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Sample records for biliary atresia

  1. 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

  2. Biliary atresia Atresia de las vías biliares.

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    Pastor Thomas Olivares

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

  3. ETIOLOGY, PATHOGENESIS AND MORPHOLOGY OF BILIARY ATRESIA

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    O. E. Iryshkin

    2012-01-01

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

  4. Congenital biliary atresia: liver injury begins at birth

    DEFF Research Database (Denmark)

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

    2009-01-01

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

  5. Ultrasonographic findings of type IIIa biliary atresia

    International Nuclear Information System (INIS)

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

  6. European biliary atresia registries: summary of a symposium

    DEFF Research Database (Denmark)

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

    2008-01-01

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

  7. Biliary atresia: Clinical advances and perspectives.

    Science.gov (United States)

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

    2016-06-01

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

  8. Pregnancy Complicated by Portal Hypertension Secondary to Biliary Atresia

    OpenAIRE

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

    2013-01-01

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

  9. Anaesthesia for biliary atresia and hepatectomy in paediatrics

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    Rebecca Jacob

    2012-01-01

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

  10. Pregnancy Complicated by Portal Hypertension Secondary to Biliary Atresia

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    O. E. O'Sullivan

    2013-01-01

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

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

    NARCIS (Netherlands)

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

    2012-01-01

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

  12. A study of associated congenital anomalies with biliary atresia

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    Lucky Gupta

    2016-01-01

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

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

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    Alice Faure

    2015-01-01

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

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

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    Carlos O. Kieling

    2008-10-01

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

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

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

    2006-08-15

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

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

    International Nuclear Information System (INIS)

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

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

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    Morita, Keiichi; Fukuzawa, Hiroaki; Maeda, Kosaku

    2015-12-01

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

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

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    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.

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

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    Thais Costa Nascentes QUEIROZ

    2014-03-01

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

  20. Endotoxin and CD14 in the progression of biliary atresia

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    Chen Ching-Mei

    2010-12-01

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

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

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

    2009-01-01

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

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

    OpenAIRE

    Mack, Cara L.

    2015-01-01

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

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

    NARCIS (Netherlands)

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

    2011-01-01

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

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

    NARCIS (Netherlands)

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

    2011-01-01

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

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

    Science.gov (United States)

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

    2007-09-01

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

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

    International Nuclear Information System (INIS)

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

  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

    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...

  8. 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.

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

    International Nuclear Information System (INIS)

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

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

    OpenAIRE

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

    2016-01-01

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

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

    OpenAIRE

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

    2013-01-01

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

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

    OpenAIRE

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

    1988-01-01

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

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

    OpenAIRE

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

    2008-01-01

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

  14. 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

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

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    Jiexiong Feng

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

  16. 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.

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

    Science.gov (United States)

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

    2013-07-01

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

  18. 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)

  19. 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.

  20. B cell deficient mice are protected from biliary obstruction in the rotavirus-induced mouse model of biliary atresia.

    Directory of Open Access Journals (Sweden)

    Amy G Feldman

    Full Text Available A leading theory regarding the pathogenesis of biliary atresia (BA is that bile duct injury is initiated by a virus infection, followed by an autoimmune response targeting bile ducts. In experimental models of autoimmune diseases, B cells have been shown to play an important role. The aim of this study was to determine the role of B cells in the development of biliary obstruction in the Rhesus rotavirus (RRV-induced mouse model of BA. Wild-type (WT and B cell-deficient (Ig-α(-/- mice received RRV shortly after birth. Ig-α(-/- RRV-infected mice had significantly increased disease-free survival rate compared to WT RRV-infected BA mice (76.8% vs. 17.5%. In stark contrast to the RRV-infected BA mice, the RRV-infected Ig-α(-/- mice did not have hyperbilirubinemia or bile duct obstruction. The RRV-infected Ig-α(-/- mice had significantly less liver inflammation and Th1 cytokine production compared to RRV-infected WT mice. In addition, Ig-α(-/- mice had significantly increased numbers of regulatory T cells (Tregs at baseline and after RRV infection compared to WT mice. However, depletion of Tregs in Ig-α(-/- mice did not induce biliary obstruction, indicating that the expanded Tregs in the Ig-α(-/- mice were not the sole reason for protection from disease. Conclusion : B cell deficient Ig-α(-/- mice are protected from biliary obstruction in the RRV-induced mouse model of BA, indicating a primary role of B cells in mediating disease pathology. The mechanism of protection may involve lack of B cell antigen presentation, which impairs T-cell activation and Th1 inflammation. Immune modulators that inhibit B cell function may be a new strategy for treatment of BA.

  1. Biliary Atresia

    Science.gov (United States)

    ... their structure. The procedure is performed in a health care provider’s office, outpatient center, or hospital by a specially trained technician, and the images are interpreted a radiologist. Anesthesia is not needed, but sedation may be ...

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

    Directory of Open Access Journals (Sweden)

    Nikou Fotouhi

    2014-01-01

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

  3. Rotavirus replication in the cholangiocyte mediates the temporal dependence of murine biliary atresia.

    Science.gov (United States)

    Mohanty, Sujit K; Donnelly, Bryan; Bondoc, Alexander; Jafri, Mubeen; Walther, Ashley; Coots, Abigail; McNeal, Monica; Witte, David; Tiao, Gregory M

    2013-01-01

    Biliary atresia (BA) is a neonatal disease that results in obliteration of the biliary tree. The murine model of BA, which mirrors the human disease, is based upon infection of newborn mice with rhesus rotavirus (RRV), leading to an obstructive cholangiopathy. The purpose of this study was to characterize the temporal relationship between viral infection and the induction of this model. BALB/c mice were infected with RRV on day of life (DOL) 0, 3, 5, and 7. Groups were characterized as early-infection (infection by DOL 3) or late-infection (infection after DOL 5). Early RRV infection induced symptoms in 95% of pups with a mortality rate of 80%. In contrast, late infection caused symptoms in only 50% of mice, and 100% of pups survived. The clinical findings correlated with histological analysis of extrahepatic biliary trees, cytokine expression, and viral titers. Primary murine cholangiocytes isolated, cultured, and infected with RRV yielded higher titers of infectious virus in those harvested from DOL 2 versus DOL 9 mice. Less interferon alpha and beta was produced in DOL 2 versus DOL 9 RRV infected primary cholangiocytes. Injection of BALB/c interferon alpha/beta receptor knockout (IFN-αβR(-/-)) pups at DOL 7 showed increased symptoms (79%) and mortality (46%) when compared to late infected wild type mice. In conclusion, the degree of injury sustained by relatively immature cholangiocytes due to more robust RRV replication correlated with more severe clinical manifestations of cholangiopathy and higher mortality. Interferon alpha production by cholangiocytes appears to play a regulatory role. These findings confirm a temporal dependence of RRV infection in murine BA and begin to define a pathophysiologic role of the maturing cholangiocyte. PMID:23844248

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

    DEFF Research Database (Denmark)

    Hasselt, P.M. van; Koning, T.J. de; Vries, E. de;

    2008-01-01

    OBJECTIVE: Newborns routinely receive vitamin K to prevent vitamin K deficiency bleeding. The efficacy of oral vitamin K administration may be compromised in infants with unrecognized cholestasis. We aimed to compare the risk of vitamin K deficiency bleeding under different prophylactic regimens...... in infants with biliary atresia. PATIENTS AND METHODS: From Dutch and Danish national biliary atresia registries, we retrieved infants who were either breastfed and received 1 mg of oral vitamin K at birth followed by 25 microg of daily oral vitamin K prophylaxis (Netherlands, 1991-2003), 2 mg of oral...... vitamin K at birth followed by 1 mg of weekly oral prophylaxis (Denmark, 1994 to May 2000), or 2 mg of intramuscular prophylaxis at birth (Denmark, June 2000-2005) or were fed by formula. We determined the absolute and relative risk of severe vitamin K deficiency and vitamin K deficiency bleeding...

  5. Alagille Syndrome Candidates for Liver Transplantation: Differentiation from End-Stage Biliary Atresia Using Preoperative CT.

    Directory of Open Access Journals (Sweden)

    Sook Min Hwang

    Full Text Available To compare preoperative CT findings before liver transplantation between patients with Alagille syndrome (AGS and those with end-stage biliary atresia (BA.The institutional review board approved this retrospective study. Eleven children with AGS (median age, 19.0 ± 13.0 months; male to female ratio, 3:8 and 109 children with end-stage BA (median age, 17.9 ± 25.8 months; male to female ratio, 37:72 who underwent abdomen CT as candidates for liver transplant were included. CT images were reviewed focusing on hepatic parenchymal changes, vascular changes, presence of focal lesions, and signs of portal hypertension.Hepatic parenchymal changes were present in 27% (3/11 of AGS patients and 100% (109/109 of end-stage BA patients (P < .001. The hepatic artery diameter was significantly smaller (1.9 mm versus 3.6 mm, P = 008, whereas portal vein diameter was larger (6.8 mm versus 5.0 mm, P < .001 in patients with AGS compared with patients with end-stage BA. No focal lesion was seen in patients with AGS, whereas 44% (48/109 of patients with end-stage BA had intrahepatic biliary cysts (39%, 43/109 and hepatic tumors (8%, 9/109 (P = .008. Splenomegaly was commonly seen in both groups (P = .082, and ascites (9% [1/11] versus 50% [54/109], P = .010 and gastroesophageal varix (0% [0/11] versus 80% [87/109], P < .001 were less common in patients with AGS than in patients with end-stage BA.Fibrotic or cirrhotic changes of the liver, presence of focal lesions, and relevant portal hypertension were less common in patients with AGS than in patients with end-stage BA.

  6. “Parenteral Nutrition Supplementation in Biliary Atresia Patients Listed for Liver Transplantation”

    Science.gov (United States)

    Sullivan, Jillian S; Sundaram, Shikha S; Pan, Zhaoxing; Sokol, Ronald J

    2011-01-01

    Objective To determine the impact of parenteral nutrition (PN) on outcomes in biliary atresia (BA) patients listed for liver transplantation (LTx). Study Design We retrospectively reviewed charts of all BA patients from 1990 through 2010 at our institution, s/p hepatoportoenterostomy, ≤ 36 months old, and listed for LTx. Initiation of PN was based on clinical indications. Results 25 PN and 22 non-PN subjects (74% female) were studied. Median PN initiation age was 7.7 months, mean duration 86 days, and mean PN energy supplied 77 kcal/kg/day. Prior to PN, triceps skinfold thickness (TSF) and mid-arm circumference (MAC) Z-scores were decreasing. After PN, TSF (p=0.003) and MAC (p<0.0001) improved significantly. The PN group had lower MAC and TSF than non-PN at time of LTx listing. Between listing and LTx, MAC and TSF improved in PN and worsened in non-PN such that both groups had the same Z-scores at LTx. PN group had a higher incidence of GI bleeding and ascites pre-LTx, but there was no difference in pre-LTx bacteremia, and post-LTx days in ICU and patient or graft survival. Conclusions PN improves nutritional status in malnourished BA patients awaiting LTx, which is associated with post-LTx outcomes comparable to those not requiring PN. PMID:21987426

  7. 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.

  8. 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.

  9. 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.

  10. 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.

  11. 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.

  12. Distinct Plasma Bile Acid Profiles of Biliary Atresia and Neonatal Hepatitis Syndrome.

    Science.gov (United States)

    Zhou, Kejun; Wang, Jun; Xie, Guoxiang; Zhou, Ying; Yan, Weihui; Pan, Weihua; Che, Yanran; Zhang, Ting; Wong, Linda; Kwee, Sandi; Xiao, Yongtao; Wen, Jie; Cai, Wei; Jia, Wei

    2015-11-01

    Biliary atresia (BA) is a severe chronic cholestasis disorder of infants that leads to death if not treated on time. Neonatal hepatitis syndrome (NHS) is another leading cause of neonatal cholestasis confounding the diagnosis of BA. Recent studies indicate that altered bile acid metabolism is closely associated with liver injury and cholestasis. In this study, we systematically measured the bile acid metabolome in plasma of BA, NHS, and healthy controls. Liver bile acids were also measured using biopsy samples from 48 BA and 16 NHS infants undergoing operative cholangiography as well as 5 normal adjacent nontumor liver tissues taken from hepatoblastoma patients as controls. Both BA and NHS samples had significantly elevated bile acid levels in plasma compared to normal controls. BA patients showed a distinct bile acid profile characterized by the higher taurochenodeoxycholic acid (TCDCA) level and lower chenodeoxycholic acid (CDCA) level than those in NHS patients. The ratio of TCDCA to CDCA in plasma was significantly higher in BA compared to healthy infants (p BSEP), and multidrug resistant protein 3 (MDR3) in BA compared to NHS. Taken together, the plasma bile acid profiles are distinct in BA, NHS, and normal infants, as characterized by the ratio of TCDCA/CDCA differentially distributed among the three groups of infants. PMID:26449593

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

    Directory of Open Access Journals (Sweden)

    Jane Maestri Brittain

    2016-01-01

    Full Text Available Biliary atresia (BA is an obliterative cholangiopathy affecting 1:10.000–14.000 of newborns. Infants with Biliary Atresia Splenic Malformation syndrome (BASM are a subgroup of BA patients with additional congenital anomalies. Untreated the disease will result in fatal liver failure within the first years of life. Kasai portoenterostomy restores bile flow and delay the progressive liver damage thereby postponing liver transplantation. An early diagnosis is of most importance to ensure the effectiveness of the operation. The 99mTc-Mebrofenin hepatobiliary scintigraphy is part of the diagnostic strategy when an infant presents jaundice due to conjugated hyperbilirubinemia (>20 µmol/L total bilirubin of which 20% is conjugated with its high sensitivity of 97%–100% in refuting BA. Rapid extraction of tracer by the liver and no visible tracer in the small bowl after 24 h is indicative of BA. Laparotomy with antegrade cholangiography is then performed giving the final diagnosis when the remains of the obliterated biliary tree are revealed in the case of BA. We present a case demonstrating some of the challenges of interpreting the 99mTc-Mebrofenin hepatobiliary scintigraphy in an infant with BASM and stress the importance that the 99mTc-Mebrofenin hepatobiliary scintigraphy is part of a spectrum of imaging modalities in diagnosing BA.

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

    Science.gov (United States)

    Maestri Brittain, Jane; Borgwardt, Lise

    2016-01-01

    Biliary atresia (BA) is an obliterative cholangiopathy affecting 1:10.000-14.000 of newborns. Infants with Biliary Atresia Splenic Malformation syndrome (BASM) are a subgroup of BA patients with additional congenital anomalies. Untreated the disease will result in fatal liver failure within the first years of life. Kasai portoenterostomy restores bile flow and delay the progressive liver damage thereby postponing liver transplantation. An early diagnosis is of most importance to ensure the effectiveness of the operation. The (99m)Tc-Mebrofenin hepatobiliary scintigraphy is part of the diagnostic strategy when an infant presents jaundice due to conjugated hyperbilirubinemia (>20 µmol/L total bilirubin of which 20% is conjugated) with its high sensitivity of 97%-100% in refuting BA. Rapid extraction of tracer by the liver and no visible tracer in the small bowl after 24 h is indicative of BA. Laparotomy with antegrade cholangiography is then performed giving the final diagnosis when the remains of the obliterated biliary tree are revealed in the case of BA. We present a case demonstrating some of the challenges of interpreting the (99m)Tc-Mebrofenin hepatobiliary scintigraphy in an infant with BASM and stress the importance that the (99m)Tc-Mebrofenin hepatobiliary scintigraphy is part of a spectrum of imaging modalities in diagnosing BA. PMID:26838802

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2015-12-15

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

  16. Supersonic shearwave elastography in the assessment of liver fibrosis for postoperative patients with biliary atresia

    Science.gov (United States)

    Chen, Shuling; Liao, Bing; Zhong, Zhihai; Zheng, Yanling; Liu, Baoxian; Shan, Quanyuan; Xie, Xiaoyan; Zhou, Luyao

    2016-01-01

    To explore an effective noninvasive tool for monitoring liver fibrosis of children with biliary atresia (BA) is important but evidences are limited. This study is to investigate the predictive accuracy of supersonic shearwave elastography (SSWE) in liver fibrosis for postoperative patients with BA and to compare it with aspartate aminotransferase to platelet ratio index (APRI) and fibrosis-4 (FIB-4). 24 patients with BA received SSWE and laboratory tests before scheduled for liver biopsy. Spearman rank coefficient and receiver operating characteristic (ROC) were used to analyze data. Metavir scores were F0 in 3, F1 in 2, F2 in 4, F3 in 7 and F4 in 8 patients. FIB-4 failed to correlate with fibrosis stage. The areas under the ROC curves of SSWE, APRI and their combination were 0.79, 0.65 and 0.78 for significant fibrosis, 0.81, 0.64 and 0.76 for advanced fibrosis, 0.82, 0.56 and 0.84 for cirrhosis. SSWE values at biopsy was correlated with platelet count (r = −0.426, P = 0.038), serum albumin (r = −0.670, P < 0.001), total bilirubin (r = 0.419, P = 0.041) and direct bilirubin levels (r = 0.518, P = 0.010) measured at 6 months after liver biopsy. Our results indicate that SSWE is a more promising tool to assess liver fibrosis than APRI and FIB-4 in children with BA. PMID:27511435

  17. 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.

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

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    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.

  19. Infant Stool Color Card Screening Helps Reduce the Hospitalization Rate and Mortality of Biliary Atresia

    Science.gov (United States)

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

    2016-01-01

    Abstract 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

  20. 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

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

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    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.

  2. 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

  3. Prognostic impact of hepatobiliary scintigraphy in diagnosis and postoperative follow-up of newborns with biliary atresia

    International Nuclear Information System (INIS)

    Aim: To investigate the prognostic relevance of hepatobiliary scintigraphy (HBS) in newborns suffering from biliary atresia (BA) for establishing the primary diagnosis and in the postoperative follow-up after portoenterostomy (Kasai). Methods: Twenty newborns with direct hyperbilirubinemia and 6 children after operative treatment of BA (Kasai) underwent HBS with Tc-99m-DEIDA. In patients without intestinal drainage, hepatocellular extraction was estimated visually and calculated semiquantitatively by means of liver/heart-ratio 5 min p.i. Results: 10/20 patients with hyperbilirubinemia did not display biliary drainage; 6 had BA, 3 intrahepatic hypoplasia, and one showed a bile plug syndrom. 4/6 with BA but none of the 4 children with diagnoses other than BA presented with a good extraction. All of the 4 children with BA, who had either pre- or postoperatively a bad extraction, needed liver transplantation due to liver failure. Both of the two newborns with BA and favourable outcome after Kasai had a good extraction in the preoperative HBS and demonstrated good intestinal drainage in the postoperative scan. Conclusion: HBS rules out BA with high accuracy by demonstrating drainage of bile into the intestine. In newborns without drainage a good extraction favours the diagnosis of BA. In newborns with BA a bad extraction seems to indicate a poor postoperative prognosis after Kasai operation. HBS might therefore help to select those children who will not benefit from portoenterostomy. Postoperatively, HBS can easily and quickly confirm the successful hepatobiliary anastomosis by demonstrating biliary drainage into the intestine. (orig.)

  4. 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.)

  5. 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.

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

    OpenAIRE

    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 prese...

  7. Hepatic clearance of {sup 99m}Tc-GSA in cases of postoperative biliary atresia. A retrospective comparison with hepatic clearance of {sup 99m}Tc-PMT

    Energy Technology Data Exchange (ETDEWEB)

    Yamazaki, Tetsuro; Yamada, Shogo [Tohoku Univ., Sendai (Japan). School of Medicine; Maruoka, Shin

    1998-11-01

    There are a few reports about usefulness of {sup 99m}Tc-GSA liver scintigraphy (GSA imaging) for the evaluation of postoperative state of biliary atresia. However, comparison of significance for the disease between GSA imaging and {sup 99m}Tc-PMT hepatobiliary imaging have not been reported. Fifty-six GSA imagings for 41 postoperative biliary atresia patients were reviewed and their scintigraphic findings and the functional parameters were compared with those obtained from {sup 99m}Tc-PMT hepatobiliary imaging. In quantitative analysis, although the hepatic clearance of both tracers calculated by graphical analysis (Patlak plot) were well correlated in most cases, a discrepancy was observed in several cases with jaundice. The authors conclude that the hepatic clearance of {sup 99m}Tc-GSA is a more accurate index of the hepatic reserve than that of {sup 99m}Tc-PMT which can be influenced by hyperbilirubinemia and that the combination of both examinations is helpful to evaluate development of liver injury in postoperative biliary atresia patients. (author)

  8. [Hepatic clearance of 99mTc-GSA in cases of postoperative biliary atresia--a retrospective comparison with hepatic clearance of 99mTc-PMT].

    Science.gov (United States)

    Yamazaki, T; Maruoka, S; Yamada, S

    1998-11-01

    There are a few reports about usefulness of 99mTc-GSA liver scintigraphy (GSA imaging) for the evaluation of postoperative state of biliary atresia. However, comparison of significance for the disease between GSA imaging and 99mTc-PMT hepatobiliary imaging have not been reported. Fifty-six GSA imagings for 41 postoperative biliary atresia patients were reviewed and their scintigraphic findings and the functional parameters were compared with those obtained from 99mTc-PMT hepatobiliary imaging. In quantitative analysis, although the hepatic clearance of both tracers calculated by graphical analysis (Patlak plot) were well correlated in most cases, a discrepancy was observed in several cases with jaundice. The authors conclude that the hepatic clearance of 99mTc-GSA is a more accurate index of the hepatic reserve than that of 99mTc-PMT which can be influenced by hyperbilirubinemia and that the combination of both examinations is helpful to evaluate development of liver injury in postoperative biliary atresia patients. PMID:9916401

  9. Hepatic tumours in children with biliary atresia: Single-centre experience in 13 cases and review of the literature

    International Nuclear Information System (INIS)

    Aim: To establish the risks of developing of hepatic tumours and to investigate their clinical and imaging findings in children with biliary atresia (BA) after Kasai portoenterostomy (Kasai). Materials and methods: Among 157 children who had undergone Kasai for BA over an 18 year period, patients who had newly developed hepatic tumours were identified. Patient demographics, clinical features, and imaging findings were retrospectively reviewed. Results: Three male and 10 female patients (mean age 3.9 years) all (8%, of 157) had single hepatic tumours, which were confirmed in 10 explanted and three non-explanted livers. Ten (77%) were benign and three (23%) were malignant. Of the benign hepatic tumours, focal nodular hyperplasia (FNH; n = 6) was the most common, followed by regenerative nodules (n = 3) and adenoma (n = 1). All FNH appeared in young children <1 year of age and showed a subcapsular location, bulging contour, and lack of central scar. Malignant tumours included two hepatocellular carcinomas and one cholangiocarcinoma. Conclusion: Hepatic tumours developed in approximately 8% of children with BA after Kasai. Although benign tumours, including FNHs and regenerative nodules, were more common than malignant tumours, screening with alpha-foetoprotein (AFP) levels and regular imaging studies are the mainstay of malignant tumour detection

  10. 胆道闭锁早期筛查现状及对策%The early screening and countermeasures of biliary atresia

    Institute of Scientific and Technical Information of China (English)

    詹江华

    2015-01-01

    It is generally accepted that Kasai operation is the best treatment for biliary atresia (BA). The implementation of early screen plan improves the age threshold at the suitable time point of Kasai operation. This is the key issue to improve the survival rate of autologous liver in biliary atresia. The monitoring of stool color card, B-ultrasonography and serum con⁃jugated bilirubin can be used for preliminary seeming biliary atresia. It is very important to improve the ability of basic unit medical staff for major improvements to the understanding of the significance of biliary atresia. In some country and district, the local government has put the screen list for BA into healthcare booklet for children. Thus, it has a significant impact of early screen plan on the diagnosis and treatment of BA, and it can improve the BA autologous liver survival time. So far, there is still no BA early screen plan in the nationwide. We should pay attention to BA, and establish early screen system in different local health unit quickly.%目前已经公认Kasai手术是治疗胆道闭锁的最佳方案,推行早期筛查计划可以使得胆道闭锁患儿在合适的时间点完成Kasai手术,这是提高胆道闭锁自体肝生存时间的关键所在。粪卡、B超检查、血清直接胆红素水平监测可以用于初步筛查胆道闭锁,而基层医疗单位相关人员的宣教工作对于提高胆道闭锁的认识意义重大。有些国家和地区已将相关的筛查项目放入新生儿健康手册中,且对于胆道闭锁的治疗有较大的帮助,大大提高其自体肝生存时间。我国尚没有在国家层面上推行胆道闭锁的早期筛查项目,应关注胆道闭锁,尽快建立胆道闭锁筛查体系。

  11. 胆道闭锁肝纤维化研究进展%Advances in the research of liver fibrosis in biliary atresia

    Institute of Scientific and Technical Information of China (English)

    王培; 冯杰雄

    2015-01-01

    胆道闭锁是一种婴幼儿肝内外胆管阻塞和肝脏进行性纤维化的疾病,病因和发病机制尚不明确,若不加以干预,多数患儿于1岁左右死亡。本文回顾了近年来新生儿及动物模型中胆道闭锁肝纤维化研究进展,提出了尚未解决的问题和今后的研究方向。%Biliary atresia is an infant disease characterized by obstruction of the internal and ex-ternal bile duct and progressive fibrosing process of the liver. Its etiology and pathogenesis remains un-clear and most infants would die in one year if thereˊs no medical intervention. In this article,we reviewed recent research progress of liver fibrosis in biliary atresia in infants and animal models,which proposed some unsettled problems and research direction in the future.

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

    International Nuclear Information System (INIS)

    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/mm2 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 (R2 = 0.398, p = 0.024 and R2 = 0.628, p < 0.001, respectively). Right LTPARs may be used for long-term follow-up of cirrhosis severity in BA patients. (orig.)

  13. 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.)

  14. 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

  15. HMGB1-promoted and TLR2/4-dependent NK cell maturation and activation take part in rotavirus-induced murine biliary atresia.

    Directory of Open Access Journals (Sweden)

    Yinrong Qiu

    2014-03-01

    Full Text Available Recent studies show that NK cells play important roles in murine biliary atresia (BA, and a temporary immunological gap exists in this disease. In this study, we found high-mobility group box-1 (HMGB1 and TLRs were overexpressed in human and rotavirus-induced murine BA. The overexpressed HMGB1 released from the nuclei of rotavirus-infected cholangiocytes, as well as macrophages, activated hepatic NK cells via HMGB1-TLRs-MAPK signaling pathways. Immature NK cells had low cytotoxicity on rotavirus-injured cholangiocytes due to low expression of TLRs, which caused persistent rotavirus infection in bile ducts. HMGB1 up-regulated the levels of TLRs of NK cells and promoted NK cell activation in an age-dependent fashion. As NK cells gained increasing activation as mice aged, they gained increasing cytotoxicity on rotavirus-infected cholangiocytes, which finally caused BA. Adult NK cells eliminated rotavirus-infected cholangiocytes shortly after infection, which prevented persistent rotavirus infection in bile ducts. Moreover, adoptive transfer of mature NK cells prior to rotavirus infection decreased the incidence of BA in newborn mice. Thus, the dysfunction of newborn NK cells may, in part, participate in the immunological gap in the development of rotavirus induced murine BA.

  16. Esophageal atresia

    Science.gov (United States)

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

  17. 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

  18. 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

  19. 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

  20. Oesophageal atresia

    DEFF Research Database (Denmark)

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

    2012-01-01

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

  1. Pulmonary Atresia

    Science.gov (United States)

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

  2. The relationship between transcutaneous bilirubin changes after surgery and the prognosis in biliary atresia%测定胆道闭锁术后皮肤胆红素动态变化与预后的关系

    Institute of Scientific and Technical Information of China (English)

    王文晓; 李龙; 张金山; 刘树立; 谢华伟; 明安晓; 崔龙; 周峻

    2011-01-01

    Objective To study the relationship between the post-op transcutaneous bilirubin changes after Surgery and the prognosis in children with biliary atresia. Methods Between July 2009to December 2009,52 children( 19 males and 33 female, age: 36 d~304 d, the mean age 83d, including 5cholestasis and 47 biliary atresia)with jaundice and 59 non-jaundiced children's (28 males and 31 females,aged 26 d~200 d and the mean age 119 d) were recruited for the study. The transcutaneous bilirubin and total serum bilirubin of children with jaundice and non-jaundice were measured at the same time. Twenty-three children with biliary atresia (8 males and 15 females, aged 36 d~ 127 d and the mean age 63 d) underwent Kasai operation. The children were divided into good or poor prognosis groups according to the serum bilirubin levels of 20 μmol/L three months after operation. Measurements were compared between the two groups during hospital stay. Results Linear regression oftranscutaneous bilirubin (x) correlated with that of serum bilirubin (y) (y = 0. 945x - 46. 273, P<0. 05). The 95% confidence interval of transcutaneous bilirubin in normal children was 33. 14~96. 14μmol/L. The transcutaneous bilirubin of children with good prognosis was significantly lower than that of children with poor prognosis on postoperative day 11 ( 159 μmol/L vs 205 μmol/L,P<0. 05) and 12(151 μmol/L vs 210μmol/L,P<0. 05). Postoperative average daily decline of transcutaneous bilirubin in children with good prognosis was faster (5. 04 μmol/L vs 2. 33 μmol/L, P<0. 05). Conclusions Transcutaneous bilirubin measurement is a safe and reliable method of monitoring the decline of jaundice of children with biliary atresia.%目的 初步探讨胆道闭锁患儿术后测定皮肤胆红素动态变化与黄疸消退及预后的关系.方法 2009年7月至12月,收治黄疸患儿52例,男19例,女33例,年龄36 d~304 d,平均年龄83 d,包括胆汁淤积5例,胆道闭锁47例,采血测血清胆

  3. Esophageal Atresia

    DEFF Research Database (Denmark)

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

    2013-01-01

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

  4. Oesophageal atresia

    Directory of Open Access Journals (Sweden)

    Spitz Lewis

    2007-05-01

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

  5. 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.

  6. Efficacy of laparoscopic operation in treating type Ⅲ child biliary atresia and its influence on postoperative infection%腹腔镜手术对Ⅲ型胆道闭锁患儿疗效及术后感染的影响

    Institute of Scientific and Technical Information of China (English)

    池小斌; 陈光明

    2014-01-01

    目的:探讨腹腔镜手术对Ⅲ型胆道闭锁患儿治疗效果及术后感染的影响。方法将85例Ⅲ型胆道闭锁患儿分为传统手术组(O P组)42例及腹腔镜手术组(L P组)43例,对比分析两组患者手术情况、术后炎症因子变化、手术前后肝功能指标变化、术后感染情况。结果 LP组手术时间较OP组长(P<0.05),术中出血量、术后视觉模拟评分(VAS)少于OP组(P<0.05),术后恢复进食时间早于OP组(P<0.05)。LP组术后感染率低于OP组(P<0.05)。两组患儿术前、术后1、3、6、12个月肝功能指标恢复差异无统计学意义(P>0.05)。结论腹腔镜手术与传统手术治疗Ⅲ型胆道闭锁患儿各有利弊,临床应根据患儿实际情况选取合适的手术方式。%Objective To investigate the effects of laparoscopic operation in treating type Ⅲ child biliary atresia and its influence on postoperative infections .Methods 85 children cases of type Ⅲ biliary atresia were randomly divided into the conventional sur-gery group(OP group ,n=42) and the laparoscopic surgery group(LP group ,n=43) .The operation situation ,postoperative changes of inflammatory factors ,changes of liver function indexes before and after operation and postoperative infection were compared be-tween the two groups .Results The operative time of the LP group was longer than that of the OP group (P0 .05) .Conclusion The laparoscopic operation and the conventional operation in treating type Ⅲ child biliary atresia have their own advantages and disadvantages .The appropriate operation mode should be selected according to the children′s actual situation .

  7. 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

  8. Atresia in the esophagus

    Directory of Open Access Journals (Sweden)

    Crisanto Abad Celuria

    2005-12-01

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

  9. Biliary scintigraphy in neonatal cytomegalovirus cholestasis

    International Nuclear Information System (INIS)

    Diagnostic value of hepatobiliary scintigraphy using mebrofenin-Te-99m was assessed in three newborns with cytomegalovirus (CMV) hepatitis and one baby with hepatitis B jaundice. All cases were affected by persistent jaundice with predominately conjugated bilirubin, alcoholic stools, anemia. One of this newborns (case number 1) was suspected of having biliary atresia due to the absence of intestinal excretion of the tracer. After three weeks intestinal passage was seen in scintiscan late after 24 h. Hepatobiliary scintigraphy represents a non-invasive diagnostic procedure which enables the detection of permeability of the biliary tract. (Author)

  10. 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.

  11. Congenital intestinal atresia.

    Science.gov (United States)

    Davenport, M; Bianchi, A

    1990-09-01

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

  12. [Congenital Esophageal Atresia].

    Science.gov (United States)

    Suzuki, Makoto; Kuwano, Hiroyuki

    2015-07-01

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

  13. National Esophageal Atresia Register.

    Science.gov (United States)

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

    2015-12-01

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

  14. 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.

  15. Intrauterine Intussusception Causing Ileal Atresia

    Directory of Open Access Journals (Sweden)

    Yogesh Kumar Sarin

    2010-11-01

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

  16. 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.

  17. 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.

  18. Atresia in the esophagus Atresia Esofágica.

    Directory of Open Access Journals (Sweden)

    Narciso Hernández Rodríguez

    2005-12-01

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

  19. 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 ...

  20. Comparison of early graft function in biliary atresia patients after living donor liver transplantation and whole liver transplantation%胆道闭锁患儿活体肝移植和全肝移植术后早期肝功能的比较

    Institute of Scientific and Technical Information of China (English)

    顾向前; 高伟; 蔡金贞; 孙超; 马楠; 董冲; 滕大洪; 郑虹

    2014-01-01

    造成的肝组织缺血损伤有关。活体肝移植可以有效缓解供肝短缺的矛盾,缩短等待供肝的时间,值得推广。%Objective To compare the early graft function in biliary atresia patients after living donor liver transplantation and whole liver transplantation. Methods 22 children underwent pediatric liver transplantation for biliary atresia from January 2014 to June 2014 in Tianjin First Central Hospital. Among them,14 patients underwent living donor liver transplantation(LDLT group)using piggyback technique with left liver lobe,8 patients underwent whole liver transplantation without bypass(WLT group). Pre-operative and intraoperative clinical data and early postoperative liver function were compared retrospectively. Results There were no significant differences in age,gender,weight,preoperative Child-Pugh score,alanine aminotransferase(ALT), aspartate aminotransferase(AST),total bilirubin(TBil),direct bilirubin(DBil),intraoperative blood loss,blood transfusion,and graft weight between these two groups(P>0.05). However,donor age was older in LDLT group〔(30.8±6.9)years vs.(8.6±6.0)months〕than that of WLT group,with longer operative time〔(11.6±1.3)hours vs.(9.2±1.5)hours〕and shorter cold ischemia time〔(72.1±21.7)minutes vs.(458.75±127.72)minutes〕(P0.05). There was no statistical significance in AST level between two groups(P>0.05). The TBil and DBil levels of WLT group were higher on POD 1~4,but lower on POD 5~7 than that of LDLT group (P>0.05). Conclusion The liver function recovered well early after operation in both groups. ALT level of LDLT group was higher than that of WLT group on POD 1~5,which was probably related to longer operative time and ischemia injury caused by operation. LDLT is being advocated as an effective method to better meet the waiting-list needs and shorten the waiting time.

  1. Abnormal expressions of iNOS and its regulatory factor in the liver of biliary atresia with progressive damage%胆道闭锁肝内iNOS及其调控因子异常表达与进行性肝损伤的相关性

    Institute of Scientific and Technical Information of China (English)

    黄磊; 魏明发; 冯杰雄; 袁继炎

    2008-01-01

    目的 研究胆道闭锁(BA)患儿肝内诱导型一氧化氮合酶(iNOS)及其上下游调控因子表达情况,并探讨其与BA进行性肝损伤发生的关系.方法 应用免疫组织化学染色法对2002年10月至2007年3月在本院行Kasai手术的38例BA患儿与16例对照儿童肝组织iNOS表达情况进行研究;应用ELASA法对BA患儿和对照组外周血总一氧化氮(NO)代谢产物浓度进行测定;应用TUNEL法对BA患儿和对照组肝内胆管上皮凋亡指数进行测定;应用免疫印记法对BA患儿和对照组肝组织NF-κB表达进行半定量分析.结果 iNOS在BA患儿肝组织异常高表达,强度为0.30±0.08,而在对照组肝组织不表达.BA患儿外周血总NO代谢产物浓度为(90.40±12.46)mol/L,明显高于对照组的(63.67±5.78)μmol/L,且BA组总NO代谢产物浓度与血清ALT水平[(152.76±29.59)U/L]呈强正相关(r=0.97).BA组肝内胆管上皮凋亡指数(54.00±11.67)%远高于正常对照组(20.72±5.63)%,且与肝组织iNOS表达强度呈强正相关(r=0.99).NF-κB出在BA患儿肝组织中的表达(0.74±0.06)明显高于正常对照组(0.22±0.03),且与iNOS表达强度呈强正相关(r=0.97).结论 iNOS异常高表达在BA患儿进行性肝损伤中发挥重要作用,该作用是由iNOS及其上下游调控因子NF-κB、NO共同作用所实现.%Objective To study the expression of induced nitric oxide synthase(iNOS)and its up-down stream regulatory factors in the liver of biliary atresia(BA)with progressive hepatic damage.Methods The iNOS expressions in the liver of 38 cases of BA(mean age 94.5±41.5 d)from Oct 2002 to Mar 2007 and 16 cases(mean age 103.1±49.1 d)without hepatic or biliary disease selected as normal control were examined by immunohistochemistry staining method respectively.The total serum NO metabolites was evaluated by ELISA method;the apoptosis index of bile duct epithelial cells was analyzed by TUNEL method and the nuclear factor-κB(NF-κB)expression was assessed by western

  2. Follicular growth, differentiation and atresia

    Institute of Scientific and Technical Information of China (English)

    JIN Xuan; LIU Yixun

    2003-01-01

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

  3. Esophageal stenosis with esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

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

    1987-03-01

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

  4. 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.

  5. 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. PMID:10656644

  6. Jejunal atresia associated with idiopathic ileal perforation

    Directory of Open Access Journals (Sweden)

    Das P

    2008-01-01

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

  7. Gallbladder Duplication Associated with Gastro-Intestinal Atresia.

    Science.gov (United States)

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

    2016-01-01

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

  8. 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.

  9. 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....

  10. Preoperative biliary drainage.

    Science.gov (United States)

    Saxena, Payal; Kumbhari, Vivek; Zein, Mohamad E L; Khashab, Mouen A

    2015-01-01

    The role of preoperative biliary drainage (PBD) in patients with distal or proximal biliary obstruction secondary to resectable tumors has been a matter for debate. A review of the literature using Medline, Embase and Cochrane databases was undertaken for studies evaluating routes of drainage (endoscopic or percutaneous) and stent types (plastic or metal) in patients with resectable disease. Preoperative biliary drainage is indicated for relief of symptomatic jaundice, cholangitis, patients undergoing neoadjuvant therapy or those patients where surgery may be delayed. Endoscopic methods are preferred over percutaneous methods because of lower complication rates. In patients with proximal biliary obstruction, PBD should be guided by imaging studies to aid in selective biliary cannulation for unilateral drainage in order to reduce the risk of cholangitis in undrained liver segments. PMID:25293587

  11. Prenatal diagnosis of horseshoe lung and esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

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

    2006-09-15

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

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

    OpenAIRE

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

    2007-01-01

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

  13. 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.

  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. PMID:27123404

  15. 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.

  16. Stenting in Malignant Biliary Obstruction.

    Science.gov (United States)

    Almadi, Majid A; Barkun, Jeffrey S; Barkun, Alan N

    2015-10-01

    Decompression of the biliary system in patients with malignant biliary obstruction has been widely accepted and implemented as part of the care. Despite a wealth of literature, there remains a significant amount of uncertainty as to which approach would be most appropriate in different clinical settings. This review covers stenting of the biliary system in cases of resectable or palliative malignant biliary obstruction, potential candidates for biliary drainage, technical aspects of the procedure, as well as management of biliary stent dysfunction. Furthermore, periprocedural considerations including proper mapping of the location of obstruction and the use of antibiotics are addressed. PMID:26431598

  17. 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.

  18. 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...

  19. 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....

  20. 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.

  1. 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)

  2. [Malignant biliary obstruction].

    Science.gov (United States)

    Hucl, Tomáš

    2016-01-01

    Pancreatic cancer and cholangiocarcinoma are the most common causes of malignant biliary obstruction. They are diseases of increasing incidence and unfavorable prognosis. Only patients with localized disease indicated for surgery have a chance of long-term survival. These patients represent less than 20 % of all patients, despite the progress in our diagnostic abilities.Locally advanced and metastatic tumors are treated with palliative chemotherapy or chemoradiotherapy; the results of such treatments are unsatisfactory. The average survival of patients with unresectable disease is 6 months and only 5-10 % of patients survive 5 years.Biliary drainage is an integral part of palliative treatment. Endoscopically or percutaneosly placed stents improve quality of life, decrease cholestasis and pruritus, but do not significantly improve survival. Biliary stents get occluded over time, possibly resulting in acute cholangitis and require repeated replacement.Photodynamic therapy and radiofrequency ablation, locally active endoscopic methods, have been increasingly used in recent years in palliative treatment of patients with malignant biliary obstruction. In photodynamic therapy, photosensitizer accumulates in tumor tissue and is activated 48 hours later by light of a specific wave length. Application of low voltage high frequency current during radiofrequency ablation results in tissue destruction by heat. Local ablation techniques can have a significant impact in a large group of patients with malignant biliary obstruction, leading to improved prognosis, quality of life and stent patency. PMID:26898789

  3. Tracheal Trifurcation Associated With Esophageal Atresia

    Directory of Open Access Journals (Sweden)

    Yogesh Kumar Sarin

    2010-11-01

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

  4. Microdeletion 22q11 and oesophageal atresia

    Science.gov (United States)

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

    1999-01-01

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


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

  5. 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.

  6. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2014-03-15

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

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

    Science.gov (United States)

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

    2015-09-01

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

  9. 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.

  10. Etiological study on isolated esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

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

    1985-01-01

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

  11. 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...

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

    Science.gov (United States)

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

    2012-03-01

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

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

    International Nuclear Information System (INIS)

    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

  14. 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.

  15. Bone conduction hearing in congenital aural atresia.

    Science.gov (United States)

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

    2016-07-01

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

  16. 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.

  17. Biliary complications following liver transplantation

    OpenAIRE

    Kochhar, Gursimran; Parungao, Jose Mari; Hanouneh, Ibrahim A; Parsi, Mansour A

    2013-01-01

    Biliary tract complications are the most common complications after liver transplantation. These complications are encountered more commonly as a result of increased number of liver transplantations and the prolonged survival of transplant patients. Biliary complications remain a major source of morbidity in liver transplant patients, with an incidence of 5%-32%. Post liver transplantation biliary complications include strictures (anastomotic and non-anastomotic), leaks, stones, sphincter of ...

  18. 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. PMID:8560608

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

    NARCIS (Netherlands)

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

    2007-01-01

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

  20. 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.

  1. Genetic players in esophageal atresia and tracheoesophageal fistula.

    NARCIS (Netherlands)

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

    2005-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Dutta Hemonta

    2009-01-01

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

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

    Science.gov (United States)

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

    2001-11-01

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

  4. 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

  5. Colchicine for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Gong, Yan; Gluud, Christian

    2005-01-01

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

  6. Azathioprine for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Gong, Yanzhang; Christensen, E; Gluud, C

    2007-01-01

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

  7. Endoscopicmanagementofpostcholecystectomy biliary leakage

    Institute of Scientific and Technical Information of China (English)

    Virendra Singh; Gurpreet Singh; Ganga R Verma; Rajesh Gupta

    2010-01-01

    BACKGROUND: Biliary leak is an uncommon but signiifcant complication following cholecystectomy. Endotherapy is an established method of treatment. However, the optimal intervention is not known. METHOD: Eighty-ifve patients with postcholecystectomy biliary leaks from July 2000 to March 2009 were retrospectively evaluated. RESULTS: The study population was 20 males and 65 females with a mean age of 42.47 years. Patients presented with abdominal pain (46), jaundice (23), fever (23), abdominal distension (42), or bilious abdominal drain (67). Endoscopic retrograde cholangiopancreatography detected a leak at the cystic duct stump in 45 patients, stricture with middle common bile duct leak in 4, leak from the right hepatic duct in 3, and a ligated common bile duct in 32. Twelve also had bile duct stones. One had a broken T-tube with stones. Endotherapy was possible in 53 patients. Three patients with stones, one with a broken T-tube with stones, and 4 with stricture of the common bile duct with a leak were managed with sphincterotomy and stenting. Eight patients with a cystic duct stump leak with stones were managed with sphincterotomy and stone extraction. Three outpatients and 12 inpatients with a cystic duct stump leak were managed with sphincterotomy and stent and sphincterotomy and nasobiliary drain, respectively. Five patients with a cystic duct stump leak were managed with stenting. Sixteen with coagulopathy were managed with only nasobiliary drain (9) or stent (7). Leak closure was achieved in 100%patients. Four developed mild pancreatitis which improved with conservative treatment. CONCLUSIONS: Endoscopic intervention is a safe and effective method of treatment of postcholecystectomy biliary leaks. However, management should be individualized based on factors such as outpatients or inpatients, presence of stone, stricture, ligature, or coagulopathy.

  8. 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.)

  9. Ursodeoxycholic acid for primary biliary cirrhosis

    DEFF Research Database (Denmark)

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

    2008-01-01

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

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

    Science.gov (United States)

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

    1976-07-01

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

  11. The diagnostic value of high-frequency ultrasonography in biliary atresia

    Institute of Scientific and Technical Information of China (English)

    Li-Ping Jiang; Yun-Chao Chen; Lu Ding; Xiao-Ling Liu; Kai-Yan Li; Dao-Zhong Huang

    2013-01-01

    BACKGROUND: It  is  a  globally  challenging  problem  to differentially diagnose biliary atresia (BA) from other disease processes  causing  infantile  cholestatic  jaundice.  The  high-frequency  ultrasonography  (HUS)  yields  much  improved spatial resolution and therefore, might show better image in BA diagnostic examination. The present study was to evaluate the HUS on the diagnosis of BA in infants with jaundice. METHODS: Fifty-one  infants  with  neonatal  jaundice  were scanned with ultrasonography. Images included gallbladder, bile duct, right hepatic artery (RHA), portal vein (PV) and triangular cord (TC) sign, magnetic resonance imaging and additionally, laboratory tests and histopathology reports were assessed. RESULTS: Twenty-three  BA  and  28  non-BA  cases  were  con-firmed.  The  sensitivity,  specificity,  and  accuracy  of  HUS  were 91.3%,  92.9%, and 92.2%, respectively. All of these indices were significantly higher than those of conventional ultrasonography (P CONCLUSION: HUS  provided  better  imaging  of  BA  and should be considered as a primary modality in the differential diagnosis of infantile jaundice.

  12. Glucocorticosteroids for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Prince, M; Christensen, E; Gluud, C

    2005-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Sajitha

    2015-03-01

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

  14. Genetics Home Reference: epidermolysis bullosa with pyloric atresia

    Science.gov (United States)

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

  15. Future developments in biliary stenting

    OpenAIRE

    Hair CD; Sejpal DV

    2013-01-01

    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...

  16. A Surviving Child With Complete Proximal Tracheal Atresia

    OpenAIRE

    Haight, Ken; Sankaran, Koravangattu; Shokeir, Mohamed

    1984-01-01

    An infant was born with an unusual combination of primitive foregut anomalies consisting of complete proximal tracheal atresia, proximal esophageal atresia and distal tracheoesophageal fistula. Before the birth, the family physician suspected an anomaly of the upper airway or esophageal occlusion on the basis of hydramnios evident at the thirty-third to thirty-fourth week of gestation, and earlier amniocentesis which indicated a normal level of α-fetoprotein. He consulted the hospital obstetr...

  17. 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.

  18. 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.

  19. 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.

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

    OpenAIRE

    Narendra Kumar Are; Nagarjuna, K.; Lavanya Kannaiyan

    2010-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Narendra Kumar Are

    2010-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1986-08-15

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

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

    Directory of Open Access Journals (Sweden)

    P Raj

    2014-07-01

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

  4. Evaluation of biliary disease by scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Ram, M.D.; Hagihara, P.F.; Kim, E.E.; Coupal, J.; Griffen, W.O.

    1981-01-01

    The value of biliary scintigraphy was studied in 180 patients with suspected biliary tract disease. Most of the patients were investigated additionally by conventional techniques such as cholecystography, cholangiography and ultrasonography. It is concluded that biliary scintigraphy is a simple and safe technique for visualization of the biliary tract. It is particularly useful in the evaluation of acute cholecystitis, in patients with iodine sensitivity obstructive from nonobstructive jaundice.

  5. Current treatment of benign biliary strictures

    OpenAIRE

    Costamagna, Guido; Boškoski, Ivo

    2013-01-01

    Endoscopy is a widely used approach for the treatment of benign biliary strictures. Most common benign biliary strictures amandable to endoscopic treatment are post-cholecystectomy, dominant biliary strictures due to primary sclerosing cholangitis, biliary anastomotic strictures occurring after liver transplantation, and common bile duct strictures due to chronic pancreatitis. Surgery is a valid option in cases of complete transection or ligation of the common bile duct, in selected patients ...

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

    OpenAIRE

    Garg, Mukesh Kumar

    2009-01-01

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

  7. 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

  8. 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.

  9. Endoscopic management of benign biliary strictures

    Institute of Scientific and Technical Information of China (English)

    Kavel; H; Visrodia; James; H; Tabibian; Todd; H; Baron

    2015-01-01

    Endoscopic management of biliary obstruction has evolved tremendously since the introduction of flexible fiberoptic endoscopes over 50 years ago. For the last several decades, endoscopic retrograde cholangiopancreatography(ERCP) has become established as the mainstay for definitively diagnosing and relieving biliary obstruction. In addition, and more recently, endoscopic ultrasonography(EUS) has gained increasing favor as an auxiliary diagnostic and therapeutic modality in facilitating decompression of the biliary tree. Here, we provide a review of the current and continually evolving role of gastrointestinal endoscopy, including both ERCP and EUS, in the management of biliary obstruction with a focus on benign biliary strictures.

  10. Percutaneous biliary drainage and stenting

    International Nuclear Information System (INIS)

    Full text: Percutaneous transhepatic cholangiography (PTC) is an X-ray or US guided procedure that involves the injection of a contrast material directly into the bile ducts inside the liver to produce pictures of them. If a blockage or narrowing is found, additional procedures may be performed: 1. insertion of a catheter to drain excess bile out of the body or both - internal and external; 2. plastic endoprothesis placement; 3. self-expandable metal stents placement to help open bile ducts or to bypass an obstruction and allow fluids to drain. Current percutaneous biliary interventions include percutaneous transhepatic cholangiography (PTC) and biliary drainage to manage benign and malignant obstructions. Internal biliary stents are either plastic or metallic, and various types of each kind are available. Internal biliary stents have several advantages. An external tube can be uncomfortable and have a psychological disadvantage. An internal stent prevents the problems related to external catheters, for example, pericatheter leakage of bile and the need for daily flushing. The disadvantages include having to perform endoscopic retrograde cholangiopancreatography (ERCP) or new PTC procedures to obtain access in case of stent obstruction. Better patency rates are reported with metallic than with plastic stents in cases of malignant obstruction, though no effect on survival is noted. Plastic internal stents are the cheapest but reportedly prone to migration. Metallic stents are generally not used in the treatment of benign disease because studies have shown poor long-term patency rates. Limited applications may include the treatment of patients who are poor surgical candidates or of those in whom surgical treatment fails. Most postoperative strictures are treated surgically, though endoscopic and (less commonly) percutaneous placement of nonmetallic stents has increasingly been used in the past few years. Now there are some reports about use of biodegradable biliary

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2002-09-01

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

  12. 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.)

  13. 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.

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

    International Nuclear Information System (INIS)

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

  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. Studies in primary biliary cirrhosis

    NARCIS (Netherlands)

    B.G. Taal

    1981-01-01

    textabstractThe specific aims of the study were: - To (re)define the clinical features of the primary biliary cirrhosis syndrome. - To develop objective criteria which combine specificity with sensitivity for the diagnosis of PBC. - To study the relation of IgM in various forms (pentameric, monomeri

  17. 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.

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

    OpenAIRE

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

    2012-01-01

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

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

    Science.gov (United States)

    Vianna, Maria L; Tobias, Karen M

    2005-01-01

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

  20. 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.

  1. Biliary pain in postcholecystectomy patients without biliary obstruction. A prospective radionuclide study.

    Science.gov (United States)

    Grimon, G; Buffet, C; André, L; Etienne, J P; Desgrez, A

    1991-03-01

    Biliary pain without obvious biliary obstruction is common in postcholecystectomy patients. We studied 20 symptomatic patients with episodes of biliary-type pain after cholecystectomy (all having undergone endoscopic retrograde cholangiography), and in 18 asymptomatic postcholecystectomy controls. We performed quantitative hepatobiliary radionuclide analysis with dimethyl-imidodiacetic acid. From a series of 90 dynamic images at 1-min intervals using a gamma camera coupled to a computer, time-activity curves were produced in regions of interest in the liver, intrahepatic biliary tree, common duct, and heart, from which quantitative biliary excretion indexes were obtained. The results demonstrate a biliary kinetic dysfunction in patients with postcholecystectomy pain without morphological abnormalities. PMID:1995268

  2. Pyloric atresia: A report of ten patients

    Directory of Open Access Journals (Sweden)

    Amine Ksia

    2013-01-01

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

  3. 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.)

  4. 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.

  5. Clinical and Molecular-Genetic Studies in Esophageal Atresia

    NARCIS (Netherlands)

    E.M. de Jong (Elisabeth)

    2010-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

  7. Duodenal atresia in association with situs inversus abdominus

    Directory of Open Access Journals (Sweden)

    Raghu Shankar

    2012-01-01

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

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

    Science.gov (United States)

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

    2004-10-01

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

  9. Successive breaks in biliary stents.

    Science.gov (United States)

    Espinel, Jesús; Pinedo, Eugenia; Ojeda, Vanesa; Guerra, María

    2016-04-01

    A 64 year-old male, was diagnosed with obstructive jaundice due to a well-differentiated pancreatic neuroendocrine tumor with liver metastases. The patient underwent endoscopic placement of covered self-expanding biliary stent (10x60 mm, Hanaro) by ERCP. He was admitted with cholangitis one year later. The following ERCP revealed a fractured stent with loss of the distal end (duodenal) and partial migration of the remaining stent to the common bile duct. The fragmented stent was removed from the common bile duct and a new, similar one was inserted. Four months later the patient was admitted with cholangitis. A new ERCP was done and biliary stent was also fragmented. It was removed and an uncovered stent (Wallflex) was inserted. PMID:27065248

  10. 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.

  11. Current Status of Biliary Metal Stents

    OpenAIRE

    Nam, Hyeong Seok; Kang, Dae Hwan

    2016-01-01

    Many advances have been achieved in biliary stenting over the past 30 years. Endoscopic stent placement has become the primary management therapy to relieve obstruction in patients with benign or malignant biliary tract diseases. Compared with plastic stents, a self-expandable metallic stent (SEMS) has been used for management in patients with malignant strictures because of a larger lumen and longer stent patency. Recently, SEMS has been used for various benign biliary strictures and leaks. ...

  12. Contemporary Management of Acute Biliary Pancreatitis

    OpenAIRE

    Orhan Ozkan

    2014-01-01

    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 ha...

  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. ERCP in acute biliary pancreatitis

    OpenAIRE

    Kapetanos, Dimitrios J

    2010-01-01

    The role of urgent endoscopic retrograde cholangiopancreatography (ERCP) in acute biliary pancreatitis is for many years a subject for disagreement among physicians. Although the evidence seemed to be in favor of performing ERCP, endoscopists usually hesitate to conform to the guidelines. ERCP is an invasive procedure, with complications which can affect patients’ outcome. Recent evidence suggests that we should probably modify our policy, recruiting less invasive procedures, like magnetic re...

  15. Diet and biliary tract cancer

    OpenAIRE

    Moerman CJ; Bueno de Mesquita HB; Runia S; Smeets FWM

    1991-01-01

    We studied the relation between diet and biliary tract cancer in a case-control study, comprising 111 incident cases and 480 controls from the general population. Food intake was assessed with a semi- quantitative food frequency questionnaire, which covered diet comprehensively. In half of the cases and 30% of the controls the information was obtained from the spouse or other relatives (indirect respondents). Mono- and disaccharides showed an elevated risk, independent from other sources of e...

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

    Directory of Open Access Journals (Sweden)

    Kazunori Masahata

    2015-09-01

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

  17. 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.

  18. Biliary complications after orthotopic liver transplantation

    NARCIS (Netherlands)

    Karimian, Negin; Westerkamp, Andrie C.; Porte, Robert J.

    2014-01-01

    Purpose of reviewThe incidence, pathogenesis and management of the most common biliary complications are summarized, with an emphasis on nonanastomotic biliary strictures (NAS) and potential strategies to prevent NAS after liver transplantation.Recent findingsNAS have variable presentations in time

  19. Cyclosporin A for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Gong, Yanzhang; Christensen, E; Gluud, C

    2007-01-01

    Cyclosporin A has been used for patients with primary biliary cirrhosis, but the therapeutic responses in randomised clinical trials have been heterogeneous.......Cyclosporin A has been used for patients with primary biliary cirrhosis, but the therapeutic responses in randomised clinical trials have been heterogeneous....

  20. D-penicillamine for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Gong, Yanzhang; Frederiksen, S L; Gluud, C

    2004-01-01

    D-penicillamine is used for patients with primary biliary cirrhosis due to its hepatic copper decreasing and immunomodulatory potentials. The results from randomised clinical trials have been inconsistent.......D-penicillamine is used for patients with primary biliary cirrhosis due to its hepatic copper decreasing and immunomodulatory potentials. The results from randomised clinical trials have been inconsistent....

  1. 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

  2. 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

  3. Current Status of Biliary Metal Stents.

    Science.gov (United States)

    Nam, Hyeong Seok; Kang, Dae Hwan

    2016-03-01

    Many advances have been achieved in biliary stenting over the past 30 years. Endoscopic stent placement has become the primary management therapy to relieve obstruction in patients with benign or malignant biliary tract diseases. Compared with plastic stents, a self-expandable metallic stent (SEMS) has been used for management in patients with malignant strictures because of a larger lumen and longer stent patency. Recently, SEMS has been used for various benign biliary strictures and leaks. In this article, we briefly review the characteristics of SEMS as well as complications of stent placement. We review the current guidelines for managing malignant and benign biliary obstructions. Recent developments in biliary stenting are also discussed. PMID:26911896

  4. Current Status of Biliary Metal Stents

    Science.gov (United States)

    Nam, Hyeong Seok; Kang, Dae Hwan

    2016-01-01

    Many advances have been achieved in biliary stenting over the past 30 years. Endoscopic stent placement has become the primary management therapy to relieve obstruction in patients with benign or malignant biliary tract diseases. Compared with plastic stents, a self-expandable metallic stent (SEMS) has been used for management in patients with malignant strictures because of a larger lumen and longer stent patency. Recently, SEMS has been used for various benign biliary strictures and leaks. In this article, we briefly review the characteristics of SEMS as well as complications of stent placement. We review the current guidelines for managing malignant and benign biliary obstructions. Recent developments in biliary stenting are also discussed. PMID:26911896

  5. Percutaneous transcather biliary biopsy with a biotoma

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Goo; Park, Eaui Dong; Ahn, In Oak [Gyeongsang National University College of Medicine, Chinju (Korea, Republic of)

    1993-07-15

    For the purpose of the precise diagnosis and proper treatment planning of obstructive jaundice, various techniques to obtain tissues from biliary stricture sites have been proposed. We performed percutaneous transcatheter biopsy of biliary strictures with a biotome in six patients with obstructive jaundice. The site of biliary stricture were distal common bile ducts (n=1), and confluence of both intrahepatic ducts (n=2). Their histologic diagnose were adenocarcinoma (n=2), chronic choleochitis (n=3), and atypical cell suspicious of malignancy (n=1). False positive or false negative results were not documented by other means (including laparotomy), when regarding atypical cell suspicious of malignancy as true positive for malignancy. Percutaneous transcatheter biliary biopsy with a biotome is easy to perform in conjunction with percutaneous transhepatic biliary drainage procedure, and can be able to obtain specific tissue for correct diagnosis.

  6. 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.

  7. 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.

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

    Science.gov (United States)

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

    2016-06-01

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

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

    OpenAIRE

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

    2013-01-01

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

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

    OpenAIRE

    Jamal A; Mesdaghi Nia S

    2000-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Jamal A

    2000-09-01

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

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

    Institute of Scientific and Technical Information of China (English)

    邵雷朋; 潘登; 王献良

    2013-01-01

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

  13. 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.

  14. 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.

  15. 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

  16. 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.

  17. 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.

  18. Endoscopic Stent Placement in the Palliation of Malignant Biliary Obstruction

    OpenAIRE

    Kim, Jin Hong

    2011-01-01

    Biliary drainage with biliary stent placement is the treatment of choice for palliation in patients with malignant biliary obstruction caused by unresectable neoplasms. In such patients, the endoscopic approach can be initially used with percutaneous radiological intervention. In patients with unresectable malignant distal bile duct obstructions, endoscopic biliary drainage with biliary stent placement has now become the main and least invasive palliative modality, which has been proven to be...

  19. CT biliary cystoscopy of gallbladder polyps

    Institute of Scientific and Technical Information of China (English)

    Ming-Wu Lou; Wei-Dong Hu; Yi Fan; Jin-Hua Chen; Zhan-Sen E; Guang-Fu Yang

    2004-01-01

    AIM: CT virtual endoscopy has been used in the study of various organs of body including the biliary tract, however,CT virtual endoseopy in diagnosis of gallbladder polyps has not yet been reported. This study was to evaluate the diagnostic value of CT virtual endoscopy in polyps of the gallbladder.METHODS: Thirty-two cases of gallbladder polyps were examined by CT virtual endoscopy, ultrasound, CT scan with oral biliary contrast separately and confirmed by operation and pathology. CT biliary cystoscopic findings were analyzed and compared with those of ultrasound and CT scan with oral biliary contrast, and evaluated in comparison with operative and pathologic findings in all cases.RESULTS: The detection rate of gallbladder polyps was 93.8%(90/96), 96.9%(93/96) and 79.2%(76/96) for CT cystoscopy, ultrasound and CT scan with oral contrast,respectively. CT biliary cystoscopy corresponded well with ultrasound as well as pathology in demonstrating the location, size and configuration of polyps. CT endoscopy was superior to ultrasound in viewing the polyps in a more precise way, 3 dimensionally from any angle in space, and showing the surface in details. CT biliary cystoscopy was also superior to CT scan with oral biliary contrast in terms of observation of the base of polyps for the presence of a pedicle, detection rates as well as image quality. The smallest polyp detected by CT biliary cystoscopy was measured 1.5 mmx2.2 mmx2.5 mm.CONCLUSION: CT biliary cystoscopy is a non-invasive and accurate technique for diagnosis and management of gallbladder polyps.

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

    Directory of Open Access Journals (Sweden)

    Khaled Al-Noury

    2011-01-01

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

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

    OpenAIRE

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

    2013-01-01

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

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

    NARCIS (Netherlands)

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

    2005-01-01

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

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

    DEFF Research Database (Denmark)

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

    1986-01-01

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

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

    Science.gov (United States)

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

    2015-12-01

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

  6. 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

  7. 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...

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

    OpenAIRE

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

    1989-01-01

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

  9. 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.)

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

    International Nuclear Information System (INIS)

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

  11. 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.

  12. Biliary papillomatosis: analysis of 18 cases

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    @@ Biliary papillomatosis (BP) is an extremely rare pathological condition, which is characterized by papillary proliferation of the bile duct epithelia. Although initially thought to be a benign entity, this tumor has been shown to have a tendency for malignant transformation.

  13. 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...

  14. Plasma exchange in primary biliary cirrhosis

    OpenAIRE

    Keeling, P W N; Kingston, P.; Bull, J.; Thompson, R. P. H.

    1981-01-01

    The symptoms of patients with primary biliary cirrhosis are frequently intractable to traditional therapy. Three patients are reported in whom several symptoms were alleviated by plasma exchange, using a Hemonetics Model 30 cell separator.

  15. How Should Biliary Stones be Managed?

    OpenAIRE

    Shim, Chan Sup

    2010-01-01

    Minimally invasive therapy is currently invaluable for the treatment of biliary stones. Clinicians should be familiar with the various endoscopic modalities that have been evolving. I reviewed the treatment of biliary stones from the common practice to pioneering procedures, and here I also briefly summarize the results of many related studies. Lithotripsy involves procedures that fragment large stones, and they can be roughly classified into two groups: intracorporeal modalities and extracor...

  16. Adenocarcinoma of the extrahepatic biliary tree.

    OpenAIRE

    Anderson, J B; Cooper, M J; Williamson, R. C.

    1985-01-01

    Increasing survival rates for carcinoma of the biliary tree could reflect the selection of patients for referral to a specialist centre as well as modern improvements in diagnosis and treatment. To determine the true incidence and outcome of biliary cancer, the records of 243 unselected Bristol patients were reviewed retrospectively over a 15-year period. Mean age was 64 years. Gallstones were associated in 38% of cases. Sixty-nine of 87 patients with gallbladder carcinoma were submitted to l...

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

    DEFF Research Database (Denmark)

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

    2009-01-01

    BACKGROUND/PURPOSE: The aims of this study are as follows: METHOD: From 1979 to 2003, 57 children have been operated by the Kasai procedure. Only 40 of these have had their portal plate removed for histologic examination. We divided the patients according to clinical outcome into a successful and...

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

    Directory of Open Access Journals (Sweden)

    Jan Iftikhar

    2006-01-01

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

  19. 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.

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

    International Nuclear Information System (INIS)

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1987-01-01

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

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

    Science.gov (United States)

    Manfredi, Michael A

    2016-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2012-12-15

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

  5. 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.

  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. The Effects of Cholecystojejunostomy and Biliary Drainage on Biliary Motor

    Institute of Scientific and Technical Information of China (English)

    郑启昌; 陈阳龙

    2002-01-01

    Summary: Simulating physiological neuronal and hormonal conditions during digestive and interdigestive periods, the study identified the changes of the motility of biliary system including bile duct and sphincter of Oddi (SO) before and after cholecystojejunostomy. Thirty-five rabbits were divided into five groups randomly. The experimental groups received the venous injection of CCK 10 ng/kg, ery thromycin 10 mg/kg, atropine 3 μg/kg and L-NAME 10 mg/kg respectively. Each rabbit under went manometry through introducing a three-lumen catheter via the papilla retrogradely, using the low-compliance papillary infusion system. Then the gallbladder and the upper segment of the jejunum was anastomosed and the manometric procedures repeated after one week. SO basal pressure was in creased, contraction amplitude decreased, contraction time shortened after cholecystojejunostomy. L-NAME, CCK and erythromycin could all excite SO. L-NAME could increase basal pressure and con traction amplitude, CCK increase basal pressure contraction amplitude and frequency, and ery-thromycin increase contraction amplitude, respectively. But comparing with that before cholecystoje junostomy, the increasing extent was decreased. The tensional and spontaneous contractions of the SO were under the control of the neural and hormonal mechanism. The anastomosis of gallbladder and jejunum and the drainage of bile made the tensional contraction stronger, but the spontaneous contraction weakened after the operation due to the decreases of the sensitivity of SO to hormonal fac tors. The clinical symptoms may not be relieved when the patients with SO dysfunction accepted cholecystojejunostomy.

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

    Directory of Open Access Journals (Sweden)

    Mbaye Fall

    2015-01-01

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

  9. 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

  10. Biliary tree and cholecyst: post surgery imaging

    Energy Technology Data Exchange (ETDEWEB)

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

    2005-03-01

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

  11. Malignant biliary obstruction treated with metallic stents

    International Nuclear Information System (INIS)

    Purpose: The outcome of 33 patients with malignant biliary obstruction treated with metallic stents is presented. Materials and methods: Thirty-eight biliary metallic stents were placed in 33 patients with malignant biliary obstruction. They were 52-71 years old (mean = 61.7); 23 were male and 10 were female. The biliary obstruction was caused by biliary carcinoma (n = 16), pancreatic carcinoma (n = 2), hepatoma (n = 2), metastasis in liver (n = 3) and lymph node metastasis (n = 10). Six types and 38 metallic stents were used. After stentering, 3 cases were treated with intrastent brachytherapy, 7 cases treated with external radiotherapy and 2 patients treated with interventional radiology. Patients were followed for 6-28 months, mean 11 months. Results: Stent placement was successful in all 33 cases. The same time, 4 patients were treated with plastic tube for external drainage only. The successful rate was 90%. Twenty-nine patients were placed in one time, 4 cases began with plastic tube which was replaced with metallic stents after 1-2 weeks. Nine had metallic stents and plastic tube, because of complicated obstruction. The external drainage catheters were removed after 15-200 days. Three patients had stents crossing the ampulla. In 28 cases (84.85%), jaundice was reduced satisfactorily while in 5 cases with complicated biliary obstruction, the result was unsatisfactory. The median survival was 7 months. After stentering, 12 cases were treated with locat chemotherapy, brachytherapy or radiotherapy. The median survival period was 10 months. Conclusion: The metallic stent provides good palliative drainage and is well tolerated by patients. It improves the survival rate when combined with radiotherapy, brachytherapy and chemotherapy in patients with malignant biliary obstruction

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

    Directory of Open Access Journals (Sweden)

    Li-Zsa Tan

    2015-07-01

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

  13. Biliary cholesterol secretion : More than a simple ABC

    NARCIS (Netherlands)

    Dikkers, Arne; Tietge, Uwe J. F.

    2010-01-01

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

  14. Reactivity of Biliatresone, a Natural Biliary Toxin, with Glutathione, Histamine, and Amino Acids.

    Science.gov (United States)

    Koo, Kyung A; Waisbourd-Zinman, Orith; Wells, Rebecca G; Pack, Michael; Porter, John R

    2016-02-15

    In our previous work, we identified a natural toxin, biliatresone, from Dysphania glomulifera and D. littoralis, endemic plants associated with outbreaks of biliary atresia in Australian neonatal livestock. Biliatresone is a very rare isoflavonoid with an α-methylene ketone between two phenyls, 1,2-diaryl-2-propenone, along with methylenedioxy, dimethoxyl, and hydroxyl functional groups, that causes extrahepatic biliary toxicity in zebrafish. The toxic core of biliatresone is a methylene in the α-position relative to the ketone of 1,2-diaryl-2-propenone that serves as an electrophilic Michael acceptor. The α-methylene of biliatresone spontaneously conjugated with water and methanol (MeOH), respectively, via Michael addition in a reverse phase high-performance liquid chromatography (RP-HPLC) analysis. We here report the reactivity of biliatresone toward glutathione (GSH), several amino acids, and other thiol- or imidazole-containing biomolecules. LC-MS and HPLC analysis of the conjugation reaction showed the reactivity of biliatresone to be in the order histidine > N-acetyl-d-cysteine (D-NAC) = N-acetyl-l-cysteine (L-NAC) > histamine > glutathione ≥ cysteine ≫ glycine > glutamate > phenylalanine, while serine and adenine had no reactivity due to intramolecular hydrogen bonding in the protic solvents. The reactivity of ethyl vinyl ketone (EVK, 1-penten-3-one), an example of a highly reactive α,ß-unsaturated ketone, toward GSH gave a 6.7-fold lower reaction rate constant than that of biliatresone. The reaction rate constant of synthetic 1,2-diaryl-2-propen-1-one (DP), a core structure of the toxic molecule, was 10-fold and 1.5-fold weaker in potency compared to the reaction rate constants of biliatresone and EVK, respectively. These results demostrated that the methylenedioxy, dimethoxyl, and hydroxyl functional groups of biliatresone contribute to the stronger reactivity of the Michael acceptor α-methylene ketone toward nucleophiles compared to that of DP

  15. 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.

  16. Biliary obstruction: Helical computed tomography cholangiography evaluation

    International Nuclear Information System (INIS)

    Helical computed tomography cholangiography (HCTC) takes advantage of the improvement in the quality of multiplanar and three-dimensional reconstructions resulting from the volumetric acquisition of data achieved with helical computed tomography and the indirect opacification of the bile ducts provided by cholangiographic contrast media. This method was used to evaluate 31 patients with suspected biliary obstruction over a one-year period and the findings were compared with those of direct cholangiography and/or surgery. Biliary opacification was sufficient to allow three-dimensional reconstructions in 90,3% of cases. Sensitivity was 81% for the diagnosis of choledochoolithiasis and 100% for ascariasis, choledochal cysts and anatomic variants of the biliary tree. Our results indicate that HCTC is a valuable alternative as a diagnostic method prior to direct cholangiography. Direct cholangiography should be reserved for those patients who require it as part of a therapeutic procedure

  17. 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.

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

    DEFF Research Database (Denmark)

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

    2016-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    Day, D.L.

    1985-04-01

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

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

    OpenAIRE

    Emmanuelle Seguier-Lipszyc; Baruch Klin

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Emmanuelle Seguier-Lipszyc

    2014-07-01

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

  2. 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.

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

    OpenAIRE

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

    1993-01-01

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

  4. 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.

  5. 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.

  6. 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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Valérie Huynh-Trudeau

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    E. Brandigi

    2013-12-01

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

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

    OpenAIRE

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

    2015-01-01

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

  11. Acute biliary pancreatitis: Diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    Hazem Zakaria

    2009-01-01

    Full Text Available Gallstones are the commonest cause of acute pancreatitis (AP, a potentially life-threatening condition, worldwide. The pathogenesis of acute pancreatitis has not been fully understood. Laboratory and radiological investigations are critical for diagnosis as well prognosis prediction. Scoring systems based on radiological findings and serologic inflammatory markers have been proposed as better predictors of disease severity. Early endoscopic retrograde cholangiopancreatography (ERCP is beneficial in a group of patients with gallstone pancreatitis. Laparoscopic cholecystectomy with preoperative endoscopic common bile duct clearance is recommended as a treatment of choice for acute biliary pancreatitis. The timing of cholecystectomy, following ERCP, for biliary pancreatitis can vary markedly depending on the severity of pancreatitis

  12. Diagnosis of liver, biliary tract and gastrointestine

    International Nuclear Information System (INIS)

    The role of RI imaging in the diagnosis of lesions of the liver, biliary tracts and gastrointestinal tracts are reviewed, and representative cases are shown. Liver scintigraphy was of value for the diagnosis of lesions limitted to the liver such as primary and metastatic liver cancer and inflammatory liver diseases. However, RI methods were less useful in the diagnosis of lesions of the biliary tracts and stomach. RI scintigraphy was more sensitive than angiography in the detection of Meckel's deverticulum, Ballet's esophagus, and gastrointestinal hemorrhage. (Tsunoda, M.)

  13. 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.

  14. CT diagnosis of biliary tract diseases

    International Nuclear Information System (INIS)

    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. (author)

  15. Persistent bronchography in a newborn with esophageal atresia.

    Science.gov (United States)

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

    2016-06-01

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

  16. Persistent bronchography in a newborn with esophageal atresia

    Directory of Open Access Journals (Sweden)

    Giuseppe De Bernardo, MD

    2016-06-01

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

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

    Science.gov (United States)

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

    1989-02-01

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

  18. 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.

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

    Science.gov (United States)

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

    2013-02-01

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

  20. 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.

  1. 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.

  2. 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.

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

    Directory of Open Access Journals (Sweden)

    Morgane Le Gouëz

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

  4. Atresia of large ovarian follicles of the rat

    Directory of Open Access Journals (Sweden)

    Maria Słomczyn´ ska

    2011-08-01

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

  5. Biliary cystadenoma and choledochal polyp: a rare association

    Directory of Open Access Journals (Sweden)

    A.Y. Lakshmi

    2013-01-01

    Full Text Available Biliary cystadenomas are rare, potentially malignant neoplasms of biliary origin occurring predominantly in middle aged women. Here we report a case of biliary cystadenoma in a young female who presented with an epigastric mass and features of obstructive jaundice. Imaging studies showed a mass in the left lobe of liver with dilated intra hepatic biliary ducts, right hepatic duct and common bile duct. Patient was subjected to left hepatectomy and hepatico-jejunostomy. Histopathology confirmed the lesion as biliary cystadenoma with choledochal polyp.

  6. 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.

  7. 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.

  8. Biliary metal stents are superior to plastic stents for preoperative biliary decompression in pancreatic cancer

    OpenAIRE

    Decker, Christopher; Christein, John D.; Phadnis, Milind A.; Mel Wilcox, C.; Varadarajulu, Shyam

    2011-01-01

    Background It is unclear whether plastic or metal stents are more suitable for preoperative biliary decompression in pancreatic cancer. The objective of this study was to compare the rate of endoscopic reinterventions in patients with pancreatic cancer undergoing plastic or self-expandable metal stent (SEMS) placements for preoperative biliary decompression. Methods This was a retrospective study of all patients with obstructive jaundice secondary to pancreatic head cancer who underwent their...

  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. Rare opening of great biliary ducts-esophagobiliary communication

    Energy Technology Data Exchange (ETDEWEB)

    Sarajlic, M.; Martincic, N.; Paskov, S.; Buljevac, M.; Durst-Zivkovic, B.

    1981-12-01

    A case report of a congenital esophagobiliary communication with atresia of left ductus hepaticus is presented. The communication of the esophagus with the duct of the left liver lobe was examined by giving Esophotrast into esophagus and by intravenous cholangio-cholecystography and tomography. Atresia of the left ductus hepaticus was detected by intraoperative cholangiography. The case is presented because of its rarity and it has only an embryological explanation.

  11. 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.

  12. 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.

  13. Fibrate treatment for primary biliary cirrhosis

    NARCIS (Netherlands)

    Cuperus, F.J.C.; Halilbasic, E.; Trauner, M.

    2014-01-01

    PURPOSE OF REVIEW: Primary biliary cirrhosis (PBC) can lead to end-stage liver disease and death. Ursodeoxycholic acid (UDCA) treatment can normalize serum liver enzymes in PBC, and such UDCA-responsive patients have a similar life expectancy as age and sex-matched controls. Nearly up to 50% of the

  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 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

  16. Linear 125I seeds strand implantation combined with biliary stenting for the treatment of malignant biliary obstruction

    International Nuclear Information System (INIS)

    Objective: To evaluate the therapeutic efficacy of linear 125I seeds strand implantation combined with biliary stenting in treating malignant biliary obstruction. Methods: Linear 125I seeds strand implantation combined with biliary stenting was carried out in 28 patients with malignant biliary obstruction. The technical success rate, the clinical efficacy, the postoperative complications and the survival rate were analyzed. Results: Both biliary stenting and 125I seeds strand implantation were successfully accomplished in all patients. No serious complications occurred. After the procedure the biliary obstruction symptoms were markedly improved and the bilirubin level was significantly reduced (P125I seeds strand implantation together with biliary stenting is safe and effective although its long-term efficacy needs to be further studied. (authors)

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

    Science.gov (United States)

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

    2016-04-01

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

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

    Directory of Open Access Journals (Sweden)

    Shahin Abdollahifakhim

    2014-01-01

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

  19. 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.

  20. 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.

  1. 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.

  2. {sup 99m}Tc-EHIDA Scintigraphic Demonstrability of Biliary Elements and Liver Function Tests in Hepatobiliary Diseases

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Choon Yul; Baik, Yong Whee; Lee, Myung Hee [Catholic University College of Medicine, Seoul (Korea, Republic of)

    1982-09-15

    bile duct, GB and duodenum was closely paralleled to the level of serum bilirubin when it is less than 10.5 mg/dl as shown in figure 1. However when the bilirubin exceed 10.5 mg/dl the time of visualization between protected reaching a flat curve or plateau around 10.5 mg/dl . The biliary tract could not be visualized when the bilirubin was higher than 10.5 mg/dl. The biliary tract could not be visualized demonstration and serum alkaline phosphatase was less strong and between scintigraphic demonstration and SGOT and SGPT was rather poor. The present clinical study confirmed the usefulness and limitation of {sup 99m}Tc-EHIDA hepatobiliary scintigraphy in visualizing and diagnosing the biliary system and duodenum when radiography and ultrasonography failed to provide useful information. Scintigraphy wa very helpful in the diagnosis of neonatal hepatitis, biliary atresia, cholecystitis and extrahepatic biliary obstruction. The hepatobiliary system and duodenum were visualized when serum bilirubin level was less than 10.5 mg/dl, SGOT 135 units, SGPT 114 units and alkaline phosphatase 52.2 KAU.

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

    Science.gov (United States)

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

    2015-01-01

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

  4. 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.

  5. 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.

  6. [Digestive endoscopy: biliary tract and pancreas].

    Science.gov (United States)

    Costamagna, Guido

    2016-06-01

    New technological developments in endoscopic retrograde cholangiopancreatography and endoscopic ultrasound, both for diagnosis and treatment of biliary and pancreatic diseases, have opened up new scenarios in the recent years. For instance, removal of large bile duct stones with endoscopic sphincterotomy followed by large balloon dilation has been proven to be a safe and effective technique. Also cholangioscopy evolved in terms of better imaging, tissue acquisition and stones management. Self-expandable metal stents are used mostly for malignant diseases, but their role in benign diseases has also been deeply investigated in the last years. The rapid evolution in the field of radiofrequency brought new devices for safer ablation and palliation of biliary tumours. Self-expandable metal stents are also used for ultrasound-guided drainage of walled off pancreatic necrosis and for choledocoduodenostomy and cholecystogastrostomy. PMID:27362720

  7. Hemobilia after percutaneous transhepatic biliary drainage

    International Nuclear Information System (INIS)

    Objective: To retrospectively analyze the occurance and management for patients with hemobilia after percutaneous transhepatic biliary drainage (PTBD). Methods: Consecutive 139 patients with inoperable obstructive jaundice were treated by PTBD. After taking percutaneous transhepatic cholangiography, procedure of puncturing the dilated biliary duct with metallic stent or plastic catheter placement was undertaken. Follow up was carded out with clinical, radiographic and laboratory evaluation. Procedure-and device-related complications were also recorded. Results: All patients went though PTBD successfully with total serum bilirnbin reducing from 360 μmol/l to 158.2 μmol/l, postoperatively. 43 cases received hemostatic. Transient hemobilia occurred in 11 cases, and severe hemobilia in other 5 cases requiring further management. Other 4 cases needed arterial embolization with another one failure. Conclusion: Hemobilia is a complication after PTBD, which can be promptly controlled with improving skillful maneuver. (authors)

  8. Recent Advances of Biliary Stent Management

    International Nuclear Information System (INIS)

    Recent progress in chemotherapy has prolonged the survival of patients with malignant biliary strictures, leading to increased rates of stent occlusion. Even we employed metallic stents which contributed to higher rates and longer durations of patency, and occlusion of covered metallic stents now occurs in about half of all patients during their survival. We investigated the complication and patency rate for the removal of covered metallic stents, and found that the durations were similar for initial stent placement and re-intervention. In order to preserve patient quality of life, we currently recommend the use of covered metallic stents for patients with malignant biliary obstruction because of their removability and longest patency duration, even though uncovered metallic stents have similar patency durations.

  9. Endoscopic management of hilar biliary strictures.

    Science.gov (United States)

    Singh, Rajiv Ranjan; Singh, Virendra

    2015-07-10

    Hilar biliary strictures are caused by various benign and malignant conditions. It is difficult to differentiate benign and malignant strictures. Postcholecystectomy benign biliary strictures are frequently encountered. Endoscopic management of these strictures is challenging. An endoscopic method has been advocated that involves placement of increasing number of stents at regular intervals to resolve the stricture. Malignant hilar strictures are mostly unresectable at the time of diagnosis and only palliation is possible.Endoscopic palliation is preferred over surgery or radiological intervention. Magnetic resonance cholangiopancreaticography is quite important in the management of these strictures. Metal stents are superior to plastic stents. The opinion is divided over the issue of unilateral or bilateral stenting.Minimal contrast or no contrast technique has been advocated during endoscopic retrograde cholangiopancreatography of these patients. The role of intraluminal brachytherapy, intraductal ablation devices, photodynamic therapy, and endoscopic ultrasound still remains to be defined. PMID:26191345

  10. Recent advances of biliary stent management.

    Science.gov (United States)

    Kida, Mitsuhiro; Miyazawa, Shiro; Iwai, Tomohisa; Ikeda, Hiroko; Takezawa, Miyoko; Kikuchi, Hidehiko; Watanabe, Maya; Imaizumi, Hiroshi; Koizumi, Wasaburo

    2012-01-01

    Recent progress in chemotherapy has prolonged the survival of patients with malignant biliary strictures, leading to increased rates of stent occlusion. Even we employed metallic stents which contributed to higher rates and longer durations of patency, and occlusion of covered metallic stents now occurs in about half of all patients during their survival. We investigated the complication and patency rate for the removal of covered metallic stents, and found that the durations were similar for initial stent placement and re-intervention. In order to preserve patient quality of life, we currently recommend the use of covered metallic stents for patients with malignant biliary obstruction because of their removability and longest patency duration, even though uncovered metallic stents have similar patency durations. PMID:22563289

  11. Radiotherapy Prolongs Biliary Metal Stent Patency in Malignant Pancreatobiliary Obstructions

    OpenAIRE

    Park, Semi; Park, Jeong Youp; Bang, Seungmin; Park, Seung Woo; Chung, Jae Bock; Song, Si Young

    2013-01-01

    Background/Aims Biliary stenting is the most effective decompressive method for treating malignant biliary obstructive jaundice. Although the main cause of stent occlusion is tumor growth, few studies have investigated whether stent patency is affected by the combination of cancer-treatment modalities. The aim of this study was to evaluate the effects of local radiotherapy on metal-stent patency in patients with malignant biliary obstruction. Methods Patients who underwent self-expandable bil...

  12. Functional Self-Expandable Metal Stents in Biliary Obstruction

    OpenAIRE

    Kwon, Chang-Il; Ko, Kwang Hyun; Hahm, Ki Baik; Kang, Dae Hwan

    2013-01-01

    Biliary stents are widely used not only for palliative treatment of malignant biliary obstruction but also for benign biliary diseases. Each plastic stent or self-expandable metal stent (SEMS) has its own advantages, and a proper stent should be selected carefully for individual condition. To compensate and overcome several drawbacks of SEMS, functional self-expandable metal stent (FSEMS) has been developed with much progress so far. This article looks into the outcomes and defects of each st...

  13. Evaluation of different percutaneous modalities for managing malfunctioning biliary stents

    OpenAIRE

    M. Alwarraky; A. Aljaky; E. Tharwa; Aziz, A.

    2015-01-01

    Background: There is no consensus regarding optimal management of biliary metal stent occlusion. Aim: To evaluate the efficacy of different percutaneous methods for managing biliary metal stent occlusion. Patients and methods: Thirty-eight patients were included in the study. Metal biliary stent occlusions were managed by insertion of another metal stent in 18 patients (group 1), insertion of an internal–external catheter in 15 patients (group 2), and mechanical cleaning in 5 patients (...

  14. Current Therapy and Future Directions in Biliary Tract Malignancies

    OpenAIRE

    Ciombor, Kristen K; Goff, Laura W.

    2013-01-01

    Cancers of the biliary tree represent a rare group of diseases with a devastating impact on patients. Gallbladder cancer is often associated with cholelithiasis. Cholangiocarcioma may arise in the setting of biliary inflammation such as primary sclerosing cholangitis but most commonly occurs in patients without a particular risk factor. Surgical removal of biliary cancer is essential for cure, but it is associated with a very high rate of recurrence and for many patients is not possible at th...

  15. Photodynamic therapy for occluded biliary metal stents

    Science.gov (United States)

    Roche, Joseph V. E.; Krasner, Neville; Sturgess, R.

    1999-02-01

    In this abstract we describe the use of photodynamic therapy (PDT) to recanalize occluded biliary metal stents. In patients with jaundice secondary to obstructed metal stents PDT was carried out 72 hours after the administration of m THPC. Red laser light at 652 nm was delivered endoscopically at an energy intensity of 50 J/cm. A week later endoscopic retrograde cholangiogram showed complete recanalization of the metal stent.

  16. Biliary tract obstruction in chronic pancreatitis

    OpenAIRE

    Abdallah, Abdul A.; Krige, Jake E J; Bornman, Philippus C.

    2007-01-01

    Bile duct strictures are a common complication in patients with advanced chronic pancreatitis and have a variable clinical presentation ranging from an incidental finding to overt jaundice and cholangitis. The diagnosis is mostly made during investigations for abdominal pain but jaundice may be the initial clinical presentation. The jaundice is typically transient but may be recurrent with a small risk of secondary biliary cirrhosis in longstanding cases. The management of a bile duct strictu...

  17. Pulmonary involvement in primary biliary cirrhosis.

    OpenAIRE

    Rodriguez-Roisin, R.; Pares, A; Bruguera, M; Coll, J; Picado, C.; Agusti-Vidal, A; Burgos, F.; Rodes, J

    1981-01-01

    The association of pulmonary fibrosis and primary biliary cirrhosis (PBC) remains controversial. To determine the frequency of pulmonary fibrosis in PBC, a carefully selected series of 14 PBC patients, seven patients with Sicca complex, and 14 control subjects have been studied. Seven of the 14 patients with PBC had Sjögren's syndrome, four of whom had some clinical evidence of pulmonary disease. Evaluation of ventilatory capacity, gas transfer factor, arterial blood gases, and lung mechanics...

  18. Colon Cancer Metastatic to the Biliary Tree.

    Science.gov (United States)

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

    2016-04-01

    Metastasis of colon adenocarcinoma is commonly found in the lung, liver, or peritoneum. Common bile duct (CBD) tumors related to adenomas from familial adenomatous polyposis metastasizing from outside of the gastrointestinal tract have been reported. We report a case of biliary colic due to metastatic colon adenocarcinoma to the CBD. Obstructive jaundice with signs of acalculous cholecystitis on imaging in a patient with a history of colon cancer should raise suspicion for metastasis to CBD. PMID:27144209

  19. 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...

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

    OpenAIRE

    Story, Brian; Gluck, Michael

    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 biliary 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 difficult to eradicate. We present 4 cases of obstructing fungal cholangitis in patients who had metal biliary stents placed for pancreatic malignancies, ...

  1. 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.

  2. 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.

  3. 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.

  4. 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

  5. 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.

  6. 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

  7. 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.

  8. Bilothorax as a complication of percutaneous transhepatic biliary drainage.

    Science.gov (United States)

    Sano, Atsushi; Yotsumoto, Takuma

    2016-01-01

    We report two cases of bilothorax that occurred as a complication of percutaneous transhepatic biliary drainage. In an 86-year-old woman who had undergone percutaneous transhepatic biliary drainage for obstructive jaundice, bilothorax occurred after accidental removal of the tube. She recovered with chest drainage only. An 83-year-old man who had undergone percutaneous transhepatic biliary drainage for cholecystitis developed bilothorax with infection. He recovered with thoracoscopic curettage. Although bilothorax is a rare complication of percutaneous transhepatic biliary drainage, appropriate diagnosis and prompt treatment is important, especially when bilothorax is accompanied by infection. PMID:26294694

  9. 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.

  10. Biliary fascioliasis--an uncommon cause of recurrent biliary colics: report of a case and brief review.

    Science.gov (United States)

    Al Qurashi, Hesham; Masoodi, Ibrahim; Al Sofiyani, Mohammad; Al Musharaf, Hisham; Shaqhan, Mohammed; All, Gamal Nasr Ahmed Abdel

    2012-01-01

    Biliary parasitosis is one of the important causes of biliary obstruction in endemic areas, however due to migration and travel the disease is known to occur in non endemic zones as well. The spectrum of biliary fascioliasis ranges from recurrent biliary colics to acute cholangitis. The long term complications are gall stones, sclerosing cholangitis and biliary cirrhosis. We describe fascioliasis as a cause of recurrent biliary colics in a young male necessitating multiple hospitalizations over a period of four years. Investigative profile had been non-contributory every time he was hospitalized for his abdominal pain prior to the current presentation. He never had cholangitis due to the worm in the common bile duct. It was only at endoscopic retrograde cholangiopancreatography (ERCP) biliary fascioliasis was discovered to be the cause of his recurrent biliary colics. After removal of the live Fasciola hepatica from the common bile duct he became symptom free and is attending our clinic for last 11 months now. Clinical spectrum of biliary fascioliasis is discussed in this report. PMID:22566787

  11. 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.

  12. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1995-02-15

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

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

    OpenAIRE

    Saeed, Muhammad; Anwar ul HAQ; Khaqan QADIR

    2014-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Science.gov (United States)

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

    1992-01-01

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

  18. Intrahepatic biliary injuries associated with radiofrequency ablation of hepatic malignancies

    Institute of Scientific and Technical Information of China (English)

    FU Ying; YANG Wei; WU Jin-yu; YAN Kun; WU Wei; XING Bao-cai; CHEN Min-hua

    2011-01-01

    Background Biliary injury after radiofrequency ablation can cause serious consequences including death. However, there are limited data regarding bile duct changes with or without complications associated with radiofrequency ablation of hepatic malignancies. This study aimed to assess the incidence, prognosis and risk factors of intrahepatic biliary injury associated with radiofrequency ablation.Methods Between June 2001 and January 2009, 638 patients with hepatic malignancies (405 with hepatocellular carcinoma, and 233 with liver metastasis) who had 955 treatment sessions were enrolled in this study. Imaging and laboratory data, the course of treatment, and patient outcomes were reviewed retrospectively. The risk factors of biliary injury and the impact on overall survival of patients were analyzed. The chi-square test, Fisher's exact test, Kaplan-Meier curves and stepwise Logistic regression model were used for statistical analysis where appropriate.Results Biliary injury was observed in 17 patients after 17 ablation sessions based on imaging findings. The overall incidence of biliary injury was 1.8% (17/955) with an average onset time of 12 weeks (2-36 weeks). Mild, moderate and severe complications of biliary injury were identified in 9, 6 and 2 cases, respectively. The median survival time after detection of biliary injury was 40 months. There seemed no notable difference in overall survival between patients with and those without biliary injuries. By multivariate analysis, vessel infiltration (P=0.034) and treatment session ≥4 times (P=0.025) were independent risk factors for biliary injury of hepatocellular carcinoma; while tumor located centrally was the only independent risk factor in the metastasis group (P=0.043).Conclusions The incidence of biliary injury was not frequent (1.8%). Through appropriate treatment, intrahepatic bile duct injuries seemed not affect the patients' long-term survival. Additionally, risk factors may be helpful for selecting

  19. 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.

  20. Treatment of malignant biliary obstruction by combined percutaneous transhepatic biliary drainage with local tumor treatment

    Institute of Scientific and Technical Information of China (English)

    Xiao-Jun Qian; Ren-You Zhai; Ding-Ke Dai; Ping Yu; Li Gao

    2006-01-01

    AIM: To evaluate the utility of local tumor therapy combined with percutaneous transhepatic biliary drainage (PTBD) for malignant obstructive biliary disease.METHODS: A total of 233 patients with malignant biliary obstruction were treated in our hospital with PTBD by placement of metallic stents and/or plastic tubes. After PTBD, 49 patients Accepted: brachytherapy or extraradiation therapy or arterial infusion chemotherapy. The patients were followed up with clinical and radiographic evaluation. The survival and stent patency rate were calculated by Kaplan-Meier survival analysis.RESULTS: Twenty-two patients underwent chemotherapy (11 cases of hepatic carcinoma, 7 cases of pancreatic carcinoma, 4 cases of metastatic lymphadenopathy), and 14 patients received radiotherapy (10 cases of cholangiocarcinoma, 4 cases of pancreatic carcinoma), and 13patients Accepted: brachytherapy (7 cases of cholangiocarcinoma, 3 cases of pancreatic carcinoma, 4 cases of metastatic lymphadenopathy). The survival rate of the local tumor treatment group at 1, 3, 6, and 12 months was 97.96%, 95.92%, 89.80%, and 32.59% respectively,longer than that of the non treatment group. The patency rate at 1, 3, 6, and 12 months was 97.96%, 93.86%,80.93%, and 56.52% respectively. The difference of patency rate was not significant between treatment group and non treatment group.CONCLUSION: Our results suggest that local tumor therapy could prolong the survival time of patients with malignant biliary obstruction, and may improve stent patency.

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

    Science.gov (United States)

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

    2012-11-01

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

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

    International Nuclear Information System (INIS)

    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.)

  3. 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.)

  4. Percutaneous management of tumoral biliary obstruction in children

    International Nuclear Information System (INIS)

    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.)

  5. Colchicine for primary biliary cirrhosis. Protocol for a Cochrane Review

    DEFF Research Database (Denmark)

    Gong, Y; Gluud, C

    2003-01-01

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

  6. 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

  7. Percutaneous management of tumoral biliary obstruction in children

    Energy Technology Data Exchange (ETDEWEB)

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

    2007-10-15

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

  8. 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.

  9. 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.

  10. Percutaneous cholangioscopy in obstructed biliary metal stents

    International Nuclear Information System (INIS)

    Purpose. To reevaluate the reasons for the occlusion of self-expanding biliary metal stents, on the basis of cholangioscopic findings. Methods. Percutaneous transhepatic cholangioscopy (PTCS) was performed in 15 patients with obstructed biliary Wallstents. The reason for stent insertion was a malignant obstruction in 14 patients; 1 had a benign biliary stricture. Conventional noncovered stents had been inserted in 12 patients; in 3 cases a polyurethane-covered prototype Wallstent had been used. Stent occlusions occurred after 1-55 months. PTCS was performed with a 2.3-mm endoscope through an 11 Fr sheath. Biopsies were taken via the working channel of the endoscope. Results. In all patients with noncovered stents the inner surface of the stent was highly irregular with seaweed-like protrusions (biopsy-proven granulation tissue). Stent incorporation varied from absent (n=1) to subtotal (n=8), but was always incomplete, no matter how long the stent had been in place. Tumor ingrowth was histologically proven in 2 patients. One patient had a large occluding concrement at the proximal end of the stent. In patients with covered stents, the inner surface appeared more regular; however, viable granulation tissue was found inside two stents and tumor ingrowth in one of them. Conclusion. PTCS showed that incorporation of the stent is virtually always incomplete. The factors contributing most to stent occlusion are the buildup of granulation tissue, bile sludge, and tumor overgrowth. Stone formation and tumor ingrowth can also be important, although less common causes of occlusion. A polyurethane stent covering could not prevent tumor ingrowth in one patient and the buildup of viable granulation tissue inside the stent in two further patients; mean stent patency in the three patients with such a stent was 3 months

  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. 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. PMID:402305

  13. 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

  14. Successful endoscopic ultrasound-guided overstenting biliary drainage through a pre-existing proximal migrated metal biliary stent.

    Science.gov (United States)

    Artifon, E L A; Takada, J; Okawa, L; Ferreira, F; Santos, M; Moura, E G H; Otoch, J P; Sakai, P

    2011-01-01

    Biliary endoscopic drainage using metallic self-expanded stents has become a well-established method for palliative treatment of malignant biliary obstruction. However, its occlusion, mainly by tumor overgrowth, is still the main complication without a standard treatment. We here describe a new method of treatment for biliary metallic stent occlusion, through the echo guided biliary drainage. We present a 68-year-old patient with metastatic pancreatic cancer previously treated for jaundice with ERCP and self-expandable metallic stent insertion. Four weeks later, the patient developed jaundice and symptoms of gastric outlet obstruction. A new ERCP confirmed obstruction of the second portion of the duodenum, due to diffuse tumor growth. EUS was performed, and the previous metal biliary stent was seen occluded at the distal portion in the common bile duct. A EUS-guided choledocododenostomy was performed and then, an overlapping self-expanding metal enteral stent was placed through the malignant obstruction. There were no early complications and the procedure was also clinically effective in relieving jaundice and gastric outlet obstruction symptoms. If ERCP fails in the management of occluded biliary metallic stents, EUS biliary drain can provide effective biliary decompression and should be considered an alternative to other endoscopic techniques. PMID:22041320

  15. 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...

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    D K Kandpal

    2013-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Nandini K Bedi

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Leecarlo Milano

    2016-02-01

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

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

    NARCIS (Netherlands)

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

    2007-01-01

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

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

    NARCIS (Netherlands)

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

    2002-01-01

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

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

    NARCIS (Netherlands)

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

    2015-01-01

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

  3. 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.

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

    Directory of Open Access Journals (Sweden)

    F. Destro

    2014-06-01

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

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

    Directory of Open Access Journals (Sweden)

    Leecarlo Millano

    2015-05-01

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

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

    Science.gov (United States)

    Fathi, Mehdi; Joudi, Marjan; Morteza, Afsaneh

    2015-12-01

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

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

    OpenAIRE

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

    2016-01-01

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

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

    NARCIS (Netherlands)

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

    2003-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Damian Bednarczyk

    2014-03-01

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

  10. Elevated serum levels of a biliary glycoprotein (BGP I) in patients with liver or biliary tract disease

    International Nuclear Information System (INIS)

    Human hepatic bile contains a glycoprotein (Biliary glycoprotein I BGP I) which cross-reacts with the carcinoembryonic antigen (CEA). A radioimmunoassay for BGP I was developed. The interference of CEA or 'non-specific cross-reacting antigen' (NCA) was small. The serum levels of BGP I were determined in healthy subjects, in patients with hepato-biliary diseases and in patients with various infections or inflammatory disorders. Healthy individuals, including pregnant women, had a serum BGP I concentration of about 0.5 - 1 mg/l. Diseases of the liver or biliary tract (e.g. hepatitis A or B, cytomegalovirus Hepatitis, obstructive jaundice or primary biliary cirrhosis) were associated with elevated serum levels of BGP I, as opposed to infectious diseases not affecting the liver mostly showing values within the normal range. Raised levels of serum BGP I activity may reflect biliary obstruction as a results of interference with normal BGP I secretion in the bile. (author)

  11. 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.

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

    Directory of Open Access Journals (Sweden)

    Lidia B. Giúdici

    2016-07-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2006-11-15

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

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

    Directory of Open Access Journals (Sweden)

    Marzieh Ghorbani

    2016-07-01

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

  15. EUS-guided biliary drainage with placement of a new partially covered biliary stent for palliation of malignant biliary obstruction: a case series.

    Science.gov (United States)

    Fabbri, C; Luigiano, C; Fuccio, L; Polifemo, A M; Ferrara, F; Ghersi, S; Bassi, M; Billi, P; Maimone, A; Cennamo, V; Masetti, M; Jovine, E; D'Imperio, N

    2011-05-01

    Endoscopic ultrasonography-guided biliary drainage (EUS-BD) has been developed as an alternative drainage technique in patients with obstructive jaundice where endoscopic retrograde cholangiopancreatography (ERCP) has failed. Between July 2008 and December 2009, 16 patients (9 men; median age 79 years) with biliopancreatic malignancy, who were candidates for alternative techniques of biliary decompression because ERCP had been unsuccessful, underwent EUS-BD with placement of a transmural or transpapillary partially covered nitinol self-expandable metal stent (SEMS). EUS-assisted cholangiography was successful in all patients, with definition of the relevant anatomy, but biliary drainage was successfully performed in only 12 (75 %) of the 16 patients (9 choledochoduodenostomies with SEMS placement and 3 biliary rendezvous procedures with papillary SEMS placement), with regression of the cholestasis. No major complications and no procedure-related deaths occurred. There was one case of pneumoperitoneum which was managed conservatively. The median follow-up was 170 days. During the follow-up, eight patients of the 12 patients in whom biliary draining was successful died; four are currently alive. None of the patients required endoscopic reintervention. This series demonstrated that EUS-BD with a partially covered SEMS has a high rate of clinical success and low complication rates, and could represent an alternative choice for biliary decompression. PMID:21271507

  16. 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.

  17. 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.

  18. Malignant biliary obstruction: From palliation to treatment

    Science.gov (United States)

    Boulay, Brian R; Birg, Aleksandr

    2016-01-01

    Malignant obstruction of the bile duct from cholangiocarcinoma, pancreatic adenocarcinoma, or other tumors is a common problem which may cause debilitating symptoms and increase the risk of subsequent surgery. The optimal treatment - including the decision whether to treat prior to resection - depends on the type of malignancy, as well as the stage of disease. Preoperative biliary drainage is generally discouraged due to the risk of infectious complications, though some situations may benefit. Patients who require neoadjuvant therapy will require decompression for the prolonged period until attempted surgical cure. For pancreatic cancer patients, self-expanding metallic stents are superior to plastic stents for achieving lasting decompression without stent occlusion. For cholangiocarcinoma patients, treatment with percutaneous methods or nasobiliary drainage may be superior to endoscopic stent placement, with less risk of infectious complications or failure. For patients of either malignancy who have advanced disease with palliative goals only, the choice of stent for endoscopic decompression depends on estimated survival, with plastic stents favored for survival of stent patency and patient survival for these patients by achieving local control of the obstructing tumor. Both photodynamic therapy and radiofrequency ablation may play a role in extending survival of patients with malignant biliary obstruction. PMID:27326319

  19. [Appropriate Biliary Drainage Methods for Unresectable Cholangiocarcinomas].

    Science.gov (United States)

    Oishi, Tatsurou; Kanemoto, Yoshiaki; Yoshioka, Yuuta; Sawada, Ryuuichirou; Sekine, Sachi; Miyanaga, Hiroto; Sakahira, Hideki; Takahashi, Hironori; Miyamoto, Katsufumi; Koyama, Takashi

    2015-11-01

    We investigated the efficacy of different biliary drainage methods for the treatment of unresectable cholangiocarcinomas. We performed a retrospective study of 28 patients with unresectable cholangiocarcinomas who underwent biliary drainage at our hospital between January 2008 and June 2014 to compare the incidence of post-drainage stent dysfunction (SD) and reintervention (RI) for SD according to primary drainage method, lesion site, and complication status (the presence or absence of cholangitis). The duration of stent patency was compared between the different stent types. No significant differences in the incidence of SD and RI were found according to primary drainage methods, lesion site, or the presence or absence of cholangitis. The mean durations of stent patency for plastic and metal stents were 2.7 months and 7.4 months, respectively, suggesting that metal stents should be selected when the estimated prognosis is ≥2 months. Furthermore, metal stent placement, rather than the additional placement of plastic stents, should be considered a feasible option in cases of SD. PMID:26805093

  20. Metallic stents in malignant biliary obstruction

    International Nuclear Information System (INIS)

    Purpose. Retrospective analysis of our results with metallic stent placement for malignant biliary strictures. We sought to determine parameters that influence stent patency. Methods. A total of 95 Wallstents were implanted in 65 patients (38 men, 27 women; mean age, 65.1 years) with malignant biliary obstruction. Serum bilirubin levels were assessed in 48 patients; the mean value prior to intervention was 15.0 mg/dl. Results. In 12 patients (21%) complications occurred as a result of percutaneous transhepatic drainage. Stent implantation was complicated in 13 patients, but was possible in all patients. A significant decrease in bilirubin level was seen in 83.3% of patients following stent implantation. Approximately 30% of patients developed recurrent jaundice after a mean 97.1 days. In 9 patients (15%) the recurrent jaundice was caused by stent occlusion due to tumor growth. The mean follow-up was 141.8 days, the mean survival 118.7 days. Patients with cholangiocarcinomas and gallbladder carcinomas had the best results. Worse results were seen in patients with pancreatic tumors and with lymph node metastases of colon and gastric cancers. Conclusions. The main predictive factors for occlusion rate and survival are the type of primary tumor, tumor stage, the decrease in bilirubin level, and the general condition of the patient

  1. Successful Endoscopic Ultrasound-Guided Transduodenal Biliary Drainage Through a Pre-Existing Duodenal Stent

    OpenAIRE

    Paul J Belletrutti; Hans Gerdes; Schattner, Mark A

    2010-01-01

    Context When ERCP fails in the setting of combined biliary and duodenal obstruction, EUS-guided biliary drainage has emerged as an alternate method of biliary decompression. Case report We present a case of a 40-year-old man with advanced pancreatic cancer and a pre-existing duodenal wall stent who subsequently develops jaundice due to biliary obstruction. An ERCP was technically unsuccessful as the papilla was inaccessible despite probing within the duodenal stent. Transduodenal biliary drai...

  2. Therapeutic transjejunal endoscopy for the treatment of biliary complications after choledochojejunostomy

    OpenAIRE

    Liu, Guo-Ping; Wen-xi ZHU; CHENG, GUANG-MING; Shu-ren MA

    2012-01-01

    The present study aimed to assess the value of endoscopic jejunostomy for post-biliary intestinal anastomosis biliary complications. The clinical data of the endoscopic therapies by jejunal approach for post-biliary intestinal anastomosis biliary complications in 13 patients (16 surgeries in total) were retrospectively analyzed. The surgical success rate was 100% (16/16). Nasobiliary tube detention was performed for 2 patients, plastic stent placement for 5 and biliary metal stent placement f...

  3. Successful endoscopic treatment of biliary stricture following mesenteric tear caused by blunt abdominal trauma

    Institute of Scientific and Technical Information of China (English)

    Dong O Kang; Tae Hyo Kim; Seung Suk You; Hyun Ju Min; Hyun Jin Kim; Woon Tae Jung; Ok Jae Lee

    2008-01-01

    Biliary duct injuries are frequently iatrogenic, being associated with surgery for gallbladder stones. However,blunt abdominal trauma such as a motor vehicle crash is a rare cause of extrahepatic biliary stricture. A few reports have been published on biliary strictures treated with endoscopic therapy. In the present study, we describe a suprapancreatic biliary stricture associated with mesenteric tear following road traffic accident.We performed endoscopic stent placement, which was successful in relieving the biliary stricture.

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

    International Nuclear Information System (INIS)

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

  5. Spectrum of biliary complications following live donor livertransplantation

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    Liver transplantation is the optimal treatment formany patients with advanced liver disease, includingdecompensated cirrhosis, hepatocellular carcinomaand acute liver failure. Organ shortage is the maindeterminant of death on the waiting list and hence livingdonor liver transplantation (LDLT) assumes importance.Biliary complications are the most common post operativemorbidity after LDLT and occur due to anatomical andtechnical reasons. They include biliary leaks, stricturesand cast formation and occur in the recipient as well asthe donor. The types of biliary complications after LDLTalong with their etiology, presenting features, diagnosisand endoscopic and surgical management are discussed.

  6. Management of benign biliary strictures: current status and perspective.

    Science.gov (United States)

    Kaffes, Arthur J

    2015-09-01

    Benign biliary strictures are common and occur either from hepato-biliary surgery or from diseases including chronic pancreatitis and primary sclerosing cholangitis, among others. The treatment of many such strictures is endoscopic with evolving new approaches especially with fully covered metal stents. The only classification system available is for postoperative strictures with the intention to guide surgical correction. There is no useful classification system to guide both assessment and management of benign biliary strictures. This proposed classification is relevant to patient care in assisting diagnosis and endoscopic management. PMID:26147976

  7. Metallic stents for management of malignant biliary obstruction

    International Nuclear Information System (INIS)

    In patients with inoperable malignant biliary obstruction, percutaneous transhepatic biliary drainage (PTBD) has been the method of choice for palliative treatment. All patients except three had undergone PTBD, and the stents were placed 5-7 days after the initial drainage procedure. Three patients underwent stent placement on the same day of PTBD. External drainage catheter is converted to various types of tube endoprostheses with associated physiologic and psychologic benefits. Tube stents, however, have some problems such as migration, occlusion, and traumatic implantation procedure. We report our experiences and clinical results of percutaneous placement of metallic stents in 40 patients with malignant biliary obstruction

  8. Metallic stents for management of malignant biliary obstruction

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Byung Hee; Do, Young Soo; Byun, Hong Sik; Kim, Kie Hwan; Chin, Soo Yil [Korea Cancer Center Hospital, Seoul (Korea, Republic of)

    1992-11-15

    In patients with inoperable malignant biliary obstruction, percutaneous transhepatic biliary drainage (PTBD) has been the method of choice for palliative treatment. All patients except three had undergone PTBD, and the stents were placed 5-7 days after the initial drainage procedure. Three patients underwent stent placement on the same day of PTBD. External drainage catheter is converted to various types of tube endoprostheses with associated physiologic and psychologic benefits. Tube stents, however, have some problems such as migration, occlusion, and traumatic implantation procedure. We report our experiences and clinical results of percutaneous placement of metallic stents in 40 patients with malignant biliary obstruction.

  9. 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.

  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. PMID:26327757

  11. Biliary enhanced MR imaging by Gd-DTPA

    Energy Technology Data Exchange (ETDEWEB)

    Ohkawa, Shinichi; Fujikura, Yuji; Kanai, Toshio (Hiratsuka City Hospital, Kanagawa (Japan)); Hiramatsu, Kyoichi

    1992-05-01

    Biliary enhanced MRI (BEMRI) by Gd-DTPA via PTCD and/or PTGBD tube for obstructive jaundice was performed in 8 patients. In all cases, biliary tract was clearly visualised as high signal intensity on T1 weighted images. On same images, primary lesion such as common bile duct cancer was also visualised as well as portal system. In addition, MR angiography (MRA) by 2D-time of flight method was performed. MRA with BEMRI shows portal encasement on the same image as biliary tract obstruction. This suggests MRA with BEMRI may replace the other modality for obstructive jaundice. (author).

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

    Science.gov (United States)

    Blackmore, Kate; Wynne, David M

    2010-08-01

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

  13. 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.

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

    International Nuclear Information System (INIS)

    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

  15. 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.

  16. Primary Biliary Cirrhosis: Environmental Risk Factors

    Directory of Open Access Journals (Sweden)

    Deepti Dronamraju

    2010-01-01

    Full Text Available Primary biliary cirrhosis (PBC is an autoimmune disease of unclear etiology. It is a chronic, progressive condition that causes intrahepatic ductal destruction ultimately leading to symptoms of cholestasis, cirrhosis and liver failure. The disease predominantly affects middle aged Caucasian women. It has a predilection to certain regions and is found in higher incidences in North America and Northern Europe. It also has a genetic predisposition with a concordance rate of 60% among monozygotic twins. Combinations of genetic and environmental factors are proposed in the pathogenesis of this disease with a compelling body of evidence that suggests a role for both these factors. This review will elucidate data on the proposed environmental agents involved the disease's pathogenesis including xenobiotic and microbial exposure and present some of the supporting epidemiologic data.

  17. Ursodeoxycholic acid for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Gong, Y.; Huang, Z.B.; Christensen, Erik;

    2008-01-01

    or liver transplantation. Binary outcomes were reported as odds ratio (OR) or relative risk (RR) and continuous outcomes as weighted mean difference, all with 95% confidence intervals (CI). Meta-regression was used to investigate the associations between UDCA effects and quality of the trial, UDCA dose...... trials have been updated. Nearly half of the trials had high risk of bias. The combined results demonstrated no significant effects favouring UDCA on mortality (OR 0.97, 95% CI 0.67 to 1.42) and mortality or liver transplantation (RR 0.92, 95% CI 0.71 to 1.21). The findings were supported by the Bayesian...... associated with adverse events, mainly weight gain. AUTHORS' CONCLUSIONS: This systematic review did not demonstrate any benefit of UDCA on mortality and mortality or liver transplantation of patients with primary biliary cirrhosis. The few beneficial effects could not be due to random errors or outcome...

  18. 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.

  19. 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.

  20. LAPAROSCOPIC ANATOMY OF THE EXTRAHEPATIC BILIARY TRACT

    Directory of Open Access Journals (Sweden)

    E. Târcoveanu

    2005-01-01

    Full Text Available Development of mini-invasive surgery determinates a rapid improvement in laparoscopic regional anatomy. As laparoscopy is becoming common in most surgical departments, basic laparoscopic anatomy is mandatory for all residents in general surgery. Successful general surgery starts in the anatomy laboratory. Successfully minim invasive surgery starts in the operative theatre with laparoscopic exploration. The initial laparoscopic view of the right upper quadrant demonstrates primarily the subphrenic spaces, abdominal surface of the diaphragm and diaphragmatic surface of the liver. The falciform ligament is a prominent dividing point between the left subphrenic space and the right subphrenic space. The ligamentum teres hepatis is seen in the free edge of the falciform. Upward traction on the gallbladder exposes the structures of Calot’s triangle and the hepatoduodenal ligament. The liver is divided into anatomic segments based on internal anatomy that is invisible to the laparoscopist. Surface landmarks include the falciform ligament and the gallbladder fossa. The surgical procedures performed laparoscopically currently include liver biopsy, wedge resection, fenestration of hepatic cysts, laparoscopic approach of the hidatid hepatic cyst, and atypical hepatectomy. We present the laparoscopic anatomy of extrahepatic biliary tract. Once the gallbladder is elevated, inspection reveals Hartmann’s pouch and the cystic duct. The typical angular junction of the cystic duct on the common duct actually occurs in a minority of patients and the length and course of the cystic duct are highly variable. The boundaries of Calot’s triangle are often not well seen. The cystic artery is often visible under the peritoneum as it runs along the surface of the gallbladder. The variations of the structures of the hepatoduodenal ligament may occur to injuries during laparoscopic cholecystectomy. Cholangiography increases the safety of dissection of biliary tract by

  1. 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.

  2. Evidence-Based Decompression in Malignant Biliary Obstruction

    International Nuclear Information System (INIS)

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

  3. 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...

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

    DEFF Research Database (Denmark)

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

    1999-01-01

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

  5. Mucin-hypersecreting biliary neoplasms: two case report

    Energy Technology Data Exchange (ETDEWEB)

    Lim, Gye Yon; Lee, Jae Mun; Park, Jeong Mi; Jung, So Lyung; Kim, Choon Yul; Shinn, Kyung Sub [Catholic University Medical College, Seoul (Korea, Republic of)

    1995-09-15

    Mucin-hypersecreting biliary neoplasm excretes excessive mucin that fills the biliary tree and results in marked dilatation of the bile ducts and obstructive jaundice. In these neoplasm, the much produced by the tumor rather than the tumor itself plays an important role in clinical course and radiologic patterns. The purpose of this paper is to report characteristic radiologic patterns of mucin-hypersecreting biliary neoplasms in two cases. These neoplasms were characterized by not only multilocular cystic hepatic mass or extra-hepatic bile duct mass resulting in marked biliary dilatation distal to the mass on US or CT, but also change of shape and extent of amorphous filling defects in the markedly dilated bile duct on serial cholangiograms.

  6. Role of stents and laser therapy in biliary strictures

    Science.gov (United States)

    Chennupati, Raja S.; Trowers, Eugene A.

    2001-05-01

    The most frequent primary cancers causing malignant obstructive jaundice were pancreatic cancer (57%), hilar biliary cancer (19% including metastatic disease), nonhilar biliary cancer (14%) and papillary cancer (10%). Endoscopic stenting has widely replaced palliative surgery for malignant biliary obstruction because of its lower risk and cost. Self-expandable metal stents are the preferred mode of palliation for hilar malignancies. Plastic stents have a major role in benign biliary strictures. Major complications and disadvantages associated with metallic stents include high cost, cholangitis. malposition, migration, unextractability, and breakage of the stents, pancreatitis and stent dysfunction. Dysfunction due to tumor ingrowth can be relieved by thermal methods (argon plasma coagulator therapy). We present a concise review of the efficacy of metallic stents for palliation of malignant strictures.

  7. Evidence-Based Decompression in Malignant Biliary Obstruction

    Energy Technology Data Exchange (ETDEWEB)

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

    2012-02-15

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

  8. 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.

  9. Biliary Sludge: A Risk Factor for ‘Idiopathic’ Pancreatitis?

    OpenAIRE

    Marotta, Paul J.; Gregor, James C; Taves, Donald H.

    1996-01-01

    Idiopathic acute pancreatitis is common. Recent evidence suggests that biliary sludge may be the etiology in many patients with this disorder. In this case-control study, admission ultrasound examinations of patients with idiopathic pancreatitis, patients with acute alcohol-associated pancreatitis and a control group were compared. Biliary sludge was found in seven of 21 patients (33%) with idiopathic pancreatitis, two of 25 (8%) with acute alcohol-associated pancreatitis and one of 63 contro...

  10. Management of Simultaneous Biliary and Duodenal Obstruction: The Endoscopic Perspective

    OpenAIRE

    Baron, Todd H

    2010-01-01

    Obstructive jaundice often develops in patients with unresectable malignancy in and around the head of the pancreas. Duodenal obstruction can also occur in these patients, and usually develops late in the disease course. Palliation of both malignant biliary and duodenal obstruction is traditionally performed with surgical diversion of the bile duct and stomach, respectively. With the advent of nonsurgical palliation of biliary obstruction using endoscopic transpapillary expandable metal stent...

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

    OpenAIRE

    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, ...

  12. 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...

  13. Primary biliary cirrhosis: geographical clustering and symptomatic onset seasonality.

    OpenAIRE

    Hamlyn, A. N.; Macklon, A F; James, O

    1983-01-01

    Patients with primary biliary cirrhosis (primary non-suppurative destructive cholangitis) in the north east region of England were studied over a five year period and, to evaluate epidemicity, compared with two contemporaneous disease series of known occurrence. These were: terminal renal failure, all causes (low or absent epidemicity n = 106) and an outbreak of echovirus 19 disease (high epidemicity n = 201). Eight primary biliary cirrhosis-affected men and 109 women from an estimated catchm...

  14. SPONTANEOUS DUODENO-BILIARY FISTULA CAUSED BY DUODENAL PEPTIC ULCER

    Directory of Open Access Journals (Sweden)

    N. Danila

    2005-07-01

    Full Text Available Spontaneous duodeno-biliary fistula represents a rare complication of chronic duodenal peptic ulcer. The authors present two cases with this pathology and also the particularities of surgical approach. Spontaneous duodeno-biliary fistula caused by chronic peptic ulcer is often a surprising diagnostic in the era of H2 blockers. The difficulties and the complexity of the diagnosis associated with the particularities of surgical technique represent the key of this rare disease.

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2011-10-01

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

  17. 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.

  18. Biliary excretion of iron and ferritin in idiopathic hemochromatosis

    International Nuclear Information System (INIS)

    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

  19. Interventional treatment on vascular and biliary complications after liver transplantation

    International Nuclear Information System (INIS)

    Objective: To evaluate the value of angiography and cholangiography on the diagnosis and interventional treatment on vascular and biliary complications after liver transplantation. Methods: Sixteen of 46 patients (15 males, 1 female, 17-60 years old) after orthotopic liver transplantation received angiography due to abnormal ultrasonography or edema of lower limbs, or cholangiography due to progressing jaundice. 15 cases received angiography and proved vascular complications and 4 cases received cholangiography and biliary complications were found. 3 of them appeared both vascular and biliary complications. Results: Hepatic artery complication was the most common complication (seen in 9/16 patients), including hepatic artery thrombosis or stenosis (6/9), bleeding (2/9) and hepatic artery-dissecting aneurysm (1/9). One case with hepatic artery thrombosis received transcatheter thrombolysis and two cases with bleeding received coil embolization. Stenosis of inferior vena cava and portal vein were observed in 6 and 2 patients respectively. After balloon angioplasty or stent placement, clinical symptom of all cases alleviated. Biliary complications including biliary stricture and anastomotic bile leak occurred in 4 patients. Jaundice decreased after percutaneous transhepatic cholangiography and drainage. Conclusion: Interventional methods offered both diagnosis and mini-invasive treatment for patients after liver transplantation with vascular and biliary complications. Balloon angiography and stent placement of venous stenosis is an useful procedure for the treatment of these problems

  20. 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.

  1. 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.

  2. 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

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

    OpenAIRE

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

    2013-01-01

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

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

    Science.gov (United States)

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

    2016-03-15

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

  5. 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

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

    Directory of Open Access Journals (Sweden)

    Sathyaprasad C Burjonrappa

    2013-06-01

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

  7. 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.

  8. 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.

  9. 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...

  10. 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.

  11. 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

  12. 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

  13. 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

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

    OpenAIRE

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

    2011-01-01

    Cholangitis arising from biliary infection dominates the prognosis in Caroli's disease. To clarify the influences of bacterial infection on the biliary cystogenesis, in vivo and in vitro studies were performed using the polycystic kidney (PCK) rat as an animal model of Caroli's disease. Cholangitis became a frequent histological finding in aged PCK rats, and neovascularization around the bile ducts also increased in aged PCK rats. Immunohistochemistry revealed that expression of vascular endo...

  15. 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....

  16. 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.

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

    Science.gov (United States)

    Yamasaki, Shuuji

    2016-01-01

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

  18. 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.

  19. 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

  20. 胆道闭锁胆汁湖形成与预后关系研究%Relationship between formation and prognosis of bile lake with biliary atresia

    Institute of Scientific and Technical Information of China (English)

    颜培宏; 刘丹丹; 詹江华; 高伟; 宋亭亭; 尚莹

    2015-01-01

    Objective To explore the relationship between postoperative intrahepatic bile lake formation and clinical disease progression.Methods From January 2012 to December 2013,liver specimens were collected from liver transplantation patients after Kasai portoenterstomy.Based on the presence of bile lake,16 specimens were divided into experimental and control groups (n =8 each).Left lobe,right lobe and porta hepatis of liver were resected respectively for paraffin-embedded sections.Hematoxylin & eosin and CK19 stains were employed for observing the morphological changes of bile lake.And the clinical data were collected for analyzing the effects of bile lake on postoperative disease progression.Results Kasai procedure age (44-86 days) and liver transplantation age (216-1,095 days)in experimental group were lower than those of control group.And liver transplantation age had significant difference (P<0.05).Bile lake often occurred close to porta hepatis in right lobe while the involvement of left lobe was rare.The shapes of bile lake were irregular,polygonal or round.In experimental group,there were 5 polygonal cases with a smaller diameter (0.5-3.0 cm).There was a wall covering of fibrous tissue with visible stones and an infiltration of inflammatory cells.Fibrous tissue abounded around bile lake and bile duct hyperplasia was not obvious; 3 round cases had a large diameter (1.3-5.0 cm).Gallstones were rare and there was a greater infiltration of inflammatory cells heavier.There was a mild hyperplasia of bile duct around lake.In experimental group,4 cases of polygonal bile lake had recurrent cholangitis,persistent jaundice,early formation of bile lake and a rapid deterioration of liver function.The levels of total bilirubin,direct bilirubin andγ-glutamyl transferase were significantly higher than peers.Liver transplantation was performed under 1 year.Conclusions The emergence of bile lake is closely correlated with an elevated risk of cholangitis.Liver function failure has a quick progression,especially polygonal bile lake.And liver transplantation should be performed as soon as possible.%目的 选取接受肝移植胆道闭锁患儿自体肝组织,观察胆汁湖结构,对比患儿临床指标及肝组织病理变化,探讨胆道闭锁患儿Kasai术后肝内胆汁湖形成与临床疾病进展之间的关系.方法 收集2012年1月至2013年12月16例Kasai术后接受肝移植的胆道闭锁患儿的自体肝脏标本,8例存在胆汁湖肝脏标本作为研究组,8例无胆汁湖肝脏标本作为对照组.分别从自体肝组织胆汁湖部位,肝脏左叶、右叶及肝门处取材,蜡块包埋,组织切片,应用HE染色及CK 19免疫组织化学染色方法,观察胆汁湖形态结构改变;同时收集患儿临床资料,分析胆汁湖形成对患儿Kasai术后临床疾病进展的影响.结果 研究组Kasai手术日龄(44~86 d)和肝移植年龄(216~1095 d)均低于对照组,其中肝移植年龄差异有统计学意义(P<0.05).胆汁湖多出现在肝右叶,肝左叶少见,发生于左、右叶的胆汁湖常靠近肝门部位.胆汁湖形状分为不规则或多角形和类圆形,研究组中多角形5例(62.5%),直径较小(0.5~3.0) cm,内壁被覆纤维组织,胆湖内可见结石,并有较多炎细胞浸润,胆湖周围纤维组织增生较重,胆管增生不明显;类圆形3例(37.5%),直径较大(1.3~5.0)cm,胆湖内结石少见,炎细胞浸润较重,胆湖周围胆管轻度增生.研究组中4例多角形胆湖患儿术后胆管炎反复发生,黄疸持续不退,胆汁湖形成早,肝功能恶化快,尤其是TBIL、DBIL和γ-GT升高程度明显高于其他患儿,肝移植时间都在1岁以内.结论 胆汁湖的出现是增加胆管炎发生风险的密切相关因素,出现肝内胆湖情况其肝脏功能衰竭进展较快,尤其是多角形胆汁湖,提示应尽早行肝移植手术.

  1. 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.

  2. Biliary tract obstruction secondary to Burkitt lymphoma

    International Nuclear Information System (INIS)

    The abdomen, in particular the ileocecal region, appendix and colon, is the most common primary site for Burkitt non Hodgkin's lymphoma (NHL). Involvement of the bile duct is rare. The authors describe a patient with abdominal NHL in which jaundice due to bile duct obstruction was the first clinical sign. Case report: a 3 year old white boy presented with one month of progressive jaundice, clay-colored stools, tea colored urine and increase of abdominal volume. Physical examination showed jaundice 3+/4+ and pale mucosa. The abdomen was moderately distended and timpanous and the liver was enlarged. Laboratory examinations confirmed cholestasis with total bilirubin of 8.2 mg/dl (direct bilirubin of 7.8 mg/dl), and microcytic and hypochromic anemia. Ultrasonography (US) and abdominal CT showed two solid tumors in hepatic hilar topography, and dilated intrahepatic biliary tree. The Doppler US showed hepatic artery and portal vein dislocation by the nodules. Comment: although jaundice occurs frequently as a late manifestation of NHL, it is rarely seen as the presenting sign. When jaundice is the first clinical sign and image studies show hepatic hilar tumor and bile duct obstruction, NHL should be considered in the differential diagnosis. (author)

  3. Primary biliary cirrhosis: From bench to bedside

    Institute of Scientific and Technical Information of China (English)

    Elias; Kouroumalis; George; Notas

    2015-01-01

    Primary biliary cirrhosis(PBC) is a chronic non-suppurative destructive intrahepatic cholangitis leading to cirrhosis after a protractive non cirrhotic stage. The etiology and pathogenesis are largely unknown and autoimmne mechanisms have been implicated to explain the pathological lesions. Many epitopes and autoantigens have been reported as crucial in the pathophysiology of the disease and T and B cells abnormalities have been described, the exact pathways leading to the destruction of small intrahepatic ductules are mostly speculative. In this review we examined the various epidemiologal and geoepidemiological data as well as the complex pathogenetic aspects of this disease, focusing on recent in vivo and in vitro studies in this field. Initiation and progression of PBC is believed to be a multifactorial process with strong infuences from the patient’s genetic background and by various environmental factors. The role of innate and adaptive immunity, including cytokines, chemokines, macrophages and the involvement of apoptosis and reactive oxygen species are outlined in detailed. The current pathogenetic aspects are presented and a novel pathogenetic theory unifying the accumulated clinical information with in vitro and in vivo data is formulated. A review of clinical manifestations and immunological and pathological diagnosis was presented. Treatment modalities, including the multiple mechanisms of action of ursodeoxycholate were finally discussed.

  4. 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.

  5. 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. PMID:3980281

  6. Primary cancers of extrahepatic biliary passages

    International Nuclear Information System (INIS)

    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

  7. Successful Endoscopic Ultrasound-Guided Transduodenal Biliary Drainage Through a Pre-Existing Duodenal Stent

    Directory of Open Access Journals (Sweden)

    Paul J Belletrutti

    2010-05-01

    Full Text Available Context When ERCP fails in the setting of combined biliary and duodenal obstruction, EUS-guided biliary drainage has emerged as an alternate method of biliary decompression. Case report We present a case of a 40-year-old man with advanced pancreatic cancer and a pre-existing duodenal wall stent who subsequently develops jaundice due to biliary obstruction. An ERCP was technically unsuccessful as the papilla was inaccessible despite probing within the duodenal stent. Transduodenal biliary drainage was achieved using EUS guidance to create a choledochoduodenostomy tract. A fully covered metal biliary stent was then deployed through the mesh of the duodenal wall stent. The patient’s jaundice and pruritus subsequently resolved. Conclusion This is the first report of successful transduodenal EUS-guided biliary drainage performed through an existing enteral wall stent and can still be considered as an alternate mode of biliary drainage in this setting.

  8. 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)....

  9. 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.

  10. Management of complications during percutaneous implementation of biliary stents

    International Nuclear Information System (INIS)

    Objective: To retrospectively analyze the complications and management of complications during percutaneous implementation of biliary stents in 17 patients. Methods: Percutaneous biliary drainage and implementation of biliary stents for malignant biliary obstruction under fluoroscopic guidance was performed in 64 patients. 17 cases developed complications. Bradycardia and hypotension was seen in 5 cases, slipping of the stent into the duodenum in 1, bile duct perforation in 2, the fine guidewire broken in the hepatic parenchyma in 2, malposition of the stent in 3, hepato-renal failure leading to hemorrhage in 2, exchanging guidewire broken in the duodenum in 1, and balloon broken leading to stent dislocation in 1, respectively. Results: Bradycardia and hypotension in the 5 cases was treated medically and the procedure was interrupted. Slipping of the stent into the duodenum was passed off spontaneously. Broken of the fine guidewire in the liver did not present any complaints until the death of the patients. Perforation of bile duct was treated conservatively with a clinical successful outcome. Malposition of the stent was treated by reimplementation of shorter stents. The broken exchanging guidewire in the duodenum during implementation was removed with the gastroscope. The hepato-renal failure leading to hemorrhage had poor prognosis. The broken balloon caused stent dislocation was treated by reimplementation of stent. Conclusion: Percutaneous implementation of stent is effective and safe for elderly patients with proximal stenosis of the biliary tract. However, one must be very careful to avoid the complications

  11. The clinical application of domestic biliary metallic stents

    International Nuclear Information System (INIS)

    Objective: To determine the clinical efficacy and safety of domestic biliary metallic stents in the management of biliary obstruction. Methods: 75 domestic biliary metallic stents were implanted in 66 of 72 patients with obstructive jaundice due to malignant (n=70) and benign stenoses (n=2). Results: The technical successful rate was 92.7% (64/66). Palliation with rid of jaundice was achieved in 96.9% (64/66) within 1 month. The 30-day mortality rate was 3% (2/66). Early complications occurred in 7.6% (5/66). 44 patients were followed from 2 to 24 months after treatment. 33 (33/42) of 42 patients with malignancy died after a median survival of 5.3 months (range 2-23 months). The remaining 9 patients (9/42) remained alive with a mean follow-up of 11.6 months. The stent patency rates were 92.8%, 89.9% and 79.4 % at 3, 6, 12 months respectively. 6 patients (6/42) presented stent occlusion within a median period of 3 months. One of two patients with benign stenoses sustained a patency duration time of 24 months. One patient died 3 months after treatment. Conclusions: The domestic biliary metallic stent implantation is efficacy and safety in the management of biliary obstruction. (authors)

  12. Resolution of tuberculous biliary stricture after medical therapy

    Institute of Scientific and Technical Information of China (English)

    Khalid E Alsawat; Abdulrahman M Aliebreen

    2006-01-01

    Tuberculosis (TB) is a very rare cause of biliary stricture that is difficult to diagnose and usually requires surgical intervention in order to rule out underlying malignant etiology. We report a 56-year-old man presented with jaundice, weight loss and poor appetite. Initial work up showed the dilated biliary system secondary to distal common bile duct stricture. Investigations to define the etiology of this stricture showed inconclusive brush cytology with absent abdominal masses and lymph nodes but enlarged mediastinal lymph nodes. Biopsy from these lymph nodes showed a non-caseating epitheliod granuloma with negative acid fast bacilli (AFB) stain.The patient had a dramatic response to empirical antituberculosis therapy. Six weeks later, culture from lymph nodes was positive for Mycobacterium tuberculosis.Three months later, follow-up cholangiogram showed complete resolution of the stricture with normalization of liver enzymes 6 mo after starting anti-tuberculosis therapy. Treatment was continued for 12 mo and the patient had a normal life with normal liver enzymes and regression of the mediastinal lymph nodes at the time when he was reported in this paper.Although 16 cases of tuberculous biliary stricture are available in the English literature, up to our knowledge,this is the second published report of tuberculous biliary stricture, which resolved completely after medical therapy alone and the second reported case from the Middle East. This report emphasizes the importance of keeping TB as a possibility of biliary stricture in this part of the world.

  13. 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.

  14. EUS-Guided Choledochoduodenostomy for Biliary Drainage in Unresectable Pancreatic Cancer: A Case Series

    OpenAIRE

    Everson LA Artifon; Jonas Takada; Luciano Okawa; Eduardo GH Moura; Paulo Sakai

    2010-01-01

    Context Endoscopic retrograde cholangiopancreatography (ERCP) is the procedure of choice for biliary decompression in patients with unresectable pancreatic cancer. However, it may be unsuccessful in 3 to 10% of cases. When ERCP is unsuccessful, the usual alternatives are percutaneous transhepatic biliary drainage or surgery. Recently, several authors have reported the use of EUS-guided biliary drainage in patients with malignant biliary obstructions, with acceptable success and complication r...

  15. Predictors for occlusion of the first inserted metallic stent in patients with malignant biliary obstruction

    OpenAIRE

    Wandong Hong; Yunfei Zhu; Yanyan Dong; Yanqing Wu; Mengtao Zhou; Haizhen Ni

    2015-01-01

    Background/Aims: Endoscopic biliary stent drainage plays an important role in the palliative treatment of malignant biliary obstruction. The aim of this study was to investigate predictors of occlusion of first metal inserted stent in patients with malignant biliary obstruction. Patients and Methods: The retrospective analysis was performed in 178 patients with malignant biliary obstruction. Factors associated with stent occlusion were analyzed by Cox regression analysis. Results: Median over...

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

    OpenAIRE

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

    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 covere...

  17. Stenting and interventional radiology for obstructive jaundice in patients with unresectable biliary tract carcinomas

    OpenAIRE

    Tsuyuguchi, Toshio; Takada, Tadahiro; Miyazaki, Masaru; Miyakawa, Shuichi; Tsukada, Kazuhiro; Nagino, Masato; Kondo, Satoshi; Furuse, Junji; Saito, Hiroya; Suyama, Masafumi; Kimura, Fumio; Yoshitomi, Hideyuki; Nozawa, Satoshi; Yoshida, Masahiro; Wada, Keita

    2008-01-01

    Together with biliary drainage, which is an appropriate procedure for unresectable biliary cancer, biliary stent placement is used to improve symptoms associated with jaundice. Owing to investigations comparing percutaneous transhepatic biliary drainage (PTBD), surgical drainage, and endoscopic drainage, many types of stents are now available that can be placed endoscopically. The stents used are classified roughly as plastic stents and metal stents. Compared with plastic stents, metal stents...

  18. 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...

  19. Complications and treatment of migrated biliary endoprostheses: A review of the literature

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Endoscopic biliary stent insertion is a well-established procedure. It is especially successful in treating postoperative biliary leaks, and may prevent surgical intervention. A routine change of endoprostheses after 3 mo is a common practice but this can be prolonged to 6 mo. We reported a colonic perforation due to biliary stent dislocation and migration to the rectosigmoid colon, and reviewed the literature.

  20. Acute Duodenal Obstruction After Percutaneous Placement of Metallic Biliary Stents: Peroral Treatment with Enteral Stents

    International Nuclear Information System (INIS)

    Three patients with malignant biliary obstruction were treated with placement of metallic biliary stents. Two patients had known partial duodenal stenosis but had no symptoms of gastrointestinal obstruction. The patients developed symptomatic duodenal obstruction early after biliary metallic stent placement. The symptomatic duodenal obstructions were successfully treated with peroral placement of duodenal stents, which obviated the need for surgical intervention

  1. 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.

  2. The application of biliary catheter folding technique in the treatment of hilar nonanastomotic biliary strictures after orthotopic liver transplantation

    International Nuclear Information System (INIS)

    Objective: To introduce a newly-designed percutaneous single catheter folding technique, by which bilateral biliary drainage and stenting can be completed through single access, and to assess the effectiveness and safety of this technique in treating hilar nonanastomotic strictures occurred after orthotopic liver transplantation. Methods: A total of 10 patients with nonanastomotic strictures, who were encountered during the period from July 2000 to July 2010 in authors' hospital, were enrolled in this study. Balloon dilatation was used for the biliary tract stenosis. After the placement of biliary drainage tube, the catheter was folded into 'Y' shape within the biliary duct at hepatic portal region, and triaxial supporting drainage, i.e. the left hepatic duct, the right hepatic duct and the common hepatic duct, was established. The technical success rate, the clinical efficacy, the complications and the recurrence were documented and analyzed. Results: Technical success rate was 100% (10/10). In 9 patients, the clinical symptoms were obviously relieved, the biochemical indexes were gradually restored to normal and the imaging findings were markedly improved. During the follow-up lasting 26 months (median), no recurrence was seen. Minor complications occurred in two cases. One patient died after he received second orthotopic liver transplantation because of failure to respond to initial treatment. Conclusion: Percutaneous transhepatic biliary catheter folding technique is technically feasible. The results of this study indicate that this technique carries satisfactory success rate and is very effective and safe for the treatment of hilar nonanastomotic strictures occurred after orthotopic liver transplantation. (authors)

  3. 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.

  4. 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.

  5. 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.

  6. Biliary Ischemia Following Embolization of a Pseudoaneurysm after Pancreaticoduodenectomy

    Directory of Open Access Journals (Sweden)

    Roger Noun

    2006-07-01

    Full Text Available Aim :To report an uncommon consequence of hepatic artery occlusion in the management of a bleeding pseudoaneurysm following pancreaticoduodenectomy. Imaging :Analysis of a case involving a single patient in which a bleeding pseudoaneurysm of the gastroduodenal arterial stump following pancreaticoduodenectomy was treated by transcatheter arterial embolization. Case report: Effective hemostasis necessitated interruption of the hepatic arterial flow and was complicated by biliary ischemia and intrahepatic biloma. Conclusion :Transarterial embolization of the hepatic artery following ancreaticoduodenectomy can result in biliary ischemia and biloma formation.

  7. Imaging tests for accurate diagnosis of acute biliary pancreatitis

    DEFF Research Database (Denmark)

    Surlin, Valeriu; Săftoiu, Adrian; Dumitrescu, Daniela

    2014-01-01

    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...... 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...

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

    Science.gov (United States)

    Kwon, Chang-Il; Lehman, Glen A

    2016-03-01

    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

  9. 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.

  10. 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.

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

    International Nuclear Information System (INIS)

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

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

    Directory of Open Access Journals (Sweden)

    Money Gupta

    2011-01-01

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

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

    OpenAIRE

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

    2012-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Rajiv Chadha

    2013-01-01

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

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

    OpenAIRE

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

    2012-01-01

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

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

    Science.gov (United States)

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

    2016-09-01

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

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

    Science.gov (United States)

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

    2013-04-01

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

  18. Interventional treatment of biliary stent restenosis: recent progress in clinical management

    International Nuclear Information System (INIS)

    Malignant obstructive jaundice is biliary obstruction disorders which are caused by various malignant tumors. Usually the disease is at its advanced stage and is inoperable when the diagnosis is confirmed. At present, percutaneous transhepatic biliary drainage (PTCD) and endoscopic or interventional implantation of plastic or self-expanding metal stent (SEMS) are the main managements in clinical practice. Due to the improved survival time, biliary stent restenosis has become a quite common clinical problem. Photodynamic therapy (PDT) and biliary tract radiofrequency ablation (RFA) have provided new therapeutic means for clinical use. Especially, with its development in technology and equipment, RFA has played more and more important role in treating biliary stent restenosis. (authors)

  19. Hepatobiliary scintigraphy in the assessment of long-term complication after biliary-enteric anastomosis: role in the diagnosis of post-operative segmental or total biliary obstruction

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jae Seung; Moon, Dae Hyuk; Lee, Sung Gyu; Lee, Yung Joo; Park, Kwang Min; Shin, Jung Woo; Ryu, Jin Sook; Lee, Hee Kyung [Asan Medicial Center, Seoul (Korea, Republic of)

    1998-07-01

    The purpose of this study was to investigate the accuracy of hepatobiliary scintigraphy (HBS) in the diagnosis of segmental or total biliary obstruction during long-term follow-up period after curative radical surgery with biliary-enteric anastomosis. The study population included 80 patients who underwent biliary-enteric anastomoses for benign (n=33) or malignant (n=47) biliary disease. Fifty-six of these 80 patients also underwent curative hepatic resection. Ninety eight hepatobiliary scintigrams using {sup 99m}Tc-DISIDA were performed at least 1 month after surgery (median 9 month). The scintigraphic criteria of total biliary obstruction we used were intestinal excretion beyond one hour or delayed hepatobiliary washout despite the presence of intestinal excretion. Segmental biliary obstruction was defined as delayed segmental excretion. The accuracy for biliary obstruction was evaluated according to different clinical situations. There were 9 instances with total biliary obstruction and 23 with segmental bile duct obstruction. Diagnosis of biliary obstruction was confirmed by percutaneous transhepatic cholangiography or surgery in 13, and follow-up clinical data for at least 6 months in 19 instances. Among the 32 instances with biliary symptoms and abnormal liver function, HBS allowed correct diagnosis in all 32(9 total, 14 segmental obstruction and 9 non-obstruction). Of the 40 with nonspecific symptom or isolated elevation of serum alkaline phosphatase, HBS diagnosed 8 of the 9 segmental biliary obstruction and 30 of the 31 non-obstruction. There were no biliary obstruction and no false positive result of scintigraphy in 26 instances which had no clinical symptom or signs of biliary obstruction. Diagnostic sensitivity of HBS was 100% (9/9) for total biliary obstruction, and 96%(22/23) for segmental bile obstruction. Specificity was 98%(39/40) in patients who had abnormal symptom or sign. Hepatobiliary scintigraphy is a highly accurate modality in the

  20. The observation and nursing of patients receiving interventional management for biliary complications occurred after liver transplantation

    International Nuclear Information System (INIS)

    Objective: To discuss the perioperative nursing norm for patients who are suffering from biliary complications occurred after liver transplantation and who will receive interventional management to treat the complications. Methods: Interventional therapies were performed in 20 patients with biliary complications due to liver transplantation. The interventional procedures performed in 20 cases included percutaneous biliary drainage (n = 13), percutaneous biliary balloon dilatation (n = 5) and biliary stent implantation (n = 7). The clinical results were observed and analyzed. Results: Biliary tract complications occurred after liver transplantation were seen frequently. Proper interventional management could markedly improve the successful rate of liver transplantation and increase the survival rate of the patients. In accordance with the individual condition, proper nursing measures should be taken promptly and effectively. Conclusion: Conscientious and effective nursing can contribute to the early detection of biliary complications and, therefore, to improve the survival rate of both the transplanted liver and the patients. (authors)

  1. 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.

  2. Percutaneous transhepatic biliary drainage for hilar cholangiocarcinoma

    International Nuclear Information System (INIS)

    Objective: To evaluate the effect of PTBD in treating malignant biliary obstruction caused by hilar cholangiocarcinoma. Methods: We retrospectively analyzed the data of 103 patients(M:62,F:41)with malignant obstructive jaundice caused by hilar cholangiocarcinoma. After taking percutaneous transhepatic cholangiography, metallic stent or plastic external catheter or external-internal catheter for drainage was deployed and then followed up was undertaken with clinical and radiographic evaluation and laboratory. examination. Results: All patients went though PTBD successfully (100%). According to Bismuth classification, all 103 cases consisted of I type(N=30), II type (N=30), III type (N=26) and IV type (N=17). Thirty-nine cases were placed with 47 stents and 64 eases with drainage tubes. 4 cases installed two stems for bilateral drainage, 2 cases installed two stents because of long segmental strictures with stent in stent, 1 case was placed with three stents, and 3 cases installed stent and plastic catheter together. Sixty-four cases received plastic catheters in this series, 35 cases installed two or more catheters for bilateral drainage, 28 cases installed external and internal drainage catheters, 12 eases installed external drainage catheters, and 24 eases installed both of them. There were 17 patients involving incorporative infection before procedure, 13 cases cured after procedure, and 15 new patients got inflammation after procedure. 13 cases showed increase of amylase (from May, 2004), 8 eases had bloody bile drainage and 1 case with pyloric obstruction. Total serum bilirubin reduced from (386 ± 162) μmol/L to (161 ± 117) μmol/L, (P<0.01) short term curative effect was related with the type of hilar cholangiocarcinoma. The survival time was 186 days(median), and 1, 3, 6, 12 month survival rate were 89.9%, 75.3%, 59.6%, 16.9%, respectively. Conclusion: Percutaneous transhepatic bile drainage is a safe and effective palliative therapy of malignant

  3. 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.

  4. 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.

  5. A novel biliary stent coated with silver nanoparticles prolongs the unobstructed period and survival via anti-bacterial activity

    OpenAIRE

    Fuchun Yang; Zhigang Ren; Qinming Chai; Guangying Cui; Li Jiang; Hanjian Chen; Zhiying Feng; Xinhua Chen; Jian Ji; Lin Zhou; Weilin Wang; Shusen Zheng

    2016-01-01

    Symptomatic biliary stricture causes life-threatening complications, such as jaundice, recurrent cholangitis and secondary biliary cirrhosis. Fully covered self-expanding metal stents (FCSEMSs) are gaining acceptance for treatments of benign biliary stricture and palliative management of malignant biliary obstructions. However, the high rate of FCSEMS obstruction limits their clinic use. In this study, we developed a novel biliary stent coated with silver nanoparticles (AgNPs) and investigate...

  6. 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.

  7. 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.

  8. Anastomotic biliary strictures after liver transplantation : Causes and consequences

    NARCIS (Netherlands)

    Verdonk, RC; Buis, CI; Porte, RJ; Van der Jagt, EJ; Limburg, AJ; Van den Berg, AP; Slooff, MJH; Peeters, PMJG; de Jong, KP; Kleibeuker, JH; Haagsma, EB

    2006-01-01

    We retrospectively studied the prevalence, presentation, results of treatment, and graft and patient survival of grafts developing an anastomotic biliary stricture (AS) in 531 adult liver transplantations performed between 1979 and 2003. Clinical and laboratory information was obtained from the hosp

  9. A sign of symptomatic chronic cholecystitis on biliary scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Al-Sheikh, W.; Hourani, M.; Barkin, J.S.; Clarke, L.P.; Ashkar, F.S.; Serafini, A.N.

    1983-02-01

    Five hundred patients with acute right-upper-quadrant pain underwent biliary scintigraphy with /sup 99m/Tc paraisopropyliminodiacetic acid. One hundred and thirty-four studies were reported normal (both gallbladder and activity in bowel are noted in 1 hr). Of the 134 studies reported as normal, 32 showed intestinal activity before gallbladder visualization during the first hour of the study. Sonography and/or oral cholecystography revealed that 24 patients had gallstones, and eight patients had no demonstrable pathology in the biliary system. Of the 134 studies, 102 showed visualization of the gallbladder before intestinal activity during the first hour of the study. Sonography and/or oral cholecystography showed that 73 patients had normal biliary system. The remaining 29 patients had gallstones. The overall sensitivity of this finding is 45%, the specificity is 90%, and the accuracy is 73%. In this group of symptomatic patients, the appearance of intestinal activity before gallbladder activity on biliary scintigraphy warrants further evaluation of these patients by sonography and/or oral cholecystography.

  10. A sign of symptomatic chronic cholecystitis on biliary scintigraphy

    International Nuclear Information System (INIS)

    Five hundred patients with acute right-upper-quadrant pain underwent biliary scintigraphy with /sup 99m/Tc paraisopropyliminodiacetic acid. One hundred and thirty-four studies were reported normal (both gallbladder and activity in bowel are noted in 1 hr). Of the 134 studies reported as normal, 32 showed intestinal activity before gallbladder visualization during the first hour of the study. Sonography and/or oral cholecystography revealed that 24 patients had gallstones, and eight patients had no demonstrable pathology in the biliary system. Of the 134 studies, 102 showed visualization of the gallbladder before intestinal activity during the first hour of the study. Sonography and/or oral cholecystography showed that 73 patients had normal biliary system. The remaining 29 patients had gallstones. The overall sensitivity of this finding is 45%, the specificity is 90%, and the accuracy is 73%. In this group of symptomatic patients, the appearance of intestinal activity before gallbladder activity on biliary scintigraphy warrants further evaluation of these patients by sonography and/or oral cholecystography

  11. Percutaneous transhepatic biliary drainage: analysis of 175 cases

    International Nuclear Information System (INIS)

    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

  12. A Different Method of Hepaticojejunostomy for Proximal Biliary Injuries

    Directory of Open Access Journals (Sweden)

    Diane M. Radford

    1996-01-01

    Full Text Available The management of proximal biliary injuries presents a surgical challenge. Anastomoses can be difficult to perform and can have poor results. We describe a method of hepaticojejunostomy done from within the Roux-en-Y loop, which can be utilized in this situation.

  13. Citation bias of hepato-biliary randomized clinical trials

    DEFF Research Database (Denmark)

    Kjaergard, Lise L; Gluud, Christian

    2002-01-01

    The objective of this study was to assess whether trials with a positive (i.e., statistically significant) outcome are cited more often than negative trials. We reviewed 530 randomized clinical trials on hepato-biliary diseases published in 11 English-language journals indexed in MEDLINE from 1985...

  14. Management of patients after recovering from acute severe biliary pancreatitis.

    Science.gov (United States)

    Dedemadi, Georgia; Nikolopoulos, Manolis; Kalaitzopoulos, Ioannis; Sgourakis, George

    2016-09-14

    Cholelithiasis is the most common cause of acute pancreatitis, accounting 35%-60% of cases. Around 15%-20% of patients suffer a severe attack with high morbidity and mortality rates. As far as treatment is concerned, the optimum method of late management of patients with severe acute biliary pancreatitis is still contentious and the main question is over the correct timing of every intervention. Patients after recovering from an acute episode of severe biliary pancreatitis can be offered alternative options in their management, including cholecystectomy, endoscopic retrograde cholangiopancreatography (ERCP) and sphincterotomy, or no definitive treatment. Delaying cholecystectomy until after resolution of the inflammatory process, usually not earlier than 6 wk after onset of acute pancreatitis, seems to be a safe policy. ERCP and sphincterotomy on index admission prevent recurrent episodes of pancreatitis until cholecystectomy is performed, but if used for definitive treatment, they can be a valuable tool for patients unfit for surgery. Some patients who survive severe biliary pancreatitis may develop pseudocysts or walled-off necrosis. Management of pseudocysts with minimally invasive techniques, if not therapeutic, can be used as a bridge to definitive operative treatment, which includes delayed cholecystectomy and concurrent pseudocyst drainage in some patients. A management algorithm has been developed for patients surviving severe biliary pancreatitis according to the currently published data in the literature. PMID:27678352

  15. 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.

  16. 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

  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. 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

  19. 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

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

    Science.gov (United States)

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

    2016-07-01

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