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Sample records for experimental biliary atresia

  1. Biliary atresia

    Directory of Open Access Journals (Sweden)

    Sinha C

    2008-01-01

    Full Text Available Biliary atresia (BA is a cholangiodestructive disease affecting biliary tract, which ultimately leads to cirrhosis, liver failure and death if not treated. The incidence is higher in Asian countries than in Europe. Up to 10% of cases have other congenital anomalies, such as polysplenia, asplenia, situs inversus, absence of inferior vena cava and pre-duodenal portal vein, for which we have coined the term Biliary Atresia Splenic Malformation (BASM syndrome. For these infants the aetiology lies within the first trimester of gestation. For others affected with BA, aetiology is more obscure and perinatal destruction of fully-formed ducts perhaps by the action of hepatotropic viruses has been suggested. Whatever the cause, the lumen of the extrahepatic duct is obliterated at a variable level and this forms the basis for the commonest classification (Types I, II, III. All patients with BA present with varying degree of conjugated jaundice, pale non-pigmented stools and dark urine. Key diagnostic tests include ultrasonography, biochemical liver function tests, viral serology, and (in our centre a percutaneous liver biopsy. In some centres, duodenal intubation and measurement of intralumenal bile is the norm. Currently BA is being managed in two stages. The first stage involves the Kasai operation, which essentially excises all extrahepatic biliary remnants leaving a transected portal plate, followed by biliary reconstruction using a Roux loop onto that plate as a portoenterostomy. If bile flow is not restored by Kasai procedure or life-threatening complications of cirrhosis ensue then consideration should be given to liver transplantation as a second stage. The outcome following the Kasai operation can be assessed in two ways: clearance of jaundice to normal values and the proportion who survive with their native liver. Clearance of jaundice (< 2 mg/dL or < 34 µmol/L after Kasai has been reported to be around 60%, whereas five years survival with

  2. Biliary atresia Atresia de las vías biliares.

    OpenAIRE

    Pastor Thomas Olivares; Ramón Villamil Martínez; Miurkis Endis Miranda

    2005-01-01

    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 Na...

  3. Biliary atresia. A surgical perspective.

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    Ohi, R

    2000-11-01

    The combination of portoenterostomy with subsequent liver transplantation is the treatment of choice for patients with biliary atresia. It is important, however, to attempt to keep the patient's own organ by continuing efforts to achieve the best possible results with portoenterostomy. Additional basic research, perhaps concerning on the role of cytokines and apoptosis in the control of biliary atresia, may provide insight into possible new medical strategies for treating patients with biliary atresia. For example, in addition to portoenterostomy, control of apoptosis at various cellular levels and of bile duct cell proliferation and maturation by manipulation of the growth factors and cytokines may become part of future treatment modalities. Another direction of research should be the control of fibrogenesis, which might be accomplished by blocking TGF-beta 1 and platelet-derived growth factor and by HGF gene therapy. The author's current strategy for surgical treatment for patients with biliary atresia include (1) early diagnosis, including prenatal diagnosis and broader use of mass screening programs, (2) hepatic portoenterostomy, without stoma formation; (3) close postoperative care, especially for prevention of postoperative cholangitis; (4) revision of portoenterostomy only in selected cases; (5) early liver transplantation in patients with absolutely failed portoenterostomy; (6) avoidance of laparotomy for the treatment of esophageal varices and hypersplenism; (7) consideration of exploratory laparotomy or primary liver transplantation for patients with advanced liver disease at the time of referral. The development of new treatment modalities based on the understanding of the pathogenesis of the disease, and especially on the biology of intrahepatic bile ducts and hepatic fibrosis, is essential.

  4. [Extrahepatic biliary atresia: diagnostic methods].

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    Cauduro, Sydney M

    2003-01-01

    To emphasize the importance of precocious diagnosis of extrahepatic biliary atresia and its direct relationship with the surgical re-establishment of the biliary flow before the second month of life. To discuss several complementary methods with the aim of selecting the ones that present better evidence, and avoiding delays in diagnosis and worse prognostic. Bibliographical researching regarding the period of 1985-2001, in Medline and MdConsult, using the following key words: neo-natal cholestasis; extrahepatic biliary atresia; neo-natal hepatitis. National and foreign articles were also elected based on the bibliography of consulted publications, and when necessary, for better understanding of the theme, opinions emitted in theses and textbooks were referred. The revision of the consulted bibliography led to the assumption that early diagnosis of EHBA and surgical treatment to reestablish the biliary flow up to 60 days of life are fundamental in order to achieve good results. Among several complementary methods of diagnosis, cholangiography by MR, US and the hepatic biopsy are the ones that provide the largest success indexes. The referring of patients bearers of EHBA to centers of references in Brazil, is still made tardily, probably due to lack of enlightenment of the doctors of primary attention, allied to bureaucratic and technological difficulties. The experience in England in relation to the "Yellow Alert" program, allowed that the number of children referred to surgical treatment before the 60 days of life increased significantly. Among the complementary methods, the MR cholangiography, ultrasonography and hepatic biopsy should be used, depending on the technological resources of the diagnosis units.

  5. Biliary atresia: Where do we stand now?

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    Govindarajan, Krishna Kumar

    2016-12-28

    The pathway from clinical suspicion to establishing the diagnosis of biliary atresia in a child with jaundice is a daunting task. However, investigations available help to point towards the correct diagnosis in reasonable time frame. Imaging by Sonography has identified several parameters which can be of utility in the diagnostic work up. Comparison of Sonography with imaging by Nuclear medicine can bring out the significant differences and also help in appropriate imaging. The battery of Biochemical tests, available currently, enable better understanding of the line-up of investigations in a given child with neonatal cholestasis. Management protocols enable standardized care with optimal outcome. The place of surgical management in biliary atresia is undisputed, although Kasai procedure and primary liver transplantation have been pitted against each other. This article functions as a platform to bring forth the various dimensions of biliary atresia.

  6. ETIOLOGY, PATHOGENESIS AND MORPHOLOGY OF BILIARY ATRESIA

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    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. 

  7. European biliary atresia registries : Summary of a symposium

    NARCIS (Netherlands)

    Petersen, C.; Harder, D.; Abola, Z.; Alberti, D.; Becker, T.; Chardot, C.; Davenport, M.; Deutschmann, A.; Khelif, K.; Kobayashi, H.; Kvist, N.; Leonhardt, J.; Melter, M.; Pakarinen, M.; Pawlowska, J.; Petersons, A.; Pfister, E. -D.; Rygl, M.; Schreiber, R.; Sokol, R.; Ure, B.; Veiga, C.; Verkade, H.; Wildhaber, B.; Yerushalmi, B.; Kelly, D.

    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. Biliary atresia in Vietnam: Management and the burden of disease.

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    Liu, Max Bolun; Huong, Thuy Bui; Hoang, Xuyen; Doan, Lan; Trinh, Shauna; Anh Nguyen, Hoa Pham; Thanh Le, Hai; Holterman, Ai-Xuan

    2017-02-01

    Biliary atresia is an idiopathic, neonatal liver disease of the bile ducts. The natural evolution of biliary atresia is known in developed countries. This study describes the clinical course of biliary atresia in Vietnam, a developing country. Chart reviews were undertaken of patients treated with or without the Kasai procedure between January 2010 and July 2013 at a children's hospital in Vietnam. Of 287 children with biliary atresia, 149 (52%) were treated without the Kasai procedure and 138 (48%) were treated with the Kasai procedure. Median age at diagnosis was 2.4 months for children treated without the Kasai procedure vs 2.3 months for those treated with the procedure. The percentages of patients in the group treated without the Kasai procedure presenting at 6 months of age were 31%, 35%, 15%, 10%, and 9%, respectively, compared to those treated with the Kasai procedure at 36% (P = .38), 44% (P = .12), 16% (P = 1.0), 4% (P = .037), and 0% (P Vietnam. The majority of biliary atresia in Vietnam remains untreated despite early presentation and reasonable outcomes after a Kasai procedure relative to Western countries. These data illustrate the high health care burden for biliary atresia in Vietnam and the need to improve education about biliary atresia and its treatment. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. [Biliary atresia - signs and symptoms, diagnosis, clinical management].

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    Orłowska, Ewa; Czubkowski, Piotr; Socha, Piotr

    Biliary atresia is a chronic cholangiopathy leading to progressive fibrosis of both intra- and extrahepatic bile ducts. The cause of the condition is unknown. Fundamental management of biliary atresia is surgical intervention and the outcomes of the treatment depend on the child's age with best results when performed within the first 2 months of life. Thus, the main role of pediatric healthcare is an urgent differential diagnosis and prompt qualification for the surgery, optimal postoperative management and early qualification for the liver transplantation in patients with persistent cholestasis. The authors discuss the clinical presentation, diagnosis and management of biliary atresia.

  10. 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...... that the detrimental cholestatic liver injury, later characteristic of BA, only begins from the time of birth despite a prenatal occlusive biliary pathology. It may be that tissue injury only occurs with the onset of the perinatal bile surge initiating periductal bile leakage and the triggering of an inflammatory...

  11. Long survival ( 21 years) after portoenterostomy for biliary atresia: A ...

    African Journals Online (AJOL)

    Long term survival for decades after portoenterostomy (Kasai procedure) for biliary atresia is rare and the association of portoenterostomy with liver cirrhosis is well known. Not much attention was given in the evaluation of the imaging features of cirrhosis caused by portoenterostomy as received by other known usual ...

  12. Biliary Atresia – An Easily Missed Cause of Jaundice amongst ...

    African Journals Online (AJOL)

    ... presents with jaundice, acholic stools / dark urine and hepatomegaly. This disease rapidly leads to liver cirrhosis and liver failure if untreated surgically. The main objective was to establish the epidemiology of patients presenting with biliary atresia and immediate surgical outcome. Methods: A review of a prospective data ...

  13. Increased expression of intercellular adhesion molecules in biliary atresia.

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    Dillon, P.; Belchis, D.; Tracy, T.; Cilley, R.; Hafer, L.; Krummel, T

    1994-01-01

    The expression of the inflammatory adhesion molecules intercellular adhesion molecule-1, vascular cell adhesion molecule-1, and endothelial leukocyte adhesion molecule-1, was studied in six infants with biliary atresia using an immunoperoxidase technique on frozen sections. Controls consisted of five patients with various conditions including total parenteral nutrition-induced cholestasis, choledochal cyst, viral hepatitis, metastatic carcinoma, and thrombotic thrombocytopenic purpura. None o...

  14. Thirty-four years' experience with biliary atresia in Denmark

    DEFF Research Database (Denmark)

    Kvist, N; Davenport, M

    2011-01-01

    Biliary atresia (BA) is a rare disease in Denmark (population ~¿5.5 million) and there has been some controversy on how smaller countries should manage such conditions to obtain the best possible outcomes. The aim of this study was to evaluate the efficacy of primary surgery (i.¿e., Kasai...

  15. Thirty-four years' experience with biliary atresia in Denmark

    DEFF Research Database (Denmark)

    Kvist, N; Davenport, M

    2011-01-01

    Biliary atresia (BA) is a rare disease in Denmark (population ∼ 5.5 million) and there has been some controversy on how smaller countries should manage such conditions to obtain the best possible outcomes. The aim of this study was to evaluate the efficacy of primary surgery (i. e., Kasai portoen...

  16. Anaesthesia for biliary atresia and hepatectomy in paediatrics

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    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.

  17. Nonparenchymal liver cells and granulomas during lamprey biliary atresia.

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    Youson, J H; Sargent, P A; Yamamoto, K; Ogilvie, D; Fisher, M M

    1987-06-01

    Transmission (thin sections and freeze-fracture replicas) and scanning electron microscopy were used to describe the nonparenchymal liver cells during the seven (1-7) stages of metamorphosis in the sea lamprey, Petromyzon marinus L., when bile ducts and canaliculi degenerate. The biliary atresia is accompanied by an increased diameter of fenestrae in the endothelium, an active phagocytosis by Kupffer cells in the sinusoids, and large lipid inclusions in perisinusoidal lipocytes (fat-storing or Ito cells). Plasma-like cells and foci of nonparenchymal cells (granulomas) are present in the liver interstitium during at least four stages of metamorphosis. The fenestrae in the sinusoidal wall are wider (up to 2.8-micron diameter) than normally reported for vertebrate livers but are likely a reflection of the morphogenetic and physiological events and consequences of the biliary atresia. Kupffer cells are involved in an extensive erythrophagocytosis, the storage of iron, and perhaps the incorporation of cellular components from hepatocytes. Lipocytes are the vitamin A-storing cells of the transforming liver and may be responsible for some perisinusoidal fibrosis. Granulomas are present during stages 3-6 and are focal areas where mononuclear leukocytes (lymphocytes and plasmalike cells), macrophages, and neutrophils have infiltrated the hepatic parenchyma. The function of the granulomas is not known; but their presence may be related to the porous nature of the sinusoidal wall, the tissue degeneration, and/or the physiological change (e.g., bile stasis) during biliary atresia.

  18. 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

    INTRODUCTION: Biliary atresia is the leading cause of liver transplantation in children. It affects 1:15,000 in Denmark. With a national birth rate of 60,000, four children are born every year with biliary atresia. Early correction of biliary obstruction is essential to prevent fatal biliary...... 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....... This percentage value has caused diagnostic trouble over the years. The objective of the present study was to investigate the possibility of changing the recommendations. METHODS: This was a retrospective analysis of the medical records of children operated for biliary atresia in the 1993-2012 period. RESULTS...

  19. Cellular and Humoral Autoimmunity Directed at Bile Duct Epithelia in Murine Biliary Atresia

    OpenAIRE

    Mack, Cara L.; Tucker, Rebecca M.; Lu, Brandy R.; Sokol, Ronald J.; Fontenot, Andrew P.; Ueno, Yoshiyuki; Gill, Ronald G.

    2006-01-01

    Biliary atresia is an inflammatory fibrosclerosing lesion of the bile ducts that leads to biliary cirrhosis and is the most frequent indication for liver transplantation in children. The pathogenesis of biliary atresia is not known; one theory is that of a virus-induced, subsequent autoimmune-mediated injury of bile ducts. The aim of this study was to determine whether autoreactive T cells and autoantibodies specific to bile duct epithelia are present in the rotavirus (RRV)- induced murine mo...

  20. Silencing of the rotavirus NSP4 protein decreases the incidence of biliary atresia in murine model.

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    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.

  1. Less-invasive MR indices of clinically evident esophageal variceal bleeding in biliary atresia patients

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    Yuan Heng Mo

    2012-09-01

    Conclusion: Less-invasive indices, including the corrected splenic length platelet ratio and the splenic volume index-to-platelet count ratio, may be valuable predictors of esophageal variceal bleeding in patients with biliary atresia.

  2. The Role of ARF6 in Biliary Atresia.

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    Mylarappa Ningappa

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

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

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    Yu-Wen Chung-Davidson

    2015-01-01

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

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

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    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.

  5. Ultrasonographic differentiation of biliary atresia and neonatal hepatitis: Reestablishment of size criteria of the gallbladder

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    Kim, Woo Sun; Cheon, Jung Eun; Koh, Young Hwan; Kim, In One; Yeon, Kyung Mo [Seoul National University College of Medicine and Institude of Radiation Medicion, Seoul (Korea, Republic of)

    2001-12-15

    To reestablish the size criterion of the gallbladder on ultrasonography (US) for the differentiation diagnosis of biliary atresia from neonatal hepatitis. Abdominal US ws performed in 201 patients with jaundice and 40 patients without evidence of jaundice or hepatobiliary illness (all with the age less than 4 months). US was performed in fasting (fasting for at least 4 hours) to measure the length of the gallbladder and calculated the area of the gallbladder lumen. The morphology of the gallbladder was classified into three types: normal, elongated and atretic. To evaluate the contractibility of the gallbladder, the length of the gallbladder and area of the gallbladder lumen was again measured 1 hour after feeding. The final diagnosis included biliary atresia in 79 patients and neonatal hepatitis in 83 patients. Differences in the length, area, and morphology of the gallbladder were statistically significant among three groups, the normal group, neonatal hepatitis group and biliary atresia group (length and area of gallbladder; normal group>neonatal hepatitis>biliary atresia). The differences in the length and area of gallbladder between pre- and postmeal state were statistically significant in the normal and neonatal hepatitis groups whereas those of biliary atresia were not significant (p=0.85). When the empirical size criterion of the gallbladder (<15 mm in length) was applied, the sensitivity, specificity and diagnostic accuracy for the differential diagnosis of biliary atresia from hepatitis were 52%, 82%, and 67%, respectively. Meanwhile, if the area criterion(<30 mm{sup 2} in area) was applied, the sensitivity, the specificity and diagnostic accuracy were 67%, 85%, and 75%, respectively. Ultrasonographic evaluation of the morphology as well as size of the gallbladder are helpful in the differential diagnosis of biliary atresia from neonatal hepatitis. Therefore, since the measurement of the area of gallbladder lumen on US reflect both size and morphology of

  6. Biliary Atresia: Indications and Timing of Liver Transplantation and Optimization of Pre-Transplant Care

    Science.gov (United States)

    Sundaram, Shikha S.; Mack, Cara L.; Feldman, Amy G.; Sokol, Ronald J.

    2016-01-01

    Biliary Atresia is a progressive, fibro-obliterative disorder of the intra and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation. Indications for liver transplant in biliary atresia include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extra-hepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for liver transplantation. Optimal pre-transplant management of these potentially life threatening complications and maximizing nutrition and growth require the expertise of a multi-disciplinary team with experience caring for biliary atresia. The timing of transplant for biliary atresia requires careful consideration of the potential risk of transplant versus the survival benefit at any given stage of disease. Children with biliary atresia often experience long wait times for transplant unless exception points are granted to reflect severity of disease. Family preparedness for this arduous process is therefore critical. PMID:27650268

  7. Technical innovation for noninvasive and early diagnosis of biliary atresia: the ultrasonographic "triangular cord" sign.

    Science.gov (United States)

    Park, W H; Choi, S O; Lee, H J

    2001-01-01

    In this article, we introduce our experience regarding a new and noninvasive diagnostic tool, using ultrasonography, for the early and definite diagnosis of biliary atresia. We have focussed on the ultrasonographic image of the cone-shaped periportal fibrous mass in infants with biliary atresia since 1992, and have finally identified a triangular or band-like periportal echogenicity ("triangular cord" sign), mainly cranial to the portal vein. Based on our experience and other reports from Japan and Singapore, the ultrasonographic triangular cord sign is a simple, time-saving, highly reliable, and definite tool in the diagnosis of biliary atresia from infantile intrahepatic cholestasis, representing a positive predictive value greater than 95%. We have proposed a new diagnostic strategy in the evaluation of infantile cholestasis, with emphasis on the ultrasonographic triangular cord sign.

  8. Physical development and cognitive performance in a monozygotic twins for biliary atresia: Report of a case and literature reviewing

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    Yang Chen

    2016-08-01

    Full Text Available A case of monozygotic twins, one of whom suffered from biliary atresia and the other one was apparently healthy, is described herein. Children with biliary atresia appear to be vulnerable to developmental lags in the physical and cognitive performance, due to malnutrition and deteriorated liver function. In our case where the child with biliary atresia, who underwent a successful kasai operation, has achieved comparable growth development and cognitive performance in comparision with her sister, which suggest that the early diagnosis and timly kasai operation could allow children with biliary artesia to grow well in the short-term following time.

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

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

    2016-01-01

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

  10. Endotoxin and CD14 in the progression of biliary atresia

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    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.

  11. Preterm Infants With Biliary Atresia : A Nationwide Cohort Analysis From The Netherlands

    NARCIS (Netherlands)

    van Wessel, Daan B E; Boere, Thomas; Hulzebos, Christian V.; de Kleine, Ruben H J; Verkade, Henkjan J

    2017-01-01

    Objectives: Biliary atresia (BA) occurs in 0.54 of 10.000 of overall live births in the Netherlands. BAhas an unfavorable prognosis: Methods: Retrospective study including Dutch preterm infants treated for BA. Parameters included gestational age, congenital anomalies, age at KPE, days between first

  12. IL‐6, TNF‐α, IL‐10, and nutritional status in pediatric patients with biliary atresia

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    Maria Ines de Albuquerque Wilasco

    2017-09-01

    Conclusion: The authors suggest that, in patients with cirrhosis secondary to biliary atresia, IL‐6 could be used as a possible supporting biomarker of deficient nutritional status and elevated IL‐10 levels could be used as a possible early‐stage supporting biomarker of deteriorating nutritional status.

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

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

    2015-11-01

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

  14. MRI-based decision tree model for diagnosis of biliary atresia.

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    Kim, Yong Hee; Kim, Myung-Joon; Shin, Hyun Joo; Yoon, Haesung; Han, Seok Joo; Koh, Hong; Roh, Yun Ho; Lee, Mi-Jung

    2018-02-23

    To evaluate MRI findings and to generate a decision tree model for diagnosis of biliary atresia (BA) in infants with jaundice. We retrospectively reviewed features of MRI and ultrasonography (US) performed in infants with jaundice between January 2009 and June 2016 under approval of the institutional review board, including the maximum diameter of periportal signal change on MRI (MR triangular cord thickness, MR-TCT) or US (US-TCT), visibility of common bile duct (CBD) and abnormality of gallbladder (GB). Hepatic subcapsular flow was reviewed on Doppler US. We performed conditional inference tree analysis using MRI findings to generate a decision tree model. A total of 208 infants were included, 112 in the BA group and 96 in the non-BA group. Mean age at the time of MRI was 58.7 ± 36.6 days. Visibility of CBD, abnormality of GB and MR-TCT were good discriminators for the diagnosis of BA and the MRI-based decision tree using these findings with MR-TCT cut-off 5.1 mm showed 97.3 % sensitivity, 94.8 % specificity and 96.2 % accuracy. MRI-based decision tree model reliably differentiates BA in infants with jaundice. MRI can be an objective imaging modality for the diagnosis of BA. • MRI-based decision tree model reliably differentiates biliary atresia in neonatal cholestasis. • Common bile duct, gallbladder and periportal signal changes are the discriminators. • MRI has comparable performance to ultrasonography for diagnosis of biliary atresia.

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

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    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

  16. Early diagnosis of extrahepatic biliary atresia in an open-access medical system.

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    Justin Hollon

    Full Text Available INTRODUCTION: Biliary atresia (BA is the most common cause of cholestatic jaundice in infancy. Early diagnosis and surgical management, ideally before 60 days of age, result in improved outcomes. We aimed to determine the age at diagnosis of BA in the Military Health System (MHS and to compare the age at diagnosis by access to care models. We hypothesized that children with BA receiving primary care in military facilities have an earlier age at diagnosis due to decreased economic and access barriers. METHODS: Data for all Tricare enrollees born in fiscal years 2004-2008 with a diagnosis of BA were extracted from MHS databases. Non-parametric tests, Kaplan-Meier curves and log rank tests compared differences in age at diagnosis by type of primary care facility, gender, prematurity and presence of additional anomalies. RESULTS: 64 subjects were identified within the five year period. Median age at diagnosis was 40 days [range 1-189], with 67% diagnosed by 60 days and 80% by 90 days. 45 (70% received civilian primary care within the MHS. There was no difference in the median age at diagnosis between subjects in the MHS with civilian primary care vs. military primary care (37 days [1-188] vs. 46 days [1-189]; p=0.58. CONCLUSION: In the MHS, two-thirds of infants with biliary atresia are diagnosed prior to 60 days of life. Gender, prematurity or presence of additional anomalies do not affect the timing of diagnosis. Civilian and military primary care models within the MHS make timely diagnoses of biliary atresia at equivalent rates.

  17. Bile duct ligature in young rats: A revisited animal model for biliary atresia

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    Matias Garrido

    2017-09-01

    Full Text Available Biliary atresia leads to cirrhosis in the vast majority of patients and constitutes the first cause of paediatric liver transplantation. Animal models allow us to understand the molecular basis and natural history of diseases. The aim of this study is to describe a surgically created animal model of biliary atresia with emphasis in long-term liver function. Forty-two 3-week-old Sprague-Dawley rats were randomly divided into two groups: bile duct ligature (BDL and control. The animals were sacrificed on the 2nd, 4th, and 6th postoperative weeks. Blood samples were collected for liver function analysis. The spleen to body weight ratio was determined. Histopathological examination of liver tissue was performed by hematoxylin-eosin and Sirius red staining. Collagen quantification was determined by using colorimetric digital image analysis and was expressed as a percentage of total liver tissue area. Quantitative real-time polymerase chain reaction was performed to analyse gene expression levels of transforming growth factor-β1 (Tgfb1 and apeline (Apln genes. Statistical analysis was performed where P<0.05 was considered significant. Animals from BDL group developed increasing cholestasis with clinical and laboratory features. Splenomegaly was detected at 4th and 6th week (P<0.05. Histological evaluation of the liver showed ductular reaction, portal fibrosis and bile plugs. Collagen area to total liver tissue area had a median of 2.5% in the control group and 6.5 %, 14.3 % and 37.7 % in BDL rats at 2nd, 4th and 6th weeks respectively (P<0.001. Tgfb1 mRNA expression level was significantly higher at 6th week (P<0.001 in BDL group when compared to control. Apln mRNA expression level was significantly higher at 4th and 6th week (P<0.001 and showed a positive linear correlation (r = 0.975, P<0.05 in BDL group when compared to control. Bile duct ligature in young rats is an animal model that recreates clinical, laboratory, histological and molecular

  18. Clinical and pathological challenges in the diagnosis of late-onset biliary atresia: four case studies

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    J.P.U. Fontenele

    2016-03-01

    Full Text Available Biliary atresia (BA is classically described at the neonatal age. However, rare cases of BA in older infants have also been reported. We report four cases of late-onset BA in infants older than 4 weeks (3 males, 1 female, and describe the diagnostic and management difficulties. One of the cases had a late-onset (29 weeks presentation with a successful surgical procedure. We highlight the importance of this unusual differential diagnosis in infants with cholestatic syndrome, who may benefit from Kasai surgery, regardless of age.

  19. Atresia biliar extra-hepática: métodos diagnósticos Extrahepatic biliary atresia: diagnostic methods

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    Sydney M. Cauduro

    2003-04-01

    Full Text Available OBJETIVO: enfatizar a importância do diagnóstico precoce da atresia biliar extra-hepática e sua relação direta com o restabelecimento cirúrgico do fluxo biliar antes do segundo mês de vida, discutindo os diversos métodos complementares utilizados, objetivando selecionar os de maior evidência, evitando retardo diagnóstico e, conseqüentemente, piora do prognóstico. MÉTODO: pesquisa bibliográfica referente ao período de 1985 a 2001, no Medline e MdConsult, através das palavras-chaves: colestase neonatal, atresia biliar extra-hepática e hepatite neonatal. Também foram selecionados artigos nacionais e estrangeiros a partir da bibliografia das publicações consultadas e, quando necessário, para melhor entendimento do tema, opiniões emitidas em teses e livros-textos foram referidas. RESULTADOS: a revisão da bibliografia consultada permite afirmar que o diagnóstico precoce das AVBEH e tratamento cirúrgico para restabelecer o fluxo biliar até 60 dias de vida são fundamentais na obtenção de bons resultados. Entre os diversos métodos complementares de diagnóstico, a colangiografia por RM, US e a biópsia hepática são os que proporcionam os maiores índices de acerto. CONCLUSÕES: o encaminhamento de pacientes portadores de AVBEH para centros de referências no Brasil ainda é efetuado tardiamente, provavelmente pela falta de esclarecimento dos médicos de atenção primária, aliada às dificuldades burocráticas e tecnológicas. A experiência da Inglaterra em relação ao programa "Alerta Amarelo", permitiu que o número de crianças encaminhadas para tratamento cirúrgico antes dos 60 dias de vida aumentasse significativamente. Entre os métodos complementares, a colangiografia por RM, a US e a biópsia hepática deverão ser utilizados, dependendo dos recursos tecnológicos das unidades de diagnóstico.OBJECTIVE: to emphasize the importance of precocious diagnosis of extrahepatic biliary atresia and its direct

  20. Course of Life Into Adulthood of Patients With Biliary Atresia : The Achievement of Developmental Milestones in a Nationwide Cohort

    NARCIS (Netherlands)

    Potgieser, Adriaan R. E.; de Vries, Willemien; Sze, Yuk K.; Sieders, Egbert; Verkade, Henkjan J.; Porte, Robert J.; Hoekstra-Weebers, Josette E. H. M.; Hulscher, Jan B. F.

    Purpose: To investigate the course of life of young adults diagnosed with biliary atresia (BA) in infancy by comparing patients who did and did not underwent transplantation with an age-matched Dutch reference group. Methods: All patients from the Dutch BA registry, aged > 18 years, were invited to

  1. Course of life into adulthood of patients with biliary atresia: the achievement of developmental milestones in a nationwide cohort

    NARCIS (Netherlands)

    Potgieser, Adriaan R. E.; de Vries, Willemien; Sze, Yuk K.; Sieders, Egbert; Verkade, Henkjan J.; Porte, Robert J.; Hoekstra-Weebers, Josette E. H. M.; Hulscher, Jan B. F.; Aronson, D. C.; Damen, G.; Escher, J. H.; van Heurn, L. W. E.; Houwen, R. H. J.; Heij, H. A.; Hulscher, J. B. F.; Kneepkens, C. M. F.; Koot, B. G.; de Langen, Z. J.; Madern, G.; van den Neucker, A. M.; Peeters, P. M. J. G.; Verkade, H. J.; de Vries, W.; van der Zee, D. C.

    2012-01-01

    To investigate the course of life of young adults diagnosed with biliary atresia (BA) in infancy by comparing patients who did and did not underwent transplantation with an age-matched Dutch reference group. All patients from the Dutch BA registry, aged >18 years, were invited to complete the course

  2. Recent progress in the etiopathogenesis of pediatric biliary disease, particularly Caroli's disease with congenital hepatic fibrosis and biliary atresia.

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    Nakanuma, Yasuni; Harada, Kenichi; Sato, Yasunori; Ikeda, Hiroko

    2010-02-01

    Recent progress in elucidating the etiopathogenesis of pediatric biliary diseases, particularly Caroli's disease with congenital hepatic fibrosis (CHF) and biliary atresia (BA), is reviewed. The former is characterized by multiple saccular dilatations of the intrahepatic bile ducts. An animal model of this disease, the PCK rat, is being extensively studied. PCK rats and Calori's disease with CHF belong to autosomal recessive polycystic kidney disease (ARPKD) with ductal plate malformation. Mutations of PKHD1 have been identified in ARPKD, and fibrocystin, a product of PKHD1 located in the cilia of bile ducts is lacking in the pathologic intrahepatic bile ducts of ARPKD. Disordered cell kinetics, including apoptosis of biliary epithelial cells (BECs), may be significantly related to ductal plate malformation, and laminin and type IV collagen were immunohistochemically reduced in the basement membrane of intrahepatic bile ducts of ARPKD, and such a reduction is an additional factor for the dilatation of bile ducts. Abundant connective tissue growth factor retained diffusely in heparan sulfate proteoglycan in the fibrous portal tracts are responsible for non-resolving hepatic fibrosis. In addition, pathologic BECs of ARPKD may acquire mesenchymal features and participate in progressive hepatic fibrosis by producing extracellular matrix molecules. In an animal model of BA, an initial virus-induced, T-cell mediated autoimmune-mediated cholangiopathy has been reported. In human BA, virus-induced apoptosis of BECs by a TNF-related apoptosis-inducing ligand followed by the progressive obliteration of bile ducts is also suggested, and epithelial mesenchymal transition of BECs induced by viral infection may be involved in the fibrotic process in sclerosing cholangitis. However, the role of viral infections in the affected tissues is controversial. Comprehensive and analytical studies of ARPKD and BA using human materials and animal models may lead to the clarification of

  3. Biliary Atresia

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    ... Hepatitis B Hepatitis C Hepatitis D Hepatitis E Liver Transplant Definition & Facts Transplant Process Transplant Surgery Living with a Liver Transplant Clinical Trials Nonalcoholic Fatty Liver Disease & NASH Definition & ...

  4. Clues to the Etiology of Bile Duct Injury in Biliary Atresia

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    Mack, Cara L.; Feldman, Amy G.; Sokol, Ronald J.

    2013-01-01

    Biliary atresia (BA) is an infantile obstructive cholangiopathy of unknown etiology with suboptimal therapy, which is responsible for 40 to 50% of all pediatric liver transplants. Although the etiology of bile duct injury in BA in unknown, it is postulated that a pre- or perinatal viral infection initiates cholangiocyte apoptosis and release of antigens that trigger a Th1 immune response that leads to further bile duct injury, inflammation, and obstructive fibrosis. Humoral immunity and activation of the innate immune system may also play key roles in this process. Moreover, recent investigations from the murine BA model and human data suggest that regulatory T cells and genetic susceptibility factors may orchestrate autoimmune mechanisms. What controls the coordination of these events, why the disease only occurs in the first few months of life, and why a minority of infants with perinatal viral infections develop BA are remaining questions to be answered. PMID:23397531

  5. Adult sea lamprey tolerates biliary atresia by altering bile salt composition and renal excretion

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    Cai, Shi-Ying; Lionarons, Daniël A.; Hagey, Lee; Soroka, Carol J.; Mennone, Albert; Boyer, James L.

    2012-01-01

    The sea lamprey (Petromyzon marinus) is a genetically programmed animal model for biliary atresia as it loses its bile ducts and gallbladder during metamorphosis. However, in contrast to patients with biliary atresia or other forms of cholestasis who develop progressive disease, the post-metamorphosis lampreys grow normally to adult size. To understand how the adult lamprey thrives without the ability to secrete bile, we examined bile salt homeostasis in larval and adult lampreys. Adult livers were severely cholestatic with levels of bile salts >1 mM, but no evidence of necrosis, fibrosis, or inflammation. Interestingly, both larvae and adults had normal plasma levels (~10 μM) of bile salts. In larvae, petromyzonol sulfate (PZS) was the predominant bile salt, whereas the major bile salts in adult liver were sulfated C27 bile alcohols. Cytotoxicity assays revealed that PZS was highly toxic. Pharmacokinetic studies in free-swimming adults revealed that ~35% of intravenously injected bromosulfophthalein (BSP) was eliminated over a 72 hr period. Collection of urine and feces demonstrated that both endogenous and exogenous organic anions, including biliverdin, bile salts and BSP, were predominantly excreted via the kidney with minor amounts also detected in feces. Gene expression analysis detected marked up-regulation of orthologs of known organic anion and bile salt transporters in the kidney with lesser effects in the intestine and gills in adults compared to larvae. These findings indicate that adult lampreys tolerate cholestasis by altering hepatic bile salt composition, while maintaining normal plasma bile salt levels predominantly through renal excretion of bile products. Therefore, we conclude that strategies to accelerate renal excretion of bile salt and other toxins should be beneficial for patients with cholestasis. PMID:23175353

  6. Laparoscopic finding of a hepatic subcapsular spider-like telangiectasis sign in biliary atresia.

    Science.gov (United States)

    Zhou, Ying; Jiang, Meng; Tang, Shao-Tao; Yang, Li; Zhang, Xi; Yang, De-Hua; Xiong, Meng; Li, Shuai; Cao, Guo-Qing; Wang, Yong

    2017-10-21

    To assess the diagnostic value of a laparoscopic finding of a hepatic subcapsular spider-like telangiectasis (HSST) sign in biliary atresia. A retrospective study was conducted first and then a validation set was used to investigate the value of an HSST sign in predicting biliary atresia (BA). In the retrospective study, laparoscopic images of the liver surface were reviewed in 126 patients with infantile cholestasis (72 BA patients and 54 non-BA cholestasis patients) and a control group of 38 patients with non-hepatic conditions. Analysis was first made by two observers separately and finally, a consensus conclusion was achieved. Then, the diagnostic value of the HSST sign was validated in an independent cohort including 45 BA and 45 non-BA patients. In the retrospective investigation, an amplified HSST sign was found in all BA patients, while we were unable to detect the HSST sign in 98.1% of the 54 non-BA patients. There was no HSST sign in any of the control subjects. In the first review, the sensitivity and specificity from one reviewer were 100% and 98.1%, respectively, and the results from the other reviewer were both 100%. The consensus sensitivity and specificity were 100% and 98.1%, respectively. The HSST sign was defined as being composed of several enlarged tortuous spider-like vascular plexuses with two to eight branches distributed on all over the liver surface, which presented as either a concentrated type or a dispersed type. In the independent validation group, the sensitivity, specificity, positive predictive value and negative predictive value of the HSST sign were 100%, 97.8%, 97.8% and 100%, respectively. The HSST sign is characteristic in BA, and laparoscopic exploration for the HSST sign is valuable in the diagnosis of BA.

  7. Subdural hemorrhage: A unique case involving secondary vitamin K deficiency bleeding due to biliary atresia.

    Science.gov (United States)

    Miyao, Masashi; Abiru, Hitoshi; Ozeki, Munetaka; Kotani, Hirokazu; Tsuruyama, Tatsuaki; Kobayashi, Naho; Omae, Tadaki; Osamura, Toshio; Tamaki, Keiji

    2012-09-10

    Extrahepatic biliary atresia (EHBA) is a rare disease characterized by progressive and obliterative cholangiopathy in infants and is one of the major causes of secondary vitamin K deficiency bleeding (VKDB) due to cholestasis-induced fat malabsorption. Breast feeding increases the tendency of bleeding in EHBA patients because breast milk contains low amounts of vitamin K. A 2-month-old female infant unexpectedly died, with symptoms of vomiting and jaundice prior to death. She had been born by uncomplicated vaginal delivery and exhibited normal growth and development with breastfeeding. There was no history of trauma. She received vitamin K prophylaxis orally. In an emergency hospital, a CT scan showed a right intracranial hematoma and mass effect with midline shift to the left. In the postmortem examination, severe atresia was observed in the whole extrahepatic bile duct. Histologically, cholestasis, periductal fibrosis, and distorted bile ductules were noted. The gallbladder was not identified. A subdural hematoma and cerebellar tonsillar herniation were found; however, no traumatic injury in any part of the body was observed. Together, these findings suggest that the subdural hemorrhage was caused by secondary vitamin K deficiency resulting from a combination of cholestasis-induced fat malabsorption and breastfeeding. Subdural hemorrhage by secondary VKDB sometimes occurs even when vitamin K prophylaxis is continued. This case demonstrated that intrinsic factors, such as secondary VKDB (e.g., EHBA, neonatal hepatitis, chronic diarrhea), should also be considered in infant autopsy cases presenting with subdural hemorrhage. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  8. IL-6, TNF-α, IL-10, and nutritional status in pediatric patients with biliary atresia,

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    Maria Ines de Albuquerque Wilasco

    Full Text Available Abstract Objectives: The objective of the present study is to evaluate whether IL-6, TNF-α, IL-10 are associated with nutritional status in patients with cirrhosis secondary to biliary atresia and compare to healthy controls. Methods: The parameters used for nutritional assessment were the standard deviation scores of height-for-age and of triceps skinfold thickness-for-age. The severity of cirrhosis was evaluated using the Child–Pugh score and PELD/MELD. Serum cytokines were measured using Cytometric Bead Array flow cytometry. Results: IL-6, TNF-α, and IL-10 were significantly higher in the cirrhosis group when compared with the control group (2.4 vs. 0.24 (p < 0.001, 0.21 vs. 0.14 (p = 0.007, and 0.65 vs. 0.36 (p = 0.004, respectively. IL-6 and IL-10 were positively correlated with disease severity (0.450 [p = 0.001] and 0.410; [p = 0.002], respectively. TNF-α did not show a significant correlation with disease severity (0.100; p = 0.478. Regarding nutritional evaluation, IL-6 was negatively correlated with the standard deviation score of height-for-age (−0.493; p < 0.001 and of triceps skinfold thickness-for-age (−0.503; p < 0.001, respectively. IL-10 exhibited a negative correlation with the standard deviation score of height-for-age (−0.476; p < 0.001 and the standard deviation score of triceps skinfold thickness-for-age (−0.388; p = 0.004. TNF-α did not show any significance in both anthropometric parameters (−0.083 (p = 0.555 and −0.161 (p = 0.253. Conclusion: The authors suggest that, in patients with cirrhosis secondary to biliary atresia, IL-6 could be used as a possible supporting biomarker of deficient nutritional status and elevated IL-10 levels could be used as a possible early-stage supporting biomarker of deteriorating nutritional status.

  9. Three-Dimensional Magnetic Resonance Cholangiopancreatography for the Diagnosis of Biliary Atresia in Infants and Neonates

    Science.gov (United States)

    Liu, Bo; Cai, Jinhua; Xu, Ye; Peng, Xuehua; Zheng, Helin; Huang, Kaiping; Yang, Jing

    2014-01-01

    Background and Objective Magnetic resonance cholangiopancreatography (MRCP) is widely accepted for visualization of the biliary system. However, the sensitivity and specificity of MRCP for the diagnosis of biliary atresia (BA) are still not fully elucidated. This study aimed to investigate the diagnostic value of three-dimensional MRCP (3D-MRCP) for BA in a large cohort of cholestatic infants and neonates. Methods One hundred ninety patients with infant jaundice underwent 3D-MRCP and one or more of the following: (1) intraoperative cholangiography, (2) laparoscopic exploration and pathological examination, or/and (3) clinical therapy. Statistical analyses were performed to determine the diagnostic accuracy of 3D-MRCP for BA. Results Our study demonstrated that 158 of 190 patients were interpreted as having BA by 3D-MRCP; of those, 103 patients were confirmed as having BA, whereas 55 patients did not have BA. Of the 32 patients interpreted as non-BA cases by 3D-MRCP, one patient was misdiagnosed. The diagnostic accuracy for 3D-MRCP was 70.53% (134 of 190), the sensitivity was 99.04% (103 of 104), the specificity was 36.05% (31 of 86), the negative predictive value was 96.88% (31 of 32), the positive predictive value was 65.19% (103 of 158), the positive likelihood ratio was 2.7473, the negative likelihood ratio was 0.0267, and the Youden index was 0.3509. Conclusions The sensitivity of 3D-MRCP in diagnosing BA was excellent, but the specificity was not as high as described in previous reports. 3D-MRCP can be an effective screening method but should be combined with other modalities to identify BA and distinguish it from other causes of infant jaundice. PMID:24505457

  10. Serial ultrasound findings associated with early liver transplantation after Kasai portoenterostomy in biliary atresia.

    Science.gov (United States)

    Jeon, T Y; Yoo, S-Y; Kim, J H; Eo, H; Lee, S-K

    2013-06-01

    To investigate the ultrasound findings associated with early liver transplantation (LT) after Kasai portoenterostomy (Kasai) in children with biliary atresia (BA). Children with BA (n = 30) who underwent Kasai were classified into early LT group (n = 17, LT within 1 year after Kasai) and Kasai alone group (n = 13, alive with their native livers). Serial ultrasound (baseline and follow-up before LT or post-Kasai 1 year) images were reviewed to investigate significant ultrasound findings related to early LT using both univariate and multivariate models. Images were reviewed focusing on the hepatic artery diameter, portal vein diameter, and signs of portal hypertension. The hepatic artery diameters in the early LT group were significantly larger than those in the Kasai alone group both at baseline (p = 0.007) and follow-up ultrasound (p ultrasound were smaller in the early LT group than the Kasai alone group (p hepatic artery diameter (hazard ratio, 20.4; 95% confidence interval, 3.7-110.6; p ultrasound (17.7; 2.6-121.8; p = 0.004) were significant predictors associated with early LT. The optimal cut-off value of the baseline hepatic artery diameter was 1.9 mm (82% sensitivity and 77% specificity). Enlarged hepatic artery at baseline ultrasound and the presence of splenomegaly at follow-up ultrasound were associated with early LT after Kasai in children with BA. Copyright © 2013 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.

  11. Hepatic pathology of biliary atresia: A new comprehensive evaluation method using liver biopsy.

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    Zhang, Shouhua; Wu, Yan; Liu, Zhiwen; Tao, Qiang; Huang, Jinshi; Yang, Wenping

    2016-05-01

    Despite its unique pathological characteristics, biliary atresia (BA) has no consensus pathological grading system. Therefore, the purpose of this study was to propose a new pathological grading system and to compare the diagnostic value of intraoperative frozen and postoperative paraffin-embedded liver sections. A total of 81 BA patients were analyzed for clinical and biochemical data, immunohistochemistry, and routine postoperative histology and intraoperative frozen pathology sections. Bile duct hyperplasia was classified into three grades (B1-B3), and fibrosis was classified into four classical grades (F1-F4). The patients included 41 males and 40 females, aged 35-150 days. The repartition, in terms of severity, of small bile duct hyperplasia and fibrosis was as follows: B1, 21 cases; B2, 41 cases; B3, 19 cases; F1, 1 case; F2, 11 cases; F3, 51 cases; and F4, 18 cases. Both grades were statistically correlated. When comparing intraoperative frozen and postoperative paraffin-embedded sections, the overall diagnostic concordance rate was 97.5%. The new proposed pathological grading system may be useful for the diagnostic and prognostic assessment of BA. In addition, intraoperative frozen liver tissue biopsy samples represent a valuable and promising adjunct to the conventional postoperative paraffin-embedded sections.

  12. Inguinal Hernias Represent the Most Frequent Surgical Complication after Kasai in Biliary Atresia Infants

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    Omid Madadi-Sanjani

    2015-01-01

    Full Text Available Biliary atresia (BA is an orphan medical condition of the newborn, resulting in end-stage liver cirrhosis due to obliterative cholangiopathy of the extrahepatic bile duct. Although Kasai’s hepatoportoenterostomy (KPE is the well-established first-line therapy, little is known about its surgical complications. 153 patients receiving open KPE treated at a single center between 1994 and 2014 were analysed retrospectively regarding short-term complications and survival with the native liver. In brief, 40.5% of patients suffered from 1–3 surgical complications, inguinal hernias (IH being most prevalent (40.0%. In BA patients, incidence of IH was associated with male gender (p=0.002, the syndromic form of BA (p=0.038, and percutaneous drainage for ascites (p=0.002. No association was found with prematurity (p=0.074 or birth weight (p=0.912 in our study. In conclusion, IH frequently develops after open KPE of BA patients, but this complication does not negatively affect the patient’s outcome. Nevertheless, inspection of the internal inguinal ring and prophylactic closure of inapparent hernias should be discussed in order to prevent secondary surgical procedures.

  13. Bone Health in a Nonjaundiced Population of Children with Biliary Atresia

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    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. The role of splenectomy before liver transplantation in biliary atresia patients.

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    Takahashi, Yoshiaki; Matsuura, Toshiharu; Yanagi, Yusuke; Yoshimaru, Koichiro; Taguchi, Tomoaki

    2016-12-01

    There is currently no unified view regarding whether liver transplantation or splenectomy should be performed for hypersplenism before liver transplantation in biliary atresia (BA) patients. We herein describe the efficacy of splenectomy before liver transplantation. Splenectomy was performed in ten patients with hypersplenism associated with BA. We retrospectively reviewed their perioperative and postoperative courses, the number of leukocytes and thrombocytes, and the MELD score. The mean age was 17.5±7.0years (range 11-31years), and the male-to-female ratio was 1:1. The platelet and leukocyte levels increased after splenectomy and returned to normal levels one month postoperatively. The mean MELD score after splenectomy was significantly decreased after splenectomy: 10±2.1 vs 7.6±1.8. In particular, PT-INR improved. Five patients underwent liver transplantation because of hepatopulmonary syndrome and repeated bouts of cholangitis, whereas the remaining five patients did not undergo liver transplantation because of improvements in the liver function (the mean follow-up period was 56months). The postoperative complications included portal vein thrombosis and intestinal perforation, but the patient survival rates remained at 100%. After splenectomy, both pancytopenia and the liver function clearly improved. Splenectomy should therefore be a treatment option for patients with hypersplenism before liver transplantation. Retrospective Comparative Study - Level III. Copyright © 2016 Elsevier Inc. All rights reserved.

  15. The extent of biliary proliferation in liver biopsies from patients with biliary atresia at portoenterostomy is associated with the postoperative prognosis.

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    Santos, Jorge L; Kieling, Carlos O; Meurer, Luise; Vieira, Sandra; Ferreira, Cristina T; Lorentz, Andrea; Silveira, Themis R

    2009-04-01

    In biliary atresia (BA), a derangement in the biliary system remains, despite portoenterostomy performance. Many factors can influence the disease progression rate. This study aimed to analyze the association between biliary proliferation extent in biopsies from BA patients and postoperative prognosis. Biliary proliferation was evaluated by a morphometric analysis of the cytokeratin 7 positivity percentage (PCK7) in wedge liver biopsies from 47 BA patients. The extent of fibrosis was evaluated by a fibrosis score (FS). The outcome 1-year native liver survival was correlated, using a multivariable regression analysis, with PCK7, FS, and age at portoenterostomy. The PCK7 ranged between 0.80% and 14.79% (M +/- SD = 7.36% +/- 4.15%). Patients who died or underwent transplantation had higher PCK7 than survivors with their native livers (P operating characteristic curve for PCK7 in relation to the outcome was 0.845 (P native liver survival, independently of age and FS (P = .002). The extent of biliary proliferation at portoenterostomy, evaluated by PCK7, was associated with 1-year native liver survival of BA patients.

  16. Use of Lactobacillus casei rhamnosus to Prevent Cholangitis in Biliary Atresia After Kasai Operation.

    Science.gov (United States)

    Lien, Tien-Hau; Bu, Ling-Nan; Wu, Jia-Feng; Chen, Huey-Ling; Chen, An-Chyi; Lai, Ming-Wei; Shih, Hsiang-Hung; Lee, I-Hsien; Hsu, Hong-Yuan; Ni, Yen-Hsuan; Chang, Mei-Hwei

    2015-05-01

    Recurrent cholangitis may aggravate cholestatic liver cirrhosis in biliary atresia (BA) after the Kasai operation. This pilot study aimed to investigate whether Lactobacillus casei rhamnosus has the prophylactic efficacy for recurrent cholangitis in comparison with the conventional neomycin prophylaxis. Twenty jaundice-free patients with BA ages 0 to 3 years who underwent a Kasai operation were enrolled and randomized into 2 groups with 10 patients each: neomycin (25 mg · kg · day for 4 days/wk) and L casei rhamnosus (8 × 10 colony-forming unit per day) groups. The treatment duration was 6 months. Bacterial stool cultures were performed before treatment and 1, 3, and 6 months after starting treatment. In addition, 10 patients with BA with similar status but without prophylaxis served as the historical control group. In the Lactobacillus group, 2 patients (20%, mean 0.03 ± 0.07 episodes per month) developed cholangitis during the study period, with the same frequency as in the neomycin group and significantly lower than that in the control group (80%, P = 0.005, mean 0.22 ± 0.16 episodes per month). The mean change in body weight z score during the 6 months in the Lactobacillus group was 0.97 ± 0.59, which was significantly better than that in the control group (-0.01 ± 0.79, P = 0.006). In bacterial stool cultures, the Lactobacillus and Escherichia coli populations significantly increased and decreased, respectively, in the Lactobacillus group. The use of L casei rhamnosus was as effective as neomycin in preventing cholangitis in patients with BA who underwent Kasai operation, and therefore could be considered as a potential alternative prophylactic regimen.

  17. Assessment of liver fibrosis by Fibroscan as compared to liver biopsy in biliary atresia.

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    Shen, Qiu-Long; Chen, Ya-Jun; Wang, Zeng-Meng; Zhang, Ting-Chong; Pang, Wen-Bo; Shu, Jun; Peng, Chun-Hui

    2015-06-14

    To evaluate liver stiffness measurement (LSM) using non-invasive transient elastography (Fibroscan) in comparison with liver biopsy for assessment of liver fibrosis in children with biliary atresia (BA). Thirty-one children with BA admitted to the Department of Pediatric Surgery of Beijing Children's Hospital from March 2012 to February 2013 were included in this study. Their preoperative LSM, liver biopsy findings, and laboratory results were studied retrospectively. The grade of liver fibrosis in all 31 patients was evaluated according to the METAVIR scoring system, which showed that 4 cases were in group F2, 20 in group F3 and 7 in group F4. There were 24 non-cirrhosis cases (F2-F3) and 7 cirrhosis cases (F4). In groups F2, F3 and F4, the mean LSM was 9.10 ± 3.30 kPa, 11.02 ± 3.31 kPa and 22.86 ± 12.43 kPa, respectively. LSM was statistically different between groups F2 and F4 (P = 0.002), and between groups F3 and F4 (P = 0.000), however, there was no statistical difference between groups F2 and F3 (P = 0.593). The area under the receiver operating characteristic curve of LSM for ≥ F4 was 0.866. The cut-off value of LSM was 15.15 kPa for ≥ F4, with a sensitivity, specificity, positive predictive value and negative predictive value of 0.857, 0.917, 0.750 and 0.957, respectively. Fibroscan can be used as a non-invasive technique to assess liver fibrosis in children with BA. The cut-off value of LSM (15.15 kPa) can distinguish cirrhotic patients from non-cirrhotic patients.

  18. Association between Promoter Hypomethylation and Overexpression of Autotaxin with Outcome Parameters in Biliary Atresia.

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    Wanvisa Udomsinprasert

    Full Text Available Biliary atresia (BA is a progressive fibroinflammatory liver disease. Autotaxin (ATX has a profibrotic effect resulting from lysophosphatidic acid activity. The purpose of this study was to examine ATX expression and ATX promoter methylation in peripheral blood leukocytes and liver tissues from BA patients and controls and investigate their associations with outcome parameters in BA patients.A total of 130 subjects (65 BA patients and 65 age-matched controls were enrolled. DNA was extracted from circulating leukocytes and liver tissues of BA patients and from and age-matched controls. ATX promoter methylation status was determined by bisulfite pyrosequencing. ATX expression was analyzed using quantitative real-time polymerase chain reaction and enzyme-linked immunosorbent assay.Decreased methylation of specific CpGs were observed at the ATX promoter in BA patients. Subsequent analysis revealed that BA patients with advanced stage had lower methylation levels of ATX promoter than those with early stage. ATX promoter methylation levels were found to be associated with hepatic dysfunction in BA. In addition, ATX expression was significantly elevated and correlated with a decrease in ATX promoter methylation in BA patients compared to the controls. Furthermore, promoter hypomethylation and overexpression of ATX were inversely associated with jaundice status, hepatic dysfunction, and liver stiffness in BA patients.Accordingly, it has been hypothesized that ATX promoter methylation and ATX expression in peripheral blood may serve as possible biomarkers reflecting the progression of liver fibrosis in postoperative BA. These findings suggest that the promoter hypomethylation and overexpression of ATX might play a contributory role in the pathogenesis of liver fibrosis in BA.

  19. Ultrasonographic Diagnosis of Biliary Atresia Based on a Decision-Making Tree Model.

    Science.gov (United States)

    Lee, So Mi; Cheon, Jung-Eun; Choi, Young Hun; Kim, Woo Sun; Cho, Hyun-Hae; Cho, Hyun-Hye; Kim, In-One; You, Sun Kyoung

    2015-01-01

    To assess the diagnostic value of various ultrasound (US) findings and to make a decision-tree model for US diagnosis of biliary atresia (BA). From March 2008 to January 2014, the following US findings were retrospectively evaluated in 100 infants with cholestatic jaundice (BA, n = 46; non-BA, n = 54): length and morphology of the gallbladder, triangular cord thickness, hepatic artery and portal vein diameters, and visualization of the common bile duct. Logistic regression analyses were performed to determine the features that would be useful in predicting BA. Conditional inference tree analysis was used to generate a decision-making tree for classifying patients into the BA or non-BA groups. Multivariate logistic regression analysis showed that abnormal gallbladder morphology and greater triangular cord thickness were significant predictors of BA (p = 0.003 and 0.001; adjusted odds ratio: 345.6 and 65.6, respectively). In the decision-making tree using conditional inference tree analysis, gallbladder morphology and triangular cord thickness (optimal cutoff value of triangular cord thickness, 3.4 mm) were also selected as significant discriminators for differential diagnosis of BA, and gallbladder morphology was the first discriminator. The diagnostic performance of the decision-making tree was excellent, with sensitivity of 100% (46/46), specificity of 94.4% (51/54), and overall accuracy of 97% (97/100). Abnormal gallbladder morphology and greater triangular cord thickness (> 3.4 mm) were the most useful predictors of BA on US. We suggest that the gallbladder morphology should be evaluated first and that triangular cord thickness should be evaluated subsequently in cases with normal gallbladder morphology.

  20. Ultrasonographic diagnosis of biliary atresia based on a decision-making tree model

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    Lee, So Mi; Cheon, Jung Eun; Choi, Young Hun; Kim, Woo Sun; Cho, Hyun Hye; Kim, In One; You, Sun Kyoung [Dept. of Radiology, Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2015-12-15

    To assess the diagnostic value of various ultrasound (US) findings and to make a decision-tree model for US diagnosis of biliary atresia (BA). From March 2008 to January 2014, the following US findings were retrospectively evaluated in 100 infants with cholestatic jaundice (BA, n = 46; non-BA, n = 54): length and morphology of the gallbladder, triangular cord thickness, hepatic artery and portal vein diameters, and visualization of the common bile duct. Logistic regression analyses were performed to determine the features that would be useful in predicting BA. Conditional inference tree analysis was used to generate a decision-making tree for classifying patients into the BA or non-BA groups. Multivariate logistic regression analysis showed that abnormal gallbladder morphology and greater triangular cord thickness were significant predictors of BA (p = 0.003 and 0.001; adjusted odds ratio: 345.6 and 65.6, respectively). In the decision-making tree using conditional inference tree analysis, gallbladder morphology and triangular cord thickness (optimal cutoff value of triangular cord thickness, 3.4 mm) were also selected as significant discriminators for differential diagnosis of BA, and gallbladder morphology was the first discriminator. The diagnostic performance of the decision-making tree was excellent, with sensitivity of 100% (46/46), specificity of 94.4% (51/54), and overall accuracy of 97% (97/100). Abnormal gallbladder morphology and greater triangular cord thickness (> 3.4 mm) were the most useful predictors of BA on US. We suggest that the gallbladder morphology should be evaluated first and that triangular cord thickness should be evaluated subsequently in cases with normal gallbladder morphology.

  1. Derivation of a disease-specific human induced pluripotent stem cell line from a biliary atresia patient.

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    Tian, Lipeng; Eldridge, Lindsey; Chaudhari, Pooja; Zhang, Linyi; Anders, Robert A; Schwarz, Kathleen B; Ye, Zhaohui; Jang, Yoon-Young

    2017-10-01

    Biliary atresia (BA) is a common cause of pediatric end-stage liver disease. While its etiology is not yet clear, evidence has suggested that BA results from interactions between genetic susceptibility and environmental factors. Disease relevant human cellular models of BA will facilitate identification of both genetic and environmental factors that are important for disease prevention and treatment. Here we report the generation of a human induced pluripotent stem cell line from a BA patient using episomal vectors. Patient-specific BA iPSC lines provide valuable tools for disease mechanism study and drug development. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

  2. Derivation of a disease-specific human induced pluripotent stem cell line from a biliary atresia patient

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    Lipeng Tian

    2017-10-01

    Full Text Available Biliary atresia (BA is a common cause of pediatric end-stage liver disease. While its etiology is not yet clear, evidence has suggested that BA results from interactions between genetic susceptibility and environmental factors. Disease relevant human cellular models of BA will facilitate identification of both genetic and environmental factors that are important for disease prevention and treatment. Here we report the generation of a human induced pluripotent stem cell line from a BA patient using episomal vectors. Patient-specific BA iPSC lines provide valuable tools for disease mechanism study and drug development.

  3. Sonic hedgehog (SHH) and glioblastoma-2 (Gli-2) expressions are associated with poor jaundice-free survival in biliary atresia.

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    Jung, Hae Yoen; Jing, Jin; Lee, Kyoung Bun; Jang, Ja-June

    2015-03-01

    Biliary atresia (BA) causes biliary obstruction in neonates. Although the Kasai operation can successfully treat certain BA cases, many patients exhibit recurrent jaundice and secondary biliary cirrhosis requiring liver transplantation. Consequently, studies of the prognostic factors of the Kasai operation are needed. Accordingly, sonic hedgehog (SHH) pathway expression at the extrahepatic bile duct (EHBD), an important bile duct repair mechanism, will be investigated via immunohistochemistry in patients with BA to examine the association with post-Kasai operation prognosis. Fifty-seven EHBD specimens were obtained during Kasai operations from 1992 to 2009. The SHH, patched (PTCH), and glioblastoma-2 (Gli-2) immunohistochemical staining results were analyzed quantitatively. Overall, 57.9% of patients had bile flow normalization after the Kasai operation; 43.1% did not. High preoperative serum total bilirubin, direct bilirubin, and aspartate aminotransferase levels were associated with sustained jaundice post-Kasai operation, as was an age ≥65days at the time of surgery (all pjaundice relapse. Strong Gli-2 and SHH expression in the EHBD might be a poor prognostic factor in Kasai operation-treated patients with BA. Copyright © 2015 Elsevier Inc. All rights reserved.

  4. Late-Onset Invasive Group B Streptococcal Infection with Serotype VIII in a Neonate Having Congenital Biliary Atresia

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    Tomoaki Takei

    2013-02-01

    Full Text Available A female newborn was admitted to our department 15 days after birth for insufficient sucking and jaundice. The patient’s blood and urine cultures were both positive for group B streptococcal (GBS infection. A maternal vaginal sample at 35 weeks’ gestation was negative for GBS in culture-based microbiologic screening. The patient recovered shortly after receiving systemic antibiotic therapy. On the basis of clinical evidence of white stool and progressive jaundice, we suspected that the newborn had complications related to congenital biliary atresia (CBA; surgery was performed. Isolates from the mother’s vaginal sample obtained when the patient was 25 days old, along with neonatal blood, revealed identical patterns (serotype VIII and sequence type 1 of GBS capsular and multilocus sequence typing, suggestive of maternal transmission. Molecular epidemiologic examination may be useful to clarify the transmission route and etiology; culture-based microbiologic screening appears to have limitations for detecting the route of transmission.

  5. The value of preoperative liver biopsy in the diagnosis of extrahepatic biliary atresia: A systematic review and meta-analysis.

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    Lee, James Y J; Sullivan, Katrina; El Demellawy, Dina; Nasr, Ahmed

    2016-05-01

    In extrahepatic biliary atresia (EHBA) obstruction of the biliary tree causes severe cholestasis leading to cirrhosis and death if left untreated in a timely manner. Infants with cholestasis may undergo many tests before EHBA diagnosis is reached. The role and place of preoperative liver biopsy in the diagnostic paradigm for EHBA have not been established. We conducted a systematic review of MEDLINE, Embase, and CENTRAL to obtain all publications describing the sensitivity/specificity/accuracy/positive predictive value (PPV)/negative predictive value (NPV) of preoperative liver biopsy in infants with cholestasis. Screening, data extraction, and quality assessment were done in duplicate. Extracted data are described narratively and analyzed using forest plots and receiver operating characteristic curves. A total of 22 articles were included. Overall, the pooled accuracy of preoperative liver biopsy was 91.7%, with a sensitivity of 91.2%, specificity of 93.0% (n=1231), PPV of 91.2%, NPV of 92.5% (n=1182), and accuracy of 91.6% (n=1106). In patients who were 60days or less at time of presentation or diagnosis, the pooled sensitivity, specificity, PPV, NPV, and accuracy were 96.4%, 96.3%, 95.8%, 96.3%, and 94.9%, respectively. Quantitative analysis demonstrated preoperative biopsy to be both highly specific and sensitive in diagnosing EHBA preoperatively. It is a highly reliable test that offers a means of arriving at an early definitive diagnosis of EHBA. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. Detection of Epstein Barr Virus by Chromogenic In Situ Hybridization in cases of extra-hepatic biliary atresia

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    Farahmand Fatemeh

    2008-04-01

    Full Text Available Abstract Introduction Extra-hepatic biliary atresia (EHBA is an important cause of neonatal cholestasis. Several infectious agents have been proposed as etiologic factors such as Rotavirus and Reovirus. There is limited data on the role of Epstein Barr virus (EBV infection in EHBA, so we decided to study the presence of EBV virus in a series of 16 proven EHBA cases by Chromogenic in situ hybridization (CISH technique. Methods In the current study a total of 16 liver wedge biopsies of proven cases of EHBA were selected in a period of 4 years. CISH staining for EBV-encoded RNA (EBER transcript was performed. Results The review of H&E-stained slides of liver biopsies revealed fibrosis and marked ductular proliferation. In CISH-stained slides, EBV trace was observed in hepatocytes in two cases and in biliary epithelium in one case of EHBA. Discussion Considering the association of hepatitis with the Epstein-Barr virus in later life, it is likely that EBV hepatitis and its complications occur in the neonatal/perinatal period. Since EHBA is a relatively rare disease, a similar study on wedge biopsies of this number of proven cases of EHBA has not been performed to date. Current observation proposes the need for a study of larger series and employing other methods for confirming the etiologic role of EBV in EHBA cases.

  7. Course of life into adulthood of patients with biliary atresia: the achievement of developmental milestones in a nationwide cohort.

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    Potgieser, Adriaan R E; de Vries, Willemien; Sze, Yuk K; Sieders, Egbert; Verkade, Henkjan J; Porte, Robert J; Hoekstra-Weebers, Josette E H M; Hulscher, Jan B F; Aronson, D C; Damen, G; Escher, J H; van Heurn, L W E; Houwen, R H J; Heij, H A; Hulscher, J B F; Kneepkens, C M F; Koot, B G; de Langen, Z J; Madern, G; van den Neucker, A M; Peeters, P M J G; Verkade, H J; de Vries, W; van der Zee, D C

    2012-06-01

    To investigate the course of life of young adults diagnosed with biliary atresia (BA) in infancy by comparing patients who did and did not underwent transplantation with an age-matched Dutch reference group. All patients from the Dutch BA registry, aged >18 years, were invited to complete the course of life questionnaire. Forty patients participated (response = 74%). Twenty-five had not undergone transplantation; 15 had undergone orthotopic liver transplantation. One significant between-group difference was found, namely in substance use and gambling. BA patients who underwent transplantation reported less use than the reference group (p = .01, moderate effect size). Additional moderate effect sizes were found for differences in psychosexual and social development and antisocial behavior. Patients who underwent transplantation had lower scores than one or both other groups. Development of BA survivors who did not undergo transplantation seems not delayed, whereas that of transplanted patients does seem somewhat delayed. However, patients who underwent transplantation display less risk behavior. Larger samples are necessary to confirm these findings. Copyright © 2012 Society for Adolescent Health and Medicine. Published by Elsevier Inc. All rights reserved.

  8. P-Selectin (CD62P) Expression in Liver Tissue of Biliary Atresia: A New Perspective in Etiopathogenesis.

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    Sira, Mostafa M; Sira, Ahmad M; Ehsan, Nermine A; Mosbeh, Asmaa

    2015-11-01

    The etiology of biliary atresia (BA) is still elusive. Inflammation plays a key role in bile duct and liver injury. The recruitment and accumulation of inflammatory cells is largely dependent on adhesion molecules. We aimed to investigate P-selectin (CD62P) expression in liver tissue in patients with BA compared with other neonatal cholestatic disorders. The study included 63 infants with neonatal cholestasis in 2 groups: BA group (n = 32) and non-BA group (n = 31) with non-BA cholestatic disorders as controls. Demographic, clinical, laboratory, ultrasonographic, and histopathological parameters were collected. P-selectin immunostaining was performed. Immunostaining in bile duct epithelium, cellular infiltrate, and vascular endothelial cells were scored as positive or negative. The frequency of P-selectin-positive endothelium, platelets, and bile duct epithelium was significantly higher in the BA group (72%, 72%, and 63%, respectively) than in the non-BA group (32%, 16%, and 13%, respectively) with P of 0.002, thrombocytosis versus 25% in the controls (P = 0.001 for both). The significant expression of P-selectin in endothelium, platelets, and bile duct epithelium in patients with BA suggests a potential role for this adhesion molecule in the pathogenesis of this devastating neonatal hepatic disorder. It further suggests that platelets in BA are activated and may have a role in the inflammatory process in BA.

  9. Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia

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    Tae Jun Park

    2014-03-01

    Full Text Available Hepatopulmonary syndrome (HPS is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE, and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%. Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.

  10. Predictive value of hepatic ultrasound, liver biopsy, and duodenal tube test in the diagnosis of extrahepatic biliary atresia in Serbian infants.

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    Boskovic, Aleksandra; Kitic, Ivana; Prokic, Dragan; Stankovic, Ivica; Grujic, Blagoje

    2014-04-01

    Extrahepatic biliary atresia (EHBA) is the most important cause of neonatal cholestasis. The validity of different diagnostic methods in the diagnosis of EHBA in developed countries has been presented elsewhere, but data from developing countries with low national incomes are scarce. The aim of this study was to investigate the relative accuracy and roles of abdominal ultrasonography, duodenal tube test (DTT), and liver biopsy in the diagnosis of EHBA in Serbia. The study included 156 infants with cholestasis admitted at the Mother and Child Health Care Institute. Data were collected according to the medical records observation technique. Extrahepatic biliary atresia was diagnosed in 72 of 156 infants with cholestasis. The frequency was insignificantly higher in females than in males (1.25:1). Most patients were diagnosed prior to 60 days of life (median 58, range 30-67). In a group of 156 infants with cholestasis, 109 had ultrasound, liver biopsy, duodenal tube test, and intraoperative cholangiography done. Liver biopsy confirmed surgical disease in 71/109 patients and denied it in 38/109 patients (sensitivity- Sn 98%, specificity- Sp 100%, diagnostic efficiency of test- DgEf 99.08%). Duodenal tube test had Sn 97%, Sp 72%, and DgEf 88.99%, and the ultrasound findings showed Sn 78%, Sp 81%, and DgEf 77.92%. Five-year survival rate after Kasai operation was 76%. A well-coordinated multidisciplinary approach is required in the assessment of suspected cases of biliary atresia. Histology examination of biopsy specimens is an integral part of the diagnostic algorithm and, therefore, plays a pivotal role in the diagnostic evaluation of this disease.

  11. Preoperative hepatic hemodynamics in the prediction of early portal vein thrombosis after liver transplantation in pediatric patients with biliary atresia.

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    Gu, Li-Hong; Fang, Hua; Li, Feng-Hua; Zhang, Shi-Jun; Han, Long-Zhi; Li, Qi-Gen

    2015-08-01

    Portal vein thrombosis (PVT) is one of the main vascular complications after liver transplantation (LT), especially in pediatric patients with biliary atresia (BA). This study aimed to assess the preoperative hepatic hemodynamics in pediatric patients with BA using Doppler ultrasound and determine whether ultrasonographic parameters may predict early PVT after LT. One hundred and twenty-eight pediatric patients with BA younger than 3 years of age underwent Doppler ultrasound within seven days before LT, between October 2006 and June 2013. The preoperative hepatic hemodynamic parameters were then compared between patients with early PVT (within 1 month following LT) and those without PVT. Receiver operating characteristic analysis was performed to determine the optimal cutoff value for predicting early PVT. Of the 128 transplant recipients, 41 (32.03%) had a hypoplastic portal vein (PV), 52 (40.63%) had hepatofugal PV flow and 40 (31.25%) had a high hepatic artery resistance index (HARI) of ≥1. Nine cases (7.03%) experienced early PVT. A PV diameter ≤4 mm (sensitivity 88.89%, specificity 72.27%), and a hepatofugal PV flow (sensitivity 77.78%, specificity 62.18%) with a high HARI ≥1 (sensitivity 77.78%, specificity 72.27%) were hepatic hemodynamic risk factors for early PVT. Hepatic hemodynamic disturbances in pediatric recipients with BA were more common. Small PV diameter (≤4 mm) and hepatofugal PV flow combined with high HARI (≥1) are strong warning signs of early PVT after LT in pediatric patients with BA. Intense monitoring of vascular patency and prophylactic thrombolytic therapy should be considered in pediatric patients undergoing LT for BA.

  12. Preoperative Serum IL-12p40 Is a Potential Predictor of Kasai Portoenterostomy Outcome in Infants with Biliary Atresia

    Directory of Open Access Journals (Sweden)

    Shaimaa Samy Goda

    2017-01-01

    Full Text Available The standard-of-care treatment for biliary atresia (BA is surgical restoration of bile flow by Kasai portoenterostomy. We aimed to study serum interleukin- (IL- 12p40, a natural antagonist for the proinflammatory IL-12p70, and its relation to surgical outcomes of BA. The study included 75 infants with neonatal cholestasis: BA group (n=25, non-BA cholestasis group (n=30, and neglected BA group (n=20, in addition to thirty healthy neonates serving as controls. IL-12p40 was measured by ELISA in all individuals and a second assessment was performed 3 months postoperatively in the BA group. The surgical outcomes were classified as successful (bilirubin ≤ 2 mg/dl or failed (bilirubin > 2 mg/dl. IL-12p40 was higher in BA compared to that in the non-BA and control groups (P values were 0.036 and <0.0001, resp. but comparable to that in the neglected BA group. Preoperative IL-12p40 levels in BA patients were significantly higher in successful Kasai compared with failed Kasai and a cutoff level of 547.47 pg/ml could predict the successful outcome with 87.5% sensitivity and 82.4% specificity. Three-month postoperative IL-12p40 tended to decrease in both the successful and failed groups. In conclusion, preoperative serum IL-12p40 is a potential predictor of Kasai outcome. Serial postoperative measurements may anticipate the failure of an initially successful operation, hence the need for liver transplantation.

  13. congenital Biliary atresia

    African Journals Online (AJOL)

    Prognosis in this child appeared to be guarded due to the late presentation to hospital and delayed diagnosis – a complication of liver cirrhosis may have already developed as manifested by the bleeding diathesis coupled with the laboratory findings which indicate hepatocellular injury. DIscUssIOn. Neonatal cholestasis ...

  14. Hepatic ADC map as an adjunct to conventional abdominal MRI to evaluate hepatic fibrotic and clinical cirrhotic severity in biliary atresia patients

    Energy Technology Data Exchange (ETDEWEB)

    Peng, Steven Shinn-Forng [National Taiwan University, Department of Medical Imaging, Medical College and Hospital, Taipei (China); Jeng, Yung-Ming [National Taiwan University, Department of Pathology, Medical College and Hospital, Taipei (China); Hsu, Wen-Ming; Ho, Ming-Chih [National Taiwan University, Department of Surgery, Medical College and Hospital, Taipei, Taiwan (China); Yang, Justin Cheng-Ta [National Taiwan University, Department of Medical Imaging, Medical College and Hospital, Taipei (China); National Taiwan University, Institute of Biomedical Engineering, College of Engineering and College of Medicine, Taipei (China)

    2015-10-15

    Hepatic apparent diffusion coefficient (ADC) values and ADC-related indices were correlated with the Mayo risk score for primary biliary cirrhosis (MRSPBC) and METAVIR scores of liver specimens to determine the clinical and pathological significance of diffusion-weighted magnetic resonance imaging (DWMRI). Thirty-two patients with biliary atresia (BA; mean age 461 days, range 11-4616 days) received magnetic resonance examinations from March 2009 to August 2013. A free-breathing DWMRI sequence was performed with the single-shot echo-planar imaging technique with b = 0 and 500 s/mm{sup 2} in all 32 BA patients and 24 controls. We used the ordinal logistic regression test and Spearman rank correlation test to analyse the relationships between the MRSPBC and METAVIR fibrosis scores and right liver-to-psoas ADC ratios (LTPARs). BA patients had significantly lower LTPARs in both hepatic lobes than controls (p < 0.01). Right LTPARs, showing moderate intraobserver agreement (intraclass correlation coefficient = 0.736) and interobserver reliability (intraclass correlation coefficient = 0.659), were negatively correlated with MRSPBC and METAVIR fibrosis scores (R{sup 2} = 0.398, p = 0.024 and R{sup 2} = 0.628, p < 0.001, respectively). Right LTPARs may be used for long-term follow-up of cirrhosis severity in BA patients. (orig.)

  15. Prognos is of living donor liver transplantation performed during different periods in infants with biliary atresia: a single-center experience with 101 cases

    Directory of Open Access Journals (Sweden)

    HUA Xiangwei

    2014-01-01

    Full Text Available ObjectiveTo compared the prognosis between infants with biliary atresia (BA who underwent living donor liver transplantation (LDLT during 2006-2009 and 2010-2012 in the Department of Liver Surgery, Renji Hospital, Shanghai Jiaotong University School of Medicine and to evaluate the prognos is of LDLT performed during different periods. MethodsA retrospective analysis was performed on the clinical data of 101 infants with BA who underwent LDLT from October 2006 to December 2012. These patients were divided into 2006-2009 group and 2010-2012 group according to the year the procedure was performed. The two groups were compared in terms of preoperative general data, intraoperative status, and treatment outcome. The Kaplan-Meier method was used for survival analysis; measurement data were analyzed by t test, and enumeration data were analyzed by Fisher exact probability test and chi-square test. ResultsThe preoperative general data showed no significant difference between the two groups. Compared with the 2006-2009 group, the 2010-2012 group had significantly less intraoperative blood loss (t=2.05, P=0.04, a significantly shorter cold ischemia time of donor liver (t=3.25, P<0.01, and significantly lower incidence of postoperative biliary complications (χ2=4.27, P=0.04, pulmonary infection (χ2=4.47, P=0.03, and acute rejection (P=0.03. The 2010-2012 group had significantly improved survival compared with the 2006-2009 group; the 1 and 2 year cumulative survival rates for the 2010-2012 group were 884% and 88.4%, respectively, versus 84.4% and 75% for the 2006-2009 group. ConclusionThe development of surgical techniques, improvement in perioperative management, and standardized follow-up can significantly increase the success rate of surgery and survival rate and reduce postoperative complications in BA patients.

  16. Long-term native liver fibrosis in biliary atresia: development of a novel scoring system using histology and standard liver tests.

    Science.gov (United States)

    Tomita, Hirofumi; Masugi, Yohei; Hoshino, Ken; Fuchimoto, Yasushi; Fujino, Akihiro; Shimojima, Naoki; Ebinuma, Hirotoshi; Saito, Hidetsugu; Sakamoto, Michiie; Kuroda, Tatsuo

    2014-06-01

    Although liver fibrosis is an important predictor of outcomes for biliary atresia (BA), postsurgical native liver histology has not been well reported. Here, we retrospectively evaluated postsurgical native liver histology, and developed and assessed a novel scoring system - the BA liver fibrosis (BALF) score for non-invasively predicting liver fibrosis grades. We identified 259 native liver specimens from 91 BA patients. Of these, 180 specimens, obtained from 62 patients aged ≥1 year at examination, were used to develop the BALF scoring system. The BALF score equation was determined according to the prediction of histological fibrosis grades by multivariate ordered logistic regression analysis. The diagnostic powers of the BALF score and several non-invasive markers were assessed by area under the receiver operating characteristic curve (AUROC) analyses. Natural logarithms of the serum total bilirubin, γ-glutamyltransferase, and albumin levels, and age were selected as significantly independent variables for the BALF score equation. The BALF score had a good diagnostic power (AUROCs=0.86-0.94, pnative liver fibrosis. Copyright © 2014 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

  17. Home-based screening for biliary atresia using infant stool colour cards: a large-scale prospective cohort study and cost-effectiveness analysis.

    Science.gov (United States)

    Schreiber, Richard A; Masucci, Lisa; Kaczorowski, Janusz; Collet, J P; Lutley, Pamela; Espinosa, Victor; Bryan, Stirling

    2014-09-01

    Biliary atresia (BA), a leading cause of paediatric liver failure and liver transplantation, manifests by three weeks of life as jaundice with acholic stools. Poor outcomes due to delayed diagnosis remain a problem worldwide. We evaluated and assessed the cost-effectiveness of methods of introducing a BA Infant Stool Colour Card (ISCC) screening programme in Canada. A prospective study at BC Women's Hospital recruited consecutive healthy newborns through six incrementally more intensive screening approaches. Under the baseline "passive" strategy, families received ISCCs at maternity, with instructions to monitor infant stool colour daily and return the ISCC by mail at age 30 days. Additional strategies were: ISCC mailed to family physician; reminder letters or telephone calls to families or physicians. Random telephone surveys of ISCC non-returners assessed total card utilization. Primary outcome was ISCC utilization rate expressed as a composite outcome of the ISCC return rate and non-returned ISCC use. Markov modelling was used to predict incremental costs and life years gained from screening (passive and reminder), compared with no screening, over a 10-year time horizon. 6,187 families were enrolled. Card utilization rates in the passive screening strategy were estimated at 60-94%. For a Canadian population, the increase in cost for passive screening, compared with no screening, is $213,584 and the gain in life years is 9.7 ($22,000 per life-year gained). A BA ISCC screening programme targeting families of newborns is feasible in Canada. Passive distribution of ISCC at maternity is potentially effective and highly cost-effective. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  18. Prognostic impact of hepatobiliary scintigraphy in diagnosis and postoperative follow-up of newborns with biliary atresia; Prognostische Wertigkeit der hepatobiliaeren Funktionszintigraphie in Diagnostik und Nachsorge der Gallengangsatresie

    Energy Technology Data Exchange (ETDEWEB)

    Rossmueller, B.; Porn, U.; Dresel, S.; Hahn, K. [Muenchen Univ. (Germany). Klinik und Poliklinik fuer Nuklearmedizin; Schuster, T. [Muenchen Univ. (Germany). Kinderchirurgische Klinik; Lang, T. [Muenchen Univ. (Germany). Kinderklinik

    2000-01-01

    Aim: To investigate the prognostic relevance of hepatobiliary scintigraphy (HBS) in newborns suffering from biliary atresia (BA) for establishing the primary diagnosis and in the postoperative follow-up after portoenterostomy (Kasai). Methods: Twenty newborns with direct hyperbilirubinemia and 6 children after operative treatment of BA (Kasai) underwent HBS with Tc-99m-DEIDA. In patients without intestinal drainage, hepatocellular extraction was estimated visually and calculated semiquantitatively by means of liver/heart-ratio 5 min p.i. Results: 10/20 patients with hyperbilirubinemia did not display biliary drainage; 6 had BA, 3 intrahepatic hypoplasia, and one showed a bile plug syndrom. 4/6 with BA but none of the 4 children with diagnoses other than BA presented with a good extraction. All of the 4 children with BA, who had either pre- or postoperatively a bad extraction, needed liver transplantation due to liver failure. Both of the two newborns with BA and favourable outcome after Kasai had a good extraction in the preoperative HBS and demonstrated good intestinal drainage in the postoperative scan. Conclusion: HBS rules out BA with high accuracy by demonstrating drainage of bile into the intestine. In newborns without drainage a good extraction favours the diagnosis of BA. In newborns with BA a bad extraction seems to indicate a poor postoperative prognosis after Kasai operation. HBS might therefore help to select those children who will not benefit from portoenterostomy. Postoperatively, HBS can easily and quickly confirm the successful hepatobiliary anastomosis by demonstrating biliary drainage into the intestine. (orig.) [German] Ziel der Studie war es, die diagnostische Wertigkeit und die prognostische Aussagekraft der hepatobiliaeren Funktionsszintigraphie (HB-FS) in der Primaerdiagnostik der Gallengangsatresie (GG-Atresie) und bei postoperativen Kontrollen nach Portoenterostomie (Kasai-OP) zu ueberpruefen. Methoden: 20 Neugeborene (Alter: 3-119 d) mit

  19. Nutritional status of patients with biliary atresia and autoimmune hepatitis related to serum levels of vitamins A, D and E Estado nutricional de pacientes com atresia biliar e hepatite autoimune e relação com os níveis séricos de vitaminas A, D e E

    Directory of Open Access Journals (Sweden)

    Margareth L. G. Saron

    2009-03-01

    Full Text Available CONTEXT: Chronic liver disease may induce to malabsorption of lipids and fat-soluble vitamins, leading to injury of nutritional status. OBJECTIVES: To evaluate the nutritional status of pediatric-age patients with autoimmune hepatitis and biliary atresia related to serum levels of vitamins A, D and E and the disease severity. METHODS: This controlled transverse study, evaluated the patients with autoimmune hepatitis and biliary atresia and a reference group paired by sex and age. The patients underwent anthropometric evaluation, alimentary inquiry and determination of serum levels of vitamins A, D and E by high performance liquid chromatography. The Mann-Whitney test, Spearman correlation coefficients and variance analysis (ANOVA were utilized for data treatment, regarding significant difference if PCONTEXTO: As doenças hepáticas crônicas podem induzir à má absorção de lipídios e vitaminas lipossolúveis e levar ao comprometimento do estado nutricional. OBJETIVOS: Avaliar o estado nutricional e relacionar com os níveis séricos de vitaminas (A, D e E e a gravidade da doença em pacientes com atresia biliar e hepatite autoimune na faixa etária pediátrica. MÉTODOS: O estudo foi transversal controlado e foram avaliados os pacientes com hepatite autoimune e atresia biliar e um grupo controle pareado por sexo e idade. Foi realizada avaliação antropométrica, aplicação do inquérito alimentar e determinação dos níveis séricos das vitaminas A, D e E pela técnica de cromatografia líquida de alta eficiência. Foram empregados os testes de Mann-Whitney, o coeficiente de correlação de Spearman e análise de variância (ANOVA, sendo considerada diferença significativa se P<0,05. RESULTADOS: O déficit nutricional mais grave foi observado nos pacientes com atresia biliar, principalmente com colestase. Em relação às vitaminas, no grupo controle, constatou-se que os níveis séricos das vitaminas A e E variaram com a idade. Os n

  20. Pulmonary atresia

    Science.gov (United States)

    As with most congenital heart diseases, there is no known cause of pulmonary atresia. The condition is linked with another type of congenital heart defect called a patent ductus arteriosus (PDA). Pulmonary atresia may occur with or without a ventricular ...

  1. Prenatal intestinal obstruction affects the myenteric plexus and causes functional bowel impairment in fetal rat experimental model of intestinal atresia.

    Directory of Open Access Journals (Sweden)

    Naziha Khen-Dunlop

    Full Text Available BACKGROUND: Intestinal atresia is a rare congenital disorder with an incidence of 3/10,000 birth. About one-third of patients have severe intestinal dysfunction after surgical repair. We examined whether prenatal gastrointestinal obstruction might effect on the myenteric plexus and account for subsequent functional disorders. METHODOLOGY/PRINCIPAL FINDINGS: We studied a rat model of surgically induced antenatal atresia, comparing intestinal samples from both sides of the obstruction and with healthy rat pups controls. Whole-mount preparations of the myenteric plexus were stained for choline acetyltransferase (ChAT and nitric oxide synthase (nNOS. Quantitative reverse transcription PCR was used to analyze mRNAs for inflammatory markers. Functional motility and permeability analyses were performed in vitro. Phenotypic studies were also performed in 8 newborns with intestinal atresia. In the experimental model, the proportion of nNOS-immunoreactive neurons was similar in proximal and distal segments (6.7±4.6% vs 5.6±4.2%, p = 0.25, but proximal segments contained a higher proportion of ChAT-immunoreactive neurons (13.2±6.2% vs 7.5±4.3%, p = 0.005. Phenotypic changes were associated with a 100-fold lower concentration-dependent contractile response to carbachol and a 1.6-fold higher EFS-induced contractile response in proximal compared to distal segments. Transcellular (p = 0.002 but not paracellular permeability was increased. Comparison with controls showed that modifications involved not only proximal but also distal segments. Phenotypic studies in human atresia confirmed the changes in ChAT expression. CONCLUSION: Experimental atresia in fetal rat induces differential myenteric plexus phenotypical as well as functional changes (motility and permeability between the two sides of the obstruction. Delineating these changes might help to identify markers predictive of motility dysfunction and to define guidelines for post

  2. Biliary System Architecture: Experimental Models and Visualization Techniques

    Czech Academy of Sciences Publication Activity Database

    Sarnová, Lenka; Gregor, Martin

    2017-01-01

    Roč. 66, č. 3 (2017), s. 383-390 ISSN 0862-8408 R&D Projects: GA MŠk(CZ) LQ1604; GA ČR GA15-23858S Institutional support: RVO:68378050 Keywords : Biliary system * Mouse model * Cholestasis * Visualisation * Morphology Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 1.461, year: 2016

  3. Tricuspid atresia

    Science.gov (United States)

    Tricuspid atresia is a type of heart disease that is present at birth ( congenital heart disease ), in which the tricuspid heart valve is missing or abnormally developed. The defect blocks blood flow from the ...

  4. Cholangiopathy with Respect to Biliary Innate Immunity

    Directory of Open Access Journals (Sweden)

    Kenichi Harada

    2012-01-01

    Full Text Available Biliary innate immunity is involved in the pathogenesis of cholangiopathies in cases of biliary disease. Cholangiocytes possess Toll-like receptors (TLRs which recognize pathogen-associated molecular patterns (PAMPs and play a pivotal role in the innate immune response. Tolerance to bacterial PAMPs such as lipopolysaccharides is also important to maintain homeostasis in the biliary tree, but tolerance to double-stranded RNA (dsRNA is not found. Moreover, in primary biliary cirrhosis (PBC and biliary atresia, biliary innate immunity is closely associated with the dysregulation of the periductal cytokine milieu and the induction of biliary apoptosis and epithelial-mesenchymal transition (EMT, forming in disease-specific cholangiopathy. Biliary innate immunity is associated with the pathogenesis of various cholangiopathies in biliary diseases as well as biliary defense systems.

  5. Facts about Pulmonary Atresia

    Science.gov (United States)

    ... Websites Information For… Media Policy Makers Facts about Pulmonary Atresia Recommend on Facebook Tweet Share Compartir Click ... pick up oxygen for the body. What is Pulmonary Atresia? Pulmonary atresia is a birth defect of ...

  6. Percutaneous transcholecystic approach for an experiment of biliary stent placement: an experimental study in dogs

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Tae Seok [Medical School of Gachon, Inchon (Korea, Republic of); Song, Ho Young; Lim, Jin Oh; Ko, Gi Young; Sung, Kyu Bo; Kim, Tae Hyung; Lee, Ho Jung [College of Medicine, Ulsan Univ., Seoul (Korea, Republic of)

    2002-06-01

    To determine, in an experimental study of biliary stent placement, the usefulness and safety of the percutaneous transcholecystic approach and the patency of a newly designed biliary stent. A stent made of 0.15-mm-thick nitinol wire, and 10 mm in diameter and 2 cm in length, was loaded in an introducer with an 8-F outer diameter. The gallbladders of seven mongrel dogs were punctured with a 16-G angiocath needle under sonographic guidance, and cholangiography was performed. After anchoring the anterior wall of the gallbladder to the abdominal wall using a T-fastener, the gallbladder body was punctured again under fluoroscopic guidance. The cystic and common bile ducts were selected using a 0.035-inch guide wire and a cobra catheter, and the stent was placed in the common bile duct. Post-stenting cholangiography was undertaken, and an 8.5-F drainage tube was inserted in the gallbladder. Two dogs were followed-up and sacrificed at 2,4 and 8 weeks after stent placement, respectively, and the other expired 2 days after stent placement. Follow-up cholangiograms were obtained before aninmal was sacrified, and a pathologic examination was performed. Stent placement was technically successful in all cases. One dog expired 2 days after placement because of bile peritonitis due to migration of the drainage tube into the peritoneal cavity, but the other six remained healthy during the follow-up period. Cholangiography performed before the sacrifice of each dog showed that the stents were patent. Pathologic examination revealed the proliferation of granulation tissue at 2 weeks, and complete endothelialization over the stents by granulation tissue at 8 weeks. Percutaneous transcholecystic biliary stent placement appears to be safe, easy and useful. After placement, the stent was patent during the follow-period.

  7. Oesophageal atresia

    Directory of Open Access Journals (Sweden)

    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

  8. Polyurethane-coated, self-expandable biliary stent: an experimental study.

    Science.gov (United States)

    Severini, A; Mantero, S; Tanzi, M C; Cigada, A; Salvetti, M; Cozzi, G; Motta, A

    1995-12-01

    We describe a self-expanding metallic biliary Gianturco-Rösch stent coated with polymeric material. The coating was designed to prevent the growth of neoplastic and reactive tissue within the biliary ducts. The stents were coated with a solvent-casting technique, which consists of dissolving polyurethane (polyether urethane or polycarbonate urethane) pellets in a solvent (dimethylacetamide), dipping the stent in the solution, and completely evaporating the solvent. In vitro mechanical characterization of the stent was performed to determine the adhesion of the coating to the metallic cage, the best introducer caliber for implantation of the device, and the relationship between the stent's diameter and radial stress. Reports in the literature on the biostability of polycarbonate urethane compared with polyether urethane prompted us to use the former material to coat the stents. The solvent technique gives a smooth internal surface of the stent wall, leaving in relief the coated structure of the stent on the external surface. The functional tests demonstrated that the coating did not compromise the original characteristics of the stent in terms of self-expandability, axial flexibility, and increased radial rigidity of the device. Functional tests verified coating stability and device handling, which are the first steps toward in vivo experimentation.

  9. Biliary Innate Immunity: Function and Modulation

    Directory of Open Access Journals (Sweden)

    Kenichi Harada

    2010-01-01

    Full Text Available Biliary innate immunity is involved in the pathogenesis of cholangiopathies in patients with primary biliary cirrhosis (PBC and biliary atresia. Biliary epithelial cells possess an innate immune system consisting of the Toll-like receptor (TLR family and recognize pathogen-associated molecular patterns (PAMPs. Tolerance to bacterial PAMPs such as lipopolysaccharides is also important to maintain homeostasis in the biliary tree, but tolerance to double-stranded RNA (dsRNA is not found. In PBC, CD4-positive Th17 cells characterized by the secretion of IL-17 are implicated in the chronic inflammation of bile ducts and the presence of Th17 cells around bile ducts is causally associated with the biliary innate immune responses to PAMPs. Moreover, a negative regulator of intracellular TLR signaling, peroxisome proliferator-activated receptor-γ (PPARγ, is involved in the pathogenesis of cholangitis. Immunosuppression using PPARγ ligands may help to attenuate the bile duct damage in PBC patients. In biliary atresia characterized by a progressive, inflammatory, and sclerosing cholangiopathy, dsRNA viruses are speculated to be an etiological agent and to directly induce enhanced biliary apoptosis via the expression of tumor necrosis factor-related apoptosis-inducing ligand (TRAIL. Moreover, the epithelial-mesenchymal transition (EMT of biliary epithelial cells is also evoked by the biliary innate immune response to dsRNA.

  10. Neonatal lupus mimicking extra hepatic biliary atresia.

    Science.gov (United States)

    Kanitkar, Madhuri; Rohini, K P V; Puri, Bipin; Nair, M N G

    2004-12-01

    Neonatal Lupus Erythematosus (NLE) is an uncommon condition manifesting with congenital complete heart block and occasionally other manifestations like hepatitis. Neonatal Lupus with severe cholestasis with positive anti SS-A/Ro and anti SS-B/La antinuclear antibodies in the mother and child is being reported.

  11. Biliary obstruction and wound healing, infection, renal function and bloodcoagulation : an experimental study in rats

    NARCIS (Netherlands)

    J.P. Snellen

    1984-01-01

    textabstractThe purpose of the study reported in this thesis, was to investigate the effects of biliary obstruction on wound healing, resistance to infection, renal function and blood coagulation. Disturbances in these processes are often reported in patients with obstructive jaundice, and may lead

  12. Placement of an implantable port catheter in the biliary stent: an experimental study in dogs

    Energy Technology Data Exchange (ETDEWEB)

    Ko, Gi Young; Lee, Im Sick; Choi, Won Chan [Asan Medical Center, Seoul (Korea, Republic of)

    2004-04-01

    To investigate the feasibility of port catheter placement following a biliary stent placement. We employed 14 mongrel dogs as test subject and after the puncture of their gaIl bladders using sonographic guidance, a 10-mm in diameter metallic stent was placed at the common duct. In 12 dogs, a 6.3 F port catheter was placed into the duodenum through the common duct and a port was secured at the subcutaneous space following stent placement. As a control group, an 8.5 F drain tube was placed into the gallbladder without port catheter placement in the remaining two dogs. Irrigation of the bile duct was performed every week by injection of saline into the port, and the port catheter was replaced three weeks later in two dogs. Information relating to the success of the procedure, complications and the five-week follow-up cholangiographic findings were obtained. Placement of a biliary stent and a port catheter was technically successful in 13 (93%) dogs, while stent migration (n=3), gallbladder rupture (n=1) and death (n=5) due to subcutaneous abscess and peritonitis also occurred. The follow-up was achieved in eight dogs (seven dogs with a port catheter placement and one dog with a drain tube placement). Irrigation of the bile duct and port catheter replacement were successfully achieved without any complications. Cholangiograms obtained five weeks after stent placement showed diffuse biliary dilation with granulation tissue formation. However, focal biliary stricture was seen in one dog with stent placement alone. Placement of a port catheter following biliary stent placement seems to be feasible. However, further investigation is necessary to reduce the current complications.

  13. Biliary system

    Science.gov (United States)

    The biliary system creates, moves, stores, and releases bile into the duodenum . This helps the body digest food. It also assists ... from the liver to the duodenum. The biliary system includes: The gallbladder Bile ducts and certain cells ...

  14. A pilot proof-of-concept study of a modified device for one-step endoscopic ultrasound-guided biliary drainage in a new experimental biliary dilatation animal model.

    Science.gov (United States)

    Lee, Tae Hoon; Choi, Jun Hyuck; Lee, Sang Soo; Cho, Hyun Deuk; Seo, Dong Wan; Park, Sang-Heum; Lee, Sung Koo; Kim, Myung-Hwan; Park, Do Hyun

    2014-05-21

    To evaluate the technical feasibility of a modified tapered metal tip and low profile introducer for one-step endoscopic ultrasound (EUS)-guided biliary drainage (EUS-BD) in a new experimental biliary dilatation porcine model. A novel dedicated device for one-step EUS-guided biliary drainage system (DEUS) introducer has size 3F tapered catheter with size 4F metal tip for simple puncture of the intestinal wall and liver parenchyma without graded dilation. A self-expandable metal stent, consisting of both uncovered and nitinol-covered portions, was preloaded into DEUS introducer. After establishment of a biliary dilatation model using endoscopic hemoclips or band ligation with argon plasma coagulation in 9 mini-pigs, EUS-BD using a DEUS was performed following 19-G needle puncture without the use of fistula dilation devices. One-step EUS-BD was technically successful in seven pigs [7/9 (77.8%) as intention to treat] without the aid of devices for fistula dilation from the high body of stomach or far distal esophagus to the intrahepatic (n = 2) or common hepatic (n = 5) duct. Primary technical failure occurred in two cases that did not show adequate biliary dilatation. In seven pigs with a successful bile duct dilatation, the technical success rate was 100% (7/7 as per protocol). Median procedure time from confirmation of the dilated bile duct to successful placement of a metallic stent was 10 min (IQR; 8.9-18.1). There were no immediate procedure-related complications. Modified tapered metal tip and low profile introducer may be technically feasible for one-step EUS-BD in experimental porcine model.

  15. Histopathological and Follicular Atresia Assessment of Rat’s Ovarian Tissue Following Experimental Chronic Spinal Cord Injury

    Directory of Open Access Journals (Sweden)

    Sam Zarbakhsh

    2017-06-01

    Full Text Available Background One of the important side effects in spinal cord injuries in both genders is sexual dysfunction. This study aimed at investigating histopathological changes of ovaries in the chronic phase after spinal cord injury. Methods Animals (adult female rats were divided to the following groups, Control (Co, Sham (Sh, and Spinal Cord Injury (SCI; each group contained 18 rats. The spinal cord of SCI animals was transected by the bilateral laminectomy method on level T9, while the rats of the sham group were incised in the same location without any spinal cord injury. Animals were sacrified at 7th, 14th and 21st day postsurgical intervention, then the body weight, and the weight and volume of ovaries were measured. The slices were stained by hematoxylin and eosin and periodic acid Schiff methods. The histomorphometric changes of the diameter of the follicle and ovum, and the thickness of granulosa layer were measured in different kinds of follicles including, Unilaminar Primary Follicle (UPF, Multilaminar Primary Follicle (MPF, Secondary Follicle (SF, and Tertiary Follicle (TF. Results The results showed that the animal weights were decreased in three SCI groups (P = 0.018. In the SCI groups, the diameter of follicle and ovum and the thickness of granulosa layer were significantly decreased in different kinds of follicles (P = 0.012. Also the thickness of zona pellucida and theca interna were significantly decreased in UPF, SF, and TF in the SCI groups on 14th and 21st day (P = 0.024. The histopathologic examination revealed widespread ovarian follicle atresia in the SCI groups on the 14th and 21st day, including numerous cell debris and inflammatory cells in the antrum atretic follicles. The ovarian stroma showed edema, fibrosis, hypercellularity, and vasodilation in the SCI group, compared to the sham or control groups. Conclusions The histopathlogic data indicated that after spinal cord injury many histologic parameter changes occurred and

  16. Emerging concepts in biliary repair and fibrosis.

    Science.gov (United States)

    Fabris, Luca; Spirli, Carlo; Cadamuro, Massimiliano; Fiorotto, Romina; Strazzabosco, Mario

    2017-08-01

    Chronic diseases of the biliary tree (cholangiopathies) represent one of the major unmet needs in clinical hepatology and a significant knowledge gap in liver pathophysiology. The common theme in cholangiopathies is that the target of the disease is the biliary tree. After damage to the biliary epithelium, inflammatory changes stimulate a reparative response with proliferation of cholangiocytes and restoration of the biliary architecture, owing to the reactivation of a variety of morphogenetic signals. Chronic damage and inflammation will ultimately result in pathological repair with generation of biliary fibrosis and clinical progression of the disease. The hallmark of pathological biliary repair is the appearance of reactive ductular cells, a population of cholangiocyte-like epithelial cells of unclear and likely mixed origin that are able to orchestrate a complex process that involves a number of different cell types, under joint control of inflammatory and morphogenetic signals. Several questions remain open concerning the histogenesis of reactive ductular cells, their role in liver repair, their mechanism of activation, and the signals exchanged with the other cellular elements cooperating in the reparative process. This review contributes to the current debate by highlighting a number of new concepts derived from the study of the pathophysiology of chronic cholangiopathies, such as congenital hepatic fibrosis, biliary atresia, and Alagille syndrome. Copyright © 2017 the American Physiological Society.

  17. Biliary sludge.

    Science.gov (United States)

    Ko, C W; Sekijima, J H; Lee, S P

    1999-02-16

    Biliary sludge was first described with the advent of ultrasonography in the 1970s. It is defined as a mixture of particulate matter and bile that occurs when solutes in bile precipitate. Its composition varies, but cholesterol monohydrate crystals, calcium bilirubinate, and other calcium salts are the most common components. The clinical course of biliary sludge varies, and complete resolution, a waxing and waning course, and progression to gallstones are all possible outcomes. Biliary sludge may cause complications, including biliary colic, acute pancreatitis, and acute cholecystitis. Clinical conditions and events associated with the formation of biliary sludge include rapid weight loss, pregnancy, ceftriaxone therapy, octreotide therapy, and bone marrow or solid organ transplantation. Sludge may be diagnosed on ultrasonography or bile microscopy, and the optimal diagnostic method depends on the clinical setting. This paper proposes a protocol for the microscopic diagnosis of sludge. There are no proven methods for the prevention of sludge formation, even in high-risk patients, and patients should not be routinely monitored for the development of sludge. Asymptomatic patients with sludge can be managed expectantly. If patients with sludge develop symptoms or complications, cholecystectomy should be considered as the definitive therapy. Further studies of the pathogenesis, natural history, and clinical associations of biliary sludge will be essential to our understanding of gallstones and other biliary tract abnormalities.

  18. Biliary ascariasis

    Energy Technology Data Exchange (ETDEWEB)

    Mensing, M.; Cruz y Rivero, M.A.; Alarcon Hernandez, C.; Garcia Himmelstine, L.; Vogel, H.

    1986-06-01

    Biliary ascariasis is a complication of intestinal ascariasis. This results in characteristic findings in the intravenous cholangiocholecystogram and in the sonogram. Characteristic signs of biliary ascariasis are, in the longitudinal section, the 'strip sign', 'spaghetti sign', 'inner tube sign', and in transverse section 'a bull's eye in the triple O'. The helminth can travel from out of the biliary duct system back into the intestinum, so that control examinations can even be negative.

  19. Application of a silver coating on plastic biliary stents to prevent biofilm formation: an experimental study using electron microscopy

    OpenAIRE

    Yamabe, Akane; Irisawa, Atsushi; Wada, Ikuo; Shibukawa, Goro; Fujisawa, Mariko; Sato, Ai; Igarashi, Ryo; Maki, Takumi; Hoshi, Koki

    2016-01-01

    Background and study aims: Biliary stent dysfunction is mainly caused by biliary sludge that forms as a result of bacterial adherence and subsequent biofilm formation on the inner surface of the stent. Silver ions arewell known to have excellent antimicrobial activity against a wide range of microorganisms. In this study, we designed and constructed silver-coated plastic stent (PS) and investigated whether the silver coating prevented bacterial adherence and biofilm formation through the use ...

  20. Rectal atresia with rectolabial fistula

    OpenAIRE

    Sharma S.; Upadhyaya V; Pandey A; Gangopadhyay A

    2008-01-01

    Rectal atresia is a rare condition in which the anus and sphincter muscles are normally developed, with usually no fistulous communication with the urinary tract. We describe an unusual case of membranous rectal atresia with recto-labial fistula. It was treated by blind perforation of rectal membrane with lay opening of fistlous tract.

  1. Biliary ascariasis

    Directory of Open Access Journals (Sweden)

    Sivakumar K

    2007-01-01

    Full Text Available Ascaris lumbricoides, or the common roundworms, has a worldwide distribution but is mainly seen in Asian and Latin American countries. In humans the usual habitat is in the small intestine, mainly in the jejunum. But if the worm load is high, which may go up to a thousand worms, the worms tend to migrate away from the usual site of habitat. This brief paper aims at highlighting the condition of biliary ascariasis through the authors′ experience in managing three such cases over a period of 1 year. The clinical features which arouse suspicion of biliary ascariasis, as well as diagnostic modalities and management, are described. Initially the cases were managed conservatively, but we conclude that if worms are retained in biliary tree for more than 10 days, surgery should be performed; since retention of degraded and calcified dead worm can occur, and this can lead to complications which are difficult to manage.

  2. A new clarification method to visualize biliary degeneration during liver metamorphosis in sea lamprey (Petromyzon marinus)

    Science.gov (United States)

    Chung-Davidson, Yu-Wen; Davidson, Peter J.; Scott, Anne M.; Walaszczyk, Erin J.; Brant, Cory O.; Buchinger, Tyler; Johnson, Nicholas S.; Li, Weiming

    2014-01-01

    Biliary atresia is a rare disease of infancy, with an estimated 1 in 15,000 frequency in the southeast United States, but more common in East Asian countries, with a reported frequency of 1 in 5,000 in Taiwan. Although much is known about the management of biliary atresia, its pathogenesis is still elusive. The sea lamprey (Petromyzon marinus) provides a unique opportunity to examine the mechanism and progression of biliary degeneration. Sea lamprey develop through three distinct life stages: larval, parasitic, and adult. During the transition from larvae to parasitic juvenile, sea lamprey undergo metamorphosis with dramatic reorganization and remodeling in external morphology and internal organs. In the liver, the entire biliary system is lost, including the gall bladder and the biliary tree. A newly-developed method called “CLARITY” was modified to clarify the entire liver and the junction with the intestine in metamorphic sea lamprey. The process of biliary degeneration was visualized and discerned during sea lamprey metamorphosis by using laser scanning confocal microscopy. This method provides a powerful tool to study biliary atresia in a unique animal model.

  3. Biliary cystadenoma

    Science.gov (United States)

    Bartolome, Miguel A Hernandez; Ruiz, Sagrario Fuerte; Romero, Israel Manzanedo; Lojo, Beatriz Ramos; Prieto, Ignacio Rodriguez; Alvira, Luis Gimenez; Carreño, Rosario Granados; Esteban, Manuel Limones

    2009-01-01

    The diagnosis of cystadenoma is rare, even more so when located in the extrahepatic bile duct. Unspecific clinical signs may lead this pathology to be misdiagnosed. The need for pathological anatomy in order to distinguish cystadenomas from simple biliary cysts is crucial. The most usual treatment nowadays is resection of the bile duct, together with cholecystectomy and Roux-en-Y reconstruction. PMID:19630118

  4. Biliary cystadenoma

    Institute of Scientific and Technical Information of China (English)

    Miguel A Hernandez Bartolome; Sagrario Fuerte Ruiz; Israel Manzanedo Romero; Beatriz Ramos Lojo; Ignacio Rodriguez Prieto; Luis Gimenez Alvira; Rosario Granados Carreno; Manuel Limones Esteban

    2009-01-01

    The diagnosis of cystadenoma is rare, even more so when located in the extrahepatic bile duct. Unspecific clinical signs may lead this pathology to be misdiagnosed. The need for pathological anatomy in order to distinguish cystadenomas from simple biliary cysts is crucial. The most usual treatment nowadays is resection of the bile duct, together with cholecystectomy and Roux-en-Y reconstruction.

  5. Biopsy - biliary tract

    Science.gov (United States)

    Cytology analysis - biliary tract; Biliary tract biopsy ... A sample for a biliary tract biopsy can be obtained in different ways. A needle biopsy can be done if you have a well-defined tumor. The biopsy site ...

  6. Pattern and Survival of Biliary Atresia Patients; Experience in ...

    African Journals Online (AJOL)

    1Departments of Surgery, Pediatric Surgery Unit, 4Pediatrics, University of Port Harcourt Teaching Hospital, .... cirrhosis. At this stage, liver transplantation would have been the option for the patients but this service is not yet available in. Nigeria. Efficient follow‑up and early detection of complications in patients who have ...

  7. European biliary atresia registries: summary of a symposium

    DEFF Research Database (Denmark)

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

    2008-01-01

    with variable follow-up revealed an overall survival of 78% (range from 41% to 92%), of whom 342 patients (37%) have had liver transplants. Survival with native liver ranged from 14% to 75%. There was a marked variance in reported management and outcome by country (e.g., referral patterns, timing of surgery...

  8. Successful Use of Biliary Duct Balloon Dilator in Repairing Post-Surgical Esophageal Stricture in premature infant

    Directory of Open Access Journals (Sweden)

    Imad Absah

    2015-06-01

    Full Text Available Congenital esophageal atresia needs to be surgically corrected as soon as possible. Some will present with post surgical stricture that needs to be dilated by a balloon dilator. This procedure is difficult in premature born due to infant esophageal size and availability of equipments. We herby report the first case in a premature infant with post surgical esophageal stricture of esophageal atresia by the use of biliary duct balloon dilator.

  9. Congenital pyloric atresia: clinical features, diagnosis, associated ...

    African Journals Online (AJOL)

    Background Congenital pyloric atresia (CPA) is very rare and usually seen as ... anomalies. Epidermolysis bullosa was seen in eight (40%) patients and multiple intestinal atresias in five (25%). Three patients had associated esophageal atresia. Pyloric diaphragm .... brane, without a muscular component (pyloric membrane.

  10. Biliary endoprosthesis

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, Jung Hyeok; Kim, Yong Sun; Jang, Byung Won; Kim, Tae Hun; Kim, Yong Joo; Kang, Duk Sik [Kyungpook National University College of Medicine, Daegu (Korea, Republic of)

    1986-02-15

    Biliary endoprosthesis could provide permanent internal biliary drainage in patient with obstructive jaundice with advantages of absence of troublesome external catheter and no loss of fluid, bile and electrolytes over external drainage. Incidence of sepsis and cholangitis be lowered. Endoprosthesis was performed in 18 patients of obstructive jaundice from January, 1985 to December, 1985 at Department of Radiology, Kyungpook National University Hospital. The results are as follows 2. The cases of obstructive jaundice included bile duct cancer in 11, stomach ca. metastasis in 3, ca. of ampulla of Vater in 1, ca. of head of pancreas in 1, CBD cancer with postop. recurrence in 1, and impacted CBD stone in 1 case. 2. The levels of obstruction were at the trifurcation in 8, CHD in 5, proximal CBD in 3, and distal CBD in 2 cases. 3. Decline of serum bilirubin level was noted in 15 cases with the most rapid decline within 1 week after the procedure. 4. The complication occurred in 5 cases. The three cases were recovered spontaneously, but one died of bile peritonitis, another experienced obstruction of endoprosthesis. 5. The endoprosthesis is beneficial in treatment of obstructive jaundice for which surgery is not indicated, and saving expensive abdominal surgery and less advantageous percutaneous external drainage, hence enhancing life quality.

  11. [Biliary lithiasis].

    Science.gov (United States)

    Payen, Jean-Louis; Muscari, Fabrice; Vibert, Eric; Ernst, Olivier; Pelletier, Gilles

    2011-06-01

    No treatment is recommended for silent gallstones. The diagnosis of acute cholecystitis is based on clinical and biological signs and on abdominal sonography. Early laparoscopic cholecystectomy is the treatment of choice, except in case of severe (grade III) cholecystitis where a percutaneous cholecystostomy associated with antibiotic therapy is recommended. The diagnostic accuracy of abdominal sonography for the diagnosis of common bile duct stones is poor. A second-line MR cholangiopancreatography or an endoscopic sonography is often needed to confirm the diagnosis. The treatment of acute cholangitis is based on both antibiotic therapy and biliary drainage. Results of the treatment of common bile duct stone with either laparoscopic surgery or with the combined endoscopic sphincterotomy plus laparoscopic cholecystectomy are comparable when performed by well-trained practitionners. The choice of the method should be based on the locally available treatment. Copyright © 2011. Published by Elsevier Masson SAS.

  12. Application of a silver coating on plastic biliary stents to prevent biofilm formation: an experimental study using electron microscopy.

    Science.gov (United States)

    Yamabe, Akane; Irisawa, Atsushi; Wada, Ikuo; Shibukawa, Goro; Fujisawa, Mariko; Sato, Ai; Igarashi, Ryo; Maki, Takumi; Hoshi, Koki

    2016-10-01

    Background and study aims: Biliary stent dysfunction is mainly caused by biliary sludge that forms as a result of bacterial adherence and subsequent biofilm formation on the inner surface of the stent. Silver ions arewell known to have excellent antimicrobial activity against a wide range of microorganisms. In this study, we designed and constructed silver-coated plastic stent (PS) and investigated whether the silver coating prevented bacterial adherence and biofilm formation through the use of electron microscopy. Material and methods: The polyurethane PS with/without silver coating were prepared in 6-inch segments. The silver-based antimicrobial agents were electrostatically applied onto the stent surface. The stents were then immersed for 5 weeks in infected human bile juice obtained from a patient with cholangitis, and electron microscopy was used to investigate the ability of the modified PS to prevent bacterial adherence and biofilm formation. Results: The bacterial flora did not change before and after immersion of stents in both the group with and without silver coating. Electron microscopic observation revealed meshwork-like structures around the bacteria, characteristic of biofilm-forming bacteria, in all stents from the control group (6/6, 100 %). On the other hand, a limited number of bacteria were observed in all stents in the silver-coated group, and no apparent biofilm formation was observed (0/6, 0 %). Conclusions: The significance of the findings from our study is the ability of silver-coated PS to prevent biofilm formation on the stent surface, which results in the prevention of stent occlusion.

  13. The Combination of Gastroschisis, Jejunal Atresia, and Colonic Atresia in a Newborn

    Directory of Open Access Journals (Sweden)

    Zachary Bauman

    2015-01-01

    Full Text Available We encountered a rare case of gastroschisis associated with jejunal atresia and colonic atresia. In our case, the jejunal atresia was not discovered for 27 days after the initial abdominal wall closure. The colonic atresia was not discovered for 48 days after initial repair of the gastroschisis secondary to the rarity of the disorder. Both types of atresia were repaired with primary hand-sewn anastomoses. Other than the prolonged parenteral nutrition and hyperbilirubinemia, our patient did very well throughout his hospital course. Based on our case presentation, small bowel atresia and colonic atresia must be considered in patients who undergo abdominal wall closure for gastroschisis with prolonged symptoms suggestive of bowel obstruction. Our case report also demonstrates primary enteric anastomosis as a safe, well-tolerated surgical option for patients with types of intestinal atresia.

  14. Preoperative management of children with esophageal atresia: current perspectives

    Directory of Open Access Journals (Sweden)

    Parolini F

    2017-01-01

    Full Text Available Filippo Parolini,1 Anna Lavinia Bulotta,1 Sonia Battaglia,1 Daniele Alberti1,2 1Department of Pediatric Surgery, “Spedali Civili” Children’s Hospital, 2Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy Abstract: Esophageal atresia remains one of the most challenging congenital anomalies of the newborn. In recent years, because of the advances in prenatal diagnosis, neonatal critical care, and surgical procedures, overall outcomes have improved substantially, including for premature children. Nowadays, most of the research is focused on medium- and long-term morbidity, with particular reference to respiratory and gastroesophageal problems; the high frequency of late sequelae in esophageal atresia warrants regular and multidisciplinary checkups throughout adulthood. Surprisingly, there are few studies on the impact of prenatal diagnosis and there is continuing debate over the prenatal and preoperative management of these complex patients. In this review, we analyze the literature surrounding current knowledge on the management of newborns affected by esophageal atresia, focusing on prenatal management and preoperative assessment. Keywords: prenatal diagnosis, esophageal atresia, tracheoesophageal fistula, ultrasound scan, tracheobronchoscopy

  15. Pyloric atresia associated with epidermolysis bullosa

    Energy Technology Data Exchange (ETDEWEB)

    Garcia Hernandez, J.B.; Orense, M.; Celorio, C.; Canga, C.

    1987-07-01

    Since the first reported case of pyloric atresia and epidermolysis bullosa in 1977 by Korber and Glasson, this association has been firmly established. We present a new case giving particular emphasis to the theory that pyloric atresia is secondary to the primary disease epidermolysis bullosa.

  16. Umbilical cord ulceration and jejunal atresia

    African Journals Online (AJOL)

    The association between umbilical cord ulceration and congenital intestinal atresia is being increasingly reported and carries a high mortality. We report on a case of jejunal atresia associated with massive fetal haemorrhage from an umbilical cord ulcer. Fetal distress noted on continuous fetal heart monitoring allowed for ...

  17. [Esophageal atresia in the Goldenhar syndrome].

    Science.gov (United States)

    Ayuso Velasco, R; Torres Aguirre, A; Enríquez Zarabozo, E; Galán Gómez, E; Blesa Sánchez, E

    2010-01-01

    Among the multiple congenital defects associated to esophagueal atresia, the characteristic ones of the Goldenhar syndrome usually are not included. The high incidence has been reported, about 5% of esophagueal atresia in patients with Goldenhar syndrome. Our experience includes two patients with this association who presented anesthetic problems and surgical complications associated with gastroesophageal reflux and esophageal anastomosis.

  18. Biliary Epithelial Apoptosis, Autophagy, and Senescence in Primary Biliary Cirrhosis

    Directory of Open Access Journals (Sweden)

    Motoko Sasaki

    2010-01-01

    Full Text Available Primary biliary cirrhosis (PBC is a chronic cholestatic liver disease characterized serologically by the high prevalence of anti-mitochondrial autoantibodies (AMAs and histologically by the cholangitis of small bile ducts, eventually followed by extensive loss of the small bile duct. An autoimmune pathogenesis is suggested by clinical and experimental studies, but there remain issues regarding the etiology, the significance of AMAs in the pathogenesis of bile duct lesions, and so on. The unique properties of apoptosis in biliary epithelial cells (BECs, in which there is exposure of autoantigen to the effectors of the immune system, are proposed to be a cause of bile duct lesions in PBC. Recent progress disclosed that cellular senescence and autophagy are involved in bile duct lesions in PBC. Senescent BECs may modulate the periductal microenvironment by expressing senescence-associated secretory phenotypes, including various chemokines, and contribute to the pathogenesis of bile duct lesions in PBC.

  19. Estudo ultrassonográfico morfométrico do fígado e trato biliar de suínos submetidos a obstrução biliar experimental Sonographic morphometry of the liver and biliary tract in porcine models submitted to experimental biliary obstruction

    Directory of Open Access Journals (Sweden)

    Aline Gomes de Campos

    2013-04-01

    Full Text Available OBJETIVO: Comparar as alterações anatômicas decorrentes de um quadro de icterícia obstrutiva experimental induzida em suínos nos períodos pré e pós-operatório por meio de exame ultrassonográfico. MATERIAIS E MÉTODOS: Seis suínos da raça Landrace, com 36 dias de idade, foram submetidos a obstrução biliar completa mediante ligadura do ducto colédoco por cirurgia videolaparoscópica. RESULTADOS: Não ocorreram dificuldades na execução dos procedimentos obstrutivos e a recuperação cirúrgica foi eficiente. Decorridos sete dias, os animais apresentaram icterícia, bilirrubinúria e acolia fecal. O exame ultrassonográfico comparativo permitiu visualizar hepatomegalia, colecistomegalia e aumento no calibre do ducto colédoco em todos os animais, assim como alterações decorrentes da colestase. A avaliação morfométrica revelou aumento significativo nos diâmetros da vesícula biliar e do lobo hepático lateral esquerdo. CONCLUSÃO: Os suínos representam um modelo experimental adequado de icterícia obstrutiva, e o exame ultrassonográfico demonstrou-se sensível e relevante no diagnóstico das alterações decorrentes de obstrução biliar extra-hepática nesses animais.OBJECTIVE: To compare, by means of ultrasonography, pre- and postoperative anatomical changes arising from experimentally induced obstructive jaundice in porcine models. MATERIALS AND METHODS: Six 36-day-old Landrace pigs underwent laparoscopically induced complete biliary obstruction by common bile duct ligation. RESULTS: No difficulty was faced during the procedures and the surgical recovery was uneventful. After seven days, the animals showed jaundice, bilirubinuria and acholic stools. Comparative ultrasonography allowed visualization of hepatomegaly, cholecystomegaly and increased caliber of the common bile duct in all the animals, as well as changes resulting from cholestasis. The morphometric analysis revealed a significant increase in diameter of the

  20. Surgical management of oesophageal atresia.

    Science.gov (United States)

    Teague, Warwick J; Karpelowsky, Jonathan

    2016-06-01

    There have been major advances in the surgery for oesophageal atresia (OA) and tracheo-oesophageal fistula(TOF) with survival now exceeding 90%. The standard open approach to OA and distal TOF has been well described and essentially unchanged for the last 60 years. Improved survival in recent decades is most attributable to advances in neonatal anaesthesia and perioperative care. Recent surgical advances include the use of thoracoscopic surgery for the repair of OA/TOF and in some centres isolated OA, thereby minimising the long term musculo-skeletal morbidity associated with open surgery. The introduction of growth induction by external traction (Foker procedure) for the treatment of long-gap OA has provided an important tool enabling increased preservation of the native oesophagus. Despite this, long-gap OA still poses a number of challenges, and oesophageal replacement still may be required in some cases. Copyright © 2016 Elsevier Ltd. All rights reserved.

  1. Primary biliary cirrhosis: Report

    Directory of Open Access Journals (Sweden)

    Filipović Branka

    2002-01-01

    Full Text Available Chronic non-suppurative destructive cholangitis, the so-called primary biliary cirrhosis, is characterised by changes, which occur in intrahepatic bile ducts in early stages and in hepatic parenchyma as the disease progresses. The disease gradually evolves into the full-blown picture of biliary cirrhosis. Primary biliary cirrhosis predominantly affects women between 35 and 60 years of age in all social classes and in all races. Our patient was a woman, old 78 years old who admitted for treatment of hypertrophie cardiomyopathy. During the routine laboratory exploration signs of cholestasis were noted: higher values of alkaline phosphatase and gamma glutamyl transferase, combined with low level of platelets, probably of autoimmune origin. Hypercholesterolaemia (7.8 mmol/L associated with normal values of triglycerides was observed. The main criterion for establishing the diagnosis of primary biliary cirrhosis was the titer of antimito-chondrial antibodies in the serum, which was 1:640. At the same time, she had a urinary infection, caused by Escherichia coli, which confirmed possible relationship between primary biliary cirrhosis and occurence of some Gramm negative bacteria, reported elsewhere. On the other hand, biopsy of the liver was just an auxiliary method, serving for the confirmation of diagnosis. Ursodeoxycholic acid was used as the main drug in the therapy of primary biliary cirrhosis. This case of primary biliary cirrhosis is a worth report because of the comorbidity with cardiac symptoms, which were covering symptoms of hepatic disorder.

  2. Closure of the cystic duct during laparoscopic cholecystectomy with a new feedback-controlled bipolar sealing system in case of biliary obstruction--an experimental study in pigs.

    Science.gov (United States)

    Shamiyeh, A; Vattay, P; Tulipan, L; Schrenk, P; Bogner, S; Danis, J; Wayand, W

    2004-01-01

    The excellent results of new devices like the new bipolar feedback-controlled sealing system (LigaSure) for closure of blood vessels encouraged surgeons to use these instruments for other structures like bile ducts. The aim of this study was to evaluate the feasibility of closure of cystic duct in case of biliary obstruction. Ten domestic pigs underwent laparoscopic cholecystectomy sealing the cystic duct with LigaSure. The common bile duct was closed with an endoclip to create a biliary hypertension. On the 12th postoperative day blood samples were taken for liver enzymes. At autopsy on day 15 the pigs were investigated for bile leaks or biliary peritonitis. The cystic duct was resected for histological examination. Seven pigs survived, one pig died during introduction of anesthesia, one on the 1st and one on the 2nd postoperative day without any findings at the autopsy. One pig out of 7 had a bile leak; the other 6 were without any sign of leakage. Histologically 3 pigs had a regular coagulation zone at the cystic duct, 3 had a total necrosis, one a partial necrosis of the mucosa only. Though there was only one insufficiency, the feedback-controlled bipolar vessel sealer cannot be recommended for biliary surgery with regard to the high rate of necrosis stated in our experiment.

  3. Choanal atresia in siblings; Case report | Kaitesi | East and Central ...

    African Journals Online (AJOL)

    Choanal atresia is an uncommon and often poorly recognized cause of unilateral or bilateral nasal obstruction. This report describes the case of bilateral choanal atresia in two consecutive siblings and describes the methods of treatment offered.

  4. Primary biliary cirrhosis.

    Science.gov (United States)

    Carey, Elizabeth J; Ali, Ahmad H; Lindor, Keith D

    2015-10-17

    Primary biliary cirrhosis is a chronic cholestatic liver disease characterised by destruction of small intrahepatic bile ducts, leading to fibrosis and potential cirrhosis through resulting complications. The serological hallmark of primary biliary cirrhosis is the antimitochondrial antibody, a highly disease-specific antibody identified in about 95% of patients with primary biliary cirrhosis. These patients usually have fatigue and pruritus, both of which occur independently of disease severity. The typical course of primary biliary cirrhosis has changed substantially with the introduöction of ursodeoxycholic acid (UDCA). Several randomised placebo-controlled studies have shown that UDCA improves transplant-free survival in primary biliary cirrhosis. However, about 40% of patients do not have a biochemical response to UDCA and would benefit from new therapies. Liver transplantation is a life-saving surgery with excellent outcomes for those with decompensated cirrhosis. Meanwhile, research on nuclear receptor hormones has led to the development of exciting new potential treatments. This Seminar will review the current understanding of the epidemiology, pathogenesis, and natural history of primary biliary cirrhosis, discuss management of the disease and its sequelae, and introduce research on new therapeutic options. Copyright © 2015 Elsevier Ltd. All rights reserved.

  5. Biliary-pleural fistulas without biliary obstruction: percutaneous catheter management

    National Research Council Canada - National Science Library

    Feld, R; Wechsler, RJ; Bonn, J

    1997-01-01

    ...: Our purpose was to report the use of percutaneous catheter drainage as a therapeutic option in the management of three patients with biliary-pleural fistulas without biliary obstructions. CONCLUSION...

  6. Laryngeal atresia in the newborn: surgical implications.

    Science.gov (United States)

    Hicks, B A; Contador, M P; Perlman, J M

    1996-10-01

    Congenital atresia of the larynx is a rare, life-threatening anomaly in which early recognition and appropriate perinatal management are essential for survival. Few long-term survivors have been reported in the literature, with most documented cases studied at necropsy. The authors present a case of a full-term male newborn with Smith and Bain Type I laryngeal atresia, who has survived and developed normally during the 10-months since birth. Rapidly progressive cyanosis following umbilical cord occlusion, lack of phonation, and no air movement with respiratory efforts are typically present and should alert the clinician to the possibility of laryngeal atresia. Positive pressure ventilatory assistance may be possible through a patent pharyngoglottic duct or tracheoesophageal fistula until a surgical airway is established. If this is not possible, emergent tracheostomy may be a lifesaving procedure in the first minutes of life.

  7. Methotrexate for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Giljaca, Vanja; Poropat, Goran; Stimac, Davor

    2010-01-01

    Methotrexate has been used to treat patients with primary biliary cirrhosis as it possesses immunosuppressive properties. The previously prepared version of this review from 2005 showed that methotrexate seemed to significantly increase mortality in patients with primary biliary cirrhosis. Since...

  8. Atresia de tráquea

    Directory of Open Access Journals (Sweden)

    Rafael Amador Morán

    2010-12-01

    Full Text Available La atresia de tráquea es una afección infrecuente en el recién nacido y la mayoría de las veces se asocia a otras malformaciones complejas sobre las cuales se han registrado en la literatura médica no más de 100 casos. Se documenta el caso de un recién nacido varón con atresia traqueal y fístula traqueoesofágica, anomalías cardíacas y gastrointestinales, que tuvo una supervivencia de 81 h.

  9. Bezafibrate for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Rudic, Jelena S; Poropat, Goran; Krstic, Miodrag N

    2012-01-01

    Treatment of primary biliary cirrhosis is complicated. There are studies suggesting that bezafibrate, alone or in combination with ursodeoxycholic acid (UDCA), is effective in the treatment of primary biliary cirrhosis, but no systematic review has summarised the evidence yet.......Treatment of primary biliary cirrhosis is complicated. There are studies suggesting that bezafibrate, alone or in combination with ursodeoxycholic acid (UDCA), is effective in the treatment of primary biliary cirrhosis, but no systematic review has summarised the evidence yet....

  10. Dysphagia in children with repaired oesophageal atresia

    NARCIS (Netherlands)

    Coppens, C.H.; Engel-Hoek, L. van den; Scharbatke, H.E.; Groot, S.A. de; Draaisma, J.M.T.

    2016-01-01

    Dysphagia is a common problem in children with repaired oesophageal atresia (OA). Abnormalities in the oropharyngeal and oesophageal phase have hardly been studied. The aims of this study were to assess the prevalence of dysphagia in children with repaired OA and to identify and differentiate oral

  11. Umbilical cord ulceration and jejunal atresia

    African Journals Online (AJOL)

    CASE REPORT. The association between umbilical cord ulceration and congenital intestinal atresia is being increasingly reported and carries a high mortality. ... known to be associated with a number of other congenital anomalies, including ... rhesus positive and both HIV and syphilis serology were negative. A scan for ...

  12. [Temporary replacement of the common biliary duct by a silicone tube as an urgent repair of iatrogenic injury. Experimental study in pigs].

    Science.gov (United States)

    Napolitano, Daniel A; Rodríguez Bertola, Xavier; Sambuelli, Gabriela M; Vial, Luis H; Torrecillas, Daniel A

    2015-03-01

    Surgery of the biliary tract is complex, and its volume has increased with iatrogenic injuries and living donor transplantation. The aim of this study was to analyze if the common duct can be temporarily replaced. We used nine 18-20 kg pigs. They were operated on, and their bile duct was replaced by a 100% silicone tube. All pigs underwent laboratory tests, magnetic resonance imaging, intraoperative ultrasound, cholangiography and biliar manometry with pathological biopsy examination within 60 days from the initial surgery. All pigs survived the first surgery over 60 days without laboratory evidence of evident cholestasis. Nine pigs were re-operated on at 60 days showing dilated common bile duct and hepatic ducts doubling its original size without dilating the intrahepatic bile ducts. There were no clinical, relevant laboratory or biopsy signs showing cholestasis. This experience represents the initial intention to find an optimal situation and prosthesis for replacement of the thin biliary tract, in surgical emergencies or palliative situations. The silicon tube, is a positive answer that remains permeable. A non-fibrotic reaction was found that allows a posterior definitive procedure, maintaining a good nutritional status. Copyright © 2014 AEC. Publicado por Elsevier España, S.L.U. All rights reserved.

  13. Measurement of needle-tip bioimpedance to facilitate percutaneous access of the urinary and biliary systems: first assessment of an experimental system.

    Science.gov (United States)

    Roberts, William W; Fugita, Oscar E; Kavoussi, Louis R; Stoianovici, Dan; Solomon, Stephen B

    2002-02-01

    Percutaneous access to the renal collecting system or biliary system is most frequently performed under image guidance. However, current techniques lack a feedback mechanism to help confirm successful access. A percutaneous needle system has been developed consisting of a modified 18-gauge percutaneous access needle that measures tissue impedance at its needle tip. Initial results in utilizing this novel system to determine successful access into dilated kidney and gall bladder specimens are reported. Impedance measurements were recorded as the needle was precisely advanced through ex vivo kidney and liver/gall bladder specimens. In an anesthetized porcine model, impedance values were recorded with laparoscopic visualization as the needle was advanced percutaneously through abdominal wall, liver, and into gall bladder. A characteristic, reproducible drop in impedance was noted with successful entry into ex vivo distended kidney and gall bladder specimens. This feature was also noted during in vivo percutaneous cholecystostomy. A measurable, characteristic drop in tissue impedance signifies successful entry into the urinary and biliary systems. This impedance needle system may facilitate current percutaneous access techniques.

  14. Primary biliary cirrhosis

    African Journals Online (AJOL)

    1990-07-07

    Jul 7, 1990 ... Primary biliary cirrhosis (PBC) or chronic non-suppura.tive destructive cholangiohepatitis is rare in southern Afnca. Eight patients with this diagnosis were identified and fully investi- gated at Groote Schuur Hospital betwe.en 1980 and 1988. Seven patients were female, all were white or coloured, and.

  15. Primary biliary cirrhosis

    NARCIS (Netherlands)

    Hohenester, Simon; Oude-Elferink, Ronald P. J.; Beuers, Ulrich

    2009-01-01

    Primary biliary cirrhosis (PBC) is an immune-mediated chronic cholestatic liver disease with a slowly progressive course. Without treatment, most patients eventually develop fibrosis and cirrhosis of the liver and may need liver transplantation in the late stage of disease. PBC primarily affects

  16. [Pathophysiology of biliary lithiasis].

    Science.gov (United States)

    Poupon, Raoul

    2007-12-15

    Gallstones affect about 15% of adults in Europe. Cholesterol gallstone is the common form of the disease. Pigment stones, resulting from bilirubin precipitation feature particular clinical settings such as chronic hemolysis, intestinal malabsorption and various biliary tract diseases. Supersaturation of bile with cholesterol, increased formation of deoxycholic acid and gallbladder hypomotility are the main factors involved in gallstones pathogenesis.

  17. Biliary-pleural fistulas without biliary obstruction: percutaneous catheter management.

    Science.gov (United States)

    Feld, R; Wechsler, R J; Bonn, J

    1997-08-01

    Our purpose was to report the use of percutaneous catheter drainage as a therapeutic option in the management of three patients with biliary-pleural fistulas without biliary obstructions. In the proper clinical setting, the CT findings of liver dome laceration or abscess, diaphragm disruption, and pleural effusion should suggest biliary-pleural fistula. Percutaneous catheter management can be curative or serve a temporizing function before surgery.

  18. Prevalence of biliary ascariasis and its relation to biliary lithiasis.

    Science.gov (United States)

    Akhter, Nasima; Islam, S M Moinul; Mahmood, Saiyeeda; Hossain, Gazi Abul; Chakraborty, Ratan Kumar

    2006-03-01

    Hepatobiliary ultrasound was carried out on 2224 consecutive patients at the Center for Nuclear Medicine and Ultrasound and at one private diagnostic center in Mymensingh for ultrasound examination of different systems. The purpose of this study was to investigate the prevalence of biliary ascariasis and its association with other biliary diseases, specially biliary lithiasis. Data regarding the presence of stone, worm or other diseases were recorded accordingly in this prospective study. There were 952 male and 1272 female patients with an age range of 5-90 years. Biliary diseases were detected in 305 patients (13.71%), of whom 97 were male (10.19%) and 208 were female (16.35%). The most common biliary disease in both sexes was cholelithiasis (11.87%), which was found in 84 male patients (8.82%) and in 180 female patients (14.15%). Other diseases found were choledocholithiasis in 14 patients (3 males, 11 females), gall bladder mass in 9 patients (3 males, 6 females), common bile duct mass in 7 patients (4 male, 3 female) and biliary ascariasis in 10 patients (3 male, 7 female). Overall prevalence of biliary ascariasis was 0.45% (0.31% in male patients and 0.55% in female patients), and age range of patients with the condition was 6-50 years. No case of biliary ascariasis was associated with cholelithiasis or choledocholithiasis. Acute cholecystitis was associated with 8 cases (80%) of biliary ascariasis. Common sonographic findings in patients with biliary ascariasis were a single long, linear or curved echogenic structure within the bile duct, without acoustic shadowing. Other findings were gall bladder distention with sludge inside, an edematous wall and mildly dilated biliary tree. Prevalence of biliary ascariasis in the study was 0.45%, with incidence being higher in female subjects (0.55%). No correlation was found between biliary ascariasis and biliary lithiasis. Most of the cases of biliary ascariasis were associated with acute cholecystitis. We concluded

  19. Intrahepatic Biliary Cystadenoma With Intracystic Gallstone Formation

    OpenAIRE

    Shize Lei; Domenico, Don R.; Howard, John M.

    1994-01-01

    Biliary cystadenoma is a rare tumor of the liver. We describe a biliary cystadenoma of the left lobe of the liver with intracystic gallstone formation. This is the first report of stone formation in biliary cystadenoma of the liver.

  20. Familial recurrence of urethral stenosis/atresia.

    Science.gov (United States)

    Siebert, Joseph R; Walker, Martin P R

    2009-08-01

    We report the familial recurrence of urethral stenosis/atresia in two sibling fetuses with bladder outlet obstruction, severe oligohydramnios, and pulmonary hypoplasia. Urethral obstruction in the fetus, when severe, results in a dilated urinary bladder (megacystis) and associated urinary anomalies (hydroureter, hydronephrosis, renal dysplasia). Distention of the fetal abdomen, the result of megacystis or urinary ascites, leads to stretching and eventually hypoplasia or even absence of abdominal muscles. This constellation of findings, known by a variety of terms including "prune belly" syndrome, is associated with a variety of urethral changes, including posterior urethral valves and urethral stenosis/atresia. One fetus manifested unilateral postaxial polydactyly of the left hand. A microdeletion of 6p25.3, identified in mother and one fetus, is not associated with a gene known to be involved in urethral development and therefore of unknown significance. (c) 2009 Wiley-Liss, Inc.

  1. 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.

  2. MRI Findings in Biliary Cystadenoma

    OpenAIRE

    Can, Güray; Tuncel, Sedat Alpaslan; Gençhellaç, Hakan; Özpuyan, Fulya; İbiş, Cem

    2015-01-01

    Biliary cystadenoma is a rare cystic hepatic neoplasm. We herein present a 44 year-old female patient with biliary cystadenoma focusing on difficulties in diagnosis because of lack of specific findings with imaging, and the need for surgery. As biliary cystadenomas have high recurrence risks and malignant transformation potential, careful follow-up should not be underestimated. Proper surgical resection of the lesion may be considered the most suitable diagnostic and therapeutic procedure, an...

  3. Pyloric atresia-Three cases and review of literature

    Directory of Open Access Journals (Sweden)

    Sandesh V Parelkar

    2014-01-01

    Full Text Available Pyloric atresia (PA is a rare congenital anomaly that constitutes approximately 1% of all intestinal atresias, and its incidence is approximately 1 in 100,000 live births. PA may occur as an isolated condition or associated with other abnormalities, the most common being Junctional epidermolysis bullosa (JEB. Evidence suggests that PA-EB (Pyloric Atresia - Epidermolysis Bullosa Syndrome is a distinct entity. In this report, we present three cases of pyloric atresia, one of which was associated with Junctional epidermolysis bullosa. The literature on the subject is also reviewed.

  4. Acute Mastoiditis in a Newborn with Aural Atresia

    Directory of Open Access Journals (Sweden)

    K. Parpounas

    2012-01-01

    Full Text Available Acute mastoiditis in the newborn is a very rare disease. Herein we report a case of a 28-day-old child with right aural atresia and ipsilateral mastoiditis requiring mastoidectomy. To our knowledge, this is the youngest case reported in the literature. Issues on diagnosis and management of mastoiditis in cases of aural atresia are further discussed. Based on our experience and on previous reported cases we conclude that mastoiditis is difficult to diagnose in a child with aural atresia. Moreover, mastoidectomy may be necessary, although identification of the facial nerve and consequent treatment of the atresia are usually too difficult to perform simultaneously.

  5. Acute mastoiditis in a newborn with aural atresia.

    Science.gov (United States)

    Parpounas, K; Bouka, K; Athanasopoulos, J; Lamprou, M; Helmis, G; Vlastos, I M

    2012-01-01

    Acute mastoiditis in the newborn is a very rare disease. Herein we report a case of a 28-day-old child with right aural atresia and ipsilateral mastoiditis requiring mastoidectomy. To our knowledge, this is the youngest case reported in the literature. Issues on diagnosis and management of mastoiditis in cases of aural atresia are further discussed. Based on our experience and on previous reported cases we conclude that mastoiditis is difficult to diagnose in a child with aural atresia. Moreover, mastoidectomy may be necessary, although identification of the facial nerve and consequent treatment of the atresia are usually too difficult to perform simultaneously.

  6. Azathioprine for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Gong, Yanzhang; Christensen, E; Gluud, C

    2007-01-01

    Azathioprine is used for patients with primary biliary cirrhosis, but the therapeutic responses in randomised clinical trials have been conflicting.......Azathioprine is used for patients with primary biliary cirrhosis, but the therapeutic responses in randomised clinical trials have been conflicting....

  7. Colchicine for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Gong, Yanzhang; Gluud, C

    2004-01-01

    Colchicine has been used for patients with primary biliary cirrhosis because of its immunomodulatory and antifibrotic potential. The therapeutical responses to colchicine in randomised clinical trials were inconsistent.......Colchicine has been used for patients with primary biliary cirrhosis because of its immunomodulatory and antifibrotic potential. The therapeutical responses to colchicine in randomised clinical trials were inconsistent....

  8. Colchicine for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Gong, Yan; Gluud, Christian

    2005-01-01

    Colchicine is used for patients with primary biliary cirrhosis due to its immunomodulatory and antifibrotic potential. The results from randomized clinical trials have, however, been inconsistent. We conducted a systematical review to evaluate the effect of colchicine for primary biliary cirrhosis....

  9. Esophageal Replacement for Long-Gap Esophageal Atresia in a ...

    African Journals Online (AJOL)

    The management of esophageal atresia in a resourcelimited environment is plagued with challenges that often lead to poor outcome. The diagnosis and management of babies with long-gap esophageal atresia adds a new dimension to these challenges. We report the success of esophageal replacement surgery for a ...

  10. Situs inversus in association with duodenal atresia | Talabi | African ...

    African Journals Online (AJOL)

    Situs inversus in association with duodenal atresia is very rare. A high index of suspicion coupled with appropriate evaluation is necessary for diagnosis and operative planning. We report a case of a 5-day-old who presented with duodenal atresia associated with polysplenia and situs inversus with a review of the medical ...

  11. Apple-peel atresia presenting as foetal intestinal obstruction ...

    African Journals Online (AJOL)

    Apple-peel atresia or Type 3 jejuno-ileal atresia (JIA) is an uncommon cause of foetal intestinal obstruction. Bowel obstruction in the foetus is diagnosed on the prenatal ultrasonography only in 50% cases. We report a case in which foetal intestinal obstruction was diagnosed on prenatal ultrasonography. The child showed ...

  12. Umbilical cord ulceration and jejunal atresia | Mackay | South ...

    African Journals Online (AJOL)

    The association between umbilical cord ulceration and congenital intestinal atresia is being increasingly reported and carries a high mortality. We report on a case of jejunal atresia associated with massive fetal haemorrhage from an umbilical cord ulcer. Fetal distress noted on continuous fetal heart monitoring allowed for ...

  13. Imaging of autoimmune biliary disease.

    Science.gov (United States)

    Yeh, Melinda J; Kim, So Yeon; Jhaveri, Kartik S; Behr, Spencer C; Seo, Nieun; Yeh, Benjamin M

    2017-01-01

    Autoimmune biliary diseases are poorly understood but important to recognize. Initially, autoimmune biliary diseases are asymptomatic but may lead to progressive cholestasis with associated ductopenia, portal hypertension, cirrhosis, and eventually liver failure. The three main forms of autoimmune biliary disease are primary biliary cirrhosis, primary sclerosing cholangitis, and IgG4-associated cholangitis. Although some overlap may occur between the three main autoimmune diseases of the bile ducts, each disease typically affects a distinct demographic group and requires a disease-specific diagnostic workup. For all the autoimmune biliary diseases, imaging provides a means to monitor disease progression, assess for complications, and screen for the development of hepatobiliary malignancies that are known to affect patients with these diseases. Imaging is also useful to suggest or corroborate the diagnosis of primary sclerosing cholangitis and IgG4-associated cholangitis. We review the current literature and emphasize radiological findings and considerations for these autoimmune diseases of the bile ducts.

  14. Membranous variety of rectal atresia - primary management in a neonate

    Science.gov (United States)

    Jayaswal, Shalika; Shah, Hemanshi; Murthy, Keshav; Bhandarkar, Kailash; Makhija, Om Prakash

    2010-01-01

    Rectal atresia is a rare form of anorectal malformation, with reported incidence of 1 to 2% and membranous variety of rectal atresia is even rarer. Most reported cases have been dealt with a staged procedure which includes sigmoid colostomy. We diagnosed and classified the variety of rectal atresia by performing an X-Ray (invertogram along with the red rubber catheter in situ.). In lesser developed geographies where MRI is not readily available or not affordable, this simple test could be used to confirm the variety of rectal atresia. However, the usual fallacies of invertogram should be considered. Here we report a neonate with membranous variety of rectal atresia managed by transanal endoscopic fulguration using bugbee passed through the cystourethroscope, without a covering sigmoid stoma. PMID:21120069

  15. Membranous variety of rectal atresia - primary management in a neonate

    Directory of Open Access Journals (Sweden)

    Jayaswal Shalika

    2010-01-01

    Full Text Available Rectal atresia is a rare form of anorectal malformation, with reported incidence of 1 to 2% and membranous variety of rectal atresia is even rarer. Most reported cases have been dealt with a staged procedure which includes sigmoid colostomy. We diagnosed and classified the variety of rectal atresia by performing an X-Ray (invertogram along with the red rubber catheter in situ.. In lesser developed geographies where MRI is not readily available or not affordable, this simple test could be used to confirm the variety of rectal atresia. However, the usual fallacies of invertogram should be considered. Here we report a neonate with membranous variety of rectal atresia managed by transanal endoscopic fulguration using bugbee passed through the cystourethroscope, without a covering sigmoid stoma.

  16. Ursodeoxycholic acid for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Gong, Yan; Huang, Zhi Bi; Christensen, Erik

    2008-01-01

    Primary biliary cirrhosis is an uncommon autoimmune liver disease with unknown aetiology. Ursodeoxycholic acid (UDCA) has been used for primary biliary cirrhosis, but the effects remain controversial.......Primary biliary cirrhosis is an uncommon autoimmune liver disease with unknown aetiology. Ursodeoxycholic acid (UDCA) has been used for primary biliary cirrhosis, but the effects remain controversial....

  17. Follicular growth and atresia in the mouse

    Energy Technology Data Exchange (ETDEWEB)

    Oakberg, E. F.

    1978-01-01

    Follicles were classified on the basis of number of layers of follicle cells, presence and degree of development of the zona pellucida, and presence of an antrum. Formation of an antrum in follicles with less than 7-8 cell layers and/or presence of necrotic cells was considered indicative of degeneration. When classified in this manner, the data suggest that follicles and their contained oocytes are committed to either normal development or atresia by the time a third layer of granulosa cells is formed.

  18. Unilateral pulmonary vein atresia: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, So Hwa; Kim, Ki Jun [Dept. of Radiology, Incheon St. Mary' s Hospital, The Catholic University of Korea College of Medicine, Incheon (Korea, Republic of)

    2014-01-15

    Unilateral pulmonary vein atresia is a rare congenital anomaly. Its symptoms begin to manifest in childhood and a broad spectrum of clinical severity has been described, ranging from asymptomatic, recurrent pulmonary infection, severe hemoptysis, to death. Only a few adult cases with this condition, with no or mild symptoms, have been reported. Pulmonary angiography has been typically used for definite diagnosis. However, pulmonary angiography may be replaced with the current developing multidetector CT. This report presents an adult case with mild symptoms, diagnosed by multidetector CT.

  19. A prospective pilot study: Can the biliary tree be visualized in children younger than 3 months on Magnetic Resonance Cholangiopancreatography?

    Energy Technology Data Exchange (ETDEWEB)

    Siles, Pascale [La Timone Children' s Hospital, Department of Radiology, Marseille (France); Aschero, Audrey; Gorincour, Guillaume; Bourliere-Najean, Brigitte; Petit, Philippe [La Timone Children' s Hospital, Department of Pediatric Radiology, Marseille (France); Roquelaure, Bertrand [La Timone Children' s Hospital, Department of Pediatrics, Marseille (France); Delarue, Arnauld [La Timone Children' s Hospital, Department of Pediatric Surgery, Marseille (France)

    2014-09-15

    Magnetic resonance cholangiopancreatography (MRCP) could aid in the diagnosis of biliary atresia, a hepatic pathology with thin, irregular or interrupted biliary ducts. There is little published evidence of MRCP appearances in normal neonates and young infants. To assess the use of MR cholangiopancreatography in visualizing the biliary tree in neonates and infants younger than 3 months with no hepatobiliary disorder, and to assess this visibility in relationship to the child's age, weight, and sedation and fasting states. Between December 2008 and October 2010 our department performed MRI of the brain, orbits and face on 16 full-term neonates and infants. Each child was younger than 3 months (90 days) and without any hepatobiliary disorders. The children were scanned with a respiratory-gated 0.54 x 0.51 x 0.4-mm{sup 3} 3-D MRCP sequence. We used a reading grid to assess subjectively the visibility of the extrahepatic bile ducts along with extrahepatic bile duct confluence. The visibility of the extrahepatic bile duct confluence was assessed against age, weight, and sedation and fasting states. The extrahepatic bile duct confluence was seen in 10 children out of 16 (62.5%). In the neonate sub-group (corrected age younger than 30 days), the MRCP was technically workable and the extrahepatic bile duct confluence was seen in four cases out of eight (50%). This visualization was up to 75% in the subgroup older than 30 days. However, statistically there was no significant difference in visibility of the extrahepatic bile duct confluence in relationship to age, weight or MRCP performance conditions (feeding, fasting or sedation). The complete normal biliary system (extrahepatic bile duct confluence included) is not consistently visualized in infants younger than 3 months old on non-enhanced MRCP. Thus the use of MRCP to exclude a diagnosis of biliary atresia is compromised at optimal time of surgery. (orig.)

  20. [Outcome of children with repaired oesophageal atresia].

    Science.gov (United States)

    Gottrand, F; Sfeir, R; Coopman, S; Deschildre, A; Michaud, L

    2008-12-01

    Although initial prognosis of oesophageal atresia is nowadays excellent with more than 95% of survival, the long-term complications are frequent. A gastro-oesophageal reflux is found in 26 to 75% of the cases, responsible for peptic oesophagitis, anastomotic stenosis and Barrett's oesophagus, risk factor of adenocarcinoma of the oesophagus. A dysphagia is frequently observed on these patients, sometimes several years after the surgery, observed in almost 45% of five-year-old children. Growth retardation is found in nearly a third of these children. Respiratory symptoms are particularly frequent, especially in the first years, associating tracheomalacia facilitating the bronchopulmonary infectious episodes (found in about 30% of 5-year-old children). Esotracheal fistula recurrence is very rare. A deformation of the rib cage is reported in 20%, and a scoliosis in 10% of the patients. However, the quality of life of these patients in the adulthood is good, and influenced by the existence of associated malformations. Even if the current prognosis of oesophageal atresia is good altogether, the frequency of the complications (digestive, respiratory, nutritional, orthopaedic) far from the initial intervention, and the necessity of a surveillance of the secondary oesophageal damages, justifies a systematic and multidisciplinary follow-up until adulthood.

  1. Histomorphological Features of Intestinal Atresia and its Clinical Correlation.

    Science.gov (United States)

    Subbarayan, Devi; Singh, Meeta; Khurana, Nita; Sathish, Agarwal

    2015-11-01

    Intestinal atresia accounts for approximately one third of all cases of neonatal intestinal obstruction. There is controversy regarding pathogenesis of congenital atresia and stenosis of small bowel. Studies regarding clinical manifestations and specific histopathological features of neonatal intestinal atresia are scarce in Indian literature. To understand the histomorphological features and thus suggest pathophysiology of cases with Intestinal Atresia. Out of 147 cases, of intestinal obstruction in newborn studied over a period of 5 years, 39 cases of intestinal atresia were found. Their histomorphological details with clinical manifestations were studied. Type II was the commonest type of atresia. Associated anomalies noted were gastroschisis, volvulus, anal stenosis, microcolon, annular pancreas, meconium cyst and duplication cyst. Histological changes observed were ulceration, flattening, abnormal villous configuration, luminal obliteration, narrowing, haemangiomatous proliferation of blood vessels, fibrosis, haemorrhage, calcification, and mesenchymal condensation around the blood vessels. Gangrene and perforation has also noted in some cases. An intrauterine intestinal ischemia due to vascular pathology followed by resorption of the bowel is the possible explanation for the development of intestinal atresia.

  2. Percutaneous transhepatic biliary endoprostheses

    Energy Technology Data Exchange (ETDEWEB)

    Lammer, J.

    1985-03-01

    Eighty biliary endoprostheses were introduced by the transhepatic route in sixty patients with obstructive jaundice. Complication rate was 21% (no mortality). Average survival time is sixteen weeks (maximum 53 weeks). In most patients, a 12 F teflon endoprosthesis was introduced four to five days after the initial catheter drainage. Patients in poor condition had a primary implant of a 9 F endoprosthesis. A combined transhepatic/transoral implantation was carried out five times. Results have shown that implantation of a prosthesis is as effective as a surgical bypass operation for palliation of obstructive jaundice and that it is better than catheter drainage. In-vitro experiments have indicated that failure of a 12 F prosthesis due to encrustation may be expected in about 23 weeks. This is in line with the survival time of patients with carcinomas.

  3. Dumping syndrome after esophageal atresia repair without antireflux surgery.

    Science.gov (United States)

    Michaud, Laurent; Sfeir, Rony; Couttenier, Frédéric; Turck, Dominique; Gottrand, Frédéric

    2010-04-01

    In childhood, the surgical treatment of gastroesophageal reflux is the main cause of dumping syndrome. We report the cases of 2 children with esophageal atresia who presented with dumping syndrome without any precipitating known factors, such as gastroesophageal reflux surgery or associated microgastria. Our data suggest (1) that dumping syndrome can occur after primary anastomosis of esophageal atresia without antireflux surgery and (2) that dumping syndrome should be considered in every child treated surgically for esophageal atresia presenting with digestive symptoms, malaise, failure to thrive, or refusal to eat. Copyright 2010 Elsevier Inc. All rights reserved.

  4. Glucocorticosteroids for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Prince, M; Christensen, E; Gluud, C

    2005-01-01

    Primary biliary cirrhosis is a chronic progressive cholestatic liver disease of presumed autoimmune etiology, characterised by the destruction of small intrahepatic bile ducts and the eventual development of cirrhosis and liver failure. Its progression may be influenced by immunosuppression...

  5. Spontaneous Biliary Peritonitis in Children

    Directory of Open Access Journals (Sweden)

    Supreethi Kohli

    2013-01-01

    Full Text Available Pediatric Spontaneous Bile duct perforation is a rare clinical condition with only around 150 cases reported worldwide. Early management gives excellent prognosis but the condition often presents a diagnostic dilemma. Hepato-biliary Technetium-99m-iminodiacetic acid scintiscan is the diagnostic investigation of choice but its availability in third world countries is limited. We present two cases of spontaneous biliary peritonitis in children, which were diagnosed without scintiscanning. The first case was a one-and -a half-year-old child, who was diagnosed with biliary peritonitis without pneumoperitoneum by a combination of Ultrasound (USG, Contrast enhanced computed tomography (CECT, and Magnetic Resonance Imaging (MRI. The child underwent USG-guided drainage and subsequent cholecystectomy with hepatico-jejunostomy. The second child also had biliary peritonitis without pneumoperitoneum, which was initially suspected on USG. CECT revealed dilated gall bladder and fluid collection in sub-hepatic space and pelvis. Abdominal paracentesis revealed presence of bile. The child responded to conservative therapy. Both are doing well on two-year follow-up. In a patient with jaundice, biliary tract abnormalities and/or free fluid, either generalized or localized to peri-cholecystic/sub-hepatic space on USG/CT/MRI, in the absence of pneumoperitoneum, suggest a diagnosis of biliary perforation even in the absence of scintiscanning.

  6. The value of biliary endoscopy.

    Science.gov (United States)

    Shore, J M; Berci, G; Morgenstern, L

    1975-04-01

    The value of biliary endoscopy was determined in 100 consecutive patients undergoing choledochotomy. Using a compact, rigid, right-angled choledochoscope with a rod-lens optical system the biliary tract was inspected for residual stomes following conventional exploration. Completion operative cholangiography and postoperative T-tube cholangiography were performed in all patients. Of 52 patients undergoing primary choledocholithotomy, the duct was cleared of all calculi in 51. A small residual stone was found by postoperative cholangiography in one patient. Exploration revealed no calculi in the ducts of the remaining 30 patients. Biliary endoscopy was of benefit to the surgeon in the majority of patients. In 17 patients, calculi missed by standard exploration were detected; in five of these, the calculi could be retrieved only under endoscopic control. In 11 patients, interpretation of operative cholangiograms was aided, while in three the endoscopic findings clarified operative strategy. The use of biliary endoscopy did not increase the postoperative morbidity or mortality rates beyond those oridinarily encountered in choledocholithotomy. Current experience indicates that the new choledhchoscope overcomes limitations of previous endoscopes and should serve as the definitive diagnostic tool for operative biliary endoscopy. The addition of this technique to the armamentarium of the biliary surgeon will play a significant role in overcoming the age-old problem of the retained common duct stone.

  7. Congenital rectovestibular fistula associated with rectal atresia: A rare occurrence

    OpenAIRE

    Rizwan Ahmad Khan; Rajendra Singh Chana

    2013-01-01

    We report a rare variety of anorectal malformation, rectal atresia associated with rectovestibular fistula. The case was successfully treated by posterior sagittal repair. The fistula was mobilized and the continuity of the rectum was established by circumferential anastomosis.

  8. Genetics Home Reference: epidermolysis bullosa with pyloric atresia

    Science.gov (United States)

    ... PA junctional epidermolysis bullosa with pyloric atresia PA-JEB Related Information How are genetic conditions and genes ... HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Ledbetter N, Mefford HC, Smith RJH, Stephens K, ...

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

    OpenAIRE

    Gfrörer, Stefan; Fiegel, Henning; Ramachandran, Priya; Rolle, Udo; Metzger, Roman

    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 proxi...

  10. Effective Treatment of Biliary Cystadenoma

    Science.gov (United States)

    Thomas, K Tyson; Welch, Derek; Trueblood, Andrew; Sulur, Paulgun; Wise, Paul; Gorden, D Lee; Chari, Ravi S.; Wright, J Kelly; Washington, Kay; Pinson, C Wright

    2005-01-01

    Objective: Evaluate experience over 15 years with treatment of this lesion. Summary Background Data: Biliary cystadenoma, a benign hepatic tumor arising from Von Meyenberg complexes, usually present as septated intrahepatic cystic lesions. Methods: Data were collected concurrently and retrospectively on patients identified from hospital medical records reviewed for pertinent International Classification of Diseases, Ninth Revision, Clinical Modification and CPT codes, pathology logs, and from operative case logs. Pathology specimens were rereviewed to confirm the diagnosis of biliary cystadenoma or biliary cystadenocarcinoma by 2 GI pathologists. Results: From October 1989 to April 2004 at our institution, 19 (18F:1M) patients had pathologically confirmed biliary cystadenomas, including one with a biliary cystadenocarcinoma. The mean age was 48 ± 15 years at initial evaluation. Complaints included abdominal pain in 74%, abdominal distension in 26%, and nausea/vomiting in 11%. Only 1 patient presented with an incidental finding. Symptoms had been present for 3 ± 5 years, with 1 to 4 different surgeons and many other physicians involved in the diagnosis or treatment prior to definitive ablation. Eight patients had undergone 20 previous treatments, including multiple percutaneous aspirations in 4 and 11 operative procedures. CT or US was diagnostic in 95%, with internal septations present in the hepatic cysts. Definitive operative intervention consisted of hepatic resection in 12 patients, enucleation in 6 patients, and fenestration and complete fulguration in 1 patient. There were no perioperative deaths. No recurrences were observed after definitive therapy, with follow-up of 4 ± 4 years. Conclusions: Biliary cystadenoma must be recognized and treated differently than most hepatic cysts. There remains a need for education about the imaging findings for biliary cystadenoma to reduce the demonstrated delay in appropriate treatment. Traditional treatment of simple

  11. Hepatic and biliary ascariasis

    Directory of Open Access Journals (Sweden)

    Anup K Das

    2014-01-01

    Full Text Available Ascariasis mainly contributes to the global helminthic burden by infesting a large number of children in the tropical countries. Hepato-biliary ascariasis (HBA is becoming a common entity now than in the past owing to the frequent usage of ultrasonograms and endoscopic diagnostic procedures in the clinical practice. There are a variety of manifestations in HBA and diagnosis depends on a high index of suspicion in endemic areas coupled with subsequent confirmation by sonographic or endoscopic demonstration of the worm. Most of them present with acute abdomen and jaundice. Oriental or recurrent pyogenic cholangiopathy is possibly the result of HBA, commonly encountered in South-East Asian countries. Conservative treatment with anthelminthic agents is used in the majority. Failure to respond to medical therapy usually indicates the need for endoscopic or surgical interventions. Overall, mortality is low and prognosis is good, but many epidemiological and immunological aspects of Ascaris infection are unclear, meaning our understanding the disease and infection still remains incomplete. Therefore, it is difficult to definitely put down a fixed modality of treatment for HBA. This underscores the need for further studies as ascariasis has the potential to adversely affect the national socio-economy by compromising the health of children and adults alike with its sheer number.

  12. Morphological evidence of apoptosis and the prevalence of apoptotic versus mitotic cells in the membrana granulosa of ovarian follicles during spontaneous and induced atresia in ewes.

    Science.gov (United States)

    Jolly, P D; Smith, P R; Heath, D A; Hudson, N L; Lun, S; Still, L A; Watts, C H; McNatty, K P

    1997-04-01

    Apoptosis is a process by which granulosa cells are thought to be deleted during ovarian follicular atresia. The aims of the present studies, using sheep as the experimental model, were to determine 1) whether morphological changes in cells composing the membrana granulosa during the process of atresia conformed with the general criteria of apoptotic cell death as assessed using tissue sections stained with hematoxylin and eosin; 2) whether cells classified as apoptotic on the basis of their morphology contained fragmented DNA using an in situ 3' end-labeling technique; and 3) the degree of apoptosis and mitosis within the granulosa cell populations of large antral follicles (> or = 3 mm in diameter) during both spontaneous and experimentally induced atresia using stereological methods. The results showed that most degenerate granulosa cells in follicles undergoing atresia display the morphological characteristics of apoptosis, suggesting that this is the most common pathway of cell deletion. Typical features were cells containing nuclei with marginated chromatin; cells with a single small densely staining nucleus (pyknotic appearance); cells with multiple smaller, densely staining nuclear fragments; and densely staining membrane-bound bodies (apoptotic bodies) either singly or in clusters. Cells with morphological features more typical of oncosis or necrosis were sometimes observed, but mainly during the later stages of atresia. All cells classified as apoptotic on the basis of morphological criteria contained fragmented DNA as measured by 3' end-labeling. Apoptotic bodies and/or cells were found in all follicles examined, including those classified as healthy. The overall prevalence of apoptotic cells plus apoptotic bodies expressed as a percentage of the total granulosa cell number per follicle varied from 0.02% to 0.20% in healthy follicles, varied from 0.21% to 2.00% in follicles in early (primary) atresia, and was > 2.0% in follicles in later (secondary

  13. Oldest biliary endoprosthesis in situ.

    Science.gov (United States)

    Consolo, Pierluigi; Scalisi, Giuseppe; Crinò, Stefano F; Tortora, Andrea; Giacobbe, Giuseppa; Cintolo, Marcello; Familiari, Luigi; Pallio, Socrate

    2013-07-16

    The advantages of endoscopic retrograde cholangiopancreatography over open surgery have made it the predominant method of treating patients with choledocholithiasis. After sphincterotomy, however, 10%-15% of common bile duct stones cannot be removed with a basket or balloon. The methods for managing "irretrievable stones" include surgery, mechanical lithotripsy, intraductal or extracorporeal shock wave lithotripsy and biliary stenting. The case presented was a referred 82-year-old Caucasian woman with a 7-year-old plastic biliary endoprosthesis in situ. To the best of our knowledge the examined endoprosthesis is the oldest endoprosthesis in situ reported in the literature. Endoscopic biliary endoprosthesis placement remains a simple and safe procedure for patients with stones that are difficult to manage by conventional endoscopic methods and for patients who are unfit for surgery or who are high surgical risks. To date no consensus has been reached regarding how long a biliary prosthesis should remain in situ. Long-term biliary stenting may have a role in selected elderly patients if stones extraction has failed because the procedure may prevent stones impaction and cholangitis.

  14. Oldest biliary endoprosthesis in situ

    Science.gov (United States)

    Consolo, Pierluigi; Scalisi, Giuseppe; Crinò, Stefano F; Tortora, Andrea; Giacobbe, Giuseppa; Cintolo, Marcello; Familiari, Luigi; Pallio, Socrate

    2013-01-01

    The advantages of endoscopic retrograde cholangiopancreatography over open surgery have made it the predominant method of treating patients with choledocholithiasis. After sphincterotomy, however, 10%-15% of common bile duct stones cannot be removed with a basket or balloon. The methods for managing “irretrievable stones” include surgery, mechanical lithotripsy, intraductal or extracorporeal shock wave lithotripsy and biliary stenting. The case presented was a referred 82-year-old Caucasian woman with a 7-year-old plastic biliary endoprosthesis in situ. To the best of our knowledge the examined endoprosthesis is the oldest endoprosthesis in situ reported in the literature. Endoscopic biliary endoprosthesis placement remains a simple and safe procedure for patients with stones that are difficult to manage by conventional endoscopic methods and for patients who are unfit for surgery or who are high surgical risks. To date no consensus has been reached regarding how long a biliary prosthesis should remain in situ. Long-term biliary stenting may have a role in selected elderly patients if stones extraction has failed because the procedure may prevent stones impaction and cholangitis. PMID:23858381

  15. Isolation and culture of biliary epithelial cells.

    OpenAIRE

    Joplin, R

    1994-01-01

    At one time it was thought that biliary epithelial cells simply formed the lining to the tubular conduits which constitute the biliary tract. Development of in vitro systems for culturing biliary epithelial cells has enabled functional studies which increasingly show that this is far from true, and that biliary epithelial cells do have important functional roles. Disruption of these functions may be involved in the generation of pathology. Most functional studies to date have utilised cells i...

  16. Intrahepatic Biliary Cystadenoma With Intracystic Gallstone Formation

    Science.gov (United States)

    Lei, Shize; Domenico, Don R.

    1994-01-01

    Biliary cystadenoma is a rare tumor of the liver. We describe a biliary cystadenoma of the left lobe of the liver with intracystic gallstone formation. This is the first report of stone formation in biliary cystadenoma of the liver. PMID:8155592

  17. Biliary Stricture Following Hepatic Resection

    Directory of Open Access Journals (Sweden)

    Jeffrey B. Matthews

    1991-01-01

    Full Text Available Anatomic distortion and displacement of hilar structures due to liver lobe atrophy and hypertrophy occasionally complicates the surgical approach for biliary stricture repair. Benign biliary stricture following hepatic resection deserves special consideration in this regard because the inevitable hypertrophy of the residual liver causes marked rotation and displacement of the hepatic hilum that if not anticipated may render exposure for repair difficult and dangerous. Three patients with biliary stricture after hepatectomy illustrate the influence of hepatic regeneration on attempts at subsequent stricture repair. Following left hepatectomy, hypertrophy of the right and caudate lobes causes an anteromedial rotation and displacement of the portal structures. After right hepatectomy, the rotation is posterolateral, and a thoracoabdominal approach may be necessary for adequate exposure. Radiographs obtained in the standard anteroposterior projection may be deceptive, and lateral views are recommended to aid in operative planning.

  18. Primary biliary cirrhosis

    Science.gov (United States)

    Kumagi, Teru; Heathcote, E Jenny

    2008-01-01

    Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure. Affected individuals are usually in their fifth to seventh decades of life at time of diagnosis, and 90% are women. Annual incidence is estimated between 0.7 and 49 cases per million-population and prevalence between 6.7 and 940 cases per million-population (depending on age and sex). The majority of patients are asymptomatic at diagnosis, however, some patients present with symptoms of fatigue and/or pruritus. Patients may even present with ascites, hepatic encephalopathy and/or esophageal variceal hemorrhage. PBC is associated with other autoimmune diseases such as Sjogren's syndrome, scleroderma, Raynaud's phenomenon and CREST syndrome and is regarded as an organ specific autoimmune disease. Genetic susceptibility as a predisposing factor for PBC has been suggested. Environmental factors may have potential causative role (infection, chemicals, smoking). Diagnosis is based on a combination of clinical features, abnormal liver biochemical pattern in a cholestatic picture persisting for more than six months and presence of detectable antimitochondrial antibodies (AMA) in serum. All AMA negative patients with cholestatic liver disease should be carefully evaluated with cholangiography and liver biopsy. Ursodeoxycholic acid (UDCA) is the only currently known medication that can slow the disease progression. Patients, particularly those who start UDCA treatment at early-stage disease and who respond in terms of improvement of the liver biochemistry, have a good prognosis. Liver transplantation is usually an option for patients with liver failure and the outcome is 70% survival at 7 years. Recently, animal models have been discovered that may provide a new insight into the pathogenesis of this disease and facilitate appreciation for novel treatment in PBC

  19. Primary biliary cirrhosis

    Directory of Open Access Journals (Sweden)

    Heathcote E Jenny

    2008-01-01

    Full Text Available Abstract Primary biliary cirrhosis (PBC is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure. Affected individuals are usually in their fifth to seventh decades of life at time of diagnosis, and 90% are women. Annual incidence is estimated between 0.7 and 49 cases per million-population and prevalence between 6.7 and 940 cases per million-population (depending on age and sex. The majority of patients are asymptomatic at diagnosis, however, some patients present with symptoms of fatigue and/or pruritus. Patients may even present with ascites, hepatic encephalopathy and/or esophageal variceal hemorrhage. PBC is associated with other autoimmune diseases such as Sjogren's syndrome, scleroderma, Raynaud's phenomenon and CREST syndrome and is regarded as an organ specific autoimmune disease. Genetic susceptibility as a predisposing factor for PBC has been suggested. Environmental factors may have potential causative role (infection, chemicals, smoking. Diagnosis is based on a combination of clinical features, abnormal liver biochemical pattern in a cholestatic picture persisting for more than six months and presence of detectable antimitochondrial antibodies (AMA in serum. All AMA negative patients with cholestatic liver disease should be carefully evaluated with cholangiography and liver biopsy. Ursodeoxycholic acid (UDCA is the only currently known medication that can slow the disease progression. Patients, particularly those who start UDCA treatment at early-stage disease and who respond in terms of improvement of the liver biochemistry, have a good prognosis. Liver transplantation is usually an option for patients with liver failure and the outcome is 70% survival at 7 years. Recently, animal models have been discovered that may provide a new insight into the pathogenesis of this disease and facilitate appreciation for

  20. Transcatheter pulmonary valve perforation and balloon dilatation in neonates with pulmonary atresia and intact ventricular septum

    NARCIS (Netherlands)

    Gerestein, C.G.; Berger, R.M.F.; Dalinghaus, M.; Bogers, A.J.J.C.; Witsenburg, M.

    2003-01-01

    BACKGROUND: Pulmonary atresia and intact ventricular septum is characterised by a great morphological variety. Treatment is not uniform. OBJECTIVE: To evaluate our experience with transcatheter valvotomy and balloon dilatation in neonates with pulmonary atresia and intact ventricular septum. DESIGN:

  1. [Sonography of vascular and biliary epigastric structures].

    Science.gov (United States)

    Triller, J; Haertel, M; Zaunbauer, W; Fuchs, W A

    1981-04-01

    Ultrasonic devices with rapid image build-up and high power of resolution enable detailed assessment of the vascular and biliary structures. Visualisation of the biliary tract of normal lumen in the extrahepatic region enables identification of a biliary obstruction at a time where dilatation of the intrahepatic biliary tract has not yet taken place. The identification of small visceral branches of the aorta abdominalis as well as of portal and systemic veins yields sonographically reliable vascular points of reference which enable, apart from the exact localisation of the biliary tract and of the pancreas, accurate topographic identification of abdominal mass lesions.

  2. Excellent long-term outcome for survivors of apple peel atresia

    NARCIS (Netherlands)

    Festen, S; Brevoord, JCD; Goldhoorn, GA; Festen, C; Hazebroek, FWJ; van Heurn, LWE; de Langen, ZJ; van der Zee, DC; Aronson, DC

    Background: Apple peel atresia is the rarest type of small bowel atresia. Because of its rare occurrence and high mortality rate, little is known about the long-term outcome of these children. Methods: The patient charts, operative reports, and office notes of 15 children with apple peel atresia

  3. Jejuno-ileal atresia: A 2-year preliminary study on presentation and ...

    African Journals Online (AJOL)

    2011-08-11

    Aug 11, 2011 ... Background/Aim: Intestinal atresia is a common cause of neonatal intestinal obstruction. Jejuno-ileum is the commonest site of intestinal atresia. Reports on Jejunoileal atresia in developing countries are still few. The purpose of this study is to determine the presentation and management outcomes of ...

  4. Duodenal Derotation and Extent Tapering Jejunoplasty as Primary Repair for Neonates With High Jejunal Atresia

    Directory of Open Access Journals (Sweden)

    Chih-Cheng Luo

    2010-10-01

    Conclusion: In very proximal high atresia, the extent of tapering is limited by the proximity of the ligament of Treitz. Duodenal derotation provides better access to the high atresia. The results of this limited experience suggest that the DDETJ procedure could provide an alternative therapy in patients with high jejunal atresia.

  5. Management of primary biliary cirrhosis

    NARCIS (Netherlands)

    Jansen, PLM; Farthing, MJG; Misiewicz, JJ

    1997-01-01

    Primary biliary cirrhosis is a slowly progressive cholestatic liver disease with autoimmune features. Antimitochondrial antibodies are detected in the serum of 95 percent of patients. The antibodies are directed against the E2 component of the pyruvate dyhydrogenase complex on the inner

  6. 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.)

  7. Apple-peel atresia presenting as foetal intestinal obstruction

    Directory of Open Access Journals (Sweden)

    Ashok Yadavrao Kshirsagar

    2011-01-01

    Full Text Available Apple-peel atresia or Type 3 jejuno-ileal atresia (JIA is an uncommon cause of foetal intestinal obstruction. Bowel obstruction in the foetus is diagnosed on the prenatal ultrasonography only in 50% cases. We report a case in which foetal intestinal obstruction was diagnosed on prenatal ultrasonography. The child showed signs of intestinal obstruction on day one after birth, for which an exploratory laparotomy was performed. Type 3 JIA was found for which resection of atretic segments with jejuno-ascending colon anastomosis was preformed.

  8. 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.

  9. Atresia pulmonar con septum interventricular intacto

    Directory of Open Access Journals (Sweden)

    Adel Eladio González Morejón

    2012-12-01

    Full Text Available La atresia pulmonar con septum interventricular intacto es una cardiopatía congénita cianótica aparentemente sencilla caracterizada por incorporar diversas variantes morfológicas y lesiones asociadas que conllevan a un diagnóstico y tratamiento más complejo del que inicialmente podría esperarse. La elevada mortalidad antes de los 6 meses de edad obliga a instaurar una conducta agresiva y precoz. Con esta revisión nos hemos propuesto efectuar la evaluación integral de la enfermedad desde sus elementos conceptuales hasta las variantes terapéuticas a adoptar, para lo cual se describen los aspectos más importantes en cuanto a morfología, fisiopatología, diagnóstico y tratamiento. Se discute, asimismo, el papel de la ecocardiografía, de la cirugía y de las técnicas de cardiología intervencionista. La estrategia fundamental del tratamiento es separar las circulaciones sistémica y pulmonar sin provocar disminución del gasto cardíaco ni aumentos en la presión venosa central. Se pretende, además, rescatar al ventrículo derecho para el circuito pulmonar siempre que la circulación coronaria no sea dependiente de aquel. Se resalta, mediante algoritmo, la importancia que reviste la integración de modalidades quirúrgicas, intervencionistas e híbridas en el acometimiento terapéutico de esta cardiopatía.

  10. A Biliary Endoprosthesis Functioning After Six Years

    Science.gov (United States)

    Iswara, K.; Siegel, Jerome H.

    1994-01-01

    The functional lifetime for biliary endoprostheses has typically been 7 months. When combined with sphincterotomy for common bile duct stones, it affords an alternative to surgery in high risk patients. Biliary endoprostheses often require replacement in these patients, though recent reports suggest they are functioning longer. We present an 85-year-old asymptomatic woman with a 6-year-old biliary endoprosthesis, believed to be the longest functioning stent reported in the literature. PMID:18493351

  11. Is biliary lithiasis associated with pancreatographic changes?

    OpenAIRE

    Barthet, M; Affriat, C; Bernard, J. P.; Berthezene, P; Dagorn, J C; Sahel, J

    1995-01-01

    The aetiological role of biliary lithiasis for chronic pancreatitis remains controversial. Previous studies based on pancreatographic studies reported changes in the pancreatic duct system caused by biliary lithiasis. This study analysed retrospectively the endoscopic retrograde cholangiopancreatography of 165 patients presenting with biliary lithiasis and of 53 controls. Among the 165 patients, 113 had choledochal stones (53 with gall bladder stones, 50 had had a cholecystectomy, 10 with a n...

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

    Science.gov (United States)

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

    1988-01-01

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

  13. Cor triloculares biatritum with tricuspid atresia in an african child

    Directory of Open Access Journals (Sweden)

    Aliyu Ibrahim

    2014-01-01

    Full Text Available Cor triloculares biatritum is a rare congenital heart disease characterized by a three-chambered heart consisting of two atria and a single ventricle; however the case of a 5-month-old African boy who in addition to this defect had an associated tricuspid atresia and an atrial septal defect is reported.

  14. Congenital rectovestibular fistula associated with rectal atresia: A rare occurrence

    Directory of Open Access Journals (Sweden)

    Rizwan Ahmad Khan

    2013-01-01

    Full Text Available We report a rare variety of anorectal malformation, rectal atresia associated with rectovestibular fistula. The case was successfully treated by posterior sagittal repair. The fistula was mobilized and the continuity of the rectum was established by circumferential anastomosis.

  15. Pyloric atresia epidermolysis bullosa aplasia cutis syndrome: a case ...

    African Journals Online (AJOL)

    The association between epidermolysis bullosa and congenital pyloric atresia is rare, but is a known distinct clinical entity with autosomal recessive inheritance. We report on a case of a baby girl born by cesarean section at 37 weeks' gestation to a G7 P7 mother and weighed 2.84 kg. At birth, there was denuded skin over ...

  16. Outcomes of surgical management of intestinal atresias | Ezomike ...

    African Journals Online (AJOL)

    Causes of death were: Sepsis with disseminated intravascular coagulation (1), sepsis from anastomotic leakage (1), septic shock (1), anesthesia.related (1), undetermined (1). Two of the mortalities (40%) had re.operation for anastomotic leak. Conclusions: Short.term survival of neonates with intestinal atresias in our unit is ...

  17. QUALITATIVE ABNORMAL FETAL BREATHING MOVEMENTS, ASSOCIATED WITH TRACHEAL ATRESIA

    NARCIS (Netherlands)

    BAARSMA, R; BEKEDAM, DJ; VISSER, GHA

    A case is reported in which qualitatively, grossly abnormal fetal breathing movements turned out to be indicative of complete tracheal atresia. Fetal breathing movements were vigorous and jerky and of large amplitude; similarly abnormal movements were observed after birth. At postmortem tracheal

  18. Esophageal atresia: long-term morbidities in adolescence and adulthood.

    NARCIS (Netherlands)

    Ijsselstijn, H.; Beelen, N.W. van; Wijnen, R.M.H.

    2013-01-01

    Survival rates in esophageal atresia (EA) patients have reached 90%. In long-term follow-up studies the focus has shifted from purely surgical or gastrointestinal evaluation to a multidisciplinary approach. We evaluated the long-term morbidity in adolescent and adult EA patients and discussed mainly

  19. Combined oesophageal atresia with upper pouch fistula and ...

    African Journals Online (AJOL)

    Upper pouch tracheoesophageal fistula occurs is less than 1% of all oesophageal atresia variants. Meconium peritonitis is a rare neonatal condition with an incidence of 1:30 000 live births. In this case report, we describe the presentation, clinical findings and management of a patient diagnosed with an oesophageal ...

  20. 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.

  1. [Biliary complications after liver transplant].

    Science.gov (United States)

    Lladó, Laura; Fabregat, Joan; Ramos, Emilio; Baliellas, Carme; Torras, Jaume; Rafecas, Antoni

    2012-01-01

    There have been biliary complications since the beginning of liver transplants, and is a topic of great interest due to its high incidence, as well as their influence on morbidity and mortality. The biliary fistula is currently uncommon and its management is straightforward. Anastomotic stenosis continues to have an incidence of 10-15%. Although the current treatment of choice is endoscopic retrograde cholangiopancreatography (ERCP), surgical treatment (hepatico-jejunostomy) continues to have an important role. Non-anastomotic stenosis has an incidence of 5-10%, and is associated with ischaemic or immunological factors, and usually involves a re-transplant. Choledocholithiasis has an incidence of 5-10%, with the treatment of choice being ERCP. However the treatment of biliary complications should be individualised. We must take into account, liver function, the general health status of the patient, and the availability and experience of the team in the different therapeutic options. Copyright © 2011 AEC. Published by Elsevier Espana. All rights reserved.

  2. 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)

  3. Atresia pulmonar con septum interventricular intacto Pulmonary atresia with intact ventricular septum, a thematic review

    Directory of Open Access Journals (Sweden)

    Adel Eladio González Morejón

    2012-12-01

    Full Text Available La atresia pulmonar con septum interventricular intacto es una cardiopatía congénita cianótica aparentemente sencilla caracterizada por incorporar diversas variantes morfológicas y lesiones asociadas que conllevan a un diagnóstico y tratamiento más complejo del que inicialmente podría esperarse. La elevada mortalidad antes de los 6 meses de edad obliga a instaurar una conducta agresiva y precoz. Con esta revisión nos hemos propuesto efectuar la evaluación integral de la enfermedad desde sus elementos conceptuales hasta las variantes terapéuticas a adoptar, para lo cual se describen los aspectos más importantes en cuanto a morfología, fisiopatología, diagnóstico y tratamiento. Se discute, asimismo, el papel de la ecocardiografía, de la cirugía y de las técnicas de cardiología intervencionista. La estrategia fundamental del tratamiento es separar las circulaciones sistémica y pulmonar sin provocar disminución del gasto cardíaco ni aumentos en la presión venosa central. Se pretende, además, rescatar al ventrículo derecho para el circuito pulmonar siempre que la circulación coronaria no sea dependiente de aquel. Se resalta, mediante algoritmo, la importancia que reviste la integración de modalidades quirúrgicas, intervencionistas e híbridas en el acometimiento terapéutico de esta cardiopatía.Pulmonary atresia with intact ventricular septum is an apparently simple cyanotic congenital cardiopathy, characterized by several morphological variants and associated lesions leading to a complex diagnosis and treatment. The high mortality rate recorded in under 6 months-old infants demands an early aggressive management to face this situation. This review was intended to make a comprehensive assessment of the disease from its basic conceptual elements to the therapeutic variants to be adopted. To this end, the most important elements of morphology, physiopathology, diagnosis and treatment were addressed, in addition to discussing

  4. A selective cyclooxygenase-2 inhibitor (Etodolac) prevents spontaneous biliary tumorigenesis in a hamster bilioenterostomy model.

    Science.gov (United States)

    Kitasato, Amane; Kuroki, Tamotsu; Adachi, Tomohiko; Ono, Shinichiro; Tanaka, Takayuki; Tsuneoka, Noritsugu; Hirabaru, Masataka; Takatsuki, Mitsuhisa; Eguchi, Susumu

    2014-01-01

    Secondary biliary carcinomas are associated with persistent reflux cholangitis after bilioenterostomy. Cyclooxygenase-2 (COX-2) has been a target for cancer prevention. The aim of this study was to evaluate the chemopreventive efficacy of long-term treatment with a selective COX-2 inhibitor medication during the natural course after bilioenterostomy without chemical induction. Syrian golden hamsters which underwent choledochojejunostomy were randomly divided into two groups: the control group (n = 31), which was fed a normal diet, and the etodolac group (n = 33), which was fed 0.01% etodolac (a selective COX-2 inhibitor) mixed in the meal. The hamsters were killed at the postoperative weeks 20-39, 40-59, 60-79, or 80-100. Biliary neoplasms, cholangitis, proliferating cell nuclear antigen labeling index (PCNA-LI) of the biliary epithelium, and prostaglandin E2 (PGE2) production were evaluated. The occurrence rates of biliary neoplasm were 43.8 and 15.2% in the control and etodolac groups, respectively (p < 0.05). The incidence of biliary neoplasm increased as time progressed in the control group, whereas it remained at a low level throughout the experimental period in the etodolac group. PGE2 products tended to be lower in the etodolac group, and PCNA-LI was significantly lower in the etodolac group (p < 0.01). These results suggest that the medication etodolac suppresses cell proliferation of the biliary epithelium, thereby preventing biliary carcinogenesis. Etodolac is expected to prevent secondary biliary carcinogenesis caused by persistent reflux cholangitis after bilioenterostomy. © 2014 S. Karger AG, Basel.

  5. Giant biliary cystadenoma: a case report.

    Science.gov (United States)

    Cecka, F; Ferko, A; Subrt, Z; Jon, B

    2011-01-01

    Biliary cystadenoma is a very rare benign cystic tumour of the liver. Fewer than 150 cases have been described in the literature so far. The authors present a case of a 29-year-old female with a giant intrahepatic biliary cystadenoma who presented with abdominal pain and obstructive jaundice. The patient was treated with left hepatectomy and now 12 months after the surgery she is in good condition with no signs of recurrence of the disease. Clinical presentation of biliary cystadenoma is not specific. Diagnosis is based on imaging methods, mainly ultrasound and CT scan. However, it is often misdiagnosed. For treatment, radical resection is advocated because a biliary cystadenoma is considered to be a premalignant lesion. The prognosis of biliary cystadenoma after complete resection is excellent. Nevertheless, there is a risk of recurrence or malignant transformation after incomplete resection.

  6. Role of intraluminal bowel echogenicity on prenatal ultrasounds to determine the anatomical level of intestinal atresia.

    Science.gov (United States)

    Goruppi, Ilaria; Arévalo, Silvia; Gander, Romy; Molino, José-Andres; Oria, Marc; Carreras, Elena; Peiro, José-Luis

    2017-01-01

    To evaluate the correlation between different degrees of bowel intraluminal echogenicity showed by prenatal ultrasounds and the anatomic level of intestinal atresia. We report three cases of intestinal atresia at different intestinal levels verified during the neonatal surgery with specific ultrasonographic prenatal features. Intensity of sonolucency was analyzed using the image-processing program ImageJ for quantitative measurements based on the gray-scale intensity values. A total of three cases are reported, a jejunal, an ileal and a colonic atresia. All cases showed intestinal dilatation. Both, jejunal and ileal atresia, showed two degrees of hypoechoic intestinal content, while colonic atresia showed hyperechogenic content dilated loop at prenatal ultrasound scan. We propose the use of prenatal ultrasounds echogenicity of intestinal dilated loop fluid content to help in determining the level of obstruction in bowel atresia. These are initial results, to be confirmed by a multicentric research with more cases.

  7. Portal Biliopathy Causing Recurrent Biliary Obstruction and Hemobilia.

    Science.gov (United States)

    Schlansky, Barry; Kaufman, John A; Bakis, Gene; Naugler, Willscott E

    2013-10-01

    A 63-year-old man with extrahepatic portal vein thrombosis presented with biliary obstruction and hemobilia after a liver biopsy. Balloon sweep of the common bile duct removed clotted blood, and cholangiogram showed a common bile duct narrowing, treated with biliary stenting. A percutaneous biliary catheter was later required for recurrent biliary obstruction and hemobilia, and repeat cholangiogram confirmed portal biliopathy-a large peri-biliary varix was compressing the common bile duct, causing biliary obstruction and intermittent portal hypertensive hemobilia. A transjugular intrahepatic portosystemic shunt was inserted, followed by embolization of the peri-biliary varix. Delayed diagnosis of portal biliopathy may lead to significant patient morbidity.

  8. 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

  9. Biliary Ascariasis: A difficult extraction

    Directory of Open Access Journals (Sweden)

    Sanjeev Kumar Thakur

    2015-01-01

    Full Text Available Hepatobiliary ascariasis (HBA is a common complication of Ascaris infestation. It is reported mostly from developing countries. It is a common cause of biliary colic and cholangitis in some parts of India. It is also proposed as an etiology of a subset of patients with recurrent pyogenic cholangitis (RPC. Conservative management, endoscopic removal of the worm wherever needed and deworming is the accepted treatment approach. We herewith present a unique challenge that we encountered during worm removal. The patient was a 35-year-old female with 3 days history of epigastric pain, fever with rigors and vomiting. Her biochemical evaluation showed mild neutrophilic leukocytosis, mild elevation of aminotransferases and alkaline phosphatase. Ultrasound abdomen showed a tubular filling defect in the common bile duct extending in to the left hepatic duct. On endoscopic retrograde cholangiopancreatography (ERCP, the extraction was difficult because of left ductal stricture and a knot at the end of the worm. Such a worm conformation is rarely reported in the literature. In addition to presenting a challenge during removal it may act as a nidus for further infections and damage to the biliary tree particularly if the worm is dead or decaying. RPC is a disease with high morbidity and mortality. HBA is argued as an inciting event in significant number of cases. Recognition of such worm conformations emphasizes the need of meticulous ductal clearance at the time of ERCP, subsequent deworming and improved sanitation to protect such case from subsequent dreaded complications.

  10. The Management of Cyanotic Spells in Children with Oesophageal Atresia

    OpenAIRE

    Mathieu Bergeron; Cohen, Aliza P.; Cotton, Robin T.

    2017-01-01

    Cyanotic spells, also known as blue spells, dying spells, or apparent life-threatening events, refer to a bluish tone visible in the mucosal membranes and skin caused by an oxygen decrease in the peripheral circulation. Although this decrease may be transient and benign, it may also be indicative of a severe underlying problem that requires immediate intervention. Children with oesophageal atresia (OA) are at risk for a number of coexisting conditions that may trigger cyanotic spells. This cu...

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

    OpenAIRE

    Fathima Niloofar; Kumaran Parvathy; Varma, Balagopal R.; Xavier Arun Mamachan

    2015-01-01

    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 m...

  12. Contemporary Hearing Rehabilitation Options in Patients with Aural Atresia

    Directory of Open Access Journals (Sweden)

    Jacky F. W. Lo

    2014-01-01

    Full Text Available Congenital aural atresia is the failure of development of the external auditory canal. It usually occurs in conjunction with microtia, which is the malformation of the auricle due to a failure of development of the external ear. Aural atresia, with or without microtia, may significantly affect the hearing and social life of the patients. It is important for every medical practitioner to be aware of the possible treatment options for hearing rehabilitation in this group of patients. In the era of modern technology, new choices, including Bone-Anchored Hearing Aid (BAHA (Cochlear Ltd. and Oticon Medical, Vibrant Soundbridge (VSB (MED-EL, Innsbruck, Austria, and Bonebridge system (BB (MED-EL, Innsbruck, Austria, provide high-end alternatives to traditional Bone Conduction Hearing Aid and Auditory Canal Reconstruction. All these options have advantages and disadvantages, and they are appropriate for different patients and/or at different ages. This paper aims to provide an overview of the management of hearing rehabilitation in congenital aural atresia patients and a discussion of each treatment option.

  13. Contemporary Hearing Rehabilitation Options in Patients with Aural Atresia

    Science.gov (United States)

    Lo, Jacky F. W.; Tsang, Willis S. S.; Yu, Joannie Y. K.; Ho, Osan Y. M.; Ku, Peter K. M.; Tong, Michael C. F.

    2014-01-01

    Congenital aural atresia is the failure of development of the external auditory canal. It usually occurs in conjunction with microtia, which is the malformation of the auricle due to a failure of development of the external ear. Aural atresia, with or without microtia, may significantly affect the hearing and social life of the patients. It is important for every medical practitioner to be aware of the possible treatment options for hearing rehabilitation in this group of patients. In the era of modern technology, new choices, including Bone-Anchored Hearing Aid (BAHA) (Cochlear Ltd. and Oticon Medical), Vibrant Soundbridge (VSB) (MED-EL, Innsbruck, Austria), and Bonebridge system (BB) (MED-EL, Innsbruck, Austria), provide high-end alternatives to traditional Bone Conduction Hearing Aid and Auditory Canal Reconstruction. All these options have advantages and disadvantages, and they are appropriate for different patients and/or at different ages. This paper aims to provide an overview of the management of hearing rehabilitation in congenital aural atresia patients and a discussion of each treatment option. PMID:24883324

  14. Photodynamic therapy for pancreatic and biliary tract carcinoma

    Science.gov (United States)

    Pereira, Stephen P.

    2009-02-01

    Patients with non-resectable pancreatic and biliary tract cancer (cholangiocarcinoma and gallbladder cancer) have a dismal outlook with conventional palliative therapies, with a median survival of 3-9 months and a 5 year survival of less than 3%. Surgery is the only curative treatment but is appropriate in less than 20% of cases, and even then is associated with a 5-year survival of less than 30%. Although most applications of photodynamic therapy (PDT) in gastroenterology have been on lesions of the luminal gut, there is increasing experimental and clinical evidence for its efficacy in cancers of the pancreas and biliary tract. Our group has carried out the only clinical study of PDT in pancreatic carcinoma reported to date, and showed that PDT is feasible for local debulking of pancreatic cancer. PDT has also been used with palliative intent in patients with unresectable cholangiocarcinoma, with patients treated with stenting plus PDT reporting improvements in cholestasis, quality of life and survival compared with historical or randomized controls treated with stenting alone. Further controlled studies are needed to establish the influence of PDT and chemotherapy on the survival and quality of life of patients with pancreatic and biliary tract carcinoma.

  15. Management of biliary perforation in children

    Directory of Open Access Journals (Sweden)

    Mirza Bilal

    2010-01-01

    Full Text Available Background: To study the aetiology, management and outcome of biliary perforations in paediatric age group. Patients and Methods: In a retrospective study, the records of patients presented with biliary peritonitis due to biliary perforations, managed from March 2006 to July 2009, are reviewed. Results: Eight male patients with biliary peritonitis due to biliary perforation were managed. These patients were divided in two groups, A and B. Group A, (n = 3 patients, had common bile duct (CBD perforation, and Group B (n=5 patients had gallbladder perforation. The presenting features were abdominal pain, fever, abdominal distension, vomiting, constipation, jaundice and signs of peritonism. The management of CBD perforations in Group A was by draining the site of perforation and biliary diversion (tube cholecystostomy. In Group B, the gallbladder perforations were managed by tube cholecystostomy in four patients and cholecystectomy in one patient, however, one patient had to be re-explored and cholecystectomy performed due to complete necrosis of gall bladder. There was no mortality in our series. All patients were asymptomatic on regular follow-up. Conclusion: Early optimal management of biliary perforations remarkably improved the very high mortality and morbidity that characterised this condition in the past.

  16. Immunological Blood Parameters in Infected and Noninfected Biliary Peritonitis

    OpenAIRE

    Bilookiy, O. V.; Rohovyy, Yu. Ye.; Bilookiy, V. V.

    2015-01-01

    This work deals with the study of immunological blood parameters in infected and noninfected biliary peritonitis. Materials and methods. 55 patients with infected and noninfected biliary peritonitis were examined. There were 21 men and 34 women at the age of 28-74 years. 14 patients suffered from noninfected biliary peritonitis, 41 patients suffered from infected biliary peritonitis. The control group included 12 practically healthy persons. Results. The development of noninfected biliary per...

  17. Apotopes and the Biliary Specificity of Primary Biliary Cirrhosis

    Science.gov (United States)

    Lleo, Ana; Selmi, Carlo; Invernizzi, Pietro; Podda, Mauro; Coppel, Ross L.; Mackay, Ian R.; Gores, Gregory J.; Ansari, Aftab A.; Van de Water, Judy; Gershwin, M. Eric

    2009-01-01

    Primary biliary cirrhosis (PBC) is characterized by antimitochondrial antibodies (AMA), directed to the E2 component of the pyruvate dehydrogenase complex (PDC-E2). Notwithstanding the presence of mitochondria in virtually all nucleated cells, the destruction in PBC is limited to small intrahepatic bile ducts. The reasons for this tissue specificity remain unknown, although biliary epithelial cells (BEC) uniquely preserve the PDC-E2 epitope following apoptosis. Notably, PBC recurs in an allogeneic transplanted liver, suggesting generic rather than host-PBC-specific susceptibility of BEC. We used cultured human intrahepatic BEC (HIBEC) and other well-characterized cell lines, including, HeLa, CaCo-2 cells, and non transformed human keratinocytes and bronchial epithelial cells (BrEpC), to determine the integrity and specific localization of PDC-E2 during induced apoptosis. All cell lines, both before and after apoptosis, were tested with sera from patients with PBC (n=30), other autoimmune liver and rheumatic diseases (n=20), and healthy individuals (n=20), a mouse monoclonal antibody against PDC-E2, and AMA with an IgA isotype. PDC-E2 was found to localize unmodified within apoptotic blebs of HIBEC, but not within blebs of various other cell lineages studied. The fact that AMA- containing sera reacted with PDC-E2 on apoptotic BEC without a requirement for permeabilization suggests that the autoantigen is accessible to the immune system during apoptosis. In conclusion, our data indicate that the tissue (cholangiocyte) specificity of the autoimmune injury in PBC is a consequence of the unique characteristics of HIBEC during apoptosis and can be explained by exposure to the immune system of intact immunoreactive PDC-E2 within apoptotic blebs. PMID:19185000

  18. Ceftriaxone-associated nephrolithiasis and biliary pseudolithiasis.

    Science.gov (United States)

    de Moor, R A; Egberts, A C; Schröder, C H

    1999-12-01

    Biliary pseudolithiasis has been reported in patients who received ceftriaxone therapy. In addition to biliary sludge formation occasional reports of ceftriaxone-induced nephrolithiasis have been published. In general, these adverse effects will develop after seven to ten days of treatment. We report on a seven-year-old boy with ceftriaxone-associated biliary pseudolithiasis and nephrolithiasis four days after initiation of treatment. Patients receiving a high dose of ceftriaxone and developing colicky abdominal pain should be considered for ultrasound and a change in antibiotic therapy if appropriate.

  19. Endoscopic Stenting and Clipping for Anastomotic Stricture and Persistent Tracheoesophageal Fistula after Surgical Repair of Esophageal Atresia in an Infant

    Directory of Open Access Journals (Sweden)

    Mohammed Amine Benatta

    2014-01-01

    Full Text Available Anastomotic stricture (AS and recurrent tracheoesophageal fistula (TEF are two complications of surgical repair of esophageal atresia (EA. Therapeutic endoscopic modalities include stenting, tissue glue, and clipping for TEF and endoscopic balloon dilation bougienage and stenting for esophageal strictures. We report herein a two-month infant with both EA and TEF who benefited from a surgical repair for EA, at the third day of life. Two months later he experienced deglutition disorders and recurrent chest infections. The esophagogram showed an AS and a TEF confirmed with blue methylene test at bronchoscopy. A partially covered self-expanding metal type biliary was endoscopically placed. Ten weeks later the stent was removed. This allows for easy passage of the endoscope in the gastric cavity but a persistent recurrent fistula was noted. Instillation of contrast demonstrated a fully dilated stricture but with a persistent TEF. Then we proceeded to placement of several endoclips at the fistula site. The esophagogram confirmed the TEF was obliterated. At 12 months of follow-up, he was asymptomatic. Stenting was effective to alleviate the stricture but failed to treat the TEF. At our knowledge this is the second case of successful use of endoclips placement to obliterate recurrent TEF after surgical repair of EA in children.

  20. 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

  1. Radiofrequency-assisted "reconstruction" of the right ventricular outflow tract in muscular pulmonary atresia with ventricular septal defect.

    OpenAIRE

    Hausdorf, G; Schulze-Neick, I.; Lange, P. E.

    1993-01-01

    A case of pulmonary atresia with ventricular septal defect is reported in which a communication was established between the right ventricle and the hypoplastic pulmonary artery by intervention, despite muscular atresia of the right ventricular outflow tract. The atresia was perforated with a special designed radiofrequency catheter (Osypka). After the creation of a canal within the muscular atresia, balloon dilatation (diameters 2, 3.5, and 7.2 mm) was performed. Arterial oxygen saturation in...

  2. Primary Biliary Cirrhosis: Family Stories

    Directory of Open Access Journals (Sweden)

    Daniel Smyk

    2011-01-01

    Full Text Available Primary biliary cirrhosis (PBC is a chronic immune-mediated cholestatic liver disease of unknown aetiology which affects mostly women in middle age. Familial PBC is when PBC affects more than one member of the same family, and data suggest that first-degree relatives of PBC patients have an increased risk of developing the disease. Most often, these familial clusters involve mother-daughter pairs, which is consistent with the female preponderance of the disease. These clusters provide evidence towards a genetic basis underlying PBC. However, clusters of nonrelated individuals have also been reported, giving strength to an environmental component. Twin studies have demonstrated a high concordance for PBC in monozygotic twins and a low concordance among dizygotic twins. In conclusion, studies of PBC in families clearly demonstrate that genetic, epigenetic, and environmental factors play a role in the development of the disease.

  3. Etiopathogenesis of primary biliary cirrhosis

    Science.gov (United States)

    Lleo, Ana; Invernizzi, Pietro; Mackay, Ian R; Prince, Harry; Zhong, Ren-Qian; Gershwin, M Eric

    2008-01-01

    Primary biliary cirrhosis (PBC) is an autoimmune disease of the liver characterized by progressive bile duct destruction eventually leading to cirrhosis and liver failure. The serological hallmark of the disease is the presence of circulating antimitochondrial antibodies (AMA). These reflect the presence of autoreactive T and B cells to the culprit antigens, the E2 subunits of mitochondrial 2-oxo-acid dehydrogenase enzymes, chiefly pyruvate dehydrogenase (PDC-E2). The disease results from a combination of genetic and environmental risk factors. Genetic predisposition is indicated by the higher familial incidence of the disease particularly among siblings and the high concordance rate among monozygotic twins. Environmental triggering events appear crucial to disrupt a pre-existing unstable immune tolerance of genetic origin allowing, after a long latency, the emergence of clinical disease. Initiating mimotopes of the vulnerable epitope of the PDC-E2 autoantigen can be derived from microbes that utilize the PDC enzyme or, alternatively, environmental xenobiotics/chemical compounds that modify the structure of native proteins to make them immunogenic. A further alternative as a source of antigen is PDC-E2 derived from apoptotic cells. In the effector phase the biliary ductular cell, by reason of its proclivity to express the antigen PDC-E2 in the course of apoptosis, undergoes a multilineage immune attack comprised of CD4+ and CD8+ T cells and antibody. In this article, we critically review the available evidence on etiopathogenesis of PBC and present interpretations of complex data, new developments and theories, and nominate directions for future research. PMID:18528930

  4. Liver transplantation for symptomatic centrohepatic biliary cystadenoma.

    Science.gov (United States)

    Romagnoli, Renato; Patrono, Damiano; Paraluppi, Gianluca; David, Ezio; Tandoi, Francesco; Strignano, Paolo; Lupo, Francesco; Salizzoni, Mauro

    2011-05-01

    Biliary cystadenoma is a rare benign cystic tumor of the liver. The mainstay of treatment is complete resection, either by enucleation or by formal hepatectomy, since incomplete removal entails not only constant recurrence but also the risk of malignant transformation to cystadenocarcinoma. A case of symptomatic centrohepatic biliary cystadenoma involving the main vasculobiliary structures of the liver is reported. After an unsuccessful attempt at resection resulting in an intrahepatic biliary injury, relief of jaundice and radical excision were achieved by total hepatectomy and liver transplantation. The patient is now alive and well 4 years after transplant, disease-free, with normal liver and renal function while receiving everolimus monotherapy. This is the first report of liver transplantation performed for the treatment of this tumor. With the case on the background, diagnostic aspects and available therapeutic strategies for biliary cystadenoma are reviewed and discussed. Copyright © 2011. Published by Elsevier Masson SAS.

  5. Bisphosphonates for osteoporosis in primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Rudic, Jelena; Giljaca, Vanja; Krstic, Miodrag N

    2011-01-01

    Bisphosphonates are widely used for treatment of postmenopausal osteoporosis. Patients with primary biliary cirrhosis often have osteoporosis - either postmenopausal or secondary to the liver disease. No systematic review or meta-analysis has assessed the effects of bisphosphonates for osteoporosis...

  6. Biliary Lithiasis with Choledocolithiasis in Children

    National Research Council Canada - National Science Library

    Bălănescu, R N; Bălănescu, L; Drăgan, G; Moga, A; Caragaţă, R

    2015-01-01

    Although biliary lithiasis has been considered a less common pathology in the pediatric population than in adults, in recent years, it has increasingly been diagnosed in children, with a prevalence of between 0.13 to 0.22...

  7. Cephalosporin-induced biliary sludge in children

    National Research Council Canada - National Science Library

    A.E. Abaturov; V.L. Babich; A.E. Lykova; A.E. Kozachkovsky; N.M. Lybenko

    2017-01-01

    ...). The purpose of the study was to establish the peculiarities of the formation of biliary sludge during the application of the third generation cephalosporins in the treatment of acute respiratory infections in children...

  8. Percutaneous transhepatic biliary drainage using endoprosthesis

    Energy Technology Data Exchange (ETDEWEB)

    Golder, W.; Rupp, N.

    1986-06-01

    Even in the presence of intrahepatic metastases or previous failure of biliary enteric anastomosis, patients suffering from malignant obstructive jaundice can bve given relief by biliary endoprosthesis. Mean survival time is four months. Other than with a catheter drain, spetic cholangitis is rarely observed after the insertion of an endoprosthesis. The intervention's risk will hardly be augmented by drainage of both lobes as often necessary.

  9. Duodenal atresia with 'apple-peel configuration' of the ileum and ...

    African Journals Online (AJOL)

    According to the current understanding, duodenal atresia is considered to be a primary malformation resulting from the errors in recanalisation in early gestation. We report a rare case of duodenal atresia with apple-peel configuration of remaining small bowel with absent superior mesenteric artery in a preterm child, which ...

  10. Different varieties of colonic atresia in a series of 13 patients: a ...

    African Journals Online (AJOL)

    Purpose The aim of this study was to review our experience in the management and outcome of colonic atresia, either isolated or associated with anorectal anomalies. Methods A total of 13 neonates with colonic atresia were operated upon in the Pediatric Surgery Unit of Zagazig University Hospital between August 2004 ...

  11. Jejunal atresia related to the use of methylene blue in genetic amniocentesis in twins

    NARCIS (Netherlands)

    van der Pol, J. G.; Wolf, H.; Boer, K.; Treffers, P. E.; Leschot, N. J.; Hey, H. A.; Vos, A.

    1992-01-01

    OBJECTIVE: To calculate the incidence of jejunal atresia in newborns in The Netherlands. To study the relation between the occurrence of jejunal atresia and genetic amniocentesis to determine a possible iatrogenic cause for the unexpected high incidence of this anomaly in twins. DESIGN:

  12. Different varieties of colonic atresia in a series of 13 patients: a ...

    African Journals Online (AJOL)

    Purpose The aim of this study was to review our experience in the management and outcome of colonic atresia, either isolated or associated with anorectal anomalies. Methods A total of 13 neonates with colonic atresia were operated upon in the Pediatric Surgery Unit of Zagazig. University Hospital between August 2004 ...

  13. 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

  14. Prevention of vitamin K deficiency bleeding in breastfed infants: lessons from the Dutch and Danish biliary atresia registries.

    NARCIS (Netherlands)

    Hasselt, PM van; Koning, TJ de; Kvist, N.; Vries, E. De; Lundin, C.R.; Berger, R.; Kimpen, J.L.; Houwen, R.H.; Jorgensen, M.H.; Verkade, H.J.; Aronsen, D.C.; Kindermann, A.; Kneepkens, C.M.; Heurn, L.W.E. van; Neucker, A.M.; Langen, Z.J. de; Peeters, P.M.; Madern, G.C.; Escher, J.H.; Zee, D.C. van der; Rieu, P.N.M.A.; Tolboom, J.J.M.

    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

  15. 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 ...... prophylaxis at birth. Our data underline the fact that event analysis in specific populations at risk can help to evaluate and improve nationwide prophylactic regimens Udgivelsesdato: 2008/4...

  16. Histologic features of the portal plate in extrahepatic biliary atresia and their impact on prognosis--a Danish study

    DEFF Research Database (Denmark)

    Mirza, Qazaz; Kvist, Nina; Petersen, Bodil Laub

    2009-01-01

    and a failure group and compared the histologic features of the portal plates in the 2 groups. Afterward, the portal plate histology from EHBA was compared with the porta hepatis area from patients dead from other causes. RESULTS: A significant difference between the success and the failure group was found......BACKGROUND/PURPOSE: The aims of this study are as follows: METHOD: From 1979 to 2003, 57 children have been operated by the Kasai procedure. Only 40 of these have had their portal plate removed for histologic examination. We divided the patients according to clinical outcome into a successful...

  17. [Internal biliary fistula of lithiasic origin].

    Science.gov (United States)

    Oancea, T; Cojocea, V; Timaru, I; Bumbac, A; Andrei, V

    1981-01-01

    The authors present 17 cases of patients with internal biliary fistulae of lithiasic origin. Nine of these were of the cholecystoduodenal type, 2 of the cholecysto-gastric type, 2 had developed between the cholecyst and the colon, and 4 were of the bilio-biliary type, between the cholecyst and the choledochus. These cases represented 1.1% of the 1,500 interventions carried out on the extra-hepatic biliary pathways over a period of 10 years, between 1971 and 1980, and 13% of the total number of interventions performed for biliary lithiasis. The advanced age of the patients, the background disease and the complications that accompanied it, as well as the associated deficiencies have given rise to diagnostic and therapeutic difficulties. A correct diagnosis before surgery was made in only two of the patients. The postoperative evolution was good in 13 of the cases (representing 76% of the total), and this confirmed the correct surgical attitude which aimed at restoration of the physiological conditions and a normal digestive and biliary transit. In cases with biliary ileus the first therapeutic measure was to secure intestinal transit.

  18. Biliary cysts: Etiology, diagnosis and management

    Science.gov (United States)

    Jabłońska, Beata

    2012-01-01

    Biliary cysts (BC) are rare dilatations of different parts of a biliary tract. They account for approximately 1% of all benign biliary diseases. BC occur the most frequently in Asian and female populations. They are an important problem for pediatricians, gastroenterologists, radiologists and surgeons. Clinical presentation and management depend on the BC type. Cholangiocarcinoma is the most serious and dangerous BC complication. The other complications associated with BC involve cholelithiasis and hepatolithiasis, cholangitis, acute and chronic pancreatitis, portal hypertension, liver fibrosis and secondary liver cirrhosis and spontaneous cyst perforation. Different BC classifications have been described in the literature. Todani classification dividing BC into five types is the most useful in clinical practice. The early diagnosis and proper treatment are very important, because BC are associated with a risk of carcinogenesis. A malignancy risk increases with the age. Radiological investigations (ultrasonography, computed tomography, endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography) play an important role in BC diagnostics. Currently, prenatal diagnosis using ultrasonography is possible. It allows to differentiate biliary disorders in fetals and to perform the early surgical treatment that improves results. In most patients, total cyst excision with Roux-Y hepaticojejunostomy is the treatment of choice. Surgical treatment of BC is associated with high success rate and low morbidity and mortality. The early treatment is associated with a lower number of complications. Patients following BC surgery require permanent and careful postoperative observation using laboratory and imaging investigations because of possibility of biliary anastomosis stricture and biliary cancer in tissue remnant. PMID:23002354

  19. [Application of (125)I seeds combined with biliary stent implantation in the treatment of malignant obstructive jaundice].

    Science.gov (United States)

    Wang, T; Liu, S; Zheng, Y B; Song, X P; Jiang, W J; Sun, B L; Wang, L G

    2016-03-23

    To study the feasibility and therapeutic effect of the application of (125)I seeds combined with biliary stent implantation on the treatment of malignant obstructive jaundice. Fifty patients with malignant obstructive jaundice treated from September 2010 to February 2013 in Yantai Yuhuangding Hospital were included in this study. Among them, 24 patients received biliary stent implantation combined with (125)I seeds intraluminal brachytherapy as experimental group, and 26 were treated by biliary stent implantation as control group.The total bilirubin, direct bilirubin and tumor markers (CA-199, CA-242, CEA) before and after surgery, the biliary stent patency status was assessed, and the survival time was evaluated. The 24 patients in experimental group were implanted with 30 (125)I seeds successfully in a total of 450 seeds. Jaundice was improved greatly in both groups. The CA-199 and CA-242 after treatment in the experimental group were significantly decreased than that before treatment (P=0.003 and P=0.004). CEA was also decreased, but showed no statistical significance (P>0.05). There were no significant improvement comparing the CA-199, CA-242 and CEA before and 2 months after surgery in the control group (P>0.05). The rate of biliary stent patency was 83.3% (20/24) in the experimental group and 57.7% (15/26) in the control group (P=0.048). The mean biliary stent patency time in the experimental group was 9.84 months (range 1-15.5 months). The mean biliary stent patency time in the control group was 5.57 months (range 0.8-9 months). There was a significant difference between the two groups (P=0.018). The median survival time was 10.2 months in the experimental group and 5.4 months in the control group (Pjaundice, therefore, is a safe and effective treatment in this malignancy.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  1. Plectin controls biliary tree architecture and stability in cholestasis.

    Science.gov (United States)

    Jirouskova, Marketa; Nepomucka, Katerina; Oyman-Eyrilmez, Gizem; Kalendova, Alzbeta; Havelkova, Helena; Sarnova, Lenka; Chalupsky, Karel; Schuster, Bjoern; Benada, Oldrich; Miksatkova, Petra; Kuchar, Martin; Fabian, Ondrej; Sedlacek, Radislav; Wiche, Gerhard; Gregor, Martin

    2017-12-20

    Plectin, a highly versatile cytolinker protein, controls intermediate filament cytoarchitecture and cellular stress response. In the present study, we investigate the role of plectin in the liver under basal conditions and in experimental cholestasis. We generated liver-specific plectin knockout (Ple Δalb ) mice and analyzed them using two cholestatic liver injury models: bile duct ligation (BDL) and 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) feeding. Primary hepatocytes and a cholangiocyte cell line were used to address the impact of plectin on keratin filament organization and stability in vitro. Plectin deficiency in hepatocytes and biliary epithelial cells led to aberrant keratin filament network organization, biliary tree malformations, and collapse of bile ducts and ductules. Further, plectin ablation significantly aggravated biliary damage upon cholestatic challenge. Coincidently, we observed a significant expansion of A6-positive progenitor cells in Ple Δalb livers. After BDL, plectin-deficient bile ducts were prominently dilated with more frequent ruptures corresponding to an increased number of bile infarcts. In addition, more abundant keratin aggregates indicated less stable keratin filaments in Ple Δalb hepatocytes. A transmission electron microscopy analysis revealed a compromised tight junction formation in plectin-deficient biliary epithelial cells. In addition, protein profiling showed increased expression of the adherens junction protein E-Cadherin, and inefficient upregulation of the desmosomal protein desmoplakin in response to BDL. In vitro analyses revealed a higher susceptibility of plectin-deficient keratin networks to stress-induced collapse, paralleled by elevated activation of p38 MAP kinase. Our study shows that by maintaining proper keratin network cytoarchitecture and biliary epithelial stability, plectin plays a critical role in protecting the liver from stress elicited by cholestasis. Plectin is a cytolinker protein capable of

  2. Intra-biliary contrast-enhanced ultrasound for evaluating biliary obstruction during percutaneous transhepatic biliary drainage: A preliminary study

    Energy Technology Data Exchange (ETDEWEB)

    Xu, Er-jiao [Department of Medical Ultrasonics, The Third Affiliated Hospital of Sun Yat-sen University, No. 600 Tianhe Road, Guangzhou 510630 (China); Zheng, Rong-qin, E-mail: zhengrq@mail.sysu.edu.cn [Department of Medical Ultrasonics, The Third Affiliated Hospital of Sun Yat-sen University, No. 600 Tianhe Road, Guangzhou 510630 (China); Su, Zhong-zhen; Li, Kai; Ren, Jie; Guo, Huan-yi [Department of Medical Ultrasonics, The Third Affiliated Hospital of Sun Yat-sen University, No. 600 Tianhe Road, Guangzhou 510630 (China)

    2012-12-15

    Objectives: The aimed of this study was to investigate the value of intra-biliary contrast-enhanced ultrasound (IB-CEUS) for evaluating biliary obstruction during percutaneous transhepatic biliary drainage (PTBD). Materials and methods: 80 patients with obstructive jaundice who underwent IB-CEUS during PTBD were enrolled. The diluted ultrasound contrast agent was injected via the drainage catheter to perform IB-CEUS. Both conventional ultrasound and IB-CEUS were used to detect the tips of the drainage catheters and to compare the detection rates of the tips. The obstructive level and degree of biliary tract were evaluated by IB-CEUS. Fluoroscopic cholangiography (FC) and computer tomography cholangiography (CTC) were taken as standard reference for comparison. Results: Conventional ultrasound displayed only 43 tips (43/80, 53.8%) of the drainage catheters within the bile ducts while IB-CEUS identified all 80 tips (80/80, 100%) of the drainage catheters including 4 of them out of the bile duct (P < 0.001). IB-CEUS made correct diagnosis in 44 patients with intrahepatic and 36 patients with extrahepatic biliary obstructions. IB-CEUS accurately demonstrated complete obstruction in 56 patients and incomplete obstruction in 21 patients. There were 3 patients with incomplete obstruction misdiagnosed to be complete obstruction by IB-CEUS. The diagnostic accuracy of biliary obstruction degree was 96.3% (77/80). Conclusion: IB-CEUS could improve the visualization of the drainage catheters and evaluate the biliary obstructive level and degree during PTBD. IB-CEUS may be the potential substitute to FC in the PTBD procedure.

  3. 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

  4. Preoperative Biliary Drainage in Patients with Obstructive Jaundice: History and Current Status

    NARCIS (Netherlands)

    van der Gaag, N.A.; Kloek, J.J.; de Castro, S.M.M.; Busch, O.R.C.; van Gulik, T.M.; Gouma, D.J.

    2009-01-01

    Preoperative biliary drainage (PBD) has been introduced to improve outcome after surgery in patients suffering from obstructive jaundice due to a potentially resectable proximal or distal bile duct/pancreatic head lesion. In experimental models, PBD is almost exclusively associated with beneficial

  5. The Management of Cyanotic Spells in Children with Oesophageal Atresia.

    Science.gov (United States)

    Bergeron, Mathieu; Cohen, Aliza P; Cotton, Robin T

    2017-01-01

    Cyanotic spells, also known as blue spells, dying spells, or apparent life-threatening events, refer to a bluish tone visible in the mucosal membranes and skin caused by an oxygen decrease in the peripheral circulation. Although this decrease may be transient and benign, it may also be indicative of a severe underlying problem that requires immediate intervention. Children with oesophageal atresia (OA) are at risk for a number of coexisting conditions that may trigger cyanotic spells. This current article will focus on the management of cyanotic spells both in children with innominate artery compression and those with tracheomalacia.

  6. 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.

  7. The Management of Cyanotic Spells in Children with Oesophageal Atresia

    Directory of Open Access Journals (Sweden)

    Mathieu Bergeron

    2017-05-01

    Full Text Available Cyanotic spells, also known as blue spells, dying spells, or apparent life-threatening events, refer to a bluish tone visible in the mucosal membranes and skin caused by an oxygen decrease in the peripheral circulation. Although this decrease may be transient and benign, it may also be indicative of a severe underlying problem that requires immediate intervention. Children with oesophageal atresia (OA are at risk for a number of coexisting conditions that may trigger cyanotic spells. This current article will focus on the management of cyanotic spells both in children with innominate artery compression and those with tracheomalacia.

  8. Atresia pulmonar con comunicación interventricular

    OpenAIRE

    Centella Hernández, Tomasa; Stanescu, Dennis; Stanescu, Sinziana

    2014-01-01

    La atresia pulmonar con comunicación interventricular es una cardiopatía congénita cianótica, severa y rara, de alta complejidad, que se caracteriza por la ausencia de conexión entre el ventrículo derecho y las arterias pulmonares. Coexiste con una comunicación interventricular. El flujo hacia el territorio pulmonar puede realizarse a través del ductus arterioso o de colaterales sistémico-pulmonares. La dificultad de esta cardiopatía viene determinada por los diferentes niveles de interrupció...

  9. Clinical Study on Using (125)I Seeds Articles Combined with Biliary Stent Implantation in the Treatment of Malignant Obstructive Jaundice.

    Science.gov (United States)

    Wang, Tao; Liu, Sheng; Zheng, Yan-Bo; Song, Xue-Peng; Sun, Bo-Lin; Jiang, Wen-Jin; Wang, Li-Gang

    2017-08-01

    Aim: To study the feasibility and curative effect of(125)I seeds articles combined with biliary stent implantation in the treatment of malignant obstructive jaundice. Patients and Methods: Fifty patients with malignant obstructive jaundice were included. Twenty-four were treated by biliary stent implantation combined with intraluminal brachytherapy by (125)I seeds articles as the experimental group, while the remaining 26 were treated by biliary stent implantation only as the control group. The goal of this study was to evaluate total bilirubin, direct bilirubin and tumor markers (cancer antigen (CA)-199, CA-242 and carcinoembryonic antigen (CEA)), as well as biliary stent patency status and survival time before and after surgery. Results: Jaundice improved greatly in both groups. The decreases of CA-199 and CA-242 had statistical significance (p=0.003 and p=0.004) in the experimental group. The ratio of biliary stent patency was 83.3% (20/24) in the experimental group and 57.7% (15/26) in the control group (p=0.048). The biliary stent patency time in the experimental group was 1~15.5 (mean=9.84) months. The biliary stent patency time in the control group was 0.8~9 (mean=5.57) months, which was statistically significant (p=0.018). The median survival time was 10.2 months in the experimental group, while 5.4 months in control group (pjaundice possibly by inhibiting the proliferation of vascular endothelial cells and the growth of tumor. Copyright© 2017, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.

  10. Biliary tree and cholecyst: post surgery imaging

    Energy Technology Data Exchange (ETDEWEB)

    Valek, Vlastimil [Department of Radiology, University Hopistal Brno, Jihlavska 20, 63900 Brno (Czech Republic)]. E-mail: v.valek@fnrbno.cz; Kala, Zdenek [Department of Surgery, University Hospital Brno, Jihlavska 20, 63900 Brno (Czech Republic); Kysela, Petr [Department of Surgery, University Hospital Brno, Jihlavska 20, 63900 Brno (Czech Republic)

    2005-03-01

    Recently, with improvements in surgical techniques there has been a substantial reduction in the incidence of biliary complications of hepatobiliary surgery. Nevertheless, bile duct injuries and other post-cholecystectomy complications are a serious problem and a major cause of morbidity and mortality. Early complications may include bile duct injury caused by mistakenly placed clips, erroneous cutting of bile ducts based on misinterpretation of biliary anatomy, periductal bile leakage that causes edema, fibrosis and secondary stricturing, and ischemia due to injury to the right hepatic artery. Bile duct strictures are the most common of the late complications and can develop a few months or many years after surgery. Early detection and accurate diagnosis have a fundamental importance for the successful treatment of these complications. Therefore, early and meaningful application of the imaging methods immediately after detection of the first symptoms is essential. Peroperative ultrasound and direct iodine contrast application into the biliary tree (operative cholangiography) are highly important for immediate visualization of the complications during surgery. Ultrasound can be used to aid in identification of ductal structures and the cholangiogram should be obtained to document the anatomy. Plain abdominal film could be made in the patients in poor clinical conditions after biliary surgery. Oral cholecystography has largely been replaced by ultrasonography (US) for evaluation of cholelithiasis and complications like post-cholecystectomy fluid collections. The same methodology replaced the conventional intravenous cholangiography. Nowadays computed tomography (CT), endoscopic retrograde cholangiopancreatography (ERCP), magnetic resonance cholangiography (MRCP) and ultrasound (US) have essential roles as primary imaging modalities after biliary tree and gallbladder surgery in the evaluation of associated complications and residual biliary stones. We review the role

  11. Ursodeoxycholic acid for patients with primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Gong, Yan; Huang, Zhibi; Christensen, Erik

    2007-01-01

    Ursodeoxycholic acid (UDCA) is used for primary biliary cirrhosis (PBC), but the beneficial effects remain controversial.......Ursodeoxycholic acid (UDCA) is used for primary biliary cirrhosis (PBC), but the beneficial effects remain controversial....

  12. Biliary duct obstruction treatment with aid of percutaneous ...

    African Journals Online (AJOL)

    Biliary duct obstruction treatment with aid of percutaneous transhepatic biliary drainage. Daniel Knap, Natalia Orlecka, Renata Judka, Aleksandra Juza, Michał Drabek, Maciej Honkowicz, Tomasz Kirmes, Bartosz Kadłubicki, Dominik Sieron, Jan Baron ...

  13. Biliary cholesterol secretion : More than a simple ABC

    NARCIS (Netherlands)

    Dikkers, Arne; Tietge, Uwe J. F.

    2010-01-01

    Biliary cholesterol secretion is a process important for 2 major disease complexes, atherosclerotic cardiovascular disease and cholesterol gallstone disease With respect to cardiovascular disease, biliary cholesterol secretion is regarded as the final step for the elimination of cholesterol

  14. Intestinal atresia and ectopia in a bovine fetus.

    Science.gov (United States)

    Lejeune, B; Miclard, J; Stoffel, M H; Meylan, M

    2011-07-01

    A 2-year-old Red Holstein cow was presented with uterine torsion at 235 days of pregnancy. The fetus extracted by cesarean section had weak vital signs and marked abdominal distention. An edematous pouch that contained tubular structures with peristaltic activity was associated with the umbilical cord. Because of poor prognosis, both dam and fetus were euthanized. At necropsy, the fetus had severe distention of the forestomachs, abomasum, and proximal small intestine; absence of distal small intestine, cecum, and proximal colon; atresia of the 2 blind ends of the intestine; and atrophy of distal colon and rectum. The tubular structures associated with the umbilical cord were identified as the segments of intestine that were absent in the fetus. Intestinal atresia combined with ectopia may be caused by local ischemia during temporary herniation and rotation of the fetal gut into the extraembryonic coelom. The close connection between ectopic intestine and amniotic sheath of the umbilical cord in this case may have facilitated vascularization and allowed development and viability of the ectopic intestine. © The Authors 2011

  15. A treatment program for babies with esophageal atresia in Belize.

    Science.gov (United States)

    Rosati, Shannon F; Maarouf, Rami; Oiticica, Claudio; Lange, Patricia; Haynes, Jeffrey; Lanning, David

    2015-11-01

    Children born with congenital anomalies in low- and middle-income countries often face a multitude of challenges. Access to pediatric surgical services is limited because of a lack of medical facilities, an adequate transportation system, and a lack of trained surgeons, anesthesiologists, and nurses, all of which leads to a high mortality rate. This is a report of a 5-y collaborative effort between the World Pediatric Project, the Children's Hospital of Richmond at Virginia Commonwealth University, and multiple organizations within the country of Belize to provide care for infants born with esophageal atresia, with or without associated tracheoesophageal fistula. A total of six infants were transferred to our institution in Richmond, VA for operative correction of their esophageal atresia. Caring for infants with congenital anomalies can be challenging, especially children from low- and middle-income countries. Through collaboration between countries and nonprofit organizations, life-saving international care can be provided to children for these conditions. Copyright © 2015 Elsevier Inc. All rights reserved.

  16. Computer-aided virtual surgery for congenital aural atresia.

    Science.gov (United States)

    Smouha, E E; Chen, D; Li, B; Liang, Z

    2001-03-01

    Computer-enhanced three-dimensional (3D) computed tomography (CT) provides accurate spatial representation of the complex surgical anatomy of congenitally atretic ears, and is superior to conventional CT for surgical planning. The surgical repair of congenital aural atresia is challenging. Conventional CT, routinely used for surgical planning, is limited in its ability to represent spatial relationships between important structures. Because of the lack of density differences between bony structures in the ear, 3D CT has thus far been useful for representing surface contour but not internal anatomy. A two-level segmentation scheme was developed to distinguish structures in the temporal bone. 3D CT reconstructions of congenital ears were produced with a high-resolution helical scanner. An interactive tool was used to mark the ossicles and facial nerve. The segmentation scheme was used to color-enhance the ossicles and otic capsule, and render the surrounding bone translucent. "Virtual surgery" was then performed by subtracting a cylindrical volume of bone lateral to the atresia plate. The enhanced 3D CT reconstructions were correlated with intraoperative video recordings. In four congenital ears, computer-enhanced 3D CT was highly predictive of the actual anatomy. Surgery was avoided in two anatomically unfavorable cases. Computer-enhanced 3D CT is a major advance over conventional CT for demonstrating the complex spatial relationships in congenitally atretic ears.

  17. Biliary Lithiasis with Choledocolithiasis in Children.

    Science.gov (United States)

    Bălănescu, R N; Bălănescu, L; Drăgan, G; Moga, A; Caragaţă, R

    2015-01-01

    Although biliary lithiasis has been considered a less common pathology in the pediatric population than in adults, in recent years, it has increasingly been diagnosed in children, with a prevalence of between 0.13 to 0.22. The elective treatment of symptomatic biliary lithiasis is cholecystectomy, the laparoscopic approach being considered the œgold standard. We present 3 cases referred to our clinic with biliary lithiasis, in which we performed laparoscopic cholecystectomy. We performed intraoperative cholangiography with a 4 Fr transcystic catheter. In the first case, the cholangiography showed a dilated CBD, without obstruction. Considering the patient'™s history, with recurrent episodes of choledocal lithiasis, we decided to perform a transcystic drainage. In the second case, cholangiography showed a normal CBD and no obstruction. In the third case cholangiography could not be performed due to technical issues. In all cases we performed retrograde laparoscopic cholecystectomy. The postoperative evolution in all cases was favorable. Studies conducted in the last years showed that laparoscopic cholecystectomy is a safe and efficient approach in the management of symptomatic biliary lithiasis in the paediatric age group. The management of choledocolithiasis is still not well defined: perioperative ERCP with ES, intraoperative cholangiography or intraoperative ultrasound were proposed as options in exploring the biliary tree. Celsius.

  18. Is biliary lithiasis associated with pancreatographic changes?

    Science.gov (United States)

    Barthet, M; Affriat, C; Bernard, J P; Berthezene, P; Dagorn, J C; Sahel, J

    1995-01-01

    The aetiological role of biliary lithiasis for chronic pancreatitis remains controversial. Previous studies based on pancreatographic studies reported changes in the pancreatic duct system caused by biliary lithiasis. This study analysed retrospectively the endoscopic retrograde cholangiopancreatography of 165 patients presenting with biliary lithiasis and of 53 controls. Among the 165 patients, 113 had choledochal stones (53 with gall bladder stones, 50 had had a cholecystectomy, 10 with a normal gall bladder), 35 had gall bladder stones without choledochal stones, 17 had cholecystectomy for gall bladder stones. Pancreatograms were analysed by measuring the diameter of the pancreatic duct in the head, the body, and the tail of the pancreas, and evaluating the regularity of the main pancreatic duct and the presence of stenosis, the regularity or the dilatation of secondary ducts, and the presence of cysts. In addition, we established a score, based on the above parameters, by which pancreatograms were classified as normal or with mild, intermediate, moderate or severe abnormalities. A multivariate analysis (stepwise multiple discriminant analysis) was performed for age, sex, presence of gall stones, presence of choledochal stones. Patients were comparable with controls for sex, alcohol consumption but were younger (55 v 68 years, p biliary lithiasis in itself is not an aetiological factor for chronic pancreatitis, older age being responsible for the abnormalities seen by pancreatography of patients with biliary lithiasis. PMID:7797128

  19. [Therapeutical options in lithiasic biliary fistula].

    Science.gov (United States)

    Ungureanu, D; Brătucu, E; Daha, C

    2001-01-01

    Chronic lythiasic cholecystitis is a disease distinguished by the pathologic changes because of the chronic inflammation of the biliary extrahepatic tree. Sometimes these morphological changes are associated with internal biliary fistulas arising spontaneously in patients with advanced calculus cholecystitis. The vast majority of fistulas result from the adherence of the inflamed gallbladder or common bile duct to an adjacent viscus and erosion of the gallstones into the adherent organ. The authors analyze an amount of 43 patients with bilio-biliary and bilio-digestive lythiasic fistulas, caused by the long evolution of chronic lythiasic atrophic cholecystitis, for 126 cases which were operated in the Surgery Department of the Caritas Clinic Hospital along 20 years. In these 126 cases the surgical strategy was determinated by the method of dealing with the pericolecystitis sclerotic blocks, by the identification methods of the biliary elements and by the approach of the main billiary way and digestive loops injuries occurred after suppressing the fistulae. The surgical solutions adapted for each separate case and the advantages of the axial drainage of prostheting the surgical reconstructions of the main biliary way as well as the results obtained along this project, are in fact the aims of this presentation.

  20. Aspects of biliary tract cancer : incidence and reproductive risk factors

    OpenAIRE

    Kilander, Carl

    2016-01-01

    Biliary tract cancer, including cancer of the extra-hepatic bile ducts, cancer of the Ampulla of Vater and gallbladder cancer, is a devastating disease with poor prognosis. The incidence of biliary tract cancer is decreasing worldwide, for unknown reasons. One of two aims of this thesis was to evaluate the Swedish Cancer Register regarding biliary tract cancer and to assess the incidence of biliary tract cancer in Sweden. Secondly, an association between sex hormone exposure, p...

  1. Portal biliopathy treated with endoscopic biliary stenting

    Directory of Open Access Journals (Sweden)

    Sung Jin Jeon

    2016-03-01

    Full Text Available Portal biliopathy is defined as abnormalities in the extra- and intrahepatic ducts and gallbladder of patients with portal hypertension. This condition is associated with extrahepatic venous obstruction and dilatation of the venous plexus of the common bile duct, resulting in mural irregularities and compression of the biliary tree. Most patients with portal biliopathy remain asymptomatic, but approximately 10% of them advance to symptomatic abdominal pain, jaundice, and fever. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography are currently used as diagnostic tools because they are noninvasive and can be used to assess the regularity, length, and degree of bile duct narrowing. Management of portal biliopathy is aimed at biliary decompression and reducing the portal pressure. Portal biliopathy has rarely been reported in Korea. We present a symptomatic case of portal biliopathy that was complicated by cholangitis and successfully treated with biliary endoscopic procedures.

  2. Portal biliopathy treated with endoscopic biliary stenting.

    Science.gov (United States)

    Jeon, Sung Jin; Min, Jae Ki; Kwon, So Young; Kim, Jun Hyun; Moon, Sun Young; Lee, Kang Hoon; Kim, Jeong Han; Choe, Won Hyeok; Cheon, Young Koog; Kim, Tae Hyung; Park, Hee Sun

    2016-03-01

    Portal biliopathy is defined as abnormalities in the extra- and intrahepatic ducts and gallbladder of patients with portal hypertension. This condition is associated with extrahepatic venous obstruction and dilatation of the venous plexus of the common bile duct, resulting in mural irregularities and compression of the biliary tree. Most patients with portal biliopathy remain asymptomatic, but approximately 10% of them advance to symptomatic abdominal pain, jaundice, and fever. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography are currently used as diagnostic tools because they are noninvasive and can be used to assess the regularity, length, and degree of bile duct narrowing. Management of portal biliopathy is aimed at biliary decompression and reducing the portal pressure. Portal biliopathy has rarely been reported in Korea. We present a symptomatic case of portal biliopathy that was complicated by cholangitis and successfully treated with biliary endoscopic procedures.

  3. [Retrograde endoscopic cholangiography after biliary surgery].

    Science.gov (United States)

    Monges, B; Richieri, J P; Pin, G; Grimaud, J C; Salducci, J

    1984-05-01

    Retrograde cholangiography was conducted in 310 patients early or late after biliary system surgery. Principal indications for the investigation were angiocholitis (37% of cases), isolated jaundice (30%) or acute hepatic colic (17%). Biliary tract lesions were excluded in 7% of patients while in the remaining 93% it was possible to determine the precise cause of the disorder. In 30% of cases the disorder was unrelated to the previous operation or the lithiasic disease, whereas in 63% it was related to the latter (remaining or reformed stone, odditis) or to the surgical procedure (stenosis, common bile duct ligature or injury to main bile duct). These results, and those reported in the ligature, suggest that retrograde cholangiography can provide a precise diagnosis and ensure adapted therapy after biliary surgery.

  4. Current endoscopic approach to indeterminate biliary strictures

    Science.gov (United States)

    Victor, David W; Sherman, Stuart; Karakan, Tarkan; Khashab, Mouen A

    2012-01-01

    Biliary strictures are considered indeterminate when basic work-up, including transabdominal imaging and endoscopic retrograde cholangiopancreatography with routine cytologic brushing, are non-diagnostic. Indeterminate biliary strictures can easily be mischaracterized which may dramatically affect patient’s outcome. Early and accurate diagnosis of malignancy impacts not only a patient’s candidacy for surgery, but also potential timely targeted chemotherapies. A significant portion of patients with indeterminate biliary strictures have benign disease and accurate diagnosis is, thus, paramount to avoid unnecessary surgery. Current sampling strategies have suboptimal accuracy for the diagnosis of malignancy. Emerging data on other diagnostic modalities, such as ancillary cytology techniques, single operator cholangioscopy, and endoscopic ultrasonography-guided fine needle aspiration, revealed promising results with much improved sensitivity. PMID:23180939

  5. Quantitative Assessment of the Rat Intrahepatic Biliary System by Three-Dimensional Reconstruction

    Science.gov (United States)

    Masyuk, Tatyana V.; Ritman, Erik L.; LaRusso, Nicholas F.

    2001-01-01

    The anatomical details of the biliary tree architecture of normal rats and rats in whom selective proliferation was induced by feeding α-naphthylisothiocyanate (ANIT) were reconstructed in three dimension using a microscopic-computed tomography scanner. The intrahepatic biliary tree was filled with a silicone polymer through the common bile duct and each liver lobe embedded in Bioplastic; specimens were then scanned by a microscopic-computed tomography scanner and modified Feldkamp cone beam backprojection algorithm applied to generate three-dimensional images. Quantitative analysis of bile duct geometry was performed using a customized software program. The diameter of the bile duct segments of normal and ANIT-fed rats progressively decreased with increasing length of the biliary tree. Diameter of bile ducts from ANIT-fed rats (range, 21 to 264 μm) was similar to that of normal rats (22 to 279 μm). In contrast, the number of bile duct segments along the major branch reproducibly doubled, the length of the bile duct segments decreased twofold, and the length of the biliary tree remained unchanged after ANIT feeding. Moreover, the total volume of the biliary tree of ANIT-fed rats was significantly greater (855 μl) than in normal rats (47 μl). Compared with normal rats, the total surface area of the biliary tree increased 26 times after ANIT-induced bile duct proliferation. Taken together, these observations quantitate the anatomical remodeling after selective cholangiocyte proliferation and strongly suggest that the proliferative process involves sprouting of new side branches. Our results may be relevant to the mechanisms by which ducts proliferate in response to hepatic injury and to the hypercholeresis that occurs after experimentally induced bile duct proliferation. PMID:11395385

  6. Origin, pattern, and mechanism of bile duct proliferation following biliary obstruction in the rat.

    Science.gov (United States)

    Slott, P A; Liu, M H; Tavoloni, N

    1990-08-01

    Proliferation of bile duct-like structures is a hepatic cellular reaction observed in most forms of human liver disease and in a variety of experimental conditions associated with liver injury. Yet the origin, means of initiation, and significance of this hyperplasia are unknown. To clarify these issues we induced bile duct proliferation in rats by ligating the common bile duct and studied (a) hepatic incorporation of [3H]thymidine by histoautoradiography, (b) hepatic morphometry, (c) biliary tree volume using [3H]taurocholate as a marker of biliary transit time, (d) immunohistochemical expression of cytokeratin no. 19, (e) the effect of indomethacin, and (f) the role of increased biliary pressure, in the absence of physiological and biochemical evidence of cholestasis, on [3H]thymidine incorporation by the bile-duct cells. The results have demonstrated that (a) the proliferating bile duct-like cells are products of the extant biliary epithelium and retain its characteristics; (b) bile duct cells divide irrespective of the size of the duct in which they are located and form a system with a lumen continuous with the preexisting one; (c) bile duct proliferation results mainly in elongation, not in circumferential enlargement or sprouting of side branches; (d) portal macrophage infiltration does not play a role in the hyperplastic reaction, and (e) increased biliary pressure is the initiating factor in bile duct cell division. Our results provide evidence that under the present conditions, ductular metaplasia of hepatocytes does not occur and there is no functioning stem cell for biliary epithelial growth segregated in any particular duct size or within the portal connective tissue.

  7. Acute abdominal pain due to biliary cystadenoma.

    Science.gov (United States)

    Van den Bergh, K; Op de Beeck, K

    2010-01-01

    We present a case of a biliary cystadenoma, a rare benign cystic tumor arising in most cases of the intrahepatic bile ducts. A 30-year old woman presented with abdominal pain in the right upper quadrant for about 10 days with increasing severity. Radiological evaluation by means of abdominal ultrasound and CT-scan revealed a multilocular cystic lesion in the right liver lobe, not present on abdominal ultrasound performed 10 years before. The diagnosis of a biliary cystadenoma was proposed and complete surgical resection of the mass was performed.

  8. CT diagnosis of biliary tract diseases

    Energy Technology Data Exchange (ETDEWEB)

    Sobota, J.; Horak, J.; Antos, Z.; Vodak, M. (Ustredni Vojenska Nemocnice, Prague (Czechoslovakia))

    1983-09-01

    The possibilities are discussed offered by computed tomography in the diagnosis of biliary tract diseases. Attention is paid to difficulties associated with the diagnosis of pathological changes in the biliary tract, and to the detection of isodense concrements where diagnostic problems are reliably resolved by PTHC and ERCP. It is therefore useful to supplement CT with other examination methods. A suitable combination is cholescintigraphy and CT with the possibility of a final diagnosis or selection of further examination, as a rule of an invasive nature.

  9. [Significance of biliary dysfunction in the pathogenesis of gallstone disease].

    Science.gov (United States)

    Il'chenko, I A; Deliukina, O V

    2011-01-01

    The paper shows the role of biliary dysfunction in the formation of biliary sludge. Found that among all motor dysfunction themost common is reduction of contractile function of the gallbladder (in 63.3% of cases), which is combined with 73.2% Oddi's sphincter hypertonus. T The combination of ursodeoxycholic acid (UDCA) with mebeverine has a better effect than monotherapy with UDCA, as increases the frequency of relief of biliary dyspepsia symptoms, normalizes the biliary tract functional status and 95% of cases leads to the elimination of biliary sludge.

  10. Extrahepatic biliary cystadenoma with mesenchymal stroma: a true biliary cystadenoma? A case report.

    LENUS (Irish Health Repository)

    Hennessey, Derek B

    2012-02-01

    Biliary cystadenomas are benign but potentially malignant cystic neoplasms, which classically contain mesenchymal stroma similar to ovarian tissue. We report a case of an extra-hepatic biliary cystadenoma with mesenchymal stroma along with a discussion of current pathological opinion. CASE PRESENTATION: A 54-year-old female presented with abdominal pain, abnormal liver function tests and a mass on ultrasound. Computerized Tomography identified a complex multi-locular cyst in the common hepatic duct. Radical excision of the lesion and a Roux-en-Y loop bilio-enteric anastomosis was performed. Histology confirmed the presence of a benign biliary cystadenoma with ovarian type stroma. CONCLUSION: Biliary cystadenomas classically contain mesenchymal stroma similar to ovarian tissue. It now appears that cystadenomas without mesenchymal stroma appear to be more akin to similar cystic lesions of the pancreas, and may represent a dissimilar neoplasm. Therefore, malignant transformation can occur, so complete excision is recommended.

  11. Extrahepatic biliary cystadenoma with mesenchymal stroma: a true biliary cystadenoma? A case report.

    Science.gov (United States)

    Hennessey, Derek B; Traynor, Oscar

    2011-06-01

    Biliary cystadenomas are benign but potentially malignant cystic neoplasms, which classically contain mesenchymal stroma similar to ovarian tissue. We report a case of an extra-hepatic biliary cystadenoma with mesenchymal stroma along with a discussion of current pathological opinion. A 54-year-old female presented with abdominal pain, abnormal liver function tests and a mass on ultrasound. Computerized Tomography identified a complex multi-locular cyst in the common hepatic duct. Radical excision of the lesion and a Roux-en-Y loop bilio-enteric anastomosis was performed. Histology confirmed the presence of a benign biliary cystadenoma with ovarian type stroma. Biliary cystadenomas classically contain mesenchymal stroma similar to ovarian tissue. It now appears that cystadenomas without mesenchymal stroma appear to be more akin to similar cystic lesions of the pancreas, and may represent a dissimilar neoplasm. Therefore, malignant transformation can occur, so complete excision is recommended.

  12. 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.

  13. El manejo de la atresia intestinal tipo IIIb sigue siendo un reto

    National Research Council Canada - National Science Library

    Gomez, Martin; Beltran, Tatiana Carolina

    2010-01-01

    La atresia intestinal constituye una de las causas mas frecuentes de obstruccion intestinal en el recien nacido y es causada por catastrofes vasculares mesentericas tardias durante el desarrollo embrionario...

  14. [Deletion 11q23 --> qter (Jacobsen Syndrome) associated with duodenal atresia and annular pancreas].

    Science.gov (United States)

    Fernández González, N; Prieto Espuñes, S; Ibáñez Fernández, A; Fernández Colomer, B; López Sastre, J; Fernández Toral, J

    2002-09-01

    Jacobsen syndrome is a rare chromosomal disorder due to terminal 11q deletion. Prominent features are growth and psychomotor retardation, trigonocephaly and a characteristic facial dysmorphism, but many different abnormalities have been reported. We present the case of a preterm male. Prenatal ultrasonography was suspicious for duodenal atresia. At birth, the boy presented the craniofacial features typical of Jacobsen syndrome, together with diffusely spread petechiae and talipes equinovarus. Hemogram revealed pancytopenia. Ultrasound examination showed left renal agenesis and confirmed the duodenal atresia. Cerebral computed tomography scan, electroencephalogram and cardiac studies showed no abnormalities. Annular pancreas was found during surgery to correct the duodenal atresia. The karyotype was 46,XY,del(11)(q23.2 --> qter), which confirmed Jacobsen syndrome.A wide spectrum of clinical features is described in Jacobsen syndrome, with phenotype-karyotype correlation. This is the first report of duodenal atresia and annular pancreas.

  15. Correlation between high-resolution computed tomography and surgical findings in congenital aural atresia.

    Science.gov (United States)

    Mehra, Y N; Dubey, S P; Mann, S B; Suri, S

    1988-02-01

    Congenital aural atresia poses a great challenge, even to a competent otologic surgeon, due to innumerable types of malformations affecting the conduction and perception of sound. Roentgenographic evaluation by plain roentgenography is inconclusive in most cases; polytomography is helping to some extent. Recent generations of high-resolution computed tomographic (CT) scanners are probably the best. Seven cases of unilateral and three cases of bilateral congenital aural atresia were evaluated with high-resolution CT. Subsequently, ten ears were operated on. Surgical findings were correlated with CT scan findings with respect to atresia plate, extent of pneumatization, ossicular anomalies, bony facial nerve canal, and inner ear. Use of CT scans bears considerable importance in the management of these types of cases. High-resolution CT scan, when targeted for maximal bony detail, is possibly the method of choice in congenital aural atresia when surgical correction is contemplated.

  16. Interruption of the aortic arch, ventricular septal defect, aortic atresia and aortopulmonary fistulous communication.

    Science.gov (United States)

    De Caro, E; Pongiglione, G; Ribaldone, D

    1998-06-01

    Interruption of the aortic arch in association with aortic atresia is a rare condition. We report the second case in literature in which survival was provided by an aortopulmonary fistulous communication.

  17. Coronary artery to pulmonary artery communications in pulmonary atresia with ventricular septal defect

    National Research Council Canada - National Science Library

    Sridhar, Anuradha; Subramanyan, Raghavan; Cherian, Kotturathu Mammen

    2013-01-01

    ...% of patients with pulmonary atresia and ventricular septal defect (PA-VSD). A diligent look for these abnormal communications is important to prevent perioperative complications and achieve a complete repair...

  18. Osteopathic manipulative treatment in the management of biliary dyskinesia.

    Science.gov (United States)

    Heineman, Katherine

    2014-02-01

    Biliary dyskinesia is a functional gastrointestinal disorder of the gallbladder and sphincter of Oddi. Diagnosis is made on the basis of symptoms of biliary colic in the absence of cholelithiasis and gallbladder inflammation. Palpatory findings of tissue texture changes at midthoracic levels (T6-T9) may correspond to visceral dysfunction related to the biliary system. Osteopathic manipulative treatment (OMT) of the T6-T9 segments can remove the feedback related to the somatic component, thereby affecting nociceptive facilitation at the spinal level and allowing the body to restore autonomic balance. Few reports in the current literature provide examples of treatment for patients with biliary dyskinesia using OMT. The author describes the case of a 51-year-old woman who presented with symptoms consistent with biliary dyskinesia. Her biliary colic completely resolved after OMT. Osteopathic evaluation and OMT should be considered a safe and effective option for conservative management of biliary dyskinesia.

  19. 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.

  20. 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.

  1. Atresia pulmonar con comunicación interventricular

    Directory of Open Access Journals (Sweden)

    Tomasa Centella Hernández

    2014-04-01

    Full Text Available La atresia pulmonar con comunicación interventricular es una cardiopatía congénita cianótica, severa y rara, de alta complejidad, que se caracteriza por la ausencia de conexión entre el ventrículo derecho y las arterias pulmonares. Coexiste con una comunicación interventricular. El flujo hacia el territorio pulmonar puede realizarse a través del ductus arterioso o de colaterales sistémico-pulmonares. La dificultad de esta cardiopatía viene determinada por los diferentes niveles de interrupción desde el ventrículo derecho hasta el territorio pulmonar, y por la diferencia anatómica de las fuentes del flujo hacia dicho territorio, lo que determina diferentes tipos de abordaje quirúrgico.

  2. Diagnosis of a biliary cystadenoma demonstrating communication with the biliary system by MRI using a hepatocyte-specific contrast agent.

    Science.gov (United States)

    Billington, P D; Prescott, R J; Lapsia, S

    2012-02-01

    Biliary cystadenomas are predominately benign tumours that have a low malignant potential. We present a case of a 30-year-old female with a histopathological confirmation of a biliary cystadenoma following resection. The diagnosis was made pre-operatively by MRI using the hepatocyte-specific contrast agent gadobenate dimeglumine (Gd-BOPTA) (MultiHance; Bracco Diagnostics Inc., Princeton, NJ). At the biliary excretory phase, delayed phase images demonstrated communication between the multilocular cystic mass and the biliary tree, which helped to confirm the diagnosis of biliary cystadenoma. This highlights the beneficial use of hepatocyte-specific agents for their dual function as an extracellular agent and a hepatobiliary agent.

  3. Glycomic analyses of ovarian follicles during development and atresia

    Science.gov (United States)

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

    2012-01-01

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

  4. 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.

  5. A pitfall in the diagnosis of bilateral choanal atresia: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Sang Tae; Lee, Shi Kyung; Rho, Eun Jin; Oh, Ga Yeoul; Kim, Jin Yong; Kim, Ki Hong; Han, Chun Hwan; Ahn, Young Min [Kangnam General Hospital Public Corporation, Seoul (Korea, Republic of)

    2001-03-01

    Choanal atresia is a rare congenital anomaly involving unilateral or bilateral posterior nasal choanal obstruction. Multiple associated anomalies have been described. We describe the case of a 1-month-old boy with bilateral choanal atresia, misdiagnosed after CT as a midline meningocele because the floor of the midline anterior cranial fossa was not ossified and secretion had accumulated in the obstructed posterior nasal choana.

  6. 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.

  7. Targeted Therapy for Biliary Tract Cancer

    Energy Technology Data Exchange (ETDEWEB)

    Furuse, Junji, E-mail: jfuruse@ks.kyorin-u.ac.jp [Department of Internal Medicine, Medical Oncology, Kyorin University School of Medicine, 6-20-2, Shinkawa, Mitaka, Tokyo, 181-8611 (Japan); Okusaka, Takuji [Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045 (Japan)

    2011-05-03

    It is necessary to establish effective chemotherapy to improve the survival of patients with biliary tract cancer, because most of these patients are unsuitable candidates for surgery, and even patients undergoing curative surgery often have recurrence. Recently, the combination of cisplatin plus gemcitabine was reported to show survival benefits over gemcitabine alone in randomized clinical trials conducted in the United Kingdom and Japan. Thus, the combination of cisplatin plus gemcitabine is now recognized as the standard therapy for unresectable biliary tract cancer. One of the next issues that need to be addressed is whether molecular targeted agents might also be effective against biliary tract cancer. Although some targeted agents have been investigated as monotherapy for first-line chemotherapy, none were found to exert satisfactory efficacy. On the other hand, monoclonal antibodies such as bevacizumab and cetuximab have also been investigated in combination with a gemcitabine-based regimen and have been demonstrated to show promising activity. Furthermore, clinical trials using new targeted agents for biliary tract cancer are also proposed. This cancer is a relatively rare and heterogeneous tumor consisting of cholangiocarcinoma and gallbladder carcinoma. Therefore, a large randomized clinical trial is necessary to confirm the efficacy of chemotherapy, and international collaboration is important.

  8. Primary biliary cirrhosis: Diagnostic and therapeutic aspects

    NARCIS (Netherlands)

    E.M.M. Kuiper (Edith)

    2010-01-01

    textabstractPrimary Biliary Cirrhosis (PBC) is a relatively rare cholestatic liver disease. The first case was described by Addison and Gull in 1851. The name PBC is generally accepted, however in fact this is a misnomer since cirrhosis is found in a minority of patients. PBC is one of the most

  9. Surgical treatment of congenital biliary duct cyst

    Directory of Open Access Journals (Sweden)

    Wang De-chun

    2012-03-01

    Full Text Available Abstract Background It is acknowledged that total cyst excision is a safe and ideal surgical treatment for congenital biliary duct cyst, compared to simple internal drainage. The aim of this study was to determine the optimal operation occasion and the effect of laparoscopy on congenital biliary duct cyst based upon total cyst excision. Methods From January 2002 to January 2011, 217 patients were admitted to Southwest Hospital for congenital biliary duct cyst. To determine the optimal surgery occasion, we divided these subjects into three groups, the infant group (age ≤ 3 years, the immaturity group (3 18 years, and then evaluated the feasibility, risk and long-term outcome after surgery in the three groups. To analyze the effect of laparoscopic technique on congenital biliary duct cyst, we divided the patients into the laparoscopy and the open surgery groups. Results Among the three groups, the morbidity from cholangiolithiasis before surgical treatment had obvious discrepancy (p 0.05. Similarly, no significant discrepancy was observed in the morbidity from postoperative complications or long-term postoperative complications (p > 0.05 between the laparoscopic and the open surgery groups. Conclusions We conclude that total cyst excision should be performed as early as possible. The optimal treatment occasion is the infant period, and laparoscopic resection may be a new safe and feasible minimally invasive surgery for this disease.

  10. [Biliary cystadenoma. Discussion of a clinical case].

    Science.gov (United States)

    Massaioli, N; Bertero, D; Buzio, M; Villata, E; Albertino, B

    1991-07-01

    A case of biliary cystadenoma is reported. The neoplasm was located at the left lobe of the liver and its diameter was 18 cm. It presented with abdominal swelling and palpable mass in epigastric region. A left hepatectomy was performed, to achieve a complete resection of the tumor.

  11. Fatal liver gas gangrene after biliary surgery

    Directory of Open Access Journals (Sweden)

    Yui Miyata

    2017-01-01

    Discussion: Liver gas gangrene is rare and has a high mortality rate. This case seems to have arisen from an immunosuppressive state after major surgery with biliary reconstruction for bile duct cancer and subsequent gastrointestinal bleeding, leading to gas gangrene of the liver.

  12. Twin pregnancy complicated by esophageal atresia, duodenal atresia, gastric perforation, and hypoplastic left heart structures in one twin: a case report and review of the literature.

    Science.gov (United States)

    Abou Chaar, Mohamad K; Meyers, Mariana L; Tucker, Bethany D; Galan, Henry L; Liechty, Kenneth W; Crombleholme, Timothy M; Marwan, Ahmed I

    2017-03-18

    The antenatal diagnosis of a combined esophageal atresia without tracheoesophageal fistula and duodenal atresia with or without gastric perforation is a rare occurrence. These diagnoses are difficult and can be suspected on ultrasound by nonspecific findings including a small stomach and polyhydramnios. Fetal magnetic resonance imaging adds significant anatomical detail and can aid in the diagnosis of these complicated cases. Upon an extensive literature review, there are no reports documenting these combined findings in a twin pregnancy. Therefore we believe this is the first case report of an antenatal diagnosis of combined pure esophageal and duodenal atresia in a twin gestation. We present a case of a 30-year-old G1P0 white woman at 22-week gestation with a monochorionic-diamniotic twin pregnancy discordant for esophageal atresia, duodenal atresia with gastric perforation, hypoplastic left heart structures, and significant early gestation maternal polyhydramnios. In this case, fetal magnetic resonance imaging was able to depict additional findings including area of gastric wall rupture, hiatal hernia, dilation of the distal esophagus, and area of duodenal obstruction and thus facilitated the proper diagnosis. After extensive counseling at our multidisciplinary team meeting, the parents elected to proceed with radiofrequency ablation of the anomalous twin to maximize the survival of the normal co-twin. The procedure was performed successfully with complete cessation of flow in the umbilical artery and complete cardiac standstill in the anomalous twin with no detrimental effects on the healthy co-twin. Prenatal diagnosis of complex anomalies in twin pregnancies constitutes a multitude of ethical, religious, and cultural factors that come into play in the management of these cases. Fetal magnetic resonance imaging provides detailed valuable information that can assist in management options including possible prenatal intervention. The combination of a cystic

  13. 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.

  14. Impact of unilateral conductive hearing loss due to aural atresia on academic performance in children.

    Science.gov (United States)

    Kesser, Bradley W; Krook, Kaelyn; Gray, Lincoln C

    2013-09-01

    This study evaluates the effect of unilateral conductive hearing loss secondary to aural atresia on elementary school children's academic performance. Case control survey and review of audiometric data. One hundred thirty-two surveys were mailed to families of children with aural atresia, and 48 surveys were sent to families of children with unilateral sensorineural hearing loss (SNHL) to identify rates of grade retention, use of any resource, and behavioral problems. Audiometric data of the cohort were tabulated. Of the 40 atresia patients, none repeated a grade, but 65% needed some resources: 12.5% currently use a hearing aid, 32.5% use(d) a frequency-modulated system in school, 47.5% had an Individualized Education Plan, and 45% utilized speech therapy. Compared to the unilateral SNHL group and a cohort of children with unilateral SNHL in an earlier study, children with unilateral atresia were less likely to repeat a grade. Children in both unilateral atresia and SNHL groups were more likely to utilize some resource in the academic setting compared to the unilateral SNHL children in the prior study. Unilateral conductive hearing loss due to aural atresia has an impact on academic performance in children, although not as profound when compared to children with unilateral SNHL. The majority of these children with unilateral atresia utilize resources in the school setting. Parents, educators, and health care professionals should be aware of the impact of unilateral conductive hearing loss and offer appropriate habilitative services. Copyright © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

  15. Percutaneous Biliary Drainage Using Open Cell Stents for Malignant Biliary Hilar Obstruction

    Energy Technology Data Exchange (ETDEWEB)

    Ahn, Sun Jun; Bae, Jae Ik; Han, Tae Sun; Won, Je Hwan; Kim, Ji Dae; Kwack, Kyu Sung; Lee, Jae Hee; Kim, Young Chul [Dept. of Radiology, Ajou University School of Medicine, Suwon (Korea, Republic of)

    2012-11-15

    To evaluate the feasibility, safety and the effectiveness of the complex assembly of open cell nitinol stents for biliary hilar malignancy. During the 10 month period between January and October 2007, 26 consecutive patients with malignant biliary hilar obstruction underwent percutaneous insertion of open cell design nitinol stents. Four types of stent placement methods were used according to the patients' ductal anatomy of the hilum. We evaluated the technical feasibility of stent placement, complications, patient survival, and the duration of stent patency. Bilobar biliary stent placement was conducted in 26 patients with malignant biliary obstruction-T (n = 9), Y (n 7), crisscross (n = 6) and multiple intersecting types (n = 4). Primary technical success was obtained in 24 of 26 (93%) patients. The crushing of the 1st stent during insertion of the 2nd stent occurred in two cases. Major complications occurred in 2 of 26 patients (7.7%). One case of active bleeding from hepatic segmental artery and one case of sepsis after procedure occurred. Clinical success was achieved in 21 of 24 (87.5%) patients, who were followed for a mean of 141.5 days (range 25-354 days). The mean primary stent patency period was 191.8 days and the mean patient survival period was 299 days. Applying an open cell stent in the biliary system is feasible, and can be effective, especially in multiple intersecting stent insertions in the hepatic hilum.

  16. A special growth manner of intrahepatic biliary cystadenoma.

    Science.gov (United States)

    Yi, Bin; Cheng, Qing-Bao; Jiang, Xiao-Qing; Liu, Chen; Luo, Xiang-Ji; Dong, Hui; Zhang, Bai-He; Wu, Meng-Chao

    2009-12-28

    We report a case of a 56-year-old woman with intrahepatic biliary cystadenoma (IBC) accompanying a tumor embolus in the extrahepatic bile duct, who was admitted to our department on October 13, 2008. Imaging showed an asymmetry dilation of the biliary tree, different bile signals in the biliary tree, a multiloculated lesion and an extrahepatic bile duct lesion with internal septation. A regular left hemihepatectomy en bloc was performed with resection of the entire tumor, during which a tumor embolus protruding into the extrahepatic bile duct and originating from biliary duct of segment 4 was revealed. Microscopically, the multiloculated tumor was confirmed to be a biliary cystadenoma with an epithelial lining composed of biliary-type cuboidal cells and surrounded by an ovarian-like stroma. An aggressive en bloc resection was recommended for the multiloculated lesion. Imaging workup, clinicians and surgeons need to be aware of this different presentation.

  17. Technical problems during percutaneous transhepatic biliary drainage

    Energy Technology Data Exchange (ETDEWEB)

    Weber, J.; Hoever, S.

    1985-11-01

    Technical problems are repeatedly encountered during percutaneous transhepatic drainage by catheter or endo-prosthesis which are caused by the position, extent and tightness of the stenosis in the biliary ducts. All means of overcoming these problems must be used, otherwise technical failure or complications become inevitable. The methods to be adopted if catheters break and endo-prostheses become displaced or occluded, and for haemorrhage, are described, as well as methods for draining multiple, proximal or intrahepatic stenoses. This is based on an experience with 74 patients on whom 130 drainage procedures have been perforemd (80 catheters, 50 endo-prostheses). All complications and mortality (five patients, 3.8%) were due to problems which could not be solved. Internal biliary drainage by means of an endoprosthesis is markedly superior to catheter drainage. (orig.).

  18. Interventional radiological treatment of malignant biliary obstruction

    Energy Technology Data Exchange (ETDEWEB)

    Saito, Hiroya; Sakurai, Yasuo; Takamura, Akio [Asahikawa Kosei General Hospital, Hokkaido (Japan)

    1997-06-01

    Metallic stents have been widely used relieve biliary obstructions as an alternative to plastic endoprostheses. In this study, we evaluate the efficacy of the metallic stents in the management of malignant biliary obstruction. If cancerous tissue was present in the bile duct, the stents embedded in the tumor and caliber of the bile duct was smaller than that of the stents. Metallic stents are indicated in case with less tumor in the bile duct. If bulky tumor exists in the bile duct, adequate combination therapy to reduce the volume of the tumor should be required. In the case of inefficient therapy, plastic endoprosthesis or covered stents should be chosen for long-term patency. (author)

  19. Intestinal obstruction for biliary lithiasis: case report

    Directory of Open Access Journals (Sweden)

    Miguel Enrique Rodriguez Rodriguez

    2017-06-01

    Full Text Available The biliary ileum is an infrequent complication of cholelithiasis, that is manifested as an intestinal obstruction, is more prevalent in aged people. The clinical square of the biliary ileum is diffuse and the Triad of Rigler is observed in less than 30% of the patient one. The surgical treatment goes from enterotomía and litotricia to the colecistectomía with treatment of the water-pipe. The case of a female, 86-year-old patient is presented that after a week of symptoms and of medical treatment it was detected the presence of a mechanical intestinal obstruction for which the patient underwent emergency surgery. As the cause of occlusion it was found a thick gallstone in a jejunal loop.

  20. Dexamethasone alters the hepatic inflammatory cellular profile without changes in matrix degradation during liver repair following biliary decompression.

    Science.gov (United States)

    Muratore, Christopher S; Harty, Mark W; Papa, Elaine F; Tracy, Thomas F

    2009-10-01

    Biliary atresia is characterized by extrahepatic bile duct obliteration along with persistent intrahepatic portal inflammation. Steroids are standard in the treatment of cholangitis following the Kasai portoenterostomy, and were advocated for continued suppression of the ongoing immunologic attack against intrahepatic ducts. Recent reports, however, have failed to demonstrate an improved patient outcome or difference in the need for liver transplant in postoperative patients treated with a variety of steroid regimes compared with historic controls. In the wake of progressive liver disease despite biliary decompression, steroids are hypothesized to suppress inflammation and promote bile flow without any supporting data regarding their effect on the emerging cellular and molecular mechanisms of liver repair. We have previously shown in a reversible model of cholestatic injury that repair is mediated by macrophages, neutrophils, and specific matrix metalloproteinase activity (MMP8); we questioned whether steroids would alter these intrinsic mechanisms. Rats underwent biliary ductal suspension for 7 d, followed by decompression. Rats were treated with IV dexamethasone or saline at the time of decompression. Liver tissue obtained at the time of decompression or after 2 d of repair was processed for morphometric analysis, immunohistochemistry, and quantitative RT-PCR. There was a dramatic effect of dexamethasone on the inflammatory component with the initiation of repair. Immunohistochemistry revealed a reduction of both ED1+ hepatic macrophages and ED2+Kupffer cells in repair compared with saline controls. Dexamethasone treatment also reduced infiltrating neutrophils by day 2. TNF-alpha expression, increased during injury in both saline and dexamethasone groups, was markedly reduced by dexamethasone during repair (day 2) whereas IL-6, IL-10, and CINC-1 remained unchanged compared with saline controls. Dexamethasone reduced both MMP8 and TIMP1 expression by day 2

  1. Tumor extension along percutaneous transhepatic biliary drainage tracts

    Energy Technology Data Exchange (ETDEWEB)

    Tersigni, R.; Bochicchio, O.; Cavallini, M.; Bufalini, G.; Alessandroni, L.; Arena, L.; Armeni, O.; Miraglia, F.; Stipa, S.; Rossi, P.

    1986-11-01

    Percutaneous Transhepatic Biliary Catheterization is commonly employed in the diagnosis and management of obstructive jaundice associated with malignant lesions. Tumor manipulation as an effort to obtain a histological diagnosis or to establish short or long-term internal-external biliary drainage is liable to disseminate the malignancy along the catheter tract. Two cases of malignant seeding of the catheter tract after biliary drainage have been observed.

  2. Molecular Identification of Biliary Isospora Belli

    OpenAIRE

    Chiu, King-Wah; Chiou, Shue-Shian; Lu, Lung-Sheng; Wu, Cheng-Kun; Eng, Hock-Liew

    2016-01-01

    Abstract This report describes the novel sampling of bile from the biliary endoscopic intervention for the molecular identification of parasite infection. A 63-year-old Vietnamese man underwent travel health examination in our hospital. Physical examination showed that his height was 159?cm and weight was 41?kg. He had a 15-year history of intermittent abdominal pain and frequent episodes of diarrhea. Laboratory tests revealed raised eosinophil count (23%, normal range [NR] 0?5), absolute eos...

  3. Colon Cancer Metastatic to the Biliary Tree

    OpenAIRE

    Strauss, Alexandra T.; Clayton, Steven B.; Markow, Michael; Mamel, Jay

    2016-01-01

    Metastasis of colon adenocarcinoma is commonly found in the lung, liver, or peritoneum. Common bile duct (CBD) tumors related to adenomas from familial adenomatous polyposis metastasizing from outside of the gastrointestinal tract have been reported. We report a case of biliary colic due to metastatic colon adenocarcinoma to the CBD. Obstructive jaundice with signs of acalculous cholecystitis on imaging in a patient with a history of colon cancer should raise suspicion for metastasis to CBD.

  4. Extrahepatic Manifestations of Primary Biliary Cholangitis

    OpenAIRE

    Chalifoux, Sara L; Konyn, Peter G; Choi, Gina; Saab, Sammy

    2017-01-01

    Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogren’s syndrome, thyroid dysfunction and systemic sclerosis. It is thought that patients with PBC are at increased risk of developing these extrahepatic manifestation...

  5. Complete biliary avulsion from blunt compression injury.

    Science.gov (United States)

    Arkovitz, M S; Liang, H; Pachter, H L; Alexander, P; Newman, R M; Gittes, G K

    1999-10-01

    The liver is the solid organ most commonly injured as a result of blunt abdominal trauma. Complete avulsion of the common hepatic duct is a rare and devastating type of hepatobiliary trauma. Here the authors report the case of a 7-year-old child who had complete biliary disruption as a result of an abdominal crush injury that was not diagnosed correctly preoperatively. The intraoperative diagnosis and treatment of this injury is discussed.

  6. Portal biliopathy treated with endoscopic biliary stenting

    OpenAIRE

    Sung Jin Jeon; Jae Ki Min; So Young Kwon; Jun Hyun Kim; Sun Young Moon; Kang Hoon Lee; Jeong Han Kim; Won Hyeok Choe; Young Koog Cheon; Tae Hyung Kim; Hee Sun Park

    2016-01-01

    Portal biliopathy is defined as abnormalities in the extra- and intrahepatic ducts and gallbladder of patients with portal hypertension. This condition is associated with extrahepatic venous obstruction and dilatation of the venous plexus of the common bile duct, resulting in mural irregularities and compression of the biliary tree. Most patients with portal biliopathy remain asymptomatic, but approximately 10% of them advance to symptomatic abdominal pain, jaundice, and fever. Magnetic reson...

  7. Environmental Factors in Primary Biliary Cirrhosis

    OpenAIRE

    Juran, Brian D.; Lazaridis, Konstantinos N.

    2014-01-01

    The etiology of the autoimmune liver disease primary biliary cirrhosis (PBC) remains largely unresolved, owing in large part to the complexity of interaction between environmental and genetic contributors underlying disease development. Observations of disease clustering, differences in geographical prevalence, and seasonality of diagnosis rates suggest the environmental component to PBC is strong, and epidemiological studies have consistently found cigarette smoking and history of urinary tr...

  8. Biliary Dysfunction in Children with Iron Deficiency

    Directory of Open Access Journals (Sweden)

    Yu.V. Marushko

    2016-04-01

    Full Text Available Background. Functional disorders of the biliary tract (FDBT remain a topical problem of pediatric gastroenterology due to the high incidence and progressive course with formation of chronic cholecystitis and cholelithiasis in patients. Iron deficiency (ID has a well-known effect on the course of the gastrointestinal tract pathology. With the aim of evaluating the state of the biliary system and clinical features of FDBT associated with ID, a case-control study was conducted in 160 children aged 9 to 17 years, who underwent in-patient treatment for exacerbation of biliary tract pathology. According to the iron metabolism tests, children were divided into 3 groups: I — 29 children with FDBT and I degree anemia; II — 91 children with FDBT and latent ID; III — 40 children with FDBT and normal iron metabolism parameters. Research methods included: the study of anamnesis, clinical examination, complete blood count, serum iron level, total iron-binding capacity, calculation of percent transferrin saturation, ultrasound of the abdominal organs and dynamic ultrasound cholecystography with choleretic breakfast. Results. The study found that children with FDBT and ID have a higher incidence of FDBT exacerbations, increased intensity of dyspeptic and asthenovegetative symptoms in case of FDBT exacerbation, increase in size, reduced contractile function of gallbladder (GB and hypotonic-hypokinetic FDBT type. Conclusions. Children with FDBT and ID have burdened course of FDBT due to the high incidence of exacerbations, severe dyspeptic and asthenovegetative symptoms in the acute phase, as well as reduced contractile function of GB and hypotonic-hypokinetic FDBT type, which is clinically unfavorable because of the risk of biliary sludge and cholelithiasis.

  9. Cephalosporin-associated biliary pseudolithiasis in children

    OpenAIRE

    A.E. Abaturov; N.Yu. Zavhorodnia

    2017-01-01

    The review presents current data about cephalosporin-associated biliary sludge and cholelithiasis formation mechanism. Risks factors of pseudolithiasis development in children, who received cephalosporins, are analyzed and systematized. Moreover, the study provides information about the prevalence of pseudolithiasis in different periods of childhood. Not only main ways of ceftriaxone-induced cholelithiasis clinical course, but also the most common complications of pseudolithiasis have been de...

  10. Cephalosporin-induced biliary sludge in children

    Directory of Open Access Journals (Sweden)

    A.E. Abaturov

    2017-09-01

    Full Text Available Background. One of the side effects of the third generation cephalosporins is the formation of cephalosporin-induced biliary pseudolithiasis (CIBPL. The purpose of the study was to establish the peculiarities of the formation of biliary sludge during the application of the third generation cephalosporins in the treatment of acute respiratory infections in children. Materials and methods. We examined 46 children aged 2 to 15 years with acute respiratory infections. Сephalosporins were administered intravenously in age doses. We evaluated the biochemical blood test and the echosonography (ultrasound of the biliary tract before the treatment and after the end of antibiotic therapy of all children. Results. According to ultrasound data, in 10 patients (21.7 % with acute respiratory infections, after a course of therapy with the third generation cephalosporins, heterogeneous contents were detected in the gallbladder lumen in the form of a hyperechoic suspension, which indicated the formation of CIBPL. The average age of patients with CIBPL was 9.05 ± 1.44 years and was within the range of 4 to 15 years. We found that in 60 % of the examined children, the formation of CIBPL proceeded subclinically. The results of a biochemical study of blood serum showed that in children with CIBPL, an increase in the activity of alanine aminotransferase, aspartate aminotransferase, and alkaline phosphatase was observed. It was found that CIBPL is detected in 22.9 % of children against the background of ceftriaxone therapy, in 9 % of patients — ceftazidime. Biliary sludge was formed in 54.5 % of patients receiving ceftriaxone in high doses (100 mg/kg/day, and in 75 % of cases after 10 days of using this antibiotic. Conclusions. The obtained results showed that against the background of using third generation cephalosporins in the treatment of acute respiratory infections, 21.7 % of children had CIBPL. A particularly high risk of developing CIBPL is observed when

  11. Intrahepatic biliary cystadenoma and cystadenocarcinoma: an experience of 30 cases.

    Science.gov (United States)

    Wang, Chu; Miao, Ruoyu; Liu, Huilin; Du, Xiaojing; Liu, Liguo; Lu, Xin; Zhao, Haitao

    2012-05-01

    Intrahepatic biliary cystadenoma and biliary cystadenocarcinoma are extremely rare neoplasms of the liver. They share similar radiological characteristics, and the clinicopathological features are poorly defined. We aim to provide an algorithm for preoperative differentiation of the two diseases. Patients who underwent liver resection between May 2001 and May 2011 at Peking Union Medical College Hospital with biliary cystadenoma (20 cases) and biliary cystadenocarcinoma (10 cases) were reviewed. Significant differences were shown in age (P=0.030), gender (P=0.002) and symptom duration (P=0.012). Most biliary cystadenomas occurred in women ≤60 years old (85%), whilst most biliary cystadenocarcinomas occurred in older males (50%). Shorter symptom duration indicated a higher risk of biliary cystadenocarcinoma. Arterial blood flow and wall/nodule enhancement tended to be more common in biliary cystadenocarcinoma, but the difference was not significant (P=0.348). A score system was developed. The case-by-case validation and leave-one-out cross-validation showed an accuracy of 95.5% and 90.9%, respectively. The discriminative accuracy for cases from another hospital during the same period was 90.9%. Older age, male gender, and shorter symptom duration are associated with higher possibility of biliary cystadenocarcinoma. Location and blood supply by radiology might be instrumental but still need further verification. Copyright © 2011 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  12. Biliary collateral veins and associated biliary abnormalities of portal hypertensive biliopathy in patients with cavernous transformation of portal vein.

    Science.gov (United States)

    Liu, Yubao; Hou, Baohua; Chen, Ren; Jin, Haosheng; Zhong, Xiaomei; Ye, Weitao; Liang, Changhong

    2015-01-01

    The purpose was to investigate magnetic resonance imaging (MRI) features of biliary collateral veins and associated biliary abnormalities of portal hypertensive biliopathy (PHB). Thirty-six patients including 18 patients with abnormal biliary changes and 18 patients as control group were involved in this study. MRI features of biliary collateral veins were analyzed. Stenosis with dilated proximal bile ducts occurred in 33.3% of patients, 27.8% of patients had irregular ductal walls, 22.2% of patients had thickened ductal walls, 16.7% of patients had angulated ductal walls, and 44.4% of patients had thickened gallbladder walls. Biliary collateral veins and associated biliary abnormalities of PHB can be detected by MRI. Copyright © 2015 Elsevier Inc. All rights reserved.

  13. A candidate gene for choanal atresia in alpaca.

    Science.gov (United States)

    Reed, Kent M; Bauer, Miranda M; Mendoza, Kristelle M; Armién, Aníbal G

    2010-03-01

    Choanal atresia (CA) is a common nasal craniofacial malformation in New World domestic camelids (alpaca and llama). CA results from abnormal development of the nasal passages and is especially debilitating to newborn crias. CA in camelids shares many of the clinical manifestations of a similar condition in humans (CHARGE syndrome). Herein we report on the regulatory gene CHD7 of alpaca, whose homologue in humans is most frequently associated with CHARGE. Sequence of the CHD7 coding region was obtained from a non-affected cria. The complete coding region was 9003 bp, corresponding to a translated amino acid sequence of 3000 aa. Additional genomic sequences corresponding to a significant portion of the CHD7 gene were identified and assembled from the 2x alpaca whole genome sequence, providing confirmatory sequence for much of the CHD7 coding region. The alpaca CHD7 mRNA sequence was 97.9% similar to the human sequence, with the greatest sequence difference being an insertion in exon 38 that results in a polyalanine repeat (A12). Polymorphism in this repeat was tested for association with CA in alpaca by cloning and sequencing the repeat from both affected and non-affected individuals. Variation in length of the poly-A repeat was not associated with CA. Complete sequencing of the CHD7 gene will be necessary to determine whether other mutations in CHD7 are the cause of CA in camelids.

  14. Is There a Right Ear Advantage in Congenital Aural Atresia?

    Science.gov (United States)

    Reed, Robert; Hubbard, Matthew; Kesser, Bradley W

    2016-12-01

    To compare speech/language development and academic progress between children with right versus left congenital aural atresia (CAA). Case control survey and review of audiometric data. Tertiary care academic practice. Children with unilateral CAA. Demographic and audiometric data; rates of grade retention, use of any hearing or learning resource, and behavioral problems. No significant differences in grade retention rate, utilization of amplification, speech language therapy, use of an individualized education program, or frequency modulated system were found between children with right versus left CAA. Children with left CAA were significantly more likely to be enrolled in special education programs (p = 0.026). Differences in reported communication problems approached significance with more difficulty noted in the right ear group (p = 0.059). Left CAA patients were also more likely to have reported behavioral problems (p = 0.0039). Contrary to the hypothesis that a normal hearing right ear confers a language advantage in patients with unilateral hearing loss, children with left CAA (normal right ear) were statistically more likely to be enrolled in a special education program and have behavioral problems. Reported communication problems were more common in right CAA patients, but this did not reach statistical significance. No differences were found in use of amplification, frequency modulated system, individualized education program, or grade retention. Further investigation of both the clinical implications and underlying psychoacoustics of unilateral hearing loss and the identification and habilitation of "at risk" unilateral hearing loss children is warranted.

  15. 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.

  16. 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.

  17. Readability and quality assessment of websites related to microtia and aural atresia.

    Science.gov (United States)

    Alamoudi, Uthman; Hong, Paul

    2015-02-01

    Many parents and children utilize the Internet for health-related information, but the quality of these websites can vary. The objective of this study was to assess the quality and readability of microtia and aural atresia related websites. The search engine Google was queried with the terms 'microtia' and 'aural atresia.' The first 30 results were evaluated, and those websites containing original information written in English were reviewed. Quality of content was assessed with the DISCERN instrument, and readability was assessed with the Flesch-Kincaid Reading Grade Level (FKGL) and the Flesch Reading Ease Score (FRES) tests. Each website was also reviewed for ownership and the date of last update. Sixteen microtia and 14 aural atresia websites were included for full review. The mean DISCERN score for microtia websites was 54.4 (SD=8.3), and for aural atresia websites it was 47.6 (SD=10.7), which indicates 'good' and 'fair' quality of content, respectively. Readability assessments showed an average reading level requiring a grade 10 education on FKGL, and only one microtia (6.3%) and one aural atresia (7.1%) websites were deemed to be at 'reasonable' reading level on FRES. High-quality websites that are considered easily comprehensible to the general public were lacking. Since parents and children may use websites when making treatment decisions, physicians should be aware of the quality of health information pertaining to their area of expertise available on the Internet. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  18. Endoscopic ultrasound-guided biliary drainage versus percutaneous transhepatic biliary drainage after failed endoscopic retrograde cholangiopancreatography: a meta-analysis

    OpenAIRE

    Baniya R; Upadhaya S; Madala S; Subedi SC; Shaik Mohammed T; Bachuwa G

    2017-01-01

    Ramkaji Baniya, Sunil Upadhaya, Seetharamprasad Madala, Subash Chandra Subedi, Tabrez Shaik Mohammed, Ghassan Bachuwa Hurley Medical Center, Michigan State University, Flint, MI, USA Abstract: The failure rate of endoscopic retrograde cholangiopancreatography for biliary cannulation is approximately 6%–7% in cases of obstructive jaundice. Percutaneous transhepatic biliary drainage (PTBD) is the procedure of choice in such cases. Endoscopic ultrasound-guided biliary drainage (EGBD) i...

  19. Decreased mortality but increased morbidity in neonates with jejunoileal atresia; a study of 114 cases over a 34-year period.

    NARCIS (Netherlands)

    Stollman, T.H.; Blaauw, I. de; Wijnen, M.H.W.A.; Staak, F.H.J.M. van der; Rieu, P.N.M.A.; Draaisma, J.M.T.; Wijnen, R.M.H.

    2009-01-01

    PURPOSE: The aim of the study was to evaluate patient demographics, classification and location of the atresia, operative management, postoperative care, and outcome in 114 infants with jejunoileal atresia (JIA) over a period of more than 3 decades. METHODS: This was a retrospective case series in a

  20. MÉTODO DE INDUÇÃO DE CIRROSE BILIAR SECUNDÁRIA COM USO DE PRÓTESE DE SILICONE METHOD FOR THE EXPERIMENTAL INDUCTION OF SECUNDARY BILIARY CIRRHOSIS IN WISTAR RATS

    Directory of Open Access Journals (Sweden)

    A. A. L. de Araujo Lima

    2001-01-01

    Full Text Available O objetivo deste experimento foi o desenvolvimento de um modelo de obstrução do ducto biliar comum através da interposição de uma prótese de silicone extrínseca ao ducto com única ligadura sem secção. Desenvolveu-se um modelo experimental alternativo, em ratos Wistar, que provoca a interrupção do fluxo bílio-duodenal com resultado satisfatório, pois houve distorção da arquitetura hepática, caracterizada por fibrose e proliferação ductal além de indicadores bioquímicos da colestase.

  1. A rare malformation: Double duodenal atresia associated with malrotation in a patient with “Cri du Chat” syndrome

    Directory of Open Access Journals (Sweden)

    Corina Zamfir

    2016-09-01

    Full Text Available Duodenal atresia is a common cause of neonatal obstruction. It is frequently associated with other malformations such as Down syndrome, cardiac anomalies, malrotation or annular pancreas. Double duodenal atresia is an exceptional malformation. There are only few publications on this subject and none are in association with “Cri du Chat” (Cat Cry syndrome. We present a newborn, prenatally diagnosed with duodenal atresia and with “Cri du Chat” syndrome. The double duodenal atresia was actually of two different types (type I and type II, associated with malrotation. The second atresia was a peroperative finding at reintervention, five days later. We wish to share our experience in order to avoid unnecessary surgery and co-morbidities.

  2. [Self-rated efficacy in bilateral aural atresia patients using bone-anchored hearing aid].

    Science.gov (United States)

    Yue, Fan; Yibei, Wang; Zhen, Wang; Pu, Wang; Xiaowei, Chen

    2015-03-01

    Using questionnaires to evaluate the audiological benefit and satisfaction of bilateral aural atresia patients with bone-anchored hearing aid (Baha). Implanted Baha user questionnaire was applied to 19 patients suffering bilateral aural atresia, and 15 of the patients were evaluated with abbreviated profile of hearing aid benefit (APHAB). Glasgow children's benefit inventory (GCBI) was used to measure subjective benefit of patients under the age of 18. The Baha user questionnaire demonstrated great satisfaction. The mean Baha scores for the subdomains of ease of communication (EC), backgroud noise (BN) and revereration (RV) were decreased by 54.6 ± 10.2 (t = 20.6, P Baha score was 56.7 ± 9.9 (t = 10.8, P Baha can significantly improve hearing ability and quality of life of patients with bilateral aural atresia.

  3. Simple Technique of Bridging Wide Gap in Esophageal Atresia with Tracheoesophageal Fistula - "Surgical Innovation".

    Science.gov (United States)

    Sharma, A K; Mangal, D

    2017-01-01

    The survival of the patients with esophageal atresia an tracheo esophageal fistula is believed to be an epitome of the success of the neonatal surgery. Restoring the continuty of the food pipe by esophagus to esophagus anastomosis is the best option. Preservation of natural esophagus by delayed repair in a wide gap esophageal atresia is a preferred technique worldwide, however such a management required prolonged hospitalization and dedicated nursing care, which is often not available in most of the centres in India. Esophageal substitutes in wide gap requires multiple operations and have long term problems, so remains the last option. I use the technique of oblique anastomosis which had distrinct advantage over circular anastomosis in the management of esophageal atresia1. This techniqe helps in bridging wide gap to some extent & minimal stricture formation.

  4. Primary biliary cirrhosis and scleroderma complicated by Barrett's ...

    African Journals Online (AJOL)

    1991-04-06

    Apr 6, 1991 ... Oesophageal problems are common in patients with sclero- derma. but the association of primary biliary cirrhosis and scleroderma is uncommon. A Barrett's oesophagus identified in a patient with primary biliary cirrhosis and scleroderma is described. The Barrett's oesophagus was probably a compli-.

  5. Primary biliary cirrhosis and scleroderma complicated by Barrett's ...

    African Journals Online (AJOL)

    Oesophageal problems are common in patients with scleroderma. but the association of primary biliary cirrhosis and scleroderma is uncommon. A Barrett's oesophagus identified in a patient with primary biliary cirrhosis and scleroderma is described. The Barrett's oesophagus was probably a complication of scleroderma ...

  6. Percutaneous transhepatic biliary drainage in malignant extrahepatic cholestasis

    Energy Technology Data Exchange (ETDEWEB)

    Hoevels, J.

    1985-03-01

    The technique of non-surgical percutaneous transhepatic biliary drainage has been improved recently. Combined internal and external drainage of bile was enabled by manipulation of a guide wire and a drainage catheter through the stenosed or obstructed section of the extrahepatic biliary ducts. Experience have been gained concerning internal drainage of bile by percutaneous transhepatic insertion of an endoprosthesis for some years now.

  7. Percutaneous management of tumoral biliary obstruction in children

    Energy Technology Data Exchange (ETDEWEB)

    Akinci, Devrim; Gumus, Burcak; Ozkan, Orhan S.; Ozmen, Mustafa N.; Akhan, Okan [Hacettepe School of Medicine, Department of Radiology, Sihhiye, Ankara (Turkey); Ekinci, Saniye [Hacettepe School of Medicine, Department of Paediatric Surgery, Sihhiye, Ankara (Turkey); Akcoren, Zuhal [Hacettepe School of Medicine, Department of Paediatric Pathology, Sihhiye, Ankara (Turkey); Kutluk, Tezer [Hacettepe School of Medicine, Department of Paediatric Oncology, Sihhiye, Ankara (Turkey)

    2007-10-15

    There is limited experience of percutaneous biliary interventions in children although they are safe and effective procedures. To evaluate the efficacy and safety of percutaneous management of tumoral biliary obstruction in children. Percutaneous biliary interventions were performed in eight children (six boys, two girls) with a mean age of 10.5 years (range 4-17 years). The interventions included percutaneous biliary drainage (five patients), percutaneous biliary drainage and placement of a self-expanding metallic stent (two patients), and percutaneous cholecystostomy (one patient). All patients had signs of obstructive jaundice and two had cholangitis. All procedures were successful. No procedure-related mortality was observed. Bilirubin levels returned to normal in four of the eight patients. Findings of cholangitis resolved in the two affected patients after the procedure and antibiotic treatment. Two patients underwent surgery after percutaneous biliary drainage procedures. A self-expanding metallic stent was placed in two patients with malignancy and the stents remained patent until death. Percutaneous biliary interventions can be performed safely for the management of tumoral biliary obstruction in children. (orig.)

  8. Extrahepatic biliary cystadenoma: an unusual cause of recurrent cholangitis.

    Science.gov (United States)

    Ray, Sukanta; Khamrui, Sujan; Mridha, Asit Ranjan; Mukherjee, Biswarup

    2010-01-01

    Extrahepatic biliary cystadenoma is a rare benign lesion with malignant potential. Fewer than 100 cases have been reported in the literature. The most common clinical presentation is obstructive jaundice. Frank cholangitis is extremely rare. The authors report a case of extrahepatic biliary cystadenoma in a 55-year-old woman who presented with recurrent episodes of cholangitis.

  9. Biliary cystadenoma of the left intrahepatic duct (2007: 2b)

    NARCIS (Netherlands)

    Seidel, R.; Weinrich, M.; Pistorius, G.; Fries, P.; Schneider, G.

    2007-01-01

    Biliary cystadenoma is a rare epithelial cystic neoplasm representing only 5% of intrahepatic cystic lesions of biliary origin. Commonly, the lesions are solitary cystic structures with multiple thin-walled septa predominantly arising from the right hepatic duct. Although the lesions are generally

  10. Indocyanine-green-loaded microballoons for biliary imaging in cholecystectomy

    Science.gov (United States)

    Mitra, Kinshuk; Melvin, James; Chang, Shufang; Park, Kyoungjin; Yilmaz, Alper; Melvin, Scott; Xu, Ronald X.

    2012-11-01

    We encapsulate indocyanine green (ICG) in poly[(D,L-lactide-co-glycolide)-co-PEG] diblock (PLGA-PEG) microballoons for real-time fluorescence and hyperspectral imaging of biliary anatomy. ICG-loaded microballoons show superior fluorescence characteristics and slower degradation in comparison with pure ICG. The use of ICG-loaded microballoons in biliary imaging is demonstrated in both biliary-simulating phantoms and an ex vivo tissue model. The biliary-simulating phantoms are prepared by embedding ICG-loaded microballoons in agar gel and imaged by a fluorescence imaging module in a Da Vinci surgical robot. The ex vivo model consists of liver, gallbladder, common bile duct, and part of the duodenum freshly dissected from a domestic swine. After ICG-loaded microballoons are injected into the gallbladder, the biliary structure is imaged by both hyperspectral and fluorescence imaging modalities. Advanced spectral analysis and image processing algorithms are developed to classify the tissue types and identify the biliary anatomy. While fluorescence imaging provides dynamic information of movement and flow in the surgical region of interest, data from hyperspectral imaging allow for rapid identification of the bile duct and safe exclusion of any contaminant fluorescence from tissue not part of the biliary anatomy. Our experiments demonstrate the technical feasibility of using ICG-loaded microballoons for biliary imaging in cholecystectomy.

  11. Risk Factors Associated with Biliary Pancreatitis in Children

    Science.gov (United States)

    Ma, Michael H.; Bai, Harrison X.; Park, Alexander J.; Latif, Sahibzada U.; Mistry, Pramod K.; Pashankar, Dinesh; Northrup, Veronika S.; Bhandari, Vineet; Husain, Sohail Z.

    2012-01-01

    Little is known about risk factors for biliary pancreatitis in children. Objectives We characterized cases of pediatric biliary pancreatitis, compared biliary with non-biliary cases, examined differences in presentation between younger and older children, and studied features distinguishing gallstone- from sludge-induced pancreatitis. Methods We evaluated 76 episodes of biliary pancreatitis from 271 cases of acute pancreatitis in children admitted to a tertiary care hospital from 1994 to 2007. Results Of the 76 cases, 55% had gallstones, 21% had sludge, and 24% had structural defects. Hispanic children had a 2.85 (p=0.01) and 5.59 (p=0.003) higher probability for biliary pancreatitis than white and black children, respectively. Median serum amylase and lipase in children with biliary pancreatitis were 64% and 49% higher, respectively, compared to other etiologies (ppancreatitis (OR=6.69, p=0.001). When comparing gallstone- with sludge-induced etiologies, obesity was an independent predictor (38% more prevalent, ppancreatitis over other etiologies. Furthermore, obesity can distinguish gallstone- from sludge-induced pancreatitis. These findings may spur prospective studies to determine the optimal evaluation and management of children with biliary pancreatitis. PMID:22002481

  12. Missed diagnosis of atresia of the right pulmonary artery in woman with left-sided pneumothorax

    DEFF Research Database (Denmark)

    Dagnegård, Hanna; Ryom, Philip

    2016-01-01

    Isolated pulmonary atresia is an uncommon condition, which can go undiagnosed for a long time in asymptomatic patients. Sometimes, diagnosis can be made at pregnancy due to respiratory symptoms. There is no known increased risk of pneumothorax. We here present a case where a second-time pregnant...... woman with an unknown atresia of the right pulmonary artery received a left-sided pneumothorax. The diagnosis was initially missed in spite of adequate imaging and the condition progressed to respiratory stop. We describe the course of diagnostics and the chosen strategy of treatment....

  13. 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.

  14. Reverse gastric tube oesophageal substitution for staged repair of oesophageal atresia and tracheo-oesophageal fistula

    Directory of Open Access Journals (Sweden)

    Christopher Olusanjo Bode

    2014-01-01

    Full Text Available The management of oesophageal atresia and tracheo-oesophageal atresia (OATOF is very challenging. While in developed countries survival of patients with this condition has improved, the outcome in many developing countries has been poor. Primary repair through a thoracotomy (or video-assisted thoracoscopic surgery where available is the gold standard treatment of OATOF. However, in our setting where patients typically present late and with minimum support resources such as Neonatal Intensive Care Unit and total parenteral nutrition; staged repair may be the only hope of survival of these patients and this communication highlights the essential steps of this mode of treatment.

  15. Portal Hypertensive Biliopathy: An Infrequent Cause of Biliary Obstruction.

    Science.gov (United States)

    Cardoso, Ricardo; Casela, Adriano; Lopes, Sandra; Agostinho, Cláudia; Souto, Paulo; Camacho, Ernestina; Almeida, Nuno; Mendes, Sofia; Gomes, Dário; Sofia, Carlos

    2015-01-01

    Biliary obstruction is usually caused by choledocholithiasis. However, in some circumstances, alternative or concurring unusual ethiologies such as portal hypertensive biliopathy (PHB) must be considered. We present the case of a 36-year-old female complaining of jaundice and pruritus. Liver function tests were compatible with biliary obstruction and the ultrasound scan of the abdomen showed dilatation of the intrahepatic biliary ducts, a dilated common bile duct (CBD) and biliary calculi. The computed tomography of the abdomen revealed a portal cavernoma encasing the CBD. Portal cavernoma, the hallmark of extrahepatic portal venous obstruction, can cause PHB. When symptomatic, chronic cholestasis is present if a dominant stricture exists whereas biliary pain and acute cholangitis occur when choledocholithiasis prevails. Management must be individualized and usually includes endoscopic therapy to address choledocholithiasis and shunt surgery for definitive treatment.

  16. Antimitochondrial antibody-negative primary biliary cirrhosis.

    Science.gov (United States)

    Mendes, Flavia; Lindor, Keith D

    2008-06-01

    There is a subset of patients who have biochemical and histologic features consistent with primary biliary cirrhosis (PBC) who lack antimitochondrial antibodies (AMA). This entity is usually referred to as AMA-negative PBC or alternatively autoimmune cholangitis. Patients who have AMA-negative PBC are believed to have a similar clinical course, response to treatment, and prognosis as their AMA-positive counterparts. As more sensitive and specific serologic tests are developed to detect serum AMA, it is possible we may find that these patients initially believed to be AMA-negative are indeed AMA-positive, suggesting a single disease process.

  17. Primary biliary cirrhosis accompanied by CREST syndrome.

    Science.gov (United States)

    Kouraklis, Gregory; Glinavou, Andromahi; Karatzas, Gabriel

    2002-09-01

    CREST syndrome, a relatively benign, slowly progressive variant of systemic scleroderma consists of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. Although the association of this syndrome with primary biliary cirrhosis (PBC) is recognized in women, it has never been described in a man. We report the rare case of a male patient with CREST syndrome accompanied by PBC, manifested by acute cholecystitis and mild jaundice. The association of the two conditions is clinically and etiologically important. Clinicians must be aware of this association, sincethe clinical features of CREST syndrome may be mild and may be thought to be complications of the underlying liver disease.

  18. Molecular Identification of Biliary Isospora Belli

    Science.gov (United States)

    Chiu, King-Wah; Chiou, Shue-Shian; Lu, Lung-Sheng; Wu, Cheng-Kun; Eng, Hock-Liew

    2016-01-01

    Abstract This report describes the novel sampling of bile from the biliary endoscopic intervention for the molecular identification of parasite infection. A 63-year-old Vietnamese man underwent travel health examination in our hospital. Physical examination showed that his height was 159 cm and weight was 41 kg. He had a 15-year history of intermittent abdominal pain and frequent episodes of diarrhea. Laboratory tests revealed raised eosinophil count (23%, normal range [NR] 0–5), absolute eosinophil count (1899/μL, NR 50–350), and levels of serum immunoglobulin E (3770 IU/mL, NR Isospora belli (IB) infection was identified by a polymerase chain reaction. Trimethoprim–sulfamethoxazole 800 mg q6h was administered for 1 month. Liver enzyme levels normalized and negative for concentration method of ova study. The patient was doing well and weighed 51 kg at the outpatient clinic visit 3 months later. This bile sampling with molecular identification has not been described in the literature. We believe that an acute IB infection through fecal-oral transmission may progress to chronic infection of the hepatobiliary system, leading to biliary obstruction and jaundice. PMID:26962840

  19. [Biliary lithiasis in childhood: therapeutic approaches].

    Science.gov (United States)

    Escobar Castro, H; García Novo, Ma D; Olivares, P

    2004-02-01

    Until recently, biliary lithiasis was considered infrequent in childhood. According to their composition, gallstones can be classified into cholesterol stones and pigment stones. The latter are mainly composed of calcium salts of unconjugated bilirubin and are divided into hard black and soft brown stones. In children, up to 75 % of gallstones are pigment stones. Their etiology is often unknown. Biliary lithiasis in children differs from that in adults and there is very little scientific evidence on the most suitable therapeutic procedures. Symptom-free stones usually have a benign course and do not require medical or surgical treatment. Symptoms are often nonspecific and include dyspepsia and chronic abdominal pain. These symptoms are an indication for ultrasonographic scan to rule out the presence of gallstones. Cholecystectomy is the definitive treatment for gallstones but is not always indicated. Medical treatment with ursodeoxycholic acid is indicated in oligosymptomatic and asymptomatic lithiasis with transparent, soft, cholesterol-rich stones and a functional bladder and in patients with a high surgical risk.

  20. Pyloric atresia: A report of ten patients | Ksia | African Journal of ...

    African Journals Online (AJOL)

    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, ...

  1. MicroRNAs: New Insight in Modulating Follicular Atresia: A Review

    Directory of Open Access Journals (Sweden)

    Tesfaye Worku

    2017-02-01

    Full Text Available Our understanding of the post-transcriptional mechanisms involved in follicular atresia is limited; however, an important development has been made in understanding the biological regulatory networks responsible for mediating follicular atresia. MicroRNAs have come to be seen as a key regulatory actor in determining cell fate in a wide range of tissues in normal and pathological processes. Profiling studies of miRNAs during follicular atresia and development have identified several putative miRNAs enriched in apoptosis signaling pathways. Subsequent in vitro and/or in vivo studies of granulosa cells have elucidated the functional role of some miRNAs along with their molecular pathways. In particular, the regulatory roles of some miRNAs have been consistently observed during studies of follicular cellular apoptosis. Continued work should gradually lead to better understanding of the role of miRNAs in this field. Ultimately, we expect this understanding will have substantial benefits for fertility management at both the in vivo or/and in vitro levels. The stable nature of miRNA holds remarkable promise in clinical use as a diagnostic tool and in reproductive medicine to solve the ever-increasing fertility problem. In this review, we summarize current knowledge of the involvement of miRNAs in follicular atresia, discuss the challenges for further work and pinpoint areas for future research.

  2. A newborn with duodenal atresia and a gastric perforation | Akçora ...

    African Journals Online (AJOL)

    Congenital duodenal atresia complicated by gastric perforation (GP) is a very rare and a very mortal condition. Only three newborns could be cured in the reported 13 cases. We report a successfully treated newborn with this complicated disease. A 2-day-old male was hospitalized with prediagnosis of duodenal obstruction.

  3. Rare congenital absence of tail (anury) and anus (atresia ani) in ...

    African Journals Online (AJOL)

    On examination it was found that the calf had no tail, the posterior sacral margin was blunt and the anal opening was absent. The case was diagnosed as congenital anury with atresia ani. The animal was sedated with 0.1 mg/kg of xylazine administered intramuscularly and under local infiltration with 2% lidocaine a circular ...

  4. Jejuno-ileal atresia: A 2-year preliminary study on presentation and ...

    African Journals Online (AJOL)

    Three babies died giving a mortality of 33.3%. Mortality is commoner in types IIIb and IV. Conclusion: Mortality is higher in complex atresia which most times will require neonatal intensive care and parenteral nutrition facilities. These are still lacking in our institution. Providing these facilities will further improve outcome.

  5. The BAHA Softband. A new treatment for young children with bilateral congenital aural atresia.

    NARCIS (Netherlands)

    Hol, M.K.S.; Cremers, C.W.R.J.; Coppens-Schellekens, W.; Snik, A.F.M.

    2005-01-01

    OBJECTIVE: To evaluate the validity of a bone-anchored hearing aid (BAHA) Softband (fitted unilaterally and bilaterally) in young children with bilateral congenital aural atresia. SUBJECTS: Two children with severe bilateral congenital conductive hearing loss, who had been fitted with a

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

    NARCIS (Netherlands)

    F.W.T. Vergouwe (Floor); H. IJsselstijn (Hanneke); R.M.H. Wijnen (René); M.J. Bruno (Marco); 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

  7. [Digestive associated malformations in esophageal atresia. A study of 125 patients (author's transl)].

    Science.gov (United States)

    Bachiller, M C; Jaureguizar, E; Martínez, A; Tovar, J A; Díez Pardo, J A; Monereo, J

    1976-01-01

    The incidence of digestive malformations associated to esophageal atresia and tracheoesophageal fistula is reviewed in a series of 125 surgically treated patients during the last five years' period. A correlation is drawn with other associated malformations. Its influence upon the mortality rate is stressed.

  8. Definition of a critical region on chromosome 18 for congenital aural atresia by arrayCGH.

    NARCIS (Netherlands)

    Veltman, J.A.; Jonkers, Y.M.H.; Nuijten, I.; Janssen, I.M.; Vliet, W. van der; Huys, E.; Vermeesch, J.; Buggenhout, G.J.C.M. van; Fryns, J.P.; Admiraal, R.J.C.; Terhal, P.; Lacombe, D.; Geurts van Kessel, A.H.M.; Smeets, D.F.C.M.; Schoenmakers, E.F.P.M.; Ravenswaaij-Arts, C.M.A. van

    2003-01-01

    Deletions of the long arm of chromosome 18 occur in approximately 1 in 10,000 live births. Congenital aural atresia (CAA), or narrow external auditory canals, occurs in approximately 66% of all patients who have a terminal deletion 18q. The present report describes a series of 20 patients with CAA,

  9. Definition of a critical region on chromosome 18 for congenital aural atresia by arrayCGH

    NARCIS (Netherlands)

    Veltman, JA; Jonkers, Y; Nuijten, [No Value; Janssen, [No Value; van der Vliet, W; Huys, E; Vermeesch, J; Van Buggenhout, G; Fryns, JP; Admiraal, R; Terhal, P; Lacombe, D; van Kessel, AG; Smeets, D; Schoenmakers, EFPM; van Ravenswaaij-Arts, CM

    Deletions of the long arm of chromosome 18 occur in similar to1 in 10,000 live births. Congenital aural atresia (CAA), or narrow external auditory canals, occurs in similar to66% of all patients who have a terminal deletion 18q. The present report describes a series of 20 patients with CAA, of whom

  10. Respiratory morbidity and growth after open thoracotomy or thoracoscopic repair of esophageal atresia

    NARCIS (Netherlands)

    Spoel, M.; Meeussen, C.J.; Gischler, S.J.; Hop, W.C.J.; Bax, N.M.A.; Wijnen, R.M.H.; Tibboel, D.; Jongste, J.C. de; Ijsselstijn, H.

    2012-01-01

    BACKGROUND: Respiratory morbidity has been described in patients who underwent repair of esophageal atresia as a neonate. We compared the influence of open thoracotomy or thoracoscopy on lung function, respiratory symptoms, and growth. METHODS: Functional residual capacity (FRC(p)), indicative of

  11. Tricuspid Atresia with Normal Axis on ECG Palliated With A Central ...

    African Journals Online (AJOL)

    Background: Tricuspid Atresia is the 3rd commonest cyanotic congenital Heart disease .It is characterized by lack of communication between the right atrium and right ventricle. ... Result: Following resuscitation, a central shunt (Ascending Aorta to main Pulmonary Artery) was constructed and the patient did well despite a ...

  12. 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.

  13. 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

    In the present study, 307 ovaries of eastern Baltic cod Gadus morhua callarias sampled during the prespawning and spawning season 2000 were analysed histologically to estimate the seasonal prevalence and intensity of atresia. The number of atretic oocytes per ovary was estimated using a combination...

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

    Science.gov (United States)

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

    2004-01-01

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

  15. 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

  16. Pre-operative biliary drainage for obstructive jaundice

    Science.gov (United States)

    Fang, Yuan; Gurusamy, Kurinchi Selvan; Wang, Qin; Davidson, Brian R; Lin, He; Xie, Xiaodong; Wang, Chaohua

    2014-01-01

    Background Patients with obstructive jaundice have various pathophysiological changes that affect the liver, kidney, heart, and the immune system. There is considerable controversy as to whether temporary relief of biliary obstruction prior to major definitive surgery (pre-operative biliary drainage) is of any benefit to the patient. Objectives To assess the benefits and harms of pre-operative biliary drainage versus no pre-operative biliary drainage (direct surgery) in patients with obstructive jaundice (irrespective of a benign or malignant cause). Search methods We searched the Cochrane Hepato-Biliary Group Controlled Trials Register, Cochrane Central Register of Controlled Clinical Trials (CENTRAL) in The Cochrane Library, MEDLINE, EMBASE, and Science Citation Index Expanded until February 2012. Selection criteria We included all randomised clinical trials comparing biliary drainage followed by surgery versus direct surgery, performed for obstructive jaundice, irrespective of the sample size, language, and publication status. Data collection and analysis Two authors independently assessed trials for inclusion and extracted data. We calculated the risk ratio (RR), rate ratio (RaR), or mean difference (MD) with 95% confidence intervals (CI) based on the available patient analyses. We assessed the risk of bias (systematic overestimation of benefit or systematic underestimation of harm) with components of the Cochrane risk of bias tool. We assessed the risk of play of chance (random errors) with trial sequential analysis. Main results We included six trials with 520 patients comparing pre-operative biliary drainage (265 patients) versus no pre-operative biliary drainage (255 patients). Four trials used percutaneous transhepatic biliary drainage and two trials used endoscopic sphincterotomy and stenting as the method of pre-operative biliary drainage. The risk of bias was high in all trials. The proportion of patients with malignant obstruction varied between 60

  17. Surgery or implantable hearing devices in children with congenital aural atresia: 25 years of our experience.

    Science.gov (United States)

    Jovankovičová, Andrea; Staník, Roman; Kunzo, Samuel; Majáková, Lucia; Profant, Milan

    2015-07-01

    Congenital aural atresia and ear deformities have been the subject of serious discussions for centuries. These malformations are associated with significant aesthetic and functional problems. Outcome of the surgical solution is rarely optimal. Despite the gradual improvement of surgical techniques the surgery still remains associated with very limited short-term and mainly long-term functional outcome. Therefore, the priority treatment in modern otology becomes implantable devices--BAHA, Bonebridge and active middle ear implants. The functional and aesthetic outcomes of aural atresia reconstruction performed at Pediatric ENT Department of Children's University Hospital were retrospectively evaluated and compared with the results prospectively obtained from implantable hearing devices (BAHA, Vibrant Soundbridge, Bonebridge), which have been implanted in patients with aural atresia at Department of ORL HNS, University Hospital Bratislava. Aural atresia reconstruction has been performed in 34 patients during last 25 years. Results of the surgery could be viewed as excellent only in three patients (gain above 30 dB). Air conduction threshold has decreased after the surgery in seven patients, and in two cases total deafness occurred after the surgery. Patients gain on average 12 dB in auditory threshold after surgery. Hearing devices were implanted to the group of 11 children in order to improve their hearing. All of them were the patients with bilateral aural atresia. After implantation a significant improvement in hearing threshold occurred in all children (30-35 dB on average). Together with results of air conduction threshold in patient with aural atresia before and after surgery and implantation we also present a standard deviation. The functional outcome of implantable hearing devices in patients with bilateral aural atresia clearly dominates over the traditional reconstructive surgery. Aesthetic results in pinna deformity management remain a major concern for

  18. Biliary sequelae following radioembolization with Yttrium-90 microspheres.

    Science.gov (United States)

    Atassi, Bassel; Bangash, Affaan K; Lewandowski, Robert J; Ibrahim, Saad; Kulik, Laura; Mulcahy, Mary F; Murthy, Ravi; Ryu, Robert K; Sato, Kent T; Miller, Frank H; Omary, Reed A; Salem, Riad

    2008-05-01

    Yttrium-90 (90Y) radioembolization has emerged as a promising and safe therapeutic modality for patients with hepatocellular carcinoma (HCC) or metastatic liver cancer. The present report describes biliary sequelae following intraarterial 90Y therapy in patients with HCC or liver metastases. All patients were treated with 90Y therapy according to standard lobar treatment protocol. Pre- and posttreatment imaging, liver function tests, and serum total bilirubin measurements were performed. Three to 6 months after treatment, biliary sequelae were evaluated with computed tomography and magnetic resonance imaging, and any liver-related laboratory adverse events were noted. A total of 327 patients (HCC, n=190; liver metastases, n=137) received 569 infusions of 90Y. At follow-up imaging, 33 patients (10.1%; liver metastases, n=26; HCC, n=7) had 40 imaging findings related to the biliary tree, including biliary necrosis (n=17), biloma (n=3), cholecystitis (n=2), gallbladder wall enhancement (n=6), gallbladder wall rent (n=3), abscess (n=1), and stricture (n=8). A total of 31 patients exhibited grade 3/4 bilirubin toxicities (13 [6.8%] with HCC, 18 [13.1%] with liver metastases). Unplanned interventions prompted by biliary sequelae were necessary in six of 327 patients (1.8%). 90Y therapy in patients with HCC or metastatic disease to the liver is associated with an acceptable rate of biliary toxicities. Further studies assessing long-term biliary sequelae are warranted.

  19. Surgical treatment of biliary tract complications after liver transplantation.

    Science.gov (United States)

    Lladó, L; Fabregat, J; Baliellas, C; Gonzalez-Castillo, A; Ramos, E; Gonzalez-Vilatarsana, E; Torras, J; Rafecas, A

    2012-01-01

    Biliary strictures are the most common biliary tract complication after liver transplantation. There are scarce data on the results of hepaticojejunostomy (HJ) in the management of biliary complications after orthotopic liver transplantation (OLT). Thus, the role of surgery in this setting remains to be established. The aim of this study was to evaluate the results of surgical treatment of patients with biliary complications at our institution. We reviewed 1000 consecutive liver transplantations performed at our institution from 1984 to 2007. We used a prospectively recorded database to identify patients who underwent HJ to treat any biliary tract complication. Overall, 62 patients (6.2%) underwent HJ, 40 for an anastomotic and 7 for a non-anastomotic stricture as well as 15 for biliary leaks. Postoperative morbidity was 16%, and postoperative mortality 1.6%. There were 7 cases of anastomotic stenosis (11.3%). Four patients (5%) required retransplantation. HJ is a safe procedure to manage biliary complications after OLT. It may be the first treatment choice especially for cases with anastomotic strictures. Copyright © 2012 Elsevier Inc. All rights reserved.

  20. High-dose-rate afterloading intracavitary irradiation and expandable metallic biliary endoprosthesis for malignant biliary obstruction

    Energy Technology Data Exchange (ETDEWEB)

    Yoshimura, Hitoshi; Ohishi, Hajime; Yoshioka, Tetsuya (Nara Medical Univ., Kashihara (Japan)) (and others)

    1989-04-01

    A double lumen catheter was developed as an applicator for the remote afterloading system (RALS) of {sup 60}Co for the intracavitary irradiation of an obstructed common bile duct due to gallbladder cancer in 1 case and by cholangiocarcinoma in 7 cases. This was followed by the biliary endoprosthesis with expandable metallic stents to maintain patency. The mean survival period after treatment was not long (14 weeks). However, removal of the external drainage tube was possible in 7 of the 8 cases, and none of the 8 cases showed dislodgement or deformity of the stent, or obstruction of the bile duct in the stent-inserted area. This combination effectively provided palliation, and has considerable potential for malignant biliary obstruction. (author).

  1. Biliary excretion of intravenous (/sup 14/C) omeprazole in humans

    Energy Technology Data Exchange (ETDEWEB)

    Lind, T.; Andersson, T.; Skanberg, I.O.; Olbe, L.

    1987-11-01

    We have studied the biliary excretion of (/sup 14/C) omeprazole in humans. The study was performed in eight healthy subjects and the technique used was based on multiple marker dilution principles with double-lumen tubes placed in both the stomach and intestine. The results obtained show a 16% biliary excretion of (/sup 14/C) omeprazole. These data suggest a minimal spillover of omeprazole from the gastric mucosa into the gastric lumen in humans. The results also agree with previous data of the fecal recovery of radiolabeled omeprazole that suggest that the fecal excretion of intravenous omeprazole in humans is entirely accounted for by biliary excretion.

  2. Recent advances in endoscopic ultrasonography-guided biliary interventions

    Science.gov (United States)

    Kawakubo, Kazumichi; Kawakami, Hiroshi; Kuwatani, Masaki; Haba, Shin; Kawahata, Shuhei; Abe, Yoko; Kubota, Yoshimasa; Kubo, Kimitoshi; Isayama, Hiroyuki; Sakamoto, Naoya

    2015-01-01

    Interventional endoscopic ultrasonography (EUS) based on EUS-guided fine-needle aspiration has rapidly spread as a minimally invasive procedure. Especially in patients with failed endoscopic retrograde cholangiopancreatography, EUS-guided biliary intervention is reported to be useful as salvage therapy. EUS-guided biliary interventions are carried out using three techniques: EUS-guided bilioenteric anastomosis, EUS-guided rendezvous procedure, and EUS-guided antegrade treatment. Although interventional EUS is not yet a standardized procedure, there have been recent advances in this field that address various biliary diseases. Here, we summarize the indications, techniques, clinical results of previous studies, and future perspectives. PMID:26327757

  3. Risk and Surveillance of Cancers in Primary Biliary Tract Disease

    Directory of Open Access Journals (Sweden)

    Valery Hrad

    2016-01-01

    Full Text Available Primary biliary diseases have been associated in several studies with various malignancies. Understanding the risk and optimizing surveillance strategy of these malignancies in this specific subset of patients are an important facet of clinical care. For instance, primary sclerosing cholangitis is associated with an increased risk for cholangiocarcinoma (which is very challenging to diagnose and when IBD is present for colorectal cancer. On the other hand, primary biliary cirrhosis patients with cirrhosis or not responding to 12 months of ursodeoxycholic acid therapy are at increased risk of hepatocellular carcinoma. In this review we will discuss in detail the risks and optimal surveillance strategies for patients with primary biliary diseases.

  4. [Biliary endoprosthesis causing ileal perforation--a case report].

    Science.gov (United States)

    Kosiński, Robert; Olędzki, Szymon; Modrzejewski, Andrzej

    2015-10-01

    We experienced ileal perforation caused by dislocated biliary endoprosthesis in 59 years old female patient. The endoprosthesis was implanted due to biliary fistula after laparoscopic cholecystectomy 2 years before the perforation. It seems that endoprosthesis dislocation and the perforation were the result of too long stay of endoprosthesis. After the surgical management and the removal of the prosthesis patient was cured. Although ileal perforation caused by dislocated biliary endoprosthesis is rare, clinicians should be aware of the possibility of its occurrence. © 2015 MEDPRESS.

  5. Biliary Lithiasis: Prevalence and Ultrasound Profile in a Service Hospital

    OpenAIRE

    Debnath, J; Chakraborty, I; Mohan, R

    2003-01-01

    Real time gray scale ultrasonography of upper abdomen was carried out in 1237 cases for varied indications. Overall prevalence of biliary lithiasis was 11.56% with female to male ratio of 4:1. Fifty years and below, female to male ratio was 6.5:1. 88.8% cases of biliary lithiasis were 60 years and below. Gall bladder dyspepsia (61.5%) and right upper quadrant pain (41%) were the main presenting features of biliary lithiasis. 26 patients (18%) presented with acute abdomen. Asymptomatic gallsto...

  6. Biliary Surgery Via Minilaparotomy — A Limited Procedure for Biliary Lithiasis

    OpenAIRE

    Takukazu Nagakawa

    1993-01-01

    Cholelithiasis until now has been treated using solvents, lithotripsy via a biliary endoscope, laser or shock wave lithotripsy, and laparoscopic cholecystectomy. have developed a new surgical treatment for cholelithiasis in which a cholecystectomy is performed through a minilaparotomy. This paper presents this new technique and discusses the principles of surgery for cholelithiasis using this technique. This procedure is performed by a 2 to 3 cm subcostal skin incision in the right hypochondr...

  7. Expanding endourology for biliary stone disease: the efficacy of intracorporeal lithotripsy on refractory biliary calculi.

    Science.gov (United States)

    Sninsky, Brian C; Sehgal, Priyanka D; Hinshaw, J Louis; McDermott, John C; Nakada, Stephen Y

    2014-07-01

    We evaluated the efficacy of ureteroscopic therapy (electrohydraulic lithotripsy [EHL] and intraductal laser lithotripsy [ILL]) in patients with challenging biliary stones secondary to anatomic variations resulting from a previous surgical procedure, including liver transplantation. A retrospective chart review was performed for all patients with previous surgical alteration of the gastrointestinal (GI) tract who underwent EHL or ILL via peroral or percutaneous access for choledocholithiasis by a single surgeon at our institution from 2000 to 2012. A database containing clinical and surgical variables was created, and long-term follow-up was conducted (3-138 months; median, 99 months). Thirteen patients (51.7±20.0 years; M:F, 10:3) in whom endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic cholangiography (PTHC), or both failed were identified. Failure of ERCP/PTHC was because of inaccessibility of the calculi in all cases. Stone clearance was achieved in 12/13 (93%) patients; 8/12 (62%) after one procedure, and 4/12 (31%) after two procedures. One patient with biliary cast syndrome needed four interventions over 9 years. Major complications were low, with only one patient with hypotension and cholangitis that resolved with 24 hours of administration of intravenous fluids and antibiotics. Both endoscopic and percutaneous lithotripsies are effective treatments for refractory biliary calculi resulting from the post-surgical GI tract. Although a staged second procedure may be necessary in patients with significant stone burden, this is significantly better than extensive open surgery.

  8. Extrahepatic Manifestations of Primary Biliary Cholangitis.

    Science.gov (United States)

    Chalifoux, Sara L; Konyn, Peter G; Choi, Gina; Saab, Sammy

    2017-11-15

    Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogren's syndrome, thyroid dysfunction and systemic sclerosis. It is thought that patients with PBC are at increased risk of developing these extrahepatic manifestations, almost all of which are autoimmune, because patients with autoimmune disease are at higher risk of developing another autoimmune condition. Due to the high prevalence of extrahepatic diseases in patients with PBC, it is important to complete a thorough medical history at the time of diagnosis. Prompt recognition of extrahepatic disease can lead to improved patient outcomes and quality of life. The following review summarizes the most common extrahepatic conditions associated with PBC.

  9. Novel therapeutic targets for primary biliary cholangitis

    Directory of Open Access Journals (Sweden)

    CHEN Rongbin

    2017-05-01

    Full Text Available Primary biliary cholangitis (PBC is a chronic autoimmune-mediated liver disease manifesting as progressive cholestasis and non-purulent inflammation in small- and medium-sized intrahepatic bile ducts. It finally progresses to liver cirrhosis and liver cancer and greatly threatens patient's life. Studies have found that ursodeoxycholic acid (UDCA can treat PBC effectively. There is a constant increase in the proportion of patients with poor response to UDCA who have undergone transplantation or died, and therefore, new therapeutic regimens should be developed as soon as possible. It is necessary to develop new drugs which act on the key processes of disease progression, such as the "upstream" immune response, the "midstream" bile duct injury, and the "downstream" fibrotic process. Combination treatment with drugs targeting different pathways is a trend for future development. This article summarizes current potential therapeutic regimens for PBC and assesses the challenges in the treatment of PBC.

  10. Refractory pruritus in primary biliary cirrhosis

    Science.gov (United States)

    Pinheiro, Nuno Cercas; Marinho, Rui Tato; Ramalho, Fernando; Velosa, José

    2013-01-01

    Pruritus is a major symptom of primary biliary cirrhosis, cholestatic autoimmune disease which affects mostly middle-age women. Often, it can be severe and refractory to multiple treatments, and mostly affecting the patient’s health-related quality of life. Intense pruritus can be itself an indication to liver transplantation, in extreme cases leading to suicide. Its physiopathology has not yet been fully elucidated, but recent studies added the elevation of autotaxin and lysophosphatidic acid to the group of classic mechanisms already linked to cholestatic pruritus. In this case report we illustrate how ultraviolet B phototherapy appears to successfully control severe pruritus and contribute to the healing of pruritic skin lesions caused by intense scratching. There is limited medical literature concerning this therapeutic approach on cholestatic pruritus, but we hope that further randomised controlled trials will successfully establish it as an effective treatment in the near future. PMID:24234429

  11. Environmental factors in primary biliary cirrhosis.

    Science.gov (United States)

    Juran, Brian D; Lazaridis, Konstantinos N

    2014-08-01

    The etiology of the autoimmune liver disease primary biliary cirrhosis (PBC) remains largely unresolved, owing in large part to the complexity of interaction between environmental and genetic contributors underlying disease development. Observations of disease clustering, differences in geographical prevalence, and seasonality of diagnosis rates suggest the environmental component to PBC is strong, and epidemiological studies have consistently found cigarette smoking and history of urinary tract infection to be associated with PBC. Current evidence implicates molecular mimicry as a primary mechanism driving loss of tolerance and subsequent autoimmunity in PBC, yet other environmentally influenced disease processes are likely to be involved in pathogenesis. In this review, the authors provide an overview of current findings and touch on potential mechanisms behind the environmental component of PBC. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  12. [Transduodenal papillosphincterotomy in treatment of biliary lithiasis].

    Science.gov (United States)

    Zola, C; Salcuni, P; Manfredi, A; Foggi, E; Bozzetti, A

    1979-01-01

    The Authors report 180 cases of transdudenal papillosphincterotomy. Medium follow-up at 5 years revealed a complete recovery in 87 of 100 controlled patients. Nine cases revealed persistence of some degree of dyspepsia, while a second operation was necessary for the remaining patients to remove recurrent calculi in 2 cases and because of Oddi's stenosis in other two cases. A 4,4% mortality operative was observed, following postoperative pancreatitis in 1,1%, duodenal fistula or haemorrhage in 1,6%, and 1,6% from other causes. On the basis of their results the Authors conclude that transduodenal papillosphincterotomy is an effective procedure in treating complicated lithiasic biliary disease when respecting the reported indications.

  13. Cephalosporin-associated biliary pseudolithiasis in children

    Directory of Open Access Journals (Sweden)

    A.E. Abaturov

    2017-05-01

    Full Text Available The review presents current data about cephalosporin-associated biliary sludge and cholelithiasis formation mechanism. Risks factors of pseudolithiasis development in children, who received cephalosporins, are analyzed and systematized. Moreover, the study provides information about the prevalence of pseudolithiasis in different periods of childhood. Not only main ways of ceftriaxone-induced cholelithiasis clinical course, but also the most common complications of pseudolithiasis have been described. The report characterizes typical clinical symptoms and sonographic features of pseudolithiasis in children. Furthermore, the research considers main methods of treatment that allow to avoid unnecessary cholecystectomy in children. For writing the review, we used such databases, as Scopus, Web of Science, MedLine, PubMed, Google Scholar, CyberLeninka, RSCI.

  14. Risk of primary biliary cirrhosis in patients with coeliac disease

    DEFF Research Database (Denmark)

    Sørensen, Henrik Toft; Thulstrup, Ane Marie; Blomqvist, P

    1999-01-01

    BACKGROUND: Several case reports, but only a few studies, have examined the coexistence of coeliac disease and primary biliary cirrhosis. AIM: To estimate the risk of primary biliary cirrhosis in two national cohorts of patients with coeliac disease in Denmark and Sweden. METHODS: Through record...... linkage all Danish patients hospitalised with coeliac disease were followed for possible occurrence of primary biliary cirrhosis from 1 January 1977 until 31 December 1992. All patients hospitalised with coeliac disease in Sweden from 1987 to 1996 were also followed in a separate analysis. RESULTS......: A total of 896 patients with coeliac disease were identified in Denmark with a median follow up period of 9.1 years for a total of 8040 person-years at risk. Two cases of primary biliary cirrhosis were observed where 0.07 were expected, giving a standardised incidence ratio of 27.6 (95% confidence...

  15. Recent progress in ERCP for biliary and pancreatic diseases

    Directory of Open Access Journals (Sweden)

    MIAO Lin

    2014-12-01

    Full Text Available In recent years, with the continuous development of endoscopic and interventional techniques, many new devices and methods have been used in clinical practice, and the application of endoscopic retrograde cholangiopancreatography (ERCP in biliary and pancreatic diseases has developed rapidly. This paper reviews and summarizes the recent progress in ERCP among patients with biliary and pancreatic diseases, including those with altered gastrointestinal anatomy, pregnant patients, patients with benign and malignant biliary strictures, and patients with pancreatic pseudocysts, as well as the application of SpyGlass, photodynamic therapy, and radiofrequency ablation, the management of ERCP-related duodenal perforation, and the prevention of post-ERCP pancreatitis. All the progress has made a great contribution to the diagnosis and treatment of biliary and pancreatic diseases.

  16. Hormone replacement for osteoporosis in women with primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Rudic, Jelena S; Poropat, Goran; Krstic, Miodrag N

    2011-01-01

    Women with primary biliary cirrhosis often suffer from postmenopausal osteoporosis due to their age, or osteoporosis secondary to their liver disease, or treatments provided for their liver disease. Hormone replacement increases bone mineral density and reduces fractures in postmenopausal women...

  17. Assessment of the Relationship between Hyperlipoproteinemias and Biliary Lithiasis

    National Research Council Canada - National Science Library

    Dan Banut; Camelia Banut

    2009-01-01

      Aim: The purpose of this study was to bring new data regarding the prevalence of biliary lithiasis in Cluj district in regards to the role of hyperlipoproteinemias as a risk factor for this pathology...

  18. [Value of tissue harmonic imaging in biliary lithiasis].

    Science.gov (United States)

    Flunker, S; Aubé, C; Anglade, E; Vuillemin, E; Bourrée, Y; Burtin, P; Caron-Poitreau, C

    2001-01-01

    To define the role of harmonic imaging in the study of biliary lithiasis. We included 50 consecutive patients with suspected biliary lithiasis. Three independent observers compared results of harmonic imaging and conventional ultrasonography. This study showed a better interobserver agreement with harmonic imaging for acoustic shadow (kappa=0.87 vs 0.68) and intra-hepatic biliary stones (kappa=0.79 vs 0.49). More stones and more lithiasic gallbladders were seen with harmonic imaging (27 vs 24) and visualization of gallbladder sludge and the acoustic shadow from stones (P=0.01) was better. Ultrasound examination appears to be easier and faster and the diagnosis is more certain with harmonic imaging than with conventional ultrasonography (P=0.005). Harmonic imaging provides more information on biliary lithiasis and a more certain diagnosis.

  19. Evidence-Based Decompression in Malignant Biliary Obstruction

    Energy Technology Data Exchange (ETDEWEB)

    Ho, Chia Sing [University of Toronto, Toronto General Hospital, Toronto (Canada); Warkentin, Andrew E [University of Toronto, 1 King& #x27; s College Circle, Toronto (Canada)

    2012-02-15

    As recent advances in chemotherapy and surgical treatment have improved outcomes in patients with biliary cancers, the search for an optimal strategy for relief of their obstructive jaundice has become even more important. Without satisfactory relief of biliary obstruction, many patients would be ineligible for treatment. We review all prospective randomized trials and recent retrospective non-randomized studies for evidence that would support such a strategy. For distal malignant biliary obstruction, an optimal strategy would be insertion of metallic stents either endoscopically or percutaneously. Evidence shows that a metallic stent inserted percutaneously has better outcomes than plastic stents inserted endoscopically. For malignant hilar obstruction, percutaneous biliary drainage with or without metallic stents is preferred.

  20. 3 T MR cholangiopancreatography appearances of biliary ascariasis

    Energy Technology Data Exchange (ETDEWEB)

    Ding, Z.X., E-mail: hangzhoudzx73@126.co [Department of Radiology, Zhejiang Provincial People' s Hospital, Hangzhou (China); Yuan, J.H. [Department of Radiology, Zhejiang Provincial People' s Hospital, Hangzhou (China); Chong, V. [Department of Diagnostic Imaging, National University Health System, Yong Loo Lin School of Medicine, National University of Singapore (Singapore); Zhao, D.J. [Department of Hepatobiliary Surgery, Zhejiang Provincial People' s Hospital, Hangzhou (China); Chen, F.H.; Li, Y.M. [Department of Radiology, Zhejiang Provincial People' s Hospital, Hangzhou (China)

    2011-03-15

    Aim: To evaluate the 3 T MR cholangiopancreatography (MRCP) appearances of biliary ascariasis. Materials and methods: Nine patients with a MRCP diagnosis of biliary ascariasis were reviewed. All patients had endoscopic retrograde cholangiopancreatography (ERCP) or surgical confirmation of the disease. Results: On thin-slab MRCP imaging, Ascaris worms are clearly demarcated within the biliary tree. All the identified worms demonstrated a characteristic three-parallel-lines appearance. The middle high-signal intensity line is sandwiched between two low-signal intensity lines and they are in turn surrounded by high signal bile. On thick-slab MRCP the worms also show the three-line sign but with less clarity. However, thick-slab MRCP has the advantage of providing three-dimensional ERCP-like images of the pancreaticobiliary system. Conclusion: The 'three-line' sign appears to be a characteristic sign of biliary ascariasis on 3 T MRCP.

  1. Functional diagnosis of biliary drainage endoprosthesis by hepatobiliary scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Ranner, G.; Lammer, J.; Fueger, G.F.; Posch, E.; Hausegger, K.A.

    1988-11-01

    In 11 patients with biliary endoprosthesis, hepatobiliary scintigraphy with dimethyliminodiacetic acid (HIDA) was performed 16 times because of an increased in the serum bilirubin level. For the demonstration of dysfunction of the endoprosthesis, this method proved to have the same sensitivity as transhepatic cholangiography, which was performed as control examination. Therefore, hepatobiliary scintigraphy, a noninvasive and well-tolerated examination, can be recommended as a primary diagnostic tool in addition to ultrasonography for assessment of the drainage function of the biliary endoprosthesis.

  2. Assessment of the Relationship between Hyperlipoproteinemias and Biliary Lithiasis

    OpenAIRE

    Dan BANUT; Camelia BANUT

    2009-01-01

    Aim: The purpose of this study was to bring new data regarding the prevalence of biliary lithiasis in Cluj district in regards to the role of hyperlipoproteinemias as a risk factor for this pathology. Material and Method: A clinical study that included 800 adult subjects with age between 18 and 90 was conducted. For each subject an ultrasonographical screening was performed in order to detect biliary lithiasis. The subjects were separated in two subgroups: lithiasic and nonlithiasic. The seru...

  3. Obesity as a Risk Factor for Biliary Lithiasis - Clinical Study

    OpenAIRE

    Dan BANUT; Camelia BANUT

    2009-01-01

    The purpose of this study is to bring new data regarding the prevalence of biliary lithiasis in our region (Cluj district) and regarding the role of obesity as a risk factor for this pathology. We performed a clinical study which included a significant number of participants - 2348 subjects. For each subject an ultrasonographical screening was performed in order to detect biliary lithiasis and to collected anthropometric data (height, weight, and body mass index). We determined the prevalence...

  4. Diagnosis and Treatment of Biliary Fistulas in the Laparoscopic Era

    Directory of Open Access Journals (Sweden)

    M. Crespi

    2016-01-01

    Full Text Available Biliary fistulas are rare complications of gallstone. They can affect either the biliary or the gastrointestinal tract and are usually classified as primary or secondary. The primary fistulas are related to the biliary lithiasis, while the secondary ones are related to surgical complications. Laparoscopic surgery is a therapeutic option for the treatment of primary biliary fistulas. However, it could be the first responsible for the development of secondary biliary fistulas. An accurate preoperative diagnosis together with an experienced surgeon on the hepatobiliary surgery is necessary to deal with biliary fistulas. Cholecystectomy with a choledocoplasty is the most frequent treatment of primary fistulas, whereas the bile duct drainage or the endoscopic stenting is the best choice in case of minor iatrogenic bile duct injuries. Roux-en-Y hepaticojejunostomy is the extreme therapeutic option for both conditions. The sepsis, the level of the bile duct damage, and the involvement of the gastrointestinal tract increase the complexity of the operation and affect early and late results.

  5. Diagnosis and Treatment of Biliary Fistulas in the Laparoscopic Era.

    Science.gov (United States)

    Crespi, M; Montecamozzo, G; Foschi, D

    2016-01-01

    Biliary fistulas are rare complications of gallstone. They can affect either the biliary or the gastrointestinal tract and are usually classified as primary or secondary. The primary fistulas are related to the biliary lithiasis, while the secondary ones are related to surgical complications. Laparoscopic surgery is a therapeutic option for the treatment of primary biliary fistulas. However, it could be the first responsible for the development of secondary biliary fistulas. An accurate preoperative diagnosis together with an experienced surgeon on the hepatobiliary surgery is necessary to deal with biliary fistulas. Cholecystectomy with a choledocoplasty is the most frequent treatment of primary fistulas, whereas the bile duct drainage or the endoscopic stenting is the best choice in case of minor iatrogenic bile duct injuries. Roux-en-Y hepaticojejunostomy is the extreme therapeutic option for both conditions. The sepsis, the level of the bile duct damage, and the involvement of the gastrointestinal tract increase the complexity of the operation and affect early and late results.

  6. Radiofrequency-assisted "reconstruction" of the right ventricular outflow tract in muscular pulmonary atresia with ventricular septal defect.

    Science.gov (United States)

    Hausdorf, G; Schulze-Neick, I; Lange, P E

    1993-04-01

    A case of pulmonary atresia with ventricular septal defect is reported in which a communication was established between the right ventricle and the hypoplastic pulmonary artery by intervention, despite muscular atresia of the right ventricular outflow tract. The atresia was perforated with a special designed radiofrequency catheter (Osypka). After the creation of a canal within the muscular atresia, balloon dilatation (diameters 2, 3.5, and 7.2 mm) was performed. Arterial oxygen saturation increased from 64% to 78%. Lateral deviation of the radiofrequency catheter resulting in a lateral perforation of the atretic muscular infundibulum was well tolerated without later sequelae. Early restenosis within two weeks necessitated the implantation of a stent within the "recanalised" atresia resulting in an increased anterograde flow to the pulmonary artery. This case shows that "recanalisation" of muscular atresia of the pulmonary artery by radiofrequency is a promising technique. Additionally, stent implantation into the infundibulum to prevent restenosis is a first step to interventional right ventricular outflow tract reconstruction.

  7. Early biliary decompression versus conservative treatment in acute biliary pancreatitis (APEC trial) : study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    Schepers, Nicolien J.; Bakker, Olaf J.; Besselink, Marc G. H.; Bollen, Thomas L.; Dijkgraaf, Marcel G. W.; van Eijck, Casper H. J.; Fockens, Paul; van Geenen, Erwin J. M.; van Grinsven, Janneke; Hallensleben, Nora D. L.; Hansen, Bettina E.; van Santvoort, Hjalmar C.; Timmer, Robin; Anten, Marie-Paule G. F.; Bolwerk, Clemens J. M.; van Delft, Foke; van Dullemen, Hendrik M.; Erkelens, G. Willemien; van Hooft, Jeanin E.; Laheij, Robert; van der Hulst, Rene W. M.; Jansen, Jeroen M.; Kubben, Frank J. G. M.; Kuiken, Sjoerd D.; Perk, Lars E.; de Ridder, Rogier J. J.; Rijk, Marno C. M.; Romkens, Tessa E. H.; Schoon, Erik J.; Schwartz, Matthijs P.; Spanier, B. W. Marcel; Tan, Adriaan C. I. T. L.; Thijs, Willem J.; Venneman, Niels G.; Vleggaar, Frank P.; van de Vrie, Wim; Witteman, Ben J.; Gooszen, Hein G.; Bruno, Marco J.

    2016-01-01

    Background: Acute pancreatitis is mostly caused by gallstones or sludge. Early decompression of the biliary tree by endoscopic retrograde cholangiography (ERC) with sphincterotomy may improve outcome in these patients. Whereas current guidelines recommend early ERC in patients with concomitant

  8. Early biliary decompression versus conservative treatment in acute biliary pancreatitis (APEC trial) : study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    Schepers, Nicolien J; Bakker, Olaf J; Besselink, Marc G H; Bollen, Thomas L; Dijkgraaf, Marcel G W; van Eijck, Casper H J; Fockens, Paul; van Geenen, Erwin J M; van Grinsven, Janneke; Hallensleben, Nora D L; Hansen, Bettina E; van Santvoort, Hjalmar C; Timmer, Robin; Anten, Marie-Paule G F; Bolwerk, Clemens J M; van Delft, Foke; van Dullemen, Hendrik M; Erkelens, G Willemien; van Hooft, Jeanin E; Laheij, Robert; van der Hulst, René W M; Jansen, Jeroen M; Kubben, Frank J G M; Kuiken, Sjoerd D; Perk, Lars E; de Ridder, Rogier J J; Rijk, Marno C M; Römkens, Tessa E H; Schoon, Erik J; Schwartz, Matthijs P; Spanier, B W Marcel; Tan, Adriaan C I T L; Thijs, Willem J; Venneman, Niels G; Vleggaar, Frank P; van de Vrie, Wim; Witteman, Ben J; Gooszen, Hein G; Bruno, Marco J

    2016-01-01

    BACKGROUND: Acute pancreatitis is mostly caused by gallstones or sludge. Early decompression of the biliary tree by endoscopic retrograde cholangiography (ERC) with sphincterotomy may improve outcome in these patients. Whereas current guidelines recommend early ERC in patients with concomitant

  9. Early biliary decompression versus conservative treatment in acute biliary pancreatitis (APEC trial) : Study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    Schepers, Nicolien J.; Bakker, Olaf J.; Besselink, Marc G H; Bollen, Thomas L.; Dijkgraaf, Marcel G W; van Eijck, Casper H J; Fockens, Paul; van Geenen, Erwin J M; van Grinsven, Janneke; Hallensleben, Nora D L; Hansen, Bettina E.; van Santvoort, Hjalmar C.; Timmer, Robin; Anten, Marie Paule G F; Bolwerk, Clemens J M; van Delft, Foke; van Dullemen, Hendrik M.; Erkelens, G. Willemien; van Hooft, Jeanin E.; Laheij, Robert; van der Hulst, René W M; Jansen, Jeroen M.; Kubben, Frank J G M; Kuiken, Sjoerd D.; Perk, Lars E.; de Ridder, Rogier J J; Rijk, Marno C M; Römkens, Tessa E H; Schoon, Erik J.; Schwartz, Matthijs P.; Spanier, B. W Marcel; Tan, Adriaan C I T L; Thijs, Willem J.; Venneman, Niels G.; Vleggaar, Frank P.; van de Vrie, Wim; Witteman, Ben J.; Gooszen, Hein G.; Bruno, Marco J.

    2016-01-01

    Background: Acute pancreatitis is mostly caused by gallstones or sludge. Early decompression of the biliary tree by endoscopic retrograde cholangiography (ERC) with sphincterotomy may improve outcome in these patients. Whereas current guidelines recommend early ERC in patients with concomitant

  10. Early biliary decompression versus conservative treatment in acute biliary pancreatitis (APEC trial): study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    Schepers, Nicolien J.; Bakker, Olaf J.; Besselink, Marc G. H.; Bollen, Thomas L.; Dijkgraaf, Marcel G. W.; van Eijck, Casper H. J.; Fockens, Paul; van Geenen, Erwin J. M.; van Grinsven, Janneke; Hallensleben, Nora D. L.; Hansen, Bettina E.; van Santvoort, Hjalmar C.; Timmer, Robin; Anten, Marie-Paule G. F.; Bolwerk, Clemens J. M.; van Delft, Foke; van Dullemen, Hendrik M.; Erkelens, G. Willemien; van Hooft, Jeanin E.; Laheij, Robert; van der Hulst, René W. M.; Jansen, Jeroen M.; Kubben, Frank J. G. M.; Kuiken, Sjoerd D.; Perk, Lars E.; de Ridder, Rogier J. J.; Rijk, Marno C. M.; Römkens, Tessa E. H.; Schoon, Erik J.; Schwartz, Matthijs P.; Spanier, B. W. Marcel; Tan, Adriaan C. I. T. L.; Thijs, Willem J.; Venneman, Niels G.; Vleggaar, Frank P.; van de Vrie, Wim; Witteman, Ben J.; Gooszen, Hein G.; Bruno, Marco J.

    2016-01-01

    Acute pancreatitis is mostly caused by gallstones or sludge. Early decompression of the biliary tree by endoscopic retrograde cholangiography (ERC) with sphincterotomy may improve outcome in these patients. Whereas current guidelines recommend early ERC in patients with concomitant cholangitis,

  11. Early biliary decompression versus conservative treatment in acute biliary pancreatitis (APEC trial): study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    Schepers, N.J.; Bakker, O.J.; Besselink, M.G.; Bollen, T.L.; Dijkgraaf, M.G.; Eijck, C.H. van; Fockens, P.; Geenen, E.J. van; Grinsven, J. van; Hallensleben, N.D.; Hansen, B.E.; Santvoort, H.C. van; Timmer, R.; Anten, M.P.; Bolwerk, C.J.; Delft, F. von; Dullemen, H.M. van; Erkelens, G.W.; Hooft, J.E. van; Laheij, R.; Hulst, R.W. van der; Jansen, J.M.; Kubben, F.J.; Kuiken, S.D.; Perk, L.E.; Ridder, R.J. de; Rijk, M.C. de; Romkens, T.E.; Schoon, E.J.; Schwartz, M.P.; Spanier, B.W.; Tan, A.C.; Thijs, W.J.; Venneman, N.G.; Vleggaar, F.P.; Vrie, W. van de; Witteman, B.J.; Gooszen, H.G.; Bruno, M.J.

    2016-01-01

    BACKGROUND: Acute pancreatitis is mostly caused by gallstones or sludge. Early decompression of the biliary tree by endoscopic retrograde cholangiography (ERC) with sphincterotomy may improve outcome in these patients. Whereas current guidelines recommend early ERC in patients with concomitant

  12. Early biliary decompression versus conservative treatment in acute biliary pancreatitis (APEC trial): Study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    N.J. Schepers (Nicolien); O.J. Bakker (Olaf ); M.G. Besselink (Marc); T.L. Bollen (Thomas); M.G.W. Dijkgraaf (Marcel); C.H.J. van Eijck (Casper); P. Fockens (Paul); E-J.M. Geenen (Erwin-Jan); J. van Grinsven (Janneke); N.D.L. Hallensleben (Nora D.L.); B.E. Hansen (Bettina); H.C. van Santvoort (Hjalmar); R. Timmer (Robin); M.-P.G.F. Anten (Marie-Paule G.F.); C.L. Bolwerk (Clemens); F. van Delft (Foke); H.M. van Dullemen (Hendrik); G.W. Erkelens (G.Willemien); J.E. van Hooft (Jeanin); C. Laheij (Claudia); R.W.M. van der Hulst (René); J.M. Jansen (Jeroen); F.J. Kubben; S.D. Kuiken (Sjoerd D.); L.E. Perk (Lars E.); R. de Ridder (Rogier); M.C.M. Rijk; T.E.H. Römkens; E.J. Schoon (Erik); M.P. Schwartz (Matthijs P.); B.W.M. Spanier (Marcel); A.C. Tan (Adriaan); W.J. Thijs; N.G. Venneman (Niels); F.P. Vleggaar (Frank); W. van de Vrie (Wim); B.J.M. Witteman (Ben); H.G. Gooszen (Hein); M.J. Bruno (Marco)

    2016-01-01

    textabstractBackground: Acute pancreatitis is mostly caused by gallstones or sludge. Early decompression of the biliary tree by endoscopic retrograde cholangiography (ERC) with sphincterotomy may improve outcome in these patients. Whereas current guidelines recommend early ERC in patients with

  13. Biliary obstruction caused by intra-biliary tumor growth from recurred hepatocellular carcinoma after radiofrequency ablation: Case report

    Energy Technology Data Exchange (ETDEWEB)

    Yi, Ji Hyun; Kim, Jae Won [Dept. of Radiology, Yeungnam University College of Medicine, Daegu (Korea, Republic of)

    2014-04-15

    A 59-year-old man with a known central hepatocellular carcinoma (HCC) underwent a trans-arterial-chemo-embolization (TACE) and a post-TACE percutaneous radiofrequency ablation (PRFA). Two months after the PRFA, the patient presented jaundice and an abdominal computed tomography was obtained. An arterial enhancing mass adjacent to the ablated necrotic lesion with a continuously coexisting mass inside the right hepatic duct, suggestive of a HCC recurrence with a direct extension to the biliary tract was found. Finally a biliary tumor obstruction has been developed and a percutaneous transhepatic biliary drainage was performed. This case of biliary obstruction caused by directly invaded recurred HCC after PRFA will be reported because of its rare occurrence.

  14. Suplementação com ácido ascórbico tem efeito citoprotetor na cirrose biliar secundária: estudo experimental em ratos jovens Ascorbic acid supplementation has a cytoprotective effect on secondary biliary cirrhosis: experimental study in young rats

    Directory of Open Access Journals (Sweden)

    Cynthia R. Matos Silva Passoni

    2008-12-01

    Full Text Available OBJETIVO: Testar se a suplementação com ácido ascórbico tem algum afeito citoprotetor em um modelo de cirrose biliar secundária em ratos jovens. MÉTODOS: Foram estudados 40 ratos Wistar desmamados no 21º dia pós-natal. Cada grupo de 10 foi submetido a um dos seguintes quatro tratamentos, até o 49º dia pós-natal, quando foram submetidos a eutanásia: 1 LC - ligadura dupla e ressecção do ducto biliar comum e administração diária de ácido ascórbico [100 mg/g de peso corporal (pc]; 2 LA - ligadura dupla e ressecção do ducto biliar comum e administração diária de veículo aquoso (1 mL/g pc; 3 SC - operação simulada e administração diária de ácido ascórbico (100 mg/g pc; 4 SA - ligadura dupla e ressecção do ducto biliar comum e administração diária de veículo aquoso (1 mL/g pc. Os ratos eram pesados diariamente. No 27º dia pós-operatório, eles receberam injeção intraperitoneal de 1,5 mg/g pc de pentobarbital sódico, e o tempo de sono induzido pelo pentobarbital foi medido. Coletou-se sangue para determinação de atividade sérica de alanina aminotransferase e de aspartato aminotransferase, níveis de albumina e globulina séricas, e o fígado foi analisado quanto à conteúdo de água e gordura. Os dados foram submetidos à ANOVA two-way, e comparações pareadas entre grupos foram testadas com o método de SNK. O nível de significância foi estabelecido em 0,05. RESULTADOS: A suplementação com ácido ascórbico atenuou os efeitos da colestase: reduziu o tempo de anestesia pelo pentobarbital, globulina sérica e o conteúdo de gordura no fígado. CONCLUSÕES: Nossos resultados corroboram a hipótese de que a suplementação com ácido ascórbico tem um efeito citoprotetor na cirrose biliar secundária.OBJECTIVE: To test whether ascorbic acid supplementation has any cytoprotective effect on a model of secondary biliary cirrhosis in young rats. METHODS: We studied 40 Wistar rats weaned at the 21st postnatal

  15. Concurrent biliary drainage and portal vein embolization in preparation for extended hepatectomy in patients with biliary cancer

    DEFF Research Database (Denmark)

    Nilsson, Jan; Eriksson, Sam; Nørgaard Larsen, Peter

    2015-01-01

    BACKGROUND: Patients with perihilar cholangiocarcinoma and gallbladder cancer extending into the hilum often present with jaundice and a small future liver remnant (FLR). If resectable, preoperative biliary drainage and portal vein embolization (PVE) are indicated. Classically, these measures have...... been performed sequentially, separated by 4-6 weeks. PURPOSE: To report on a new regime where percutaneous transhepatic biliary drainage (PTBD) and PVE are performed simultaneously, shortening the preoperative process. MATERIAL AND METHODS: Six patients were treated with concurrent PTBD and PVE under...

  16. Percutaneous intraductal radiofrequency ablation combined with biliary stent placement for malignant biliary obstruction: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Lijun Guan

    2016-01-01

    Full Text Available Percutaneous management of unresectable malignant biliary obstruction remains challenging. Biliary radiofrequency ablation (RFA using the Habib EndoHBP catheter is a new palliation therapy for malignant biliary obstruction. We report our initial experience of RFA for the management of malignant biliary obstruction. A 58-year-old male was diagnosed with gallbladder cancer. Intraductal bipolar RFA was delivered at power of 10 W for 120 s, followed by stent placement. The patient had immediate stricture improvements after RFA. No severe adverse event occurred. Percutaneous RFA seems to be safe and feasible for the treatment of malignant biliary obstruction. Further studies are warranted.

  17. A Case of Malignant Biliary Obstruction with Severe Obesity Successfully Treated by Endoscopic Ultrasonography-Guided Biliary Drainage

    Science.gov (United States)

    Yamasaki, Shuuji

    2016-01-01

    Here, we present a case of malignant biliary tract obstruction with severe obesity, which was successfully treated by endoscopic ultrasonography-guided biliary drainage (EUS-BD). A female patient in her sixties who had been undergoing chemotherapy for unresectable pancreatic head cancer was admitted to our institution for obstructive jaundice. She had diabetes mellitus, and her body mass index was 35.1 kg/m2. Initially, endoscopic retrograde cholangiopancreatography (ERCP) was performed, but bile duct cannulation was unsuccessful. Percutaneous transhepatic biliary drainage (PTBD) from the left hepatic biliary tree also failed. Although a second PTBD attempt from the right hepatic lobe was accomplished, biliary tract bleeding followed, and the catheter was dislodged. Consequently, EUS-BD (choledochoduodenostomy), followed by direct metallic stent placement, was performed as a third drainage method. Her postprocedural course was uneventful. Following discharge, she spent the rest of her life at home without recurrent jaundice or readmission. In cases of severe obesity, we consider EUS-BD, rather than PTBD, as the second drainage method of choice for distal malignant biliary obstruction when ERCP fails. PMID:27698669

  18. A Case of Malignant Biliary Obstruction with Severe Obesity Successfully Treated by Endoscopic Ultrasonography-Guided Biliary Drainage

    Directory of Open Access Journals (Sweden)

    Takashi Obana

    2016-01-01

    Full Text Available Here, we present a case of malignant biliary tract obstruction with severe obesity, which was successfully treated by endoscopic ultrasonography-guided biliary drainage (EUS-BD. A female patient in her sixties who had been undergoing chemotherapy for unresectable pancreatic head cancer was admitted to our institution for obstructive jaundice. She had diabetes mellitus, and her body mass index was 35.1 kg/m2. Initially, endoscopic retrograde cholangiopancreatography (ERCP was performed, but bile duct cannulation was unsuccessful. Percutaneous transhepatic biliary drainage (PTBD from the left hepatic biliary tree also failed. Although a second PTBD attempt from the right hepatic lobe was accomplished, biliary tract bleeding followed, and the catheter was dislodged. Consequently, EUS-BD (choledochoduodenostomy, followed by direct metallic stent placement, was performed as a third drainage method. Her postprocedural course was uneventful. Following discharge, she spent the rest of her life at home without recurrent jaundice or readmission. In cases of severe obesity, we consider EUS-BD, rather than PTBD, as the second drainage method of choice for distal malignant biliary obstruction when ERCP fails.

  19. Biliary Infection May Exacerbate Biliary Cystogenesis Through the Induction of VEGF in Cholangiocytes of the Polycystic Kidney (PCK) Rat

    Science.gov (United States)

    Ren, Xiang Shan; Sato, Yasunori; Harada, Kenichi; Sasaki, Motoko; Yoneda, Norihide; Lin, Zhen Hua; Nakanuma, Yasuni

    2011-01-01

    Cholangitis arising from biliary infection dominates the prognosis in Caroli's disease. To clarify the influences of bacterial infection on the biliary cystogenesis, in vivo and in vitro studies were performed using the polycystic kidney (PCK) rat as an animal model of Caroli's disease. Cholangitis became a frequent histological finding in aged PCK rats, and neovascularization around the bile ducts also increased in aged PCK rats. Immunohistochemistry revealed that expression of vascular endothelial growth factor (VEGF) was increased in PCK rat biliary epithelium. In vitro, PCK cholangiocytes overexpressed VEGF, and the supernatant of cultured PCK cholangiocytes significantly increased the proliferative activity, migration, and tube formation of cultured rat vascular endothelial cells. Stimulation with lipopolysaccharide (LPS) further induced VEGF expression in PCK cholangiocytes, which might be mediated by signaling pathways involving phosphatidylinositol 3-kinase (PI3K)-Akt and c-Jun N-terminal kinase (JNK). Both LPS and VEGF increased cell proliferative activity in PCK cholangiocytes, and siRNA against VEGF significantly reduced LPS-induced cell proliferation. Thus, LPS-induced overexpression of VEGF in the biliary epithelium may lead to hypervascularity around the bile ducts; concurrently, LPS and VEGF act as cell proliferation factors for cholangiocytes. Biliary infection may thus exacerbate biliary cystogenesis in PCK rats. PMID:22015458

  20. 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.

  1. Atresia Hymenalis with Haematometrocolpos: A Benefit of Teaching a Case Series and Review of the Literature

    LENUS (Irish Health Repository)

    Whitla, L

    2017-09-01

    Imperforate hymen or atresia hymenalis is a rare clinical presentation, the condition is estimated to be present in 0.5\\/1000 females1. It is the most common obstructive anomaly of the female genital tract, and results from failure of canalization of the vaginal plate in utero. Atresia hymenalis can present in neonates as bulging hymen due to accumulation of secretions by the uterovaginal mucosa2,3. Usually the imperforate hymen is asymptomatic until menarche, when haematometrocolpos results in symptoms such as abdominal pain, abdominal mass, urinary retention and constipation. Many cases are diagnosed in the evaluation of primary amenorrhoea or recurrent abdominal pain2,3. We present 4 cases diagnosed over a 7 month period in our ED.

  2. 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. Copyright © 2015 Elsevier Ltd. All rights reserved.

  3. Atresia anal com fístula retovaginal em ovino: relato de caso

    Directory of Open Access Journals (Sweden)

    M.L. Antonioli

    Full Text Available RESUMO A atresia anal com fístula retovaginal, é considerada uma afecção congênita rara nos ovinos. Em virtude disso, buscou-se descrever o reparo cirúrgico e cuidados pós-operatórios em uma borrega que apresentava essa afecção. Nesse sentido, uma borrega, sem raça definida, de 25 dias de idade e pesando 7,2kg, apresentou sinais de distensão abdominal e defecação pela vulva. A afecção foi diagnosticada por meio do exame clínico e confirmada radiograficamente, constatando-se atresia anal do tipo III em associação com a fístula.

  4. BAER testing in a dog with bilateral external ear canal atresia.

    Science.gov (United States)

    Anwer, Cona; Schwarz, Tobias; Volk, Susan W; Vite, Charles

    2011-01-01

    A 3 yr old male castrated Labrador retriever presented for evaluation and treatment of bilateral atresia of the external ear canals. The owners reported that the dog could hear only loud and high-pitched noises. Computed tomography of the head revealed intact vertical and horizontal ear canals filled with debris and a debris-filled right tympanic bulla. Air- and bone-conducted brainstem auditory evoked response (BAER) testing revealed an elevated response threshold to air-conducted stimuli and greater amplitude waveforms evoked by bone-conducted stimuli. The ear canals were surgically corrected via lateral ear canal resection. BAER testing postoperatively revealed a decrease in the air-conducted BAER threshold. This case is an example of the use of bone-conducted BAER testing to aid in the diagnosis of conductive deafness, and in determining prognosis for normal hearing after surgical treatment of external ear canal atresia.

  5. Ileal atresia and multiple jejunal perforations in a premature neonate with gestational alloimmune liver disease

    Directory of Open Access Journals (Sweden)

    Ryan M. McAdams, MD

    2017-04-01

    Full Text Available Recovery after surgical repair of an ileal atresia with or without intestinal perforation requires prolonged exposure to parenteral nutrition (PN that may lead to PN-associated liver disease. Early liver failure and cholestasis out of proportion for PN exposure may be a harbinger for gestational alloimmune liver disease (GALD, a potentially life-threatening condition that often requires liver transplant if not treated in a timely manner. This case report presents a premature neonate with ileal atresia and multiple jejunal perforations who developed liver failure and was later determined to have GALD. Recognition of clinical and laboratory findings consistent with GALD is essential to promote early treatment, which can enhance neonatal outcomes and impact future pregnancies.

  6. Poor Reproducibility of Gallbladder Ejection Fraction by Biliary Scintigraphy for Diagnosis of Biliary Dyskinesia.

    Science.gov (United States)

    Rose, J Bart; Fields, Ryan C; Strasberg, Steven M

    2018-02-01

    Twenty percent of cholecystectomies in the US are performed for a diagnosis of biliary dyskinesia. Diagnosis is made by measuring gallbladder ejection fraction (GbEF) using hepatobiliary scintigraphy. Our purpose was to evaluate the reproducibility of GbEF measurements. This is a retrospective review of patients referred for cholecystectomy, from 2010 to 2016, with a diagnosis of biliary dyskinesia based on a GbEF test, who then underwent a repeat GbEF test. Thirty consecutive patients were identified by hospital records. Re-testing of GbEF was performed at least 6 weeks after the initial test using Tc-99m and slow injection of sincalide at 0.02 mcg/kg. On re-testing, 16 of 30 patients (53%) patients had a normal GbEF of >35%, ie the initial test result was not reproducible in them. Age, sex, days between testing, and initial GbEF did not differ between groups. The 14 patients who re-tested positive for biliary dyskinesia with reduced GbEF were significantly more likely to have episodic pain than steady pain. Re-testing frequently resulted in change in management in that most patients who re-tested in the normal range were not offered cholecystectomy. Hepatobiliary scintigraphy with GbEF is a poorly reproducible test. Re-testing resulted in a change in management in many patients who then avoided cholecystectomy. Strong consideration should be given to repeating hepatobiliary scintigraphy with GbEF before cholecystectomy in patients with an initial positive test. Copyright © 2017 American College of Surgeons. Published by Elsevier Inc. All rights reserved.

  7. Efficacy of percutaneous intraductal radiofrequency ablation combined with biliary stenting in treatment of malignant biliary obstruction

    Directory of Open Access Journals (Sweden)

    ZHANG Kai

    2015-05-01

    Full Text Available ObjectiveTo investigate the safety and feasibility of percutaneous intraductal radiofrequency ablation (palliative therapy in the treatment of malignant obstructive jaundice. MethodsThis study included 20 patients with unresectable malignant obstructive jaundice, who were treated in the First Affiliated Hospital of Zhengzhou University from January 2013 to March 2014. Nine of them (test group underwent percutaneous intraductal radiofrequency ablation plus metallic biliary stent placement. The other 11 similar cases (control group underwent metallic biliary stent placement alone after successful percutaneous transhepatic cholangiography. Operative complications and remission of jaundice were observed, and the stent patency after at 3 and 6 months after operation was evaluated and compared between the two groups. ResultsAll patients were followed completely by outpatient or telephone. The stent patency rate at 3 months after operation was 9/9 in the test group and 8/11 in the control group (χ2=2.888, P=0.218, and the stent patency rates at 6 months were 7/8 and 3/11, respectively (χ2=6.739, P=0.02. During follow-up, one case in the test group died of gastrointestinal bleeding at 113 d after operation; one case in the control group died of liver failure at 57 d after operation and one case died of disseminated intravascular coagulation at 142 d. ConclusionPercutaneous intraductal radiofrequency ablation is safe and feasible in the treatment of malignant biliary obstruction, and the preliminary efficacy in prolonging the patency of self-expanding metallic stent is satisfactory. However, this therapy needs to be further verified via large-sample randomized controlled studies.

  8. Helicobacter species are associated with possible increase in risk of biliary lithiasis and benign biliary diseases

    Science.gov (United States)

    Pandey, Manoj

    2007-01-01

    Background Hepato-biliary tract lithiasis is common and present either as pain or as asymptomatic on abdominal ultrasonography for other causes. Although the DNA of Helicobacter species are identified in the gallbladder bile, tissue or stones analyzed from these cases, still a causal relationship could not be established due to different results from different geographical parts. Methods A detailed search of pubmed and pubmedcentral was carried out with key words Helicobacter and gallbladder, gallstones, hepaticolithiasis, cholelithiasis and choledocholithiasis, benign biliary diseases, liver diseases. The data was entered in a data base and meta analysis was carried out. The analysis was carried out using odds ratio and a fixed effect model, 95% confidence intervals for odds ratio was calculated. Chi square test for heterogeneity was employed. The overall effect was calculated using Z test. Results A total of 12 articles were identified. One study used IgG for diagnosis while others used the PCR for Ure A gene, 16 S RNA or Cag A genes. A couple of studies used culture or histopathology besides the PCR. The cumulative results show a higher association of Helicobacter with chronic liver diseases (30.48%), and stone diseases (42.96%)(OR 1.77 95% CI 1.2–2.58; Z = 2.94, p = 0.003), the effect of each could not be identified as it was difficult to isolate the effect of helicobacter due to mixing of cases in each study. Conclusion The results of present meta analysis shows that there is a slight higher risk of cholelithiasis and benign liver disease (OR 1.77), however due to inherent inability to isolate the effect of stone disease from that of other benign lesions it is not possible to say for sure that Helicobacter has a casual relationship with benign biliary disease or stone disease or both. PMID:17708763

  9. Helicobacter species are associated with possible increase in risk of biliary lithiasis and benign biliary diseases

    Directory of Open Access Journals (Sweden)

    Pandey Manoj

    2007-08-01

    Full Text Available Abstract Background Hepato-biliary tract lithiasis is common and present either as pain or as asymptomatic on abdominal ultrasonography for other causes. Although the DNA of Helicobacter species are identified in the gallbladder bile, tissue or stones analyzed from these cases, still a causal relationship could not be established due to different results from different geographical parts. Methods A detailed search of pubmed and pubmedcentral was carried out with key words Helicobacter and gallbladder, gallstones, hepaticolithiasis, cholelithiasis and choledocholithiasis, benign biliary diseases, liver diseases. The data was entered in a data base and meta analysis was carried out. The analysis was carried out using odds ratio and a fixed effect model, 95% confidence intervals for odds ratio was calculated. Chi square test for heterogeneity was employed. The overall effect was calculated using Z test. Results A total of 12 articles were identified. One study used IgG for diagnosis while others used the PCR for Ure A gene, 16 S RNA or Cag A genes. A couple of studies used culture or histopathology besides the PCR. The cumulative results show a higher association of Helicobacter with chronic liver diseases (30.48%, and stone diseases (42.96%(OR 1.77 95% CI 1.2–2.58; Z = 2.94, p = 0.003, the effect of each could not be identified as it was difficult to isolate the effect of helicobacter due to mixing of cases in each study. Conclusion The results of present meta analysis shows that there is a slight higher risk of cholelithiasis and benign liver disease (OR 1.77, however due to inherent inability to isolate the effect of stone disease from that of other benign lesions it is not possible to say for sure that Helicobacter has a casual relationship with benign biliary disease or stone disease or both.

  10. Helicobacter species are associated with possible increase in risk of biliary lithiasis and benign biliary diseases.

    Science.gov (United States)

    Pandey, Manoj

    2007-08-20

    Hepato-biliary tract lithiasis is common and present either as pain or as asymptomatic on abdominal ultrasonography for other causes. Although the DNA of Helicobacter species are identified in the gallbladder bile, tissue or stones analyzed from these cases, still a causal relationship could not be established due to different results from different geographical parts. A detailed search of pubmed and pubmedcentral was carried out with key words Helicobacter and gallbladder, gallstones, hepaticolithiasis, cholelithiasis and choledocholithiasis, benign biliary diseases, liver diseases. The data was entered in a data base and meta analysis was carried out. The analysis was carried out using odds ratio and a fixed effect model, 95% confidence intervals for odds ratio was calculated. Chi square test for heterogeneity was employed. The overall effect was calculated using Z test. A total of 12 articles were identified. One study used IgG for diagnosis while others used the PCR for Ure A gene, 16 S RNA or Cag A genes. A couple of studies used culture or histopathology besides the PCR. The cumulative results show a higher association of Helicobacter with chronic liver diseases (30.48%), and stone diseases (42.96%)(OR 1.77 95% CI 1.2-2.58; Z = 2.94, p = 0.003), the effect of each could not be identified as it was difficult to isolate the effect of helicobacter due to mixing of cases in each study. The results of present meta analysis shows that there is a slight higher risk of cholelithiasis and benign liver disease (OR 1.77), however due to inherent inability to isolate the effect of stone disease from that of other benign lesions it is not possible to say for sure that Helicobacter has a casual relationship with benign biliary disease or stone disease or both.

  11. Specific echocardiographic findings useful for the diagnosis of common pulmonary vein atresia

    Directory of Open Access Journals (Sweden)

    Hiroyuki Nagasawa

    2015-12-01

    Full Text Available In this paper, we report a case of common pulmonary vein atresia, which is a very rare disease characterized by cyanosis, heart failure and pulmonary hypertension. Reverse flow in the pulmonary artery at end-diastole as well as in the isthmus of the aorta from early systole to end-diastole detected by echocardiography were found to be specific features useful in diagnosing the disease.

  12. 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. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. Early neonatal death and congenital left coronary abnormalities: ostial atresia, stenosis and anomalous aortic origin.

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    Laux, Daniela; Bessières, Bettina; Houyel, Lucile; Bonnière, Maryse; Magny, Jean-François; Bajolle, Fanny; Boudjemline, Younes; Bonnet, Damien

    2013-04-01

    Congenital left coronary artery abnormalities such as ostial stenosis or atresia are extremely rare. Diagnosis in the neonate has not been reported. To describe five neonates with left coronary artery orifice abnormalities and discuss pathophysiology, diagnosis and treatment options, with a focus on the importance of autopsy in unexpected neonatal death. Retrospective assessment of medical files of neonates with left coronary abnormalities seen during a 12-year period (2000-2012). Three neonates with anatomical (n=2) and functional (n=1) left coronary stenosis and two neonates with ostial atresia were identified. The three infants with coronary stenosis died within minutes to days after birth because of cardiac failure refractory to intensive care treatment; at autopsy, left coronary ostial stenosis (n=2) and high take-off with acute angle origin and tangential vertical course (n=1) were diagnosed. The fourth neonate was in cardiac failure due to critical aortic stenosis; left coronary ostial atresia was diagnosed during an emergency catheter procedure and the infant died after aortic valve dilatation. The fifth infant had a cardiac arrest on the third day of life; she was diagnosed with left coronary ostial atresia by coronary angiography and died during attempted revascularization surgery at 2 weeks of life. Congenital coronary ostial abnormalities can lead to severe heart failure and unexpected neonatal death. Systematic examination of the coronary arteries should be part of any neonatal autopsy. Coronary angiography remains the diagnostic method of choice despite advances in non-invasive imaging. Revascularization surgery seems indicated in symptomatic children based on small patient series. Copyright © 2013. Published by Elsevier Masson SAS.

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

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    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.

  15. Ileal atresia and multiple jejunal perforations in a premature neonate with gestational alloimmune liver disease

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    McAdams, Ryan M.

    2017-01-01

    Recovery after surgical repair of an ileal atresia with or without intestinal perforation requires prolonged exposure to parenteral nutrition (PN) that may lead to PN-associated liver disease. Early liver failure and cholestasis out of proportion for PN exposure may be a harbinger for gestational alloimmune liver disease (GALD), a potentially life-threatening condition that often requires liver transplant if not treated in a timely manner. This case report presents a premature neonate with il...

  16. Pulmonary squamous cell carcinoma associated with repaired congenital tracheoesophageal fistula and esophageal atresia.

    Science.gov (United States)

    Esquibies, Americo E; Zambrano, Eduardo; Ziai, James; Kesebir, Deniz; Touloukian, Robert J; Egan, Marie E; Reyes-Múgica, Miguel; Bazzy-Asaad, Alia

    2010-02-01

    We report a 19-year-old man with pulmonary squamous cell carcinoma (SCC) who had a history of vertebral, anal, cardiac, tracheal, esophageal, renal, and radial limb defects (VACTERL) association and tracheoesophageal fistula (TEF) + esophageal atresia (EA) repair as an infant. Children that undergo TEF + EA repair may have an increased risk for developing cancer as they reach adulthood. (c) 2010 Wiley-Liss, Inc.

  17. Thoracic skeletal anomalies following surgical treatment of esophageal atresia. Lessons from a national cohort.

    Science.gov (United States)

    Bastard, François; Bonnard, Arnaud; Rousseau, Véronique; Gelas, Thomas; Michaud, Laurent; Irtan, Sabine; Piolat, Christian; Ranke-Chrétien, Aline; Becmeur, François; Dariel, Anne; Lamireau, Thierry; Petit, Thierry; Fouquet, Virginie; Le Mandat, Aurélie; Lefebvre, Francis; Allal, Hossein; Borgnon, Josephine; Boubnova, Julia; Habonimana, Edouard; Panait, Nicoleta; Buisson, Philippe; Margaryan, Marc; Michel, Jean-Luc; Gaudin, Jean; Lardy, Hubert; Auber, Frédéric; Borderon, Corinne; De Vries, Philine; Jaby, Olivier; Fourcade, Laurent; Lecompte, Jean François; Tolg, Cécilia; Delorme, Benoit; Schmitt, Françoise; Podevin, Guillaume

    2017-07-21

    Thoracotomy as surgical approach for esophageal atresia treatment entails the risk of deformation of the rib cage and consequently secondary thoracogenic scoliosis. The aim of our study was to assess these thoracic wall anomalies on a large national cohort and search for factors influencing this morbidity. Pediatric surgery departments from our national network were asked to send recent thoracic X-ray and operative reports for patients born between 2008 and 2010 with esophageal atresia. The X-rays were read in a double-blind manner to detect costal and vertebral anomalies. Among 322 inclusions from 32 centers, 110 (34.2%) X-rays were normal and 25 (7.7%) displayed thoracic malformations, including 14 hemivertebrae. We found 187 (58.1%) sequelae of surgery, including 85 costal hypoplasia, 47 other types of costal anomalies, 46 intercostal space anomalies, 21 costal fusions and 12 scoliosis, with some patients suffering from several lesions. The rate of patients with these sequelae was not influenced by age at intervention, weight at birth, type of atresia, number of thoracotomy or size of the center. The rate of sequelae was higher following a classical thoracotomy (59.1%), whatever the way that thoracotomy was performed, compared to nonconverted thoracoscopy (22.2%; p=0.04). About 60 % of the patients suffered from a thoracic wall morbidity caused by the thoracotomy performed as part of surgical treatment of esophageal atresia. Minimally invasive techniques reduced thoracic wall morbidity. Further studies should be carried out to assess the potential benefit of minimally invasive approaches to patient pulmonary functions and on the occurrence of thoracogenic scoliosis in adulthood. Level III retrospective comparative treatment study. Copyright © 2017 Elsevier Inc. All rights reserved.

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

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    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.

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

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    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.

  20. Report of congenital colonobladder fitula with atresia ani in a lamb and treatment by surgery

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    Mohsen Vahar

    2015-06-01

    Full Text Available Colonobladder fistula and anal atresia, including congenital malformations in animals, occur due to genetics, environmental factors and vascular insufficiency. A male lamb was brought to a private veterinary clinic in Amol city, Mazandaran province, Iran. The lamb had clinical symptoms such as lethargy, dehydration, closed anus and watery feces in the genital organ. After taking radiograph and clinical examinations, it was found that the lamb was suffering from both colonobladder fistula and anal atresia; and emergency surgery was done. First, aseptic conditions and analgesia at the lumbosacral region of the trunk were put into practice and after cutting the abdominal muscles, accumulated feces in intestine were removed via an intestinal incision. The intestine was separated from the bladder via an incision and the bladder was sutured. Then, after creating an artificial hole in the base of the tail as the animal's new anus, the intestine was sutured to the original anus. At the end of surgery, serum therapy with sugar-salt dextrose 5% NaCl 0.9% solution and antibiotic therapy with penicillin and streptomycin were performed. This report is the first report of an unusual lamb with anal atresia and colonobladder fistula in the north of Iran. This surgery was achievable and is an obligatory surgery in affected animal rather than alternative euthanasia.

  1. New successful one-step surgical repair for apple peel atresia

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    Machmouchi M

    2011-11-01

    Full Text Available Mahmoud MachmouchiDepartment of Pediatrics, Royal Commission Hospital, Jubail, Saudi ArabiaAbstract: A new successful surgical approach in two identical twins delivered with intestinal atresia, “apple peel” type is reported. This technique consists of: (1 an end-to-end oblique primary anastomosis with single layer inverted 5/0 Vicryl® sutures (Ethicon, Inc, Somerville, NJ; (2 proximal tube jejunostomy using Foley catheter, size 10 French, inserted through a stab wound in the left upper quadrant and entering the proximal dilated loop at about 10 cm proximal from its blind end (site of anastomosis; and (3 transanastomotic stenting using feeding tube, size 6 French, exteriorized in conjunction with the Foley catheter and reaching the lumen of the distal loop for more than 20 cm. The postoperative course was uneventful and progressive oral feed became ad libitum around day 40 postoperative. This procedure is simple, performed in one stage, and responds to the most obligatory requirements of this congenital malformation.Keywords: proximal jejunal atresia, intestinal atresia, surgical repair, anastomosis

  2. Neonatal gastric outlet obstruction by isolated pyloric atresia, an often forgotten diagnosis.

    Science.gov (United States)

    Mboyo, Antoine; Clermidi, Pauline; Podevin, Guillaume; Patkowski, Dariusz; Baglaj, Maciej; Gerus, Sylwester; Lalioui, Abdelfetah; Napoli-Cocci, Stéphan de; Robert, Michel

    2016-04-01

    Pyloric atresia (PA) is a rare condition, and may be misdiagnosed and especially confused for duodenal atresia pre-operatively. We looked for clues to avoiding pre-operative misdiagnosis and hence allow the best neonatal medical and surgical management. A retrospective case-note review was carried out of the five patients managed in four centres with the diagnosis of isolated PA. We focused on antenatal ultrasound findings, postnatal clinical and radiological features, operative findings, surgical procedures and outcomes. Four patients had polyhydramnios and one double bubble sign on antenatal ultrasound. After birth, non-bilious vomiting and upper abdominal distension were the main symptoms. Gastric decompression showed non-bilious gastric fluid. Radiological findings were a large gastric air bubble with no gas beyond in all cases. The diagnosis of duodenal atresia was postulated at first in all cases. The diagnosis of PA was established peroperatively. One patient referred late, died 13-day post-operatively of cardiopulmonary failure secondary to a severe pneumonia that may be related to aspiration syndrome. Outcomes were otherwise satisfactory. Even though it is a rare diagnosis, PA has a specific clinical and radiological presentation underlined here that should be kept in mind when managing a neonate with a gastric outlet obstruction.

  3. A Tutorial on Implantable Hearing Amplification Options for Adults with Unilateral Microtia and Atresia

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    Joannie Ka Yin Yu

    2014-01-01

    Full Text Available Background. Patients with unilateral atresia and microtia encounter problems in sound localization and speech understanding in noise. Although there are four implantable hearing devices available, there is little discussion and evidence on the application of these devices on patients with unilateral atresia and microtia problems. Objective. This paper will review the details of these four implantable hearing devices for the treatment of unilateral atresia. They are percuteaneous osseointegrated bone anchored hearing aid, Vibrant Soundbridge middle ear implant, Bonebridge bone conduction system, and Carina fully implantable hearing device. Methods. Four implantable hearing devices were reviewed and compared. The clinical decision process that led to the recommendation of a device was illustrated by using a case study. Conclusions. The selection of appropriate implantable hearing devices should be based on various factors, including radiological findings and patient preferences, possible surgical complications, whether the device is Food and Drug Administration- (FDA-/CE-approved, and the finances. To ensure the accurate evaluation of candidacy and outcomes, the evaluation methods should be adapted to suite the type of hearing device.

  4. Complete occipitalization of the atlas with bilateral external auditory canal atresia.

    Science.gov (United States)

    Dolenšek, Janez; Cvetko, Erika; Snoj, Žiga; Meznaric, Marija

    2017-02-18

    Fusion of the atlas with the occipital bone is a rare congenital dysplasia known as occipitalization of the atlas, occipitocervical synostosis, assimilation of the atlas, or atlanto-occipital fusion. It is a component of the paraxial mesodermal maldevelopment and commonly associated with other dysplasias of the craniovertebral junction. External auditory canal atresia or external aural atresia is a rare congenital absence of the external auditory canal. It occurs as the consequence of the maldevelopment of the first pharyngeal cleft due to defects of cranial neural crest cells migration and/or differentiation. It is commonly associated with the dysplasias of the structures derived from the first and second pharyngeal arches including microtia. We present the coexistence of the occipitalization of the atlas and congenital aural atresia, an uncommon combination of the paraxial mesodermal maldevelopment, and defects of cranial neural crest cells. The association is most probably syndromic as minimal diagnostic criteria for the oculoariculovertebral spectrum are fulfilled. From the clinical point of view, it is important to be aware that patients with microtia must obtain also appropriate diagnostic imaging studies of the craniovetebral junction due to eventual concomitant occipitalization of the atlas and frequently associated C1-C2 instability.

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

    Science.gov (United States)

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

    2009-06-14

    To investigate the spatial distribution patterns of anorectal atresia/stenosis in China. Data were collected from the Chinese Birth Defects Monitoring Network (CBDMN), a hospital-based 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. 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. The findings in this research allow the display of the spatial distribution patterns of anorectal atresia

  6. Sex Differences Associated with Primary Biliary Cirrhosis

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    Daniel S. Smyk

    2012-01-01

    Full Text Available Primary biliary cirrhosis (PBC is a cholestatic liver disease of autoimmune origin, characterised by the destruction of small intrahepatic bile ducts. The disease has an unpredictable clinical course but may progress to fibrosis and cirrhosis. The diagnostic hallmark of PBC is the presence of disease-specific antimitochondrial antibodies (AMA, which are pathognomonic for the development of PBC. The disease overwhelmingly affects females, with some cases of male PBC being reported. The reasons underlying the low incidence of males with PBC are largely unknown. Epidemiological studies estimate that approximately 7–11% of PBC patients are males. There does not appear to be any histological, serological, or biochemical differences between male and female PBC, although the symptomatology may differ, with males being at higher risk of life-threatening complications such as gastrointestinal bleeding and hepatoma. Studies on X chromosome and sex hormones are of interest when studying the low preponderance of PBC in males; however, these studies are far from conclusive. This paper will critically analyze the literature surrounding PBC in males.

  7. Sex Differences Associated with Primary Biliary Cirrhosis

    Science.gov (United States)

    Smyk, Daniel S.; Rigopoulou, Eirini I.; Pares, Albert; Billinis, Charalambos; Burroughs, Andrew K.; Muratori, Luigi; Invernizzi, Pietro; Bogdanos, Dimitrios P.

    2012-01-01

    Primary biliary cirrhosis (PBC) is a cholestatic liver disease of autoimmune origin, characterised by the destruction of small intrahepatic bile ducts. The disease has an unpredictable clinical course but may progress to fibrosis and cirrhosis. The diagnostic hallmark of PBC is the presence of disease-specific antimitochondrial antibodies (AMA), which are pathognomonic for the development of PBC. The disease overwhelmingly affects females, with some cases of male PBC being reported. The reasons underlying the low incidence of males with PBC are largely unknown. Epidemiological studies estimate that approximately 7–11% of PBC patients are males. There does not appear to be any histological, serological, or biochemical differences between male and female PBC, although the symptomatology may differ, with males being at higher risk of life-threatening complications such as gastrointestinal bleeding and hepatoma. Studies on X chromosome and sex hormones are of interest when studying the low preponderance of PBC in males; however, these studies are far from conclusive. This paper will critically analyze the literature surrounding PBC in males. PMID:22693524

  8. [Pancreas and biliary tract: recent developments].

    Science.gov (United States)

    de-Madaria, Enrique

    2014-09-01

    Acute pancreatitis (AP) is a common disease that is associated with significant morbidity and considerable mortality. In this article, developments relating to this disease that were presented in DDW 2014 are reviewed. Pancreatic steatosis could be a cause of recurrent AP. Patients with DM have an increased incidence of AP and pancreatic cancer. The use of anti-TNF drugs in inflammatory bowel disease may protect against the occurrence of AP. The presence of pancreas divisum protects against acute biliary pancreatitis. The PANCODE system for describing local complications of AP has good interobserver agreement, when the new definitions of the revised Atlanta classification are applied. The use of prophylactic antibiotics in early-stage AP predisposes the development of intra-abdominal fungal infections. Fluid sequestration in AP is linked with young age, alcoholism and indicators of systemic inflammatory response syndrome. The most common cause of mortality in AP is early onset of multiple organ failure, not pancreatic necrosis infection. Patients with AP and vitamin D deficiency could benefit from taking vitamin D supplements. Moderate fluid administration in emergencies (500-1000 mL) could be associated with better AP development. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

  9. Primary cancers of extrahepatic biliary passages.

    Science.gov (United States)

    Mittal, B; Deutsch, M; Iwatsuki, S

    1985-04-01

    We analyzed the records of 22 patients with cancers of extrahepatic biliary passages (EHBP) to understand their natural histories and patterns of failure and to evaluate the effectiveness of various treatments. None of the preoperative investigations consistently defined the entire extent of tumor. Percutaneous transhepatic cholangiography (PTHC) was the most helpful (100%) in accurately defining the site of ductal obstruction. Computed tomography was helpful in diagnosing liver metastases in 53% and primary tumor mass in 23% of patients. The most common sites of tumor failure or persistence were: liver (67%), tumor bed (56%), peritoneum (22%), porta hepatis and lymph nodes (17%). The median survival for the entire group was 6.8 months. Surgery plays an important role in managing these tumors and in defining tumor extent for subsequent adjuvant irradiation. Patients receiving radiation doses greater than or equal to 70 TDF had a longer median survival (11 months) than patients receiving less than 70 TDF (4.4 months). All three patients, who were alive and free of disease greater than 1 year, received radiation doses greater than or equal to 70 TDF. From our data, it is difficult to comment on the effectiveness of chemotherapy. We have made suggestions regarding radiation volume and doses to various structures. The need for entering these patients into multi-institutional clinical trials is stressed.

  10. Primary cancers of extrahepatic biliary passages

    Energy Technology Data Exchange (ETDEWEB)

    Mittal, B.; Deutsch, M.; Iwatsuki, S.

    1985-04-01

    The records of 22 patients with cancers of extrahepatic biliary passages (EHBP) were analyzed to understand their natural histories and patterns of failure and to evaluate the effectiveness of various treatments. None of the preoperative investigations consistently defined the entire extent of tumor. Percutaneous transhepatic cholangiography (PTHC) was the most helpful (100%) in accurately defining the site of ductal obstruction. Computed tomography was helpful in diagnosing liver metastases in 53% and primary tumor mass in 23% of patients. The most common sites of tumor failure or persistence were: liver (67%), tumor bed (56%), peritoneum (22%), porta hepatis and lymph nodes (17%). The median survival for the entire group was 6.8 months. Surgery plays an important role in managing these tumors and in defining tumor extent for subsequent adjuvant irradiation. Patients receiving radiation doses greater than or equal to 70 TDF had a longer median survival (11 months) than patients receiving less than 70 TDF (4.4 months). All three patients, who were alive and free of disease greater than 1 year, received radiation doses greater than or equal to 70 TDF. From the data, it is difficult to comment on the effectiveness of chemotherapy. The authors have made suggestions regarding radiation volume and doses to various structures. The need for entering these patients into multi-institutional clinical trials is stressed.

  11. Ceftriaxone-associated biliary pseudolithiasis in children.

    Science.gov (United States)

    Biner, Betül; Oner, Naci; Celtik, Coşkun; Bostancioğlu, Musa; Tunçbilek, Nermin; Güzel, Ahmet; Karasalihoğlu, Serap

    2006-06-01

    Ceftriaxone is known to induce reversible precipitations, known as pseudolithiasis, in the gallbladder and urinary tract. The aim of this study was to investigate the incidence and predisposing factors that contribute to this side effect. A prospective study was conducted in 156 children admitted for the treatment of various infections with different daily ceftriaxone doses (50 mg/kg, 75 mg/kg, and 100 mg/kg). Sonographic examinations of the gallbladder and urinary tract were performed before treatment on the third and seventh day of therapy, and at the first and second month after the end of treatment. Patients with positive findings were followed with weekly sonographic examinations until the abnormality resolved. Abnormal gallbladder sonograms were demonstrated in 27 children (17%); 16 of them (10%) had gallbladder lithiasis, 11 had gallbladder sludge (7%) (n = 4 on the third day, n = 23 on the seventh day), and 1 developed urolithiasis (0.6%). Five children (19%) were symptomatic. The abnormalities resolved after a mean of 16 days (range 10-30 days). Patients with pseudolithiasis were older and treated with higher drug doses than those with normal sonographic findings (P Biliary pseudolithiasis (and infrequently nephrolithiasis) usually occurs in children receiving high doses of ceftriaxone. It is generally asymptomatic. When this reversible complication becomes symptomatic, unnecessary cholecystectomy should be avoided.

  12. A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis

    NARCIS (Netherlands)

    Nevens, Frederik; Andreone, Pietro; Mazzella, Giuseppe; Strasser, Simone I.; Bowlus, Christopher; Invernizzi, Pietro; Drenth, Joost P. H.; Pockros, Paul J.; Regula, Jaroslaw; Beuers, Ulrich; Trauner, Michael; Jones, David E.; Floreani, Annarosa; Hohenester, Simon; Luketic, Velimir; Shiffman, Mitchell; van Erpecum, Karel J.; Vargas, Victor; Vincent, Catherine; Hirschfield, Gideon M.; Shah, Hemant; Hansen, Bettina; Lindor, Keith D.; Marschall, Hanns-Ulrich; Kowdley, Kris V.; Hooshmand-Rad, Roya; Marmon, Tonya; Sheeron, Shawn; Pencek, Richard; MacConell, Leigh; Pruzanski, Mark; Shapiro, David; Angus, Peter; Roberts, Stuart; Vogel, Wolfgang; Graziadei, Ivo; de Lédinghen, Victor; Berg, Thomas; Gotthardt, Daniel; Hartmann, Heinz; Kremer, Andreas E.; Lammert, Frank; Manns, Michael P.; Rust, Christian; Schramm, Christoph; Trautwein, Christian; Zeuzem, Stefan; Carbone, Marco; van Nieuwkerk, Carin C. M. J.; Celinski, Krzysztof; Gonciarz, Maciej; Hartleb, Marek; Milkiewicz, Piotr; Parés, Albert; Bramley, Peter; Thorburn, Douglas; Mookerjee, Rajeshwar P.; Burroughs, Andrew; Chapman, Roger; Dillon, John F.; Greer, John A.; Tripathi, Dhiraj; McCune, Anne; Ryder, Stephen; Bacon, Bruce R.; Naik, Jahnavi; Wang, Lan Sun; Bodenheimer, Henry C.; Bowlus, Christopher L.; Chalasani, Naga; Forman, Lisa M.; Gordon, Stuart C.; Luketic, Velimir A.; Mayo, Marlyn; Muir, Andrew J.; Reddy, K. Gautham; Talwalker, Jayant T.; Vierling, John M.

    2016-01-01

    BACKGROUND Primary biliary cholangitis ( formerly called primary biliary cirrhosis) can progress to cirrhosis and death despite ursodiol therapy. Alkaline phosphatase and bilirubin levels correlate with the risk of liver transplantation or death. Obeticholic acid, a farnesoid X receptor agonist, has

  13. A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis

    NARCIS (Netherlands)

    Nevens, F.; Andreone, P.; Mazzella, G.; Strasser, S.I.; Bowlus, C.; Invernizzi, P.; Drenth, J.P.H.; Pockros, P.J.; Regula, J.; Beuers, U.; Trauner, M.; Jones, D.E.; Floreani, A.; Hohenester, S.; Luketic, V.; Shiffman, M.; Erpecum, K.J. van; Vargas, V.; Vincent, C.; Hirschfield, G.M.; Shah, H.; Hansen, B.; Lindor, K.D.; Marschall, H.U.; Kowdley, K.V.; Hooshmand-Rad, R.; Marmon, T.; Sheeron, S.; Pencek, R.; MacConell, L.; Pruzanski, M.; Shapiro, D.

    2016-01-01

    BACKGROUND: Primary biliary cholangitis (formerly called primary biliary cirrhosis) can progress to cirrhosis and death despite ursodiol therapy. Alkaline phosphatase and bilirubin levels correlate with the risk of liver transplantation or death. Obeticholic acid, a farnesoid X receptor agonist, has

  14. A Placebo-controlled trial of obeticholic acid in primary biliary cholangitis

    NARCIS (Netherlands)

    F. Nevens (F.); P. Andreone (Pietro); Mazzella, G.; S. Strasser (Simone); C.L. Bowlus (Christopher L.); P. Invernizzi (Pietro); J.P.H. Drenth (Joost); Pockros, P.J.; J. Regula (J.); U. Beuers (Ulrich); Trauner, M.; D.E.J. Jones (David); A. Floreani (Annarosa); Hohenester, S.; Luketic, V.; R.E. Peccei (Riccardo); K.J. van Erpecum (Karel); Vargas, V.; C. Vincent (Catherine); G.M. Hirschfield (Gideon); Shah, H.; B.E. Hansen (Bettina); K.D. Lindor (Keith); H.-U. Marschall; Kowdley, K.V.; Hooshmand-Rad, R.; Marmon, T.; Sheeron, S.; Pencek, R.; MacConell, L.; Pruzanski, M.; Shapiro, D.

    2016-01-01

    textabstractBACKGROUND Primary biliary cholangitis (formerly called primary biliary cirrhosis) can progress to cirrhosis and death despite ursodiol therapy. Alkaline phosphatase and bilirubin levels correlate with the risk of liver transplantation or death. Obeticholic acid, a farnesoid X receptor

  15. Endoscopic treatment of acute biliary pancreatitis: A national survey among Dutch gastroenterologists

    NARCIS (Netherlands)

    van Geenen, Erwin-Jan M.; Mulder, Chris J. J.; van der, Donald L.; Fockens, Paul; Bruno, Marco J.

    2010-01-01

    Objective. Based on the ampullary obstruction and reflux theory, six endoscopic retrograde cholangiopancreatography (ERCP) studies have investigated the effect of (early) biliary decompression versus conservative management on the course and outcome of patients with acute biliary pancreatitis (ABP)

  16. Endoscopy Ultra- Sonography -guided biliary drainage in the surgical -endoscopy era

    National Research Council Canada - National Science Library

    Artifon, Everson L A; Pinhata Otoch, Jose; Yábar, Alejandro; Poli-de-Figueiredo, Luiz F; Sakai, Paulo; Rasslan, Samir

    2011-01-01

    US-guided hepatico - gastrostomy, choledocho-duodenostomy and choledocho-antrostomy are advanced procedures on biliary and pancreatic endoscopy and together make up the echo-guided biliary drainage...

  17. Usefulness of Endoscopic Managements in Patients with Ceftriaxone-Induced Pseudolithiasis Causing Biliary Obstruction

    Directory of Open Access Journals (Sweden)

    Yasuhiro Doi

    2017-01-01

    Full Text Available Ceftriaxone (CTRX is known to cause reversible biliary stones/sludge, which is called biliary pseudolithiasis. We report two rare cases of biliary obstruction by pseudolithiasis shortly after completing CTRX treatment. Stones and sludge, which had not been detected before CTRX administration, appeared in the gallbladder and common bile duct and led to biliary obstruction and acute cholangitis. The obstructions were successfully treated with endoscopic retrograde biliary drainage and endoscopic sphincterotomy. CTRX-induced biliary pseudolithiasis has been reported mainly in children and adolescents but is also seen in adults with similar incidence rate. Although CTRX-induced biliary pseudolithiasis is usually asymptomatic and disappears spontaneously after discontinuing the drug, some patients develop biliary obstruction. Endoscopic managements should be considered in such cases.

  18. Endoscopic ultrasound-guided biliary drainage versus percutaneous transhepatic biliary drainage after failed endoscopic retrograde cholangiopancreatography: a meta-analysis

    Directory of Open Access Journals (Sweden)

    Baniya R

    2017-04-01

    Full Text Available Ramkaji Baniya, Sunil Upadhaya, Seetharamprasad Madala, Subash Chandra Subedi, Tabrez Shaik Mohammed, Ghassan Bachuwa Hurley Medical Center, Michigan State University, Flint, MI, USA Abstract: The failure rate of endoscopic retrograde cholangiopancreatography for biliary cannulation is approximately 6%–7% in cases of obstructive jaundice. Percutaneous transhepatic biliary drainage (PTBD is the procedure of choice in such cases. Endoscopic ultrasound-guided biliary drainage (EGBD is a novel technique that allows biliary drainage by echoendoscopy and fluoroscopy using a stent from the biliary tree to the gastrointestinal tract. Information in PubMed, Scopus, clinicaltrials.gov and Cochrane review were analyzed to obtain studies comparing EGBD and PTBD. Six studies fulfilled the inclusion criteria. Technical (odds ratio (OR: 0.34; confidence interval (CI 0.10–1.14; p=0.05 and clinical (OR: 1.48; CI 0.46–4.79; p=0.51 success rates were not statistically significant between the EGBD and PTBD groups. Mild adverse events were nonsignificantly different (OR: 0.36; CI 0.10–1.24; p=0.11 but not the moderate-to-severe adverse events (OR: 0.16; CI 0.08–0.32; p≤0.00001 and total adverse events (OR: 0.34; CI 0.20–0.59; p≤0.0001. EGBD is equally effective but safer than PTBD. Keywords: failed endoscopic retrograde cholangiopancreatography, percutaneous transhepatic biliary drainage, endoscopic ultrasound-guided biliary drainage, technical success, clinical success

  19. The history of oesophageal atresia and tracheo-oesophageal fistula--1670-1984.

    Science.gov (United States)

    Myers, N A

    1986-01-01

    The history of oesophageal atresia commenced in the year 1670 with Durston's description of "A narrative of a monstrous birth at Plymouth". However, the most significant contribution in the 17th century was made by Gibson, who clearly described the clinical picture and necropsy findings in a baby with a proximal oesophageal atresia and a distal tracheo-oesophageal fistula. The 18th century was singularly lacking in contributions to the literature, but there were many important presentations during the 19th century. Early in that century Martin in France and Hill in United States of America contributed to the literature; subsequently, many anecdotal references can be found particularly in England, and ultimately a surgical attempt to correct the anomaly was made in 1888 by Steele in London. With this, the pre-surgical era gave way to the surgical era, and at a later stage pre-survival era was to give way to the survival phase. Reference will be made in the text to these various phases in the history and to the many pioneering surgical endeavours which took place in the earlier years of the 20th century. It will be seen that the surgical efforts employed palliative procedures, indirect attacks on the problem and finally direct attacks. The classic successes by indirect methods which were achieved by Ladd and Leven in babies born in 1939 were to be followed by the first successful end-to-end anastomosis which was achieved in Ann Arbor, Michigan, by Cameron Haight. Others had attempted primary anastomosis, and Lanman in particular deserves mention. However, it was Cameron Haight who opened a new era in the history of the treatment of oesophageal atresia; the recent history is largely concerned with increasing experience and refinements in technique. For the future historian many pathways can still be explored, including oesophageal replacement, the unstable trachea, follow-up studies and research projects. This history also included comments on the history of the less

  20. Bone-anchored hearing aids (Baha) in congenital aural atresia: personal experience.

    Science.gov (United States)

    Ricci, Giampietro; Volpe, Antonio Della; Faralli, Mario; Longari, Fabrizio; Lancione, Carla; Varricchio, Alfonso Maria; Frenguelli, Antonio

    2011-03-01

    Aural atresia is a congenital disease constituted by partial or complete lack of development of the external auditory canal, which is generally associated with malformations of the auricle and middle ear. Reconstruction of the auditory canal and correction of any deformities of the middle ear have yielded unpredictable results and variable functional outcomes, and there is a high rate of complications. Therefore, the use of bone-conduction hearing aids, such as the Baha, may represent a valid alternative for subjects who have conductive hearing loss with cochlear reserve that, as a rule, is fully conserved. The aim of this work is to reexamine our experience with the management of conductive and mixed hearing loss using the Baha system in children with bilateral aural atresia. We examined 31 patients with bilateral congenital aural atresia in whom a Baha system had been implanted. The patients, 16 males and 15 females, were between 5 and 14 years of age (mean 8.7). The following parameters were assessed for each patient: mean preoperative air and bone conduction for frequencies between 0.5 and 4kHz; mean preoperative threshold with conventional bone-conduction hearing aids; mean postoperative threshold with the Baha system; improvement in quality of life evaluated with the Glasgow Children's Benefit Inventory; rate and type of surgical complications. The mean preoperative air- and bone-conduction thresholds were 51.2±12.5 and 14.1±6.3dB HL, respectively. The mean preoperative threshold with a conventional bone-conduction hearing aid was 29.3±7.2dB HL, and the mean postoperative threshold with the Baha system was 18.1±7.5dB HL. Quality of life improved for all operated patients. The results of our study of the Baha system to treat patients with bilateral aural atresia were extremely satisfactory compared both with those of surgical reconstruction of the auditory canal and those of traditional bone-conduction hearing aids. Furthermore, great improvement was noted

  1. Biliary Surgery Via Minilaparotomy — A Limited Procedure for Biliary Lithiasis

    Directory of Open Access Journals (Sweden)

    Takukazu Nagakawa

    1993-01-01

    Full Text Available Cholelithiasis until now has been treated using solvents, lithotripsy via a biliary endoscope, laser or shock wave lithotripsy, and laparoscopic cholecystectomy. have developed a new surgical treatment for cholelithiasis in which a cholecystectomy is performed through a minilaparotomy. This paper presents this new technique and discusses the principles of surgery for cholelithiasis using this technique. This procedure is performed by a 2 to 3 cm subcostal skin incision in the right hypochondrium. More than 400 patients were treated by this technique. This procedure is not different in terms of blood loss .or operation time from conventional methods, and no significant complications have occurred. Intraoperative X-ray examination is performed routinely because of easy insertion of a tube from the cystic duct into the bile duct. Reduction of the length of the incision greatly facilitates postoperative recovery, shortening the hospital length-of-stay to within 3 days. The surgical manipulation of only a limited area of the upper abdomen is unlikely to induce postoperative syndromes, such as adhesions or ileus. Following this experience, a biliary drainage procedure based on cholangionmanomery and primary closure of the choledochotomy was introduced. This approach allowed even patients with choledocholithiasis to undergo a minilaparotomy and be discharged within one week.

  2. Sclerosing Cholangitis and Primary Biliary Cirrhosis—a Disease Spectrum?

    Science.gov (United States)

    Fee, Henry J.; Gewirtz, Harold; Schiller, Juan; Longmire, William P.

    1977-01-01

    Sclerosing diseases of the biliary system encompass a spectrum ranging from primary sclerosing cholangitis (usually of the extrahepatic biliary tree) to primary biliary cirrhosis of the intrahepatic bile canaliculi. In a study of 35 patients with primary intra- and extrahepatic biliary sclerosis, age of onset, sex distribution, symptomatology, associated diseases, radiographic abnormalities and chemical profile were considered. The difficulty of differentiating sclerosing cholangitis and biliary cirrhosis from other causes of obstructive jaundice preoperatively was stressed, in addition to points of differential clinical and laboratory findings. The etiology of these entities as well as the possibility that they represent variant clinical manifestations of the same disease process were also considered. Mechanical and pharmacological treatment alternatives that were attempted included drainage procedures, the easiest and most widely used of which was the T-tube. However, this could prove to be a source of infection and should therefore be removed early, inasmuch as cholangitis represents a major cause of morbidity. Steroids have been used with varying effectiveness; subjective improvement was generally attained, although objective improvement has been difficult to document. When choleuretics and cholestyramine were administered, we noted significant palliation. Antibiotics were reserved for treatment of cholangitis. Until the underlying etiology of this rare malignant sclerosing process is found, only symptomatic treatment can be offered. PMID:921353

  3. Linking human beta retrovirus infection with primary biliary cirrhosis.

    Science.gov (United States)

    Mason, A L; Zhang, G

    2010-01-01

    Several environmental agents have been linked with primary biliary cirrhosis (PBC) that include bacteria, xenobiotics and viruses. A human beta retrovirus (HBRV) related to mouse mammary tumor virus has been cloned and characterized from patients with PBC. This agent can be detected in the majority of patients' perihepatic lymph nodes by immunochemistry and RT-PCR. The HBRV has recently been isolated in culture and integration sites have been identified in the genome of patients to provide convincing evidence of beta retrovirus infection in patients. Three lines of evidence support a role for the virus in PBC. First, the beta retrovirus is linked with aberrant expression of mitochondrial protein(s) on the biliary epithelium cell (BEC) surface, a disease specific phenotype. Second, the related agent, mouse mammary tumor virus has been linked with autoimmune biliary disease in the NOD.c3c4 mouse model for PBC. In this mouse model, the virus is localized to diseased biliary epithelium that also display aberrant expression of the mitochondrial autoantigens. In translational studies, both patients with PBC and NOD.c3c4 mice demonstrate significant improvement in biliary disease with combination antiviral therapy. An overview of the biological relevance of the beta retrovirus infection in PBC will be discussed in this review. Copyright 2010 Elsevier Masson SAS. All rights reserved.

  4. [Application of endoscopic ultrasound in diagnostics of biliary lithiasis].

    Science.gov (United States)

    Mesihović, Rusmir; Gribajcević, Mehmed; Masić, Izet

    2006-01-01

    Endoscopic ultrasound (EUS) and Magnetic Endoscopic Cholangiopancreatography (MRCP) are important supplementary methods used for endoscopic extraction of common bile duct stones. The technical characteristics of both methods are excellent. If EUS is used for diagnostic purposes in diagnostic of biliary tree disorders there are almost no adverse consequences compared with other indication for this method. Nevertheless, the results are related to experience of physician. Advance of EUS compared to MRCP is its sensitivity and specificity even in cases where stones are small and dilation of biliary tract is minor. The role of EUS and ERCP in reveal of biliary tree stones and disorders is clearly defined. Comparasion of these two methods should be prospective and used in cases where definitive diagnosis is unclear. If EUS reveal stones in biliary tree then ERCP should be done in same session. Beside its role in excluding biliary pancreatitis, EUS can be used for examination of patient with acute and recurrent pancreatitis and is excellent for revealing ductal and parenchymal abnormalities of pancreas.

  5. Management of post-surgical biliary leakage with percutaneous transhepatic biliary drainage (PTBD) and occlusion balloon (OB) in patients without dilatation of the biliary tree: preliminary results

    Energy Technology Data Exchange (ETDEWEB)

    Pedicini, Vittorio; Poretti, Dario; Brambilla, Giorgio [Humanitas Clinical Institute, Department of Diagnostic Imaging, Rozzano, Milan (Italy); Mauri, Giovanni; Trimboli, Manuela [Humanitas Clinical Institute, Department of Diagnostic Imaging, Rozzano, Milan (Italy); University of Milan School of Medicine, Unit of Radiology, Ospedale San Paolo, Milan (Italy); Sconfienza, Luca Maria; Sardanelli, Francesco [University of Milan School of Medicine, Unit of Radiology, IRCCS Policlinico San Donato, Department of Medical and Surgical Sciences, Milan (Italy); Cornalba, Gianpaolo [University of Milan School of Medicine, Unit of Radiology, Ospedale San Paolo, Milan (Italy)

    2010-05-15

    To compare the outcome of patients affected by biliary leak after major biliary surgery and treated with percutaneous transhepatic biliary drainage (PTBD) alone with that of similar patients treated with PTBD and concurrent positioning of an occlusion balloon (PTBD-OB). We retrospectively reviewed the results of the use of PTBD or PTBD-OB performed at our institution from 2004 to 2008 in patients with post-surgical biliary leak. Sixteen patients entered the evaluation. PTDB alone was performed in 9 men (age 59.7 {+-} 13.4 years [mean {+-} standard deviation]; median 60 years), while PTBD-OB was performed in 7 patients (5 men, 2 women, age 71.3 {+-} 9.3 years; median 68 years). No significant difference between groups was found regarding age (p = 0.064) and sex (p = 0.175) distribution, number of procedures (3.1 {+-} 1.62; 2 vs 1.7 {+-} 1.1; 1, p = 0.151), and days of disease before recovery, death or modification of treatment (51.6 {+-} 66.8; 23 vs 18.6 {+-} 15.1; 14, p = 0.266). The number of patients treated with PTBD-OB who fully recovered (7/7, 100%) was significantly higher than that of patients treated with PTBD alone (4/9, 44%, p = 0.034). This procedure appears to be clinically effective, being associated with a higher probability of recovery in patients treated for post-surgical biliary leak. Further studies are needed to confirm these preliminary results. (orig.)

  6. Management of post-surgical biliary leakage with percutaneous transhepatic biliary drainage (PTBD) and occlusion balloon (OB) in patients without dilatation of the biliary tree: preliminary results.

    Science.gov (United States)

    Pedicini, Vittorio; Poretti, Dario; Mauri, Giovanni; Trimboli, Manuela; Brambilla, Giorgio; Sconfienza, Luca Maria; Cornalba, Gianpaolo; Sardanelli, Francesco

    2010-05-01

    To compare the outcome of patients affected by biliary leak after major biliary surgery and treated with percutaneous transhepatic biliary drainage (PTBD) alone with that of similar patients treated with PTBD and concurrent positioning of an occlusion balloon (PTBD-OB). We retrospectively reviewed the results of the use of PTBD or PTBD-OB performed at our institution from 2004 to 2008 in patients with post-surgical biliary leak. Sixteen patients entered the evaluation. PTDB alone was performed in 9 men (age 59.7 +/- 13.4 years [mean +/- standard deviation]; median 60 years), while PTBD-OB was performed in 7 patients (5 men, 2 women, age 71.3 +/- 9.3 years; median 68 years). No significant difference between groups was found regarding age (p = 0.064) and sex (p = 0.175) distribution, number of procedures (3.1 +/- 1.62; 2 vs 1.7 +/- 1.1; 1, p = 0.151), and days of disease before recovery, death or modification of treatment (51.6 +/- 66.8; 23 vs 18.6 +/- 15.1; 14, p = 0.266). The number of patients treated with PTBD-OB who fully recovered (7/7, 100%) was significantly higher than that of patients treated with PTBD alone (4/9, 44%, p = 0.034). This procedure appears to be clinically effective, being associated with a higher probability of recovery in patients treated for post-surgical biliary leak. Further studies are needed to confirm these preliminary results.

  7. Biliary cystadenoma and cystadenocarcinoma; Ultrasound, CT and Angiography

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Byung Ihn; Han, Man Chung; Kim, Chu Wan [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1984-03-15

    Biliary cystadenoma and cystadenocarcinoma are rare neoplasms of the biliary ductal system. The authors present 2 cases of biliary cystadenoma and 3 cases of cystadenocarcinoma. The typical sonographic appearance of these tumors is a large intrahepatic globular or ovoid thick walled cystic mass which often contains multiple septations with papillary growth and solid portion. Low-level internal echoes may be seen within the cystic mass. The characteristic CT findings of these tumors are multiloculated cystic mass, thick septations with papillary projections and solid portion within the cystic mass. Angiographical characteristics of these tumors are hypovascular mass with frequent abnormal clusters of tumor vessels within the walls on arterial phase and accumulation of contrast material along the wall or internal septation on delayed film.

  8. Twelve-Year-Old Girl with Primary Biliary Cirrhosis

    Directory of Open Access Journals (Sweden)

    Ivana Kitic

    2012-01-01

    Full Text Available Primary biliary cirrhosis (PBC is a slowly progressive cholestatic liver disease of autoimmune etiology. The initial presentation of PBC is varies from asymptomatic, abnormal liver biochemical tests to overt cirrhosis. Unlike other autoimmune liver diseases, PBC has rarely been reported in childhood. We report a case of primary biliary cirrhosis in a 12-year-old girl. In addition to characteristic histology features, strongly positive antimitochondrial antibodies, increased liver enzyme levels, increased serum quantitative immunoglobulin M levels, and cholestasis were discovered. She had been treated with ursodeoxycholic acid. In the world literature, we found only few pediatric patients of primary biliary cirrhosis. Aetiology, pathogenesis, the long-term natural history, and prognosis remain obscure. Due to increased awareness of early-onset PBC, rather than typical older ones, further pediatric cases may be discovered.

  9. Minimally Invasive Management of Acute Biliary Tract Disease during Pregnancy

    Directory of Open Access Journals (Sweden)

    Luis Tomás Chiappetta Porras

    2009-01-01

    Full Text Available Background. Acute biliary diseases during pregnancy have been classically managed conservatively. Advances in minimally invasive surgery and the high recurrence rate of symptoms observed changed this management. Methods. This is a prospective observational study. Initial management was medical. Unresponsive patients were treated with minimally invasive techniques including gallbladder percutaneous aspiration or cholecystostomy, endoscopic retrograde cholangiography, and laparoscopic cholecystectomy, depending on the pregnancy trimester and underlying diagnosis. Results. 122 patients were admitted. 69 (56.5% were unresponsive to medical treatment. Recurrent gallbladder colic was the most frequent indication for minimally invasive intervention, followed by acute cholecystitis, choledocholithiasis, and acute biliary pancreatitis. 8 patients were treated during the first trimester, 54 during the second, and 7 during the last trimester. There was no fetal morbidity or mortality. Maternal morbidity was minor with no mortality. Conclusion. Acute biliary tract diseases during pregnancy may be safely treated with minimally invasive procedures according to the underlying diagnosis and to the trimester of pregnancy.

  10. Biliary Lithiasis: Prevalence and Ultrasound Profile in a Service Hospital.

    Science.gov (United States)

    Debnath, J; Chakraborty, I; Mohan, R

    2003-01-01

    Real time gray scale ultrasonography of upper abdomen was carried out in 1237 cases for varied indications. Overall prevalence of biliary lithiasis was 11.56% with female to male ratio of 4:1. Fifty years and below, female to male ratio was 6.5:1. 88.8% cases of biliary lithiasis were 60 years and below. Gall bladder dyspepsia (61.5%) and right upper quadrant pain (41%) were the main presenting features of biliary lithiasis. 26 patients (18%) presented with acute abdomen. Asymptomatic gallstones were found in 25 (17.5%) cases. Solitary calculus was less common than multiple calculi with a ratio of 1:4. Amongst cases of multiple calculi the small sized (2-4 mm) variety was the maximum (49%) followed by medium sized (5-10mm) and large sized (>10mm) calculi respectively.

  11. [Transient biliary lithiasis associated with the use of ceftriaxone].

    Science.gov (United States)

    Alvarez-Coca González, J; Cebrero García, M; Vecilla Rivelles, M C; Alonso Cristobo, M; Torrijos Roman, C

    2000-10-01

    Up to 40% of ceftriaxone is excreted unchanged into the bile and, due to its high calcium-binding affinity, it may form a salt that can provoke biliary lithiasis. Echography revealed that biliary lithiasis was present in 12-45% of patients treated with ceftriaxone as early as the second day of treatment. Lithiasis is usually asymptomatic and disappears in less than 2 months. Symptomatic cases and patients requiring cholecystectomy have been described in the literature. We present four children, evaluated in 1999, who presented asymptomatic cholelithiasis between the second and fourth day of ceftriaxone treatment. Biliary lithiasis was found after 2-4 days of treatment, with resolution of the lithiasis between 1-4 months after the end of therapy.

  12. Long-Term Outcomes of Patients with Tracheoesophageal Fistula/Esophageal Atresia: Survey Results from Tracheoesophageal Fistula/Esophageal Atresia Online Communities.

    Science.gov (United States)

    Acher, Charles Wynn; Ostlie, Daniel J; Leys, Charles M; Struckmeyer, Shannon; Parker, Matthew; Nichol, Peter F

    2016-12-01

    Introduction Outcome studies of tracheoesophageal fistula (TEF) and/or esophageal atresia (EA) are limited to retrospective chart reviews. This study surveyed TEF/EA patients/parents engaged in social media communities to determine long-term outcomes. Materials and Methods A 50-point survey was designed to study presentation, interventions, and ongoing symptoms after repair in patients with TEF/EA. It was validated using a test population and made available on TEF/EA online communities. Results In this study, 445 subjects completed the survey during a 2-month period. Mean age of patients when surveyed was 8.7 years (0-61 years) and 56% were male. Eighty-nine percent of surveys were completed by the parent of the patient. Sixty-two percent of patients underwent repair in the first 7 days of life. Standard open repair was most common (56%), followed by primary esophageal replacement (13%) and thoracoscopic repair (13%). Out of 405, 106 (26%) patients had postoperative leak. Postoperative leak was least likely in primary esophageal replacement (18%) and standard open repair (19%). Leak occurred in 32% of patients who had thoracoscopic repair; 31% (128/413) reported long-gap atresia, which was significantly associated with increased risk of postoperative leak (54/128, 42%) when compared with standard short-gap atresia (odds ratio, 3.5; p = 0.001). Out of 409, 221 (54%) patients reported dysphagia after repair, with only 77/221 (34.8%) reporting resolution by age 5. Out of 381, 290 (76%) patients reported symptoms of gastroesophageal reflux disease (GERD). There was no difference in dysphagia rates or GERD symptoms based on type of initial repair. Antireflux surgery was required in 63/290, 22% of patients with GERD (15% of all patients) and 27% of these patients who had surgery required more than one procedure antireflux procedure. The most common was Nissen fundoplication (73%), followed by partial wrap (14%). Reflux recurred in 32% of patients after

  13. Biweekly cisplatin and gemcitabine in patients advanced biliary tract cancer.

    Science.gov (United States)

    Ahn, Daniel H; Reardon, Josh; Ahn, Chul W; Bupathi, Manojkumar; Mikhail, Sameh; Wu, Christina Sing-Ying; Bekaii-Saab, Tanios

    2017-11-07

    Treatment with cisplatin with gemcitabine (CG) demonstrates a survival benefit in patients with advanced biliary tract cancer (ABTC). However, the weekly administration can add significant toxicities that may prohibit prolonged treatment. Based on previous studies, we implemented a modified biweekly regimen of GC in an attempt to optimize the prescribed regimen with an improved toxicity profile, added convenience to patients while maintaining efficacy. Patients with ABTC were treated with fixed dose rate (FDR) gemcitabine (1000mg/m(2) /min) and cisplatin 20mg/m(2) on days 1 and 15 of every 28-day cycle. Patients received treatment until time of progression, death or discontinuation due to intolerance. Collected data included demographics, clinico-pathologic features, toxicities and survival. Kaplan-Meier curves were used to calculate the median overall survival (OS) and progression free survival (PFS). The study included 107 evaluable pts with unresectable ABTC who received the biweekly regimen. Sites of tumor included gallbladder (21.5%), ampullary (3.7%) and bile duct (74.8%). Median number of cycles was 6 (1-27). Median PFS was 8.34 (6.74, 9.23) months and median OS was 10.32 (9.10, 11.43) months. Most common grade ≥3 adverse events included neutropenia (11%), fatigue (10%) and thrombocytopenia (6.4%). Biweekly FDR GC in ABTC is associated with a more favorable toxicity profile while maintaining efficacy similar to that observed in prior clinical trials. Minimal toxicities were observed despite a prolonged course for many patients. Further prospective trials should consider evaluating the role of biweekly GC regimen in ABTC, including a potentially more favorable platform in novel experimental strategies. This article is protected by copyright. All rights reserved. © 2017 UICC.

  14. Diet and biliary tract cancer risk in Shanghai, China.

    Science.gov (United States)

    Nelson, Shakira M; Gao, Yu-Tang; Nogueira, Leticia M; Shen, Ming-Chang; Wang, Bingsheng; Rashid, Asif; Hsing, Ann W; Koshiol, Jill

    2017-01-01

    Trends in biliary tract cancer incidence rates have increased in Shanghai, China. These trends have coincided with economic and developmental growth, as well as a shift in dietary patterns to a more Westernized diet. To examine the effect of dietary changes on incident disease, we evaluated associations between diet and biliary tract cancers amongst men and women from a population-based case-control study in Shanghai, China. Biliary tract cancer cases were recruited from 42 collaborating hospitals in urban Shanghai, and population-based controls were randomly selected from the Shanghai Household Registry. Food frequency questionnaire data were available for 225 gallbladder, 190 extrahepatic bile duct, and 68 ampulla of Vater cancer cases. A total of 39 food groups were created and examined for associations with biliary tract cancer. Interestingly, only four food groups demonstrated a suggested association with gallbladder, extrahepatic bile duct, or ampulla of Vater cancers. The allium food group, consisting of onions, garlic, and shallots showed an inverse association with gallbladder cancer (OR: 0.81, 95% CI: 0.68-0.97). Similar trends were seen in the food group containing seaweed and kelp (OR: 0.79, 95% CI: 0.67-0.96). In contrast, both preserved vegetables and salted meats food groups showed positive associations with gallbladder cancer (OR:1.27, 95% CI: 1.06-1.52; OR: 1.18, 95% CI: 1.02-1.37, respectively). Each of these four food groups showed similar trends for extrahepatic bile duct and ampulla of Vater cancers. The results of our analysis suggest intake of foods with greater anti-inflammatory properties may play a role in decreasing the risk of biliary tract cancers. Future studies should be done to better understand effects of cultural changes on diet, and to further examine the impact diet and inflammation have on biliary tract cancer incidence.

  15. Diet and biliary tract cancer risk in Shanghai, China.

    Directory of Open Access Journals (Sweden)

    Shakira M Nelson

    Full Text Available Trends in biliary tract cancer incidence rates have increased in Shanghai, China. These trends have coincided with economic and developmental growth, as well as a shift in dietary patterns to a more Westernized diet. To examine the effect of dietary changes on incident disease, we evaluated associations between diet and biliary tract cancers amongst men and women from a population-based case-control study in Shanghai, China. Biliary tract cancer cases were recruited from 42 collaborating hospitals in urban Shanghai, and population-based controls were randomly selected from the Shanghai Household Registry. Food frequency questionnaire data were available for 225 gallbladder, 190 extrahepatic bile duct, and 68 ampulla of Vater cancer cases. A total of 39 food groups were created and examined for associations with biliary tract cancer. Interestingly, only four food groups demonstrated a suggested association with gallbladder, extrahepatic bile duct, or ampulla of Vater cancers. The allium food group, consisting of onions, garlic, and shallots showed an inverse association with gallbladder cancer (OR: 0.81, 95% CI: 0.68-0.97. Similar trends were seen in the food group containing seaweed and kelp (OR: 0.79, 95% CI: 0.67-0.96. In contrast, both preserved vegetables and salted meats food groups showed positive associations with gallbladder cancer (OR:1.27, 95% CI: 1.06-1.52; OR: 1.18, 95% CI: 1.02-1.37, respectively. Each of these four food groups showed similar trends for extrahepatic bile duct and ampulla of Vater cancers. The results of our analysis suggest intake of foods with greater anti-inflammatory properties may play a role in decreasing the risk of biliary tract cancers. Future studies should be done to better understand effects of cultural changes on diet, and to further examine the impact diet and inflammation have on biliary tract cancer incidence.

  16. 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)....

  17. Non-VACTERL-type anomalies are frequent in patients with esophageal atresia/tracheo-esophageal fistula and full or partial VACTERL association.

    NARCIS (Netherlands)

    Jong, E.M. de; Felix, J.F.; Deurloo, J.A.; Dooren, M.F. van; Aronson, D.C.; Torfs, C.P.; Heij, H.A.; Tibboel, D.

    2008-01-01

    BACKGROUND: The VACTERL association is the nonrandom co-occurrence of Vertebral anomalies, Anal atresia, Cardiovascular malformations, Tracheo-esophageal fistula (TEF) and/or Esophageal atresia (EA), Renal anomalies, and/or Limb-anomalies. The full phenotype of patients with EA/TEF and other

  18. Pulmonary blood supply by a branch from the distal ascending aorta in pulmonary atresia with ventricular septal defect: differential diagnosis of fifth aortic arch.

    Science.gov (United States)

    Yoo, S J; Moes, C A; Burrows, P E; Molossi, S; Freedom, R M

    1993-10-01

    A patient with pulmonary atresia and a ventricular septal defect is described in whom an arterial branch from the distal ascending aorta supplied segments of both lungs. The branch is considered to represent a persistent fifth aortic arch. The possible morphogenesis and differential diagnosis of a communication between the ascending aorta and the pulmonary artery in pulmonary atresia with ventricular septal defect are discussed.

  19. Identification and manipulation of biliary metaplasia in pancreatic tumors.

    Science.gov (United States)

    Delgiorno, Kathleen E; Hall, Jason C; Takeuchi, Kenneth K; Pan, Fong Cheng; Halbrook, Christopher J; Washington, M Kay; Olive, Kenneth P; Spence, Jason R; Sipos, Bence; Wright, Christopher V E; Wells, James M; Crawford, Howard C

    2014-01-01

    Metaplasias often have characteristics of developmentally related tissues. Pancreatic metaplastic ducts are usually associated with pancreatitis and pancreatic ductal adenocarcinoma. The tuft cell is a chemosensory cell that responds to signals in the extracellular environment via effector molecules. Commonly found in the biliary tract, tuft cells are absent from normal murine pancreas. Using the aberrant appearance of tuft cells as an indicator, we tested if pancreatic metaplasia represents transdifferentiation to a biliary phenotype and what effect this has on pancreatic tumorigenesis. We analyzed pancreatic tissue and tumors that developed in mice that express an activated form of Kras (Kras(LSL-G12D/+);Ptf1a(Cre/+) mice). Normal bile duct, pancreatic duct, and tumor-associated metaplasias from the mice were analyzed for tuft cell and biliary progenitor markers, including SOX17, a transcription factor that regulates biliary development. We also analyzed pancreatic tissues from mice expressing transgenic SOX17 alone (ROSA(tTa/+);Ptf1(CreERTM/+);tetO-SOX17) or along with activated Kras (ROSAtT(a/+);Ptf1a(CreERTM/+);tetO-SOX17;Kras(LSL-G12D;+)). Tuft cells were frequently found in areas of pancreatic metaplasia, decreased throughout tumor progression, and absent from invasive tumors. Analysis of the pancreatobiliary ductal systems of mice revealed tuft cells in the biliary tract but not the normal pancreatic duct. Analysis for biliary markers revealed expression of SOX17 in pancreatic metaplasia and tumors. Pancreas-specific overexpression of SOX17 led to ductal metaplasia along with inflammation and collagen deposition. Mice that overexpressed SOX17 along with Kras(G12D) had a greater degree of transformed tissue compared with mice expressing only Kras(G12D). Immunofluorescence analysis of human pancreatic tissue arrays revealed the presence of tuft cells in metaplasia and early-stage tumors, along with SOX17 expression, consistent with a biliary phenotype

  20. Unilocular extrahepatic biliary cystadenoma mimicking choledochal cyst: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ju Hyun; Lee, Dong Ho; Kim, Hyoung Jung; Ko, Young Tae; Lim, Joo Won; Yang, Moon Ho [Kyunghee University Hospital, Seoul (Korea, Republic of)

    2004-12-15

    We report here on a case of extrahepatic biliary cystadenoma arising from the common hepatic duct. A 42-year-old woman was evaluated by us to find the cause of her jaundice. Ultrasonography and CT showed a cystic dilatation of the common hepatic duct and also marked dilatation of the intrahepatic duct. Direct cholangiography demonstrated a large filling defect between the left hepatic duct and the common hepatic duct; dilatation of the intrahepatic duct was also demonstrated. Following excision of the cystic mass, it was pathologically confirmed as a unilocular biliary mucinous cystadenoma arising from the common hepatic duct.

  1. Biliary cystadenoma in a Maltese dog: clinical and diagnostic findings.

    Science.gov (United States)

    Moon, So-Jeung; Kim, Ju-Won; Sur, Jung-Hyang; Jeong, Soon-Wuk; Park, Hee-Myung

    2011-12-01

    A 14-year-old Maltese dog presented for complete medical examination due to intermittent vomiting and diarrhea observed during the previous two days. A single, solitary, lobulated cystic mass was observed in the liver upon ultrasonographic and computed tomographic examination. After surgical hepatic resection to remove the mass, histological examination revealed a multilocular cyst lined by cuboidal to columnar epithelial cells, which is consistent with biliary cystadenoma. Here, we report the clinical, clinicopathological, histopathological, and diagnostic imaging findings of biliary cystadenoma in a dog.

  2. Unilocular Extrahepatic Biliary Cystadenoma Mimicking Choledochal Cyst: A Case Report

    Science.gov (United States)

    Park, Ju-Hyun; Kim, Hyoung Jung; Ko, Young Tae; Lim, Joo Won; Yang, Moon-Ho

    2004-01-01

    We report here on a case of extrahepatic biliary cystadenoma arising from the common hepatic duct. A 42-year-old woman was evaluated by us to find the cause of her jaundice. Ultrasonography and CT showed a cystic dilatation of the common hepatic duct and also marked dilatation of the intrahepatic duct. Direct cholangiography demonstrated a large filling defect between the left hepatic duct and the common hepatic duct; dilatation of the intrahepatic duct was also demonstrated. Following excision of the cystic mass, it was pathologically confirmed as a unilocular biliary mucinous cystadenoma arising from the common hepatic duct. PMID:15637480

  3. [Mini invasive treatment of common biliary duct lithiasis].

    Science.gov (United States)

    Deacu, Adriana

    2003-01-01

    The aim of the paper is to show modern techniques of treatment in common biliary duct lithiasis, in the context of the remarkable development of laparoscopic surgery. The text, trays to mark some directions for the surgeon to know when and which of the modern techniques to chose for safe treatment of common biliary duct lithiasis. The criteria are bound to the moment of diagnosis, clinical state of the patient and laboratory tests. Of course, a final commitment will be taken after an objective evaluation of the technique possibilities and the experience of the team.

  4. Estudo angiográfico da circulação pulmonar na tetralogia de Fallot com atresia pulmonar Angiographic study of pulmonary circulation in tetralogy of Fallot with pulmonary atresia

    Directory of Open Access Journals (Sweden)

    Marco Aurélio Santos

    2005-02-01

    Full Text Available OBJETIVO: Identificar os tipos de suprimento sangüíneo vascular pulmonar na tetralogia de Fallot com atresia pulmonar por meio de estudo hemodinâmico. MÉTODOS: Foram submetidos a estudo cineangiocardiográfico 56 pacientes portadores de tetralogia de Fallot com atresia pulmonar com idade de 20 dias a 4 anos e efetuadas injeções de contraste nas seguintes estruturas vasculares: 1 veia pulmonar encunhada, 2 colaterais aortopulmonares, 3 aorta torácica e 4 ductus arteriosus e/ou shunt sistêmico pulmonar. RESULTADOS: Dos 56 pacientes, 15 tinham o suprimento sangüíneo pulmonar através de colaterais aortopulmonares, em 36 o suprimento sangüíneo pulmonar era feito isoladamente pelo ductus arteriosus e em 5 pelo ductus arteriosus e colaterais aortopulmonares. Conforme a presença ou ausência de estruturas vasculares que compõem a circulação pulmonar na tetralogia de Fallot com atresia pulmonar e do tipo de perfusão vascular pulmonar, os doentes foram classificados em 6 tipos. CONCLUSÃO: Em função da grande complexidade e extrema variabilidade do suprimento sangüíneo pulmonar na tetralogia de Fallot com atresia pulmonar torna-se possível, com este tipo de abordagem, a obtenção de informações, suficientemente necessárias, para o correto manuseio clínico-cirúrgico.OBJECTIVE: To identify the types of pulmonary vascular blood supply in tetralogy of Fallot with pulmonary atresia by use of hemodynamic study. METHODS: Fifty-six patients with tetralogy of Fallot and pulmonary atresia, and ages ranging from 20 days to 4 years, underwent cineangiocardiographic study with contrast medium injections in the following vascular structures: 1 wedged pulmonary vein; 2 aortopulmonary collaterals; 3 thoracic aorta; and 4 ductus arteriosus or systemic-pulmonary shunt. RESULTS: In the 56 patients studied, pulmonary blood was supplied as follows: in 15, by aortopulmonary collaterals; in 36, only by the ductus arteriosus; and in 5, by the ductus

  5. 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.

  6. Bronchoscopy and Fogarty Balloon Insertion of Distal Tracheo-Oesophageal Fistula for Oesophageal Atresia Repair With Video Illustration.

    Science.gov (United States)

    Rinkel, Rico; Van Poll, Daan; Sibarani-Ponsen, Renate; Sleeboom, Christien; Bakx, Roel

    2017-01-01

    During repair of esophageal atresia with distal tracheo-esophageal fistula, air leakage through the fistula during mechanical ventilation can cause respiratory demise. From February 2012 until November 2014, all patients with esophageal atresia and distal tracheo-esophageal fistula were subjected to preoperative tracheobronchoscopy. Relatively distal fistulas were cannulated with a Fogarty catheter and blocked by insufflation (video illustration). Relatively proximal distal fistulas were sealed by precise placement of a cuffed ventilation tube. Nine of 12 patients received Fogarty balloon placement. The fistula of the remaining 3 patients were sealed by careful tube placement. No complications related to tracheobronchoscopy or Fogarty placement were noted. All procedures were uneventful. Preoperative tracheobronchoscopy to evaluate the usefulness of Fogarty balloon insertion or correct tube placement for distal tracheo-esophageal fistula is a safe and easy to perform procedure that can avoid complications in type C esophageal atresia repair. © The Author(s) 2016.

  7. Biliary Interventions: Tools and Techniques of the Trade, Access, Cholangiography, Biopsy, Cholangioscopy, Cholangioplasty, Stenting, Stone Extraction, and Brachytherapy.

    Science.gov (United States)

    Ahmed, Osman; Mathevosian, Sipan; Arslan, Bulent

    2016-12-01

    Therapeutic access to the biliary system is generally limited to endoscopic or percutaneous approaches. A variety of percutaneous transhepatic biliary interventions are applicable for the diagnosis and treatment of biliary system pathologies, the majority of which may be performed in conjunction with one another. The backbone of nearly all of these interventions is percutaneous transhepatic cholangiography for opacification of the biliary tree, after which any number of therapeutic or diagnostic modalities may be pursued. We describe an overview of the instrumentation and technical approaches for several fundamental interventional procedures, including percutaneous transhepatic cholangiography and internal/external biliary drainage, endobiliary biopsy techniques, cholangioscopy, cholangioplasty and biliary stenting, biliary stone extraction, and intraluminal brachytherapy.

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

    Science.gov (United States)

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

    2016-11-01

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

  9. Woman with surgical reconstruction of anal atresia who realized pregnancy with in vitro fertilisation

    Directory of Open Access Journals (Sweden)

    Garalejić Eliana

    2010-01-01

    Full Text Available Introduction. Anal atresia is a congenital anomaly, very life threatening and urgent. Surgical treatment of this anomaly consists of colostomy first, and then of anal reconstruction. Case report. We presented a 31-year old female with the surgery treatment of anal atresia in the early childhood. In the reproductive period, due to tubal infertily, the patient was included in the program of in vitro fertilization (IVF, in the Clinic for Gynecology and Obstetrics 'Narodni front', Belgrade. Within this program a long protocol of ovarian stimulation was performed. Ultrasonographic and color Doppler monitoring of the patient was applied by the use of an ultrasonographic apparatus type Siemens Acuson X 150, while any hormonal examinations were performed by an Architect Abbott unit. During the IVF program, the growth of follicules was controlled by the use of ultrasonography, microcirculation of the ovaries and the uterus was marked by a power-pulsating color Doppler, and hormonal examination was performed starting from the day of stimulation up to the day of injecting Pregnyl®. The patient was administered Suprefact® (buserelin sc from the 21st day of the menstrual cycle, as well as from the 3rd day of the cycle, for totally 11 days. The patient was given 29 ampoules of Gonal F® (recombinant human FSH 75 IJ im and 15 ampoules of Menopur® (menotrophin im. Due to a modified pelvic anatomy, the left ovary aspiration was disabled, while the right ovary aspired seven oocytes successfully. Three embryos were inserted in the uterus. The delivery was performed by cesarean section. Conclusion. In the reported patient with a modified pelvic anatomy due to four corrective surgeries of anal atresia, and tubal infertility in the reproductive period, the method of choice for the realization of pregnancy was the IVF procedure. The realized pregnancy and the delivery could be considered highly successful in regard to possible risks.

  10. Outcomes at one-year post anastomosis from a national cohort of infants with oesophageal atresia.

    Directory of Open Access Journals (Sweden)

    Benjamin Allin

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

  11. Aesthetic and hearing rehabilitation in patients with bilateral microtia-atresia.

    Science.gov (United States)

    Fan, Xinmiao; Wang, Yibei; Wang, Pu; Fan, Yue; Chen, Yu; Zhu, Yuanli; Chen, Xiaowei

    2017-10-01

    To evaluate the safety and efficacy of auricle reconstruction and active transcutaneous bone-conduction implantation in patients with bilateral microtia-atresia. Patients were chosen prospectively, with each being his/her own control. The setting was a tertiary referral center. Twelve patients, aged 6-18 years, with bilateral microtia-atresia suffering from bilateral conductive hearing loss. All had an upper bone conduction threshold limit of 45 dB HL at frequencies of 0.5-4 kHz. Patient satisfaction with the reconstructed auricle was rated as highly satisfactory, basically satisfactory, or unsatisfactory. Mean pure-tone thresholds and speech audiometry test results were compared among patients unaided, with a soft-band Bonebridge, and with an implanted Bonebridge. Subjective satisfaction was analyzed using three questionnaires: the Abbreviated Profile of Hearing Aid Benefit (APHAB), the Glasgow children's benefit inventory (GCBI), and the International Outcome Inventory for Hearing Aids (IOI-HA). All patients who underwent auricle reconstruction expressed satisfaction with their appearance. The mean pure-tone thresholds of unaided patients and those with soft-band and implanted Bonebridge were 55.25 ± 3.43 dBHL, 31.37 ± 3.03 dBHL, and 21.25 ± 2.16 dBHL, respectively. The mean speech discrimination scores measured in a sound field with a presentation level of 65 dB SPL under these three conditions were 46.0 ± 0.11%, 80.0 ± 0.09%, and 94.0 ± 0.02%, respectively. Questionnaires demonstrated patients' benefits and satisfaction with this surgery. The surgical procedure involving auricle reconstruction and Bonebridge implantation was safe and effective for patients with bilateral microtia-atresia, solving both appearance and hearing problems. Copyright © 2017. Published by Elsevier B.V.

  12. [Efficacy of BAHA softband in young children with bilateral congenital aural atresia].

    Science.gov (United States)

    Zhang, Ying; Fan, Yue; Wang, Yibei; Chen, Xiaowei

    2014-02-18

    To retrospectively analyze the auditory and speech development of young children with bilateral congenital aural atresia after using bone-anchored hearing aid (BAHA) softband. From August 2010 to January 2013, a total of 12 patients aged at a range of 3 months to 6 years with bilateral aural atresia using BAHA softband were divided into under 4-year-old group (n = 7, an average age of 14 months) and over 4-year-old group (n = 5, an average age of 77 months). The air and bone auditory thresholds of the under 4-year-old group were assessed by auditory brainstem response (ABR). The Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to evaluate early auditory development.Soundfield pure tone audiometry (PTA) was applied in the over 4-year-old group. The Standard Chinese lexical neighborhood test (LNT) was conducted to evaluate the speech discrimination scores unaided and aided for 6, 12 and 24 months. For the under 4-year-old group, the unaided air and bone conduction hearing thresholds were 70-90(73 ± 12) and 15-25(21 ± 4) dB nHL respectively.IT-MAIS score improved significantly after using softband for 6 months in comparisons with unaided situations. The average hearing threshold of patients in the over 4-year-old group was (63 ± 6) dB HL unaided versus (31 ± 4)dB HL aided. The average decline in pure-tone threshold was (32 ± 3)dB HL with BAHA softbands and LNT demonstrated improvements in the speech discrimination score. The differences of paired comparison were significant (all P hearing ability of children with bilateral congenital aural atresia improves significantly after using BAHA softband. And its application at a young age can guarantee a relatively normal hearing development and avoid oral communication impairment.

  13. Late Clinical Outcomes of the Fontan Operation in Patients with Tricuspid Atresia

    Directory of Open Access Journals (Sweden)

    Mastalir Eduardo T.

    2002-01-01

    Full Text Available OBJECTIVE: Evaluation of the long-term clinical results of the Fontan operation in patients with tricuspid atresia. METHODS: A retrospective analysis was made at the Instituto de Cardiologia do Rio Grande do Sul (Institute of Cardiology of Rio Grande do Sul, from August 1980 through January 2000, of 25 patients with a long-term follow-up, out of a series of 36 patients who underwent the Fontan operation or one of its variants due to tricuspid atresia. Their mean age at surgery was 5.4±3.1 years, and their mean weight was 15.8±6.1 kg, the majority of them (63.9% being males. Four patients underwent the classical Fontan operation, 12 the Kreutzer variant, 6 the Björk variant, 9 total cavopulmonary shunt with a fenestrated tube, and 5 total cavopulmonary shunt with a nonfenestrated tube. RESULTS: The patients were followed-up on an outpatient basis, with a mean long-term survival time of 5.5±4.2 years (50 days to 17.8 years and a late mortality rate of 8%. Arterial saturation increased from 77.2±18.8% in the preoperative period to 91±6.7% upon the last outpatient visit (p>0.05. At the final check, most (67% patients were asymptomatic and 87% could tolerate exercise. Ten (40% patients experienced some kind of complication during the long-term follow-up, such as cardiac arrhythmia, cyanosis, protein-losing enteropathy, neurological events, right heart failure, intolerance to exercise and reoperation. CONCLUSION: The results indicate that, once the immediate postoperative period is over, during which the adaptations to the new circulatory physiology occur, the evolution of patients with tricuspid atresia who underwent the Fontan operation is satisfactory, in spite of a low, yet significant, morbidity.

  14. [Rehabilitation of hypoplastic pulmonary arteries and anatomic correction of pulmonary atresia with interventricular communication].

    Science.gov (United States)

    Chetaille, P; Fraisse, A; Ghez, O; Kreitmann, B; Voisin, M; Aubert, F; Metras, D

    2001-05-01

    Conventional treatment of pulmonary atresia with ventricular septal defect (VSD), hypoplastic pulmonary arteries (PA) and major aorto-pulmonary collaterals (MAPCAs) is controversial: from symptomatic and palliative treatment for some authors to surgery with unifocalisation of collaterals for others. These treatments never use native pulmonary arteries as only source of pulmonary flow, but create "neo-pulmonary arteries". Nine cases of pulmonary atresia with VSD, hypoplastic PA and MAPCAs were treated by rehabilitation of native PA through a staged approach: 1) surgical neonatal connection between right ventricule (RV) and hypoplastic PA, 2) evaluation and interventionnal catheterism with angioplasty of PA stenosis and closure of collaterals, 3) complete surgical correction with reconstruction of right outflow track and PA and closure of VSD. After first surgical stage of RV-PA connection at the mean age of 4.8 months (+/- 5.6 months), 8 patients were alive and underwent 22 cardiac catheterisms (mean of 2.7 per patient), with angioplasty of PA, and occlusion of MAPCAs in 6 and 2 patients respectively. Seven patients underwent complete anatomical correction at the mean age of 28.8 months (+/- 17.7 months) with one late death. The 6 remaining patients had encouraging hemodynamic status (RV pressure/LV pressure ratio at 0.6 +/- 0.26; mean left and right distal pulmonary pressure at 15.2 mmHg (+/- 9.1 mmHg)), and good functionnal status (3 in NYHA functionnal class 1, and 3 in class 2), for a mean follow-up of 79.5 months (+/- 41.4 months). One patient had reoperation on right outflow track stenosis, 6 years after correction. This small series enhances the feasibility of a staged approach with rehabilitation of small PA, allowing complete surgical correction with the native PA with good hemodynamic and functional results in pulmonary atresia, with VSD, hypoplastic PA and MAPCAs.

  15. Transcriptome profiling of the theca interna from bovine ovarian follicles during atresia.

    Directory of Open Access Journals (Sweden)

    Nicholas Hatzirodos

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

  16. Epidermolysis Bullosa with Pyloric Atresia and Aplasia Cutis in a Newborn Due to Homozygous Mutation in ITGB4.

    Science.gov (United States)

    Kayki, Gozdem; Bozkaya, Davut; Ozaltin, Fatih; Orhan, Diclehan; Kaymaz, Figen; Korkmaz, Emine; Yigit, Sule

    2017-08-01

    Epidermolysis bullosa with pyloric atresia (EB-PA) is an autosomal recessive disorder due to mutations in ITGA6 and/or ITGB4, resulting in altered expression of α6β4 integrin. EB-PA can also occur with aplasia cutis. We present a newborn with EB-PA and aplasia cutis, born of consanguineous parents, with a homozygous c.3793+1G>A mutation affecting ITGB4, previously described only in the heterozygous state with other mutations. The previously unreported homozygous c.3793+1G>A mutation affecting ITGB4 causes a severe form of junctional epidermolysis bullosa with pyloric atresia and aplasia cutis.

  17. Case report: Isolated unilateral pulmonary vein atresia diagnosed on 128-slice multidetector CT

    Directory of Open Access Journals (Sweden)

    Rashmi Dixit

    2011-01-01

    Full Text Available Unilateral pulmonary venous atresia is an uncommon entity that is generally believed to be congenital. Most patients present in infancy or childhood with recurrent chest infections or hemoptysis. Pulmonary angiography is usually used for definitive diagnosis. However, the current multislice CT scanners may obviate the need for pulmonary angiography. We report two cases diagnosed using 128-slice CT angiography. On the CT angiography images both these cases demonstrated absent pulmonary veins on the affected side, with a small pulmonary artery and prominent bronchial or other systemic arterial supply.

  18. ATRESIA CONGÉNITA DEL OÍDO Y SU MANEJO

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    Dr. Daniel Orfila

    2016-11-01

    Los implantes cocleares siguen siendo los más usados y en las que se tiene mayor experiencia. Las prótesis de conducción ósea implantables o semi implantables cambiaron el manejo de las atresias y malformaciones de oído externo y medio. Pese a lo prometedor que se visualiza el presente y futuro con el uso de estos dispositivos, siempre se debe tener presente que requieren de un acto quirúrgico para su implantación y que no están exentas de complicaciones, por lo cual se debe elegir juiciosamente la prótesis a usar.

  19. High-dose omeprazole in esophagitis with stenosis after surgical treatment of esophageal atresia.

    Science.gov (United States)

    Van Biervliet, S; Van Winckel, M; Robberecht, E; Kerremans, I

    2001-09-01

    The authors describe 4 children with recurrent stenosis and persistent esophagitis after secondary repair of a long gap esophageal atresia. They underwent an esophageal reconstruction by elongation of the lesser gastric curvature according to Schärli at the age of 11 to 14 months. All had esophagitis grade III to IV (Savary-Miller classification), esophageal stenosis, and failure to thrive. Effective treatment of the esophagitis and prevention of stenosis consisted in high doses of omeprazole (1.9 to 2.5 mg/kg/d). After this treatment, the need for esophageal dilatation disappeared, and nutritional status normalized. Copyright 2001 by W.B. Saunders Company.

  20. The great debate: open or thoracoscopic repair for oesophageal atresia or diaphragmatic hernia.

    Science.gov (United States)

    Davenport, Mark; Rothenberg, Steven S; Crabbe, David C G; Wulkan, Mark L

    2015-02-01

    Controversy exists over the best method and technique of repair of oesophageal atresia and diaphragmatic hernia. Open surgical repairs have a long established history of over 60 years of experience. Set against this has been a series of successful thoracoscopic repairs of both congenital anomalies reported over the past decade. This review was based upon a four-handed debate on the merits and weaknesses of the two contrasting surgical philosophies and reviews existing literature, techniques, complications, and importantly outcome and results. Copyright © 2015 Elsevier Inc. All rights reserved.

  1. 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. Published by Elsevier B.V.

  2. Liver alkaline phosphatase: a missing link between choleresis and biliary inflammation.

    Science.gov (United States)

    Poupon, Raoul

    2015-06-01

    Several lines of evidence show that serum alkaline phosphatase (AP) is not only a signpost of cholestasis but also a surrogate marker of the severity of primary biliary cirrhosis and primary sclerosing cholangitis. In the present opinion article, we review and discuss the putative role of liver AP in health and in cholestatic diseases. In inflammatory cholestatic conditions, loss of activity of liver AP (resulting from its relocation from canaliculi and the acidic milieu) might promote hyper-adenosine triphosphate-bilia, lipopolysaccharide overload, and subsequent exacerbation and perpetuation of inflammation. Drugs that can restore the polarity of hepatocytes and canalicular export of bile acids or act as bile alkalinity modifiers are predicted to exert anti-inflammatory effects and to benefit both primary biliary cirrhosis and primary sclerosing cholangitis. Oral administration of intestinal AP could be a valid therapeutic intervention that deserves further study under experimental conditions as well as in human diseases. Overall, the key role of the liver microenvironment that might shape the different facets of the inflammatory processes in fibrosing cholangiopathies is highlighted. © 2015 by the American Association for the Study of Liver Diseases.

  3. Biliary duct obstruction treatment with aid of percutaneous ...

    African Journals Online (AJOL)

    Daniel Knap

    2015-08-19

    Aug 19, 2015 ... carried out. 2.1. Procedure evaluation. Patients abstained from oral intake or were on a clear liquid .... (1.08%) had biliary tract hemorrhage to the peritoneal cavity. [Table 2]. 3.4. Survival. Mortality in ... ies.9,14,17,20 However, because of the anatomy of portal triads, the procedure must be performed with ...

  4. Outcomes of Laparoscopic Cholecystectomy for Biliary Dyskinesia in Children.

    Science.gov (United States)

    Lai, Sarah W; Rothenberg, Steven S; Kay, Saundra M; Shipman, Kristin E; Slater, Bethany J

    2017-08-01

    To determine the outcomes of laparoscopic cholecystectomy as a treatment for biliary dyskinesia in children. With ethics approval, a retrospective chart review was performed on children (resolution. Laparoscopic cholecystectomy was performed in 215 children with biliary dyskinesia (156/215 [72.6%] female, age 13.8 ± 3.4 years, body mass index [BMI] 22.3 ± 6.3 kg/m 2 ). 181/206 (87.9%) had EF 181 (89.5%). Chronic cholecystitis was found in 183/213 (85.9%) and unexpected cholelithiasis in 4/213 (1.9%) on pathology. Postoperatively, 6/181 (3.3%) had wound infections and 8/181 (4.4%) required common bile duct stents for the following indications: 6 sphincter of Oddi dysfunction, 1 choledocholithiasis, and 1 stricture. Virgin abdomen (odds ratio [OR] 4.03, confidence interval [95% CI] 1.12-14.53, P = .0460) and follow-up resolution for biliary dyskinesia in children. Virgin abdomen and follow-up <6 months were associated with better outcomes. Prospective long-term studies comparing surgical and nonoperative management of biliary dyskinesia are required to determine the utility of cholecystectomy.

  5. Incidence and management of biliary leakage after hepaticojejunostomy

    NARCIS (Netherlands)

    de Castro, Steve M. M.; Kuhlmann, Koert F. D.; Busch, Olivier R. C.; van Delden, Otto M.; Laméris, Johan S.; van Gulik, Thomas M.; Obertop, Hugo; Gouma, Dirk J.

    2005-01-01

    This study analyzes the change in the management of biliary leakage after hepaticojejunostomy. Between 1993 and 2003 all patients (n = 1033) were studied with a hepaticojejunostomv as part of a pancreatoduodenectomy (n = 486), proximal bile duct resection (without liver resection) (n = 35), and

  6. Fibrates for Primary Biliary Cholangitis: What's All the Hype?

    Science.gov (United States)

    Levy, Cynthia

    2017-01-01

    Ursodeoxycholic acid is the first-line therapy for primary biliary cholangitis. However, a subset of patients fail to show biochemical response. For these patients, adjuvant therapies are warranted. Obeticholic acid was conditionally approved as a second-line drug. Evidence is building up in favor of fibrates, which are available for off-label use.

  7. Portal Hypertensive Biliopathy: An Infrequent Cause of Biliary Obstruction

    Directory of Open Access Journals (Sweden)

    Ricardo Cardoso

    2015-03-01

    Discussion: Portal cavernoma, the hallmark of extrahepatic portal venous obstruction, can cause PHB. When symptomatic, chronic cholestasis is present if a dominant stricture exists whereas biliary pain and acute cholangitis occur when choledocholithiasis prevails. Management must be individualized and usually includes endoscopic therapy to address choledocholithiasis and shunt surgery for definitive treatment.

  8. Adverse effects of barium sulfate in the biliary tract.

    Science.gov (United States)

    Walsham, Anna; Larsen, Jörg

    2008-06-01

    Reflux of barium sulfate preparations into the biliary tract is rare, but serious complications have been recorded. To consider the implications of such reflux through enterobiliary stents, the literature was reviewed. A case illustrating such an occurrence is presented. Based upon the limited literature available, barium suspension may be retained in particular circumstances and cause or contribute to stent occlusion.

  9. Clogging of biliary endoprostheses. A morphologic and bacteriologic study

    DEFF Research Database (Denmark)

    Dowidar, N; Kolmos, H J; Lyon, H

    1991-01-01

    The main problem encountered with the use of biliary endoprostheses is their tendency to clog, which necessitates their exchange. We studied this problem by performing light and electron microscopy and bacterial cultures on both unused and clinically used endoprostheses. These examinations showed...... to the endoprosthesis is the desired goal and may be achieved by improving their manufacture and design....

  10. Percutaneous transhepatic biliary drainage: analysis of 175 cases

    Energy Technology Data Exchange (ETDEWEB)

    Suh, Kyung Jin; Lee, Sang Kwon; Kim, Tae Hun; Kim, Yong Joo; Kang, Duk Sik [College of Medicine, Kyungpook National Univ., Daegu (Korea, Republic of)

    1990-10-15

    Percutaneous transhepatic biliary drainage is a safe, effective and palliative means of treatment in biliary obstruction, especially in cases with malignant obstruction which are inoperable. 175 cases of transhepatic biliary drainage were performed on 119 patients with biliary obstruction from January 1985 to June 1989 at Kyung-pook National University Hospital. The causes of obstructive jaundice were 110 malignant diseases and 9 benign diseases. The most common indication for drainage was palliative intervention of obstruction secondary to malignant tumor in 89 cases. 86 cases of external drainage were performed including 3 cases of left duct approach, 29 cases of external-internal drainage and 60 cases of endoprosthesis. In external and external-internal drainages, immediate major complications (11.9%) occurred, including not restricted to, but sepsis, bile peritonitis and hemobilia. Delayed major complications (42.9%) were mainly catheter related. The delayed major complication of endoprosthesis resulted from obstruction of the internal stent. The mean time period to reobstruction of the internal stent was about 12 weeks. To improve management status, regular follow-up is required, as is education of both patients and their families as to when immediate clinical attention is mandated. Close communication amongst the varying medical specialities involved will be necessary to provide optional treatment for each patient.

  11. Malfunctioning Plastic Biliary Endoprosthesis: Percutaneous Transhepatic Balloon Pulling Technique

    Science.gov (United States)

    Rossi, Umberto G.; Rigamonti, Paolo; Cariati, Maurizio

    2013-01-01

    Percutaneous transhepatic removal techniques for malfunctioning plastic biliary endoprosthesis are considered safe and efficient second-line strategies, when endoscopic procedures are not feasible. We describe the percutaneous transhepatic balloon pulling technique in a patient with an unresectable malignant hilar cholangiocarcinoma. PMID:23984158

  12. Malfunctioning Plastic Biliary Endoprosthesis: Percutaneous Transhepatic Balloon Pulling Technique

    Directory of Open Access Journals (Sweden)

    Umberto G. Rossi

    2013-01-01

    Full Text Available Percutaneous transhepatic removal techniques for malfunctioning plastic biliary endoprosthesis are considered safe and efficient second-line strategies, when endoscopic procedures are not feasible. We describe the percutaneous transhepatic balloon pulling technique in a patient with an unresectable malignant hilar cholangiocarcinoma.

  13. Biliary sludge and recurrent ketoacidosis: a case report

    Directory of Open Access Journals (Sweden)

    Kalra Sanjay

    2009-12-01

    Full Text Available Abstract A five year old boy, weighing 14 kg with no family history of diabetes, presented in frank diabetic ketoacidosis. He recovered, but continued to have episodes of ketoacidosis. He was diagnosed to have biliary sludge, which recovered with insulin treatment.

  14. Documenting correct assessment of biliary anatomy during laparoscopic cholecystectomy

    NARCIS (Netherlands)

    Buddingh, K. T.; Morks, A. N.; Hoedemaker, H. O. ten Cate; Blaauw, C. B.; van Dam, G. M.; Ploeg, R. J.; Hofker, H. S.; Nieuwenhuijs, V. B.

    Background Correct assessment of biliary anatomy can be documented by photographs showing the "critical view of safety" (CVS) but also by intraoperative cholangiography (IOC). Methods Photographs of the CVS and IOC images for 63 patients were presented to three expert observers in a random and

  15. Primary biliary cirrhosis following lactobacillus vaccination for recurrent vaginitis

    NARCIS (Netherlands)

    Bogdanos, Dimitrios; Pusl, Thomas; Rust, Christian; Vergani, Diego; Beuers, Ulrich

    2008-01-01

    Background/Aims:Antimitochondrial antibodies directed against the E2 subunit of the pyruvate dehydrogenase complex, PDC-E2, and other mitochondrial 2-oxoacid dehydrogenases (AMA-M2) are the hallmark for diagnosis (of primary biliary cirrhosis (PBC). AMA-M2 formation as an early step in the

  16. Clogging of biliary endoprostheses. A morphologic and bacteriologic study

    DEFF Research Database (Denmark)

    Dowidar, N; Kolmos, H J; Lyon, H

    1991-01-01

    The main problem encountered with the use of biliary endoprostheses is their tendency to clog, which necessitates their exchange. We studied this problem by performing light and electron microscopy and bacterial cultures on both unused and clinically used endoprostheses. These examinations showed...

  17. Effect of probenecid on the biliary excretion of belotecan.

    Science.gov (United States)

    Namkoong, Eun-Mi; Kim, In-Wha; Kim, Dae-Duk; Chung, Suk-Jae; Shim, Chang-Koo

    2007-11-01

    The purpose of this study was to investigate the effect of probenecid, an inhibitor of the MRP2/ ABCC transporter, on the pharmacokinetics and transport of belotecan (7-[2-(N-isopropylamino)ethyl]-(20S)-camptothecin). The effect of probenecid on the pharmacokinetics of belotecan was studied in rats. When belotecan was injected as a bolus dose of 5 mg/kg after probenecid was infused at a rate of 42.8 mg/2 mL/h/kg, the cumulative biliary excretion amounts and biliary clearance (CL(b)) of belotecan decreased (28.29 +/- 2.83 versus 19.96 +/- 1.45% of dose and 161.01 +/- 26.95 versus 92.66 +/- 1.45 mL/min/kg), whereas the systemic pharmacokinetics did not change. This indicates that the MRP2 transporter is involved in the biliary excretion of belotecan. The involvement of MRP2 in the secretory transport was further characterized using Caco-2 cell monolayers expressing MRP2. The apparent permeability across Caco-2 cell monolayers from basolateral to apical was 2.3 times greater than that from the apical to the basolateral side at the 50 microM belotecan. In addition, probenecid significantly decreased the basolateral-to-apical transport of belotecan (52.9%). These results indicate that MRP2 is involved in the secretory transport of belotecan in biliary excretion.

  18. Management of intrahepatic biliary lithiasis after pancreatic cancer surgery.

    Science.gov (United States)

    Pezzolla, Angela; Lattarulo, Serafina; De Luca, Giuseppe Massimiliano; Borrello, Gianluca; Fucilli, Fabio; Marano, Giuseppe; Fabiano, Gennaro; Palasciano, Nicola

    2008-01-01

    Intrahepatic biliary lithiasis is fairly rare in western countries. In the case described here, liver stones had developed as a late consequence of biliary derivative surgery, which is well known to lead to this complication. However, this case is unusual because people who have undergone radical surgery for cancer of the head of the pancreas seldom survive long enough for liver stones to develop. Treatment for this 65-year-old woman, previously submitted to duodeno-cephalopancreatectomy, involved percutaneous balloon bilioplasty, with several passages in order to open the anastomosis. We then positioned two inner-outer biliary drains, through which repeated lavages were done. Finally, the patient underwent laser lithotripsy of the intrahepatic calculi and the fragments were cleared using a Dormia basket. Repeated cholangiographic monitoring showed progressively fewer stones, until the intrahepatic biliary tree was finally completely clear 120 days after the initial diagnosis. At the last follow-up, the patient was healthy, with normal blood values, considering her overall condition.

  19. [Acute disorders of the biliary tract in geriatric patients].

    Science.gov (United States)

    Pannella, A; Ragaglia, G; Laboranti, F; Fossa, M; Picchio, G L; Zambianchi, M; Zanotto, P

    1992-09-30

    Acute pathologies of the biliary tract in geriatric patients were examined in this study taking into account the major causes, treatment used and results obtained. All patients aged over 65 who had been hospitalised during the past 17 years for acute pathologies of the biliary tract (564 cases, equivalent to 34.2% of all in-patients suffering from acute biliary pathologies) were included in the study. These patients were then subdivided into 3rd and 4th age groups (65-74 and lithiasic disease of the cholecystus (61.2%) and VBP (17.7%), whereas 45 patients, equivalent to 49.5%, presented tumours with jaundice. Out of a total of 179 cases in patients in the 4th age group, equivalent to 39.3%, 119 (66.5%) were suffering from lithiasic cholecystitis and 16 (8.9%) from calcolosis of the VBP with jaundice. Cancer of the pancreas head was diagnosed in 27 patients (58.7%), whereas 9 (19.6%) had obstruent cancer of the biliary tract. The Authors conclude that both the preoperative preparation, the choice of operation and postoperative treatment give satisfactory results with a very low early mortality (0.8% in non-tumour cases and 6.9% in tumour cases).

  20. Biliary phosphatidylcholine and lysophosphatidylcholine profiles in sclerosing cholangitis.

    Science.gov (United States)

    Gauss, Annika; Ehehalt, Robert; Lehmann, Wolf-Dieter; Erben, Gerhard; Weiss, Karl-Heinz; Schaefer, Yvonne; Kloeters-Plachky, Petra; Stiehl, Adolf; Stremmel, Wolfgang; Sauer, Peter; Gotthardt, Daniel Nils

    2013-09-07

    To analyze phospholipid profiles in intrahepatic bile from patients with primary sclerosing cholangitis (PSC) and secondary sclerosing cholangitis (SSC). Intrahepatic bile specimens collected via endoscopic retrograde cholangiography from 41 patients were analyzed. Fourteen of these patients were diagnosed with PSC, 10 with SSC, 11 with choledocholithiasis or no identifiable biliary disease, and 6 with cholangiocellular carcinoma (CCC). Bile acid, cholesterol, protein, and bilirubin contents as well as pancreas lipase activity in bile were determined by biochemical methods. Phosphatidylcholine (PC) and lysophosphatidylcholine (LPC) species were quantified using nano-electrospray ionization tandem mass spectrometry. Bile from all the examined patient groups showed a remarkably similar PC and LPC species composition, with only minor statistical differences. Total biliary PC concentrations were highest in controls (8030 ± 1843 μmol/L) and lowest in patients with CCC (1969 ± 981 μmol/L) (P = 0.005, controls vs SSC and CCC, respectively, P < 0.05). LPC contents in bile were overall low (4.2% ± 1.8%). Biliary LPC/PC ratios and ratios of biliary PC to bilirubin, PC to cholesterol, PC to protein, and PC to bile acids showed no intergroup differences. PC and LPC profiles being similar in patients with or without sclerosing cholangitis, these phospholipids are likely not of major pathogenetic importance in this disease group.