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Sample records for long-gap esophageal atresia

  1. National Esophageal Atresia Register.

    PubMed

    Sfeir, Rony; Michaud, Laurent; Sharma, Duyti; Richard, Florence; Gottrand, Frédéric

    2015-12-01

    National Esophageal Atresia was created in 2008 by the National Reference Center for Esophageal Congenital Abnormalities created in 2006. Primary goal was estimation of live birth prevalence in France. A national network of surgeons and pediatricians was initiated and entire teams dealing with esophageal atresia accepted to participate in an exhaustive national register. A questionnaire was validated by a national committee and data were centralized in our center. Scientific exploitation showed that such database is useful for health authorities as for medical professionals. Live birth prevalence in France is at 1.9/10,000 births. Prenatal diagnosis is more common but its effect on prevalence is not yet fully understood. Associated congenital abnormalities are frequent and major malformations with termination of pregnancy can influence prevalence. PMID:26642387

  2. Short-gap Isolated Esophageal Atresia Causing Stridor Due to Compression of the Trachea.

    PubMed

    Sekmenli, Tamer; Ciftci, İlhan; Sivri, Mesut; Koplay, Mustafa

    2015-12-01

    Isolated esophageal atresias are reported always to be associated with long gap in the literature. In this manuscript, we aimed to discuss the imaging and surgical treatment methods of an isolated esophageal atresia case with 'short gap' who had stridor due to compression of the trachea by dilated upper esophageal pouch and had not identified previously in the literature. PMID:26843741

  3. Short-gap Isolated Esophageal Atresia Causing Stridor Due to Compression of the Trachea

    PubMed Central

    Sekmenli, Tamer; Ciftci, İlhan; Sivri, Mesut; Koplay, Mustafa

    2015-01-01

    Isolated esophageal atresias are reported always to be associated with long gap in the literature. In this manuscript, we aimed to discuss the imaging and surgical treatment methods of an isolated esophageal atresia case with ‘short gap’ who had stridor due to compression of the trachea by dilated upper esophageal pouch and had not identified previously in the literature. PMID:26843741

  4. Difficult esophageal atresia: trick and treat.

    PubMed

    Conforti, Andrea; Morini, Francesco; Bagolan, Pietro

    2014-10-01

    Although most patients with esophageal atresia (EA) and tracheo-esophageal fistula (TEF) may benefit from "standard" management, which is deferred emergency surgery, some may present unexpected elements that change this paradigm. Birth weight, associated anomalies, and long gap can influence the therapeutic schedule of the patients with EA/TEF and can make their treatment tricky. As a consequence, detailed information on these aspects gives the power to develop a decision-making process as correct as possible. In this article, we will review the most important factors influencing the treatment of patients with EA/TEF and will share our experience on the diagnostic and therapeutic tips that may provide pivotal help in the management of such patients.

  5. A new approach for the management of esophageal atresia without tracheo-esophageal fistula.

    PubMed

    Bedi, Nandini K; Grewal, Alka G; Rathore, Shubhra; George, Uttam

    2016-01-01

    Long gap esophageal atresia (OA) is a challenging condition. While discussing the various methods of management available to us, we report the use of magnetic resonance imaging in a case of pure OA to judge the gap between two ends of the esophagus. PMID:27365910

  6. Surgical advances in the fetus and neonate: esophageal atresia.

    PubMed

    Kunisaki, Shaun M; Foker, John E

    2012-06-01

    This article focuses on selected topics in the diagnosis and management of patients with esophageal atresia (EA) with or without tracheoesophageal fistula. The current status of prenatal diagnosis and recent advances in surgical techniques, including thoracoscopic repair for short-gap EA and tension-induced esophageal growth for long-gap EA, are reviewed. Although no consensus exists among pediatric surgeons regarding the role of these procedures in the treatment of EA, one can reasonably expect that, as they evolve, their application will become more widespread in this challenging patient population.

  7. Congenital esophageal stenosis associated with esophageal atresia.

    PubMed

    McCann, F; Michaud, L; Aspirot, A; Levesque, D; Gottrand, F; Faure, C

    2015-04-01

    Congenital esophageal stenosis (CES) is a rare clinical condition but is frequently associated with esophageal atresia (EA). The aim of this study is to report the diagnosis, management, and outcome of CES associated with EA. Medical charts of CES-EA patients from Lille University Hospital, Sainte-Justine Hospital, and Montreal Children's Hospital were retrospectively reviewed. Seventeen patients (13 boys) were included. The incidence of CES in patients with EA was 3.6%. Fifteen patients had a type C EA, one had a type A EA, and one had an isolated tracheoesophageal fistula. Seven patients had associated additional malformations. The mean age at diagnosis was 11.6 months. All but two patients had non-specific symptoms such as regurgitations or dysphagia. One CES was diagnosed at the time of surgical repair of EA. In 12 patients, CES was suspected based on abnormal barium swallow. In the remaining four, the diagnostic was confirmed by esophagoscopy. Eleven patients were treated by dilation only (1-3 dilations/patient). Six patients underwent surgery (resection and anastomosis) because of failure of attempted dilations (1-7 dilations/patient). Esophageal perforation was encountered in three patients (18%). Three patients had histologically proven tracheobronchial remnants. CES associated with EA is frequent. A high index of suspicion for CES must remain in the presence of EA. Dilatation may be effective to treat some of them, but perforation is frequent. Surgery may be required, especially in CES secondary to ectopic tracheobronchial remnants.

  8. Eosinophilic esophagitis in children with esophageal atresia.

    PubMed

    Dhaliwal, J; Tobias, V; Sugo, E; Varjavandi, V; Lemberg, D; Day, A; Bohane, T; Ledder, O; Jiwane, A; Adams, S; Henry, G; Dilley, A; Shi, E; Krishnan, U

    2014-01-01

    Eosinophilic esophagitis (EoE) has only rarely been reported in esophageal atresia (EA) patients. A retrospective case analysis of all EA patients born at our center between January 1999 and April 2012 was performed. A total of 113 of patients were identified; 10 patients were excluded as a result of inadequate data. Eighteen patients (17%) were diagnosed with EoE. The average number of eosinophilis was 30/high-power field (HPF) (19/HPF-80/HPF). The median age for diagnosis of EoE was 1 year and 6 months (8 months-8 years and 7 months). Children with EoE had a significantly greater incidence of reflux symptoms, dysphagia, tracheomalacia, and 'hypoxic spells' (P < 0.05). EoE patients also underwent significantly more surgery including fundoplication and aortopexy when compared with those without EoE (P < 0.0001). Although the incidence of gastrostomy was greater in the EoE group (33% vs. 13%), this was not statistically significant. Half of the EoE patients had a coexisting atopic condition at time of diagnosis. The commonest condition was asthma 7/18 (38%) followed by specific food allergy 6/18 (33%). EoE was treated in 11 patients with either swallowed fluticasone or budesonide slurry. All improved clinically. Histologically, five had complete resolution and six had partial improvement. Six children with EoE were treated with acid suppression alone. All improved clinically, and 5/6 had subsequent histological resolution. One child who received acid suppression and an exclusion diet also improved. Seven patients (38%) had an esophageal stricture at time of EoE diagnosis. Five were dilated at time of the initial endoscopy, prior to the diagnosis of EoE being available. Two patients had resolution of their strictures on medical treatment of their EoE alone and did not require further dilatation. EoE was seen in 17% of children with EA in this study. EoE should be considered in EA patients with persistent symptoms on standard reflux treatment, increasing

  9. Endoscopic Management of Anastomotic Esophageal Strictures Secondary to Esophageal Atresia.

    PubMed

    Manfredi, Michael A

    2016-01-01

    The reported incidence of anastomotic stricture after esophageal atresia repair has varied in case series from as low as 9% to as high as 80%. The cornerstone of esophageal stricture treatment is dilation with either balloon or bougie. The goal of esophageal dilation is to increase the luminal diameter of the esophagus while also improving dysphagia symptoms. Once a stricture becomes refractory to esophageal dilation, there are several treatment therapies available as adjuncts to dilation therapy. These therapies include intralesional steroid injection, mitomycin C, esophageal stent placement, and endoscopic incisional therapy. PMID:26616905

  10. Thoracoscopic esophageal atresia repair made easy. An applicable trick.

    PubMed

    Hiradfar, Mehran; Shojaeian, Reza; Gharavi Fard, Mohammad; Joodi, Marjan; Sabzevari, Alireza; Nazarzade, Reza

    2013-03-01

    Thoracoscopic repair of esophageal atresia is becoming more popular but technical difficulties in handsewn anastomosis still remain challenging. This article presents an easy and applicable maneuver by passing the trans-esophageal tube before starting to suture in order to minimize the gap, reduce the tension over primary sutures and provide a better visualization of posterolateral parts of the anastomosis in thoracoscopic esophageal atresia repair. Using this maneuver makes tying easier and minimizes grasping and crushing damages to the anastomotic site.

  11. Esophageal atresia and anal atresia in a newborn with heterotaxia combined with other congenital defects

    PubMed Central

    Smigiel, Robert; Misiak, Blazej; Golebiowski, Waldemar; Lebioda, Arleta; Dorobisz, Urszula; Zielinska, Marzena; Patkowski, Dariusz

    2012-01-01

    Heterotaxia (HTX) is a heterogeneous group of laterality defects characterized by abnormal discordance of asymmetric thoracic and abdominal organs. Esophageal anomalies occur rarely in HTX cases although additional defects associated with esophageal atresia are common. We report on a rare case of a neonate with HTX and multiple congenital malformations as well as specific facial dysmorphism, corresponding only to a few cases described in literature. Clinical examination of the proband revealed esophageal atresia with distal tracheoesophageal fistula, anal atresia, abdominal situs inversus, dextrocardia with complex congenital heart defect and left lung agenesis. A complex genetic analysis revealed no genetic abnormalities. Despite extensive diagnostic procedures, the cause of the laterality sequence disruption remains unclear, indicating its multifactorial etiology.

  12. Esophageal atresia and anal atresia in a newborn with heterotaxia combined with other congenital defects.

    PubMed

    Smigiel, Robert; Misiak, Blazej; Golebiowski, Waldemar; Lebioda, Arleta; Dorobisz, Urszula; Zielinska, Marzena; Patkowski, Dariusz

    2012-03-01

    Heterotaxia (HTX) is a heterogeneous group of laterality defects characterized by abnormal discordance of asymmetric thoracic and abdominal organs. Esophageal anomalies occur rarely in HTX cases although additional defects associated with esophageal atresia are common. We report on a rare case of a neonate with HTX and multiple congenital malformations as well as specific facial dysmorphism, corresponding only to a few cases described in literature. Clinical examination of the proband revealed esophageal atresia with distal tracheoesophageal fistula, anal atresia, abdominal situs inversus, dextrocardia with complex congenital heart defect and left lung agenesis. A complex genetic analysis revealed no genetic abnormalities. Despite extensive diagnostic procedures, the cause of the laterality sequence disruption remains unclear, indicating its multifactorial etiology. PMID:27625802

  13. Motility, digestive and nutritional problems in Esophageal Atresia.

    PubMed

    Gottrand, Madeleine; Michaud, Laurent; Sfeir, Rony; Gottrand, Frédéric

    2016-06-01

    Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a rare congenital malformation. Digestive and nutritional problems remain frequent in children with EA both in early infancy and at long-term follow-up. These patients are at major risk of presenting with gastroesophageal reflux and its complications, such as anastomotic strictures. Esophageal dysmotility is constant, and can have important consequences on feeding and nutritional status. Patients with EA need a systematic follow-up with a multidisciplinary team. PMID:26752295

  14. Repair of esophageal atresia with proximal fistula using endoscopic magnetic compression anastomosis (magnamosis) after staged lengthening.

    PubMed

    Dorman, Robert M; Vali, Kaveh; Harmon, Carroll M; Zaritzky, Mario; Bass, Kathryn D

    2016-05-01

    We describe the treatment of a patient with long-gap esophageal atresia with an upper pouch fistula, mircogastria and minimal distal esophageal remnant. After 4.5 months of feeding via gastrostomy, a proximal fistula was identified by bronchoscopy and a thoracoscopic modified Foker procedure was performed reducing the gap from approximately 7-5 cm over 2 weeks of traction. A second stage to ligate the fistula and suture approximate the proximal and distal esophagus resulted in a gap of 1.5 cm. IRB and FDA approval was then obtained for endoscopic placement of 10-French catheter mounted magnets in the proximal and distal pouches promoting a magnetic compression anastomosis (magnamosis). Magnetic coupling occurred at 4 days and after magnet removal at 13 days an esophagram demonstrated a 10 French channel without leak. Serial endoscopic balloon dilation has allowed drainage of swallowed secretions as the baby learns bottling behavior at home. PMID:27012861

  15. Long Upper Pouch in Esophageal Atresia: A Rare Variant.

    PubMed

    Yhoshu, Enono; Mahajan, Jai Kumar; Dash, Vedarth

    2016-01-01

    The earliest clinical sign of esophageal atresia (EA) is excessive salivation and the diagnosis is made by failure to pass an infant feeding tube (IFT) into the stomach. The diagnostic errors may occur due to presence of an unusually long upper pouch, when the IFT seems to pass into the stomach. We describe one such case and review the relevant literature. PMID:26793598

  16. Management of patients with combined tracheoesophageal fistula, esophageal atresia, and duodenal atresia

    PubMed Central

    Nabzdyk, Christoph S.; Chiu, Bill; Jackson, Carl-Christian; Chwals, Walter J.

    2014-01-01

    INTRODUCTION Patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia (DA) pose a rare management challenge. PRESENTATION OF CASE Three patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia safely underwent a staged approach inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week. None of the patients suffered significant pre- or post-operative complications and our follow-up data (between 12 and 24 months) suggest that all patients eventually outgrow their reflux and respiratory symptoms. DISCUSSION While some authors support repair of all defects in one surgery, we recommend a staged approach. A gastrostomy tube is placed first for gastric decompression before TEF ligation and EA repair can be safely undertaken. The repair of the DA can then be performed within 3–7 days under controlled circumstances. CONCLUSION A staged approach of inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week resulted in excellent outcomes. PMID:25460495

  17. Ultrastructural Changes of the Smooth Muscle in Esophageal Atresia.

    PubMed

    Al-Shraim, Mubarak M; Eid, Refaat A; Musalam, Adel Osman; Radad, Khaled; Ibrahim, Ashraf H M; Malki, Talal A

    2015-01-01

    Esophageal atresia (EA) with or without tracheo-esophageal fistula (TEF) is a relatively rare congenital anomaly. Despite the advances in the management techniques and neonatal intensive care, esophageal dysmotility remains a very common problem following EA/TEF repair. Our current study aimed to describe the most significant ultrastructural changes of the smooth muscle cells (SMCs) trying to highlight some of the underlying mechanisms of esophageal dysmotility following EA/TEF repair. Twenty-three biopsies were obtained from the tip of the lower esophageal pouch (LEP) of 23 patients during primary repair of EA/TEF. Light microscopic examination was performed with hematoxylin and eosin (HE), and Van Gieson's stains. Ultrastructural examination was done using transmission electron microscopy (TEM). Histopathological examination showed distortion of smooth muscle layer and deposition of an abundant amount of fibrous tissue in-between smooth muscles. Using TEM, SMCs exhibited loss of the cell-to-cell adhesion, mitochondrial vacuolation, formation of myelin figures, and apoptotic fragmentation. There were also plasmalemmal projections and formation of ghost bodies. Interestingly, SMCs were found extending pseudopodia-like projections around adjacent collagen fibers. Engulfed collagen fibers by SMCs underwent degradation within autophagic vacuoles. Degeneration of SMCs and deposition of abundant extracellular collagen fibers are prominent pathological changes in LEP of EA/TEF. These changes might contribute to the pathogenesis of esophageal dysmotility in patients who have survived EA/TEF. PMID:26548437

  18. Predictive factors for complications in children with esophageal atresia and tracheoesophageal fistula.

    PubMed

    Shah, R; Varjavandi, V; Krishnan, U

    2015-04-01

    The objective of this study was to describe the incidence of complications in children with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) at a tertiary pediatric hospital and to identify predictive factors for their occurrence. A retrospective chart review of 110 patients born in or transferred to Sydney Children's Hospital with EA/TEF between January 1999 and December 2010 was done. Univariate and multivariate regression analyses were performed to identify predictive factors for the occurrence of complications in these children. From univariate analysis, early esophageal stricture formation was more likely in children with 'long-gap' EA (odds ratio [OR] = 16.32). Patients with early strictures were more likely to develop chest infections (OR = 3.33). Patients with severe tracheomalacia were more likely to experience 'cyanotic/dying' (OR = 180) and undergo aortopexy (OR = 549). Patients who had gastroesophageal reflux disease were significantly more likely to require fundoplication (OR = 10.83) and undergo aortopexy (OR = 6.417). From multivariate analysis, 'long-gap' EA was a significant predictive factor for late esophageal stricture formation (P = 0.007) and for gastrostomy insertion (P = 0.001). Reflux was a significant predictive factor for requiring fundoplication (P = 0.007) and gastrostomy (P = 0.002). Gastrostomy insertion (P = 0.000) was a significant predictive factor for undergoing fundoplication. Having a prior fundoplication (P = 0.001) was a significant predictive factor for undergoing a subsequent aortopexy. Predictive factors for the occurrence of complications post EA/TEF repair were identified in this large single centre pediatric study.

  19. An Unusual Case of Foreign Body Lodged in the Laryngopharynx of Neonate with Esophageal Atresia.

    PubMed

    Gupta, Rahul; Saxena, Manisha; Paul, Rozy; Gubbi, Sharan; Mathur, Praveen

    2016-01-01

    A blunt‑tipped red rubber catheter is used to confirm the presence of esophageal atresia in any newborn with drooling of saliva and frothing from the mouth. Failure to pass it beyond 10cms into the esophagus is considered diagnostic. We here in report an extremely rare case of broken tip of red rubber catheter lodged in the laryngopharynx of 2-day-old neonate of esophageal atresia with distal tracheoesophageal fistula. During endotracheal intubation foreign body was accidentally removed. PMID:26793600

  20. Persistent bronchography in a newborn with esophageal atresia.

    PubMed

    De Bernardo, Giuseppe; Sordino, Desiree; Giordano, Maurizio; Doglioni, Nicoletta; Trevisanuto, Daniele

    2016-06-01

    Esophageal atresia (EA) with tracheoesophageal fistula occurs in about 1:2,500 births. We report a case of persistent bronchography in a newborn with EA and distal tracheoesophageal fistula. A large amount of barium sulfate was injected for mistake by a tube directly in the right bronchus to evaluate the patency of the esophagus. The infant showed signs of respiratory distress; he was intubated and transported at children's Hospital Santobono Pausilipon. Here, it was performed a chest X-ray that confirmed the opacification of the right bronchial tree, and it was suspected an EA type 3b. The literature recommends that: evaluation of the patency of the esophagus, with an iodinate contrast medium, should be done in a pediatric specialized center for high risk of lung aspiration. PMID:27257464

  1. Esophageal atresia associated with a rare vascular ring and esophageal duplication diverticulum: a case report and review of the literature.

    PubMed

    Escobar, Mauricio A; Welke, Karl F; Holland, Randall M; Caty, Michael G

    2012-10-01

    Esophageal atresia with tracheoesophageal fistula (EA-TEF) associated with a right aortic arch poses a dilemma to the pediatric surgeon, often necessitating an operative approach via a left thoracotomy. A right aortic arch may be associated with a vascular ring, and EA-TEF, too, has been reported in association with a vascular ring. Rarely, esophageal atresia is associated with a second esophageal anomaly, such as a so-called "esophageal lung." To our knowledge, there is no report of all three in one patient. We report the first case of a patient with associated EA-TEF, vascular ring (diverticulum of Kommerell), and esophageal lung. The literature is reviewed for these rare entities.

  2. Thoracoscopic repair of esophageal atresia with a distal fistula – lessons from the first 10 operations

    PubMed Central

    Zaborowska, Kamila; Rogowski, Błażej; Kalińska, Anita; Nosek, Marzena; Golonka, Anna; Lesiuk, Witold; Obel, Marcin

    2015-01-01

    Introduction Thoracoscopic esophageal atresia (EA) repair was first performed in 1999, but still the technique is treated as one of the most complex pediatric surgical procedures. Aim The study presents a single-center experience and learning curve of thoracoscopic repair of esophageal atresia and tracheo-esophageal (distal) fistula. Material and methods From 2012 to 2014, 10 consecutive patients with esophageal atresia and tracheo-esophageal fistula were treated thoracoscopically in our center. There were 8 girls and 2 boys. Mean gestational age was 36.5 weeks and mean weight was 2230 g. Four children had associated anomalies. The surgery was performed after stabilization of the patient between the first and fourth day after birth. Five patients required intubation before surgery for respiratory distress. Bronchoscopy was not performed before the operation. Results In 8 patients, the endoscopic approach was successfully used thoracoscopically, while in 2 patients conversion to an open thoracotomy was necessary. In all patients except 1, the anastomosis was patent, with no evidence of leak. One patient demonstrated a leak, which did not resolve spontaneously, necessitating surgical repair. In long-term follow-up, 1 patient required esophageal dilatation of the anastomosis. All patients are on full oral feeding. Conclusions The endoscopic approach is the method of choice for the treatment of esophageal atresia in our center because of excellent visualization and precise atraumatic preparation even in neonates below a weight of 2000 g. PMID:25960794

  3. Real-time fetal magnetic resonance imaging for the dynamic visualization of the pouch in esophageal atresia.

    PubMed

    Salomon, L J; Sonigo, P; Ou, P; Ville, Y; Brunelle, F

    2009-10-01

    Esophageal atresia is the principal cause of congenital esophageal obstruction. Prenatal suspicion of esophageal atresia is usually based on the presence of polyhydramnios together with an absent stomach bubble. More recently, visualization of the dilatation of the blind-ending esophagus (esophageal pouch) during fetal swallowing has been reported and proposed as the most reliable sign for predicting esophageal atresia. Improvement of radiofrequency and computer technology as well as parallel data acquisition has greatly reduced magnetic resonance (MR) scanning time, allowing visualization of the fetus in cine-mode using fast imaging employing steady-state acquisition (FIESTA). We describe the application of FIESTA sequences in fetuses with suspected esophageal atresia for visualization of the esophageal pouch using MR imaging.

  4. Evaluating Necessity of Azygos Vein Ligation in Primary Repair of Esophageal Atresia.

    PubMed

    Fathi, Mehdi; Joudi, Marjan; Morteza, Afsaneh

    2015-12-01

    Surgery has dramatically improved survival of infants with esophageal atresia. However, early and late complications of these surgeries affect the future life of this population. A probable step toward minimizing such complications is through modifying the technique of surgery. We evaluated two groups of esophageal atresia undergoing surgery with two different techniques including preservation and ligation of the Azygos vein and compared early complications, duration of surgery, and hospital and neonatal intensive care unit (NICU) stay between them. A total number of 24 patients with mean age of 24 to 48 h, who were diagnosed with esophageal atresia, were included in the study. All cases were randomly allocated in two groups: group A (case group) in which patients underwent surgery with preservation of the Azygos vein and group B (control group) in which patients underwent the former surgical method with ligation of the Azygos vein. Incidence of early complications, duration of surgery, and NICU and hospital stay were compared between the two groups. None of the complications occurred in either group. Duration of surgery, NICU stay, and hospital stay were not statistically significant between the groups. Preserving the Azygos vein during esophageal atresia surgery is probably a good modification of the classic technique. PMID:26730061

  5. Contractile profile of esophageal and gastric fundus strips in experimental doxorubicin-induced esophageal atresia.

    PubMed

    Capeto, F A; Lima, F J B; Okoba, W; Ramos, F L; Messias, T F A; Rigonatto, G A; Sbragia, L; Magalhães, P J C; Melo-Filho, A A

    2015-05-01

    Esophageal atresia (EA) is characterized by esophageal and gastric motility changes secondary to developmental and postsurgical damage. This study evaluated the in vitro contractile profile of the distal esophagus and gastric fundus in an experimental model of EA induced by doxorubicin (DOXO). Wistar pregnant rats received DOXO 2.2 mg/kg on the 8th and 9th gestational days. On day 21.5, fetuses were collected, sacrificed, and divided into groups: control, DOXO without EA (DOXO-EA), and DOXO with EA (DOXO+EA). Strips from the distal esophagus and gastric fundus were mounted on a wire myograph and isolated organ-bath system, respectively, and subjected to increasing concentrations of carbamylcholine chloride (carbachol, CCh). The isolated esophagus was also stimulated with increasing concentrations of KCl. In esophagus, the concentration-effect curves were reduced in response to CCh in the DOXO+EA and DOXO-EA groups compared to the control group (P<0.05). The maximum effect values (Emax) for DOXO+EA and DOXO-EA were significantly lower than control (P<0.05), but the half-maximal effective concentration (EC50) values were not significantly different when the three groups were compared (P>0.05). In response to KCl, the distal esophagus samples in the three groups were not statistically different with regard to Emax or EC50 values (P>0.05). No significant difference was noted for EC50 or Emax values in fundic strips stimulated with CCh (P>0.05). In conclusion, exposure of dams to DOXO during gestation inhibited the contractile behavior of esophageal strips from offspring in response to CCh but not KCl, regardless of EA induction. The gastric fundus of DOXO-exposed offspring did not have altered contractile responsiveness to cholinergic stimulation.

  6. Contractile profile of esophageal and gastric fundus strips in experimental doxorubicin-induced esophageal atresia

    PubMed Central

    Capeto, F.A.; Lima, F.J.B.; Okoba, W.; Ramos, F.L.; Messias, T.F.A.; Rigonatto, G.A.; Sbragia, L.; Magalhães, P.J.C.; Melo-Filho, A.A.

    2015-01-01

    Esophageal atresia (EA) is characterized by esophageal and gastric motility changes secondary to developmental and postsurgical damage. This study evaluated the in vitro contractile profile of the distal esophagus and gastric fundus in an experimental model of EA induced by doxorubicin (DOXO). Wistar pregnant rats received DOXO 2.2 mg/kg on the 8th and 9th gestational days. On day 21.5, fetuses were collected, sacrificed, and divided into groups: control, DOXO without EA (DOXO-EA), and DOXO with EA (DOXO+EA). Strips from the distal esophagus and gastric fundus were mounted on a wire myograph and isolated organ-bath system, respectively, and subjected to increasing concentrations of carbamylcholine chloride (carbachol, CCh). The isolated esophagus was also stimulated with increasing concentrations of KCl. In esophagus, the concentration-effect curves were reduced in response to CCh in the DOXO+EA and DOXO-EA groups compared to the control group (P<0.05). The maximum effect values (Emax) for DOXO+EA and DOXO-EA were significantly lower than control (P<0.05), but the half-maximal effective concentration (EC50) values were not significantly different when the three groups were compared (P>0.05). In response to KCl, the distal esophagus samples in the three groups were not statistically different with regard to Emax or EC50 values (P>0.05). No significant difference was noted for EC50 or Emax values in fundic strips stimulated with CCh (P>0.05). In conclusion, exposure of dams to DOXO during gestation inhibited the contractile behavior of esophageal strips from offspring in response to CCh but not KCl, regardless of EA induction. The gastric fundus of DOXO-exposed offspring did not have altered contractile responsiveness to cholinergic stimulation. PMID:25760030

  7. [Diagnostic image (113). A neonate blowing bubbles. Esophageal atresia with tracheoesophageal fistula, Hirschsprung disease and suspected Down's syndrome].

    PubMed

    Postema, R R; Meradji, M; Langemeijer, R A Th M

    2002-11-01

    In a male neonate 'blowing bubbles', three diagnoses were seen on a combined thoracic and abdominal X-ray: esophageal atresia with tracheoesophageal fistula, Hirschsprung's disease, and suspected Down's syndrome (because of the presence of II pairs of ribs).

  8. ESOPHAGEAL ATRESIA WITH RECURRENT TRACHEOESOPHAGEAL FISTULAS AND MICRODUPLICATION 22q11.23.

    PubMed

    Puvabanditsin, S; Garrow, E; February, M; Yen, E; Mehta, R

    2015-01-01

    The microduplication 22q11.2 syndrome has a wide range of clinical manifestations. The phenotype ranges from normal to mental retardation and congenital anomalies. Esophageal atresia/tracheoesophageal fistula (EA/TEF) has recently been linked with the Tbx1 gene mutation located on the long arm of chromosome 22(22q11.21). We report a case with 1.4 Mb 22q11.23 duplication detected by array-CGH. The father of this infant has the same interstitial microduplication but with a normal phenotype. The phenotype seen in our case is type C (3B) esophageal atresia, tracheoesophageal fistula, and ventricular septal defect. Our patient underwent primary repair of OA/TEF malformations, which was later complicated by pneumonia and a recurrent TEF. PMID:26625662

  9. Primary intrathoracic gastric volvulus in the neonatal period: a differential diagnosis of esophageal atresia

    PubMed Central

    El Azzouzi, Driss

    2014-01-01

    Intrathoracic gastric volvulus in the neonatal period is a rare surgical emergency. Delays in diagnosis and treatment are life-threatening due to progressive deterioration of the gastric walls. Presentation in this period can be confused with the possibility of esophageal atresia or esophageal web. The upper gastrointestinal tract contrast study is diagnostic in this disease. The authors report a case of acute intrathoracic gastric volvulus diagnosis by radiologic-contrast-study in 1-day-old girl that was confirmed at surgery. The physiopathology, classification and different presentations of this entity are briefly reviewed. PMID:25309661

  10. [Recurrent tracheoesophageal fistula in type III esophageal atresia. Diagnosis and treatment are not easy].

    PubMed

    Lepeytre, C; Roquelaure, B; de Lagausie, P; Merrot, T; Dubus, J-C

    2014-07-01

    Recurrent tracheoesophageal fistula (TEF) is possible after repair of congenital esophageal atresia. The frequency of recurrent TEF is observed in about 10% of the cases. Within a cohort of 67 children with type III esophageal atresia repaired between 1998 and 2009, we aimed to identify the number of children with recurrent TEF, the risk factors for this condition, and the treatment proposed. The sex ratio was 1.7. Surgery was performed between 4 and 36 hours of life. Five children (7.5%) had a recurrent TEF, usually during the first 3 months, revealed by respiratory symptoms related to feeding in some cases. We noted that recurrent TEF was more frequent with anastomotic leakage (P=0.09) or postsurgical pneumothorax (P<0.01). The diagnosis was made in four cases out of five by a methylene blue test performed during a tracheobronchial endoscopy. Surgical treatment was noted in four children, with three postsurgical secondary effects. One child was treated by endoscopy and an esophageal clip. With a median follow-up of 52 months, no recurrence was noted. The recurrence of TEF may be linked to postsurgical events. Diagnosis is not easy and treatment is not clearly codified. Endoscopic treatment may be an advantageous option to surgery, likely with less morbidity.

  11. Thoracoscopic Esophageal Atresia with Tracheoesophageal Fistula Repair: The First Iranian Group Report, Passing the Learning Curve

    PubMed Central

    Hiradfar, Mehran; Gharavifard, Mohammad; Shojaeian, Reza; Joodi, Marjan; Nazarzadeh, Reza; Sabzevari, Alireza; Yal, Nazila; Eslami, Reza; Mohammadipour, Ahmad; Azadmand, Ali

    2016-01-01

    Background: Thoracoscopic treatment of esophageal atresia and tracheoesophageal fistula (EA+TEF) is accepted as a superior technique at least in cosmetic point of view but it is considered as an advance endoscopic procedure that needs a learning curve to be performed perfectly. This is the first report of Iranian group pediatric surgeons in thoracoscopic approach to EA. Methods and Materials: Since 2010, twenty four cases with EA+TEF underwent thoracoscopic approach in Sarvar Children Hospital (Mashhad -Iran). During the first 6 months, thoracoscopic approach to 6 cases of EA+TEF was converted to open procedure because of technical and instrumental problems. The first case of successful thoracoscopic EA repair was accomplished in 2010 and since then, 10 cases of EA+ TEF among 18 patients were treated successfully with thoracoscopic approach Results: Overall conversion rate was 58.3% but conversion rate after the primary learning curve period, was 35.7%. The main conversion causes include difficulties in esophageal anastomosis, limited exposure and deteriorating the patient's condition. Anastomotic leak and stenosis were observed in 20% and 40% respectively. Overall mortality rate was 4.2%. Conclusion: Thoracoscopic repair of esophageal atresia seems feasible and safe with considerable superiorities to the conventional method although acceptable results needs a prolonged learning curve and advanced endoscopic surgical skill. Clear judgment about the best surgical intervention for EA according to all cosmetic and functional outcomes needs further studies. PMID:27471677

  12. Short-term neurodevelopmental outcome of babies operated on for low-risk esophageal atresia: a pilot study.

    PubMed

    Aite, L; Bevilacqua, F; Zaccara, A; Ravà, L; Valfrè, L; Conforti, A; Braguglia, A; Bagolan, P

    2014-01-01

    Data on the neurodevelopmental outcome of esophageal atresia (EA) survivors are scarce, controversial, and based on small samples. This is an observational prospective longitudinal study on a selected cohort of low-risk EA survivors. We considered a low-risk EA survivor a patient with the following characteristics: gestational age >32 weeks, no long gap, no genetic or chromosomic anomaly associated with neurodevelopmental delay, and no further major surgical congenital anomalies. Infants were evaluated with scales derived from the Bayley Scales of Infant and Toddler Development - 3rd Edition at 6 and 12 months, with a score of 100 considered normal for each scale. Analysis of variance was used to assess differences of cognitive and motor development. Linear regression was used to assess the impact of the following clinical and sociodemographic variables: gender, birthweight, gestational age, length of hospital stay, number of surgeries and number of esophageal dilatations during first hospitalization, days of mechanical ventilation, weight at follow up, number of surgeries and esophageal dilatations at follow up, parental age, educational level, and socioeconomic status. Thirty children form the object of the study. The mean (standard deviation [SD]) cognitive scale's score was 93.7 (7.5) and 98.2 (9.6) at 6 and 12 months, respectively (P < 0.05). The mean (SD) motor scale's score was 97.6 (9.3) and 98.0 (12.1) at 6 and 12 months, respectively (P = n.s.). Children with a body weight <5° percentile at 12 months showed a mean (SD) cognitive score significantly lower when compared with those with a body weight >5° percentile: 88.8 (6.3) and 100.5 (8.9), respectively. At 12 months, children with unemployed mothers had a mean (SD) motor score significantly lower when compared with those in the other socioeconomic classes: 87.7 (9.8) and 100.6 (12.4), respectively. In conclusion, parents of babies operated on for low-risk EA can be reassured about neurodevelopmental

  13. Transthoracic single port with peroral assistance: an animal experiment to assess a less invasive technique for human esophageal atresia repair.

    PubMed

    Henriques-Coelho, Tiago; Soares, Tony R; Miranda, Alice; Moreira-Pinto, João; Correia-Pinto, Jorge

    2012-12-01

    Thoracoscopic repair of esophageal atresia has becoming the gold standard in many centers because it allows a better cosmetic result and avoids the musculoskeletal sequelae of a thoracotomy. Natural orifice translumenal endocopic surgery (NOTES) is a new surgical paradigm, and its human application has already been started in some procedures. In the present study, we explore the feasibility of performing an esophagoesophageal anastomosis using a single transthoracic single port combined with a peroral access in a rabbit model to simulate repair of esophageal atresia by hybrid NOTES in a human newborn. Adult male rabbits (Oryctolagus cuniculus, n=28) were used to perform the surgical protocol. We used a transthoracic telescope with a 3-mm working channel and a flexible endoscope with a 2.2-mm working channel by peroral access. We performed total esophagotomy with peroral scissors followed by an esophagoesophageal anastomosis achieved with a rigid transthoracic scope helped by the peroral operator. Extracorporeal transthoracic knots were performed to complete the anastomosis. The anastomoses were examined in loco and ex loco, after animal sacrifice. We successfully accomplished a complete esophageal anastomosis in all rabbits using a combination of transthoracic and peroral 3-mm instruments. This study provides important insights for a possible translation of hybrid NOTES to human newborns with esophageal atresia. Forward studies to accomplish their feasibility in human newborns will still be necessary.

  14. Esophageal atresia

    MedlinePlus

    ... gagging, and choking with attempted feeding Drooling Poor feeding ... Before birth, a mother's ultrasound may show too much amniotic ... nose into the stomach. If the feeding tube can’t pass all the ...

  15. Thoracoscopic repair of esophageal atresia with tracheoesophageal fistula: Basics of technique and its nuances

    PubMed Central

    Kanojia, Ravi Prakash; Bhardwaj, Neerja; Dwivedi, Deepak; Kumar, Raj; Joshi, Saajan; Samujh, Ram; Rao, K. L. N.

    2016-01-01

    Aim: To review the technique of thoracoscopic repair of esophageal atresia with tracheoesophageal fistula (TREAT) and results reported in literature and with authors’ experience. Patients and Methods: The technique of TREAT was reviewed in detail with evaluation in patients treated at authors’ institution. The patients were selected based on selection criteria and were followed postoperatively. The results available in literature were also reviewed. Results: A total of 29 patients (8 females) were operated by TREAT. Mean age was 2.8 days (range 2-6 days). Mean weight was 2.6 kg (range 1.8-3.2 kg). There was a leak in four patients, and two patients had to be diverted. They are now awaiting definitive repair. Twenty-one patients have completed a mean follow-up of 1.5 years and are doing well except for two patients who had a stricture and underwent serial esophageal dilatations. The results from current literature are provided in tabulated form. Conclusions: TREAT is now a well-established procedure and currently is the preferred approach wherever feasible. The avoidance of thoracotomy is a major advantage to the newborn and is proven to benefit the recovery in the postoperative patient. The technique demonstrated, and the tweaks reported make the procedure easy and is helpful to beginners. The outcome is very much comparable to the open repair as proven by various series. Various parameters like leak rate, anastomotic stricture are the same. The outcome is comparable if you TREAT these babies well. PMID:27365905

  16. Anophthalmia-esophageal atresia syndrome caused by an SOX2 gene deletion in monozygotic twin brothers with markedly discordant phenotypes.

    PubMed

    Zenteno, Juan Carlos; Perez-Cano, Hector J; Aguinaga, Monica

    2006-09-15

    The clinical combination of anophthalmia/microphthalmia and esophageal atresia was first recognized in 1988 as a distinct variable multi-system malformation syndrome and since then at least 17 cases of the disease have been described, all of them sporadic in occurrence. We report a heterozygous SOX2 gene mutation underlying the syndrome of anophthalmia/microphthalmia-esophageal atresia and demonstrate that this entity can be associated to considerable clinical variability as shown by the discordant ocular phenotype observed in monozygotic twin brothers carrying an SOX2 deletion. This is the first report describing a strikingly discordant eye phenotype in monozygotic twins with the condition, with one of our patients being the first reported individual carrying an SOX2 lesion associated with unilateral eye defect. We discuss the probable sources for this remarkable phenotypic heterogeneity of the anophthalmia/microphthalmia syndrome in individuals with an identical genetic constitution.

  17. Prevalence of Esophageal Atresia among 18 International Birth Defects Surveillance Programs

    PubMed Central

    Nassar, Natasha; Leoncini, Emanuele; Amar, Emmanuelle; Arteaga-Vázquez, Jazmín; Bakker, Marian K.; Bower, Carol; Canfield, Mark A.; Castilla, Eduardo E.; Cocchi, Guido; Correa, Adolfo; Csáky-Szunyogh, Melinda; Feldkamp, Marcia L.; Khoshnood, Babak; Landau, Danielle; Lelong, Nathalie; López-Camelo, Jorge S.; Lowry, R. Brian; McDonnell, Robert; Merlob, Paul; Métneki, Julia; Morgan, Margery; Mutchinick, Osvaldo M.; Palmer, Miland N.; Rissmann, Anke; Siffel, Csaba; Sìpek, Antonin; Szabova, Elena; Tucker, David; Mastroiacovo, Pierpaolo

    2015-01-01

    BACKGROUND The prevalence of esophageal atresia (EA) has been shown to vary across different geographical settings. Investigation of geographical differences may provide an insight into the underlying etiology of EA. METHODS The study population comprised infants diagnosed with EA during 1998 to 2007 from 18 of the 46 birth defects surveillance programs, members of the International Clearinghouse for Birth Defects Surveillance and Research. Total prevalence per 10,000 births for EA was defined as the total number of cases in live births, stillbirths, and elective termination of pregnancy for fetal anomaly (ETOPFA) divided by the total number of all births in the population. RESULTS Among the participating programs, a total of 2943 cases of EA were diagnosed with an average prevalence of 2.44 (95% confidence interval [CI], 2.35–2.53) per 10,000 births, ranging between 1.77 and 3.68 per 10,000 births. Of all infants diagnosed with EA, 2761 (93.8%) were live births, 82 (2.8%) stillbirths, 89 (3.0%) ETOPFA, and 11 (0.4%) had unknown outcomes. The majority of cases (2020, 68.6%), had a reported EA with fistula, 749 (25.5%) were without fistula, and 174 (5.9%) were registered with an unspecified code. CONCLUSIONS On average, EA affected 1 in 4099 births (95% CI, 1 in 3954–4251 births) with prevalence varying across different geographical settings, but relatively consistent over time and comparable between surveillance programs. Findings suggest that differences in the prevalence observed among programs are likely to be attributable to variability in population ethnic compositions or issues in reporting or registration procedures of EA, rather than a real risk occurrence difference. PMID:22945024

  18. Structural and numerical changes of chromosome X in patients with esophageal atresia.

    PubMed

    Brosens, Erwin; de Jong, Elisabeth M; Barakat, Tahsin Stefan; Eussen, Bert H; D'haene, Barbara; De Baere, Elfride; Verdin, Hannah; Poddighe, Pino J; Galjaard, Robert-Jan; Gribnau, Joost; Brooks, Alice S; Tibboel, Dick; de Klein, Annelies

    2014-09-01

    Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is a relatively common birth defect often associated with additional congenital anomalies such as vertebral, anal, cardiovascular, renal and limb defects, the so-called VACTERL association. Yet, little is known about the causal genetic factors. Rare case reports of gastrointestinal anomalies in children with triple X syndrome prompted us to survey the incidence of structural and numerical changes of chromosome X in patients with EA/TEF. All available (n=269) karyotypes of our large (321) EA/TEF patient cohort were evaluated for X-chromosome anomalies. If sufficient DNA material was available, we determined genome-wide copy number profiles with SNP array and identified subtelomeric aberrations on the difficult to profile PAR1 region using telomere-multiplex ligation-dependent probe amplification. In addition, we investigated X-chromosome inactivation (XCI) patterns and mode of inheritance of detected aberrations in selected patients. Three EA/TEF patients had an additional maternally inherited X chromosome. These three female patients had normal random XCI patterns. Two male EA/TEF patients had small inherited duplications of the XY-linked SHOX (Short stature HOmeoboX-containing) locus. Patients were small for gestational age at birth (

  19. Tricuspid atresia

    MedlinePlus

    Tri atresia; Valve disorder - tricuspid atresia; Congenital heart - tricuspid atresia; Cyanotic heart disease - tricuspid atresia ... Tricuspid atresia is an uncommon form of congenital heart disease. It affects about 5 in every 100, ...

  20. [Medium-term outcome, follow-up, and quality of life in children treated for type III esophageal atresia].

    PubMed

    Lepeytre, C; De Lagausie, P; Merrot, T; Baumstarck, K; Oudyi, M; Dubus, J-C

    2013-10-01

    The aim of this study was to evaluate the medium-term outcome (health status, medical and surgical French National Health Authority-recommended follow-up, and quality of life) of children born with type III esophageal atresia (EA). Previous events (during the perinatal period, associated abnormalities, respiratory and digestive complications) of children treated for type III EA at the Marseille university hospitals between 1999 and 2009 were noted. Parents completed a standardized questionnaire concerning the health of their children during the previous year, and a quality-of-life questionnaire (PedsQL 4.0) was also completed by children aged more than 8 years. Among the 68 children treated, 44 responded to our solicitation (mean age, 7.6 years; range, 3-12.8 years). Previous important events were : pneumonia(s) (65%), asthma before the age of 3 years (66%), hospitalization for a respiratory event (45%), fundoplication (20%), and esophageal dilatation (45%). We noted current chronic cough (16%), asthma (30%), dysphagia (39%), and symptomatic gastroesophageal reflux (9%). National guidelines were not respected, except for the surgical indications in children aged less than 6 years. The quality-of-life scores (n=43 children) were similar to healthy controls but were negatively influenced by a gastrostomy procedure (P=0.020), pneumonia (P=0.013), and hospitalization due to a respiratory event (P=0.006) or a digestive event (P=0.010), and also by current asthma (P=0.004). In conclusion, despite recurrent respiratory or digestive symptoms and inadequate recommended follow-up, the quality of life of children treated for type III of EA is good.

  1. Copy number detection in discordant monozygotic twins of Congenital Diaphragmatic Hernia (CDH) and Esophageal Atresia (EA) cohorts.

    PubMed

    Veenma, Danielle; Brosens, Erwin; de Jong, Elisabeth; van de Ven, Cees; Meeussen, Connie; Cohen-Overbeek, Titia; Boter, Marjan; Eussen, Hubertus; Douben, Hannie; Tibboel, Dick; de Klein, Annelies

    2012-03-01

    The occurrence of phenotypic differences between monozygotic (MZ) twins is commonly attributed to environmental factors, assuming that MZ twins have a complete identical genetic make-up. Yet, recently several lines of evidence showed that both genetic and epigenetic factors could have a role in phenotypic discordance after all. A high occurrence of copy number variation (CNV) differences was observed within MZ twin pairs discordant for Parkinson's disease, thereby stressing on the importance of post-zygotic mutations as disease-predisposing events. In this study, the prevalence of discrepant CNVs was analyzed in discordant MZ twins of the Esophageal Atresia (EA) and Congenital Diaphragmatic Hernia (CDH) cohort in the Netherlands. Blood-derived DNA from 11 pairs (7 EA and 4 CDH) was screened using high-resolution SNP arrays. Results showed an identical copy number profile in each twin pair. Mosaic chromosome gain or losses could not be detected either with a detection threshold of 20%. Some of the germ-line structural events demonstrated in five out of eleven twin pairs could function as a susceptible genetic background. For example, the 177-Kb loss of chromosome 10q26 in CDH pair-3 harbors the TCF7L2 gene (Tcf4 protein), which is implicated in the regulation of muscle fiber type development and maturation. In conclusion, discrepant CNVs are not a common cause of twin discordancy in these investigated congenital anomaly cohorts. PMID:22071887

  2. Normal exon copy number of the GLI2 and GLI3 genes in patients with esophageal atresia.

    PubMed

    Bednarczyk, D; Smigiel, R; Patkowski, D; Laczmanska, I; Lebioda, A; Laczmanski, L; Sasiadek, M M

    2013-01-01

    Esophageal atresia (EA) is a congenital developmental defect of the alimentary tract concerning the interruption of the esophagus with or without connection to the trachea. The incidence of EA is 1 in 3000-3500 of live-born infants, and occurs in both isolated and syndromic (in combination with abnormalities in other organ systems) forms. The molecular mechanisms underlying the development of EA are poorly understood. Knockout studies in mice indicate that genes like Sonic hedgehog, Gli2, and Gli3 play a role in the etiology of EA. These facts led us to hypothesize that Sonic hedgehog-GLI gene rearrangements are associated with EA in humans. To test this hypothesis, we screened patients with isolated and syndromic EA for GLI2 and/or GLI3 microrearrangements using methods to estimate the copy number (Multiplex Ligation-dependent Probe Amplification, real-time polymerase chain reaction). To our best knowledge this is the first study assessing copy number of GLI2 and GLI3 genes in patients with EA. PMID:23442119

  3. Determinants of gap length in esophageal atresia with tracheoesophageal fistula and the impact of gap length on outcome

    PubMed Central

    Rassiwala, Muffazzal; Choudhury, Subhasis Roy; Yadav, Partap Singh; Jhanwar, Praveen; Agarwal, Raghu Prakash; Chadha, Rajiv; Debnath, Pinaki Ranjan

    2016-01-01

    Aim: This study was aimed at identifying factors which may affect the gap length in cases of esophageal atresia with tracheoesophageal fistula (EA-TEF) and whether gap length plays any role in determining the outcome. Materials and Methods: All consecutive cases of EA-TEF were included and different patient parameters were recorded. Plain radiographs with a nasogastric tube in the upper esophagus were taken. Patients were grouped into T1-T2; T2-T3; T3-T4; and T4 depending on the thoracic vertebral level of the arrest of the tube. During surgery, the gap length between the pouches was measured using a Vernier caliper and the patients were grouped into A, B, and C (gap length >2.1 cm; >1-≤2 cm and ≤1 cm). The operative gap groups were compared with the radiography groups and the other recorded parameters. Results: Total numbers of cases were 69. Birth weight was found to be significantly lower in Group A (mean = 2.14 kg) as compared to Group B (mean = 2.38 kg) and Group C patients (mean = 2.49 kg) (P = 0.016). The radiographic groups compared favorably with the intraoperative gap length groups (P < 0.001). The need for postoperative ventilation (70.83% in Group A vs. 36.84% in Group C, P = 0.032) and mortality (62.5%, 26.9% and 15.8% in Group A, B, and C, respectively, P = 0.003) co-related significantly with the gap length. Conclusion: Birth weight had a direct reciprocal relationship with the gap length. Radiographic assessment correlated with intraoperative gap length. Higher gap length was associated with increased need for postoperative ventilation and poor outcome. PMID:27365907

  4. Pulmonary atresia

    MedlinePlus

    ... disease - pulmonary atresia; Cyanotic heart disease - pulmonary atresia; Valve - disorder pulmonary atresia ... septum may also have a poorly developed tricuspid valve. They may also have an underdeveloped right ventricle ...

  5. Duodenal atresia

    MedlinePlus

    ... would. Many infants with duodenal atresia also have Down syndrome. Duodenal atresia is often associated with other birth ... abnormality. Other problems (such as those related to Down syndrome) must be treated as appropriate. Outlook (Prognosis) Recovery ...

  6. Esophagitis

    MedlinePlus

    ... swelling of the esophagus. The esophagus is the tube that leads from the back of the mouth to the stomach. Causes Esophagitis is often caused by stomach fluid that flows back into the esophagus. The fluid contains acid ...

  7. Respiratory distress of the newborn: congenital laryngeal atresia.

    PubMed

    Ambrosio, Art; Magit, Anthony

    2012-11-01

    Congenital laryngeal atresia is a rare cause of respiratory distress of the newborn. The defect may be isolated or occur in association with other congenital abnormalities, notably the presence of a tracheoesophageal fistula, esophageal atresia, encephalocele, or Congenital High Airway Obstructive Syndrome (CHAOS). We present the case of a newborn with no identified intrapartum abnormalities with respiratory distress at birth secondary to near-complete laryngeal atresia. Management included tracheostomy, repeated endoscopic incisions, and serial balloon dilatations employing the topical use of Mitomycin C. Seven year follow-up was significant for mobilization of the true vocal cords bilaterally, as well as successful decannulation.

  8. The effects of acute tension increase on rat esophageal muscle contractions: An in vitro study.

    PubMed

    Soyer, Tutku; Kalkışım, Said; Yalcin, Sule; Müderrisoğlu, Ahmet; Taş, Sadık Taşkın; Tanyel, Feridun Cahit; Ertunç, Mert; Sara, Yıldırım

    2015-10-01

    In long-gap esophageal atresia surgeries, anastomoses can be tensioned by several traction methods in order to establish esophageal continuity. It is unclear whether the etiology of esophageal dysmotility after traction is related with esophageal atresia itself or tensioned esophagus. Therefore, we evaluated the effects of acute in vitro esophageal tension application on esophageal muscle contractility in rats. 26 Wistar rats weighing 250-300 g were included to the study. After diethyl ether anesthesia, proximal segment (PS) and distal segment (DS) of esophagus were removed and suspended in an isolated organ bath kept at 37°C, Krebs-Henseleit solution. Rats were enrolled into four groups including control group (CG, n=14) without tension, 5 g (5G, n=4), 15 g (15G, n=4) and 25 g (25G, n=4) tension groups. In all groups, contractile responses to electrical field stimulation (EFS), carbachol and KCl, and relaxation responses to serotonin were obtained. In CG, higher contractile responses were obtained in PS than DS after EFS. Both PS and DS showed higher contractile amplitudes in 5G with respect to that of CG, 15G and 25G (p<0.05). In 5G, contractile responses to carbachol were significantly increased in both PS and DS with respect to CG (p<0.05). However, contractile amplitudes in response to carbachol were decreased in PS when tension was increased to 15 g and 25 g. In DS, contractile responses in 15G and 25G were lower than 5G, and still higher than CG. Serotonin relaxation responses in PS were decreased when compared to CG at tension levels of 5 g, 15 g and 25 g (p<0.05). In DS, responses to serotonin were also decreased in tension groups. PS had higher contraction amplitudes than DS when contractile responses were obtained by high K(+) (p<0.05). Tension groups of both PS and DS showed increased contractions to high K(+) compared to CG (p<0.05). Increased esophageal tension led to increase in cholinergic responses of smooth muscles as well as in EFS

  9. Complicated intestinal atresias.

    PubMed

    Miller, R C

    1979-05-01

    In this group of 45 intestinal atresia patients (duodenum, 16; jejunum, 24; ileum five) at the University of Mississippi Medical Center, individual hospitalizations ranged up to 245 days. Twelve patients required multiple operations, and the overall mortality rate was 22% (ten patients). While the patients with duodenal atresia had the greatest incidence of other congenital anomalies, including Down's syndrome, the patients with jejunal atresia presented with the most challenging surgical problems. Of the 24 jejunal atresia patients, only three had a single, simple area of obstruction. The remainder were complicated by other gastrointestinal lesions (five patients), by multiple areas of atresia (seven patients) including those in one surviving patient with 22 separate atretic segments, and by the Christmas tree deformity (nine patients). Intraoperative management of the complicated atresia should include: 1) grouping of multiple atresias during resection, 2) adequate resection of the dilated proximal atonic loop, 3) end-to-end anastomoses, 4) avoidance of intraluminal catheters, 5) additional resection of a segment of the distal loop in the Christmas tree deformity and 6) consideration of the shish kebab technique for multiple atretic webs. Postoperative management should involve early intravenous nutrition and repeated exploration for continued obstruction.

  10. Tracheoesophageal fistula and esophageal atresia repair

    MedlinePlus

    ... stomach) may be placed to give your child nutrition. Your child will have another surgery later to repair the ... either of these problems. Your baby will receive nutrition by vein ... or until other problems have been treated or have gone away.

  11. Passage of nasogastric tube through tracheo-esophageal fistula into stomach: A rare event.

    PubMed

    Kamble, Ravikiran Shankar; Gupta, Rahulkumar; Gupta, Abhaya; Kothari, Paras; Dikshit, K Vishesh; Kesan, Krishnakumar; Mudkhedkar, Kedar

    2014-07-16

    Esophageal atresia with tracheo-oesophageal fistula (TEF) occurs in 1 in 3500 live births. Anorectal malformation is found to be associated with 14% of TEF. Esophageal atresia with TEF is a congenital anomaly which classically presents as excessive frothing from the mouth and respiratory distress. Rarely gastric position of the feeding tube in a case of TEF can be obtained delaying the diagnosis of TEF. We had an uncommon situation where a nasogastric tube reached the stomach through the trachea and tracheo-esophageal fistula, leading to misdiagnosis in a case of esophageal atresia with tracheoesophageal fistula. By using a stiff rubber catheter instead of a soft feeding tube for the diagnosis of esophageal atresia and TEF, such situation can be avoided.

  12. A Surviving Child With Complete Proximal Tracheal Atresia

    PubMed Central

    Haight, Ken; Sankaran, Koravangattu; Shokeir, Mohamed

    1984-01-01

    An infant was born with an unusual combination of primitive foregut anomalies consisting of complete proximal tracheal atresia, proximal esophageal atresia and distal tracheoesophageal fistula. Before the birth, the family physician suspected an anomaly of the upper airway or esophageal occlusion on the basis of hydramnios evident at the thirty-third to thirty-fourth week of gestation, and earlier amniocentesis which indicated a normal level of α-fetoprotein. He consulted the hospital obstetrics and neonatology departments, which were thus prepared to deal with a potential airway problem at the birth. At birth, prompt airway management, including tracheostomy, prevented anoxic damage to the child. Features which should alert the physician to tracheal obstruction include antenatal polyhydramnios; severe respiratory distress without an audible cry and palpable distal trachea in the newborn; and failure to advance the endotracheal tube beyond the infant's vocal cords. PMID:21279046

  13. Newborn Screening for Biliary Atresia

    PubMed Central

    Wang, Kasper S.

    2016-01-01

    Biliary atresia is the most common cause of pediatric end-stage liver disease and the leading indication for pediatric liver transplantation. Affected infants exhibit evidence of biliary obstruction within the first few weeks after birth. Early diagnosis and successful surgical drainage of bile are associated with greater survival with the child’s native liver. Unfortunately, because noncholestatic jaundice is extremely common in early infancy, it is difficult to identify the rare infant with cholestatic jaundice who has biliary atresia. Hence, the need for timely diagnosis of this disease warrants a discussion of the feasibility of screening for biliary atresia to improve outcomes. Herein, newborn screening for biliary atresia in the United States is assessed by using criteria established by the Discretionary Advisory Committee on Heritable Disorders in Newborns and Children. Published analyses indicate that newborn screening for biliary atresia by using serum bilirubin concentrations or stool color cards is potentially life-saving and cost-effective. Further studies are necessary to evaluate the feasibility, effectiveness, and costs of potential screening strategies for early identification of biliary atresia in the United States. PMID:26620065

  14. Esophageal Cancer

    MedlinePlus

    ... esophagus, and chest wall Lung Cancer Esophageal Cancer Gastroesophageal Reflux Disease Barrett’s Esophagus Chest Wall Tumors Mediastinal Tumors ... Section Navigation Select Topic Lung Cancer Esophageal Cancer Gastroesophageal Reflux Disease Barrett’s Esophagus Chest Wall Tumors Mediastinal Tumors ...

  15. Esophageal cancer

    MedlinePlus

    Cancer - esophagus ... Esophageal cancer is not common in the United States. It occurs most often in men over 50 years old. There are two main types of esophageal cancer: squamous cell carcinoma and adenocarcinoma. These two types ...

  16. Esophageal Cancer

    MedlinePlus

    ... from your throat to your stomach. Early esophageal cancer usually does not cause symptoms. Later, you may ... You're at greater risk for getting esophageal cancer if you smoke, drink heavily, or have acid ...

  17. [Biliary atresia and polysplenia syndrome].

    PubMed

    Kerkeni, Yosra; Ksia, Amine; Zitouni, Hayet; Belghith, Mohsen; Lassad, Sahnoun; Krichene, Imed; Mekki, Mongi; Nouri, Abdellatif

    2015-01-01

    Polysplenia syndrome is a rare malformation characterized by the association of multiple rates and other congenital anomalies dominated by cardiac, vascular, intestinal and bile malformations. We report the observation of a patient operated in the neonatal period (3 days) for an upper intestinal obstruction with situs inversus. Surgical exploration noted the presence of multiple rates, a preduodenal vein, a biliary atresia and a duodenal atresia. The surgical procedures performed were a latero-lateral duodeno-duodenostomy and hepatoportoenterostomy of KASAI with simple immediate and delayed outcomes. The follow up was of 23 years. We recall the epidemiological characteristics of this malformative association and we discuss the role played by the prognosis of polysplenia syndrome in the evolution of biliary atresia. The diagnosis and treatment of biliary atresia are always urgent to increase the chances of success of the Kasai, and the chances of prolonged survival with native liver. However, almost all long-term survivors (even anicteric) have biliary cirrhosis, which requires lifelong follow up. PMID:26815511

  18. A study of associated congenital anomalies with biliary atresia

    PubMed Central

    Gupta, Lucky; Bhatnagar, Veereshwar

    2016-01-01

    Background/Purpose: This study aims to analyze the incidence and type of various associated anomalies among infants with extrahepatic biliary atresia (EHBA), compare their frequency with those quoted in the existing literature and assess their role in the overall management. Materials and Methods: A retrospective study was performed on 137 infants who underwent the Kasai procedure for EHBA during the past 12 years. The medical records were reviewed for the incidence and type of associated anomalies in addition to the details of the management of the EHBA. Results: Of the137 infants, 40 (29.2%) were diagnosed as having 58 anomalies. The majority of patients had presented in the 3rd month of life; mean age was 81 ± 33 days (range = 20-150 days). There were 32 males and 8 females; boys with EHBA had a higher incidence of associated anomalies. Of these 40 patients, 22 (37.9%) had vascular anomalies, 13 patients (22.4%) had hernias (umbilical-10, inguinal-3), 7 patients (12.1%) had intestinal malrotation, 4 patients (6.8%) had choledochal cyst, 1 patient (1.7%) had Meckel's diverticulum, 3 patients (5%) had undergone prior treatment for jejunoileal atresias (jejunal-2, ileal-1), 2 patients (3.4%) had undergone prior treatment for esophageal atresia and tracheoesophageal fistula, 2 patients (3.4%) had spleniculi, and 2 patients (3.4%) were diagnosed as having situs inversus. Conclusions: The most common associated anomalies in our study were related to the vascular variation at the porta hepatis and the digestive system. The existence of anomalies in distantly developing anatomic regions in patients with EHBA supports the possibility of a “generalized” insult during embryogenesis rather than a “localized” defect. In addition, male infants were observed to have significantly more associated anomalies as compared with the female infants in contrast to earlier reports. PMID:26862288

  19. Eosinophilic esophagitis.

    PubMed

    Kedia, Saurabh; Baruah, Bhaskar Jyoti; Makharia, Govind; Ahuja, Vineet

    2015-01-01

    Eosinophilic esophagitis (EoE) is a clinico-pathological entity characterised by symptoms of esophageal dysfunction and eosinophilia on esophageal mucosal biopsies in the absence of other causes of esophageal eosinophilia. It is a chronic inflammatory condition of esophagus often characterized by refractory reflux symptoms in children and dysphagia in adults. It occurs as a result of Th2 inflammatory response to environmental triggers (food antigens) in genetically predisposed individuals. The diagnostic criteria include symptoms of esophageal dysfunction, esophageal eosinophilia (> 15/hpf), and a PPI trial (persistent eosinophilia after 8 weeks of PPI). Mainstay of treatment at present is topical steroids and dietary therapy. Maintenance treatment should be considered to prevent long term complications. PMID:27522734

  20. Esophagitis - infectious

    MedlinePlus

    ... conditions that suppress or weaken your immune system Organisms (germs) that cause esophagitis include fungi, yeast, and viruses. Common organisms include: Candida albicans Cytomegalovirus (CMV) Herpes simplex virus ( ...

  1. Esophageal Cancer.

    PubMed

    Alsop, Benjamin R; Sharma, Prateek

    2016-09-01

    Esophageal cancer carries a poor prognosis among gastrointestinal malignancies. Although esophageal squamous cell carcinoma predominates worldwide, Western nations have seen a marked rise in the incidence of esophageal adenocarcinoma that parallels the obesity epidemic. Efforts directed toward early detection have been difficult, given that dysplasia and early cancer are generally asymptomatic. However, significant advances have been made in the past 10 to 15 years that allow for endoscopic management and often cure in early stage esophageal malignancy. New diagnostic imaging technologies may provide a means by which cost-effective, early diagnosis of dysplasia allows for definitive therapy and ultimately improves the overall survival among patients. PMID:27546839

  2. [Esophageal dysphagia].

    PubMed

    Thumshirn, M

    2007-04-01

    Dysphagia can be caused by a number of disorders such as benign or malignant obstruction of the esophagus, inflammatory alterations of the mucosa or primary esophageal motility disorders. Endoscopic evaluation is recommended for all patients to exclude malignancy and to establish or confirm a diagnosis. This article provides an overview of the most frequent inflammatory and functional esophageal disorders causing dysphagia. Clinical findings, diagnostic procedures and therapeutic management of primary esophageal motility disorders such as achalasia and diffuse esophageal spasm as well as of GERD and eosinophilic esophagitis are discussed. The diagnosis of achalasia is made by barium swallow with fluoroscopy and by manometry. Therapeutic options for achalasia are pneumatic dilatation of the esophagogastric junction, laparoscopic cardiomyotomy combined with fundoplication and botulinum toxin injection of the lower esophageal sphincter Diffuse esophageal spasm is manometrically characterized by normal peristalsis intermittently interrupted by simultaneous contractions. Potential medical therapies are PPIs for underlying GERD, smooth-muscle relaxants and antidepressant medications. GERD is a multifaceted disease caused by abnormal reflux of gastric contents into the esophagus leading to chronic symptoms or mucosal damage. Therapy includes lifestyle modifications, acid suppressive medications mainly by PPI and laparoscopic fundoplication in selected patients. Eosinophilic esophagitis is a chronic inflammatory disorder of the esophagus diagnosed histologically. The main symptom of eosinophilic esophagitis is dysphagia for solid food with imminent risk of food impaction. Systemic or topical corticosteroids are the therapy of choice.

  3. Genetics Home Reference: esophageal atresia/tracheoesophageal fistula

    MedlinePlus

    ... cases of isolated EA/TEF , no specific genetic changes or environmental factors have been conclusively determined to be the cause. Non-isolated or syndromic forms of EA/TEF can be caused by changes in single genes or in chromosomes, or they ...

  4. Congenital nasolacrimal atresia in 4 alpacas

    PubMed Central

    Sandmeyer, Lynne S.; Bauer, Bianca S.; Breaux, Carrie B.; Grahn, Bruce H.

    2011-01-01

    Four alpacas, 2 wk to 1 y of age, were diagnosed with congenital atresia of the nasal puncta of the nasolacrimal duct. Dacryocystorhinography confirmed and localized the atresia. All animals were treated successfully by surgical creation of nasal puncta and patency was ensured by placement of a surgical stent for several weeks. PMID:21629429

  5. Gallbladder Duplication Associated with Gastro-Intestinal Atresia

    PubMed Central

    Gupta, Rahul; Gupta, Shilpi; Sharma, Pramila; Bhandari, Anu; Gupta, Arun Kumar; Mathur, Praveen

    2016-01-01

    Gallbladder duplication in association with other GIT anomalies is a rare entity. We report two neonates; one with duodenal atresia and the other newborn with pyloric atresia, ileal atresia and colonic atresia, both were associated with gallbladder duplication which has not been reported earlier. PMID:27123398

  6. Isolated Vaginal Agenesis Associated with Multiple Gastrointestinal Anomalies: A Case Report.

    PubMed

    Angotti, R; Molinaro, F; Bulotta, A L; Ferrara, F; Sica, M; Bindi, E; Messina, M

    2016-01-01

    More than 50% of infants with esophageal atresia have associated anomalies. We present a case report of a 46XX neonate with long-gap esophageal atresia and tracheoesophageal fistula (EA/TEF), anorectal malformation, bowel duplication and vaginal agenesis. This is an unusual association of abnormalities which had not yet described in literature. PMID:27433450

  7. Isolated Vaginal Agenesis Associated with Multiple Gastrointestinal Anomalies: A Case Report

    PubMed Central

    Angotti, R; Molinaro, F; Bulotta, AL; Ferrara, F; Sica, M; Bindi, E; Messina, M

    2016-01-01

    More than 50% of infants with esophageal atresia have associated anomalies. We present a case report of a 46XX neonate with long-gap esophageal atresia and tracheoesophageal fistula (EA/TEF), anorectal malformation, bowel duplication and vaginal agenesis. This is an unusual association of abnormalities which had not yet described in literature. PMID:27433450

  8. Current Updates on Choanal Atresia

    PubMed Central

    Kwong, Kelvin M.

    2015-01-01

    Choanal atresia (CA) is a relatively uncommon but well-recognized condition characterized by the anatomical closure of the posterior choanae in the nasal cavity. Since the original description back in the early eighteenth century, there have been controversies regarding its exact pathogenesis, the optimal surgical approach, and the use of adjunct treatments such as post-surgical stenting and anti-neoplastic agents, despite of abundant literature available. The emergence and development of new technologies play a significant role in the management of this condition. This review provides a comprehensive clinical update on CA and identifies areas for future study based on the existing available literature. PMID:26106591

  9. The Canadian Biliary Atresia Registry: Improving the care of Canadian infants with biliary atresia

    PubMed Central

    Butler, Alison E; Schreiber, Richard A; Yanchar, Natalie; Emil, Sherif; Laberge, Jean-Martin

    2016-01-01

    Biliary atresia is the most common cause of end-stage liver disease and liver cirrhosis in children, and the leading indication for liver transplantation in the paediatric population. There is no cure for biliary atresia; however, timely diagnosis and early infant age at surgical intervention using the Kasai portoenterostomy optimize the prognosis. Late referral is a significant problem in Canada and elsewhere. There is also a lack of standardized care practices among treating centres in this country. Biliary atresia registries currently exist across Europe, Asia and the United States. They have provided important evidence-based information to initiate changes to biliary atresia care in their countries with improvements in outcome. The Canadian Biliary Atresia Registry was initiated in 2013 for the purpose of identifying best standards of care, enhancing public education, facilitating knowledge translation and advocating for novel national public health policy programs to improve the outcomes of Canadian infants with biliary atresia. PMID:27398049

  10. Esophageal perforation

    MedlinePlus

    ... object or caustic chemicals, such as household cleaners, disk batteries, and battery acid Trauma or injury to ... may have esophageal perforation. Prevention These injuries are hard to prevent. Alternative Names Perforation of the esophagus ...

  11. Esophageal anastomosis.

    PubMed

    Yuan, Y; Wang, K-N; Chen, L-Q

    2015-01-01

    This review gives an overview of the esophageal anastomosis. The history, various techniques and substitution organs, their advantages and disadvantages, healing mechanism, complications, and actual trend of this essential part of esophageal surgery are described. The history of the esophageal anastomosis extending from the first anastomosis in 1901 to today has undergone more than one century. In the early days, the success rate of the anastomosis was extremely low. As the technology progressed, the anastomosis got significant achievement. Various anastomotic techniques are currently being used. However, controversies exist on the choice of anastomotic method concerning the success rate, postoperative complication and quality of life. How to choose the method, no one can give the best answer. We searched the manuscripts about the esophageal anastomoses in recent years and studied the controversy questions about the anastomosis. Performing an esophageal anastomosis is a technical matter, and suture healing is independent of the patient's biologic situation. Every anastomosis technique has its own merit, but the outcomes were different if it was performed by different surgeons, and we also found that the complication rate of the anastomosis was mainly associated with the surgeons. So the surgeons should learn from their previous experience and others to avoid technical errors.

  12. Esophageal Microbiome in Eosinophilic Esophagitis

    PubMed Central

    Harris, J. Kirk; Fang, Rui; Wagner, Brandie D.; Choe, Ha Na; Kelly, Caleb J.; Schroeder, Shauna; Moore, Wendy; Stevens, Mark J.; Yeckes, Alyson; Amsden, Katie; Kagalwalla, Amir F.; Zalewski, Angelika; Hirano, Ikuo; Gonsalves, Nirmala; Henry, Lauren N.; Masterson, Joanne C.; Robertson, Charles E.; Leung, Donald Y.; Pace, Norman R.; Ackerman, Steven J.; Furuta, Glenn T.; Fillon, Sophie A.

    2015-01-01

    Objective The microbiome has been implicated in the pathogenesis of a number of allergic and inflammatory diseases. The mucosa affected by eosinophilic esophagitis (EoE) is composed of a stratified squamous epithelia and contains intraepithelial eosinophils. To date, no studies have identified the esophageal microbiome in patients with EoE or the impact of treatment on these organisms. The aim of this study was to identify the esophageal microbiome in EoE and determine whether treatments change this profile. We hypothesized that clinically relevant alterations in bacterial populations are present in different forms of esophagitis. Design In this prospective study, secretions from the esophageal mucosa were collected from children and adults with EoE, Gastroesophageal Reflux Disease (GERD) and normal mucosa using the Esophageal String Test (EST). Bacterial load was determined using quantitative PCR. Bacterial communities, determined by 16S rRNA gene amplification and 454 pyrosequencing, were compared between health and disease. Results Samples from a total of 70 children and adult subjects were examined. Bacterial load was increased in both EoE and GERD relative to normal subjects. In subjects with EoE, load was increased regardless of treatment status or degree of mucosal eosinophilia compared with normal. Haemophilus was significantly increased in untreated EoE subjects as compared with normal subjects. Streptococcus was decreased in GERD subjects on proton pump inhibition as compared with normal subjects. Conclusions Diseases associated with mucosal eosinophilia are characterized by a different microbiome from that found in the normal mucosa. Microbiota may contribute to esophageal inflammation in EoE and GERD. PMID:26020633

  13. Eosinophilic esophagitis

    PubMed Central

    Gupte, Anand R; Draganov, Peter V

    2009-01-01

    Eosinophilic esophagitis is increasingly recognized in adults. The diagnosis is based on the presence of both typical symptoms and pathologic findings on esophageal biopsy. Patients usually present with dysphagia, food impaction and/or reflux-like symptoms, and biopsy of the esophagus shows more than 15 eosinophils per high-power field. In addition, it is essential to exclude the presence of known causes of tissue eosinophilia such as gastroesophageal reflux disease, infections, malignancy, collagen vascular diseases, hypersensitivity, and inflammatory bowel disease. There are no standardized protocols for the therapy of eosinophilic esophagitis. A variety of therapeutic approaches including acid suppression, dietary modifications, topical corticosteroids and endoscopic dilation can be used alone or in combination. PMID:19115464

  14. Pill esophagitis.

    PubMed

    Kikendall, J W

    1999-06-01

    Nine hundred seventy-nine cases of pill esophagitis due to nearly 100 different medications are reviewed. Pill-induced injuries occur when caustic medicinal pills dissolve in the esophagus rather than passing rapidly into the stomach as intended. Most patients suffer only self-limited pain, but esophageal hemorrhage, stricture, and perforation may occur, and fatal injuries have been reported. The incidence of this iatrogenic injury can be reduced but not eliminated by emphasizing the importance of taking pills while upright and with plenty of fluids. PMID:10372925

  15. Primary Transanal Management of Rectal Atresia in a Neonate.

    PubMed

    M, Braiek; A, Ksia; I, Krichen; S, Belhassen; K, Maazoun; S, Ben Youssef; N, Kechiche; M, Mekki; A, Nouri

    2016-01-01

    Rectal atresia (RA) with a normal anus is a rare anomaly. We describe a case of rectal atresia in a newborn male presenting with an abdominal distension and failure of passing meconium. The rectal atresia was primarily operated by transanal route.

  16. Primary Transanal Management of Rectal Atresia in a Neonate

    PubMed Central

    M, Braiek; A, Ksia; I, Krichen; S, Belhassen; K, Maazoun; S, Ben youssef; N, Kechiche; M, Mekki; A, Nouri

    2016-01-01

    Rectal atresia (RA) with a normal anus is a rare anomaly. We describe a case of rectal atresia in a newborn male presenting with an abdominal distension and failure of passing meconium. The rectal atresia was primarily operated by transanal route. PMID:27123404

  17. The use of ileocolic segment for esophageal replacement in children

    PubMed Central

    Bal, Harshjeet Singh; Sen, Sudipta

    2016-01-01

    Aims: To evaluate and describe the procedure and outcome of ileocolic replacement of esophagus. Materials and Methods: We review 7 children with esophageal injuries, who underwent esophageal replacement using ileocolic segment in Christian Medical College, Vellore, India between 2006 and 2014. Results: The ileocolic segment was used in 7 children with scarred or inadequate esophagus. There were 4 girls and 3 boys, who underwent esophageal replacement using isoperistaltic ileocolic segment in this period. Age at presentation varied from 1 month to 14 years with an average of 4.6 years. The indications for ileocolic replacements were corrosive strictures in 5, failed esophageal atresia repair in one and gastric volvulus related esophageal stricture in another. The average follow-up duration was 37 months. One child with corrosive stricture lost to follow-up and died 2 years later in another center. Other 6 children were free of dysphagia till the last follow-up. Conclusions: Although the ileocolic segment is not commonly used for esophageal substitution, it can be useful in special situations where the substitution needs to reach the high cervical esophagus and also where the stomach is scarred and not suitable for gastric pull-up. PMID:27365904

  18. Intestinal atresia and stenosis: a review comparing its morphology.

    PubMed

    Johnson, R

    1986-03-01

    A review of the literature on intestinal atresia of domestic animal species and humans was done. The 5 types of intestinal occlusions described in human infants are atresia type 1, atresia type 2, atresia type 3, stenosis, and the "apple peel" or "Christmas tree" deformity. The intestinal defects described in domestic animal species such as the bovine, equine and porcine are similar to those of human infants. The "T-formation", an intestinal defect of the bovine resembling atresia type 3, and rectal stricture, an acquired intestinal defect of the porcine resembling stenosis, were described recently. Intestinal atresia is similar in several species and these similarities raise the questions as to whether the pathogenic mechanism and possibly etiologies of intestinal atresia are similar in these species.

  19. Microdeletion 22q11 and oesophageal atresia

    PubMed Central

    Digilio, M. C.; Marino, B.; Bagolan, P.; Giannotti, A.; Dallapiccola, B.

    1999-01-01

    Oesophageal atresia (OA) is a congenital defect associated with additional malformations in 30-70% of the cases. In particular, OA is a component of the VACTERL association. Since some major features of the VACTERL association, including conotruncal heart defect, radial aplasia, and anal atresia, have been found in patients with microdeletion 22q11.2 (del(22q11.2)), we have screened for del(22q11.2) by fluorescent in situ hybridisation (FISH) in 15 syndromic patients with OA. Del(22q11.2) was detected in one of them, presenting with OA, tetralogy of Fallot, anal atresia, neonatal hypocalcaemia, and subtle facial anomalies resembling those of velocardiofacial syndrome. The occurrence of del(22q11.2) in our series of patients with OA is low (1/15), but this chromosomal anomaly should be included among causative factors of malformation complexes with OA. In addition, clinical variability of del(22q11.2) syndrome is further corroborated with inclusion of OA in the list of the findings associated with the deletion.


Keywords: microdeletion 22q11; oesophageal atresia; VACTERL association; velocardiofacial syndrome PMID:10051013

  20. Multiple gastrointestinal atresias in two consecutive siblings.

    PubMed

    Gahukamble, D B; Gahukamble, L D

    2002-03-01

    Two consecutive female siblings with multiple gastrointestinal atresias are described. The history of consanguinity in the parents and the presence of extensive typical pathological lesions suggest a genetically-induced developmental fault in the alimentary tract during the early embryonic period.

  1. Review of esophageal injuries and stenosis: Lessons learn and current concepts of management

    PubMed Central

    Ramareddy, Raghu Sampalli; Alladi, Anand

    2016-01-01

    Aim: To review the patients with esophageal injuries and stenosis with respect to their etiology, clinical course, management, and the lessons learnt from these. Materials and Methods: Retrospective descriptive observation review of children with esophageal injuries and stenosis admitted between January 2009 and April 2015. Results: Eighteen children with esophageal injuries of varied etiology were managed and included, seven with corrosive injury, five with perforation due to various causes, three with mucosal erosion, two with trachea esophageal fistula (TEF), and one wall erosion. The five children who had perforation were due to poststricture dilatation in a child with esophageal atresia and secondary to foreign body impaction or its attempted retrieval in four. Alkaline button cell had caused TEF in two. Three congenital esophageal stenosis (CES) had presented with dysphagia and respiratory tract infection. Six corrosive stricture and two CES responded to dilatation alone and one each of them required surgery. Four of the children with esophageal perforation were detected early and required drainage procedure (1), diversion (1), and medical management (2). Pseudo diverticulum was managed expectantly. Among TEF, one had spontaneous closure and other one was lost to follow-up. All the remaining nineteen children have recovered well except one CES had mortality. Conclusion: Esophageal injuries though rare can be potentially devastating and life-threatening. PMID:27365909

  2. Esophageal pH monitoring

    MedlinePlus

    pH monitoring - esophageal; Esophageal acidity test ... esophagitis You may need to have the following tests if your doctor suspects esophagitis : Barium swallow Esophagogastroduodenoscopy (also called upper GI endoscopy)

  3. Colon atresia, facial hemiaplasia, and anophthalmia: a case report.

    PubMed

    Szavay, Philipp O; Schliephake, Henning; Hubert, Oliver; Glüer, Sylvia

    2002-10-01

    A case of a newborn with atresia of the transverse colon and right facial hemiaplasia, anophthalmia, and cerebral dysfunction is reported. Colon atresia is a rare cause of congenital bowel obstruction and often associated with other malformations such as abdominal wall defects, gastrointestinal, cardiac, urogenital, and musculosceletal lesions. Facial hemiaplasia may arise in frame of chromosomal defects or as a result of neurovascular compromise caused by congenital amniotic bands. However, the combination of colon atresia and facial hemiaplasia has not been reported before.

  4. Esophageal dilation in eosinophilic esophagitis.

    PubMed

    Richter, Joel E

    2015-10-01

    Tissue remodeling with scaring is common in adult EoE patients with long standing disease. This is the major factor contributing to their complaints of solid food dysphagia and recurrent food impactions. The best tests to define the degree of remodeling are barium esophagram, high resolution manometry and endoscopy. Many physicians are fearful to dilate EoE patients because of concerns about mucosal tears and perforations. However, multiple recent case series attest to the safety of esophageal dilation and its efficacy with many patients having symptom relief for an average of two years. This chapter will review the sordid history of esophageal dilation in EoE patients and outline how to perform this procedure safely. The key is graduated dilation over one to several sessions to a diameter of 15-18 mm. Postprocedural pain is to be expected and mucosal tears are a sign of successful dilation, not complications. In some healthy adults, occasional dilation may be preferred to regular use of medications or restricted diets. This approach is now supported by recent EoE consensus statements and societal guidelines.

  5. A Rare Case of Jejunal Atresia Due to Intrauterine Intussusception.

    PubMed

    Joshi, Sanjeev B; Kinhal, Vidyadhar; Desai, Mahesh; Tilak; Choudhari, Fazal Ur Rehman

    2015-09-01

    Intestinal atresia is generally caused by intrauterine vascular obstructions involving mesenteric vessels. Intrauterine intussusceptions (IUI) are one of these disruptive events. Intestinal intussusceptions affects children commonly between 3 months and 3 years of age, but it rarely affects in intrauterine life. The relationship between intrauterine intussusception and intestinal atresia has been demonstrated by few cases in literature, suggesting intrauterine intussusception as a rare cause of intestinal atresia. We report a 7-day-old full term neonate presenting with intrauterine intussusceptions (jejuno-jejunal) resulting in jejunal atresia. PMID:26500958

  6. Desloratadine Induced Pill Esophagitis

    PubMed Central

    Alkim, Huseyin; Iscan, Mustafa

    2012-01-01

    Pill induced esophagitis is a rare complication mostly seen in patients using tetracycline and its derivatives or non-steroidal anti-inflammatory drugs. Here we present a 37 years old female patient experiencing pill esophagitis after taking desloratadine without liquid immediately before going to bed. This was the first pill esophagitis case related with desloratadine reported in the literature. Pill esophagitis is a preventable complication that consists of giving simple advice of how and when to take medication.

  7. Surgical management of tricuspid atresia and anomalous left brachiocephalic vein.

    PubMed

    Koutlas, T C; Wernovsky, G; Slack, M C; Weinberg, P M; Spray, T L

    1998-06-01

    An anomalous left brachiocephalic vein is an uncommon systemic venous anomaly, which usually has no clinical significance. We describe a case of tricuspid atresia with such an anomalous left brachiocephalic vein. The presence of this unusual venous anomaly had a number of implications in the surgical management of the tricuspid atresia. PMID:9647100

  8. Endoscopic management of esophageal stenosis in children: New and traditional treatments.

    PubMed

    Dall'Oglio, Luigi; Caldaro, Tamara; Foschia, Francesca; Faraci, Simona; Federici di Abriola, Giovanni; Rea, Francesca; Romeo, Erminia; Torroni, Filippo; Angelino, Giulia; De Angelis, Paola

    2016-02-25

    Post-esophageal atresia anastomotic strictures and post-corrosive esophagitis are the most frequent types of cicatricial esophageal stricture. Congenital esophageal stenosis has been reported to be a rare but typical disease in children; other pediatric conditions are peptic, eosinophilic esophagitis and dystrophic recessive epidermolysis bullosa strictures. The conservative treatment of esophageal stenosis and strictures (ES) rather than surgery is a well-known strategy for children. Before planning esophageal dilation, the esophageal morphology should be assessed in detail for its length, aspect, number and level, and different conservative strategies should be chosen accordingly. Endoscopic dilators and techniques that involve different adjuvant treatment strategies have been reported and depend on the stricture's etiology, the availability of different tools and the operator's experience and preferences. Balloon and semirigid dilators are the most frequently used tools. No high-quality studies have reported on the differences in the efficacies and rates of complications associated with these two types of dilators. There is no consensus in the literature regarding the frequency of dilations or the diameter that should be achieved. The use of adjuvant treatments has been reported in cases of recalcitrant stenosis or strictures with evidence of dysphagic symptoms. Corticosteroids (either systemically or locally injected), the local application of mitomycin C, diathermy and laser ES sectioning have been reported. Some authors have suggested that stenting can reduce both the number of dilations and the treatment length. In many cases, this strategy is effective when either metallic or plastic stents are utilized. Treatment complications, such esophageal perforations, can be conservatively managed, considering surgery only in cases with severe pleural cavity involvement. In cases of stricture relapse, even if such relapses occur following the execution of well

  9. Endoscopic management of esophageal stenosis in children: New and traditional treatments

    PubMed Central

    Dall’Oglio, Luigi; Caldaro, Tamara; Foschia, Francesca; Faraci, Simona; Federici di Abriola, Giovanni; Rea, Francesca; Romeo, Erminia; Torroni, Filippo; Angelino, Giulia; De Angelis, Paola

    2016-01-01

    Post-esophageal atresia anastomotic strictures and post-corrosive esophagitis are the most frequent types of cicatricial esophageal stricture. Congenital esophageal stenosis has been reported to be a rare but typical disease in children; other pediatric conditions are peptic, eosinophilic esophagitis and dystrophic recessive epidermolysis bullosa strictures. The conservative treatment of esophageal stenosis and strictures (ES) rather than surgery is a well-known strategy for children. Before planning esophageal dilation, the esophageal morphology should be assessed in detail for its length, aspect, number and level, and different conservative strategies should be chosen accordingly. Endoscopic dilators and techniques that involve different adjuvant treatment strategies have been reported and depend on the stricture’s etiology, the availability of different tools and the operator’s experience and preferences. Balloon and semirigid dilators are the most frequently used tools. No high-quality studies have reported on the differences in the efficacies and rates of complications associated with these two types of dilators. There is no consensus in the literature regarding the frequency of dilations or the diameter that should be achieved. The use of adjuvant treatments has been reported in cases of recalcitrant stenosis or strictures with evidence of dysphagic symptoms. Corticosteroids (either systemically or locally injected), the local application of mitomycin C, diathermy and laser ES sectioning have been reported. Some authors have suggested that stenting can reduce both the number of dilations and the treatment length. In many cases, this strategy is effective when either metallic or plastic stents are utilized. Treatment complications, such esophageal perforations, can be conservatively managed, considering surgery only in cases with severe pleural cavity involvement. In cases of stricture relapse, even if such relapses occur following the execution of well

  10. Jejunal atresia with absent mesentery and a helical ileum.

    PubMed

    Zerella, J T; Martin, L W

    1976-11-01

    Of 59 infants with jejunoileal atresia, seven presented with absence of mesentery, the main superior mesenteric artery ceasing to exist beyond the origin of the right colic or ileocolic artery, and a helical ileum. This distinctive form of jejunal atresia has been recognized with increasing frequency in recent years. Distal to the atresia, the small bowel receives its blood supply retrograde from an artery derived from the ileocolic or right colic arcades, and the ileum coils around its nutrient artery in an "apple peel" or "Christmas tree" deformity. The first three patients in this report died. The lastion, but they recovered as their malabsorption gradually disappeared. The treatment includes resection of the dilated bowel, as in other atresias. Resection of part of the distal bowel may be required for additional atresias or for poor vascularity with questionable viability. Postoperative malabsorption generally requires intravenous hyperalimentation.

  11. Colonic atresia associated with annular pancreas: An extremely rare and previously unreported association

    PubMed Central

    Halder, Pankaj; Kumar, Rajarshi; Mukhopadhyay, Madhumita; Mandal, Kartik Chandra; Mukhopadhyay, Biswanath

    2015-01-01

    Colonic atresia is the rarest entity among the all intestinal atresias and may be associated with anterior abdominal wall defect, small intestinal atresia, Hirschsprung's disease, and other anomalies. Here, we are reporting a case of colonic atresia associated with annular pancreas, which has not been reported previously to the best of our knowledge. PMID:26166992

  12. Fetal Heterotaxy with Tricuspid Atresia, Pulmonary Atresia, and Isomerism of the Right Atrial Appendages at 22 Weeks

    PubMed Central

    Solomon, Julia E.; Stock, John H.; Richardson, Randy R.; Silverman, Norman H.

    2013-01-01

    We report the accurate prenatal diagnosis at 22 weeks gestation of right atrial isomerism in association with tricuspid atresia. Several distinctive sonographic features of isomerism of the right atrial appendages were present in this fetus: complex cardiac abnormality, ventriculoarterial discordance, juxtaposition of the aorta and the inferior vena cava to the right side, pulmonary atresia, and anomalous pulmonary venous return to the morphological right atrium. Tricuspid atresia, which is an extremely rare lesion within heterotaxy spectrum disorders, was present. Postnatal investigations confirmed all prenatally diagnosed abnormalities, with additional findings of pulmonary atresia with discontinuous pulmonary arteries and bilateral arterial ducts, asplenia, and bilateral eparterial bronchi. To our knowledge, tricuspid atresia in the setting of isomerism of the right atrial appendages has not previously been diagnosed or reported prenatally. Because of the complexity of cardiac lesions that may be present in cases of atrial isomerism, these disorders should be considered even if sonographic findings are uncommon or atypical. PMID:24147244

  13. From Reflux Esophagitis to Esophageal Adenocarcinoma.

    PubMed

    Souza, Rhonda F

    2016-01-01

    Reflux esophagitis causes Barrett's metaplasia, an abnormal esophageal mucosa predisposed to adenocarcinoma. Medical therapy for reflux esophagitis focuses on decreasing gastric acid production with proton pump inhibitors. We have reported that reflux esophagitis in a rat model develops from a cytokine-mediated inflammatory injury, not from a caustic chemical (acid) injury. In this model, refluxed acid and bile stimulate the release of inflammatory cytokines from esophageal squamous cells, recruiting lymphocytes first to the submucosa and later to the luminal surface. Emerging studies on acute reflux esophagitis in humans support this new concept, suggesting that reflux-induced cytokine release may be a future target for medical therapies. Sometimes, reflux esophagitis heals with Barrett's metaplasia, a process facilitated by reflux-related nitric oxide (NO) production and Sonic Hedgehog (Hh) secretion by squamous cells. We have shown that NO reduces expression of genes that promote a squamous cell phenotype, while Hh signaling induces genes that mediate the development of the columnar cell phenotypes of Barrett's metaplasia. Agents targeting esophageal NO production or Hh signaling conceivably could prevent the development of Barrett's esophagus. Persistent reflux promotes cancer in Barrett's metaplasia. We have reported that acid and bile salts induce DNA damage in Barrett's cells. Bile salts also cause NF-x03BA;B activation in Barrett's cells, enabling them to resist apoptosis in the setting of DNA damage and likely contributing to carcinogenesis. Oral treatment with ursodeoxycholic acid prevents the esophageal DNA damage and NF-x03BA;B activation induced by toxic bile acids. Altering bile acid composition might be another approach to cancer prevention. PMID:27331918

  14. Right esophageal lung in a preterm child with VACTERL association and Mayer-Rokitansky-Kuster-Hauser syndrome.

    PubMed

    Linke, F; Kraemer, W; Ansorge, M; Brzezinska, R; Berger, S

    2005-04-01

    We report on a preterm girl (birth weight 1,200 g) with a right esophageal lung in esophageal atresia type VIag (according to the extended classification of Kluth). Additionally, the child suffered from an atrioseptal defect, a dextrocardia with a left descending aorta, a duodenal atresia, a high type of anal atresia (VACTERL association), agenesis of the left kidney, and agenesis of the vagina, uterus, and ovarian tubes (Mayer-Rokitansky-Kuster-Hauser syndrome or incomplete MURCS association). The child was treated with an emergency gastrostomy because of increasing abdominal dilatation. Thereafter, the parents refused further surgical treatment, and the child was maintained on basic therapy. After an uneventful period of 4 weeks, the child died of an acute massive aspiration. This case shows that sufficient spontaneous ventilation is possible in esophageal lung as long as a gastrostomy is kept on suction to prevent overinflation of the affected lung and the stomach. Ethical aspects have to be considered when treatment is planned in cases of prematurity and associated malformations when a chance of good survival is rather limited. The stepwise approach as proposed in the present case appears to be the only possible therapeutic regimen that can be offered in this complicated condition.

  15. Structural changes occurring during atresia in sheep ovarian follicles.

    PubMed

    Hay, M R; Cran, D G; Moor, R M

    1976-07-01

    The structural changes that characterize primary, secondary and tertiary atresia in sheep Graafian follicles have been studied by means of histological, histochemical and ultrastructural techniques. In primary atresia vacuoles representing swollen endoplasmic reticulum are prominent along the antral border together with disorganized granulosa cells containing pyknotic nuclei. Phagocytic cells, which increase in number as atresia progresses, were seen within the membrana granulosa and are considered to be transformed granulosa cells. Even in follicles classified as nonatretic, a few antral vacuoles and occasional pyknotic nuclei are present. During secondary atresia there is a large increase in the number of cells with pyknotic nuclei; many of these nuclei had been extruded and had fused to form the characteristic Feulgen-positive atretic bodies found along the edge of the antral cavity. These bodies usually have a diameter of up to 15 mum but occasionally reached as much as 400 mum. A second area of degeneration is frequently present in the membrana granulosa, two or three cell layers from the basal lamina, and it is at this level that exfoliation of granulosa cells occurs in tertiary atresia. In contrast to the membrana granulosa, there are during secondary atresia, only slight indications of degeneration in the cumulus. In tertiary atresia the membrana granulosa is highly disorganized; the atretic bodies are often fewer in number than at earlier stages. The basal lamina remains essentially intact. It is at this stage that the first clear signs of degeneration occur in the theca interna. Despite some disintegration of the cumulus, the integrity of the oocyte is maintained and its nucleus remains vesicular. Changes in the thecal microcirculation may plan a key role in atresia: adjacent to the basal lamina of non-atretic follicles, there is a well-developed capillary network which is significantly reduced as atresia progresses. PMID:991198

  16. [Biliary atresias operated with favourable results: predictable outcome].

    PubMed

    Broto, J; Gil Vernet, J M; Ormaechea, M

    2005-01-01

    Since 1975, our experience in the treatment of biliary atresia with Kasai's technique has improved little by little, achieving 65% favourable outcome in the last five years. We define "good results" as the complete restoration of biliary flow and normalization of bilirrubin levels. The long-term evolution of these good results can be diverse. The objective of the present work is to analyze the outcome of patients in our series in whom a favourable initial response was achieved, as well as evaluating their present situation and future perspectives. The authors present a total of 17 patients operated by Kasai's technique since 1985, that constitutes the group with good results in our series. The controls were based on general analysis, liver function and periodic ultrasound explorations. All received a standardized medical treatment consisting of vitamin supplements (A, D3, E, K) minerals (zinc, calcium, phosphate, iron) ursodexoxicolic acid, luminal,as well as close control of calorie intake. In two patients the levels of bilirrubine were progressively increased with time, stabilizing at between 5/6 mgs/100 ml, with progressive hepatic hardening, appearance of splenomegalia, indirect signs of portal hypertension and a slight deterioration of hepatic function. One received a transplant at age 12 with Quick levels below 50%. The other, aged 16, continues with an acceptable hepatic function and good quality of life under recommendation of transplant. Eleven patients with ages ranging from fourteen months to seventeen years presented slight and firm hepatomegalia, moderate portal hypertension, GOT 71 +/- 8 mg/100 ml, GPT 97 +/- 11 mg/100 ml and normal bilirrubine levels. From this group, 3 patients, all under five years of age, experienced bleeding from esophageal varices which were controlled by sclerosis and medical treatment (propanolol and isosorbide dinitrate). Recently, one three year-old patient developed a hepatocarcinoma of rapid, mortal evolution. Since then

  17. Atresia revisited: two basic patterns of atresia of bovine antral follicles.

    PubMed

    Irving-Rodgers, H F; van Wezel, I L; Mussard, M L; Kinder, J E; Rodgers, R J

    2001-11-01

    Our observations of bovine follicles indicated that the original histological classifications of atresia were inaccurate. A detailed histological, ultrastructural and immunohistochemical study of antral follicles from bovine ovaries collected from an abattoir and from animals whose large follicles had been monitored by ultrasonography was conducted to investigate this further. Nidogen and CD68 were immunolocalized to observe the follicular basal lamina and macrophages, respectively. In randomly collected ovaries, approximately one quarter of all antral follicles were undergoing antral atresia, as designated in this study. Antral atresia was characterized by early destruction of the layers of the membrana granulosa closest to the antrum, whereas the most basal cells remained intact. Numerous pyknotic nuclei were observed in the most antral layers and in the antrum close to the membrana granulosa. This is the classic description of atretic follicles and was observed at all sizes of follicle development and almost universally in large follicles (> 5 mm in diameter), including dominant follicles. Basal atretic follicles, as designated in this study, were almost as prevalent as the antral atretic follicles, and were characterized by initial destruction of the most basal layer of granulosa cells, whereas the cells in the most antral layers remained associated with each other and were predominantly healthy. Pyknotic nuclei and the nuclei of dying basal cells budded into apoptotic bodies were observed rarely. The basal lamina of basal atretic follicles was often breached by macrophages, which were phagocytosing dying basal granulosa cells. The theca was characterized by an increased deposition of collagen, and the cells were orientated randomly, rather than lying parallel to the membrana granulosa as in healthy follicles. Basal atresia occurred in small (< 5 mm in diameter) follicles only. Importantly, these basal atretic follicles were originally identified incorrectly in

  18. Tricuspid atresia: analysis of coronary artery distribution and ventricular morphology.

    PubMed Central

    Deanfield, J E; Tommasini, G; Anderson, R H; Macartney, F J

    1982-01-01

    There is still disagreement concerning the precise nature of the anterior ventricular chamber in "tricuspid atresia". Some argue that it is a right ventricle, while our own previous studies have suggested it is comparable to the outlet chamber seen in classical "single ventricle". We have compared the morphology of the anterior ventricular chambers in 48 examples of tricuspid atresia (absent right atrioventricular connection), 24 hearts with double inlet to the left ventricular chamber, and 15 hearts with pulmonary atresia and intact ventricular septum. Since there is further disagreement concerning the nature of the coronary artery which delimits the posterior extent of the ventricular septum in these hearts, we analysed the position of this vessel relative to external reference points on the atrioventricular junction. No significant difference was found with respect to the morphometry of hearts with tricuspid atresia (absent right atrioventricular connection) and those with double inlet. In both groups, however, significant differences were shown between hearts with ventriculoarterial concordance and discordance. Coronary artery disposition was the same in both groups and different from that found in hearts with pulmonary atresia and intact septum. We conclude that the ventricular morphology is comparable in hearts with tricuspid atresia (absent right atrioventricular connection) and those with double inlet to a left ventricular chamber. The lesions are distinguishable by their atrioventricular connection which is nevertheless univentricular in both. Images PMID:7138712

  19. Myoarchitecture and connective tissue in hearts with tricuspid atresia

    PubMed Central

    Sanchez-Quintana, D; Climent, V; Ho, S; Anderson, R

    1999-01-01

    Objective—To compare the atrial and ventricular myoarchitecture in the normal heart and the heart with tricuspid atresia, and to investigate changes in the three dimensional arrangement of collagen fibrils.
Methods—Blunt dissection and cell maceration with scanning electron microscopy were used to study the architecture of the atrial and ventricular musculature and the arrangement of collagen fibrils in three specimens with tricuspid atresia and six normal human hearts.
Results—There were significant modifications in the myoarchitecture of the right atrium and the left ventricle, both being noticeably hypertrophied. The middle layer of the ventricle in the abnormal hearts was thicker than in the normal hearts. The orientation of the superficial layer in the left ventricle in hearts with tricuspid atresia was irregular compared with the normal hearts. Scanning electron microscopy showed coarser endomysial sheaths and denser perimysial septa in hearts with tricuspid atresia than in normal hearts.
Conclusions—The overall architecture of the muscle fibres and its connective tissue matrix in hearts with tricuspid atresia differed from normal, probably reflecting modelling of the myocardium that is inherent to the malformation. This is in concordance with clinical observations showing deterioration in pump function of the dominant left ventricle from very early in life.

 Keywords: tricuspid atresia; congenital heart defects; connective tissue; fibrosis PMID:9922357

  20. Radiation Therapy, Paclitaxel, and Carboplatin With or Without Trastuzumab in Treating Patients With Esophageal Cancer

    ClinicalTrials.gov

    2016-11-02

    Adenocarcinoma of the Gastroesophageal Junction; Esophageal Adenocarcinoma; Stage IB Esophageal Cancer; Stage IIA Esophageal Cancer; Stage IIB Esophageal Cancer; Stage IIIA Esophageal Cancer; Stage IIIB Esophageal Cancer

  1. [Minimal Change Esophagitis].

    PubMed

    Ryu, Han Seung; Choi, Suck Chei

    2016-01-25

    Gastroesophageal reflux disease (GERD) is defined as a condition which develops when the reflux of gastric contents causes troublesome symptoms and long-term complications. GERD can be divided into erosive reflux disease and non-erosive reflux disease based on endoscopic findings defined by the presence of mucosal break. The Los Angeles classification excludes minimal changes as an evidence of reflux esophagitis because of poor interobserver agreement. In the Asian literature, minimal changes are considered as one of the endoscopic findings of reflux esophagitis, but the clinical significance is still controversial. Minimal change esophagitis is recognized quite frequently among patients with GERD and many endoscopists recognize such findings in their clinical practice. This review is intended to clarify the definition of minimal change esophagitis and their histology, interobserver agreement, and symptom association with GERD.

  2. Imaging of esophageal cancer

    PubMed Central

    Iyer, R; DuBrow, R

    2004-01-01

    Esophageal cancer is a relatively uncommon gastrointestinal malignancy but carries a poor prognosis unless it is of early stage and can be surgically resected for cure. Resectability is determined by the stage of disease at diagnosis and therefore accurate staging is of importance in patients diagnosed with esophageal cancer. Imaging studies that play a role in the evaluation of esophageal cancer include barium studies, computed tomography, endoscopic ultrasound and positron emission tomography. Imaging provides important information regarding the local extent and any distant spread of disease, which in turn helps in determining optimal management for these patients. This review discusses the imaging findings that may be encountered with various imaging modalities in the diagnosis, staging and follow-up of esophageal cancer. PMID:18250021

  3. [Tricuspid atresia. Clinical course in 120 children].

    PubMed

    Rosado-Buzzo, A A; Santamaría-Díaz, H; Gómez-Gómez, M; Alva-Espinosa, C; Maulen-Radovan, X; Palacios-Macedo, X

    1987-01-01

    We describe the clinical course of 120 children with tricuspid atresia (TA) attended in the Hospital de Cardiología y Neumología, "Dr. Luis Méndez", del Centro Médico Nacional. There were 61 males and 59 females. The age of presentation was in 79 newborn babies, seventeen between one and six months old, 20 between six and 24 months old, and four with two or more years old. The clinical picture was hypoxic spells in 89% and congestive heart failure in the others. The chest film showed cardiomegaly in 85%, with diminished pulmonary flow in 48%, increased flow in 27.5%, and normal in 9%. The electrocardiogram with superior left axis deviation in 94%, right atrial hypertrophy in 58%, left atrial hypertrophy in 47.5% and left ventricular hypertrophy in 96%. TA was classified as type I in 103 children, type Ic in 70, Ib in 27 and Ia in six, and type II in seventeen children, with eight IIc, six IIb and three IIa. In 44 the management was medical, 63 underwent systemic-pulmonary anastomosis, 37 of them with Blalock-Taussig shunt and Fontan procedure in thirteen children. There were 21 deaths. This survey is compared with the literature and from this point we make management recommendations.

  4. Incidence of hepatotropic viruses in biliary atresia.

    PubMed

    Rauschenfels, Stefan; Krassmann, Miriam; Al-Masri, Ahmed N; Verhagen, Willem; Leonhardt, Johannes; Kuebler, Joachim F; Petersen, Claus

    2009-04-01

    Biliary atresia (BA) is the most frequent indication for paediatric liver transplantation. We tested the hypothesis of a viral aetiology of this disease by screening liver samples of a large number of BA patients for the common human hepatotropic viruses. Moreover, we correlated our findings to the expression of Mx protein, which has been shown to be significantly up-regulated during viral infections. Seventy-four liver biopsies (taken during Kasai portoenterostomy) were tested by polymerase chain reaction (PCR) for DNA viruses (herpes simplex virus [HSV], Epstein-Barr virus [EBV], varicella zoster virus [VZV], cytomegalovirus [CMV], adenovirus, parvovirus B19 and polyoma BK) and RNA viruses (enteroviruses, rotavirus and reovirus 3). Mx protein expression was assessed by immunohistochemistry. Virus DNA/RNA was found in less than half of the biopsies (8/74 CMV, 1/74 adenovirus; 21/64 reovirus, 1/64 enterovirus). A limited number presented with double infection. Patients that had detectable viral RNA/DNA in their liver biopsies were significantly older than virus-free patients (P = 0.037). The majority (54/59) of the liver biopsies showed expression of Mx proteins in hepatocytes, bile ducts and epithelium. Our data suggest that the known hepatotropic viruses do not play a major role in the aetiology and progression of BA. Their incidence appears to be, rather, a secondary phenomenon. Nonetheless, the inflammatory response in the livers of BA patients mimics that observed during viral infections.

  5. Biliary atresia: Clinical advances and perspectives.

    PubMed

    Nizery, Laure; Chardot, Christophe; Sissaoui, Samira; Capito, Carmen; Henrion-Caude, Alexandra; Debray, Dominique; Girard, Muriel

    2016-06-01

    Biliary atresia (BA) is a rare and severe inflammatory and obliterative cholangiopathy that affects both extra- and intrahepatic bile ducts. BA symptoms occur shortly after birth with jaundice, pale stools and dark urines. The prognosis of BA has dramatically changed in the last decades: before the Kasai operation most BA patients died, while nowadays with the sequential treatment with Kasai operation±liver transplantation BA patient survival is close to 90%. Early diagnosis is very important since the chances of success of the Kasai procedure decrease with time. The causes of BA remain actually unknown but several mechanisms including genetic and immune dysregulation may probably lead to the obliterative cholangiopathy. Current research focuses on the identification of blood or liver factors linked to the pathogenesis of BA that could become therapeutic targets and avoid the need for liver transplantation. No similar disease leading to total obstruction of the biliary tree exists in older children or adults. But understanding the physiopathology of BA may highlight the mechanisms of other destructive cholangiopathies, such as sclerosing cholangitis. PMID:26775892

  6. [Eosinophilic esophagitis: update 2012].

    PubMed

    Jo, Yunju

    2012-07-01

    Eosinophilic esophagitis (EoE) with adults, as a new disease emerging during the last decade, is a clinicopathologic disorder of the esophagus characterized by a dense esophageal eosinophilic infiltration and typical esophageal symptoms. As numerous studies about EoE had been reported during last several years, updated consensus of EoE was reported in July 2011. The conceptual definition of EoE is coming. EoE is defined as a chronic, immune/antigen-mediated esophageal disease characterized clinically by symptoms related to esophageal dysfunction and histologically by eosinophil-predominat inflammation. Other important addition is genotyping feature that implicates thymic stromal lymphopoietin genes or filagrrin as EoE susceptibility genes. The majority of patients has the concurrent allergic disease, especially food or aeroallergen sensitization. Main therapeutic options include topical steroids and dietary modification. Recent issues of EoE include a new concept for proton pump inhibitor-responsive esophageal eosinophilia that it should be excluded to diagnose EoE.

  7. Recurrent pneumothorax associated with bronchial atresia: report of a case.

    PubMed

    Tanaka, Kazuhisa; Suzuki, Hidemi; Nakajima, Takahiro; Tagawa, Tetsuzo; Iwata, Takekazu; Mizobuchi, Teruaki; Yoshida, Shigetoshi; Yoshino, Ichiro

    2015-10-01

    We herein report a case of recurrent pneumothorax associated with congenital bronchial atresia. A 26-year-old male presented with chest pain. Chest roentgenograms showed left pneumothorax, a left apical bulla and an area of hyperlucency in the left upper lung field, and chest computed tomography revealed a discontinuation of the left superior bronchus. Additionally, both ventilation and perfusion scintigraphy showed a defect in the left superior segment. A thoracoscopy-assisted left superior segmentectomy was performed, and a pathological examination indicated left superior segmental bronchial atresia, which might have predisposed the peripheral lung to emphysematous conditions. No relapse was observed 6 months after the operation. Although this entity is rare, congenital bronchial atresia should be considered in the differential diagnosis when a patient has suffered from a recurrent spontaneous pneumothorax.

  8. Imaging findings of bronchial atresia in fetuses, neonates and infants.

    PubMed

    Alamo, Leonor; Vial, Yvan; Gengler, Carole; Meuli, Reto

    2016-03-01

    Congenital lung malformations are increasingly detected before birth. However, bronchial atresia is rarely identified in utero and not always recognized in neonates. There are two types of atresia: 1) proximal, located at the level of the mainstem or the proximal lobar bronchi, which is extremely rare and usually lethal during pregnancy, causing a tremendous volume increase of the distal involved lung with secondary hypoplasia of the normal lung, and 2) peripheral, located at the segmental/subsegmental bronchial level, which may present as an isolated lesion or as part of a complex congenital malformation. Prenatal findings are mostly nonspecific. Postnatal exams show overinflated lung areas and focal bronchial dilations. The typical fluid-filled bronchoceles are not always observed in neonates but develop progressively in the first months of life. This pictorial essay describes the spectrum of imaging findings of bronchial atresia in fetuses, neonates and infants. PMID:26646151

  9. [Congenital nasal obstruction due to choanal atresia: case series].

    PubMed

    Zanetta, Adrián; Rodríguez, Hugo; Quiroga, Víctor; Cuestas, Giselle; Tiscornia, Carlos

    2012-04-01

    Nasal obstruction in neonates is a potential fatal condition because of their exclusive nasal breathing. The most common congenital causes include choanal atresia, dermoid cyst, glioma and encephalocele. Choanal atresia is the most common congenital nasal anomaly. When bilateral, it presents with respiratory distress at birth. Unilateral atresia is manifested by respiratory failure and unilateral rhinorrhea, and may go along unnoticed. Diagnosis is suspected in the absence of airfow in the nasal cavity and for the inability to advance a nasogastric tube. Diagnosis is confirmed by endoscopic examination and computed tomography. The definitive treatment is surgical, and there are different techniques and surgical approaches. We describe our experience with 7 patients with this disease, successfully treated by transnasal endoscopic technique. PMID:22451289

  10. Retrospective Study of a Series of Choanal Atresia Patients

    PubMed Central

    Manica, Denise; Schweiger, Cláudia; Netto, Cátia C Saleh; Kuhl, Gabriel

    2013-01-01

    Introduction Although it has been more than 250 years since the first description of choanal atresia (CA), there are still doubts about this abnormality. The differences between unilateral and bilateral forms are seldom discussed. Objectives Aggregate data from patients diagnosed with CA, grouping patients with unilateral and bilateral forms. Methods Retrospective study. Results Eighteen patients were included: 12 (66.6%) presented bilateral atresia, of which 77.8% were mixed bony-membranous type and 22.2% were pure bony type. From the 12 patients with bilateral atresia, 10 presented related malformations, 3 of whom had CHARGE syndrome (coloboma, heart defects, choanal atresia, retardation of growth and development, genitourinary problems, ear abnormalities). From the remaining 6 patients with unilateral atresia, only 2 showed malformations, 1 renal and 1 cardiac. All patients with unilateral atresia needed only 1 surgical procedure, and patients with the bilateral form needed a median of 2.85 interventions (p = 0.003). The median age of surgical procedure in the unilateral group was 6 years, ranging from 6 months to 18 years, and in the bilateral group was 25 days, ranging from 6 days to 6 years (p = 0.003). The median interval between diagnosis and surgery was 9 months in the unilateral group, ranging from 1 month to 18 years, and in the bilateral group was 1 day, ranging from 1 day to 2 months (p = 0.001). Discussion and Conclusions Success rates with the endoscopic approach vary from 62 to 100%. Nonetheless, most of these reports present results without considering the number of compromised sides. In our opinion, unilateral and bilateral cases involve distinct patients (taking into account the related malformations), have diverging clinical presentations, and show discrepant restenosis rates and therefore could be considered in different groups of analysis. PMID:25992054

  11. Clinical Implications and Pathogenesis of Esophageal Remodeling in Eosinophilic Esophagitis

    PubMed Central

    Hirano, Ikuo; Aceves, Seema S.

    2014-01-01

    In eosinophilic esophagitis (EoE), remodeling changes are manifest histologically in both the epithelium as well as in the subepithelium where lamina propria (LP) fibrosis, expansion of the muscularis propria and increased vascularity occur. The major clinical symptoms and complications of EoE are largely consequences of esophageal remodeling. Important mediators of the process include IL-5, IL-13, TGFβ1, mast cells, fibroblasts and eosinophils. Methods to detect remodeling effects include upper endoscopy, histopathology, barium esophagram, endoscopic ultrasonography, esophageal manometry, and functional luminal imaging. These modalities provide evidence of organ dysfunction that include focal and diffuse esophageal strictures, expansion of the mucosa and subepithelium, esophageal motor abnormalities and reduced esophageal distensibility. Complications of food impaction and perforations of the esophageal wall have been associated with reduction in esophageal caliber and increased esophageal mural stiffness. The therapeutic benefits of topical corticosteroids and elimination diet therapy in resolving mucosal eosinophilic inflammation of the esophagus are evident. Available therapies, however, have demonstrated variable ability to reverse existing remodeling changes of the esophagus. Systemic therapies that include novel, targeted biologic agents have the potential of addressing subepithelial remodeling. Esophageal dilation remains a useful, adjunctive therapeutic maneuver in symptomatic adults with esophageal stricture. As novel treatments emerge, it is essential that therapeutic endpoints account for the fundamental contributions of esophageal remodeling to overall disease activity. PMID:24813517

  12. Atresia of the right atrioventricular orifice with atrioventricular concordance.

    PubMed Central

    Dickinson, D F; Wilkinson, J L; Smith, A; Anderson, R H

    1979-01-01

    Three hearts are described in which a fibrous membrane was interposed between the right atrium and a formed but hypoplastic right ventricle, which possessed recognisable inlet, trabecular, and infundibular components. In these hearts the distribution of the conducting tissue was as expected for concordant atrioventricular connections, and contrasts with that seen in the classical type of 'tricuspid atresia'. The distinctive morphological and histological features of these specimens lend further support to our view that the majority of cases of atresia of the right atrioventricular orifice should be regarded as coming within the designation of 'the univentricular heart'. Images PMID:475940

  13. Congenital biliary atresia and jaundice in lambs and calves.

    PubMed

    Harper, P; Plant, J W; Unger, D B

    1990-01-01

    An outbreak of congenital biliary atresia and jaundice is described, in which approximately 300 crossbred lambs and 9 crossbred calves died. The affected animals failed to thrive, developed jaundice and white scours and died within 4 weeks of birth. A common feature of this outbreak and a similar occurrence 24 years previously was the grazing of plants growing on the exposed silt foreshores of Burrinjuck Dam by ewes and cows in the early stages of pregnancy. Epidemiological and pathological findings suggested that a toxic insult to the foetus in early gestation caused choledysgenesis and biliary atresia, leading to diffuse, subacute to chronic cholangiohepatopathy and cirrhosis.

  14. Achalasia and Esophageal Motility Disorders

    MedlinePlus

    ... esophagus, and chest wall Lung Cancer Esophageal Cancer Gastroesophageal Reflux Disease Barrett’s Esophagus Chest Wall Tumors Mediastinal Tumors ... Section Navigation Select Topic Lung Cancer Esophageal Cancer Gastroesophageal Reflux Disease Barrett’s Esophagus Chest Wall Tumors Mediastinal Tumors ...

  15. General Information about Esophageal Cancer

    MedlinePlus

    ... Research Esophageal Cancer Treatment (PDQ®)–Patient Version General Information About Esophageal Cancer Go to Health Professional Version ... the PDQ Adult Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  16. Rhizomelia with anal atresia and anophthalmia: a new syndrome?

    PubMed

    Ozlem, Giray; Elçin, Bora; Ayfer, Ulgenalp; Oguz, Ateş; Erdener, Ozer; Derya, Erçal

    2008-01-01

    We report a newborn who presented with an unreported combination of anophthalmia, anal atresia, rhizomelia, dextrocardia and corpus callosum agenesis. Clinical and postmortem findings did not match any previously described syndromes with the type of anomalies seen in this patient. We suggested that this combination of congenital malformations might represent a new syndrome.

  17. Colonic atresia in cattle: A prospective study of 43 cases

    PubMed Central

    Ducharme, Norm G.; Arighi, Mimi; Horney, F. Don; Barker, Ian K.; Livesey, Michael A.; Hurtig, Mark H.; Johnson, Roger P.

    1988-01-01

    This prospective study was initiated to document the success rate obtained in the treatment of colonic atresia in calves, identify factors that influence survival rate, and to report the histopathological appearance of the proximal blind end of the ascending colon. Forty-three calves with intestinal obstruction due to colonic atresia were admitted to the Ontario Veterinary College between September 1982 and May 1986. Parameters recorded prospectively in this study included age, breed, sex, history, vital signs, acid-base and electrolyte status, location of intestinal atresia, medical and surgical management, and outcome. The typical history and clinical signs included failure to pass meconium or feces, decreased appetite, and progressive depression and abdominal distension. The most common site of colonic atresia was the midportion of the spiral loop of the ascending colon (n = 25). Of the 43 calves, three (7%) were euthanized at surgery, 21 (49%) died in the hospital, and 19 (44%) survived and were discharged from the hospital. Four of the surviving calves died subsequent to discharge giving an overall long-term (mean 15.9 months) survival rate of 35%. No significant risk factors were identified, although experienced surgeons showed a trend towards increased survival rate. ImagesFigure 2.Figure 3.Figure 4.Figure 5. PMID:17423141

  18. Theca interna: the other side of bovine follicular atresia.

    PubMed

    Clark, Leigh J; Irving-Rodgers, Helen F; Dharmarajan, Arun M; Rodgers, Raymond J

    2004-10-01

    Currently, histological classifications of ovarian follicular atresia are almost exclusively based on the morphology of the membrana granulosa without reference to the theca interna. Atresia in the bovine small antral ovarian follicle has been redefined into antral or basal atresia where cell death commences initially within antral or basal regions of the membrana granulosa, respectively. To examine cell death in the theca interna in the two types of atretic follicles, bovine ovaries were collected and processed for immunohistochemistry and light microscopy. Follicles were classified as healthy, antral atretic, or basal atretic. Follicle diameter was recorded and sections stained with lectin from Bandeiraea simplicifolia to identify endothelial cells or with an antibody to cytochrome P450 cholesterol side-chain cleavage to identify steroidogenic cells and combined with TUNEL labeling to identify dead cells. The numerical density of steroidogenic cells within the theca interna was significantly reduced (P < 0.001) in basal atretic follicles in comparison with other follicles. Cell death was greater in both endothelial cells (P < 0.05) and steroidogenic cells (P < 0.01) of the theca interna of basal atretic follicles compared with healthy and antral atretic follicles. Thus, we conclude that the theca interna is susceptible to cell death early in atresia, particularly in basal atretic follicles. PMID:15175236

  19. Esophageal phytobezoar in a horse.

    PubMed

    MacDonald, M H; Richardson, D W; Morse, C C

    1987-12-01

    A 23-year-old Thoroughbred stallion was admitted to the hospital for treatment of acute esophageal obstruction. Clinical examination and contrast radiography confirmed the presence of an esophageal obstruction. The horse was euthanatized, and examination revealed a bolus of feed material occluding the esophageal lumen 6 cm caudal to the thoracic inlet, with underlying necrosis of the esophageal mucosa. A large pulsion diverticulum was identified in the caudocervical portion of the esophagus. Apparently, the phytobezoar was formed within the esophageal diverticulum and subsequently became dislodged, occluding the esophagus.

  20. [Esophageal motility disorders].

    PubMed

    Dughera, L; Battaglia, E; Emanuelli, G

    2001-09-01

    Esophageal motility abnormalities are usually diagnosed when esophageal manometry is performed in patients with unexplained non-cardiac chest pain, non obstructive dysphagia or as a part of the preoperative evaluation for surgery of gastroesophageal reflux. Classification of these abnormalities has been a subject of controversy. These esophageal contraction abnormalities can be separated manometrically from the motor pattern seen in normal subjects, however, their clinical relevance is still unclear and debated. Many patients demonstrate motility abnormalities in the manometry laboratories, but may lack correlation with their presenting symptoms. Medical treatment can decrease symptoms particularly chest pain or acid reflux but there is no significant changes in the manometric patterns. Such motor abnormalities may not reflect a true disease state, but they could be markers of other abnormalities and they can modify the initial manometric findings in time.

  1. Minimally invasive surgery for esophageal achalasia

    PubMed Central

    Chen, Huan-Wen

    2016-01-01

    Esophageal achalasia is due to the esophagus of neuromuscular dysfunction caused by esophageal functional disease. Its main feature is the lack of esophageal peristalsis, the lower esophageal sphincter pressure and to reduce the swallow’s relaxation response. Lower esophageal muscular dissection is one of the main ways to treat esophageal achalasia. At present, the period of muscular layer under the thoracoscope esophagus dissection is one of the treatment of esophageal achalasia. Combined with our experience in minimally invasive esophageal surgery, to improved incision and operation procedure, and adopts the model of the complete period of muscular layer under the thoracoscope esophagus dissection in the treatment of esophageal achalasia. PMID:27499977

  2. Assessing esophageal dysphagia.

    PubMed

    Kruger, Danielle

    2014-05-01

    Dysphagia, or difficulty swallowing, is a common problem. Although most cases are attributable to benign disease processes, dysphagia is also a key symptom in several malignancies, making it an important symptom to evaluate. The differential diagnosis of dysphagia requires an understanding of deglutition, in particular the oropharyngeal versus esophageal stages. Stroke is the leading cause of oropharyngeal dysphagia, which is common in older adults and frequently presents as part of a broader complex of clinical manifestations. In esophageal dysphagia, difficulty swallowing is often the main complaint and is caused by localized neuromuscular disorders or obstructive lesions.

  3. Health Related Quality of Life in Patients with Biliary Atresia Surviving with their Native Liver

    PubMed Central

    Sundaram, Shikha S.; Alonso, Estella M.; Haber, Barbara; Magee, John C.; Fredericks, Emily; Kamath, Binita; Kerkar, Nanda; Rosenthal, Philip; Shepherd, Ross; Limbers, Christine; Varni, James W.; Robuck, Patricia; Sokol, Ronald J.; Liver, Childhood

    2014-01-01

    Objectives To quantify health related quality of life (HRQOL) of patients with biliary atresia with their native livers and compare them with healthy children and patients with biliary atresia post-liver transplant (LT) and to examine the relationship between HRQOL and medical variables. Study design A cross-sectional HRQOL study of patients with biliary atresia with their native livers (ages 2-25 years) was conducted and compared with healthy and post-LT biliary atresia samples using PedsQL™ 4.0 child self and parent proxy reports, a validated measure of physical/psychosocial functioning. Results 221 patients with biliary atresia with native livers (54% female, 67% white) were studied. patient self and parent proxy reports showed significantly poorer HRQOL than healthy children across all domains (p < 0.001), particularly in emotional and psychosocial functioning. Child self and parent proxy HRQOL scores from patients with biliary atresia with their native livers and post-LT biliary atresia were similar across all domains (p=NS). Child self and parent proxy reports showed moderate agreement across all scales, except social functioning (poor to fair agreement). On multivariate regression analysis, black race and elevated total bilirubin were associated with lower Total and Psychosocial HRQOL summary scores. Conclusions HRQOL in patients with biliary atresia with their native livers is significantly poorer than healthy and similar to post-LT biliary atresia children. These findings identify significant opportunities to optimize the overall health of patients with biliary atresia. PMID:23746866

  4. Molecular Phenotyping in Predicting Response in Patients With Stage IB-III Esophageal Cancer Receiving Combination Chemotherapy

    ClinicalTrials.gov

    2015-12-18

    Stage IB Esophageal Adenocarcinoma; Stage IIA Esophageal Adenocarcinoma; Stage IIB Esophageal Adenocarcinoma; Stage IIIA Esophageal Adenocarcinoma; Stage IIIB Esophageal Adenocarcinoma; Stage IIIC Esophageal Adenocarcinoma

  5. Clinical and etiological heterogeneity in patients with tracheo-esophageal malformations and associated anomalies.

    PubMed

    Brosens, Erwin; Ploeg, Mirjam; van Bever, Yolande; Koopmans, Anna E; IJsselstijn, Hanneke; Rottier, Robbert J; Wijnen, Rene; Tibboel, Dick; de Klein, Annelies

    2014-08-01

    Esophageal Atresia (EA) is a severe developmental defect of the foregut that presents with or without a Tracheo-Esophageal Fistula (TEF). The prevalence of EA/TEF over time and around the world has been relatively stable. EA/TEF is manifested in a broad spectrum of anomalies: in some patients it manifests as an isolated atresia or fistula, but in over half it affects several organ systems. While the associated malformations are often those of the VACTERL spectrum (Vertebral, Anorectal, Cardiac, Tracheo-Esophageal, Renal and Limb), many patients are affected by other malformations, such as microcephaly, micrognathia, pyloric stenosis, duodenal atresia, a single umbilical artery, and anomalies of the genitourinary, respiratory and gastrointestinal systems. Though EA/TEF is a genetically heterogeneous condition, recurrent genes and loci are sometimes affected. Tracheo-Esophageal (TE) defects are in fact a variable feature in several known single gene disorders and in patients with specific recurrent Copy Number Variations and structural chromosomal aberrations. At present, a causal genetic aberration can be identified in 11-12% of patients. In most, EA/TEF is a sporadic finding; the familial recurrence rate is low (1%). As this suggests that epigenetic and environmental factors also contribute to the disease, non-syndromic EA/TEF is generally believed to be a multifactorial condition. Several population-based studies and case reports describe a wide range of associated risks, including age, diabetes, drug use, herbicides, smoking and fetal alcohol exposure. The phenotypical and genetic heterogeneity seen in EA/TEF patients indicates not one underlying cause, but several. Unraveling the complex multifactorial and heterogeneous etiology of EA/TEF and associated features will require large cohorts of patients. Combined statistical analysis of component findings, genome sequencing, and genome wide association studies will elucidate new causal genetic defects and

  6. Esophagitis in Adolescents.

    PubMed

    Putnam, Philip E

    2016-01-01

    Esophagitis is the end result of a variety of insults to epithelial homeostasis. Eosinophilic esophagitis is a manifestation of non-IgE-mediated food allergy that most commonly affects the esophagus of males who have other atopic phenomena. Reflux esophagitis reflects repeated exposure to acidic gastric contents because of failure of the normal protections afforded by the LES. Because certain histologic features can be present in either condition, endoscopic biopsy alone does not distinguish them. Their symptoms overlap, but the treatment options are very different, such that making a formal diagnosis by following consensus guidelines is essential. A treatment protocol designed to manage the inflammation by controlling the provocative factors (acid for GERD and food antigens for EoE) or suppressing the inflammation (ie, topical steroids for EoE) should result in normalization of the mucosa and resolution of symptoms. Eosinophilic esophagitis is a chronic condition that rarely remits spontaneously, so any therapeutic modality will need to be continued indefinitely. PMID:27363230

  7. Bonebridge Implantation for Conductive Hearing Loss in a Patient with Oval Window Atresia.

    PubMed

    Kim, Minbum

    2015-08-01

    The occurrence of oval window atresia is a rare anomaly with conductive hearing loss. Traditional atresia surgeries involve challenging surgical techniques with risks of irreversible inner ear damage. Recent reports on Bonebridge (Medel, Innsbruck, Austria), a novel implantable bone conduction hearing aid system, assert that the device is safe and effective for conductive hearing loss. We present a case of Bonebridge implantation in an eight-year-old girl with bilateral oval window atresia. PMID:26381009

  8. Appearances are Deceptive - Passing a Nasogastric Tube does Not Always Rule Out Oesophageal Atresia.

    PubMed

    Kumar, Manish; Thomas, Niranjan

    2016-04-01

    Oesophageal atresia/trachea-Oesophageal fistula is commonly diagnosed in the newborn period by inability to pass a nasogastric tube (NGT). We present the instance of a newborn baby where the diagnosis of oesophageal atresia was delayed because of an apparent successful passage of nasogastric tube to the stomach. Failure to reinsert the NGT raised the suspicion of oesophageal atresia which was confirmed by contrast study showing blind upper oesophageal pouch.

  9. Choanal atresia in a patient with the deletion (9p) syndrome

    SciTech Connect

    Shashi, V.; Golden, W.L.; Fryburg, J.S.

    1994-01-01

    The authors report on a child with choanal atresia and deletion 9p. A review of the literature documented one previous instance of choanal atresia in a patient with del(9p). Choanal atresia may be part of the spectrum of malformations in the deletion (9p) syndrome and its presence should prompt a search for this particular deletion as part of the differential diagnosis. 9 refs., 3 figs.

  10. Bonebridge Implantation for Conductive Hearing Loss in a Patient with Oval Window Atresia.

    PubMed

    Kim, Minbum

    2015-08-01

    The occurrence of oval window atresia is a rare anomaly with conductive hearing loss. Traditional atresia surgeries involve challenging surgical techniques with risks of irreversible inner ear damage. Recent reports on Bonebridge (Medel, Innsbruck, Austria), a novel implantable bone conduction hearing aid system, assert that the device is safe and effective for conductive hearing loss. We present a case of Bonebridge implantation in an eight-year-old girl with bilateral oval window atresia.

  11. Effect of gastroesophageal reflux on esophageal speech.

    PubMed

    Mathis, J G; Lehman, G A; Shanks, J C; Blom, E D; Brunelle, R L

    1983-12-01

    Gastroesophageal reflux has been incriminated as a factor-inhibiting acquisition of esophageal speech after laryngectomy. Fourteen proficient esophageal speakers and 10 nonproficient speakers underwent esophageal manometry, esophageal pH probe testing, and Bernstein acid perfusion testing. Additionally, 175 laryngectomized members of Lost Chord Clubs answered mailed questionnaires about the frequency of reflux symptoms. Nonproficient and proficient esophageal speakers had a similar frequency of gastroesophageal reflux by pH probe testing, esophageal mucosal acid sensitivity by Bernstein testing, lower esophageal sphincter pressures, and gastroesophageal reflux symptoms. Gastroesophageal reflux does not appear to be a major factor in preventing esophageal speech.

  12. Anaesthesia for biliary atresia and hepatectomy in paediatrics

    PubMed Central

    Jacob, Rebecca

    2012-01-01

    The scope of this article precludes an ‘in depth’ description of all liver problems and I will limit this review to anaesthesia for biliary atresia — a common hepatic problem in the very young — and partial hepatectomy in older children. I will not be discussing the problems of anaesthetising children with hepatitis, cirrhosis, congenital storage diseases or liver failure. Extrahepatic biliary obstruction is an obliterative cholangiopathy of infancy which is fatal if untreated. Diagnosis involves exclusion of other causes of neonatal jaundice and treatment involves a hepatico portoenterostomy carried out at the earliest. This is a review of current concepts in anaesthesia and postoperative management of neonates with extrahepatic biliary atresia. Anaesthesia for hepatic resection has seen great changes in recent times with the improvement in surgical techniques, technology and a better understanding of the underlying physiology. These are reviewed along with the problems of postoperative pain management. PMID:23293387

  13. Two Cases of Malleostapedotomy in Congenital Oval Window Atresia

    PubMed Central

    Ahn, Sang Hyeon; Kim, Da Hee; Choi, Jae Young

    2013-01-01

    Congenital anomaly of the oval window with an abnormal facial nerve course is an uncommon embryological defect, which is related to the underdevelopment of second branchial arch derivatives. Some treatments for improving hearing levels are available; these include hearing aids, vestibulotomy, neo-oval window formation, and stapes surgeries, including incudostapedotomy and malleostapedotomy. However, surgery for congenital anomalies of the oval window has rarely been described, usually in very small series of patients. We describe two cases of congenital anomalies of the oval window with aberrant facial nerve courses. One was a 40-year-old male diagnosed with unilateral congenital oval window atresia; the other was a 10-year-old male diagnosed with bilateral congenital oval window atresia. We also describe the clinical manifestations and treatment outcomes of malleostapedotomy for congenital anomalies of the oval window with aberrant facial nerve courses. PMID:24653925

  14. Eosinophilic esophagitis: current treatment.

    PubMed

    Redd, Matthew; Schey, Ron

    2013-03-01

    Eosinophilic esophagitis (EoE) is a relatively new entity with a significant amount of increased recognition over the last decade. The mainstay treatments of EoE are designed to eliminate the causative allergens or to reduce their effects on the esophageal mucosa. Common treatments include dietary modification, proton pump inhibitors, systemic and topical corticosteroids, and endoscopic treatments. As the pathogenesis of EoE is explored, new and novel treatments are being studied that target specific pathways and chemokines identified in as precipitating agents of EoE. This is a rapidly evolving field with significant ongoing research and clinical studies. Our review will therefore focus on current and novel treatment approaches to the disease.

  15. Esophageal carcinoma: CT findings

    SciTech Connect

    Quint, L.E.; Glazer, G.M.; Orringer, M.B.; Gross, B.H.

    1985-04-01

    Preoperative CT scans of 33 patients with esophageal cancer were reviewed to assess staging accuracy and define the role of CT in patients being considered for transhiatal blunt esophagectomy. Surgical and pathological verification was obtained in all cases. Only 13 tumors were staged correctly according to the TNM classification. In addition, CT was not useful in assessing resectability because of its low accuracy in evaluating aortic invasion and the fact that few patients had tracheobronchial or aortic invasion or hepatic metastases at presentation.

  16. Hypnotherapy for Esophageal Disorders.

    PubMed

    Riehl, Megan E; Keefer, Laurie

    2015-07-01

    Hypnotherapy is an evidence based intervention for the treatment of functional bowel disorders, particularly irritable bowel syndrome. While similar in pathophysiology, less is known about the utility of hypnotherapy in the upper gastrointestinal tract. Esophageal disorders, most of which are functional in nature, cause painful and uncomfortable symptoms that impact patient quality of life and are difficult to treat from a medical perspective. After a thorough medical workup and a failed trial of proton pump inhibitor therapy, options for treatment are significantly limited. While the pathophysiology is likely multifactorial, two critical factors are believed to drive esophageal symptoms--visceral hypersensitivity and symptom hypervigilance. The goal of esophageal directed hypnotherapy is to promote a deep state of relaxation with focused attention allowing the patient to learn to modulate physiological sensations and symptoms that are not easily addressed with conventional medical intervention. Currently, the use of hypnosis is suitable for dysphagia, globus, functional chest pain/non-cardiac chest pain, dyspepsia, and functional heartburn. In this article the authors will provide a rationale for the use of hypnosis in these disorders, presenting the science whenever available, describing their approach with these patients, and sharing a case study representing a successful outcome. PMID:26046715

  17. Staging Early Esophageal Cancer.

    PubMed

    Old, O J; Isabelle, M; Barr, H

    2016-01-01

    Staging esophageal cancer provides a standardized measure of the extent of disease that can be used to inform decisions about therapy and guide prognosis. For esophageal cancer, the treatment pathways vary greatly depending on stage of disease, and accurate staging is therefore crucial in ensuring the optimal therapy for each patient. For early esophageal cancer (T1 lesions), endoscopic resection can be curative and simultaneously gives accurate staging of depth of invasion. For tumors invading the submucosa or more advanced disease, comprehensive investigation is required to accurately stage the tumor and assess suitability for curative resection. A combined imaging approach of computed tomography (CT), positron emission tomography (PET), and endoscopic ultrasound (EUS) offers complementary diagnostic information and gives the greatest chance of accurate staging. Staging laparoscopy can identify peritoneal disease and small superficial liver lesions that could be missed on CT or PET, and alters management in up to 20 % of patients. Optical diagnostic techniques offer the prospect of further extending the possibilities of endoscopic staging in real time. Optical coherence tomography can image superficial lesions and could provide information on depth of invasion for these lesions. Real-time lymph node analysis using optical diagnostics such as Raman spectroscopy could be used to support immediate endoscopic therapy without waiting for results of cytology or further investigations. PMID:27573772

  18. [Endoscopic Therapy for Esophageal Cancer].

    PubMed

    Sakai, Makoto; Kuwano, Hiroyuki

    2016-07-01

    Endoscopic treatment for esophageal neoplasms includes endoscopic resection, argon plasma coagulation(APC), photodynamic therapy( PDT) and stent placement. Endoscopic resection is widely used as an effective, less invasive treatment for superficial esophageal carcinoma in Japan. APC is considered to be safe and effective treatment for superficial esophageal carcinoma which cannot be resected endoscopically because of severe comorbidities, as well as for local recurrence after endoscopic resection or chemoradiotherapy. PDT is thought to be an effective option as salvage treatment for local failure after chemoradiotherapy. Stent placement mainly using self-expanding metallic stents have been used as a minimally invasive and effective modality for the palliative treatment of malignant esophageal obstruction. Endoscopic treatment is expected to have more important role in the treatment of esophageal neoplasms in the future. PMID:27440040

  19. [The bubble-blowing neonate: a red flag for oesophageal atresia].

    PubMed

    Zwaan, Esther; Meij-de Vries, Annebeth; van Deutekom, Arend W; van der Steeg, Alida F W; Sleeboom, Christien; Heij, Hugo A

    2013-01-01

    When a newborn develops feeding difficulties, is blowing bubbles or has excessive saliva, it is important to consider the diagnosis of oesophageal atresia. Prenatal detection of oesophageal atresia is difficult. Postnatal bubble blowing, resulting from the inability to swallow excessive oropharyngeal secretions, is pathognomonic. These symptoms should trigger the midwife or physician to consider this diagnosis. We present three cases to illustrate the difficulties of early recognition and the consequences of a late detection of this condition. The first and third cases show that a delayed diagnosis of oesophageal atresia can lead to dangerous situations. Our second case illustrates that not every poorly drinking neonate has oesophageal atresia, and that this diagnosis may be rejected if the physician is able to pass a nasogastric tube. In every neonate who is unable to drink, we advise attempting to pass a nasogastric tube and taking a chest x-ray to distinguish between oesophageal atresia and other causes of feeding problems.

  20. Gastro-esophageal reflux time parameters and esophagitis in children

    SciTech Connect

    Baulieu, F.; Baulieu, J.; Maurage, C.; Casset, D.; Itti, R.

    1985-05-01

    The aim of this work was to study the correlation between the reflux timing and the presence of esophagitis, an inconstant but serious complication of gastro-esophageal reflux (GER). The hypothesis was that reflux occurring late after meal can be incriminated more than early reflux in esophagitis genesis. 32 children with GER (mean age = 10.5 months, 2 to 30 months) had esophagoscopy and scintigraphy in the same week. The children were classified in two groups according to esophagoscopy: group 1 (n = 18) no esophagitis, group 2 (n = 14) esophaqgitis. The scintigraphy involved the ingestion of 0.5 mCi Tc-99m sulfur colloid milk mixture, followed by esophageal and gastric activity recording (one image per minute for 1 hour). The reflux was assessed from contrast enhanced images and esophageal time activity curves. Reflux intensity was quantitated by reflux index (Re). Mean reflux time was calculated as the mean esophageal activity peaks time (t-bar). Finally a composite parameter was calculated as the mean reflux time weighted by the relative intensity of each reflux peak (t-barw). Re was not found to be different between the two groups. t-bar was significantly higher in group 2: t-bar = 29.6 +- 3.0 mn (mean +- SD) than in group 1: t-bar = 24.5 +- 6.8 mn; rho <0.02. The difference between the two groups was enhanced by intensity weighting: group 1: t-barw = 16.6 +- 6.3 mn, group 2: t-barw = 33.5 +- 7.1 mn rho <0.001. t-barw value was not correlated to esophagitis grade. These results suggest that late reflux is more likely responsible of esophagitis.

  1. [Prevalence of erosive esophagitis and peptic esophageal strictures].

    PubMed

    Vasilevskiĭ, D I; Skurikhin, S S; Luft, A V; Mednikov, S N; Silant'ev, D S; Kulagin, V I; Dvoretskiĭ, S Iu; Bagnenko, S F

    2015-01-01

    Gastroesophageal reflux disease is a widespread among population in economically developed countries including Russia. It was analyzed the results of 34 903 endoscopic examinations of upper gastrointestinal tract in ethnically and socially homogeneous population of Leningrad region with symptoms of gastric dispepsia. Procedures were performed for the period 2007-2013. Prevalence of erosive esophagitis was 4.9%. Peptic esophageal strictures due to chronic reflux-associated inflammation were revealed in 0.2% of examined patients (3.7% of patients with erosive esophagitis). Obtained data allow to considergastroesophageal reflux disease as a socially significant problem in Russia requiring close attention and further study.

  2. Intraluminal esophageal diverticulum.

    PubMed

    Funakoshi, O; Soma, Y; Takasugi, T; Munakata, A; Yoshida, Y

    1990-02-01

    An intraluminal esophageal diverticulum (IED) is an uncommon entity defined as a double-layered mucosal pouch lying within the lumen of the esophagus. Its characteristic radiological finding is an intraluminal barium collection surrounded by a radiolucent halo. True IED, which is different from a transient radiological artifact, has not been previously reported in the international literature. This article describes the first case of true IED. Differential diagnosis between a true lesion and a transient flow artifact on barium meal is discussed. PMID:2106464

  3. Aetiology of biliary atresia: what is actually known?

    PubMed Central

    2013-01-01

    Biliary atresia (BA) is a rare disease of unknown etiology and unpredictable outcome, even when there has been timely diagnosis and exemplary surgery. It has been the commonest indication for liver transplantation during childhood for the past 20 years. Hence much clinical and basic research has been directed at elucidating the origin and pathology of BA. This review summarizes the current clinical variations of BA in humans, its occasional appearance in animals and its various manifestations in the laboratory as an experimental model. PMID:23987231

  4. Familial pulmonary atresia. Its occurrence with a ventricular septal defect.

    PubMed

    DiChiara, J A; Pieroni, D R; Gingell, R L; Bannerman, R M; Vlad, P

    1980-05-01

    To our knowledge, this is the first reported case of pulmonary atresia with a ventricular septal defect that occurred in a father and his son. A multifactorial mode of determination that resulted from an interaction between genetic predisposition and environmental factors was assumed, since the family history did not show other members with congenital heart defects. Based on the background and on information from the multifactorial theory of inheritance, a counseling plan was formulated for these parents. A similar approach is recommended for the many surgical survivors of previously uncorrectable complex congenital heart defects as they approach reproductive age.

  5. Esophageal Lipoma: A Rare Tumor

    PubMed Central

    Feldman, Jeremy; Tejerina, Manfred; Hallowell, Michael

    2012-01-01

    Esophageal lipomas are rare tumors, making up 0.4% of all digestive tract benign neoplasms. Most of these lesions are clinically silent as a result of their small size, however, the majority of lesions over 4 cm have been reported to cause dysphagia, regurgitation and/or epigastralgia. We report a case of a 53 year-old African American female who presented with dysphagia. Computed tomography of the chest and esophagram confirmed esophageal lipoma as the cause of the patient’s symptoms. Accurately diagnosing an esophageal lipoma is crucial in order to rule out potential malignant lesions, relieve patient symptoms and plan the appropriate treatment. PMID:23365708

  6. Eosinophilic gastroenteritis with esophageal involvement.

    PubMed

    Dobbins, J W; Sheahan, D G; Behar, J

    1977-06-01

    A patient with a lifelong history of asthma and hay fever was investigated because of symptoms of esophageal spasm. Esophageal biopsies revealed elongated papillae and basal zone hyperplasia of the epithelial layer with eosinophilic infiltration of the lamina propria and muscularis mucosae. There was no evidence of reflux. Small bowel biopsies revealed a flat mucosal pattern with absent or blunted villi, tall columar surface epithelium, and eosinophilic infiltration of the lamina propria. He did not respond to a gluten-free diet. This patient is thought to have eosinophilic gatroenteritis with esophageal involvement, the first such case reported.

  7. Treatment of advanced esophageal cancer

    SciTech Connect

    Kelsen, D.

    1982-12-01

    When radiation therapy is used for palliation of obstruction in patients with advanced esophageal carcinoma, an improvement in dysphagia can be expected in approximately 50% of patients. Major objective responses have rarely been quantitied but, in one study, were seen in 33% patients. Recurrence of dysphagia is usually seen within 2-6 months of treatment. Radiation toxicities and complications, even when used with palliative intent, can be substantial and include esophagitis, tracheoesophageal or esophageal-aortic fistula, mediastinitis, hemorrhage, pneumonitis, and myelosuppression. (JMT)

  8. Nuclear medicine and esophageal surgery

    SciTech Connect

    Taillefer, R.; Beauchamp, G.; Duranceau, A.C.; Lafontaine, E.

    1986-06-01

    The principal radionuclide procedures involved in the evaluation of esophageal disorders that are amenable to surgery are illustrated and briefly described. The role of the radionuclide esophagogram (RE) in the diagnosis and management of achalasia, oculopharyngeal muscular dystrophy and its complications, tracheoesophageal fistulae, pharyngeal and esophageal diverticulae, gastric transposition, and fundoplication is discussed. Detection of columnar-lined esophagus by Tc-99m pertechnetate imaging and of esophageal carcinoma by Ga-67 citrate and Tc-99m glucoheptonate studies also is presented. 37 references.

  9. Constitution and in vivo test of micro-porous tubular scaffold for esophageal tissue engineering.

    PubMed

    Hou, Lei; Jin, Jiachang; Lv, Jingjing; Chen, Ling; Zhu, Yabin; Liu, Xingyu

    2015-11-01

    Current clinical techniques in treating long-gap esophageal defects often lead to complications and high morbidity. Aiming at long-gap synthetic esophageal substitute, we had synthesized a biodegradable copolymer, poly(L-lactide-co-caprolactone) (PLLC), with low glass transition temperature. In this work, we developed a tubular PLLC porous scaffold using a self-designed tubular mold and thermal induced phase separation (TIPS) method. In order to enhance the interaction between tissue and scaffold, fibrin, a natural fibrous protein derived from blood fibrinogen, was coated on the scaffold circumferential surface. The fibrin density was measured to be 1.23 ± 0.04 mg/cm(2). Primary epithelial cell culture demonstrated the improved in vitro biocompatibility. In animal study with partial scaffold implantation, in situ mucosa regeneration was observed along the degradation of the scaffold. These indicate that fibrin incorporated PLLC scaffold can greatly improve epithelial regeneration in esophagus repair, therefore serve as a good candidate for long-term evaluation of post-implantation at excision site.

  10. Esophageal Intramural Pseudodiverticulosis and Concomitant Eosinophilic Esophagitis: A Case Series.

    PubMed

    Scaffidi, Michael A; Garg, Ankit; Ro, Brandon; Wang, Christopher; Yang, Tony T C; Plener, Ian S; Grin, Andrea; Colak, Errol; Grover, Samir C

    2016-01-01

    Background. Esophageal intramural pseudodiverticulosis (EIPD) is an idiopathic benign chronic disease characterized by flask-like outpouchings of the esophageal wall. It is unknown whether there is a genuine association between EIPD and eosinophilic esophagitis (EoE). Aims. To investigate a possible relationship between EIPD and EoE. Methods. Patients with radiographic or endoscopic evidence of pseudodiverticulosis were identified from the database at a single academic center. Cases were analyzed in three areas: clinical information, endoscopic findings, and course. Results. Sixteen cases of esophageal pseudodiverticulosis were identified. Five patients had histologic evidence of eosinophilic esophagitis. Patients with EoE had pseudodiverticula in the mid-to-distal esophagus while those with EIPD had pseudodiverticula predominantly in the proximal esophagus (p < 0.001). EoE with pseudodiverticulosis occurred in younger patients (p < 0.019). Food bolus obstructions were more common in patients with EoE and pseudodiverticulosis than in EIPD (p < 0.034). Conclusions. This is the first case series supporting a potential association between EoE and pseudodiverticulosis. We also identify characteristic features of pseudodiverticulosis that may raise clinical suspicion of underlying eosinophilic esophagitis. PMID:27648438

  11. Esophageal Intramural Pseudodiverticulosis and Concomitant Eosinophilic Esophagitis: A Case Series

    PubMed Central

    Scaffidi, Michael A.; Garg, Ankit; Ro, Brandon; Wang, Christopher; Yang, Tony T. C.; Plener, Ian S.; Grin, Andrea; Colak, Errol

    2016-01-01

    Background. Esophageal intramural pseudodiverticulosis (EIPD) is an idiopathic benign chronic disease characterized by flask-like outpouchings of the esophageal wall. It is unknown whether there is a genuine association between EIPD and eosinophilic esophagitis (EoE). Aims. To investigate a possible relationship between EIPD and EoE. Methods. Patients with radiographic or endoscopic evidence of pseudodiverticulosis were identified from the database at a single academic center. Cases were analyzed in three areas: clinical information, endoscopic findings, and course. Results. Sixteen cases of esophageal pseudodiverticulosis were identified. Five patients had histologic evidence of eosinophilic esophagitis. Patients with EoE had pseudodiverticula in the mid-to-distal esophagus while those with EIPD had pseudodiverticula predominantly in the proximal esophagus (p < 0.001). EoE with pseudodiverticulosis occurred in younger patients (p < 0.019). Food bolus obstructions were more common in patients with EoE and pseudodiverticulosis than in EIPD (p < 0.034). Conclusions. This is the first case series supporting a potential association between EoE and pseudodiverticulosis. We also identify characteristic features of pseudodiverticulosis that may raise clinical suspicion of underlying eosinophilic esophagitis. PMID:27648438

  12. Esophageal Intramural Pseudodiverticulosis and Concomitant Eosinophilic Esophagitis: A Case Series

    PubMed Central

    Scaffidi, Michael A.; Garg, Ankit; Ro, Brandon; Wang, Christopher; Yang, Tony T. C.; Plener, Ian S.; Grin, Andrea; Colak, Errol

    2016-01-01

    Background. Esophageal intramural pseudodiverticulosis (EIPD) is an idiopathic benign chronic disease characterized by flask-like outpouchings of the esophageal wall. It is unknown whether there is a genuine association between EIPD and eosinophilic esophagitis (EoE). Aims. To investigate a possible relationship between EIPD and EoE. Methods. Patients with radiographic or endoscopic evidence of pseudodiverticulosis were identified from the database at a single academic center. Cases were analyzed in three areas: clinical information, endoscopic findings, and course. Results. Sixteen cases of esophageal pseudodiverticulosis were identified. Five patients had histologic evidence of eosinophilic esophagitis. Patients with EoE had pseudodiverticula in the mid-to-distal esophagus while those with EIPD had pseudodiverticula predominantly in the proximal esophagus (p < 0.001). EoE with pseudodiverticulosis occurred in younger patients (p < 0.019). Food bolus obstructions were more common in patients with EoE and pseudodiverticulosis than in EIPD (p < 0.034). Conclusions. This is the first case series supporting a potential association between EoE and pseudodiverticulosis. We also identify characteristic features of pseudodiverticulosis that may raise clinical suspicion of underlying eosinophilic esophagitis.

  13. Vibrant soundbridge in aural atresia: does severity matter?

    PubMed

    McKinnon, B J; Dumon, T; Hagen, R; Lesinskas, E; Mlynski, R; Profant, M; Spindel, J; Van Beek-King, J; Zernotti, M

    2014-07-01

    Congenital aural atresia (CAA) poses significant challenges to surgical remediation. Both bone anchored hearing aids (BAHA) and the Vibrant Soundbridge (VSB) have been considered as alternatives or adjuncts to conventional atresiaplasty. A consensus statement on VSB implantation in children and adolescents recommended against implantation when the Jahrsdoerfer score was less than 8. More recent publications suggest that patients with Jahrsdoerfer scores between three and seven may benefit from VSB implantation. The purpose of this study was to further investigate the outcomes of VSB implantation in CAA. The study was a multi-center, retrospective review. A retrospective review of data (patient's demographic, clinical, implant and audiological information) from four collaborating centers that have performed VSB implantation in CAA was performed. Outcomes based on severity of the atresia using the Jahrsdoerfer and Yellon-Branstetter scoring systems were also evaluated. Data from 28 patients from the four centers revealed no iatrogenic facial nerve injuries or change in bone thresholds. Post-operative speech threshold and speech recognition was, respectively, 39 dB and 94%. Jahrsdoerfer and Yellon scores ranged from 4 to 9 and 4 to 12, respectively. The scores did not correlate to or predict outcomes. Three individual elements of the scores did correlate to initial, but not long-term outcomes. Atresiaplasty and BAHA in the management of CAA are not complete solutions. VSB may offer an alternative in these surgically complex patients for achieving amplification, though better metrics for patient selection need to be developed. LEVEL OF EVIDENCE : IV. PMID:24030853

  14. Atresia of left atrioventricular orifice. Anatomical investigation in 62 cases.

    PubMed Central

    Thiene, G; Daliento, L; Frescura, C; De Tommasi, M; Macartney, F J; Anderson, R H

    1981-01-01

    Sixty-two hearts without a patent exit from the left atrium to the ventricular mass have been studied. All had situs solitus and laevocardia. The material consisted of 32 cases with coexistent aortic atresia and 30 cases with patent aortic root. Five hearts with aortic atresia were biventricular with atrioventricular concordance and imperforate left atrioventricular valves, and 27 hearts were univentricular of right ventricular type, with absent left atrioventricular connection. The anatomy of this group was uniform, with extreme hypoplasia of all the left cardiac segments. Among the cases with patent aortic root, five were biventricular, with atrioventricular concordance and imperforate left atrioventricular valves, and 24 had absent left atrioventricular connection, 15 with univentricular heart of right ventricular type and nine with univentricular heart of left ventricular type. The final heart had double inlet univentricular of left ventricular type with an imperforate left atrioventricular valve. In this second group the aorta was larger in cases with discordant ventriculoarterial connection or in those with double outlet from the main ventricular chamber. A normal sized aorta without aortic arch obstruction was observed in nine instances. These are of great interest in terms of surgical anatomy since definitive palliation may be feasible. Images PMID:7225254

  15. Pathogenesis of biliary atresia: defining biology to understand clinical phenotypes

    PubMed Central

    Asai, Akihiro; Miethke, Alexander; Bezerra, Jorge A.

    2016-01-01

    Biliary atresia is a severe cholangiopathy of early infancy that destroys extrahepatic bile ducts and disrupts bile flow. With a poorly defined disease pathogenesis, treatment consists of the surgical removal of duct remnants followed by hepatoportoenterostomy. Although this approach can improve the short-term outcome, the liver disease progresses to end-stage cirrhosis in most children. Further improvement in outcome will require a greater understanding of the mechanisms of biliary injury and fibrosis. Here, we review progress in the field, which has been fuelled by collaborative studies in larger patient cohorts and the development of cell culture and animal model systems to directly test hypotheses. Advances include the identification of phenotypic subgroups and stages of disease based on clinical, pathological and molecular features. Stronger evidence exists for viruses, toxins and gene sequence variations in the aetiology of biliary atresia, triggering a proinflammatory response that injures the duct epithelium and produces a rapidly progressive cholangiopathy. The immune response also activates the expression of type 2 cytokines that promote epithelial cell proliferation and extracellular matrix production by nonparenchymal cells. These advances provide insight into phenotype variability and might be relevant to the design of personalized trials to block progression of liver disease. PMID:26008129

  16. Environmental Causes of Esophageal Cancer

    PubMed Central

    Kamangar, Farin; Chow, Wong-Ho; Abnet, Christian; Dawsey, Sanford

    2009-01-01

    Synopsis This articles reviews the environmental risk factors and predisposing conditions for the two main histological types of esophageal cancer, esophageal squamous cell carcinoma (ESCC) and esophageal adenocarcinoma (EA). Tobacco smoking, excessive alcohol consumption, drinking maté, low intake of fresh fruits and vegetables, achalasia, and low socioeconomic status increase the risk of ESCC. Results of investigations on several other potential risk factors, including opium consumption, intake of hot drinks, eating pickled vegetables, poor oral health, and exposure to human papillomavirus, polycyclic aromatic hydrocarbons, N-nitroso compounds, acetaldehyde, and fumonisins are also discussed. Gastroesophageal reflux, obesity, tobacco smoking, hiatal hernia, achalasia, and probably absence of H. pylori in the stomach increase the risk of EA. Results of studies investigating other factors, including low intake of fresh fruits and vegetables, consumption of carbonated soft drink, use of H2 blockers, non-steroidal anti-inflammatory drugs, and drugs that relax the lower esophageal sphincter are also discussed. PMID:19327566

  17. Caustic ingestion and esophageal function

    SciTech Connect

    Cadranel, S.; Di Lorenzo, C.; Rodesch, P.; Piepsz, A.; Ham, H.R. )

    1990-02-01

    The aim of the present study was to investigate esophageal motor function by means of krypton-81m esophageal transit scintigraphy and to compare the results with the functional and morphological data obtained by means of triple lumen manometry and endoscopy. In acute and subacute stages of the disease, all clinical, anatomical, and functional parameters were in good agreement, revealing significant impairment. In chronic stages, the severity of the dysphagia was not correlated to the importance of the residual stenosis. Conversely, 81mKr esophageal transit and manometric's findings were in good agreement with the clinical symptoms, during the entire follow-up period ranging between 3 months to 7 years. The 81mKr test is undoubtedly the easiest and probably the most physiological technique currently available for long-term functional evaluation of caustic esophagitis.

  18. Drugs Approved for Esophageal Cancer

    Cancer.gov

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for esophageal cancer. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

  19. Understanding the sensory irregularities of esophageal disease.

    PubMed

    Farmer, Adam D; Brock, Christina; Frøkjaer, Jens Brøndum; Gregersen, Hans; Khan, Sheeba; Lelic, Dina; Lottrup, Christian; Drewes, Asbjørn Mohr

    2016-08-01

    Symptoms relating to esophageal sensory abnormalities can be encountered in the clinical environment. Such sensory abnormalities may be present in demonstrable disease, such as erosive esophagitis, and in the ostensibly normal esophagus, such as non-erosive reflux disease or functional chest pain. In this review, the authors discuss esophageal sensation and the esophageal pain system. In addition, the authors provide a primer concerning the techniques that are available for investigating the autonomic nervous system, neuroimaging and neurophysiology of esophageal sensory function. Such technological advances, whilst not readily available in the clinic may facilitate the stratification and individualization of therapy in disorders of esophageal sensation in the future. PMID:26890720

  20. Understanding the sensory irregularities of esophageal disease.

    PubMed

    Farmer, Adam D; Brock, Christina; Frøkjaer, Jens Brøndum; Gregersen, Hans; Khan, Sheeba; Lelic, Dina; Lottrup, Christian; Drewes, Asbjørn Mohr

    2016-08-01

    Symptoms relating to esophageal sensory abnormalities can be encountered in the clinical environment. Such sensory abnormalities may be present in demonstrable disease, such as erosive esophagitis, and in the ostensibly normal esophagus, such as non-erosive reflux disease or functional chest pain. In this review, the authors discuss esophageal sensation and the esophageal pain system. In addition, the authors provide a primer concerning the techniques that are available for investigating the autonomic nervous system, neuroimaging and neurophysiology of esophageal sensory function. Such technological advances, whilst not readily available in the clinic may facilitate the stratification and individualization of therapy in disorders of esophageal sensation in the future.

  1. Uses of esophageal function testing: dysphagia.

    PubMed

    Yazaki, Etsuro; Woodland, Philip; Sifrim, Daniel

    2014-10-01

    Esophageal function testing should be used for differential diagnosis of dysphagia. Dysphagia can be the consequence of hypermotility or hypomotility of the muscles of the esophagus. Decreased esophageal or esophagogastric junction distensibility can provoke dysphagia. The most well established esophageal dysmotility is achalasia. Other motility disorders can also cause dysphagia. High-resolution manometry (HRM) is the gold standard investigation for esophageal motility disorders. Simultaneous measurement of HRM and intraluminal impedance can be useful to assess motility and bolus transit. Impedance planimetry measures distensibility of the esophageal body and gastroesophageal junction in patients with achalasia and eosinophilic esophagitis. PMID:25216909

  2. Congenital bilateral adult choanal atresia undiagnosed until the second decade: How we did it

    PubMed Central

    Lokesh, P.; Panda, Naresh K.

    2016-01-01

    Background: Bilateral congenital choanal atresia that presents in adulthood is rare. There are only eight reported cases in the literature. Method: We present a ninth case of adult bilateral choanal atresia diagnosed at the age of 20 years. Can a bilateral choanal atresia present so late in life when such a condition is considered incompatible with life? Results and conclusion: Clinical details, diagnosis, and surgical steps with a clear depiction of photographs and comparison with all other previously reported cases, which can help novel otolaryngologists in their clinical practice are discussed. PMID:27658184

  3. Congenital bilateral adult choanal atresia undiagnosed until the second decade: How we did it

    PubMed Central

    Lokesh, P.; Panda, Naresh K.

    2016-01-01

    Background: Bilateral congenital choanal atresia that presents in adulthood is rare. There are only eight reported cases in the literature. Method: We present a ninth case of adult bilateral choanal atresia diagnosed at the age of 20 years. Can a bilateral choanal atresia present so late in life when such a condition is considered incompatible with life? Results and conclusion: Clinical details, diagnosis, and surgical steps with a clear depiction of photographs and comparison with all other previously reported cases, which can help novel otolaryngologists in their clinical practice are discussed.

  4. Cutaneous Metastases From Esophageal Adenocarcinoma

    PubMed Central

    Triantafyllou, Stamatina; Georgia, Doulami; Gavriella-Zoi, Vrakopoulou; Dimitrios, Mpistarakis; Stulianos, Katsaragakis; Theodoros, Liakakos; Georgios, Zografos; Dimitrios, Theodorou

    2015-01-01

    The aim of this study is to present 2 rare cases of cutaneous metastases originated from adenocarcinoma of the gastro-esophageal junction, thus, underline the need for early diagnosis and possible treatment of suspicious skin lesions among patients with esophageal malignancy. Metastatic cancer to the skin originated from internal malignancies, mostly lung cancer, breast cancer, and colorectal cancer, constitute 0.5 to 9% of all metastatic cancers.5,8,15 Skin metastases, mainly from squamous cell carcinomas of the esophagus, are rarely reported. Cutaneous metastasis is a finding indicating progressiveness of the disease.17 More precisely, median survival is estimated approximately 4.7 months.2,14 This study is a retrospective review of 2 cases of patients with adenocarcinoma of the esophagus and a review of the literature. Two patients aged 60 and 32 years old, respectively, underwent esophagectomy. Both pathologic reports disclosed adenocarcinoma of the gastro-esophageal junction staged T3 N2 M0 (stage IIIB). During follow-up time, the 2 patients were diagnosed with cutaneous metastases originated from the primary esophageal tumor 11 and 4 months after surgery, respectively. The first patient is alive 37 months after diagnosis, while the second one died 16 months after surgery. Cutaneous metastasis caused by esophageal adenocarcinoma is possible. Therefore, follow-up of patients who were diagnosed with esophageal malignancy and underwent esophagectomy is mandatory in order to reveal early surgical stages. PMID:25785344

  5. Esophageal malignancy: A growing concern

    PubMed Central

    Chai, Jianyuan; Jamal, M Mazen

    2012-01-01

    Esophageal cancer is mainly found in Asia and east Africa and is one of the deadliest cancers in the world. However, it has not garnered much attention in the Western world due to its low incidence rate. An increasing amount of data indicate that esophageal cancer, particularly esophageal adenocarcinoma, has been rising by 6-fold annually and is now becoming the fastest growing cancer in the United States. This rise has been associated with the increase of the obese population, as abdominal fat puts extra pressure on the stomach and causes gastroesophageal reflux disease (GERD). Long standing GERD can induce esophagitis and metaplasia and, ultimately, leads to adenocarcinoma. Acid suppression has been the main strategy to treat GERD; however, it has not been proven to control esophageal malignancy effectively. In fact, its side effects have triggered multiple warnings from regulatory agencies. The high mortality and fast growth of esophageal cancer demand more vigorous efforts to look into its deeper mechanisms and come up with better therapeutic options. PMID:23236223

  6. Eosinophilic Esophagitis in Pediatric and Adolescent Patients

    MedlinePlus

    ... and Adolescent Patients Eosinophilic Esophagitis in Pediatric and Adolescent Patients Basics Overview Eosinophilic esophagitis also known as ( ... children may have vomiting and abdominal pain, and adolescents may complain of the feeling of food getting ...

  7. 21 CFR 876.5365 - Esophageal dilator.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... and weighted with mercury or a metal olive-shaped weight that slides on a guide, such as a string or... esophageal or gastrointestinal bougies and the esophageal dilator (metal olive). (b) Classification. Class...

  8. 21 CFR 876.5365 - Esophageal dilator.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... and weighted with mercury or a metal olive-shaped weight that slides on a guide, such as a string or... esophageal or gastrointestinal bougies and the esophageal dilator (metal olive). (b) Classification. Class...

  9. 21 CFR 876.5365 - Esophageal dilator.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... and weighted with mercury or a metal olive-shaped weight that slides on a guide, such as a string or... esophageal or gastrointestinal bougies and the esophageal dilator (metal olive). (b) Classification. Class...

  10. 21 CFR 876.5365 - Esophageal dilator.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... and weighted with mercury or a metal olive-shaped weight that slides on a guide, such as a string or... esophageal or gastrointestinal bougies and the esophageal dilator (metal olive). (b) Classification. Class...

  11. Under pressure: a contribution to the pathogenesis of acquired ileal atresia

    PubMed Central

    Houben, C H; Lo, A W I; Tsui, S Y; Chan, K W

    2013-01-01

    An acquired ileal atresia is a rare occurrence. A 3-week-old neonate is presented, who developed postnatally a type 2 ileal atresia and an ileal stenosis within a pre-existing internal hernia secondary to an adhesion band. The literature reports a total of eight cases (4 females) with acquired ileal atresia in babies ranging in age from 3 weeks to 2 years (median 4 months). Mechanical forces (eg, adhesion band, intussusception and volvulus) onto the intestine are most frequently (75%) implicated as the primary event. The distal ileum is most often affected and a type 3A atresia is identified in six of eight (75%) cases. PMID:24225736

  12. Late presentation of gastric tube ulcer perforation after oesophageal atresia repair.

    PubMed

    Hazebroek, Eric J; Hazebroek, Frans W J; Leibman, Steven; Smith, Garett S

    2008-07-01

    Ulcer formation in intrathoracic grafts after oesophageal replacement is considered an infrequent complication of the procedure. We present a rare case of a gastric tube ulcer with perforation, more than 30 years after gastric tube interposition for oesophageal atresia.

  13. Trilogy of foregut, midgut and hindgut atresias presenting in reverse order

    PubMed Central

    Patel, Ramnik V; Jackson, Paul; De Coppi, Paolo; Pierro, Agostino

    2014-01-01

    We present a case of triple gut atresias (foregut, midgut and hindgut) with multiple congenital anomalies presenting as imperforate anus. Abdominal radiography showed the double bubble sign. Upper gastrointestinal study through a nasogastric tube confirmed duodenal obstruction. Exploratory laparotomy, duodeno-duodenostomy for duodenal atresia and a left descending colostomy for anorectal malformation were performed. During extubation, the nasogastric tube came out and could not be reinserted by the anaesthetic team under laryngoscopic guidance. A chest radiograph showed the tube curled in the upper pouch. Bronchoscopy and oesophagoscopy confirmed oesophageal atresia (OA) with a distal tracheoesophageal fistula (TOF). The patient underwent right-sided extrapleural thoracotomy and division of the fistula with primary repair of OA uneventfully. Triple gut atresias presenting in reverse order with multiple anomalies is rare and passage of a nasogastric tube into the stomach in the presence of OA+TOF is exceptional. Alimentary tract obstruction should be corrected in proximal to distal direction. PMID:24835810

  14. Esophageal Helicobacter pylori colonization aggravates esophageal injury caused by reflux

    PubMed Central

    Chu, Yun-Xiang; Wang, Wei-Hong; Dai, Yun; Teng, Gui-Gen; Wang, Shu-Jun

    2014-01-01

    AIM: To investigate esophageal Helicobacter pylori (H. pylori) colonization on esophageal injury caused by reflux and the related mechanisms. METHODS: An esophagitis model, with acid and bile reflux, was surgically produced in male rats. The rats were randomly divided into either: (1) an esophagogastroduodenal anastomosis (EGDA) group; (2) an EGDA with H. pylori infection group; (3) a pseudo-operation with H. pylori infection group; or (4) a pseudo-operation group. All rats were kept for 36 wk. Based on the location of H. pylori colonization, the EGDA rats with H. pylori infection were subdivided into those with concomitant esophageal H. pylori colonization or those with only gastric H. pylori colonization. The esophageal injuries were evaluated grossly and microscopically. The expressions of CDX2 and MUC2 were determined by real-time polymerase chain reaction (RT-PCR) and immunohistochemistry. Ki-67 antigen expression was determined by immunohistochemistry. The mRNA levels of cyclin D1, c-Myc, Bax and Bcl-2 were determined by RT-PCR. Cell apoptosis was evaluated using the TdT-mediated dUTP nick-end labeling method. RESULTS: Esophagitis, Barrett’s esophagus (BE), and esophageal adenocarcinoma (EAC) developed in rats that underwent EGDA. When comparing rats with EGDA and concomitant esophageal H. pylori colonization to EGDA-only rats, the severity of injury (87.9 ± 5.2 vs 77.2 ± 8.6, macroscopically, 92.5 ± 8.0 vs 83.8 ± 5.5, microscopically, both P < 0.05) and the incidences of BE (80.0% vs 33.3%, P = 0.055) and EAC (60.0% vs 11.1%, P < 0.05) were increased. These increases were associated with upregulation of CDX2 and MUC2 mRNA (10.1 ± 5.4 vs 3.0 ± 2.9, 8.4 ± 4.6 vs 2.0 ± 3.2, respectively, Ps < 0.01) and protein (8.1 ± 2.3 vs 3.3 ± 3.1, 7.3 ± 4.0 vs 1.8 ± 2.7, respectively, all P < 0.05). The expression of Ki-67 (8.9 ± 0.7 vs 6.0 ± 1.7, P < 0.01) and the presence of apoptotic cells (8.3 ± 1.1 vs 5.3 ± 1.7, P < 0.01) were also increased

  15. Prevention and Treatment of Esophageal Stenosis after Endoscopic Submucosal Dissection for Early Esophageal Cancer

    PubMed Central

    Wen, Jing; Lu, Zhongsheng; Liu, Qingsen

    2014-01-01

    Endoscopic submucosal dissection (ESD) for the treatment of esophageal mucosal lesions is associated with a risk of esophageal stenosis, especially for near-circumferential or circumferential esophageal mucosal defects. Here, we review historic and modern studies on the prevention and treatment of esophageal stenosis after ESD. These methods include prevention via pharmacological treatment, endoscopic autologous cell transplantation, endoscopic esophageal dilatation, and stent placement. This short review will focus on direct prevention and treatment, which may help guide the way forward. PMID:25386186

  16. A Comprehensive Review of Esophageal Stents

    PubMed Central

    Hong, Jinwha; Lam-Tsai, Yvette; Gress, Frank

    2012-01-01

    Esophageal stents are important tools for palliative treatment of inoperable esophageal malignancies. With the development of multiple self-expandable stents, there are now several therapeutic options for managing benign and malignant esophageal diseases. This paper discusses the various types of esophageal stents currently available, indications for their placement, challenges and complications that gastroenterologists face when placing these stents, and some of the innovations that will become available in the near future. PMID:23293566

  17. Endoscopic repair of unilateral choanal atresia with the KTP laser: a one stage procedure.

    PubMed

    Tzifa, K T; Skinner, D W

    2001-04-01

    This paper, describes the endoscopic repair of unilateral choanal atresia with the KTP laser, a one-stage procedure, with no requirement for stenting. Three patients are presented with unilateral choanal atresia, aged six, nine and 38-years-old. The procedure combines the excellent endoscopic visualization, with the good haemostatic and penetrating properties of the KTP laser. Follow up was between 12 months and four years with all choanae remaining patent, no dilatation was required. No surgical complications were noted.

  18. Gastric Duplication Cyst in Association with Duodenal Atresia in a Neonate

    PubMed Central

    Mirshemirani, Alireza; Roshanzamir, Fatollah; Razavi, Sajad; Sarafi, Mehdi

    2016-01-01

    Concurrence of duodenal atresia and gastric duplication cyst is extremely rare entity. We report a 6-day-old female neonate who presented with neonatal intestinal obstruction. X-ray abdomen showed double bubble sign. At laparotomy, a huge cystic structure attached to greater curvature of the stomach along with duodenal atresia of second part of duodenum was found. The cystic structure was excised and duodeno-duodenostomy performed. Histopathology report confirmed it gastric duplication cyst. PMID:26816679

  19. Ovarian Follicular Atresia of Ewes during Spring Puerperium

    PubMed Central

    Vlčková, Radoslava; Sopková, Drahomíra; Pošivák, Ján; Valocký, Igor

    2012-01-01

    The distribution of healthy and atretic follicles on the ovarian surface of improved Valachian ewes 17, 24, and 32 days postpartum is reported in this study. The number of healthy follicles was higher on day 24 postpartum and their mean diameter tended to increase to day 32 (P < 0.05) with the greatest diameter of 5 mm. 78–81% of atretic follicles ≥3 mm in diameter was observed where apoptosis began in the follicular cells situated at the follicular cavity. The early atretic follicles are characterized by the presence of mitotic pictures. In one ewe 24 days postpartum, small regressive follicular cysts were observed. Contracting atresia is characterized by thickening of the theca interna even to 190 μm. Progesterone and oestradiol-17β concentrations were maintained at relatively low levels, but with no significant difference between the days postpartum. PMID:22567543

  20. Biliary atresia: Will blocking inflammation tame the disease?

    PubMed Central

    Bessho, Kazuhiko; Bezerra, Jorge A.

    2014-01-01

    Biliary atresia is the most common cholangiopathy of childhood. With complete obstruction of segments or the entire length of extrahepatic bile ducts, the timely pursuit of hepatoportoenterostomy is the best strategy to restore bile drainage. However, even with prompt surgical intervention, ongoing injury of intrahepatic bile ducts and progressive cholangiopathy lead to end-stage cirrhosis. The pace of disease progression is not uniform; it may relate to clinical forms of disease and/or staging of liver pathology at diagnosis. Although the etiology of disease is not yet defined, several biological processes have been linked to pathogenic mechanisms of bile duct injury. Among them, there is increasing evidence that the immune system targets the duct epithelium and disrupts bile flow. In this review, we discuss how careful clinical phenotyping, staging of disease, and pre-clinical research give insight into response to ongoing trials and rational design of new therapies to block progression of disease. PMID:21226614

  1. Biventricular Repair of Pulmonary Atresia After Fontan Palliation.

    PubMed

    D'Souza, Travis F; Samuel, Bennett P; Hillman, Neal D; Vettukattil, Joseph J; Haw, Marcus P

    2016-04-01

    Fontan palliation is used when biventricular repair (BVR) is not possible. Early outcomes are acceptable; however, the long-term sequelae include protein-losing enteropathy, declining functional status, increased pulmonary vascular resistance, heart failure, and hepatic and renal dysfunction. These adverse events are characteristic of persistent venous hypertension and may be avoided if restoring biventricular circulation is possible. Arrhythmias are a common adverse event, particularly in patients with an atriopulmonary connection, which may lead to acute decompensation and early death. We describe a 30-year-old woman who underwent successful BVR for pulmonary atresia with intact ventricular septum and demonstrate that where favorable anatomy exists with a failing Fontan, BVR should be considered. PMID:27000578

  2. Biventricular Repair of Pulmonary Atresia After Fontan Palliation.

    PubMed

    D'Souza, Travis F; Samuel, Bennett P; Hillman, Neal D; Vettukattil, Joseph J; Haw, Marcus P

    2016-04-01

    Fontan palliation is used when biventricular repair (BVR) is not possible. Early outcomes are acceptable; however, the long-term sequelae include protein-losing enteropathy, declining functional status, increased pulmonary vascular resistance, heart failure, and hepatic and renal dysfunction. These adverse events are characteristic of persistent venous hypertension and may be avoided if restoring biventricular circulation is possible. Arrhythmias are a common adverse event, particularly in patients with an atriopulmonary connection, which may lead to acute decompensation and early death. We describe a 30-year-old woman who underwent successful BVR for pulmonary atresia with intact ventricular septum and demonstrate that where favorable anatomy exists with a failing Fontan, BVR should be considered.

  3. Prenatal diagnosis of isolated congenital pyloric atresia in a sibling.

    PubMed

    Usui, Noriaki; Kamiyama, Masafumi; Kimura, Takuya; Kamata, Shinkichi; Nose, Keisuke; Fukuzawa, Masahiro

    2013-02-01

    Although familial occurrence of congenital pyloric atresia (CPA) has been frequently reported in the past, many of these cases were associated with epidermolysis bullosa (EB), and familial isolated CPA was a relatively rare condition. We prenatally diagnosed and successfully treated a sibling of a subject with isolated CPA, who was diagnosed prenatally by fetal ultrasonography based on the findings of a distended stomach combined with polyhydramnios. The first case was a 2398-g female infant born at 36 weeks of gestation, who had been prenatally diagnosed as CPA. The second case, a younger sister of the first case, was a female infant weighing 2434 g, who had been also diagnosed as CPA by fetal ultrasonography at the check-up for the polyhydramnios of the same mother. Neither of the infants showed dermal lesions such as EB, and both underwent pyloroplasty with an excision of the pyloric membrane successfully after birth. PMID:23409992

  4. Gallium-67 imaging in candidal esophagitis

    SciTech Connect

    Rundback, J.H.; Goldfarb, C.R.; Ongseng, F. )

    1990-01-01

    Ga-67 scanning has been used to evaluate esophageal carcinoma. It has demonstrated candidal infection in other body sites and, in one previous case, in the esophagus. The authors present a case of diffuse esophageal uptake of Ga-67 in esophageal candidiasis.

  5. Methoxychlor and its metabolites inhibit growth and induce atresia of baboon antral follicles.

    PubMed

    Gupta, Rupesh K; Aberdeen, Graham; Babus, Janice K; Albrecht, Eugene D; Flaws, Jodi A

    2007-08-01

    Methoxychlor (MXC), an organochlorine pesticide, inhibits growth and induces atresia of antral follicles in rodents. MXC metabolites, mono-OH MXC (mono-OH) and bis-OH MXC (HPTE), are thought to be more toxic than the parent compound. Although studies have examined effects of MXC in rodents, few studies have evaluated the effects of MXC in primates. Therefore, the present study tested the hypothesis that MXC, mono-OH, and HPTE inhibit growth and induce atresia of baboon antral follicles. To test this hypothesis, antral follicles were isolated from adult baboon ovaries and cultured with vehicle (dimethylsulfoxide; DMSO), MXC (1-100 micro g/ml), mono-OH (0.1-10 micro g/ml), or HPTE (0.1-10 micro g/ml) for 96 hr. Growth was monitored at 24 hr intervals. After culture, follicles were processed for histological evaluation of atresia. MXC, mono-OH, and HPTE significantly inhibited follicular growth and increased atresia compared to DMSO. Moreover, the adverse effects of MXC and its metabolites on growth and atresia in baboon antral follicles were observed at lower (100-fold) doses than those causing similar effects in rodents. These data suggest that MXC and its metabolites inhibit growth and induce atresia of baboon antral follicles, and that primate follicles are more sensitive to MXC than rodent follicles.

  6. The extended Kasai portoenterostomy for biliary atresia: A preliminary report

    PubMed Central

    Ramachandran, Priya; Safwan, Mohamed; Srinivas, Sankaranarayanan; Shanmugam, Naresh; Vij, Mukul; Rela, Mohamed

    2016-01-01

    Aims: In 1955, Professor Morio Kasai first performed a hepatic portoenterostomy. Since then, the procedure has changed the lives of children with biliary atresia (BA). We report our initial experience in performing “extended” Kasai portoenterostomy (KPE), a modification of the original procedure. Materials and Methods: Since 2013, we have used the technique of “extended KPE” and prospectively recorded data on all children undergoing this operation. Data on demographics, clinical features, liver function tests, and perioperative cholangiogram findings were collected. Outcome of KPE was measured by Jaundice Disappearance Rate (JDR) and Native Liver Survival Rate (NLSR). We present our preliminary results from a 30-month period (February 2013 to May 2015). Results: Thirty-one children underwent KPE during this period (19 males) and only 1 child had biliary atresia splenic malformation (BASM). The mean age at KPE was 73 ± 24 days. Five (16.1%) children were more than 90 days old at the time of KPE. Fourteen children cleared jaundice (JDR 45.2%). Eleven (35.5%) children developed episodes of cholangitis, of whom 8 had early cholangitis (within 3 months of the operation). The proportion of children who survived with their own liver 6 months after KPE (NLSR) was 84.2%. Of those children older than 90 days, 2 cleared jaundice and have survived with their native livers for more than 16 months. Conclusion: In our preliminary report of 31 children, we conclude that the extended KPE leads to increased jaundice clearance and improved NLSR in children with BA. PMID:27046977

  7. Glycomic analyses of ovarian follicles during development and atresia

    PubMed Central

    Hatzirodos, Nicholas; Nigro, Julie; Irving-Rodgers, Helen F.; Vashi, Aditya V.; Hummitzsch, Katja; Caterson, Bruce; Sullivan, Thomas R.; Rodgers, Raymond J.

    2012-01-01

    To examine the detailed composition of glycosaminoglycans during bovine ovarian follicular development and atresia, the specialized stromal theca layers were separated from the stratified epithelial granulosa cells of healthy (n = 6) and atretic (n = 6) follicles in each of three size ranges: small (3–5 mm), medium (6-9 mm) and large (10 mm or more) (n = 29 animals). Fluorophore-assisted carbohydrate electrophoresis analyses (on a per cell basis) and immunohistochemistry (n = 14) were undertaken. We identified the major disaccharides in thecal layers and the membrana granulosa as chondroitin sulfate-derived ∆uronic acid with 4-sulfated N-acetylgalactosamine and ∆uronic acid with 6-sulfated N-acetylgalactosamine and the heparan sulfate-derived Δuronic acid with N-acetlyglucosamine, with elevated levels in the thecal layers. Increasing follicle size and atresia was associated with increased levels of some disaccharides. We concluded that versican contains 4-sulfated N-acetylgalactosamine and it is the predominant 4-sulfated N-acetylgalactosamine proteoglycan in antral follicles. At least one other non- or 6-sulfated N-acetylgalactosamine proteoglycan(s), which is not decorin or an inter-α-trypsin inhibitor family member, is present in bovine antral follicles and associated with hitherto unknown groups of cells around some larger blood vessels. These areas stained positively for chondroitin/dermatan sulfate epitopes [antibodies 7D4, 3C5, and 4C3], similar to stem cell niches observed in other tissues. The sulfation pattern of heparan sulfate glycosaminoglycans appears uniform across follicles of different sizes and in healthy and atretic follicles. The heparan sulfate products detected in the follicles are likely to be associated with perlecan, collagen XVIII or betaglycan. PMID:22057033

  8. Outcomes of esophageal surgery, especially of the lower esophageal sphincter.

    PubMed

    Bonavina, Luigi; Siboni, Stefano; Saino, Greta I; Cavadas, Demetrio; Braghetto, Italo; Csendes, Attila; Korn, Owen; Figueredo, Edgar J; Swanstrom, Lee L; Wassenaar, Eelco

    2013-10-01

    This paper includes commentaries on outcomes of esophageal surgery, including the mechanisms by which fundoduplication improves lower esophageal sphincter (LES) pressure; the efficacy of the Linx™ management system in improving LES function; the utility of radiologic characterization of antireflux valves following surgery; the correlation between endoscopic findings and reported symptoms following antireflux surgery; the links between laparoscopic sleeve gastrectomy and decreased LES pressure, endoscopic esophagitis, and gastroesophageal reflux disease (GERD); the less favorable outcomes following fundoduplication among obese patients; the application of bioprosthetic meshes to reinforce hiatal repair and decrease the incidence of paraesophageal hernia; the efficacy of endoluminal antireflux procedures, and the limited efficacy of revisional antireflux operations, underscoring the importance of good primary surgery and diligent work-up to prevent the necessity of revisional procedures. PMID:24117632

  9. Eosinophilic esophagitis in an octogenarian

    PubMed Central

    Trifan, Anca; Stoica, Oana; Chihaia, Catalin-Alexandru; Danciu, Mihai; Stanciu, Carol; Singeap, Ana-Maria

    2016-01-01

    Abstract Introduction: Eosinophilic esophagitis (EoE) is a chronic, immune/antigen-mediated disease characterized clinically by symptoms related to esophageal dysfunction and histologically by a marked eosinophilic infiltrate in the esophageal mucosa. What was once considered a rare disease has nowadays become one of the most frequent esophageal diseases in the Western countries, occupying a place just next to the gastroesophageal reflux disease. EoE etiology and pathogenesis remain largely unknown, although most studies consider that allergic and genetic factors play the most important role. Methods: We report the case of EoE in an elderly male (octogenarian), giving a brief review of the current data related to epidemiology, pathogenesis, diagnosis, and treatment of the disease. Results: Dysphagia to solid foods was the leading symptom, and endoscopic findings included white exudates, longitudinal furrows, and concentric mucosal rings, all suggestive for EoE. Diagnosis relied on histological findings in esophageal mucosal biopsies (>30 eosinophils per high power field). He was treated with topical steroids for 8 weeks, symptoms improved gradually and the patient remained in remission at the 8-month follow-up. Conclusion: This case emphasizes that EoE may occur in very old patients and gastroenterologists should have a high index of suspicion of this disorder in any elderly with dysphagia and endoscopic relevant features. PMID:27741150

  10. The Pathophysiology of Eosinophilic Esophagitis

    PubMed Central

    Raheem, Mayumi; Leach, Steven T.; Day, Andrew S.; Lemberg, Daniel A.

    2014-01-01

    Eosinophilic esophagitis (EoE) is an emerging disease characterized by esophageal eosinophilia (>15eos/hpf), lack of responsiveness to acid-suppressive medication and is managed by allergen elimination and anti-allergy therapy. Although the pathophysiology of EoE is currently unsubstantiated, evidence implicates food and aeroallergen hypersensitivity in genetically predisposed individuals as contributory factors. Genome-wide expression analyses have isolated a remarkably conserved gene-expression profile irrespective of age and gender, suggesting a genetic contribution. EoE has characteristics of mainly TH2 type immune responses but also some TH1 cytokines, which appear to strongly contribute to tissue fibrosis, with esophageal epithelial cells providing a hospitable environment for this inflammatory process. Eosinophil-degranulation products appear to play a central role in tissue remodeling in EoE. This remodeling and dysregulation predisposes to fibrosis. Mast-cell-derived molecules such as histamine may have an effect on enteric nerves and may also act in concert with transforming growth factor-β to interfere with esophageal musculature. Additionally, the esophageal epithelium may facilitate the inflammatory process under pathogenic contexts such as in EoE. This article aims to discuss the contributory factors in the pathophysiology of EoE. PMID:24910846

  11. [Caustic esophagitis: surgical management].

    PubMed

    Huamán, M; Santibáñez, G; Ayala, L; Jáuregui, F; Madalengoitia, G

    1990-01-01

    The authors present 28 report cases of severe caustic esophagitis who underwent surgery at the Hospital Nacional Edgardo Rebagliati Martins I.P.S.S., from 1987 to February 1991. All were treated by esophagocoloplasty and when the proximal cervical esophagus was injured, by pharingocoloplasty. Eighteen patients (64.28%) were female and 10 (35.72%) male. Whose ages ranged between 15 and 75 years with an average of 34 years. Ten patients (36%) suffered pharingocoloplasty and in the remaining 18 (64%) a esophagocoloplasty (esophagogastric colonic interposition) was made. Postoperative morbidity corresponded in 5 cases (18%) to respiratory Infections, in 3 cases (11%) pneumothorax, in 2 cases (7%) pleural effusions and only one case presented a small cervical leak. All of them were satisfactory solved by conservatory medical treatment. Only two cases (7%) with late postoperative stenosis required reintervention and a simple cervical plasty was made. There were no complications as mediastinitis, necrosis of the colon graft hemorrhage. Operatory mortality was 0% and the long term follow up of all patients is satisfactory, having our first report cases more than 4 years of postoperative follow up.

  12. A Treatment Option for Esophageal Intramural Pseudodiverticulosis.

    PubMed

    Tyberg, Amy; Jodorkovsky, Daniela

    2014-04-01

    Esophageal intramural pseudodiverticulosis (EIPD) is a rare condition often presenting with esophageal strictures. Treatment is often limited to endoscopic dilatation and treatment of the underlying esophageal pathology. We present a case of a patient with longstanding GERD on famotidine (she experienced anaphylaxis with proton pump inhibitors [PPIs]) who presented with dysphagia and weight loss. Work-up revealed a diagnosis of EIPD with a 5-mm mid-esophageal stricture. Therapy with dilatation was unsuccessful until the addition of sucralfate, after which dilatation was successful and symptoms resolved. In patients who are unable to take PPIs, the addition of sucralfate may enhance the success of dilatations of esophageal strictures and EIPD. PMID:26157852

  13. Hyperthermochemoradiotherapy for esophageal cancer (review).

    PubMed

    Maehara, Y; Kuwano, H; Kitamura, K; Matsuda, H; Sugimachi, K

    1992-01-01

    Hyperthermia is effective for the treatment of cancer when applied concomitantly with chemotherapy and irradiation. However, it is difficult to heat deep portions of the body including the esophagus. Cancer of the esophagus still poses considerable treatment problems, with a poor 5-year survival rate after surgery, an even worse outlook after radiation and surgery, and a not very satisfactory response to chemotherapy. We, therefore, devised an electrode for radio frequency, and we have been successfully using this electrode in the treatment of esophageal cancer. The 5-year survival rates of patients with esophageal cancer, given either preoperative hyperthermochemoradiotherapy or chemoradiotherapy, were 43.2 and 14.7%, respectively. Immediate improvement of subjective complaints and decrease or elimination of the cancer lesion are so distinct that this treatment, by means of an endotract antenna, shows promise as a modality for esophageal lesions.

  14. Surgical treatments for esophageal cancers

    PubMed Central

    Allum, William H.; Bonavina, Luigi; Cassivi, Stephen D.; Cuesta, Miguel A.; Dong, Zhao Ming; Felix, Valter Nilton; Figueredo, Edgar; Gatenby, Piers A.C.; Haverkamp, Leonie; Ibraev, Maksat A.; Krasna, Mark J.; Lambert, René; Langer, Rupert; Lewis, Michael P.N.; Nason, Katie S.; Parry, Kevin; Preston, Shaun R.; Ruurda, Jelle P.; Schaheen, Lara W.; Tatum, Roger P.; Turkin, Igor N.; van der Horst, Sylvia; van der Peet, Donald L.; van der Sluis, Peter C.; van Hillegersberg, Richard; Wormald, Justin C.R.; Wu, Peter C.; Zonderhuis, Barbara M.

    2015-01-01

    The following, from the 12th OESO World Conference: Cancers of the Esophagus, includes commentaries on the role of the nurse in preparation of esophageal resection (ER); the management of patients who develop high-grade dysplasia after having undergone Nissen fundoplication; the trajectory of care for the patient with esophageal cancer; the influence of the site of tumor in the choice of treatment; the best location for esophagogastrostomy; management of chylous leak after esophagectomy; the optimal approach to manage thoracic esophageal leak after esophagectomy; the choice for operational approach in surgery of cardioesophageal crossing; the advantages of robot esophagectomy; the place of open esophagectomy; the advantages of esophagectomy compared to definitive chemoradiotherapy; the pathologist report in the resected specimen; the best way to manage patients with unsuspected positive microscopic margin after ER; enhanced recovery after surgery for ER: expedited care protocols; and long-term quality of life in patients following esophagectomy. PMID:25266029

  15. Prevalence of Eosinophilic Esophagitis and Lymphocytic Esophagitis in Adults with Esophageal Food Bolus Impaction.

    PubMed

    Truskaite, Kotryna; Dlugosz, Aldona

    2016-01-01

    Background. The relation of esophageal food bolus impaction (FBI) to eosinophilic esophagitis (EoE) and lymphocytic esophagitis (LyE) is unclear. The aim of this study was to determine the prevalence of EoE and LyE among adults with FBI. Methods. In this retrospective study we analyzed data from all patients referred for gastroscopy during the past 5 years, because of a present or recent episode of FBI. Results. We found 238 patients with FBI (median age 51 (17-96), 71% males). Endoscopic therapy was required in 143 patients. Esophageal biopsies were obtained in 185 (78%) patients. All biopsies were assessed for numbers of eosinophils and lymphocytes. EoE was found in 18% of patients who underwent biopsy. We found 41 patients (22%) who fulfilled the criteria for both EoE and LyE (EoE/LyE). LyE was found in the 9% of patients with FBI. EoE together with EoE/LyE was the leading cause of FBI in patients ≤50 years (64%). GERD was the leading cause of FBI among patients older than 50 years (42%). Conclusions. Our study showed that EoE was the leading cause of FBI in particular among young adults. Our study highlights the need for esophageal biopsies in any patient with FBI.

  16. Prevalence of Eosinophilic Esophagitis and Lymphocytic Esophagitis in Adults with Esophageal Food Bolus Impaction

    PubMed Central

    Truskaite, Kotryna

    2016-01-01

    Background. The relation of esophageal food bolus impaction (FBI) to eosinophilic esophagitis (EoE) and lymphocytic esophagitis (LyE) is unclear. The aim of this study was to determine the prevalence of EoE and LyE among adults with FBI. Methods. In this retrospective study we analyzed data from all patients referred for gastroscopy during the past 5 years, because of a present or recent episode of FBI. Results. We found 238 patients with FBI (median age 51 (17–96), 71% males). Endoscopic therapy was required in 143 patients. Esophageal biopsies were obtained in 185 (78%) patients. All biopsies were assessed for numbers of eosinophils and lymphocytes. EoE was found in 18% of patients who underwent biopsy. We found 41 patients (22%) who fulfilled the criteria for both EoE and LyE (EoE/LyE). LyE was found in the 9% of patients with FBI. EoE together with EoE/LyE was the leading cause of FBI in patients ≤50 years (64%). GERD was the leading cause of FBI among patients older than 50 years (42%). Conclusions. Our study showed that EoE was the leading cause of FBI in particular among young adults. Our study highlights the need for esophageal biopsies in any patient with FBI. PMID:27547221

  17. Prevalence of Eosinophilic Esophagitis and Lymphocytic Esophagitis in Adults with Esophageal Food Bolus Impaction.

    PubMed

    Truskaite, Kotryna; Dlugosz, Aldona

    2016-01-01

    Background. The relation of esophageal food bolus impaction (FBI) to eosinophilic esophagitis (EoE) and lymphocytic esophagitis (LyE) is unclear. The aim of this study was to determine the prevalence of EoE and LyE among adults with FBI. Methods. In this retrospective study we analyzed data from all patients referred for gastroscopy during the past 5 years, because of a present or recent episode of FBI. Results. We found 238 patients with FBI (median age 51 (17-96), 71% males). Endoscopic therapy was required in 143 patients. Esophageal biopsies were obtained in 185 (78%) patients. All biopsies were assessed for numbers of eosinophils and lymphocytes. EoE was found in 18% of patients who underwent biopsy. We found 41 patients (22%) who fulfilled the criteria for both EoE and LyE (EoE/LyE). LyE was found in the 9% of patients with FBI. EoE together with EoE/LyE was the leading cause of FBI in patients ≤50 years (64%). GERD was the leading cause of FBI among patients older than 50 years (42%). Conclusions. Our study showed that EoE was the leading cause of FBI in particular among young adults. Our study highlights the need for esophageal biopsies in any patient with FBI. PMID:27547221

  18. Dietary treatment of eosinophilic esophagitis.

    PubMed

    Gonsalves, Nirmala; Kagalwalla, Amir F

    2014-06-01

    Emerging evidence supports impaired epithelial barrier function as the key initial event in the development of eosinophilic esophagitis (EoE) and other allergic diseases. Symptom resolution, histologic remission, and prevention of both disease and treatment-related complications are the goals of treatment. Successful dietary treatments include elemental, empirical elimination and allergy test directed diets. Dietary therapy with exclusive elemental diet offers the best response. Cow's milk, wheat, egg, soy, peanut/tree nut, and fish/shellfish are the 6 food antigens most likely to induce esophageal inflammation.

  19. Imaging and Clinicopathologic Features of Esophageal Gastrointestinal Stromal Tumors

    PubMed Central

    Winant, Abbey J.; Gollub, Marc J.; Shia, Jinru; Antonescu, Christina; Bains, Manjit S.; Levine, Marc S.

    2016-01-01

    OBJECTIVE The purpose of this article is to describe the imaging and clinicopathologic characteristics of esophageal gastrointestinal stromal tumors (GISTs) and to emphasize the features that differentiate esophageal GISTs from esophageal leiomyomas. MATERIALS AND METHODS A pathology database search identified all surgically resected or biopsied esophageal GISTs, esophageal leiomyomas, and esophageal leiomyosarcomas from 1994 to 2012. Esophageal GISTs were included only if imaging studies (including CT, fluoroscopic, or 18F-FDG PET/CT scans) and clinical data were available. RESULTS Nineteen esophageal mesenchymal tumors were identified, including eight esophageal GISTs (42%), 10 esophageal leiomyomas (53%), and one esophageal leiomyosarcoma (5%). Four patients (50%) with esophageal GIST had symptoms, including dysphagia in three (38%), cough in one (13%), and chest pain in one (13%). One esophageal GIST appeared on barium study as a smooth submucosal mass. All esophageal GISTs appeared on CT as well-marginated predominantly distal lesions, isoattenuating to muscle, that moderately enhanced after IV contrast agent administration. Compared with esophageal leiomyomas, esophageal GISTs tended to be more distal, larger, and more heterogeneous and showed greater IV enhancement on CT. All esophageal GISTs showed marked avidity (mean maximum standardized uptake value, 16) on PET scans. All esophageal GISTs were positive for c-KIT (a cell-surface transmembrane tyrosine kinase also known as CD117) and CD34. On histopathology, six esophageal GISTs (75%) were of the spindle pattern and two (25%) were of a mixed spindle and epithelioid pattern. Five esophageal GISTs had exon 11 mutations (with imatinib sensitivity). Clinical outcome correlated with treatment strategy (resection plus adjuvant therapy or resection alone) rather than risk stratification. CONCLUSION Esophageal GISTs are unusual but clinically important mesenchymal neoplasms. Although esophageal GISTs and

  20. Fecundity regulation by atresia in turbot Scophthalmus maximus in the Baltic Sea.

    PubMed

    Nissling, A; Thorsen, A; da Silva, F F G

    2016-04-01

    Down-regulation of fecundity through oocyte resorption was assessed in Baltic Sea turbot Scophthalmus maximus at three locations in the period from late vitellogenesis in April to spawning during June to July. The mean ± s.d. total length of the sampled fish was 32.7 ± 3.1 cm and mean ± s.d. age was 6.2 ± 1.5 years. Measurements of atresia were performed using the 'profile method' with the intensity of atresia adjusted according to the 'dissector method' (10.6% adjustment; coefficient of determination was 0.675 between methods). Both prevalence (portion of fish with atresia) and intensity (calculated as the average proportion of atretic cells in fish displaying atresia) of atresia were low in prespawning fish, but high from onset of spawning throughout the spawning period. Atretic oocytes categorized as in early alpha and in late alpha state occurred irrespective of maturity stage from late prespawning individuals up to late spawning fish, showing that oocytes may become atretic throughout the spawning period. Observed prevalence of atresia throughout the spawning period was almost 40% with an intensity of c. 20%. This indicates extensive down-regulation, i.e. considerably lower realized (number of eggs spawned) v. potential fecundity (number of developing oocytes), suggesting significant variability in reproductive potential. The extent of fecundity regulation in relation to fish condition (Fulton's condition factor) is discussed, suggesting an association between levels of atresia and fish condition. PMID:26928526

  1. Efficacy of Intensity Modulated Radiation Therapy After Surgery in Early Stage of Esophageal Carcinoma;

    ClinicalTrials.gov

    2016-07-30

    Esophageal Neoplasm; Esophageal Cancer TNM Staging Primary Tumor (T) T2; Esophageal Cancer TNM Staging Primary Tumor (T) T3; Esophageal Cancer TNM Staging Regional Lymph Nodes (N) N0; Esophageal Cancer TNM Staging Distal Metastasis (M) M0

  2. Clinical Study of Time Optimizing of Endoscopic Photodynamic Therapy on Esophageal and/or Gastric Cardiac Cancer

    ClinicalTrials.gov

    2015-12-10

    Stage I Esophageal Adenocarcinoma; Stage II Esophageal Adenocarcinoma; Stage III Esophageal Adenocarcinoma; Stage I Esophageal Squamous Cell Carcinoma; Stage II Esophageal Squamous Cell Carcinoma; Stage III Esophageal Squamous Cell Carcinoma

  3. Esophageal manifestations of celiac disease.

    PubMed

    Lucendo, A J

    2011-09-01

    Celiac disease (CD) may often be associated with various motor disorders affecting the different segments of the digestive tract, including the esophagus. Although it has not been universally reported, some available evidences indicate that pediatric and adult celiac patients could manifest a higher frequency of esophagitis and gastroesophageal reflux disease-related symptoms compared to nonceliac patients. In addition, several published studies have consistently shown the efficacy of a gluten-free diet in rapidly controlling esophageal symptoms and in preventing their recurrence. Since the participation of gluten in the esophageal symptoms of CD seems clear, its intimate mechanisms have yet to be elucidated, and several hypothesis have been proposed, including the specific immune alterations characterizing CD, the reduction in nutrient absorption determining the arrival of intact gluten to distal gastrointestinal segments, and various dysregulations in the function of gastrointestinal hormones and peptides. Recent studies have suggested the existence of a possible relationship between CD and eosinophilic esophagitis, which should be more deeply investigated.

  4. Esophageal Cancer Risk Prediction Models

    Cancer.gov

    Developing statistical models that estimate the probability of developing esophageal cancer over a defined period of time will help clinicians identify individuals at higher risk of specific cancers, allowing for earlier or more frequent screening and counseling of behavioral changes to decrease risk.

  5. Right atrial outlet atresia with straddling left atrioventricular valve. A form of double outlet atrium.

    PubMed Central

    Coto, E O; Calabro, R; Marsico, F; Lopez Arranz, J S

    1981-01-01

    We present three hearts showing right atrial outlet atresia with straddling left atrioventricular valve; clinical data of two of the patients are reported. For semantic and practical reasons we believe the terms tricuspid or mitral atresia should not be used in these cases, and atrial outlet atresia is preferable; for similar reasons we prefer to use stradding left or right atrioventricular valve. These anomalies represent an interesting form of double outlet atrium and diagnosis clinically may be possible if the existence of the anomaly is considered. Differentiation from common atrioventricular canal with atrial outlet atresia is important since total repair, which is possible for this last anomaly, does not seem feasible in cases like ours. Classifications of tricuspid atresia may have to be enlarged to include cases with double outlet from either ventricular chamber or anatomically corrected malposition. Characterisation of the right ventricular chamber is difficult; we believe that description of its morphology and the type and mode of its connections should be used. Images PMID:7470345

  6. Applications of titanium mesh tubing in external ear canal reconstruction in congenital aural atresia.

    PubMed

    Chen, Junming; Liang, Hairong; Wang, Yuejian; Yu, Youjun

    2015-04-01

    The aim of this study was to observe the effect of a titanium tube on external auditory canal reconstruction in congenital aural atresia and to assess the tube's effectiveness in preventing external canal stenosis or atresia after reconstruction. Reconstruction of the external ear canal with a titanium mesh tube was performed in 16 patients (16 ears) with congenital aural atresia at the First People's Hospital of Foshan. The titanium mesh tube was removed 1 year after surgery. The patients were followed up for 2 years (2 ± 0.3 years), and all of the patients had formed a new external ear canal. There was no local infection, granulation tissue, re-stenosis, or atresia in any of the patients after surgery. All of the patients were content with their newly formed external ear canal. Titanium mesh tubing is safe and effective for reconstruction of the external ear canal during surgery for congenital aural atresia.

  7. The Sea Lamprey as an Etiological Model for Biliary Atresia

    PubMed Central

    Chung-Davidson, Yu-Wen; Yeh, Chu-Yin; Li, Weiming

    2015-01-01

    Biliary atresia (BA) is a progressive, inflammatory, and fibrosclerosing cholangiopathy in infants that results in obstruction of both extrahepatic and intrahepatic bile ducts. It is the most common cause for pediatric liver transplantation. In contrast, the sea lamprey undergoes developmental BA with transient cholestasis and fibrosis during metamorphosis, but emerges as a fecund adult with steatohepatitis and fibrosis in the liver. In this paper, we present new histological evidence and compare the sea lamprey to existing animal models to highlight the advantages and possible limitations of using the sea lamprey to study the etiology and compensatory mechanisms of BA and other liver diseases. Understanding the signaling factors and genetic networks underlying lamprey BA can provide insights into BA etiology and possible targets to prevent biliary degeneration and to clear fibrosis. In addition, information from lamprey BA can be used to develop adjunct treatments for patients awaiting or receiving surgical treatments. Furthermore, the cholestatic adult lamprey has unique adaptive mechanisms that can be used to explore potential treatments for cholestasis and nonalcoholic steatohepatitis (NASH). PMID:26101777

  8. Brain abscess in hepatopulmonary syndrome associated with biliary atresia.

    PubMed

    Morita, Keiichi; Fukuzawa, Hiroaki; Maeda, Kosaku

    2015-12-01

    The first-choice therapy for biliary atresia (BA) is Kasai hepatoportoenterostomy, which has been shown to greatly improve outcome. Various long-term complications, however, such as portal hypertension and hepatopulmonary syndrome (HPS), can occur in patients with native liver. A rare case of brain abscess in an 11-year-old girl with HPS associated with BA is reported. The patient underwent hepatoportoenterostomy for BA at 53 days of age, with resolution of hyperbilirubinemia. At 10 years of age, she was diagnosed with severe HPS with right-to-left shunting, and preparations for liver transplantation proceeded. Three months after the diagnosis, she had a right parietal brain abscess. Given that the brain abscess enlarged in size, surgical drainage of the brain abscess was performed. The postoperative course was uneventful, but a slight left hemiplegia remained at discharge. The presumed mechanism of abscess formation in HPS may be right-to-left bacterial transit through intrapulmonary vascular dilatations and/or arteriovenous fistulae. PMID:26711920

  9. The Modified Kimura's Technique for the Treatment of Duodenal Atresia

    PubMed Central

    Zuccarello, Biagio; Spada, Antonella; Centorrino, Antonio; Turiaco, Nunzio; Chirico, Maria Rosaria; Parisi, Saveria

    2009-01-01

    Background/Purpose. Kimura's diamond-shaped-duodenoduodenostomy (DSD) is a known technique for the correction of congenital intrinsic duodenal obstruction. We present a modification of the technique and review the advantages of this new technique. Methods. From 1992 to 2006, 14 newborns were treated for duodenal atresia. We inverted the direction of the duodenal incisions: a longitudinal incision was made in the proximal duodenum while the distal was opened by transverse incision. Results. Our “inverted-diamond-shaped-duodenoduodenostomy” (i-DSD) allowed postoperative oral feeding to start on days 2 to 3, peripheral intravenous fluids discontinuity on days 3 to 8 (median values 3.6); time to achieve full oral feeds on days 8 to 12 (median values 9.4); the length of hospitalisation ranged from 10 and 14 days (median value 11.2). No complications related to the anastomosis, by Viz leakage, dehiscence, biliary stasis, or stenosis were observed. Conclusions. The i-DSD provides a safe procedure to protect the ampulla of Vater from injury and avoids any formation of a blind loop. The results show that patients who have i-DSD achieve full oral feeds in a very short time period and, consequently, the length of hospitalisation is also significantly reduced. PMID:19946416

  10. Transjugular Intrahepatic Portosystemic Shunts in Children with Biliary Atresia

    SciTech Connect

    Huppert, Peter E.; Goffette, Pierre; Sokal, Emil M.; Schweizer, Paul; Claussen, Claus D.

    2002-12-15

    Purpose: We retrospectively evaluated the technical and long-term clinical results of transjugularintrahepatic portosystemic shunts (TIPS) in children with portal hypertension and biliary atresia (BA). Methods: Nine children with BA and recurrent bleeding from esophagogastric and/or intestinal varices were treated by TIPS at the age of 34-156 months and followed-up in two centers. Different types of stents were used. Results: Shunt insertion succeeded in all patients, but in two a second procedure was necessary. Seven procedures lasted more than 3 hr, mainly due to difficult portal vein puncture.Variceal bleeding ceased in all patients; however, 16 reinterventions were performed in eight patients for clinical reasons (n =11) and sonographically suspected restenosis (n =5). Four patients underwent successful liver transplantation 4-51 months after TIPS and five are in good clinical conditions 64-75 months after TIPS. Conclusions: TIPS in children with BA is technically difficult, mainly due to periportal fibrosis and small portal veins. Frequency of reinterventions seems to be higher compared with adults.

  11. Esophageal wall blood perfusion during contraction and transient lower esophageal sphincter relaxation in humans

    PubMed Central

    Jiang, Yanfen; Bhargava, Valmik; Kim, Young Sun

    2012-01-01

    We recently reported that esophageal contraction reduces esophageal wall perfusion in an animal study. Our aim was to determine esophageal wall blood perfusion (EWBP) during esophageal contraction and transient lower esophageal sphincter relaxations (TLESRs) in humans. We studied 12 healthy volunteers. A custom-designed laser Doppler probe was anchored to the esophageal wall, 4–6 cm above the LES, by use of the Bravo pH system so that the laser light beam stay directed toward the esophageal mucosa. A high-resolution manometry equipped with impedance electrodes recorded esophageal pressures and reflux events. Synchronized pressure, impedance, pH, and EWBP recordings were obtained during dry and wet swallows and following a meal. Stable recordings of laser Doppler EWBP were only recorded when the laser Doppler probe was firmly anchored to the esophageal wall. Esophageal contractions induced by dry and wet swallows resulted in 46 ± 9% and 60 ± 10% reduction in the EWBP, respectively (compared to baseline). Reduction in EWBP was directly related to the amplitude (curvilinear fit) and duration of esophageal contraction. Atropine reduced the esophageal contraction amplitude and decreased the EWBP reduction associated with esophageal contraction. TLESRs were also associated with reduction in the EWBP, albeit of smaller amplitude (29 ± 3%) but longer duration (19 ± 2 s) compared with swallow-induced esophageal contractions. We report 1) an innovative technique to record EWBP for extended time periods in humans and 2) contraction of circular and longitudinal muscle during peristalsis and selective longitudinal muscle contraction during TLESR causes reduction in the EWBP; 3) using our innovative technique, future studies may determine whether esophageal wall ischemia is the cause of esophageal pain/heartburn. PMID:22790599

  12. Methoxychlor induces atresia by altering Bcl2 factors and inducing caspase activity in mouse ovarian antral follicles in vitro.

    PubMed

    Basavarajappa, Mallikarjuna S; Karman, Bethany N; Wang, Wei; Gupta, Rupesh K; Flaws, Jodi A

    2012-12-01

    Methoxychlor (MXC) is an organochlorine pesticide widely used in many countries against various species of insects that attack crops and domestic animals. MXC reduces fertility by increasing atresia (death) of antral follicles in vivo. MXC also induces atresia of antral follicles after 96 h in vitro. The current work tested the hypothesis that MXC induces morphological atresia at early time points (24 and 48 h) by altering pro-apoptotic (Bax, Bok, Casp3, and caspase activity) and anti-apoptotic (Bcl2 and Bcl-xL) factors in the follicles. The results indicate that at 24 h, MXC increased Bcl-xL and Bax mRNA levels and increased the ratio of Bax/Bcl2. At 48-96 h, MXC induced morphological atresia. At 24-96 h, MXC increased caspase activities. These data suggest that MXC may induce atresia by altering Bcl2 factors and inducing caspase activities in antral follicles.

  13. Sudden fetal death associated with both duodenal atresia and umbilical cord ulcer: a case report and review.

    PubMed

    Anami, Ai; Morokuma, Seiichi; Tsukimori, Kiyomi; Kondo, Haruhiko; Nozaki, Masahiro; Sueishi, Katsuo; Nakano, Hitoo

    2006-04-01

    We encountered one case of duodenal atresia complicated by massive intrauterine hemorrhage due to the perforation of an umbilical cord ulceration (UCU). UCU is an extremely rare complication in the perinatal period. Although the prenatal diagnosis of upper intestinal atresia has been established, little is known about the association between UCU and upper intestinal atresia. In this article, we report our case, review past articles, and discuss the underlying pathophysiological mechanisms of the cause of an UCU. Given the characteristic sites of upper intestinal atresia, we speculate that regurgitation of gastric or intestinal juice into the amniotic fluid could be responsible for the development of UCU. We also believe that close observation is required for patients who have upper intestinal atresia.

  14. The Changing Face of Esophageal Cancer

    PubMed Central

    Melhado, Rachel E.; Alderson, Derek; Tucker, Olga

    2010-01-01

    The two main histological esophageal cancer types, adenocarcinoma and squamous cell carcinoma, differ in incidence, geographic distribution, ethnic pattern and etiology. This article focuses on epidemiology with particular reference to geographic and temporal variations in incidence, along with a review of the evidence supporting environmental and genetic factors involved in esophageal carcinogenesis. Squamous cell carcinoma of the esophagus remains predominantly a disease of the developing world. In contrast, esophageal adenocarcinoma is mainly a disease of western developed societies, associated with obesity and gastro-esophageal reflux disease. There has been a dramatic increase in the incidence of adenocarcinoma in developed countries in parallel with migration of both esophageal and gastric adenocarcinomas towards the gastro-esophageal junction. PMID:24281163

  15. Diagnosis of osteopetrosis in bilateral congenital aural atresia: Turning point in treatment strategy

    PubMed Central

    Verma, Ritu; Jana, Manisha; Bhalla, Ashu Seith; Kumar, Arvind; Kumar, Rakesh

    2016-01-01

    Aural atresia is a rare congenital malformation of the external and middle ear. There are several syndromic associations of this anomaly with those involving the first and second branchial arches. Occurrence of aural atresia with sclerosing skeletal dysplasia is unknown and has never been reported. The co-existence of a sclerosing dysplasia can make the surgical treatment in aural atresia difficult and risky; and the auditory improvement may not be as expected. Moreover, internal auditory canal narrowing and hence sensorineural hearing loss in sclerosing dysplasia might add to the already existing conductive hearing loss in such patients. In this case report we have described an unknown association of bilateral microtia with sclerosing skeletal dysplasia (autosomal dominant osteopetrosis) and clinical implications of these two conditions occurring together leading to a change in the management plan. PMID:27170934

  16. Anaesthetic Management of a Patient with Synchronous Kartagener Syndrome and Biliary Atresia

    PubMed Central

    Kendigelen, Pınar; Tütüncü, Ayşe Çiğdem; Erbabacan, Şafak Emre; Kaya, Güner; Altındaş, Fatiş

    2015-01-01

    Kartagener syndrome is an autosomal recessive disorder characterized by primary ciliary dyskinesia accompanied by sinusitis, bronchiectasis, and situs inversus. Synchronous extrahepatic biliary atresia and Kartagener syndrome are very rare. During the preoperative preparation of patients with Kartagener syndrome, special attention is required for the respiratory and cardiovascular system. It is important to provide suitable anaesthetic management to avoid problems because of ciliary dysfunction in the perioperative period. Further, maintaining an effective pain control with regional anaesthetic methods reduces the risk of pulmonary complications. Infants with biliary atresia operated earlier have a higher chance of survival. Hepatic dysfunction and decrease in plasma proteins are important for the kinetics of drugs. In this presentation, the anaesthetic management of patients with synchronous Kartagener syndrome and biliary atresia, both of which are rare diseases, is evaluated. PMID:27366497

  17. Diagnosis of osteopetrosis in bilateral congenital aural atresia: Turning point in treatment strategy.

    PubMed

    Verma, Ritu; Jana, Manisha; Bhalla, Ashu Seith; Kumar, Arvind; Kumar, Rakesh

    2016-05-01

    Aural atresia is a rare congenital malformation of the external and middle ear. There are several syndromic associations of this anomaly with those involving the first and second branchial arches. Occurrence of aural atresia with sclerosing skeletal dysplasia is unknown and has never been reported. The co-existence of a sclerosing dysplasia can make the surgical treatment in aural atresia difficult and risky; and the auditory improvement may not be as expected. Moreover, internal auditory canal narrowing and hence sensorineural hearing loss in sclerosing dysplasia might add to the already existing conductive hearing loss in such patients. In this case report we have described an unknown association of bilateral microtia with sclerosing skeletal dysplasia (autosomal dominant osteopetrosis) and clinical implications of these two conditions occurring together leading to a change in the management plan. PMID:27170934

  18. Anaesthetic Management of a Patient with Synchronous Kartagener Syndrome and Biliary Atresia.

    PubMed

    Kendigelen, Pınar; Tütüncü, Ayşe Çiğdem; Erbabacan, Şafak Emre; Kaya, Güner; Altındaş, Fatiş

    2015-06-01

    Kartagener syndrome is an autosomal recessive disorder characterized by primary ciliary dyskinesia accompanied by sinusitis, bronchiectasis, and situs inversus. Synchronous extrahepatic biliary atresia and Kartagener syndrome are very rare. During the preoperative preparation of patients with Kartagener syndrome, special attention is required for the respiratory and cardiovascular system. It is important to provide suitable anaesthetic management to avoid problems because of ciliary dysfunction in the perioperative period. Further, maintaining an effective pain control with regional anaesthetic methods reduces the risk of pulmonary complications. Infants with biliary atresia operated earlier have a higher chance of survival. Hepatic dysfunction and decrease in plasma proteins are important for the kinetics of drugs. In this presentation, the anaesthetic management of patients with synchronous Kartagener syndrome and biliary atresia, both of which are rare diseases, is evaluated. PMID:27366497

  19. Scanning electron microscopic changes in granulosa cells during follicular atresia in Caprine ovary.

    PubMed

    Bhardwaj, J K; Sharma, R K

    2011-01-01

    During this study, topographic changes in healthy and atretic granulosa cells have been investigated during follicular atresia in goat ovary. Under scanning electron microscopy atresia was marked by asymmetrical shrinkage and vacuolization of cytoplasm. The specific topographical alterations observed in atretic cells were loss of micro extensions, disruption of cell-cell interaction, and smooth-textured membrane with a number of uneven depressions and ruffles. Some portions of the cell membrane were marked by extensive shrinkage due to condensation of cytosol. Irregular membrane at occasions was studded with blunt microextensions. The findings of present investigation will help in understanding the cellular changes in granulosa cells during follicular atresia and will find applications in screening of follicles for in vitro culture, in vitro fertilization and Embryo transfer technology. PMID:21254113

  20. Methoxychlor inhibits growth and induces atresia of antral follicles through an oxidative stress pathway.

    PubMed

    Gupta, Rupesh K; Miller, Kimberly P; Babus, Janice K; Flaws, Jodi A

    2006-10-01

    The mammalian ovary contains antral follicles, which are responsible for the synthesis and secretion of hormones that regulate estrous cyclicity and fertility. The organochlorine pesticide methoxychlor (MXC) causes atresia (follicle death via apoptosis) of antral follicles, but little is known about the mechanisms by which MXC does so. Oxidative stress is known to cause apoptosis in nonreproductive and reproductive tissues. Thus, we tested the hypothesis that MXC inhibits growth and induces atresia of antral follicles through an oxidative stress pathway. To test this hypothesis, antral follicles isolated from 39-day-old CD-1 mice were cultured with vehicle control (dimethylsulfoxide [DMSO]), MXC (1-100 microg/ml), or MXC + the antioxidant N-acetyl cysteine (NAC) (0.1-10 mM). During culture, growth was monitored daily. At the end of culture, follicles were processed for quantitative real-time polymerase chain reaction of Cu/Zn superoxide dismutase (SOD1), glutathione peroxidase (GPX), and catalase (CAT) mRNA expression or for histological evaluation of atresia. The results indicate that exposure to MXC (1-100 microg/ml) inhibited growth of follicles compared to DMSO controls and that NAC (1-10 mM) blocked the ability of MXC to inhibit growth. MXC induced follicular atresia, whereas NAC (1-10 mM) blocked the ability of MXC to induce atresia. In addition, MXC reduced the expression of SOD1, GPX, and CAT, whereas NAC reduced the effects of MXC on their expression. Collectively, these data indicate MXC causes slow growth and increased atresia by inducing oxidative stress.

  1. microRNA-222 modulates liver fibrosis in a murine model of biliary atresia

    SciTech Connect

    Shen, Wen-jun; Dong, Rui; Chen, Gong Zheng, Shan

    2014-03-28

    Highlights: • The RRV infected group showed cholestasis, retardation and extrahepatic biliary atresia. • miR-222 was highly expressed, and PPP2R2A was inhibited in the murine biliary atresia model. • miR-222 profoundly modulated the process of fibrosis in the murine biliary atresia model. • miR-222 might represent a potential target for improving biliary atresia prognosis. - Abstract: microRNA-222 (miR-222) has been shown to initiate the activation of hepatic stellate cells, which plays an important role in the pathogenesis of liver fibrosis. The aim of our study was to evaluate the role of miR-22 in a mouse model of biliary atresia (BA) induced by Rhesus Rotavirus (RRV) infection. New-born Balb/c mice were randomized into control and RRV infected groups. The extrahepatic bile ducts were evaluated. The experimental group was divided into BA group and negative group based on histology. The expression of miR-222, protein phosphatase 2 regulatory subunit B alpha (PPP2R2A), proliferating cell nuclear antigen (PCNA) and phospho-Akt were detected. We found that the experimental group showed signs of cholestasis, retardation and extrahepatic biliary atresia. No abnormalities were found in the control group. In the BA group, miR-222, PCNA and Akt were highly expressed, and PPP2R2A expression was significantly inhibited. Our findings suggest that miR-222 profoundly modulated the process of fibrosis in the murine BA model, which might represent a potential target for improving BA prognosis.

  2. From reflux esophagitis to Barrett's esophagus and esophageal adenocarcinoma.

    PubMed

    Wang, Rui-Hua

    2015-05-01

    The occurrence of gastroesophageal reflux disease is common in the human population. Almost all cases of esophageal adenocarcinoma are derived from Barrett's esophagus, which is a complication of esophageal adenocarcinoma precancerous lesions. Chronic exposure of the esophagus to gastroduodenal intestinal fluid is an important determinant factor in the development of Barrett's esophagus. The replacement of normal squamous epithelium with specific columnar epithelium in the lower esophagus induced by the chronic exposure to gastroduodenal fluid could lead to intestinal metaplasia, which is closely associated with the development of esophageal adenocarcinoma. However, the exact mechanism of injury is not completely understood. Various animal models of the developmental mechanisms of disease, and theoretical and clinical effects of drug treatment have been widely used in research. Recently, animal models employed in studies on gastroesophageal reflux injury have allowed significant progress. The advantage of using animal models lies in the ability to accurately control the experimental conditions for better evaluation of results. In this article, various modeling methods are reviewed, with discussion of the major findings on the developmental mechanism of Barrett's esophagus, which should help to develop better prevention and treatment strategies for Barrett's esophagus.

  3. Humans, Mice, and Mechanisms of Intestinal Atresias: A Window into Understanding Early Intestinal Development

    PubMed Central

    Nichol, Peter F.; Reeder, Amy; Botham, Robert

    2012-01-01

    Introduction Intestinal atresias have long been hypothesized to result from either failure of recanalization of the intestinal lumen or in utero vascular accidents. Recent work in animal models is now calling for a reassessment of these widely held paradigms. Purpose In this review, we will examine the data that led to the original hypotheses and then evaluate more recent work challenging these hypotheses. Furthermore, we will discuss how defining the mechanism of atresia formation in animal models may provide insight into early intestinal development and the mechanism of lengthwise intestinal growth. Conclusion Such insight will be critical in developing regenerative therapies for patients with intestinal failure. PMID:21116726

  4. Idiopathic neonatal hepatitis or extrahepatic biliary atresia? The role of liver biopsy.

    PubMed

    Kheir, Abdelmoneim Em; Ahmed, Wisal Ma; Gaber, Israa; Gafer, Sara Ma; Yousif, Badreldin M

    2016-01-01

    Cholestasis in early infancy represents a diagnostic dilemma and most of these infants suffer either from extrahepatic biliary atresia or idiopathic neonatal hepatitis. Differentiation between the two conditions may be extremely difficult both clinically and biochemically, and a diagnostic liver biopsy is usually required. We report on a Sudanese infant who presented at the age of 4 weeks with prolonged cholestatic jaundice, abdominal ultrasound was inconclusive, HIDA scan was suggestive of extrahepatic biliary atresia and the diagnosis of idiopathic neonatal hepatitis was only reached by liver biopsy. The infant made full recovery on supportive treatment during a one year follow up period. PMID:27651555

  5. [Stenting and redilatation of the ductus arteriosus in univentricular heart with pulmonary atresia. A case report].

    PubMed

    Hernández-González, Martha; Solorio, Sergio; Martínez-Sánchez, Arturo; Abundes, Arturo; Ledesma, Mariano; Alva, Carlos

    2002-01-01

    Complex congenital heart disease with pulmonary atresia is ductus arteriosus-dependent. Two palliative treatments have been available for years: a systemic pulmonary shunt and prostaglandin E1 infusion. Recently, interventional cardiology has offered a new procedure for these patients: stenting of the patent arterial duct. We report a case with univentricular heart and pulmonary atresia in which percutaneous balloon angioplasty and stenting of the arterial ductus were performed successfully. Nineteen months later the patient is in good clinical conditions with 75% of oxygen saturation.

  6. [CHARGE association: report of a clinical case with anal atresia and rectovaginal fistula].

    PubMed

    Rodonò, A; Romeo, M G; Sanges, G; Distefano, G

    1997-01-01

    CHARGE association is a non-random constellation of Coloboma, Heart Disease, Atresia of the choana, Retarded mental development and growth, Genital hypoplasia, Ear anomalies and deafness. We report on a newborn with CHARGE association. In addition to the typical features our case presented anal atresia and rectovaginal fistula. Our patient had congenital cardiopathy and feeding problems and died at the age of 3 months. Most case of CHARGE association appear to have a primary defect of pharyngeal incoordination that cause aspiration of secretion that is an important cause of death in the nonsurvivors.

  7. Idiopathic neonatal hepatitis or extrahepatic biliary atresia? The role of liver biopsy

    PubMed Central

    Ahmed, Wisal MA; Gaber, Israa; Gafer, Sara MA; Yousif, Badreldin M

    2016-01-01

    Cholestasis in early infancy represents a diagnostic dilemma and most of these infants suffer either from extrahepatic biliary atresia or idiopathic neonatal hepatitis. Differentiation between the two conditions may be extremely difficult both clinically and biochemically, and a diagnostic liver biopsy is usually required. We report on a Sudanese infant who presented at the age of 4 weeks with prolonged cholestatic jaundice, abdominal ultrasound was inconclusive, HIDA scan was suggestive of extrahepatic biliary atresia and the diagnosis of idiopathic neonatal hepatitis was only reached by liver biopsy. The infant made full recovery on supportive treatment during a one year follow up period. PMID:27651555

  8. Pradaxa-induced esophageal ulcer.

    PubMed

    Wood, Michele; Shaw, Paul

    2015-10-09

    Pradaxa (dabigatran) is a direct thrombin inhibitor approved for prevention of stroke and systemic embolism in patients with non-valvular atrial fibrillation. We describe a case of esophageal ulceration associated with Pradaxa administration in a 75-year-old man. The patient reported difficulty swallowing and a burning sensation after taking his first dose of Pradaxa. An esophagogastroduodenoscopy (EGD) revealed linear ulcerations in the mid-esophagus. Pradaxa was held beginning the day before the EGD. The patient reported that his pain and difficulty swallowing resolved on stopping Pradaxa. Pradaxa is formulated with a tartaric acid excipient to reduce variability in absorption. We hypothesise that the capsule lodged in the patient's esophagus and the tartaric acid may have caused local damage resulting in an esophageal ulcer. It is important to educate patients on proper administration of Pradaxa, to decrease the risk of this rare, but potentially serious adverse event.

  9. Allergic Mechanisms in Eosinophilic Esophagitis

    PubMed Central

    Wechsler, Joshua B; Bryce, Paul J

    2014-01-01

    Paralleling the overall trend in allergic diseases, Eosinophilic Esophagitis is rapidly increasing in incidence. It is associated with food antigen-triggered, eosinophil-predominant inflammation and the pathogenic mechanisms have many similarities to other chronic atopic diseases, such as eczema and allergic asthma. Studies in animal models and from patients over the last 15 years have suggested that allergic sensitization leads to food-specific IgE and T-helper lymphocyte type 2 cells, both of which appear to contribute to the pathogenesis along with basophils, mast cells, and antigen-presenting cells. This review will outline our current understandings of the allergic mechanisms that drive eosinophilic esophagitis, drawing from clinical and translational studies in humans as well as experimental animal models. PMID:24813516

  10. Distal esophageal spasm: an update.

    PubMed

    Achem, Sami R; Gerson, Lauren B

    2013-09-01

    Distal esophageal spasm (DES) is an esophageal motility disorder that presents clinically with chest pain and/or dysphagia and is defined manometrically as simultaneous contractions in the distal (smooth muscle) esophagus in ≥20% of wet swallows (and amplitude contraction of ≥30 mmHg) alternating with normal peristalsis. With the introduction of high resolution esophageal pressure topography (EPT) in 2000, the definition of DES was modified. The Chicago classification proposed that the defining criteria for DES using EPT should be the presence of at least two premature contractions (distal latency<4.5 s) in a context of normal EGJ relaxation. The etiology of DES remains insufficiently understood, but evidence links nitric oxide (NO) deficiency as a culprit resulting in a disordered neural inhibition. GERD frequently coexists in DES, and its role in the pathogenesis of symptoms needs further evaluation. There is some evidence from small series that DES can progress to achalasia. Treatment remains challenging due in part to lack of randomized placebo-controlled trials. Current treatment agents include nitrates (both short and long acting), calcium-channel blockers, anticholinergic agents, 5-phosphodiesterase inhibitors, visceral analgesics (tricyclic agents or SSRI), and esophageal dilation. Acid suppression therapy is frequently used, but clinical outcome trials to support this approach are not available. Injection of botulinum toxin in the distal esophagus may be effective, but further data regarding the development of post-injection gastroesophageal reflux need to be assessed. Heller myotomy combined with fundoplication remains an alternative for the rare refractory patient. Preliminary studies suggest that the newly developed endoscopic technique of per oral endoscopic myotomy (POEM) may also be an alternative treatment modality. PMID:23892829

  11. Acute Esophageal Necrosis: An Update

    PubMed Central

    Inayat, Faisal; Hurairah, Abu; Virk, Hafeez Ul Hassan

    2016-01-01

    Acute esophageal necrosis (AEN) or “black esophagus” is a rare clinical entity with an unclear etiology. It is diagnosed at upper gastrointestinal endoscopy with the presence of strikingly black necrotic esophagus. The treatment is primarily medical, but the prognosis is generally poor due to advanced age and comorbid illnesses in patients who develop AEN. Herein, we discussed the implications of poor glycemic control in regards with AEN and undertook a literature review of this rare diagnosis. PMID:27583242

  12. Acute Esophageal Necrosis: An Update.

    PubMed

    Inayat, Faisal; Hurairah, Abu; Virk, Hafeez Ul Hassan

    2016-07-01

    Acute esophageal necrosis (AEN) or "black esophagus" is a rare clinical entity with an unclear etiology. It is diagnosed at upper gastrointestinal endoscopy with the presence of strikingly black necrotic esophagus. The treatment is primarily medical, but the prognosis is generally poor due to advanced age and comorbid illnesses in patients who develop AEN. Herein, we discussed the implications of poor glycemic control in regards with AEN and undertook a literature review of this rare diagnosis. PMID:27583242

  13. ["Esophageal" angina and angina pectoris].

    PubMed

    Bortolotti, M; Labriola, E; Sarti, P; Brunelli, F; Mazza, M; Barbara, L

    1991-04-15

    In the last few years the non cardiac angina-like chest pain has encompassed more and more agitation not only in many patients but also in cardiologists, gastroenterologists and psychologists, as it involves socio-economic, pathophysiologic and therapeutic problems. The socio-economic aspect is well explained by the fact that in the USA at least 200,000 patients a year suffering from non cardiac angina-like chest pain, even when coronary arteriography has demonstrated normal coronary vessels, nevertheless continue to require cardiologic examinations and, if no one has clearly demonstrated the origin of their pain, they continue to live as invalids in constant fear of myocardial infarction. The discovery that the esophagus may be one of the causes of chest pain in these patients presenting with a previous diagnosis of "atypical" angina pectoris, unfortunately cannot resolve definitively the problem. An association of esophageal angina in patients with angina pectoris treated for long periods of time with Ca-antagonists and nitroderivatives has been described. In addition, the provocative or spontaneous tests to demonstrate the esophageal origin of chest pain give only a "likely" and not a "definite" diagnosis of esophageal angina. This also means to no "gold standard" text exist. Lastly, the "likely" diagnosis of esophageal angina is made in only about 50% of patients leaving the problem of the remaining 50% unanswered. These uncertainties induce some psychologists to assert that the cause of non cardiac angina-like chest pain is in the head ("panic disorder") and not in the esophagus, where the observed motor disorders should be an epiphenomenon.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:2067672

  14. Incremental Value of MR Cholangiopancreatography in Diagnosis of Biliary Atresia

    PubMed Central

    Sung, Siyoun; Jeon, Tae Yeon; Yoo, So-Young; Hwang, Sook Min; Choi, Young Hun; Kim, Woo Sun; Choe, Yon Ho; Kim, Ji Hye

    2016-01-01

    Purpose To evaluate the incremental value of a combination of magnetic resonance cholangiopancreatography (MRCP) and ultrasonography (US), compared to US alone, for diagnosing biliary atresia (BA) in neonates and young infants with cholestasis. Materials and Methods The institutional review board approved this retrospective study. The US and MRCP studies were both performed on 64 neonates and young infants with BA (n = 41) or without BA (non-BA) (n = 23). Two observers reviewed independently the US alone set and the combined US and MRCP set, and graded them using a five-point scale. Diagnostic performance was compared using pairwise comparison of the receiver operating characteristics (ROC) curve. The sensitivity, specificity, accuracy, positive predictive value (PPV), and negative predictive value were assessed. Results The diagnostic performance (the area under the ROC curve [Az]) for diagnosing BA improved significantly after additional review of MRCP images; Az improved from 0.688 to 0.901 (P = .015) for observer 1 and from 0.676 to 0.901 (P = .011) for observer 2. The accuracy of MRCP combined with US (observer 1, 95% [61/64]; observer 2 92% [59/64]) and PPV (observer 1, 95% [40/42]; observer 2 91% [40/44]) were significantly higher than those of US alone for both observers (accuracy: observer 1, 73% [47/64], P = 0.003; observer 2, 72% [46/64], P = 0.004; PPV: observer 1, 76% [35/46], P = 0.016; observer 2, 76% [34/45], P = 0.013). Interobserver agreement of confidence levels was good for US alone (ĸ = 0.658, P < .001) and was excellent for the combined set of US and MRCP (ĸ = 0.929, P < .001). Conclusion Better diagnostic performance was achieved with the combination of US and MRCP than with US alone for the evaluation of BA in neonates and young infants with cholestasis. PMID:27341698

  15. Novel device to sample the esophageal microbiome--the esophageal string test.

    PubMed

    Fillon, Sophie A; Harris, J Kirk; Wagner, Brandie D; Kelly, Caleb J; Stevens, Mark J; Moore, Wendy; Fang, Rui; Schroeder, Shauna; Masterson, Joanne C; Robertson, Charles E; Pace, Norman R; Ackerman, Steven J; Furuta, Glenn T

    2012-01-01

    A growing number of studies implicate the microbiome in the pathogenesis of intestinal inflammation. Previous work has shown that adults with esophagitis related to gastroesophageal reflux disease have altered esophageal microbiota compared to those who do not have esophagitis. In these studies, sampling of the esophageal microbiome was accomplished by isolating DNA from esophageal biopsies obtained at the time of upper endoscopy. The aim of the current study was to identify the esophageal microbiome in pediatric individuals with normal esophageal mucosa using a minimally invasive, capsule-based string technology, the Enterotest™. We used the proximal segment of the Enterotest string to sample the esophagus, and term this the "Esophageal String Test" (EST). We hypothesized that the less invasive EST would capture mucosal adherent bacteria present in the esophagus in a similar fashion as mucosal biopsy. EST samples and mucosal biopsies were collected from children with no esophageal inflammation (n = 15) and their microbiome composition determined by 16S rRNA gene sequencing. Microbiota from esophageal biopsies and ESTs produced nearly identical profiles of bacterial genera and were different from the bacterial contents of samples collected from the nasal and oral cavity. We conclude that the minimally invasive EST can serve as a useful device for study of the esophageal microbiome. PMID:22957025

  16. Pharmacologic influence on esophageal varices

    SciTech Connect

    Lunderquist, A.; Owman, T.; Alwmark, A.; Gullstrand, P.; Hall-Angeras, M.; Joelsson, B.; Tranberg, K.G.; Pettersson, K.I.

    1983-06-01

    Selective catherization of the left gastric vein was performed after percutaneous transhepatic portography (PTP) in patients with portal hypertension and esophageal varices. Following the hypothesis that drugs increasing the lower esophageal sphincter (LES) pressure may obstruct the variceal blood flow throught the lower esophagus, the effect of different drugs (i.e., intravenous injection of vasopressin, pentagastrin, domperidone and somatostatin and subcutaneous injection of metacholine) on the variceal blood flow was examined. Vasopressin did not change the variceal blood flow; pentagastrine, with its known effect of increasing the LES pressure produced a total interruption of the flow in four of eight patients; domperiodone, also known to increase the LES pressure obstructed the variceal blood flow in the only patient examined with this drug; somatostatin has no reported action on the LES but blocked the flow in one of two patients; and metacholine, reported to increase the LES pressure did not produce any change in the flow in the three patients examined. LES pressure was recorded before and during vasopressin infusion in seven patients with portal hypertension and esophageal varices. No reaction on the pressure was found. The patient number in the study is small and the results are nonuniform but still they suggest that drugs increasing the LES tonus might be useful to control variceal blood flow.

  17. Clinical Application of Esophageal High-resolution Manometry in the Diagnosis of Esophageal Motility Disorders

    PubMed Central

    van Hoeij, Froukje B; Bredenoord, Albert J

    2016-01-01

    Esophageal high-resolution manometry (HRM) is replacing conventional manometry in the clinical evaluation of patients with esophageal symptoms, especially dysphagia. The introduction of HRM gave rise to new objective metrics and recognizable patterns of esophageal motor function, requiring a new classification scheme: the Chicago classification. HRM measurements are more detailed and more easily performed compared to conventional manometry. The visual presentation of acquired data improved the analysis and interpretation of esophageal motor function. This led to a more sensitive, accurate, and objective analysis of esophageal motility. In this review we discuss how HRM changed the way we define and categorize esophageal motility disorders. Moreover, we discuss the clinical applications of HRM for each esophageal motility disorder separately. PMID:26631942

  18. Pralatrexate and Oxaliplatin in Treating Patients With Unresectable or Metastatic Esophageal, Stomach, or Gastroesophageal Junction Cancer

    ClinicalTrials.gov

    2016-01-11

    Adenocarcinoma of the Gastroesophageal Junction; Esophageal Undifferentiated Carcinoma; Gastric Adenocarcinoma; Gastric Squamous Cell Carcinoma; Recurrent Esophageal Adenocarcinoma; Recurrent Esophageal Squamous Cell Carcinoma; Recurrent Gastric Carcinoma; Stage IIIB Esophageal Adenocarcinoma; Stage IIIB Esophageal Squamous Cell Carcinoma; Stage IIIB Gastric Cancer; Stage IIIC Esophageal Adenocarcinoma; Stage IIIC Esophageal Squamous Cell Carcinoma; Stage IIIC Gastric Cancer; Stage IV Esophageal Adenocarcinoma; Stage IV Esophageal Squamous Cell Carcinoma; Stage IV Gastric Cancer; Undifferentiated Gastric Carcinoma

  19. Ovarian follicular atresia is mediated by heterophagy, autophagy, and apoptosis in Prochilodus argenteus and Leporinus taeniatus (Teleostei: Characiformes).

    PubMed

    Santos, H B; Thomé, R G; Arantes, F P; Sato, Y; Bazzoli, N; Rizzo, E

    2008-12-01

    We investigated apoptosis, cell proliferation antigen (PCNA), and heat shock protein (HSP70) during ovarian follicular atresia in two freshwater teleost species from the São Francisco River basin, Brazil: curimatã-pacu, Prochilodus argenteus and piau-jejo, Leporinus taeniatus. Fishes were maintained in captivity after the reproductive period and ovarian regression was assessed by gonadosomatic index for three stages: early, advanced, and late regression. Follicular atresia was analysed by light and transmission electron microscopy, as well as by TUNEL and immunohistochemistry for HSP70 and PCNA. During early regression, atretic follicles exhibited zona pellucida breakdown, yolk degeneration, and hypertrophied follicular cells (e.g., granulosa in mammals). Intense heterophagy to engulf the yolk, and autophagy were detected in the follicular cells during advanced and late atresia. The TUNEL assay detected DNA fragmentation, mainly in late follicular atresia. The apoptosis rate of the follicular cells increased up to 10% during follicular atresia in both species and was negatively correlated with follicular area. Immunohistochemistry reaction for HSP70 stained the follicular cells strongly during advanced atresia, when they are intensively involved in yolk engulfment, whereas the reaction for PCNA labelled theca cells. We inferred that heterophagy, autophagy, and apoptosis contributed to follicular atresia in teleost ovaries, thereby achieving a more efficient removal of the degenerating oocyte and dying follicular cells. Additionally, HSP70 may protect the follicular cells before apoptosis when they are involved in yolk engulfment, and cell proliferation in the theca contributed to ovarian remodelling. PMID:18701155

  20. 21 CFR 868.1910 - Esophageal stethoscope.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Esophageal stethoscope. 868.1910 Section 868.1910 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES ANESTHESIOLOGY DEVICES Diagnostic Devices § 868.1910 Esophageal stethoscope....

  1. 21 CFR 868.1910 - Esophageal stethoscope.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Esophageal stethoscope. 868.1910 Section 868.1910 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES ANESTHESIOLOGY DEVICES Diagnostic Devices § 868.1910 Esophageal stethoscope....

  2. 21 CFR 868.1910 - Esophageal stethoscope.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Esophageal stethoscope. 868.1910 Section 868.1910 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES ANESTHESIOLOGY DEVICES Diagnostic Devices § 868.1910 Esophageal stethoscope....

  3. 21 CFR 868.1910 - Esophageal stethoscope.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Esophageal stethoscope. 868.1910 Section 868.1910 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES ANESTHESIOLOGY DEVICES Diagnostic Devices § 868.1910 Esophageal stethoscope....

  4. 21 CFR 868.1910 - Esophageal stethoscope.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 8 2013-04-01 2013-04-01 false Esophageal stethoscope. 868.1910 Section 868.1910 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES ANESTHESIOLOGY DEVICES Diagnostic Devices § 868.1910 Esophageal stethoscope....

  5. 21 CFR 878.3610 - Esophageal prosthesis.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 8 2013-04-01 2013-04-01 false Esophageal prosthesis. 878.3610 Section 878.3610 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3610 Esophageal...

  6. 21 CFR 878.3610 - Esophageal prosthesis.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Esophageal prosthesis. 878.3610 Section 878.3610 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3610 Esophageal...

  7. 21 CFR 878.3610 - Esophageal prosthesis.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Esophageal prosthesis. 878.3610 Section 878.3610 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3610 Esophageal...

  8. 21 CFR 878.3610 - Esophageal prosthesis.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Esophageal prosthesis. 878.3610 Section 878.3610 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3610 Esophageal...

  9. 21 CFR 878.3610 - Esophageal prosthesis.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Esophageal prosthesis. 878.3610 Section 878.3610 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3610 Esophageal...

  10. Esophageal Impedance Monitoring: Clinical Pearls and Pitfalls.

    PubMed

    Ravi, Karthik; Katzka, David A

    2016-09-01

    The development of intraluminal esophageal impedance monitoring has improved our ability to detect and measure gastroesophageal reflux without dependence on acid content. This ability to detect previously unrecognized weak or nonacid reflux episodes has had important clinical implications in the diagnosis and management of gastroesophageal reflux disease (GERD). In addition, with the ability to assess bolus transit within the esophageal lumen, impedance monitoring has enhanced the recognition and characterization of esophageal motility disorders in patients with nonobstructive dysphagia. The assessment of the intraluminal movement of gas and liquid has also been proven to be of diagnostic value in conditions such as rumination syndrome and excessive belching. Further, alternative applications of impedance monitoring, such as the measurement of mucosal impedance, have provided novel insights into assessing esophageal mucosal integrity changes as a consequence of inflammatory change. Future applications for esophageal impedance monitoring also hold promise in esophageal conditions other than GERD. However, despite all of the clinical benefits afforded by esophageal impedance monitoring, important clinical and technical shortcomings limit its diagnostic value and must be considered when interpreting study results. Overinterpretation of studies or application of impedance monitoring in patients can have deleterious clinical implications. This review will highlight the clinical benefits and limitations of esophageal impedance monitoring and provide clinical pearls and pitfalls associated with this technology.

  11. 21 CFR 876.5365 - Esophageal dilator.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Esophageal dilator. 876.5365 Section 876.5365 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GASTROENTEROLOGY-UROLOGY DEVICES Therapeutic Devices § 876.5365 Esophageal dilator....

  12. Esophageal Impedance Monitoring: Clinical Pearls and Pitfalls.

    PubMed

    Ravi, Karthik; Katzka, David A

    2016-09-01

    The development of intraluminal esophageal impedance monitoring has improved our ability to detect and measure gastroesophageal reflux without dependence on acid content. This ability to detect previously unrecognized weak or nonacid reflux episodes has had important clinical implications in the diagnosis and management of gastroesophageal reflux disease (GERD). In addition, with the ability to assess bolus transit within the esophageal lumen, impedance monitoring has enhanced the recognition and characterization of esophageal motility disorders in patients with nonobstructive dysphagia. The assessment of the intraluminal movement of gas and liquid has also been proven to be of diagnostic value in conditions such as rumination syndrome and excessive belching. Further, alternative applications of impedance monitoring, such as the measurement of mucosal impedance, have provided novel insights into assessing esophageal mucosal integrity changes as a consequence of inflammatory change. Future applications for esophageal impedance monitoring also hold promise in esophageal conditions other than GERD. However, despite all of the clinical benefits afforded by esophageal impedance monitoring, important clinical and technical shortcomings limit its diagnostic value and must be considered when interpreting study results. Overinterpretation of studies or application of impedance monitoring in patients can have deleterious clinical implications. This review will highlight the clinical benefits and limitations of esophageal impedance monitoring and provide clinical pearls and pitfalls associated with this technology. PMID:27325223

  13. Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals Associated with Left Pulmonary Artery Interruption

    PubMed Central

    Mun, Da-Na; Park, Chun Soo; Kim, Young-Hwue; Goo, Hyun Woo

    2016-01-01

    A multistage plan and multidisciplinary approach are the keys to successful repair in patients with pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs). In this article, we present a multidisciplinary approach adopted to treat a patient with PA with VSD and MAPCAs associated with left pulmonary artery interruption. PMID:27733998

  14. [Scimitar syndrome associated to pulmonary atresia with interventricular communication. First reported case].

    PubMed

    Alva, Carlos; Valero, Gillermo; Martínez, Arturo; Riera, Carlos; David, Felipe; Jiménez, Santiago; Ortegón, José; Sánchez, Agustín; López, Diana; Ramírez, Erick

    2004-01-01

    We present the case of a 15 months-old male with Scimitar Syndrome associated with ventricular septal defect and pulmonary atresia. The diagnosis was made by cardiac catheterization and angiography and was confirmed by the necropsy. Clinical and surgical considerations of this exceptional association were made. To the best of our knowledge this is the first case reported in the relevant literature.

  15. Maternal Residential Atrazine Exposure and Risk for Choanal Atresia and Stenosis in Offspring

    PubMed Central

    Agopian, A.J.; Cai, Yi; Langlois, Peter H.; Canfield, Mark A.; Lupo, Philip J.

    2014-01-01

    Objective To assess the relationship between estimated residential maternal exposure to atrazine during pregnancy and risk for choanal atresia or stenosis in offspring. Study Design Data for 280 nonsyndromic cases and randomly selected, population-based controls delivered during 1999 to 2008 were obtained from the Texas Birth Defects Registry. County-level estimates of atrazine levels obtained from the United States Geological Survey were assigned to cases and controls based on maternal county of residence at delivery. Unconditional logistic regression was used to assess the relationship between maternal residential atrazine exposure and risk for choanal atresia or stenosis in offspring. Results Compared to offspring of mothers with low levels of estimated residential atrazine exposure, those with high levels had nearly a two-fold increase in risk for choanal atresia or stenosis (adjusted odds ratio: 1.79, 95% confidence interval: 1.17–2.74). A significant linear trend was also observed with increasing levels of atrazine exposure (adjusted P = 0.002). Conclusions A link between maternal exposure to endocrine disruptors, such as atrazine, and choanal atresia risk is plausible based on previous findings. Our results further support this hypothesis. PMID:23036484

  16. Coexisting urogenital anomaly and duodenal atresia in two atypical Holt–Oram syndrome

    PubMed Central

    Ali, Tuncer Ahmet; Afra, Karavelioğlu; Didem, Baskin Embleton; Muhsin, Elmas

    2016-01-01

    Holt–Oram syndrome (HOS) is a rare autosomal dominant disorder, characterized by upper limb dysplasia and congenital cardiac defect. We report two cases with HOS, first associated with renal agenesis, coronal hypospadias, urethral duplication and second associated with duodenal atresia and horseshoe kidney that have not been reported in English literature.

  17. Coexisting urogenital anomaly and duodenal atresia in two atypical Holt–Oram syndrome

    PubMed Central

    Ali, Tuncer Ahmet; Afra, Karavelioğlu; Didem, Baskin Embleton; Muhsin, Elmas

    2016-01-01

    Holt–Oram syndrome (HOS) is a rare autosomal dominant disorder, characterized by upper limb dysplasia and congenital cardiac defect. We report two cases with HOS, first associated with renal agenesis, coronal hypospadias, urethral duplication and second associated with duodenal atresia and horseshoe kidney that have not been reported in English literature. PMID:27695215

  18. Agenesis of the dorsal mesentery without jejunoilial atresia ("apple peel small bowel").

    PubMed

    Hull, J D; Kiesel, J L; Proudfoot, W H; Belin, R P

    1975-04-01

    Agenesis of the dorsal mesentery with apple peel or Christmas tree deformity but without small-bowel atresia can occur beyond the neonatal period. The recognition of this entity is imperative as it is also associated with a marginal artery which may be the only blood supply to the majority of small bowel. Preservation of this vessel is necessary to avoid catastrophic bowel death.

  19. [Endoscopic surgery for benign esophageal diseases].

    PubMed

    Ozawa, Soji

    2006-07-01

    Gastroesophageal reflux disease (GERD) and esophageal achalasia are common benign esophageal diseases. Today minimally invasive surgery is recommended to treat these diseases. Surgical indications for GERD are failure of medical management, medical complications attributable to a large hiatal hernia, 'atypical' symptoms (asthma, hoarseness, cough, chest pain, aspiration), etc. according to the Society of American Gastrointestinal Endoscopic Surgeons (SAGES) guidelines. Laparoscopic Nissen fundoplication has emerged as the most widely accepted procedure for GERD patients with normal esophageal motility. Partial fundoplication (e.g., Toupet fundoplication) is also considered to decrease the possibility of postoperative dysphagia. Although pneumatic dilatation has been the first line treatment for esophageal achalasia, laparoscopic Heller myotomy and partial fundoplication (e.g., Dor fundoplication) to prevent reflux is preferred by most gastroenterologists and surgeons as the primary treatment modality. Laparoscopic surgery for GERD and esophageal achalasia are effective in most patients and safe in all patients. Finally, laparoscopic surgery should be performed only by skilled surgeons.

  20. Unusual Presentation of a Metastatic Esophageal Carcinoma

    PubMed Central

    Orlicka, Katarzyna; Maynard, Stéphanie; Bouin, Mickael

    2012-01-01

    Esophageal cancer most commonly presents with upper digestive symptoms such as dysphagia. Lymph nodes are among the most common metastatic sites of this type of cancer. We report the case of a 53-year-old man presenting with unusual sole presenting features of esophageal cancer. The patient sought medical attention for abdominal pain without dysphagia, which was first investigated with an abdominal computed tomography scan. A large abdominal mass was discovered on imaging. Biopsies of this mass were in keeping with esophageal squamous cell cancer. With this finding, gastroscopy was performed, confirming the presence of primary esophageal cancer. This is a rare presentation of esophageal cancer without upper gastrointestinal symptoms. This case reinforces the value of biopsy for any neoplastic mass, especially in a context of unusual symptoms. PMID:22679417

  1. [Clinical features and pathophysiology of acute esophageal mucosal lesion].

    PubMed

    Ihara, Yutaro; Hizawa, Kazuoki; Fujita, Kouhei; Matsuno, Yuichi; Sakuma, Tsutomu; Esaki, Motohiro; Iida, Mitsuo

    2016-04-01

    Acute esophageal mucosal lesions (AEMLs) are categorized into black esophagitis (type B) and non-black esophagitis (type NB) on endoscopy. To clarify the distinct pathophysiology, we compared the clinical features and hematological findings at onset among 17 patients with type B esophagitis and 6 patients with type NB esophagitis. In type B esophagitis, time to endoscopy after onset was significantly shorter, and blood levels of lactate, urea nitrogen, creatinine, and glucose were higher than in type NB esophagitis. However, there were no significant intergroup differences in the incidences of other predisposing factors, such as diabetic ketoacidosis or esophageal hernias. These findings suggest that AEMLs are caused by acid reflux and peripheral vascular insufficiency, the latter being more associated with type B esophagitis by its etiology. In addition, blood lactate may indicate the severity of AEML, leading to black esophagitis. PMID:27052393

  2. The Role of ARF6 in Biliary Atresia

    PubMed Central

    Glessner, Joseph; Ashokkumar, Chethan; Ranganathan, Sarangarajan; Min, Jun; Higgs, Brandon W.; Sun, Qing; Haberman, Kimberly; Schmitt, Lori; Vilarinho, Silvia; Mistry, Pramod K.; Vockley, Gerard; Dhawan, Anil; Gittes, George K.; Hakonarson, Hakon; Jaffe, Ronald; Subramaniam, Shankar; Shin, Donghun; Sindhi, Rakesh

    2015-01-01

    Background & Aims Altered extrahepatic bile ducts, gut, and cardiovascular anomalies constitute the variable phenotype of biliary atresia (BA). Methods To identify potential susceptibility loci, Caucasian children, normal (controls) and with BA (cases) at two US centers were compared at >550000 SNP loci. Systems biology analysis was carried out on the data. In order to validate a key gene identified in the analysis, biliary morphogenesis was evaluated in 2-5-day post-fertilization zebrafish embryos after morpholino-antisense oligonucleotide knockdown of the candidate gene ADP ribosylation factor-6 (ARF6, Mo-arf6). Results Among 39 and 24 cases at centers 1 and 2, respectively, and 1907 controls, which clustered together on principal component analysis, the SNPs rs3126184 and rs10140366 in a 3’ flanking enhancer region for ARF6 demonstrated higher minor allele frequencies (MAF) in each cohort, and 63 combined cases, compared with controls (0.286 vs. 0.131, P = 5.94x10-7, OR 2.66; 0.286 vs. 0.13, P = 5.57x10-7, OR 2.66). Significance was enhanced in 77 total cases, which included 14 additional BA genotyped at rs3126184 only (p = 1.58x10-2, OR = 2.66). Pathway analysis of the 1000 top-ranked SNPs in CHP cases revealed enrichment of genes for EGF regulators (p<1 x10-7), ERK/MAPK and CREB canonical pathways (p<1 x10-34), and functional networks for cellular development and proliferation (p<1 x10-45), further supporting the role of EGFR-ARF6 signaling in BA. In zebrafish embryos, Mo-arf6 injection resulted in a sparse intrahepatic biliary network, several biliary epithelial cell defects, and poor bile excretion to the gall bladder compared with uninjected embryos. Biliary defects were reproduced with the EGFR-blocker AG1478 alone or with Mo-arf6 at lower doses of each agent and rescued with arf6 mRNA. Conclusions The BA-associated SNPs identify a chromosome 14q21.3 susceptibility locus encompassing the ARF6 gene. arf6 knockdown in zebrafish implicates early biliary

  3. Giant mid-esophageal diverticulum. Conservative treatment of postoperative leakage.

    PubMed

    Dallatomasina, S; Casaccia, M; Chessa, M; Serrano, J; Nardi, I; Troilo, B; Miggino, M; Valente, U

    2009-01-01

    Mid-esophageal diverticula are rare entities. Only symptomatic patients usually receive surgical treatment. Esophageal leakage is one of the most common complications after these procedures. Though in literature, operative management is the preferred treatment for esophageal fistula, conservative approach is described in case of small leaks. We report a case of an operated giant mid-esophageal diverticulum complicated with an esophageal fistula. The patient underwent a surgical treatment and recovered completely.

  4. Role of advanced diagnostics for eosinophilic esophagitis.

    PubMed

    Hirano, Ikuo

    2014-01-01

    In eosinophilic esophagitis (EoE), diagnostic tests aid in the identification of pathophysiologic consequences and accurate detection of the disease. The EoE Endoscopic Reference Score (EREFS) classifies and grades the severity of the five major endoscopically identified esophageal features of EoE (edema, rings, exudates, furrows and strictures). The EREFS may be useful in the evaluation of disease severity and as an objective outcome of response to therapy. pH monitoring identifies the presence of abnormal degrees of acid exposure in the esophagus that characterizes gastroesophageal reflux disease. The presence of acid reflux, however, does not indicate that the reflux is responsible for esophageal eosinophilia. Esophageal manometry has not demonstrated a characteristic abnormality with sufficient sensitivity to make the test of diagnostic value in clinical practice. On the other hand, manometric characteristics of esophageal pressurization and longitudinal muscle dysfunction may help identify important pathophysiologic consequences of EoE. Esophageal impedance testing has demonstrated increased baseline mucosal impedance that correlates with increased epithelial permeability in EoE. Reduced mucosal integrity may provide intraluminal allergens access to antigen-presenting cells, serving as an early event in the pathogenesis of EoE. The functional luminal impedance probe (FLIP) provides quantitative assessment of esophageal mural compliance, a physiologic correlate of remodeling in EoE. Studies using FLIP have associated reductions in esophageal distensibility in EoE with the important outcome of food impaction risk. Finally, confocal endomicroscopy, multiphoton fluorescence microscopy and novel eosinophil-enhancing contrast agents are emerging methods that may allow for in vivo visualization of esophageal eosinophilic inflammation, thereby improving the detection and understanding of this emerging disease. PMID:24603385

  5. A solitary large radioiodine accumulative lung lesion in high-dose 131i therapeutic scan: bronchial atresia with mucocele.

    PubMed

    Lee, Won Hyoung; Park, Jung Mi; Kwak, Jeong Ja

    2015-02-01

    We reported a large radioiodine accumulative lung lesion on I therapeutic whole-body scan performed in a 50-year-old woman for thyroid cancer ablation therapy. Previously, her chest radiography and contrast-enhanced chest CT images showed bronchial atresia in the left upper lobar bronchus and mucus-filled dilated distal bronchus. Bronchial mucocele was confirmed by CT-guided percutaneous transthoracic needle aspiration. Bronchial atresia is a rare congenital abnormality associated with the mucocele.

  6. [Oral blastomycosis, laryngeal papillomatosis and esophageal tuberculosis].

    PubMed

    Montoya, Manuel; Chumbiraico, Robert; Ricalde, Melvin; Cazorla, Ernesto; Hernández-Córdova, Gustavo

    2012-06-01

    Esophageal involvement is an extremely rare complication of tuberculosis even in countries with high prevalence of infection. We report the case of a 57 year-old hiv-seronegative patient with simultaneous diagnoses of oral blastomycosis and laryngeal papillomatosis. Both were confirmed by anatomopathological analysis. The esophageal biopsy revealed granulomatous esophagitis with necrosis and ziehl-neelsen stain showed acid-fast alcohol resistant bacilli suggestive of tuberculosis. The patient's history included pulmonary tuberculosis twice and previous abandonment of therapy. Thus, it was necessary to use oral itraconazole combined with second-line anti-tuberculosis drugs administered through a gastrostomy tube. The clinical development was favorable. PMID:22858774

  7. Updates on esophageal and gastric cancers

    PubMed Central

    Gallo, Amy; Cha, Charles

    2006-01-01

    Esophageal and gastric cancers are both common and deadly. Patients present most often after disease progression and survival is therefore poor. Due to demographic variability and recent changes in disease incidence, much emphasis has been placed on studying risk factors for both esophageal and gastric cancers. However, with increasing understanding of these diseases, low survival rates persist and continued intensive studies are necessary to optimize treatment plans. This review article discusses updates in the evolving epidemiology, clinical presentation, risk factors, and diagnostic and treatment modalities of esophageal and gastric cancers. PMID:16718845

  8. Esophageal stent placement as a therapeutic option for iatrogenic esophageal perforation in children

    PubMed Central

    Ahmad, Alsafadi; Wong Kee Song, Louis M.; Absah, Imad

    2016-01-01

    Iatrogenic esophageal perforation (IEP) is a potentially serious adverse event of interventional endoscopy. The approach to IEP varies from surgical repair for large perforations to conservative treatment for small contained perforations. We report a case of an 18-month-old girl with congenital esophageal stenosis suffering a large esophageal perforation after a trial of stricture dilatation, which was successfully managed by the placement of fully covered stent. Hence, in selected cases, esophageal stent placement is a feasible alternative to invasive surgery in managing IEP. PMID:27144142

  9. [Laparoscopic surgery for esophageal achalasia].

    PubMed

    Ozawa, S; Ando, N; Ohgami, M; Kitagawa, Y; Kitajima, M

    2000-04-01

    Laparoscopic surgery for esophageal achalasia was first reported by Shimi et al. in 1991. Subsequently the procedure has been performed all over the world and laparoscopic Heller myotomy and Dor fundoplication (Heller and Dor operation) is now thought to be the operation of first choice. It is indicated for patients who are resistant to medical therapy (calcium blocker etc.) or have pneumatic dilatation and those with frequent aspiration at night. As Csendes et al. reported that surgical treatment was better than pneumatic dilatation and as laparoscopic surgery is less invasive, the indications for the laparoscopic Heller and Dor operation can include all achalasia patients except those who respond to medical therapy, do not accept surgery, or cannot tolerate surgery. We successfully performed the laparoscopic Heller and Dor operation on 22 patients, all of whom had an uneventful postoperative course. Manometric evaluation, endoscopic examination, and 24-hour pH monitoring showed good results. There are six important technical points: 1) flexible laparoscopy; 2) pneumoperitoneum; 3) gauze in the abdominal cavity to absorb blood; 4) laparosonic coagulating shears; 5) extracorporeal knot-tying technique; and 6) intracorporeal knot-tying technique. If an experienced surgeon is in charge, the laparoscopic Heller and Dor operation is an ideal, minimally invasive treatment for esophageal achalasia.

  10. Methoxychlor inhibits growth and induces atresia through the aryl hydrocarbon receptor pathway in mouse ovarian antral follicles.

    PubMed

    Basavarajappa, Mallikarjuna S; Hernández-Ochoa, Isabel; Wang, Wei; Flaws, Jodi A

    2012-08-01

    Methoxychlor (MXC) is an organochlorine pesticide used against pests that attack crops, vegetables, and livestock. MXC inhibits growth and induces atresia (death) of mouse ovarian antral follicles in vitro. Since several studies indicate that many chemicals act through the aryl hydrocarbon receptor (AHR) pathway, the current study tested the hypothesis that MXC binds to the AHR to inhibit growth and induce atresia of antral follicles. The data indicate that MXC binds to AHR. Further, a relatively high dose of MXC (100μg/ml) inhibits growth and induces atresia in both wild-type (WT) and AHR null (AHRKO) follicles, whereas a lower dose of MXC (10μg/ml) inhibits growth and induces atresia in WT, but not in AHRKO follicles. These data indicate that AHR deletion partially protects antral follicles from MXC induced slow growth and atresia. Collectively, these data show that MXC may act through the AHR pathway to inhibit follicle growth and induce atresia in antral follicles of the ovary.

  11. Palliative Treatment of Esophageal Cancer.

    PubMed

    Ahmad; Goosenberg; Frucht; Coia

    1994-07-01

    Palliative interventions for advanced esophageal cancer include surgery, radiation therapy, chemotherapy, chemoradiation, endoscopic procedures, and combinations of the above. Palliative esophagectomy or bypass procedures are difficult to justify in these patients because their life expectancy is so short. Palliative external beam radiation to doses of 50 to 60 Gy is successful in 50% to 70% of patients. The addition of brachytherapy may improve these results. One third to one half of patients treated with radiation develop benign or maglinant stricture. Although response rates to combination chemotherapy are only 50% at best, the majority of patients do have improvement of dysphagia. These regimens are commonly used as part of a multidisciplinary approach with radiation andøor surgery, rather than as a sole modality of treatment. Chemoradiation regimens results in better survival than treatment with radiation alone, and provide palliation of dysphagia in up to 90% of patients. Although acute toxicity of chemoradiation is more severe than radiation alone, this is of limited duration. Chemoradiation may be the treatment of choice for the majority of patients with locally advanced esophageal cancer. Endoscopic techniques are available that provide palliation of dysphagia. The most commonly used technique is esophageal dilatation, either alone or before performing other palliative procedures such as laser therapy or stent placement. The most significant limitation of dilatation alone is that palliation is short-lived and most patients require repeat dilatations. Esophageal stents offer a high degree of palliation, but procedure-related morbidity and mortality rates are not insignificant. Expandable metal stents are associated with few complications but tumor ingrowth through the metallic mesh is frequent. Conventional plastic stents are not affected by tumor ingrowth but can migrate. Endoscopic laser therapy also provides symptoms relief and complication rates are

  12. Optimizing the US Diagnosis of Biliary Atresia with a Modified Triangular Cord Thickness and Gallbladder Classification.

    PubMed

    Zhou, Lu-Yao; Wang, Wei; Shan, Quan-yuan; Liu, Bao-xian; Zheng, Yan-ling; Xu, Zuo-feng; Xu, Ming; Pan, Fu-shun; Lu, Ming-de; Xie, Xiao-yan

    2015-10-01

    Purpose To evaluate the diagnostic performance of ultrasonography (US) in the identification and exclusion of biliary atresia with a modified triangular cord thickness metric together with a gallbladder classification scheme, as well as hepatic artery (HA) diameter and liver and spleen size, in a large sample of jaundiced infants. Materials and Methods The ethics committee approved this study, and written informed parental consent was obtained. In 273 infants with conjugated hyperbilirubinemia (total bilirubin level ≥ 31.2 μmol/L, with direct bilirubin level > indirect bilirubin level), detailed abdominal US was performed to exclude biliary atresia. Biliary atresia was found in 129 infants and ruled out in 144. A modified triangular cord thickness was measured at the anterior branch of the right portal vein, and a gallbladder classification scheme was identified that incorporated the appearance of the gallbladder and a gallbladder length-to-width ratio of up to 5.2 when the lumen was visualized, as well as HA diameter and liver and spleen size. Reference standard diagnosis was based on results of one or more of the following: surgery, liver biopsy, cholangiography, and clinical follow-up. Area under the receiver operating characteristic curve (AUC) analysis, binary logistic regression analysis, Fisher exact test, and unpaired t test were performed. Results Triangular cord thickness, HA diameter, ratio of gallbladder length to gallbladder width, liver size, and spleen size exhibited statistically significant differences (all P < .05) between the group with biliary atresia and the group without. AUCs of triangular cord thickness, ratio of gallbladder length to width, and HA diameter were 0.952, 0.844, and 0.838, respectively. Logistic regression analysis demonstrated that these three US parameters were significantly associated (all P < .05) with biliary atresia. The combination of triangular cord thickness and gallbladder classification could yield comparable AUCs

  13. Managing eosinophilic esophagitis: challenges and solutions

    PubMed Central

    Shah, Nisha A; Albert, Dustin M; Hall, Noah M; Moawad, Fouad J

    2016-01-01

    Eosinophilic esophagitis (EoE) is a chronic and progressive immune-mediated condition defined by symptoms of esophageal dysfunction and dense eosinophilic infiltration of the esophageal mucosa. Therapies consist of anti-eosinophilic medications and specialized diets aimed to decrease the progression of EoE and alleviate its symptoms, namely, dysphagia and food impaction. Assessing response to therapy remains challenging, as treatment end points are not well defined and currently consist of clinical, histologic, and endoscopic features. Newer validated measures may help standardize treatment end points. Emerging data support the use of maintenance therapy, which may reduce disease progression. Optimal dosages, delivery techniques, and duration of treatment need to be determined. When features of fibrostenosis develop, esophageal dilation is a safe and effective adjunctive strategy for improving symptoms. In EoE cases refractory to conventional treatments, newer therapies targeting inflammatory mediators and cytokines are on the horizon. PMID:27695356

  14. Regenerative Medicine Strategies for Esophageal Repair

    PubMed Central

    Londono, Ricardo

    2015-01-01

    Pathologies that involve the structure and/or function of the esophagus can be life-threatening. The esophagus is a complex organ comprising nonredundant tissue that does not have the ability to regenerate. Currently available interventions for esophageal pathology have limited success and are typically associated with significant morbidity. Hence, there is currently an unmet clinical need for effective methods of esophageal repair. The present article presents a review of esophageal disease along with the anatomic and functional consequences of each pathologic process, the shortcomings associated with currently available therapies, and the latest advancements in the field of regenerative medicine with respect to strategies for esophageal repair from benchtop to bedside. PMID:25813694

  15. Managing eosinophilic esophagitis: challenges and solutions

    PubMed Central

    Shah, Nisha A; Albert, Dustin M; Hall, Noah M; Moawad, Fouad J

    2016-01-01

    Eosinophilic esophagitis (EoE) is a chronic and progressive immune-mediated condition defined by symptoms of esophageal dysfunction and dense eosinophilic infiltration of the esophageal mucosa. Therapies consist of anti-eosinophilic medications and specialized diets aimed to decrease the progression of EoE and alleviate its symptoms, namely, dysphagia and food impaction. Assessing response to therapy remains challenging, as treatment end points are not well defined and currently consist of clinical, histologic, and endoscopic features. Newer validated measures may help standardize treatment end points. Emerging data support the use of maintenance therapy, which may reduce disease progression. Optimal dosages, delivery techniques, and duration of treatment need to be determined. When features of fibrostenosis develop, esophageal dilation is a safe and effective adjunctive strategy for improving symptoms. In EoE cases refractory to conventional treatments, newer therapies targeting inflammatory mediators and cytokines are on the horizon.

  16. Esophageal papilloma: Flexible endoscopic ablation by radiofrequency

    PubMed Central

    del Genio, Gianmattia; del Genio, Federica; Schettino, Pietro; Limongelli, Paolo; Tolone, Salvatore; Brusciano, Luigi; Avellino, Manuela; Vitiello, Chiara; Docimo, Giovanni; Pezzullo, Angelo; Docimo, Ludovico

    2015-01-01

    Squamous papilloma of the esophagus is a rare benign lesion of the esophagus. Radiofrequency ablation is an established endoscopic technique for the eradication of Barrett esophagus. No cases of endoscopic ablation of esophageal papilloma by radiofrequency ablation (RFA) have been reported. We report a case of esophageal papilloma successfully treated with a single session of radiofrequency ablation. Endoscopic ablation of the lesion was achieved by radiofrequency using a new catheter inserted through the working channel of endoscope. The esophageal ablated tissue was removed by a specifically designed cup. Complete ablation was confirmed at 3 mo by endoscopy with biopsies. This case supports feasibility and safety of as a new potential indication for BarrxTM RFA in patients with esophageal papilloma. PMID:25789102

  17. Alcohol, Obesity Could Raise Esophageal Cancer Risk

    MedlinePlus

    ... page: https://medlineplus.gov/news/fullstory_160133.html Alcohol, Obesity Could Raise Esophageal Cancer Risk A third ... now linked to 11 types of cancer and alcohol links to six," she said in an institute ...

  18. Multidisciplinary management for esophageal and gastric cancer.

    PubMed

    Boniface, Megan M; Wani, Sachin B; Schefter, Tracey E; Koo, Phillip J; Meguid, Cheryl; Leong, Stephen; Kaplan, Jeffrey B; Wingrove, Lisa J; McCarter, Martin D

    2016-01-01

    The management of esophageal and gastric cancer is complex and involves multiple specialists in an effort to optimize patient outcomes. Utilizing a multidisciplinary team approach starting from the initial staging evaluation ensures that all members are in agreement with the plan of care. Treatment selection for esophageal and gastric cancer often involves a combination of chemotherapy, radiation, surgery, and palliative interventions (endoscopic and surgical), and direct communication between specialists in these fields is needed to ensure appropriate clinical decision making. At the University of Colorado, the Esophageal and Gastric Multidisciplinary Clinic was created to bring together all experts involved in treating these diseases at a weekly conference in order to provide patients with coordinated, individualized, and patient-centered care. This review details the essential elements and benefits of building a multidisciplinary program focused on treating esophageal and gastric cancer patients.

  19. Multidisciplinary management for esophageal and gastric cancer

    PubMed Central

    Boniface, Megan M; Wani, Sachin B; Schefter, Tracey E; Koo, Phillip J; Meguid, Cheryl; Leong, Stephen; Kaplan, Jeffrey B; Wingrove, Lisa J; McCarter, Martin D

    2016-01-01

    The management of esophageal and gastric cancer is complex and involves multiple specialists in an effort to optimize patient outcomes. Utilizing a multidisciplinary team approach starting from the initial staging evaluation ensures that all members are in agreement with the plan of care. Treatment selection for esophageal and gastric cancer often involves a combination of chemotherapy, radiation, surgery, and palliative interventions (endoscopic and surgical), and direct communication between specialists in these fields is needed to ensure appropriate clinical decision making. At the University of Colorado, the Esophageal and Gastric Multidisciplinary Clinic was created to bring together all experts involved in treating these diseases at a weekly conference in order to provide patients with coordinated, individualized, and patient-centered care. This review details the essential elements and benefits of building a multidisciplinary program focused on treating esophageal and gastric cancer patients. PMID:27217796

  20. Clinical application of endoscopic ultrasonography for esophageal achalasia.

    PubMed

    Minami, Hitomi; Inoue, Haruhiro; Isomoto, Hajime; Urabe, Shigetoshi; Nakao, Kazuhiko

    2015-04-01

    Endoscopic ultrasonography (EUS) has been widely used for evaluating the nature of diseases of various organs. The possibility of applying EUS for esophageal motility diseases has not been well discussed despite its versatility. At present, peroral endoscopic myotomy (POEM) for esophageal achalasia and related diseases has brought new attention to esophageal diseases because POEM provides a more direct approach to the inner structures of the esophageal wall. In the present study, we discuss the clinical utility of EUS in evaluating and treating esophageal motility diseases such as esophageal achalasia and related diseases. PMID:25573637

  1. RELATIONSHIP BETWEEN ESOPHAGITIS GRADES AND HELICOBACTER PYLORI

    PubMed Central

    RIBEIRO, Patrícia Fernanda Saboya; KUBRUSLY, Luiz Fernandao; NASSIF, Paulo Afonso Nunes; RIBEIRO, Irma Cláudia Saboya; BERTOLDI, Andressa de Souza; BATISTÃO, Venessa Caroline

    2016-01-01

    ABSTRACT Background: The Helicobacter pylori infection (HP) is related to the development of gastric lesions and lymphoma; however, it is not known if there is a relation with gastroesophageal reflux disease and reflux esophagitis. Aim: To evaluate HP's relationship with esophagitis in patients undergoing upper endoscopy. Methods: Observational, retrospective and cross-sectional study, being evaluated 9576 patients undergoing outpatient endoscopic examination during the period between January and December 2015. Were included patients with any esophageal alteration at the examination; greater than 18; of both genders; independent of the complaint or the reason for the examination, illness or drug use. Were excluded those with active bleeding during the examination and in use of anticoagulants. The variables gender, age, esophagitis and result of the urease test, were studied. For statistical analysis was used the Epi Info software 7.1.5.2. Results: Most of the samples consisted of women and the overall average age was 46.54±16.32 years. The presence of infection was balanced for gender: 1204 (12.56%) women and 952 (13.92%) men. Relating degree of esophagitis HP- and HP+ was observed that the type A was the most common (58.79%, n=1460); 604 (24.32%) had grade B; 334 (13.45%) grade C, and 85 (3.42%) grade D. In the relation between the grade of esophagitis with gender, esophagitis A was predominant in women and present in 929 (63.33%), followed by type B, 282 (46.68%), 136 C (40.71%) and D 30 (35.29%). In men 531 (36.36%) showed type A, 322 (53.31%) B, 198 (59.28%) C, and 55 (64.70%) D. Among the groups 40-50 and over 60 years there was a significant difference in whether have or not have HP+. Conclusion: There is no significant difference between HP infection and the different grades of esophagitis. PMID:27759772

  2. Left main coronary artery atresia and associated cardiac defects: report on concomitant surgical treatment.

    PubMed

    Jatene, Marcelo; Juaneda, Ignacio; Miranda, Rogerio Dos Anjos; Gato, Rafaella; Marcial, Miguel Lorenzo Barbero

    2011-10-01

    A 9-year-old boy with congenital atresia of the left main coronary artery underwent myocardial revascularization. Coarctation of the aorta and ventricular septal defect were diagnosed at the age of 1 year. At age 7 years, the child presented with syncope while exercising. Preoperative evaluation included cardiac catheterization which revealed the unexpected finding of congenital atresia of the left main coronary artery with origin of the circumflex artery from the right coronary artery. Surgical correction included myocardial revascularization by means of left internal mammary artery graft to the anterior descending coronary artery, coarctation resection, and ventricular septal defect repair. The patient recovered uneventfully. We report the details of this extremely rare case with successful concomitant surgical management of the congenital coronary artery anomaly and the associated structural heart disease. PMID:23804483

  3. Growth and development after oesophageal atresia surgery: Need for long-term multidisciplinary follow-up.

    PubMed

    IJsselstijn, Hanneke; Gischler, Saskia J; Toussaint, Leontien; Spoel, Marjolein; Zijp, Monique H M van der Cammen-van; Tibboel, Dick

    2016-06-01

    Survival rates in oesophageal atresia patients have reached over 90%. In long-term follow-up studies the focus has shifted from purely surgical or gastrointestinal evaluation to a multidisciplinary approach. We reviewed the literature on the long-term morbidity of these patients and discuss mainly issues of physical growth and neurodevelopment. We conclude that growth problems - both stunting and wasting - are frequently seen, but that sufficient longitudinal data are lacking. Therefore, it is unclear whether catch-up growth into adolescence and adulthood occurs. Data on determinants of growth retardation are also lacking in current literature. Studies on neurodevelopment beyond preschool age are scarce but oesophageal atresia patients seem at risk for academic problems and motor function delay. Many factors contribute to the susceptibility to growth and development problems and we propose a multidisciplinary follow-up schedule into adulthood future care which may help improve quality of life.

  4. Does postoperative ventilation have an effect on the integrity of the anastomosis in repaired oesophageal atresia?

    PubMed

    Beasley, S W

    1999-04-01

    Several authors have claimed that the use of postoperative ventilation or graded withdrawal of respiratory support reduces the incidence of anastomotic complications after repair of oesophageal atresia, particularly where the gap between the oesophageal ends has been extensive or where the anastomosis has been constructed under tension. Careful review of their data reveals little objective evidence to either support or refute this contention. Many institutions are achieving low leakage rates following oesophageal anastomosis in oesophageal atresia, but to date there has been no controlled study to show that the use of neck flexion, muscle paralysis, intubation and assisted ventilation postoperatively influences the integrity of the anastomosis. The sequence of observations that led to the presumed relationship between postoperative ventilation and oesophageal leak is reviewed. It would appear that the effect of postoperative ventilation and paralysis on the oesophageal anastomosis is yet to be determined. PMID:10365344

  5. Multiple orifices and cholangiography with a "fire-like" appearance after Kasai hepatoportoenterostomy for biliary atresia.

    PubMed

    Yokoyama, Kensuke; Hatanaka, Hisashi; Inoue, Mikihiro; Yano, Tomonori; Numao, Norikatsu; Ushio, Jun; Lefor, Alan Kawarai; Tamada, Kiichi; Yamamoto, Hironori

    2016-09-01

    A 21-year-old female underwent a Kasai hepatoportoenterostomy with Roux-en-Y reconstruction for typeIII biliary atresia at age 63 days. At the age of 19 years, she developed cholangitis and CT scan revealed hepatolithiasis. She presented for treatment of the intrahepatic stone and the hepatportoenterostomy was directly visualized with double-balloon endoscopy (DBE). Endoscopic findings showed multiple intrahepatic bile ducts open to the jejunum through multiple orifices. Cholangiography showed narrowing of intrahepatic bile duct branches with a "fire-like" appearance. These findings have not been previously reported, since endoscopic approaches to patients with a hepaticojejunostomy were limited. DBE was useful to directly visualize the anastomosis in a patient status-post the Kasai operation for biliary atresia with a Rouxen-Y reconstruction. PMID:27502010

  6. Choledochal cyst and biliary atresia in the neonate: Imaging findings in five cases

    SciTech Connect

    Torrisi, J.M.; Haller, J.O.; Velcek, F.T. )

    1990-12-01

    The radiologic findings in five neonates with choledochal cyst associated with extra-hepatic biliary atresia are described. All five patients (age range, 13-72 days) presented with jaundice and acholic stools. In all four patients who underwent sonographic examination, a cystic structure separate from the gallbladder representing the choledochal cyst was shown. The diagnosis of atresia of the distal common bile duct was made preoperatively in all cases by hepatobiliary scintigraphy. Diagnosis was confirmed by surgical findings and was demonstrated by intraoperative cholangiography in four cases. All patients were successfully treated with surgical intervention within 1 month from the time of diagnosis. Early detection of this rare disorder, which may be distinct from choledochal cyst found in children and adults, is important to prevent fatal complications of biliary obstruction. The combined use of sonography and hepatobiliary scintigraphy can correctly identify this subset of patients with persistent neonatal jaundice and provide valuable information for prompt surgical management.

  7. [A fully-implantable active hearing device in congenital auricular atresia].

    PubMed

    Siegert, R; Neumann, C

    2014-07-01

    Active implantable hearing devices were primarily developed for sensorineural hearing loss. The vibrator coupling mechanisms were oriented towards normal middle ear anatomy and function. The aim of this project was to modify the only fully implantable hearing device with an implantable microphone for application in congenital auricular atresia, Carina™, and to introduce the modified device into the clinic. A special prosthesis was developed for the transducer and its individual coupling achieved by a special cramping system. The system was implanted in 5 patients with congenital auricular atresia. Audiological results were good; with patients' hearing gain exceeding 30 dB HL. Anatomic limits to the system's indications and technical drawbacks are also discussed. PMID:25056646

  8. Do large hiatal hernias affect esophageal peristalsis?

    PubMed Central

    Roman, Sabine; Kahrilas, Peter J; Kia, Leila; Luger, Daniel; Soper, Nathaniel; Pandolfino, John E

    2013-01-01

    Background & Aim Large hiatal hernias can be associated with a shortened or tortuous esophagus. We hypothesized that these anatomic changes may alter esophageal pressure topography (EPT) measurements made during high-resolution manometry (HRM). Our aim was to compare EPT measures of esophageal motility in patients with large hiatal hernias to those of patients without hernia. Methods Among 2000 consecutive clinical EPT, we identified 90 patients with large (>5 cm) hiatal hernias on endoscopy and at least 7 evaluable swallows on EPT. Within the same database a control group without hernia was selected. EPT was analyzed for lower esophageal sphincter (LES) pressure, Distal Contractile Integral (DCI), contraction amplitude, Contractile Front Velocity (CFV) and Distal Latency time (DL). Esophageal length was measured on EPT from the distal border of upper esophageal sphincter to the proximal border of the LES. EPT diagnosis was based on the Chicago Classification. Results The manometry catheter was coiled in the hernia and did not traverse the crural diaphragm in 44 patients (49%) with large hernia. Patients with large hernias had lower average LES pressures, lower DCI, slower CFV and shorter DL than patients without hernia. They also exhibited a shorter mean esophageal length. However, the distribution of peristaltic abnormalities was not different in patients with and without large hernia. Conclusions Patients with large hernias had an alteration of EPT measurements as a consequence of the associated shortened esophagus. However, the distribution of peristaltic disorders was unaffected by the presence of hernia. PMID:22508779

  9. Diagnosis and management of esophageal achalasia.

    PubMed

    Stavropoulos, Stavros N; Friedel, David; Modayil, Rani; Parkman, Henry P

    2016-01-01

    Achalasia is a rare esophageal motility disorder that is usually idiopathic in origin. It is characterized by dysphagia, and patients often have chest pain, regurgitation, weight loss, and an abnormal barium radiograph showing esophageal dilation with narrowing at the gastroesophageal junction. Abnormal or absent esophageal peristalsis and impaired relaxation of the lower esophageal sphincter (LES) are typically seen on esophageal manometry. The advent of high resolution manometry (HRM) has allowed more precise diagnosis of achalasia, subtype designation, and differentiation from other esophageal motor disorders with an initial seminal publication in 2008 followed by further refinements of what has been termed the Chicago classification. Potential treatments include drugs, endoscopic botulinum toxin injection, balloon dilation, traditional surgery (usually laparoscopic Heller myotomy; LHM), and a novel, less invasive, natural orifice transluminal endoscopic surgery (NOTES) approach to Heller myotomy termed peroral endoscopic myotomy (POEM). The first human POEM was performed in 2008, with the first publication appearing in 2010 and evidence now rapidly accumulating showing POEM to be comparable to traditional surgery in terms of clinical success and radiologic and manometric post-therapy outcomes. This review discusses the diagnosis and management of achalasia with particular emphasis on the recent developments of HRM and POEM, which arguably represent the most important advances in the field since the advent of laparoscopic Heller myotomy in the 1990s. PMID:27625387

  10. Effect of total laryngectomy on esophageal motility

    SciTech Connect

    Hanks, J.B.; Fisher, S.R.; Meyers, W.C.; Christian, K.C.; Postlethwait, R.W.; Jones, R.S.

    1981-01-01

    Total laryngectomy for cancer can result in dysphagia and altered esophageal motility. Manometric changes in the upper esophageal sphincter (UES), and in proximal and distal esophageal function have been reported. However, most studies have failed to take into account radiation therapy and appropriate controls. We selected ten male patients (54.3 +/- 1.9 yr) for longitudinal manometric evaluation prior to laryngectomy then at two weeks and again six months later. No patient received preoperative radiation therapy, had a previous history of esophageal surgery, or developed a postoperative wound infection or fistula. Seven of ten patients had positive nodes and received 6,000-6,600 rads postoperative radiation therapy. Preoperatively 4 of 10 patients complained of dysphagia which did not significantly change following surgery and radiation. Two of three patients who did not complain of dysphagia preoperatively and received radiation postoperatively developed dysphagia. No patient without dysphagia preoperatively who received no radiation therapy developed symptoms. Our studies show that laryngectomy causes alterations in the UES resting and peak pressures but not in the proximal or distal esophagus, or the lower esophageal sphincter. These data also imply radiation therapy may be associated with progressive alterations in motility and symptomatology. Further study regarding the effects of radiation on esophageal motility and function are urged.

  11. Technique of percutaneous laser-assisted valve dilatation for valvar atresia in congenital heart disease.

    PubMed Central

    Rosenthal, E; Qureshi, S A; Kakadekar, A P; Anjos, R; Baker, E J; Tynan, M

    1993-01-01

    OBJECTIVE--To investigate the efficacy and safety of transcatheter laser-assisted valve dilatation for atretic valves in children with congenital heart disease. DESIGN--Prospective clinical study. SETTING--Supraregional paediatric cardiology centre. SUBJECTS--Eleven children (aged 1 day-11 years; weight 2.1-35.7 kg) with atresia of pulmonary (10) or tricuspid (one) valve underwent attempted laser-assisted valve dilatation as part of the staged treatment of their cyanotic heart disease. INTERVENTION--After delineating the atretic valve by angiography and/or echocardiography a 0.018 inch "hot tip" laser wire was used to perforate the atretic valve. Subsequently the valve was dilated with conventional balloon dilatation catheters up to the valve annulus diameter. RESULTS--Laser-assisted valve dilatation was successfully accomplished in nine children. In two neonates with pulmonary valve atresia, intact ventricular septum, and coexistent infundibular atresia the procedure resulted in cardiac tamponade: one died immediately and one later at surgery. During a follow up of 1-17 months (mean 11) two infants with pulmonary valve atresia and intact ventricular septum died (one with congestive cardiac failure). The remainder are either well palliated and do not require further procedures (three), or are awaiting further transcatheter or surgical procedures because of associated defects (four). CONCLUSIONS--Laser-assisted valve dilatation is a promising adjunct to surgery in this high risk group of patients. It may avoid surgery in some patients, and may reduce the number of surgical procedures in those requiring staged operations. Images PMID:8343325

  12. Split-graft technique in neonatal heart transplant for aortic atresia.

    PubMed

    Gil-Jaurena, Juan-Miguel; González-López, María-Teresa; Pita-Fernández, Ana; Pérez-Caballero, Ramón

    2016-10-01

    We describe a neonate with aortic atresia and hypoplastic aorta, listed for heart transplant after extracorporeal membrane oxygenation resuscitation and ductal stenting. The donor aorta was detached from the graft, after an isolated arch reconstruction prior to the transplant itself in a routine fashion. To the best of our knowledge, this is the first reported case of neonatal arch reconstruction before transplantation performed with grafts from the same donor in a split-way strategy.

  13. Large Right Ventricular Clot in Pulmonary Atresia With Intact Ventricular Septum: In Defense of Biventricular Approach.

    PubMed

    Dutta, Nilanjan; Ghosh, Rajarshi; Awasthy, Neeraj; Iyer, Parvathi U; Girotra, Sumir; Iyer, Krishna S

    2016-09-01

    Thrombus formation within the right ventricle (RV) in the setting of pulmonary atresia with intact ventricular septum (PAIVS) is not a very common occurrence and can be catastrophic. We present the case of a seven-month-old child with PAIVS and RV clot who successfully underwent biventricular repair. We discuss the interesting case and the rationale for management by means of biventricular repair over single ventricle repair when feasible in such a setting.

  14. Split-graft technique in neonatal heart transplant for aortic atresia.

    PubMed

    Gil-Jaurena, Juan-Miguel; González-López, María-Teresa; Pita-Fernández, Ana; Pérez-Caballero, Ramón

    2016-10-01

    We describe a neonate with aortic atresia and hypoplastic aorta, listed for heart transplant after extracorporeal membrane oxygenation resuscitation and ductal stenting. The donor aorta was detached from the graft, after an isolated arch reconstruction prior to the transplant itself in a routine fashion. To the best of our knowledge, this is the first reported case of neonatal arch reconstruction before transplantation performed with grafts from the same donor in a split-way strategy. PMID:27354464

  15. Tricuspid atresia with christmas disease (hemophilia B). Report of a case.

    PubMed

    Lawson, R; Rullman, D; Brodeur, M; Starr, A

    1975-04-01

    A case combining Christmas disease and tricuspid atresia is presented. A severe coagulation defect in a hypoxic, polycythemic patient poses an added grave risk to major surgery. In the presence of marked and worsening symptoms, however, major surgery can be performed in these difficult cases. Close liason with a skilled hematology department is essential. Minimal tissue dissection is advised, and the usefulness of a reversed saphenous vein conduit is described.

  16. Inflammatory response of esophageal epithelium in combined-type esophagitis in rats: a transcriptome analysis.

    PubMed

    Naito, Yuji; Kuroda, Masaaki; Uchiyama, Kazuhiko; Mizushima, Katsura; Akagiri, Satomi; Takagi, Tomohisa; Handa, Osamu; Kokura, Satoshi; Yoshida, Norimasa; Ichikawa, Hiroshi; Yoshikawa, Toshikazu

    2006-11-01

    Recent studies have shown that esophageal mucosal inflammatory response is involved in the pathophysiology of gastro-esophageal reflux disease. The aim of the present study was to identify specific gene expression profiles of the esophageal mucosa in a rat model of combined-type chronic reflux esophagitis. Esophagogastroduodenal anastomosis was carried out in male Wistar rats by anastomosing the jejunum to the gastroesophageal junction under diethyl-ether inhalation anesthesia. Esophageal epithelial cells were obtained from esophagi of rats by laser capture microdissection. Preparation of cRNA and target hybridization were performed according to the Affymetrix GeneChip eukaryotic small sample target labeling assay protocol. The gene expression profile was evaluated by the rat toxicology U34 GeneChip. Array data analysis was carried out using Affymetrix GeneChip operating software, ingenuity pathway analysis software, and Gene Springs software. A comparison between esophagitis and sham-operated rats 2 weeks after the operation revealed that 368 probes (36%) were significantly affected, i.e. 185 probes were up-regulated, and 183 probes were down-regulated, both at levels of at least 1.5-fold in the esophagitis rats. Ingenuity signal analysis of 207 affected probes revealed the interleukin-6 signaling pathway as the most significantly affected caronical pathway. In addition, the expression of many genes associated with cytokine and transcription factor was enhanced in the esophagitis rats. This transcriptome approach provided insight into genes and putative genetic pathways thought to be affected by stimulation with gastroduodenal refluxates.

  17. Staging of biliary atresia at diagnosis by molecular profiling of the liver

    PubMed Central

    2010-01-01

    Background Young age at portoenterostomy has been linked to improved outcome in biliary atresia, but pre-existing biological factors may influence the rate of disease progression. In this study, we aimed to determine whether molecular profiling of the liver identifies stages of disease at diagnosis. Methods We examined liver biopsies from 47 infants with biliary atresia enrolled in a prospective observational study. Biopsies were scored for inflammation and fibrosis, used for gene expression profiles, and tested for association with indicators of disease severity, response to surgery, and survival at 2 years. Results Fourteen of 47 livers displayed predominant histological features of inflammation (N = 9) or fibrosis (N = 5), with the remainder showing similar levels of both simultaneously. By differential profiling of gene expression, the 14 livers had a unique molecular signature containing 150 gene probes. Applying prediction analysis models, the probes classified 29 of the remaining 33 livers into inflammation or fibrosis. Molecular classification into the two groups was validated by the findings of increased hepatic population of lymphocyte subsets or tissue accumulation of matrix substrates. The groups had no association with traditional markers of liver injury or function, response to surgery, or complications of cirrhosis. However, infants with an inflammation signature were younger, while those with a fibrosis signature had decreased transplant-free survival. Conclusions Molecular profiling at diagnosis of biliary atresia uncovers a signature of inflammation or fibrosis in most livers. This signature may relate to staging of disease at diagnosis and has implications to clinical outcomes. PMID:20465800

  18. [Abnormal Serum Total Protein Measurement by Lipoprotein-X in an Infant with Biliary Atresia].

    PubMed

    Futatsugi, Akiko; Hidaka, Eiko; Kubota, Noriko; Nishijima, Fumie; Yoshizawa, Katsumi; Ishimine, Nau; Sugano, Mitsutoshi; Hori, Atsushi; Hidaka, Hiroya

    2015-11-01

    Lipoprotein-X (LP-X) in cholestatic jaundice causes abnormal reaction in assays for low-density lipoprotein-cholesterol, but the effects on other test items are unknown. Here, we report an infant with biliary atresia showing abnormal reaction in total serum protein assay using the biuret method, and lipoprotein-X (LP-X) was then detected. In this 11-month-old female infant, jaundice was observed at 2 months old, and a diagnosis of biliary atresia was made. On biochemical tests at 12 months old, the total serum protein concentrations detected by the biuret method were very high, and the response curve and linearity of dilution were abnormal. LP-X was detected by agar electrophoresis. In addition and recovery experiments with normal serum fractionation of the patient's LP-X-rich lipoprotein fraction prepared by ultracentrifugation, normal γ-globulin fractionation showed an abnormal reaction by the biuret method. In infants with biliary atresia, we showed that the total serum protein assay by the biuret method was influenced by LP-X-rich lipoprotein, which may be caused by abnormal reaction of LP-X and γ-globulin. [Case Report].

  19. Atresia of the Aortic Arch in 4-Year-Old Child: A Clinical Case Study

    PubMed Central

    Nigro Stimato, Vittoria; Didier, Dominique; Beghetti, Maurice; Tissot, Cécile

    2015-01-01

    Atresia of the aortic arch is a rare congenital heart defect with a high mortality when associated with other intracardiac defects. Cardiac magnetic resonance (CMR) provides the exact anatomy of the aortic arch and collateral circulation and is useful to diagnose-associated aortic arch anomalies. This report describes the case of a 4-year-old child with atresia of the aortic arch, referred to our institution with the diagnosis of aortic coarctation and bicuspid aortic valve. On clinical exam, the femoral pulses were not palpable and there was a significant differential blood pressure between the upper and lower limbs. The echocardiography showed a severely stenotic bicuspid aortic valve but was limited for the exact description of the aortic arch. CMR showed absence of lumen continuity between the ascending and descending aorta distal to the left subclavian artery, extending over 5 mm, with the presence of a bend in the arch and diverticulum on either side of the zone of discontinuity, suggesting the diagnosis atresia of the aortic arch rather than coarctation or interruption. The patient benefited from a successful surgical commissurotomy of the aortic valve and reconstruction of the aortic arch with a homograft. The post-operative CMR confirmed the good surgical result. This case emphasizes the utility of CMR to provide good anatomical information to establish the exact diagnosis and the operative strategy. PMID:25853109

  20. Hyberbaric oxygen increases atresia in normal & steroid induced PCO rat ovaries

    PubMed Central

    2012-01-01

    Background In this study, we investigated the effect of hyperbaric oxygen therapy (HBOT) on the morphology of estradiol valerate (EV) induced polycystic ovary (PCO) to find a new treatment modality for improvement of PCO. Methods The rats were divided into four groups. Group1, control; group 2, PCO group; group 3, PCO with HBOT group and group 4, normal ovary with HBOT. PCO was induced by a single intramuscular injection of 4 mg EV in adult cycling rats. Other rats with normal ovaries had oil injection as placebo. HBOT was applied to third and fourth groups for six weeks. Histopathologic evaluation of ovaries of all groups were performed & compared. Results Six weeks of HBOT was resulted in increase in follicular atresia, decrease in the number of primary, secondary, tertiary follicles and decrease in the number of fresh corpus luteum in normal rat ovary. HBOT on polycystic rat ovary, resulted in significant increase in atretic follicles which were already present. Conclusions HBOT of six weeks itself, changed ovarian morphology in favor of atresia both in PCO group and control group. This result of aggravated follicular atresia after HBOT on EV induced PCO may be due to long-term exposure in our protocol which with this state seems to be inapplicable in the improvement of PCO morphology. PMID:22309835

  1. Congenital cholesteatoma of the infratemporal fossa with congenital aural atresia and mastoiditis: a case report

    PubMed Central

    2012-01-01

    Background Congenital cholesteatoma may be expected in abnormally developed ear, it may cause bony erosion of the middle ear cleft and extend to the infratemporal fossa. We present the first case of congenital cholesteatoma of the infratemporal fossa in a patient with congenital aural atresia that has been complicated with acute mastoiditis. Case presentation A sixteen year old Egyptian male patient presented with congenital cholesteatoma of the infratemporal fossa with congenital aural atresia complicated with acute mastoiditis. Two weeks earlier, the patient suffered pain necessitating hospital admission, magnetic resonance imaging revealed a soft tissue mass in the right infratemporal fossa. On presentation to our institute, Computerized tomography was done as a routine, it proved the diagnosis of mastoiditis, pure tone audiometry showed an air-bone gap of 60 dB. Cortical mastoidectomy was done for treatment of mastoiditis, removal of congenital cholesteatoma was carried out with reconstruction of external auditory canal. Follow-up of the patient for 2 years and 3 months showed a patent, infection free external auditory canal with an air-bone gap has been reduced to 35db. One year after the operation; MRI was done and it showed no residual or recurrent cholesteatoma. Conclusions Congenital cholesteatoma of the infratemporal fossa in cases of congenital aural atresia can be managed safely even if it was associated with mastoiditis. It is an original case report of interest to the speciality of otolaryngology. PMID:22731118

  2. Methoxychlor-induced atresia in the mouse involves Bcl-2 family members, but not gonadotropins or estradiol.

    PubMed

    Borgeest, Christina; Miller, Kimberly P; Gupta, Rupesh; Greenfeld, Chuck; Hruska, Kathleen S; Hoyer, Patricia; Flaws, Jodi A

    2004-06-01

    Methoxychlor (MXC) is an organochlorine pesticide that increases the rate of ovarian atresia. To date, little is known about the mechanism by which MXC induces atresia. Because Bcl-2 (an antiapoptotic factor), Bax (a proapoptotic factor), gonadotropins, and estradiol are important regulators of atresia in the ovary, the purpose of this study was first to examine whether MXC-induced atresia occurred through alterations in Bcl-2 or Bax, and second, to examine the effect of MXC on gonadotropins, estradiol, and their receptors. CD-1 mice were dosed with 8-64 mg kg(-1) day(-1) MXC or vehicle (sesame oil). Ovaries were subjected to analysis of antral follicle numbers, Bcl-2, Bax, estrogen receptor, and follicle-stimulating hormone receptor levels. Blood was used to measure gonadotropins and estradiol. In some experiments, mice that overexpressed Bcl-2 or mice that were deficient in Bax were dosed with MXC or vehicle and their ovaries were analyzed for atresia. MXC caused a dose-dependent increase in the percentage of atretic antral follicles compared with controls at the 32 and 64 mg kg(-1) day(-1) doses of MXC. MXC treatment did not result in changes in Bcl-2 levels, but it did result in an increase in Bax levels in antral follicles. MXC treatment did not affect gonadotropin or estradiol levels, nor did it affect the levels of follicle-stimulating hormone or estrogen receptors. Mice that overexpressed Bcl-2 or mice that were deficient in Bax were protected from MXC-induced atresia. These data suggest that MXC induces atresia through direct effects on the Bax and Bcl-2 signaling pathways in the ovary.

  3. Evaluation of gastric tube with esophageal thermister (Thermosump).

    PubMed

    Koyama, K; Ochiai, R; Takahashi, J; Takeda, J; Sekiguchi, H; Fukushima, K

    1992-07-01

    The accuracy and the feasibility of esophageal temperature measured by a new gastric tube. Thermosump, which is incorporated with a esophageal thermister, was evaluated in anesthetized dogs (n = 6) and men (n = 59). In dogs, esophageal temperature measured by Thermosump was correlated well with the temperatures measured by the conventional esophageal thermister, and also by the pulmonary artery catheter (r = 0.98, 0.98, respectively). In anesthetized men, correlation between esophageal temperature by Thermosump and rectal, or bladder temperature was good during surgery of extremities (r = 0.81, 0.80, respectively). But during abdominal surgery, correlation between esophageal and bladder temperature was relatively poor (r = 0.50). Insertion of the tube, and suction of gastric fluid through the tube were easy without any complication. This newly developed gastric tube with a esophageal thermister was safe, and useful for measuring esophageal temperature.

  4. [FEATURES OF TREATMENT OF EOSINOPHILIC ESOPHAGITIS IN SCHOOLCHILDREN].

    PubMed

    Horodylovska, M I

    2015-01-01

    The inclusion of probiotic L. reuteri into the complex therapy of eosinophilic esophagitis significantly affect the outcomes of children--there was significant decrease in the number of eosinophils in the esophageal mucosa of children. PMID:26118052

  5. [Current status and perspectives of radiotherapy for esophageal cancer].

    PubMed

    Wu, S X; Wang, L H

    2016-09-23

    Esophageal cancer is one of the most common cancers in China. More than 80% of esophageal cancer patients are diagnosed at a late stage and are not eligible for surgery. Radiotherapy is one of the most important modalities in esophageal cancer treatment. Here we reviewed the advances in esophageal cancer radiotherapy and radiotherapy-based combined-modality therapy, such as optimization of radiation dose and target volume, application of precise radiotherapy technique and the integration of radiotherapy with chemotherapy and targeted therapy.

  6. Review of the Burden of Esophageal Cancer in Malaysia.

    PubMed

    Siti-Azrin, Ab Hamid; Wan-Nor-Asyikeen, Wan Adnan; Norsa'adah, Bachok

    2016-01-01

    Esophageal cancer is one of the top leading causes of cancer-related deaths in Malaysia. To date, neither the prevalence nor incidence of esophageal cancer nationally have been recorded. Esophageal cancer remains a major and lethal health problem even if it is not common in Malaysia. The late presentation of esophageal cancer makes it a difficult and challenging medical problem. Therefore, more governmental and non-governmental organizations of Malaysia should emphasize primary and secondary prevention strategies. PMID:27644604

  7. Broken Esophageal Stent Successfully Treated by Interventional Radiology Technique

    SciTech Connect

    Zelenak, Kamil; Mistuna, Dusan; Lucan, Jaroslav; Polacek, Hubert

    2010-06-15

    Esophageal stent fractures occur quite rarely. A 61-year-old male patient was previously treated for rupture of benign stenosis, occurring after dilatation, by implanting an esophageal stent. However, a year after implantation, the patient suffered from dysphagia caused by the broken esophageal stent. He was treated with the interventional radiology technique, whereby a second implantation of the esophageal stent was carried out quite successfully.

  8. Concomitant herpetic and eosinophilic esophagitis--a causality dilemma.

    PubMed

    Monsanto, P; Almeida, N; Cipriano, M A; Gouveia, H; Sofia, C

    2012-09-01

    Eosinophilic and herpetic esophagitis are listed as independent causes of dysphagia, especially in young adult males. However, herpetic esophagitis rarely affects immunocompetent individuals. We report the case of a young, not immunocompromised patient, admitted because of severe dysphagia secondary to herpes simplex virus esophagitis. After complete resolution, an endoscopic and histologic reevaluation established the diagnosis of eosinophilic esophagitis. The potential association between the two conditions is discussed.

  9. Evaluation of urgent esophagectomy in esophageal perforation

    PubMed Central

    de AQUINO, José Luis Braga; de CAMARGO, José Gonzaga Teixeira; CECCHINO, Gustavo Nardini; PEREIRA, Douglas Alexandre Rizzanti; BENTO, Caroline Agnelli; LEANDRO-MERHI, Vânia Aparecida

    2014-01-01

    Background Esophageal trauma is considered one of the most severe lesions of the digestive tract. There is still much controversy in choosing the best treatment for cases of esophageal perforation since that decision involves many variables. The readiness of medical care, the patient's clinical status, the local conditions of the perforated segment, and the severity of the associated injuries must be considered for the most adequate therapeutic choice. Aim To demonstrate and to analyze the results of urgent esophagectomy in a series of patients with esophageal perforation. Methods A retrospective study of 31 patients with confirmed esophageal perforation. Most injuries were due to endoscopic dilatation of benign esophageal disorders, which had evolved with stenosis. The diagnosis of perforation was based on clinical parameters, laboratory tests, and endoscopic images. ‪The main surgical technique used was transmediastinal esophagectomy followed by reconstruction of the digestive tract in a second surgical procedure. Patients were evaluated for the development of systemic and local complications, especially for the dehiscence or stricture of the anastomosis of the cervical esophagus with either the stomach or the transposed colon. Results Early postoperative evaluation showed a survival rate of 77.1% in relation to the proposed surgery, and 45% of these patients presented no further complications. The other patients had one or more complications, being pulmonary infection and anastomotic fistula the most frequent. The seven patients (22.9%) who underwent esophageal resection 48 hours after the diagnosis died of sepsis. At medium and long-term assessments, most patients reported a good quality of life and full satisfaction regarding the surgery outcomes. Conclusions Despite the morbidity, emergency esophagectomy has its validity, especially in well indicated cases of esophageal perforation subsequent to endoscopic dilation for benign strictures. PMID:25626932

  10. Chemoprevention of esophageal squamous cell carcinoma

    SciTech Connect

    Stoner, Gary D. Wang Lishu; Chen Tong

    2007-11-01

    Esophageal squamous cell carcinoma (SCC) is responsible for approximately one-sixth of all cancer-related mortality worldwide. This malignancy has a multifactorial etiology involving several environmental, dietary and genetic factors. Since esophageal cancer has often metastasized at the time of diagnosis, current treatment modalities offer poor survival and cure rates. Chemoprevention offers a viable alternative that could well be effective against the disease. Clinical investigations have shown that primary chemoprevention of this disease is feasible if potent inhibitory agents are identified. The Fischer 344 (F-344) rat model of esophageal SCC has been used extensively to investigate the biology of the disease, and to identify chemopreventive agents that could be useful in human trials. Multiple compounds that inhibit tumor initiation by esophageal carcinogens have been identified using this model. These include several isothiocyanates, diallyl sulfide and polyphenolic compounds. These compounds influence the metabolic activation of esophageal carcinogens resulting in reduced genetic (DNA) damage. Recently, a few agents have been shown to inhibit the progression of preneoplastic lesions in the rat esophagus into tumors. These agents include inhibitors of inducible nitric oxide synthase (iNOS), cyclooxygenase-2 (COX-2), vascular endothelial growth factor (VEGF) and c-Jun [a component of activator protein-1 (AP-1)]. Using a food-based approach to cancer prevention, we have shown that freeze-dried berry preparations inhibit both the initiation and promotion/progression stages of esophageal SCC in F-344 rats. These observations have led to a clinical trial in China to evaluate the ability of freeze-dried strawberries to influence the progression of esophageal dysplasia to SCC.

  11. [Acute necrotizing esophagitis (black esophagus) with secondary severe stenosis].

    PubMed

    Gómez, Álvaro A; Guerrero, Diego; Hani, Albis C; Cañadas, Raúl

    2015-01-01

    We report the case of a 67 years old patient with a history of diabetes mellitus, atrial fibrillation and chronic renal failure, who developed diabetic ketoacidosis and severe sepsis, later presenting an acute necrotizing esophagitis, and then a esophageal stenosis requiring treatment with self-expanding esophageal prosthesis with good clinical results. PMID:26802889

  12. Black esophagus (acute esophageal necrosis) after spinal anesthesia.

    PubMed

    Román Fernández, A; López Álvarez, A; Fossati Puertas, S; Areán González, I; Varela García, O; Viaño López, P M

    2014-01-01

    Acute esophagic necrosis or black esophagus is an uncommon clinical entity that owes its name to the endoscopic view of the necrotic esophageal mucosa. It is always related with a critical medical condition and usually has an ischemic etiology. We report the first case of acute esophageal necrosis after a spinal anesthetic for partial hip joint arthroplasty. We discuss the underlying pathophysiological mechanisms.

  13. [Esophageal reconstruction--using gastric tube instead of whole stomach].

    PubMed

    Chen, Keneng

    2014-09-01

    Stomach is the first choice for esophageal reconstruction following esophagectomy. In the earlier days, however, whole stomach pulling-up was the major surgery, which had some shortcomings. Recently, gastric tube has gained wide acceptance for esophageal reconstruction. This paper summarized the anatomical and physiological advantage of stomach, the disadvantage of whole stomach, and benefits of gastric tube for esophageal reconstruction.

  14. Conservative surgical treatment of reflux esophagitis and esophageal stricture.

    PubMed Central

    Herrington, J L; Wright, R S; Edwards, W H; Sawyers, J L

    1975-01-01

    During a recent 3-year period, 17 consecutive patients were seen with advanced fibrotic esophageal strictures secondary to alkaline-acid-pepsin reflux. From detailed preoperative evaluations alone it was impossible to determine whether therapy should consist of excisional surgery, esophagogastroplasty or intra-operative dilatation with correction of reflux. Only at operation could the length, extent, degree and severity of the stricture be fully determined. Each of the 17 patients was treated by controlled dilatation, coupled with an antireflux procedure. This simplified approach proved successful on strictures thought preoperatively to be undilatable. It appears that this conservative approach is applicable to many advanced strictures and excisional and plastic procedures should be reserved for those cases that prove unyielding to intraoperative dilatation. The true appraisal of a reflux stricture and the choice of surgical procedure is best determined at the operating table. Images Fig. 5A. Fig. 5B. Fig. 6. Fig. 7. Fig. 8. Fig. 9. Fig. 10. Fig. 11. Fig. 12. Fig. 13. Fig. 14. Fig. 15. Fig. 16. Fig. 17. Fig. 18. Fig. 19. Fig. 20. Fig. 21. PMID:1130874

  15. FOLFOX-6 Induction Chemotherapy Followed by Esophagectomy and Post-operative Chemoradiotherapy in Patients With Esophageal Adenocarcinoma

    ClinicalTrials.gov

    2016-09-15

    Adenocarcinoma of the Esophagus; Adenocarcinoma of the Gastroesophageal Junction; Adenocarcinoma of the Gastric Cardia; Stage IIIA Esophageal Cancer; Stage IIIB Esophageal Cancer; Stage IIIC Esophageal Cancer

  16. Esophageal surgery in minimally invasive era

    PubMed Central

    Bencini, Lapo; Moraldi, Luca; Bartolini, Ilenia; Coratti, Andrea

    2016-01-01

    The widespread popularity of new surgical technologies such as laparoscopy, thoracoscopy and robotics has led many surgeons to treat esophageal diseases with these methods. The expected benefits of minimally invasive surgery (MIS) mainly include reductions of postoperative complications, length of hospital stay, and pain and better cosmetic results. All of these benefits could potentially be of great interest when dealing with the esophagus due to the potentially severe complications that can occur after conventional surgery. Moreover, robotic platforms are expected to reduce many of the difficulties encountered during advanced laparoscopic and thoracoscopic procedures such as anastomotic reconstructions, accurate lymphadenectomies, and vascular sutures. Almost all esophageal diseases are approachable in a minimally invasive way, including diverticula, gastro-esophageal reflux disease, achalasia, perforations and cancer. Nevertheless, while the limits of MIS for benign esophageal diseases are mainly technical issues and costs, oncologic outcomes remain the cornerstone of any procedure to cure malignancies, for which the long-term results are critical. Furthermore, many of the minimally invasive esophageal operations should be compared to pharmacologic interventions and advanced pure endoscopic procedures; such a comparison requires a difficult literature analysis and leads to some confounding results of clinical trials. This review aims to examine the evidence for the use of MIS in both malignancies and more common benign disease of the esophagus, with a particular emphasis on future developments and ongoing areas of research. PMID:26843913

  17. The Tumor Microenvironment in Esophageal Cancer

    PubMed Central

    Lin, Eric W.; Karakasheva, Tatiana A.; Hicks, Philip D.; Bass, Adam J.; Rustgi, Anil K.

    2016-01-01

    Esophageal cancer is a deadly disease, ranking sixth among all cancers in mortality. Despite incremental advances in diagnostics and therapeutics, esophageal cancer still carries a poor prognosis, and thus there remains a need to elucidate the molecular mechanisms underlying this disease. There is accumulating evidence that a comprehensive understanding of the molecular composition of esophageal cancer requires attention to not only tumor cells but also the tumor microenvironment, which contains diverse cell populations, signaling factors, and structural molecules that interact with tumor cells and support all stages of tumorigenesis. In esophageal cancer, environmental exposures can trigger chronic inflammation, which leads to constitutive activation of pro-inflammatory signaling pathways that promote survival and proliferation. Anti-tumor immunity is attenuated by cell populations such as myeloid-derived suppressor cells (MDSCs) and regulatory T cells (Tregs), as well as immune checkpoints like programmed death-1 (PD-1). Other immune cells such as tumor-associated macrophages can have other pro-tumorigenic functions, including the induction of angiogenesis and tumor cell invasion. Cancer-associated fibroblasts secrete growth factors and alter the extracellular matrix (ECM) to create a tumor niche and enhance tumor cell migration and metastasis. Further study of how these TME components relate to the different stages of tumor progression in each esophageal cancer subtype will lead to development of novel and specific TME-targeting therapeutic strategies, which offer considerable potential especially in the setting of combination therapy. PMID:26923327

  18. Chronic Recurrent Esophageal Diverticulitis - A Rare Entity

    PubMed Central

    Manne, Ashish; Smith, Ioana; Hatchett, Jeremy; Juneau, Jeffrey; Kodali, Sudha; Malik, Talha A.; Weber, Fred H.

    2013-01-01

    In this report, we seek to shed light on a 44-year-old Caucasian male with a known history of an esophageal diverticulum, who was transferred to our facility after an upper endoscopy at an outside hospital suggested a purulent discharge emanating from the mouth of a mid-esophageal diverticulum. A barium swallow done at the outside institution had reportedly demonstrated an 8 cm long barium collection parallel to and anterolateral to the mid-and distal esophagus which terminated several centimeters proximal to the gastroesophageal junction. At our facility, antibiotics (piperacillin/tazobactam) were continued, and a double-contrast esophagram was performed. The presence of an unusual mid-esophageal diverticulum was confirmed. He clinically improved after a 3-day course of intravenous broad-spectrum antibiotics. No surgical or endoscopic repair was elected as the patient opted for continued medical management. While esophageal diverticula are not rare in humans, to our knowledge, this is the first report of development of esophageal diverticulitis in humans. We believe that antibiotic coverage in addition to dietary restriction is the logical mainstay of acute therapy. Optimal antibiotic coverage should likely include oral flora aerobes and anaerobes. Once symptoms resolve, diverticula may be managed expectantly.

  19. Pharmacological Management of Esophageal Food Bolus Impaction

    PubMed Central

    Khayyat, Yasir Mohammed

    2013-01-01

    Background. Soft esophageal bolus impaction is an emergency that requires skilled endoscopic removal if persistent obstructive symptoms do not resolve spontaneously after careful observation. Expedited care of these patients is crucial to avoid respiratory and mechanical complications. Other possible options for management include medical agents used to manage it prior to performing endoscopy if access to endoscopy was not available or declined by the patient. Aim. To review the available pharmacological and other nonmedicinal options and their mechanism of relief for soft esophageal impaction. Method. Pubmed, Medline and Ovid were used for search of MESH terms pertinent including “foreign body, esophageal, esophageal bolus and medical” for pharmacological and non medicinial agents used for management of esophageal soft bolus impaction as well as manual review of the cross-references. Results. Several agents were identified including Buscopan, Glucagon, nitrates, calcium channel blockers, and papaveretum. Non medicinal agents are water, effervescent agents, and papain. No evidence was found to suggest preference or effectiveness of use of a certain pharmacological agent compared to others. Buscopan, Glucagon, benzodiazepines, and nitrates were studied extensively and may be used in selected patients with caution. Use of papain is obsolete in management of soft bolus impaction. PMID:23738071

  20. Mutations in SOX2 cause anophthalmia-esophageal-genital (AEG) syndrome.

    PubMed

    Williamson, Kathleen A; Hever, Ann M; Rainger, Joe; Rogers, R Curtis; Magee, Alex; Fiedler, Zdenek; Keng, Wee Teik; Sharkey, Freddie H; McGill, Niolette; Hill, Clare J; Schneider, Adele; Messina, Mario; Turnpenny, Peter D; Fantes, Judy A; van Heyningen, Veronica; FitzPatrick, David R

    2006-05-01

    We report heterozygous, loss-of-function SOX2 mutations in three unrelated individuals with Anophthalmia-Esophageal-Genital (AEG) syndrome. One previously reported case [Rogers, R.C. (1988) Unknown cases. Proceedings of the Greenwood Genetic Center. 7, 57.] has a 2.7 Mb deletion encompassing SOX2 and associated with a cryptic translocation t(3;7)(q28;p21.3). The deletion and translocation breakpoints on chromosome 3q are >8.6 Mb apart and both chromosome rearrangements have occurred de novo. Another published case [Petrackova et al. (2004) Association of oesophageal atresia, anophthalmia and renal duplex. Eur. J. Pediatr., 163, 333-334.] has a de novo nonsense mutation, Q55X. A previously unreported case with severe bilateral microphthalmia and oesophageal atresia has a de novo missense mutation, R74P, that alters a highly evolutionarily conserved residue within the high mobility group domain, which is critical for DNA-binding of SOX2. In a yeast one-hybrid assay, this mutation abolishes Sox2-induced activation of the chick delta-crystallin DC5 enhancer. Four other reported AEG syndrome cases were extensively screened and do not have detectable SOX2 mutations. Two of these cases have unilateral eye malformations. SOX2 mutations are known to cause severe bilateral eye malformations but this is the first report implicating loss of function mutations in this transcription factor in oesophageal malformations. SOX2 is expressed in the developing foregut in mouse and zebrafish embryos and an apparently normal pattern of expression is maintained in Shh-/- mouse embryos, suggesting either that Sox2 acts upstream of Shh or functions in a different pathway. Three-dimensional reconstructions of the major morphological events in the developing foregut and eye from Carnegie Stages 12 and 13 human embryos are presented and compared with the data from model organisms. SOX2, with NMYC and CHD7, is now the third transcriptional regulator known to be critical for normal oesophageal

  1. [Antegrade flow in the aorta ascendens despite aortic atresia: 2 case reports with retrograde coronary perfusion through coronary fistulas and sinusoids].

    PubMed

    Jux, C; Kaulitz, R; von Wasielewski, R; Peuster, M; Fink, C; Paul, T; Hausdorf, G

    2000-06-01

    In aortic atresia, coronary perfusion normally occurs through retrograde blood flow in the ascending aorta. We report on two patients with antegrade flow in the ascending aorta despite aortic atresia. In one patient with hypoplastic left heart syndrome (aortic atresia, severe mitral stenosis), an intact interatrial septum/premature closure of the foramen ovale was found. While no other way of left atrial or ventricular decompression was found, echocardiography, angiography and the post-mortem examination showed left ventricular to coronary sinusoids as the sole pathway for systemic oxygenation. In a second patient with complex congenital heart disease, including aortic atresia, antegrade flow in the ascending aorta was through a left coronary fistula with shunt flow originating from the pulmonary trunk. This report describes systemic perfusion depending on retrograde coronary flow due to coronary-cameral (sinusoids) and coronary arterio-venous fistulas leading to the phenomenon of antegrade blood flow in the ascending aorta despite aortic atresia. PMID:10929434

  2. Endoscopic treatment of esophageal achalasia

    PubMed Central

    Esposito, Dario; Maione, Francesco; D’Alessandro, Alessandra; Sarnelli, Giovanni; De Palma, Giovanni D

    2016-01-01

    Achalasia is a motility disorder of the esophagus characterized by dysphagia, regurgitation of undigested food, chest pain, weight loss and respiratory symptoms. The most common form of achalasia is the idiopathic one. Diagnosis largely relies upon endoscopy, barium swallow study, and high resolution esophageal manometry (HRM). Barium swallow and manometry after treatment are also good predictors of success of treatment as it is the residue symptomatology. Short term improvement in the symptomatology of achalasia can be achieved with medical therapy with calcium channel blockers or endoscopic botulin toxin injection. Even though few patients can be cured with only one treatment and repeat procedure might be needed, long term relief from dysphagia can be obtained in about 90% of cases with either surgical interventions such as laparoscopic Heller myotomy or with endoscopic techniques such pneumatic dilatation or, more recently, with per-oral endoscopic myotomy. Age, sex, and manometric type by HRM are also predictors of responsiveness to treatment. Older patients, females and type II achalasia are better after treatment compared to younger patients, males and type III achalasia. Self-expandable metallic stents are an alternative in patients non responding to conventional therapies. PMID:26839644

  3. An Overview of the Diagnosis and Management of Eosinophilic Esophagitis

    PubMed Central

    Singla, Manish B; Moawad, Fouad J

    2016-01-01

    Eosinophilic esophagitis (EoE) is a chronic inflammatory condition characterized by symptoms of esophageal dysfunction and eosinophilic infiltration of the esophageal mucosa. The diagnosis requires esophageal biopsies demonstrating at least 15 eosinophils per high-powered field following a course of high-dose proton pump inhibitors. Management of EoE consists of the three Ds: drugs, dietary therapy, and esophageal dilation. In this review, we discuss the epidemiology, pathogenesis, diagnosis, and treatment of EoE to include the role of emerging therapies. PMID:26986655

  4. Congenital esophageal stenosis owing to tracheobronchial remnants

    PubMed Central

    Rebelo, Priscila Guyt; Ormonde, João Victor C.; Ormonde, João Baptista C.

    2013-01-01

    OBJECTIVE To emphasize the need of an accurate diagnosis of congenital esophageal stenosis due to tracheobronchial remnants, since its treatment differs from other types of congenital narrowing. CASE DESCRIPTION Four cases of lower congenital esophageal stenosis due to tracheobronchial remnants, whose definitive diagnosis was made by histopathology. Except for the last case, in which a concomitant anti-reflux surgery was not performed, all had a favorable outcome after resection and anastomosis of the esophagus. COMMENTS The congenital esophageal stenosis is an intrinsic narrowing of the organâ€(tm)s wall associated with its structural malformation. The condition can be caused by tracheobronchial remnants, fibromuscular stenosis or membranous diaphragm and the first symptom is dysphagia after the introduction of solid food in the diet. The first-choice treatment to tracheobronchial remnants cases is the surgical resection and end-to-end anastomosis of the esophagus. PMID:24142326

  5. Minimally invasive surgery for esophageal cancer.

    PubMed

    Santillan, Alfredo A; Farma, Jeffrey M; Meredith, Kenneth L; Shah, Nilay R; Kelley, Scott T

    2008-10-01

    Esophageal cancer represents a major public health problem worldwide. Several minimally invasive esophagectomy (MIE) techniques have been described and represent a safe alternative for the surgical management of esophageal cancer in selected centers with high volume and expertise in them. This article reviews the most recent and largest series evaluating MIE techniques. Recent larger series have shown MIE to be equivalent in postoperative morbidity and mortality rates to conventional surgery. MIE has been associated with less blood loss, less postoperative pain, and decreased intensive care unit and hospital length of stay compared with conventional surgery. Despite limited data, conventional surgery and MIE have shown no significant difference in survival, stage for stage. The myriad of MIE techniques complicates the debate of defining the optimal surgical approach for treating esophageal cancer. Randomized controlled trials comparing MIE with conventional open esophagectomy are needed to clarify the ideal procedure with the lowest postoperative morbidity, best quality of life after surgery, and long-term survival.

  6. Endoscopic options for early stage esophageal cancer

    PubMed Central

    Shah, Pari M.

    2015-01-01

    Surgery has traditionally been the preferred treatment for early stage esophageal cancer. Recent advances in endoscopic treatments have been shown to be effective and safe. Endoscopic mucosal resection (EMR) and endoscopic submucosal dissection (ESD) allow endoscopists to remove small, superficial lesions, providing tumor specimen that can be examined for accurate pathologic tumor staging and assessment of adequacy of resection. Endoscopic ablation procedures, including photodynamic therapy (PDT) and radio frequency ablation (RFA), have also been shown to safely and effectively treat esophageal dysplasia and early stage neoplasia, with excellent long-term disease control. Both approaches are becoming more widely available around the world, and provide an alternative, safe, low risk strategy for treating early stage disease, making combined endoscopic therapy the recommended treatment of choice for early stage esophageal cancers. PMID:25642334

  7. Methoxychlor directly affects ovarian antral follicle growth and atresia through Bcl-2- and Bax-mediated pathways.

    PubMed

    Miller, Kimberly P; Gupta, Rupesh K; Greenfeld, Chuck R; Babus, Janice K; Flaws, Jodi A

    2005-11-01

    Methoxychlor (MXC) is an organochlorine pesticide and reproductive toxicant. While in vivo studies indicate that MXC exposure increases antral follicle atresia, in part by altering apoptotic regulators (Bcl-2 and Bax), they do not distinguish whether MXC does so via direct or indirect mechanisms. Therefore, we utilized an in vitro follicle culture system to test the hypothesis that MXC is directly toxic to antral follicles, and that overexpression of anti-apoptotic Bcl-2, or deletion of pro-apoptotic Bax, protects antral follicles from MXC-induced toxicity. Antral follicles were isolated from wild-type (WT), Bcl-2 overexpressing (Bcl-2 OE), or Bax deficient (BaxKO) mice, and exposed to dimethylsulfoxide (control) or MXC (1-100 microg/ml) for 96 h. Follicle diameters were measured every 24 h to assess growth. After 96 h, follicles were histologically evaluated for atresia or collected for quantitative PCR analysis of Bcl-2 and Bax mRNA levels. MXC (10-100 microg/ml) significantly inhibited antral follicle growth at 72 and 96 h, and increased atresia (100 microg/ml) compared to controls at 96 h. Furthermore, MXC increased Bax mRNA levels between 48-96 h and decreased Bcl-2 mRNA levels at 96 h. While MXC inhibited growth of WT antral follicles beginning at 72 h, it did not inhibit growth of Bcl-2 OE or BaxKO follicles until 96 h. MXC also increased atresia of small and large WT and BaxKO antral follicles over controls, but it did not increase atresia of large Bcl-2 OE antral follicles over controls. These data suggest that MXC directly inhibits follicle growth partly by Bcl-2 and Bax pathways, and increases atresia partly through Bcl-2 pathways.

  8. Alterations in gene expression during fasting-induced atresia of early secondary ovarian follicles of coho salmon, Oncorhynchus kisutch.

    PubMed

    Yamamoto, Yoji; Luckenbach, J Adam; Young, Graham; Swanson, Penny

    2016-11-01

    Molecular processes that either regulate ovarian atresia or are consequences of atresia are poorly understood in teleost fishes. We hypothesized that feed restriction that perturbs normal ovarian growth and induces follicular atresia would alter ovarian gene expression patterns. Previtellogenic, two-year old coho salmon (Oncorhynchus kisutch) were subjected to prolonged fasting to induce atresia or maintained on a normal feeding schedule that would promote continued ovarian development. To identify genes that were specifically up- or down-regulated during oocyte growth in healthy, growing fish compared to fasted fish, reciprocal suppression subtractive hybridization (SSH) cDNA libraries were generated using ovaries from fed and fasted animals. Differential expression of genes identified by SSH was confirmed with quantitative PCR. The SSH library representing genes elevated in ovaries of fed fish relative to those of fasted fish contained steroidogenesis-related genes (e.g., hydroxy-delta-5-steroid dehydrogenase), Tgf-beta superfamily members (e.g., anti-Mullerian hormone) and cytoskeletal intermediate filament proteins (e.g., type I keratin s8). Overall, these genes were associated with steroid production, cell proliferation and differentiation, and ovarian epithelialization. The library representing genes elevated in ovaries of fasted fish relative to fed fish contained genes associated with apoptosis (e.g., programmed cell death protein 4), cortical alveoli (e.g., alveolin), the zona pellucida (e.g., zona pellucida protein c), and microtubules (e.g., microtubule associated protein tau). Elevated expression of this suite of genes was likely associated with the initiation of atresia and/or a reduced rate of follicle development in response to fasting. This study revealed ovarian genes involved in normal early secondary oocyte growth and potential early markers of atresia.

  9. Alterations in gene expression during fasting-induced atresia of early secondary ovarian follicles of coho salmon, Oncorhynchus kisutch.

    PubMed

    Yamamoto, Yoji; Luckenbach, J Adam; Young, Graham; Swanson, Penny

    2016-11-01

    Molecular processes that either regulate ovarian atresia or are consequences of atresia are poorly understood in teleost fishes. We hypothesized that feed restriction that perturbs normal ovarian growth and induces follicular atresia would alter ovarian gene expression patterns. Previtellogenic, two-year old coho salmon (Oncorhynchus kisutch) were subjected to prolonged fasting to induce atresia or maintained on a normal feeding schedule that would promote continued ovarian development. To identify genes that were specifically up- or down-regulated during oocyte growth in healthy, growing fish compared to fasted fish, reciprocal suppression subtractive hybridization (SSH) cDNA libraries were generated using ovaries from fed and fasted animals. Differential expression of genes identified by SSH was confirmed with quantitative PCR. The SSH library representing genes elevated in ovaries of fed fish relative to those of fasted fish contained steroidogenesis-related genes (e.g., hydroxy-delta-5-steroid dehydrogenase), Tgf-beta superfamily members (e.g., anti-Mullerian hormone) and cytoskeletal intermediate filament proteins (e.g., type I keratin s8). Overall, these genes were associated with steroid production, cell proliferation and differentiation, and ovarian epithelialization. The library representing genes elevated in ovaries of fasted fish relative to fed fish contained genes associated with apoptosis (e.g., programmed cell death protein 4), cortical alveoli (e.g., alveolin), the zona pellucida (e.g., zona pellucida protein c), and microtubules (e.g., microtubule associated protein tau). Elevated expression of this suite of genes was likely associated with the initiation of atresia and/or a reduced rate of follicle development in response to fasting. This study revealed ovarian genes involved in normal early secondary oocyte growth and potential early markers of atresia. PMID:27320185

  10. [Endoscopic ultrasonic diagnosis of esophageal cancer].

    PubMed

    Kouzu, T; Ogino, Y; Isono, K

    1986-08-01

    Endoscopic Ultrasonography (EUS) has been developed rapidly and is becoming a new routine examination of the digestive diseases. In this thesis, the usefulness of EUS with reference to the diagnosis of the depth and the margins of the cancer invasion and the metastatic lymph nodes is described. Furthermore, the judgment of the efficacy of the combined therapy including radiotherapy, chemotherapy and immunotherapy will be possible with EUS. The information from EUS is useful to determine the treatment plan of esophageal cancer. Therefore, EUS is expected to become a preoperative necessary examination of cases with esophageal cancer. PMID:3537360

  11. Eosinophilic esophagitis in adults: An update

    PubMed Central

    Ahmed, Monjur

    2016-01-01

    Eosinophilic esophagitis is a worldwide chronic allergic disease of the esophagus. In the last decade, there is an epidemic of this entity in the western world. Mostly seen in children and young adults, patients present with dysphagia or food impaction in the emergency room. Characteristic endoscopic findings, esophageal eosinophilia and non-responsiveness to proton pump inhibitors help make the diagnosis. Avoidance of food allergens, administration of steroidal anti-inflammatory medications and dilation of the esophagus are the mainstays of treatment. Investigations are ongoing for mucosal healing and optimum maintenance treatment. PMID:27158535

  12. Esophageal web in Plummer-Vinson syndrome.

    PubMed

    Okamura, H; Tsutsumi, S; Inaki, S; Mori, T

    1988-09-01

    In Plummer-Vinson syndrome, esophagography often reveals a web at the anterior wall of the cervical esophagus. The pathogenesis of the esophageal web and the cause of dysphagia in this syndrome were investigated radiographically, endoscopically, manometrically, and histologically. It was considered that the web seen in the esophagogram may have been formed due to the restriction of dilation of the esophageal wall, which results from repetitive inflammation and the subsequent healing process. Dysphagia in this syndrome may be explained by a decrease in swallowing power. Iron deficiency anemia may play the main role in the above histological changes and the resulting decrease in swallowing power.

  13. Venous Myocardial Infarction in an Infant with Obstructed Totally Anomalous Pulmonary Venous Drainage and Coronary Sinus Ostial Atresia

    PubMed Central

    Prasad, Deepa; Strainic, James P.; Pandya, Khyati; Kouretas, Peter C.

    2016-01-01

    We report a rare causal association between obstructed supracardiac totally anomalous pulmonary venous drainage and coronary sinus ostial atresia. Our 12-week-old patient developed venous myocardial infarction secondary to coronary venous hypertension because her sole route of coronary venous drainage was obstructed. She recovered after the obstruction was relieved by balloon dilation. Surgical repair then included anastomosis of the pulmonary venous confluence to the left atrium, ligation of the vertical vein, and unroofing of the coronary sinus. Coronary sinus ostial atresia is rarely diagnosed before autopsy. PMID:27777530

  14. Transcriptome Profiling of the Theca Interna from Bovine Ovarian Follicles during Atresia

    PubMed Central

    Hatzirodos, Nicholas; Irving-Rodgers, Helen F.; Hummitzsch, Katja; Rodgers, Raymond J.

    2014-01-01

    The theca interna is a specialized stromal layer that envelops each growing ovarian follicle. It contains capillaries, fibroblasts, immune cells and the steroidogenic cells that synthesize androgens for conversion to estradiol by the neighboring granulosa cells. During reproductive life only a small number of follicles will grow to a sufficient size to ovulate, whereas the majority of follicles will undergo regression/atresia and phagocytosis by macrophages. To identify genes which are differentially regulated in the theca interna during follicular atresia, we undertook transcriptome profiling of the theca interna from healthy (n = 10) and antral atretic (n = 5) bovine follicles at early antral stages (<5 mm). Principal Component Analyses and hierarchical classification of the signal intensity plots for the arrays showed primary clustering into two groups, healthy and atretic. A total of 543 probe sets were differentially expressed between the atretic and healthy theca interna. Further analyses of these genes by Ingenuity Pathway Analysis and Gene Ontology Enrichment Analysis Toolkit software found most of the genes being expressed were related to cytokines, hormones and receptors as well as the cell cycle and DNA replication. Cell cycle genes which encode components of the replicating chromosome complex and mitotic spindle were down-regulated in atretic theca interna, whereas stress response and inflammation-related genes such as TP53, IKBKB and TGFB1 were up-regulated. In addition to cell cycle regulators, upstream regulators that were predicted to be inhibited included Retinoblastoma 1, E2 transcription factor 1, and hepatocyte growth factor. Our study suggests that during antral atresia of small follicles in the theca interna, arrest of cell cycle and DNA replication occurs rather than up- regulation of apoptosis-associated genes as occurs in granulosa cells. PMID:24956388

  15. Effects of melatonin on follicular atresia and granulosa cell apoptosis in the porcine.

    PubMed

    He, Yamei; Deng, Honghui; Jiang, Zhongliang; Li, Qingwang; Shi, Meihong; Chen, Huali; Han, Zengsheng

    2016-08-01

    The accumulation of reactive oxygen species is detrimental to the health of the ovarian follicle. The protective, antioxidant properties of melatonin, an endogenous component of porcine follicular fluid, on apoptosis of granulosa cells were evaluated in this study. Porcine granulosa cells from medium-sized (3-5 mm), healthy follicles were cultured in serum-free conditions with melatonin (0, 0.01, 0.1, 1.0, 10, and 100 ng/mL) with or without its receptor antagonist, luzindole, followed by evaluation of apoptotic markers in the treated cells. Results revealed that endogenous, intrafollicular melatonin concentration decreased as follicular atresia progressed, whereas the percentage of apoptotic granulosa cells increased. Spontaneous apoptosis of granulosa cells, triggered by serum deprivation in vitro, was remarkably blocked by melatonin (1.0 ng/mL melatonin, 32.7 ± 0.5%, vs. control, 47.0 ± 1.0%; P < 0.05). Treatment with 1.0 ng/mL of melatonin also significantly elevated MT2, SOD1, and GPX4 while lowering FASL, CHOP, and GRP78 mRNA abundance compared to the untreated control. The anti-apoptotic effect and some changes of apoptotic-relevant genes in granulosa cells invoked by melatonin supplementation were markedly blocked by luzindole, suggesting that melatonin could prevent the apoptosis of porcine granulosa cells during follicular atresia via its membrane receptors and its free-radical-scavenging activity. These findings provide new insights into the regulatory mechanism of melatonin in follicular atresia-related functions. Mol. Reprod. Dev. 83: 692-700, 2016 © 2016 Wiley Periodicals, Inc. PMID:27391761

  16. Anatomic Variants on Computed Tomography in Congenital Aural Atresia and Stenosis

    PubMed Central

    Qin, Feng-hua; Dai, Peidong; Yang, Lin

    2015-01-01

    Objectives To quantitatively analyzing the anatomic variants on temporal computed tomography (CT) in congenital external auditory canal stenosis (EACS), congenital aural atresia (CAA), and normal ear structure. Methods Through a retrospective study, we analyzed 142 temporal high-resolution CT studies performed in 71 microtia patients. The following 6 parameters were compared among the three groups: Marx classification, medial canal diameter, vertical facial nerve (VFN) anterior displacement, tegmen mastoideum position, tympanic cavity volume, and malleus-incus joint or malleus-incus complex (MIC) area. Results The results showed that the microtia distributions in the Marx classification in these three groups were significantly different, as 86% (31 of 35) of ears with major microtia (third-degree dysplasia) had an atresia, and in 54.8% (23 of 42) of the minor microtic (first-degree or second-degree) ears, the bony or cartilaginous part of the external auditory canal was stenotic. Measurement data also showed that the potential medial canal diameter of the atresia group was obviously shorter than that of the stenosis group. The VFN anterior displacement and temporomandibular joint backward-shift together lead to medial canal diameters in ears with atresic canals that is smaller than those with stenotic canals. The tegmen mastoideum position was not significantly different between the three groups. Conclusion The mal-development of the external auditory canal is significantly associated with auricle and middle ear developmental anomalies. Compared with CAA ears, EACS have better development of the auricle, canal, tympanic cavity and MIC and relatively safer surgical operation except for the position of the tegmen mastoideum and the VFN. PMID:26622948

  17. Temporal and topographic changes in DNA synthesis after induced follicular atresia

    SciTech Connect

    Greenwald, G.S. )

    1989-07-01

    Hamsters were hypophysectomized on the morning of estrus (Day 1) and injected immediately with 30 IU pregnant mare's serum (PMS). This was followed on Day 4 by the injection of an antiserum to PMS (PMS-AS) that initiated follicular atresia (Time zero). From 0 to 72 h after PMS-AS, the animals were injected with (3H)thymidine and killed 4 h later. One ovary was saved for autoradiography and histology; from the other ovary, 5-10 large antral follicles were dissected and pooled, and incorporation into DNA was determined by scintillation counting. DNA synthesis dropped sharply between 12 and 18 h, coinciding with a fall in labeling index of the cumulus oophorus and thecal endothelial cells and a sharp fall in thecal vascularity. In contrast, for the mural granulosa cells bordering on the antral cavity, labeling index dropped sharply between 8 and 12 h when thecal vascularity was still high. The earliest sign of atresia was evident by 4 h in cumulus cells when, paradoxically, DNA synthesis was still high. It took 3 days for atresia of the antral follicles to progress to advanced stages, as evidenced by pseudo-pronuclei in the free floating ovum, further erosion of the mural granulosa, and minimal DNA/follicle. However, the theca still retained its histological integrity and contained no pyknotic cells. Although by 48 h the granulosal compartment was in disarray (DNA/follicle significantly different from earlier values), the egg was still viable, as judged by maximal fluorescence after the addition of fluoroscein diacetate.

  18. Characterization of glycoside residues of porcine zona pellucida and ooplasm during follicular development and atresia.

    PubMed

    Pastor, Luis M; Lucas, Xiomara; Pallares, Jacinto; Bernal-Mañas, Carmen M; Martinez, Emilio A; Roca, Jordi; Vazquez, Juan Maria; Morales, Eva; Beltran, Esther; Zuasti, Adelina; Ferrer, Concepcion

    2008-09-01

    The glycoside residues (glycoconjugates, GC) of the zona pellucida (ZP) glycoproteins are important during the first phases of fecundation. Our aim in this work was to determine the lectin affinity pattern of porcine ZP in order to analyze the changes that take place during: (a) preantral folliculogenesis, (b) the follicular atresia process, and (c) antral growth. Several prepubertal and adult pig ovaries and different sized antral follicles were used. Conventional carbohydrate histochemical techniques and peroxidase and digoxigenin (DIG) lectins were used to reveal the acid groups and the glycosidic residues of the ZP. It was seen that the ZP forms in the preantral follicles throughout their growth period. In primordial and primary follicles, ZP in the process of formation showed neutral GC. SBA, RCA-I, MAA, WGA lectins, and AAA after methylation-saponification (MS) were positive in the ZP of primordial and primary follicles. The affinity for SBA, RCA-I, MAA, and WGA increased in the multilaminar-primary follicles and new affinities for UEA-I and LFA were observed. After MS, AAA, SNA, PNA, and SBA reactivity was observed. The ZP of antral follicle oocytes of different sizes showed the same lectin pattern as multilaminar-primary follicles. The oocyte ooplasm and the follicular fluid of large antral follicles showed less affinity for WGA and LFA lectins and less intensive staining with AB (pH 2.5). Atresia did not change the antral or preantral follicle oocyte ZP lectin pattern. In conclusion, the follicles showed substantial changes in their ZP glycosidic composition as they developed, especially, during the change from primary to multilaminar-primary follicles. The ZP glycosidic composition showed no significant change during the growth of antral follicles and follicular atresia in our study.

  19. Outcomes at One-Year Post Anastomosis from a National Cohort of Infants with Oesophageal Atresia

    PubMed Central

    Allin, Benjamin; Knight, Marian; Johnson, Paul; Burge, David

    2014-01-01

    Background and Aims We aimed to provide a contemporaneous assessment of outcomes at one-year post oesophageal atresia/tracheoesophageal fistula (OA-TOF) repair, focussing particularly on post-operative complications. It is generally accepted that oesophageal stricture is the most common complication and causes significant morbidity. We also aimed to assess the efficacy of prophylactic anti-reflux medication (PARM) in reducing stricture formation. Method A prospective, multi-centre cohort study of all infants live-born with oesophageal atresia in the United Kingdom and Ireland in 2008/9 was performed, recording clinical management and outcomes at one year. The effect of PARM on stricture formation in infants with the type-c anomaly was assessed using logistic regression analysis. Results 151 infants were live-born with oesophageal atresia in the defined reporting period, 126 of whom had the type-c anomaly. One-year follow-up information was returned for 105 infants (70%); the mortality rate was 8.6% (95% CI 4.7–14.3%). Post-operative complications included anastomotic leak (5.4%), recurrent fistula (3.3%) and oesophageal stricture (39%). Seventy-six (60%) of those with type-c anomaly were alive at one-year with returned follow-up, 57(75%) of whom had received PARM. Of these, 24 (42%) developed a stricture, compared to 4 (21%) of those who had not received PARM (adjusted odds ratio 2.60, 95% CI 0.71–9.46, p = 0.147). Conclusions This study provides a benchmark for current outcomes and complication rates following OA-TOF repair, with oesophageal stricture causing significant morbidity. The use of PARM appeared ineffective in preventing strictures. This study creates enough doubt about the efficacy of PARM in preventing stricture formation to warrant further investigation of its use with a randomised controlled trial. PMID:25153838

  20. Perineal choristoma and atresia ani in 2 female Holstein Friesian calves.

    PubMed

    Binanti, D; Prati, I; Locatelli, V; Pravettoni, D; Sironi, G; Riccaboni, P

    2013-01-01

    Atresia ani, a congenital anomaly of the anus, can be associated with other types of malformation. Two female Holstein Friesian calves had imperforate anus, rectovaginal fistula, and perineal choristomas. In one case, the choristoma was composed of mature adipose and fibrous tissue with nephrogenic rests. In the other calf, the choristoma consisted of fragments of trabecular bone coated by cartilage and containing marrow, mixed with mature adipose and fibrous tissue, striated muscle fibers, nerves, and vessels. This combination of malformations resembles the association of anorectal malformations and perineal masses in children.

  1. Anal atresia, abnormal genitalia, and absent thumb: congenital malformations associated with mosaic ring chromosome 13.

    PubMed

    Ocak, Z; Ozlu, T; Vural, M

    2013-01-01

    Because of the deletion of a segment of the chromosome during the formation of a ring, several clinical findings may be associated with ring chromosomes. Ring chromosome 13 is one of such disorders in which the genotype-phenotype correlation is stronger by virtue of the accumulating literature. It can be associated with multiple congenital abnormalities and severe mental retardation. We report a case with mosaic ring chromosome 13 whose prenatal ultrasound revealed bilateral ventriculomegaly. Anal atresia, unidentifiable external genitalia, and an absent thumb were observed in the postmortem examination.

  2. Familial distal foregut atresia in a family with likely autosomal dominant inheritance pattern.

    PubMed

    Robinson, Ian; Gill, Harinder; Ng, Li Yen; Hayes, Roisin

    2012-11-01

    Familial occurrence of distal foregut atresia (DFA) (Type 1) is rare. Diagnosis is based upon the clinical symptomatology and confirmed by radiological studies, surgery and histology. A number of reports have described families in which several family members have been involved and suggested an autosomal recessive mode of inheritance. Little is known about the underlying genetic causes or indeed the likely pathogenic mechanism. We report a family in which there are five affected cases including three siblings where the DFA appears to be inherited in an autosomal dominant inheritance pattern with reduced penetrance.

  3. Surgical repair of coronary sinus orifice atresia with persistent left superior vena cava in heterotaxia.

    PubMed

    Takabayashi, Shin; Shimpo, Hideto; Yokoyama, Kazuto

    2007-05-01

    A 6-month-old boy was diagnosed with coronary sinus orifice atresia, double-outlet right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and moderate common atrioventricular valve regurgitation associated with heterotaxy syndrome. Cardiac venous flow drained through a persistent left superior vena cava. We decided to perform coronary sinus orifice unroofing through the right atrium under a guide using a bougie. The persistent left superior vena cava was divided. Bidirectional Glenn anastomosis and edge-to-edge common atrioventricular valve repair were concomitantly performed. After a 1-year follow-up period, the patient is alive and well without any ischemic event.

  4. Duodenal and jejunal atresia with agenesis of the dorsal mesentery: "apple peel" small bowel.

    PubMed

    Zivković, S M; Milosević, V R

    1979-05-01

    Two newborn infants with duodenal and jejunal atresia and agenesis of the dorsal mesentery represent our surgical experience with "apple peel" small bowel or "christmas tree" demormity. The first patient had the typical appearance of this condition. The postoperative course was complicated by hyperbilirubinemia, septicemia, and disseminated intravascular coagulation. The infant is in satisfactory condition 1.5 years after operation. The second patient had agenesis of the dorsal mesentery without spiraling of the bowel around its vascular stalk. The child died after 1 month, with complete absence of extrahepatic bile ducts as seen at a second laparotomy. Neither child had been subjected to gastrostomy.

  5. Distal Esophageal Duplication Cyst with Gastro-Esophageal Reflux Disease: A Rare Association and a Management Challenge.

    PubMed

    Jan, Iftikhar Ahmad; Al Nuaimi, Asma; Al Hamoudi, Basma; Al Naqbi, Khalid; Bilal, Mohammad

    2016-02-01

    Esophageal duplication cysts are rare congenital abnormalities of the foregut and may be associated with other conditions. Association of esophageal duplication with Gastro-Esophageal Reflux Disease (GERD) has not been reported in children. We are reporting a case of a 16 months baby who had antenatal diagnosis of diaphragmatic hernia. Postnatal CTchest, however, suggested a distal esophageal duplication cyst and a contrast esophagogram showed grade-IV GER. A thoracoscopy in another hospital excluded esophageal duplication at that time. Later, he presented with hematemesis in our department and was re-evaluated. Repeat CTconfirmed a persistent 2.5 x 1.3 cm cyst in distal esophagus. Upper GI endoscopy suggested grade-II esophagitis with a wide patent gastro-esophageal junction. The child was treated with left thoracotomy, excision of the duplication cyst and thoracic fundoplication. He had an uneventful post-operative recovery and is doing well at 6 months follow-up. PMID:26876405

  6. Family history of esophageal cancer increases the risk of esophageal squamous cell carcinoma.

    PubMed

    Chen, Tiantian; Cheng, Hongwei; Chen, Xingdong; Yuan, Ziyu; Yang, Xiaorong; Zhuang, Maoqiang; Lu, Ming; Jin, Li; Ye, Weimin

    2015-01-01

    A population-based case-control was performed to explore familial aggregation of esophageal squamous cell carcinoma (ESCC). Family history of cancer was assessed by a structured questionnaire, and from which 2 cohorts of relatives of cases and controls were reconstructed. Unconditional logistic regression and Cox proportional hazards regression were applied for case-control design and reconstructed cohort design, respectively. We observed a close to doubled risk of ESCC associated with a positive family history of esophageal cancer among first degree relatives (odds ratio [OR] = 1.85, 95% confidence interval [CI]: 1.42-2.41), after adjusting age, sex, family size and other confounders. The excess risks of ESCC increased with the increasing of first-degree relatives affected by esophageal cancer (p < 0.001). In particular, those individuals whose both parents with esophageal cancer had an 8-fold excess risk of ESCC (95% CI: 1.74-36.32). The reconstructed cohort analysis showed that the cumulative risk of esophageal cancer to age 75 was 12.2% in the first-degree relatives of cases and 7.0% in those of controls (hazard ratio = 1.91, 95% CI: 1.54-2.37). Our results suggest family history of esophageal cancer significantly increases the risk for ESCC. Future studies are needed to understand how the shared genetic susceptibility and/or environmental exposures contribute to the observed excess risk.

  7. PPI-responsive esophageal eosinophilia and eosinophilic esophagitis: More similarities than differences

    PubMed Central

    Eluri, Swathi; Dellon, Evan S.

    2015-01-01

    Purpose of review To discuss the clinical, endoscopic, and histologic features, pathogenesis, and disease mechanisms of proton pump inhibitor–responsive esophageal eosinophilia (PPI-REE), and to highlight similarities and differences with eosinophilic esophagitis (EoE). Recent findings PPI-REE is a condition in which patients have clinical and histologic findings similar to EoE, but achieve complete remission with proton pump inhibitor (PPI) treatment. More than one-third of patients who have esophageal symptoms associated with esophageal eosinophilia respond to PPI treatment. Emerging data elucidating the pathogenesis of PPI-REE have shown that Th2-related inflammatory factors such as IL-13, IL-5, eotaxin-3, and major basic protein (MBP) are elevated in PPI-REE, similar to EoE. PPI-REE also shares a genetic expression signature with EoE that reverses with PPI treatment. Mechanisms proposed to explain the PPI response include an acid-independent, anti-inflammatory action of PPIs and PPI-induced restoration of esophageal barrier function. Summary Multiple features of PPI-REE overlap extensively with EoE. This raises the question of whether PPI-REE is merely a subtype of EoE rather than an independent condition. This similarity may have future implications for algorithms informing evaluation and treatment of esophageal eosinophilia. PMID:26039722

  8. Histomorphological and Immunophenotypic Features of Pill-Induced Esophagitis

    PubMed Central

    Kim, Su Hwan; Kim, Won; Lee, Kook Lae; Byeon, Sun-ju; Choi, Euno; Chang, Mee Soo

    2015-01-01

    The aim of this study was to investigate histomorphological and immunophenotypic features in pill-induced esophagitis. We comparatively evaluated the histomorphological, immunophenotypic features of pill-induced esophagitis vs. reflux esophagitis, as well as clinical information and endoscopic findings. Fifty-two tissue pieces from 22 cases of pill-induced esophagitis, 46 pieces from 20 reflux esophagitis, and 16 pieces from 14 control samples were subjected to immunohistochemistry for inflammatory infiltrates (CD3 for T lymphocyte, CD20 for B lymphocyte, CD56 for NK cell, CD68 for macrophage, CD117 for mast cell) and eosinophil chemotaxis-associated proteins (Erk, leptin, leptin receptor, pSTAT3, phospho-mTOR). As a result, Histomorphology showed that a diffuse pattern of dilated intercellular spaces was more frequently observed in pill-induced esophagitis, while reactive atypia and subepithelial papillary elongation were more often found in reflux esophagitis (P < 0.05, respectively). Interestingly, intraepithelial eosinophilic microabscess, intraepithelial pustule and diffuse pattern of dilated intercellular spaces were observed in 14% (3 cases), 9% (2 cases) and 32% (7 cases) of pill-induced esophagitis, respectively, but in no cases of reflux esophagitis. Regarding intraepithelial inflammatory infiltrates in pill-induced esophagitis, T lymphocytes were the most common cells, followed by eosinophil; 11 and 7 in one x400 power field, respectively. Intraepithelial pSTAT3-positive pattern was more frequently observed in pill-induced esophagitis than in reflux esophagitis, at 45% (10 cases) versus 10% (2 cases), respectively (P < 0.05). Considering the distal esophageal lesion only, intraepithelial pustule, diffuse dilated intercellular spaces and stromal macrophages were more frequently found in distal pill-induced esophagitis, whereas reactive atypia and intraepithelial mast cells in reflux esophagitis (P < 0.05, respectively). In conclusion, diffuse dilated

  9. Preoperative therapy in locally advanced esophageal cancer

    PubMed Central

    Garg, Pankaj Kumar; Sharma, Jyoti; Jakhetiya, Ashish; Goel, Aakanksha; Gaur, Manish Kumar

    2016-01-01

    Esophageal cancer is an aggressive malignancy associated with dismal treatment outcomes. Presence of two distinct histopathological types distinguishes it from other gastrointestinal tract malignancies. Surgery is the cornerstone of treatment in locally advanced esophageal cancer (T2 or greater or node positive); however, a high rate of disease recurrence (systemic and loco-regional) and poor survival justifies a continued search for optimal therapy. Various combinations of multimodality treatment (preoperative/perioperative, or postoperative; radiotherapy, chemotherapy, or chemoradiotherapy) are being explored to lower disease recurrence and improve survival. Preoperative therapy followed by surgery is presently considered the standard of care in resectable locally advanced esophageal cancer as postoperative treatment may not be feasible for all the patients due to the morbidity of esophagectomy and prolonged recovery time limiting the tolerance of patient. There are wide variations in the preoperative therapy practiced across the centres depending upon the institutional practices, availability of facilities and personal experiences. There is paucity of literature to standardize the preoperative therapy. Broadly, chemoradiotherapy is the preferred neo-adjuvant modality in western countries whereas chemotherapy alone is considered optimal in the far East. The present review highlights the significant studies to assist in opting for the best evidence based preoperative therapy (radiotherapy, chemotherapy or chemoradiotherapy) for locally advanced esophageal cancer.

  10. Current Management of Eosinophilic Esophagitis 2015.

    PubMed

    Richter, Joel E

    2016-02-01

    Eosinophilic esophagitis (EoE) is a chronic inflammatory condition characterized by esophageal dysfunction and eosinophilic infiltrate (≥15/hpf) in the esophageal epithelium and the absence of other potential causes of eosinophilia. The prevalence is increasing and is the most common cause of solid food dysphagia in children and young adults. This article will review the diagnosis and management of EoE based on consensus conferences, systematic reviews, and meta-analysis and highlights seminal studies in our evolving treatment of this disease. However, all answers are not available and I will remark about the lessons learned in my clinical practice seeing EoE patients over the last 25 years. The complicated etiology of the complaint of dysphagia in EoE patients will be reviewed. The importance of utilizing endoscopy, biopsies, and barium esophagram to help define the 2 phenotypes (inflammatory, fibrostenosis) of EoE will be highlighted. The controversy about PPI-responsive esophageal eosinophilia will be discussed and contrasted with idiopathic EoE. Finally, the 3 treatment options for EoE (drugs, diet, dilation) will be reviewed in detail and a useful clinical management algorithm presented.

  11. Esophageal testing: What we have so far

    PubMed Central

    de Bortoli, Nicola; Martinucci, Irene; Bertani, Lorenzo; Russo, Salvatore; Franchi, Riccardo; Furnari, Manuele; Tolone, Salvatore; Bodini, Giorgia; Bolognesi, Valeria; Bellini, Massimo; Savarino, Vincenzo; Marchi, Santino; Savarino, Edoardo Vincenzo

    2016-01-01

    Gastroesophageal reflux disease (GERD) is a common disorder of the gastrointestinal tract. In the last few decades, new technologies have evolved and have been applied to the functional study of the esophagus, allowing for the improvement of our knowledge of the pathophysiology of GERD. High-resolution manometry (HRM) permits greater understanding of the function of the esophagogastric junction and the risks associated with hiatal hernia. Moreover, HRM has been found to be more reproducible and sensitive than conventional water-perfused manometry to detect the presence of transient lower esophageal sphincter relaxation. Esophageal 24-h pH-metry with or without combined impedance is usually performed in patients with negative endoscopy and reflux symptoms who have a poor response to anti-reflux medical therapy to assess esophageal acid exposure and symptom-reflux correlations. In particular, esophageal 24-h impedance and pH monitoring can detect acid and non-acid reflux events. EndoFLIP is a recent technique poorly applied in clinical practice, although it provides a large amount of information about the esophagogastric junction. In the coming years, laryngopharyngeal symptoms could be evaluated with up and coming non-invasive or minimally invasive techniques, such as pepsin detection in saliva or pharyngeal pH-metry. Future studies are required of these techniques to evaluate their diagnostic accuracy and usefulness, although the available data are promising. PMID:26909230

  12. 21 CFR 868.5650 - Esophageal obturator.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Esophageal obturator. 868.5650 Section 868.5650 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED... patient during emergency resuscitation by occluding (blocking) the esophagus, thereby permitting...

  13. 21 CFR 868.5650 - Esophageal obturator.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Esophageal obturator. 868.5650 Section 868.5650 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED... patient during emergency resuscitation by occluding (blocking) the esophagus, thereby permitting...

  14. Perception of Syllable Stress in Esophageal Speech.

    ERIC Educational Resources Information Center

    Walker, Christopher Niles; Morris, Hughlett L.

    1988-01-01

    Ten esophageal speakers and ten normal speakers produced repetitions of the disyllable /mama/ using five different conditions of syllable stress. Nine normal listeners judged both relative and absolute syllable stress. Reliable judgments were made of the syllable stress, and speakers were able to effect systematic changes in listener perceptions…

  15. Does surgery correct esophageal motor dysfunction in gastroesophageal reflux

    SciTech Connect

    Russell, C.O.; Pope, C.E.; Gannan, R.M.; Allen, F.D.; Velasco, N.; Hill, L.D.

    1981-09-01

    The high incidence of dysphagia in patients with symptomatic gastroesophageal reflux (GER) but no evidence of peptic stricture suggests esophageal motor dysfunction. Conventional methods for detecting dysfunction (radiologic and manometric examinations) often fail to detect abnormality in these patients. Radionuclide transit (RT), a new method for detecting esophageal motor dysfunction, was used to prospectively assess function in 29 patients with symptomatic GER uncomplicated by stricture before and three months after antireflux surgery (HILL). The preoperative incidence of dysphagia and esophageal dysfunction was 73% and 52%, respectively. During operation (Hill repair), intraoperative measurement of the lower esophageal sphincter pressure was performed and the LESP raised to levels between 45 and 55 mmHg. The preoperative lower esophageal sphincter pressure was raised from a mean of 8.6 mmHg, to mean of 18.5 mmHg after operation. No patient has free reflux after operation. Postoperative studies on 20 patients demonstrated persistence of all preoperative esophageal dysfunction despite loss of dysphagia. RT has demonstrated a disorder of esophageal motor function in 52% of patients with symptomatic GER that may be responsible for impaired esophageal clearance. This abnormality is not contraindication to surgery. The results indicate that construction of an effective barrier to reflex corrects symptoms of reflux, even in the presence of impaired esophageal transit. Radionuclide transit is a safe noninvasive test for assessment of esophageal function.

  16. Exudative retinopathy, cerebral calcifications, duodenal atresia, preaxial polydactyly, micropenis, microcephaly and short stature: a new syndrome?

    PubMed

    Isidor, Bertrand; Le Meur, Guylène; Conti, Carole; Caldagues, Emmanuelle; Lainey, Elodie; Launay, Elise; Leclair, Marc David; Le Francois, Thomas; Pichon, Olivier; Boisseau, Pierre; Migraine, Audrey; Keren, Boris; Le Caignec, Cédric; Crow, Yanick J; David, Albert

    2013-08-01

    The association of Coats disease with intrauterine growth retardation, intracranial calcification, leukodystrophy, brain cysts, osteopenia, and gastrointestinal bleeding defines Coats plus syndrome caused by mutations in the CTC1 gene, encoding conserved telomere maintenance component 1. Here, we report on a child with exudative retinopathy, cerebral calcifications, duodenal atresia, preaxial polydactyly, micropenis, microcephaly, and short stature, in whom no mutations in CTC1 were found. Our patient shares some features seen in other diseases associated with telomere shortening including Hoyeraal-Hreidarsson and Revesz syndromes. We therefore measured telomere length by Flow-Fish which was normal. The association of duodenal atresia and microcephaly also suggested a diagnosis of Feingold syndrome. However, direct sequencing of MYCN was normal, and we did not detect any hemizygous deletion of the miR-17∼92 polycistronic miRNA cluster. To our knowledge, the phenotype we report on has not been described previously, leading us to speculate that this condition may represent a new syndrome.

  17. Definition of a Critical Region on Chromosome 18 for Congenital Aural Atresia by ArrayCGH

    PubMed Central

    Veltman, Joris A.; Jonkers, Yvonne; Nuijten, Inge; Janssen, Irene; van der Vliet, Walter; Huys, Erik; Vermeesch, Joris; Van Buggenhout, Griet; Fryns, Jean-Pierre; Admiraal, Ronald; Terhal, Paulien; Lacombe, Didier; van Kessel, Ad Geurts; Smeets, Dominique; Schoenmakers, Eric F. P. M.; van Ravenswaaij-Arts, Conny M.

    2003-01-01

    Deletions of the long arm of chromosome 18 occur in ∼1 in 10,000 live births. Congenital aural atresia (CAA), or narrow external auditory canals, occurs in ∼66% of all patients who have a terminal deletion 18q. The present report describes a series of 20 patients with CAA, of whom 18 had microscopically visible 18q deletions. The extent and nature of the chromosome-18 deletions were studied in detail by array-based comparative genomic hybridization (arrayCGH). High-resolution chromosome-18 profiles were obtained for all patients, and a critical region of 5 Mb that was deleted in all patients with CAA could be defined on 18q22.3-18q23. Therefore, this region can be considered as a candidate region for aural atresia. The array-based high-resolution copy-number screening enabled a refined cytogenetic diagnosis in 12 patients. Our approach appeared to be applicable to the detection of genetic mosaicisms and, in particular, to a detailed delineation of ring chromosomes. This study clearly demonstrates the power of the arrayCGH technology in high-resolution molecular karyotyping. Deletion and amplification mapping can now be performed at the submicroscopic level and will allow high-throughput definition of genomic regions harboring disease genes. PMID:12740760

  18. A novel association of alveolar capillary dysplasia, atypical duodenal atresia, and subglottic stenosis.

    PubMed

    Shimizu, Takeru; Fukuda, Taeko; Inomata, Shinichi; Satsumae, Tsuyoshi; Tanaka, Makoto

    2011-04-01

    Alveolar capillary dysplasia (ACD), which is a rare and lethal congenital pulmonary anomaly found in newborns, begins its onset or causes deterioration of the infant's condition some time after birth. Various congenital anomalies in combination with ACD have been reported, except for subglottic stenosis. Therefore, we aim to report a novel association in a case of ACD with the combination of atypical duodenal atresia and subglottic stenosis. The male infant was scheduled for duodeno-duodenostomy because a double-bubble sign was observed on a chest radiograph. He arrived at the operating theater without any symptoms. After induction of general anesthesia, although mask ventilation was performed without difficulties throughout the entire procedure, oxygen saturation values of the upper and lower extremities dissociated after several attempts of intubation. Surgery was canceled because of instability of the respiratory condition. Respiratory insufficiency worsened progressively, and the infant died at 5 days of age. An autopsy confirmed ACD and revealed cartilaginous subglottic stenosis, which had made intubation difficult. This report highlights the hazards of the onset and worsening of ACD, and the importance of thorough echocardiography before surgery when atypical duodenal atresia is suspected. Anesthesiologists should also be prepared for the difficulty of intubation.

  19. Esophageal Manometry in Patients with Chest Pain and Normal Coronary Arteriogram.

    PubMed

    Ferguson, S C; Hodges, K; Hersh, T; Jinich, H

    1981-02-01

    Evaluation of the esophagus is helpful in determining the source of chest pain. Eighteen per cent of 72 patients with a normal coronary angiogram had esophageal disease as a source of chest pain. Eight had diffuse esophageal spasm, four had reflux esophagitis and one had an esophageal ulcer. Five of eight patients with diffuse esophageal spasm had relief of symptoms with nitroglycerin. Despite normal coronary arteriogram and normal esophageal manometry 42 of 49 other patients had relief of chest pain with nitroglycerin.

  20. Estrogen receptor alpha overexpressing mouse antral follicles are sensitive to atresia induced by methoxychlor and its metabolites.

    PubMed

    Paulose, Tessie; Hannon, Patrick R; Peretz, Jackye; Craig, Zelieann R; Flaws, Jodi A

    2012-06-01

    Methoxychlor (MXC) and its metabolites bind to estrogen receptors (ESRs) and increase ovarian atresia. To test whether ESR alpha (ESR1) overexpressing (ESR1 OE) antral follicles are more sensitive to atresia compared to controls, we cultured antral follicles with vehicle, MXC (1-100 μg/ml) or metabolites (0.1-10 μg/ml). Results indicate that MXC and its metabolites significantly increase atresia in ESR1 OE antral follicles at lower doses compared to controls. Activity of pro-apoptotic factor caspase-3/7 was significantly higher in ESR1 OE treated antral follicles compared to controls. ESR1 OE mice dosed with MXC 64 mg/kg/day had an increased percentage of atretic antral follicles compared to controls. Furthermore, pro-caspase-3 levels were found to be significantly lower in ESR1 OE ovaries than controls dosed with MXC 64 mg/kg/day. These data suggest that ESR1 OE ovaries are more sensitive to atresia induced by MXC and its metabolites in vitro and in vivo compared to controls.

  1. Natural Killer Cells Promote Long-Term Hepatobiliary Inflammation in a Low-Dose Rotavirus Model of Experimental Biliary Atresia

    PubMed Central

    Squires, James E.; Shivakumar, Pranavkumar; Mourya, Reena; Bessho, Kazuhiko; Walters, Stephanie; Bezerra, Jorge A.

    2015-01-01

    Biliary atresia is a rapidly progressive obstructive cholangiopathy of infants. Mechanistic studies in the mouse model of Rhesus rotavirus (RRV)-induced biliary atresia have linked the importance of effector lymphocytes to the pathogenesis of extrahepatic bile duct (EHBD) injury and obstruction in experimental biliary atresia; however, studies of the progressive liver injury have been limited by early death of newborn mice. Here, we aimed to determine 1) if a lower inoculum of RRV induces obstruction of EHBDs while allowing for ongoing liver inflammation, and 2) if NK cells regulate intrahepatic injury. The administration of 0.25x106 fluorescence forming units of RRV induced an obstructive extrahepatic cholangiopathy, but allowed for restoration of the duct epithelium, increased survival, and the development of a progressive intrahepatic inflammatory injury with molecular and cellular signatures equivalent to the traditional infectious model. Investigating the mechanisms of liver injury, we found that NK cell depletion at the onset of jaundice decreased liver inflammation, suppressed the expression of fibrosis and inflammation/immunity genes, lowered plasma ALT and bilirubin and improved survival. Conclusions Lower inoculation of RRV-induced progressive liver injury and fibrosis via NK cells. These findings point to the potential use of NK cell-depleting strategies to block progression of liver disease in biliary atresia. PMID:25992581

  2. Radiohumeral synostosis, femoral bowing, other skeletal anomalies and anal atresia, a variant example of Antley-Bixler syndrome?

    PubMed

    Antich, J; Iriondo, M; Lizarraga, I; Manzanares, R; Cusi, V

    1993-01-01

    We report a newborn with radiohumeral synostosis, femoral bowing, anal atresia, a prominent nose (pear shaped nose), slender ribs, long tapering fingers with distal camptodactyly, genital hypoplasia and a neonatal humeral fracture. Among the possible differential diagnoses a variant example of Antley-Bixler syndrome is considered to be the most likely final diagnosis.

  3. Characteristics and frequency of transient relaxations of the lower esophageal sphincter in patients with reflux esophagitis.

    PubMed

    Mittal, R K; McCallum, R W

    1988-09-01

    Electromyogram of the submental muscles, esophageal manometry, and pH studies were simultaneously performed in an unselected group of 12 patients with subjective and objective evidence of gastroesophageal reflux (GER) disease to determine the frequency of transient relaxation of the lower esophageal sphincter (LES) and mechanisms of GER. Findings from these patients were compared with data from 10 asymptomatic healthy volunteers. Recordings were obtained for 1 h in the fasting state and 3 h after a standard 850-kcal meal. Transient relaxation of the LES was the only mechanism of acid reflux in normal subjects and accounted for 73.0% of the episodes of acid reflux in patients with GER disease. In both normal subjects and patients with GER, a large number of transient relaxations were associated at their onset with an attenuated submental EMG complex, a small pharyngeal contraction, and an esophageal contraction. The incidences of these associated events were similar in the two study populations. The frequency of transient relaxation of the LES in patients with GER was identical to that of controls. The frequency did not differ even in 9 patients with GER disease who had endoscopic esophagitis. Thirty-six percent of transient relaxations in the normal subjects were accompanied by pH evidence of reflux, but in the GER patients with endoscopic esophagitis 65% of the transient LES relaxations resulted in a reflux event. Acid reflux at the moment of deep inspiration was the second most common mechanism of GER in our patients. Four patients who demonstrated this mechanism had hiatal hernias and more severe esophagitis than the rest of the group. Our findings confirm that transient relaxation of the LES is the major mechanism of GER in patients with reflux esophagitis. However, the similar frequency of this relaxation in GER patients and in healthy asymptomatic subjects suggests that factors other than transient LES relaxation play an important role in the pathogenesis of

  4. Fungal Esophagitis in a Child with Insulin Dependent Diabetes Mellitus.

    PubMed

    Saeed, Anjum; Assiri, Asaad; Zaidi, Zafar; Alsheikh, Abdulmalik

    2016-08-01

    Esophagitis in children is not uncommon, mostly due to gastro-esophageal reflux. Other conditions like eosinophilic and infective esophagitis need to be elucidated in differential diagnoses. Fungal orCandida esophagitisusually occurs in high risk children who are immune-compromised, malnourished, on steroid therapy or have uncontrolled diabetes mellitus. An eleven-year girl presented with uncontrolled type I diabetes mellitus and recurrent epigastric pain with vomiting. Her oral intake was satisfactory. There was no dysphagia and odynophagia. Physical examination was normal with good oral hygiene. Failure in responding to conventional medications led to endoscopic evaluation, which revealed white patches and esophageal inflammation and diagnosed as fungal esophagitis on histopathology. Although infective esophagitis is encountered sporadically in pediatric age group, but it should always be considered in high risk individuals and when conventional medication fails to resolve the symptoms. PMID:27539771

  5. Detection of esophageal ulcerations with technetium-99m albumin sucralfate

    SciTech Connect

    Goff, J.S.; Adcock, K.A.; Schmelter, R.

    1986-07-01

    Technetium-99m albumin-sucralfate ((/sup 99m/Tc)Su) can be used to demonstrate peptic ulcer disease in man and animals. We evaluated the usefulness of (/sup 99m/Tc)Su for detecting various grades of esophagitis. (/sup 99m/Tc)Su adhered to the distal esophagus for up to 3 hr in five of six patients with esophageal ulcers but adhered to only two of nine with lesser degrees of esophagitis. No adherence was seen in five patients without esophagitis. Thus, (/sup 99m/Tc)Su may not be useful for detecting any but the most severe grade of esophagitis. Based on these results, we speculate that the previously documented beneficial effects of sucralfate on mild to moderate esophagitis may be due to other mechanisms besides adherence to the ulcerated mucosa.

  6. Genes Regulating Epithelial Polarity Are Critical Suppressors of Esophageal Oncogenesis

    PubMed Central

    Li, Xiu-Min; Wang, Hui; Zhu, Li-Li; Zhao, Run-Zhen; Ji, Hong-Long

    2015-01-01

    Esophageal cancer is an aggressive disease featured by early lymphatic and hematogenous dissemination, and is the sixth leading cause of cancer-related deaths worldwide. The proper formation of apicobasal polarity is essential for normal epithelium physiology and tissue homeostasis, while loss of polarity is a hallmark of cancer development including esophageal oncogenesis. In this review, we summarized the stages of esophageal cancer development associated with the loss or deregulation of epithelial cell apicobasal polarity. Loss of epithelial apicobasal polarity exerts an indispensable role in the initiation of esophageal oncogenesis, tumor progression, and the advancement of tumors from benign to malignant. In particular, we reviewed the involvement of several critical genes, including Lkb1, claudin-4, claudin-7, Par3, Lgl1, E-cadherin, and the Scnn1 gene family. Understanding the role of apicobasal regulators may lead to new paradigms for treatment of esophageal tumors, including improvement of prognostication, early diagnosis, and individually tailored therapeutic interventions in esophageal oncology. PMID:26185530

  7. Prenatal Intestinal Obstruction Affects the Myenteric Plexus and Causes Functional Bowel Impairment in Fetal Rat Experimental Model of Intestinal Atresia

    PubMed Central

    Khen-Dunlop, Naziha; Sarnacki, Sabine; Victor, Anais; Grosos, Celine; Menard, Sandrine; Soret, Rodolphe; Goudin, Nicolas; Pousset, Maud; Sauvat, Frederique; Revillon, Yann; Cerf-Bensussan, Nadine; Neunlist, Michel

    2013-01-01

    Background Intestinal atresia is a rare congenital disorder with an incidence of 3/10 000 birth. About one-third of patients have severe intestinal dysfunction after surgical repair. We examined whether prenatal gastrointestinal obstruction might effect on the myenteric plexus and account for subsequent functional disorders. Methodology/Principal Findings We studied a rat model of surgically induced antenatal atresia, comparing intestinal samples from both sides of the obstruction and with healthy rat pups controls. Whole-mount preparations of the myenteric plexus were stained for choline acetyltransferase (ChAT) and nitric oxide synthase (nNOS). Quantitative reverse transcription PCR was used to analyze mRNAs for inflammatory markers. Functional motility and permeability analyses were performed in vitro. Phenotypic studies were also performed in 8 newborns with intestinal atresia. In the experimental model, the proportion of nNOS-immunoreactive neurons was similar in proximal and distal segments (6.7±4.6% vs 5.6±4.2%, p = 0.25), but proximal segments contained a higher proportion of ChAT-immunoreactive neurons (13.2±6.2% vs 7.5±4.3%, p = 0.005). Phenotypic changes were associated with a 100-fold lower concentration-dependent contractile response to carbachol and a 1.6-fold higher EFS-induced contractile response in proximal compared to distal segments. Transcellular (p = 0.002) but not paracellular permeability was increased. Comparison with controls showed that modifications involved not only proximal but also distal segments. Phenotypic studies in human atresia confirmed the changes in ChAT expression. Conclusion Experimental atresia in fetal rat induces differential myenteric plexus phenotypical as well as functional changes (motility and permeability) between the two sides of the obstruction. Delineating these changes might help to identify markers predictive of motility dysfunction and to define guidelines for post-surgical care. PMID:23667464

  8. HER2 amplification, overexpression and score criteria in esophageal adenocarcinoma

    PubMed Central

    Hu, Yingchuan; Bandla, Santhoshi; Godfrey, Tony E.; Tan, Dongfeng; Luketich, James D.; Pennathur, Arjun; Qiu, Xing; Hicks, David G.; Peters, Jeffrey; Zhou, Zhongren

    2011-01-01

    The HER2 oncogene was recently reported to be amplified and overexpressed in esophageal adenocarcinoma. However, the relationship of HER2 amplification in esophageal adenocarcinoma with prognosis has not been well defined. The scoring systems for clinically evaluating HER2 in esophageal adenocarcinoma are not established. The aims of the study were to establish a HER2 scoring system and comprehensively investigate HER2 amplification and overexpression in esophageal adenocarcinoma and its precursor lesion. Using a tissue microarray, containing 116 cases of esophageal adenocarcinoma, 34 cases of BE, 18 cases of low grade dysplasia and 15 cases of high grade dysplasia, HER2 amplification and overexpression were analyzed by HercepTest and CISH methods. The amplification frequency in an independent series of 116 esophageal adenocarcinoma samples was also analyzed using Affymetrix SNP 6.0 microarrays. In our studies, we have found that HER2 amplification does not associate with poor prognosis in total 232 esophageal adenocarcinoma patients by CISH and high density microarrays. We further confirm the similar frequency of HER2 amplification by CISH (18.10%; 21/116) and SNP 6.0 microarrays (16.4%, 19/116) in esophageal adenocarcinoma. HER2 protein overexpression was observed in 12.1 % (14/116) of esophageal adenocarcinoma and 6.67% (1/15) of HGD. No HER2 amplification or overexpression was identified in BE or LGD. All HER2 protein overexpression cases showed HER2 gene amplification. Gene amplification was found to be more frequent by CISH than protein overexpression in esophageal adenocarcinoma (18.10% vs 12.9%). A modified two-step model for esophageal adenocarcinoma HER-2 testing is recommend for clinical esophageal adenocarcinoma HER-2 trial. PMID:21460800

  9. Adult-Onset Esophageal Crohn’s Disease

    PubMed Central

    Kasarala, George; Durrett, Sam

    2016-01-01

    Crohn’s disease (CD) is an idiopathic inflammatory bowel disease that can involve any part of the gastrointestinal tract. Esophageal involvement is rarely seen in adults, especially at the initial diagnosis of CD. Esophageal symptoms as primary manifestations of the disease are extremely rare. We report a case of a CD with esophageal involvement at the time of her initial diagnosis of CD. PMID:27761477

  10. Surgical treatment analysis of idiopathic esophageal achalasia

    PubMed Central

    de AQUINO, José Luis Braga; SAID, Marcelo Manzano; PEREIRA, Douglas Rizzanti; do AMARAL, Paula Casals; LIMA, Juliana Carolina Alves; LEANDRO-MERHI, Vânia Aparecida

    2015-01-01

    Background Idiopathic esophageal achalasia is an inflammatory disease of unknown origin, characterized by aperistalsis of the esophageal body and failure of the lower esophageal sphincter in response to swallowing, with consequent dysphagia. Aim To demonstrate the results of surgical therapy in these patients, evaluating the occurred local and systemic complications. Methods Were studied retrospectively 32 patients, 22 of whom presented non-advanced stage of the disease (Stage I/II) and 10 with advanced disease (Stage III/IV). All of them had the clinical conditions to be submitted to surgery. The diagnoses were done by clinical, endoscopic, cardiological, radiological and esophageal manometry analysis. Pre-surgical evaluation was done with a questionnaire based on the most predisposing factors in the development of the disease and the surgical indication was based on the stage of the disease. Results The patients with non-advanced stages were submitted to cardiomyotomy with fundoplication, wherein in the post-surgical early assessment, only one (4,4%) presented pulmonary infection, but had a good outcome. In patients with advanced disease, seven were submitted to esophageal mucosectomy preserving the muscular layer, wherein one patient (14,2%) presented dehiscence of gastric cervical esophagus anastomosis as well as pulmonary infection; all of these complications were resolved with proper specific treatment; the other three patients with advanced stage were submitted to transmediastinal esophagectomy; two of them presented hydropneumothorax with good evolution, and one of them also presented fistula of the cervical esophagogastric anastomosis, but with spontaneous healing after conservative treatment and nutritional support. The two patients with fistula of the cervical anastomosis progressed to stenosis, with good results after endoscopic dilations. In the medium and long term assessment done in 23 patients, all of them reported improvement in life quality, with

  11. Desmoglein-1 regulates esophageal epithelial barrier function and immune responses in eosinophilic esophagitis

    PubMed Central

    Sherrill, J D; KC, K; Wu, D; Djukic, Z; Caldwell, J M; Stucke, E M; Kemme, K A; Costello, M S; Mingler, M K; Blanchard, C; Collins, M H; Abonia, J P; Putnam, P E; Dellon, E S; Orlando, R C; Hogan, S P; Rothenb, M E

    2014-01-01

    The desmosomal cadherin desmoglein-1 (DSG1) is an essential intercellular adhesion molecule that is altered in various human cutaneous disorders; however, its regulation and function in allergic disease remains unexplored. Herein, we demonstrate a specific reduction in DSG1 in esophageal biopsies from patients with eosinophilic esophagitis (EoE), an emerging allergic disorder characterized by chronic inflammation within the esophageal mucosa. Further, we show that DSG1 gene silencing weakens esophageal epithelial integrity, and induces cell separation and impaired barrier function (IBF) despite high levels of desmoglein-3 (DSG3). Moreover, DSG1 deficiency induces transcriptional changes that partially overlap with the transcriptome of inflamed esophageal mucosa; notably, periostin, a multipotent pro-inflammatory extracellular matrix molecule, is the top induced overlapping gene. We further demonstrate that IBF is a pathological feature in EoE, which can be partially induced through the downregulation of DSG1 by interleukin-13 (IL-13). Taken together, these data identify a functional role for DSG1 and its dysregulation by IL-13 in the pathophysiology of EoE and suggest that the loss of DSG1 may potentiate allergic inflammation through the induction of pro-inflammatory mediators such as periostin. PMID:24220297

  12. Desmoglein-1 regulates esophageal epithelial barrier function and immune responses in eosinophilic esophagitis.

    PubMed

    Sherrill, J D; Kc, K; Wu, D; Djukic, Z; Caldwell, J M; Stucke, E M; Kemme, K A; Costello, M S; Mingler, M K; Blanchard, C; Collins, M H; Abonia, J P; Putnam, P E; Dellon, E S; Orlando, R C; Hogan, S P; Rothenberg, M E

    2014-05-01

    The desmosomal cadherin desmoglein-1 (DSG1) is an essential intercellular adhesion molecule that is altered in various human cutaneous disorders; however, its regulation and function in allergic disease remains unexplored. Herein, we demonstrate a specific reduction in DSG1 in esophageal biopsies from patients with eosinophilic esophagitis (EoE), an emerging allergic disorder characterized by chronic inflammation within the esophageal mucosa. Further, we show that DSG1 gene silencing weakens esophageal epithelial integrity, and induces cell separation and impaired barrier function (IBF) despite high levels of desmoglein-3. Moreover, DSG1 deficiency induces transcriptional changes that partially overlap with the transcriptome of inflamed esophageal mucosa; notably, periostin (POSTN), a multipotent pro-inflammatory extracellular matrix molecule, is the top induced overlapping gene. We further demonstrate that IBF is a pathological feature in EoE, which can be partially induced through the downregulation of DSG1 by interleukin-13 (IL-13). Taken together, these data identify a functional role for DSG1 and its dysregulation by IL-13 in the pathophysiology of EoE and suggest that the loss of DSG1 may potentiate allergic inflammation through the induction of pro-inflammatory mediators such as POSTN. PMID:24220297

  13. Intramural esophagic hematoma secondary to coumarinic anticoagulation: a case report

    PubMed Central

    2009-01-01

    Esophagic Intramural Hematoma is an uncommon clinical condition, with a prognosis which is essentially benign. On most cases, a predisposing or precipitating factor may be seen, with the most common ones being the history of esophagic instrumentation, food impactations and thrombocytopenia. In the following manuscript, the authors present the case of a 54-years-old male with history of valve replacement surgery, who was treated at the Clinica Cardiovascular (Medellin, Colombia), with a clinical case of Intramural Esophagic Hematoma that was later confirmed to be due to a Coumarinic overanticoagulation. On this case, it is evidenced that Intramural Esophagic Hematoma is an unrecognized complication of Courmarinic anticoagulation therapy. PMID:20069068

  14. [A case of esophageal achalasia followed by Parkinson's disease].

    PubMed

    Mitani, Maki; Kawamoto, Kunihiko; Funakawa, Itaru; Jinnai, Kenji

    2005-08-01

    In 1992, a 63 year-old woman complained of dysphagia and chest pain, and was diagnosed with esophageal achalasia. Three years later, she developed resting tremor, cog-wheel rigidity, and retro-pulsion, and was diagnosed with Parkinson's disease and given appropriate medication. Several years later, intractable vomitting and aspiration pneumonia developed, and the lower esophageal sphincter was dilated using a pneumatic balloon dilator under gastroscopic guidance in 2004. That procedure improved her symptoms and the esophageal dilation was visualized on chest CT images. Herein, we report this rare case of esophageal achalasia followed by Parkinson's disease and discuss the relationship between the two diseases.

  15. Eosinophilic esophagitis as paraneoplastic syndrome in a patient with ganglioneuroblastoma.

    PubMed

    Prader, S; Spalinger, J; Caduff, J; Hürlimann, S; Rischewski, J

    2015-05-01

    A 16-month-old boy presented with failure to thrive despite sufficient caloric intake, hypersalivation, abdominal pain, chronic diarrhea and blepharitis. An eosinophilic esophagitis (EoE) was diagnosed by esophageal biopsy. Dietary restrictions and topical steroid treatment lead to no improvement. Further diagnostic work-up revealed an intrathoracal, paraspinal ganglioneuroblastoma. After operative extirpation of the tumour, all initial symptoms resolved. An esophageal control biopsy 4 weeks after tumour resection was normal. This is the first report of eosinophilic esophagitis as part of a paraneoplastic syndrome in a patient with a malignant disease other than a carcinoma. PMID:25985452

  16. Transcriptome profiling of granulosa cells from bovine ovarian follicles during atresia

    PubMed Central

    2014-01-01

    Background The major function of the ovary is to produce oocytes for fertilisation. Oocytes mature in follicles surrounded by nurturing granulosa cells and all are enclosed by a basal lamina. During growth, granulosa cells replicate and a large fluid-filled cavity (the antrum) develops in the centre. Only follicles that have enlarged to over 10 mm can ovulate in cows. In mammals, the number of primordial follicles far exceeds the numbers that ever ovulate and atresia or regression of follicles is a mechanism to regulate the number of oocytes ovulated and to contribute to the timing of ovulation. To better understand the molecular basis of follicular atresia, we undertook transcriptome profiling of granulosa cells from healthy (n = 10) and atretic (n = 5) bovine follicles at early antral stages (< 5 mm). Results Principal Component Analysis (PCA) and hierarchical classification of the signal intensity plots for the arrays showed primary clustering into two groups, healthy and atretic. These analyses and size-frequency plots of coefficients of variation of signal intensities revealed that the healthy follicles were more heterogeneous. Examining the differentially-expressed genes the most significantly affected functions in atretic follicles were cell death, organ development, tissue development and embryonic development. The overall processes influenced by transcription factor gene TP53 were predicted to be activated, whereas those of MYC were inhibited on the basis of known interactions with the genes in our dataset. The top ranked canonical pathway contained signalling molecules common to various inflammatory/fibrotic pathways such as the transforming growth factor-β and tumour necrosis factor-α pathways. The two most significant networks also reflect this pattern of tissue remodelling/fibrosis gene expression. These networks also contain molecules which are present in the canonical pathways of hepatic fibrosis/hepatic stellate cell activation and transforming

  17. Sphingosine-1-phosphate and ceramide are associated with health and atresia of bovine ovarian antral follicles.

    PubMed

    Hernández-Coronado, C G; Guzmán, A; Espinosa-Cervantes, R; Romano, M C; Verde-Calvo, J R; Rosales-Torres, A M

    2015-02-01

    The follicle destiny towards ovulation or atresia is multi-factorial in nature and involves outcries, paracrine and endocrine factors that promote cell proliferation and survival (development) or unchain apoptosis as part of the atresia process. In several types of cells, sphingosine-1-phospate (S1P) promotes cellular proliferation and survival, whereas ceramide (CER) triggers cell death, and the S1P/CER ratio may determine the fate of the cell. The aim of present study was to quantify S1P and CER concentrations and their ratio in bovine antral follicles of 8 to 17 mm classified as healthy and atretic antral follicles. Follicles were dissected from cow ovaries collected from a local abattoir. The theca cell layer, the granulosa cells and follicular fluid were separated, and 17β-estradiol (E2) and progesterone (P4) concentrations were measured in the follicular fluid by radioimmunoassay. Based on the E2/P4 ratio, the follicles were classified as healthy (2.2±0.3) or atretic (0.2±0.3). In both follicular compartments (granulosa and theca cell layer), sphingolipids were extracted and S1P and CER concentrations were quantified by HPLC (XTerra RP18; 5 µm, 3.0×150 mm column). Results showed that in both follicular compartments, S1P concentrations were higher in healthy antral follicles than in atretic antral follicles (P<0.05). The concentration of CER in the granulosa cells was higher in atretic antral follicles than in healthy antral follicles, but no differences were observed in the theca cell layer. The S1P/CER ratio in both follicular compartments was also higher in healthy antral follicles. Interestingly, in these follicles, there was a 45-fold greater concentration of S1P than CER in the granulosa cells (P<0.05), whereas in the theca cell layer, S1P had only a 14-fold greater concentration than CER when compared with atretic antral follicles. These results suggest that S1P plays a role in follicle health, increasing cellular proliferation and survival. In

  18. Outcome of Jejuno-Ileal Atresia Associated with Intraoperative Finding of Volvulus of Small Bowel

    PubMed Central

    Sinha, Shalini; Sarin, Yogesh Kumar

    2012-01-01

    Aim: To compare the outcome of patients with jeuno-ileal atresia (JIA) associated with the intraoperative finding of volvulus of small bowel (group A) with that of JIA without volvulus (group B). Materials and Methods: It is a retrospective observational study conducted at one of the two units of Pediatric Surgery, in a tertiary care public hospital of India, from January 2001 to December 2010. Hospital records were retrieved and analyzed. During this time period, 65 patients with JIA were operated of which 40 (61.5%) had ileal atresia (IA) and 25 (38.5%) had jejunal atresia (JA). Eleven (16.9%) patients had associated intraoperative finding of volvulus of small bowel (Group A) and were studied and compared with group B- not associated with intraoperative findings of volvulus of small bowel (n=54). The demography, clinical features, operative findings, associated anomalies, anastomotic leakage, and outcome were compared. Results: Group A comprising of 6 boys and 5 girls, had 8 IA and 3 JA; one case each of Type 3b and Type 4 JIA was seen. Associated anomalies included meconium ileus (n=2), Down’s syndrome (n=1) and malrotation (n=1). Anastomotic leak rate was 75% for IA and 66.7% for JA. The mortality was 91% in Group A, 100% for IA and 67% for JA. Group B comprising of 37 boys and 17 girls, had 32 IA and 22 JA; 2 cases of Type 4 and 1 case of Type 3b JIA was seen. Associated anomalies were malrotation (n=2), meconium ileus (n=1), exomphalos (n=1), gastroschisis (n=1) and ileal duplication cyst (n=1). The anastomotic leak rate for JA was 8/21 (38.1%) and IA was 3/28 (10.7%); persistent obstruction was seen in 3/21(14.3%) JA and 1/28 (3.6%) IA patients. In group B, overall mortality rate was 8/22 (36.4%) for JA and 9/32 (28%) for IA. The morbidity and mortality was significantly higher in group A when compared to group B. Conclusions: JIA associated with volvulus (without malrotation) is a sinister entity with a dismal outcome in our experience. PMID:26023396

  19. Sphingosine-1-phosphate and ceramide are associated with health and atresia of bovine ovarian antral follicles.

    PubMed

    Hernández-Coronado, C G; Guzmán, A; Espinosa-Cervantes, R; Romano, M C; Verde-Calvo, J R; Rosales-Torres, A M

    2015-02-01

    The follicle destiny towards ovulation or atresia is multi-factorial in nature and involves outcries, paracrine and endocrine factors that promote cell proliferation and survival (development) or unchain apoptosis as part of the atresia process. In several types of cells, sphingosine-1-phospate (S1P) promotes cellular proliferation and survival, whereas ceramide (CER) triggers cell death, and the S1P/CER ratio may determine the fate of the cell. The aim of present study was to quantify S1P and CER concentrations and their ratio in bovine antral follicles of 8 to 17 mm classified as healthy and atretic antral follicles. Follicles were dissected from cow ovaries collected from a local abattoir. The theca cell layer, the granulosa cells and follicular fluid were separated, and 17β-estradiol (E2) and progesterone (P4) concentrations were measured in the follicular fluid by radioimmunoassay. Based on the E2/P4 ratio, the follicles were classified as healthy (2.2±0.3) or atretic (0.2±0.3). In both follicular compartments (granulosa and theca cell layer), sphingolipids were extracted and S1P and CER concentrations were quantified by HPLC (XTerra RP18; 5 µm, 3.0×150 mm column). Results showed that in both follicular compartments, S1P concentrations were higher in healthy antral follicles than in atretic antral follicles (P<0.05). The concentration of CER in the granulosa cells was higher in atretic antral follicles than in healthy antral follicles, but no differences were observed in the theca cell layer. The S1P/CER ratio in both follicular compartments was also higher in healthy antral follicles. Interestingly, in these follicles, there was a 45-fold greater concentration of S1P than CER in the granulosa cells (P<0.05), whereas in the theca cell layer, S1P had only a 14-fold greater concentration than CER when compared with atretic antral follicles. These results suggest that S1P plays a role in follicle health, increasing cellular proliferation and survival. In

  20. First successful repair of an aortico-to-right ventricular tunnel (ARVT) in d-transposition of the great arteries with aortic valve atresia and ventricular septal defect.

    PubMed

    Wagner, Robert; Vollroth, Marcel; Daehnert, Ingo; Kostelka, Martin

    2015-04-01

    The presented case reports on the first successful complex biventricular repair in a neonate with an aortico-to-right ventricular tunnel and dextrotransposition of the great arteries complicated by aortic atresia.

  1. Peroral endoscopic myotomy for esophageal achalasia

    PubMed Central

    Inoue, Haruhiro; Ikeda, Haruo; Sato, Hiroki; Sato, Chiaki; Hokierti, Chananya

    2014-01-01

    Peroral endoscopic myotomy (POEM) is one of the alternative treatment for achalasia. Due to concept of natural orifice transluminal endoscopic surgery (NOTES), it becomes popular and widely accepted. With the endoluminal technique, submucosal tunnel was created followed by endoscopic myotomy. POEM is not only indicated in classical achalasia but also other abnormal esophageal motility disorders. Moreover, failures of endoscopic treatment or surgical attempted cases are not contraindicated for POEM. The second attempted POEM is also safe and technically feasible. Even though the legend of success of POEM is fruitful, the possible complications are very frightened. Good training and delicate practice will reduce rate of complications. This review provides a summary of current state-of-the-art of POEM, including indication equipments, technique and complications. This perfect procedure may become the treatment of choice of achalasia and some esophageal motility disorders in the near future. PMID:25333007

  2. Spontaneous enterogastric reflux gastritis and esophagitis.

    PubMed Central

    Gowen, G F

    1985-01-01

    Enterogastric reflux gastritis and esophagitis is best known after gastric resections and pyloroplasty but it also occurs spontaneously in the nonoperated patient. Forty-two patients are presented who meet the criteria for the diagnosis: constant burning epigastric pain, worse after meals, unrelieved by antacids and diet; endoscopic demonstration of a gastric bile pool; endoscopic biopsy proof of gastritis and esophagitis; and hypochlorhydria. Patients with mild and moderate stages of the disease can benefit from metoclopramide therapy which improves the gastric emptying mechanism. Of the surgical patients with intractable symptoms, 90% were women, 90% had marked hypochlorhydria, 83% had biliary disease, current or remote, and 50% had anemia. With vagotomy, antrectomy, and Roux-Y anastomosis 45-60 cm downstream, the clinical response has been most encouraging. PMID:3970596

  3. Parenteral nutrition in esophageal cancer patients.

    PubMed Central

    Daly, J M; Massar, E; Giacco, G; Frazier, O H; Mountain, C F; Dudrick, S J; Copeland, E M

    1982-01-01

    A review of operative therapy in 244 patients with esophageal cancer from 1960 to 1980 was done to evaluate the impact of TPN in 72 patients treated from 1973 to 1980 with 43 non-TPN patients treated during the same period and to 129 patients operated upon before 1973. Mean age, sex distribution, site, stage, and treatment of the disease were similar for the two study groups. The TPN group lost less weight during treatment (3 lbs vs. 11 lbs) and had fewer overall complications postoperatively (24% vs. 41%). Significant reductions in major wound, infectious, and postoperative complications were noted in these patients who received at least 5 days of preoperative TPN compared with postoperative TPN or the non-TPN groups (4% vs. 24% and 23%). Malnourished esophageal cancer patients can more safely undergo aggressive operative therapy and radiation treatment when adequate perioperative nutritional support is added to the treatment armamentarium. PMID:6807225

  4. Early esophageal cancer screening in China.

    PubMed

    Gao, Qin-Yan; Fang, Jing-Yuan

    2015-12-01

    In China, the incidence of esophageal cancer (EC) and its related mortality are high. Screening strategies aiming at early diagnosis can improve the prognosis. Researches on detection of early EC, especially in China are reviewed. Compared to esophageal balloon cytology or routine endoscopy, chromoendoscopy with Lugol's staining and biopsy appears to be the gold standard for early EC diagnosis in China today. Narrow-band imaging endoscopy, Confocal Laser endomicroscopy and other novel diagnostic approaches are more and more widely used in developed urban areas, but cost and lack of essential training to the endoscopists have made their use limited in rural areas. No specific biomarkers or serum markers were strongly commended to be used in screening strategies currently, which need to be evaluated in future. Trials on organized screening have been proposed in some regions of china with high disease prevalence. Screening in these areas has been shown to be cost effective. PMID:26651250

  5. Current strategies in chemoradiation for esophageal cancer

    PubMed Central

    Lloyd, Shane

    2014-01-01

    Chemoradiotherapy (CRT) has an important role in the treatment of esophageal cancer in both the inoperable and the pre-operative settings. Pre-operative chemoradiation therapy is generally given to 41.4-50.4 Gy with platinum or paclitaxel based chemotherapy. The most common definitive dose in the U.S. is 50-50.4 Gy. New advances in CRT for esophageal cancer have come from looking for ways to minimize toxicity and maximize efficacy. Recent investigations for minimizing toxicity have focused advanced radiation techniques such as IMRT and proton therapy, have sought to further define normal tissue tolerances, and have examined the use of tighter fields with less elective clinical target volume coverage. Efforts to maximize efficacy have included the use of early positron emission tomography (PET) response directed therapy, molecularly targeted therapies, and the use of tumor markers that predict response. PMID:24982764

  6. Early esophageal cancer screening in China.

    PubMed

    Gao, Qin-Yan; Fang, Jing-Yuan

    2015-12-01

    In China, the incidence of esophageal cancer (EC) and its related mortality are high. Screening strategies aiming at early diagnosis can improve the prognosis. Researches on detection of early EC, especially in China are reviewed. Compared to esophageal balloon cytology or routine endoscopy, chromoendoscopy with Lugol's staining and biopsy appears to be the gold standard for early EC diagnosis in China today. Narrow-band imaging endoscopy, Confocal Laser endomicroscopy and other novel diagnostic approaches are more and more widely used in developed urban areas, but cost and lack of essential training to the endoscopists have made their use limited in rural areas. No specific biomarkers or serum markers were strongly commended to be used in screening strategies currently, which need to be evaluated in future. Trials on organized screening have been proposed in some regions of china with high disease prevalence. Screening in these areas has been shown to be cost effective.

  7. Reversal of lower esophageal sphincter hypotension and esophageal aperistalsis after treatment for hypothyroidism

    SciTech Connect

    Eastwood, G.L.; Braverman, L.E.; White, E.M.; Vander Salm, T.J.

    1982-08-01

    A 65-year-old woman suffered from both chronic gastroesophageal reflux, which was complicated by columnar metaplasia (Barrett's epithelium), and profound hypothyroidism. An esophageal motility tracing showed absence of peristalsis in the lower esophagus and the lower esophageal sphincter (LES) could not be identified. Thyroid replacement therapy, in conjunction with antacid and cimetidine treatment, was associated not only with improvement in the gastroesophageal reflux symptoms, but also with a return of esophageal peristalsis and LES pressure to normal. To support our clinical observations, we rendered four cats hypothyroid with /sup 131/I and documented a fall in LES pressure. We propose that abnormal smooth-muscle function of the esophagus may be another manifestation of the gastrointestinal motility disturbances which are associated with hypothyroidism.

  8. Advances in Clinical Management of Eosinophilic Esophagitis

    PubMed Central

    Dellon, Evan S.; Liacouras, Chris A.

    2014-01-01

    EoE is a chronic immune/antigen-mediated clinicopathologic condition that has become an increasingly important cause of upper gastrointestinal morbidity in adults and children over the past 2 decades. It is diagnosed based on symptoms of esophageal dysfunction, the presence of at least 15 eosinophils/high-power field in esophageal biopsies, and exclusion of competing causes of esophageal eosinophilia, including proton pump inhibitor-responsive esophageal eosinophilia (PPI-REE). We review what we have recently learned about the clinical aspects of EoE, discussing the clinical, endoscopic, and histologic features of EoE in adults and children. We explain the current diagnostic criteria and challenges to diagnosis, including the role of gastroesophageal reflux disease and PPI-REE. It is also important to consider the epidemiology of EoE (current incidence of 1/10,000 new cases per year and prevalence of 0.5-1/1,000 cases per year) and disease progression. We review the main treatment approaches and new treatment options; EoE can be treated with topical corticosteroids such as fluticasone and budesonide, or dietary strategies, such as amino acid-based formulas, allergy test-directed elimination diets, and non-directed empiric elimination diets. Endoscopic dilation has also become an important tool for treatment of fibrostenostic complications of EoE. There are number of unresolved issues in EoE, including phenotypes, optimal treatment endpoints, the role of maintenance therapy, and treatment of refractory EoE. The care of patients with EoE and the study of the disease span many disciplines—EoE is ideally managed by a multidisciplinary team of gastroenterologists, allergists, pathologists, and dieticians. PMID:25109885

  9. [Minimally Invasive Treatment of Esophageal Benign Diseases].

    PubMed

    Inoue, Haruhiro

    2016-07-01

    As a minimally invasive treatment of esophageal achalasia per-oral endoscopic myotomy( POEM) was developed in 2008. More than 1,100 cases of achalasia-related diseases received POEM. Success rate of the procedure was more than 95%(Eckerdt score improvement 3 points and more). No serious( Clavian-Dindo classification III b and more) complication was experienced. These results suggest that POEM becomes a standard minimally invasive treatment for achalasia-related diseases. As an off-shoot of POEM submucosal tumor removal through submucosal tunnel (per-oral endoscopic tumor resection:POET) was developed and safely performed. Best indication of POET is less than 5 cm esophageal leiomyoma. A novel endoscopic treatment of gastroesophageal reflux disease (GERD) was developed. Anti-reflux mucosectomy( ARMS) is nearly circumferential mucosal reduction of gastric cardia mucosa. ARMS is performed in 56 consecutive cases of refractory GERD. No major complications were encountered and excellent clinical results. Best indication of ARMS is a refractory GERD without long sliding hernia. Longest follow-up case is more than 10 years. Minimally invasive treatments for esophageal benign diseases are currently performed by therapeutic endoscopy. PMID:27440038

  10. A Review of Esophageal Chest Pain

    PubMed Central

    Coss-Adame, Enrique

    2015-01-01

    Noncardiac chest pain is a term that encompasses all causes of chest pain after a cardiac source has been excluded. This article focuses on esophageal sources for chest pain. Esophageal chest pain (ECP) is common, affects quality of life, and carries a substantial health care burden. The lack of a systematic approach toward the diagnosis and treatment of ECP has led to significant disability and increased health care costs for this condition. Identifying the underlying cause(s) or mechanism(s) for chest pain is key for its successful management. Common etiologies include gastroesophageal reflux disease, esophageal hypersensitivity, dysmotility, and psychological conditions, including panic disorder and anxiety. However, the pathophysiology of this condition is not yet fully understood. Randomized controlled trials have shown that proton pump inhibitor therapy (either omeprazole, lansoprazole, or rabeprazole) can be effective. Evidence for the use of antidepressants and the adenosine receptor antagonist theophylline is fair. Psychological treatments, notably cognitive behavioral therapy, may be useful in select patients. Surgery is not recommended. There remains a large unmet need for identifying the phenotype and prevalence of pathophysiologic mechanisms of ECP as well as for well-designed multicenter clinical trials of current and novel therapies. PMID:27134590

  11. Eosinophilic esophagitis: From pathophysiology to treatment

    PubMed Central

    D’Alessandro, Alessandra; Esposito, Dario; Pesce, Marcella; Cuomo, Rosario; De Palma, Giovanni Domenico; Sarnelli, Giovanni

    2015-01-01

    Eosinophilic esophagitis (EoE) is a chronic immune disease, characterized by a dense eosinophilic infiltrate in the esophagus, leading to bolus impaction and reflux-like symptoms. Traditionally considered a pediatric disease, the number of adult patients with EoE is continuously increasing, with a relatively higher incidence in western countries. Dysphagia and food impaction represent the main symptoms complained by patients, but gastroesophageal reflux-like symptoms may also be present. Esophageal biopsies are mandatory for the diagnosis of EoE, though clinical manifestations and proton pump inhibitors responsiveness must be taken into consideration. The higher prevalence of EoE in patients suffering from atopic diseases suggests a common background with allergy, however both the etiology and pathophysiology are not completely understood. Elimination diets are considered the first-line therapy in children, but this approach appears less effective in adults patients, who often require steroids; despite medical treatments, EoE is complicated in some cases by esophageal stricture and stenosis, that require additional endoscopic treatments. This review summarizes the evidence on EoE pathophysiology and illustrates the safety and efficacy of the most recent medical and endoscopic treatments. PMID:26600973

  12. Lymphocytic Esophagitis: A Diagnosis of Increasing Frequency

    PubMed Central

    Cohen, Shirley; Saxena, Aditi; Waljee, Akbar; Piraka, Cyrus; Purdy, Julianne; Appelman, Henry; McKenna, Barbara; Elmunzer, B. Joseph; Singal, Amit G.

    2012-01-01

    Background Despite being found with increasing frequency on esophageal biopsies, the clinical significance of lymphocytic esophagitis (LE) remains poorly understood. Goals The primary aim of our study was to characterize the clinical presentation and natural history of LE among adult patients. Study We retrospectively reviewed records for all 81 adult patients at the University of Michigan Medical Center who had a histopathological diagnosis of LE between January 1998 and November 2009. Patient demographics, clinical history, laboratory data, and imaging results from the time of diagnosis were obtained through review of computerized medical records. A telephone survey was conducted to collect natural history data. Results The number of LE diagnoses increased over time, with 81.5% (n=66) of patients being diagnosed in the last three years. The most frequent symptoms at the time of presentation were dysphagia (n=54), chest/abdominal pain (n=36), and heartburn (n=38). The majority (58.6%) of patients reported improvement in their initial gastrointestinal symptoms – most commonly associated with initiation of a proton pump inhibitor. Upon follow-up, most patients reported a good quality of life and satisfaction with their current health status. Conclusions Lymphocytic esophagitis is a new clinical entity with an increasing incidence. LE appears to have a benign natural history, with most patients reporting an improvement in symptoms and satisfaction with their health-related quality of life. Prospective studies are needed to better characterize the natural history and potential treatments for this clinical entity. PMID:22751335

  13. Esophageal Stricture Prevention after Endoscopic Submucosal Dissection

    PubMed Central

    Jain, Deepanshu; Singhal, Shashideep

    2016-01-01

    Advances in diagnostic modalities and improvement in surveillance programs for Barrett esophagus has resulted in an increase in the incidence of superficial esophageal cancers (SECs). SEC, due to their limited metastatic potential, are amenable to non-invasive treatment modalities. Endoscopic ultrasound, endoscopic mucosal resection, and endoscopic submucosal dissection (ESD) are some of the new modalities that gastroenterologists have used over the last decade to diagnose and treat SEC. However, esophageal stricture (ES) is a very common complication and a major cause of morbidity post-ESD. In the past few years, there has been a tremendous effort to reduce the incidence of ES among patients undergoing ESD. Steroids have shown the most consistent results over time with minimal complications although the preferred mode of delivery is debatable, with both systemic and local therapy having pros and cons for specific subgroups of patients. Newer modalities such as esophageal stents, autologous cell sheet transplantation, polyglycolic acid, and tranilast have shown promising results but the depth of experience with these methods is still limited. We have summarized case reports, prospective single center studies, and randomized controlled trials describing the various methods intended to reduce the incidence of ES after ESD. Indications, techniques, outcomes, limitations, and reported complications are discussed. PMID:26949124

  14. Methoxychlor induces atresia of antral follicles in ERalpha-overexpressing mice.

    PubMed

    Tomic, Dragana; Frech, Maria Silvina; Babus, Janice K; Gupta, Rupesh K; Furth, Priscilla A; Koos, Robert D; Flaws, Jodi A

    2006-09-01

    Methoxychlor (MXC) is a pesticide that is known to bind to estrogen receptor alpha (ERalpha) and to induce atresia of antral ovarian follicles. Although studies have shown that MXC is toxic to the ovary, we hypothesize that perturbation to the estrogen-signaling system (i.e., increase or decrease in estrogen sensitivity) might alter ovarian responsiveness to MXC. Thus, we examined whether ERalpha overexpression alters the ability of MXC to increase follicle atresia. To do so, we employed a transgenic mouse model in which ERalpha can be inducibly overexpressed in animal tissues (ERalpha overexpressors). We dosed female controls and ERalpha overexpressors with sesame oil (vehicle control) or MXC (32 and 64 mg/kg/day) for 20 days. After dosing, the ovaries were collected for histological evaluation of follicle numbers and follicle atresia, while blood was collected for measurements of hormones. Estrous cycles were determined in all animals to ensure that all were terminated during estrus. Although there were no significant effects of MXC on the numbers of primordial, primary, and preantral follicles in both controls and ERalpha overexpressors, there was an effect on antral follicles. Specifically, our data indicate that 32 and 64 mg/kg MXC increased the percentage of atretic follicles compared to vehicle in both control and ERalpha overexpressor groups. Moreover, there was a clear trend toward greater sensitivity to 64 mg/kg MXC in ERalpha-overexpressing mice compared to control animals. Specifically, at the 64-mg/kg MXC dose, ERalpha-overexpressing mice had a significantly higher percentage of atretic follicles compared to control animals (controls = 21.5 +/- 3%, n = 5; ERalpha overexpressors = 37 +/- 23%, n = 9, p < or = 0.05 vs. controls). After 20 days of dosing, there were no differences in estradiol levels between controls and ERalpha-overexpressing mice in all treatment groups. Follicle-stimulating hormone (FSH) levels were similar in sesame oil-treated control

  15. Use of Corticosteroids After Hepatoportoenterostomy for Bile Drainage in Infants With Biliary Atresia

    PubMed Central

    Bezerra, Jorge A.; Spino, Cathie; Magee, John C.; Shneider, Benjamin L.; Rosenthal, Philip; Wang, Kasper S.; Erlichman, Jessi; Haber, Barbara; Hertel, Paula M.; Karpen, Saul J.; Kerkar, Nanda; Loomes, Kathleen M.; Molleston, Jean P.; Murray, Karen F.; Romero, Rene; Schwarz, Kathleen B.; Shepherd, Ross; Suchy, Frederick J.; Turmelle, Yumirle P.; Whitington, Peter F.; Moore, Jeffrey; Sherker, Averell H.; Robuck, Patricia R.; Sokol, Ronald J.

    2015-01-01

    IMPORTANCE Biliary atresia is the most common cause of end-stage liver disease in children. Controversy exists as to whether use of steroids after hepatoportoenterostomy improves clinical outcome. OBJECTIVE To determine whether the addition of high-dose corticosteroids after hepatoportoenterostomy is superior to surgery alone in improving biliary drainage and survival with the native liver. DESIGN, SETTING, AND PATIENTS The multicenter, double-blind Steroids in Biliary Atresia Randomized Trial (START) was conducted in 140 infants (mean age, 2.3 months) between September 2005 and February 2011 in the United States; follow-up ended in January 2013. INTERVENTIONS Participants were randomized to receive intravenous methylprednisolone (4 mg/kg/d for 2 weeks) and oral prednisolone (2 mg/kg/d for 2 weeks) followed by a tapering protocol for 9 weeks (n = 70) or placebo (n = 70) initiated within 72 hours of hepatoportoenterostomy. MAIN OUTCOMES AND MEASURES The primary end point (powered to detect a 25% absolute treatment difference) was the percentage of participants with a serum total bilirubin level of less than 1.5 mg/dL with his/her native liver at 6 months posthepatoportoenterostomy. Secondary outcomes included survival with native liver at 24 months of age and serious adverse events. RESULTS The proportion of participants with improved bile drainage was not statistically significantly improved by steroids at 6 months posthepatoportoenterostomy (58.6% [41/70] of steroids group vs 48.6% [34/70] of placebo group; adjusted relative risk, 1.14 [95% CI, 0.83 to 1.57]; P = .43). The adjusted absolute risk difference was 8.7% (95% CI, −10.4% to 27.7%). Transplant-free survival was 58.7% in the steroids group vs 59.4% in the placebo group (adjusted hazard ratio, 1.0 [95% CI, 0.6 to 1.8]; P = .99) at 24 months of age. The percentage of participants with serious adverse events was 81.4% [57/70] of the steroids group and 80.0% [56/70] of the placebo group (P > .99); however

  16. Results of surgery in 88 consecutive cases of extrahepatic biliary atresia.

    PubMed Central

    Howard, E R; Driver, M; McClement, J; Mowat, A P

    1982-01-01

    Of 88 cases of extrahepatic biliary atresia, satisfactory bile flow has been established in 46% of the patients who have undergone portoenterostomies and in 25% of patients with hepaticojejunostomies. Histological analysis of the extrahepatic biliary tissue has not shown a consistent correlation with outcome of operation, except that the patients with one or two large residual ducts lined with columnar epithelium have a better chance of developing bile flow. Cholangitis developed in 43% of the cases, and co-trimoxazole was not shown to have any beneficial effect in a small prospective trial in 18 patients. Severe haemorrhage from oesophageal varices has occurred in 4 jaundice-free survivors. Seventeen patients are now over 3 years of age and thriving but many show persistent elevation of liver enzymes. PMID:7086788

  17. Incidental Finding of Inferior Vena Cava Atresia Presenting with Deep Venous Thrombosis following Physical Exertion

    PubMed Central

    Koppisetty, Shalini; Smith, Alton G.; Dhillon, Ravneet K.

    2015-01-01

    Inferior vena cava atresia (IVCA) is a rare but well described vascular anomaly. It is a rare risk factor for deep venous thrombosis (DVT), found in approximately 5% of cases of unprovoked lower extremity (LE) DVT in patients <30 years of age. Affected population is in the early thirties, predominantly male, often with a history of major physical exertion and presents with extensive or bilateral DVTs. Patients with IVC anomalies usually develop compensatory circulation through the collateral veins with enlarged azygous/hemizygous veins. Despite the compensatory circulation, the venous drainage of the lower limbs is often insufficient leading to venous stasis and thrombosis. We describe a case of extensive and bilateral deep venous thrombosis following physical exertion in a thirty-six-year-old male patient with incidental finding of IVCA on imaging. PMID:26640723

  18. Horizontal ventricular septum with dextroversion: hearts with and without aortic atresia.

    PubMed

    Thilenius, O G; Bharati, S; Lev, M; Karp, R B; Arcilla, R A

    1987-01-01

    Two hearts with horizontal ventricular septum, dextroversion (situs solitus), ventricular septal defects, and malaligned great vessels are reported. One of the hearts had aortic atresia and the infant died; the other patient had a Fontan-type physiologic correction. Reviewing the literature, the following conclusions are drawn: (a) Hearts with horizontal ventricular septum and those with criss-cross atrioventricular connections may be the result of different degrees of rotation of the ventricular muscle mass. This rotation is not likely to be postseptational but preseptational. (b) Only those hearts with a complete 180 degrees rotation should be called criss-cross hearts. (c) Partial rotation results in a horizontal septum such that the right ventricle is invariably superior, regardless of atrioventricular concordance or discordance, situs solitus or inversus, or dextroversion. (d) Physiologic surgical correction is often possible but has to be tailored to the details of each heart.

  19. External auditory canal atresia of probable congenital origin in a dog.

    PubMed

    Schmidt, K; Piaia, T; Bertolini, G; De Lorenzi, D

    2007-04-01

    A nine-month-old Labrador retriever was referred to the Clinica Veterinaria Privata San Marco because of frequent headshaking and downward turning of the right ear. Clinical examination revealed that there was no external acoustic meatus in the right ear. Computed tomography confirmed that the vertical part of the right auditory canal ended blindly, providing a diagnosis of external auditory canal atresia. Cytological examination and culture of fluid from the canal and the bulla revealed only aseptic cerumen; for this reason, it was assumed that the dog was probably affected by a congenital developmental deformity of the external auditory canal. Reconstructive surgery was performed using a "pull-through" technique. Four months after surgery the cosmetic and functional results were satisfactory.

  20. Two Case Reports of FGF23-Induced Hypophosphatemia in Childhood Biliary Atresia.

    PubMed

    Wasserman, Halley; Ikomi, Chijioke; Hafberg, Einar T; Miethke, Alexander G; Bove, Kevin E; Backeljauw, Philippe F

    2016-08-01

    Cholestatic liver disease has long been associated with childhood rickets, secondary to impaired absorption of fat-soluble vitamin D. Elevated serum levels of fibroblast growth factor 23 (FGF23), secondary to genetic defects or tumor-induced osteomalacia, causes hypophosphatemic rickets in childhood. We present 2 infants with end-stage liver disease due to biliary atresia (BA) who developed hypophosphatemia with renal phosphate wasting. Serum FGF23 levels were elevated more than 8 times the upper limit of normal, and the older infant showed radiographic evidence of rickets. Both infants required large supplements of phosphate in addition to calcitriol. Following liver transplantation, FGF23 normalized in both patients and phosphate and calcitriol supplementation were discontinued. Immunohistochemistry revealed ectopic overexpression of FGF23 by hepatocytes in the BA liver. These observations highlight a unique cause of hypophosphatemic rickets in childhood and suggest the need for further investigation into the relationship between BA and other cholestatic disorders, and bone metabolism. PMID:27462066

  1. Upper Gastrointestinal Symptoms Predictive of Candida Esophagitis and Erosive Esophagitis in HIV and Non-HIV Patients

    PubMed Central

    Takahashi, Yuta; Nagata, Naoyoshi; Shimbo, Takuro; Nishijima, Takeshi; Watanabe, Koji; Aoki, Tomonori; Sekine, Katsunori; Okubo, Hidetaka; Watanabe, Kazuhiro; Sakurai, Toshiyuki; Yokoi, Chizu; Mimori, Akio; Oka, Shinichi; Uemura, Naomi; Akiyama, Junichi

    2015-01-01

    Abstract Upper gastrointestinal (GI) symptoms are common in both HIV and non-HIV-infected patients, but the difference of GI symptom severity between 2 groups remains unknown. Candida esophagitis and erosive esophagitis, 2 major types of esophagitis, are seen in both HIV and non-HIV-infected patients, but differences in GI symptoms that are predictive of esophagitis between 2 groups remain unknown. We aimed to determine whether GI symptoms differ between HIV-infected and non-HIV-infected patients, and identify specific symptoms of candida esophagitis and erosive esophagitis between 2 groups. We prospectively enrolled 6011 patients (HIV, 430; non-HIV, 5581) who underwent endoscopy and completed questionnaires. Nine upper GI symptoms (epigastric pain, heartburn, acid regurgitation, hunger cramps, nausea, early satiety, belching, dysphagia, and odynophagia) were evaluated using a 7-point Likert scale. Associations between esophagitis and symptoms were analyzed by the multivariate logistic regression model adjusted for age, sex, and proton pump inhibitors. Endoscopy revealed GI-organic diseases in 33.4% (2010/6.011) of patients. The prevalence of candida esophagitis and erosive esophagitis was 11.2% and 12.1% in HIV-infected patients, respectively, whereas it was 2.9% and 10.7 % in non-HIV-infected patients, respectively. After excluding GI-organic diseases, HIV-infected patients had significantly (P < 0.05) higher symptom scores for heartburn, hunger cramps, nausea, early satiety, belching, dysphagia, and odynophagia than non-HIV-infected patients. In HIV-infected patients, any symptom was not significantly associated with CD4 cell count. In multivariate analysis, none of the 9 GI symptoms were associated with candida esophagitis in HIV-infected patients, whereas dysphagia and odynophagia were independently (P < 0.05) associated with candida esophagitis in non-HIV-infected patients. However, heartburn and acid regurgitation were independently (P < 0

  2. Biliary Atresia

    MedlinePlus

    ... Griffin Rodgers, Director of the NIDDK Clinical Trials Current research studies and how you can volunteer Community Outreach and Health Fairs Science-based information and tips for planning an outreach effort or community event For Health Care Professionals Patient and provider resources ...

  3. Pulmonary Atresia

    MedlinePlus

    ... Defects_UCM_307037_Article.jsp Single Ventricle Defects Texas Adult Congenital Heart Center (TACH) www.bcm.edu/ ... comments. Terms of Use and Privacy Policy © Copyright Texas Heart Institute All rights reserved.

  4. [Morphological changes in esophageal mucosa in children with overweight].

    PubMed

    Dubrovskaia, M I; Tertychnyĭ, A S; Mukhina, Iu G; Volodina, I I; Mamchenko, S I

    2010-01-01

    In present work we studied the morphological features of the esophageal mucosa in 63 children with endoscopic diagnosis of the distal esophagitis having overweight and normal weight of a body. The biopsies were taken at level of 3 cm above a Z-line and at level of 1 cm above a Z-line. Dystrophic and dysregenerative changes were revealed at the majority of children and half of children had inflammatory changes of the esophageal mucosa regardless of weight of a body. These changes are more pronounced at level of 1 cm above a Z-line, their occurrence decreases with a distance from low esophageal sphincter. We used the pathology score system for assess the esophageal biopsies. According our scale we obtained following results: at level of 1 cm above Z-lines at 95% of children had the normal, minimum or mild features of esophagitis regardless of weight of a body. Morphological evidence of a reflux esophagitis was diagnosed statistically more often at level of 1 cm above Z lines in comparison with level of 3 cm above Z-lines (p < 0.01) as among children with overweight of the body (78 and 43% accordingly), and among children with normal weight of the body (78 and 35% accordingly). The obtained data will be allowed to avoid hyperdiagnostics of esophageal lesions in children. PMID:20405708

  5. Prevention of esophageal strictures after endoscopic submucosal dissection

    PubMed Central

    Kobayashi, Shinichiro; Kanai, Nobuo; Ohki, Takeshi; Takagi, Ryo; Yamaguchi, Naoyuki; Isomoto, Hajime; Kasai, Yoshiyuki; Hosoi, Takahiro; Nakao, Kazuhiko; Eguchi, Susumu; Yamamoto, Masakazu; Yamato, Masayuki; Okano, Teruo

    2014-01-01

    Endoscopic mucosal resection (EMR) and endoscopic submucosal dissection (ESD) have recently been accepted as less invasive methods for treating patients with early esophageal cancers such as squamous cell carcinoma and dysplasia of Barrett’s esophagus. However, the large defects in the esophageal mucosa often cause severe esophageal strictures, which dramatically reduce the patient’s quality of life. Although preventive endoscopic balloon dilatation can reduce dysphagia and the frequency of dilatation, other approaches are necessary to prevent esophageal strictures after ESD. This review describes several strategies for preventing esophageal strictures after ESD, with a particular focus on anti-inflammatory and tissue engineering approaches. The local injection of triamcinolone acetonide and other systemic steroid therapies are frequently used to prevent esophageal strictures after ESD. Tissue engineering approaches for preventing esophageal strictures have recently been applied in basic research studies. Scaffolds with temporary stents have been applied in five cases, and this technique has been shown to be safe and is anticipated to prevent esophageal strictures. Fabricated autologous oral mucosal epithelial cell sheets to cover the defective mucosa similarly to how commercially available skin products fabricated from epidermal cells are used for skin defects or in cases of intractable ulcers. Fabricated autologous oral-mucosal-epithelial cell sheets have already been shown to be safe. PMID:25386058

  6. A Rare Disease of the Digestive Tract: Esophageal Melanosis

    PubMed Central

    Destek, Sebahattin; Gul, Vahit Onur; Ahioglu, Serkan; Erbil, Yesim

    2016-01-01

    Esophageal melanosis which is characterized by melanocytic proliferation in the squamous epithelium of the esophagus and melanin accumulatin of esophageal mucosa (EM) is a rare disease of the digestive system. Although esophageal melanosis is considered to be a benign disease, its etiology is not cleared and has been reported to be the precursor lesion of esophageal primary melanomas. In this report, we aimed to note esophageal melanosis in a 55-year-old female case who applied to our clinic with difficulty in swallowing, burning behind the breastbone in the stomach, heartburn, indigestion, and pain in the upper abdomen after endoscopic and pathologic evaluation. Complaints dropped with anti-acid therapy and case was followed by intermittent endoscopic procedures because of precursor melanocytic lesions. PMID:27785326

  7. Bevacizumab and Combination Chemotherapy Before Surgery in Treating Patients With Locally Advanced Esophageal or Stomach Cancer

    ClinicalTrials.gov

    2016-03-01

    Adenocarcinoma of the Esophagus; Adenocarcinoma of the Gastroesophageal Junction; Diffuse Adenocarcinoma of the Stomach; Intestinal Adenocarcinoma of the Stomach; Mixed Adenocarcinoma of the Stomach; Squamous Cell Carcinoma of the Esophagus; Stage IA Esophageal Cancer; Stage IA Gastric Cancer; Stage IB Esophageal Cancer; Stage IB Gastric Cancer; Stage IIA Esophageal Cancer; Stage IIA Gastric Cancer; Stage IIB Esophageal Cancer; Stage IIB Gastric Cancer; Stage IIIA Esophageal Cancer; Stage IIIA Gastric Cancer; Stage IIIB Esophageal Cancer; Stage IIIB Gastric Cancer; Stage IIIC Esophageal Cancer; Stage IIIC Gastric Cancer

  8. Emerging techniques and efficacy of endoscopic esophageal reconstruction and lumen restoration for complete esophageal obstruction

    PubMed Central

    Perbtani, Yaseen; Suarez, Alejandro L.; Wagh, Mihir S.

    2016-01-01

    Background and study aims: Complete esophageal obstruction (CEO) is a rare occurrence characterized by progressive esophageal stricture, which eventually causes lumen obliteration. With recent advances in flexible endoscopy, various innovative techniques exist for restoring luminal continuity. The primary aim of this study was to assess the efficacy and safety of patients undergoing combined antegrade-retrograde endoscopic dilation for CEO at our institution. The secondary aim was to review and highlight emerging techniques, outcomes, and adverse events after endoscopic treatment of CEO. Patients and methods: Our electronic endoscopy database was retrospectively reviewed to identify patients who underwent combined antegrade and retrograde endoscopy for CEO. Patient and procedural data collected included gender, age, technical success, pre- and post-dysphagia scores, and adverse events. Results: Six patients (67 % male, mean age 71.6 years [range 63 – 80]) underwent technically successful esophageal reconstruction with combined antegrade-retrograde endoscopy. All patients noted improvement in dysphagia with mean pre-procedure dysphagia score of 4 reduced to 1.33 (range 0 – 3) post-procedure. There were no adverse events and mean follow-up time was 17.3 months (range 3 – 48). Conclusions: Combined antegrade and retrograde endoscopic therapy for CEO is feasible and safe. We present our experience with endoscopic management of complete esophageal obstruction, and highlight emerging techniques, outcomes and adverse events related to this minimally invasive modality. PMID:26878039

  9. Patterns of esophageal inhibition during swallowing, pharyngeal stimulation, and transient LES relaxation. Lower esophageal sphincter.

    PubMed

    Pouderoux, Philippe; Verdier, Eric; Kahrilas, Peter J

    2003-02-01

    Lower esophageal sphincter (LES) relaxation and esophageal body inhibition co-occur during esophageal peristalsis but not necessarily during pharyngeal stimulation or transient LES relaxation (tLESR). This study examined these relationships and the impact on reflux. Nine young volunteers were studied. An artificial high-pressure zone (HPZ) was established, and pH was recorded 8 and 5 cm proximal to the LES. Pharyngeal stimulation was by water injection and gastric distension with liquid or gas. Peristalsis, pharyngeal stimulation, and spontaneous events were recorded. Swallowing relaxed the LES in 100% of trials (the HPZ in 80%) and caused no reflux. Pharyngeal stimulation relaxed the LES in two-thirds of trials, had no effect on the HPZ, and caused no reflux. Gastric distension was associated with 117 tLESRs, 48% with acid reflux, and 32% with gas reflux; there was no effect on the HPZ. We conclude that LES relaxation is a necessary but not sufficient condition for reflux. LES relaxation and esophageal body inhibition are independent events that may be concurrent (swallowing) or dissociated (tLESR).

  10. Generation and Characterization of an Immortalized Human Esophageal Myofibroblast Line.

    PubMed

    Niu, Chao; Chauhan, Uday; Gargus, Matthew; Shaker, Anisa

    2016-01-01

    Stromal cells with a myofibroblast phenotype present in the normal human esophagus are increased in individuals with gastro-esophageal reflux disease (GERD). We have previously demonstrated that myofibroblasts stimulated with acid and TLR4 agonists increase IL-6 and IL-8 secretion using primary cultures of myofibroblasts established from normal human esophagus. While primary cultures have the advantage of reflecting the in vivo environment, a short life span and unavoidable heterogeneity limits the usefulness of this model in larger scale in vitro cellular signaling studies. The major aim of this paper therefore was to generate a human esophageal myofibroblast line with an extended lifespan. In the work presented here we have generated and characterized an immortalized human esophageal myofibroblast line by transfection with a commercially available GFP-hTERT lentivirus. Immortalized human esophageal myofibroblasts demonstrate phenotypic, genotypic and functional similarity to primary cultures of esophageal myofibroblasts we have previously described. We found that immortalized esophageal myofibroblasts retain myofibroblast spindle-shaped morphology at low and high confluence beyond passage 80, and express α-SMA, vimentin, and CD90 myofibroblast markers. Immortalized human esophageal myofibroblasts also express the putative acid receptor TRPV1 and TLR4 and retain the functional capacity to respond to stimuli encountered in GERD with secretion of IL-6. Finally, immortalized human esophageal myofibroblasts also support the stratified growth of squamous esophageal epithelial cells in 3D organotypic cultures. This newly characterized immortalized human esophageal myofibroblast cell line can be used in future cellular signaling and co-culture studies. PMID:27055018

  11. Eosinophilic Esophagitis: Epithelial Mesenchymal Transition Contributes to Esophageal Remodeling and Reverses with Treatment

    PubMed Central

    Kagalwalla, Amir F.; Akhtar, Noorain; Woodruff, Samantha A.; Rea, Bryan A.; Masterson, Joanne C.; Mukkada, Vincent; Parashette, Kalyan R.; Du, Jian; Fillon, Sophie; Protheroe, Cheryl A.; Lee, James J.; Amsden, Katie; Melin-Aldana, Hector; Capocelli, Kelley E.; Furuta, Glenn T.; Ackerman, Steven J.

    2012-01-01

    Background Mechanisms underlying esophageal remodeling with subepithelial fibrosis in eosinophilic esophagitis (EoE) have not been delineated. Objectives To explore a role for Epithelial Mesenchymal Transition (EMT) in EoE, and whether EMT resolves with treatment. Methods Esophageal biopsies from 60 children were immunostained for epithelial (cytokeratin) and mesenchymal (vimentin) EMT biomarkers, and EMT quantified. Subjects studied had EoE (n=17), EoE-indeterminate (n=15), GERD (n=7) or normal esophagus (n=21). EMT was analyzed for relationships to diagnosis, eosinophils, and indices of subepithelial fibrosis, eosinophil peroxidase (EPX) and TGF-β immunostaining. EMT was assessed in pre- and post-treatment biopsies from 18 EoE subjects treated with elemental diet, six-food elimination diet, or topical corticosteroids (n=6/group). Results TGF-β1 treatment of esophageal epithelial cells in vitro for 24hrs induced upregulation of mesenchymal genes characteristic of EMT including N-cadherin (3.3-fold), vimentin (2.1-fold) and fibronectin (7.5-fold). EMT in esophageal biopsies was associated with EoE (or indeterminate EoE), but not GERD or normal esophagus, and was correlated to eosinophils (r=0.691), EPX (r=0.738) and TGF-β (r=0.520) immunostaining, and fibrosis (r=0.644) indices. EMT resolved with EoE treatments that induced clinicopathologic remission with reduced eosinophils. EMT decreased significantly post-treatment by 74.1% overall in the 18 treated EoE subjects; pre- vs. post-treatment EMT scores–3.17±0.82 vs. 0.82±0.39 (p<0.001), with similar decreases within treatment groups. Pre-/post-treatment EMT was strongly correlated with eosinophils for combined (r=0.804, p< 0.001) and individual treatment groups. Conclusions EMT likely contributes to subepithelial fibrosis in EoE, resolves with treatments that decrease esophageal inflammation, and its resolution correlates with decreased numbers of esophageal eosinophils. PMID:22465212

  12. Banding of Nonrestrictive Large Hypertensive Aortopulmonary Collaterals in Patients With Ventricular Septal Defect and Pulmonary Atresia Presenting in Suboptimal Condition.

    PubMed

    Dutta Baruah, Sudip; Singh, Vishal Kamalaprasad; Marwah, Ashutosh; Sharma, Rajesh

    2016-09-01

    Infants with pulmonary atresia and nonrestrictive ventricular septal defect with large hypertensive aortopulmonary collaterals demand early surgical intervention. This presentation in the extremely low-weight child or in the moribund septic child may preclude single-stage repair even if anatomically suited. We propose that such infants may be temporized by means of banding of individual aortopulmonary collaterals as a means of bridging to a second-stage complete repair. Two such cases are presented. PMID:27587500

  13. Di(2-ethylhexyl) phthalate inhibits antral follicle growth, induces atresia, and inhibits steroid hormone production in cultured mouse antral follicles

    SciTech Connect

    Hannon, Patrick R. Brannick, Katherine E. Wang, Wei Gupta, Rupesh K. Flaws, Jodi A.

    2015-04-01

    Di(2-ethylhexyl) phthalate (DEHP) is a ubiquitous environmental toxicant found in consumer products that causes ovarian toxicity. Antral follicles are the functional ovarian units and must undergo growth, survival from atresia, and proper regulation of steroidogenesis to ovulate and produce hormones. Previous studies have determined that DEHP inhibits antral follicle growth and decreases estradiol levels in vitro; however, the mechanism by which DEHP elicits these effects is unknown. The present study tested the hypothesis that DEHP directly alters regulators of the cell cycle, apoptosis, and steroidogenesis to inhibit antral follicle functionality. Antral follicles from adult CD-1 mice were cultured with vehicle control or DEHP (1–100 μg/ml) for 24–96 h to establish the temporal effects of DEHP on the follicle. Following 24–96 h of culture, antral follicles were subjected to gene expression analysis, and media were subjected to measurements of hormone levels. DEHP increased the mRNA levels of cyclin D2, cyclin dependent kinase 4, cyclin E1, cyclin A2, and cyclin B1 and decreased the levels of cyclin-dependent kinase inhibitor 1A prior to growth inhibition. Additionally, DEHP increased the mRNA levels of BCL2-associated agonist of cell death, BCL2-associated X protein, BCL2-related ovarian killer protein, B-cell leukemia/lymphoma 2, and Bcl2-like 10, leading to an increase in atresia. Further, DEHP decreased the levels of progesterone, androstenedione, and testosterone prior to the decrease in estradiol levels, with decreased mRNA levels of side-chain cleavage, 17α-hydroxylase-17,20-desmolase, 17β-hydroxysteroid dehydrogenase, and aromatase. Collectively, DEHP directly alters antral follicle functionality by inhibiting growth, inducing atresia, and inhibiting steroidogenesis. - Highlights: • DEHP inhibits antral follicle growth by dysregulating cell cycle regulators. • DEHP induces antral follicle atresia by dysregulating apoptosis regulators. • DEHP

  14. Congenital diaphragmatic hernia associated with oesophageal atresia and tracheo-oesophageal fistula in a low birth weight infant

    PubMed Central

    Abdul Haium, Abdul Alim; Sim, Siam Wee; Ong, Lin Yin; Rajadurai, Victor Samuel

    2013-01-01

    The cooccurrence of congenital diaphragmatic hernia and oesophageal atresia with distal tracheo-oesophageal fistula is very rare and carries high mortality. Very few anecdotal case reports and one case series have been reported in the literature. We report a case of a late preterm, low birth weight infant with this rare association who was successfully managed by staged surgical approach and had good outcome. PMID:23964045

  15. Reverse Szabo technique for stenting a single major aorto-pulmonary collateral vessel in pulmonary atresia with ventricular septal defect

    PubMed Central

    Breinholt, John P.; Rao, Sri O.; Buchneva, Olga V.

    2016-01-01

    Management of pulmonary atresia with ventricular septal defect (PA-VSD) in the neonatal period presents numerous challenges. Endovascular stenting of the ductus arteriosus or of a collateral vessel in ductal-dependent pulmonary circulation as an alternative to the Blalock-Taussig (BT) shunt has become increasingly popular in the last decades. The utilization of the reverse Szabo (anchor-wire) technique for single collateral vessel stenting in a case of PA-VSD is described.

  16. Efficacy of Lower-Extremity Venous Thrombolysis in the Setting of Congenital Absence or Atresia of the Inferior Vena Cava

    SciTech Connect

    Ganguli, Suvranu Kalva, Sanjeeva; Oklu, Rahmi; Walker, T. Gregory; Datta, Neil; Grabowski, Eric F.; Wicky, Stephan

    2012-10-15

    Purpose: A rare but described risk factor for deep venous thrombosis (DVT), predominately in the young, is congenital agenesis or atresia of the inferior vena cava (IVC). The optimal management for DVT in this subset of patients is unknown. We evaluated the efficacy of pharmacomechanical catheter-directed thrombolysis (PCDT) followed by systemic anticoagulation in the treatment of acute lower-extremity DVT in the setting of congenital IVC agenesis or atresia. Materials and Methods: Between November of 2005 and May of 2010, six patients (three women [average age 21 years]) were referred to our department with acute lower-extremity DVT and subsequently found to have IVC agenesis or atresia on magnetic resonance imaging. A standardized technique for PCDT (the Angiojet Rheolytic Thrombectomy System followed by the EKOS Microsonic Accelerated Thrombolysis System) was used for all subjects. Successful thrombolysis was followed by systemic heparinization with transition to Coumadin or low molecular-weight heparin and compression stockings. Subjects were followed-up at 1, 3, and then every 6 months after the procedure with clinical assessment and bilateral lower-extremity venous ultrasound. Results: All PCDT procedures were technically successful. No venous stenting or angioplasty was performed. The average thrombolysis time was 28.6 h (range 12-72). Two patients experienced heparin-induced thrombocytopenia, and one patient developed a self-limited knee hemarthrosis, No patients were lost to follow-up. The average length of follow-up was 25.8 {+-} 20.2 months (range 3.8-54.8). No incidence of recurrent DVT was identified. There were no manifestations of postthrombotic syndrome. Conclusions: PCDT followed by systemic anticoagulation and the use of compression stockings appears to be safe and effective in relatively long-term follow-up treatment of patients who present with acute DVT and IVC agenesis or atresia.

  17. Esophageal transection with automatic stapler device for bleeding esophageal varices. Case report and review of the literature.

    PubMed

    Bayer, I; Kyzer, S; Chaimoff, C

    1989-01-01

    The EEA stapler offers a relative easy technique for the control of severe bleeding from esophageal varices. Two cases are reported in which this technique in combination with proximal gastric devascularization and splenectomy was found to be superior to the performance of portosystemic shunt in emergency cases. The results of portocaval shunts created during severe bleeding from esophageal varices are not satisfactory because of the high mortality rate varying between 36% and 47% (1, 2) and the development of postoperative encephalopathy. For these reasons the tendency today, in cases of severe bleeding from esophageal varices where conservative treatment was unsuccessful, is that the surgeon should attack the bleeding area itself (3). Support for this opinion may be found in the publications of Sugiura and Futagawa (4, 5) and of Koyama et al. (6) about their long-term good results obtained after esophageal transection for bleeding esophageal varices. PMID:2785322

  18. Genomewide Scan for Anal Atresia in Swine Identifies Linkage and Association With a Chromosome Region on Sus scrofa Chromosome 1

    PubMed Central

    Wiedemann, Sabine; Fries, Ruedi; Thaller, Georg

    2005-01-01

    Anal atresia is a rare and severe disorder in swine occurring with an incidence of 0.1–1.0%. A whole-genome scan based on affected half-sibs was performed to identify susceptibility loci for anal atresia. The analysis included 27 families with a total of 95 animals and 65 affected piglets among them. Animals were genotyped for 126 microsatellite markers distributed across the 18 autosomal porcine chromosomes and the X chromosome, covering an estimated 2080 cM. Single-point and multipoint nonparametric linkage scores were calculated using the computer package ALLEGRO 1.0. Significant linkage results were obtained for chromosomes 1, 3, and 12. Markers on these chromosomes and additionally on chromosomes for which candidate genes have been postulated in previous studies were subjected to the transmission disequilibrium test (TDT). The test statistic exceeded the genomewide significance level for adjacent markers SW1621 (P = 7 × 10−7) and SW1902 (P = 3 × 10−3) on chromosome 1, supporting the results of the linkage analysis. A specific haplotype associated with anal atresia that could prove useful for selection against the disorder was revealed. Suggestive linkage and association were also found for markers S0081 on chromosome 9 and SW957 on chromosome 12. PMID:16020797

  19. Etiology and Prevention of Esophageal Cancer

    PubMed Central

    Yang, Chung S.; Chen, Xiaoxin; Tu, Shuiping

    2016-01-01

    Background Esophageal cancer (EC) occurs commonly, especially in Asia, and is the sixth leading cause of cancer deaths worldwide. Recently, great progress has been made in research on the etiology and prevention of EC. Summary The major risk factors for esophageal squamous cell carcinoma (ESCC) are tobacco smoking and alcohol drinking, which act synergistically. Dietary parameters, including dietary carcinogens and insufficiency of micronutrients, could also be important risk factors in certain areas. A common etiological factor for both EC and some other cancers are low levels of intake of fruits and vegetables. With improvements in diet and drinking water in developing countries, the incidence of ESCC decreased. However, in economically well-developed countries, the incidence of esophageal adenocarcinoma (EAC) has markedly increased in the past 40 years. The major etiological factor for EAC is gastroesophageal reflux, which is also an etiological factor for gastric cardia adenocarcinoma (GCA). In certain areas of China, the occurrence of GCA is closely related to ESCC. Susceptibility genes for EC are starting to be discovered, and this may help to identify high-risk groups that have more need for preventive measures. Mitigation of the risk factors, early detection and treatment of precancerous lesions are effective approaches for prevention. Smoking cessation, avoidance of excessive alcohol, meat and caloric consumption, increasing physical activity and frequent consumption of vegetables and fruits are prudent lifestyle modifications for the prevention of EC as well as other diseases. Key Message The etiology of EC includes tobacco smoking, alcohol drinking, low levels of intake of fruits and vegetables as well as gastroesophageal reflux and susceptibility genes. Practical Implications A healthy lifestyle including smoking cessation, increasing physical activity, consumption of vegetables as well as reduction of alcohol intake and caloric consumption are major

  20. Esophageal stricture in a cougar (Puma concolor).

    PubMed

    Desmarchelier, Marion; Lair, Stéphane; Defarges, Alice; Lécuyer, Manon; Langlois, Isabelle

    2009-06-01

    A 7-mo-old female cougar (Puma concolor) was presented with a 2-wk history of anorexia and a 1-wk history of regurgitation. Barium contrast esophagogram and gastroesophagoscopy revealed the presence of a segmental intraluminal esophageal stricture in the middle third of the esophagus. The stricture was potentially secondary to a previous anesthetic episode. Three endoscopic balloon dilations allowed increasing the luminal diameter to a size that enabled the cougar to eat food softened with water without any signs of discomfort or regurgitation. Two months after being discharged, the cougar was doing well, had gained weight and was eating horsemeat softened with water.

  1. Misdiagnosed Chest Pain: Spontaneous Esophageal Rupture

    PubMed Central

    Inci, Sinan; Gundogdu, Fuat; Gungor, Hasan; Arslan, Sakir; Turkyilmaz, Atila; Eroglu, Atila

    2013-01-01

    Chest pain is one of themost common complaints expressed by patients presenting to the emergency department, and any initial evaluation should always consider life-threatening causes. Esophageal rupture is a serious condition with a highmortality rate. If diagnosed, successful therapy depends on the size of the rupture and the time elapsed between rupture and diagnosis.We report on a 41-year-old woman who presented to the emergency department complaining of left-sided chest pain for two hours. PMID:27122690

  2. Endoscopic assessment and management of early esophageal adenocarcinoma.

    PubMed

    Hammoud, Ghassan M; Hammad, Hazem; Ibdah, Jamal A

    2014-08-15

    Esophageal carcinoma affects more than 450000 people worldwide and the incidence is rapidly increasing. In the United States and Europe, esophageal adenocarcinoma has superseded esophageal squamous cell carcinoma in its incidence. Esophageal cancer has a high mortality rates secondary to the late presentation of most patients at advanced stages. Endoscopic screening is recommended for patients with multiple risk factors for cancer in Barrett's esophagus. These risk factors include chronic gastroesophageal reflux disease, hiatal hernia, advanced age, male sex, white race, cigarette smoking, and obesity. The annual risk of esophageal cancer is approximately 0.25% for patients without dysplasia and 6% for patients with high-grade dysplasia. Twenty percent of all esophageal adenocarcinoma in the United States is early stage with disease confined to the mucosa or submucosa. The significant morbidity and mortality of esophagectomy make endoscopic treatment an attractive option. The American Gastroenterological Association recommends endoscopic eradication therapy for patients with high-grade dysplasia. Endoscopic modalities for treatment of early esophageal adenocarcinoma include endoscopic resection techniques and endoscopic ablative techniques such as radiofrequency ablation, photodynamic therapy and cryoablation. Endoscopic therapy should be precluded to patients with no evidence of lymphovascular invasion. Local tumor recurrence is low after endoscopic therapy and is predicted by poor differentiation of tumor, positive lymph node and submucosal invasion. Surgical resection should be offered to patients with deep submucosal invasion. PMID:25132925

  3. Endoscopic assessment and management of early esophageal adenocarcinoma

    PubMed Central

    Hammoud, Ghassan M; Hammad, Hazem; Ibdah, Jamal A

    2014-01-01

    Esophageal carcinoma affects more than 450000 people worldwide and the incidence is rapidly increasing. In the United States and Europe, esophageal adenocarcinoma has superseded esophageal squamous cell carcinoma in its incidence. Esophageal cancer has a high mortality rates secondary to the late presentation of most patients at advanced stages. Endoscopic screening is recommended for patients with multiple risk factors for cancer in Barrett’s esophagus. These risk factors include chronic gastroesophageal reflux disease, hiatal hernia, advanced age, male sex, white race, cigarette smoking, and obesity. The annual risk of esophageal cancer is approximately 0.25% for patients without dysplasia and 6% for patients with high-grade dysplasia. Twenty percent of all esophageal adenocarcinoma in the United States is early stage with disease confined to the mucosa or submucosa. The significant morbidity and mortality of esophagectomy make endoscopic treatment an attractive option. The American Gastroenterological Association recommends endoscopic eradication therapy for patients with high-grade dysplasia. Endoscopic modalities for treatment of early esophageal adenocarcinoma include endoscopic resection techniques and endoscopic ablative techniques such as radiofrequency ablation, photodynamic therapy and cryoablation. Endoscopic therapy should be precluded to patients with no evidence of lymphovascular invasion. Local tumor recurrence is low after endoscopic therapy and is predicted by poor differentiation of tumor, positive lymph node and submucosal invasion. Surgical resection should be offered to patients with deep submucosal invasion. PMID:25132925

  4. Clinical and endoscopic characteristics of drug-induced esophagitis

    PubMed Central

    Kim, Su Hwan; Jeong, Ji Bong; Kim, Ji Won; Koh, Seong-Joon; Kim, Byeong Gwan; Lee, Kook Lae; Chang, Mee Soo; Im, Jong Pil; Kang, Hyoun Woo; Shin, Cheol Min

    2014-01-01

    AIM: To investigate clinical, endoscopic and pathological characteristics of drug-induced esophagitis. METHODS: Data for patients diagnosed with drug-induced esophagitis from April 2002 to May 2013 was reviewed. Patients diagnosed with malignancy, viral or fungal esophagitis were excluded. Clinical, endoscopic and pathological characteristics of patients diagnosed with drug-induced esophagitis were analyzed. RESULTS: Seventy-eight patients were diagnosed with drug-induced esophagitis. Their mean age was 43.9 ± 18.9 years and 35.9% were male. Common symptoms were chest pain (71.8%), odynophagia (38.5%) and dysphagia (29.5%). The endoscopic location was in the middle third of esophagus in 78.2%. Endoscopic findings were ulcer (82.1%), erosion (17.9%), ulcer with bleeding (24.4%), coating with drug material (5.1%), impacted pill fragments (3.8%) and stricture (2.6%). Kissing ulcers were observed in 43.6%. The main causative agents were antibiotics and non-steroidal anti-inflammatory drugs. All the patients were treated with proton pump inhibitors (PPIs) or sucralfate, and the causative drugs were discontinued. Nineteen patients with drug-induced esophagitis were followed up with endoscopy and revealed normal findings, scars or healing ulcers. CONCLUSION: Drug-induced esophagitis mainly presents as chest pain, odynophagia and dysphagia, and may be successfully treated with PPIs and discontinuation of the causative drug. Kissing ulcers were observed in 43.6%. PMID:25152603

  5. An optimized lead system for long-term esophageal electrocardiography.

    PubMed

    Niederhauser, T; Haeberlin, A; Marisa, T; Mattle, D; Abächerli, R; Goette, J; Jacomet, M; Vogel, R

    2014-04-01

    Long-term electrocardiography (ECG) featuring adequate atrial and ventricular signal quality is highly desirable. Routinely used surface leads are limited in atrial signal sensitivity and recording capability impeding complete ECG delineation, i.e. in the presence of supraventricular arrhythmias. Long-term esophageal ECG might overcome these limitations but requires a dedicated lead system and recorder design. To this end, we analysed multiple-lead esophageal ECGs with respect to signal quality by describing the ECG waves as a function of the insertion level, interelectrode distance, electrode shape and amplifier's input range. The results derived from clinical data show that two bipolar esophageal leads, an atrial lead with short (15 mm) interelectrode distance and a ventricular lead with long (80 mm) interelectrode distance provide non-inferior ventricular signal strength and superior atrial signal strength compared to standard surface lead II. High atrial signal slope in particular is observed with the atrial esophageal lead. The proposed esophageal lead system in combination with an increased recorder input range of ±20 mV minimizes signal loss due to excessive electrode motion typically observed in esophageal ECGs. The design proposal might help to standardize long-term esophageal ECG registrations and facilitate novel ECG classification systems based on the independent detection of ventricular and atrial electrical activity.

  6. New Endoscopic Indicator of Esophageal Achalasia: “Pinstripe Pattern”

    PubMed Central

    Minami, Hitomi; Isomoto, Hajime; Miuma, Satoshi; Kobayashi, Yasutoshi; Yamaguchi, Naoyuki; Urabe, Shigetoshi; Matsushima, Kayoko; Akazawa, Yuko; Ohnita, Ken; Takeshima, Fuminao; Inoue, Haruhiro; Nakao, Kazuhiko

    2015-01-01

    Background and Study Aims Endoscopic diagnosis of esophageal achalasia lacking typical endoscopic features can be extremely difficult. The aim of this study was to identify simple and reliable early indicator of esophageal achalasia. Patients and Methods This single-center retrospective study included 56 cases of esophageal achalasia without previous treatment. As a control, 60 non-achalasia subjects including reflux esophagitis and superficial esophageal cancer were also included in this study. Endoscopic findings were evaluated according to Descriptive Rules for Achalasia of the Esophagus as follows: (1) esophageal dilatation, (2) abnormal retention of liquid and/or food, (3) whitish change of the mucosal surface, (4) functional stenosis of the esophago-gastric junction, and (5) abnormal contraction. Additionally, the presence of the longitudinal superficial wrinkles of esophageal mucosa, “pinstripe pattern (PSP)” was evaluated endoscopically. Then, inter-observer diagnostic agreement was assessed for each finding. Results The prevalence rates of the above-mentioned findings (1–5) were 41.1%, 41.1%, 16.1%, 94.6%, and 43.9%, respectively. PSP was observed in 60.7% of achalasia, while none of the control showed positivity for PSP. PSP was observed in 26 (62.5%) of 35 cases with shorter history < 10 years, which usually lacks typical findings such as severe esophageal dilation and tortuosity. Inter-observer agreement level was substantial for food/liquid remnant (k = 0.6861) and PSP (k = 0.6098), and was fair for abnormal contraction and white change. The accuracy, sensitivity, and specificity for achalasia were 83.8%, 64.7%, and 100%, respectively. Conclusion “Pinstripe pattern” could be a reliable indicator for early discrimination of primary esophageal achalasia. PMID:25664812

  7. Characteristics of transient lower esophageal sphincter relaxation in humans.

    PubMed

    Mittal, R K; McCallum, R W

    1987-05-01

    Transient lower esophageal sphincter relaxations (TLESR) were studied in 10 normal healthy subjects. Electrical activity of mylohyoid muscle measured by an electromyogram (MEMG), pressures from pharynx, three esophageal sites, lower esophageal sphincter, and stomach were simultaneously recorded for 1 h, while fasting and 3 h after an 850 kcal meal. Reflux of acid into esophagus and/or occurrence of belching accompanying a TLESR was also monitored. TLESRs occurred with an equal frequency in fasting and postprandial state (6.2 vs. 6.4 h). However, frequency of an acid reflux during a TLESR was much greater postprandially than after fasting (44.8 vs. 9.6%). Belching coincided with 8% of TLESRs. A small MEMG complex and a small pharyngeal complex were present at onset of TLESR in 41.6 and 26.9% of instances, respectively. TLESRs were then categorized as either postswallow, if it occurred within 10 s of a preceding swallow-induced LES relaxation, or isolated, if its onset to previous swallow was greater than 10 s. Esophageal contractions were noticed at onset of 84% of isolated TLESRs. When present at two distal sites, this contraction was always of a simultaneous nature. Esophageal contractions at onset of postswallow TLESR were less frequent (33.3%) but when present were usually observed at the proximal esophageal site. At completion of a TLESR, the LES never recovered without an associated esophageal contraction, the latter was either swallow mediated or a spontaneous simultaneous esophageal contraction. Our data indicate that 1) MEMG and pharyngeal motor events may accompany TLESRs; and 2) esophageal contraction frequently heralds the onset, and it always occurs at completion of a TLESR.(ABSTRACT TRUNCATED AT 250 WORDS)

  8. Characterizing the inflammatory response in esophageal mucosal biopsies in children with eosinophilic esophagitis

    PubMed Central

    Sayej, Wael N; Ménoret, Antoine; Maharjan, Anu S; Fernandez, Marina; Wang, Zhu; Balarezo, Fabiola; Hyams, Jeffrey S; Sylvester, Francisco A; Vella, Anthony T

    2016-01-01

    Eosinophilic esophagitis (EoE) is an emerging allergic, IgE- and non-IgE (Th2 cell)-mediated disease. There are major gaps in the understanding of the basic mechanisms that drive the persistence of EoE. We investigated whether esophageal biopsies from children with EoE demonstrate an inflammatory response that is distinct from normal controls. We prospectively enrolled 84 patients, of whom 77 were included in our analysis, aged 4–17 years (12.8±3.8 years; 81% males). Five esophageal biopsies were collected from each patient at the time of endoscopy. Intramucosal lymphocytes were isolated, phenotyped and stimulated with phorbol 12-myristate 13-acetate/ionomycin to measure their potential to produce cytokines via flow cytometry. We also performed cytokine arrays on 72-h biopsy culture supernatants. CD8+ T cells, compared with CD4+ T cells, synthesized more TNF-α and interferon (IFN)-γ after mitogen stimulation in the EoE-New/Active vs EoE-Remission group (P=0.0098; P=0.02) and controls (P=0.0008; P=0.03). Culture supernatants taken from explant esophageal tissue contained 13 analytes that distinguished EoE-New/Active from EoE-Remission and Controls. Principal component analysis and cluster analysis based on these analytes distinctly separated EoE-New/Active from EoE-Remission and Controls. In summary, we have identified a previously unappreciated role for CD8+ T lymphocytes with potential to produce TNF-α and IFN-γ in EoE. Our results suggest that CD8+ T cells have a role in the persistence or progression of EoE. We have also identified a panel of analytes produced by intact esophageal biopsies that differentiates EoE-New/Active from EoE-Remission and controls. Our results suggest that esophageal epithelial cells may have specific immune effector functions in EoE that control the type and amplitude of inflammation. PMID:27525061

  9. Positive esophageal proximal resection margin: an important prognostic factor for esophageal cancer that warrants adjuvant therapy

    PubMed Central

    Wang, Yun-Cang; Deng, Han-Yu; Wang, Wen-Ping; He, Du; Ni, Peng-Zhi; Hu, Wei-Peng; Wang, Zhi-Qiang

    2016-01-01

    Background Positive esophageal proximal resection margin (ERM+) following esophagectomy was considered as incomplete or R1 resection. The clinicopathological data and long-term prognosis of esophageal cancer (EC) patients with ERM+ after esophagectomy were still unknown. Therefore, the aim of this study was to assess the clinical significance of ERM+ and its therapeutic option. Methods From November 2008 to December 2014, 3,594 patients with histologically confirmed EC underwent radical resection in our department. Among them there were 37 patients (1.03%) who had ERM+. ERM+ was defined as carcinoma or atypical hyperplasia (severe or moderate) at the residual esophageal margin in our study. For comparison, another 74 patients with negative esophageal proximal resection margin (ERM−) were propensity-matched at a ratio of 1:2 as control group according to sex, age, tumor location and TNM staging. The relevant prognostic factors were investigated by univariate and multivariate regression analysis. Results In this large cohort of patients, the rate of ERM+ was 1.03%. The median survival time was 35.000 months in patients with ERM+, significantly worse than 68.000 months in those with ERM− (Chi-square =4.064, P=0.044). Survival in patients with esophageal residual atypical hyperplasia (severe or moderate) was similar to those with esophageal residual carcinoma. Survival rate in stage I–II was higher than that in stage III–IV (Chi-square =27.598, P=0.000) in ERM−; But there was no difference between the two subgroups of patients in ERM+. Furthermore, in those patients with ERM+, survival was better in those who having adjuvant therapy, compared to those without adjuvant therapy (Chi-square =5.480, P=0.019). And the average survival time which was improved to a well situation for ERM+ patients who have adjuvant therapy was 68.556 months which is comparable to average survival time (65.815 months) of ERM− for those patients who are at earlier stages

  10. Endoscopic and Abdominal Management of Complete Benign Esophageal Obstruction

    PubMed Central

    2016-01-01

    Benign esophageal strictures leading to complete esophageal occlusion are well known. In the pre-endoscopic era, such cases required surgery, but over the last decade, various novel endoscopic techniques have been developed to prevent morbidity and mortality. A 37-year-old man presented after 1 year of dysphagia and weight loss, and was found to have complete esophageal obstruction, not allowing even passage of guidewire. We used a combination antegrade endoscopic abdominal procedures to deploy a stent, obviating the need for surgery. His symptoms improved dramatically, and the stent was successfully removed 12 weeks later. He is now swallowing normally and has gained significant weight. PMID:27144192

  11. Esophageal Perforation with Unilateral Fluidothorax Caused by Nasogastric Tube

    PubMed Central

    Müller, Martin; Reiterer, Friedrich; Pilhatsch, Alexander; Gürtl-Lackner, Barbara; Urlesberger, Berndt

    2016-01-01

    Preterm infants are highly susceptible to injuries following necessary and often life-saving medical interventions. Esophageal perforation is a rare, yet serious complication that can be caused by aerodigestive tract suction, endotracheal intubation, or nasogastric tube placement. We present the case of a neonate born at 23 weeks plus three days of gestation with chest radiography showing malposition of the nasogastric feeding tube and massive right-sided effusion of Iopamidol in the pleural cavity due to esophageal perforation. In addition, the article summarizes common signs and symptoms associated with esophageal perforation in infants and discusses diagnostic approaches. PMID:27803831

  12. Esophageal intramural pseudodiverticulosis as a diagnostic and therapeutic problem

    PubMed Central

    Gątarek, Juliusz; Orłowski, Tadeusz

    2016-01-01

    The article presents the case of a 68-year-old patient with alcohol dependence syndrome, who was admitted, in serious condition, to the Department of Surgery due to esophageal intramural lesions of unclear etiology. The imaging studies showed no signs of transmural perforation of the esophageal wall. Esophagogastroscopy revealed intramural fluid reservoirs and small oval cavities with smooth edges in the esophageal mucosa. The patient was treated conservatively with parenteral nutrition and rehabilitation. Subsequently, the patient was transferred to the intensive care unit because of cardiorespiratory failure. Despite adequate pharmacological treatment, the patient died. PMID:27785146

  13. High Mobility Group A proteins in esophageal carcinomas.

    PubMed

    Palumbo Júnior, Antonio; Da Costa, Nathalia Meireles; Esposito, Francesco; Fusco, Alfredo; Pinto, Luis Felipe Ribeiro

    2016-09-16

    We have recently shown that HMGA2 is overexpressed in esophageal squamous cell carcinoma (ESCC) and its detection allows to discriminate between cancer and normal surrounding tissue proposing HMGA2 as a novel diagnostic marker. Interestingly, esophageal adenocarcinoma shows an opposite behavior with the overexpression of HMGA1 but not HMGA2. Moreover, we show that the suppression of HMGA2 in 2 ESCC cell lines reduces the malignant phenotype. Then, this paper highlights a differential induction of the HMGA proteins, depending on the cancer histological type, and reinforces the perspective of an innovative esophageal cancer therapy based on the suppression of the HMGA protein function and/or expression.

  14. Plummer-Vinson Syndrome with Proximal Esophageal Web.

    PubMed

    Changela, Kinesh; Haeri, Nami Safai; Krishnaiah, Mahesh; Reddy, Madhavi

    2016-05-01

    Plummer-Vinson Syndrome is a condition where iron deficiency is associated with difficulty swallowing due to the presence of an esophageal web. Deficiency of iron-dependent oxidative enzymes causes gradual degradation of the pharyngeal muscles which lead to mucosal atrophy and formation of webs. Although it is a very rare condition, an increased risk of esophageal squamous cell carcinoma makes its identification very important. Dilation of the esophageal web using a Savary dilator is a more effective and safer approach compared to conventional balloon dilation.

  15. Simultaneous Esophageal Squamous Cell Carcinoma and Adenocarcinoma: A Case Report

    PubMed Central

    Maleki, Iradj; Shekarriz, Ramin; Nosrati, Anahita; Orang, Elahe

    2015-01-01

    Esophageal squamous cell carcinoma is a rather common cancer in northern Iran. Incidence of adenocarcinoma of esophagus has an increasing trend in Iran. Co-existence of both cancers in one patient is very rare. We report a middle age woman from northern Iran with a typical presentation of esophageal cancer, who was found to have a dual esophageal cancer. The disease was found in the advanced stage with pulmonary metastasis at the presentation. Palliative chemo-radiotherapy induced partial clinical response PMID:26609356

  16. Simultaneous Esophageal Squamous Cell Carcinoma and Adenocarcinoma: A Case Report.

    PubMed

    Maleki, Iradj; Shekarriz, Ramin; Nosrati, Anahita; Orang, Elahe

    2015-10-01

    Esophageal squamous cell carcinoma is a rather common cancer in northern Iran. Incidence of adenocarcinoma of esophagus has an increasing trend in Iran. Co-existence of both cancers in one patient is very rare. We report a middle age woman from northern Iran with a typical presentation of esophageal cancer, who was found to have a dual esophageal cancer. The disease was found in the advanced stage with pulmonary metastasis at the presentation. Palliative chemo-radiotherapy induced partial clinical response. PMID:26609356

  17. Electrodes for long-term esophageal electrocardiography.

    PubMed

    Niederhauser, Thomas; Haeberlin, Andreas; Marisa, Thanks; Jungo, Michael; Goette, Josef; Jacomet, Marcel; Abacherli, Roger; Vogel, Rolf

    2013-09-01

    The emerging application of long-term and high-quality ECG recording requires alternative electrodes to improve the signal quality and recording capability of surface skin electrodes. The esophageal ECG has the potential to overcome these limitations but necessitates novel recorder and lead designs. The electrode material is of particular interest, since the material has to ensure conflicting requirements like excellent biopotential recording properties and inertness. To this end, novel electrode materials like PEDOT and silver-PDMS as well as established electrode materials such as stainless steel, platinum, gold, iridium oxide, titanium nitride, and glassy carbon were investigated by long-term electrochemical impedance spectroscopy and model-based signal analysis using the derived in vitro interfacial properties in conjunction with a dedicated ECG amplifier. The results of this novel approach show that titanium nitride and iridium oxide featuring microstructured surfaces did not degrade when exposed to artificial acidic saliva. These materials provide low electrode potential drifts and insignificant signal distortion superior to surface skin electrodes making them compatible with accepted standards for ambulatory ECG. They are superior to the noble and polarizable metals such as platinum, silver, and gold that induced more signal distortions and are superior to esophageal stainless steel electrodes that corrode in artificial saliva. The study provides rigorous criteria for the selection of electrode materials for prolonged ECG recording by combining long-term in vitro electrode material properties with ECG signal quality assessment.

  18. Genetic landscape of esophageal squamous cell carcinoma.

    PubMed

    Gao, Yi-Bo; Chen, Zhao-Li; Li, Jia-Gen; Hu, Xue-Da; Shi, Xue-Jiao; Sun, Zeng-Miao; Zhang, Fan; Zhao, Zi-Ran; Li, Zi-Tong; Liu, Zi-Yuan; Zhao, Yu-Da; Sun, Jian; Zhou, Cheng-Cheng; Yao, Ran; Wang, Su-Ya; Wang, Pan; Sun, Nan; Zhang, Bai-Hua; Dong, Jing-Si; Yu, Yue; Luo, Mei; Feng, Xiao-Li; Shi, Su-Sheng; Zhou, Fang; Tan, Feng-Wei; Qiu, Bin; Li, Ning; Shao, Kang; Zhang, Li-Jian; Zhang, Lan-Jun; Xue, Qi; Gao, Shu-Geng; He, Jie

    2014-10-01

    Esophageal squamous cell carcinoma (ESCC) is one of the deadliest cancers. We performed exome sequencing on 113 tumor-normal pairs, yielding a mean of 82 non-silent mutations per tumor, and 8 cell lines. The mutational profile of ESCC closely resembles those of squamous cell carcinomas of other tissues but differs from that of esophageal adenocarcinoma. Genes involved in cell cycle and apoptosis regulation were mutated in 99% of cases by somatic alterations of TP53 (93%), CCND1 (33%), CDKN2A (20%), NFE2L2 (10%) and RB1 (9%). Histone modifier genes were frequently mutated, including KMT2D (also called MLL2; 19%), KMT2C (MLL3; 6%), KDM6A (7%), EP300 (10%) and CREBBP (6%). EP300 mutations were associated with poor survival. The Hippo and Notch pathways were dysregulated by mutations in FAT1, FAT2, FAT3 or FAT4 (27%) or AJUBA (JUB; 7%) and NOTCH1, NOTCH2 or NOTCH3 (22%) or FBXW7 (5%), respectively. These results define the mutational landscape of ESCC and highlight mutations in epigenetic modulators with prognostic and potentially therapeutic implications. PMID:25151357

  19. Early esophageal cancer detection using RF classifiers

    NASA Astrophysics Data System (ADS)

    Janse, Markus H. A.; van der Sommen, Fons; Zinger, Svitlana; Schoon, Erik J.; de With, Peter H. N.

    2016-03-01

    Esophageal cancer is one of the fastest rising forms of cancer in the Western world. Using High-Definition (HD) endoscopy, gastroenterology experts can identify esophageal cancer at an early stage. Recent research shows that early cancer can be found using a state-of-the-art computer-aided detection (CADe) system based on analyzing static HD endoscopic images. Our research aims at extending this system by applying Random Forest (RF) classification, which introduces a confidence measure for detected cancer regions. To visualize this data, we propose a novel automated annotation system, employing the unique characteristics of the previous confidence measure. This approach allows reliable modeling of multi-expert knowledge and provides essential data for real-time video processing, to enable future use of the system in a clinical setting. The performance of the CADe system is evaluated on a 39-patient dataset, containing 100 images annotated by 5 expert gastroenterologists. The proposed system reaches a precision of 75% and recall of 90%, thereby improving the state-of-the-art results by 11 and 6 percentage points, respectively.

  20. Targeting AMCase reduces esophageal eosinophilic inflammation and remodeling in a mouse model of egg induced eosinophilic esophagitis

    PubMed Central

    Cho, Jae Youn; Rosenthal, Peter; Miller, Marina; Pham, Alexa; Aceves, Seema; Sakuda, Shohei; Broide, David H

    2014-01-01

    Studies of AMCase inhibition in mouse models of lung eosinophilic inflammation have produced conflicting results with some studies demonstrating inhibition of eosinophilic inflammation and others not. No studies have investigated the role of AMCase inhibition in eosinophilic esophagitis (EoE). We have used a mouse model of egg (OVA) induced EoE to determine whether pharmacologic inhibition of AMCase with allosamidin reduced eosinophilic inflammation and remodeling in the esophagus in EoE. Administration of intra-esophageal OVA for 6 weeks to BALB/c mice induced increased levels of esophageal eosinophils, mast cells, and features of esophageal remodeling (fibrosis, basal zone hyperplasia, deposition of the extracellular matrix protein fibronectin). Administration of intraperitoneal (ip) allosamidin to BALB/c mice significantly inhibited AMCase enzymatic activity in the esophagus. Pharmacologic inhibition of AMCase with ip allosamidin inhibited both OVA induced increases in esophageal eosinophilic inflammation and OVA induced esophageal remodeling (fibrosis, epithelial basal zone hyperplasia, extracellular matrix deposition of fibronectin). This inhibition of eosinophilic inflammation in the esophagus by ip allosamidin was associated with reduced eotaxin-1 expression in the esophagus. Oral allosamidin inhibited eosinophilic inflammation in the epithelium but did not inhibit esophageal remodeling. These studies suggest that pharmacologic inhibition of AMCase results in inhibition of eosinophilic inflammation and remodeling in the esophagus in a mouse model of egg induced EoE partially through effects in the esophagus on reducing chemokines (i.e. eotaxin-1) implicated in the pathogenesis of EoE. PMID:24239745

  1. Secondary aorto-esophageal fistula after thoracic aortic aneurysm endovascular repair treated by covered esophageal stenting.

    PubMed

    Tao, Mary; Shlomovitz, Eran; Darling, Gail; Roche-Nagle, Graham

    2016-08-16

    Thoracic endovascular aortic repair for thoracic aortic aneurysms is an accepted alternative to open surgery, especially in patients with significant comorbidities. The procedure itself has a low risk of complications and fistulas to surrounding organs are rarely reported. An 86-year-old patient was admitted to our hospital with gastro intestinal (GI) bleeding and a suspected aortoesophageal fistula. Eight months prior, the patient had undergone a stent graft repair of a mycotic thoracic aneurysm. Computerized tomography angiography and upper GI endoscopy confirmed an aortoesophageal fistula, which was treated by esophageal stenting. With early recognition, esophageal stenting may have a role in the initial emergency control of bleeding from and palliation of aortoesophageal fistula. PMID:27574612

  2. Secondary aorto-esophageal fistula after thoracic aortic aneurysm endovascular repair treated by covered esophageal stenting

    PubMed Central

    Tao, Mary; Shlomovitz, Eran; Darling, Gail; Roche-Nagle, Graham

    2016-01-01

    Thoracic endovascular aortic repair for thoracic aortic aneurysms is an accepted alternative to open surgery, especially in patients with significant comorbidities. The procedure itself has a low risk of complications and fistulas to surrounding organs are rarely reported. An 86-year-old patient was admitted to our hospital with gastro intestinal (GI) bleeding and a suspected aortoesophageal fistula. Eight months prior, the patient had undergone a stent graft repair of a mycotic thoracic aneurysm. Computerized tomography angiography and upper GI endoscopy confirmed an aortoesophageal fistula, which was treated by esophageal stenting. With early recognition, esophageal stenting may have a role in the initial emergency control of bleeding from and palliation of aortoesophageal fistula. PMID:27574612

  3. Comprehensive Genomic Profiling of Advanced Esophageal Squamous Cell Carcinomas and Esophageal Adenocarcinomas Reveals Similarities and Differences

    PubMed Central

    Johnson, Adrienne; Ali, Siraj M.; Klempner, Samuel J.; Bekaii-Saab, Tanios; Vacirca, Jeffrey L.; Khaira, Depinder; Yelensky, Roman; Chmielecki, Juliann; Elvin, Julia A.; Lipson, Doron; Miller, Vincent A.; Stephens, Philip J.; Ross, Jeffrey S.

    2015-01-01

    Background. Esophageal squamous cell carcinomas (ESCCs) and esophageal adenocarcinomas (EACs) account for >95% of esophageal malignancies and represent a major global health burden. ESCC is the dominant histology globally but represents a minority of U.S. cases, with EAC accounting for the majority of U.S. cases. The patient outcomes for advanced ESCC and EAC are poor, and new therapeutic options are needed. Using a sensitive sequencing assay, we compared the genomic profiles of ESCC and EAC with attention to identification of therapeutically relevant genomic alterations. Methods. Next-generation sequencing-based comprehensive genomic profiling was performed on hybridization-captured, adaptor ligation-based libraries to a median coverage depth of >650× for all coding exons of 315 cancer-related genes plus selected introns from 28 genes frequently rearranged in cancer. Results from a single sample were evaluated for all classes of genomic alterations (GAs) including point mutations, short insertions and deletions, gene amplifications, homozygous deletions, and fusions/rearrangements. Clinically relevant genomic alterations (CRGAs) were defined as alterations linked to approved drugs and those under evaluation in mechanism-driven clinical trials. Results. There were no significant differences by sex for either tumor type, and the median age for all patients was 63 years. All ESCCs and EACs were at an advanced stage at the time of sequencing. All 71 ESCCs and 231 EACs featured GAs on profiling, with 522 GAs in ESCC (7.4 per sample) and 1,303 GAs in EAC (5.6 per sample). The frequency of clinically relevant GAs in ESCC was 94% (2.6 per sample) and 93% in EAC (2.7 per sample). CRGAs occurring more frequently in EAC included KRAS (23% EAC vs. 6% ESCC) and ERBB2 (23% EAC vs. 3% ESCC). ESCC samples were enriched for CRGA in PIK3CA (24% ESCC vs. 10% EAC), PTEN (11% ESCC vs. 4% EAC), and NOTCH1 (17% ESCC vs. 3% EAC). Other GAs that differed significantly between histologic

  4. The epidemiology of tracheo-oesophageal fistula and oesophageal atresia in Europe. EUROCAT Working Group.

    PubMed Central

    Depaepe, A; Dolk, H; Lechat, M F

    1993-01-01

    The total prevalence rate of tracheo-oesophageal fistula and oesophageal atresia in 15 EUROCAT registries covering 1,546,889 births during 1980-8 was 2.86 per 10,000. There was a decreasing prevalence rate over time (3.5 per 10,000 in 1980-2, 2.7 in 1983-5, 2.5 in 1986-8). Ten per cent of cases were associated with chromosomal anomalies and of the remaining cases, half were multiply malformed. Sixty two per cent of cases were males. There was a significantly increased risk for mothers of less than 20 years of age (odds ratio compared with mothers of 25-29 = 1.82, 95% confidence interval 1.23 to 2.67). There were no apparent epidemiological differences between isolated and multiply malformed cases in secular trend, sex ratio, or maternal age. Both isolated and multiply malformed cases tended to be premature and small for gestational age. There was variation between centres in survival of affected liveborn children up to 1 year of age. PMID:8333763

  5. Acquired unilateral pulmonary vein atresia in a 3-year-old boy.

    PubMed

    Firdouse, Mohammed; Agarwal, Arnav; Grosse-Wortmann, Lars; Mondal, Tapas K

    2015-03-01

    While unilateral pulmonary venous atresia (UPVA) most commonly presents as an extremely rare late embryological defect resulting in complete occlusion or absence of the PV pathway, it may also be an acquired pathology. We present a 3-year-old boy who presented with mild respiratory distress. Neonatal echocardiographic investigations revealed normal mediastinal anatomy and pulmonary vasculature with a bicuspid aortic valve. However, follow-up Doppler investigation revealed a pulmonary artery size difference with minimal forward flow and reverse flow during diastole. Absence of the left pulmonary veins and the presence of collaterals draining to the innominate vein confirmed the diagnosis of acquired UPVA. Our case represents the first case of acquired UPVA in conjunction with a normally functioning bicuspid aortic valve. The Doppler flow patterns discussed might be of significant interest to pediatricians, cardiologists and imaging specialists. These findings suggest that acquired UPVA should be considered in the differential diagnosis of such patients when radiographic and echocardiographic findings may rule out other more common diagnoses. While the management of such a condition remains unclear and conservative management was agreed upon for our patient, the vulnerability of such cases warrants timely diagnosis and routine monitoring.

  6. Methylation Microarray Studies Highlight PDGFA Expression as a Factor in Biliary Atresia

    PubMed Central

    Cofer, Zenobia C.; Cui, Shuang; EauClaire, Steven F.; Kim, Cecilia; Tobias, John W.; Hakonarson, Hakon; Loomes, Kathleen M.; Matthews, Randolph P.

    2016-01-01

    Biliary atresia (BA) is a progressive fibro-inflammatory disorder that is the leading indication for liver transplantation in children. Although there is evidence implicating genetic, infectious, environmental, and inflammatory causes, the etiology of BA remains unknown. We have recently reported that cholangiocytes from BA patients showed decreased DNA methylation relative to disease- and non-disease controls, supporting a potential role for DNA hypomethylation in BA etiopathogenesis. In the current study, we examined the methylation status of specific genes in human BA livers using methylation microarray technology. We found global DNA hypomethylation in BA samples as compared to disease- and non-disease controls at specific genetic loci. Hedgehog pathway members, SHH and GLI2, known to be upregulated in BA, were both hypomethylated, validating this approach as an investigative tool. Another region near the PDGFA locus was the most significantly hypomethylated in BA, suggesting potential aberrant expression. Validation assays confirmed increased transcriptional and protein expression of PDGFA in BA livers. We also show that PDGF-A protein is specifically localized to cholangiocytes in human liver samples. Injection of PDGF-AA protein dimer into zebrafish larvae caused biliary developmental and functional defects. In addition, activation of the Hedgehog pathway caused increased expression of PDGF-A in zebrafish larvae, providing a previously unrecognized link between PDGF and the Hedgehog pathway. Our findings implicate DNA hypomethylation as a specific factor in mediating overexpression of genes associated with BA and identify PDGF as a new candidate in BA pathogenesis. PMID:27010479

  7. Methylation Microarray Studies Highlight PDGFA Expression as a Factor in Biliary Atresia.

    PubMed

    Cofer, Zenobia C; Cui, Shuang; EauClaire, Steven F; Kim, Cecilia; Tobias, John W; Hakonarson, Hakon; Loomes, Kathleen M; Matthews, Randolph P

    2016-01-01

    Biliary atresia (BA) is a progressive fibro-inflammatory disorder that is the leading indication for liver transplantation in children. Although there is evidence implicating genetic, infectious, environmental, and inflammatory causes, the etiology of BA remains unknown. We have recently reported that cholangiocytes from BA patients showed decreased DNA methylation relative to disease- and non-disease controls, supporting a potential role for DNA hypomethylation in BA etiopathogenesis. In the current study, we examined the methylation status of specific genes in human BA livers using methylation microarray technology. We found global DNA hypomethylation in BA samples as compared to disease- and non-disease controls at specific genetic loci. Hedgehog pathway members, SHH and GLI2, known to be upregulated in BA, were both hypomethylated, validating this approach as an investigative tool. Another region near the PDGFA locus was the most significantly hypomethylated in BA, suggesting potential aberrant expression. Validation assays confirmed increased transcriptional and protein expression of PDGFA in BA livers. We also show that PDGF-A protein is specifically localized to cholangiocytes in human liver samples. Injection of PDGF-AA protein dimer into zebrafish larvae caused biliary developmental and functional defects. In addition, activation of the Hedgehog pathway caused increased expression of PDGF-A in zebrafish larvae, providing a previously unrecognized link between PDGF and the Hedgehog pathway. Our findings implicate DNA hypomethylation as a specific factor in mediating overexpression of genes associated with BA and identify PDGF as a new candidate in BA pathogenesis. PMID:27010479

  8. Global methylation, oxidative stress, and relative telomere length in biliary atresia patients.

    PubMed

    Udomsinprasert, Wanvisa; Kitkumthorn, Nakarin; Mutirangura, Apiwat; Chongsrisawat, Voranush; Poovorawan, Yong; Honsawek, Sittisak

    2016-01-01

    Alu and LINE-1 elements are retrotransposons with a ubiquitous presence in the human genome that can cause genomic instability, specifically relating to telomere length. Genotoxic agents may induce methylation of retrotransposons, in addition to oxidative DNA damage in the form of 8-hydroxy-2'-deoxyguanosine (8-OHdG). Methylation of retrotransposons induced by these agents may contribute to biliary atresia (BA) etiology. Here, we investigated correlations between global methylation, 8-OHdG, and relative telomere length, as well as reporting on Alu and LINE-1 hypomethylation in BA patients. Alu and LINE-1 hypomethylation were found to be associated with elevated risk of BA (OR = 4.07; 95% CI: 2.27-7.32; P < 0.0001 and OR = 3.51; 95% CI: 1.87-6.59; P < 0.0001, respectively). Furthermore, LINE-1 methylation was associated with liver stiffness in BA patients (β coefficient = -0.17; 95% CI: -0.24 to -0.10; P < 0.0001). Stratified analysis revealed negative correlations between Alu and LINE-1 methylation and 8-OHdG in BA patients (P < 0.0001). In contrast, positive relationships were identified between Alu and LINE-1 methylation and relative telomere length in BA patients (P < 0.0001). These findings suggest that retrotransposon hypomethylation is associated with plasma 8-OHdG and telomere length in BA patients. PMID:27243754

  9. GERD, Barrett's Esophagus and the Risk for Esophageal Cancer

    MedlinePlus

    ... Facts About Common Colon Cancer Screening Tests PATIENTS GERD, Barrett's Esophagus and the Risk for Esophageal Cancer ... commonly in Caucasians as well as people with gastroesophageal reflux disease (GERD). This cancer is increasing in frequency. ...

  10. [Intrathoracic esophageal perforation of unknown cause in four horses].

    PubMed

    Graubner, C; Gerber, V; Imhasly, A; Gorgas, D; Koch, C

    2011-10-01

    Three horses (age 17 - 23 years) were referred to the equine clinic of the University of Berne due to colic, fever, tachycardia and tachypnea. All horses showed pleural effusion. Clinical findings in 2 of the horses were highly suggestive of an intra-thoracic esophageal perforation. Severe septic pleuropneumonia without suspicion of an esophageal lesion was diagnosed in the 3rd horse. In addition, an 11 year old stallion was referred to the equine clinic for treatment of a presumptive large colon impaction. The horse was given laxatives after nasogastric intubation. Subsequent dramatic clinical deterioration and signs consistent with severe pleuropneumonia suggest that esophageal perforation had occurred when passing the nasogastric tube. All 4 horses were euthanized due to a poor prognosis. Esophageal perforation was diagnosed or confirmed post mortem in all cases. A hypertrophy of the tunica muscularis of the intra-thoracic esophagus was found in 3 of 4 horses. PMID:21971675

  11. Adding Targeted Therapy to Treatment for Esophageal Cancer

    Cancer.gov

    In this phase III clinical trial, people with confirmed HER2-positive locally advanced esophageal cancer will be randomly assigned to receive preoperative radiation therapy and chemotherapy, with or without trastuzumab.

  12. Eosinophilic Esophagitis: The "Not-So-Rare" Disease.

    PubMed

    Goh, Vi Lier

    2016-02-01

    Eosinophilic esophagitis (EoE) is a relatively newly described disorder with increasing incidence. Patients with EoE may present at all ages from childhood through adulthood. Presenting symptoms may vary from feeding refusal, gagging, and/or vomiting in the younger population, dysphagia, chest pain, and abdominal pain in adolescents, as well as emergent food impactions. However, there are strict diagnostic criteria that must be met to make the diagnosis. Specifically, the diagnosis of EoE requires at least 15 eosinophils per high-powered field in the esophageal biopsies and symptoms of esophageal dysfunction after other causes, such as gastroesophageal reflux disease and proton pump inhibitor-responsive esophageal eosinophilia, have been ruled out. Common treatments include diet modifications and/or topical corticosteroids. PMID:26878186

  13. Preoperative Chemotherapy, Radiation Improve Survival in Esophageal Cancer (Updated)

    Cancer.gov

    Patients with esophageal cancer who received chemotherapy and radiation before surgery survived, on average, nearly twice as long as patients treated with surgery alone, according to results of a randomized clinical trial published May 31, 2012, in NEJM.

  14. [Management of the esophageal candidiasis by the primary care physician].

    PubMed

    Behrens, Garance; Bocherens, Astrid; Senn, Nicolas

    2014-05-14

    Esophageal candidiasis is one of the most common opportunistic infections in patients infected by human immunodeficiency virus (HIV). This pathology is also found in patients without overt immunodeficiency. Other risk factors are known to be associated with this disease like inhaled or systemic corticosteroid treatment or proton-pump inhibitors and H2 receptor antagonists. In the absence of identified risk factors, a primary immune deficiency should be sought. Prevention of esophageal candidiasis is based primarily on the identification of risk factors, and a better control of them. This article presents a review of the physiopathology, clinical presentation and management of esophageal candidiasis by primary care physicians. We will also discuss ways of preventing esophageal candidiasis when necessary.

  15. Microscopic esophagitis and Barrett's esophagus: the histology report.

    PubMed

    Fiocca, Roberto; Mastracci, Luca; Milione, Massimo; Parente, Paola; Savarino, Vincenzo

    2011-03-01

    Gastro-esophageal reflux disease (GERD) is the most common digestive disease in industrialized countries (Europe and North America) and is associated with microscopic changes in the squamous epithelium. However, biopsy is not presently included in the routine diagnostic flow chart of GERD. In contrast, esophageal biopsy is mandatory when diagnosing Barrett's esophagus. High quality histology reports are necessary to provide information on diagnosis and can also be important for research and epidemiological studies. It has been evident for decades that pathology reports vary between institutions and even within a single institution. Standardization of reporting is the best way to ensure that information necessary for patient management is included in pathology reports. This paper details the histological criteria for diagnosing GERD-associated microscopic esophagitis, other forms of esophagitis with specific features and columnar metaplasia in the lower esophagus (Barrett's esophagus). It provides a detailed description of appropriate sampling criteria, individual lesions and how they contribute to the histology report.

  16. Gene-environment interactions in esophageal cancer.

    PubMed

    Matejcic, Marco; Iqbal Parker, M

    2015-01-01

    Esophageal cancer (EC) is one of the most common malignancies in low- and medium-income countries and represents a disease of public health importance because of its poor prognosis and high mortality rate in these regions. The striking variation in the prevalence of EC among different ethnic groups suggests a significant contribution of population-specific environmental and dietary factors to susceptibility to the disease. Although individuals within a demarcated geographical area are exposed to the same environment and share similar dietary habits, not all of them will develop the disease; thus genetic susceptibility to environmental risk factors may play a key role in the development of EC. A wide range of xenobiotic-metabolizing enzymes are responsible for the metabolism of carcinogens introduced via the diet or inhaled from the environment. Such dietary or environmental carcinogens can bind to DNA, resulting in mutations that may lead to carcinogenesis. Genes involved in the biosynthesis of these enzymes are all subject to genetic polymorphisms that can lead to altered expression or activity of the encoded proteins. Genetic polymorphisms may, therefore, act as molecular biomarkers that can provide important predictive information about carcinogenesis. The aim of this review is to discuss our current knowledge on the genetic risk factors associated with the development of EC in different populations; it addresses mainly the topics of genetic polymorphisms, gene-environment interactions, and carcinogenesis. We have reviewed the published data on genetic polymorphisms of enzymes involved in the metabolism of xenobiotics and discuss some of the potential gene-environment interactions underlying esophageal carcinogenesis. The main enzymes discussed in this review are the glutathione S-transferases (GSTs), N-acetyltransferases (NATs), cytochrome P450s (CYPs), sulfotransferases (SULTs), UDP-glucuronosyltransferases (UGTs), and epoxide hydrolases (EHs), all of which

  17. Anginal pain of esophageal origin: clinical presentation, prevalence, and prognosis.

    PubMed

    Davies, H A

    1992-05-27

    Since 1768, when Heberden recognized a relationship of angina pectoris with eating, the close resemblance between angina-like pain of esophageal and cardiac origin has led to diagnostic confusion, with the role of the esophagus being, in turn, over- and underemphasized as a cause of symptoms. Although the classic features of angina do not distinguish the origin of the pain, certain other symptoms may identify esophageal pain. These include an inconsistent correlation of exercise with pain, periods of prolonged remission, provocation of pain by posture, association with other esophageal symptoms, relief by antacids, radiation of pain down the right arm and into the back, occurrence of pain at night, continuation of pain as a background ache, and relief from nitroglycerine delayed by 10 minutes or longer. However, while certain symptoms may alert the clinician to the possibility that angina-like pain is due to esophageal disease, no single symptom or combination of symptoms is infallible; there is no alternative to careful assessment. Esophageal disease accounts for the greatest number of patients with chest pain of unknown origin. The prevalence of angina-like esophageal pain in unselected emergency admissions with suspected myocardial infarction is 10-20%. Approximately one third or more of patients with angina and normal coronary arteries have esophageal problems. We have followed patients with angina-like esophageal pain for 9 years. Although prognosis remains good, confirming the original noncardiac diagnosis, greater than 80% of patients continue to have chest pain of undiminished intensity, and half are limited in their ability to work. Reassurance appeared to have one beneficial result: Patients were less likely to consult a physician after a positive diagnosis had been made.

  18. [An epidemiological analysis on the geographic factors of esophageal cancer].

    PubMed

    Song, J

    1992-12-01

    The author collects the data of esophageal cancer mortality (1971-1973) of 78 counties in Hubei Province and the data of topography, climate, soil, rock formation and geochemical elements, including 40 suspected factors. The method of linear correlation and multiple stepwise regression are used for the comprehensive analysis of relation between the geographical factors and esophageal cancer. The result is that four factors metamorphic rock, zinc, copper, chromium are suspected factors. It suggests that the four factors will need future study.

  19. Candida Glabrata Esophagitis: new case reports and management

    PubMed Central

    Macêdo, Danielle Patrícia Cerqueira; da Silva, Vanessa Karina Alves; de Almeida Farias, Aline Mary; de Melo, Luciana Resende Bandeira; Wilheim, Ana Botler; Neves, Rejane Pereira

    2008-01-01

    Candida esophagitis (CE) is a common opportunistic infection in the immunocompromised host. C. glabrata is rarely cited as agent of CE and has been underestimated due to lack of proper identification. In this study, two cases of C. glabrata esophagitis in AIDS and chagasic patients are reported. Diagnosis of Candida species should be considered an important key for the ideal choice of antifungal therapy against this mycosis. PMID:24031216

  20. Androgens and esophageal cancer: What do we know?

    PubMed Central

    Sukocheva, Olga A; Li, Bin; Due, Steven L; Hussey, Damian J; Watson, David I

    2015-01-01

    Significant disparities exist between genders for the development and progression of several gastro-intestinal (GI) diseases including cancer. Differences in incidence between men vs women for colon, gastric and hepatocellular cancers suggest a role for steroid sex hormones in regulation of GI carcinogenesis. Involvement of intrinsic gender-linked mechanisms is also possible for esophageal adenocarcinoma as its incidence is disproportionally high among men. However, the cause of the observed gender differences and the potential role of androgens in esophageal carcinogenesis remains unclear, even though the cancer-promoting role of androgen receptors (AR) shown in other cancers such as prostate and bladder suggests this aspect warrants exploration. Several studies have demonstrated expression of ARs in esophageal cancer. However, only one study has suggested a potential link between AR signaling and outcome - poorer prognosis. Two groups have analyzed data from cohorts with prostate cancer and one of these found a decreased incidence of esophageal squamous and adenocarcinoma after androgen deprivation therapy. However, very limited information is available about the effects of androgen and AR-initiated signaling on esophageal cancer cell growth in vitro and in vivo. Possible mechanisms for androgens/AR involvement in the regulation of esophageal cancer growth are considered, and the potential use of AR as a prognostic factor and clinical target is highlighted, although insufficient evidence is available to support clinical trials of novel therapies. As esophageal adenocarcinoma is a gender linked cancer with a large male predominance further studies are warranted to clarify the role of androgens and ARs in shaping intracellular signaling and genomic responses in esophageal cancer. PMID:26034350

  1. Data analyses and perspectives on laparoscopic surgery for esophageal achalasia

    PubMed Central

    Tsuboi, Kazuto; Omura, Nobuo; Yano, Fumiaki; Hoshino, Masato; Yamamoto, Se-Ryung; Akimoto, Shunsuke; Masuda, Takahiro; Kashiwagi, Hideyuki; Yanaga, Katsuhiko

    2015-01-01

    In general, the treatment methods for esophageal achalasia are largely classified into four groups, including drug therapy using nitrite or a calcium channel blocker, botulinum toxin injection, endoscopic therapy such as endoscopic balloon dilation, and surgery. Various studies have suggested that the most effective treatment of esophageal achalasia is surgical therapy. The basic concept of this surgical therapy has not changed since Heller proposed esophageal myotomy for the purpose of resolution of lower esophageal obstruction for the first time in 1913, but the most common approach has changed from open-chest surgery to laparoscopic surgery. Currently, the laparoscopic surgery has been the procedure of choice for the treatment of esophageal achalasia. During the process of the transition from open-chest surgery to laparotomy, to thoracoscopic surgery, and to laparoscopic surgery, the necessity of combining antireflux surgery has been recognized. There is some debate as to which type of antireflux surgery should be selected. The Toupet fundoplication may be the most effective in prevention of postoperative antireflux, but many medical institutions have selected the Dor fundoplication which covers the mucosal surface exposed by myotomy. Recently, a new endoscopic approach, peroral endoscopic myotomy (POEM), has received attention. Future studies should examine the long-term outcomes and whether POEM becomes the gold standard for the treatment of esophageal achalasia. PMID:26478674

  2. Research advances in esophageal diseases: bench to bedside

    PubMed Central

    di Pietro, Massimiliano

    2013-01-01

    Over the last year, significant steps have been made toward understanding the pathogenesis of esophageal diseases and translating this knowledge to clinical practice. Gastroesophageal reflux disease (GERD) is the most common outpatient diagnosis in gastroenterology and has a high prevalence in the general population. As many as 40% of patients with GERD have incomplete response to medical therapy, and the pathophysiological mechanisms underlying lack of response are now better understood. Novel medical and minimally invasive interventions are available to optimize management of GERD. Esophageal cancer, regardless of the histological subtype, has among the worst survival statistics among all malignancies. Taking advantage of technological advances in genome sequencing, the mutational spectra in esophageal cancer are now emerging, offering novel avenues for targeted therapies. Early diagnosis is another strand for improving survival. While genome-wide association studies are providing insights into genetic susceptibility, novel approaches to early detection of cancer are being devised through the use of biomarkers applied to esophageal samples and as part of imaging technologies. Dysmotility and eosinophilic esophagitis are the differential diagnoses in patients with dysphagia. New pathophysiological classifications have improved the management of motility disorders. Meanwhile, exciting progress has been made in the endoscopic management of these conditions. Eosinophilic esophagitis is still a relatively new entity, and the pathogenesis remains poorly understood. However, it is now clear that an allergic reaction to food plays an important role, and dietary interventions as well as biologic agents to block the inflammatory cascade are novel, promising fields of clinical research. PMID:24167725

  3. Esophageal eosinophilia in pediatric patients with cerebral palsy

    PubMed Central

    de Nápolis, Ana Carolina Ramos; Alves, Flavia Araujo; Rezende, Erica Rodrigues Mariano de Almeida; Segundo, Gesmar Rodrigues Silva

    2015-01-01

    ABSTRACT Objective: To describe the clinical picture, test results, and clinical evolution of patients with cerebral palsy associated with diagnosis of eosinophilic esophagitis, monitored at tertiary centre. Methods: Cross-sectional, retrospective and descriptive study that evaluated the medical records data of pediatric patients with diagnosis of cerebral palsy and eosinophilic esophagitis in a tertiary center of pediatric gastroenterology between August 2005 and August 2013. Results: Seven out of 131 patients with cerebral palsy had the diagnosis of eosinophilic esophagitis. The mean age at diagnosis of eosinophilic esophagitis was 52.3 months and the mean number of eosinophils in esophagus was 35 per high-power field. Symptoms more frequent were recurrent vomiting and disphagia. Endoscopic alterations found were mucosal thickening, vertical lines, mucosal opacificacion and white plaques. Conclusion: The frequency of eosinophilic esophagitis found was higher than in general pediatric population. The investigation of eosinophilic esophagitis should be done regularly in those patients, once this entity could overlap other gastrointestinal diseases. PMID:26154544

  4. Olfactory stimuli provoke diffuse esophageal spasm: reversal by ipratropium bromide.

    PubMed

    Triadafilopoulos, G; Tsang, H P

    1996-10-01

    Diffuse esophageal spasm (DES) is a motor disorder of the esophageal smooth muscle characterized by multiple spontaneous contractions and by swallow-induced contractions that are of simultaneous onset, large amplitude, long duration, and repetitive occurrence. Although the pathogenesis of DES is unknown, provocative studies with cholinergic stimulation, esophageal balloon distention, or acid instillation have suggested involvement of both sensory and motor mechanisms. This report describes a patient with DES who would predictably become symptomatic with dysphagia and chest pain upon inhalation of perfume or other strong odors. Using esophageal scintigraphy to quantitate and analyze esophageal transit in this patient, we report for the first time that olfactory stimulation triggers episodes of DES and that such phenomena are mediated through the vagus nerve, because they can be ameliorated by the administration of ipratropium bromide. These observations suggest a new (sensory) pathway for the induction of DES and raise the intriguing possibility that inhaled anticholinergics may have a therapeutic role in the management of spastic esophageal motility disorders.

  5. Eosinophilic esophagitis: New insights in pathogenesis and therapy.

    PubMed

    Guarino, Michele Pier Luca; Cicala, Michele; Behar, Jose

    2016-02-01

    Eosinophilic esophagitis (EoE) is a clinico-pathological entity with esophageal symptoms and dense esophageal eosinophilic infiltration throughout the esophagus that may persist despite treatment with proton pump inhibitors. This eosinophilic infiltration is usually absent in the stomach, small intestine and colon, although there are a number of reports of patients with a multi-organ involvement. EoE is associated with abnormalities involving TH2-dependent immunity, with multiple environmental factors strongly contributing to disease expression. The layer of the esophagus affected by the eosinophilic infiltration causes the specific symptoms. Esophageal involvement results mostly in dysphagia for solids that can be severe enough to cause recurrent esophageal obstruction with typical endoscopic features suggesting esophageal remodeling and pathological changes of eosinophilic infiltration of the mucosa, sub-epithelial fibrosis and muscle hypertrophy. This disease is frequently associated with other allergic conditions such as allergic asthma, allergic dermatitis and eosinophilia. The treatment of patients with EoE depends on the severity of the symptoms and of the inflammatory process as well as to their response to a gradual step-up treatment. The first line of treatment consists of steroid containing local inhalers. If unresponsive they are then treated with oral steroids. Intravenous interleukin blockers seem to have a consistent positive therapeutic effect. PMID:26855813

  6. Eosinophilic esophagitis: New insights in pathogenesis and therapy

    PubMed Central

    Guarino, Michele Pier Luca; Cicala, Michele; Behar, Jose

    2016-01-01

    Eosinophilic esophagitis (EoE) is a clinico-pathological entity with esophageal symptoms and dense esophageal eosinophilic infiltration throughout the esophagus that may persist despite treatment with proton pump inhibitors. This eosinophilic infiltration is usually absent in the stomach, small intestine and colon, although there are a number of reports of patients with a multi-organ involvement. EoE is associated with abnormalities involving TH2-dependent immunity, with multiple environmental factors strongly contributing to disease expression. The layer of the esophagus affected by the eosinophilic infiltration causes the specific symptoms. Esophageal involvement results mostly in dysphagia for solids that can be severe enough to cause recurrent esophageal obstruction with typical endoscopic features suggesting esophageal remodeling and pathological changes of eosinophilic infiltration of the mucosa, sub-epithelial fibrosis and muscle hypertrophy. This disease is frequently associated with other allergic conditions such as allergic asthma, allergic dermatitis and eosinophilia. The treatment of patients with EoE depends on the severity of the symptoms and of the inflammatory process as well as to their response to a gradual step-up treatment. The first line of treatment consists of steroid containing local inhalers. If unresponsive they are then treated with oral steroids. Intravenous interleukin blockers seem to have a consistent positive therapeutic effect. PMID:26855813

  7. Impact of Weight Loss Surgery on Esophageal Physiology

    PubMed Central

    Naik, Rishi D.; Choksi, Yash A.

    2015-01-01

    Bariatric surgery has come to the forefront of weight loss treatment due to its complex interactions via anatomic, physiologic, and neurohormonal changes leading to sustained weight loss. Unlike lifestyle and pharmacologic options, which fail to show long-term sustained weight loss, bariatric surgery has been shown to decrease overall mortality and morbidity. Bariatric surgery can be purely restrictive, such as laparoscopic adjustable gastric band (LAGB) or laparoscopic sleeve gastrectomy (LSG), or restrictive-malabsorptive, such as Roux-en-Y gastric bypass (RYGB). These surgeries cause specific anatomic changes that promote weight loss; however, they also have unintended effects on the esophagus, particularly in terms of gastroesophageal reflux disease (GERD) and esophageal motility. Via restrictive surgery, LAGB has been widely reported to cause significant weight loss, although studies have also shown an increase and worsening of GERD as well as elevated rates of esophageal dilation, aperistalsis, and alterations in lower esophageal sphincter pressure. Along with LAGB, LSG has shown not only a worsening of GERD, but also the formation of de novo GERD in patients who were asymptomatic before the operation. In a restrictive-malabsorptive approach, RYGB has been reported to improve GERD and preserve esophageal motility. Bariatric surgery is a burgeoning field with immense implications on overall mortality. Future randomized, controlled trials are needed to better understand which patients should undergo particular surgeries, with greater emphasis on esophageal health and prevention of GERD and esophageal dysmotility. PMID:27134597

  8. Data analyses and perspectives on laparoscopic surgery for esophageal achalasia.

    PubMed

    Tsuboi, Kazuto; Omura, Nobuo; Yano, Fumiaki; Hoshino, Masato; Yamamoto, Se-Ryung; Akimoto, Shunsuke; Masuda, Takahiro; Kashiwagi, Hideyuki; Yanaga, Katsuhiko

    2015-10-14

    In general, the treatment methods for esophageal achalasia are largely classified into four groups, including drug therapy using nitrite or a calcium channel blocker, botulinum toxin injection, endoscopic therapy such as endoscopic balloon dilation, and surgery. Various studies have suggested that the most effective treatment of esophageal achalasia is surgical therapy. The basic concept of this surgical therapy has not changed since Heller proposed esophageal myotomy for the purpose of resolution of lower esophageal obstruction for the first time in 1913, but the most common approach has changed from open-chest surgery to laparoscopic surgery. Currently, the laparoscopic surgery has been the procedure of choice for the treatment of esophageal achalasia. During the process of the transition from open-chest surgery to laparotomy, to thoracoscopic surgery, and to laparoscopic surgery, the necessity of combining antireflux surgery has been recognized. There is some debate as to which type of antireflux surgery should be selected. The Toupet fundoplication may be the most effective in prevention of postoperative antireflux, but many medical institutions have selected the Dor fundoplication which covers the mucosal surface exposed by myotomy. Recently, a new endoscopic approach, peroral endoscopic myotomy (POEM), has received attention. Future studies should examine the long-term outcomes and whether POEM becomes the gold standard for the treatment of esophageal achalasia. PMID:26478674

  9. [Structural and functional organization of the upper esophageal sphincter].

    PubMed

    Baĭtinger, V F; Saks, F F; Ettinger, A P

    1989-01-01

    Using traditional anatomical and histological methods, the muscle envelope of the pharynx-esophagus junction was investigated in humans and dogs. In the upper (cranial) portion of the esophagus of man and dogs, an inferior anatomical sphincter was detected which histologically can be referred to the group of rhabdo-sphincters. The upper esophageal sphincter is a purely esophageal structure which in man is located at a distance of 25-30 cm from the maxillary incisors. In adult humans, it is 25-30 mm long and is situated obliquely to the long esophageal axis. The posterior semicircle of the sphincter is located higher than the anterior one. In the area of the upper esophageal sphincter the esophageal wall is of different thickness. Due to the muscle envelope and submucous membrane of the base, the right wall is 1.7-2.0 times thicker than the left, anterior or posterior wall. The data obtained from fiber esophagoscopy of patients and electromyography of the pharynx-esophagus junction of dogs have shown that the upper (cranial) esophageal sphincter control food passage from the pharynx to the esophagus and prevents food reflux to the laryngopharynx, protecting airways from aspiration. PMID:2741289

  10. Effect of bolus composition on esophageal transit: concise communication

    SciTech Connect

    Fisher, R.S.; Malmud, L.S.; Appelgate, G.; Rock, E.; Lorber, S.H.

    1982-10-01

    The technique of esophageal scintigraphy was developed as a sensitive, quantitative, noninvasive test of esophageal transit. Esophageal scintigraphy was performed in 40 asymptomatic normal volunteers in order to determine the effect on esophageal transit of the following: body posture (sitting vs. supine), liquid vs. solid, the solid being either a standard gelatin capsule of the size used for antibiotic capsules, or a cube of solid food such as cooked chicken liver. The results showed that liquids emptied completely from the esophagus after one swallow whether supine or sitting. Capsules or liver cubes, when ingested without water, frequently remained in the esophagus for up to two hours without the subject's having any sensation that the solid had not left the esophagus. Both capsules and liver cubes cleared the esophagus better in the upright than in the supine position. When gelatin capsules were swallowed with as little as 15 ml of water, but after a preliminary sip of water, there was complete transit in each case. The study suggests that the practice of assisting patients into a sitting position and instructing them to take a sip of water before attempting to swallow a capsule will assure better transit of the capsule even when swallowed with as little as 15 ml of water. This may reduce the incidence of esophagitis following oral antibiotics, and of esophageal erosions from aspirin-containing medications.

  11. Effect of bolus composition on esophageal transit: concise communication

    SciTech Connect

    Fisher, R.S.; Malmud, L.S.; Applegate, G.; Rock, E.; Lorber, S.H.

    1982-10-01

    The technique of esophageal scintigraphy was developed as a sensitive, quantitative, noninvasive test of esophageal transit. Esophageal scintigraphy was performed in 40 asymptomatic normal volunteers in order to determine the effect on esophageal transit of the following: body posture (sitting vs. supine), liquid vs. solid, the solid being either a standard number4 gelatin capsule of the size used for antibiotic capsules, or a cube of solid food such as cooked chicken liver. The results showed that liquids emptied completely from the esophagus after one swallow, whether supine or sitting. Capsules or liver cubes, when ingested without water, frequently remained in the esophagus for up to two hours without the subject's having any sensation that the solid had not left the esophagus. Both capsules and liver cubes cleared the esophagus better in the upright than in the supine position. When gelatin capsules were swallowed with as little as 15 ml of water, but after a preliminary sip of water, there was complete transit in each case. The study suggests that the practice of assisting patients into a sitting position and instructing them to take a sip of water before attempting to swallow a capsule will assure better transit of the capsule even when swallowed with as little as 15 ml of water. This may reduce the incidence of esophagitis following oral antibiotics, and of esophageal erosions from aspirin-containing medications.

  12. [Congenital laryngo-tracheo-esophageal cleft].

    PubMed

    Sørensen, J A; Godballe, C; Jørgensen, K; Pedersen, S A

    1989-01-01

    A typical case of congenital laryngo-trachea-esophageal cleft (LTEC) is presented with a Review of the literature. LTEC is a rare congenital anomaly caused by defective fusion of the septum between larynx/trachea and hypopharynx/esophagus. The septum is formed by fusion of two lateral folds growing medially in very early foetal life. Fusion progresses in a cranial direction. Disturbances in septum formation result in LTEC. The disease gives respiratory problems with aspiration and excessive salivary production. The diagnosis is best made by intubating the larynx and examining the postcricoid region and anterior wall of the esophagus endoscopically. Stapling of the stomach, tracheostomy and secondary operative closure of the cleft has proved effective in the treatment of LTEC. PMID:2911891

  13. Advances in Radiotherapy Management of Esophageal Cancer

    PubMed Central

    Verma, Vivek; Moreno, Amy C.; Lin, Steven H.

    2016-01-01

    Radiation therapy (RT) as part of multidisciplinary oncologic care has been marked by profound advancements over the past decades. As part of multimodality therapy for esophageal cancer (EC), a prime goal of RT is to minimize not only treatment toxicities, but also postoperative complications and hospitalizations. Herein, discussion commences with the historical approaches to treating EC, including seminal trials supporting multimodality therapy. Subsequently, the impact of RT techniques, including three-dimensional conformal RT, intensity-modulated RT, and proton beam therapy, is examined through available data. We further discuss existing data and the potential for further development in the future, with an appraisal of the future outlook of technological advancements of RT for EC. PMID:27775643

  14. Thallium cardiac stressing by esophageal pacing

    SciTech Connect

    Allen, M.L.; Vacek, J.L.; Preston, D.F.; Robinson, R.G.; Feldkamp, M.J. )

    1989-09-01

    Forty-three patients were examined with the transesophageal pacing method of cardiac stressing and thallium imaging. Transesophageal cardiac pacing, using a pill electrode or a permanent pacemaker lead, is a safe alternative for patients who are physically unable to exercise. Prior studies suggest that transvenous right atrial pacing with thallium injection is equivalent to physical exercise thallium studies in the detection of coronary artery disease. The esophageal pacing bipolar electrode similarly increases heart rate without the necessity of transvenous pacing or fluoroscopy and without the adverse side effects often seen when using pharmacologic stressing agents (i.e., dipyridamole). The results compare well with cardiac catheterization, echocardiographic, and electrocardiographic results. Cardiac paced stress testing requires no sedation, is performed on an out-patient basis, and causes little if any discomfort for the patient.

  15. GWAS identifies four novel eosinophilic esophagitis loci.

    PubMed

    Sleiman, Patrick M A; Wang, Mei-Lun; Cianferoni, Antonella; Aceves, Seema; Gonsalves, Nirmala; Nadeau, Kari; Bredenoord, Albert J; Furuta, Glenn T; Spergel, Jonathan M; Hakonarson, Hakon

    2014-11-19

    Eosinophilic esophagitis (EoE) is an allergic disorder characterized by infiltration of the oesophagus with eosinophils. We had previously reported association of the TSLP/WDR36 locus with EoE. Here we report genome-wide significant associations at four additional loci; c11orf30 and STAT6, which have been previously associated with both atopic and autoimmune diseases, and two EoE-specific loci, ANKRD27 that regulates the trafficking of melanogenic enzymes to epidermal melanocytes and CAPN14, that encodes a calpain whose expression is highly enriched in the oesophagus. The identification of five EoE loci, not only expands our aetiological understanding of the disease but may also represent new therapeutic targets to treat the most debilitating aspect of EoE, oesophageal inflammation and remodelling.

  16. Lymphatic spreading and lymphadenectomy for esophageal carcinoma

    PubMed Central

    Ji, Xiang; Cai, Jie; Chen, Yao; Chen, Long-Qi

    2016-01-01

    Esophageal carcinoma (EC) is a highly lethal malignancy with a poor prognosis. One of the most important prognostic factors in EC is lymph node status. Therefore, lymphadenectomy has been recognized as a key that influences the outcome of surgical treatment for EC. However, the lymphatic drainage system of the esophagus, including an abundant lymph-capillary network in the lamina propria and muscularis mucosa, is very complex with cervical, mediastinal and celiac node spreading. The extent of lymphadenectomy for EC has always been controversial because of the very complex pattern of lymph node spreading. In this article, published literature regarding lymphatic spreading was reviewed and the current lymphadenectomy trends for EC are discussed. PMID:26843917

  17. Palliative Endoscopic Therapy of Esophageal Cancer

    PubMed Central

    Rabenstein, Thomas

    2015-01-01

    Summary Background This is a review of endoscopic therapy in the setting of palliative management of patients suffering from esophageal cancer (EC). Unfortunately, many cases of EC present in a stage of disease in which curative therapy is not possible. The maintenance of quality of life includes the ability to swallow and of oral feeding, pain control, and the prevention of bleeding. Methods A review of the current literature was performed. Results Many endoscopic methods are available for the management of dysphagia, of which dilation, endoluminal tumor destruction, stenting, and brachytherapy are the most common. Conclusion Surgical palliation should be avoided as much as possible since the alternatives show at least the same efficacy and have fewer complications. PMID:26989392

  18. Safrole-DNA adducts in tissues from esophageal cancer patients: clues to areca-related esophageal carcinogenesis.

    PubMed

    Lee, Jang-Ming; Liu, Tsung-Yung; Wu, Deng-Chyang; Tang, Hseau-Chung; Leh, Julie; Wu, Ming-Tsang; Hsu, Hsao-Hsun; Huang, Pei-Ming; Chen, Jin-Shing; Lee, Chun-Jean; Lee, Yung-Chie

    2005-01-01

    Epidemiological studies have demonstrated that areca quid chewing can be an independent risk factor for developing esophageal cancer. However, no studies are available to elucidate the mechanisms of how areca induces carcinogenesis in the esophagus. Since the areca nut in Taiwan contains a high concentration of safrole, a well-known carcinogenic agent, we analyzed safrole-DNA adducts by the 32P-postlabelling method in tissue specimens from esophageal cancer patients. In total, we evaluated 47 patients with esophageal cancer (16 areca chewers and 31 non-chewers) who underwent esophagectomy at the National Taiwan University Hospital between 1996 and 2002. Of the individuals with a history of habitual areca chewing (14 cigarette smokers and two non-smokers), one of the tumor tissue samples and five of the normal esophageal mucosa samples were positive for safrole-DNA adducts. All patients positive for safrole-DNA adducts were also cigarette smokers. Such adducts could not be found in patients who did not chew areca, irrespective of their habits of alcohol consumption or cigarette smoking (p<0.001, comparing the areca chewers with non-chewers). The genotoxicity of safrole was also tested in vitro in three esophageal cell lines and four cultures of primary esophageal keratinocytes. In two of the esophageal keratinocyte cultures, adduct formation was increased by treatment with safrole after induction of cytochrome P450 by 3-methyl-cholanthrene. This paper provides the first observation of how areca induces esophageal carcinogenesis, i.e., through the genotoxicity of safrole, a component of the areca juice.

  19. Di(2-ethylhexyl) phthalate inhibits antral follicle growth, induces atresia, and inhibits steroid hormone production in cultured mouse antral follicles

    PubMed Central

    Hannon, Patrick R.; Brannick, Katherine E.; Wang, Wei; Gupta, Rupesh K.; Flaws, Jodi A.

    2015-01-01

    Di(2-ethylhexyl) phthalate (DEHP) is a ubiquitous environmental toxicant found in consumer products that causes ovarian toxicity. Antral follicles are the functional ovarian units and must undergo growth, survival from atresia, and proper regulation of steroidogenesis to ovulate and produce hormones. Previous studies have determined that DEHP inhibits antral follicle growth and decreases estradiol levels in vitro; however, the mechanism by which DEHP elicits these effects is unknown. The present study tested the hypothesis that DEHP directly alters regulators of the cell cycle, apoptosis, and steroidogenesis to inhibit antral follicle functionality. Antral follicles from adult CD-1 mice were cultured with vehicle control or DEHP (1-100μg/ml) for 24-96 hr to establish the temporal effects of DEHP on the follicle. Following 24-96 hr of culture, antral follicles were subjected to gene expression analysis, and media were subjected to measurements of hormone levels. DEHP increased the mRNA levels of cyclin D2, cyclin dependent kinase 4, cyclin E1, cyclin A2, and cyclin B1 and decreased the levels of cyclin-dependent kinase inhibitor 1A prior to growth inhibition. Additionally, DEHP increased the mRNA levels of BCL2-associated agonist of cell death, BCL2-associated X protein, BCL2-related ovarian killer protein, B-cell leukemia/lymphoma 2, and Bcl2-like 10, leading to an increase in atresia. Further, DEHP decreased the levels of progesterone, androstenedione, and testosterone prior to the decrease in estradiol levels, with decreased mRNA levels of side-chain cleavage, 17α-hydorxylase-17,20-desmolase, 17β-hydroxysteroid dehydrogenase, and aromatase. Collectively, DEHP directly alters antral follicle functionality by inhibiting growth, inducing atresia, and inhibiting steroidogenesis. PMID:25701202

  20. Neonatal diabetes, gallbladder agenesis, duodenal atresia, and intestinal malrotation caused by a novel homozygous mutation in RFX6.

    PubMed

    Concepcion, Jennifer P; Reh, Christina S; Daniels, Mark; Liu, Xiaoming; Paz, Veronica P; Ye, Honggang; Highland, Heather M; Hanis, Craig L; Greeley, Siri Atma W

    2014-02-01

    Recently, bi-allelic mutations in the transcription factor RFX6 were described as the cause of a rare condition characterized by neonatal diabetes with pancreatic and biliary hypoplasia and duodenal/jejunal atresia. A male infant developed severe hyperglycemia (446 mg/dL) within 24 h of birth. Acute abdominal concerns by day five necessitated exploratory surgery that revealed duodenal atresia, gallbladder agenesis, annular pancreas and intestinal malrotation. He also exhibited chronic diarrhea and feeding intolerance, cholestatic jaundice, and subsequent liver failure. He died of sepsis at four months old while awaiting liver transplantation. The phenotype of neonatal diabetes with intestinal atresia and biliary agenesis clearly pointed to RFX6 as the causative gene; indeed, whole exome sequencing revealed a novel homozygous RFX6 mutation c.779A>C; p.Lys260Thr (K260T). This missense mutation also changes the consensus 5' splice donor site before intron 7 and is thus predicted to cause disruption in splicing. Both parents, who were not known to be related, were heterozygous carriers. Targeted genetic testing based on consideration of phenotypic features may reveal a cause among the many genes now associated with heterogeneous forms of monogenic neonatal diabetes. Our study demonstrates the feasibility of using modern sequencing technology to identify one such rare cause. Continued research is needed to determine the possible cost-effectiveness of this approach, especially when clear phenotypic clues are absent. Further study of patients with RFX6 mutations should clarify its role in pancreatic, intestinal and enteroendocrine cellular development and explain features such as the diarrhea exhibited in our case.

  1. The inflammatory phenotype of the fibrous plate is distinct from the liver and correlates with clinical outcome in biliary atresia.

    PubMed

    Arva, Nicoleta C; Russo, Pierre A; Erlichman, Jessi; Hancock, Wayne W; Haber, Barbara A; Bhatti, Tricia R

    2015-03-01

    Biliary atresia is an inflammatory cholangiopathy of still undetermined etiology. Correlations between histologic findings and clinical outcome in this disease have largely been based on evaluation of liver parenchyma. This study aimed to characterize the pattern of inflammation within the biliary remnant and identify associations between the type and degree of inflammation and clinical outcome as reflected by the transplant-free interval. The inflammation within the fibrous plates and livers of 41 patients with biliary atresia was characterized using immunohistochemical markers and the cell populations were digitally quantified. The type and quantity of cells within the infiltrate were then correlated with length of time from Kasai portoenterostomy until transplant. Histologic and immunohistochemical features of the biliary remnant allowed stratification of patients into "inflammatory plate" and "fibrotic plate" groups. Overall there was no significant difference in transplant-free interval between the two cohorts; however, there was a trend towards a longer time to transplant among patients in the "fibrotic plate" group. In addition, the composition of the inflammatory infiltrate in the fibrous plate was distinctly different from that present in the liver and only the characteristics of the inflammation in the fibrous plate, in particular the number of Foxp3+ T regulatory lymphocytes correlated with clinical outcome. The results of this study support the view of the extra-hepatic biliary tree as the primary site of injury in BA with the changes seen in the liver as secondary manifestations of outflow obstruction. The association between specific inflammatory cell subtypes within the fibrous plate and the length of transplant-free interval also supports the role of the immune system in the initial process of bile duct damage in biliary atresia.

  2. The inflammatory phenotype of the fibrous plate is distinct from the liver and correlates with clinical outcome in biliary atresia.

    PubMed

    Arva, Nicoleta C; Russo, Pierre A; Erlichman, Jessi; Hancock, Wayne W; Haber, Barbara A; Bhatti, Tricia R

    2015-03-01

    Biliary atresia is an inflammatory cholangiopathy of still undetermined etiology. Correlations between histologic findings and clinical outcome in this disease have largely been based on evaluation of liver parenchyma. This study aimed to characterize the pattern of inflammation within the biliary remnant and identify associations between the type and degree of inflammation and clinical outcome as reflected by the transplant-free interval. The inflammation within the fibrous plates and livers of 41 patients with biliary atresia was characterized using immunohistochemical markers and the cell populations were digitally quantified. The type and quantity of cells within the infiltrate were then correlated with length of time from Kasai portoenterostomy until transplant. Histologic and immunohistochemical features of the biliary remnant allowed stratification of patients into "inflammatory plate" and "fibrotic plate" groups. Overall there was no significant difference in transplant-free interval between the two cohorts; however, there was a trend towards a longer time to transplant among patients in the "fibrotic plate" group. In addition, the composition of the inflammatory infiltrate in the fibrous plate was distinctly different from that present in the liver and only the characteristics of the inflammation in the fibrous plate, in particular the number of Foxp3+ T regulatory lymphocytes correlated with clinical outcome. The results of this study support the view of the extra-hepatic biliary tree as the primary site of injury in BA with the changes seen in the liver as secondary manifestations of outflow obstruction. The association between specific inflammatory cell subtypes within the fibrous plate and the length of transplant-free interval also supports the role of the immune system in the initial process of bile duct damage in biliary atresia. PMID:25624184

  3. The association between reflux esophagitis and airway hyper-reactivity in patients with gastro-esophageal reflux

    PubMed Central

    Karbasi, Ashraf; Ardestani, Mohammad Emami; Ghanei, Mostafa; Harandi, Ali Amini

    2013-01-01

    Background: The association of gastro-esophageal reflux (GER) with a wide variety of pulmonary disorders was recognized. We aimed to evaluate the effect of GER-induced esophagitis on airway hyper-reactivity (AHR) in patients and the response to treatment. Materials and Methods: In this cohort study, 30 patients attending the gastrointestinal clinic of a university hospital with acid reflux symptoms were included. All patients were evaluated endoscopically and divided into case group with esophagitis and control group without any evidence of esophagitis. Spirometry and methacholine test were done in all patients before and after treatment of GER with pantoprazole 40 mg daily for six months. Results: There was a significant difference in the rate of positive methacholine test between the cases (40%) and the controls (6.7%) prior to anti-acid therapy (P < 0.0001). After six months of treatment, the frequency of positive methacholine test diminished from 40 to 13.3% in the case group (P < 0.05) but did not change in the controls (P = 0.15). Conclusion: The presence of esophagitis due to GER would increase the AHR and treatment with pantoperazole would decrease AHR in patients with proved esophagitis and no previous history of asthma after six months. PMID:24250694

  4. Single primitive ventricle with normally related great arteries and atresia of the left A-V valve.

    PubMed Central

    Coto, E O; Raggio, J M; Malo, P; Sainz, C; Aparisi, R; Gomez-Ullate, J M

    1978-01-01

    A child aged 2 years and 9 months was angiocardiographically diagnosed to have a single ventricle with normally related great arteries and atresia of the left A-V valve. A Blalock-Hanlon procedure and division of a large patent ductus arteriosus were followed by reduction in pulmonary artery pressure, but after operation the patient showed signs of left ventricular failure unresponsive to medical treatment, necessitating pulmonary artery banding. We have found only three similar published cases, and this is the only one with full angiographic documentation. Images PMID:725830

  5. Evidence From Human and Zebrafish That GPC1 Is a Biliary Atresia Susceptibility Gene

    PubMed Central

    Cui, Shuang; Leyva-Vega, Melissa; Tsai, Ellen A.; Eauclaire, Steven F.; Glessner, Joseph T.; Hakonarson, Hakon; Devoto, Marcella; Haber, Barbara A.; Spinner, Nancy B.; Matthews, Randolph P.

    2013-01-01

    BACKGROUND & AIMS Biliary atresia (BA) is a progressive fibroinflammatory disorder of infants involving the extrahepatic and intrahepatic biliary tree. Its etiology is unclear but is believed to involve exposure of a genetically susceptible individual to certain environmental factors. BA occurs exclusively in the neonatal liver, so variants of genes expressed during hepatobiliary development could affect susceptibility. Genome-wide association studies previously identified a potential region of interest at 2q37. We continued these studies to narrow the region and identify BA susceptibility genes. METHODS We searched for copy number variants that were increased among patients with BA (n = 61) compared with healthy individuals (controls; n = 5088). After identifying a candidate gene, we investigated expression patterns of orthologues in zebrafish liver and the effects of reducing expression, with morpholino antisense oligonucleotides, on biliary development, gene expression, and signal transduction. RESULTS We observed a statistically significant increase in deletions at 2q37.3 in patients with BA that resulted in deletion of one copy of GPC1, which encodes glypican 1, a heparan sulfate proteoglycan that regulates Hedgehog signaling and inflammation. Knockdown of gpc1 in zebrafish led to developmental biliary defects. Exposure of the gpc1 morphants to cyclopamine, a Hedgehog antagonist, partially rescued the gpc1-knockdown phenotype. Injection of zebrafish with recombinant Sonic Hedgehog led to biliary defects similar to those of the gpc1 morphants. Liver samples from patients with BA had reduced levels of apical GPC1 in cholangiocytes compared with samples from controls. CONCLUSIONS Based on genetic analysis of patients with BA and zebrafish, GPC1 appears to be a BA susceptibility gene. These findings also support a role for Hedgehog signaling in the pathogenesis of BA. PMID:23336978

  6. Expression of the interferon-induced Mx proteins in biliary atresia.

    PubMed

    Al-Masri, Abdul Nasser; Flemming, Peer; Rodeck, Burkhard; Melter, Michael; Leonhardt, Johannes; Petersen, Claus

    2006-06-01

    Biliary atresia (BA) is a rare disease of the newborn for which the Kasai procedure is curative only for a few of the patients. The dilemma is that all therapeutic attempts to cure the disease are symptomatic because the etiology is still unclear. One theory suggests a progressive inflammatory process, possibly induced by a viral infection. The aim of the present study was to investigate the activity of type I interferons (IFNs) in the livers of patients with BA. Mx proteins, which mediate an early innate immune response, are a very sensitive marker for type I IFN activity (eg, to viral infection). Liver biopsies were taken during the Kasai procedure from 13 newborns with BA who were serologically negative for hepatotropic viruses. Age-matched controls originated from 7 patients with neonatal cholestasis (eg, inspissated bile syndrome), 3 aborted fetuses, and a 10-year-old child. The immunostaining procedure (alkaline phosphatase anti-alkaline phosphatase) was performed with Mx-specific monoclonal antibody. Immunostaining for Mx proteins was positive in the hepatocytes of all newborns with BA, whereas the intrahepatic bile ducts were positive in all but one. In the control group, 8 of 11 liver samples were Mx-negative. This is the first study dealing with the detection of type I IFN activity in the liver of patients with BA. This observation supports the etiologic consideration of type I IFN-mediated immune response. Although positive findings of viruses in patients with BA are still inconsistent, the present study retraces the progressive inflammatory process in BA one more step toward its beginning.

  7. Global methylation, oxidative stress, and relative telomere length in biliary atresia patients

    PubMed Central

    Udomsinprasert, Wanvisa; Kitkumthorn, Nakarin; Mutirangura, Apiwat; Chongsrisawat, Voranush; Poovorawan, Yong; Honsawek, Sittisak

    2016-01-01

    Alu and LINE-1 elements are retrotransposons with a ubiquitous presence in the human genome that can cause genomic instability, specifically relating to telomere length. Genotoxic agents may induce methylation of retrotransposons, in addition to oxidative DNA damage in the form of 8-hydroxy-2′-deoxyguanosine (8-OHdG). Methylation of retrotransposons induced by these agents may contribute to biliary atresia (BA) etiology. Here, we investigated correlations between global methylation, 8-OHdG, and relative telomere length, as well as reporting on Alu and LINE-1 hypomethylation in BA patients. Alu and LINE-1 hypomethylation were found to be associated with elevated risk of BA (OR = 4.07; 95% CI: 2.27–7.32; P < 0.0001 and OR = 3.51; 95% CI: 1.87–6.59; P < 0.0001, respectively). Furthermore, LINE-1 methylation was associated with liver stiffness in BA patients (β coefficient = −0.17; 95% CI: −0.24 to −0.10; P < 0.0001). Stratified analysis revealed negative correlations between Alu and LINE-1 methylation and 8-OHdG in BA patients (P < 0.0001). In contrast, positive relationships were identified between Alu and LINE-1 methylation and relative telomere length in BA patients (P < 0.0001). These findings suggest that retrotransposon hypomethylation is associated with plasma 8-OHdG and telomere length in BA patients. PMID:27243754

  8. Supersonic shearwave elastography in the assessment of liver fibrosis for postoperative patients with biliary atresia

    PubMed Central

    Chen, Shuling; Liao, Bing; Zhong, Zhihai; Zheng, Yanling; Liu, Baoxian; Shan, Quanyuan; Xie, Xiaoyan; Zhou, Luyao

    2016-01-01

    To explore an effective noninvasive tool for monitoring liver fibrosis of children with biliary atresia (BA) is important but evidences are limited. This study is to investigate the predictive accuracy of supersonic shearwave elastography (SSWE) in liver fibrosis for postoperative patients with BA and to compare it with aspartate aminotransferase to platelet ratio index (APRI) and fibrosis-4 (FIB-4). 24 patients with BA received SSWE and laboratory tests before scheduled for liver biopsy. Spearman rank coefficient and receiver operating characteristic (ROC) were used to analyze data. Metavir scores were F0 in 3, F1 in 2, F2 in 4, F3 in 7 and F4 in 8 patients. FIB-4 failed to correlate with fibrosis stage. The areas under the ROC curves of SSWE, APRI and their combination were 0.79, 0.65 and 0.78 for significant fibrosis, 0.81, 0.64 and 0.76 for advanced fibrosis, 0.82, 0.56 and 0.84 for cirrhosis. SSWE values at biopsy was correlated with platelet count (r = −0.426, P = 0.038), serum albumin (r = −0.670, P < 0.001), total bilirubin (r = 0.419, P = 0.041) and direct bilirubin levels (r = 0.518, P = 0.010) measured at 6 months after liver biopsy. Our results indicate that SSWE is a more promising tool to assess liver fibrosis than APRI and FIB-4 in children with BA. PMID:27511435

  9. Thoracic Discitis as a Complication of Self-Expanding Metallic Stents in Esophageal Carcinoma

    SciTech Connect

    McQueen, A. S.; Eljabu, W.; Latimer, J. Raju, P. P. J.

    2011-02-15

    The role of metallic stents in the palliation of esophageal cancer is well established. Self-expanding metal stents (SEMSs) are frequently used, as they provide an effective and safe method of relieving malignant dysphagia. A number of complications are associated with the use of SEMSs, including esophageal perforation. We report a case of thoracic discitis occurring in a patient with advanced esophageal malignancy, treated with SEMSs. We propose that the likely etiology in this patient was esophageal perforation by a metallic stent.

  10. Nondestructive measurement of esophageal biaxial mechanical properties utilizing sonometry

    NASA Astrophysics Data System (ADS)

    Aho, Johnathon M.; Qiang, Bo; Wigle, Dennis A.; Tschumperlin, Daniel J.; Urban, Matthew W.

    2016-07-01

    Malignant esophageal pathology typically requires resection of the esophagus and reconstruction to restore foregut continuity. Reconstruction options are limited and morbid. The esophagus represents a useful target for tissue engineering strategies based on relative simplicity in comparison to other organs. The ideal tissue engineered conduit would have sufficient and ideally matched mechanical tolerances to native esophageal tissue. Current methods for mechanical testing of esophageal tissues both in vivo and ex vivo are typically destructive, alter tissue conformation, ignore anisotropy, or are not able to be performed in fluid media. The aim of this study was to investigate biomechanical properties of swine esophageal tissues through nondestructive testing utilizing sonometry ex vivo. This method allows for biomechanical determination of tissue properties, particularly longitudinal and circumferential moduli and strain energy functions. The relative contribution of mucosal-submucosal layers and muscular layers are compared to composite esophagi. Swine thoracic esophageal tissues (n  =  15) were tested by pressure loading using a continuous pressure pump system to generate stress. Preconditioning of tissue was performed by pressure loading with the pump system and pre-straining the tissue to in vivo length before data was recorded. Sonometry using piezocrystals was utilized to determine longitudinal and circumferential strain on five composite esophagi. Similarly, five mucosa-submucosal and five muscular layers from thoracic esophagi were tested independently. This work on esophageal tissues is consistent with reported uniaxial and biaxial mechanical testing and reported results using strain energy theory and also provides high resolution displacements, preserves native architectural structure and allows assessment of biomechanical properties in fluid media. This method may be of use to characterize mechanical properties of tissue engineered esophageal

  11. Nondestructive measurement of esophageal biaxial mechanical properties utilizing sonometry

    NASA Astrophysics Data System (ADS)

    Aho, Johnathon M.; Qiang, Bo; Wigle, Dennis A.; Tschumperlin, Daniel J.; Urban, Matthew W.

    2016-07-01

    Malignant esophageal pathology typically requires resection of the esophagus and reconstruction to restore foregut continuity. Reconstruction options are limited and morbid. The esophagus represents a useful target for tissue engineering strategies based on relative simplicity in comparison to other organs. The ideal tissue engineered conduit would have sufficient and ideally matched mechanical tolerances to native esophageal tissue. Current methods for mechanical testing of esophageal tissues both in vivo and ex vivo are typically destructive, alter tissue conformation, ignore anisotropy, or are not able to be performed in fluid media. The aim of this study was to investigate biomechanical properties of swine esophageal tissues through nondestructive testing utilizing sonometry ex vivo. This method allows for biomechanical determination of tissue properties, particularly longitudinal and circumferential moduli and strain energy functions. The relative contribution of mucosal–submucosal layers and muscular layers are compared to composite esophagi. Swine thoracic esophageal tissues (n  =  15) were tested by pressure loading using a continuous pressure pump system to generate stress. Preconditioning of tissue was performed by pressure loading with the pump system and pre-straining the tissue to in vivo length before data was recorded. Sonometry using piezocrystals was utilized to determine longitudinal and circumferential strain on five composite esophagi. Similarly, five mucosa–submucosal and five muscular layers from thoracic esophagi were tested independently. This work on esophageal tissues is consistent with reported uniaxial and biaxial mechanical testing and reported results using strain energy theory and also provides high resolution displacements, preserves native architectural structure and allows assessment of biomechanical properties in fluid media. This method may be of use to characterize mechanical properties of tissue engineered

  12. Role of robotic-assisted surgery in benign esophageal diseases.

    PubMed

    Saurabh, Shireesh; Unger, Eric; Grossman, Julie; Couto, Francisco; Singh, Namrata; Lind, David Scott; Panait, Lucian; Castellanos, Andres

    2014-06-01

    Laparoscopic treatment of benign esophageal conditions is technically complex with several inherent limitations. Robotic-assisted surgery provides technical improvement and helps to overcome some of these limitations. We therefore report a single surgeon's experience in management of benign esophageal diseases by robotic-assisted surgery. Over a period of 8 consecutive years, a retrospective chart review was performed of 105 patients who underwent robotic-assisted surgery for benign esophageal diseases by a single surgeon. Demographic data and outcome measures were studied. The robotic-assisted procedures included 85 Nissen fundoplications with and without mesh repair, 12 Heller myotomies and eight para-esophageal hernia repairs. The mean total operating time was lowest for the Nissen group (94 min) and highest for the para-esophageal group (183 min). Operating time decreased from a mean of 105 min in the first 20 cases to 84 min in the last 20 cases for the Nissen group (P = 0.014). The mean length of stay was 1.3, 1.6, 1.5 and 4.8 days for the groups, respectively. Persistent symptoms of dysphagia/reflux/dysphonia requiring further investigation were seen in nine (8 %) of these patients. Two of these patients required repeat Nissen fundoplication in the mesh group. Our complication rate, total operating time and length of stay for robotic-assisted benign esophageal surgery are comparable to those reported in the literature. When performed by an experienced surgeon, robotic-assisted surgery is safe and effective in the management of benign esophageal diseases.

  13. Nondestructive measurement of esophageal biaxial mechanical properties utilizing sonometry.

    PubMed

    Aho, Johnathon M; Qiang, Bo; Wigle, Dennis A; Tschumperlin, Daniel J; Urban, Matthew W

    2016-07-01

    Malignant esophageal pathology typically requires resection of the esophagus and reconstruction to restore foregut continuity. Reconstruction options are limited and morbid. The esophagus represents a useful target for tissue engineering strategies based on relative simplicity in comparison to other organs. The ideal tissue engineered conduit would have sufficient and ideally matched mechanical tolerances to native esophageal tissue. Current methods for mechanical testing of esophageal tissues both in vivo and ex vivo are typically destructive, alter tissue conformation, ignore anisotropy, or are not able to be performed in fluid media. The aim of this study was to investigate biomechanical properties of swine esophageal tissues through nondestructive testing utilizing sonometry ex vivo. This method allows for biomechanical determination of tissue properties, particularly longitudinal and circumferential moduli and strain energy functions. The relative contribution of mucosal-submucosal layers and muscular layers are compared to composite esophagi. Swine thoracic esophageal tissues (n  =  15) were tested by pressure loading using a continuous pressure pump system to generate stress. Preconditioning of tissue was performed by pressure loading with the pump system and pre-straining the tissue to in vivo length before data was recorded. Sonometry using piezocrystals was utilized to determine longitudinal and circumferential strain on five composite esophagi. Similarly, five mucosa-submucosal and five muscular layers from thoracic esophagi were tested independently. This work on esophageal tissues is consistent with reported uniaxial and biaxial mechanical testing and reported results using strain energy theory and also provides high resolution displacements, preserves native architectural structure and allows assessment of biomechanical properties in fluid media. This method may be of use to characterize mechanical properties of tissue engineered esophageal

  14. Does Dysphagia Indicate Recurrence of Benign Esophageal Strictures?

    PubMed Central

    Borgström, Anders; Fork, Frans-Thomas; Lövdahl, Eje

    1995-01-01

    Esophageal dilatation in dysphagic patients with benign strictures is usually considered successful if the patients' dysphagia is alleviated. However, the relation between dysphagia and the diameter of a stricture is not well understood. Moreover, the dysphagia may also be caused by an underlying esophageal motor disorder. In order to compare symptoms and objective measurements of esophageal stricture, 28 patients were studied with interview and a radiologic esophagram. The latter included swallowing of a solid bolus. All patients underwent successful balloon dilatation at least one month prior to this study. Recurrence of a stricture with a diameter of less than 13 mm was diagnosed by the barium swallow in 21 patients. Recurrence of dysphagia was seen in 15 patients. Thirteen patients denied any swallowing symptoms. Chest pain was present in 9 patients. Of 15 patients with dysphagia 2 (13%) had no narrowing but severe esophageal dysmotility. Of 13 patients without dysphagia 9 (69%) had a stricture with a diameter of 13 mm or less. Of 21 patients with a stricture of 13 mm or less 14 (67%) were symptomatic while 7 (33%) were asymptomatic. Four of 11 patients with retrosternal pain had a stricture of less than 10 mm. Three patients with retrosternal pain and obstruction had severe esophageal dysmotility. Whether or not the patients have dysphagia may be more related to diet and eating habits than to the true diameter of their esophageal narrowing. We conclude that the clinical history is non-reliable for evaluating the results of esophageal stricture dilatation. In order to get an objective measurement of therapeutic outcome, barium swallow including a solid bolus is recommended. PMID:18493375

  15. Preferential Secretion of Thymic Stromal Lymphopoietin (TSLP) by Terminally Differentiated Esophageal Epithelial Cells: Relevance to Eosinophilic Esophagitis (EoE).

    PubMed

    Chandramouleeswaran, Prasanna M; Shen, Dawen; Lee, Anna J; Benitez, Alain; Dods, Kara; Gambanga, Fiona; Wilkins, Benjamin J; Merves, Jamie; Noah, Yuliana; Toltzis, Sarit; Yearley, Jennifer H; Spergel, Jonathan M; Nakagawa, Hiroshi; Malefyt, Rene deWaal; Muir, Amanda B; Wang, Mei-Lun

    2016-01-01

    Eosinophilic esophagitis (EoE) is a chronic Th2 and food antigen-mediated disease characterized by esophageal eosinophilic infiltration. Thymic stromal lymphopoetin (TSLP), an epithelial derived cytokine which bridges innate and Th2-type adaptive immune responses in other allergic conditions, is overexpressed in esophageal biopsies of EoE subjects. However, the triggers of TSLP expression in the esophageal epithelium are unknown. The objective of the current study was to characterize TSLP expression in human esophageal epithelium in EoE in vivo and to determine the role of food antigens upon epithelial TSLP expression in vitro. Using immunohistochemistry (IHC), we localized TSLP in esophageal biopsies of active EoE (≥15 eos/hpf), inactive EoE (<15 eos/hpf) and non-EoE control subjects, and found that TSLP expression was restricted to the differentiated suprabasal layer of the epithelium in actively inflamed EoE biopsies. Consistent with these results in vivo, inducible TSLP protein secretion was higher in CaCl2 differentiated telomerase-immortalized esophageal epithelial cells (EPC2-hTERT) compared to undifferentiated cells of the basal phenotype, following stimulation with the TLR3 ligand poly(I:C). To determine whether food antigens could directly induce epithelial TSLP secretion, differentiated and undifferentiated primary esophageal epithelial cells from EoE and non-EoE subjects were challenged with food antigens clinically relevant to EoE: Chicken egg ovalbumin (OVA), wheat, and milk proteins beta-lactoglobulin (blg) and beta-casein. Food antigens failed to induce TSLP secretion by undifferentiated cells; in contrast, only OVA induced TSLP secretion in differentiated epithelial cells from both EoE and control cell lines, an effect abolished by budesonide and NF-κb inhibition. Together, our study shows that specific food antigens can trigger innate immune mediated esophageal TSLP secretion, suggesting that esophageal epithelial cells at the barrier surface

  16. Preferential Secretion of Thymic Stromal Lymphopoietin (TSLP) by Terminally Differentiated Esophageal Epithelial Cells: Relevance to Eosinophilic Esophagitis (EoE)

    PubMed Central

    Chandramouleeswaran, Prasanna M.; Shen, Dawen; Lee, Anna J.; Benitez, Alain; Dods, Kara; Gambanga, Fiona; Wilkins, Benjamin J.; Merves, Jamie; Noah, Yuliana; Toltzis, Sarit; Yearley, Jennifer H.; Spergel, Jonathan M.; Nakagawa, Hiroshi; Malefyt, Rene deWaal; Muir, Amanda B.; Wang, Mei-Lun

    2016-01-01

    Eosinophilic esophagitis (EoE) is a chronic Th2 and food antigen-mediated disease characterized by esophageal eosinophilic infiltration. Thymic stromal lymphopoetin (TSLP), an epithelial derived cytokine which bridges innate and Th2-type adaptive immune responses in other allergic conditions, is overexpressed in esophageal biopsies of EoE subjects. However, the triggers of TSLP expression in the esophageal epithelium are unknown. The objective of the current study was to characterize TSLP expression in human esophageal epithelium in EoE in vivo and to determine the role of food antigens upon epithelial TSLP expression in vitro. Using immunohistochemistry (IHC), we localized TSLP in esophageal biopsies of active EoE (≥15 eos/hpf), inactive EoE (<15 eos/hpf) and non-EoE control subjects, and found that TSLP expression was restricted to the differentiated suprabasal layer of the epithelium in actively inflamed EoE biopsies. Consistent with these results in vivo, inducible TSLP protein secretion was higher in CaCl2 differentiated telomerase-immortalized esophageal epithelial cells (EPC2-hTERT) compared to undifferentiated cells of the basal phenotype, following stimulation with the TLR3 ligand poly(I:C). To determine whether food antigens could directly induce epithelial TSLP secretion, differentiated and undifferentiated primary esophageal epithelial cells from EoE and non-EoE subjects were challenged with food antigens clinically relevant to EoE: Chicken egg ovalbumin (OVA), wheat, and milk proteins beta-lactoglobulin (blg) and beta-casein. Food antigens failed to induce TSLP secretion by undifferentiated cells; in contrast, only OVA induced TSLP secretion in differentiated epithelial cells from both EoE and control cell lines, an effect abolished by budesonide and NF-κb inhibition. Together, our study shows that specific food antigens can trigger innate immune mediated esophageal TSLP secretion, suggesting that esophageal epithelial cells at the barrier surface

  17. Esophageal reflexes modulate frontoparietal response in neonates: Novel application of concurrent NIRS and provocative esophageal manometry.

    PubMed

    Jadcherla, Sudarshan R; Pakiraih, Joanna F; Hasenstab, Kathryn A; Dar, Irfaan; Gao, Xiaoyu; Bates, D Gregory; Kashou, Nasser H

    2014-07-01

    Central and peripheral neural regulation of swallowing and aerodigestive reflexes is unclear in human neonates. Functional near infrared spectroscopy (NIRS) is a noninvasive method to measure changes in oxyhemoglobin (HbO) and deoxyhemoglobin (HbD). Pharyngoesophageal manometry permits evaluation of aerodigestive reflexes. Modalities were combined to investigate feasibility and to test neonatal frontoparietal cortical changes during pharyngoesophageal (visceral) stimulation and/or swallowing. Ten neonates (45.6 ± 3.0 wk postmenstrual age, 4.1 ± 0.5 kg) underwent novel pharyngoesophageal manometry concurrent with NIRS. To examine esophagus-brain interactions, we analyzed cortical hemodynamic response (HDR) latency and durations during aerodigestive provocation and esophageal reflexes. Data are presented as means ± SE or percent. HDR rates were 8.84 times more likely with basal spontaneous deglutition compared with sham stimuli (P = 0.004). Of 182 visceral stimuli, 95% were analyzable for esophageal responses, 38% for HDR, and 36% for both. Of analyzable HDR (n = 70): 1) HbO concentration (μmol/l) baseline 1.5 ± 0.7 vs. 3.7 ± 0.7 poststimulus was significant (P = 0.02), 2) HbD concentration (μmol/l) between baseline 0.1 ± 0.4 vs. poststimulus -0.5 ± 0.4 was not significant (P = 0.73), and 3) hemispheric lateralization was 21% left only, 29% right only, and 50% bilateral. During concurrent esophageal and NIRS responses (n = 66): 1) peristaltic reflexes were present in 74% and HDR in 61% and 2) HDR was 4.75 times more likely with deglutition reflex vs. secondary peristaltic reflex (P = 0.016). Concurrent NIRS with visceral stimulation is feasible in neonates, and frontoparietal cortical activation is recognized. Deglutition contrasting with secondary peristalsis is related to cortical activation, thus implicating higher hierarchical aerodigestive protective functional neural networks. PMID:24789204

  18. Videothoracoscopic management of middle esophageal diverticulum with secondary bronchoesophageal fistula: report of a case.

    PubMed

    Braghetto, Italo; Cardemil, Gonzalo; Schwartz, Eitan; Valladares, Hector; Rencoret, Guillermo; Estay, Rene; Rodriguez-Navarro, Alberto J

    2008-01-01

    Middle esophageal diverticulum is rare, but can result in bronchoesophageal fistula. Previous reports have described open surgical techniques to treat esophageal diverticula, but few have evaluated the effectiveness of a videothoracoscopy approach. We report a case of middle esophageal diverticulum associated with bronchoesophageal fistula, managed successfully with videothoracoscopy. We also review the relevant literature.

  19. Intersex and oocyte atresia in a mussel population from the Biosphere's Reserve of Urdaibai (Bay of Biscay).

    PubMed

    Ortiz-Zarragoitia, Maren; Cajaraville, Miren P

    2010-07-01

    Urdaibai is a Biosphere's Reserve, containing rich bivalve populations. In the present work we aimed to characterize the reproductive cycle and to study possible disturbances in reproduction in mussel populations from Urdaibai. During an annual gametogenic cycle (January 2003-March 2004) samples of gonads were collected bimonthly and gamete development (gonad index), histopathology of mantle tissue and vitellogenin-like protein levels by alkali labile phosphate (ALP) method were measured. No significant changes were detected in ALP levels out of those related to gametogenic development in females. However, severe oocyte atresia was observed in female mussels in January and March 2003. Male mussels showed low ALP values with the exception of high ALP levels in March 2003. High prevalence of hermaphrodite/intersex mussels (26%) was detected in March 2004. Hermaphrodites/intersex and oocyte atresia were not found in mussels from the nearby Abra estuary. In conclusion, these results suggest that mussels inhabiting the Urdaibai estuary could be exposed to toxic chemicals such as endocrine disruptors.

  20. Potential Pitfalls on the (99m)Tc-Mebrofenin Hepatobiliary Scintigraphy in a Patient with Biliary Atresia Splenic Malformation Syndrome.

    PubMed

    Maestri Brittain, Jane; Borgwardt, Lise

    2016-01-01

    Biliary atresia (BA) is an obliterative cholangiopathy affecting 1:10.000-14.000 of newborns. Infants with Biliary Atresia Splenic Malformation syndrome (BASM) are a subgroup of BA patients with additional congenital anomalies. Untreated the disease will result in fatal liver failure within the first years of life. Kasai portoenterostomy restores bile flow and delay the progressive liver damage thereby postponing liver transplantation. An early diagnosis is of most importance to ensure the effectiveness of the operation. The (99m)Tc-Mebrofenin hepatobiliary scintigraphy is part of the diagnostic strategy when an infant presents jaundice due to conjugated hyperbilirubinemia (>20 µmol/L total bilirubin of which 20% is conjugated) with its high sensitivity of 97%-100% in refuting BA. Rapid extraction of tracer by the liver and no visible tracer in the small bowl after 24 h is indicative of BA. Laparotomy with antegrade cholangiography is then performed giving the final diagnosis when the remains of the obliterated biliary tree are revealed in the case of BA. We present a case demonstrating some of the challenges of interpreting the (99m)Tc-Mebrofenin hepatobiliary scintigraphy in an infant with BASM and stress the importance that the (99m)Tc-Mebrofenin hepatobiliary scintigraphy is part of a spectrum of imaging modalities in diagnosing BA. PMID:26838802