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https://www.readbyqxmd.com/read/29666202/nutritional-role-of-amniotic-fluid-clues-from-infants-with-congenital-obstruction-of-the-digestive-tract
#1
Nigel J Hall, Melanie Drewett, David Burge
AIMS: To investigate the role played by amniotic fluid in late fetal nutrition by analysis of infants born with digestive tract atresia. METHODS: Birth weight (BW), gestational age and gender of infants born with oesophageal (OA), duodenal (DA), jejunal (JA) and ileal atresia (IA) were recorded and BW Z-scores compared. Infants with incomplete obstruction (stenosis), chromosomal or syndromic conditions and multiple congenital malformations were excluded. Term infants admitted with suspected postnatal intestinal obstruction in whom no congenital malformation was found were used as a control group...
April 17, 2018: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/29664868/active-transcutaneous-bone-conduction-implant-middle-fossa-placement-technique-in-children-with-bilateral-microtia-and-external-auditory-canal-atresia
#2
Carolina Der, Sofía Bravo-Torres, Nicolás Pons
AIM: The aim of this study is to present the middle fossa technique (MFT) as an alternative for patients who cannot undergo traditional surgery for active transcutaneous bone conduction implants (ATBCI) due to their altered anatomy or desire for future aesthetic reconstruction. DESIGN: A case series descriptive study was designed. The MFT was developed. Preoperative and postoperative information from 24 patients with external auditory canal atresia (EACA) and implanted with ATBCI was reviewed...
April 16, 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29664866/long-term-hearing-outcome-of-canaloplasty-with-partial-ossicular-replacement-in-congenital-aural-atresia
#3
Jungmin Ahn, Gwanghui Ryu, Minwoong Kang, Yang-Sun Cho
OBJECTIVE: The aim of this study was to correlate the postoperative hearing outcomes with regard to the length of prosthesis of the partial ossicular replacement prosthesis (PORP) in patients with congenital aural atresia. STUDY DESIGN: Retrospective review of medical records. SETTING, PATIENTS, INTERVENTION, MAIN OUTCOME MEASURE: The medical records of 131 patients (132 ears) who underwent canaloplasty with PORP by a single surgeon from 2011 to 2016 were reviewed for demographic data, Jahrsdoerfer score, grade of microtia, length of prosthesis, and audiometric outcomes...
April 16, 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29664428/-case-report-and-the-surgical-treatment-of-two-cases-with-pulmonary-atresia-in-which-pulmonary-arteries-is-circulated-by-coronary-arteries
#4
Kahraman Yakut, Kürşad Tokel, Birgül Varan, İlkay Erdoğan, Murat Özkan
Pulmonary atresia (PA) and ventricular septal defect (VSD) can occur in a variety of ways, from simple valve atresia to a condition in which circulation to the pulmonary bed occurs through collateral arteries separated from the aorta and there are no real pulmonary arteries, or they are present but hypoplastic. The size of the pulmonary arteries and concomitant complex cardiac lesions are important in making decisions about treatment and correctional alternatives. While complete correction surgeries in the style of a correction of tetralogy of Fallot are performed in simpler cases, many very invasive procedures are also performed and the resulting quality of life is very variable...
April 2018: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/29664229/a-classic-twin-study-of-lower-urinary-tract-obstruction-report-of-3-cases-and-literature-review
#5
Sandra Frese, Alexander Weigert, Bernd Hoppe, Markus Feldkötter, Michael Ludwig, Stefanie Weber, Katarzyna Kiliś-Pstrusińska, Marcin Zaniew, Heiko Reutter, Alina C Hilger
OBJECTIVE: The aim of the present study was to investigate genetic effects in the formation of congenital lower urinary tract obstruction (LUTO) comprising posterior urethral valves (PUV), urethral atresia, and urethras with variable degrees of stenosis. METHODS: A classic twin study was performed by assessing LUTO twin pairs from the literature. Furthermore, data regarding 3 previously unreported twin pairs with PUV from University of Bonn, Essen and Wrocławs own in-house databases were added...
April 17, 2018: Lower Urinary Tract Symptoms
https://www.readbyqxmd.com/read/29662930/biliary-atresia-clinical-series
#6
Bárbara Neto, Mariana Borges-Dias, Eunice Trindade, José Estevão-Costa, José Miguel Campos
Introduction: Biliary atresia is the main cause of death by hepatic failure and the main indication for liver transplant in children. This study aims to analyze the population with this diagnosis, treated between 2000 and 2015 at Hospital de São João. Material and Methods: Descriptive, observational, and retrospective study, including the patients with biliary atresia, diagnosed and treated between January 1, 2000 and December 31, 2015. We analyzed epidemiologic, clinical, biochemical, and image data, as well as registered complications and present status...
March 2018: GE Portuguese Journal of Gastroenterology
https://www.readbyqxmd.com/read/29662927/biliary-atresia-too-few-too-many-centers
#7
EDITORIAL
Jorge Amil Dias
No abstract text is available yet for this article.
March 2018: GE Portuguese Journal of Gastroenterology
https://www.readbyqxmd.com/read/29662811/management-of-coronary-sinus-ostial-atresia-during-a-staged-operation-of-a-functional-single-ventricle
#8
Seung Ri Kang, Won Kyoun Park, Bo Sang Kwon, Jae Kon Ko, Hyun Woo Goo, Jeong-Jun Park
Coronary sinus ostial atresia (CSOA) with persistent left superior vena cava (LSVC) in the absence of an unroofed coronary sinus is a benign and rare anomaly that may be taken lightly in most instances. However, if overlooked in patients undergoing univentricular heart repair such as bidirectional Glenn or Fontan-type surgery, fatal surgical outcomes may occur due to coronary venous drainage failure. We report a case of CSOA with a persistent LSVC that was managed through coronary sinus rerouting during a total cavopulmonary connection, and provide a review of the literature regarding this rare anomaly...
April 2018: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29656724/large-right-ventricular-sinusoids-in-an-infant-with-aorta-left-ventricular-tunnel-and-proximal-right-coronary-artery-atresia
#9
Peter C Chen, Joseph A Spinner, Jeffrey S Heinle
We report a 1-month-old infant diagnosed with an aorta-left ventricular tunnel, ventricular septal defect, and right coronary atresia with right ventricular sinusoids. The patient's anatomy and physiology did not indicate right-ventricular-dependent coronary circulation, and therefore right ventricular decompression could be performed without compromising coronary perfusion during surgical correction. A detailed understanding of the coronary anatomy is critical in managing this defect when coronary anomalies are present...
April 16, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29649824/end-organ-saturation-differences-in-early-neonatal-transition-for-left-versus-right-sided-congenital-heart-disease
#10
Gabriel Altit, Shazia Bhombal, Theresa A Tacy, Valerie Y Chock
BACKGROUND: For neonates with congenital heart disease (CHD), left-sided (LL) and right-sided (RL) single ventricular physiologies (LL, hypoplastic left heart syndrome; RL, tricuspid atresia or pulmonary atresia with intact ventricular septum) may demonstrate distinct changes in tissue saturation in the first 72 h of life. Near-infrared spectroscopy (NIRS) can measure regional cerebral saturation (Csat) and renal saturation (Rsat) to clarify differences between LL and RL over time. OBJECTIVES: Our primary objective was to measure changes in Csat and Rsat in the first 72 h of life using NIRS between CHD infants with LL compared to RL...
April 12, 2018: Neonatology
https://www.readbyqxmd.com/read/29648693/anomalous-systemic-and-pulmonary-veins-an-unusual-coexistence
#11
Priyadarshini Arunakumar, Anoop Ayyappan, Deepa Sasikumar, K M Krishnamoorthy
A 12-year-old child presented with recurrent respiratory infections and was diagnosed with Scimitar syndrome. Drainage of IVC and course of aberrant arterial supply from aorta were not clear by echocardiogram, and hence, additional imaging was planned. CT unraveled the presence of a rare combination of anomalous pulmonary venous connection (APVC) to IVC, with near-atresia/severe stenosis of IVC, superior to the drainage of scimitar vein. There was a prominent azygos vein with preferential contrast opacification on lower limb injection...
April 12, 2018: Echocardiography
https://www.readbyqxmd.com/read/29643837/insulin-like-growth-factor-binding-proteins-and-igfbp-proteases-a-dynamic-system-regulating-the-ovarian-folliculogenesis
#12
REVIEW
Sabine Mazerbourg, Philippe Monget
The aim of the present article is to update our understanding of the expression of the insulin-like growth factor binding proteins (IGFBPs), IGFBP proteases and their implication in the different processes of ovarian folliculogenesis in mammals. In the studied species, IGFs and several small-molecular weight IGFBPs (in particular IGFBP-2 and IGFBP-4) are considered, respectively, as stimulators and inhibitors of follicular growth and maturation. IGFs play a key role in sensitizing ovarian granulosa cells to FSH action during terminal follicular growth...
2018: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/29643126/case-of-membranous-oesophageal-atresia
#13
Jia-Liang Zhou, Wu-Ping Ge, Xiao-Chun Zhu, Yuan-Long Fang, Tao Song, Song Tian
No abstract text is available yet for this article.
April 11, 2018: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/29628411/impact-of-esophageal-atresia-on-the-success-of-fundoplication-for-gastroesophageal-reflux
#14
Samantha A Pellegrino, Sebastian K King, Elizabeth McLeod, Alisa Hawley, Jo-Anne Brooks, John M Hutson, Warwick J Teague
OBJECTIVES: Fundoplication is commonly performed in patients with a history of esophageal atresia (EA), however, the success of this surgery is reduced, as reflected by an increased rate of redo fundoplication. We aimed to determine whether EA impacts the prevalence of fundoplication, its timing, and performance of a redo operation. STUDY DESIGN: A single-center, retrospective review of all patients undergoing fundoplication over a 20-year period (1994-2013) was performed...
April 5, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29628345/aortic-to-right-ventricular-shunt-for-pulmonary-atresia-with-intact-ventricular-septum-and-bilateral-coronary-ostial-atresia
#15
Hajime Sakurai, Takahisa Sakurai, Naoki Ohashi, Hiroshi Nishikawa
No abstract text is available yet for this article.
March 17, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29628209/outcomes-in-patients-with-short-bowel-syndrome-after-autologous-intestinal-reconstruction-does-etiology-matter
#16
Federica Pederiva, Alberto Sgrò, Riccardo Coletta, Basem Khalil, Antonino Morabito
BACKGROUND: Short bowel syndrome (SBS) is the most common cause of intestinal failure in children. Many factors have been investigated in an attempt to define which parameters influence most survival and ability to wean off parenteral nutrition (PN). The aim of this study was to investigate if aetiology of SBS affects the outcomes in paediatric patients treated with autologous gastrointestinal reconstructive surgery. METHODS: All children with SBS who underwent autologous gastrointestinal reconstructive surgery between 2002 and 2012 were retrospectively reviewed and outcome measures were recorded...
March 12, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29628208/complex-gastroschisis-clinical-spectrum-and-neonatal-outcomes-at-a-referral-center
#17
Pablo Laje, Maria V Fraga, William H Peranteau, Holly L Hedrick, Nahla Khalek, Juliana S Gebb, Julie S Moldenhauer, Mark P Johnson, Alan W Flake, N Scott Adzick
AIM OF THE STUDY: To evaluate the outcomes of neonates with complex gastroschisis (GC), and correlate outcomes with each type of complication. METHODS: Retrospective review of patients with complex GC owing to prenatal and/or postnatal abdominal complications; 2008-2016. Primary outcomes: time to discontinue parenteral nutrition (off-PN), length of stay (LOS) and neonatal survival. MAIN RESULTS: We treated 58 patients with complex gastroschisis owing to abdominal complications, which were: intestinal necrosis at birth (n=9), intestinal atresia (n=16), medical necrotizing enterocolitis (NEC) (n=15), surgical NEC (n=1), in utero volvulus (n=1), vanishing gastroschisis (n=2), severe intestinal dysmotility (n=1), delayed abdominal closure (n=3), abdominal compartment syndrome (n=2) and hiatal hernia/severe gastroesophageal reflux disease (GERD; n=11)...
March 14, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29624817/fatal-hyperammonemia-associated-with-disseminated-serratia-marcescens-infection-in-a-pediatric-liver-transplant-recipient
#18
Stephen Mouat, Jonathan Bishop, Emma Glamuzina, Simon Chin, Emma J Best, Helen M Evans
Hyperammonemia is a rare and important complication post-liver transplantation. We review a case of a 5-month-old boy with biliary atresia who received a split liver transplant following a variceal bleed. The transplant was complicated by recurrent portal vein thrombosis. Colonized with Serratia marcescens pretransplant, he developed disseminated infection associated with very high levels of ammonia that led to his death. It is important to be aware of serum ammonia levels in patients with portal vein thrombosis, particularly in the setting of gastrointestinal bleeding and sepsis...
April 6, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29622552/clival-malformations-in-charge-syndrome
#19
E S Mahdi, M T Whitehead
BACKGROUND AND PURPOSE: CHARGE syndrome is a multisystemic congenital disorder, most commonly including coloboma, heart malformations, choanal atresia, developmental delay, and genital and ear anomalies. The diagnostic criteria for CHARGE syndrome have been refined with time. However, limited reports describe skull base and craniocervical junction abnormalities. Recently, a coronal clival cleft has been identified in association with CHARGE syndrome. The aim of our study was to assess the prevalence of clival pathology in CHARGE syndrome...
April 5, 2018: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29622396/the-distended-fetal-hypopharynx-a-sensitive-and-novel-sign-for-the-prenatal-diagnosis-of-esophageal-atresia
#20
Sarah Tracy, Terry L Buchmiller, Offir Ben-Ishay, Carol E Barnewolt, Susan A Connolly, David Zurakowski, Andrew Phelps, Judy A Estroff
BACKGROUND/PURPOSE: Although advances have been made in the prenatal diagnosis of esophageal atresia (EA), most neonates are not identified until after birth. The distended hypopharynx (DHP) has been suggested as a novel prenatal sign for EA. We assess its diagnostic accuracy and predictive value on ultrasound (US) and magnetic resonance imaging (MRI), both alone and in combination with the esophageal pouch (EP) and secondary signs of EA (polyhydramnios and a small or absent fetal stomach)...
March 7, 2018: Journal of Pediatric Surgery
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