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Esophagic atresia

Felipe Donoso, Helene Engstrand Lilja
Background Since 2005, infants with esophageal atresia (EA) in our unit are given prophylactic proton pump inhibitors (PPI) after repair until 1 year of age. The aims of this study were to identify risk factors for anastomotic strictures (AS) and to assess the efficacy of postoperative PPI prophylaxis in reducing the incidence of AS compared with symptomatic PPI. Methods Patients who underwent EA repair from 1994 to 2013 in our unit were included in this retrospective observational study approved by the local ethics review board...
October 21, 2016: European Journal of Pediatric Surgery
Sigrid Bairdain, David Zurakowski, Sara O Vargas, Nicole Stenquist, Molly McDonald, Meghan C Towne, David T Miller, Russell W Jennings, David B Kantor, Pankaj B Agrawal
BACKGROUND: Long-gap esophageal atresia (LGEA) may have clinical and syndromic presentations different from those of esophageal atresia (EA) that affects shorter segments of the esophagus (non-LGEA). This may suggest unique underlying developmental mechanisms. OBJECTIVES: We sought to characterize clinical differences between LGEA and non-LGEA by carefully phenotyping a cohort of EA patients, and furthermore to assess molecular genetic findings in a subset of them...
October 19, 2016: Neonatology
Jessica Menzies, Jennifer Hughes, Steven Leach, Yvonne Belessis, Usha Krishnan
OBJECTIVES: Growth and feeding problems have been described in children with Esophageal Atresia (EA). Ongoing gastrointestinal and respiratory complications such as Gastroesophageal Reflux Disease (GERD), esophageal dysmotility, strictures and respiratory infections may contribute. The aim of the study was to document the prevalence of malnutrition and feeding difficulties and examine predictive factors which may influence feeding and growth in children attending a multidisciplinary EA clinic in Sydney Australia...
October 13, 2016: Journal of Pediatric Gastroenterology and Nutrition
Seyed Abdollah Mousavi, Hasan Karami, Hossein Saneian
INTRODUCTION: The duodenum is the most common site for congenital intestinal obstruction. The duodenal web with a central hole can present without any overt signs of obstruction at a later age. MATERIAL AND METHODS: Over a 7-year period, children with congenital intestinal obstruction were identified in this study. The complications and operative findings of patients with duodenal web with conditions such as wind sock deformity and delayed diagnosis were evaluated in this study...
October 1, 2016: Archives of Medical Science: AMS
Hiroki Nakamura, Takafumi Kawano, Katsumi Yoshizawa, Hideaki Nakajima, Kazuto Suda, Hiroyuki Koga, Kazuhiko Nakame, Satoshi Ieiri, Shigeru Takamizawa, Naoto Urushihara, Toshihiro Yanai, Atsuyuki Yamataka
PURPOSE: We present a first report of the long-term follow-up of biliary atresia (BA) patients who became anicteric with the native liver (ANL; total bilirubin <1.5mg/dL) after redo-Kasai. METHODS: Forty-six redo-Kasai cases (1984-2015) were the subjects for this study. ANL ratios were determined using the Kaplan-Meier estimate. RESULTS: BA type was I (n=3), II (n=1), and III (n=42). Mean ages (initial/redo) at Kasai were 60.3/231.9days, respectively...
September 16, 2016: Journal of Pediatric Surgery
Fernando Alberca de Las Parras, María Navalón Rubio, Juan Egea Valenzuela
INTRODUCTION: Refractory esophageal stenosis (RES) is a major health problem in the pediatric population. Several techniques such as stent placement or C-mitomycin (CM) have been described as alternative treatments. We present our experience with both techniques, in our case with biodegradable stents (BS) and sometimes the association with stents and CM. MATERIAL AND METHODS: Six patients have been included: 2 post-operative fistulas in patients with type I esophageal atresia; 1 operated atresia without fistula; and 3 caustic strictures...
October 2016: Revista Española de Enfermedades Digestivas
Antonio Perez-Aytes, Purificacion Marin-Reina, Virginia Boso, Ana Ledo, John C Carey, Maximo Vento
Mycophenolate mofetil (MMF) is probably the most common employed immunosuppressant drug in recipients of solid organ transplant and in many autoimmune diseases. In vitro studies, a significant number of single clinical observations and a recent study from a group of different European teratogen information services, have provided very consistent data supporting the existence of a specific MMF embryopathy. The typical malformative pattern of MMF embryopathy includes external ear anomalies ranging from hypoplastic pinna (microtia) to complete absence of pinna (anotia); cleft lip, with or without cleft palate, and ocular anomalies as iris or chorioretinal coloboma and anophthalmia/microphthalmia...
September 14, 2016: European Journal of Medical Genetics
Sushma Malik, Mani Singhal, Shruti Sudhir Jadhav, Charusheela Sujit Korday, Chitra Shivanand Nayak
BACKGROUND: Hirschsprung's disease is one of the commonest causes of intestinal obstruction in neonates because of gut motility disorder. It is characterized as a complex genetic heterogenous disorder with variable inheritance. Hirschsprung's disease occurs as an isolated phenotype in majority (70 %) of cases. In other cases it may be associated with syndromes (such as Down's syndrome, Waardenburg syndrome, congenital central hypoventilation, or cartilage-hair hypoplasia) or with a spectrum of congenital anomalies involving neurological, cardiovascular, or urological systems or with sensorineural anomalies...
2016: Journal of Medical Case Reports
Yung Fong Tsai, Hsiu Pin Chen, Fu Chao Liu, Shih Hao Liu, Chun Yu Chen, Chih Wen Cheng, Jr Rung Lin
Post-transplant malignancy is a major cause of late mortality for liver transplant recipients (LTRs). This nationwide population-based cohort study investigated the cancer type, incidence, and risk factors associated with post-transplant malignancies in 2938 Taiwanese LTRs who underwent transplantation between 1998 and 2012. Data from the National Health Insurance Research Database were extracted on the basis of the International Classification of Disease, Ninth Revision, Clinical Modification codes. Among these patients, 284 post-transplant malignancies were diagnosed...
September 10, 2016: Oncotarget
C Jason Smithers, Thomas E Hamilton, Michael A Manfredi, Lawrence Rhein, Peter Ngo, Dorothy Gallagher, John E Foker, Russell W Jennings
PURPOSE: Recurrent trachea-esophageal fistula (recTEF) is a frequent (5%-10%) complication of congenital TEF (conTEF) and esophageal atresia (EA) repair. In addition, postoperative acquired TEF (acqTEF) can occur in addition to or even in the absence of prior conTEF in the setting of esophageal anastomotic complications. Reliable repair often proves difficult by endoluminal or standard surgical techniques. We present the results of an approach that reliably identifies the TEF and facilitates airway closure as well as repair of associated tracheal and esophageal problems...
August 31, 2016: Journal of Pediatric Surgery
Tiffany Busa, Nicoleta Panait, Kathia Chaumoitre, Nicole Philip, Chantal Missirian
Terminal 7q deletion is rarely reported in the literature. Holoprosencephaly and sacral dysgenesis are found in association with this deletion, due to haploinsufficiency of SHH and HLBX9 genes respectively. We report on a 2-year-old boy with 7q35-36.3 deletion encompassing SHH identified by oligonucleotide array comparative genomic hybridization. In addition to other frequent features, the patient presented with esophageal atresia and tracheoeosophageal fistula diagnosed at birth. This case, together with two others previously described, one presenting with esophageal atresia, the other with congenital esophageal stenosis, confirms the possible association between congenital esophageal malformations and 7q terminal deletion including SHH...
October 2016: European Journal of Medical Genetics
Waleed Gibreel, Benjamin Zendejas, Ryan M Antiel, Geoffrey Fasen, Christopher R Moir, Abdalla E Zarroug
OBJECTIVES: The aim of the study was to evaluate and study the full spectrum of swallowing dysfunction and long-term disease-specific outcomes in adults with surgically corrected esophageal atresia/tracheaesophageal fistula (EA/TEF). BACKGROUND: Long-term outcomes for adults who underwent EA/TEF repair because infants are lacking. METHODS: We developed a disease-specific swallowing dysfunction questionnaire (SDQ) to assess swallowing dysfunction and quality of life (QOL) of adult patients with surgically corrected EA/TEF...
September 6, 2016: Annals of Surgery
Nataliya S Demikova, Yulia V Vydrych, Marina A Podolnaya, Aleksandra S Lapina, Aliy Yu Asanov
BACKGROUND: This study examined the prevalence of esophageal atresia (EA) and the relationship between EA and demographic factors in the Russian Federation. METHODS: Data were obtained from a population-based congenital malformations registry across 14 years (2000-2013) in 24 regions of the Russian Federation and included cases of EA among live births and stillbirths. RESULTS: The total number of births was 6,478,706. There were 1317 cases of isolated EA, resulting in a rate of 2...
October 2016: Birth Defects Research. Part A, Clinical and Molecular Teratology
Usha Krishnan, Hayat Mousa, Luigi Dall'Oglio, Nusrat Homaira, Rachel Rosen, Christophe Faure, Frédéric Gottrand
BACKGROUND: Esophageal atresia (EA) is one of the most common congenital digestive anomalies. With improvements in surgical techniques and intensive care treatments, the focus of care of these patients has shifted from mortality to morbidity and quality-of-life issues. These children face gastrointestinal (GI) problems not only in early childhood but also through adolescence and adulthood. There is, however, currently a lack of a systematic approach to the care of these patients. The GI working group of International Network on Esophageal Atresia comprises members from ESPGHAN/NASPGHAN and was charged with the task of developing uniform evidence-based guidelines for the management of GI complications in children with EA...
November 2016: Journal of Pediatric Gastroenterology and Nutrition
Mark Safe, Jemma Cho, Usha Krishnan
OBJECTIVE: The aim of the study was to evaluate and compare multichannel intraluminal impedance-pH (MII-pH) monitoring with standard investigations including pH testing for detecting gastroesophageal reflux disease (GERD) in children. METHODS: A retrospective review of all MII-pH studies performed between July 2007 and March 2013 at Sydney Children's Hospital. Results from MII-pH testing, esophagogastroduodenoscopy (EGD), barium meal and pepsin assay, symptoms, underlying comorbidities, age, and medication usage were evaluated...
November 2016: Journal of Pediatric Gastroenterology and Nutrition
Maissa Rayyan, Nathalie Rommel, Jan Tack, Jan Deprest, Karel Allegaert
Esophageal atresia (EA) is a congenital malformation defined by the discontinuity of the esophagus occurring in 2.4 in 10,000 births. As survival rates are high, the significant medical morbidity became more relevant. Short-term and long-term morbidities involve the respiratory and gastrointestinal system in the majority of the patients. The impact of this morbidity seems large enough to inspire researchers to develop experimental animal models that may help understanding the pathogenesis and pathophysiology...
August 17, 2016: European Journal of Pediatric Surgery
Maximiliano Alejo Maricic, María Marcela Bailez, Susana P Rodriguez
UNLABELLED: We present the results of the validation of an inanimate model created for training thoracoscopic treatment of esophageal atresia with lower tracheoesophageal fistula (EA/TEF). MATERIALS AND METHODS: We used different domestic materials such as a piece of wood (support), corrugated plastic tubes (PVC) of different sizes to simulate ribs, intercostal spaces, trachea and spine and tubular latex balloons to simulate the esophagus and lungs to make the basic model...
September 2016: Journal of Pediatric Surgery
Jens Dingemann, Rüdiger Szczepanski, Gundula Ernst, Ute Thyen, Benno Ure, Melanie Goll, Ingo Menrath
Aim of the Study A history of esophageal atresia (EA) may result in chronic morbidity. The transition of patients from pediatric to adult care has been recognized as an important factor to maintain disease-specific follow-up and prevent exacerbation of chronic disease. Patient education is recognized as a necessary part of transition programs for children with chronic diseases. Structured education programs for patients with EA have not yet been developed. We aimed to evaluate the efficacy of a transition-specific educational program in adolescents with a history of EA...
August 14, 2016: European Journal of Pediatric Surgery
Júlio Rocha Pimenta, Alexandre Rodrigues Ferreira, Eleonora Druve Tavares Fagundes, Thaís Costa Nascentes Queiroz, Regiane Aparecida Nascimento Baptista, Eduardo Guimarães de Araújo Moreira, Camilo Brandão de Resende, Paulo Fernando Souto Bittencourt, Simone Diniz Carvalho, José Andrade Franco Neto, Francisco José Penna
OBJECTIVE: Bleeding of esophageal varices is the main cause of morbidity and mortality in children with portal hypertension. It is important to understand the factors related with a bleeding episode to evaluate more effective primary prophylaxis. This study aims to describe the endoscopic and laboratory findings associated with upper gastrointestinal bleeding (UGIB) secondary to esophageal varices. METHOD: A cross-sectional study with 103 children and adolescents with cirrhosis, divided into a group that had experienced an episode of upper UGIB (35 patients) and a group without a history of UGIB (68 patients), was carried out...
August 4, 2016: Journal of Pediatric Gastroenterology and Nutrition
Yu Feng, Runsen Chen, Xiaonan Li, Xuming Mo
BACKGROUND: Esophageal atresia (EA) is a common birth defect that occurs with tracheoesophageal fistula (TEF), although etiological studies on EA/TEF have produced inconsistent results. METHODS: The aim of this study was to examine the association between environmental factors during pregnancy and the risk of EA/TEF in a Chinese population. Cases of isolated EA and nonisolated EA and unaffected controls were identified between July 2005 and November 2015, and face-to-face questionnaires concerning exposure to environmental factors were administered to the birth mothers of 130 cases and 400 controls...
October 2016: Birth Defects Research. Part A, Clinical and Molecular Teratology
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