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Sample records for fungal cholangitis complicating

  1. 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...

  2. [Acute cholangitis secondary to ascariasis and complicated by liver abscesses].

    Science.gov (United States)

    Rakotonaivo, A; Ranoharison, H D; Razarimahefa, S H; Rakotozafindrabe, R; Rabenjanahary, T H; Ramanampamonjy, R M

    2015-01-01

    Acute cholangitis secondary to ascariasis is rare and occurs mainly in areas of high endemicity. The clinical presentation is non-specific, sometimes complicated by liver abscess. Abdominal ultrasound plays an important role in diagnosis and therapeutic surveillance. We report the case of a 35-year-old Malagasy woman with an acute cholangitis secondary to ascariasis and complicated by liver abscesses and its course to full recovery under medical treatment.

  3. Cholangitis

    Science.gov (United States)

    ... duct is blocked by something, such as a gallstone or tumor. The infection causing this condition may ... liver. Risk factors include a previous history of gallstones , sclerosing cholangitis, HIV, narrowing of the common bile ...

  4. Hepatic microabscess with ascending cholangitis complicated by endoscopic retrograde cholangiopancreatogram (ERCP): A case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Mi Young; Auh, Yong Ho; Lee, Moon Gyu [Asan Medical Center, College of Medicine, University of Ulasn, Seoul (Korea, Republic of)

    1994-07-15

    Complicated hepatic microabscess secondary to ascending cholangitis following ERCP (Endoscopic retrograde cholangiopancreatogram) is rare, and needs to be differentiated from other microabscesses, metastasis or Caroli's disease. We experienced a case of hepatic microabscess associated with septic cholangitis following ERCP. Cholangiogram showed multiple sac-like abscess pockets with characteristic biliary communication, and CT scan revealed multiple low attenuated lesions. At the resolving stage of cholangitic microabscess, CT scan showed partial rim enhancement of the abscesses and disproportional dilatation of intrahepatic ducts. The residual parenchymal enhancement surrounding the resolved microabscess pockets and dilatated biliary ducts, however, remained even after clinical recovery.

  5. Sclerosing cholangitis

    Science.gov (United States)

    ... lining of the abdomen and abdominal organs) and varices (enlarged veins) Biliary cirrhosis (inflammation of the bile ... Complications may include: Bleeding esophageal varices Cancer in the ... of the biliary system (cholangitis) Narrowing of the bile ducts ...

  6. Acute pancreatitis and cholangitis: A complication caused by a migrated gastrostomy tube

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Percutaneous endoscopic gastrostomy (PEG) is generally considered safe with a low rate of serious complications. However, dislocation of the PEG-tube into the duodenum can lead to serious complications.An 86-year old Japanese woman with PEG-tube feeding sometimes vomited after her family doctor replaced the PEG-tube without radiologic confirmation. At her hospitalization, she complained of severe tenderness at the epigastric region and the PEG-tube was drawn into the stomach. Imaging studies showed that the tip of PEG-tube with the inflated balloon was migrated into the second portion of the duodenum, suggesting that it might have obstructed the bile and pancreatic ducts,inducing cholangitis and pancreatitis. After the PEG-tube was replaced at the appropriate position, vomiting and abdominal tenderness improved dramatically and laboratory studies became normal immediately. Our case suggests that it is important to secure PEG-tube at the level of skin, especially after replacement.

  7. Life-threatening hemobilia caused by hepatic artery pseudoaneurysm:A rare complication of chronic cholangitis

    Institute of Scientific and Technical Information of China (English)

    Tsu-Te Liu; Ming-Chih Hou; Han-Chieh Lin; Full-Young Chang; Shou-Dong Lee

    2003-01-01

    Hemobilia is one of the causes of obscure gastrointestinal haemorrhage. Most cases of hemobilia are of iatrogenic or traumatic origin. Hemobilia caused by a hepatic artery pseudoaneurysm due to ascending cholangitis is very rare and its mechanism is undear. We report a 74-year-old woman with a history of surgery for choledocholithiasis 30 years ago, suffering from a protracted course of life-threatening gastrointestinal bleeding. A small intestines series and endoscopic retrograde cholangiopancreatography revealed a chronic cholangitis with marked contrast reflux into the biliary tree. Angiography confirmed the bleeding from a pseudoaneurysm of the middle hepatic artery. Coil embolization achieved successful hemostasis. We discussed the mechanism and reviewed the literature.

  8. Clinical features and prognosis of patients with primary biliary cholangitis complicated by hepatitis virus infection

    Directory of Open Access Journals (Sweden)

    ZHAO Dantong

    2017-08-01

    Full Text Available ObjectiveTo investigate the clinical features and prognosis of patients with primary biliary cholangitis(PBC complicated by hepatitis virus infection. MethodsA total of 16 patients who were admitted to Beijing YouAn Hospital from October 2004 to October 2012 and diagnosed with PBC complicated by hepatitis virus infection were enrolled, among whom 7 had chronic hepatitis B virus infection, 3 had hepatitis C, 4 had hepatitis E, 1 had hepatitis B and hepatitis C, and 1 had hepatitis A. A total of 76 hospitalized patients with PBC alone were enrolled as controls. The two groups were compared in terms of clinical features, laboratory markers, and autoantibodies, and follow-up visits were performed to investigate prognostic features. The independent samples t-test was used for comparison of normally distributed continuous data, and the Mann-Whitney U rank sum test was used for comparison of non-normally distributed continuous data; the chi-square test or Fisher′s exact test was used for comparison of categorical data. The Kaplan-Meier method was used to calculate survival rates and the log-rank test was used to compare survival rates between groups. ResultsCompared with the control group, the PBC-hepatitis virus infection group had significantly lower proportion of female patients (χ2=12.22, P=0.002, alkaline phosphatase (U=225.00, P<0.001, CHO (U=363.50, P=0.036, and IgG level (t=2.79, P=0.007, and no patients in the PBC-hepatitis virus infection group experienced abdominal wall varices, upper gastrointestinal bleeding, or hepatic encephalopathy. The PBC-hepatitis virus infection group had various autoantibodies including anti-nuclear antibody, smooth muscle antibody, anti-parietal cell antibody (APCA, anti-liver specific protein antibody, and anti-myocardial antibody, as well as a significantly higher APCA positive rate than the control group (25% vs 3.9%, χ2=5.608, P=0.016. The median follow-up time was 49.5 months (2-312 months. The PBC

  9. Sinonasal Fungal Infections and Complications: A Pictorial Review

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    Jose Gavito-Higuera

    2016-01-01

    Full Text Available Fungal infections of the nose and paranasal sinuses can be categorized into invasive and non-invasive forms. The clinical presentation and course of the disease is primarily determined by the immune status of the host and can range from harmless or subtle presentations to life threatening complications. Invasive fungal infections are categorized into acute, chronic or chronic granulomatous entities. Immunocompromised patients with poorly controlled diabetes mellitus, HIV and patients receiving chemotherapy or chronic oral corticosteroids are mostly affected. Mycetoma and Allergic Fungal Rhinosinusitis are considered non-invasive forms. Computer tomography is the gold-standard in sinonasal imaging and is complimented by Magnetic resonance imaging (MRI as it is superior in the evaluation of intraorbital and intracranial extensions. The knowledge and identification of the characteristic imaging patterns in invasive - and non- invasive fungal rhinosinusitis is crucial and the radiologist plays an important role in refining the diagnosis to prevent a possible fatal outcome.

  10. A Rare Devastating Complication of Lasik: Bilateral Fungal Keratitis

    Directory of Open Access Journals (Sweden)

    H. Taylan Sekeroglu

    2010-01-01

    Full Text Available Purpose. To report an unusual case of severe bilateral fungal keratitis following laser in situ keratomileusis (LASIK. Method. A 48-year-old man developed bilateral diffuse corneal infiltration two weeks after LASIK. The corneal scrapings revealed fungal filaments but cultures were negative. Results. The corneal ulceration was improved on the left eye whereas spontaneous perforation occurred and finally evisceration was needed on the right eye despite topical and systemic antifungal treatment. Conclusions. Fungal keratitis, especially with bilateral involvement, is a very rare and serious complication of LASIK surgery. Clinical suspicion is crucial because most of fungal keratitis are misdiagnosed as bacterial keratitis and can lead serious visual results, even eye loss.

  11. Spontaneous fungal peritonitis: a devastating complication of cirrhosis.

    Science.gov (United States)

    Bremmer, Derek N; Garavaglia, Jeffrey M; Shields, Ryan K

    2015-07-01

    Spontaneous bacterial peritonitis is a well-known complication of cirrhosis; however, spontaneous fungal peritonitis (SFP) is less well-recognised and described. Our objective was to determine the clinical characteristics, treatment outcomes and factors associated with death among patients with SFP. We performed a retrospective cohort study using the primary outcome of all-cause mortality at 28 days. Twenty-five patients were included; Candida species were the causative pathogen in all cases. At the onset of SFP, patients were critically ill, median APACHE II and MELD scores were 22 and 30.3, respectively. The 28-day mortality rate was 56%; six patients died prior to culture positivity. Among the remaining patients, there were no differences in rates of death by treatment regimen (P = 0.55). APACHE II score at the onset of SFP was an independent predictor of death (OR = 1.46, 95% CI = 1.02-2.08, P = 0.04). In conclusion, SFP develops among critically ill patients with cirrhosis and is associated with high rates of death. Directed antifungal therapy did not improve patient outcomes. Future studies assessing the benefit of early or pre-emptive antifungal therapy are warranted. © 2015 Blackwell Verlag GmbH.

  12. Clinicomicrobiological analysis of patients with cholangitis

    Directory of Open Access Journals (Sweden)

    S M Shenoy

    2014-01-01

    Full Text Available Acute cholangitis is inflammation of biliary ductal system from infection with an associated biliary obstruction. This retrospective study was done to determine the factors responsible for cholangitis and the microbiological profile of the bile in patients with cholangitis. In the study involving 348 patients, 36.4% had associated malignancy. A total of 54% of the bile samples were positive for aerobic culture. Nearly 66-73% of the Escherichia coli and Klebsiella isolates were Extended spectrum beta lactamases (ESBL producers. Two isolates of Candida spps were also obtained. Polymicrobial infection was seen in 31.5% of the culture positive cases. Ideal antibiotics in case of cholangitis would be those which are excreted in the bile such as third-generation cephalosporins, ureidopenicillins, carbapenems and fluoroquinolones to combat resistance and polymicrobial aetiology. Anti-fungal drugs may also be necessary if the patient is not responding to biliary decompression and antibacterial agents to prevent fungaemia.

  13. Primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    Marina G Silveira

    2008-01-01

    Full Text Available Primary sclerosing cholangitis (PSC is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in end-stage liver disease and reduced life expectancy. PSC primarily affects young and middle-aged men, often in association with underlying inflammatory bowel disease. The etiology of PSC includes immune-mediated components and elements of undefined nature. A cholestatic picture of liver biochemistries with elevations in serum alkaline phosphatase, nonspecific autoantibodies such as perinuclear antineutrophilic antibody, antinuclear antibodies and smooth muscle antibodies, and diffuse multifocal biliary strictures, resulting in a ‘beaded’ appearance on radiographic studies, are the hallmarks of the disease. No effective medical therapy is currently available, although clinical studies are in progress. Ursodeoxycholic acid at high doses (28 mg/kg/day to 30 mg/kg/day is the most promising agent but is unproven so far. Liver transplantation is currently the only life-extending therapy for patients with end-stage disease, although recurrent disease can be observed in the transplanted liver. The multiple complications of PSC include pruritus, fatigue, vitamin deficiencies, metabolic bone disease, peristomal varices, bacterial cholangitis, dominant biliary strictures, gallbladder stones and polyps, and malignancy, particularly cholangiocarcinoma, which is the most lethal complication of PSC.

  14. Riskfactorsandincidenceofacutepyogenic cholangitis

    Institute of Scientific and Technical Information of China (English)

    Yun-Sheng Qin; Qi-Yong Li; Fu-Chun Yang and Shu-Sen Zheng

    2012-01-01

    BACKGROUND: Acute cholangitis varies from mild to severe form. Acute suppurative cholangitis (ASC), the severe form of acute cholangitis, is a fatal disease and requires urgent biliary decompression. Which patients are at a high risk of ASC and need emergency drainage is still unclear. The present study aimed to identify the factors for determining early-stage ASC and distinguishing ASC from acute cholangitis. METHODS:  We analyzed 359 consecutive patients with acute cholangitis  who  had  been  admitted  to  the  First  Afifliated Hospital,  Zhejiang  University  School  of  Medicine  from January 2004 to May 2011. Emergency endoscopic retrograde cholangiopancreatography (ERCP) was carried out in all patients to decompress or clear the stones by experienced endoscopists. Clinical and therapeutic data were collected, and univariate and multivariate analyses were performed to identify the potential risk factors of ASC. RESULTS: Of the 359 patients, 1 was excluded because of failure of ERCP drainage. Of the remaining 358 patients with an average age of 62.7 years (range 17-90), 162 were diagnosed with ASC, and 196 with non-ASC. ENBD catheters were placed in 343 patients (95.8%), of whom 182 patients had stones removed at the same time, and plastic stent was placed in 25 patients (7.0%). Clinical conditions were improved quickly after emergency biliary drainage in all patients. Complications were identiifed in 11 patients (3.1%): mild pancreatitis occurred in 8 patients and hemorrhage in 3 patients. There was no

  15. Duct-to-duct reconstruction in liver transplantation for primary sclerosing cholangitis is associated with fewer biliary complications in comparison with hepaticojejunostomy

    NARCIS (Netherlands)

    Sutton, Michael E.; Bense, Rico D.; Lisman, Ton; van der Jagt, Eric J.; van den Berg, Aad P.; Porte, Robert J.

    2014-01-01

    There is no consensus on the preferred type of biliary reconstruction for patients undergoing orthotopic liver transplantation (OLT) for primary sclerosing cholangitis (PSC). The aim of this study was to compare long-term outcomes after OLT for PSC using either duct-to-duct anastomosis or Roux-en-Y

  16. Invasive Fungal Rhinosinusitis versus Bacterial Rhinosinusitis with Orbital Complications: A Case-Control Study

    OpenAIRE

    2013-01-01

    Background. Invasive fungal rhinosinusitis with orbital complications (IFSwOC) is a life-threatening condition. The incidence of mortality has been reported to be up to 80 percent. This study was conducted to determine the risk factors, presentations, clinical, and imaging findings that could help to manage this condition promptly. Methods. We conducted a case-control study of 100 patients suffering from rhinosinusitis with orbital complications. The risk factors, clinical presentations, radi...

  17. Minimal trephination penetrating keratoplasty for severe fungal keratitis complicated with hypopyon.

    Science.gov (United States)

    Liu, Yang; Jia, Hui; Shi, Xiaoru; Wang, Jiao; Ning, Yan; He, Bing; Wang, Chunmei; Zheng, Xiaodong

    2013-12-01

    To report outcomes after minimal trephination penetrating keratoplasty (PKP) in the treatment of severe fungal keratitis complicated with hypopyon. Retrospective case series. Series of 19 eyes in 19 patients with severe fungal keratitis complicated with hypopyon that received minimal trephination PKP. The host trephination was made equal to or smaller than the margin of the corneal lesion. Fluconazole (0.2%) was used to irrigate the trephined edge and anterior chamber during surgery, followed by irrigation of the anterior chamber with a 0.02% fluconazole solution after graft transplantation. Postoperative complications, graft rejection, transparency rate, and visual acuity were recorded. Patients were followed postoperatively for 18 to 34 months (mean 28.6 months). At 18 months after PKP, 18 grafts (94.7%) remained clear and 14 eyes (73.7%) had improved visual acuity. Three eyes (15.8%) with secondary glaucoma complications after PKP were treated with subsequent trabeculectomy. Recurrent infection was found in only 1 eye (5.26%) after transplantation and was successfully managed. Immune graft rejections were not observed in any patient during the follow-up period. The minimal trephination technique in combination with antifungal therapy was effective in the treatment of severe fungal keratitis with large corneal lesions and hypopyon. Copyright © 2013 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.

  18. Invasive Fungal Rhinosinusitis versus Bacterial Rhinosinusitis with Orbital Complications: A Case-Control Study

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    Patorn Piromchai

    2013-01-01

    Full Text Available Background. Invasive fungal rhinosinusitis with orbital complications (IFSwOC is a life-threatening condition. The incidence of mortality has been reported to be up to 80 percent. This study was conducted to determine the risk factors, presentations, clinical, and imaging findings that could help to manage this condition promptly. Methods. We conducted a case-control study of 100 patients suffering from rhinosinusitis with orbital complications. The risk factors, clinical presentations, radiological findings, medical and surgical managements, durations of hospital stay, and mortality rate data were collected. Results. Sixty-five patients were diagnosed with IFSwOC, while the other thirty-five patients composed the control group. The most important risk factor for IFSwOC was diabetes mellitus. Visual loss and diplopia were the significant symptom predictors. The significant clinical predictors were nasal crust, oculomotor nerve, and optic nerve involvement. The CT findings of IFSwOC were sinus wall erosion and hyperdensity lesions. The mortality rate was 25.71 percent in the IFSwOC group and 3.17 percent in the control group. Conclusions. Invasive fungal rhinosinusitis with orbital complications is symptomatic of a high mortality rate. The awareness of a patient’s risk factors, the presenting symptoms, signs of fungal invasion, and aggressive management will determine the success of any treatment procedures.

  19. Clinical features and management of primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Marina G Silveira; Keith D Lindor

    2008-01-01

    Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts,resulting in cirrhosis and need for liver transplantation and reduced life expectancy.The majority of cases occur in young and middle-aged men,often in association with inflammatory bowel disease.The etiology of primary sclerosing cholangitis includes immune-mediated components and elements of undefined nature.No effective medical therapy has been identified.The multiple complications of primary sclerosing cholangitis include metabolic bone disease,dominant strictures,bacterial cholangitis,and malignancy,particularly cholangiocarcinoma,which is the most lethal complication of primary sclerosing cholangitis.Liver transplantation is currently the only life-extending therapeutic alternative for patients with end-stage disease,although recurrence in the allografted liver has been described.A PSC-like variant attracting attention is cholangitis marked by raised levels of the immunoglobulin G4 subclass,prominence of plasma cells within the lesions,and steroid responsiveness.

  20. Ampullary duodenal diverticulum and cholangitis

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    Joaquim Mendes Castilho Netto

    Full Text Available CONTEXT: Ampullary duodenal diverticulum complicated by cholangitis is little known in clinical practice, especially when there are no gallstones in the common bile duct or there is no biliary tree ectasia or hyperamylasemia. A case of this association is presented, in which the surgical treatment was a biliary-enteric bypass. CASE REPORT: A 74-year-old diabetic white woman was admitted to the Taubaté University Hospital, complaining of pain in the right upper quadrant, jaundice and fever with chills (Charcot's triad. She had had cholecystectomy 30 years earlier. She underwent clinical treatment with parenteral hydration, insulin, antibiotics and symptomatic drugs. Imaging examinations were provided for diagnosis: ultrasound, computed tomography and endoscopic retrograde cholangiopancreatography. The surgical treatment consisted of choledochojejunostomy utilizing a Roux-en-y loop. The postoperative period progressed without incidents, and a DISIDA scan demonstrated the presence of dynamic biliary excretion. The patient remained asymptomatic when seen at outpatient follow-up.

  1. [Syndrome overlap: autoimmune hepatitis and autoimmune cholangitis].

    Science.gov (United States)

    Guerra Montero, Luis; Ortega Alvarez, Félix; Marquez Teves, Maguin; Asato Higa, Carmen; Sumire Umeres, Julia

    2016-01-01

    Autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis and autoimmune cholangitis are chronic autoimmune liver disease, usually present separate, the cases where characteristics of two of the above is observed liver disease is commonly referred to as Overlap Syndromes (OS). Although there is no consensus on specific criteria for the diagnosis of OS identification of this association is important for initiating appropriate treatment and prevent its progression to cirrhosis or at least the complications of cirrhosis and death. We report the case of awoman aged 22 cirrhotic which debuted are edematous ascites, severe asthenia and jaundice compliant diagnostics SS criteria and initially present any response to treatment with ursodeoxycholic acid and oral corticosteroids, but ultimately finished performing a transplant orthotopic liver.

  2. TG13 miscellaneous etiology of cholangitis and cholecystitis.

    Science.gov (United States)

    Higuchi, Ryota; Takada, Tadahiro; Strasberg, Steven M; Pitt, Henry A; Gouma, Dirk J; Garden, O James; Büchler, Markus W; Windsor, John A; Mayumi, Toshihiko; Yoshida, Masahiro; Miura, Fumihiko; Kimura, Yasutoshi; Okamoto, Kohji; Gabata, Toshifumi; Hata, Jiro; Gomi, Harumi; Supe, Avinash N; Jagannath, Palepu; Singh, Harijt; Kim, Myung-Hwan; Hilvano, Serafin C; Ker, Chen-Guo; Kim, Sun-Whe

    2013-01-01

    This paper describes typical diseases and morbidities classified in the category of miscellaneous etiology of cholangitis and cholecystitis. The paper also comments on the evidence presented in the Tokyo Guidelines for the management of acute cholangitis and cholecystitis (TG 07) published in 2007 and the evidence reported subsequently, as well as miscellaneous etiology that has not so far been touched on. (1) Oriental cholangitis is the type of cholangitis that occurs following intrahepatic stones and is frequently referred to as an endemic disease in Southeast Asian regions. The characteristics and diagnosis of oriental cholangitis are also commented on. (2) TG 07 recommended percutaneous transhepatic biliary drainage in patients with cholestasis (many of the patients have obstructive jaundice or acute cholangitis and present clinical signs due to hilar biliary stenosis or obstruction). However, the usefulness of endoscopic naso-biliary drainage has increased along with the spread of endoscopic biliary drainage procedures. (3) As for biliary tract infections in patients who underwent biliary tract surgery, the incidence rate of cholangitis after reconstruction of the biliary tract and liver transplantation is presented. (4) As for primary sclerosing cholangitis, the frequency, age of predilection and the rate of combination of inflammatory enteropathy and biliary tract cancer are presented. (5) In the case of acalculous cholecystitis, the frequency of occurrence, causative factors and complications as well as the frequency of gangrenous cholecystitis, gallbladder perforation and diagnostic accuracy are included in the updated Tokyo Guidelines 2013 (TG13). Free full-text articles and a mobile application of TG13 are available via http://www.jshbps.jp/en/guideline/tg13.html.

  3. Bacterial cholangitis causing secondary sclerosing cholangitis: a case report

    NARCIS (Netherlands)

    H.R. van Buuren (Henk); A.C.T.M. Depla (Annekatrien); P.C.J. ter Borg (Pieter)

    2002-01-01

    textabstractBACKGROUND: Although bacterial cholangitis is frequently mentioned as a cause of secondary sclerosing cholangitis, it appears to be extremely rare, with only one documented case ever reported. CASE PRESENTATION: A 48-year-old woman presented with an episode of acute bil

  4. Bacterial cholangitis causing secondary sclerosing cholangitis: A case report

    NARCIS (Netherlands)

    P.C.J. ter Borg (Pieter); H.R. van Buuren (Henk); A.C.T.M. Depla (Annekatrien)

    2002-01-01

    textabstractBackground: Although bacterial cholangitis is frequently mentioned as a cause of secondary sclerosing cholangitis, it appears to be extremely rare, with only one documented case ever reported. Case presentation: A 48-year-old woman presented with an episode of acute biliary pancreatitis

  5. Glucocorticosteroids for primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Chen, Weikeng; Gluud, C

    2004-01-01

    Glucocorticosteroids have been suggested for primary sclerosing cholangitis, which is characterised by chronic inflammation and fibrosis in the intrahepatic and extrahepatic biliary tree.......Glucocorticosteroids have been suggested for primary sclerosing cholangitis, which is characterised by chronic inflammation and fibrosis in the intrahepatic and extrahepatic biliary tree....

  6. Glucocorticosteroids for primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Giljaca, Vanja; Poropat, Goran; Stimac, Davor

    2010-01-01

    Primary sclerosing cholangitis is a chronic cholestatic disease of intrahepatic and extrahepatic biliary ducts, characterised by chronic periductal inflammation and sclerosis of the ducts, which results in segmental stenoses of bile ducts, cholestasis, fibrosis, and ultimately, liver cirrhosis....... Patients with primary sclerosing cholangitis are at higher risk of cholangiocarcinoma as well as of colonic neoplasia, since primary sclerosing cholangitis is associated with inflammatory bowel disease in more than 80% of the patients. Several therapeutic modalities have been proposed for primary...... sclerosing cholangitis, like ursodeoxycholic acid, glucocorticosteroids, and immunomodulatory agents, but none has been successful in reversing the process of the disease. To date, liver transplantation is the only definite therapeutic solution for patients with advanced primary sclerosing cholangitis...

  7. Cutting edge issues in primary sclerosing cholangitis.

    Science.gov (United States)

    Bowlus, Christopher L

    2011-10-01

    Primary sclerosing cholangitis (PSC) is a chronic inflammatory liver disease characterized by the destruction of medium- to large-sized bile ducts and intense concentric fibrosis. Complications from PSC include bacterial cholangitis, cirrhosis, and cholangiocarcinoma and a therapy that might alter the natural history of the disease remains lacking. Our understanding of the pathogenesis of PSC also remains rudimentary but the strong association between PSC and inflammatory bowel disease suggest causal links between the diseases. The male predominance in PSC, lack of a defined, pathogenic auto-antigen, and the potential role of the innate immune system suggest that PSC may be due to dysregulation of immunity rather than a classic autoimmune disease. However, PSC shares several genetic susceptibility loci with other autoimmune diseases including the human leukocyte antigen DRB01*03 haplotype. The precise immune response of PSC is largely unknown but likely involves activation of the innate immune system by bacterial components delivered to the liver via the portal vein. Induction of adhesion molecules and chemokines leads to the recruitment of intestinal lymphocytes. Bile duct injury results from the sustained inflammation and production of inflammatory cytokines. Biliary strictures may cause further damage as a result of bile stasis and recurrent secondary bacterial cholangitis. Progress in our basic understanding of PSC is desperately needed in order to rationally design new therapeutic approaches to this disease.

  8. Common complication of percutaneous transhepatic cholangic drainage and its prevention%经皮肝穿刺胆管引流术的常见并发症及防治

    Institute of Scientific and Technical Information of China (English)

    柴文晓; 车明; 郑宁刚; 王江业

    2011-01-01

    Objective To analyze the complications of percutaneous transhepatic cholangic drainage (PTCD) and the prevention measures.Methods The data of 220 patients with obstruction jaundice who underwent PTCD were retrospectively analyzed.Results Among all 220 patients, extrahepatic obstructive jaundice was detected in 218 patients, including 9 benign obstruction and 209 malignant obstruction.Intrahepatic obstructive jaundice was found in 2 patients, including 1 of sclerosing cholangitis and 1 of liver metastasis with diffuse invasion of intrahepatic bile duct.Puncture was successful in 218 (218/220, 99.09%) patients with extrahepatic obstructive jaundice, but not in the 2 cases of intrahepatic jaundice.Nine patients died 24-72 h after PTCD.Intraoperative gallbladder-cardiac reflex was observed in 35 patients, hemorrhage in 6, biliary infection in 16, pleural injury in 3, while biliary peritonitis, acute renal failure, bile excessive secretion, acute myocardial infarction in anterior wall after the operation occurred in one case, respectively.Drainage tube dislocation occurred in 14 patients.Conclusion Complications frequently occur in patients treated with PTCD.Some of the serious complications may lead to death, but most of them can be prevented with positive treatment.%目的 分析经皮肝穿刺胆管引流术(PTCD)的常见并发症,提出防治措施.方法 对220例接受PTCD治疗的梗阻性黄疸患者的资料进行回顾性分析.结果 220例患者中,218例为肝外梗阻性黄疸,其中良性梗阻9例,恶性梗阻209例;2例为肝内梗阻性黄疸,其中硬化性胆管炎1例,肝转移瘤肝内胆管广泛受侵1例.穿刺成功率为99.09%,穿刺失败的2例均为肝内梗阻性黄疸.9例患者于PTCD术后24~72 h内死亡.PTCD术中出现胆心反射35例,术后出血6例,感染16例,胸膜损伤3例,胆汁性腹膜炎1例,急性肾衰竭1例,引流管脱出14例,胆汁过度分泌1例,急性前壁心肌梗死1例.结论 PTCD的并发症种类较

  9. Primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    Chapman Roger

    2006-10-01

    Full Text Available Abstract Primary sclerosing cholangitis (PSC is a chronic cholestatic liver disease of unknown aetiology characterised by inflammation and fibrosis of the biliary tree. The mean age at diagnosis is 40 years and men are affected twice as often as women. There is a reported annual incidence of PSC of 0.9–1.31/100,000 and point prevalence of 8.5–13.6/100,000. The onset of PSC is usually insidious and many patients are asymptomatic at diagnosis or have mild symptoms only such as fatigue, abdominal discomfort and pruritus In late stages, splenomegaly and jaundice may be a feature. In most, the disease progresses to cirrhosis and liver failure. Cholangiocarcinoma develops in 8–30% of patients. PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis. The disease is diagnosed on typical cholangiographic and histological findings and after exclusion of secondary sclerosing cholangitis. Median survival has been estimated to be 12 years from diagnosis in symptomatic patients. Patients who are asymptomatic at diagnosis, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis. Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect.

  10. Cholangitis with septic shock caused by Raoultella planticola.

    Science.gov (United States)

    Yokota, Kazuhisa; Gomi, Harumi; Miura, Yoshimasa; Sugano, Kentaro; Morisawa, Yuji

    2012-03-01

    Raoultella planticola (formerly Klebsiella planticola) is a Gram-negative bacterium that has been rarely reported in association with human infection. Here we describe a case of cholangitis complicated with septic shock caused by R. planticola in an immunocompromised patient with advanced cancer who underwent endoscopic retrograde cholangiopancreatography to extract common bile duct stones. The infection was cleared by piperacillin-tazobactam treatment.

  11. Primary sclerosing cholangitis and pregnancy

    Directory of Open Access Journals (Sweden)

    Casper Q. Kammeijer

    2011-08-01

    Full Text Available Primary sclerosing cholangitis is a progressive disease, and coincidentally in pregnancy it is rare. It is characterized by progressive inflammation and destruction of bile ducts finally resulting in liver failure. A rare case of primary sclerosing cholangitis in pregnancy is presented. The course of the pregnancy was marked by threatened preterm delivery and exacerbation of cholestasis. She was successfully treated with ursodeoxycholic acid (UDCA. Although, primary sclerosing cholangitis has both maternal and fetal effects on pregnancy, the overall outcome is favorable. Only few cases have been reported using high dose ursodeoxycholic acid for primary sclerosing cholangitis in pregnancy, it often improves pruritus but has no protection against stillbirth. Data on the safety to the fetus or neonate and long-term outcome are scarce.

  12. Concurrent systemic AA amyloidosis can discriminate primary sclerosing cholangitis from IgG4-associated cholangitis.

    Science.gov (United States)

    Kato, Takehiro; Komori, Atsumasa; Bae, Sung-Kwan; Migita, Kiyoshi; Ito, Masahiro; Motoyoshi, Yasuhide; Abiru, Seigo; Ishibashi, Hiromi

    2012-01-14

    Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis, except epidemic schistosomiasis. Among them, primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues, giving rise to systemic amyloidosis, due to a progressive and unresolved inflammatory process, and its possible association with inflammatory bowel diseases. Nevertheless, only one such case has been reported in the literature to date. We report a 69-year-old Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis, without any evidence of other inflammatory disorders. As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4, the presence of IgG4(+) plasma cells was examined systemically, resulting in unexpected documentation of Congo-red-positive amyloid deposits, but not IgG4(+) plasma cells, in the liver, stomach and salivary glands. Elevated serum IgG4 is the hallmark of IgG4-related disease, including IgG4-associated cholangitis, but it has also been demonstrated in certain patients with PSC. Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.

  13. Concurrent systemic AA amyloidosis can discriminate primary sclerosing cholangitis from IgG4-associated cholangitis

    Institute of Scientific and Technical Information of China (English)

    Takehiro Kato; Atsumasa Komori; Sung-Kwan Bae; Kiyoshi Migita; Masahiro Ito; Yasuhide Motoyoshi; Seigo Abiru; Hiromi Ishibashi

    2012-01-01

    Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis, except epidemic schistosomiasis. Among them, primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues, giving rise to systemic amyloidosis, due to a progressive and unresolved inflammatory process, and its possible association with inflammatory bowel diseases. Nevertheless, only one such case has been reported in the literature to date. We report a 69-yearold Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis, without any evidence of other inflammatory disorders. As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4, the presence of IgG4+ plasma cells was examined systemically, resulting in unexpected documentation of Congo-red-positive amyloid deposits, but not IgG4+ plasma cells, in the liver, stomach and salivary glands. Elevated serum IgG4 is the hallmark of IgG4-related disease, including IgG4-associated cholangitis, but it has also been demonstrated in certain patients with PSC. Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.

  14. Bile acids for primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Chen, Weikeng; Gluud, C

    2003-01-01

    Bile acids have been used for treating primary sclerosing cholangitis, but their beneficial and harmful effects remain unclear.......Bile acids have been used for treating primary sclerosing cholangitis, but their beneficial and harmful effects remain unclear....

  15. Long-term follow-up of children and adoles-cents with primary sclerosing cholangitis and autoimmune sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Vratislav Smolka; Eva Karaskova; Oksana Tkachyk; Kvetoslava Aiglova; Jiri Ehrmann; Kamila Michalkova; Michal Konecny; Jana Volejnikova

    2016-01-01

    BACKGROUND: Sclerosing cholangitis (SC) is a chronic cho-lestatic hepatobiliary disease with uncertain long-term prog-nosis in pediatric patients. This study aimed to evaluate long-term results in children with SC according to the types of SC. METHODS: We retrospectively followed up 25 children with SC over a period of 4-17 years (median 12). The diagnosis of SC was based on biochemical, histological and cholangio-graphic ifndings. Patients fuliflling diagnostic criteria for probable or deifnite autoimmune hepatitis at the time of diag-nosis were deifned as having autoimmune sclerosing cholangi-tis (ASC); other patients were included in a group of primary sclerosing cholangitis (PSC). The incidence of the following complications was studied: obstructive cholangitis, portal hy-pertension, advanced liver disease and death associated with the primary disease. RESULTS: Fourteen (56%) patients had PSC and 11 (44%) had ASC. Patients with ASC were signiifcantly younger at the time of diagnosis (12.3 vs 15.4 years,P=0.032) and had higher IgG levels (22.7 vs 17.2 g/L,P=0.003). The mentioned compli-cations occurred in 4 (16%) patients with SC, exclusively in the PSC group: one patient died from colorectal cancer, one patient underwent liver transplantation and two patients, in whom severe bile duct stenosis was present at diagnosis, were endoscopically treated for acute cholangitis. Furthermore, two other children with ASC and 2 children with PSC had elevated aminotransferase levels. The 10-year overall survival was 95.8% in all patients, 100% in patients without complicated liver disease, and 75.0% in patients with complications. CONCLUSION: In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC.

  16. Primary Sclerosing Cholangitis

    Directory of Open Access Journals (Sweden)

    A Stiehl

    2000-01-01

    Full Text Available Primary sclerosing cholangitis (PSC is a chronic cholestatic liver disease characterized by fibrosing inflammation and obliteration of intra- and/or extrahepatic bile ducts. The disease is one of the most common cholestatic diseases in adults and is diagnosed with increasing frequency. It is very often associated with ulcerative colitis. Patients with PSC have an increased incidence of bile duct carcinomas, and those with ulcerative colitis also have an increased incidence of colonic carcinomas. In end-stage disease, liver transplantation is the treatment of choice. Immunosuppressive treatment has little effect. Ursodeoxycholic acid (UDCA, which has been shown to improve liver histology and survival in patients with primary biliary cirrhosis, has a beneficial effect in PSC, provided that patients who develop major duct stenoses are treated endoscopically. The aim is to treat patients as early as possible to prevent progression to the advanced stages of the disease. During treatment with UDCA, stenoses of major ducts may develop, and early endoscopic dilation is highly effective. Because UDCA treatment improves but does not cure cholestatic liver diseases, permanent treatment seems to be necessary. Such prolonged treatment with UDCA may be recommended because, until now, no side effects have been reported. In patients with end-stage disease, UDCA is not effective and liver transplantation is indicated.

  17. Autoimmune pancreatitis and cholangitis

    Institute of Scientific and Technical Information of China (English)

    Niraj; Jani; James; Buxbaum

    2015-01-01

    Autoimmune pancreatitis(AIP) is part of a systemic fibrosclerotic process characterized by lymphoplasmacytic infiltrate with immunoglobulin G subtype-4(Ig G4) positive cells. It characteristically presents with biliary obstruction due to mass-like swelling of the pancreas. Frequently AIP is accompanied by extra-pancreaticmanifestations including retroperitoneal fibrosis, thyroid disease, and salivary gland involvement. Auto-antibodies, hypergammaglobulemia, and prompt resolution of pancreatic and extrapancreatic findings with steroids signify its autoimmune nature. Refractory cases are responsive to immunomodulators and rituximab. Involvement of the biliary tree, termed IgG 4 associated cholangiopathy, mimics primary sclerosing cholangitis and is challenging to manage. High IgG 4 levels and swelling of the pancreas with a diminutive pancreatic duct are suggestive of autoimmune pancreatitis. Given similarities in presentation but radical differences in management and outcome, differentiation from pancreatic malignancy is of paramount importance. There is controversy regarding the optimal diagnostic criterion and steroid trials to make the diagnosis. Additionally, the retroperitoneal location of the pancreas and requirement for histologic sampling, makes tissue acquisition challenging. Recently, a second type of autoimmune pancreatitis has been recognized with similar clinical presentation and steroid response though different histology, serologic, and extrapancreatic findings.

  18. Complication of Salmonella Bacteremia in a Case of Treated Fungal Endophthalmitis

    Directory of Open Access Journals (Sweden)

    J. Malathi

    2012-01-01

    Full Text Available This is to report a case of bacteremia caused by Salmonella typhi in a treated unilateral fungal endogenous endophthalmitis in an 18-year-old male from one of the South Asian countries. Microbiological and molecular investigations were carried out on the eviscerated material and routine blood culture was carried out. Direct examination of eviscerated material revealed the presence of fungal filaments. However, Salmonella typhi was isolated from both specimens, which was confirmed by Polymerase chain reaction targeting the 16SrRNA gene, sequencing, and random amplification of polymorphic DNA showed that they belonged to the same clone. The presence of Salmonella bacteremia in a treated unilateral fungal endophthalmitis, among young adult patients is rare and systemic symptoms should be investigated.

  19. Acute Obstructive Cholangitis after Transarterial Chemoembolization: the Effect of Percutaneous Transhepatic Removal of Tumor Fragment

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Kyu Ho; Cho, Young Kwon; An, Jin Kyung; Woo, Jeong Joo; Kim, Hyun Sook; Choi, Yun Sun [Eulji Medical Center, Eulji University College of Medicine, Seoul (Korea, Republic of)

    2009-04-15

    Acute obstructive cholangitis due to the migration of necrotized tumor fragment is a rare complication occurring after a transarterial chemoembolization. The percutaneous tumor removal procedure following percutaneous transhepatic biliary drainage is an appropriate treatment over endoscopic removal for the relief of acute cholangitis in this case. Following this serial management, no invasive hepatocellular carcinoma of the bile duct recurred after two years of follow-up.

  20. Pregnancy in primary sclerosing cholangitis

    NARCIS (Netherlands)

    Wellge, Bjoern E.; Sterneck, Martina; Teufel, Andreas; Rust, Christian; Franke, Andre; Schreiber, Stefan; Berg, Thomas; Guenther, Rainer; Kreisel, Wolfgang; Eulenburg, Christine Zu; Braun, Felix; Beuers, Ulrich; Galle, Peter R.; Lohse, Ansgar W.; Schramm, Christoph

    2011-01-01

    Background There is a paucity of data on fertility or pregnancy in patients with primary sclerosing cholangitis (PSC). Objective To assess fertility in PSC by comparing the number of children in a large cohort of PSC patients to healthy controls and to investigate the outcome of pregnancy, as well a

  1. MRCP findings in recurrent pyogenic cholangitis

    Energy Technology Data Exchange (ETDEWEB)

    Jain, Manoj [Department of Radiology, AMRI Hospitals, Salt Lake, JC-16 and 17 Salt Lake City, Kolkata 700091 (India)], E-mail: jainmanoj70@hotmail.com; Agarwal, Ajay [Department of Radiology, AMRI Hospitals, Salt Lake, JC-16 and 17 Salt Lake City, Kolkata 700091 (India)], E-mail: doctorajay2002@yahoo.co.in

    2008-04-15

    Objective: In this essay, we present the spectrum of intrahepatic and extrahepatic bile duct abnormalities seen on MRCP in patients with recurrent pyogenic cholangitis. Conclusion: MRCP is a promising, noninvasive alternative to more invasive direct cholangiography for evaluating the intrahepatic and extrahepatic bile ducts in patients with recurrent pyogenic cholangitis.

  2. Bile acids for primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Poropat, Goran; Giljaca, Vanja; Stimac, Davor

    2011-01-01

    Primary sclerosing cholangitis is a progressive chronic cholestatic liver disease that usually leads to the development of cirrhosis. Studies evaluating bile acids in the treatment of primary sclerosing cholangitis have shown a potential benefit of their use. However, no influence on patients...

  3. D-penicillamine for primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Klingenberg, S L; Chen, Weikeng

    2006-01-01

    Primary sclerosing cholangitis is a cholestatic disease. D-penicillamine is suggested as a treatment option due to its copper reducing and immunomodulatory potential.......Primary sclerosing cholangitis is a cholestatic disease. D-penicillamine is suggested as a treatment option due to its copper reducing and immunomodulatory potential....

  4. Intraperitoneal pseudocyst formation: complication of fungal peritonitis in continuous ambulatory peritoneal dialysis.

    Science.gov (United States)

    Sahpazova, E; Ruso, B; Kuzmanovska, D

    2007-10-01

    A 14-year-old girl, with end-stage renal disease on continuous ambulatory peritoneal dialysis (CAPD) the last 4 years, after an episode of Candida albicans was switched to hemodialysis. One month later she came back because of a palpable-painful abdominal mass and abdominal distention. Computed tomography (CT) and ultrasound examination demonstrated a demarkated fluid collection in the lower abdomen and pelvis. The cyst was drained percutaneously and the culture disclosed candida albicans which was treated with fluconasole. Two months later, the girl was admitted again with the same symptoms. An investigative laparotomy was undergone and the cyst was drained again. Fluid cultures were negative. CT abdomen examination six months later was negative for cyst relapse. In conclusion, intraperitoneal pseudocyst is a serious complication of CAPD. Surgical intervention may be preferable to percutaneous drainage.

  5. Intraperitoneal pseudocyst formation: Complication of fungal peritonitis in continuous ambulatory peritoneal dialysis

    Science.gov (United States)

    Sahpazova, E; Ruso, B; Kuzmanovska, D

    2007-01-01

    A 14-year-old girl, with end-stage renal disease on continuous ambulatory peritoneal dialysis (CAPD) the last 4 years, after an episode of Candida albicans was switched to hemodialysis. One month later she came back because of a palpable painful abdominal mass and abdominal distention. Computed tomography (CT) and ultrasound examination demonstrated a demarkated fluid collection in the lower abdomen and pelvis. The cyst was drained percutaneously and the culture disclosed candida albicans which was treated with fluconasole. Two months later, the girl was admitted again with the same symptoms. An investigative laparotomy was undergone and the cyst was drained again. Fluid cultures were negative. CT abdomen examination six months later was negative for cyst relapse. In conclusion, intraperitoneal pseudocyst is a serious complication of CAPD. Surgical intervention may be preferable to percutaneous drainage. PMID:19582199

  6. Ascariasis as an Unexpected Cause of Acute Pancreatitis with Cholangitis: A Rare Case Report from Urban Area

    OpenAIRE

    Pochamana Phisalprapa; Varayu Prachayakul

    2013-01-01

    Context Ascaris lumbricoides is one of the most common parasitic infestations of human gastrointestinal tract. This parasiticinfestation might be asymptomatic and only few studies on extraintestinal ascariasis have been reported. Ascending cholangitis and acute pancreatitis as a result of the Ascaris lumbricoides migrating into the biliary system and pancreatic duct were very rare complications. Here, we presented a case report of biliary ascariasis induced acute pancreatitis with cholangitis...

  7. 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.

  8. Chemotherapy-induced sclerosing cholangitis

    Energy Technology Data Exchange (ETDEWEB)

    Sandrasegaran, K.; Alazmi, W.M.; Tann, M.; Fogel, E.L.; McHenry, L.; Lehman, G.A

    2006-08-15

    Aim: To review the computed tomography (CT), magnetic resonance imaging (MRI) and cholangiographic findings of chemotherapy-induced sclerosing cholangitis (CISC). Methods: Between January 1995 and December 2004, 11 patients in the endoscopic retrograde cholangiography database were identified with CISC. Twelve CT, four MRI, 69 endoscopic and nine antegrade cholangiographic studies in these patients were reviewed. Serial change in appearance and response to endoscopic treatment were recorded. Results: CISC showed segmental irregular biliary dilatation with strictures of proximal extrahepatic bile ducts. The distal 5 cm of common bile duct was not affected in any patient. CT and MRI findings included altered vascular perfusion of one or more liver segments, liver metastases or peritoneal carcinomatosis. Biliary strictures needed repeated stenting in 10 patients (mean: every 4.7 months). Cirrhosis (n = 1) or confluent fibrosis (n = 0) were uncommon findings. Conclusion: CISC shares similar cholangiographic appearances to primary sclerosing cholangitis (PSC). Unlike PSC, biliary disease primarily involved ducts at the hepatic porta rather than intrahepatic ducts. Multiphasic contrast-enhanced CT or MRI may show evidence of perfusion abnormalities, cavitary liver lesions, or metastatic disease.

  9. Risk of waitlist mortality in patients with primary sclerosing cholangitis and bacterial cholangitis.

    Science.gov (United States)

    Goldberg, David S; Camp, Amanda; Martinez-Camacho, Alvaro; Forman, Lisa; Fortune, Brett; Reddy, K Rajender

    2013-03-01

    Patients with primary sclerosing cholangitis (PSC) are at increased risk for bacterial cholangitis because of biliary strictures and bile stasis. A subset of PSC patients suffer from repeated episodes of bacterial cholangitis, which can lead to frequent hospitalizations and impaired quality of life. Although waitlist candidates with PSC and bacterial cholangitis frequently receive exception points and/or are referred for living donor transplantation, the impact of bacterial cholangitis on waitlist mortality is unknown. We performed a retrospective cohort study of all adult waitlist candidates with PSC who were listed for initial transplantation between February 27, 2002 and June 1, 2012 at the University of Pennsylvania and the University of Colorado-Denver. During this period, 171 PSC patients were waitlisted for initial transplantation. Before waitlisting, 38.6% (66/171) of the patients had a history of bacterial cholangitis, whereas 28.0% (44/157) of the patients with at least 1 Model for End-Stage Liver Disease update experienced cholangitis on the waitlist. During follow-up, 30 patients (17.5%) were removed from the waitlist for death or clinical deterioration, with 46.7% (14/30) developing cholangiocarcinoma. Overall, 12 of the 82 waitlist candidates (14.6%) who ever had an episode of cholangitis were removed for death or clinical deterioration, whereas 18 of the 89 candidates (20.2%) without cholangitis were removed (P = 0.34 for a comparison of the 2 groups). No patients were removed because of bacterial cholangitis. In multivariate competing-risk models, a history of bacterial cholangitis was not associated with an increased risk of waitlist removal for death or clinical deterioration (subhazard ratio = 0.67, 95% confidence interval = 0.65-0.70, P PSC and bacterial cholangitis do not have an increased risk of waitlist mortality. The data call into question the systematic granting of exception points or referral for living donor transplantation due to a

  10. Assessment of acute cholangitis by MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Eun, Hyo Won, E-mail: namsanae@gmail.com [Health Promotion Center, Asan Medical Center, University of Ulsan, 388-1 Poongnap-dong, Songpa-gu, Seoul 138-736 (Korea, Republic of); Kim, Jung Hoon, E-mail: jhkim2008@gmail.com [Department of Radiology and Institute of Radiation Medicine, Seoul National University College of Medicine, 101 Daehang-no, Chongno-gu, Seoul 110-744 (Korea, Republic of); Hong, Seong Sook, E-mail: hongses@hosp.sch.ac.kr [Department of Radiology, Soonchunhyang University Hospital, 657 Hannam-Dong, Youngsan-Ku, Seoul 140-743 (Korea, Republic of); Kim, Young Jae, E-mail: rtwodtwo@hosp.sch.ac.kr [Department of Radiology, Soonchunhyang University Hospital, 657 Hannam-Dong, Youngsan-Ku, Seoul 140-743 (Korea, Republic of)

    2012-10-15

    Purpose: The purpose of this study is to assess the common MRI findings of acute cholangitis compared with those of non-acute cholangitis. Materials and methods: During a 31-month period, we performed MRCP and contrast-enhanced MRI on 173 patients with biliary abnormalities including duct dilatation or stricture. The causes of the biliary abnormalities included biliary stone disease (n = 85), cholangiocarcinoma (n = 47), periampullary cancer (n = 20), GB cancer (n = 4), and others (n = 17). Among 173 patients, 66 consecutive patients were confirmed with acute cholangitis diagnosed according to the Tokyo guideline, and 107 patients were confirmed as having non-acute cholangitis. Two radiologists retrospectively and independently accessed the MR findings, including the cause of biliary abnormality, increased periductal signal intensity on T2-weighted images, the transient periductal signal difference, and the presence of abscess, thrombosis, and ragged duct. They also measured the dilated duct and the thickened wall. The Student t-test and the Pearson chi-square were used. The κ statistics were used to determine interobserver agreement. Logistic regression was used to identify the MR findings that predicted acute cholangitis. Results: MRI correctly accessed the cause of biliary abnormality in 163 patients (94%). The statistically common findings for acute cholangitis were as follows: increased periductal signal intensity on T2-weighted imaging (n = 26, 39%, p < 0.05); transient periductal signal difference (n = 31, 47%, p < 0.05); abscess (n = 18, 27%, p < 0.05); thrombosis (n = 12, 18%, p < 0.05); and ragged duct (n = 11, 17%, p < 0.05). Interobserver agreement was good to excellent for each finding (κ = 0.74–0.97). The wall thickness showed a statistically significant difference between the acute cholangitis and the non-acute cholangitis group (2.65 mm:2.32 mm, p < 0.05), however, there was no significant difference in duct dilatation in the two groups. The

  11. Primary sclerosing cholangitis and liver transplantation

    NARCIS (Netherlands)

    Klompmaker, IJ; Haagsma, EB; Jansen, PLM; Slooff, MJH

    1996-01-01

    Primary sclerosing cholangitis is a chronic disease, strongly associated with ulcerative colitis and cholangiocarcinoma. Ulcerative colitis itself does not influence the liver transplant results. However; intensified screening after liver transplantation for carcinoma of the colon may be necessary.

  12. Etiopathogenesis of primary sclerosing cholangitis.

    Science.gov (United States)

    Chapman, Roger; Cullen, Sue

    2008-06-07

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology but lymphocytic portal tract infiltration is suggestive of an immune-mediated basis for this disease. Associations with inflammatory bowel disease (IBD) especially ulcerative colitis (UC), and with particular autoimmune diseases, as well as the genetic associations further suggest PSC may be an immune-mediated disease. The immunogenetics of PSC have been the subject of active research and several HLA and non-HLA associated genes have been implicated in the development of the disease. Lymphocytes derived from the inflamed gut may enter the liver via the enterohepatic circulation to cause hepatic disease. PSC may be triggered in genetically susceptible individuals by infections or toxins entering the portal circulation through a permeable colon and hence evoking an abnormal immune response.

  13. Pathogenic aspects of acute cholangitis

    Directory of Open Access Journals (Sweden)

    V. Borisenko

    2014-10-01

    Full Text Available The research is aimed at the study of dynamic pathomorphological changes of choledoch and acute cholangitis development factors determined during the experiment. 36 rats of Wistar line were under trial. The main group consisted of 30 animals undergoing the open laparotomy, choledoch ligation and puncture modeling of acute cholangitis by E. coli culture in 1 х 108 CFU/ml concentration under general anesthesia. 6 healthy rats were included in the control group. Samples of general biliary duct under autopsy for pathomorphological study were taken on the 3rd, 7th, 14th, 21st and 30th day. In panoramic samples colored by hematoxilin and eozin the degree of dystrophic, necrobiotic, hemodynamic, inflammatory and atrophic manifestations’ changes were studied. Average depth of choledoch wall and height of its epithelial lining were morphometrically estimated. Collagen of the IV type as well as expressing receptors to CD34 were defined with the help of monoclonal antibodies in choledoch epithelial cells of basal membranes and choledoch vessels endotheliocytes. In choledoch, enhancement of edema and inflammatory infiltration by lymphoplasmocytic elements with the admixture of neutrofils with granulation tissue was detected from the 3rd up to the 30th day of the experiment. From the 14th day formation of bile clots of blood was detected in choledoch clearance, part of which was locked to its de-epitheliolized internal surface. According to morphometrical study data, choledoch wall depth increased from 261.1 ± 3.13 µm on the 3rd day to 572.5 ± 3.42 µm on the 30th day of the experiment. Mucosa membrane has lost its folding on the 14th day, epitheliocytes flattening was replaced by their destruction with fragments rejection into the duct lumen by the 30th day of the experiment. The epithelium height index decreased from 14.8 ± 0.09 µm on the 3rd day to 11.7 ± 0.15 µm on the 30 day of the experiment. Collagen of the IV type fluorescence intensity of

  14. Autoantibodies in primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Johannes Roksund Hov; Kirsten Muri Boberg; Tom H Karlsen

    2008-01-01

    The aetiology of primary sclerosing cholangitis (PSC)is not known and controversy exists as to whether PSC should be denominated an autoimmune disease.A large number of autoantibodies have been detected in PSC patients,but the specificity of these antibodies is generally low,and the frequencies vary largely between different studies.The presence of autoantibodies in PSC may be the result of a nonspecific dysregulation of the immune system,but the literature in PSC points to the possible presence of specific antibody targets in the biliary epithelium and in neutrophil granulocytes.The present review aims to give an overview of the studies of autoantibodies in PSC,with a particular emphasis on the prevalence,clinical relevance and possible pathogenetic importance of each individual marker.

  15. Etiopathogenesis of primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Roger Chapman; Sue Cullen

    2008-01-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology but lymphocytic portal tract infiltration is suggestive of an immune-mediated basis for this disease.Associations with inflammatory bowel disease (IBD) especially ulcerative colitis (UC),and with particular autoimmune diseases,as well as the genetic associations further suggest PSC may be an immune-mediated disease.The immunogenetics of PSC have been the subject of active research and several HLA and non-HLA associated genes have been implicated in the development of the disease.Lymphocytes derived from the inflamed gut may enter the liver via the enterohepatic circulation to cause hepatic disease.PSC may be triggered in genetically susceptible individuals by infections or toxins entering the portal circulation through a permeable colon and hence evoking an abnormal immune response.

  16. Discrimination of suppurative cholangitis from nonsuppurative cholangitis with computed tomography (CT)

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Nam Kyung [Department of Diagnostic Radiology, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute, Pusan National University, Busan 602-739 (Korea, Republic of); Kim, Suk [Department of Diagnostic Radiology, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute, Pusan National University, Busan 602-739 (Korea, Republic of)], E-mail: kimsuk@medigate.net; Lee, Jun Woo; Kim, Chang Won [Department of Diagnostic Radiology, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute, Pusan National University, Busan 602-739 (Korea, Republic of); Kim, Gwang Ha; Kang, Dae Hwan [Department of Gastrointestinal Internal Medicine, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute, Pusan National University, Busan 602-739 (Korea, Republic of); Jo, Hong Jae [Department of Surgery, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute, Pusan National University, Busan 602-739 (Korea, Republic of)

    2009-03-15

    Purpose: Suppurative cholangitis is characterized by obstruction, inflammation, and pyogenic infection of the biliary tract. This disease represents a true emergency. The purpose of this study was to compare the computed tomography (CT) findings between acute calculous suppurative and nonsuppurative cholangitis and to determine if there are findings that assist in the differential diagnosis. Materials and methods: Fifteen patients with acute suppurative cholangitis were enrolled in this study. Findings at endoscopic retrograde cholangiopancreaticography (ERCP) were the standard of reference for suppurative cholangitis. To compare the findings of suppurative cholangitis with those of nonsuppurative cholangitis, 35 patients with nonsuppurative cholangitis were randomly selected. The following findings were evaluated: the presence of papillitis, the presence of stones in the ampulla, the presence of intrahepatic stones, the presence of early inhomogeneous enhancement of the liver, the degree of bile duct dilatation, the degree of bile duct wall thickening and presence of cholecystitis. Sensitivity and specificity for each of the individual findings were calculated. Statistical analyses were performed the Pearson {chi}{sup 2} test, Fisher's exact test and the Mann-Whitney U test. Results: Papillitis showed the highest specificity 86% with 60% sensitivity. Marked inhomogeneous enhancement of the liver during the arterial phase showed 80% specificity with 60% sensitivity. In multivariate logistic analysis, papillitis and marked early inhomogeneous enhancement of the liver were the most significant predictors of acute suppurative cholangitis. The combination of these two CT findings improved specificity (97% specificity) for the diagnosis of suppurative cholangitis. Conclusion: Papillitis and marked early inhomogeneous enhancement of the liver were found to be the most discriminative CT findings for the diagnosis of acute suppurative cholangitis and the

  17. Primary sclerosing cholangitis and Biliary cirrhosis associated with Ulcerative colitis

    Directory of Open Access Journals (Sweden)

    Aleem Ahmed Khan

    2011-07-01

    Full Text Available Primary sclerosing cholangitis and Ulcerative colitis are caused by progressive inflammation of the bile duct and large intestine respectively. The existence of any plausible association between Primary sclerosing cholangitis and Ulcerative colitis remains highly elusive. Little is known about the incidence and prevalence of primary sclerosing cholangitis with concomitant Ulcerative colitis in the Indian subcontinent. We report a case of Primary sclerosing cholangitis with long standing Ulcerative colitis which later also developed Primary biliary cirrhosis.

  18. MRCP in primary sclerosing cholangitis; MRCP bei primaerer sklerosierender Cholangitis

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    Weber, C.; Krupski, G.; Lorenzen, J.; Adam, G. [Universitaetsklinikum Hamburg-Eppendorf (Germany). Klinik und Poliklinik fuer Diagnostische und Interventionelle Radiologie; Grotelueschen, R.; Rogiers, X. [Universitaetsklinikum Hamburg-Eppendorf (Germany). Abt. fuer Hepatobilaere Chirurgie; Seitz, U. [Universitaetsklinikum Hamburg-Eppendorf (Germany). Klinik fuer Interdisziplinaere Endoskopie

    2003-02-01

    Purpose: Evaluation of MR-cholangiopancreaticography (MRCP) for the diagnosis of primary sclerosing cholangitis (PSC) in correlation with endoscopic retrograde cholangiopancreaticography (ERCP) and in comparison to the diagnostic accuracy of various T2-weighted sequences. Methods and Materials: Fifty-five patients (34 males, 21 females: mean age 40 years, range 16 to 65 years) with suspected PSC were examined in a 1.5 T MR unit (Magnetom Vision, Siemens, Erlangen), using breath-hold transverse and coronal HASTE, paracoronal RARE and thin-sliced HASTE (TS-HASTE) sequences. Applying a five-point-scale, two blinded investigators assessed the image quality for ROC analysis. Morphologic criteria of PSC were documented and correlated with ERCP, which served as the gold standard, and sensitivity, specificity and diagnostic accuracy were calculated. Results: PSC was confirmed in 40 of 55 patients (ERCP 55 of 55, liver biopsy 37 of 55), with concomitant chronic ulcerative colitis in 27 and Crohn's disease in 6 of the 40 patients. Qualitative analysis of the image quality showed no significant difference between RARE, HASTE and thin-sliced HASTE sequences (3.4/3.5/3.2). The RARE sequence had the highest sensitivity (97%), specificity (64%) and accuracy (84%) for the detection of PSC. The difference between HASTE and thin-sliced HASTE was statistically significant (p<0.01). Of the 40 patients with confirmed PSC, 29 were followed by MRI and 3 underwent a liver transplantation within the follow-up period. A Klatskin tumor, which was misdiagnosed by MRCP, was diagnosed by brush biopsy in 1 of the 40 patients. Interobserver variability was adequate to good (kappa 0.4 to 0.7), depending on the chosen sequence. (orig.) [German] Ziel: Wertigkeit der MRCP bei der Diagnostik der primaeren sklerosierenden Cholangitis (PSC) in Korrelation zur endoskopischen retrograden Cholangiopankreatikographie (ERCP) und Vergleich der diagnostischen Treffsicherheit verschiedener T{sub 2

  19. Primary Sclerosing Cholangitis: Multiple Phenotypes, Multiple Approaches.

    Science.gov (United States)

    Sarkar, Souvik; Bowlus, Christopher L

    2016-02-01

    Primary sclerosing cholangitis (PSC) is a heterogeneous, idiopathic, inflammatory disorder frequently associated with inflammatory bowel diseases. PSC patients may be classified into several subphenotypes. Investigations of pediatric, nonwhite, and female PSC patients have revealed distinguishing features. The natural history of PSC is variable in progression with numerous possible clinical outcomes. PSC patients may suffer bacterial cholangitis, cholangiocarcinoma, or colorectal adenocarcinoma. Treatments focusing on bile acid therapy and immunosuppression have not proven beneficial. Interest in PSC and international collaboration has led to improved understanding of the heterogeneity and the genetic structure and introduced possible effective therapeutics.

  20. A rare presentation of orbital complication of invasive fungal sinusitis in an immunocompetent young boy-a rare case

    Directory of Open Access Journals (Sweden)

    S Gaur

    2012-09-01

    Full Text Available We present a case of allergic fungal sinusitis (AFS in a 24 -year old man with history of left sided nasal obstruction and discharge since few years. Since few months he developed epiphora in the left eye associated with discomfort on eye movements. Patient was examined and CT with contrast was done. CT contrast showed an enhancing lesion in Left maxillary and ethmoid sinuses and erosion of the inferior bony wall of the orbit and medial wall of maxillary sinus. Though most patients of fungal sinusitis are immunocompromised but this patient was young male immunocompetent and made an unusual presentation with visual epiphora and painful eye movements. CT showed bony erosion of the Left inferior Bony wall of the Orbit and medial wall of Maxillary Sinus. After through examination and specific investigations, the patient was posted for surgery. We planed for Cald well –Luc’s Surgery and Endoscopic excision of the mass .Histological examination was reported as non malignant and microscopy showed Fungal Hyphae. After the surgery patient was discharged satisfactorily within couple of days and followed up regularly. Journal of College of Medical Sciences-Nepal,2012,Vol-8,No-1, 48-51 DOI: http://dx.doi.org/10.3126/jcmsn.v8i1.6826

  1. Endoscopic management of acute cholangitis in elderly patients

    Institute of Scientific and Technical Information of China (English)

    Naresh Agarwal; Barjesh Chander Sharma; Shiv K Sarin

    2006-01-01

    AIM: To evaluate clinical presentation, etiology, complications and response to treatment in elderly patients with acute cholangitis.METHODS: Demographics, etiology of biliary obstruction, clinical features, complications and associated systemic diseases of 175 patients with acute cholangitis were recorded. Endoscopic biliary drainage was performed using nasobiliary drain or stent. The complications related to ERCP, success of biliary drainage, morbidity, mortality and length of hospital stay were evaluated.RESULTS: Of 175 patients, 52 aged≥60 years (groupⅠ,age<60 years; groupⅡ,age≥60 years) and 105 were men. Fever was present in 38 of 52 patients of group Ⅱ compared to 120 of 123 in group Ⅰ. High fever (fever≥38.0℃) was more common in group Ⅰ (118/120 vs 18/38). Hypotension (5/123 vs 13/52),altered sensorium (3/123 vs 19/52), peritonism (22/123 vs 14/52), renal failure (5/123 vs 14/52) and associated comorbid diseases (4/123 vs 21/52) were more common in group Ⅱ. Biliopancreatic malignancy was a common cause of biliary obstruction in group Ⅱ (n = 34) and benign diseases in group Ⅰ (n = 120). Indications for biliary drainage were any one of the following either singly or in combination: a fever of≥38.0℃ (n=136),hypotension (n=18), peritonism (n=36), altered sensorium (n=22), and failure to improve within 72h of conservative management (n=22). High grade fever was more common indication of biliary drainage in group Ⅰ and hypotension, altered sensorium, peritonism and failure to improve within 72 h of conservative management were more common indications in group Ⅱ. Endoscopic biliary drainage was achieved in 172 patients (nasobiliary drain: 56 group Ⅰ, 24 group Ⅱ,stent: 64 group Ⅰ, 28 group Ⅱ) without any significant age related difference in the success rate. Abdominal pain, fever, jaundice, hypotension, altered sensorium,peritonism and renal failure improved after median time of 5 d in 120 patients in group Ⅰ (2-15 d

  2. Prospective risk assessment of endoscopic retrograde cholangiography in patients with primary sclerosing cholangitis. Dutch PSC Study Group.

    Science.gov (United States)

    van den Hazel, S J; Wolfhagen, E H; van Buuren, H R; van de Meeberg, P C; Van Leeuwen, D J

    2000-10-01

    Direct endoscopic retrograde cholangiopancreatography (ERCP) has become the standard for establishing the diagnosis of primary sclerosing cholangitis (PSC), while endoscopic procedures play an increasingly important therapeutic role. However, many believe that this procedure carries a significant risk of infection and other complications. We assessed the incidence of complications within 1 week of ERCP in patients with PSC. In a multicenter study, patients who underwent ERCP for (suspected) PSC were prospectively followed for the occurrence of complications after the procedure. A total of 106 ERCPs performed in 83 patients were evaluated. Complications occurred on ten occasions (9%): pancreatitis (n = 3), cholangitis (n = 2), increase of cholestasis (n = 2), postsphincterotomy bleeding (n = 1), cystic duct perforation (n = 1), and venous thrombosis (n = 1). All complications resolved quickly with proper therapy. Complications were more likely when ERCP was done to evaluate specific complaints such as jaundice or recurrent cholangitis (9/59) than after a purely diagnostic ERCP (1/47 relative risk [RR] 7.2, 95% confidence interval [CI] 1.00 to 153). Therapeutic interventions performed during ERCP (e.g. placement of endoprosthesis, dilation of strictures) also increased the risk of postprocedural complications (RR 4.5, 95 % CI 0.94 to 30). ERCP is a safe method for establishing the diagnosis of PSC in asymptomatic patients (2 % complication rate). Although ERCP in symptomatic patients carries a higher risk (14%), this can be justified by the benefits of endoscopic therapy.

  3. Clotrimazole/betamethasone diproprionate: a review of costs and complications in the treatment of common cutaneous fungal infections.

    Science.gov (United States)

    Greenberg, H L; Shwayder, Tor A; Bieszk, Nella; Fivenson, David P

    2002-01-01

    The use of antifungal/corticosteroid combinations as topical therapy for dermatophytoses has been criticized as being less effective, more expensive, and the cause of more adverse cutaneous reactions than antifungal monotherapy. The combination of clotrimazole and betamethasone diproprionate (Lotrisone) is a mix of an azole antifungal and a high-potency corticosteroid, and is one of the most widely prescribed of these combinations. Our objective was to describe the beneficial and deleterious effects of Lotrisone in the treatment of common cutaneous fungal infections and its relative cost-effectiveness. We did a literature review documenting clinical trial data and adverse reactions to Lotrisone and collected a cost analysis of topical antifungal prescribing data over a 2-month period from a large midwestern staff-model health maintenance organization (HMO). Lotrisone is approved by the U.S. Food and Drug Administration (FDA) for the treatment of tinea pedis, tinea cruris, and tinea corporis in adults and children more than 12 years of age. Treatment is limited to 2 weeks in the groin area and 4 weeks on the feet. The most concerning adverse effects of Lotrisone were reported in children and included treatment failure, striae distensae, hirsuitism, and growth retardation. This combination was also reported to have decreased efficacy in clearing candidal and Trichophyton infections as compared to single-agent antifungals. Lotrisone was considerably more expensive than clotrimazole alone and was found to account for more than 50% of topical antifungal expenditures as prescribed by primary care physicians, but only 7% of topical antifungals prescribed by dermatologists. We found that Lotrisone was shown to have the potential to induce many steroid-related side effects and to be less cost effective than antifungal monotherapy. This combination should be used judiciously in the treatment of cutaneous fungal infections and may not be appropriate for use in children.

  4. Efficacy and safety of emergency endoscopic retrograde cholangiopancreatography for acute cholangitis in the elderly

    Science.gov (United States)

    Tohda, Gen; Ohtani, Masahiro; Dochin, Masaki

    2016-01-01

    AIM To investigate the efficacy and safety of emergency endoscopic retrograde cholangiopancreatography (ERCP) in elderly patients with acute cholangitis. METHODS From June 2008 to May 2016, emergency ERCPs were performed in 207 cases of acute cholangitis at our institution. Patients were classified as elderly if they were aged 80 years and older (n = 102); controls were under the age of 80 years (n = 105). The patients’ medical records were retrospectively reviewed for comorbidities, laboratory data, etiology of cholangitis (presence of biliary stones, biliary stricture and malignancy), details of the ERCP (therapeutic approaches, technical success rates, procedure duration), ERCP-related complications and mortality. RESULTS The frequency of comorbidities was higher in the elderly group than the control group (91.2% vs 67.6%). Periampullary diverticulum was observed in the elderly group at a higher frequency than the control group (24.5% vs 13.3%). Between the groups, there was no significant difference in the technical success rates (95.1% vs 95.2%) or endoscopic procedure durations. With regard to the frequency of ERCP-related complications, there was no significant difference between the two groups (6.9% vs 6.7%), except for a lower rate of post-ERCP pancreatitis in the elderly group than in the control group (1.0% vs 3.8%). Neither angiographic nor surgical intervention was required in any of the cases with ERCP-related complications. There was no mortality during the observational periods. CONCLUSION Emergency ERCP for acute cholangitis can be performed safely even in elderly patients aged 80 years and older. PMID:27729744

  5. IgG4 Related Sclerosing Cholangitis

    Directory of Open Access Journals (Sweden)

    D. Joshi

    2014-01-01

    Full Text Available IgG4 related disease (IgG4-RD is a multisystemic disorder which has only recently been recognized. IgG4 related sclerosing cholangitis (IgG4-SC is the biliary manifestation of the disease, often in association with autoimmune pancreatitis (AIP. In this review, we provide an overview of IgG4-RD, with a focus on the biliary manifestations. In particular, we describe the important differential diagnoses of IgG4-SC that need to be considered, namely, primary sclerosing cholangitis (PSC and cholangiocarcinoma, and provide a management algorithm. Finally, we highlight future directions and unanswered questions which will provide new insights into this exciting and evolving disease entity.

  6. Sclerosing Cholangitis after Transcatheter Arterial Chemoembolization:a Case Report

    Institute of Scientific and Technical Information of China (English)

    Kai Qu; Chang Liu; Qi-fei Wu; Bo Wang; Aasef Mohamed Ali Mansoor; Hao Qin; Qiang Ma; Ya-min Liu

    2011-01-01

    @@ SCLEROSING cholangitis represents progressing jaundice or/and paroxysmal symptom of cholangitis, finally developing to end-stage of liver disease.When compared with primary sclerosing cholangitis (PSC), there are no apparent differences in pathology and clinical manifestation in secondary sclerosing cholangitis (SSC).But unlike PSC, SSC always has underlying causes, the most common being biliary obstruction, surgical trauma and ischemic injury of bile duct during liver transplantation .1,2 Repeated transcatheter arterial chemoembolization (TACE) leading to progressive SSC was rarely reported.Because of its rapid and irreversible progression, once SSC begins, it is difficult to deal with.Therefore, clinicians need to pay more attention to it.

  7. [Eosinophilic cholangitis from almost normal appearance to the bile duct sclerosis similar to primary sclerosing cholangitis].

    Science.gov (United States)

    Kimura, Yoshito; Yamashita, Yukimasa; Mikami, Sakae; Ono, Hiroshi; Itai, Ryosuke; Matsumoto, Yoshihide; Yamada, Satoshi; Takada, Mariko; Sumitomo, Yasuhiko

    2013-02-01

    A 48-year-old man was admitted to our hospital because of eosinophilia and liver dysfunction. Initial abdominal CT and MRI (MRCP) finding showed almost normal liver and bile duct. Liver biopsy demonstrated mild portal infiltration of lymphocytes and eosinophils. Definitive diagnosis was difficult, but we suspected autoimmune disease. Oral steroid administration was started, which led to a rapid improvement of eosinophilia and liver dysfunction. Dose reduction of steroid administration resulted in exacerbation of eosinophilia and liver dysfunction. Follow-up MRCP and ERCP study revealed biliary strictures similar to primary sclerosing cholangitis (PSC). A second liver biopsy revealed dense infiltration composed of lymphocytes and eosinophils in the portal area. Therefore we diagnosed eosinophilic cholangitis. This is the first case of eosinophilic cholangitis, observed after changes of the bile duct from an almost normal appearance to diffuse sclerosing and narrowing similar to PSC by imaging and pathological studies.

  8. IgG4-associated cholangitis.

    Science.gov (United States)

    Nath, Vikas; Lewin, Jack; Subramony, Charu; Shenoy, Veena

    2014-12-01

    We report a young female patient with IgG4-associated cholangitis (IAC) who presented with common bile duct (CBD) stricture and review the features that distinguish IAC from both primary sclerosing cholangitis (PSC) and other types of secondary sclerosing cholangitis (SSC). IAC is a biliary manifestation of IgG4-related sclerosing disease, an autoimmune condition characterized by elevated serum IgG4 and infiltrates containing lymphocytes and IgG4-positive plasma cells, accompanied by sclerosis. On endoscopic retrograde cholangiopancreatography, IAC consists of segmental biliary strictures with a predilection for the distal CBD, whereas in PSC the strictures are more band-like; other types of SSC often demonstrate unifocal ductal obstructions, sometimes associated with choleliths. On histologic examination, the bile duct wall in IAC contains a denser lymphocytic infiltrate and sparser sclerosis than in PSC; other types of SSC can be distinguished histologically by the types of inflammatory cells present. Unlike those of PSC, IAC-related strictures are reversible with corticosteroids.

  9. Recurrent acute pancreatitis and cholangitis in a patient with autosomal dominant polycystic kidney disease

    Directory of Open Access Journals (Sweden)

    Kambiz Yazdanpanah

    2013-01-01

    Full Text Available Autosomal dominant polycystic kidney disease (ADPKD is an inherited disorder associated with multiple cyst formation in the different organs. Development of pancreatic cyst in ADPKD is often asymptomatic and is associated with no complication. A 38-year-old man with ADPKD was presented with six episodes of acute pancreatitis and two episodes of cholangitis in a period of 12 months. Various imaging studies revealed multiple renal, hepatic and pancreatic cysts, mild ectasia of pancreatic duct, dilation of biliary system and absence of biliary stone. He was managed with conservative treatment for each attack. ADPKD should be considered as a potential risk factor for recurrent acute and/or chronic pancreatitis and cholangitis.

  10. Acute Cholangitis After Bilioenteric Anastomosis for Bile Duct Injuries.

    Science.gov (United States)

    Ortiz-Brizuela, Edgar; Sifuentes-Osornio, José; Manzur-Sandoval, Daniel; Terán-Ellis, Santiago Mier Y; Ponce-de-León, Sergio; Torres-González, Pedro; Mercado, Miguel Ángel

    2017-07-25

    The study aims to describe the clinical features, microbiology, and associated factors of acute cholangitis (AC) after bilioenteric anastomosis (BEA) for biliary duct injury (BDI). Additionally, we assessed the performance of the Tokyo Guidelines 2013 (TG13) recommendations in these patients. We conducted a case-control study of 524 adults with a history of BEA for BDI from January 2000 to January 2014. A propensity score adjustment was performed for the analysis of the independent role of the main factors identified during the univariate logistic regression procedure. We identified 117 episodes of AC in 70 patients; 51.3% were definitive AC according to the TG13 diagnostic criteria, and 39.3% did not fulfill the imaging criteria of AC. A history of post-operative biliary complications (OR 2.55, 95% CI 1.38-4.70) and the bile duct confluence preservation (OR 0.46, 95% CI 0.24-0.87) were associated with AC. Eighty-nine percent of the microorganisms were Enterobacteriaceae; of them, 28% were extended spectrum β-lactamase (ESBL) producers. AC is a common complication after BEA and must be suspected even in the absence of imaging findings, particulary in patients with a history of post-operative biliary complications, and/or without bile duct confluence preserved. An empirical treatment for ESBL-producing Enterobacteriaceae may be appropriate in patients living in countries with a high rate of bacterial drug resistance.

  11. Curcumin and its promise as an anticancer drug: An analysis of its anticancer and antifungal effects in cancer and associated complications from invasive fungal infections.

    Science.gov (United States)

    Chen, Jin; He, Zheng-Min; Wang, Feng-Ling; Zhang, Zheng-Sheng; Liu, Xiu-zhen; Zhai, Dan-Dan; Chen, Wei-Dong

    2016-02-05

    Invasive fungal infections (IFI) are important complications of cancer, and they have become a major cause of morbidity and mortality in cancer patients. Effective anti-infection therapy is necessary to inhibit significant deterioration from these infections. However, they are difficult to treat, and increasing antifungal drug resistance often leads to a relapse. Curcumin, a natural component that is isolated from the rhizome of Curcuma longa plants, has attracted great interest among many scientists studying solid cancers over the last half century. Interestingly, curcumin provides an ideal alternative to current therapies because of its relatively safe profile, even at high doses. To date, curcumin's potent antifungal activity against different strains of Candida, Cryptococcus, Aspergillus, Trichosporon and Paracoccidioides have been reported, indicating that curcumin anticancer drugs may also possess an antifungal role, helping cancer patients to resist IFI complications. The aim of this review is to discuss curcumin's dual pharmacological activities regarding its applications as a natural anticancer and antifungal agent. These dual pharmacological activities are expected to lead to clinical trials and to improve infection survival among cancer patients. Copyright © 2015 Elsevier B.V. All rights reserved.

  12. Analysis of MRI manifestation of cholangitis%胆管炎的MRI表现分析

    Institute of Scientific and Technical Information of China (English)

    杨景震; 霍英杰; 张玉; 袁静; 赵永强; 邹翠洁

    2013-01-01

    Objective To evaluate ultra high-field Magnetic Resonance imaging in diagnosis and imaging features of cholangitis.Methods The authors completed a retrospective analysis of MRI findings in 8patients with cholangitis.Resuits 2 patients had complicated with cholangitis postcholecystectomy; 4 patients with recurrent pyogenic cholangitis (RPC); 2 patients with autoimmune pancreatitis (AIP).MRI showed mainly:morphological changes of bile duct,with stricture and dilatation; changes of bile duct wall,with thickness and contrast enhancement.Other abnormal signs with cholangitis were found including the early enhancements in the portal vaginae vasorum,adjacent structures and hepatic parenchyma near cholangitis area,mutiple small abscesses of hepatic parenchyma and pancreas's morphological and signal abnormity.Conclusion 3.0 T MR imaging in cholangitis can provide more pathologic details,and s MR enterrography with a 3.0 T scanner can provide more pathologic details,contrast agent enhancement effectness with high nensitivity could reflect arterial overperfusion in lesions,which could be the valuable imaging in diagnosis of cholangitis.The highquailty imaging of hepatic,biliary,pancreatic various series has significant advantages in identification of related diseases.%目的 探讨超高场强MR用于胆管炎诊断的MRI特征.方法 对8例胆管炎的MRI表现进行回顾性分析.结果 胆囊切除术后并发感染性胆管炎2例,复发性化脓性胆管炎(recurrent pyogenic cholangitis,RPC)4例,自身免疫性胰腺炎(autoimmune pancreatitis,AIP)并发胆管炎2例.主要MRI所见:胆管形态的改变表现为狭窄与扩张;胆管壁的改变表现为增厚及强化;与胆管炎伴随的其它异常包括肝门血管鞘及邻近结构、胆管炎邻近的肝实质早期强化,肝实质小脓肿,胰腺形态及信号异常.结论 3.0T MR在胆管炎诊断中可获取更多的病理信息,其中,所反映出的病变区动脉过度灌注具有高敏感性,在

  13. Autoimmune Cholangitis: A Variant Syndrome of Autoimmune Hepatitis

    OpenAIRE

    Brij Sharma; Sujeet Raina; Rajesh Sharma

    2014-01-01

    Autoimmune cholangitis (AIC) or autoimmune cholangiopathy is a chronic inflammation of liver and a variant syndrome of autoimmune hepatitis (AIH). We present a case of an adult female who had biochemical features of cholestasis and transaminasemia but aminotransferases were not in the hepatitis range and had histological evidence of bile duct injury which was subsequently diagnosed as autoimmune cholangitis.

  14. Fungal Sinusitis

    Science.gov (United States)

    ... Marketplace Find an ENT Doctor Near You Fungal Sinusitis Fungal Sinusitis Patient Health Information News media interested ... sinusitis results. There Are Four Types Of Fungal Sinusitis: Mycetoma Fungal Sinusitis produces clumps of spores, a " ...

  15. An unusual presentation of primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Franklin Whitney Goldwire; William E Norris; Jonathan M Koff; Zachary D Goodman; Milton T Smith

    2008-01-01

    This case report describes the unusual presentation of a patient who had findings which were initially suggestive of a type IV choledochal cyst.Her liver biopsy demonstrated biliary cirrhosis.She was treated with endoscopic retrograde cholangiopancreatography and biliary stent exchanges over one year.Her cholangiogram one year later demonstrated resolution of the biliary cystic dilation which led to her initial diagnosis,with beading and stricturing of the hepatic ducts consistent with primary sclerosing cholangitis.Liver-associated enzymes and physical findings also improved.A liver biopsy one year later demonstrated a marked improvement in hepatic fibrosis with no evidence of cirrhosis.

  16. Perinatal events and the risk of developing primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Annika Bergquist; Scott M Montgomery; Ulrika Lund; Anders Ekbom; Rolf Olsson; Stefan Lindgren; Hanne Prytz; Rolf Hultcrantz; Ulrika Broomé

    2006-01-01

    AIM: To investigate whether perinatal events,intrauterine or postpartum, are associated with the development of primary sclerosing cholangitis (PSC) later in life.METHODS: Birth records from 97 patients with adult PSC in Sweden were reviewed. Information on perinatal events including medications and complications during pregnancy, gestation length, birth weight and length were collected. Two control children of the same sex were selected for each subject. Conditional multiple logistic regression was used to assess associations of the perinatal measures with development of PSC.RESULTS: No significant associations were found between gestational age, birth length, breastfeeding,and the majority of medical complications including infections or medication during pregnancy for the mothers or postpartum for the children. Vaginal bleeding and peripheral oedema showed associations with PSC,with matched odds ratios of 5.70 (95% CI, 1.13-28.83)and 2.28 (95% CI, 1.04-5.03), respectively.CONCLUSION: The associations of vaginal bleeding and oedema with subsequent PSC cannot readily be explained, so our findings do not strongly support the hypothesis of a significant role of perinatal events as a risk for the development of PSC later in life.

  17. Sclerosing cholangitis: Clinicopathologic features, imaging spectrum, and systemic approach to differential diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Ni Eun [Dept. of Radiology, Research Institute of Radiological Science, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, So Yeon; Lee, Seung Soo; Byun, Jae Ho; Kim, Hyoung Jung; Kim, Jin Hee; Lee, Moon Gyu [Dept. of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    2016-02-15

    Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes that include immunoglobulin G4-related sclerosing disease, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndrome-related cholangitis, and eosinophilic cholangitis. In this review, we suggest a systemic approach to the differential diagnosis of sclerosing cholangitis based on the clinical and laboratory findings, as well as the typical imaging features on computed tomography and magnetic resonance (MR) imaging with MR cholangiography. Familiarity with various etiologies of sclerosing cholangitis and awareness of their typical clinical and imaging findings are essential for an accurate diagnosis and appropriate management.

  18. Sclerosing cholangitis following severe trauma: Description of a remarkable disease entity with emphasis on possible pathophysiologic mechanisms

    Institute of Scientific and Technical Information of China (English)

    Johannes Benninger; Rainer Grobholz; Yurdaguel Oeztuerk; Christoph H. Antoni; Eckhart G. Hahn; Manfred V. Singer; Richard Strauss

    2005-01-01

    AIM: Persistent cholestasis is a rare complication of severe trauma or infections, Little is known about the possible pathomechanisms and the clinical course,METHODS: Secondary sclerosing cholangitis was diagnosed in five patients with persistent jaundice after severe trauma (one burn injury, three accidents, one power current injury). Medical charts were retrospectively reviewed with regard to possible trigger mechanisms for cholestasis, and the clinical course was recorded.RESULTS: Diagnosis of secondary sclerosing cholangitis was based in all patients on the primary sclerosing cholangitis (PSC)-like destruction of the intrahepatic bile ducts at cholangiography after exclusion of PSC. In four patients, arterial hypotension with subsequent ischemia may have caused the bile duct damage, whereas in the case of power current injury direct thermal damage was assumed to be the trigger mechanism. The course of secondary liver fibrosis was rapidly progressive and proceeded to liver cirrhosis in all four patients with a follow-up >2 years. Therapeutic possibilities were limited.CONCLUSION: Posttraumatic sclerosing cholangitis is a rare but rapidly progressive disease, probably caused by ischemia of the intrahepatic bile ducts via the peribiliary capillary plexus due to arterial hypotension. Gastroenterologists should be aware of this disease in patients with persistent cholestasis after severe trauma.

  19. Evolving concepts in primary sclerosing cholangitis.

    Science.gov (United States)

    Krones, Elisabeth; Graziadei, Ivo; Trauner, Michael; Fickert, Peter

    2012-03-01

    Patients suffering from primary sclerosing cholangitis (PSC) show considerable differences regarding clinical manifestations (i.e. large duct versus small-duct PSC, presence or absence of concomitant inflammatory bowel disease), disease progression, risk for malignancy and response to therapy, raising the question whether PSC may represent a mixed bag of diseases of different aetiologies. The growing list of secondary causes and diseases 'mimicking' or even overlapping with PSC (e.g. IgG4-associated sclerosing cholangitis), which frequently causes problems in clear-cut discrimination from classic PSC and the emerging knowledge about potential disease modifier genes (e.g. variants of CFTR, TGR5 and MDR3) support such a conceptual view. In addition, PSC in children differs significantly from PSC in adults in several aspects resulting in distinct therapeutic concepts. From a clinical perspective, appropriate categorization and careful differential diagnosis are essential for the management of concerned patients. Therefore, the aim of the current review is to summarize current and evolving pathophysiological concepts and to provide up-to-date perspectives including future treatment strategies for PSC.

  20. Current strategies for endoscopic management of acute cholangitis.

    Science.gov (United States)

    Isayama, Hiroyuki; Yasuda, Ichiro; Tan, Damien

    2017-04-01

    At the pancreatobiliary session of Endoscopic Forum Japan (EFJ) 2016, current strategies for the endoscopic management of acute cholangitis were discussed. The topics consisted of two major parts, namely endoscopic management of acute cholangitis caused by common bile duct stones (CBDS) and biliary stent occlusion. Endoscopists from nine Japanese high-volume centers along with two overseas centers participated in the questionnaires and discussion. Strategies for management of cholangitis due to CBDS were agreed upon, and the clinical guideline of acute cholangitis (Tokyo guidelines 2013) was accepted. The best timing for drainage in Grade 2 (moderate) cholangitis urgent or early (strategies differed according to the stricture location (distal or hilar) and stent type initially placed (Covered or uncovered metal stent). Strategies for management of cholangitis caused by CBD stones are well established according to the TG13. More evidence is required before further recommendations can be made with regards to cholangitis due to stent occlusion. We aim to clarify this in the near future with questionnaires and consensus from experts. © 2017 The Authors. Digestive Endoscopy © 2017 Japan Gastroenterological Endoscopy Society.

  1. Systematic review: management options for primary sclerosing cholangitis and its variant forms - IgG4-associated cholangitis and overlap with autoimmune hepatitis.

    Science.gov (United States)

    Culver, E L; Chapman, R W

    2011-06-01

    Primary sclerosing cholangitis (PSC) remains a challenging disease to manage. The main goals are prevention of disease progression and reduction of the increased cancer risk. To review the management strategies for PSC and its variant forms based on published studies. Publications were identified using Pubmed, Medline and Ovid search engines. Distinguishing PSC from variants, such as IgG4-associated cholangitis, and overlap with autoimmune hepatitis is essential to guide treatment decisions. There is no proven efficacious medical treatment for PSC. Ursodeoxycholic acid has been disappointing in low and moderate doses, and potentially dangerous in higher doses, although its role and optimal dose in chemoprevention requires investigation. The novel bile acid, 24-norursodeoxycholic acid, has shown promise in mouse models; human trials are in progress. Dominant strictures are optimally managed by dilatation and stenting to relieve obstructive complications, although exclusion of biliary malignancy is essential. Liver transplantation is the only proven therapy for those with advanced disease. Cholangiocarcinoma remains the most unpredictable and feared complication. In highly selected groups, neo-adjuvant chemoradiation with liver transplantation seems promising, but requires further validation. Screening for inflammatory bowel disease and surveillance for colorectal carcinoma should not be overlooked. The effective management of PSC and its variants is hindered by uncertainties regarding pathogenesis of disease and factors responsible for its progression. Genome studies may help to identify further targets for drug therapy and factors leading to malignant transformation. © 2011 Blackwell Publishing Ltd.

  2. Acute Cholangitis following Biliary Obstruction after Duodenal OTSC Placement in a Case of Large Chronic Duodenocutaneous Fistula

    Directory of Open Access Journals (Sweden)

    Yaseen Alastal

    2015-01-01

    Full Text Available Over-the-Scope Clip system, also called “Bear Claw,” is a novel endoscopic modality used for closure of gastrointestinal defect with high efficacy and safety. We present a patient with history of eosinophilic gastroenteritis and multiple abdominal surgeries including Billroth II gastrectomy complicated by a large chronic duodenocutaneous fistula from a Billroth II afferent limb to the abdominal wall. Bear Claw clip was used for closure of this fistula. The patient developed acute cholangitis one day after placement of the Bear Claw clip. Acute cholangitis due to papillary obstruction is a potential complication of Bear Claw placement at the dome of the duodenal stump (afferent limb in patient with Billroth II surgery due to its close proximity to the major papilla.

  3. Post–Endoscopic Retrograde Cholangiopancreaticography complications in liver transplanted patients, a single-center experience

    DEFF Research Database (Denmark)

    Ambrus, R B; Svendsen, Lars Bo; Hillingsø, J G

    2015-01-01

    patients during a 9-year period. RESULTS: A total of 292 ERCPs were included. Overall post-ERCP complications occurred in 24 procedures (8.2%): pancreatitis in 8 (2.7%), bleeding in 5 (1.7%), and cholangitis in 13 (4.5%) procedures. Simultaneous pancreatitis and cholangitis, and simultaneous bleeding...... and cholangitis occurred after two procedures, respectively. Multivariate analysis concerning overall complications identified biliary sphincterotomy (p = 0.006) and time since liver transplantation within 90 days postoperatively (p = 0.044) as risk factors for post-ERCP complications. Specifically concerning...... post-ERCP pancreatitis (PEP), it was found that pre-ERCP cholangitis was another independent risk factor for PEP (p = 0.026). Stent in the biliary tract prior to ERCP seemed to be protective (p = 0.041). CONCLUSIONS: Complications were of surprisingly mild degree. The rates of post-ERCP complications...

  4. Primary sclerosing cholangitis in children: utility of magnetic resonance cholangiopancreatography

    Energy Technology Data Exchange (ETDEWEB)

    Chavhan, Govind B.; Babyn, Paul S.; Manson, David E. [The Hospital for Sick Children, Department of Diagnostic Imaging, Toronto (Canada); Roberts, Eve [The Hospital for Sick Children, Department of Gastroenterology, Toronto (Canada); Moineddin, Rahim [University of Toronto, Department of Family and Community Medicine, Toronto (Canada)

    2008-08-15

    Even when histologic findings are highly suggestive of primary sclerosing cholangitis (PSC), cholangiographic correlation is required for the diagnosis. The present gold standard, endoscopic retrograde cholangiopancreatography (ERCP), is invasive and associated with complications. To evaluate the usefulness of magnetic resonance cholangiopancreatography (MRCP) in diagnosing PSC in children. MRCP studies were retrospectively reviewed in 20 children with clinical suspicion of PSC and who had undergone liver biopsy. MRCP studies were considered positive or negative for PSC depending on the presence or absence of dilatation, irregularity, multifocal strictures and beading of the bile ducts. Twenty children (14 boys, 6 girls) with an average age of 13 years qualified for the study. Of 19 diagnostic MRCP studies, 16 were called positive and 3 were called negative. An overall diagnosis of PSC-positive was assigned to all 19 patients based on clinical and laboratory findings, biopsy results and cholangiographic data. Based on this overall diagnosis, MRCP was 84% sensitive and accurate to diagnose PSC in children. MRCP can be a valuable tool in diagnosing pediatric PSC that can demonstrate major intra- and extrahepatic ducts in most cases. An unequivocally positive MRCP study should not be followed by ERCP for diagnosing PSC in children. (orig.)

  5. Pathogenesis and medical therapy of primary sclerosing cholangitis. Any news?

    NARCIS (Netherlands)

    van den Berg, A; Jansen, PLM

    1999-01-01

    Primary sclerosing cholangitis is characterized by inflammation and fibrosis of the intra- and extrahepatic bile ducts. Medical therapy has focused on anticholestatic, antiinflammatory and immunosuppressive drugs. Although inflammation, fibrosis and cholestasis may all occur at the same time, inflam

  6. Unusual Presentation of Primary Sclerosing Cholangitis

    Directory of Open Access Journals (Sweden)

    BC Pineau

    1997-01-01

    Full Text Available A 23-year-old man presenting with acute pancreatitis and autoimmune hemolytic anemia was diagnosed with primary sclerosing cholangitis (PSC without evidence of ulcerative colitis. This constellation of rare associations constitutes a unique mode of presentation of PSC. Within two years he also developed ankylosing spondylitis with sacroiliitis. Disordered immune regulation as a major factor in the mechanism of injury in PSC is supported by its increased association with other immunologically mediated disorders, most notably ulcerative colitis. Autoimmune hemolytic anemia, however, has been reported to be associated with PSC on only two occasions, and ankylosing spondylitis in the absence of ulcerative colitis is also unusual. In addition, the presentation of PSC with acute pancreatitis has rarely been described. This patient presented with several unusual features of PSC.

  7. Genetic epidemiology of primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    The aetiology of primary sclerosing cholangitis (PSC) is not known. A more than 80-fold increased risk of PSC among first-degree relatives emphasizes the importance of genetic factors. Genetic associations within the human leukocyte antigen (HLA) complex on chromosome 6p21 were detected in PSC 25 years ago. Subsequent studies have substantiated beyond doubt that one or more genetic variants located within this genetic region are important. The true identities of these variants, however,remain to be identified. Several candidate genes at other chromosomal loci have also been investigated. However,according to strict criteria for what may be denominated a susceptibility gene in complex diseases, no such gene exists for PSC today. This review summarises present knowledge on the genetic susceptibility to PSC, as well as genetic associations with disease progression and clinical subsets of particular interest (inflammatory bowel disease and cholangiocarcinoma).

  8. Extrahepatic Manifestations of Primary Biliary Cholangitis.

    Science.gov (United States)

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

    2017-03-16

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

  9. A case of cutaneous scleroderma with primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    H P Nandeesh

    2014-01-01

    Full Text Available Sclerosing cholangitis comprises of a spectrum of cholestatic conditions that are characterized by patchy fibrosis, inflammation and destruction of intra hepatic and extrahepatic ducts. We report a case of a 42 year old woman who presented with darkening of skin with yellowish discolouration of the eyes. Clinical examination revealed icterus, taut skin with hepatosplenomegaly. Liver function tests showed a cholestatic picture. Skin biopsy showed features of cutaneous scleroderma. MRCP and Liver biopsy was suggestive of sclerosing cholangitis.

  10. Resected case of eosinophilic cholangiopathy presenting with secondary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Fumihiko Miura; Takehide Asano; Hodaka Amano; Masahiro Yoshida; Naoyuki Toyota; Keita Wada; Kenichiro Kato; Tadahiro Takada; Junichi Fukushima; Fukuo Kondo; Hajime Takikawa

    2009-01-01

    Eosinophilic cholangiopathy is a rare condition characterized by eosinophilic infiltration of the biliary tract and causes sclerosing cholangitis. We report a patient with secondary sclerosing cholangitis with eosinophilic cholecystitis. A 46-year-old Japanese man was admitted to our hospital with jaundice.Computed tomography revealed dilatation of both the intrahepatic and extrahepatic bile ducts, diffuse thickening of the wall of the extrahepatic bile duct, and thickening of the gallbladder wall. Under the diagnosis of lower bile duct carcinoma, he underwent pyloruspreserving pancreatoduodenectomy and liver biopsy.On histopathological examination, conspicuous fibrosis was seen in the lower bile duct wall. In the gallbladder wall, marked eosinophilic infiltration was seen. Liver biopsy revealed mild portal fibrosis. He was diagnosed as definite eosinophilic cholecystitis with sclerosing cholangitis with unknown etiology. The possible etiology of sclerosing cholangitis was consequent fibrosis from previous eosinophilic infiltration in the bile duct. The clinicopathological findings of our case and a literature review indicated that eosinophilic cholangiopathy could cause a condition mimicking primary sclerosing cholangitis (PSC). Bile duct wall thickening in patients (PSC). Bile duct wall thickening in patients with eosinophilic cholangitis might be due to fibrosis of the bile duct wall. Eosinophilic cholangiopathy might be confused as PSC with eosinophilia.

  11. NEUTROPHIL CYTOPLASMIC AUTOANTIBODIES AFTER LIVER-TRANSPLANTATION IN PATIENTS WITH PRIMARY SCLEROSING CHOLANGITIS

    NARCIS (Netherlands)

    Haagsma, E.B.; MULDER, A.H.L.; Gouw, A.S.H.; MEERMAN, L.; Slooff, M.JH; Kallenberg, Cees; Horst, G.

    1993-01-01

    The immunopathogenic importance of neutrophil cytoplasmic autoantibodies in ulcerative colitis and primary sclerosing cholangitis is unknown. These autoantibodies were investigated before and after liver transplantation in 9 patients with primary sclerosing cholangitis. Sera from 10 patients transpl

  12. Primary sclerosing cholangitis - the Norwegian experience.

    Science.gov (United States)

    Schrumpf, Erik; Boberg, Kirsten Muri; Karlsen, Tom H

    2015-06-01

    Research related to primary sclerosing cholangitis (PSC) has since 1980 been a major activity at the Oslo University Hospital Rikshospitalet. The purpose of this publication is to describe the development of this research, the impact of this research on the clinical handling of the patients, and finally to describe what we believe are the most urgent, remaining problems to be solved. During the early years, our research dealt primarily with clinical aspects of the disease. The concomitant inflammatory bowel disease (IBD) seen in most patients with PSC was a major interest and we also started looking into genetic associations of PSC. Prognosis, malignancy development and treatment with special emphasis on transplantation have later been dealt with. These activities has had impact on several aspects of PSC management; when and how to diagnose PSC and variant forms of PSC, how to handle IBD in PSC and how to deal with the increased rate of malignancy? The problems remaining to be solved are many. What is the role of the gut and the gut microbiota in the development of PSC? Do the PSC patients have an underlying disturbance in the bile homeostasis? And how does the characteristic type of fibrosis in PSC develop? The genetic studies have supported a role for the adaptive immune system in the disease development, but how should this be dealt with? Importantly, the development of malignancy in PSC is still not understood, and we lack appropriate medical treatment for our patients.

  13. Primary sclerosing cholangitis: diagnosis, prognosis, and management.

    Science.gov (United States)

    Singh, Siddharth; Talwalkar, Jayant A

    2013-08-01

    Primary sclerosing cholangitis (PSC) is a chronic immune-mediated disease of the liver of unclear etiology, characterized by chronic inflammation and fibrosis of bile ducts. It primarily affects middle-aged men and is associated with 4-fold increased mortality as compared with an age- and sex-matched population. Progressive biliary and hepatic damage results in portal hypertension and hepatic failure in a significant majority of patients over a 10- to 15-year period from the initial diagnosis. In addition, PSC confers a markedly increased risk of hepatobiliary cancer, including cholangiocarcinoma and gallbladder cancer, as compared with the general population, and cancer is the leading cause of mortality in patients with PSC. It is associated with inflammatory bowel disease in 70% of patients and increases the risk of colorectal cancer almost 10-fold. Despite significant research efforts in this field, the pathogenic mechanisms of PSC are still incompletely understood, although growing evidence supports the role of genetic and immunologic factors. There are no proven medical therapies that alter the natural course of the disease. Thus, liver transplantation is the only available treatment for patients with advanced PSC, with excellent outcomes in this population.

  14. 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.

  15. Sclerosing cholangitis associated with autoimmune pancreatitis differs from primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Terumi Kamisawa; Kensuke Takuma; Hajime Anjiki; Naoto Egawa; Masanao Kurata; Goro Honda; Kouji Tsuruta

    2009-01-01

    AIM: To clarify the characteristic features of biliary lesions in patients with autoimmune pancreatitis (AIP) and compare them with those of primary sclerosing cholangitis (PSC). METHODS: The clinicopathological characteristics of 34 patients with sclerosing cholangitis (SC) associated with AIP were compared with those of 4 patients with PSC. RESULTS: SC with AIP occurred predominantly in elderly men. Obstructive jaundice was the most frequent initial symptom in SC with AIP. Only SC patients with AIP had elevated serum IgG4 levels, and sclerosing diseases were more frequent in these patients. SC patients with AIP responded well to steroid therapy. Segmental stenosis of the lower bile duct was observed only in SC patients with AIP, but a beaded and prunedtree appearance was detected only in PSC patients. Dense infiltration of IgG4-positive plasma cells was detected in the bile duct wall and the periportal area, as well as in the pancreas, of SC patients with AIP. CONCLUSION: SC with AIP is distinctly different from PSC. The two diseases can be discriminated based on cholangiopancreatographic findings and serum IgG4 levels.

  16. Falciform ligament abscess from left sided portal pyaemia following malignant obstructive cholangitis

    Directory of Open Access Journals (Sweden)

    Warren Leigh R

    2012-12-01

    left portal venous thrombosis, which was likely to be a delayed discovery and was managed conservatively. We present this patient’s operative images and radiographic findings, which may explain the pathophysiology behind this rare complication. We hypothesize that cholangitis, with secondary portal pyaemia and tracking via the paraumbilical veins, can cause infectious seeding of the falciform ligament, with consequent abscess formation.

  17. Diagnosis, Differential Diagnosis, and Epidemiology of Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Ponsioen, Cyriel Y

    2015-01-01

    According to recent guidelines, primary sclerosing cholangitis (PSC) is diagnosed when a patient has a cholestatic liver enzyme profile, characteristic bile duct changes on imaging, and when secondary causes of sclerosing cholangitis are excluded. In patients with a clinical suspicion but normal cholangiography, a liver biopsy is indicated to establish a diagnosis of small duct PSC. Several other disease entities such as IgG4-associated cholangitis (IAC), cholangiocarcinoma (CCA), and secondary causes of sclerosing cholangitis such as choledocholithiasis, AIDS-cholangiopathy, ischemia, surgical bile duct trauma, or mast cell cholangiopathy can mimic PSC. IAC can be differentiated from PSC by applying the HISORt criteria including the serum IgG4 level. In cases where serum IgG4 is less than 2 × ULN, the ratio of IgG4/IgG1 >0.24 is indicative for IAC. Choledocholithiasis with recurrent cholangitis as a cause of sclerosing cholangitis can pose a conundrum, since PSC itself is associated with an increased prevalence of gallstones. The epidemiology of PSC worldwide has been poorly described. Incidence and prevalence rates vary from 0-1.3 and 0-16.2 per 100,000 inhabitants respectively. However, these figures are not based on population-based cohorts. A recent large population-based cohort from the Netherlands reported an incidence of 0.5 and a prevalence of 6/100,000. Approximately 10% fulfil the criteria for small duct PSC. At diagnosis of PSC, concurrent inflammatory bowel disease (IBD), primarily ulcerative colitis or Crohn's colitis is present in 50%, but increasing to 80%, 10 years or more after diagnosis. Conversely, 3% of IBD patients will develop PSC. PSC predisposes to malignancy. The estimated cumulative risk of developing CCA after 30 years is 20%. For colorectal carcinoma in PSC/colitis patients, the estimated cumulative risk at 30 years is 13%.

  18. Destructive Cholangitis in an Adult Jack Russell Terrier

    Directory of Open Access Journals (Sweden)

    Atsushi Kodama

    2012-01-01

    Full Text Available A 4-year-old female Jack Russell terrier dog exhibited vomiting and severe jaundice of the visible mucous membranes and skin. Ultrasonography revealed diffuse areas of high echogenicity and focal areas of low echogenicity in the left lobe of the liver. On macroscopic observation of the biopsied liver specimen, many scattered irregularly shaped red spots were observed on the liver surface and on the cut surface. Histopathologically, there was loss of the interlobular bile duct and cholangitis accompanied by infiltration of pigment-laden macrophages in the Glisson’s capsule. Therefore, in the present case the dog was diagnosed with destructive cholangitis.

  19. A focus on acute cholecystitis and acute cholangitis

    Directory of Open Access Journals (Sweden)

    Massimo Sartelli

    2012-01-01

    Full Text Available Biliary infections are very common intra-abdominal infections. Laparoscopic cholecystectomy for acute cholecystitis and endoscopic retrograde management of acute cholangitis play important roles in the treatment of biliary infections. Also antimicrobial therapy is nevertheless important in the overall management of biliary infections. A multidisciplinary team of physicians, including surgeons trained in laparoscopic techniques, interventional gastroenterologists, and interventional radiologists may improve outcomes of patients with biliary infections. This review focuses the clinical presentation, diagnosis, and state of the art management of acute cholecystitis and acute cholangitis.

  20. A focus on acute cholecystitis and acute cholangitis

    Institute of Scientific and Technical Information of China (English)

    Massimo Sartelli; Cristian Tranà

    2012-01-01

    Biliary infections are very common intra-abdominal infections. Laparoscopic cholecystectomy for acute cholecystitis and endoscopic retrograde management of acute cholangitis play important roles in the treatment of biliary infections. Also antimicrobial therapy is nevertheless important in the overall management of biliary infections. A multidisciplinary team of physicians, including surgeons trained in laparoscopic techniques, interventional gastroenterologists, and interventional radiologists may improve outcomes of patients with biliary infections. This review focuses the clinical presentation, diagnosis, and state of the art management of acute cholecystitis and acute cholangitis.

  1. [CLINICAL CASE OF COMBINATION OF PRIMARY SCLEROSING CHOLANGITIS WITH NONSPECIFIC ULCERATIVE COLITIS IN TWINS MONOZYGOTIC].

    Science.gov (United States)

    Gubergrits, N B; Belyayeva, N V; Klochkov, A Ye; Fomenko, P G; Lukashevich, G M

    2015-01-01

    The article presents discussion of basic hypotheses of pathogenesis of primary sclerosing cholangitis (PSC): genetically conditioned pathology, autoimmune pathology, result of inflammatory reaction in bile ducts, cholangiopathy. The authors presents a clinical case of monozygotic twins with association of PSC and nonspecific ulcerative colitis (NUC). The first twin had a severe course of PSC and mild course of NUC; he died due to bacterial complications of cholangitis. The second twin--patient B--had an opposite situation: severe course of NUC, while PSC was suspected only after determination of cholestasis biochemical markers. As soon as cholestasis was revealed, patients B was treated with Ursofalk and Budenofalk (2001). He received Salofalk as a remedy of basic therapy for NUC. Repeated liver biopsy (2005) showed no progression of PSC, but there were present minimal biochemical signs of cholestasis. So, it is necessary to investigate the first degree relatives of patients with PSC. The timely administered treatment in some cases gives the possibility of the control of the disease course.

  2. Fungal arthritis

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000444.htm Fungal arthritis To use the sharing features on this page, please enable JavaScript. Fungal arthritis is swelling and irritation (inflammation) of a joint ...

  3. Fungal Meningitis

    Science.gov (United States)

    ... Schedules Preteen & Teen Vaccines Meningococcal Disease Sepsis Fungal Meningitis Language: English Español (Spanish) Recommend on Facebook Tweet ... the brain or spinal cord. Investigation of Fungal Meningitis, 2012 In September 2012, the Centers for Disease ...

  4. Traditional Chinese Medicine for Treating 22 Cases of Diabetes Complicated with Fungal Vaginitis%中药灌洗治疗糖尿病合并真菌性阴道炎22例

    Institute of Scientific and Technical Information of China (English)

    刘秀丽

    2015-01-01

    Objective Fungal vaginitis is a high incidence of disease among women, and the incidence of the disease is high. The aim of this study was to evaluate the diagnosis and treatment of Chinese traditional Chinese medicine for the treatment of diabetic patients with fungal vaginitis. Methods 22 cases of patients with diabetes mellitus complicated with fungal vaginitis in Fenyang hospital from January 2014 to January 2015 were studied. The concept, etiology, symptoms, di-agnosis and treatment of traditional Chinese medicine were summarized. Results The patients with diabetes mellitus com-plicated with fungal vaginitis were treated with the traditional Chinese medicine, and the patients were cured. Conclusion Traditional Chinese medicine in the treatment of diabetic patients with fungal vaginitis is very good, it is worth promoting.%目的 真菌性阴道炎是妇女群体中发生率较高的疾病,在糖尿病女性中发病的案例更是很高. 该研究的目的是对糖尿病合并真菌性阴道炎诊断和中药灌洗治疗的项目进行应用评价. 方法 选取2014年1月—2015年1月山西省汾阳医院的22例糖尿病合并真菌性阴道炎患者为研究对象,总结糖尿病合并真菌性阴道炎的概念、病因、症状及其诊断和中药灌洗治疗的疗效. 结果 采用中药灌洗的治疗方法后糖尿病合并真菌性阴道炎患者的病情逐渐得到控制并痊愈. 结论 中药灌洗治疗糖尿病合并真菌性阴道炎的疗效很好,值得推广.

  5. Immune-mediated diseases in primary sclerosing cholangitis

    NARCIS (Netherlands)

    Lamberts, Laetitia E.; Janse, Marcel; Haagsma, Elizabeth B.; van den Berg, Arie P.; Weersma, Rinse K.

    2011-01-01

    Background: Primary sclerosing cholangitis is a chronic cholestatic liver disease. An immune aetiology is suggested by associations between PSC and inflammatory bowel disease. Data on concomitant prevalence of other immune-mediated diseases is limited. Aim: To assess the prevalence of concomitant im

  6. Novel developments in IBD-related sclerosing cholangitis

    NARCIS (Netherlands)

    C.Y. Ponsioen

    2011-01-01

    Primary sclerosing cholangitis is often regarded as an autoimmune disorder and occurs frequently in relation to inflammatory bowel disease. The ongoing fibro-obliterative process of the biliary tree ensues in liver failure or cholangiocarcinoma in 12-18 years. PSC patients with concurrent IBD are at

  7. Secondary sclerosing cholangitis in a critically ill patient.

    Science.gov (United States)

    Weiss, Krista E; Willmann, Juergen K; Jeffrey, R Brooke; Desser, Terry S

    2016-04-01

    Critically ill patients are commonly imaged for liver dysfunction. An often fatal condition, secondary sclerosing cholangitis, is an important and likely under-recognized hepatic condition in these patients. In presenting this case report, we hope to raise awareness of this condition amongst radiologists as well as other physicians caring for the critically ill.

  8. Immune-mediated diseases in primary sclerosing cholangitis

    NARCIS (Netherlands)

    Lamberts, Laetitia E.; Janse, Marcel; Haagsma, Elizabeth B.; van den Berg, Arie P.; Weersma, Rinse K.

    2011-01-01

    Background: Primary sclerosing cholangitis is a chronic cholestatic liver disease. An immune aetiology is suggested by associations between PSC and inflammatory bowel disease. Data on concomitant prevalence of other immune-mediated diseases is limited. Aim: To assess the prevalence of concomitant

  9. Distinctive inflammatory bowel disease phenotype in primary sclerosing cholangitis

    NARCIS (Netherlands)

    de Vries, A. Boudewijn; Janse, Marcel; Blokzijl, Hans; Weersma, Rinse K.

    2015-01-01

    AIM: To review the current literature for the specific clinical characteristics of inflammatory bowel disease (IBD) associated with primary sclerosing cholangitis (PSC). METHODS: A systematical review for clinical characteristics of IBD in PSC was performed by conducting a broad search for "primary

  10. Genome-Wide Association Analysis in Primary Sclerosing Cholangitis

    NARCIS (Netherlands)

    T.H. Karlsen; A. Franke; E. Melum; A.. Kaser; J.R. Hov; T. Balschun; B.A. Lie; A. Bergquist; C. Schramm; T.J. Weismüller; D. Gotthardt; C. Rust; E.E.R. Philipp; T. Fritz; L. Henckaerts; R. Weersma; P. Stokkers; C.Y. Ponsioen; C. Wijmenga; M. Sterneck; M. Nothnagel; J. Hampe; A. Teufel; H. Runz; P. Rosenstiel; A. Stiehl; S. Vermeire; U. Beuers; M. Manns; E. Schrumpf; K.M. Boberg; S. Schreiber

    2010-01-01

    BACKGROUND & AIMS: We aimed to characterize the genetic susceptibility to primary sclerosing cholangitis (PSC) by means of a genome-wide association analysis of single nucleotide polymorphism (SNP) markers. METHODS: A total of 443,816 SNPs on the Affymetrix SNP Array 5.0 (Affymetrix, Santa Clara, CA

  11. Clinical efficacy of cyclophosphamide in treatment of primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    程鹏

    2014-01-01

    Objective To investigate the clinical efficacy of cy-clophosphamide in the treatment of primary sclerosing cholangitis(PSC).Methods Twenty-four patients with PSC who received treatment in the department of gastroenterology in our hospital from January 2004 to December2012 were selected as subjects and divided into observation group(n=13)and control

  12. Primary sclerosing cholangitis and disease distribution in inflammatory bowel disease.

    LENUS (Irish Health Repository)

    O'Toole, Aoibhlinn

    2012-04-01

    The relationship between site of intestinal inflammation and primary sclerosing cholangitis (PSC) development in inflammatory bowel disease (IBD) has not been studied extensively, but may be important in understanding the pathogenesis of PSC. We aimed to determine patterns of disease distribution in IBD patients with and without PSC.

  13. (Post-)genomics approaches in fungal research

    NARCIS (Netherlands)

    Aguilar-Pontes, María Victoria; de Vries, Ronald P; Zhou, M.; van den Brink, J.

    2014-01-01

    To date, hundreds of fungal genomes have been sequenced and many more are in progress. This wealth of genomic information has provided new directions to study fungal biodiversity. However, to further dissect and understand the complicated biological mechanisms involved in fungal life styles, functio

  14. New insights into autoimmune cholangitis through animal models.

    Science.gov (United States)

    Trauner, Michael; Fickert, Peter; Baghdasaryan, Anna; Claudel, Thierry; Halilbasic, Emina; Moustafa, Tarek; Wagner, Martin; Zollner, Gernot

    2010-01-01

    Improving our understanding of the pathogenesis of chronic immune-mediated cholangiopathies such as primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), as well as the development of novel diagnostic, prognostic and therapeutic tools for these disorders critically depends on easily reproducible animal models. Recently, several spontaneous mouse models for PBC (not requiring previous manipulations for breakdown of immunotolerance) have been reported, including NOD.c3c4 and NOD.c3c4-derived mice, IL-2Ralpha(-/-) mice, dominant negative TGF-beta receptor II mice and Ae2(a,b)(-/-) mice. To date, no animal model exhibits all of the attributes of PSC. Rodent models induced by bacterial cell components or colitis may help to explain the strong association between PSC and inflammatory bowel disease. Other models include direct injury to biliary epithelia, peribiliary vascular endothelia or portal venous endothelia. Mice with targeted disruption of the Mdr2 (Abcb4) gene encoding a canalicular phospholipid flippase (Mdr2(-/-) mice) spontaneously develop sclerosing cholangitis with macroscopic and microscopic features of human PSC. Another example for a transporter involved in the pathogenesis of sclerosing cholangitis is the cystic fibrosis transmembrane conductance regulator (CFTR/ABCC7). Xenobiotics and drugs may also lead to bile duct injury and biliary fibrosis via direct toxic and indirect immune-mediated injury. Hydrophobic bile acids, such as lithocholic acid, cause bile duct injury and destructive cholangitis with periductal fibrosis resembling sclerosing cholangitis. These models have enhanced our understanding of the pathogenesis of PBC and PSC and will hopefully result in improved treatment of these disorders.

  15. Costs of conservative treatment of patients with cholangitis and cholesystitis

    Directory of Open Access Journals (Sweden)

    Mikić Sandra Stefan

    2014-01-01

    Full Text Available Introduction. There are no guidelines for treating bacterial infections in our country. Antibacterial drugs are selected empirically, which is not always in accordance with the recommended therapy coming from the countries that have their own pharmacotherapy protocols. This study reviews the differences between the standard conservative treatment of cholangitis/cholecystitis and treatment implemented in accordance with current pharmacotherapy guidelines of other countries. The assessment of the therapeutic efficacy of antibacterial drugs implemented in the treatment of cholangitis/cholecystitis and economic viability of that implementation was one of the aims of this study, and another one was comparison of the efficiency and cost of medical treatment using physician’s therapy of choice and treatment according to pharmacotherapy guidelines. Material and Methods. The study was carried out over a threeyear period at the Department of Infectious Diseases of the Clinical Center of Vojvodina. It included 72 patients with the diagnosis of cholangitis/cholecystitis who were divided into two groups. The control group was treated according to the physician’s therapy of choice, while the test group was treated in accordance with pharmacotherapy guidelines. The efficiency and cost of medical treatment were determined for both groups. Results. The comparison of the efficiency and costs of medical treatment of cholangitis/cholecystitis before and after introducing pharmacotherapy guidelines showed no significant differences in the efficiency of administered therapies. However, the cost of medical treatment carried out in accordance with pharmacotherapy guidelines showed a decrease of 17.24%. Conclusion. Medical treatment using physician’s antibacterial therapy of choice and the one which is in accordance with pharmacotherapy guidelines for treating cholangitis/cholecystitis show no differences in terms of efficiency. However, medical treatment proved

  16. Significance of peribiliary oedema on computed tomography in diagnosis and severity assessment of acute cholangitis

    Energy Technology Data Exchange (ETDEWEB)

    Akaike, Gensuke [Department of Radiology, St. Luke' s International Hospital (Japan); Ishiyama, Mitsutomi, E-mail: issie1009@yahoo.co.jp [Department of Radiology, St. Luke' s International Hospital (Japan); Suzuki, Shoko [Department of Gastroenterology, Japan Red Cross Musashino Hospital (Japan); Fujita, Yoshiyuki [Department of Gastroenterology, St. Luke' s International Hospital (Japan); Ohde, Sachiko [Center for Clinical Epidemiology, St. Luke' s Life Science Institute (Japan); Saida, Yukihisa [Department of Radiology, St. Luke' s International Hospital (Japan)

    2013-09-15

    Objectives: To evaluate usefulness of peribiliary oedema on computed tomography (CT) in diagnosing acute cholangitis and assessing its severity. Materials and methods: Sixty patients (male 59%, mean age 67.3 years) who underwent endoscopic retrograde biliary drainage (ERBD) for suspected biliary obstruction within 6 h after contrast-enhanced CT were evaluated. Two radiologists performed a consensus evaluation of CT for the presence of peribiliary oedema. Patients were divided into the cholangitis group and the non-cholangitis group based on clinical and ERBD findings, and CT results were compared between the two groups. In the cholangitis group, laboratory values and blood culture results were compared between those with and without peribiliary oedema. Chi-squared test was used for analyses. Results: Of 60 enrolled patients, there were 46 patients in the cholangitis group and 14 patients in the non-cholangitis group. Peribiliary oedema was seen in 24/46 (52.2%) patients in the cholangitis group and 3/14 (23.3%) patients in the non-cholangitis group (p = 0.043). In the cholangitis group, positive blood culture was seen in 12/24 (50%) patients with peribiliary oedema and 4/22 (18.1%) patients without it (p = 0.03). Conclusions: Peribiliary oedema appears to be useful for diagnosis and severity assessment of acute cholangitis.

  17. Successful Treatment of Primary Sclerosing Cholangitis with a Steroid and a Probiotic

    Directory of Open Access Journals (Sweden)

    Masaki Shimizu

    2012-05-01

    Full Text Available Primary sclerosing cholangitis (PSC is a serious disease that not only affects quality of life but can also have a significant effect on patient survival. The treatment for PSC is primarily supportive with the aim of controlling cholestatic symptoms and preventing complications. Ursodeoxycholic acid may induce biochemical improvements in affected patients; however, long-term pediatric studies to determine its possible benefits in young patients are lacking. Thus, the treatment of pediatric PSC remains a significant clinical challenge. We describe a patient with PSC and undetermined colitis who was treated with a combination of a steroid, salazosulfapyridine, and a probiotic. This treatment provided benefits both for PSC and the undetermined colitis. These findings suggest that bacterial flora and gut inflammation are closely associated with the pathogenesis of inflammatory bowel disease-related PSC. Suppression of bowel inflammation and maintenance of bacterial homeostasis may be important for treating PSC.

  18. Congenital double bile duct presenting as recurrent cholangitis in a child

    Directory of Open Access Journals (Sweden)

    K.D. Chakravarty

    2015-12-01

    Full Text Available Double common bile duct (DCBD is a rare congenital anomaly. Most of these bile duct anomalies are associated with bile duct stones, anomalous pancreaticobiliary junction (APBJ, pancreatitis and bile duct or gastric cancers. Early detection and treatment is important to avoid long term complications. Surgical resection of the anomalous bile duct and reconstruction of the biliary enteric anastomosis is the treatment of choice. We report a rare case of DCBD anomaly in a girl, who presented with recurrent cholangitis. She had type Va DCBD anomaly. She underwent successful resection of the bile duct and reconstruction of the biliary enteric anastomosis. Preoperative imaging and diagnosis of the congenital biliary anomaly is very important to avoid intraoperative bile duct injury. Review of the literature shows very few cases of type Va DCBD, presenting with either bile duct stones or APBJ.

  19. Primary sclerosing cholangitis-What is the difference between east and west?

    Institute of Scientific and Technical Information of China (English)

    Ali Shorbagi; Yusuf Bayraktar

    2008-01-01

    Primary sclerosing cholangitis (PSC) is a chronic, progressive, cholestatic liver disease characterized by inflammation and fibrotic obliteration of the hepatic biliary tree. It is commonly associated with inflammatory bowel disease (IBD). A number of complications can occur which require special consideration, the most important of which is the development of cholangiocellular carcinoma (CCC). Unfortunately, no medical therapy is currently available for the underlying liver disease. Liver transplantation is an effective, life-extending option for patients with advanced PSC. Geographical variations between East and West include a second peak for age with a lower association with IBD in a Japanese population and female predominance in a lone study from Turkey. The clinical and biochemical Mayo criteria may not be universally applicable, as different patients show variations regarding the initial presentation and natural course of the disease. Directing research towards explaining these geographical differences and understanding the pathogenesis of PSC is required in order to develop better therapies for this devastating disease.

  20. [Fungal keratitis].

    Science.gov (United States)

    Bourcier, T; Sauer, A; Letscher-Bru, V; Candolfi, E

    2011-10-01

    Fungal keratitis (keratomycosis) is a rare but severe cause of infectious keratitis. Its incidence is constant, due to steroids or immunosuppressive treatments and contact lenses. Pathogens often invade corneas with chronic diseases of the ocular surface but fungal keratitis is also observed following injuries with plant foreign objects. The poor prognosis of these infections is related both to fungal virulence, decreased host defense, as well as delays in diagnosis. However, new antimycotic treatments allow better management and prognosis.

  1. Characterization of animal models for primary sclerosing cholangitis (PSC).

    Science.gov (United States)

    Fickert, Peter; Pollheimer, Marion J; Beuers, Ulrich; Lackner, Carolin; Hirschfield, Gideon; Housset, Chantal; Keitel, Verena; Schramm, Christoph; Marschall, Hanns-Ulrich; Karlsen, Tom H; Melum, Espen; Kaser, Arthur; Eksteen, Bertus; Strazzabosco, Mario; Manns, Michael; Trauner, Michael

    2014-06-01

    Primary sclerosing cholangitis (PSC) is a chronic cholangiopathy characterized by biliary fibrosis, development of cholestasis and end stage liver disease, high risk of malignancy, and frequent need for liver transplantation. The poor understanding of its pathogenesis is also reflected in the lack of effective medical treatment. Well-characterized animal models are utterly needed to develop novel pathogenetic concepts and study new treatment strategies. Currently there is no consensus on how to evaluate and characterize potential PSC models, which makes direct comparison of experimental results and effective exchange of study material between research groups difficult. The International Primary Sclerosing Cholangitis Study Group (IPSCSG) has therefore summarized these key issues in a position paper proposing standard requirements for the study of animal models of PSC.

  2. IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis

    Directory of Open Access Journals (Sweden)

    Allon Kahn

    2015-01-01

    Full Text Available IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.

  3. Xanthogranulomatous cholangitis causing obstructive jaundice: A case report

    Institute of Scientific and Technical Information of China (English)

    Susumu Kawate; Susumu Ohwada; Hayato Ikota; Kunihiro Hamada; Kenji Kashiwabara; Yasuo Morishita

    2006-01-01

    This article reports the case of a 34-year-old woman with xanthogranulomatous cholangitis who developed obstructive jaundice. Microscopically, the bile duct was surrounded and narrowed by a xanthogranulomatous lesion, but no xanthogranulomatous cholecystitis was seen. Although percutaneous cholangiograms done via the transhepatic biliary drainage showed smooth narrowing of the upper to middle bile duct, the cytology of bile was diagnosed as class V adenocarcinoma.Therefore, right extended hepatectomy and extrahepatic bile duct resection were performed. The differentiation of benign and malignant strictures at the hepatic hilum is often difficult. Xanthogranulomatous cholangitis is one possible diagnosis of a bile duct stricture. Precise review of all the preoperative information is required to make a correct diagnosis.

  4. Liver Hydatid Cyst and Acute Cholangitis: a Case Report.

    Science.gov (United States)

    Nemati Honar, Behzad; Hayatollah, Gholamhossein; Nikshoar, Mohammadreza; Forootan, Mojgan; Feizi, Ali Mohammad

    2016-04-01

    Amongst the cause of cystic hepatic disease, hydatid cyst is common in the Asia, South America, and Africa. The definitive therapy for hepatic hydatid disease is surgical resection. Rupture of the hydatid cyst into the biliary tree can lead to serious cholangitis. In this report, a 22-year-old man is presented with the signs and symptoms of obstructive jaundice and cholangitis. Ultrasonography reported dilated common bile duct (CBD) with sludge and stones, a hydatid cyst adjacent to the gall bladder and mild thickening of gallbladder wall without a stone. MRCP revealed dilated CBD with a cyst in segment fifth of liver. Due to signs and symptoms of obstructive jaundice in addition to lab data and imaging modalities, the ruptured hydatid cyst into a biliary tree was considered, and surgical intervention was performed to extract daughter vesicles from the CBD. Post intervention, signs and symptoms and cholestasis enzymes were subsided.

  5. Biliary Drainage Method and Temporal Trends in Patients Admitted with Cholangitis: A National Audit

    Directory of Open Access Journals (Sweden)

    Julia McNabb-Baltar

    2013-01-01

    Full Text Available BACKGROUND: In patients presenting with ascending cholangitis, better outcomes are reported in those undergoing endoscopic retrograde cholangiopancreatography (ERCP compared with surgical drainage.

  6. Autoimmune cholangitis mimicking a klatskin tumor: a case report

    Directory of Open Access Journals (Sweden)

    Zwirewich Charles

    2011-09-01

    Full Text Available Abstract Introduction Autoimmune cholangitis remains an elusive manifestation of immunoglobulin G4-associated systemic disease most commonly encountered in patients with autoimmune pancreatitis. No strict diagnostic criteria have been described to date and diagnosis mainly relies on a combination of clinical and histopathologic findings. It is hence even more challenging to diagnose autoimmune cholangitis in patients with late or atypical presentations, such as without concomitant pancreatic involvement. Early diagnosis of this rare disorder can significantly improve outcomes considering high rates of surgical intervention, as well as high relapse rates in the absence of steroid treatment. To the best of our knowledge the literature is quite sparse on cases with atypical presentations of autoimmune cholangitis. Case presentation We report a case of a previously healthy 65-year-old man of Middle-Eastern origin, with a history of pancreatic insufficiency of unknown etiology, evaluated for elevated liver function tests found incidentally on a routine physical examination. Imaging studies revealed an atrophic pancreas and biliary duct dilatation consistent with obstruction. Subsequent endoscopic retrograde cholangiopancreatography showed a bile duct narrowing pattern suggestive of cholangiocarcinoma, but brushings failed to reveal malignant cells. Our patient proceeded to undergo surgical resection. Histological examination of the resected mass revealed lymphoplasmacytic infiltrate with no malignant features. Our patient returned three months later with persistently high liver function tests and no evidence of biliary obstruction on imaging. A presumptive diagnosis of autoimmune cholangitis was made and our patient's symptoms resolved after a short course of an oral steroid regimen. Post factum staining of the resection specimen revealed an immunoglobulin G4 antibody positive immune cell infiltrate, consistent with the proposed diagnosis. Conclusion

  7. Inflammatory bowel disease of primary sclerosing cholangitis: a distinct entity?

    Science.gov (United States)

    Nakazawa, Takahiro; Naitoh, Itaru; Hayashi, Kazuki; Sano, Hitoshi; Miyabe, Katsuyuki; Shimizu, Shuya; Joh, Takashi

    2014-03-28

    This is a review of the characteristic findings of inflammatory bowel disease (IBD) associated with primary sclerosing cholangitis (PSC) and their usefulness in the diagnosis of sclerosing cholangitis. PSC is a chronic inflammatory disease characterized by idiopathic fibrous obstruction and is frequently associated with IBD. IBD-associated with PSC (PSC-IBD) shows an increased incidence of pancolitis, mild symptoms, and colorectal malignancy. Although an increased incidence of pancolitis is a characteristic finding, some cases are endoscopically diagnosed as right-sided ulcerative colitis. Pathological studies have revealed that inflammation occurs more frequently in the right colon than the left colon. The frequency of rectal sparing and backwash ileitis should be investigated in a future study based on the same definition. The cholangiographic findings of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are similar to those of PSC. The rare association between IBD and IgG4-SC and the unique characteristics of PSC-IBD are useful findings for distinguishing PSC from IgG4-SC.

  8. Recurrent pyogenic cholangitis : angiographic findings and its significance

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Young Chan; Kim, Eui Jong; Oh, Joo Hyung; Yoon, Yup; Lim, Joo Won; Kim, Ihn Sub [Kyunghee Univ. Hospital, Seoul (Korea, Republic of)

    1997-07-01

    To analyse the angiographic findings of recurrent pyogenic cholangitis Hepatic arteriography and portography were performed preoperatively in 34 patients with intrahepatic stones and recurrent cholangitis. Twenty five of these underwent partial resection of the liver and in nine, the biliary tract was drained. Hepatic arteriogram and portogram findings correlated with liver atrophy and were analysed retrospectively by two radiologists;angiographic and pathologic findings also correlated. In the arterial phase, abnormal stained areas, were seen in 17 of 34 cases, periarterial staining in 14, mass-like staining in two and a mixed pattern in one. The hepatic artery showed spastic change in 11 of 34 cases and tortuous change in 17. Arteriovenous shunting was not seen. In the portal phase, abnormal findings of the portal veins were noted in 16 cases; decreased size and nonvisualization were seen in eleven patients, and decreased size only, in five. Fifteen cases showed liver atrophy;in 13 of these, portal vein abnormalities were also present. In recurrent pyogenic cholangitis, angiographic findings may be normal or findings of abnormal periarterial staining, mass-like staining, spastic and tortuous change of the hepatic artery, and abnormal portal vein can be present. The differential diagnosis of hepatitis, hepatic mass and cirrhosis should be considered.

  9. Clogging and other complications of endoscopic biliary endoprostheses

    DEFF Research Database (Denmark)

    Dowidar, N; Moesgaard, F; Matzen, Peter

    1991-01-01

    signs of cholangitis occurred in 15% of the patients, whereas other late complications were rather infrequent. Although 10-french endoprostheses had a significantly longer patency period than 7-french--that is, 6 months versus 2 months (p = 0.01)--the proportion of patients in whom clogging occurred...

  10. Fungal rhinosinusitis.

    Science.gov (United States)

    Netkovski, J; Shirgoska, B

    2012-01-01

    Fungi are a major part of the ecosystem. In fact, over 250 fungal species have been reported to produce human infections. More than ever, fungal diseases have emerged as major challenges for physicians and clinical microbiologists. The aim of this study was to summarize the diagnostic procedures and endoscopic surgical treatment of patients with fungal rhinosinusitis. Eleven patients, i.e. 10% of all cases with chronic inflammation of paranasal sinuses, were diagnosed with fungal rhinosinusitis. Ten of them were patients with a noninvasive form, fungus ball, while only one patient was classified in the group of chronic invasive fungal rhinosinusitis which was accompanied with diabetes mellitus. All patients underwent nasal endoscopic examination, skin allergy test and had preoperative computed tomography (CT) scans of the sinuses in axial and coronal plane. Functional endoscopic sinus surgery was performed in 10 patients with fungus ball, while a combined approach, endoscopic and external, was done in the immunocompromised patient with the chronic invasive form of fungal rhinosinusitis. Most cases (9/11) had unilateral infection. In 9 cases infection was restricted to a single sinus, and here the maxillary sinus was most commonly affected (8/9) with infections in other patients being restricted to the sphenoid sinus (1/9). Two patients had infections affecting two or more sinuses. In patients with an invasive form of the fungal disease there was involvement of the periorbital and orbital tissues. In patients with fungus ball the mycelia masses were completely removed from the sinus cavities. Long-term outcome was positive in all the operated patients and no recurrence was detected. The most frequent fungal agent that caused rhinosinusitis was Aspergillus. Mucor was identified in the patient with the invasive form. Endoscopic examination of the nasal cavity and CT scanning of paranasal sinuses followed by endoscopic sinus surgery were represented as valuable

  11. Analysis of risk factors of patients with chronic liver failure complicated invasive fungal infections%慢性肝衰竭患者侵袭性真菌感染的危险因素分析

    Institute of Scientific and Technical Information of China (English)

    胡耀仁; 胡爱荣

    2009-01-01

    Objective To evaluate the risk factors of chronic liver failure(CLF) complicated invnsive fungal infections(IFI) and prevention and treatment. Methods The clinical data and risk factors of 52 patients with CLF complicated IFI were analyzed retrospectively and were compared with those not complicated IFI. Risk factors were analyzed by chi-square test and Logistic regression test and Ridit test. Results In 52 patients with CLF complicated IFI, there were 69 fungal infections in different tissue and organs, the most were in oral cavity, but other tissue and organs especially bellows infections were rising. Candida albieans infeefions were the most, but cryptococcus neoformans infections and aspergillus infections were rising. The risk factors included species of bacteria infections, serum total bilimbin, hospital days, times of antibiotics using, number of invasive operation,species of antibiotics and degrees of aseites. The mortality of patients with CLF complicated IFI were much higher than those not complicated IFI. Conclusion Patients with CLF complicated IFI have poor progress and prognosis. The effective prevent methods are treating primary disease actively, reducing hospital days, detecting patients' body fluids closely, identifying source of infection as early as possible, using antibiotics correctly, reducing or avoiding invasive operation, using immunoactivators and disinfecting air regularly.%目的 探讨慢性肝衰竭(Chronic liver failure,CLF)患者合并侵袭性真菌感染(Invnsivefunsal infections,IFl)的危险因素及防治措施.方法 回顾性分析52例CLF合并IFI患者的I临床特点、危险因素以及预后,并与随机选取同期住院的52例CLF未合并真菌感染患者作为对照.结果 52例真菌感染者发生了69例次不同部位感染,感染部位虽然以浅部口腔为主,但是其他部位感染有上升趋势,尤其是肺部感染;感染真菌种属虽然仍以白色念珠菌为主,但是新型隐

  12. Fungal allergens.

    OpenAIRE

    1995-01-01

    Airborne fungal spores occur widely and often in far greater concentrations than pollen grains. Immunoglobulin E-specific antigens (allergens) on airborne fungal spores induce type I hypersensitivity (allergic) respiratory reactions in sensitized atopic subjects, causing rhinitis and/or asthma. The prevalence of respiratory allergy to fungi is imprecisely known but is estimated at 20 to 30% of atopic (allergy-predisposed) individuals or up to 6% of the general population. Diagnosis and immuno...

  13. Immunoglobulin G4-Associated Cholangitis: One Variant of Immunoglobulin G4-Related Systemic Disease

    NARCIS (Netherlands)

    Y.A. Alderlieste; B.D.J. van den Elzen; E.A.J. Rauws; U. Beuers

    2009-01-01

    IgG4-associated cholangitis (IAC) is a recently defined disease entity which shares a number of clinical, biochemical, and radiological features with primary sclerosing cholangitis (PSC). In contrast to PSC, IAC responds to immunosuppressive treatment, is not associated with inflammatory bowel disea

  14. Primary sclerosing cholangitis in patients with ulcerative colitis: two case reports

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, Se Hwan; Lee, Dong Ho; Choi, Dong Sik; Ko, Young Tae [Kyunghee University Hospital, Seoul (Korea, Republic of)

    1999-02-01

    Primary sclerosing cholangitis, a chronic cholestatic liver disease, is uncommon and is characterized by inflammation and fibrosis of the bile ducts. It frequently occurs in association with ulcerative colitis. We describe two cases of primary sclerosing cholangitis in patients with ulcerative colitis.

  15. The complicated duodenal diverticulum: retrospective analysis of 11 cases.

    Science.gov (United States)

    de Perrot, Thomas; Poletti, Pierre-Alexandre; Becker, Christoph D; Platon, Alexandra

    2012-01-01

    A series of rare complicated duodenal diverticula were reported with emphasis on causes for misdiagnosis. Patients with a discharge diagnosis of complicated duodenal diverticulum were retrospectively obtained. Computed tomographic (CT) reports and findings were reviewed. Complications consisted of diverticulitis (n=2), perforation (n=7), or obstructive cholangitis (n=2). CT imaging demonstrated a duodenal diverticular structure with findings due to the kind of complications. At the time of CT interpretation, a complicated duodenal diverticulum was suspected in 5 out of 11 patients. Awareness of the duodenal diverticulum and complications may improve the diagnostic value of CT in this setting. Copyright © 2012 Elsevier Inc. All rights reserved.

  16. Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings

    Energy Technology Data Exchange (ETDEWEB)

    Itoh, S., E-mail: shigekimiyo@luck.ocn.ne.j [Department of Technical Radiology, Nagoya University School of Health Sciences, Nagoya (Japan); Nagasaka, T. [Department of Pathology, Nagoya University Graduate School of Medicine, Nagoya (Japan); Suzuki, K.; Satake, H.; Ota, T.; Naganawa, S. [Department of Radiology, Nagoya University Graduate School of Medicine, Nagoya (Japan)

    2009-11-15

    Aim: To assess the clinical, computed tomography (CT), and pathological findings in patients with lymphoplasmacytic sclerosing cholangitis. Materials and methods: Fifteen consecutive patients (four women and 11 men, mean age 71 years) with lymphoplasmacytic sclerosing cholangitis and without the characteristic features of underlying disorders causing benign biliary strictures were retrospectively recruited. Two radiologists evaluated multiphase contrast-enhanced CT images acquired with 0.5 or 1-mm collimation. One pathologist performed all histological examinations, including IgG4 immunostaining. Results: The intrahepatic biliary ducts showed dilatation in all 15 patients, but only seven presented with jaundice. Although laboratory data were not available in all patients, serum gammaglobulin and IgG levels were elevated in five of six patients and six of eight patients, respectively. Anti-nuclear antibody was detected in three of six patients. The involved biliary ducts showed the following CT findings: involvement of the hilar biliary duct (14/15), a mean wall thickness of 4.9 mm, a smooth margin (10/15), a narrow but visible lumen (6/15), hyper-attenuation during the late arterial phase (9/15), homogeneous hyper-attenuation during the delayed phase (11/11), and no vascular invasion (14/15). Abnormal findings in the pancreas and urinary tract were detected in eight of 15 patients. In 13 patients with adequate specimens, moderate to severe lymphoplasmacytic infiltration associated with dense fibrosis was observed. Infiltration of IgG4-positive plasma cells was moderate or severe in nine patients and minimal or absent in four patients. Conclusion: Lymphoplasmacytic sclerosing cholangitis exhibits relatively characteristic clinical and CT findings, although they are not sufficiently specific for differentiation from other biliary diseases.

  17. Fungal nail infection

    Science.gov (United States)

    Nails - fungal infection; Onychomycosis; Infection - fungal - nails; Tinea unguium ... hair, nails, and outer skin layers. Common fungal infections include: Athlete's foot Jock itch Ringworm on the ...

  18. Sclerosing cholangitis: cholescintigraphy with Tc-99m-labeled DISIDA

    Energy Technology Data Exchange (ETDEWEB)

    Ament, A.E.; Bick, R.J.; Miraldi, F.D.; Haaga, J.R.; Wiedenmann, S.D.

    1984-04-01

    Four patients with primary sclerosing cholangitis (PCS) were examined with the hepatobiliary agent Tc-99m-labeled DISIDA (diisopropylphenylcarbamoyl iminodiacetic acid), and the results correlated with those of invasive cholangiography. Cholescintigraphy is superior to cholangiography in cases of suspected PSC where there is nonfilling of biliary radicals due to high-grade stenosis. The finding of delayed hepatic parenchymal clearance can allow estimation of the degree of obstruction of the various branches of the major bile ducts. Cholescintigraphy offers a noninvasive method of investigating patients with suspected sclerosing chloangitis, leading to earlier diagnosis. Confirmation with invasive cholangiographic procedures is recommended.

  19. Cancer Risk and Surveillance in Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Folseraas, Trine; Boberg, Kirsten Muri

    2016-02-01

    Primary sclerosing cholangitis (PSC) is a chronic, progressive disease characterized by inflammatory and fibrosing strictures of the biliary tree. PSC is associated with a high lifetime risk of hepatobiliary and colorectal cancers. The nature of the carcinogenic process in PSC is not well established. The lack of diagnostic methods for early detection and the limited therapeutic options for cholangiocarcinoma constitute a major challenge in the current handling of PSC patients. The article reviews the risk for cancer development in PSC and discusses surveillance strategies for PSC-associated cancers.

  20. Role of the Microbiota and Antibiotics in Primary Sclerosing Cholangitis

    Directory of Open Access Journals (Sweden)

    James H. Tabibian

    2013-01-01

    Full Text Available Primary sclerosing cholangitis (PSC is an idiopathic, progressive, cholestatic liver disease with considerable morbidity and mortality and no established pharmacotherapy. In addition to the long-recognized association between PSC and inflammatory bowel disease, several lines of preclinical and clinical evidence implicate the microbiota in the etiopathogenesis of PSC. Here we provide a concise review of these data which, taken together, support further investigation of the role of the microbiota and antibiotics in PSC as potential avenues toward elucidating safe and effective pharmacotherapy for patients afflicted by this illness.

  1. Autoimmune pancreatitis associated with primary sclerosing cholangitis: MR imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Eerens, I.; Vanbeckevoort, D.; Van Hoe, L. [University Hospital, Leuven (Belgium). Dept. of Radiology; Vansteenbergen, W. [Dept. of Hepatology, University Hospitals KU, Leuven (Belgium)

    2001-08-01

    Autoimmune pancreatitis is a relatively rare type of chronic pancreatitis that may be associated with other autoimmune disorders. The imaging features of this entity may be misleading and suggest the presence of a malignant tumour. We present a case in which MR imaging allowed us to diagnose autoimmune pancreatitis associated with primary sclerosing cholangitis, which is another autoimmune-related disease. Typical MR characteristics of autoimmune pancreatitis include focal or diffuse enlargement of the pancreas, the absence of parenchymal atrophy and significant dilation proximal to the site of stenosis, the absence of peripancreatic spread, the clear demarcation of the lesion and the presence of a peripancreatic rim. (orig.)

  2. Ulcerative Colitis associated with Sclerosing Cholangitis and Autoimmune Hepatitis

    Directory of Open Access Journals (Sweden)

    Hoduț Andrei

    2013-04-01

    Full Text Available Introduction: Ulcerative colitis is a chronic intestinal inflammation, part of inflammatory bowel disease, which also includes Crohn’s disease. Both have extraintestinal manifestations, but those that tend to occur more commonly with ulcerative colitis include chronic active hepatitis, pyoderma gangrenosum and ankylosing spondylitis. Many individuals present with overlapping non-diagnostic features of more than one of these conditions that is referred to in the literature as autoimmune overlap syndrome. Sclerosing cholangitis associated with IBD is often referred to as overlap syndrome.

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

    Science.gov (United States)

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

    2016-02-01

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

  4. [The method of biliary tracts drainage of patients with biliodigestive anastomosis at obstructive jaundice and acute cholangitis].

    Science.gov (United States)

    Agaev, B A; Agaev, R M; Gasymov, R Sh

    2011-01-01

    The internal draining of biliary tracts of patients with obstructive jaundice and acute cholangitis is considered most acceptable, but deficiency of this method is anastomosis failure, stricture and purulent-septic complications hazard. The investigations performed on 79 patients with obstructive jaundice, 36 of which were included in control group, 43 - in main group. To patients of main group with internal draining intraoperatively through the nose and biliodigestive anastomosis into the biliary tract transmitted tube with quartz optical monofiber for endocholedocheal laser irradiation in one lumen and hollow other, serve for drainage and irrigation of bile-excreting way with ozonized physiological solution. The application of endocholedocheal laser radiation and ozonetherapy in combination with suggested at biliodigestive anastomosis biliary tract drainage method allowed to earlier elimination of inflammatory process biliary tract, correct the bile lipids per oxidations disturbances and elevate activity of antioxidant protection system, to reduced the time of patients stay at hospital after surgery period from 20,3±1,24 day in control group to 12,7±0,653 day in main group (pjaundice and acute cholangitis.

  5. Fungal keratitis

    Directory of Open Access Journals (Sweden)

    Sonal S Tuli

    2011-02-01

    Full Text Available Sonal S TuliUniversity of Florida, Gainesville, FL, USA  Clinical question: What is the most appropriate management of fungal keratitis?Results: Traditionally, topical Natamycin is the most commonly used medication for filamentous fungi while Amphotericin B is most commonly used for yeast. Voriconazole is rapidly becoming the drug of choice for all fungal keratitis because of its wide spectrum of coverage and increased penetration into the cornea.Implementation: Repeated debridement of the ulcer is recommended for the penetration of topical medications. While small, peripheral ulcers may be treated in the community, larger or central ulcers, especially if associated with signs suggestive of anterior chamber penetration should be referred to a tertiary center. Prolonged therapy for approximately four weeks is usually necessary.Keywords: fungal keratitis, keratomycosis, antifungal medications, debridement

  6. Immunoglobulin G4-related cholangitis: a variant of IgG4-related systemic disease.

    Science.gov (United States)

    Novotný, Ivo; Dítě, Petr; Trna, Jan; Lata, Jan; Husová, Libuše; Geryk, Edvard

    2012-01-01

    IgG4-related sclerosing cholangitis as part of IgG4 systemic-related diseases is commonly associated with autoimmune pancreatitis. Major clinical manifestations of IgG4-related sclerosing diseases are apparent in the organs in which tissue fibrosis with obstructive phlebitis is pathologically induced. IgG4-related sclerosing cholangitis is included within the heterogeneous group of 'sclerosing cholangitis'. Sclerosing cholangitis may be associated with choledocholithiasis, infection or biliary malignancies. Sclerosing cholangitis of unknown etiology is called primary sclerosing cholangitis (PSC). Conservative therapy of PSC is usually unsuccessful, the disease involves extra- and/or intrahepatic biliary tree, and the end point of this disease is liver cirrhosis. Typically, PSC is identified at the age of 30 to 40 years, and the disease is frequently associated with inflammatory bowel diseases. On the other hand, IgG4-related sclerosing cholangitis is not associated with inflammatory bowel diseases. In patients with IgG4-related sclerosing cholangitis, a first symptom can be obstructive jaundice, whereas obstructive jaundice is rarely present in PSC. Clinically, patients with IgG4-related sclerosing cholangitis are older at diagnosis compared to patients with PSC. A typical diagnostic feature of IgG4-related sclerosing cholangitis is elevation of serum immunoglobulin G4. In patients with IgG4-related sclerosing cholangitis, response to steroid therapy is high; in patients with PSC corticosteroid therapy is unsuccessful. Histochemically abundant infiltration of IgG4-positive plasma cells is detected in the biliary duct wall. Histologically, we can identify dense lymphoplasmacytic infiltration of the bile duct wall, transmural fibrosis, lymphoplasmacytic infiltration and fibrosis in the periportal area of the liver - a typically obliterative phlebitis. The biliary epithelium is usually intact in contrast to PSC, where mucosal erosion is often present. Steroids are the

  7. 1例房颤合并真菌感染患者室性心动过速诱因分析%Inducement Analysis of 1 Case of Ventricular Tachycardia in Patient with Atrial Fibrillation Complicated with Fungal Infection

    Institute of Scientific and Technical Information of China (English)

    郑重践; 林妙娴

    2014-01-01

    To analyze the cause of ventricular tachycardia in patient with atrial fibrillation complicated with fungal infection during the course of treatment.An increased risk of arrhythmia will be caused by many factors, such as hypokalemia, fluconazole combined with ami-odarone, et al.However, greater risk will be caused by a combination of amiodarone and fluconazole (0.4 g・ d-1 ).Great attention should be paid to the dosage of fluconazole under the necessary combination of fluconazole and amiodarone.%分析1例房颤合并真菌感染患者治疗过程中出现室性心动过速的诱因。可能导致本例室性心律失常发生的因素有低钾血症、氟康唑与胺碘酮联用,但氟康唑加量至0.4 g・ d -1时联合使用胺碘酮风险更大。临床在不可避免将氟康唑与胺碘酮联用时,应注意氟康唑的给药剂量。

  8. 熊去氧胆酸对胆道微结石性胆管炎的预防价值探讨%Value of ursodeoxycholic acid for preventing recurrence of bile microlithiasis-associated cholangitis

    Institute of Scientific and Technical Information of China (English)

    胡雅兵; 沈云志; 沈璐

    2010-01-01

    Objective To explore clinical strategies of treating in bile microlithiasis-associated cholangitis and preventing the recurrence of ursodeoxycholic acid(UDCA).Methods Forty-one cases of bile microlthiasis-associated cholangitis from January 2006 to July 2007 Were analyzed and followed up.All patients underwent endoscopic therapy who were divided into UDCA group(21 cases)and non-UDCA group (20 cases)according to the use of UDCA or not besides endoscopic therapy.After using UDCA for 12 months,the complications and the incidence rate of recurrent cholangitis in each group were compared.Results Forty-one cases had no serious complications after endoscopic therapy.None had recurrent cholangitis in UDCA group,4 cases in non-UDCA group got recurrent cholangitis.One case developed pancreatitis.There was statistical significance between the two groups(P=0.021).Conclusion Endoscopic therapy is a safe and effective treatment of bile microlithiasis-associated cholangitis,and UDCA can prevent the recurrent cholangitis effectively after endoscopic therapy.%目的 探讨胆道微结石性胆管炎内镜治疗效果与熊去氧胆酸预防复发的作用.方法 对2006年1月至2007年7月确诊的41例胆道微结石性胆管炎患者的临床资料进行分析、随访.所有患者均行内镜治疗,根据内镜治疗后是否服用熊去氧胆酸将患者随机分为单纯内镜治疗组(20例)和内镜治疗+熊去氧胆酸组(21例).随访12个月比较两组胆管炎复发率.结果 41例患者经内镜治疗后未发生严重并发症.随访12个月,单纯内镜治疗组中,4例胆管炎复发,1例发生胰腺炎;内镜治疗+熊去氧胆酸组中无胆管炎复发,两组比较差异有统计学意义(P=0.021.结论 内镜治疗胆道微结石性胆管炎是-安全有效的方法,术后服用熊去氧胆酸可预防胆管炎复发.

  9. Fungal prions.

    Science.gov (United States)

    Staniforth, Gemma L; Tuite, Mick F

    2012-01-01

    For both mammalian and fungal prion proteins, conformational templating drives the phenomenon of protein-only infectivity. The conformational conversion of a protein to its transmissible prion state is associated with changes to host cellular physiology. In mammals, this change is synonymous with disease, whereas in fungi no notable detrimental effect on the host is typically observed. Instead, fungal prions can serve as epigenetic regulators of inheritance in the form of partial loss-of-function phenotypes. In the presence of environmental challenges, the prion state [PRION(+)], with its resource for phenotypic plasticity, can be associated with a growth advantage. The growing number of yeast proteins that can switch to a heritable [PRION(+)] form represents diverse and metabolically penetrating cellular functions, suggesting that the [PRION(+)] state in yeast is a functional one, albeit rarely found in nature. In this chapter, we introduce the biochemical and genetic properties of fungal prions, many of which are shared by the mammalian prion protein PrP, and then outline the major contributions that studies on fungal prions have made to prion biology.

  10. Fungal Entomopathogens

    Science.gov (United States)

    Fungal entomopathogens are important biological control agents worldwide and have been the subject of intense research for more than100 years. They exhibit both sexual and asexual reproduction and produce different types of infective propagules. Their mode of action against insects involves attachme...

  11. [PRIMARY SCLEROSING CHOLANGITIS ASSOCIATED TO ULCERATIVECOLITIS AS A CAUSE OF CIRRHOSIS

    Science.gov (United States)

    Tagle, Martín; Bussalleu, Alejandro

    1999-01-01

    We report case of a peruvian patient with cirrhosis due to primary sclerosing cholangitis, associated with ulcerative colitis. The patient presented initially with intermittent diarrhea, manifesting features of chronic liver failure which progressed rapidly.Primary sclerosing cholangitis is a progressive diasease affecting intra and extrahepatic billary radicles, and is associated with ulcerative colitis in the majority of cases. The diagnosis is made by cholangiography, showing areas of stenosis, irregularity and dilatation of the biliary tree.In this article we report the clinical presentation and course of the patient, reviewing recent literature with emphasis in the association between primary sclerosing cholangitis and ulcerative colitis.

  12. Recurrent gastric ulcer and cholangitis caused by ectopic drainage of the bile duct into the stomach

    Directory of Open Access Journals (Sweden)

    Bauer, Katrin

    2017-08-01

    Full Text Available We report a case of recurrent gastritis with pyloric stenosis and cholangitis due to a rare variation in bile duct anatomy. A 72-year-old female patient showed recurrent gastral ulcers and biliary colic with cholangitis caused by gallstones in the main bile duct with an ectopic orifice in the prepyloric region and concurrent inflammatory pyloric stenosis. After temporarily successful endoscopic treatment with stenting and pyloric dilatation, the patient suffered from recurrent cholangitis. Finally, the abnormal biliary anatomy required surgical treatment with biliodigestive anastomosis.

  13. 急性白血病并肺部真菌感染57例早期诊断与防治策略%Early Diagnosis,Treatment and Prevention of Childhood Acute Leukemia Complicated with Pulmonary Fungal Infection

    Institute of Scientific and Technical Information of China (English)

    邵静波; 蒋慧; 李红; 陆正华; 杨静薇; 杨为群

    2011-01-01

    Objective To analyze the clinical features and investigate the early diagnosis and treatment of childhood acute leukemia complicated with pulmonary fungal infection (PFI). Methods The clinical data of 132 cases of childhood acute leukemia were retrospectively analyzed. Fifty -seven patients, who had the PFI,including 5 times with confirmed diagnosis,30 times with clinical diagnosis,and 25 times with recommended diagnosis were diagnosed according to European Organization for Research and Treatment of Cancer. Results 1. The incidence rate of PFI in the childhood acute leukemia was 43.18% (57/132 cases). The median time from occurring PFI to confirmed leukemia was 7.2 months,among which 70% (42/60 cases) were within 12 months while 30% ( 18/60 cases) more than 12 months. 2. The representation of chest CT was multiform,mainly for inflammatory exudate,interstitial changes,and cloud flocculeut nodules. The severe patients were accompanied by pleural effusion or acute respiratory distress syndrome(ARDS). 3. Seventy -one point seven percent(43/60 cases)of PFI were neutropenia ( < 1.5 × 109 L-1 ) ,in which 40.0% were ngranulocytosis ( < 0. 5 × 109L-1 ). 4. The positive rate of fungal G test was 70.5% ( 31/44 cases) wille negative was 29.5% ( 13/44 cases). Totally 7 candida strains and 2 mierozyme strains were obtained from 57 cases of PFI. 5. The total cure rate was 95.0%. Cure rates of single drug and combined anti fungal were 53.3% and 41.7%. Conclusions 1. Fungal infection is one of the main pathogens in childhood acute leukemia complicated by severe infection. 2. Chest CT shows important value in the diagnosis of PFI. The positive rate of microorganism culture is low in children. 3. Early diagnosis and treatment are the key to improve curative effect. Better prognosis can be gained if those patients with early respiratory failure or ARDS get oxygen or mechanical ventilation in time.%目的 分析急性白血病并肺部真菌感染(PFI)患儿的

  14. Motion – Patients with Primary Sclerosing Cholangitis Should Undergo Early Liver Transplantation: Arguments for the Motion

    Directory of Open Access Journals (Sweden)

    Young-Mee Lee

    2002-01-01

    Full Text Available Primary sclerosing cholangitis (PSC is a condition of unknown etiology that causes progressive inflammation, fibrosis and obliteration of the intrahepatic and extrahepatic biliary tree. There is no medical cure, and ursodeoxycholic acid and other drugs have not been shown to affect the natural history of the disease. Endoscopic dilation is of value only in the relief of symptoms and complications related to dominant strictures. Cholangiocarcinoma occurs in a substantial minority of cases, especially those with ulcerative colitis and cirrhosis, and is often not clinically apparent before surgery. There are no good serologic tests for early cancers. Because this tumour has such a dismal prognosis, some authorities recommend that liver transplantation be undertaken before its development. This procedure is the only curative option for PSC, and excellent survival rates have been reported. There is evidence that early transplantation, before end stage liver disease or cholangiocarcinoma have developed, improves the survival and quality of life of patients with PSC. Because it is the only procedure of proven benefit, patients with PSC should be considered for liver transplantation early in the course of the disease.

  15. The Role of Genetic and Immune Factors for the Pathogenesis of Primary Sclerosing Cholangitis in Childhood

    Science.gov (United States)

    Campos Silva, Soraya Luiza; Marques de Miranda, Débora; Ferreira, Alexandre Rodrigues

    2016-01-01

    Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by chronic inflammation of the biliary tree resulting in liver fibrosis. PSC is more common in male less than 40 years of age. The diagnosis of PSC is based on clinical, laboratory, image, and histological findings. A biochemical profile of mild to severe chronic cholestasis can be observed. Endoscopic retrograde cholangiography is the golden standard method for diagnosis, but magnetic resonance cholangiography is currently also considered a first-line method of investigation. Differences in clinical and laboratory findings were observed in young patients, including higher incidence of overlap syndromes, mostly with autoimmune hepatitis, higher serum levels of aminotransferases and gamma-glutamyl transferase, and lower incidence of serious complications as cholangiocarcinoma. In spite of the detection of several HLA variants as associated factors in large multicenter cohorts of adult patients, the exact role and pathways of these susceptibility genes remain to be determined in pediatric population. In addition, the literature supports a role for an altered immune response to pathogens in the pathogenesis of PSC. This phenomenon contributes to abnormal immune system activation and perpetuation of the inflammatory process. In this article, we review the role of immune and genetic factors in the pathogenesis of PSC in pediatric patients. PMID:27882046

  16. Biliary strictures and recurrence after liver transplantation for primary sclerosing cholangitis: A retrospective multicenter analysis.

    Science.gov (United States)

    Hildebrand, Tatiana; Pannicke, Nadine; Dechene, Alexander; Gotthardt, Daniel N; Kirchner, Gabriele; Reiter, Florian P; Sterneck, Martina; Herzer, Kerstin; Lenzen, Henrike; Rupp, Christian; Barg-Hock, Hannelore; de Leuw, Philipp; Teufel, Andreas; Zimmer, Vincent; Lammert, Frank; Sarrazin, Christoph; Spengler, Ulrich; Rust, Christian; Manns, Michael P; Strassburg, Christian P; Schramm, Christoph; Weismüller, Tobias J

    2016-01-01

    Liver transplantation (LT) is the only definitive treatment for patients with end-stage liver disease due to primary sclerosing cholangitis (PSC), but a high rate of biliary strictures (BSs) and of recurrent primary sclerosing cholangitis (recPSC) has been reported. In this multicenter study, we analyzed a large patient cohort with a long follow-up in order to evaluate the incidence of BS and recPSC, to assess the impact on survival after LT, and to identify risk factors. We collected clinical, surgical, and laboratory data and records on inflammatory bowel disease (IBD), immunosuppression, recipient and graft outcome, and biliary complications (based on cholangiography and histology) of all patients who underwent LT for PSC in 10 German transplant centers between January 1990 and December 2006; 335 patients (68.4% men; mean age, 38.9 years; 73.5% with IBD) underwent transplantation 8.8 years after PSC diagnosis with follow-up for 98.8 months. The 1-, 5-, and 10-year recipient and graft survival was 90.7%, 84.8%, 79.4% and 79.1%, 69.0%, 62.4%, respectively. BS was diagnosed in 36.1% after a mean time of 3.9 years, and recPSC was diagnosed in 20.3% after 4.6 years. Both entities had a significant impact on longterm graft and recipient survival. Independent risk factors for BS were donor age, ulcerative colitis, chronic ductopenic rejection, bilirubin, and international normalized ratio (INR) at LT. Independent risk factors for recPSC were donor age, IBD, and INR at LT. These variables were able to categorize patients into risk groups for BS and recPSC. In conclusion, BS and recPSC affect longterm graft and patient survival after LT for PSC. Donor age, IBD, and INR at LT are independent risk factors for BS and recPSC and allow for risk estimation depending on the recipient-donor constellation.

  17. Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel disease

    Institute of Scientific and Technical Information of China (English)

    Rebecca Saich; Roger Chapman

    2008-01-01

    Primary sclerosing cholangitis (PSC) is a chronic progressive disorder of unknown aetiology characterised by chronic inflammation and stricture formation of the biliary tree. Symptoms include itch and lethargy and in advanced cases cholangitis and end-stage liver disease, however increasing numbers of asymptomatic individuals are being identified. The disease is rare in the general population but is strongly associated with inflammatory bowel disease (IBD) affecting up to 5% of patients with Ulcerative Colitis, with a slightly lower prevalence (up to 3.6%) in Crohns disease. The strength of this association means that the vast majority (> 90%) of patients with PSC also have IBD, although many may have only mild gastro-intestinal symptoms. Usually IBD presents before PSC, although vice-versa can occur and the onset of both conditions can be separated in some cases by many years. Mean age of diagnosis of PSC is in the fifth decade of life with a strong male predominance.Risk is increased in those with a family history of PSC, suggesting a genetic predisposition and the disease is almost exclusive to non-smokers. The ulcerative colitis associated with PSC is characteristically mild, runs a quiescent course, is associated with rectal sparing, more severe right sided disease, backwash ilieitis and has a high risk of pouchiUs post-colectomy. Most worrisome is the high risk of colorectal malignancy which necessitates routine colonoscopic surveillance. Cholangiocarcinoma is also a frequent complication of PSC with a 10%-15% lifetime risk of developing this condition. Treatment with high dose ursodeoxycholic acid offers some chemoprotective effects against colorectal malignancy and may decrease symptoms, biochemical and histological progression of liver disease. Small duct PSC patients characteristically have normal cholangiography, and liver biopsy is required for diagnosis, it appears to have a more favourable prognosis. Autoimmune Hepatitis (AIH) is also more prevalent

  18. MMP-2 is a disease-modifying gene in primary sclerosing cholangitis

    NARCIS (Netherlands)

    Korkmaz, Kerem Sebib; de Rooij, Bert-Jan F.; van Hoek, Bart; Janse, Marcel; Coenraad, Minneke J.; van der Reijden, Johan J.; Weersma, Rinse K.; Porte, Robert J.; Voorneveld, Philip W.; Baranski, Andrzej G.; Verspaget, Hein W.

    2014-01-01

    BackgroundPrimary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the bile ducts, frequently necessitating orthotopic liver transplantation (OLT), often accompanied by inflammatory bowel disease (IBD). Matrix metalloproteinases (MMPs) are associated with fibrotic diseases caused by

  19. Severe acute cholangitis after endoscopic sphincterotomy induced by barium examination: A case report

    Institute of Scientific and Technical Information of China (English)

    Zhen-Hai Zhang; Ya-Guang Wu; Cheng-Kun Qin; Zhong-Xue Su; Jian Xu; Guo-Zhe Xian; Shuo-DongWu

    2012-01-01

    Endoscopic sphincterotomy (EST) is considered as a possible etiological factor for severe cholangitis.We herein report a case of severe cholangitis after endoscopic sphincterotomy induced by barium examination.An adult male patient presented with epigastric pain was diagnosed as having choledocholithiasis by ultrasonography.EST was performed and the stone was completely cleaned.Barium examination was done 3d after EST and severe cholangitis appeared 4 h later.The patient was recovered after treated with tienam for 4 d.Barium examination may induce severe cholangitis in patients after EST,although rare,barium examination should be chosen cautiously.Cautions should be also used when EST is performed in patients younger than 50 years to avoid the damage to the sphincter of Oddi.

  20. Primary sclerosing cholangitis – The arteriosclerosis of the bile duct?

    Directory of Open Access Journals (Sweden)

    Trauner Michael

    2007-01-01

    Full Text Available Abstract Primary sclerosing cholangitis (PSC is a chronic inflammatory disease of unknown aetiology affecting the large bile ducts and characterized by periductal fibrosis and stricture formation, which ultimately result in biliary cirrhosis and liver failure. Arteriosclerosis involves the accumulation of altered lipids and lipoproteins in large arteries; this drives inflammation and fibrosis and ultimately leads to narrowing of the arteries and hypoperfusion of dependent organs and tissues. Knowledge of the causative factors is crucial to the understanding of disease mechanisms and the development of specific treatment. Based on pathogenetic similarities between PSC and arteriosclerosis, we hypothesize that PSC represents "arteriosclerosis of the bile duct" initiated by toxic biliary lipids. This hypothesis is based on common molecular, cellular, and morphological features providing the conceptual framework for a deeper understanding of their pathogenesis. This hypothesis should stimulate translational research to facilitate the search for novel treatment strategies for both diseases.

  1. Management of Primary Sclerosing Cholangitis: Conventions and Controversies

    Directory of Open Access Journals (Sweden)

    Natasha Chandok

    2012-01-01

    Full Text Available Primary sclerosing cholangitis (PSC is a chronic inflammatory cholangiopathy that results in fibrotic strictures and dilations of the intra- and extrahepatic bile ducts. PSC is uncommon, occurs predominantly in males and has a strong association with inflammatory bowel disease. While the pathogenesis of PSC has not been fully elucidated, emerging evidence supports roles for the innate and adaptive immune systems, and genome-wide analyses have identified several genetic associations. Using the best available evidence, the present review summarizes the current understanding of the diagnosis, pathogenesis and management of PSC. Despite its rarity, there is an urgent need for collaborative research efforts to advance therapeutic options for PSC beyond liver transplantation.

  2. Recurrence and rejection in liver transplantation for primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Bjarte Fosby; Tom H Karlsen; Espen Melum

    2012-01-01

    Primary sclerosing cholangitis (PSC) is a chronic progressive inflammatory disease affecting the bile ducts, leading to fibrosis and eventually cirrhosis in most patients. Its etiology is unknown and so far no effective medical therapy is available. Liver transplantation (LTX) is the only curative treatment and at present PSC is the main indication for LTX in the Scandinavian countries. Close to half of the PSC patients experience one or more episodes of acute cellular rejection (ACR) following transplantation and approximately 1/5 of the transplanted patients develop recurrent disease in the graft. In addition, some reports indicate that ACR early after LTX for PSC can influence the risk for recurrent disease. For these important post-transplantation entities affecting PSC patients, we have reviewed the current literature on epidemiology, pathogenesis, treatment and the possible influence of rejection on the risk of recurrent disease in the allograft.

  3. Primary sclerosing cholangitis: Updates in diagnosis and therapy

    Institute of Scientific and Technical Information of China (English)

    Piero Portincasa; Michele Vacca; Antonio Moschetta; Michele Petruzzelli; Giuseppe Palasciano; Karel J. van Erpecum; Gerard P. van Berge-Henegouwen

    2005-01-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic syndrome of unknown origin mostly found in males, and characterized by diffuse inflammation and fibrosis of both intra- and extra-hepatic bile ducts. So far, PSC is considered as an autoimmune hepatobiliary disease. In most cases the progression of PSC towards liver cirrhosis and liver failure is slow but irreversible, and liver transplantation is currently the only definitive treatment. In recent years,PSC has been an area of active research worldwide with great interest in etiology, pathogenesis, diagnosis, and therapeutic options such as hydrophilic ursodeoxycholic acid and immunosuppressive agent tacrolimus. Recent updates on clinical and therapeutic aspects of PSC are discussed in the present review.

  4. Primary sclerosing cholangitis associated with inflammatory bowel disease: an update.

    Science.gov (United States)

    Rossi, Roberta E; Conte, Dario; Massironi, Sara

    2016-02-01

    Primary sclerosing cholangitis (PSC) is a chronic progressive disease, usually associated with underlying inflammatory bowel diseases (IBDs), with a prevalence of 60-80% in western countries. Herein, we review the current knowledge about the association between PSC and IBD in terms of clinical approach and long-term patient management. A PubMed search was conducted for English-language publications from 2000 through 2015 using the following keywords: primary sclerosing cholangitis, inflammatory bowel disease, ulcerative colitis, Crohn's disease, diagnosis, therapy, follow-up, and epidemiology. In terms of diagnosis, liver function tests and histology are currently used. The medical treatment options for PSC associated with IBD do not differ from the cases of PSC alone, and include ursodeoxycholic acid and immunosuppressive agents. These treatments do not seem to improve survival, even if ursodeoxycholic acid given at low doses may be chemopreventive against colorectal cancer (CRC). Liver transplantation is the only potential curative therapy for PSC with reported survival rates of 85 and 70% at 5 and 10 years after transplant; however, there is a risk for PSC recurrence, worsening of IBD activity, and de-novo IBD occurrence after liver transplantation. PSC-IBD represents an important public health concern, especially in view of the increased risk for malignancy, including CRC. Long-life annual surveillance colonoscopy is usually recommended, although the exact timescale is still unclear. Further studies are required both to clarify whether annual colonoscopy is cost-effective, especially in younger patients, and to identify potential pharmaceutical agents and genetic targets that may retard disease progression and protect against CRC.

  5. Recurrence of primary sclerosing cholangitis in pediatric liver transplant recipients.

    Science.gov (United States)

    Venkat, Veena L; Ranganathan, Sarangarajan; Mazariegos, George V; Sun, Qing; Sindhi, Rakesh

    2014-06-01

    There is little detailed clinical information on recurrent primary sclerosing cholangitis (rPSC) after liver transplantation in children. Our purpose was to describe the characteristics of children who had experienced rPSC after liver transplantation so that we could identify potential risk factors for recurrence. Clinical information for pediatric patients undergoing transplantation for primary sclerosing cholangitis (PSC) was retrospectively reviewed, and variables related to the pretransplant diagnosis of PSC and posttransplant variables were abstracted. The studied variables included the following: cytomegalovirus/Epstein-Barr virus status, early/late rejection, induction regimen, immunosuppression in the first year, steroid-resistant rejection, diagnosis of inflammatory bowel disease, and human leukocyte antigen markers commonly associated with PSC. A diagnosis of rPSC was made on the basis of radiographic features, histology, or both. Twelve patients underwent liver transplantation for PSC between 1993 and 2012. Patients received tacrolimus for maintenance immunosuppression after induction with steroids (n = 6) or thymoglobulin (n = 6). Three patients were diagnosed with rPSC 44, 60, and 62 months after transplantation. A fourth patient underwent retransplantation for graft failure with features of both hepatic artery stenosis and rPSC. This patient had distinct histological features of rPSC in the second graft. Three of the 4 patients were 7 years old or younger at the diagnosis of PSC. The patient and graft survival rates were similar for the steroid and thymoglobulin groups. All 4 children with rPSC received steroid-free thymoglobulin induction. In conclusion, our observation of an association between thymoglobulin, and age less than 10 years at the diagnosis of PSC, and rPSC adds to the existing suggestion of a link between the immune environment and the pathogenesis of rPSC. Defining the natural history of rPSC and searching for the etiology and

  6. Urgent ERCP for acute cholangitis reduces mortality and hospital stay in elderly and very elderly patients

    Institute of Scientific and Technical Information of China (English)

    Chan Sun Park; Hee Seok Jeong; Ki Bae Kim; Joung-Ho Han; Hee Bok Chae; Sei Jin Youn; Seon Mee Park

    2016-01-01

    BACKGROUND: Acute cholangitis in old people is a cause of mortality and prolonged hospital stay. We evaluated the effects of methods and timing of biliary drainage on the outcomes of acute cholangitis in elderly and very elderly patients. METHODS: We analyzed 331 patients who were older than 75 years and were diagnosed with acute calculous cholangitis. They were admitted to our hospital from 2009 to 2014. Pa-tients’ demographics, severity grading, methods and timing of biliary drainage, mortality, and hospital stay were retrospec-tively obtained from medical records. Clinical parameters and outcomes were compared between elderly (75-80 years,n=156) and very elderly (≥81 years,n=175) patients. We analyzed the effects of methods [none, endoscopic retrograde cholangio-pancreatography (ERCP), percutaneous transhepatic biliary drainage, or failure] and timing (urgent or early) of biliary drainage on mortality and hospital stay in these patients. RESULTS: Acute cholangitis in older patients manifested as atypical symptoms characterized as infrequent Charcot’s triad (4.2%) and comorbidity in one-third of the patients. Patients were graded as mild, moderate, and severe cholangitis in 104 (31.4%), 175 (52.9%), and 52 (15.7%), respectively. Urgent bili-ary drainage (≤24 hours) was performed for 80.5% (247/307) of patients. Very elderly patients tended to have more severe grades and were treated with sequential procedures of tran-sient biliary drainage and stone removal at different sessions. Hospital stay was related to methods and timing of biliary drainage. Mortality was very low (1.5%) and not related to patient age but rather to the success or failure of biliary drainage and severity grading of the acute cholangitis. CONCLUSIONS: The methods and timing used for biliary drainage and severity of cholangitis are the major determi-nants of mortality and hospital stay in elderly and very elderly patients with acute cholangitis. Urgent successful ERCP is mandatory for

  7. Sclerosing cholangitis with autoimmune pancreatitis versus primary sclerosing cholangitis: comparison on endoscopic retrograde cholangiography, MR cholangiography, CT, and MRI

    Energy Technology Data Exchange (ETDEWEB)

    Kim; Jin Hee; Byun, Jae Ho; Kim, So Yeon; Lee, Seung Soo; Kim, Hyoung Jung; Lee, Moon-Gyu [Dept. of Radiology and Research Inst. of Radiology, Univ. of Ulsan Coll. of Medicine, Asan Medical Center, Seoul (Korea, Republic of)], e-mail: jhbyun@amc.seoul.kr; Kim, Myung-Hwan [Dept. of Internal Medicine, Univ. of Ulsan Coll. of Medicine, Asan Medical Center, Seoul (Korea, Republic of)

    2013-07-15

    Background: It is essential to differentiate sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) from primary sclerosing cholangitis (PSC) as the treatment and prognosis of the two diseases are totally different. Purpose: To compare image findings of SC-AIP and PSC on endoscopic retrograde cholangiography (ERC), magnetic resonance cholangiography (MRC), computed tomography (CT), and magnetic resonance imaging (MRI). Material and Methods: Two radiologists retrospectively reviewed ERC, MRC, CT, and MRI in 28 SC-AIP and 23 PSC patients in consensus. Factors evaluated included the length, location, and multiplicity of bile duct stricture, the presence of characteristic cholangiographic features of PSC on ERC and MRC, and the presence, location, thickness, and pattern of bile duct wall thickening on CT and MRI. Results: On ERC, focal stricture, multifocal and intrahepatic bile duct stricture, and beaded, pruned-tree, and diverticulum-like appearance were more frequent in PSC than in SC-AIP patients (P = 0.006). On MRC, multifocal and intrahepatic bile duct stricture and pruned-tree appearance were more frequent in PSC than in SC-AIP patients (P = 0.044). On CT and MRI, the bile duct wall was thicker (5.1 mm vs. 3.1 mm; P = 0.033 and 4.3 mm vs. 3.0 mm; P = 0.01, respectively) in SC-AIP than in PSC patients. PSC was more frequently associated with intrahepatic bile duct wall thickening on both CT (93% vs. 50%; P = 0.024) and MRI (100% vs. 50%; P = 0.023) than SC-AIP. Conclusion: The combination of ERC or MRC with cross-sectional images, including CT and MRI, may be helpful in differentiating between SC-AIP and PSC.

  8. Fungal Eye Infections

    Science.gov (United States)

    ... Treatment & Outcomes Statistics More Resources Fungal Nail Infections Histoplasmosis Definition Symptoms People at Risk & Prevention Sources Diagnosis & ... CDC at Work Global Fungal Diseases Cryptococcal Meningitis Histoplasmosis ... Resistance Resources Laboratory Submission Information Reportable Fungal ...

  9. Fungal infection following renal transplantation.

    Science.gov (United States)

    Gallis, H A; Berman, R A; Cate, T R; Hamilton, J D; Gunnells, J C; Stickel, D L

    1975-09-01

    Twenty-seven deep fungal infections developed in 22 of 171 patients following renal transplantation. These infections included cryptococcosis (ten), nocardiosis (seven), candidiasis (four), aspergillosis (two), phycomycosis (two), chromomycosis (one), and subcutaneous infection with Phialophora gougeroti (one). Twelve infections occurred in living-related and ten in cadaveric recipients. Nineteen of the 22 patients were male. Infections occurred from 0 to 61 months after transplantation. Complicating non-fungal infections were present concomitantly in 15 patients. Thirteen patients died, eight probably as a result of fungal infection. Appropriate diagnostic procedures yielded a diagnosis in 20 of 27 infections, and therapy was begun in 18 patients. Serologic, culture, and biopsy procedures useful in making rapid diagnoses are advocated in the hope of increasing survival.

  10. Screening for malignancy in primary sclerosing cholangitis (PSC).

    Science.gov (United States)

    Khaderi, Saira A; Sussman, Norman L

    2015-04-01

    Primary sclerosing cholangitis (PSC) is a frequently progressive and fatal disease. Death from cancer occurs in a significant subset of patients with PSC. Patients with PSC have a 10 to 15 % lifetime risk of developing cholangiocarcinoma (CCA). About one third of CCAs are present in the first year after a diagnosis of PSC; the remainder are present with a frequency of about 1.5 % each year. Patients with concomitant PSC and inflammatory bowel disease (IBD) have a 4-fold higher risk of colorectal cancer (CRC) than patients with IBD alone and a 10-fold higher risk of CRC than the general population. The risk does not diminish with liver transplantation. This patient population also has a high frequency of carcinoma in gallbladder mass lesions. The risk for hepatocellular carcinoma (HCC) in the presence of cirrhosis is uncertain-two large cohort studies suggest that HCC is not as common as in other causes of cirrhosis. Although AASLD guidelines do not recommend routine screening for liver tumors in patients with PSC, we recommend MRI/MRCP and serum CA 19-9 levels in patients with PSC every 6 months to screen for CCA, HCC, pancreatic cancer, and gallbladder cancer. Screening colonoscopy at the diagnosis of PSC and surveillance colonoscopies every 1-2 years should be performed in those with PSC and IBD.

  11. Geoepidemiology of primary sclerosing cholangitis: a critical review.

    Science.gov (United States)

    Tanaka, Atsushi; Takikawa, Hajime

    2013-10-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin, characterized by progressive destruction of bile ducts caused by diffuse inflammation and fibrosis. Previous epidemiological studies in Northern Europe and North America demonstrated that incidence and prevalence rates are ranging from 0.5 to 1.3 and from 3.85 to 16.2 per 100,000 inhabitants per year, respectively. It is of note that the incidence of PSC appears to be gradually increasing. We have extensively reviewed the geoepidemiology of PSC and attempted to place it in context with the incidence in Japan. In 2012, the clinical diagnostic criteria of IgG4-SC were established and published by the Japan Biliary Association, rendering it possible for physicians to clinically differentiate PSC from IgG4-SC. We conducted a new nationwide survey for PSC as well as IgG4-SC, and have identified 197 patients with PSC and 43 patients with IgG4-SC without pancreatic involvement. In this survey we estimated prevalence rate of PSC in Japan as 0.95, lower than those in North America and European countries. Also we identified other unique features of Japanese PSC patients, including 2 peaks in age distribution at diagnosis and fewer presences of comorbid inflammatory bowel diseases, occurring in only 34% of PSC. This data is placed in the perspective of the international experience on PSC.

  12. Gallbladder emptying in patients with primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Karouk Said; Nick Edsborg; Nils Albiin; Annika Bergquist

    2009-01-01

    AIM: To assess gallbladder emptying and its association with cholecystitis and abdominal pain in patients with primary sclerosing cholangitis (PSC). METHODS: Twenty patients with PSC and ten healthy subjects were investigated. Gallbladder fasting volume, ejection fraction and residual volume after ingestion of a test meal were compared in patients with PSC and healthy controls using magnetic resonance imaging. Symptoms, thickness and contrast enhancement of the gallbladder wall and the presence of cystic duct strictures were also assessed. RESULTS: Median fasting gallbladder volume in patients with PSC [67 (19-348) mL] was twice that in healthy controls [32 (16-55) mL] ( P < 0.05). The median postprandial gallbladder volume in patients with PSC was significantly larger than that in healthy controls ( P < 0.05). There was no difference in ejection fraction, gallbladder emptying volume or mean thickness of the gallbladder wall between PSC patients and controls. Contrast enhancement of the gallbladder wall in PSC patients was higher than that in controls; (69% ± 32%) and (42% ± 21%) ( P < 0.05). No significant association was found between the gallbladder volumes and occurrence of abdominal pain in patients and controls. CONCLUSION: Patients with PSC have increased fasting gallbladder volume. Gallbladder Mucosal dysfunction secondary to chronic cholecystitis, may be a possible mechanism for increased gallbladder.

  13. Magnetic resonance cholangiopancreatography in primary sclerosing cholangitis in children

    Energy Technology Data Exchange (ETDEWEB)

    Ferrara, Calogero; Valeri, Gianluca; Salvolini, Luca [Department of Radiology, University of Ancona (Italy); Giovagnoni, Andrea [Department of Radiology, University of Modena (Italy)

    2002-06-01

    Background: Primary sclerosing cholangitis (PSC) is a chronic cholestatic syndrome of unknown aetiology. Diagnosis is based on clinical and laboratory data in conjunction with imaging of the biliary tree using endoscopic retrograde cholangiopancreatography (ERCP). Objective: To evaluate the clinical usefulness of MR cholangiopancreatography (MRCP) in the diagnosis of PSC in children. Materials and methods: Twenty-one children with clinical and laboratory suspicion of PSC were enrolled. MRCP was performed using a superconductive system with a phased-array coil. Rapid acquisition with relaxation enhancement (RARE) T2-weighted and half-Fourier single-shot turbo-spin-echo (HASTE) sequences were used. The distribution and extent of biliary tree changes were evaluated and classified according to Majoie's classification. A comparison between MRCP and ERCP was performed blind in all cases to evaluate the usefulness of MRI. Results: In 13 cases (62%), MRCP showed abnormalities of the biliary tree which were considered positive for PSC, while in 8 cases there were no signs of PSC. Both MRCP and ERCP correctly identified changes in 13 cases and excluded abnormalities in 5. MRCP had a sensitivity of 81%, specificity of 100%, negative predictive value of 62%, positive predictive value of 100% and an accuracy of 85%. Conclusions: MRCP can be proposed as the preliminary non-invasive imaging modality for the diagnosis of PSC in children. (orig.)

  14. Phenotyping and auto-antibody production by liver-infiltrating B cells in primary sclerosing cholangitis and primary biliary cholangitis.

    Science.gov (United States)

    Chung, Brian K; Guevel, Bardia T; Reynolds, Gary M; Gupta Udatha, D B R K; Henriksen, Eva Kristine Klemsdal; Stamataki, Zania; Hirschfield, Gideon M; Karlsen, Tom Hemming; Liaskou, Evaggelia

    2017-02-01

    Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are immune-mediated biliary diseases that demonstrate prominent and restricted genetic association with human leukocyte antigen (HLA) alleles. In PBC, anti-mitochondrial antibodies (AMA) are specific and used as diagnostic biomarkers. PSC-relevant auto-antibodies remain controversial despite a distinct HLA association that mirrors archetypical auto-antigen driven disorders. Herein, we compared antibody-secreting B cells (ASCs) in PSC and PBC liver explants to determine if liver-infiltrating ASCs represent an opportune and novel source of disease-relevant auto-antibodies. Using enzymatic digestion and mechanical disruption, liver mononuclear cells (LIMCs) were isolated from fresh PSC and PBC explants and plasmablast (CD19+CD27+CD38(hi)CD138-) and plasma cell (CD19+CD27+CD38(hi)CD138+) ASCs were enumerated by flow cytometry. We observed 45-fold fewer plasma cells in PSC explants (n = 9) compared to PBC samples (n = 5, p < 0.01) and 10-fold fewer IgA-, IgG- and IgM-positive ASCs (p < 0.05). Liver-infiltrating ASCs from PSC and PBC explants were functional and produced similar concentrations of IgA, IgG and IgM following 2 weeks of culture. Antibody production by PBC ASCs (n = 3) was disease-specific as AMA to pyruvate dehydrogenase complex E2 subunit (PDC-E2) was detected by immunostaining, immunoblotting and ELISA. Antibody profiling of PSC supernatants (n = 9) using full-length recombinant human protein arrays (Cambridge Protein Arrays) revealed reactivities to nucleolar protein 3 (5/9) and hematopoietic cell-specific Lyn substrate 1 (3/9). Array analysis of PBC supernatants (n = 3) detected reactivities to PDC-E2 and hexokinase 1 (3/3). In conclusion, we detected unique frequencies of liver-infiltrating ASCs in PSC and PBC and in so doing, highlight a feasible approach for understanding disease-relevant antibodies in PSC. Copyright © 2016. Published by Elsevier Ltd.

  15. Antineutrophil cytoplasmic antibodies in bile are associated with disease activity in primary sclerosing cholangitis.

    Science.gov (United States)

    Lenzen, Henrike; Weismüller, Tobias J; Negm, Ahmed A; Wlecke, Jenny; Loges, Stephanie; Strassburg, Christian P; Manns, Michael P; Lankisch, Tim O

    2013-10-01

    OBJECTIVE. Primary sclerosing cholangitis (PSC) is an autoimmune cholestatic liver disease of unknown etiology. The role of antineutrophil cytoplasmic antibodies (ANCAs) in the serum of patients with PSC remains unclear. We hypothesized that ANCA may be detectable in bile, potentially providing diagnostic and prognostic information. METHODS. Serum and bile were prospectively collected during endoscopic retrograde cholangiography (ERC) in 72 patients with PSC and other non-PSC obstructive biliary diseases. ANCA measurements were performed by indirect immunofluorescence (IIF). RESULTS. Immunoglobulin G (IgG) ANCA was detected significantly more often in the bile of PSC patients (15/39; 38%) than without (2/33; 6%) (p = 0.001). IgG ANCA in bile was associated with a ten times higher risk of PSC (p = 0.005). In addition, IgG ANCA positivity in bile was associated with the presence of dominant strictures (p = 0.03), cholangiographic severity (p = 0.004), number of ERC (p = 0.01) and interventions performed (p = 0.03). However, IgG ANCA in bile did not correlate with transplantation, cholangiocarcinoma or death. No association was observed between ANCA positivity in sera and ANA and ASCA positivity in sera or bile with the above-mentioned clinical features. CONCLUSIONS. The presence of ANCA in the bile of patients with PSC is a novel finding and highly suggestive of PSC. Biliary IgG ANCA correlates with the severity of bile duct strictures and the ensuing number of ERCs and interventions. Therefore, a positive ANCA status in bile may serve as a diagnostic and prognostic marker of the disease progression and biliary complications.

  16. Diagnostic challenges of Wilson's disease presenting as acute pancreatitis, cholangitis, and jaundice.

    Science.gov (United States)

    Nussinson, Elchanan; Shahbari, Azmi; Shibli, Fahmi; Chervinsky, Elena; Trougouboff, Philippe; Markel, Arie

    2013-11-27

    Wilson's disease is a rare disorder of copper transport in hepatic cells, and may present as cholestatic liver disease; pancreatitis and cholangitis are rarely associated with Wilsons's disease. Moreover, cases of Wilson's disease presenting as pigmented gallstone pancreatitis have not been reported in the literature. In the present report, we describe a case of a 37-year-old man who was admitted with jaundice and abdominal pain. The patient was diagnosed with acute pancreatitis, cholangitis, and obstructive jaundice caused by pigmented gallstones that were detected during retrograde cholangiopancreatography. However, because of his long-term jaundice and the presence of pigmented gallstones, the patient underwent further evaluation for Wilson's disease, which was subsequently confirmed. This patient's unique presentation exemplifies the overlap in the clinical and laboratory parameters of Wilson's disease and cholestasis, and the difficulties associated with their differentiation. It suggests that Wilson's disease should be considered in patients with pancreatitis, cholangitis, and severe protracted jaundice caused by pigmented gallstones.

  17. Progression of Tokyo Guidelines and Japanese Guidelines for management of acute cholangitis and cholecystitis.

    Science.gov (United States)

    Mayumi, Toshihiko; Someya, Kazuki; Ootubo, Hiroki; Takama, Tatsuo; Kido, Takashi; Kamezaki, Fumihiko; Yoshida, Masahiro; Takada, Tadahiro

    2013-12-01

    The Japanese Guidelines for management of acute cholangitis and cholecystitis were published in 2005 as the first practical guidelines presenting diagnostic and severity assessment criteria for these diseases. After the Japanese version, the Tokyo Guidelines (TG07) were reported in 2007 as the first international practical guidelines. There were some differences between the two guidelines, and some weak points in TG07 were pointed out, such as low sensitivity for diagnosis and the presence of divergence between severity assessment and clinical judgment for acute cholangitis. Therefore, revisions were started to not only make them up to date but also concurrent with the same diagnostic and severity assessment criteria. The Revision Committee for the revision of TG07 (TGRC) performed validation studies of TG07 and new diagnostic and severity assessment criteria of acute cholangitis and cholecystitis. These were retrospective multi-institutional studies that collected cases of acute cholangitis, cholecystitis, and non-inflammatory biliary disease. TGRC held 35 meetings as well as international email exchanges with co-authors abroad and held three International Meetings. Through these efforts, TG13 improved the diagnostic sensitivity for acute cholangitis and cholecystitis, and presented criteria with extremely low false positive rates. Furthermore, severity assessment criteria adapted for clinical use, flowcharts, and many new diagnostic and therapeutic modalities were presented. The world's first management bundles of acute cholangitis and cholecystitis were also presented. The revised Japanese version was published with the same content as TG13. An electronic application of TG13 that can help to diagnose and assess the severity of these diseases using the criteria of TG13 was made for free download.

  18. Eosinophilic cholangitis is a potentially underdiagnosed etiology in indeterminate biliary stricture

    Science.gov (United States)

    Walter, Dirk; Hartmann, Sylvia; Herrmann, Eva; Peveling-Oberhag, Jan; Bechstein, Wolf O; Zeuzem, Stefan; Hansmann, Martin-Leo; Friedrich-Rust, Mireen; Albert, Jörg G

    2017-01-01

    AIM To investigate presence and extent of eosinophilic cholangitis (EC) as well as IgG4-related disease in patients with indeterminate biliary stricture (IBS). METHODS All patients with diagnosis of sclerosing cholangitis (SC) and histopathological samples such as biopsies or surgical specimens at University Hospital Frankfurt from 2005-2015 were included. Histopathological diagnoses as well as further clinical course were reviewed. Tissue samples of patients without definite diagnosis after complete diagnostic work-up were reviewed regarding presence of eosinophilic infiltration and IgG4 positive plasma cells. Eosinophilic infiltration was as well assessed in a control group of liver transplant donors and patients with primary sclerosing cholangitis. RESULTS one hundred and thirty-five patients with SC were included. In 10/135 (13.5%) patients, no potential cause of IBS could be identified after complete diagnostic work-up and further clinical course. After histopathological review, a post-hoc diagnosis of EC was established in three patients resulting in a prevalence of 2.2% (3/135) of all patients with SC as well as 30% (3/10) of patients, where no cause of IBS was identified. 2/3 patients with post-hoc diagnosis of EC underwent surgical resection with suspicion for malignancy. Diagnosis of IgG4-related cholangitis was observed in 7/135 patients (5.1%), whereas 3 cases were discovered in post-hoc analysis. 6/7 cases with IgG4-related cholangitis (85.7%) presented with eosinophilic infiltration in addition to IgG4 positive plasma cells. There was no patient with eosinophilic infiltration in the control group of liver transplant donors (n = 27) and patients with primary sclerosing cholangitis (n = 14). CONCLUSION EC is an underdiagnosed benign etiology of SC and IBS, which has to be considered in differential diagnosis of IBS. PMID:28246478

  19. Recurrent pyogenic cholangitis: The pattern of thickening of the extrahepatic bile duct on CT

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Tae Hoon; Lim, Jae Hoon; Ko, Young Tae; Lee, Dong Ho; Jeong, Yu Mee; Lee, Eil Seong [Kang Hee University Hospital, Seoul (Korea, Republic of)

    1993-05-15

    The pattern of thickening of the extrahepatic bile duct on computed tomography was analysed in 30 cases with recurrent pyogenic cholangitis diagnosed by surgery (n=19) or by clinical basis (n=11). The mean wall thickness of the extrahepatic bile duct was 3.3 mm (range, 1-6.3 mm). Diffuse thickening of the extrahepatic bile ductal wall was demonstrated in 26 of 30 cases. Diffuse thickening of the extrahepatic bile duct in recurrent pyogenic cholangitis may be differentiated from focal thickening of duct in a common duct cancer or pancreatic cancer.

  20. Therapy of Primary Sclerosing Cholangitis--Today and Tomorrow.

    Science.gov (United States)

    Halilbasic, Emina; Fuchs, Claudia; Hofer, Harald; Paumgartner, Gustav; Trauner, Michael

    2015-01-01

    Primary sclerosing cholangitis (PSC) represents a fibro-obliterative bile duct disease with unpredictable individual clinical course that may progress to liver cirrhosis and malignancy. Due to our incomplete understanding of the etiology and pathogenesis of this disease, the therapeutic options are still rather limited. Bile acids play a key role in mediating cholangiocellular and hepatocellular injury in cholangiopathies such as PSC. Therefore, strategies targeting bile composition and homeostasis are valid approaches in PSC. Ursodeoxycholic acid (UDCA) is the paradigm therapeutic bile acid and its role in medical therapy of PSC is still under debate. Promising novel bile acid-based therapeutic options include 24-norursodeoxycholic acid (norUDCA), a side chain-shortened C23 homologue of UDCA, and bile acid receptor/farnesoid X receptor agonists (e.g. obeticholic acid). Other nuclear receptors such as fatty acid-activated peroxisome proliferator-activated receptors, vitamin D receptor and vitamin A receptors (retinoic acid receptor, retinoid X receptor) are also of potential interest and can be targeted by already available drugs. Furthermore, drugs targeting the gut-liver axis (e.g. intregrin blockers such as vedolizumab, antibiotics) appear promising, based on the close link of PSC to inflammatory bowel disease and the emerging relevance of the gut microbiome for the development of PSC. Finally, fibrosis represents a valid therapeutic target for anti-fibrotic drugs (e.g. simtuzumab) in PSC as paradigm fibro-obliterative disease. This review summarizes the current status and recent progress in the development of targeted therapeutic approaches based on increasing knowledge about the pathogenesis of this disease.

  1. Duration of Inflammatory Bowel Disease Is Associated With Increased Risk of Cholangiocarcinoma in Patients With Primary Sclerosing Cholangitis and IBD

    Science.gov (United States)

    Gulamhusein, Aliya F.; Eaton, John E.; Tabibian, James H.; Atkinson, Elizabeth J.; Juran, Brian D.; Lazaridis, Konstantinos N.

    2016-01-01

    OBJECTIVES Primary sclerosing cholangitis (PSC) often coexists with inflammatory bowel disease (IBD) and can be complicated by cholangiocarcinoma (CCA), a lethal malignancy for which reliable predictors remain unknown. We aimed to characterize the influence of colectomy and IBD duration on risk of CCA in patients with PSC-IBD. METHODS A retrospective review of patients with PSC-IBD seen at the Mayo Clinic, Rochester, between January 2005 and May 2013 was performed. The primary outcome was time to development of CCA and our goal was to determine whether the risk differed between patients with and without colectomy. Risk factors were assessed using univariable and multivariable Cox proportional hazard models where colectomy, IBD disease duration, and development of advanced liver disease were treated as time-dependent covariates. RESULTS A total of 399 patients with PSC-IBD were included in the study, of whom 137 had a colectomy and 123 patients developed CCA. Age-adjusted univariate Cox proportional hazard models demonstrated that colectomy (hazard ratio (HR) 1.53, 95% confidence interval (CI) 1.05–2.22, P =0.02) and duration of IBD (HR 1.37, 95% CI 1.15–1.63, P PSC-IBD, and colectomy itself does not modify this risk. These findings identify a subset of patients who are at high risk of this lethal complication and in need of close surveillance. PMID:27002801

  2. Outcome of patients with primary sclerosing cholangitis and ulcerative colitis undergoing colectomy

    Institute of Scientific and Technical Information of China (English)

    Sombat; Treeprasertsuk; Einar; Bjrnsson; Emmanouil; Sinakos; Emma; Weeding; Keith; D; Lindor

    2013-01-01

    AIM:To study the outcomes of primary sclerosing cholangitis(PSC) patients with ulcerative colitis(UC) undergoing colectomy.METHODS:We identified 193 patients with PSC and UC undergoing colectomy at the Mayo Clinic(Rochester,MN,United States),between January 1,1995 and December 31,2008 using a computerized record system.Eighty-nine patients were excluded due to unclear diagnosis,liver transplantation prior to colectomy,age less than 18 years,inadequate follow-up data or known cases of cholangiocarcinoma.We retrospectively reviewed data from patient medical records.Clinical information,date of colectomy,preoperative and follow-up liver tests and pathological findings of the colon were reviewed.The Mayo risk score at baseline was calculated to obtain survival estimates for up to 4 years of follow-up.The primary endpoint was defined by the presence of all-cause mortality and/or liver decompensation requiring liver transplantation.All patients who did not have a clinical note on December 31,2008 were considered as patients with an incomplete follow-up unless they reached a study endpoint(death or underwent liver transplantation) prior to that date.The study was approved by the Institutional Review Boards of the Mayo Clinic.RESULTS:Of the 2441 patients with PSC observed in this period,104 patients(4.3%) had UC and underwent colectomy and were included.The median age was 43.2 years,and 67% were male.The leading indications for colectomy were severe colonic inflammation(49%),the presence of colonic dysplasia during routine surveillance(42%) and bowel perforation(3%).Twenty-six patients were lost to follow-up after a median duration of 3.9 years.The remaining 78 patients included 52 patients(66.7%) who were followed for a median duration of 5.5 years and 26 patients(33.3%) who developed primary endpoints including death(n = 13) or underwent liver transplantation(n = 13) with a median follow up of 2.6 years.For the secondary endpoint,the liver complications within 1 mo

  3. Bile proteomic profiles differentiate cholangiocarcinoma from primary sclerosing cholangitis and choledocholithiasis

    NARCIS (Netherlands)

    Lankisch, Tim O; Metzger, Jochen; Negm, Ahmed A; Vosskuhl, Katja; Schiffer, Eric; Siwy, Justyna; Weismüller, Tobias J; Schneider, Andrea S; Thedieck, Kathrin; Baumeister, Ralf; Zürbig, Petra; Weissinger, Eva M; Manns, Michael P; Mischak, Harald; Wedemeyer, Jochen

    2011-01-01

    UNLABELLED: Early detection of malignant biliary tract diseases, especially cholangiocarcinoma (CC) in patients with primary sclerosing cholangitis (PSC), is very difficult and often comes too late to give the patient a therapeutic benefit. We hypothesize that bile proteomic analysis distinguishes C

  4. More Right-sided IBD-associated Colorectal Cancer in Patients with Primary Sclerosing Cholangitis

    NARCIS (Netherlands)

    Claessen, M. M. H.; Lutgens, M. W. M. D.; van Buuren, H. R.; Oldenburg, B.; Stokkers, P. C. F.; van der Woude, C. J.; Hommes, D. W.; de Jong, D. J.; Dijkstra, G.; van Bodegraven, A. A.; Siersema, P. D.; Vleggaar, F. P.

    2009-01-01

    Background: Patients with inflammatory bowel disease (IBD) and concurrent primary sclerosing cholangitis (PSC) have a higher risk of developing colorectal cancer (CRC) than IBD patients without PSC. The aim of this Study was to investigate potential clinical differences between patients with CRC in

  5. Primary biliary cirrhosis and autoimmune cholangitis are not associated with coeliac disease in Crete

    Directory of Open Access Journals (Sweden)

    Dimoulios Philippos

    2002-03-01

    Full Text Available Abstract Background An increased prevalence of coeliac disease in patients with primary biliary cirrhosis has been recently reported. However, in other studies the association has not been confirmed. There have been no formal attempts to systematically evaluate patients with autoimmune cholangitis for coeliac disease. Methods Sera from 62 patients with primary biliary cirrhosis, 17 with autoimmune cholangitis and 100 blood donors were screened for anti-gliadin, anti-endomysial, anti-reticulin, and IgA class antibodies to guinea pig liver-derived tissue transglutaminase. Eighteen untreated coeliacs served as methodological controls. Analyses were performed by using the χ2 and Fischer's exact tests. Results Anti-gliadin antibodies were detected in 21% of patients with primary biliary cirrhosis, 35% of patients with autoimmune cholangitis, and 3% of controls (p Conclusions We were unable to demonstrate an increased risk of coeliac disease in patients with primary biliary cirrhosis and autoimmune cholangitis. Our results confirm the previously reported high prevalence of false-positive anti-gliadin and guinea pig liver-derived anti-tissue transglutaminase antibodies in patients with chronic liver disease.

  6. Steroid-refractory ulcerative colitis and associated primary sclerosing cholangitis treated with infliximab

    Science.gov (United States)

    Duca, Ileana; Ramírez de la Piscina, Patricia; Estrada, Silvia; Calderón, Rosario; Spicakova, Katerina; Urtasun, Leire; Marra-López, Carlos; Zabaleta, Salvador; Bengoa, Raquel; Marcaide, María Asunción; García-Campos, Francisco

    2013-01-01

    Primary sclerosing cholangitis is an infrequent extraintestinal manifestation of ulcerative colitis. Damage to bile ducts is irreversible and medical therapies to prevent progression of the disease are usually ineffective. We describe a patient with long-standing ulcerative colitis, which was refractory to corticosteroid therapy who developed primary sclerosing cholangitis (biochemical stage II/IV) in the course of his pancolitis. Treatment with infliximab (5 mg/kg as an induction dose followed by maintenance doses every two months) was indicated because of steroid-dependent disease associated to primary sclerosing cholangitis as well as sacroiliitis and uveitis and previous episode of severe azathioprine-related hepatic toxicity. At present, after two years of follow-up, the patient is asymptomatic with normal liver tests and complete resumption of daily life activities. This case draws attention to the usefulness of anti-tumor necrosis factor-alpha therapy for the management of primary sclerosing cholangitis as extraintestinal manifestation of inflammatory bowel disease. PMID:23382642

  7. Genome-wide association analysis in primary sclerosing cholangitis identifies two non-HLA susceptibility loci

    NARCIS (Netherlands)

    Melum, Espen; Franke, Andre; Schramm, Christoph; Weismueller, Tobias J.; Gotthardt, Daniel Nils; Offner, Felix A.; Juran, Brian D.; Laerdahl, Jon K.; Labi, Verena; Bjoernsson, Einar; Weersma, Rinse K.; Henckaerts, Liesbet; Teufel, Andreas; Rust, Christian; Ellinghaus, Eva; Balschun, Tobias; Boberg, Kirsten Muri; Ellinghaus, David; Bergquist, Annika; Sauer, Peter; Ryu, Euijung; Hov, Johannes Roksund; Wedemeyer, Jochen; Lindkvist, Bjoern; Wittig, Michael; Porte, Robert J.; Holm, Kristian; Gieger, Christian; Wichmann, H-Erich; Stokkers, Pieter; Ponsioen, Cyriel Y.; Runz, Heiko; Stiehl, Adolf; Wijmenga, Cisca; Sterneck, Martina; Vermeire, Severine; Beuers, Ulrich; Villunger, Andreas; Schrumpf, Erik; Lazaridis, Konstantinos N.; Manns, Michael P.; Schreiber, Stefan; Karlsen, Tom H.

    Primary sclerosing cholangitis (PSC) is a chronic bile duct disease affecting 2.4-7.5% of individuals with inflammatory bowel disease. We performed a genome-wide association analysis of 2,466,182 SNPs in 715 individuals with PSC and 2,962 controls, followed by replication in 1,025 PSC cases and

  8. A case of postoperative recurrent cholangitis after pancreaticoduodenectomy successfully treated by tract conversion surgery.

    Science.gov (United States)

    Shiihara, Masahiro; Miura, Osamu; Konishi, Kozo; Takeo, Sachiko; Kakimoto, Tadatoshi; Hidaka, Gen; Shibui, Yuichi; Minamisono, Yoshikazu; Toda, Tomohiro; Uemura, Shuichiro; Yamamoto, Masakazu

    2016-07-08

    A 69-year-old man, who had undergone pylorus-preserving pancreaticoduodenectomy (PD) (Imanaga procedure) for duodenum papilla cancer 13 years prior, had a history of repeated hospitalization due to cholangitis since the third year after surgery and liver abscess at the 10th year after surgery. Gastrointestinal series indicated no stenosis after the cholangiojejunostomy. However, reflux of contrast media into the bile duct and persistence of food residues were observed. We considered the cholangitis to be caused by reflux and persistence of food residues into the bile duct. So, we performed the tract conversion surgery, Imanaga procedure to Child method. The postoperative course was good even after re-initiating dietary intake. He was discharged on the 19th day after surgery. He has not experienced recurrent cholangitis for 18 months. For patients with post-PD recurrent cholangitis caused by reflux of food residues like ours, surgical treatment should be considered because tract conversion may be an effective solution. Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author 2016.

  9. A case of postoperative recurrent cholangitis after pancreaticoduodenectomy successfully treated by tract conversion surgery

    Science.gov (United States)

    Shiihara, Masahiro; Miura, Osamu; Konishi, Kozo; Takeo, Sachiko; Kakimoto, Tadatoshi; Hidaka, Gen; Shibui, Yuichi; Minamisono, Yoshikazu; Toda, Tomohiro; Uemura, Shuichiro; Yamamoto, Masakazu

    2016-01-01

    A 69-year-old man, who had undergone pylorus-preserving pancreaticoduodenectomy (PD) (Imanaga procedure) for duodenum papilla cancer 13 years prior, had a history of repeated hospitalization due to cholangitis since the third year after surgery and liver abscess at the 10th year after surgery. Gastrointestinal series indicated no stenosis after the cholangiojejunostomy. However, reflux of contrast media into the bile duct and persistence of food residues were observed. We considered the cholangitis to be caused by reflux and persistence of food residues into the bile duct. So, we performed the tract conversion surgery, Imanaga procedure to Child method. The postoperative course was good even after re-initiating dietary intake. He was discharged on the 19th day after surgery. He has not experienced recurrent cholangitis for 18 months. For patients with post-PD recurrent cholangitis caused by reflux of food residues like ours, surgical treatment should be considered because tract conversion may be an effective solution. PMID:27402542

  10. Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis

    NARCIS (Netherlands)

    Weismueller, Tobias J.; Trivedi, Palak J; Bergquist, Annika; Imam, Mohamad; Lenzen, Henrike; Ponsioen, Cyriel Y.; Holm, Kristian; Gotthardt, Daniel; Faerkkilae, Martti A.; Marschall, Hanns-Ulrich; Thorburn, Douglas; Weersma, Rinse K.; Fevery, Johan; Mueller, Tobias; Chazouilleres, Olivier; Schulze, Kornelius; Lazaridis, Konstantinos N.; Almer, Sven; Pereira, Stephen P.; Levy, Cynthia; Mason, Andrew L.; Naess, Sigrid; Bowlus, Christopher L.; Floreani, Annarosa; Halilbasic, Emina; Yimam, Kidist K.; Milkiewicz, Piotr; Beuers, Ulrich; Huynh, Dep K.; Pares, Albert; Manser, Christine N.; Dalekos, George N.; Eksteen, Bertus; Invernizzi, Pietro; Berg, Christoph P.; Kirchner, Gabi I.; Sarrazin, Christoph; Zimmer, Vincent; Fabris, Luca; Braun, Felix; Marzioni, Marco; Juran, Brian D.; Said, Karouk; Rupp, Christian; Jokelainen, Kalle; de Valle, Maria Benito; Saffioti, Francesca; Cheung, Angela; Trauner, Michael; Schramm, Christoph; Chapman, Roger W.; Karlsen, Tom H.; Schrumpf, Erik; Strassburg, Christian P.; Manns, Michael P.; Lindor, Keith D; Hirschfield, Gideon M.; Hansen, Bettina E.; Boberg, Kirsten M.

    BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is an orphan hepatobiliary disorder associated with inflammatory bowel disease (IBD). We aimed to estimate the risk of disease progression based on distinct clinical phenotypes in a large international cohort of patients with PSC. METHODS: We

  11. More right-sided IBD-associated colorectal cancer in patients with primary sclerosing cholangitis

    NARCIS (Netherlands)

    M.M.H. Claessen; M.W.M.D. Lutgens; H.R. van Buuren; B. Oldenburg; P.C.F. Stokkers; C.J. van der Woude; D.W. Hommes; D.J. de Jong; G. Dijkstra; A.A. van Bodegraven; P.D. Siersema; F.P. Vleggaar

    2009-01-01

    Background: Patients with inflammatory bowel disease (IBD) and concurrent primary sclerosing cholangitis (PSC) have a higher risk of developing colorectal cancer (CRC) than IBD patients without PSC. The aim of this Study was to investigate potential clinical differences between patients with CRC in

  12. More right-sided IBD-associated colorectal cancer in patients with primary sclerosing cholangitis.

    NARCIS (Netherlands)

    Claessen, M.M.; Lutgens, M.W.; Buuren, H.R. van; Oldenburg, B.; Stokkers, P.C.; Woude, C.J. van der; Hommes, D.W.; Jong, D.J. de; Dijkstra, G.; Bodegraven, A.A. van; Siersema, P.D.; Vleggaar, F.P.

    2009-01-01

    BACKGROUND: Patients with inflammatory bowel disease (IBD) and concurrent primary sclerosing cholangitis (PSC) have a higher risk of developing colorectal cancer (CRC) than IBD patients without PSC. The aim of this study was to investigate potential clinical differences between patients with CRC in

  13. Colorectal carcinogenesis in patients with primary sclerosing cholangitis and inflammatory bowel disease

    NARCIS (Netherlands)

    Claessen, M.M.H.

    2010-01-01

    Patiënten met inflammatoire darmziekte (IBD) hebben een verhoogd risico op het ontstaan van colorectaal carcinoom (CRC; 20-jaars risico 8%). Wij vonden dat het risico op CRC bij patiënten die naast IBD ook bekend zijn met de galwegaandoening primaire scleroserende cholangitis (PSC) nog veel hoger is

  14. Primary sclerosing cholangitis is associated with a distinct phenotype of inflammatory bowel disease

    NARCIS (Netherlands)

    Boonstra, K.; Erpecum, K.J. van; Nieuwkerk, K.M. van; Drenth, J.P.H.; Poen, A.C.; Witteman, B.J.; Tuynman, H.A.; Beuers, U.; Ponsioen, C.Y.

    2012-01-01

    BACKGROUND: Primary sclerosing cholangitis (PSC) is strongly associated with inflammatory bowel disease (IBD). The aim of this study was to assess the IBD phenotype associated with PSC in a large well-phenotyped population-based PSC cohort using endoscopic and histopathologic criteria. METHODS: PSC

  15. Mortality and cancer risk related to primary sclerosing cholangitis in a Swedish population-based cohort

    NARCIS (Netherlands)

    de Valle, Maria Benito; Bjornsson, Einar; Lindkvist, Bjorn

    2012-01-01

    Background: Population-based studies on the epidemiology of primary sclerosing cholangitis (PSC) are sparse. Aims: To investigate mortality and risk of cancer, and to identify risk factors for hepatobiliary cancer and the combined end-point liver related death or liver transplantation (OLT) in a pop

  16. Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    de Vries, Elisabeth M G; de Krijger, Manon; Färkkilä, Martti

    2017-01-01

    Histologic scoring systems specific for primary sclerosing cholangitis (PSC) are not validated. We recently determined the applicability and prognostic value of three histological scoring systems in a single PSC cohort. The aim of this study was to validate their prognostic use and reproducibility...

  17. Primary sclerosing cholangitis and primary biliary cirrhosis: epidemiology, risk factors, and outcome

    NARCIS (Netherlands)

    Boonstra, K.

    2014-01-01

    Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are chronic cholestatic liver diseases of unknown etiology. True population-based epidemiological studies are scarce, especially in PSC. There is a need for large population-based studies combining meticulous case-finding and c

  18. Immunosuppression after liver transplantation for primary sclerosing cholangitis influences activity of inflammatory bowel disease

    DEFF Research Database (Denmark)

    Jørgensen, Kristin Kaasen; Lindström, Lina; Cvancarova, Milada

    2013-01-01

    Previous studies have shown conflicting results regarding the course of inflammatory bowel disease (IBD) after liver transplantation in patients with primary sclerosing cholangitis (PSC). We studied the progression of IBD in patients with PSC who have undergone liver transplantation. We also...

  19. cal, therapeutic and histo-morphological studies in primary biliary cirrhosis and primary sclerosing cholangitis

    NARCIS (Netherlands)

    H.J.F. Hoogstraten (Hubert)

    1999-01-01

    textabstractPrimary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are chronic cholestatic liver diseases, biochemically characterised by an elevated serum bilirubin, alkaline phosphatase (APh) and y-glutamyl transferase (y-GT). Although PBC and PSC have their own distinctive clini

  20. Alcohol consumption in patients with primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Hannes Hagstr(o)m; Per St(a)l; Knut Stokkeland; Annika Bergquist

    2012-01-01

    AIM:To assess the alcohol drinking patterns in a cohort of primary sclerosing cholangitis (PSC) patients and the possible influence on the development of fibrosis.METHODS:Ninety-six patients with PSC were evaluated with a validated questionnaire about a patient's lifetime drinking habits:the lifetime drinking history (LDH) questionnaire.In addition,clinical status,transient elastography and biochemistry values were analysed and registered.Patients were defined as having either significant or non-significant fibrosis.Significant fibrosis was defined as either an elastography value of ≥ 17.3 kPa or the presence of clinical signs of cirrhosis.Patients were divided into two groups depending on their alcohol consumption patterns; no/low alcohol consumption (one drink or unit/d) and moderate/high alcohol consumption (≥ 1 drink or unit/d).LDH data were calculated to estimate lifetime alcohol intake (LAI),current alcohol intake,drinks per year before and after diagnosis of PSC.We also calculated the number of episodes of binge-drinking (defined as consuming ≥ 5 drinks per occasion) in total,before and after the diagnosis of PSC.RESULTS:The mean LAI was 3882 units of alcohol,giving a mean intake after onset of alcohol consumption of 2.6 units per week.Only 9% of patients consumed alcohol equal to or more than one unit per day.Current alcohol intake in patients with significant fibrosis (n =26) was less than in patients without significant fibrosis (n =70),as shown by lower values of phosphatidylethanol (B-PEth) (0.1 μmol/L vs 0.33 μmol/L,respectively,P =0.002) and carbohydrate-deficient transferrin (CDT)(0.88% vs 1.06%,respectively,P =0.02).Self-reported LAI was similar between the two groups.Patients with significant fibrosis reduced their alcohol intake after diagnosis from 103 to 88 units per year whereas patients without fibrosis increased their alcohol intake after PSC diagnosis from 111 to 151 units/year.There were no correlations between elastography

  1. Recurrent pyogenic cholangitis : efficacy of two-phase helical CT

    Energy Technology Data Exchange (ETDEWEB)

    Jeong, Ki Ho; Cho, June Sik; Shin, Kyung Sook; Lee, Se Hyo; Yu, Ho Jun; Park, Jin Yong; Kim, Young Min [College of Medicine, Chungnam National University, Taejon (Korea, Republic of)

    2000-01-01

    To evaluate the usefulness of two-phase helical CT in patients with recurrent pyogenic cholangitis (RPC) for the detection of acute inflammation and assessment of the degree of portal vein (PV) stenosis as a cause of hepatic parenchymal atrophy. We retrospectively reviewed two-phase CT findings in 30 patients with RPC diagnosed by CT, ERCP (endoscopic retrograde cholangiopancreatography), and surgery. Two-phase helical CT scans were obtained 30 sec (arterial phase, AP) and 70 sec (portal phase, PP) after the start of IV administration of contrast material. Without prior information, we analyzed periductal parenchymal and ductal wall enhancement during the AP and PP, and the degree of PV stenosis during the PP. Acute inflammation was diagnosed on the basis of symptoms and laboratory findings. To evaluate the relationship between parenchymal a trophy and PV stenosis, the degree of PV stenosis in affected parenchyma was classified as one of three types (mild, less than 25%; moderate, 25-75%; severe, greater than 75%), as compared with the diameter of normal PV in unaffected parenchyma. Ten of the 30 patients underwent CT during the acute inflammatory stage and 20 during the remission stage. Of the ten patients with acute inflammation, eight (80%) showed transient periductal parenchymal enhancement during the AP (p less than 0.05), which correlated closely with acute inflammation. Only three (15%) of the 20 patients with remission, however, showed transient parenchymal enhancement during this phase, at which time ductal wall enhancement was seen in three (30%) of the ten patients with acute inflammation and in seven (35%) of the 20 who showed remission (p greater than 0.05). There was no significant difference in parenchymal and ductal wall enhancement during the PP between patients with acute inflammation and those who showed remission (p greater than 0.05). Hepatic parenchymal atrophy of the lesion was seen in 24 patients. Among these, PV stenosis was mild in five

  2. (Post-)genomics approaches in fungal research.

    Science.gov (United States)

    Aguilar-Pontes, María Victoria; de Vries, Ronald P; Zhou, Miaomiao

    2014-11-01

    To date, hundreds of fungal genomes have been sequenced and many more are in progress. This wealth of genomic information has provided new directions to study fungal biodiversity. However, to further dissect and understand the complicated biological mechanisms involved in fungal life styles, functional studies beyond genomes are required. Thanks to the developments of current -omics techniques, it is possible to produce large amounts of fungal functional data in a high-throughput fashion (e.g. transcriptome, proteome, etc.). The increasing ease of creating -omics data has also created a major challenge for downstream data handling and analysis. Numerous databases, tools and software have been created to meet this challenge. Facing such a richness of techniques and information, hereby we provide a brief roadmap on current wet-lab and bioinformatics approaches to study functional genomics in fungi.

  3. Entomopathogenic fungal endophytes

    Science.gov (United States)

    Fungal endophytes are quite common in nature and some of them have been shown to have adverse effects against insects, nematodes, and plant pathogens. An introduction to fungal endophytes will be presented, followed by a discussion of research aimed at introducing Beauveria bassiana as a fungal endo...

  4. Inoperable Pancreatic Cancer Patients Who Have Prolonged Survival Exhibit an Increased Risk of Cholangitis

    Science.gov (United States)

    Buxbaum, James L; Biggins, Scott W; Bagatelos, Karen C; Inadomi, John M; Ostroff, James W

    2012-01-01

    Context Endoscopically placed metal stents, which are patent for 4-9 months, have been the favored decompressive strategy for biliary obstruction due to inoperable pancreatic cancer in order to minimize interventions. However, in the past decade chemotherapeutic options have improved survival. This raises the question of whether metal stents will continue to be the optimal method of decompression. Objective We performed a study to determine the outcome of patients with non-operatively managed pancreatic adenocarcinoma with regards to the development of cholangitis. Design We reviewed all ERCP performed for malignant distal biliary obstruction in between December 1999 and December 2005 at University of California, San Francisco (UCSF). Patients Only patients who received chemotherapy for pancreatic adenocarcinoma were included. Patients who underwent surgical biliary diversion procedures were excluded. Primary outcome measurement The primary outcome was the development of cholangitis requiring hospitalization. Results Among 200 patients with malignant distal biliary obstruction who underwent endoscopic biliary decompression procedures, 54 met study criterion. Metal stents were employed in 90.7% of these cases. The median survival of this population was 12.7 months (range: 2.6-34.6 months). Only 3 of 26 patients (11.5%) surviving one year or less developed cholangitis compared to 13 of 28 (46.5%) who survived more than one year. Thus patients surviving greater than one year had a five fold increase in the odds of developing cholangitis (odds ratio: 4.92; P=0.017). Conclusions This cohort of inoperable pancreatic cancer patients undergoing chemotherapy survived longer than the expected patent period of metal stents employed for biliary decompression. The occurrence of cholangitis requiring hospitalization does increase markedly among long term survivors. PMID:21737900

  5. Mycoviruses : future therapeutic agents of invasive fungal infections in humans?

    NARCIS (Netherlands)

    van de Sande, W. W. J.; Lo-Ten-Foe, J. R.; van Belkum, A.; Netea, M. G.; Kullberg, B. J.; Vonk, A. G.

    Invasive fungal infections are relatively common opportunistic infections in immunocompromised patients and are still associated with a high mortality rate. Furthermore, these infections are often complicated by resistance or refractoriness to current antimicrobial agents. Therefore, an urgent need

  6. Fungal peritonitis in children on peritoneal dialysis.

    NARCIS (Netherlands)

    Raaijmakers, R.; Schroder, C.; Monnens, L.A.H.; Cornelissen, E.A.M.; Warris, A.

    2007-01-01

    Fungal peritonitis is a rare but serious complication in children on peritoneal dialysis (PD). In this study, risk factors were evaluated, and therapeutic measures were reviewed. A retrospective, multi-centre study was performed in 159 Dutch paediatric PD patients, between 1980 and 2005 (3,573 month

  7. Fungal peritonitis in children on peritoneal dialysis.

    NARCIS (Netherlands)

    Raaijmakers, R.; Schroder, C.; Monnens, L.A.H.; Cornelissen, E.A.M.; Warris, A.

    2007-01-01

    Fungal peritonitis is a rare but serious complication in children on peritoneal dialysis (PD). In this study, risk factors were evaluated, and therapeutic measures were reviewed. A retrospective, multi-centre study was performed in 159 Dutch paediatric PD patients, between 1980 and 2005 (3,573 month

  8. Abdominal Complications after Severe Burns

    Science.gov (United States)

    2009-05-01

    abdominal compartment syndrome, schemic bowel, biliary disease , peptic ulcer disease and astritis requiring laparotomy, small bowel obstruction, rimary fungal...complications in- luded trauma exploratory laparotomy, abdominal com- artment syndrome, ischemic bowel, biliary disease , peptic lcer disease and gastritis, large...because it was almost uniformly ssociated with serious lethal burns. This was in the period efore effective gastric acid control, explaining the high

  9. The Natural History of Inflammatory Bowel Disease and Primary Sclerosing Cholangitis after Liver Transplantation – a Single-Centre Experience

    Directory of Open Access Journals (Sweden)

    Karli J Moncrief

    2010-01-01

    Full Text Available OBJECTIVE: To describe the natural history of primary sclerosing cholangitis (PSC and inflammatory bowel disease (IBD after liver transplant, the predictors of PSC and IBD recurrence, and the interaction of these disease processes.

  10. The analysis of bacteria strains and sensitivity of bacteria to antibiotics in acute obstructive cholangitis with suppuration

    Institute of Scientific and Technical Information of China (English)

    顾彬

    2013-01-01

    Objective To analyze the changes of bacteria stains in acute obstructive cholangitis with suppuration(AOSC) and sensitivity of different bacteria strains to antibiotics in recent decade. Methods The data of bacterial

  11. Diagnosis of invasive fungal infections

    Directory of Open Access Journals (Sweden)

    Anna Maria Barbui

    2013-01-01

    Full Text Available A proper diagnostic strategy of invasive fungal infections (IFI is a very important component in the management of infectious complications in hematological patients. A good diagnostic approach should be adapted to the patient in relation to the underlying disease, stage of disease, localization of infection and immune status. None of the diagnostic markers can be entirely adopted for medical decision making, and sometimes it’s useful to use the combination of several microbiological tests.The diagnosis of IFI must therefore have a multidisciplinary approach that includes clinical suspicion, microbiological results and radiological evidence.

  12. Residual intrahepatic stones after percutaneous biliary extraction : longterm follow up of complications

    Energy Technology Data Exchange (ETDEWEB)

    Yoo, Seung Min; Shim, Hyung Jin; Lee, Hwa Yeon; Lim, Sang Jun; Park, Hyo Jin; Kim, Yang Soo; Choi, Young Hee [Chungang Univ. College of Medicine, Seoul (Korea, Republic of); Kwak, Byung Kuck [National Medical Center, Seoul (Korea, Republic of); Park, Ji Young [Sung Ae Hospital, Seoul (Korea, Republic of)

    1997-08-01

    To evaluate and compare the radiologic and clinical follow-up of complications between a group in whom stone removal after percutaneous biliary extraction had been complete, and a group in whom this had been incomplete. Twenty-two patients in whom stone removal had been incomplete, and 20 from whom stones had been completely removed were evaluated with particular attention to complications such as cholangitis, liver abscess, biliary sepsis, and pain. Cholangitis was diagnosed on the basis of typical clinical symptoms such as pain, high fever, jaundice and leukocytosis. Pain without other cholangitic symptoms was excluded. Liver abscess was diagnosed by percutaneous aspiration of pus, and biliary sepsis by bacterial growth on blood culture, or laboratory findings such as increased fibrinogen products, decreased fibrinogen, and increased prothrombin time with cholangitic symptoms. 'Complete removal' means no residual stones on follow-up sonogram and cholangiogram performed within three to seven days after pecutaneous biliary extraction. Mean follow-up period was 26.5 months in the incomplete removal group and 34.2 months in the complete removal group. In twelve of 22 patients (54.5%) in the incomplete removal group, complications occurred, as follows:cholangitis, ten cases (45.5%);liver absces, one (4.5%);biliary sepsis, one (4.5%);and pain, seven(31.8%). In contrast, only two of twenty patients (10%) in the complete removal group suffered complications, all of which involved the recurrence of stones in the common duct, and cholangitis. Complete removal of intrahepatic stones significantly helps to reduce the incidence of possible complications. Even in the case of an impacted stone, aggressive interventional procedures, aimed at complete removal, should be considered. If nonsurgical procedures fail, early partial hepatectomy should be considered, particulary for the stones localized in the left intrahepatic duct.

  13. Fungal infections in severe acute pancreatitis.

    Science.gov (United States)

    Kochhar, Rakesh; Noor, Mohd Talha; Wig, Jaidev

    2011-06-01

    Severe acute pancreatitis (SAP) is associated with significant morbidity and mortality. The majority of deaths related to SAP are the result of infectious complications. Although bacterial infections are most commonly encountered, fungal infections are increasingly being recognized. Candida is the most common fungal infection. The occurrence of fungal infection in patients with acute pancreatitis adversely affects the clinical course, leading to a higher incidence of systemic complications, and possibly mortality as well. Important risk factors for fungal infection in patients with acute pancreatitis include broad-spectrum antibiotics, prolonged hospitalization and surgical/endoscopic interventions, use of total parenteral nutrition, and mechanical ventilation. Patients with higher severity of pancreatitis are at a greater risk. The pathogenesis of fungal infection in patients with acute pancreatitis is multifactorial. Translocation of microorganisms across the gut epithelium, lymphocyte dysfunction, and the virulence of the invading microorganisms play important roles. Histological demonstration of fungi remains the gold standard of diagnosis, but a positive biopsy is rarely obtained. The role of biomarkers in the diagnosis is being investigated. As early diagnosis and treatment can lead to improved outcome, a high index of suspicion is required for prompt diagnosis. Limiting the use of broad-spectrum antibiotics, early introduction of enteral nutrition, and timely change of vascular catheters are important preventive strategies. The role of antifungal prophylaxis remains controversial. Surgical necrosectomy with antifungal therapy is the most widely used treatment approach. Clinical trials on antifungal prophylaxis are needed, and indications for surgical intervention need to be clearly defined.

  14. The challenges of liver transplantation in children with primary sclerosing cholangitis.

    Science.gov (United States)

    Venkat, Veena L; Ranganathan, Sarangarajan; Sindhi, Rakesh

    2015-03-01

    Primary sclerosing cholangitis (PSC) in children can progress to end-stage liver disease requiring liver transplantation. PSC poses many challenges beginning with evaluation and classification of ductal involvement and overlap syndromes, few options for medical management and unique risks in the post-transplant period. The construct that PSC may be an autoimmune disease is based on positive autoantibodies, association with inflammatory bowel disease, linkage to HLA type and overlap/autoimmune sclerosing cholangitis; however, PSC is not responsive to standard immunosuppression. Study of PSC and post-transplant outcomes in children may provide a unique background in which to study this challenging disease. This is particularly intriguing in the subset of patients diagnosed in the first decade of life, suggesting a strong link to predisposing genetic susceptibility and immune dysregulation. Long-term, multicenter effort is likely to be the only mechanism to study this rare disease in children and to improve outcomes in the future.

  15. Bacteremia Due to Arthrobacter creatinolyticus in an Elderly Diabetic Man with Acute Cholangitis.

    Science.gov (United States)

    Yamamoto, Kei; Hayakawa, Kayoko; Nagamatsu, Maki; Fujiya, Yoshihiro; Mawatari, Momoko; Kutsuna, Satoshi; Takeshita, Nozomi; Tamura, Saeko; Mezaki, Kazuhisa; Ohmagari, Norio

    2017-03-24

    An 87-year-old man with poorly controlled diabetic mellitus presented with fever, bedsores, and elevated hepatobiliary enzyme levels. He was diagnosed with bacteremia with acute cholangitis due to Arthrobacter species, which are Gram-positive, aerobic, catalase-positive, coryneform bacteria belonging to the family Microbacteriaceae. Doripenem and subsequencial sulbactam/ampicillin treatment were used for the acute cholangitis, and the bacteremia was treated with a 2-week course of vancomycin. The bacteremia was misidentified by the phenotyping assay (API Coryne test), but was identified as Arthrobacter creatinolyticus by 16S rRNA and matrix-assisted laser-desorption/ionization time-of-flight mass spectrometry. To our knowledge, this is the first report of a human case of A. creatinolyticus bacteremia.

  16. A case of eosinophilic cholangitis: Imaging findings of contrast-enhanced ultrasonography, cholangioscopy, and intraductal ultrasonography

    Institute of Scientific and Technical Information of China (English)

    Naoki Matsumoto; Masahiko Sugitani; Kiyoshi Yokoyama; Kazuhiko Nakai; Toshiki Yamamoto; Takeshi Otani; Masahiro Ogawa; Naohide Tanaka; Ariyoshi Iwasaki; Yasuyuki Arakawa

    2007-01-01

    A 38-year-old woman was referred to our institution due to epigastralgia. She presented with obstructive jaundice and eosinophilia. Endoscopic retrograde cholangiopancreatography showed diffuse narrowing from the distal common bile duct to the bifurcation of the hepatic ducts. An endoscopic plastic biliary stent was inserted; the specimen obtained from the common bile duct wall revealed dense infiltration by eosinophils.Treatment was started with prednisolone 60 mg daily.The patient's biliary stenosis and eosinophilia gradually improved. Eosinophilic infiltration in the lungs or stomach is relatively common, but it is rare in the common bile dud. Most of the reported cases of eosinophilic cholangitis presented with eosinophilia; our patient's eosinophil count was over 1000/mm3. Since our patient had allergies to pollen and house dust, a relationship between the allergies and the eosinophilic cholangitis was suspected, but no cause was identified.

  17. Severe infection with multidrug-resistant Salmonella choleraesuis in a young patient with primary sclerosing cholangitis

    Science.gov (United States)

    Ferstl, Philip G; Reinheimer, Claudia; Jozsa, Katalin; Zeuzem, Stefan; Kempf, Volkhard AJ; Waidmann, Oliver; Grammatikos, Georgios

    2017-01-01

    Massive global spread of multidrug-resistant (MDR) Salmonella spp. expressing extended-spectrum beta-lactamase (ESBL) and additional resistance to fluoroquinolones has often been attributed to high international mobility as well as excessive use of oral antibiotics in livestock farming. However, MDR Salmonella spp. have not been mentioned as a widespread pathogen in clinical settings so far. We demonstrate the case of a 25-year-old male with primary sclerosing cholangitis who tested positive for MDR Salmonella enterica serotype Choleraesuis expressing ESBL and fluoroquinolone resistance. The pathogen was supposedly acquired during a trip to Thailand, causing severe fever, cholangitis and pancreatitis. To our knowledge, this is the first report of Salmonella enterica serotype Choleraesuis in Europe expressing such a multidrug resistance pattern. ESBL resistance of Salmonella enterica spp. should be considered in patients with obstructive biliary tract pathology and travel history in endemic countries. PMID:28373776

  18. First case of IgG4-related sclerosing cholangitis associated with autoimmune hemolytic anemia.

    Science.gov (United States)

    Masutani, Hironori; Okuwaki, Kosuke; Kida, Mitsuhiro; Yamauchi, Hiroshi; Imaizumi, Hiroshi; Miyazawa, Shiro; Iwai, Tomohisa; Takezawa, Miyoko; Koizumi, Wasaburo

    2014-07-14

    To our knowledge, patients with immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) associated with autoimmune hemolytic anemia (AIHA) have not been reported previously. Many patients with IgG4-SC have autoimmune pancreatitis (AIP) and respond to steroid treatment. However, isolated cases of IgG4-SC are difficult to diagnose. We describe our experience with a patient who had IgG4-SC without AIP in whom the presence of AIHA led to diagnosis. The patient was a 73-year-old man who was being treated for dementia. Liver dysfunction was diagnosed on blood tests at another hospital. Imaging studies suggested the presence of carcinoma of the hepatic hilus and primary sclerosing cholangitis, but a rapidly progressing anemia developed simultaneously. After the diagnosis of AIHA, steroid treatment was begun, and the biliary stricture improved. IgG4-SC without AIP was thus diagnosed.

  19. Freshwater Fungal Infections

    Directory of Open Access Journals (Sweden)

    Dennis J. Baumgardner

    2017-01-01

    Full Text Available Fungal infections as a result of freshwater exposure or trauma are fortunately rare. Etiologic agents are varied, but commonly include filamentous fungi and Candida. This narrative review describes various sources of potential freshwater fungal exposure and the diseases that may result, including fungal keratitis, acute otitis externa and tinea pedis, as well as rare deep soft tissue or bone infections and pulmonary or central nervous system infections following traumatic freshwater exposure during natural disasters or near-drowning episodes. Fungal etiology should be suspected in appropriate scenarios when bacterial cultures or molecular tests are normal or when the infection worsens or fails to resolve with appropriate antibacterial therapy.

  20. Antibacterial Therapy of Acute Cholecystitis and Cholangitis (According to Tokyo Guidelines2013

    Directory of Open Access Journals (Sweden)

    Yu.M. Stepanov

    2015-06-01

    Antibiotics should be used wisely in antimicrobial therapy in each institution, region and country. The recent global spread of antibiotic resistance gives us a warning in the modern practice. Tokyo Guidelines 2013 provide practical guidance for physicians and surgeons involved in the treatment of community-acquired and hospital acute biliary infection. Much remains uncertain in this view. Continuous monitoring of local resistance to antibiotics and further studies in acute cholecystitis and cholangitis should be justified.

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

    Science.gov (United States)

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

    2015-05-01

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

  2. A case of cholestatic hepatitis associated with histologic features of acute cholangitis

    Directory of Open Access Journals (Sweden)

    Takeuchi H

    2011-11-01

    Full Text Available Hajime Takeuchi1, Toru Kaneko1, Toshikazu Otsuka1, Kumiko Tahara1, Tadashi Motoori2, Makoto Ohbu3, Masaya Oda4, Hiroaki Yokomori11Department of Internal Medicine; 2Division of Pathology, Kitasato Medical Center Hospital, Kitasato University, Saitama; 3Department of Pathology, School of Allied Health Sciences, Kitasato University, Sagamihara, Kanagawa; 4Department of Internal Medicine, Saitama Social Insurance Hospital, Saitama; 5Organized Center of Clinical Medicine, International University of Health and Welfare, Sanno Hospital, Tokyo, JapanAbstract: This report describes a case showing histologic features of acute cholangitis with an over-the-counter drug. A 48-year-old woman was diagnosed with general malaise and progressive jaundice. A thorough review of her medical history revealed that the patient had taken an over-the-counter drug, Pabron Gold®, which she had used previously, that may have caused liver injury. Laboratory investigations revealed jaundice and liver dysfunction. Endoscopic retrograde cholangiography detected no extrahepatic biliary duct dilatation or stones. Liver biopsy indicated acute cholangitis involving neutrophils and eosinophils. Electron microscopy revealed fragmented nuclei, indicating that the degenerative bile duct-related epithelial cells were in an apoptotic process.Keywords: liver injury, over-the-counter drug, histologic features, acute cholangitis, electron microscopy, Pabron Gold

  3. Mechanism Underlying Protective Effect of Danbiqing Granule on Experimental Acute Bacterial Cholangitis in Rabbits

    Institute of Scientific and Technical Information of China (English)

    LINXiu-zhen; GONGYan-ling; WANGHong-bo

    2003-01-01

    Aim To study the mechanism of protective effects of Danbiqing granule (DBQ) on experimental acute bacterial cholangitis in rabbits. Methods The acute bacterial cholangitis was induced by injecting 1 mL of 1 × l03 cuf·mL-1 Escherchia coli suspension into common bile duit. The serum nitrous oxide (NO) levels were measured using nitric acid reductase kit. Phospholipase A2 (PLA2) activity was assayed by a method of acid titration (microassay). Serum tumor necrosis factor-α (TNF-α), inferleukin-6 (IL-6) and plasma thromboxane B2 ( TXB2 ), 6-keto-platelet growth factor 1(PGF1α) were determined by radioimmunoassay. Results Compared with control group, serum NO, PLA2, TNF-α, IL-6 and plasma TXB2 levels increased significantly in model group ( P < 0.01) while those of DBQ groutm decreased significantly( P < 0.01). Conclusion DBQ dramatically inhibits the overproduction of pro-inflammatory factor PLA2 and inflammatory cytokine. Hence, the mechanism of DBQ underlying anti-inflammatory and protective effect against acute bacterial cholangitis in rabbits has been revealed.

  4. Radiological diagnosis of primary sclerosing cholangitis: value of ERC and CT; Radiologische Diagnostik der Primaer Sklerosierenden Cholangitis: Wertigkeit von ERC und CT

    Energy Technology Data Exchange (ETDEWEB)

    Kollmann, F.D. [Strahlenklinik und Poliklinik, Klinikum Rudolf Virchow, Freie Univ. Berlin (Germany); Maeurer, J. [Strahlenklinik und Poliklinik, Klinikum Rudolf Virchow, Freie Univ. Berlin (Germany); Hintze, R.E. [Chirurgische Klinik, Klinikum Rudolf Virchow, Freie Univ. Berlin (Germany); Adler, A. [Klinik fuer Innere Medizin mit Schwerpunkt Gastroenterologie, Klinikum Rudolf Virchow, Freie Univ. Berlin (Germany); Veltzke, W. [Klinik fuer Innere Medizin mit Schwerpunkt Gastroenterologie, Klinikum Rudolf Virchow, Freie Univ. Berlin (Germany); Lohmann, R. [Chirurgische Klinik, Klinikum Rudolf Virchow, Freie Univ. Berlin (Germany); Felix, R. [Strahlenklinik und Poliklinik, Klinikum Rudolf Virchow, Freie Univ. Berlin (Germany)

    1994-12-31

    To evaluate the use of computed tomography (CT) in primary sclerosing cholangitis (PSC) as compared to endoscopic-retrograde cholangiography (ERC), imaging studies of 24 patients were reviewed. 19 patients were studied by ERC, 19 by CT and 14 by both. In 17 cases, ERC confirmed PSC. One cholangiogram suggested a tumor. CT reflected PSC in only 11 cases, while three patients displayed a mass lesion. Thus, ERC remains the standard imaging technique for diagnosing PSC, whereas CT proves beneficial in excluding hepatic masses. (orig.) [Deutsch] Um den Stellenwert der endoskopisch-retrograden Cholangiographie (ERC) bei Primaer Sklerosierender Cholangitis (PSC) mit dem der Computertomographie (CT) zu vergleichen, wurden Untersuchungen von 24 Patienten mit der klinischen Verdachtsdiagnose PSC retrospektiv ausgewertet. 19 Patienten wurden mit ERC untersucht, 19 mit CT und 14 mit beiden Verfahren. Durch ERC konnte in 17 Faellen die Diagnose bestaetigt werden. In einem Patienten fand sich eine tumorverdaechtige Laesion. Im CT konnten nur in elf Faellen Gallengangsveraenderungen nachgewiesen werden, in drei Untersuchungen jedoch hepatische Raumforderungen. Die ERC bleibt damit die aussagekraeftigere Untersuchung zur Bestaetigung einer PSC-Verdachtsdiagnose, waehrend CT vor allem zur Tumordiagnostik bei PSC-Patienten indiziert ist. (orig.)

  5. 白血病化疗后粒细胞缺乏伴真菌感染患者抗菌药物的应用研究%Study on use of antimicrobial drugs by leukemia patients complicated with agranulocytosis and fungal infections after chemotherapy

    Institute of Scientific and Technical Information of China (English)

    古力巴旦木·艾则孜; 袁海龙; 曹海洲; 陈刚; 曲建华

    2016-01-01

    目的 探讨白血病化疗后粒细胞缺乏伴真菌感染患者抗菌药物的应用,为临床白血病治疗提供药物治疗依据.方法 回顾性分析2013年8月-2014年10月医院收治的120例白血病化疗后粒细胞缺乏伴真菌感染患者临床资料;所有中性粒细胞<0.5×109/L患者均应用粒细胞集落刺激因子,白血病粒细胞缺乏伴发热患者首先经验性抗细菌治疗,直至获得准确的病原学培养结果 选用抗菌药物;对疑似真菌感染的患者,先给予氟康唑治疗5~12 d;拟诊或临床诊断以及确诊真菌感染后分层使用抗真菌药物;调查患者治疗后临床症状改善情况,采用SPSS 20.0软件对数据进行统计分析.结果 120例感染患者检出真菌以白色假丝酵母菌为主,占60.8%;经抗真菌药物治疗后治疗有效112例,有效率为93.3%;治疗24~48 h后体温下降,临床症状有所改善;患者应用粒细胞集落刺激因子3~5 d后,血白细胞升至1.0×109/L以上,中性粒细胞升至0.5×109/L以上;8例患者因感染性休克死亡,病死率6.7%;16例发生不良反应,发生率13.3%.结论 白血病患者化疗后粒细胞缺乏伴真菌感染患者,应根据病原菌鉴定和药敏试验的结果 合理选择抗菌药物,并对真菌感染患者采用预防性抗真菌治疗.%OBJECTIVE To explore the use of antibiotics by leukemia patients complicated with agranulocytosis and fungal infections after chemotherapy so as to provide guidance for drug therapy .METHODS The clinical data of 120 leukemia patients complicated with agranulocytosis and fungal infections after chemotherapy who were treated in the hospital from Aug 2013 to Oct 2014 were retrospectively analyzed .The patients with neutrophils counts less than 0 .5 × 109/L were treated with granulocyte colony stimulating factors ,the leukemia agranulocytosis patients complicated with fever were firstly given the empirical antibacterial treatment and then treated with antibiotics till the

  6. Complications - National

    Data.gov (United States)

    U.S. Department of Health & Human Services — Complications - national data. This data set includes national-level data the hip/knee complication measure, and the Agency for Healthcare Research and Quality...

  7. Complications - State

    Data.gov (United States)

    U.S. Department of Health & Human Services — The Complications measures - state data. This data set includes state-level data for the hip/knee complication measure, and the Agency for Healthcare Research and...

  8. Complications - Hospital

    Data.gov (United States)

    U.S. Department of Health & Human Services — Complications - provider data. This data set includes provider data for the hip/knee complication measure, and the Agency for Healthcare Research and Quality (AHRQ)...

  9. Pregnancy Complications

    Science.gov (United States)

    ... To receive Pregnancy email updates Enter email Submit Pregnancy complications Complications of pregnancy are health problems that ... pregnancy. Expand all | Collapse all Health problems before pregnancy Before pregnancy, make sure to talk to your ...

  10. [Common bile duct stones and their complications].

    Science.gov (United States)

    Millat, B; Borie, F

    2000-12-01

    At the time of cholecystectomy for symptomatic cholelithiasis, 7-20% of patients have common bile duct stones. Nearly one third of them are asymptomatic. Routine cholangiography during cholecystectomy allows the diagnosis and treatment of common bile duct stones during the same operation. Selective indication for the diagnosis of common bile duct stones based on the positive predictive value of indicators limits treatment to symptomatic cases. No single indicator is however completely accurate in predicting common bile duct stones and the natural history of asymptomatic cases is uncertain. Endoscopic stone extraction preceding cholecystectomy is not superior to one-stage surgical treatment. Diagnosis and treatment of common bile duct stones are feasible laparoscopically. Complications of common bile duct stones are cholangitis and acute pancreatitis; if severe, they require specific therapeutic approaches.

  11. Cholangitis and multiple liver abscesses after percutaneous ethanol injection (PEI for recurrent hepatocellular carcinoma (HCC Colangitis y abscesos hepáticos múltiples tras la inyección percutánea de etanol (IPE en el tratamiento del carcinoma hepatocelular recurrente

    Directory of Open Access Journals (Sweden)

    Fernando Macias-García

    2013-02-01

    Full Text Available Percutaneous ablation procedures are minimally invasive treatments for unresectable early stage hepatocellular carcinoma (HCC. These techniques are usually safe, but rare and even fatal complications have been described. We present a fatal result after percutaneous ethanol injection (PEI for the treatment of a recurrent HCC in a non-cirrhotic liver, with subsequent development of diffuse cholangitis and multiple liver abscesses. Although percutaneous drainage and intensive antibiotic treatment were employed, the patient finally died. We discuss about the etiology and the physiopathology of this rare complication in which the therapeutic options are limited and usually unsuccessful.

  12. [Pulmonary fungal infection in patients with AIDS].

    Science.gov (United States)

    Denis, B; Lortholary, O

    2013-10-01

    Fungal infections are the most common opportunistic infections (OI) occurring during the course of HIV infection, though their incidence has decreased dramatically with the introduction of highly active antiretroviral therapy (cART). Most cases occur in untreated patients, noncompliant patients or patients whose multiple antiretroviral regimens have failed and they are a good marker of the severity of cellular immunodepression. Pneumocystis jiroveci pneumonia is the second most frequent OI in France and cryptococcosis remains a major problem in the Southern Hemisphere. With the increase in travel, imported endemic fungal infection can occur and may mimic other infections, notably tuberculosis. Fungal infections often have a pulmonary presentation but an exhaustive search for dissemination should be made in patients infected with HIV, at least those at an advanced stage of immune deficiency. Introduction of cART in combination with anti-fungal treatment depends on the risk of AIDS progression and on the risk of cumulative toxicity and the immune reconstitution inflammatory syndrome (IRIS) if introduced too early. Fungal infections in HIV infected patients remain a problem in the cART era. IRIS can complicate the management and requires an optimised treatment regime. Copyright © 2013. Published by Elsevier Masson SAS.

  13. Fungal Genomics Program

    Energy Technology Data Exchange (ETDEWEB)

    Grigoriev, Igor

    2012-03-12

    The JGI Fungal Genomics Program aims to scale up sequencing and analysis of fungal genomes to explore the diversity of fungi important for energy and the environment, and to promote functional studies on a system level. Combining new sequencing technologies and comparative genomics tools, JGI is now leading the world in fungal genome sequencing and analysis. Over 120 sequenced fungal genomes with analytical tools are available via MycoCosm (www.jgi.doe.gov/fungi), a web-portal for fungal biologists. Our model of interacting with user communities, unique among other sequencing centers, helps organize these communities, improves genome annotation and analysis work, and facilitates new larger-scale genomic projects. This resulted in 20 high-profile papers published in 2011 alone and contributing to the Genomics Encyclopedia of Fungi, which targets fungi related to plant health (symbionts, pathogens, and biocontrol agents) and biorefinery processes (cellulose degradation, sugar fermentation, industrial hosts). Our next grand challenges include larger scale exploration of fungal diversity (1000 fungal genomes), developing molecular tools for DOE-relevant model organisms, and analysis of complex systems and metagenomes.

  14. MicroRNAs in Serum and Bile of Patients with Primary Sclerosing Cholangitis and/or Cholangiocarcinoma.

    Directory of Open Access Journals (Sweden)

    Torsten Voigtländer

    Full Text Available Patients with primary sclerosing cholangitis (PSC are at high risk for the development of cholangiocarcinoma (CC. Analysis of micro ribonucleic acid (MiRNA patterns is an evolving research field in biliary pathophysiology with potential value in diagnosis and therapy. Our aim was to evaluate miRNA patterns in serum and bile of patients with PSC and/or CC.Serum and bile from consecutive patients with PSC (n = 40 (serum, n = 52 (bile, CC (n = 31 (serum, n = 19 (bile and patients with CC complicating PSC (PSC/CC (n = 12 (bile were analyzed in a cross-sectional study between 2009 and 2012. As additional control serum samples from healthy individuals were analyzed (n = 12. The miRNA levels in serum and bile were determined with global miRNA profiling and subsequent miRNA-specific polymerase chain reaction-mediated validation.Serum analysis revealed significant differences for miR-1281 (p = 0.001, miR-126 (p = 0.001, miR-26a (p = 0.001, miR-30b (p = 0.001 and miR-122 (p = 0.034 between patients with PSC and patients with CC. All validated miRNAs were significantly lower in healthy individuals. MiR-412 (p = 0.001, miR-640 (p = 0.001, miR-1537 (p = 0.003 and miR-3189 (p = 0.001 were significantly different between patients with PSC and PSC/CC in bile.Patients with PSC and/or CC have distinct miRNA profiles in serum and bile. Furthermore, miRNA concentrations are different in bile of patients with CC on top of PSC indicating the potential diagnostic value of these miRNAs.

  15. Clinical Feasibility and Usefulness of CT Fluoroscopy-Guided Percutaneous Transhepatic Biliary Drainage in Emergency Patients with Acute Obstructive Cholangitis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ji Hyung [Sam Anyang Hospital, Anyang (Korea, Republic of)

    2009-04-15

    To evaluate the feasibility of CT fluoroscopy (CTF)-guided percutaneous transhepatic biliary drainage (PTBD) in emergency patients with acute obstructive cholangitis. The study included 28 patients admitted to the emergency center due to obstructive jaundice and found to require urgent biliary drainage, as well as judged to have a suitable peripheral bile duct for a CTF-guided puncture (at least 4 mm in width). Prior to the CTF-guided puncture, a CT scan was performed to evaluate bile duct dilatation and the underlying causes of biliary obstruction. If the patient was judged to be a suitable candidate, a CTF-guided PTBD was performed in the same CT unit without additional fluoroscopic guidance. Technical feasibility of the procedure was investigated with the evaluation of overall success rate and causes of failure. A hepatic puncture was attempted at the left lobe in 23 patients and right lobe in five patients. The procedure was successful in 24 of 28 patients (86%) Successful biliary puncture was achieved on the first attempt in 16 patients, the second attempt in five patients, and the third attempt in three patients. The causes of failure included guide wire twisting in one patient, biliary puncture failure in two patients, and poor visualization of the guide wire in one patient. There were no significant procedure-related complication. The CTF-guided PTBD is technically feasible and highly successful in patients judged to have a suitable indication. Moreover, although the procedure is unfamiliar and inconvenient to interventionalists, it has economical advantages in that it saves time and manpower. We believe this method can be used in the emergency patients requiring urgent biliary drainage as an alternative for the fluoroscopy-guided PTBD.

  16. Fungal DNA barcoding.

    Science.gov (United States)

    Xu, Jianping

    2016-11-01

    Fungi are ubiquitous in both natural and human-made environments. They play important roles in the health of plants, animals, and humans, and in broad ecosystem functions. Thus, having an efficient species-level identification system could significantly enhance our ability to treat fungal diseases and to monitor the spatial and temporal patterns of fungal distributions and migrations. DNA barcoding is a potent approach for rapid identification of fungal specimens, generating novel species hypothesis, and guiding biodiversity and ecological studies. In this mini-review, I briefly summarize (i) the history of DNA sequence-based fungal identification; (ii) the emergence of the ITS region as the consensus primary fungal barcode; (iii) the use of the ITS barcodes to address a variety of issues on fungal diversity from local to global scales, including generating a large number of species hypothesis; and (iv) the problems with the ITS barcode region and the approaches to overcome these problems. Similar to DNA barcoding research on plants and animals, significant progress has been achieved over the last few years in terms of both the questions being addressed and the foundations being laid for future research endeavors. However, significant challenges remain. I suggest three broad areas of research to enhance the usefulness of fungal DNA barcoding to meet the current and future challenges: (i) develop a common set of primers and technologies that allow the amplification and sequencing of all fungi at both the primary and secondary barcode loci; (ii) compile a centralized reference database that includes all recognized fungal species as well as species hypothesis, and allows regular updates from the research community; and (iii) establish a consensus set of new species recognition criteria based on barcode DNA sequences that can be applied across the fungal kingdom.

  17. Oral Manifestations and Complications of Diabetes Mellitus

    Science.gov (United States)

    Al-Maskari, Awatif Y.; Al-Maskari, Masoud Y.; Al-Sudairy, Salem

    2011-01-01

    Diabetes mellitus is a chronic disease affecting all age groups. It is one of the leading causes of mortality and morbidity worldwide. Many chronic macrovascular and microvascular complications of diabetes have been reported in the literature with few reports about oral complications. This article aims to review and increase the awareness of oral manifestations and complications of diabetes mellitus and to stimulate research on the subject. It treats in depth some of the complications such as periodontal disease, fungal infection and salivary dysfunction while other complications are mentioned briefly. PMID:21969888

  18. 卡泊芬净治疗粒细胞缺乏血液病患者合并侵袭性真菌感染的临床效果%Clinical Effect of Caspofungin in the Treatment of Neutropenic Patients with Hematological Disease Complicated with Invasive Fungal Infection

    Institute of Scientific and Technical Information of China (English)

    肖晓芳; 王娟平; 陈喆

    2016-01-01

    目的:探讨卡泊芬净治疗粒细胞缺乏血液病患者合并侵袭性真菌感染的临床效果。方法:随机选取吉安市中心人民医院2012年3月至2016年3月收治的粒细胞缺乏血液病合并侵袭性真菌感染患者40例,依据治疗方法将这些患者分为卡泊芬净组(n=20)和伏立康唑组(n=20)两组,对两组患者的临床疗效、不良反应发生情况进行统计分析。结果:卡泊芬净组患者治疗的总有效率95.0%(19/20)显著高于伏立康唑组70.0%(14/20)(P<0.05),不良反应发生率5.0%(1/20)显著低于伏立康唑组20.0%(4/20)(P<0.05)。结论:卡泊芬净治疗粒细胞缺乏血液病患者合并侵袭性真菌感染的临床效果较伏立康唑好,值得在临床推广使用。%Objective: Study the clinical effect of caspofungin on neutropenic patients with hematological disease complicated with invasive fungal infection.Method: 40 cases granulocyte lack blood disease combined with the invasion of fungal infection inJi'an Central People's Hospital from March 2012 to March 2016 were randomly divided into two groups, the caspofungin group (n=20) and voriconazole group (n=20), and then the clinical curative effect of two groups of patients, adverse reactions occurrence were evaluated. Result:The caspofungin group with total efficiency of 95% (19/20) was significantly higher than that in the voriconazole group 70.0%(14/20) (P<0.05), and adverse reactions occurred rate of 5.0% (1/20) was significantly lower than that of voriconazole was 20.0% (4/20) (P<0.05).Conclusion: The clinical effect of caspofungin in the treatment of granulocyte lack blood disease patients with invasive fungal infection is better than voriconazole. Therefore, it is worth to popularize in the clinical use.

  19. Fungal arthritis and osteomyelitis.

    Science.gov (United States)

    Kohli, Rakhi; Hadley, Susan

    2005-12-01

    Fungal arthritis and osteomyelitis are uncommon diseases and generally present in an indolent fashion. The incidence of fungal bone and joint dis-ease is increasing with an increase in the prevalence of factors predisposing to invasive fungal disease, such as the use of central venous catheters, broad spectrum antibiotics, immunosuppression, and abdominal surgery. Definitive diagnosis relies on bone or synovial culture or biopsy. Successful management has traditionally consisted of amphotericin B in combination with surgical debridement. Given the rarity of this disease, treatment is not well defined, but reports of success with the use of azole antifungal agents, including itraconazole, fluconazole, voriconazole, and posaconazole, are promising.

  20. Sphenoidal fungal sinusitis with intracranial extension An interesting Case Report

    Directory of Open Access Journals (Sweden)

    Balasubramanian Thiagarajan

    2013-08-01

    Full Text Available Isolated sphenoid sinusitis is rather rare. Fungal sinusitis is common in immunocompromised patients. In this case report the authors describe an immunocompetent patient with isolated sphenoid fungal sinusitis with intracranial extension with a review of published literature.Unfavorable location and poor ventilation have been attributed as the probable factors involved in isolated sphenoid sinusitis. Considering the location of sphenoid sinus (close to skull base, optic nerve and great vessels infections involving this sinus is fraught with dangerous complications.

  1. Insect pathology and fungal entomopathogens

    Science.gov (United States)

    Fungi that occur inside asymptomatic plant tissues are known as fungal endophytes. Different genera of fungal entomopathogens have been reported as naturally occurring fungal endophytes, and it has been shown that it is possible to inoculate plants with fungal entomopathogens, making them endophytic...

  2. JGI Fungal Genomics Program

    Energy Technology Data Exchange (ETDEWEB)

    Grigoriev, Igor V.

    2011-03-14

    Genomes of energy and environment fungi are in focus of the Fungal Genomic Program at the US Department of Energy Joint Genome Institute (JGI). Its key project, the Genomics Encyclopedia of Fungi, targets fungi related to plant health (symbionts, pathogens, and biocontrol agents) and biorefinery processes (cellulose degradation, sugar fermentation, industrial hosts), and explores fungal diversity by means of genome sequencing and analysis. Over 50 fungal genomes have been sequenced by JGI to date and released through MycoCosm (www.jgi.doe.gov/fungi), a fungal web-portal, which integrates sequence and functional data with genome analysis tools for user community. Sequence analysis supported by functional genomics leads to developing parts list for complex systems ranging from ecosystems of biofuel crops to biorefineries. Recent examples of such 'parts' suggested by comparative genomics and functional analysis in these areas are presented here

  3. Fungal infections of the orbit

    Directory of Open Access Journals (Sweden)

    Bipasha Mukherjee

    2016-01-01

    Full Text Available Fungal infections of the orbit can lead to grave complications. Although the primary site of inoculation of the infective organism is frequently the sinuses, the patients can initially present to the ophthalmologist with ocular signs and symptoms. Due to its varied and nonspecific clinical features, especially in the early stages, patients are frequently misdiagnosed and even treated with steroids which worsen the situation leading to dire consequences. Ophthalmologists should be familiar with the clinical spectrum of disease and the variable presentation of this infection, as early diagnosis and rapid institution of appropriate therapy are crucial elements in the management of this invasive sino-orbital infection. In this review, relevant clinical, microbiological, and imaging findings are discussed along with the current consensus on local and systemic management. We review the recent literature and provide a comprehensive analysis. In the immunocompromised, as well as in healthy patients, a high index of suspicion must be maintained as delay in diagnosis of fungal pathology may lead to disfiguring morbidity or even mortality. Obtaining adequate diagnostic material for pathological and microbiological examination is critical. Newer methods of therapy, particularly oral voriconazole and topical amphotericin B, may be beneficial in selected patients.

  4. Silver-nanoparticle-coated biliary stent inhibits bacterial adhesion in bacterial cholangitis in swine

    Institute of Scientific and Technical Information of China (English)

    Wei Wen; Li-Mei Ma; Wei He; Xiao-Wei Tang; Yin Zhang; Xiang Wang; Li Liu; Zhi-Ning Fan

    2016-01-01

    BACKGROUND: One of the major limitations of biliary stents is the stent occlusion, which is closely related to the over-growth of bacteria. This study aimed to evaluate the feasibility of a novel silver-nanoparticle-coated polyurethane (Ag/PU) stent in bacterial cholangitis model in swine. METHODS: Ag/PU was designed by coating silver nanopar-ticles on polyurethane (PU) stent. Twenty-four healthy pigs with bacterial cholangitis using Ag/PU and PU stents were ran-domly divided into an Ag/PU stent group (n=12) and a PU stent group (n=12), respectively. The stents were inserted by standard endoscopic retrograde cholangiopancreatography. Laboratory assay was performed for white blood cell (WBC) count, alanine aminotransferase (ALT), interleukin-1β (IL-1β), tumor necrosis factor-α (TNF-α) at baseline time, 8 hours, 1, 2, 3, and 7 days after stent placements. The segment of bile duct containing the stent was examined histologically ex vivo. Implanted bili-ary stents were examined by a scan electron microscope. The amount of silver release was also measured in vitro. RESULTS: The number of inflammatory cells and level of ALT, IL-1β and TNF-α were significantly lower in the Ag/PU stent group than in the PU stent group. Hyperplasia of the mucosa was more severe in the PU stent group than in the Ag/PU stent group. In contrast to the biofilm of bacteria on the PU stent, fewer bacteria adhered to the Ag/PU stent. CONCLUSIONS: PU biliary stents modified with silver nanoparticles are able to alleviate the inflammation of pigs with bacterial cholangitis. Silver-nanoparticle-coated stents are resistant to bacterial adhesion.

  5. In vitro activity of moxifloxacin and piperacillin/sulbactam against pathogens of acute cholangitis

    Institute of Scientific and Technical Information of China (English)

    Andreas Weber; Wolfgang Huber; Klaus Kamereck; Philipp Winkle; Petra Voland; Hans Weidenbach; Roland M Schmid; Christian Prinz

    2008-01-01

    AIM:To analyze the in vitro activity of moxifioxacin and piperacillin/sulbactam against pathogens isolated from patients with acute cholangitis.METHODS:In this prospective study a total of 65 patients with acute cholangitis due to biliary stone obstruction (n = 7),benign biliary stricture (n =16),and malignant biliary stricture (n = 42) were investigated with regard to spectrum of bacterial infection and antibiotic resistance.Pathogens were isolated from bile cultures in all study patients.In 22 febrile patients,blood cultures were also obtained.In vitro activity of moxifloxacin and piperacillin/sulbactam was determined by agar diffusion.RESULTS:Thirty-one out of 65 patients had positive bile and/or blood cultures.In 31 patients,63 isolates with 17 different species were identified.The predominant strains were Enterococcus species (26/63),Ecoli (13/63) and Klebsiella species (8/63).A comparable in vitro activity of moxifloxacin and piperacillin/sulbactam was observed for E.coli and Klebsiella species.In contrast,Enterococcus species had higher resistances towards moxifloxacin.Overall bacteria showed antibiotic resistances in vitro of 34.9% for piperacillin/sulbactam and 36.5% for moxifloxacin.CONCLUSION:Enterococcus species,E.coli andKlebsiella species were the most common bacteria isolated from bile and/or blood from patients with acute cholangitis.Overall,a mixed infection with several species was observed,and bacteria showed a comparable in vitro activity for piperacillin/sulbactam and moxifloxacin.

  6. Systematic Biliary Sphincterotomy in Acute Gallstone Pancreatitis without Cholangitis?

    Directory of Open Access Journals (Sweden)

    Manley C Uy

    2009-11-01

    Full Text Available Drs. Shrode and Kahaleh [1] noted correctly that two out of the three mortalities in the early ERCP group of the Oria study [3] were not reported to be directly due to ERCP. However, it is worthwhile to take into consideration that one mortality was attributed to progressive respiratory failure despite early ERCP and sphincterotomy. Taking note that the incidence of severe and mild pancreatitis and other demographic and clinical characteristics were grossly the same in both groups, only one patient died in the early conservative group. Furthermore, both groups had the same number of patients undergoing surgery (45 early ERCP vs. 47 early conservative, respectively but nobody in the early conservative group died from biliary surgery as compared to the mortality in the early ERCP group which was attributed to elective biliary surgery. it was interesting that there was the absence of mortality in the early conservative group despite more complicated surgeries due to the larger number of patients necessitating transcystic ductal stone clearance and/or laparoscopic/open choledochotomy for main bile duct stones (one in the early ERCP vs. 19 in the early conservative management group. The large number of early conservative management patients with positive intraoperative cholangiography (40%, meaning persistent common bile duct stone, also raises doubts as to the need for early ERCP with sphincterotomy [3]. As to the study of Folsch et al. [4], although Drs. Shrode and Kahaleh [1] correctly noted that 22 patients in the early conservative management group required ERCP and that four patients died from cholecystitis and/or jaundice, they failed to note that there were fewer patients who died from respiratory and renal failure than in the early ERCP group. The two aforementioned organ failures are both possible complications of acute pancreatitis. This was in a background of the same demographic characteristics (including severity of pancreatitis [4

  7. Colorectal neoplasia in patients with primary sclerosing cholangitis undergoing liver transplantation

    DEFF Research Database (Denmark)

    Jørgensen, Kristin Kaasen; Lindström, Lina; Cvancarova, Milada

    2012-01-01

    is controversial. We aimed to compare the risk of colorectal neoplasia in PSC-IBD patients before and after Ltx and to identify risk factors for colorectal neoplasia post-transplant. MATERIAL AND METHODS: In a multicenter study within the Nordic Liver Transplant Group, we assessed the risk of colorectal neoplasia......OBJECTIVE: Several studies have implicated primary sclerosing cholangitis (PSC) as an additional risk factor for colorectal neoplasia in inflammatory bowel disease (IBD). Some reports have indicated that the risk is even higher in PSC-IBD patients after liver transplantation (Ltx), but this issue...

  8. Successful Treatment of Recurrent Primary Sclerosing Cholangitis after Orthotopic Liver Transplantation with Oral Vancomycin

    Directory of Open Access Journals (Sweden)

    Yinka K. Davies

    2013-01-01

    Full Text Available Primary sclerosing cholangitis (PSC is a progressive, cholestatic disease of the liver that is marked by inflammation of the bile ducts and damage to the hepatic biliary tree. Approximately 60–70% of patients also have inflammatory bowel disease and progression of PSC can lead to ulcerative colitis and cirrhosis of the liver. Due to limited understanding of the etiology and mechanism of PSC, the only existing treatment option is orthotopic liver transplantation (OLT; however, recurrence of PSC, after OLT is estimated to be between 5% and 35%. We discuss the successful treatment of a pediatric patient, with recurrent PSC, after OLT with oral Vancomycin.

  9. 胰十二指肠切除术后胆管炎%Cholangitis after pancreaticoduodenectomy

    Institute of Scientific and Technical Information of China (English)

    郭克建; 秦阳

    2002-01-01

    @@ 胰十二指肠切除术后胆管炎(cholangitis following pancreaticoduodencetomy, CPD)是一个易被人们忽视的少见并发症.与常见的急性胆管炎相比,CPD的症状较轻,加之接受了对机体影响较大的胰十二指肠切除术(PD),故难以推测这些不适的感觉究竟是由何种原因所致.

  10. Diagnostic approach using endosonography guided fine needle aspiration for lymphadenopathy in primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Shintaro Tsukinaga; Hisao Tajiri; Hiroo Imazu; Yujiro Uchiyama; Hiroshi Kakutani; Akira Kuramoti; Masayuki Kato; Keisuke Kanazawa; Tsuyoshi Kobayashi; Yasuyuki Searashi

    2007-01-01

    We report a case of primary sclerosing cholangitis (PSC) with benign lyphadenopathy which was diagnosed with endosonography guided fine needle aspiration (EUS-FNA).A 65-year-old woman was admitted to Jikei University Hospital with severe jaundice. Although endoscopic retrograde cholangiography and liver biopsy revealed the findings consistent with PSC, abdominal computed tomography revealed numerous large perihepatic lymph nodes with a maximum diameter of more than 3 cm.Therefore, EUS-FNA was done in order to exclude malignant lymphadenopathy, and adequate specimens obtained by EUS-FNA showed reactive hyperplasia of lymphnode. The patients were scheduled to undergo liver transplantation.

  11. MR cholangiography in the diagnosis of sclerosing cholangitis in Langerhans' cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Koenig, C.W.; Pfannenberg, C.; Truebenbach, J.; Remy, C.; Claussen, C.D. [Tuebingen Univ. (Germany). Abt. fuer Radiologische Diagnostik; Boehmer, G.M. [Dept. of Gastroenterology, Univ. of Tuebingen (Germany); Ruck, P. [Inst. of Pathology, Univ. of Tuebingen (Germany)

    2001-12-01

    Langerhans' cell histiocytosis (LCH) is a disorder of histiocytic proliferation that primarily affects infants. Imaging findings of a rare case of lung and liver involvement in an adult are presented. High-resolution computed tomography (HRCT) of the lungs showed confluent thin-walled cystic air spaces compatible with advanced LCH. Liver CT and MRI revealed unspecific signs of fatty infiltration. Irregular widening of peripheral bile ducts was displayed in breath-hold MR cholangiography. This pattern is considered characteristic for sclerosing cholangitis and should support the diagnosis of LCH in case of concomitant cystic pulmonary disease, even in adult patients. (orig.)

  12. [Pathogenesis of invasive fungal infections].

    Science.gov (United States)

    Garcia-Vidal, Carolina; Carratalà, Jordi

    2012-03-01

    Invasive fungal infections remain a life-threatening disease. The development of invasive fungal disease is dependent on multiple factors, such us colonization and efficient host immune response. We aimed to review the pathogenesis of invasive fungal infections, in particular, those caused by Candida and Aspergillus. For this we propose, to describe the fungal characteristics, to detail the host defence mechanisms against fungus and to analyse the host risk factors for invasive fungal infection.

  13. Cataract complications

    Directory of Open Access Journals (Sweden)

    David Yorston

    2008-03-01

    Full Text Available Any eye surgeon, no matter how experienced, will occasionally encounter a serious cataract complication. Although complications may be devastating for the patient and are always distressing for the surgeon, are they really a major issue for VISION 2020? The evidence says that they are.

  14. A Descriptive Study Of Foot Complications In Diabetic Patients With ...

    African Journals Online (AJOL)

    Symptomatic peripheral neuropathy in a diabetic patient may be associated with the ... skin and fungal infections were the most frequent lesions seen in diabetic ... Foot complications other than foot ulcers may occur in diabetic patients with ...

  15. Fungal endocarditis: current challenges.

    Science.gov (United States)

    Tattevin, Pierre; Revest, Matthieu; Lefort, Agnès; Michelet, Christian; Lortholary, Olivier

    2014-10-01

    Whilst it used to affect mostly intravenous drug users and patients who underwent valvular surgery with suboptimal infection control procedures, fungal endocarditis is now mostly observed in patients with severe immunodeficiency (onco-haematology), in association with chronic central venous access and broad-spectrum antibiotic use. The incidence of fungal endocarditis has probably decreased in most developed countries with access to harm-reduction policies (i.e. needle exchange programmes) and with improved infection control procedures during cardiac surgery. Use of specific blood culture bottles for diagnosis of fungal endocarditis has decreased due to optimisation of media and automated culture systems. Meanwhile, the advent of rapid techniques, including fungal antigen detection (galactomannan, mannan/anti-mannan antibodies and β-1,3-d-glucans) and PCR (e.g. universal fungal PCR targeting 18S rRNA genes), shall improve sensitivity and reduce diagnostics delays, although limited data are available on their use for the diagnosis of fungal endocarditis. New antifungal agents available since the early 2000s may represent dramatic improvement for fungal endocarditis: (i) a new class, the echinocandins, has the potential to improve the management of Candida endocarditis owing to its fungicidal effect on yeasts as well as tolerability of increased dosages; and (ii) improved survival in patients with invasive aspergillosis with voriconazole compared with amphotericin B, and this may apply to Aspergillus sp. endocarditis as well, although its prognosis remains dismal. These achievements may allow selected patients to be cured with prolonged medical treatment alone when surgery is considered too risky.

  16. Keratomycosis complicating pterygium excision.

    Science.gov (United States)

    Merle, Harold; Guyomarch, Jérôme; Joyaux, Jean-Christophe; Dueymes, Maryvonne; Donnio, Angélique; Desbois, Nicole

    2011-01-01

    The authors describe a case of keratomycosis that appeared after the exeresis of a pterygium. A 48-year-old patient had been referred with a red right eye associated with an abscess of the cornea along the ablation zone of the pterygium. The surgery had been performed a month beforehand. The abscess was 6 mm high and 4 mm wide. The authors instigated a treatment that included amphotericin B (0.25%) after noticing a clinical aspect evoking a fungal keratitis and finding several septate filaments on direct examination. On day 10, a Fusarium dimerum was isolated on Sabouraud agar. After 15 days of treatment, the result was favorable and the size of the ulceration as well as the size of the abscess had progressively decreased. The antifungal treatment was definitively stopped at 14 weeks. Infectious-related complications of the pterygium surgery are rare and are essentially caused by bacterial agents. Secondary infections by fungus are rare. There have been two previous cases reported: one that appeared 15 years after radiotherapy and another that appeared at 3 weeks post surgery, consecutive to the use of mitomycin C. To the authors' knowledge, this is the first case of a keratomycosis due to F. dimerum reported that complicated the exeresis of a pterygium without the use of an adjuvant antihealing treatment. Pterygium surgery is a common procedure; nevertheless, ophthalmologists need to be aware of the existence of potential infectious complications.

  17. Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis

    Directory of Open Access Journals (Sweden)

    P J Lorenzoni

    2011-01-01

    Full Text Available Myasthenia gravis (MG is an immune-mediated disease that compromises the postsynaptic membrane of the neuromuscular junction. Primary sclerosing cholangitis (PSC is considered an immune-mediated cholestatic liver disease. Both MG and PSC include an autoimmune pathogenesis, so there is some evidence that patients with MG or PSC have a higher risk of developing autoantibodies and other immune disorders than normal controls, but the coexistence of these two disorders has never been documented. We report a 40-year-old woman who presented with MG when she was 20 years old and developed PSC 20 years after a thymectomy. Liver biochemistry revealed cholestasis. Magnetic resonance imaging showed multifocal strictures and beads involving the intrahepatic bile ducts. A liver biopsy confirmed sclerosing cholangitis. Serological analysis demonstrated positive autoantibodies (Anti-nuclear antibodies, anti-smooth muscle antibodies. Repetitive stimulation had a decremental response, and antibodies to acetylcholine receptors were detectable. To our knowledge, this is the first case of PSC in a patient with MG. The main characteristics of both MG and PSC combination are discussed.

  18. A case of autoimmune cholangitis misdiagnosed for cholangiocarcinoma: How to avoid unnecessary surgical intervention?

    Directory of Open Access Journals (Sweden)

    Ignjatović Igor I.

    2015-01-01

    Full Text Available Introduction. Autoimmune cholangitis or immunoglobulin G4-associated cholangitis (IAC has been recently regarded as a new clinical and histopathological entity and is a part of a complex autoimmune disorder - IgG4-related systemic disease (ISD. ISD is an autoimmune disease with multi-organic involvement, characterized with IgG4-positive plasmocytic infiltration of various tissues and organs with a consequent sclerosis, which responds well to steroid therapy. Most commonly affected organs are the pancreas (autoimmune pancreatitis, [AIP] and the common bile duct (IAC. IAC and cholangiocarcinoma (CCA share many clinical, laboratory and imaging findings. Case Outline. We present a case of a 60-year-old male with a biliary stricture of a common bile duct, which was clinically considered as a bile duct carcinoma and treated surgically. Definite histopathological findings and immunohistochemistry revealed profound chronic inflammation, showing lymphoplasmacytic IgG-positive infiltration of a resected part of a common bile duct, highly suggestive for the diagnosis of IAC. In addition, postoperative IgG4 serum levels were also increased. Conclusion. It is of primary clinical importance to make a difference between IAC and CCA, in order to avoid unnecessary surgical intervention. Therefore, IAC should be considered in differential diagnosis in similar cases.

  19. Impaired Hepatic Adaptation to Chronic Cholestasis induced by Primary Sclerosing Cholangitis

    Science.gov (United States)

    Milkiewicz, Malgorzata; Klak, Marta; Kempinska-Podhorodecka, Agnieszka; Wiechowska-Kozlowska, Anna; Urasinska, Elzbieta; Blatkiewicz, Malgorzata; Wunsch, Ewa; Elias, Elwyn; Milkiewicz, Piotr

    2016-01-01

    Pathogenesis of primary sclerosing cholangitis (PSC) may involve impaired bile acid (BA) homeostasis. We analyzed expressions of factors mediating enterohepatic circulation of BA using ileal and colonic (ascending and sigmoid) biopsies obtained from patients with PSC with and without ulcerative colitis (UC) and explanted PSC livers. Two-fold increase of BA-activated farnesoid X receptor (FXR) protein levels were seen in ascending and sigmoid colon of PSC patients with correspondingly decreased apical sodium-dependent BA transporter (ASBT) gene expression. This was associated with increased OSTβ protein levels in each part of analyzed gut. An intestinal fibroblast growth factor (FGF19) protein expression was significantly enhanced in ascending colon. Despite increased hepatic nuclear receptors (FXR, CAR, SHP), and FGF19, neither CYP7A1 suppression nor CYP3A4 induction were observed. The lack of negative regulation of BA synthesis may be accountable for lower levels of cholesterol observed in PSC in comparison to primary biliary cholangitis (PBC). In conclusion, chronic cholestasis in PSC induces adaptive changes in expression of BA transporters and FXR in the intestine. However hepatic impairment of expected in chronic cholestasis downregulation of CYP7A1 and upregulation of CYP3A4 may promote BA-induced liver injury in PSC. PMID:28008998

  20. Complicated Pancreatitis

    NARCIS (Netherlands)

    Bakker, O.J.

    2015-01-01

    Research questions addressed in this thesis: What is the accuracy of serum blood urea nitrogen as early predictor of complicated pancreatitis? ; What is difference in clinical outcome between patients with pancreatic parenchymal necrosis and patients with extrapancreatic necrosis without necrosis

  1. Complicated Pancreatitis

    NARCIS (Netherlands)

    Bakker, O.J.

    2015-01-01

    Research questions addressed in this thesis: What is the accuracy of serum blood urea nitrogen as early predictor of complicated pancreatitis? ; What is difference in clinical outcome between patients with pancreatic parenchymal necrosis and patients with extrapancreatic necrosis without necrosis

  2. Diphtheria Complications

    Science.gov (United States)

    ... Search Form Controls Cancel Submit Search The CDC Diphtheria Note: Javascript is disabled or is not supported ... message, please visit this page: About CDC.gov . Diphtheria Home About Diphtheria Causes and Transmission Symptoms Complications ...

  3. Invasive fungal infections in acute leukemia.

    Science.gov (United States)

    Bhatt, Vijaya R; Viola, George M; Ferrajoli, Alessandra

    2011-08-01

    Invasive fungal infection (IFI) is among the leading causes for morbidity, mortality, and economic burden for patients with acute leukemia. In the past few decades, the incidence of IFI has increased dramatically. The certainty of diagnosis of IFI is based on host factors, clinical evidence, and microbiological examination. Advancement in molecular diagnostic modalities (e.g. non-culture-based serum biomarkers such as β-glucan or galactomannan assays) and high-resolution radiological imaging has improved our diagnostic approach. The early use of these diagnostic tests assists in the early initiation of preemptive therapy. Nonetheless, the complexity of IFI in patients with leukemia and the limitations of these diagnostic tools still mandate astute clinical acumen. Its management has been further complicated by the increasing frequency of infection by non-Aspergillus molds (e.g. zygomycosis) and the emergence of drug-resistant fungal pathogens. In addition, even though the antifungal armamentarium has expanded rapidly in the past few decades, the associated mortality remains high. The decision to initiate antifungal treatment and the choice of anti-fungal therapy requires careful consideration of several factors (e.g. risk stratification, local fungal epidemiologic patterns, concomitant comorbidities, drug-drug interactions, prior history of antifungal use, overall cost, and the pharmacologic profile of the antifungal agents). In order to optimize our diagnostic and therapeutic management of IFI in patients with acute leukemia, further basic research and clinical trials are desperately needed.

  4. Two cases of primary sclerosing cholangitis - ERCP-documentation in immunesuppressive therapy of the last 3 years

    Energy Technology Data Exchange (ETDEWEB)

    Mendel, R.; Droste, R.; Bade, J.; Hebestreit, H.P.; Georgi, M.

    1985-02-01

    X-ray characteristics of two cases of primary sclerosing cholangitis are described. A discussion of clinical, immunological and histological features of the disease and the 3-years progress of a 40 year old man during treatment with immunesuppressive therapy (Imurek at the beginning with cortisone) is shown. The arrest of the progression of the disease is supposed.

  5. Predictors of Successful Clinical and Laboratory Outcomes in Patients with Primary Sclerosing Cholangitis Undergoing Endoscopic Retrograde Cholangiopancreatography

    Directory of Open Access Journals (Sweden)

    R Enns

    2003-01-01

    Full Text Available Endoscopic retrograde cholangiopancreatography (ERCP in patients with primary sclerosing cholangitis (PSC can be a challenging and sometimes gratifying opportunity for therapeutic intervention. Although there often appears to be initial radiological improvement after ERCP, the benefit as measured by serial estimations of subsequent liver enzymes is questionable. The fluctuating course of the inflammatory process makes the interpretation of serology even more difficult.

  6. Extended analysis of a genome-wide association study in primary sclerosing cholangitis detects multiple novel risk loci

    NARCIS (Netherlands)

    Folseraas, Trine; Melum, Espen; Rausch, Philipp; Juran, Brian D.; Ellinghaus, Eva; Shiryaev, Alexey; Laerdahl, Jon K.; Ellinghaus, David; Schramm, Christoph; Weismueller, Tobias J.; Gotthard, Daniel Nils; Hov, Johannes Roksund; Clausen, Ole Petter; Weersma, Rinse K.; Janse, Marcel; Boberg, Kirsten Muri; Bjornsson, Einar; Marschall, Hanns-Ulrich; Cleynen, Isabelle; Rosenstiel, Philip; Holm, Kristian; Teufel, Andreas; Rust, Christian; Gieger, Christian; Wichmann, H-Erich; Bergquist, Annika; Ryu, Euijung; Ponsioen, Cyriel Y.; Runz, Heiko; Sterneck, Martina; Vermeire, Severine; Beuers, Ulrich; Wijmenga, Cisca; Schrumpf, Erik; Manns, Michael P.; Lazaridis, Konstantinos N.; Schreiber, Stefan; Baines, John F.; Franke, Andre; Karlsen, Tom H.

    Background & Aims: A limited number of genetic risk factors have been reported in primary sclerosing cholangitis (PSC). To discover further genetic susceptibility factors for PSC, we followed up on,a second tier of single nucleotide polymorphisms (SNPs) from a genome-wide association study (GWAS).

  7. Dense genotyping of immune-related disease regions identifies nine new risk loci for primary sclerosing cholangitis

    NARCIS (Netherlands)

    Liu, Jimmy Z.; Hov, Johannes Roksund; Folseraas, Trine; Ellinghaus, Eva; Rushbrook, Simon M.; Doncheva, Nadezhda T.; Andreassen, Ole A.; Weersma, Rinse K.; Weismueller, Tobias J.; Eksteen, Bertus; Invernizzi, Pietro; Hirschfield, Gideon M.; Gotthardt, Daniel Nils; Pares, Albert; Ellinghaus, David; Shah, Tejas; Juran, Brian D.; Milkiewicz, Piotr; Rust, Christian; Schramm, Christoph; Mueller, Tobias; Srivastava, Brijesh; Dalekos, Georgios; Noethen, Markus M.; Herms, Stefan; Winkelmann, Juliane; Mitrovic, Mitja; Braun, Felix; Ponsioen, Cyriel Y.; Croucher, Peter J. P.; Sterneck, Martina; Teufel, Andreas; Mason, Andrew L.; Saarela, Janna; Leppa, Virpi; Dorfman, Ruslan; Alvaro, Domenico; Floreani, Annarosa; Onengut-Gumuscu, Suna; Rich, Stephen S.; Thompson, Wesley K.; Schork, Andrew J.; Naess, Sigrid; Thomsen, Ingo; Mayr, Gabriele; Koenig, Inke R.; Hveem, Kristian; Cleynen, Isabelle; Gutierrez-Achury, Javier; Ricano-Ponce, Isis; van Heel, David; Bjoernsson, Einar; Sandford, Richard N.; Durie, Peter R.; Melum, Espen; Vatn, Morten H.; Silverberg, Mark S.; Duerr, Richard H.; Padyukov, Leonid; Brand, Stephan; Sans, Miquel; Annese, Vito; Achkar, Jean-Paul; Boberg, Kirsten Muri; Marschall, Hanns-Ulrich; Chazouilleres, Olivier; Bowlus, Christopher L.; Wijmenga, Cisca; Schrumpf, Erik; Vermeire, Severine; Albrecht, Mario; Rioux, John D.; Alexander, Graeme; Bergquist, Annika; Cho, Judy; Schreiber, Stefan; Manns, Michael P.; Farkkila, Martti; Dale, Anders M.; Chapman, Roger W.; Lazaridis, Konstantinos N.; Franke, Andre; Anderson, Carl A.; Karlsen, Tom H.

    Primary sclerosing cholangitis (PSC) is a severe liver disease of unknown etiology leading to fibrotic destruction of the bile ducts and ultimately to the need for liver transplantation(1-3). We compared 3,789 PSC cases of European ancestry to 25,079 population controls across 130,422 SNPs genotyped

  8. Reduced FOXP3(+) regulatory T cells in patients with primary sclerosing cholangitis are associated with IL2RA gene polymorphisms

    NARCIS (Netherlands)

    Sebode, M.; Peiseler, M.; Franke, B.; Schwinge, D.; Schoknecht, T.; Wortmann, F.; Quaas, A.; Petersen, B.S.; Ellinghaus, E.; Baron, U.; Olek, S.; Wiegard, C.; Weiler-Normann, C.; Lohse, A.W.; Herkel, J.; Schramm, C.

    2014-01-01

    BACKGROUND & AIMS: Recently, genome wide association studies in primary sclerosing cholangitis (PSC) revealed associations with gene polymorphisms that potentially could affect the function of regulatory T cells (Treg). The aim of this study was to investigate Treg in patients with PSC and to associ

  9. A morphological and immunohistochemical study of the effects of prednisolone or ursodeoxycholic acid on liver histology in feline lymphocytic cholangitis

    NARCIS (Netherlands)

    Otte, Corma Ma; Rothuizen, Jan; Favier, Robert P; Penning, Louis C; Vreman, Sandra

    2014-01-01

    Feline lymphocytic cholangitis (LC) has been commonly treated with prednisolone, and more recently with ursodeoxycholic acid (UDCA). Previously, we found that prednisolone treatment resulted in a statistically longer survival time than treatment with UDCA. In order to explain this difference, we

  10. Fluvoxamine for fatigue in primary biliary cirrhosis and primary sclerosing cholangitis: a randomised controlled trial [ISRCTN88246634].

    NARCIS (Netherlands)

    E. van Os (Erik); W.W. van den Broek (Walter); B.E. Hansen (Bettina); H.R. van Buuren (Henk); P.C.J. ter Borg (Pieter)

    2004-01-01

    textabstractBACKGROUND: Fatigue is a major clinical problem in many patients with primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). An effective treatment has not been defined. Recently, a large proportion of patients with these diseases was found to have sy

  11. Endotoxin adsorption therapy using polymyxin B-immobilized fiber as a treatment for septic shock-associated severe acute cholangitis.

    Science.gov (United States)

    Inoue, Yoshihiro; Fujino, Yasuhisa; Onodera, Makoto; Kikuchi, Satoshi; Takahashi, Gaku; Kojika, Masahiro; Endo, Shigeatsu

    2013-10-01

    The application of endotoxin adsorption therapy for severe acute cholangitis is controversial. We present a survival case of septic shock and multiple organ failure due to severe acute cholangitis. The patient was treated by endotoxin adsorption therapy using polymyxin B-immobilized fiber because he continued to remain in shock even after successful endoscopic nasobiliary drainage. The patient was an 84-year-old male diagnosed with acute cholangitis and acute pancreatitis who was transferred to our department because of shock and severe dyspnea. The patient had already developed acute respiratory failure, acute renal failure, and disseminated intravascular coagulation. We performed endoscopic nasobiliary drainage immediately, but the patient continued to remain in shock and plasma endotoxin level was markedly elevated at 133.6 pg/mL. Therefore, we performed direct hemoperfusion with polymyxin B-immobilized fiber. On starting the hemoperfusion, blood pressure and urine volume increased, and the plasma endotoxin level reduced considerably. On the basis of our experience in this case, we think that direct hemoperfusion with polymyxin B-immobilized fiber may be a useful modality in the management of severe acute cholangitis.

  12. Leukemoid reaction, a rare manifestation of autoimmune hemolytic anemia in a case of small duct primary sclerosing cholangitis.

    Science.gov (United States)

    Salagre, Kaustubh D; Sahay, Ravindra Nath; Patil, Anuja; Pati, Anuja; Joshi, Amita; Shukla, Akash

    2013-10-01

    A 48 year old lady presented with jaundice and exertional breathlesness. Her laboratory reports showed anaemia, reticulocytosis, leucocytosis, elevated Lactate Dehydrogenase (LDH), alkaline phosphatase levels, hyperbillirubinemia and positive direct Coomb's test. After ruling out all the other causes of autoimmunity and hemolytic anemia, she was diagnosed as leukemoid reaction due to autoimmune hemolytic anemia with primary sclerosing cholangitis. Patient showed immediate improvement after corticosteroids.

  13. Guideline for diagnosis, prophylaxis and treatment of invasive fungal infection post burn injury in China 2013

    Directory of Open Access Journals (Sweden)

    Gaoxing Luo

    2014-04-01

    Full Text Available Invasive fungal infection is one of the major complication of severe burns which can induce local or systemic inflammatory response and cause serious substantial damage to the patient. The incidence of fungal infection for burn victims is increasing dramatically during recent years. This guideline, organized by Chinese Society of Burn Surgeons, aims to standardize the diagnosis, prevention and treatment of burn invasive fungal infection. It can be used as one of the tools for treatment of major burn patients.

  14. Allergic Fungal Rhinosinusitis.

    Science.gov (United States)

    Hoyt, Alice E W; Borish, Larry; Gurrola, José; Payne, Spencer

    2016-01-01

    This article reviews the history of allergic fungal rhinosinusitis and the clinical, pathologic, and radiographic criteria necessary to establish its diagnosis and differentiate this disease from other types of chronic rhinosinusitis. Allergic fungal rhinosinusitis is a noninvasive fungal form of sinus inflammation characterized by an often times unilateral, expansile process in which the typical allergic "peanut-butter-like" mucin contributes to the formation of nasal polyps, hyposmia/anosmia, and structural changes of the face. IgE sensitization to fungi is a necessary, but not sufficient, pathophysiologic component of the disease process that is also defined by microscopic visualization of mucin-containing fungus and characteristic radiological imaging. This article expounds on these details and others including the key clinical and scientific distinctions of this diagnosis, the pathophysiologic mechanisms beyond IgE-mediated hypersensitivity that must be at play, and areas of current and future research.

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

    Science.gov (United States)

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

    2013-08-01

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

  16. Fungal Wound Infection

    Centers for Disease Control (CDC) Podcasts

    2016-01-28

    Dr. David Tribble, acting director of the infectious disease clinical research program at Uniformed Services University of the Health Sciences, discusses fungal wound infections after combat trauma.  Created: 1/28/2016 by National Center for Emerging and Zoonotic Infectious Diseases (NCEZID).   Date Released: 1/28/2016.

  17. Fungal pathogens of Proteaceae

    NARCIS (Netherlands)

    Crous, P.W.; Summerell, B.A.; Swart, L.; Denman, S.; Taylor, J.E.; Bezuidenhout, C.M.; Palm, M.E.; Marincowitz, S.; Groenewald, J.Z.

    2011-01-01

    Species of Leucadendron, Leucospermum and Protea (Proteaceae) are in high demand for the international floriculture market due to their brightly coloured and textured flowers or bracts. Fungal pathogens, however, create a serious problem in cultivating flawless blooms. The aim of the present study

  18. Fungal pathogens of Proteaceae

    NARCIS (Netherlands)

    Crous, P.W.; Summerell, B.A.; Swart, L.; Denman, S.; Taylor, J.E.; Bezuidenhout, C.M.; Palm, M.E.; Marincowitz, S.; Groenewald, J.Z.

    2012-01-01

    Species of Leucadendron, Leucospermum and Protea (Proteaceae) are in high demand for the international floriculture market due to their brightly coloured and textured flowers or bracts. Fungal pathogens, however, create a serious problem in cultivating flawless blooms. The aim of the present study

  19. Management of biliary and duodenal complications of chronic pancreatitis.

    Science.gov (United States)

    Vijungco, Joseph D; Prinz, Richard A

    2003-11-01

    Biliary stricture and duodenal obstruction have been increasingly recognized as complications of chronic pancreatitis. The anatomical relationship of the distal common bile duct and the duodenum with the head of the pancreas is the main factor for their involvement in chronic pancreatitis. In hospitalized patients with pancreatitis, the incidence of biliary stricture and duodenal obstruction is reported to be about 6% and 1.2%, respectively. For patients requiring an operation for chronic pancreatitis the incidence increases to 35% for biliary stricture and 12% for duodenal obstruction. Fibrosis around the distal common bile duct can cause stenosis with obstruction of bile flow. Clinically, the presentation of these patients ranges from being asymptomatic with elevated alkaline phosphatase or bilirubin, or both, to being septic with cholangitis. Jaundice, cholangitis, hyperbilirubinemia, and persistent elevation of serum alkaline phosphatase occur more frequently in patients with pancreatitis with a biliary stricture. A twofold elevation of alkaline phosphatase is a marker of possible common duct stenosis in patients with chronic pancreatitis. The incidence of both biliary cirrhosis and cholangitis in these patients is about 10%. ERCP reveals a characteristic long, smoothly tapered stricture of the intrapancreatic common bile duct. In duodenal obstruction, the factors that convert self-limiting edema to chronic fibrosis and stricture formation are unknown, but ischemia superimposed on inflammation may be the major cause. These patients present with a prolonged history of nausea and vomiting. Barium studies typically show a long constricting lesion of the duodenum, and endoscopy reveals reactive inflammatory changes in a narrowed duodenum. Operation is indicated in patients with common bile duct strictures secondary to chronic pancreatitis when there is evidence of cholangitis, biliary cirrhosis, common duct stones, progression of stricture, elevation of alkaline

  20. Complicated rhinosinusitis

    NARCIS (Netherlands)

    Hansen, F.S.

    2016-01-01

    Complicated rhinosinusitis: a title chosen for its multi-interpretable nature. In the Oxford dictionary ‘complicated’ is defined as ‘consisting of many interconnecting parts or elements’ and ‘involving many different and confusing aspects’ as well as ‘involving complications’ in medicine. It is the

  1. Complications and management of forgotten long-term biliary stents.

    Science.gov (United States)

    Sohn, Se Hoon; Park, Jae Hyun; Kim, Kook Hyun; Kim, Tae Nyeun

    2017-01-28

    To evaluate complications and management outcomes of retained long-term plastic biliary stents. Endoscopic plastic biliary stent placement was performed in 802 patients at Yeungnam University Hospital between January 2000 and December 2014. Follow-up loss with a subsequently forgotten stent for more than 12 mo occurred in 38 patients. We retrospectively examined the cause of biliary stent insertion, status of stents, complications associated with biliary stents and management outcomes of long-term plastic biliary stents. Continuous variables were analyzed using the t test. Observed frequencies in subsets of the study population were compared using Fisher's exact test and χ(2) tests. Statistical significance was defined as P < 0.05 (two-tailed). Mean age of patients was 73.7 ± 12 years and male-to-female ratio was 2.2:1. Indications of plastic biliary stent insertion were bile duct stones (63.2%, 24/38) and benign bile duct stricture (52.6%, 20/38). Mean duration of retained plastic stent was 22.6 ± 12.2 mo, and in 10 cases (26.3%), stents were retained for more than 24 mo. Common bile duct (CBD) stones or sludge were found in most cases (92.1%, 35/38). The most common complication was acute cholangitis (94.7%, 36/38). Stent removal by endoscopic approach was successfully performed in 92.1% (35/38) of the cases. In 3 cases, an additional plastic stent was inserted alongside the previous stent due to failure of the stent removal. Endoscopic removal of bile duct stones was successful in 73.7% (28/38) of the cases. When patients were divided into two groups by duration of stent placement (12 to 24 mo vs over 24 mo), there were no differences in the development of cholangitis, presence of biliary stones, and success rate of endoscopic removal of stones and biliary stents. The most common complication of retained long-term plastic biliary stents was acute cholangitis associated with CBD stones. Endoscopic management was successfully performed in most cases.

  2. 卡泊芬净治疗恶性血液病化疗后中性粒细胞缺乏合并深部真菌感染的临床观察%Clinical study on neutropenia complicated with deep fungal infection in patients with malignant hematological diseases after chemotherapy.

    Institute of Scientific and Technical Information of China (English)

    汪玉芳; 陈世明

    2013-01-01

    目的 分析卡泊芬净治疗恶性血液病化疗后中性粒细胞缺乏合并深部真菌感染临床效果及安全性.方法 选取2009年3月至2012年5月收治恶性血液病化疗后中性粒细胞缺乏合并深部真菌感染患者50例,采用随机数字表法分为两性霉素B组和卡泊芬净组,分别采用两性霉素B和卡泊芬净静脉滴注治疗;比较两组患者临床治疗总有效率及不良反应发生率等.结果 两性霉素B组总有效率(72.0%)与卡泊芬净组患者(76.0%)比较无显著差异(P>0.05);两性霉素B组不良反应发生率(36.0%)明显高于卡泊芬净组(P 0.05 ). The incidence rate of adverse reactions in caspofungin group ( 0% )was significantly lower than that of amphotericin B group( 36.0% )( P >0.05 ). Conclusion Caspofungin in treatment of deep fungal infection complicated with neutropenia in patients with malignant hematological diseases after chemotherapy has perfect therapeutic efficacy and few adverse reactions.

  3. Oral Vancomycin Therapy in a Child with Primary Sclerosing Cholangitis and Severe Ulcerative Colitis

    Science.gov (United States)

    Buness, Cynthia; Miloh, Tamir

    2016-01-01

    Primary sclerosing cholangitis (PSC), a rare progressive liver disease characterized by cholestasis and bile duct fibrosis, has no accepted, effective therapy known to delay or arrest its progression. We report a 15 year old female patient diagnosed with PSC and moderate chronic active ulcerative colitis (UC) who achieved normalization of her liver enzymes and bile ducts, and resolution of her UC symptoms with colonic mucosal healing, after treatment with a single drug therapy of the antibiotic oral vancomycin. We postulate that the oral vancomycin may be acting both as an antibiotic by altering the intestinal microbiome and as an immunomodulator. Oral vancomycin may be a promising treatment for PSC that needs to be further studied in randomized trials. PMID:27738604

  4. Pathogenesis of primary sclerosing cholangitis and advances in diagnosis and management.

    Science.gov (United States)

    Eaton, John E; Talwalkar, Jayant A; Lazaridis, Konstantinos N; Gores, Gregory J; Lindor, Keith D

    2013-09-01

    Primary sclerosing cholangitis (PSC), first described in the mid-1850s, is a complex liver disease that is heterogeneous in its presentation. PSC is characterized by chronic cholestasis associated with chronic inflammation of the biliary epithelium, resulting in multifocal bile duct strictures that can affect the entire biliary tree. Chronic inflammation leads to fibrosis involving the hepatic parenchyma and biliary tree, which can lead to cirrhosis and malignancy. The etiology of PSC is not fully understood, which in part explains the lack of effective medical therapy for this condition. However, we have begun to better understand the molecular pathogenesis of PSC. The recognition of specific clinical subtypes and their pattern of progression could improve phenotypic and genotypic classification of the disease. We review our current understanding of this enigmatic disorder and discuss important topics for future studies.

  5. Ulcerative Colitis, Autoimmune Hemolytic Anemia and Primary Sclerosing Cholangitis in a Child

    Directory of Open Access Journals (Sweden)

    Susan M Gilmour

    1996-01-01

    Full Text Available A 15-month-old female who initially presented with autoimmune hemolytic anemia (AIHA is described. She developed bloody stools and was diagnosed with ulcerative colitis (UC. Investigations of persistent hepatomegaly revealed primary sclerosing cholangitis (PSC. The association of AIHA, UC and PSC has never been reported. All these conditions entail impaired immunoregulation. Patients with a clustering of autoimmune diseases may help to delineate the pathogenesis of UC. Autoimmune phenomena may be prominent in inflammatory bowel disease. UC, in particular, exhibits a high incidence of associated autoimmune diseases including hypothyroidism, PSC, vitiligo and alopecia areata. AIHA is well described in 0.5% to 1.0% of adult UC patients but has not been reported in children with UC.

  6. Primary sclerosing cholangitis, Crohn's disease and HLA-B27 in black South African women.

    Science.gov (United States)

    Buchel, O C; Bosch, F J; Janse van Rensburg, J; Bezuidenhout, E; de Vries, C S; van Zyl, J H; Middlecote, B D; de K Grundling, H; Fevery, J

    2012-12-01

    Crohn's disease is rare in South African black people and primary sclerosing cholangitis (PSC) is also rare in black patients with IBD, from South Africa. The presence of HLA-B27 is generally associated with seronegative spondylo-arthropathies and correlates with the occurrence of ankylosing spondylitis, recurrent mouth ulcers and uveitis, in patients with IBD. We describe two women with the combination of Crohn's disease, PSC and HLA-B27 from our cohort of the last 5 years of three black patients with Crohn's disease. Crohn's disease, PSC and HLA-B27 respectively, occur rarely in black South Africans and their concurrent presence in two black women suggests a pathogenetic link of HLA-B27 between Crohn's disease and PSC in this population. Female gender might be an additional determinant in this setting.

  7. Chemokine and chemokine receptors in autoimmunity: the case of primary biliary cholangitis.

    Science.gov (United States)

    Choi, Jinjung; Selmi, Carlo; Leung, Patrick S C; Kenny, Thomas P; Roskams, Tania; Gershwin, M Eric

    2016-06-01

    Chemokines represent a major mediator of innate immunity and play a key role in the selective recruitment of cells during localized inflammatory responses. Beyond critical extracellular mediators of leukocyte trafficking, chemokines and their cognate receptors are expressed by a variety of resident and infiltrating cells (monocytes, lymphocytes, NK cells, mast cells, and NKT cells). Chemokines represent ideal candidates for mechanistic studies (particularly in murine models) to better understand the pathogenesis of chronic inflammation and possibly become biomarkers of disease. Nonetheless, therapeutic approaches targeting chemokines have led to unsatisfactory results in rheumatoid arthritis, while biologics against pro-inflammatory cytokines are being used worldwide with success. In this comprehensive review we will discuss the evidence supporting the involvement of chemokines and their specific receptors in mediating the effector cell response, utilizing the autoimmune/primary biliary cholangitis setting as a paradigm.

  8. Biliary Cast Formation with Sclerosing Cholangitis in Critically Ill Patient: Case Report and Literature Review

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, O Nyoung; Park, Chang Keun [Daegu Fatima Hospital, Daegu (Korea, Republic of); Cho, Seung Hyun [Kyungpook National University, School of Medicine, Daegu (Korea, Republic of); Mun, Sung Hee [Catholic University of Daegu, School of Medicine, Daegu (Korea, Republic of)

    2012-06-15

    Sclerosing cholangitis in critically ill patients (SC-CIP) is a rare condition that is not familiar to many radiologists. In addition, the associated imaging findings have not been described in the radiological literature. We report a case of biliary cast formation with SC-CIP and describe the radiological findings of CT, magnetic resonance cholangiopancreatography (MRCP), and endoscopic retrograde cholangiography (ERC). A diagnosis of SC-CIP should be considered in intensive care unit (ICU) patients with persistent cholestasis during or after a primary illness. The typical CT, MRCP and ERC findings include new biliary casts in the intrahepatic duct with multiple irregular strictures, dilatations, and relative sparing of the common bile duct.

  9. 急性白血病化疗后粒细胞缺乏患者深部真菌感染临床分析%Clinical analysis of neutropenia complicated by deep fungal infections in acute leukemia patients after chemotherapy

    Institute of Scientific and Technical Information of China (English)

    王羽; 展新荣; 张彦平

    2013-01-01

    OBJECTIVE To investigate and compare the therapeutic effects and security of amphotericin B and caspofungin on treatment of neutropenia complicated by deep fungal infections after chemotherapy for acute leukemia.METHODS A total of 60 patients with neutropenia complicated by deep fungal infection after chemotherapy of acute leukemia were chosen in the period from Jan 2010 to Mar 2012 and were randomly divided into two groups including the amphotericin B group (30 patients) with amphotericin B treatment by intravenous infusion for initial dose of 5 mg/d and maintenance dose of 30 mg/d and the caspofungin group (30 patients) with caspofungin treatment by intravenous infusion for initial dose of 80 mg/d and maintenance dose of 40 mg/d,then the total effective rate of the clinical treatment and the incidence of adverse reactions were compared between the two groups.RESULTS The total effective rate of the clinical treatment of the amphotericin B group and the caspofungin group were separately 73.3 % and 76.7 %,the difference was not statistically significant; the incidence rates of the adverse reactions including high fever and chills,hypokalemia,pain,elevation of alanine aminotransferase,and elevation of serum creatinine were separately 16.7%,3.3%,9.9%,3.3%,and 3.3% in the amphotericin B group and were separately 0,0,0,3.3 %,and 0 % in the caspofungin group,the incidence of the adverse reactions of the caspofungin group was significantly lower than that of the amphotericin B group,the difference was statistically significant(P<0.05).CONCLUSION Compared with the treatment with amphotericin B,the caspofungin can efficiently improve clinical symptoms,kill fungi and have few adverse reactions in the treatment of neutropenia complicated hy deep fungal infections after chemotherapy of acute leukemia.%目的 探讨两性霉素B与卡泊芬净治疗急性白血病化疗后中性粒细胞缺乏合并深部真菌感染临床疗效及安全性,为

  10. Quantitation of the Rank-Rankl Axis in Primary Biliary Cholangitis

    Science.gov (United States)

    Zhang, Haiyan; Miao, Qi; Yang, Fang; Peng, Yanshen; Chen, Xiaoyu; Tang, Ruqi; Wang, Qixia; Qiu, Dekai; Fang, Jingyuan; Sobacchi, Cristina; Villa, Anna; Di Tommaso, Luca; Roncalli, Massimo; Gershwin, M. Eric; Ma, Xiong; Invernizzi, Pietro

    2016-01-01

    There is substantial data that suggests an abnormality of innate immunity in patients with primary biliary cholangitis (PBC) which includes the transcription factor nuclear factor-kB (NF-kB) and well as downstream inflammatory signaling pathways. In addition, ImmunoChip analysis has identified a novel PBC-associated locus near the receptor activator of NF-kB ligand (RANKL) gene. Based on these observations, we investigated the role of the RANKL axis in the liver of patients with PBC compared to controls. We used immunohistochemistry to quantitate liver expression of RANKL, its receptor (RANK), and importantly the decoy receptor osteoprotegerin (OPG), including a total of 122 liver samples (PBC = 37, primary sclerosing cholangitis = 20, autoimmune hepatitis = 26, chronic hepatitis B = 32 and unaffected controls = 7). In addition, we studied RANKL-RANK-OPG co-localization in CD4 and CD8 T cells, B cells, dendritic cells, macrophages, NK, NKT cells, hepatocytes, and cholangiocytes. We report herein that RANK is constitutively expressed by cholangiocytes in both unaffected and diseased liver. However, cholangiocytes from PBC express significantly higher levers of RANK than either the unaffected controls or liver diseased controls. CD4, CD8 and CD19 cells with in the portal areas around bile ducts in PBC express significantly higher levels of RANKL compared to controls. Importantly, the overall hepatic RANKL level and the ratio of hepatic RANKL/OPG correlated with disease severity in PBC. In conclusion, our data indicate a role of RANK-RANKL axis in the innate immune activation in PBC and we hypothesize that the damaged cholangiocytes, which express high levels of RANK, lead to the recruitment of RANKL positive cells and ultimately the classic portal tract infiltrates. PMID:27631617

  11. Role of endoscopy in the diagnosis of autoimmune pancreatitis and immunoglobulin G4-related sclerosing cholangitis.

    Science.gov (United States)

    Kamisawa, Terumi; Ohara, Hirotaka; Kim, Myung Hwan; Kanno, Atsushi; Okazaki, Kazuichi; Fujita, Naotaka

    2014-09-01

    Autoimmune pancreatitis (AIP) must be differentiated from pancreatic carcinoma, and immunoglobulin (Ig)G4-related sclerosing cholangitis (SC) from cholangiocarcinoma and primary sclerosing cholangitis (PSC). Pancreatographic findings such as a long narrowing of the main pancreatic duct, lack of upstream dilatation, skipped narrowed lesions, and side branches arising from the narrowed portion suggest AIP rather than pancreatic carcinoma. Cholangiographic findings for PSC, including band-like stricture, beaded or pruned-tree appearance, or diverticulum-like outpouching are rarely observed in IgG4-SC patients, whereas dilatation after a long stricture of the bile duct is common in IgG4-SC. Transpapillary biopsy for bile duct stricture is useful to rule out cholangiocarcinoma and to support the diagnosis of IgG4-SC with IgG4-immunostaining. IgG4-immunostaining of biopsy specimens from the major papilla advances a diagnosis of AIP. Contrast-enhanced endoscopic ultrasonography (EUS) and EUS elastography have the potential to predict the histological nature of the lesions. Intraductal ultrasonographic finding of wall thickening in the non-stenotic bile duct on cholangiography is useful for distinguishing IgG4-SC from cholangiocarcinoma. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) is widely used to exclude pancreatic carcinoma. To obtain adequate tissue samples for the histological diagnosis of AIP, EUS-Tru-cut biopsy or EUS-FNA using a 19-gauge needle is recommended, but EUS-FNA with a 22-gauge needle can also provide sufficient histological samples with careful sample processing after collection and rapid motion of the FNA needles within the pancreas. Validation of endoscopic imaging criteria and new techniques or devices to increase the diagnostic yield of endoscopic tissue sampling should be developed.

  12. Toxicogenomic analysis reveals profibrogenic effects of trichloroethylene in autoimmune-mediated cholangitis in mice.

    Science.gov (United States)

    Kopec, Anna K; Sullivan, Bradley P; Kassel, Karen M; Joshi, Nikita; Luyendyk, James P

    2014-10-01

    Epidemiological studies suggest that exposure to environmental chemicals increases the risk of developing autoimmune liver disease. However, the identity of specific chemical perpetrators and the mechanisms whereby environmental chemicals modify liver disease is unclear. Previous studies link exposure to trichloroethylene (TCE) with the development of autoimmune liver disease and exacerbation of autoimmunity in lupus-prone MRL mice. In this study, we utilized NOD.c3c4 mice, which spontaneously develop autoimmune cholangitis bearing resemblance to some features of primary biliary cirrhosis. Nine-week-old female NOD.c3c4 mice were given TCE (0.5 mg/ml) or its vehicle (1% Cremophor-EL) in drinking water for 4 weeks. TCE had little effect on clinical chemistry, biliary cyst formation, or hepatic CD3+ T-cell accumulation. Hepatic microarray profiling revealed a dramatic suppression of early growth response 1 (EGR1) mRNA in livers of TCE-treated mice, which was verified by qPCR and immunohistochemical staining. Consistent with a reported link between reduced EGR1 expression and liver fibrosis, TCE increased hepatic type I collagen (COL1A1) mRNA and protein levels in livers of NOD.c3c4 mice. In contrast, TCE did not increase COL1A1 expression in NOD.ShiLtJ mice, which do not develop autoimmune cholangitis. These results suggest that in the context of concurrent autoimmune liver disease with a genetic basis, modification of hepatic gene expression by TCE may increase profibrogenic signaling in the liver. Moreover, these studies suggest that NOD.c3c4 mice may be a novel model to study gene-environment interactions critical for the development of autoimmune liver disease.

  13. Keratomycosis complicating pterygium excision

    Directory of Open Access Journals (Sweden)

    Merle H

    2011-10-01

    Full Text Available Harold Merle1, Jérôme Guyomarch1, Jean-Christophe Joyaux1, Maryvonne Dueymes2, Angélique Donnio1, Nicole Desbois2 1Department of Ophthalmology, 2Laboratory of Microbiology, University Hospital of Fort-de-France, Martinique, French West Indies Abstract: The authors describe a case of keratomycosis that appeared after the exeresis of a pterygium. A 48-year-old patient had been referred with a red right eye associated with an abscess of the cornea along the ablation zone of the pterygium. The surgery had been performed a month beforehand. The abscess was 6 mm high and 4 mm wide. The authors instigated a treatment that included amphotericin B (0.25% after noticing a clinical aspect evoking a fungal keratitis and finding several septate filaments on direct examination. On day 10, a Fusarium dimerum was isolated on Sabouraud agar. After 15 days of treatment, the result was favorable and the size of the ulceration as well as the size of the abscess had progressively decreased. The antifungal treatment was definitively stopped at 14 weeks. Infectious-related complications of the pterygium surgery are rare and are essentially caused by bacterial agents. Secondary infections by fungus are rare. There have been two previous cases reported: one that appeared 15 years after radiotherapy and another that appeared at 3 weeks post surgery, consecutive to the use of mitomycin C. To the authors' knowledge, this is the first case of a keratomycosis due to F. dimerum reported that complicated the exeresis of a pterygium without the use of an adjuvant antihealing treatment. Pterygium surgery is a common procedure; nevertheless, ophthalmologists need to be aware of the existence of potential infectious complications. Keywords: keratitis, corneal abscess, Fusarium

  14. [Clinically documented fungal infections].

    Science.gov (United States)

    Kakeya, Hiroshi; Kohno, Shigeru

    2008-12-01

    Proven fungal infections are diagnosed by histological/microbiological evidence of fungi at the site of infection and positive blood culture (fungemia). However, invasive diagnosing examinations are not always applied for all of immunocompromised patients. Clinically documented invasive fungal infections are diagnosed by typical radiological findings such as halo sign on chest CT plus positive serological/molecular evidence of fungi. Serological tests of Aspergillus galactomannan antigen and beta-glucan for aspergillosis and cryptococcal glucuronoxylomannan antigen for cryptococcosis are useful. Hence, none of reliable serological tests for zygomycosis are available so far. In this article, risk factors, sign and symptoms, and diagnostic methods for clinically documented cases of invasive aspergillosis, pulmonary cryptococcosis, and zygomycosis with diabates, are reviewed.

  15. CNS fungal meningitis to the "Top of the basilar"

    Institute of Scientific and Technical Information of China (English)

    Logan CS; Kirschner RC; Simonds GR

    2013-01-01

    Central nervous system(CNS) infections are a rare complication of epidural steroid injections and without strong clinical suspicion, fungal organisms may be overlooked among the long differential of causes of meningitis.Rare sequela of fungal meningitis is the development of stroke.To our knowledge, we present the first case of post epidural steroid injection(ESI) fungal meningitis leading toa basilar artery stroke, otherwise known as“top of the basilar” syndrome.We present a49-year-old female with a history ofESIs who presented to the emergency department with headache, neck stiffness, and abdominal pain.She was discharged after her labs and symptoms were deemed inconsistent with meningitis.She was eventually admitted and twelve days after her originalED visit, she was diagnosed with meningitis and started on anti-fungal treatment.She was discharged88 days later but was readmitted due to left sided weakness and mental status changes.She quickly lost motor and bulbar functions.AnMRA showed diminished distal flow through the basilar artery, suggesting near complete occlusion.Although appropriate long term anti-fungal treatment was started, the patient still succumbed to a rare vascular event.Physicians who are treating patients forESI meningitis should be aware of the potential for vasculitic and encephalitic complications.

  16. An impacted pancreatic stone in the papilla induced acute obstructive cholangitis in a patient with chronic pancreatitis.

    Science.gov (United States)

    Yoo, Kwang-Ho; Kwon, Chang-Il; Yoon, Sang-Wook; Kim, Won Hee; Lee, Jung Min; Ko, Kwang Hyun; Hong, Sung Pyo; Park, Pil Won

    2012-03-01

    Obstructive jaundice is very rarely caused by impaction of a pancreatic stone in the papilla. We report here on a case of obstructive jaundice with acute cholangitis that was caused by an impacted pancreatic stone in the papilla in a patient with chronic pancreatitis. A 48-year-old man presented with acute obstructive cholangitis. Abdominal computed tomography with the reconstructed image revealed distal biliary obstruction that was caused by a pancreatic stone in the pancreatic head, and there was also pancreatic ductal dilatation and parenchymal atrophy of the pancreatic body and tail with multiple calcifications. Emergency duodenoscopy revealed an impacted pancreatic stone in the papilla. Precut papillotomy using a needle knife was performed, followed by removal of the pancreatic stone using grasping forceps. After additional sphincterotomy, a large amount of dark-greenish bile juice gushed out. The patient rapidly improved and he has remained well.

  17. Analysis on the imaging features of AIDS with pulmonary fungal infection

    Institute of Scientific and Technical Information of China (English)

    GAO Jian-bo; ZHANG Yong-gao; YUE Song-wei; LI Hong-jun; NING Pei-gang; GUO Hua; XIAO Hui-juan

    2010-01-01

    Background Pulmonary fungal infection is one type of the common opportunistic infections in AIDS patients. The disease is hard to diagnose because of its complicated imaging features. The objective of this study was to investigate the imaging performance characteristics of pulmonary fungal infection in AIDS patients.Methods Fifty-one patients with AIDS complicated with pulmonary fungal infection and 56 patients of non-AIDS with pulmonary fungal infection were examined by CT scans and high-resolution CT scans. The contrast enhanced scans were performed in patients with the mass or suspected enlarged mediastinal lymph nodes. Results were compared between the two groups.Results The most common fungal infection in the two groups of patients was Candida albicans. The infection rates were 54.8% (28 cases) in the group (AIDS patients with pulmonary fungal infection) and 58.3% (32 cases) in another group (non-AIDS patients with pulmonary fungal infection). In the two groups, the difference in diffuse distribution and the difference in incidence of affected upper and lower lobes in the bilateral lung fields were statistically significant. The differences in patchy or large consolidation shadow, cavitas, enlarged lymph nodes in mediastinum and pleural effusion were also significant when comparing the two groups.Conclusions The lesion in most of AIDS patients with pulmonary fungal infection tends to exhibit diffuse distribution,patchy or large consolidation shadow covering a more extensive region. The differences between AIDS with pulmonary fungal infection and non-AIDS with pulmonary fungal infection are statistically significant in lesion location and complicated imaging features. The most common fungal infection in AIDS patients is Candida albicans.

  18. Diagnosis and Treatment of Severe Acute Cholangitis%重症急性胆管炎的诊治

    Institute of Scientific and Technical Information of China (English)

    王文龙; 郑英键

    2003-01-01

    @@ 胆道系统发生的急性细菌性感染,包括化脓性的严重炎症,称为急性胆管炎(acute cholangitis,AC).它是胆道外科各种疾病的常见合并症,多在胆道存在梗阻因素的基础上发生.

  19. Pulmonary complications in renal transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Jung Bin; Choi, Yo Won; Jeon, Seok Chol; Park, Choong Ki; Lee, Seung Rho; Hahm, Chang Kok; Joo, Kyung Bin [Hanyang University College of Medicine, Seoul (Korea, Republic of)

    2003-04-01

    To evaluate the radiographic and CT findings of pulmonary complications other than pulmonary edema arising from renal transplantation. Among 393 patients who had undergone renal transplantation at our hospital during a previous ten-year period, 23 with pulmonary complications other than pulmonary edema were included in this study. The complications involved were infection caused by CMV (n=6), bacteria (n=4), fungus (n=4), tuberculosis (n=2), varicella (n=1) or chlamydia (n=1), and malignancy involving lung cancer (n=4) or Kaposi's sarcoma (n=1). Two chest radiologists reviewed all images. The complications manifesting mainly as pulmonary nodules were lung cancer (4/4), tuberculosis (1/2), and Kaposi's sarcoma (1/1). Pulmonary consolidation was a main feature in bacterial infection (4/4), fungal infection (3/4), tuberculosis (1/2), chlamydial infection (1/1), and varicellar pneumonia (1/1). Ground-glass attenuation was a main CT feature in CMV pneumonia (4/6), and increased interstitial making was a predominant radiographic feature in CMV pneumonia (2/6). The main radiologic features described above can be helpful for differential diagnosis of the pulmonary complications of renal transplantation.

  20. Fungal osteomyelitis and septic arthritis.

    Science.gov (United States)

    Bariteau, Jason T; Waryasz, Gregory R; McDonnell, Matthew; Fischer, Staci A; Hayda, Roman A; Born, Christopher T

    2014-06-01

    Management of fungal osteomyelitis and fungal septic arthritis is challenging, especially in the setting of immunodeficiency and conditions that require immunosuppression. Because fungal osteomyelitis and fungal septic arthritis are rare conditions, study of their pathophysiology and treatment has been limited. In the literature, evidence-based treatment is lacking and, historically, outcomes have been poor. The most common offending organisms are Candida and Aspergillus, which are widely distributed in humans and soil. However, some fungal pathogens, such as Histoplasma, Blastomyces, Coccidioides, Cryptococcus, and Sporothrix, have more focal areas of endemicity. Fungal bone and joint infections result from direct inoculation, contiguous infection spread, or hematogenous seeding of organisms. These infections may be difficult to diagnose and eradicate, especially in the setting of total joint arthroplasty. Although there is no clear consensus on treatment, guidelines are available for management of many of these pathogens.

  1. Risk factors for mucocutaneous fungal infections in patients with type 2 diabetes mellitus

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    Düriye Deniz Demirseren

    2014-06-01

    Full Text Available Background and design: Mucocutaneous fungal infections are common in patients with diabetes mellitus (DM. However, fungal infections do not develop in every patient with DM. In this study, we aimed to determine the risk factors for developing mucocutaneous fungal infections in patients with type 2 DM. Materials and methods: A total of 302 type 2 DM patients with mucocutaneous fungal infections and 326 type 2 DM patients without mucocutaneous fungal infections were enrolled. Demographic and clinical features, HbA1c levels, DM durations, body mass indexes (BMIs, and DM related complications of patients were compared and risk factors for developing mucocutaneous fungal infections were determined. Results: Of the 302 patients with mucocutaneous fungal infections, 81.2% (n=245 had dermatophytosis, 16.9% (n=51 had candidal infections, 2.0% (n=6 had pityriasis versicolor. Frequency of male gender, diabetic nephropathy, neuropathy and retinopathy; DM durations and ages of patients were all significantly higher in diabetic patients with fungal infections than patients without fungal infections (all p<0.05. Male gender, age ≥ 50 years, nephropathy and neuropathy were independently associated with developing fungal infection in type 2 DM patients. In subgroup analyses, independent risk factors for dermatophytosis were male gender, age ≥ 50 years, DM duration ≥5 years, and nephropathy. For candidiasis, these factors were BMI≥30 and neuropathy. Conclusion: Elderly, male gender, diabetic neuropathy annd nephropathy are closely associated with developing mucocutaneous infections in patients with type 2 DM.

  2. Failures, complications and interventionalist radiation exposures in 108 cases of attempted biliary endoprostheses

    Energy Technology Data Exchange (ETDEWEB)

    Le Page, J.R. (Roberts University, Tulsa, OK (USA))

    In a series of 108 patients in whom we placed 116 biliary drainage prostheses we were unable to place endoprostheses as intended in 67.2 % and were forced to use only external drainage catheters in 25.9 %. There were no total failures. We encountered 11 % serious complications: severe bleeding; cholangitis; pancreatitis; hypotensive shock, and hyponatremia. Most seriously, we consider the radiation received to the hands (2500mR), eyes and thyroid (17 mR) of the operator unacceptably high for the short term benefit of a patient with a brief life expectancy and the internal/external drainage at 30 % of this dose is preferable to the endoprosthesis.

  3. Fungal Agents as a Cause of Nasal Polyposis

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    Mohammad Nejadkazem

    2015-01-01

    Full Text Available Introduction: Sinonasal polyposis is the most common tumor of nasal cavity and sinuses. Its complications are but not limited to sinusitis, breathing difficulties, hyposmia, anosmia and bone erosion. Methods and materials: A total of 98 patients with sinonasal polyposis were examined for suspicious causative fungal agent. Results: Direct microscopy and culture confirmed fungal agent in 8 patients (8.1% from which 3 cases had Alternaria spp, 1 patient Aspergillus spp, 1 patient Bipolaris spp, and 3 patients yeast. Conclusion: Fungi may be considered as a potential cause of sinonasal polyposis.   Keywords: Sinonasal Polyposis, Rhinosinusitis, Fungi

  4. Acute fulminant invasive fungal sinusitis with cavernous sinus syndrome.

    Science.gov (United States)

    Chi, Tzu-Hang; Chen, Hsien-Shen; Yuan, Chien-Han; Su, Feng-Ming

    2014-11-01

    Acute fulminant invasive fungal sinusitis is most commonly found in immunocompromised patients with conditions such as diabetes mellitus, malignancies and acquired immune deficiency syndrome. The most common pathogens are Aspergillus and Mucoraceae and the sinus most frequently involved is the maxillary sinus. Fever, rhinorrhea, facial pain, headache, and diplopia are common presenting symptoms. Complications of this infection include intracranial and / or intraorbital spread of the infection; the prognosis is poor. Here, a rare case of acute fulminant invasive fungal sinusitis with cavernous sinus syndrome is reported.

  5. Characteristics and determinants of ambient fungal spores in Hualien, Taiwan

    Science.gov (United States)

    Ho, Hsiao-Man; Rao, Carol Y.; Hsu, Hsiao-Hsien; Chiu, Yueh-Hsiu; Liu, Chi-Ming; Chao, H. Jasmine

    Characteristics and determinants of ambient aeroallergens are of much concern in recent years because of the apparent health impacts of allergens. Yet relatively little is known about the complex behaviors of ambient aeroallergens. To address this issue, we monitored ambient fungal spores in Hualien, Taiwan from 1993-1996 to examine the compositions and temporal variations of fungi, and to evaluate possible determinants. We used a Burkard seven-day volumetric spore trap to collect daily fungal spores. Air pollutants, meteorological factors, and Asian dust events were included in the statistical analyses to predict fungal levels. We found that the most dominant fungal categories were ascospores, followed by Cladosporium and Aspergillus/Penicillium. The majority of the fungal categories had significant diurnal and seasonal variations. Total fungi, Cladosporium, Ganoderma, Arthrinium/Papularia, Cercospora, Periconia, Alternaria, Botrytis, and PM 10 had significantly higher concentrations ( p<0.05) during the period affected by Asian dust events. In multiple regression models, we found that temperature was consistently and positively associated with fungal concentrations. Other factors correlated with fungal concentrations included ozone, particulate matters with an aerodynamic diameter less than 10 μm (PM 10), relative humidity, rainfall, atmospheric pressure, total hydrocarbons, carbon monoxide, nitrogen dioxide, and sulfur dioxide. Most of the fungal categories had higher levels in 1994 than in 1995-96, probably due to urbanization of the study area. In this study, we demonstrated complicated interrelationships between fungi and air pollution/meteorological factors. In addition, long-range transport of air pollutants contributed significantly to local aeroallergen levels. Future studies should examine the health impacts of aeroallergens, as well as the synergistic/antagonistic effects of weather, and local and global-scale air pollutions.

  6. IgG4-Associated Cholangitis--A Mimic of PSC.

    Science.gov (United States)

    Beuers, Ulrich; Hubers, Lowiek M; Doorenspleet, Marieke; Maillette de Buy Wenniger, Lucas; Klarenbeek, Paul L; Boonstra, Kirsten; Ponsioen, Cyriel; Rauws, Erik; de Vries, Niek

    2015-01-01

    IgG4-associated cholangitis (IAC) is an inflammatory disorder of the biliary tract representing a major manifestation of IgG4-related disease (IgG4-RD) often with elevation of serum IgG4 levels, infiltration of IgG4+ plasma cells in the affected tissue and good response to immunosuppressive treatment. Its first description may go back to 150 years ago. The clinical presentation of IAC is often misleading, mimicking other biliary diseases such as primary sclerosing cholangitis (PSC) or cholangiocarcinoma. The HISORt criteria--histopathological, imaging, and serological features (sIgG4), other organ manifestations of IgG4-RD and response to treatment--are the standard for the diagnosis of IAC. In this overview of a recent lecture, we summarize our original findings on IgG4-RD that (i) dominant IgG4+ B-cell clones identified by advanced next generation sequencing (NGS) are highly specific for IgG4-RD (meanwhile confirmed by others), are a highly accurate diagnostic marker to distinguish IgG4-RD from PSC and biliary/pancreatic malignancies and may be crucial in unravelling the pathophysiology of IgG4-RD; (ii) sIgG4/sIgG1 >0.24 have additional diagnostic value in comparison to sIgG4 in differentiating IAC from PSC; (iii) blood IgG4 mRNA is a highly accurate diagnostic marker comparable to NGS and may become an easily available and affordable diagnostic standard for distinguishing IgG4-RD from PSC and biliary/pancreatic malignancies; and (iv) 'blue collar work' with long-term exposure to solvents, paints, oil products or industrial gases may be a risk factor for development of IgG4-RD. These findings may contribute to the understanding of the pathophysiology and to the early diagnosis and adequate treatment of IgG4-RD.

  7. Sphincterotomy with endoscopic biliary drainage for severe acute cholangitis: a meta-analysis

    Science.gov (United States)

    Sawas, Tarek; Arwani, Noura; Al Halabi, Shadi; Vargo, John

    2017-01-01

    Aims To investigate the role of endoscopic sphincterotomy (ES) with endoscopic biliary drainage (EBD) in acute severe obstructive cholangitis management by performing a meta-analysis of controlled trials. Method We searched PubMed and Embase for controlled studies that compared endoscopic drainage with ES versus Non-ES in acute obstructive cholangitis. Two reviewers selected the studies and extracted the data. Disagreement was addressed by a third reviewer. Heterogeneity of the studies was analyzed by Cochran’s Q statistics. A Mantel–Haenszel risk ratio was calculated utilizing a random effects model. Results Four controlled studies met our inclusion criteria with 392 participants (201 ES, 191 Non-ES). The outcomes were drainage insertion success rate, drainage effectiveness, post drainage pancreatitis, bleeding, procedure duration, perforation, cholecystitis, and 30-day mortality. Drainage insertion success rate was identical in both groups (RR: 1.00, 95 %CI% 0.96 – 1.04). Effective drainage was not significantly different (RR: 1.11, 95 %CI 0.73 – 1.7). There was no significant difference in the incidence of pancreatitis post EBD between the ES and Non-ES groups at 3 % and 4 %, respectively (RR: 0.73, 95 %CI 0.24 – 2.27). However, there was a significant increase in post EBD bleeding with ES compared to Non-ES (RR: 8.58, 95 %CI 2.03 – 36.34). Thirty-day mortality was similar between ES and Non-ES groups at 0.7 % and 1 %, respectively (RR: 0.5, 95 %CI 0.05 – 5.28). Conclusion Our findings show that EBD without ES is an effective drainage technique and carries less risk for post procedure bleeding. Patients who are critically ill and have coagulopathy should be spared from undergoing ES in the acute phase. PMID:28229129

  8. MR imaging of primary sclerosing cholangitis - Additional value of diffusion-weighted imaging and ADC measurement

    Energy Technology Data Exchange (ETDEWEB)

    Djokicc Kovac, Jelena [Center for Radiology and Magnetic Resonance Imaging, Clinical Center Serbia, Belgrade (Serbia)], e-mail: jelenadjokic2003@yahoo.co.uk; Maksimovic, Ruzica [Center for Radiology and Magnetic Resonance Imaging, Clinical Center Serbia, Belgrade (Serbia); Faculty of Medicine, Univ. of Belgrade, Belgrade (Serbia); Jesic, Rada [Clinic for Gastroenterohepatology, Clinical Center Serbia, Belgrade (Serbia); Faculty of Medicine, Univ. of Belgrade, Belgrade (Serbia); Stanisavljevic, Dejana [Inst. for Statistics, Faculty of Medicine, Univ. of Belgrade, Belgrade (Serbia); Kovac, Bojan [Military Medical Academy, Belgrade (Serbia)

    2013-04-15

    Background: Primary sclerosing cholangitis (PSC) is a cholestatic liver disease with chronic inflammation and progressive destruction of biliary tree. Magnetic resonance (MR) examination with diffusion-weighted imaging (DWI) allows analysis of morphological liver parenchymal changes and non-invasive assessment of liver fibrosis. Moreover, MR cholangiopancreatography (MRCP), as a part of standard MR protocol, provides insight into bile duct irregularities. Purpose: To evaluate MR and MRCP findings in patients with primary sclerosing cholangitis and to determine the value of DWI in the assessment of liver fibrosis. Material and Methods: The following MR findings were reviewed in 38 patients: abnormalities in liver parenchyma signal intensity, changes in liver morphology, lymphadenopathy, signs of portal hypertension, and irregularities of intra- and extrahepatic bile ducts. Apparent diffusion coefficient (ADC) was calculated for six locations in the liver for b = 800 s/mm{sup 2}. Results: T2-weighted hyperintensity was seen as peripheral wedge-shaped areas in 42.1% and as periportal edema in 28.9% of patients. Increased enhancement of liver parenchyma on arterial-phase imaging was observed in six (15.8%) patients. Caudate lobe hypertrophy was present in 10 (26.3%), while spherical liver shape was noted in 7.9% of patients. Liver cirrhosis was seen in 34.2% of patients; the most common pattern was micronodular cirrhosis (61.5%). Other findings included lymphadenopathy (28.9%), signs of portal hypertension (36.7%), and bile duct irregularities (78.9%). The mean ADCs (x10{sup -3} mm{sup 2}/s) were significantly different at stage I vs. stages III and IV, and stage II vs. stage IV. No significant difference was found between stages II and III. For prediction of stage {>=}II and stage {>=}III, areas under receiver-operating characteristic curves were 0.891 and 0.887, respectively. Conclusion: MR with MRCP is a necessary diagnostic procedure for diagnosis of PSC and

  9. Fungal biodiversity to biotechnology.

    Science.gov (United States)

    Chambergo, Felipe S; Valencia, Estela Y

    2016-03-01

    Fungal habitats include soil, water, and extreme environments. With around 100,000 fungus species already described, it is estimated that 5.1 million fungus species exist on our planet, making fungi one of the largest and most diverse kingdoms of eukaryotes. Fungi show remarkable metabolic features due to a sophisticated genomic network and are important for the production of biotechnological compounds that greatly impact our society in many ways. In this review, we present the current state of knowledge on fungal biodiversity, with special emphasis on filamentous fungi and the most recent discoveries in the field of identification and production of biotechnological compounds. More than 250 fungus species have been studied to produce these biotechnological compounds. This review focuses on three of the branches generally accepted in biotechnological applications, which have been identified by a color code: red, green, and white for pharmaceutical, agricultural, and industrial biotechnology, respectively. We also discuss future prospects for the use of filamentous fungi in biotechnology application.

  10. Managing acute invasive fungal sinusitis.

    Science.gov (United States)

    Dwyhalo, Kristina M; Donald, Carrlene; Mendez, Anthony; Hoxworth, Joseph

    2016-01-01

    Acute invasive fungal sinusitis is the most aggressive form of fungal sinusitis and can be fatal, especially in patients who are immunosuppressed. Early diagnosis and intervention are crucial and potentially lifesaving, so primary care providers must maintain a high index of suspicion for this disease. Patients may need to be admitted to the hospital for IV antifungal therapy and surgical debridement.

  11. Current management of fungal infections.

    NARCIS (Netherlands)

    Meis, J.F.G.M.; Verweij, P.E.

    2001-01-01

    The management of superficial fungal infections differs significantly from the management of systemic fungal infections. Most superficial infections are treated with topical antifungal agents, the choice of agent being determined by the site and extent of the infection and by the causative organism,

  12. The evolution of fungal epiphytes

    NARCIS (Netherlands)

    Hongsanan, S.; Sánchez-Ramírez, S.; Crous, P.W.; Ariyawansa, H.A.; Zhao, R.L.; Hyde, K.D.

    2016-01-01

    Fungal epiphytes are a polyphyletic group found on the surface of plants, particularly on leaves, with a worldwide distribution. They belong in the phylum Ascomycota, which contains the largest known number of fungal genera. There has been little research dating the origins of the common ancestors o

  13. Geoepidemiology, clinical manifestations and outcome of primary biliary cholangitis in Greece.

    Science.gov (United States)

    Gatselis, Nikolaos K; Zachou, Kalliopi; Lygoura, Vasiliki; Azariadis, Kalliopi; Arvaniti, Pinelopi; Spyrou, Elias; Papadamou, Georgia; Koukoulis, George K; Dalekos, George N; Rigopoulou, Eirini I

    2017-07-01

    Primary biliary cholangitis (PBC) is a disease with rising prevalence and considerable geographical variation. To describe the prevalence, spatial and time distribution, baseline characteristics, response to treatment, outcome and the validity of GLOBE score in a large cohort of Greek PBC patients as an independent validation of this score has not been done so far. The last 16years, 482 PBC patients (86.5% females) were evaluated and analysed retrospectively, using a prospectively collected database. Special attention was paid to the assessment of treatment response according to GLOBE score. Age at initial evaluation was 56.3±13.7years. Among 432 Thessaly residents, prevalence was 582/million (non-homogeneous distribution). Nineteen districts showed a prevalence >800/million. Symptomatic disease onset could be identified in 91 patients, with a significant peak during spring (P=0.03). At diagnosis, 43.6% were asymptomatic and 16.2% cirrhotic. Male sex (P=0.02), older age (P0.30 had significantly worse prognosis (P<0.001) with 5-, 10-, and 15-year survival rates of 84%, 50% and 42%. There is increased PBC prevalence in Thessaly with remarkable geographic clustering and seasonal variability. PBC is diagnosed at early stages although males had a more advanced disease. GLOBE score applies perfectly in Greek patients and this will likely help detecting patients that may benefit from new therapies. Copyright © 2017 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

  14. Morphologic features of chronic hepatitis associated with primary sclerosing cholangitis and chronic ulcerative colitis

    Energy Technology Data Exchange (ETDEWEB)

    Ludwig, J.; Barham, S.S.; LaRusso, N.F.; Elveback, L.R.; Wiesner, R.H.; McCall, J.T.

    Histologic, ultrastructural, chemical, and statistical methods were used to study liver biopsy and autopsy specimens from 43 patients who had primary sclerosing cholangitis (PSC), with or without chronic ulcerative colitis (CUC), and from 19 patients who had CUC without PSC. In all study groups, essentially the same abnormalities were found in the hepatic parenchyma outside the major bile ducts, although nondiagnostic tissue samples were observed also. Specimens from patients with extrahepatic PSC were indistinguishable from those patients with combined extra- and intrahepatic PSC. Common findings included periductal fibrosis and inflammation, portal edema and fibrosis, focal proliferation of bile ducts and ductules, focal bile duct obliteration and loss of bile ducts, copper deposition, and cholestasis. Proliferation of bile ducts in some portal tracts and obliteration or absence of bile duct in others were the most characteristic changes. In most specimens, inflammatory changes appeared mild, yet biliary cirrhosis had developed in 34% of the patients. Specimens from patients with PSC, with or without CUC, more often contained bile and strikingly increased stainable copper (Grades 2 and 3) than did specimens from patients with CUC without PSC. Hepatic copper contents, measured by atomic absorption spectrophotometry, also were higher in specimens from patients with PSC. Study of PCS specimens by transmission electron microscopy and by energy-dispersive X-ray microanalysis revealed that most copper was sequestered in lipolysosomes. The recognition of strikingly similar morphologic features in many liver specimens from patients with either PSC or CUC or both suggests that the causes of these conditions are closely related.

  15. The relation between plasma tyrosine concentration and fatigue in primary biliary cirrhosis and primary sclerosing cholangitis

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    Vrolijk Jan

    2005-03-01

    Full Text Available Abstract Background In primary biliary cirrhosis (PBC and primary sclerosing cholangitis (PSC fatigue is a major clinical problem. Abnormal amino acid (AA patterns have been implicated in the development of fatigue in several non-hepatological conditions but for PBC and PSC no data are available. This study aimed to identify abnormalities in AA patterns and to define their relation with fatigue. Methods Plasma concentrations of tyrosine, tryptophan, phenylalanine, valine, leucine and isoleucine were determined in plasma of patients with PBC (n = 45, PSC (n = 27, chronic hepatitis C (n = 22 and healthy controls (n = 73. Fatigue and quality of life were quantified using the Fisk fatigue severity scale, a visual analogue scale and the SF-36. Results Valine, isoleucine, leucine were significantly decreased in PBC and PSC. Tyrosine and phenylalanine were increased (p Conclusion In patients with PBC and PSC, marked abnormalities in plasma AA patterns occur. Normal tyrosine concentrations, compared to increased concentrations, may be associated with fatigue and diminished quality of life.

  16. 原发性硬化性胆管炎%PRIMARY SCLEROSING CHOLANGITIS

    Institute of Scientific and Technical Information of China (English)

    钟大昌

    2001-01-01

    Objective To study current developments in research of primarysclerosing cholangitis(PSC). Methods The literatures in recent years were reviewed. Results A number of evidences suggested that immune mechanisms played an important role in etiology of PSC. Its diagnosis was primarily made by cholangiography and all used therapies only relieved symptoms and improved hepatic function temporarily. Hepatic transplantation for advanced cases has been proven effective. Conclusion The etiology is still unknown and no therapies is effective in halting the disease progression but hepatic transplantation, so the prognosis is poor.%目的 了解原发性硬化性胆管炎研究进展。方法 复习近年来有关文献。结果 较多证据提示其病因与免疫机制相关;确诊主要凭胆管造影;各种疗法仅能暂时缓解症状及改善肝功能;肝移植可治疗晚期病例。结论 其病因仍不清楚,除肝移植外尚无有效阻止病变进展的治疗方法,远期预后差。

  17. Genetic Abnormalities in Biliary Brush Samples for Distinguishing Cholangiocarcinoma from Benign Strictures in Primary Sclerosing Cholangitis

    Science.gov (United States)

    Timmer, Margriet R.; Lau, Chiu T.; Meijer, Sybren L.; Fockens, Paul; Rauws, Erik A. J.; Ponsioen, Cyriel Y.; Calpe, Silvia; Krishnadath, Kausilia K.

    2016-01-01

    Background. Primary sclerosing cholangitis (PSC) is a chronic inflammatory liver disease and is strongly associated with cholangiocarcinoma (CCA). The lack of efficient diagnostic methods for CCA is a major problem. Testing for genetic abnormalities may increase the diagnostic value of cytology. Methods. We assessed genetic abnormalities for CDKN2A, TP53, ERBB2, 20q, MYC, and chromosomes 7 and 17 and measures of genetic clonal diversity in brush samples from 29 PSC patients with benign biliary strictures and 12 patients with sporadic CCA or PSC-associated CCA. Diagnostic performance of cytology alone and in combination with genetic markers was evaluated by sensitivity, specificity, and area under the curve analysis. Results. The presence of MYC gain and CDKN2A loss as well as a higher clonal diversity was significantly associated with malignancy. MYC gain increased the sensitivity of cytology from 50% to 83%. However, the specificity decreased from 97% to 76%. The diagnostic accuracy of the best performing measures of clonal diversity was similar to the combination of cytology and MYC. Adding CDKN2A loss to the panel had no additional benefit. Conclusion. Evaluation of MYC abnormalities and measures of clonal diversity in brush cytology specimens may be of clinical value in distinguishing CCA from benign biliary strictures in PSC. PMID:27127503

  18. Leukocytapheresis Therapy Improved Cholestasis in a Patient Suffering from Primary Sclerosing Cholangitis with Ulcerative Colitis

    Directory of Open Access Journals (Sweden)

    Minoru Itou

    2009-04-01

    Full Text Available Primary sclerosing cholangitis (PSC is an autoimmune disease of the hepatobiliary system for which effective therapy has not been established. Leukocytapheresis (LCAP therapy is known to effective in patients with ulcerative colitis (UC. In addition, effects of LCAP therapy were reported on some autoimmune diseases such as Crohn’s disease, rheumatoid arthritis and rapidly progressive glomerulonephritis. Here we report the case of a 29-year-old man with PSC associated with UC who was treated with LCAP therapy. He had a 16-year history of UC and a 12-year history of PSC. Although he was under treatment with prednisolone and ursodeoxycholic acid, exacerbation of UC and PSC-associated cholestasis were seen. Since he showed side effects of prednisolone, he was treated with LCAP. Not only improvement of UC, but also decreased serum alkaline phosphatase, γ-guanosine triphosphate and total bile acids, suggesting improvement of PSC-associated cholestaisis, were seen after treatment with LCAP. Our experience with this case suggests that LCAP therapy could be a new effective therapeutic strategy for patients with PSC associated with UC.

  19. Criteria Used in Clinical Practice to Guide Immunosuppressive Treatment in Patients with Primary Sclerosing Cholangitis.

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    Kornelius Schulze

    Full Text Available Current guidelines recommend immunosuppressive treatment (IT in patients with primary sclerosing cholangitis (PSC and elevated aminotransferase levels more than five times the upper limit of normal and elevated serum IgG-levels above twice the upper limit of normal. Since there is no evidence to support this recommendation, we aimed to assess the criteria that guided clinicians in clinical practice to initiate IT in patients with previously diagnosed PSC.This is a retrospective analysis of 196 PSC patients from seven German hepatology centers, of whom 36 patients had received IT solely for their liver disease during the course of PSC. Analyses were carried out using methods for competing risks.A simplified autoimmune hepatitis (AIH score >5 (HR of 36, p5 and a mHAI score >3, suggesting concomitant features of AIH, influenced the decision to introduce IT during the course of PSC. In German clinical practice, the cutoffs used to guide IT may be lower than recommended by current guidelines.

  20. Hepatobiliary scintigraphy in chronic intrahepatic cholestasis. Diagnosis of primary sclerosing cholangitis

    Energy Technology Data Exchange (ETDEWEB)

    Aburano, Tamio; Takayama, Teruhiko; Shuke, Noriyuki

    1987-05-01

    Primary sclerosing cholangitis (PSC) is a rare disease of unknown origin, leading to chronic intermittent cholestasis. Due to its low incidence, insidious clinical onset and varied clinical picture, the diagnosis is often delayed by years. PSC is sometimes diagnosed falsely as another disease of chronic intermittent cholestasis, primary biliary cirrhosis (PBC). In the present study, the hepatobiliary imaging with Tc-99m diethyl IDA was done in a total of 14 patients with chronic intermittent cholestasis including 3 patients with PSC and 11 patients with PBC, in order to decide its clinical usefulness as a noninvasive method for the differentiation between PSC and PBC. All three patients with PSC showed a typical pattern of radionuclide stasis within the area of intrahepatic and/or extrahepatic ductal system, representing the stenosis on endoscopic retrograde cholangiogram. On the other hand, none of 11 patients with PBC showed any radionuclide stasis within the area of intrahepatic and/or extrahepatic ductal system. This result suggests that the radionuclide hepatobiliary imaging may be a noninvasive method for investigating patients with chronic intermittent cholestasis, leading to earlier differentiation between PSC and PBC.

  1. Genetic Abnormalities in Biliary Brush Samples for Distinguishing Cholangiocarcinoma from Benign Strictures in Primary Sclerosing Cholangitis

    Directory of Open Access Journals (Sweden)

    Margriet R. Timmer

    2016-01-01

    Full Text Available Background. Primary sclerosing cholangitis (PSC is a chronic inflammatory liver disease and is strongly associated with cholangiocarcinoma (CCA. The lack of efficient diagnostic methods for CCA is a major problem. Testing for genetic abnormalities may increase the diagnostic value of cytology. Methods. We assessed genetic abnormalities for CDKN2A, TP53, ERBB2, 20q, MYC, and chromosomes 7 and 17 and measures of genetic clonal diversity in brush samples from 29 PSC patients with benign biliary strictures and 12 patients with sporadic CCA or PSC-associated CCA. Diagnostic performance of cytology alone and in combination with genetic markers was evaluated by sensitivity, specificity, and area under the curve analysis. Results. The presence of MYC gain and CDKN2A loss as well as a higher clonal diversity was significantly associated with malignancy. MYC gain increased the sensitivity of cytology from 50% to 83%. However, the specificity decreased from 97% to 76%. The diagnostic accuracy of the best performing measures of clonal diversity was similar to the combination of cytology and MYC. Adding CDKN2A loss to the panel had no additional benefit. Conclusion. Evaluation of MYC abnormalities and measures of clonal diversity in brush cytology specimens may be of clinical value in distinguishing CCA from benign biliary strictures in PSC.

  2. Primary Sclerosing Cholangitis as a Premalignant Biliary Tract Disease: Surveillance and Management.

    Science.gov (United States)

    Rizvi, Sumera; Eaton, John E; Gores, Gregory J

    2015-11-01

    Primary sclerosing cholangitis (PSC) is a premalignant biliary tract disease that confers a significant risk for the development of cholangiocarcinoma (CCA). The chronic biliary tract inflammation of PSC promotes pro-oncogenic processes such as cellular proliferation, induction of DNA damage, alterations of the extracellular matrix, and cholestasis. The diagnosis of malignancy in PSC can be challenging because inflammation-related changes in PSC may produce dominant biliary tract strictures mimicking CCA. Biomarkers such as detection of methylated genes in biliary specimens represent noninvasive techniques that may discriminate malignant biliary ductal changes from PSC strictures. However, conventional cytology and advanced cytologic techniques such as fluorescence in situ hybridization for polysomy remain the practice standard for diagnosing CCA in PSC. Curative treatment options of malignancy arising in PSC are limited. For a subset of patients selected by using stringent criteria, liver transplantation after neoadjuvant chemoradiation is a potential curative therapy. However, most patients have advanced malignancy at the time of diagnosis. Advances directed at identifying high-risk patients, early cancer detection, and development of chemopreventive strategies will be essential to better manage the cancer risk in this premalignant disease. A better understanding of dysplasia definition and especially its natural history is also needed in this disease. Herein, we review recent developments in our understanding of the risk factors, pathogenic mechanisms of PSC associated with CCA, as well as advances in early detection and therapies.

  3. Prevalence of Systemic Sclerosis in Primary Biliary Cholangitis Using the New ACR/EULAR Classification Criteria.

    Science.gov (United States)

    Zheng, Boyang; Vincent, Catherine; Fritzler, Marvin J; Senécal, Jean-Luc; Koenig, Martial; Joyal, France

    2017-01-01

    Systemic sclerosis (SSc) is a well-established disease associated with primary biliary cholangitis (PBC). However, the original 1980 American College of Rheumatology (ACR) criteria have poor sensitivity, especially for the detection of earlier SSc in previous studies. The objective was to evaluate the prevalence of SSc in patients with PBC using more sensitive 2001 LeRoy and Medsger criteria and the 2013 ACR/European League Against Rheumatism (EULAR) classification criteria. The secondary objective was to evaluate the frequency of individual clinical features. One hundred consecutive patients with PBC without previously diagnosed SSc were recruited between 2005 and 2007 from a tertiary care gastroenterology clinic. All patients underwent a complete clinical examination, determination of SSc-specific antibodies, and a nailfold capillary microscopy. Fulfillment of the 3 different criteria sets was analyzed, along with individual disease features. Of 100 patients with PBC, 1% met the ACR 1980 criteria, 22% met the 2001 LeRoy and Medsger criteria for early SSc, and 17% the 2013 ACR/EULAR criteria. Raynaud phenomenon, SSc-related antibodies, and SSc capillaroscopic patterns were the most prevalent findings, with the highest sensitivities to help guide future screening. Our data show a high prevalence of SSc in patients with PBC with probable underestimation by previous studies using the original ACR criteria. Comorbid SSc should be actively searched for based on newly described criteria to improve detection and increase benefits of earlier treatment.

  4. Reduced Coffee Consumption Among Individuals with Primary Sclerosing Cholangitis but Not Primary Biliary Cirrhosis

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    Lammert, Craig; Juran, Brian D.; Schlicht, Erik; Xie, Xiao; Atkinson, Elizabeth J.; de Andrade, Mariza; Lazaridis, Konstantinos N.

    2014-01-01

    Background & Aims Coffee consumption has been associated with decreased risk of liver disease and related outcomes. However, coffee drinking has not been investigated among patients with cholestatic autoimmune liver diseases, primary biliary cirrhosis (PBC), or primary sclerosing cholangitis (PSC). We investigated the relationship between coffee consumption and risk of PBC and PSC in a large North American cohort. Methods Lifetime coffee drinking habits were determined from responses to questionnaires from 606 patients with PBC, 480 with PSC, and 564 healthy volunteers (controls). Patients (those with PBC or PSC) were compared to controls utilizing the Wilcoxon rank sum test for continuous variables and c2 method for discrete variables. Logistic regression was used to analyze the estimate the effects of different coffee parameters (time, frequency, and type of coffee consumption) after adjusting for age, sex, smoking status, and education level. Results Patients with PBC and controls did not differ in coffee parameters. However, 24% of patients with PSC had never drank coffee compared to 16% of controls (Pcoffee drinking coffee (46.6% vs 66.7% for controls, Pcoffee protected against proctocolectomy (hazard ratio=0.34, PCoffee consumption is lower among patients with PSC, but not PBC, compared to controls. PMID:24440215

  5. Novel therapeutics for primary biliary cholangitis: Toward a disease-stage-based approach.

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    Mousa, Hani S; Carbone, Marco; Malinverno, Federica; Ronca, Vincenzo; Gershwin, M Eric; Invernizzi, Pietro

    2016-09-01

    Primary biliary cholangitis (PBC; previously "primary biliary cirrhosis") is a cholestatic, putatively autoimmune-mediated liver disease with a clear female preponderance affecting the intrahepatic small and medium-size bile ducts and resulting in bile duct destruction, ductopenia and portal fibrosis that progresses slowly to biliary cirrhosis. Despite suboptimal response in one third of patients treated with ursodeoxycholic acid (UDCA), this remains the only FDA-approved agent for this disease. In this review, we cover recent advances in research that have yielded numerous agents currently at different stages of the drug pipeline, some of which are expected to be approved in the near future. We also discuss accumulating evidence supporting the use of older agents (fibrates and glucocorticoids) as an adjunctive therapy to UDCA in non-responsive patients. We suggest that with the imminent expansion of the therapeutic armamentarium for PBC, a more comprehensive approach - ideally taking into account not only biochemical markers of disease stage - is needed to better select patients in whom these strategies might be most useful. Studies are also needed to compare the relative efficacy of different proposed second-line treatments not only against UDCA monotherapy.

  6. Gene Expression by PBMC in Primary Sclerosing Cholangitis: Evidence for Dysregulation of Immune Mediated Genes

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    Christopher A. Aoki

    2006-01-01

    Full Text Available Primary sclerosing cholangitis (PSC is a chronic disease of the bile ducts characterized by an inflammatory infiltrate and obliterative fibrosis. The precise role of the immune system in the pathogenesis of PSC remains unknown. We used RNA microarray analysis to identify immune-related genes and pathways that are differentially expressed in PSC. Messenger RNA (mRNA from peripheral blood mononuclear cells (PBMC was isolated from both patients with PSC and age and sex matched healthy controls. Samples from 5 PSC patients and 5 controls were analyzed by microarray and based upon rigorous statistical analysis of the data, relevant genes were chosen for confirmation by RT-PCR in 10 PSC patients and 10 controls. Using unsupervised hierarchical clustering, gene expression in PSC was statistically different from our control population. Interestingly, genes within the IL-2 receptor beta, IL-6 and MAP Kinase pathways were found to be differently expressed in patients with PSC compared to controls. Further, individual genes, TNF-α induced protein 6 (TNFaip6 and membrane-spanning 4-domains, subfamily A (ms4a were found to be upregulated in PSC while similar to Mothers against decapentaplegic homolog 5 (SMAD 5 was downregulated. In conclusion, several immune-related pathways and genes were differentially expressed in PSC compared to control patients, giving further evidence that this disease is systemic and immune-mediated.

  7. Motion – Patients with Primary Sclerosing Cholangitis Should Undergo Early Liver Transplantation: Arguments against the Motion

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    Jeffrey S Crippin

    2002-01-01

    Full Text Available Liver transplantation is an accepted form of treatment for patients with primary sclerosing cholangitis (PSC and can provide long term survival. Cholangiocarcinoma occurs in 10% to 20% of patients with PSC, is difficult to diagnose and has a poor prognosis. It has been proposed that liver transplantation be undertaken early in the course of the PSC, before cancer develops. Such a proposal would have significant implications for the method of assigning priority to patients awaiting liver transplantation. Other patients on the waiting list would experience further delays, while there is no proven benefit for PSC patients. Few patients with this disease are removed from the waiting list because they developed cancer. If one were to state that PSC patients warrant special consideration because of the hypothetical risk of cholangiocarcinoma, the same argument could be applied to patients with hepatitis C and other causes of cirrhosis, who are at increased risk of hepatocellular carcinoma. The transplant allocation system is applied in an equitable fashion to patients with a large variety of liver diseases. Alteration of this system to benefit a small number of patients with PSC would violate the principles on which it was created, and cannot be justified.

  8. Imaging and estimation of the prognostic features of primary sclerosing cholangitis by ultrasonography and MR cholangiography

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    Oikarinen, H.; Paeaekkoe, E.; Suramo, I.; Paeivaensalo, M.; Tervonen, O. [Oulu Univ. Hospital (Finland). Dept. of Diagnostic Radiology; Lehtola, J.; Aukee, J. [Oulu Univ. Hospital (Finland). Dept. of Medicine

    2001-03-01

    Purpose: To evaluate the ability of US and MR cholangiography (MRC) to detect bile duct changes and prognostic signs of primary sclerosing cholangitis (PSC) seen at endoscopic retrograde cholangiography (ERC). Material and Methods: In a prospective study, 9 patients with PSC underwent US, MRC, MR imaging and ERC of the bile ducts and the liver. Eight age- and sex-matched control patients were examined with MRC, MR imaging and ERC. A segmental comparison was performed to assess the ability of MRC-MR and US to reveal the accurate ductal involvement in different segments of the biliary tree and the specific criteria of poor prognostic outcome in PSC. The ability of MRC-MR to detect the presence of PSC in different patients was analysed blindly. Results: MRC-MR depicted changes of PSC correctly in 9 patients (radiologist 1) and in 8 patients with 1 false-positive finding (radiologist 2) in the blinded analysis. In the segmental comparison, MRC missed especially bile duct dilatation. MRC was too pessimistic in the evaluation of the outcome. US detected features suggestive of PSC in 8 patients (radiologist 3). US was unable to show the predictors of poor outcome. Conclusion: MRC and US seem to be useful in the detection of PSC. US is unable and MRC is too pessimistic to estimate the outcome of PSC.

  9. Primary sclerosing cholangitis and cholangiocarcinoma as a diagnostic and therapeutic dilemma.

    Science.gov (United States)

    van Leeuwen, D J; Reeders, J W

    1999-01-01

    Differentiating primary sclerosing cholangitis (PSC) from cholangiocarcinoma (CC) can be a diagnostic challenge with major therapeutic implications. In case of advanced or symptomatic PSC, liver transplantation (OLTx) can be life saving with excellent long-term outcome. However, the outcome of CC diagnosed prior or during OLTx is dismal. PSC is a premalignant condition associated with a risk of developing cholangio- or hepatocellular carcinoma in > 15% of patients. Imaging diagnoses should be integrated into the further clinical data. It is the sudden, rapid and irreversible deterioration of the patient's condition, and the rapid progression of cholangiographic abnormalities, which may strongly point towards a malignancy or a malignant evolution in case of PSC. Brush cytology, (guided) biopsy, and tumor markers such as Ca 19.9 and CEA levels can be of some help, but confirmation of malignancy is often associated with a poor outcome and exclusion from liver transplantation. Clinical deterioration of the PSC patient and signs indicating advanced liver damage are a justification to evaluate patients for liver transplantation. Early transplantation should be considered in appropriate patients.

  10. Genetic Association of PTPN22 Polymorphisms with Autoimmune Hepatitis and Primary Biliary Cholangitis in Japan

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    Umemura, Takeji; Joshita, Satoru; Yamazaki, Tomoo; Komatsu, Michiharu; Katsuyama, Yoshihiko; Yoshizawa, Kaname; Tanaka, Eiji; Ota, Masao

    2016-01-01

    Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are liver-specific autoimmune conditions that are characterized by chronic hepatic damage and often lead to cirrhosis and hepatic failure. Specifically, the protein tyrosine phosphatase N22 (PTPN22) gene encodes the lymphoid protein tyrosine phosphatase, which acts as a negative regulator of T-cell receptor signaling. A missense single nucleotide polymorphism (SNP) (rs2476601) in PTPN22 has been linked to numerous autoimmune diseases in Caucasians. In the present series, nine SNPs in the PTPN22 gene were analyzed in 166 patients with AIH, 262 patients with PBC, and 322 healthy controls in the Japanese population using TaqMan assays. Although the functional rs3996649 and rs2476601 were non-polymorphic in all subject groups, the frequencies of the minor alleles at rs1217412, rs1217388, rs1217407, and rs2488458 were significantly decreased in AIH patients as compared with controls (all Pc < 0.05). There were no significant relationships with PTPN22 SNPs in PBC patients. Interestingly, the AAGTCCC haplotype was significantly associated with resistance to both AIH (odds ratio [OR] = 0.58, P = 0.0067) and PBC (OR = 0.58, P = 0.0048). SNPs in the PTPN22 gene may therefore play key roles in the genetic resistance to autoimmune liver disease in the Japanese. PMID:27406031

  11. Treatment of primary biliary cholangitis ursodeoxycholic acid non-responders: A systematic review.

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    Suraweera, Duminda; Rahal, Harman; Jimenez, Melissa; Viramontes, Matthew; Choi, Gina; Saab, Sammy

    2017-05-18

    Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic cholestatic liver disease characterized by an immune mediated destruction of intrahepatic bile ducts. Ursodeoxycholic acid (UDCA) has been the primary medication for the treatment of PBC, resulting in improved liver tests, resolution of symptoms and increased transplant free survival. However, not all patients respond to UDCA. The aim of this systematic review is to provide an evidence based assessment of the medications that have been studied in patients who are refractory to UDCA. We performed a systematic literature search on MEDLINE and the Cochrane Database of Systematic Reviews of the published literature. A total of 23 articles fulfilling our inclusion criteria were found. Several studies have shown an improvement in liver biochemistries with the use of obeticholic acid in conjunction with UDCA. Fibrates, including fenofibrate and bezafibrate, have evidence supporting benefit in this population but need more robust studies to confirm these observational results. Neither obeticholic acid nor fibrates have shown to increase transplant free survival. While there may be some benefit with methotrexate, colchicine, budesonide, mycophenolate mofetil and azathioprine, these findings were not consistent and the benefits were marginal. Further investigation is needed. In patients with PBC refractory to UDCA, obeticholic acid or a fibrate is a reasonable choice as an adjunctive treatment to UDCA. Further investigation with randomized controlled trials is needed to provide high quality evidence to formulate standardized therapies in this difficult to treat population. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  12. Impact of Microbes on the Pathogenesis of Primary Biliary Cirrhosis (PBC and Primary Sclerosing Cholangitis (PSC

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    Jochen Mattner

    2016-11-01

    Full Text Available Primary biliary cirrhosis (PBC and primary sclerosing cholangitis (PSC represent the major clinical entities of chronic cholestatic liver diseases. Both disorders are characterized by portal inflammation and slowly progress to obliterative fibrosis and eventually liver cirrhosis. Although immune-pathogenic mechanisms have been implicated in the pathogenesis of PBC and PSC, neither disorder is considered to be a classical autoimmune disease, as PSC and PBC patients do not respond to immune-suppressants. Furthermore, the decreased bile flow resulting from the immune-mediated tissue assault and the subsequent accumulation of toxic bile products in PBC and PSC not only perpetuates biliary epithelial damage, but also alters the composition of the intestinal and biliary microbiota and its mutual interactions with the host. Consistent with the close association of PSC and inflammatory bowel disease (IBD, the polyclonal hyper IgM response in PBC and (auto-antibodies which cross-react to microbial antigens in both diseases, an expansion of individual microbes leads to shifts in the composition of the intestinal or biliary microbiota and a subsequent altered integrity of epithelial layers, promoting microbial translocation. These changes have been implicated in the pathogenesis of both devastating disorders. Thus, we will discuss here these recent findings in the context of novel and alternative therapeutic options.

  13. Hydatid cyst and fungal infection: a case report

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    Haji Nasrollah E

    2009-02-01

    Full Text Available "nBackground: Hydatid cyst is a zoonosis rarely occurred except in endemic areas that capable of making pulmonary cavities fascilating fungus growth within it. Aspergillo-ma is a glob formed by hyphae from saprophyte growth of aspergillous specious in previously performed cavities within pulmonary parenchyma. "nCase report: A 28 years old male patient without any comorbidity presented in emergency department with progressive two month dyspnea. Tube thoracostomy is done because of respiratory distress and massive hydropneumothorax. Thoracotomy and lobectomy is performed due to complicated hydatid cyst. Histopathologic investigation reveals hydatid cyst layers with fungal hyphae within it on granulomatous background. "nConclusion: Hydatid cyst with fungal contamination must be mentioned in differentials of dyspnea with lower segment lung cavities, especially in endemic areas. Thoracic CT scan with IV contrast can reveal fungus ball. Surgery is a treatment of choice and capitonage can be a prophylactic measure from secondary fungal infection in hydatid cyst surgery.

  14. Recurrent Cholangitis by Biliary Stasis Due to Non-Obstructive Afferent Loop Syndrome After Pylorus-Preserving Pancreatoduodenectomy: Report of a Case

    Science.gov (United States)

    Sanada, Yukihiro; Yamada, Naoya; Taguchi, Masanobu; Morishima, Kazue; Kasahara, Naoya; Kaneda, Yuji; Miki, Atsushi; Ishiguro, Yasunao; Kurogochi, Akira; Endo, Kazuhiro; Koizumi, Masaru; Sasanuma, Hideki; Fujiwara, Takehito; Sakuma, Yasunaru; Shimizu, Atsushi; Hyodo, Masanobu; Sata, Naohiro; Yasuda, Yoshikazu

    2014-01-01

    We report a 71-year-old man who had undergone pylorus-preserving pancreatoduodenectomy (PPPD) using PPPD-IV reconstruction for cholangiocarcinoma. For 6 years thereafter, he had suffered recurrent cholangitis, and also a right liver abscess (S5/8), which required percutaneous drainage at 9 years after PPPD. At 16 years after PPPD, he had been admitted to the other hospital because of acute purulent cholangitis. Although medical treatment resolved the cholangitis, the patient was referred to our hospital because of dilatation of the intrahepatic biliary duct (B2). Peroral double-balloon enteroscopy revealed that the diameter of the hepaticojejunostomy anastomosis was 12 mm, and cholangiography detected intrahepatic stones. Lithotripsy was performed using a basket catheter. At 1 year after lithotripsy procedure, the patient is doing well. Hepatobiliary scintigraphy at 60 minutes after intravenous injection demonstrated that deposit of the tracer still remained in the upper afferent loop jejunum. Therefore, we considered that the recurrent cholangitis, liver abscess, and intrahepatic lithiasis have been caused by biliary stasis due to nonobstructive afferent loop syndrome. Biliary retention due to nonobstructive afferent loop syndrome may cause recurrent cholangitis or liver abscess after hepaticojejunostomy, and double-balloon enteroscopy and hepatobiliary scintigraphy are useful for the diagnosis of nonobstructive afferent loop syndrome. PMID:25058778

  15. Small duct autoimmune sclerosing cholangitis and Crohn colitis in a 10-year-old child. A case report and review of the literature

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    Larsen Erling

    2012-08-01

    Full Text Available Abstract Autoimmune sclerosing cholangitis is an overlap syndrome characterized by features of both autoimmune hepatitis and primary sclerosing cholangitis, the latter usually involving the large bile ducts. Autoimmune sclerosing cholangitis occurs more often in children than in adults and is frequently associated with inflammatory bowel disease, predominantly ulcerative colitis. We report a unique case of a 10-year-old Danish boy with severe small duct autoimmune sclerosing cholangitis and synchronic Crohn colitis. He was referred with a history of weight loss, abdominal pain, vomiting and diarrhea. Biochemical anomalies included elevated alanine aminotransferase, γ-glutamyl transferase and immunoglobulin G levels and the presence of smooth muscle antibodies and perinuclear antineutrophil cytoplasmic antibodies but normal alkaline phosphatase. Liver biopsy specimen revealed features of both autoimmune hepatitis and sclerosing cholangitis, the latter characterized by acute, hyperplastic and destructive inflammation – granulocytic epithelial lesion – of the small ducts. Magnetic resonance cholangiography was normal. Colonoscopic biopsies showed chronic inflammatory changes of the caecum and the ascending and transverse colon compatible with Crohn disease. Ursodeoxycholic acid and immunosuppressive treatment was initiated and within four weeks of treatment the general condition improved. Normalization of aminotransferase was seen at 21 weeks and γ-glutamyl transferase at 72 weeks after first admittance, while immunoglobulin G remained slightly increased. Virtual slides The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1418596609736470

  16. Familial occurrence of autoimmune liver disease with overlapping features of primary biliary cholangitis and autoimmune hepatitis in a mother and her daughter.

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    Omori, Kaoru; Yoshida, Kanako; Yokota, Masaki; Daa, Tsutomu; Kan, Masahiro

    2016-10-01

    We encountered two patients with overlapping features of primary biliary cholangitis and autoimmune hepatitis within the same family. A 68-year-old woman presented at our hospital from a previous medical institution because of the diagnosis of primary biliary cholangitis. Her 49-year-old daughter was admitted with liver dysfunction 4 years later. When compared, these two related patients were found to have overlapping features of primary biliary cholangitis and autoimmune hepatitis. Their human leukocyte antigen haplotype was DRB1*04:05/DRB1*15:02. The clinical and biochemical findings of these two patients immediately improved following treatment with a combination of prednisolone and ursodeoxycholic acid, in accordance with the Japanese guidelines. It is extremely important to identify such pathological conditions as quickly as possible, particularly with the appearance of severe liver dysfunction due to liver cirrhosis, as observed in our case. The Japanese guidelines are considered to be a realistic and useful clinical policy for the swift and efficient treatment of patients with overlapping features of primary biliary cholangitis and autoimmune hepatitis. We suggest that our two patients presented with a genetic predisposition to autoimmune liver disease with overlapping features of primary biliary cholangitis and autoimmune hepatitis within the same family.

  17. Epidemiology of fungal infections and risk factors in newborn patients

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    Paolo Manzoni

    2013-07-01

    Full Text Available The incidence of fungal infections among newborn babies is increasing, owing mainly to the in­creased ability to care and make survive immature infants at higher specific risk for fungal infections. The risk is higher in infants with very low and extremely low birth weight, in babies receiving total parenteral nutrition, in neonates with limited barrier effect in the gut, or with central venous catheter or other devices where fungal biofilms can originate. Also neonates receiving broad spectrum antibiotics, born through caesarian section or non-breastfed can feature an increased, specific risk. Most fungal infections in neonatology occur in premature children, are of nosocomial origin, and are due to Candida species. Colonization is a preliminary step, and some factors must be considered for the diagnosis and grading process: the iso­lation site, the number of colonized sites, the intensity of colonization, and the Candida subspecies. The most complicated patients are at greater risk of fungal infections, and prophylaxis or pre-emptive therapy should often be considered. A consistent decisional tree in neonatology is yet to be defined, but some efforts have been made in order to identify characteristics that should guide the prophylaxis or treatment choices. A negative blood culture and the absence of symptoms aren’t enough to rule out the diagnosis of fungal infections in newborn babies. Similarly, laboratory tests have been validated only for adults. The clinical judgement is of utmost importance in the diagnostic process, and should take into account the presence of clinical signs of infection, of a severe clinical deterioration, as well as changes in some laboratory tests, and also the presence and characteristics of a pre-existing fungal colonization.http://dx.doi.org/10.7175/rhc.v14i1S.856

  18. Risk factors for invasive fungal infections in liver transplant recipients

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    LIAO Yong; Thomas Hartmann; AO Jun-hong; YANG Rong-ya

    2012-01-01

    To the editor:Invasive fungal infections (IFIs) are significant complications in liver transplant recipients, which are associated with high morbidity/mortality and higher healthcare costs.The incidence of IFIs is mainly influenced by the patients'clinical condition,the level of immune suppression,surgical factors,and the technical complexity of the surgery.We read with great interest the research article by Shi et al.1 Although they have reached a high curative ratio with their "experiential" therapy based on their previous clinical experience,they did not provide us with detailed,definite criteria for identifying suspected patients and allowing for their early "experiential" treatment.Updated,standardized guidelines from the Invasive Fungal Infections Cooperative Group in Europe and the Mycoses Study Group in the United States provide definitions of patients,who are eligible for enrollment in clinical trials.The "Probable" and "Possible" IFIs were defined based on specific host factors,clinical manifestations of fungal infection and mycological findings.The current diagnostic methods for fungal infections lack sensitivity and specificity,so understanding the risk factors associated with fungal infections in liver transplant recipients may improve identification of high-risk patients and guide appropriate initiation of early antifungal treatment.

  19. 硬化性胆管炎的影像诊断和鉴别诊断%The imaging and differential diagnosis of sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    杨艳; 董莘

    2016-01-01

    硬化性胆管炎包括一系列以肝内外胆管炎症、纤维化和狭窄为特征的慢性及进展性胆汁郁积性肝病。硬化性胆管炎可以分为原发性硬化性胆管炎(PSC)和继发性硬化性胆管炎,前者病因未明;后者致病原因不同,包括IgG4-相关性硬化性胆管炎、复发性化脓性胆管炎、缺血性胆管炎、获得性免疫缺陷综合征相关性胆管炎和嗜酸性胆管炎。本研究在文献评阅的基础上,综合了不同系列硬化性胆管炎的影像表现,强调用系统方法鉴别诊断,提示支持诊断恶性胆道狭窄而非硬化性胆管炎的影像特征为:①门静脉期相对肝实质明显强化的狭窄节段;②长的狭窄长度(>12 mm);③明显的胆管壁增厚(>3 mm),不清楚的外边缘;④管腔不规则;以及不对称狭窄(70)。排除恶性胆道狭窄后,才是区分PSC和继发性硬化性胆管炎;还应结合人口统计、实验室检查和患者既往病史一并考虑。同时也讨论了磁共振胆管造影和内镜胆管造影在硬化性胆管炎诊断和鉴别诊断中作用以及治疗选项的临床意义。%Sclerosing cholangitis includes a spectrum of chronic, variably progressive cholestatic liver disease characterized by inflammation, fibrosis and stricture of the intrahepatic and extrahepatic bile ducts. Sclerosing cholangitis can be divided into primary sclerosing cholangitis of unidentified etiology and secondary sclerosing cholangitis caused by various identifiable etiologies, including immunoglobulin G4-related sclerosing cholangitis, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndromerelated cholangitis and eosinophilic cholangitis. In this article, we describe the imaging findings of the various spectrums of sclerosing cholangitis with anemphasis on a systemic approach in differential diagnosis. It is very important for magnetic resonance cholangiography and endoscopic

  20. Fungal periprosthetic joint infection in total knee arthroplasty: a systematic review

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    Oliver Jakobs

    2015-03-01

    Full Text Available Fungal periprosthetic joint infection (PJI is a rare but devastating complication following total knee arthroplasty (TKA. A standardized procedure regarding an accurate treatment of this serious complication of knee arthroplasty is lacking. In this systematic review, we collected data from 36 studies with a total of 45 reported cases of a TKA complicated by a fungal PJI. Subsequently, an analysis focusing on diagnostic, medicaments and surgical procedures in the pre-, intra- and postoperative period was performed. Candida spp. accounts for about 80% (36 out of 45 cases of fungal PJIs and is therefore the most frequently reported pathogen. A systemic antifungal therapy was administered in all but one patient whereas a local antifungal therapy, e.g. the use of an impregnated spacer, is of inferior relevance. Resection arthroplasty with delayed re-implantation (two-stage revision was the surgical treatment of choice. However, in 50% of all reported cases the surgical therapy was heterogeneous. The outcome under a combined therapy was moderate with recurrent fungal PJI in 11 patients and subsequent bacterial PJI as a main complication in 5 patients. In summary, this systematic review integrates data from up to date 45 reported cases of a fungal PJI of a TKA. On the basis of the current literature strategies for the treatment of this devastating complication after TKA are discussed

  1. Malnutrition is related to a higher frequency of serious complications in patients with cirrhosis

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    E. Pérez-Reyes

    2016-01-01

    Conclusions: The presence of serious complications, such as, ascites and development of bacterial and fungal infections, was more frequent between cirrhotic patients with malnutrition than in those well-nourished.

  2. Late Complications following Endoscopic Sphincterotomy for Choledocholithiasis: A Swedish Population-Based Study

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    A. Langerth

    2014-01-01

    Full Text Available In order to assess the risk of long-term complications following endoscopic sphincterotomy (ES for common bile duct stones (CBDS, we conducted a cohort study. The study included 1,113 patients who underwent ES for CBDS in six different hospitals in central Sweden between 1977 and 1990. Through the use of the Swedish population registry, each patient was assigned five population-based controls matched for sex and age. Linkage to the Inpatient Registry yielded information on morbidity and mortality for the patients as well as for the controls. After one year of washout, there were 964 patients available for follow-up. The mean age was 70.6 years, 57% were women, and the mean length of follow-up was 8.9 years. The patients’ overall morbidity was significantly higher and we observed a tendency towards increased mortality as well. Recurrent CBDS was diagnosed in 4.1% of the patients. Acute cholangitis with a hazard ratio (HR of 36 (95%CI 11–119.4 was associated with recurrent CBDS in 39% of the patients. HR for acute pancreatitis was 6.2 (95%CI 3.4–11.3 and only one patient had CBDS at the same time. In conclusion, we consider acute pancreatitis and cholangitis both as probable long-term complications after ES.

  3. PR3-ANCA: a promising biomarker in primary sclerosing cholangitis (PSC.

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    Laura M Stinton

    Full Text Available The only recognized biomarker for primary sclerosing cholangitis (PSC is atypical anti-neutrophil cytoplasmic antibodies (aANCA, which, in addition to having low sensitivity and specificity, is an indirect immunofluorescence (IIF test lacking the advantages of high throughput and objectivity. Recent reports have shown that antibodies to proteinase-3 (PR3-ANCA might add diagnostic value in inflammatory bowel disease (IBD, specifically in ulcerative colitis (UC. As PSC is associated with IBD, the objective of this study was to evaluate the frequency and clinical significance of PR3-ANCA in a large cohort of patients.A total of 244 PSC and 254 control [autoimmune hepatitis (AIH, primary biliary cirrhosis (PBC, hepatitis C viral infection (HCV, hepatitis B viral infection (HBV, and healthy controls] sera and their clinical correlations were retrospectively analyzed for PR3-ANCA determined by ELISA and a new chemiluminescence immunoassay (CIA. Testing was also performed for aANCA by IIF.When measured by CIA, PR3-ANCA was detected in 38.5% (94/244 of PSC patients compared to 10.6% (27/254 controls (p<0.0001. By ELISA, PR3-ANCA was detected in 23.4% (57/244 of PSC patients compared to 2.7% (6/254 controls (p<0.0001. PR3-ANCA in PSC patients was not associated with the presence or type of underlying IBD, and, in fact, it was more frequent in Crohn's disease (CD patients with PSC than previously reported in CD alone. PR3-ANCA in PSC measured by CIA correlated with higher liver enzymes.PR3-ANCA is detected in a significant proportion of PSC patients compared to other liver diseases including PBC and AIH. PR3-ANCA is associated with higher liver enzyme levels in PSC, and is not solely related to underlying IBD.

  4. TGR5: pathogenetic role and/or therapeutic target in fibrosing cholangitis?

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    Keitel, Verena; Reich, Maria; Häussinger, Dieter

    2015-06-01

    Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease affecting the intrahepatic and extrahepatic biliary tree leading to bile duct strictures, progressive cholestasis, and development of liver fibrosis and cirrhosis. The pathogenesis of PSC is still elusive; however, both an immune-mediated injury of the bile ducts as well as increased recruitment of intestinal-primed T lymphocytes to the biliary tracts seem to contribute to disease development and progression. TGR5 (Gpbar-1) is a G-protein-coupled receptor responsive to bile acids, which is expressed in cholangiocytes, intestinal epithelial cells, and macrophages of the liver and intestine as well as in CD14-positive monocytes of the peripheral blood. Activation of TGR5 in biliary epithelial cells promotes chloride and bicarbonate secretion, triggers cell proliferation, and prevents apoptotic cell death. In immune cells, stimulation of TGR5 inhibits cytokine expression and secretion, thus reducing systemic as well as hepatic and intestinal inflammation. The expression pattern of TGR5 in the liver and intestine as well as the potential protective functions of TGR5 suggest a role for this receptor in the pathogenesis of PSC. While mutations in the coding region of the TGR5 gene are too rare to contribute to overall disease susceptibility, the expression and localization of the receptor have not been studied in PSC livers. Pharmacological activation of TGR5 in mice promotes protective mechanisms in biliary epithelial cells and reduces hepatic and systemic inflammation; however, it also provokes pruritus. Further studies are needed to predict the potential benefits as well as side effects of TGR5 agonist treatment in PSC patients.

  5. Greater Biosynthetic Liver Dysfunction in Primary Sclerosing Cholangitis Suggests Co-existent or Impending Cholangiocarcinoma

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    Trilianos, Panagiotis; Agnihotri, Abhishek; Ucbilek, Enver; Gurakar, Ahmet

    2016-01-01

    Background and Aim: Patients with primary sclerosing cholangitis (PSC) who develop cholangiocarcinoma (CCA) have a median survival of less than 6 months. In half of cases, PSC and CCA will be diagnosed either concurrently or within a year of one another. The aim of the present study is to demonstrate that the degree of biochemical liver dysfunction is associated with concomitant or impending CCA. Methods: We did a chart review of patients diagnosed with PSC and CCA up to 18 months from presentation (“CCA” group) as well as patients with PSC that underwent transplantation with no sign of CCA in their explanted liver (“nCCA” group). Along with demographic data and follow-up length, we recorded their presenting liver function tests, including alanine and aspartate aminotransferases (ALT, AST), total bilirubin (TBil), alkaline phosphatase (ALP), international normalization ratio (INR), and serum Ca 19-9 levels. Differences between mean values of the two groups were analyzed with a student’s t-test. Results: Twenty-four patients were included. The “CCA” group consisted of eight patients, and the “non-CCA” group had 16 patients. There was no significant difference between the two groups in their presenting values of ALT, ALP, or serum Ca 19-9. However, the “CCA” group had significantly higher levels of AST, TBil, and INR. Conclusion: Patients with PSC and concurrent or impending CCA appear to exhibit significantly greater biochemical liver dysfunction than those who do not develop CCA. Therefore, newly-diagnosed PSC patients presenting with these findings may warrant more rigorous evaluation. PMID:27047765

  6. Evaluation of Biliary Calprotectin as a Biomarker in Primary Sclerosing Cholangitis

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    Gauss, Annika; Sauer, Peter; Stiehl, Adolf; Rupp, Christian; Krisam, Johannes; Leopold, Yvonne; Kloeters-Plachky, Petra; Stremmel, Wolfgang; Gotthardt, Daniel

    2016-01-01

    Abstract Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the bile ducts with limited therapeutic options except liver transplantation. Reliable biomarkers to predict the disease course are unavailable, and currently employed disease activity scores such as the Mayo risk score (MRS) have limitations. The present study aims to evaluate biliary calprotectin as a marker of disease activity and prognosis in PSC. This is a monocentric retrospective observational study. Calprotectin concentrations were measured by an enzyme-linked immunosorbent assay in bile samples collected by endoscopic retrograde cholangiography from 106 PSC patients and 20 controls. Biliary calprotectin concentrations were compared between the 2 groups. In PSC patients, results were evaluated with regard to the presence of dominant bile duct stenoses, bile microbiology, MRS, survival free of liver transplantation, and necessity for bile duct interventions in the further disease course. Median (interquartile ranges) biliary calprotectin concentrations were higher in PSC patients than in controls (3646 ng/mL, 249–9748 vs 116 ng/mL, 104–655; P PSC cohort, higher biliary calprotectin concentrations were associated with the presence of microbes in bile (P = 0.02), the occurrence of dominant bile duct stenosis at any time in the disease course (P = 0.005), and the necessity for future bile duct interventions (P = 0.02). Patients with biliary calprotectin concentrations above a cut-off of 11,610 ng/mL displayed significantly shorter transplantation-free survival than those with biliary calprotectin concentrations ≤11,610 ng/mL (P 11,610 ng/mL) as a risk factor of shorter transplantation-free survival of PSC patients (P 142.5 U/L) (P = 0.006), high MRS (≥2) (P PSC, so that further studies for evaluation of calprotectin in this disease are warranted. PMID:27124059

  7. Magnetic resonance cholangiography compared with endoscopic retrograde cholangiography in the diagnosis of primary sclerosing cholangitis

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    Hossein Ahrar

    2014-01-01

    Full Text Available Background: Magnetic resonance cholangiography (MRC has gained popularity for diagnosing primary sclerosing cholangitis (PSC. We determined the accuracy of MRC compared with endoscopic retrograde cholangiography (ERC for diagnosing PSC. Materials and Methods: This retrospective case-control study was conducted on patients referred to an outpatient gastroenterology clinic from 2001 to 2013. Patients with established diagnosis of PSC who had undergone MRC and ERC within a 6-month interval were included. Controls were selected from patients who had undergone imaging for reasons other than PSC evaluation. Disease outcome at the study time and liver biochemistry data at diagnosis and 1-year thereafter were retrieved. Diagnostic accuracy of MRC in comparison with ERC was evaluated. Results: A total of 46 definite PSC patients (age at diagnosis = 36.8 ± 11.6 years, 33 male were found. Diagnostic imaging for PSC was ERC alone in 12, MRC alone in 23, and ERC plus MRC in 11 patients. Controls were 89 patients mostly with bile stones. The sensitivity, specificity, and positive and negative likelihood ratios of MRC was 90.9%, 95.5%, 20.23, and 0.10, respectively. Early PSC was found more frequently by MRC compared with ERC (30.4% vs. 8.3%, P = 0.146. No significant difference was found between imaging modalities with regards to patients′ outcome (P = 0.786 or liver biochemistry at diagnosis or 1-year thereafter (P >0.05. Conclusion: Starting diagnostic imaging for PSC with MRC seems better and may provide diagnosis of PSC at its earlier phase. Further studies with larger sample of patients and longer follow-ups are warranted.

  8. A morphometric study of the hepatic arterioles in end-stage primary sclerosing cholangitis.

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    Fiel, M Isabel; Sima, Hamid R; Azarian, Amirabbas; Schiano, Thomas D

    2015-02-01

    Primary sclerosing cholangitis (PSC) is typified by a heterogeneous histology with periductal fibroinflammatory lesions. The hepatic arterioles in PSC have not been well characterized. Using image analysis, we sought to examine the dimensions of hepatic arterioles in PSC. We identified 30 livers from patients transplanted for PSC as well as 10 explants each from cirrhotic patients (serving as controls) having primary biliary cirrhosis, hepatitis C (HCV), and alcoholic liver disease. At least two representative hematoxylin and eosin-stained slides were selected, and ten cross-sectioned hepatic arterioles were photographed for image analysis. The vessels were measured at their longest span and width based on the outer portions of the tunica media. Wall thickness was measured at its thickest portion from the intima to the outer portion of the tunica media; the perimeter of the luminal area was outlined by the endothelial lining, generating the total luminal area. Mean arteriolar length, width, and wall thickness (p = 0.012, p = 0.004, p = 0.001, respectively) were greater in the PSC group; luminal area was similar between the groups. When compared to the individual sub-groups, wall thickness of arterioles in PSC remained significantly greater. End-stage PSC has even larger-sized arterioles and greater wall thickness as compared to that of other cirrhotic livers. This increased wall thickness found in PSC cannot be solely attributed to cirrhosis itself. These vessel changes may potentially be the result of, or contribute to, the pathogenesis of PSC.

  9. Mutational characterization of the bile acid receptor TGR5 in primary sclerosing cholangitis.

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    Johannes R Hov

    Full Text Available BACKGROUND: TGR5, the G protein-coupled bile acid receptor 1 (GPBAR1, has been linked to inflammatory pathways as well as bile homeostasis, and could therefore be involved in primary sclerosing cholangitis (PSC a chronic inflammatory bile duct disease. We aimed to extensively investigate TGR5 sequence variation in PSC, as well as functionally characterize detected variants. METHODOLOGY/PRINCIPAL FINDINGS: Complete resequencing of TGR5 was performed in 267 PSC patients and 274 healthy controls. Six nonsynonymous mutations were identified in addition to 16 other novel single-nucleotide polymorphisms. To investigate the impact from the nonsynonymous variants on TGR5, we created a receptor model, and introduced mutated TGR5 constructs into human epithelial cell lines. By using confocal microscopy, flow cytometry and a cAMP-sensitive luciferase assay, five of the nonsynonymous mutations (W83R, V178M, A217P, S272G and Q296X were found to reduce or abolish TGR5 function. Fine-mapping of the previously reported PSC and UC associated locus at chromosome 2q35 in large patient panels revealed an overall association between the TGR5 single-nucleotide polymorphism rs11554825 and PSC (odds ratio = 1.14, 95% confidence interval: 1.03-1.26, p = 0.010 and UC (odds ratio = 1.19, 95% confidence interval 1.11-1.27, p = 8.5 x 10(-7, but strong linkage disequilibrium precluded demarcation of TGR5 from neighboring genes. CONCLUSIONS/SIGNIFICANCE: Resequencing of TGR5 along with functional investigations of novel variants provided unique insight into an important candidate gene for several inflammatory and metabolic conditions. While significant TGR5 associations were detected in both UC and PSC, further studies are needed to conclusively define the role of TGR5 variation in these diseases.

  10. Primary sclerosing cholangitis is characterised by intestinal dysbiosis independent from IBD

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    Sabino, João; Vieira-Silva, Sara; Machiels, Kathleen; Joossens, Marie; Falony, Gwen; Ballet, Vera; Ferrante, Marc; Van Assche, Gert; Van der Merwe, Schalk; Vermeire, Severine; Raes, Jeroen

    2016-01-01

    Objective Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease often leading to end-stage liver disease. Its pathogenesis remains largely unknown, although frequent concomitant IBD hints towards common factors underlying gut and bile duct inflammation. Considering the mounting evidence on the involvement of the intestinal microbiota in initiating and determining IBD phenotype, we investigated intestinal microbiota composition in patients with PSC. Design Stool samples were collected from 147 individuals (52 patients with PSC, 52 age, gender and body mass index-matched healthy volunteers, 13 UC and 30 patients with Crohn's disease). An independent validation cohort of 14 PSC and 14 matched controls was recruited. 16S rDNA sequencing of faecal DNA was performed (Illumina MiSeq). Results The microbiota of patients with PSC was characterised by decreased microbiota diversity, and a significant overrepresentation of Enterococcus (p=3.76e-05), Fusobacterium (p=3.76e-05) and Lactobacillus (p=0.0002) genera. This dysbiosis was present in patients with PSC with and without concomitant IBD and was distinct from IBD, and independent of treatment with ursodeoxycholic acid. A decision tree based on abundances of these three genera allowed reliable classification in the validation cohort. In particular, one operational taxonomic unit belonging to the Enterococcus genus was associated with increased levels of serum alkaline phosphatase (p=0.048), a marker of disease severity. Conclusions We here present the first report of PSC-associated faecal dysbiosis, independent from IBD signatures, suggesting the intestinal microbiota could be a contributing factor in PSC pathogenesis. Further studies are needed to confirm these findings and assess causality. PMID:27207975

  11. S100A9 is a biliary protein marker of disease activity in primary sclerosing cholangitis.

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    Lisa Reinhard

    Full Text Available BACKGROUND AND AIMS: Bile analysis has the potential to serve as a surrogate marker for inflammatory and neoplastic disorders of the biliary epithelium and may provide insight into biliary pathophysiology and possible diagnostic markers. We aimed to identify biliary protein markers of patients with primary sclerosing cholangitis (PSC by a proteomic approach. METHODS: Bile duct-derived bile samples were collected from PSC patients (n = 45 or patients with choledocholithiasis (n = 24, the control group. Liquid chromatography-tandem mass spectrometry (LC-MS/MS was performed to analyse the proteins, 2-D-gel patterns were compared by densitometry, and brush cytology specimens were analysed by RT-PCR. RESULTS: A reference bile-duct bile proteome was established in the control group without signs of inflammation or maligancy comprising a total of 379 non-redundant biliary proteins; 21% were of unknown function and 24% had been previously described in serum. In PSC patients, the biliary S100A9 expression was elevated 95-fold (p<0.005, serum protein expression was decreased, and pancreatic enzyme expression was unchanged compared to controls. The S100A9 expression was 2-fold higher in PSC patients with high disease activity than in those with low activity (p<0.05. The brush cytology specimens from the PSC patients with high disease activity showed marked inflammatory activity and leukocyte infiltration compared to the patients with low activity, which correlated with S100A9 mRNA expression (p<0.05. CONCLUSIONS: The bile-duct bile proteome is complex and its analysis might enhance the understanding of cholestatic liver disease. Biliary S100A9 levels may be a useful marker for PSC activity, and its implication in inflammation and carcinogenesis warrants further investigation.

  12. Risk of secondary osteoporosis due to lobular cholestasis in non-cirrhotic primary biliary cholangitis.

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    Seki, Anna; Ikeda, Fusao; Miyatake, Hirokazu; Takaguchi, Koichi; Hayashi, Shosaku; Osawa, Toshiya; Fujioka, Shin-Ichi; Tanaka, Ryoji; Ando, Masaharu; Seki, Hiroyuki; Iwasaki, Yoshiaki; Yamamoto, Kazuhide; Okada, Hiroyuki

    2017-09-01

    It remains unclear whether primary biliary cholangitis (PBC) represents a risk factor for secondary osteoporosis. A case-control study was conducted to examine bone mineral density and bone turnover markers in middle-aged postmenopausal PBC patients without liver cirrhosis. We compared the incidence of low bone mineral density between propensity-score matched subgroups of PBC patients and healthy controls and investigated the mechanisms underlying unbalanced bone turnover in terms of the associations between bone turnover markers and PBC-specific histological findings. Our analysis included 128 consecutive PBC patients, all postmenopausal women aged in their 50s or 60s, without liver cirrhosis or fragility fracture at the time of PBC diagnosis. The prevalence of osteoporosis was significantly higher in the PBC group than in the control group (26% vs 10%, P = 0.015, the Fisher exact probability test). In most PBC patients (95%), the level of bone-specific alkaline phosphatase was above the normal range, indicating increased bone formation. On the other hand, the urine type I collagen-cross-linked N-telopeptide showed variable levels among our PBC patients, indicating unbalanced bone resorption. Advanced fibrosis was associated with low bone turnover. Lobular cholestasis, evaluated as aberrant keratin 7 expression in hepatocytes, showed significant negative correlations with bone formation and resorption, indicating low bone turnover. Our results show that, compared with healthy controls, even non-cirrhotic PBC patients have significantly higher risk of osteoporosis. Moreover, lobular cholestasis was associated with low bone turnover, suggesting this feature of PBC may itself cause secondary osteoporosis in PBC patients. © 2017 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.

  13. Refinement of the MHC risk map in a scandinavian primary sclerosing cholangitis population.

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    Sigrid Næss

    Full Text Available Genetic variants within the major histocompatibility complex (MHC represent the strongest genetic susceptibility factors for primary sclerosing cholangitis (PSC. Identifying the causal variants within this genetic complex represents a major challenge due to strong linkage disequilibrium and an overall high physical density of candidate variants. We aimed to refine the MHC association in a geographically restricted PSC patient panel.A total of 365 PSC cases and 368 healthy controls of Scandinavian ancestry were included in the study. We incorporated data from HLA typing (HLA-A, -B, -C, -DRB3, -DRB1, -DQB1 and single nucleotide polymorphisms across the MHC (n = 18,644; genotyped and imputed alongside previously suggested PSC risk determinants in the MHC, i.e. amino acid variation of DRβ, a MICA microsatellite polymorphism and HLA-C and HLA-B according to their ligand properties for killer immunoglobulin-like receptors. Breakdowns of the association signal by unconditional and conditional logistic regression analyses demarcated multiple PSC associated MHC haplotypes, and for eight of these classical HLA class I and II alleles represented the strongest association. A novel independent risk locus was detected near NOTCH4 in the HLA class III region, tagged by rs116212904 (odds ratio [95% confidence interval] = 2.32 [1.80, 3.00], P = 1.35×10-11.Our study shows that classical HLA class I and II alleles, predominantly at HLA-B and HLA-DRB1, are the main risk factors for PSC in the MHC. In addition, the present assessments demonstrated for the first time an association near NOTCH4 in the HLA class III region.

  14. Primary sclerosing cholangitis in genetically diverse populations listed for liver transplantation: Unique clinical and HLA associations

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    Bowlus, Christopher L.; Li, Chin-Shang; Karlsen, Tom H.; Lie, Benedicte A.; Selmi, Carlo

    2010-01-01

    Primary sclerosing cholangitis (PSC) is well characterized in European populations. We aimed to characterize clinical characteristics and human leukocyte antigen (HLA) associations in a population of European American, Hispanic and African-American PSC patients listed for liver transplantation. Demographic, clinical, and HLA data stratified by population from 6,767 liver transplant (LT) registrants of the United Network for Organ Sharing (UNOS) with a diagnosis of PSC (4.7% of registrants) were compared to registrants with other diagnoses. Compared to European Americans and Hispanics, African American cases were significantly younger (46.6 ± 13.7, 42.3 ± 15.9, and 39.7 ± 13.1, respectively; p = 0.002), listed with a higher Model of End Stage Liver Disease (MELD) score (15.2 ± 7.5, 14.9 ± 7.6, and 18.1 ± 9.3, respectively; p = 0.001), and less frequently noted to have inflammatory bowel disease (71.4% versus 60.5%, p < 0.01) compared to European Americans. In multivariate analysis, African origin was a significant factor associated with listing for LT with PSC (OR relative to European Americans 1.33, 95% C.I. 1.27 – 1.41). HLA associations in European Americans, Hispanics and African Americans with PSC compared to alcoholic liver disease were detected for HLA-B8, HLA-DR13 and the protective HLA-DR4. However, HLA-DR3, which is in linkage disequilibrium with HLA-B8, only showed associations in European Americans and Hispanics. African Americans with PSC listed for LT differ clinically from European Americans and Hispanics. The association with HLA-B8 but not HLA-DR3 in African Americans should make possible the refinement of the HLA associations in PSC. PMID:21031548

  15. Recurrent blood eosinophilia in ulcerative colitis is associated with severe disease and primary sclerosing cholangitis.

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    Barrie, Arthur; Mourabet, Marwa El; Weyant, Katherine; Clarke, Kofi; Gajendran, Mahesh; Rivers, Claudia; Park, Seo Young; Hartman, Douglas; Saul, Melissa; Regueiro, Miguel; Yadav, Dhiraj; Binion, David G

    2013-01-01

    Eosinophils are implicated in the pathogenesis of inflammatory bowel disease (IBD). A subset of IBD patients develops blood eosinophilia, and the clinical profile of these patients is undefined. We sought to characterize IBD patients with and without eosinophilia. We studied a prospective registry of 1,176 IBD patients followed in a tertiary referral center. Patients who developed eosinophilia at any time were identified by electronic medical record query. We performed a chart review case-control study comparing patients with recurrent eosinophilia versus randomly selected disease-matched patients with no history of eosinophilia. Histological analysis was performed on selected cases and controls. Eosinophilia at any time was more prevalent in ulcerative colitis (UC) patients than Crohn's disease patients (22.2 versus 12.7%), as was recurrent eosinophilia (3.4 versus 0.7%). UC patients with recurrent eosinophilia were predominantly male compared with the control UC population (81.3 versus 46.9%) and had higher rates of colectomy for either medically refractory disease or dysplasia/cancer than control UC patients (56.3 versus 15.6%). Primary sclerosing cholangitis (PSC) occurred in 37.5% of UC patients with recurrent eosinophilia compared with only 3.1% in the UC controls. Histological analysis of random diagnostic samples from UC patients with recurrent eosinophilia demonstrated a normal eosinophil pattern as seen in the control UC population. Eosinophilia-associated UC is a subgroup of IBD associated with severe colitis and PSC. Further studies are warranted to characterize molecular mechanisms underlying eosinophilia-associated UC and to determine optimal approaches for therapy.

  16. Primary sclerosing cholangitis in Turkish patients:characteristic features and prognosis

    Institute of Scientific and Technical Information of China (English)

    Hilmi Ataseven; Erkan Parlak; İlhami Yüksel; Ömer Başar; İbrahim Ertuğrul; NurgülŞaşmaz; BurhanŞahin

    2009-01-01

    BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by destruction and ifbrosis of the bile ducts. This study aimed to demonstrate the hepatic and extrahepatic characteristic ifndings and prognostic outcomes of Turkish patients with PSC. METHODS: The medical records of 35 consecutive patients with PSC from January 1988 to June 2007 were recorded prospectively. From the time of diagnosis, clinical features and laboratory data were collected. RESULTS: The mean age of the 35 patients was 41.69 years (range 15-80 years) at the time of diagnosis; 14 (40%) were female, and 21 (60%) were male. The mean duration of follow-up was 58.86 months (1-180 months). Twenty (57.1%) of the patients with PSC were asymptomatic and 22 (62.9%) had inlfammatory bowel disease. At the time of diagnosis, 20 (57.1%) of the patients had both intra- and extra-hepatic PSC. Twenty-one (60%) of the patients, who had undergone ERCP for stent placement, had dominant bile duct stenosis. Cholangiocarcinoma was found in 2 (5.7%) of the patients and cirrhosis was detected in 7 (20%); 5 (14.3%) underwent liver transplantation. The median follow-up time after liver transplantation was 23 months and all are still alive. Six (17.1%) patients died. CONCLUSIONS: PSC has a clinical course varied from advanced liver disease requiring liver transplantation within a short time to being asymptomatic for decades. The prognosis of Turkish patients with PSC is also disappointing as described in other studies.

  17. Primary Biliary Cholangitis Alters Functional Connections of the Brain's Deep Gray Matter.

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    Mosher, Victoria A L; Swain, Mark G; Pang, Jack X Q; Kaplan, Gilaad G; Sharkey, Keith A; MacQueen, Glenda M; Goodyear, Bradley G

    2017-07-27

    Fatigue, itch, depressed mood, and cognitive impairment significantly impact the quality of life of many patients with primary biliary cholangitis (PBC). Previous neuroimaging studies of non-hepatic diseases suggest that these symptoms are often associated with dysfunction of deep gray matter brain regions. We used resting-state functional magnetic resonance imaging (rsfMRI) to determine whether PBC patients exhibit altered functional connections of deep gray matter. Twenty female non-cirrhotic PBC patients and 21 age/gender-matched controls underwent rsfMRI. Resting-state functional connectivity (rsFC) of deep gray matter brain structures (putamen, thalamus, amygdala, hippocampus) was compared between groups. Fatigue, itch, mood, cognitive performance, and clinical response to ursodeoxycholic acid (UDCA) were assessed, and their association with rsFC was determined. Relative to controls, PBC patients exhibited significantly increased rsFC between the putamen, thalamus, amygdala, and hippocampus, as well as with frontal and parietal regions. Reduced rsFC of the putamen and hippocampus with motor and sensory regions of the brain were also observed. Fatigue, itch, complete response to UDCA, and verbal working memory performance were also associated with altered rsFC of deep gray matter. These rsFC changes were independent of biochemical disease severity. PBC patients have objective evidence of altered rsFC of the brain's deep gray matter that is in part linked to fatigue severity, itch, response to UDCA therapy, and cognitive performance. These results may guide future approaches to define how PBC leads to altered brain connectivity and provide insight into novel targets for treating PBC-associated brain dysfunction and behavioral symptoms.

  18. The fingerprint of antimitochondrial antibodies and the etiology of primary biliary cholangitis.

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    Shuai, Zongwen; Wang, Jinjun; Badamagunta, Madhu; Choi, Jinjung; Yang, Guoxiang; Zhang, Weici; Kenny, Thomas P; Guggenheim, Kathryn; Kurth, Mark J; Ansari, Aftab A; Voss, John; Coppel, Ross L; Invernizzi, Pietro; Leung, Patrick S C; Gershwin, M Eric

    2017-01-18

    The identification of environmental factors that lead to loss of tolerance has been coined the holy grail of autoimmunity. Our work has focused on the reactivity of antimitochondrial autoantibodies (AMA) to chemical xenobiotics and has hypothesized that a modified peptide within PDC-E2, the major mitochondrial autoantigen, will have been immunologically recognized at the time of loss of tolerance. Herein, we successfully applied intein technology to construct a PDC-E2 protein fragment containing amino acid residues 177-314 of PDC-E2 by joining a recombinant peptide spanning residues 177-252 (PDC-228) with a 62-residue synthetic peptide from 253 to 314 (PP), which encompasses PDC-E2 inner lipoyl domain (ILD). We named this intein-constructed fragment PPL. Importantly, PPL, as well as lipoic acid conjugated PPL (LA-PPL) and xenobiotic 2-octynoic acid conjugated PPL (2OA-PPL), are recognized by AMA. Of great importance, AMA has specificity for the 2OA-modified PDC-E2 ILD peptide backbone distinct from antibodies that react with native lipoylated PDC-E2 peptide. Interestingly, this unique AMA subfraction is of the immunoglobulin M isotype and more dominant in early-stage primary biliary cholangitis (PBC), suggesting that exposure to 2OA-PPL-like compounds occurs early in the generation of AMA. To understand the structural basis of this differential recognition, we analyzed PPL, LA-PPL, and 2OA-PPL using electron paramagnetic resonance spectroscopy, with confirmations by enzyme-linked immunosorbent assay, immunoblotting, and affinity antibody analysis. We demonstrate that the conformation of PDC-E2 ILD is altered when conjugated with 2OA, compared to conjugation with lipoic acid.

  19. Disease activity and cancer risk in inflammatory bowel disease associated with primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Harry Sokol; Jacques Cosnes; Olivier Chazouilleres; Laurent Beaugerie; Emmanuel Tiret; Raoul Poupon; Philippe Seksik

    2008-01-01

    AIM: To investigate the phenotype of inflammatory bowel disease associated with primary sclerosing cholangitis (PSC-IBD).METHODS: Data from 75 PSC-]BD patients evaluated in our tertiary center between 1963 and 2006 were collected and compared to 150 IBD patients without PSC, matched for sex, birth date, IBD diagnosis date and initial disease location regarding ileal, different colonic segments, and rectum, respectively.RESULTS: While PSC-IBD patients received more 5-aminosalicylates (8.7 years/patient vs 2.9 years/patient, P<0.001), they required less immunosuppressors (24% vs 46% at 10 years; P<0.001) and less intestinal resection (10% vs 44% at 10 years, P<0.001). The 25-year cumulative rate of colectomy was 25.1% in PSC-IBD and 37.3% in controls (P=0.004). The 25-year cumulative rate of colorectal cancer was 23.4% in PSC-IBD vs 0% in controls (P=0.002). PSC was the only independent risk factor for the development of colorectal cancer (OR=10.8; 95%CI, 3.7-31.3). Overall survival rate without liver transplantation was reduced in PSC-IBD patients (67% vs 91% in controls at 25 years, P=0.001).CONCLUSION: This study confirms that patients with PSC-IBD have a particular disease phenotype independent of the initial disease location. Although their disease is less active and they use more 5-aminosalicylates, they present a higher risk of colorectal cancer.

  20. Recurrent blood eosinophilia in ulcerative colitis is associated with severe disease and primary sclerosing cholangitis

    Science.gov (United States)

    Barrie, Arthur; Mourabet, Marwa El; Weyant, Katherine; Clarke, Kofi; Gajendran, Mahesh; Rivers, Claudia; Park, Seo Young; Hartman, Douglas; Saul, Melissa; Regueiro, Miguel; Yadav, Dhiraj; Binion, David G.

    2012-01-01

    Background and Aims Eosinophils are implicated in the pathogenesis of inflammatory bowel disease (IBD). A subset of IBD patients develops blood eosinophilia and the clinical profile of these patients is undefined. We sought to characterize IBD patients with and without eosinophilia. Methods We studied a prospective registry of 1176 IBD patients followed in a tertiary referral center. Patients who developed eosinophilia at any time were identified by electronic medical record query. We performed a chart review case-control study comparing patients with recurrent eosinophilia versus randomly selected disease matched patients with no history of eosinophilia. Histological analysis was performed on selected cases and controls. Results Eosinophilia at any time was more prevalent in ulcerative colitis (UC) patients than Crohn’s disease patients (22.2% vs. 12.7%), as was recurrent eosinophilia (3.4% vs. 0.7%). UC patients with recurrent eosinophilia were predominantly male compared to the control UC population (81.3% vs. 46.9%) and had higher rates of colectomy for either medically refractory disease or dysplasia/cancer than control UC patients (56.3% vs. 15.6%). Primary sclerosing cholangitis (PSC) occurred in 37.5% of UC patients with recurrent eosinophilia compared to only 3.1% in the UC controls. Histological analysis of random diagnostic samples from UC patients with recurrent eosinophilia demonstrated a normal eosinophil pattern as seen in the control UC population. Conclusions Eosinophilia-associated UC is a subgroup of IBD associated with severe colitis and PSC. Further studies are warranted to characterize molecular mechanisms underlying eosinophilia-associated UC and to determine optimal approaches for therapy. PMID:22855293

  1. The prevalence, incidence and natural history of primary sclerosing cholangitis in an ethnically diverse population

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    Li Chin-Shang

    2011-07-01

    Full Text Available Abstract Background Primary sclerosing cholangitis (PSC is a rare chronic cholestatic liver disease often associated with inflammatory bowel diseases (IBD. Current epidemiological data are limited to studies of predominantly Caucasian populations. Our aim was to define the epidemiology of PSC in a large, ethnically diverse US population. Methods The Northern California Kaiser Permanente (KP database includes records from over 3 million people and was searched for cases of PSC between January 2000 and October 2006. All identified charts were reviewed for diagnosis confirmation, IBD co-morbidity, and major natural history endpoints. Results We identified 169 (101 males cases fulfilling PSC diagnostic criteria with a mean age at diagnosis of 44 years (range 11-81. The age-adjusted point prevalence was 4.15 per 100,000 on December 31, 2005. The age-adjusted incidence per 100,000 person-years was not significantly greater in men 0.45 (95% CI 0.33 - 0.61 than women 0.37 (95% CI 0.26 - 0.51. IBD was present in 109/169 (64.5% cases and was significantly more frequent in men than women with PSC (73.3% and 51.5%, respectively, p = 0.005. The cumulative average yearly mortality rate was 1.9%. Age and serum sodium, creatinine and bilirubin at diagnosis and albumin at last entry were identified as significant factors associated with death, liver transplant or cholangiocarcinoma. Conclusions The incidence and prevalence of PSC observed in a representative Northern California population are lower compared to previous studies in Caucasian populations and this might reflect differences in the incidence of PSC among various ethnic groups.

  2. Pancreatic changes in patients with primary sclerosing cholangitis: MR cholangiopancreatography and MRI findings

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    Ozkavukcu, Esra [Ankara University School of Medicine, Department of Radiology, Ankara Universitesi Tip Fakueltesi, Cebeci Hastanesi, Cebeci, Ankara 06100 (Turkey)], E-mail: eozkavukcu@gmail.com; Erden, Ayse; Erden, Ilhan [Ankara University School of Medicine, Department of Radiology, Ankara Universitesi Tip Fakueltesi, Cebeci Hastanesi, Cebeci, Ankara 06100 (Turkey)

    2009-04-15

    Purpose: To evaluate the possible pancreatic changes and their frequencies in patients with primary sclerosing cholangitis (PSC) on MR cholangiopancreatography (MRCP), and conventional abdominal MRI. Materials and Methods: Patient group consisted of 29 PSC (13 male, 16 female) cases, whereas cohort 1 consisted of 12 female patients with primary biliary cirrhosis, and cohort 2 consisted of 17 patients (6 male, 11 female) with non-immune chronic liver disease. Two radiologists retrospectively evaluated the MR examinations paying special attention to the pancreatic size (atrophy or enlargement), T1- and T2-signal intensity of the pancreas, focal pancreatic lesion, capsule-like rim, peripancreatic edema or fluid, fascial thickening, and pancreatic ducts (dilatation or narrowing). The results are expressed as percentages. Three groups were compared using Pearson chi-square test for each feature. However, only p-value for 'dilatation of the pancreatic duct' was determined, whereas p-value could not be calculated because of the insufficient number of subjects/sequences for the other features. Results: Twelve PSC patients (41.3%) had pancreatic abnormalities. The most common pancreatic changes in PSC patients were decreased T1-signal intensity (44%) and dilatation of the pancreatic duct (13.8%), respectively. Increased T2-signal intensity was also shown in 2 PSC patients (6.9%). Conclusion: Even PSC patients without any sign of pancreatitis, can show MR changes in the pancreatic parenchyma or the pancreatic duct. The etiologies of these changes, and whether they are unique to PSC, are still controversial. Histopathological studies bringing light to these pancreatic changes are needed.

  3. Circulating follicular helper T cells presented distinctively different responses toward bacterial antigens in primary biliary cholangitis.

    Science.gov (United States)

    Zhou, Zun-Qiang; Tong, Da-Nian; Guan, Jiao; Li, Mei-Fang; Feng, Qi-Ming; Zhou, Min-Jie; Zhang, Zheng-Yun

    2017-10-01

    Primary biliary cholangitis (PBC) is a chronic and progressive cholestatic liver disease with unknown causes. The initiation of PBC is associated with bacterial infections and abnormal immune correlates, such as the presence of self-reactive anti-mitochondrial antibodies and shifted balance of T cell subsets. In particular, the CD4(+)CXCR5(+) follicular helper T (Tfh) cells are highly activated in PBC patients and are significantly associated with PBC severity, but the underlying reasons are unknown. In this study, we found that the circulating CD4(+)CXCR5(+) T cells were enriched with the interferon (IFN)-γ-secreting Th1-subtype and the interleukin (IL)-17-secreting Th17-subtype, but not the IL-4-secreting Th2 subtype. We further demonstrated that a host of microbial motifs, including Pam3CSK4, poly(I:C), LPS, imiquimod, and CpG, could significantly stimulate IFN-γ, IL-17, and/or IL-21 from circulating CD4(+)CXCR5(+) T cells in PBC patients, especially in the presence of monocytes and B cells. Whole bacterial cells of Escherichia coli, Novosphingobium aromaticivorans, and Mycobacterium gordonae, could also potently stimulate IFN-γ, IL-17, and/or IL-21 production from circulating CD4(+)CXCR5(+) T cells. But interestingly, while the whole cell could potently stimulate circulating CD4(+)CXCR5(+) T cells from both healthy controls and PBC patients, the cell protein lysate could only potently stimulate circulating CD4(+)CXCR5(+) T cells from PBC patients, but not those from healthy controls, suggesting that circulating CD4(+)CXCR5(+) T cells in PBC patients had distinctive antigen-specificity from those in healthy individuals. Together, these data demonstrated that bacterial antigen stimulation is a potential source of aberrant Tfh cell activation in PBC patients. Copyright © 2017. Published by Elsevier B.V.

  4. Autoimmune pancreatocholangitis, non-autoimmune pancreatitis and primary sclerosing cholangitis: a comparative morphological and immunological analysis.

    Directory of Open Access Journals (Sweden)

    Irene Esposito

    Full Text Available BACKGROUND: Autoimmune pancreatocholangitis (AIPC is an emerging, not completely characterized disease. Aim of this study was the comprehensive evaluation of a series of AIPC patients, who were diagnosed and treated in a European institution between January 2003 and July 2006. METHODOLOGY/PRINCIPAL FINDINGS: Thirty-three patients with histologically confirmed AIPC were analyzed and compared to 20 patients with non-autoimmune chronic pancreatitis (CP and 14 patients with primary sclerosing cholangitis (PSC. Clinical features and conventional histopathology were taken into account. Immunohistochemistry and real-time quantitative PCR were used for the characterization of the inflammatory infiltrate and the stromal reaction. AIPC was localized in the pancreatic head in 94% of the patients. Intra- and/or extrapancreatic biliary tract involvement was present in 64% of the cases. The number of infiltrating T-lymphocytes, macrophages and total plasma cells was significantly higher in AIPC than in CP (3-, 4- and 8-fold increase, respectively. The absolute number of IgG4-positive plasma cells was higher in AIPC than in CP and PSC (7-fold and 35-fold increase, respectively, but significance was only reached in comparison with PSC. CXCR5- and CXCL13-positive cells were almost exclusively detected in AIPC. CONCLUSIONS/SIGNIFICANCE: AIPC is mainly a disease of the pancreatic head with possible extension into the periphery of the gland and/or into the biliary tract/gallbladder. The morphology of AIPC, as well as the immune- and stromal reaction is characteristic and comparable between cases with and without biliary tract involvement. Immunological markers (IgG4, CXCR5, CXCL13 can be of diagnostic relevance in specific settings.

  5. Hospitalized Patients and Fungal Infections

    Science.gov (United States)

    ... These types of infections are called healthcare-associated infections (HAIs). Hospital staff and healthcare providers do everything they can ... IV tube) can increase your risk for fungal infection. During your hospital stay you may need a central venous catheter, ...

  6. Fungal Entomopathogens in the Rhizosphere

    Science.gov (United States)

    Entomopathogenic fungi are found in a wide variety of fungal groups. The order Hypocreales contains the largest number of entomogenous fungi, including two of the most widely studied, Beauveria bassiana (Balsamo) Vuillemin (Hypocreales: Clavicipitaceae) and Metarhizium anisopliae (Metchnikoff) Sorok...

  7. Fungal genomics beyond Saccharomyces cerevisiae?

    DEFF Research Database (Denmark)

    Hofmann, Gerald; Mcintyre, Mhairi; Nielsen, Jens

    2003-01-01

    Fungi are used extensively in both fundamental research and industrial applications. Saccharomyces cerevisiae has been the model organism for fungal research for many years, particularly in functional genomics. However, considering the diversity within the fungal kingdom, it is obvious that the a......Fungi are used extensively in both fundamental research and industrial applications. Saccharomyces cerevisiae has been the model organism for fungal research for many years, particularly in functional genomics. However, considering the diversity within the fungal kingdom, it is obvious...... that the application of the existing methods of genome, transcriptome, proteome and metabolome analysis to other fungi has enormous potential, especially for the production of food and food ingredients. The developments in the past year demonstrate that we have only just started to exploit this potential....

  8. Treatment of Allergic Fungal Sinusitis:a Report of 31 Cases

    Institute of Scientific and Technical Information of China (English)

    LIHua-bin; LINZi-ping; XUWan-yun; XUJin

    2004-01-01

    Objective: To evaluate the effects of different surgical procedures on allergic fungal sinusitis. Methods: Thirty-one patients with allergic fungal sinusitis under endoscopic sinus surgery(24 cases) and Caldwell-Luc operation( 7 cases) after medical treatment were investigated. Results: No complication was observed during one year's follow-up. No patient who conducted endoscopic sinus surgery reacurred and 3 patients who received traditional surgery required a second operation. Conclusion: This confirmed endoscopic sinus surgery provided a minl-traumatic and effective treatment. Combined treatment of surgical and medical ways was useful to the development of allergic fungal sinusitis.

  9. Fungal microbiota dysbiosis in IBD

    Science.gov (United States)

    Sokol, Harry; Leducq, Valentin; Aschard, Hugues; Pham, Hang-Phuong; Jegou, Sarah; Landman, Cecilia; Cohen, David; Liguori, Giuseppina; Bourrier, Anne; Nion-Larmurier, Isabelle; Cosnes, Jacques; Seksik, Philippe; Langella, Philippe; Skurnik, David; Richard, Mathias L; Beaugerie, Laurent

    2017-01-01

    Objective The bacterial intestinal microbiota plays major roles in human physiology and IBDs. Although some data suggest a role of the fungal microbiota in IBD pathogenesis, the available data are scarce. The aim of our study was to characterise the faecal fungal microbiota in patients with IBD. Design Bacterial and fungal composition of the faecal microbiota of 235 patients with IBD and 38 healthy subjects (HS) was determined using 16S and ITS2 sequencing, respectively. The obtained sequences were analysed using the Qiime pipeline to assess composition and diversity. Bacterial and fungal taxa associated with clinical parameters were identified using multivariate association with linear models. Correlation between bacterial and fungal microbiota was investigated using Spearman's test and distance correlation. Results We observed that fungal microbiota is skewed in IBD, with an increased Basidiomycota/Ascomycota ratio, a decreased proportion of Saccharomyces cerevisiae and an increased proportion of Candida albicans compared with HS. We also identified disease-specific alterations in diversity, indicating that a Crohn's disease-specific gut environment may favour fungi at the expense of bacteria. The concomitant analysis of bacterial and fungal microbiota showed a dense and homogenous correlation network in HS but a dramatically unbalanced network in IBD, suggesting the existence of disease-specific inter-kingdom alterations. Conclusions Besides bacterial dysbiosis, our study identifies a distinct fungal microbiota dysbiosis in IBD characterised by alterations in biodiversity and composition. Moreover, we unravel here disease-specific inter-kingdom network alterations in IBD, suggesting that, beyond bacteria, fungi might also play a role in IBD pathogenesis. PMID:26843508

  10. Spontaneous fungal peritonitis: Epidemiology, current evidence and future prospective.

    Science.gov (United States)

    Fiore, Marco; Leone, Sebastiano

    2016-09-14

    Spontaneous bacterial peritonitis is a complication of ascitic patients with end-stage liver disease (ESLD); spontaneous fungal peritonitis (SFP) is a complication of ESLD less known and described. ESLD is associated to immunodepression and the resulting increased susceptibility to infections. Recent perspectives of the management of the critically ill patient with ESLD do not specify the rate of isolation of fungi in critically ill patients, not even the antifungals used for the prophylaxis, neither optimal treatment. We reviewed, in order to focus the epidemiology, characteristics, and, considering the high mortality rate of SFP, the use of optimal empirical antifungal therapy the current literature.

  11. Fungal allergy in asthma-state of the art and research needs.

    Science.gov (United States)

    Denning, David W; Pashley, Catherine; Hartl, Domink; Wardlaw, Andrew; Godet, Cendrine; Del Giacco, Stefano; Delhaes, Laurence; Sergejeva, Svetlana

    2014-01-01

    Sensitization to fungi and long term or uncontrolled fungal infection are associated with poor control of asthma, the likelihood of more severe disease and complications such as bronchiectasis and chronic pulmonary aspergillosis. Modelling suggests that >6.5 million people have severe asthma with fungal sensitizations (SAFS), up to 50% of adult asthmatics attending secondary care have fungal sensitization, and an estimated 4.8 million adults have allergic bronchopulmonary aspergillosis (ABPA). There is much uncertainty about which fungi and fungal allergens are relevant to asthma, the natural history of sensitisation to fungi, if there is an exposure response relationship for fungal allergy, and the pathogenesis and frequency of exacerbations and complications. Genetic associations have been described but only weakly linked to phenotypes. The evidence base for most management strategies in ABPA, SAFS and related conditions is weak. Yet straightforward clinical practice guidelines for management are required. The role of environmental monitoring and optimal means of controlling disease to prevent disability and complications are not yet clear. In this paper we set out the key evidence supporting the role of fungal exposure, sensitisation and infection in asthmatics, what is understood about pathogenesis and natural history and identify the numerous areas for research studies.

  12. The Fungal Biome of the Oral Cavity.

    Science.gov (United States)

    Chandra, Jyotsna; Retuerto, Mauricio; Mukherjee, Pranab K; Ghannoum, Mahmoud

    2016-01-01

    Organisms residing in the oral cavity (oral microbiota) contribute to health and disease, and influence diseases like gingivitis, periodontitis, and oral candidiasis (the most common oral complication of HIV-infection). These organisms are also associated with cancer and other systemic diseases including upper respiratory infections. There is limited knowledge regarding how oral microbes interact together and influence the host immune system. Characterizing the oral microbial community (oral microbiota) in health and disease represents a critical step in gaining insight into various members of this community. While most of the studies characterizing oral microbiota have focused on bacterial community, there are few encouraging studies characterizing the oral mycobiome (the fungal component of the oral microbiota). Our group recently characterized the oral mycobiome in health and disease focusing on HIV. In this chapter we will describe the methods used by our group for characterization of the oral mycobiome.

  13. Recent progress in vaccines against fungal diseases.

    Science.gov (United States)

    Cassone, Antonio; Casadevall, Arturo

    2012-08-01

    Diseases caused by fungi are increasingly impacting the health of the human population and now account for a large fraction of infectious disease complications in individuals with impaired immunity or breached tissue defenses. Antifungal therapy is often of limited effectiveness in these patients, resulting into treatment failures, chronic infections and unacceptable rates of mortality, morbidity and their associated costs. Consequently there is a real medical need for new treatments and preventive measures to combat fungal diseases and, toward this goal, safe and efficacious vaccines would constitute major progress. After decades of complacency and neglect of this critically important field of research, remarkable progress has been made in recent years. A number of highly immunogenic and protective vaccine formulations in preclinical setting have been developed, and at least two have undergone Phase 1 clinical trials as preventive and/or therapeutic tools against candidiasis.

  14. Overlap of primary biliary cirrhosis and primary sclerosing cholangitis – a rare coincidence or a new syndrome

    Directory of Open Access Journals (Sweden)

    Arun Jeevagan

    2010-05-01

    Full Text Available Arun JeevaganGeneral Medicine, Ipswich NHS Hospital, Ipswich, UKBackground: Overlap syndrome has been introduced to the field of hepatology to describe the coexistence of two or more autoimmune hepatic conditions in the same individual. This is a rare clinical case of a patient diagnosed with primary biliary cirrhosis (PBC who later developed primary sclerosing cholangitis (PSC. This is a unique case as no other cases with a similar pattern have been reported. Overlap syndrome does not include the coexistence of PBC and PSC as a distinctive syndrome so far.Case report: A middle-aged woman suffering from PBC for 17 years got admitted with clinical and biochemical features of cholestatic syndrome. A provisional diagnosis of worsening PBC was proved wrong by magnetic resonance cholangiopancreatography, which revealed typical benign stricture and dilatation of common bile duct with typical beading appearance suggestive of PSC. The patient was stented and treated with an increased dose of ursodeoxycholic acid (UDCA which improved the symptoms and the biochemical picture.Conclusion: This is a clear overlap of PBC–PSC. It is very difficult to say whether it is a rare coincidence or a new overlap syndrome, but there are no clear guidelines for management of these patients. Currently, the treatment involves endoscopic duct dilatation, UDCA, and regular follow-ups to rule out hepato-biliary tumor occurrence.Keywords: primary biliary cirrhosis, primary sclerosing cholangitis, hepatic overlap syndrome

  15. Platynosomum fastosum-induced chronic intrahepatic cholangitis and Spirometra spp. infections in feral cats from Grand Cayman.

    Science.gov (United States)

    Headley, S A; Gillen, M A; Sanches, A W D; Satti, M Z

    2012-06-01

    The occurrence of platynosomiasis and intestinal sparganosis is described in feral cats from Grand Cayman, Cayman Islands. Spirometra spp. was observed within the intestine of 18.18% (10/55) of cats; 1.18% (1/55) of cats demonstrated gross and histological manifestation of parasitism by Platynosomum fastosum, but 14.5% (8/55) of cats had the characteristic pathological manifestations of P. fastosum-induced intrahepatic cholangitis without the concomitant presence of the intraductal trematode. Combined parasitism (Spirometra spp. and P. fastosum) was observed in 9.09% (5/55) of feral cats. Significant pathological findings were only associated with the hepatic fluke, P. fastosum, and were grossly characterized by moderate hepatomegaly with enlarged and dilated bile ducts. Examples of cestodes with morphological features characteristic of Spirometra spp. were observed within the small intestine without any associated pathological lesion. The histopathological evaluation of liver fragments revealed chronic intrahepatic cholangitis with and without the associated intraductal trematode, and was characterized by marked periductal fibrosis, adenomatous proliferation of bile duct epithelium, dilation of intrahepatic bile ducts and portal accumulations of inflammatory cells. The occurrence of the cestode in feral cats coupled with factors that are unique to Grand Cayman makes this island the ideal location for sporadic cases of human sparganosis.

  16. Outcome and Genetic Factors in IgG4-Associated Autoimmune Pancreatitis and Cholangitis: A Single Center Experience

    Directory of Open Access Journals (Sweden)

    Matthias Buechter

    2017-01-01

    Full Text Available Introduction. Most investigations on autoimmune pancreatitis (AIP were published on Asian cohorts while those on Caucasians are limited. However, there might be differences related to the origin. Patients and Methods. We analyzed 36 patients and compared type 1 (AIP1 with type 2 (AIP2. Results. The majority of patients suffered from AIP1 (55.6%. AIP1 patients were significantly older than AIP2 patients (54.4 versus 40.8 years. Moreover, 85.0% of AIP1 patients had concurrent autoimmune cholangitis (AIC while 18.8% of AIP2 patients suffered from overlap to ulcerative colitis (UC. However, AIP1 patients revealed a cholestatic course and had significantly higher immunoglobulin G4 levels (IgG4. When compared to allele frequencies in healthy controls, in patients with AIP1 HLA-B8 reached statistical significance. Response to steroids was excellent in both groups, but we noticed high rates of relapse especially in AIP1 patients. Finally, 3 patients with AIP1 were diagnosed with cholangiocellular carcinoma (CCC. Conclusion. In contrast to Asian studies, we found an almost equal distribution of AIP1 and AIP2 patients in our German cohort. AIP2 patients were younger and mostly of female gender whereas AIP1 patients revealed higher IgG4 levels and involvement of the biliary tract in sense of IgG4-associated cholangitis.

  17. A patient presenting with cholangitis due to Stenotrophomonas maltophilia and Pseudomonas aeruginosa successfully treated with intrabiliary colistine

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    Pablo N. Pérez

    2014-05-01

    Full Text Available Anatomical barriers for antibiotic penetration can pose a particular challenge in the clinical setting. Stenotrophomonas maltophilia (SM and Pseudomonas aeruginosa (PA are two pathogens capable of developing multiple drug-resistance (MDR mechanisms. We report the case of a 56-year-old female patient with a permanent percutaneous transhepatic biliary drainage (PTBD, who was admitted to our hospital with a cholangitis due to a MDR Escherichia coli strain. Upon admission, culture-guided antimicrobial therapy was conducted and the biliary catheter was replaced, with poor clinical response. Subsequently, SM and PA were detected. Treatment with fosfomycin and colistine was initiated, again without adequate response. Systemic colistine and tigecycline along with an intrabiliary infusion of colistine for 5 days was then used, followed by parenteral fosfomycin and tigecycline for 7 days. The patient was then successfully discharged. This is the first case report we are aware of on the use of intrabiliary colistine. It describes a new approach to treating cholangitis by MDR bacteria in patients with a PTBD.

  18. Fungal-Fungal Interactions in Leaf-Cutting Ant Agriculture

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    Sunshine A. Van Bael

    2011-01-01

    Full Text Available Many organisms participate in symbiotic relationships with other organisms, yet studies of symbioses typically have focused on the reciprocal costs and benefits within a particular host-symbiont pair. Recent studies indicate that many ecological interactions involve alliances of symbionts acting together as mutualistic consortia against other consortia. Such interacting consortia are likely to be widespread in nature, even if the interactions often occur in a cryptic fashion. Little theory and empirical data exist concerning how these complex interactions shape ecological outcomes in nature. Here, we review recent work on fungal-fungal interactions between two consortia: (i leaf-cutting ants and their symbiotic fungi (the latter grown as a food crop by the former and (ii tropical plants and their foliar endophytes (the cryptic symbiotic fungi within leaves of the former. Plant characteristics (e.g., secondary compounds or leaf physical properties of leaves are involved in leaf-cutting ant preferences, and a synthesis of published information suggests that these plant traits could be modified by fungal presence. We discuss potential mechanisms for how fungal-fungal interactions proceed in the leaf-cutting ant agriculture and suggest themes for future research.

  19. Serious fungal infections in Ecuador.

    Science.gov (United States)

    Zurita, J; Denning, D W; Paz-Y-Miño, A; Solís, M B; Arias, L M

    2017-06-01

    There is a dearth of data from Ecuador on the burden of life-threatening fungal disease entities; therefore, we estimated the burden of serious fungal infections in Ecuador based on the populations at risk and available epidemiological databases and publications. A full literature search was done to identify all epidemiology papers reporting fungal infection rates. WHO, ONU-AIDS, Index Mundi, Global Asthma Report, Globocan, and national data [Instituto Nacional de Estadística y Censos (INEC), Ministerio de Salud Pública (MSP), Sociedad de Lucha Contra el Cáncer (SOLCA), Instituto Nacional de Donación y Trasplante de Órganos, Tejidos y Células (INDOT)] were reviewed. When no data existed, risk populations were used to estimate frequencies of fungal infections, using previously described methodology by LIFE. Ecuador has a variety of climates from the cold of the Andes through temperate to humid hot weather at the coast and in the Amazon basin. Ecuador has a population of 15,223,680 people and an average life expectancy of 76 years. The median estimate of the human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) population at risk for fungal disease (<200 CD4 cell counts) is ∼10,000, with a rate of 11.1% (1100) of histoplasma, 7% (700) of cryptococcal meningitis, and 11% (1070) of Pneumocystis pneumonia. The burden of candidemia is 1037. Recurrent Candida vaginitis (≥4 episodes per year) affects 307,593 women aged 15-50 years. Chronic pulmonary aspergillosis probably affects ∼476 patients following tuberculosis (TB). Invasive aspergillosis is estimated to affect 748 patients (∼5.5/100,000). In addition, allergic bronchopulmonary aspergillosis (ABPA) in asthma and severe asthma with fungal sensitization (SAFS) were estimated to affect 26,642 and 45,013 people, respectively. Our estimates indicate that 433,856 (3%) of the population in Ecuador is affected by serious fungal infection.

  20. Risk factors for complications in patients with ulcerative colitis.

    Science.gov (United States)

    Manser, Christine N; Borovicka, Jan; Seibold, Frank; Vavricka, Stephan R; Lakatos, Peter L; Fried, Michael; Rogler, Gerhard

    2016-04-01

    Patients with ulcerative colitis may develop extraintestinal manifestations like erythema nodosum or primary sclerosing cholangitis or extraintestinal complications like anaemia, malabsorption or they may have to undergo surgery. The aim of this study was to investigate potential risk factors for complications like anaemia, malabsorption or surgery in ulcerative colitis. Data on 179 patients with ulcerative colitis were retrieved from our cross-sectional and prospective Swiss Inflammatory Bowel Disease Cohort Study data base for a median observational time of 4.2 years. Data were compared between patients with (n = 140) or without (n = 39) complications. Gender, age at diagnosis, smoking status, disease extent, delay of diagnosis or therapy, mesalamine (5-ASA) systemic and topical therapy, as well as other medication were analysed as potential impact factors. In the multivariate regression analysis a delay of 5-ASA treatment by at least two months (odds ratio (OR) 6.21 (95% confidence interval (CI) 2.13-18.14), p = 0.001) as well as a delay with other medication with thiopurines (OR 6.48 (95% CI 2.01-20.91), p = 0.002) were associated with a higher risk for complications. This significant impact of a delay of 5-ASA therapy was demonstrated for extraintestinal manifestations (EIMs) as well as extraintestinal complications (EICs). Extensive disease as well as therapy with methotrexate showed a significantly increased risk for surgery (extensive disease: OR 2.62 (1.02-6.73), p = 0.05, methotrexate: OR 5.36 (1.64-17.58), p = 0.006). A delay of 5-ASA therapy of more than two months in the early stage of ulcerative colitis (UC) constitutes a risk for complications during disease course. Extensive disease is associated with a higher risk for surgery.

  1. Genome-Wide Association Analysis in Primary Sclerosing Cholangitis and Ulcerative Colitis Identifies Risk Loci at GPR35 and TCF4

    NARCIS (Netherlands)

    Ellinghaus, David; Folseraas, Trine; Holm, Kristian; Ellinghaus, Eva; Melum, Espen; Balschun, Tobias; Laerdahl, Jon K.; Shiryaev, Alexey; Gotthardt, Daniel N.; Weismueller, Tobias J.; Schramm, Christoph; Wittig, Michael; Bergquist, Annika; Bjornsson, Einar; Marschall, Hanns-Ulrich; Vatn, Morten; Teufel, Andreas; Rust, Christian; Gieger, Christian; Wichmann, H-Erich; Runz, Heiko; Sterneck, Martina; Rupp, Christian; Braun, Felix; Weersma, Rinse K.; Wijmenga, Cisca; Ponsioen, Cyriel Y.; Mathew, Christopher G.; Rutgeerts, Paul; Vermeire, Severine; Schrumpf, Erik; Hov, Johannes R.; Manns, Michael P.; Boberg, Kirsten M.; Schreiber, Stefan; Franke, Andre; Karlsen, Tom H.

    2013-01-01

    Approximately 60%-80% of patients with primary sclerosing cholangitis (PSC) have concurrent ulcerative colitis (UC). Previous genome-wide association studies (GWAS) in PSC have detected a number of susceptibility loci that also show associations in UC and other immune-mediated diseases. We aimed to

  2. Dual Roles of IFN-γ and IL-4 in the Natural History of Murine Autoimmune Cholangitis: IL-30 and Implications for Precision Medicine.

    Science.gov (United States)

    Syu, Bi-Jhen; Loh, Chia-En; Hsueh, Yu-Hsin; Gershwin, M Eric; Chuang, Ya-Hui

    2016-10-10

    Primary biliary cirrhosis (PBC) is a progressive autoimmune liver disease with a long natural history. The pathogenesis of PBC is thought to be orchestrated by Th1 and/or Th17. In this study, we investigated the role of CD4(+) helper T subsets and their cytokines on PBC using our previous established murine model of 2-OA-OVA immunization. We prepared adeno-associated virus (AAV)-IFN-γ and AAV-IL-4 and studied their individual influences on the natural history of autoimmune cholangitis in this model. Administration of IFN-γ significantly promotes recruitment and lymphocyte activation in the earliest phases of autoimmune cholangitis but subsequently leads to downregulation of chronic inflammation through induction of the immunosuppressive molecule IL-30. In contrast, the administration of IL-4 does not alter the initiation of autoimmune cholangitis, but does contribute to the exacerbation of chronic liver inflammation and fibrosis. Thus Th1 cells and IFN-γ are the dominant contributors in the initiation phase of this model but clearly may have different effects as the disease progress. In conclusion, better understanding of the mechanisms by which helper T cells function in the natural history of cholangitis is essential and illustrates that precision medicine may be needed for patients with PBC at various stages of their disease process.

  3. Genome-wide association study of primary sclerosing cholangitis identifies new risk loci and quantifies the genetic relationship with inflammatory bowel disease

    NARCIS (Netherlands)

    Ji, Sun-Gou; Juran, Brian D.; Mucha, Soeren; Folseraas, Trine; Jostins, Luke; Melum, Espen; Kumasaka, Natsuhiko; Atkinson, Elizabeth J.; Schlicht, Erik M; Liu, Jimmy Z.; Shah, Tejas; Gutierrez-Achury, Javier; Boberg, Kirsten M.; Bergquist, Annika; Vermeire, Severine; Eksteen, Bertus; Durie, Peter R.; Farkkila, Martti; Mueller, Tobias; Schramm, Christoph; Sterneck, Martina; Weismueller, Tobias J.; Gotthardt, Daniel Nils; Ellinghaus, David; Braun, Felix; Teufel, Andreas; Laudes, Mattias; Lieb, Wolfgang; Jacobs, Gunnar; Beuers, Ulrich; Weersma, Rinse K.; Wijmenga, Cisca; Marschall, Hanns-Ulrich; Milkiewicz, Piotr; Pares, Albert; Kontula, Kimmo; Chazouilleres, Olivier; Invernizzi, Pietro; Goode, Elizabeth; Spiess, Kelly; Moore, Carmel; Sambrook, Jennifer; Ouwehand, Willem H.; Roberts, David J; Danesh, John; Floreani, Annarosa; Gulamhusein, Aliya F; Eaton, John E; Schreiber, Stefan; Coltescu, Catalina; Bowlus, Christopher L.; Luketic, Velimir A.; Odin, Joseph A; Chopra, Kapil B; Kowdley, Kris V; Chalasani, Naga; Manns, Michael P.; Srivastava, Brijesh; Mells, George; Sandford, Richard N.; Alexander, Graeme; Gaffney, Daniel J; Chapman, Roger W.; Hirschfield, Gideon M.; de Andrade, Mariza; Rushbrook, Simon M.; Franke, Andre; Karlsen, Tom H.; Lazaridis, Konstantinos N.; Anderson, Carl A.

    Primary sclerosing cholangitis (PSC) is a rare progressive disorder leading to bile duct destruction; similar to 75% of patients have comorbid inflammatory bowel disease (IBD). We undertook the largest genome-wide association study of PSC (4,796 cases and 19,955 population controls) and identified

  4. Genome-wide association study of primary sclerosing cholangitis identifies new risk loci and quantifies the genetic relationship with inflammatory bowel disease

    NARCIS (Netherlands)

    Ji, Sun-Gou; Juran, Brian D; Mucha, Sören; Folseraas, Trine; Jostins, Luke; Melum, Espen; Kumasaka, Natsuhiko; Atkinson, Elizabeth J; Schlicht, Erik M; Liu, Jimmy Z; Shah, Tejas; Gutierrez-Achury, Javier; Boberg, Kirsten M; Bergquist, Annika; Vermeire, Severine; Eksteen, Bertus; Durie, Peter R; Farkkila, Martti; Müller, Tobias; Schramm, Christoph; Sterneck, Martina; Weismüller, Tobias J; Gotthardt, Daniel N; Ellinghaus, David; Braun, Felix; Teufel, Andreas; Laudes, Mattias; Lieb, Wolfgang; Jacobs, Gunnar; Beuers, Ulrich; Weersma, Rinse K; Wijmenga, Cisca; Marschall, Hanns-Ulrich; Milkiewicz, Piotr; Pares, Albert; Kontula, Kimmo; Chazouillères, Olivier; Invernizzi, Pietro; Goode, Elizabeth; Spiess, Kelly; Moore, Carmel; Sambrook, Jennifer; Ouwehand, Willem H; Roberts, David J; Danesh, John; Floreani, Annarosa; Gulamhusein, Aliya F; Eaton, John E; Schreiber, Stefan; Coltescu, Catalina; Bowlus, Christopher L; Luketic, Velimir A; Odin, Joseph A; Chopra, Kapil B; Kowdley, Kris V; Chalasani, Naga; Manns, Michael P; Srivastava, Brijesh; Mells, George; Sandford, Richard N; Alexander, Graeme; Gaffney, Daniel J; Chapman, Roger W; Hirschfield, Gideon M; de Andrade, Mariza; Rushbrook, Simon M; Franke, Andre; Karlsen, Tom H; Lazaridis, Konstantinos N; Anderson, Carl A

    2016-01-01

    Primary sclerosing cholangitis (PSC) is a rare progressive disorder leading to bile duct destruction; ∼75% of patients have comorbid inflammatory bowel disease (IBD). We undertook the largest genome-wide association study of PSC (4,796 cases and 19,955 population controls) and identified four new ge

  5. Laparoscopic exploration of the common bile duct and removal of dead worm in a patient of cholangitis after endoscopic retrograde cholangiopancreatography failure

    Directory of Open Access Journals (Sweden)

    Chalkoo Mushtaq

    2009-01-01

    Full Text Available We describe a dead ascaris-induced extrahepatic bilary obstruction in a young female who presented with acute cholangitis. The dead ascaris was removed by laparoscopic exploration of common bile duct after endoscopic retrograde cholangiopancreatography failure. Patient had an uneventful hospital course after the procedure and was discharged afebrile after 3 days of hospital stay.

  6. Liver sinusoidai endothelial cell injury by neutrophils in rats with acute obstructive cholangitis

    Institute of Scientific and Technical Information of China (English)

    Jian-Ping Gong; Chuan-Xin Wu; Chang-An Liu; Sheng-Wei Li; Yu-Jun Shi; Xu-Hong Li; Yong Peng

    2002-01-01

    AIM: The objective of this study is to elucidate the potentialrole of poly-morphonuclear neutrophils (PMN) in thedevelopment of such a sinusoidal endothelial cell (SEC)injury during early acute obstructive cholangitis (AOC) inrats.METHODS: Twenty one Wistar rats were divided into threegroups: the AOC group, the bile duct ligated group (BDLgroup), and the sham operation group (SO group ) . Thecommon bile duct (CBD) of rats in AOC group was duallyligated and 0.2 mi of the E. coli O111 B4 (5 × 109 cfu/ml)suspension was injected into the upper segment, in BDLgroup, only the CBD was ligated and in SO group, neitherinjection of E. coil suspension nor CBD ligation was done,but the same operative procedure. Such group consisted ofseven rats, all animals were killed 6 h after the operation.Morphological changes of the liver were observed underlight and electron microscope. Expression of intercellularadhesion molecule-1 (ICAM-1) mRNA in hepatic tissue wasdetermined with reverse transcription polymerase chainreaction ( RT-PCR ). The serum levels of alanineaminotransferase (ALT) were determined with anutoanalygerand cytokine-induced neutrophil chemoattractant (ClNC)was determined by enzyme-linked immunosorbent assay( ELISA).RESULTS: Neutrophils was accumulated in the hepaticsinusoids and sinusoidal endothelial cell injury existed inAOC group. In contrast, in rats of BDL group, all thefeatures of SEC damage were greatly reduced. Expressionof ICAM- 1 mRNA in hepatic tissue in three groups were 7.54±0.82, 2.87 ± 0.34, and 1.01 ± 0.12, respectively. Therewere significant differences among three groups ( P< 0.05).The serum ClNC levels in the three groups were 188 ± 21 ng@L-1 , 94 ± 11 ng@ L-1 , and 57 ± 8 ng@ L-1 , respectively. Therewere also significant differences among the three groups ( P< 0.05). Activity of the senum ALT was 917 ± 167 nkat@ L1 , 901 ±171 nkat@ L-1, and 908 ± 164 nkat@L-1, respectively, ( P> 0.05).CONCLUSION: Hepatic SEC injury occurs earlier

  7. Evaluation of Biliary Calprotectin as a Biomarker in Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Gauss, Annika; Sauer, Peter; Stiehl, Adolf; Rupp, Christian; Krisam, Johannes; Leopold, Yvonne; Kloeters-Plachky, Petra; Stremmel, Wolfgang; Gotthardt, Daniel

    2016-04-01

    Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the bile ducts with limited therapeutic options except liver transplantation. Reliable biomarkers to predict the disease course are unavailable, and currently employed disease activity scores such as the Mayo risk score (MRS) have limitations. The present study aims to evaluate biliary calprotectin as a marker of disease activity and prognosis in PSC.This is a monocentric retrospective observational study. Calprotectin concentrations were measured by an enzyme-linked immunosorbent assay in bile samples collected by endoscopic retrograde cholangiography from 106 PSC patients and 20 controls. Biliary calprotectin concentrations were compared between the 2 groups. In PSC patients, results were evaluated with regard to the presence of dominant bile duct stenoses, bile microbiology, MRS, survival free of liver transplantation, and necessity for bile duct interventions in the further disease course.Median (interquartile ranges) biliary calprotectin concentrations were higher in PSC patients than in controls (3646 ng/mL, 249-9748 vs 116 ng/mL, 104-655; P calprotectin concentrations were associated with the presence of microbes in bile (P = 0.02), the occurrence of dominant bile duct stenosis at any time in the disease course (P = 0.005), and the necessity for future bile duct interventions (P = 0.02). Patients with biliary calprotectin concentrations above a cut-off of 11,610 ng/mL displayed significantly shorter transplantation-free survival than those with biliary calprotectin concentrations ≤11,610 ng/mL (P calprotectin concentration (>11,610 ng/mL) as a risk factor of shorter transplantation-free survival of PSC patients (P 142.5 U/L) (P = 0.006), high MRS (≥2) (P calprotectin may be a valuable additional marker for disease activity and a predictor of outcome in PSC, so that further studies for evaluation of calprotectin in this disease are warranted.

  8. Microbiological diagnostics of fungal infections

    Directory of Open Access Journals (Sweden)

    Corrado Girmenia

    2013-07-01

    Full Text Available Laboratory tests for the detection of fungal infections are easy to perform. The main obstacle to a correct diagnosis is the correlation between the laboratory findings and the clinical diagnosis. Among pediatric patients, the most common fungal pathogen is Candida. The detection of fungal colonization may be performed through the use of chromogenic culture media, which allows also the identification of Candida subspecies, from which pathogenicity depends. In neonatology, thistest often drives the decision to begin a empiric therapy; in this regard, a close cooperation between microbiologists and clinicians is highly recommended. Blood culture, if positive, is a strong confirmation of fungal infection; however, its low sensitivity results in a high percentage of false negatives, thus decreasing its reliability. Molecular diagnostics is still under evaluation, whereas the detection of some fungal antigens, such as β-D-glucan, galactomannan, mannoprotein, and cryptococcal antigen in the serum is used for adults, but still under evaluations for pediatric patients.http://dx.doi.org/10.7175/rhc.v4i1S.862

  9. Fungal artificial chromosomes for mining of the fungal secondary metabolome

    OpenAIRE

    2015-01-01

    Background With thousands of fungal genomes being sequenced, each genome containing up to 70 secondary metabolite (SM) clusters 30–80 kb in size, breakthrough techniques are needed to characterize this SM wealth. Results Here we describe a novel system-level methodology for unbiased cloning of intact large SM clusters from a single fungal genome for one-step transformation and expression in a model host. All 56 intact SM clusters from Aspergillus terreus were individually captured in self-rep...

  10. ERCP术后胆管炎的相关因素分析%Analysis of the Relative Factors for Post-ERCP Cholangitis

    Institute of Scientific and Technical Information of China (English)

    张雨雨; 刘坤

    2016-01-01

    This study was aim to deifne the incidence of post-ERCP cholangitis by a retrospective review of the patients who underwent ERCP in our hospital from January 2015 to October 2015, analysis the relative factors. There are 131 clinical cases which underwent ERCP were involved in this study, 18 clinical cases developed cholangitis after ERCP, divided into two groups according to the occurrence of post-ERCP cholangitis. SPSS 22.0 was used for the statistical analysis. Relative factors (age, gender, operation during ERCP, obstruction site) were analyzed to predict post-ERCP cholangitis. Obstruction site is statistical y signiifcant. 10 cholangitis patients sent etiological examination and 9 bacteria were detected, most of bacteria were gram negative bacili.%对131例行内镜下逆行胰胆管造影术(ERCP)的住院患者进行回顾性调查,18例发生ERCP术后胆管炎。将患者按是否发生ERCP术后胆管炎分为术后胆管炎组和非胆管炎组,分析相关因素(年龄、性别、术中操作、梗阻部位)对ERCP术后胆管炎发生的影响,结果显示梗阻部位与术后胆管炎有关。18例ERCP术后胆管炎患者10例送检病原学检查,胆汁培养共获得菌株9株,以革兰氏阴性杆菌为主。

  11. The Fungal Defensin Family Enlarged

    Directory of Open Access Journals (Sweden)

    Jiajia Wu

    2014-08-01

    Full Text Available Fungi are an emerging source of peptide antibiotics. With the availability of a large number of model fungal genome sequences, we can expect that more and more fungal defensin-like peptides (fDLPs will be discovered by sequence similarity search. Here, we report a total of 69 new fDLPs encoded by 63 genes, in which a group of fDLPs derived from dermatophytes are defined as a new family (fDEF8 according to sequence and phylogenetic analyses. In the oleaginous fungus Mortierella alpine, fDLPs have undergone extensive gene expansion. Our work further enlarges the fungal defensin family and will help characterize new peptide antibiotics with therapeutic potential.

  12. Invasive Fungal Infections Secondary to Traumatic Injury

    Directory of Open Access Journals (Sweden)

    Ryan Kronen

    2017-09-01

    Full Text Available Invasive fungal infection (IFI is a rare but serious complication of traumatic injury. The purpose of this article is to review the epidemiology, natural history, mycology, risk factors, diagnosis, treatment, and outcomes associated with post-traumatic IFI in military and civilian populations. The epidemiology of post-traumatic IFI is poorly characterized, but incidence appears to be rising. Patients often suffer from severe injuries and require extensive medical interventions. Fungi belonging to the order Mucorales are responsible for most post-traumatic IFI in both civilian and military populations. Risk factors differ between these cohorts but include specific injury patterns and comorbidities. Diagnosis of post-traumatic IFI typically follows positive laboratory results in the appropriate clinical context. The gold standard of treatment is surgical debridement in addition to systemic antifungal therapy. Patients with post-traumatic IFI may be at greater risk of amputation, delays in wound healing, hospital complications, and death as compared to trauma patients who do not develop IFI. More research is needed to understand the factors surrounding the development and management of post-traumatic IFI to reduce the significant morbidity and mortality associated with this disease.

  13. Fungal laryngitis in immunocompetent patients.

    Science.gov (United States)

    Ravikumar, A; Prasanna Kumar, S; Somu, L; Sudhir, B

    2014-01-01

    The diagnosis of fungal laryngitis is often overlooked in immunocompetent patients because it is commonly considered a disease of the immunocompromised. Further confusion is caused by clinical and histological similarity to more common conditions like Leukoplakia. Demonstration of hyperkeratosis particularly if associated with intraepithelial neutrophils on biopsy should trigger a search for fungus using specialized stains. These patients usually present with hoarseness of voice. Pain is present inconsistently along with dysphagia and odynophagia. We present three cases of fungal laryngitis in immunocompetent patients out of which one underwent microlaryngeal surgery with excision biopsy. All these patients responded well with oral antifungal therapy.

  14. Orbital Complications of Sinusitis A Review

    Directory of Open Access Journals (Sweden)

    Balasubramanian Thiagarajan

    2014-08-01

    Full Text Available Abstract: Introduction: Despite availability of excellent antibiotics, orbital complications’ following sinusitis is rather common. With the emergence of fungal sinusitis orbital involvement by the disease is getting frequent. Prevalence of life style disorders like diabetes mellitus has added to the woes. This article attempts to review the entire gamut of orbital complications following sinus infections. Aim: This study aims at analyzing orbital complications following sinusitis at Government Stanley Medical College Hospital during the period 2009 – 2013. Study design: Retrospective study Methodology: Cases with rhinosinusitis treated in our Institution during the period 2009-2013 were taken up for analysis. 112 patients were chosen for the study. Their case records were analyzed. CT scan images taken during the time of admission were also evaluated. Patients with orbitalcomplications following rhinosinusitis were included. Chandler’s classification was used to categorize the stage of disease. Results: 112 patients were included in the study. 76 Male patients 36 Female patients 26 patients developed orbital complications Number of male patients with orbital complication – 22 Number of female patients with orbital complication – 4 Number of patients who died due to complications - 2 Conclusion: High degree of suspicion, early diagnosis and aggressive medical management of Chandler’s categories I and II will go a long way in preventing irreparable damage to vision. All our patients were managed initially with parenteral antibiotics. Patients who do not show improvement even after 4 days of antibiotic therapy were taken up for surgical management.

  15. Bacterial and Fungal Endophthalmitis.

    Science.gov (United States)

    Durand, Marlene L

    2017-07-01

    Endophthalmitis is a severe eye infection that may result in permanent loss of useful vision in the affected eye. Most cases are exogenous and occur as a complication of cataract surgery, an intravitreal injection, or penetrating ocular trauma. Endogenous endophthalmitis results from hematogenous seeding of the eye by bacteria or fungi, but bacteremia or fungemia may be transient and patients may present without symptoms of systemic infection. Nearly all endophthalmitis patients present with decreased vision, and some also have eye pain. Eye examination usually reveals a hypopyon and intraocular inflammation. Diagnosis is clinical, supported by cultures of the vitreous and/or aqueous or by blood cultures in some endogenous cases. Molecular diagnostic techniques have been used in research laboratories for pathogen identification in endophthalmitis and offer the possibility of rapid diagnosis, including in culture-negative cases. Intravitreal injection of antibiotics is the most important component of treatment; some cases also benefit from surgical debridement of the vitreous by a vitrectomy. The visual outcome depends partly on the pathogen: coagulase-negative staphylococcal endophthalmitis has a better prognosis than does streptococcal endophthalmitis, for example. Endophthalmitis is a medical emergency, and prompt diagnosis and treatment are essential for saving vision.

  16. Protective immune responses to fungal infections.

    Science.gov (United States)

    Rivera, A

    2014-09-01

    The incidence of fungal infections has been on the rise over several decades. Fungal infections threaten animals, plants and humans alike and are thus of significant concern to scientists across disciplines. Over the last decade, significant advances on fungal immunology have lead to a better understanding of important mechanisms of host protection against fungi. In this article, I review recent advances of relevant mechanisms of immune-mediated protection to fungal infections.

  17. Diagnosis and management of bile stone disease and its complications.

    Science.gov (United States)

    Cremer, Anneline; Arvanitakis, Marianna

    2016-03-01

    Bile stone disease is one of the most prevalent gastroenterological diseases with a considerable geographical and ethnic variation. Bile stones can be classified according their origin, their localization and their biochemical structure. Development and clinical expression depend on a complex interaction between congenital and acquired risk factors. Indeed, bile stones can be either asymptomatic, or cause biliary colic or complications such as acute cholecystitis, jaundice, cholangitis and acute pancreatitis. Diagnosis is based on a combination of clinical features, laboratory findings and imaging techniques and correct identification of symptomatic gallstone patients is essential before cholecystectomy. Transabdominal ultrasonography is the gold standard for the diagnosis of gallstones. However, endoscopic ultrasonography, magnetic resonance cholangiopancreatography and intraoperative cholangiography may also play a role in the diagnosis of bile stones. Management includes prevention measures against modifiable risk factors. Biliary colic and acute cholecystitis are common indications of laparoscopic cholecystectomy, while endoscopic retrograde cholangiopancreatography (ERCP) with endoscopic biliary sphincterotomy and stone extraction is the gold standard for the treatment of common bile duct (CBD) stones. Timing of ERCP and cholecystectomy are of critical importance in the management. Lithotripsy modalities are generally reserved for patients with technically difficult CBD stone removal. Percutaneous access combined with lithotripsy may be helpful for complicated intrahepatic stones.

  18. Association Between HLA Haplotypes and Increased Serum Levels of IgG4 in Patients With Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Berntsen, Natalie L; Klingenberg, Olav; Juran, Brian D; Benito de Valle, Maria; Lindkvist, Björn; Lazaridis, Konstantinos N; Boberg, Kirsten Muri; Karlsen, Tom H; Hov, Johannes Roksund

    2015-05-01

    Increased serum levels of IgG4 have been reported in 9%-15% of patients with primary sclerosing cholangitis (PSC); it is not clear whether this increase contributes to pathogenesis. We performed genetic analyses of the HLA complex in patients with PSC from Norway, Sweden, and from the United States. We found an association between levels of IgG4 above the upper reference limit and specific HLA haplotypes. These patients had a significantly lower frequency of the strongest PSC risk factor, HLA-B*08, than patients without increased IgG4, and significantly higher frequencies of HLA-B*07 and HLA-DRB1*15. HLA genotype therefore might affect the serum concentration of IgG4, and increased IgG4 might be a marker of a distinct phenotype of PSC.

  19. 化脓性胆管炎的研究进展%Research progress of acute suppurative cholangitis

    Institute of Scientific and Technical Information of China (English)

    杨川义

    2013-01-01

    急性化脓性胆管炎(acute suppurative cholangitis,ASC)是由于急性胆管梗阻并继发化脓性感染所致,是肝外胆道梗阻的一种严重并发症.本病起病急骤,病情复杂而进展迅速,并发症多而且病死率高,是良性胆道疾患引起病人死亡的主要病症,已引起临床普遍重视.本文对急性化脓性胆管炎的病因、病理变化、诊断标准以及治疗等研究进展作一综述.

  20. Imaging fungal infections in children

    NARCIS (Netherlands)

    Ankrah, Alfred O.; Sathekge, Mike M; Dierckx, Rudi A.J.O.; Glaudemans, Andor W.J.M.

    2016-01-01

    Fungal infections in children rarely occur, but continue to have a high morbidity and mortality despite the development of newer antifungal agents. It is essential for these infections to be diagnosed at the earliest possible stage so appropriate treatment can be initiated promptly. The addition of

  1. Microbiology of systemic fungal infections

    Directory of Open Access Journals (Sweden)

    Chakrabarti A

    2005-01-01

    Full Text Available The increased incidence of systemic fungal infections in the past two decades has been overwhelming. Earlier, it was pathogenic dimorphic fungi, which were known to cause systemic infections. However, starting from the 1960s, opportunistic fungi started causing more number of infections, especially in the immunocompromised host. More recently, newer and less common fungal agents are being increasingly associated with infection in immunosuppressed hosts. Amongst dimorphic fungi, infections due to Histoplasma capsulatum and Penicillium marneffei are increasingly reported in patients with AIDS in India. H. capsulatum is found country wide, but P. marneffei remains restricted to Manipur state. Although both varieties of C. neoformans , C. neoformans var. neoformans (serotypes A & D, and C. neoformans var. gattii (serotypes B & C are reported in India, most of the cases reported are of serotype A. Increased incidence of cryptococcosis is reported from all centers with the emergence of AIDS. Systemic infection due to species under Candida , Aspergillus and zygomycetes is widely prevalent in nosocomial setting, and outbreaks due to unusual fungi are reported occasionally from tertiary care centers. This global change in systemic fungal infections has emphasized the need to develop good diagnostic mycology laboratories in this country and to recognize this increasingly large group of potential fungal pathogens.

  2. Fungal endophyte diversity in Sarracenia

    Science.gov (United States)

    Fungal endophytes were isolated from four species of the carnivorous pitcher plant genus Sarracenia: S. minor, S. oreophila, S. purpurea, and S. psittacina. Twelve taxa of fungi, eight within the Ascomycota and four within the Basidiomycota, were identified based on PCR amplification and sequencing ...

  3. Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis

    Science.gov (United States)

    Weismüller, Tobias J.; Trivedi, Palak J.; Bergquist, Annika; Imam, Mohamad; Lenzen, Henrike; Ponsioen, Cyriel Y.; Holm, Kristian; Gotthardt, Daniel; Färkkilä, Martti A.; Marschall, Hanns-Ulrich; Thorburn, Douglas; Weersma, Rinse K.; Fevery, Johan; Mueller, Tobias; Chazouillères, Olivier; Schulze, Kornelius; Lazaridis, Konstantinos N.; Almer, Sven; Pereira, Stephen P.; Levy, Cynthia; Mason, Andrew; Naess, Sigrid; Bowlus, Christopher L.; Floreani, Annarosa; Halilbasic, Emina; Yimam, Kidist K.; Milkiewicz, Piotr; Beuers, Ulrich; Huynh, Dep K.; Pares, Albert; Manser, Christine N.; Dalekos, George N.; Eksteen, Bertus; Invernizzi, Pietro; Berg, Christoph P.; Kirchner, Gabi I.; Sarrazin, Christoph; Zimmer, Vincent; Fabris, Luca; Braun, Felix; Marzioni, Marco; Juran, Brian D.; Said, Karouk; Rupp, Christian; Jokelainen, Kalle; Benito de Valle, Maria; Saffioti, Francesca; Cheung, Angela; Trauner, Michael; Schramm, Christoph; Chapman, Roger W.; Karlsen, Tom H.; Schrumpf, Erik; Strassburg, Christian P.; Manns, Michael P.; Lindor, Keith D.; Hirschfield, Gideon M.; Hansen, Bettina E.; Boberg, Kirsten M.

    2017-01-01

    BACKGROUND & AIMS Primary sclerosing cholangitis (PSC) is an orphan hepatobiliary disorder associated with inflammatory bowel disease (IBD). We aimed to estimate the risk of disease progression based on distinct clinical phenotypes in a large international cohort of patients with PSC. METHODS We performed a retrospective outcome analysis of patients diagnosed with PSC from 1980 through 2010 at 37 centers in Europe, North America, and Australia. For each patient, we collected data on sex, clinician-reported age at and date of PSC and IBD diagnoses, phenotypes of IBD and PSC, and date and indication of IBD-related surgeries. The primary and secondary endpoints were liver transplantation or death (LTD) and hepatopancreatobiliary malignancy, respectively. Cox proportional hazards models were applied to determine the effects of individual covariates on rates of clinical events, with time-to-event analysis ascertained through Kaplan-Meier estimates. RESULTS Of the 7121 patients in the cohort, 2616 met the primary endpoint (median time to event of 14.5 years) and 721 developed hepatopancreatobiliary malignancy. The most common malignancy was cholangiocarcinoma (n = 594); patients of advanced age at diagnosis had an increased incidence compared with younger patients (incidence rate: 1.2 per 100 patient-years for patients younger than 20 years old, 6.0 per 100 patient-years for patients 21–30 years old, 9.0 per 100 patient-years for patients 31–40 years old, 14.0 per 100 patient-years for patients 41–50 years old, 15.2 per 100 patient-years for patients 51–60 years old, and 21.0 per 100 patient-years for patients older than 60 years). Of all patients with PSC studied, 65.5% were men, 89.8% had classical or large-duct disease, and 70.0% developed IBD at some point. Assessing the development of IBD as a time-dependent covariate, Crohn’s disease and no IBD (both vs ulcerative colitis) were associated with a lower risk of LTD (unadjusted hazard ratio [HR], 0.62; P

  4. Obeticholic acid for the treatment of primary biliary cholangitis in adult patients: clinical utility and patient selection

    Directory of Open Access Journals (Sweden)

    Bowlus CL

    2016-09-01

    Full Text Available Christopher L Bowlus Division of Gastroenterology and Hepatology, University of California Davis, Davis, CA, USA Abstract: Primary biliary cholangitis (PBC, previously known as primary biliary “cirrhosis”, is a rare autoimmune liver disease characterized by the hallmark autoantibodies to mitochondrial antigens and immune-mediated destruction of small bile duct epithelial cells leading to cholestasis and cirrhosis. Surprisingly, while immune modulators have not been effective in the treatment of PBC, supplementation with the hydrophilic bile acid (BA ursodeoxycholic acid (UDCA has been demonstrated to slow the disease progression. However, a significant minority of PBC patients do not have a complete response to UDCA and remain at risk of continued disease progression. Although the mechanisms of action are not well understood, UDCA provided proof of concept for BA therapy in PBC. Obeticholic acid (OCA, a novel derivative of the human BA chenodeoxycholic acid, is a potent agonist of the nuclear hormone receptor farnesoid X receptor, which regulates BA synthesis and transport. A series of clinical trials of OCA in PBC, primarily in combination with UDCA, have established that OCA leads to significant reductions in serum alkaline phosphatase that are predicted to lead to improved clinical outcomes, while dose-dependent pruritus has been the most common adverse effect. On the basis of these studies, OCA was given conditional approval by the US Food and Drug Administration with plans to establish the long-term clinical efficacy of OCA in patients with advanced PBC. Keywords: primary biliary cholangitis, nuclear receptors, farnesoid X receptor, bile acid, obeticholic acid, ursodeoxycholic acid

  5. HRCT findings of chest complications in patients with leukemia

    Energy Technology Data Exchange (ETDEWEB)

    Tanaka, Nobuyuki; Matsumoto, Tsuneo; Miura, Gouji; Emoto, Takuya; Matsunaga, Naofumi [Department of Radiology, Yamaguchi University School of Medicine (Japan)

    2002-06-01

    High-resolution CT (HRCT) findings of several chest complications occurring in leukemic patients were reviewed. Although most entities show non-specific HRCT findings including ground-glass opacity and air-space consolidation, characteristic findings are observed in several pulmonary complications including Pneumocystis carinii pneumonia, fungal infections, miliary tuberculosis, leukemic infiltration, pulmonary edema, bronchiolitis obliterans, and bronchiolitis obliterans organizing pneumonia. A combination of these characteristic HRCT findings and the information obtained from the clinical setting may help in achieving a correct diagnosis of chest complications occurring in leukemic patients. (orig.)

  6. Subseafloor basalts as fungal habitats

    Science.gov (United States)

    Ivarsson, M.

    2012-09-01

    The oceanic crust is believed to host the largest potential habitat for microbial life on Earth, yet, still we lack substantial information about the abundance, diversity, and consequence of its biosphere. The last two decades have involved major research accomplishments within this field and a change in view of the ocean crust and its potential to harbour life. Here fossilised fungal colonies in subseafloor basalts are reported from three different seamounts in the Pacific Ocean. The fungal colonies consist of various characteristic structures interpreted as fungal hyphae, fruit bodies and spores. The fungal hyphae are well preserved with morphological characteristics such as hyphal walls, septa, thallic conidiogenesis, and hyphal tips with hyphal vesicles within. The fruit bodies consist of large (∼50-200 µm in diameter) body-like structures with a defined outer membrane and an interior filled with calcite. The fruit bodies have at some stage been emptied of their contents of spores and filled by carbonate-forming fluids. A few fruit bodies not filled by calcite and with spores still within support this interpretation. Spore-like structures (ranging from a few µm to ∼20 µm in diameter) are also observed outside of the fruit bodies and in some cases concentrated to openings in the membrane of the fruit bodies. The hyphae, fruit bodies and spores are all closely associated with a crust lining the vein walls that probably represent a mineralized biofilm. The results support a fungal presence in deep subseafloor basalts and indicate that such habitats were vital between ∼81 and 48 Ma.

  7. The Chemical Basis of Fungal Bioluminescence.

    Science.gov (United States)

    Purtov, Konstantin V; Petushkov, Valentin N; Baranov, Mikhail S; Mineev, Konstantin S; Rodionova, Natalja S; Kaskova, Zinaida M; Tsarkova, Aleksandra S; Petunin, Alexei I; Bondar, Vladimir S; Rodicheva, Emma K; Medvedeva, Svetlana E; Oba, Yuichi; Oba, Yumiko; Arseniev, Alexander S; Lukyanov, Sergey; Gitelson, Josef I; Yampolsky, Ilia V

    2015-07-06

    Many species of fungi naturally produce light, a phenomenon known as bioluminescence, however, the fungal substrates used in the chemical reactions that produce light have not been reported. We identified the fungal compound luciferin 3-hydroxyhispidin, which is biosynthesized by oxidation of the precursor hispidin, a known fungal and plant secondary metabolite. The fungal luciferin does not share structural similarity with the other eight known luciferins. Furthermore, it was shown that 3-hydroxyhispidin leads to bioluminescence in extracts from four diverse genera of luminous fungi, thus suggesting a common biochemical mechanism for fungal bioluminescence.

  8. Acute Cholangitis following Intraductal Migration of Surgical Clips 10 Years after Laparoscopic Cholecystectomy

    Directory of Open Access Journals (Sweden)

    Natalie E. Cookson

    2015-01-01

    Full Text Available Background. Laparoscopic cholecystectomy represents the gold standard approach for treatment of symptomatic gallstones. Surgery-associated complications include bleeding, bile duct injury, and retained stones. Migration of surgical clips after cholecystectomy is a rare complication and may result in gallstone formation “clip cholelithiasis”. Case Report. We report a case of a 55-year-old female patient who presented with right upper quadrant pain and severe sepsis having undergone an uncomplicated laparoscopic cholecystectomy 10 years earlier. Computed tomography (CT imaging revealed hyperdense material in the common bile duct (CBD compatible with retained calculus. Endoscopic retrograde cholangiopancreatography (ERCP revealed appearances in keeping with a migrated surgical clip within the CBD. Balloon trawl successfully extracted this, alleviating the patient’s jaundice and sepsis. Conclusion. Intraductal clip migration is a rarely encountered complication after laparoscopic cholecystectomy which may lead to choledocholithiasis. Appropriate management requires timely identification and ERCP.

  9. Fungal rhinosinusitis: what every allergist should know.

    Science.gov (United States)

    Callejas, C A; Douglas, R G

    2013-08-01

    The interaction between fungi and the sinonasal tract results in a diverse range of diseases with an equally broad spectrum of clinical severity. The classification of these interactions has become complex, and this review seeks to rationalize and simplify the approach to fungal diseases of the nose and paranasal sinuses. These conditions may be discussed under two major headings: non-invasive disease (localized fungal colonization, fungal ball and allergic fungal rhinosinusitis) and invasive disease (acute invasive rhinosinusitis, chronic invasive rhinosinusitis and granulomatous invasive rhinosinusitis). A diagnosis of fungal rhinosinusitis is established by combining findings on history, clinical examination, laboratory testing, imaging and histopathology. The immunocompetence of the patient is of great importance, as invasive fungal rhinosinusitis is uncommon in immunocompetent patients. With the exception of localized fungal colonization, treatment of all forms of fungal rhinosinusitis relies heavily on surgery. Systemic antifungal agents are a fundamental component in the treatment of invasive forms, but are not indicated for the treatment of the non-invasive forms. Antifungal drugs may have a role as adjuvant therapy in allergic fungal rhinosinusitis, but evidence is poor to support recommendations. Randomized controlled trials need to be performed to confirm the benefit of immunotherapy in the treatment of allergic fungal rhinosinusitis. In this article, we will summarize the current literature, addressing the controversies regarding the diagnosis and management of fungal rhinosinusitis, and focussing on those aspects which are important for clinical immunologists and allergists.

  10. Aortoventricular Dissociation and Refractory Fungal Endocarditis Caused by a Rare Pathogen Lichtheimia: A Surgical and Medical Management Strategy.

    Science.gov (United States)

    Terrien, Christopher M; Edwards, Niloo M

    2017-01-01

    We report a rare case of prosthetic valve fungal endocarditis caused by Lichtheimia, a subspecies of the order Mucorales. The patient experienced complicated prosthetic valve endocarditis less than 2 months after uneventful coronary artery bypass grafting (CABG) and 2 aortic valve replacements. Ultimately surgical management required aortic root replacement and lifelong antimicrobial agents. We believe this is the first case of fungal endocarditis caused by Lichtheimia. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  11. Pregnancy Complications: Preeclampsia

    Science.gov (United States)

    ... Close X Home > Complications & Loss > Pregnancy complications > Preeclampsia Preeclampsia E-mail to a friend Please fill in ... even if you’re feeling fine. What is preeclampsia? Preeclampsia is a condition that can happen after ...

  12. Complications and Deaths - Hospital

    Data.gov (United States)

    U.S. Department of Health & Human Services — Complications and deaths - provider data. This data set includes provider data for the hip/knee complication measure, the Agency for Healthcare Research and Quality...

  13. Complications and Deaths - National

    Data.gov (United States)

    U.S. Department of Health & Human Services — Complications and deaths - national data. This data set includes national-level data for the hip/knee complication measure, the Agency for Healthcare Research and...

  14. Pregnancy Complications: Anemia

    Science.gov (United States)

    ... Close X Home > Complications & Loss > Pregnancy complications > Anemia Anemia E-mail to a friend Please fill in ... anemia at a prenatal care visit . What causes anemia? Usually, a woman becomes anemic (has anemia) because ...

  15. Pregnancy Complications: Bacterial Vaginosis

    Science.gov (United States)

    ... Complications & Loss > Pregnancy complications > Bacterial vaginosis and pregnancy Bacterial vaginosis and pregnancy E-mail to a friend Please ... this page It's been added to your dashboard . Bacterial vaginosis (also called BV or vaginitis) is an infection ...

  16. Mechanisms of diabetic complications

    National Research Council Canada - National Science Library

    Forbes, Josephine M; Cooper, Mark E

    2013-01-01

    .... These complications occur in the majority of individuals with both type 1 and type 2 diabetes. Among the most prevalent microvascular complications are kidney disease, blindness, and amputations, with current therapies only slowing disease progression...

  17. Eye Complications in IBD

    Science.gov (United States)

    ... Home > Resources > Eye Complications in IBD Go Back Eye Complications in IBD Email Print + Share Approximately 10% ... doctor’s attention sooner rather than later. TYPES OF EYE DISORDERS UVEITIS One of the most common eye ...

  18. An autopsy case showing massive fibrinoid necrosis of the portal tracts of the liver with cholangiographic findings similar to those of primary sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    Hiroshi Hano; Ichiro Takagi; Keisuke Nagatsuma; Tomoe Lu; Chenxi Meng; Satoru Chiba

    2007-01-01

    An 81-year-old Japanese man with jaundice was strongly suspected clinically of having primary sclerosing cholangitis based on clinical examinations and later died of hepatic failure. The entire course of the disease lasted about 10 mo. The autopsy revealed extensive fibrinoid necrosis in the liver, kidney, spleen, pancreas,lung, lymph nodes, and pleura. Particularly extensive fibrinoid necrosis in the portal tracts of the liver induced severe stenoses of the intrahepatic bile ducts, resulting in cholestasis in association with prominent liver injury.There were no findings indicating primary sclerosing cholangitis. The hepatic lesions in this case did not coincide with any known disease including collagen diseases. To clarify the cause of irregular stenoses of the intrahepatic biliary trees on cholangiographic findings, we postulate that some form of immunological derangement might be involved in pathogenesis of fibrinoid necrosis.However, the true etiology remains unknown.

  19. Efficacy of percutaneous transhepatic choledochoplasty of the biliary stricture, using a 16F silastic catheter, in patients with recurrent pyogenic cholangitis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hak Jin; Lee, Suck Hong; Moon, Tae Yong; Kim, Byung Soo [Pusan National Univ. College of Medicine, Pusan (Korea, Republic of)

    1997-08-01

    To evaluate the efficacy of percutaneous transhepatic choledochoplasty of the main biliary stricture, using a 16F silastic foley catheter, in patients suffering from recurrent pyogenic cholangitis In nine patients who had undergone stenting procedure at the site of the main stricture after the total removal of biliary stones, a 16F silastic catheter was held in position for 1-4 (mean, 2.7) months. Using ultrasonogrophy, we assessed the diameter of the bile duct 5cm above the main stricture. During the follow-up period of 1-30 (mean, 18.1) months, the diameter of the bile duct above the main stricture was, in all cases, under 3mm. In patients with recurrent pyogenic cholangitis, choledochoplasty of the biliary stricture, using a 16F silastic catheter, was therapeutically effective.

  20. Spontaneous Fungal Peritonitis in Ascites of Cardiac Origin

    Science.gov (United States)

    Gandhi, Seema; Attar, Bashar M.

    2017-01-01

    Spontaneous fungal peritonitis (SFP) is an infrequent but severe complication most commonly described in patients with liver cirrhosis. We present the first case of culture-proven SFP occurring in cardiogenic ascites. The diagnosis of SFP was clinically challenging as the initial ascites was consistent with the more common diagnosis of spontaneous bacterial peritonitis (SBP). The patient did not respond to antibacterial therapy, however, and the final diagnosis was only made with positive ascitic cultures that grew Candida glabrata. SFP should be considered in patients with either cardiac or cirrhotic ascites and have a delayed or lack of response to traditional SBP treatment.

  1. Fungal keratitis in Lattice dystrophy

    Directory of Open Access Journals (Sweden)

    Chatterjee Samrat

    2010-01-01

    Full Text Available We report a case of fungal keratitis occurring in a patient with lattice dystrophy. A 57-year-old farmer presented with a corneal ulcer following probable entry of paddy husk in the right eye, of one month duration. Corneal scraping revealed pigmented fungal filaments while culture grew Alternaria alternata. Treatment with 5% natamycin eye drops and 1% atropine healed the infection in four weeks. We would like to draw attention to the fact that the cornea in lattice dystrophy is prone to frequent erosions and is a compromised epithelial barrier to invasion by microorganisms. Patients must be made aware of this fact and should seek attention at the earliest following any trivial trauma. Management of minor corneal abrasions in them should be directed at healing the epithelium with adequate lubricants and preventing infection with topical antibiotic prophylaxis.

  2. Structural aspects of fungal allergens.

    Science.gov (United States)

    Crameri, Reto

    2015-03-01

    Despite the increasing number of solved crystal structures of allergens, the key question why some proteins are allergenic and the vast majority is not remains unanswered. The situation is not different for fungal allergens which cover a wide variety of proteins with different chemical properties and biological functions. They cover enzymes, cell wall, secreted, and intracellular proteins which, except cross-reactive allergens, does not show any evidence for structural similarities at least at the three-dimensional level. However, from a diagnostic point of view, pure allergens biotechnologically produced by recombinant technology can provide us, in contrast to fungal extracts which are hardly producible as standardized reagents, with highly pure perfectly standardized diagnostic reagents.

  3. Fungal metabolites with anticancer activity.

    Science.gov (United States)

    Evidente, Antonio; Kornienko, Alexander; Cimmino, Alessio; Andolfi, Anna; Lefranc, Florence; Mathieu, Véronique; Kiss, Robert

    2014-05-01

    Covering: 1964 to 2013. Natural products from bacteria and plants have played a leading role in cancer drug discovery resulting in a large number of clinically useful agents. In contrast, the investigations of fungal metabolites and their derivatives have not led to a clinical cancer drug in spite of significant research efforts revealing a large number of fungi-derived natural products with promising anticancer activity. Many of these natural products have displayed notable in vitro growth-inhibitory properties in human cancer cell lines and select compounds have been demonstrated to provide therapeutic benefits in mouse models of human cancer. Many of these compounds are expected to enter human clinical trials in the near future. The present review discusses the reported sources, structures and biochemical studies aimed at the elucidation of the anticancer potential of these promising fungal metabolites.

  4. Nattrassia mangiferae causing fungal keratitis

    Directory of Open Access Journals (Sweden)

    Kindo A

    2010-01-01

    Full Text Available We report a case of fungal keratitis caused by the coelomycetous fungus Nattrassia mangiferae in a 70 year old gentleman, agriculturist by occupation, with a history of injury to his right eye. The scraping showed narrow septate fungal hyphae on a KOH mount, isolation of a fast growing black mould, which demonstrated hyphae and arthroconidia of varying widths typical of the Scytalidium synanamorph (S. dimidiatum. The formation of the pycnidia, which at maturity, expressed conidia. The patient was started on topical itraconazole one hourly and topical atropine thrice a day. The patient was lost to follow up hence we are not able to comment on the final outcome of the patient.

  5. Complications of blepharoplasty.

    Science.gov (United States)

    Morax, Serge; Touitou, Valerie

    2006-12-01

    The complications of blepharoplasty are infrequent, most often minor and transient, and rarely major and permanent with functional or aesthetic consequences. Treatment is above all preventive with screening of "at risk" patients in whom blepharoplasty would be contra-indicated. Patients must be informed of possible risks through informative booklets stressing the most important points. The complications may affect vision. Partial or complete visual loss due to ischemic optic neuropathy, or rarely to compression of the ocular globe by intraorbital hemorrhage, is the most serious complication. Other visual complications include oculomotor disorders, keratoconjunctivitis sicca, epiphora, and chemosis of lymphatic origin. Eyelid complications are more frequent: ptosis of the upper eyelid or lagophthalmia caused by incorrect resection of the skin, scarring, and eyelid fold anomalies. The most severe aesthetic complication is the malposition of the lower eyelid resulting in retraction, lagophthalmia, ectropion, deformation of the external canthus, or lower eyelid tissue relaxation. These malpositions are often minor, sometimes reversible, but they can be major, with psychological, aesthetic, and functional consequences. Other local complications include enophthalmia and hypo- or hypercorrection. General complications may include pigmentation anomalies or infections extending as far as the orbital fat tissue. Finally, complications observed after the newer procedures of laser surgery include ectropion, burns and residual redness. Complications related to periocular injections of filling material are also mentioned. The discussion of these complications is followed by a comprehensive review of the prevention, diagnosis and management of the complications after blepharoplasty.

  6. Complicated Horseshoe Kidney

    Energy Technology Data Exchange (ETDEWEB)

    Kim, K. S.; Kim, S. R.; Cha, K. S.; Park, S. S. [Chung Ang University College of Medicine, Seoul (Korea, Republic of)

    2010-05-15

    Horseshoe kidney is an important urological anomaly when it is complicated or accompanied by other diseases. Recently we have experienced four cases of horseshoe kidney which were complicated with hydronephrosis, renal stone and adrenal pheochromocytoma. With review of literatures, we emphasize the importance of detection of these complications.

  7. Fungal contaminants in cytopathology specimens

    Directory of Open Access Journals (Sweden)

    Prashant Sharma

    2014-02-01

    Full Text Available A pseudo-epidemic of environmental fungi, most likely by Fusarium spp., leading to inappropriate investigations for disseminated systemic mycosis is described. Subtle diagnostic clues, including the specimens affected, the nature of the host response, and the type of fungal elements noted helped to determine the nature of contaminants. The potential pitfall can be avoided by the knowledge of pertinent disease biology, prompt consultation for infectious diseases, and investigations of the potential environmental sources followed by source control.

  8. Systems biology of fungal infection

    Directory of Open Access Journals (Sweden)

    Fabian eHorn

    2012-04-01

    Full Text Available Elucidation of pathogenicity mechanisms of the most important human pathogenic fungi, Aspergillus fumigatus and Candida albicans, has gained great interest in the light of the steadily increasing number of cases of invasive fungal infections.A key feature of these infections is the interaction of the different fungal morphotypes with epithelial and immune effector cells in the human host. Because of the high level of complexity, it is necessary to describe and understand invasive fungal infection by taking a systems biological approach, i.e., by a comprehensive quantitative analysis of the non-linear and selective interactions of a large number of functionally diverse, and frequently multifunctional, sets of elements, e.g., genes, proteins, metabolites, which produce coherent and emergent behaviours in time and space. The recent advances in systems biology will now make it possible to uncover the structure and dynamics of molecular and cellular cause-effect relationships within these pathogenic interactions.We review current efforts to integrate omics and image-based data of host-pathogen interactions into network and spatio-temporal models. The modelling will help to elucidate pathogenicity mechanisms and to identify diagnostic biomarkers and potential drug targets for therapy and could thus pave the way for novel intervention strategies based on novel antifungal drugs and cell therapy.

  9. Fungal genome resources at NCBI.

    Science.gov (United States)

    Robbertse, B; Tatusova, T

    2011-09-01

    The National Center for Biotechnology Information (NCBI) is well known for the nucleotide sequence archive, GenBank and sequence analysis tool BLAST. However, NCBI integrates many types of biomolecular data from variety of sources and makes it available to the scientific community as interactive web resources as well as organized releases of bulk data. These tools are available to explore and compare fungal genomes. Searching all databases with Fungi [organism] at http://www.ncbi.nlm.nih.gov/ is the quickest way to find resources of interest with fungal entries. Some tools though are resources specific and can be indirectly accessed from a particular database in the Entrez system. These include graphical viewers and comparative analysis tools such as TaxPlot, TaxMap and UniGene DDD (found via UniGene Homepage). Gene and BioProject pages also serve as portals to external data such as community annotation websites, BioGrid and UniProt. There are many different ways of accessing genomic data at NCBI. Depending on the focus and goal of research projects or the level of interest, a user would select a particular route for accessing genomic databases and resources. This review article describes methods of accessing fungal genome data and provides examples that illustrate the use of analysis tools.

  10. Gastrointestinal complications in renal transplant recipients.

    Science.gov (United States)

    Ponticelli, Claudio; Passerini, Patrizia

    2005-06-01

    Gastrointestinal complications are frequent in renal transplant recipients and can include oral lesions, esophagitis, peptic ulcer, diarrhea, colon disorders and malignancy. Oral lesions may be caused by drugs such as cyclosporine and sirolimus, by virus or fungal infections. Leukoplakia may develop in patients with Epstein-Barr virus (EBV) infection. The commonest esophageal disorder is represented by fungal esophagitis usually caused by candida. A number of patients may suffer from nausea, vomiting and gastric discomfort. These disorders are more frequent in patients treated with mycophenolate mofetil (MMF). Peptic ulcer is more rare than in the past. Patients with a history of peptic ulcer are particularly prone to this complication. Other gastroduodenal disorders are caused by cytomegalovirus (CMV) and herpes simplex infection. Diarrhea is a frequent disorder which may be caused by pathogen microorganisms or by immunosuppressive agents. The differential diagnosis may be difficult. Colon disorders mainly consist of hemorrhage, usually sustained by CMV infection, or perforation which may be caused by diverticulitis or intestinal ischemia. Colon cancer, anal carcinoma, and EBV-associated lymphoproliferative disorders are particularly frequent in transplant recipients. A particular gastric lymphoma called mucosa-associated lymphoid tissue (MALT) lymphoma may develop in renal transplant patients. It usually responds to the eradication of Helicobacter pylori.

  11. The Diagnostic Value of Endoscopic Balloon Catheter Usage for Detecting Early-Stage Primary Sclerosing Cholangitis in Endoscopic Retrograde Cholangiopancreatography:A Case Report

    Directory of Open Access Journals (Sweden)

    Burhan Ozdil

    2010-02-01

    Full Text Available A 34-year-old woman was admitted to our clinic with abdominal pain, jaundice and pruritus. Endoscopic retrograde cholangiopancreatography was performed for cholestasis. Endoscopic retrograde cholangiopancreatography (ERCP was judged as normal, after a standard ERCP cannula was used for the cholangiogram. However, marked canalicular irregularities were identified in cholangiography when pressurized contrast agent was administrated via balloon catheter. This cholangiographic view was thought to reveal an early-stage alteration of sclerosing cholangitis. Primary sclerosing cholangitis (PSC is a chronic cholestatic liver disease characterized by destruction and fibrosis of the bile ducts. The diagnosis of PSC is based on typical cholangiographic findings, supported by nonspecific clinical signs and symptoms, cholestatic liver biochemical tests, and liver biopsy. Cholangiography is considered to be the gold standard for the diagnosis of PSC. The diagnosis is easy when diffuse multifocal biliary strictures, the hallmarks of the disease, resulting in a ‘beaded’ appearance on ERCP is detected. However, it may reveal a normal image in an early stage of the disease when bile duct changings are not prominent. We think that balloon catheter ERCP appears to facilitate the diagnosis of early-stage primary sclerosing cholangitis.

  12. Associating pancreaticostomy and biliary-irrigation for staged pancreaticoduodenectomy approach to pancreatic intraductal papillary mucinous neoplasm with recurrent cholangitis and severe jaundice

    Science.gov (United States)

    Dai, Chao; Lou, Siyuan; Zhou, Fan

    2016-01-01

    Abstract Patient concerns: A 63-year-old man was hospitalized with history of abdominal pain since more than 1 year, and that of fever with chills since 2 weeks. Diagnoses: Based on the laboratory investigations and radiologic findings, a preliminary diagnosis of pancreatic intraductal papillary mucinous neoplasm (IPMN) with recurrent cholangitis and severe jaundice was made. Interventions: An initial attempt at endoscopic and image-guided drainage proved unsuccessful. Due to cholangitis, liver dysfunction, and hypoalbuminemia, the patient was deemed to be medically unfit for radical surgery. Therefore we considered a novel strategy of associating pancreaticostomy and biliary-irrigation for staged pancreaticoduodenectomy (APBSP). In the first stage, biliary tract double irrigation (endoscopic nasobiliary drainage and T-tube) in combination with pancreaticostomy was performed, which alleviated the symptoms and helped improve the general condition of the patient. In the second stage, radical pancreaticoduodenectomy was performed. Outcomes: Over a follow-up period of 23 months, no recurrence occurred. Lessons: In this report, we present a previously unreported treatment strategy for pancreatic IPMN with recurrent cholangitis and jaundice. The innovative treatment approach may help advance the understanding and management of this condition. PMID:27902614

  13. Allergen Immunotherapy in an HIV+ Patient with Allergic Fungal Rhinosinusitis

    OpenAIRE

    2015-01-01

    Patients with HIV/AIDS can present with multiple types of fungal rhinosinusitis, fungal balls, granulomatous invasive fungal rhinosinusitis, acute or chronic invasive fungal rhinosinusitis, or allergic fungal rhinosinusitis (AFRS). Given the variable spectrum of immune status and susceptibility to severe infection from opportunistic pathogens it is extremely important that clinicians distinguish aggressive fungal invasive fungal disease from the much milder forms such as AFRS. Here we descr...

  14. Complications of mandibular fractures.

    Science.gov (United States)

    Zweig, Barry E

    2009-03-01

    Before any definitive treatment of mandibular fractures, the patient needs to be evaluated for more potentially life-threatening injuries. Complications can and do occur with treatment of mandibular fractures and can occur during any of the phases of treatment. The development of an accurate diagnosis and appropriate treatment plan is vital in achieving optimal success and decreasing complications. Knowledge of the anatomy and the principles of bone healing is also an important factor in preventing complications. To limit long-term untoward effects, complications should be recognized early and the appropriate treatment should be started before a minor complication becomes a complex one that is more difficult to manage.

  15. Complications of strabismus surgery

    Directory of Open Access Journals (Sweden)

    Scott E Olitsky

    2015-01-01

    Full Text Available All surgeries carry risks of complications, and there is no way to avoid ever having a complication. Strabismus surgery is no different in this regard. There are methods to reduce the risk of a complication during or after surgery, and these steps should always be taken. When a complication occurs, it is important to first recognize it and then manage it appropriately to allow for the best outcome possible. This article will discuss some of the more common and/or most devastating complications that can occur during or after strabismus surgery as well as thoughts on how to avoid them and manage them should they happen.

  16. [Complications of urinary calculi].

    Science.gov (United States)

    Joual, A; Fekak, H; Rabii, R; el Moussasoui, A; Benjelloun, S

    1996-01-01

    Urinary stones is a frequent disease whose renal complications can engage both functional and vital prognosis. We report 769 complicated cases observed 10 years. The diagnosis was made by intravenous urography and ultrasonography. 607 cases were mechanical complications, 582 hydronephrosis, 25 anuria, 262 were infectious complications, 82 chronic pyelonephritis, 60 pyonephrosis, 10 perinephric abscess. Treatment included adapted antibiotic therapy, ureteral catheter in case of anuria ; surgical extraction of the stone nephrectomy was performed in 100 patients. Results were generally good. 9 patients had endstage chronic renal failure. The high frequency of urinary stone complications is due to the fact that most patients consult late. The diagnosis must obviously be made.

  17. Diagnosis of primary sclerosing cholangitis: prospective comparison of MR cholangiography with endoscopic retrograde cholangiography; Diagnostik der primaer sklerosierenden Cholangitis: prospektiver Vergleich von MR-Cholangiographie mit endoskopisch retrograder Cholangiographie

    Energy Technology Data Exchange (ETDEWEB)

    Oberholzer, K.; Mildenberger, P.; Grebe, P.; Bantelmann, M.; Thelen, M. [Mainz Univ. (Germany). Klinik fuer Radiologie; Lohse, A.W. [Mainz Univ. (Germany). Inst. fuer Innere Medizin; Schadeck, T.

    1998-12-01

    Purpose: To assess the accuracy of MR cholangiography (MRC) in the diagnosis of primary sclerosing cholangitis (PSC) in comparison to endoscopic retrograde cholangiography (ERC). Method: 20 patients with PSC were examined by ERC and MRC (1.0 T. HASTE sequence). Visualization and pathologic changes of the extra- and intrahepatic bile ducts were evaluated with both methods. Results: Mural irregularities of the common bile duct were seen with MRC in 6/7 cases, stenoses and dilatation of the common bile duct were detected correctly in all patients. Diffuse, multifocal strictures of the intrahepatic bile duct were the most common intrahepatic findings and correctly diagnosed in all patients. Mural irregularities of the intrahepatic ducts in early stages may be missed by MRC because of the limited spatial resolution. MRC is superior to ERC in visualization of nonopacified intrahepatic ducts. Conclusions: MRC is a reliable, non-invasive method to detect typical diagnostic features of PSC. It should be considered as an adjunct to ERC in patients with suspected PSC for primary diagnosis and as an alternate method for follow-up studies. (orig.) [Deutsch] Ziel: Ueberpruefung der MR-Cholangiographie (MRC) als alternative Methode zur endoskopisch retrograden Cholangiographie (ERC) bei der Diagnostik der primaer sklerosierenden Cholangitis (PSC). Patienten und Methoden: 20 Patienten mit PSC wurden mit der ERC und MRC (1.0 T, HASTE-Sequenz) untersucht. Die Untersuchungen wurden hinsichtlich Darstellbarkeit und pathologischer Veraenderungen des extra- und intrahepatischen Gallengangsystems ausgewertet. Ergebnisse: Wandunregelmaessigkeiten des D. choledochus und hepaticus wurden mit der MRC in 6/7 Faellen richtig beurteilt, Stenosen oder Dilatationen sind bei allen Patienten richtig erkannt worden. Diffuse, multifokale Strikturen und Dilatationen intrahepatischer Gallengaenge konnten mit der MRC ueberlegen praesentiert werden, waehrend mit der ERC aufgrund unzureichender

  18. Phylogenetic Distribution of Fungal Sterols

    Science.gov (United States)

    Weete, John D.; Abril, Maritza; Blackwell, Meredith

    2010-01-01

    Background Ergosterol has been considered the “fungal sterol” for almost 125 years; however, additional sterol data superimposed on a recent molecular phylogeny of kingdom Fungi reveals a different and more complex situation. Methodology/Principal Findings The interpretation of sterol distribution data in a modern phylogenetic context indicates that there is a clear trend from cholesterol and other Δ5 sterols in the earliest diverging fungal species to ergosterol in later diverging fungi. There are, however, deviations from this pattern in certain clades. Sterols of the diverse zoosporic and zygosporic forms exhibit structural diversity with cholesterol and 24-ethyl -Δ5 sterols in zoosporic taxa, and 24-methyl sterols in zygosporic fungi. For example, each of the three monophyletic lineages of zygosporic fungi has distinctive major sterols, ergosterol in Mucorales, 22-dihydroergosterol in Dimargaritales, Harpellales, and Kickxellales (DHK clade), and 24-methyl cholesterol in Entomophthorales. Other departures from ergosterol as the dominant sterol include: 24-ethyl cholesterol in Glomeromycota, 24-ethyl cholest-7-enol and 24-ethyl-cholesta-7,24(28)-dienol in rust fungi, brassicasterol in Taphrinales and hypogeous pezizalean species, and cholesterol in Pneumocystis. Conclusions/Significance Five dominant end products of sterol biosynthesis (cholesterol, ergosterol, 24-methyl cholesterol, 24-ethyl cholesterol, brassicasterol), and intermediates in the formation of 24-ethyl cholesterol, are major sterols in 175 species of Fungi. Although most fungi in the most speciose clades have ergosterol as a major sterol, sterols are more varied than currently understood, and their distribution supports certain clades of Fungi in current fungal phylogenies. In addition to the intellectual importance of understanding evolution of sterol synthesis in fungi, there is practical importance because certain antifungal drugs (e.g., azoles) target reactions in the synthesis of

  19. Phylogenetic distribution of fungal sterols.

    Directory of Open Access Journals (Sweden)

    John D Weete

    Full Text Available BACKGROUND: Ergosterol has been considered the "fungal sterol" for almost 125 years; however, additional sterol data superimposed on a recent molecular phylogeny of kingdom Fungi reveals a different and more complex situation. METHODOLOGY/PRINCIPAL FINDINGS: The interpretation of sterol distribution data in a modern phylogenetic context indicates that there is a clear trend from cholesterol and other Delta(5 sterols in the earliest diverging fungal species to ergosterol in later diverging fungi. There are, however, deviations from this pattern in certain clades. Sterols of the diverse zoosporic and zygosporic forms exhibit structural diversity with cholesterol and 24-ethyl -Delta(5 sterols in zoosporic taxa, and 24-methyl sterols in zygosporic fungi. For example, each of the three monophyletic lineages of zygosporic fungi has distinctive major sterols, ergosterol in Mucorales, 22-dihydroergosterol in Dimargaritales, Harpellales, and Kickxellales (DHK clade, and 24-methyl cholesterol in Entomophthorales. Other departures from ergosterol as the dominant sterol include: 24-ethyl cholesterol in Glomeromycota, 24-ethyl cholest-7-enol and 24-ethyl-cholesta-7,24(28-dienol in rust fungi, brassicasterol in Taphrinales and hypogeous pezizalean species, and cholesterol in Pneumocystis. CONCLUSIONS/SIGNIFICANCE: Five dominant end products of sterol biosynthesis (cholesterol, ergosterol, 24-methyl cholesterol, 24-ethyl cholesterol, brassicasterol, and intermediates in the formation of 24-ethyl cholesterol, are major sterols in 175 species of Fungi. Although most fungi in the most speciose clades have ergosterol as a major sterol, sterols are more varied than currently understood, and their distribution supports certain clades of Fungi in current fungal phylogenies. In addition to the intellectual importance of understanding evolution of sterol synthesis in fungi, there is practical importance because certain antifungal drugs (e.g., azoles target reactions in

  20. CNS fungal meningitis to the “Top of the basilar”

    Directory of Open Access Journals (Sweden)

    CS Logan

    2013-01-01

    Full Text Available Central nervous system (CNS infections are a rare complication of epidural steroid injections and without strong clinical suspicion, fungal organisms may be overlooked among the long differential of causes of meningitis. Rare sequela of fungal meningitis is the development of stroke. To our knowledge, we present the first case of post epidural steroid injection (ESI fungal meningitis leading to a basilar artery stroke, otherwise known as “top of the basilar” syndrome. We present a 49-year-old female with a history of ESIs who presented to the emergency department with headache, neck stiffness, and abdominal pain. She was discharged after her labs and symptoms were deemed inconsistent with meningitis. She was eventually admitted and twelve days after her original ED visit, she was diagnosed with meningitis and started on anti-fungal treatment. She was discharged 88 days later but was readmitted due to left sided weakness and mental status changes. She quickly lost motor and bulbar functions. An MRA showed diminished distal flow through the basilar artery, suggesting near complete occlusion. Although appropriate long term anti-fungal treatment was started, the patient still succumbed to a rare vascular event. Physicians who are treating patients for ESI meningitis should be aware of the potential for vasculitic and encephalitic complications.

  1. SLE合并原发性硬化性胆管炎1例%Systemic lupus erythematosus complicated by primary sclerosing cholangitis: a case report

    Institute of Scientific and Technical Information of China (English)

    王津生; 李学平; 冒长峙

    2002-01-01

    报道1例SLE合并原发性硬化性胆管炎(PSC).患者女,1975年确诊SLE,经皮质类固醇治疗病情缓解.1997年发现胆囊炎,行胆囊切除后仍有反复发热、上腹痛、全身皮肤及巩膜黄染.肝酶学、血清胆红素及腹部B超均有异常改变.1999年2月确诊为PSC.其后PSC病情时有反复,但狼疮本病病情一直平稳.现口服小剂量皮质类固醇维持治疗.

  2. Invasive filamentous fungal infections associated with renal transplant tourism.

    Science.gov (United States)

    Shoham, S; Hinestrosa, F; Moore, J; O'Donnell, S; Ruiz, M; Light, J

    2010-08-01

    'Transplant tourism,' the practice of traveling abroad to acquire an organ, has emerged as an issue in kidney transplantation. We treated a patient who developed invasive aspergillosis of the allograft vascular anastomosis after receiving a kidney transplant in Pakistan, prompting us to review the literature of invasive mycoses among commercial organ transplant recipients. We reviewed all published cases of infections in solid organ transplant recipients who bought their organs abroad and analyzed these reports for invasive fungal infections. Including the new case reported here, 19 cases of invasive fungal infections post commercial kidney transplant occurring in 17 patients were analyzed. Infecting organisms were Aspergillus species (12/19; 63%), Zygomycetes (5/19; 26%), and other fungi (2/19; 5%). Invasive mold infections were present at the transplanted graft in 6/17 patients (35%) with graft loss or death in 13/17 (76%) of patients and overall mortality (10/17) 59%. Invasive fungal infections, frequently originating at the graft site, have emerged as a devastating complication of commercial renal transplant and are associated with high rates of graft loss and death.

  3. STUDY OF FUNGAL INFECTION IN PATIENTS WITH LEUKAEMIA

    Directory of Open Access Journals (Sweden)

    F Zaini

    1994-06-01

    Full Text Available Fungal infections most commonly occur as complications in immunocompromised patients especially in patients with haematologic malignancies. Fifty two leukaemic patients (29 All, 22 AML, 1CML examined for fungal infections. The majority of those patients had fever of undetermined origin, profound leucopenia and had been under treatment of cytotoxics, corticosteroids and antibiotics for prolonged period of time. The study group consisted of 29 males and 23 females and ranged in age from 4 months to 55 years. Diagnosis was established by demonstration of fungus in direct and cultural examinations. In the present study one case of pulmonary aspergillosis due to Aspergillus flavus associated with candidiasis due to Candida albicans, one case of pulmonary mucoromycosis due to Mucor sp. Associated with candidaenia due to C.tropicalis, one case of pulmonary geotrichosis due to Geotrichum candidum, one case fungaemia due to Rhodotorula rubra associated with an undetermined black yeast, two cases of pulmonary candidiasis due to C.albicans and C.tropicalis were observed. C.albicans was the most frequent fungal invader in this study and pulmonary mucoromycosis and geotrichosis are reported for the first time from Iran.

  4. Complications of ERCP: a prospective study

    DEFF Research Database (Denmark)

    Christensen, Merete; Matzen, Peter; Schulze, Svend

    2004-01-01

    -related mortality rate was 1.0%. Post-ERCP pancreatitis occurred in 3.8% of patients (3 deaths). Hemorrhage or perforation occurred with 0.9% and 1.1%, respectively, of the procedures (3 deaths). One perforation that resulted in the death of the patient occurred after placement of an endoprosthesis. Cholangitis...

  5. Real-time in vivo imaging of fungal migration to the central nervous system.

    Science.gov (United States)

    Shi, Meiqing; Colarusso, Pina; Calaruso, Pina; Mody, Christopher H

    2012-12-01

    Recent technical advances have afforded valuable new insights into the pathogenesis of fungal infections in the central nervous system (CNS), which continue to cause devastating complications, particularly in immunocompromised individuals. To cause CNS mycosis, organisms such as Cryptococcus neoformans become blood borne and progress through a series of pathogenic checkpoints that culminate in fungal replication in the brain. Critical steps include fungal arrest in the vasculature of the brain, interaction and signalling of the fungal and endothelial cells leading to transmigration with subsequent parenchymal invasion and fungal replication in the CNS. Previous studies that made use of in vitro and ex vivo approaches contributed greatly to our understanding of brain invasion by fungi. However, the knowledge gained from previous studies relied on in vitro models that did not account for vascular haemodynamics. For this reason, more refined approaches that model blood flow and vascular anatomy are required, andultimately studying fungal invasion and dissemination in vivo. Indeed, in vivo imaging (also known as intravital imaging) has emerged as a valuable technique to probe host-pathogen interactions. In this review, with a focus on C. neoformans, we will provide an overview of the applications of the prior techniques and recent advances, their strengths and limitations in characterizing the migration of fungi into the brain, and unanswered questions that may provide new directions for research.

  6. Small Gallstone Size and Delayed Cholecystectomy Increase the Risk of Recurrent Pancreatobiliary Complications After Resolved Acute Biliary Pancreatitis.

    Science.gov (United States)

    Kim, Sung Bum; Kim, Tae Nyeun; Chung, Hyun Hee; Kim, Kook Hyun

    2017-03-01

    Acute biliary pancreatitis (ABP) is a severe complication of gallstone disease with considerable mortality, and its recurrence rate is reported as 50-90% for ABP patients who do not undergo cholecystectomy. However, the incidence of and risk factors for recurrent pancreatobiliary complications after the initial improvement of ABP are not well established in the literature. The aims of this study were to determine the risk factors for recurrent pancreatobiliary complications and to compare the outcomes between early (within 2 weeks after onset of pancreatitis) and delayed cholecystectomy in patients with ABP. Patients diagnosed with ABP at Yeungnam University Hospital from January 2004 to July 2016 were retrospectively reviewed. The following risk factors for recurrent pancreatobiliary complications (acute pancreatitis, acute cholecystitis, and acute cholangitis) were analyzed: demographic characteristics, laboratory data, size and number of gallstones, severity of pancreatitis, endoscopic sphincterotomy, and timing of cholecystectomy. Patients were categorized into two groups: patients with recurrent pancreatobiliary complications (Group A) and patients without pancreatobiliary complications (Group B). Of the total 290 patients with ABP (age 66.8 ± 16.0 years, male 47.9%), 56 (19.3%) patients developed recurrent pancreatobiliary complications, of which 35 cases were acute pancreatitis, 11 cases were acute cholecystitis, and 10 cases were acute cholangitis. Endoscopic sphincterotomy and cholecystectomy were performed in 134 (46.2%) patients and 95 (32.8%) patients, respectively. Age, sex, BMI, diabetes, number of stone, severity of pancreatitis, and laboratory data were not significantly correlated with recurrent pancreatobiliary complications. The risk of recurrent pancreatobiliary complications was significantly increased in the delayed cholecystectomy group compared with the early cholecystectomy group (45.5 vs. 5.0%, p gallstone less than or equal to 5

  7. Invasive fungal infections after natural disasters.

    Science.gov (United States)

    Benedict, Kaitlin; Park, Benjamin J

    2014-03-01

    The link between natural disasters and subsequent fungal infections in disaster-affected persons has been increasingly recognized. Fungal respiratory conditions associated with disasters include coccidioidomycosis, and fungi are among several organisms that can cause near-drowning pneumonia. Wound contamination with organic matter can lead to post-disaster skin and soft tissue fungal infections, notably mucormycosis. The role of climate change in the environmental growth, distribution, and dispersal mechanisms of pathogenic fungi is not fully understood; however, ongoing climate change could lead to increased disaster-associated fungal infections. Fungal infections are an often-overlooked clinical and public health issue, and increased awareness by health care providers, public health professionals, and community members regarding disaster-associated fungal infections is needed.

  8. Extended complications of urethroplasty

    Directory of Open Access Journals (Sweden)

    Hosam S. Al-Qudah

    2005-08-01

    Full Text Available INTRODUCTION: An extensive study of complications following urethroplasty has never been published. We present 60 urethroplasty patients who were specifically questioned to determine every possible early and late complication. MATERIALS AND METHODS: Retrospective chart review of urethroplasty patients between August 2000 and March 2004. An "open format" questioning style allowed maximal patient reporting of all complications, no matter how minor. RESULTS: 60 patients underwent 62 urethroplasties (24 anterior anastomotic, 19 buccal mucosal and 10 fasciocutaneous, 9 posterior anastomotic with mean follow-up of 29 months. Early complications occurred in 40%, but only 3% were major (rectal injury and urosepsis. Early minor complications included scrotal swelling, scrotal ecchymosis and urinary urgency. Late complications occurred in 48%, but only 18% were significant (erectile dysfunction, chordee and fistula. Late minor complications included a feeling of wound tightness, scrotal numbness and urine spraying. Fasciocutaneous urethroplasty caused the most significant complications, and buccal mucus urethroplasty the least, while also resulting in the lowest recurrence rate (0%. CONCLUSIONS: Serious complications after urethroplasty (3% early and 18% late appear similar to those reported elsewhere, but minor bothersome complications appear to occur in much higher numbers than previously published (39% early and 40% late. While all the early complications were resolved and most (97% were minor, less than half of the late complications were resolved, although most (82% were minor. These complication rates should be considered when counseling urethroplasty patients, and generally tend to support the use of buccal mucosal onlay urethroplasty as it had the lowest rate of serious side effects.

  9. Prevalence and clinical profile of fungal rhinosinusitis

    OpenAIRE

    2016-01-01

    Background: There are only a few landmark studies from the Indian subcontinent on fungal rhinosinusitis. The lack of awareness among clinicians regarding the varying clinical presentations of fungal rhinosinusitis prompted us to undertake this study. Objective: To determine the prevalence, etiologic basis, clinical features, radiologic features, and microscopic features of fungal rhinosinusitis, and to evaluate the various treatment modalities available. Methods: This was a prospective study ...

  10. Reconstructing fungal natural product biosynthetic pathways.

    Science.gov (United States)

    Lazarus, C M; Williams, K; Bailey, A M

    2014-10-01

    Large scale fungal genome sequencing has revealed a multitude of potential natural product biosynthetic pathways that remain uncharted. Here we describe some of the methods that have been used to explore them via heterologous gene expression. We focus on filamentous fungal hosts and discuss the technological challenges and successes behind the reconstruction of fungal natural product pathways. Optimised, efficient heterologous expression of reconstructed biosynthetic pathways promises progress in the discovery of novel compounds that could be utilised by the pharmaceutical and agrochemical industries.

  11. Cutaneous Aspergillus ustus in a lung transplant recipient: emergence of a new opportunistic fungal pathogen.

    Science.gov (United States)

    Vagefi, Parsia A; Cosimi, A Benedict; Ginns, Leo C; Kotton, Camille N

    2008-01-01

    Opportunistic fungal infections remain a significant complication in immunosuppressed patients, especially those having undergone solid-organ transplantation. We report a 39-year-old patient who represents the second case of cutaneous Aspergillus ustus infection in a solid-organ transplant recipient, and the first documented case after lung transplantation. The patient's cutaneous lower extremity aspergillosis responded to a combination of intravenous liposomal amphotericin B, caspofungin and topical terbinafine cream, with a concomitant reduction in immunosuppression. A. ustus is an emerging opportunistic fungal pathogen in transplant recipients.

  12. Seeking Clarity within Cloudy Effluents: Differentiating Fungal from Bacterial Peritonitis in Peritoneal Dialysis Patients

    Science.gov (United States)

    Chavada, Ruchir; Kok, Jen; van Hal, Sebastiaan; Chen, Sharon C-A.

    2011-01-01

    Background Fungal peritonitis is a serious complication of peritoneal dialysis (PD) therapy with the majority of patients ceasing PD permanently. The aims of this study were to identify risk factors and clinical associations that may discriminate between fungal from bacterial peritonitis. Methods We retrospectively identified episodes of fungal peritonitis from 2001–2010 in PD patients at Liverpool and Westmead Hospitals (Australia). Fungal peritonitis cases were matched in a 1∶2 ratio with patients with bacterial peritonitis from each institution's dialysis registry, occurring closest in time to the fungal episode. Patient demographic, clinical and outcome data were obtained from the medical records. Results Thirty-nine episodes of fungal peritonitis (rate of 0.02 episodes per patient-year of dialysis) were matched with 78 episodes of bacterial peritonitis. Candida species were the commonest pathogens (35/39; 90% episodes) with Candida albicans (37%), Candida parapsilosis (32%) and Candida glabrata (13%) the most frequently isolated species. Compared to bacterial peritonitis, fungal peritonitis patients had received PD for significantly longer (1133 vs. 775 catheter-days; p = 0.016), were more likely to have had previous episodes of bacterial peritonitis (51% vs. 10%; p = 0.01), and to have received prior antibacterial therapy (51% vs. 10%; p = 0.01). Patients with fungal peritonitis were less likely to have fever and abdominal pain on presentation, but had higher rates of PD catheter removal (79% vs. 22%; pperitonitis (26.1 days vs. 12.6 days; p = 0.017), but the all-cause 30-day mortality rate was similar in both groups. Fluconazole was a suitable empiric antifungal agent; with no Candida resistance detected. Conclusion Prompt recognition of clinical risk factors, initiation of antifungal therapy and removal of PD catheters are key considerations in optimising outcomes. PMID:22145033

  13. [Drugs used in prophylaxis and treatment of fungal infections in immunosuppressed children].

    Science.gov (United States)

    Balwierz, Walentyna

    2004-01-01

    Contemporary treatment of cancer (intensive chemotherapy, radiotherapy, radical surgery, stem cell transplantation) and severe non-neoplastic blood diseases resulted in significant improvement of treatment results. Currently over 70% of children with cancer can be cured. However, at the same time number of severe complications, including life-threatening infections began to increase. In recent years fungal infections, which constitute approximately 10% of all infections, emerged as an important issue. Their most common etiology is Candida (>85%) and Aspergillus (approx. 1.5%). Fungal infections still result in high mortality (50-95%) in immuno-suppressed patients. Thus it is important to improve diagnosis and prophylaxis, as well as to optimize treatment. Results of treatment of deep organ fungal infections are still unsatisfactory. Currently used drugs show multiple organ toxicity, which limits their use in sufficiently high and effective doses. It is possible to decrease toxicity of currently known drugs, like amphotericin B, by using liposomal formulations. This allows for significant increase of the effective dose without increasing toxicity and improves the drug therapeutic index. There is extensive research on new generations of antifungal drugs whose mechanism of actions is based on specific, unique properties of fungal cells. Preliminary results of research on caspofungin and voriconazole are promising. Important factors improving results of treatment of deep organ fungal infections are so-called immunomodulators. Our presentation will review currently available antifungal drugs and guidelines for treatment of specific fungal infections. The plan of antifungal treatment must include not only the species of fungal pathogen, but also the site and extent of infection, as well as patient status, including stage of primary disease, previous therapy and previous organ damage. Rational management would allow to choose appropriate antifungal drug, optimize dosage

  14. Fungal Endocarditis: Update on Diagnosis and Management.

    Science.gov (United States)

    Pasha, Ahmed Khurshid; Lee, Justin Z; Low, See-Wei; Desai, Hem; Lee, Kwan S; Al Mohajer, Mayar

    2016-10-01

    Fungal endocarditis is an extremely debilitating disease associated with high morbidity and mortality. Candida spp. are the most common isolated organisms in fungal endocarditis. It is most prevalent in patients who are immunosuppressed and intravenous drug users. Most patients present with constitutional symptoms, which are indistinguishable from bacterial endocarditis, hence a high index of suspicion is required for pursuing diagnosis. Diagnosis of fungal endocarditis can be very challenging: most of the time, blood cultures are negative or take a long time to yield growth. Fungal endocarditis mandates an aggressive treatment strategy. A medical and surgical combined approach is the cornerstone of therapy. Copyright © 2016 Elsevier Inc. All rights reserved.

  15. [Complications of operative hysteroscopy].

    Science.gov (United States)

    Agostini, A; Bretelle, F; Cravello, L; Ronda, I; Roger, V; Blanc, B

    2003-05-24

    Assess the prevalence and severity of the various complications of operative hysteroscopy, the context in which they occur and the treatments proposed. A single-center observational study from 1/1/90 to 1/1/99 including 2,116 surgical hysteroscopies (resection of a fibroma (782) or polyp (422), section of a septate uterus (199), synechia uteri (90) and endometrectomy (623)). There were 74 complications (3.5%). The most frequent was uterine perforation (34 cases (1.61%)). There were 13 cases of haemorrhage (0.61%), 16 cases of post-surgical fever (0.76%) and 11 metabolic complications (0.47%). Synechia uteri was the surgical intervention with the greatest risk of complications. The complications of surgical hysteroscopy are rare and relatively benign. Uterine perforation appears to predominate. In our study, the risk of complication was enhanced in the case of synechia uteri.

  16. Regulation of the fungal secretome.

    Science.gov (United States)

    McCotter, Sean W; Horianopoulos, Linda C; Kronstad, James W

    2016-08-01

    The ability of countless representatives of the Kingdom Fungi to adapt to and proliferate in diverse environments is facilitated by regulation of their secretomes to respond to changes in environmental conditions and to mediate interactions with other organisms. Secretome changes often fulfill common functions of nutrient acquisition, facilitation of host/symbiont interactions, cell wall modification, and optimization of the enzyme suite to adapt to new environmental resources. In this review, we expand on our recent work on signaling and the secretome in the pathogenic fungus Cryptococcus neoformans to consider a range of selected examples of regulation of fungal secretomes. These examples include the impact of carbon source and aspects of the response to plant and animal hosts. Additionally, the influence of key protein kinases (e.g., Pka1, Snf1) and transcription factors (e.g., Rim101/PacC) is highlighted to illustrate some underlying regulatory factors influencing the secretome. Although there is a wealth of information about fungal secretomes from both experimentation and genome sequence mining, there are also major gaps in our knowledge about the complete composition of fungal secretomes and mechanisms of dynamic change. For example, a more comprehensive understanding of the composition and regulation of the secretome will require consideration of the emerging roles of unconventional secretion and extracellular vesicles in delivering proteins outside the cell. Overall, changes in the secretome are well documented in diverse fungi and the underlying mechanisms are currently under investigation; however, there remain unknown steps in the regulation of secretory pathways and gaps in understanding the regulation of unconventional secretion, which warrant further research.

  17. Sigmoid volvulus complicating pregnancy

    Directory of Open Access Journals (Sweden)

    De Utpal

    2005-07-01

    Full Text Available Sigmoid volvulus complicating pregnancy is an extremely rare complication with fewer than 76 cases reported in literature. We report a case of sigmoid volvulus complicating pregnancy. The sigmoid colon was resected and Hartman′s colostomy was performed. The patient had a successful recovery. Aggressive resuscitation followed by early surgical intervention should be undertaken to reduce maternal and fetal morbidity and mortality.

  18. 5.5.Fungal disease

    Institute of Scientific and Technical Information of China (English)

    1993-01-01

    930234 Penicilliosis marneffei report of a caseand review of literatures.KANG Xiaoming (康晓明),et al.Nanjing Army General Hosp,210002.Chin J Tuberc & Respir Dis 1992;15(6):336—338.Penicilliosis marneffei is a rare deep fungal in-fection.Southeast Asia is the endemic area.Inthe literatures before 1990,29 cases were re-ported and most of them were diagnosed patho-logically from autopsy.Since 1989 there havebeen more reports of P.marneffei in the HIV in-fected individuals or graft recipient,so far as

  19. Immune response to fungal infections.

    Science.gov (United States)

    Blanco, Jose L; Garcia, Marta E

    2008-09-15

    The immune mechanisms of defence against fungal infections are numerous, and range from protective mechanisms that were present early in evolution (innate immunity) to sophisticated adaptive mechanisms that are induced specifically during infection and disease (adaptive immunity). The first-line innate mechanism is the presence of physical barriers in the form of skin and mucous membranes, which is complemented by cell membranes, cellular receptors and humoral factors. There has been a debate about the relative contribution of humoral and cellular immunity to host defence against fungal infections. For a long time it was considered that cell-mediated immunity (CMI) was important, but humoral immunity had little or no role. However, it is accepted now that CMI is the main mechanism of defence, but that certain types of antibody response are protective. In general, Th1-type CMI is required for clearance of a fungal infection, while Th2 immunity usually results in susceptibility to infection. Aspergillosis, which is a disease caused by the fungus Aspergillus, has been the subject of many studies, including details of the immune response. Attempts to relate aspergillosis to some form of immunosuppression in animals, as is the case with humans, have not been successful to date. The defence against Aspergillus is based on recognition of the pathogen, a rapidly deployed and highly effective innate effector phase, and a delayed but robust adaptive effector phase. Candida albicans, part of the normal microbial flora associated with mucous surfaces, can be present as congenital candidiasis or as acquired defects of cell-mediated immunity. Resistance to this yeast is associated with Th1 CMI, whereas Th2 immunity is associated with susceptibility to systemic infection. Dermatophytes produce skin alterations in humans and other animals, and the essential role of the CMI response is to destroy the fungi and produce an immunoprotective status against re-infection. The resolution

  20. The postanesthetic period. Complications.

    Science.gov (United States)

    Malamed, S F

    1987-01-01

    Postanesthetic complications can occur even in the best of circumstances. Proper preparation of the staff, aggressive monitoring of the recovering patient, and early recognition and management of the complications are essential if the outcome is to be successful. In reviewing postanesthetic complications, two factors are present in the overwhelming majority of situations--hypoxia and hypercarbia--often the direct result of inadequate monitoring during the postanesthetic period. The anesthetic procedure is not over once the anesthetic agents are discontinued. The skillful anesthetist is aware of the possibilities of postoperative complications and prevents problems by employing enhanced monitoring techniques during the recovery phase.

  1. Complications of nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Se Jin Park

    2011-08-01

    Full Text Available Nephrotic syndrome (NS is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox, thromboembolism (e.g., venous thromboembolism and pulmonary embolism, hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension, cardiovascular problems (e.g., hyperlipidemia, acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception. The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS.

  2. Complications of mechanical ventilation

    Directory of Open Access Journals (Sweden)

    Drašković Biljana

    2011-01-01

    Full Text Available Mechanical ventilation of the lungs, as an important therapeutic measure, cannot be avoided in critically ill patients. However, when machines take over some of vital functions there is always a risk of complications and accidents. Complications associated with mechanical ventilation can be divided into: 1 airway-associated complications; 2 complications in the response of patients to mechanical ventilation; and 3 complications related to the patient’s response to the device for mechanical ventilation. Complications of artificial airway may be related to intubation and extubation or the endotracheal tube. Complications of mechanical ventilation, which arise because of the patient’s response to mechanical ventilation, may primarily cause significant side effects to the lungs. During the last two decades it was concluded that mechanical ventilation can worsen or cause acute lung injury. Mechanical ventilation may increase the alveolar/capillary permeability by overdistension of the lungs (volutrauma, it can exacerbate lung damage due to the recruitment/derecruitment of collapsed alveoli (atelectrauma and may cause subtle damages due to the activation of inflammatory processes (biotrauma. Complications caused by mechanical ventilation, beside those involving the lungs, can also have significant effects on other organs and organic systems, and can be a significant factor contributing to the increase of morbidity and mortality in critically ill of mechanically ventilated patients. Complications are fortunately rare and do not occur in every patient, but due to their seriousness and severity they require extensive knowledge, experience and responsibility by health-care workers.

  3. Acute Central Nervous System Complications in Pediatric Acute Lymphoblastic Leukemia.

    Science.gov (United States)

    Baytan, Birol; Evim, Melike Sezgin; Güler, Salih; Güneş, Adalet Meral; Okan, Mehmet

    2015-10-01

    The outcome of childhood acute lymphoblastic leukemia has improved because of intensive chemotherapy and supportive care. The frequency of adverse events has also increased, but the data related to acute central nervous system complications during acute lymphoblastic leukemia treatment are sparse. The purpose of this study is to evaluate these complications and to determine their long term outcome. We retrospectively analyzed the hospital reports of 323 children with de novo acute lymphoblastic leukemia from a 13-year period for acute neurological complications. The central nervous system complications of leukemic involvement, peripheral neuropathy, and post-treatment late-onset encephalopathy, and neurocognitive defects were excluded. Twenty-three of 323 children (7.1%) suffered from central nervous system complications during acute lymphoblastic leukemia treatment. The majority of these complications (n = 13/23; 56.5%) developed during the induction period. The complications included posterior reversible encephalopathy (n = 6), fungal abscess (n = 5), cerebrovascular lesions (n = 5), syndrome of inappropriate secretion of antidiuretic hormone (n = 4), and methotrexate encephalopathy (n = 3). Three of these 23 children (13%) died of central nervous system complications, one from an intracranial fungal abscess and the others from intracranial thrombosis. Seven of the survivors (n = 7/20; 35%) became epileptic and three of them had also developed mental and motor retardation. Acute central neurological complications are varied and require an urgent approach for proper diagnosis and treatment. Collaboration among the hematologist, radiologist, neurologist, microbiologist, and neurosurgeon is essential to prevent fatal outcome and serious morbidity. Copyright © 2015 Elsevier Inc. All rights reserved.

  4. Feline cholecystitis and acute neutrophilic cholangitis: clinical findings, bacterial isolates and response to treatment in six cases.

    Science.gov (United States)

    Brain, Philip H; Barrs, Vanessa R; Martin, Patricia; Baral, Randolph; White, Joanna D; Beatty, Julia A

    2006-04-01

    Clinicopathological findings from six cats with confirmed cholecystitis or acute neutrophilic cholangitis are presented. Historical findings included lethargy and anorexia or inappetence of up to five days duration. On physical examination all cats were pyrexic and four out of six were jaundiced and had cranial abdominal pain. Bile samples were obtained by cholecystocentesis at exploratory coeliotomy (two cases) or by percutaneous, ultrasound-guided cholecystocentesis (four cases). Gall bladder rupture and bile peritonitis occurred subsequent to ultrasound-guided cholecystocentesis in one case. The most common bacterial isolate was Escherichia coli (four cases); E coli was isolated alone in two cases, in combination with a Streptococcus species (one case) and in combination with a Clostridium species (one case). Streptococcus species alone was isolated from one case, as was Salmonella enterica serovar Typhimurium. The latter is the first reported case of Salmonella-associated cholecystitis in a cat. Concurrent pancreatic or intestinal disease was detected histologically in three cases. All cases were treated with antimicrobials based on in vitro susceptibility results. Treatment was successful in five cases. One cat with concurrent diffuse epitheliotropic intestinal lymphoma was euthanased. Percutaneous ultrasound-guided cholecystocentesis is an effective, minimally-invasive technique enabling identification of bacterial isolates in cats with inflammatory hepatobiliary disease.

  5. Cholangiocarcinoma Secondary to Primary Sclerosing Cholangitis in Explanted Livers: A Single-Center Study in the South of Iran

    Directory of Open Access Journals (Sweden)

    Geramizadeh

    2015-12-01

    Full Text Available Background Primary sclerosing cholangitis (PSC is a chronic disease, characterized by chronic inflammation and fibrosis of bile duct epithelial cells. This is a significant contributory factor to the development of malignancy, most commonly cholangiocarcinoma (CCA, which is the second most common malignant liver tumor. Objectives For the first time in Iran, we intend to describe our experience with cases of PSC, with and without CCA, in explanted livers, and compare our results with those found in other areas of the world. Patients and Methods The study population comprised 181 individuals with a diagnosis of PSC who had undergone liver transplantation in the main liver transplant center of Iran, the largest center of hepatobiliary surgery in the south of that country, over a 3-year period between 2012 and 2014. All explanted livers, with and without CCA, were evaluated. Results Of the 181 patients, 16 were found to have CCA, two of whom had been diagnosed after pathologic study of the explanted livers. Therefore it appeared that 8.8% of the patients with PSC in our center had developed CCA before liver transplantation. Conclusions A comparison of our results with those obtained from other centers in both Western and Asian countries (which reported CCA in 3.6% - 36.5% of patients with PSC, shows that the incidence of CCA in the patients we studied is intermediate.

  6. Asymptomatic De Novo Inflammatory Bowel Disease Late After Liver Transplantation for Primary Sclerosing Cholangitis: A Case Report.

    Science.gov (United States)

    Åberg, F; Abdulle, A; Mäkelä, A; Nissinen, M

    2015-11-01

    Guidelines recommend colonoscopy screening for possible asymptomatic inflammatory bowel disease (IBD) in all patients diagnosed with primary sclerosing cholangitis (PSC). PSC-IBD warrants regular dysplasia-surveillance colonoscopy. However, no consensus exists regarding follow-up colonoscopy in PSC patients without IBD who remain asymptomatic. We describe a 43-year-old female who had undergone liver transplantation (LT) due to advanced PSC. Previous colonoscopies had been normal. The post-transplantation course was uneventful, with no rejections and signs of PSC recurrence. Immunosuppression was by tacrolimus monotherapy. She was asymptomatic with normal inflammation markers. A protocol colonoscopy, performed as general dysplasia surveillance 8 years post-transplantation, revealed mucopurulent-covered small superficial ulcerations and erythema diffusely distributed from the cecal to sigmoid colon with intervening normal mucosa and rectal sparing. Histologic examination showed patchy chronic colitis with crypt architectural distortion and mild-moderate inflammation activity. Infection samples were negative. Findings complied with de novo IBD, type unclassified. In conclusion, the link between PSC and clinically silent IBD may manifest after the PSC diagnosis and even several years after LT. Given the increased colorectal cancer risk associated with PSC, IBD, and LT, repeat colonoscopy might be warranted in PSC patients without IBD at initial assessment, and also after LT.

  7. Dense genotyping of immune-related disease regions identifies nine new risk loci for primary sclerosing cholangitis.

    Science.gov (United States)

    Liu, Jimmy Z; Hov, Johannes Roksund; Folseraas, Trine; Ellinghaus, Eva; Rushbrook, Simon M; Doncheva, Nadezhda T; Andreassen, Ole A; Weersma, Rinse K; Weismüller, Tobias J; Eksteen, Bertus; Invernizzi, Pietro; Hirschfield, Gideon M; Gotthardt, Daniel Nils; Pares, Albert; Ellinghaus, David; Shah, Tejas; Juran, Brian D; Milkiewicz, Piotr; Rust, Christian; Schramm, Christoph; Müller, Tobias; Srivastava, Brijesh; Dalekos, Georgios; Nöthen, Markus M; Herms, Stefan; Winkelmann, Juliane; Mitrovic, Mitja; Braun, Felix; Ponsioen, Cyriel Y; Croucher, Peter J P; Sterneck, Martina; Teufel, Andreas; Mason, Andrew L; Saarela, Janna; Leppa, Virpi; Dorfman, Ruslan; Alvaro, Domenico; Floreani, Annarosa; Onengut-Gumuscu, Suna; Rich, Stephen S; Thompson, Wesley K; Schork, Andrew J; Næss, Sigrid; Thomsen, Ingo; Mayr, Gabriele; König, Inke R; Hveem, Kristian; Cleynen, Isabelle; Gutierrez-Achury, Javier; Ricaño-Ponce, Isis; van Heel, David; Björnsson, Einar; Sandford, Richard N; Durie, Peter R; Melum, Espen; Vatn, Morten H; Silverberg, Mark S; Duerr, Richard H; Padyukov, Leonid; Brand, Stephan; Sans, Miquel; Annese, Vito; Achkar, Jean-Paul; Boberg, Kirsten Muri; Marschall, Hanns-Ulrich; Chazouillères, Olivier; Bowlus, Christopher L; Wijmenga, Cisca; Schrumpf, Erik; Vermeire, Severine; Albrecht, Mario; Rioux, John D; Alexander, Graeme; Bergquist, Annika; Cho, Judy; Schreiber, Stefan; Manns, Michael P; Färkkilä, Martti; Dale, Anders M; Chapman, Roger W; Lazaridis, Konstantinos N; Franke, Andre; Anderson, Carl A; Karlsen, Tom H

    2013-06-01

    Primary sclerosing cholangitis (PSC) is a severe liver disease of unknown etiology leading to fibrotic destruction of the bile ducts and ultimately to the need for liver transplantation. We compared 3,789 PSC cases of European ancestry to 25,079 population controls across 130,422 SNPs genotyped using the Immunochip. We identified 12 genome-wide significant associations outside the human leukocyte antigen (HLA) complex, 9 of which were new, increasing the number of known PSC risk loci to 16. Despite comorbidity with inflammatory bowel disease (IBD) in 72% of the cases, 6 of the 12 loci showed significantly stronger association with PSC than with IBD, suggesting overlapping yet distinct genetic architectures for these two diseases. We incorporated association statistics from 7 diseases clinically occurring with PSC in the analysis and found suggestive evidence for 33 additional pleiotropic PSC risk loci. Together with network analyses, these findings add to the genetic risk map of PSC and expand on the relationship between PSC and other immune-mediated diseases.

  8. Giant Splenorenal Shunt in a Young Patient with Autoimmune Hepatitis/Primary Biliary Cholangitis Overlap Syndrome and Portal Vein Thrombosis

    Directory of Open Access Journals (Sweden)

    F. Chegai

    2017-01-01

    Full Text Available We present a case of giant Splenorenal Shunt (SRS associated with portal vein thrombosis in a 37-year-old woman with a twelve-year history of autoimmune hepatitis/primary biliary cholangitis overlap syndrome. At the moment of the CT examination laboratory tests showed creatinine 1.5 mg/dl, bilirubin 1.5 mg/dl, INR 3, and Na 145 mmol/l and the Model End-Stage Liver Disease score was 24. Extensive calcified thrombosis causing complete occlusion of the portal vein lumen and partially occluding the origin of the superior mesenteric vein was present and a small calcified thrombus in the Splenic Vein lumen was also evident. SRS was located among the spleen hilum and the left kidney with a maximum diameter of 3.25 cm and was associated with dilatation of left renal vein and inferior vena cava. After a multidisciplinary evaluation the patient was put on the Regional Liver Transplant waiting list and liver transplantation was performed successfully. Although portal vein thrombosis and SRS are common occurrences in cirrhotic patients, the impact in the natural history of the disease is still unclear. Careful management and accurate imaging protocols are essential in the evaluation of those patients.

  9. Recurrent pyogenic cholangitis: clinico-pathologic correlation of focal attenuation differences on multi-phasic spiral CT

    Energy Technology Data Exchange (ETDEWEB)

    Jeong, Jun Yong; Han, Joon Koo; Kim, Tae Kyoung; Kim, Seog Joon; Kim, Hyun Bum; Choi, Byung Ihn [College of Medicine, Seoul National Univ., Seoul (Korea, Republic of)

    2002-02-01

    To determine the clinical and the pathologic significance of the focal attenuation differences (FAD) and bile duct wall enhancement occurring in recurrent pyogenic cholangitis (RPC) and seen at multiphasic spiral CT. Among the multiphasic (non-contrast, arterial and portal or delayed phase) spiral CT findings of 60 consecutive patients, two types of FAD were noted during the non-contrast phase. These were Type A (iso) and Type B (low attenuation), and their distribution pattern (lobar versus patchy, multifocal) and the and the presence or absence of bile duct wall enhancement were recorded. The radiologic findings were correlated with the clinical and pathologic findings. Two types of FAD were noted in 40 of the 60 patients. Active in flammation was present in 19 of the 27 with Type-A and in ten of the 15 in whom the presence of RPC was pathologically proven. Ten of the 13 with Type-B FAD were in a subclinical state, and nine of the ten in whom RPC was pathologically proven had chronic inflammation. Among 20 patients who did not have FAD, RPC was subclinical in 18 and dormant in nine of the eleven in whom its presence was pathologically proven (p<0.001). Clinico-pathologic correlation with bile duct wall enhancement and the distribution pattern of FAD showed no statistical significance. The inflammatory activity of RPC can be predicted by analysis of the FAD seen at multiphasic spiral CT.

  10. Detection of Cholangiocarcinoma with Magnetic Resonance Spectroscopy of Bile in Patients with and without Primary Sclerosing Cholangitis

    Energy Technology Data Exchange (ETDEWEB)

    Albiin, N.; Smith, I.C.P.; Arnelo, U.; Lindberg, B.; Bergquist, A.; Dolenko, B.; Bryksina, N.; Bezabeh, T. (Divs. of Radiology and Surgery, Dept. for Clinical Science, Intervention, Technology, Karolinska Inst., Stockholm (Sweden))

    2008-10-15

    Background: Early detection of cholangiocarcinoma (CC) is very difficult, especially in patients with primary sclerosing cholangitis (PSC) who are at increased risk of developing CC. Purpose: To evaluate 1H magnetic resonance spectroscopy (1H-MRS) of bile as a diagnostic marker for CC in patients with and without PSC. Material and Methods: The institutional review board approved the study, and all patients gave informed consent. Bile from 49 patients was sampled and investigated using 1H-MRS. MR spectra of bile samples from 45 patients (18 female; age range 22-87 years, mean age 57 years) were analyzed both conventionally and using computerized multivariate analysis. Sixteen of the patients had CC, 18 had PSC, and 11 had other benign findings. Results: The spectra of bile from CC patients differed from the benign group in the levels of phosphatidylcholine, bile acids, lipid, and cholesterol. It was possible to distinguish CC from benign conditions in all patients with malignancy. Two benign non-PSC patients were misclassified as malignant. The sensitivity, specificity, and accuracy were 88.9%, 87.1%, and 87.8%, respectively. Conclusion: With 1H-MRS of bile, cholangiocarcinoma could be discriminated from benign biliary conditions with or without PSC

  11. Fluvoxamine for fatigue in primary biliary cirrhosis and primary sclerosing cholangitis: a randomised controlled trial [ISRCTN88246634

    Directory of Open Access Journals (Sweden)

    Hansen Bettina E

    2004-07-01

    Full Text Available Abstract Background Fatigue is a major clinical problem in many patients with primary biliary cirrhosis (PBC and primary sclerosing cholangitis (PSC. An effective treatment has not been defined. Recently, a large proportion of patients with these diseases was found to have symptoms of depression. Because fatigue is a frequent symptom of depression and there is some evidence that treatment with an antidepressant improves fatigue in patients with fibromyalgia, we hypothesised that the antidepressant fluvoxamine might improve fatigue related to PBC and PSC. Methods Fatigued patients were randomised to receive fluvoxamine (75 mg BID or placebo for a six-week period. Fatigue and quality of life were quantified using a visual analogue scale, the Fisk Fatigue Severity Scale, the Multidimensional Fatigue Inventory and the SF-36. Results Seventeen and 16 patients were allocated to fluvoxamine and placebo, respectively. There was no statistically significant beneficial effect of fluvoxamine on fatigue or quality of life. The median VAS scores in the fluvoxamine and placebo groups were 7.40 and 7.45 at day 0, 6.9 and 7.15 at day 14, 7.45 and 7.65 at day 42 and 7.8 and 8.0 four weeks after treatment discontinuation. Conclusion We found no evidence for a beneficial effect of fluvoxamine on fatigue in these patients with cholestatic liver disease and severe chronic fatigue.

  12. Optimal Fungal Space Searching Algorithms.

    Science.gov (United States)

    Asenova, Elitsa; Lin, Hsin-Yu; Fu, Eileen; Nicolau, Dan V; Nicolau, Dan V

    2016-10-01

    Previous experiments have shown that fungi use an efficient natural algorithm for searching the space available for their growth in micro-confined networks, e.g., mazes. This natural "master" algorithm, which comprises two "slave" sub-algorithms, i.e., collision-induced branching and directional memory, has been shown to be more efficient than alternatives, with one, or the other, or both sub-algorithms turned off. In contrast, the present contribution compares the performance of the fungal natural algorithm against several standard artificial homologues. It was found that the space-searching fungal algorithm consistently outperforms uninformed algorithms, such as Depth-First-Search (DFS). Furthermore, while the natural algorithm is inferior to informed ones, such as A*, this under-performance does not importantly increase with the increase of the size of the maze. These findings suggest that a systematic effort of harvesting the natural space searching algorithms used by microorganisms is warranted and possibly overdue. These natural algorithms, if efficient, can be reverse-engineered for graph and tree search strategies.

  13. COMPLICATIONS OF PERCUTANEOUS NEPHROLITHOTOMY

    Directory of Open Access Journals (Sweden)

    Ottra

    2016-03-01

    Full Text Available BACKGROUND The increasing global prevalence of nephrolithiasis continues to burden the health care delivery systems of developing nations. Percutaneous Nephrolithotomy (PCNL is considered the standard treatment for many types of calculi. This study focuses on the complications of PCNL in private practice setting at a peripheral center using the modified Clavien system and role of Guy’s stone score as a predictor of stone free rate and complications. METHODS This is a prospective cohort study of 480 patients who underwent PCNL during August 2011 to July 2015. The complications were classified according to modified Clavien system and correlated with the stone complexity as per the Guy’s stone score. RESULTS It was found that overall 120 complications were reported in 480 patients with the incidence of complications of Grade I, II, IIIa, IIIb, IVb being 48 (10%, 38 (7.9%, 15 (3.5%, 12 (2.5% and 4 (0.8% respectively. As per the Guy’s stone score there were 336, 104 and 40 cases belonging to GSS I, II and III respectively. All grades of complications were more common in GSS II and III. The stone clearance was found to be complete in 95%, 82% and 75% of GSS I, II, III respectively. CONCLUSION The stone complexity is related to complication rate and GSS helps to predict stone free rate and complications

  14. Pellagra complicating Crohn's disease.

    OpenAIRE

    Zaki, I.; Millard, L

    1995-01-01

    We report a 53-year-old patient with clinical features of pellagra as a complication of Crohn's disease. His symptoms improved rapidly on taking oral nicotinic acid and vitamin B complex. We suggest the paucity of reported cases of pellagra in Crohn's disease is a reflection of poor recognition of this complication.

  15. Celiac disease (CD, ulcerative colitis (UC, and primary sclerosing cholangitis (PSC in one patient: a family study Enfermedad celiaca (EC, colitis ulcerosa (CU y colangitis esclerosante primaria (CEP asociadas en el mismo paciente: estudio familiar

    Directory of Open Access Journals (Sweden)

    V. Cadahía

    2005-12-01

    Full Text Available We discuss the case of a 17-year-old male who at the age of 7 was diagnosed with celiac disease (CD together with ulcerative colitis (UC and primary sclerosing cholangitis (PSC. The patient was treated with gluten-free diet and immunosuppressive drugs (azathioprine, and currently remains asymptomatic. The patient's younger, 12-year-old sister was diagnosed with CD when she was 1.5 years old, and at 7 years she developed type-I diabetes mellitus, which was difficult to control. A family study was made, and both parents were found to be affected with silent CD. All were DQ2 (+. In relation to the case and family study, we provide a series of comments related to CD and its complications.Presentamos el caso de un varón de 17 años, que a la edad de 7 años fue diagnosticado de enfermedad celiaca (EC junto con una colitis ulcerosa (CU y una colangitis esclerosante primaria (CEP asociadas. Fue tratado con DSG e inmuno-supresores tipo azatioprina y se encuentra asintomático en la actualidad. Su hermana menor de 12 años, fue diagnosticada de EC cuando tenía 1,5 años y a los 7 años desarrolló una DM tipo 1 de difícil control. Se realizó un estudio familiar y ambos padres están afectos de una EC silente. Todos ellos son DQ2 (+. A propósito del caso y estudio familiar, se hacen una serie de consideraciones sobre la enfermedad celiaca y el desarrollo de complicaciones.

  16. Bulimia Nervosa - medical complications.

    Science.gov (United States)

    Mehler, Philip S; Rylander, Melanie

    2015-01-01

    As with anorexia nervosa, there are many medical complications associated with bulimia nervosa. In bulimia nervosa, these complications are a direct result of both the mode and the frequency of purging behaviours. For the purposes of this article, we will review in detail the many complications of the two major modes of purging, namely, self-induced vomiting and laxative abuse; these two account for more than 90% of purging behaviours in bulimia nervosa. Some of these complications are potentially extremely dangerous and need to be well understood to effectively treat patients with bulimia nervosa. Other methods of purging, such as diuretic abuse, are much less frequently utilized and will only be mentioned briefly. In a subsequent article, the treatments of these medical complications will be presented.

  17. A novel class of fungal lipoxygenases

    NARCIS (Netherlands)

    Heshof, R.; Jylhä, S.; Haarmann, T.; Jørgensen, A.L.W.; Dalsgaard, T.K.; Graaff, de L.H.

    2014-01-01

    Lipoxygenases (LOXs) are well-studied enzymes in plants and mammals. However, fungal LOXs are less studied. In this study, we have compared fungal LOX protein sequences to all known characterized LOXs. For this, a script was written using Shell commands to extract sequences from the NCBI database an

  18. [Complications of blepharoplasty].

    Science.gov (United States)

    Morax, S

    2004-06-01

    Blepharoplasty complications are infrequent, most often minor and transitory, rarely major and permanent with functional or esthetic consequences. Treatment is above all preventive: screening at risk patients with a history of ophthalmic problems, but also general illnesses that would contraindicate blepharoplasty. Patients must be informed of possible risks through informative booklets with the most important points underlined. Complications can be purely ophthalmological, the more serious sequelae being partial or complete visual loss due to ischemic optical neuropathy, with very poor prognosis, or more rarely compression of the ocular globe by intraorbital hemorrhage, which has a better prognosis provided the origins are quickly recognized and treated immediately. Other visual complications include oculomotor problems, keratoconjunctivitis sicca, epiphora, and chemosis of lymphatic origin. Eyelid complications are more frequent: ptosis on the upper eyelid or lagophthalmia caused by incorrect resection of the skin, scarring and eyelid fold anomalies. The most serious esthetic complication is the malposition of the lower eyelid, which can manifest as retraction, lagophthalmia, ectropion, deformation of the external canthus, or lower eyelid tissue relaxation. These malpositions are quite often minor, sometimes reversible, but at times major, with psychological, esthetic and functional consequences that are difficult for the patient. Other local complications also arise: enophthalmia with a sunken lid, as well as under- and overcorrection. General complications can include scarring related to pigmentation problems and residual hematomas, and exceptionally infections going as far as the orbital fat tissue. Finally, other complications are related to new laser surgical techniques that are responsible for ectropion of the lower eyelid and even burns or residual redness, or complications related to periocular injections of filling material. A comprehensive review of

  19. Matrix regeneration therapy:a solution to enhance healing in fungal keratitis

    Institute of Scientific and Technical Information of China (English)

    Siti Raihan Ishak; Ahmad Nurfahmi Akhtar Ali; Hayati Abdul Aziz; Mohtar Ibrahim; Denis Barritault; Wan Hazabbah Wan Hitam

    2014-01-01

    Corneal ulcers, especially of fungal origin, are a relatively common clinical entity within the spectrum of keratitis in tropical countries. The persistence of a non-healing epithelial defect is a known complication of these ulcers. Despite advances in medical therapy, the management of this condition is still challenging. CACICOL20® is a new ophthalmic matrix therapy that has been proved efficient as a corneal healing agent. To the best of our knowledge there have been reports of the limited use of matrix therapy in ocular healing, specifically in fungal keratitis. We report 2 cases of the efficacy of it as an adjuvant to topical amphotericin B in treating non-healing epithelial defects secondary to fungal corneal ulcers.

  20. Matrix regeneration therapy: a solution to enhance healing in fungal keratitis

    Directory of Open Access Journals (Sweden)

    Siti Raihan Ishak

    2014-01-01

    Full Text Available Corneal ulcers, especially of fungal origin, are a relatively common clinical entity within the spectrum of keratitis in tropical countries. The persistence of a non-healing epithelial defect is a known complication of these ulcers. Despite advances in medical therapy, the management of this condition is still challenging. CACICOL20® is a new ophthalmic matrix therapy that has been proved efficient as a corneal healing agent. To the best of our knowledge there have been reports of the limited use of matrix therapy in ocular healing, specifically in fungal keratitis. We report 2 cases of the efficacy of it as an adjuvant to topical amphotericin B in treating non-healing epithelial defects secondary to fungal corneal ulcers.

  1. Allergic fungal sinusitis causing nasolacrimal duct obstruction.

    Science.gov (United States)

    Kim, Charles; Kacker, Ashutosh; Chee, Ru-Ik; Lelli, Gary J

    2013-04-01

    Allergic fungal sinusitis is thought to represent a chronic autoimmune reaction directed against fungal elements within the sinuses, and is commonly seen in individuals with a history of chronic sinusitis that is refractory to medical therapy. The authors present a case of allergic fungal sinusitis involving the lacrimal drainage system. A 54-year-old woman initially presented with recurrent erythema and induration of the left nasolacrimal sac due to dacryocystitis, which was unresponsive to treatment with topical and systemic antibiotics. Radiological evaluation demonstrated the presence of multiple soft tissue masses along the medial canthi. During subsequent endoscopic dacryocystorhinostomy, significant amounts of allergic mucin were found within the sinuses and marked eosinophilia was present within tissue obtained from the lacrimal sac, findings highly suggestive of allergic fungal sinusitis. A diagnosis of allergic fungal sinusitis should be considered in patients presenting with epiphora in the appropriate clinical context. However, involvement of the lacrimal drainage system is an exceedingly unusual presentation.

  2. Histone Acetylation in Fungal Pathogens of Plants

    Directory of Open Access Journals (Sweden)

    Junhyun Jeon

    2014-03-01

    Full Text Available Acetylation of histone lysine residues occurs in different organisms ranging from yeast to plants and mammals for the regulation of diverse cellular processes. With the identification of enzymes that create or reverse this modification, our understanding on histone acetylation has expanded at an amazing pace during the last two decades. In fungal pathogens of plants, however, the importance of such modification has only just begun to be appreciated in the recent years and there is a dearth of information on how histone acetylation is implicated in fungal pathogenesis. This review covers the current status of research related to histone acetylation in plant pathogenic fungi and considers relevant findings in the interaction between fungal pathogens and host plants. We first describe the families of histone acetyltransferases and deacetylases. Then we provide the cases where histone acetylation was investigated in the context of fungal pathogenesis. Finally, future directions and perspectives in epigenetics of fungal pathogenesis are discussed.

  3. Fungal Adaptations to Mutualistic Life with Ants

    DEFF Research Database (Denmark)

    Kooij, Pepijn Wilhelmus

    . However, in rare occasions fungal symbionts might come into contact with symbionts from other colonies. I showed that in both leaf-cutting ant genera incompatibility reactions between fungal strains can avoid intermixing of different strains, and that these reactions strengthen when genetic distance...... successful. To understand the evolutionary development of domestication of the fungus over the phylogeny of the Attine ants, I compared the average number of nuclei per cell for the fungal symbionts, for each of the different groups of fungus-growing ants. I found that the fungal symbionts of the paleo...... is increased. This pattern, however, becomes distorted when fungal symbionts are contested across ant genera. The most important mechanism in the succession of this mutualism of leaf-cutting ants is the controlled degradation of plant material. I show that in the area of Gamboa, Panama, the two leaf...

  4. Soil fungal community responses to global changes

    DEFF Research Database (Denmark)

    Haugwitz, Merian Skouw

    Global change will affect the functioning and structure of terrestrial ecosystems and since soil fungi are key players in organic matter decomposition and nutrient turnover, shifts in fungal community composition might have a strong impact on soil functioning. The main focus of this thesis...... and nutrient availability and storage. By combining molecular methods such as 454 pyrosequencing and quantitative PCR of fungal ITS amplicons with analyses of soil enzymes, nutrient pools of carbon, nitrogen and phosphorus we were able to characterize soil fungal communities as well as their impact on nutrient...... was therefore to investigate the impact of global environmental changes on soil fungal communities in a temperate and subartic heath ecosystem. The objective was further to determine global change effects on major functional groups of fungi and analyze the influence of fungal community changes on soil carbon...

  5. Redefining the Chronic-Wound Microbiome: Fungal Communities Are Prevalent, Dynamic, and Associated with Delayed Healing

    Directory of Open Access Journals (Sweden)

    Lindsay Kalan

    2016-09-01

    Full Text Available Chronic nonhealing wounds have been heralded as a silent epidemic, causing significant morbidity and mortality especially in elderly, diabetic, and obese populations. Polymicrobial biofilms in the wound bed are hypothesized to disrupt the highly coordinated and sequential events of cutaneous healing. Both culture-dependent and -independent studies of the chronic-wound microbiome have almost exclusively focused on bacteria, omitting what we hypothesize are important fungal contributions to impaired healing and the development of complications. Here we show for the first time that fungal communities (the mycobiome in chronic wounds are predictive of healing time, associated with poor outcomes, and form mixed fungal-bacterial biofilms. We longitudinally profiled 100, nonhealing diabetic-foot ulcers with high-throughput sequencing of the pan-fungal internal transcribed spacer 1 (ITS1 locus, estimating that up to 80% of wounds contain fungi, whereas cultures performed in parallel captured only 5% of colonized wounds. The “mycobiome” was highly heterogeneous over time and between subjects. Fungal diversity increased with antibiotic administration and onset of a clinical complication. The proportions of the phylum Ascomycota were significantly greater (P = 0.015 at the beginning of the study in wounds that took >8 weeks to heal. Wound necrosis was distinctly associated with pathogenic fungal species, while taxa identified as allergenic filamentous fungi were associated with low levels of systemic inflammation. Directed culturing of wounds stably colonized by pathogens revealed that interkingdom biofilms formed between yeasts and coisolated bacteria. Combined, our analyses provide enhanced resolution of the mycobiome during impaired wound healing, its role in chronic disease, and impact on clinical outcomes.

  6. Cardiovascular complications of cirrhosis

    DEFF Research Database (Denmark)

    Møller, Søren; Henriksen, Jens Henrik

    2008-01-01

    and electrophysiological abnormalities, an entity that is different from alcoholic heart muscle disease. Being clinically latent, cirrhotic cardiomyopathy can be unmasked by physical or pharmacological strain. Consequently, caution should be exercised in the case of stressful procedures, such as large volume paracentesis......Cardiovascular complications of cirrhosis include cardiac dysfunction and abnormalities in the central, splanchnic and peripheral circulation, and haemodynamic changes caused by humoral and nervous dysregulation. Cirrhotic cardiomyopathy implies systolic and diastolic dysfunction....... The clinical significance of cardiovascular complications and cirrhotic cardiomyopathy is an important topic for future research, and the initiation of new randomised studies of potential treatments for these complications is needed.  ...

  7. Chapter 8: Invasive fungal rhinosinusitis.

    Science.gov (United States)

    Duggal, Praveen; Wise, Sarah K

    2013-01-01

    Invasive fungal rhinosinusitis (IFRS) is a disease of the paranasal sinuses and nasal cavity that typically affects immunocompromised patients in the acute fulminant form. Early symptoms can often mimic rhinosinusitis, while late symptoms can cause significant morbidity and mortality. Swelling and mucosal thickening can quickly progress to pale or necrotic tissue in the nasal cavity and sinuses, and the disease can rapidly spread and invade the palate, orbit, cavernous sinus, cranial nerves, skull base, carotid artery, and brain. IFRS can be life threatening if left undiagnosed or untreated. While the acute fulminant form of IFRS is the most rapidly progressive and destructive, granulomatous and chronic forms also exist. Diagnosis of IFRS often mandates imaging studies in conjunction with clinical, endoscopic, and histopathological examination. Treatment of IFRS consists of reversing the underlying immunosuppression, antifungal therapy, and aggressive surgical debridement. With early diagnosis and treatment, IFRS can be treated and increase patient survival.

  8. Scabies, lice, and fungal infections.

    Science.gov (United States)

    Taplin, D; Meinking, T L

    1989-09-01

    Scabies and pediculosis capitis are frequent and often unrecognized causes of multiple streptococcal and staphylococcal pyodermas. Permethrin 1 per cent creme rinse (NIX) for head lice, and permethrin 5 per cent topical cream for scabies are new, highly effective, safe, and cosmetically elegant treatments which have shown superiority over older remedies. In populations in which pediculosis and scabies have resisted traditional lindane therapy, patients promptly responded to these permethrin products. Scabies in nursing homes is a persistent and expanding problem which demands a high level of diagnostic suspicion and an integrated approach to management. For fungal infections, several new broad-spectrum oral and topical agents have been introduced. Their successful use is enhanced by appropriate diagnostic tests which can be performed in the office setting. Recommendations and references are given to assist the physician in diagnosis and choice of therapy.

  9. Innate Defense against Fungal Pathogens.

    Science.gov (United States)

    Drummond, Rebecca A; Gaffen, Sarah L; Hise, Amy G; Brown, Gordon D

    2014-11-10

    Human fungal infections have been on the rise in recent years and proved increasingly difficult to treat as a result of the lack of diagnostics, effective antifungal therapies, and vaccines. Most pathogenic fungi do not cause disease unless there is a disturbance in immune homeostasis, which can be caused by modern medical interventions, disease-induced immunosuppression, and naturally occurring human mutations. The innate immune system is well equipped to recognize and destroy pathogenic fungi through specialized cells expressing a broad range of pattern recognition receptors (PRRs). This review will outline the cells and PRRs required for effective antifungal immunity, with a special focus on the major antifungal cytokine IL-17 and recently characterized antifungal inflammasomes.

  10. Comparison of indoor air sampling and dust collection methods for fungal exposure assessment using quantitative PCR.

    Science.gov (United States)

    Cox, Jennie; Indugula, Reshmi; Vesper, Stephen; Zhu, Zheng; Jandarov, Roman; Reponen, Tiina

    2017-08-31

    Evaluating fungal contamination indoors is complicated because of the many different sampling methods utilized. In this study, fungal contamination was evaluated using five sampling methods and four matrices for results. The five sampling methods were a 48 hour indoor air sample collected with a Button™ inhalable aerosol sampler and four types of dust samples: a vacuumed floor dust sample, newly settled dust collected for four weeks onto two types of electrostatic dust cloths (EDCs) in trays, and a wipe sample of dust from above floor surfaces. The samples were obtained in the bedrooms of asthmatic children (n = 14). Quantitative polymerase chain reaction (qPCR) was used to analyze the dust and air samples for the 36 fungal species that make up the Environmental Relative Moldiness Index (ERMI). The results from the samples were compared by four matrices: total concentration of fungal cells, concentration of fungal species associated with indoor environments, concentration of fungal species associated with outdoor environments, and ERMI values (or ERMI-like values for air samples). The ERMI values for the dust samples and the ERMI-like values for the 48 hour air samples were not significantly different. The total cell concentrations of the 36 species obtained with the four dust collection methods correlated significantly (r = 0.64-0.79, p sampling methods (r = 0.68-0.86, p samples primarily because of differences in concentrations of Cladosporium cladosporioides Type 1 and Epicoccum nigrum. A representative type of dust sample and a 48 hour air sample might both provide useful information about fungal exposures.

  11. Infection and Other Complications

    Science.gov (United States)

    ... 6) Position Papers (9) LSAP Perspective (9) National Lymphedema Network is now hiring! The NLN is looking ... and improve your overall health. Other Complications of Lymphedema The sudden onset of swelling anywhere in the ...

  12. Pertussis (Whooping Cough) Complications

    Science.gov (United States)

    ... CDC Cancel Submit Search The CDC Pertussis (Whooping Cough) Note: Javascript is disabled or is not supported ... friendly Fact Sheet Pertussis Vaccination Pregnancy and Whooping Cough Clinicians Disease Specifics Treatment Clinical Features Clinical Complications ...

  13. Pregnancy Complications: Bacterial Vaginosis

    Science.gov (United States)

    ... Loss > Pregnancy complications > Bacterial vaginosis and pregnancy Bacterial vaginosis and pregnancy E-mail to a friend Please ... page It's been added to your dashboard . Bacterial vaginosis (also called BV or vaginitis) is an infection ...

  14. Complications of Mumps

    Science.gov (United States)

    ... Serology Publications and Resources Multimedia MMWR Articles Outbreak Articles Related Links World Health Organization Medline Plus Complications of Mumps Language: English Español (Spanish) Recommend on Facebook Tweet Share Compartir ...

  15. Complications of Circumcision

    Directory of Open Access Journals (Sweden)

    Aaron J. Krill

    2011-01-01

    Full Text Available In the United States, circumcision is a commonly performed procedure. It is a relatively safe procedure with a low overall complication rate. Most complications are minor and can be managed easily. Though uncommon, complications of circumcision do represent a significant percentage of cases seen by pediatric urologists. Often they require surgical correction that results in a significant cost to the health care system. Severe complications are quite rare, but death has been reported as a result in some cases. A thorough and complete preoperative evaluation, focusing on bleeding history and birth history, is imperative. Proper selection of patients based on age and anatomic considerations as well as proper sterile surgical technique are critical to prevent future circumcision-related adverse events.

  16. Complications of shoulder dystocia.

    Science.gov (United States)

    Dajani, Nafisa K; Magann, Everett F

    2014-06-01

    Complications of shoulder dystocia are divided into fetal and maternal. Fetal brachial plexus injury (BPI) is the most common fetal complication occurring in 4-40% of cases. BPI has also been reported in abdominal deliveries and in deliveries not complicated by shoulder dystocia. Fractures of the fetal humerus and clavicle occur in about 10.6% of cases of shoulder dystocia and usually heal with no sequel. Hypoxic ischemic brain injury is reported in 0.5-23% of cases of shoulder dystocia. The risk correlates with the duration of head-to-body delivery and is especially increased when the duration is >5 min. Fetal death is rare and is reported in 0.4% of cases. Maternal complications of shoulder dystocia include post-partum hemorrhage, vaginal lacerations, anal tears, and uterine rupture. The psychological stress impact of shoulder dystocia is under-recognized and deserves counseling prior to home discharge.

  17. Dental Implant Complications.

    Science.gov (United States)

    Liaw, Kevin; Delfini, Ronald H; Abrahams, James J

    2015-10-01

    Dental implants have increased in the last few decades thus increasing the number of complications. Since many of these complications are easily diagnosed on postsurgical images, it is important for radiologists to be familiar with them and to be able to recognize and diagnose them. Radiologists should also have a basic understanding of their treatment. In a pictorial fashion, this article will present the basic complications of dental implants which we have divided into three general categories: biomechanical overload, infection or inflammation, and other causes. Examples of implant fracture, loosening, infection, inflammation from subgingival cement, failure of bone and soft tissue preservation, injury to surround structures, and other complications will be discussed as well as their common imaging appearances and treatment. Lastly, we will review pertinent dental anatomy and important structures that are vital for radiologists to evaluate in postoperative oral cavity imaging.

  18. Concomitant autoimmune cholangitis and occult hepatitis B virus infection: a case report%自身免疫性胆管炎合并隐匿性乙型肝炎1例

    Institute of Scientific and Technical Information of China (English)

    曾经章; 杨京; 张权; 李伟

    2011-01-01

    自身免疫性胆管炎临床上少见,合并隐匿性乙型肝炎更为罕见,由于本例患者合并上述两种疾病,治疗上存在矛盾因素,通过综合治疗后取得较好疗效.%Autoimmune cholangitis is rarely seen clinically, and concomitant autoimmune cholangitis and occult hepatitis B is particularly rare. As there are conflicts in the treatment of the two diseases, a comprehensive treatment is required to achieve better results.

  19. Lack of evidence for association of primary sclerosing cholangitis and primary biliary cirrhosis with risk alleles for Crohn's disease in Polish patients

    Directory of Open Access Journals (Sweden)

    Gaj Pawel

    2008-08-01

    Full Text Available Abstract Background Numerous papers have addressed the association of mutations and polymorphisms of susceptibility genes with autoimmune inflammatory disorders. We investigated whether polymorphisms that confer susceptibility to Crohn's disease could be classified also as predisposing factors for the development of primary sclerosing cholangitis and primary biliary cirrhosis in Polish patients. Methods The study included 60 patients with CD, 77 patients with PSC, of which 61 exhibited IBD (40 UC, 8 CD, and 13 indeterminate colitis, and 144 patients with PBC. All the patients were screened against Crohn's disease associating genetic polymorphisms. The polymorphisms were chosen according to previously confirmed evidence for association with Crohn's disease, including Pro268Ser, Arg702Trp, Gly908Arg and 1007fs in NOD2/CARD15, Leu503Phe/-207G>C in SLC22A4/OCTN1/SLC22A5/OCTN2, Arg30Gln in DLG5, Thr300Ala in ATG16L1, and Arg381Gln, His3Gln and exon-3'UTR in IL23R. Genotyping was carried out using TaqMan SNP genotyping assays. Results We confirmed a strong association between three NOD2/CARD15 gene variants (Pro268Ser, OR = 2.52, 95% CI = 1.34 – 4.75; (Arg702Trp, OR = 6.65, 95% CI = 1.99 – 22.17; (1007fs, OR = 9.59, 95% CI = 3.94 – 23.29, and a weak association between both the protective OCTN1/OCTN2 CC haplotype (OR = 0.28, 95% CI = 0.08 – 0.94, and a variant of ATG16L1 gene (Thr300Ala, OR = 0.468, 95% CI = 0.24 – 0.90 with Crohn's disease. In contrast, none of the polymorphisms exhibited association with susceptibility to primary sclerosing cholangitis and primary biliary cirrhosis, including a group of primary sclerosing cholangitis patients with concurrent IBD. Conclusion Although the clinical data indicate non-random co-occurrence of inflammatory bowel disease and primary sclerosing cholangitis, consistently with the previously published studies, no genetic association was found between the genetic variants predisposing to Crohn

  20. Complications of pancreatic surgery

    Directory of Open Access Journals (Sweden)

    Åke Andrén-Sandberg

    2011-01-01

    Full Text Available Many diseases, including pancreatitis benign tumors and cancer, may require pancreas surgery. Pancreatic resection can lead to a prolonged survival in pancreatic cancer and even a potential chance for cure. However, the pancreatic surgery can result in complications, and high postoperative morbidity rates are still presence. This article reviews the pancreatic abstracts of American Pancreas Club 2011, which involves the more common complications, their prevention and treatment.