-invasive-treatment-of-the-esophageal-atresia
#17
Salvatore Fabio Chiarenza, Maria Luisa Conighi, Andrea Conforti, Ciro Esposito, Maria Escolino, Fabio Beretta, Maurizio Cheli, Vincenzo Di Benedetto, Maria Grazia Scuderi, Giovanni Casadio, Maurizio Marzaro, Leon Francesco Fascetti, Claudio Vella, Cosimo Bleve, Daniela Codric, Paolo Caione, Pietro Bagolan
Not available.
October 4, 2017: La Pediatria Medica e Chirurgica: Medical and Surgical Pediatrics
https://www.readbyqxmd.com/read/29034612/variability-index-of-tacrolimus-serum-levels-in-pediatric-liver-transplant-recipients-younger-than-12%C3%A2-years-non-adherence-or-risk-of-non-adherence
#18
Janete Teresinha Pires de Oliveira, Carlos Oscar Kieling, Anaís Back da Silva, Joel Stefani, Maria Carolina Witkowski, Camila Ribas Smidt, Carolina R Mariano da Rocha, Vania Naomi Hirakata, Maria da Graça Grossini, Maria Lúcia Zanotelli, Sandra Maria Gonçalves Vieira
MLVI has been used to assess adherence. To determine the MLVI in children <12 years of age at transplantation and to identify demographic correlates and consequences for the graft. This is a retrospective study of 50 outpatients (4.0 ± 3.5 years), at least 13-month post-liver transplantation. The outcomes evaluated were MLVI, ALT > 60 IU/L, ACR, death, and graft loss. We analyzed demographic and socioeconomic characteristics, indication for transplantation, and type of donor. Student's t test and the chi-square test were used...
October 15, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29033814/a-case-of-aplasia-cutis-congenita-type-vi-bart-syndrome
#19
Yasmin Alfayez, Sahar Alsharif, Adel Santli
Aplasia cutis congenita type VI, also known as Bart syndrome, is a rare genetic mechanobullous disorder characterized by congenital localized absence of skin, mucocutaneous blistering lesions, and nail abnormalities. We present the case of a 4-h-old male newborn who presented with complete absence of skin over the anteromedial aspect of both lower legs associated with nail dystrophy since birth. After a few days, he developed blisters that were consistent with epidermolysis bullosa in histopathological examination...
May 2017: Case Reports in Dermatology
https://www.readbyqxmd.com/read/29030699/prematurity-and-biliary-atresia-a-30-year-observational-study
#20
Natalie Durkin, Maesha Deheragoda, Mark Davenport
AIM OF STUDY: The diagnosis of biliary atresia (BA) remains challenging and delay can lead to significant morbidity with time to surgery a key factor in determining outcome. Prematurity may impact on outcome potentially delaying diagnosis. We sought to assess whether the premature BA infants (PBA) have a delayed time to surgery and as such, worse outcomes? METHODS: Review of a single-centre prospectively maintained database. Prematurity was defined as delivery < 37/40 gestation...
October 13, 2017: Pediatric Surgery International
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