WorldWideScience

Sample records for biliary atresia

  1. Biliary Atresia

    Science.gov (United States)

    ... Liver Disease & NASH Definition & Facts Symptoms & Causes Diagnosis Treatment Eating, Diet, & Nutrition Clinical Trials Biliary Atresia Cirrhosis Hemochromatosis Hepatitis A through E (Viral Hepatitis) Hepatitis ...

  2. Biliary atresia

    Science.gov (United States)

    ... Elsevier; 2016:chap 356. Suchy FJ. Anatomy, histology, embryology, developmental anomalies, and pediatric disorders of the biliary ... M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health ...

  3. Biliary atresia: pathogenesis and treatment.

    Science.gov (United States)

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

    1998-01-01

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

  4. Biliary atresia associated with meconium peritonitis.

    Science.gov (United States)

    Chung, Mei-Yung; Ko, Tan-Yung; Huang, Chung-Bin; Lee, Chiang-Hsuan; Hsieh, Chih-Sung

    2006-01-01

    Biliary atresia, malrotation, meconium peritonitis and transient hypothyroidism are occasionally seen in neonatal infants. Biliary atresia associated with malrotation has been reported in some patients with polysplenia syndrome, but biliary atresia associated with meconium peritonitis has only been described by a few investigators. Here we present a case of meconium peritonitis due to malrotation with volvulus, followed by biliary atresia and transient hypothyroidism during early infancy.

  5. Association of Biliary Atresia with Jejuno-Ileal Atresia

    Directory of Open Access Journals (Sweden)

    Alireza Alam Sahebpour

    2009-09-01

    Full Text Available Background:Jejuno-ileal atresia (JIA is a congenital anomaly characterized clinically by bilious vomiting and abdominal distension. The incidence of JIA is between 1: 330 to 1: 3000 live births in different parts of the world. It has been associated with various congenital anomalies but the association of JIA with biliary atresia is extremely rare (0-3.2 %. Case Presentation:We herein present a case of jejunal atresia with meconium peritonitis associated with biliary atresia. The patient was a boy who was born at 39 weeks of gestation with polyhydramnious detected on Prenatal Ultra Sonography done at 8th month of gestation. Conclusion:It is important to explore gallbladder in cases of JIA especially when associated with meconium peritonitis.

  6. Biliary atresia Atresia de las vías biliares.

    Directory of Open Access Journals (Sweden)

    Pastor Thomas Olivares

    2005-12-01

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

  7. ETIOLOGY, PATHOGENESIS AND MORPHOLOGY OF BILIARY ATRESIA

    Directory of Open Access Journals (Sweden)

    O. E. Iryshkin

    2012-01-01

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

  8. Current options for management of biliary atresia.

    Science.gov (United States)

    Gallo, Amy; Esquivel, Carlos O

    2013-03-01

    It is encouraging that we are improving the technical aspects of treatment modalities for biliary atresia. However, it is clear that more needs to be done to best develop new treatment plans while applying the modalities we have (porto-enterostomy or liver transplantation or both) in a way that will afford the best survival and quality-of-life. This review article will discuss a number of points that are vital to improving care and illustrates the need to further scrutinize treatment decisions.

  9. Congenital biliary atresia: liver injury begins at birth

    DEFF Research Database (Denmark)

    Makin, Erica; Quaglia, Alberto; Kvist, Nina

    2009-01-01

    -note review for infants with definite BA who underwent laparotomy within first week of life. RESULTS: Three infants were identified who had occlusive BA evident on the first day of life. In all cases, their liver was grossly normal, and histologic changes were trivial. CONCLUSION: This suggests......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...

  10. Anaesthesia for biliary atresia and hepatectomy in paediatrics

    Directory of Open Access Journals (Sweden)

    Rebecca Jacob

    2012-01-01

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

  11. Hepatobiliary scintigraphy for early diagnosis of biliary atresia

    DEFF Research Database (Denmark)

    Brittain, Jane Maestri; Kvist, Nina; Johansen, Lars Søndergaard;

    2016-01-01

    INTRODUCTION: The aim of this study was to evaluate the validity of (99m)Technetium-trimethylbromo-iminodiacetic acid hepatobiliary scintigraphy (HS) for the diagnosis of biliary atresia (BA). METHODS: From January 2005 to December 2009, a total of 47 infants with conjugated hyperbilirubinaemia...... sensitivity and NPV prevent un-necessary surgery. Because of the low specificity of HS in diagnosing BA, it should be part of a multimodality imaging strategy when the result supports a clinical suspicion of BA. In cases with non-draining HS and normal GGTP blood levels, supplemental imaging modalities...

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

    OpenAIRE

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

    2015-01-01

    AIM: 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).

  13. A study of associated congenital anomalies with biliary atresia

    Directory of Open Access Journals (Sweden)

    Lucky Gupta

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Priya Ramachandran

    2016-01-01

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

  15. The Role of ARF6 in Biliary Atresia.

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2006-08-15

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

  18. Maternal microchimerism in the livers of patients with Biliary atresia

    Directory of Open Access Journals (Sweden)

    Magrane Greg

    2004-07-01

    Full Text Available Abstract Background Biliary atresia (BA is a neonatal cholestatic disease of unknown etiology. It is the leading cause of liver transplantation in children. Many similarities exist between BA and graft versus host disease suggesting engraftment of maternal cells during gestation could result in immune responses that lead to BA. The aim of this study was to determine the presence and extent of maternal microchimerism (MM in the livers of infants with BA. Methods Using fluorescent in situ hybridization (FISH, 11 male BA & 4 male neonatal hepatitis (NH livers, which served as controls, were analyzed for X and Y-chromosomes. To further investigate MM in BA, 3 patients with BA, and their mothers, were HLA typed. Using immunohistochemical stains, the BA livers were examined for MM. Four additional BA livers underwent analysis by polymerase chain reaction (PCR for evidence of MM. Results By FISH, 8 BA and 2 NH livers were interpretable. Seven of eight BA specimens showed evidence of MM. The number of maternal cells ranged from 2–4 maternal cells per biopsy slide. Neither NH specimen showed evidence of MM. In addition, immunohistochemical stains confirmed evidence of MM. Using PCR, a range of 1–142 copies of maternal DNA per 25,000 copies of patients DNA was found. Conclusions Maternal microchimerism is present in the livers of patients with BA and may contribute to the pathogenesis of BA.

  19. 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

    in infants with biliary atresia. PATIENTS AND METHODS: From Dutch and Danish national biliary atresia registries, we retrieved infants who were either breastfed and received 1 mg of oral vitamin K at birth followed by 25 microg of daily oral vitamin K prophylaxis (Netherlands, 1991-2003), 2 mg of oral...

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

    Science.gov (United States)

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

    2015-06-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2014-03-28

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

  3. Endotoxin and CD14 in the progression of biliary atresia

    Directory of Open Access Journals (Sweden)

    Chen Ching-Mei

    2010-12-01

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

  4. Hepatocellular carcinoma in the native liver of a 38-year-old female patient with biliary atresia

    Directory of Open Access Journals (Sweden)

    Yutaka Kanamori

    2015-11-01

    Full Text Available We report a rare case of hepatocellular carcinoma in native liver in a case of biliary atresia. The patient was a 38-year-old female with three children who had an aggressive tumor, resulting in her subsequent death. We also review 14 reports, published previously in the English language medical literature, concerning hepatocellular carcinoma originating from native liver in biliary atresia cases and discuss the possible etiology, and propose more careful follow up for the patients with biliary atresia who suffer from repetitive cholangitis and/or experience the child delivery.

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

    Directory of Open Access Journals (Sweden)

    Praveen Mathur

    2014-01-01

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

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

    DEFF Research Database (Denmark)

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

    2015-01-01

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

  7. 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

    . 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......: During the period, 73 patients where operated with a portoenterostomy ad modum Kasai. Patients older than 84 days at the time of operation were excluded, 54 patients were available for analysis. Conjugated bilirubin in μmol/l and the percentage value were significantly above the DHMA threshold limit......: mean 129.7 μmol/l (42-334 μmol/l) and 73% (28-97%), respectively. CONCLUSION: The total amount of conjugated bilirubin above 20 μmol/l is sufficient to require further evaluation for biliary atresia. The percentage value is unnecessary and may cause confusion. FUNDING: none. TRIAL REGISTRATION...

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

    Directory of Open Access Journals (Sweden)

    Elisa de Carvalho

    2007-04-01

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

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

    Directory of Open Access Journals (Sweden)

    Jiexiong Feng

    Full Text Available Biliary atresia is a common disease in neonates which causes obstructive jaundice and progressive hepatic fibrosis. Our previous studies indicate that rotavirus infection is an initiator in the pathogenesis of experimental biliary atresia (BA through the induction of increased nuclear factor-kappaB and abnormal activation of the osteopontin inflammation pathway. In the setting of rotavirus infection, rotavirus nonstructural protein 4 (NSP4 serves as an important immunogen, viral protein 7 (VP7 is necessary in rotavirus maturity and viral protein 4 (VP4 is a virulence determiner. The purpose of the current study is to clarify the roles of NSP4, VP7 and VP4 in the pathogenesis of experimental BA. Primary cultured extrahepatic biliary epithelia were infected with Rotavirus (mmu18006. Small interfering RNA targeting NSP4, VP7 or VP4 was transfected before rotavirus infection both in vitro and in vivo. We analyzed the incidence of BA, morphological change, morphogenesis of viral particles and viral mRNA and protein expression. The in vitro experiments showed NSP4 silencing decreased the levels of VP7 and VP4, reduced viral particles and decreased cytopathic effect. NSP4-positive cells had strongly positive expression of integrin subunit α2. Silencing of VP7 or VP4 partially decreased epithelial injury. Animal experiments indicated after NSP4 silencing, mouse pups had lower incidence of BA than after VP7 or VP4 silencing. However, 33.3% of VP4-silenced pups (N = 6 suffered BA and 50% of pups (N = 6 suffered biliary injury after VP7 silencing. Hepatic injury was decreased after NSP4 or VP4 silencing. Neither VP4 nor VP7 were detected in the biliary ducts after NSP4. All together, NSP4 silencing down-regulates VP7 and VP4, resulting in decreased incidence of BA.

  10. Outcomes in children with biliary atresia following liver transplantation

    Institute of Scientific and Technical Information of China (English)

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

    2013-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Madhavi Nori

    2013-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2000-01-01

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

  13. Interleukin-32 production associated with biliary innate immunity and proinflammatory cytokines contributes to the pathogenesis of cholangitis in biliary atresia.

    Science.gov (United States)

    Okamura, A; Harada, K; Nio, M; Nakanuma, Y

    2013-08-01

    Biliary atresia (BA) is thought to be associated with infections by viruses such as Reoviridae and is characterized histologically by fibrosclerosing cholangitis with proinflammatory cytokine-mediated inflammation. Interleukin (IL)-32 affects the continuous inflammation by increasing the production of proinflammatory cytokines. In this study, the role of IL-32 in the cholangitis of BA was examined. Immunohistochemistry for IL-32 and caspase 1 was performed using 21 samples of extrahepatic bile ducts resected from BA patients. Moreover, using cultured human biliary epithelial cells (BECs), the expression of IL-32 and its induction on stimulation with a Toll-like receptor [(TLR)-3 ligand (poly(I:C)] and proinflammatory cytokines was examined. BECs composing extrahepatic bile ducts showing cholangitis expressed IL-32 in BA, but not in controls. Caspase 1 was expressed constantly on BECs of both BA and control subjects. Furthermore, poly(I:C) and proinflammatory cytokines [(IL-1β, interferon (IFN)-γ and tumour necrosis factor (TNF)-α] induced IL-32 expression strongly in cultured BECs, accompanying the constant expression of TLR-3 and caspase 1. Our results imply that the expression of IL-32 in BECs was found in the damaged bile ducts of BA and induced by biliary innate immunity via TLR-3 and proinflammatory cytokines. These findings suggest that IL-32 is involved initially in the pathogenic mechanisms of cholangitis in BA and also plays an important role in the amplification and continuance of periductal inflammatory reactions. It is therefore tempting to speculate that inhibitors of IL-32 could be useful for attenuating cholangitis in BA.

  14. 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.

  15. γδ T cells and Foxp3(+) Treg cells infiltration in children with biliary atresia and its significance.

    Science.gov (United States)

    Li, Kang; Zhang, Xi; Tang, Shao-Tao; Yang, Li; Cao, Guo-Qing; Li, Shuai; Yang, De-Hua

    2015-01-01

    To investigate the changes in the proportion of γδ T cells and Foxp3(+) Treg cells in children with BA (biliary atresia). The distribution of γδ T cells in the liver tissues and the proportion of γδ T cells and Foxp3(+) Treg cells were observed and detected in BA Group (32 cases) and control group (CG) (12 cases) by using immunohistochemical methods and flow cytometry. The periportal bile duct of liver in BA Group was surrounded by a large number of γδ T cells and a certain degree of Foxp3(+) Treg cells infiltration. Additionally, the proportion of γδ T cells and Foxp3(+) Treg cells was significantly higher than that in CG (PTreg cells (PTreg cell proliferation in liver tissues of patients with biliary atresia exacerbated the progressive inflammatory injury of bile ducts.

  16. Serum adiponectin and transient elastography as non-invasive markers for postoperative biliary atresia

    Directory of Open Access Journals (Sweden)

    Udomsinprasert Wanvisa

    2011-02-01

    Full Text Available Abstract Background Biliary atresia (BA is a progressive inflammatory disorder of the extrahepatic bile ducts leading to the obliteration of bile flow. The purpose of this study was to determine serum adiponectin in BA patients and to investigate the relationship of adiponectin with clinical parameters and liver stiffness scores. Methods Sixty BA patients post Kasai operation and 20 controls were enrolled. The mean age of BA patients and controls was 9.6 ± 0.7 and 10.1 ± 0.7 years, respectively. BA patients were classified into two groups according to their serum total bilirubin (TB levels (non-jaundice, TB Results BA patients had markedly higher serum adiponectin levels (15.5 ± 1.1 vs. 11.1 ± 1.1 μg/ml, P = 0.03 and liver stiffness than controls (30.1 ± 3.0 vs. 5.1 ± 0.5 kPa, P P P r = 0.58, r = 0.46, and r = 0.60, P Conclusions Serum adiponectin and liver stiffness values were higher in BA patients compared with normal participants. The elevated serum adiponectin levels also positively correlated with the degree of hepatic dysfunction and liver fibrosis. Accordingly, serum adiponectin and transient elastography could serve as the useful non-invasive biomarkers for monitoring the severity and progression in postoperative BA.

  17. Transient elastography for predicting esophageal/gastric varices in children with biliary atresia

    Directory of Open Access Journals (Sweden)

    Siripon Nipaporn

    2011-04-01

    Full Text Available Abstract Background Transient elastography (TE is an innovative, noninvasive technique to assess liver fibrosis by measuring liver stiffness in patients with chronic liver diseases. The purpose of this study has been to explore the accuracy of TE and clinical parameters in predicting the presence of esophageal/gastric varices in children with biliary atresia (BA following portoenterostomy. Methods Patients with BA status post portoenterostomy and normal children were recruited. Splenomegaly and presence of EV/GV were determined by physical examination and endoscopy, respectively. Aspartate transaminase to platelet ratio index (APRI was used as a serum fibrosis marker. TE was performed by using FibroScan. Data was expressed as mean ± SD. Results Seventy-three BA patients (male:female = 32:41; age 9.11 ± 5.64 years and 50 normal controls (male:female = 19:31; age 11.00 ± 3.31 years were enrolled. The liver stiffness score of BA patients was significantly higher than that of normal controls (27.37 ± 22.48 and 4.69 ± 1.03 kPa; p Conclusions Transient elastography is a useful tool for predicting the presence of EV/GV. In addition, basic physical examination, routine biochemical and hematological tests, are still worthwhile and correlate well with the presence of EV/GV in patients with BA post portoenterostomy.

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

    Directory of Open Access Journals (Sweden)

    Rachel A. Kramer

    2009-01-01

    Full Text Available Objectives. To assess bone health in a cohort of nonjaundiced children with biliary atresia (BA and the effect of growth and development on bone outcomes. Methods. Children ages one to eighteen years receiving care from Children's Hospital of Philadelphia were recruited. Each child was seen once and assessed for growth, pubertal development, concurrent medications, bilirubin, ALT, albumin, vitamin D status, bone mineral density (BMD, and bone mineral content (BMC of the lumbar spine and whole body. Results. BMD declined significantly with age, and upon further analysis with a well-phenotyped control cohort, it was found that BMC was significantly decreased for both lumbar spine and whole body, even after adjustment for confounding variables. An age interaction was identified, with older subjects having a significantly greater impairment in BMC. Conclusions. These preliminary results demonstrate that children with BA, including those without jaundice, are likely to have compromised bone health even when accounting for height and puberty, which are common confounding factors in chronic disease. Further investigation is needed to identify the determinants of poor bone mineral status and to develop strategies to prevent osteoporosis later in life.

  19. Ultrasonic diagnosis of biliary atresia: A retrospective analysis of 20 patients

    Institute of Scientific and Technical Information of China (English)

    Shi-Xing Li; Yao Zhang; Mei Sun; Bo Shi; Zhong-Yi Xu; Ying Huang; Zhi-Qin Mao

    2008-01-01

    AIM: To investigate the clinical value of ultrasonographic diagnosis of biliary atresia (BA), a retrospective analysis of the sonogram of 20 children with BA was undertaken.METHODS: Ultrasonography (US) was performed in 20 neonates and infants with BA, which was confirmed with cholangiography by operation or abdominoscopy. The presence of triangular cord, the size and echo of liver, the changes in empty stomach gallbladder and postprandial gallbladder were observed and recorded.RESULTS: The triangular cord could be observed at the porta hepatis (thickness: 0.3-0.6cm) in 10 cases. Smaller triangular cord (0.2-0.26cm) can be observed in 3 cases. The gallbladder was not observed in 2 cases, and 1 case showed a streak gallbladder without capsular space. The gallbladders of 15 cases were flat and small. The gallbladders of 2 cases were of normal size and appearance, however, there was no postprandial contraction. The livers of all cases showed hepatomegaly and hetreogeneous echogenicity. Statistical analysis was performed to compare the hepatomegaly and hetreogeneous echogenicity and the stage of hepatic fibrosis.CONCLUSION: The presence of the triangular cord at the porta hepatis is specific. However, it is not the only diagnostic criterion, since flat and small gallbladder and poor contraction are also of important diagnostic and differential diagnostic significance. The degree of hepatomegaly and hetreogeneous echogenicity is proportional with liver fibrosis, and able to indicate the duration of course and prognosis.

  20. There is no association between K469E ICAM-1 gene polymorphism and biliary atresia

    Institute of Scientific and Technical Information of China (English)

    Paisarn Vejchapipat; Naruemol Jirapanakom; Nutchanart Thawornsuk; Apiradee Theamboonlers; Voranush Chongsrisawat; Soottiporn Chittmittrapap; Yong Poovorawan

    2005-01-01

    AIM: To determine whether there was an association between inter-cellular adhesion molecule-1 (ICAM-1) gene polymorphism and biliary atresia (BA), and to investigate the relationship between serum soluble ICAM-1 (sICAM-1)and clinical outcome in BA patients after surgical treatment.METHODS: Eighty-three BA patients and 115 normal controls were genotyped. K469EICAM-1 polymorphism was analyzed using PCR assay. Serum sICAM-1 was determined using ELISA method from 72 BA patients. In order to evaluate the association between these variables and their clinical outcome, the patients were categorized into two groups:patients without jaundice and those with persistent jaundice.RESULTS: There were no significant differences between BA patients and controls in terms of gender, K469E ICAM-1genotypes, and alleles. The proportion of patients having serum sICAM-1 ≥3 500 ng/mL in persistent jaundice group was significantly higher than that in the other group. In addition, there was no association between K469EICAM-1polymorphism and the status of jaundice in BA patients after Kasai operation.CONCLUSION: ICAM-1 possibly plays an important and active role in the disease progression. However, the process is not associated with genetic variation of K469EICAM-1 polymorphism.

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

    Science.gov (United States)

    Udomsinprasert, Wanvisa; Kitkumthorn, Nakarin; Mutirangura, Apiwat; Chongsrisawat, Voranush; Poovorawan, Yong; Honsawek, Sittisak

    2017-01-01

    Objective 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. Methods 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. Results 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. Conclusion 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. PMID:28052132

  2. Telomere Length in Peripheral Blood Leukocytes Is Associated with Severity of Biliary Atresia.

    Directory of Open Access Journals (Sweden)

    Wanvisa Udomsinprasert

    Full Text Available The purpose of this study was to investigate the association of telomere length in peripheral blood leukocytes with the severity of biliary atresia (BA.One hundred and fourteen BA patients and 114 age-matched healthy controls were enrolled. Relative telomere length (RTL was assessed using a quantitative real-time polymerase chain reaction. Multivariate regression analysis was used to estimate RTL as an independent risk factor of BA. Receiver operating characteristic curve analysis was used to calculate the accuracy of biomarkers in the prediction of liver cirrhosis.BA patients had significantly shorter telomeres than healthy controls (p < 0.0001. The RTL in BA patients with jaundice was considerably lower than that of patients without jaundice (p = 0.005. Moreover, RTL was markedly shorter in patients with cirrhosis (F4, as compared to patients with mild fibrosis (F2 and non-fibrosis (F0-F1, p < 0.0001. Logistic regression analysis indicated that short RTL was associated with a higher risk of liver cirrhosis in BA. Tertile analysis showed a dose-response effect for this association (p trend < 0.0001. Additionally, RTL in BA children revealed a negative correlation with age (r = -0.50, p < 0.001. We noted an association between reduction of RTL and liver stiffness scores, adjusted for age and gender (b = -0.01, p < 0.0001. Short RTL can be employed to distinguish cirrhosis patients from non-cirrhosis patients (AUC = 0.78. Further analysis showed a linear correlation between leukocyte RTL and liver RTL in BA patients (r = 0.83, p < 0.001.The findings of this study provide evidence that telomere shortening is associated with an elevated risk of liver cirrhosis in BA.

  3. RRAS: A key regulator and an important prognostic biomarker in biliary atresia

    Institute of Scientific and Technical Information of China (English)

    Rui Zhao; Hao Li; Chun Shen; Shan Zheng

    2011-01-01

    AIM: To characterize the differentially expressed gene profiles in livers from biliary atresia (BA) patients including,ascertain genes, functional categories and pathways that play a central role in the pathogenesis of BA,and identify the novel prognostic markers for BA.METHODS: Liver tissue samples from control patients,neonatal cholestasis patients, and BA patients at the age of 90 d were pooled for DNA microarray analysis. Bioinformatics analysis was performed using, series test cluster of gene ontology,and Pathway-Finder software. Reverse-transcription polymerase chain reaction was performed to confirm changes in selected genes. Relation between RRAS gene expression and prognosis of 40 BA patients was analyzed in a 2-year follow-up study.RESULTS: The 4 identified significant gene expression profiles could confidently separate BA liver tissue from normal and other diseased liver tissues. The included genes were mainly involved in inflammation response and reconstruction of cellular matrix. The significant pathways associated with BA were primarily involved in autoimmune response, activation of T lymphocytes and its related cytokines. The RRAS , POMC , SLC26A6 and STX3 genes were important regulatory modules in pathogenesis of BA. The expression of RRAS was negatively correlated with the elimination rate of jaundice and positively correlated with the occurrence rate of cholangitis.CONCLUSION: Autoimmune response mediated by T lymphocytes may play a vital role in the pathogenesis of BA. The RRAS gene is an important regulatory module in the pathogenesis of BA, which may serve as a novel prognostic marker for BA.

  4. Pattern and survival of biliary atresia patients; Experience in Southern Nigeria

    Directory of Open Access Journals (Sweden)

    Philemon E Okoro

    2013-01-01

    Full Text Available Background: Biliary atresia (BA has been a challenge to surgeons worldwide. Beyond the revolutionary surgical technique popularised by Kasai, liver transplantation has added renewed hope in the long-term outcome. In Nigeria, where late presentation is very common, there is need to assess the long-term results of the treatment options available to us. Aim: We aimed to evaluate the presentation and management outcome of BA and the long-term survival of BA patients seen in our practice. Materials and Methods: Cases of BA seen between January 2007 and December 2011 in three tertiary health facilities in South East Nigeria were included. Data obtained included age at presentation, clinical features, treatment offered and age at the time of death. Analysis was with the SPSS 17.0. Results: Twenty four patients comprising 10 (41.7% males and 14 (58.3% females were included in the study. The mean age of presentation was 4.02 (±214 months; range 1.75-11.0 months. Fifteen (62.5% patients had surgery while 9 (37.5% received medical treatment only. The mean age at death was 14.2 (±8.1 months; range 2.5-30 months. Conclusion: BA poses a daunting challenge in our practice. Outcome of treatment is still discouraging. We identified late presentation, lack of facilities to make early diagnosis, lack of adequately trained manpower to manage these children and lack of post-operative care and support for patients as the major challenges in the management of BA children in our region.

  5. Comparison of Accuracy of Ultrasonography and Isotope scan in Diagnosis of Extrahepatic Biliary Atresia

    Directory of Open Access Journals (Sweden)

    M. Nemati

    2008-01-01

    Full Text Available Background/Objective: Infantile cholestasis continues to represent a diagnostic chalange. Prolonges conjugated hyperbilirubinemia can be caused by extrahepatic biliary atresia (EHBA or by nonobstructive metabolic and inflammatory causes. "nNowadays, isotopscanning including HIDA is the most popular method to rule out of EHBA and finally liver biopsy is the last step in preoperative confirmation of EHBA. Currently the role of ultrasound in these patients is a dilemma and contraversial. "nPatients and Methods: In this prospective study, 49 infants with prolonged jaundice were studied between January 2004 and April 2006 in Tabriz children's Hospital. All of these infants were evaluated with ultrasonography and isotopscan and finally all of them were biopsied under guide of sonography. "nIn their Sonogrphic evaluation, absence or pres-ence of gall bladder, its size and wall thickness and also its depletion after nutrition was controlled and beside this, triangular cord sign (TACS was checked in all of them."nResults: In diagnosis of EHBA, the PPV, NPV and accuracy of nonvisulaization of gall bladder is 75%, 81.4%, 79% respectively. The accuracy of postnutrition depletion of gall blodder in diagnosis of EHBA is 90%. "nThe PPV of TACS in diagnosis of EHBA is 100% and its NVP and accuracy are 78.5%, 80%."nOverall accuracy, PPV, NPV, specificity and sensitivity of sonographic technics (in combination with each other is 93%, 92%, 94%, 97%, 86% respectively."nOn the other hand, though the NPV and sensitivity of isotopscan is 100% but due to high false positive findings, its PPV, specificity and accuracy are low (46.6%, 51.3%, 66% respectively."nConclusion: Ultrasonographic findings are more accurate, more specific and its positive results are more reliable than isotopscanning in diagnosis of EHBA.

  6. The correlation between plasma cytokine levels in jaundice-free children with biliary atresia

    Institute of Scientific and Technical Information of China (English)

    Zhi-Hong Jian; Li-Ching Wang; Chieh-Chung Lin; Jiaan-Der Wang

    2015-01-01

    Background: T helper (Th) cell cytokines modulate inflammation and play a role in biliary atresia (BA). The aim of the study is a cross-sectional assessment of the levels of Th cytokines in the jaundice-free post Kasai procedure patients. Methods: There were 40 jaundice-free patients with BA and 28 normal controls enrolled. Patients were divided into 3 groups, including normal liver function, impaired liver function, and portal hypertension. Plasma concentration of Th1 [interferon-γ (INF-γ), interleukin (IL)-2], Th2 (IL-4, IL-10), Th3 [transforming growth factor-β1 (TGF-β1)], Th17 (IL-17) cytokines, and stromal cell-derived factor-1α (SDF-1α) were investigated. Results: The IFN-γ level was significantly higher in the BA patients with impaired liver function and portal hypertension than controls (P<0.0001 andP<0.0001, respectively). There was a signifi cantly increase of TGF-β1 in all BA groups compared with controls (P=0.003). The reduction of SDF-1α expression was found in BA groups (P<0.0001). IL-10 levels significantly correlated with aspartate aminotransferase to platelet ratio index (r=0.496, P=0.001). For the cytokine correlations, there were no correlations of Th1, Th2 and Th17 cytokine with the other measured cytokines, but TGF-β1 was negatively correlated with SDF-1α levels (r=-0.327,P=0.039). Conclusions: IFN-γ and IL-10 are likely to be involved in the disease progression in BA. Besides, TGF-β1 is found to be a suppression marker associated with SDF-1α levels and reduced production of TGF-β1 may be associated with the disease progression.

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

    Directory of Open Access Journals (Sweden)

    Vikram Khanna

    2016-01-01

    Full Text Available Aim: To evaluate the incidence of portal hypertension (PHT in biliary atresia (BA patients and to monitor its progress after Kasai portoenterostomy (KP by measuring nitric oxide (NO levels in peripheral blood. Materials and Methods: A prospective cross-sectional study conducted over a period of 2 years. Intraoperative portal pressure (PP and blood NO levels at presentation, 1-month, 3-month, and 6-month follow-up, were correlated with clinical and biochemical parameters in BA patients. The mean NO level in age-matched control group was 4.64 ± 2.32 μmol/L. Results: Thirty-four BA patients underwent KP over a period of 2 years. The mean age of presentation was 2.7 months (range 1-4 months. The mean intraoperative PP was 21.3 ± 5.4 mmHg. The mean PP in patients aged 90 days was 18.53 ± 4.45 mmHg, 20.33 ± 3.07 mmHg, and 26.5 ± 5.01 mmHg, respectively. The mean PP in the patients who underwent successful KP was 16.75 ± 3.54 mmHg while for those who continued to have jaundice it was 23.94 ± 4.63 mmHg (P < 0.001. NO levels closely followed the PP as shown by the regression equation NO = 4.79 + 0.64 PP mmHg, R2 = 0.69. The mean NO level at presentation was 18.48 ± 4.17 μmol/L and at 1-month, 3-month, and 6-month follow-up was 11.94 ± 5.62 μmol/L, 10.79 ± 6.02 μmol/L, and 9.93 ± 6.53 μmol/L, respectively (P < 0.001. The difference in NO levels was also statistically significant between the patients who cleared jaundice and those with persisting jaundice. Conclusion: All BA patients had PHT at presentation. PHT worsens with age and has an adverse effect on outcome of KP. NO levels in blood closely follow PP and higher levels are associated with poor outcome.

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

    Science.gov (United States)

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

    2016-01-01

    Aim: To evaluate the incidence of portal hypertension (PHT) in biliary atresia (BA) patients and to monitor its progress after Kasai portoenterostomy (KP) by measuring nitric oxide (NO) levels in peripheral blood. Materials and Methods: A prospective cross-sectional study conducted over a period of 2 years. Intraoperative portal pressure (PP) and blood NO levels at presentation, 1-month, 3-month, and 6-month follow-up, were correlated with clinical and biochemical parameters in BA patients. The mean NO level in age-matched control group was 4.64 ± 2.32 μmol/L. Results: Thirty-four BA patients underwent KP over a period of 2 years. The mean age of presentation was 2.7 months (range 1-4 months). The mean intraoperative PP was 21.3 ± 5.4 mmHg. The mean PP in patients aged 90 days was 18.53 ± 4.45 mmHg, 20.33 ± 3.07 mmHg, and 26.5 ± 5.01 mmHg, respectively. The mean PP in the patients who underwent successful KP was 16.75 ± 3.54 mmHg while for those who continued to have jaundice it was 23.94 ± 4.63 mmHg (P < 0.001). NO levels closely followed the PP as shown by the regression equation NO = 4.79 + 0.64 PP mmHg, R2 = 0.69. The mean NO level at presentation was 18.48 ± 4.17 μmol/L and at 1-month, 3-month, and 6-month follow-up was 11.94 ± 5.62 μmol/L, 10.79 ± 6.02 μmol/L, and 9.93 ± 6.53 μmol/L, respectively (P < 0.001). The difference in NO levels was also statistically significant between the patients who cleared jaundice and those with persisting jaundice. Conclusion: All BA patients had PHT at presentation. PHT worsens with age and has an adverse effect on outcome of KP. NO levels in blood closely follow PP and higher levels are associated with poor outcome. PMID:27046973

  9. Distal splenorenal shunt with splenopancreatic disconnection for portal hypertension in biliary atresia.

    Science.gov (United States)

    Hasegawa, T; Tamada, H; Fukui, Y; Tanano, H; Okada, A

    1999-01-01

    This study evaluated the long-term effects of distal splenorenal shunt with splenopancreatic disconnection (DSRS-SPD) on portal hypertension (PH) in biliary atresia (BA) patients. Five patients with BA underwent DSRS-SPD at the age of 3.3 to 8.5 years. They had been free from jaundice after hepatic portoenterostomy (HPE); however, they gradually developed gastroesophageal varices and hypersplenism. Portal venous pressure after anastomosis was 37.2 +/- 6.1 cmH2O, as high as that before anastomosis (37.8 +/- 3.3 cmH2O). Postoperatively, liver function tests became worse within 2 weeks; however, they returned to preoperative levels within 1 month without any further treatment. No patient developed a significant encephalopathy throughout the observed period. During follow-up of 4 to 12 years, the shunt was patent in all patients. Spleen size decreased after operation. Abdominal-wall venous dilatation completely disappeared in two of four patients. The platelet counts gradually increased and were significantly higher 3 years (126.6 +/- 59.3 x 10(3)/mm3) after DSRS-SPD than preoperative values (66.0 +/- 24.2 x 10(3)/mm3). White blood cell counts showed no significant changes. No patient developed a gastrointestinal hemorrhage postoperatively, although three had had repeated hemorrhages before the operation. Two patients showed disappearance of varices endoscopically at 2 years and 7 months after DSRS-SPD, respectively, but had recurrent varices at 7 and 11 years, respectively. The endoscopic findings regarding varices 3 to 7 years after DSRS-SPD were as follows: decreased number (80%); decreased length (40%); improvement of form (20%); improvement of fundamental color (60%); disappearance of red-color sign (100%); disappearance of gastric varices (75%); and disappearance of acute gastric mucosal lesions (100%). Although one patient later underwent liver transplantation because of progression of liver cirrhosis, all five are doing well. From these results, DSRS-SPD may

  10. MR Cholangiography and Dynamic Examination of Duodenal Fluid in the Differential Diagnosis between Extrahepatic Biliary Atresia and Infantile Hepatitis Syndrome

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    In order to evaluate the value of magnetic resonance cholangiography (MRC) and dynamic examination of duodenal fluid in the differential diagnosis between extrahepatic biliary atresia (EHBA) and infantile hepatitis syndrome (IHS), 52 pa tients with infantile cholestatic jaundice were examined by MRC and duodenal fluid examination. Original interpretations were compared with clinical outcome. Calculated sensitivity of duodenal fluid examination in diagnosis of EHBA was 100%, and specificity was 91.1%. Sensitivity of MRC in the diagnosis of EHBA was 94.4 % and specificity 88.24 %. The sensitivity of MRC and examination of duodenal fluid combined in diagnosis of EHBA was 94.4 % and specificity 97.06 %. We are led to conclude that MRC and dynamic examination of duodenal fluid are useful in the differential diagnosis between IHS and EHBA and the combined use of the two techniques yield better resutls.

  11. Custom-made covered transjugular intrahepatic portosystemic shunt (TIPS) in an infant with trisomy 22 and biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Chlapoutaki, Chrysanthi Emmanouil; Franchi-Abella, Stephanie; Pariente, Daniele [Bicetre Hospital University Paris XI, Assistance Publique Hopitaux de Paris, Department of Paediatric Radiology, Paris (France); Habes, Dalila [Bicetre Hospital University Paris XI, Assistance Publique Hopitaux de Paris, Pediatric Hepatology and National Reference Center for Biliary Atresia, Paris (France)

    2009-07-15

    We report an 8-month-old girl with portal hypertension secondary to biliary atresia. The decision to treat with TIPS was made at the age of 8 months due to recurrent variceal bleeding. The procedure was carried out with a 6-mm bare stent due to her small size. Radiological follow-up with Doppler US showed gradual stenosis and finally occlusion of the stent 80 days after implantation. Revision was performed with placement of an additional 6-mm expanded polytetrafluoroethylene (e-PTFE) stent-graft that had remained patent for 9 months, proving that in small children with a portal vein diameter less than 8 mm, the combination of a bare stent and stent-graft can provide excellent results. (orig.)

  12. Evaluation of usefulness of [sup 99m]Tc-GSA liver scintigraphy in postoperative biliary atresia patients

    Energy Technology Data Exchange (ETDEWEB)

    Kurono, Kenji; Mizutani, Hirokazu; Ohba, Satoru; Hashimoto, Takashi; Yura, Jirou (Nagoya City Univ. (Japan). Faculty of Medicine)

    1994-06-01

    Liver scintigraphy was performed using a newly developed radiopharmaceutical, [sup 99m]Tc-DTPA-galactosyl-human serum albumin ([sup 99m]Tc-GSA), in 17 patients after hepatic portenterostomy for biliary atresia (age: 3 months-14 years old, body weight: 5.8-39 kg). These patients were divided into two groups; good hepatic function group was 3 cases which had no symptom after operation, and poor hepatic function group was 14 cases which needed some therapeutic procedures for ascending cholangitis, GI tract bleeding etc. Dynamic data were obtained by a gamma camera during 20 minutes after intravenous injection of 1.5-3.0 mg (92.5-185 MBq) of [sup 99m]Tc-GSA. The livers were clearly delineated in all cases. We calculated HH[sub 15] (clearance index) for the index of clearance and LHL[sub 15] (receptor index) for the index of hepatic uptake. In good hepatic function group, HH[sub 15] was 0.396[+-]0.059 (mean[+-]S.D.), LHL[sub 15] was 0.960[+-]0.009. In poor hepatic function group, HH[sub 15] was 0.629[+-]0.080, LHL[sub 15] was 0.875[+-]0.060. The difference between the mean value of both indices was statistically significant. Using single photon emission CT, inhomogenisity of liver accumulation of [sup 99m]Tc-GSA was clarified, and focal hepatocyte damage, which could not be detected on CT, was able to be estimated. We consider that [sup 99m]Tc-GSA liver scintigraphy is a useful study for continuous observation of the patients with biliary atresia. (author).

  13. Rhesus rotavirus VP4 sequence-specific activation of mononuclear cells is associated with cholangiopathy in murine biliary atresia.

    Science.gov (United States)

    Walther, Ashley; Mohanty, Sujit K; Donnelly, Bryan; Coots, Abigail; Lages, Celine S; Lobeck, Inna; Dupree, Phylicia; Meller, Jaroslaw; McNeal, Monica; Sestak, Karol; Tiao, Greg

    2015-09-15

    Biliary atresia (BA), a neonatal obstructive cholangiopathy, remains the most common indication for pediatric liver transplantation in the United States. In the murine model of BA, Rhesus rotavirus (RRV) VP4 surface protein determines biliary duct tropism. In this study, we investigated how VP4 governs induction of murine BA. Newborn mice were injected with 16 strains of rotavirus and observed for clinical symptoms of BA and mortality. Cholangiograms were performed to confirm bile duct obstruction. Livers and bile ducts were harvested 7 days postinfection for virus titers and histology. Flow cytometry assessed mononuclear cell activation in harvested cell populations from the liver. Cytotoxic NK cell activity was determined by the ability of NK cells to kill noninfected cholangiocytes. Of the 16 strains investigated, the 6 with the highest homology to the RRV VP4 (>87%) were capable of infecting bile ducts in vivo. Although the strain Ro1845 replicated to a titer similar to RRV in vivo, it caused no symptoms or mortality. A Ro1845 reassortant containing the RRV VP4 induced all BA symptoms, with a mortality rate of 89%. Flow cytometry revealed that NK cell activation was significantly increased in the disease-inducing strains and these NK cells demonstrated a significantly higher percentage of cytotoxicity against noninfected cholangiocytes. Rotavirus strains with >87% homology to RRV's VP4 were capable of infecting murine bile ducts in vivo. Development of murine BA was mediated by RRV VP4-specific activation of mononuclear cells, independent of viral titers.

  14. Primary Biliary Cholangitis (Primary Biliary Cirrhosis)

    Science.gov (United States)

    ... Liver Disease & NASH Definition & Facts Symptoms & Causes Diagnosis Treatment Eating, Diet, & Nutrition Clinical Trials Biliary Atresia Cirrhosis Hemochromatosis Hepatitis A through E (Viral Hepatitis) Hepatitis ...

  15. Dendritic Cells Regulate Treg-Th17 Axis in Obstructive Phase of Bile Duct Injury in Murine Biliary Atresia.

    Science.gov (United States)

    Liu, Yong-Jun; Li, Kang; Yang, Li; Tang, Shao-Tao; Wang, Xin-Xing; Cao, Guo-Qing; Li, Shuai; Lei, Hai-Yan; Zhang, Xi

    2015-01-01

    Several cell types are considered to be effector cells in bile duct injury in rhesus rotavirus (RRV)-induced experimental biliary atresia (BA). Here, we identified an increased T helper 17 (Th17) cell population in a BA mode. By depleting the Th17 cells, the BA symptoms (onset of jaundice, acholic stools and retarded growth) were attenuated and the survival rate was improved. Furthermore, we found that in mice with BA, the percentage of CD4+CD25highFoxp3+ T regulatory (Treg) cells decreased along with the increased percentage of Th17 cells. However, the absolute numbers of Treg and Th17 cells were both increased in liver of RRV-injected mice compared to saline-injected mice. The proportion of Th17 cells at 7 days post-infection was decreased if Treg cells isolated from normal adult mice, but not Treg cells from the livers of mice with BA, were intraperitoneally transferred on day 5 of life. In vitro experiments also showed that Treg cells from mice with BA had a diminished suppressive effect on Th17 cell generation. To determine the mechanisms, we investigated the production of cytokines in the liver. The level of IL-6, which has been shown to be abundantly secreted by activated dendritic cells (DCs), was remarkably elevated. Importantly, in a Treg/Th17 cell suppression assay, IL-6 was demonstrated to paralyze the Treg cells' suppressive effect on Th17 cells and eventually the unrestrained increase of Th17 cells contributed to bile duct injury. In conclusion, the DC-regulated Treg-Th17 axis, probably in conjunction with other effector T cells, aggravates progressive inflammatory injury at the time of ductal obstruction.

  16. Cross-sectional Multi-center Analysis of Portal Hypertension in 163 Children and Young Adults with Biliary Atresia

    Science.gov (United States)

    Shneider, Benjamin L.; Abel, Bob; Haber, Barbara; Karpen, Saul J.; Magee, John C.; Romero, Rene; Schwarz, Kathleen; Bass, Lee M.; Kerkar, Nanda; Miethke, Alexander G.; Rosenthal, Philip; Turmelle, Yumirle; Robuck, Patricia R.; Sokol, Ronald J.

    2012-01-01

    Objectives Biliary atresia (BA) frequently results in portal hypertension (PHT), complications of which lead to significant morbidity and mortality. The Childhood Liver Disease Research and Education Network (ChiLDREN) was utilized to perform a cross-sectional multi-centered analysis of PHT in children with BA. Methods BA subjects receiving medical management at a ChiLDREN site were enrolled. A priori, clinically evident PHT was defined as “definite” when there was either 1) history of a complication of PHT or 2) clinical findings consistent with PHT (both splenomegaly and thrombocytopenia). PHT was denoted as “possible” if one of the findings was present in the absence of a complication, while PHT was “absent” if none of the criteria were met. Results 163 subjects were enrolled between May 2006 and December 2009. At baseline, definite PHT was present in 49%, possible in 17% and absent in 34% of subjects. Demographics, growth and anthropometrics were similar amongst the 3 PHT categories. ALT, GGTP, and sodium levels were similar, while there were significant differences in AST, AST/ALT, albumin, total bilirubin, PT, WBC, platelet count and AST/platelet between definite and absent PHT. Thirty-four percent of those with definite PHT had either PT > 15s or albumin < 3 g/L. Conclusions Clinically definable PHT is present in two thirds of North American long-term BA survivors with their native livers. The presence of PHT is associated with measures of hepatic injury and dysfunction, although in this selected cohort the degree of hepatic dysfunction is relatively mild and growth is preserved. PMID:22903006

  17. 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.)

  18. Tratamento cirúrgico da atresia de vias biliares: a experiência do hospital municipal jesus, 1997-2000 Surgical treatment of biliary atresia: the hospital municipal jesus experience 1997-2000

    Directory of Open Access Journals (Sweden)

    Lisieux Eyer de Jesus

    2001-04-01

    Full Text Available OBJETIVO: Analisar a indicação, resultados e limitações da portoenterostomia para o tratamento da atresia de vias biliares em hospital infantil terciário no Rio de Janeiro-RJ/Brasil. MÉTODO: Foram estudados prospectivamente oito pacientes submetidos à portoenterostomia do tipo Kasai 1, num período de três anos (1997-2000. Todos foram submetidos à antibioticoterapia profilática, uso de colerético, reavaliação médica mensal nos primeiros seis meses de seguimento e reavaliação laboratorial, ultra-sonográfica e através de endoscopia digestiva alta em prazos programados. RESULTADOS: Em cinco pacientes foi possível obter drenagem biliar estável e satisfatória por > 6 meses pós-operatórios. Quatro faleceram no período de observação (um por insuficiência hepática, um por colangite, dois por hemorragias digestivas, inclusive os três pacientes em que não foi obtida drenagem biliar satisfatória. Quatro persistem em bom estado geral, anictéricos, um deles com fibrose hepática residual grave e provável indicação futura de transplante hepático. Detectamos problemas graves com relação ao encaminhamento tardio de doentes para tratamento e para disponibilizar transplante hepático quando necessário. CONCLUSÕES: Os resultados da portoenterostomia são compensadores quando é possível obter bom débito biliar no pós-operatório. Em pacientes em que não é obtida boa drenagem biliar o pós-operatório é tormentoso e o óbito é esperado até o segundo ano de vida sem o uso de transplante hepático. É necessário disponibilizar transplante hepático pediátrico em nosso meio e conscientizar a comunidade médica para o encaminhamento precoce de bebês ictéricos além dos primeiros 15 dias de vida para investigação e tratamento em unidades especializadas.BACKGROUND: Indications, results and limitations of portoenterostomy to treat biliary atresia in a referral pediatric hospital in Rio de Janeiro, Brazil, are discussed

  19. Advances of immunologic dysregulation mechanism in biliary atresia%胆道闭锁免疫失调机制的研究进展

    Institute of Scientific and Technical Information of China (English)

    王江; 郑珊

    2014-01-01

    胆道闭锁是一类累及新生儿肝胆系统的危害严重的疾病,该病在东亚民族高发.目前认为可能与遗传易感、病毒感染及免疫失调等有关,其中免疫失调可能是胆道闭锁发病的中心环节.病毒感染而继发胆管上皮自身免疫炎症损伤的学说目前逐渐获得国内外同行的认可,现对胆道闭锁免疫损伤失调机制的研究进展进行综述.%Biliary atresia(BA) is a destructive inflammatory obliterative cholangiopathy of neonates affecting both intrahepatic and extrahepatic bile ducts.BA is more common in east Asia.Genetic susceptibility,viral infections,and immune dysregulation may be related to BA,but immune dysregulation may play a key hole in the pathogenesis of BA.A current view of the pathogenesis of BA is that it may involve both a primary perinatal hepatobiliary viral infection and a secondary generation of an autoimmune-mediated bile duct epithelial injury.The etiology of BA is unknown,but there is evidence for the involvement of immunologic dysregulation mechanisms,which will be discussed in this review.

  20. Evaluation of blood levels of nitric oxide as a means of differentiation between neonatal hepatitis and extrahepatic biliary atresia: A pilot study

    Science.gov (United States)

    Goel, Prabudh; Bhatnagar, Veereshwar; Das, Nibhriti; Kalaivani, Mani

    2015-01-01

    Aim: The differentiation between neonatal hepatitis (NH) and extrahepatic biliary atresia (EHBA) is not always possible despite all the currently available diagnostic modalities. In this study, an attempt has been made to evaluate the role of nitric oxide (NO) levels in the peripheral blood to differentiate between the two conditions, one requiring early surgical intervention (EHBA) and the other amenable to conservative medical management (NH). Patients and Methods: Twenty patients who presented to the pediatric surgical service, over a 2 years period, with features of neonatal cholestasis were enrolled in the study. The diagnostic workup included documentation of history and clinical examination, biochemical liver function tests, ultrasonography, hepatobiliary scintigraphy (HS), and magnetic resonance cholangio-pancreaticography (MRCP). These patients did not show excretion on HS and intrahepatic ducts on MRCP. Hence, they were subjected to mini-laparotomy and operative cholangiography (OC). The EHBA patients were treated with the Kasai's portoenterostomy procedure, and the extrahepatic ducts were flushed with normal saline in NH patients. All patients were evaluated preoperatively for levels of NO in the peripheral blood by the Greiss reaction spectrophotometrically at 540 nm. Normal values were determined from a cohort of controls. The median (range) levels of NO in patients with EHBA and NH were compared, and the statistical significance of the difference was calculated by applying the Wilcox Rank Sum test. A P = 0.05 was considered as significant. Results: Of the 20 patients enrolled in the study, 17 patients were treated for EHBA (Group I) and the remaining 3 patients had patent ducts on OC and were thus diagnosed as NH (Group II). The mean age of the patients in Groups I and II was comparable: 2.79 ± 0.75 and 2.67 ± 0.58 months, respectively (P = 0.866). The median NO levels were significantly elevated in each of the two groups as compared to the controls

  1. Infant Stool Color Card Screening Helps Reduce the Hospitalization Rate and Mortality of Biliary Atresia: A 14-Year Nationwide Cohort Study in Taiwan.

    Science.gov (United States)

    Lee, Min; Chen, Solomon Chih-Cheng; Yang, Hsin-Yi; Huang, Jui-Hua; Yeung, Chun-Yan; Lee, Hung-Chang

    2016-03-01

    Biliary atresia (BA) is a significant liver disease in children. Since 2004, Taiwan has implemented a national screening program that uses an infant stool color card (SCC) for the early detection of BA. The purpose of this study was to examine the outcomes of BA cases before and after the launch of this screening program. The objectives of this study were to evaluate the rates of hospitalization, liver transplantation (LT), and mortality of BA cases before and after the program, and to examine the association between the hospitalization rate and survival outcomes.This was a population-based cohort study. BA cases born during 1997 to 2010 were identified from the Taiwan National Health Insurance Research Database. Sex, birth date, hospitalization date, LT, and death data were collected and analyzed. The hospitalization rate by 2 years of age (Hosp/2yr) was calculated to evaluate its association with the outcomes of LT or death.Among 513 total BA cases, 457 (89%) underwent the Kasai procedure. Of these, the Hosp/2yr was significantly reduced from 6.0 to 6.9/case in the earlier cohort (1997-2004) to 4.9 to 5.3/case in the later cohort (2005-2010). This hospitalization rate reduction was followed by a reduction in mortality from 26.2% to 15.9% after 2006. The Cox proportional hazards model showed a significant increase in the risk for both LT (hazard ratio [HR] = 1.14, 95% confidence interval [CI] = 1.10-1.18) and death (HR = 1.05, 95% CI = 1.01-1.08) for each additional hospitalization. A multivariate logistic regression model found that cases with a Hosp/2yr >6 times had a significantly higher risk for both LT (adjusted odds ratio [aOR] = 4.35, 95% CI = 2.82-6.73) and death (aOR = 1.75, 95% CI = 1.17-2.62).The hospitalization and mortality rates of BA cases in Taiwan were significantly and coincidentally reduced after the launch of the SCC screening program. There was a significant association between the hospitalization rate and final

  2. 活体肝移植治疗儿童胆道闭锁44例%Forty-four living donor liver transplantations for children with biliary atresia

    Institute of Scientific and Technical Information of China (English)

    朱建军; 王鑫; 罗毅; 沈丛欢; 邢天宇; 奚志峰; 夏强; 张建军; 李齐根; 徐宁; 陈小松; 薛峰; 韩龙志; 夏雷

    2011-01-01

    Objective To observe the outcomes of living donor liver transplantation (LDLT) for children with biliary atresia (BA) and to summarize the clinical experiences. Methods Forty-four BA patients (26 boys and 18 girls) underwent LDLT between October 2006 and December 2010. Mean (SD) and median (range) age at operation was (12.1 ± 9.0) months and 9 (6-60) months,respectively. The 44 donors were lineal relatives to the consorted recipients. Their mean (SD) and median (range) age at operation was (32. 7 ± 8. 0) months and 31 (20~54) years, respectively. All donor graft types were the left lateral segments with compatible ABO blood groups. Clinical data,including pre-operative evaluations, surgical technique, postoperative management and outcomes in all donors and recipients were retrospectively analyzed. Results All donors were followed up for (17. 5 ± 13. 3) months. No donor mortality was encountered, with a minimal morbidity and no long-term sequelae. Nine out of 44 recipients died. Three patients died of portal vein thrombosis (PVT), one of hepatic artery thrombosis (HAT), two of biliary complications, one of surgical site infections, one of abdominal bleeding and one of pulmonary infection. The overall 1-year and 2-year cumulative survival rate in recipients was 81. 2% and 76. 1 %, respectively. No re-transplantation was done. Postoperative complications included PVT, HAT, biliary leakage and refluxing cholangitis, pulmonary infections,surgical site infections and acute rejection. Conclusion LDLT has been the effective treatment for pediatric recipients with BA and provides favorable prognosis. To improve prognosis of recipients, the key points are pre-operative evaluations, surgical technique, and postoperative management%目的 观察活体肝移植治疗儿童胆道闭锁的效果,总结其临床经验.方法 2006年10月至2010年12月间共有44例胆道闭锁患儿接受了活体肝移植,其中男性26例,女性18例,年龄(12.1±9.0)个月,中位数为9

  3. Relationship between hepatic CTGF expression and routine blood tests at the time of liver transplantation for biliary atresia: hope or hype for a biomarker of hepatic fibrosis

    Directory of Open Access Journals (Sweden)

    Haafiz A

    2011-04-01

    Full Text Available Allah Haafiz1, Christian Farrington1, Joel Andres1, Saleem Islam21Hepatology and Liver Transplantation, Division of Pediatric Gastroenterology, Hepatology and Nutrition, 2Division of Pediatric Surgery, University of Florida College of Medicine, Gainesville, FL, USABackground: Progressive hepatic fibrosis (HF is a prominent feature of biliary atresia (BA, the most common indication for liver transplantation (LT in children. Despite its importance in BA, HF is not evaluated in routine patient care because the invasiveness of liver biopsy makes histologic monitoring of fibrosis unfeasible. Therefore, the identification of noninvasive markers to assess HF is desirable especially in children.Purpose: The main goal of this pilot project was to establish an investigational framework correlating hepatic expression of fibrogenic markers with routine blood tests in BA.Methods: Using liver explants from patients with BA (n = 26, immune-expression of connective tissue growth factor (CTGF, a key fibrogenic cytokine was determined using horseradish-labeled antibodies. Expression intensities of lobular (L-CTGF and portal (P-CTGF CTGF were determined by using ImageJ software. These CTGF intensities were correlated with blood tests performed at the time of LT. Correlation coefficients were determined for each blood test variable versus mean L-CTGF and P-CTGF expression intensities. A P-value of less than 0.05 was considered statistically significant.Results: All patients had end-stage liver disease and persistent cholestasis at the time of LT. Kendall tau (t rank correlation coefficient for L-CTGF and white blood cell (WBC was inversed (—0.52; P ≤ 0.02. Similar but statistically nonsignificant inverse relationships were noted between L-CTGF and prothrombin time (PT (—0.15; P ≤ 0.4, international normalized ratio (INR (—0.14; P ≤ 0.5, and platelet count (—0.36; P ≤ 0.09. Inversed (t rank correlation coefficients were also evident between P

  4. Anatomy and embryology of the biliary tract.

    Science.gov (United States)

    Keplinger, Kara M; Bloomston, Mark

    2014-04-01

    Working knowledge of extrahepatic biliary anatomy is of paramount importance to the general surgeon. The embryologic development of the extrahepatic biliary tract is discussed in this article as is the highly variable anatomy of the biliary tract and its associated vasculature. The salient conditions related to the embryology and anatomy of the extrahepatic biliary tract, including biliary atresia, choledochal cysts, gallbladder agenesis, sphincter of Oddi dysfunction, and ducts of Luschka, are addressed.

  5. Clinical characteristics surgical treatment and outcome of biliary atresia%胆道闭锁的临床现状与思索

    Institute of Scientific and Technical Information of China (English)

    施诚仁; 施佳; 蔡威; 吴晔明; 陈其民; 王俊; 王捍平; 严志龙; 张弛; 潘伟华

    2010-01-01

    目的 回顾性分析1999年至2008年十年间收治的胆道闭锁患儿临床资料,为今后临床诊断、防治提供有益资料.方法 收集共184例胆道闭锁患儿,其中34例放弃治疗(18.47%),余150例作基本概况,临床表现、诊断、外科处理和转归等分析.男86例,女64例,求医时年龄(56.20±39.44)d,其中55例>90 d(36.67%),诉大便转白时间为(22.33±20.3)d.均疑黄疸消退迟缓>2周,有进行性血胆红素增高;临床诊断方法有B超检查(91.33%),同位素闪烁检查(53.33%),血胆红素、酶测定(100%),影像学检查(58.66%).结果 手术探查时年龄(81.11±31.01)d,行Kasai手术率仅66%(99/150),随访率77.33%(116/150),随访9个月~8.2年,平均(23.77±16.4)个月,死亡56例(48.27%),肝移植6例(5.17%),正常生活30例(25.86%),带门脉高压等合并症生存24例(20.69%).结论 胆道闭锁是新生儿高胆红素血症中常见疾病.①其病因学说多种多样,大便转白在出生后(22.33±20.3)d,此现象以Schreiber提出的观点能较合理解释;②诊断无特异敏感的方法,还是以多种检查综合评估为好.腹部B超无创,值得进一步探索;③本组病死率较高48.27%,与文献报告有差距;④终末期肝移植是唯一有效方法,但本组仅6例,可能与经济、传统观念、供肝者条件不好等有关.%Objective To retrospectively analyze the clinical characteristics,surgical treatment and outcome of biliary atresia(BA)during the last decade Methods From 1999 to 2008,the clinical data of 184 patients with BA were collected.Among them,34 patients gave up further treatment.The basic conditions,clinical manifestations,diagnosis,surgical treatment and outcome of the other 150 patients were retrospectively analyzed.Results The 150 patients with a mean age of 56.02±39.44 days included 86 males and 64 females.Fifty five patients were older than 90 days at the time of diagnose.Common complaints were presented with jaundice longer than 2 weeks,white stools and

  6. 胆道闭锁、胆总管囊肿与巨细胞病毒感染%Biliary Atresia and Congenital Choledochal Cyst and the Infection of Cytomengalovlrus

    Institute of Scientific and Technical Information of China (English)

    王慧贞; 李正; 黎明; 吕绳敏; 刘兰青; 吴佩英; 刘春复

    1989-01-01

    为探讨胆道闭锁及胆总管囊肿与病毒感染的关系,作者进行了研究.结果发现9例胆道闭锁患儿有2例从肝组织中检出巨细胞病毒,5例胆总管囊肿患儿有2侈4从肝组织中,1例从唾液腺中检出巨细胞病毒.阳性率为35.7%.作者认为,胆道闭锁、胆总管囊肿专巨细病毒感染关系密切.%Nine cases of biliary atresia (BA) and 5 cases of congenital choledechal cyst (CCC) have been studied since 1985.Their HBsAg were all neagtive.The virus has been separated from the preoperetive urine,the liver biopsies intraoperatively and salivery gland obtained at autopsy.It was further identified by cell sensitivity test,inderect immunofluorescence test,anticomplement immunofluorescence test and monoclonal antibody indirect immunofluorescence test.The cytomegalovirus (CMV) was found in the liver in 2 cases of 9 BA (22.2%),and 2 cases of 5 CCC,and in one of the 2 CCC are found in urine simultaneously,from the salivery gland in another case after death (60%).The positive rate totals is 35.7%.The results indicate that the infection of cytomegalovirus in the patients with biliary anomalies is commen (35.7%).CMV might come from the mother's uterus.

  7. 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.

  8. Correlation between human cytomegalovirus and CD4+ T cell's cytokines in biliary atresia%人巨细胞病毒感染与胆道闭锁CD4+T细胞因子的相关性研究

    Institute of Scientific and Technical Information of China (English)

    张锐忠; 余家康; 谢丽芳; 欧阳润仙; 夏慧敏

    2011-01-01

    目的 检测胆道闭锁患儿肝脏组织中人巨细胞病毒滴度与11种CD4+T细胞因子表达水平之间的相关性,并探讨其意义.方法 分别采用荧光定量PCR和流式微球技术对29例胆道闭锁患儿肝脏组织中的巨细胞病毒滴度和CD4+T细胞表达的11种细胞因子(IL-12p70、IFN-γ、IL-2、IL-10、IL-8、IL-6、IL4、IL5、IL-1β、TNF-α和TNF-β)进行定量检测,并统计两者之间的相关系数.结果 人巨细胞病毒阳性者15例(51.7%),15例胆道闭锁患儿肝脏组织中人巨细胞病毒滴度与11种细胞因子具有不同程度的相关性,其中与部分细胞因子(IFN-γ、IL-2、IL-10、IL-4、TNF-α及TNF-β)呈强正相关,以IFN-γ的相关性最强(r=0.796),且该病毒的滴度与Th1类细胞因子总量(r=0.914)、促炎因子总量(r=0.913)及11种细胞因子总量(r=0.893)呈强正相关.结论 胆道闭锁可能是由人巨细胞病毒感染所促发,主要由CD4+Th1细胞及其细胞因子所介导的免疫炎症性疾病.%Objective To examine the relationship between human cytomegalovirus and CD4+ T cell's cytokines in biliary atresia. Methods HCMV titres and 11 cytokines (IL-12p70,IFN-γ,IL-2,IL-10, IL-8, IL-6, IL-4, IL- 5, IL- 1β, TNF-α and TNF-β) were assayed with Fluorescent quantitative PCR(FQ-PCR) and flowing microsphere technology respectively. The correlation coefficients were then calculated. Results Fifteen liver specimens(51.7%)were positive for HCMV. The titers of human cytomegalovirus showed different correlations with the 11 cytokines and strong positive correlation with some cytokines( IFN-γ、 IL-2、IL-1 0 、 IL-4 、 TNF-α and TNF-β), IFN-γ is the strongest one( r = 0. 796).Moreover, it shows strong positive correlation with the total of Th1 cytokines(r = 0. 914), pro-inflammatory cytokines(r = 0. 913)and the total of 11 cytokines(r= 0. 893). Conclusions The immune reaction of biliary atreisa may be triggered by the human cytomegalovirus, promoted by the CD4+ Th1

  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. 胆道闭锁患儿肝移植术后的监护与治疗%Postoperative intensive care of biliary atresia patients treated with living donor liver transplantation

    Institute of Scientific and Technical Information of China (English)

    邓玉华; 郭春宝; 张明满; 李英存

    2011-01-01

    目的 回顾分析22例胆道闭锁患儿(23例次,其中1例行再次肝移植)肝移植术后的重症监护管理经验,探讨并发症的发生率以及病原菌与患儿并发症预后之间的联系.方法 统计分析22例平均体重<8.8 kg的婴幼儿在ICU的相关临床资料,包括药物的使用情况(肾上腺素能激动剂、抗高血压药、利尿剂、镇静止痛药)及主要并发症(排异反应11例,外科并发症16例,感染18例)的诊断、评估及治疗,其中抗生素的选用主要根据药敏试验结果决定.结果 最常见的术后并发症包括感染(18例)、消化道出血(3例)、血管并发症(4例).1例死于原发性无功能肝,11例出现排异反应.最常见的病原微生物包括表皮葡萄球菌(7例),不动杆菌属(6例),铜绿假单胞菌(7例).ICU平均住院时间为10 d,机械通气平均时间37.6 h.多巴酚丁胺、前列腺素E1、多巴胺的平均使用时间分别为3.3 d,7.5 d,8.8 d.术后胃肠外营养的平均起始时间为12 h,进食起始时间平均72 h.结论 术后监护是保证婴幼儿肝移植成功的关键之一.%Objective To summarize experience of pediatric intensive care and explore the incidence of complications, the involved pathogens among liver recipients to determine the effective strategies for preventing complications. Methods Between June 2006 and July 2009, 35 children under the age of 14 yr received 35 liver transplantations (LTs) performed at the center. A retrospective review of 22 infants weighing 8. 8 kg or less underwent 23 transplants was conducted. Indication for transplantation was biliary atresia. Central venous pressure and arterial blood pressure were monitored continuously and fluid monitoring was performed every 2 hours in the first postoperative week. Blood loss, ascites, and intraoperative transudate loss were primarily replaced with 5% albumin and crystalloids to maintain a central venous pressure between 4 and 6 cm H2O. Oral food intake was allowed as

  11. High-throughput sequencing of circulating miRNAs in patients with biliary atresia%胆道闭锁循环miRNA的高通量测序分析

    Institute of Scientific and Technical Information of China (English)

    彭小芳; 付捷; 温哲; 庞舒尹; 陈奕豪; 高坎坎; 辜少玲; 杨丽媛; 陈峥嵘

    2016-01-01

    目的 用高通量二代测序方法(next generation sequencing,NGS)分析胆道闭锁(biliary atresia,BA)患儿外周血中循环miRNA的表达特征,为寻找新的疾病相关无创诊断标志物提供依据.方法 分别收集2013年1月至2014年5月到本中心就诊的29例BA(疾病组),10例婴儿肝炎综合征、5例Citrin基因缺陷导致的新生儿肝内胆汁淤积症(NICCD)、5例胆总管囊肿(疾病对照组)和29例健康对照(正常对照组)血浆样本.选取前期收集的疾病组和正常对照组样本各9例用于高通量测序,将两组儿童分别按性别和年龄匹配分为3组,将血浆样本按上述分组进行组内等比例混合,提取血浆混合池中的总RNA,以Illumina HiSeq 2000测序仪分析混合样品中的全部miRNA序列.组间差异比较采用t检验,靶基因预测软件TargetScan,miRanda和PITA对差异miRNA的功能进行预测;并以实时荧光定量聚合酶链反应(reverse transcription quantitative real-time polymerase chain reaction,RT-qPCR)对差异表达显著的miRNA进行验证.结果 本研究成功构建BA患儿循环miRNA表达谱;BA患儿组与正常对照组中有188个循环miRNA表达存在差异(P<0.05),包括87个上调、101个下调,56个为新发现的miRNA,其余皆为数据库中的已知miRNA.表达差异大于2倍的miRNA有146个,77个上调、69个下调.其中,表达量高且为各样本组恒定表达的miRNA有8个,均为已知miRNA,包括上调的has-miR-122-5p、-100-5p、-432-5p,下调的has-miR-140-3p、-10b-5p、-26a-5p、-126-3p、-744-5p;功能预测显示,这8个差异miRNA与肝胆发育、肝纤维化、细胞凋亡等相关;RT-qPCR验证结果表明,BA组血浆hsa-miR-122-5p、-100-5p表达量显著高于正常对照,而hsa-miR-140-3p、-10b-5p、-126-3p显著低于正常对照组(均P<0.05),与测序结果一致.结论 本研究结果全面展示了BA患儿外周血循环中miRNA的表达特征,其中一些差异性的miRNA有可能作为BA无创

  12. Application of Z score in evaluating the growth of biliary atresia patients after Kasai procedure%胆道闭锁Kasai术后自肝生存患儿体格发育Z评分

    Institute of Scientific and Technical Information of China (English)

    王丽; 陈亚军; 张廷冲; 冯欣; 彭春辉; 庞文博; 王增萌; 沈秋龙

    2016-01-01

    Objective To evaluate the physical growth of biliary atresia (BA) patients after Kasai procedure by using Z score and compare the data among different groups.Methods Using a cross section method,96 children with postoperative BA were evaluated for height/length and weight.WHO AnthroPlus 2007 was used for calculating Z score.Analysis of variance or t test were used for comparing Z scores among different groups.Results The prevalence of stunting (height for age Z-score,HAZ<-2),underweight (weight for age Z-score,WAZ<-2) and wasting (weight for height Z-score,WHZ<-2) were 14.58%(14/96),5.21%(5/96) and 3.13%(3/96);overall mean Z scores of HAZ,WAZ,WHZ-0.38 ± 0.17,0.33 ± 0.17 and 0.88 ± 0.17;mean Z scores of HAZ,WAZ,WHZ of <12 months group-0.27 ± 0.12,-0.55 ± 0.37 and-0.30 ± 0.25 respectively.According to age,WHZ of <60 days,60~90 days and >90 days were 1.08 ± 0.27,0.85 ± 0.25 and-0.26 ±0.24 respectively.Conclusions After Kasai operation,BA patients suffer from malnutrition.And the problem of stunting is more serious.Poor nutritional status is more frequent for those aged < 12months.Thus nutritional status of infants is particularly worrisome.As for operative age,>90 days group has more serious nutritional problems so that early Kasai operation helps improve nutritional status.%目的 本研究旨在对胆道闭锁Kasai术后自肝生存患儿的体格发育进行评价,了解胆道闭锁Kasai术后自肝生存患儿营养状况.方法 本研究采用横断面调查方式,于2014年10月至2015年1月对首都医科大学附属北京儿童医院普外科胆道闭锁Kasai术后自肝生存定期门诊复查的96例患儿进行体格测量(身高/身长、体重).采用WHO Anthro Plus 2007软件,计算年龄别身高/身长Z评分(Height for Age Z-score,HAZ)、年龄别体重Z评分(Weight for Age Z-score,WAZ)、身高别体重Z评分(Weight for Height Z-score,WHZ).统计我中心Ksasi术后自肝生存患儿生长迟缓、低体重、消

  13. Jejunum ileal intestinal atresia.

    Directory of Open Access Journals (Sweden)

    Claudio J. Puente Fonseca

    2005-12-01

    Full Text Available The intestinal atresia is one of the most important causes of intestinal obstruction in newborn. They constitute aorund 95% of total intestinal obstructions in this age group. Most of intestinal atresias are jejunoieal atresia. Although it is not frequent their relationship with other congenital anomalies, has been described the association in some cases with defects of intestine rotation, meconium peritonitis, with meconium ileus and rarely with the Hirschsprung diseases. The hereditary character has also been described in certain multiple intestinal atresias. We presented the Good Clinical Practices Guideline for Jejunoileal atresia, approved by consensus in the 1st National Good Clinical Practices Workshop in Pediatric Surgery (Cienfuegos, Cuba, March 7 – 9, 2002.

  14. Biliary ascariasis.

    Science.gov (United States)

    Arcilla, C A; Varilla, A

    1978-01-01

    The presenting clinical features of a series of cases of biliary tract ascariasis are described, in particular the characteristic colicky pain which occurs. The term 'ascaritic biliary pain' is introduced for the symptom diagnostic of live Ascaris adult worms inside the biliary passages. Methods of diagnosis before, and in the course of, operation are discussed and seem adequate. The management of worms discovered in the bile ducts, and those in the proximal small bowel, is described.

  15. 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.

  16. 腹腔镜与开放Kasai手术治疗Ⅲ型胆道闭锁疗效的前瞻性对比研究%Laparoscopic versus open Kasai portoenterostomy in infants with type Ⅲ biliary atresia: a prospective study

    Institute of Scientific and Technical Information of China (English)

    孙旭; 李龙; 叶茂; 刁美; 明安晓; 吴璇昭

    2013-01-01

    Objective To compare the clinical outcomes in laparoscopic versus conventional Kasai portoenterostomy in patients with type Ⅲ biliary atresia.Methods Ninety-five patients with type Ⅲ biliary atresia underwent Kasai operation in the Capital Pediatric Institution between September 2009 and August 2011.They were randomized assigned into 2 groups according to the surgical procedures:laparoscopic Kasai portoenterostomy group (LP group,n =48) and open Kasai portoenterostomy group (OP group,n =47).In LP group,4 patients were converted to open surgery during operation.The rest 44 cases of the LP group and 47 cases of OP group were recruited in this study.The clinical data including intraoperative blood loss,operation time,postoperative hospital stay,start of food intake,liver function improvement,jaundice clearance rate,incidence of cholangitis,postoperative complications and mortality were analyzed.Results There were no significant differences of patients' gender and age at operation between the LP group and the OP groups.Compared with those underwent open surgery,the patients underwent laparoscopic Kasai procedure had a longer operation time (172.32 ± 29 min vs.149.66 ± 27.91 min,P<0.01),less intraoperative blood loss (8.07 ± 3.09 ml vs.17.55 ± 3.59ml,P<0.01),and earlier start of food intake after surgery (2.80 ± 1.36 days vs.3.47 ± 0.65 days,P =0.02).However,no statistical difference of postoperative hospital stay was found between these 2 groups (LP 12.55 ± 3.92 days vs.OP 13.57 ± 3.03 days,P =0.16).The median follow-up period was 16 months of the LP group and 17 months of the OP group.There are no significant differences between these 2 groups in postoperative jaundice clearance rate (LP 43.18 % vs.OP 51.06%,P =0.45),incidence of cholangitis (LP 59.09% vs.OP 59.57%,P =0.96),mortality (6 months after surgery:LP 18.18% vs.OP 14.89%,P =0.67; 1 year after surgery:LP 21.88%vs.OP 27.78%,P =0.58),and liver function improvement after operation

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

    Directory of Open Access Journals (Sweden)

    Vijay C Pujar

    2012-01-01

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

  18. 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.

  19. 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.

  20. 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 ...

  1. 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.

  2. 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.

  3. Unusual biliary scan appearance in a child with a transplanted liver with hepatic arterial thrombosis: a case report.

    Science.gov (United States)

    Porn, U; Howman-Giles, R; Shun, A; Dorney, S; Uren, R

    2000-02-01

    A 5-year-old girl with biliary atresia and a subsequent Kasai procedure is described. She had clinical symptoms suggestive of rejection after a recent orthotopic liver transplant A hepatobiliary scan showed partial hepatic infarction and a biloma in the infarcted area.

  4. A unique case of segmental vasal atresia

    Science.gov (United States)

    Alalayet, Yasen Fayez; Alkasim, F; Shiba, N; Aldhuayan, I; Alhamaidi, S; Alghamdi, G; Aljobair, F; Shoura, J; Alkhlaif, R

    2014-01-01

    We report a case of a 2-year-old boy who presented with an empty left scrotum. Clinical examination revealed a left palpable undescended testis. During orchidopexy, segmental atresia of the vas deferens was found, and microsurgical repair was carried out. Segmental vasal atresia is an extremely rare condition and is infrequently diagnosed, especially in the pediatric age group. This is the first reported case of segmental vasal atresia without an association with cystic fibrosis. PMID:25419520

  5. 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.

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

    Directory of Open Access Journals (Sweden)

    Yogender Singh Kadian

    2014-07-01

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

  7. [Unilateral choanal atresia: a missed diagnosis].

    Science.gov (United States)

    Joshua, Ben-Zion; Gluck, Ofer; Puterman, Mark

    2012-11-01

    Bilateral choanal atresia is a congenital anomaly usually diagnosed at birth. In contrast, unilateral atresia causes variable degrees of nasal stuffiness and discharge that might mask the diagnosis and delay the proper treatment. We present five cases of unilateral atresia in which the correct diagnosis was delayed and erroneous treatments were instituted--two adults who had undergone unnecessary septal and turbinate surgery, two older children who were treated medically and one patient who was treated for epiphora. Insufficient awareness of this entity occurring in adults and older children and other possible causes of diagnostic error are discussed. A unilateral mucoid or watery rhinorrhea and obstruction, that lasts from early childhood without evidence of sinusitis and not responding to any medical treatment, should alert the physician to consider unilateral atresia and to perform endoscopic and computed tomography examinations at an early age. Careful interpretation of the computed tomography scan including the axial planes is required.

  8. Epidemiology of small intestinal atresia in Europe

    DEFF Research Database (Denmark)

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

    2012-01-01

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

  9. [Tricuspid atresia: A therapeutic problem].

    Science.gov (United States)

    Mata, L A; Espino Vela, J; Castro, A; Cárcamo Tercero, J; Martínez Ríos, M A; Pliego, J; Attié, F

    1975-01-01

    1. The authors present 80 cases of tricuspid atresia of the Children's Cardiology Service of the Instituto Nacional de Cardiología de México. They propose their own classification based on the position of the visceral suits and of the great arteries and the possible existance of a persistent truncus arteriosus. 2. The requirements that should be fulfiled to establish an anatomic and hemodynamic diagnosis are determined; and the need to know in detail the anatomy of the right ventricle; of its exit chamber, of the pulmonary valve and of the pulmonary truncus is emphasized; as well as the measures in the auricles and the left ventricle. 3. They insist upon the need, when the pulmonar arterial pressure cannot be measured directly, to introduce a catheter in a pulmonar vein, because the pressures obtained are quite similar to the pressure in the pulmonar arterial territory. 4. The fundamental data of the anatomic and hemodynamic study are discussed and illustrated. They confirm the close relation that exists between the diameter of the exit chamber of the right ventricle and of the valvular ring, the pulmonary truncus and its branches. 5. In all cases of tricuspid atresia with AQRS deviated to the right and downwards in the frontal plane; transposition of the great arteries should be thought of when it deviates to the left and upwards, in patients with crossed great arteries. 6. In the cases operated with sistemic-pulmonary fistula, there was an intrahospital mortality range of 25%, similar to that obtained by most authors. 7. The paliative surgical procedures are analyzed, and those that at present could be considered as "corrective" such as Fontan and Kreutzer's operations. The need, to gather several diagnostic parameters. To establish the surgical indication in the sistemicpulmonary and venus shunts, is emphasized.

  10. Extrahepatic bile duct atresia from the pathologist’s perspective: pathological features and differential diagnosis

    Directory of Open Access Journals (Sweden)

    Peter Van Eyken

    2014-06-01

    Full Text Available Extrahepatic biliary atresia (EHBA refers to stenosis or atresia of the extrahepatic biliary tree. It accounts for 25-30% of cases of neonatal cholestasis. If left untreated, EHBA progresses to biliary cirrhosis and is universally fatal within the first 2 years of life. Early diagnosis is crucial since surgical treatment (Kasai procedure is the only treatment option. Histopathologic examination of liver biopsy specimens is a key element in the diagnostic work-up of infants with suspected EHBA. Pathologic diagnosis aims at excluding non-surgically correctable causes of neonatal cholestasis thereby leading to surgical exploration for confirmation of the diagnosis. All published data indicate that pathologists can diagnose EHBA with high sensitivity, high specificity and reasonable interobserver agreement. The most useful histologic features in the diagnosis of EHBA are portal tract changes including ductular proliferation and bile plugs in ducts and ductules. These lesions are not pathognomonic but can be seen in extrahepatic obstruction of any cause. Total parenteral nutrition (TPN-associated cholestasis and alpha1-antitrypsin (A1AT deficiency cannot be differentiated from EHBA without access to clinical data and may lead to false-positive diagnosis. False-negative interpretation may be caused by early age at diagnosis or by small/indequate specimens. The pathologist also plays a role in the examination of the resected fibrotic segment and of explant specimens. Histopathology can yield prognostic information, being also an indispensable tool in research for the possible pathogenesis of this disease. A well-coordinated, multidisciplinary approach is required in the assessment of suspected cases of EHBA.  Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014 · Cagliari (Italy · October 25th, 2014 · The role of the clinical pathological dialogue in

  11. Microtia and congenital aural atresia.

    Science.gov (United States)

    Genc, Selahattin; Kahraman, Erkan; Ozel, Halil Erdem; Arslan, Ilker Burak; Demir, Ahmet; Selcuk, Adin

    2012-11-01

    The purpose of this study was to show the clinical characteristics of microtia and congenital aural atresia cases in Turkey and to make the classification. For this purpose, records of 28 patients with microtia who were admitted to the ENT Clinic of Eskisehir Military Hospital, Turkey, between 1995 and 2011 and 3 patients admitted to the ENT outpatient clinic of Kocaeli Derince Education and Research Hospital, Turkey, were analyzed retrospectively. Of the total 31 patients with microtia (35 microtic ears), involvement of the right ear of 20 patients (64.5%), the left ear of 7 patients (22.5%), and bilateral involvement in 4 patients (12.9%) were observed. There was a unilateral involvement in 27 patients (87.1%). According to the Marx grading, 2 patients (5.7%) had grade 1 malformation, 3 (8.6%) had grade 2 malformation, 29 (82.9%) had grade 3 malformation, and 1 (2.9%) had grade 4 malformation (anotia). Although the characteristics of microtia vary in different population, the results in Turkey are consistent with those in the literature.

  12. Current knowledge on esophageal atresia

    Institute of Scientific and Technical Information of China (English)

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

    2012-01-01

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

  13. Electrophysiologic studies in tricuspid atresia.

    Science.gov (United States)

    Serratto, M; Pahlajani, D B

    1978-12-01

    Five patients with tricuspid atresia underwent His bundle studies with the recording catheter placed close to the mitral valve ring. Right atrial pacing and measurement of the refractory periods were performed in three. The prolonged intraatrial conduction time found in all patients is thought to be caused by a hypertrophied and dilated right atrium with increased internodal distance due to stretching of internodal pathways. The A-H interval was normal in all; the H-V interval was short in three and normal in two. The pattern of left axis deviation in the group with a short H-V interval is thought to be due to early origin of the posterior branches of the left bundle branch from the bundle of His and early activation of the posteroinferior parts of the left ventricle. The pattern of left axis deviation in the group with a normal H-V interval may be related to the previously reported anomalous course of the left bundle. Atrial pacing produced a normal response. The refractory periods were within normal range, suggesting functional integrity of conduction through the atrioventricular node and bundle branches.

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

    Science.gov (United States)

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

    2016-01-01

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

  15. Wind sock deformity in rectal atresia

    Directory of Open Access Journals (Sweden)

    Hosseini Seyed

    2009-01-01

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

  16. 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....

  17. 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...

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

    Directory of Open Access Journals (Sweden)

    Rohit S Loomba

    2015-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2005-04-01

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

  20. [Postoperative biliary peritonitis].

    Science.gov (United States)

    Kurbonov, K M; Daminova, N M; Mukhiddinov, N D

    2008-01-01

    An analysis of results of surgical treatment of 91 patients with postoperative biliary peritonitis has shown that frequency of postoperative biliary peritonitis depended on the volume and character of operative interventions, as well as on technical errors made at the preoperative period and during operation. The presence of latent hepatic insufficiency made for the development of polyorganic insufficiency, had negative influence on the course of the disease and might be one of the causes of high postoperative lethality (up to 23.1%) in this category of patients.

  1. Biliary ascariasis: a review.

    Science.gov (United States)

    Shah, Omar Javed; Zargar, Showkat Ali; Robbani, Irfan

    2006-08-01

    Ascariasis, a helminthic infection of humans, is the most common parasitic infestation of the gastrointestinal tract. It infects about 25% of the world's population; around 20 thousand deaths occur per year from an adverse clinical course of the disease. This review is focused on biliary ascariasis, examining in some detail the pathogenesis of the disease with special reference to postcholecystectomy ascariasis and related issues. Although an endemic disease of tropical and subtropical countries, increasing population migration facilitated by fast improving communication facilities demands that clinicians everywhere be familiar with the clinical profile and management of biliary ascariasis.

  2. Multiple gastrointestinal atresias in two consecutive siblings.

    Science.gov (United States)

    Gahukamble, D B; Gahukamble, L D

    2002-03-01

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

  3. Liver and Biliary System

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    2010223 The characteristics of peripheral lymphocytic subsets and cytokines in primary biliary and their changes to drug treatment. TANG Min(唐敏),et al. Dept Rheumatol,PUMC Hosp,PUMC & CAMS,Beijing 100032.Chin J Intern Med 2010;49(2):129-133.

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

    Science.gov (United States)

    Miyano, Go; Morita, Keiichi; Kaneshiro, Masakatsu; Miyake, Hiromu; Koyama, Mariko; Nouso, Hiroshi; Yamoto, Masaya; Nakano, Reiji; Tanaka, Yasuhiko; Nishiguchi, Tomizo; Kawamura, Takakazu; Fukumoto, Koji; Urushihara, Naoto

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Go Miyano

    2015-01-01

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

  6. Percutaneous biliary drainage in acute suppurative cholangitis with biliary sepsis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyung Lyul; Cho, June Sik; Kwon, Soon Tae; Lee, Sang Jin; Rhee, Byung Chull [Chungnam National University College of Medicine, Daejeon (Korea, Republic of)

    1993-11-15

    Acute suppurative cholangitis is a severe inflammatory process of the bile duct occurred as result of partial or complete obstruction of the bile duct, and may manifest clinically severe form of disease, rapidly deteriorating to life-threatening condition. We analyzed emergency percutaneous transhepatic biliary drainage in 20 patients of acute suppurative cholangitis with biliary sepsis to evaluate the therapeutic effect and complication of the procedure. The underlying cause were 12 benign disease(stones) and eight malignant tumors and among eight malignant tumors, bile duct stones(n=4) and clonorchiasis(n=1) were combined. Percutaneous transhepatic biliary drainage was performed successfully in 17 of 20 patients resulting in improvement of general condition and failed in three patients. The procedure were preterminated due to the patient's condition in two and biliary-proto fistula was developed in one. After biliary decompression by percutaneous transhepatic biliary drainage, effective and successful elective surgery was performed in nine cases, which were seven biliary stones and two biliary cancer with stones. Our experience suggest that emergency percutaneous transhepatic biliary drainage is an initial and effective treatment of choice for acute suppurative cholangitis with sepsis and a safe alternative for nonsurgical treatment.

  7. Urogenital abnormalities and atresia of the gastrointestinal tract

    OpenAIRE

    2005-01-01

    Introduction. The goal of the study was to investigate the frequency of urogenital congenital abnormalities among atresias of the digestive system and analyze fetal maldevelopment. The study also deals with gastrointestinal and urogenital embryology. Material and methods. This retrospektive study analyzed the clinical status of 55 new-borns admitted to the Pediatric Surgery Clinic in Novi Sad due to atresia of the gastrointestinal tract during 1995-2003. All atresias were classified at primor...

  8. 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.

  9. Biliary ascariasis: an uncommon cause for recurrent biliary colic after biliary sphincterotomy and common bile duct stone removal.

    Science.gov (United States)

    Thandassery, Ragesh Babu; Jha, Ashish Kumar; Goenka, Mahesh Kumar

    2014-04-01

    Endoscopic retrograde cholangiography is the most commonly used technique for removal of common bile duct stones. Biliary sphincterotomy during the procedure facilitates stone retrieval from the common bile duct. However, sphincterotomy ablates the normal biliary sphincter mechanism. This facilitates duodeno-biliary reflex and can result in inward migration of luminal parasite into the biliary system. In areas where ascariasis is endemic there is an increased risk of biliary ascariasis in postbiliary sphincterotomy patients. We report an unusual case where a patient presented with recurrent biliary colic after cholecystectomy and common bile duct stone extraction and was diagnosed to have biliary ascariasis with the help of endoscopic ultrasound examination of the biliary system.

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

    Science.gov (United States)

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

    2015-09-01

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

  11. Biliary ascariasis. A case report.

    Science.gov (United States)

    Sarihan, H; Gürkök, S; Sari, A

    1995-01-01

    Ascaris lumbricoides is a worldwide intestinal infestation that may cause various complications. Biliary ascariasis, however, is a rare condition. We describe a child with biliary ascariasis. The patient's clinical symptoms were pain, vomiting and abdominal tenderness, and she was thought to have acute appendicitis. However, laboratory examination revealed high serum alkaline phosphatase and amylase levels, and ultrasonography and percutaneous cholangiography demonstrated biliary ascariasis. The patient was successfully treated with mebendazole and antispasmolytic drugs.

  12. Is AMH a regulator of follicular atresia?

    OpenAIRE

    Seifer, David B.; Merhi, Zaher

    2014-01-01

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

  13. Future developments in biliary stenting

    Directory of Open Access Journals (Sweden)

    Hair CD

    2013-06-01

    Full Text Available Clark D Hair,1 Divyesh V Sejpal21Department of Medicine, Section of Gastroenterology and Hepatology, Baylor College of Medicine, Houston, TX, USA; 2Department of Medicine, Section of Gastroenterology, Hofstra North Shore-LIJ School of Medicine, North Shore University Hospital, Manhasset, NY, USAAbstract: Biliary stenting has evolved dramatically over the past 30 years. Advancements in stent design have led to prolonged patency and improved efficacy. However, biliary stenting is still affected by occlusion, migration, anatomical difficulties, and the need for repeat procedures. Multiple novel plastic biliary stent designs have recently been introduced with the primary goals of reduced migration and improved ease of placement. Self-expandable bioabsorbable stents are currently being investigated in animal models. Although not US Food and Drug Administration approved for benign disease, fully covered self-expandable metal stents are increasingly being used in a variety of benign biliary conditions. In malignant disease, developments are being made to improve ease of placement and stent patency for both hilar and distal biliary strictures. The purpose of this review is to describe recent developments and future directions of biliary stenting.Keywords: plastic stents, self-expandable metal stents, drug eluting stents, bioabsorbable stents, malignant biliary strictures, benign biliary strictures

  14. Jejunum for bridging long-gap esophageal atresia

    NARCIS (Netherlands)

    Bax, Klaas (N) M. A.

    2009-01-01

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

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

    OpenAIRE

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

    2015-01-01

    Intestinal atresia is generally caused by intrauterine vascular obstructions involving mesenteric vessels. Intrauterine intussusceptions (IUI) are one of these disruptive events. Intestinal intussusceptions affects children commonly between 3 months and 3 years of age, but it rarely affects in intrauterine life. The relationship between intrauterine intussusception and intestinal atresia has been demonstrated by few cases in literature, suggesting intrauterine intussusception as a rare cause ...

  16. 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.)

  17. Neonatal cholestasis mimicking biliary atresia: Could it be urinary tract infection?

    Science.gov (United States)

    Pereira, Noella Maria Delia; Shah, Ira

    2017-01-01

    Cholestasis can occur in newborns due to infections. However, the manifestations of the underlying infections usually dominate the presentation. We present a 2-month-old infant who presented with jaundice and no fever or signs of systemic illness. Liver biopsy was suggestive of cholangitis. He was subsequently detected to have urinary tract infection with Klebsiella pneumoniae. The child was treated with appropriate antibiotics for 2 weeks following which the cholestasis resolved. Thus, neonatal cholestasis due to infections can also occur in the post-neonatal period without clinical manifestations of an underlying infection.

  18. 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.

  19. [Coincidental finding of biliary ascariasis].

    Science.gov (United States)

    Kristensen, Anders Donatsky; Achiam, Michael

    2010-04-19

    We describe a case of biliary ascariasis in a 27-year-old Philippine au-pair with recurrent upper abdominal pain. Hepatobiliary ascariasis is rarely seen in non-endemic areas. The diagnosis is important because severe complications can occur. If migrated into the biliary tree, it is recommended to perform endoscopic extraction combined with antihelmintic therapy. In severe cases, the recommendation is laparoscopic cholecystectomy and peroperative exploration of the common hepatic duct.

  20. Spontaneous biliary peritonitis in children.

    Science.gov (United States)

    Kohli, Supreethi; Singhal, Anu; Arora, Anita; Singhal, Sanjeev

    2013-01-01

    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.

  1. 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.

  2. Follicular atresia in the prepubertal spiny mouse (Acomys cahirinus) ovary.

    Science.gov (United States)

    Hułas-Stasiak, Monika; Gawron, Antoni

    2011-10-01

    This study was designed to determine follicular atresia in the newborn and the prepubertal spiny mouse. We analyzed the processes of follicle loss using classical markers of apoptosis (TUNEL reaction, active caspase-3) and autophagy (Lamp1). Numerous small clear vacuoles and autophagosomes as well as strong Lamp1 staining were observed in dying oocytes of all follicle types, especially of the primordial and primary ones. Active caspase 3 and the TUNEL reaction were detected only in the granulosa cells of large secondary and antral follicles. The expression of apoptosis and autophagy markers was also changing during the prepubertal period. Western blot analysis indicated that at the moment of birth, females undergo an increased rate of follicular atresia mediated by autophagy, while apoptosis is the dominant form of ovarian atresia in consecutive postnatal days. On the basis of these observations, we concluded that apoptosis and autophagy are involved in follicular atresia and these processes are cell and developmental stage-specific.

  3. SMALL BOWEL DIVERTICULOSIS WITH JEJUNAL ATRESIA

    Directory of Open Access Journals (Sweden)

    Srinivas

    2015-07-01

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

  4. Novel Biliary Reconstruction Techniques During Liver Transplantation

    Science.gov (United States)

    Carmody, Ian C.; Romano, John; Bohorquez, Humberto; Bugeaud, Emily; Bruce, David S.; Cohen, Ari J.; Seal, John; Reichman, Trevor W.; Loss, George E.

    2017-01-01

    Background: Biliary complications remain a significant problem following liver transplantation. Several surgical options can be used to deal with a significant size mismatch between the donor and recipient bile ducts during the biliary anastomosis. We compared biliary transposition to recipient biliary ductoplasty in cadaveric liver transplant. Methods: A total of 33 reconstructions were performed from January 1, 2005 to December 31, 2013. In the biliary transposition group (n=23), 5 reconstructions were performed using an internal stent (5 or 8 French pediatric feeding tube), and 18 were performed without. Of the 10 biliary ductoplasties, 2 were performed with a stent. All patients were managed with standard immunosuppression and ursodiol. Follow-up ranged from 2 months to 5 years. Results: No patients in the biliary transposition group required reoperation; 1 patient had an internal stent removed for recurrent unexplained leukocytosis, and 2 patients required endoscopic retrograde cholangiography and stent placement for evidence of stricture. Three anastomotic leaks occurred in the biliary ductoplasty group, and 2 patients in the biliary ductoplasty group required reoperation for biliary complications. Conclusion: Our results indicate that biliary reconstruction can be performed with either biliary transposition or biliary ductoplasty. These techniques are particularly useful when a significant mismatch in diameter exists between the donor and recipient bile ducts.

  5. Endoscopic surgery in a child with unilateral choanal atresia

    Directory of Open Access Journals (Sweden)

    Diego HELLÍN-MESEGUER

    2016-03-01

    Full Text Available Introduction and objective: Choanal atresia is rare, being twice as common in women than in men, and in 50% of cases approximately it is associated with other congenital anomalies. Although there has been some controvesia about the treatment of choice of this malformation, in the last decade, the introduction of endoscopes in sinus surgery has improved the outcome. Case description: We report the case of a child who has left chronic rhinorrhea with persistent nasal obstruction. By endoscopy and CT diagnosis, left choanal atresia is confirmed. Unilateral choanal atresia endoscopic surgery is performed by creating flaps with intranasal mucosa, preventing nasal packing. Discussion: Different techniques have been considered for the treatment of choanal atresia, which include traditional and endoscopic approach. The results of endoscopic surgery in the choanal atresia vary according to different authors. The two most important aspects of these results are the creation of nasal mucosa flaps and avoiding nasal packing. Conclusions: Surgical treatment of unilateral choanal atresia by endoscopic surgery can reduce the risk of restenosis.

  6. 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.

  7. 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

  8. Spontaneous rupture of intrahepatic biliary ducts with biliary peritonitis.

    Science.gov (United States)

    Aydin, Unal; Yazici, Pinar; Coker, Ahmet

    2007-01-01

    Spontaneous rupture of intrahepatic biliary ducts is a rare cause of acute abdomen due to biliary peritonitis. We report a 92-year-old woman with 48-h history of upper abdominal pain, nausea and vomiting and peritoneal signs. CT scan showed free fluid in the abdomen and mild dilatation of the common bile duct. Exploratory laparotomy showed bile in the abdominal cavity with leak-age from a ruptured bile duct radicle in segment 3, as confirmed on intraoperative cholangiography. She underwent cholecystectomy, choledochotomy with removal of gallstones, repair of the perforation with primary suture and placement of a T-tube. She had an uneventful recovery.

  9. Multidetector CT of emergent biliary pathologic conditions.

    Science.gov (United States)

    Patel, Neel B; Oto, Aytekin; Thomas, Stephen

    2013-01-01

    Various biliary pathologic conditions can lead to acute abdominal pain. Specific diagnosis is not always possible clinically because many biliary diseases have overlapping signs and symptoms. Imaging can help narrow the differential diagnosis and lead to a specific diagnosis. Although ultrasonography (US) is the most useful imaging modality for initial evaluation of the biliary system, multidetector computed tomography (CT) is helpful when US findings are equivocal or when biliary disease is suspected. Diagnostic accuracy can be increased by optimizing the CT protocol and using multiplanar reformations to localize biliary obstruction. CT can be used to diagnose and stage acute cholecystitis, including complications such as emphysematous, gangrenous, and hemorrhagic cholecystitis; gallbladder perforation; gallstone pancreatitis; gallstone ileus; and Mirizzi syndrome. CT also can be used to evaluate acute biliary diseases such as biliary stone disease, benign and malignant biliary obstruction, acute cholangitis, pyogenic hepatic abscess, hemobilia, and biliary necrosis and iatrogenic complications such as biliary leaks and malfunctioning biliary drains and stents. Treatment includes radiologic, endoscopic, or surgical intervention. Familiarity with CT imaging appearances of emergent biliary pathologic conditions is important for prompt diagnosis and appropriate clinical referral and treatment.

  10. 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

  11. [Endoscopic management of postoperative biliary fistulas].

    Science.gov (United States)

    Farca, A; Moreno, M; Mundo, F; Rodríguez, G

    1991-01-01

    Biliary fistulas have been managed by surgical correction with no good results. From 1986 to 1990, endoscopic therapy was attempted in 24 patients with postoperative persistent biliary-cutaneous fistulas. Endoscopic retrograde cholangiography demonstrated residual biliary stones in 19 patients (79%). The mean fistula drainage was 540 ml/day, and in 75% the site of the fistula was near the cistic duct stump. Sphincterotomy with or without biliary stent placement resulted in rapid resolution of the fistula in 23 patients (95.8%). In those patients treated with biliary stents the fistula healed spectacularly in 24-72 hrs.

  12. Transnasal endoscopic repair of acquired posterior choanal stenosis and atresia

    Institute of Scientific and Technical Information of China (English)

    WANG Qin-ying; WANG Shen-qing; LIN Shan; CHEN Hai-hong; LU Yu-yu

    2008-01-01

    Background There are conqenital and acquired choanal atresias and many approaches have been used for their repair.We assessed the clinical effect of power instrument.endoscopic repair of acquired choanaI stenosis and atresia.Methods Nineteen patients,aged from 32 to 61 years,with acquired choanal stenosis and atresia (from trauma in 5 cases and from radiotherapy after nasopharyngeal carcinoma in 14:6 bilateral and 13 unilateral cases),underwent transnasal endoscopic repair of choanal stenosis and atresia.No patient had stenting.Antibiotic and local glucocorticoid were administered postoperatively.Results Eiqhteen patients remained free of symptoms for 12-40 months after the surgery,and the diameter of the neochoana was more than 1 cm after the procedure.One patient required revision surgery and recovered completely with no restenosis at 12 months after the second surgery.There were no postoperative complications.Histology of the resected tissue revealed respiratory epithelial-lined stromal tissue with chronic inflammation,edema and fibrosis,but no tumor cells.Conclusions Transnasal endoscopic approach is a useful procedure for the repair of acquired choanal stenosis and atresia:it is highly successful,safe and effective with swift recovery and short time of hospitalizalion.It is very important in postoperative care to remove any granulation or polyps at the site of the neochoana at that time.

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

    Institute of Scientific and Technical Information of China (English)

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

    2012-01-01

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

  14. 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

    2002-01-01

    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 fro

  15. New development of biliary surgery in China

    Institute of Scientific and Technical Information of China (English)

    Zhi Qiang Huang

    2000-01-01

    @@CHARACTERISTICS OF BILIARY CALCULOUS DISEASES IN CHINA: THE CHANGING SCOPE Diseases of the biliary tract in China is complicated with the prevalence of primary infection of the bile duct system. In the middle of the 20th century, biliary infection, biliary parasitic infestation, and biliary stones made up the three chief components of biliary diseases in China. As to the calculous diseases of the biliary tract, the relative incidence of primary bile duct stones accounted for 50% of the total cases. Therefore, calculous disease accounted for 60.1% among 228 surgical cases in the Chongqing Southwest Hospital, and 60 of the 80 common bile duct stones were primary bile duct origin ( including primary intrahepatic duct stones)[1,2].

  16. Influence of the Biliary System on Biliary Bacteria Revealed by Bacterial Communities of the Human Biliary and Upper Digestive Tracts.

    Science.gov (United States)

    Ye, Fuqiang; Shen, Hongzhang; Li, Zhen; Meng, Fei; Li, Lei; Yang, Jianfeng; Chen, Ying; Bo, Xiaochen; Zhang, Xiaofeng; Ni, Ming

    2016-01-01

    Biliary bacteria have been implicated in gallstone pathogenesis, though a clear understanding of their composition and source is lacking. Moreover, the effects of the biliary environment, which is known to be generally hostile to most bacteria, on biliary bacteria are unclear. Here, we investigated the bacterial communities of the biliary tract, duodenum, stomach, and oral cavity from six gallstone patients by using 16S rRNA amplicon sequencing. We found that all observed biliary bacteria were detectable in the upper digestive tract. The biliary microbiota had a comparatively higher similarity with the duodenal microbiota, versus those of the other regions, but with a reduced diversity. Although the majority of identified bacteria were greatly diminished in bile samples, three Enterobacteriaceae genera (Escherichia, Klebsiella, and an unclassified genus) and Pyramidobacter were abundant in bile. Predictive functional analysis indicated enhanced abilities of environmental information processing and cell motility of biliary bacteria. Our study provides evidence for the potential source of biliary bacteria, and illustrates the influence of the biliary system on biliary bacterial communities.

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

    Science.gov (United States)

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

    2015-10-01

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

  18. 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.)

  19. Retrospective Study of a Series of Choanal Atresia Patients

    Science.gov (United States)

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

    2013-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Fathima Niloofar

    2015-01-01

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

  1. Radiological interventions in malignant biliary obstruction

    Institute of Scientific and Technical Information of China (English)

    Kumble Seetharama Madhusudhan; Shivanand Gamanagatti; Deep Narayan Srivastava; Arun Kumar Gupta

    2016-01-01

    Malignant biliary obstruction is commonly caused by gall bladder carcinoma, cholangiocarcinoma and metastatic nodes. Percutaneous interventions play an important role in managing these patients. Biliary drainage, which forms the major bulk of radiological interventions, can be pal iative in inoperable patients or pre-operative to improve liver function prior to surgery. Other interventions include cholecystostomy and radiofrequency ablation. We present here the indications, contraindications, technique and complications of the radiological interventions performed in patients with malignant biliary obstruction.

  2. Westernblottinginthediagnosisof duodenal-biliary and pancreaticobiliary relfuxesinbiliarydiseases

    Institute of Scientific and Technical Information of China (English)

    Guo-Zhe Xian; Shuo-Dong Wu; Chun-Chih Chen; Yang Su

    2009-01-01

    BACKGROUND: Currently adopted diagnostic methods for duodenal-biliary and pancreaticobiliary relfuxes carry many lfaws, so the incidence of the two relfuxes demands further larger sample size studies. This study aimed to evaluate Western blotting for the diagnosis of relfuxes in biliary diseases. METHODS: An oral radionuclide 99mTc-DTPA test (radio-nuclide, RN) was conducted for the observation of duodenal-biliary relfux prior to measuring bile radioactivity and Western blotting for detecting bile enterokinase (EK). Pancreaticobiliary relfux was assessed by biochemical and Western blotting tests for biliary amylase activity and trypsin-1, respectively. In accordance with bile sample origin, our samples were classiifed into ductal bile and gall bile groups;based on each individual biliary disease, we further classiifed the ductal bile group into ifve sub-groups, and the gall bile group into four sub-groups. Western blotting was conducted to assess the two relfuxes in biliary diseases. RESULTS: Consistencies were noted between EK and RN tests when diagnosing duodenal-biliary relfux (P0.05); in the common bile duct cyst group, the EK positive rate was signiifcantly lower than the trypsin-1 positive rate (P CONCLUSIONS: Western blotting can accurately relfect duodenal-biliary and pancreaticobiliary relfuxes. EK has greater sensitivity than RN for duodenal-biliary relfux. The majority of biliary amylase and lipase comes from the pancreas in all biliary diseases;pancreaticobiliary relfux is the predominant source in the common bile duct cyst group and duodenal-biliary relfux is responsible for the ductal pigment stone group.

  3. Fatigue in primary biliary cirrhosis

    OpenAIRE

    Cauch-Dudek, K; Abbey, S; Stewart, D; Heathcote, E

    1998-01-01

    Background—Fatigue is a frequent and debilitating symptom in patients with primary biliary cirrhosis (PBC). 
Aims—To study fatigue in relation to sleep, depression, and liver disease severity. 
Methods—Patients with PBC completed validated self report questionnaires measuring fatigue, sleep quality, depression, and functional capacity. Verbally reported fatigue and observer rated measure of depression and ursodeoxycholic acid (UDCA) use were recorded. Liver biochemistry and ...

  4. EUS-Guided Biliary Drainage

    Directory of Open Access Journals (Sweden)

    Marc Giovannini

    2012-01-01

    Full Text Available The echoendoscopic biliary drainage is an option to treat obstructive jaundices when ERCP drainage fails. These procedures compose alternative methods to the side of surgery and percutaneous transhepatic biliary drainage, and it was only possible by the continuous development and improvement of echoendoscopes and accessories. The development of linear setorial array echoendoscopes in early 1990 brought a new approach to diagnostic and therapeutic dimenion on echoendoscopy capabilities, opening the possibility to perform punction over direct ultrasonographic view. Despite of the high success rate and low morbidity of biliary drainage obtained by ERCP, difficulty could be found at the presence of stent tumor ingrown, tumor gut compression, periampulary diverticula, and anatomic variation. The echoendoscopic technique starts performing punction and contrast of the left biliary tree. When performed from gastric wall, the access is made through hepatic segment III. From duodenum, direct common bile duct punction. Dilatation is required before stent introduction, and a plastic or metallic stent is introduced. This phrase should be replaced by: diathermic dilatation of the puncturing tract is required using a 6F cystostome. The technical success of hepaticogastrostomy is near 98%, and complications are present in 36%: pneumoperitoneum, choleperitoneum, infection, and stent disfunction. To prevent bile leakage, we have used the 2 stent techniques, the first stent introduced was a long uncovered metallic stent (8 or 10 cm, and inside this first stent a second fully covered stent of 6 cm was delivered to bridge the bile duct and the stomach. Choledochoduodenostomy overall success rate is 92% and described complications include, in frequency order, pneumoperitoneum and focal bile peritonitis, present in 19%. By the last 10 years, the technique was especially performed in reference centers, by ERCP experienced groups, and this seems to be a general

  5. Migration of a biliary stent causing duodenal perforation and biliary peritonitis.

    Science.gov (United States)

    Issa, Hussain; Nahawi, Mamdouh; Bseiso, Bahaa; Al-Salem, Ahmed

    2013-10-16

    Migration of endoscopically placed biliary stents is a well-recognized complication of endoscopic retrograde cholangiopancreatography. Less than 1% of migrated stents however cause intestinal perforation. We present a case of a migrated biliary stent that resulted in duodenal perforation and biliary peritonitis.

  6. Migration of a biliary stent causing duodenal perforation and biliary peritonitis

    OpenAIRE

    Issa, Hussain; Nahawi, Mamdouh; Bseiso, Bahaa; Al-Salem, Ahmed

    2013-01-01

    Migration of endoscopically placed biliary stents is a well-recognized complication of endoscopic retrograde cholangiopancreatography. Less than 1% of migrated stents however cause intestinal perforation. We present a case of a migrated biliary stent that resulted in duodenal perforation and biliary peritonitis.

  7. 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

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

    Directory of Open Access Journals (Sweden)

    Shraddha Verma

    2013-03-01

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

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

    Directory of Open Access Journals (Sweden)

    Katragadda Laxmi Narsimha Rao

    2013-04-01

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

  10. 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.

  11. Spontaneous oesophageal rupture with duodenal atresia in a newborn infant.

    OpenAIRE

    1981-01-01

    A newborn infant with spontaneous oesophageal rupture associated with duodenal atresia is described. The diagnosis was established by the pattern of hydropneumothorax on the chest x-ray film and by the aspiration of gastric juice by thoracocentesis. Necropsy showed a tear, which extended from the oesophagogastric junction to the upper portion of the stomach.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2009-09-15

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2013-11-15

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

  14. Oesophageal atresia with tracheoesophageal fistula and anal atresia in a patient with a de novo microduplication in 17q12

    NARCIS (Netherlands)

    Smigiel, R.; Marcelis, C.L.M.; Patkowski, D.; Leeuw, N. de; Bednarczyk, D.; Barg, E.; Mascianica, K.; Maria Sasiadek, M.; Brunner, H.G.

    2014-01-01

    Oesophageal atresia (OA) and tracheoesophageal fistula (TOF) are foregut malformations with a heterogeneous etiology. OA/TOF may occur as an isolated anomaly or as part of a syndrome. Chromosomal anomalies have been reported in 6-10% of OA/TOF. Several genes have been implicated in cases of syndromi

  15. Ursodeoxycholic acid for primary biliary cirrhosis

    DEFF Research Database (Denmark)

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

    2012-01-01

    Ursodeoxycholic acid is administered to patients with primary biliary cirrhosis, a chronic progressive inflammatory autoimmune-mediated liver disease with unknown aetiology. Despite its controversial effects, the U.S. Food and Drug Administration has approved its usage for primary biliary cirrhosis....

  16. Biliary ascariasis: radiological clue to diagnosis

    OpenAIRE

    2015-01-01

    Ascariasis is caused by Ascaris lumbricoides. It is the most common helminthic infection seen worldwide. Ascariasis is an endemic disease in our country. This is due to the prevailing poor sanitary conditions and low level of education. Biliary ascariasis is an uncommon cause of obstructive jaundice. We report a case of biliary ascariasis in a young labourer who presented with acute abdominal pain.

  17. Biliary Dyskinesia in Children: A Systematic Review.

    Science.gov (United States)

    Santucci, Neha R; Hyman, Paul E; Harmon, Carroll M; Schiavo, Julie H; Hussain, Sunny Z

    2017-02-01

    Cholecystectomy rates for biliary dyskinesia in children are rising in the United States, but not in other countries. Biliary dyskinesia is a validated functional gallbladder disorder in adults, requiring biliary colic in the diagnosis. In contrast, most studies in children require upper abdominal pain, absent gallstones on ultrasound, and an abnormal gallbladder ejection fraction (GBEF) on cholecystokinin-stimulated cholescintigraphy for diagnosis. We aimed to systematically review existing literature in biliary dyskinesia in children, determine the validity and reliability of diagnostic criteria, GBEF, and to assess outcomes following cholecystectomy. We performed a systematic review following the PRISMA checklist and searched 7 databases including PubMed, Scopus, Embase, Ovid, MEDLINE, ProQuest, Web of Science, and the Cochrane library. Bibliographies of articles were screened for additional studies. Our search terms yielded 916 articles of which 28 were included. Three articles were manually added from searched references. We reviewed 31 peer-reviewed publications, all retrospective chart reviews. There was heterogeneity in diagnostic criteria and GBEF values. Outcomes after laparoscopic cholecystectomy varied from 34% to 100% success, and there was no consensus concerning factors influencing outcomes. The observational, retrospective study designs that comprised our review limited interpretation of safety and efficacy of the investigations and treatment in biliary dyskinesia in children. Symptoms of biliary dyskinesia overlapped with functional dyspepsia. There is a need for consensus on symptoms defining biliary dyskinesia, validation of testing required for diagnosis of biliary dyskinesia, and randomized controlled trials comparing medical versus surgical management in children with upper abdominal pain.

  18. Biliary acute pancreatitis: a review

    Institute of Scientific and Technical Information of China (English)

    Osvaldo M. Tiscomia; Susana Hamamura; Enriqueta S. de Lehmann; Graciela Otero; Hipólito Waisman; Patricia Tiscornia-Wasserman; Simmy Bank

    2000-01-01

    @@INTRODUCTION It is axiomatic that the most effective and soundly based plan of treatment of any disorder is one aimed at the mechanism or mechanisms responsible for its development[1]. This basic notion, coupled with recent reports[2- 11] in which, surprisingly there is a total lack of reference to the probable involvement of autonomic-arc-reflexes in the physiopathogenesis of biliary acute pancreatitis have prompted this presentation. Undoubtedly, this disease entity has numerous causes, an obscure physiopathology, few effective remedies, and, often, an unpredictable outcome. At the turn of the century, Opie[12,13] brought to light the association between gallstone migration and acute pancreatitis.

  19. Association of Duodenal Atresia, Malrotation, and Atrial Septal Defect in a Down-Syndrome Patient

    Directory of Open Access Journals (Sweden)

    R Angotti

    2016-04-01

    Full Text Available Duodenal atresia is the frequent cause of neonatal intestinal obstruction. The association between duodenal atresia, intestinal malrotation, cardiac anomalies and Down syndrome is infrequently reported. We present a prenatally suspected case of duodenal atresia which was associated with malrotation and atrial septal defect in a patient of Down syndrome. Duodenotomy and resection of web was performed in addition to Ladd’s procedure. Postoperative course remained uneventful.

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

    Science.gov (United States)

    Kumar, Manish; Thomas, Niranjan

    2016-04-01

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

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

    Directory of Open Access Journals (Sweden)

    Marina Gomes Pedreira

    2010-06-01

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

  2. Congenital esophageal atresia with tracheo-esophageal fistula

    Energy Technology Data Exchange (ETDEWEB)

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

    1970-10-15

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

  3. A newborn with duodenal atresia and a gastric perforation

    Directory of Open Access Journals (Sweden)

    Akcora Bulent

    2010-01-01

    Full Text Available 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. Twelve hours later, significant abdominal distention occurred promptly. At laparotomy, GP and preampullary duodenal atresia were detected. Gastrorrhaphy and duodenoduodenostomy were performed in the same operation. The patient was discharged on the 15th postoperative day. This complicated disease can be treated by early diagnosis and surgical intervention. We choose one-stage operation because of the clean peritoneal cavity. However, generalized peritonitis may require two-stage operation in delayed cases.

  4. Unilateral pulmonary vein atresia: A rare case of hemoptysis

    Directory of Open Access Journals (Sweden)

    Cindy S. DeMastes-Crabtree

    2015-01-01

    Full Text Available We present a rare case of hemoptysis secondary to isolated unilateral pulmonary vein atresia. Isolated pulmonary vein atresia is a rare condition in which patients typically acquire a diagnosis in infancy and early childhood [Mataciunas et al.; Pourmoghadam et al.]. Our patient presented during puberty with several previous episodes of hemoptysis prior to her admission and diagnosis. The initial diagnosis was suspected in our patient from chest computerized tomography (CT, and confirmed with cardiac catheterization and pulmonary angiography. Treatment aim is to preserve lung function and minimize irreversible pulmonary remodeling [Pourmoghadam et al.; Harrison et al.]. Conservative monitoring can be considered with milder or asymptomatic cases, while others may require preoperative collateral artery banding, surgical anastomosis between the pulmonary vein (PV & left atrium (LA and even pneumonectomy [Pourmoghadam et al.].

  5. 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

  6. Imaging findings of biliary hamartomas

    Institute of Scientific and Technical Information of China (English)

    Rong-Qin Zheng; Bo Zhang; Masatoshi Kudo; Hirokazu Onda; Tatsuo Inoue

    2005-01-01

    AIM: To evaluate the imaging findings of biliary hamartomas (von Meyenburg complexes, VMCs) and discuss the differential diagnosis with other related diseases.METHODS: Imaging findings of biliary hamartomas on ultrasonography (US), computed tomography (CT), magnetic resonance imaging (MRI), MR cholangiopancreatography (MRCP)and hepatobiliary scintigraphy were retrospectively analyzed in six patients.RESULTS: On ultrasound images, five of the six cases showed multiple small hyper- and hypo-echoic lesions with comet-tail echoes, especially when magnified by US with the usage of zoom function. In all the six cases,multiple tiny hypodense lesions less than 10 mm in diameter were revealed as scattered throughout the liver with no enhancement on CT. These tiny lesions were demonstrated to be hyper- and hypo-intensity on T2- and TI-weighed images, respectively, in three patients who underwent MRI examinations. MRCP was performed in two patients, and clearly showed multiple tiny irregular- and round-shaped hyper-intensity lesions.MRCP and hepatobiliary scintigraphy showed normal appearances of intra- and extra-hepatic bile ducts in two and one patients, respectively.CONCLUSION: Imaging modalities are useful in the diagnosis and differential diagnosis of VMCs. A correct diagnosis might be obtained when typical imaging findings are present even without a histological confirmation.

  7. 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.

  8. 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.

  9. Heterogeneity of the intrahepatic biliary epithelium

    Institute of Scientific and Technical Information of China (English)

    Shannon Glaser; Heather Francis; Sharon DeMorrow; Gene LeSage; Giammarco Fava; Marco Marzioni; Julie Venter; Gianfranco Alpini

    2006-01-01

    The objectives of this review are to outline the recent findings related to the morphological heterogeneity of the biliary epithelium and the heterogeneous pathophysiological responses of different sized bile ducts to liver gastrointestinal hormones and peptides and liver injury/toxins with changes in apoptotic, proliferative and secretory activities. The knowledge of biliary function is rapidly increasing because of the recognition that biliary epithelial cells (cholangiocytes) are the targets of human cholangiopathies, which are characterized by proliferation/damage of bile ducts within a small range of sizes. The unique anatomy, morphology, innervation and vascularization of the biliary epithelium are consistent with function of cholangiocytes within different regions of the biliary tree. The in vivo models [e.g., bile duct ligation (BDL), partial hepatectomy, feeding of bile acids,carbon tetrachloride (CCl4) or α-naphthylisothiocyanate (ANIT)] and the in vivo experimental tools [e.g., freshly isolated small and large cholangiocytes or intrahepatic bile duct units (IBDU) and primary cultures of small and large murine cholangiocytes] have allowed us to demonstrate the morphological and functional heterogeneity of the intrahepatic biliary epithelium.These models demonstrated the differential secretory activities and the heterogeneous apoptotic and proliferative responses of different sized ducts. Similar to animal models of cholangiocyte proliferation/injury restricted to specific sized ducts, in human liver diseases bile duct damage predominates specific sized bile ducts.Future studies related to the functional heterogeneity of the intrahepatic biliary epithelium may disclose new pathophysiological treatments for patients with cholangiopathies.

  10. 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...

  11. Endoscopic palliation of malignant biliary stricture

    Institute of Scientific and Technical Information of China (English)

    Sanjay M Salgado; Monica Gaidhane; Michel Kahaleh

    2016-01-01

    Malignant biliary strictures often present late after the window for curative resection has elapsed. In such patients, the goal of therapy is typically focused on palliation. While historically, palliative measures were performed surgically, the advent of endoscopic intervention offers minimally invasive options to provide relief of symptoms, improve quality of life, and in some cases, increase survival of these patients. Some of these therapies, such as endoscopic biliary decompression, have become mainstays of treatment for decades, whereas newer modalities, including radiofrequency ablation, and photodynamic therapy offer additional options for patients with incurable biliary malignancies.

  12. 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.

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

    Directory of Open Access Journals (Sweden)

    Chadha Rajiv

    2006-01-01

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

  14. UPDATE ON PRIMARY BILIARY CIRRHOSIS

    Science.gov (United States)

    Invernizzi, Pietro; Selmi, Carlo; Gershwin, M. Eric

    2010-01-01

    Primary biliary cirrhosis (PBC) is an autoimmune chronic liver disease characterized by progressive bile duct destruction eventually leading to cirrhosis, liver failure, and death. The autoimmune pathogenesis is supported by a plethora of experimental and clinical data, such as the presence of autoreactive T cells and serum autoantibodies. The etiology remains unknown, although evidence suggests a role for both genetic susceptibility and environmental factors that remain to be determined. In fact, a number of chemicals and infectious agents have been proposed to induce the disease in predisposed individuals. The recent availability of several murine models will significantly help in understanding pathphysiology mechanisms. In this review, we critically summarize the most recent data on the etiopathogenesis of PBC, discuss the latest theories and developments, and suggest directions for future research. PMID:20359968

  15. 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.

  16. Etiopathogenesis of primary biliary cirrhosis

    Institute of Scientific and Technical Information of China (English)

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

    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 preexisting unstable immune tolerance of genetic origin allowing,after a long latency,the emergence of clinical disease.Initiating mimetopes 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.

  17. 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...

  18. 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

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

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

    NARCIS (Netherlands)

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

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Bilal Mirza

    2012-04-01

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

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

    DEFF Research Database (Denmark)

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

    2008-01-01

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

  2. Computed tomography and obstructive biliary disease.

    Science.gov (United States)

    Havrilla, T R; Haaga, J R; Alfidi, R J; Reich, N E

    1977-05-01

    Forty-four patients with confirmed biliary diseases were studied to determine the value of computed tomography (CT) in the diagnosis of biliary pathology. The results indicate that CT is useful and highly accurate in differentiating between obstructive (surgical) and nonobstructive (medical) disease entities. Of the cases with proved obstruction, 88% were correctly identified. All of the nonobstructive cases had positive CT correlation. In addition, the underlying cause of the occlusion was determined in the majority of cases.

  3. 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

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

    NARCIS (Netherlands)

    van Hasselt, Peter M.; de Koning, Tom J.; Kvist, Nina; de Vries, Elsemieke; Lundin, Christina Rydahl; Berger, Ruud; Kimpen, Jan L. L.; Houwen, Roderick H. J.; Jorgensen, Marianne Horby; Verkade, Henkjan J.

    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 propylactic regimens in i

  5. 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.)

  6. Effect of biliary obstruction and internal biliary drainage on hepatic cytochrome P450 isozymes in rats

    Institute of Scientific and Technical Information of China (English)

    Shintaro Fukushima; Hiroyasu Okuno; Nobuyuki Shibatani; Yoshitsugu Nakahashi; Toshihito Seki; Kazuichi Okazaki

    2008-01-01

    AIM: To investigate the total cytochrome P450 (CYP)content, microsomal mixed-function oxidase (MFO)activity, and expression of mRNAs for various CYP isozymes in a simple rat model of reversible obstructive jaundice.METHODS: Obstructive jaundice was created in male rats by causing bile duct obstruction with polyester tape.In another group of rats, bile duct obstruction was followed by internal biliary drainage after releasing the tape.The expression of various CYP isozyme mRNAs was semi-quantitatively assessed by competitive RTPCR.RESULTS: The total CYP content and microsomal MFO activity showed a significant decrease after biliary obstruction, but returned to respective control levels after biliary drainage.A marked reduction in the expression of CYPIA2, 2B1/2, 2Cll, 2E1, 3A1, and 3A2 mRNA was detected during biliary obstruction,while expression increased significantly toward the control level after biliary drainage.Although expression of CYP4A1 mRNA showed no reduction during biliary obstruction, it still increased significantly after biliary drainage.CONCLUSION: These results suggest that not only obstructive jaundice, but also the subsequent internal biliary drainage may affect regulatory medications of the synthesis of individual CYP isozymes differently.

  7. Biliary duct obstruction treatment with aid of percutaneous transhepatic biliary drainage

    Directory of Open Access Journals (Sweden)

    Daniel Knap

    2016-06-01

    Conclusions: PTBD is an effective method of biliary tract decompression and it is an important alternative to endoscopic drainage. This method is indicated in patients with neoplastic obstruction of biliary tract with low expected survival rate and thus is a palliative procedure.

  8. [Amniotic band sequence and bilateral choanal atresia: a case report].

    Science.gov (United States)

    Del Toro-Valero, Azucena; Estrada-De la Fuente, Alejandro; Velázquez Santana, Héctor; Glicerio González, Jorge; Navarro Meza, María Cristina; Ortega-Hinojosa, Lilia; López-Cardona, María Guadalupe

    2011-08-01

    Amniotic band sequence (ABS) is a group malformation that mainly affects limbs; clinically, constriction rings and lymphedema of the fingers, arms and legs, acrosyndactyly and pseudosyndactyly are observed; also there is congenital amputation of limbs due to distal swelling. Less frequently, craniofacial and trunk involvement are reported in some patients. Etiology is still unknown and most cases are isolated. In this report we present the case of a 45-day-old male with diagnosis of SBA and bilateral choanal atresia as attached finding, and review possible causes of SBA and associated alterations.

  9. 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.

  10. Timing of cholecystectomy after mild biliary pancreatitis: a systematic review.

    NARCIS (Netherlands)

    Baal, M.C.P.M. van; Besselink, M.G.; Bakker, O.J.; Santvoort, H.C. van; Schaapherder, A.F.; Nieuwenhuijs, V.B.; Gooszen, H.G.; Ramshorst, B. van; Boerma, D.

    2012-01-01

    OBJECTIVES: To determine the risk of recurrent biliary events in the period after mild biliary pancreatitis but before interval cholecystectomy and to determine the safety of cholecystectomy during the index admission. BACKGROUND: Although current guidelines recommend performing cholecystectomy earl

  11. 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 originat

  12. Biliary Tract Disorders, Gallbladder Disorders, and Gallstone Pancreatitis

    Science.gov (United States)

    ... of biliary disorders? What is the biliary tract? Enfermedades de la Vesicula y de la Via Biliar, ... Biliar - Ricardo Morgenstern, MD and Ruben Acosta, MD Enfermedades de la Vesicula y de la Via Biliar, ...

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

    Science.gov (United States)

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

    2014-07-01

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

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

    Directory of Open Access Journals (Sweden)

    Madonia Maurizio

    2011-08-01

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

  15. Delayed Recognition of Type 1 Sigmoid-Colon Atresia: The Perforated Web Variety

    Directory of Open Access Journals (Sweden)

    Ghulam Mustafa

    2010-08-01

    Full Text Available Colonic atresias are the rare malformations of the colon and constitute about 1.7 to 15% of all gastrointestinal (GI atresias. A 6-month old infant presented with recurrent episodes of sub-acute intestinal obstruction since birth. During the index admission, patient had clinical signs of complete intestinal obstruction. The patient was operated and type I sigmoid-colon atresia found which on further exploration tuned out to be of perforated mucosal web variety. The resection of the involved part of colon and a primary end to oblique colo-colic anastomosis was performed.

  16. Contemporary Management of Acute Biliary Pancreatitis

    Directory of Open Access Journals (Sweden)

    Orhan Ozkan

    2014-03-01

    Full Text Available Acute biliary pancreatitis is one of the major causes of acute pancreatitis.Gallstones, biliary sludge and microlithiasis, especially in pancreatitis without detectable reason, can be the cause of acute pancreatitis. Acute biliary pancreatitis has many controversions in the literature, and its classification and guidelines are being updated very frequently. Atlanta classifications which determine the definitions and guidelines about acute pancreatitis were renewed and published in 2013. It has various clinical aspects, ranging from a mild form which is easily treated, to a severe form that causes complications leading to mortality. The pathogenesis of this disease has not been fully elucidated and several theories have been suggested. New scoring systems and laboratory methods such as proteomics have been suggested for both diagnosis and to predict disease severity, and research on these topics is still in progress. Novel therapeutic approaches with technological developments such as ERCP, ES, MRCP, and EUS are also suggested.

  17. Advanced endoscopic imaging of indeterminate biliary strictures

    Institute of Scientific and Technical Information of China (English)

    James; H; Tabibian; Kavel; H; Visrodia; Michael; J; Levy; Christopher; J; Gostout

    2015-01-01

    Endoscopic evaluation of indeterminate biliary stric-tures(IDBSs) has evolved considerably since the development of flexible fiberoptic endoscopes over 50 years ago. Endoscopic retrograde cholangiography pancreatography(ERCP) was introduced nearly a decade later and has since become the mainstay of therapy for relieving obstruction of the biliary tract. However, longstanding methods of ERCP-guided tissue acquisition(i.e., biliary brushings for cytology and intraductal forceps biopsy for histology) have demon-strated disappointing performance characteristics in distinguishing malignant from benign etiologies of IDBSs. The limitations of these methods have thus helped drive the search for novel techniques to enhance the evaluation of IDBSs and thereby improve diagnosis and clinical care. These modalities include, but are not limited to, endoscopic ultrasound, intraductal ultrasound, cholangioscopy, confocal endomicroscopy, and optical coherence tomography. In this review, we discuss established and emerging options in the evaluation of IDBSs.

  18. Current endoscopic approach to indeterminate biliary strictures

    Institute of Scientific and Technical Information of China (English)

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

    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.

  19. Obstructive jaundice induced by biliary ascariasis.

    Science.gov (United States)

    Keating, Aine; Quigley, James Aidan; Genterola, Al Frederick

    2012-12-13

    Ascaris lumbricoides is one of the most prevalent parasitic infections, especially in developing countries. Its presence can lead to a multitude of presentations, one of the rarer ones being obstructive jaundice due to migration of the worm in to the biliary tree. We describe a case of a man who presented as an emergency to the general surgeons complaining of abdominal pain, fever, jaundice and vomiting. Ultrasound was used and the diagnosis of biliary ascariasis was made. The patient underwent surgery consisting of a cholecystectomy, common bile duct exploration and T-tube choledochostomy. Our report highlights the varied aetiology of obstructive jaundice and the importance of including biliary ascariasis in the differential diagnosis of the jaundiced patient, especially from endemic areas.

  20. Update on Primary Biliary Cirrhosis

    Directory of Open Access Journals (Sweden)

    Jenny Heathcote

    2000-01-01

    Full Text Available The diagnosis of primary biliary cirrhosis (PBC is most often made in the asymptomatic phase, sometimes before the development of abnormal liver biochemistry. The antimitochondrial antibody remains the predominant hallmark, although not all patients test positive, even when the most sensitive techniques are used. The etiology of PBC remains elusive; studies suggest that the interlobular bile duct destruction is immune based, and associated autoimmune diseases are common. There are no surrogate markers that predict outcome in asymptomatic patients, whose chance of survival is less than that of age- and sex-matched populations but much better than the median survival of eight years in patients with symptomatic PBC. Symptoms common in this disease are fatigue, pruritus and xanthelasma, as well as complications of portal hypertension and osteoporosis. Treatment includes symptomatic and preventive measures, as well as specific therapeutic measures. Immunosuppressive therapy has yielded disappointing results in the long term management of PBC, and the only therapy shown to improve survival is the hydrophobic dihydroxy bile acid ursodeoxycholic acid. Treatment at a dose of 13 to 15 mg/kg/day is optimal, given in separate doses or as a single dose at least 4 h from giving the oral anion exchange resin cholestyramine, which may be used to control pruritus. However, liver transplantation remains the only cure for this disease, and the best postoperative survival is seen in patients whose serum bilirubin does not exceed 180 µmol/L at the time of liver transplantation. Recurrence takes place but is rarely symptomatic and does not deter from the benefits of transplantation.

  1. Biliary cysts: Etiology, diagnosis and management

    Institute of Scientific and Technical Information of China (English)

    Beata Jablo(n)ska

    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.

  2. 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.

  3. Laproscopic Management of Wandering Biliary Ascariasis

    Directory of Open Access Journals (Sweden)

    Umesh Jethwani

    2012-01-01

    Full Text Available Ascariasis is one of the most common helminthic diseases in humans, occurring mostly in countries with low standards of public health and hygiene, thereby making ascariasis highly endemic in developing countries. In endemic areas, 30% of adults and 60–70% of children harbour the adult worm. Biliary ascariasis is a rare cause of obstructive jaundice. Conventional management involves endoscopic extraction of worm. We are reporting a rare case of ascaris which induced extrahepatic biliary obstruction in a young male who presented with acute cholangitis. The ascaris was removed by laparoscopic exploration of the common bile duct. Postoperative period was uneventful.

  4. 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.

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

    Science.gov (United States)

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

    2008-07-01

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

  6. 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.

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

    DEFF Research Database (Denmark)

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

    2016-01-01

    spawned) v. potential fecundity (number of developing oocytes), suggesting significant variability in reproductive potential. The extent of fecundity regulation in relation to fish condition (Fulton’s condition factor) is discussed, suggesting an association between levels of atresia and fish condition......Down-regulation of fecundity through oocyte resorption was assessed in Baltic Sea turbot Scophthalmus maximus at three locations in the period from late vitellogenesis in April to spawning during June to July. The mean±s.d. total length of the sampled fish was 32⋅7±3⋅1 cm and mean±s.d. age was 6......⋅2±1⋅5 years. Measurements of atresia were performed using the ‘profile method’ with the intensity of atresia adjusted according to the ‘dissectormethod’ (10⋅6% adjustment; coefficient of determination was 0⋅675 between methods). Both prevalence (portion of fish with atresia) and intensity (calculated...

  8. 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.

  9. 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.

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

    Directory of Open Access Journals (Sweden)

    E. Brandigi

    2013-12-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

  12. 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.

  13. 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.

  14. IDUS for Biliary and Pancreatic Duct Lesions

    Institute of Scientific and Technical Information of China (English)

    Takao ltoi

    2008-01-01

    @@ In the recent decade, wire-guided intraductal US(IDUS), which can be passed through the working channel of standard duodenoscopes to provide high-frequency ultrasound images, has been developed as a newly diagnostic tool for biliary and pancreatic duct lesions.

  15. Biliary ascariasis on magnetic resonance cholangiopancreatography

    Directory of Open Access Journals (Sweden)

    Mohammad A Hashmi

    2009-01-01

    Full Text Available A 17-year-old girl presented with features of biliary obstruction. Magnetic resonance cholangi-pancreatography revealed typical linear signals in common bile duct, which appears like Ascaris lumbricoides. The diagnosis was confirmed by endoscopic removal of the worm.

  16. Management of biliary complications after livertransplantation

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    Biliary complications (BC) currently represent a majorsource of morbidity after liver transplantation. Althoughrefinements in surgical technique and medical therapyhave had a positive influence on the reduction of postoperativemorbidity, BC affect 5% to 25% of transplantedpatients. Bile leak and anastomotic stricturesrepresent the most common complications. Nowadays,a multidisciplinary approach is required to managesuch complications in order to prevent liver failure andretransplantation.

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

    Science.gov (United States)

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

    2007-08-01

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

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

    Directory of Open Access Journals (Sweden)

    Elena Kubovičova

    2011-12-01

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

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

    Directory of Open Access Journals (Sweden)

    Li Xue-zhong

    2011-12-01

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

  20. Biliary leaks after laparoscopic cholecystectomy:timetostentortimetodrain

    Institute of Scientific and Technical Information of China (English)

    Haim Pinkas; Patrick G. Brady

    2008-01-01

    BACKGROUND: Endoscopic retrograde cholangiopan-creatography (ERCP) with placement of a biliary stent or nasobiliary (NB) drain is the procedure of choice for treatment of post-cholecystectomy bile duct leaks. The aim of this study was to compare the effect of NB drainage versus internal biliary stenting on rates of leak closure, time elapsed until drain or stent removal, length of hospital stay and number of required endoscopic procedures. METHODS: Charts were reviewed on 20 patients who underwent laparoscopic cholecystectomy complicated by Luschka or cystic duct leak. Ten patients were treated with NB drains connected to low intermittent suction and repeat NB cholangiograms were performed until leak closure was observed. Ten patients were treated with internal biliary stents. Biliary sphincterotomies were performed for stone extraction or a presumed papillary stenosis. Large bilomas were drained percutaneously prior to stenting. RESULTS: In all 20 patients, a cholangiogram and successful placement of a NB drain or internal stent was achieved. Four patients (20%) were found to have bile duct stones, which were extracted following a sphincterotomy. Sixteen patients required percutaneous drains to evacuate large bilomas prior to biliary instrumentation. Fifteen cystic duct leaks and 5 Luschka duct leaks were reviewed. There were no complications related to ERCP. Closure of the leak was documented within 2 to 11 days (mean 4.7±0.9 days) in patients receiving a NB drain. The drains were removed non-endoscopically following leak closure. The internal stent group required stenting for 14 to 53 days (mean 29.1±4.4 days). The stent was then removed endoscopically after documentation of leak closure. Bile leaks following laparoscopic cholecystectomy closed rapidly after NB drainage and did not require repeat endoscopy for removal of the NB drain, resulting in fewer ERCPs required for treatment of biliary leaks. Internal biliary stents were in place longer owing

  1. Biliary tract infections caused by Aeromonas species.

    Science.gov (United States)

    Chao, C M; Lai, C C; Tang, H J; Ko, W C; Hsueh, P-R

    2013-02-01

    This study investigated the clinical and microbiological characteristics of patients with Aeromonas infections of the biliary tract. Patients with bile cultures positive for Aeromonas species during the period July 2004 to December 2011 were identified from a computerized database of a hospital in Taiwan. Patients with Aeromonas infections of the biliary tract were further identified. During the study period, a total of 1,142 isolates of Aeromonas species were obtained from 750 patients. Of those patients, 91 (12.1 %) had Aeromonas infections of the biliary tract. The annual incidence (episodes per 10,000 patient-days) of biliary tract infections caused by all Aeromonas species was 0.31 in 2007, 0.12 in 2010, and 0.27 in 2011. A. hydrophila was the most common species isolated (n = 41, 45.1 %), followed by A. caviae (n = 30, 33.0 %), A. veronii biovar sobria (n = 15, 16.5 %), and A. veronii biovar veronii (n = 5, 5.5 %). The majority of patients (n = 77, 84.6 %) had polymicrobial infections. Hepatobiliary stones (n = 50, 54.9 %) and hepatobiliary cancer (n = 38, 41.8 %) were the most common underlying diseases, followed by diabetes mellitus (n = 29, 31.9 %) and liver cirrhosis (n = 7, 7.7 %). The in-hospital mortality rate was 8.8 %. Infection-related mortality was associated with underlying immunocompromised condition (p = 0.044) and use of mechanical ventilation (p = 0.004), but was not associated with inappropriate antibiotic usage or concomitant bacteremia (n = 8, 8.8 %). In conclusion, biliary tract infections caused by Aeromonas species are not uncommon and can develop in both immunocompromised and immunocompetent patients; however, patients with underlying hepatobiliary diseases are particularly susceptible to these infections.

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

    Directory of Open Access Journals (Sweden)

    Gürkan Altun

    2013-01-01

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

  3. 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.

  4. 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.

  5. Evaluation of the biliary tract in patients with functional biliary symptoms

    Institute of Scientific and Technical Information of China (English)

    Peter Funch-Jensen; Asbj(φ)rn Mohr Drewes; László Madácsy

    2006-01-01

    The aim of this paper was to describe functional biliary syndromes and methods for evaluation of the biliary tract in these patients. Functional biliary symptoms can be defined as biliary symptoms without demonstrable organic substrate. Two main syndromes exist: Gallbladder dysfunction and sphincter of Oddi dysfunction. The most important investigative tools are cholescintigraphy and endoscopic sphincter of Oddi manometry. In gallbladder dysfunction a scintigraphic gallbladder ejection fraction below 35% can select patients who will benefit from cholecystectomy. Endoscopic sphincter of Oddi manometry is considered the gold standard in sphincter of Oddi dysfunction but recent development in scintigraphic methods is about to change this. Thus,calculation of hilum-to-duodenum transit time and duodenal appearance time on cholescintigraphy have proven useful in these patients. In conclusion, ambient methods can diagnose functional biliary syndromes.However, there are still a number of issues where further knowledge is needed. Probably the next step forward will be in the area of sensory testing and impedance planimetric methods.

  6. An Unusual Presentation of Biliary Ascariasis

    Directory of Open Access Journals (Sweden)

    Arsad Bashir Khan, Sanjay Kumar Bhasin, Rajesh Kumar Bhagat, R. K. Chrungoo

    2007-01-01

    Full Text Available Ascariasis is one of the most common disease in human being worldwide. Ascariasis is 2nd to gall stone asa cause of biliary symptoms. The invasion of biliary tract by round worms during early post operativeperiod is an infrequent but serious complication. We present 42 years old man operated for cholelithiasiswith Choledocholthiasis on whom choledochotomy and T -Tube insertion was done. On 5th postoperativeday Ascaris extruded peri-T-Tube and immediate T - Tube cholangiogram done that showed multiplefilling defects in Common Bile Duct (CBD. Patient was managed with saline irrigation of CBD viaT-Tube and anti-heliminthic was given. In view of its rarity and unusual presentation the case is beingreported.

  7. [Biliary ileus--potential complication of cholecystolithiasis].

    Science.gov (United States)

    Okolicány, R; Prochotský, A; Skultéty, J; Sekác, J; Mifkovic, A

    2008-11-01

    Biliary ileus is a rare complication of cholecystolithiasis. The condition occurs predominantly in the elderly with incidence rates of 1-4%, according to the literature data. Most commonly, it develops as a complication of cholelithiasis which remained untreated or was managed conservatively, or as a complication of a gallbladder decubitus necrosis. The condition results in a cholecysto-duodenal fistula. In this case, the cholecystolithiasis is latent or is clinically manifested in a third of the patients. A total of 1560 cholecystectomies (1345 L-CHE and 215 conventional CHE) were performed in our clinic during a five-year period. Biliary ileus was an indication for operation only in two subjects, during the studied period. In the both cases, the diagnosis was established intraoperatively, although upon re-examination of the visualization modalities views (upright native abdominal views, CT scans) the authors concluded that the primary cause of the ileus could have already been identified, based on the above views.

  8. [Biliary peritonitis: retrospective analysis of the disease].

    Science.gov (United States)

    Sirakov, M; Chupetlovski, S; Panov, Ts; Iliev, I

    2001-01-01

    The authors have done a biliary retrospective analysis of 10 deceased patients with a diagnosis of biliary peritonitis (BP). It is reported that the patients who died of BP are 9.5% of all who died of diffuse purulent peritonitis. All deceased patients were advanced and well advanced in years with prolonged complaints--in over 50% of them over 5 years. The complications set in 50% of the cases are recorded in the postoperative period. The perforation of the gallbladder and the biliopancreatitis are in the second place. Intrabiliary fistulas with 4 patients and 1 with a vesicocolon fistula; tumor of the pancreas with 4 patients; empyema of the gallbladder--3; diabetes with 3 of the patients and others are recorded as accompanying troubles which complicate the operation and the outcome of it. The time between the beginning of the complication and the operation with all the deceased patients is over 72 hours.

  9. Endoscopic management of benign biliary strictures.

    Science.gov (United States)

    Rustagi, Tarun; Jamidar, Priya A

    2015-01-01

    Benign biliary strictures are a common indication for endoscopic retrograde cholangiopancreatography (ERCP). Endoscopic management has evolved over the last 2 decades as the current standard of care. The most common etiologies of strictures encountered are following surgery and those related to chronic pancreatitis. High-quality cross-sectional imaging provides a road map for endoscopic management. Currently, sequential placement of multiple plastic biliary stents represents the preferred approach. There is an increasing role for the treatment of these strictures using covered metal stents, but due to conflicting reports of efficacies as well as cost and complications, this approach should only be entertained following careful consideration. Optimal management of strictures is best achieved using a team approach with the surgeon and interventional radiologist playing an important role.

  10. Biliary peritonitis following percutaneous nephrolithotomy: Minimally invasive management

    OpenAIRE

    Yadav, Siddharth; Singh, Animesh; Singh, Prabhjot

    2015-01-01

    Percutaneous nephrolithotomy (PCNL) is a standard procedure for large renal calculi but has potential for complications. Rarely, biliary tract injury can occur during PCNL that can lead to biliary peritonitis with sepsis. Such cases are usually managed by emergent cholecystectomy. We present a case of biliary peritonitis resulting from gall bladder injury during PCNL, managed minimally invasively with an abdominal drain and endoscopic retrograde cholangiography with common bile duct stenting.

  11. Biliary peritonitis following percutaneous nephrolithotomy: Minimally invasive management.

    Science.gov (United States)

    Yadav, Siddharth; Singh, Animesh; Singh, Prabhjot

    2015-01-01

    Percutaneous nephrolithotomy (PCNL) is a standard procedure for large renal calculi but has potential for complications. Rarely, biliary tract injury can occur during PCNL that can lead to biliary peritonitis with sepsis. Such cases are usually managed by emergent cholecystectomy. We present a case of biliary peritonitis resulting from gall bladder injury during PCNL, managed minimally invasively with an abdominal drain and endoscopic retrograde cholangiography with common bile duct stenting.

  12. Malignant Biliary Obstruction: Evidence for Best Practice

    Science.gov (United States)

    Pu, Leonardo Zorrón Cheng Tao; Singh, Rajvinder; Loong, Cheong Kuan; de Moura, Eduardo Guimarães Hourneaux

    2016-01-01

    What should be done next? Is the stricture benign? Is it resectable? Should I place a stent? Which one? These are some of the questions one ponders when dealing with biliary strictures. In resectable cases, ongoing questions remain as to whether the biliary tree should be drained prior to surgery. In palliative cases, the relief of obstruction remains the main goal. Options for palliative therapy include surgical bypass, percutaneous drainage, and stenting or endoscopic stenting (transpapillary or via an endoscopic ultrasound approach). This review gathers scientific foundations behind these interventions. For operable cases, preoperative biliary drainage should not be performed unless there is evidence of cholangitis, there is delay in surgical intervention, or intense jaundice is present. For inoperable cases, transpapillary stenting after sphincterotomy is preferable over percutaneous drainage. The use of plastic stents (PS) has no benefit over Self-Expandable Metallic Stents (SEMS). In case transpapillary drainage is not possible, Endoscopic Ultrasonography- (EUS-) guided drainage is still an option over percutaneous means. There is no significant difference between the types of SEMS and its indication should be individualized. PMID:26981114

  13. Excretion of biliary compounds during intrauterine life

    Institute of Scientific and Technical Information of China (English)

    Rocio IR Macias; Jose JG Marin; Maria A Serrano

    2009-01-01

    In adults, the hepatobiliary system, together with thekidney, constitute the main routes for the eliminationof several endogenous and xenobiotic compounds intobile and urine, respectively. However, during intrauterinelife the biliary route of excretion for cholephiliccompounds, such as bile acids and biliary pigments, isvery poor. Although very early in pregnancy the fetal liver produces bile acids, bilirubin and biliverdin, these compounds cannot be efficiently eliminated by the fetal hepatobiliary system, owing to the immaturity of the excretory machinery in the fetal liver. Therefore, the potentially harmful accumulation of cholephilic compounds in the fetus is prevented by their elimination across the placenta. Owing to the presence of detoxifying enzymes and specific transport systems at different locations of the placental barrier, such as the endothelial cells of chorionic vessels and trophoblast cells, this organ plays an important role in the hepatobiliary-like function during intrauterine life. The relevance of this excretory function in normal fetal physiology is evident in situations where high concentrations of biliary compounds are accumulated in the mother. This may result in oxidative stress and apoptosis, mainly in the placenta and fetal liver, which might affect normal fetal development and challenge the fate of the pregnancy. The present article reviews current knowledge of the mechanisms underlying the hepatobiliary function of the fetal-placental unit and the repercussions of several pathological conditions on this tandem.

  14. Excretion of biliary compounds during intrauterine life

    Science.gov (United States)

    Macias, Rocio IR; Marin, Jose JG; Serrano, Maria A

    2009-01-01

    In adults, the hepatobiliary system, together with the kidney, constitute the main routes for the elimination of several endogenous and xenobiotic compounds into bile and urine, respectively. However, during intrauterine life the biliary route of excretion for cholephilic compounds, such as bile acids and biliary pigments, is very poor. Although very early in pregnancy the fetal liver produces bile acids, bilirubin and biliverdin, these compounds cannot be efficiently eliminated by the fetal hepatobiliary system, owing to the immaturity of the excretory machinery in the fetal liver. Therefore, the potentially harmful accumulation of cholephilic compounds in the fetus is prevented by their elimination across the placenta. Owing to the presence of detoxifying enzymes and specific transport systems at different locations of the placental barrier, such as the endothelial cells of chorionic vessels and trophoblast cells, this organ plays an important role in the hepatobiliary-like function during intrauterine life. The relevance of this excretory function in normal fetal physiology is evident in situations where high concentrations of biliary compounds are accumulated in the mother. This may result in oxidative stress and apoptosis, mainly in the placenta and fetal liver, which might affect normal fetal development and challenge the fate of the pregnancy. The present article reviews current knowledge of the mechanisms underlying the hepatobiliary function of the fetal-placental unit and the repercussions of several pathological conditions on this tandem. PMID:19230042

  15. Reality named endoscopic ultrasound biliary drainage

    Institute of Scientific and Technical Information of China (English)

    Hugo; Gon?alo; Guedes; Roberto; Iglesias; Lopes; Joel; Fernandez; de; Oliveira; Everson; Luiz; de; Almeida; Artifon

    2015-01-01

    Endoscopic ultrasound(EUS) is used for diagnosis and evaluation of many diseases of the gastrointestinal (GI) tract. In the past, it was used to guide a cholangio-graphy, but nowadays it emerges as a powerful thera-peutic tool in biliary drainage. The aims of this review are: outline the rationale for endoscopic ultrasound-guided biliary drainage(EGBD); detail the procedural technique; evaluate the clinical outcomes and limitations of the method; and provide recommendations for the practicing clinician. In cases of failed endoscopic retro-grade cholangiopancreatography(ERCP), patients are usually referred for either percutaneous transhepatic biliary drainage(PTBD) or surgical bypass. Both these procedures have high rates of undesirable complications. EGBD is an attractive alternative to PTBD or surgery when ERCP fails. EGBD can be performed at two locations: transhepatic or extrahepatic, and the stent can be inserted in an antegrade or retrograde fashion. The drainage route can be transluminal, duodenal or trans-papillary, which, again, can be antegrade or retrograde [rendezvous(EUS-RV)]. Complications of all techniques combined include pneumoperitoneum, bleeding, bile leak/peritonitis and cholangitis. We recommend EGBD when bile duct access is not possible because of failed cannulation, altered upper GI tract anatomy, gastric outlet obstruction, a distorted ampulla or a periampullary diverticulum, as a minimally invasive alternative to surgery or radiology.

  16. Our experience of biliary ascariasis in children

    Directory of Open Access Journals (Sweden)

    Wani M

    2006-01-01

    Full Text Available Ascariasis is highly endemic in Kashmir valley as temperate climate and wet soil provide excellent conditions for its development. We studied 198 cases (11% of biliary ascariasis out of 1,800 patients of worm infestation from December 2001 to December 2004 in the age group of 4-15 years. In our study, we found biliary ascariasis to be more common in female children, and most common clinical presentation was biliary colic in 143 patients, cholangitis in 28, cholecystitis in 15, liver abscess 7, pancreatitis 4 and postoperative worm infestation in 1 patient. Ultrasonography of abdomen was the diagnostic tool of choice with hundred percent results. Most of the patients were managed conservatively. ERCP was not done in children because of need of general anesthesia and difficulty in performing the procedure. Surgical intervention was required in 23 patients (cholecystectomy with CBD exploration in 5, choledochotomy alone in 13, drainage of liver abscess in 3, choledochoduodenostomy in 1 and peritoneal lavage in 1.

  17. Malignant Biliary Obstruction: Evidence for Best Practice

    Directory of Open Access Journals (Sweden)

    Leonardo Zorrón Cheng Tao Pu

    2016-01-01

    Full Text Available What should be done next? Is the stricture benign? Is it resectable? Should I place a stent? Which one? These are some of the questions one ponders when dealing with biliary strictures. In resectable cases, ongoing questions remain as to whether the biliary tree should be drained prior to surgery. In palliative cases, the relief of obstruction remains the main goal. Options for palliative therapy include surgical bypass, percutaneous drainage, and stenting or endoscopic stenting (transpapillary or via an endoscopic ultrasound approach. This review gathers scientific foundations behind these interventions. For operable cases, preoperative biliary drainage should not be performed unless there is evidence of cholangitis, there is delay in surgical intervention, or intense jaundice is present. For inoperable cases, transpapillary stenting after sphincterotomy is preferable over percutaneous drainage. The use of plastic stents (PS has no benefit over Self-Expandable Metallic Stents (SEMS. In case transpapillary drainage is not possible, Endoscopic Ultrasonography- (EUS- guided drainage is still an option over percutaneous means. There is no significant difference between the types of SEMS and its indication should be individualized.

  18. Obstructing fungal cholangitis complicating metal biliary stent placement in pancreatic cancer

    Institute of Scientific and Technical Information of China (English)

    Brian; Story; Michael; Gluck

    2010-01-01

    Biliary obstructions can lead to infections of the biliary system, particularly in patients with occluded biliary stents. Fungal organisms are frequently found in bili-ary aspirates of patients who have been on antibiotics and have stents; however, fungal masses, or "balls", that fully obstruct the biliary system are uncommon and exceedingly diff icult to eradicate. We present 4 cases of obstructing fungal cholangitis in patients who had metal biliary stents placed for pancreatic malignancies, and subsequen...

  19. Surgical intervention for esophageal atresia in patients with trisomy 18.

    Science.gov (United States)

    Nishi, Eriko; Takamizawa, Shigeru; Iio, Kenji; Yamada, Yasumasa; Yoshizawa, Katsumi; Hatata, Tomoko; Hiroma, Takehiko; Mizuno, Seiji; Kawame, Hiroshi; Fukushima, Yoshimitsu; Nakamura, Tomohiko; Kosho, Tomoki

    2014-02-01

    Trisomy 18 is a common chromosomal aberration syndrome involving growth impairment, various malformations, poor prognosis, and severe developmental delay in survivors. Although esophageal atresia (EA) with tracheoesophageal fistula (TEF) is a potentially fatal complication that can only be rescued through surgical correction, no reports have addressed the efficacy of surgical intervention for EA in patients with trisomy 18. We reviewed detailed clinical information of 24 patients with trisomy 18 and EA who were admitted to two neonatal intensive care units in Japan and underwent intensive treatment including surgical interventions from 1982 to 2009. Nine patients underwent only palliative surgery, including six who underwent only gastrostomy or both gastrostomy and jejunostomy (Group 1) and three who underwent gastrostomy and TEF division (Group 2). The other 15 patients underwent radical surgery, including 10 who underwent single-stage esophago-esophagostomy with TEF division (Group 3) and five who underwent two-stage operation (gastrostomy followed by esophago-esophagostomy with TEF division) (Group 4). No intraoperative death or anesthetic complications were noted. Enteral feeding was accomplished in 17 patients, three of whom were fed orally. Three patients could be discharged home. The 1-year survival rate was 17%: 27% in those receiving radical surgery (Groups 3 and 4); 0% in those receiving palliative surgery (Groups 1 and 2). Most causes of death were related to cardiac complications. EA is not an absolute poor prognostic factor in patients with trisomy 18 undergoing radical surgery for EA and intensive cardiac management.

  20. Measurement of Gastric Circumference in Foetuses with Oesophageal Atresia.

    Science.gov (United States)

    Hoopmann, M; Kagan, K O; Borgmeier, F; Seitz, G; Arand, J; Wagner, P

    2015-11-01

    Background: The specific recognition of oesophageal atresia (OA) with or without a tracheal fistula in a foetus is a diagnostic challenge for prenatal medicine. The aim of the present work is to analyse the value of the measurement of gastric size in the diagnosis of this significant malformation. Materials and Methods: Altogether, the examinations of 433 pregnancies between the 18.4 and 39.1 weeks of gestation were retrospectively analysed. 59 of these foetuses exhibited an OA. By means of a linear regression analysis with normal foetuses, significant parameters influencing gastric size were examined. Subsequently the gastric sizes were transformed into z values and a comparison was made between OA with and without fistulae with the help of t tests. Results: In the normal foetuses there was a significant association between the gastric circumference and the abdominal circumference (circumference = 6.809 + 0.179 × abdominal circumference, r = 0.686, p values in the normal group and in the groups of OA with fistula and without fistula amounted to 0.0 (SD 1.0), -1.3 (SD 2.2) and -4.5 (SD 1.0). Conclusion: Measurements of the gastric circumference below the 5th percentile should lead to further diagnostic measures, especially when associated with polyhydramnios. Although OA without a fistula is always conspicuous, only about one in three OAs with fistula are associated with a significantly smaller stomach.

  1. 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.

  2. 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.

  3. Management of biliary ascariasis in pregnancy.

    Science.gov (United States)

    Shah, Omar Javed; Robanni, Irfan; Khan, Fayaz; Zargar, Showkat Ali; Javid, Gul

    2005-10-01

    Ascariasis is a helminthic infection of humans caused by the nematode Ascaris lumbricoides. Biliary ascariasis is one of the most common and well described entities caused by ascaris. In endemic areas pregnant women are prone to develop biliary ascariasis. Its management poses a great challenge to both the attending surgeon and the endoscopist. Between January 1993 and March 2003, 15 cases of biliary ascariasis were seen in pregnant patients in our institution. Ultrasonography was used as the main investigative tool. Treatment involved management by conservative, endoscopic, and surgical methods, taking due care of both the mother and the fetus. Ten patients (66.6%) were in the third trimester of pregnancy, and 10 (66.6%) patients were in their third pregnancy. Ultrasonography proved to be the best tool for diagnosing and monitoring worms inside the biliary ductal system. Nine (60%) patients responded to the conservative treatment; endoscopic extraction was successful in 4 (66.6%) patients. Surgical treatment was required in 2 (13.3%) patients. One (6.6%) patient had had spontaneous abortion at 12 weeks gestation, and one (6.6%) patient had a premature labor. The remaining patients had normal pregnancies. Management of biliary ascariasis in pregnancy is a challenge for both the attending surgeon and the endoscopist. Safe and effective management requires special attention to the gestational age and accurate recognition of the specific pathology in the patient. The majority of patients respond to conservative treatment, but endoscopic extraction may be needed in nonresponsive cases. Lead shielding of the fetus and limitation of the total fluoroscopic exposure during therapeutic endoscopy can minimize the teratogenic risk of ionizing radiation. Failures of endoscopic extraction may lead to surgical intervention, which carries risks of fetal wastage and premature labor. Routine worming of women in the child-bearing years is recommended in endemic areas of ascariasis

  4. 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

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

    Science.gov (United States)

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

    2012-12-01

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

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

    Science.gov (United States)

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

    2006-04-01

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

  7. Biliary microlithiasis, sludge, crystals, microcrystallization,andusefulnessof assessmentofnucleationtime

    Institute of Scientific and Technical Information of China (English)

    Vasitha Abeysuriya; Kemal I Deen; Navarathne MM Navarathne

    2010-01-01

    BACKGROUND:The process of microcrystallization, its sequel and the assessment of nucleation time is ignored. This systematic review aimed to highlight the importance of biliary microlithiasis, sludge, and crystals, and their association with gallstones, unexplained biliary pain, idiopathic pancreatitis, and sphincter of Oddi dysfunction. DATA SOURCES:Three reviewers performed a literature search of the PubMed database. Key words used were"biliary microlithiasis","biliary sludge","bile crystals","cholesterol crystallisation","bile microscopy","microcrystal formation of bile", "cholesterol monohydrate crystals", "nucleation time of cholesterol", "gallstone formation", "sphincter of Oddi dysfunction"and"idiopathic pancreatitis". Additional articles were sourced from references within the studies from the PubMed search. RESULTS:We found that biliary microcrystals account for almost all patients with gallstone disease, 7% to 79% with idiopathic pancreatitis, 83% with unexplained biliary pain, and 25%to 60%with altered biliary and pancreatic sphincter function. Overall, the detection of biliary microcrystals in gallstone disease has a sensitivity ranging from 55%to 87%and a speciifcity of 100%. In idiopathic pancreatitis, the presence of microcrystals ranges from 47%to 90%. A nucleation time less than 10 days in hepatic bile or ultra-ifltered gallbladder bile has a speciifcity of 100%for cholesterol gallstone disease. CONCLUSIONS:Biliary crystals are associated with gallstone disease, idiopathic pancreatitis, sphincter of Oddi dysfunction, unexplained biliary pain, and post-cholecystectomy biliary pain. Pathways of cholesterol super-saturation, crystallisation, and gallstone formation have been described with scientiifc support. Bile microscopy is a useful method to detect microcrystals and the assessment of nucleation time is a good method of predicting the risk of cholesterol crystallisation.

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

    Science.gov (United States)

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

    2016-01-01

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

  9. Simultaneous Vibrant Soundbridge Implantation and 2nd Stage Auricular Reconstruction for Microtia with Aural Atresia.

    Science.gov (United States)

    Lim, Lynne Hsueh Yee; Xiang, Ling; Del Prado, Jocelynne; Ee Chin, Ling; Beltrame, Millo Achille

    2011-07-01

    Aural atresia and severe microtia are associated malformations that result in problems with hearing and cosmesis, associated speech and language difficulties and diminished self-esteem. In cases where middle ear ossiculoplasty and aural atresia canalplasty are expected to give poor hearing outcomes that would eventually require the use of hearing aids, bone anchored hearing aids or active middle ear implants may be better options. This case report describes a simultaneous Vibrant Soundbridge implantation and 2(nd) stage auricular reconstruction with rib graft cartilage for an 11-year-old boy with grade III microtia and aural atresia 8 months after the 1(st) stage reconstruction. Audiometric results of the Vibrant Soundbridge aided ear were comparable to that of the contralateral hearing aid aided ear.

  10. Simultaneous vibrant soundbridge implantation and 2nd stage auricular reconstruction for microtia with aural atresia

    Directory of Open Access Journals (Sweden)

    Lynne Hsueh Yee Lim

    2011-09-01

    Full Text Available Aural atresia and severe microtia are associated malformations that result in problems with hearing and cosmesis, associated speech and language difficulties and diminished self-esteem. In cases where middle ear ossiculoplasty and aural atresia canalplasty are expected to give poor hearing outcomes that would eventually require the use of hearing aids, bone anchored hearing aids or active middle ear implants may be better options. This case report describes a simultaneous Vibrant Soundbridge implantation and 2nd stage auricular reconstruction with rib graft cartilage for an 11-year-old boy with grade III microtia and aural atresia 8 months after the 1st stage reconstruction. Audiometric results of the Vibrant Soundbridge aided ear were comparable to that of the contralateral hearing aid aided ear.

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

    Science.gov (United States)

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

    2006-10-01

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

  12. Congenital external auditory canal atresia and stenosis: temporal bone CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Dong Hoon; Kim, Bum Soo; Jung, So Lyung; Kim, Young Joo; Chun, Ho Jong; Choi, Kyu Ho; Park, Shi Nae [College of Medicine, Catholic Univ. of Korea, Seoul (Korea, Republic of)

    2002-04-01

    To determine the computed tomographic (CT) findings of atresia and stenosis of the external auditory canal (EAC), and to describe associated abnormalities in surrounding structures. We retrospectively reviewed the axial and coronal CT images of the temporal bone in 15 patients (M:F=8:7;mean age, 15.8 years) with 16 cases of EAC atresia (unilateral n=11, bilateral n=1) and EAC stenosis (unilateral n=3). Associated abnormalities of the EAC, tympanic cavity, ossicles, mastoid air cells, eustachian tube, facial nerve course, mandibular condyle and condylar fossa, sigmoid sinus and jugular bulb, and the base of the middle cranial fossa were evaluated. Thirteen cases of bony EAC atresia (one bilateral), with an atretic bony plate, were noted, and one case of unilateral membranous atresia, in which a soft tissue the EAC. A unilateral lesion occurred more frequently on the right temporal bone (n=8, 73%). Associated abnormalities included a small tympanic cavity (n=8, 62%), decreased mastoid pneumatization (n=8, 62%), displacement of the mandibular condyle and the posterior wall of the condylar fossa (n=7, 54%), dilatation of the Eustachian tube (n=7, 54%), and inferior displacement of the temporal fossa base (n=8, 62%). Abnormalities of ossicles were noted in the malleolus (n=12, 92%), incus (n=10, 77%) and stapes (n=6, 46%). The course of the facial nerve was abnormal in four cases, and abnormality of the auditory canal was noted in one. Among three cases of EAC stenosis, ossicular aplasia was observed in one, and in another the location of the mandibular condyle and condylar fossa was abnormal. In the remaining case there was no associated abnormality. Atresia of the EAC is frequently accompanied by abnormalities of the middle ear cavity, ossicles, and adjacent structures other than the inner ear. For patients with atresia and stenosis of this canal, CT of the temporal bone is essentially helpful in evaluating these associated abnormalities.

  13. Prenatal Diagnosis and Pathology of Laryngeal Atresia in Congenital High Airway Obstruction Syndrome

    Directory of Open Access Journals (Sweden)

    Piya Chaemsaithong

    2012-01-01

    Full Text Available Congenital high airway obstruction syndrome is a rare but life-threatening condition. Therefore, prenatal diagnosis is important. The obstruction can be due to laryngeal/tracheal atresia or external compression. While a differential diagnosis with congenital cystic adenomatoid malformation (CCAM type III may be difficult, it is still possible with ultrasonography. In this study, we report a case of bilateral echogenic lungs with hydrops fetalis. After the prenatal diagnosis of laryngeal atresia, the couple opted to have an elective termination of pregnancy performed at 20 weeks of gestation. The diagnosis was confirmed by a complete pathological examination.

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

    Directory of Open Access Journals (Sweden)

    Alpana

    2013-06-01

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

  15. Newborn with congenital facial palsy and bilateral anotia/atresia of external auditory canal: Rare occurrence

    Science.gov (United States)

    Mahale, Rohan R.; Mehta, Anish; John, Aju Abraham; Buddaraju, Kiran; Shankar, Abhinandan K.; Rangasetty, Srinivasa

    2016-01-01

    Congenital facial palsy (CFP) is clinically defined as facial palsy of the seventh cranial nerve which is present at birth or shortly thereafter. It is generally considered to be either developmental or acquired in origin. Facial palsy of developmental origin is associated with other anomalies including those of pinna and external auditory canal, which range from mild defects to severe microtia and atresia. We report a 2-day-old male newborn that had right CFP with bilateral anotia and atresia of external auditory canals which is rare. PMID:27857806

  16. 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....

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

    Directory of Open Access Journals (Sweden)

    Rahul Gupta

    2016-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

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

    2015-01-01

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

  19. 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.

  20. Atresia de la vena porta en un canino : Reporte de un caso

    OpenAIRE

    Allende, Miriam G.; Acosta, Walter; Baschar, H. A.; Massone, Adriana R.; Diez, M. L.; Amo, Alicia N. del

    2006-01-01

    La atresia vascular portal es una anomalía congénita poco frecuente en los caninos. Los shunts porto sistémicos (SPS) son comunicaciones entre la vena porta y el sistema venoso, de manera que la sangre portal pasa a la circulación general sin ingresar al hígado. Los mismos pueden ser congénitos o adquiridos y presentar una ubicación intra o extra hepática. En asociación a la atresia portal se han hallado shunts simples congénitos y múltiples adquiridos. El presente trabajo describe la present...

  1. Atresia de coanas, revisión y una mirada desde la evidencia

    OpenAIRE

    2014-01-01

    La atresia de coanas es la anomalía congénita nasal más común. Cuando es bilateral, se presenta con dificultad respiratoria desde el nacimiento. La atresia unilateral se manifiesta con insuficiencia ventilatoria y rinorrea unilateral, pudiendo pasar inadvertida. El diagnóstico se sospecha ante la ausencia de paso de aire en las fosas nasales y la imposibilidad de hacer progresar una sonda nasogástrica. Se confirma mediante examen endoscópico y tomografía computarizada. El tratamiento definiti...

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

    Science.gov (United States)

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

    2016-01-01

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

  3. 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.)

  4. The role of sonography in imaging of the biliary tract.

    Science.gov (United States)

    Foley, W Dennis; Quiroz, Francisco A

    2007-06-01

    Sonography is the recommended initial imaging test in the evaluation of patients presenting with right upper quadrant pain or jaundice. Dependent upon clinical circumstances, the differential diagnosis includes choledocholithiasis, biliary stricture, or tumor. Sonography is very sensitive in detection of mechanical biliary obstruction and stone disease, although less sensitive for detection of obstructing tumors, including pancreatic carcinoma and cholangiocarcinoma. In patients with sonographically documented cholelithiasis and choledocholithiasis, laparoscopic cholecystectomy with operative clearance of the biliary stone disease is usually performed. In patients with clinically suspected biliary stone disease, without initial sonographic documentation of choledocholithiasis, endoscopic ultrasound or magnetic resonance cholangiopancreatography is the next logical imaging step. Endoscopic ultrasound documentation of choledocholithiasis in a postcholecystectomy patient should lead to retrograde cholangiography, sphincterotomy, and clearance of the ductal calculi by endoscopic catheter techniques. In patients with clinical and sonographic findings suggestive of malignant biliary obstruction, a multipass contrast-enhanced computed tomography (CT) examination to detect and stage possible pancreatic carcinoma, cholangiocarcinoma, or periductal neoplasm is usually recommended. Assessment of tumor resectability and staging can be performed by CT or a combination of CT and endoscopic ultrasound, the latter often combined with fine needle aspiration biopsy of suspected periductal tumor. In patients whose CT scan suggests hepatic hilar or central intrahepatic biliary tumor, percutaneous cholangiography and transhepatic biliary stent placement is usually followed by brushing or fluoroscopically directed fine needle aspiration biopsy for tissue diagnosis. Sonography is the imaging procedure of choice for biliary tract intervention, including cholecystostomy, guidance for

  5. Ceftriaxone-associated nephrolithiasis and biliary pseudolithiasis in a child

    Energy Technology Data Exchange (ETDEWEB)

    Prince, Jeffrey S. [Department of Radiology, UCSD Medical Center, 200 West Arbor Dr., Mail Code 8756, San Diego, CA 92103-8756 (United States); Senac, Melvin O. [Department of Radiology, Children' s Hospital and Health Center, 3020 Children' s Way, San Diego, CA 92123-4282 (United States)

    2003-09-01

    Ceftriaxone is a widely used third-generation cephalosporin. It is generally very safe, but complications of biliary pseudolithiasis and, rarely, nephrolithiasis have been reported in children. These complications generally resolve spontaneously with cessation of the ceftriaxone therapy; however, they may symptomatically mimic more serious clinical problems, such as cholecystitis. We report a case of both ceftriaxone-induced biliary pseudolithiasis and nephrolithiasis. (orig.)

  6. Biliary ascariasis: MR cholangiography findings in two cases

    Energy Technology Data Exchange (ETDEWEB)

    Hwang, Cheol Mok; Kim, Tae Kyoung; Ha, Hyun Kwon; Kim, Pyo Nyun; Lee, Moon Gyu [Ulsan University College of Medicine, Seoul (Korea, Republic of)

    2001-09-01

    We describe the imaging features of two cases of biliary ascariasis. Ultrasonography and CT showed no specific abnormal findings, but MR cholangiography clearly demonstrated an intraductal linear filling defect that led to the correct diagnosis. MR cholangiography is thus a useful technique for the diagnosis of biliary ascariasis.

  7. Biliary reflux detection in anomalous union of the pancreatico-biliary duct patients

    Institute of Scientific and Technical Information of China (English)

    Suk Keu Yeom; Seung Wha Lee; Sang Hoon Cha; Hwan Hoon Chung; Bo Kyung Je; Baek Hyun Kim; Jong Jin Hyun

    2012-01-01

    AIM:To demonstrate the imaging findings of biliopancreatic and pancreatico-biliary reflux in patients with anomalous union of the pancreatico-biliary duct (AUPBD)on gadoxetic acid-enhanced functional magnetic resonance cholangiography (fMRC).METHODS:This study included six consecutive patients (two men and four women; mean age 47.5 years) with AUPBD.All subjects underwent endoscopic retrograde cholangiopancreatography (ERCP); one subject also underwent bile sampling of the common bile duct (CBD) to measure the amylase level because his gadoxetic acidenhanced fMRC images showed evidence of pancreatico-biliary reflux of pancreatic secretions.Of the five patients with choledochal cysts,four underwent pyloruspreserving pancreaticoduodenectomy.RESULTS:The five cases of choledochal cysts were classified as Todani classification I.In three of the six patients with AUPBD,injected contrast media reached the distal CBD and pancreatic duct on delay images,suggesting biliopancreatic reflux.In two of these six patients,a band-like filling defect was noted in the CBD on pre-fatty meal images,which decreased in size on delayed post-fatty meal images,suggesting pancreatico-biliary reflux of pancreatic secretions,and the bile sampled from the CBD in one patient had an amylase level of 113 000 IU/L.In one of the six patients with AUPBD,contrast media did not reach the distal CBD due to multiple CBD stones.CONCLUSION:Gadoxetic acid-enhanced fMRC successfully demonstrated biliopancreatic reflux of bile and pancreatico-biliary reflux of pancreatic secretions in patients with AUPBD with and without choledochal cysts.

  8. Medical image of the week: ascending cholangitis from biliary obstruction

    Directory of Open Access Journals (Sweden)

    Wong C

    2013-04-01

    Full Text Available A 79 year old man with a history of quadriplegia presented to an outside hospital in septic shock. He was found to have an elevated total bilirubin of 10 mg/dL, direct bilirubin of 7 mg/dL, alkaline phosphatase of 405 U/L, and lipase of 370 U/L. Imaging showed cholelithiasis with likely intra- and extrahepatic biliary duct dilatation. The patient underwent placement of a biliary drain with clinical improvement. Additional imaging was requested prior to endoscopic retrograde cholangiopancreatography (ERCP, but magnetic resonance cholangiopancreatography (MRCP was unavailable due to metallic implants. Interventional radiology performed a cholangiogram using the biliary drain which confirmed biliary obstruction. ERCP was then performed, with significant biliary sludge found and two stents placed.

  9. Parasitic disease of the liver and biliary tree

    Directory of Open Access Journals (Sweden)

    Mohamed Abdulrahman

    1997-01-01

    Full Text Available Several parasites infest liver or biliary tree, either during their maturation stages or as adult worms. Bile iry tree parasites may cause pancreatitis, cholecystitis, biliary tree obstruction, recurrent cholangitis, biliary tree strictures and some may lead to cholangiocarcinoma. This review discusses the hepatobiliary parasites, and shows our experience in diagnosis and management of these parasites. Ultrasonography of the liver is diagnostic in schistosomiasis, hydatid cysts, amebic liver abscess, ascariasis and other biliary tree parasites showing bile duct dilatation. Percutaneous aspiration under ultrasonography guidance of hydatid liver cysts or amebic abscess are effective measures in management. Endoscopic retrograde cholangiopancreatography (ERCP is safe and effective in diagnosis and management of biliary tree parasites.

  10. Effect of preoperative biliary drainage on outcome of classical pancreaticoduodenectomy

    Institute of Scientific and Technical Information of China (English)

    Chandra Shekhar Bhati; Chandrashekhar Kubal; Pankaj Kumar Sihag; Ankur Atal Gupta; Raj Kamal Jenav; Nicholas G Inston; Jagdish M Mehta

    2007-01-01

    AIM: To investigate the role of preoperative biliary drainage (PBD) in the outcome of classical pancreaticodu odenectomy.METHODS: A 10-year retrospective data analysis was performed on patients (n = 48) undergoing pancreaticoduodenectomy from March 1994 to March 2004 in department of surgery at SMS medical college, Jaipur, India. Demographic variables, details of preoperative stenting, operative procedure and post operative complications were noted.RESULTS: Preoperative biliary drainage was performed in 21 patients (43.5%). The incidence of septic complications was significantly higher in patients with biliary stent placement (P < 0.05, 0 vs 4). This group of patients also had a significantly higher minor biliary leak rate. Mortality and hospital stay in each group was comparable.CONCLUSION: Within this study population the use of PBD by endoscopic stenting was associated with a high incidence of infective complications. These findings do not support the routine use of biliary stenting in patients prior to pancreatico-duodenectomy.

  11. Amylase creatinine clearance ratio after biliary surgery.

    Science.gov (United States)

    Donaldson, L A; McIntosh, W; Joffe, S N

    1977-01-01

    The amylase creatinine clearance ratio (ACCR) is considered to be a more sensitive index of acute pancreatitis than the serum amylase level. Serial ACCR estimations were undertaken in 25 patients undergoing an elective cholecystectomy. Using accepted criteria, 28% of these patients developed, in the postoperative period, biochemical evidence of pancreatic gland damage, although the serum amylase level remained normal. This raised ACCR was particularly noted in patients who had undergone an exploration of the common bile duct. The ACCR would appear to be a more sensitive index of pancreatic gland disruption secondary to biliary surgery than the serum amylase level.

  12. Risk factors of severe ischemic biliary complications after liver transplantation

    Institute of Scientific and Technical Information of China (English)

    Ming-FengWang; Zhong-Kui Jin; Da-Zhi Chen; Xian-Liang Li; Xin Zhao; Hua Fan

    2011-01-01

    BACKGROUND: Ischemia-related biliary tract complications remain high after orthotopic liver transplantation. Severe ischemic biliary complications often involve the hepatic duct bifurcation and left hepatic duct, resulting finally in obstructive jaundice. Prevention and management of such complications remain a challenge for transplant surgeons. METHODS: All 160 patients were followed up for at least 180 days after transplantation. One-way analysis of variance (ANOVA) and comparative univariate analysis were made using 3 groups (no complications; mild complications;severe complications), to analyze risk factors associated with biliary complications. Multiple logistic regression and linear regression analysis were used to analyze independent risk factors for severe ischemic biliary complications, after excluding other confounding factors. RESULTS: By ANOVA and comparative univariate analysis, the risk factors associated with biliary complications were preoperative bilirubin level (P=0.007) and T-tube stenting of the anastomosis (P=0.016). Multiple logistic regression analysis showed that the use of T-tube and preoperative serum bilirubin were not independent risk factors for severe ischemic biliary complications after orthotopic liver transplantation. Chi-square analysis indicated that in the incidence of severe ischemic biliary lesions, bile duct second warm ischemic time longer than 60 minutes was a significant risk factor. Linear regression demonstrated a negative correlation between cold preservation time and warm ischemia time. CONCLUSIONS: Preoperative serum bilirubin level and the use of T-tube stenting of the anastomosis were independent risk factors for biliary complications after liver transplantation, but not for severe ischemic biliary complications. The second warm ischemia time of bile duct longer than 60 minutes and prolonged bile duct second warm ischemia time combined with cold preservation time were significant risk factors for severe

  13. Hepato-biliary clinical trials and their inclusion in the Cochrane Hepato-Biliary Group register and reviews

    DEFF Research Database (Denmark)

    Klingenberg, Sarah Louise; Nikolova, Dimitrinka; Alexakis, Nicholas

    2011-01-01

    The Cochrane Hepato-Biliary Group (CHBG) is one of the 52 collaborative review groups within The Cochrane Collaboration. The activities of the CHBG focus on collecting hepato-biliary randomized clinical trials (RCT) and controlled clinical trials (CCT), and including them in systematic reviews wi...

  14. Trends in pediatric ostomy surgery: intestinal diversion for necrotizing enterocolitis and biliary diversion for biliary hypoplasia syndromes.

    Science.gov (United States)

    Bastawrous, A A; Torosian, M B; Statter, M B; Arensman, R M

    1995-11-01

    Ostomies are placed in children for different indications than in the older population. Many ostomies of childhood are placed because of congenital or neonatal problems that require temporary or long-term diversion to stabilize the neonatal patient. Necrotizing enterocolitis, the most common reason for placement of neonatal colostomies and ileostomies, is increasing in frequency as more prematurely born infants survive. Recently, there has been an increase in treatment of various biliary hypoplasia syndromes with biliary cutaneous diversion. Children with biliary hypoplasia syndromes are a challenging group of patients who frequently can be helped by ostomies. This article reviews current information on biliary cutaneous diversion for the biliary hypoplasia syndromes and intestinal diversion for necrotizing enterocolitis.

  15. Biliary ascariasis in a bile duct stones-removed female patient

    OpenAIRE

    2013-01-01

    Biliary ascariasis is a common problem in rural areas in China. The common presentations include biliary colic, acute cholangitis, obstructive jaundice, choledocholithiasis and acute cholecystitis. Here, we describe a case with biliary ascariasis two days after endoscopic sphincterotomy for choledocholithiasis. A living ascaris was successfully removed by endoscopic retrograde cholangiopancreatography. This case indicated that biliary ascariasis is not an uncommon complication of endoscopic s...

  16. The Unfinished Business of Primary Biliary Cirrhosis

    Science.gov (United States)

    Selmi, Carlo; Zuin, Massimo; Gershwin, M. Eric

    2008-01-01

    Summary In nearly every multifactorial human disease, there are three periods that characterize our understanding and definition. First, there is a period in which there is rapid accumulation of descriptive data. Second, there is a longer and slower period as information is obtained that redefines and expands basic and clinical knowledge that lacks the final and important area of understanding aetiology and therapeutic intervention. Third, which is much less common for most diseases, is the vigorous definition of pathobiology and treatment. These phases are well illustrated by our current understanding of primary biliary cirrhosis (PBC). The term PBC was first used nearly 60 years ago and for the first 40 or so years, the primary research efforts were directed at clinical definitions and pathology. Subsequently, with the advent of molecular biology, there began a rigorous dissection of the immune response and, in particular, a better understanding of anti-mitochondrial antibodies. These efforts have greatly helped in our understanding of not only the effector mechanisms of disease, but also the uniqueness of the primary target tissue, biliary epithelium. However, this research has still not led to successful translation for specific therapy. PMID:18640737

  17. Cholangiocyte anion exchange and biliary bicarbonate excretion

    Institute of Scientific and Technical Information of China (English)

    Jesús M Banales; Jesús Prieto; Juan F Medina

    2006-01-01

    Primary canalicular bile undergoes a process of fluidization and alkalinization along the biliary tract that is influenced by several factors including hormones, innervation/neuropeptides, and biliary constituents. Theexcretion of bicarbonate at both the canaliculi and the bile ducts is an important contributor to the generation of the so-called bile-salt independent flow. Bicarbonate is secreted from hepatocytes and cholangiocytes through parallel mechanisms which involve chloride efflux through activation of Cl- channels, and further bicarbonate secretion via AE2/SLC4A2-mediated Cl-/HCO3-exchange. Glucagon and secretin are two relevant hormones which seem to act very similarly in their target cells (hepatocytes for the former and cholangiocytes for the latter). These hormones interact with their specific G protein-coupled receptors, causing increases in intracellular levels of cAMP and activation of cAMP-dependent Cl- and HCO3- secretory mechanisms. Both hepatocytes and cholangiocytes appear to have cAMP-responsive intracellular vesicles in which AE2/SLC4A2 colocalizes with cell specific Cl- channels (CFTR in cholangiocytes and not yet determined in hepatocytes) and aquaporins (AQP8 in hepatocytes and AQP1 in cholangiocytes). cAMP-induced coordinated trafficking of these vesicles to either canalicular or cholangiocyte lumenal membranes and further exocytosis results in increased osmotic forces and passive movement of water with net bicarbonate-rich hydrocholeresis.

  18. [Environmental factors and primary biliary cirrhosis].

    Science.gov (United States)

    Chen, L P; Zhao, H; Lyu, B; Cheng, J L

    2016-07-20

    The complex interplay between immune factors and genetic susceptibility plays an essential role in autoimmune diseases. This is especially true for primary biliary cirrhosis (PBC). PBC is an autoimmune cholestatic liver disease characterized by the destruction of the small intrahepatic bile ducts and the presence of high-titer antimitochondrial antibodies (AMA). Among the currently known risk factors, genetic predisposition remains to be the dominant one. However, it is insufficient to explain the different geographic distributions of PBC and the incomplete concordance in identical twins. This suggests an association between specific environmental factors and the development of PBC. Nevertheless, a clear and rational association of environmental factors with primary biliary cirrhosis has not yet been fully elucidated. Our current understanding of the environmental triggers of PBC is limited to numerous suspected factors involved in its development, such as xenobiotics, electrophilic drugs, infection, and other physical, chemical, and even biological factors. Although the factors leading to the breakdown of immune tolerance in PBC are still largely unknown, related geoepidemiological studies may help us better understand the impact of the environment. In addition, a better understanding of the interplay between environmental factors and PBC is the critical step toward improving our management and control of PBC and autoimmunity in general.

  19. Endoscopic treatment of malignant biliary strictures.

    Science.gov (United States)

    Rustagi, Tarun; Jamidar, Priya A

    2015-01-01

    Endoscopic stenting is a widely accepted strategy for providing effective drainage in both extrahepatic and intrahepatic malignant strictures. In patients with extrahepatic malignancies, uncovered self-expanding metal stents (SEMS) provide excellent palliation. Hilar malignancies are probably best palliated by placement of uncovered SEMS although some disagreement exists among experts regarding the type and number of stents for optimal palliation. Preoperative biliary drainage (PBD) is commonly performed although a higher risk of complications and the lack of clear benefit raise questions about this practice. Certain groups of patients such as those with markedly elevated bilirubin levels, and in those in whom neoadjuvant therapy is planned, are good candidates for PBD. Considerable controversy exists regarding the optimal method as well as type of stent for PBD in patients with hilar malignancies. Novel endoscopic therapies, including photodynamic therapy and radiofrequency ablation, have emerged as potential adjuvant therapies in the management of malignant bile duct strictures but need further long-term evaluation to establish survival benefit. This review focuses on the current status of endoscopic therapies for malignant biliary obstructions.

  20. 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.

  1. Unilateral pulmonary agenesis and esophageal atresia with a tracheoesphageal fistula-23 year followup

    Directory of Open Access Journals (Sweden)

    Michael Curci

    2015-04-01

    Full Text Available Pulmonary agenesis, esophageal atresia and a tracheoesophageal fistula (EA + TEF are a rare combined congenital anomalies associated with a high morbidity and mortality. For those patients that have survived these malformation, there has been limited long-term follow up. This case report describes a 23-year followup with evaluation of the patient's pulmonary, cardiac and gastrointestinal function.

  2. 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 transcutaneo

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

    Science.gov (United States)

    Worku, Tesfaye; Rehman, Zia Ur; Talpur, Hira Sajjad; Bhattarai, Dinesh; Ullah, Farman; Malobi, Ngabu; Kebede, Tesfaye; Yang, Liguo

    2017-01-01

    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. PMID:28208755

  4. 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.

  5. ‘Latent’ Portal Hypertension in Benign Biliary Obstruction

    Science.gov (United States)

    Ibrarullah, Md.; Sikora, S. S.; Agarwal, D. K.; Kapoor, V. K.; Kaushik, S. P.

    1996-01-01

    A prospective study was undertaken to evaluate the changes in portal venous pressure in patients with benign biliary obstruction (BBO) but without overt clinical, endoscopic or radiological evidence of portal hypertension. Portal venous pressure was measured at laparotomy in 20 patients (10 each with either benign biliary stricture or choledocholithiasis) before and after biliary decompression. Pressure was found to be on the high side in seven patients (>25 cm of saline in three patients and > 30 cm of saline in four). The mean fall of pressure was 3.4 cm of saline after biliary decompression. No correlation could, however, be found between portal venous pressure and duration of biliary obstruction, serum bilirubin or bile duct pressure. Liver histology showed mild to moderate cholestatic changes but maintained portal architecture in all. Benign biliary obstruction may therefore, lead to elevation of portal pressure, even though the patient may not necessarily have any clinical, endoscopic or radiological manifestations of portal hypertension. The pathogenesis of this ‘latent’ portal hypertension is probably multifactorial. If biliary obstruction is left untreated the development of overt portal hypertension may become a possibility in the future. PMID:8725455

  6. The expression of IFN-gamma and its receptor in the liver of experimental biliatry atresia%小鼠胆道闭锁肝脏组织干扰素-γ及其受体mRNA表达

    Institute of Scientific and Technical Information of China (English)

    汤绍涛; 马亚贞; 杨瑛

    2008-01-01

    Objective To study the expression pattern of IFN-γ and IFN-γ receptor in the liver of biliary atresia(BA)mice.Methods Biliary atresia were induced in mice by intra-peritoneal RRV injection.Forty-five mice were of BA group and 24 mice which received 2%FCS-MEM intraperitoneal-ly were used as control The expression of IFN-γ7 and IFN-γ mRNA in livers of mice were detected with RT-PER.Results Compared to the control group,the expression of IFN-γ in the liver of BA mice at different ages was much higher,It increased first and then deereased,peaking on the 9th days after inoculating with RRv.The level was aboUt thirty times that of the control group.Meanwhile the IFN-γ R mRNA expression also increased first and then decreased,and it peaked on the 9th day.It was significantly higher than control group on the 3,6,9,14th day.The level dropped back to that of the control on the 21th day.Conclusions The expression of IFN-γ and its receptor increased almost synchronously in the livers of BA mice,a possible explanation could be receptor specific effects of in-terferon-γ on the signal transduction levels.%目的 动态检测小鼠胆道闭锁(biliary atresia,BA)模型肝脏组织中干扰素-γ(IFN-γ)及其受体IFN-γ R mRNA的表达情况,探索其与BA发生的关系.方法 采用腹腔注射恒河猴轮状病毒建立BALB/c新生小鼠BA模型,其巾BA组45只,对照组24只(腹腔注射病毒培养液).应用RT-PCR方法检测小鼠模型肝脏中IFN-γ及其受体mRNA的表达.结果 与对照组小鼠相比,不同鼠龄BA组肝内IFN-γ mRNA的表达水平均明显高于对照组,并出现先升高后降低趋势,第九天时达到峰值,约为对照组30倍.IFN-γ受体mRNA表达水平同样出现先升高后下降趋势,第九天达到最高水平,明显高于对照组.IFN-γ受体mRNA在第3、6、9、14天时明显高于对照组,到第21天时恢复到基线水平.结论 小鼠BA模型肝组织中IFN-γ及其受体mRNA表达水平基本同步上调,IFN-γ通

  7. An imaging study of the facial nerve canal in congenital aural atresia.

    Science.gov (United States)

    Zhao, Shouqin; Han, Demin; Wang, Zhenchang; Li, Jie; Qian, Yanni; Ren, Yuanyuan; Dong, Jiyong

    2015-01-01

    We conducted a prospective study to investigate the abnormalities of the facial nerve canal in patients with congenital aural atresia by computed tomography (CT). Our study population was made up of 99 patients--68 males and 31 females, aged 6 to 22 years (mean: 13.5)--who had unilateral congenital aural atresia without any inner ear malformations. We compared our findings in these patients with those in 50 controls-33 males and 17 females, aged 5 to 22 years (mean: 15.0)-who had normal ears. We classified the congenital aural atresia patients into three groups (A, B, and C) according to their Jahrsdoerfer grading scale score (≥8; 6 or 7; and ≤5, respectively). The course of the facial nerve canal in both the controls and the study patients was determined by temporal bone CT with multiplanar reconstruction. The distances from different parts of the facial nerve canal to surrounding structures were also measured. The course of the facial nerve canal in the normal ears did not vary much, and there were no statistically significant differences according to head side and sex. In groups B and C, the tympanic segment, mastoid segment, and angle of the second genu of the facial nerve canal were all significantly smaller than those of the controls (p < 0.01 in all cases). Statistically, the tympanic segment of the facial nerve canal in patients with congenital aural atresia was downwardly displaced. The mastoid segment of the facial nerve canal in these patients was more anterior than that of the controls. We conclude that congenital aural atresia is often accompanied by abnormalities of the facial nerve canal, especially in the tympanic segment, the mastoid segment, and the second genu. We found that the lower the Jahrsdoerfer score was, the shorter the tympanic segment was and the more forward the mastoid segment was.

  8. DUCTAL STENTING IN PULMONARY ATRESIA NEONATES WITH MULTIPLE CONGENITAL ANOMALIES AND SEPTIC CONDITION

    Directory of Open Access Journals (Sweden)

    Laurentia Utari Wibisono

    2015-10-01

    Full Text Available Neonates with  pulmonary  atresia  usually  appear  normal  at  birth with  pulmonary  circulationmaintained by the presence of a patent ductus arteriosus (PDA. Rapid deterioration will suddenlyocccur  if the duct close. Surgical shunt  is still be used as a standard protocol  in many centers as apalliative procedure. We report a 2 days-old, low birth weight, and mild cyanotic neonate with pulmonaryatresia and PDA accompanied by atresia ani, bladder and cloaca extropy, ambiguous genitalia andsepsis. We decided to perform PDA stenting because our patient have a high surgical shunt risk. Thisprocedure was very  important  to keep  the duct remains open until patient ready  for  total surgicalcorrection. [MEDICINA 2015;46:42-45].Neonatus dengan atresia pulmonal biasanya tampak normal saat lahir dengan adanya patent ductusarteriosus (PDA yang memelihara aliran darah paru. Kondisi neonatus akan segera memburuk jikaduktus menutup. Pembuatan shunt dengan pembedahan merupakan protokol standar yang masihdikerjakan di banyak pusat kesehatan. Kami melaporkan neonatus berusia 2 hari dengan berat badanlahir rendah dan sianosis ringan dengan diagnosis atresia pulmonal, PDA, atresia ani, ekstropi buli-buli dan kloaka, jenis kelamin ambigu, dan sepsis. Kami memutuskan untuk melakukan pemasanganstent pada PDA karena pasien kami memiliki risiko yang tinggi untuk pembedahan (pembuatan shunt.Tindakan ini sangat penting untuk menjaga duktus tetap terbuka sampai pasien siap untuk dilakukanoperasi koreksi. [MEDICINA 2015;46:42-45].

  9. Management of oesophageal atresia in a developing country: Is primary repair forbidden?

    Directory of Open Access Journals (Sweden)

    Samuel Osei-Nketiah

    2016-01-01

    Full Text Available Background: The aim of the study was to evaluate the outcomes of initial surgical procedures for oesophageal atresia at our institution. Primary repair of oesophageal atresia at our centre was perceived to be associated with a high mortality rate. In view of this, almost all patients seen since January 2014 were offered initial surgery for staged repair. Materials and Methods: A retrospective review of records of infants with oesophageal atresia seen at the centre from January 2007 to December 2014 was used in this study. Results: Eighty-five cases of oesophageal atresia were seen over the study period. Of these, 74 (87.1 had surgery performed. Surgical outcome was, however, indicated in 67 of the 74, with overall survival rate of 40.3%. Among the 67 patients, early primary repair was performed in 24 (35.8% with a survival rate of 45.8% (11 patients. Totally, 12 (17.9% of the 67 had initial procedure for delayed primary repair, with a survival rate of 16.7% (2 patients. The remaining 31 (46.3% patients had initial surgery for staged repair, with survival rate of 45.2% (14 patients. There was no association between the type of surgery and the surgical outcome (χ2 = 3.396, df = 2, P = 0.183. Conclusion: The overall surgical survival rate of 40.3% for oesophageal atresia at our institution is low. This study did not show any difference in the survival rate of 45.8% and 45.2% associated with primary repair and staged repair respectively.

  10. 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

    2015-08-28

    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.

  11. Gianturco metallic biliary stent in malignant biliary obstruction: results of follow-up in dead patients

    Energy Technology Data Exchange (ETDEWEB)

    Roh, Byung Suk; Kim, Chan Soo; Lee, Kyung Soo; Choi, See Sung; Won, Jong Jin; Kim, Haak Cheul; Chae, Kwon Mook [Wonkwang University School of Medicine, Iri (Korea, Republic of)

    1994-04-15

    In order to study the patency, restenosis, efficacy, and complication of the metallic stent in the course of treatment of malignant biliary obstruction, the results of follow up of the dead patients after stent insertion were reviewed. Self-expandable Gianturco metallic stent with 10-mm diameter was successfully inserted in 33 patients: 10 with Klatskin tumor, 7 with common bile duct cancer, 7 with gallbladder cancer, 5 with pancreatic cancer, 2 with recurred stomach cancer, one with periampullary cancer, one with hepatocellular carcinoma. The overall duration of survival and patency of the stents in 33 patients were 5.2 months(1-12 months) and 4.9 months(1-14 months), respectively. Restenosis of metallic stents was found in 9 cases(27%), after 6.1 months in average. Causes of stent occlusion were overgrowing of tumor in 5, overgrowing and ingrowing of tumor in 3, extraductal dislodgement in one case. Two cases of symptomatic cholangitis after stent placement were successfully treated with percutaneous cholecystostomy. Three cases of destruction and migration of metallic stents were found after 6 months. On the basis of our experience, insertion of Gianturco metallic biliary stent is an acceptable treatment method in the malignant biliary obstruction, especially for whom short term survival is expected.

  12. Endoscopic management of hilar biliary strictures

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    Hilar biliary strictures are caused by various benign andmalignant conditions. It is difficult to differentiate benignand malignant strictures. Postcholecystectomy benignbiliary strictures are frequently encountered. Endoscopicmanagement of these strictures is challenging.Anendoscopic method has been advocated that involvesplacement of increasing number of stents at regularintervals to resolve the stricture. Malignanthilar stricturesare mostly unresectable at the time of diagnosis and onlypalliation is possible.Endoscopic palliation is preferredover surgery or radiological intervention. Magneticresonance cholangiopancreaticographyis quite importantin the managementof these strictures. Metal stents aresuperior to plastic stents. The opinion is divided over theissue of unilateral or bilateral stenting.Minimal contrastor no contrast technique has been advocated duringendoscopicretrograde cholangiopancreatography ofthese patients. The role of intraluminal brachytherapy,intraductal ablation devices, photodynamic therapy, andendoscopic ultrasound still remains to be defined.

  13. Bile acid signaling and biliary functions

    Directory of Open Access Journals (Sweden)

    Hannah Jones

    2015-03-01

    Full Text Available This review focuses on various components of bile acid signaling in relation to cholangiocytes. Their roles as targets for potential therapies for cholangiopathies are also explored. While many factors are involved in these complex signaling pathways, this review emphasizes the roles of transmembrane G protein coupled receptor (TGR5, farnesoid X receptor (FXR, ursodeoxycholic acid (UDCA and the bicarbonate umbrella. Following a general background on cholangiocytes and bile acids, we will expand the review and include sections that are most recently known (within 5–7 years regarding the field of bile acid signaling and cholangiocyte function. These findings all demonstrate that bile acids influence biliary functions which can, in turn, regulate the cholangiocyte response during pathological events.

  14. Endoscopic therapy of benign biliary strictures

    Institute of Scientific and Technical Information of China (English)

    Joel R Judah; Peter V Draganov

    2007-01-01

    Benign biliary strictures are being increasingly treated with endoscopic techniques. The benign nature of the stricture should be first confirmed in order to ensure appropriate therapy. Surgery has been the traditional treatment, but there is increasing desire for minimally invasive endoscopic therapy. At present, endoscopy has become the first line approach for the therapy of postliver transplant anastomotic strictures and distal (Bismuth Ⅰ and Ⅱ) post-operative strictures. Strictures related to chronic pancreatitis have proven more difficult to treat,and endoscopic therapy is reserved for patients who are not surgical candidates. The preferred endoscopic approach is aggressive treatment with gradual dilation of the stricture and insertion of multiple plastic stents. The use of uncovered self expandable metal stents should be discouraged due to poor long-term results. Treatment with covered metal stents or bioabsorbable stents warrants further evaluation. This area of therapeutic endoscopy provides an ongoing opportunity for fresh research and innovation.

  15. Primary Biliary Cirrhosis and Hemolytic Anemia Confusing Serum Bilirubin Levels

    Directory of Open Access Journals (Sweden)

    M Brackstone

    2000-01-01

    Full Text Available Hemolysis is observed in more than 50% of patients with cirrhosis. However, there has been little documention of the association of primary biliary cirrhosis with autoimmune hemolytic anemia. Two cases, found within a single practice, of primary biliary cirrhosis coexisting with autoimmune hemolysis and a third case coexisting with hereditary spherocytosis are presented. Anemia in such patients is commonly attributed to chronic disease, and hyperbilirubinemia is attributed to primary biliary cirrhosis. These patients were considered for liver transplantation until the diagnosis of a comorbid hemolytic process was established. This association may be more prevalent than previously recognized. A diagnosis of comorbid hemolysis must always be considered in context with anemia and serum bilirubin levels that rise out of proportion to the severity of the primary biliary cirrhosis.

  16. Hepatolithiasis with biliary ascariasis – a case report

    Directory of Open Access Journals (Sweden)

    Khithani AS

    2003-12-01

    Full Text Available Abstract Background Biliary ascariasis is regarded as possible etiological factor for hepatolithiasis. Here we report one case of a patient with hepatolithiasis with biliary ascariasis who developed a liver abscess, which was treated with partial hepatectomy. Case presentation A young adult female presented with epigastric pain and vomiting with repeated attacks of cholangitis. ERCP showed evidence of multiple intrahepatic calculi with the development of abscess in the left lobe of liver. The patient underwent partial hepatectomy and was found to have biliary ascariasis on histology. She was treated with antihelmenthic therapy and has had an uneventful postoperative period of 2 years. Conclusion Biliary ascariasis with hepatolithiasis, although rare, should be considered in endemic countries.

  17. 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.

  18. 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...

  19. Biochemical and radiological predictors of malignant biliary strictures

    Institute of Scientific and Technical Information of China (English)

    Ibrahim A. Al-Mofleh; Abdulrahman M. Aljebreen; Saleh M. Al-Amri; Rashed S. Al-Rashed; Faleh Z. Al-Faleh; Hussein M. Al-Freihi; Ayman A. Abdo; Arthur C. Isnani

    2004-01-01

    AIM: Differentiation of benign biliary strictures (BBS) from malignant biliary strictures (MBS) remains difficult despite improvement in imaging and endoscopic techniques. The aim of this study was to identify the clinical, biochemical and or radiological predictors of malignant biliary strictures.METHODS: We retrospectively reviewed all charts of patients who had biliary strictures (BS) on endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous cholangiography (PTC) in case of unsuccessful ERCP from March 1998 to August 2002. Patient characteristics, clinical features, biochemical, radiological and biopsy results were all recorded. Stricture etiology was determined based on cytology,biopsy or clinical follow-up. A receiver operator characteristic (ROC) curve was constructed to determine the optimal laboratory diagnostic criterion threshold in predicting MBS.RESULTS: One hundred twenty six patients with biliary strictures were enrolled, of which 72 were malignant. The mean age for BBS was 53 years compared to 62.4 years for MBS (P=0.0006). Distal bile duct stricture was mainly due to a malignant process 48.6% vs 9% (P=0.001). Alkaline phosphates and AST levels were more significantly elevated in MBS (P=0.0002). R OC curve showed that a bilirubin level of 84 pmol/L or more was the most predictive of MBS with a sensitivity of 98.6%, specificity of 59.3% and a positive likelihood ratio of 2.42 (95% CI=0.649-0.8L0). Proximal biliary dilatation was more frequently encountered in MBS compared to BBS, 73.8% vs39.5% (P=0.0001). Majority of BBS (87%) and MBS (78%) were managed endoscopically.CONCLUSION: A serum bilirubin level of 84 μmol/L or greater is the best predictor of MBS. Older age, proximal biliary dilatation, higher levels of bilirubin, alkaline phosphatase, ALT and AST are all associated with MBS. ERCP is necessary to diagnose and treat benign and malignant biliary strictures.

  20. Biliary cholesterol secretion: More than a simple ABC

    Institute of Scientific and Technical Information of China (English)

    Arne; Dikkers; Uwe; JF; Tietge

    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 f inal step for the elimination of cholesterol originating from cholesterol-laden macrophage foam cells in the vessel wall in a pathway named reverse cholesterol transport. On the other hand, cholesterol hypersecretion into the bile is considered the main pathophys...

  1. Biliary cholesterol secretion: More than a simple ABC

    OpenAIRE

    Dikkers, Arne; Tietge, Uwe JF

    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 originating from cholesterol-laden macrophage foam cells in the vessel wall in a pathway named reverse cholesterol transport. On the other hand, cholesterol hypersecretion into the bile is considered the ...

  2. Complications of the extrahepatic biliary surgery in companion animals.

    Science.gov (United States)

    Mehler, Stephen J

    2011-09-01

    Surgery of the biliary tract is demanding and is associated with several potentially life-threatening complications. Veterinarians face challenges in obtaining accurate diagnosis of biliary disease, surgical decision-making, surgical hemostasis and bile peritonitis. Intensive perioperative monitoring is required to achieve early recognition of common postoperative complications. Proper treatment and ideally, avoidance of surgical complications can be achieved by gaining a clear understanding physiology, anatomy, and the indications for hepatobiliary surgery.

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

    Science.gov (United States)

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

    2012-08-01

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

  4. On the mechanical behavior of the human biliary system

    Institute of Scientific and Technical Information of China (English)

    Xiaoyu Luo; Wenguang Li; Nigel Bird; Swee Boon Chin; NA Hill; Alan G Johnson

    2007-01-01

    This paper reviews the progress made in understanding the mechanical behaviour of the biliary system.Gallstones and diseases of the biliary tract affect more than 10% of the adult population. The complications of gallstones, i.e. acute pancreatitis and obstructive jandice, can be lethal, and patients with acalculous gallbladder pain often pose diagnostic difficulties and undergo repeated ultrasound scans and oral cholecystograms. Moreover, surgery to remove the gallbladder in these patients, in an attempt to relieve the symptoms, gives variable results. Extensive research has been carried out to understand the physiological and pathological functions of the biliary system, but the mechanism of the pathogenesis of gallstones and pain production still remain poorly understood. It is believed that the mechanical factors play an essential role in the mechanisms of the gallstone formation and biliary diseases. However, despite the extensive literature in clinical studies, only limited work has been carried out to study the biliary system from the mechanical point of view. In this paper, we discuss the state of art knowledge of the fluid dynamics of bile flow in the biliary tract, the solid mechanics of the gallbladder and bile ducts, recent mathematical and numerical modelling of the system,and finally the future challenges in the area.

  5. Biliary excretion of iron and ferritin in idiopathic hemochromatosis

    Energy Technology Data Exchange (ETDEWEB)

    Hultcrantz, R.; Angelin, B.; Bjoern-Rasmussen, E.E.; Ewerth, S.; Einarsson, K.

    1989-06-01

    The role of biliary excretion of iron and ferritin in iron overload was studied and evaluated. Ten patients with idiopathic hemochromatosis and two groups of controls (14 gallstone patients and 16 healthy subjects) were included. Liver tissue (obtained by percutaneous or operative biopsy) was investigated with light microscopy and transmission electron microscopy in combination with x-ray microanalysis. Fasting bile samples were obtained through duodenal aspiration or at cholecystectomy. Iron was determined in liver tissue and bile using atomic absorption spectroscopy, and ferritin was determined in serum and bile with a radioimmunoassay technique. All patients with hemochromatosis had iron-positive staining as seen in light microscopy. Electron microscopy showed iron-containing proteins in the lysosomes and cytosol of liver parenchymal cells, and this observation was supported by x-ray microanalysis. Hepatic iron concentration was increased about eightfold in the patients with hemochromatosis (p less than 0.001). Biliary iron concentration, expressed per millimole of bile acid, was increased about twofold (p less than 0.05) and biliary ferritin concentration about fivefold (p less than 0.001) in hemochromatosis. Four of the patients with hemochromatosis were reexamined after completed treatment with venesection; this resulted in normalized biliary concentrations of iron and ferritin. We conclude that biliary secretion of ferritin occurs in humans and that both iron and ferritin excretion are enhanced in hepatic iron overload. The apparently limited capacity of biliary iron excretion may be of importance for the hepatic iron accumulation in hemochromatosis.

  6. Determination of cholesterol in human biliary calculus by TLC scanning

    Institute of Scientific and Technical Information of China (English)

    Yin Kang Yang; Kai Xiong Qiu; Yu Zhu Zhan; Er Yi Zhan; Hai Ming Yang; Ping Zheng

    2000-01-01

    AIM To study the physico-chemical properties of biliary calculus and the relationship between the calculusformation and the phase change of liquid crystal, providing the best evidence for the biliary calculusprevention and treatment.METHODS The cholesterol contents in thirty one cases of biliary calculus in Kunming were determined bydouble-wave-length TLC scanning with high efficiency silica gel films.RESULTS Under magnifiers, the granular biliary calculus from 31 patients were classified according totheir section structures and colours, as cholesterol cholelith, 25 cases; bilirubin cholelith, 4 cases andcompound cholelith, 2 cases. By TLC scanning, it was found that the content of cholesterol in human biliarycalculus was 71%- 100%, about 80% cholesterol bilestones whose cholesterol content was more than 90%being pure cholesterol bilestones.CONCLUSION Cholesterol bilestone is the main human biliary calculus in Kunming, which was inaccordance with X-ray analysis. Compared with the related reports, it is proved that the proportion ofcholesterol bilestones to biliary calculus is increasing because of the improved life standard and the decreaseof bilirubin bilestones resulted from bile duct ascariasis or bacteria infection in China since 90s, and that theincrease of cholesterol in-take leads to the increase of cholesterol metabolism disorder

  7. 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. PMID:26819608

  8. 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.

  9. Pancreatitis of biliary origin, optimal timing of cholecystectomy (PONCHO trial): study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    Bouwense, S.A.W.; Besselink, M.G.; Brunschot, S. van; Bakker, O.J.; Santvoort, H.C. van; Schepers, N.J.; Boermeester, M.A.; Bollen, T.L.; Bosscha, K.; Brink, M.A.; Bruno, M.J.; Consten, E.C.; Dejong, C.H.; Duijvendijk, P. van; Eijck, C.H. van; Gerritsen, J.J.; Goor, H. van; Heisterkamp, J.; Hingh, I.H.J.T. de; Kruyt, P.M.; Molenaar, I.Q.; Nieuwenhuijs, V.B.; Rosman, C.; Schaapherder, A.F.; Scheepers, J.J.; Spanier, M.B.; Timmer, R.; Weusten, B.L.; Witteman, B.J.; Ramshorst, B. van; Gooszen, H.G.; Boerma, D.; for the Dutch Pancreatitis Study, G.; Verbeek, A.L.

    2012-01-01

    ABSTRACT: BACKGROUND: After an initial attack of biliary pancreatitis, cholecystectomy minimizes the risk of recurrent biliary pancreatitis and other gallstone-related complications. Guidelines advocate performing cholecystectomy within 2 to 4 weeks after discharge for mild biliary pancreatitis. Dur

  10. Pancreatitis of biliary origin, optimal timing of cholecystectomy (PONCHO trial) : Study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    Bouwense, Stefan A.; Besselink, Marc G.; van Brunschot, Sandra; Bakker, Olaf J.; van Santvoort, Hjalmar C.; Schepers, Nicolien J.; Boermeester, Marja A.; Bollen, Thomas L.; Bosscha, Koop; Brink, Menno A.; Bruno, Marco J.; Consten, Esther C.; Dejong, Cornelis H.; van Duijvendijk, Peter; van Eijck, Casper H.; Gerritsen, Jos J.; van Goor, Harry; Heisterkamp, Joos; de Hingh, Ignace H.; Kruyt, Philip M.; Molenaar, I. Quintus; Nieuwenhuijs, Vincent B.; Rosman, Camiel; Schaapherder, Alexander F.; Scheepers, Joris J.; Spanier, Marcel B. W.; Timmer, Robin; Weusten, Bas L.; Witteman, Ben J.; van Ramshorst, Bert; Gooszen, Hein G.; Boerma, Djamila

    2012-01-01

    Background: After an initial attack of biliary pancreatitis, cholecystectomy minimizes the risk of recurrent biliary pancreatitis and other gallstone-related complications. Guidelines advocate performing cholecystectomy within 2 to 4 weeks after discharge for mild biliary pancreatitis. During this w

  11. Pancreatitis of biliary origin, optimal timing of cholecystectomy (PONCHO trial): study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    S.A.W. Bouwense (Stefan); M.G. Besselink (Marc); S. van Brunschot (Sandra); O.J. Bakker (Olaf ); H.C. van Santvoort (Hjalmar); N.J. Schepers (Nicolien); M.A. Boermeester (Marja); T.L. Bollen (Thomas); K. Bosscha (Koop); M.A. Brink (Menno); M.J. Bruno (Marco); E.C. Consten (Esther); C.H. Dejong (Cees); P. van Duijvendijk (Peter); C.H.J. van Eijck (Casper); J.J. Gerritsen (Jos); H. van Goor (Harry); J. Heisterkamp (Joos); I.H.J.T. de Hingh (Ignace); Ph.M. Kruyt (Philip); I.Q. Molenaar (I.Quintus); V.B. Nieuwenhuijs (Vincent); C. Rosman (Camiel); A.F.M. Schaapherder (Alexander); J.J. Scheepers (Joris); B.W.M. Spanier (Marcel); R. Timmer (Robin); B.L. Weusten (Bas); B.J.M. Witteman (Ben); B. van Ramshorst (Bert); H.G. Gooszen (Hein); D. Boerma (Djamila)

    2012-01-01

    textabstractBackground: After an initial attack of biliary pancreatitis, cholecystectomy minimizes the risk of recurrent biliary pancreatitis and other gallstone-related complications. Guidelines advocate performing cholecystectomy within 2 to 4 weeks after discharge for mild biliary pancreatitis. D

  12. Hepato-biliary clinical trials and their inclusion in the Cochrane Hepato-Biliary Group register and reviews

    DEFF Research Database (Denmark)

    Klingenberg, Sarah Louise; Nikolova, Dimitrinka; Alexakis, Nicholas

    2011-01-01

    The Cochrane Hepato-Biliary Group (CHBG) is one of the 52 collaborative review groups within The Cochrane Collaboration. The activities of the CHBG focus on collecting hepato-biliary randomized clinical trials (RCT) and controlled clinical trials (CCT), and including them in systematic reviews...... with meta-analyses of the trials. In this overview, we present the growth of The CHBG Controlled Trials Register, as well as the systematic reviews that have been produced since March 1996....

  13. 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.

  14. Gastric serosal tear due to congenital pyloric atresia: A rare anomaly, a rare complication

    Directory of Open Access Journals (Sweden)

    Mithat Gunaydin

    2011-01-01

    Full Text Available Congenital pyloric atresia (CPA is a very rare malformation with unknown aetiology. It has has numerous complications including gastric perforation, aspiration pneumonia. Gastric perforations in newborns occur by three mechanisms: trauma, ischaemia, or spontaneous. Here, we report a newborn with CPA presenting with gastric serosal tearing without full-cut gastric perforation. The diagnosis was confirmed with the help of plain abdominal radiograph, ultrasound, contrast-study, and at operation. Treatment of CPA is surgery irrespective of the type of atresia. We performed serosa repair and then the solid, cord-like atretic pylorus was excised with accompanying gastroduodenostomy. Our patient had an uneventful course and was discharged at the end of the second postoperative week.

  15. 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.

  16. Biliary leakage after urgent cholecystectomy: Optimizationof endoscopic treatment

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    AIM To investigate the results of endoscopic treatmentof postoperative biliary leakage occurring after urgentcholecystectomy with a long-term follow-up.METHODS: This is an observational database studyconducted in a tertiary care center. All consecutivepatientswho underwent endoscopic retrograde cholangiography(ERC) for presumed postoperative biliaryleakage after urgent cholecystectomy in the periodbetween April 2008 and April 2013 were consideredfor this study. Patients with bile duct transection andbiliary strictures were excluded. Biliary leakage wassuspected in the case of bile appearance from eitherpercutaneous drainage of abdominal collection orabdominal drain placed at the time of cholecystectomy.Procedural and main clinical characteristics of allconsecutive patients with postoperative biliary leakageafter urgent cholecystectomy, such as indication forcholecystectomy, etiology and type of leakage, ERCfindings and post-ERC complications, were collectedfrom our electronic database. All patients in whomthe leakage was successfully treated endoscopicallywere followed-up after they were discharged from thehospital and the main clinical characteristics, laboratorydata and common bile duct diameter were electronicallyrecorded.RESULTS: During a five-year period, biliary leakagewas recognized in 2.2% of patients who underwenturgent cholecystectomy. The median time fromcholecystectomy to ERC was 6 d (interquartile range,4-11 d). Endoscopic interventions to manage biliaryleakage included biliary stent insertion with or withoutbiliary sphincterotomy. In 23 (77%) patients after firstendoscopic treatment bile flow through existing surgicaldrain ceased within 11 d following biliary therapeuticendoscopy (median, 4 d; interquartile range, 2-8 d).In those patients repeat ERC was not performed and the biliary stent was removed on gastroscopy. In seven(23%) patients repeat ERC was done within one tofourth week after their first ERC, depending on theextent

  17. Cystic lung changes in a thin section CT in an asymptomatic young adult with unilateral pulmonary vein atresia: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Seung Choul; Yi, Jeong Geun; Park, Jeong Hee [Konkuk Univ. Medical Center, Seoul (Korea, Republic of)

    2012-07-15

    Unilateral pulmonary vein atresia is a rare anomaly, usually associated with symptoms of recurrent hemoptysis and pneumonia in early childhood. Only one report of an asymptomatic adult patient can be found in the literature. We present the case of an asymptomatic patient with unilateral right pulmonary vein atresia in a 20 year old man. Chest radiograph and multidetector computed tomography showed not only pulmonary vein atresia, pulmonary artery hypoplasia, but also cystic lung changes on thin section CT, along with septal and bronchovascular bundle thickening, and ground-glass opacity. Unilateral pulmonary vein atresia could be another disease which can show cystic lung changes on thin section chest CT.

  18. Efficacy of multiple biliary stenting for refractory benign biliary strictures due to chronic calcifying pancreatitis

    Science.gov (United States)

    Ohyama, Hiroshi; Mikata, Rintaro; Ishihara, Takeshi; Sakai, Yuji; Sugiyama, Harutoshi; Yasui, Shin; Tsuyuguchi, Toshio

    2017-01-01

    AIM To investigate endoscopic therapy efficacy for refractory benign biliary strictures (BBS) with multiple biliary stenting and clarify predictors. METHODS Ten consecutive patients with stones in the pancreatic head and BBS due to chronic pancreatitis who underwent endoscopic therapy were evaluated. Endoscopic insertion of a single stent failed in all patients. We used plastic stents (7F, 8.5F, and 10F) and increased stents at intervals of 2 or 3 mo. Stents were removed approximately 1 year after initial stenting. BBS and common bile duct (CBD) diameter were evaluated using cholangiography. Patients were followed for ≥ 6 mo after therapy, interviewed for cholestasis symptoms, and underwent liver function testing every visit. Patients with complete and incomplete stricture dilations were compared. RESULTS Endoscopic therapy was completed in 8 (80%) patients, whereas 2 (20%) patients could not continue therapy because of severe acute cholangitis and abdominal abscess, respectively. The mean number of stents was 4.1 ± 1.2. In two (20%) patients, BBS did not improve; thus, a biliary stent was inserted. BBS improved in six (60%) patients. CBD diameter improved more significantly in the complete group than in the incomplete group (6.1 ± 1.8 mm vs 13.7 ± 2.2 mm, respectively, P = 0.010). Stricture length was significantly associated with complete stricture dilation (complete group; 20.5 ± 3.0 mm, incomplete group; 29.0 ± 5.1 mm, P = 0.011). Acute cholangitis did not recur during the mean follow-up period of 20.6 ± 7.3 mo. CONCLUSION Sequential endoscopic insertion of multiple stents is effective for refractory BBS caused by chronic calcifying pancreatitis. BBS length calculation can improve patient selection procedure for therapy. PMID:28101303

  19. 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.

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

    Directory of Open Access Journals (Sweden)

    Preeti Tyagi

    2016-01-01

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

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

    OpenAIRE

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

    1985-01-01

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

  2. 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.

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

    Directory of Open Access Journals (Sweden)

    Veena Raja

    2015-09-01

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

  4. 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.

  5. Destruction of the germinal disc region of an immature preovulatory chicken follicle induces atresia and apoptosis.

    Science.gov (United States)

    Yao, H H; Volentine, K K; Bahr, J M

    1998-09-01

    The germinal disc region (GDR), which contains the germinal disc and overlying granulosa cells, is essential for completion of maturation of the preovulatory chicken follicle. The current study was conducted to test the hypothesis that destruction of the GDR (GDRX) of an immature preovulatory chicken follicle blocks ovulation, induces apoptosis, and causes atresia. The GDR of immature preovulatory follicles (F2) were destroyed by freezing with dry ice (3 mm in diameter) 48-50 h before ovulation. As a control for the effect of freezing, a nonGDR portion (a portion of the follicular wall opposite to the GDR relative to the follicular stalk) of other F2 follicles were destroyed (nonGDRX). Treatment of F2 follicles by GDRX caused atresia and blocked ovulation of all treated follicles (6 of 6), whereas none of the nonGDRX follicles (0 of 5) underwent atresia. Treatment of follicles by GDRX induced apoptotic DNA fragmentation (laddering) in theca and granulosa layers obtained from the frozen area and in the theca layer obtained from the follicular wall distal to the frozen area. In contrast, apoptosis was only present in theca and granulosa layers in the frozen area of the nonGDRX follicle. Furthermore, the in situ DNA end-labeling technique demonstrated that in the GDRX follicle 24 h after treatment, cells in the theca interna, endothelial cells in blood vessels of the theca externa, and a few granulosa cells underwent apoptosis. These results indicate that destruction of the GDR of an immature preovulatory follicle causes atresia and apoptosis and blocks ovulation. These novel findings suggest that the GDR maintains development of the chicken preovulatory follicle by producing one or more survival factors. Without the GDR, chicken follicles cannot develop further and they eventually die.

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

    Science.gov (United States)

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

    2016-09-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1983-06-15

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

  8. High-Dose-Rate intraluminal brachytherapy for biliary obstruction by secondary malignant biliary tumors

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Won Sup; Kim, Tae Hyun; Yang, Dae Sik; Choi, Myung Sun; Kim, Chul Yong [College of Medicine, Korea Univ., Seoul (Korea, Republic of)

    2003-03-01

    To analyze the survival period, prognostic factors and complications of patients having under gone high-dose-rate intraluminal brachytherapy (HDR-ILB) as a salvage radiation therapy, while having a catheter, for percutaneous transhepatic biliary drainage (PTBD), inserted due to biliary obstruction caused by a secondary malignant biliary tumor. A retrospective study was performed on 24 patients having undergone HDR-ILB, with PTBD catheter insertion, between December 1992 and August 2001, Their median age was 58.5, ranging from 35 to 82 years. The primary cancer site were the stomach, gallbladder, liver, pancreas and the colon, with 12, 6, 3, 2 and 1 cases, respectively. Eighteen patients were treated with external beam radiation therapy and HDR-ILB, while six were treated with HDR-ILB only. The total external beam, and brachytherapy radiations dose were 30-61.2 and 9-30 Gy, with median doses of 50 and 15 Gy, respectively. Of the 24 patients analyzed, 22 died during the follow-up period, with a median survival of 7.3 months. The 6 and 12 months survival rates were 54.2 (13 patients) and 20.8% (5 patients), respectively. The median survivals for stomach and gallbladder cancers were 7.8 and 10.2 months, respectively. According to the univariate analysis, a significant factor affecting survival of over one year was the total radiation dose (over 50 Gy) (0=0.0200), with all the patients surviving more than one year had been irradiated with more than 50 Gy. The acute side effects during the radiation therapy were managed with conservative treatment. During the follow-up period, 5 patients showed symptoms of cholangitis due to the radiation therapy. An extension to the survival of those patients treated with HDR-ILB is suggested compared to the median historical survival of those patients treated with external biliary drainage. A boost radiation dose could be effectively given, by performing HDR-ILB, which is a prognostic factor. In addition, the acute complications of

  9. Malignant biliary obstruction: treatment with interventional radiology

    Institute of Scientific and Technical Information of China (English)

    翟仁友; 钱晓军; 戴定可; 于平

    2003-01-01

    Objective To evaluate the method of palliative drainage by means of metallic indwelling stents or plastic tubes for patients with malignant biliary obstruction. Methods From January 1995 to Febuary 2001, 243 consecutive patients (161 men and 82 women; aged 26-91 years, mean of 61.3 years) with malignant biliary obstruction were treated with transhepatic placement of metallic stents and/or plastic tubes. Among them, 47 patients had pancreatic carcinoma, 98 cholangiocarcinoma, 28 metastatic carcinoma and 60 hepatic carcinoma. 169 stents of nine types were used in this series. After stenting, 47 patients were treated for local tumors. Procedure- and device-related complications were recorded. Patient survival and stent patency rates were calculated with Kaplan-Meier survival analysis.Results One hundred and three patients underwent successfully stent placement for the first time. Others had their stents installed 1-2 weeks after catheterization. Stents were used in 132 patients. Ninety-five patients were treated with a single stent. Seventeen patients had two stents installed for bilateral drainage,20 patients had two stents installed from top to bottom to create stenting of adequate ength, and 12 patients had stents placed across the ampulla. The 2-month mortality rate was 8.64% (21/243). Major complications occurred in two patients (0.8%, 2/243). Minor complications included self-limited bleeding into the drainage tubes and fever. The average patency of the initial stent was 7.5 months and average survival was 9 months. Thirteen patients received brachytherapy in their stents, 15 extra radiation therapy, and 19 intra-arterial infusion chemotherapy. The 47 patients treated for local tumors had an average survival of 11.3 months (log rank 32.8,P<0.001) with an average patency of 9.7 months(log rank 4.7,P<0.05).Conclusion Percutaneous transhepatic bile drainage as a palliative procedure is well tolerated by patients. After stenting, treatment for local tumor may

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

    Directory of Open Access Journals (Sweden)

    Abdel-Aziz Mosaad

    2012-06-01

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

  11. Vibrant Soundbridge(®) in preschool children with unilateral aural atresia: acceptance and benefit.

    Science.gov (United States)

    Leinung, M; Zaretsky, E; Lange, B P; Hoffmann, V; Stöver, T; Hey, C

    2017-01-01

    The aim of this pilot study was to determine the acceptance and benefit from the middle ear implant system Vibrant Soundbridge(®) (VSB, MED-EL Corporation, Austria) by means of a questionnaire, compared to a previously used conventional bone conducting hearing device, in preschool children with unilateral congenital aural atresia. Prospective cohort study. All nine children with unilateral congenital aural atresia used the VSB and had previous experience with a bone conducting hearing device. The benefit from the VSB was evaluated by questionnaires concerning acceptance of hearing aids, handling, listening effort, behavior, quality of life, and the duration of daily use and compared to the experience with the bone conducting hearing device. In addition, to quantify the benefit from the VSB use, audiological assessment (pure-tone audiometry via free field testing, speech audiometry, and localization test) was performed with and without VSB. The questionnaires and audiological test results were compared pairwise. According to all questionnaire areas, children benefited significantly more from the VSB compared to bone conducting hearing device (ps VSB. Children performed significantly better with the VSB than without it in the audiological assessment. Children with unilateral aural atresia benefited significantly more from the VSB compared to a conventional bone conducting hearing device according to the parents' questionnaires and yielded better results in the audiometry and localization test with the VSB than without it.

  12. A Tutorial on Implantable Hearing Amplification Options for Adults with Unilateral Microtia and Atresia

    Directory of Open Access Journals (Sweden)

    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.

  13. 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.

  14. New successful one-step surgical repair for apple peel atresia

    Directory of Open Access Journals (Sweden)

    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

  15. Nal-IRI With 5-fluorouracil (5-FU) and Leucovorin or Gemcitabine Plus Cisplatin in Advanced Biliary-tract Cancer

    Science.gov (United States)

    2017-02-03

    Adenocarcinoma Metastatic; Biliary Tract Cancer; Adenocarcinoma of the Biliary Tract; Adenocarinoma Locally Advanced; Non-Resectable Hepatocellular Carcinoma; Intrahepatic Bile Duct Carcinoma; Extrahepatic Bile Duct Carcinoma

  16. Geographic Clusters of Primary Biliary Cirrhosis

    Directory of Open Access Journals (Sweden)

    Saif Abu-Mouch

    2003-01-01

    Full Text Available Genetic and environmental factors have been widely suggested to contribute to the pathogenesis of primary biliary cirrhosis (PBC, an autoimmune disease of unknown etiology leading to destruction of small bile ducts. Interestingly, epidemiologic data indicate a variable prevalence of the disease in different geographical areas. The study of clusters of PBC may provide clues as to possible triggers in the induction of immunopathology. We report herein four such unique PBC clusters that suggest the presence of both genetic and environmental factors in the induction of PBC. The first cluster is represented by a family of ten siblings of Palestinian origin that have an extraordinary frequency of PBC (with 5/8 sisters having the disease. Second, we describe the cases of a husband and wife, both having PBC. A family in which PBC was diagnosed in two genetically unrelated individuals, who lived in the same household, represents the third cluster. Fourth, we report a high prevalence of PBC cases in a very small area in Alaska. Although these data are anedoctal, the study of a large number of such clusters may provide a tool to estimate the roles of genetics and environment in the induction of autoimmunity.

  17. Innate immunity and primary biliary cirrhosis.

    Science.gov (United States)

    Selmi, Carlo; Lleo, Ana; Pasini, Simone; Zuin, Massimo; Gershwin, M Eric

    2009-02-01

    There has been a rapid growth in our understanding of the molecular bases of primary biliary cirrhosis (PBC). These efforts were initiated when the immunodominant mitochondrial autoantigen was cloned and sequenced. Using the recombinant cloned antigen as a tool, research has focused on the effector mechanisms of disease and the uniqueness of the primary target tissue, the intrahepatic bile ducts. Most recently, there have been experimental data suggesting that innate immunity changes may be critical to the initiation and perpetuation of the autoimmune injury, as in the case of the enhanced response of monocytes and memory B cells to infectious stimulation and environmental mimics. These observations are important as they help fill in the many gaps which remain on the most difficult subject of autoimmunity, etiology. Indeed, based on the available data, several experimental models of PBC have been developed. These models illustrate and suggest that PBC can be initiated by several mechanisms, all of which lead to loss of tolerance to the mitochondrial antigens. However, once this adaptive response develops, it appears that much of the subsequent pathology is exacerbated by innate responses. We suggest that future therapeutic efforts in PBC will depend heavily on understanding the nature of this innate immune responses and methodology to blunt their cytotoxicity.

  18. Autotaxin, Pruritus and Primary Biliary Cholangitis (PBC).

    Science.gov (United States)

    Sun, Ying; Zhang, Weici; Evans, Jilly F; Floreani, Annarosa; Zou, Zhengsheng; Nishio, Yukiko; Qi, Ruizhao; Leung, Patrick S C; Bowlus, Christopher L; Gershwin, M Eric

    2016-08-01

    Autotaxin (ATX) is a 125-kD type II ectonucleotide pyrophosphatase/phosphodiesterase (ENPP2 or NPP2) originally discovered as an unknown "autocrine motility factor" in human melanoma cells. In addition to its pyrophosphatase/phosphodiesterase activities ATX has lysophospholipase D (lysoPLD) activity, catalyzing the conversion of lysophosphatidylcholine (LPC) into lysophosphatidic acid (LPA). ATX is the only ENPP family member with lysoPLD activity and it produces most of the LPA in circulation. In support of this, ATX heterozygous mice have 50% of normal LPA plasma levels. The ATX-LPA signaling axis plays an important role in both normal physiology and disease pathogenesis and recently has been linked to pruritus in chronic cholestatic liver diseases, including primary biliary cholangitis (PBC). Several lines of evidence have suggested that a circulating puritogen is responsible, but the identification of the molecule has yet to be definitively identified. In contrast, plasma ATX activity is strongly associated with pruritus in PBC, suggesting a targetable molecule for treatment. We review herein the biochemistry of ATX and the rationale for its role in pruritus.

  19. 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.

  20. 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.

  1. Usefulness of a Biliary Manipulation Catheter in Percutaneous Transhepatic Biliary Drainage

    Energy Technology Data Exchange (ETDEWEB)

    Paek, Auh Whan [Dept. of Radiology, Virginia University Heath Center, Charlottesville (United States); Won, Je Hwan; Lee, Jei Hee; Sun, Joo Sung; Kwak, Kyu Sung; Bae, Jae Ik [Dept. of Radiology, Ajou University School of Medicine, Suwon (Korea, Republic of)

    2011-04-15

    To evaluate usefulness of a manipulation catheter in percutaneous transhepatic biliary drainage (PTBD). A biliary manipulation catheter was used for the aspiration of retained bile and lesion crossing during an initial PTBD in 91 consecutive patients over a 6 month period. This catheter allowed for a 0.035 inch guide wire made of 5F short steel braided polyurethane. The terminal 1 cm segment was tapered and 45 degree angulated. Two side holes were made in the terminal segment to facilitate the aspiration of bile. The safety of this procedure was evaluated based on whether the catheters caused complications during insertion and manipulation, and whether cholangitis was aggravated after the procedure. Effectiveness of the procedure was evaluated based on the ability to aspirate retained bile and to cross the lesion. Both the insertion of a 0.035 inch hydrophilic guide wire and aspiration of sufficient retained bile were successful with the catheter. Crossing the common bile duct (CBD) lesion had a 98.1% success rate during the initial PTBD. Crossing the hilar obstruction lesion was had a 94.7% success rate to the CBD and 92.1% to the contralateral lobe. Cholangitis improved in 97% of cases, and aggravated transiently in only 3% of cases after PTBD.

  2. 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.

  3. Cancers of the pancreas and biliary tract: epidemiological considerations.

    Science.gov (United States)

    Fraumeni, J F

    1975-11-01

    The epidemiological patterns for pancreatic and biliary cancers reveal more differences than similarities. Pancreatic carcinoma is common in western countries, although 2 Polynesian groups (New Zealand Maoris and native Hawaiians) have the highest rates internationally. In the United States the disease is rising in frequency, predominating in males and in blacks. The rates are elevated in urban areas, but geographic analysis uncovered no clustering of contiguous counties except in southern Louisiana. The origin of pancreatic cancer is obsure, but a twofold increased risk has been documented for cigarette smokers and diabetic patients. Alcohol, occupational agents, and dietary fat have been suspected, but not proven to be risk factors. Except for the rare hereditary form of pancreatitis, there are few clues to genetic predisposition. In contrast, the reported incidence of biliary tract cancer is highest in Latin American populations and American Indians. The tumor predominates in females around the world, except for Chinese and Japanese who show a male excess. In the United States the rates are higher in whites than blacks, and clusters of high-risk counties have been found in the north central region, the southwest, and Appalachia. The distribution of biliary tumors parallels that of cholesterol gallstones, the major risk factor for biliary cancer. Insights into biliary carcinogenesis depend upon clarification of lithogenic influences, such as pregnancy, obesity, and hyperlipoproteinemia, exogenous estrogens, familial tendencies, and ethnic-geographic factors that may reflect dietary habits. Noncalculous risk factors for biliary cancer include ulcerative colitis, clonorchiasis, Gardner's syndrome, and probably certain industrial exposures. Within the biliary tract, tumors of the gallbladder and bile duct show epidemiological distinctions. In contrast to gallbladder cancer, bile duct neoplasms predominate in males; they are less often associated with stones and more

  4. MR and CT cholangiography in evaluation of the biliary tract

    Energy Technology Data Exchange (ETDEWEB)

    Masui, T.; Fujiwara, T.; Ichijo, K.; Imaoka, I.; Naito, M. [Seirei Mikatabara General Hospital, Hamamatsu (Japan). Dept. of Radiology; Takehara, Y.; Kaneko, M. [Hamamatsu Univ. School of Medicine (Japan). Dept. of Radiology; Yamamoto, H.; Watahiki, H. [Seirei Mikatabara General Hospital, Hamamatsu (Japan). Dept. of Gastroenterology

    1998-09-01

    Objective: To compare MR and CT cholangiography (MRC and CTC) in evaluating the anatomy of the extrahepatic biliary tract and the pathology related to the gallbladder. Material and Methods: Twenty-three patients underwent MRC and CTC with a biliary contrast medium for investigation of biliary disease. 3D displays of both were also obtained. Endoscopic retrograde cholangiography was performed in 17 patients, and the pathology of all 23 was evaluated. Results: Overall, the image quality was higher with CTC than with MRC (4.7 vs 3.9, p<0.05). The cystic duct was demonstrated better by CTC than MRC (p<0.05). Multiplanar reformation (MPR) and source images provided additional information to that obtained from 3D MRC and CTC images. Gallstones were revealed in 6 patients by CTC and in 5 of these 6 by MRC. In 2 patients with cholecystitis, CTC demonstrated gallbladder wall thickening but MRC did not. In 3 patients with adenomyomatosis, MRC demonstrated Rokitansky-Aschoff sinuses (RAS) while CTC demonstrated focal gallbladder wall thickening in all 3 and RAS in 1 of them. Conclusion: Both MRC and CTC provided anatomical and pathological information about the biliary system. With both techniques, however, either MPR or source images proved necessary in addition for evaluating the biliary system anatomy and pathology. The gallbladder wall was depicted clearly in source CTC, but MRC is recommended for the evaluation of adenomyomatosis because its depicts RAS clearly. (orig.)

  5. Contrast-free endoscopic stent insertion in malignant biliary obstruction

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    AIM: To present a case series of MRCP-guided endoscopic biliary stent placement, performed entirely without contrast injection.METHODS: Contrast-free endoscopic biliary drainage was attempted in 20 patients with malignant obstruction,unsuitable for resection on the basis of tumor extent or medical illness. MRCP images were used to confirm the diagnosis of tumor, to exclude other biliary diseases and to demonstrate the stenoses as well as dilation of proximal liver segments. The procedure was carried out under conscious sedation. Patients were placed in the left lateral decubitus position. The endoscope was inserted, the papilla identified and cannulated by a papillotome. A guide wire was inserted and guided deeply into the biliary tree, above the stenosis, by fluoroscopy. A papillotomy approximately 1 cm. long was performed and the papillotome was exchanged with a guiding-catheter. A 10 Fr, Amsterdam-type plastic stent,7 to 15 cm long, was finally inserted over the guide wire/guiding catheter by a pusher tube system.RESULTS: Successful stent insertion was achieved in all patients. There were no major complications. Successful drainage, with substantial reduction in bilirubin levels,was achieved in all patients.CONCLUSION: This new method of contrast-free endoscopic stenting in malignant biliary obstruction is a safe and effective method of palliation. However, a larger, randomized study comparing this new approach with the standard procedure is needed to confirm the findings of the present study.

  6. Placement of removable metal biliary stent in post-orthotopic liver transplantation anastomotic stricture

    Institute of Scientific and Technical Information of China (English)

    Hoi-Poh; Tee; Martin; W; James; Arthur; J; Kaffes

    2010-01-01

    Postoperative biliary strictures are the most common cause of benign biliary stricture in Western countries, secondary to either operative injury or bile duct anastomotic stricture following orthotopic liver transplantation(OLT).Surgery or endoscopic interventions are the mainstay of treatment for benign biliary strictures.We aim to report the outcome of 2 patients with refractory anastomotic biliary stricture post-OLT,who had successful temporary placement of a prototype removable covered self-expandable m...

  7. Biliary ascariasis in a bile duct stones-removed female patient.

    Science.gov (United States)

    Wang, Jun; Pan, Yang-Lin; Xie, Yan; Wu, Kai-Chun; Guo, Xue-Gang

    2013-09-28

    Biliary ascariasis is a common problem in rural areas in China. The common presentations include biliary colic, acute cholangitis, obstructive jaundice, choledocholithiasis and acute cholecystitis. Here, we describe a case with biliary ascariasis two days after endoscopic sphincterotomy for choledocholithiasis. A living ascaris was successfully removed by endoscopic retrograde cholangiopancreatography. This case indicated that biliary ascariasis is not an uncommon complication of endoscopic sphincterotomy in some regions where ascariasis is epidemic.

  8. 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.)

  9. Best options for preoperative biliary drainage in patients with Klatskin tumors.

    Science.gov (United States)

    Jo, Jung Hyun; Chung, Moon Jae; Han, Dai Hoon; Park, Jeong Youp; Bang, Seungmin; Park, Seung Woo; Song, Si Young; Chung, Jae Bock

    2017-01-01

    Operative treatment combined with PBD has been established as a safe management strategy for Klatskin tumors. However, controversy exists regarding the preferred technique for PBD among percutaneous transhepatic biliary drainage (PTBD), endoscopic biliary stenting (EBS), and endoscopic nasobiliary drainage (ENBD). This study aimed to identify the best technique for preoperative biliary drainage (PBD) in Klatskin tumor patients.

  10. Endoscopic ultrasound guided biliary and pancreatic duct interventions

    Institute of Scientific and Technical Information of China (English)

    David; Prichard; Michael; F; Byrne

    2014-01-01

    When endoscopic retrograde cholangio-pancreatog-raphy fails to decompress the pancreatic or biliary system, alternative interventions are required. In this situation, endosonography guided cholangio-pancrea-tography(ESCP), percutaneous radiological therapy or surgery can be considered. Small case series reporting the initial experience with ESCP have been superseded by comprehensive reports of large cohorts. Although these reports are predominantly retrospective, they demonstrate that endoscopic ultrasound(EUS) guided biliary and pancreatic interventions are associated with high levels of technical and clinical success. The proce-dural complication rates are lower than those seen with percutaneous therapy or surgery. This article describes and discusses data published in the last five years relat-ing to EUS-guided biliary and pancreatic intervention.

  11. Primary Biliary Mixed Adenoneuroendocrine Carcinoma (MANEC): A Short Review.

    Science.gov (United States)

    Acosta, Andres M; Wiley, Elizabeth Louise

    2016-10-01

    Mixed adenoneuroendocrine carcinomas (MANECs) are composite neoplasms with areas of adenocarcinoma or squamous cell carcinoma intermingled with neuroendocrine carcinoma or neuroendocrine tumor, each composing at least 30% of the neoplasm. MANECs are very infrequent overall, and they are more commonly diagnosed in the appendix, colon, and stomach. Biliary MANECs are particularly rare, and their histogenesis is debated because neuroendocrine cells are seldom identified in the normal biliary tract. They can show one of the 3 different architectural patterns described in Lewin's original classification: collision tumors, combined lesions, or amphicrine neoplasms. The neuroendocrine component is usually of a high grade, with small or large cell cytomorphology, whereas the adenocarcinoma component is either an intestinal or biliary type. Clinical presentation is characterized by locally advanced disease at the time of initial diagnosis. Recent studies suggest that treatment should be guided by the most aggressive histologic component.

  12. Analysis of the pathomorphology of intra-and extrahepatic biliary system of biliary atresia%胆道闭锁肝内外胆系组织病理形态学分析

    Institute of Scientific and Technical Information of China (English)

    郑珊; 罗义; 王炜; 肖现民

    2007-01-01

    目的 通过胆道闭锁(BA)肝门纤维块、肝脏组织的病理及其超微结构观察,对肝门成纤维细胞分化程度进行评分,并与肝纤维化分级进行相关分析.方法 选取BA患儿作为研究对象,术中取肝门纤维块及肝脏组织标本;研究同期选取疑似BA经术中胆道造影除外BA,诊断为胆汁淤积综合征和先天性胆管扩张症患儿作为对照组,留取肝脏组织标本.在光镜和电镜下观察标本的病理改变,以及肝细胞、毛细胆管和肝门成纤维细胞的超微结构.采用SPSS 140软件,半定量比较BA与对照组肝脏纤维化的差异,检验肝门纤维块成纤维细胞活跃程度与肝纤维化分级的相关性.结果 2005年7月至2006年5月复旦大学附属儿科医院收治的21例BA Kasai根治术病例,手术平均年龄(66±20)d;对照组为5例胆汁淤积综合征和10例先天性胆管扩张症患儿.BA组肝组织病理改变主要是肝内门脉区胆管炎症及纤维化形成,肝纤维化程度明显高于同年龄胆汁淤积综合征和先天性胆管扩张症患儿;肝门纤维块毛细胆管增生,部分管腔闭锁、狭窄,腔内炎细胞浸润及部分淤胆,大量间质成分增生;电镜下肝门成纤维细胞活跃、肝脏毛细胆管上皮微绒毛缺失、肝细胞及肝血窦内电子致密物质增多及部分毛细胆管扩张;肝门成纤维细胞分化程度与肝组织纤维化程度相关(P=0.04).结论 BA肝组织病理改变主要是肝内门脉区胆管炎症及严重纤维化形成;超微结构改变提示肝门部成纤维细胞活跃,其分化程度与肝纤维化程度相关.

  13. 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)....

  14. 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 anomali

  15. Atresia brônquica congênita: relato de dois casos. Contribuição da tomografia computadorizada ao diagnóstico Congenital bronchial atresia: report of two cases. Contribution of CT scan to diagnosis

    Directory of Open Access Journals (Sweden)

    ALECSANDRA CALIL MOYSES FAURE

    2000-06-01

    Full Text Available Atresia brônquica congênita é uma anomalia rara, caracterizada pela presença de broncocele com hiperinsuflação distal. Relatam-se dois casos dessa anomalia e descrevem-se os achados clínicos e radiológicos que suportam o seu diagnóstico sem a necessidade de uso de métodos invasivos ou cirúrgicos.Bronchial atresia is a rare, congenital anomaly characterized by the presence of bronchocele with distal hyperinflation. The authors report two cases of segmental bronchial atresia and describe the clinical and roentgenographic findings supporting the diagnosis in the absence of other invasive diagnostic modalities or surgical exploration.

  16. 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. PMID:28288186

  17. ENDOSCOPIC STENT FOR PALLIATING MALIGNANT AND BENIGN BILIARY OBSTRUCTION

    Institute of Scientific and Technical Information of China (English)

    缪林; 范志宁; 季国忠; 文卫; 蒋国斌; 吴萍; 刘政; 黄光明

    2004-01-01

    Objective: To study the techniques of placement of memory alloy plating gold biliary stent and plastic stent for palliation of malignant and benign biliary obstruction, and to assess its clinical effectiveness. Methods: The patients in plastic stent group included paplilla of duodenum inflamational strictures (n=24), common bile duct inflammational inferior segment strictures (n=4), choledocholithiasis (n=5), bile leak (n=11), bile duct surgery injurey (n=7) and pancreatic carcinoma (n=1). The patients in plating gold stent group included common bile duct carcinoma (n=5) and pancreatic carcinoma (n=6). Under fluoroscopic guidance the stent was inserted into biliary obstruction sites from oral cavity in all cases. Complications, liver function and blood serum amylase were investigated during the study period. Results: Successful stent placement was achieved in all cases. After operation of 7 days, in gold biliary stent groups, the rates of decrease of blood serum total bilirubin, glutamic-pyruvic transaminase, r-glutamyl transpeptidase and alkaline phosphatase were 67.16%, 58.37%, 40.63% and 41.54% respectively. In plastic stent group, the rates of decrease of STB, ALT, r-GT and AKP were 53.24%, 55.03%, 37.15%, 34.12% respectively. Early complication included post-ERCP pancreatitis and cholangititis. Occlusion of stent was the major late complication. Conclusion: Memory alloy plating gold biliary stent and plastic stent were safe and efficacious methods for malignant and benign biliary obstruction, and could improve patient's living quality. Plastic stent was an efficient complement for therapy of bile leak and bile duct injury.

  18. [Congenital broncho-biliary fistula: a case report].

    Science.gov (United States)

    Pérez, Cinthia G; Reusmann, Aixa

    2016-10-01

    Congenital tracheo-or-bronchobiliary fistula or congenital he-patopulmonary fistula is a rare malformation with high morbidity and mortality if the diagnosis is not made early. The tracheo-or-bronchobiliary fistula is a communication between the respiratory (trachea or bronchus) and biliary tract. To date, only 35 cases have been published worldwide. We report a case of a neonate with right pneumonia and bilious fluid in the endotracheal tube. Diagnosis was made using bronchoscopy with fluoroscopy. Videothoracoscopy was used to remove the bronchobiliary fistula. Subsequently, a left he-patectomy with Roux-en-Y biliary-digestive anastomosis was performed as bile ductus hypoplasia was present.

  19. BACTERIOLOGICAL INVESTIGATION ON Fasciola hepatica AND CATTLE BILIARY DUCTS

    Directory of Open Access Journals (Sweden)

    A. Panebianco

    2009-03-01

    Full Text Available The aim of this work was to investigate the occurrence of bacteria in Fasciola hepatica and into cattle biliary ducts containing the parasite. A total of 24 liver and 58 F. hepatica samples were analysed. In all biliary ducts and in 62,06% of parasite Enterobacteriaceae were isolated. The bacterial specie more frequently isolated from parasite were Citrobacter freundii (34%, Proteus mirabilis (18%, Providencia rettgeri (12%, Staphylococcus spp. (18%, Enterobacter spp. (12%. There doesn’t appear to be a correlation between bacterial specie from parasite and ducts. The Authors conclude with some related inspective consideration.

  20. Spontaneous biliary peritonitis in acalculous cholecystitis: fact or misdiagnosis?

    Science.gov (United States)

    Sökmen, S; Coker, A; Unek, T

    2001-01-01

    It is often speculated that an inflamed gallbladder weeps bile to produce bile peritonitis. This may be so, but more likely the problem is a peritoneal effusion in a jaundiced patient which thus resembles bile. So-called "spontaneous or idiopathic biliary peritonitis" in acute acalculous cholecystitis without a proven cause is a further example of this very rare condition. Spontaneous perforations of the extrahepatic biliary ductal system associated with acalculous cholecystitis are uncommon albeit reported in adults. Most patients present with an acute abdomen and are operated upon urgently without diagnostic iter. A recent experience with such a case prompted a thorough review of 27 similar cases previously reported.

  1. [Biliary peritonitis after traumatic rupture of a choledochal cyst].

    Science.gov (United States)

    Bouali, O; Trabanino, C; Abbo, O; Destombes, L; Baunin, C; Galinier, P

    2015-07-01

    Choledochal cysts are rare congenital malformations of the biliary tract. Traumatic rupture of a choledochal cyst can be misleading. An 11-year-old boy was admitted for peritonitis and intestinal occlusion after blunt abdominal trauma, evolving over 48 h. Laparotomy revealed bile ascites and a suspected duodenal perforation. After referral to our center, a CT scan showed a perforated choledochal cyst. Six months later, a complete excision of the cyst was successfully performed. This treatment is mandatory because of the risk of further complications such as lithiasis, pancreatitis, cholangitis, biliary cirrhosis, and malignant transformation (cholangiocarcinoma).

  2. Primary biliary cirrhosis--experience in University Hospital, Kuala Lumpur.

    Science.gov (United States)

    Mohammed, R; Goh, K L; Wong, N W

    1996-03-01

    Primary biliary cirrhosis is an uncommon disease amongst Malaysians. Over a 12-year period, between 1979 and 1991, only seven patients with clinical, biochemical and histologic evidence of primary biliary cirrhosis were identified in University Hospital Kuala Lumpur. All were Chinese females between the ages of 30 to 55 years. The presenting complaint was pruritus in 5 patients. All except one patient was jaundiced when the diagnosis was made. These patients were followed up from 1 to 11 years. Three deaths were reported, one from massive hemetemesis and two from liver failure.

  3. Intraductal radiofrequency ablation for management of malignant biliary obstruction.

    Science.gov (United States)

    Rustagi, Tarun; Jamidar, Priya A

    2014-11-01

    Self-expandable metal stents (SEMS) are the current standard of care for the palliative management of malignant biliary strictures. Recently, endoscopic ablative techniques with direct affect to local tumor have been developed to improve SEMS patency. Several reports have demonstrated the technical feasibility and safety of intraductal radiofrequency ablation (RFA), by both endoscopic and percutaneous approaches, in palliation of malignant strictures of the bile duct. Intraductal RFA has also been used in the treatment of occlusion of both covered and uncovered SEMS occlusion from tumor ingrowth or overgrowth. This article provides a comprehensive review of intraductal RFA in the management of malignant biliary obstruction.

  4. Primary biliary cholangitis associated with warm autoimmune hemolytic anemia.

    Science.gov (United States)

    Gonzalez-Moreno, Emmanuel I; Martinez-Cabriales, Sylvia A; Cruz-Moreno, Miguel A; Borjas-Almaguer, Omar D; Cortez-Hernandez, Carlos A; Bosques-Padilla, Francisco J; Garza, Aldo A; Gonzalez-Gonzalez, Jose A; Garcia-Compean, Diego; Ocampo-Candiani, Jorge; Maldonado-Garza, Hector J

    2016-02-01

    There are many autoimmune diseases associated with primary biliary cholangitis (PBC), known as primary biliary cirrhosis; however, the association between PBC and warm autoimmune hemolytic anemia (wAIHA) has rarely been reported. It is documented that hemolysis is present in over 50% of the patients with chronic liver disease, regardless of the etiologies. Due to the clear and frequent relationship between PBC and many autoimmune diseases, it is reasonable to suppose that wAIHA may be another autoimmune disorder seen in association with PBC. Here we reported a 53-year-old female patient diagnosed with wAIHA associated with PBC.

  5. Mechanisms of Biliary Plastic Stent Occlusion and Efforts at Prevention

    Science.gov (United States)

    Kwon, Chang-Il; Lehman, Glen A.

    2016-01-01

    Biliary stenting via endoscopic retrograde cholangiopancreatography has greatly improved the quality of patient care over the last 30 years. Plastic stent occlusion limits the life span of such stents. Attempts to improve plastic stent patency duration have mostly failed. Metal stents (self-expandable metal stents [SEMSs]) have therefore replaced plastic stents, especially for malignant biliary strictures. SEMS are at least 10 times more expensive than plastic stents. In this focused review, we will discuss basic mechanisms of plastic stent occlusion, along with a systematic summary of previous efforts and related studies to improve stent patency and potential new techniques to overcome existing limitations. PMID:27000422

  6. A case of biliary stones and anastomotic biliary stricture after liver transplant treated with the rendez - vous technique and electrokinetic lithotritor

    Institute of Scientific and Technical Information of China (English)

    Marta Di Pisa; Mario Traina; Roberto Miraglia; Luigi Maruzzelli; Riccardo Volpes; Salvatore Piazza; Angelo Luca; Bruno Gridelli

    2008-01-01

    The paper studies the combined radiologic and endoscopic approach (rendezvous technique) to the treatment of the biliary complications following liver transplant. The "rendez-vous" technique was used with an electrokinetic lithotripter, in the treatment of a biliary anastomotic stricture with multiple biliary stones in a patient who underwent orthotopic liver transplant. In this patient, endoscopic or percutaneous transhepatic management of the biliary complication failed. The combined approach, percutaneous transhepatic and endoscopic treatment (rendez-vous technique) with the use of an electrokinetic lithotritor, was used to solve the biliary stenosis and to remove the stones.Technical success, defined as disappearance of the biliary stenosis and stone removal, was obtained in just one session, which definitively solved the complications.The combined approach of percutaneous transhepatic and endoscopic (rendez-vous technique) treatment, in association with an electrokinetic lithotritor, is a safe and feasible alternative treatment, especially after the failure of endoscopic and/or percutaneous trans-hepatic isolated procedures.

  7. Estudo angiográfico da circulação pulmonar na tetralogia de Fallot com atresia pulmonar Angiographic study of pulmonary circulation in tetralogy of Fallot with pulmonary atresia

    OpenAIRE

    2005-01-01

    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 sistêmi...

  8. 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

  9. 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.

  10. 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.

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

    Science.gov (United States)

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

    2015-01-01

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

  12. Estudo angiográfico da circulação pulmonar na tetralogia de Fallot com atresia pulmonar

    OpenAIRE

    2005-01-01

    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 sistêmi...

  13. Esophageal atresia with tracheoesophageal fistula in a patient with 7q35-36.3 deletion including SHH gene.

    Science.gov (United States)

    Busa, Tiffany; Panait, Nicoleta; Chaumoitre, Kathia; Philip, Nicole; Missirian, Chantal

    2016-10-01

    Terminal 7q deletion is rarely reported in the literature. Holoprosencephaly and sacral dysgenesis are found in association with this deletion, due to haploinsufficiency of SHH and HLBX9 genes respectively. We report on a 2-year-old boy with 7q35-36.3 deletion encompassing SHH identified by oligonucleotide array comparative genomic hybridization. In addition to other frequent features, the patient presented with esophageal atresia and tracheoeosophageal fistula diagnosed at birth. This case, together with two others previously described, one presenting with esophageal atresia, the other with congenital esophageal stenosis, confirms the possible association between congenital esophageal malformations and 7q terminal deletion including SHH.

  14. Primary biliary cirrhosis and ulcerative colitis: A case report and review of literature

    Institute of Scientific and Technical Information of China (English)

    Wen-Bin Xiao; Yu-Lan Liu

    2003-01-01

    AIM: To summarize the characteristics of patients suffered from primary biliary cirrhosis associated with ulcerative colitis.METHODS: To report a new case and review the literature.RESULTS: There were 18 cases (including our case) of primary biliary cirrhosis complicated with ulcerative colitis reported in the literature. Compared with classical primary biliary cirrhosis, the patients were more often males and younger similar. The bowel lesions were usually mild with proctitis predominated. While ulcerative colitis was diagnosed before primary biliary cirrhosis in 13 cases, the presentation of primary biliary cirrhosis was earlier than that of ulcerative colitis in our new case reported here. The prevalence of primary biliary cirrhosis among patients of ulcerative colitis was almost 30 times higher than in general population.CONCLUSION: Association of primary biliary cirrhosis with ulcerative colitis is rare. It should be considered in the differential diagnosis of hepatobiliary disease in patients with ulcerative colitis, and vice versa.

  15. Respiratory Care of Infants and Children with Congenital Tracheo-Oesophageal Fistula and Oesophageal Atresia.

    Science.gov (United States)

    Sadreameli, Sara C; McGrath-Morrow, Sharon A

    2016-01-01

    Despite acute respiratory and chronic respiratory and gastro-intestinal complications, most infants and children with a history of oesophageal atresia / trachea-oesophageal fistula [OA/TOF] can expect to live a fairly normal life. Close multidisciplinary medical and surgical follow-up can identify important co-morbidities whose treatment can improve symptoms and optimize pulmonary and nutritional outcomes. This article will discuss the aetiology, classification, diagnosis and treatment of congenital TOF, with an emphasis on post-surgical respiratory management, recognition of early and late onset complications, and long-term clinical outcomes.

  16. Tratamiento quirúrgico de la atresia de coanas: Caso clínico

    OpenAIRE

    Ágreda M,Beatriz; Urpegui G,Ángel; Vallés V,Héctor

    2013-01-01

    La atresia de coanas es la alteración congénita más frecuente del desarrollo nasal. Puede ser unilateral o bilateral, y según eso, la clínica puede aparecer desde el momento del nacimiento, o bien permanecerá asintomática hasta la edad adulta. En el neonato, si es bilateral, se manifiesta por una disnea inspiratoria y una cianosis cíclica al lactar, que se alivian con el llanto; y en el adulto, la clínica es de insuficiencia respiratoria nasal unilateral, con rinorrea. El tratamiento es quirú...

  17. Current status of preoperative drainage for distal biliary obstruction

    Institute of Scientific and Technical Information of China (English)

    Harutoshi; Sugiyama; Toshio; Tsuyuguchi; Yuji; Sakai; Rintaro; Mikata; Shin; Yasui; Yuto; Watanabe; Dai; Sakamoto; Masato; Nakamura; Reina; Sasaki; Jun-ichi; Senoo; Yuko; Kusakabe; Masahiro; Hayashi; Osamu; Yokosuka

    2015-01-01

    Preoperative biliary drainage(PBD) was developed to improve obstructive jaundice, which affects a number of organs and physiological mechanisms in patients waiting for surgery. However, its role in patients who will undergo pancreaticoduodenectomy for biliary obstruction remains controversial. This article aims to review the current status of the use of preoperative drainage for distal biliary obstruction. Relevant articles published from 1980 to 2015 were identified by searching MEDLINE and Pub Med using the keywords "PBD", "pancreaticoduodenectomy", and "obstructive jaundice". Additional papers were identified by a manual search of the references from key articles. Current studies have demonstrated that PBD should not be routinely performed because of the postoperative complications. PBD should only be considered in carefully selected patients, particularly in cases where surgery had to be delayed. PBD may be needed in patients with severe jaundice, concomitant cholangitis, or severe malnutrition. The optimal method of biliary drainage has yet to be confirmed. PBD should be performed by endoscopic routes rather than by percutaneous routes to avoid metastatic tumor seeding. Endoscopic stenting or nasobiliary drainage can be selected. Although more expensive, the use of metallic stents remains a viable option to achieve effective drainage without cholangitis and reintervention.

  18. 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.

    2012-01-01

    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 blind

  19. Bile acid and immunosuppressive therapy in primary biliary cirrhosis

    NARCIS (Netherlands)

    F.H.J. Wolfhagen (Franciscus)

    1995-01-01

    textabstractPrimary Biliary Cirrhosis (PBC) is a chronic, cholestatic liver disease characterized by non-suppurative destruction of interlobular and septal bile ducts, with subsequent liver damage and eventually development of cirrhosis. The disease is relatively rare with an estimated annual incide

  20. 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.

  1. Fibrolamellar hepatocellular carcinoma with biliary tumor thrombus: an unreported association.

    Science.gov (United States)

    De Gaetano, Anna Maria; Nure, Erida; Grossi, Ugo; Frongillo, Francesco; Russo, Rosellina; Vecchio, Fabio Maria; Lirosi, Maria Carmen; Sganga, Gabriele; Felice, Carla; Bonomo, Lorenzo; Agnes, Salvatore

    2013-10-01

    Fibrolamellar hepatocellular carcinoma (FHCC) is a rare malignant tumor of hepatocyte origin occurring earlier in life than typical hepatocellular carcinoma (HCC). We describe a distinctive case of FHCC with biliary tumor thrombus (BTT) in a 25-year-old Caucasian patient, pointing out the imaging features supported by histopathology.

  2. Effect of pancreatic biliary reflux as a cofactor in cholecystitis.

    Science.gov (United States)

    Amr, Abdel Raouf; Hamdy, Hussam Mohamed; Nasr, Magid Mahmoud; Hedaya, Mohammed Saied; Hassan, Ahmed Mohamed Abdelaziz

    2012-04-01

    This study assessed the effect of pancreatico-biliary reflux (PBR) as co-factor in the process of chronic cholecystitis by measurement of the levels of active pancreatic enzyme amylase in gallbladder bile and serum of patients undergoing cholecystectomy. Pancreatic Amylase levels in bile from the gallbladder and serum were measured during surgery in 68 patients with chronic calcular cholecystitis subjected to elective open or laparoscopic cholecystectomy in the National Hepatology and Tropical Medicine Research Institution and Theodore Bilharz Research Institute. Bile amylase was detected in 64 patients (94.1%) indicating pancreatico-biliary reflux. Biliary amylase level ranged from 20-50 IU/L in 42 patients (61.76%), below 20 IU/l in 14 patients (20.59%), over 50 IU/L in 8 patients (11.76%) and undetectable in two patients. According to gallbladder bile amylase, the incidence of Occult PBR in patients operated upon for chronic calcular cholecystitis was 94.1%. The reason should be clarified by further research and wider scale study. Routinely investigating biliary amylase in every patient having cholecystitis can be a method for early detection of precancerous lesions.

  3. Biliary manometry in choledochal cyst with abnormal choledochopancreatico ductal junction.

    Science.gov (United States)

    Iwai, N; Tokiwa, K; Tsuto, T; Yanagihara, J; Takahashi, T

    1986-10-01

    Intraoperative manometry of the biliary tract and measurement of amylase levels in choledochal cysts were performed in seven patients, aged 14 months to 5 years, with choledochal cysts, in an investigation of the pathophysiology of the biliary tract. An abnormal choledochopancreatico ductal junction was observed in these seven patients by preoperative endoscopic retrograde cholangiopancreaticography (ERCP) or intraoperative cholangiograms. All six patients examined showed a high amylase level in the choledochal cyst (5,450 to 46,500 Somogyi Units). The intraoperative manometry of the biliary tract showed that a remarkable high pressure zone as was found in the area of sphincter of Oddi was not found in the area of abnormal choledochopancreatico ductal junction. The pressure recordings also demonstrated that the sphincter of Oddi pressure in the patient with choledochal cyst was increased by gastrin stimulation. On the contrary, no pressure reaction to gastrin or secretin was found in the area of abnormal choledochopancreatic ductal junction. From these results it seems that free reflux of pancreatic juice into the biliary system occurs, and the reflux stream depends upon the pressure gradient between pancreatic ductal pressure and common bile duct pressure because of the lack of a sphincter function at the choledochopancreatico ductal junction.

  4. Osteopontin Deficiency Alters Biliary Homeostasis and Protects against Gallstone Formation.

    Science.gov (United States)

    Lin, Jing; Shao, Wei-Qing; Chen, Zong-You; Zhu, Wen-Wei; Lu, Lu; Cai, Duan; Qin, Lun-Xiu; Jia, Hu-Liang; Lu, Ming; Chen, Jin-Hong

    2016-08-03

    The precipitation of excess biliary cholesterol as solid crystals is a prerequisite for cholesterol gallstone formation, which occurs due to disturbed biliary homeostasis. Biliary homeostasis is regulated by an elaborate network of genes in hepatocytes. If unmanaged, the cholesterol crystals will aggregate, fuse and form gallstones. We have previously observed that the levels of osteopontin (OPN) in bile and gallbladder were reduced in gallstone patients. However, the role and mechanism for hepatic OPN in cholesterol gallstone formation is undetermined. In this study, we found that the expression of hepatic OPN was increased in gallstone patients compared with gallstone-free counterparts. Then, we observed that OPN-deficient mice were less vulnerable to cholesterol gallstone formation than wild type mice. Further mechanistic studies revealed that this protective effect was associated with alterations of bile composition and was caused by the increased hepatic CYP7A1 expression and the reduced expression of hepatic SHP, ATP8B1, SR-B1 and SREBP-2. Finally, the correlations between the expression of hepatic OPN and the expression of these hepatic genes were validated in gallstone patients. Taken together, our findings reveal that hepatic OPN contributes to cholesterol gallstone formation by regulating biliary metabolism and might be developed as a therapeutic target for gallstone treatments.

  5. Ampullary carcinoma: Effect of preoperative biliary drainage on surgical outcome

    Institute of Scientific and Technical Information of China (English)

    Sheikh Anwar Abdullah; Tarun Gupta; Khairul Azhar Jaafar; Yaw Fui Alexander Chung; London Lucien Peng Jin Ooi; Steven Joseph Mesenas

    2009-01-01

    AIM: To evaluate the influence of preoperative biliary drainage on morbidity and mortality after surgical resection for ampullary carcinoma. METHODS: We analyzed retrospectively data for 82 patients who underwent potentially curative surgery for ampullary carcinoma between September 1993 and July 2007 at the Singapore General Hospital, a tertiary referral hospital. Diagnosis of ampullary carcinoma was confirmed histologically. Thirty-five patients underwent preoperative biliary drainage (PBD group), and 47 were not drained (non-PBD group). The mode of biliary drainage was endoscopic retrograde cholangiopancreatography ( n = 33) or percutaneous biliary drainage ( n = 2). The following parameters were analyzed: wound infection, intra-abdominal abscess, intra-abdominal or gastrointestinal bleeding, septicemia, biliary or pancreatic leakage, pancreatitis, gastroparesis, and re-operation rate. Mortality was assessed at 30 d (hospital mortality) and also longterm. The statistical endpoint of this study was patient survival after surgery. RESULTS: The groups were wel l matched for demographic criteria, clinical presentation and operative characteristics, except for lower hemoglobin in the non- PBD group (10.9 ± 1.6 vs 11.8 ± 1.6 in the PBD group). group (10.9 ± 1.6 vs 11.8 ± 1.6 in the PBD group). Of the parameters assessing postoperative morbidity, incidence of wound infection was significantly less in the PBD than the non-PBD group [1 (2.9%) vs 12 (25.5%)]. However, the rest of the parameters did not differ significantly between the groups, i.e. sepsis [10 (28.6%) vs 14 (29.8%)], intra-abdominal bleeding [1 (2.9%) vs 5 (10.6%)], intra-abdominal abscess [1 (2.9%) vs 8 (17%)], gastrointestinal bleeding [3 (8.6%) vs 5 (10.6%)], pancreatic leakage [2 (5.7%) vs 3 (6.4%)], biliary leakage [2 (5.7%) vs 3 (6.4%)], pancreatitis [2 (5.7%) vs 2 (4.3%)], gastroparesis [6 (17.1%) vs 10 (21.3%)], need for blood transfusion [10 (28.6%) vs 17 (36.2%)] and re-operation rate [1 (2

  6. Importance of early diagnosis of pancreaticobiliary maljunction without biliary dilatation

    Institute of Scientific and Technical Information of China (English)

    Kensuke Takuma; Terumi Kamisawa; Taku Tabata; Seiichi Hara; Sawako Kuruma; Yoshihiko Inaba; Masanao Kurata

    2012-01-01

    AIM:To clarify the strategy for early diagnosis of pancreaticobiliary maljunction (PBM) without biliary dilatation and to pathologically examine gallbladder before cancer develops.METHODS:The anatomy of the union of the pancreatic and bile ducts was assessed by using endoscopic retrograde cholangiopancreatography (ERCP).Patients with a long common channel in which communication between the pancreatic and bile ducts was maintained even during sphincter contraction were diagnosed as having PBM.Of these,patients in which the maximal diameter of the bile duct was less than 10 mm were diagnosed with PBM without biliary dilatation.The process of diagnosing 54 patients with PBM without biliary dilatation was retrospectively investigated.Histopathological analysis of resected gallbladder specimens from 8 patients with PBM without biliary dilatation or cancer was conducted.RESULTS:Thirty-six PBM patients without biliary dilatation were diagnosed with gallbladder cancer after showing clinical symptoms such as abdominal or back pain (n =16) or jaundice (n =12).Radical surgery for gallbladder cancer was only possible in 11 patients (31%)and only 4 patients (11%) survived for 5 years.Eight patients were suspected as having PBM without biliary dilatation from the finding of gallbladder wall thickening on ultrasound and the diagnosis was confirmed by ERCP and/or magnetic resonance cholangiopancreatography (MRCP).The median age of these 8 patients was younger by a decade than PBM patients with gallbladder cancer.All 8 patients underwent prophylactic cholecystectomy and bile duct cancer has not occurred.Wall thickness and mucosal height of the 8 resected gallbladders were significantly greater than controls,and hyperplastic changes,hypertrophic muscular layer,subserosal fibrosis,and adenomyomatosis were detected in 7 (88%),5 (63%),7 (88%) and 5 (63%) patients,respectively.Ki-67 labeling index was high and K-ras mutation was detected in 3 of 6 patients

  7. Endoscopic ultrasound-guided biliary drainage with placement of a fully covered metal stent for malignant biliary obstruction

    Institute of Scientific and Technical Information of China (English)

    Tae Hyeon Kim; Seong Hun Kim; Hyo Jeong Oh; Young Woo Sohn; Seung Ok Lee

    2012-01-01

    AIM:To determine the utility of endoscopic ultrasoundguided biliary drainage (EUS-BD) with a fully covered self-expandable metal stent for managing malignant biliary stricture.METHODS:We collected data from 13 patients who presented with malignant biliary obstruction and underwent EUS-BD with a nitinol fully covered selfexpandable metal stent when endoscopic retrograde cholangiopancreatography (ERCP) fails.EUS-guided choledochoduodenostomy (EUS-CD) and EUS-guided hepaticogastrostomy (EUS-HG) was performed in 9 patients and 4 patients,respectively.RESULTS:The technical and functional success rate was 92.3% (12/13) and 91.7% (11/12),respectively.Using an intrahepatic approach (EUS-HG,n =4),there was mild peritonitis (n =1) and migration of the metal stent to the stomach (n =1).With an extrahepatic approach (EUS-CD,n =10),there was pneumoperitoneum (n =2),migration (n =2),and mild peritonitis (n =1).All patients were managed conservatively with antibiotics.During follow-up (range,1-12 mo),there was re-intervention (4/13 cases,30.7%) necessitated by stent migration (n =2) and stent occlusion (n =2).CONCLUSION:EUS-BD with a nitinol fully covered self-expandable metal stent may be a feasible and effective treatment option in patients with malignant biliary obstruction when ERCP fails.

  8. Balloon dilatation biopsy of the biliary stricture through the percutaneous transhepatic biliary drainage tract: Feasibility and diagnostic accuracy

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Ji Hoon; Ryeom, Hun Kyu; Jang, Yun Jin; Kim, Gab Chul; Cho, Seung Hyun; Song, Jung Hup [Kyungpook National University Hospital, Daegu (Korea, Republic of)

    2016-01-15

    To evaluate the feasibility and diagnostic accuracy of the balloon dilatation biopsy for the biliary stricture through the percutaneous transhepatic biliary drainage (PTBD) tract. The study included 35 patients who underwent balloon dilatation biopsy for the biliary stricture through the PTBD tract. Balloon dilatation was done with a balloon catheter of 10-mm or 12-mm diameter. Soft tissue adherent to the retrieved balloon catheter and soft tissue components separated by gauze filtration of evacuated bile were sampled for histopathologic examination. The results were compared with the final diagnosis which was made by clinical and imaging follow-up for mean 989 days (n = 34) and surgery with histopathologic examination (n = 1). Procedure-related complications and diagnostic accuracy were assessed. Tissues suitable for histopathologic examination were obtained in 31 out of 35 patients (88.6%). In 3 patients, self-limiting hemobilia was noted. No major complication was noted. The sensitivity, specificity, diagnostic accuracy, positive and negative predictive values for diagnosis of malignant stricture were 70.0%, 100%, 90.3%, 100%, and 87.5%, respectively. Balloon dilatation biopsy of the biliary stricture through the PTBD tract is a feasible and accurate diagnostic method. It can be a safe alternative to the endoscopic retrograde cholangiography biopsy or forceps biopsy through the PTBD tract.

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

    Directory of Open Access Journals (Sweden)

    Thaís Gomes Rocha

    2010-05-01

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

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

    Directory of Open Access Journals (Sweden)

    Felipe Purcell de Araújo

    2009-09-01

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

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

    Science.gov (United States)

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

    2011-04-01

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

  12. A pilot investigation of feeding problems in children with esophageal atresia.

    Science.gov (United States)

    Baird, R; Levesque, D; Birnbaum, R; Ramsay, M

    2015-04-01

    While many long-term complications of esophageal atresia (EA) have been well investigated, little is known about feeding difficulties in children after surgical correction of EA and its impact on caregivers. This study investigates the feeding behaviors of children with EA through a validated feeding questionnaire. The Montreal Children's Hospital Feeding Scale (MCH-FS) was filled out by the primary caregiver during patient follow-up visits in the multidisciplinary EA clinic. Demographic information, EA subtype, associated anomalies and outcomes were recorded. Results were compared between groups and to a normative sample. Thirty caregivers have completed the MCH-FS; 26 patients had type C atresia (86.7%). In comparison to controls, 17.5% of EA cases are one standard deviation above the mean feeding difficulty score, while 6.7% (n = 2) cases are greater than two standard deviations above normative values. Typical EA patients (type C who were not born Feeding difficulties of patients with typical EA appear mild. Likely explanations include the use of early protocolized care and intensive multidisciplinary care in follow up. Nonetheless, patients with complicated EA (non-type C) and their caregivers tend to experience significant feeding difficulties. Early targeted care may be required for this patient subset, and additional cases will be investigated to confirm these preliminary findings and explore further risk factors of feeding problem in this cohort.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2014-10-11

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

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

    Science.gov (United States)

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

    2016-07-01

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

  15. Compound heterozygous mutations in TTC7A cause familial multiple intestinal atresias and severe combined immunodeficiency.

    Science.gov (United States)

    Yang, W; Lee, P P W; Thong, M-K; Ramanujam, T M; Shanmugam, A; Koh, M-T; Chan, K-W; Ying, D; Wang, Y; Shen, J J; Yang, J; Lau, Y L

    2015-12-01

    Familial multiple intestinal atresias is an autosomal recessive disease with or without combined immunodeficiency. In the last year, several reports have described mutations in the gene TTC7A as causal to the disease in different populations. However, exact correlation between different genotypes and various phenotypes are not clear. In this study, we report identification of novel compound heterozygous mutations in TTC7A gene in a Malay girl with familial multiple intestinal atresias and severe combined immunodeficiency (MIA-SCID) by whole exome sequencing. We found two mutations in TTC7A: one that destroyed a putative splicing acceptor at the junction of intron 17/exon 18 and one that introduced a stop codon that would truncate the last two amino acids of the encoded protein. Reviewing the recent reports on TTC7A mutations reveals correlation between the position and nature of the mutations with patient survival and clinical manifestations. Examination of public databases also suggests carrier status for healthy individuals, making a case for population screening on this gene, especially in populations with suspected frequent founder mutations.

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

    Energy Technology Data Exchange (ETDEWEB)

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

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

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

    Directory of Open Access Journals (Sweden)

    EM García-González

    2012-01-01

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

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

    Science.gov (United States)

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

    2012-06-01

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

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

    Institute of Scientific and Technical Information of China (English)

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

    2009-01-01

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

  20. Current status of endoscopic biliary drainage for unresectable malignant hilar biliary strictures

    Institute of Scientific and Technical Information of China (English)

    Hironari; Kato; Koichiro; Tsutsumi; Hirofumi; Kawamoto; Hiroyuki; Okada

    2015-01-01

    The management of jaundice and cholangitis is important for improving the prognosis and quality of life of patients with unresectable malignant hilar biliary strictures(UMHBS). In addition, effective chemotherapy, such as a combination of gemcitabine and cisplatin, requires the successful control of jaundice and cholangitis. However, endoscopic drainage for UMHBS is technical demanding, and continuing controversies exist in the selection of the most appropriate devices and techniques for stent deployment. Although metallic stents(MS) are superior to the usual plastic stents in terms of patency, an extensive comparison between MS and "inside stents", which are deployed above the sphincter of Oddi, is necessary. Which techniques are preferred remains as yet unresolved: for instance, whether to use a unilateral or bilateral drainage, or a stent-in-stent or side-by-side method for the deployment of bilateral MS, although a new cell design and thin delivery system for MS allowed us to accomplish successful deployments of bilateral MS. The development of techniques and devices for re-intervention after stent occlusion is also imperative. Further critical investigations of more effective devices and techniques, and increased randomized controlled trials are warranted to resolve these important issues.

  1. Fractalkine and Other Chemokines in Primary Biliary Cirrhosis

    Science.gov (United States)

    Shimoda, Shinji; Selmi, Carlo; Gershwin, M. Eric

    2012-01-01

    Primary biliary cirrhosis (PBC) is characterized by the autoimmune injury of small intrahepatic bile duct. On this basis, it has been suggested that the targeted biliary epithelial cells (BEC) play an active role in the perpetuation of autoimmunity by attracting immune cells via chemokine secretion. To address this issue, we challenged BEC using multiple toll-like receptor (TLR) ligands as well as autologous liver infiltrating mononuclear cells (LMNC) with subsequent measurement of BEC phenotype and chemokine production and LMNC chemotaxis by quantifying specific chemokines, specially CX3CL1 (fractalkine). We submit the hypothesis that BEC are in fact the innocent victims of the autoimmune injury and that the adaptive immune response is critical in PBC. PMID:22235377

  2. Fractalkine and Other Chemokines in Primary Biliary Cirrhosis

    Directory of Open Access Journals (Sweden)

    Shinji Shimoda

    2012-01-01

    Full Text Available Primary biliary cirrhosis (PBC is characterized by the autoimmune injury of small intrahepatic bile duct. On this basis, it has been suggested that the targeted biliary epithelial cells (BEC play an active role in the perpetuation of autoimmunity by attracting immune cells via chemokine secretion. To address this issue, we challenged BEC using multiple toll-like receptor (TLR ligands as well as autologous liver infiltrating mononuclear cells (LMNC with subsequent measurement of BEC phenotype and chemokine production and LMNC chemotaxis by quantifying specific chemokines, specially CX3CL1 (fractalkine. We submit the hypothesis that BEC are in fact the innocent victims of the autoimmune injury and that the adaptive immune response is critical in PBC.

  3. Imaging findings of biliary and nonbiliary complications following laparoscopic surgery

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Jin-Young; Kim, Joo Hee; Lim, Joon Seok; Oh, Young Taik; Kim, Ki Whang [Yonsei University College of Medicine, Department of Diagnostic Radiology, Seoul (Korea); Yonsei University College of Medicine, Research Institute of Radiological Science, Seoul (Korea); Kim, Myeong-Jin [Yonsei University College of Medicine, Department of Diagnostic Radiology, Seoul (Korea); Yonsei University College of Medicine, Brain Korea 21 Project for Medical Science, Seoul (Korea); Yonsei University College of Medicine, Research Institute of Radiological Science, Seoul (Korea); Yonsei University College of Medicine, Institute of Gastroenterology, Seoul (Korea); Park, Mi-Suk [Yonsei University College of Medicine, Department of Diagnostic Radiology, Seoul (Korea)

    2006-09-15

    Laparoscopic techniques are evolving for a wide range of surgical procedures although they were initially confined to cholecystectomy and exploratory laparoscopy. Recently, surgical procedures performed with a laparoscope include splenectomy, adrenalectomy, gastrectomy, and myomectomy. In this article, we review the spectrum of complications and illustrate imaging features of biliary and nonbiliary complications after various laparoscopic surgeries. Biliary complications following laparoscopic cholecystectomy include bile ductal obstruction, bile leak with bile duct injury, dropped stones in the peritoneal cavity, retained CBD stone, and port-site metastasis. Nonbiliary complications are anastomotic leakage after partial gastrectomy, gangrenous cholecystitis after gastrectomy, hematoma at the anastomotic site following gastrectomy, gastric infarction after gastrectomy, port-site metastasis after gastrectomy, hematoma after splenectomy, renal infarction after adrenalectomy, and active bleeding after myomectomy of the uterus. (orig.)

  4. New and emerging treatment options for biliary tract cancer

    Directory of Open Access Journals (Sweden)

    Noel MS

    2013-10-01

    Full Text Available Marcus S Noel, Aram F Hezel James P Wilmot Cancer Center, University of Rochester, Rochester, NY, USA Abstract: Biliary tract cancer (BTC is a group of relatively rare tumors with a poor prognosis. The current standard of care consists of doublet chemotherapy (platinum plus gemcitabine; however, even with cytotoxic therapy, the median overall survival is less than 1 year. The genetic basis of BTC is now more clearly understood, allowing for the investigation of targeted therapy. Combinations of doublet chemotherapy with antiepidermal growth factor receptor agents have provided modest results in Phase II and Phase III setting, and responses with small molecule inhibitors are limited. Moving forward as we continue to characterize the genetic hallmarks of BTC, a stepwise, strategic, and cooperative approach will allow us to make progress when developing new treatments. Keywords: biliary tract cancer, cholangiocarcinoma, genetics, targeted therapy

  5. Primary Biliary Cirrhosis Is a Generalized Autoimmune Epithelitis

    Directory of Open Access Journals (Sweden)

    Jun Gao

    2015-03-01

    Full Text Available Primary biliary cirrhosis (PBC is a chronic progressive autoimmune cholestatic liver disease characterized by highly specific antimitochondrial antibodies (AMAs and the specific immune-mediated injury of small intrahepatic bile ducts. Unique apoptotic feature of biliary epithelial cells (BECs may contribute to apotope presentation to the immune system, causing unique tissue damage in PBC. Perpetuation of inflammation may result in senescence of BECs, contributing to irreversible loss of bile duct. In addition to the classic liver manifestations, focal inflammation and tissue damage are also seen in salivary glands and urinary tract in a significant proportion of PBC patients. These findings provide potent support to the idea that molecular mimicry may be involved in the breakdown of autoimmune tolerance and mucosal immunity may lead to a systematic epithelitis in PBC patients. Thus, PBC is considered a generalized epithelitis in clinical practice.

  6. Analysis of Etiology and Drug Resistance of Biliary Infections

    Institute of Scientific and Technical Information of China (English)

    王欣; 李秋; 邹声泉; 孙自庸; 朱峰

    2004-01-01

    The bile was collected from fro patients with biliary infections, with the bacterium isolated to study the sensitivity of each kind of the bacterium to several antibiotics in common use. Except G- bacterium, we also found some kinds of G+ bacterium in infection bile. G- bacterium were not sensitive to Clindamycin, G+ bacterium were sensitive to Ciprofloxacin. Escherichia coli,Xanthomonas maltophilia, Enterobacter cloacae, Pseudomonas aeruginosa were sensitive to Ampicillin. G+ bacterium were not sensitive to Azactam. Enterococcus faecalis, Enterococcus faecium,Enterobacter cloacae were not sensitive to Ceftazidime. Enterococcus faecalis, Staphylococcus coagulase negative, Staphylococcus epidermidis, Pseudomonas aeruginosa were not sensitive to Ceftriaxone Sodium. We didn't found any bacterium resistance Imipenem. The possibility of the existence of G+ bacterium as well as drug resistance should be considered n patients with biliary infections.The value of susceptibility test should be respected to avoid drug abuse of antibiotics.

  7. Primary biliary cystadenocarcinoma mimicking a complicated hydatid cyst.

    Science.gov (United States)

    Genç, V; Cakmak, A; Akbari, M; Orozakunov, E; Ersöz, S

    2010-01-01

    Cystadenocarcinoma of the liver is a rare neoplasm that originates from hepatobiliary epithelium. Primary location of this tumor is generally intrahepatic and most cases are in the right hepatic lobe. Herein we present a case of intrahepatic cystadenocarcinoma in a 53-year-old man who had been followed up for 8 years as hydatic cyst disease of the liver. He was admitted to our hospital with jaundice and loss of appetite. Ultrasound and computed tomography showed a cystic lesion that looked like type II cyst hidatic. Thereafter magnetic resonance imaging revealed a cystic lesion associated with biliary tree. On diagnostic laparotomy peritoneal infiltrations were observed and pathologic examination revealed a biliary cystadenocarcinoma and peritonitis carcinomatosa was diagnosed. Unfortunately correct diagnosis was extremely late and no curative management was possible.

  8. Laparoscopic T-tube choledochotomy for biliary lithiasis.

    Science.gov (United States)

    Garteiz Martínez, Denzil; Sánchez, Alejandro Weber; López Acosta, María Elena

    2008-01-01

    T-tube choledochotomy has been an established practice in common bile duct exploration for many years. Although bile leaks, biliary peritonitis, and long-term postoperative strictures have been reported and are directly associated with the placement or removal of the T-tube, the severity of these complications may often be underestimated by surgeons. We present the case of a 31-year-old male patient who developed biliary peritonitis and septic shock after removal of a T-tube and illustrate one of the catastrophic events that may follow such procedures. Literature shows that these complications may occur more frequently and have higher morbidity and mortality than other less invasive procedures. This article reviews the advances in laparoscopic and endoscopic techniques, which provide alternative therapeutic approaches to choledocholithiasis and allow the surgeon to avoid having to perform a choledochotomy with T-tube drainage.

  9. Percutaneous transhepatic biliary drainage using large needle: Complications and usefulness

    Energy Technology Data Exchange (ETDEWEB)

    Park, Noh Kyoung; Lim, Hyun Yang; Lee, Ji Yeon; Lee, Kil Jun; Tae, Seok; Shin, Kyoung Ja; Lee, Sang Chun [Seoul Red Cross Hospital, Seoul (Korea, Republic of)

    1994-06-15

    To analyse complications and to review usefulness of large needle(18G) in percutaneous transhepatic biliary drainage(PTBD). 46 patients underwent PTBD in 52 occasions using large needle. 44 of 46 patients had either definite or suspicious malignant biliary obstruction, and most patients were poor in general condition. Complications were classified as acute type if developed within 30 days and as delayed type if developed after 30 days. Acute type was subdivided into severe and mild forms. Severe forms of complications were death(5.8%), septicemia(3.8%), and bile peritonitis(1.9%). There were not different from the rate of complication in PTBD using fine needle but the procedure was much more simple. We PTBD using large needle is a simple and safe procedure for prompt bile decompression.

  10. Reliability of pre- and intraoperative tests for biliary lithiasis

    Energy Technology Data Exchange (ETDEWEB)

    Escallon, A. Jr.; Rosales, W.; Aldrete, J.S.

    1985-05-01

    The records of 242 patients, operated consecutively for biliary lithiasis, were analyzed to determine the reliability of oral cholecystography (OCG), ultrasonography (US), and HIDA in detecting biliary calculi. Preoperative interpretations were correlated to operative findings. OCG obtained in 138 patients was accurate in 92%. US obtained in 150 was correct in 89%. The accuracy of HIDA was 92% in acute and 78% in chronic cholecystitis. Intraoperative cholangiography (IOC) done in 173 patients indicated the need for exploratory choledochotomy in 24; 21 had choledocholithiasis. These observations suggest that OCG and US are very accurate, but not infallible, in detecting cholelithiasis. US should be done first; when doubt persists, the addition of OCG allows the preoperative diagnosis of gallstones in 97% of the cases. HIDA is highly accurate but not infallible in detecting acute calculous cholecystitis. IOC is very reliable in detecting choledocholithiasis; thus, its routine is justifiable.

  11. Sphingosine-1-phosphate and ceramide are associated with health and atresia of bovine ovarian antral follicles.

    Science.gov (United States)

    Hernández-Coronado, C G; Guzmán, A; Espinosa-Cervantes, R; Romano, M C; Verde-Calvo, J R; Rosales-Torres, A M

    2015-02-01

    The follicle destiny towards ovulation or atresia is multi-factorial in nature and involves outcries, paracrine and endocrine factors that promote cell proliferation and survival (development) or unchain apoptosis as part of the atresia process. In several types of cells, sphingosine-1-phospate (S1P) promotes cellular proliferation and survival, whereas ceramide (CER) triggers cell death, and the S1P/CER ratio may determine the fate of the cell. The aim of present study was to quantify S1P and CER concentrations and their ratio in bovine antral follicles of 8 to 17 mm classified as healthy and atretic antral follicles. Follicles were dissected from cow ovaries collected from a local abattoir. The theca cell layer, the granulosa cells and follicular fluid were separated, and 17β-estradiol (E2) and progesterone (P4) concentrations were measured in the follicular fluid by radioimmunoassay. Based on the E2/P4 ratio, the follicles were classified as healthy (2.2±0.3) or atretic (0.2±0.3). In both follicular compartments (granulosa and theca cell layer), sphingolipids were extracted and S1P and CER concentrations were quantified by HPLC (XTerra RP18; 5 µm, 3.0×150 mm column). Results showed that in both follicular compartments, S1P concentrations were higher in healthy antral follicles than in atretic antral follicles (P<0.05). The concentration of CER in the granulosa cells was higher in atretic antral follicles than in healthy antral follicles, but no differences were observed in the theca cell layer. The S1P/CER ratio in both follicular compartments was also higher in healthy antral follicles. Interestingly, in these follicles, there was a 45-fold greater concentration of S1P than CER in the granulosa cells (P<0.05), whereas in the theca cell layer, S1P had only a 14-fold greater concentration than CER when compared with atretic antral follicles. These results suggest that S1P plays a role in follicle health, increasing cellular proliferation and survival. In

  12. Hepatocellular carcinoma (Letter to the editor)

    DEFF Research Database (Denmark)

    Békássy, Albert N.; Garwicz, Stanislaw; Jensen, O.A.

    1994-01-01

    Øjenpatologi, Alagill's syndrome, hepatic carcinoma, biliary atresia, histopathology, liver, child, liver cirrhosis, autopsy, eyes, AFP......Øjenpatologi, Alagill's syndrome, hepatic carcinoma, biliary atresia, histopathology, liver, child, liver cirrhosis, autopsy, eyes, AFP...

  13. Predicting when Biliary Excretion of Parent Drug is a Major Route of Elimination in Humans

    OpenAIRE

    Hosey, Chelsea M.; Broccatelli, Fabio; Benet, Leslie Z.

    2014-01-01

    Biliary excretion is an important route of elimination for many drugs, yet measuring the extent of biliary elimination is difficult, invasive, and variable. Biliary elimination has been quantified for few drugs with a limited number of subjects, who are often diseased patients. An accurate prediction of which drugs or new molecular entities are significantly eliminated in the bile may predict potential drug-drug interactions, pharmacokinetics, and toxicities. The Biopharmaceutics Drug Disposi...

  14. Use of amplatzer vascular plug to treat a biliary cutaneous fistula

    Energy Technology Data Exchange (ETDEWEB)

    Ierardi, Anna Maria; Fontana, Federico; Mangini, Manica; Piacentino, Filippo; Cocozza, Eugenio; Frankowska, Emila; Floridi, Chaiara; Carrafiello, Gianpaolo [Unisubria, Varese (Italy)

    2013-10-15

    Several substances have been used in an attempt to sclerose biliary ducts associated with persistent biliary-cutaneous fistula (BCF). The AMPLATZER Vascular Plug (AVP; AGA Medical, USA) system is a recently developed endovascular occlusion device, introduced as an alternative to permanent embolic materials (metallic coils or acrylic glue), in the occlusion of large and medium-calibre arteries and veins. We report a successful use of the AVP to embolize BCF, developed after the removal of an internal-external biliary drainage.

  15. Biliary cryptococcosis in a child: MR imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Jyoti Das, Chandan; Hari, Smriti [All India Institute of Medical Science, Department of Radiology, New Delhi (India); Shyam Pangtey, Ghan [All India Institute of Medical Science, Department of Medicine, New Delhi (India); Hari, Pankaj [All India Institute of Medical Science, Department of Paediatrics, New Delhi (India); Kumar Das, Anup [All India Institute of Medical Science, Department of Pathology, New Delhi (India)

    2006-08-15

    Cryptococcosis is a systemic mycosis with a worldwide distribution. It frequently occurs in patients who are immunologically compromised or chronically ill. Clinical manifestations are usually confined to the central nervous system, lungs and skin. Involvement of the hepatobiliary system is very rare. We describe the MR imaging appearance of a rare case of disseminated cryptococcosis in an immunocompetent child in whom the clinical presentation was dominated by biliary and lymph nodal involvement. (orig.)

  16. Primary biliary cirrhosis:What do autoantibodies tell us?

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    Primary biliary cirrhosis(PBC) is a chronic,progressive,cholestatic,organ-specific autoimmune disease of unknown etiology.It predominantly affects middle-aged women,and is characterized by autoimmune-mediated destruction of small-and medium-size intrahepatic bile ducts,portal inflammation and progressive scarring,which without proper treatment can ultimately lead to fibrosis and hepatic failure.Serum autoantibodies are crucial tools for differential diagnosis of PBC.While it is currently accepted that antim...

  17. Immunoglobulin G4-related pancreatic and biliary diseases

    OpenAIRE

    Hisham Al-Dhahab; Julia McNabb-Baltar; Said Al-Busafi; Alan N Barkun

    2013-01-01

    BACKGROUND: Autoimmune pancreatitis and autoimmune cholangitis are new clinical entities that are now recognized as the pancreaticobiliary manifestations of immunoglobulin (Ig) G4-related disease.OBJECTIVE: To summarize important clinical aspects of IgG4-related pancreatic and biliary diseases, and to review the role of IgG4 in the diagnosis of autoimmune pancreatitis (AIP) and autoimmune cholangitis (AIC).METHODS: A narrative review was performed using the PubMed database and the following k...

  18. Embryonal rhabdomyosarcoma of the biliary tree mimicking a choledochal cyst

    Directory of Open Access Journals (Sweden)

    Nemade Bhushan

    2007-01-01

    Full Text Available Embryonal rhabdomyosarcoma (ERMS of biliary tree is a rare type of mesenchymal neoplasm diagnosed at surgery or by preoperative liver biopsy. We present a one year eight months old child who mimicked a choledochal cyst and was eventually treated with surgery, chemotherapy with IRS IV protocol and adjuvant postoperative radiotherapy to surgical bed with 6 MV photons to a dose of 5040cGy in 28 fractions.

  19. Biliary stone causing afferent loop syndrome and pancreatitis

    Institute of Scientific and Technical Information of China (English)

    André Roncon Dias; Roberto Iglesias Lopes

    2006-01-01

    We report the case of an 84-year-old female who had a partial gastrectomy with Billroth-Ⅱ anastomosis 24years ago for a benign peptic ulcer who now presented an acute pancreatitis secondary to an afferent loop syndrome. The syndrome was caused by a gallstone that migrated through a cholecystoenteric fistula. This is the first description in the literature of a biliary stone causing afferent loop syndrome.

  20. [Biliary ileus: diagnostic and therapeutic aspects. Report of a case].

    Science.gov (United States)

    Narjis, Y; Chelala, E; Dessily, M; Allé, J-L

    2010-01-01

    We report a rare case of small bowel gallstone obstruction in a patient with cholecystointestinal fistula. Diagnosis of this condition is usually difficult and only achieved at surgery. We review the radiological findings, particularly the CT findings, helpful for diagnosis: ectopic gallstone, biliary gas and fistula. Early preoperative diagnosis could reduce morbidity and mortality. Treatment is surgical with enterolithotomy. There is some controversy over the need to repair the fistula.

  1. Hyperammonemia: A Report of Maternal Biliary Cirrhosis and Neonatal Outcome

    OpenAIRE

    Hussamy, Deana J.; Nelson, David B.; Shivvers, Stephan A.

    2013-01-01

    Although uncommon during pregnancy, cirrhosis results in multiple medical complications impacting both mother and fetus. Previous reports suggest liver dysfunction in pregnancy causes accumulation of neurotoxins within the maternal compartment that increases neonatal morbidity through placental transfer. We present a case of a 36-year-old G2P1 female with history of biliary cirrhosis presenting at 32-weeks' gestation with hepatic congestion progressing to hepatic encephalopathy prompting del...

  2. Acute celiac trunk thrombosis revealed by biliary peritonitis.

    Science.gov (United States)

    Jerraya, H; Sbaï, A; Khalfallah, M; Dziri, C

    2015-11-01

    Acute thrombosis of the celiac trunk is a very uncommon condition, which is a life-threatening emergency. The clinical presentation is highly variable depending on the extent of the ischemic territory. We report a case of biliary peritonitis related to an acute thrombosis of the celiac trunk. This case highlights the role of abdominal computed tomography in the diagnosis of acute upper abdominal pain.

  3. Biliary-colonic fistula caused by cholecystectomy bile duct injury

    Institute of Scientific and Technical Information of China (English)

    Francisco Igor B Macedo; Victor J Casillas; James S Davis; Joe U Levi and Danny Sleeman

    2013-01-01

    Biliary-colonic  fistula  is  a  rare  complication after laparoscopic cholecystectomy. We present a case of post-cholecystectomy  iatrogenic  biliary  injury  that  resulted  in  a fistula  between  the  common  hepatic  duct  and  large  bowel. Magnetic  resonance  cholangiopancreatography  provided good  visualization  of  injury  even  with  concurrent  normal level of alkaline phosphatase. Radiologic findings and surgical management of this condition are discussed in detail.

  4. Clinical Significance of Autoantibodies in Primary Biliary Cirrhosis

    OpenAIRE

    Nakamura, Minoru

    2014-01-01

    Antimitochondrial, anti-gp210, anti-sp100, and anticentromere antibodies are specifically detected in primary biliary cirrhosis (PBC). In clinical practice, they are useful for the diagnosis of PBC or for evaluating disease severity, clinical phenotype, and long-term outcome. In the typical or classical form of PBC which shows slow progressive loss of small bile ducts with a parallel increase in liver fibrosis, anti-gp210 antibodies are a strong risk factor for progression to jaundice and hep...

  5. Sex hormones in postmenopausal women with primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Becker, U; Almdal, T; Christensen, E;

    1991-01-01

    To evaluate serum sex hormone profiles in nonalcoholic postmenopausal women with liver disease, 25 women with primary biliary cirrhosis (11 in cirrhotic stage) and 46 healthy controls were studied. The patients had significantly (p less than 0.05) elevated serum concentrations of estrone and andr......To evaluate serum sex hormone profiles in nonalcoholic postmenopausal women with liver disease, 25 women with primary biliary cirrhosis (11 in cirrhotic stage) and 46 healthy controls were studied. The patients had significantly (p less than 0.05) elevated serum concentrations of estrone...... and androstenedione and significantly (p less than 0.05) lower concentrations of estrone sulfate, dehydroepiandrosterone sulfate and 5 alpha-dihydrotestosterone compared with the 46 controls. Serum concentrations of sex hormone binding globulin, testosterone, non-sex hormone binding globulin-bound testosterone...... and non-protein-bound testosterone did not differ significantly (p greater than 0.05) between primary biliary cirrhosis patients and controls. Patients in the cirrhotic stage had significantly (p less than 0.05) higher concentrations of sex hormone binding globulin than did controls. Patients...

  6. Pathogenesis of primary biliary cirrhosis: A unifying model

    Institute of Scientific and Technical Information of China (English)

    Elias Kouroumalis; George Notas

    2006-01-01

    Primary biliary cirrhosis (PBC) is a disease of unknown etiology leading to progressive destruction of small intrahepatic bile ducts and eventually to liver cirrhosis and failure. It is characterised by female predominance and serum auto-antibodies to mitochondrial antigens targeting the E2 components of the 2-oxoacid dehydrogenase complex. Although they are associated with disease pathogenesis, no concrete evidence has been presented so far. Epidemiological data indicate that a geographical clustering of cases and possible environmental factors are implicated in pathogenesis. A number of genetic factors play a role in determining disease susceptibility or progression, although no definitive conclusion has been reached so far. A key factor to immune pathogenesis is considered to be the breakdown of immune tolerance, either through molecular mimicry or through the so called determinant density model. Tn this review, the available data regarding the pathogenesis of primary biliary cirrhosis are described and discussed. A new unifying hypothesis based on early endothelin overproduction in primary biliary cirrhosis (PBC) is presented and discussed.

  7. Radiological findings in biliary fistula and gallstone ileus

    Energy Technology Data Exchange (ETDEWEB)

    Oikarinen, H. [Univ. Hospital, Oulu (Finland). Dept. of Diagnostic Radiology; Paeivaensalo, M. [Univ. Hospital, Oulu (Finland). Dept. of Diagnostic Radiology; Tikkakoski, T. [Univ. Hospital, Oulu (Finland). Dept. of Diagnostic Radiology; Saarela, A. [Univ. Hospital, Oulu (Finland). Dept. of Surgery

    1996-11-01

    Purpose: Biliary fistual and gallstone ileus are rarely found. The diagnosis is difficult and may be delayed until operation. We reviewed the radiological findings in a retrospective material. Material and Methods: The cases of 16 patients treated for biliary fistula were analyzed with respect to findings at imaging. Ten patients had a spontaneous fistula. Nine of them had an internal bilioduodenal fistula and one had an external fistula with stones passing through a subcutaneous abscess. Five patients also had gallstone ileus and one patient a rare gastric outlet obstruction caused by a gallstone (Bouveret`s syndrome). Six patients had an iatrogenic fistula. One of them had internal bile ascites and 5 an external fistula, one of which was a biliocystic fistula resulting from attempted hepatic cyst sclerotherapy. Results: Various imaging modalities were used and there was often a delay in the diagnosis. Imaging did not show the fistula itself in any of the spontaneous cases. However, a nonvisualized or shrunken gallbladder seen at US often coexisted in these cases. CT yielded the diagnosis in one case of gallstone ileus, and a Gastrografin metal yielded it in the case of Bouveret`s syndrome. Fistulography and cholangiography provided a correct diagnosis of fistula in all cases of iatrogenic biliocutaneous fistulas. Conclusion: Patients with biliary fistula usually undergo examinations with nonspecific results. The imaging findings could be more specific if the possibility of this diagnosis were remembered. (orig.).

  8. [Biliary ileus--a rare complication of cholecystolithiasis].

    Science.gov (United States)

    Lakyová, L; Radonak, J; Vajó, J; Toncr, I; Kudlác, M

    2007-10-01

    The diagnosis of ileus caused by biliary stones occurs very rarely, with the range of 2 % worldwide. This complication of cholecystolithiasis caused by the stone fistulation into gastrointestinal tract and its subsequent obstruction occurs mostly in elderly and has a high mortality rate. During the course of ten years (1996-2006) in the 2nd surgical clinic FNLP in Kosice, 1640 cholecystectomies and 255 operations, due to the obstruction ileus, were performed. Biliary ileus was recorded in four cases. In two cases, the reason being an obstruction caused by a travelled stone into jejunum, one event was caused by a mechanic wedge of a stone in duodenum and in the last event rectosigma was obturated. The diagnostic is relatively difficult because of a nonspecific symptomatology and often negative anamnesis of previous problems with gallstones. According to literature, the most reliable diagnostic method is computer tomography (CT). In our case, abdominal ultrasonography was successful, which pointed out this diagnosis. Gastroscopy localized the place of obstruction, but not its cause and X-ray image showed aerobilia two times. By the use of magnetic resonance cholangiopancreatography (MRCP) the diagnosis was not positively confirmed. Only in one out of four cases, there was a suspicion of the diagnosis of the biliary ileus, which makes its detectability 25%. The aim of this retrospective analysis is the comparison of diagnostic method-options and their range of success in the diagnosis of acute abdomen in our clinic and the entries in the world literature.

  9. EXTRAHEPATIC BILIARY DUCTAL VARIATIONS AND ITS CLINICAL SIGNIFICANCE

    Directory of Open Access Journals (Sweden)

    Lakshmidevi

    2016-04-01

    Full Text Available BACKGROUND Incidence of liver and Gallbladder diseases are significantly increasing in recent years. Cholecystectomy either by laparoscopic or open is the commonest surgical procedure performed by General surgeon. Liver Transplantation is emerging as one of the top most transplantation surgery as well. Variations in anatomy of Gallbladder, bile ducts and the arteries that supply them and liver are important to the surgeon, because failure to recognize them may lead to inadvertent ductal ligation, biliary leaks, haemobilia, haemorrhage and strictures after cholecystectomy and may complicate Liver transplantation surgeries. Recognition of these anomalies and normal variants may avoid diagnostic errors, aid in surgical planning and prevent inadvertent ductal injury. OBJECTIVES To study the variations in Extrahepatic Biliary Ductal system with its clinical significance. RESULTS AND CONCLUSION Extrahepatic biliary ductal system studying under various methods are established; many variations in this region and understanding of these variations is undoubtedly important for operating surgeons. Starting from open cholecystectomy or laparoscopic cholecystectomy to cadaveric liver harvesting to recent advances like “Living donor liver transplantation with duct-toduct anastomosis” (LDLT, grading of tumours like hilar cholangiocarcinoma requires definitive knowledge of the anatomy of the ductal and arterial system. Hence, we believe this study is not only confined to anatomists, but definitely be a useful guideline for general and laparoscopic surgeons, oncosurgeons and to transplant surgeons.

  10. Cholesterol crystal binding of biliary immuno globulin A: visualization by fluorescence light microscopy

    Institute of Scientific and Technical Information of China (English)

    Frank Lammert; Stefan Sudfetd; Norbert Busch; Siegfried Matern

    2001-01-01

    AIM To assess potential contributions of biliary IgA for crystal agglomeration into gallstones, we visualized cholesterol crystal binding of biliary IgA.METHODS Crystal-binding biliary proteins were extracted from human gallbladder bile using lectin affinity chromatography. Biliary IgA was isolated from the bound protein fraction by immunoaffinity chromatography. Pure cholesterol monohydrate crystals were incubated with biliary IgA and fluoresceine isothiocyanate (FITC)-conjugated anti-lgA at 37C. Samples were examined under polarizing and fluorescence light microscopy with digital image processing.RESULTS Binding of biliary IgA to cholesterol monohydrate crystals could be visualized with FITC-conjugated anti-lgA antibodies. Peak fluorescence occurred at crystal edges and dislocations. Controls without biliary IgA or with biliary IgG showed no significant fluorescence.CONCLUSION Fluorescence light microscopy provided evidence for cholesterol crystal binding of biliary IgA. Cholesterol crystalbinding proteins like IgA might be important mediators of crystal agglomeration and growth of cholesterol gallstones by modifying the evolving crystal structures in vivo.

  11. Diagnosis and treatment of biliary sludge, cast and stone following liver transplantation

    Institute of Scientific and Technical Information of China (English)

    Lianbao Kong; Xuehao Wang; Fen Zhang

    2006-01-01

    Objective: To investigate etiological facts and treatment of biliary sludge, cast and stone following orthotopic liver transplantation(OLT). Methods: A review was made using data collected from 81 cases with OLTs performed in our center from February 2003 to January 2004, and confirmed by retrospective study. Etiological factors of biliary sludge, cast and stone following OLT were analyzed, and treatment of biliary sludge, cast and stone following OLT were discussed. Result: Nine cases of biliary sludge, cast and stone were diagnosed and the incidence rate was 11.1%. Of these, five were biliary sludge and cast, 2 were bile stone and 2 were necrotic debris. Two cases with hepatic artery embolism received retransplantation and survived. The other one with hepatic artery embolism was ameliorated with nasobiliary drainage by ERCP. Two cases with biliary sludge and cast were resolved by non-operative treatment. Four cases were reoperated, 2 resolved and 2 cases died. Conclusion: Biliary injury and ischemia reperfusion injury, reject reaction, infection and changes of bile kinetics are the important factors causing biliary sludge, cast and stone following OLT. Shortening the time of cold and heat ischemia reperfusion injury of liver, reducing the injury of the blood supply of donor bile duct, actively preventing and early treating of infection and rejection reaction might reduce the incidence rate of biliary sludge, cast and stone following OLT.

  12. MINIINVASIVE METHODS IN THE TREATMENT OF BILIARY COMPLICATIONS AFTER LIVER TRANSPLANTATION

    Directory of Open Access Journals (Sweden)

    A. Y. Popov

    2011-01-01

    Full Text Available Complications of the biliary tract like bile leaks, strictures are an important cause of morbidity and mortality after liver transplantation. The frequency of such complications ranges from 5 to 25%. In most cases, biliary complications can be treated by endoscopy and/or methods of interventional radiology. This article is about the clinical case of liver transplantation with duct-to-duct biliary reconstruction, complicated by bile leaks, biliary tract stricture. Surgical correction of complications was ineffective. We used miniinvasive methods and got po- sitive clinical outcome after radiology intervention for prosthetics of the common bile duct. 

  13. First successful repair of an aortico-to-right ventricular tunnel (ARVT) in d-transposition of the great arteries with aortic valve atresia and ventricular septal defect.

    Science.gov (United States)

    Wagner, Robert; Vollroth, Marcel; Daehnert, Ingo; Kostelka, Martin

    2015-04-01

    The presented case reports on the first successful complex biventricular repair in a neonate with an aortico-to-right ventricular tunnel and dextrotransposition of the great arteries complicated by aortic atresia.

  14. Methoxychlor induces atresia of antral follicles in ERalpha-overexpressing mice.

    Science.gov (United States)

    Tomic, Dragana; Frech, Maria Silvina; Babus, Janice K; Gupta, Rupesh K; Furth, Priscilla A; Koos, Robert D; Flaws, Jodi A

    2006-09-01

    Methoxychlor (MXC) is a pesticide that is known to bind to estrogen receptor alpha (ERalpha) and to induce atresia of antral ovarian follicles. Although studies have shown that MXC is toxic to the ovary, we hypothesize that perturbation to the estrogen-signaling system (i.e., increase or decrease in estrogen sensitivity) might alter ovarian responsiveness to MXC. Thus, we examined whether ERalpha overexpression alters the ability of MXC to increase follicle atresia. To do so, we employed a transgenic mouse model in which ERalpha can be inducibly overexpressed in animal tissues (ERalpha overexpressors). We dosed female controls and ERalpha overexpressors with sesame oil (vehicle control) or MXC (32 and 64 mg/kg/day) for 20 days. After dosing, the ovaries were collected for histological evaluation of follicle numbers and follicle atresia, while blood was collected for measurements of hormones. Estrous cycles were determined in all animals to ensure that all were terminated during estrus. Although there were no significant effects of MXC on the numbers of primordial, primary, and preantral follicles in both controls and ERalpha overexpressors, there was an effect on antral follicles. Specifically, our data indicate that 32 and 64 mg/kg MXC increased the percentage of atretic follicles compared to vehicle in both control and ERalpha overexpressor groups. Moreover, there was a clear trend toward greater sensitivity to 64 mg/kg MXC in ERalpha-overexpressing mice compared to control animals. Specifically, at the 64-mg/kg MXC dose, ERalpha-overexpressing mice had a significantly higher percentage of atretic follicles compared to control animals (controls = 21.5 +/- 3%, n = 5; ERalpha overexpressors = 37 +/- 23%, n = 9, p < or = 0.05 vs. controls). After 20 days of dosing, there were no differences in estradiol levels between controls and ERalpha-overexpressing mice in all treatment groups. Follicle-stimulating hormone (FSH) levels were similar in sesame oil-treated control

  15. Horizontal ventricular septum with dextroversion: hearts with and without aortic atresia.

    Science.gov (United States)

    Thilenius, O G; Bharati, S; Lev, M; Karp, R B; Arcilla, R A

    1987-01-01

    Two hearts with horizontal ventricular septum, dextroversion (situs solitus), ventricular septal defects, and malaligned great vessels are reported. One of the hearts had aortic atresia and the infant died; the other patient had a Fontan-type physiologic correction. Reviewing the literature, the following conclusions are drawn: (a) Hearts with horizontal ventricular septum and those with criss-cross atrioventricular connections may be the result of different degrees of rotation of the ventricular muscle mass. This rotation is not likely to be postseptational but preseptational. (b) Only those hearts with a complete 180 degrees rotation should be called criss-cross hearts. (c) Partial rotation results in a horizontal septum such that the right ventricle is invariably superior, regardless of atrioventricular concordance or discordance, situs solitus or inversus, or dextroversion. (d) Physiologic surgical correction is often possible but has to be tailored to the details of each heart.

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

    Directory of Open Access Journals (Sweden)

    Shalini Koppisetty

    2015-01-01

    Full Text Available Inferior vena cava atresia (IVCA is a rare but well described vascular anomaly. It is a rare risk factor for deep venous thrombosis (DVT, found in approximately 5% of cases of unprovoked lower extremity (LE DVT in patients <30 years of age. Affected population is in the early thirties, predominantly male, often with a history of major physical exertion and presents with extensive or bilateral DVTs. Patients with IVC anomalies usually develop compensatory circulation through the collateral veins with enlarged azygous/hemizygous veins. Despite the compensatory circulation, the venous drainage of the lower limbs is often insufficient leading to venous stasis and thrombosis. We describe a case of extensive and bilateral deep venous thrombosis following physical exertion in a thirty-six-year-old male patient with incidental finding of IVCA on imaging.

  17. External auditory canal atresia of probable congenital origin in a dog.

    Science.gov (United States)

    Schmidt, K; Piaia, T; Bertolini, G; De Lorenzi, D

    2007-04-01

    A nine-month-old Labrador retriever was referred to the Clinica Veterinaria Privata San Marco because of frequent headshaking and downward turning of the right ear. Clinical examination revealed that there was no external acoustic meatus in the right ear. Computed tomography confirmed that the vertical part of the right auditory canal ended blindly, providing a diagnosis of external auditory canal atresia. Cytological examination and culture of fluid from the canal and the bulla revealed only aseptic cerumen; for this reason, it was assumed that the dog was probably affected by a congenital developmental deformity of the external auditory canal. Reconstructive surgery was performed using a "pull-through" technique. Four months after surgery the cosmetic and functional results were satisfactory.

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

    Directory of Open Access Journals (Sweden)

    Muhammad SAEED*

    2014-12-01

    Full Text Available How to Cite This Article: Saeed M, Haq A, Qadir Kh.Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia. Iran J Child Neurol. 2014 Autumn;8(4: 76-79.AbstractObjectiveBart’s syndrome is defined as congenital localized absence of skin, and associated with epidermolysis bullosa. A newborn with Bart’s syndrome is reported because it is a very rare condition, especially when associated with corpus callosum agenesis and concomitant choanal atresia. Clinically it is characterized by raw beefy areas of denuded skin mainly on hands and feet.We report a rare case of a term female newborn born to non-consanguineous parents who presented with congenital absence of skin in, face, trunk and extremities. To the best of our knowledge, this is the first report presenting a case of Bart’s syndrome associated with corpus callosum agenesis.ReferencesBart BJ, Garlin RJ, Anderson VE, Lynch FW. Congenital localized absence of skin and associated abnormalities resembling epidermolysis bullosa. A new syndrome. Arch Dermatol 1966; 93: 296-304.Bart BJ. Epidermolysis bullosa and congenital localized absence of skin. Arch Dermatol 1970; 101: 78-81.Skoven I, Drzewiecki KT. Congenital localized skin defect and epidermolysis bullosa hereditaria letalis. Acta Derm Venereol 1979; 59: 533-537.Wojnarowska FT, Eady RA, Wells RS. Dystrophic epidermolysis bullosa presenting with congenital localized absence of skin: report of four cases. Br J Dermatol 1983; 108: 477-483.Kanzler MH, Smoller B, Woodley DT. Congenital localized absence of the skin as a manifestation of epidermolysis bullosa. Arch Dermatol 1992; 128:1087-90.Maman E, Maor E, Kachko L, Carmi R. Epidermolysis bullosa, pyloric atresia, aplasia cutis congenita: histopathological delineation of an autosomal recessive disease. Am J Med Genet 1998; 78: 127-133.McCarthy MA, Clarke T, Powell FC. Epidermolysis bullosa and aplasia cutis. Int J Derm 1991; 30: 481-484.Puvabanditsin S, Garrow E, Daeun K

  19. Pulmonary Atresia with Intact Ventricular Septum and Absence of Tricuspid Valve. A Report of Four Cases

    Directory of Open Access Journals (Sweden)

    Adel E. González Morejón

    2013-12-01

    Full Text Available Pulmonary atresia with intact ventricular septum suggests a simple congenital heart malformation. However, nothing could be further from the truth. Among the morphological peculiarities of this condition, the presence of congenitally unguarded tricuspid valve orifice is rarely observed. The present paper aims at describing a series of four patients with both malformations detected in the William Soler Children’s Heart Center from 1986 through 2012. Echocardiographic diagnosis could be accurately performed in the last two patients; identification of the initial cases was established by the findings at necropsy. All patients underwent Blalock – Taussig shunt as initial palliative treatment, which was associated with balloon atrioseptostomy in two cases. Only one of the last children mentioned survived. Vital prognosis of patients suffering from this association of malformations lies in early echocardiographic detection.

  20. Long-term pulmonary function in esophageal atresia-A case-control study

    DEFF Research Database (Denmark)

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

    2017-01-01

    BACKGROUND: Esophageal atresia (EA) is a congenital anomaly associated with substantial pulmonary morbidity throughout childhood. AIM: The aim of this study was to evaluate pulmonary complications among 59 five to 15-year-old children and adolescents with surgically corrected congenital EA. METHODS......: Participants underwent a structured interview, spirometry, body plethysmography, mannitol challenge test, skin prick test, as well as measurements of the diffusion capacity, airway resistance, fraction of exhaled NO, and specific immunoglobulin E in serum. A control group consisted of 25 children being.......0001). Fifteen patients (28.8%) with EA had obstructive ventilatory impairment, compared to nine patients (17.3%) with restrictive ventilatory impairment, while one had a combination. CONCLUSIONS: The present study demonstrated significantly decreased pulmonary characteristics in EA patients. Restrictive...

  1. Trps1 regulates biliary epithelial-mesenchymal transition and has roles during biliary fibrosis in liver grafts: a preliminary study.

    Directory of Open Access Journals (Sweden)

    Cheng Zhe

    Full Text Available To investigate the role(s of Trps1 in non-anastomotic biliary stricture (NABS following liver transplantation.Immunohistochemical and histological techniques were used to detect Trps1, E-cadherin, CK19, vimentin, α-SMA, and collagen deposition. Human intrahepatic biliary epithelial cells (HIBECs were infected with a Trps1 adenovirus, or transfected with Trps1 short-interfering RNAs (siRNAs. Reverse transcription polymerase chain reaction (RT-PCR assays and western blotting were used to determine expression levels of epithelial and mesenchymal markers, and Trps1 in HIBECs.Expression of Trps1 and epithelial markers was down-regulated or absent in NABS liver samples. Mesenchymal markers were seen in biliary epithelial cells (BECs, with collagen deposited around the bile duct. Trps1 expression positively correlated with epithelial markers. Expression of epithelial marker mRNAs and proteins in HIBECs decreased with prolonged cold preservation (CP, while mesenchymal marker expression increased. A 12-h CP period led to increased Trps1 mRNA and protein levels. Expression of E-cadherin was increased in HIBECs following Trps1 adenovirus infection and CP/reperfusion injury (CPRI, with vimentin expression levels reduced and CPRI-mediated epithelial-mesenchymal transition (EMT inhibited. Transfection of HIBECs with Trps1 siRNAs in conjunction with CPRI revealed that E-cadherin expression was decreased, vimentin expression was increased, and CPRI-mediated EMT was promoted.Trps1 is involved in NABS pathogenesis following liver transplantation and negatively correlates with BEC EMT and biliary fibrosis in liver grafts. Trps1 demonstrates antagonistic effects that could reverse EMT.

  2. Transcatheter pulmonary valve perforation using chronic total occlusion wire in pulmonary atresia with intact ventricular septum

    Science.gov (United States)

    Bakhru, Shweta; Marathe, Shilpa; Saxena, Manish; Verma, Sudeep; Saileela, Rajan; Dash, Tapan K; Koneti, Nageswara Rao

    2017-01-01

    Background: Perforation of pulmonary valve using radiofrequency ablation in pulmonary atresia with intact ventricular septum (PA IVS) is a treatment of choice. However, significant cost of the equipment limits its utility, especially in the developing economies. Objective: To assess the feasibility, safety, and efficacy of perforation of pulmonary valve using chronic total occlusion (CTO) wires in patients with PA IVS as an alternative to radiofrequency ablation. Methods: This is a single-center, nonrandomized, retrospective study conducted during June 2008 to September 2015. Twenty-four patients with PA IVS were selected for the procedure during the study period. The median age and weight of the study population were 8. days and 2.65 kg, respectively. Four patients were excluded after right ventricular angiogram as they showed right ventricular-dependent coronary circulation. The pulmonary valve perforation was attempted using various types of CTO wires based on the tip load with variable penetrating characteristics. Results: The procedure was successful in 16 of twenty patients using CTO wires: Shinobi in nine, Miracle in four, CROSS-IT in two, and Conquest Pro in one. Two patients had perforation of right ventricular outflow tract (RVOT). Pericardiocentesis was required in one patient to relieve cardiac tamponade. Later, the same patient underwent successful hybrid pulmonary valvotomy. The other patient underwent ductus arteriosus (DA) stenting. Balloon atrial septostomy was needed in three cases with systemic venous congestion. Desaturation was persistent in five cases necessitating DA or RVOT stenting to augment pulmonary blood flow. There were two early and two late deaths. The mean follow-up was 22.66 ± 16 months. Three patients underwent one and half ventricle repair and one Blalock–Taussig shunt during follow-up. Conclusion: Perforation of the pulmonary valve can be done successfully using CTO wires in selected cases of pulmonary atresia with intact

  3. [Prognostic factors related to mortality of children with atresia of bile ducts].

    Science.gov (United States)

    Monroy-Teniza, Zuhy Arlette; Flores-Calderón, Judith; Villasís-Keever, Miguel Ángel

    2015-01-01

    Introducción: la atresia de vías biliares (AVB) es el resultado final de un proceso destructivo, idiopático e inflamatorio que afecta los conductos biliares intra y extrahepáticos, dando lugar a fibrosis y progresivamente a cirrosis biliar. El objetivo fue identificar los factores relacionados con la mortalidad en niños con AVB. Métodos: estudio observacional, longitudinal, analítico y retrospectivo aplicado a pacientes con diagnóstico de atresia de vías biliares atendidos entre los años 2008 y 2012 en hospital de tercer nivel. Resultados: se incluyeron un total de 66 pacientes con AVB; 49 (74.2 %) fueron niñas. Solo se realizó operación de Kasai a 47, la edad al momento del envío fue de 4.5 meses. Al comparar los grupos con y sin Kasai, resultaron estadísticamente significativas la edad menor al momento del envío y la edad del diagnóstico, así como un menor puntaje en el PELD score y en el CHILD-PUGH para el grupo con Kasai. Los pacientes que fallecieron tuvieron una calificación de PELD estadísticamente mayor (mediana 20) que los que vivieron (mediana 13), p = 0.004. El factor relacionado directamente con la mortalidad, fue el antecedente de cirugía de Kasai con una RM de 0.17 (IC 95 %: 0.04-0.71; p = 0.016). Conclusiones: el pronóstico de los niños con AVB continúa siendo sombrío, dado que se diagnostican en etapas tardías. El factor más importante relacionado con la mortalidad en estos pacientes es la realización de cirugía de Kasai.

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

    Directory of Open Access Journals (Sweden)

    H Davari

    2006-01-01

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

  5. Pulmonary Atresia

    Science.gov (United States)

    ... Kawasaki Disease Long Q-T Syndrome Marfan Syndrome Metabolic Syndrome Mitral Valve Prolapse Myocardial Bridge Myocarditis Obstructive Sleep Apnea Pericarditis Peripheral Vascular Disease Rheumatic Fever Sick Sinus Syndrome Silent Ischemia Stroke Sudden ...

  6. Tricuspid Atresia

    Science.gov (United States)

    ... congenital heart defect, including: A mother who had German measles (rubella) or another viral illness during early ... a family member or friend along, if possible. Learning that your baby has a serious heart condition ...

  7. Clinical Evaluation of ERCP and Naobiliary Drainage for Biliary Fungal Infection--A Report of Five Cases of Severe Combined Bacterial and Fungal Infection of Biliary Tract

    Institute of Scientific and Technical Information of China (English)

    ZHAO Qiu; LIAO Jiazhi; QIN Hua; WANG Jialong

    2005-01-01

    This study studied the use of ERCP and nasobiliary tube in the diagnosis of fungal infection of biliary tract and the efficacy of combined use of local administration via nasobiliary tube and intravenous antifungal treatment for severe biliary tract fungal infection. 5 patients in our series,with age ranging from 47 to 68 y (mean 55.8), were diagnosed as having mixed bacterial and fungal infection of biliary tract as confirmed by smear or/and culture of bile obtained by ERCP and nasobiliary drainage. Besides routine anti-bacteria therapy, all patients received local application of fluconazole through nasobiliary tube and intravenous administration of fluconazole or itraconazole in terms of the results of in vitro sensitivity test. The mean duration of intravenous fluconazole or itraconazole was 30 days (24-40 days), and that of local application of fluconazole through nasobiliary drainage tube was 19 days (8-24 days). During a follow-up period of 3-42 months, all patient's fungal infection of biliary tract was cured. It is concluded that on the basis of typical clinical features of biliary tract infection, fungal detection of smear/culture of bile obtained by ERCP was the key for the diagnosis of fungal infection of biliary tract. Local application antifungal drug combined with intravenous anti-fungal drugs might be an effective and safe treatment for fungal infection of biliary tract.

  8. Operação de Fontan-Kreutzer em anomalias cardíacas complexas outras que não atresia tricúspide lb, ventrículo único e atresia pulmonar com septo ventricular íntegro The operation of Fontan-Kreutzer in complex congenital anomalies other than lb isolated tricuspid atresia, single ventricle and pulmonary atresia with intact septum

    Directory of Open Access Journals (Sweden)

    Miguel Barbero-Marcial

    1988-08-01

    Full Text Available Oitenta e quatro pacientes foram submetidos a operação tipo Fontan, no Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, entre 1979 e 1989; as idades variaram entre 5 meses e 31 anos. Estes pacientes foram colocados em 5 grupos anatômicos: 1 atresia tricúspide la, 3 pacientes (1 óbito, 33,3%; 2 atresia tricúspide lb, 48 pacientes (5 óbitos, 10,4%; 3 ventrículo único: 18 pacientes (2 óbitos, 11,1%; 4 atresia pulmonar com septo interventricular íntegro, 5 pacientes (1 óbito, 20,0%; 5 anomalias complexas, 10 pacientes (zero óbitos. O grupo das anomalias complexas foi dividido em 2 subgrupos: pacientes com situs solitus e pacientes com situs inversus. A maioria destes pacientes foi submetida a procedimentos adicionais: em 5 pacientes, uma das valvas A-V foi fechada; em 1 paciente, foram realizadas valvoplastia mitral e septação do átrio único; em 1 paciente, foi realizada a secção de feixe anômalo (feixe de Kent. A evolução tardia (6 meses a 8 anos revelou que 9 pacientes encontram-se em classe funcional I e 1 na classe funcional II (NYHA. Baseados nos resultados obtidos, concluímos que a operação de Fontan modificada é uma alternativa válida em anomalias cianóticas complexas.Between 1979 and 1989, 84 patients underwent a Fontan operation or one of its modifications; the ages ranged from 5 months to 31 years. Patients were placed into five anatomic groups: 1 tricuspid atresia la (3 cases, 1 death, 33.3%; 2 tricuspid atresia lb (48 cases, 5 deaths, 10.4%; 3 single ventricle (18 cases, 2 deaths, 11.1%; 4 pulmonary atresia, intact ventricular septum (5 cases, 1 death, 20.0%; 5 complex anomalies (10 cases, no deaths. The subgroup of complex anomalies was divided in: with situs inversus and with situs solitus. In the majority of these patients, additional procedures were performed; in 5, one A-V valve was closed; in 2, complex atrial septation was done; in 1, a mitral

  9. Influence of curcumin on cyclosporin-induced reduction of biliary bilirubin and cholesterol excretion and on biliary excretion of cyclosporin and its metabolites.

    Science.gov (United States)

    Deters, M; Siegers, C; Hänsel, W; Schneider, K P; Hennighausen, G

    2000-06-01

    We investigated the ability of curcumin, which can be extracted from different Curcuma species, to prevent cyclosporin-induced reduction of biliary bilirubin and cholesterol excretion, and its influence on biliary excretion of cyclosporin (CS) and its metabolites in the bile fistula model in rats. I.v. injection of curcumin (25 and 50 mg/kg) after 30 min increased dose-dependently basal bile flow (30 microliters/kg/min) up to 200%, biliary bilirubin excretion (3000 pmol/kg/min) up to 150%, and biliary cholesterol excretion (22 nmol/kg/min) up to 113%. CS (30 mg/kg) reduced bile flow to 66% and biliary excretion of bilirubin and of cholesterol to 33% of the basal value 30 min after i.v. injection. I.v. administration of curcumin (25 and 50 mg/kg) 30 min after CS increased bile flow dose dependently again to 130% for 1 hour and biliary excretion of cholesterol and of bilirubin to 100% of the basal value for 30 and 150 min, respectively. Injection of curcumin 15 min before CS prevented the CS-induced drop of bile flow at 50 mg/kg and reduction of biliary bilirubin excretion already at 25 mg/kg until the end of the experiment (180 min). The CS-induced reduction of biliary cholesterol excretion, however, was not prevented by curcumin. Finally, the biliary excretions of CS (1200 ng/kg/min) and its metabolites (1200 ng/kg/min) were slightly reduced by curcumin at a dose of 50 mg/kg (to 83% of the initial values). The clinical importance of these controversial effects remains to be shown.

  10. The origin of biliary ductular cells that appear in the spleen after transplantation of hepatocytes.

    Science.gov (United States)

    Fukuda, Kenji; Sugihara, Ayako; Nakasho, Keiji; Tsujimura, Tohru; Yamada, Naoko; Okaya, Atsuhito; Sakagami, Masafumi; Terada, Nobuyuki

    2004-01-01

    Transplantation of rat hepatocytes into the syngeneic rat spleen results in the appearance of cytokeration (CK)-19-positive biliary cells that form ductules. The exact origin of CK-19-positive cells is not known and the possibility that they are derived from biliary cells or precursors of oval cells in transplanted hepatocyte preparations has been raised. In the present study, we found that the number of CK-19-positive biliary cells increased rapidly after transplantation of hepatocytes, reached the maximum at 4 weeks, and then gradually decreased. However, a Ki-67 labeling index of CK-19-positive biliary cells was low and showed no significant changes throughout the experimental period. In addition, no or few CK-19-positive cells appeared in the spleen after transplantation of nonparenchymal liver cells enriched with biliary cells. These results showed that biliary cells were not the source of CK-19-positive cells in the spleen. Impairment of precursors of oval cells in the liver by administration of 4,4'-diaminodiphenylmethane 24 h before transplantation of hepatocytes did not prevent the appearance of CK-19-positive biliary cells in the spleen. Moreover, transplantation of nonparenchymal cells carrying an increased number of oval cells by means of treatment with 2-acetylaminofluorene and partial hepatectomy resulted in no appearance of CK-19-positive biliary cells in the spleen. These results ruled out oval cells as the origin of CK-19-positive biliary cells in the spleen. Because CK-19-positive biliary cells appeared in the spleen only when hepatocyte fractions were transplanted, we suggest transdifferentiation of heptocytes may be the mechanism by which CK-19-positive biliary cells are generated.

  11. Single-operator cholangioscopy in patients requiring evaluation of bile duct disease or therapy of biliary stones (with videos)

    DEFF Research Database (Denmark)

    Chen, Yang K; Parsi, Mansour A; Binmoeller, Kenneth F;

    2011-01-01

    The feasibility of single-operator cholangioscopy (SOC) for biliary diagnostic and therapeutic procedures was previously reported.......The feasibility of single-operator cholangioscopy (SOC) for biliary diagnostic and therapeutic procedures was previously reported....

  12. Síndrome de Treacher Collins com atresia coanal: relato de caso e revisão de suas características Treacher Collins syndrome with choanal atresia: a case report and review of disease features

    Directory of Open Access Journals (Sweden)

    Eduardo C. Andrade

    2005-02-01

    Full Text Available A Síndrome de Treacher Collins ou disostose mandibulofacial apresenta-se com deformidades crânio-faciais, tendo expressão e severidade variável. É uma malformação congênita que envolve o primeiro e segundo arcos branquiais. A Síndrome de Treacher Collins é rara e sua incidência está estimada em uma faixa de 1:40000 a 1:70000 nascidos vivos. Esta síndrome é caracterizada por anormalidades dos pavilhões auriculares, hipoplasia dos ossos da face, obliqüidade antimongolóide das fendas palpebrais com coloboma palpebral inferior e fissura palatina. A Síndrome de Treacher Collins raramente está associada com atresia coanal. Estes pacientes são apropriadamente acompanhados por uma equipe multidisciplinar que inclui cirurgiões crânio-faciais, oftalmologistas, fonoaudiologistas, cirurgiões dentistas e otorrinolaringologistas. Relatamos neste artigo um caso raro de Síndrome de Treacher Collins com atresia coanal, uma revisão da patologia e intervenção multidisciplinar.Treacher Collins Syndrome - or mandibulofacial dysostosis - is a rare condition that presents several craniofacial deformities of different levels. This is a congenital malformation involving the first and second branchial arches. Incidence is estimated to range between 1-40,000 to 1-70,000 of live births. The disorder is characterized by abnormalities of the auricular pinna, hypoplasia of facial bones, antimongoloid slanting palpebral fissures with coloboma of the lower eyelids and cleft palate. Treacher Collins Syndrome is rarely associated with choanal atresia. A multidisciplinary team, including craniofacial surgeon, ophthalmologist, speech therapist, dental surgeon and otorhinolaryngologist, is the most appropriate setting to manage these patients. This study reports a rare case of Treacher Collins Syndrome with choanal atresia, presenting literature review and multidisciplinary intervention.

  13. Primary biliary cirrhosis-specific autoantibodies in first degree relatives of Greek primary biliary cirrhosis patients

    Institute of Scientific and Technical Information of China (English)

    Theodoros A Zografos; Nikolaos Gatselis; Kalliopi Zachou; Christos Liaskos; Stella Gabeta; George K Koukoulis; George N Dalekos

    2012-01-01

    AIM:To determine the prevalence and significance of primary biliary cirrhosis (PBC)-specific autoantibodies in first-degree relatives (FDRs) of Greek PBC patients.METHODS:The presence of antimitochondrial antibodies (AMA) and PBC-specific antinuclear antibodies (ANA) were determined using indirect immunofluorescence assays,dot-blot assays,and molecularly based enzyme-linked immunosorbent assays in 101 asymptomatic for liver-related symptoms FDRs of 44 PBC patients.In order to specify our results,the same investigation was performed in 40 healthy controls and in a disease control group consisting of 40 asy.mptomatic for liver-related symptoms FDRs of patients with other autoimmune liver diseases namely,autoimmune hepatitis-1 or primary sclerosing cholangitJs (AIH-1/PSC).RESULTS:AMA positivity was observed in 19 (only 4 with abnormal liver function tests) FDRs of PBC patients and none of the healthy controls.The prevalence of AMA was significantly higher in FDRs of PBC patients than in AIH-1/PSC FDRs and healthy controls [18.8%,95% confidence interval (CI):12%-28.1% vs 2.5%,95% CI:0.1%-14.7%,P =0.01; 18.8%,95% CI:12%-28.1% vs 0%,95% CI:0%-10.9%,P =0.003,respectively].PBC-specific ANA positivity was observed in only one FDR from a PSC patient.Multivariate analysis showed that having a proband with PBC independently associated with AMA positivity (odds ratio:11.24,95% CI:1.27-25.34,P =0.03) whereas among the investigated comorbidities and risk factors,a positive past history for urinary tract infections (UTI)was also independently associated with AMA detection in FDRs of PBC patients (odds ratio:3.92,95% CI:1.25-12.35,P =0.02).CONCLUSION:In FDRs of Greek PBC patients,AMA prevalence is significantly increased and independently associated with past UTI.PBC-specific ANA were not detected in anyone of PBC FDRs.

  14. Partial trisomy 4q and partial monosomy 9p in a girl with choanal atresia and various dysmorphic findings.

    Science.gov (United States)

    Cakmak-Genc, Gunes; Karakas-Celik, Sevim; Dursun, Ahmet; Piskin, İbrahim Etem

    2015-09-01

    We report a new-born girl with partial trisomy of 4q28-qter and partial monosomy of 9p24-9ter. Our patient has choanal atresia, hypertelorism, wide nasal bridge, high arched palate, discrete nipples, heart defects, myoclonic seizures and various dysmorphic findings. Standard chromosomal analysis with G-banding with Trypsin-Giemsa revealed 46,XX,der(9)t(4;9)(q28;p24) resulting from the mother's t(4,9) (q28;p24) karyotype. Deletions of the terminal part of 9p and partial trisomy of chromosome 4q are rare chromosomal alterations. To our knowledge, this is the first report of choanal atresia in a patient with a partial trisomy of 4q28-qter and partial monosomy 9p24-9ter combination, which were detected by integrated cytogenetic and genomic analysis.

  15. Gaseous distention of the hypopharynx and cervical esophagus with nasal CPAP: a mimicker of pharyngeal perforation and esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Walor, David; Berdon, Walter; Holt, Peter D.; Fox, Matthew [Columbia University Medical Center, Department of Radiology, New York, NY (United States); Children' s Hospital of New York, New York, NY (United States); Anderson, Nicole [Columbia University Medical Center, Department of Neonatology, New York, NY (United States); Children' s Hospital of New York, New York, NY (United States)

    2005-12-01

    Nasal continuous positive airway pressure (CPAP) has been used since 1975 as the initial treatment for respiratory distress syndrome (RDS) in very premature infants. Gaseous distention of the abdomen (CPAP belly) is a common secondary effect of CPAP. Gaseous distention of the hypopharynx is also common. To determine the incidence of hypopharyngeal distention in infants on CPAP. We performed a retrospective review of the chest radiographs of 57 premature infants treated with CPAP during a 4-week period to find the presence and degree of hypopharyngeal distention. Of the 57 radiographs, 14 (25%) revealed gaseous distention of the hypopharynx and/or cervical esophagus. On occasion, this raised concern for pharyngeal perforation or esophageal atresia. Awareness that CPAP-related hypopharyngeal distention is common should help radiologists avoid erroneous consideration of esophageal atresia or hypopharyngeal perforation. (orig.)

  16. Biliary stenting: indications, choice of stents and results: European Society of Gastrointestinal Endoscopy (ESGE) clinical guideline.

    Science.gov (United States)

    Dumonceau, J-M; Tringali, A; Blero, D; Devière, J; Laugiers, R; Heresbach, D; Costamagna, G

    2012-03-01

    This article is part of a combined publication that expresses the current view of the European Society of Gastrointestinal Endoscopy about endoscopic biliary stenting. The present Clinical Guideline describes short-term and long-term results of biliary stenting depending on indications and stent models; it makes recommendations on when, how, and with which stent to perform biliary drainage in most common clinical settings, including in patients with a potentially resectable malignant biliary obstruction and in those who require palliative drainage of common bile duct or hilar strictures. Treatment of benign conditions (strictures related to chronic pancreatitis, liver transplantation, or cholecystectomy, and leaks and failed biliary stone extraction) and management of complications (including stent revision) are also discussed. A two-page executive summary of evidence statements and recommendations is provided. A separate Technology Review describes the models of biliary stents available and the stenting techniques, including advanced techniques such as insertion of multiple plastic stents, drainage of hilar strictures, retrieval of migrated stents and combined stenting in malignant biliary and duodenal obstructions.The target readership for the Clinical Guideline mostly includes digestive endoscopists, gastroenterologists, oncologists, radiologists, internists, and surgeons while the Technology Review should be most useful to endoscopists who perform biliary drainage.

  17. Percutaneous transhepatic removal of biliary stones: Clinical analysis of 16 cases

    Energy Technology Data Exchange (ETDEWEB)

    Ryeom, Hun Kyu; Sim, Jae In; Park, Auh Whan; Kim, Yong Joo [Kyungpook National University College of Medicine, Taegu (Korea, Republic of); Kim, Hee Jin [Taegu Fatima Hospital, Taegu (Korea, Republic of)

    1993-11-15

    Percutaneous transhepatic biliary drainage (PTBD) is widely used to control cholangitis, sepsis, or jaundice caused by biliary tree obstruction. The PTBD tract can be used in percutaneous biliary stone extraction in pre- or post-operative state when ERCP is failed or operation is contraindicated. We performed 16 cases of percutaneous transhepatic biliary stone removal. Locations of biliary stones are combined intrahepatic and extrahepatic in 8 cases(50%), only extrahepatic in 7 cases (44%), and only intrahepatic in 1 cases(6%). The number of stones was single in 6 cases and multiple in 10 cases. Over all success rate was 81% (13/16), 93% (14/15) in extrahepatic stones and 78% (7/9) in intrahepatic stones. In 5 of 6 caes, complete stone removed was impossible due to marked tortuosity of T-tube tract or peripherally located stones, complete removal of biliary stones was achieved via a new PTBD tract. No significant pre- or post-procedure complication was occurred. Percutaneous removal of biliary stones via PTBD tract is an effective and safe alternative method in difficult cases in the management of biliary tract stones.

  18. Interventional Endoscopy Database for Pancreatico-biliary, Gastrointestinal and Esophageal Disorders

    Science.gov (United States)

    2017-02-16

    Ampullary Cancer; Duodenal Cancer; Bile Duct Cancer; Bile Duct Disorders; Gallstones; Obstructive Jaundice; Pancreatic Disorders (Noncancerous); Colorectal Cancer; Esophageal Cancer; Barrett's Esophagus; Gastric Malignancies; Pancreatic Cancer; Pediatric Gastroenterology; Cholangiocarcinoma; Pancreatic Pseudocysts; Acute and Chronic Pancreatitis; Recurrent Pancreatitis; Cholangitis; Bile Leak; Biliary Strictures; Pancreatic Divisum; Biliary and Pancreatic Stones; Choledocholithiasis

  19. Biliary peritonitis due to fistulous tract rupture following a T-tube removal.

    Science.gov (United States)

    Sakorafas, George H; Stafyla, Vania; Tsiotos, Gregory G

    2005-06-24

    We present a patient with biliary peritonitis following a T-tube removal. The patient underwent laparotomy; a rupture of the fistulous tract around the T-tube was found. A Nelaton catheter was inserted through this opening and advanced toward the biliary tree and secured in place by a suture ligature. Postoperative course was uneventful.

  20. Double Sigmoid colon perforation due to migration of a biliary stent.

    Science.gov (United States)

    Malgras, B; Pierret, C; Tourtier, J-P; Olagui, G; Nizou, C; Duverger, V

    2011-10-01

    Migration of pancreatico-biliary stents is a rare event, usually benign, but which can lead to severe complications such as digestive tube perforation. We report the case of a patient with double sigmoid perforation due to distal migration of a biliary stent placed to decompress a pancreatic head carcinoma.

  1. Incidence of port-site metastasis after undergoing robotic surgery for biliary malignancies

    Institute of Scientific and Technical Information of China (English)

    Quan-Da Liu; Jun-Zhou Chen; Xiao-Ya Xu; Tao Zhang; Ning-Xin Zhou

    2012-01-01

    AIM:To investigate the incidence of clinically detected port-site metastasis (PSM) in patients who underwent robotic surgery for biliary malignancies.METHODS:Using a prospective database,the patients undergoing fully robotic surgery for biliary malignancies between January 2009 and January 2011 were included.Records of patients with confirmed malignancy were reviewed for clinicopathological data and information about PSM.RESULTS:Sixty-four patients with biliary tract cancers underwent robotic surgery,and sixty patients met the inclusion criteria.The median age was 67 year (range:40-85 year).During a median 15-mo follow-up period,two female patients were detected solitary PSM after robotic surgery.The incidence of PSM was 3.3%.Patient 1 underwent robotic anatomatic left hemihepatectomy and extraction of biliary tumor thrombi for an Klatskin tumor.She had a subcutaneous mass located at the right lateral abdominal wall near a trocar scar.Patient 2 underwent robotic pancreaticoduodenectomy for distal biliary cancer.She had two metachronous subcutaneous mass situated at the right lateral abdominal wall under a same trocar scar at 7 and 26 mo.The pathology of the excised PSM masses confirmed metastatic biliary adenocarcinoma.COMCLUSION:The incidence of PSMs after robotic surgery for biliary malignancies is relatively low,and biliary cancer can be an indication of robotic surgery.

  2. Incidence of underlying biliary neoplasm in patients after major hepatectomy for preoperative benign hepatolithiasis

    Science.gov (United States)

    Park, Hyeong Min; Cho, Chol Kyoon; Koh, Yang Seok; Kim, Hee Joon; Park, Eun Kyu

    2016-01-01

    Backgrounds/Aims Despite hepatolithiasis being a risk factor for biliary neoplasm including cholangiocarcinoma, the incidence of underlying biliary neoplasm is unknown in patients with preoperative benign hepatolithiasis. The aim of this study was to evaluate the incidence of underlying biliary neoplasm in patients who underwent major hepatectomy for preoperative benign hepatolithiasis. Methods Between March 2005 and December 2015, 73 patients who underwent major hepatectomy for preoperative benign hepatolithiasis were enrolled in this study. The incidence and pathological differentiation of concomitant biliary neoplasm were retrospectively determined by review of medical records. Postoperative complications after major hepatectomy were evaluated. Results Concomitant biliary neoplasm was pathologically confirmed in 20 patients (27.4%). Biliary intraepithelial neoplasia (BIN) was detected in 12 patients (16.4%), and 1 patient (1.4%) had intraductal papillary mucinous neoplasm (IPMN), as the premalignant lesion. Cholangiocarcinoma was pathologically confirmed in 7 patients (9.6%). Preoperative imaging of the 73 patients revealed biliary stricture at the first branch of bile duct in 31 patients (42.5%), and at the second branch of bile duct in 39 patients (53.4%). Postoperative complications developed in 14 patients (19.1%). Almost all patients recovered from complications, including intra-abdominal abscess (9.6%), bile leakage (4.1%), pleural effusion (2.7%), and wound infection (1.4%). Only 1 patient (1.4%) died from aspiration pneumonia. Conclusions The incidence of underlying biliary neoplasm was not negligible in the patients with hepatolithiasis, despite meticulous preoperative evaluations. PMID:28261696

  3. Altered intestinal bile salt biotransformation in a cystic fibrosis (Cftr(-/-)) mouse model with hepato-biliary pathology

    NARCIS (Netherlands)

    Bodewes, Frank A. J. A.; van der Wulp, Mariette Y. M.; Beharry, Satti; Doktorova, Marcela; Havinga, Rick; Boverhof, Renze; Phillips, M. James; Durie, Peter R.; Verkade, Henkjan J.

    2015-01-01

    Background: Cftr(-/-tm1UC) mice develop progressive hepato-biliary pathology. We hypothesize that this liver pathology is related to alterations' in biliary bile hydrophobicity and bile salt metabolism in Cftr(-/-tm1Unc) mice. Methods: We determined bile production, biliary and fecal bile salt- and

  4. Di(2-ethylhexyl) phthalate inhibits antral follicle growth, induces atresia, and inhibits steroid hormone production in cultured mouse antral follicles

    Energy Technology Data Exchange (ETDEWEB)

    Hannon, Patrick R., E-mail: phannon2@illinois.edu; Brannick, Katherine E., E-mail: kbran@illinois.edu; Wang, Wei, E-mail: Wei.Wang2@covance.com; Gupta, Rupesh K., E-mail: drrupesh@yahoo.com; Flaws, Jodi A., E-mail: jflaws@illinois.edu

    2015-04-01

    Di(2-ethylhexyl) phthalate (DEHP) is a ubiquitous environmental toxicant found in consumer products that causes ovarian toxicity. Antral follicles are the functional ovarian units and must undergo growth, survival from atresia, and proper regulation of steroidogenesis to ovulate and produce hormones. Previous studies have determined that DEHP inhibits antral follicle growth and decreases estradiol levels in vitro; however, the mechanism by which DEHP elicits these effects is unknown. The present study tested the hypothesis that DEHP directly alters regulators of the cell cycle, apoptosis, and steroidogenesis to inhibit antral follicle functionality. Antral follicles from adult CD-1 mice were cultured with vehicle control or DEHP (1–100 μg/ml) for 24–96 h to establish the temporal effects of DEHP on the follicle. Following 24–96 h of culture, antral follicles were subjected to gene expression analysis, and media were subjected to measurements of hormone levels. DEHP increased the mRNA levels of cyclin D2, cyclin dependent kinase 4, cyclin E1, cyclin A2, and cyclin B1 and decreased the levels of cyclin-dependent kinase inhibitor 1A prior to growth inhibition. Additionally, DEHP increased the mRNA levels of BCL2-associated agonist of cell death, BCL2-associated X protein, BCL2-related ovarian killer protein, B-cell leukemia/lymphoma 2, and Bcl2-like 10, leading to an increase in atresia. Further, DEHP decreased the levels of progesterone, androstenedione, and testosterone prior to the decrease in estradiol levels, with decreased mRNA levels of side-chain cleavage, 17α-hydroxylase-17,20-desmolase, 17β-hydroxysteroid dehydrogenase, and aromatase. Collectively, DEHP directly alters antral follicle functionality by inhibiting growth, inducing atresia, and inhibiting steroidogenesis. - Highlights: • DEHP inhibits antral follicle growth by dysregulating cell cycle regulators. • DEHP induces antral follicle atresia by dysregulating apoptosis regulators. • DEHP

  5. Efficacy of Lower-Extremity Venous Thrombolysis in the Setting of Congenital Absence or Atresia of the Inferior Vena Cava

    Energy Technology Data Exchange (ETDEWEB)

    Ganguli, Suvranu, E-mail: sganguli@partners.org; Kalva, Sanjeeva; Oklu, Rahmi; Walker, T. Gregory; Datta, Neil [Massachusetts General Hospital and Harvard Medical School, Division of Vascular Imaging and Intervention, Department of Imaging (United States); Grabowski, Eric F. [Massachusetts General Hospital and Harvard Medical School, Division of Hematology and Oncology, Department of Pediatrics (United States); Wicky, Stephan [Massachusetts General Hospital and Harvard Medical School, Division of Vascular Imaging and Intervention, Department of Imaging (United States)

    2012-10-15

    Purpose: A rare but described risk factor for deep venous thrombosis (DVT), predominately in the young, is congenital agenesis or atresia of the inferior vena cava (IVC). The optimal management for DVT in this subset of patients is unknown. We evaluated the efficacy of pharmacomechanical catheter-directed thrombolysis (PCDT) followed by systemic anticoagulation in the treatment of acute lower-extremity DVT in the setting of congenital IVC agenesis or atresia. Materials and Methods: Between November of 2005 and May of 2010, six patients (three women [average age 21 years]) were referred to our department with acute lower-extremity DVT and subsequently found to have IVC agenesis or atresia on magnetic resonance imaging. A standardized technique for PCDT (the Angiojet Rheolytic Thrombectomy System followed by the EKOS Microsonic Accelerated Thrombolysis System) was used for all subjects. Successful thrombolysis was followed by systemic heparinization with transition to Coumadin or low molecular-weight heparin and compression stockings. Subjects were followed-up at 1, 3, and then every 6 months after the procedure with clinical assessment and bilateral lower-extremity venous ultrasound. Results: All PCDT procedures were technically successful. No venous stenting or angioplasty was performed. The average thrombolysis time was 28.6 h (range 12-72). Two patients experienced heparin-induced thrombocytopenia, and one patient developed a self-limited knee hemarthrosis, No patients were lost to follow-up. The average length of follow-up was 25.8 {+-} 20.2 months (range 3.8-54.8). No incidence of recurrent DVT was identified. There were no manifestations of postthrombotic syndrome. Conclusions: PCDT followed by systemic anticoagulation and the use of compression stockings appears to be safe and effective in relatively long-term follow-up treatment of patients who present with acute DVT and IVC agenesis or atresia.

  6. Reconstruction of congenital microtia-atresia: outcomes with the Medpor/bone-anchored hearing aid-approach.

    Science.gov (United States)

    Romo, Thomas; Morris, Luc G T; Reitzen, Shari D; Ghossaini, Soha N; Wazen, Jack J; Kohan, Darius

    2009-04-01

    Ideal surgery for congenital microtia-atresia would offer excellent cosmetic and hearing rehabilitation, with minimal morbidity. Classic approaches require multiple procedures, including rib cartilage harvest and aural atresia repair. Our facial plastic and otologic team approach incorporates a high-density porous polyethylene (Medpor, Porex Surgical, Newnan, GA) auricular framework, followed by single-stage bone-anchored hearing aid (BAHA) implantation. We evaluated the efficacy, safety, and morbidity of this 2-stage dual system approach. A prospective database of microtia patients was used to identify patients undergoing combined Medpor/BAHA auricular reconstruction and hearing rehabilitation between 2003 and 2006. The first stage involves placement of a Medpor framework beneath a temporoparietal fascia flap, followed by a second-stage procedure for lobule transposition and BAHA implantation. Twenty-five patients (28 ears) were evaluated. Aesthetic quality of the implants was excellent, with a high degree of framework detail visible, and a postauricular crease created in all patients. All patients were satisfied with the cosmetic result. There were no major Medpor complications such as infection, extrusion, loss of implant, or flap necrosis, and a 10.7% incidence of minor complications requiring operative revision. BAHA significantly improved hearing in all patients, with a complication rate of 31.8%, mainly skin overgrowth and cellulitis. The Medpor/BAHA dual plastic-otologic approach to microtia-atresia has produced excellent cosmetic results and hearing outcomes, which compare favorably to traditional microtia-atresia repair. This is a 2-stage aesthetic and functional protocol with an acceptably low rate of complications, which safely and efficiently achieves both aesthetic and functional goals.

  7. Atresia yeyunal tipo iiib o en cáscara de manzana. Reporte de caso clínico

    Directory of Open Access Journals (Sweden)

    Álvaro Cano Salazar

    2013-10-01

    Full Text Available Title: Jejunal atresia iiib type or apple peel type. Case report.ResumenLa atresia intestinal es causa importante de obstrucción en el recién nacido. La ecografía prenatal hace diagnóstico temprano y permite un tratamiento quirúrgico inmediato, mejorando la sobrevida de los pacientes, y a su vez elevando las probabilidades de éxito en la corrección quirúrgica. La sinología clínica, y los exámenes radiográficos simples y con contraste, permiten hacer el diagnóstico en más del 95% de los casos. Se presenta un caso clínico en el cual gracias a la ecografía prenatal se realizó diagnóstico precoz, y se efectuó corrección quirúrgica inmediata mediante anastomosis término–terminal. (DUAZARY 2012 No. 1, 68 - 71AbstractIntestinal atresia is a important cause of intestinal obstruction in the newborn. The prenatal ultrasound ago allows early diagnosis and surgical treatment immediately, improving patient survival affected by this disease, and in turn raising chances of successful surgical correction. The clinical symptoms, simple radiographic examinations and contrast, allow the diagnosis in over 95% of cases. Here is a case in which by prenatal ultrasound diagnosis is made early and immediate surgical correction was performed by anastomosis-terminal.Keywords: Intestinal Atresia; Ultrasonography; Prenatal Diagnosis; Newborn.

  8. Biliary Duct-to-Duct Reconstruction with a Tunneled Retroperitoneal T-Tube During Liver Transplantation: a Novel Approach to Decrease Biliary Leaks After T-Tube Removal.

    Science.gov (United States)

    Navez, Julie; Mohkam, Kayvan; Darnis, Benjamin; Cazauran, Jean-Baptiste; Ducerf, Christian; Mabrut, Jean-Yves

    2017-04-01

    The benefit of placing a T-tube for duct-to-duct biliary reconstruction during orthotopic liver transplantation (OLT) remains controversial because it could be associated with specific complications, especially at the time of T-tube removal. While the utility of T-tube during OLT represents an eternal debate, only a few technical refinements of T-tube placement have been described since the report of the original technique by Starzl and colleagues. Herein, we present a novel technique of T-tube placement for duct-to-duct biliary reconstruction during OLT, using a tunneled retroperitoneal route. On the basis of our experience of 305 patients who benefitted from the reported technique, the placement of a tunneled retroperitoneal biliary T-tube appears to be safe and results in a low rate of biliary complications, especially at the time of T-tube removal.

  9. Apoptosis in Granulosa cells during follicular atresia:relationship with steroids and insulin-like growth factors

    Institute of Scientific and Technical Information of China (English)

    Yuan Song YU; Hong Shu SUI; Zheng Bin HAN; Wei LI; Ming Jiu LUO; Jing He TAN

    2004-01-01

    It is well known that during mammalian ovarian follicular development, the majority of follicles undergo atresia at various stages of their development. However, the mechanisms controlling this selection process remain unknown. In this study, we investigated apoptosis in granulosa cells during goat follicular atresia by terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL). The changes in the levels of steroids, insulin-like growth factors (IGFs) and IGF receptors were studied by radioimmunoassay (RIA) and semi-quantitative reverse transcription-PCR. We found that the percentage of apoptotic granulosa cells in the atretic (A) follicles was significantly higher than that in the slightly atretic (SA) and healthy (H) follicles. The level of estradiol and the ratio of estradiol to progesterone in H follicles were significantly higher than those in A follicles. On the other hand, the level of progesterone was not significantly different among these follicle types. We also found that the level of IGF-I in H follicles was higher than in SA and A follicles, whereas the amount of IGF-Ⅱ did not vary significantly. The expression of IGF receptor also decreased in A follicles as compared to that in H and SA follicles. These results suggested that estradiol and IGF-I might be involved in controlling apoptosis in granulosa cells during follicular atresia.

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

    Science.gov (United States)

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

    2012-12-01

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

  11. Novel Approach to Bile Duct Damage in Primary Biliary Cirrhosis: Participation of Cellular Senescence and Autophagy

    Directory of Open Access Journals (Sweden)

    Motoko Sasaki

    2012-01-01

    Full Text Available Primary biliary cirrhosis (PBC is characterized by antimitochondrial autoantibodies (AMAs in patients' sera and histologically by chronic nonsuppurative destructive cholangitis in small bile ducts, eventually followed by extensive bile duct loss and biliary cirrhosis. The autoimmune-mediated pathogenesis of bile duct lesions, including the significance of AMAs, triggers of the autoimmune process, and so on remain unclear. We have reported that cellular senescence in biliary epithelial cells (BECs may be involved in bile duct lesions and that autophagy may precede the process of biliary epithelial senescence in PBC. Interestingly, BECs in damaged bile ducts show characteristicsof cellular senescence and autophagy in PBC. A suspected causative factor of biliary epithelial senescence is oxidative stress. Furthermore, senescent BECs may modulate the microenvironment around bile ducts by expressing various chemokines and cytokines called senescence-associated secretory phenotypes and contribute to the pathogenesis in PBC.

  12. Imaging tests for accurate diagnosis of acute biliary pancreatitis.

    Science.gov (United States)

    Şurlin, Valeriu; Săftoiu, Adrian; Dumitrescu, Daniela

    2014-11-28

    Gallstones represent the most frequent aetiology of acute pancreatitis in many statistics all over the world, estimated between 40%-60%. Accurate diagnosis of acute biliary pancreatitis (ABP) is of outmost importance because clearance of lithiasis [gallbladder and common bile duct (CBD)] rules out recurrences. Confirmation of biliary lithiasis is done by imaging. The sensitivity of the ultrasonography (US) in the detection of gallstones is over 95% in uncomplicated cases, but in ABP, sensitivity for gallstone detection is lower, being less than 80% due to the ileus and bowel distension. Sensitivity of transabdominal ultrasonography (TUS) for choledocolithiasis varies between 50%-80%, but the specificity is high, reaching 95%. Diameter of the bile duct may be orientative for diagnosis. Endoscopic ultrasonography (EUS) seems to be a more effective tool to diagnose ABP rather than endoscopic retrograde cholangiopancreatography (ERCP), which should be performed only for therapeutic purposes. As the sensitivity and specificity of computerized tomography are lower as compared to state-of-the-art magnetic resonance cholangiopancreatography (MRCP) or EUS, especially for small stones and small diameter of CBD, the later techniques are nowadays preferred for the evaluation of ABP patients. ERCP has the highest accuracy for the diagnosis of choledocholithiasis and is used as a reference standard in many studies, especially after sphincterotomy and balloon extraction of CBD stones. Laparoscopic ultrasonography is a useful tool for the intraoperative diagnosis of choledocholithiasis. Routine exploration of the CBD in cases of patients scheduled for cholecystectomy after an attack of ABP was not proven useful. A significant rate of the so-called idiopathic pancreatitis is actually caused by microlithiasis and/or biliary sludge. In conclusion, the general algorithm for CBD stone detection starts with anamnesis, serum biochemistry and then TUS, followed by EUS or MRCP. In the end

  13. Effect of diethyl ether on the biliary excretion of acetaminophen.

    Science.gov (United States)

    Watkins, J B; Siegers, C P; Klaassen, C D

    1984-10-01

    The biliary and renal excretion of acetaminophen and its metabolites over 8 hr was determined in rats exposed to diethyl ether by inhalation for 1 hr. Additional rats were anesthetized with urethane (1 g/kg ip) while control animals were conscious throughout the experiment (surgery was performed under hexobarbital narcosis: 150 mg/kg ip; 30-min duration). The concentration of UDP-glucuronic acid was decreased 80% in livers from ether-anesthetized rats but was not reduced in urethane-treated animals when compared to that in control rats. The concentration of reduced glutathione was not affected by either urethane or diethyl ether. Basal bile flow was not altered by the anesthetic agents. Bile flow rate after acetaminophen injection (100 mg/kg iv) was increased slightly over basal levels for 2 hr in hexobarbital-treated control rats, was unaltered in urethane-anesthetized animals, and was decreased throughout the 8-hr experiment in rats exposed to diethyl ether for 1 hr. In control and urethane-anesthetized animals, approximately 30-35% of the total acetaminophen dose (100 mg/kg iv) was excreted into bile in 8 hr, while only 16% was excreted in rats anesthetized with diethyl ether. Urinary elimination (60-70% of the dose) was not altered by exposure to ether. Separation of metabolites by reverse-phase high-pressure liquid chromatography showed that ether decreased the biliary elimination of unchanged acetaminophen and its glucuronide, sulfate, and glutathione conjugates by 47, 40, 49, and 73%, respectively, as compared to control rats. Excretion of unchanged acetaminophen and the glutathione conjugate into bile was depressed in urethane-anesthetized animals by 45 and 66%, respectively, whereas elimination of the glucuronide and sulfate conjugates was increased by 27 and 50%, respectively. These results indicate that biliary excretion is influenced by the anesthetic agent and that diethyl ether depresses conjugation with sulfate and glutathione as well as glucuronic

  14. Spontaneous biliary peritonitis: Is bed side diagnosis possible?

    Directory of Open Access Journals (Sweden)

    Vijai Datta Upadhyaya

    2013-01-01

    Full Text Available Background: Spontaneous biliary peritonitis is a rare cause of acute abdomen. In spontaneous biliary peritonitis there is perforation in the wall of the extra-hepatic or intra-hepatic duct occurs without any traumatic or iatrogenic injury and have been described more often in neonates. The symptoms may be acute or insidious delaying the diagnosis. Present manuscript deals with diagnosis and management of these cases. Materials and Methods: This is a prospective study and all patients of suspected biliary peritonitis presented during Dec 2010 to Feb 2012 were included in the study. After preliminary investigations in all patients abdominal paracentesis was done and in cases where intra-abdominal fluid bilirubin level was several fold higher than serum bilirubin level were subjected to exploratory laparotomy. Further investigation like T-tube cholangiogram and magnetic resonance cholangiopancreatography (MRCP was done to rule out choledochal cyst before leveling these cases as SPBD. Results: A total of 6 patients were included in present series commonest presenting symptom was progressive abdominal distension without signs of overt peritonitis followed by progressive jaundice, fever and abdominal pain. On exploration site of perforation was observed in 50% of cases and in 50% of cases bile duct was not dilated. Second surgery was not required in 34% of cases. There was no mortality or significant morbidity in our series. Conclusion: Spontaneous perforation of bile duct is rare disease and high index of suspicion is required for diagnosis. Simple bed side test can help in diagnosis but T tube cholangiogram or MRCP are must to rule out choledochal cyst.

  15. Experimental evidence on the immunopathogenesis of primary biliary cirrhosis

    Science.gov (United States)

    Selmi, Carlo; Meda, Francesca; Kasangian, Anaid; Invernizzi, Pietro; Tian, Zhigang; Lian, Zhexiong; Podda, Mauro; Gershwin, M Eric

    2010-01-01

    Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease for which an autoimmune pathogenesis is supported by clinical and experimental data, including the presence of autoantibodies and autoreactive T cells. The etiology remains to be determined, yet data suggest that both a susceptible genetic background and unknown environmental factors determine disease onset. Multiple infectious and chemical candidates have been proposed to trigger the disease in a genetically susceptible host, mostly by molecular mimicry. Most recently, several murine models have been reported, including genetically determined models as well as models induced by immunization with xenobiotics and bacteria. PMID:20029462

  16. Abandonment of endoprosthetic drainage technique in malignant biliary obstruction.

    Science.gov (United States)

    Mendez, G; Russell, E; LePage, J R; Guerra, J J; Posniak, R A; Trefler, M

    1984-09-01

    This report discusses a 3-year experience with the techniques of internal/external catheter and endoprosthetic stent drainage in 175 patients with obstructive jaundice. In 166 patients, the obstruction was bypassed satisfactorily. The complications encountered with these techniques are compared, and the radiation doses involved are emphasized. It is not necessary to put patients through the extra time and pain related to the placement of the endoprosthesis, because internal/external catheter placement produces the same desired result of biliary decompression without the higher radiation doses to the radiologist.

  17. Wandering biliary ascariasis with hepatic abscess in a postmenopausal woman.

    Science.gov (United States)

    Nahar, N; Khan, N; Islam, S M; Chakraborty, R K; Rima, S Z; Alam, M N; Roy, A S

    2014-10-01

    Hepatobiliary ascariasis is common in developing countries where there is a low standard of public health and hygiene. We are reporting a rare case of ascariasis which induced multiple liver abscesses in a post menopausal woman who presented with fever, anorexia, nausea, vomiting and mild hepatomegaly. Ultrasonography revealed biliary ascariasis with multiple hepatic abscesses. Laparoscopic drainage of hepatic abscesses was done and microscopic examination of drainage materials showed decorticated eggs of Ascaris Lumbricoides. The post operative recovery of the patient was uneventful. Ultrasonography is a reliable modality to diagnose and follow up of such cases.

  18. Endoscopic management of biliary leaks after laparoscopic cholecystectomy.

    Science.gov (United States)

    Rustagi, Tarun; Aslanian, Harry R

    2014-09-01

    Laparoscopic cholecystectomy has become the procedure of choice for management of symptomatic cholelithiasis. Although it has distinct advantages over open cholecystectomy, bile leak is more common. Endoscopic retrograde cholangiopancreatography is the diagnostic and therapeutic modality of choice for management of postcholecystectomy bile leaks and has a high success rate with the placement of plastic biliary stents. Repeat endoscopic retrograde cholangiopancreatography with placement of multiple plastic stents, a covered metal stent, or possibly cyanoacrylate therapy may be effective in refractory cases. This review will discuss the indications, efficacy, and complications of endoscopic therapy.

  19. Long-term effects of forgotten biliary stents: a case series and literature review

    Science.gov (United States)

    Odabasi, Mehmet; Arslan, Cem; Akbulut, Sami; Abuoglu, Haci Hasan; Ozkan, Erkan; Yildiz, Mehmet Kamil; Eris, Cengiz; Gunay, Emre; Tekesin, Kemal; Muftuoglu, Tolga

    2014-01-01

    There are many studies about the biliary stents, however there is a little information about the long-term stayed forgotten biliary stents except a few case reports. We have reported the results of a number of cases with biliary stents that were forgotten or omitted by the patient and the endoscopist. During February 2010 to May 2013, five patients were referred to the general surgery clinic of Haydarpasa Numune Training and Research Hospital, Istanbul Turkey. Past history and medical documents submitted by the patient did not indicate a replacement of the biliary stent in 3 patients. Two patients knew that they had biliary stents. We also conducted a literature review via the PubMed and Google Scholar databases of English language studies published until March 2014 on forgotten biliary stent. There were 3 men and 2 women ranging in age from 22 to 68 years (mean age 41.6 years). Patients presented with pain in the upper abdomen, jaundice, fever, abnormal liver function tests or dilatation of the biliary tract alone or in combination. Patients’ demographic findings are presented in Table 1. A review of three cases reported in the English medical literature also discussed. The mean duration of the patency of the stent is about 12 months. The biliary stenting is performed either with plastic or metal stents, studies recommending their replacement after 3-6 months. Patients with long stayed forgotten biliary stents are inevitably treated with surgical intervention. We recommend for all endoscopic retrograde cholangiopancreatography units provide a stent registry system that the stents placed for various therapeutic procedures are not forgotten both by the patient as well as the physician. There should be a deadline for biliary stents in the registry system for each patient. PMID:25232385

  20. Sedations and analgesia in patients undergoing percutaneous transhepatic biliary drainage

    Energy Technology Data Exchange (ETDEWEB)

    Hatzidakis, A.A.; Charonitakis, E.; Athanasiou, A.; Tsetis, D.; Chlouverakis, G.; Papamastorakis, G.; Roussopoulou, G.; Gourtsoyiannis, N.C

    2003-02-01

    AIM: To present our experience using intravenous sedoanalgesia for percutaneous biliary drainage. MATERIALS AND METHODS: This study comprised 100 patients, all of whom were continuously monitored [electrocardiogram (ECG), blood pressure, pulse oxymetry] and received an initial dose of 2 mg midazolam followed by 0.02 mg fentanyl. Before every anticipated painful procedure, a maintenance dose of 0.01 mg fentanyl was administered. If the procedure continued and the patient became aware, another 1 mg midazolam was given. This was repeated if patients felt pain. A total dose of 0.08 mg fentanyl and 7 mg midazolam was never exceeded. Immediately after the procedure, the nurse was asked to evaluate patients' pain score. The patients were asked 3 h later to complete a visual 10-degree pain score scale. RESULTS: The average dose of fentanyl and midazolam was 0.042 mg (0.03-0.08 mg) and 4.28 mg (2-7 mg), respectively. Only one patient recorded the procedure as painful. The scores given by the attending nurse (1-7 points, mean 2.9) correlated well with those given by the patients (1-6 points, mean 2.72). No complications were noted. CONCLUSION: According to our experience, interventional radiologists practising biliary procedures can administer low doses of midazolam and minimize the doses of fentanyl, without loss of adequate sedation and analgesia. Hatzidakis, A. A. et al. (2003). Clinical Radiology58, 121-127.

  1. Primary Biliary Cirrhosis and the Nuclear Pore Complex

    Science.gov (United States)

    Duarte-Rey, Carolina; Bogdanos, Dimitrios; Yang, Chen-Yen; Roberts, Krista; Leung, Patrick S.C.; Anaya, Juan-Manuel; Worman, Howard J.; Gershwin, M. Eric

    2012-01-01

    Experimental models of autoimmune diseases have led to the conclusion that an immune response to nuclear antigens is a sentinel marker for loss of tolerance and potential tissue damage. Various proteins are targets of antinuclear antibodies in a variety of autoimmune diseases, ranging from systemic rheumatologic disorders to diseases affecting specific organs such as the liver. Autoantibodies against specific nuclear constituents have also been used as probes to understand the structure and the function of the targeted components and their relevance to disease pathogenesis. Approximately a quarter of patients with primary biliary cirrhosis (PBC) have antibodies targeting proteins of the nuclear pore complex (NPC), a multi-protein structure that mediates molecular transport across the nuclear envelope. Autoantibodies against the integral membrane glycoprotein gp210 and nucleoporin p62 appear to be highly specific for PBC, an autoimmune disease characterized by progressive destruction of intrahepatic biliary epithelial cells. This review discusses the diagnostic and clinical relevance of anti-NPC antibodies in PBC and the possibility that this autoimmune response may arise as a result of molecular mimicry. PMID:22487189

  2. Successful surgical management of an extrahepatic biliary cystadenocarcinoma

    Directory of Open Access Journals (Sweden)

    Márcio Lobo Guimarães

    2011-10-01

    Full Text Available Extrahepatic bile duct cancer is an uncommon disease, and few cases are curable by surgery. We report a case of extrahepatic biliary cystadenocarcinoma (BCAC associated with atrophy of the left hepatic lobe. A 54-year old male was admitted with painless obstructive jaundice and a hepatic palpable mass noticed one month before presentation. Liver functions tests were consistent with cholestatic damage and serum carbohydrate antigen 19.9 (CA 19-9 was increased before treatment. Magnetic resonance imaging (MRI disclosed dilatation of the left hepatic bile duct with irregular wall thickening close to the hepatic confluence, and atrophy of left hepatic lobe. The patient was submitted to en bloc extended left hepatectomy with resection of caudate lobe, hilar lymphadenectomy, and suprapancreatic biliary tree resection. All surgical margins were grossly negative, and postoperative course was uneventful, except for a minor bile leak. The patient was discharged on the 15th postoperative day; he is alive without tumor recurrence one year after primary therapy. Although technically challenging, extended en bloc resection is feasible in adults with extrahepatic BCAC and can improve survival with acceptable and manageable morbidity.

  3. Role for mycobacterial infection in pathogenesis of primary biliary cirrhosis?

    Institute of Scientific and Technical Information of China (English)

    Daniel Smyk; Eirini I Rigopoulou; Yoh Zen; Robin Daniel Abeles; Charalambos Billinis; Albert Pares; Dimitrios P Bogdanos

    2012-01-01

    Primary biliary cirrhosis (PBC) is a progressive cholestatic liver disease characterized by the immunemediated destruction of biliary epithelial cells in small intrahepatic bile ducts.The disease is characterized by circulating antimitochondrial antibodies (AMAs) as well as disease-specific antinuclear antibodies,cholestatic liver function tests,and characteristic histological features,including granulomas.A variety of organisms are involved in granuloma formation,of which mycobacteria are the most commonly associated.This has led to the hypothesis that mycobacteria may be involved in the pathogenesis of PBC,along with other infectious agents.Additionally,AMAs are found in a subgroup of patients with mycobacterial infections,such as leprosy and pulmonary tuberculosis.Antibodies against species-specific mycobacterial proteins have been reported in patients with PBC,but it is not clear whether these antibodies are specific for the disease.In addition,data in support of the involvement of the role of molecular mimicry between mycobacterial and human mitochondrial antigens as triggers of cross-reactive immune responses leading to the loss of immunological tolerance,and the induction of pathological features have been published.Thus,antibodies against mycobacterial heat shock protein appear to cross-recognize AMA-specific autoantigens,but it is not clear whether these autoantibodies are mycobacterium-species-specific,and whether they are pathogenic or incidental.The view that mycobacteria are infectious triggers of PBC is intriguing,but the data provided so far are not conclusive.

  4. Real-time ultrasonographic findings of biliary ascariasis

    Energy Technology Data Exchange (ETDEWEB)

    Woo, Seong Ku; Sung, Dong Wook; Ko, Young Tae; Kim, Ho Kyun; Kim, Soon Yong [Kyung Hee University Hospital, Seoul (Korea, Republic of)

    1983-06-15

    Although the ultrasonography has been regarded as a main procedure in the diagnosis of bile duct and gallbladder diseases, a few reports concerning the ultrasonographic findings of biliary ascariasis appeared in the literature. Seven cases of real-time ultrasonographic diagnosis of biliary ascariasis were made in our hospital during last 15 months. In six, the diagnosis was confirmed by surgery or radiographic examination. The results were as follows: 1. The characteristic long, thick, highly reflective echo without distal acoustic shadowing in the extra- and/or intra-hepatic ducts were seen in all six proven cases. 2. The echogenic, nonshadowing images of the worms were seen as single stripe in five cases (containing a central, longitudinal, anechoic tube, suggesting its digestive tract in three cases) or multiple stripes in one case. But so-called coiled appearance or amorphous fragments were not seen in our cases. 3. Associated findings were as follows: mild to moderate dilatation of the extrahepatic duct in all six proven cases, mild dilatation of the intrahepatic ducts in 3 cases, mild dilatation of the pancreatic duct in 1 case, intrahepatic stone in 1 case, contracted gallbladder containing single stone in 1 case and evidence of cholecystitis in 4 cases. 4. One false positive diagnosis of ascariasis in the gallbladder is considered due to reverberation artefact. No false negative was seen.

  5. Endoscopic treatment of biliary complications after liver transplantation

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    AIM: To evaluate the efficacy of endoscopic treatment in patients who undergo OLTx or LRLTx and develop biliary complications. METHODS: This is a prospective, observational study of patients who developed biliary complications, after OLTx and LRLTx, with duct-to-duct anastomosis p erformed between June 2003 and June 2007. Endoscopic Retrograde Cholangiopancreatography (ERCP) was considered unsuccessful when there was evidence of continuous bile leakage despite endoscopic stent placement, or persistence of stenosis after 1 year, despite multiple dilatation and stent placement. When the ERCP failed, a percutaneous trans-hepatic approach (PTC) or surgery was adopted. RESULTS: From .lune 2003 to .lune 2007, 261 adult patients were transplanted in our institute, 68 from living donors and 193 from cadaveric donors. In the OLTx group the rate of complications was 37.3%, while in the LRLTx group was 64.7%. The rate of FRCP failure was 19.4% in the OLTx group and 38.6% in LRLTx group. In OLTx group, 1 patient was re- transplanted and 8 patients died. In the LRLTx group, 2 patients underwent OLTx and 8 patients died. The follow-up was 23.3 + 13.13 mo and 21.02:1:14.10 raG, respectively. CONCLUSION: Albhough ERCP is quite an effective mode of managing post-transplant bile duct complications, a significant number of patients need other types of approach. Further prospective studies are necessary in order to establish whether other endoscopic protocols or new devices, could improve the current results.

  6. Targeting Angiogenesis in Biliary Tract Cancers: An Open Option

    Directory of Open Access Journals (Sweden)

    Valeria Simone

    2017-02-01

    Full Text Available Biliary tract cancers (BTCs are characterized by a bad prognosis and the armamentarium of drugs for their treatment is very poor. Although the inflammatory status of biliary tract represents the first step in the cancerogenesis, the microenvironment also plays a key role in the pathogenesis of BTCs, promoting tumor angiogenesis, invasion and metastasis. Several molecules, such as vascular endothelial growth factor (VEGF and fibroblast growth factor (FGF, are involved in the angiogenesis process and their expression on tumor samples has been explored as prognostic marker in both cholangiocarcinoma and gallbladder cancer. Recent studies evaluated the genomic landscape of BTCs and evidenced that aberrations in several genes enrolled in the pro-angiogenic signaling, such as FGF receptor-2 (FGFR-2, are characteristic of BTCs. New drugs targeting the signaling pathways involved in angiogenesis have been tested in preclinical studies both in vitro and in vivo with promising results. Moreover, several clinical studies tested monoclonal antibodies against VEGF and tyrosine kinase inhibitors targeting the VEGF and the MEK/ERK pathways. Herein, we evaluate both the pathogenic mechanisms of BTCs focused on angiogenesis and the preclinical and clinical data available regarding the use of new anti-angiogenic drugs in these malignancies.

  7. Urinary and biliary metabolites of daidzin and daidzein in rats.

    Science.gov (United States)

    Yasuda, T; Kano, Y; Saito, K; Ohsawa, K

    1994-10-01

    Examination was made of the urinary and biliary excretion of metabolites of daidzin and daidzein, the major components of roots of Pueraria lobata Ohwi (Leguminosae) in rats. The urine of rats administered daidzin orally contained four major metabolites, daidzein 7,4'-di-O-sulfate (M-1), daidzein 7-O-beta-D-glucuronide (M-2), daidzein 4'-O-sulfate (M-3), daidzein (M-4), as determined from spectroscopic and chemical data. The urine of rats treated with daidzein contained M-2--M-4 in the above metabolites. Total cumulative amounts of the four metabolites excreted in the urine at 48 h following the oral administration of daidzin and daidzein were approximately 4.8% and 4.6% of the doses administered, respectively. The bile of rats administered daidzin orally contained M-1--M-4. Daidzein 7-O-beta-D-glucuronide 4'-O-sulfate (M-5), a major biliary metabolite, was identified by the high-performance liquid chromatography (HPLC), liquid chromatography-mass spectrometry (LC-MS) and nuclear magnetic resonance (NMR) spectra. At least daidzin appeared to be hydrolyzed to aglycone after absorption in the body, and as a part of metabolites, M-1--M-4 having free hydroxyl, glucuronided or sulfated hydroxyls at the C-7 position, may then be excreted in the urine and bile.

  8. Oesophageal atresia: a 10-year experience of a Paediatric Intensive Care Unit

    Directory of Open Access Journals (Sweden)

    Liliana Pinho

    2016-03-01

    Full Text Available Background/Purpose: Oesophageal atresia (OA is a congenital malformation with a variable prognosis. The aims were to establish OA’s incidence in the central region, to characterize infants with OA admitted and to compare its clinical outcome after surgical repair, according to OA classifi cation. Methods: A retrospective review of infants with OA admitted to a PICU, after surgical repair, between 2002 and 2011. Patient characteristics, OA’s classifi cation, surgery, morbidity and mortality were analyzed. Two groups were compared according to OA classifi cation. Results: Thirty-four infants were admitted, out of which 65% were male, with a median gestational age of 36 weeks and birth weight of 2310g. Nineteen of them presented other malformations, mainly cardiac. Nine cases were classifi ed as long-gap OA. Fistula ligation and primary oesophageal anastomosis was the most common surgical option (n=27. Early complications occurred in 13 infants (38%, mostly anastomotic leak, and were similar according to gap length (p=0.704. PICU stay and mechanical ventilation were longer in long-gap OA patients (p=0.009 and p<0.001 respectively and in infants with other malformations (p=0.027 and p=0.003 respectively. There was no mortality. Conclusions: The frequency of OA associated malformations implies a systematic screening of these patients. Gap length and presence of associated malformations were the major determinants of length of intensive care stay and ventilation days in OA patients.

  9. Distribution of Interstitial Cells of Cajal in the Esophagus of Fetal Rats with Esophageal Atresia

    Directory of Open Access Journals (Sweden)

    Caner Isbir

    2016-04-01

    Full Text Available Aim: Scarcity of the interstitial cells of Cajal (ICC is related to motility disorders. In the study, we aimed to evaluate the number and density of ICCs in the fetal rat esophagus in the adriamycin - esophageal atresia (EA model. Material and Method: Rat fetuses were divided into three groups as a control, adriamycin group without EA and adriamycin group with EA. Four doses of adriamycin, 2 mg/kg each, were injected intraperitoneally to the adriamycin group rats between on 6 and 9 days of gestation. The presence of ICCs in the esophagus of the rat fetuses was determined by using an immunohistochemistry technique (c-kit, CD117. The average numbers of ICCs were calculated with microscopic evaluation by using a visual scoring system (range1 to 3. Results: Seven fetuses were included in each group. The ICCs score 3 distributions of fetuses were 5 (72% fetuses in the control group, 3 (43% fetuses in the adriamycin group without EA, 1 (14% fetus in the adriamycin group with EA. It have been found that there was a marked reduction of ICCs distribution in the adriamycin group with EA compared to control group (p 0.05. Discussion: ICCs density was significantly decreased in the rat fetuses with EA compared to the fetuses without EA. These findings support the idea that ICCs density may be congenitally abnormal in EA. This may be led to dismotility seen in the operated esophagus due to EA.

  10. Comparison between Two Cecostomy Techniques for Treatment of Atresia Coli in Cattle and Buffalo Calves

    Directory of Open Access Journals (Sweden)

    Mohamad Abdelfattah Abdelrhman, Aiman Mahmoud Seddek*1 and Hossam Ahmad Bakr2

    2013-07-01

    Full Text Available The study aimed at determination of the cecostomy technique that will preserve the long-term maximal diameter of the created fistula. The subjects of the study were 79 newly born cattle and buffalo calves suffered from atresia coli, recti and ani. The calves were grouped into four groups for evaluation of incisional and herniation cecostomy techniques either on the right or left flank. With each technique, 4 cm diameter permanent fistula was created and evaluated at the 1stweek, and 1st, 4th and 8th months post-surgery. Both of the short and long-term complications were recorded and managed conservatively. Follow up could be achieved for 45 calves only and all of them showed stunted growth. The results revealed wider stoma in the calves operated by herniation technique, and in those operated on the right flank, moreover the operated cattle calves had wider stoma than buffalo calves. It is concluded that the right flank herniation technique has a beneficial effect on long-term preserving of a wider diameter of the created fistula.

  11. [In utero exposure to benzodiazepine. Is there a risk for anal atresia with lorazepam?].

    Science.gov (United States)

    Bonnot, O; Vollset, S-E; Godet, P-F; d'Amato, T; Dalery, J; Robert, E

    2003-01-01

    categories: congenital anomalies of heart, cleft lip and/or cleft palate, neural tube defects, other anomalies of central nervous system, hypospadias, urinary malformations, anal atresia, other digestive anomalies, limb reduction defects, and genetic anomalies, including chromosome aberrations and monogenic conditions. Other malformations were grouped in an eleventh category. The interesting aspect of this study is that it takes into account the BZD metabolism. It is worth noting that the hepatic catabolism of benzodiazepine is a very complex one, because it leads to derived molecules which are sometimes active and/or present in the common metabolic route of major commercial drugs. Our hypothesis is that if one BZD is associated specifically to a certain type of congenital defect, we may find this BZD to be overrepresented, as compared with other BZDs, in newborns exhibiting some type of congenital defect. The analysis was run according to a case-control approach. Odd ratios (OR) and their 95% confidence intervals were calculated by logistic regression with adjustment for maternal age and parity. When one category of defects was considered, infants having the corresponding malformation were considered as cases, while infants with other malformations were considered as controls. In a similar way infants having being exposed to a given drug were considered as exposed, while infants exposed to any other drug were considered as unexposed. The analysis then was run in 4 steps. Step 1: full sample. With 13,703 cases. We observed no increased risk for any specific malformation type associated with use of BZD. Step 2: further defining drug exposure as a specific BZD, and all others unexposed, a significant association was seen between lorazepam and anal atresia. OR=6.2 (95% CI2.4-15.7, p=0.01). Step 3: this finding was upheld and no other emerged when exposure was defined as the drug or any of its active metabolites. This step was performed because hepatic catabolism of BDZs leads

  12. Anesthesia for Congenital Esoshageal Atresia%先天性食管闭锁的麻醉

    Institute of Scientific and Technical Information of China (English)

    王大柱; 郭奉玲; 孙乃珠; 李凤荣; 王秀珍

    1988-01-01

    The artical reports the experience of anesthesia for 40 cases with congenital esophageal atresia.Most of the infants had a low birth weight and had complication of pneumonia or other malformations,so the anesthesia was very difficult. The patients were intubated after acetylpromazine and promethazine injected intramuseulary.Anesthesia was maintained by ketamine,diazepam or dolantine.the respiration was assisted by the modifications of the"T" piece system. An appropriate anesthesia was directly related to the postoperative survival rate.No death and no su ch side effects as laryngospasm or asphyxia resulted from ketamine or diazepare.The function of cardiovascular system was stzble.The infants were conscious and could be extu bated soon after operation.%本文报道40例先天性食管闭锁手术的麻醉,其中早产儿占50%.全部患儿合并肺炎,50%以上患儿还合并有多种畸形.采用清醒插管,氯胺酮,安定麻醉、应用根据T管原理改良的小儿呼吸囊管理呼吸,取得了良好效果,麻醉、手术过程平稳,循环稳定,术终苏醒迅速.

  13. Surgical Repair of Congenital Recto-Vaginal Fistula with Atresia Ani in a Cow Calf

    Directory of Open Access Journals (Sweden)

    Abdul Shakoor, Sayyed Aun Muhammad, Muhammad Younus and Muhammd Kashif

    2012-05-01

    Full Text Available A three days old cross bred cow calf affected with recto-vaginal fistula along with atresia ani and fusion of vulvular lips was presented to the Veterinary Teaching Hospital, College of Veterinary and Animal Sciences, Jhang. Emergency surgery was done after the infiltration of local anesthesia at the approximated site of anus. Blind rectal pouch was incised to suture it with the skin by using black breaded silk #1. A 3 inches long incision was made at the site of vulvular fusion to restore the normal genitalia. After exposing the defective site, 4 inches long slit causing direct communication between rectum and vagina (Recto-vaginal fistula was obliterated with chromic cat gut # 1. Having washed with normal saline, Neomycine Sulphate ointment was smeared twice a day at the newly constructed anal opening for two weeks. A course of antibiotic consisting of penicillin and streptomycin was administered intramuscularly for five days. The sutures were removed on 14th post-operative day. Animal was recovered uneventfully without recurrence of any defect up to follow up of 2 months.

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

    Institute of Scientific and Technical Information of China (English)

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

    2014-01-01

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

  15. A Taenia crassiceps metacestode factor enhances ovarian follicle atresia and oocyte degeneration in female mice.

    Science.gov (United States)

    Solano, S; Zepeda, N; Copitin, N; Fernandez, A M; Tato, P; Molinari, J L

    2015-01-01

    The histopathological effects of Taenia crassiceps infection or T. crassiceps metacestode factor inoculation on the mouse ovary were determined using six female mice in three groups: infected mice, mice inoculated with the metacestode factor and control mice. The control group was subcutaneously inoculated with healthy peritoneal fluid. The infected group was intraperitoneally inoculated with 40 T. crassiceps metacestodes, and the metacestode factor group was subcutaneously inoculated with T. crassiceps metacestode factor (MF). Light and electron microscopy and TUNEL (terminal deoxynucleotidyl transferase (TdT)-mediated dUTP nick end labelling) assays revealed a significant increase in ovarian follicular atresia (predominantly in antral/preovulatory stages of development), oocyte degeneration (P< 0.05), and a decrease in the amount of corpus luteum in follicles of mice infected and inoculated with MF compared with the control group. Significant abnormalities of the granulosa cells and oocytes of the primordial, primary and secondary ovarian follicles occurred in both treated mouse groups (P< 0.05) compared with no degeneration in the control group. These pathological changes in female mice either infected with T. crassiceps metacestodes or inoculated with T. crassiceps MF may have consequences for ovulation and fertility.

  16. Biliary Cast Syndrome: Hepatic Artery Resistance Index, Pathological Changes, Morphology and Endoscopic Therapy

    Directory of Open Access Journals (Sweden)

    Hu Tian

    2015-01-01

    Full Text Available Background: Biliary cast syndrome (BCS was a postoperative complication of orthotopic liver transplantation (OLT, and the reason for BSC was considered to relate with ischemic type biliary lesions. This study aimed to evaluate the relationship between BCS following OLT and the hepatic artery resistance index (HARI, and to observe pathological changes and morphology of biliary casts. Methods: Totally, 18 patients were diagnosed with BCS by cholangiography following OLT using choledochoscope or endoscopic retrograde cholangiopancreatography. In addition, 36 patients who did not present with BCS in the corresponding period had detectable postoperative HARI on weeks 1, 2, 3 shown by color Doppler flow imaging. The compositions of biliary casts were analyzed by pathological examination and scanning electron microscopy. Results: HARI values of the BCS group were significantly decreased as compared with the non-BCS group on postoperative weeks 2 and 3 (P 1 (OR = 1.300; 1.223; and 1.889, respectively. The OR of HARI 3 was statistically significant (OR = 1.889; 95% confidence interval = 1.166-7.490; P = 0.024. The compositions of biliary casts were different when bile duct stones were present. Furthermore, vascular epithelial cells were found by pathological examination in biliary casts. Conclusions: HARI may possibly serve as an independent risk factor and early predictive factor of BCS. Components and formation of biliary casts and bile duct stones are different.

  17. A Double-Layered Covered Biliary Metal Stent for the Management of Unresectable Malignant Biliary Obstruction: A Multicenter Feasibility Study

    Science.gov (United States)

    Park, Jin-Seok; Jeong, Seok; Lee, Don Haeng; Moon, Jong Ho; Lee, Kyu Taek; Dong, Seok Ho

    2016-01-01

    Background/Aims The covered self-expandable metal stent (CMS) was developed to prevent tumor ingrowth-induced stent occlusion during the treatment of malignant biliary obstruction. However, complications such as cholecystitis, pancreatitis, and stent migration can occur after the endoscopic insertion of CMSs. The aim of the present study was to assess the efficacy and safety of a double-layered CMS (DCMS) for the management of malignant bile duct obstruction. Methods DCMSs were endoscopically introduced into 59 patients with unresectable malignant extrahepatic biliary obstruction at four tertiary referral centers, and the patient medical records were retrospectively reviewed. Results Both the technical and functional success rates were 100%. Procedure-related complications including pancreatitis, cholangitis, stent migration, and liver abscess occurred in five patients (8.5%). The median follow-up period was 265 days (range, 31 to 752 days). Cumulative stent patency rates were 68.2% and 40.8% at 6 and 12 months, respectively. At the final follow-up, the rate of stent occlusion was 33.9% (20/59), and the median stent patency period was 276 days (range, 2 to 706 days). Conclusions The clinical outcomes of DCMSs were comparable to the outcomes previously reported for CMSs with respect to stent patency period and complication rates. PMID:27172927

  18. Ultrasound-guided percutaneous transhepatic biliary drainage: Experiences in 146 patients

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jai Keun [Sohwa Children' s Hospital, Seoul(Korea, Republic of); Yu, Jeong Sik; Kim, Ki Whang; Chung, Soo Yoon; Jeong, Mi Gyoung [Yonsei University College of Medicine, Seoul (Korea, Republic of); Choi, Deuk Lin; Kwon, Gui Hyang; Lee, Hae Kyung [Soonchunhyang University College of Medicine, Seoul (Korea, Republic of)

    1999-03-15

    Percutaneous biliary drainage is an important technique for palliative therapy of obstructive biliary disease and diagnostic information. The purpose of this study is to review and evaluate the experiences of ultrasound-guided percutaneous transhepatic biliary drainage. Ultrasound-guided percutaneous transhepatic biliary drainage was performed on 146 occasions in 134 patients. The causes of biliary obstruction were: benign diseases (19 cases, 14.2%) such as bile duct stones or stricture, cholangiocarcinoma (37 cases, 27.6%), pancreatic carcinoma (35 cases, 26.1%), metastasis (22 cases, 16.5%), gall bladder cancer (14 cases, 10.4%), ampulla of Vater cancer (4 cases, 3.0%), hepatocellular carcinoma (3 cases, 2.2%). Retrospectively reviewing medical records, we found out frequency of external or external/internal biliary drainages, puncture of left or right hepatic duct, and presence of bileinfection. Ultrasound-guided percutaneous transhepatic biliary drainage was compared with conventional biliary drainage of previous reports on the basis of frequency of complications. External (124 procedures, 84.9%) and external/internal biliary drainage (22 procedures, 15.1%) were carried out by puncture of dilated right (59.6%) or left (40.4%) intrahepatic duct. Sixty-nine complications occurred in 47 patients. Catheter related complications (33/69, 47.8%) were most common: catheter dislodgement (17/69, 24.6%), malfunction (9/69, 13.1%), leakage (7/69, 10.1%). Other minor complications such as simple fever (16/69, 23.2%), cholangitis (7/69, 10.1%), hemobilia (4/69, 5.8%), biloma (2/69, 2.9%) and wound infection (1/69, 1.5%) occurred. Major complications including sepsis (4/69, 5.8%) and bile peritonitis (2/69, 2.9%) were also noted. Puncture-related complications such as hemobilia, biloma and bile peritonitis occurred in 8 cases (5.5%). Comparing with conventional X-ray guided drainage, ultrasound-guided percutaneous transhepatic biliary drainage is a safe procedure for

  19. Biliary cystadenoma with mesenchymal stroma: Report of a case and review of the literature

    Institute of Scientific and Technical Information of China (English)

    Andreas Manouras; Haridimos Markogiannakis; Emmanuel Lagoudianakis; Vangelogiannis Katergianna

    2006-01-01

    Biliary cystadenomas are rare, cystic neoplasms of the biliary ductal system that usually occur in middleaged women. They cannot be safely differentiated from cystadenocarcinomas before operation and should always be considered for resection. Cystadenomas have a strong tendency to recur, particularly following incomplete excision, and a potential of malignant transformation.Therefore, complete resection is the therapy of choice and thorough histopathologic evaluation is imperative. A case of benign biliary cystadenoma with mesenchymal stroma is presented along with a review of the relative literature addressing the clinical presentation, histology,histogenesis, differential diagnosis, imaging features,treatment and prognosis of this interesting and rare entity.

  20. Endoscopic management of malignant biliary stenosis: update and highlights for standard clinical practice

    Directory of Open Access Journals (Sweden)

    María José Domper-Arnal

    Full Text Available The present review describes the various indications of biliary stent placement in patients with biliary malignancies. It deals in depth with biliary accesses and their effectiveness, as well as with the use of different stents according to lesion type and expected patient survival. For liver hilum lesions, which are somewhat more complex, the usefulness of and need for unilateral or bilateral drainage is assessed, as it is the most appropriate method. All in all, this is an up-to-date literature review that may help clinicians in their daily decision-making, as well as to improve and optimize patient outcomes.