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https://www.readbyqxmd.com/read/28424985/postoperative-complications-and-functional-outcome-after-esophageal-atresia-repair-results-from-longitudinal-single-center-follow-up
#1
Florian Friedmacher, Birgit Kroneis, Andrea Huber-Zeyringer, Peter Schober, Holger Till, Hugo Sauer, Michael E Höllwarth
BACKGROUND: Esophageal atresia (EA) and tracheoesophageal fistula (TEF) represent major therapeutic challenges, frequently associated with serious morbidities following surgical repair. The aim of this longitudinal study was to assess temporal changes in morbidity and mortality of patients with EA/TEF treated in a tertiary-level center, focusing on postoperative complications and their impact on long-term gastroesophageal function. METHODS: One hundred nine consecutive patients with EA/TEF born between 1975 and 2011 were followed for a median of 9...
April 19, 2017: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
https://www.readbyqxmd.com/read/28422842/the-role-of-renoscintigraphy-and-surgery-in-the-management-of-page-kidney-a-case-report
#2
Yu-Li Chiu, Chin Hu, Sin-Di Lee, Po-Yin Chen, Cheng-Pin Wang
RATIONALE: Page kidney is an uncommon condition that hypertension occurs secondary to microvascular ischemia and alternation of small-vessel hemodynamics due to external compression of renal parenchyma and activation of the renin-angiotensin-aldosterone system. There are no specific guidelines for the management of Page kidney in the literatures. PATIENT CONCERNS: A 17-year-old teenager who had Fontan procedure for tricuspid and pulmonary atresia in early childhood suffered from sudden onset of severe left flank pain during cardiac catheterization procedure...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28422365/successful-vaginal-delivery-at-term-after-vaginal-reconstruction-with-labium-minus-flaps-in-a-patient-with-vaginal-atresia-a-rare-case-report
#3
Yu Liu, Yi-Feng Wang
We report a case of successful vaginal delivery after vaginal reconstruction with labium minus flaps in a 23-year-old patient with congenital vaginal atresia. The patient primarily presented with amenorrhea and cyclic abdominal pain; transabdominal ultrasonography revealed an enlarged uterus due to hematometra and absence of the lower segment of the vagina. Eight years ago, she had undergone an unsuccessful attempt at canalization at a local hospital. Upon referral to our hospital, she underwent vaginal reconstruction with labium minus flaps...
April 19, 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28421172/aspiration-risk-and-respiratory-complications-in-patients-with-esophageal-atresia
#4
REVIEW
Thomas Kovesi
Chronic, long-term respiratory morbidity (CRM) is common in patients with a history of repaired congenital esophageal atresia, typically associated with tracheoesophageal fistula (EA/TEF). EA/TEF patients are at high risk of having aspiration, and retrospective studies have associated CRM with both recurrent aspiration and atopy. However, studies evaluating the association between CRM in this population and either aspiration or atopy have reported conflicting results. Furthermore, CRM in this population may be due to other related conditions as well, such as tracheomalacia and/or recurrent infections...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28420535/technique-for-myocardial-protection-in-pulmonary-atresia-intact-ventricular-septum
#5
Ronald K Woods
No abstract text is available yet for this article.
April 2, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28417152/review-of-surgical-and-anesthetic-management-for-esophageal-atresia-with-tracheoesophageal-fistula-unilateral-pulmonary-agenesis-and-dextrocardia
#6
Rebecca M Rentea, Tolulope A Oyetunji, John Erkmann, Joshua Q Knowlton, Richard J Hendrickson
Association of unilateral severe pulmonary atresia or agenesis and esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) and dextrocardia is a rare and highly lethal combination. We report a case of a full-term female infant who had EA with TEF, right lung agenesis, and dextrocardia. Repair of the fistula took place on day of life 3. We describe anesthetic and surgical concerns of this patient's case which are keys to a good operative outcome as well as follow-up for the patient.
April 17, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28416017/patient-complexity-and-genotype-phenotype-correlations-in-biliary-atresia-a-cross-sectional-analysis
#7
Guo Cheng, Patrick Ho-Yu Chung, Edwin Kin-Wai Chan, Man-Ting So, Pak-Chung Sham, Stacey S Cherny, Paul Kwong-Hang Tam, Maria-Mercè Garcia-Barceló
BACKGROUND: Biliary Atresia (BA) is rare and genetically complex, and the pathogenesis is elusive. The disease course is variable and can represent heterogeneity, which hinders effective disease management. Deciphering the BA phenotypic variance is a priority in clinics and can be achieved by the integrative analysis of genotype and phenotype. We aim to explore the BA phenotypic features and to delineate the source of its variance. METHODS: The study is a cross-sectional observational study collating with case/control association analysis...
April 17, 2017: BMC Medical Genomics
https://www.readbyqxmd.com/read/28414679/misses-opportunity-tof-diagnoses-in-4th-decade-of-life
#8
O Baniahmad, T Jarreau, A Johnson
CASE: A 40 year old woman with a history of HIV, congestive heart failure secondary to an unknown congenital heart defect, and hypertension presented to our emergency department with worsening edema. On room air, oxygen saturation was 55 percent . On 5L of oxygen via nasal cannula, oxygen saturation was 88 percent . Physical examination was notable for central cyanosis, facial and lid edema, a II/VI holosystolic murmur across right chest radiating to entire right back hemithorax, decreased breath sounds at bases with pulmonary crackles, clubbing of fingers and edema of bilateral lower extremities...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28414605/use-of-a-novel-surgical-approach-for-treatment-of-complete-bilateral-membranous-choanal-atresia-in-an-alpaca-cria
#9
Karine Pader, Patrick M Burns, Anne-Claire Brisville, Marjolaine Rousseau, Laurent Blond, Geoffrey Truchetti, Hélène Lardé, Catherine Lapointe, David Francoz
CASE DESCRIPTION A 4-hour-old 6.3-kg (13.9-lb) female alpaca cria was evaluated because of severe respiratory distress and difficulty nursing since birth. CLINICAL FINDINGS The cria had open-mouth breathing and cyanotic membranes, with no airflow evident from either nostril. Supplemental oxygen was delivered, and the patient was anesthetized and intubated orotracheally; a CT evaluation of the head confirmed bilateral membranous obstruction of the nasal cavities, consistent with complete bilateral choanal atresia...
May 1, 2017: Journal of the American Veterinary Medical Association
https://www.readbyqxmd.com/read/28413308/congenital-pouch-colon-with-rectal-atresia-revisited
#10
Praveen Mathur, Rahul Gupta, Pradeep Kumar Gupta
We are adding two cases of congenital pouch colon with rectal atresia (RA) to the already published five cases. The presentation of RA is often delayed owing to the presence of normal anus, however, clinical evaluation, plain abdominal radiograph, and urine examination for meconuria, may help in detecting this rare association early.
April 2017: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/28413304/simultaneous-single-staged-repair-of-anorectal-malformation-with-tracheoesophageal-fistula-lessons-learned
#11
Ajay Narayan Gangopadhyay, Vaibhav Pandey
INTRODUCTION: Anorectal malformation (ARM) associated esophageal atresia (EA) with tracheoesophageal fistula (TEF) spawns special therapeutic propositions. The outcome of these patients banks on numerous factors. We performed this study with an aim to compare the outcome of single-staged simultaneous primary repair of both anomalies versus staged repair of these disorders. MATERIALS AND METHODS: Retrospective review of cases with ARM and associated EA with TEF managed over a period of 5 years from July 2010 to June 2015 after ethical approval was undertaken...
April 2017: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/28412031/alterations-in-hyolaryngeal-elevation-after-esophageal-anastomosis-a-possible-mechanism-for-airway-aspiration
#12
Numan Demir, Selen Serel Arslan, Sule Yalcin, Ayşe Karaduman, Feridun Cahit Tanyel, Tutku Soyer
AIM: A prospective study was performed to evaluate anatomical alterations and hyolaryngeal elevation (HE) by videofluoroscopic swallowing study (VFSS) in patients with esophageal atresia-tracheoesophageal fistula (EA-TEF). METHODS: Patients operated for EA-TEF were evaluated for age, sex, type of atresia and time to esophageal anastomosis. All patients were evaluated by videofluoroscopic swallowing study (VFSS). Penetration-Aspiration scale (PAS≥7 is considered as aspiration), distance between upper esophageal sphincter and 2nd cervical vertebrae (UES-C2) and hyolaryngeal elevation (HE) were evaluated by the same deglutitionist who was blind to the study...
April 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28410946/right-atrial-dysfunction-in-the-fetus-with-severely-regurgitant-tricuspid-valve-disease-a-potential-source-of-cardiovascular-compromise
#13
Lisa W Howley, Nee Scze Khoo, Anita J Moon-Grady, Sonali S Patel, Fayeza Alrais, Wayne Tworetzky, Timothy Colen, Paul Brooks, Jean Trines, Tiina Ojala, Lisa K Hornberger
BACKGROUND: In severe right heart obstruction (RHO), redistribution of cardiac output to the left ventricle (LV) is well tolerated by the fetal circulation. Although the same should be true of severely regurgitant tricuspid valve disease (rTVD) with reduced or no output from the right ventricle, affected fetuses more frequently develop hydrops or suffer intrauterine demise. We hypothesized that right atrium (RA) function is altered in rTVD but not in RHO, which could contribute to differences in outcomes...
April 11, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/28410632/evolution-of-left-ventricular-size-in-late-survivors-of-surgery-for-hypoplastic-left-heart-syndrome
#14
Markus Bjurbom, Ajay J Iyengar, Florian Moenkemeyer, Igor E Konstantinov, Christian P Brizard, Yves d'Udekem
BACKGROUND: Aortic atresia and mitral atresia are predictors of adverse events in early life in patients with hypoplastic left heart syndrome, but it is unclear whether late outcomes are also affected, and whether this impact is related to a small hypertrophied left ventricle (LV) that impairs right ventricle (RV) function. METHODS: Thirty patients with hypoplastic left heart syndrome surviving with a Fontan procedure were identified. Follow-up echocardiograms were available at birth, before the Fontan procedure, and several years after...
April 12, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28410264/esophageal-atresia-tracheoesophageal-fistula-repair-complicated-by-tracheomalacia-a-case-report-of-successful-management-of-respiratory-distress-using-caudal-morphine
#15
Joanna M Dion, Anthony M-H Ho, Andrea Winthrop, Michael P Flavin
We report a case of severe respiratory distress in a neonate who was not endotracheally intubated soon after esophageal atresia/tracheoesophageal fistula (EA/TEF) repair. In this serious situation, any form of emergency respiratory support or definitive airway management may compromise the esophageal anastomosis and fistula repair. The cause of respiratory distress in the early postoperative period after EA/TEF is multifactorial, and in this case, included symptomatic tracheomalacia, which is commonly associated with EA/TEF...
April 13, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28409148/position-paper-of-inoea-working-group-on-long-gap-esophageal-atresia-for-better-care
#16
David C van der Zee, Pietro Bagolan, Christophe Faure, Frederic Gottrand, Russell Jennings, Jean-Martin Laberge, Marcela Hernan Martinez Ferro, Benoît Parmentier, Rony Sfeir, Warwick Teague
INoEA is the International Network of Esophageal Atresia and consists of a broad spectrum of pediatric specialties and patient societies. The working group on long-gap esophageal atresia (LGEA) set out to develop guidelines regarding the definition of LGEA, the best diagnostic and treatment strategies, and highlight the necessity of experience and communication in the management of these challenging patients. Review of the literature and expert discussion concluded that LGEA should be defined as any esophageal atresia (EA) that has no intra-abdominal air, realizing that this defines EA with no distal tracheoesophageal fistula (TEF)...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28408057/congenital-bi-segmental-bronchial-atresia-with-areas-of-congenital-pulmonary-airway-malformation
#17
Zuhal Bayramoglu, Emine Caliskan, Emine Dilek Yilmazbayhan
No abstract text is available yet for this article.
April 10, 2017: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/28407396/a-further-family-of-stromme-syndrome-carrying-cenpf-mutation
#18
Ferda Ozkinay, Tahir Atik, Esra Isik, Zeliha Gormez, Mahmut Sagiroglu, Ozlem Atan Sahin, Nergul Corduk, Huseyin Onay
Stromme syndrome is a rare genetic disorder characterized by microcephaly, anterior ocular chamber anomalies, and "apple peel" type jejunal atresia. Here, we report a Stromme syndrome family with two affected siblings with a homozygous truncating frameshift mutation in CENPF. A 3-month-old girl was hospitalized due to prenatally diagnosed microcephaly, microphthalmia, and dysmorphological features. The history of a previous child with the same findings in addition to "apple peel" intestinal atresia had been noted...
April 13, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28407283/acoustic-radiation-force-impulse-imaging-for-assessing-liver-fibrosis-preoperatively-in-infants-with-biliary-atresia-comparison-with-liver-fibrosis-biopsy-pathology
#19
Fen Gao, Ya-Qing Chen, Jing Fang, Sheng-Li Gu, Luan Li, Xiao-Ying Wang
OBJECTIVES: The purpose of this study was to investigate the diagnostic performance of acoustic radiation force impulse (ARFI) in assessing liver fibrosis preoperatively in infants with biliary atresia (BA). METHODS: A total of 50 consecutive infants with BA and 50 healthy infants who underwent ARFI examination were recruited. Siemens Acuson S2000 in Virtual Touch Quantification mode (Siemens Medical Solutions, Mountain View, CA) was used to measure shear wave speeds (SWSs)...
April 13, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28403505/the-role-of-endoscopic-retrograde-cholangiopancreatography-in-the-diagnosis-of-biliary-atresia-14-years-experience
#20
Ahmed A Negm, Claus Petersen, Andrea Markowski, Birgit Luettig, Kristina I Ringe, Tim O Lankisch, Michael P Manns, Benno Ure, Andrea S Schneider
No abstract text is available yet for this article.
April 12, 2017: European Journal of Pediatric Surgery
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