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Sample records for anomalies anal atresia

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

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    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

  2. A study of associated congenital anomalies with biliary atresia

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    Lucky Gupta

    2016-01-01

    Full Text Available 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 3 rd 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.

  3. MULTIPLE ASSOCIATED ANOMALIES IN PATIENTS OF DUODENAL ATRESIA: A CASE SERIES

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    Bilal Mirza

    2012-04-01

    Full Text Available Duodenal atresia has been reported in association with various malformations and syndromes common being Down syndrome, malrotation, and annular pancreas. Its association with multiple anomalies is rare and scarcely reported in literature. Herein 3 cases of duodenal atresia associated with multiple congenital anomalies are being reported.

  4. [Epithelium and anal glands in rectal pouches and fistula. Histologic studies of swine with congenital anal atresia].

    Science.gov (United States)

    Lambrecht, W; Kluth, D; Lierse, W

    1989-02-01

    The epithelial coating of the rectal pouch and fistula was studied morphologically in 33 newborn piglets with high and low forms of anal atresia and was found to be similar to the epithelial coating of the anal canal in normal piglets: the typical epithelium of the rectum changed its character into transitional epithelium at the region of the internal sphincter which surrounded the fistulae in all animals. In the caudal part of the fistula the transitional epithelium was followed by squamous epithelium. Only in male piglets with deformities and recto-urethral fistulae no squamous epithelium was found. In these cases transitional epithelium covered all parts of the fistula and the region of the internal sphincter. Anal glands were found in all animals, with or without anorectal malformations. They always invaded the internal sphincter. According to our morphological studies the fistula in anorectal malformations represents an ectopic anal canal.

  5. Rectal atresia and anal stenosis: the difference in the operative technique for these two distinct congenital anorectal malformations.

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    Lane, V A; Wood, R J; Reck, C; Skerritt, C; Levitt, M A

    2016-04-01

    Rectal atresia and anal stenosis are rare forms of anorectal malformations. The aim of the definitive surgical repair in such cases is to preserve the anal canal, the dentate line, and the sphincter complex. We present a case of rectal atresia and anal stenosis to demonstrate the differences in the operative repair. The techniques described leave the anterior wall of the very distal anal canal untouched in both rectal stenosis and anal atresia; however, the dissection of the rectum differs. The atretic rectum in rectal atresia is mobilized and sutured to the anal canal circumferentially. In anal stenosis, the posterior rectum is mobilized in the form of rectal advancement, and the posterior 180° is anastomosed directly to the skin (as in a standard PSARP) with preservation of the anal canal as the anterior 180° of the final anoplasty. These patients have an excellent prognosis for bowel control and fecal continence, and therefore, complete mobilization and resection of the anal canal must be avoided. PMID:26902368

  6. Atresia of the bilateral pulmonary veins: a rare and dismal anomaly identified on cardiac CT

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    Goo, Hyun Woo; Park, Sang-Hyub; Koo, Hyun Jung; Cho, Young Hoon; Lee, Eunsol [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Seoul (Korea, Republic of)

    2014-08-15

    Imaging findings of bilateral pulmonary vein atresia have not been described. To describe cardiac CT findings and clinical outcomes of bilateral pulmonary vein atresia. Three newborns with bilateral pulmonary vein atresia were encountered at our institution during a period of 8 years. We evaluated prenatal echocardiographic findings, clinical presentations, postnatal echocardiographic findings, chest radiographic findings, cardiac CT findings and clinical outcomes. All newborns presented immediately after birth with severe cyanosis, respiratory distress and acidosis that were unresponsive to medical management. Prenatal and postnatal echocardiographic studies and chest radiography were misleading, inconclusive or nonspecific in making the diagnosis in these children; however cardiac CT clearly demonstrated atresia of the bilateral pulmonary veins with multiple small mediastinal collateral veins and pulmonary edema. Surgical treatments were not feasible for this anomaly. Their clinical outcomes were universally dismal and all infants died within 3 days. Cardiac CT provides an accurate diagnosis of bilateral pulmonary vein atresia and leads to prompt treatment decision in these children. (orig.)

  7. Atresia of the bilateral pulmonary veins: a rare and dismal anomaly identified on cardiac CT

    International Nuclear Information System (INIS)

    Imaging findings of bilateral pulmonary vein atresia have not been described. To describe cardiac CT findings and clinical outcomes of bilateral pulmonary vein atresia. Three newborns with bilateral pulmonary vein atresia were encountered at our institution during a period of 8 years. We evaluated prenatal echocardiographic findings, clinical presentations, postnatal echocardiographic findings, chest radiographic findings, cardiac CT findings and clinical outcomes. All newborns presented immediately after birth with severe cyanosis, respiratory distress and acidosis that were unresponsive to medical management. Prenatal and postnatal echocardiographic studies and chest radiography were misleading, inconclusive or nonspecific in making the diagnosis in these children; however cardiac CT clearly demonstrated atresia of the bilateral pulmonary veins with multiple small mediastinal collateral veins and pulmonary edema. Surgical treatments were not feasible for this anomaly. Their clinical outcomes were universally dismal and all infants died within 3 days. Cardiac CT provides an accurate diagnosis of bilateral pulmonary vein atresia and leads to prompt treatment decision in these children. (orig.)

  8. Atresia anal, fístula uretrorretal congênita, bolsa escrotal acessória e pseudo-hermafroditismo em bezerro mestiço Anal atresia, congenital urethrorectal fistula, accessory scrotum and pseudohermafroditism in a crossbred calf

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    Thaís Gomes Rocha

    2010-05-01

    Full Text Available Neste trabalho, é descrito o caso de um bezerro mestiço recém-nascido que apresentava atresia anal tipo 2, fístula uretrorretal congênita, bolsa escrotal bífida e pseudo-hermafroditismo masculino. O principal sinal clínico era a eliminação de fezes por meio do óstio prepucial, uma apresentação incomum em casos de fístula uretrorretal em animais machos. Apesar de o quadro de atresia anal ser relativamente comum nessa espécie, os outros defeitos congênitos encontrados são pouco frequentes.In this study, the case of a newborn calf, which presented type 2 anal atresia, congenital urethrorectal fistula, bifid scrotum and male pseudohermafroditism is described. The main clinical sign was the elimination of feces by the prepucial ostium, an unusual finding in cases of urethrorectal fistula in male animals. Although anal atresia is relatively common in bovines, the other congenital defects found in this case are uncommon.

  9. Associação de agenesia sacrococcígea e atresia anal em gato sem raça definida Sacrococcygeal agenesis association and anal atresia in mixed breed cats

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    Felipe Purcell de Araújo

    2009-09-01

    Full Text Available O presente trabalho teve como objetivo descrever o caso de um felino, que desde o nascimento apresentou atresia anal, ausência de cauda e malformação dos membros pélvicos. Ao exame radiográfico, pôde-se observar presença de agenesia da sétima vértebra lombar, sacro e vértebras coccígeas, espinha bífida, meningocele, hiperflexão dos joelhos e desvio valgo dos tarsos, diagnosticando-se agenesia sacrococcígea associada à atresia anal.This paper has the objective to report a case of a cat that since birth had anal atresia, absence of tail and malformation of the pelvic member. The radiographic examination revealed agenesis of the seventh lumbar vertebra, sacral and coccygeal vertebrae, spina bifida, meningocele, hyperflexion of the knees and tarsal valgus deviation, diagnosing sacrococcygeal agenesis associated with anal atresia.

  10. Atresia anal en perros y gatos: conceptos actuales a partir de tres casos clínicos Anal atresia in dogs and cats: the scope from three clinical cases

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    EM García-González

    2012-01-01

    Full Text Available La atresia anal se define como la falta de comunicación del recto y el perineo a través del ano, siendo esta la malformación anorrectal reportada con mayor frecuencia en el perro y el gato. Está asociada a alteraciones en la diferenciación de la cloaca en el embrión en desarrollo; sin embargo los mecanismos fisiopatológicos involucrados hasta el momento no han sido completamente esclarecidos. En el presente documento se expone la experiencia en el diagnóstico y manejo de tres pacientes (dos perros y un gato con atresia anal, presentamos los resultados del análisis de la frecuencia de esta patología en nuestro centro hospitalario, realizamos una revisión detallada de las teorías de los mecanismos fisiopatológicos involucrados en el desarrollo embrionario y con base en estos criterios, sugerimos la clasificación del tipo de atresia anal más apropiada partiendo del análisis de las propuestas existentes y su relación con los conceptos actuales de la anatomía embriológica.Anal atresia is defined as the lack of a complete communication between rectum and the anus, it is the most common anorectal malformation and has been observed mainly in dogs. It has been associated to alterations of anogenital differentiation of the cloaca in the embryo, however, the exact mechanisms remain unclear. Different criteria have been proposed in the literature to classify anal atresia. This study shows the diagnosis, treatment and outcome of three cases (two dogs and one cat with anal atresia. The frequency of this malformation in our hospital is reported as well as a discussion of the theories of the processes involved in the development of anal atresia, and a revised classification for this pathology according to embryo anatomy and development is proposed.

  11. Congenital anorectal atresia: MR imaging of late post-operative appearances in adult patients with anal incontinence

    International Nuclear Information System (INIS)

    To describe the MR imaging findings in adults presenting with anal incontinence following pull-through perineoplasty for anorectal atresia. 15 adults (12 male, 3 female; age 22-52 years) with anal incontinence following a prior perineal pull-through procedure as an infant for anorectal atresia were identified retrospectively. MR imaging was performed using either an endoanal coil or body coil. MR images were reviewed by three observers who noted whether pelvic floor and sphincter muscles were present and, if so, whether they were thinned or not. Data were tabulated and raw frequencies determined. Images were unavailable for one patient, leaving 14 for analysis. Anal stenosis prevented endoanal coil placement in 5. The pull-through was anatomically correct in 12 (86 %) patients but was misdirected in 2. Thinned muscle was seen in 11 (79 %) patients. External sphincter thinning was commonest (present in 10 patients), with levator plate thinning least common (present in 4 patients). Only one patient had thinning of all muscle groups. MR imaging may be used to determine the extent and quality of residual pelvic floor and anal sphincter muscle in adults who have functional disability following pull-through perineoplasty for anorectal agenesis. (orig.)

  12. Congenital anorectal atresia: MR imaging of late post-operative appearances in adult patients with anal incontinence

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    Gartner, Louise; Peiris, Chand; Marshall, Michele [St. Mark' s Hospital, Department of Intestinal Imaging, London (United Kingdom); Taylor, Stuart A.; Halligan, Steve [University College London, Centre for Medical Imaging, London (United Kingdom)

    2013-12-15

    To describe the MR imaging findings in adults presenting with anal incontinence following pull-through perineoplasty for anorectal atresia. 15 adults (12 male, 3 female; age 22-52 years) with anal incontinence following a prior perineal pull-through procedure as an infant for anorectal atresia were identified retrospectively. MR imaging was performed using either an endoanal coil or body coil. MR images were reviewed by three observers who noted whether pelvic floor and sphincter muscles were present and, if so, whether they were thinned or not. Data were tabulated and raw frequencies determined. Images were unavailable for one patient, leaving 14 for analysis. Anal stenosis prevented endoanal coil placement in 5. The pull-through was anatomically correct in 12 (86 %) patients but was misdirected in 2. Thinned muscle was seen in 11 (79 %) patients. External sphincter thinning was commonest (present in 10 patients), with levator plate thinning least common (present in 4 patients). Only one patient had thinning of all muscle groups. MR imaging may be used to determine the extent and quality of residual pelvic floor and anal sphincter muscle in adults who have functional disability following pull-through perineoplasty for anorectal agenesis. (orig.)

  13. Associated congenital anomalies: Vestibular fistula, duodenal atresia and obstructive refluxing megaureter

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    Bukarica Svetlana S.

    2004-01-01

    Full Text Available Congenital gastrointestinal anomalies include spectrum of malformations, simple ones as well as complex, which are associated with anomalies of other organs and systems of organs. Most infants (50-60% with imperforate anus also have genitourinary, cardiovascular, gastrointestinal and vertebral anomalies. This case report is of a female newborn with prenatally diagnosed duodenal atresia and right hydronephrosis. Clinical investigation revealed an imperforate anus with vestibular fistula. A plain abdominal x-ray showed a "double-bubble” sign, characteristic for duodenal atresia. Intraluminal membrane was excised and sigmoid colostomy was performed in general anesthesia on the first day of life. Ultrasound and intravenous pyelography confirmed a hydronephrotic right kidney and obstructive megaureter after Pena. In the fifth month of life, the ureter was tapered and reimplanted. Six months later, posterior sagittal anorectoplasty was performed. In the sewentheenth month of life the colostomy was closed. This child attained normal growth and development, normal bowel control, fecal continence without soiling, obstipation and urinary infections.

  14. [Congenital Esophageal Atresia].

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    Suzuki, Makoto; Kuwano, Hiroyuki

    2015-07-01

    In this report, we describe the esophageal atresia in terms of current surgical management on the basis of our experience and literatures. Traditionally, infants with esophageal atresia have presented shortly after birth because of an inability to pass an orogastric tube, respiratory distress, or an inability to tolerate feeding. And also, an isolated trachea-esophageal fistula (TEF) usually cases coughing, recurrent pneumonia, or choking during feedings. To ignore these symptoms is to risk a delayed diagnosis. The condition may be associated with other major congenital anomalies such as those seen in the vertebral, anal, cardiac, tracheo-esophageal, renal/radial (VACTER) association, or it may be an isolated defect. Therapeutic strategies for esophageal atresia are a prevention of pulmonary complication by TEF closing and an early establishment of enteral alimentation. We promptly repair healthy infants without performing a gastrostomy and delay repair in infants with high-risk factors such as associated severe cardiac anomaly and respiratory insufficiency. Esophageal atresia has been classically approached through a thoracotomy. The disadvantages of such a thoracotomy have been recognized for a long time, for example winged scapula, elevation of fixation of shoulder, asymmetry of the chest wall, rib fusion, scoliosis, and breast and pectoral muscle maldevelopment. To avoid such disadvantages, thoracoscopic repair was recently reported. PMID:26197921

  15. Possible Waardenburg syndrome with gastrointestinal anomalies.

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    Nutman, J; Steinherz, R; Sivan, Y; Goodman, R M

    1986-01-01

    We describe a patient with possible Waardenburg syndrome associated with anal atresia and oesophageal atresia with tracheooesophageal fistula. Three other published cases with atretic gastrointestinal anomalies associated with the Waardenburg syndrome are reviewed. We conclude that the association between atretic lesions of the gastrointestinal tract and the Waardenburg syndrome may be a significant one.

  16. Atresia ani in the dog: a retrospective study.

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    Vianna, Maria L; Tobias, Karen M

    2005-01-01

    Congenital anomalies of the rectum and anus are rare in dogs. The most frequently reported anomaly is atresia ani. Four types of atresia ani have been reported, including congenital anal stenosis (Type I); imperforate anus alone (Type II) or combined with more cranial termination of the rectum as a blind pouch (Type III); and discontinuity of the proximal rectum with normal anal and terminal rectal development (Type IV). An increased incidence was found in females and in several breeds, including miniature or toy poodles and Boston terriers. Surgical repair is the treatment of choice, but postoperative complications can occur, including fecal incontinence and colonic atony secondary to prolonged preoperative distension. PMID:16141183

  17. 先天性肛门闭锁的影响因素分析%Risk factors for congenital anal atresia

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    高晓燕; 张爱民; 廖铁军; 郭伟忠; 潘学军; 潘敏仪; 肖厚兰; 朱金林; 吴龙耀; 黄祖琳; 高平明; 吴时光; 麦智广; 周杰; 黄润忠; 张水堂; 钟焕琼; 廖友明

    2016-01-01

    目的探讨新生儿先天性肛门闭锁发生的影响因素。方法以2011年1月至2014年12月广东省佛山市17家医院收治的70例先天性肛门闭锁患儿作为病例组,选择与病例组同期住院、无肛门闭锁及其他严重畸形的新生儿70例作为对照组。采用单因素分析和多因素logistic 回归分析探讨先天性肛门闭锁发生的影响因素。结果单因素分析显示,病例组和对照组两组在母亲是否高龄、孕早期是否口服叶酸、孕早期有无感染、有无羊水过多及新生儿性别方面比较差异有统计学意义(P<0.05)。多因素logistic 回归分析显示,孕早期感染(OR=18.776)和男性新生儿(OR=9.304)为先天性肛门闭锁的危险因素,母亲孕早期口服叶酸(OR=0.086)是保护因素。结论母亲孕早期感染为先天性肛门闭锁发生的高危因素;男性新生儿患先天性肛门闭锁的风险大于女性;母亲孕早期补充叶酸可减少先天性肛门闭锁的发生。%ObjectiveTo investigate the risk factors for the development of congenital anal atresia in neonates. MethodsA total of 70 neonates who were admitted to 17 hospitals in Foshan, China from January 2011 to December 2014 were enrolled as case group, and another 70 neonates who were hospitalized during the same period and had no anal atresia or other severe deformities were enrolled as control group. Univariate and multivariate logistic regression analyses were used to investigate the risk factors for the development of congenital anal atresia.ResultsThe univariate analysis revealed that the age of mothers, presence of oral administration of folic acid, infection during early pregnancy, and polyhydramnios, and sex of neonates showed signiifcant differences between the case and control groups (P<0.05). The multivariate logistic regression analysis revealed that infection during early pregnancy (OR=18.776) and male neonates (OR=9.304) were risk factors for

  18. Radiologic imaging of congenital gastrointestinal anomalies in infants

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    Leny Zabidi

    2014-11-01

    Full Text Available Background Congenital gastrointestinal anomalies may manifest signs or symptoms in the first few days of life, most commonly in the form of obstructions. Radiologic imaging plays an important role in diagnosis confirmation and surgical correction plans. Most cases may be diagnosed by plain radiographs alone, but CT scans and MRI may be needed to make accurate diagnoses, especially in difficult cases. Objective To report radiologic imaging findings in infants with congenital gastrointestinal anomalies. Methods For this retrospective, cross-sectional study we took secondary data from medical records of infants with congenital gastrointestinal anomalies in Dr. Kariadi Hospital, Semarang, Indonesia from January 2010 – June 2011. Diagnosis of congenital anomalies was confirmed by clinical manifestation and radiologic imaging. Radiologic findings were reviewed by a single radiologist on duty at that time. Data is presented in the form of frequency distribution. Results Subjects consisted of 50 males and 23 females. The most common complaints were vomiting in 14 subjects (19%, abdominal distension in 31 subjects (43%, and fecal passage dysfunction in 28 subjects (38%. Radiologic imaging of subjects with congenital gastrointestinal anomalies revealed the following conditions: anal atresia in 28 subjects (38%, congenital megacolon in 21 subjects (29%, esophageal atresia in 14 subjects (19%, duodenal atresia in 9 subjects (12%, and pyloric atresia in 1 subject (2%. Conclusion Using radiologic imaging of infants with congenital gastrointestinal anomalies, the most to least common conditions found were anal atresia, congenital megacolon, esophageal atresia, duodenal atresia, and pyloric atresia.

  19. Esophageal Atresia

    DEFF Research Database (Denmark)

    Pedersen, Rikke Neess; Markøw, Simone; Kruse-Andersen, Søren;

    2013-01-01

    Esophageal atresia (EA) is one of the most frequent congenital alimentary tract anomalies with a considerable morbidity throughout childhood. This study evaluates the gastroesophageal problems in 5-15year old children with EA and aims to identify factors predisposing to esophagitis in EA....

  20. Microdeletion 22q11 and oesophageal atresia

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    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

  1. PYLORIC ATRESIA IN ASSOCIATION WITH MULTIPLE COLONIC ATRESIAS IN A NEONATE: AN UNREPORTED ASSOCIATION

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    Vijay C Pujar

    2012-01-01

    Full Text Available Pyloric atresia is rare cause of gastrointestinal obstruction in neonates and usually occurs as an isolated anomaly. They have been associated with multiple small bowel and colonic atresias but not reported in association with isolated multiple colonic atresias. A case of pyloric atresia oc-curring in association with multiple colonic atresias is being reported here.

  2. Anal atresia, coloboma, microphthalmia, and nasal skin tag in a female patient with 3.5 Mb deletion of 3q26 encompassing SOX2.

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    Salem, Nabeel J M; Hempel, Maja; Heiliger, Katrin-Janine; Hosie, Stuart; Meitinger, Thomas; Oexle, Konrad

    2013-06-01

    A full term female newborn presented with prominent forehead, bilateral microphthalmia, iris coloboma and cataract, wide intercanthal distance, large, low-set and protruding ears, skin tag at the left nasal nostril, imperforate anus with rectovestibular fistula, and postnatal growth delay with brachymicrocephaly. A marker chromosome was not detectable and the copy number of 22q11 was normal. However, array CGH revealed a 3.5 Mb microdeletion of chromosome region 3q26.32-3q26.33 (chr. 3: 178,598,162-182,114,483; hg19) which comprised the SOX2 gene. While SOX2 haploinsufficiency is known to cause microphthalmia and coloboma, it has not been described before in patients with anal atresia.

  3. Gallbladder Duplication Associated with Gastro-Intestinal Atresia.

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    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

  4. Esophageal atresia

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    Esophageal atresia is a disorder of the digestive system in which the esophagus does not develop properly. The ... Esophageal atresia (EA) is a congenital defect. This means it occurs before birth. There are several types. In ...

  5. Oesophageal atresia

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    Spitz Lewis

    2007-05-01

    Full Text Available Abstract Oesophageal atresia (OA encompasses a group of congenital anomalies comprising of an interruption of the continuity of the oesophagus with or without a persistent communication with the trachea. In 86% of cases there is a distal tracheooesophageal fistula, in 7% there is no fistulous connection, while in 4% there is a tracheooesophageal fistula without atresia. OA occurs in 1 in 2500 live births. Infants with OA are unable to swallow saliva and are noted to have excessive salivation requiring repeated suctioning. Associated anomalies occur in 50% of cases, the majority involving one or more of the VACTERL association (vertebral, anorectal, cardiac, tracheooesophageal, renal and limb defects. The aetiology is largely unknown and is likely to be multifactorial, however, various clues have been uncovered in animal experiments particularly defects in the expression of the gene Sonic hedgehog (Shh. The vast majority of cases are sporadic and the recurrence risk for siblings is 1%. The diagnosis may be suspected prenatally by a small or absent stomach bubble on antenatal ultrasound scan at around 18 weeks gestation. The likelihood of an atresia is increased by the presence of polyhydramnios. A nasogastric tube should be passed at birth in all infants born to a mother with polyhydramnios as well as to infants who are excessively mucusy soon after delivery to establish or refute the diagnosis. In OA the tube will not progress beyond 10 cm from the mouth (confirmation is by plain X-ray of the chest and abdomen. Definitive management comprises disconnection of the tracheooesophageal fistula, closure of the tracheal defect and primary anastomosis of the oesophagus. Where there is a "long gap" between the ends of the oesophagus, delayed primary repair should be attempted. Only very rarely will an oesophageal replacement be required. Survival is directly related to birth weight and to the presence of a major cardiac defect. Infants weighing over

  6. Neonate with VACTERL Association and a Branchial Arch Anomaly without Hydrocephalus.

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    Velazquez, Danitza; Pereira, Elaine; Havranek, Thomas

    2016-03-01

    VACTERL (vertebral anomalies, anal atresia, cardiac defect, tracheoesophageal fistula, renal anomaly, limb anomalies) is an association of anomalies with a wide spectrum of phenotypic expression. While the majority of cases are sporadic, there is evidence of an inherited component in a small number of patients as well as the potential influence of nongenetic risk factors (maternal diabetes mellitus). Presence of hydrocephalus has been reported in VACTERL patients (VACTERL-H) in the past, with some displaying branchial arch anomalies. We report the unique case of an infant of diabetic mother with VACTERL association and a branchial arch anomaly-in the absence of hydrocephalus.

  7. Oesophageal atresia

    DEFF Research Database (Denmark)

    Pedersen, Rikke Neess; Calzolari, Elisa; Husby, Steffen;

    2012-01-01

    To describe prevalence, prenatal diagnosis and epidemiological data on oesophageal atresia from 23 well-defined European regions and compare the prevalence between these regions.......To describe prevalence, prenatal diagnosis and epidemiological data on oesophageal atresia from 23 well-defined European regions and compare the prevalence between these regions....

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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

  10. Pulmonary Atresia

    Science.gov (United States)

    ... to repair the defect. Return to main topic: Congenital Heart Disease See on other sites: MedlinePlus https://medlineplus.gov/ency/article/001091.htm Pulmonary atresia American Heart Association www. ...

  11. Associated congenital anomalies among cases with Down syndrome.

    Science.gov (United States)

    Stoll, Claude; Dott, Beatrice; Alembik, Yves; Roth, Marie-Paule

    2015-12-01

    which associated anomalies are most common in cases with DS with associated anomalies. In this study we observed a higher percentage of associated anomalies than in the other reported series as well as an increase in the incidence of duodenal atresia, urinary system anomalies, musculoskeletal system anomalies, and respiratory system anomalies, and a decrease in the incidence of anal atresia, annular pancreas, and limb reduction defects. In conclusion, we observed a high prevalence of total congenital anomalies and specific patterns of malformations associated with Down syndrome which emphasizes the need to evaluate carefully all cases with Down syndrome for possible associated major congenital anomalies.

  12. Associated congenital anomalies among cases with Down syndrome.

    Science.gov (United States)

    Stoll, Claude; Dott, Beatrice; Alembik, Yves; Roth, Marie-Paule

    2015-12-01

    which associated anomalies are most common in cases with DS with associated anomalies. In this study we observed a higher percentage of associated anomalies than in the other reported series as well as an increase in the incidence of duodenal atresia, urinary system anomalies, musculoskeletal system anomalies, and respiratory system anomalies, and a decrease in the incidence of anal atresia, annular pancreas, and limb reduction defects. In conclusion, we observed a high prevalence of total congenital anomalies and specific patterns of malformations associated with Down syndrome which emphasizes the need to evaluate carefully all cases with Down syndrome for possible associated major congenital anomalies. PMID:26578241

  13. Epidemiology of small intestinal atresia in Europe

    DEFF Research Database (Denmark)

    Best, Kate E; Tennant, Peter W G; Addor, Marie-Claude;

    2012-01-01

    The epidemiology of congenital small intestinal atresia (SIA) has not been well studied. This study describes the presence of additional anomalies, pregnancy outcomes, total prevalence and association with maternal age in SIA cases in Europe.......The epidemiology of congenital small intestinal atresia (SIA) has not been well studied. This study describes the presence of additional anomalies, pregnancy outcomes, total prevalence and association with maternal age in SIA cases in Europe....

  14. Congenital tracheoesophagel fistula with no esophageal atresia (H-type) in adult (a case report)

    Energy Technology Data Exchange (ETDEWEB)

    Byun, W. M.; Jung, K. H.; Cho, K. H.; Kim, S. Y.; Hwang, M. S.; Chang, J. C.; Chung, M. K.; Lee, C. J. [Yeungnam University College of Medicine, Gyengsan (Korea, Republic of)

    1986-08-15

    Congenital tracheoesophageal fistula with no esophageal atresia is considered a rate type anomaly, especially in adult age. We report a case of isolated tracheoesophageal fistula with no esophageal atresia with review of literature.

  15. Esophageal stenosis with esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Thomason, M.A.; Gay, B.B.

    1987-03-01

    Esophageal atresia with tracheosophageal fistula may be associated rarely with distal esophageal stenosis. Three patients are reported with this combination of esophageal anomalies. In addition the clinical and radiologic features of 24 patients previously reported in the literature are reviewed. Careful evaluation of the distal esophagus during postoperative contrast studies in patients with esophageal atresia should be obtained to exclude distal stenosis. The presence of unrecognized distal esophageal stenosis may lead to complications of postoperative anastomotic leaks, poor healing of the anastomosis, aspiration, and impaction of a solid food bolus proximal to the stenosis.

  16. Genetic players in esophageal atresia and tracheoesophageal fistula.

    NARCIS (Netherlands)

    Brunner, H.G.; Bokhoven, J.H.L.M. van

    2005-01-01

    Esophageal atresia is a common and serious developmental anomaly, of which the causes remain largely unknown. Studies in vertebrate models indicate the importance of the sonic hedgehog pathway in esophageal atresia, but its relevance to the human condition remains to be defined. Now, three genes hav

  17. Atresia of the gastrointestinal tract: imaging evaluation; Atresia do trato gastrintestinal: avaliacao por metodos de imagem

    Energy Technology Data Exchange (ETDEWEB)

    Figueiredo, Sizenildo da Silva; Nobrega, Bruno Barcelos da; Ribeiro, Luiza Helena Vilela; Costa, Marlos Augusto Bittencourt; Monteiro, Soraya Silveira; Lederman, Henrique Manoel [Universidade Federal de Sao Paulo (UNIFESP/EPM), SP (Brazil). Dept. de Diagnostico por Imagem]. E-mail: brunoradiol@hotmail.com; Oliveira, Galba Leite [Irmandade Santa Casa de Misericordia de Sao Paulo, SP (Brazil). Dept. de Diagnostico por Imagem; Esteves, Edward [Goias Univ., Goiania, GO (Brazil). Faculdade de Medicina. Dept. de Cirurgia e Urologia Pediatrica

    2005-04-01

    A wide spectrum of congenital anomalies may affect the gastrointestinal tract at any level from the esophagus to the anus. Atresia is an important cause of gastrointestinal obstruction with high morbidity rate in neonates. Different pathogenetic mechanisms could cause this malformation and the two classical explanations are: a defect of recanalization of the intestinal tube or an interruption of blood supply during intrauterine life. The authors present a literature review with an iconographic essay of imaging findings in children with gastrointestinal atresia. (author)

  18. Wind sock deformity in rectal atresia

    Directory of Open Access Journals (Sweden)

    Hosseini Seyed

    2009-01-01

    Full Text Available Rectal atresia is a rare anorectal deformity. It usually presents with neonatal obstruction and it is often a complete membrane or severe stenosis. Windsock deformity has not been reported in rectal atresia especially, having been missed for 2 years. A 2-year-old girl reported only a severe constipation despite having a 1.5-cm anal canal in rectal examination with scanty discharge. She underwent loop colostomy and loopogram, which showed a wind sock deformity of rectum with mega colon. The patient underwent abdominoperineal pull-through with good result and follow-up. This is the first case of the wind sock deformity in rectal atresia being reported after 2 years of age.

  19. Anomalies.

    Science.gov (United States)

    Online-Offline, 1999

    1999-01-01

    This theme issue on anomalies includes Web sites, CD-ROMs and software, videos, books, and additional resources for elementary and junior high school students. Pertinent activities are suggested, and sidebars discuss UFOs, animal anomalies, and anomalies from nature; and resources covering unexplained phenonmenas like crop circles, Easter Island,…

  20. A Surviving Child With Complete Proximal Tracheal Atresia

    OpenAIRE

    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 obstetr...

  1. Embryogenesis of esophageal atresia: Is localized vascular accident a factor?

    Directory of Open Access Journals (Sweden)

    Dutta Hemonta

    2009-01-01

    Full Text Available Several theories on embryogenesis of esophageal atresia have been proposed, none could explain the whole spectrum of this anomaly. We report a new variant of esophageal atresia in which the two blind pouches were joined by an atretic band. Histology of the atretic part showed groups of striated muscle arranged haphazardly without any lumen. The existing theories on etiology of esophageal atresia cannot explain this variant. However, localized vascular accident during intrauterine life resulting in disturbances in regional microcirculation could be a possible factor as demonstrated by Louw and Barnard in relation to jejunoileal atresia. This is contrary to the current understanding that disproportionate growth of the horizontal esophageal folds results in esophageal atresia.

  2. Functional pulmonary atresia in newborn with normal intracardiac anatomy: Successful treatment with inhaled nitric oxide and pulmonary vasodilators

    Directory of Open Access Journals (Sweden)

    Gürkan Altun

    2013-01-01

    Full Text Available Functional pulmonary atresia is characterized by a structurally normal pulmonary valve that does not open during right ventricular ejection. It is usually associated with Ebstein′s anomaly, Uhl′s anomaly, neonatal Marfan syndrome and tricuspid valve dysplasia. However, functional pulmonary atresia is rarely reported in newborn with anatomically normal heart. We report a newborn with functional pulmonary atresia who had normal intracardiac anatomy, who responded to treatment with nitric oxide and other vasodilator therapy successfully.

  3. Atresia in the esophagus

    Directory of Open Access Journals (Sweden)

    Crisanto Abad Celuria

    2005-12-01

    Full Text Available Esophageal atresia is the congenital lack of continuity of the esophagus with or without communication to the airway. Incidence is estimateed in 1 by 3000 to 4500 alive newborns. Cases in brothers are found and children of parents with atresia esophagus, nevertheless not a hereditary is recognized; also there is a greater frequency in twins. We presented the Good Clinical Practices Guideline for Esophageal atresia, approved by consensus in the 2nd National Good Clinical Practices Workshop in Pediatric Surgery (Manzanillo, Cuba, September 31 - October 3, 2002.

  4. Management of congenital choanal atresia: A pedodontist′s role

    Directory of Open Access Journals (Sweden)

    Fathima Niloofar

    2015-01-01

    Full Text Available Choanal atresia is a rare cryptic congenital anomaly with developmental failure of the nasal cavity to communicate with the nasophaynx, resulting in a significant physiological and anatomical alteration of the dento-facial complex. It is considered as a life-threatening or lethal congenital malformation in an obligatory nasal breathing neonate. Elective transnasal endoscopic repair is the most preferred and minimally invasive procedure to correct this anomaly. This case report describes the multi-disciplinary team approach that included pedodontists to manage the case of a 6-month-old infant with choanal atresia suffering from breathlessness, feeding difficulty, and disturbed sleep.

  5. Epidemiology of small intestinal atresia in Europe: a register-based study.

    LENUS (Irish Health Repository)

    Best, Kate E

    2012-09-01

    The epidemiology of congenital small intestinal atresia (SIA) has not been well studied. This study describes the presence of additional anomalies, pregnancy outcomes, total prevalence and association with maternal age in SIA cases in Europe.

  6. Chronic Pulmonary Aspergillosis Complicating Bronchial Atresia

    Directory of Open Access Journals (Sweden)

    Mazen O. Al-Qadi

    2014-01-01

    Full Text Available Bronchial atresia is a rare pulmonary developmental anomaly characterized by the presence of a focal obliteration of a segmental or lobar bronchial lumen. The lung distal to the atretic bronchus is typically emphysematous along with the presence of mucus filled ectatic bronchi (mucoceles. BA is usually asymptomatic but pulmonary infections can rarely develop in the emphysematous lung distal to the atretic bronchus. We present a unique case of chronic pulmonary aspergillosis (CPA in a patient with BA with no evidence of immune dysfunction. The patient was treated initially with voriconazole and subsequently underwent surgical excision of the involved area. On follow-up, she has done extremely well with no evidence for recurrence. In summary, we describe the first case of chronic pulmonary aspergillosis in an immunocompetent patient with bronchial atresia.

  7. Duodenal atresia in association with situs inversus abdominus

    Directory of Open Access Journals (Sweden)

    Raghu Shankar

    2012-01-01

    Full Text Available We report a rare association of duodenal atresia with situs inversus abdominus in a newborn. The infantogram revealed "reverse double-bubble sign" without dextrocardia. The sonography and echocardiography confirmed the diagnosis of situs inversus abdominus with multiple cardiac anomalies. Laparotomy and a duodenoduodenostomy were carried out.

  8. Current knowledge on esophageal atresia

    Institute of Scientific and Technical Information of China (English)

    Paulo Fernando Martins Pinheiro; Ana Cristina Sim(o)es e Silva; Regina Maria Pereira

    2012-01-01

    Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus.The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care,neonatal anesthesia,ventilatory and nutritional support,antibiotics,early surgical intervention,surgical materials and techniques.Indeed,mortality is currently limited to those cases with coexisting severe life-threatening anomalies.The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed.The primary surgical correction for EA and TEF is the best option in the absence of severe malformations.There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still controversial.The primary complications during the postoperative period are leak and stenosis of the anastomosis,gastro-esophageal reflux,esophageal dysmotility,fistula recurrence,respiratory disorders and deformities of the thoracic wall.Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair.The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses.Much remains to be studied regarding this condition.This manuscript provides a literature review of the current knowledge regarding EA.

  9. Congenital intestinal atresia.

    Science.gov (United States)

    Davenport, M; Bianchi, A

    1990-09-01

    Surgery for infants with intestinal atresia has evolved along with the development of specialized neonatal surgical units. This once fatal condition now carries a better than 85% chance of survival and an excellent long-term prognosis. Recent advances in bowel preservation techniques have reduced morbidity and improved gut function in both the long and the short term. PMID:2257399

  10. National Esophageal Atresia Register.

    Science.gov (United States)

    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

  11. Delay in diagnosis of congenital anal stenosis

    OpenAIRE

    Weledji, Elroy P; Motaze Sinju

    2016-01-01

    Although a minor anorectal malformation the delay in diagnosis and treatment of anal stenosis may result in significant early or late complications. Early inspection of the perineum in the neonate to pick up and correct anorectal malformation improves long term outcome but this requires proper anal examination or it could be missed. We present and discuss a case of delayed diagnosis of congenital anal stenosis (a low anorectal anomaly) with an imminent colonic perforation. Severe anal stenosi...

  12. Role of Multislice Computed Tomography and Local Contrast in the Diagnosis and Characterization of Choanal Atresia

    Directory of Open Access Journals (Sweden)

    Khaled Al-Noury

    2011-01-01

    Full Text Available Objective. To illustrate the role of multislice computed tomography and local contrast instillation in the diagnosis and characterization of choanal atresia. To review the common associated radiological findings. Methods. We analyzed 9 pediatric patients (5 males and 4 females with suspected choanal atresia by multislice computed tomography. We recorded the type of atresia plate and other congenital malformations of the skull. Results. Multislice computed tomography with local contrast installed delineated the posterior choanae. Three patients had unilateral mixed membranous and bony atresia. Three patients had unilateral pure bony atresia. Only 1 of 7 patients have bilateral bony atresia. It also showed other congenital anomalies in the head region. One patient is with an ear abnormality. One patient had congenital nasal pyriform aperture stenosis. One of these patients had several congenital abnormalities, including cardiac and renal deformities and a hypoplastic lateral semicircular canal. Of the 6 patients diagnosed to have choanal atresia, 1 patient had esophageal atresia and a tracheoesophageal fistula. The remaining patients had no other CHARGE syndrome lesions. Conclusions. Local Contrast medium with the application of the low-dose technique helps to delineate the cause of the nasal obstruction avoiding a high radiation dose to the child.

  13. Dysphagia among Adult Patients who Underwent Surgery for Esophageal Atresia at Birth

    Directory of Open Access Journals (Sweden)

    Valérie Huynh-Trudeau

    2015-01-01

    Full Text Available BACKGROUND: Clinical experiences of adults who underwent surgery for esophageal atresia at birth is limited. There is some evidence that suggests considerable long-term morbidity, partly because of dysphagia, which has been reported in up to 85% of adult patients who undergo surgery for esophageal atresia. The authors hypothesized that dysphagia in this population is caused by dysmotility and/or anatomical anomalies.

  14. Intrauterine Intussusception Causing Ileal Atresia

    Directory of Open Access Journals (Sweden)

    Yogesh Kumar Sarin

    2010-11-01

    Full Text Available Intrauterine intussusception (IUI is the one of the rarest recognized causes of jejuno-ileal atresia (JIA. We report on a 15-day old full-term neonate presenting with features of intestinal obstruction, wherein on exploration, a visible ileo-ileal intussusception resulting in ileal atresia was found. The relevant literature has been reviewed.

  15. Congenital aplasia of the optic chiasm and esophageal atresia: a case report

    Directory of Open Access Journals (Sweden)

    Madonia Maurizio

    2011-08-01

    Full Text Available Abstract Introduction The complete absence of the chiasm (chiasmal aplasia is a rare clinical condition. Hypoplasia of the optic nerve and congenital nystagmus are almost invariably associated characteristics. Microphthalmos or anophthalmos are common features in chiasmal aplasia, while central nervous system abnormalities are less frequent. Esophageal atresia can be isolated or syndromic. In syndromic cases, it is frequently associated with cardiac, limb, renal or vertebral malformations and anal atresia. More rarely, esophageal atresia can be part of anophthalmia-esophageal-genital syndrome, which comprises anophthalmia or microphthalmia, genital abnormalities, vertebral defects and cerebral malformations. Here, a previously unreported case of chiasmal aplasia presenting without microphthalmos and associated with esophageal atresia is described. Case presentation Aplasia of the optic chiasm was identified in a Caucasian Italian 8-month-old boy with esophageal atresia. An ultrasound examination carried out at 21 weeks' gestation revealed polyhydramnios. Intrauterine growth retardation, esophageal atresia and a small atrial-septal defect were subsequently detected at 28 weeks' gestation. Repair of the esophageal atresia was carried out shortly after birth. A jejunostomy was carried out at four months to facilitate enteral feeding. The child was subsequently noted to be visually inattentive and to be neurodevelopmentally delayed. Magnetic resonance imaging revealed chiasmal aplasia. No other midline brain defects were found. His karyotype was normal. Conclusion If achiasmia is a spectrum, our patient seems to depict the most severe form, since he appears to have an extremely severe visual impairment. This is in contrast to most of the cases described in the literature, where patients maintain good--or at least useful-- visual function. To the best of our knowledge, the association of optic nerve hypoplasia, complete chiasmal aplasia, esophageal

  16. Delay in diagnosis of congenital anal stenosis

    Directory of Open Access Journals (Sweden)

    Elroy P. Weledji

    2016-03-01

    Full Text Available Although a minor anorectal malformation the delay in diagnosis and treatment of anal stenosis may result in significant early or late complications. Early inspection of the perineum in the neonate to pick up and correct anorectal malformation improves long term outcome but this requires proper anal examination or it could be missed. We present and discuss a case of delayed diagnosis of congenital anal stenosis (a low anorectal anomaly with an imminent colonic perforation. Severe anal stenosis will always require examination under anesthesia with graded Hegar's dilatation followed by postoperative maintenance. The Hegar dilator is thus both diagnostic and therapeutic in congenital anal stenosis.

  17. Atresia in the esophagus Atresia Esofágica.

    Directory of Open Access Journals (Sweden)

    Narciso Hernández Rodríguez

    2005-12-01

    Full Text Available Esophageal atresia is the congenital lack of continuity of the esophagus with or without communication to the airway. Incidence is estimateed in 1 by 3000 to 4500 alive newborns. Cases in brothers are found and children of parents with atresia esophagus, nevertheless not a hereditary is recognized; also there is a greater frequency in twins. We presented the Good Clinical Practices Guideline for Esophageal atresia, approved by consensus in the 2nd National Good Clinical Practices Workshop in Pediatric Surgery (Manzanillo, Cuba, September 31 - October 3, 2002.
    La atresia esofágica es la falta congénita de continuidad del esófago con o sin comunicación a la vía aérea. Se estima una incidencia de 1 cada 3000 a 4 500 neonatos vivos. Se encuentran casos en hermanos e hijos de padres con atresia de esófago, sin embargo no existe un patrón hereditario establecido, también hay una mayor frecuencia en gemelos. Se presenta la Guía de Buenas Prácticas Clínicas para Atresia esofágica, aprobada por consenso en el 2º Taller Nacional de Buenas Prácticas Clínicas en Cirugía Pediátrica (Manzanillo, 31 de septiembre al 3 de octubre del 2002.

  18. [Computerized tomography of the organs of the small pelvis in children with anorectal atresia].

    Science.gov (United States)

    Sitkovskiĭ, N B; Babiĭ, Ia S; Kaplan, V M; Dan'shin, T I; Sil'chenko, M I; Bodnar', V V; Gbenu, A S

    1992-01-01

    In 12 children with the different forms of anorectal atresia, for studying the state of a sphincter apparatus of the rectum and assessment of quality of its bringing down into the perineum after proctoplasty, computerized tomography of the organs of a small pelvis was used. Underdeveloped and undifferentiated musculus levator ani in children with high anorectal atresia and fistula to the urinary bladder was revealed. The method permits to establish exact location of the intestine brought down relative to musculus levator ani and external anal sphincter. PMID:1518247

  19. An integrated imaging approach for diagnosis of cervico-vaginal outflow defects and associated genital anomalies

    Directory of Open Access Journals (Sweden)

    Soha Talaat Hamed

    2015-12-01

    Conclusion: Ultrasound examination using different approaches remains the initial investigation for all patients with simple müllerian anomalies. MRI examination could be reserved for more complex anomalies, long cervicovaginal atresia and for cases of urogenital sinus syndrome.

  20. Biliary atresia: pathogenesis and treatment.

    Science.gov (United States)

    Bates, M D; Bucuvalas, J C; Alonso, M H; Ryckman, F C

    1998-01-01

    Biliary atresia is a disorder of infants in which there is obliteration or discontinuity of the extrahepatic biliary system, resulting in obstruction of bile flow. Untreated, the resulting cholestasis leads to progressive conjugated hyperbilirubinemia, cirrhosis, and hepatic failure. Biliary atresia has an incidence of approximately one in 10,000 live births worldwide. Evidence to date supports a number of pathogenic mechanisms for the development of biliary atresia. An infectious cause, such as by a virus, would seem most pausible in many cases. The clinical observation that biliary atresia is rarely encountered in premature infants would support an agent acting late in gestation. However, no infectious or toxic agent has been conclusively implicated in biliary atresia. Genetic mechanisms likely play important roles, even regarding susceptibility to other specific causes, but no gene whose altered function would result in obstruction or atresia of the biliary tree has been identified. The variety of clinical presentations support the notion that the proposed mechanisms are not mutually exclusive but may play roles individually or in combination in certain patients. Biliary atresia, when untreated, is fatal within 2 years, with a median survival of 8 months. The natural history of biliary atresia has been favorably altered by the Kasai portoenterostomy. Approximately 25 to 35% of patients who undergo a Kasai portoenterostomy will survive more than 10 years without liver transplantation. One third of the patients drain bile but develop complications of cirrhosis and require liver transplantation before age 10. For the remaining one third of patients, bile flow is inadequate following portoenterostomy and the children develop progressive fibrosis and cirrhosis. The portoenterostomy should be done before there is irreversible sclerosis of the intrahepatic bile ducts. Consequently, a prompt evaluation is indicated for any infant older than 14 days with jaundice to

  1. SMALL BOWEL DIVERTICULOSIS WITH JEJUNAL ATRESIA

    Directory of Open Access Journals (Sweden)

    Srinivas

    2015-07-01

    Full Text Available A 24 year s old primigravida had presented to us in third trimester with an antenatal ultrasound diagnosis of intestinal obstruction in the fetus and polyhydramnios. The parents were counseled and the obstetrician was advised to conduct the delivery as per obstetric indication, the patient delivered at 36 wks by an emergency LSCS. The baby was 1.8Kgs in weight and had an APGAR score of 9 and 10 at 1 and 5 minutes of life. Baby had significant bilious aspirate and did not pass meconium, it did not have dysmorphic featu res or any other associated anomalies, abdomen was soft and no masses were palpable. X ray abdomen revealed three gas bubbles with distal paucity of gas (Triple bubble sign. With the above findings a diagnosis of jejunal atresia was made and a laparotomy was planned on day 2 of life.

  2. Atresia coli in a Japanese black calf diagnosed by a barium sulphate enema contrast radiograph in the standing position: a case report

    International Nuclear Information System (INIS)

    A three day-old Japanese black calf was admitted with a history of abdominal distension and absence of defecation. Dilated loops of the small intestine and hypoplasia of the colon and rectum was observed upon a contrast radiographic examination in the standing position. At necropsy atresia coli with undeveloped rectum and patent anal opening was found. We conclude that a contrast radiograph in the standing position is useful for diagnosing atresia coli in such cases

  3. Congenital esophageal atresia with tracheo-esophageal fistula

    Energy Technology Data Exchange (ETDEWEB)

    Rhee, Chung Sik [Ewha Womans University College of Medicine, Seoul (Korea, Republic of)

    1970-10-15

    Three cases of esophageal atresia with tracheo-esophageal fistula. 1). Case 1: A female infant birth Wt. 1.95 kg , Apgar Score 10, Skeletal anomalies, was delivered after a pregnancy compeicated by hydroamnious on Aug. 17, 1970. The family history was not contributory. 2) Case 2: A male infant birth Wt. 2.8 kg , Apgar Score 8, was forcep delivered after a pregnancy on Feb. 8, 1970. This infant is twin. The family history was not contributory. 3) Case 3: A female infant birth Wt. 2.22 kg , Apgar Score 10, was C-section after a pregnancy on May, 16. 1970. The family history was not contributory. All cases: After 24 hours 5% glucose solution was given and immediately vomited and some of it regurgitating through the nose and mouth with associated cyanosis and dyspnea. A catheter was inserted through the nose into the esophagus under diagnosis of the esophageal atresia.

  4. Follicular growth, differentiation and atresia

    Institute of Scientific and Technical Information of China (English)

    JIN Xuan; LIU Yixun

    2003-01-01

    Only limited numbers of primordial follicles in mammalian ovary grow and differentiate to reach the stage of dominate follicles and ovulate. 99% of the follicles in the ovary undergo atresia at various stages of development. Regulation of follicular growth, development and atresia is a complex process and involves interactions between endocrine factors and intraovarian regulators. This review summarized:ⅰ) FSH may not be a survival factor in regulating slow-growing preantral follicles. Some locally produced growth factors, activin and orphan receptors might play a more important role at this stage. ⅱ) Estrogen, activin/inhibin and follistatin coordinate with FSH to regulate and control follicle differentiation. ⅲ) There are two types of follicular atresia induced by apoptosis which originates from GC or oocyte, respectively. Early translation of tPA mRNA into tPA protein in oocyte may be associated with oocyte apoptosis.

  5. Anal atresia and the Klein-Waardenburg syndrome.

    OpenAIRE

    Nutman, J; Nissenkorn, I; Varsano, I; Mimouni, M.; Goodman, R M

    1981-01-01

    A 3-month-old male infant with type I Klein-Waardenburg syndrome with an imperforated anus and a perineal fistula is reported. The possible association of this gastrointestinal malformation with the KW syndrome is discussed.

  6. Diagnosis and treatment of gastroesophageal reflux in patients with esophageal atresia

    NARCIS (Netherlands)

    J.H.L.J. Bergmeijer (Jan Hein)

    2002-01-01

    textabstractIn the last two decades, surgical treatment of children born with esophageal atresia has become a standard procedure. Postoperative mortality- now negligible in those born at term without other severe anomalies- mainly relates to patients with associated severe congenital cardiac malform

  7. Screening and Surveillance in Esophageal Atresia Patients: Current Knowledge and Future Perspectives

    NARCIS (Netherlands)

    F.W.T. Vergouwe (Florence); H. IJsselstijn (Hanneke); R.M.H. Wijnen (Rene); M.J. Bruno (Marco); V.M.C.W. Spaander (Manon)

    2015-01-01

    textabstractEsophageal atresia (EA) is a rare congenital anomaly. Enhanced operative techniques and intensive care treatment have improved survival among children with repaired EA (range, 93–95%). Many (up to 67%) suffer from gastroesophageal reflux (GER). The high incidence of GER and improved surv

  8. Biliary atresia Atresia de las vías biliares.

    Directory of Open Access Journals (Sweden)

    Pastor Thomas Olivares

    Full Text Available Biliary atresia is an obstructive and progressive process of unknown etiology that affects intra and/or extrahepatics biliary tracts and cause neonatal jaundice by serious obstruction of the biliary flow. It is presented with a frequency of 1 by each 10 000 – 15000 born alive. Is not hereditary and the most probable etiology is the progressive inflammatory illness of the biliary tracts. We presented the Good Clinical Practices Guideline for Biliary atresia, approved by consensus in the 4th National Good Clinical Practices Workshop in Pediatric Surgery (Las Tunas, Cuba, March, 2005.
    La atresia de vías biliares es un proceso obstructivo y progresivo de etiología desconocida que afecta las vías biliares intra y/o extrahepáticas y causa ictericia neonatal por obstrucción grave del flujo biliar. Se presenta con una frecuencia de 1 por cada 10 000 – 15 000 nacidos vivos. No es hereditaria y la etiología más probable es la enfermedad inflamatoria progresiva de las vías biliares. Se presenta la Guía de Buenas Prácticas Clínicas para Atresia intestinal yeyunoileal, aprobada por consenso en el 4º Taller Nacional de Buenas Prácticas Clínicas en Cirugía Pediátrica (Las Tunas, marzo 2005.

  9. THORACOSCOPIC REPAIR OF ESOPHAGEAL ATRESIA WITH DISTAL FISTULA IN A NEW BORN: A TECHNICAL CASE REPORT

    Directory of Open Access Journals (Sweden)

    E. Brandigi

    2013-12-01

    Full Text Available Introduction. Esophageal atresia encompasses a group of congenital anomalies comprising of an interruption of the esophageal continuity with or without a persistent communication with the trachea. Esophageal atresia with tracheoesophageal fistula (type C accounts for 85% of all esophageal atresia. Minimally invasive approach to correct esophageal atresia with distal fistula is becoming more generally accepted. The outcome of these technique are critically analyzed and compared with results from open repair. We present one case of type IIIB esophageal atresia treated by a thoracoscopic approach. Case Report. The patient was a 2-days-old infant male, weight 3 kg with esophageal atresia and distal tracheoesophageal fistula without other associated disease. A polidramanios was detected in prenatal age by a prenatal ultrasound evaluation. He underwent to a thoracoscopic repair of the defect. The operation was approached through the right chest using a three-trocar technique (three 5-mm with the patient placed in a three-quarter prone position. The azygos vein was ligated by Ligature device. The fistula was ligated by two resorbable stiches suture and dissected, the proximal esophagus was opened and an anastomosis was made over a 6 Ch nasogastric tube with interrupted and resorbable stiches suture. On the postoperative day 7, gastrografin swallow was performed and oral feeding was started. The patient’s six month upper Gastrointestinal barium studies was normal. Discussion and conclusion. Thoracoscopic repair of esophageal atresia is considered to be one of the more advanced and most difficult pediatric surgical procedures and it undoubtely has necessary an elevated learning curve. The minimally invasive approach was larged accepted in the last ten years also for the well documented sequelae of traditional open repair of esophageal atresia. More experience is needed to determine the exact place of this approach.

  10. Diagnosis and treatment of the congenital intestinal atresia in newborn infants%先天性肠闭锁88例诊疗体会

    Institute of Scientific and Technical Information of China (English)

    邵雷朋; 潘登; 王献良

    2013-01-01

    Objective To study the diagnosis and treatment of the congenital intestinal atresia in newborn infants, discuss the factors affecting prognosis. Methods The clinic data of 88 cases with the congenital intestinal atresia, including the imaging data, pathological types, surgery methods and curative effective, was retrospectively analyzed. Results 76 of 88 cases were cured, total cure rate was 86.3%. 8 cases were operated secondly because of adhesive elius or stoma fistula. 12 cases including 3 cases with volvulus, 2 cases with SBS, 1 case with biliary atresia, 1 case with anal atresia, gave up treatment during or after operation. Conclusion Early diagnosis, reducing the motality of the infants associated other anomalies, choosing the proper operational methods, choosing the proper length intestinal resection, can reduce the complication and degrade the mortality.%  目的探讨新生儿肠闭锁的外科诊断、治疗及降低死亡率的影响因素.方法回顾性分析新生儿肠闭锁88例的临床资料、影像、病理类型、手术治疗及疗效.结果本组治愈76例,治愈率86.3%,其中8例进行了2次手术(包括6例粘连性肠梗阻、2例吻合口瘘).术后死亡及放弃治疗12例(包括合并肠扭转3例,短肠综合征2例,胆道闭锁1例,肛门闭锁1例).结论早诊断,增加患儿出生体重,降低合并严重畸形患儿出生率,选择合适的手术方式,根据闭锁两端肠管的病理改变拟定的肠切除范围可作为临床参考,可减少并发症发生,降低死亡率.

  11. Tracheal Atresia with Segmental Esophageal Duplication: An Unusual Anatomic Arrangement.

    Science.gov (United States)

    Gaerty, Kirsten; Thomas, Joseph T; Petersen, Scott; Tan, Edwin; Kumar, Sailesh; Gardener, Glenn; Armes, Jane

    2016-01-01

    An unusual anatomic configuration of segmental tracheal agenesis/atresia with esophageal duplication on autopsy in a fetus that demised in utero at 29 weeks is reported. The mother was scanned initially for a cardiac anomaly at 20 weeks and on follow-up scan at 27 weeks had polyhydramnios and underwent amnioreduction. The final autopsy diagnosis was vertebral, ano-rectal, cardiac, tracheoesophageal, renal, and limb malformations (VACTERL). We discuss the autopsy findings along with the embryological mechanisms and compare the configuration with Floyd's classification for tracheal agenesis. The difficulties in prenatal diagnosis are discussed. PMID:26367770

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

    Directory of Open Access Journals (Sweden)

    Rossella Angotti

    2016-07-01

    Full Text Available 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.

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

    Science.gov (United States)

    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

  14. Immediate primary anastomosis for isolated oesophageal atresia: A single-centre experience

    Directory of Open Access Journals (Sweden)

    Ibrahim Uygun

    2015-01-01

    Full Text Available Background: Isolated oesophageal atresia without tracheo-oesophageal fistula represents a major challenge for most paediatric surgeons. Here, we present our experience with six neonates with isolated oesophageal atresia who successfully underwent immediate primary anastomosis using multiple Livaditis circular myotomy. Materials and Methods: All six neonates were gross type A isolated oesophageal atresia (6%, from among 102 neonates with oesophageal atresia, treated between January 2009 and December 2013. Five neonates were female; one was male. The mean birth weight was 2300 (range 1700-3100 g. Results: All six neonates successfully underwent immediate primary anastomosis using multiple myotomies (mean 3; range 2-4 within 10 (median 3 days after birth. The gap under traction ranged from 6 to 7 cm. One neonate died of a major cardiac anomaly. Another neonate was lost to follow-up after being well for 3 months. Three anastomotic strictures were treated with balloon dilatation, and four anastomotic leaks were treated conservatively. The mean duration of follow-up was 33 months. Conclusions: To treat isolated oesophageal atresia, an immediate primary anastomosis can be achieved using multiple myotomies. Although, this approach is associated with high complication rates, as are other similar approaches, these complications can be overcome.

  15. Jejunal atresia associated with idiopathic ileal perforation

    Directory of Open Access Journals (Sweden)

    Das P

    2008-01-01

    Full Text Available Jejunoileal atresia is one of the common causes of neonatal intestinal obstruction. Intestinal perforation with meconium peritonitis in the neonatal period, which carries a high mortality rate, is also common. The association of jejunal atresia with idiopathic ileal perforation is very rare.

  16. Pyloric atresia: A report of ten patients

    Directory of Open Access Journals (Sweden)

    Amine Ksia

    2013-01-01

    Full Text Available Pyloric atresia (PA is uncommon. It occurs in 1:100000 live births. Neonates usually present soon after birth with copious non-bilious vomiting. The treatment is surgical and its prognosis is poor, especially, when it is associated with epidermolysis bullosa (EB. The aim of this study was to evaluate the clinical presentation, diagnosis, operative management, post-operative courses, and outcome in infant with PA, based in our cases and literature review. Charts of 10 patients who underwent surgery for PA in the department of paediatric surgery in a Teaching Hospital in Tunisia (Monastir between 1990 and 2012 were reviewed. Data were analysed for demographic, clinical, therapeutic, and prognostic characteristics. The average of age at presentation was 2 days and there were six males and four females. The main presenting symptoms were non-bilious vomiting in 90% of cases. Abdominal X-ray showed gastric dilatation with an absence of gas in the rest of the intestinal tract in 90%, and a pneumoperitoneum in one. The surgical approach was laparotomy in all cases. Gastric perforation was observed in one patient and was completely repaired. The distribution of the anatomic variations was type A in nine cases and type B in one. Five patients underwent excision of the diaphragm and Heineke-Mikulicz pyloroplasty and gastroduodenostomy in the other five cases. Identified associated anomalies were Down′s syndrome in one and EB in 2 (20%, one family has three affected sibling. Post-operative mortality rate was 70%. No standard surgical approach can be adopted a better management of PA or the associated anomalies depends on an early diagnosis and the availability of neonatal intensive care unit.

  17. Anal acoustic reflectometry

    DEFF Research Database (Denmark)

    Mitchell, Peter J; Klarskov, Niels; Telford, Karen J;

    2011-01-01

    Anal acoustic reflectometry is a new technique of assessing anal sphincter function. Five new variables reflecting anal canal function are measured: the opening and closing pressure, the opening and closing elastance, and hysteresis.......Anal acoustic reflectometry is a new technique of assessing anal sphincter function. Five new variables reflecting anal canal function are measured: the opening and closing pressure, the opening and closing elastance, and hysteresis....

  18. Congenital pouch colon in a girl associated with bilateral atresia of cervix uteri and uterus didelphys

    OpenAIRE

    Rajiv Chadha; Manju Puri; Rahul Saxena; Surendrakumar Agarwala; Archana Puri; Subhasis Roy Choudhury

    2013-01-01

    This report describes a girl with congenital pouch colon (CPC), uterus didelphys with septate vagina, and a cloacal anomaly. The girl underwent cloacal reconstruction at the age of 15 months. Subsequently, at puberty, the child had primary amenorrhea with severe cyclic abdominal pain due to endometriosis of both the uteruses and adnexal cysts with hematometra and hematosalpinx. Laparotomy with removal of both uteri and the left fallopian tube was performed. Both uteri had atresia of the cervi...

  19. Congenital Pyloric Atresia with Distal Duodenal Atresia- Role of CT Scan

    Directory of Open Access Journals (Sweden)

    Yogender Singh Kadian

    2014-07-01

    Full Text Available The mainstay of diagnosis of congenital pyloric atresia is by plain X-ray of the abdomen showing a large gas bubble with no gas distally. But very rarely it can be associated with distal duodenal atresia when the baby may present as lump abdomen. In such a situation apart from the X-ray, another radiological investigation is needed to delineate the exact nature of the lump. Since the role of ultrasonography is limited in intestinal pathologies and contrast studies are not informative in atresias, the CT scan is the ideal choice. We had managed a case of pyloric atresia with similar presentation with preoperative CT scan.

  20. Congenital pouch colon in a girl associated with bilateral atresia of cervix uteri and uterus didelphys

    Directory of Open Access Journals (Sweden)

    Rajiv Chadha

    2013-01-01

    Full Text Available This report describes a girl with congenital pouch colon (CPC, uterus didelphys with septate vagina, and a cloacal anomaly. The girl underwent cloacal reconstruction at the age of 15 months. Subsequently, at puberty, the child had primary amenorrhea with severe cyclic abdominal pain due to endometriosis of both the uteruses and adnexal cysts with hematometra and hematosalpinx. Laparotomy with removal of both uteri and the left fallopian tube was performed. Both uteri had atresia of the cervix uteri. This report emphasizes the need for comprehensive evaluation and a long-term management strategy for associated gynecologic anomalies in girls with CPC, especially with regard to patency of the outflow tract.

  1. Biliary Atresia Associated with Polysplenia Syndrome, Dextrocardia, Situs Inversus Totalis and Malrotation of Intestines

    Directory of Open Access Journals (Sweden)

    Praveen Mathur

    2014-01-01

    Full Text Available Biliary atresia (BA is a rare disease and the end result of a destructive, inflammatory cholangiopathy, leading to fibrosis and biliary cirrhosis. It is classified into syndromic variety with various congenital anomalies and non-syndromic (isolated anomaly. We present here a 1-month-old female child with the syndromic variety of BA associated with polysplenia syndrome, dextrocardia, situs inversus totalis and malrotation of intestines. She developed jaundice in the first week of life. Kasai operation was performed but she developed cholangitis and septicemia 2.5 months after surgery and succumbed later.

  2. Congenital pouch colon in a girl associated with bilateral atresia of cervix uteri and uterus didelphys.

    Science.gov (United States)

    Chadha, Rajiv; Puri, Manju; Saxena, Rahul; Agarwala, Surendrakumar; Puri, Archana; Choudhury, Subhasis Roy

    2013-04-01

    This report describes a girl with congenital pouch colon (CPC), uterus didelphys with septate vagina, and a cloacal anomaly. The girl underwent cloacal reconstruction at the age of 15 months. Subsequently, at puberty, the child had primary amenorrhea with severe cyclic abdominal pain due to endometriosis of both the uteruses and adnexal cysts with hematometra and hematosalpinx. Laparotomy with removal of both uteri and the left fallopian tube was performed. Both uteri had atresia of the cervix uteri. This report emphasizes the need for comprehensive evaluation and a long-term management strategy for associated gynecologic anomalies in girls with CPC, especially with regard to patency of the outflow tract. PMID:23798813

  3. Prenatal diagnosis of horseshoe lung and esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Goldberg, Shlomit; Ringertz, Hans [Stanford University School of Medicine, Radiology Department, Stanford, CA (United States); Barth, Richard A. [Stanford University School of Medicine, Radiology Department, Stanford, CA (United States); Lucile Packard Children' s Hospital, Radiology, Palo Alto, CA (United States)

    2006-09-15

    We present a case of horseshoe lung (HL) and esophageal atresia suspected prenatally on US imaging and confirmed with fetal MRI. Prenatal diagnosis of HL and esophageal atresia allowed for prenatal counseling and informed parental decisions. (orig.)

  4. Thoracoscopic elongation of the esophagus in long gap esophageal atresia.

    OpenAIRE

    Zee, D.C.; Vieira Travassos, D.; Kramer, W.L.M.; Tytgat, S. H. A. J.

    2007-01-01

    Long gap esophageal atresia in which a primary anastomosis cannot be achieved remains a challenge. Elongation of the esophagus by traction on the 2 ends has been previously described. With the advent of thoracoscopic repair of esophageal atresia, there have thus far been no reports of thoracoscopic repair of long gap esophageal atresia. This paper describes the first successful repair of long gap esophageal atresia by thoracoscopic traction of the 2 esophageal ends and delayed thoracoscopic a...

  5. Ventriculomegaly with non-CNS anomalies

    International Nuclear Information System (INIS)

    We correlated fetal magnetic resonance (MR) imaging findings with postnatal clinical findings to assess ventriculomegaly with non-CNS anomalies. From 2002 to 2010, 52 fetuses underwent a MRI for evaluation of ventriculomegaly after ultrasonography (US). Ten of the 52 demonstrated anomalies outside the central nervous system (CNS), including trisomy 8, trisomy 18, X-linked hydrocephalus, CHARGE/Potter sequences, VATER association, oral-facial-digital syndrome, esophageal atresia type C, or external auditory canal stenosis. Examinations were performed between 24 and 35 weeks' gestation. MR imaging was performed in a 1.5-tesla unit using a phased-array coil without preparation. Fetal MR imaging showed abnormalities of the kidney, bladder, duodenum, and thumbs but did not permit diagnosis of esophageal atresia type C or craniofacial, anorectal, or skeletal anomalies. Cardiac anomaly was most frequent, but fetal MR imaging did not allow final diagnosis of congenital heart disease. On both US and MR imaging, esophageal atresia type C and anorectal anomaly were undetected; normal rectal signal in a case of anorectal anomaly without urorectal fistula did not lead to suspicion of anomaly. Observation of adducted thumbs on MR imaging is an important sign of X-linked hydrocephalus. The slice area used in this study did not cover polydactyly, which accompanies oral-facial-digital syndrome. US and MR imaging are complementary imaging methods used to evaluate ventriculomegaly. Fetal MR imaging should cover the kidney, bladder, and fingers. Further work is needed to determine the anomalies that can be clearly detected by fetal MR imaging. (author)

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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

  8. Tricuspid atresia and pulmonary atresia in a child with Rubinstein-Taybi syndrome

    Directory of Open Access Journals (Sweden)

    Rohit S Loomba

    2015-01-01

    Full Text Available Rubinstein-Taybi syndrome is a well-characterized condition causing distinctive physical characteristics, intellectual disability, and multiple congenital malformations. Cardiac abnormalities are found in a third of individuals with this condition and usually consist of isolated septal defects or patent ductus arteriosus, although more complex congenital lesions have been described. We present the first reported case of tricuspid atresia and pulmonary atresia with hypoplasia of the right ventricle in the setting of Rubenstein-Taybi syndrome.

  9. Tracheal agenesis with broncho-esophageal fistula in VACTERL / TACRD association

    Directory of Open Access Journals (Sweden)

    Suresh R. S. Mandrekar

    2013-01-01

    Full Text Available Tracheal agenesis (TA is an extremely rare malformation. We report here autopsy findings in a case of TA with bronchoesophageal fistula of Floyd type III. The other malformations present included laryngeal atresia, Right lung hypolobulation, ventricular septal defect in membranous portion, bilateral cystic renal dysplasia, spleninculus, Meckel′s diverticulum, and imperforate anus. The constellations of malformations present in our case have overlapping features with Vertebral anomalies, Anal atresia, Cardiovascular anomalies, Tracheo-esophageal fistula, Esophageal atresia, Renal anomalies, Limb anomalies and Tracheal atresia or laryngo tracheal atresia, Cardiac anomalies, Renal anomalies, Duodenal atresia association described previously in the literature.

  10. Imaging of facial anomalies.

    Science.gov (United States)

    Castillo, M; Mukherji, S K

    1995-01-01

    Anomalies of the face may occur in its lower or middle segments. Anomalies of the lower face generally involve the derivatives of the branchial apparatus and therefore manifest as defects in the mandible, pinnae, external auditory canals, and portions of the middle ears. These anomalies are occasionally isolated, but most of them occur in combination with systemic syndromes. These anomalies generally do not occur with respiratory compromise. Anomalies of the midface may extend from the upper lip to the forehead, reflecting the complex embryology of this region. Most of these deformities are isolated, but some patients with facial clefts, notably the midline cleft syndrome and holoprosencephaly, have anomalies in other sites. This is important because these patients will require detailed imaging of the face and brain. Anomalies of the midface tend to involve the nose and its air-conducting passages. We prefer to divide these anomalies into those with and without respiratory obstruction. The most common anomalies that result in airway compromise include posterior choanal stenoses and atresias, bilateral cysts (mucoceles) of the distal lacrimal ducts, and stenosis of the pyriform (anterior) nasal aperture. These may be optimally evaluated with computed tomography (CT) and generally require immediate treatment to ensure adequate ventilation. Rare nasal anomalies that also result in airway obstruction are agenesis of the pharynx, agenesis of the nose, and hypoplasia of the nasal alae. Agenesis of the nasopharynx and nose are complex anomalies that require both CT and magnetic resonance imaging (MRI). The diagnosis of hypoplasia of the nasal alae is a clinical one; these anomalies do not require imaging studies. Besides facial clefts, anomalies of the nose without respiratory obstruction tend to be centered around the nasofrontal region. This is the site of the most common sincipital encephaloceles. Patients with frontonasal and nasoethmoidal encephaloceles require both

  11. ETIOLOGY, PATHOGENESIS AND MORPHOLOGY OF BILIARY ATRESIA

    Directory of Open Access Journals (Sweden)

    O. E. Iryshkin

    2012-01-01

    Full Text Available Biliary atresia is an inflammatory fibrosing process, which invades both extrahepatic and intrahepatic bile ducts. There are two forms of biliary atresia: embryonic form, associated with another visceral malformations, and perinatal form, which is an isolated disease. Biliary atresia is a heterogeinc disease which may appear as a result of different etiological factors, the most important of them are genetics, viral infection, vascular factors, toxins and disregulation of immune system. The characteristic morphological features of biliaryatresia are: intracellular and ductular cholestasis, ductular proliferation, giant cell transformation, portal and perilobular oedema and/or fibrosis. As the patalogical process progresses, ductopenia appears, and fibrosis turns to cirrhosis. Embryonic form mophologically manifests with ductal plate malformation is, what is an important diagnostic criterion. Pro- gnosis of biliary atresia depends on form of disease, age of patient and type of surgical operation. Today surgical treatment includes two steps: palliative portoenterostomia, performed in neonatal period and further liver trans- plantation. However liver transplantation is more preferable as first and single-step treatment. 

  12. Tracheal Trifurcation Associated With Esophageal Atresia

    Directory of Open Access Journals (Sweden)

    Yogesh Kumar Sarin

    2010-11-01

    Full Text Available We report a newborn with esophageal atresia (EA in whom right tracheal bronchus (TB and a tracheal diverticulum were identified intra-operatively. The right TB was further confirmed on MRI scan performed post-operatively. Such a tracheal trifurcation associated with EA has not been reported hitherto from Indian subcontinent.

  13. Multiple gastrointestinal atresias in two consecutive siblings.

    Science.gov (United States)

    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.

  14. The Artificial Anal Sphincter

    OpenAIRE

    Christiansen, John

    2000-01-01

    The artificial anal sphincter as treatment for end stage anal incontinence was first described in 1987. Published series concern a total of 42 patients, with a success rate of approximately 80%. Infection has been the most serious complication, but a number of technical complications related to the device have also occurred and required revisional procedures in 40% to 60% of the patients. The artificial anal sphincter may be used for the same indications as dynamic graciloplasty except in pat...

  15. [Comprehensive evaluation of long-term results of surgical treatment and principles of postoperative rehabilitation of children with anorectal atresia].

    Science.gov (United States)

    Kaplan, V M; Sitkovskiĭ, N B; Babiĭ, Ia M; Chernienko, Iu L; Gbenu, A S; Kondratskiĭ, N N; Takoeva, T I; Bodnar', V V

    1992-01-01

    The examination and treatment of 22 children at the age of from 2 to 14 years after surgical correction of fistulous forms of atresia of the anus and rectum was carried out. In all the patients, besides of general clinical investigations, the special studies of the large intestine with the use of roentgenologic and functional methods were performed. A scale-table for assessment of a degree of anal incompetence have been developed, criteria for choice of rational tactics for rehabilitation of the patients established. PMID:1453622

  16. Anal condyloma acuminatum.

    Science.gov (United States)

    McCutcheon, Tonna

    2009-01-01

    Anal condyloma acuminatum is a human papillomavirus (HPV) that affects the mucosa and skin of the anorectum and genitalia. Anal condyloma acuminatum is the most commonly diagnosed sexually transmitted disease in the United States. To date, there are more than 100 HPV types, with HPV-6, HPV-10, and HPV-11 predominately found in the anogenital region and causing approximately 90% of genital warts. Risk factors for anal condyloma acuminatum include multiple sex partners, early coital age, anal intercourse, and immunosuppression. Transmission occurs by way of skin-to-skin contact through sexual intercourse, oral sex, anal sex, or other contact involving the genital area. The virus may remain latent for months to years until specific mechanisms cause production of viral DNA, leading to the presentation of anal condyloma acuminatum.Patients with anal condyloma acuminatum may be asymptomatic or present with presence of painless bumps, itching, and discharge or bleeding. It is not uncommon to have involvement of more than one area, and multiple lesions may also be present and extend into the anal canal or rectum. To date, there is no serologic testing or culture to detect anal condyloma acuminatum; therefore, diagnosis is made clinically or by detection of HPV DNA. Multiple factors determine the choice of treatment, which may range from patient-applied medications to surgical intervention. Despite treatment choice, recurrence rates are high, indicating the importance of patient education on prevention of HPV infection and reinfection. Unfortunately, at this time, no cure exists for anal condyloma acuminatum; however, recently Gardasil and Cervarix (in Australia only) vaccines have become available and are showing promising results. PMID:19820442

  17. Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation

    Energy Technology Data Exchange (ETDEWEB)

    Githu, Tangayi [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Radiology of Huntsville, P.C., Huntsville, AL (United States); Merrow, Arnold C.; Lee, Jason K. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Garrison, Aaron P. [Cincinnati Children' s Hospital Medical Center, Department of Surgical Services, Division of Pediatric General and Thoracic Surgery, Cincinnati, OH (United States); Akron Children' s Hospital, Pediatric Surgery, Akron, OH (United States); Brown, Rebeccah L. [Cincinnati Children' s Hospital Medical Center, Department of Surgical Services, Division of Pediatric General and Thoracic Surgery, Cincinnati, OH (United States)

    2014-03-15

    Hereditary multiple intestinal atresia (HMIA) is an extremely uncommon cause of congenital bowel obstruction. The morbidity and mortality of this disease differ significantly from those of isolated intestinal atresias and non-hereditary forms of multiple intestinal atresia. Most notably, despite successful operative repairs of the atresias found in this disease, HMIA maintains a 100% lethality rate from continued post-operative intestinal failure and an associated severe immunodeficiency. We present a case of HMIA evaluated with fetal MRI and subsequently diagnosed by a combination of corroborative postnatal imaging with surgical exploration and pathological examination. (orig.)

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

    Science.gov (United States)

    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

  19. Etiological study on isolated esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Szendrey, T.; Danyi, G.; Czeizel, A.

    1985-01-01

    A study group of 160 index patients with isolated esophageal atresia, a control group of 160 matched healthy controls, and the first-degree relatives of patients and controls were examined; epidemiological, family planning, teratological, and genetic data were obtained by personal interview in the study and control groups. One half of the index patients were male. Intrauterine growth retardation, a higher proportion of mothers under 19 or over 30 years of age, and less skilled professions of the parents were found in the study group. There were more extramarital conceptions, more pregnancies in spite of the use of contraceptive pills, and more delayed conceptions in index patients mothers. The teratogens studied did not have an obvious pathological effect here. The sib occurrence of isolated esophageal atresia was 0.43%, which did not correspond well to the expected figure of 1.34% based on the polygenic model.

  20. Is AMH a regulator of follicular atresia?

    OpenAIRE

    Seifer, David B.; Merhi, Zaher

    2014-01-01

    We discuss the hypothesis that AMH is an intraovarian regulator that inhibits follicular atresia within the human ovary. Several indirect lines of evidence derived from clinical and basic science studies in a variety of different patient populations and model systems collectively support this hypothesis. Evidence presented herein include 1) timing of onset of menopause in women with polycystic ovary syndrome, 2) site of cellular origin and timing of AMH production, 3) AMH’s influence on other...

  1. Ultrasonographic findings of type IIIa biliary atresia

    International Nuclear Information System (INIS)

    To describe the ultrasonographic (US) findings of type IIIa biliary atresia. We retrospectively reviewed a medical database of patients pathologically confirmed to have biliary atresia, Kasai type IIIa, between January 2002 and May 2013 (n=18). We evaluated US findings including the visible common bile duct (CBD), triangular cord thickness, gallbladder size and shape, and subcapsular flow on color Doppler US; laboratory data; and pathological hepatic fibrosis grades. We divided them into two groups-those with visible (group A) and invisible (group B) CBD on US-and compared all parameters between the two groups. CBD was visible on US in five cases (27.8%; group A) and invisible in 13 cases (72.2%; group B). US was performed at an earlier age in group A than in group B (median, 27 days vs. 60 days; P=0.027) with the maximal age of 51 days. A comparison of the US findings revealed that the triangular cord thickness was smaller (4.1 mm vs. 4.9 mm; P=0.004) and the gallbladder length was larger (20.0 mm vs. 11.7 mm; P=0.021) in group A. The gallbladder shape did not differ between the two groups, and the subcapsular flow was positive in all cases of both groups. There was no significant difference in the laboratory data between the two groups. Upon pathological analysis, group A showed low-grade and group B showed low- to high-grade hepatic fibrosis. When CBD is visible on US in patients diagnosed with type IIIa biliary atresia, other US features could have a false negative status. A subcapsular flow on the color Doppler US would be noted in the type IIIa biliary atresia patients.

  2. Esophageal atresia: Factors influencing survival - Experience at an Indian tertiary centre

    Directory of Open Access Journals (Sweden)

    Tandon R

    2008-01-01

    Full Text Available Objective: To study the clinical profile of the cases of esophageal atresia (EA and/or tracheoesophageal fistula (TEF and various factors affecting the surgical and early postoperative management and their outcome. Materials and Methods: A prospective analysis of 127 cases of EA from February 2004 to May 2006 was performed. Waterston prognostic criteria were used for grading. Results: EA with TEF was the commonest type in 117 cases (92%. Associated congenital anomalies were present in 52 (41% patients, the commonest being the cardiac anomalies, which was followed by the gastrointestinal anomalies. VACTERL was found in 6 (5% cases. Prematurity, associated congenital anomalies, gap between esophageal ends and preoperative respiratory status were the significant factors affecting the survival ( P = < 0.001. Primary extrapleural repair was the surgical approach in most of the patients. Azygos vein was preserved in 46 cases and no retropleural drainage was used in 27 cases. Staged procedures were performed in 19 cases, including 6 cases of isolated esophageal atresia. Pneumonitis and sepsis were the most common early postoperative complications (42%. Hypoxia and cardiorespiratory arrest were the most common causes of mortality (11 cases. Anastomotic leak complicated 13 cases, including 9 major and 4 minor leaks. Major leak followed by sepsis caused 7 deaths. Survival as per Waterston criteria was 100% in group A, 83% in group B and 22% in group C. Conclusion Factors affecting the survival are major or life-threatening associated anomalies, long gap, pneumonia and sepsis at presentation or that acquired during hospitalization and major leaks. The high incidence of low birth weight, delayed diagnosis, poor referral, low-socio economic status and lack of advanced neonatological back up are important contributory factors to poor outcome.

  3. Bone conduction hearing in congenital aural atresia.

    Science.gov (United States)

    Zhang, Lichun; Gao, Na; Yin, Yanbo; Yang, Lin; Xie, Youzhou; Chen, Ying; Dai, Peidong; Zhang, Tianyu

    2016-07-01

    Previous researches focusing on BC hearing mechanisms proved that the two routes, (1) EAC sound radiation and (2) inertial of ossicular chain, partially contribute to normal BC hearing. Therefore, the BC hearing for those patients with congenital aural atresia should partially decrease theoretically due to their abnormal anatomy. However, there are not many studies which mention these patients' BC hearing up till now. The objective of this study is to investigate congenital aural atresia patient's BC hearing by analysis of pre-surgical audiogram and to study their potential BC hearing mechanisms using animal modeling and their ABR measurements. The study methoed involves analyzing 75 patients' pre-operative audiogram. Then we produced an animal model by surgery to measure their BC hearing threshold changes. Clinical data showed that those patients had some BC hearing loss; and there were 25 cases (25/75, 33.3 %) which present with typical Carhart's Notch. The animal experiments proved that inertia of ossicular chain contribute to partial BC hearing, which demonstrated that the inertia produced more affects on high frequencies by comparing with low frequencies. The patients with congenital aural atresia present BC hearing loss, which could be mainly ascribed to the absence of inertia of ossicular chain. PMID:26205153

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

  6. Congenital biliary atresia: liver injury begins at birth

    DEFF Research Database (Denmark)

    Makin, Erica; Quaglia, Alberto; Kvist, Nina;

    2009-01-01

    BACKGROUND: The timing of onset of liver injury in biliary atresia (BA) is not known, although in approximately 10% of cases, biliary pathologic condition associated with the biliary atresia splenic malformation syndrome must begin well before birth. METHODS: The study involved retrospective case...

  7. European biliary atresia registries: summary of a symposium

    DEFF Research Database (Denmark)

    Petersen, C.; Harder, D.; Abola, Z.;

    2008-01-01

    Biliary atresia (BA) is a rare but potentially devastating disease. The European Biliary Atresia Registry (EBAR) was set up to improve data collection and to develop a pan-national and interdisciplinary strategy to improve clinical outcomes. From 2001 to 2005, 100 centers from 22 countries...

  8. Thoracoscopic elongation of the esophagus in long gap esophageal atresia.

    NARCIS (Netherlands)

    van der Zee, D.C.; Vieira Travassos, D.; Kramer, W.L.M.; Tytgat, S.H.A.J.

    2007-01-01

    Long gap esophageal atresia in which a primary anastomosis cannot be achieved remains a challenge. Elongation of the esophagus by traction on the 2 ends has been previously described. With the advent of thoracoscopic repair of esophageal atresia, there have thus far been no reports of thoracoscopic

  9. Jejunum for bridging long-gap esophageal atresia

    NARCIS (Netherlands)

    Bax, Klaas (N) M. A.

    2009-01-01

    OBJECTIVE: Exploring pros and cons of bridging long-gap esophageal atresia with an orthotopic jejunal pedicle graft. Retrospective series of 19 patients. METHODS: From 1988 through 2005, 19 patients with long-gap esophageal atresia received a jejunal graft. Median age at reconstruction was 76 days.

  10. HIV-associated anal cancer

    OpenAIRE

    Newsom-Davis, Thomas; Bower, Mark

    2010-01-01

    HIV-associated anal carcinoma, a non-AIDS-defining cancer, is a human papillomavirus-associated malignancy with a spectrum of preinvasive changes. The standardized incidence ratio for anal cancer in patients with HIV/AIDS is 20-50. Algorithms for anal cancer screening include anal cytology followed by high-resolution anoscopy for those with abnormal findings. Outpatient topical treatments for anal intraepithelial neoplasia include infrared coagulation therapy, trichloroacetic acid, and imiqui...

  11. Anal cancer; Cancer du canal anal

    Energy Technology Data Exchange (ETDEWEB)

    Fesneau, M.; Champeaux-Orange, E. [Service de radiotherapie, Centre regional universitaire de cancerologie Henry-S.-Kaplan CHU de Tours, Hopital Bretonneau, 37 - Tours (France); Champeaux-Orange, E. [Service d' oncologie-radiotherapie, Centre hospitalier regional d' Orleans, 45 - Orleans (France); Hennequin, C. [Service de cancerologie-radiotherapie, hopital Saint-Louis, 75 - Paris (France)

    2010-07-01

    Anal canal epidermoid carcinomas represent 1.2% of digestive cancers and 6% of ano-rectal cancers. For localized diseases, the treatment is based on radiotherapy with or without chemotherapy (5-FU and cisplatin or mitomycin), according to tumour and nodal extension. The recommended treatment dose is 45 Gy in the anal canal, the mesorectum, para-rectal lymph nodes, and inguinal lymph nodes. An additional dose of 15 to 20 Gy is delivered in the initial tumour for good responders. Salvage surgery is necessary in case of poor response. The organs at risk to be considered are bladder, femur heads, small intestine and vulva. The objective of this work is to summarize the epidemiological and radio-anatomic and prognostic characteristics of this tumour. The conformal radiotherapy technique is illustrated by a case report. (authors)

  12. Incidental Finding of Inferior Vena Cava Atresia Presenting with Deep Venous Thrombosis following Physical Exertion

    Directory of Open Access Journals (Sweden)

    Shalini Koppisetty

    2015-01-01

    Full Text Available 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.

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

    Science.gov (United States)

    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 (basal atretic follicles were originally identified incorrectly in the literature. Thus, on the basis of the results of

  14. Expanded spectrum of limb anomalies in the VATER association

    Energy Technology Data Exchange (ETDEWEB)

    Fernbach, S.K.; Glass, R.B.J.

    1988-04-01

    The radiographs of 230 children who had undergone neonatal surgery for imperforate anus and/or esophageal atresia/tracheoesophageal fistula were reviewed. Of the 31 children with limb anomalies thus detected, the 24 who had no radiologic or laboratory evidence of chromosomal abnormality form the basis of this report. In 16 children the limb anomalies fell within the commonly described spectrum of the VATER association. In the other 8 children and in 3 of the children with typical VATER limb anomalies additional anomalies were encountered: Sprengel deformity (2), hypoplasia of the humerus (3), radioulnar synostosis (1), midline anomalies of the hand (1), absence of the pubis, femur, tibia, and fibula and two rays of the foot (1), and other foot deformities. Subtle anomalies of the hand were common and included: clinodactyly, syndactyly, shortening of the middle phalanx of the fifth digit, and rotary malposition of the digits.

  15. Obstetric anal sphincter injuries

    Institute of Scientific and Technical Information of China (English)

    Remon Keriakos; Deepa Gopinath

    2015-01-01

    Obstetric anal sphincter injuries can be associated with significant short and long term consequences causing devastating impacts on the quality of lives of young, otherwise healthy women. The major consequence is anal incontinence which may be short or long term and vary in severity. The other consequences include pain, infection, dyspareunia and sexual dysfunction. This may in turn result in considerable economic burden to health care providers and patients. It also has an implication on future deliveries. Although it can never be eliminated, it can be reduced by improving practice, training and provision of high quality multidisciplinary care in order to reduce long-term morbidity. Obstetric anal sphincter injuries are also a source of litigation which can be distressing to both patients and clinicians. The aim of this review article is to explore the available evidence on epidemiology, strategies for preventions, prognosis and also how to deal with governance issues.

  16. [Anal intraepithelial neoplasia].

    Science.gov (United States)

    de Parades, Vincent; Fathallah, Nadia; Barret, Maximilien; Zeitoun, Jean-David; Lemarchand, Nicolas; Molinié, Vincent; Weiss, Laurence

    2013-01-01

    Anal intraepithelial lesions are caused by chronic infection with oncogenic types of human papillomavirus. Their incidence and prevalence are increasing, especially among patients with HIV infection. Their natural history is not well known, but high-grade intraepithelial lesions seem to have an important risk to progress to squamous cell carcinoma. Their treatment can be achieved by many ways (surgery, coagulation, imiquimod, etc.) but there is a high rate of recurrent lesions. Pretherapeutic evaluation should benefit from high-resolution anoscopy. Periodic physical examination and anal cytology may probably be interesting for screening the disease among patients with risk factors. Vaccine against oncogenic types of papillomavirus may prevent the development of anal intraepithelial neoplasia. PMID:23122632

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

    Science.gov (United States)

    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

  18. Atresia biliar: continuamos operando tarde Biliary atresia: we still operate too late

    OpenAIRE

    Carlos O. Kieling; dos Santos, Jorge L.; Sandra M.G. Vieira; Cristina T Ferreira; Ana R. R. Linhares; Andréa L. Lorentz; da Silveira, Themis R.

    2008-01-01

    OBJETIVO: Analisar a idade na cirurgia de crianças com atresia biliar e a sobrevida sem necessidade de transplante de fígado. O estudo foi realizado no Hospital de Clínicas de Porto Alegre. MÉTODOS: Foram revisados os prontuários dos pacientes operados entre 1982 e 2007, residentes no Rio Grande do Sul. RESULTADOS: Dos 112 casos estudados de crianças com atresia biliar, 38 (33,9%) ocorreram de 1982 a 1989, 46 (41,1%) de 1990 a 1999 e 28 (25,0%) a partir de 2000. Em 12 (10,7%) casos, não foi r...

  19. Spectrum of bronchopulmonary anomalies associated with tracheoesophageal malformations

    Energy Technology Data Exchange (ETDEWEB)

    Benson, J.E.; Fletcher, B.D.; Olsen, M.

    1985-09-01

    The combination of tracheoesophageal and pulmonary malformations is unusual and reportedly carries a high mortality. We have observed six patients with esophageal atresia and tracheoesophageal fistula and one with a bronchoesophageal fistula who had associated bronchopulmonary anomalies ranging from lobar hypoplasia and agenesis to unilateral pulmonary hypoplasia or agenesis. All of the pulmonary malformations were right-sided. Vertebral or rib anomalies were present in five patients, congenital heart disease in two, imperforate anus in one and one patient had radial aplasia and Pierre Robin syndrome. The bronchopulmonary anomalies complicated the surgical care of tracheoesophageal malformations and required radiologic differentiation from aspiration pneumonia and atelectasis. Six of the seven patients survived. Mortality and morbidity were related to complications and associated cardiac anomalies as well as severity of the lung anomaly.

  20. UNILATERAL CHOANAL ATRESIA - PRESENTATION IN VARIED AGE GROUP S

    Directory of Open Access Journals (Sweden)

    Sajitha

    2015-03-01

    Full Text Available Choanal atresia is an uncommon and poorly recognized cause of unilateral nasal obstruction in adults. We discuss here two cases of unilateral choanal atresia , one adult and a child. Both of them had nasal obstruction as their chief complaint. Both patients underwent a Trans nasal endoscopic excision of choanal atresia with assi stance of powered instrument and repair of the choana with stent. Both the patients were followed up regularly for a period of one year and were found to be symptom free with a well healed and patent choana. These case reports highlight the possibility of considering choanal atresia as a differential diagnosis in all patients presenting with unilateral nasal obstruction. CT scan and Nasal endoscopy are the investigations of choice. Trans nasal endoscopic approach with stenting provides an excellent method o f management.

  1. Genetics Home Reference: epidermolysis bullosa with pyloric atresia

    Science.gov (United States)

    ... cause epidermolysis bullosa with pyloric atresia. J Invest Dermatol. 2005 Jan;124(1):111-5. Citation on ... on Epidermolysis Bullosa, Santiago, Chile, 2005. Int J Dermatol. 2007 Aug;46(8):781-94. Citation on ...

  2. Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula: A case report with prenatal diagnosis

    Directory of Open Access Journals (Sweden)

    Go Miyano

    2015-01-01

    Full Text Available We describe herein a case of unilateral pulmonary agenesis (PA with oesophageal atresia (EA/tracheoesophageal fistula (TEF that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4 th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.

  3. Solitary median maxillary central incisor, short stature, choanal atresia/midnasal stenosis (SMMCI) syndrome.

    Science.gov (United States)

    Hall, R K; Bankier, A; Aldred, M J; Kan, K; Lucas, J O; Perks, A G

    1997-12-01

    This article describes a series of 21 consecutive cases, each involving a solitary median maxillary central incisor; the patients were seen in the Department of Dentistry or the Victorian Clinical Genetics Unit, Murdoch Institute, at the Royal Children's Hospital, Melbourne, from 1966 to 1997. The spectrum of anomalies and associated features present in these cases--solitary median maxillary central incisor, choanal atresia, and holoprosencephaly--is described, and the literature related to the features, including genetic studies in these conditions, is reviewed. We relate our findings in these cases to current knowledge of developmental embryology. It is hoped that the findings, together with our interpretation of them, will help to clarify understanding of solitary median maxillary central incisor syndrome. This syndrome was previously considered a simple midline defect of the dental lamina, but it is now recognized as a possible predictor of holoprosencephalies of varying degrees in the proband, in members of the proband's family, and in the family's descendants. PMID:9431535

  4. Do retractile testes have anatomical anomalies?

    Science.gov (United States)

    Anderson, Kleber M.; Costa, Suelen F.; Sampaio, Francisco J.B.; Favorito, Luciano A.

    2016-01-01

    ABSTRACT Objectives: To assess the incidence of anatomical anomalies in patients with retractile testis. Materials and Methods: We studied prospectively 20 patients (28 testes) with truly retractile testis and compared them with 25 human fetuses (50 testes) with testis in scrotal position. We analyzed the relations among the testis, epididymis and patency of the processus vaginalis (PV). To analyze the relations between the testis and epididymis, we used a previous classification according to epididymis attachment to the testis and the presence of epididymis atresia. To analyze the structure of the PV, we considered two situations: obliteration of the PV and patency of the PV. We used the Chi-square test for contingency analysis of the populations under study (p patent processus vaginalis and epididymal anomalies. PMID:27564294

  5. Surgical Outcomes in Esophageal Atresia and Tracheoesophageal Fistula: A Comparison between Primary and Delayed Repair

    Directory of Open Access Journals (Sweden)

    H Davari

    2006-01-01

    Full Text Available Background: The purpose of this study was to investigate outcomes of surgical repair of esophageal atresia (EA or tracheoesophageal fistula (TEF in newborns, with respect to incidence of death and other complications in early or late operations. Methods: Charts of all 80 infants with EA/TEF, operated in Alzahra hospital (A tertiary hospital of Isfahan University of Medical Sciences from 2002 to 2004 were reviewed. Patients were designed in two groups as, primary and delayed repair groups. Patients demographics, frequency of associated anomalies, and details of management and outcomes were studied. Results: There were 48 male and 32 female patients with a frequency of 28(35% preterm infant and mean birth weight of 2473±595 g. Overall survival rate was 71.2%. Mortality rate in delayed repair group was significantly higher than the other one (22.5% vs. 6.3% but with matching, according to full term/preterm proportion, the significant differences were failed. Female sex and being preterm were the most powerful predictors of death (nearly odds ratio=7 for both. Conclusion: in this study mortality and complications rates are higher in delayed repair than early one, although our data proposed that in absence of sever life threatening anomalies the most important factor for death is gestational age and female sex, and primary repair is opposed to it. Although mortality rate and complications are equal in two strategies, with matching cases for being preterm, but primary repair stays the better choice due to economic considerations. Keywords: tracheoesophageal fistula, esophageal atresia, delayed repair, primary repair, outcome

  6. A Rare Association of Congenital Diaphragmatic Hernia with Lower Esophageal Atresia and Perforation

    OpenAIRE

    Narendra Kumar Are; Nagarjuna, K.; Lavanya Kannaiyan

    2010-01-01

    Congenital diaphragmatic hernia is known to be associated with esophageal atresia, which is a rare association. We report a rare occurrence of congenital diaphragmatic hernia and lower esophageal atresia.

  7. A Rare Association of Congenital Diaphragmatic Hernia with Lower Esophageal Atresia and Perforation

    Directory of Open Access Journals (Sweden)

    Narendra Kumar Are

    2010-01-01

    Full Text Available Congenital diaphragmatic hernia is known to be associated with esophageal atresia, which is a rare association. We report a rare occurrence of congenital diaphragmatic hernia and lower esophageal atresia.

  8. Hereditary Multiple Gastrointestinal Atresia associated with Choledochal Cyst: A Rare Entity with Management Dilemma

    Directory of Open Access Journals (Sweden)

    P Raj

    2014-07-01

    Full Text Available Multiple intestinal atresias are rare and its treatment is challenging. Here, we present a case of multiple gastrointestinal atresia associated with choledochal cyst posing us a surgical challenge.

  9. Case report: Upper neck pouch sign in the antenatal diagnosis of esophageal atresia

    OpenAIRE

    Garg, Mukesh Kumar

    2009-01-01

    Prenatal diagnosis of esophageal atresia remains a challenge for the imaging consultant. On antenatal USG, the finding of an absent or small stomach in the setting of polyhydramnios used to be considered suspicious of esophageal atresia. However, these findings have a low positive predictive value. The upper neck pouch sign is another sign that helps in the antenatal diagnosis of esophageal atresia. In this paper, I report a case of esophageal atresia that was diagnosed on USG at 27 weeks of ...

  10. The Harlequin phenomenon after thoracoscopic repair of esophageal atresia and tracheoesophageal fistula: Is there any coincidence?

    Directory of Open Access Journals (Sweden)

    Katarzyna A. Maścianica

    2015-11-01

    Full Text Available The experience and number of endoscopic surgery procedures applied in newborns are constantly growing, showing efficacy in many thoracic and abdominal conditions. However, there is an ongoing debate concerning the safety of this technique and the influence on the developing organism. Due to the relatively recent introduction of these methods in the therapy of congenital anomalies, we still have quite little knowledge about their long-term sequelae. The Harlequin phenomenon is a complex, little known and probably heterogeneous anomaly. It involves episodes of sharply demarcated erythema with the dividing line running exactly along the midline. We observe the appearance of episodes of unilateral flushing and sweating on the left half of the body in children who have been operated on using the thoracoscopic approach in the first days of life, due to esophageal atresia and distal tracheooesophageal fistula (EA/TEF, which are very similar to the Harlequin phenomenon. During long term follow-up visits, parents of 2 out of 55 children, who had been operated on during the period 2005–2011, turned our attention to these unusual symptoms. To our knowledge, this is the first report of the Harlequin phenomenon following esophageal surgery. The aim of the study is to analyze the possible pathogenesis of the observed anomaly, in the context of the existing congenital defect, and the operative technique used.

  11. Anal anatomy and normal histology.

    Science.gov (United States)

    Pandey, Priti

    2012-12-01

    The focus of this article is the anatomy and histology of the anal canal, and its clinical relevance to anal cancers. The article also highlights the recent histological and anatomical changes to the traditional terminology of the anal canal. The terminology has been adopted by the American Joint Committee on Cancer, separating the anal region into the anal canal, the perianal region and the skin. This paper describes the gross anatomy of the anal canal, along with its associated blood supply, venous and lymphatic drainage, and nerve supply. The new terminology referred to in this article may assist clinicians and health care providers to identify lesions more precisely through naked eye observation and without the need for instrumentation. Knowledge of the regional anatomy of the anus will also assist in management decisions.

  12. Changes of smooth muscle contractile filaments in small bowel atresia

    Institute of Scientific and Technical Information of China (English)

    Stefan Gfroerer; Henning Fiegel; Priya Ramachandran; Udo Rolle; Roman Metzger

    2012-01-01

    AIM:To investigate morphological changes of intestinal smooth muscle contractile fibres in small bowel atresia patients.METHODS:Resected small bowel specimens from small bowel atresia patients (n =12) were divided into three sections (proximal,atretic and distal).Standard histology hematoxylin-eosin staining and enzyme immunohistochemistry was performed to visualize smooth muscle contractile markers α-smooth muscle actin (SMA) and desmin using conventional paraffin sections of the proximal and distal bowel.Small bowel from agematched patients (n =2) undergoing Meckel's diverticulum resection served as controls.RESULTS:The smooth muscle coat in the proximal bowel of small bowel atresia patients was thickened compared with control tissue,but the distal bowel was unchanged.Expression of smooth muscle contractile fibres SMA and desmin within the proximal bowel was slightly reduced compared with the distal bowel and control tissue.There were no major differences in the architecture of the smooth muscle within the proximal bowel and the distal bowel.The proximal and distal bowel in small bowel atresia patients revealed only minimal differences regarding smooth muscle morphology and the presence of smooth muscle contractile filament markers.CONCLUSION:Changes in smooth muscle contractile filaments do not appear to play a major role in postoperative motility disorders in small bowel atresia.

  13. Biliary atresia: Clinical advances and perspectives.

    Science.gov (United States)

    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

  14. Anal incontinence in women with recurrent obstetric anal sphincter rupture

    DEFF Research Database (Denmark)

    Bøgeskov, Reneé; Nickelsen, Carsten Nahne Amtoft; Secher, Niels Jørgen

    2015-01-01

    UNLABELLED: Abstract Objectives: To determine the risk of recurrent anal sphincter rupture (ASR), and compare the risk of anal incontinence (AI) after recurrent ASR, with that seen in women with previous ASR who deliver by caesarean section or vaginally without sustaining a recurrent ASR. METHODS...

  15. Pharmacomechanical thrombectomy and catheter-directed thrombolysis of acute lower extremity deep venous thrombosis in a 9-year-old boy with inferior vena cava atresia.

    Science.gov (United States)

    Hamidian Jahromi, Alireza; Coulter, Amy H; Bass, Patrick; Zhang, Wayne W; Tan, Tze-Woei

    2015-04-01

    Lower extremity deep venous thrombosis (DVT) is uncommon in the pediatric population, but it can be associated with severe symptoms and potential long-term morbidity secondary to post-thrombotic syndrome. Inferior vena cava (IVC) atresia can predispose a patient to the development of extremity DVT. There is no clear consensus on optimal management of extensive extremity DVT in pediatric patients, especially in patients with IVC anomalies. We report a case of iliofemoral DVT in a 9-year-old boy with IVC atresia and presumed protein S deficiency that was treated successfully using pharmacomechanical thrombectomy and catheter-directed thrombolysis. He was maintained on long-term anticoagulation and remained symptom free at 6 months' follow-up.

  16. Atresia biliar: continuamos operando tarde Biliary atresia: we still operate too late

    Directory of Open Access Journals (Sweden)

    Carlos O. Kieling

    2008-10-01

    Full Text Available OBJETIVO: Analisar a idade na cirurgia de crianças com atresia biliar e a sobrevida sem necessidade de transplante de fígado. O estudo foi realizado no Hospital de Clínicas de Porto Alegre. MÉTODOS: Foram revisados os prontuários dos pacientes operados entre 1982 e 2007, residentes no Rio Grande do Sul. RESULTADOS: Dos 112 casos estudados de crianças com atresia biliar, 38 (33,9% ocorreram de 1982 a 1989, 46 (41,1% de 1990 a 1999 e 28 (25,0% a partir de 2000. Em 12 (10,7% casos, não foi realizada a portoenterostomia. A idade na cirurgia variou de 25 a 297 dias (mediana: 80,5; IIQ25-75: 61,3-109,0 dias; em 20,5% dos casos, a idade das crianças foi menor do que 60 dias. Não houve diferença na idade, no momento do diagnóstico, entre as 3 décadas. Os pacientes do interior do estado (mediana: 87,0; IIQ25-75: 69,0-115,0 dias foram encaminhados significativamente (p = 0,007 mais tarde do que os da região metropolitana de Porto Alegre (RS (mediana: 68,0; IIQ25-75: 55,5-98,0 dias. A proporção de pacientes com menos de 60 dias foi significativamente menor (p = 0,013 nos oriundos do interior. A sobrevida com fígado nativo do total dos pacientes foi de 46,2% em 2 anos, diminuindo progressivamente até 15,3% em 20 anos. Os pacientes operados com menos de 60 dias tiveram maior sobrevida com fígado nativo (log rank OBJECTIVE: To analyze the age at surgery for children with biliary atresia and their survival periods without need for liver transplantation. The study was performed at Hospital de Clínicas de Porto Alegre, in Porto Alegre, state of Rio Grande do Sul, Brazil. METHODS: The medical records of patients operated between 1982 and 2007 who were residents of the state of Rio Grande do Sul were reviewed. RESULTS: Of the 112 cases of children with biliary atresia studied, 38 (33.9% occurred between 1982 and 1989, 46 (41.1% between 1990 and 1999 and 28 (25.0% after 2000. Portoenterostomy was not performed for 12 cases (10.7%. Age at surgery

  17. Hypertrophied anal papillae and fibrous anal polyps, should they be removed during anal fissure surgery?

    Institute of Scientific and Technical Information of China (English)

    Pravin J. Gupta

    2004-01-01

    AIM: Hypertrophied anal papillae and fibrous anal polyps are not given due importance in the proctology practice.They are mostly ignored being considered as normal structures. The present study was aimed to demonstrate that hypertrophied anal papillae and fibrous anal polyps could cause symptoms to the patients and that they should be removed in treatment of patients with chronic fissure in anus.METHODS: Two groups of patients were studied. A hundred patients were studied in group A in which the associated fibrous polyp or papillae were removed by radio frequency surgical device after a lateral subcutaneous sphincterotomy for relieving the sphincter spasm. Another group of a hundred patients who also had papillae or fibrous polyps, were treated by lateral sphincterotomy alone. They were followed up for one year.RESULTS: Eighty-nine percent patients from group A expressed their satisfaction with the treatment in comparison to only 64% from group B who underwent sphincterotomy alone with the papillae or anal polyps left untreated. Group A patients showed a marked reduction with regard to pain and irritation during defecation (P= 0.0011),pricking or foreign body sensation in the anus (P = 0.0006)and pruritus or wetness around the anal verge (P = 0.0008).CONCLUSION: Hypertrophied anal papillae and fibrous anal polyps should be removed during treatment of chronic anal fissure. This would add to effectiveness and completeness of the procedure.

  18. Videofluoroscopy of deglutition in children after repair of esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Hoermann, M.; Pokieser, P.; Scharitzer, M.; Memarsadeghi, M.; Partik, B. [Univ. Hospital, Vienna (Austria). Dept. of Radiology; Pumberger, W. [Univ. Hospital, Vienna (Austria). Dept. of Pediatric Surgery; Ekberg, O. [Univ. Hospital MAS, Malmoe (Sweden). Dept. of Diagnostic Radiology

    2002-09-01

    Purpose: To evaluate the functional disorders of the oral and pharyngeal phases of deglutition after repair of esophageal atresia in children. Material and Methods: 19 children (10 girls, 9 boys, mean age 22 months) underwent videofluoroscopy of deglutition after repair of esophageal atresia. The videofluoroscopic studies were assessed according to functional and morphological changes in the oral, pharyngeal and esophageal phases. The persistence of radiologic findings on videofluoroscopy was determined. Results: The oral phase was normal in all patients. The main functional disorder of the pharyngeal phase was aspiration in 7 (37%) children. A completely normal deglutition in the pharyngeal and esophageal phases was not seen in any patient. Conclusion: Videofluoroscopy after repair of esophageal atresia is helpful in differentiation of functional and morphological disorders that can lead to prandial aspiration and have an influence on the decision about continued therapy.

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

    Energy Technology Data Exchange (ETDEWEB)

    Alamo, Leonor; Meuli, Reto [University Hospital of Lausanne (CHUV) and University of Lausanne (UNIL), Department of Diagnostic and Interventional Radiology, Lausanne (Switzerland); Vial, Yvan [University Hospital of Lausanne (CHUV) and University of Lausanne (UNIL), Department of Obstetrics and Gynecology, Lausanne (Switzerland); Gengler, Carole [University Hospital of Lausanne (CHUV) and University of Lausanne (UNIL), Department of Pathology, Lausanne (Switzerland)

    2016-03-15

    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. (orig.)

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

    Science.gov (United States)

    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.

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

    International Nuclear Information System (INIS)

    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. (orig.)

  2. Recurrent Abortion in a Patient with Ebstein Anomaly

    Directory of Open Access Journals (Sweden)

    Abdolmohammad Ranjbar

    2014-04-01

    Full Text Available Most maternal cardiac disease in Western societies is now congenital in origin. This relates to the significant improvements in congenital cardiac surgery during the last years. Some patients will present for the first time in pregnancy with symptoms and learn that they have congenital heart disease. So all patients should have a detailed evaluation and appropriate counseling before pregnancy. Ebstein anomaly is a rare and complex congenital heart disorder occurring in 1 per 200 000 live births and, first described by a German physician Wilhelm Ebstein in 1866 in a report titled, “Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation. This anomaly accounts for 0.5%–0.7% of cases of congenital heart disease. The average life duration of patients with Ebstein's anomaly is 25–30 years. The malformation consists of apical displacement of the tricuspid valve with resultant regurgitation and enlargement of the right heart chambers, resulting in arrhythmias and heart failure. The common cardiac anomalies associated with the condition are atrial septal defect (ASD in 90% of patients, anatomic or functional tricuspid atresia in 30%, Wolff-Parkinson-White (WPW syndrome in 15% and less commonly ventricular septal defect (VSD, pulmonic stenosis or atresia and mitral valve prolapse. Survival into adulthood is common and patients present with cyanosis, dyspnea and palpitations.

  3. Jejunoileal atresia and cystic fibrosis: don’t miss it

    OpenAIRE

    Siersma Carolien L; Rottier Bart L; Hulscher Jan BF; Bouman Katelijne; van Stuijvenberg Margriet

    2012-01-01

    Abstract Background While an increased prevalence of cystic fibrosis (CF) in patients with jejunal atresia and ileal atresia (JIA) has been described previously, it still may not be a practice routine to indicate a sweat test or DNA test for CFTR mutations in newborns presenting with JIA. Leading textbooks do not mention JIA as a possible presenting clinical feature of CF. We describe two cases of JIA with a delayed diagnosis of CF (4 months [post mortem] and 19 months). This led to a retrosp...

  4. [Surgery of anal fistulas].

    Science.gov (United States)

    Ricchi, E; Carriero, A; Spallanzani, A; Fundarò, S; Heydari, A; Piccoli, M; Gelmini, R

    1997-06-01

    The authors report a study on 120 patients with anal fistula (111 males and 9 females). The average age was 44.3 years (median 44, SD +/- 14.807). 64.1% of patients had an intersphincteric fistula, 23.3% hanal transphincteric fistula, 1.6% a suprasphincteric fistula, 7.5% a horseshoe fistula and the 3.3% an extrasphincteric fistula. We treated 14 patients (11.66%) with direct surgical treatment. The other 106 had various types of treatment depending on the localisation and the involvement of the anorectal sphincter. We had 11 cases (9.1%) of complications, such as recurrence in 5 patients (4.1%) transitory incontinence in 2 cases (1.6%) and finally postoperative bleeding in 3 patients (2.5%). PMID:9324655

  5. Radiological evaluation congenital gastrointestinal tract anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Young Hee; Kim, Ock [Hanil Hospital, Seoul (Korea, Republic of); Jang, Jung Wha [Seoul Nationl Hospital, Seoul (Korea, Republic of)

    1983-06-15

    With the improvements, during recent years, in the control of the infections and nutritional diseases the subject of congenital malformation becomes of increasing importance. The radiologic signs are crucial for prompt diagnosis of anomalies of alimentary tract and with early identification of resulting complication, surgical therapy is usually life-saving. 30 cases of congenital anomalies of alimentary tract in infants were reviewed in respect of age, sex, incidence and radiological findings. The results are summarized as follows; 1, The most common lesion was hypertrophic pyloric stenosis, followed by congenital megacolon and anorectal anomaly, tracheoesophageal fistula, intestinal atresia. 2. Male outnumbered female in most congenital anomalies of alimentary tract. 25 cases were under the age of 1 month. 3. Common symptoms of upper gastrointestinal tract obstruction are vomiting and abdominal distension. In the obstruction of lower gastrointestinal tract, abdominal distension and failure of meconium passage were noted. 4. Roentgenologic findings were as follows, a. Chest A-P and lateral view: In tracheoesophageal fistula, saccular dilatation of upper esophagus and displacement of trachea anterolaterally were the most common finding. b. Simple abdomen: Obstructive pattern of proximal portion of duodenum shows in 11 cases, of distal bowel shows in 16 cases. Duodenal atresia showed 'double bubble' sign, hypertrophic pyloric stenosis showed marked gastric distension, paucity of air in small bowel and increases gastric peristalsis were the most common findings. Hirschsprung's disease showed absenced of rectal gas almostly. The variable length between blind hindgut to anus was seen in anorectal anomalies. c. Esophagogram: Blind sac of upper esophagus was seen at the 4th thoracic spinal level and displacement of trachea anterolaterally. 1 case of tracheoesophageal fistula had an intact esophageal lumen. d. Upper G-I series: In hypertrophic pyloric

  6. ESOPHAGEAL ATRESIA WITH DISTAL TRACHEOESOPHAGEAL FISTULA: SURGERY TREATMENT AND A LONG TERM FOLLOW UP

    Directory of Open Access Journals (Sweden)

    E. Cerchia

    2012-12-01

    Full Text Available A study carried out at the Paediatric Surgery Units in Siena and Toulouse evaluated the long term follow-up of patients treated for esophageal atresia (EA, between 1988 and 2007. We analyzed the long term follow-up of 57 patients with III type EA. We evaluated the residual symptoms in three time intervals: in 1st years, between 2nd to 5th year and over 5th years from surgery. The considered parameters were: feeding difficulties, respiratory problems, gastro-esophageal reflux, growth impairment, chest anomalies. Feeding difficulty appeared in 26 patients in the 1st year of life, in 28 between the 2nd and 5th year and in 6 after the 5th year. Respiratory problems occurred in 35 children in 1st year, in 30 between the 2nd and 5th year and in 17 after the 5th year. Gastro-esophageal reflux occurred in 30 patients in 1st year, in 22 between the 2nd and 5th year and in 8 over the 5th year. Defects of growth were detected in 17 patients in 1st year of life, in 16 between the 2nd and 5th year and in 5 after the 5th year. Chest anomalies were diagnosed in 7 children. Our analysis established a time limit (5th year postoperatively over which an undeniable improvement of life quality can be expected.

  7. Magnetic compression anastomosis as a nonsurgical treatment for esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Zaritzky, Mario [Hospital de Ninos de La Plata, Department of Radiology, Buenos Aires (Argentina); University of Chicago Medical Center, Department of Radiology, Chicago, IL (United States); Ben, Ricardo [Hospital de Ninos de La Plata, Department of Gastroenterology, Buenos Aires (Argentina); Zylberg, Gaston I.; Yampolsky, Brian [Hospital de Ninos de La Plata, Department of Radiology, Buenos Aires (Argentina)

    2009-09-15

    We describe a unique technique to promote a nonsurgical esophageal anastomosis with magnets in children with esophageal atresia. To evaluate the efficacy of magnetic lengthening of atretic esophageal ends to produce an anastomosis and to communicate our results after more than 2 years of follow-up. Between September 2001 and March 2004, five children were selected for treatment. Two of the children had esophageal atresia without fistula (type A) and three had atresia with fistula converted to type A surgically; however, surgeons failed to achieve an anastomosis because of the width of the gap. Neodymium-iron-boron magnets were used. Daily chest radiographs were taken until union of the magnets was observed. They were then replaced with an orogastric tube. Anastomosis was achieved in all patients in an average of 4.8 days. One patient, with signs of early sepsis, was successfully treated with antibiotics. In four of the five patients, esophageal stenosis developed. At the time of this report, two patients were free of treatment and on an oral diet (after 26 months), two patients required periodic balloon dilatation, and one patient had recently undergone surgery due to recurrent esophageal stenosis not amenable to balloon dilatation. Magnetic esophageal anastomosis is a feasible method in selected patients with esophageal atresia. Esophageal anastomosis was achieved in all patients. The only observed complication of significance was esophageal stenosis. One patient needed surgery because of stenosis. (orig.)

  8. Esophageal Atresia and Tracheoesophageal Fistula with Unilateral Pulmonary Agenesis - Hypoplasia

    Directory of Open Access Journals (Sweden)

    Katragadda Laxmi Narsimha Rao

    2013-04-01

    Full Text Available Association of unilateral severe pulmonary hypoplasia or agenesis and esophageal atresia (EA with or without tracheoesophageal fistula (TEF is an exceedingly rare and highly lethal combination. We report a case of full term male baby who had EA with TEF and right lung hypoplasia, managed at our centre. He is alive and doing well at 10 years of age.

  9. Clinical and Molecular-Genetic Studies in Esophageal Atresia

    NARCIS (Netherlands)

    E.M. de Jong (Elisabeth)

    2010-01-01

    markdownabstract__Abstract__ Esophageal atresia (EA) and trachea-esophageal fistula (TEF) [MIM 189960] are severe developmental defects that aff ect one in 3,500 newborns. Slightly more boys are aff ected than girls. In The Netherlands, approximately 70 children are born annually with this congenit

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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 basal atretic follicles in comparison with other follicles. Cell death was greater in both endothelial cells (P 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

  12. Comparative study of anal acoustic reflectometry and anal manometry in the assessment of faecal incontinence

    DEFF Research Database (Denmark)

    Hornung, B R; Mitchell, P J; Carlson, G L;

    2012-01-01

    Anal acoustic reflectometry (AAR) is a reproducible technique providing a novel physiological assessment of anal sphincter function. It may have advantages over conventional anal manometry. The aims of this study were to determine the ability of AAR and anal manometry to identify changes in anal...

  13. Unilateral pulmonary veins atresia: evaluation by computed tomography; Atresia unilateral das veias pulmonares: avaliacao por tomografia computadorizada

    Energy Technology Data Exchange (ETDEWEB)

    Eifer, Diego Andre; Arsego, Felipe Veras, E-mail: felipesoarestorres@gmail.com [Hospital de Clinicas de Porto Alegre (HCPA), RS (Brazil). Serv. de Radiologia; Torres, Felipe Soares [Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS (Brazil). Escola de Medicina

    2013-11-15

    Unilateral pulmonary vein atresia is a rare congenital condition. In addition to cardiac malformations or pulmonary hypertension, patients may present with recurrent pulmonary infections or hemoptysis in childhood or adolescence. The authors report a case where the typical findings of such condition were observed at computed tomography in an adult patient. (author)

  14. Electrocautery for Precancerous Anal Lesions

    Science.gov (United States)

    Results from a randomized clinical trial conducted in Amsterdam suggest that electrocautery is better than topical imiquimod or fluorouracil at treating potentially precancerous anal lesions in HIV-positive men who have sex with men.

  15. Modern management of anal fistula.

    Science.gov (United States)

    Limura, Elsa; Giordano, Pasquale

    2015-01-01

    Ideal surgical treatment for anal fistula should aim to eradicate sepsis and promote healing of the tract, whilst preserving the sphincters and the mechanism of continence. For the simple and most distal fistulae, conventional surgical options such as laying open of the fistula tract seem to be relatively safe and therefore, well accepted in clinical practise. However, for the more complex fistulae where a significant proportion of the anal sphincter is involved, great concern remains about damaging the sphincter and subsequent poor functional outcome, which is quite inevitable following conventional surgical treatment. For this reason, over the last two decades, many sphincter-preserving procedures for the treatment of anal fistula have been introduced with the common goal of minimising the injury to the anal sphincters and preserving optimal function. Among them, the ligation of intersphincteric fistula tract procedure appears to be safe and effective and may be routinely considered for complex anal fistula. Another technique, the anal fistula plug, derived from porcine small intestinal submucosa, is safe but modestly effective in long-term follow-up, with success rates varying from 24%-88%. The failure rate may be due to its extrusion from the fistula tract. To obviate that, a new designed plug (GORE BioA®) was introduced, but long term data regarding its efficacy are scant. Fibrin glue showed poor and variable healing rate (14%-74%). FiLaC and video-assisted anal fistula treatment procedures, respectively using laser and electrode energy, are expensive and yet to be thoroughly assessed in clinical practise. Recently, a therapy using autologous adipose-derived stem cells has been described. Their properties of regenerating tissues and suppressing inflammatory response must be better investigated on anal fistulae, and studies remain in progress. The aim of this present article is to review the pertinent literature, describing the advantages and limitations of

  16. Inter-Rater Agreement of Anal Cytology

    OpenAIRE

    Darragh, Teresa M.; Tokugawa, Diane; Castle, Philip E.; Follansbee, Stephen; Borgonovo, Sylvia; LaMere, Brandon J.; Schwartz, Lauren; Gage, Julia C.; Fetterman, Barbara; Lorey, Thomas; Wentzensen, Nicolas

    2012-01-01

    Most anal cancers are caused by persistent infections with carcinogenic human papillomaviruses (HPV). Similar to cervical carcinogenesis, the progression from HPV infection to anal cancer goes through precancerous lesions that can be treated to prevent invasion. In analogy to cervical cytology, anal cytology has been proposed as a screening tool for anal cancer precursors in high-risk populations. We analyzed the inter-observer reproducibility of anal cytology in a population of 363 HIV-infec...

  17. Identifying, characterizing, and classifying congenital anomalies of the coronary arteries.

    Science.gov (United States)

    Shriki, Jabi E; Shinbane, Jerold S; Rashid, Mollie A; Hindoyan, Antereas; Withey, James G; DeFrance, Anthony; Cunningham, Mark; Oliveira, George R; Warren, Bill H; Wilcox, Alison

    2012-01-01

    The clinical manifestations of coronary artery anomalies vary in severity, with some anomalies causing severe symptoms and cardiovascular sequelae and others being benign. Cardiovascular computed tomography (CT) has emerged as the standard of reference for identification and characterization of coronary artery anomalies. Therefore, it is important for the reader of cardiovascular CT images to be thoroughly familiar with the spectrum of coronary artery anomalies. Hemodynamically significant anomalies include atresia, origin from the pulmonary artery, interarterial course, and congenital fistula. Non-hemodynamically significant anomalies include duplication; high origin; a prepulmonic, transseptal, or retroaortic course; shepherd's crook right coronary artery; and systemic termination. In general, coronary arteries with an interarterial course are associated with an increased risk of sudden cardiac death. Coronary artery anomalies that result in shunting, including congenital fistula and origin from the pulmonary artery, are also commonly symptomatic and may cause steal of blood from the myocardium. Radiologists should be familiar with each specific variant and its specific constellation of potential implications. PMID:22411942

  18. Potential Pitfalls on the 99mTc-Mebrofenin Hepatobiliary Scintigraphy in a Patient with Biliary Atresia Splenic Malformation Syndrome

    Directory of Open Access Journals (Sweden)

    Jane Maestri Brittain

    2016-01-01

    Full Text Available 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 99mTc-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 99mTc-Mebrofenin hepatobiliary scintigraphy in an infant with BASM and stress the importance that the 99mTc-Mebrofenin hepatobiliary scintigraphy is part of a spectrum of imaging modalities in diagnosing BA.

  19. Potential Pitfalls on the (99m)Tc-Mebrofenin Hepatobiliary Scintigraphy in a Patient with Biliary Atresia Splenic Malformation Syndrome.

    Science.gov (United States)

    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

  20. Coronary to pulmonary fistula as the primary source of pulmonary blood supply in pulmonary atresia with ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Isman Firdaus

    2004-12-01

    Full Text Available A communication between the coronary and pulmonary arteries, so called coronary to pulmonary fistula, is a rare source of pulmonary supply in pulmonary atresia (PA with ventricular septal defect (VSD. A 4 year old girl referred to National Cardiovascular Center Harapan Kita, Jakarta with symptoms and signs of increased pulmonary blood flow since infancy and was confirmed by the chest x-rays. Heart examination revealed normal first heart sound with single loud second heart sound and an ejection systolic murmur at the pulmonary area. ECG demonstrated sinus rhythm with normal axis and biventricular hypertrophy. Echocardiography was performed and truncus arteriosus (TA type I was suspected with perimembranus VSD, overriding of the aorta, and dilated main pulmonary artery. But on cardiac catheterization studies, a non obstructive fistula was found between the left coronary and main pulmonary artery coexisted with PA and VSD. A successful surgery was performed subsequently and confirmed the above diagnosis. Although there were episodes of pulmonary hypertension crisis during early post operative course, she was then discharge from the hospital in a good condition. Since irreversible pulmonary vascular disease may develop in a non restrictive coronary to pulmonary fistula, early recognition of this anomaly is very important for better surgical result. (Med J Indones 2004; 13: 237-40Keywords: coronary to pulmonary fistula, pulmonary atresia, ventricular septal defect

  1. A rare variant case of pure esophageal atresia with an atretic segment

    Directory of Open Access Journals (Sweden)

    Kazunori Masahata

    2015-09-01

    Full Text Available Pure esophageal atresia is typically characterized by a long gap between the upper and lower pouches, with a gasless abdomen and no fistula. The association of pure esophageal atresia with an atretic segment is extremely rare. We report a rare variant case of pure esophageal atresia in which the two blind esophageal pouches were joined by an atretic segment. Excision of the atretic segment and primary anastomosis were performed successfully.

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

    Science.gov (United States)

    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.

  3. BIRTH INJURY RELATED UNILATERAL ANTERIOR NARES PARTIAL FIBROUS ATRESIA: A RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Jayagar

    2016-02-01

    Full Text Available Congenital atresia of anterior nares has been rarely reported and it may co-exist along with posterior choanal atresia. (1,2 In our case, birth injury caused by forceps delivery has caused unilateral anterior nares partial atresia. Twenty eight years old male patient came with the complaints of left nostril blocked sensation since birth, aggravated with attacks of upper respiratory tract infections. On examination he had left anterior nares partial atresia caused by fibrous bands as a result of birth injury due to instrumental delivery. Rhinoplasty performed to open-up left nostril and patient relieved of his symptoms and also on cosmetic appearance

  4. Avaliação da atresia maxilar associada ao tipo facial Evaluation of maxillary atresia associated with facial type

    Directory of Open Access Journals (Sweden)

    Marina Gomes Pedreira

    2010-06-01

    Full Text Available OBJETIVOS: associar a atresia maxilar aos tipos faciais, verificar o dimorfismo entre os gêneros masculino e feminino, bem como a correlação entre os gêneros e os tipos faciais. MÉTODOS: inicialmente, a amostra constou de 258 telerradiografias da cabeça, em norma lateral. Após aplicada a análise do Vert de Ricketts, foram excluídas 108 telerradiografias, por não satisfazerem o critério de seleção. Portanto, a amostra final foi de 150 telerradiografias e 150 modelos pertencentes a 150 indivíduos brancos na faixa etária de 14 anos a 18 anos e 11 meses, independentemente do tipo de má oclusão. A mesma foi dividida em: 50 mesofaciais; 50 braquifaciais e 50 dolicofaciais. Para os 150 modelos, aplicou-se a análise de Schwarz. RESULTADOS: a presença da atresia maxilar na amostra selecionada correspondeu a 64% nos dolicofaciais, 58% nos braquifaciais e 52% nos mesofaciais. CONCLUSÕES: não houve evidência de associação da atresia com o tipo facial. Quanto ao dimorfismo de gênero, foi proporcionalmente maior para o dolicofacial masculino enquanto o feminino não apresentou proporções diferentes.OBJECTIVES: To associate maxillary atresia with facial types, investigating whether dimorphism occurs between males and females and evaluating the percentage of such dimorphism according to gender and facial type. METHODS: Initially, the sample consisted of 258 lateral cephalometric radiographs. After analyzing Ricketts' VERT index, 108 radiographs were excluded for not meeting the selection criteria. Therefore, the sample consisted of 150 lateral cephalometric radiographs and 150 models of 150 Caucasian individuals aged 14 years to 18 years and 11 months, regardless of malocclusion type. The sample was divided into 50 mesofacials, 50 brachyfacials and 50 dolichofacials. The Schwarz's analysis was applied to all 150 models. RESULTS: The presence of maxillary atresia in the sample consisted of 64% in dolichofacials, 58% in brachyfacials

  5. Anaesthesia for biliary atresia and hepatectomy in paediatrics

    Directory of Open Access Journals (Sweden)

    Rebecca Jacob

    2012-01-01

    Full Text Available 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.

  6. Pregnancy Complicated by Portal Hypertension Secondary to Biliary Atresia

    Directory of Open Access Journals (Sweden)

    O. E. O'Sullivan

    2013-01-01

    Full Text Available Biliary atresia is a rare idiopathic neonatal cholestatic disease characterized by the destruction of both the intra- and extrahepatic biliary ducts. As the disease is progressive all cases will develop portal fibrosis, cirrhosis, and portal hypertension with the sequelae of varices, jaundice, and eventually liver failure requiring a transplant. Survival rates have improved considerably with many females living well in to be childbearing age. Due to the complexity of the disease these pregnancies are considered, high risk. We report the antenatal, intrapartum, and postpartum managements of a pregnancy complicated by biliary atresia. Furthermore, we highlight the importance of a multidisciplinary team approach in optimizing obstetric care for this high risk group.

  7. Surgery for Crohn's anal fistulas.

    Science.gov (United States)

    Sugita, A; Koganei, K; Harada, H; Yamazaki, Y; Fukushima, T; Shimada, H

    1995-11-01

    The aim of this study was to analyze the features of Crohn's anal fistulas and to evaluate the efficacy of seton treatment. In 119 patients with Crohn's disease, the incidence of anal fistula was 56% (67/119), with no significant difference in the incidence among patients with ileitis, colitis, and ileocolitis. "Intractable" anal fistulas were found in 17% of patients with ileitis, compared to 64% of those with colitis (P = 0.051) and 68% of those with ileocolitis (P = 0.014). Seton treatment, i.e., non-cutting, long-term seton drainage, was performed for 21 patients (5 with intersphincteric, and 16 with transsphincteric fistulas). In the 16-month follow up, 9 patients required redrainage for recurrent fistulous abscess, mainly because of progressive colorectal disease. Finally, a good result was obtained in 17 of the 21 patients (81%) and no recurrent fistulous abscess developed in the 8 patients in whom all setons were removed. Anal continence was preserved in all the patients. These results indicate that anal fistulas with Crohn's ileitis were cured more easily than those with colitis or ileocolitis, and that seton treatment was effective for intersphincteric fistula with multiple fistula openings and for transphincteric fistulas in patients exhibiting remission of intestinal Crohn's disease. PMID:8563879

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

    Science.gov (United States)

    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

  9. Neonatal Bartter syndrome associated with ileal atresia and cystic fibrosis

    OpenAIRE

    Akuma, A. O.; Mittal, S K; Sambo, A. A.

    2013-01-01

    A rare case of neonatal Bartter syndrome presenting with severe hyperkalemia is reported in a preterm child born to consanguineous parents. This child also had ileal atresia, and meconium plugs were found at laparotomy. The diagnosis of cystic fibrosis was subsequently made on genetic testing. Despite full intensive care management and surgical interventions, he died of respiratory failure after 70 days. This is the first reported case of such conglomeration of pathologies in a newborn child....

  10. Pregnancy Complicated by Portal Hypertension Secondary to Biliary Atresia

    OpenAIRE

    O. E. O'Sullivan; Crosby, D.; B. Byrne; Regan, C.

    2013-01-01

    Biliary atresia is a rare idiopathic neonatal cholestatic disease characterized by the destruction of both the intra- and extrahepatic biliary ducts. As the disease is progressive all cases will develop portal fibrosis, cirrhosis, and portal hypertension with the sequelae of varices, jaundice, and eventually liver failure requiring a transplant. Survival rates have improved considerably with many females living well in to be childbearing age. Due to the complexity of the disease these pregnan...

  11. Prenatal diagnosis of small bowel atresia: A case report

    OpenAIRE

    Jamal A; Mesdaghi Nia S

    2000-01-01

    In this paper we report a case of jejunal atresia that was detected pre-nataly. Polyhydramnios and multiple dilated bowel loops in the lower part of fetal abdomen in ulterasonography helped us to diagnose this abnormality. Post-natal barium enema showed it and also it was confirmed by laparotomy confirmed it. Pre-natal diagnosis is important in these cases to facilitate delivery in centers where expert team of specialists are available to allow optimal neonatal care.

  12. Prenatal diagnosis of small bowel atresia: A case report

    Directory of Open Access Journals (Sweden)

    Jamal A

    2000-09-01

    Full Text Available In this paper we report a case of jejunal atresia that was detected pre-nataly. Polyhydramnios and multiple dilated bowel loops in the lower part of fetal abdomen in ulterasonography helped us to diagnose this abnormality. Post-natal barium enema showed it and also it was confirmed by laparotomy confirmed it. Pre-natal diagnosis is important in these cases to facilitate delivery in centers where expert team of specialists are available to allow optimal neonatal care.

  13. Treatment strategies in the management of jejunoileal and colonic atresia

    Directory of Open Access Journals (Sweden)

    Chadha Rajiv

    2006-01-01

    Full Text Available BACKGROUND/PURPOSE: The purpose of this prospective study was to review the operative findings, treatment strategies, as well as the results of management of 46 consecutive cases of jejunoileal and colonic atresia, managed over a 2-year period. MATERIALS AND METHODS: There were 42 patients with jejunoileal atresia (JIA and 4 with colonic atresia (CA. The 4 group types were: type I-membranous (n=20, type II- blind ends separated by a fibrous cord (n=6, type IIIa- blind ends with a V-shaped mesenteric defect (n=10, type IIIb- apple-peel atresia (n=4 and type IV- multiple atresias (n=6. Primary surgery for JIA consisted of resection with a single anastomosis (n=37, anastomosis after tapering jejunoplasty (n=3, multiple anastomosis (n=1 and a Bishop-Koop ileostomy (n=1. For CA, resection with primary anastomosis was performed. A single end-to-oblique anastomosis after adequate resection of dilated proximal bowel, was the preferred surgical procedure. In the absence of facilities for administering TPN, early oral/nasogastric (NG tube feeding was encouraged. In patients with anastomotic dysfunction, conservative treatment of the obstruction followed after its resolution by gradually increased NG feeds, was the preferred treatment protocol. RESULTS: Late presentation or diagnosis with hypovolemia, electrolyte imbalance, unconjugated hyperbilirubinemia (n=25 and sepsis (n=6, were significant preoperative findings. After resection and anastomosis, significant shortening of bowel length was seen in 16 patients (34.7%. Postoperative complications included an anastomotic leak (n=3, a perforation proximal to the anastomosis in 1 and anastomotic dysfunction in 5 patients. Full oral or NG tube feeding was possible only by the 13th to 31st postoperative day (POD, after the primary surgery in patients with anastomotic dysfunction and those undergoing reoperation. Overall, 38 patients survived (82.6%. Mortality was highest in patients with type IIIb or type IV

  14. Role of preoperative tracheobronchoscopy in newborns with esophageal atresia:A review

    Institute of Scientific and Technical Information of China (English)

    Filippo; Parolini; Giovanni; Boroni; Stefania; Stefini; Cristina; Agapiti; Tullia; Bazzana; Daniele; Alberti

    2014-01-01

    Preoperative tracheobronchoscopy(TBS) in the diag-nostic assessment of newborns affected by esophageal atresia(EA) was described in 1981. Nevertheless, the value of the procedure is actually much debated; only a few studies have clearly explored the advantages of TBS and this procedure is not yet routinely included inthe diagnostic and therapeutic assessment in many in-ternational pediatric surgery settings. Routine preoper-ative TBS is a safe procedure that enables the accurate examination of the tracheobronchial tree, the visualiza-tion of tracheoesophageal fistula and the diagnosis of tracheomalacia or associated respiratory anomalies. When a distal fistula is found, its occlusion with a Fog-arty balloon catheter improves mechanical ventilation and facilitates surgical repair. This review provides a detailed overview on the use of TBS in newborns with EA, focusing on technical aspects, anesthesiological management, indications and limits. The benefits and risks of the procedure are also compared with alterna-tive diagnostic tools, such as an esophageal contrast study, computed tomography scan and ultrasound.

  15. Anomaly Structure of Supergravity and Anomaly Cancellation

    CERN Document Server

    Butter, Daniel

    2009-01-01

    We display the full anomaly structure of supergravity, including new D-term contributions to the conformal anomaly. This expression has the super-Weyl and chiral U(1)_K transformation properties that are required for implementation of the Green-Schwarz mechanism for anomaly cancellation. We outline the procedure for full anomaly cancellation. Our results have implications for effective supergravity theories from the weakly coupled heterotic string theory.

  16. Unusual Cause of Esophageal Obstruction in a Neonate Presenting as Esophageal Atresia

    OpenAIRE

    Vijay C Pujar; Joshi, Shirin S; Dhaded, Sangappa M

    2013-01-01

    Esophageal atresia is the commonest cause of obstruction to esophageal lumen in neonates. Foreign bodies in newborns are extremely rare. We report a rare case of esophageal obstruction closely mimicking atresia due to foreign bodies inserted in a female neonate with homicidal intension.

  17. Development of an adenocarcinoma of the esophagus 22 years after primary repair of a congenital atresia

    NARCIS (Netherlands)

    Pultrum, BB; Bijleveld, CM; de Langen, ZJ; Plukker, TTM

    2005-01-01

    Esophageal cancer development after previous atresia repair is extremely rare in young patients. We present the clinical course of a patient who developed an adenocarcinoma of the esophagus at the age of 22 years, after repair of a tracheoesophageal fistula with esophageal atresia in the neonatal pe

  18. Development of infantile hypertrophic pyloric stenosis in patients treated for oesophageal atresia. A case report

    DEFF Research Database (Denmark)

    Qvist, N; Rasmussen, L; Hansen, L P;

    1986-01-01

    Two cases of infantile hypertrophic pyloric stenosis (IHPS) developed in 74 patients treated for oesophageal atresia. Treatment of oesophageal atresia is frequently followed by vomiting and failure to thrive due to gastrooesophageal reflux or anastomotic stricture. The diagnose of IHPS must...

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

    Science.gov (United States)

    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

  20. Thoracoscopic traction technique in long gap esophageal atresia : entering a new era

    NARCIS (Netherlands)

    van der Zee, David C.; Gallo, Gabriele; Tytgat, Stefaan H. A.

    2015-01-01

    To describe the evolution from delayed management of long gap esophageal atresia to thoracoscopic treatment directly after birth without the placement of a gastrostomy. Long gap esophageal atresia remains a challenge for pediatric surgeons. Over the years, several techniques have been described to d

  1. Seasonal prevalence and intensity of follicular atresia in Baltic cod Gadus morhua callarias L

    DEFF Research Database (Denmark)

    Kraus, Gerd; Tomkiewicz, Jonna; Diekmann, R.;

    2008-01-01

    of atresia, i.e. number of atretic oocytes in relation to normally developed vitellogenic oocytes, was low amounting to 1.4% on average. Similar to prevalence, relative intensity of atresia differed significantly between maturity stages and increased with decreasing female condition. The population egg loss...

  2. Choanal atresia as a feature of ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome.

    OpenAIRE

    Christodoulou, J; McDougall, P N; Sheffield, L J

    1989-01-01

    We report here a father and daughter with digital abnormalities, nasolacrimal duct obstruction, and variable alopecia. The father had a cleft lip and palate and the daughter had choanal atresia. We propose they both have the EEC syndrome and show the variable expressivity of this disorder. Choanal atresia has not been previously reported in this condition.

  3. Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review.

    NARCIS (Netherlands)

    Kumagi, T.; Drenth, J.P.H.; Guttman, O.; Ng, V.; Lilly, L.; Therapondos, G.; Hiasa, Y.; Michitaka, K.; Onji, M.; Watanabe, Y.; Sen, S.; Griffiths, W.; Roberts, E.; Heathcote, J.; Hirschfield, G.M.

    2012-01-01

    BACKGROUND: Biliary atresia is a progressive biliary injury which occurs only in infants. AIMS: To review the experience of patients surviving into adulthood without the need for liver transplantation in childhood. METHODS: A multicentre review of patients with biliary atresia treated surgically who

  4. Preferential Associated Anomalies in 818 Cases of Microtia in South America

    Science.gov (United States)

    Luquetti, Daniela V; Cox, Timothy C; Lopez-Camelo, Jorge; Dutra, Maria da Graça; Cunningham, Michael L; Castilla, Eduardo E

    2013-01-01

    The etiology of microtia remains unknown in most cases. The identification of patterns of associated anomalies (i.e., other anomalies that occur with a given congenital anomaly in a higher than expected frequency), is a methodology that has been used for research into the etiology of birth defects. We conducted a study based on cases of microtia that were diagnosed from more than 5 million live (LB)- and stillbirths (SB) examined in hospitals participating in ECLAMC (Latin American Collaborative Study of Congenital Malformations) between 1967 and 2009. We identified 818 LB and SB with microtia and at least one additional non-related major congenital anomaly (cases) and 15,969 LB and SB with two or more unrelated major congenital anomalies except microtia (controls). A logistic regression analysis was performed to identify the congenital anomalies preferentially associated with microtia. Preferential associations were observed for 10 congenital anomalies, most of them in the craniofacial region, including facial asymmetry, choanal atresia, and eyelid colobomata. The analysis by type of microtia showed that for anomalies such as cleft lip and palate, macrostomia, and limb reduction defects, the frequency increased with the severity of the microtia. In contrast, for other anomalies the frequency tended to be the same across all types of microtia. Based on these results we will integrate data on the developmental pathways related to preferentially associated congenital anomalies for future studies investigating the etiology of microtia. PMID:23554119

  5. Definitive management of isolated esophageal atresia: Experience at NICH Karachi

    Directory of Open Access Journals (Sweden)

    Jan Iftikhar

    2006-01-01

    Full Text Available Background: Definitive treatment of isolated esophageal atresia (IEA is still controversial. A study was conducted to review cases of IEA in our department with a view to evaluate the long term results of definitive surgery in these patients. Materials and Methods: Nine consecutive patients with IEA were included in the study. All babies initially underwent cervical esophagostomy and feeding gastrostomy. One baby also had anorectal atresia and needed a colostomy. In 6 babies, end esophagostomy was performed while in 3 babies, lateral esophagostomy was performed. Of the 6 babies with end esophagostomy, two underwent jejunal Interpositioning, two had serial extra-thoracic lengthening and two reverse gastric tube interpositiong. Three babies with lateral esophagostomy were planned for delayed primary repair. Results: Both babies with jejunal interpositiong initially did well; serious gastro-esophageal reflux occurred in one baby who needed antireflux surgery. At 6 years follow-up both children had redundant Jejunum. Recurrent Respiratory tract infection remained a problem in one child while other is doing well. Of the two babies with extrathoracic lengthening it was possible to perform end to end anastomosis in one baby but in the other extrathoracic lengthening did not work and gastric transpositioning was performed. Of the two babies with reverse gastric tube interpositioning one developed esophageal stenosis at the site of anastomosis and is on serial dilatations. Other died due to anastomotic leak and mediastinitis. In all the three babies with lateral esophagostomy spontaneous lengthening of esophagus was noted. End to end anastomosis was possible in two of these babies who are doing well. One is waiting definitive surgery. Number of complications, hospital stay and cost of surgery was least in patients with lateral esophagostomy. Conclusion: Various methods of definitive treatment have been proposed for isolated esophageal atresia but each

  6. Bilateral congenital lacrimal fistulas in an adult as part of ectrodactyly-ectodermal dysplasia-clefting syndrome: A rare anomaly

    OpenAIRE

    Debangshu Ghosh; Somnath Saha; Sumit Kumar Basu

    2015-01-01

    Ectrodactyly-ectodermal dysplasia and clefting syndrome or "Lobster claw" deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD) obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mil...

  7. Bronchial atresia in a neonate with congenital cytomegalovirus infection

    Directory of Open Access Journals (Sweden)

    Abdullah A Yousef

    2013-01-01

    Full Text Available Bronchial atresia (BA is characterized by a mucus-filled bronchocele in a blind-ending segmental or lobar bronchus with hyperinflation of the obstructed segment of the lung. We describe a neonate who presented on his 9 th day of life with respiratory distress. Chest computed tomography showed a soft tissue density involving the right middle lobe (RML. RML lobectomy confirmed the diagnosis of BA. Cytomegalovirus was detected by polymerase chain reaction in blood, urine, and tracheal aspirates which may provide further insight into the pathogenesis of BA.

  8. Study on congenital microtia and atresia after external canal plasty

    International Nuclear Information System (INIS)

    In treating congenital microtia and atresia, we conduct simultaneous external canal plasty, tympanoplasty, and auricle elevation with plastic surgeons about 6 months after auricleplasty by only plastic surgeons. The results are good both cosmetically and functionally. We report 13 cases in which hearing did not improve satisfactorily after surgery using postoperative high-resolution computed tomography (HRCT) of the temporal bone. Lateral healing was seen in 9 (69%), new bone proliferation in 3 (23%), malpositioning of a cartilage block in 2 (15%), and both lateral healing and malpositioning of a cartilage block in 1 (7.6%). (author)

  9. Diagnosis and evaluation of esophageal atresia by direct sagittal CT

    Energy Technology Data Exchange (ETDEWEB)

    Tam, P.K.H.; Saing, H.; Chan, F.L.

    1987-01-01

    Direct sagittal CT is possible in newborns because of their small body-size. With this noninvasive investigation, we were able to establish a correct diagnosis in two neonates with esophageal atresia. Moreover, the demonstration of the air-filled proximal pouch and distal tracheoesophageal fistula along their whole lengths allowed exclusion of the possibility of a proximal pouch fistula and gave knowledge of the exact distance of the two segments of the esophagus needed to be bridged to allow anastomosis, thus providing additional valuable information for the surgeon preoperatively..

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

    Science.gov (United States)

    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

  11. Chiral anomalies and differential geometry

    Energy Technology Data Exchange (ETDEWEB)

    Zumino, B.

    1983-10-01

    Some properties of chiral anomalies are described from a geometric point of view. Topics include chiral anomalies and differential forms, transformation properties of the anomalies, identification and use of the anomalies, and normalization of the anomalies. 22 references. (WHK)

  12. Internal anal sphincter: an anatomic study.

    Science.gov (United States)

    Uz, A; Elhan, A; Ersoy, M; Tekdemir, I

    2004-01-01

    The anatomy of the internal anal sphincter and surrounding structures was investigated in 24 cadavers using a surgical microscope (6-25 x magnification). An understanding of the anatomy of the internal anal sphincter is helpful in avoiding complications during surgical procedures in the anorectal region. The external anal sphincter was composed of three ellipsoid rings of skeletal muscle (subcutaneous, superficial, and deep) that encircle the anal canal; in contrast, we found that the internal anal sphincter was composed of flat rings of smooth muscle bundles stacked one on top of the other, like the slats of a Venetian blind. In each anal canal, the average number of ring-like slats observed was 26.33 +/- 2.93 (range = 20-30) and each was covered by its own fascia. The smooth muscle fibers and fascia coalesced at three equidistant points around the anal canal to form three columns that extended distally into the lumen and differed in form from the other anal columns. When viewed from an anterior position, the columns were located anteriorly at the observer's right (5 o'clock position), posteriorly at the right (1 o'clock position), and laterally at the left (9 o'clock position). This heretofore unreported anatomy of the internal anal sphincter may play an important role in closing off the lumen of the anal canal and maintaining bowel continence. PMID:14695582

  13. Oesophageal atresia: Diagnosis and prognosis in Dakar, Senegal

    Directory of Open Access Journals (Sweden)

    Mbaye Fall

    2015-01-01

    Full Text Available Background: Oesophageal atresia is a neonatal emergency surgery whose prognosis has improved significantly in industrialised countries in recent decades. In sub-Saharan Africa, this malformation is still responsible for a high morbidity and mortality. The objective of this study was to analyse the diagnostic difficulties and its impact on the prognosis of this malformation in our work environment. Patients and Methods: We conducted a retrospective study over 4 years on 49 patients diagnosed with esophageal atresia in the 2 Paediatric Surgery Departments in Dakar. Results: The average age was 4 days (0-10 days, 50% of them had a severe pneumonopathy. The average time of surgical management was 27 h (6-96 h. In the series, we noted 10 preoperative deaths. The average age at surgery was 5.7 days with a range of 1-18 days. The surgery mortality rate is 28 patients (72% including 4 late deaths. Conclusion: The causes of death were mainly sepsis, cardiac decompensation and anastomotic leaks.

  14. Barrett's esophagus and eosinophilic esophagitis in a young pediatric patient with esophageal atresia

    Directory of Open Access Journals (Sweden)

    Li-Zsa Tan

    2015-07-01

    Full Text Available We report the case and follow up of a 4 year old girl who was born with esophageal atresia and was found to have concomitant Barrett's esophagus and eosinophilic esophagitis. Development of metaplasia has generally been regarded as a chronic, long term complication post esophageal atresia repair, however this case highlights the need for early and regular endoscopic surveillance in this vulnerable patient population. In addition to this, eosinophilic esophagitis has only rarely been reported in patients with concomitant Barrett's esophagus. Our patient is the youngest in reported literature with Barrett's changes post esophageal atresia repair, and the first such reported patient with both eosinophilic esophagitis and Barrett's esophagus.

  15. MR Imaging of Fetal Gastrointestinal System Anomaly%MRI在胎儿消化系统发育异常中的应用

    Institute of Scientific and Technical Information of China (English)

    胡晓华; 罗先富; 李洁; 陈文新; 陈娟; 王军; 吴晶涛

    2011-01-01

    Objective To explore the diagnostic value of MRI in fetal congenital anomaly of gastrointestinal system. Materials and Methods Ten pregnant women with gestation from 20 to 36 weeks with gastrointenslinal system anomalies diagnosed by US received MR imaging,SSFSE sequence was performed in the coronal, sagittal and axial plane of the fetus body and the head. MRI findings were compared with autopsy. Results In these 10 fetuses,2 cases were small bowel atre-sia confirmed by autopsy,one of the cases was obstructed in the proximal of the jejunum,the another was obstructed in the middle of ileum with ascites, most of the dilated bowel appeared hyperintensity signal; congenital anal atresia in 5 cases, hy-pointensity meconium was seen in the dilated bowel; diaphragmatic hernia in 2 cases which all were in the left sided, in-trathoracic herniated organs in one case was the stomach,another was bowel; abdominal mass in one case, which appeared as hyperintensity signal in the abdomen was congenital mesenteric cyst confirmed by autopsy. Conclusion Prenatal MR] is effective in the assessment of fetal congenital anomaly of gastrointestinal system.%目的 探讨MRI在胎儿消化系统发育异常方面的应用价值.资料与方法 对10例孕20周以上超声提示存在消化系统发育异常的胎儿行MR检查.采用单次激发快速自旋回波序列(SSFSE),选择胎儿头颅、躯干的常规体位,并重点行胎儿躯干横断位、冠状位、矢状位扫描,将产前MR表现与引产后尸体解剖结果相对照.结果 10例消化系统发育异常胎儿中,先天性小肠闭锁2例;引产后尸检证实闭锁部位分别为空肠近段和回肠中段,后者同时合并胎儿腹腔积液,SSFSE上见扩张肠管以高信号为主;先天性肛门闭锁5例,SSFSE上扩张肠管内可见低信号胎粪;膈疝2例,均位于左侧,疝入物1例为胃腔、1例为部分肠管,MR图像上表现为高信号胃腔、肠管上升至心脏水平;腹部包块1例,引产

  16. Surgical management of agenesis of the vulva with atresia ani-et-distal recti in a heifer calf: A case report

    Directory of Open Access Journals (Sweden)

    Moses N Wamaitha

    2015-09-01

    Full Text Available Successful surgical intervention of atresia ani-et-distal recti with agenesis of vulva in a heifer calf is reported in this paper. The calf aging 1-day was presented with a history of swelling at the perineal region. In clinical examination, both vulval and anal openings were found absent. Initial surgical operation under sedation and local anesthesia was done to correct these abnormalities. As a result, the calf could urinate, but attempted defecation was not successful. Surgical operation was done again at the anal region to resolve this condition. Post-operatively, tetracycline spray was applied on both surgical wounds immediately after surgery, and repeated twice daily for 10 days. Amoxicillin trihydrate (at 750 mg/kg bwt and Phenylbutazone (at 400 mg/kg bwt were administered intramuscularly once, and the treatment was repeated on every alternate day up to 10 days of post-surgery. Finally, the calf was able to urinate and defecate normally, and the surgical wounds healed uneventfully within 14 days. [J Adv Vet Anim Res 2015; 2(3.000: 369-372

  17. Elements of an anal dysplasia screening program.

    Science.gov (United States)

    Jay, Naomi

    2011-01-01

    The incidence of anal cancer in HIV-infected men who have sex with men (MSM) is highly elevated compared to the general population, as is the incidence of its precursor lesion, high-grade anal intraepithelial neoplasia (HGAIN). MSM in general and other immunocompromised populations are also at higher risk. Treatment of HGAIN may prevent development of cancer, similar to the decrease in cervical cancers that has occurred since the advent of cervical cancer screening programs in women. Cervical cancer screening tools have been adapted and validated for screening, diagnosis, and treatment of anal HGAIN. Anal cancer screening programs have now been available for more than a decade, although they are not yet standards of care. Incorporating screening procedures into practice depends on the available resources in a particular community. This article discusses the procedures for anal cancer screening including cytology, digital anal rectal examinations, high-resolution anoscopy, and biopsy. PMID:22035526

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

    DEFF Research Database (Denmark)

    Nissling, A.; Thorsen, A.; da Silva, Filipa

    2016-01-01

    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......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 ‘dissectormethod’ (10⋅6% adjustment; coefficient of determination was 0⋅675 between methods). Both prevalence (portion of fish with atresia) and intensity (calculated...

  19. Aortic arch in neonates with esophageal atresia: Preoperative assessment using CT

    Energy Technology Data Exchange (ETDEWEB)

    Day, D.L.

    1985-04-01

    Preoperative localization of the aortic arch is important in infants with esophageal atresia to prevent morbidity and death from an ipsilateral surgical approach. Computed tomography is an accurate, noninvasive technique for determining the position of the aortic arch.

  20. Hypertrophic Pyloric Stenosis Following Repair of Esophageal Atresia and Tracheo-Esophageal Fistula

    OpenAIRE

    Emmanuelle Seguier-Lipszyc; Baruch Klin

    2014-01-01

    Two cases of hypertrophic pyloric stenosis (HPS) developed after a few weeks of repair of an esophageal atresia and tracheo-esophageal fistula (EA&TEF). Both cases were dealt successfully with laparoscopic pyloromyotomy.

  1. Hypertrophic Pyloric Stenosis Following Repair of Esophageal Atresia and Tracheo-Esophageal Fistula

    Directory of Open Access Journals (Sweden)

    Emmanuelle Seguier-Lipszyc

    2014-07-01

    Full Text Available Two cases of hypertrophic pyloric stenosis (HPS developed after a few weeks of repair of an esophageal atresia and tracheo-esophageal fistula (EA&TEF. Both cases were dealt successfully with laparoscopic pyloromyotomy.

  2. Syndromic association of cleft palate, bilateral choanal atresia, curly hair, and congenital hypothyroidism.

    OpenAIRE

    Buntincx, I M; Van Overmeire, B; Desager, K; Van Hauwaert, J

    1993-01-01

    We describe a patient with the unusual association of cleft palate, bilateral choanal atresia, curly hair, and congenital hypothyroidism. This association has been reported before in two brothers and may represent a new syndrome.

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

    Science.gov (United States)

    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.

  4. Tracheobronchial Branching Anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Min Ji; Kim, Young Tong; Jou, Sung Shick [Soonchunhyang University, Cheonan Hospital, Cheonan (Korea, Republic of); Park, A Young [Soonchunhyang University College of Medicine, Asan (Korea, Republic of)

    2010-04-15

    There are various congenital anomalies with respect to the number, length, diameter, and location of tracheobronchial branching patterns. The tracheobronchial anomalies are classified into two groups. The first one, anomalies of division, includes tracheal bronchus, cardiac bronchus, tracheal diverticulum, pulmonary isomerism, and minor variations. The second one, dysmorphic lung, includes lung agenesis-hypoplasia complex and lobar agenesis-aplasia complex

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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

  7. Thoracoscopic traction technique in long gap esophageal atresia: entering a new era

    OpenAIRE

    van der Zee, David C.; Gallo, Gabriele; Tytgat, Stefaan H. A.

    2015-01-01

    Objective To describe the evolution from delayed management of long gap esophageal atresia to thoracoscopic treatment directly after birth without the placement of a gastrostomy. Background Long gap esophageal atresia remains a challenge for pediatric surgeons. Over the years, several techniques have been described to deal with the problem of the distance between the proximal and distal esophagus. More recently, a traction technique has been advocated. With the advent of minimal invasive surg...

  8. Persistent bronchography in a newborn with esophageal atresia.

    Science.gov (United States)

    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

  9. Persistent bronchography in a newborn with esophageal atresia

    Directory of Open Access Journals (Sweden)

    Giuseppe De Bernardo, MD

    2016-06-01

    Full Text Available 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.

  10. Fetal lung growth in laryngeal atresia and tracheal agenesis.

    Science.gov (United States)

    Scurry, J P; Adamson, T M; Cussen, L J

    1989-02-01

    Three cases of airway obstruction in fetuses born at 21, 32 and 40 weeks gestation are reported. The first had laryngeal atresia, cystic dysplastic kidneys, oligohydramnios and immense fluid-filled lungs. The second had upper tracheal agenesis, a tracheo-oesophageal fistula, a cystic dysplastic horseshoe kidney, oligohydramnios and normal-sized lungs. The third had a pin-hole mucosal tract through an otherwise atretic larynx, normal kidneys, no oligohydramnios and normal-sized lungs. Lung weight:body weight ratios, radial alveolar or radial canalicular counts and point-counting of sections of lungs in cases 1 and 2 show that laryngeal or tracheal obstruction may prevent or reduce the pulmonary hypoplasia associated with renal dysplasia, and in cases 2 and 3, that grossly enlarged, hyperplastic lungs may not be seen unless obstruction is complete. PMID:2730470

  11. Ovarian Follicular Atresia of Ewes during Spring Puerperium

    Directory of Open Access Journals (Sweden)

    Radoslava Vlčková

    2012-01-01

    Full Text Available 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.

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

    Science.gov (United States)

    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

  13. Bilateral congenital choanal atresia and osteoma of ethmoid sinus with supernumerary nostril: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Li Xue-zhong

    2011-12-01

    Full Text Available Abstract Introduction Congenital choanal atresia is a relatively rare deformity, especially bilateral congenital choanal atresia. We report a case of bilateral congenital choanal atresia in a 22-year-old Chinese man, who was also diagnosed with congenital right accessory nasal deformity, osteoma of his left ethmoid sinus and congenital keratoleukoma of his right eye. Case presentation A 22-year-old Chinese man presented with mouth breathing, sleep snoring and difficult feeding after birth, with no olfactory sensation. Three-dimensional computed tomography revealed bilateral choanal atresia and a high density bony shadow in his left ethmoid sinus that extended to his left frontal sinus. Conclusions Choanal atresia is often accompanied by other congenital abnormalities. To the best of our knowledge, this is the first report of choanal atresia accompanied by congenital accessory nasal deformity and congenital keratoleukoma.

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

    Science.gov (United States)

    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.

  15. OVARIAN FOLLICLE ATRESIA IN DAIRY COWS IN RELATION TO THE BODY CONDITION

    Directory of Open Access Journals (Sweden)

    Elena Kubovičova

    2011-12-01

    Full Text Available The aim of the study was to define types of atresia in cow ovarian follicles in relation to their body condition. The ovaries were recovered from slaughtered cyclic Czech Fleckvieh dairy cows of three body condition scores: BCS1 (emaciation, BCS2 (tendency to emaciation and BCS3 (average body condition status, classified according to a 5-point scale of BCS. The ovarian tissue sections were processed for histological analysis and stained with basic fuchsine and toluidine blue. For acidic mukopolysacharides a combination of PAS-technique with Alcian blue was used. In cows with BCS 1 the percentage of ovulated follicles was decreased up to 19% compared to the BCS 2 (76.6% or BCS 3 (68.7% animals. Among the non-ovulated follicles the ratio of cystic atresia was highly increased in cow with BCS 1 and 2 compared to the BCS 3. Also, the ratio of atresia associated with luteinization was substantially higher in cows with lower BCS grade (BCS 1, when compared with cows with average body condition status (BCS 3. No significant differences in the occurrence of obliterative atresia among cows with different BCS grades were observed. Higher occurrence of acidic mukopolysaccharides in small and large antral follicles from cows with lower BCS (1 and 2 was revealed. Our observations indicate that in emaciated dairy cows the most frequent forms of follicle degeneration are cystic atresia and atresia with luteinization.

  16. Posttraumatic Stress Reactions in Parents of Children Esophageal Atresia.

    Directory of Open Access Journals (Sweden)

    Morgane Le Gouëz

    Full Text Available The aim of this study was to investigate psychological stress in parents of children with esophageal atresia and to explore factors associated with the development of Posttraumatic Stress disorder (PTSD.Self-report questionnaires were administered to parents of children with EA. Domains included: (1 sociodemographic data, current personal difficulties, assessment scales for the quality of life and for the global health status of the child (2 French-validated versions of the Perinatal Posttraumatic Stress disorder Questionnaire and of the State-Trait Anxiety Inventory. Associations between PTSD and severity of the neonatal course, presence of severe sequelae at 2 years of age, and quality of life and global health status of children according to their parents' perception were studied.A Tertiary care University Hospital.Among 64 eligible families, 54 parents of 38 children (59% participated to the study. PTSD was present in 32 (59% parents; mothers were more frequently affected than fathers (69 vs 46%, p = 0.03. Four mothers (8% had severe anxiety. PTSD was neither associated with neonatal severity nor with severe sequelae at 2 years. Parents with PTSD rated their child's quality of life and global health status significantly lower (7.5 vs 8.6; p = 0.01 and 7.4 vs 8.3; p = 0.02 respectively.PTSD is frequent in parents of children with esophageal atresia, independently of neonatal severity and presence of severe sequelae at 2 years of age. Our results highlight the need for a long-term psychological support of families.

  17. Atresia of large ovarian follicles of the rat

    Directory of Open Access Journals (Sweden)

    Maria Słomczyn´ ska

    2011-08-01

    Full Text Available In the rat, at the beginning of pregnancy a cohort of antral follicles develops until the preovulatory stage. However, these follicles, differentiating in the hyperprolactinemic milieu, produce only small amount of estradiol, do not ovulate and undergo rapid degeneration. They constitute an interesting physiological model of atresia. In the present study, we analysed the development and subsequent degeneration of such follicles. The study was performed on Wistar female rats killed in succession between days 1-9 of pregnancy. Excised ovaries were submitted to a routine histological procedure. Paraffin sections were subjected to hematoxylin and eosin staining or in situ DNA labelling. Histological and TUNEL staining revealed that the investigated group of follicles grew slower than that on the corresponding days of the estrous cycle and reached a preovulatory size and morphological appearance on day 5 of pregnancy. They did not ovulate and between days 6 and 9 of pregnancy an increasing number of apoptotic cells appeared within these follicles. They were localized predominantly in the antral granulosa layer, especially near the cumulus oophorus complex (COC and in the region linking the COC with the follicular wall. The COC and the theca layer were much less affected. In late stages of atresia, also cumulus cells became apoptotic but degenerating oocytes did not exhibit positive TUNEL staining. Only limited number of the theca cells have undergone apoptosis and generally they were not hypertrophied. Our findings indicate that much smaller than normal amount of intrafollicular estradiol was sufficient to support a normal, according to the morphological criteria, although slower development of antral follicles to the late preovulatory stage.

  18. Viscoelastic assessment of anal canal function using acoustic reflectometry

    DEFF Research Database (Denmark)

    Mitchell, Peter J; Klarskov, Niels; Telford, Karen J;

    2012-01-01

    Anal acoustic reflectometry is a new reproducible technique that allows a viscoelastic assessment of anal canal function. Five new variables reflecting anal canal function are measured: the opening and closing pressure, opening and closing elastance, and hysteresis.......Anal acoustic reflectometry is a new reproducible technique that allows a viscoelastic assessment of anal canal function. Five new variables reflecting anal canal function are measured: the opening and closing pressure, opening and closing elastance, and hysteresis....

  19. Esophageal Anastomosis Medial to Preserved Azygos Vein in Esophageal Atresia with Tracheoesophageal Fistula: Restoration of Normal Mediastinal Anatomy

    Directory of Open Access Journals (Sweden)

    Kumar Abdul Rashid

    2012-09-01

    Full Text Available Objective: We intended to prospectively study the technical feasibility and advantages of esophageal anastomosis medial to the preserved azygos vein in neonates diagnosed with esophageal atresia with tracheoesophageal fistula (EA/TEF. The results were compared to the cases where azygos vein was either not preserved, or the anastomosis was done lateral to the arch of preserved azygos vein. Material and methods: A total of 134 patients with EA/TEF were admitted between January 2007 and July 2008 of which 116 underwent primary repair. Eleven patients with long gap esophageal atresia with or without tracheoesophageal fistula and 7 patients who expired before surgery were excluded. Patients were randomly divided in three groups comparable with respect to the gestational age, age at presentation, sex, birth weight, associated anomalies and the gap between the pouches after mobilization: Group A (azygos vein ligated and divided, Group B (azygos vein preserved with esophageal anastomosis lateral to the vein, and Group C azygos vein preserved with esophageal anastomosis medial to the vein. All the patients were operated by extra-pleural approach. The three groups were compared with respect to operative time and early postoperative complications like pneumonitis, anastomotic leaks and mortality. Odds ratio and Chi square test were used for the statistical analysis. Results: Group A, B and C had 35, 43 and 38 patients respectively. No significant difference was observed in average operative time in the 3 groups. Though incidence of postoperative pneumonitis was higher in group A (28% as compared to group B (13.95% and group C (11.62%, it was not statistically significant (p > 0.005. Anastomotic leak occurred in 7 patients in group A (20%, 6 patients in group B (13.95% and 4 patients (10.52% in group C (p > 0.005. Group A had 3 major and 4 minor anastomotic leaks; group B had 2 major and 4 minor leaks and group C had 1 major and 3 minor leaks. There were10

  20. Nocturnal faecal soiling and anal masturbation.

    OpenAIRE

    A. F. Clark; Tayler, P J; Bhate, S R

    1990-01-01

    Two cases of late onset faecal soiling as a result of anal masturbation in children who were neither mentally handicapped nor psychotic were studied. The role of soiling in aiding the young person and his family to avoid separating and maturing is highlighted. We suggest that the association of anal masturbation and resistant nocturnal soiling may be unrecognised.

  1. Synchronous rectal adenocarcinoma and anal canal adenocarcinoma

    Institute of Scientific and Technical Information of China (English)

    GU Jin; LI Jiyou; YAO Yunfeng; LU Aiping; WANG Hongyi

    2007-01-01

    It is difficult to distinguish a tectal carcinoma with anal metastases from coexistent synchronous anorectal carcinomas.The therapeutic strategy for rectal and anal carcinoma is so different that it should be clearly identified.Here,we report on the case of a 63-year-old man who presented with an upper-third rectal adenocarcinoma.Five months after resection,he developed an adenocarcinoma in the anal canal.The histological slides of both tumors were reviewed and immunohistochemical studies for cytokeratins(CKs)7 and 20 were performed.The index tumor demonstrated CK 7-/CK 20+and the second showed CK7+/CK20+.For this reason,we believe the present case had synchronous adenocarcinomas arising from anal canal and the rectum separately.It is very important to difierentiate the anorectal lesions pathologically because of the impact on the therapeutic options available,especially for the lesion arising in the anal canal.

  2. Supra-transumbilical laparotomy (STL approach for small bowel atresia repair: Our experience and review of the literature

    Directory of Open Access Journals (Sweden)

    Ernesto Leva

    2013-01-01

    Full Text Available Background: Supra-Transumbilical Laparotomy (STL has been used in paediatric surgery for a broad spectrum of abdominal procedures. We report our experience with STL approach for small bowel atresia repair in newborns and review previous published series on the topic. Patients and Methods: Fourteen patients with small bowel atresia were treated via STL approach at our Institution over a 5-year period and their charts were retrospectively reviewed. Results: STL procedure was performed at mean age of 3.1 day. No malrotation disorders were detected with pre-operative contrast enema. Eight patients (54.1% presented jejunal atresia, five (35.7% ileal atresia, and one (7.1% multiple ileal and jejunal atresias. Standard repair with primary end-to-back anastomosis was performed in all but one patient. In the newborn with multiple atresia, STL incision was converted in supra-umbilical transverse incision due to difficulty of exposition. After surgery, one patient developed anastomotic stricture, and another developed occlusion due to adhesions: Both infants required second laparotomy. No infections of the umbilical site were recorded, and cosmetic results were excellent in all patients. Conclusions: Increasing evidence suggests that STL approach for small bowel atresia is feasible, safe and provides adequate exposure for small bowel atresia surgery. When malrotation and colonic/multiple atresia are pre-operatively ruled out, STL procedure can be choosen as first approach.

  3. Usefulness of intraoperative bronchoscopy during surgical repair of a congenital cardiac anomaly with possible airway obstruction: three cases report.

    Science.gov (United States)

    Oh, JongEun; Kim, Jung-Won; Shin, Won-Jung; Gwak, Mijeung; Park, Pyung Hwan

    2016-02-01

    Compression of the airway is relatively common in pediatric patients, although it is often an unrecognized complication of congenital cardiac and aortic arch anomalies. Aortopexy has been established as a surgical treatment for tracheobronchial obstruction associated with vascular anomaly, aortic arch anomaly, esophageal atresia, and tracheoesophageal fistula. The tissue-to-tissue arch repair technique could result in severe airway complication such as compression of the left main bronchus which was not a problem before the correction. We report three cases of corrective open heart surgery monitored by intraoperative bronchoscopy performed during prebypass, and performed immediately before weaning from bypass, to evaluate tracheobronchial obstruction caused by congenital, complex cardiac anomalies in the operating room. PMID:26885306

  4. Double-outlet right ventricle with absent left ventricle and mitral atresia in a fetus with a deletion 22q12.

    Science.gov (United States)

    L'herminé-Coulomb, Aurore; Houyel, Lucille; Aboura, Azzedine; Audibert, François; Dal Soglio, Dorothée; Tachdjian, Gérard

    2004-09-01

    Interstitial deletions of chromosomal region 22q12 are rare. We report the prenatal diagnosis of a de novo interstitial deletion 22q12. The fetus was karyotyped because of a complex cardiac anomaly. Conventional and molecular cytogenetics showed a female karyotype with a de novo pericentric inversion of one chromosome 22 associated with a deletion of the chromosomal region 22q12 leading to a partial monosomy 22q12. At autopsy, the fetus showed double-outlet right ventricle (DORV) with absent left ventricle and mitral atresia. This observation suggests that one or several genes for the early looping step of heart development may reside in chromosomal region 22q12. Further studies are needed to identify these genes, and to search microdeletions of 22q12 region in patients with DORV.

  5. Thrombectomy and surgical reconstruction for extensive iliocaval thrombosis in a patient with agenesis of the retrohepatic vena cava and atresia of the left renal vein.

    Science.gov (United States)

    La Spada, Michele; Stilo, Francesco; Carella, Giuseppe; Salomone, Ignazio; Benedetto, Filippo; De Caridi, Giovanni; Spinelli, Francesco

    2011-08-01

    In 80% of the patients presenting with deep-venous thrombosis (DVT), a risk factor can be identified. An absent or hypoplastic infrarenal vena cava is a rare risk factor for DVT in young adults. In these cases, the prevalence of congenital anomalies of the inferior vena cava (IVC) is estimated at 0.5% of the general population, up to 5% in young people. The association with coagulopathy increases the risk of DVT. We report a case of a young man who presented with a massive caval and iliofemoral-popliteal thrombosis in presence of the agenesis of retrohepatic inferior vena cava and atresia of the left renal vein. Open thrombectomy and caval reconstruction with a polytetrafluoroethylene graft were performed. Surgical option with vein reconstruction was preferred to prevent new episodes of thrombosis and the risk of acute renal failure.

  6. Intestinal atresia, encephalocele, and cardiac malformations in infants with 47,XXX: Expansion of the phenotypic spectrum and a review of the literature.

    Science.gov (United States)

    Bağci, Soyhan; Müller, Andreas; Franz, Axel; Heydweiller, Andreas; Berg, Christoph; Nöthen, Markus M; Bartmann, Peter; Reutter, Heiko

    2010-01-01

    Identification of the 47,XXX karyotype often occurs adventitiously during prenatal fetal karyotyping in cases of advanced maternal age. Although most females with 47,XXX appear healthy at birth, various types of congenital malformations have been reported, of which urinary tract anomalies are the most frequent. We report on 2 newborns with 47,XXX and congenital cardiac defects, one of whom had duodenal atresia and the other an occipital encephalocele. This expands the spectrum of malformations reported in association with the triple-X syndrome. We also present a review of the literature on non-urinary tract malformations in females with 47,XXX. We conclude that prenatal identification of the 47,XXX karyotype is an indication for detailed fetal ultrasonography which should include examination of multiple organ systems. Such prenatal screening for possible associated congenital malformations should help to ensure optimal perinatal clinical management of 47,XXX cases.

  7. Atresia das vias biliares extra-hepáticas: conhecimentos atuais e perspectivas futuras Extrahepatic biliary atresia: current concepts and future directions

    Directory of Open Access Journals (Sweden)

    Elisa de Carvalho

    2007-04-01

    Full Text Available OBJETIVOS: Apresentar uma revisão atualizada sobre atresia das vias biliares extra-hepáticas, com ênfase em etiopatogenia, abordagens diagnósticas e terapêuticas e prognóstico. FONTES DOS DADOS: Foram selecionadas pelos sites de busca médica (MEDLINE e PubMed pesquisas relacionadas à atresia biliar, utilizando as seguintes palavras-chave: biliary atresia,etiopathogenesis, diagnosis, treatment, prognosis, children. SÍNTESE DOS DADOS A atresia das vias biliares extra-hepáticas é a principal indicação de transplante hepático na faixa etária pediátrica. Quanto à etiologia, o citomegalovírus, o reovírus e o rotavírus têm sido os agentes mais estudados como possíveis desencadeadores da obstrução imunomediada da árvore biliar. A resposta imune, especialmente o predomínio da resposta TH1 e do interferon gama, a susceptibilidade genética e as alterações do desenvolvimento embrionário da árvore biliar são aspectos que podem participar na etiopatogênese da obliteração das vias biliares extra-hepáticas. Ainda hoje, o único tratamento disponível é a portoenterostomia, cujos resultados são melhores quando realizada nos primeiros 2 meses de vida. Em relação ao prognóstico, as crianças não tratadas vão a óbito na totalidade, por complicações relacionadas à hipertensão portal e à cirrose hepática, e mesmo os casos tratados necessitam, em sua maioria, do transplante hepático. CONCLUSÃO: A atresia das vias biliares extra-hepáticas continua sendo a principal indicação de transplante hepático em crianças, e a mudança deste panorama depende de um melhor entendimento da etiopatogenia da obstrução biliar nos diferentes fenótipos da doença. Investigações futuras a respeito do papel do interferon gama e de outras citocinas são necessárias para avaliar se esses aspectos seriam potenciais alvos de intervenção terapêutica.OBJECTIVE: To provide an updated review on extrahepatic biliary atresia, focusing

  8. Limb mammary syndrome: a new genetic disorder with mammary hypoplasia, ectrodactyly, and other Hand/Foot anomalies maps to human chromosome 3q27.

    OpenAIRE

    Van Bokhoven, H.; Jung, M; Smits, A P; van Beersum, S.; Rüschendorf, F; Van Steensel, M; Veenstra, M; Tuerlings, J.H.; Mariman, E.C.; Brunner, H.G.; Wienker, T. F.; Reis, A.; Ropers, H H; Hamel, B C

    1999-01-01

    We report on a large Dutch family with a syndrome characterized by severe hand and/or foot anomalies, and hypoplasia/aplasia of the mammary gland and nipple. Less frequent findings include lacrimal-duct atresia, nail dysplasia, hypohydrosis, hypodontia, and cleft palate with or without bifid uvula. This combination of symptoms has not been reported previously, although there is overlap with the ulnar mammary syndrome (UMS) and with ectrodactyly, ectodermal dysplasia, and clefting syndrome. Al...

  9. Anal intraepithelial neoplasia: review and recommendations for screening and management.

    Science.gov (United States)

    Smyczek, Petra; Singh, Ameeta E; Romanowski, Barbara

    2013-11-01

    Anal cancer is a rare malignancy of the distal gastrointestinal tract, often associated with human papillomavirus, the most common sexually transmitted infection worldwide. Currently available screening methods for anal intraepithelial neoplasia, a precursor for anal cancer, combine anal Papanicolaou cytology and high resolution anoscopy with biopsy of suspicious lesions. Significant barriers to establishing anal cancer screening programmes include the small number of healthcare professionals performing high resolution anoscopy and the lack of data showing that anal cancer screening can reduce morbidity and mortality related to anal carcinoma. Despite several controversies surrounding anal cancer screening, the rising incidence of this disease in some groups supports routine screening programmes in high-risk populations, especially in HIV-positive men who have sex with men. This review outlines the epidemiology of anal intraepithelial neoplasia and anal cancer and summarizes issues related to the introduction of anal cancer screening programmes. PMID:23970583

  10. Anal cancer and intraepithelial neoplasia screening: Areview

    Institute of Scientific and Technical Information of China (English)

    2016-01-01

    This review focuses on the early diagnosis of anal cancer and its precursor lesions through routine screening. Anumber of risk-stratification strategies as well as screeningtechniques have been suggested, and currently littleconsensus exists among national societies. Much ofthe current clinical rationale for the prevention of analcancer derives from the similar tumor biology of cervicalcancer and the successful use of routine screening toidentify cervical cancer and its precursors early in thedisease process. It is thought that such a strategy ofidentifying early anal intraepithelial neoplasia will reducethe incidence of invasive anal cancer. The low prevalenceof anal cancer in the general population prevents theuse of routine screening. However, routine screeningof selected populations has been shown to be a morepromising strategy. Potential screening modalities includedigital anorectal exam, anal Papanicolaou testing, humanpapilloma virus co-testing, and high-resolution anoscopy.Additional research associating high-grade dysplasiatreatment with anal cancer prevention as well as directcomparisons of screening regimens is necessary todevelop further anal cancer screening recommendations.

  11. Prophylactic HPV vaccination and anal cancer.

    Science.gov (United States)

    Stier, Elizabeth A; Chigurupati, Nagasudha L; Fung, Leslie

    2016-06-01

    The incidence of anal cancer is increasing. High risk populations include HIV-positive men who have sex with men (MSM), HIV-negative MSM, HIV-positive women and heterosexual men and women with a history of cervical cancer. HPV has been detected in over 90% of anal cancers. HPV16 is the most common genotype detected in about 70% of anal cancers. The quadrivalent HPV (qHPV) vaccine has been demonstrated to prevent vaccine associated persistent anal HPV infections as well as anal intraepithelial neoplasia grades 2-3 (AIN2+) in young MSM not previously infected. A retrospective analysis also suggests that qHPV vaccination of older MSM treated for AIN2+ may significantly decrease the risk of recurrence of the AIN2+. The HPV types detected in anal cancer are included in the 9-valent vaccine. Thus, the 9-valent HPV vaccine, when administered to boys and girls prior to the onset of sexual activity, should effectively prevent anal cancer. PMID:26933898

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

    Science.gov (United States)

    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

  13. Anal erogeneity: the goose and the rat.

    Science.gov (United States)

    Shengold, L

    1982-01-01

    A case is presented in which the patient's traumatically derived intense anal erogeneity (associated with traumatic anxiety as well as with castration anxiety) inhibited his phallic sensations and potency and also his power to sustain productive thought. His passive cravings were disguised and reacted against in his compulsive-exhibitionistically phallic role of a Don Juan. He described at least two levels of anal feelings: a dangerous but exciting, tolerable or even pleasurable tension associated with the imago of the goose; and an unbearable, terrifying overcharged level embodied in the imago of the rat. (He had read of, and had felt himself identified with, Freud's Rat Man.) Contrasts are presented with François Rabelais' account of the instinctual development and anal training of Gargantua, in which the connotations of the goose lead to a happy anal, phallic and intellectual control. Generalizations are ventured about the crucial attainment of command over the anal sphincter for the taming of 'primal affect'(Fliess). With early psychopathology there is a defensive overcathexis of anal control (and of anal mechanisms and character traits) to try to contain over-stimulation. In contrast true anal mastery contributes to the acquisition of optimal genital feelings and functioning and to the capacity for sustaining integrative thinking so necessary for 'owning' one's affects and impulses, and therefore for a feeling of identity. Finally, some remarks of Freud on Rabelais are reviewed in relation to levels of urethral erogeneity, seen as developmental way stations between the anal and the phallic, and partaking of both.

  14. Familial Poland anomaly.

    OpenAIRE

    David, T J

    1982-01-01

    The Poland anomaly is usually a non-genetic malformation syndrome. This paper reports two second cousins who both had a typical left sided Poland anomaly, and this constitutes the first recorded case of this condition affecting more than one member of a family. Despite this, for the purposes of genetic counselling, the Poland anomaly can be regarded as a sporadic condition with an extremely low recurrence risk.

  15. VERJETNOSTNE VARNOSTNE ANALIZE JEDRSKE ELEKTRARNE V ZAUSTAVITVI

    OpenAIRE

    Antončič, Mitja

    2016-01-01

    Pričujoča magistrska naloga obravnava verjetnostne varnostne analize jedrske elektrarne v zaustavitvenih stanjih. Verjetnostne varnostne analize so namenjene ocenjevanju in izboljšanju varnosti kompleksnih sistemov, tudi jedrskih elektrarn. Skozi analizo izvemo možne neželene dogodke, do katerih lahko pride v sistemu, verjetnost nastopa teh dogodkov, način njihovega razvoja in končne posledice. Na podlagi analize lahko določimo pomembnost posameznih komponent, kar je koristna informacija pri ...

  16. Relation between electromyography and anal manometry of the external anal sphincter.

    OpenAIRE

    Sørensen, M; Tetzschner, T; Rasmussen, O O; Christiansen, J

    1991-01-01

    Thirteen patients with faecal incontinence and 26 control subjects were studied to investigate whether a quantitative electromyographic (EMG) signal could be correlated to anal manometry. Three different electrodes were used--a concentric needle electrode, a disposable sponge electrode, and a hard anal plug electrode. The maximum amplitude of the EMG recording was used as a quantitative parameter. Linear regression showed significant correlation between EMG and anal manometry with the sponge ...

  17. Ultrasound-guided percutaneous cholecysto-cholangiography for the exclusion of biliary atresia in infants

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Kyung Min; Ryeom, Hun Kyu; Choe, Byung Ho; Kim, Kap Cheol; Kim, Jong Yeol; Lee, Jong Min; Kim, Hye Jeong; Lee, Hee Jung [Kyungpook National University Hospital, Daegu (Korea, Republic of)

    2006-08-15

    The aim of this study is to determine the feasibility and effectiveness of performing an ultrasound-guided percutaneous cholecysto-cholangiogram (PCC) for excluding biliary atresia as the cause of neonatal jaundice. Between Oct. 2003 and Feb. 2005, six ultrasound-guided PCC procedures were performed to five jaundiced infants (4 females and 1 male; mean age: 60 days old) for whom possibility of biliary atresia could not be ruled out by the DISIDA scan as the cause of their neonatal jaundice. Gallbladder puncture was performed under ultrasound guidance with a 23-gauge needle. Contrast material injection during fluoroscopic examination was performed after dilatation of the gallbladder lumen with normal saline under ultrasound guidance. The criteria used for excluding biliary atresia were complete visualization of the extrahepatic biliary trees and/or contrast excretion into the duodenum. The complications and final diagnosis was assessed according to the clinical and laboratory findings. The procedures were successful in all the patients without any complication. Biliary atresia could be ruled out in all the patients. The final diagnosis was neonatal cytomegalovirus hepatitis in two patients, total parenteral nutrition-associated cholestasis in two patients, and combined cytomegalovirus hepatitis and total parenteral nutrition-associated cholestasis in one patient. Ultrasound-guided PCC is a feasible and effective method for the early definitive exclusion of biliary atresia as the cause of neonatal jaundice. By the technique of injecting normal saline before contrast injection, PCC can be done even in a totally collapsed or very small gallbladder.

  18. Association of Nasal Nostril Stenosis with Bilateral Choanal Atresia: A Case Report

    Directory of Open Access Journals (Sweden)

    Shahin Abdollahifakhim

    2014-01-01

    Full Text Available Introduction: Neonatal nasal airway obstruction induces various degrees of respiratory distress. The management of this disease, including surgical repair, will depend on the severity and location of the obstruction. We describe here a case of congenital nasal nostril stenosis that required surgical repair for stenting of both nares after coanal atresia repair.   Case Report: A 2 days old female newborn referred to neonatal department of Tabriz Children’s Hospital affiliated to the University of Medical Sciences of Tabriz, Iran on the 3rd of December, 2011 immediately after birth with respiratory distress due to bilateral coanal atresia and nasal hypoplasia with very small nostrils. CT scan showed normal brain and bilateral choanal atresia with normal size Pyriform apertures.   Conclusion: Nasal obstruction can lead to airway compromise and respiratory distress. Congenital bony nasal deformities are being recognized as an important cause of newborn airway obstruction. Nasal hypoplasia is seen in many craniofacial syndromes. Although our patient had hypoplastic nostrils with respiratory distress due to bilateral coanal atresia, correction of hypoplastic nostrils was necessary for completing the operation of choanal atresia.

  19. Ultrasound-guided percutaneous cholecysto-cholangiography for the exclusion of biliary atresia in infants

    International Nuclear Information System (INIS)

    The aim of this study is to determine the feasibility and effectiveness of performing an ultrasound-guided percutaneous cholecysto-cholangiogram (PCC) for excluding biliary atresia as the cause of neonatal jaundice. Between Oct. 2003 and Feb. 2005, six ultrasound-guided PCC procedures were performed to five jaundiced infants (4 females and 1 male; mean age: 60 days old) for whom possibility of biliary atresia could not be ruled out by the DISIDA scan as the cause of their neonatal jaundice. Gallbladder puncture was performed under ultrasound guidance with a 23-gauge needle. Contrast material injection during fluoroscopic examination was performed after dilatation of the gallbladder lumen with normal saline under ultrasound guidance. The criteria used for excluding biliary atresia were complete visualization of the extrahepatic biliary trees and/or contrast excretion into the duodenum. The complications and final diagnosis was assessed according to the clinical and laboratory findings. The procedures were successful in all the patients without any complication. Biliary atresia could be ruled out in all the patients. The final diagnosis was neonatal cytomegalovirus hepatitis in two patients, total parenteral nutrition-associated cholestasis in two patients, and combined cytomegalovirus hepatitis and total parenteral nutrition-associated cholestasis in one patient. Ultrasound-guided PCC is a feasible and effective method for the early definitive exclusion of biliary atresia as the cause of neonatal jaundice. By the technique of injecting normal saline before contrast injection, PCC can be done even in a totally collapsed or very small gallbladder

  20. Anal Disorders - Multiple Languages: MedlinePlus

    Science.gov (United States)

    ... Supplements Videos & Tools You Are Here: Home → Multiple Languages → All Health Topics → Anal Disorders URL of this page: https://medlineplus.gov/languages/analdisorders.html Other topics A-Z A B ...

  1. Competing Orders and Anomalies.

    Science.gov (United States)

    Moon, Eun-Gook

    2016-01-01

    A conservation law is one of the most fundamental properties in nature, but a certain class of conservation "laws" could be spoiled by intrinsic quantum mechanical effects, so-called quantum anomalies. Profound properties of the anomalies have deepened our understanding in quantum many body systems. Here, we investigate quantum anomaly effects in quantum phase transitions between competing orders and striking consequences of their presence. We explicitly calculate topological nature of anomalies of non-linear sigma models (NLSMs) with the Wess-Zumino-Witten (WZW) terms. The non-perturbative nature is directly related with the 't Hooft anomaly matching condition: anomalies are conserved in renormalization group flow. By applying the matching condition, we show massless excitations are enforced by the anomalies in a whole phase diagram in sharp contrast to the case of the Landau-Ginzburg-Wilson theory which only has massive excitations in symmetric phases. Furthermore, we find non-perturbative criteria to characterize quantum phase transitions between competing orders. For example, in 4D, we show the two competing order parameter theories, CP(1) and the NLSM with WZW, describe different universality class. Physical realizations and experimental implication of the anomalies are also discussed. PMID:27499184

  2. Anomalies and topology

    International Nuclear Information System (INIS)

    The lectures given cover the topological effects in gauge field theories, fermionic chiral anomalies, and some relationships between the two. Gauge field theories in three and four space-time dimensions are considered. Topological terms as external U(1) functional gauge potential connections in field space are discussed. Both the structure and physical impact of anomalies are described. 17 refs

  3. Competing Orders and Anomalies

    Science.gov (United States)

    Moon, Eun-Gook

    2016-08-01

    A conservation law is one of the most fundamental properties in nature, but a certain class of conservation “laws” could be spoiled by intrinsic quantum mechanical effects, so-called quantum anomalies. Profound properties of the anomalies have deepened our understanding in quantum many body systems. Here, we investigate quantum anomaly effects in quantum phase transitions between competing orders and striking consequences of their presence. We explicitly calculate topological nature of anomalies of non-linear sigma models (NLSMs) with the Wess-Zumino-Witten (WZW) terms. The non-perturbative nature is directly related with the ’t Hooft anomaly matching condition: anomalies are conserved in renormalization group flow. By applying the matching condition, we show massless excitations are enforced by the anomalies in a whole phase diagram in sharp contrast to the case of the Landau-Ginzburg-Wilson theory which only has massive excitations in symmetric phases. Furthermore, we find non-perturbative criteria to characterize quantum phase transitions between competing orders. For example, in 4D, we show the two competing order parameter theories, CP(1) and the NLSM with WZW, describe different universality class. Physical realizations and experimental implication of the anomalies are also discussed.

  4. Laparoscopic cystojejunostomy for type I cystic biliary atresia in children

    Directory of Open Access Journals (Sweden)

    Alice Faure

    2015-01-01

    Full Text Available Purpose: The use of laparoscopy in the treatment of biliary atresia (BA is still debated. We report our strategy using laparoscopy in type I cystic BA. Materials and Methods: We reviewed the records of patients treated for BA from 2002-2013. When the diagnosis was suspected, an ultrasound was performed. If it showed a cyst > 5 mm in the hilum with no patent gallbladder, we performed an initial explorative laparoscopy. In the case of a patent biliary tree above the cyst, a laparoscopic cystojejunostomy was performed. In cases of absent communication (type III, conversion and portoenterostomy were performed. Pre and postoperative data and overall survival rate with the native liver were reviewed. Results: Forty-four children were treated for BA. Six presented with a cystic form diagnosed by US. Three children had type I BA; three had type III BA. No postoperative complications were noted. Median follow-up was 62.2 months (22.7-93.5. One patient died of a cardiac malformation. The five remaining patients are alive with their native liver. Of the 38 treated for noncystic BA, 16 were transplanted. Conclusion: We confirmed the prognosis of cystic BA, which is less severe than noncystic BA. Our strategy using laparoscopy allowed for the confirmation and qualification of the type of BA. In type I, complete treatment by laparoscopy has been performed safely.

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

    Science.gov (United States)

    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

  6. Anal intraepitelial neoplasia: a narrative review

    OpenAIRE

    Garazi Elorza; Yolanda Saralegui; José María Enríquez-Navascués; Carlos Placer; Leyre Velaz

    2016-01-01

    Anal intraepitelial neoplasia (AIN) constitutes a major health problem in certain risk groups, such as patients with immunosuppression of varied origin, males who have sexual relations with other males, and females with a previous history of vaginal or cervical abnormalities in cytology. Its relationship with the human papillomavirus (HPV) infection has been well documented; however, many of the factors involved in the progression and regression of the viral infection to dysplasia and anal ca...

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  9. Comparison of neonatal tolerance to thoracoscopic and open repair of esophageal atresia with tracheoesophageal fistula

    Institute of Scientific and Technical Information of China (English)

    MA Li; LIU Yong-zhe; MA Ya-qun; ZHANG Sheng-suo; PAN Ning-ling

    2012-01-01

    Background Advances in minimally invasive surgical techniques and neonatal intensive care for neonates have allowed for repair of the neonatal esophageal atresia with tracheoesophageal fistula (EA/TEF) to be approached endoscopically.However,thoracoscopic surgery in children is still performed in only a few centers throughout the world.The aim of this study was to compare the neonatal tolerance to the thoracoscopic repair (TR) and the open repair (OR)and also to discuss anesthetic management in thoracoscopic procedure.Methods We performed a prospective study enrolling newborns diagnosed with EA with distal TEF (type C) receiving the repair surgery between June 2009 and January 2012 in our institution.Data collected included the newborns' gestational age and weight at the time of the operation,operative time,parameters of intraoperative mechanical ventilation,oxygenation,end-tidal carbon dioxide (ETCO2),and analysis of blood gases.Time to extubation and length of stay were also recorded.Results Intravenous induction with muscle paralysis followed by pressure-control ventilation and tracheal intubation regardless of the position of the fistula can be performed uneventfully in EA/TEF newborns with no additional airway anomalies and large,pericarinal fistulas in our experiences.The thoracoscopic approach appeared to take longer than the open approach.During the procedure of repair,hypercarbia and acidosis developed immediately 1 hour after pneumothorax in both groups.CO2 insufflation did have additional influence on the respiratory function of the newborns in the TR group; values of PaCO2 and ETCO2 were higher in the TR group but the difference did not reach statistical significance.By the end of the procedure,values of PaCO2 and ETCO2 returned to the baseline levels while pH did not,but all parameters made no difference in the two groups.Besides,time to extubation was shorter in the TR group.Conclusions Thoracoscopic repair of EA/TEF is comparable to the open repair

  10. An assessment of quality of life of operated cases of esophageal atresia in the community

    Directory of Open Access Journals (Sweden)

    Harshjeet Singh Bal

    2016-01-01

    Full Text Available Aims: To evaluate the outcome of the operated children of esophageal atresia (EA focusing on their early and late morbidity and mortality and quality of life (QoL of survivors. Settings and Design: A cross-sectional follow-up with retrospective analysis of available medical and surgical records of children who underwent repair for EA. Materials and Methods: The medical records of the children who underwent repair for EA during the period from 2000 to 2011 at the Christian Medical College Hospital, Vellore, were collected retrospectively. Patients with parents were invited to visit the hospital for follow-up and nutritional status, digestive and respiratory symptoms, status of associated anomalies and QoL assessment of children done. QoL assessment was done using the PedsQL™ 4.0 generic core scales questionnaire comprising 4 scale scores: physical, emotional, social functioning, and school functioning. Mean scores are calculated based on a 5-point response scale for each item and transformed to a 0-100 scale with a higher score representing better QoL. Statistical Analysis Used: Statistical Package for Social Sciences (SPSS version 16 using Chi-square or Fisher′s exact test. Results: Of 79 patients operated during the said period, there were 10 deaths and a total of 69 (87% children survived. Of the 66 patients available for follow-up, we interviewed 30 parents and children while for the remaining 36 children, out-patients charts were reviewed retrospectively. Mean follow-up duration was 3.56 years. The height and weight for age measurement showed 47% and 56% of children respectively as below the 5 th percentile. Main problems faced by operated EA children were of the respiratory (26% and gastroesophageal (36% tracts. In spite of the mentioned problems faced, the overall QoL of this group appeared good. In 23 of 30 patients, who answered PedsQL™ , more than 70% had scores >85 out of 100 in QoL scoring. Conclusions: While survivals of the

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

    Science.gov (United States)

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

    Shen, Wen-jun; Dong, Rui; Chen, Gong, E-mail: chengongzlp@hotmail.com; 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.

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

    Directory of Open Access Journals (Sweden)

    Rahul Gupta

    2016-01-01

    Full Text Available 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.

  14. Successful pregnancy by IVF in a patient with congenital cervical atresia

    Institute of Scientific and Technical Information of China (English)

    Achour Radhouane; Basly Mohamed; Ben Aissa Imen; Ferjaoui Aymen; NEJI Khaled

    2015-01-01

    Congenital cervical atresia and hypoplasia are rare abnormalities that generally require reconstructive or extirpative procedures to relieve outflow tract obstruction. Infertility is a common sequel, and only four previous pregnancies have been reported. We report a case of successful pregnancy afterin-vitro fertilization in a 32-year-old patient with congenital cervical atresia diagnosed at the age of 28 years. She was referred to our unit and had a succeful pregnancy afterin-vitro fertilization. Caesarean section was perfomed at 38 weeks gestation. A healthy male baby weighing 3 650 g was safely delivered.

  15. Congenital bronchial atresia coexistent with intralobar pulmonary sequestration: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Han, Young Min; Ku, Ja Hong; Lee, Dong Keun; Chung, Kyung Ho; Kim, Chong Soo; Sohn, Myung Hee; Choi, Ki Chul [Chonbuk National University Medical School, Jeonju (Korea, Republic of)

    1995-02-15

    Bronchial atresia coexistent with intralobar pulmonary sequestration is so rare that only two cases have been reported in the literature. We report a case of congential bronchial atresia coexistent with intralobar pulmonary sequestation in a 51-year-old woman. Computed tomography showed the branching mass with hyperinflation of adjacent pulmonary parenchyma in the medial segment of the right middle lobe and a large thin-walled cystic mass with air-fluid levels in the medial basal segment of the right lower lobe. Selective inferior phrenic arteriography showed two aberrant arteries supplying the large cystic mass in the right lower lobe. The venous drainage was through the right pulmonary vein.

  16. BICORNUATE UTERUS WITH CERVICAL ATRESIA AND VAGINAL AGENESIS ASSOCIATED WITH OVARIAN ENDOMETRIOSIS - A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Alpana

    2013-06-01

    Full Text Available ABSTRACT: Vaginal agenesis combined with a functional uterus is a rare type of Mullerian duct an omaly. Only 7 – 8% of patients with vaginal agenesis have a functional uterus. Women born with vaginal agenesis, cervical atresia combined with a functioning endometrium typically present with hematometra , disabling pelvic pain and progressively worsening en dometriosis. Almost all need an abdominal hysterectomy for relief. We report a case of severe endometriosis in a 29 years old woman having a bicornuate uterus with cervical atresia and vaginal agenesis.

  17. Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia

    OpenAIRE

    Saeed, Muhammad; Anwar ul HAQ; Khaqan QADIR

    2014-01-01

    How to Cite This Article: Saeed M, Haq A, Qadir Kh.Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia. Iran J Child Neurol. 2014 Autumn;8(4): 76-79.AbstractObjectiveBart’s syndrome is defined as congenital localized absence of skin, and associated with epidermolysis bullosa. A newborn with Bart’s syndrome is reported because it is a very rare condition, especially when associated with corpus callosum agenesis and concomitant choanal atresia. Clinically it is characterized...

  18. Scintigraphic hepatobiliary function studies in newborn infants to diagnose biliary hypoplasia or atresia

    International Nuclear Information System (INIS)

    The results obtained from scintigraphic hepatobiliary function studies, intraoperative cholangiography and histological examinations in a total of 17 infants suspected of having biliary atresia were compared and analysed with reference to the clinical signs and symptoms observed. In most cases, the individual diagnostic procedures led to consistent findings, even though there were some variations in the clinical picture. Patient outcome is largely determined by the site of atresia, due to which fact surgical correction should be carried out as soon as possible, in any case before the 8th week post partum. (TRV)

  19. [Nasal breath recovery and rhinoplasty in cleft lip and palate patient with unilateral choanal atresia].

    Science.gov (United States)

    Chkadua, T Z; Ivanova, M D; Daminov, R O; Brusova, L A; Savvateeva, D M

    2016-01-01

    The paper presents the analysis of clinical case of endoscopic nasal breath restoration and elimination of the secondary cleft lip nasal deformity in 27 years old patient with unilateral choanal atresia and secondary nasal deformity after rhinocheiloplasty. Preoperative examination revealed the absence of nasal breathing on collateral side due to complete bone choanal atresia. Surgical treatment included endoscopic choanal repair, elimination of the secondary nasal deformity, septoplasty, conchotomy and lateroposition of the inferior conchae. The treatment resulted in nasal breath restoration and elimination of nasal deformity. Long-term follow-up at 1 and 12 months post-operatively proved stable positive aesthetic and functional results. PMID:26925564

  20. Anal acoustic reflectometry: a new technique for assessing anal sphincter function

    DEFF Research Database (Denmark)

    Mitchell, P J; Klarskov, N; Hosker, G;

    2010-01-01

    Anal acoustic reflectometry (AAR) is a new technique that offers an assessment of anal sphincter function by the measurement of additional parameters not available with conventional manometry. The aim of this study is to describe the technique, methodology and initial pilot study results....

  1. Anomalies on orbifolds

    Energy Technology Data Exchange (ETDEWEB)

    Arkani-Hamed, Nima; Cohen, Andrew G.; Georgi, Howard

    2001-03-16

    We discuss the form of the chiral anomaly on an S1/Z2 orbifold with chiral boundary conditions. We find that the 4-divergence of the higher-dimensional current evaluated at a given point in the extra dimension is proportional to the probability of finding the chiral zero mode there. Nevertheless the anomaly, appropriately defined as the five dimensional divergence of the current, lives entirely on the orbifold fixed planes and is independent of the shape of the zero mode. Therefore long distance four dimensional anomaly cancellation ensures the consistency of the higher dimensional orbifold theory.

  2. Molecular dysregulation of renal development:Congenital anomalies of the kidney and urinary tract

    Institute of Scientific and Technical Information of China (English)

    Mark Daniel Wilson

    2015-01-01

    Congenital anomalies of the kidney and urinary tract (CAKUT) occur in approximately 1 in 500 foetal ultrasound examinations. The CAKUT phenotype can involve varying degrees of renal dysplasia, renal hypoplasia, urinary tract obstruction, ureteropelvic anomalies such as megaureter, ureteral atresia, ectopic ureteral orifice, and duplex collecting system The nephrogenic (mesenchymal) and the ductogenic (ureteric) events are regulated by transcription factors, proto-oncogenes and growth factors in a complex fashion. Dysregulation of specific molecular pathways has been implicated as a primary mechanism for CAKUT. This review will attempt to clarify the molecular basis of CAKUT by focusing on these key developmental pathways. First, however, an examination of normal metanephric kidney development is necessary. Furthermore, clinical aspects of CAKUT, including prenatal diagnosis and current treatments, will be introduced. Through the critical evaluation of a range of diverse scientific literature, it is hoped that an overview of the current status of this important area of developmental anatomy is achieved.

  3. Molecular dysregulation of renal development: Congenital anomalies of the kidney and urinary tract

    Directory of Open Access Journals (Sweden)

    Mark Daniel Wilson

    2015-03-01

    Full Text Available Congenital anomalies of the kidney and urinary tract (CAKUT occur in approximately 1 in 500 foetal ultrasound examinations. The CAKUT phenotype can involve varying degrees of renal dysplasia, renal hypoplasia, urinary tract obstruction, ureteropelvic anomalies such as megaureter, ureteral atresia, ectopic ureteral orifice, and duplex collecting system The nephrogenic (mesenchymal and the ductogenic (ureteric events are regulated by transcription factors, proto-oncogenes and growth factors in a complex fashion. Dysregulation of specific molecular pathways has been implicated as a primary mechanism for CAKUT. This review will attempt to clarify the molecular basis of CAKUT by focusing on these key developmental pathways. First, however, an examination of normal metanephric kidney development is necessary. Furthermore, clinical aspects of CAKUT, including prenatal diagnosis and current treatments, will be introduced. Through the critical evaluation of a range of diverse scientific literature, it is hoped that an overview of the current status of this important area of developmental anatomy is achieved.

  4. Theoretically Optimal Distributed Anomaly Detection

    Data.gov (United States)

    National Aeronautics and Space Administration — A novel general framework for distributed anomaly detection with theoretical performance guarantees is proposed. Our algorithmic approach combines existing anomaly...

  5. Endotoxin and CD14 in the progression of biliary atresia

    Directory of Open Access Journals (Sweden)

    Chen Ching-Mei

    2010-12-01

    Full Text Available Abstract Background Biliary atresia (BA is a typical cholestatic neonatal disease, characterized by obliteration of intra- and/or extra-hepatic bile ducts. However, the mechanisms contributing to the pathogenesis of BA remain uncertain. Because of decreased bile flow, infectious complications and damaging endotoxemia occur frequently in patients with BA. The aim of this study was to investigate endotoxin levels in patients with BA and the relation of these levels with the expression of the endotoxin receptor, CD14. Methods The plasma levels of endotoxin and soluble CD14 were measured with a pyrochrome Limulus amebocyte lysate assay and enzyme-linked immunosorbent assay in patients with early-stage BA when they received the Kasai procedure (KP, in patients who were jaundice-free post-KP and followed-up at the outpatient department, in patients with late-stage BA when they received liver transplantation, and in patients with choledochal cysts. The correlation of CD14 expression with endotoxin levels in rats following common bile duct ligation was investigated. Results The results demonstrated a significantly higher hepatic CD14 mRNA and soluble CD14 plasma levels in patients with early-stage BA relative to those with late-stage BA. However, plasma endotoxin levels were significantly higher in both the early and late stages of BA relative to controls. In rat model, the results demonstrated that both endotoxin and CD14 levels were significantly increased in liver tissues of rats following bile duct ligation. Conclusions The significant increase in plasma endotoxin and soluble CD14 levels during BA implies a possible involvement of endotoxin stimulated CD14 production by hepatocytes in the early stage of BA for removal of endotoxin; whereas, endotoxin signaling likely induced liver injury and impaired soluble CD14 synthesis in the late stages of BA.

  6. Outcomes in children with biliary atresia following liver transplantation

    Institute of Scientific and Technical Information of China (English)

    Li-Ying Sun; Chong Dong; Jin-Peng Tu; Jian Wang; Yi-He Liu; Yuan Liu; Li-Xin Yu; Yu Wang; Jing Li and Zhong-Yang Shen; Yun-Sheng Yang; Zhi-Jun Zhu; Wei Gao; Lin Wei; Xiao-Ye Sun; Wei Qu; Wei Rao; Zhi-Gui Zeng

    2013-01-01

    BACKGROUND: Congenital biliary atresia is a rare condition characterized  by  idiopathic  dysgenesis  of  the  bile  ducts.  If untreated,  congenital  biliary  atresia  leads  to  liver  cirrhosis, liver failure and premature death. The present study aimed to evaluate  the  outcomes  of  orthotopic  liver  transplantation  in children with biliary atresia. METHOD: We retrospectively analyzed 45 patients with biliary atresia  who  had  undergone  orthotopic  liver  transplantation from September 2006 to August 2012. RESULTS: The median age of the patients was 11.0 months (5-102). Of the 45 patients, 41 were younger than 3 years old. Their median weight was 9.0 kg (4.5-29.0), 34 of the 45 patients were less than 10 kg. Thirty-one patients had undergone Kasai portoenterostomy prior to orthotopic liver transplantation. We performed 30 living donor liver transplants and 15 split liver transplants. Six patients died during a follow-up. The median follow-up time of surviving patients was 11.4 months (1.4-73.7). The overall 1-, 2- and 3-year survival rates were 88.9%, 84.4% and 84.4%, respectively. CONCLUSION: With  advances  in  surgical  techniques and  management,  children  with  biliary  atresia  after  liver transplantation  can  achieve  satisfactory  survival  in  China, although there remains a high risk of complications in the early postoperative period.

  7. Learning about Poland Anomaly

    Science.gov (United States)

    ... performed too early, while the individual is growing, asymmetry can result or be made greater than before. ... Anomaly About.com- Poland Syndrome [rarediseases.about.com] Information about Poland syndrome produced by Mary Kugler, M.S. ...

  8. Scattering anomaly in optics

    CERN Document Server

    Silveirinha, Mario G

    2016-01-01

    In time-reversal invariant electronic systems the scattering matrix is anti-symmetric. This property enables an effect, designated here as "scattering anomaly", such that the electron transport does not suffer from back reflections, independent of the specific geometry of the propagation path or the presence of time-reversal invariant defects. In contrast, for a generic time-reversal invariant photonic system the scattering matrix is symmetric and there is no similar anomaly. Here, it is theoretically proven that despite these fundamental differences there is a wide class of photonic platforms - in some cases formed only by time-reversal invariant media - in which the scattering anomaly can occur. It is shown that an optical system invariant under the action of the composition of the time-reversal, parity and duality operators is characterized by an anti-symmetric scattering matrix. Specific examples of photonic platforms wherein the scattering anomaly occurs are given, and it is demonstrated with full wave n...

  9. Anomaly Detection in Sequences

    Data.gov (United States)

    National Aeronautics and Space Administration — We present a set of novel algorithms which we call sequenceMiner, that detect and characterize anomalies in large sets of high-dimensional symbol sequences that...

  10. Neutrino anomalies without oscillations

    Indian Academy of Sciences (India)

    Sandip Pakvasa

    2000-01-01

    I review explanations for the three neutrino anomalies (solar, atmospheric and LSND) which go beyond the `conventional' neutrino oscillations induced by mass-mixing. Several of these require non-zero neutrino masses as well.

  11. Skyrmions and anomalies

    International Nuclear Information System (INIS)

    The author summarizes the works presented at the meeting on skyrmions and anomalies. He divides the principal issues of this workshop into five categories: QCD effective lagrangians, chiral bags and the Cheshire cat principle, strangeness problem, phenomenology, mathematical structure

  12. Evidence for the "midline" hypothesis in associated defects of laterality formation and multiple midline anomalies.

    Science.gov (United States)

    Gilbert-Barness, E; Debich-Spicer, D; Cohen, M M; Opitz, J M

    2001-07-15

    A male infant was liveborn at 38 weeks of gestation to a G4P1AB2, 22-year-old, mother. Polyhydramnios and multiple congenital anomalies were noted by ultrasonography; the infant died 5 min after birth. At autopsy, the infant had multiple defects of blastogenesis including midline anomalies with asplenia and abnormalities of laterality formation. The laterality defects were unusual in that they combined asplenia with hypoplastic, symmetrically unilobate lungs and bilateral hyparterial bronchi more consistent with polysplenia, abdominal situs inversus with midline stomach, symmetric liver, and left gallbladder. No intracardiac abnormalities were present, but there was azygous continuation of the inferior vena cava. Additional multiple midline defects included bronchoesophageal fistula, duodenal atresia, absence of posterior leaf of diaphragm; horseshoe adrenal gland; microcephaly; Dandy-Walker anomaly with agenesis of cerebellar vermis and occipital encephalocele; holoprosencephaly with orbital encephalocele, midline defect of the orbital plate of the skull, bilateral anophthalmia, double proboscis with bilateral choanal atresia, midline upper lip and palatal cleft; single-lobed thyroid; hypoplastic external genitalia with midline cleft of scrotum, long tapering fingers, and defects of the cranium at the sites of orbital and occipital encephaloceles. Defects of laterality frequently are associated with other complex midline anomalies, which both result from a disturbance of pattern formation during blastogenesis, i.e., the induction of the progenitor fields. The latter are the result of the establishment of upstream expression domains of growth and transcription factors and other morphogens. Many of these and other genetic systems, expressed asymmetrically around the midline, are responsible for laterality formation and are the result of upstream and subsequent downstream gene expression cascades through the expression of genes such as HOX genes; bFGF; transforming

  13. Congenital laryngeal anomalies,

    OpenAIRE

    Rutter, Michael J.

    2014-01-01

    Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the ma...

  14. The Pioneer Anomaly

    CERN Document Server

    de Diego, Jose A

    2008-01-01

    Analysis of the radio-metric data from Pioneer 10 and 11 spacecrafts has indicated the presence of an unmodeled acceleration starting at 20 AU, which has become known as the Pioneer anomaly. The nature of this acceleration is uncertain. In this paper we give a description of the effect and review some relevant mechanisms proposed to explain the observed anomaly. We also discuss on some future projects to investigate this phenomenon.

  15. Volume anomaly in ferrimagnetism

    OpenAIRE

    Pascard, H.; Globus, A.

    1981-01-01

    The volume anomaly ΔV/V due to the magnetic energy corresponding to the exchange interactions is experimentally determined for YIG. The experimental values (from 77 K to Tc) agree with the values deduced from the theoretical expression based on the Néel's theories of volume anomaly and of ferrimagnetism. These results are compared with those obtained by other authors on ferromagnetic and antiferromagnetic materials with localized magnetic moments : a reduced curve is obtained.

  16. Anomalies and Entanglement Entropy

    CERN Document Server

    Nishioka, Tatsuma

    2015-01-01

    We initiate a systematic study of entanglement and Renyi entropies in the presence of gauge and gravitational anomalies in even-dimensional quantum field theories. We argue that the mixed and gravitational anomalies are sensitive to boosts and obtain a closed form expression for their behavior under such transformations. Explicit constructions exhibiting the dependence of entanglement entropy on boosts is provided for theories on spacetimes with non-trivial magnetic fluxes and (or) non-vanishing Pontryagin classes.

  17. Dual diaphragmatic anomalies.

    Science.gov (United States)

    Padmanabhan, Arjun; Thomas, Abin Varghese

    2016-01-01

    Although diaphragmatic anomalies such as an eventration and hiatus hernia are commonly encountered in incidental chest X-ray imaging, the presence of concomitant multiple anomalies is extremely rare. This is all the more true in adults. Herein, we present the case of a 75-year-old female, while undergoing a routine chest X-ray imaging, was found to have eventration of right hemidiaphragm along with a hiatus hernia as well. PMID:27625457

  18. Dual diaphragmatic anomalies

    Directory of Open Access Journals (Sweden)

    Arjun Padmanabhan

    2016-01-01

    Full Text Available Although diaphragmatic anomalies such as an eventration and hiatus hernia are commonly encountered in incidental chest X-ray imaging, the presence of concomitant multiple anomalies is extremely rare. This is all the more true in adults. Herein, we present the case of a 75-year-old female, while undergoing a routine chest X-ray imaging, was found to have eventration of right hemidiaphragm along with a hiatus hernia as well.

  19. SADM potentiometer anomaly investigations

    Science.gov (United States)

    Wood, Brian; Mussett, David; Cattaldo, Olivier; Rohr, Thomas

    2005-07-01

    During the last 3 years Contraves Space have been developing a Low Power (1-2kW) Solar Array Drive Mechanism (SADM) aimed at small series production. The mechanism was subjected to two test programmes in order to qualify the SADM to acceptable levels. During the two test programmes, anomalies were experienced with the Potentiometers provided by Eurofarad SA and joint investigations were undertaken to resolve why these anomalies had occurred. This paper deals with the lessons learnt from the failure investigation on the two Eurofarad (rotary) Potentiometer anomaly. The Rotary Potentiometers that were used were fully redundant; using two back to back mounted "plastic tracks". It is a pancake configuration mounted directly to the shaft of the Slip Ring Assembly at the extreme in-board end of the SADM. It has no internal bearings. The anomaly initially manifested itself as a loss of performance in terms of linearity, which was first detected during Thermal Vacuum testing. A subsequent anomaly manifested itself by the complete failure of the redundant potentiometer again during thermal vacuum testing. This paper will follow and detail the chain of events following this anomaly and identifies corrective measures to be applied to the potentiometer design and assembly process.

  20. The Holographic Supercurrent Anomaly

    CERN Document Server

    Chaichian, Masud

    2004-01-01

    The \\gamma-trace anomaly of supersymmetry current in a supersymmetric gauge theory shares a superconformal anomaly multiplet with the chiral R-symmetry anomaly and the Weyl anomaly, and its holographic reproduction is a valuable test to the AdS/CFT correspondence conjecture. We investigate how the \\gamma-trace anomaly of the supersymmetry current of {\\cal N}=1 four-dimensional supersymmetric gauge theory in an {\\cal N}=1 conformal supergravity background can be extracted out from the ${\\cal N}=2$ gauged supergravity in five dimensions. It is shown that the reproduction of this super-Weyl anomaly originates from the following two facts: First the {\\cal N}=2 bulk supersymmetry transformation converts into {\\cal N}=1 superconformal transformation on the boundary, which consists of {\\cal N}=1 supersymmetry transformation and special conformal supersymmetry (or super-Weyl) transformation; second the supersymmetry variation of the bulk action of five-dimensional gauged supergravity is a total derivative. The non-co...

  1. Proctology - diseases of the anal region.

    Science.gov (United States)

    Kreuter, Alexander

    2016-04-01

    Proctology is a medical subspecialty that encompasses diseases of the perianal region, anal canal, and rectum. Dermatologists play a pivotal role in this realm, as inflammatory perianal disorders, infectious and sexually transmitted diseases, as well as perianal tumors and their precursor lesions fall within the core competency of dermatology. In a concise manner, the present article highlights all relevant disease groups in the field of proctology. With a particular focus on aspects pertinent to dermatologists, this includes inflammatory disorders, "classic" proctologic diseases, sexually transmitted diseases, malignancies of the anal region, as well as pathogen-induced diseases. Despite the wide variety of disorders, there are only five key symptoms prompting patients to consult a proctologist, including anal pruritus and burning, discharge, bleeding, pain, and foreign body sensation. A simple algorithm, which incorporates these symptoms as well as key clinical features, may assist in quickly establishing the correct diagnosis in everyday clinical practice. PMID:27027745

  2. Bilateral congenital lacrimal fistulas in an adult as part of ectrodactyly-ectodermal dysplasia-clefting syndrome: A rare anomaly.

    Science.gov (United States)

    Ghosh, Debangshu; Saha, Somnath; Basu, Sumit Kumar

    2015-10-01

    Ectrodactyly-ectodermal dysplasia and clefting syndrome or "Lobster claw" deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD) obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mild mental retardation, otitis media, and sinusitis. Lacrimal duct obstruction and fistula were managed successfully with endoscopic dacryocystorhinostomy (DCR) which is a good alternative to lacrimal probing or open DCR in such a case. PMID:26655010

  3. Bilateral congenital lacrimal fistulas in an adult as part of ectrodactyly-ectodermal dysplasia-clefting syndrome: A rare anomaly

    Directory of Open Access Journals (Sweden)

    Debangshu Ghosh

    2015-01-01

    Full Text Available Ectrodactyly-ectodermal dysplasia and clefting syndrome or "Lobster claw" deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mild mental retardation, otitis media, and sinusitis. Lacrimal duct obstruction and fistula were managed successfully with endoscopic dacryocystorhinostomy (DCR which is a good alternative to lacrimal probing or open DCR in such a case.

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

    Science.gov (United States)

    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

  5. Laparoscopic and thoracoscopic gastric pull-up for pure esophageal atresia in early infancy

    Directory of Open Access Journals (Sweden)

    D K Kandpal

    2013-01-01

    Full Text Available In the developing countries, the babies with pure esophageal atresia undergo an esophagostomy and feeding gastrostomy at birth. It assists in early discharge from hospital. Esophageal substitution in these babies around six months is recommended. We report the first laparoscopic and thoracoscopic gastric pull up in early infancy from India.

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

    Directory of Open Access Journals (Sweden)

    Nandini K Bedi

    2016-01-01

    Full Text Available 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.

  7. Predictors of mortality in newborns with esophageal atresia: a 6-year study in a single institution

    Directory of Open Access Journals (Sweden)

    Leecarlo Milano

    2016-02-01

    Full Text Available Background Esophageal atresia, with or without fistula, is a congenital defect that causes high morbidity and mortality in newborns. Risk factors of mortality need to be identified to establish the best approach for treating this condition in order to decrease morbidity and mortality.Objective To identify factors associated with mortality in newborns with oesophageal atresia.Methods We reviewed all newborns with esophageal atresia using data from their medical records at Sardjito General Hospital from January 2007 to December 2012. Potential risk factors were analyzed using Chi-square test, with a level of significance of P<0.05.Results Of 31 newborns that met our criteria, only 5 survived, and all 5 had one-stage surgery (primary anastomosis with fistula ligation. Thrombocytopenia and sepsis increased the risk of death with OR 10.857 (95%CI 1.029 to 114.578 and OR 13.333 (95% CI 1.242 to 143.151, respectively. However, anemia had a protective effect against mortality with OR 0.688 (95%CI 0.494 to 0.957.Conclusion Thrombocytopenia and sepsis are the risk factors associated with mortality in newborns with esophageal atresia at our institution. Anemia has a protective effect against mortality.

  8. Esophageal atresia and tracheoesophageal fistula in children of women exposed to diethylstilbestrol in utero

    NARCIS (Netherlands)

    Felix, Janine F.; Steegers-Theunissen, Regine P. M.; de Walle, Hermien E. K.; de Klein, Annelies; Torfs, Claudine P.; Tibboel, Dick

    2007-01-01

    OBJECTIVE: This study was undertaken to study the possible risk to mothers exposed in utero to diethylstilbestrol for offspring with esophageal atresia/ tracheoesophageal fistula. STUDY DESIGN: Information on the mothers ' in utero exposure to diethylstilbestrol was obtained from 3 sources: question

  9. Anesthetic management for surgery of esophagus atresia in a newborn with Goldenhar's syndrome

    Directory of Open Access Journals (Sweden)

    Rosana Guerrero-Domínguez

    2015-08-01

    Full Text Available BACKGROUND AND OBJECTIVES: Goldenhar's syndrome is a polymalformative condition consisting of a craniofacial dysostosis that determines difficult airway in up to 40% of cases. We described a case of a newborn with Goldenhar's syndrome with esophageal atresia and tracheoesophageal fistula who underwent repair surgery.CASE REPORT: We report the case of a 24-h-old newborn with Goldenhar's syndrome. He had esophageal atresia with distal tracheoesophageal fistula. It was decided that an emergency surgery would be performed for repairing it. It was carried out under sedation, intubation with fibrobronchoscope distal to the fistula, to limit the air flow into the esophagus, and possible abdominal distension. Following complete repair of the esophageal atresia and fistula ligation, the patient was transferred to the intensive care unit and intubated under sedation and analgesia.CONCLUSIONS: The finding of a patient with Goldenhar's syndrome and esophageal atresia assumes an exceptional situation and a challenge for anesthesiologists, since the anesthetic management depends on the patient comorbidity, the type of tracheoesophageal fistula, the usual hospital practice and the skills of the anesthesiologist in charge, with the main peculiarity being maintenance of adequate pulmonary ventilation in the presence of a communication between the airway and the esophagus. Intubation with fibrobronchoscope distal to the fistula deals with the management of a probably difficult airway and limits the passage of air to the esophagus through the fistula.

  10. Heteropic gastric pancreas associated with type II esophageal atresia with acute gastrointestinal bleeding

    Directory of Open Access Journals (Sweden)

    F. Destro

    2014-06-01

    Full Text Available Heterotopic pancreas is defined as pancreatic tissue found outside its normal localization without vascular or anatomic communication with the pancreatic gland. It is usually diagnosed incidentally, but it may also be responsible for acute clinical pictures. We discuss an interesting case of ectopic pancreas associated with type II esophageal atresia and presenting with gastrointestinal acute bleeding.

  11. Predictors of mortality in newborns with esophageal atresia: a 6-year study in a single institution

    Directory of Open Access Journals (Sweden)

    Leecarlo Millano

    2015-05-01

    Full Text Available Background Esophageal atresia, with or without fistula, is a congenital defect that causes high morbidity and mortality in newborns. Risk factors of mortality need to be identified to establish the best approach for treating this condition in order to decrease morbidity and mortality. Objective To identify factors associated with mortality in newborns with oesophageal atresia. Methods We reviewed all newborns with esophageal atresia using data from their medical records at Sardjito General Hospital from January 2007 to December 2012. Potential risk factors were analyzed using Chi-square test, with a level of significance of P<0.05. Results Of 31 newborns that met our criteria, only 5 survived, and all 5 had one-stage surgery (primary anastomosis with fistula ligation. Thrombocytopenia and sepsis increased the risk of death with OR 10.857 (95%CI 1.029 to 114.578 and OR 13.333 (95% CI 1.242 to 143.151, respectively. However, anemia had a protective effect against mortality with OR 0.688 (95%CI 0.494 to 0.957. Conclusion Thrombocytopenia and sepsis are the risk factors associated with mortality in newborns with esophageal atresia at our institution. Anemia has a protective effect against mortality. [Paediatr Indones. 2015;55:131-5.].

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

    Science.gov (United States)

    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

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

    OpenAIRE

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

    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.

  14. Right ventricular collagen and fibronectin levels in patients with pulmonary atresia and ventricular septal defect

    NARCIS (Netherlands)

    Peters, Theodorus H F; de Jong, Peter L; Klompe, Lennart; Berger, Rolf M F; Saxena, Pramod R; Sharma, Hari S; Bogers, Ad J J C; Berger, Rudolphus

    2003-01-01

    Pulmonary atresia (PA) with ventricular septal defect (VSD) is an extreme form of tetralogy of Fallot with characteristic right ventricular hypertrophy. To reduce the right ventricular overload, these children have to undergo staged corrective surgery to restore physiological pulmonary perfusion. We

  15. Prevention of the murine model of biliary atresia following live rotavirus vaccination of dams

    Science.gov (United States)

    Bondoc, Alexander J; Jafri, Mubeen A; Donnelly, Bryan; Mohanty, Sujit K; McNeal, Monica M; Ward, Richard L; Tiao, Greg M

    2009-01-01

    Purpose Biliary atresia (BA) is a neonatal disease that results in the obliteration of the biliary tree. The murine model of biliary atresia (BA) has been established where rhesus rotavirus (RRV) infection of newborn mice leads to an obstructive cholangiopathy. We determined whether maternal, post-conception rotavirus vaccination could prevent the murine model of biliary atresia. Materials and Methods Female mice were mated and injected intraperitoneally with one of the following materials: purified rotavirus strains RRV or Wa, high or low dose Rotateq® (a pentavalent rotavirus vaccine (PRV)), purified recombinant viral antigens of rotavirus (VP6) or influenza (NP), or saline. B-cell-deficient females also underwent post-conception PRV injection. Maternal vaccination with PRV improves survival of pups infected with RRV. Results Maternal vaccination with PRV improves survival of pups infected with RRV. Serum rotavirus IgG, but not IgA, levels were increased in pups delivered from dams who received RRV, Wa, PRV, or VP6, but in the case of the Wa, PRV, and VP6 groups, these antibodies were not neutralizing. Post-conception injection of high dose PRV did not improve survival of pups born to B-cell deficient dams. Conclusion Maternal vaccination against RRV can prevent the rotavirus-induced murine model of biliary atresia in newborn mouse pups. PMID:19635292

  16. Twenty-year transplant-free survival rate among patients with biliary atresia.

    NARCIS (Netherlands)

    Vries, W. de; Homan-van der Veen, J.; Hulscher, J.B.F.; Hoekstra-Weebers, J.E.H.M.; Houwen, R.H.J.; Verkade, H.J.; Aronson, C.; Escher, J.H.; Heurn, L.W.E. van; Heij, H.A.; Kindermann, A.; Kneepkens, C.M.; Langen, Z.J. de; Madern, G.C.; Neucke, M. van den; Peeters, P.M.; Rieu, P.N.M.A.; Tolboom, J.J.M.; Zee, D.C. van der

    2011-01-01

    BACKGROUND & AIMS: Surgical treatment with Kasai portoenterostomy has improved the prognosis for patients with biliary atresia, although most patients ultimately require liver transplantation. Well-described patients with long-term, transplant-free survival are scarce; we assessed liver status and h

  17. Twenty-Year Transplant-Free Survival Rate Among Patients With Biliary Atresia

    NARCIS (Netherlands)

    de Vries, Willemien; Homan-Van der Veen, Jenneke; Hulscher, Jan B. F.; Hoekstra-Weebers, Josette E. H. M.; Houwen, Roderick H. J.; Verkade, Henkjan J.

    2011-01-01

    BACKGROUND & AIMS: Surgical treatment with Kasai portoenterostomy has improved the prognosis for patients with biliary atresia, although most patients ultimately require liver transplantation. Well-described patients with long-term, transplant-free survival are scarce; we assessed liver status and h

  18. The role of genetic and environmental factors in the etiology of esophageal atresia and tracheo-esophageal fistula

    Directory of Open Access Journals (Sweden)

    Damian Bednarczyk

    2014-03-01

    Full Text Available Esophageal atresia and tracheo-esophageal fistula are severe congenital malformations, whose etiology is still poorly understood. So far, numerous genetic and environmental factors that may contribute to the occurrence of these defects have been described and the literature is dominated by the view of their common involvement in the etiology and pathogenesis of congenital esophageal atresia. In this review the authors present current knowledge on the embryogenesis of the esophagus and trachea, discuss environmental risk factors, and also list and describe genetic alterations identified so far in patients with congenital esophageal atresia.

  19. Modified end-to-end anastomosis for the treatment of congenital tracheal stenosis with a bridging bronchus.

    Science.gov (United States)

    Stock, Cameron; Nathan, Meena; Murray, Ryan; Rahbar, Reza; Fynn-Thompson, Francis

    2015-01-01

    An infant with a ventricular septal defect; Vertebral anomalies, Anal atresia, Cardiac anomalies, Tracho Esophageal fistula (TEF), Renal anomalies, Limb anomalies syndrome; and tracheal stenosis with a bridging bronchus underwent repair of the ventricular septal defect and trachea-bronchial reconstruction at age 11 months. Herein we describe our surgical approach to resection of the bridging bronchus and a technique using a modified end-to-end tracheal anastomosis for the correction of this complex anomaly. PMID:25555968

  20. Seoseid loov kunstiteose analüüs / Anneli Porri

    Index Scriptorium Estoniae

    Porri, Anneli, 1980-

    2013-01-01

    Kunstiteose analüüsi seosest riikliku õppekavaga, ülevaade kunstiteose analüüsimeetoditest kunstiteaduses ning metoodilistest tähelepanekutest kunsti ja visuaalkultuuri kujutiste vaatamise kohta gümnaasiumi kunstitunnis

  1. PRIMENA SWOT ANALIZE NA SISTEM INTEGRALNOG TRANSPORTA VOJSKE SRBIJE

    OpenAIRE

    Dragan Pamučar

    2008-01-01

    U radu je prikazana primena SWOT analize na sistem integralnog transporta Vojske Srbije. Kao rezultat analize predstavljeni su ciljevi daljeg usavršavanja integralnog transporta, kao i mogući problemi generisani strateškim upravljanjem.

  2. Esophageal atresia: long-term interdisciplinary follow-up

    Directory of Open Access Journals (Sweden)

    Lidia B. Giúdici

    2016-07-01

    Full Text Available Background: We provide protocolized interdisciplinary follow-up to babies born with Esophageal Atresia (EA. There are few reports in Argentina about follow-up of EA patients.Objective: To describe outcomes in follow-up of EA patients at 1, 3 and 6 years old and to compare outcomes at age 1 with those at age 6.Methods: Prospective, longitudinal, analytic study of the cohort of babies born with EA, admitted to the follow-up program from 11/01/03 to 10/31/14. Follow-up includes: growth (weight > 10th centile, WHO, neurology-psychomotor development, audiology, vision, genetic, mental health, surgical reintervention, phonostomatology, language, pulmonology, re-hospitalization for clinical causes, lost to follow-up. Outcomes were described at age 1, 3 and 6. We included all EA patients who had reached age 1 at the start of this study.Results: 27 babies were admitted; 30% had long-gap EA; 18% presented VACTERL association; 23 children met inclusion criteria. Genetics  was assessed in 18 newborns (78%; a chromosomal map was performed in 11 babies; 3 had an abnormal karyotype. Mental health: 5/14 of the assessed children showed problems. Phonostomatology: 11 newborns checked (6 required treatment, 4 recovered at age 1. Pulmonologist evaluated 18 babies (7 with recurrent wheezing, 6 with moderate tracheomalacia. Gastroenterology and endoscopy: 80% presented gastroesophageal reflux (GER grade 3-4, and 50% showed a pathologic pHmetry. Lost to follow-up: age 1, 2 (8%; age 3, 3 (17%; age 6, 3 (23%. Normal outcomes observed are the following. Age 1 – growth: 81%; neurologic-psychomotor developmental index (NPDI: 76%; audiology: 95%; vision: 85%; language: 62%; re-hospitalization for clinical causes: 38%; surgical reinterventions: 47%. Age 3 – growth: 78%; NPDI: 50%; audiology: 93%; vision: 93%; language: 43%; re-hospitalization: 35%; surgical reinterventions: 14%. Age 6 – growth: 50%; NPDI: 30%; audiology: 90%; vision: 40%; language: 50%; re

  3. Tracheoesophageal fistula without esophageal atresia: are pull-back tube esophagograms needed for diagnosis?

    Energy Technology Data Exchange (ETDEWEB)

    Laffan, E.E.; Daneman, A.; Kerrigan, D.; Manson, D.E. [Univ. of Toronto (Canada). Dept. of Diagnostic Imaging; Ein, S.H. [Univ. of Toronto (Canada). Div. of General Surgery

    2006-11-15

    A pull-back tube esophagogram (PBTE) is widely accepted in the literature as the radiological investigation of choice for the diagnosis of tracheoesophageal fistula without esophageal atresia. However, PBTE is rarely performed in our institution, as we have been successful in confirming the presence of such fistulae with a contrast material swallow (CS). We hypothesized that PBTE is not the radiological investigation of choice for the diagnosis of the fistula in this condition. We sought to determine what proportion of patients with tracheoesophageal fistula without esophageal atresia can be diagnosed promptly by a CS and what the indications are for a PBTE. We retrospectively analyzed the clinical and radiological findings in patients with tracheoesophageal fistula without esophageal atresia to determine whether the fistula was diagnosed with a CS or PBTE. We identified 20 children (13 female and 7 male) with tracheoesophageal fistula without esophageal atresia. Their age at diagnosis ranged from 3 days to 168 months with a median of 9 days. The diagnosis was documented by CS in 12, PBTE in 7 and CT in 1. In three of the seven who had the fistula documented by PBTE, a previous CS had shown contrast material in the trachea, but no fistulous tract or aspiration was identified. We believe that CS should be the examination of choice in most patients suspected of having a tracheoesophageal fistula without esophageal atresia. A PBTE is indicated in patients who are intubated or are at significant risk of aspiration. Furthermore, a PBTE is also indicated in patients who are intubated or are at significant risk of aspiration. Furthermore, a PBTE is also indicated where contrast material is seen in the airway on CS and there is uncertainty whether this is due to aspiration or a fistula.

  4. Effects of Early Feeding Support on the Postoperative Weight Gain Status of Infants with Esophageal Atresia

    Directory of Open Access Journals (Sweden)

    Marzieh Ghorbani

    2016-07-01

    Full Text Available Background: Infants may lose their body resources after surgery due to inadequate nutrient intake and undergoing long periods of fasting after surgery for esophageal atresia, increases risk of several complications. Aim: This study aimed to evaluate the effects of early feeding support on the postoperative weight gain status of infants with esophageal atresia. Method: This randomized, controlled clinical trial was conducted on 36 infants with esophageal atresia (type C selected 48 hours after surgery during July 2015-March 2016 at Dr. Sheikh Hospital of Mashhad, Iran. In the intervention group, detecting no lack of leakage on chest X-ray, feeding was initiated and the control group received routine feeding. Neonatal weight changes were measured daily using a digital scale (TANITA model since the first day after the surgery and one month after discharge from the hospital. Data was analysed using SPSS version 16 by independent T-test and Chi-square. Results: Mean neonatal weight on admission was 2558.1±337.4 grams in the intervention group and 2547.6±856 grams in the control group (P=0.47. Results of independent T-test showed that daily weight gain before and after feeding was significantly higher in the intervention group compared to the control group (P=0.01. Moreover, weight gain one month after discharge had a significant difference between infants of the intervention and control groups (P=0.03. Implications for Practice: According to the results of this study, early feeding support could improve the weight index of neonates with esophageal atresia. Considering the possible complications and long-term consequences of surgery, early initiation of feeding could be an appropriate remedial measure in infants with esophageal atresia.

  5. The Anal Pap Smear: Cytomorphology of squamous intraepithelial lesions

    OpenAIRE

    Arain Shehla; Walts Ann; Thomas Premi; Bose Shikha

    2005-01-01

    Abstract Background Anal smears are increasingly being used as a screening test for anal squamous intraepithelial lesions (ASILs). This study was undertaken to assess the usefulness and limitations of anal smears in screening for ASILs. Methods The cytomorphological features of 200 consecutive anal smears collected in liquid medium from 198 patients were studied and findings were correlated with results of surgical biopsies and/or repeat smears that became available for 71 patients within six...

  6. Teaching Men's Anal Pleasure: Challenging Gender Norms with "Prostage" Education

    Science.gov (United States)

    Branfman, Jonathan; Ekberg Stiritz, Susan

    2012-01-01

    To help students critique sex/gender norms, sexuality educators should address men's anal pleasure. Men's anal receptivity blurs accepted binaries like male/female, masculine/feminine, and straight/queer. By suppressing men's receptivity, the taboo against men's anal pleasure helps legitimize hegemonic sex/gender beliefs--and the sexism,…

  7. Anomalies without Massless Particles

    CERN Document Server

    Gurlanik, Z

    1994-01-01

    Baryon and lepton number in the standard model are violated by anomalies, even though the fermions are massive. This problem is studied in the context of a two dimensional model. In a uniform background field, fermion production arise from non-adiabatic behavior that compensates for the absence of massless modes. On the other hand, for localized instanton-like configurations, there is an adiabatic limit. In this case, the anomaly is produced by bound states which travel across the mass gap. The sphaleron corresponds to a bound state at the halfway point.

  8. Congenital laryngeal anomalies,

    Directory of Open Access Journals (Sweden)

    Michael J. Rutter

    2014-12-01

    Full Text Available Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the main aspects concerning airway management of infants will be analyzed. Conclusions: It is critical for clinicians to understand issues relevant to the airway management of infants.

  9. KRAS and BRAF mutations in anal carcinoma

    DEFF Research Database (Denmark)

    Serup-Hansen, Eva; Linnemann, Dorte; Høgdall, Estrid;

    2015-01-01

    The EGF receptor (EGFR) is expressed in most cases of anal carcinomas. Anecdotal benefit from EGFR-targeted therapy has been reported in anal cancer and a negative correlation with Kirsten Ras (KRAS) mutation status has been proposed. The purpose of this retrospective study was to investigate...... the frequency and the prognostic value of KRAS and BRAF mutations in a large cohort of patients with anal cancer. One hundred and ninety-three patients with T1-4N0-3M0-1 anal carcinoma were included in the study. Patients were treated with curative (92%) or palliative intent (8%) between January 2000...... and January 2010. KRAS mutations were detected using Therascreen(®)KRAS real-time PCR assay (Qiagen) and V600E or V600D/K BRAF mutations were uncovered using Pyrosequencing. The frequency of KRAS and BRAF mutations was low; KRAS mutations were detected in 1.6% and BRAF mutations in 4.7% of the biopsies...

  10. [Diagnostics and conservative treatment of anal incontinence].

    Science.gov (United States)

    Geile, Dorothea; Osterholzer, Georg; Rosenberg, Robert

    2004-01-01

    Anal incontinence is diagnosed primarily by clinical and proctologic examination. Etiological factors of the disease are found in 85% of the patients by additional examinations. Motility dysfunction of colon and rectum has to be excluded (stenosis, dyschezia, internal hernias). Because anal incontinence is a multifactorial disease as a rule, the single compounds have to be diagnosed and have to undergo therapy. Accordingly, useful investigations are: endorectal ultrasound (defect of muscle, inflammatory or tumour infiltration), manometry (alteration of either anal resting pressure and/or anal squeezing pressure) and surface electromyography (ability of contraction, duration of contraction, strength). Neurophysiological examinations are: needle electromyography, pudendal nerve latency time measurement (PNLT). The occurrence of nerve damage determines the outcome of operative intervention! Conservative treatment is indicated in 80 to 90% of all patients, even higher when one includes all patients in the perioperative period. Possible therapy modalities are: nutrition consultation, physiotherapy, pelvic floor training, biofeedback training of pelvic floor and sphincter muscles, electrostimulation and the combination of both (EMG-triggered electrostimulation). Short-term results are satisfying in up to 85% of patients, but later, successful results depend on the patient's willingness or ability to continue training, and on his/her age.

  11. Treatment of non-IBD anal fistula

    DEFF Research Database (Denmark)

    Lundby, Lilli; Hagen, Kikke; Christensen, Peter;

    2015-01-01

    The course of the fistula tract in relation to the anal sphincter is identified by clinical examination under general anaesthesia using a fistula probe and injection of fluid into the external fistula opening. In the event of a complex fistula or in the case of fistula recurrence, this should be ...

  12. High-resolution magnetic resonance imaging of the anal sphincter using a dedicated endoanal receiver coil

    Energy Technology Data Exchange (ETDEWEB)

    DeSouza, N.M.; Williams, A.D.; Gilderdale, D.J. [Dept. of Radiology, Imperial College School of Medicine, London (United Kingdom)

    1999-04-01

    The use of a surface coil in MR imaging improves signal-to-noise ratio of adjacent tissues of interest. We therefore devised an endoanal receiver coil for imaging the anal sphincter. The probe is solid and re-usable: it comprises a saddle geometry receiver with integral tuning, matching and decoupling. It is placed in the anal canal and immobilised externally. Both in vitro and in vivo normal anatomy is identified. The mucosa is high signal intensity, the submucosa low signal intensity, the internal sphincter uniformly high signal intensity and the external sphincter low signal intensity on T1- and T2-weighted images. In females, the transverse perineal muscle bridges the inferior part of the external sphincter anteriorly. In perianal sepsis, collections and the site of the endoanal opening are identified. In early-onset fecal incontinence following obstetric trauma/surgery, focal sphincter defects are demonstrated; in late-onset fecal incontinence external sphincter atrophy is seen. In fecally incontinent patients with scleroderma, forward deviation of the anterior sphincter musculature with descent of rectal air and feces into the anal canal is noted. The extent of sphincter invasion is assessed in low rectal tumours. In children with congenital anorectal anomalies, abnormalities of the muscle components are defined using smaller-diameter coils. Such information is invaluable in the assessment and surgical planning of patients with a variety of anorectal pathologies. (orig.) With 15 figs., 26 refs.

  13. A case of solitary median maxillary central incisor (SMMCI) syndrome with bilateral pyriform aperture stenosis and choanal atresia.

    Science.gov (United States)

    Blackmore, Kate; Wynne, David M

    2010-08-01

    Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities. It may present with life threatening respiratory distress in the neonate secondary to nasal malformations. These include pyriform aperture stenosis and choanal atresia. We present the first reported case of simultaneous choanal atresia and pyriform aperture stenosis in a neonate with solitary median maxillary central incisor syndrome. The clinical presentation and the management of congenital pyriform aperture stenosis are discussed. PMID:20627328

  14. Definitive exclusion of biliary atresia in infants with cholestatic jaundice: the role of percutaneous cholecysto-cholangiography.

    Science.gov (United States)

    Nwomeh, Benedict C; Caniano, Donna A; Hogan, Mark

    2007-09-01

    Definitive exclusion of biliary atresia in the infant with cholestatic jaundice usually requires operative cholangiography. This approach suffers from the disadvantage that sick infants are subjected to a time-consuming and potentially negative surgical exploration. The purpose of this study was to determine if percutaneous cholecystocholangiography (PCC) prevents unnecessary laparotomy in infants whose cholestasis is caused by diseases other than biliary atresia. This study is a 10 year retrospective review of all infants with persistent direct hyperbilirubinemia and inconclusive biliary nuclear scans who underwent further evaluation for suspected biliary atresia. A gallbladder ultrasound (US) was obtained in all patients. When the gallbladder was visualized, further imaging by PCC was done under intravenous sedation; otherwise, the standard operative cholangiogram (OCG) was performed, with liver biopsy as indicated. The primary outcome was the diagnostic accuracy of PCC, especially with respect to preventing a laparotomy. There were 35 infants with suspected biliary atresia, with a mean age of 8 weeks (range 1-14 weeks). Nine infants whose gallbladder was visualized by ultrasound underwent PCC that definitively excluded biliary atresia. Of this group, the most frequent diagnosis (five patients) was total parenteral nutrition-associated cholestasis. The other 26 infants with absent or decompressed gallbladder had laparotomy and OCG, which identified biliary atresia in 16 patients (61%). Laparotomy was avoided in all 9 patients who underwent PCC, thus reducing the negative laparotomy rate by 47%. There were no complications associated with PCC. Several alternative techniques to operative cholangiogram have been described for the definitive exclusion of biliary atresia, but many of these have distinct drawbacks. Advances in interventional radiology techniques have permitted safe percutaneous contrast evaluation of the biliary tree. Identification of a normal gall

  15. Presence of accessory penis, colonic duplication and several other congenital anomalies in a child: a very rare association.

    Science.gov (United States)

    Chatterjee, Sayan; Mondal, Prabodh Chandra; Pandey, Shashi Bhushan; Achar, Arun

    2014-10-01

    An accessory penis is a very rare anomaly. Only five cases have been reported thus far to our knowledge. We present the case of a child aged 2 years and 10 months who had a penis-like structure (containing phallus and glans) attached to the right buttock. Associated anomalies were a non-communicating type of colonic duplication, a paramedian stenosed anal opening, a horse-shoe kidney, posterior urethral valves, scoliosis of the lumbo-sacral spine, polydactyly and equino-varus deformity of the right foot. As far as we can tell, this is the first report of an accessory penis associated with colonic duplication and other congenital anomalies.

  16. Anomaly Busters II

    International Nuclear Information System (INIS)

    The anomaly busters had struck on the first day of the Kyoto meeting with Yoji Totsuka of Tokyo speaking on baryon number nonjjonservation and 'related topics'. The unstable proton is a vital test of grand unified pictures pulling together the electroweak and quark/gluon forces in a single field theory

  17. Minnesota Bouguer Anomaly Grid

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — A 1.5 kilometer Bouguer anomaly grid for the state of Minnesota. Number of columns is 404 and number of rows is 463. The order of the data is from the lower left to...

  18. Bolivian Bouguer Anomaly Grid

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — A 1 kilometer Bouguer anomaly grid for the country of Bolivia.Number of columns is 550 and number of rows is 900. The order of the data is from the lower left to...

  19. North Atlantic Temperature Anomaly

    OpenAIRE

    Vukcevic, M.A.

    2009-01-01

    The author postulates the existence of a high correlation between North Atlantic Temperature Anomaly and the variations of magnetic field over the Hudson Bay region. Post-glacial uplift and convection in the underlying mantle uplift (as reflected in changes of the area's magnetic intensity) are making significant contribution to the Atlantic basin climate change.

  20. Anomalies and elliptic operators

    International Nuclear Information System (INIS)

    The coefficients of asymptotic expansion Spexp(-tA) at t→0 are calculated for the quantum field theory operators. It is shown how to apply these results to the calculations of axial and conformal anomalies, the charge renormalization in gauge theory and effective action in twodimensional electrodynamics

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

    Science.gov (United States)

    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.

  2. The Pioneer Anomaly

    Directory of Open Access Journals (Sweden)

    Viktor T. Toth

    2010-09-01

    Full Text Available Radio-metric Doppler tracking data received from the Pioneer 10 and 11 spacecraft from heliocentric distances of 20-70 AU has consistently indicated the presence of a small, anomalous, blue-shifted frequency drift uniformly changing with a rate of ~6 × 10–9 Hz/s. Ultimately, the drift was interpreted as a constant sunward deceleration of each particular spacecraft at the level of aP = (8.74 ± 1.33 × 10–10 m/s2. This apparent violation of the Newton's gravitational inverse square law has become known as the Pioneer anomaly; the nature of this anomaly remains unexplained. In this review, we summarize the current knowledge of the physical properties of the anomaly and the conditions that led to its detection and characterization. We review various mechanisms proposed to explain the anomaly and discuss the current state of efforts to determine its nature. A comprehensive new investigation of the anomalous behavior of the two Pioneers has begun recently. The new efforts rely on the much-extended set of radio-metric Doppler data for both spacecraft in conjunction with the newly available complete record of their telemetry files and a large archive of original project documentation. As the new study is yet to report its findings, this review provides the necessary background for the new results to appear in the near future. In particular, we provide a significant amount of information on the design, operations and behavior of the two Pioneers during their entire missions, including descriptions of various data formats and techniques used for their navigation and radio-science data analysis. As most of this information was recovered relatively recently, it was not used in the previous studies of the Pioneer anomaly, but it is critical for the new investigation.

  3. Astrometric solar system anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Nieto, Michael Martin [Los Alamos National Laboratory; Anderson, John D [PROPULSION LABORATORY

    2009-01-01

    There are at least four unexplained anomalies connected with astrometric data. perhaps the most disturbing is the fact that when a spacecraft on a flyby trajectory approaches the Earth within 2000 km or less, it often experiences a change in total orbital energy per unit mass. next, a secular change in the astronomical unit AU is definitely a concern. It is increasing by about 15 cm yr{sup -1}. The other two anomalies are perhaps less disturbing because of known sources of nongravitational acceleration. The first is an apparent slowing of the two Pioneer spacecraft as they exit the solar system in opposite directions. Some astronomers and physicists are convinced this effect is of concern, but many others are convinced it is produced by a nearly identical thermal emission from both spacecraft, in a direction away from the Sun, thereby producing acceleration toward the Sun. The fourth anomaly is a measured increase in the eccentricity of the Moon's orbit. Here again, an increase is expected from tidal friction in both the Earth and Moon. However, there is a reported unexplained increase that is significant at the three-sigma level. It is produent to suspect that all four anomalies have mundane explanations, or that one or more anomalies are a result of systematic error. Yet they might eventually be explained by new physics. For example, a slightly modified theory of gravitation is not ruled out, perhaps analogous to Einstein's 1916 explanation for the excess precession of Mercury's perihelion.

  4. Prevalence of anal human papillomavirus infection and anal HPV-related disorders in women: a systematic review.

    Science.gov (United States)

    Stier, Elizabeth A; Sebring, Meagan C; Mendez, Audrey E; Ba, Fatimata S; Trimble, Debra D; Chiao, Elizabeth Y

    2015-09-01

    The aim of this study was to systematically review the findings of publications addressing the epidemiology of anal human papillomavirus (HPV) infection, anal intraepithelial neoplasia, and anal cancer in women. We conducted a systematic review among publications published from Jan. 1, 1997, to Sept. 30, 2013, to limit to publications from the combined antiretroviral therapy era. Three searches were performed of the National Library of Medicine PubMed database using the following search terms: women and anal HPV, women anal intraepithelial neoplasia, and women and anal cancer. Publications were included in the review if they addressed any of the following outcomes: (1) prevalence, incidence, or clearance of anal HPV infection, (2) prevalence of anal cytological or histological neoplastic abnormalities, or (3) incidence or risk of anal cancer. Thirty-seven publications addressing anal HPV infection and anal cytology remained after applying selection criteria, and 23 anal cancer publications met the selection criteria. Among HIV-positive women, the prevalence of high-risk (HR)-HPV in the anus was 16-85%. Among HIV-negative women, the prevalence of anal HR-HPV infection ranged from 4% to 86%. The prevalence of anal HR-HPV in HIV-negative women with HPV-related pathology of the vulva, vagina, and cervix compared with women with no known HPV-related pathology, varied from 23% to 86% and from 5% to 22%, respectively. Histological anal high-grade squamous intraepithelial lesions (anal intraepithelial neoplasia 2 or greater) was found in 3-26% of the women living with HIV, 0-9% among women with lower genital tract pathology, and 0-3% for women who are HIV negative without known lower genital tract pathology. The incidence of anal cancer among HIV-infected women ranged from 3.9 to 30 per 100,000. Among women with a history of cervical cancer or cervical intraepithelial neoplasia 3, the incidence rates of anal cancer ranged from 0.8 to 63.8 per 100,000 person-years, and in

  5. Increased conjugated bilirubin is sufficient to initiate screening for biliary atresia

    DEFF Research Database (Denmark)

    Madsen, Stine Skipper; Kvist, Nina; Thorup, Jørgen

    2015-01-01

    cirrhosis. The Danish Health and Medicines Authority (DHMA) demands diagnostic evaluation of children with elevated level of serum bilirubin after two weeks of age. Biliary atresia has to be excluded if conjugated bilirubin level is above than 20 μmol/l, and/or more than 20% of total bilirubin......: During the period, 73 patients where operated with a portoenterostomy ad modum Kasai. Patients older than 84 days at the time of operation were excluded, 54 patients were available for analysis. Conjugated bilirubin in μmol/l and the percentage value were significantly above the DHMA threshold limit......: mean 129.7 μmol/l (42-334 μmol/l) and 73% (28-97%), respectively. CONCLUSION: The total amount of conjugated bilirubin above 20 μmol/l is sufficient to require further evaluation for biliary atresia. The percentage value is unnecessary and may cause confusion. FUNDING: none. TRIAL REGISTRATION...

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

    Science.gov (United States)

    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.

  7. Risk factors of recurrent anal sphincter ruptures

    DEFF Research Database (Denmark)

    Jangö, Hanna; Langhoff-Roos, J; Rosthøj, Steen;

    2012-01-01

    Please cite this paper as: Jangö H, Langhoff-Roos J, Rosthøj S, Sakse A. Risk factors of recurrent anal sphincter ruptures: a population-based cohort study. BJOG 2012;00:000-000 DOI: 10.1111/j.1471-0528.2012.03486.x. Objective  To determine the incidence and risk factors of recurrent anal sphincter...... were used to determine risk factors of recurrent ASR. Main outcome measures  The incidence of recurrent ASR and odds ratios for possible risk factors of recurrent ASR: age, body mass index, grade of ASR, birthweight, head circumference, gestational age, presentation, induction of labour, oxytocin...... augmentation, epidural, episiotomy, vacuum extraction, forceps, shoulder dystocia, delivery interval and year of second delivery. Results  Out of 159 446 women, 7336 (4.6%) experienced an ASR at first delivery, and 521 (7.1%) had a recurrent ASR (OR 5.91). The risk factors of recurrent ASR in the multivariate...

  8. Squamous cell carcinoma of the anal canal.

    LENUS (Irish Health Repository)

    Martin, F T

    2012-01-31

    Squamous cell carcinoma ofthe anal canal represents 1.5% of all malignancies affectingthe gastrointestinal tract. Over the past 20 years dramatic changes have been seen in both the epidemiological distribution of the disease and in the therapeutic modalities utilised to manage it. CLINICAL MANAGEMENT: Historically abdominoperineal resection had been the treatment of choice with local resection reserved for early stage disease. Work by Nigro et al. has revolutionised how we currently manage carcinoma of the anal canal, demonstrating combined modality chemoradiotherapy as an appropriate alternative to surgical resection with the benefit of preserving sphincter function. Surgery is then reserved for recurrent disease with salvage abdominoperineal resection. This article reviews current literature and highlights the changing therapeutic modalities with selected clinical cases

  9. What Causes Biliary Atresia? Unique Aspects of the Neonatal Immune System Provide Clues to Disease Pathogenesis

    OpenAIRE

    Mack, Cara L.

    2015-01-01

    Biliary atresia (BA) is the most frequent identifiable cause of neonatal cholestasis and the majority of patients will need liver transplantation for survival. Despite surgical intervention with the Kasai portoenterostomy, significant fibrosis and cirrhosis develops early in life. An increased understanding of what causes this inflammatory fibrosing cholangiopathy will lead to therapies aimed at protecting the intrahepatic biliary system from immune-mediated damage. This review focuses on stu...

  10. Multidetector Computed Tomography Assessment in Biliary Atresia for the Diagnosis of Portosystemic Collaterals before Liver Transplant

    International Nuclear Information System (INIS)

    Introduction: Chronic liver disease increases portal vein pressure and modifies splanchnic circulation. This is particularly significant in infants with biliary atresia. Large collaterals steal portal flow and increase the risk of post transplant portal vein thrombosis. Objective: to describe different types of portosystemic collaterals prior to liver transplantation with low-dose multidetector CT (MDCT) in patients with biliary atresia. Material and methods: 13 patients with severe liver dysfunction due to biliary atresia underwent low-dose 64-MDCT before liver transplantation (effective tube current ranged from 20 to 120 mAs according to weight, with a kilo voltage of 80-120 for all CT). Hepatic arterial and portal venous phases were performed after IV contrast administration [1.5-2 ml/kg]. The mean age of the study group was1 year (range, 4 months to 3.6 years). Two radiologists reviewed the CT images to determine the grade and types of the portosystemic collaterals. Results: A total of 16 CT scans were obtained.the most common portosystemic collaterals found were esophageal (11), gastric submucosal (8), gastric adventitial (7, splenic (7), hemorrhoidal (10), mesenteric [dilated or tortuous branches of the inferior mesenteric vein (8)], retroperitoneal varices [gastro renal shunt (10), splenorenal shunt (4)] and dilated or tortuous left gastric vein (13). Conclusion: MDCT provides important information on venous system patency, presence of varices, and location of venous shunts in pediatric patients with biliary atresia going to liver transplant. in addition, it is critical to detect collaterals that are not evident on ultrasound in order to avoid the steal phenomenon that may lead to portal vein thrombosis and graft failure.

  11. Equol inhibits growth, induces atresia, and inhibits steroidogenesis of mouse antral follicles in vitro.

    Science.gov (United States)

    Mahalingam, Sharada; Gao, Liying; Gonnering, Marni; Helferich, William; Flaws, Jodi A

    2016-03-15

    Equol is a non-steroidal estrogen metabolite produced by microbial conversion of daidzein, a major soy isoflavone, in the gut of some humans and many animal species. Isoflavones and their metabolites can affect endogenous estradiol production, action, and metabolism, potentially influencing ovarian follicle function. However, no studies have examined the effects of equol on intact ovarian antral follicles, which are responsible for sex steroid synthesis and further development into ovulatory follicles. Thus, the present study tested the hypothesis that equol inhibits antral follicle growth, increases follicle atresia, and inhibits steroidogenesis in the adult mouse ovary. To test this hypothesis, antral follicles isolated from adult CD-1 mice were cultured with vehicle control (dimethyl sulfoxide; DMSO) or equol (600 nM, 6 μM, 36 μM, and 100 μM) for 48 and 96 h. Every 24h, follicle diameters were measured to monitor growth. At 48 and 96 h, the culture medium was subjected to measurement of hormone levels, and the cultured follicles were subjected to gene expression analysis. Additionally, follicles were histologically evaluated for signs of atresia after 96 h of culture. The results indicate that equol (100 μM) inhibited follicle growth, altered the mRNA levels of bcl2-associated X protein and B cell leukemia/lymphoma 2, and induced follicle atresia. Further, equol decreased the levels of estradiol, testosterone, androstenedione, and progesterone, and it decreased mRNA levels of cholesterol side-chain cleavage, steroid 17-α-hydroxalase, and aromatase. Collectively, these data indicate that equol inhibits growth, increases atresia, and inhibits steroidogenesis of cultured mouse antral follicles. PMID:26876617

  12. Sirenomelia and ndash; Mermaid Syndrome with Oesophageal Atresia: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Veena Raja

    2015-09-01

    Full Text Available Sirenomelia or Mermaid syndrome is a very rare congenital deformity in which legs are fused together and commonly associated with abnormal kidney development, genital, and rectal abnormalities. In this present case, sirenomelia was associated with oesophageal atresia, which is a rare association and occurs in about 20-35% of cases. [J Interdiscipl Histopathol 2015; 3(3.000: 113-116

  13. Portal pressure and blood nitric oxide levels as predictors of outcome in biliary atresia

    OpenAIRE

    Vikram Khanna; Veereshwar Bhatnagar; Sandeep Agarwala; Maddur Srinivas; Nibhriti Das; Manoj Kumar Singh

    2016-01-01

    Aim: To evaluate the incidence of portal hypertension (PHT) in biliary atresia (BA) patients and to monitor its progress after Kasai portoenterostomy (KP) by measuring nitric oxide (NO) levels in peripheral blood. Materials and Methods: A prospective cross-sectional study conducted over a period of 2 years. Intraoperative portal pressure (PP) and blood NO levels at presentation, 1-month, 3-month, and 6-month follow-up, were correlated with clinical and biochemical parameters in BA patients. T...

  14. Management of Duodenal Atresia in the setting of Congenital Leukemia with Massive Hepatomegaly

    Directory of Open Access Journals (Sweden)

    Sathyaprasad C Burjonrappa

    2013-06-01

    Full Text Available Down's syndrome (DS is associated with duodenal atresia (DA in about 8-10% of cases. Transient Myeloproliferative Disorder (TMD/Acute Myeloid Leukemia (AML is also associated with the trisomy 21 mutation. The occurrence of the two conditions together complicates the diagnosis and surgical management of the DA. We discuss the technical aspects of management of the DA in this clinical setting.

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

    Science.gov (United States)

    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.

  16. Radiological evaluation of pulmonary atresia: An analysis of cineangiography in 32 cases

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Dong Ho; Yeon, Kyung Mo; Park, Jae Hyung; Han, Man Chung; Yoon, Yong Soo [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1983-06-15

    Total 32 cases of pulmonary atresia were diagnosed radiographically at Seoul National University Hospital from March, 1979 to August 1982. Some characteristic radiological findings were analyzed in chest PA and cineangiographies. The results were as follows: 1. In the evaluation of chest PA, cardiomegaly was noticed in 16 cases, dextrocardia in 8 cases, elevated cardiac apex in 7 cases and right-sided aortic arch in 6 cases. The pulmonary vascularties were mildly decreased in 20 cases, markedly decreased in 9 cases and decreased with reticular pattern in 3 cases. 2. As final diagnoses after cineangiography, pulmonary atresia was associated with Tetralogy of Fallot variant in 17 cases, transposition of great vessels in 7 cases, single ventricle in 5 cases, tricuspid atresia in 2 cases and intact ventricular septum in 1 case. 3. The classification according to the pattern of pulmonary artery is main pulmonary trunk with PDA(Type Ia) in 10 cases, pulmonary arterial confluence with PDA (Type Ib) in 10 cases, no pulmonary arterial confluence with PDA (Type Ic) in 5 cases, main pulmonary trunk without PDA (Type IIa) in 0 case, pulmonary arterial confluence without PDA (Type IIb) in 5 cases, and no pulmonary arterial confluence without PDA ( Type IIc) in 2 cases. 4. Pulmonary wedge venography was done and successful in 8 cases. Among them, confluence between right and left pulmonary arteries was noticed in 5 cases. 5. Biventricular cineangiograpy and/or pulmonary wedge venography, if necessary, is essential for the accurate diagnosis of pulmonary atresia to demonstrate detailed anatomy of pulmonary artery.

  17. Extrahepatic biliary atresia with choledochal cyst: Prenatal MRI predicted and post natally confirmed: A case report

    OpenAIRE

    Madhavi Nori; J Venkateshwarlu; Vijaysekhar,; Raghavendra Prasad, G.

    2013-01-01

    Extrahepatic biliary atresia (EHBA) is an uncommon cause of neonatal jaundice. Antenatal Magnetic Resonance Imaging (MRI) diagnosis of EHBA has not been published to the best of our knowledge till date. EHBA with cystic component is likely to be mistaken for choledochal cyst. A case that was antenatally predicted and postnatally confirmed by surgery and histopathology is being reported. All imaging signs are analyzed herewith. Imaging helps in the prediction of EHBA and also helps in early po...

  18. Non-invasive assessment of pulmonary blood supply after staged repair of pulmonary atresia.

    OpenAIRE

    Del Torso, S.; Kelly, M J; Kalff, V; Stellin, G; Mee, R B; Venables, A W

    1985-01-01

    Radionuclide studies were performed to determine pulmonary blood flow in six children who had undergone surgery for pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary arteries with or without major aortopulmonary collateral arteries. Lung blood flow was assessed from both particle perfusion lung scans and the pulmonary and systemic phase of a radionuclide dynamic flow study. Five patients had perfusion defects identified on the particle perfusion lung scan. In three of th...

  19. A functional study on small intestinal smooth muscles in jejunal atresia

    Directory of Open Access Journals (Sweden)

    Preeti Tyagi

    2016-01-01

    Full Text Available Aim: The present study was aimed to assess the contractile status of neonatal small intestinal smooth muscle of dilated pre-atretic part of intestinal atresia to resolve debatable issues related to mechanisms of persistent dysmotility after surgical repair. Materials and Methods: A total of 34 longitudinally sectioned strips were prepared from pre-atretic dilated part of freshly excised 8 jejunal atresia type III a cases. Spontaneous as well as acetylcholine- and histamine-induced contractions were recorded in vitro by using organ bath preparations. Chemically evoked contractions were further evaluated after application of atropine (muscarinic blocker, pheniramine (H1 blocker, and lignocaine (neuronal blocker to ascertain receptors and neuronal involvement. Histological examinations of strips were made by using Masson trichrome stain to assess the fibrotic changes. Results: All 34 strips, except four showed spontaneous contractions with mean frequency and amplitude of 5.49 ± 0.26/min and 24.41 ± 5.26 g/g wet tissue respectively. The response to ACh was nearly twice as compared to histamine for equimolar concentrations (100 μM. ACh (100 μM induced contractions were attenuated (by 60% by atropine. Histamine (100 μM-induced contractions was blocked by pheniramine (0.32 μM and lignocaine (4 μM by 74% and 78%, respectively. Histopathological examination showed varying degree of fibrotic changes in muscle layers. Conclusions: Pre-atretic dilated part of jejunal atresia retains functional activity but with definitive histopathologic abnormalities. It is suggested that excision of a length of pre-atretic part and early stimulation of peristalsis by locally acting cholinomimetic or H1 agonist may help in reducing postoperative motility problems in atresia patients.

  20. Stoma-Related Variceal Bleeding: An Under-Recognized Complication of Biliary Atresia

    OpenAIRE

    Smith, Sam; Wiener, Eugene S.; Starzl, Thomas E.; Rowe, Marc I.

    1988-01-01

    The medical records of 52 children with biliary atresia treated by portoenterostomy and evaluated for liver transplantation were reviewed to determine the frequency of stoma variceal bleeding and the optimal strategies for prevention and treatment. Eighteen patients had had prior stoma closure, four by preperitoneal closure without takedown from the abdominal wall. Three of the four developed occult variceal bleeding from the stoma closure site. Twenty-two patients had a stoma present at eval...

  1. Management of Anal Squamous Intraepithelial Lesions

    OpenAIRE

    Pineda, Carlos E.; Welton, Mark L.

    2009-01-01

    Anal squamous intraepithelial lesions include both low-grade squamous intraepithelial lesions (LSIL) and high-grade squamous intraepithelial lesions (HSIL) and are caused by chronic infection with the human papillomavirus (HPV). The disease is increasing in both incidence and prevalence, especially among patients with the following risk factors: homosexual men, acquired or iatrogenic immunosuppression, and presence of other HPV-related diseases. Although the natural history of the disease is ...

  2. Anomalies, Branes, and Currents

    OpenAIRE

    Cheung, Yeuk-Kwan E.; Yin, Zheng

    1997-01-01

    When a D-brane wraps around a cycle of a curved manifold, the twisting of its normal bundle can induce chiral asymmetry in its worldvolume theory. We obtain the general form of the resulting anomalies for D-branes and their intersections. They are not cancelled among themselves, and the standard inflow mechanism does not apply at first sight because of their apparent lack of factorizability and the apparent vanishing of the corresponding inflow. We show however after taking into consideration...

  3. Comparison between anal endosonography and digital examination in the evaluation of anal fistulae.

    Science.gov (United States)

    Choen, S; Burnett, S; Bartram, C I; Nicholls, R J

    1991-04-01

    A prospective trial was performed comparing the accuracy of digital examination and anal endosonography in defining the anatomy of anal fistulae. Before operation 38 consecutive patients were assessed by the consultant in charge of the case, by a research fellow and by anal endosonography involving two radiologists. These findings were compared with the operative findings. Consultants correctly identified 26 of 33 internal openings, 29 of 34 primary tracks and 15 of 21 secondary tracks. The research fellow correctly identified 26 internal openings, 24 primary tracks and 10 secondary tracks. There was no significant difference between the accuracy of consultants and the research fellow. Anal endosonography identified 10 internal openings based on initial criteria. This rose to 24 when revised ultrasonographic criteria were applied. There was no statistical difference between consultant assessment and anal ultrasonography in correctly identifying intersphincteric and transphincteric tracks. Ultrasonography is unable to assess primary superficial, suprasphincteric and extrasphincteric tracks or secondary supralevator and infralevator tracks. Consultant assessment of secondary supralevator and infralevator tracks was correct in 78 per cent of cases. PMID:2032103

  4. The Pioneer Anomaly

    CERN Document Server

    Turyshev, Slava G

    2010-01-01

    Radio-metric Doppler tracking data received from the Pioneer 10 and 11 spacecraft from heliocentric distances of 20-70 AU has consistently indicated the presence of a small, anomalous, blue-shifted frequency drift uniformly changing with a rate of ~6 x 10^{-9} Hz/s. Ultimately, the drift was interpreted as a constant sunward deceleration of each particular spacecraft at the level of a_P = (8.74 +/- 1.33) x 10^{-10} m/s^2. This apparent violation of the Newton's gravitational inverse-square law has become known as the Pioneer anomaly; the nature of this anomaly remains unexplained. In this review, we summarize the current knowledge of the physical properties of the discovered effect and the conditions that led to its detection and characterization. We review various mechanisms proposed to explain the anomaly and discuss the current state of efforts to determine its nature. A comprehensive new investigation of the anomalous behavior of the two Pioneers has begun recently. The new efforts rely on the much-extend...

  5. Development of a pulse height analizer

    International Nuclear Information System (INIS)

    The development of a Pulse Height Analizer is described. This equipment is essential to analize data coming from detectors producing information codified in pulse amplitudes. The system developed consist of a Signal Input Module connected to a Controller Module based on a 8085A microprocessor capable to memorize pulses up to 1 uS in 256 channels with a resolution better than 20 mV. A Communication Module with a serial interface is used for data transfer to a host computer using RS232c protocol. The Monitoring and Operation Module consist of a hexadecimal Keybord, a 6 digit 7-segment display and a XY analog output enabling real time visualization of data on a XY monitor. The hardware and the software designed for this low cost system were optimized to obtain a typical dead time of approximately 100 uS. As application, this device was used to adquire curves at the Small Angle X-ray Scattering Laboratory in this Department. The apparatus performance was tested by comparing its data with a Northern Pulse Height Analizer model NS633 output, with favorable results. (Author)

  6. Anal intraepitelial neoplasia: a narrative review

    Directory of Open Access Journals (Sweden)

    Garazi Elorza

    2016-01-01

    Full Text Available Anal intraepitelial neoplasia (AIN constitutes a major health problem in certain risk groups, such as patients with immunosuppression of varied origin, males who have sexual relations with other males, and females with a previous history of vaginal or cervical abnormalities in cytology. Its relationship with the human papillomavirus (HPV infection has been well documented; however, many of the factors involved in the progression and regression of the viral infection to dysplasia and anal carcinoma are unknown. AIN can be diagnosed through cytology of the anal canal or biopsy guided by high-resolution anoscopy. However, the need for these techniques in high-risk groups remains controversial. Treatment depends on the risk factors and given the high morbidity and high recurrence rates the utility of the different local treatments is still a subject of debate. Surgical biopsy is justified only in the case of progression suggesting lesions. The role of the vaccination in high-risk patients as primary prevention has been debated by different groups. However, there is no general consensus on its use or on the need for screening this population.

  7. Anal intraepitelial neoplasia: A narrative review.

    Science.gov (United States)

    Elorza, Garazi; Saralegui, Yolanda; Enríquez-Navascués, Jose María; Placer, Carlos; Velaz, Leyre

    2016-01-01

    Anal intraepitelial neoplasia (AIN) constitutes a major health problem in certain risk groups, such as patients with immunosuppression of varied origin, males who have sexual relations with other males, and females with a previous history of vaginal or cervical abnormalities in cytology. Its relationship with the human papillomavirus (HPV) infection has been well documented; however, many of the factors involved in the progression and regression of the viral infection to dysplasia and anal carcinoma are unknown. AIN can be diagnosed through cytology of the anal canal or biopsy guided by high-resolution anoscopy. However, the need for these techniques in high-risk groups remains controversial. Treatment depends on the risk factors and given the high morbidity and high recurrence rates the utility of the different local treatments is still a subject of debate. Surgical biopsy is justified only in the case of progression suggesting lesions. The role of the vaccination in high-risk patients as primary prevention has been debated by different groups. However, there is no general consensus on its use or on the need for screening this population. PMID:26765233

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

    Directory of Open Access Journals (Sweden)

    Abdel-Aziz Mosaad

    2012-06-01

    Full Text Available Abstract 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.

  9. BILIARY ATRESIA: evaluation on two distinct periods at a reference pediatric service

    Directory of Open Access Journals (Sweden)

    Thais Costa Nascentes QUEIROZ

    2014-03-01

    Full Text Available Context Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that pre­sents with biliary obstruction exclusively in the neonatal period. Objectives To assess the differences regarding age at referral, age at surgery, duration of propaedeutics and waiting time for surgery between two groups of infants in different periods. Methods Retrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011. Results Biliary atresia was diagnosed in 129 infants, being 48 in casuistic I and 81 in casuistic II. The median age at admission was 94 and 60 days, respectively (P = 0.0001. On evaluating patients who had undergone portoenterostomy before 120 days of age, no difference was observed regarding the duration of propaedeutics or waiting time for surgery (P = 0.15, but difference was found when comparing the age at surgery (P = 0.002. Among those infants with no biliary flow and without liver transplantation or death after 18 post-operative months, the estimated probability of survival was 44.6% and 38.7% in casuistics I and II, respectively. In casuistic I, all infants who showed biliary flow were alive during the observation period and, in casuistic II, 80.3% were alive after 7 years of follow-up. Conclusions Even though patients were admitted and treated earlier, it is clear that surgery could be done sooner. Delay in referral and timely propaedeutics were the main contributors.

  10. VATER/VACTERL Association and Caudal Regression with Xq25-q27.3 Microdeletion: A Case Report.

    Science.gov (United States)

    Puvabanditsin, Surasak; Van Gurp, James; February, Melissa; Khalil, Marwa; Mayne, Julia; Ai McConnell, Jennifer; Mehta, Rajeev

    2016-01-01

    We report a term female neonate with vertebral anomalies, anal and urethral atresia, esophageal atresia with tracheoesophageal fistula (TEF), renal agenesis, pulmonary hypoplasia, genital and sacral appendages, and a single umbilical artery. Genetic studies revealed a 20.91 Mb interstitial deletion of the long arm of X chromosome: Xq25-q27.3. This is a new case of VATER/VACTERL association with Xq25 microdeletion. PMID:26881326

  11. Isolated congenital tracheal stenosis in a preterm newborn.

    Science.gov (United States)

    Krause, Ulrich; Rödel, Ralph M W; Paul, Thomas

    2011-09-01

    Severe tracheal stenosis, resulting in functional atresia of the trachea is a rare congenital malformation with an estimated occurrence of two in 100,000 newborns. If no esophagotracheal fistula is present to allow for spontaneous breathing, this condition is usually fatal. We report on a male infant born at 32 weeks of gestation. The patient presented with respiratory distress immediately after delivery due to severe congenital tracheal stenosis resulting in functional atresia of the trachea. Endotracheal intubation failed and even emergency tracheotomy did not allow ventilation of the patient lungs. The patient finally succumbed to prolonged hypoxia due to functional tracheal atresia. The etiology of tracheal atresia and tracheal stenosis is still unclear, but both conditions are frequently combined with other anomalies of the VACTERL (vertebral anomalies, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal/radial anomalies and limb defects) and TACRD (tracheal agenesis, cardiac, renal and duodenal malformations) association. Conclusion Successful treatment of severe congenital tracheal stenosis and tracheal atresia depends on either prenatal diagnosis or recognition of this condition immediately after birth to perform tracheotomy without delay. Nevertheless, despite any efforts, the therapeutical results of severe tracheal stenosis and tracheal atresia are still unsatisfactory. PMID:21590265

  12. Electrophysiological observations on the human pudendo-anal reflex.

    OpenAIRE

    Varma, J S; Smith, A N; McInnes, A

    1986-01-01

    A reproducible electrophysiological technique is described to determine the latency of reflex contraction of the external anal sphincter in response to stimulation of the dorsal genital nerve: the pudendo-anal reflex. This was studied in 38 asymptomatic control subjects and 20 women with neurogenic faecal incontinence, supplemented by determination of the mean motor unit potential duration (MUPD) of the external anal sphincter and anorectal manometry. The reflex latency in the control group w...

  13. Current treatment options for management of anal intraepithelial neoplasia

    OpenAIRE

    Weis SE

    2013-01-01

    Stephen E Weis1,2 1Division of Dermatology, Department of Internal Medicine, University of North Texas Health Science Center at Fort Worth, 2Preventive Medicine Clinic, Tarrant County Public Health, Fort Worth, TX, USA Abstract: Anal squamous cell cancer is an uncommon malignancy caused by infection with oncogenic strains of Human papilloma virus. Anal cancer is much more common in immunocompromised persons, including those infected with Human immunodeficiency virus. High-grade anal intraepi...

  14. Environmental scan of anal cancer screening practices: worldwide survey results

    OpenAIRE

    Patel, Jigisha; Salit, Irving E.; Berry, Michael J.; de Pokomandy, Alexandra; Nathan, Mayura; Fishman, Fred; Palefsky, Joel; Tinmouth, Jill

    2014-01-01

    Anal squamous cell carcinoma is rare in the general population but certain populations, such as persons with HIV, are at increased risk. High-risk populations can be screened for anal cancer using strategies similar to those used for cervical cancer. However, little is known about the use of such screening practices across jurisdictions. Data were collected using an online survey. Health care professionals currently providing anal cancer screening services were invited to complete the survey ...

  15. Urinary System anomalies at birth

    Directory of Open Access Journals (Sweden)

    Sharada B. Menasinkai

    2015-06-01

    Full Text Available Background: Congenital anomalies of urinary system are common and are found in 3-4% of population, and lethal urinary anomalies account for 10% of termination of pregnancy. Methods: A study was done to know the incidence of congenital anomalies at birth for the period of 4 months from May 99 - Sept 99 at Cheluvamba hospital attached to Mysore medical college. Congenital anomalies in the still births, live births and aborted fetuses >20 weeks were studied along with the case history and ultrasound reports. Aborted fetuses and still born babies were collected for autopsy after the consent of parents. These babies were fixed in 10% formalin and autopsy was done after fixing, and anomalies were noted. Results: Total births during study period were 3000. There were 61 babies with congenital anomalies and 6 babies had anomalies of urinary system. Among the urinary system anomalies 1 baby had bilateral renal agenesis, 1 baby had unilateral renal agenesis with anophthalmia (Fraser syndrome, 2 babies had Multicystic dysplastic kidney disease (MCDK and 1 live baby had hydronephrosis due to obstruction at pelvi ureteric junction, and 1 live female baby had polycystic kidneys. Conclusion: Incidence of urinary system anomalies in the present study was 2 per 1000 births. U/S detection of urinary anomalies varies with period of gestation, amniotic fluid volume and visualisation of urinary bladder. Autopsy helps to detect renal agenesis. [Int J Res Med Sci 2015; 3(3.000: 743-748

  16. Analýza vybrané firmy

    OpenAIRE

    Křemečková, Tereza

    2013-01-01

    Tato bakalářská práce obsahuje celkovou analýzu firmy Pegas NONWOVENS. Práce se zabývá analýzami SWOT a SLEPTE, Porterovým pětifaktorovým modelem, Kralickovým Quick testem a vybranými ukazateli finanční analýzy. Na základě zpracování těchto analýz jsou navržena doporučení, která by mohla zlepšit situaci firmy.

  17. Environmental scan of anal cancer screening practices: worldwide survey results

    International Nuclear Information System (INIS)

    Anal squamous cell carcinoma is rare in the general population but certain populations, such as persons with HIV, are at increased risk. High-risk populations can be screened for anal cancer using strategies similar to those used for cervical cancer. However, little is known about the use of such screening practices across jurisdictions. Data were collected using an online survey. Health care professionals currently providing anal cancer screening services were invited to complete the survey via email and/or fax. Information was collected on populations screened, services and treatments offered, and personnel. Over 300 invitations were sent; 82 providers from 80 clinics around the world completed the survey. Fourteen clinics have each examined more than 1000 patients. Over a third of clinics do not restrict access to screening; in the rest, eligibility is most commonly based on HIV status and abnormal anal cytology results. Fifty-three percent of clinics require abnormal anal cytology prior to performing high-resolution anoscopy (HRA) in asymptomatic patients. Almost all clinics offer both anal cytology and HRA. Internal high-grade anal intraepithelial neoplasia (AIN) is most often treated with infrared coagulation (61%), whereas external high-grade AIN is most commonly treated with imiquimod (49%). Most procedures are performed by physicians, followed by nurse practitioners. Our study is the first description of global anal cancer screening practices. Our findings may be used to inform practice and health policy in jurisdictions considering anal cancer screening

  18. Environmental scan of anal cancer screening practices: worldwide survey results.

    Science.gov (United States)

    Patel, Jigisha; Salit, Irving E; Berry, Michael J; de Pokomandy, Alexandra; Nathan, Mayura; Fishman, Fred; Palefsky, Joel; Tinmouth, Jill

    2014-08-01

    Anal squamous cell carcinoma is rare in the general population but certain populations, such as persons with HIV, are at increased risk. High-risk populations can be screened for anal cancer using strategies similar to those used for cervical cancer. However, little is known about the use of such screening practices across jurisdictions. Data were collected using an online survey. Health care professionals currently providing anal cancer screening services were invited to complete the survey via email and/or fax. Information was collected on populations screened, services and treatments offered, and personnel. Over 300 invitations were sent; 82 providers from 80 clinics around the world completed the survey. Fourteen clinics have each examined more than 1000 patients. Over a third of clinics do not restrict access to screening; in the rest, eligibility is most commonly based on HIV status and abnormal anal cytology results. Fifty-three percent of clinics require abnormal anal cytology prior to performing high-resolution anoscopy (HRA) in asymptomatic patients. Almost all clinics offer both anal cytology and HRA. Internal high-grade anal intraepithelial neoplasia (AIN) is most often treated with infrared coagulation (61%), whereas external high-grade AIN is most commonly treated with imiquimod (49%). Most procedures are performed by physicians, followed by nurse practitioners. Our study is the first description of global anal cancer screening practices. Our findings may be used to inform practice and health policy in jurisdictions considering anal cancer screening. PMID:24740973

  19. Risk of Anal Cancer in People Living with HIV: Addressing Anal Health in the HIV Primary Care Setting.

    Science.gov (United States)

    Walker, Crystal Martin; Likes, Wendy; Bernard, Marye; Kedia, Satish; Tolley, Elizabeth

    2016-01-01

    Anal health and anal cancer are rarely addressed in HIV primary care. We sought to understand factors that impeded or promoted addressing anal health in HIV primary care from providers' perspectives. In this exploratory study, HIV primary care providers from the Mid-South region of the United States participated in brief individual interviews. We analyzed transcribed data to identify barriers and facilitators to addressing anal health. Our study sample included five physicians and four nurse practitioners. The data revealed a number of barriers such as perception of patient embarrassment, provider embarrassment, external issues such as time constraints, demand of other priorities, lack of anal complaints, lack of resources, and gender discordance. Facilitators included awareness, advantageous circumstances, and the patient-provider relationship. Anal health education should be prioritized for HIV primary care providers. Preventive health visits should be considered to mitigate time constraints, demands for other priorities, and unequal gender opportunities. PMID:27080925

  20. Acute iliofemoral venous thrombosis in patients with atresia of the inferior vena cava can be treated successfully with catheter-directed thrombolysis

    DEFF Research Database (Denmark)

    Broholm, Rikke; Jørgensen, Maja; Just, Sven;

    2011-01-01

    To assess the effectiveness and clinical outcomes of catheter-directed thrombolysis in patients with atresia of the inferior vena cava (IVC) and acute iliofemoral deep vein thrombosis (DVT).......To assess the effectiveness and clinical outcomes of catheter-directed thrombolysis in patients with atresia of the inferior vena cava (IVC) and acute iliofemoral deep vein thrombosis (DVT)....

  1. Observational manifestations of anomaly inflow

    OpenAIRE

    Boyarsky, Alexey; Ruchayskiy, Oleg; Shaposhnikov, Mikhail

    2005-01-01

    In theories with chiral couplings, one of the important consistency requirements is that of the cancellation of a gauge anomaly. In particular, this is one of the conditions imposed on the hypercharges in the standard model. However, anomaly cancellation condition of the standard model looks unnatural from the perspective of a theory with extra dimensions. Indeed, if our world were embedded into an odd-dimensional space, then the full theory would be automatically anomaly-free. In this paper ...

  2. Meconial peritonitis in a rare association of partial ileal apple-peel atresia with small abdominal wall defect

    Directory of Open Access Journals (Sweden)

    V. Insinga

    2014-06-01

    Full Text Available Intestinal atresia type III B (apple peel and gastroschisis are both congenital malformations who require early surgical correction in neonatal age. Their association is very rare. We present the case of a full term infant with partial apple peel ileal atresia and a small defect of the anterior abdominal wall, complicated by in utero intestinal perforation and subsequent meconial peritonitis. We observed a partial atresia of small intestine, with involvement of terminal ileus savings of jejunum and a large part of the proximal ileum, small anterior abdominal wall defect with herniation of few bowel loops, intestinal malrotation. Paralytic ileus and infections are the main causes of morbidity and mortality at neonatal age. In our case, in spite of the mild phenotype, prognosis has been complicated by the onset of functional bowel obstruction, caused by chemical peritonitis resulting from contact with either amniotic fluid and meconium.

  3. Anomaly poles as common signatures of chiral and conformal anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Armillis, Roberta, E-mail: roberta.armillis@le.infn.i [Dipartimento di Fisica, Universita del Salento and INFN Sezione di Lecce, Via Arnesano, 73100 Lecce (Italy); Coriano, Claudio, E-mail: claudio.coriano@le.infn.i [Dipartimento di Fisica, Universita del Salento and INFN Sezione di Lecce, Via Arnesano, 73100 Lecce (Italy); Department of Physics, University of Crete, Heraklion, Crete (Greece); Delle Rose, Luigi, E-mail: luigi.dellerose@le.infn.i [Dipartimento di Fisica, Universita del Salento and INFN Sezione di Lecce, Via Arnesano, 73100 Lecce (Italy)

    2009-12-07

    One feature of the chiral anomaly, analyzed in a perturbative framework, is the appearance of massless poles which account for it. They are identified by a spectral analysis of the anomaly graph and are usually interpreted as being of an infrared origin. Recent investigations show that their presence is not just confined in the infrared, but that they appear in the effective action under the most general kinematical conditions, even if they decouple in the infrared. Further studies reveal that they are responsible for the non-unitary behaviour of these theories in the ultraviolet (UV) region. We extend this analysis to the case of the conformal anomaly, showing that the effective action describing the interaction of gauge fields with gravity is characterized by anomaly poles that give the entire anomaly and are decoupled in the infrared (IR), in complete analogy with the chiral case. This complements a related analysis by Giannotti and Mottola on the trace anomaly in gravity, in which an anomaly pole has been identified in the corresponding correlator using dispersion theory in the IR. Our extension is based on an exact computation of the off-shell correlation function involving an energy-momentum tensor and two vector currents (the gauge-gauge-graviton vertex) which is responsible for the appearance of the anomaly.

  4. Technická analýza

    OpenAIRE

    NĚMEC, Ondřej

    2014-01-01

    Předmětem diplomové práce je technická analýza – vytvoření investičních strategií. V teoretické části jsou popsána teoretická východiska vztahující se k technické analýze a indikátorům. V praktické části je zmapována současná situace v prostředí investování na forexu – porovnání brokerů, výběr platformy apod. Vlastní řešení potom obsahuje popis investičních strategií, které byly naprogramovány v jazyku Meta Quotes Language 4 a testovány a optimalizovány pomocí genetických algoritmů v prostřed...

  5. Rare Upper Airway Anomalies.

    Science.gov (United States)

    Windsor, Alanna; Clemmens, Clarice; Jacobs, Ian N

    2016-01-01

    A broad spectrum of congenital upper airway anomalies can occur as a result of errors during embryologic development. In this review, we will describe the clinical presentation, diagnosis, and management strategies for a few select, rare congenital malformations of this system. The diagnostic tools used in workup of these disorders range from prenatal tests to radiological imaging, swallowing evaluations, indirect or direct laryngoscopy, and rigid bronchoscopy. While these congenital defects can occur in isolation, they are often associated with disorders of other organ systems or may present as part of a syndrome. Therefore workup and treatment planning for patients with these disorders often involves a team of multiple specialists, including paediatricians, otolaryngologists, pulmonologists, speech pathologists, gastroenterologists, and geneticists. PMID:26277452

  6. Survey of anal sphincter dysfunction using anal manometry in patients with fecal incontinence: a possible guide to therapy

    Science.gov (United States)

    Mandaliya, Rohan; DiMarino, Anthony J.; Moleski, Stephanie; Rattan, Satish; Cohen, Sidney

    2015-01-01

    Background Despite the surge of new medical and surgical approaches to treat fecal incontinence, the types of sphincter abnormalities in patients with incontinence have not been well characterized. We aimed to categorize anal sphincter dysfunction using anorectal manometry in patients with fecal incontinence as a potential guide for improved treatment. Methods A retrospective review of 162 consecutive patients with fecal incontinence referred for anorectal manometry was performed. Resting anal pressure and maximal squeeze pressure were considered as measures of internal anal sphincter and external anal sphincter function respectively. Results Mean age of the patients was 63 years (13-89); females (81.5%) and males (18.5%). 74% of the patients had sphincter dysfunction on anorectal manometry. Internal anal sphincter dysfunction was present in 62% patients vs. external anal sphincter dysfunction present in 44% patients. 80% females had abnormal manometry vs. 44% in males (P<0.0001). Internal anal sphincter dysfunction was present in 68% females vs. 37% in males (P=0.0026). Conclusions Overall, abnormal anorectal manometry studies revealed that internal anal sphincter dysfunction is the most common finding, alone or in combination with external anal sphincter dysfunction. We suggest that anorectal manometry may be important to delineate anal sphincter function prior to using newer therapeutic mechanical devices. Future studies using pharmacological agents to increase internal anal sphincter tone may be of clinical importance. Finally, the classification of fecal incontinence based on the type of sphincter dysfunction may be an improved guide in the selection of newer agents in treating fecal incontinence. PMID:26423466

  7. [The anal incontinence-- study on 20 operated cases].

    Science.gov (United States)

    Iusuf, T; Sârbu, V; Grasa, C; Cristache, C; Botea, F

    2001-01-01

    The authors present 20 cases operated for anal incontinence. Two techniques were performed: direct repair (18 cases) and Musset-Cottrell procedure (2 cases). The results were excellent in 12 cases, good in 5 cases and satisfactory in 3 cases. The method of choice seems to be the direct repair of the anal sphincter after a proper local and general preparation. PMID:12731180

  8. Kirurgisk behandling af anale fistler ved Crohns sygdom

    DEFF Research Database (Denmark)

    Heyckendorff-Diebold, Tina; Maeda, Yasuko; Buntzen, Steen;

    2012-01-01

    The treatment of transsphincteric anal fistulas in Crohn's disease is a balance between the elimination of the sepsis and the functional outcome. Loose setons can be used as a preoperative drainage or chronic treatment. Fibrin glue and the anal fistula plug are methods with excellent functional o...

  9. Obstetrical anal sphincter injuries and outcome of primary repair

    Directory of Open Access Journals (Sweden)

    Gauri J. Desai

    2016-10-01

    Conclusions: Nulliparity, instrumental delivery, increasing birth weight were high risk factors for obstetrical anal sphincter injuries. Obstetrician needs to be more careful while delivering a patient with multiple risk factors for OASIs. Primary suturing of anal sphincter injury with good post-operative care has a favorable outcome. [Int J Reprod Contracept Obstet Gynecol 2016; 5(10.000: 3568-3571

  10. Anal cancer and intraepithelial neoplasia screening: A review.

    Science.gov (United States)

    Leeds, Ira L; Fang, Sandy H

    2016-01-27

    This review focuses on the early diagnosis of anal cancer and its precursor lesions through routine screening. A number of risk-stratification strategies as well as screening techniques have been suggested, and currently little consensus exists among national societies. Much of the current clinical rationale for the prevention of anal cancer derives from the similar tumor biology of cervical cancer and the successful use of routine screening to identify cervical cancer and its precursors early in the disease process. It is thought that such a strategy of identifying early anal intraepithelial neoplasia will reduce the incidence of invasive anal cancer. The low prevalence of anal cancer in the general population prevents the use of routine screening. However, routine screening of selected populations has been shown to be a more promising strategy. Potential screening modalities include digital anorectal exam, anal Papanicolaou testing, human papilloma virus co-testing, and high-resolution anoscopy. Additional research associating high-grade dysplasia treatment with anal cancer prevention as well as direct comparisons of screening regimens is necessary to develop further anal cancer screening recommendations. PMID:26843912

  11. Sotsiaalvõrgustike analüüs / Innar Liiv

    Index Scriptorium Estoniae

    Liiv, Innar, 1982-

    2005-01-01

    Sotsiaalvõrgustike analüüs (social network analys - SNA) on tehnikate, meetodite ning vahendite kogum, mis aitab avastada mustreid sotsiaalsetes struktuurides. Analüüsi kasutamisest energeetikaettevõtte Enron ja kohalike ettevõtete võrgustike näitel. Skeemid

  12. Pulmonary ventilation and perfusion abnormalities and ventilation perfusion imbalance in children with pulmonary atresia or extreme tetralogy of Fallot

    International Nuclear Information System (INIS)

    Xenon-133 lung ventilation and perfusion scans were done preoperatively after cardiac catheterization and cineangiocardiography in 19 children; 6 had pulmonary atresia with an intact ventricular septum and hypoplastic right ventricle, 4 pulmonary atresia with associated complex univentricular heart, and 9 extreme Tetralogy of Fallot. The four patients with discrepancies in the sizes of the left and right pulmonary arteries on angiography had marked asymmetry of pulmonary perfusion and ventilation-perfusion imbalance on scintigraphy. Similar degrees of asymmetry and imbalance were present in 6 of the 15 children with equal-size pulmonary vessels. Asymmetry of pulmonary perfusion and ventilation-perfusion imbalance were associated with a poor prognosis

  13. Esophageal atresia and tracheoesophageal fistula: Effect of pleural cover on anastomotic dehiscence

    Directory of Open Access Journals (Sweden)

    Money Gupta

    2011-01-01

    Full Text Available Background: A significant number of esophageal atresia and tracheoesophageal fistula patients have long gaps and a high propensity to leak. Anastomotic leak in esophageal atresia is associated with a significant morbidity and mortality. Aim : In a prospective randomized trial, we analyzed the risk factors leading to anastomotic dehiscence and studied the effect of pleural wrap as an additional vascular cover around the esophageal anastomosis. Materials and Methods: Forty patients were divided into two groups A and B randomly. In 20 patients of group A, pleural wrap was utilized for covering the anastomosis and in 20 patients of group B, no such wrap was utilized. Results: Both the groups were comparable regarding age, sex, weight, gap length, tension at anastomosis and the hospital stay. The overall leak rate was 25% (10/40 in both the groups. The leak rate was not significantly different in two groups whenever a gap length was less than 2 cm or more than 3 cm. However, for a gap length of 2-3 cm, the leak rate in group A was 18% (2/11 and in group B was 50% (4/8 (P = 0.05. Thirty percent (3/10 of patients, whose anastomosis was under tension, leaked in group A as compared to 75% (6/8 in group B patients (P = 0.001. Conclusions: Use of pleural wrap was associated with less anastomotic dehiscence in patients with moderate gap esophageal atresia (2-3 cm especially when the anastomosis was under tension.

  14. Jejunoileal Atresia: Factors Affecting the Outcome and Long-term Sequelae

    OpenAIRE

    Calisti, Alessandro; Olivieri, Claudio; Coletta, Riccardo; Briganti, Vito; Oriolo, Lucia; Giannino, Giuseppina

    2012-01-01

    Context: Jejunoileal atresia (JIA) is a common abnormality. The outcome is conditioned by several variables. Nutritional problems, and long-term sequelae are described among those who survive. Aim: To correlate the type of JIA and its management to the outcome and long-term quality of life. Settings and Design: Forty-three cases over a 17-year period (1992–2009). Perinatal data, management, and outcome were extracted from the clinical notes. The cases that had survived were contacted to get i...

  15. Extrahepatic biliary atresia with choledochal cyst: Prenatal MRI predicted and post natally confirmed: A case report

    Directory of Open Access Journals (Sweden)

    Madhavi Nori

    2013-01-01

    Full Text Available Extrahepatic biliary atresia (EHBA is an uncommon cause of neonatal jaundice. Antenatal Magnetic Resonance Imaging (MRI diagnosis of EHBA has not been published to the best of our knowledge till date. EHBA with cystic component is likely to be mistaken for choledochal cyst. A case that was antenatally predicted and postnatally confirmed by surgery and histopathology is being reported. All imaging signs are analyzed herewith. Imaging helps in the prediction of EHBA and also helps in early postnatal surgical referral which in turn improves the results of Kasai′s portoenterostomy.

  16. ESOPHAGEAL ATRESIA WITH DISTAL TRACHEOESOPHAGEAL FISTULA: SURGERY TREATMENT AND A LONG TERM FOLLOW UP

    OpenAIRE

    E. Cerchia; F. Molinaro; M. Pavone; E Bindi; R. Angotti; Ferrara, F.; Messina, M.

    2012-01-01

    A study carried out at the Paediatric Surgery Units in Siena and Toulouse evaluated the long term follow-up of patients treated for esophageal atresia (EA), between 1988 and 2007. We analyzed the long term follow-up of 57 patients with III type EA. We evaluated the residual symptoms in three time intervals: in 1st years, between 2nd to 5th year and over 5th years from surgery. The considered parameters were: feeding difficulties, respiratory problems, gastro-esophageal reflux, growth impairme...

  17. Pulmonary atresia with intact ventricular septum and hypoplastic right ventricle in an Arabian foal.

    Science.gov (United States)

    Krüger, M U; Wünschmann, A; Ward, C; Stauthammer, C D

    2016-09-01

    Pulmonary atresia with intact ventricular septum, rudimentary tricuspid valve, hypoplastic right ventricle, and right-to-left atrial shunting were identified in a four-day-old, male Arabian foal with clinical signs of cyanotic heart disease. Pulmonary blood flow was apparently derived from a ductus arteriosus. Echocardiographic evaluation revealed the majority of cardiac abnormalities and also findings compatible with right-sided congestive heart failure. Congenital cardiac defects have a high incidence in this breed, and this is the first description of this combination of congenital cardiac defects. PMID:27283083

  18. Extrahepatic biliary atresia with choledochal cyst: Prenatal MRI predicted and post natally confirmed: A case report.

    Science.gov (United States)

    Nori, Madhavi; Venkateshwarlu, J; Vijaysekhar; Prasad, G Raghavendra

    2013-07-01

    Extrahepatic biliary atresia (EHBA) is an uncommon cause of neonatal jaundice. Antenatal Magnetic Resonance Imaging (MRI) diagnosis of EHBA has not been published to the best of our knowledge till date. EHBA with cystic component is likely to be mistaken for choledochal cyst. A case that was antenatally predicted and postnatally confirmed by surgery and histopathology is being reported. All imaging signs are analyzed herewith. Imaging helps in the prediction of EHBA and also helps in early postnatal surgical referral which in turn improves the results of Kasai's portoenterostomy. PMID:24347854

  19. Assessing portal hypertension in post-operative biliary atresia patients using 2D magnetic resonance angiography

    Energy Technology Data Exchange (ETDEWEB)

    Hiki, Saori; Horikoshi, Kentarou; Kobayashi, Hiroyuki; Yamataka, Atsuyuki; Miyano, Takeshi; Kuwatsuru, Ryouhei; Katayama, Hitoshi [Juntendo Univ., Tokyo (Japan). School of Medicine

    2000-01-01

    2D magnetic resonance angiography (MRA) was performed in 38 post-operative biliary atresia (BA) patients. Collateral circulation other than esophageal varices that could not be observed with endoscopy was detected. By using contrast, the portal vasculature could be clearly delineated. MRA can be performed without using general anesthesia obviating the nead for hospitalization. At present, MRA alone is not sufficient for the complete assessment of varices in post-operative BA patients, and mucosal changes cannot be detected without endoscopy. However, combined with endoscopy, it provides valuable additional follow-up information without the need for general anesthesia or hospitalization. (author)

  20. Surgical treatment of congenital esophageal atresia in neonates%新生儿食管闭锁的外科治疗

    Institute of Scientific and Technical Information of China (English)

    潘征夏; 吴春; 李洪波; 王刚; 李勇刚; 代江涛; 安永; 杨杰先

    2011-01-01

    目的 总结新生儿食管闭锁的诊断和治疗经验.方法 回顾性分析我院2002年6月至2010年6月收治的新生儿食管闭锁61例,男34例,女27例.手术年龄18 h~7 d,平均(2.5±0.6)d,体重1 500~4 000 g,其中低体重儿(<2 500g)16例.61例中按Gross病理解剖分类:Ⅰ型2例,Ⅲa型32例,Ⅲb型27例,Ⅲa型中有1例远端食管有局限狭窄,开口约0.2 cm;合并畸形:先天性心脏病15例,肠道畸形4例,泌尿系畸形3例.结果 61例中2例Ⅰ型食管闭锁先行近端食管引流、胃造瘘,2周后行结肠代食管手术,59例Ⅲ型均Ⅰ期食管气管瘘切断缝扎、食管端端吻合术,Ⅲa型中远端食管有1例局限性狭窄,行纵切横缝解除狭窄.术后并发单侧或双侧严重肺炎42例,硬肿症3例,近期吻合口狭窄24例,吻合口瘘3例.除1例术后2d合并肠穿孔死亡,3例放弃治疗(均为早期病例:1例为术后1周出现核黄疸,2例术后合并严重肺部感染不能脱离呼吸机),余57例均痊愈出院.术后随访3个月~8年,轻度胃食管反流3例,余均进食良好,生长发育正常.结论 尽早诊断、及时手术,积极预防和治疗并发症,新生儿食管闭锁可取得良好的效果.%Objective To summarize the diagnosis and treatment for congenital esophageal atresia (CEA) in neonates. Methods From June 2002 to June 2010, 61 neonates with congenital esophageal atresia underwent surgery at this center. Of these patients, 34 were boys and 27 were girls. Their age ranged from 18 hours to 7 days (mean, 2. 5 ±0. 6 days). Their weight ranged from 1500 grams to 4000 grams. Sixteen patients were very low-birth-weight infants (<1500 g). According to the anomalies of CEA, 2 were diagnosed with type Ⅰ CEA, and the other 59 were type Ⅲ CEA (32 type Ⅲa and 27 type Ⅲb). The most common associated anomalies were cardiac anomalies (16, 26%), followed by intestinal anomalies (5, 8%) and renal anomalies (3, 5%). Results The 2 cases with type Ⅰ CEA underwent

  1. Seismic data fusion anomaly detection

    Science.gov (United States)

    Harrity, Kyle; Blasch, Erik; Alford, Mark; Ezekiel, Soundararajan; Ferris, David

    2014-06-01

    Detecting anomalies in non-stationary signals has valuable applications in many fields including medicine and meteorology. These include uses such as identifying possible heart conditions from an Electrocardiography (ECG) signals or predicting earthquakes via seismographic data. Over the many choices of anomaly detection algorithms, it is important to compare possible methods. In this paper, we examine and compare two approaches to anomaly detection and see how data fusion methods may improve performance. The first approach involves using an artificial neural network (ANN) to detect anomalies in a wavelet de-noised signal. The other method uses a perspective neural network (PNN) to analyze an arbitrary number of "perspectives" or transformations of the observed signal for anomalies. Possible perspectives may include wavelet de-noising, Fourier transform, peak-filtering, etc.. In order to evaluate these techniques via signal fusion metrics, we must apply signal preprocessing techniques such as de-noising methods to the original signal and then use a neural network to find anomalies in the generated signal. From this secondary result it is possible to use data fusion techniques that can be evaluated via existing data fusion metrics for single and multiple perspectives. The result will show which anomaly detection method, according to the metrics, is better suited overall for anomaly detection applications. The method used in this study could be applied to compare other signal processing algorithms.

  2. Trace Anomaly in Geometric Discretization

    OpenAIRE

    Czech, Bartlomiej

    2007-01-01

    I develop the simplest geometric-discretized analogue of two dimensional scalar field theory, which qualitatively reproduces the trace anomaly of the continuous theory. The discrete analogue provides an interpretation of the trace anomaly in terms of a non-trivial transformation of electric-magnetic duality-invariant modes of resistor networks that accommodate both electric and magnetic charge currents.

  3. Algebraic study of chiral anomalies

    Indian Academy of Sciences (India)

    Juan Mañes; Raymond Stora; Bruno Zumino

    2012-06-01

    The algebraic structure of chiral anomalies is made globally valid on non-trivial bundles by the introduction of a fixed background connection. Some of the techniques used in the study of the anomaly are improved or generalized, including a systematic way of generating towers of ‘descent equations’.

  4. Anomaly mediation deformed by axion

    International Nuclear Information System (INIS)

    We show that in supersymmetric axion models the axion supermultiplet obtains a sizable F-term due to a non-supersymmetric dynamics and it generally gives the gaugino masses comparable to the anomaly mediation contribution. Thus the gaugino mass relation predicted by the anomaly mediation effect can be significantly modified in the presence of axion to solve the strong CP problem

  5. Chronic anal fissure: new approaches to chemical sphincterotomy

    Directory of Open Access Journals (Sweden)

    Mohammad hassan Emami

    2008-06-01

    Full Text Available

    • An anal fissure is a split in the mucosa extending from the anal verge towards the dentate line. It currently affects 10% of patients attending proctology clinics. Recent studies have highlighted the role of increased internal anal sphincter pressure and decreased anodermal blood flow in the pathogenesis of chronic anal fissures. Acute fissures usually heal with conservative management. Fissures lasting greater than two months with features of chronicity, are unlikely to heal with conservative management. Lateral internal sphincterotomy has been the treatment of choice for chronic anal fissures. Because of the disability associated with surgery for healing anal fissure and the risk of incontinence, medical alternatives for surgery have been sought. Among different chemical agents, Glyceryl trinitrate (GTN has been shown to be the first line treatment for chronic anal fissure but the transient sphincteric relaxation effect of pharmacologic agents such as GTN, makes them less effective than surgery. Although we have different forms of GTN products, they do not show a long acting effect on relaxing and enhancing the perfusion of anal sphincter, because they are soon metabolized. Most difficulties with current usage of drugs are due to poor compliance of patients. It can be taken into consideration that new formulation and novel combination of GTN with other treatments, in slow releasing forms may lead to acceptable strategies in the management of chronic anal fissure. As authors’ experience in this field, other clinical trials on the drug combination and slow releasing formulations are warranted to generate new data on the subject.
    • Key word: chronic anal fissure, review, chemical sphincterotomy 

  6. Anomaly Poles as Common Signatures of Chiral and Conformal Anomalies

    CERN Document Server

    Armillis, Roberta; Rose, Luigi Delle

    2009-01-01

    One feature of the chiral anomaly, analyzed in a perturbative framework, is the appearance of massless poles which account for it. They are identified by a spectral analysis of the anomaly graph and are usually interpreted as being of an infrared origin. Recent investigations shown that their presence is not just confined in the infrared, but that they appear in the effective action under the most general kinematical conditions, even if they decouple in the infrared. Further studies reveal that they are responsible for the non-unitary behaviour of these theories in the ultraviolet (UV) region. We extend this analysis to the case of the conformal anomaly, showing that the effective action describing the interaction of gauge fields with gravity is characterized by anomaly poles that give the entire anomaly and are decoupled in the infrared (IR), in complete analogy with the chiral case. This complements a related analysis by Giannotti and Mottola on the trace anomaly in gravity, in which an anomaly pole has bee...

  7. Trends in incidence of anal cancer and high-grade anal intraepithelial neoplasia in Denmark, 1978-2008

    DEFF Research Database (Denmark)

    Nielsen, Ann; Plum, Christian Edinger Munk; Kjaer, Susanne K

    2012-01-01

    The aim of the study was to determine the incidences of anal cancer and high-grade anal intraepithelial neoplasia (AIN2/3) over time in Danish women and men. Describing the burden of anal cancer and AIN may be valuable in future evaluations of the human papillomavirus (HPV) vaccine. We included all......-associated histological types levelled out or even declined during the 30 years of observation. In women, the increase in HPV-associated cancers was more pronounced among those under 60 years of age. Our findings indicate that vaccines against HPV might play an important role in the prevention of anal cancer and its...... cases of AIN2/3 during the study period. The average annual percentage change of 5% between 1998 and 2008 represents a steep increase in the incidence of AIN in both genders. Furthermore, the incidence rate of HPV-associated anal cancers increased significantly, whereas that of non-HPV...

  8. Respiratory Distress Secondary to Rhabdomyosarcoma of the Tongue and Co-existent Choanal Atresia.

    Science.gov (United States)

    Chatopadhayay, Rahul; Tiwari, Preeti; Gangopadhyay, A N; Pandey, Vaibhav

    2016-07-01

    Whilst rhabdomyosarcoma (RMS) is the third most common solid tumour in children, congenital RMS of the tongue is extremely rare and usually present as painless progressive mass since birth (Dagher and Helman in Oncologist 4:34-44, 1999; Childs and Goudy in Int J Pediatr Otorhinolaryngol 5:126-128, 2010). In neonates, presentation with respiratory distress is unexpected as neonates are preferential nasal breathers and restricted oral breathing due to tumour usually poses no problem. We herein report a case of rhabdomyosarcoma of the tongue with co-existent unilateral choanal atresia, presenting with respiratory distress. The baby developed upper respiratory tract infection following which developed severe respiratory distress. Airway symptoms were precipitated as there was combined obstruction of both the nostrils due to infection or adenoid enlargement and unilateral chonal atresia. Treatment of respiratory distress in the presence of RMS and bilateral nasal pathology must first prioritise the security of the airway, before taking a multi-factorial approach to the therapy of the lingual mass (Childs and Goudy in Int J Pediatr Otorhinolaryngol 5:126-128, 2010). This case illustrates the importance of vigilance with respect to co-existent nasal pathology, in order to avoid the occurrence of complete airway obstruction. We therefore feel that any diagnosis of lingual RMS should warrant a formal examination of both nasal cavities. PMID:27408448

  9. Treatment of colon conduit redundancy in a child with esophageal atresia.

    Science.gov (United States)

    Glasser, James G; Reddy, P Prithvi; Adkins, E Stanton

    2006-03-01

    We recently revised a redundant colon conduit in a boy who was born with isolated esophageal atresia. In view of the paucity of reports dealing with correction of this common complication of esophageal replacement, it seemed appropriate to report our experience. Because of effective medical therapy of acid peptic disease, patients who formerly required conduit replacement may now be candidates for revision; however, the medical literature does not specify when conduit revision, as opposed to conduit replacement, is indicated; also, no guidance is provided regarding what constitutes effective operative revision. Innovative techniques that stretch and elongate the atretic esophagus will likely lessen the use of conduits in esophageal atresia; nevertheless, colon conduits are useful in many other clinical situations and will remain an essential part of the armamentarium of pediatric, general, and thoracic surgeons. This report highlights the DeMeester and Tannuri technique, whereby a colon conduit is prepared like a Roux limb. The mesentery is divided only once; the conduit's blood supply is not severed from the distal mesocolon. This innovation improves a conduit's blood supply and lessens its attendant complications. Lastly, we describe a muscle splitting, posterolateral thoracotomy technique that is simpler than the alternatives and is useful in a variety of clinical situations. PMID:16553129

  10. Tumour necrosis factor-alpha gene polymorphisms in Iranian patients with biliary atresia

    Directory of Open Access Journals (Sweden)

    Nikou Fotouhi

    2014-01-01

    Full Text Available Background: Biliary atresia (BA is a progressive inflammatory destructive process of the bile ducts. This study evaluated the relationship between single-nucleotide polymorphisms in the promoter region of tumour necrosis factor-alpha (TNF-α gene and bilaiary atresia. Materials and Methods: Genomic deoxyribonucleic acid from 16 patients with established diagnosis of BA and 36 patients with INC was obtained. The genotypes of TNF-α-1031 (T/C and TNF-α-308 (G/A were determined using the restriction fragment length polymorphism-polymerase chain reaction and the results were analysis with proper statistic software. Results: The frequencies of T/T, T/C in TNF-α-1031 and G/G, G/A in TNF-α-308 were as same as control group. Moreover, we have same deduction for allele frequency and haplotypes analysis (T allele: 84.37%; G allele: 87.5% in BA patients (T allele: 80.56%; G allele: 86.11% in controls. In all cases variants of polymorphism did not affect the severity or incidence of BA disease. Conclusion: although no significant associations were found between BA and control groups, it seems meaningful that since the nature of BA is multi factorial. Next step will be considering a new target such as downstream modulation of the TNF-α pathway or other cytokines and chemokines which act directly/indirectly.

  11. The contribution of fetal MR imaging to the assessment of oesophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Hochart, V.; Verpillat, P.; Bigot, J.; Avni, F.E. [CHRU Lille, Jeanne de Flandre Hospital, Department of Pediatric Radiology, Lille (France); Langlois, C. [EA2694 USDL CHRU Lille, Department of Biostatistics, Lille (France); Garabedian, C.; Debarge, V.H. [CHRU Lille, Jeanne de Flandre Hospital, Department of Obstetrics and Gynecology, Lille (France); Sfeir, R. [CHRU Lille, Jeanne de Flandre Hospital, Department of Pediatric Surgery, Reference Center for Congenital Anomalies of the Esophagus, Lille (France)

    2014-10-11

    Present challenges are to improve the diagnosis rate of oesophageal atresia (OA) and evaluate as completely as possible a fetus affected by OA, specifically the type of OA and the length of the gap. Our aim was to evaluate the accuracy of fetal MR imaging (fMRI) for diagnosis of OA. We reviewed fMRI performed because of sonographic suspicion of an OA. The signs reviewed included stomach size, ''pouch sign'', bowing of the trachea and visualization of the lower oesophageal lumen. The fetuses were assigned by consensus as having or not having EA, as well as having a tracheaoesophageal fistula (TOF). All findings were correlated with postnatal data. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated. Se, Sp, PPV and NPV of the technique were respectively 91 %, 100 %, 100 % and 88 %. The presence of the pouch sign yielded corresponding values of 82 %, 100 %, 100 % and 78 %. Mid-tracheal bowing was correlated positively with EA. The type of atresia was correctly evaluated in 90 % of patients. fMRI is useful for the diagnosis of EA through the visualization of the oesophageal pouch or through associated signs such as tracheal bowing. Visualization of the lower oesophageal lumen seems to be a good sign of TEF. (orig.)

  12. The contribution of fetal MR imaging to the assessment of oesophageal atresia

    International Nuclear Information System (INIS)

    Present challenges are to improve the diagnosis rate of oesophageal atresia (OA) and evaluate as completely as possible a fetus affected by OA, specifically the type of OA and the length of the gap. Our aim was to evaluate the accuracy of fetal MR imaging (fMRI) for diagnosis of OA. We reviewed fMRI performed because of sonographic suspicion of an OA. The signs reviewed included stomach size, ''pouch sign'', bowing of the trachea and visualization of the lower oesophageal lumen. The fetuses were assigned by consensus as having or not having EA, as well as having a tracheaoesophageal fistula (TOF). All findings were correlated with postnatal data. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated. Se, Sp, PPV and NPV of the technique were respectively 91 %, 100 %, 100 % and 88 %. The presence of the pouch sign yielded corresponding values of 82 %, 100 %, 100 % and 78 %. Mid-tracheal bowing was correlated positively with EA. The type of atresia was correctly evaluated in 90 % of patients. fMRI is useful for the diagnosis of EA through the visualization of the oesophageal pouch or through associated signs such as tracheal bowing. Visualization of the lower oesophageal lumen seems to be a good sign of TEF. (orig.)

  13. Karyotype analysis of a male exhibiting Meckel's diverticulum and aural atresia

    Energy Technology Data Exchange (ETDEWEB)

    Frizzell, B.; Hicks, M.F. (David Lipscomb Univ., Nashville, TN (United States))

    Patau's Syndrome is caused by inheritance of an extra chromosome 13. It is characterized primarily by severe mental retardation, cleft palate, and retarded growth. Most fetuses expressing Patau's Syndrome spontaneously abort, and those that are born usually die before one year. Both Meckel's diverticulum and aural atresia are defects found in patients with Patau's at levels higher than those in the general population. An otherwise asymptomatic male expressing only Meckel's diverticulum and aural atresia has a female sibling whose son expressed Patau's syndrome. Twenty percent of patients with Patau's show a translocation of part of chromosome 13 to another D chromosome. If a translocation were the cause of the expression of Patau's in this family, it is possible that the normal male inherited a balanced translocation and the Patau's male received an unbalanced translocation. A karyotype analysis of the non-Patau's male was done to determine if such a translocation were present.

  14. Anaesthetic Management for Cataract Surgery in VACTERL Syndrome Case Report

    Directory of Open Access Journals (Sweden)

    Sonal S Khatavkar

    2009-01-01

    Full Text Available Eight year old girl, weighing 14 kg with VACTERL syndrome V: Vertebral anomalies, A: Anal malformation, C: Cardiovascular defect, TE: Tracheal and esophageal malformation, R:Renal agenesis, L: Limb anomalies., underwent cataract surgery under general anaesthesia. She had multiple congenital anomalies like esophageal atresia, imperfo-rate anus (corrected, single kidney& radial aplasia. Anticipating problems of gastro-esophageal reflux& chronic renal failure, successful management was done.

  15. Binning of satellite magnetic anomalies

    Science.gov (United States)

    Goyal, H. K.; Vonfrese, R. R. B.; Hinze, W. J.

    1985-01-01

    Crustal magnetic anomaly signals over satellite orbits were simulated to investigate numerical averaging as an anomaly estimator. Averaging as an anomaly estimator involves significant problems concerning spatial and amplitude smoothing of the satellite magnetic observations. The results of simulations suggest that the error of numerical averaging constitutes a small and relatively minor component of the total error-budget of higher orbital anomaly estimates, whereas for lower orbital estimates numerical averaging error increases substantially. As an alternative to numerical averaging, least-squares collocation was investigated and observed to produce substantially more accurate anomaly estimates, particularly as the orbital elevation of prediction was decreased towards the crustal sources. In contrast to averaging, collocation is a significantly more resource-intensive procedure to apply because of the practical, but surmountable problems related to establishing and inverting the covariance matrix for accurate anomaly prediction. However, collocation may be much more effectively used to exploit the anomaly details contained in the lower orbital satellite magnetic data for geologic analysis.

  16. Analýza vybrané firmy

    OpenAIRE

    Žahourek, Martin

    2010-01-01

    V dnešní nelehké situaci musí organizace obzvlášť pečlivě sledovat svoje hospodaření a plánovat. Cílem této práce je zhodnotit aktuální situace společnosti Centropen a.s.. Použitými nástroji jsou finanční analýza, fundamentální analýza, SLEPTE analýza a Porterova analýza pěti sil. Výsledky těchto analýz jsou dále zpracovány ve SWOT analýza, ta je potom porovnána se SWOT analýzou k situaci z roku 2006. Today, in the hard situation, must organization very carefully observe its economy and pl...

  17. Chylothorax after Primary Repair of Esophageal Atresia with Tracheo-esophageal Fistula: Successful Management by Biological Fibrin Glue

    Directory of Open Access Journals (Sweden)

    Anjan K Dhua

    2012-08-01

    Full Text Available A neonate, who had undergone primary repair of esophageal atresia with trachea-esophageal fistula, developed right pleural effusion in the postoperative period. It was initially misdiagnosed as an anastomotic leak, but later confirmed to be chylothorax. Conservative treatment failed. Application of biological fibrin glue (sealant on the mediastinum through a thoracotomy was curative.

  18. A two-center comparative study of gastric pull-up and jejunal interposition for long gap esophageal atresia

    NARCIS (Netherlands)

    Gallo, Gabriele; Zwaveling, Sander; Van der Zee, David C.; Bax, Klaas N.; de Langen, Zacharias J.; Hulscher, Jan B. F.

    2015-01-01

    Purpose: When restoration of the anatomical continuity in case of long gap esophageal atresia (LGEA) is not feasible, esophageal replacement surgery becomes mandatory. The aim of this paper is to critically compare the experience of two tertiary referral centers in The Netherlands performing either

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

    Science.gov (United States)

    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.

  20. Atresia duodenal asociada a invaginación intestinal secundaria a divertículo de meckel en un lactante de tres meses

    OpenAIRE

    Rubén Martín Alvarez-Solís; Fredy Chablé-Montero; Marcela Vargas-Vallejo; David Bulnes Mendizábal; Armando Quero-Hernández; José Cabrera-Aguirre

    2006-01-01

    La asociación entre atresia duodenal e invaginación intestinal no se ha reportado en la literatura. Presentamos las características clínicas y quirúrgicas de un paciente masculino de tres meses de edad que presenta atresia duodenal asociada a invaginación intestinal secundaria a un divertículo de Meckel perforado e invertido. Dentro de las causas de vómitos en lactantes se incluyen las atresias intestinales. Aunque es raro se han reportado casos en otras edades pediátricas. La invaginación in...

  1. Spatial distribution patterns of anorectal atresia/stenosis in China: Use of two-dimensional graph-theoretical clustering

    Institute of Scientific and Technical Information of China (English)

    Ping Yuan; Liang Qiao; Li Dai; Yan-Ping Wang; Guang-Xuan Zhou; Ying Han; Xiao-Xia Liu; Xun Zhang; Yi Cao; Juan Liang; Jun Zhu

    2009-01-01

    AIM:To investigate the spatial distribution patterns of anorectal atresia/stenosis in China.METHODS:Data were collected from the Chinese Birth Defects Monitoring Network (CBDMN),a hospitalbased congenital malformations registry system.All fetuses more than 28 wk of gestation and neonates up to 7 d of age in hospitals within the monitoring sites of the CBDMN were monitored from 2001 to 2005.Two-dimensional graph-theoretical clustering was used to divide monitoring sites of the CBDMN into different clusters according to the average incidences of anorectal atresia/stenosis in the different monitoring sites.RESULTS:The overall average incidence of anorectal atresia/stenosis in China was 3.17 per 10 000 from 2001 to 2005.The areas with the highest average incidences of anorectal atresia/stenosis were almost always focused in Eastern China.The monitoring sites were grouped into 6 clusters of areas.Cluster 1 comprised the monitoring sites in Heilongjiang Province,Jilin Province,and Liaoning Province;Cluster 2 was composed of those in Fujian Province,Guangdong Province,Hainan Province,Guangxi Zhuang Autonomous Region,south Hunan Province,and south Jiangxi Province;Cluster 3 consisted of those in Beijing Municipal City,Tianjin Municipal City,Hebei Province,Shandong Province,north Jiangsu Province,and north Anhui Province;Cluster 4 was made up of those in Zhejiang Province,Shanghai Municipal City,south Anhui Province,south Jiangsu Province,north Hunan Province,north Jiangxi Province,Hubei Province,Henan Province,Shanxi Province and Inner Mongolia Autonomous Region;Cluster 5 consisted of those in Ningxia Hui Autonomous Region,Gansu Province and Qinghai Province;and Cluster 6 included those in Shaanxi Province,Sichuan Province,Chongqing Municipal City,Yunnan Province,Guizhou Province,Xinjiang Uygur Autonomous Province and Tibet Autonomous Region.CONCLUSION:The findings in this research allow the display of the spatial distribution patterns of anorectal atresia/stenosis in

  2. Anal transition zone in the surgical management of ulcerative colitis

    Institute of Scientific and Technical Information of China (English)

    Jennifer Holder-Murray; Alessandro Fichera

    2009-01-01

    Preservation of the anal transition zone has long been a significant source of controversy in the surgical management of ulcerative colitis. The two techniques for restorative proctocolectomy and ileal pouch anal anastomosis (RPC IPAA) in common practice are a stapled anastomosis and a handsewn anastomosis;these techniques differ in the amount of remaining rectal mucosa and therefore the presence of the anal transition zone following surgery. Each technique has advantages and disadvantages in long-term functional outcomes, operative and postoperative complications, and risk of neoplasia. Therefore, we propose a selective approach to performing a stapled RPC IPAA based on the presence of dysplasia in the preoperative endoscopic evaluation.

  3. Metachronous tubulovillous and tubular adenomas of the anal canal

    OpenAIRE

    NOZAWA, HIROAKI; ISHIHARA, SOICHIRO; Morikawa, Teppei; Tanaka, Junichiro; YASUDA, KOJI; Ohtani, Kensuke; Nishikawa, Takeshi; Tanaka, Toshiaki; Kiyomatsu, Tomomichi; Kawai, Kazushige; Hata, Keisuke; Kazama, Shinsuke; Yamaguchi, Hironori; Sunami, Eiji; Kitayama, Joji

    2015-01-01

    Anal canal adenoma is an extremely rare disease that has the potential to transform into a malignant tumor. We herein presented a rare case of metachronous multiple adenomas of the anal canal. A 48-year-old woman underwent total colonoscopy following a positive fecal blood test. A 9-mm villous polyp arising from the posterior wall of the anal canal was removed by snare polypectomy. Histologically, the tumor was tubulovillous adenoma with high-grade dysplasia and the cut end was negative for t...

  4. DO CHANGES IN ANAL SPHINCTER ANATOMY CORRELATE WITH ANAL FUNCTION IN WOMEN WITH A HISTORY OF VAGINAL DELIVERY?

    Directory of Open Access Journals (Sweden)

    Sthela Maria MURAD-REGADAS

    2014-09-01

    Full Text Available Objectives To evaluate anal sphincter anatomy using three-dimensional ultrasonography (3-DAUS in incontinent women with vaginal delivery, correlate anatomical findings with symptoms of fecal incontinence and determine the effect of vaginal delivery on anal canal anatomy and function. Methods Female with fecal incontinence and vaginal delivery were assessed with Wexner’s score, manometry, and 3DAUS. A control group comprising asymptomatic nulliparous was included. Anal pressure, the angle of the defect and length of the external anal sphincter (EAS, the anterior and posterior internal anal sphincter (IAS, the EAS + puborectal and the gap were measured and correlated with score. Results Of the 62, 49 had fecal incontinence and 13 were asymptomatic. Twenty five had EAS defects, 8 had combined EAS+IAS defects, 16 had intact sphincters and continence scores were similar. Subjects with sphincter defects had a shorter anterior EAS, IAS and longer gap than women without defects. Those with a vaginal delivery and intact sphincters had a shorter anterior EAS and longer gap than nulliparous. We found correlations between resting pressure and anterior EAS and IAS length in patients with defects. Conclusions Avaliar a anatomia do esfíncter anal usando ultra-sonografia tridimensional (3D-US em mulheres incontinentes com parto vaginal, correlacionar os achados anatômicos com sintomas de incontinência fecal e, determinar o efeito do parto vaginal sobre a anatomia e função do canal anal.

  5. 脊柱肛门肾脏气管桡骨联合综合征3例报告

    Institute of Scientific and Technical Information of China (English)

    张其海

    2000-01-01

    @@ 脊柱肛门肾脏气管桡骨联合综合征(Vertebral defect,anal atresia,tracheo-esophageal fistula,renal anomalies,radial dysplasia combinative syndrom,VATER综合征)是小儿罕见畸形,本院10余年来收治3例,现报告如下.

  6. The classification and staging of cancerous growths of the anal canal

    International Nuclear Information System (INIS)

    In this chapter authors give information about frequency of cancerous growths of the anal canal, general analysis of observations the classification and staging of cancerous growths of the anal canal, clinical-anatomy classification of cancerous growths of the anal canal and staging of cancerous growths of anal canal

  7. MRI in evaluating atrophy of the external anal sphincter in patients with fecal incontinence

    NARCIS (Netherlands)

    M.P. Terra; R.G.H. Beets-Tan; V.P.M. van der Hulst; M. Deutekom; M.G.W. Dijkgraaf; P.M.M. Bossuyt; A.C. Dobben; C.G.M.I. Baeten; J. Stoker

    2006-01-01

    OBJECTIVE. External anal sphincter atrophy seen at endoanal MRI may predict poor outcome of surgical anal sphincter repair for an external anal sphincter defect. The purposes of this study were to compare external phased-array MRI to endoanal MRI for depicting external anal sphincter atrophy in pati

  8.  Surgical excision of extensive anal condylomata is a safe operation without risk of anal stenosis

    Directory of Open Access Journals (Sweden)

    Konrad Wroński

    2012-03-01

    Full Text Available  Introduction:Anal condylomata acuminata was a well-known disease in ancient times but in recent years there has been a rapidly increasing number of people who suffer from this disease. The main cause of this disease is infection of human papilloma virus (HPV which occurs through sexual contact.Currently there are three different ways to treat anal condylomata. Small changes of anal condylomata can be treated with local therapeutic agents, but the best results of treatment of extensive changes are obtained by surgical techniques.Material/Methods:The study group consisted of 30 patients with diagnosed extensive anal condylomata who underwent surgery in Mikolaj Pirogow High Specialized Hospital in Lodz. The survey was conducted from 2007 to 2011. Patients had been directed to the surgical ward by general surgeons and practitioners, proctologists and urologists. The diagnosis was made after proctological assessment in the knee-chest position.Results:All patients underwent surgery and had complete macroscopic electroexcision of anal condylomata. In the research group there was no mortality. Postoperative complications occurred in 4 (13.3�20patients – postoperative bleeding. Strong pain was present in 14 (46.7�20patients but only in the postoperative period. During postoperative follow-up there was no observed infection in the anal region or recurrence of disease. In the operated group there were no observed cosmetic deformations of the anus and/or the anal canal, narrow anal canal or functional fecal incontinence symptoms.Conclusions:Surgical treatment of anal condylomata is an effective and safe method for the patient. In our research there were no serious postoperative complications or recurrence of the disease during the follow-up period.

  9. ALP hints from cooling anomalies

    CERN Document Server

    Giannotti, Maurizio

    2015-01-01

    We review the current status of the anomalies in stellar cooling and argue that, among the new physics candidates, an axion-like particle would represent the best option to account for the hinted additional cooling.

  10. Notes on Anomaly Induced Transport

    CERN Document Server

    Landsteiner, Karl

    2016-01-01

    Chiral anomalies give rise to dissipationless transport phenomena such as the chiral magnetic and vortical effects. In these notes I review the theory from a quantum field theoretic, hydrodynamic and holographic perspective. A physical interpretation of the otherwise somewhat obscure concepts of consistent and covariant anomalies will be given. Vanishing of the CME in strict equilibrium will be connected to the boundary conditions in momentum space imposed by the regularization. The role of the gravitational anomaly will be explained. That it contributes to transport in an unexpectedly low order in the derivative expansion can be easiest understood via holography. Anomalous transport is supposed to play also a key role in understanding the electronics of advanced materials, the Dirac- and Weyl (semi)metals. Anomaly related phenomena such as negative magnetoresistivity, anomalous Hall effect, thermal anomalous Hall effect and Fermi arcs can be understood via anomalous transport. Finally I briefly review a holo...

  11. On renormalization of axial anomaly

    International Nuclear Information System (INIS)

    It is shown that multiplicative renormalization of the axial singlet current results in renormalization of the axial anomaly in all orders of perturbation theory. It is a necessary condition for the Adler - Bardeen theorem being valid. 10 refs.; 2 figs

  12. Gravitational anomaly and transport phenomena

    OpenAIRE

    Landsteiner, Karl; Megías Fernández, Eugenio; Pena-Benítez, Francisco

    2011-01-01

    Quantum anomalies give rise to new transport phenomena. In particular, a magnetic field can induce an anomalous current via the chiral magnetic effect and a vortex in the relativistic fluid can also induce a current via the chiral vortical effect. The related transport coefficients can be calculated via Kubo formulas. We evaluate the Kubo formula for the anomalous vortical conductivity at weak coupling and show that it receives contributions proportional to the gravitational anomaly coefficie...

  13. Satellite elevation magnetic anomaly maps

    Science.gov (United States)

    Braile, L. W.; Hinze, W. J. (Principal Investigator)

    1982-01-01

    The problem of inverting 2 deg average MAGSAT scalar anomalies for the region 80 W, 60 E longitude and 40 S, 70 N latitude was attempted on the LARS computer; however, the effort was aborted due to insufficient allocation of CPU-time. This problem is currently being resubmitted and should be implemented shortly for quantitative comparison with free-air gravity anomaly, geothermal, and tectonic data.

  14. Boundary Anomalies and Correlation Functions

    OpenAIRE

    Huang, Kuo-Wei(C.N. Yang Institute for Theoretical Physics, Department of Physics and Astronomy, Stony Brook University, Stony Brook, NY, 11794, U.S.A.)

    2016-01-01

    It was shown recently that boundary terms of conformal anomalies recover the universal contribution to the entanglement entropy and also play an important role in the boundary monotonicity theorem of odd-dimensional quantum field theories. Motivated by these results, we investigate relationships between boundary anomalies and the stress tensor correlation functions in conformal field theories. In particular, we focus on how the conformal Ward identity and the renormalization group equation ar...

  15. Situs anomalies on prenatal MRI

    International Nuclear Information System (INIS)

    Objective: Situs anomalies refer to an abnormal organ arrangement, which may be associated with severe errors of development. Due regard being given to prenatal magnetic resonance imaging (MRI) as an adjunct to ultrasonography (US), this study sought to demonstrate the in utero visualization of situs anomalies on MRI, compared to US. Materials and methods: This retrospective study included 12 fetuses with situs anomalies depicted on fetal MRI using prenatal US as a comparison modality. With an MRI standard protocol, the whole fetus was assessed for anomalies, with regard to the position and morphology of the following structures: heart; venous drainage and aorta; stomach and intestines; liver and gallbladder; and the presence and number of spleens. Results: Situs inversus totalis was found in 3/12 fetuses; situs inversus with levocardia in 1/12 fetuses; situs inversus abdominis in 2/12 fetuses; situs ambiguous with polysplenia in 3/12 fetuses, and with asplenia in 2/12 fetuses; and isolated dextrocardia in 1/12 fetuses. Congenital heart defects (CHDs), vascular anomalies, and intestinal malrotations were the most frequent associated malformations. In 5/12 cases, the US and MRI diagnoses were concordant. Compared to US, in 7/12 cases, additional MRI findings specified the situs anomaly, but CHDs were only partially visualized in six cases. Conclusions: Our initial MRI results demonstrate the visualization of situs anomalies and associated malformations in utero, which may provide important information for perinatal management. Using a standard protocol, MRI may identify additional findings, compared to US, which confirm and specify the situs anomaly, but, with limited MRI visualization of fetal CHDs.

  16. Space weather and space anomalies

    Directory of Open Access Journals (Sweden)

    L. I. Dorman

    2005-11-01

    Full Text Available A large database of anomalies, registered by 220 satellites in different orbits over the period 1971-1994 has been compiled. For the first time, data from 49 Russian Kosmos satellites have been included in a statistical analysis. The database also contains a large set of daily and hourly space weather parameters. A series of statistical analyses made it possible to quantify, for different satellite orbits, space weather conditions on the days characterized by anomaly occurrences. In particular, very intense fluxes (>1000 pfu at energy >10 MeV of solar protons are linked to anomalies registered by satellites in high-altitude (>15000 km, near-polar (inclination >55° orbits typical for navigation satellites, such as those used in the GPS network, NAVSTAR, etc. (the rate of anomalies increases by a factor ~20, and to a much smaller extent to anomalies in geostationary orbits, (they increase by a factor ~4. Direct and indirect connections between anomaly occurrence and geomagnetic perturbations are also discussed.

  17. Aeromagnetic anomalies over faulted strata

    Science.gov (United States)

    Grauch, V.J.S.; Hudson, Mark R.

    2011-01-01

    High-resolution aeromagnetic surveys are now an industry standard and they commonly detect anomalies that are attributed to faults within sedimentary basins. However, detailed studies identifying geologic sources of magnetic anomalies in sedimentary environments are rare in the literature. Opportunities to study these sources have come from well-exposed sedimentary basins of the Rio Grande rift in New Mexico and Colorado. High-resolution aeromagnetic data from these areas reveal numerous, curvilinear, low-amplitude (2–15 nT at 100-m terrain clearance) anomalies that consistently correspond to intrasedimentary normal faults (Figure 1). Detailed geophysical and rock-property studies provide evidence for the magnetic sources at several exposures of these faults in the central Rio Grande rift (summarized in Grauch and Hudson, 2007, and Hudson et al., 2008). A key result is that the aeromagnetic anomalies arise from the juxtaposition of magnetically differing strata at the faults as opposed to chemical processes acting at the fault zone. The studies also provide (1) guidelines for understanding and estimating the geophysical parameters controlling aeromagnetic anomalies at faulted strata (Grauch and Hudson), and (2) observations on key geologic factors that are favorable for developing similar sedimentary sources of aeromagnetic anomalies elsewhere (Hudson et al.).

  18. Analýza vybrané firmy

    OpenAIRE

    Jandová, Jana

    2010-01-01

    Tato bakalářská práce utváří celkový obraz o vybrané firmě pomocí SWOT analýzy, SLEPTE analýzy, Porterovy analýzy konkurence a analyzuje její finanční situaci od roku 2006 do roku 2008 prostřednictvím soustav poměrových ukazatelů. Z výsledků všech analýz jsou navržena doporučení ke zlepšení stávající situace firmy. This bachelor’s thesis presents a picture of a selected firm by applying SWOT and SLEPTE analysis and Porter´s Five Forces analysis. Next follows an analysis of the financial st...

  19. Softwarová podpora analýzy rizik

    OpenAIRE

    Psota, Michal

    2013-01-01

    Tato práce se zabývá teoretickým popisem možností řízení rizik především v oblasti informačních technologií a popisuje metody sloužící k analýze rizik. Rozebrané metody jsou analýza stromu událostí, analýza stromu poruch, FMEA, HAZOP a Markovova analýza. Praktická část práce zahrnuje návrh a implementaci aplikace, která vizualizuje rozhodovací stromy a určuje pravděpodobnosti jednotlivých prvků.

  20. What Are the Risk Factors for Anal Cancer?

    Science.gov (United States)

    ... have few or no known risk factors. Human papilloma virus (HPV) infection Most squamous cell anal cancers ... to be linked to infection by the human papilloma virus (HPV), the same virus that causes cervical ...

  1. Analýza vybrané firmy

    OpenAIRE

    Peloušková, Tereza

    2013-01-01

    Předmětem bakalářská práce je analýza společnosti MMB Consulting, s.r.o., která se zabývá realizací staveb v Jihomoravském kraji. Obsahem práce je vypracování SLEPTE analýzy, Porterova modelu pěti konkurenčních sil a SWOT analýzy. Na základě provedených analýz jsou nakonec navrženy způsoby zlepšení současné situace společnosti. Subject of this thesis is the analysis the MMB Consulting company, which is engaged in construction of buildings in the South Moravian region. The thesis includes S...

  2. Tubulovillous adenoma of anal canal: A case report

    Institute of Scientific and Technical Information of China (English)

    Bhupinder S Anand; Gordana Verstovsek; George Cole

    2006-01-01

    Tumors arising from the anal canal are usually of epithelial origin and are mostly squamous cell carcinoma or basal cell carcinoma. We present a case of benign anal adenomas arising from the anus, an extremely rare diagnosis. A 78-year-old white man presented with rectal bleeding of several months duration. Examination revealed a 4 cm friable mass attached to the anus by a stalk. At surgery, the mass was grasped with a Babcock forceps and was resected using electrocautery.Microscopic examination revealed a tubulovillus adenoma with no areas of high grade dysplasia or malignant transformation. The squamocolumnar junction was visible at the edges of the lesion confirming the anal origin of the tumor. We believe the tubulovillus adenoma arose from either an anal gland or its duct that opens into the anus. Although seen rarely, it is important to recognize and treat these tumors at an early stage because of their potential to transform into adenocarcinoma.

  3. Prenatal Diagnosis and Postnatal Ultrasound Findings of Cloacal Anomaly: A Case Report

    Directory of Open Access Journals (Sweden)

    Lívia Teresa Moreira Rios

    2012-01-01

    Full Text Available Cloacal malformation is an extremely rare fetal pathological condition that presents as a variety of defects. It predominantly affects females, with prevalence of 1 in 50,000 births. Prenatal ultrasonography on a 20-year-old caucasian woman (G4P1A2 at 33 weeks of pregnancy showed the fetus having a large cystic mass in the lower abdomen with a single septum, bilateral hydronephrosis, ambiguous genitalia, and a single umbilical artery. The pregnancy developed accentuated oligohydramnios, and presence of a fetal brain-sparing effect was diagnosed using arterial Doppler velocimetry. The newborn showed abdominal distension, ambiguous genitalia, and rectal atresia, with a single perineal opening. Pelvic ultrasound done on the first day after delivery revealed the presence of a large retrovesical septated cystic mass of dense content in the fetal abdomen, and bilateral hydronephrosis. Hysterotomy was performed, and 70 mL of dense liquid was drained through an abdominal colostomy. The infant died on the 27th day of life as a result of infectious complications. Prenatal diagnosing of female urogenital anomalies is usually difficult because of their rarity, different types of manifestation, and lack of characteristic ultrasound signs. Presence of a septated cyst with dense content in the fetal abdomen confirms the finding of hydrometrocolpos, thus raising clinical suspicion of a cloacal anomaly.

  4. Ectrodactyly ectodermal dysplasia clefting (EEC) syndrome: a rare cause of congenital lacrimal anomalies.

    Science.gov (United States)

    Elmann, Solly; Hanson, Sarah A; Bunce, Christopher N; Shinder, Roman

    2015-01-01

    A 9-year-old girl with a medical history significant for ectrodactyly ectodermal dysplasia clefting (EEC) syndrome was referred for evaluation of congenital left-sided epiphora. The patient had undergone successful right external dacryocystorhinostomy at age 5 to treat congenital right-sided epiphora. On examination, several ocular anomalies were noted, including absence of the upper eyelid puncta, absence of the left inferior punctum, a left lacrimal fistula opening at the left caruncle, increased left tear lake, bilateral hypoplastic meibomian glands, mild conjunctival injection, and thin eyelid cilia and brow hair. Systemic findings included cleft lip and palate status-post repair, ectrodactyly of the hands and feet, adontia and microdontia, a pointed nose, and lightly pigmented, dry hair and skin. The patient underwent examination under anesthesia and left conjunctivodacryocystorhinostomy with insertion of a Jones tube with resolution of lacrimation postoperatively. To the authors' knowledge, this is the second report detailing management of congenital lacrimal anomalies in EEC syndrome, and the first describing management of punctal atresia with conjunctivodacryocystorhinostomy and Jones tube placement. PMID:24801258

  5. Anal sphincter EMG in the diagnosis of parkinsonian syndromes

    DEFF Research Database (Denmark)

    Winge, K; Jennum, Poul Jørgen; Løkkegaard, Annemette;

    2010-01-01

    The role of electromyography (EMG) recorded from the external anal sphincter (EAS) in the diagnosis of atypical parkinsonian syndromes is a matter for continuous debate. Most studies addressing this issue are retrospective.......The role of electromyography (EMG) recorded from the external anal sphincter (EAS) in the diagnosis of atypical parkinsonian syndromes is a matter for continuous debate. Most studies addressing this issue are retrospective....

  6. Innovations in chronic anal fissure treatment: A systematic review

    OpenAIRE

    2010-01-01

    A chronic anal fissure is a common perianal condition. This review aims to evaluate both existing and new therapies in the treatment of chronic fissures. Pharmacological therapies such as glyceryl trinitrate (GTN), Diltiazem ointment and Botulinum toxin provide a relatively non-invasive option, but with higher recurrence rates. Lateral sphincterotomy remains the gold standard for treatment. Anal dilatation has no role in treatment. New therapies include perineal support devices, Gonyautoxin i...

  7. Anal cancer and intraepithelial neoplasia screening: A review

    OpenAIRE

    Leeds, Ira L.; Fang, Sandy H

    2016-01-01

    This review focuses on the early diagnosis of anal cancer and its precursor lesions through routine screening. A number of risk-stratification strategies as well as screening techniques have been suggested, and currently little consensus exists among national societies. Much of the current clinical rationale for the prevention of anal cancer derives from the similar tumor biology of cervical cancer and the successful use of routine screening to identify cervical cancer and its precursors earl...

  8. 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.

    Science.gov (United States)

    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.

  9. Botulinum toxin A in the treatment of anal fissure

    Directory of Open Access Journals (Sweden)

    Stanković Nebojša

    2004-01-01

    Full Text Available Background. This paper presents our first experience in the treatment of primary anal fissure by injection of botulinum A toxin into the internal sphincter. Methods. The study group of the retrospective study included 12 outpatients (8 females and 4 males, mean age 42 (range 18-60. During the period 2000-2003, after unsuccessful conservative treatment, patients were treated with the injections of botulinum A toxin, 100 units on both sides of the anal fissure laterally into the internal anal sphincter (50 units on either side. The patients were clinically evaluated 3, 7, and 30 days, and 3 and 6 months after the treatment. Results. Three fissures had healed after a month, and seven after 3 months. Two remained unhealed, but asymptomatic. There was no incontinence of flatus or faeces after 3 months of the treatment. After temporary healing, two fissures relapsed after 6 months, and these patients had the adequate tonus of the anal sphincter muscles. Except for the temporary incontinence, there were neither other side-efects, nor serious complications. Conclusion. Injection of botulinum A toxin achieved good results in the treatment of anal fissure. The appropriate use makes this method safe as an alternative to surgical treatment of anal fissure.

  10. Reflexo pudendo-anal em mulheres normais Pudendo-anal reflex in normal women

    Directory of Open Access Journals (Sweden)

    Geraldo de Aguiar Cavalcanti

    2004-09-01

    Full Text Available São descritas observações do registro do reflexo pudendo-anal em mulheres sem queixas de incontinência urinária. Foram estudadas 31 voluntárias adultas, com estimulação elétrica bilateral e independente do clitóris e registros de superfície em ambos os lados do músculo esfíncter externo do ânus (EEA. As respostas foram obtidas com pulsos duplos de 0,2 ms de duração e intervalos de 5 ms, aplicados a uma freqüência menor que 0,5 Hz. Foram medidas as latências iniciais das respostas. Não foram evidenciadas diferenças entre as respostas obtidas de cada lado do EEA e nem entre os lados, com relação aos estímulos. Uma das voluntárias não apresentou respostas após estimulação de um dos lados. Não foram observadas diferenças relacionadas a paridade total e nem com a presença de partos vaginais. A idade e o índice de massa corpórea não se correlacionaram com as respostas. Em 12% das respostas, a medida das latências foi dificultada pela baixa relação sinal-ruído.The pudendo-anal reflex was studied in a sample of 31 normal women. Responses were obtained after bilateral independent stimulation of the clitoris, with surface recordings from both sides of the external anal sphincter. Reponses were elicited with double-pulses of 0,2 ms duration with a interstimulus interval of 5 ms, frequency of stimulation was lower than 0,5 Hz. A minimal of four responses were recorded after supramaximal stimulation. In one volunteer no response was recorded after unilateral stimulation. Latencies of the responses from the right and left sides of the anal sfincter after right and left stimulation were 36.35±6.37, 36.28±6.23, 35.88±4.68, 36.44±4.45ms, respectively. No relation was detected between latencies and age, body mass index and parity (considering either total parity or vaginal delivery only. In 12% of the recordings uncertainty was introduced in the latency measurements related to a poor signal-noise ratio.

  11. High prevalence of high grade anal intraepithelial neoplasia in HIV-infected women screened for anal cancer.

    Science.gov (United States)

    Hou, June Y; Smotkin, David; Grossberg, Robert; Suhrland, Mark; Levine, Rebecca; Smith, Harriet O; Negassa, Abdissa; McAndrew, Thomas C; Einstein, Mark H

    2012-06-01

    There is no consensus on optimal screening for anal cancer (AC) in HIV+ women. Seven hundred fifteen unique asymptomatic women in a high-prevalence HIV+ community were screened for AC with anal cytology and triage to high-resolution anoscopy after routine screening was implemented in a large urban hospital system. Of these, 75 (10.5%) had an abnormal anal cytology and 29 (38.7%) of those with an abnormality had high-grade anal intraepithelial neoplasia (AIN). Women with poorly controlled HIV were significantly more likely to have high-grade AIN (P = 0.03). Given the high rate of AIN in screened HIV-infected women, routine AC screening in all HIV-infected women should be strongly considered. PMID:22466085

  12. Prenatal sonographic diagnosis of focal musculoskeletal anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Ryu, Jung Kyu; Cho, Jeong Yeon; Lee, Young Ho; Kim, Ei Jeong; Chun, Yi Kyeong [Samsung Cheil Hospital, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2002-09-15

    Focal musculoskeletal anomalies are various and may be an isolated finding or may be found in conjunction with numerous associations, including genetic syndromes, Karyotype abnormals, central nervous system anomalies and other general musculoskeletal disorders. Early prenatal diagnosis of these focal musculoskeletal anomalies nor only affects prenatal care and postnatal outcome but also helps in approaching other numerous associated anomalies.

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

    Science.gov (United States)

    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

  14. The multifactorial origins of respiratory morbidity in patients surviving neonatal repair of esophageal atresia

    Directory of Open Access Journals (Sweden)

    Ana Catarina eFragoso

    2014-05-01

    Full Text Available Esophageal atresia with or without tracheoesophageal fistula (EA±TEF occurs in 1 out of every 3000 births. Current survival approaches 95% and research is therefore focused on morbidity and health-related quality of life issues. Up to 50% of neonates with EA±TEF have one or more additional malformations including those of the respiratory tract that occur in a relatively high proportion of them and particularly of those with VACTERL association. Additionally, a significant proportion of survivors suffer abnormal pulmonary function and chronic respiratory tract disease. The present review summarizes the current knowledge about the nature of these symptoms in patients treated for EA±TEF, and explores the hypothesis that disturbed development and maturation of the respiratory tract could contribute to their pathogenesis.

  15. Horizontal ventricular septum with dextroversion: hearts with and without aortic atresia.

    Science.gov (United States)

    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.

  16. Methoxychlor induces atresia of antral follicles in ERalpha-overexpressing mice.

    Science.gov (United States)

    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

  17. External auditory canal atresia of probable congenital origin in a dog.

    Science.gov (United States)

    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.

  18. Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia

    Directory of Open Access Journals (Sweden)

    Muhammad SAEED*

    2014-12-01

    Full Text Available How to Cite This Article: Saeed M, Haq A, Qadir Kh.Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia. Iran J Child Neurol. 2014 Autumn;8(4: 76-79.AbstractObjectiveBart’s syndrome is defined as congenital localized absence of skin, and associated with epidermolysis bullosa. A newborn with Bart’s syndrome is reported because it is a very rare condition, especially when associated with corpus callosum agenesis and concomitant choanal atresia. Clinically it is characterized by raw beefy areas of denuded skin mainly on hands and feet.We report a rare case of a term female newborn born to non-consanguineous parents who presented with congenital absence of skin in, face, trunk and extremities. To the best of our knowledge, this is the first report presenting a case of Bart’s syndrome associated with corpus callosum agenesis.ReferencesBart BJ, Garlin RJ, Anderson VE, Lynch FW. Congenital localized absence of skin and associated abnormalities resembling epidermolysis bullosa. A new syndrome. Arch Dermatol 1966; 93: 296-304.Bart BJ. Epidermolysis bullosa and congenital localized absence of skin. Arch Dermatol 1970; 101: 78-81.Skoven I, Drzewiecki KT. Congenital localized skin defect and epidermolysis bullosa hereditaria letalis. Acta Derm Venereol 1979; 59: 533-537.Wojnarowska FT, Eady RA, Wells RS. Dystrophic epidermolysis bullosa presenting with congenital localized absence of skin: report of four cases. Br J Dermatol 1983; 108: 477-483.Kanzler MH, Smoller B, Woodley DT. Congenital localized absence of the skin as a manifestation of epidermolysis bullosa. Arch Dermatol 1992; 128:1087-90.Maman E, Maor E, Kachko L, Carmi R. Epidermolysis bullosa, pyloric atresia, aplasia cutis congenita: histopathological delineation of an autosomal recessive disease. Am J Med Genet 1998; 78: 127-133.McCarthy MA, Clarke T, Powell FC. Epidermolysis bullosa and aplasia cutis. Int J Derm 1991; 30: 481-484.Puvabanditsin S, Garrow E, Daeun K

  19. The role of gastrostomy in the staged operation of esophageal atresia

    Directory of Open Access Journals (Sweden)

    Hosseini Seyed

    2008-01-01

    Full Text Available Introduction: The aim of this study is to recommend criteria for selection of patients who benefited from the use of gastrostomy rather than emergency fistula closure during the staged operation of esophageal atresia (EA. Materials and Methods: Between August 2004 and July 2006, 75 cases of EA, were consecutively operated. Nineteen out of 75 (25% underwent routine gastrostomy because they required a type of staged operation: Group I: Five cases with pure atresia had gastrostomy and esophagostomy; Group II: Six with severe pneumonia and congenital heart disease (Waterson class C had gastrostomy and conservative management; Group III: Eight with long gap EA (2-4 vertebras; four out of 8 cases underwent primary anastomosis with tension and the other four had delayed primary anastomosis plus primary gastrostomy. Results: GI: Only three cases survived after esophageal substitution; GII: Three out of six cases with severe pneumonia (fistula size: f > 2.5 mm underwent emergency fistula closure with only one survival, but all (f < 2.5 mm recovered without complication, GIII: Four patients with long gap and primary anastomosis with tension developed anastomotic leakage; they required gastrostomy following the leakage, except for those with delayed primary anastomosis, and all of them recovered without early complications. Conclusion : All the cases with long gap, although two esophageal ends can be reached with tension, should undergo delayed primary closure with primary gastrostomy. Those were brought with Waterson class C and the fistula size greater than 2.5 mm should undergo emergency fistula closure; however, if fistula size was less than 2.5 mm, it is better to be delayed by primary gastrostomy for stabilization. In this study, we had a better outcome with gastric tube for substitution than colon interposition in infants.

  20. Congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula: clinical and radiologic features

    Energy Technology Data Exchange (ETDEWEB)

    Yoo, Hye Jin; Kim, Woo Sun; Cheon, Jung-Eun; Shin, Su-Mi; Kim, In-One; Yeon, Kyung Mo [Seoul National University College of Medicine and the Institute of Radiation Medicine, Department of Radiology, Seoul (Korea); Yoo, So-Young [Seoul National University College of Medicine and the Institute of Radiation Medicine, Department of Radiology, Seoul (Korea); Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea); Park, Kwi-Won; Jung, Sung-Eun [Seoul National University Children' s Hospital, Department of Pediatric Surgery, Seoul (Korea)

    2010-08-15

    Congenital esophageal stenosis (CES) can be associated with esophageal atresia/tracheoesophageal fistula (EA/TEF). Because there are a variety of degrees of obstruction and symptoms of CES, it is frequently difficult to make a pre- and post-operative diagnosis of the distal CES associated with EA/TEF. To evaluate the clinical and radiologic features of congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula. We retrospectively reviewed postoperative esophagograms and medical records of 187 children (107 boys, 80 girls) who had primary repair of EA/TEF from 1992 to 2009 at our institution. We evaluated the incidence of CES, clinical findings, radiologic features and management of CES in these children. CES was diagnosed in 22 of 187 EA/TEF children (12%); one child had double CES lesions, for a total of 23 lesions. Ten of those 22 children (45%) had presented with significant symptoms of esophageal obstruction. The diagnosis of CES was delayed in 10 children (45%) until 1-10 years of age. On esophagogram, CES (n = 23) was located in the distal esophagus (n = 20, 87%) or mid-esophagus (n = 3, 13%). The degree of stenosis was severe (n = 6, 26%), moderate (n = 10, 43%), or mild (n = 7, 30%). Eight children, including two with unsuccessful esophageal balloon dilatation of CES, were treated surgically. Histologic examination revealed tracheobronchial remnant (n = 7) or fibromuscular hyperplasia (n = 1). One child with surgically treated CES developed achalasia at the age of 3 years 9 months. Esophagography after EA/TEF repair should be performed with a high index of suspicion for the presence of distal CES, because the diagnosis and adequate management of CES can often be delayed. (orig.)

  1. Silencing of the rotavirus NSP4 protein decreases the incidence of biliary atresia in murine model.

    Directory of Open Access Journals (Sweden)

    Jiexiong Feng

    Full Text Available Biliary atresia is a common disease in neonates which causes obstructive jaundice and progressive hepatic fibrosis. Our previous studies indicate that rotavirus infection is an initiator in the pathogenesis of experimental biliary atresia (BA through the induction of increased nuclear factor-kappaB and abnormal activation of the osteopontin inflammation pathway. In the setting of rotavirus infection, rotavirus nonstructural protein 4 (NSP4 serves as an important immunogen, viral protein 7 (VP7 is necessary in rotavirus maturity and viral protein 4 (VP4 is a virulence determiner. The purpose of the current study is to clarify the roles of NSP4, VP7 and VP4 in the pathogenesis of experimental BA. Primary cultured extrahepatic biliary epithelia were infected with Rotavirus (mmu18006. Small interfering RNA targeting NSP4, VP7 or VP4 was transfected before rotavirus infection both in vitro and in vivo. We analyzed the incidence of BA, morphological change, morphogenesis of viral particles and viral mRNA and protein expression. The in vitro experiments showed NSP4 silencing decreased the levels of VP7 and VP4, reduced viral particles and decreased cytopathic effect. NSP4-positive cells had strongly positive expression of integrin subunit α2. Silencing of VP7 or VP4 partially decreased epithelial injury. Animal experiments indicated after NSP4 silencing, mouse pups had lower incidence of BA than after VP7 or VP4 silencing. However, 33.3% of VP4-silenced pups (N = 6 suffered BA and 50% of pups (N = 6 suffered biliary injury after VP7 silencing. Hepatic injury was decreased after NSP4 or VP4 silencing. Neither VP4 nor VP7 were detected in the biliary ducts after NSP4. All together, NSP4 silencing down-regulates VP7 and VP4, resulting in decreased incidence of BA.

  2. GEOMAGNETIC ANOMALY FIELD VECTOR OFF WESTERN AUSTRALIA

    OpenAIRE

    ノギ, ヨシフミ; エグチ, ヨシアキ; セアマ, ノブカズ; イセザキ, ノブヒロ; Yoshifumi, NOGI; Yoshiaki, EGUCHI; Nobukazu, SEAMA; Nobuhiro, ISEZAKI

    1993-01-01

    Vector data of the geomagnetic anomaly field were obtained during the 32nd Japanese Antarctic Research Expedition (JARE-32) off Western Australia. The strikes of the magnetic boundaries at their position were derived from vector data of the geomagnetic anomaly field. These strikes were interpreted as the directions of magnetic anomaly lineations originated either by seafloor spreading (seafloor spreading anomaly) or by morphological structures (structural magnetic anomaly). Some strikes of st...

  3. PSEUDO MAGNETIC ANOMALIES IN THE ANTARCTIC SEA

    OpenAIRE

    マツモト, タケシ; カミヌマ, カツタダ; Takeshi, MATSUMOTO; Katsutada, Kaminuma

    1988-01-01

    Pseudo magnetic anomaly in the Antarctic Sea has been calculated using the gravity data derived from altimetric geoid. Comparison of the pseudo magnetic anomaly thus calculated with the theoretical magnetic anomaly predicted from topography has been made with respect to the large fracture zones composed of short-wavelength ridges and troughs in the Southeastern Pacific, which shows that these two anomalies coincide well with each other. Gravity anomaly calculated from topography only also coi...

  4. Prenatal Sonographic Diagnosis of Focal Musculoskeletal Anomalies

    OpenAIRE

    Ryu, Jung Kyu; Cho, Jeong Yeon; Choi, Jong Sun

    2003-01-01

    Focal musculoskeletal anomalies vary, and can manifest as part of a syndrome or be accompanied by numerous other conditions such as genetic disorders, karyotype abnormalities, central nervous system anomalies and other skeletal anomalies. Isolated focal musculoskeletal anomaly does, however, also occur; its early prenatal diagnosis is important in deciding prenatal care, and also helps in counseling parents about the postnatal effects of numerous possible associated anomalies. We have encount...

  5. Biliary Atresia

    Science.gov (United States)

    ... their structure. The procedure is performed in a health care provider’s office, outpatient center, or hospital by a specially trained technician, and the images are interpreted a radiologist. Anesthesia is not needed, but sedation may be ...

  6. Graph anomalies in cyber communications

    Energy Technology Data Exchange (ETDEWEB)

    Vander Wiel, Scott A [Los Alamos National Laboratory; Storlie, Curtis B [Los Alamos National Laboratory; Sandine, Gary [Los Alamos National Laboratory; Hagberg, Aric A [Los Alamos National Laboratory; Fisk, Michael [Los Alamos National Laboratory

    2011-01-11

    Enterprises monitor cyber traffic for viruses, intruders and stolen information. Detection methods look for known signatures of malicious traffic or search for anomalies with respect to a nominal reference model. Traditional anomaly detection focuses on aggregate traffic at central nodes or on user-level monitoring. More recently, however, traffic is being viewed more holistically as a dynamic communication graph. Attention to the graph nature of the traffic has expanded the types of anomalies that are being sought. We give an overview of several cyber data streams collected at Los Alamos National Laboratory and discuss current work in modeling the graph dynamics of traffic over the network. We consider global properties and local properties within the communication graph. A method for monitoring relative entropy on multiple correlated properties is discussed in detail.

  7. Conformal Anomalies and Gravitational Waves

    CERN Document Server

    Meissner, Krzysztof A

    2016-01-01

    We argue that the presence of conformal anomalies in gravitational theories can lead to observable modifications to Einstein's equations via the induced anomalous effective actions, whose non-localities can overwhelm the smallness of the Planck scale. The fact that no such effects have been seen in recent cosmological or gravitational wave observations therefore imposes strong restrictions on the field content of possible extensions of Einstein's theory: all viable theories should have vanishing conformal anomalies. We then show that, among presently known theories, a complete cancellation of conformal anomalies in $D=4$ for both the $C^2$ invariant and the Euler (Gauss-Bonnet) invariant $E_4$ can only be achieved for $N$-extended supergravities with $N\\geq 5$, as well as for M theory compactified to four dimensions.

  8. Current treatment options for management of anal intraepithelial neoplasia.

    Science.gov (United States)

    Weis, Stephen E

    2013-01-01

    Anal squamous cell cancer is an uncommon malignancy caused by infection with oncogenic strains of Human papilloma virus. Anal cancer is much more common in immunocompromised persons, including those infected with Human immunodeficiency virus. High-grade anal intraepithelial neoplasia (HGAIN), the precursor of anal cancer, is identified by clinicians providing care for patients with anorectal disease, and is increasingly being identified during screening of immunosuppressed patients for anal dysplasia. The traditional treatment for HGAIN has been excision of macroscopic disease with margins. This approach is effective for patients with small unifocal HGAIN lesions. Patients with extensive multifocal HGAIN frequently have recurrence of HGAIN after excision, and may have postoperative complications of anal stenosis or fecal incontinence. This led to the suggestion by some that treatment for HGAIN should be delayed until patients developed anal cancer. Alternative approaches in identification and treatment have been developed to treat patients with multifocal or extensive HGAIN lesions. High-resolution anoscopy combines magnification with anoscopy and is being used to identify HGAIN and determine treatment margins. HGAIN can then be ablated with a number of modalities, including infrared coagulation, CO2 laser, and electrocautery. These methods for HGAIN ablation can be performed with local anesthesia on outpatients and are relatively well tolerated. High-resolution anoscopy-directed HGAIN ablation is evolving into a standard approach for initial treatment and then subsequent monitoring of a disease which should be expected to be recurrent. Another treatment approach for HGAIN is topical treatment, principally with 5-fluorouracil or imiquimod. Topical therapies have the advantage of being nonsurgical and are well suited for treating widespread multifocal disease. Topical treatments have the disadvantage of requiring extended treatment courses and causing a symptomatic

  9. Síndrome de Treacher Collins com atresia coanal: relato de caso e revisão de suas características Treacher Collins syndrome with choanal atresia: a case report and review of disease features

    Directory of Open Access Journals (Sweden)

    Eduardo C. Andrade

    2005-02-01

    Full Text Available A Síndrome de Treacher Collins ou disostose mandibulofacial apresenta-se com deformidades crânio-faciais, tendo expressão e severidade variável. É uma malformação congênita que envolve o primeiro e segundo arcos branquiais. A Síndrome de Treacher Collins é rara e sua incidência está estimada em uma faixa de 1:40000 a 1:70000 nascidos vivos. Esta síndrome é caracterizada por anormalidades dos pavilhões auriculares, hipoplasia dos ossos da face, obliqüidade antimongolóide das fendas palpebrais com coloboma palpebral inferior e fissura palatina. A Síndrome de Treacher Collins raramente está associada com atresia coanal. Estes pacientes são apropriadamente acompanhados por uma equipe multidisciplinar que inclui cirurgiões crânio-faciais, oftalmologistas, fonoaudiologistas, cirurgiões dentistas e otorrinolaringologistas. Relatamos neste artigo um caso raro de Síndrome de Treacher Collins com atresia coanal, uma revisão da patologia e intervenção multidisciplinar.Treacher Collins Syndrome - or mandibulofacial dysostosis - is a rare condition that presents several craniofacial deformities of different levels. This is a congenital malformation involving the first and second branchial arches. Incidence is estimated to range between 1-40,000 to 1-70,000 of live births. The disorder is characterized by abnormalities of the auricular pinna, hypoplasia of facial bones, antimongoloid slanting palpebral fissures with coloboma of the lower eyelids and cleft palate. Treacher Collins Syndrome is rarely associated with choanal atresia. A multidisciplinary team, including craniofacial surgeon, ophthalmologist, speech therapist, dental surgeon and otorhinolaryngologist, is the most appropriate setting to manage these patients. This study reports a rare case of Treacher Collins Syndrome with choanal atresia, presenting literature review and multidisciplinary intervention.

  10. Boundary anomalies and correlation functions

    Science.gov (United States)

    Huang, Kuo-Wei

    2016-08-01

    It was shown recently that boundary terms of conformal anomalies recover the universal contribution to the entanglement entropy and also play an important role in the boundary monotonicity theorem of odd-dimensional quantum field theories. Motivated by these results, we investigate relationships between boundary anomalies and the stress tensor correlation functions in conformal field theories. In particular, we focus on how the conformal Ward identity and the renormalization group equation are modified by boundary central charges. Renormalized stress tensors induced by boundary Weyl invariants are also discussed, with examples in spherical and cylindrical geometries.

  11. Boundary Anomalies and Correlation Functions

    CERN Document Server

    Huang, Kuo-Wei

    2016-01-01

    It was shown recently that boundary terms of conformal anomalies recover the universal contribution to the entanglement entropy and also play an important role in the boundary monotonicity theorem of odd-dimensional quantum field theories. Motivated by these results, we investigate relationships between boundary anomalies and the stress tensor correlation functions in conformal field theories. In particular, we focus on how the conformal Ward identity and the renormalization group equation are modified by boundary central charges. Renormalized stress tensors induced by boundary Weyl invariants are also discussed, with examples in spherical and cylindrical geometries.

  12. Prevention of vitamin K deficiency bleeding in breastfed infants: lessons from the Dutch and Danish biliary atresia registries

    DEFF Research Database (Denmark)

    Hasselt, P.M. van; Koning, T.J. de; Vries, E. de;

    2008-01-01

    OBJECTIVE: Newborns routinely receive vitamin K to prevent vitamin K deficiency bleeding. The efficacy of oral vitamin K administration may be compromised in infants with unrecognized cholestasis. We aimed to compare the risk of vitamin K deficiency bleeding under different prophylactic regimens...... in infants with biliary atresia. PATIENTS AND METHODS: From Dutch and Danish national biliary atresia registries, we retrieved infants who were either breastfed and received 1 mg of oral vitamin K at birth followed by 25 microg of daily oral vitamin K prophylaxis (Netherlands, 1991-2003), 2 mg of oral...... vitamin K at birth followed by 1 mg of weekly oral prophylaxis (Denmark, 1994 to May 2000), or 2 mg of intramuscular prophylaxis at birth (Denmark, June 2000-2005) or were fed by formula. We determined the absolute and relative risk of severe vitamin K deficiency and vitamin K deficiency bleeding...

  13. Gaseous distention of the hypopharynx and cervical esophagus with nasal CPAP: a mimicker of pharyngeal perforation and esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Walor, David; Berdon, Walter; Holt, Peter D.; Fox, Matthew [Columbia University Medical Center, Department of Radiology, New York, NY (United States); Children' s Hospital of New York, New York, NY (United States); Anderson, Nicole [Columbia University Medical Center, Department of Neonatology, New York, NY (United States); Children' s Hospital of New York, New York, NY (United States)

    2005-12-01

    Nasal continuous positive airway pressure (CPAP) has been used since 1975 as the initial treatment for respiratory distress syndrome (RDS) in very premature infants. Gaseous distention of the abdomen (CPAP belly) is a common secondary effect of CPAP. Gaseous distention of the hypopharynx is also common. To determine the incidence of hypopharyngeal distention in infants on CPAP. We performed a retrospective review of the chest radiographs of 57 premature infants treated with CPAP during a 4-week period to find the presence and degree of hypopharyngeal distention. Of the 57 radiographs, 14 (25%) revealed gaseous distention of the hypopharynx and/or cervical esophagus. On occasion, this raised concern for pharyngeal perforation or esophageal atresia. Awareness that CPAP-related hypopharyngeal distention is common should help radiologists avoid erroneous consideration of esophageal atresia or hypopharyngeal perforation. (orig.)

  14. Gaseous distention of the hypopharynx and cervical esophagus with nasal CPAP: a mimicker of pharyngeal perforation and esophageal atresia

    International Nuclear Information System (INIS)

    Nasal continuous positive airway pressure (CPAP) has been used since 1975 as the initial treatment for respiratory distress syndrome (RDS) in very premature infants. Gaseous distention of the abdomen (CPAP belly) is a common secondary effect of CPAP. Gaseous distention of the hypopharynx is also common. To determine the incidence of hypopharyngeal distention in infants on CPAP. We performed a retrospective review of the chest radiographs of 57 premature infants treated with CPAP during a 4-week period to find the presence and degree of hypopharyngeal distention. Of the 57 radiographs, 14 (25%) revealed gaseous distention of the hypopharynx and/or cervical esophagus. On occasion, this raised concern for pharyngeal perforation or esophageal atresia. Awareness that CPAP-related hypopharyngeal distention is common should help radiologists avoid erroneous consideration of esophageal atresia or hypopharyngeal perforation. (orig.)

  15. Current treatment options for management of anal intraepithelial neoplasia

    Directory of Open Access Journals (Sweden)

    Weis SE

    2013-06-01

    Full Text Available Stephen E Weis1,2 1Division of Dermatology, Department of Internal Medicine, University of North Texas Health Science Center at Fort Worth, 2Preventive Medicine Clinic, Tarrant County Public Health, Fort Worth, TX, USA Abstract: Anal squamous cell cancer is an uncommon malignancy caused by infection with oncogenic strains of Human papilloma virus. Anal cancer is much more common in immunocompromised persons, including those infected with Human immunodeficiency virus. High-grade anal intraepithelial neoplasia (HGAIN, the precursor of anal cancer, is identified by clinicians providing care for patients with anorectal disease, and is increasingly being identified during screening of immunosuppressed patients for anal dysplasia. The traditional treatment for HGAIN has been excision of macroscopic disease with margins. This approach is effective for patients with small unifocal HGAIN lesions. Patients with extensive multifocal HGAIN frequently have recurrence of HGAIN after excision, and may have postoperative complications of anal stenosis or fecal incontinence. This led to the suggestion by some that treatment for HGAIN should be delayed until patients developed anal cancer. Alternative approaches in identification and treatment have been developed to treat patients with multifocal or extensive HGAIN lesions. High-resolution anoscopy combines magnification with anoscopy and is being used to identify HGAIN and determine treatment margins. HGAIN can then be ablated with a number of modalities, including infrared coagulation, CO2 laser, and electrocautery. These methods for HGAIN ablation can be performed with local anesthesia on outpatients and are relatively well tolerated. High-resolution anoscopy-directed HGAIN ablation is evolving into a standard approach for initial treatment and then subsequent monitoring of a disease which should be expected to be recurrent. Another treatment approach for HGAIN is topical treatment, principally with 5

  16. Bisphenol A Inhibits Follicle Growth and Induces Atresia in Cultured Mouse Antral Follicles Independently of the Genomic Estrogenic Pathway1

    OpenAIRE

    Peretz, Jackye; Craig, Zelieann R.; Flaws, Jodi A.

    2012-01-01

    Bisphenol A (BPA) is an estrogenic chemical used to manufacture many commonly used plastic and epoxy resin-based products. BPA ubiquitously binds to estrogen receptors throughout the body, including estrogen receptor alpha (ESR1) in the ovary. Few studies have investigated the effects of BPA on ovarian antral follicles. Thus, we tested the hypothesis that BPA alters cell cycle regulators and induces atresia in antral follicles via the genomic estrogenic pathway, inhibiting follicle growth. To...

  17. Congenital Esophageal Atresia Surgery Treatment Experience%先天性食管闭锁外科治疗经验

    Institute of Scientific and Technical Information of China (English)

    侯可峰; 薛枫; 窦欣欣; 王培倩

    2015-01-01

    目的:总结分析先天性食管闭锁的诊断及外科治疗,观察其治疗效果,总结经验。方法对我院2011年1月~2014年11月收治的16例先天性食管闭锁型患儿的临床资料进行回顾分析,对食管闭锁的诊治、术后主要并发症的影响因素及诊断治疗进行分析。结果16例患儿全部康复出院,中仅有1例发生吻合口漏,未见明显吻合口狭窄。结论食管闭锁的分型是影响治疗效果的重要因素,外科吻合技术的提高是手术成功的关键。%Objective To Summarize and analyze the diagnosis and surgical treatment of congenital esophageal atresia, observe the treatment effect, summarize the experience. Methods The clinical data of our hospital in 2011January to 2014 year in November 16 cases of congenital esophageal atresia were analyzed retrospectively, analyzed main inlfuencing operation of esophageal atresia, diagnosis and treatment of complications of diagnostic and treatment factors. Results 16 patients were discharged from the hospital, only 1 cases of anastomotic leakage, no anastomotic stenosis. Conclusion Esophageal atresia type is an important factor to affect the treatment effect, surgical technique is the key to improve the success of operation.

  18. Di(2-ethylhexyl) phthalate inhibits antral follicle growth, induces atresia, and inhibits steroid hormone production in cultured mouse antral follicles

    Energy Technology Data Exchange (ETDEWEB)

    Hannon, Patrick R., E-mail: phannon2@illinois.edu; Brannick, Katherine E., E-mail: kbran@illinois.edu; Wang, Wei, E-mail: Wei.Wang2@covance.com; Gupta, Rupesh K., E-mail: drrupesh@yahoo.com; Flaws, Jodi A., E-mail: jflaws@illinois.edu

    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

  19. Efficacy of Lower-Extremity Venous Thrombolysis in the Setting of Congenital Absence or Atresia of the Inferior Vena Cava

    Energy Technology Data Exchange (ETDEWEB)

    Ganguli, Suvranu, E-mail: sganguli@partners.org; Kalva, Sanjeeva; Oklu, Rahmi; Walker, T. Gregory; Datta, Neil [Massachusetts General Hospital and Harvard Medical School, Division of Vascular Imaging and Intervention, Department of Imaging (United States); Grabowski, Eric F. [Massachusetts General Hospital and Harvard Medical School, Division of Hematology and Oncology, Department of Pediatrics (United States); Wicky, Stephan [Massachusetts General Hospital and Harvard Medical School, Division of Vascular Imaging and Intervention, Department of Imaging (United States)

    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.

  20. Analýza vybrané firmy

    OpenAIRE

    Ligurská, Pavlína

    2014-01-01

    Bakalářská práce je zaměřena na analýzu vybrané firmy. Práce je rozdělena na teoretickou a praktickou část. V teoretické části jsou popsány metody, které byly použity pro analyzování firmy. V praktické části jsou aplikovány jednotlivé metody, Pro analýzu vnějšího prostředí byla použita analýza SLEPTE, pro analýzu odvětví byl použit Porterův model pěti konkurenčních sil, v další části se práce zabývá identifikací silných a slabých stránek firmy. V závěru práce jsou zhodnoceny výsledky analýz a...

  1. Analýza vybrané firmy

    OpenAIRE

    Rysková, Diana

    2013-01-01

    Bakalářská práce se zabývá zhodnocením situace vybraného podniku prostřednictvím analýz vnějšího a vnitřního okolí, mezi které patří SLEPTE analýza, SWOT analýza a Porterův model pěti konkurenčních sil. Teoretická část vystihuje podstatu jednotlivých analýz, které jsou následně aplikovány v praktické části bakalářské práce. Na základě výsledků z analýz jsou navržena řešení zjištěných problémových oblastí, směřující k upevnění postavení společnosti na trhu a jejího možného růstu. The bachel...

  2. The Anal Pap Smear: Cytomorphology of squamous intraepithelial lesions

    Directory of Open Access Journals (Sweden)

    Arain Shehla

    2005-01-01

    Full Text Available Abstract Background Anal smears are increasingly being used as a screening test for anal squamous intraepithelial lesions (ASILs. This study was undertaken to assess the usefulness and limitations of anal smears in screening for ASILs. Methods The cytomorphological features of 200 consecutive anal smears collected in liquid medium from 198 patients were studied and findings were correlated with results of surgical biopsies and/or repeat smears that became available for 71 patients within six months. Results Adequate cellularity was defined as an average of 6 or more nucleated squamous cells/hpf. A glandular/transitional component was not required for adequacy. Dysplastic cells, atypical parakeratotic cells and bi/multinucleated cells were frequent findings in ASIL while koilocytes were infrequent. Smears from LSIL cases most frequently showed mildly dysplastic and bi/multinucleate squamous cells followed by parakeratotic cells (PK, atypical parakeratotic cells (APK, and koilocytes. HSIL smears contained squamous cells with features of moderate/severe dysplasia and many APKs. Features of LSIL were also found in most HSIL smears. Conclusions In this study liquid based anal smears had a high sensitivity (98% for detection of ASIL but a low specificity (50% for predicting the severity of the abnormality in subsequent biopsy. Patients with cytologic diagnoses of ASC-US and LSIL had a significant risk (46–56% of HSIL at biopsy. We suggest that all patients with a diagnosis of ASC-US and above be recommended for high resolution anoscopy with biopsy.

  3. Metachronous tubulovillous and tubular adenomas of the anal canal.

    Science.gov (United States)

    Nozawa, Hiroaki; Ishihara, Soichiro; Morikawa, Teppei; Tanaka, Junichiro; Yasuda, Koji; Ohtani, Kensuke; Nishikawa, Takeshi; Tanaka, Toshiaki; Kiyomatsu, Tomomichi; Kawai, Kazushige; Hata, Keisuke; Kazama, Shinsuke; Yamaguchi, Hironori; Sunami, Eiji; Kitayama, Joji; Fukayama, Masashi; Watanabe, Toshiaki

    2015-01-01

    Anal canal adenoma is an extremely rare disease that has the potential to transform into a malignant tumor. We herein presented a rare case of metachronous multiple adenomas of the anal canal. A 48-year-old woman underwent total colonoscopy following a positive fecal blood test. A 9-mm villous polyp arising from the posterior wall of the anal canal was removed by snare polypectomy. Histologically, the tumor was tubulovillous adenoma with high-grade dysplasia and the cut end was negative for tumor cells. Six years later, an elevated lesion, macroscopically five millimeters in size, was detected in the left wall of the anal canal in a follow-up colonoscopy. Local excision of the tumor was performed, and the lesion was pathologically confirmed to be tubular adenoma with high-grade dysplasia limited to the mucosa. The patient is currently alive without any evidence of recurrence for six months after surgery. Although she had a past history of cervical cancer, the multiple tumors arising in the anal canal were unlikely to be related to human papilloma virus infection. Our case report underscores the importance of careful observations throughout colonoscopy to detect precancerous lesions, particularly in anatomically narrow segments. PMID:26249723

  4. All's Well That Ends Well: Shakespeare's treatment of anal fistula.

    Science.gov (United States)

    Cosman, B C

    1998-07-01

    Textual and contextual evidence suggests that the French king's fistula, a central plot device in Shakespeare's play All's Well That Ends Well, is a fistula-in-ano. Anal fistula was known to the lay public in Shakespeare's time. In addition, Shakespeare may have known of the anal fistula treatise of John Arderne, an ancestor on Shakespeare's mother's side. Shakespeare's use of anal fistula differs from all previous versions of the story, which first appeared in Boccaccio's Decameron and from its possible historical antecedent, the fistula of Charles V of France. This difference makes sense given the conventions of Elizabethan comedy, which included anal humor. It is also understandable when one looks at what wounds in different locations mean in European legend. In this light, it is not surprising that subsequent expurgations treat Boccaccio's and Shakespeare's fistulas differently, censoring only Shakespeare's. This reading has implications for the staging of All's Well That Ends Well, and for our view of the place of anal fistulas in cultural history.

  5. Concomitant slide tracheoplasty and cardiac operation for congenital tracheal stenosis associated with VACTERL.

    Science.gov (United States)

    Wu, En-Ting; Wang, Ching-Chia; Lin, Ming-Tai; Huang, Pei-Ming; Chen, Shyh-Jye; Huang, Chi-Hsiang; Hwang, Haw-Kwei; Chen, Ming-Ren; Huang, Shu-Chien

    2013-10-01

    The association of congenital tracheal stenosis and tracheoesophageal (TE) fistula is rare. Here, we report 2 patients with tracheobronchial stenosis (complete cartilage ring) involving the lower trachea and right bronchus. Both patients had associated VACTERL (vertebral anomalies, anal atresia, cardiovascular anomalies, TE, renal, and limb defects) congenital cardiac defects and tracheal diverticula after repair of the TE fistula in type C esophageal atresia. The stenotic segment began at the orifice of the TE fistula, which became diverticula after the TE fistula was repaired. Concomitant repair of congenital cardiac defects and a slide tracheoplasty with elimination of the diverticula were performed successfully. PMID:24088476

  6. Apoptosis in Granulosa cells during follicular atresia:relationship with steroids and insulin-like growth factors

    Institute of Scientific and Technical Information of China (English)

    Yuan Song YU; Hong Shu SUI; Zheng Bin HAN; Wei LI; Ming Jiu LUO; Jing He TAN

    2004-01-01

    It is well known that during mammalian ovarian follicular development, the majority of follicles undergo atresia at various stages of their development. However, the mechanisms controlling this selection process remain unknown. In this study, we investigated apoptosis in granulosa cells during goat follicular atresia by terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL). The changes in the levels of steroids, insulin-like growth factors (IGFs) and IGF receptors were studied by radioimmunoassay (RIA) and semi-quantitative reverse transcription-PCR. We found that the percentage of apoptotic granulosa cells in the atretic (A) follicles was significantly higher than that in the slightly atretic (SA) and healthy (H) follicles. The level of estradiol and the ratio of estradiol to progesterone in H follicles were significantly higher than those in A follicles. On the other hand, the level of progesterone was not significantly different among these follicle types. We also found that the level of IGF-I in H follicles was higher than in SA and A follicles, whereas the amount of IGF-Ⅱ did not vary significantly. The expression of IGF receptor also decreased in A follicles as compared to that in H and SA follicles. These results suggested that estradiol and IGF-I might be involved in controlling apoptosis in granulosa cells during follicular atresia.

  7. Gaugino-Assisted Anomaly Mediation

    International Nuclear Information System (INIS)

    We present a model of supersymmetry breaking mediated through a small extra dimension. Standard model matter multiplets and a supersymmetry-breaking (or ''hidden'') sector are confined to opposite four-dimensional boundaries while gauge multiplets live in the bulk. The hidden sector does not contain a singlet and the dominant contribution to gaugino masses is via anomaly-mediated supersymmetry breaking. Scalar masses get contributions from both anomaly mediation and a tiny hard breaking of supersymmetry by operators on the hidden-sector boundary. These operators contribute to scalar masses at one loop and in most of parameter space, their contribution dominates. Thus it is easy to make all squared scalar masses positive. As no additional fields or symmetries are required below the Planck scale, we consider this the simplest working model of anomaly mediation. The gaugino spectrum is left untouched and the phenomenology of the model is roughly similar to anomaly mediated supersymmetry breaking with a universal scalar mass added. We identify the main differences in the spectrum between this model and other approaches. We also discuss mechanisms for generating the μ term and constraints on additional bulk fields. (author)

  8. Gaugino-assisted anomaly mediation

    International Nuclear Information System (INIS)

    I present a model of supersymmetry breaking mediated through a small extra dimension. Standard model matter multiplets and a supersymmetry-breaking (or 'hidden') sector are confined to opposite four-dimensional boundaries while gauge multiplets live in the bulk. The hidden sector does not contain a singlet and the dominant contribution to gaugino masses is via anomaly-mediated supersymmetry breaking. Scalar masses get contributions from both anomaly mediation and a tiny hard breaking of supersymmetry by operators on the hidden-sector boundary. These operators contribute to scalar masses at one loop and in most of parameter space, their contribution dominates. Thus it is easy to make all squared scalar masses positive. As no additional fields or symmetries are required below the Planck scale, this is among the simplest working models of anomaly mediation. The gaugino spectrum is left untouched and the phenomenology of the model is roughly similar to anomaly mediated supersymmetry breaking with a universal scalar mass added. Finally, the main differences in the spectrum between this model and other approaches are identified. This talk is based on work [1] done in collaboration with David E. Kaplan

  9. Prenatal sonographic diagnosis of fetal esophageal atresia%胎儿食道闭锁产前超声诊断分析

    Institute of Scientific and Technical Information of China (English)

    胡静怡; 邓学东

    2011-01-01

    Objective To investigate prenatal sonographic characteristics of fetal esophageal atresia ( EA ) in order to increase the prenatal detection rate of EA.Methods The sonographic findings in a total of 9 fetuses with EA which prenatally diagnosed by ultrasound and confirmed by autopsy or postnatal examination were retrospectively analyzed. Results Of all cases, 3 were detected with less than or equal to24-week gestation, in which 2 cases were shown with the non-visualization gastric bubble associated with obvious structural anomaly and chromosomal abnormalities , the autopsy results were isolated EA; one case had a small gastric bubble, polyhydramnios,double outlet right ventricle and single umbilical artery and the post-mortem examination result were EA with TEF. Other 6 cases were diagnosed beyond 28-week gestation, all fetuses had morphological changes of gastric bubble, 3 had non-visualization gastric bubble, 3 were shown with the small gastric bubble;4 were shown with the dilated proximal esophageal pouch;3 had fetal growth restriction ( FGR ). Six cases were found EA with TEF by postnatal examination. Conclusion Fetal EA has the characteristic ultrasonographic appearances. Prenatal sonography may be of important value in the diagnosis of fetal esophageal atresia.%目的 探讨胎儿食道闭锁(EA)产前声像图特征,以提高胎儿食道闭锁产前超声诊断符合率.方法 收集产前超声诊断、尸检或产后检查证实为胎儿食道闭锁病例共9例,分析其主要超声表现.结果 9例胎儿中3例孕周≤24周时检出,其中2例超声表现为胃泡不显示,伴有其他结构明显异常,染色体检查异常,尸检结果为单纯食道闭锁;1例超声表现为小胃泡、羊水过多伴右心室双出口、单脐动脉,尸检结果为食道闭锁伴食管气管瘘;其余6例胎儿孕周>28周时检出,其中3例超声表现为小胃泡、3例胃泡不显示;4例胎儿可见近端食道囊状扩张;3例羊水过多;3

  10. Analýza vybrané firmy

    OpenAIRE

    Peš, Martin

    2010-01-01

    Hlavním cílem diplomové práce je analyzovat skutečný a aktuální stav společnosti Laufen CZ, s.r.o. a navržení opatření. Jedná se o analýzu vnitřního a vnějšího prostředí společnosti. Jak se firma vyrovnala s dopady finanční a hospodářské krize nám sdělí analýzy SWOT, SLEPT, Quick test, Fundamentální analýza a Porterův model 5 sil.

  11. Hrvatsko nazivlje u analitičkoj kemiji

    OpenAIRE

    Kaštelan-Macan (ur.), M.

    2008-01-01

    U radu je prikazan povijesni razvoj prirodoslovnoga nazivlja s naglaskom na nazivlje u analitičkoj kemiji te današnje stanje i nastojanja da se to nazivlje ujednači i normira u skladu s hrvatskom jezičnom normom. Konačni cilj znanstvenoga projekta Hrvatsko nazivlje u analitičkoj kemiji, koji Ministarstvo znanosti, obrazovanja i športa podupire unutar znanstvenoga programa Hrvatska standardnojezična leksikologija i leksikografija jest izraditi cjeloviti enciklopedijski rječnik hrvatskoga anali...

  12. Anomaly detection in online social networks

    CERN Document Server

    Savage, David; Yu, Xinghuo; Chou, Pauline; Wang, Qingmai

    2016-01-01

    Anomalies in online social networks can signify irregular, and often illegal behaviour. Anomalies in online social networks can signify irregular, and often illegal behaviour. Detection of such anomalies has been used to identify malicious individuals, including spammers, sexual predators, and online fraudsters. In this paper we survey existing computational techniques for detecting anomalies in online social networks. We characterise anomalies as being either static or dynamic, and as being labelled or unlabelled, and survey methods for detecting these different types of anomalies. We suggest that the detection of anomalies in online social networks is composed of two sub-processes; the selection and calculation of network features, and the classification of observations from this feature space. In addition, this paper provides an overview of the types of problems that anomaly detection can address and identifies key areas of future research.

  13. Focal skin defect, limb anomalies and microphthalmia.

    NARCIS (Netherlands)

    Jackson, K.E.; Andersson, H.C.

    2004-01-01

    We describe two unrelated female patients with congenital single focal skin defects, unilateral microphthalmia and limb anomalies. Growth and psychomotor development were normal and no brain malformation was detected. Although eye and limb anomalies are commonly associated, clinical anophthalmia and

  14. Empaatia kogemus kunstiteoses kujutatud subjekti suhtes: fenomenoloogiline analüüs / Marge Paas

    Index Scriptorium Estoniae

    Paas, Marge, 1976-

    2015-01-01

    Analüüsitakse vaataja esteetilist kogemust kunstiteose suhtes. Autor tugineb Edith Steini empaatia kogemuse uurimusele ja fenomenoloogilisele uurimismeetodile. Vaataja empaatia kogemuse analüüs Maarit Murka maalisarjas "Hairpower" kujutatud subjekti suhtes

  15. Modifiable risk factors of obstetric anal sphincter injury in primiparous women

    DEFF Research Database (Denmark)

    Jango, Hanna; Langhoff-Roos, Jens; Rosthøj, Susanne;

    2014-01-01

    To determine modifiable risk factors and incidence of obstetric anal sphincter injury (OASIS) in primiparous women.......To determine modifiable risk factors and incidence of obstetric anal sphincter injury (OASIS) in primiparous women....

  16. An unusual presentation of Kabuki syndrome: clinical overlap with CHARGE syndrome.

    Science.gov (United States)

    Verhagen, Judith M A; Oostdijk, Wilma; Terwisscha van Scheltinga, Cecilia E J; Schalij-Delfos, Nicoline E; van Bever, Yolande

    2014-09-01

    Kabuki syndrome is a rare genetic disorder characterized by intellectual disability and multiple congenital anomalies, including short stature, peculiar facial appearance, skeletal anomalies, a variety of visceral malformations and abnormal dermatoglyphic patterns. We describe a case of Kabuki syndrome presenting with atypical features, consisting of bilateral microphthalmia, coloboma, anal atresia and panhypopituitarism, showing considerable phenotypic overlap with CHARGE syndrome. This report demonstrates that clinical follow-up and molecular genetic testing can be useful for establishing the correct diagnosis. PMID:24862881

  17. Anomaly Constraints on Monopoles and Dyons

    OpenAIRE

    Csaki, Csaba; Terning, John; Shirman, Yuri

    2010-01-01

    Fermions with magnetic charges can contribute to anomalies. We derive the axial anomaly and gauge anomalies for monopoles and dyons, and find eight new gauge anomaly cancelation conditions in a general theory with both electric and magnetic charges. As a byproduct we also extend the Zwanziger two-potential formalism to include the theta parameter, and elaborate on the condition for CP invariance in theories with fermionic dyons.

  18. Theory of Geological Anomaly in Remote Sensing

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Geological anomaly is geological body or complex body with obviously different compositions, structures or orders of genesis as compared with those in the surrounding areas. Geological anomaly, restrained by the geological factors closely associated with ore-forming process, is an important clue to ore deposits. The geological anomaly serves as a geological sign to locate ore deposits. Therefore, it is very important to study how to define the characteristics of geological anomaly and further to locate the changes in these characteristics. In this paper, the authors propose the geological anomaly based on the remote-sensing images and data, and expound systematically such image features as scale, size, boundary, morphology and genesis of geological anomalies. Then the authors introduce the categorization of the geological anomalies according to their geneses. The image characteristics of some types of geological anomalies, such as the underground geological anomaly, are also explained in detail. Based on the remote-sensing interpretation of these geological anomalies, the authors conclude that the forecasting and exploration of ore deposits should be focused on the following three aspects: (1) the analysis of geological setting and geological anomaly; (2) the analysis of circular geological anomaly, and (3) the comprehensive forecasting of ore deposits and the research into multi-source information.

  19. Fetal renal anomalies : diagnosis, management, and outcome

    NARCIS (Netherlands)

    Damen-Elias, Henrica Antonia Maria

    2004-01-01

    In two to three percent of fetuses structural anomalies can be found with prenatal ultrasound investigation. Anomalies of the urinary tract account for 15 to 20% of these anomalies with a detection rate of approximately of 90%. In Chapter 2, 3 and 4 we present reference curves for size and growth of

  20. HPV infection and intraepithelial lesions from the anal region: how to diagnose?

    OpenAIRE

    Newton Sérgio de Carvalho; Aliana Meneses Ferreira; Camila Caroline Tremel Bueno

    2011-01-01

    In the last years, the prevalence of HPV infection in the anal region has increased, especially in some groups like homosexual and HIV-positive people. Since this infection can be associated with the development of squamous anal cancer due to its progression from HPV infection to anal intraepithelial neoplasia (AIN) and finally to cancer, the screening and evaluation of these conditions are important. Anal cytology and high resolution anoscopy are good methods that are available and can be us...

  1. Survey of Anomaly Detection Methods

    Energy Technology Data Exchange (ETDEWEB)

    Ng, B

    2006-10-12

    This survey defines the problem of anomaly detection and provides an overview of existing methods. The methods are categorized into two general classes: generative and discriminative. A generative approach involves building a model that represents the joint distribution of the input features and the output labels of system behavior (e.g., normal or anomalous) then applies the model to formulate a decision rule for detecting anomalies. On the other hand, a discriminative approach aims directly to find the decision rule, with the smallest error rate, that distinguishes between normal and anomalous behavior. For each approach, we will give an overview of popular techniques and provide references to state-of-the-art applications.

  2. Schottky Anomaly and Hadronic Spectrum

    CERN Document Server

    Biswas, Aritra; Sinha, Nita

    2015-01-01

    We show that the hadronic "heat capacity" calculated as a function of temperature may be used to infer the possible presence of different scales underlying the dynamical structure of hadronic resonances using the phenomenon of Schottky anomaly. We first demonstrate this possibility with well known meson spectrum in various channels and comment on the possibility of using this method as a diagnostic to distinguish the exotic states.

  3. Calculation of the commutator anomaly

    International Nuclear Information System (INIS)

    It is well-known that, for the non-Abelian gauge theory coupled to the chiral fermions, which is described by a Lagrangian, the anomaly of the non-Abelian current is given by the descent form of the Chern-Simon five form; D*Ja = (-1/24π2)tr[d(AdA + 1/2A3)], where A = Aμdxμ = -itaAμadxμ. Recently, Faddeev argued that, for such an anomalous system, the gauge symmetry would be represented in a generalized sense of projective representation; V(g1)V(g2)Ψ(A) = exp[iα2(g1, g2; A)] X V(g1g2)Ψ(A), where Ψ(A) is the wave functional and the phase factor α2 depends on both the group elements and the gauge field configuration A. Here, it is convenient to consider the infinitesimal version of the above argument. The generator of infinitesimal gauge transformation in the temporal gauge A0 = 0 is given by Ga(x) = δiEia + gfabcAibEic - gΨ-barγ0taΨ, and the infinitesimal form of the previous product rule can be written as the equal time commutation relation; [Ga(x), Gb(y)] = ifabcGc(x)δ3(x-y) + ab(x,y,A), where is related to α2. In this equation, the first term is what is expected as the normal commutation relation of the generator, while the second term is an anomaly, which is designated here as the commutator anomaly. Then the present study concentrates on operations to determine the anomaly term. (Nogami, A.)

  4. Quantum tunneling and trace anomaly

    International Nuclear Information System (INIS)

    We compute the corrections, using the tunneling formalism based on a quantum WKB approach, to the Hawking temperature and Bekenstein-Hawking entropy for the Schwarzschild black hole. The results are related to the trace anomaly and are shown to be equivalent to findings inferred from Hawking's original calculation based on path integrals using zeta function regularization. Finally, exploiting the corrected temperature and periodicity arguments we also find the modification to the original Schwarzschild metric which captures the effect of quantum corrections.

  5. KIDNEY ANOMALIES: HORSE SHOE KIDNEY

    OpenAIRE

    Hemalatha; Komarabattina; Nageshwar Rao; Kotikala Prabhakara

    2015-01-01

    INTRODUCTION : Horse Shoe Kidney was first recognized during an autopsy by De Carpi in 1521. This anomaly consists of two distinct renal masses lying vertically on either side of the midline and connected at their respective lower poles by a parenchymatous or fibrous isthmus that crosses the mid pl ane of the body. This isthmus lies at the level of 4th lumbar vertebra just beneath the origin of inferior mesenteric ...

  6. Anomaly mediation in superstring theory

    International Nuclear Information System (INIS)

    We study anomaly mediated supersymmetry breaking in type IIB string theory and use our results to test the supergravity formula for anomaly mediated gaugino masses. We compute 1-loop gaugino masses for models of D3-branes on orbifold singularities with 3-form fluxes by calculating the annulus correlator of 3-form flux and two gauginos in the zero momentum limit. Consistent with supergravity expectations we find both anomalous and running contributions to 1-loop gaugino masses. For background Neveu-Schwarz H-flux we find an exact match with the supergravity formula. For Ramond-Ramond flux there is an off-shell ambiguity that precludes a full matching. The anomaly mediated gaugino masses, while determined by the infrared spectrum, arise from an explicit sum over UV open string winding modes. We also calculate brane-to-brane tree-level gravity mediated gaugino masses and show that there are two contributions coming from the dilaton and from the twisted modes, which are suppressed by the full T6 volume and the untwisted T2 volume respectively. (orig.)

  7. Anomaly mediation in superstring theory

    Energy Technology Data Exchange (ETDEWEB)

    Conlon, Joseph P. [Rudolf Peierls Center for Theoretical Physics, Oxford (United Kingdom); Balliol College, Oxford (United Kingdom); Goodsell, Mark [Deutsches Elektronen-Synchrotron (DESY), Hamburg (Germany); Palti, Eran [Centre de Physique Theoretique, Ecole Polytechnique, CNRS, Palaiseau (France)

    2010-08-15

    We study anomaly mediated supersymmetry breaking in type IIB string theory and use our results to test the supergravity formula for anomaly mediated gaugino masses. We compute 1-loop gaugino masses for models of D3-branes on orbifold singularities with 3-form fluxes by calculating the annulus correlator of 3-form flux and two gauginos in the zero momentum limit. Consistent with supergravity expectations we find both anomalous and running contributions to 1-loop gaugino masses. For background Neveu-Schwarz H-flux we find an exact match with the supergravity formula. For Ramond-Ramond flux there is an off-shell ambiguity that precludes a full matching. The anomaly mediated gaugino masses, while determined by the infrared spectrum, arise from an explicit sum over UV open string winding modes. We also calculate brane-to-brane tree-level gravity mediated gaugino masses and show that there are two contributions coming from the dilaton and from the twisted modes, which are suppressed by the full T{sup 6} volume and the untwisted T{sup 2} volume respectively. (orig.)

  8. Cause-Specific Colostomy Rates After Radiotherapy for Anal Cancer: A Danish Multicentre Cohort Study

    DEFF Research Database (Denmark)

    Sunesen, Kåre G; Nørgaard, Mette; Lundby, Lilli;

    2011-01-01

    In anal cancer, colostomy-free survival is a measure of anal sphincter preservation after treatment with radiotherapy or chemoradiotherapy. Failure to control anal cancer and complications of treatment are alternative indications for colostomy. However, no data exist on cause-specific colostomy...

  9. Comparison between Two Cecostomy Techniques for Treatment of Atresia Coli in Cattle and Buffalo Calves

    Directory of Open Access Journals (Sweden)

    Mohamad Abdelfattah Abdelrhman, Aiman Mahmoud Seddek*1 and Hossam Ahmad Bakr2

    2013-07-01

    Full Text Available The study aimed at determination of the cecostomy technique that will preserve the long-term maximal diameter of the created fistula. The subjects of the study were 79 newly born cattle and buffalo calves suffered from atresia coli, recti and ani. The calves were grouped into four groups for evaluation of incisional and herniation cecostomy techniques either on the right or left flank. With each technique, 4 cm diameter permanent fistula was created and evaluated at the 1stweek, and 1st, 4th and 8th months post-surgery. Both of the short and long-term complications were recorded and managed conservatively. Follow up could be achieved for 45 calves only and all of them showed stunted growth. The results revealed wider stoma in the calves operated by herniation technique, and in those operated on the right flank, moreover the operated cattle calves had wider stoma than buffalo calves. It is concluded that the right flank herniation technique has a beneficial effect on long-term preserving of a wider diameter of the created fistula.

  10. “Parenteral Nutrition Supplementation in Biliary Atresia Patients Listed for Liver Transplantation”

    Science.gov (United States)

    Sullivan, Jillian S; Sundaram, Shikha S; Pan, Zhaoxing; Sokol, Ronald J

    2011-01-01

    Objective To determine the impact of parenteral nutrition (PN) on outcomes in biliary atresia (BA) patients listed for liver transplantation (LTx). Study Design We retrospectively reviewed charts of all BA patients from 1990 through 2010 at our institution, s/p hepatoportoenterostomy, ≤ 36 months old, and listed for LTx. Initiation of PN was based on clinical indications. Results 25 PN and 22 non-PN subjects (74% female) were studied. Median PN initiation age was 7.7 months, mean duration 86 days, and mean PN energy supplied 77 kcal/kg/day. Prior to PN, triceps skinfold thickness (TSF) and mid-arm circumference (MAC) Z-scores were decreasing. After PN, TSF (p=0.003) and MAC (p<0.0001) improved significantly. The PN group had lower MAC and TSF than non-PN at time of LTx listing. Between listing and LTx, MAC and TSF improved in PN and worsened in non-PN such that both groups had the same Z-scores at LTx. PN group had a higher incidence of GI bleeding and ascites pre-LTx, but there was no difference in pre-LTx bacteremia, and post-LTx days in ICU and patient or graft survival. Conclusions PN improves nutritional status in malnourished BA patients awaiting LTx, which is associated with post-LTx outcomes comparable to those not requiring PN. PMID:21987426

  11. Serum adiponectin and transient elastography as non-invasive markers for postoperative biliary atresia

    Directory of Open Access Journals (Sweden)

    Udomsinprasert Wanvisa

    2011-02-01

    Full Text Available Abstract Background Biliary atresia (BA is a progressive inflammatory disorder of the extrahepatic bile ducts leading to the obliteration of bile flow. The purpose of this study was to determine serum adiponectin in BA patients and to investigate the relationship of adiponectin with clinical parameters and liver stiffness scores. Methods Sixty BA patients post Kasai operation and 20 controls were enrolled. The mean age of BA patients and controls was 9.6 ± 0.7 and 10.1 ± 0.7 years, respectively. BA patients were classified into two groups according to their serum total bilirubin (TB levels (non-jaundice, TB Results BA patients had markedly higher serum adiponectin levels (15.5 ± 1.1 vs. 11.1 ± 1.1 μg/ml, P = 0.03 and liver stiffness than controls (30.1 ± 3.0 vs. 5.1 ± 0.5 kPa, P P P r = 0.58, r = 0.46, and r = 0.60, P Conclusions Serum adiponectin and liver stiffness values were higher in BA patients compared with normal participants. The elevated serum adiponectin levels also positively correlated with the degree of hepatic dysfunction and liver fibrosis. Accordingly, serum adiponectin and transient elastography could serve as the useful non-invasive biomarkers for monitoring the severity and progression in postoperative BA.

  12. Transient elastography for predicting esophageal/gastric varices in children with biliary atresia

    Directory of Open Access Journals (Sweden)

    Siripon Nipaporn

    2011-04-01

    Full Text Available Abstract Background Transient elastography (TE is an innovative, noninvasive technique to assess liver fibrosis by measuring liver stiffness in patients with chronic liver diseases. The purpose of this study has been to explore the accuracy of TE and clinical parameters in predicting the presence of esophageal/gastric varices in children with biliary atresia (BA following portoenterostomy. Methods Patients with BA status post portoenterostomy and normal children were recruited. Splenomegaly and presence of EV/GV were determined by physical examination and endoscopy, respectively. Aspartate transaminase to platelet ratio index (APRI was used as a serum fibrosis marker. TE was performed by using FibroScan. Data was expressed as mean ± SD. Results Seventy-three BA patients (male:female = 32:41; age 9.11 ± 5.64 years and 50 normal controls (male:female = 19:31; age 11.00 ± 3.31 years were enrolled. The liver stiffness score of BA patients was significantly higher than that of normal controls (27.37 ± 22.48 and 4.69 ± 1.03 kPa; p Conclusions Transient elastography is a useful tool for predicting the presence of EV/GV. In addition, basic physical examination, routine biochemical and hematological tests, are still worthwhile and correlate well with the presence of EV/GV in patients with BA post portoenterostomy.

  13. Bone Health in a Nonjaundiced Population of Children with Biliary Atresia

    Directory of Open Access Journals (Sweden)

    Rachel A. Kramer

    2009-01-01

    Full Text Available Objectives. To assess bone health in a cohort of nonjaundiced children with biliary atresia (BA and the effect of growth and development on bone outcomes. Methods. Children ages one to eighteen years receiving care from Children's Hospital of Philadelphia were recruited. Each child was seen once and assessed for growth, pubertal development, concurrent medications, bilirubin, ALT, albumin, vitamin D status, bone mineral density (BMD, and bone mineral content (BMC of the lumbar spine and whole body. Results. BMD declined significantly with age, and upon further analysis with a well-phenotyped control cohort, it was found that BMC was significantly decreased for both lumbar spine and whole body, even after adjustment for confounding variables. An age interaction was identified, with older subjects having a significantly greater impairment in BMC. Conclusions. These preliminary results demonstrate that children with BA, including those without jaundice, are likely to have compromised bone health even when accounting for height and puberty, which are common confounding factors in chronic disease. Further investigation is needed to identify the determinants of poor bone mineral status and to develop strategies to prevent osteoporosis later in life.

  14. Ultrasonic diagnosis of biliary atresia: A retrospective analysis of 20 patients

    Institute of Scientific and Technical Information of China (English)

    Shi-Xing Li; Yao Zhang; Mei Sun; Bo Shi; Zhong-Yi Xu; Ying Huang; Zhi-Qin Mao

    2008-01-01

    AIM: To investigate the clinical value of ultrasonographic diagnosis of biliary atresia (BA), a retrospective analysis of the sonogram of 20 children with BA was undertaken.METHODS: Ultrasonography (US) was performed in 20 neonates and infants with BA, which was confirmed with cholangiography by operation or abdominoscopy. The presence of triangular cord, the size and echo of liver, the changes in empty stomach gallbladder and postprandial gallbladder were observed and recorded.RESULTS: The triangular cord could be observed at the porta hepatis (thickness: 0.3-0.6cm) in 10 cases. Smaller triangular cord (0.2-0.26cm) can be observed in 3 cases. The gallbladder was not observed in 2 cases, and 1 case showed a streak gallbladder without capsular space. The gallbladders of 15 cases were flat and small. The gallbladders of 2 cases were of normal size and appearance, however, there was no postprandial contraction. The livers of all cases showed hepatomegaly and hetreogeneous echogenicity. Statistical analysis was performed to compare the hepatomegaly and hetreogeneous echogenicity and the stage of hepatic fibrosis.CONCLUSION: The presence of the triangular cord at the porta hepatis is specific. However, it is not the only diagnostic criterion, since flat and small gallbladder and poor contraction are also of important diagnostic and differential diagnostic significance. The degree of hepatomegaly and hetreogeneous echogenicity is proportional with liver fibrosis, and able to indicate the duration of course and prognosis.

  15. There is no association between K469E ICAM-1 gene polymorphism and biliary atresia

    Institute of Scientific and Technical Information of China (English)

    Paisarn Vejchapipat; Naruemol Jirapanakom; Nutchanart Thawornsuk; Apiradee Theamboonlers; Voranush Chongsrisawat; Soottiporn Chittmittrapap; Yong Poovorawan

    2005-01-01

    AIM: To determine whether there was an association between inter-cellular adhesion molecule-1 (ICAM-1) gene polymorphism and biliary atresia (BA), and to investigate the relationship between serum soluble ICAM-1 (sICAM-1)and clinical outcome in BA patients after surgical treatment.METHODS: Eighty-three BA patients and 115 normal controls were genotyped. K469EICAM-1 polymorphism was analyzed using PCR assay. Serum sICAM-1 was determined using ELISA method from 72 BA patients. In order to evaluate the association between these variables and their clinical outcome, the patients were categorized into two groups:patients without jaundice and those with persistent jaundice.RESULTS: There were no significant differences between BA patients and controls in terms of gender, K469E ICAM-1genotypes, and alleles. The proportion of patients having serum sICAM-1 ≥3 500 ng/mL in persistent jaundice group was significantly higher than that in the other group. In addition, there was no association between K469EICAM-1polymorphism and the status of jaundice in BA patients after Kasai operation.CONCLUSION: ICAM-1 possibly plays an important and active role in the disease progression. However, the process is not associated with genetic variation of K469EICAM-1 polymorphism.

  16. Distinct Plasma Bile Acid Profiles of Biliary Atresia and Neonatal Hepatitis Syndrome.

    Science.gov (United States)

    Zhou, Kejun; Wang, Jun; Xie, Guoxiang; Zhou, Ying; Yan, Weihui; Pan, Weihua; Che, Yanran; Zhang, Ting; Wong, Linda; Kwee, Sandi; Xiao, Yongtao; Wen, Jie; Cai, Wei; Jia, Wei

    2015-11-01

    Biliary atresia (BA) is a severe chronic cholestasis disorder of infants that leads to death if not treated on time. Neonatal hepatitis syndrome (NHS) is another leading cause of neonatal cholestasis confounding the diagnosis of BA. Recent studies indicate that altered bile acid metabolism is closely associated with liver injury and cholestasis. In this study, we systematically measured the bile acid metabolome in plasma of BA, NHS, and healthy controls. Liver bile acids were also measured using biopsy samples from 48 BA and 16 NHS infants undergoing operative cholangiography as well as 5 normal adjacent nontumor liver tissues taken from hepatoblastoma patients as controls. Both BA and NHS samples had significantly elevated bile acid levels in plasma compared to normal controls. BA patients showed a distinct bile acid profile characterized by the higher taurochenodeoxycholic acid (TCDCA) level and lower chenodeoxycholic acid (CDCA) level than those in NHS patients. The ratio of TCDCA to CDCA in plasma was significantly higher in BA compared to healthy infants (p BSEP), and multidrug resistant protein 3 (MDR3) in BA compared to NHS. Taken together, the plasma bile acid profiles are distinct in BA, NHS, and normal infants, as characterized by the ratio of TCDCA/CDCA differentially distributed among the three groups of infants. PMID:26449593

  17. Distribution of Interstitial Cells of Cajal in the Esophagus of Fetal Rats with Esophageal Atresia

    Directory of Open Access Journals (Sweden)

    Caner Isbir

    2016-04-01

    Full Text Available Aim: Scarcity of the interstitial cells of Cajal (ICC is related to motility disorders. In the study, we aimed to evaluate the number and density of ICCs in the fetal rat esophagus in the adriamycin - esophageal atresia (EA model. Material and Method: Rat fetuses were divided into three groups as a control, adriamycin group without EA and adriamycin group with EA. Four doses of adriamycin, 2 mg/kg each, were injected intraperitoneally to the adriamycin group rats between on 6 and 9 days of gestation. The presence of ICCs in the esophagus of the rat fetuses was determined by using an immunohistochemistry technique (c-kit, CD117. The average numbers of ICCs were calculated with microscopic evaluation by using a visual scoring system (range1 to 3. Results: Seven fetuses were included in each group. The ICCs score 3 distributions of fetuses were 5 (72% fetuses in the control group, 3 (43% fetuses in the adriamycin group without EA, 1 (14% fetus in the adriamycin group with EA. It have been found that there was a marked reduction of ICCs distribution in the adriamycin group with EA compared to control group (p 0.05. Discussion: ICCs density was significantly decreased in the rat fetuses with EA compared to the fetuses without EA. These findings support the idea that ICCs density may be congenitally abnormal in EA. This may be led to dismotility seen in the operated esophagus due to EA.

  18. Primary radiation therapy in the treatment of anal carcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Cantril, S.T. (Children' s Hospital of San Francisco, CA); Green, J.P.; Schall, G.L.; Schaupp, W.C.

    1983-09-01

    From 1966 to 1981, 47 patients with a diagnosis of anal carcinoma were irradiated. This group was composed of 23 males and 24 females, with age ranging from 38 to 84 years (average 64.4 years). Five patients were treated preoperatively and 34 were treated definitively with cancericidal doses of irradiation. Acute radiation reactions requiring a rest-break were noted in 28% of patients, but all were managed as outpatients without untoward chronic sequelae. Chronic complications were noted in 13 patients, including two patients who required colostomy for severe anal stenosis and two who required A-P resection for large painful ulcers. Twenty-eight of 35 patients (80%) treated with irradiation alone have remained locally controlled without further treatment. An additional four have been salvaged by surgery. Only three patients had interstitial implants as part of their treatment course. Actuarial survival at five years for the N/sub 0/ patients and the group as a whole are 95.6 and 79.3%, respectively. It is concluded that external beam irradiation alone, properly fractionated to cancericidal doses, can control anal carcinoma with acceptable morbidity rates and without the use of either chemotherapy or interstitial implants in most cases. There is also a strong correlation suggesting that anal intercourse and male homosexuality play a significant role in the etiology of this disease.

  19. Aspectos funcionales de la psicoterapia analítico funcional.

    Directory of Open Access Journals (Sweden)

    Javier Virués Ortega

    2003-01-01

    Full Text Available Se considera una nueva psicoterapia de orientación conductual: la Psicoterapia Analítico Funcional. Se describen brevemente sus fundamentos teóricos, metodológicos y aplicados. Se discuten sus aspectos funcionales desde un punto de vista contextual.

  20. Analýza vybrané firmy

    OpenAIRE

    Zavoralová, Eva

    2012-01-01

    Diplomová práce je zaměřena na analýzu realitní společnosti M&M reality holding, a.s.. V rámci mapování společnosti se zaměřuji na rozbor oborového okolí pomocí PEST analýzy, Porterův model pěti konkurenčních sil, SWOT analýzu a finanční analýzu. V závěru práce navrhuji doporučení, která by měla vést ke zlepšení současné situace. The master´s thesis is focused on the analysis of real estate company M&M reality holding, a.s.. The mapping of the analysis focuses on the professional environme...