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Sample records for acute cholangitis due

  1. Pathogenic aspects of acute cholangitis

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    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

  2. 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.

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

    International Nuclear Information System (INIS)

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

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

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    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.

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

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    Choi, Kyu-Ho; Cho, Young Kwon; An, Jin Kyung; Woo, Jeong-Joo; Kim, Hyun Sook; Choi, Yun-Sun

    2009-01-01

    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.

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

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    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. PMID:27309273

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

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    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.

  8. Early diagnosis and treatment of severe acute cholangitis

    Institute of Scientific and Technical Information of China (English)

    Wei-Zhong Zhang; Yi-Shao Chen; Jin-Wei Wang; Xue-Rong Chen

    2002-01-01

    AIM: To investigate the diagnostic standard for earlyidentification of severe acute cholangitis in order to lowerthe incidence of morbidity and mortality rate.METHODS: A diagnostic standard was proposed in thisstudy as follows: documented biliary duct obstruction byultrasound or computerized tomography or other imagingtools with the manifestation of systemic inflammatoryresponse syndrome (SIRS). The surgical proceduresincluded emergency common bile duct exploration with Ttube insertion or cholecystostomy with secondary commonbile duct exploration. And incidence of postoperativemultiple organ dysfunction syndrome (MODS), duration ofsystemic inflammatory response and hospital mortality wereanalyzed.RESULTS: Fourty - three patients conforming to thediagnostic standard described above were employed in thisstudy. 1 patient was admitted in acutely ill condition andcomplicated with acute relapse of chronic bronchitis,cholecystostomy procedure was performed but the patientwas complicated with postoperative acute lung injury whiclwas treated by assisted mechanical ventilation for 5 d; 2 wllater, two- stage common bile duct Exploration and T tubeinsertion were performed. The remaining 42 patientsunderwent primary common bile duct exploration and T tubeinsertion, 1 developed acute lung injury and recovered 3 dlater, 2 patients developedl acute renal dysfunction, 1 ofwhich recovered 2 d later and the other died on d 4. For allpatients, the postoperative systemic inflammatory responsepersisted for 2 to 8 d with median of 3 d.CONCLUSION: Early diagnosis of severe acute cholangitiscan be made using this diagnostic standard, furtherdevelopment of systemic inflammatory response could beprevented and incidence of MODS as well as hospitalmortality decreased.

  9. 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.

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

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    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.

  11. [A case of portal vein thrombosis complicating acute cholangitis treated successfully with danaparoid sodium].

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    Oku, Takatomi; Kubo, Yasunori; Miseki, Tetsuya; Sakai, Takashi; Yamakawa, Atsushi; Sugata, Hideaki

    2010-11-01

    An 81-year-old woman was referred to our hospital with a diagnosis of acute cholangitis. Endoscopic retrograde cholangiography revealed a common bile duct (CBD) stone. In addition, CT showed thrombus of the right portal vein. Endoscopic sphincterotomy was performed to remove the CBD stone. Thrombosis was treated successfully with danaparoid sodium. It was speculated that the treatment of the acute cholangitis induced thrombolysis by the auto-fibrinolysis system and danaparoid sodium prevented the development of thrombus formation in this case. PMID:21071899

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

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    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.

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

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    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

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

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    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...

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

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    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.

  16. Recurrent Acute Pancreatitis and Cholangitis in a Patient with Autosomal Dominant Polycystic Kidney Disease

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    Kambiz Yazdanpanah; Navid Manouchehri; Elinaz Hosseinzadeh; Mohammad Hassan Emami; Mehdi Karami; Amir Hossein Sarrami

    2013-01-01

    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,...

  17. Comparison of Laboratory Data of Acute Cholangitis Patients Treated with or without Immunosuppressive Drugs

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    Minoru Tomizawa; Fuminobu Shinozaki; Rumiko Hasegawa; Yoshinori Shirai; Noboru Ichiki; Yasufumi Motoyoshi; Takao Sugiyama; Shigenori Yamamoto; Makoto Sueishi

    2014-01-01

    Objective. Symptoms and laboratory data between acute cholangitis (AC) patients treated with and AC patients treated without immunosuppressive drugs (corticosteroids or methotrexate) were compared to identify factors that can be meaningful to the diagnosis of AC. Methods. The Wilcoxon signed-rank test was used for comparison of baseline variables between the patients with AC treated with immunosuppressive drugs and those without it. The chi-squared test was used in the analysis of the symptom...

  18. 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.

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

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    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

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

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    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

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

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    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

  2. [Acute cholangitis in interstenosis space accompanied by two-component unit of the main bile duct].

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    Yurchenko, V V

    2016-02-01

    Palliative treatment of obstructive jaundice with the help of biliary endoprosthesis due to the possible complication of post-intervention in the form of insolvency stent deformation or dislocation. The study features in the postoperative period of the main bile duct endoprosthesis about their two-component unit, described the syndrome of the closed space of the bile ducts. On the basis of observation of 14 patients with the given anatomical feature of endoscopic, who were underwent stenting, was assessed frequency of the syndrome and possibilities of its prevention. Interstenosis space expansion of the main bile duct can be a reason for local cholangitis. For the prevention of cholangitis, it should be carried out a separate drainage of interstenosis space with the help of endoprosthesis or by proximal supra-stenotic extension of two or more stents. PMID:27263209

  3. Acute chylous peritonitis due to acute pancreatitis

    OpenAIRE

    2012-01-01

    We report a case of acute chylous ascites formation presenting as peritonitis (acute chylous peritonitis) in a patient suffering from acute pancreatitis due to hypertriglyceridemia and alcohol abuse. The development of chylous ascites is usually a chronic process mostly involving malignancy, trauma or surgery, and symptoms arise as a result of progressive abdominal distention. However, when accumulation of “chyle” occurs rapidly, the patient may present with signs of peritonitis. Preoperative...

  4. 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.

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

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    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.

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

    International Nuclear Information System (INIS)

    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

  7. Acute chylous peritonitis due to acute pancreatitis

    Institute of Scientific and Technical Information of China (English)

    Georgios K Georgiou; Haralampos Harissis; Michalis Mitsis; Haralampos Batsis; Michalis Fatouros

    2012-01-01

    We report a case of acute chylous ascites formation presenting as peritonitis (acute chylous peritonitis) in a patient suffering from acute pancreatitis due to hypertriglyceridemia and alcohol abuse.The development of chylous ascites is usually a chronic process mostly involving malignancy,trauma or surgery,and symptoms arise as a result of progressive abdominal distention.However,when accumulation of "chyle" occurs rapidly,the patient may present with signs of peritonitis.Preoperative diagnosis is difficult since the clinical picture usually suggests hollow organ perforation,appendicitis or visceral ischemia.Less than 100 cases of acute chylous peritonitis have been reported.Pancreatitis is a rare cause of chyloperitoneum and in almost all of the cases chylous ascites is discovered some days (or even weeks) after the onset of symptoms of pancreatitis.This is the second case in the literature where the patient presented with acute chylous peritonitis due to acute pancreatitis,and the presence of chyle within the abdominal cavity was discovered simultaneously with the establishment of the diagnosis of pancreatitis.The patient underwent an exploratory laparotomy for suspected perforated duodenal ulcer,since,due to hypertriglyceridemia,serum amylase values appeared within the normal range.Moreover,abdominal computed tomography imaging was not diagnostic for pancreatitis.Following abdominal lavage and drainage,the patient was successfully treated with total parenteral nutrition and octreotide.

  8. Acute chylous peritonitis due to acute pancreatitis.

    Science.gov (United States)

    Georgiou, Georgios K; Harissis, Haralampos; Mitsis, Michalis; Batsis, Haralampos; Fatouros, Michalis

    2012-04-28

    We report a case of acute chylous ascites formation presenting as peritonitis (acute chylous peritonitis) in a patient suffering from acute pancreatitis due to hypertriglyceridemia and alcohol abuse. The development of chylous ascites is usually a chronic process mostly involving malignancy, trauma or surgery, and symptoms arise as a result of progressive abdominal distention. However, when accumulation of "chyle" occurs rapidly, the patient may present with signs of peritonitis. Preoperative diagnosis is difficult since the clinical picture usually suggests hollow organ perforation, appendicitis or visceral ischemia. Less than 100 cases of acute chylous peritonitis have been reported. Pancreatitis is a rare cause of chyloperitoneum and in almost all of the cases chylous ascites is discovered some days (or even weeks) after the onset of symptoms of pancreatitis. This is the second case in the literature where the patient presented with acute chylous peritonitis due to acute pancreatitis, and the presence of chyle within the abdominal cavity was discovered simultaneously with the establishment of the diagnosis of pancreatitis. The patient underwent an exploratory laparotomy for suspected perforated duodenal ulcer, since, due to hypertriglyceridemia, serum amylase values appeared within the normal range. Moreover, abdominal computed tomography imaging was not diagnostic for pancreatitis. Following abdominal lavage and drainage, the patient was successfully treated with total parenteral nutrition and octreotide.

  9. Acute chylous peritonitis due to acute pancreatitis.

    Science.gov (United States)

    Georgiou, Georgios K; Harissis, Haralampos; Mitsis, Michalis; Batsis, Haralampos; Fatouros, Michalis

    2012-04-28

    We report a case of acute chylous ascites formation presenting as peritonitis (acute chylous peritonitis) in a patient suffering from acute pancreatitis due to hypertriglyceridemia and alcohol abuse. The development of chylous ascites is usually a chronic process mostly involving malignancy, trauma or surgery, and symptoms arise as a result of progressive abdominal distention. However, when accumulation of "chyle" occurs rapidly, the patient may present with signs of peritonitis. Preoperative diagnosis is difficult since the clinical picture usually suggests hollow organ perforation, appendicitis or visceral ischemia. Less than 100 cases of acute chylous peritonitis have been reported. Pancreatitis is a rare cause of chyloperitoneum and in almost all of the cases chylous ascites is discovered some days (or even weeks) after the onset of symptoms of pancreatitis. This is the second case in the literature where the patient presented with acute chylous peritonitis due to acute pancreatitis, and the presence of chyle within the abdominal cavity was discovered simultaneously with the establishment of the diagnosis of pancreatitis. The patient underwent an exploratory laparotomy for suspected perforated duodenal ulcer, since, due to hypertriglyceridemia, serum amylase values appeared within the normal range. Moreover, abdominal computed tomography imaging was not diagnostic for pancreatitis. Following abdominal lavage and drainage, the patient was successfully treated with total parenteral nutrition and octreotide. PMID:22563182

  10. 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 ...

  11. 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.

  12. A new endoscopic therapeutic method for acute obstructive suppurative cholangitis post Roux-en-Y anastomosis: endoscopic retrograde cholangiography through jejunostomy

    Directory of Open Access Journals (Sweden)

    Zhuo YANG

    2012-01-01

    Full Text Available  Objective  To probe the value of endoscopic retrograde cholangiography (ERC through jejunostomy in patients in whom ERC could not be performed via the mouth after Roux-en-Y anastomosis on the upper gastrointestinal tract. Methods  In two patients suffering from acute obstructive suppurative cholangitis after a radical operation for cholangiocarcinoma, ERC could not be performed through the mouth due to the presence of a long non-functional jejunal loop. A jejunostomy was first done in the afferent loop of the jejunum, and a gastroscope was then inserted via the jejunostomy and passed retrogradely, to find the stoma of the cholangiointestinal anastomosis. ERC was then successfully performed, and followed by endoscopic retrograde biliary drainage (ERBD. Results  The operation was successful. It was found that cholangio-jejunostomy stoma was narrow, and a large amount of purulent mucus was present in the enlarged intrahepatic duct. ERC was done to enlarge the stoma, and a stent was placed into the main branch of the intrahepatic duct. Two patients achieved surgical success and smooth recovery after the operation. Conclusion  ERC through a jejunostomy in the patients who had Roux-en-Y cholangiojejunostomy following radical resection for cholangiocarcinoma, is a safe and effective surgical procedure.

  13. 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.

  14. 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....

  15. Activation of TLR-4 and liver injur y via NF-kappa B in rat with acute cholangitis

    Institute of Scientific and Technical Information of China (English)

    Hong Yu; Shuo-Dong Wu

    2008-01-01

    BACKGROUND:Toll-like receptors (TLRs) are a family of type 1 transmembrane receptors, which can recognize different pathogen-associated molecular patterns. Among them, TLR-4 is speciifc to lipopolysaccharide. It transfers the infection signal into the cell and promotes the translocation of nuclear factor kappa B (NF-κB) to the nucleus and the subsequent transcriptional activation of genes encoding pro-and anti-inlfammatory cytokines and chemokines. Acute cholangitis (AC) is a common biliary tract infection in oriental countries, and often leads to liver injury. The activation of TLR-4 and its signiifcance in liver injury in rats with AC remain unclear. METHODS:Rat models of AC (biliary tract obstruction+E. coli injection, n=36) and control models (biliary tract obstruction+saline, n=18) were made. Liver tissue injury was investigated by pathological examination. The levels of serum TNF-α and IL-10 were measured by enzyme-linked immunosorbent assay, and the expressions of TLR-4, NF-κB mRNAs and proteins in the liver were detected by RT-PCR, immunohistochemical staining and Western blotting, respectively. RESULTS: Severe liver tissue injury in rats with AC was evident as shown by pathological examination. TLR-4 and NF-κB were strongly expressed in the cytoplasm of hepatocytes in the AC group. They were negative or slightly positive in the control group. TLR-4 mRNA and protein in the liver of rats with AC increased 1 hour after biliary tract ligation and E. coli injection, and peaked at 6 hours after surgery. Twenty-four hours later, they began to decrease. The expression of TLR-4 was paralleled by that of NF-κB in the liver and TNF-αin serum. CONCLUSION:The higher expression of TLR-4 in the liver of rats with AC may be involved in liver injury through the activation of NF-κB and release of cytokines such as TNF-α.

  16. 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

  17. 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.

  18. Acute Pancreatitis due to Rupture of the Hydatid Cyst into the Biliary Tract: A Case Report

    Directory of Open Access Journals (Sweden)

    Zulfu Bayhan

    2014-02-01

    Full Text Available Hydatid disease is still endemic in our country and in many parts of the world. Liver is the most common site of hydatid disease. Rupture of hydatid cysts of liver into the biliary ducts can be seen as a complication. Obstructive jaundice, acute cholangitis and much more rarely acute pancreatitis may occur due to rupture of hydatid cysts into the biliary ducts. In this case report, a 38-year-old male patient with findings of acute pancreatitis and obstructive jaundice due to rupture of hydatid cyst into the biliary tract were presented . In our case, Endoscopic retrograde cholangiopancreatography (ERCP was performed to the patient with diagnostic and therapeutic purposes. Parts of germinative membrane located within the common bile duct and causing the obstruction of papillary orifice was seen in ERCP. Germinative membrane components were removed during the process. Endoscopic sphincterotomy was performed to the patient. After the procedure, the clinical findings and laboratory findings of the patient returned to normal rapidly. As a result, it should be noted that rupture of hepatic hydatid cysts into the bile ducts might be a rare cause of biliary pancreatitis. It must be kept in mind that endoscopic sphincterotomy and endoscopic removal of cyst membranes is a very important method for the diagnosis and treatment of the acute pancreatitis and obstructive jaundice due to rupture of hydatid cyst into the biliary tract. [Natl J Med Res 2014; 4(1.000: 92-94

  19. Acute Paraplegia due to Thoracic Hematomyelia

    Science.gov (United States)

    Celik, Bahattin; Canbek, Ihsan; Karavelioğlu, Ergun

    2016-01-01

    Spontaneous intraspinal intramedullary hemorrhage is a rare entity with the acute onset of neurologic symptoms. The etiology of idiopathic spontaneous hematomyelia (ISH) is unknown, and there are few published case reports. Hematomyelia is mostly associated with trauma, but the other nontraumatic etiologies are vascular malformations, tumors, bleeding disorders, syphilis, syrinx, and myelitis. MRI is a good choice for early diagnosis. Hematomyelia usually causes acute spinal cord syndrome due to the compression and destruction of the spinal cord. A high-dose steroid treatment and surgical decompression and evacuation of hematoma are the urgent solution methods. We present idiopathic spontaneous hematomyelia of a previously healthy 80-year-old male with a sudden onset of back pain and paraplegia.

  20. Acute hepatitis due to brucellosis: case report

    Directory of Open Access Journals (Sweden)

    Nevil AYKIN

    2009-06-01

    Full Text Available Brucella infection is a systemic disease. It rarely causes local infections like hepatitis in gastrointestinal system. In this article we would like to present an acute hepatitis case related to brucella infection that followed up in our clinic. A male, 30 year-old patient hospitalized due to common muscle pain, high fever and vomiting. During the physical examination the patient’s skin, scleras and mucosal membranes were icteric, the liver was 2-3 cm palpable and tender. Laboratory findings were as follows: AST:1190 U/L; ALT:715 U/L; GGT:961 U/L; ALP:369 U/L; total bilirubin:4.6 mg/dL; direct bilirubin:2.1 mg/dL. Viral markers were found to be negative. We started treatment with streptomicine and doxicycline since, the patient’s standard brucella tube agglutination test was positive (1/60 and brucella spp produced in his blood culture. From the second day of the treatment, we started to get clinical response. On the 17th day of the treatment, he discharged from the hospital because ALT, AST and bilirubine level were found normal and his treatment was completed to the 8 weeks. Brucella is continuing to be an important health problem especially who live in surrounding countryside and have to keep in mind in the differential diagnosis of the acute hepatitis.

  1. 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.

  2. Acute generalized exanthematous pustulosis due to tetrazepam.

    Science.gov (United States)

    Thomas, E; Bellón, T; Barranco, P; Padial, A; Tapia, B; Morel, E; Alves-Ferreira, J; Martín-Esteban, M

    2008-01-01

    Tetrazepam is a benzodiazepine that is widely used in Spain as a muscle relaxant, with occasional cutaneous side effects. We report a patient who developed a generalized pruriginous cutaneous reaction compatible with acute generalized exanthematous pustulosis (AGEP) due to tetrazepam. Patch tests with bromazepam, diazepam, and tetrazepam were negative at 48 and 72 hours; however, the tetrazepam patch showed a positive reaction at 10 days. Immunohistochemical studies revealed a mononuclear infiltrate composed of CD4+ and CD8+ T lymphocytes. Analysis of interleukin (IL) 8 expression by quantitative polymerase chain reaction revealed increased IL-8 mRNA levels in patch test-positive skin. Lymphoblast transformation test (LTT) was positive with tetrazepam but not with diazepam. Positive patch test and LTT suggested that tetrazepam-specific lymphocytes might be responsible for a T cell-mediated reaction. These results support previous data suggesting an important role for IL-8 and drug-specific T cells in the pathogenesis ofAGEP and imply that the reaction was specific to tetrazepam with no cross-reactivity to other benzodiazepines. PMID:18447141

  3. Use of a partially covered self-expandable metallic stent to treat a biliary stricture secondary to chronic pancreatitis complicated by recurrent cholangitis: a case report.

    Science.gov (United States)

    Okabe, Yoshinobu; Ishida, Yusuke; Sasaki, Yu; Ushijima, Tomoyuki; Sugiyama, Gen; Tsuruta, Osamu

    2012-05-01

    The patient was a 69 year old man who had been diagnosed with alcoholic chronic pancreatitis and lower common bile duct (CBD) stricture. He subsequently developed cholangitis 2-3 times a year, and we replaced the endoscopic biliary stent (EBS) each time. In April 2010, he was admitted because of complication by a liver abscess and acute cholangitis. We performed percutaneous transhepatic liver abscess drainage. The inflammatory findings then rapidly improved, but the patient developed acute cholangitis due to the sludge and the stones. Then, we placed a partially covered self-expandable metallic stent (C-SEMS) in the lower CBD and performed endoscopic lithotripsy through the C-SEMS, and the cholangitis subsequently improved. Two weeks after, we removed the C-SEMS endoscopically and replaced it with a 10 Fr plastic stent; since then there have been no recurrences of cholangitis. Our experience in this case suggested that when a plastic stent is placed long-term to treat a biliary stricture associated with chronic pancreatitis, it might be useful to also control biliary sludge and stones using a C-SEMS. PMID:22533753

  4. Acute Pancreatitis Due to Pravastatin Therapy

    Directory of Open Access Journals (Sweden)

    Anagnostopoulos GK

    2003-05-01

    Full Text Available CONTEXT: Few data exist about the incidence of drug-induced pancreatitis in the general population. Drugs are related to the etiology of pancreatitis in about 1.4-2% of cases. Statins are generally well tolerated. Acute pancreatitis has been reported in a few cases treated with atorvastatin, fluvastatin, lovastatin and simvastatin. CASE REPORT: We report the case of a 56-year-old patient who, after 6 months of treatment with pravastatin 20 mg once daily for hypercholesterolemia, presented with acute pancreatitis. Other causes of the disease were ruled out. Five months later, the patient, on his own initiative, reintroduced pravastatin and acute pancreatitis recurred after 3 days. CONCLUSION: To our knowledge this is the first report of pravastatin-induced pancreatitis and further strengthens the fact that statins may cause acute pancreatitis.

  5. Emergency thyroidectomy: Due to acute respiratory failure

    OpenAIRE

    Zulfu Bayhan; Sezgin Zeren; Bercis Imge Ucar; Isa Ozbay; Yalcin Sonmez; Metin Mestan; Onur Balaban; Nilufer Araz Bayhan; Mehmet Fatih Ekici

    2014-01-01

    INTRODUCTION: Giant cervical and mediastinal goiter may lead to acute respiratory failure caused by laryngotracheal compression and airway obstruction. Here, we present a case admitted to the emergency service with a giant goiter along with respiratory failure and poor general health status, which required urgent surgical intervention. PRESENTATION OF CASE: A 71-year-old female admitted to the emergency room with shortness of breath and poor general health status resulting from a giant cer...

  6. Acute Respiratory Distress Due to Methane Inhalation

    OpenAIRE

    Jo, Jun Yeon; Kwon, Yong Sik; Lee, Jin Wook; Park, Jae Seok; Rho, Byung Hak; Choi, Won-Il

    2013-01-01

    Inhalation of toxic gases can lead to pneumonitis. It has been known that methane gas intoxication causes loss of consciousness or asphyxia. There is, however, a paucity of information about acute pulmonary toxicity from methane gas inhalation. A 21-year-old man was presented with respiratory distress after an accidental exposure to methane gas for one minute. He came in with a drowsy mentality and hypoxemia. Mechanical ventilation was applied immediately. The patient's symptoms and chest rad...

  7. Gastric Necrosis due to Acute Massive Gastric Dilatation

    Directory of Open Access Journals (Sweden)

    Ibrahim Aydin

    2013-01-01

    Full Text Available Gastric necrosis due to acute massive gastric dilatation is relatively rare. Vascular reasons, herniation, volvulus, acute gastric dilatation, anorexia, and bulimia nervosa play a role in the etiology of the disease. Early diagnosis and treatment are highly important as the associated morbidity and mortality rates are high. In this case report, we present a case of gastric necrosis due to acute gastric dilatation accompanied with the relevant literature.

  8. Acute oral poisoning due to chloracetanilide herbicides.

    Science.gov (United States)

    Seok, Su-Jin; Choi, Sang-Cheon; Gil, Hyo-Wook; Yang, Jong-Oh; Lee, Eun-Young; Song, Ho-Yeon; Hong, Sae-Yong

    2012-02-01

    Chloracetanilide herbicides (alachlor, butachlor, metachlor) are used widely. Although there are much data about chronic low dose exposure to chloracetanilide in humans and animals, there are few data about acute chloracetanilide poisoning in humans. This study investigated the clinical feature of patients following acute oral exposure to chloracetanilide. We retrospectively reviewed the data on the patients who were admitted to two university hospitals from January 2006 to December 2010. Thirty-five patients were enrolled. Among them, 28, 5, and 2 cases of acute alachlor, metachlor, butachlor poisoning were included. The mean age was 49.8 ± 15.4 yr. The poison severity score (PSS) was 17 (48.6%), 10 (28.6%), 5 (14.3%), 2 (5.7%), and 1 (2.9%) patients with a PSS of 0, 1, 2, 3, and 4, respectively. The age was higher for the symptomatic patients (1-4 PSS) than that for the asymptomatic patients (0 PSS) (43.6 ± 15.2 vs 55.7 ± 13.5). The arterial blood HCO₃⁻ was lower in the symptomatic patients (1-4 PSS) than that in the asymptomatic patients (0 PSS). Three patients were a comatous. One patient died 24 hr after the exposure. In conclusion, although chloracetanilide poisoning is usually of low toxicity, elder patients with central nervous system symptoms should be closely monitored and cared after oral exposure.

  9. Acute Myocardial Infarction Due To Electrical Injury

    OpenAIRE

    Uzkeser M et al.

    2011-01-01

    Rhythm abnormalities (conduction defects, tachycardia, and arrhythmia)due to electric shock are common. Rarely, myocardial infarction may beseen in these patients. This situation is generally caused by coronaryartery vasospasm and direct myocardial damage. In this report, wepresent a rare case of myocardial infarction due to electric shock.

  10. Early maternal death due to acute encephalitis

    Directory of Open Access Journals (Sweden)

    M Vidanapathirana

    2014-03-01

    Full Text Available Maternal death in an unmarried woman poses a medico-legal challenge. A 24-year-old unmarried schoolteacher, residing at a boarding place, had been admitted to hospital in a state of cardiac arrest. At the autopsy, mild to moderate congestion of subarachnoid vessels and oedema of the brain was noted. An un-interfered foetus of 15 weeks with an intact sac and placental tissues were seen. Genital tract injuries were not present. Histopathological examination showed diffuse perivascular cuffing by mononuclear cells suggestive of viral encephalitis, considering the circumstances of death and the social stigma of pregnancy in this unmarried teacher, the possibility of attempted suicide by ingestion of a poison was considered. Abrus precatorius (olinda seeds commonly found in the area is known to produce acute encephalitis as well as haemorrhagic gastroenteritis and pulmonary congestion was also considered as a possible cause for this unusual presentation

  11. Cardiogenic shock due to acute tramadol intoxication.

    Science.gov (United States)

    Perdreau, Elodie; Iriart, Xavier; Mouton, Jean-Baptiste; Jalal, Zakaria; Thambo, Jean-Benoît

    2015-01-01

    Tramadol is a common analgesic, widely prescribed because of its efficiency and safety. We report the case of a 7-year-old child admitted in cardiac intensive care unit for cardiogenic shock due to tramadol intoxication. Without any past history, the child was admitted at emergency room for generalised convulsion, followed by respiratory distress. Cardiogenic shock was suspected after clinical examination and chest X-ray and confirmed by transthoracic echocardiography showing low left ventricular ejection fraction (1 mg/L) and O-desmethyltramadol (>1.5 mg/L). Hemodynamic support by inotropic drug infusion and diuretics was necessary. Left ventricular function normalised after 2 days of treatment allowing drug infusion weaning. Cardiac magnetic resonance imaging performed 3 days after admission confirmed normal left ventricular ejection fraction and volumes without evidence of late gadolinium enhancement. Cardiogenic shock due to tramadol intoxication is rare but exists. Negative inotropic effect of high doses of tramadol has been suspected. Quick recovery is possible.

  12. 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....

  13. 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...

  14. 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.

  15. 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.

  16. 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.

  17. Acute small bowel obstruction due to chicken bone bezoar

    Directory of Open Access Journals (Sweden)

    Vetpillai P

    2012-12-01

    Full Text Available Preadeepan Vetpillai,1 Ayo Oshowo21CT2 Surgery in General, Charing Cross Hospital, 2Colorectal and Laparoscopic Surgery, Whittington Hospital, London, UKAbstract: Acute intestinal obstruction due to foreign bodies, or bezoar, is a rare occurrence in an adult with a normal intestinal tract. We report an unusual case of a 43-year-old black man with no previous abdominal surgery and no significant medical history who presented with an acute episode of small bowel obstruction due to an impacted undigested chicken bone.Keywords: small bowel obstruction, chicken bone, bezoar

  18. 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.

  19. 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.

  20. Acute Suppurative Cholangitis Patients with Diabetes Mellitus Perioperative Nursing Care%急性化脓性胆管炎合并糖尿病患者的围手术期护理

    Institute of Scientific and Technical Information of China (English)

    尉晓菊

    2014-01-01

    Objective To construct new acute suppurative cholangitis in patients with diabetes mel itus perioperative nursing process,enrich the content of the liver and gal bladder surgery nursing, improve the nursing process. Methods:January 2012-January 2013 in our hospital in liver and gal bladder surgery for acute suppurative cholangitis requires data review procedure of 60 patients with diabetes,patients were randomly divided into group,30 cases in the control group 30 cases,the team with a new mode of nursing process,the control group given surgical routine care,wound healing and hospitalization days were observed. Results:the new nursing process group of patients in the general case, wound healing,are bet er than the routine nursing group, hospitalization days of the new nursing process group is (7.2+1.5)days,routine nursing group was (10.5+2.0)days. Conclusion:the new perioperative nursing process for acute suppurative cholangitis combined with diabetes with stable vital signs, the wound healed wel , the advantages of shorter hospitalization time,can be used in liver and gal bladder surgery to expand.%目的:构造新的急性化脓性胆管炎合并糖尿病患者的围手术期护理程序,充实肝胆外科护理内容,完善护理工作流程。方法选择2012年1月~2013年1月在我院肝胆外科因急性化脓性胆管炎需手术的糖尿病患者60例进行资料回顾,将患者随机分为研究组30例,对照组30例,研究组患者给予新模式的护理流程,对照组给予外科常规护理,观察患者伤口愈合情况以及住院天数。结果新护理流程组患者在一般情况,伤口愈合方面均优于常规护理组,住院天数方面新护理流程组为(7.2±1.5)d,常规护理组为(10.5±2.0)d。结论采用新的围手术期护理程序,对于急性化脓性胆管炎合并糖尿病患者来说,具有生命体征平稳,伤口愈合良好,住院时间短的优点,可以在肝胆外科扩大运用。

  1. 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....

  2. Fulminant hepatic failure (FHF) due to acute hepatitis C.

    Science.gov (United States)

    Younis, Bilal Bin; Arshad, Rozina; Khurhsid, Saima; Masood, Junaid; Nazir, Farhan; Tahira, Maham

    2015-01-01

    Acute hepatitis C (HCV) infection has been identified as an important cause of fulminant hepatic failure (FHF), characterized by rapid deterioration of liver function from massive hepatic necrosis leading to encephalopathy and multi-organ failure. We admitted a female patient at Shalamar Hospital with jaundice, fever, encephalopathy and coagulopathy of short duration with no history of any comorbidity. Her hepatitis viral screen revealed positive anti HCV. Her viral loads were also high. A diagnosis of FHF due to acute HCV infection was made. Patient was treated conservatively and improved gradually. In summary, acute HCV can cause FHF and should be ruled out in patients with FHF of unknown cause in an endemic country for HCV like Pakistan.

  3. 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.

  4. 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.

  5. [PRIMARY SCLEROSING CHOLANGITIS

    Science.gov (United States)

    Dantas, Waldomiro

    2000-01-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic and progressive disease, of unknown etiology, characterized by inflammation and fibrosis of intra-hepatic and extra-hepatic biliary tree. Two thirds of the patients have, simultaneously, ulcerative colitis (UC); on the other hand, PSC is the most common chronic hepatic disease in patients with inflammatory bowel disease (IBD). Patients who have both diseases simultaneously are prone to develop colorectal carcinoma and cholangiocarcinoma. The disease predominantly affects young men, may be asymptomatic or presented as fluctuating jaundice, pruritus and increased levels of the cholestasis biochemical markers, or as secondary billiary cirrhosis. The diagnostic gold standard are the cholangiographic abnormalities, consisting of multifocal stenosis and dilatations, involving both the intrahepatic and the extrahepatic biliary tree. Liver biopsy is useful only for staging the disease and to confirm the diagnosis, in the atypical forms. Ursodeoxycholic acid is a promising drug, nowadays, mainly in the first stages of the disease, in spite of doubtful efficacy. Combination therapy, using ursodeoxicolic acid, methotrexate and alternating antibiotics monthly may be sucessfull in PSC before bile strictures occur. Liver transplantation is the only life-saving therapeutic alternative, able to improve significantly the survival and the life quality of the patients. PMID:12140588

  6. Acute Pancreatitis and Splenic Vein Thrombosis due to Hypertriglyceridemia

    OpenAIRE

    Ercan Gündüz; Recep Dursun; Mustafa İçer; Yılmaz Zengin; Cahfer Güloğlu

    2015-01-01

    Acute pancreatitis (AP) is a condition characterised by the activation of the normally inactive digestive enzymes due to an etiological factor and digestion of the pancreatic tissues, resulting in extensive inflammation and leading to local, regional, and systemic complications in the organism. It may vary from the mild edematous to the hemorrhagic and severely necrotising form. The most common causes are biliary stones and alcohol abuse. In this case study, we would like to present a patient...

  7. Acute Anterolateral Myocardial Infarction Due to Aluminum Phosphide Poisoning

    OpenAIRE

    Bita Dadpour; Zohre Oghabian

    2013-01-01

    Aluminum phosphide (AlP) is a highly effective rodenticide which is used as a suicide poison. Herein, a 24 year-old man who’d intentionally ingested about 1liter of alcohol and one tablet of AlP is reported. Acute myocardial infarction due to AlP poisoning has been occurred secondary to AIP poisoning. Cardiovascular complications are poor prognostic factors in AlP poisoning

  8. Acute Anterolateral Myocardial Infarction Due to Aluminum Phosphide Poisoning

    Directory of Open Access Journals (Sweden)

    Bita Dadpour

    2013-08-01

    Full Text Available Aluminum phosphide (AlP is a highly effective rodenticide which is used as a suicide poison. Herein, a 24 year-old man who’d intentionally ingested about 1liter of alcohol and one tablet of AlP is reported. Acute myocardial infarction due to AlP poisoning has been occurred secondary to AIP poisoning. Cardiovascular complications are poor prognostic factors in AlP poisoning

  9. Acute Pancreatitis and Splenic Vein Thrombosis due to Hypertriglyceridemia

    Directory of Open Access Journals (Sweden)

    Ercan Gündüz

    2015-01-01

    Full Text Available Acute pancreatitis (AP is a condition characterised by the activation of the normally inactive digestive enzymes due to an etiological factor and digestion of the pancreatic tissues, resulting in extensive inflammation and leading to local, regional, and systemic complications in the organism. It may vary from the mild edematous to the hemorrhagic and severely necrotising form. The most common causes are biliary stones and alcohol abuse. In this case study, we would like to present a patient with AP due to hypertriglyceridemia (HPTG, which is a rare cause of pancreatitis, and splenic vein thrombosis, which is a rare complication of pancreatitis.

  10. Acute Pancreatitis and Splenic Vein Thrombosis due to Hypertriglyceridemia.

    Science.gov (United States)

    Gündüz, Ercan; Dursun, Recep; İçer, Mustafa; Zengin, Yılmaz; Güloğlu, Cahfer

    2015-01-01

    Acute pancreatitis (AP) is a condition characterised by the activation of the normally inactive digestive enzymes due to an etiological factor and digestion of the pancreatic tissues, resulting in extensive inflammation and leading to local, regional, and systemic complications in the organism. It may vary from the mild edematous to the hemorrhagic and severely necrotising form. The most common causes are biliary stones and alcohol abuse. In this case study, we would like to present a patient with AP due to hypertriglyceridemia (HPTG), which is a rare cause of pancreatitis, and splenic vein thrombosis, which is a rare complication of pancreatitis.

  11. Acute infarction of corpus callosum due to transient obstructive hydrocephalus.

    Science.gov (United States)

    Kaymakamzade, Bahar; Eker, Amber

    2016-01-01

    Acute ischemia of the corpus callosum (CC) is not a well-known feature in patients with acute hydrocephalus. Herein, we describe a case with acute CC infarction due to another rare entity; transient obstructive hydrocephalus. A 66-year-old male was admitted with sudden onset right-sided hemiparesia. CT demonstrated a hematoma on the left basal ganglia with extension to all ventricles. The following day, the patient's neurological status progressed to coma and developed bilateral pyramidal signs. MRI demonstrated obstructive hydrocephalus and acute diffuse infarction accompanied by elevation of the CC. On the same day there was improvement in his neurological status with significant decrease in ventricular size and complete resolution of the clot in the third ventricle. The mechanism of signal abnormalities is probably related with the neural compression of the CC against the falx. Presumably, the clot causing obstruction in the third ventricle dissolved or decayed by the help of fibrinolytic activity of CSF, which was raised after IVH and caused spontaneous improvement of hydrocephalus. Bilateral neurological symptoms suggest diffuse axonal damage and normalization of the intracranial pressure should be performed on the early onset of clinical detorioration in order to prevent axonal injury.

  12. 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.

  13. 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.

  14. Acute pulmonary alveolar proteinosis due to exposure to cotton dust

    Directory of Open Access Journals (Sweden)

    Thind Gurcharan

    2009-01-01

    Full Text Available Secondary pulmonary alveolar proteinosis (PAP is rare but may occur in association with malignancy, certain infections, and exposure to inorganic or organic dust and some toxic fumes. This case report describes the second recorded case of PAP due to exposure to cotton dust. A 24-year-old man developed PAP after working as a spinner for eight years without respiratory protection. He was admitted as an emergency patient with very severe dyspnea for four months and cough for several years. Chest X-ray showed bilateral diffuse alveolar consolidation. He died 16 days later, and a diagnosis of acute pulmonary alveolar proteinosis was made at autopsy. The histopathology demonstrated alveoli and respiratory bronchioles filled with characteristic periodic acid Schiff-positive material, which also revealed birefringent bodies of cotton dust under polarized light. Secondary PAP can be fatal and present with acute respiratory failure. The occupational history and characteristic pathology can alert clinicians to the diagnosis.

  15. Small Bowel Perforation due to Gossypiboma Caused Acute Abdomen

    Directory of Open Access Journals (Sweden)

    Tahsin Colak

    2013-01-01

    Full Text Available Gossypiboma, an infrequent surgical complication, is a mass lesion due to a retained surgical sponge surrounded by foreign body reaction. In this case report, we describe gossypiboma in the abdominal cavity which was detected 14 months after the hysterectomy due to acute abdominal pain. Gossypiboma was diagnosed by computed tomography (CT. The CT findings were a rounded mass with a dense central part and an enhancing wall. In explorative laparotomy, small bowel loops were seen to be perforated due to inflammation of long standing gossypiboma. Jejunal resection with end-to-end anastomosis was performed. The patient was discharged whithout complication. This case was presented to point to retained foreign body (RFB complications and we believed that the possibility of a retained foreign body should be considered in the differential diagnosis of who had previous surgery and complained of pain, infection, or palpable mass.

  16. 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

  17. Diagnostic criteria for acute liver failure due to Wilson disease

    Institute of Scientific and Technical Information of China (English)

    Christoph Eisenbach; Olivia Sieg; Wolfgang Stremmel; Jens Encke; Uta Merle

    2007-01-01

    AIM: To describe the diagnostic criteria for acute liver failure due to Wilson disease (WD), which is an uncommon cause of acute liver failure (ALF).METHODS: We compared findings of patients presenting with ALF due to WD to those with ALF of other etiologies.RESULTS: Previously described criteria, such as low alkaline phosphatase activity, ratio of low alkaline phosphatase to total bilirubin or ratio of high aspartate aminotransferase (AST) to alanine aminotransferase (ALT), failed to identify patients with ALF due to WD. There were significant differences in low ALT and AST activities (53 ± 43 vs 1982 ± 938, P < 0.0001 and 87 ± 44 vs 2756 ± 2941, P = 0.037, respectively), low choline esterase activity (1.79 ± 1.2 vs 4.30 ± 1.2, P = 0.009), high urine copper concentrations (93.4 ± 144.0 vs 3.5 ± 1.8, P = 0.001) and low hemoglobin (7.0 ± 2.2 vs 12.6 ± 1.8, P < 0.0001) in patients with ALF caused by WD as compared with other etiologies. Interestingly, 4 of 7 patients with ALF due to WD survived without liver transplantation.CONCLUSION: In ALF, these criteria can help establish a diagnosis of WD. Where applicable, slit-lamp examination for presence of Kayser-Fleischer rings and liver biopsy for determination of hepatic copper concentration still remain important for the diagnosis of ALF due to WD. The need for liver transplantation should be evaluated carefully as the prognosis is not necessarily fatal.

  18. 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.

  19. 重症急性胆管炎患者的死亡风险因素分析%Analysis of mortality risk factors for acute cholangitis of severse type

    Institute of Scientific and Technical Information of China (English)

    杜海伟; 秦鸣放; 王庆; 李湧; 曹占国

    2013-01-01

    目的 探讨重症急性胆管炎(ACST)患者的死亡风险因素,为临床及时诊治提供依据.方法 回顾性分析天津市南开医院微创外科中心2011年6月1日~ 2012年1月1日收治的61例ACST患者的一般资料、治疗方法及病情变化等,采用Wilcoxon秩和检验、Pearson x2检验对患者预后进行12个相关单因素分析,采用二分类Logistic线性回归行死亡风险多因素分析.结果 61例ACST患者中9例死亡.单因素分析显示,白细胞计数、中性粒细胞百分比升高、血小板减少、休克、呼吸衰竭、意识障碍是ACST患者的死亡危险因素;多因素分析显示,影响ACST患者预后因素的强度按风险系数排列依次为意识障碍、休克、血小板计数≤75×109/L、呼吸衰竭、中性粒细胞百分比>90%.结论 意识障碍、休克、血小板计数≤75×109/L、呼吸衰竭、中性粒细胞百分比>90%是ACST患者的死亡风险因素;急诊鼻胆管引流(ENBD)可作为ACST明确诊断后的首选治疗方法.%Objective To explore the mortality risk factors of the severe acute cholangitis (acute cholangitis of severse type, ACST) and to provide the evidence for clinical therapy. Methods Analyzed retrospectively the general conditions, therapeutic method and clinical changes of 61 patients treated in Nankai Hospital Minimally Invasive Surgery Center of Tianjin during the 1st June 2011 to the 1st January 2012, the 12 relative factors were analyzed by Wilcoxon rank sum test and Pearson x2 test and followed by multivariate analysis using Logistic regression model for death factors. Results There were nine death cases in 61 patients with ACST. The univariate analysis display that white blood cell count, neutrophil percentage increased, thrombocytopenia, shock, respiratory failure, disturbance of consciousness were ACST patients risk factors from the 12 risk factors, and multivariate analysis from these risk factors showed that the risk coefficient

  20. Acute kidney injury and rhabdomyolysis due to multiple wasp stings

    OpenAIRE

    Hemachandar Radhakrishnan

    2014-01-01

    In most patients, wasp stings cause local reactions and rarely anaphylaxis. Acute kidney injury and rhabdomyolysis are unusual complications of wasp stings. We report a case of acute kidney injury and rhabdomyolysis secondary to multiple wasp stings. A 55-year-old farmer developed multi organ dysfunction with acute kidney injury and rhabdomyolysis 3 days after he had sustained multiple wasp stings. The etiology of acute kidney injury is probably both rhabdomyolysis and acute tubular necrosis....

  1. Acute metheamoglobinemia due to nitrobenzene poisoning: Case series

    Directory of Open Access Journals (Sweden)

    Harish Kumar S, Ujjawal Kumar, Raghavendra Prasad B N, Kiran BJ, Anil Kumar M

    2014-04-01

    Full Text Available Nitrobenzene is a nitrite compound; its toxic effects are due to its ability to convert hemoglobin to methaemoglobin by oxidizing iron. The clinical features of nitrobenzene poisoning vary based on the concentration of methaemoglobin in blood. Immediate identification based on clinical features, odour of the compound with supporting evidence of increased methaemoglobin levels will help in a timely intervention thus preventing fatal outcome. Early haemodynamic and ventilator support along with administration of methylene blue as an antidote has been proved crucial in saving some lives. An acute nitrobenzene poisoning presenting with methaemoglobinemia is becoming quite common in this part of the country. Here authorsreport a series of cases of nitrobenzene poisoning where immediate clinical evaluation, with repeated intravenous methylene blue saved three patients, but two patients presenting late and with heavy exposure could not be saved

  2. A rare presentation of hypopituitarism in hepatic overlap syndrome of autoimmune hepatitis and autoimmune cholangitis

    OpenAIRE

    Gupta V; Singh H.; Talapatra P; Ray S

    2016-01-01

    Autoimmune cholangitis is the antimitochondrial antibody-negative autoimmune hepatopathy with clinical and histological features similar to that of primary biliary cirrhosis. Autoimmune cholangitis has a predominant cholestatic phase. However, transaminasemia might be dominant in certain patients, indicating associated autoimmune hepatitis. Such an autoimmune hepatopathy has been termed as hepatic overlap syndrome. Due to the autoimmune nature of the disease, associated diseases of other orga...

  3. Severe acute caffeine poisoning due to intradermal injections: Mesotherapy hazard

    Directory of Open Access Journals (Sweden)

    Perković-Vukčević Nataša

    2012-01-01

    Full Text Available Introduction. Caffeine is indicated in the treatment of migraine headaches, as well as neonatal apnea and bradycardia syndrome. In mild poisoning, the most prevalent symptoms are nausea, vomiting, diarrhea, tremor, anxiety and headache. In more severe cases, symptoms consist of heart rythym abnormalities, myocardial infarction and seizures. Due to its common lipolytic effect, caffeine is used in mesotherapy, usually in combination with drugs of similar effect. We presented a patient with acute iatrogenic caffeine poisoning. Case report. A 51-year-old woman, with preexisting hypertension and hypertensive cardiomyopathy was subjected to cosmetic treatment in order to remove fat by intradermal caffeine injections. During the treatment the patient felt sickness, an urge to vomit, and a pronounced deterioration of general condition. Upon examination, the patient exhibited somnolence, hypotension and nonsustained ventricular tachycardia, which was sufficient enough evidence for further hospitalization. On admission to the intensive care unit the patient was anxious with increased heart rate, normotensive, with cold, damp skin, and visible traces of injection sites with surrounding hematomas on the anterior abdominal wall. Paroxysmal supraventricular tachycardia (PSVT on electrocardiographic monitoring was found. The laboratory analysis determined a lowered potassium level of 2.1 mmol/L (normal range 3,5 - 5.2 mmol/L, and a toxicological analysis (liquid chromatography with ultraviolet detection proved a toxic concentration of caffeine in plasma - 85.03 mg/L (toxic concentration over 25 mg/L. On application of intensive therapy, antiarrhythmics, and substitution of potassium, as well as both symptomatic and supportive therapy, there was a significant recovery. The patient was discharged without any sequele within four days. Conclusion. A presented rare iatrogenic acute caffeine poisoning occured due to massive absorption of caffeine from the

  4. Acute isolated appendicitis due to Aspergillus carneus in a neutropenic child with acute myeloid leukemia.

    Science.gov (United States)

    Decembrino, Nunzia; Zecca, Marco; Tortorano, Anna Maria; Mangione, Francesca; Lallitto, Fabiola; Introzzi, Francesca; Bergami, Elena; Marone, Piero; Tamarozzi, Francesca; Cavanna, Caterina

    2016-01-01

    We describe a case of isolated acute appendicitis due to Aspergillus carneus in a neutropenic child with acute myeloid leukemia (AML) treated according to the AIEOP AML 2002/01 protocol. Despite prophylaxis with acyclovir, ciprofloxacin and fluconazole administered during the neutropenic phase, 16 days after the end of chemotherapy the child developed fever without identified infective foci, which prompted a therapy shift to meropenem and liposomial amphotericin B. After five days of persisting fever he developed ingravescent abdominal lower right quadrant pain. Abdominal ultrasound was consistent with acute appendicitis and he underwent appendectomy with prompt defervescence. PAS+ fungal elements were found at histopathology examination of the resected vermiform appendix, and galactomannan was low positive. A. carneus, a rare species of Aspergillus formerly placed in section Flavipedes and recently considered a member of section Terrei, was identified in the specimen. Treatment with voriconazole was promptly started with success. No other site of Aspergillus localization was detected. Appendicitis is rarely caused by fungal organisms and isolated intestinal aspergillosis without pulmonary infection is unusual. To our knowledge, this is the first report of infection due to A. carneus in a child and in a primary gastrointestinal infection.

  5. 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...

  6. Acute toxemic schistosomiasis complicated by a acute flaccid paraplegia due to schistosomal myeloradiculopathy in Sudan

    International Nuclear Information System (INIS)

    A 55-year old Sudanese physician presented with one month history of diarrhea, loss of weight 10 kg and low grade nocturnal fever. Following colonoscopy, he rapidly developed paraparesis and retention of urine. Magnetic resonance imaging (MRI) of the spinal cord showed low cord lesion suggestive of transverse myelitis. We present a detailed account of diagnostic and management challenges and a literature review of the final diagnosis of acute toxemic schistosomiasis, complicated by acute flaccid paraplegia due to schistosomal myeloradiculopathy. We are reporting this case to increase the awareness of physicians of schistosomal myeloradiculopathy, as it needs urgent specific treatment praziquantel and steroids. An early follow-up with MRI of the spinal cord 2 weeks treatment may help in preventing unnecessary neurosurgical intervention. Bilharziasis may be contracted on the banks of river White Nile in urban areas. Finally clinicians should make use of the Google search for diagnosis in difficult cases. (author)

  7. An Acute Disseminated Encephalomyelitis Case due to Mycobacterium tuberculosis

    Directory of Open Access Journals (Sweden)

    Hale Turan Özden

    2016-03-01

    Full Text Available Acute disseminated encephalomyelitis (ADEM is an inflammatory and demyelinating disorder of the central nervous system that is characterized by multifocal involvement of the white matter. Our patient was a 27-year-old female patient who had given birth to a baby with caesarean in another hospital. After four days upon the parturition, she was admitted to our hospital’s general intensive care unit with a poor general status, confusion and a fever. She was diagnosed with ADEM according to the clinical, laboratory and radiological findings. In addition to her antibiotic treatment (meropenem that had been given in the previous health care facility, corticosteroid therapy was also started. The patient passed away due to the ventilator-associated pneumonia infection on the 10th day of her admission. Mycobacterium tuberculosis proliferation was observed in the cerebrospinal fluid after her death. As it is reported in literature, tuberculosis is a rare cause of ADEM. In conclusion, it should be noted that M. tuberculosis can be a rare cause of ADEM in regions where the disease is endemic. J Microbiol Infect Dis 2016;6(1: 28-31

  8. Acute Dystonia Due to Citalopram Treatment: A Case Series

    OpenAIRE

    Moosavi, S. Mohammad; Ahmadi, Mahshid; Monajemi, Mani B.

    2014-01-01

    Introduction: Abnormal movements such as acute dystonia, dyskinesia, parkinsonism, exacerbation of Parkinson disease, akathisia and possibly neuroleptic malignant syndrome may be associated with the use of selective serotonin reuptake inhibitors (SSRIs) rarely. Citalopram, a typical SSRI, used in serotonergic dysfunction related disorders, potentially can cause extrapyramidal symptoms such as acute dystonia. Methods: In a retrospective survey on patients referred to psychiatric clinic between...

  9. Acute Generalized Exanthematous Pustulosis Due to Insect Bites?

    OpenAIRE

    Bhat, Yasmeen J; Iffat Hassan; Peerzada Sajad; Atiya Yaseen; Rohi Wani

    2015-01-01

    Acute generalized exanthematous pustulosis is a rare severe cutaneous adverse reaction pattern that is mostly caused by the intake of drugs and rarely associated with viral infections, food allergens or toxins. Here we present the report of three patients who got admitted in our hospital for generalized pustulosis and fever after insect bites. The diagnosis of acute generalized exanthematous pustulosis was made by EuroSCAR scoring. The drug etiology was excluded and spider bite was implicated...

  10. Endoscopic treatment of severe acute cholangitis accompanied with multiple organ dysfunction syndrome%伴发多器官功能不全综合征的重症急性胆管炎的内镜治疗

    Institute of Scientific and Technical Information of China (English)

    杨波; 麻树人; 周文平; 袁旭东; 张宁

    2009-01-01

    Objective To evaluate the endoscopic managements of acute cholangitis of severe type (ACST) accompanied with multiple organ dysfunction syndrome (MODS). Methods A total of 122 ACST patients accompanied with MODS from January 2000 to October 2008 underwent endoscopic treatment in two time periods. In critical phase, emergent endoscopic retrograde cholangiopancreatography (ERCP) plus en-doscopic naso-biliary drainage (ENBD) were performed to correct critical situation of the patients. After sta-bilization, endoscopic sphincterotomy (EST) plus stone removal, EST plus stent placement, or laparoscopy was performed according to the causes of ACST. Results Emergent endoscopic managements succeeded in all patients of critical phase. At third day post-operation, a reduction in white blood cell count, serum total bilirubin, body temperature, and rate of patients with shock, mental symptoms and purulent bile juice was a-chieved. Recovery rate of dysfunction organs was 60.2% at one week after emergent procedure, and 82. 6% at 2 weeks post-operation. Selective EST plus stone removal was performed in 36 patients with a success rate n one session at 91.7%. Laparoscopic cholecystectomy was performed in 85 patients with a success rate of 95.3%. Stent was placed in 16 patients with an effective rate of 81.3% at 3 months post the procedure. No severe complication or death occurred during the whole therapeutic course. The 6-month survival rate of 10 cancer cases was 70%. Conclusion Therapeutic ERCP plus ENBD is the first choice for acute severe cholangitis accompanied with MODS, while EST plus biliary lithotomy, or EST plus stent placement, or com-bined laparoscopy are ideal methods for subsequent treatment.%目的 探讨伴发多器官功能不全综合征(MODS)的重症急性胆管炎(ACST)患者的内镜治疗价值.方法 对2000年1月-2008年10月期间122例伴发多器官功能不全综合征的ACST病例,分两个阶段进行内镜治疗.危重期以挽救患者生命为目

  11. 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.

  12. Acute Renal Failure due to Non-Traumatic Rhabdomyolysis

    Directory of Open Access Journals (Sweden)

    Nagehan Aslan

    2016-04-01

    Full Text Available Rhabdomyolysis is a musculoskeletal clinical and biochemical syndrome which is seen associated with traumatic and non-traumatic causes and is known as muscular dystrophy. Rhabdomyolysis which develops following crush-type trauma (Crush syndrome is rarely seen but is a well-known clinical event in the etiology of acute renal failure. Non-traumatic rhabdomyolysis is rare. The case is here presented of a patient who was diagnosed with rhabdomyolysis on presentation with acute renal failure and to whom repeated dialysis was applied.

  13. Sudden psychotic episode probably due to meningoencephalitis and Chlamydia pneumoniae acute infection

    OpenAIRE

    Canas Nuno; Coromina Marta; Correa Bernardo; Xavier Miguel; Guimarães João

    2005-01-01

    Abstract Background Since 9% to 20% of all cases of acute psychosis presenting to an Emergency Department (ED) are due to a general medical condition, cautious medical workup should be mandatory in such patients. Differential diagnosis must consider conditions as diverse as renal failure or CNS infection. Acute Chlamydia pneumoniae infection usually causes a self-limited respiratory syndrome. Rarely, acute neurological complications occur, with acute meningoencephalitis most frequently report...

  14. Acute Carotid Artery Stent Thrombosis Due to Dual Antiplatelet Resistance

    Energy Technology Data Exchange (ETDEWEB)

    Köklü, Erkan, E-mail: drerkankoklu@gmail.com; Arslan, Şakir; Yüksel, İsa Öner; Bayar, Nermin [Antalya Education and Research Hospital, Clinic of Cardiology (Turkey); Koç, Pınar [Antalya Education and Research Hospital, Clinic of Radiology (Turkey)

    2015-08-15

    Carotid artery stenting (CAS) is a revascularization modality that is an alternative to carotid endarterectomy. The efficacy of CAS in primary and secondary prevention from ischemic stroke has been demonstrated in various trials. Acute thrombosis of CAS is a rare complication that can lead to dramatic and catastrophic consequences. We discuss a case of acute CAS thrombosis in a patient who had previously undergone successful CAS. CAS was performed in a 73-year-old man who had had dysarthria lasting 2 weeks with 95 % stenosis in his left internal carotid artery. An acute cerebrovascular event resulting in right-sided hemiplegia developed 24 h after the procedure. Computed tomographic carotid angiography revealed complete occlusion of the stent with thrombus. The cause of stent thrombosis was thought to be antiaggregant resistance to both acetylsalicylic acid and clopidogrel. The most important cause of acute CAS thrombosis is inadequate or ineffective antiaggregant therapy. Evaluating patients who are candidates for CAS for acetylsalicylic acid and clopidogrel resistance may preclude this complication.

  15. Acute Carotid Artery Stent Thrombosis Due to Dual Antiplatelet Resistance

    International Nuclear Information System (INIS)

    Carotid artery stenting (CAS) is a revascularization modality that is an alternative to carotid endarterectomy. The efficacy of CAS in primary and secondary prevention from ischemic stroke has been demonstrated in various trials. Acute thrombosis of CAS is a rare complication that can lead to dramatic and catastrophic consequences. We discuss a case of acute CAS thrombosis in a patient who had previously undergone successful CAS. CAS was performed in a 73-year-old man who had had dysarthria lasting 2 weeks with 95 % stenosis in his left internal carotid artery. An acute cerebrovascular event resulting in right-sided hemiplegia developed 24 h after the procedure. Computed tomographic carotid angiography revealed complete occlusion of the stent with thrombus. The cause of stent thrombosis was thought to be antiaggregant resistance to both acetylsalicylic acid and clopidogrel. The most important cause of acute CAS thrombosis is inadequate or ineffective antiaggregant therapy. Evaluating patients who are candidates for CAS for acetylsalicylic acid and clopidogrel resistance may preclude this complication

  16. 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.

  17. Probable acute disseminated encephalomyelitis due to Haemophilus influenzae meningitis.

    Science.gov (United States)

    Beleza, Pedro; Ribeiro, Manuel; Pereira, João; Ferreira, Carla; Jordão, Maria José; Almeida, Fátima

    2008-05-01

    We report the case of a 17-year-old male on long-term steroid therapy for minimal lesion glomerulopathy who, after an upper respiratory infection, presented with Haemophilus influenzae type b meningitis. Twenty-four hours later he developed depression of consciousness which progressed to coma and left hemiparesis. Brain magnetic resonance imaging (MRI) revealed multiple lesions (hyperintense on T2 and slightly hypointense on Tl) involving mainly white matter suggestive of inflammation. MRI features were compatible with acute disseminated encephalomyelitis (ADEM), although a differential diagnosis included cerebritis or vasculitis, secondary to bacterial meningitis. The patient was treated with high-dose steroids which resulted in a gradual improvement followed by complete clinical recovery. We propose a diagnosis of ADEM was the best diagnosis because of the radiological features and response to steroids. The occurrence of ADEM associated with acute meningitis, however rare, represents an important diagnostic challenge for the clinician. PMID:18355336

  18. 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.

  19. 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.

  20. Acute Liver Toxicity due to Efavirenz/Emtricitabine/Tenofovir

    Directory of Open Access Journals (Sweden)

    Rashmee Patil

    2015-01-01

    Full Text Available The fixed-dose combination of Efavirenz/Emtricitabine/Tenofovir is a first-line agent for the treatment of HIV; however few cases have reported hepatotoxicity associated with the drug. We report a case of Efavirenz/Emtricitabine/Tenofovir-associated hepatotoxicity presenting mainly with hepatocellular injury characterized by extremely elevated aminotransferase levels, which resolved without acute liver failure or need for liver transplant referral.

  1. Death due to acute tetrachloroethylene intoxication in a chronic abuser.

    Science.gov (United States)

    Amadasi, Alberto; Mastroluca, Lavinia; Marasciuolo, Laura; Caligara, Marina; Sironi, Luca; Gentile, Guendalina; Zoja, Riccardo

    2015-05-01

    Volatile substances are used widespread, especially among young people, as a cheap and easily accessible drug. Tetrachloroethylene is one of the solvents exerting effects on the central nervous system with experiences of disinhibition and euphoria. The case presented is that of a 27-year-old female, found dead by her father at home with cotton swabs dipped in the nostrils. She was already known for this type of abuse and previously admitted twice to the hospital for nonfatal acute poisonings. The swabs were still soaked in tetrachloroethylene. Toxicological and histological investigations demonstrated the presence of an overlap between chronic intake of the substance (with high concentrations in sites of accumulation, e.g., the adipose tissue, and contemporary tissue damage, as histologically highlighted) and acute intoxication as final cause of death, with a concentration of 158 mg/L in cardiac blood and 4915 mg/kg in the adipose tissue. No other drugs or medicines were detected in body fluids or tissues, and to our knowledge, this is the highest concentration ever detected in forensic cases. This peculiar case confirms the toxicity of this substance and focuses on the importance of complete histological and toxicological investigations in the distinction between chronic abuse and acute intoxication.

  2. Acute respiratory failure due to ehrlichiosis - CT findings: case report

    International Nuclear Information System (INIS)

    Ehrlichiosis is a rare disease, with approximately 400 cases having been documented in the US since its recognition in 1986. Most of the reported cases were in the southeastern US, although 6 cases have been described in Washington state. Although most of these reported patients were admitted to hospital, severe complications developed in only a small proportion. Findings on chest imaging have been described in 3 children. To our knowledge, this is the first reported case of computed tomographic (CT) findings in a young adult with erhlichiosis in whom acute respiratory failure developed. (author)

  3. Spontaneous Uterine Perforation due to Pyometra Presenting as Acute Abdomen

    Directory of Open Access Journals (Sweden)

    Loabat Geranpayeh

    2006-01-01

    Full Text Available Spontaneous perforation of the uterus is rare, its incidence being about 0.01%–0.05%. We report a rare case of diffuse peritonitis caused by spontaneously perforated pyometra. A 63-year-old woman with severe abdominal pain was admitted to our hospital. Laparotomy was performed because of the suspicion of gastrointestinal perforation with generalized peritonitis. At laparotomy, about 900 mL of pus was found in the peritoneal cavity. There were no abnormal findings in the alimentary tract, liver, or gallbladder. A total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. Pathological investigation of the surgical specimen revealed endometritis and myometritis of the uterus; but there was no evidence of malignancy, and the cervical canal was patent. Although spontaneously perforated pyometra is rare, a perforated pyometra should therefore also be considered when elderly women present with acute abdominal pain.

  4. Acute Otitis due to Vibrio fluvialis after Swimming

    Directory of Open Access Journals (Sweden)

    Ping-Jen Chen

    2012-01-01

    Full Text Available A 40-year-old female presented with purulent exudate through the left auditive duct and pain in the left ear region, which intensified during mastication. After collection of the pus from the left ear lesion, amoxicillin-clavulanic acid for seven days was prescribed for a presumed diagnosis of acute otitis. Four days later, the pus culture grew V. fluvialis which is further identified by API 20E identification system (bioMérieux. Following the successful completion of a course of antibiotics, the patient recovered completely and without complication. To the best of our knowledge, this is the first case of Vibrio fluvialis otitis after swimming in an immunocompetent patient.

  5. Pulmonary hypertension due to acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    S.A. Ñamendys-Silva

    2014-10-01

    Full Text Available Our aims were to describe the prevalence of pulmonary hypertension in patients with acute respiratory distress syndrome (ARDS, to characterize their hemodynamic cardiopulmonary profiles, and to correlate these parameters with outcome. All consecutive patients over 16 years of age who were in the intensive care unit with a diagnosis of ARDS and an in situ pulmonary artery catheter for hemodynamic monitoring were studied. Pulmonary hypertension was diagnosed when the mean pulmonary artery pressure was >25 mmHg at rest with a pulmonary artery occlusion pressure or left atrial pressure <15 mmHg. During the study period, 30 of 402 critically ill patients (7.46% who were admitted to the ICU fulfilled the criteria for ARDS. Of the 30 patients with ARDS, 14 met the criteria for pulmonary hypertension, a prevalence of 46.6% (95% CI; 28-66%. The most common cause of ARDS was pneumonia (56.3%. The overall mortality was 36.6% and was similar in patients with and without pulmonary hypertension. Differences in patients' hemodynamic profiles were influenced by the presence of pulmonary hypertension. The levels of positive end-expiratory pressure and peak pressure were higher in patients with pulmonary hypertension, and the PaCO2 was higher in those who died. The level of airway pressure seemed to influence the onset of pulmonary hypertension. Survival was determined by the severity of organ failure at admission to the intensive care unit.

  6. Acute skin lesions due to localized ``hot particle`` radiation exposures

    Energy Technology Data Exchange (ETDEWEB)

    Baum, J.W.; Carsten, A.L.; Kaurin, D.G.L.; Schaefer, C.W.

    1996-06-01

    Purpose of the studies was to determine incidence and severity of lesions resulting from localized deposition of dose to the skin from small (<0.5 mm) discrete radioactive particles. Hanford mini-swine were exposed to localized doses from 0.2 to over 600 Gy (averaged over 1 cm{sup 2} at 70{mu}m depth) from isotopes having max beta particle energies from about 0.3-3 MeV. Incidence of erythema and scabs (indicating ulceration) were scored routinely for up to 71 days post-irradiation. Responses followed normal probability distributions, and thus, no true threshold could be defined. Ten and 50% incidence rates were deduced using probit analyses. Lowest dose producing 10% incidence was about 1 Gy for exposures to Yb-175 (0.5 MeV max energy) beta particles. Severity of lesions was estimated using diameters and persistence. From preliminary considerations of probability of induction, size, and persistence of acute lesions, a special limit for hot particle exposures in the range of 5-50 Gy may be reasonable, with an action level between about 1 Gy and the limit.

  7. A rare case of acute renal infarction due to idiopathic renal arterial thrombosis

    Institute of Scientific and Technical Information of China (English)

    FU Zhi-fang; ZHANG Zhi-gang; LIU Xin-min

    2008-01-01

    @@ Because of the lack of specific clinical signs and symptoms,acute renal infarction is apt to be missed in diagnosis and hence has a"low"incidence.In this report,a case of acute renal infarction due to idiopathic renal arterial thrombosis is presented.

  8. [Risk of acute hepatic insufficiency in children due to chronic accidental overdose of paracetamol (acetaminophen)

    NARCIS (Netherlands)

    Hameleers-Snijders, P.; Hogeveen, M.; Smeitink, J.A.M.; Kramers, C.; Draaisma, J.M.T.

    2007-01-01

    Two girls aged 4 and 3 years, respectively, experienced acute liver failure due to accidental ingestion of supratherapeutic doses of paracetamol (90 mg/kg/day or more). Recognition of chronic paracetamol intoxication as a cause of acute hepatic failure is often delayed. It is important to consider t

  9. Factors associated with emergency department visits due to acute asthma

    Directory of Open Access Journals (Sweden)

    Dalcin P.T.R.

    2004-01-01

    Full Text Available It is important to identify characteristics related to poor disease control and frequent visits to the emergency department (ED. The objective of the present study was to compare the characteristics of patients attending the adult ED for treatment of asthma exacerbation with those attending an asthma specialist clinic (AC in the same hospital, and to determine the factors associated with frequent visits to the ED. We conducted a cross-sectional survey of consecutive patients (12 years and older attending the ED (N = 86 and the AC (N = 86. Significantly more ED patients than AC patients reported ED visits in the past year (95.3 vs 48.8%; P < 0.001 and had difficulty performing work (81.4 vs 49.4%; P < 0.001. Significantly more AC than ED patents had been treated with inhaled corticosteroids (75.6 vs 18.6%; P < 0.001 used to increase or start steroid therapy when an attack was perceived (46.5 vs 20.9%; P < 0.001 and correctly used a metered-dose inhaler (50.0 vs 11.6%; P < 0.001. The history of hospital admissions (odds ratio, OR, 4.00 and use of inhaled corticosteroids (OR, 0.27 were associated with frequent visits to the ED. In conclusion, ED patients were more likely than AC patients to be dependent on the acute use of the ED, were significantly less knowledgeable about asthma management and were more likely to suffer more severe disease. ED patients should be considered an important target for asthma education. Facilitating the access to ambulatory care facilities might serve to reduce asthma morbidity.

  10. Acute Psychotic Symptoms due to Benzydamine Hydrochloride Abuse with Alcohol

    Directory of Open Access Journals (Sweden)

    Yahya Ayhan Acar

    2014-01-01

    Full Text Available Benzydamine hydrochloride is a locally acting nonsteroidal anti-inflammatory drug. Benzydamine hydrochloride overdose can cause stimulation of central nervous system, hallucinations, and psychosis. We presented a young man with psychotic symptoms due to benzydamine hydrochloride abuse. He received a total dose of 1000 mg benzydamine hydrochloride with alcohol for its hallucinative effects. Misuse of benzydamine hydrochloride must be considered in differential diagnosis of first-episode psychosis and physicians should consider possibility of abuse in prescribing.

  11. Acute Psychotic Symptoms due to Benzydamine Hydrochloride Abuse with Alcohol

    OpenAIRE

    Yahya Ayhan Acar; Mustafa Kalkan; Rıdvan Çetin; Erdem Çevik; Orhan Çınar

    2014-01-01

    Benzydamine hydrochloride is a locally acting nonsteroidal anti-inflammatory drug. Benzydamine hydrochloride overdose can cause stimulation of central nervous system, hallucinations, and psychosis. We presented a young man with psychotic symptoms due to benzydamine hydrochloride abuse. He received a total dose of 1000 mg benzydamine hydrochloride with alcohol for its hallucinative effects. Misuse of benzydamine hydrochloride must be considered in differential diagnosis of first-episode psycho...

  12. Acute pulmonary alveolar proteinosis due to exposure to cotton dust

    OpenAIRE

    Thind Gurcharan

    2009-01-01

    Secondary pulmonary alveolar proteinosis (PAP) is rare but may occur in association with malignancy, certain infections, and exposure to inorganic or organic dust and some toxic fumes. This case report describes the second recorded case of PAP due to exposure to cotton dust. A 24-year-old man developed PAP after working as a spinner for eight years without respiratory protection. He was admitted as an emergency patient with very severe dyspnea for four months and cough for several years. Ches...

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

    Energy Technology Data Exchange (ETDEWEB)

    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

  14. Acute colonic obstruction due to benign prostatic hypertrophy.

    LENUS (Irish Health Repository)

    Mac Giobuin, S

    2012-02-01

    A seventy two year old man presented to the Emergency Department with clinical features of colonic obstruction. Subsequent radiological investigations confirmed this impression and revealed the aetiology to be compression of the sigmoid colon against the sacrum by a massively distended urinary bladder. Chronic urinary retention due to benign prostatic hypertrophy is an extremely unusual cause of large bowel obstruction. Little in this patient\\'s clinical findings suggested this aetiology. We reviewed the literature in this area and highlight the benefits of CT scanning over contrast studies.

  15. 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.

  16. Effectiveness of Thrombolytic Therapy in Acute Embolic Stroke due to Infective Endocarditis

    OpenAIRE

    Sontineni, Siva P.; Mooss, Aryan N.; Andukuri, Venkata G.; Susan Marie Schima; Dennis Esterbrooks

    2009-01-01

    Objective. To identify the role of thrombolytic therapy in acute embolic stroke due to infective endocarditis. Design. Case report. Setting. University hospital. Patient. A 70-year-old male presented with acute onset aphasia and hemiparesis due to infective endocarditis. His head computerized tomographic scan revealed left parietal sulcal effacement. He was given intravenous tissue plasminogen activator with significant resolution of the neurologic deficits without complications. Main Outcome...

  17. Acute oxalate nephropathy due to ‘Averrhoa bilimbi’ fruit juice ingestion

    OpenAIRE

    Bakul, G.; Unni, V. N.; Seethaleksmy, N. V.; A Mathew; Rajesh, R; Kurien, G.; J. RAJESH; Jayaraj, P. M.; Kishore, D. S.; Jose, P. P.

    2013-01-01

    Irumban puli (Averrhoa bilimbi) is commonly used as a traditional remedy in the state of Kerala. Freshly made concentrated juice has a very high oxalic acid content and consumption carries a high risk of developing acute renal failure (ARF) by deposition of calcium oxalate crystals in renal tubules. Acute oxalate nephropathy (AON) due to secondary oxalosis after consumption of Irumban puli juice is uncommon. AON due to A. bilimbi has not been reported before. We present a series of ten patien...

  18. 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.

  19. 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.

  20. A Case with Repeated Recurrent Acute Coronary Syndrome due to Pseudoephedrine Use: Kounis Syndrome

    Directory of Open Access Journals (Sweden)

    Metin Çeliker

    2014-01-01

    Full Text Available Allergic reaction-associated acute coronary syndrome picture is defined as Kounis syndrome. Although drug use is the most common cause of allergic reaction, foods and environmental factors may also play a role in the etiology. Herein, a case with acute coronary syndrome that developed two times at 8-month interval due to pseudoephedrine use for upper respiratory tract infection is presented.

  1. Acute Respiratory Failure due to Neuromyelitis Optica Treated Successfully with Plasmapheresis

    Directory of Open Access Journals (Sweden)

    Massa Zantah

    2016-01-01

    Full Text Available Neuromyelitis Optica (NMO is a demyelinating autoimmune disease involving the central nervous system. Acute respiratory failure from cervical myelitis due to NMO is known to occur but is uncommon in monophasic disease and is treated with high dose steroids. We report a case of a patient with NMO who developed acute respiratory failure related to cervical spinal cord involvement, refractory to pulse dose steroid therapy, which resolved with plasmapheresis.

  2. A case of acute psychosis due to vitamin b12 deficiency

    OpenAIRE

    Sayın, Selim; Arslan, Erol; Demirbaş, Şeref; ESER, Mithat; Kenan SAĞLAM

    2014-01-01

    Patients with vitamin B12 deficiency may present with hematologic, neuropsychiatric and cardiovascular symptoms. A clinical Picture of acute psychosis can be seen in rare cases and if not corrected, vitamin B12 deficiency can lead to permanent demage. Acute psychosis and symptoms of anemia with sixty-year-old male patient was evaluated and found that vitamin B12 deficiency due to the terminal ileitis. The patient was healed without permanent damage with replacement therapy.The diagnosis and t...

  3. Acute Respiratory Failure due to Neuromyelitis Optica Treated Successfully with Plasmapheresis

    OpenAIRE

    Massa Zantah; Coyle, Timothy B.; Debapriya Datta

    2016-01-01

    Neuromyelitis Optica (NMO) is a demyelinating autoimmune disease involving the central nervous system. Acute respiratory failure from cervical myelitis due to NMO is known to occur but is uncommon in monophasic disease and is treated with high dose steroids. We report a case of a patient with NMO who developed acute respiratory failure related to cervical spinal cord involvement, refractory to pulse dose steroid therapy, which resolved with plasmapheresis.

  4. Acute Neonatal Parotitis with Late-Onset Septic Shock due to Streptococcus agalactiae

    OpenAIRE

    Boulyana, M.

    2014-01-01

    Acute neonatal parotitis (ANP) is a very rare disease. Most cases are managed conservatively; early antibiotics and adequate hydration may reduce the need for surgery. The most common cause of ANP is Staphylococcus aureus. We report a rare case of acute neonatal parotitis with late-onset septic shock due to Streptococcus agalactiae. The diagnosis was confirmed with ultrasound and isolation of Streptococcus agalactiae from blood culture. The patient was treated successfully with 10 days of int...

  5. Sudden psychotic episode probably due to meningoencephalitis and Chlamydia pneumoniae acute infection

    Directory of Open Access Journals (Sweden)

    Canas Nuno

    2005-09-01

    Full Text Available Abstract Background Since 9% to 20% of all cases of acute psychosis presenting to an Emergency Department (ED are due to a general medical condition, cautious medical workup should be mandatory in such patients. Differential diagnosis must consider conditions as diverse as renal failure or CNS infection. Acute Chlamydia pneumoniae infection usually causes a self-limited respiratory syndrome. Rarely, acute neurological complications occur, with acute meningoencephalitis most frequently reported. Diagnosis requires a high level of suspicion and is difficult to confirm. Case report We describe a 22 year-old female Caucasian who, three days after a mild pharingitis, developed an acute psychosis with exuberant symptoms interspersed with periods of lucidity, in a background of normal consciousness and orientation. Initial medical and imagiological workup were inconclusive. After 20 days of unsuccessful treatment with antipsychotics she developed a high fever and was re-evaluated medically. Lumbar puncture revealed an inflammatory cerebrospinal fluid. MRI showed irregular thickening and nodularity of the lateral ventricles' lining. An anti-Chlamydia pneumoniae IgM antibody titter of 85 IU/ml was detected. All symptoms cleared after treatment with antibiotics and corticosteroids. Conclusion This is, to our knowledge, the first reported case of acute CP-associated meningoencephalitis manifesting as an acute psychotic episode. It illustrates the principle that non-organic psychiatric syndromes must remain a diagnosis of exclusion in first-time acute psychosis.

  6. Splenic artery pseudoaneurysm due to acute pancreatitis in a 6-year-old boy with acute lymphoblastic leukaemia treated with L-aspariginase

    DEFF Research Database (Denmark)

    Larsen, Cæcilie Crawley; Laursen, Christian B; Dalby, Kasper;

    2014-01-01

    Acute pancreatitis is a rare phenomenon in children but its incidence seems to be increasing. In children, it is generally caused due to systemic illness, biliary disease, trauma, idiopathy and side effects of medicines like L-aspariginase. Acute pancreatitis is difficult to diagnose in children...... pseudoaneurysm due to acute pancreatitis in a 6-year-old boy with acute lymphoblastic leukaemia treated with L-aspariginase. He presented with fever, irritability and pain in his left groin region....

  7. Acute oxalate nephropathy due to ‘Averrhoa bilimbi’ fruit juice ingestion

    Science.gov (United States)

    Bakul, G.; Unni, V. N.; Seethaleksmy, N. V.; Mathew, A.; Rajesh, R.; Kurien, G.; Rajesh, J.; Jayaraj, P. M.; Kishore, D. S.; Jose, P. P.

    2013-01-01

    Irumban puli (Averrhoa bilimbi) is commonly used as a traditional remedy in the state of Kerala. Freshly made concentrated juice has a very high oxalic acid content and consumption carries a high risk of developing acute renal failure (ARF) by deposition of calcium oxalate crystals in renal tubules. Acute oxalate nephropathy (AON) due to secondary oxalosis after consumption of Irumban puli juice is uncommon. AON due to A. bilimbi has not been reported before. We present a series of ten patients from five hospitals in the State of Kerala who developed ARF after intake of I. puli fruit juice. Seven patients needed hemodialysis whereas the other three improved with conservative management. PMID:23960349

  8. 内镜治疗Roux-en-Y术后并发急性梗阻性化脓性胆管炎的新方法——经腹壁小肠造瘘逆行进镜ERC%A new endoscopic therapeutic method for acute obstructive suppurative cholangitis post Rouxen-Y anastomosis: endoscopic retrograde cholangiography through jejunostomy

    Institute of Scientific and Technical Information of China (English)

    杨卓; 高峰; 麻树人; 程广明; 刘国平; 李顺明; 韩磊; 赵志锋; 赵云峰; 高飞

    2012-01-01

    Objective The present study was to probe the value of endoscopic retrograde cholangiography (ERC) through jejunostomy in patients in whom ERC could not ba performed via the mouth after Roux-en-Y anastomosis on the upper gastrointestinal tract. Methods In two patients were suffering from acute obstructive suppurative cholangitis after a radical operation for cholangiocarcinoma, ERC could not done through the mouth due to the presence of a long non-functional intestinal loop. A jejunostomy was first done in the afferent loop of the jejunam. A gastroscope was then inserted via the jejunostomy, and it was passed retrogradely, to find the stoma of the cholangio-intestinal anastomosis. ERC was then successfully performed, and it was followed by endoscopic retrograde biliary drainage (ERBD). Results The operation was successful. It was found that cholangio-jejunostomy stoma was narrow, and a large amount of purulent mucus was present in the enlarged intrahepatic duct. ERC was done to enlarge the stoma, and a stent was placed into the main branch of the intrahepatic duct. The operation was successfully done in two patients, and they recovered smoothly after the operation. Conclusion ERC through a jejunostomy in patients who had had Roux-en-Y cholangio-jejunostomy following radical resection for cholangiocarcinoma, is a safe and effective surgical procedure.%目的 探讨上消化道重建术后患者无法经口完成内镜逆行胆管造影术(ERC)时经腹壁入路小肠造瘘ERC的价值.方法 2例胆管癌根治术后再发急性梗阻性化脓性胆管炎患者,因无功能空肠襻迂曲、过长,无法完成经口ERC,先行腹壁切开找到输入襻小肠造瘘,自瘘口送入胃镜,沿无功能空肠襻逆行进镜找到胆肠吻合口,行ERC并胆管支架置入术(ERBD).结果 行腹壁切开并成功于无功能空肠襻小肠造瘘,经造瘘口沿空肠襻逆行进镜发现胆肠吻合口狭窄,肝内胆管可见大量脓性黏液,遂扩张吻合口行ERC,

  9. 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.

  10. 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.

  11. [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.

  12. 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.

  13. Migrated endoclip and stone formation after cholecystectomy: A new danger of acute pancreatitis

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Endoclip migration into the common bile duct following laparoscopic cholecystectomy (LC) is an extremely rare complication. Migrated endoclip into the common bile duct can cause obstruction, serve as a nidus for stone formation, and cause cholangitis. We report a case of obstructive jaundice and acute biliary pancreatitis due to choledocholithiasis caused by a migrated endoclip 6 mo after LC. The patient underwent early endoscopic retrograde cholangiopancreatography (ERCP) with endoscopic sphincterotomy and stone extraction.

  14. Acute Neonatal Parotitis with Late-Onset Septic Shock due to Streptococcus agalactiae

    Directory of Open Access Journals (Sweden)

    M. Boulyana

    2014-01-01

    Full Text Available Acute neonatal parotitis (ANP is a very rare disease. Most cases are managed conservatively; early antibiotics and adequate hydration may reduce the need for surgery. The most common cause of ANP is Staphylococcus aureus. We report a rare case of acute neonatal parotitis with late-onset septic shock due to Streptococcus agalactiae. The diagnosis was confirmed with ultrasound and isolation of Streptococcus agalactiae from blood culture. The patient was treated successfully with 10 days of intravenous antibiotics and supportive measures. Despite being rare, streptococcal ANP should be considered in the etiological diagnosis of neonatal sepsis. Early diagnosis and appropriate antibiotic might prevent serious complications.

  15. Percutaneous mechanical assist for severe cardiogenic shock due to acute right ventricular failure.

    Science.gov (United States)

    Kipp, Ryan; Raval, Amish N

    2015-05-01

    Acute right ventricular failure can lead to severe cardiogenic shock and death. Recovery may be achieved with early supportive measures. In many patients, intravenous fluid and inotropic resuscitation is inadequate to improve cardiac output. In these cases, percutaneous mechanical assist may provide a non-surgical bridge to recovery. Herein, we describe a case series of patients with severe, refractory cardiogenic shock due to acute right ventricular failure who received a continuous flow percutaneous ventricular device primarily utilizing the right internal jugular vein for out flow cannula placement.

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

    Directory of Open Access Journals (Sweden)

    James L Buxbaum

    2011-07-01

    Full Text Available 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.

  17. Ventricular enlargement due to acute hypernatremia in a patient with a ventriculoperitoneal shunt.

    Science.gov (United States)

    Andres, Robert H; Pendharkar, Arjun V; Kuhlen, Dominique; Mariani, Luigi

    2010-07-01

    Patients requiring CSF shunts frequently have comorbidities that can influence water and electrolyte balances. The authors report on a case involving a ventriculoperitoneal shunt in a patient who underwent intravenous hyperhydration and withdrawal of vasopressin substitution prior to scheduled high-dose chemotherapy regimen for a metastatic suprasellar germinoma. After acute neurological deterioration, the patient underwent CT scanning that demonstrated ventriculomegaly. A shunt tap revealed no flow and negative opening pressure. Due to suspicion of proximal shunt malfunction, the comatose patient underwent immediate surgical exploration of the ventricle catheter, which was found to be patent. However, acute severe hypernatremia was diagnosed during the procedure. After correction of the electrolyte disturbances, the patient regained consciousness and made a good recovery. Although rare, the effects of acute severe hypernatremia on brain volume and ventricular size should be considered in the differential diagnosis of ventriculoperitoneal shunt failure. PMID:19911884

  18. Recurrent Episodes of Acute Pancreatitis Due to Duodenal Gangliocytic Paraganglioma: Report of a Case

    Directory of Open Access Journals (Sweden)

    Natalia Asimakopoulou

    2014-03-01

    Full Text Available Context Gangliocytic paraganglioma is a rare tumor, almost always located in the second portion of the duodenum, andmanifested with upper gastrointestinal bleeding and abdominal pain. To date, only one case of duodenal gangliocyticparaganglioma presented with recurrent acute pancreatitis has been reported in the literature. Case report We present a72-year-old woman admitted to the hospital due to recurrent episodes of acute pancreatitis. Paraclinical examinationsshowed a polypoid mass in the second portion of duodenum which was removed surgically by local excision. Thepreoperative differential diagnosis was suggestive with gastrointestinal stromal tumor or adenoma. The histopathologyexamination revealed a duodenal gangliocytic paraganglioma. After a follow up period of seventeen months the patientremained without clinical evidence of tumor recurrence. Conclusion Our case report draws attention to the need forincluding in our differential diagnosis of recurrent acute pancreatitis the mechanical obstruction of the pancreatic duct dueto this tumor.

  19. Acute liver failure due to non-exertional heatstroke after sauna.

    Science.gov (United States)

    Erarslan, Elife; Yüksel, Ilhami; Haznedaroglu, Serap

    2012-01-01

    Acute liver failure is defined as rapid loss of liver function that patients without previously recognized liver disease sustain a liver damage. Acute liver failure due to non-exertional heatstroke has rarely been reported. We reported here an unusual case of heat stroke induced acute liver failure (ALF) after sauna. A 63 year old man without previously recognized liver and other systemic disease was admitted for loss of consciousness and impaired liver function after sauna. Despite intensive supportive care, ALF developed. Liver transplantation was planned but the patient died on the sixth day of hospitalization. Non-exertional heatstroke induced ALF is a rare and serious condition. ALF caused by non-exertional heatstroke which requires liver transplantation for definitive solution should be kept in mind in early period.

  20. Acute respiratory failure due to refeeding syndrome and hypophosphatemia induced by hypocaloric enteral nutrition.

    Science.gov (United States)

    Patel, Utpal; Sriram, Krishnan

    2009-03-01

    We report a case of acute respiratory failure due to refeeding syndrome caused by hypocaloric enteral tube feeds. A 60-y-old obese man, with a diagnosis of esophageal carcinoma with local metastases, underwent feeding jejunostomy tube insertion. Enteral tube feeding was initiated at small volumes providing 4.4 kcal x kg(-1) x d(-1) and gradually increased over 48 h to 29 kcal x kg(-1) x d(-1) (based on adjusted body weight). The patient then developed acute respiratory distress requiring intubation and ventilatory support. Serum phosphorus (P) level was extremely low at 4 d to adequately correct the electrolyte derangements. Successful liberation from mechanical ventilation was then possible. In chronically malnourished patients undergoing nutritional support, even hypocaloric feeding should be considered a risk factor for developing refeeding syndrome leading to severe and acute electrolyte fluid-balance and metabolic abnormalities.

  1. [Acute Pyelonephritis and Candidemia Due to Candida lusitaniae: A Case Report].

    Science.gov (United States)

    Tsuboi, Motoyuki; Uno, Shunsuke; Hase, Ryota; Yano, Yudai; Sando, Eiichiro; Otsuka, Yoshihito; Hosokawa, Naoto

    2016-03-01

    Although candiduria is becoming increasingly common among hospitalized patients, Candida lusitaniae is a rare pathogen that account for less than 1% of Candida species isolated from urine. Ascending pyelonephritis and candidemia due to Candida species are uncommon complications. We report herein on a case of acute pyelonephritis and candidemia due to C. lusitaniae. A 66-year-old man presented with a high fever during hospitalization at our hospital following septic shock due to ischial osteomyelitis treated with tazobactam/piperacillin for 29 days. We suspected acute pyelonephritis, and urinary Gram staining showed only yeasts and leucocytes. The next day, blood culture and urine culture tested positive and showed yeast-like fungi. We diagnosed acute pyelonephritis and candidemia due to Candida species and started treatment with fluconazole. C. lusitaniae was identified on the hospital day 34 and treated with fluconazole for 14 days. Candida albicans was the most prevalent species isolated from the urinary tract, however non-albicans Candida species have emerged and are now dominant because of the advent and increasing use of fluconazole. C. lusitaniae is a rare but important pathogen, that is generally susceptible to fluconazole and resistant to amphotericin B. It is necessary to choose an appropriately effective antifungal drug based on identification of the fungal species.

  2. Triple Diuretics and Aquaretic Strategy for Acute Decompensated Heart Failure due to Volume Overload

    Directory of Open Access Journals (Sweden)

    Rita Jermyn

    2013-01-01

    Full Text Available Diuretics, including furosemide, metolazone, and spironolactone, have historically been the mainstay of therapy for acute decompensated heart failure patients. The addition of an aquaretic-like vasopressin antagonist may enhance diuresis further. However, clinical experience with this quadruple combination is lacking in the acute setting. We present two hospitalized patients with acute decompensated heart failure due to massive fluid overload treated with a combination strategy of triple diuretics in conjunction with the aquaretic tolvaptan. The first patient lost 72.1 lbs. (32.7 kg with an average urine output of 3.5 to 7.5 L/day over eight days on combined therapy with furosemide, metolazone, spironolactone, and tolvaptan. The second patient similarly achieved a weight loss of 28.2 lbs. (12.8 kg over 4 days on the same treatment. Both patients maintained stable serum sodium, potassium, and creatinine over this period and remained out of the hospital for more than 30 days. Thus, patients hospitalized with acute decompensated heart failure due to volume overload can achieve euvolemia rapidly and without electrolytes disturbances using this regimen, while being under the close supervision of a team of cardiologists and nephrologists. Additionally, this therapy can potentially decrease the need for ultrafiltration and the length of hospital stay.

  3. Triple Diuretics and Aquaretic Strategy for Acute Decompensated Heart Failure due to Volume Overload

    Science.gov (United States)

    Estrada, Chelsea; Patel, Sagar; Weisfelner Bloom, Michelle; Wadhwa, Nand K.

    2013-01-01

    Diuretics, including furosemide, metolazone, and spironolactone, have historically been the mainstay of therapy for acute decompensated heart failure patients. The addition of an aquaretic-like vasopressin antagonist may enhance diuresis further. However, clinical experience with this quadruple combination is lacking in the acute setting. We present two hospitalized patients with acute decompensated heart failure due to massive fluid overload treated with a combination strategy of triple diuretics in conjunction with the aquaretic tolvaptan. The first patient lost 72.1 lbs. (32.7 kg) with an average urine output of 3.5 to 7.5 L/day over eight days on combined therapy with furosemide, metolazone, spironolactone, and tolvaptan. The second patient similarly achieved a weight loss of 28.2 lbs. (12.8 kg) over 4 days on the same treatment. Both patients maintained stable serum sodium, potassium, and creatinine over this period and remained out of the hospital for more than 30 days. Thus, patients hospitalized with acute decompensated heart failure due to volume overload can achieve euvolemia rapidly and without electrolytes disturbances using this regimen, while being under the close supervision of a team of cardiologists and nephrologists. Additionally, this therapy can potentially decrease the need for ultrafiltration and the length of hospital stay. PMID:24829808

  4. [Necrotizing tonsillitis and renal vein thrombosis due to acute myeloid leukaemia].

    Science.gov (United States)

    Akram, Javed; Josefsson, Pernilla; Rømeling, Frans

    2012-09-01

    A 37-year-old woman was admitted to hospital with severe tonsillitis with unilateral necrotizing tonsillitis. She suddenly got fever, malaise, difficulties swallowing, pain in the throat and deterioration despite four days of penicillin treatment. During hospitalisation, she experienced abdominal pain, and blood tests showed pancytopenia. She was transferred to a haematological department, where a bone marrow biopsy showed acute myeloid leukaemia. Subsequently, an abdominal computed tomography with intravenous contrast revealed bilateral renal vein thrombosis, probably because of coagulopathy due to leukaemia.

  5. Unusual presentations of acute kidney injury and neurologic complications due to snake bite

    OpenAIRE

    Hamid Noshad; Reza Rikhtegar; Mehdi Hagdoost; Masood Dinevari; Hosein Mahmoodi

    2015-01-01

    Introduction: Vascularity of kidneys is very high, so these organs are potentially susceptible to be affected with toxins including snake venom. Hypersensitivity to snake venous could cause some neurological problem. Case Report: We present a 14-year-old boy with acute kidney injury (AKI) due to snake bite. After a few days, kidney failure with hematuria was developed. His serum creatinine level rose to 3 mg/dl and following 2 weeks gradually and d...

  6. Post-renal acute renal failure due to a huge bladder stone.

    Science.gov (United States)

    Celik, Orcun; Suelozgen, Tufan; Budak, Salih; Ilbey, Yusuf Ozlem

    2014-06-30

    A 63-year old male was referred to our emergency unit due to acute renal failure. The level of serum renal function tests levels, blood urea nitrogen (BUN)/creatinine, were 63 mmol/L/848 μmol/L. CT (Computarised Tomography) scan showed a huge bladder stone (5 cm x 6 cm x 5 cm) with increased bladder wall thickness. Post-renal acute renal failure due to bilateral ureterohydronephrosis was diagnosed. The huge bladder stone was considered to be the cause of ureterohydronephrosis and renal failure. The patient was catheterised and received haemodialysis immediately. He received haemodialysis four times during ten days of hospitalization and the level of serum renal function tests levels (BUN/ creatinine) decreased 18 mmol/L/123 μmol/L. After improvement of renal function, we performed cystoscopy that demonstrated normal prostatic urethra and bladder neck and bilaterally normal ureteral orifices. Bladder wall was roughly trabeculated and Bladder outlet was completely obstructed by a huge bladder stone. After cystoscopy open, cystolithotomy was performed to remove calcium phosphate and magnesium ammonium phosphate stone weighing 200 g removed. Four days after operation the patient was discharged uneventfully and urethral catheter was removed on the seventh day. Post-renal acute renal failure due to large bladder stones is rare in literature. According to the our knowledge; early diagnosis of the stone avoid growth to large size and prevent renal failure.

  7. Acute metabolic decompensation due to influenza in a mouse model of ornithine transcarbamylase deficiency

    Directory of Open Access Journals (Sweden)

    Peter J. McGuire

    2014-02-01

    Full Text Available The urea cycle functions to incorporate ammonia, generated by normal metabolism, into urea. Urea cycle disorders (UCDs are caused by loss of function in any of the enzymes responsible for ureagenesis, and are characterized by life-threatening episodes of acute metabolic decompensation with hyperammonemia (HA. A prospective analysis of interim HA events in a cohort of individuals with ornithine transcarbamylase (OTC deficiency, the most common UCD, revealed that intercurrent infection was the most common precipitant of acute HA and was associated with markers of increased morbidity when compared with other precipitants. To further understand these clinical observations, we developed a model system of metabolic decompensation with HA triggered by viral infection (PR8 influenza using spf-ash mice, a model of OTC deficiency. Both wild-type (WT and spf-ash mice displayed similar cytokine profiles and lung viral titers in response to PR8 influenza infection. During infection, spf-ash mice displayed an increase in liver transaminases, suggesting a hepatic sensitivity to the inflammatory response and an altered hepatic immune response. Despite having no visible pathological changes by histology, WT and spf-ash mice had reduced CPS1 and OTC enzyme activities, and, unlike WT, spf-ash mice failed to increase ureagenesis. Depression of urea cycle function was seen in liver amino acid analysis, with reductions seen in aspartate, ornithine and arginine during infection. In conclusion, we developed a model system of acute metabolic decompensation due to infection in a mouse model of a UCD. In addition, we have identified metabolic perturbations during infection in the spf-ash mice, including a reduction of urea cycle intermediates. This model of acute metabolic decompensation with HA due to infection in UCD serves as a platform for exploring biochemical perturbations and the efficacy of treatments, and could be adapted to explore acute decompensation in other

  8. A Rare Side Effect due to TNF-Alpha Blocking Agent: Acute Pleuropericarditis with Adalimumab

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    Hakan Ozkan

    2013-01-01

    Full Text Available Tumor necrosis factor-alpha antagonism is an important treatment strategy in patients with rheumatoid arthritis, psoriatic arthritis, vasculitis, and ankylosing spondylitis. Adalimumab is one of the well-known tumor necrosis factor-alpha blocking agents. There are several side effects reported in patients with adalimumab therapy. Cardiac side effects of adalimumab are rare. Only a few cardiac side effects were reported. A 61-year-old man treated with adalimumab for the last 6 months due to psoriatic arthritis presented with typically acute pleuropericarditis. Chest X-ray and echocardiography demonstrated marked pericardial effusion. Patient was successfully evaluated for the etiology of acute pleuro-pericarditis. Every etiology was excluded except the usage of adalimumab. Adalimumab was discontinued, and patient was treated with 1200 mg of ibuprofen daily. Control chest X-ray and echocardiography after three weeks demonstrated complete resolution of both pleural and pericardial effusions. This case clearly demonstrated the acute onset of pericarditis with adalimumab usage. Acute pericarditis and pericardial effusion should be kept in mind in patients with adalimumab treatment.

  9. [A case of eosinophilic pneumonia due to Nicolase (serrapeptase) after recovery from acute eosinophilic pneumonia].

    Science.gov (United States)

    Kai, Naoko; Shirai, Ryo; Hirata, Norio; Iwata, Atsuko; Umeki, Kenji; Ishii, Hiroshi; Kishi, Kenji; Tokimatsu, Issei; Hiramatsu, Kazufumi; Kadota, Jun-ichi

    2009-03-01

    A case of eosinophilic pneumonia due to Nicolase (serrapeptase) after recovery from acute eosinophilic pneumonia is described. A 32-year-old woman was previously admitted to another hospital because of acute onset of dyspnea accompanied by cough and fever. Chest X-ray films revealed diffuse infiltration in both lungs two days after her symptoms occurred. Her bronchoalveolar lavage fluid showed 13% eosinophils and transbronchial lung biopsy specimen also showed many eosinophils infiltrating in the lesions of the bronchial submucosa and alveolar septa. No infectious causes or related drugs were found. Acute eosinophilic pneumonia was diagnosed, and her condition improved gradually without steroid treatment. Because she recovered clinically and radiologically, she was discharged from hospital. Half a month later she was treated with Nicolase because of pharyngitis. She was admitted to the hospital again because of dyspnea, cough and fever three days after commencing to take Nicolase. Chest X-ray films also revealed diffuse infiltration in both lungs with pleural effusion, and her bronchoalveolar lavage fluid showed 37% eosinophils. When the drug lymphocyte stimulation test was performed, it was positive for Nicolase. Therefore drug-induced eosinophilic pneumonia was diagnosed. This is a very rare case of Nicolase (serrapeptase)-induced eosinophilic pneumonia after recovering from acute eosinophilic pneumonia.

  10. Acute Spontaneous Subdural Hematoma due to Rupture of a Tiny Cortical Arteriovenous Malformation.

    Science.gov (United States)

    Choi, Hyuk Jin; Lee, Jae Il; Nam, Kyoung Hyup; Ko, Jun Kyeung

    2015-12-01

    Acute subdural hematoma (SDH) of arterial origin is rare, especially SDH associated with an arteriovenous malformation (AVM) is extremely rare. The authors report a case of acute spontaneous SDH due to rupture of a tiny cortical AVM. A 51-year-old male presented with sudden onset headache and mentality deterioration without a history of trauma. Brain CT revealed a large volume acute SDH compressing the right cerebral hemisphere with subfalcine and tentorial herniation. Emergency decompressive craniectomy was performed to remove the hematoma and during surgery a small (5 mm sized) conglomerated aciniform mass with two surrounding enlarged vessels was identified on the parietal cortex. After warm saline irrigation of the mass, active bleeding developed from a one of the vessel. The bleeding was stopped by coagulation and the vessels were removed. Histopathological examination confirmed the lesion as an AVM. We concluded that a small cortical AVM existed at this area, and that the cortical AVM had caused the acute SDH. Follow up conventional angiography confirmed the absence of remnant AVM or any other vascular abnormality. This report demonstrates rupture of a cortical AVM is worth considering when a patient presents with non-traumatic SDH without intracerebral hemorrhage or subarachnoid hemorrhage. PMID:26819690

  11. 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.

  12. 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.

  13. Acute Intrathoracic Gastric Volvulus due to Diaphragmatic Hernia: A Rare Emergency Easily Overlooked

    Directory of Open Access Journals (Sweden)

    Hyung Hun Kim

    2011-05-01

    Full Text Available Acute intrathoracic gastric volvulus occurs when the stomach undergoes organoaxial torsion in the chest due to either concomitant enlargement of the hiatus or a diaphragmatic hernia. Iatrogenic diaphragmatic hernia can occur after hiatal hernia repair and other surgical procedures, such as nephrectomy, esophagogastrectomy and splenopancreatectomy. We describe a 49-year-old woman who presented to our emergency department with acute moderate epigastric soreness and vomiting. She had undergone extensive gynecologic surgery including splenectomy 1 year before. The chest radiograph obtained in the emergency department demonstrated an elevated gastric air-fluid level in the left lower lung field. An urgent gastroscopy showed twisted structural abnormality of the stomach body. A computed tomography scan demonstrated the distended stomach, located in the left lower hemithorax through a left diaphragmatic defect. Emergent transthoracic repair was performed. Postoperative recovery was uneventful, and the patient did not experience any pain or difficulty with eating.

  14. 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.

  15. 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.

  16. Rhabdomyolysis, acute kidney injury and transverse myelitis due to naive heroin exposure

    Directory of Open Access Journals (Sweden)

    Ankur Gupta

    2011-01-01

    Full Text Available Heroin exposure can cause various complications like seizures, stroke, spongiform encephalopathy, transverse myelopathy, plexopathy, compartment syndrome, rhabdomyolysis and renal failure due to various mechanisms. We report here a young male who smoked heroin for the first time and developed transverse myelitis, rhabdomyolysis and acute kidney injury requiring dialysis. His renal recovery was complete by four weeks, while neurological improvement occurred 8 to 12 weeks later. This case suggests a common pathogenic mechanism of heroin intoxication involving multiple systems of the body.

  17. Acute scrotum due to arterial bleeding mimicking non-seminomatous germ cell tumor

    Institute of Scientific and Technical Information of China (English)

    F.Christoph; M.Schradert; A.Amirmaki; K.Miller

    2004-01-01

    Men with testicular tumors usually present with painless increase in testis size incidentally noticed by the patient. We report a case of a young patient presenting as an emergency with acute onset of massive right-sided testicular pain without previous injury. After physical examination testicular torsion could not be excluded. Ultrasound examination of the tesds was suspicious for tesdcular tumor. Surgical exploration of the right testis by inguinal approach was performed revealing subcapsular arterial bleeding due to a small nonseminomatous germ cell tumor non-palpable on clinical examination. (Asian J Andro12004 Dec;6:379-381)

  18. A case of acute epididymo-orchitis due to Pseudomonas aeruginosa presenting as ARDS in an immunocompetent host

    Institute of Scientific and Technical Information of China (English)

    Sameer Singhal; DD Wagh; Shivali Kashikar; Yeshwant Lonkar

    2011-01-01

    Acute eididymo-orchitis is the most common cause of intrascrotal inflammation, and retrograde ascent of pathogens is the usual route of infection. Here we intend to present a case of young boy, not sexually active, suffering from acute epididymo-orchitis due to Pseudomonas aeruginosa presented with acute respiratory distress syndrome. Proper timely diagnosis of the primary cause and prompt treatment including support with non invasive ventilation lead to a favourable outcome in the same case.

  19. A case of acute epididymo-orchitis due to Pseudomonas aeruginosa presenting as ARDS in an immunocompetent host

    Institute of Scientific and Technical Information of China (English)

    Sameer; Singhal; DD; Wagh; Shivali; Kashikar; Yeshwant; Lonkar

    2011-01-01

    Acute eididymo-orchitis is the most common cause of intrascrolal inflammation,and retrograde ascent of pathogens is the usual route of infection.Here we intend to present a case of young hoy. not sexually active,suffering from acute epididymo-orchitis due lo Pseudomonas aeruginosa presented with acute respiratory distress syndrome.Proper timely diagnosis of the primary cause and prompt treatment including support with nun invasive ventilation lead lo a favourable outcome in the same case.

  20. [Acute encephalopathy due to late-onset maple syrup urine disease in a school boy].

    Science.gov (United States)

    Qu, Su-Qing; Yang, Li-Cai; Luan, Zuo; Du, Kan; Yang, Hui

    2012-03-01

    Maple syrup urine disease is a common amino acids metabolic disease. In most patients, onset occurs in the neonatal period and infancy. In this study, the case of a school boy with acute encephalopathy due to late-onset maple syrup urine disease is summarized. The boy (8.5 years) was admitted because of acute encephalopathy after suffering from infection for two days at the age of eight and a half years. Metabolic acidosis, hyperuricemia and decreased protein level in cerebrospinal fluid were found by general laboratory tests. Magnetic resonance imaging of the brain revealed signal intensity abnormalities in the bilateral cerebellum dentate nucleus, brainstem, thalamus, putamen, caudate nucleus and cortex of the cerebral hemispheres. On T1WI and T2WI scanning, hyperintensive signal was found. Blood leucine and valine were significantly elevated. Urinary 2-hydroxy isovaleric acid, 3-hydroxybutyric acid, 2-keto isovaleric acid, and 2-keto acid also increased. Both the blood amino acid and urine organic acid profiles led to the diagnosis of maple syrup urine disease. In the acute period, the patient was treated with a large dose of vitamin B1, glucose, L-carnitine and a protein-restrict diet. The patient's condition improved significantly after five days of treatment, and he recovered completely two days later. Afterwards, treatment with vitamin B1, L-carnitine and a protein-restrict diet (1 g/kg/day) was continued. One and a half months later, blood amino acids and urine organic acids returned to normal. Magnetic resonance imaging of the brain also indicated a great improvement. It was concluded that inborn metabolic disease should be considered in the patients with an onset similar to acute encephalopathy. Early diagnosis and proper treatment can prevent brain damage and improve prognosis.

  1. Therapeutic options for acute cough due to upper respiratory infections in children.

    Science.gov (United States)

    Paul, Ian M

    2012-02-01

    Cough due to upper respiratory tract infections (URIs) is one of the most frequent complaints encountered by pediatric health-care providers, and one of the most disruptive symptoms for children and families. Despite the frequency of URIs, there is limited evidence to support the few therapeutic agents currently available in the United States (US) to treat acute cough due to URI. Published, well-designed, contemporary research supporting the efficacy of narcotics (codeine, hydrocodone) and US Food and Drug Administration (FDA)-approved over-the-counter (OTC) oral antitussives and expectorants (dextromethorphan, diphenhydramine, chlophedianol, and guaifenesin) is absent for URI-associated pediatric cough. Alternatively, honey and topically applied vapor rubs may be effective antitussives.

  2. Therapeutic options for acute cough due to upper respiratory infections in children.

    Science.gov (United States)

    Paul, Ian M

    2012-02-01

    Cough due to upper respiratory tract infections (URIs) is one of the most frequent complaints encountered by pediatric health-care providers, and one of the most disruptive symptoms for children and families. Despite the frequency of URIs, there is limited evidence to support the few therapeutic agents currently available in the United States (US) to treat acute cough due to URI. Published, well-designed, contemporary research supporting the efficacy of narcotics (codeine, hydrocodone) and US Food and Drug Administration (FDA)-approved over-the-counter (OTC) oral antitussives and expectorants (dextromethorphan, diphenhydramine, chlophedianol, and guaifenesin) is absent for URI-associated pediatric cough. Alternatively, honey and topically applied vapor rubs may be effective antitussives. PMID:21892785

  3. LAPAROSCOPIC CHOLECYSTECTOMY FOR ACUTE CHOLECYSTITIS DUE TO CHOLELITHIASIS IN GERIATRIC PATIENTS

    Directory of Open Access Journals (Sweden)

    D. Vasincu

    2007-07-01

    Full Text Available This study is focused on the results of laparoscopic cholecystectomy for acute cholecystitis due to cholelithiasis in geriatric patients. The records of all patients from „St. Joan” Emergency Hospital Iaşi from January 2001 to December 2005 and from General Surgery Clinic in S. Orsola - M. Malpighi Hospital Bologna – Italia from January 2003 to December 2004 who undergo cholecystectomy were reviewed. The entire series consists of 557 patients. This report identifies 161 patients who were aged 65 years or older at the time of laparoscopic cholecystectomy. The results are reported in three progressive age groups: 65 to 74, 75 to 84, and over 85 years. At 101 geriatric patients was practiced laparoscopic cholecystectomy. Gallstones were present in all patients. In this series of laparoscopic cholecystectomy, 32.6% had an emergency procedure and the remainder had an elective or semielective procedure. The conversion rate was 5.6%. 74.2% of the patients were discharged home after 3-5 days of postoperative observation. Laparoscopic cholecystectomy is a safe procedure and should be recommended for all geriatric patients who have acute cholecystitis due to cholelithiasis before the development of complications.

  4. Evidence Report: Risk of Acute Radiation Syndromes Due to Solar Particle Events

    Science.gov (United States)

    Carnell, Lisa; Blattnig, Steve; Hu, Shaowen; Huff, Janice; Kim, Myung-Hee; Norman, Ryan; Patel, Zarana; Simonsen, Lisa; Wu, Honglu

    2016-01-01

    Crew health and performance may be impacted by a major solar particle event (SPE), multiple SPEs, or the cumulative effect of galactic cosmic rays (GCR) and SPEs. Beyond low-Earth orbit, the protection of the Earth's magnetosphere is no longer available, such that increased shielding and protective mechanisms are necessary in order to prevent acute radiation sickness and impacts to mission success or crew survival. While operational monitoring and shielding are expected to minimize radiation exposures, there are EVA scenarios outside of low-Earth orbit where the risk of prodromal effects, including nausea, vomiting, anorexia, and fatigue, as well as skin injury and depletion of the blood-forming organs (BFO), may occur. There is a reasonable concern that a compromised immune system due to high skin doses from a SPE or due to synergistic space flight factors (e.g., microgravity) may lead to increased risk to the BFO. The primary data available at present are derived from analyses of medical patients and persons accidentally exposed to acute, high doses of low-linear energy transfer (LET) (or terrestrial) radiation. Data more specific to the space flight environment must be compiled to quantify the magnitude of increase of this risk and to develop appropriate protection strategies. In particular, information addressing the distinct differences between solar proton exposures and terrestrial exposure scenarios, including radiation quality, dose-rate effects, and non-uniform dose distributions, is required for accurate risk estimation.

  5. 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).

  6. Genetic epidemiology of primary sclerosing cholangitis

    Science.gov (United States)

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

    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). PMID:17907284

  7. 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.

  8. 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.

  9. Acute liver failure due to Human Herpesvirus 6 in an infant

    Directory of Open Access Journals (Sweden)

    G.M. Tronconi

    2012-10-01

    Full Text Available We report a case of a 4-months infant with fever in the absence of other specific symptoms that has rapidly and unexpectedly developed acute liver failure (ALF with coagulopathy and complicated with bone marrow failure without encephalopathy. The main viral infection agents (hepatitis virus A, B, C, Citomegalovirus, Ebstain Barr virus, Parvovirus B19, Adenovirus, drug-induced hepatotoxicity and metabolic disorders associated to ALF were excluded. Quantitative determination of Human Herpesvirus 6 (HHV6 genome was positive with a significant number of copies for mL. A favorable evolution of the clinical symptoms and a progressive hematochemical resolution were obtained. Plasma and Vitamin K were administrated as a support therapy for treating coagulopathy. The present case report and the cases’ review from the literature, evidence the importance of always including screening for HHV6 infection in the diagnostic approach to acute onset of liver failure. HHV6 is a common virus in the pediatric population with a greater number of cases of fulminant viral non-A, non-B, non-C hepatitis in immunocompetent patients due to this virus: these forms have often a high mortality rate and maybe necessitate liver transplantation; for this reason correct etiological agent identification is mandatory for the prognosis and it has to be based on the quantitative search of the virus’s genome. Pathogenesis of liver-induced damage associated to HHV6 remains unclear; however in vitro studies demonstrate the potential hepatotoxicity effects of this virus.

  10. [Acute liver failure due to human herpesvirus 6 in an infant].

    Science.gov (United States)

    Tronconi, G M; Mariani, B; Pajno, R; Fomasi, M; Cococcioni, L; Biffi, V; Bove, M; Corsin, P; Garbetta, G; Barera, G

    2012-01-01

    We report a case of a 4-months infant with fever in the absence of other specific symptoms that has rapidly and unexpectedly developed acute liver failure (ALF) with coagulopathy and complicated with bone marrow failure without encephalopathy. The main viral infection agents (hepatitis virus A, B, C, Citomegalovirus, Ebstain Barr virus, Parvovirus B19, Adenovirus), drug-induced hepatotoxicity and metabolic disorders associated to ALF were excluded. Quantitative determination of Human Herpesvirus 6 (HHV6) genome was positive with a significant number of copies for mL. A favorable evolution of the clinical symptoms and a progressive hematochemical resolution were obtained. Plasma and Vitamin K were administrated as a support therapy for treating coagulopathy. The present case report and the cases' review from the literature, evidence the importance of always including screening for HHV6 infection in the diagnostic approach to acute onset of liver failure. HHV6 is a common virus in the pediatric population with a greater number of cases of fulminant viral non-A, non-B, non-C hepatitis in immunocompetent patients due to this virus: these forms have often a high mortality rate and maybe necessitate liver transplantation; for this reason correct etiological agent identification is mandatory for the prognosis and it has to be based on the quantitative search of the virus's genome. Pathogenesis of liver-induced damage associated to HHV6 remains unclear; however in vitro studies demonstrate the potential hepatotoxicity effects of this virus. PMID:23342747

  11. Small Intestinal Obstruction with Intussusception due to Acute Myeloid Leukemia: A Case Report

    Directory of Open Access Journals (Sweden)

    Sangeeta Kini

    2012-01-01

    Full Text Available Myeloid sarcoma is known to precede the development of acute myeloid leukemia (AML and can be the only clinical manifestation. Gastrointestinal involvement by AML is rare with the commonest site being small intestine. Patients present with vague abdominal pain and/or obstruction. Prognosis is usually poor as most of them rapidly progress to AML. We report a case of 25-year-old man with complaints of abdominal pain and vomiting of one-year duration. OGD scopy revealed infiltration of lesser curvature of stomach. Subsequently patient came back within a week with signs and symptoms of acute intestinal obstruction for which an ileal resection was done. Although the histology of stomach biopsy and ileal segments showing similar features were thought to be non-Hodgkin's lymphoma, immunohistochemistry confirmed the diagnosis of myeloid sarcoma. Bone marrow investigations confirmed involvement by AML. Patient succumbed to the disease due to extensive involvement of AML. This case highlights the primary gastrointestinal manifestation of AML which can often prove to be a diagnostic difficulty clinically and histologically. Prompt diagnosis is essential to hasten the management.

  12. [Subdural empyema due to gemella morbillorum as a complication of acute sinusitis].

    Science.gov (United States)

    Boto, Leonor Reis; Calado, Cláudia; Vieira, Marisa; Camilo, Cristina; Abecasis, Francisco; Campos, Alexandre R; Correia, Manuela

    2011-01-01

    Subdural empyema is a life-threatening infection that may complicate acute sinusitis. The authors report the case of a previously healthy 10 year-old girl who presented with subdural empyema due to Gemella morbillorum after an untreated maxillary, ethmoidal and esphenoidal sinusitis. Despite immediate drainage of the empyema and underlying primary infection and treatment with broad spectrum antibiotics, she later developed frontal cerebritis and refractory intracranial hypertension, needing urgent decompressive craniectomy. She recovered gradually, maintaining to date slight right hemyparesis and aphasia. Even though it is considered a low virulence organism, G. morbillorum has been increasingly described in central nervous system infection. In this case, the prompt institution of broad spectrum antibiotics and surgical drainage, as well as the agressive treatment of complications, including decompressive craniectomy, were crucial to the patient's recovery. PMID:22015038

  13. Acute generalized exanthematous pustulosis due to sulfamethoxazol with positive lymphocyte transformation test (LTT).

    Science.gov (United States)

    Anliker, Mark David; Wüthrich, Brunello

    2003-01-01

    We studied an acute generalized exanthematous pustulosis (AGEP) due to sulfamethoxazol in a 48-year-old woman with unusual findings in allergy testing. The histological picture provided evidence for a pustular drug eruption and leukocytoclastic vasculitis. Skin testing with sulfamethoxazol was negative for immediate-type reaction (scratch test) and delayed-type reaction (epicutaneous testing). A lymphocyte transformation test (LTT) showed a significant lymphocyte stimulation (stimulation index 5.04/2.61) toward sulfamethoxazol (200/100 mg/ml) by measuring the rate of built-in tritium-thymidine in the DNS of the patients lymphocytes, implicating a drug-specific hypersensibility of lymphocytes; we could be dealing with a combined type III and IV reaction by Coombs and Gell in this case. LTT may play a possible role in the determination of drug allergy in AGEP despite negative skin testing. PMID:12861854

  14. Abdominal obesity and prolonged prone positioning increase risk of developing sclerosing cholangitis in critically ill patients with influenza A-associated ARDS

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    Weig Thomas

    2012-12-01

    Full Text Available Abstract Background Secondary sclerosing cholangitis is a severe disease of the biliary tract. Over the last decade, several cases of sclerosing cholangitis in critically ill patients (SC-CIP were reported. Reports in the literature so far are characterized by a wide variety of underlying causes of critical illness, thereby hindering a risk-factor analysis. We report on a homogenous cohort of critically ill patients with influenza A (H1N1 pneumonia and severe acute respiratory distress syndrome (ARDS, of whom a subgroup developed sclerosing cholangitis, allowing for probing of risk factors associated with SC-CIP. Methods Twenty-one patients (5 female, 16 male, 46.3 ± 10.8 years with severe ARDS due to H1N1 pneumonia were retrospectively divided into two groups, characterized by the presence (n = 5 and absence of SC-CIP (n = 16. A large array of clinical data, laboratory parameters, and multi-detector computed tomography-derived measures were compared. Results Both patient groups showed severe pulmonary impairment. Severity of disease on admission day and during the first 14 days of treatment showed no difference. The patients developing SC-CIP had a higher body mass index (BMI (37.4 ± 6.0 kg/m2 vs. 29.3 ± 6.8 kg/m2; P = 0.029 and a higher volume of intraperitoneal fat (8273 ± 3659 cm3 vs. 5131 ± 2268 cm3; P = 0.033 and spent a longer cumulative period in the prone position during the first 14 days (165 ± 117 h vs. 78 ± 61 h; P = 0.038. Conclusion Our results suggest that obesity, intraperitoneal fat volume, and a longer cumulative duration spent in the prone position may put patients with ARDS at risk of developing SC-CIP. These results lead us to propose that the prone position should be carefully deployed, particularly in abdominally obese patients, and that frequent checks be made for early hepatic dysfunction.

  15. A Rare Cause of Acute Abdomen: Urinary Bladder Rupture due to Foley Catheter

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    Ömer Engin

    2013-07-01

    Full Text Available Urinary bladder rupture is usually found due to blunt or penetrating traumas and iatrogenic injuries, and spontaneous intraperitoneal perforation is very rare. 57 years old male patient was consulted to general surgery department due to abdominal pain, nausia-vomiting and fever. He had Diabetes Mellitus, Multiple Sclerosis, Leriche Syndrome and operation for benign prostatic hypertrophy 5 years ago on his medical history which the case catheterised him urinary bladder himself frequently in nonsterile conditions. He did not care about sterility. On physical examination, suprapubic defence and rebound was noted more prominently on right lower quadrant. He was taken to operating room with the diagnosis of acute abdomen. On exploration, infection was seen on all layers of the whole urinary bladder, abscess formation and necrosis causing a microperforation which is detected by methylene blue was also present. Intraabdominal abscess drainage, cystostomy and primary suturing of the urinary bladder was performed. Patient died due to sepsis at the 9.th post operative day.

  16. Unusual presentations of acute kidney injury and neurologic complications due to snake bite

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    Hamid Noshad

    2015-06-01

    Full Text Available Introduction: Vascularity of kidneys is very high, so these organs are potentially susceptible to be affected with toxins including snake venom. Hypersensitivity to snake venous could cause some neurological problem. Case Report: We present a 14-year-old boy with acute kidney injury (AKI due to snake bite. After a few days, kidney failure with hematuria was developed. His serum creatinine level rose to 3 mg/dl and following 2 weeks gradually and decreased to normal level without any special treatment except for anti-venom, which was not prescribed inappropriate time (this type of AKI is not reported previously. He had seizure attacks, which were according to magnetic resonance imaging due to posterior reversible encephalopathy syndrome (PRES (This neurologic complication has been seen in other kidney injuries but up to now it was not reported in snake bite victims. Conclusion: Sanke venom could cause PRES due to AKI and seizure could be one of the most important complications in snake bite.

  17. Brain injury due to acute organophosphate poisoning Magnetic resonance imaging manifestation and pathological characteristics

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    BACKGROUND: Acute organophosphate poisoning can cause injuries of multiple visceras; especially,central nervous system injury can increase risk factors of patients with severe acute organophosphate poisoning. An application of modem image may increase diagnostic rate of brain injury in an earlier period and provide evidences for clinical treatment.OBJECTIVE: To reveal imaging manifestations, pathological characteristics and multi-ways injured mechanism of brain injury due to acute organophosphate poisoning.DESIGN: Contrast observational study.SETTING: Department of Medical Image, the Second Hospital of Hebei Medical University.MATERIALS: The experiment was carried out in the Department of Nerve Molecule Imaging Medicine and Laboratory of Neurology, the Second Hospital of Hebei Medical University from August 2003 to February 2004. A total of 30 healthy cats weighing 2.8 - 3.5 g and of both genders were selected from Animal Experimental Center of Hebei Medical University.METHODS: Thirty healthy cats were randomly divided into control group (n =5) and intoxication group (n=25). Cats in the control group were subcutaneously injected with 0.3 mL/kg saline at four points; while, cats in the intoxication group were subcutaneously injected with 400 g/L 0.3 mL/kg O,O-dimethyl-S-(methoxycarbonylmethyl) thiophosphate at four points. Two minutes after intoxication, cats received muscular injection with 0.5 mg/kg atropine sulfate, and then, brain tissues were collected from parietal lobe, basal ganglia, hippocampus, cerebellum and brain stem were observed at 3, 6, 24 hours, 3 and 7 days after intoxication respectively under optic microscope and electron microscope and expressions of acetylcholinesterase (AChE), choline acetyltransferase (ChAT), glial fibrillary acidic protein (GFAP),glutamic acid (Glu) and γ-amino butyric acid after immunohistochemical staining.MAIN OUTCOME MEASURES: Results of MRI examinations; histological changes under optic microscope and electron

  18. Primary percutaneous coronary intervention for acute myocardial infarction in a pediatric patient with giant coronary aneurysm due to Kawasaki disease.

    Science.gov (United States)

    Mongiovì, Maurizio; Alaimo, Annalisa; Vernuccio, Federica; Pieri, Daniele

    2014-01-01

    We report a case of acute myocardial infarction in an 8-year-old boy with a history of Kawasaki disease and giant coronary aneurysms in the right and left coronary arteries. We performed coronary angiography and percutaneous coronary intervention 4 hours after the onset of symptoms. This case suggests that primary percutaneous coronary intervention might be safe and effective in the long-term treatment of acute myocardial infarction due to coronary sequelae of Kawasaki.

  19. 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.

  20. 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.

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

    Science.gov (United States)

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

    2016-08-01

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

  2. 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.

  3. 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

  4. Acute Kidney Injury due to Crescentic Glomerulonephritis in a Patient with Polycystic Kidney Disease

    OpenAIRE

    Maggard, Reuben; Makary, Raafat; Monteiro, Carmela l.; James, Leighton R.

    2013-01-01

    Polycystic kidney disease is an inherited condition, characterized by the development of cysts in the kidney, as well as in other organs. Patients with polycystic kidney can suffer from the same causes of acute kidney injury as the general population. Nephritic syndrome is an uncommon cause of acute kidney injury in the general population and less common in patients with polycystic kidney disease. We report the second case of crescentic glomerulonephritis, causing acute kidney injury, in a pa...

  5. A Case of Recurrent Acute Pancreatitis Due to Intra-Articular Corticosteroid Injection

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    Patompong Ungprasert

    2014-03-01

    Full Text Available Context Corticosteroid is a well-established cause of drug-induced pancreatitis. However, acute pancreatitis from intraarticularinjection of corticosteroid has never been described. Case report A 69-year-old male presented with acuteabdominal pain and was diagnosed with acute pancreatitis. The patient had one episode of acute pancreatitis two yearearlier. Both episodes occurred after intra-articular cortisone injection. Investigations for other causes of pancreatitis werenegative. Conclusion We report the first case of acute pancreatitis from intra-articular corticosteroid injection. Physiciansshould be aware of this adverse reaction of corticosteroid that can even occur with local administration.

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

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    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.

  7. Paraquat poisoning: an experimental model of dose-dependent acute lung injury due to surfactant dysfunction

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    M.F.R. Silva

    1998-03-01

    Full Text Available Since the most characteristic feature of paraquat poisoning is lung damage, a prospective controlled study was performed on excised rat lungs in order to estimate the intensity of lesion after different doses. Twenty-five male, 2-3-month-old non-SPF Wistar rats, divided into 5 groups, received paraquat dichloride in a single intraperitoneal injection (0, 1, 5, 25, or 50 mg/kg body weight 24 h before the experiment. Static pressure-volume (PV curves were performed in air- and saline-filled lungs; an estimator of surface tension and tissue works was computed by integrating the area of both curves and reported as work/ml of volume displacement. Paraquat induced a dose-dependent increase of inspiratory surface tension work that reached a significant two-fold order of magnitude for 25 and 50 mg/kg body weight (P<0.05, ANOVA, sparing lung tissue. This kind of lesion was probably due to functional abnormalities of the surfactant system, as was shown by the increase in the hysteresis of the paraquat groups at the highest doses. Hence, paraquat poisoning provides a suitable model of acute lung injury with alveolar instability that can be easily used in experimental protocols of mechanical ventilation

  8. Acute Myeloid Leukemia Relapse Presenting as Complete Monocular Vision Loss due to Optic Nerve Involvement.

    Science.gov (United States)

    Patel, Shyam A

    2016-01-01

    Acute myeloid leukemia (AML) involvement of the central nervous system is relatively rare, and detection of leptomeningeal disease typically occurs only after a patient presents with neurological symptoms. The case herein describes a 48-year-old man with relapsed/refractory AML of the mixed lineage leukemia rearrangement subtype, who presents with monocular vision loss due to leukemic eye infiltration. MRI revealed right optic nerve sheath enhancement and restricted diffusion concerning for nerve ischemia and infarct from hypercellularity. Cerebrospinal fluid (CSF) analysis showed a total WBC count of 81/mcl with 96% AML blasts. The onset and progression of visual loss were in concordance with rise in peripheral blood blast count. A low threshold for diagnosis of CSF involvement should be maintained in patients with hyperleukocytosis and high-risk cytogenetics so that prompt treatment with whole brain radiation and intrathecal chemotherapy can be delivered. This case suggests that the eye, as an immunoprivileged site, may serve as a sanctuary from which leukemic cells can resurge and contribute to relapsed disease in patients with high-risk cytogenetics. PMID:27668104

  9. Efficacy of extracorporeal albumin dialysis for acute kidney injury due to cholestatic jaundice nephrotoxicity.

    Science.gov (United States)

    Sens, Florence; Bacchetta, Justine; Rabeyrin, Maud; Juillard, Laurent

    2016-01-01

    We report a case of a 37-year-old man with Maturity Onset Diabetes of the Youth (MODY) type 5, admitted for an episode of cholestasis and a simultaneous acute kidney injury (AKI). Chronic liver disease was due to a mutation in the transcription factor 2 (TCF2) gene, thus highlighting the need for a close liver follow-up in these patients. AKI was attributed to a cholemic nephropathy based on the following rationale: (1) alternative diagnoses were actively ruled out; (2) the onset of AKI coincided with the onset of severe hyperbilirubinaemia; (3) renal pathology showed large bile tubular casts and a marked tubular necrosis and (4) creatinine serum dramatically decreased when bilirubin levels improved after the first sessions of extracorporeal albumin dialysis (ECAD), thus suggesting its role in renal recovery. Even though cholestasis can precipitate renal injury, the diagnosis of cholemic nephropathy could require a renal biopsy at times. Future studies should confirm the benefits of ECAD in cholemic nephropathy. PMID:27389722

  10. 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%.

  11. Noninvasive ventilation in acute respiratory failure due to H1N1 influenza

    OpenAIRE

    Mohapatra, Prasanta R.; Naveen Dutt; Sushant Khanduri; Baijayantimala Mishra; Janmeja, Ashok K

    2011-01-01

    We present a case of severe H1N1 influenza with hypoxemic acute respiratory failure necessitating mechanical ventilation benefited from noninvasive positive pressure ventilation (NIPPV). The NIPPV may be of great use in treating patients with H1N1-related acute respiratory distress syndrome in a resource poor setting or when invasive ventilator is unavailable.

  12. Acute respiratory failure due to thyroid storm developing immediately after delivery

    OpenAIRE

    Kitazawa, Chie; Aoki, Shigeru; Takahashi, Tsuneo; Hirahara, Fumiki

    2015-01-01

    Key Clinical Message Acute respiratory failure occurs in less than 0.1% of pregnancies. Thyroid storm should be included in the differential diagnosis of possible causes of acute respiratory failure occurring immediately after delivery, and delivery is a high risk factor for thyroid storm in pregnant women with thyrotoxicosis.

  13. A Rare and Unexpected Side-Effect of Cannabis Use: Abdominal Pain due to Acute Pancreatitis

    OpenAIRE

    Mehmet Husamettin Akkucuk; Mustafa Erbayrak

    2015-01-01

    Acute pancreatitis is a frequently encountered disorder in patients presenting to emergency units. Biliary system disorders, alcohol consumption, infections, and drugs are among the causes of acute pancreatitis. However, it is sometimes difficult to determine the etiology of this disorder, particularly if the patient does not wish to disclose his consumption of cannabis, the use of which is illegal.

  14. Destructive Cholangitis in an Adult Jack Russell Terrier

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    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.

  15. Melioidosis in acute cholangitis of diabetic patient: a forgotten diagnosis

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    Mohamad N

    2012-08-01

    Full Text Available Nasir Mohamad,1 Suresh Ponnusamy,2 Sunita Devi,3 Rishya Manikam,4 Ilya Irinaz Idrus,1 Nor Hidayah Abu Bakar51Department of Emergency Medicine, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Malaysia; 2AIMST University, Bedong, Malaysia; 3Hospital Sultan Abdul Halim, Sungai Petani, Malaysia; 4University Malaya Medical Centre, Kuala Lumpur, Malaysia; 5Department of Pathology, Hospital Raja Perempuan Zainab II, Kota Bharu, MalaysiaAbstract: Melioidosis presents with a wide range of clinical presentations, which include severe community-acquired pneumonia, septicemia, central nervous system infection, and less severe soft tissue infection. Hence, its diagnosis depends heavily on the clinical microbiology laboratory for culture. In this case report, we describe an atypical presentation of melioidosis in a 52-year-old man who had fever, right upper-abdominal pain, and jaundice for 15 days. Melioidosis caused by Burkholderia pseudomallei was subsequently diagnosed from blood culture. As a primary care physician, high suspicion index is of great importance. High suspicion index of melioidosis in a high-risk group patient, such as the patient with diabetes mellitus and diabetic foot, is crucial in view of atypical presentations of pseudomonas sepsis. A correct combination of antibiotic administration in the early phase of therapy will determine its successful outcome.Keywords: Burkholderia pseudomallei, atypical, high suspicion, primary care

  16. Emergency adrenalectomy due to acute heart failure secondary to complicated pheochromocytoma: a case report

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    Padillo Francisco J

    2011-05-01

    Full Text Available Abstract Pheochromocytomas are catecholamine producing tumors arising mostly from chromaffin cells of the adrenal medulla. The most common clinical presentation is hypertension, mainly in the form of paroxymal episodes. Cardiovascular manifestations include malignant arrhythmia and catecholamine cardiomyopathy, mimicking acute coronary syndromes and acute heart failure. There are reports of pheochromocytomas presenting as acute coronary syndrome and rapidly leading to cardiogenic shock; the failure of intensive medical treatment in these cases has prompted the need for emergency adrenalectomy as the only remaining option. We report on a case of complicated pheochromocytoma presenting as cardiogenic shock, in which emergency adrenalectomy was performed following a total lack of response to intensive medical treatment.

  17. Acute Coronary Syndrome Due to Spontaneous Coronary Artery Dissection in a Middle-Aged Man

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    Davran Cicek

    2014-08-01

    Full Text Available True spontaneous coronary artery dissection (SCAD is an extremely rare but important cause of acute coronary syndrome, with only about 200 cases reported in the literature. Diagnosis is often made at autopsy. Risk factors include oral contraceptive use, atherosclerotic disease and the peripartum period. SCAD should be considered when a healthy young patient presents with the onset of acute myocardial ischemic syndrome. A timely diagnosis and intervention are mandatory as SCAD can cause sudden death. We present a case of SCAD with an uncommon clinical presentation of acute coronary syndrome and without identifiable risk factors, and successfully treated with non-invasive (medical therapy.

  18. Thiamine Deficiency in a Developed Country: Acute Lactic Acidosis in Two Neonates Due to Unsupplemented Parenteral Nutrition.

    Science.gov (United States)

    Salvatori, Guglielmo; Mondì, Vito; Piersigilli, Fiammetta; Capolupo, Irma; Pannone, Veronica; Vici, Carlo Dionisi; Rizzo, Cristiano; Dotta, Andrea

    2016-08-01

    Thiamine is a water-soluble vitamin implicated in several metabolic processes. Its deficiency, due to prolonged parenteral nutrition without adequate vitamin supplementation, can lead to multiorgan failure characterized by cardiovascular impairment and metabolic acidosis refractory to bicarbonate administration. Only thiamine administration allows the remission of symptoms. We report 2 preterm infants with acute thiamine deficiency due to prolonged parenteral nutrition without adequate vitamin supplementation.

  19. Acute respiratory distress syndrome due to viral pneumonitis in case of varicella zoster in adult: case report

    OpenAIRE

    Anaz Binazeez; Saurabh Kothari; Dhaval Dave; Manish Pendse; Divya Lala; Smita Patil; Archana Bhate

    2015-01-01

    Chickenpox, is a highly contagious disease caused by infection with varicella zoster virus (VZV). The disease is often more severe in adults than children. Here we present a case of adult male suffering from chicken pox who presented with complication of acute respiratory distress syndrome [ARDS] due to viral pneumonitis. Due to his late presentation, despite of giving antivirals, patient had a fatal outcome. So this case highlights the necessity and importance of early administration of a...

  20. Thiamine Deficiency in a Developed Country: Acute Lactic Acidosis in Two Neonates Due to Unsupplemented Parenteral Nutrition.

    Science.gov (United States)

    Salvatori, Guglielmo; Mondì, Vito; Piersigilli, Fiammetta; Capolupo, Irma; Pannone, Veronica; Vici, Carlo Dionisi; Rizzo, Cristiano; Dotta, Andrea

    2016-08-01

    Thiamine is a water-soluble vitamin implicated in several metabolic processes. Its deficiency, due to prolonged parenteral nutrition without adequate vitamin supplementation, can lead to multiorgan failure characterized by cardiovascular impairment and metabolic acidosis refractory to bicarbonate administration. Only thiamine administration allows the remission of symptoms. We report 2 preterm infants with acute thiamine deficiency due to prolonged parenteral nutrition without adequate vitamin supplementation. PMID:25591974

  1. Acute renal failure due to phenazopyridine (Pyridium) overdose: case report and review of the literature.

    Science.gov (United States)

    Onder, Ali Mirza; Espinoza, Veronica; Berho, Mariana E; Chandar, Jayanthi; Zilleruelo, Gaston; Abitbol, Carolyn

    2006-11-01

    Phenazopyridine (Pyridium) is a commonly used urinary tract analgesic. It has been associated with yellow skin discoloration, hemolytic anemia, methemoglobinemia, and acute renal failure, especially in patients with preexisting kidney disease. We report a 17-year-old female with vertically transmitted human immunodeficiency virus (HIV) infection, presenting with acute renal failure and methemoglobinemia following a suicidal attempt with a single 1,200 mg ingestion of Pyridium. She had no prior evidence of HIV nephropathy. The patient had a progressive nonoliguric renal failure on the 3rd day following the ingestion. She was treated with N-acetylcysteine, intravenous carnitine, and alkalinization of the urine. Her kidney biopsy revealed acute tubular necrosis with no glomerular changes. After 7 days of conservative management, she was discharged home with normal kidney function. To our knowledge, this is the second smallest amount of Pyridium overdose resulting in acute renal failure with no previous history of kidney disease. PMID:16897003

  2. A homosexual japanese man with acute hepatitis due to hepatitis B virus genotype ae, concurrent with amebic colitis

    Directory of Open Access Journals (Sweden)

    Sakaguchi,Kohsaku

    2007-02-01

    Full Text Available We report herein a case with acute hepatitis due to hepatitis B virus genotype Ae, concurrent with amebic colitis. A 39-year-old homosexual Japanese man was admitted to our hospital with jaundice. Laboratory tests showed an elevation of transaminase and positivity for hepatitis B surface antigen and IgM-type antibody to hepatitis B core antigen. The hepatitis B virus genotype was determined to be Ae. Furthermore, a mud-like stool with blood and mucous had sometimes been noted during the past 3 years, and amebic colitis was shown by colonofi berscopy during hospitalization. The patient was diagnosed with acute hepatitis B, concurrent with amebic colitis, and was successfully treated with lamivudine and metronidazole. In Japanese patients with acute hepatitis B virus genotype A infection, homosexual activity tends to be high. Furthermore, in Japanese homosexual men, amebiasis has been increasing. Thus, in Japanese patients with acute hepatitis B, a determination of genotype should be performed in order to investigate the route of transmission of hepatitis B virus, and a search for amebiasis should be performed in patients with acute hepatitis due to hepatitis B virus genotype A. Furthermore, education of homosexual men regarding hepatitis B virus, hepatitis B virus vaccination, and amebiasis is urgently required.

  3. Acute Placental Infection Due to Klebsiella pneumoniae: Report of a Unique Case

    OpenAIRE

    Sheikh, Salwa S.; Amr, Samir S.; Janice M. Lage

    2005-01-01

    A 40-year-old woman, gravida 9, with seven healthy children and a history of one abortion (p 7 + 1) , presented at 18 weeks of gestation with fever and malodorous vaginal discharge. Ultrasound revealed a macerated fetus. The placenta showed acute chorioamnionitis and acute villitis with microabscess formation. Blood and vaginal cultures both grew Klebsiella pneumoniae. This is the first reported case in English literature of Klebsiella pneumoniae causing suppurative placentitis leading to fet...

  4. Acute placental infection due to Klebsiella pneumoniae: report of a unique case.

    OpenAIRE

    Janice M. Lage; Amr, Samir S.; Sheikh, Salwa S.

    2005-01-01

    A 40-year-old woman, gravida 9, with seven healthy children and a history of one abortion (p 7 + 1) , presented at 18 weeks of gestation with fever and malodorous vaginal discharge. Ultrasound revealed a macerated fetus. The placenta showed acute chorioamnionitis and acute villitis with microabscess formation. Blood and vaginal cultures both grew Klebsiella pneumoniae. This is the first reported case in English literature of Klebsiella pneumoniae causing suppurative placentitis leading to fet...

  5. Endoscopic management of afferent loop syndrome after a pylorus preserving pancreatoduodenecotomy presenting with obstructive jaundice and ascending cholangitis.

    Science.gov (United States)

    Kim, Jae Kyung; Park, Chan Hyuk; Huh, Ji Hye; Park, Jeong Youp; Park, Seung Woo; Song, Si Young; Chung, Jaebock; Bang, Seungmin

    2011-09-01

    Afferent loop syndrome is a rare complication of gastrojejunostomy. Patients usually present with abdominal distention and bilious vomiting. Afferent loop syndrome in patients who have undergone a pylorus preserving pancreaticoduodenectomy can present with ascending cholangitis. This condition is related to a large volume of reflux through the biliary-enteric anastomosis and static materials with bacterial overgrowth in the afferent loop. Patients with afferent loop syndrome after pylorus preserving pancreaticoduodenectomy frequently cannot be confirmed as surgical candidates due to poor medical condition. In that situation, a non-surgical palliation should be considered. Herein, we report two patients with afferent loop syndrome presenting with obstructive jaundice and ascending cholangitis. The patients suffered from the recurrence of pancreatic cancer after pylorus preserving pancreaticoduodenectomy. The diagnosis of afferent loop syndrome was confirmed, and the patients were successfully treated by inserting an endoscopic metal stent using a colonoscopic endoscope. PMID:22741115

  6. Biliary emergencies: pancreatitis, cholangitis, and more.

    Science.gov (United States)

    Mitchell, Robert M S; Byrne, Michael F

    2003-04-01

    The most common cause of acute pancreatitis is gallstones, although many other etiological factors have been identified. The management of the initial episode depends on the severity of the attack and the etiology. In most patients, acute pancreatitis has a benign, self-limited course. However, in the minority who develop infected pancreatic necrosis the mortality can reach 25%. The early assessment of severity and aggressive management of these patients is critical. The roles of endoscopic retrograde cholangiopancreatography, surgical intervention, enteral feeding and use of antibiotics in acute pancreatitis are discussed in this article. Finally, the origin of recurrent acute pancreatitis is discussed, with particular reference to conditions such as pancreas divisum and sphincter of Oddi dysfunction whose role in the development of acute pancreatitis is controversial, and to hereditary or familial pancreatitis.

  7. 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

  8. 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

  9. 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

  10. 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.

  11. 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

  12. 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

  13. 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.

  14. Validation of a cholangiographic prognostic model in primary sclerosing cholangitis

    NARCIS (Netherlands)

    C.Y. Ponsioen; J.B. Reitsma; K.M. Boberg; L. Aabakken; E.A. Rauws; E. Schrumpf

    2010-01-01

    Background and study aims: We previously developed a prognostic model for primary sclerosing cholangitis (PSC), which was primarily based on a cholangiographic classification of the intra- and extrahepatic biliary tree lesions. The aim of the present study was to validate the performance of this mod

  15. Continuous Regional Arterial Infusion Therapy for Acute Necrotizing Pancreatitis Due to Mycoplasma pneumoniae Infection in a Child

    International Nuclear Information System (INIS)

    A case of acute necrotizing pancreatitis due to Mycoplasma pneumoniae infection was treated in an 8-year-old girl. She experienced acute pancreatitis during treatment for M. pneumoniae. Contrast-enhanced computed tomographic scan revealed necrotizing pancreatitis. The computed tomographic severity index was 8 points (grade E). A protease inhibitor, ulinastatin, was provided via intravenous infusion but was ineffective. Continuous regional arterial infusion therapy was provided with gabexate mesilate (FOY-007, a protease inhibitor) and meropenem trihydrate, and the pancreatitis improved. This case suggests that infusion therapy is safe and useful in treating necrotizing pancreatitis in children.

  16. Acute ischemic stroke in a child due to basilar artery occlusion treated successfully with a stent retriever.

    Science.gov (United States)

    Savastano, Luis; Gemmete, Joseph J; Pandey, Aditya S; Roark, Christopher; Chaudhary, Neeraj

    2016-08-01

    Ischemic strokes in childhood are rare. Thrombolytic therapy with intravenous (IV) tissue plasminogen activator (tPA) has been the main intervention for the management of pediatric stroke patients, but safety data are lacking and efficacy has been questioned. Recently, successful endovascular treatments for acute ischemic stroke in children have been reported with increasing frequency, suggesting that mechanical thrombectomy can be a safe and effective treatment. We present the case of a 22-month-old child with acute ischemic stroke due to basilar artery occlusion that was successfully treated with a stent retriever. PMID:26156170

  17. 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.

  18. 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.

  19. 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

  20. Acute sensorineural hearing loss and severe otalgia due to scrub typhus

    Directory of Open Access Journals (Sweden)

    Kim Dong-Min

    2009-10-01

    Full Text Available Abstract Background Scrub typhus is an acute febrile illness caused by Orientia tsutsugamushi. Case presentations We encountered a patient with sensorineural hearing loss complicating scrub typhus, and three patients with scrub typhus who complained of otalgia, which was sudden onset, severe, paroxysmal, intermittent yet persistent pain lasting for several seconds, appeared within 1 week after the onset of fever and rash. The acute sensorineural hearing loss and otalgia were resolved after antibiotic administration. Conclusion When patients in endemic areas present with fever and rash and have sensorineural hearing loss or otalgia without otoscopic abnormalities, clinicians should suspect scrub typhus and consider empirical antibiotic therapy.

  1. A porcine model of acute, haematogenous, localized osteomyelitis due to Staphylococcus aureus

    DEFF Research Database (Denmark)

    Johansen, Louise Kruse; Frees, Dorte; Aalbæk, Bent;

    2011-01-01

    A porcine model of acute, haematogenous, localized osteomyelitis was established. Serial dilutions of Staphylococcus aureus [5–50–500–5000–50 000 CFU/kg body weight (BW) suspended in saline or saline alone] were inoculated into the right brachial artery of pigs (BW 15 kg) separated into six groups...... developed microabscesses in bones of the infected legs. In the centre of microabscesses, S. aureus was regularly demonstrated together with necrotic neutrophils. Often, bone lesions resulted in trabecular osteonecrosis. The present localized model of acute haematogenous osteomyelitis revealed a pattern of...

  2. Acute Hemolysis with Renal Failure due to Clostridium Bacteremia in a Patient with AML

    Science.gov (United States)

    Medrano-Juarez, R. M.; Sotello, D.; D'Cuhna, L.; Payne, J. D.

    2016-01-01

    We present a case of acute hemolytic anemia, renal failure, and Clostridium perfringens bacteremia in a patient with acute myelogenous leukemia. The high fatality of C. perfringens bacteremia requires that clinicians recognize and rapidly treat patients at risk for this infection. Although other hemolytic processes are in the differential diagnosis of these events, the presence of high fever, chills, and rapidly positive blood cultures may help narrow the diagnosis. Most cases of C. perfringens bacteremia have a concomitant coinfection, which makes broad spectrum empiric therapy essential. There is a high mortality rate of C. perfringens infections associated with leukemia.

  3. Pretransplant HLA mistyping in diagnostic samples of acute myeloid leukemia patients due to acquired uniparental disomy

    NARCIS (Netherlands)

    Dubois, V.; Sloan-Bena, F.; Cesbron, A.; Hepkema, B. G.; Gagne, K.; Gimelli, S.; Heim, D.; Tichelli, A.; Delaunay, J.; Drouet, M.; Jendly, S.; Villard, J.; Tiercy, J-M

    2012-01-01

    Although acquired uniparental disomy (aUPD) has been reported in relapse acute myeloid leukemia (AML), pretransplant aUPD involving chromosome 6 is poorly documented. Such events could be of interest because loss of heterozygosity (LOH) resulting from aUPD in leukemic cells may lead to erroneous res

  4. [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.

  5. Endovascular stent graft treatment of acute thoracic aortic transections due to blunt force trauma.

    LENUS (Irish Health Repository)

    Bjurlin, Marc A

    2012-02-01

    Endovascular stent graft treatment of acute thoracic aortic transections is an encouraging minimally invasive alternative to open surgical repair. Between 2006 and 2008, 16 patients with acute thoracic aortic transections underwent evaluation at our institution. Seven patients who were treated with an endovascular stent graft were reviewed. The mean Glasgow Coma Score was 13.0, probability of survival was .89, and median injury severity score was 32. The mean number of intensive care unit days was 7.7, mean number of ventilator support days was 5.4, and hospital length of stay was 10 days. Mean blood loss was 285 mL, and operative time was 143 minutes. Overall mortality was 14%. Procedure complications were a bleeding arteriotomy site and an endoleak. Endovascular treatment of traumatic thoracic aortic transections appears to demonstrate superior results with respect to mortality, blood loss, operative time, paraplegia, and procedure-related complications when compared with open surgical repair literature.

  6. Prolonged dysphagia due to Listeria-rhombencephalitis with brainstem abscess and acute polyradiculoneuritis.

    Science.gov (United States)

    Smiatacz, Tomasz; Kowalik, Maciej Michal; Hlebowicz, Maria

    2006-06-01

    We report a case of previously healthy student with acute rhombencephalitis and brainstem abscess caused by Listeria monocytogenes. The disease begun with uncharacteristic prodromal symptoms of gastrointestinal infection followed by headache and vertigo. After hospital admission the patient rapidly deteriorated, presenting pronounced dysphagia and respiratory failure requiring mechanical ventilation. The diagnosis was established upon clinical symptoms of infection, brainstem involvement, typical MRI findings and positive for L. monocytogenes blood culture. Infection was complicated by acute, demyelinating neuropathy, diagnosed upon clinical symptoms of frail palsy confirmed by ENG. Initially introduced empirical doxycyclin/ceftriaxon treatment was subsequently changed to targeted ampicillin/gentamycin therapy, mechanical ventilation, intravenous human immunoglobulin treatment, tracheostomy and endoscopic gastrostomy. Prolonged dysphagia resolved after rehabilitation. After one year the patient remains well with only slight dysmetria. PMID:16260041

  7. Lingual Haematoma due to Tenecteplase in a Patient with Acute Myocardial Infarction

    Directory of Open Access Journals (Sweden)

    Muhlis Bal

    2013-01-01

    Full Text Available The use of intravenous thrombolytic agents has revolutionised the treatment of acute myocardial infarction. However, the improvement in mortality rate achieved with these drugs is tempered by the risk of serious bleeding complications, including intracranial haemorrhage. Tenecteplase is a genetically engineered mutant tissue plasminogen activator. Haemorrhagic complications of tissue plasminogen activator (tPA are well known. Compared to other tPAs, tenecteplase use leads to lower rates of bleeding complications. Here, we report a case of unusual site of spontaneous bleeding, intralingual haematoma during tenecteplase therapy following acute myocardial infarction, which caused significant upper airway obstruction and required tracheotomy to maintain the patient’s airway. Clinical dilemmas related to securing the airway or reversing the effects of tissue plasminogen activator are discussed.

  8. Erythema multiforme due to arsenic trioxide in a case of acute promyelocytic leukemia: A diagnostic challenge

    Directory of Open Access Journals (Sweden)

    Girish V Badarkhe

    2016-01-01

    Full Text Available Erythema multiforme (EM is an acute, self-limited, Type IV hypersensitivity reactions associated with infections and drugs. In this case of acute promyelocytic leukemia, EM diagnosed during the induction phase was mistakenly attributed to vancomycin used to treat febrile neutropenia during that period. However, the occurrence of the lesions of EM again during the consolidation phase with arsenic trioxide (ATO lead to a re-evaluation of the patient and both the Naranjo and World Health Organization-Uppsala Monitoring Centre scale showed the causality association as “probable.” The rash responded to topical corticosteroids and antihistamines. This rare event of EM being caused by ATO may be attributed to the genetic variation of methyl conjugation in the individual which had triggered the response, and the altered metabolic byproducts acted as a hapten in the subsequent keratinocyte necrosis.

  9. Erythema multiforme due to arsenic trioxide in a case of acute promyelocytic leukemia: A diagnostic challenge.

    Science.gov (United States)

    Badarkhe, Girish V; Sil, Amrita; Bhattacharya, Sabari; Nath, Uttam Kumar; Das, Nilay Kanti

    2016-01-01

    Erythema multiforme (EM) is an acute, self-limited, Type IV hypersensitivity reactions associated with infections and drugs. In this case of acute promyelocytic leukemia, EM diagnosed during the induction phase was mistakenly attributed to vancomycin used to treat febrile neutropenia during that period. However, the occurrence of the lesions of EM again during the consolidation phase with arsenic trioxide (ATO) lead to a re-evaluation of the patient and both the Naranjo and World Health Organization-Uppsala Monitoring Centre scale showed the causality association as "probable." The rash responded to topical corticosteroids and antihistamines. This rare event of EM being caused by ATO may be attributed to the genetic variation of methyl conjugation in the individual which had triggered the response, and the altered metabolic byproducts acted as a hapten in the subsequent keratinocyte necrosis. PMID:27114640

  10. Acute liver failure due to primary amyloidosis in a nephrotic syndrome: a swiftly progressive course.

    Science.gov (United States)

    Cardoso, Brigite Aguiar; Leal, Rita; Sá, Helena; Campos, Mário

    2016-01-01

    AL amyloidosis is a clonal plasma cell proliferative disorder characterised by extracellular tissue deposits of insoluble fibrils derived from κ or λ immunoglobulin light chains. The most common organs affected by AL amyloidosis are the kidney, presenting with nephrotic syndrome and/or progressive renal dysfunction, and the heart, with restrictive cardiomyopathy. Hepatic deposition of fibrils occurs in half the cases but the liver is rarely the predominantly affected organ. The most common presentation of hepatic amyloidosis is hepatomegaly with elevated alkaline phosphatase. Acute liver failure with cholestasis and jaundice is a rare complication, with a prevalence of approximately 5%, and is usually associated with a worse prognosis. We report a case of a 39-year-old man admitted to our nephrology department with an unusual presentation of primary amyloidosis with nephrotic syndrome and acute liver failure, complicated by obstructive cholestasis resulting in death 2 months after diagnosis. PMID:26965175

  11. Severe Acute Pancreatitis Due to Tamoxifen-Induced Hypertriglyceridemia with Positive Rechallenge

    OpenAIRE

    Jaballah Sakhri; Houssem Harbi; Rached Ltaief; Chaker Ben Salem; Neila Fathallah

    2010-01-01

    Context Pancreatitis is a very rare adverse effect of tamoxifen with only six cases of tamoxifen-associated pancreatitis reported in the English literature until now. In these cases, rechallenge with tamoxifen was not carried out. Case report We report a case of recurrent severe acute pancreatitis in a 44-year-old female induced by tamoxifen therapy and review the literature with regards to tamoxifen-associated pancreatitis. Conclusion Clinicians should be aware of the risks of developing sev...

  12. Acute kidney injury and disseminated intravascular coagulation due to mercuric chloride poisoning

    OpenAIRE

    Dhanapriya, J.; Gopalakrishnan, N; Arun, V.; Dineshkumar, T.; Sakthirajan, R.; Balasubramaniyan, T.; Haris, M

    2016-01-01

    Mercury is a toxic heavy metal and occurs in organic and inorganic forms. Inorganic mercury includes elemental mercury and mercury salts. Mercury salts are usually white powder or crystals, and widely used in indigenous medicines and folk remedies in Asia. Inorganic mercury poisoning causes acute kidney injury (AKI) and gastrointestinal manifestations and can be life-threatening. We describe a case with unknown substance poisoning who developed AKI and disseminated intravascular coagulation (...

  13. Acute liver failure due to Human Herpesvirus 6 in an infant

    OpenAIRE

    G.M. Tronconi; B. Mariani; R. Pajno; M. Fomasi; L. Cococcioni; Biffi, V.; Bove, M.; P. Corsin; G. Garbetta; Barera, G

    2012-01-01

    We report a case of a 4-months infant with fever in the absence of other specific symptoms that has rapidly and unexpectedly developed acute liver failure (ALF) with coagulopathy and complicated with bone marrow failure without encephalopathy. The main viral infection agents (hepatitis virus A, B, C, Citomegalovirus, Ebstain Barr virus, Parvovirus B19, Adenovirus), drug-induced hepatotoxicity and metabolic disorders associated to ALF were excluded. Quantitative determination of Human Herpesvi...

  14. Acute respiratory distress syndrome due to pulmonary involvement by neoplastic plasma cells in multiple myeloma

    OpenAIRE

    Marmor, D B; Farber, J. L.; Gottlieb, J E

    2006-01-01

    Pulmonary involvement with multiple myeloma occurs infrequently and may be difficult to distinguish from more common primary lung tumours, metastatic disease, or other pleural and parenchymal abnormalities. A patient who developed acute respiratory distress syndrome (ARDS) was subsequently found to have multiple myeloma with involvement of lung parenchyma by neoplastic plasma cells. Only one other report of ARDS in association with multiple myeloma was found, and there are no previous reports...

  15. Acute respiratory distress syndrome due to viral pneumonitis in case of varicella zoster in adult: case report

    Directory of Open Access Journals (Sweden)

    Anaz Binazeez

    2015-12-01

    Full Text Available Chickenpox, is a highly contagious disease caused by infection with varicella zoster virus (VZV. The disease is often more severe in adults than children. Here we present a case of adult male suffering from chicken pox who presented with complication of acute respiratory distress syndrome [ARDS] due to viral pneumonitis. Due to his late presentation, despite of giving antivirals, patient had a fatal outcome. So this case highlights the necessity and importance of early administration of antivirals, especially in adult pox, to tackle the complications of disease and get a favourable outcome. [Int J Res Med Sci 2015; 3(12.000: 3924-3927

  16. 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.

  17. 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.

  18. 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.

  19. Barr humbug: acute cerebellar ataxia due to Epstein-Barr virus.

    Science.gov (United States)

    Davies, Benjamin; Machin, Nicholas; Lavin, Timothy; Ul Haq, Mian Ayaz

    2016-01-01

    Epstein-Barr virus (EBV) infection is associated with neurological sequellae, but rarely there is acute cerebellar ataxia (ACA) in an adult. We present a novel case of a 26-year-old man, who presented with ACA. He had normal MRI and CSF analysis. Serum testing confirmed active EBV. A course of oral prednisolone 1 mg/kg for 4 weeks, with a subsequent taper was started. He made a full recovery within 3 weeks of presentation. PMID:27558189

  20. Acute renal failure due to rhabdomyolysis following dengue viral infection: a case report

    OpenAIRE

    Wijesinghe, Aruna; Gnanapragash, Nanthini; Ranasinghe, Gayan; Ragunathan, Murugapillai K

    2013-01-01

    Introduction With more than one-third of the world’s population living in areas at risk for transmission, dengue fever is a leading cause of illness and death in the tropics and subtropics. Despite the high incidence of dengue fever, rhabdomyolysis leading to acute renal failure is an extremely rare complication of dengue fever. Only a few such cases have been reported in the literature. Case presentation We describe the case of a 42-year-old, previously healthy Sri Lankan Sinhalese man who d...

  1. Acute heart failure associated with congenital complete atrioventricular block due to neonatal lupus: case series report

    International Nuclear Information System (INIS)

    Neonatal lupus (NL) defines a set of clinical syndromes characterized by maternal autoantibodies against the RNA protein complex (Ro/SSA or La/SSB) that cross the placenta and potentially lead to fetal tissue damage. Little is known about other cardiac manifestations of NL different from congenital heart block (CHB), as heart failure (HF). Four cases of LN with BCC and acute HF at the first three days of life on average were reported. No complications or deaths were reported at 4,9 months of follow-up. All infants had anti-Ro high titles at the time of diagnosis.

  2. 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

  3. Cases of acute gastroenteritis due to calicivirus in outbreaks: clinical differences by age and aetiological agent.

    Science.gov (United States)

    Sala, M R; Broner, S; Moreno, A; Arias, C; Godoy, P; Minguell, S; Martínez, A; Torner, N; Bartolomé, R; de Simón, M; Guix, S; Domínguez, A

    2014-08-01

    The Caliciviridae family includes norovirus and sapovirus, which both cause acute gastroenteritis (AGE). Currently, norovirus is the most common cause of AGE in all age groups in many countries. We analysed clinical differences in reported cases of acute gastroenteritis caused by caliciviruses (AGC) by age group and agent involved. We conducted a descriptive study of AGE outbreaks reported to the Public Health Agency of Catalonia (Spain) in 2010 and 2011. The odds ratios (ORs) and corresponding 95% confidence intervals (CIs) were calculated to estimate the association between clinical symptoms and age. Clinical differences between the clinical manifestations of sapovirus and norovirus infection in children aged cases caused by norovirus more frequently presented with vomiting and fever (p cases caused by sapovirus more frequently presented with diarrhoea (p 0.013). Determination of the clinical differences associated with cases in outbreaks according to the age of the majority of cases and the symptoms most frequently detected may aid decision making and guide aetiological investigations and the adoption of prevention and control measures.

  4. Acute renal failure due to abdominal compartment syndrome: report on four cases and literature review

    Directory of Open Access Journals (Sweden)

    Cleva Roberto de

    2001-01-01

    Full Text Available We report on 4 cases of abdominal compartment syndrome complicated by acute renal failure that were promptly reversed by different abdominal decompression methods. Case 1: A 57-year-old obese woman in the post-operative period after giant incisional hernia correction with an intra-abdominal pressure of 24 mm Hg. She was sedated and curarized, and the intra-abdominal pressure fell to 15 mm Hg. Case 2: A 73-year-old woman with acute inflammatory abdomen was undergoing exploratory laparotomy when a hypertensive pneumoperitoneum was noticed. During the surgery, enhancement of urinary output was observed. Case 3: An 18-year-old man who underwent hepatectomy and developed coagulopathy and hepatic bleeding that required abdominal packing, developed oliguria with a transvesical intra-abdominal pressure of 22 mm Hg. During reoperation, the compresses were removed with a prompt improvement in urinary flow. Case 4: A 46-year-old man with hepatic cirrhosis was admitted after incisional hernia repair with intra-abdominal pressure of 16 mm Hg. After paracentesis, the intra-abdominal pressure fell to 11 mm Hg.

  5. Clinicoradiological Correlation of Macropsia due to Acute Stroke: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Mayra Johana Montalvo

    2014-01-01

    Full Text Available Dysmetropsia (macropsia, micropsia, teleopsia, or pelopsia most commonly results from retinal pathologies, epileptic seizure, neoplastic lesions, viral infection, or psychoactive drugs. Vascular lesions are an uncommon cause of dysmetropsia. Vascular hemimicropsia, although rare, has been more frequently described in the literature, whereas hemimacropsia from acute ischemic injury is exceedingly rare. We describe a patient presenting in the emergency room (ER with visual perception disturbances characterized by a distorted perception of the size of objects, compatible with left hemimacropsia. Magnetic resonance imaging (MRI of the brain showed an acute occipitotemporal ischemic injury corresponding to the posterior cerebral artery (PCA territory. The location of the lesion is consistent with previous case reports that suggest that hemimacropsia is associated with the occipitotemporal projection, which plays a decisive role in the visual identification of objects by interconnecting the striate, prestriate, and inferior temporal areas. The difference of our case as compared to previous case reports is that the lesion in our patient spared Brodmann area 17 (calcarine cortex and therefore did not present symptoms of quadrantanopsia. Instead, the patient presented isolated hemimacropsia, therefore suggesting that the anatomical lesion causing hemimacropsia is located in the ventral portion of the occipitotemporal projection, more specifically Brodmann areas 18 (parastriate and 19 (peristriate.

  6. Pulmonary fibrosis following pneumonia due to acute Legionnaires' disease. Clinical, ultrastructural, and immunofluorescent study.

    Science.gov (United States)

    Chastre, J; Raghu, G; Soler, P; Brun, P; Basset, F; Gibert, C

    1987-01-01

    During a recent nosocomial outbreak, 20 critically ill patients with acute Legionnaires' disease were admitted to the intensive care unit of Hopital Bichat, Paris. Pulmonary specimens were obtained at surgery or immediately after death in 12 patients and were examined by light, immunofluorescent, and electron microscopy. Five of these 12 patients showed evidence of pulmonary fibrosis. In all of these five patients, infection with Legionella pneumophila was evidenced by bacteriologic methods, and other diseases known to cause fibrosis were excluded. The condition of four patients deteriorated rapidly with respiratory failure, and they died with pulmonary fibrosis. Only one patient finally recovered but was left with pulmonary sequelae. Two distinctive morphologic patterns were observed, one in which interstitial fibrosis was predominant and one in which intra-alveolar organization and fibrosis were also present. The alveolar epithelial lining and the basement membranes were disrupted in all patients, as evidenced by ultrastructural observations and by immunofluorescent studies showing gaps in the distribution of type 4 collagen and laminin. Types 1 and 3 collagen accumulated in areas corresponding to thickened interstitium and intra-alveolar fibrosis. Thus, some patients who survive the acute pneumonia of Legionnaires' disease may develop pulmonary fibrosis, and this process may lead to functional impairment or death despite prompt and appropriate treatment. PMID:3539546

  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. Cerebral blood flow and liver function in patients with encephalopathy due to acute and chronic liver diseases

    DEFF Research Database (Denmark)

    Almdal, T; Schroeder, T; Ranek, L

    1989-01-01

    The purpose of the present investigation was to study changes in cerebral blood flow (CBF) in hepatic encephalopathy, to ascertain whether this was related to the changes in liver function and whether these changes gave any prognostic information. CBF, determined by the intravenous xenon-133 method......, and liver functions, assessed by the prothrombin index, bilirubin concentration, and the galactose elimination capacity, were studied in patients with acute fulminant liver failure and in patients with encephalopathy due to chronic liver diseases--that is, cirrhosis of various etiologies. The CBF range...... any differences between patients with acute or chronic liver diseases or the different degrees of hepatic encephalopathy. In conclusion, a marked reduction of the CBF was seen in hepatic encephalopathy, irrespective of the etiology of the disease....

  9. Sindrome confusional agudo por abstinencia aguda de nicotina Delirium due to acute nicotine withdrawal

    Directory of Open Access Journals (Sweden)

    Manuel Klein

    2002-08-01

    Full Text Available El síndrome confusional agudo (SCA o delirium en pacientes hospitalizados es un problema frecuente y grave. Se caracteriza por síntomas de comienzo agudo y curso fluctuante con inatención, pensamiento desorganizado, y con distintos niveles de alteración de la conciencia.En la bibliografía consultada, el SCA como manifestación de un síndrome de abstinencia aguda nicotínica fue descripto en solo ocho casos. Presentamos el caso de un tabaquista grave que, internado por una reagudización de su enfermedad pulmonar obstructiva crónica (EPOC, presentó un cuadro de SCA al tercer día de abstinencia tabacal, cediendo los síntomas tras la administración de un parche de nicotina. Lo descripto sugiere que en pacientes internados que presentan SCA y agitación, con fuertes antecedentes de tabaquismo, un simple ensayo con un parche de nicotina puede ofrecer en pocas horas una notable respuesta terapéutica y a su vez un test confirmatorio. El reconocimiento del SCA como forma de presentación de la abstinencia nicotínica permitirá identificar casos habitualmente complejos en los que se podrá implementar una sencilla y eficaz alternativa terapéutica.Delirium or acute confusional state among hospitalized patients is a frequent and serious problem. It is characterized by acute onset symptoms, fluctuating course, impaired attention, unorganized thinking, and altered level of conciousness. Delirium, as a manifestation of acute nicotine withdrawal syndrome has been reported in the reviewed literature only in eight cases. We report the case of a heavy smoker admitted because of a reagudization of his chronic obstructive pulmonary disease. At the third day of nicotine abstinence, he developed delirium with a rapid improvement of his symptoms after treatment with a transdermal nicotine patch. This description suggests that in hospitalized heavy smokers who develop delirium with agitation, a simple trial with a nicotine patch can offer a dramatic

  10. Liver transplantation for acute hepatic failure due to chemotherapy-induced HBV reactivation in lymphoma patients

    Institute of Scientific and Technical Information of China (English)

    Timothée Noterdaeme; Luc Longrée; Christian Bataille; Arnaud Deroover; Anne Lamproye; Jean Delwaide; Yves Beguin; Pierre Honoré; Olivier Detry

    2011-01-01

    Hepatitis B (HBV) reactivation induced by chemotherapy is problem encountered recently in the management of malignant diseases. Chemotherapy-induced HBV reactivation may ultimately lead to terminal acute liver failure. Liver transplantation (LT) currently remains the only definitive treatment option for such cases, but is generally denied to patients suffering from malignancy. Here, the authors describe 2 cases of cancer-free and HBV graft re-infection-free survival after LT performed for terminal liver failure arising from HBV reactivation induced by chemotherapy for advanced stage lymphoma. These 2 cases, and some other reports in the literature, may suggest that patients suffering from hematologic malignancies and terminal liver disease can be considered for LT if the prognosis of their hematologic malignancy is good.

  11. Acute Myocardial Infarction in a Young Lady due to Vitamin B12 Deficiency Induced Hyperhomocysteinemia

    Science.gov (United States)

    Shamkani, Warkaa Al; Jafar, Nagham Saeed; Narayanan, Sunil Roy; Rajappan, Anil Kumar

    2015-01-01

    Hyper-homocysteinemia is a risk factor for coronary artery disease in young patients. A 32 years old female without any conventional risk factors except obesity presented with acute anterior wall myocardial infarction (MI). Her echocardiography showed anterior wall hypokinesia with moderate left ventricular dysfunction. Angiography showed tight stenosis of the proximal left anterior descending (LAD) and borderline lesion in left circumflex coronary artery (LCX). She underwent percutaneous coronary intervention (PCI) to LAD with good result. Her blood tests showed low vitamin B12, folate and serum iron levels and elevated serum homocysteine level. She was given folic acid and vitamin B12 and her homocysteine levels normalized. This case demonstrates that hyperhomocysteinemia caused by nutritional deficiency of vitamin co factors may lead to MI. Hyperhomocysteinemia should be considered in the evalauation of young people with MI, especially those without conventional risk factors. PMID:25838876

  12. Acute pulmonary edema following liposuction due to heart failure and atypical pneumonia.

    Science.gov (United States)

    Wollina, Uwe; Graf, Andreas; Hanisch, Volkmar

    2015-05-01

    Microcannular liposuction in tumescent anesthesia is the most effective treatment for painful lipedema. Tumescent anesthesia is an established and safe procedure in local analgesia when performed according to guidelines. Major adverse effects are rare. In patients with advanced lipedema, however, the commonly presented comorbidities bear additional risks.We report on post-surgical acute pulmonary edema after tumescent liposuction according to guidelines in a 52-year-old female patient with lipedema of the legs. We discuss in detail possible scenarios that might be involved in such emergency. In the present case the most likely was a retarded community acquired atypical pneumonia with aggravation of pre-existent comorbidities.A combined treatment with intravenous b-lactam antibiosis, positive pressure ventilation, and continuous venovenous hemodialysis and filtration resulted in complete remission in a couple of days. In conclusion, tumescent liposuction of advanced lipedema patients should only be performed in well-trained centers with sufficient infrastructure.

  13. Intervention analysis of introduction of rotavirus vaccine on hospital admissions rates due to acute diarrhea

    Directory of Open Access Journals (Sweden)

    Maria de Lourdes Teixeira Masukawa

    2014-10-01

    Full Text Available The aim of this study is to investigate the impact of rotavirus vaccine on hospitalization rates for acute diarrhea in children younger than 5 years old after the introduction of the vaccine in 2006. A descriptive analytical observational study was carried out of the hospitalization rates occurred between 2000 and 2011 in 22 Regional Health Centers of Paraná State, Brazil. The effect of the vaccine was assessed by applying the SARIMA/Box-Jenkins time series methodology of intervention analysis, which allows verifying the slopes of the series are different after the introduction of the vaccine and estimating the magnitude of these effects for children younger than five years of age, by age group, for each region center. It was verified a statistically significant reduction by center/month on hospitalization rates for children 1 year old and younger, with averages of 47% and 58%, respectively, in December 2011.

  14. Acute hepatitis due to hepatitis A virus subgenotype IA as an imported infectious disease from Indonesia.

    Science.gov (United States)

    Utsumi, Takako; Yano, Yoshihiko; Amin, Mochamad; Lusida, Maria I; Soetjipto; Hotta, Hak; Hayashi, Yoshitake

    2014-10-01

    A 25-year-old Japanese man was admitted with general malaise and fever, which had developed 12 days after coming back to Japan from Indonesia. Blood examination revealed elevated transaminase levels and positivity for the IgM anti-HAV antibody; therefore, he was diagnosed with acute hepatitis A. HAV-RNA was detected in his serum and phylogenetically classified as subgenotype IA. The partial genome in the VP1/P2A region was consistent with the strain recently isolated from Surabaya, which indicated that he had been infected during his stay in Indonesia. Thus, HAV vaccination is recommended before visiting HAV-endemic countries for a long period of time.

  15. Acute Myocardial Infarction in a Young Lady due to Vitamin B12 Deficiency Induced Hyperhomocysteinemia

    Directory of Open Access Journals (Sweden)

    Warkaa Al Shamkani

    2015-01-01

    Full Text Available Hyper-homocysteinemia is a risk factor for coronary artery disease in young patients. A 32 years old female without any conventional risk factors except obesity presented with acute anterior wall myocardial infarction (MI. Her echocardiography showed anterior wall hypokinesia with moderate left ventricular dysfunction. Angiography showed tight stenosis of the proximal left anterior descending (LAD and borderline lesion in left circumflex coronary artery (LCX. She underwent percutaneous coronary intervention (PCI to LAD with good result. Her blood tests showed low vitamin B12, folate and serum iron levels and elevated serum homocysteine level. She was given folic acid and vitamin B12 and her homocysteine levels normalized. This case demonstrates that hyperhomocysteinemia caused by nutritional deficiency of vitamin co factors may lead to MI. Hyperhomocysteinemia should be considered in the evalauation of young people with MI, especially those without conventional risk factors.

  16. Acute Myocardial Infarction in a Young Lady due to Vitamin B12 Deficiency Induced Hyperhomocysteinemia.

    Science.gov (United States)

    Shamkani, Warkaa Al; Jafar, Nagham Saeed; Narayanan, Sunil Roy; Rajappan, Anil Kumar

    2015-01-01

    Hyper-homocysteinemia is a risk factor for coronary artery disease in young patients. A 32 years old female without any conventional risk factors except obesity presented with acute anterior wall myocardial infarction (MI). Her echocardiography showed anterior wall hypokinesia with moderate left ventricular dysfunction. Angiography showed tight stenosis of the proximal left anterior descending (LAD) and borderline lesion in left circumflex coronary artery (LCX). She underwent percutaneous coronary intervention (PCI) to LAD with good result. Her blood tests showed low vitamin B12, folate and serum iron levels and elevated serum homocysteine level. She was given folic acid and vitamin B12 and her homocysteine levels normalized. This case demonstrates that hyperhomocysteinemia caused by nutritional deficiency of vitamin co factors may lead to MI. Hyperhomocysteinemia should be considered in the evalauation of young people with MI, especially those without conventional risk factors.

  17. Acute respiratory failure, due to severe obstructive sleep apnoea syndrome, managed with nasal positive pressure ventilation.

    Science.gov (United States)

    Sturani, C; Galavotti, V; Scarduelli, C; Sella, D; Rosa, A; Cauzzi, R; Buzzi, G

    1994-12-01

    The complications of endotracheal intubation are particularly frequent in patients with obstructive sleep apnoea syndrome (OSAS). We prospectively tested nasal ventilation in such patients admitted for acute respiratory failure. Six consecutive patients, aged 17-70 yrs, were selected for the study. All patients were confused or severely obtunded, Glasgow Coma Score (GCS) 10 (SD 2). With nasal bi-level positive airways pressure (BiPAP) all these patients improved clinical status and arterial blood gas values, avoiding intubation and invasive mechanical ventilation. The median pH increased from 7.26 (SD 0.06) to 7.36 (0.01) and to 7.43 (0.02) after, 1-3 and 24 h of nasal ventilation, respectively. Nasal ventilation lasted an average of 21 (3) h on the first day. All patients were discharged home after a median hospital stay of 28 (11) days. PMID:7711717

  18. How to reduce avoidable admissions due to acute diabetes complications?: Interrelation between primary and specialized attention in a diabetes unit

    Directory of Open Access Journals (Sweden)

    N. V. García-Talavera Espín

    2012-12-01

    Full Text Available Introduction: Type 2 Diabetes Mellitus is a serious health problem. In the year 2030 it will affect 366 million people around the world. Objective: Evaluate the effectiveness of a mixed intervention and reducing the amount and seriousness of acute complications in diabetics from our Health Area. Materials and method: Protocols of action as well as information documents were produced. Diabetes Unit coordinated educational activities in the different support levels of the Area VII of Murcia. Information talks were provided for the people in charge of the Diabetes Unit in every Care Center and Service of the Health Area. Personalized training was provided for patients treated in the differet Care levels. The study comprised three stages. Information leaflets were spread and talks offered to the patient regarding in house handling of hypo and hyper glycemia. Results: A reduction of 39% of the emergencies due to acute non complicated diabetes was achieved, as well as a reduction of 47.6% of hospital admissions. There was a reduction of 67.8% of the amount of total hospital stays for the group of patients under 35 years who were admitted into the hospital due to type 1 or 2 diabetes mellitus that didn't show any complications (GRD295. Conclusions: There was a reduction of more than thirty percent in the emergencies due to acute decompensations in the disease and a significant reduction in the avoidable hospital stays in the young adult, thus improving the patients' life quality and reducing the social cost of the diabetic patient.

  19. Reconstructive procedure of lower lip defect due to ecthyma gangrenosum--a rare complication of acute lymphoblastic leukemia.

    Science.gov (United States)

    Özkaya, Özay; Üsçetin, İlker; Egemen, Onur; Bingöl, Derya; Akan, Mithat

    2012-05-01

    Ecthyma gangrenosum is a rare invasive cutaneous infection mostly caused by Pseudomonas aeruginosa in immunosuppressed patients, especially during neutropenic states. Embolic lesions usually accompanying with septicemia of P. aeruginosa are typically painless, round, and centrally necrotic with a surrounding erythematous halo. Facial involvement occurred only in 6% of the patients, which may result to complicated defects for reconstruction. In this article, we aimed to present a case with relapse of acute lymphoblastic leukemia presented to our clinic with 50% defect of the lower lip due to ecthyma gangrenosum and its late-term reconstruction. PMID:22627426

  20. A Case of Acute Prosthesis Migration after Femoral Head Replacement due to Osteomalacia by FGF23-Induced Tumor

    Directory of Open Access Journals (Sweden)

    Shinya Hayashi

    2012-01-01

    Full Text Available Fibroblast growth factor 23 (FGF23 was recently identified as an important factor involved in the development of hypophosphatemic rickets and osteomalacia. We experienced a rare case of acute prosthesis migration after hemihip arthroplasty due to FGF23-induced tumor. The patient underwent femoral head replacement because of femoral neck fracture, but prosthesis migration was occurred at 1 week after operation. The patient took various examinations, and FGF23-induced tumor was found in his right wrist. The tumor was resected, and he underwent total hip arthroplasty 8 month later. Finally, he was able to obtain free gait without pain.

  1. A homosexual japanese man with acute hepatitis due to hepatitis B virus genotype ae, concurrent with amebic colitis

    OpenAIRE

    Sakaguchi, Kohsaku; Kobashi, Haruhiko; Takaki, Akinobu; Kato, Jun; Nawa, Toru; Tatsukawa, Masashi; Ishikawa, Shin; Iwasaki, Yoshiaki; Miyake, Yasuhiro; Shiratori, Yasushi

    2007-01-01

    We report herein a case with acute hepatitis due to hepatitis B virus genotype Ae, concurrent with amebic colitis. A 39-year-old homosexual Japanese man was admitted to our hospital with jaundice. Laboratory tests showed an elevation of transaminase and positivity for hepatitis B surface antigen and IgM-type antibody to hepatitis B core antigen. The hepatitis B virus genotype was determined to be Ae. Furthermore, a mud-like stool with blood and mucous had sometimes been noted during the past ...

  2. A homosexual japanese man with acute hepatitis due to hepatitis B virus genotype ae, concurrent with amebic colitis

    OpenAIRE

    Miyake, Yasuhiro; Iwasaki, Yoshiaki; Ishikawa, Shin; Tatsukawa, Masashi; Nawa, Toru; Kato, Jun; Takaki, Akinobu; Kobashi, Haruhiko; Sakaguchi, Kohsaku; Shiratori, Yasushi

    2007-01-01

    We report herein a case with acute hepatitis due to hepatitis B virus genotype Ae, concurrent withamebic colitis. A 39-year-old homosexual Japanese man was admitted to our hospital with jaundice.Laboratory tests showed an elevation of transaminase and positivity for hepatitis B surface antigen and IgM-type antibody to hepatitis B core antigen. The hepatitis B virus genotype was determined to be Ae. Furthermore, a mud-like stool with blood and mucous had sometimes been noted during thepast 3 y...

  3. Mortality due to acute adverse drug reactions in Galicia: 1997-2011.

    Science.gov (United States)

    Miguel-Arias, Domingo; Pereiro Gómez, César; Bermejo Barrera, Ana M; López de Abajo Rodríguez, Benito; Sobrido Prieto, María

    2016-01-01

    The aim of this research is to study all people who died in the Autonomous Community of Galicia from acute death after drugconsumption (ADR) in which there was judicial intervention during the period from 1997 to 2011, according to inclusion and exclusión criteria established by the National Drug Plan for the entire national territory. Sociodemographic and clinical characteristics of deceased subjects were studied, in order to identify key risk factors and/or vulnerable populations.A total of 805 deaths were recorded. The distribution by provinces and municipalities corresponds to the areas of greatest population, incidence of consumption and proximity to the coast. The average age of these patients was 34.34 years, with a gradual increase over years. Most of them were male (91.2%) and single (47.7). 43.5% of the deceased habitually used the parenteral route of administration and 36.4% had positive HIV serology. The most frequently-detected substances corresponded to opiates (heroin: 61.3%, methadone: 35.6%), followed by cocaine (53.7%), although the most common pattern was that of poly-consumption. ADR mortality figures remain relatively stable throughout the study period. The predominant pattern is that of males, opiates and a long history of consumption. PMID:26990265

  4. CT in the evaluation of patients on ECMO due to acute respiratory failure

    International Nuclear Information System (INIS)

    Heading AbstractBackground. In patients with acute severe respiratory failure (ARF) treated with extracorporeal membrane oxygenation (ECMO) the radiological evaluation has until now almost exclusively relied on bedside radiography and US. At St. Goeran/Karolinska ECMO centre CT has become a routine complement to bedside examinations.Objective. To review retrospectively the frequency, indications and findings on CT of patients with ARF on ECMO and to evaluate the risk of complications associated with transportation for CT examinations.Materials and methods. One hundred twelve neonates, children and adults were treated with ECMO from May 1994 to January 2001. Forty-six per cent of these patients had CT examinations on one or more occasions during ECMO, giving a total number of 238 examination sites on 104 occasions. All CT examinations were performed in the Paediatric Radiology Department and included a 10-min transport using a mobile ECMO system.Results. CT was more often performed in older patients and in patients with long ECMO runs. The main indications were suspected complications of ECMO and/or the underlying disease or a delay in clinical improvement. In 57% of the CT occasions, significant findings affecting treatment were revealed. There were no complications associated with the examinations or transport.Conclusions. CT is safe and useful in evaluation of patients with ARF during ECMO. (orig.)

  5. Acute flaccid paralysis due to West nile virus infection in adults: A paradigm shift entity

    Directory of Open Access Journals (Sweden)

    Boby Varkey Maramattom

    2014-01-01

    Full Text Available Three cases of acute flaccid paralysis (AFP with preceding fever are described. One patient had a quadriparesis with a florid meningoencephalitic picture and the other two had asymmetric flaccid paralysis with fasciculations at the onset of illness. Magnetic resonance imaging in two cases showed prominent hyperintensitities in the spinal cord and brainstem with prominent involvement of the grey horn (polio-myelitis. Cerebrospinal fluid (CSF polymerase chain reaction was positive for West Nile virus (WNV in the index patient. All three cases had a positive WNV immunoglobulin M antibody in serum/CSF and significantly high titer of WNV neutralizing antibody in serum, clearly distinguishing the infection from other Flaviviridae such as Japanese encephalitis. WNV has been recognized in India for many decades; however, AFP has not been adequately described. WNV is a flavivirus that is spread by Culex mosquitoes while they take blood meals from humans and lineage 1 is capable of causing a devastating neuro-invasive disease with fatal consequences or severe morbidity. We describe the first three laboratory confirmed cases of WNV induced AFP from Kerala and briefly enumerate the salient features of this emerging threat.

  6. Acute respiratory distress syndrome due to vivax malaria: case report and literature review

    Directory of Open Access Journals (Sweden)

    André V. Lomar

    2005-10-01

    Full Text Available Severe pulmonary involvement in malaria has been frequently reported in cases of Plasmodium falciparum infection, but rarely in vivax malaria. Among the 11 previous cases of vivax-related severe respiratory involvement described in the literature, all except one developed it after the beginning of anti-malarial treatment; these appear to correspond to an exacerbation of the inflammatory response. We report the case of a 43-year-old Brazilian woman living in a malaria-endemic area, who presented acute respiratory distress syndrome (ARDS caused by P. vivax before starting anti-malarial treatment. The diagnosis was made based on microscopic methods. A negative rapid immunochromatographic assay, based on the detection of Histidine Rich Protein-2 (HRP-2 of P. falciparum, indicated that falciparum malaria was unlikely. After specific anti-plasmodial therapy and intensive supportive care, the patient was discharged from the hospital. We conclude that vivax malaria-associated ARDS can develop before anti-malarial therapy.

  7. Invasive infection in an acute myeloblastic leukemia patient due to triazole-resistant Candida tropicalis.

    Science.gov (United States)

    de Carvalho Parahym, Ana Maria Rabelo; da Silva, Carolina Maria; Leão, Mariele Porto Carneiro; Macario, Michele Chianca; Filho, Gustavo Antônio da Trindade Meira Henriques; de Oliveira, Neiva Tinti; Neves, Rejane Pereira

    2011-11-01

    Non-albicans Candida species are being increasingly reported as causes of nosocomial fungal infections. For example, invasive candidiasis caused by C. tropicalis has been associated with hematologic malignancies. In this study, we report a fatal case of fungemia and a possible urinary and pulmonary infection in a leukemia patient that was due to a strain of C. tropicalis resistant to 2 triazole antifungals.

  8. 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

  9. Expectations about recovery from acute non-specific low back pain predict absence from usual work due to chronic low back pain : a systematic review

    NARCIS (Netherlands)

    Hallegraeff, J.M.; Krijnen, W.P.; van der Schans, C.P.; de Greef, M.H.G.

    2012-01-01

    Question: Do negative expectations in patients after the onset of acute low back pain increase the odds of absence from usual work due to progression to chronic low back pain? Design: Systematic review with meta-analysis of prospective inception cohort studies. Participants: Adults with acute or sub

  10. Studies of the ionizing radiation effects on the effluents acute toxicity due to anionic surfactants

    International Nuclear Information System (INIS)

    Several studies have shown the negative effects of surfactants, as detergents active substance, when discharged on biological sewage wastewater treatment plants. High toxicity may represent a lower efficiency for biological treatment. When surfactants are in aquatic environment they may induce a loss of grease revetment on birds (feather). Depending on the surfactant concentration, several damages to all biotic systems can happen. Looking for an alternative technology for wastewater treatment, efficient for surfactant removal, the present work applied ionizing radiation as an advanced oxidation process for affluents and effluents from Suzano Treatment Station. Such wastewater samples were submitted to radiation using an electron beam from a Dynamic Electron Beam Accelerator from Instituto de Pesquisas Energeticas e Nucleares. In order to assess this proposed treatment efficacy, it was performed acute toxicity evaluation with two test-organisms, the crustacean Daphnia similis and the luminescent bacteria Vibrio fischeri. The studied effluents were: one from a chemical industry (IND), three from sewage plant (affluents - GG, GM and Guaio) and the last biologically treated secondary effluent (EfF), discharged at Tiete river. The applied radiation doses varied from 3 kGy to 50 kGy, being 50 kGy enough for surfactant degradation contained at industrial effluent. For GG, GM and Guaio samples, doses of 6 kGy and 10 kGy were efficient for surfactant and toxicity reduction, representing an average removal that varied from 71.80% to 82.76% and toxicity from 30% to 91% for most the effluents. The final effluent was less toxic than the others and the radiation induced an average 11% removal for anionic surfactant. The industrial effluents were also submitted to an aeration process in order to quantify the contribution of surfactant to the whole sample toxicity, once it was partially removed as foam and several fractions were evaluated for toxicity. (author)

  11. Acute hemolytic transfusion reactions due to multiple alloantibodies including anti-E, anti-c and anti-Jkb.

    Science.gov (United States)

    Park, Tae Sung; Kim, Ki Uk; Jeong, Woo Jin; Kim, Hyung Hoi; Chang, Chulhun L; Chung, Joo Seop; Cho, Goon Jae; Lee, Eun Yup; Son, Han Chul

    2003-12-01

    We report a case of two consecutive episodes of acute hemolytic transfusion reactions (HTRs) due to multiple alloantibodies in a 34-yr-old man who suffered from avascular necrosis of left femoral head. He received five units of packed red blood cells (RBCs) during surgery. Then the transfusion of packed RBCs was required nine days after the surgery because of the unexplained drop in hemoglobin level. The transfusion of the first two units resulted in fever and brown-colored urine, but he received the transfusion of another packed RBCs the next day. He experienced even more severe symptoms during the transfusion of the first unit. We performed antibody screening test, and it showed positive results. Multiple alloantibodies including anti-E, anti-c and anti-Jkb were detected by antibody identification study. Acute HTRs due to multiple alloantibodies were diagnosed, and the supportive cares were done for 6 days. We suggest the antibody screening test should be included in the panel of pretransfusion tests for safer transfusion, and it is particularly mandatory for the patients with multiple transfusions, pregnant women, and preoperative patients. PMID:14676451

  12. Regional myocardial extraction of a radioiodinated branched chain fatty acid during right ventricular pressure overload due to acute pulmonary hypertension

    International Nuclear Information System (INIS)

    To determine whether branched chain fatty acid extraction is reduced during right ventricular (RV) dysfunction due to acute pulmonary artery hypertension, studies were done in 6 anesthetized dogs. Regional branched chain fatty acid extraction was measured by comparing the myocardial uptake of I-125 labeled 15-[p-(iodophenyl)]-3-methylpentadecanoic acid (I-PDA) to myocardial blood flow. Acute pulmonary hypertension was induced by incremental intravenous injection of 100 micron diameter glass beads into six pentobarbital anesthetized, mechanically ventilated dogs. Myocardial blood flow was measured by radiolabeled microspheres both under baseline conditions and during pulmonary hypertension. Mean RV pressure rose from 12 +- 2 (mean +- SEM) to 30 +-3mmHg resulting in a 225 +- 16% increase in RV stroke work. RV ejection fraction, as assessed by gated blood pool scans fell from 39 +- 2 to 18 +- 2%. Left ventricular (LV) pressures, stroke work and ejection fraction were unchanged. Myocardial blood flow increased 132 + 59% in the RV free wall and 67 +- 22% in the RV septum. LV blood flow was unchanged. Despite increased RV work and myocardial blood flow, no differences were noted in the branched chain fatty acid extraction ratios among LV or RV free walls or septum. The authors conclude that early RV dysfunction associated with pulmonary artery hypertension is not due to inadequate myocardial blood flow or branched chain fatty acid extraction

  13. ACUTE PHOSPHATE NEPHROPATHY DUE TO USE OF PHOSPHOROUS ENEMA: CASE REPORT

    OpenAIRE

    Jamshid, Hamdard; Rumeyza, Kazancıoğlu; Işın, Kılıçaslan; YASEMINÖZLÜK; Murat, Alay; Ruhper, Çekin; Reha, Erkoç; Gauhar, Rakhymzhan

    2014-01-01

    The number of colonoscopies is rising worldwide due to national screening programs. In the USA, for example, approximately 14.2 million colonoscopies are performed every year. Adequate cleansing of the bowel before colonoscopy is important in order to obtaingood results, and many preparations are used as purgatives. One such kind of purgative is an enema containing sodium phosphate.Sodium phosphate containing purgatives are widely used Bezmialem Vakif University Faculty of Medicine, Departmen...

  14. Acute Stridor and Respiratory Failure due to Retrosternal Subglottic Stenosis of Unknown Origin

    OpenAIRE

    Linda Smith; Nicola Jane Willis; Tharindu Vithanage; Gerben Keijzers; Tara Cochrane

    2013-01-01

    Respiratory failure due to subglottic stenosis is a rare but serious condition. A 22-year-old male presented to the emergency department (ED) with shortness of breath, stridor, and change in tone of voice. The patient did not complain of B-symptoms (fever, weight loss, and night sweats). In the week before this presentation, he was diagnosed with an upper respiratory tract infection with associated bronchospasm and discharged on oral antibiotics and inhaled salbutamol without effect. He devel...

  15. Acute Cardiac Failure in a Pregnant Woman due to Thyrotoxic Crisis

    Directory of Open Access Journals (Sweden)

    Nao Okuda

    2012-01-01

    Full Text Available Introduction. Cardiac failure during pregnancy is usually related to preeclampsia/eclampsia, rarely to hyperthyroidism. While hyperthyroidism can easily lead to hypertensive cardiac failure and may harm the fetus, it is sometimes difficult to distinguish hyperthyroidism from normal pregnancy. Case Presentation. We encountered a case of 41-year-old pregnant woman with hypertensive cardiac failure. Because we initially diagnosed as pre-eclampsia/eclampsia, Caesarian section was performed. However, her symptoms still persisted after delivery. After thyroid function test results taken on the day of admission were obtained on the fourth day, we could diagnose that her cardiac failure was caused by thyrotoxic crisis. Conclusions. Hypertensive cardiac failure due to hyperthyroidism during pregnancy is rare and difficult to diagnose because of similar presentation of normal pregnancy. However, physicians should be aware of the risks posed by hyperthyroidism during pregnancy.

  16. Acute Stridor and Respiratory Failure due to Retrosternal Subglottic Stenosis of Unknown Origin

    Directory of Open Access Journals (Sweden)

    Tharindu Vithanage

    2013-01-01

    Full Text Available Respiratory failure due to subglottic stenosis is a rare but serious condition. A 22-year-old male presented to the emergency department (ED with shortness of breath, stridor, and change in tone of voice. The patient did not complain of B-symptoms (fever, weight loss, and night sweats. In the week before this presentation, he was diagnosed with an upper respiratory tract infection with associated bronchospasm and discharged on oral antibiotics and inhaled salbutamol without effect. He developed hypercapnic respiratory failure in the ED after a coughing episode. A normal nasopharyngoscopic examination and a subtle mediastinal abnormality on chest radiograph lead to a working diagnosis of retrosternal subglottic obstruction. The complexities of his airway management and suggestions for multidisciplinary approach are discussed.

  17. Coronary stenting with cardiogenic shock due to acute ascending aortic dissection

    Institute of Scientific and Technical Information of China (English)

    Yuichi; Hanaki; Kazuhiko; Yumoto; Seigen; I; Hajime; Aoki; Tomoyuki; Fukuzawa; Takahiro; Watanabe; Kenichi; Kato

    2015-01-01

    A 65-year-old man developed chest pain under cardiogenic shock. Coronary angiography revealed severe stenosis from the ostium of the left main coronary artery(LMCA) to the left anterior descending artery(LAD). Intravascular ultrasound(IVUS) identified a large hematoma that originated from the aorta and extended into the LAD, thereby compressing the true lumen. Type A aortic dissection(TAAD) that involved the LMCA was diagnosed by IVUS. Coronary stenting was performed via the LMCA to the proximal LAD, which resulted in coronary blood flow restoration and no further propagation of dissection. Elective surgical aortic repair was performed 2 wk after the stenting. LMCA stenting under IVUS guidance is effective for prompt diagnosis and precise stent deployment in patients with cardiogenic shock due to TAAD with LMCA dissection.

  18. 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.

  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. 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

  1. Acute Pancreatitis due to pH-Dependent Mesalazine That Occurred in the Course of Ulcerative Colitis

    Directory of Open Access Journals (Sweden)

    Yoshinori Arai

    2011-10-01

    Full Text Available We report the case of a 26-year-old male who presented with acute pancreatitis during the course of treatment for pancolitic ulcerative colitis (UC with a time-dependent mesalazine formulation, prednisolone and azathioprine (AZA. Despite a review of his clinical history and various tests, the cause of pancreatitis could not be determined. Since drug-induced pancreatitis was considered possible, administration of the time-dependent mesalazine preparation and AZA was discontinued, and conservative treatment for acute pancreatitis was performed. The pancreatitis promptly improved with these treatments, but drug lymphocyte stimulation test (DLST for both the time-dependent mesalazine formulation and AZA was negative. A pH-dependent mesalazine formulation was given for maintenance therapy of UC. Subsequently, as the pancreatitis relapsed, drug-induced pancreatitis was strongly suspected. Administration of mesalazine was discontinued, and pancreatitis was smoothly in remission by conservative treatment. According to the positive DLST result for the pH-dependent mesalazine formulation and the clinical course, a diagnosis of pH-dependent mesalazine-induced pancreatitis due to this formulation was made. During the clinical course of UC, occurrence of drug-induced pancreatitis must always be considered.

  2. The Role of Inspiratory Muscle Training in Sickle Cell Anemia Related Pulmonary Damage due to Recurrent Acute Chest Syndrome Attacks.

    Science.gov (United States)

    Camcıoğlu, Burcu; Boşnak-Güçlü, Meral; Karadallı, Müşerrefe Nur; Akı, Şahika Zeynep; Türköz-Sucak, Gülsan

    2015-01-01

    Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA) had been suffering from recurrent acute chest syndrome (ACS). Aim. To examine the effects of inspiratory muscle training (IMT) on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity was evaluated using six-minute walk test, respiratory muscle strength using mouth pressure device, hand grip strength using hand-held dynamometer, pain using Visual Analogue Scale, fatigue using Fatigue Severity Scale, dyspnea using Modified Medical Research Council Scale, and health related quality of life using European Organization for Research and Treatment of Cancer QOL measurement. Results. A significant improvement has been demonstrated in respiratory muscle strength, functional exercise capacity, pain, fatigue, dyspnea, and quality of life. There was no admission to emergency department due to acute chest syndrome in the following 12 months after commencing regular erythrocytapheresis. Conclusion. This is the first report demonstrating the beneficial effects of inspiratory muscle training on functional exercise capacity, respiratory muscle strength, pain, fatigue, dyspnea, and quality of life in a patient with recurrent ACS. PMID:26060589

  3. Independent lung ventilation in a newborn with asymmetric acute lung injury due to respiratory syncytial virus: a case report

    Directory of Open Access Journals (Sweden)

    Di Nardo Matteo

    2008-06-01

    Full Text Available Abstract Introduction Independent lung ventilation is a form of protective ventilation strategy used in adult asymmetric acute lung injury, where the application of conventional mechanical ventilation can produce ventilator-induced lung injury and ventilation-perfusion mismatch. Only a few experiences have been published on the use of independent lung ventilation in newborn patients. Case presentation We present a case of independent lung ventilation in a 16-day-old infant of 3.5 kg body weight who had an asymmetric lung injury due to respiratory syncytial virus bronchiolitis. We used independent lung ventilation applying conventional protective pressure controlled ventilation to the less-compromised lung, with a respiratory frequency proportional to the age of the patient, and a pressure controlled high-frequency ventilation to the atelectatic lung. This was done because a single tube conventional ventilation protective strategy would have exposed the less-compromised lung to a high mean airways pressure. The target of independent lung ventilation is to provide adequate gas exchange at a safe mean airways pressure level and to expand the atelectatic lung. Independent lung ventilation was accomplished for 24 hours. Daily chest radiograph and gas exchange were used to evaluate the efficacy of independent lung ventilation. Extubation was performed after 48 hours of conventional single-tube mechanical ventilation following independent lung ventilation. Conclusion This case report demonstrates the feasibility of independent lung ventilation with two separate tubes in neonates as a treatment of an asymmetric acute lung injury.

  4. The Role of Inspiratory Muscle Training in Sickle Cell Anemia Related Pulmonary Damage due to Recurrent Acute Chest Syndrome Attacks

    Directory of Open Access Journals (Sweden)

    Burcu Camcıoğlu

    2015-01-01

    Full Text Available Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA had been suffering from recurrent acute chest syndrome (ACS. Aim. To examine the effects of inspiratory muscle training (IMT on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity was evaluated using six-minute walk test, respiratory muscle strength using mouth pressure device, hand grip strength using hand-held dynamometer, pain using Visual Analogue Scale, fatigue using Fatigue Severity Scale, dyspnea using Modified Medical Research Council Scale, and health related quality of life using European Organization for Research and Treatment of Cancer QOL measurement. Results. A significant improvement has been demonstrated in respiratory muscle strength, functional exercise capacity, pain, fatigue, dyspnea, and quality of life. There was no admission to emergency department due to acute chest syndrome in the following 12 months after commencing regular erythrocytapheresis. Conclusion. This is the first report demonstrating the beneficial effects of inspiratory muscle training on functional exercise capacity, respiratory muscle strength, pain, fatigue, dyspnea, and quality of life in a patient with recurrent ACS.

  5. [Nitric oxide inhalation as an effective therapy for acute respiratory distress syndrome due to near-drowning: a case report].

    Science.gov (United States)

    Takano, Y; Hirosako, S; Yamaguchi, T; Saita, N; Suga, M; Kukita, I; Okamoto, K; Ando, M

    1999-12-01

    A 16-year-old boy with acute respiratory distress syndrome (ARDS) due to near-drowning was admitted to our hospital. ARDS was treated with low-level nitric oxide (NO) inhalation (ranging from 4 ppm to 1 ppm) for 24 days. Oxygenation was improved and pulmonary hypertension was reduced after NO inhalation, but systemic blood pressure, heart rate, and cardiac output were not affected. PaO2 improved from 153 Torr to 354 Torr under identical ventilating conditions (F1O2 1.0), and mean pulmonary arterial pressure fell from 40 mm Hg to 27 mmHg. It has been reported that NO inhalation alleviates ventilation-flow mismatch and pulmonary hypertension. It is unclear, however, whether this therapy improves the prognosis for ARDS. In our patient, NO inhalation was effective in alleviating the oxygenation impairment and pulmonary hypertension associated with ARDS.

  6. Acute renal failure and arterial hypertension due to sub capsular hematoma: is percutaneous drainage a feasible treatment?

    DEFF Research Database (Denmark)

    Kobel, Marie Cæcilie; Nielsen, Tommy Kjærgaard; Graumann, Ole

    did not respond to antihypertensive treatment, Page kidney was suspected. A pigtail catheter was placed in the haematoma and, shortly after drainage, the diuresis resumed and plasma creatinine together with blood pressure decreased. This condition had previously been managed by open surgery......Percutaneous drainage proved to be successful in managing a renal subcapsular haematoma that was causing acute renal failure and hypertension in a 74-year-old woman. The patient presented with oliguria, nausea and malaise 2 days after a ureteronephroscopic procedure with biopsies of a suspected...... urothelial neoplasm in the right renal pelvis. The left kidney had recently been removed due to renal cell carcinoma. At admission, the patient's blood pressure and plasma creatinine levels were massively elevated. Ultrasonography revealed a moderate right-sided renal subcapsular haematoma. When the patient...

  7. Acute small bowel obstruction due to ileal endometriosis: A case report and review of the most recent literature

    Directory of Open Access Journals (Sweden)

    Unalp Recai Haluk

    2012-01-01

    Full Text Available Introduction. Endometriosis is defined as the presence of benign endometrial glands and stroma outside the normal anatomical location. Endometriosis of the small bowel, especially symptomatic small bowel involvement, is very unusual. Case report. We presented a 45-year-old woman with acute intestinal obstruction due to ileal endometriosis The patient complained of severe abdominal pain, nausea and vomitting. Immediate laparotomy was carried out. Above the ileocecal valve there was an ulcerated, edematous and fragile segmental lesion that caused intestinal obstruction. Histology of this ileal segment revealed endometriosis and an annular stricture that again showed foci of endometriosis. Conclusion. In reproductiveage women with the symptoms of intestinal obstruction, intestinal endometriosis should be kept in mind.

  8. Bacteremia due to Leuconostoc pseudomesenteroides in a Patient with Acute Lymphoblastic Leukemia: Case Report and Review of the Literature

    Science.gov (United States)

    Nakamura, Akiko; Fujieda, Atsushi; Katayama, Naoyuki

    2016-01-01

    Leuconostoc species are vancomycin-resistant Gram-positive cocci. Infections due to Leuconostoc species have been reported in various immunocompromised patients, but little is known about such infection in patients with hematologic malignancies. We report a case of Leuconostoc infection in a 44-year-old woman with acute lymphoblastic leukemia. The patient developed a high fever despite antimicrobial therapy with doripenem after induction chemotherapy. After an isolate from blood cultures was identified as L. pseudomesenteroides, we changed the antibiotics to piperacillin-tazobactam and gentamicin, after which the patient recovered from the infection. Physicians should be aware of Leuconostoc species as causative pathogen if they encounter Gram-positive cocci bacteremia resistant to standard antibiotics such as vancomycin and teicoplanin, especially in patients with hematologic malignancies.

  9. Brachial Plexopathy due to Myeloid Sarcoma in a Patient With Acute Myeloid Leukemia After Allogenic Peripheral Blood Stem Cell Transplantation.

    Science.gov (United States)

    Ha, Yumi; Sung, Duk Hyun; Park, Yoonhong; Kim, Du Hwan

    2013-04-01

    Myeloid sarcoma is a solid, extramedullary tumor comprising of immature myeloid cells. It may occur in any organ; however, the invasion of peripheral nervous system is rare. Herein, we report the case of myeloid sarcoma on the brachial plexus. A 37-year-old woman with acute myelogenous leukemia achieved complete remission after chemotherapy. One year later, she presented right shoulder pain, progressive weakness in the right upper extremity and hypesthesia. Based on magnetic resonance images (MRI) and electrophysiologic study, a provisional diagnosis of brachial plexus neuritis was done and hence steroid pulse therapy was carried out. Three months later the patient presented epigastric pain. After upper gastrointestinal endoscopy, myeloid sarcoma of gastrointestinal tract was confirmed pathologically. Moreover, 18-fluoride fluorodeoxyglucose positron emission tomography showed a fusiform shaped mass lesion at the brachial plexus overlapping with previous high signal lesion on the MRI. Therefore, we concluded the final diagnosis as brachial plexopathy due to myeloid sarcoma. PMID:23705126

  10. Bacteremia due to Leuconostoc pseudomesenteroides in a Patient with Acute Lymphoblastic Leukemia: Case Report and Review of the Literature.

    Science.gov (United States)

    Ino, Kazuko; Nakase, Kazunori; Suzuki, Kei; Nakamura, Akiko; Fujieda, Atsushi; Katayama, Naoyuki

    2016-01-01

    Leuconostoc species are vancomycin-resistant Gram-positive cocci. Infections due to Leuconostoc species have been reported in various immunocompromised patients, but little is known about such infection in patients with hematologic malignancies. We report a case of Leuconostoc infection in a 44-year-old woman with acute lymphoblastic leukemia. The patient developed a high fever despite antimicrobial therapy with doripenem after induction chemotherapy. After an isolate from blood cultures was identified as L. pseudomesenteroides, we changed the antibiotics to piperacillin-tazobactam and gentamicin, after which the patient recovered from the infection. Physicians should be aware of Leuconostoc species as causative pathogen if they encounter Gram-positive cocci bacteremia resistant to standard antibiotics such as vancomycin and teicoplanin, especially in patients with hematologic malignancies. PMID:27635268

  11. Anemia and performance status as prognostic markers in acute hypercapnic respiratory failure due to chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Haja Mydin H

    2013-03-01

    Full Text Available Helmy Haja Mydin, Stephen Murphy, Howell Clague, Kishore Sridharan, Ian K TaylorDepartment of Respiratory Medicine, Sunderland Royal Infirmary, Sunderland, United KingdomBackground: In patients with acute hypercapnic respiratory failure (AHRF during exacerbations of COPD, mortality can be high despite noninvasive ventilation (NIV. For some, AHRF is terminal and NIV is inappropriate. However there is no definitive method of identifying patients who are unlikely to survive. The aim of this study was to identify factors associated with inpatient mortality from AHRF with respiratory acidosis due to COPD.Methods: COPD patients presenting with AHRF and who were treated with NIV were studied prospectively. The forced expiratory volume in 1 second (FEV1, World Health Organization performance status (WHO-PS, clinical observations, a composite physiological score (Early Warning Score, routine hematology and biochemistry, and arterial blood gases prior to commencing NIV, were recorded.Results: In total, 65 patients were included for study, 29 males and 36 females, with a mean age of 71 ± 10.5 years. Inpatient mortality in the group was 33.8%. Mortality at 30 days and 12 months after admission were 38.5% and 58.5%, respectively. On univariate analysis, the variables associated with inpatient death were: WHO-PS ≥ 3, long-term oxygen therapy, anemia, diastolic blood pressure < 70 mmHg, Early Warning Score ≥ 3, severe acidosis (pH < 7.20, and serum albumin < 35 g/L. On multivariate analysis, only anemia and WHO-PS ≥ 3 were significant. The presence of both predicted 68% of inpatient deaths, with a specificity of 98%.Conclusion: WHO-PS ≥ 3 and anemia are prognostic factors in AHRF with respiratory acidosis due to COPD. A combination of the two provides a simple method of identifying patients unlikely to benefit from NIV.Keywords: acute exacerbations of COPD, noninvasive ventilation, emphysema, prognostic markers

  12. Hyponatremia in aneurysmal subarachnoid hemorrhage is due to the syndrome of inappropriate antidiuresis and acute glucocorticoid deficiency

    LENUS (Irish Health Repository)

    Hannon, M J

    2011-06-01

    Hyponatraemia is the most common electrolyte abnormality following subarachnoid haemorrhage (SAH) and contributes to increased morbidity and mortality. Retrospective data suggests that the syndrome of inappropriate diuresis (SIAD) is the most common cause of hyponatraemia in SAH, though cerebral salt wasting has been postulated by some workers to be the predominant abnormality. Data which has shown acute glucocorticoid deficiency following SAH has suggested that some cases of euvolaemic hyponatraemia may also be caused by this mechanism.We prospectively studied the hormonal and haemodynamic influences involved in the development of hyponatraemia in 100 patients (61% female, median age 53 (range 16-82)) with non-traumatic aneurysmal SAH. Each patient had plasma sodium (pNa), urea, osmolality, glucose and 0900h cortisol (PC), and urinary sodium and osmolality measured on days 1, 2, 3, 4, 6, 8, 10 and 12 following SAH. Fluid balance and haemodynamic parameters were recorded daily. Results were compared with 15 patients admitted to ITU following vascular surgery. A PC<300nmol\\/L in a patient in ITU was regarded clinically as inappropriately low.49% of patients developed hyponatraemia (pNa<135 mmol\\/L), including 14% who developed clinically significantly hyponatraemia (pNa<130 mmol\\/L). 36\\/49 (73.4%) developed hyponatraemia between days 1 and 3 post SAH. The median duration of hyponatraemia was 3 days (range 1–10 days).In 35\\/49 (71.4%), hyponatraemia was due to SIAD as defined by standard diagnostic criteria. 14% of SAH patients had at least one PC<300nmol\\/L; 5 of these (35.7%) developed hyponatraemia. In 4 patients hyponatraemia was preceded by acute cortisol deficiency and responded to hydrocortisone treatment. In contrast, all controls had PC>500 nmol\\/L on day 1, and >300 nmol on days 3–12. There were no cases of cerebral salt wasting. There was no relationship between the incidence of hyponatraemia and the defined anatomical territory or severity of

  13. Reduction of death rate due to acute myocardial infarction in subjects with cancers through systemic restoration of impaired nitric oxide.

    Directory of Open Access Journals (Sweden)

    Rajeshwary Ghosh

    Full Text Available INTRODUCTION: Excessive aggregation of platelets at the site of plaque rupture on the coronary artery led to the formation of thrombus which is reported to precipitate acute myocardial infarction (AMI. Nitric oxide (NO has been reported to inhibit platelet aggregation and induce thrombolysis through the in situ formation of plasmin. As the plasma NO level in AMI patients from two different ethnic groups was reduced to 0 µM (median compared to 4.0 µM (median in normal controls, the effect of restoration of the NO level to normal ranges on the rate of death due to AMI was determined. METHODS AND RESULTS: The restoration of plasma NO level was achieved by a sticking small cotton pad (10×25 mm containing 0.28 mmol sodium nitroprusside (SNP in 0.9% NaCl to the abdominal skin of the participants using non-toxic adhesive tape which was reported to normalize the plasma NO level. The participants (8,283 were volunteers in an independent study who had different kinds of cancers and did not wish to use any conventional therapy for their condition but opted to receive SNP "pad" for their condition for 3 years. The use of SNP "pad" which normalized (≈4.0 µM the plasma NO level that in consequence reduced the death rate due to AMI, among the participants, was found to be significantly reduced compared to the death due to AMI in normal population. CONCLUSION: Our data suggested that the use of SNP "pad" significantly reduced the death due to AMI. TRIAL REGISTRATION: www.ctri.nic.in CTRI/2013/12/004236.

  14. [A case of acute chronic respiratory failure due to fat embolism syndrome after the left femoral neck fracture].

    Science.gov (United States)

    Oda, Keishi; Kawanami, Toshinori; Yatera, Kazuhiro; Ogoshi, Takaaki; Kozaki, Minako; Nagata, Shuya; Nishida, Chinatsu; Yamasaki, Kei; Ishimoto, Hiroshi; Mukae, Hiroshi

    2011-09-01

    A 78 year old Japanese woman was transferred to our hospital for the treatment of a fracture of the left femoral neck in April, 2010. She had been taking oral corticosteroid (prednisolone 5 mg/day) for the treatment of idiopathic interstitial pneumonia since 2003, and had been treated by home oxygen therapy since 2007. She fell in the restroom at home and hurt herself, and was transferred to our hospital for treatment of a left femoral neck fracture in April, 2010. Her respiratory status was stable just after the transfer; however, she was transferred to the intensive care unit and started to receive mechanical ventilation due to rapidly progressive respiratory failure on the fourth day after admission. Chest X-ray and computed tomography revealed rapid progression of bilateral ground-glass attenuations, and acute exacerbation of interstitial pneumonia was clinically suspected. However, the elevation of D-dimer over time and characteristic findings of petechial hemorrhagic lesions on her palpebral conjunctivae and neck with microscopic findings of phagocytized lipid in alveolar macrophages in her endobronchial secretion led to the diagnosis of fat embolism syndrome. She was successfully treated with high-dose corticosteroid and sivelestat sodium, and she was discharged on the 21st day after admission. Although a differential diagnosis of acute exacerbation of interstitial pneumonia and fat embolism syndrome was necessary and difficult in the present case, characteristic findings of petechial hemorrhagic lesions of skin, palpebral conjunctiva and lipid-laden alveolar macrophages in endotracheal aspirate were useful for the accurate and prompt diagnosis of fat embolism syndrome. PMID:21913383

  15. 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

  16. 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.

  17. 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.

  18. 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.

  19. Risk factors and outcome in patients with primary sclerosing cholangitis with persistent biliary candidiasis

    OpenAIRE

    Rupp, Christian; Bode, Konrad Alexander; Chahoud, Fadi; Wannhoff, Andreas; Friedrich, Kilian; Weiss, Karl-Heinz; Sauer, Peter; Stremmel, Wolfgang; Gotthardt, Daniel Nils

    2014-01-01

    Background Candidiasis is commonly observed in patients with primary sclerosing cholangitis (PSC), but the clinical risk factors associated with its presence have not been fully investigated. In this study, we aimed to analyse the incidence, risk factors, and transplantation-free survival in primary sclerosing cholangitis (PSC) patients with persistent biliary candidiasis. Methods We retrospectively analysed patients diagnosed with PSC who were admitted to our department during 2002 to 2012. ...

  20. Risk factors and outcome in patients with primary sclerosing cholangitis with persistent biliary candidiasis

    OpenAIRE

    Rupp, Christian; Bode, Konrad Alexander; Chahoud, Fadi; Wannhoff, Andreas; Friedrich, Kilian; Weiss, Karl-Heinz; Sauer, Peter; Stremmel, Wolfgang; Gotthardt, Daniel N

    2014-01-01

    Background: Candidiasis is commonly observed in patients with primary sclerosing cholangitis (PSC), but the clinical risk factors associated with its presence have not been fully investigated. In this study, we aimed to analyse the incidence, risk factors, and transplantation-free survival in primary sclerosing cholangitis (PSC) patients with persistent biliary candidiasis. Methods: We retrospectively analysed patients diagnosed with PSC who were admitted to our department during 2002 to 20...

  1. 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.

  2. 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

  3. The high incidence of acute hemolysis due to favism in Ahvaz, Iran-clinical features and laboratory findings

    Institute of Scientific and Technical Information of China (English)

    Seyyed Mohammad Hasan Aletayeb; Bashir Chomeili; Mehri Taheri; Tahereh Ziaei Kajbaf; Mehran Hakimzadeh; Majid Aminzadeh; Morteza Shojaei Moghadam; Susan Maleki

    2010-01-01

    Objective:To collect comprehensive information about the features of favic patients in Ahvaz (Capital of Khouzestan, Iran) and analyze the extent of the differences with their corresponding in other regions. Methods:A total of 103 patients with acute hemolysis admitted to pediatric division of Abouzar Hospital located in the city of Ahvaz, Iran during 21st of June 2008 to 20th of June 2009 were analyzed retrospectively. Results:95.14%of the patients had favism while 4.86%of them underwent hemolysis due to other reasons. These patients were male (68.93%) and female children (31.06%) admitted mostly during the spring season. The three main symptoms were urine discoloration, jaundice and vomiting. At the admission time, the main hematologic findings were as follows:G6PD sufficient status (45.63%), G6PD deficient status (54.36%) and hemoglobin concentration:2.5-11.8 (mean±SD:6.45±2.12) g/dL. Conclusions:In conclusion, Ahvaz was determined as a black zone for favism in which the disease can be considered a life threatening health problem. Moreover, slight differences were observed in the three main symptoms compared with favic patients in other regions.

  4. Treatment of Cough and Dyspnea due to Acute Bronchitis by Plaster for Cough and Dyspnea-A Report of 735 Cases

    Institute of Scientific and Technical Information of China (English)

    陈振甫; 周文秀; 高举先; 孙江桥

    2002-01-01

    @@ In the light of the theory of treating the internal disease externally, an externally used plaster for treating cough and dyspnea due to acute bronchitis (Ke Chuan Yi Tie Kang 咳喘一贴康) was successfully applied to 735 cases of acute bronchitis (the treatment group), with the other 423 cases treated with routine western drugs as controls. The results showed that the cure rate in the treatment group was significantly higher than that in the control group (P<0.01); and that in the treatment group, the cure rate for the wind-cold type of acute bronchitis was significantly higher than that for the wind-heat type of acute bronchitis (P<0.01).

  5. Percutaneous coronary intervention for acute myocardial infarction in a pediatric patient with coronary aneurysm and stenosis due to Kawasaki disease.

    Science.gov (United States)

    Drossner, David M; Chappell, Clay; Rab, Tanveer; Kim, Dennis

    2012-06-01

    We report the case of an acutely ill 3-year-old female, with a previous medical history of Kawasaki disease, who presented to care with an acute myocardial infarction. We describe the coordinated therapies employed by pediatric and adult cardiologists aimed to establish coronary revascularization.

  6. 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.

  7. [Survival by a young woman with malnutrition due to alcoholism and eating disorders and with acute respiratory distress syndrome due to severe pneumonia who showed increased serum neutrophil elastase activity].

    Science.gov (United States)

    Nakajima, Hirokazu; Sawaguchi, Hirochiyo; Nakajima, Shigenori

    2006-11-01

    A 30-year-old woman with malnutrition due to alcoholism and eating disorders was found to have acute respiratory distress syndrome (ARDS) and sepsis due to severe Streptococcus pneumoniae pneumonia. S. pneumoniae was detected by an in vitro rapid immunochromatographic assay for S. pneumoniae antigen in urine on the day of admission and by blood culture 2 days after admission. Symptoms and laboratory findings improved after treatment with sivelestat sodium hydrate, antibiotics, and mechanical ventilation. Treatment with sivelestat sodium hydrate also decreased serum neutrophil elastase activity. This case demonstrates the usefulness of early treatment with sivelestat sodium hydrate in ARDS due to severe pneumonia.

  8. Peptic ulcers accompanied with gastrointestinal bleeding, pylorus obstruction and cholangitis secondary to choledochoduodenal fistula: A case report

    Science.gov (United States)

    XI, BIN; JIA, JUN-JUN; LIN, BING-YI; GENG, LEI; ZHENG, SHU-SEN

    2016-01-01

    Peptic ulcers are an extremely common condition, usually occurring in the stomach and proximal duodenum. However, cases of peptic ulcers accompanied with multiple complications are extremely rare and hard to treat. The present case reinforces the requirement for the early recognition and correct treatment of peptic ulcers accompanied with multiple complications. A 67-year-old man presented with recurrent abdominal pain, fever and melena. The laboratory results showed anemia (hemoglobin 62 g/l) and hypoproteinemia (23 g/l). Abdominal imaging examinations revealed stones in the gallbladder and right liver, with air in the dilated intrahepatic and extrahepatic bile ducts. Endoscopic retrograde cholangiopancreatography failed due to a deformed pylorus. The patient was finally diagnosed with peptic ulcers accompanied with gastrointestinal (GI) bleeding, pylorus obstruction and cholangitis secondary to a choledochoduodenal fistula during an emergency pancreatoduodenectomy, which was performed due to a massive hemorrhage of the GI tract. The patient recovered well after the surgery. PMID:26870237

  9. Non-invasive mechanical ventilation in acute respiratory failure due to chronic obstructive pulmonary disease: correlates for success.

    OpenAIRE

    Ambrosino, N; Foglio, K; Rubini, F.; Clini, E.; Nava, S.; M. Vitacca

    1995-01-01

    BACKGROUND--Non-invasive mechanical ventilation is increasingly used in the treatment of acute respiratory failure in patients with chronic obstructive pulmonary disease (COPD). The aim of this study was to identify simple parameters to predict the success of this technique. METHODS--Fifty nine episodes of acute respiratory failure in 47 patients with COPD treated with non-invasive mechanical ventilation were analysed, considering each one as successful (78%) or unsuccessful (22%) according t...

  10. 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.

  11. 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.

  12. Disseminated fusariosis and endogenous fungal endophthalmitis in acute lymphoblastic leukemia following platelet transfusion possibly due to transfusion-related immunomodulation

    Directory of Open Access Journals (Sweden)

    Yong Ku

    2011-11-01

    Full Text Available Abstract Background To report a case of disseminated fusariosis with endogenous endophthalmitis in a patient with acute lymphoblastic leukemia. Transfusion-associated immune modulation secondary to platelet transfusion could play an important role in the pathophysiology of this case. Case Presentation A 9 year-old male with acute lymphoblastic leukemia complicated by pancytopenia and disseminated Intravascular coagulation was given platelet transfusion. He developed disseminated fusariosis and was referred to the ophthalmology team for right endogenous endophthalmitis. The infection was controlled with aggressive systemic and intravitreal antifungals. Conclusion Patients with acute lymphoblastic leukemia are predisposed to endogenous fungal endophthalmitis. Transfusion-associated immune modulation may further increase host susceptibility to such opportunistic infections.

  13. Acute liver failure due to concomitant arterial, portal and biliary injury during laparoscopic cholecystectomy: is transplantation a valid life-saving strategy? A case report

    Directory of Open Access Journals (Sweden)

    Goldaracena Nicolas

    2009-09-01

    Full Text Available Abstract Background Combined iatrogenic vascular and biliary injury during cholecystectomy resulting in ischemic hepatic necrosis is a very rare cause of acute liver failure. We describe a patient who developed fulminant liver failure as a result of severe cholestasis and liver gangrene secondary to iatrogenic combine injury or the hepatic pedicle (i.e. hepatic artery, portal vein and bile duct during laparoscopic cholecystectomy. Case presentation A 40-years-old woman underwent laparoscopic cholecystectomy for acute cholecystitis. During laparoscopy, a severe bleeding at the liver hilum motivated the conversion to open surgery. Many sutures were placed across the parenchyma for bleeding control. After 48 hours, she rapidly deteriorated with encephalopathy, coagulopathy, persistent hypotension and progressive organ dysfunction including acute renal failure requiring hemodialysis and mechanical ventilation. An angiography documented an occlusion of right hepatic artery and right portal vein. In the clinical of acute liver failure secondary to liver gangrene, severe coagulopathy and progressive secondary multi-organ failure, the patient was included in the waiting list for liver transplantation. Two days later, the patient was successfully transplanted with initial adequate liver graft function. However, she developed bilateral pneumonia and severe gastrointestinal bleeding and finally died 24 days after transplantation due to bilateral necrotizing pneumonia. Conclusion The occurrence of acute liver failure due to portal triad injury during laparoscopic cholecystectomy is a catastrophic complication. Probably, the indication of liver transplantation as a life-saving strategy in patients with late diagnosis, acute liver failure, severe coagulopathy and progressive secondary multi-organ failure could be considered but only minimizing immunosuppressive regimen to avoid postoperative infections.

  14. 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.

  15. 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.)

  16. 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.

  17. 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.

  18. 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.

  19. 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.)

  20. Xenobiotics and loss of tolerance in primary biliary cholangitis.

    Science.gov (United States)

    Wang, Jinjun; Yang, Guoxiang; Dubrovsky, Alana Mari; Choi, Jinjung; Leung, Patrick S C

    2016-01-01

    Data from genome wide association studies and geoepidemiological studies established that a combination of genetic predisposition and environmental stimulation is required for the loss of tolerance in primary biliary cholangitis (PBC). The serologic hallmark of PBC are the presence of high titer anti-mitochondrial autoantibodies (AMA) that recognize the lipoyl domain of the mitochondrial pyruvate dehydrogenase E2 (PDC-E2) subunit. Extensive efforts have been directed to investigate the molecular basis of AMA. Recently, experimental data has pointed to the thesis that the breaking of tolerance to PDC-E2 is a pivotal event in the initial etiology of PBC, including environmental xenobiotics including those commonly found in cosmetics and food additives, suggesting that chemical modification of the PDC-E2 epitope may render its vulnerable to become a neo-antigen and trigger an immune response in genetically susceptible hosts. Here, we will discuss the natural history, genetics and immunobiology of PBC and structural constraints of PDC-E2 in AMA recognition which makes it vulnerable to chemical modification. PMID:26755880

  1. [Association between ulcerative colitis and primary esclerosing cholangitis].

    Science.gov (United States)

    Aguilar Sanchez, Victor; Guzman Rojas, Patricia; Bravo Paredes, Eduar; Rios Perez, Cristian

    2016-01-01

    Ulcerative Colitis (UC) is associated to Primary Sclerosing Cholangitis (PSC) in 80% of cases, and this association is more common than the one with Crohn’s disease. Nevertheless, the prevalence of PSC in patients with UC is only 2.9% in Latin America. We present the case of a female patient who presents a clinical history characterized for chronic diarrhea of one year of evolution, associated to fever, oral ulcers and loss of weight. In the laboratory results there is an elevation in the following: alkaline phosphatase, GGT, ALT and AST, for that reason we decide to do an abdominal ultrasound finding a hepatomegaly. In the colonoscopy we found an ulcerative colitis. Later, we do a magnetic resonance cholangiopancreatography, because of the diagnosis of UC and the abnormalities at the liver function tests, diagnosing PSC associated to UC. At that moment, the patient starts treatment with sulfasalazine that is stopped because of an adverse effect, starting prednisone and azathioprine. The patient then is discharged with the medication already mentioned and has a favorable clinical outcome. We decide to report the case because is the second reported case in Peru, being this association not commonly found in the South hemispheric. PMID:27409097

  2. Development of a duodenal gallstone ileus with gastric outlet obstruction (Bouveret syndrome four months after successful treatment of symptomatic gallstone disease with cholecystitis and cholangitis: a case report

    Directory of Open Access Journals (Sweden)

    Winnekendonk Guido

    2010-11-01

    Full Text Available Abstract Introduction Cases of gallstone ileus account for 1% to 4% of all instances of mechanical bowel obstruction. The majority of obstructing gallstones are located in the terminal ileum. Less than 10% of impacted gallstones are located in the duodenum. A gastric outlet obstruction secondary to a gallstone ileus is known as Bouveret syndrome. Gallstones usually enter the bowel through a biliary enteral fistula. Little is known about the formation of such fistulae in the course of gallstone disease. Case presentation We report the case of a 72-year-old Caucasian woman born in Germany with a gastric outlet obstruction due to a gallstone ileus (Bouveret syndrome, with a large gallstone impacted in the third part of the duodenum. Diagnostic investigations of our patient included plain abdominal films, gastroscopy and abdominal computed tomography, which showed a biliary enteric fistula between the gallbladder and the duodenal bulb. Our patient was successfully treated by laparotomy, duodenotomy, extraction of the stone, cholecystectomy, and resection of the fistula in a one-stage surgical approach. Histopathological examination showed chronic and acute cholecystitis, with perforated ulceration of the duodenal wall and acute purulent inflammation of the surrounding fatty tissue. Four months prior to developing a gallstone ileus our patient had been hospitalized for cholecystitis, a large gallstone in the gallbladder, cholangitis and a small obstructing gallstone in the common biliary duct. She had been treated with endoscopic retrograde cholangiopancreatography, endoscopic biliary sphincterotomy, balloon extraction of the common biliary duct gallstone, and intravenous antibiotics. At the time of her first presentation, abdominal ultrasound and endoscopic examination (including esophagogastroduodenoscopy and endoscopic retrograde cholangiopancreatography had not shown any evidence of a biliary enteral fistula. In the four months preceding the

  3. 盐酸丙帕他莫致急性肝损伤%Acute liver injury due to propacetamol hydrochloride

    Institute of Scientific and Technical Information of China (English)

    杨思芸; 潘昭平; 胡玉兰; 柳华; 王龙飞; 胡晓燕

    2015-01-01

    1例45岁男性患者行左侧颌面部和颈部肿物切除术,术前丙氨酸转氨酶( ALT)19 U/L,天冬氨酸转氨酶(AST)32 U/L,γ-谷氨酰转移酶(GGT)146 U/L。术后给予盐酸丙帕他莫2.0 g入0.9%氯化钠注射液100 ml静脉滴注,1次/12 h。术后第8天复查肝功能示ALT 642 U/L,AST 692 U/L, GGT 368 U/L。患者无恶心、呕吐、腹胀、纳差等不适症状,皮肤、巩膜无黄染,肝区无压痛。考虑为盐酸丙帕他莫所致急性肝损伤。当日停用盐酸丙帕他莫,给予多烯磷脂酰胆碱、还原型谷胱甘肽、N-乙酰半胱氨酸等保肝治疗。停用盐酸丙帕他莫第8天,ALT 87 U/L,AST 341 U/L,GGT 354 U/L;第18天,ALT 84 U/L,AST 36 U/L,GGT 276 U/L;第32天,ALT 24 U/L,AST 17 U/L,GGT 36 U/L。%A 45-year-old male received a lump resection on left maxillofacial and cervical region. Before treatment,the laboratory test results were as follows:alanine aminotransferase( ALT)19 U/L, aspartate aminotransferase( AST)32 U/L,gamma glutamyltransferase( GGT)146 U/L. He was given an intravenous infusion of propacetamol hydrochloride 2. 0 g in 0. 9% sodium chloride 100 ml twice daily. The laboratory tests were as follows:alanine aminotransferase 642 U/L,aspartate aminotransferase 692 U/L, gamma glutamyltransferase 368 U/L on day 8 after operation. The patient had no symptoms of nausea, vomiting,abdominal distention,and poor appetite. There was no tenderness in his hepatic zones. He was considered as acute liver injury due to propacetamol hydrochloride. Propacetamol hydrochloride was discontinued immediately and liver-protective drugs such as polyene phosphatidylcholine, reduced glutathione,and N-acetyl cysteine were given. On day 8 of drug withdrawal,the results of laboratory tests were as follows:ALT 87 U/L,AST 341 U/L,GGT 354 U/L. On day 32 of drug withdrawal,the laboratory tests results were as follows:ALT 24 U/L,AST 17 U/L,GGT 36 U/L.

  4. Production of DNA Double Strand Breaks in Human Cells due to Acute Exposure to Tritiated Water (HTO)

    International Nuclear Information System (INIS)

    The average and maximum energies of the beta emission from 3H are 5.69 keV and 18.6 keV respectively. The average range in water (or soft tissues), around 0.5 1/4m (500 nm), is considerably less than the typical diameter of a cell (10-30 1/4m), and even of a cell nucleus (5-10 1/4m), thus the micro-location of the tritium atom may well be crucial in determining its biochemical consequences. Due to the high ionization density of the beta particles emitted by tritium (about 400 ion pairs/1/4m) possible interaction of tritium beta radiation with DNA may play a significant role. Tritiated water (HTO) is the main chemical form in which tritium is found in the environment. In the body it may be retained as organically bound tritium (OBT), binding to biological molecules or remaining as OBT with various degrees of solubility. OBT can be retained in the human body much longer than HTO and therefore the dose arising from OBT can reach 50% of the total tritium dose . Histones are major protein components of chromatin. They function as spools around which DNA winds and play an important role in the regulation of gene expression. In the absence of histones, the DNA in chromosomes would be unmanageably long, as human cells each have about 1.8 m of DNA. During mitosis, DNA is duplicated and condensed, resulting in about 120 1/4m of chromosomes. It was recently reported that the phosphorylation of histone H2AX on serine residue 139 (D3-H2AX) is associated with Double Strand Breaks (DSB) sites in DNA), which indicates the possibility of research based on the detection of DSBs in DNA. The phosphorylated megabase chromatin domain surrounding the DSB can be immunostained and visualized as discrete foci by fluorescence microscopy, as each DNA DSB formed produces a visible D3-H2AX focus. Since 1 Gy of radiation produces approximately 60 DSBs/cell, doses of a few mGy should be distinguishable from the background, and it was recently shown that the exposure to 1 mGy of X-rays induces a

  5. Norovirus infections in children under 5 years of age hospitalized due to the acute viral gastroenteritis in northeastern Poland

    OpenAIRE

    Oldak, E.; Sulik, A.; Rozkiewicz, D.; Liwoch-Nienartowicz, N.

    2011-01-01

    The primary aim of this study was to evaluate the frequency and seasonality of norovirus infection in hospitalized Polish children under 5 years of age, and a secondary aim was to compare the clinical severity of norovirus and rotavirus disease. The prospective surveillance study was carried out from July 2009 through June 2010. Stool samples from 242 children hospitalized due to acute viral gastroenteritis were tested for rotavirus group A and adenovirus with commercial immunochromatographic...

  6. Posttraumatic stress due to an acute coronary syndrome increases risk of 42-month major adverse cardiac events and all-cause mortality

    OpenAIRE

    Edmondson, Donald; Rieckmann, Nina; Shaffer, Jonathan A.; Joseph E Schwartz; Burg, Matthew M.; Davidson, Karina W.; Clemow, Lynn; Shimbo, Daichi; Kronish, Ian M.

    2011-01-01

    Approximately 15% of patients with acute coronary syndromes (ACS) develop posttraumatic stress disorder (PTSD) due to their ACS event. We assessed whether ACS-induced PTSD symptoms increase risk for major adverse cardiac events (MACE) and all-cause mortality (ACM) in an observational cohort study of 247 patients (aged 25–93 years; 45% women) hospitalized for an ACS at one of 3 academic medical centers in New York and Connecticut between November 2003 and June 2005. Within 1 week of admission,...

  7. BIOPSY PROVEN ACUTE TUBULAR NECROSIS DUE TO RHABDOMYOLYSIS IN A DENGUE FEVER PATIENT: A CASE REPORT AND REVIEW OF LITERATURE

    Directory of Open Access Journals (Sweden)

    Liliany P. Repizo

    2014-01-01

    Full Text Available Renal histology results are very scarce in dengue-associated rhabdomyolysis patients developing acute kidney injury (AKI. We report a case of dengue fever-induced AKI associated to rhabdomyolysis with a renal biopsy showing acute tubular necrosis (ATN and renal deposition of myoglobin. A 28-year-old patient who presented dengue fever (DF complicated by severe AKI and rhabdomyolysis is described. The patient required hemodialysis for three weeks. A renal biopsy revealed ATN with positive staining for myoglobin in the renal tubuli. The patient was discharged with recovered renal function. In conclusion, this case report described a biopsy proven ATN associated to DF-induced rhabdomyolysis, in which renal deposition of myoglobin was demonstrated. We suggest that serum creatine phosphokinase should be monitored in DF patients to allow for an early diagnosis of rhabdomyolysis and the institution of renal protective measures.

  8. [Investigation of vectors and reservoirs in an acute Chagas outbreak due to possible oral transmission in Aguachica, Cesar, Colombia].

    Science.gov (United States)

    Soto, Hugo; Tibaduiza, Tania; Montilla, Marleny; Triana, Omar; Suárez, Diana Carolina; Torres Torres, Mariela; Arias, María Teresa; Lugo, Ligia

    2014-04-01

    Colombia recorded 11 cases of acute Chagas disease and 80 cases of oral contamination with Trypanosoma cruzi. The current study analyzes the entomological and parasitological characteristics of the outbreak in Aguachica, Cesar Department, in 2010. An interdisciplinary group of health professionals and regional university personnel conducted the laboratory tests in the patients and the investigation of the transmission focus. Eleven cases of acute Chagas diseases were detected in a single family in a dwelling with domiciliated triatomines and Rhodnius pallescens, Pantrongylus geniculatus, Eratyrus cuspidatus, and two Didelphis marsupialis opossums infected with T. cruzi in Attalea butyracea and Elaeis oleifera palm trees in the urban area of Aguachica. The study analyzes the role of R. pallescens and palm trees in the wild cycle of T. cruzi and in oral transmission of Chagas disease. Sporadic incursions by wild R. pallescens, P. geniculatus, and E. cuspidatus from the nearby palm trees into human dwellings may cause increasingly frequent outbreaks of oral Chagas disease. PMID:24896050

  9. [Investigation of vectors and reservoirs in an acute Chagas outbreak due to possible oral transmission in Aguachica, Cesar, Colombia].

    Science.gov (United States)

    Soto, Hugo; Tibaduiza, Tania; Montilla, Marleny; Triana, Omar; Suárez, Diana Carolina; Torres Torres, Mariela; Arias, María Teresa; Lugo, Ligia

    2014-04-01

    Colombia recorded 11 cases of acute Chagas disease and 80 cases of oral contamination with Trypanosoma cruzi. The current study analyzes the entomological and parasitological characteristics of the outbreak in Aguachica, Cesar Department, in 2010. An interdisciplinary group of health professionals and regional university personnel conducted the laboratory tests in the patients and the investigation of the transmission focus. Eleven cases of acute Chagas diseases were detected in a single family in a dwelling with domiciliated triatomines and Rhodnius pallescens, Pantrongylus geniculatus, Eratyrus cuspidatus, and two Didelphis marsupialis opossums infected with T. cruzi in Attalea butyracea and Elaeis oleifera palm trees in the urban area of Aguachica. The study analyzes the role of R. pallescens and palm trees in the wild cycle of T. cruzi and in oral transmission of Chagas disease. Sporadic incursions by wild R. pallescens, P. geniculatus, and E. cuspidatus from the nearby palm trees into human dwellings may cause increasingly frequent outbreaks of oral Chagas disease.

  10. Acute myocardial infarction mimicking squamous cell lung cancer with bone metastases due to hypercalcemia: a case report

    Institute of Scientific and Technical Information of China (English)

    FANG Chong-feng; XU Geng; CHEN Yang-xin

    2010-01-01

    @@ Acute myocardial infarction (AMI), the most severe coronary artery disease, is one of the most frequent cardiac emergencies, and early diagnosis and treatment are very important to decrease the subsequent cardiac adverse events such as malignant arrhythmia and sudden cardiac death. But in fact, lots of diseases are similar to AMI in clinical practice, of which the most common are myocarditis, pulmonary embolism in department of cardiology. Here we report a case of AMI-like squamous cell lung cancer with bone metastases.

  11. Independent lung ventilation in a newborn with asymmetric acute lung injury due to respiratory syncytial virus: a case report

    OpenAIRE

    Di Nardo Matteo; Perrotta Daniela; Stoppa Francesca; Cecchetti Corrado; Marano Marco; Pirozzi Nicola

    2008-01-01

    Abstract Introduction Independent lung ventilation is a form of protective ventilation strategy used in adult asymmetric acute lung injury, where the application of conventional mechanical ventilation can produce ventilator-induced lung injury and ventilation-perfusion mismatch. Only a few experiences have been published on the use of independent lung ventilation in newborn patients. Case presentation We present a case of independent lung ventilation in a 16-day-old infant of 3.5 kg body weig...

  12. Brachial Plexopathy due to Myeloid Sarcoma in a Patient With Acute Myeloid Leukemia After Allogenic Peripheral Blood Stem Cell Transplantation

    OpenAIRE

    Ha, Yumi; Sung, Duk Hyun; Park, Yoonhong; Kim, Du Hwan

    2013-01-01

    Myeloid sarcoma is a solid, extramedullary tumor comprising of immature myeloid cells. It may occur in any organ; however, the invasion of peripheral nervous system is rare. Herein, we report the case of myeloid sarcoma on the brachial plexus. A 37-year-old woman with acute myelogenous leukemia achieved complete remission after chemotherapy. One year later, she presented right shoulder pain, progressive weakness in the right upper extremity and hypesthesia. Based on magnetic resonance images ...

  13. Acute abdomen due to spontaneous splenic rupture as the first presentation of lung malignancy: a case report

    Directory of Open Access Journals (Sweden)

    Arulraj Nolan

    2011-09-01

    Full Text Available Abstract Introduction Spontaneous splenic rupture is well recognized in the context of hematological malignancies (lymphoproliferative and myeloproliferative disorders; a few case reports have also linked solid tumors, such as pancreatic and liver cancer, with the occurrence of spontaneous splenic rupture. This is the first case report of lung cancer as a likely cause of spontaneous splenic rupture. Case presentation A 61-year-old Caucasian woman presented to our hospital with non-specific symptoms. She developed an 'acute' abdomen and went into a state of shock within twelve hours of her presentation. She was diagnosed with spontaneous splenic rupture with radiology and following a laparotomy. She made an uneventful recovery postoperatively and was simultaneously found to have a bronchial adenocarcinoma. Conclusion Spontaneous splenic rupture is a potentially fatal but often unrecognized cause of acute abdomen. It should be routinely considered in the differential diagnosis of acute ('surgical' abdomen and when present it should be promptly dealt with, most commonly with a laparotomy. Once the diagnosis is confirmed there should be an aggressive drive to identify an underlying etiology; malignancy is the commonest culprit. Solid tumors should be considered as underlying causes despite being less common than hematological neoplasms. This case report demonstrates lung malignancy as an underlying precipitating cause of spontaneous splenic rupture.

  14. Aspiration thrombectomy in a case of acute myocardial infarction due to coronary emboli in a patient with peripartum cardiomyopathy and mural thrombus.

    Science.gov (United States)

    Abdulbaki, Abdulrahman; Kocherla, Cyrus; Modi, Kalgi

    2015-01-01

    Acute coronary syndrome (ACS) due to embolic phenomenon in the setting of peripartum cardiomyopathy (PPCM) and left ventricular mural thrombus is a rare occurrence. There have been two known cases described in medical literature. We present a unique case in which catheter-based aspiration thrombectomy was used to successfully treat a patient with ACS due to coronary emboli in the setting of PPCMand left ventricular mural thrombus. We believe this to be the first report of the use of aspiration thrombectomy in such a clinical case.

  15. Factors Associated with Death Due to 2009 Influenza A (H1N1) Virus Infection and Acute Respiratory Distress Syndrome in Beijing, 2009-2011

    Institute of Scientific and Technical Information of China (English)

    Jin-qian; Zhang; Li-cheng; Zhang; Na; Ren; Ming; Zhang; Li-min; Guo; Xing-wang; Li; Jun; Cheng

    2012-01-01

    Objective Patients with H1N1 virus infection were hospitalized and quarantined, and some of them developed into acute respiratory failure, and were transfered to the medical intensive care unit of Beijing Ditan Hospital, Capital Medical University in Beijing, China. Methods The clinical features and preliminary epidemiologic findings among 30 patients with confirmed H1N1 virus infection who developed into acute respiratory failure for ventilatory support were investigated. Results A total of 30 patients(37.43 ± 18.80 years old) with 2009 influenza A(H1N1) related acute respiratory distress syndrome(ARDS) received treatment with mechanical ventilation, 15 cases of whom were male and 17 cases died of ARDS. Fatal cases were significantly associated with an APACHE Ⅱ score(P = 0.016), but not with PaO 2 /FIO 2(P = 0.912) and chest radiograph(P = 0.333). The most common complication was acute renal failure(n = 9). Five patients received extracorporeal membrane oxygenation(ECMO), 3 of whom died and the others survived. The major causes of death were multiple organ dysfunction syndrome(MODS)(39%), intractable respiratory failure(27%) and sepsis(20%). Conclusions Most patients with respiratory failure due to influenza A(H1N1) virus infection were young, with a high mortality, particularly associated with APACHE Ⅱ score, secondary infection of lung or type 2 diabetes mellitus.

  16. [Bilateral renal vein thrombosis and acute renal failure due to inferior vena cava filter thrombosis. Report of one case].

    Science.gov (United States)

    Vega, Jorge; Díaz, Rienzi

    2014-11-01

    Bilateral renal vein thrombosis is an unusual etiology of acute renal failure and usually is associated with nephrotic syndrome. We report a 77-year-old man, consulting in the emergency room for anuria that appeared 24 hours after a syncope. The patient was carrier of an inferior vena cava filter prophylactically installed 17 months earlier and was not receiving anticoagulation. Serum creatinine on admission was 5.45 mg/dl and blood urea nitrogen was 54 mg/dl. Computed tomography and Doppler ultrasonography showed an extensive thrombosis of inferior vena cava and both renal veins. Heparin therapy was started with a rapid recovery of renal function and diuresis.

  17. Low cost continuous femoral nerve block for relief of acute severe cancer related pain due to pathological fracture femur

    Directory of Open Access Journals (Sweden)

    Rachel Cherian Koshy

    2010-01-01

    Full Text Available Pathological fractures in cancer patient cause severe pain that is difficult to control pharmacologically. Even with good pain relief at rest, breakthrough and incident pain can be unmanageable. Continuous regional nerve blocks have a definite role in controlling such intractable pain. We describe two such cases where severe pain was adequately relieved in the acute phase. Continuous femoral nerve block was used as an efficient, cheap and safe method of pain relief for two of our patients with pathological fracture femur. This method was proved to be quite efficient in decreasing the fracture-related pain and improving the level of well being.

  18. Posterior reversible encephalopathy syndrome (PRES, an acute neurological syndrome due to reversible multifactorial brain edema: a case report

    Directory of Open Access Journals (Sweden)

    Camilla Cicognani

    2013-04-01

    Full Text Available Background: The essential features of Posterior Reversible Encephalopathy Syndrome (PRES are headache, mental changes, seizures, visual symptoms and often arterial hypertension. Brain RMN typically shows cortico-sottocortical parieto-occipital edema, with a bilateral and symmetric distribution. PRES develops in clinical conditions as hypertensive encephalopathy, preeclampsia/ eclampsia, autoimmune diseases, after transplantation, infections and as an adverse effect of immunosuppressive drugs or chemotherapy. It usually completely reverses with treatment, although permanent sequelae are possible in case of delayed or missed diagnosis. Case report: We describe the case of a transsexual (M!F and tetraplegic patient, admitted for neck and low back pain. She suddenly developed headache, confusion, seizures and severe hypertension with normal blood tests. RMN showed multiple cortico-sottocortical areas of vasogenic and citotoxic edema in temporo-occipital, parietal, frontal, and cerebellar regions. Soon after the beginning of the antihypertensive therapy, clinical recovery was observed, as well as the disappearance of edema at RMN. Discussion and conclusions: Although PRES is usually associated with definite pathological conditions, it is not always the case, as was for the patient here described, who had no predisposing factors in her past clinical history, and presented hypertension only in the acute phase of the syndrome. Since, moreover, PRES usually presents with acute non specific features and it can be misdiagnosed with other serious diseases, the clinician will be helped by the knowledge of this syndrome to promptly start diagnostic workup and treatments, and avoid permanent neurological deficits.

  19. Acute meningoencephalomyelitis due to varicella-zoster virus in an AIDS patient: report of a case and review of the literature

    Directory of Open Access Journals (Sweden)

    Marcelo Corti

    2011-12-01

    Full Text Available Varicella-zoster virus (VZV meningoencephalomyelitis is a rare but severe neurological complication of VZV reactivation in immunocompromised patients. We report the case of an HIV-infected individual who developed an acute and severe meningoencephalomyelitis accompanied by a disseminated cutaneous eruption due to VZV. The presence of VZV DNA in cerebrospinal fluid was confirmed by polymerase chain reaction (PCR technique. The patient started undergoing an intravenous acyclovir therapy with a mild recovery of neurological manifestations. Varicella-zoster virus should be included as a cause of acute meningoencephalomyelitis in patients with AIDS. Early diagnosis followed by specific therapy should modify the rapid and fulminant course for this kind of patients.

  20. [Multiple organ failure presumably due to alkylating agents used as preconditioning drugs for autologous peripheral blood stem cell transplantation in an acute promyelocytic leukemia].

    Science.gov (United States)

    Ida, Tori; Hashimoto, Shigeo; Suzuki, Nobuaki; Ebe, Yusuke; Yano, Toshio; Sato, Naoko; Koike, Tadashi

    2016-01-01

    A 52-year-old male was diagnosed as having acute promyelocytic leukemia (APL) in 2006. He received induction chemotherapy including all-trans retinoic acid and initially achieved a complete remission (CR). After several courses of consolidation therapy combining anthracyclines and cytarabine, he maintained CR. In 2009, an APL relapse was diagnosed, and he was treated with arsenic trioxide. Since he achieved a second CR, he underwent autologous peripheral blood stem cell transplantation (auto-PBSCT) with a conditioning regimen consisting of busulfan and melphalan. At four months after auto-PBSCT, he developed a pneumothorax and acute respiratory failure. He died despite intensive therapy. Autopsy findings included various atypical and apoptotic cells in his pulmonary tissue. These changes were confirmed in multiple organs throughout the body, suggesting them to be drug-induced. The findings in this case suggested multiple organ failure due to alkylating agents.

  1. 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

  2. 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

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

    NARCIS (Netherlands)

    K. Boonstra

    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

  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. 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

  6. 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

  7. 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

  8. 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

  9. 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.

  10. Ulcerative colitis and esclerosant cholangitis in children and adolescents; case report and literature revision

    International Nuclear Information System (INIS)

    In spite of being considered rare in the pediatric practice, the intestinal inflammatory illness is recognized now with more frequency in children of all the ages. In fact, 25 to 30% of all the patients with Crohn illness, and 20% the patients with ulcerative colitis, they consult before the 20 years. A case is presented of patient of 15 years with primary esclerosant cholangitis

  11. Mutational Characterization of the Bile Acid Receptor TGR5 in Primary Sclerosing Cholangitis

    NARCIS (Netherlands)

    Hov, Johannes R.; Keitel, Verena; Laerdahl, Jon K.; Spomer, Lina; Ellinghaus, Eva; ElSharawy, Abdou; Melum, Espen; Boberg, Kirsten M.; Manke, Thomas; Balschun, Tobias; Schramm, Christoph; Bergquist, Annika; Weismueller, Tobias; Gotthardt, Daniel; Rust, Christian; Henckaerts, Liesbet; Onnie, Clive M.; Weersma, Rinse K.; Sterneck, Martina; Teufel, Andreas; Runz, Heiko; Stiehl, Adolf; Ponsioen, Cyriel Y.; Wijmenga, Cisca; Vatn, Morten H.; Stokkers, Pieter C. F.; Vermeire, Severine; Mathew, Christopher G.; Lie, Benedicte A.; Beuers, Ulrich; Manns, Michael P.; Schreiber, Stefan; Schrumpf, Erik; Haeussinger, Dieter; Franke, Andre; Karlsen, Tom H.

    2010-01-01

    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 sequ

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

    NARCIS (Netherlands)

    E. Melum; A. Franke; C. Schramm; T.J. Weismüller; D.N. Gotthardt; F.A. Offner; B.D. Juran; J.K. Laerdahl; V. Labi; E. Björnsson; R.K. Weersma; L. Henckaerts; A. Teufel; C. Rust; E. Ellinghaus; T. Balschun; K.M. Boberg; D. Ellinghaus; A. Bergquist; P. Sauer; E. Ryu; J.R. Hov; J. Wedemeyer; B. Lindkvist; M. Wittig; R.J. Porte; K. Holm; C. Gieger; H.E. Wichmann; P. Stokkers; C.Y. Ponsioen; H. Runz; A. Stiehl; C. Wijmenga; M. Sterneck; S. Vermeire; U. Beuers; A. Villunger; E. Schrumpf; K.N. Lazaridis; M.P. Manns; S. Schreiber; T.H. Karlsen

    2011-01-01

    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 2,17

  13. 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

  14. 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...... studied risk factors, including medical therapy, that could influence on IBD disease activity....

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

    Directory of Open Access Journals (Sweden)

    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.

  16. Indications and Outcomes in Liver Transplantation in Patients With Primary Sclerosing Cholangitis in Norway

    Science.gov (United States)

    Andersen, Ina M.; Fosby, Bjarte; Boberg, Kirsten M.; Clausen, Ole P. F.; Jebsen, Peter; Melum, Espen; Line, Pål D.; Foss, Aksel; Schrumpf, Erik; Karlsen, Tom H.

    2015-01-01

    Background Primary sclerosing cholangitis (PSC) is 1 of the leading causes of liver transplantation (LTX) in Scandinavia, and an increasing number of PSC patients have been transplanted in Norway during the last 2 decades. This trend is partly attributable to the recently established practice in Norway of offering LTX to PSC patients with cholangiocellular dysplasia. Based on the controversy associated with this practice, we herein aimed to report the main features and outcomes of our LTX program in PSC. Methods The primary indication for LTX (quality of life/end-stage liver disease or suspected neoplasia) was retrospectively determined for 222 patients undergoing LTX for PSC or other autoimmune liver diseases (primary biliary cirrhosis/autoimmune hepatitis) with at least 5 years of follow-up. Results In PSC patients impaired quality of life (43.5%) and end-stage liver disease (38.4%) were the most frequent indications for LTX, whereas suspected neoplasia accounted for 18.1%. The proportion of PSC patients with manifest encephalopathy, variceal bleeding, or ascites declined over time. In patients with suspected neoplasia as the primary indication for LTX (n = 25), neoplasia was confirmed in the explanted liver in 20 patients (80%). Five-year survival rates for PSC patients transplanted between 2001 and 2009 were 91.9% for patients receiving LTX due to impaired quality of life or end-stage liver disease and 83.3% for suspected neoplasia. Conclusions The PSC patients are increasingly listed for LTX at an earlier stage of their liver disease. In patients with suspected neoplasia before LTX, 5-year survival was acceptable, despite confirmation of neoplasia in 80% of the liver explants. PMID:27500239

  17. Refinement of the MHC Risk Map in a Scandinavian Primary Sclerosing Cholangitis Population

    Science.gov (United States)

    Næss, Sigrid; Lie, Benedicte A.; Melum, Espen; Olsson, Marita; Hov, Johannes R.; Croucher, Peter J. P.; Hampe, Jochen; Thorsby, Erik; Bergquist, Annika; Traherne, James A.; Schrumpf, Erik; Boberg, Kirsten Muri; Schreiber, Stefan; Franke, Andre; Karlsen, Tom H.

    2014-01-01

    Background 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. Methodology/Principal Findings 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). Conclusions/Significance 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. PMID:25521205

  18. An unusual case of acute kidney injury due to vancomycin lessons learnt from reliance on eGFR.

    Science.gov (United States)

    Barraclough, Katherine; Harris, Marianne; Montessori, Val; Levin, Adeera

    2007-08-01

    We present a case of renal impairment in an emaciated HIV-infected male that initially went unrecognized because of reliance on serum creatinine and estimated glomerular filtration rate (eGFR). Inaccurate vancomycin dosing led to toxic drug levels (66 mg/l), associated with acute and severe worsening of kidney function. This occurred in the context of escalating doses of vancomycin given in the presence of changing kidney function, albeit kidney function that always remained well within the normal range (serum creatinine 29 - 42 mumol/l). In the absence of other plausible explanations, a presumptive diagnosis of vancomycin nephrotoxicity was made. Given the rarity of this diagnosis in the current era, we discuss the pathophysiology of vancomycin nephrotoxicity. We also explore the potential reasons for inaccuracy of GFR prediction equations in the HIV population, and discuss the potential pitfalls associated with application of eGFR or even serum creatinine without appropriate understanding of their limitations. We believe our case highlights a number of important teaching points: Vancomycin nephrotoxitiy is rare but can occur in the setting of kidney dysfunction. Current assessment of kidney function using creatinine and eGFR requires awareness of the clinical caveats in which these measures may be misleading. Acute changes in kidney function, irrespective of the test used, should be contextualized to the individual situation. Persons with HIV and low muscle mass constitute a specific subgroup in whom assessment of kidney function may be problematic using creatinine. We support ongoing efforts to develop or refine equations for specific unique and easily identifiable populations. PMID:17470454

  19. 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

  20. Diphtheria, pertussis (whooping cough, and tetanus vaccine induced recurrent seizures and acute encephalopathy in a pediatric patient: Possibly due to pertussis fraction

    Directory of Open Access Journals (Sweden)

    Mahendra K Patel

    2012-01-01

    Full Text Available A 5-month-old male patient developed recurrent seizures and acute encephalopathy possibly due to first dose of diphtheria, pertussis (whooping cough, and tetanus (DPT vaccine used for routine immunization. Postreaction computed tomography (CT scan of brain, magnetic resonance imaging (MRI of brain, and electroencephalogram were normal. Pertussis fraction of DPT vaccine is responsible for this reaction. It is suggested that acellular pertussis vaccine should be used instead of whole cell vaccine because it is associated with lower frequency of neurological complications, such as seizures, encephalopathy, and hypotensive episodes. However, acellular pertussis-containing vaccines are currently not affordable in most developing countries.

  1. Determination of behavioral reactions of a child of 3-6 ages group to be hospitalized due to an acute illness

    OpenAIRE

    Zümrüt Başbakkal; Sibel Sönmez; Nesrin Şen Celasin; Figen Esenay

    2010-01-01

    The study is executed with mothers of children aged 3-6 (n=170) whose children were hospitalized for the first time between the dates of 15.07.2003 and 15.06.2006, who were reachable by phone and accepted to participate in the study aiming determination of behavioral reactions of a child of 3-6 ages group to be hospitalized due to an acute illness.In this study, for data gathering "Personal Information Form" including 15 questions and "Inquiry Form of Behavior Changes of  3-6 Ages Group Child...

  2. Repeated lung lavage with extracorporeal membrane oxygenation treating severe acute respiratory distress syndrome due to nasogastric tube malposition for enternal nutrition: a case report.

    Science.gov (United States)

    Kao, Xiaoming; Yu, Wenkui; Zhu, Weiming; Li, Ning; Li, Jieshou

    2012-01-01

    Enternal nutritional support, a frequently applied technique for providing nutrition and energy, played a pivotal role in the treatment of high risk patients. However, severe complications induced by malposition of nasogastric tube caused great danger and even death to the patients. In this case report, we present a patient with severe acute respiratory distress syndrome (ARDS) induced by bronchopleural fistula (BPF) due to malposition of nasogastric tube. Repeated lung lavage combined with extracorporeal membrane oxygenation (ECMO) was performed after transferring to the ICU of our hospital. Finally, the patient recovered and discharged 7 days after admission.

  3. Acute liver failure due to natural killer-like T-cell leukemia/lymphoma: A case report and review of the Literature

    Institute of Scientific and Technical Information of China (English)

    Evan S Dellon; Shannon R Morris; Wozhan Tang; Cherie H Dunphy; Mark W Russo

    2006-01-01

    Acute liver failure (ALF) is a medical emergency requiring immediate evaluation for liver transplantation. We describe an unusual case of a patient who presented with ascites, jaundice, and encephalopathy and was found to have ALF due to natural killer (NK)-like T cell leukemia/lymphoma. The key immunophenotype was CD2+, CD3+, CD7+, CD56+. This diagnosis, which was based on findings in the peripheral blood and ascitic fluid, was confirmed with liver biopsy, and was a contraindication to liver transplantation. A review of the literature shows that hematologic malignancies are an uncommon cause of fulminant hepatic failure, and that NK-like T-cell leukemia/lymphoma is a relatively recently recognized entity which is characteristically CD3+ and CD56+. This case demonstrates that liver biopsy is essential in diagnosing unusual causes of acute liver failure, and that infiltration of the liver with NK-like T-cell lymphoma/leukemia can cause acute liver failure.

  4. Acute portal vein thrombosis due to chronic relapsing pancreatitis: a fistula between a pancreatic pseudocyst and the splenic vein.

    Science.gov (United States)

    Kikuchi, Masahiro; Nishizaki, Yasuhiro; Tsuruya, Kota; Hamada, Ikuko; Higashi, Toru; Sakuma, Keiko; Shiozawa, Hirokazu; Aoki, Jun; Nagashima, Rena; Koizumi, Jun; Arase, Yoshitaka; Shiraishi, Koichi; Matsushima, Masashi; Mine, Tetsuya

    2014-02-01

    Portal vein thrombosis (PVT) is a relatively common complication in patients with liver cirrhosis, but several other causes might play an important role in PVT pathogenesis. We present a case of alcoholic chronic pancreatitis complicated by acute extensive PVT. The patient was managed conservatively with danaparoid sodium at first, but the thrombosis gradually extended. We then tried radiological intervention using the direct transhepatic and transjugular intrahepatic postsystemic shunt approaches. Although we were able to successfully catheterize the percutaneous transhepatic portal vein (PTP), we could not achieve recanalization of the portal vein. Therefore, PTP catheterization and systemic intravenous infusion of urokinase and heparin was performed to prevent further progression of the thrombosis and cavernous transformation was finally achieved. Computed tomography (CT) and magnetic resonance cholangiopancreatography revealed a pancreatic stone which had possibly induced dilatation of the tail duct and formation of a pancreatic pseudocyst and caused intractable pancreatitis. We performed endoscopic retrograde cholangiopancreatography and placed a stent in the pancreatic duct, which completely cured the pancreatitis. Retrospectively, the previous CT with curved multi-planar reconstruction was reviewed and a fistula was detected between the pancreatic pseudocyst and splenic vein. We concluded that the etiology of the PVT was not only inflammatory extension from pancreatitis but also a fistula between the pancreatic duct and the splenic vein. PMID:26183509

  5. Automatic Detection of CT Perfusion Datasets Unsuitable for Analysis due to Head Movement of Acute Ischemic Stroke Patients

    Directory of Open Access Journals (Sweden)

    Fahmi Fahmi

    2014-01-01

    Full Text Available Head movement during brain Computed Tomography Perfusion (CTP can deteriorate perfusion analysis quality in acute ischemic stroke patients. We developed a method for automatic detection of CTP datasets with excessive head movement, based on 3D image-registration of CTP, with non-contrast CT providing transformation parameters. For parameter values exceeding predefined thresholds, the dataset was classified as ‘severely moved’. Threshold values were determined by digital CTP phantom experiments. The automated selection was compared to manual screening by 2 experienced radiologists for 114 brain CTP datasets. Based on receiver operator characteristics, optimal thresholds were found of respectively 1.0°, 2.8° and 6.9° for pitch, roll and yaw, and 2.8 mm for z-axis translation. The proposed method had a sensitivity of 91.4% and a specificity of 82.3%. This method allows accurate automated detection of brain CTP datasets that are unsuitable for perfusion analysis.

  6. Successful Salvage of a Renal Allograft after Acute Renal Vein Thrombosis due to May-Thurner Syndrome

    Directory of Open Access Journals (Sweden)

    Omkar U. Vaidya

    2012-01-01

    Full Text Available A 68-year-old Caucasian female with a past medical history of a deceased donor kidney transplant four months prior was admitted with a two-day history of anuria and acute kidney injury. A renal ultrasound demonstrated thrombus in the transplanted kidney's renal vein that extended into the left iliac vein as well as into the left femoral venous system. Catheter-guided tissue thrombolytics were infused directly into the clot. Within twelve hours of initiating thrombolytic infusion, there was brisk urine output. Interval venography demonstrated decreasing clot burden. At the time of discharge her creatinine was 0.78 mg/dL, similar to her baseline value prior to presentation. The patient was noted to have May-Thurner syndrome on intravascular ultrasound (IVUS. Angioplasty followed by stent placement was done. Unique to our case report was the timing of the presentation of renal vein thrombosis (four months after transplant and the predisposing anatomy consistent with May-Thurner syndrome, which was diagnosed with IVUS and successfully treated with local thrombolytics.

  7. Acute portal vein thrombosis due to chronic relapsing pancreatitis: a fistula between a pancreatic pseudocyst and the splenic vein.

    Science.gov (United States)

    Kikuchi, Masahiro; Nishizaki, Yasuhiro; Tsuruya, Kota; Hamada, Ikuko; Higashi, Toru; Sakuma, Keiko; Shiozawa, Hirokazu; Aoki, Jun; Nagashima, Rena; Koizumi, Jun; Arase, Yoshitaka; Shiraishi, Koichi; Matsushima, Masashi; Mine, Tetsuya

    2014-02-01

    Portal vein thrombosis (PVT) is a relatively common complication in patients with liver cirrhosis, but several other causes might play an important role in PVT pathogenesis. We present a case of alcoholic chronic pancreatitis complicated by acute extensive PVT. The patient was managed conservatively with danaparoid sodium at first, but the thrombosis gradually extended. We then tried radiological intervention using the direct transhepatic and transjugular intrahepatic postsystemic shunt approaches. Although we were able to successfully catheterize the percutaneous transhepatic portal vein (PTP), we could not achieve recanalization of the portal vein. Therefore, PTP catheterization and systemic intravenous infusion of urokinase and heparin was performed to prevent further progression of the thrombosis and cavernous transformation was finally achieved. Computed tomography (CT) and magnetic resonance cholangiopancreatography revealed a pancreatic stone which had possibly induced dilatation of the tail duct and formation of a pancreatic pseudocyst and caused intractable pancreatitis. We performed endoscopic retrograde cholangiopancreatography and placed a stent in the pancreatic duct, which completely cured the pancreatitis. Retrospectively, the previous CT with curved multi-planar reconstruction was reviewed and a fistula was detected between the pancreatic pseudocyst and splenic vein. We concluded that the etiology of the PVT was not only inflammatory extension from pancreatitis but also a fistula between the pancreatic duct and the splenic vein.

  8. Acute psychosis due to non-paraneoplastic anti-NMDA-receptor encephalitis in a teenage girl: Case report.

    Science.gov (United States)

    Kramina, Sandra; Kevere, Laura; Bezborodovs, Nikita; Purvina, Santa; Rozentals, Guntis; Strautmanis, Jurgis; Viksna, Zane

    2015-12-01

    Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a disease occurring when antibodies produced by the body's own immune system attack NMDA-type glutamate receptors in the brain. Most anti-NMDAR encephalitis cases are associated with paraneoplastic syndrome. We analyze the case of a 15-year-old girl who was hospitalized in a child psychiatry clinic in Riga, Latvia, with de novo acute polymorphic psychotic disorder gradually progressing to a catatonic state. The patient received antipsychotic and electroconvulsive therapy with no beneficial effect. The council of doctors discussed differential diagnoses of schizophrenia-induced catatonia and the autoimmune limbic encephalitis-induced catatonic condition. When the diagnosis of anti-NMDAR autoimmune encephalitis was finally confirmed by repeated immunological assays (specific immunoglobulin [Ig] G and IgM in her blood serum and cerebrospinal fluid), and a paraneoplastic process was ruled out, she was started on immunomodulating therapy (methylprednisolone, Ig, plasmapheresis, rituximab), which changed the course of her disease. On immunomodulating treatment, her physical and mental health have gradually improved to almost complete reconvalescence. Psychiatrists should consider anti-NMDAR encephalitis as a differential diagnosis in first-episode psychosis patients presenting with disorientation, disturbed consciousness, pronounced cognitive deficits, movement disorder, dysautonomia, or rapid deterioration, and test for specific IgG NR1 autoantibodies, even if there are no specific findings on routine neuroimaging, electroencephalography (EEG), or cerebrospinal fluid tests. PMID:26663628

  9. A Giant Mesenteric Desmoid Tumor Revealed by Acute Pulmonary Embolism due to Compression of the Inferior Vena Cava

    Science.gov (United States)

    Palladino, Elisa; Nsenda, Joseph; Siboni, Renaud; Lechner, Christian

    2014-01-01

    Patient: Male, 69 Final Diagnosis: Mesenteric desmoid tumor Symptoms: — Medication: — Clinical Procedure: — Specialty: Surgery Objective: Rare disease Background: Intra-abdominal fibromatosis is a benign rare tumor of fibrous origin with a significant potential for local invasion and no ability to metastasize, but it can recur. The etiology of desmoid tumors is unknown. It is often associated with conditions such as familial adenomatous polyposis and Gardner syndrome. Case Report: We report the case of a 69-year-old man who presented to our hospital with an acute pulmonary embolism. The patient had a past history of colic surgery for a polyp with a high-grade dysplasia. Pulmonary angiography showed partial occlusion of the right superior lobe artery and partial occlusion of the middle lobe artery. The patient was given thrombolytic therapy. Abdominal computerized tomography revealed a mesenterial giant mass with compression of the inferior vena cava (IVC). A biopsy of the mass, confirming aggressive fibromatosis. A laparotomy was performed, which revealed a massive growth occupying the abdomen and attached to the previous ileocolic anastomosis. One day after surgery, his condition deteriorated. Conclusions: This report underlines the potential of imaging investigations of abdomen and vena cava if pulmonary embolism is suspected, especially when there is no evidence of peripheral venous thrombosis or other predisposing factors. Unfortunately, data on the surgical management of desmoid tumor is scarce. Therefore, the standard of treatment is a surgical resection for resectable tumors. PMID:25180474

  10. PO02 - Clinical profile of children admitted to a paediatric intensive care unit due to acute clinical deterioration

    DEFF Research Database (Denmark)

    Jensen, Claus Sixtus; Aagaard, Hanne; Olesen, Hanne Vebert;

    2016-01-01

    Theme: Intensive care Background: There has been an increased number of critically ill patients admitted to paediatric departments. Only a few studies have described the various causes of unplanned admission to paediatric intensive care units (PICU) due to clinical deterioration. However, an...... critical ill children in paediatric wards....

  11. 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.

  12. A pilot study exploring the role of glucocorticoid receptor variants in primary biliary cirrhosis and primary sclerosing cholangitis

    NARCIS (Netherlands)

    A. Hagendorf; H.R. van Buuren (Henk); J.W. Koper (Jan); S.W.J. Lamberts (Steven); P.C.J. ter Borg (Pieter)

    2004-01-01

    textabstractBACKGROUND: In primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) significant therapeutic effects of glucocorticoids have not been documented. The most important clinical problem in patients with these diseases is fatigue, which is occasionally inv

  13. Rapid diagnosis of acute hemorrhagic conjunctivitis due to coxsackievirus A24 variant by real-time one-step RT-PCR.

    Science.gov (United States)

    Lévêque, Nicolas; Lahlou Amine, Idriss; Tcheng, Remy; Falcon, Delphine; Rivat, Nathalie; Dussart, Philippe; Muyembe, Jean-Jacques; Chomel, Jean-Jacques; Norder, Helene; Eugene, Maxime; Lina, Bruno

    2007-06-01

    Coxsackievirus A24 variant is, together with enterovirus 70 and adenoviruses, the major etiological agent involved in acute hemorrhagic conjunctivitis outbreaks worldwide. However, the standard virus isolation method followed by serotyping or VP1 region sequencing is time-consuming. A rapid method for the detection of coxsackievirus A24 variant from conjunctival swab specimens would be useful in the context of explosive and extensive outbreaks. A one-step real-time RT-PCR assay based on TaqMan technology was thus developed and assessed on 36 conjunctival swabs from outbreaks of conjunctivitis in Morocco in 2004 due to a coxsackievirus A24 variant and in Corsica in 2006 due to adenovirus type 3, and 83 virus strains including 41 coxsackievirus A24 variant collected in French Guiana and Guadeloupe in 2003, in the Democratic Republic of the Congo in 2003, in Morocco in 2004 and 42 other virus species genetically close or known to be responsible for conjunctivitis. All the conjunctival swabs from coxsackievirus A24 variant related outbreak and the 41 coxsackievirus A24 variant strains were tested positive by the RT-PCR assay within 4h. This novel single-tube real-time RT-PCR assay is sensitive and specific, and consists in a reliable and faster alternative to the viral culture for recent and future acute hemorrhagic conjunctivitis outbreaks caused by coxsackievirus A24 variant.

  14. 心肌桥引起急性心肌梗死一例报道%Acute Myocardial Infarction Due to Myocardial Bridge:One Case Report

    Institute of Scientific and Technical Information of China (English)

    刘娟娟; 李慧; 彭明辉; 周敬; 傅士杰; 陈晓泓; 江孙芳

    2014-01-01

    A portion of the artery or artery tree runs under the superficial myocardial fibers for a short distance,and we define the myocardial fibers as " myocardial bridge",and we regard the coronary artery as " intramural coronary artery". The clinical performance of myocardial bridge are varied,and most of myocardial bridge are asymptomatic,and there are very few cases of acute myocardial infarction due to myocardial bridge. In this article,we reported a patient with acute myocardial infarc-tion due to myocardial bridge,in order to strengthen the understanding of the disease for physicians.%冠状动脉的某一段或其分支的某一段走行于室壁心肌纤维之间,该部分的心肌纤维束即为冠状动脉心肌桥,而这段冠状动脉则称为壁冠状动脉。心肌桥临床表现变异较大,多数无临床症状,而由其引起的急性心肌梗死更不多见。本文报道1例由孤立性心肌桥引起急性心肌梗死患者的临床特征及治疗过程,以使临床医师对该病的认识进一步加强。

  15. Effects of Melittin Treatment in Cholangitis and Biliary Fibrosis in a Model of Xenobiotic-Induced Cholestasis in Mice

    OpenAIRE

    Kyung-Hyun Kim; Hyun-Jung Sung; Woo-Ram Lee; Hyun-Jin An; Jung-Yeon Kim; Sok Cheon Pak; Sang-Mi Han; Kwan-Kyu Park

    2015-01-01

    Cholangiopathy is a chronic immune-mediated disease of the liver, which is characterized by cholangitis, ductular reaction and biliary-type hepatic fibrosis. There is no proven medical therapy that changes the course of the disease. In previous studies, melittin was known for attenuation of hepatic injury, inflammation and hepatic fibrosis. This study investigated whether melittin provides inhibition on cholangitis and biliary fibrosis in vivo. Feeding 3,5-diethoxycarbonyl-1,4-dihydrocollidin...

  16. Clinical characteristics of acute lower respiratory tract infections due to 13 respiratory viruses detected by multiplex PCR in children

    Directory of Open Access Journals (Sweden)

    Jeong-Sook Lim

    2010-03-01

    Full Text Available Purpose : This study was performed to investigate the epidemiologic and clinical features of 13 respiratory viruses in children with acute lower respiratory tract infections (ALRIs. Methods : Nasopharyngeal aspirates were prospectively obtained from 325 children aged 15 years or less from May 2008 to April 2009 and were tested for the presence of 13 respiratory viruses by multiplex real-time-polymerase chain reaction (RT-PCR. Results : Viruses were identified in 270 children (83.1%. Co-infections with ?#242; viruses were observed in 71 patients (26.3 %. Respiratory syncytial virus (RSV was the most common virus detected (33.2%, followed by human rhinovirus (hRV (19.1%, influenza virus (Flu A (16.9%, human metapneumovirus (hMPV (15.4%, parainfluenza viruses (PIVs (8.3%, human bocavirus (hBoV (8.0%, adenovirus (ADV (5.8%, and human coronavirus (hCoV (2.2%. Clinical diagnoses of viral ALRIs were bronchiolitis (37.5%, pneumonia (34.5%, asthma exacerbation (20.9%, and croup (7.1%. Clinical diagnoses of viral bronchiolitis and pneumonia were frequently demonstrated in patients who tested positive for RSV, hRV, hMPV, or Flu A. Flu A and hRV were most commonly identified in children older than 3 years and were the 2 leading causes of asthma exacerbation. hRV C was detected in 14 (4.3% children, who were significantly older than those infected with hRV A (mean±SD, 4.1±3.5 years vs. 1.7±2.3 years; P=0.009. hBoV was usually detected in young children (2.3±3.4 years with bronchiolitis and pneumonia. Conclusion : This study described the features of ALRI associated with 13 respiratory viruses in Korean children. Additional investigations are required to define the roles of newly identified viruses in children with ALRIs.

  17. Intra-arterial thrombolysis using rt-PA in patients with acute stroke due to vessel occlusion of anterior and/or posterior cerebral circulation

    International Nuclear Information System (INIS)

    The aim of our study was to evaluate the safety and efficacy of intra-arterial (IA) thrombolysis using recombinant tissue plasminogen activator (rt-PA) in patients with acute stroke due to occlusion in the anterior or posterior circulation. We retrospectively analyzed the clinical and radiological data of 88 consecutive patients with acute ischemic stroke who underwent emergency cerebral angiography for the purpose of subsequent IA thrombolysis. The neurological deficit on admission and discharge was graded using the National Institutes of Health Stroke Scale (NIHSS) score. Baseline computer tomography (CT) scans were examined for any signs indicative of cerebral ischemia. The angiographic findings were classified according to the Thrombolysis in Myocardial Infarction (TIMI) score for myocardial infarction. Follow-up CT scans were examined for hemorrhagic complication. Of the 88 patients who underwent IA thrombolysis, 63 presented with complete or partial arterial occlusion in the suspected perfusion area. In these 63 patients, the median NIHSS score dropped from 15 points on admission to 10 points at discharge. The recanalization rate was 52.6% for partial and complete reperfusion. In-hospital mortality was 20.6% (9.1% for carotid, 44.4% for basilar territory occlusion). Intracerebral bleeding (ICB) occurred in 38.6% of the patients with occlusion in the anterior circulation, resulting in these patients presenting a worse clinical outcome than those without ICB. Only minor extracranial bleedings occurred in 20.6% of patients. Patients with ICB had a significantly higher frequency of ischemic signs on the baseline CT scan. Occlusion of a cerebral artery is present in about 75% of the patients eligible for thrombolytic therapy. Intra-arterial thrombolysis using rt-PA in patients with acute ischemic stroke can achieve re-vascularization, although ICB remains the major risk factor affecting its efficacy. (orig.)

  18. Intra-arterial thrombolysis using rt-PA in patients with acute stroke due to vessel occlusion of anterior and/or posterior cerebral circulation

    Energy Technology Data Exchange (ETDEWEB)

    Tountopoulou, Argyro; Ahl, Bjoern; Weissenborn, Karin [Hannover Medical School, Department of Neurology and Clinical Neurophysiology, Hannover (Germany); Becker, Hartmut; Goetz, Friedrich [Hannover Medical School, Department of Neuroradiology, Hannover (Germany)

    2008-01-15

    The aim of our study was to evaluate the safety and efficacy of intra-arterial (IA) thrombolysis using recombinant tissue plasminogen activator (rt-PA) in patients with acute stroke due to occlusion in the anterior or posterior circulation. We retrospectively analyzed the clinical and radiological data of 88 consecutive patients with acute ischemic stroke who underwent emergency cerebral angiography for the purpose of subsequent IA thrombolysis. The neurological deficit on admission and discharge was graded using the National Institutes of Health Stroke Scale (NIHSS) score. Baseline computer tomography (CT) scans were examined for any signs indicative of cerebral ischemia. The angiographic findings were classified according to the Thrombolysis in Myocardial Infarction (TIMI) score for myocardial infarction. Follow-up CT scans were examined for hemorrhagic complication. Of the 88 patients who underwent IA thrombolysis, 63 presented with complete or partial arterial occlusion in the suspected perfusion area. In these 63 patients, the median NIHSS score dropped from 15 points on admission to 10 points at discharge. The recanalization rate was 52.6% for partial and complete reperfusion. In-hospital mortality was 20.6% (9.1% for carotid, 44.4% for basilar territory occlusion). Intracerebral bleeding (ICB) occurred in 38.6% of the patients with occlusion in the anterior circulation, resulting in these patients presenting a worse clinical outcome than those without ICB. Only minor extracranial bleedings occurred in 20.6% of patients. Patients with ICB had a significantly higher frequency of ischemic signs on the baseline CT scan. Occlusion of a cerebral artery is present in about 75% of the patients eligible for thrombolytic therapy. Intra-arterial thrombolysis using rt-PA in patients with acute ischemic stroke can achieve re-vascularization, although ICB remains the major risk factor affecting its efficacy. (orig.)

  19. 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.

  20. [A case of secondary sclerosing cholangitis caused by chemotherapy with nab-paclitaxel].

    Science.gov (United States)

    Matsuo, Taku; Nakamura, Yukiko; Suzuki, Koji

    2015-05-01

    A 73-year-old woman had received 9 months of chemotherapy with nab-paclitaxel for locally advanced breast cancer. During the treatment, she was well and showed no major side effects except for alopecia and arthralgia. The tumor showed a tendency to reduction. However, chemotherapy was discontinued because of liver dysfunction. MRCP and ERCP demonstrated multiple stenoses of the hepatic ducts and the intrahepatic bile ducts. We diagnosed chemotherapy-induced sclerosing cholangitis caused by nab-paclitaxel. Treatment with ursodeoxycholic acid and steroid was ineffective. We added bezafibrate, which resulted in a gradual improvement in liver function. To the best of our knowledge, this is the first reported case of nab-paclitaxel-induced secondary sclerosing cholangitis. PMID:25947025

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

    OpenAIRE

    Story, Brian; Gluck, Michael

    2010-01-01

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

  3. Difficulties with neurological prognostication in a young woman with delayed-onset generalised status myoclonus after cardiac arrest due to acute severe asthma

    Directory of Open Access Journals (Sweden)

    Arvind Rajamani

    2011-01-01

    Full Text Available Neurological prognostication in cardiac arrest survivors is difficult, especially when the primary etiology is respiratory arrest. Prognostic factors designed to have zero false-positive rates to robustly confirm poor outcome are usually inadequate to rule out poor outcomes (i.e., high specificity and low sensitivity. One of the least understood prognosticators is generalised status myoclonus (GSM, with case reports confusing GSM, isolated myoclonic jerks and post-hypoxic intention myoclonus (Lance Adams syndrome [LAS]. With several prognostic indicators (including status myoclonus having been validated in the pre-hypothermia era, their current relevance is debatable. New modalities such as brain magnetic resonance imaging (MRI and continuous electroencephalography are being evaluated. We describe here a pregnant woman resuscitated from a cardiac arrest due to acute severe asthma, and an inability to reach a consensus based on published guidelines, with a brief overview of myoclonus, LAS and the role of MRI brain in assisting prognostication.

  4. High-volume plasma exchange in a patient with acute liver failure due to non-exertional heat stroke in a sauna.

    Science.gov (United States)

    Chen, Kuan-Jung; Chen, Tso-Hsiao; Sue, Yuh-Mou; Chen, Tzay-Jinn; Cheng, Chung-Yi

    2014-10-01

    Heat stroke is a life-threatening condition characterized by an increased core body temperature (over 40°C) and a systemic inflammatory response, which may lead to a syndrome of multiple organ dysfunction. Heat stroke may be due to either strenuous exercise or non-exercise-induced exposure to a high environmental temperature. Current management of heat stroke is mostly supportive, with an emphasis on cooling the core body temperature and preventing the development of multiple organ dysfunction. Prognosis of heat stroke depends on the severity of organ involvement. Here, we report a rare case of non-exercise-induced heat stroke in a 73-year-old male patient who was suffering from acute liver failure after prolonged exposure in a hot sauna room. We successfully managed this patient by administering high-volume plasma exchange, and the patient recovered completely after treatment.

  5. 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.)

  6. 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.

  7. 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.

  8. Determination of behavioral reactions of a child of 3-6 ages group to be hospitalized due to an acute illness

    Directory of Open Access Journals (Sweden)

    Zümrüt Başbakkal

    2010-02-01

    Full Text Available The study is executed with mothers of children aged 3-6 (n=170 whose children were hospitalized for the first time between the dates of 15.07.2003 and 15.06.2006, who were reachable by phone and accepted to participate in the study aiming determination of behavioral reactions of a child of 3-6 ages group to be hospitalized due to an acute illness.In this study, for data gathering "Personal Information Form" including 15 questions and "Inquiry Form of Behavior Changes of  3-6 Ages Group Children After Being Hospitalized" with 30 questions were used. Date gathering forms were carried out as pre-test by using face-to-face interview method with mothers of 3-6 aged children who were hospitalized for the first time and were in first 12 hours of hospitalization. "Inquiry Form of Behavior Changes of 3-6 Ages Group Children After Being Hospitalized" was re-carried out with mothers by phone 1 month after children being discharged from hospital.In analyzing of datas statistical programme of SPSS 13.0 for Windows was used. In statistical evaluation;  number-percent dispersion, Wilcoxon Sing Rank test and Paired Sample-t test were used.According to the results obtained from the study, 57.6% of children are male with age average of 4,46±1,18 and 52.3% of them were hospitalized due to Gastroentestinal System Illnesses. A significant difference was determined between average points of behavior changes of 3-6 ages group children hospitalized due to an acute illness before hospitalized (10,735±4,882 and after being discharged from hospital (15,0476±4,306. In the study, it is observed that there are some behavioral changes in children after being hospitalized such as being cranky before going to bed and during eating, disquiet, bed-wetting, seperation anxiety, excessive attachment to a parent, to need help even for the things he/she could accomplish, to have fear from new environments, people or objects, bad temper attacks, fear of doctor/nurse and hospital

  9. Acute Respiratory Distress due to Thymoma in a Patient Treated with TK Inhibitor: A Case Report and Review of the Current Treatment Options

    Directory of Open Access Journals (Sweden)

    P. Zarogoulidis

    2011-03-01

    Full Text Available Thymic malignancies are rare intrathoracic tumors that may be aggressive and difficult to treat in advanced stage. Surgery is the cornerstone of the management of thymomas: it is significant for the definite histopathological diagnosis and staging, and in most cases, it constitutes the first step of the treatment strategy. For patients with primary unresectable thymomas, the multimodal treatment schedule nowadays includes neoadjuvant chemotherapy, extensive surgery, adjuvant radiotherapy, and in some cases, adjuvant chemotherapy. A patient with a history of stage III COPD and an undiagnosed thoracic mass was admitted to the intensive care unit with acute respiratory distress. A radiologic evaluation by CT scan revealed a mass of 13 cm in diameter at the mediastinum. Fine needle aspiration was performed and revealed a thymoma. Due to poor performance status, the patient was not able to undergo surgery. He refused to be treated with neither chemotherapy nor radiotherapy, but due to EGFR overexpression, treatment with TK inhibitor was suggested. Fine needle aspiration biopsy is commonly used to identify metastasis to the mediastinum. However, it is less often employed as a primary diagnostic tool for tumors, particularly thymic neoplasms. The use of targeted therapies for the treatment of thymic malignancies has been described in the literature. Over the past years, significant efforts have been made to dissect the molecular pathways involved in the carcinogenesis of these tumors. Insights have been obtained following anecdotal clinical responses to targeted therapies, and large-scale genomic analyses have been conducted.

  10. Successful low-dose azathioprine for myasthenia gravis despite hepatopathy from primary sclerosing cholangitis: a case report

    Directory of Open Access Journals (Sweden)

    Höflich Sonja

    2010-11-01

    Full Text Available Abstract Introduction Although myasthenia gravis is frequently associated with other disorders, it has not been reported together with primary sclerosing cholangitis, complicating the administration of liver-toxic immunosuppressive therapy. Case presentation A 73-year-old Caucasian woman with a history of arterial hypertension, thyroid dysfunction, glaucoma, right-sided ptosis and later generalized weakness, was diagnosed with myasthenia gravis. Additionally, primary sclerosing cholangitis was detected, initially prohibiting the administration of immunosuppressants. Despite treatment with steroids and pyridostigmine she repeatedly experienced myasthenic crises. After the fifth crisis and after antibody titers had reached levels > 100 nmol/L during two years of follow-up, it was decided to restart azathioprine. Interestingly, low-dose azathioprine (1.5 mg/kg/day was well tolerated, had a positive clinical and immunological effect and did not worsen primary sclerosing cholangitis. Conclusion Myasthenia gravis may occur together with primary sclerosing cholangitis in the same patient. Mild immunosuppression with azathioprine is feasible and effective in such a patient, without worsening myasthenia gravis or primary sclerosing cholangitis.

  11. Determination of behavioral reactions of a child of 3-6 ages group to be hospitalized due to an acute illnessDetermination of behavioral reactions of a child of 3-6 ages group to be hospitalized due to an acute illness

    OpenAIRE

    Nesrin Şen Celasin; Sibel Sönmez; Zümrüt Başbakkal; Figen Esenay

    2010-01-01

    The study is executed with mothers of children aged 3-6 (n=170) whose children were hospitalized for the first time between the dates of 15.07.2003 and 15.06.2006, who were reachable by phone and accepted to participate in the study aiming determination of behavioral reactions of a child of 3-6 ages group to be hospitalized due to an acute illness.In this study, for data gathering "Personal Information Form" including 15 questions and "Inquiry Form of Behavior Changes of 3-6 Ages Group Child...

  12. Avoidable Portion of Tobacco-Attributable Acute Care Hospital Days and Its Cost Due to Implementation of Different Intervention Strategies in Canada

    Directory of Open Access Journals (Sweden)

    Jürgen Rehm

    2009-08-01

    Full Text Available The impact of four effective population-based interventions, focusing on individual behavioural change and aimed at reducing tobacco-attributable morbidity, was assessed by modeling with respect to effects on reducing prevalence rates of cigarette smoking, population-attributable fractions, reductions of disease-specific morbidity and its cost for Canada. Results revealed that an implementation of a combination of four tobacco policy interventions would result in a savings of 33,307 acute care hospital days, which translates to a cost savings of about $37 million per year in Canada. Assuming 40% coverage rate for all individually based interventions, the two most effective interventions, in terms of avoidable burden due to morbidity, would be nicotine replacement therapy and physicians’ advice, followed by individual behavioural counselling and increasing taxes by 10%. Although a sizable reduction in the number of hospital days and accumulated costs could be achieved, overall these interventions would reduce less than 3% of all tobacco-attributable costs in Canada.

  13. Miocarditis fulminante y enfermedad diarreica aguda por Coxsackie virus B6 Fulminant myocarditis and acute gastroenteritis due to Coxsackie virus B6

    Directory of Open Access Journals (Sweden)

    Germán Málaga

    2011-03-01

    Full Text Available Presentamos el caso de una paciente joven que presentó choque cardiogénico por virus Coxsakie B6. La paciente acudió a una clínica particular con un cuadro clínico compatible con gastroenterocolitis aguda a la que después de una hora de estar recibiendo hidratación y manejo del cuadro diagnosticado, se agregó hipotensión que llegó al estado de choque, hipoxemia severa y compromiso pulmonar bilateral intersticial por lo que ingresó a Unidad de Cuidados Intensivos, donde recibió manejo de soporte. Debido al cuadro clínico y elevación de enzimas cardiacas se sospechó de compromiso cardiaco, la ecocardiografía evidenció cambios sugerentes de miocarditis. La evolución fue favorable y se le pudo dar de alta después de una semana. El diagnóstico etiológico del cuadro se hizo en el seguimiento, presentando serología con elevación de títulos para virus Coxsakie B6.We present the case of a young woman who suffered cardiogenic due to by Coxsackie virus B6. The patient attended a private clinic with an acute gastroenteritis and after one hour of receiving hydratation,she developed hypotension and shock, severe hypoxemia and bilateral lung infiltrate. The patient entered the Intensive Care Unit, where she received hemodynamic support. Due to the clinical picture and cardiac enzymes increase, a cardiac failure was suspected and the echocardiographic findings suggested "myocarditis". The evolution was successful and Coxsackie B6 virus infection diagnosis was made during the follow up by increase of the levels of antibodies for virus Coxsackie B6.

  14. 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.

  15. 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.)

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

    International Nuclear Information System (INIS)

    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.)

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

    Science.gov (United States)

    Melum, Espen; Franke, Andre; Schramm, Christoph; Weismüller, Tobias J; Gotthardt, Daniel Nils; Offner, Felix A; Juran, Brian D; Laerdahl, Jon K; Labi, Verena; Björnsson, 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, Björn; 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

    2015-01-01

    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 2,174 controls. We detected non-HLA associations at rs3197999 in MST1 and rs6720394 near BCL2L11 (combined P = 1.1 × 10−16 and P = 4.1 × 10−8, respectively). PMID:21151127

  18. 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.

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

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    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.

  20. Effects of Melittin Treatment in Cholangitis and Biliary Fibrosis in a Model of Xenobiotic-Induced Cholestasis in Mice

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    Kyung-Hyun Kim

    2015-08-01

    Full Text Available Cholangiopathy is a chronic immune-mediated disease of the liver, which is characterized by cholangitis, ductular reaction and biliary-type hepatic fibrosis. There is no proven medical therapy that changes the course of the disease. In previous studies, melittin was known for attenuation of hepatic injury, inflammation and hepatic fibrosis. This study investigated whether melittin provides inhibition on cholangitis and biliary fibrosis in vivo. Feeding 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC to mice is a well-established animal model to study cholangitis and biliary fibrosis. To investigate the effects of melittin on cholangiopathy, mice were fed with a 0.1% DDC-containing diet with or without melittin treatment for four weeks. Liver morphology, serum markers of liver injury, cholestasis markers for inflammation of liver, the degree of ductular reaction and the degree of liver fibrosis were compared between with or without melittin treatment DDC-fed mice. DDC feeding led to increased serum markers of hepatic injury, ductular reaction, induction of pro-inflammatory cytokines and biliary fibrosis. Interestingly, melittin treatment attenuated hepatic function markers, ductular reaction, the reactive phenotype of cholangiocytes and cholangitis and biliary fibrosis. Our data suggest that melittin treatment can be protective against chronic cholestatic disease in DDC-fed mice. Further studies on the anti-inflammatory capacity of melittin are warranted for targeted therapy in cholangiopathy.

  1. Effects of Melittin Treatment in Cholangitis and Biliary Fibrosis in a Model of Xenobiotic-Induced Cholestasis in Mice.

    Science.gov (United States)

    Kim, Kyung-Hyun; Sung, Hyun-Jung; Lee, Woo-Ram; An, Hyun-Jin; Kim, Jung-Yeon; Pak, Sok Cheon; Han, Sang-Mi; Park, Kwan-Kyu

    2015-09-01

    Cholangiopathy is a chronic immune-mediated disease of the liver, which is characterized by cholangitis, ductular reaction and biliary-type hepatic fibrosis. There is no proven medical therapy that changes the course of the disease. In previous studies, melittin was known for attenuation of hepatic injury, inflammation and hepatic fibrosis. This study investigated whether melittin provides inhibition on cholangitis and biliary fibrosis in vivo. Feeding 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) to mice is a well-established animal model to study cholangitis and biliary fibrosis. To investigate the effects of melittin on cholangiopathy, mice were fed with a 0.1% DDC-containing diet with or without melittin treatment for four weeks. Liver morphology, serum markers of liver injury, cholestasis markers for inflammation of liver, the degree of ductular reaction and the degree of liver fibrosis were compared between with or without melittin treatment DDC-fed mice. DDC feeding led to increased serum markers of hepatic injury, ductular reaction, induction of pro-inflammatory cytokines and biliary fibrosis. Interestingly, melittin treatment attenuated hepatic function markers, ductular reaction, the reactive phenotype of cholangiocytes and cholangitis and biliary fibrosis. Our data suggest that melittin treatment can be protective against chronic cholestatic disease in DDC-fed mice. Further studies on the anti-inflammatory capacity of melittin are warranted for targeted therapy in cholangiopathy. PMID:26308055

  2. 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

  3. 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.

  4. 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.

    2013-01-01

    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(1-3). We compared 3,789 PSC cases of European ancestry to 25,079 population controls across 130,422 SNPs genotyped

  5. The effect of silver nitrate, chloroformic garlic extract and normal saline in induction of sclerosing cholangitis in rabbits

    International Nuclear Information System (INIS)

    Objective was to the effects of 0.5% silver nitrate, 20% chloroformic garlic extract and 0.9% normal saline in induction of sclerosing cholangitis in the bile ducts of rabbits. During a 6-months period from April to September 2006 in Shiraz University Laboratory Animal Research Center, we selected 3 equal groups of rabbits. We injected 0.5% silver nitrate, 20% chloroformic garlic extract and 0.9% normal saline into the bile ducts of each group. The animals were euthanized and autopsied after 4 months and the liver and bile ducts were removed and studied histopathologically. Cholangiography was undertaken to evaluate the presence and extent of any sclerosing cholangitis. Animals showed sclerosing cholangitis in silver nitrate group (7 [58%]), one (8%) in chloroformic garlic extract group and one (7%) in normal saline group. The difference between silver nitrate and chloroformic garlic extract groups were statistically significant and similar results were noticed between chloroformic garlic extract and normal saline groups. Twenty percent of chloroformic garlic extract had fewer complications such as sclerosing cholangitis, compared to other materials. (author)

  6. Diagnostic Dilemma in a Patient with Jaundice: How to Differentiate between Autoimmune Pancreatitis, Primary Sclerosing Cholangitis and Pancreas Carcinoma

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    Matthias Buechter

    2012-04-01

    Full Text Available A 68-year-old male patient was referred to our institution in May 2011 for a suspected tumor in the pancreatic head with consecutive jaundice. Using magnetic resonance imaging, further differentiation between chronic inflammation and a malignant process was not possible with certainty. Apart from cholestasis, laboratory studies showed increased values for CA 19-9 to 532 U/ml (normal <37 U/ml and hypergammaglobulinemia (immunoglobulin G, IgG of 19.3% (normal 8.0–15.8% with an elevation of the IgG4 subtype to 2,350 mg/l (normal 52–1,250 mg/l. Endoscopic retrograde cholangiopancreatography revealed a prominent stenosis of the distal ductus hepaticus communis caused by pancreatic head swelling and also a bihilar stenosis of the main hepatic bile ducts. Cytology demonstrated inflammatory cells without evidence of malignancy. Under suspicion of autoimmune pancreatitis with IgG4-associated cholangitis, immunosuppressive therapy with steroids and azathioprine was started. Follow-up endoscopic retrograde cholangiopancreatography after 3 months displayed regressive development of the diverse stenoses. Jaundice had disappeared and blood values had returned to normal ranges. Moreover, no tumor of the pancreatic head was present in the magnetic resonance control images. Due to clinical and radiological similarities but a consecutive completely different prognosis and therapy, it is of fundamental importance to differentiate between pancreatic cancer and autoimmune pancreatitis. Especially, determination of serum IgG4 levels and associated bile duct lesions induced by inflammation should clarify the diagnosis of autoimmune pancreatitis and legitimate immunosuppressive therapy.

  7. Extended Analysis of a Genome-Wide Association Study in Primary Sclerosing Cholangitis Detects Multiple Novel Risk Loci

    Science.gov (United States)

    Folseraas, Trine; Melum, Espen; Rausch, Philipp; Juran, Brian D.; Ellinghaus, Eva; Shiryaev, Alexey; Laerdahl, Jon K.; Ellinghaus, David; Schramm, Christoph; Weismüller, Tobias J.; Gotthardt, Daniel Nils; Hov, Johannes Roksund; Clausen, Ole Petter; Weersma, Rinse K.; Janse, Marcel; Boberg, Kirsten Muri; Björnsson, 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.

    2012-01-01

    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). Methods We analyzed 45 SNPs in 1221 PSC cases and 3508 controls. The association results from the replication analysis and the original GWAS (715 PSC cases and 2962 controls) were combined in a meta-analysis comprising 1936 PSC cases and 6470 controls. We performed an analysis of bile microbial community composition in 39 PSC patients by 16S rRNA sequencing. Results Seventeen SNPs representing 12 distinct genetic loci achieved nominal significance (Preplication<0.05) in the replication. The most robust novel association was detected at chromosome 1p36 (rs3748816; Pcombined=2.1×10−8) where the MMEL1 and TNFRSF14 genes represent potential disease genes. Eight additional novel loci showed suggestive evidence of association (Prepl<0.05). FUT2 at chromosome 19q13 (rs602662; Pcomb=1.9×10−6, rs281377; Pcomb = 2.1×10−6 and rs601338; Pcomb=2.7×10−6) is notable due to its implication in altered susceptibility to infectious agents. We found that FUT2 secretor status and genotype defined by rs601338 significantly influences biliary microbial community composition in PSC patients. Conclusions We identify multiple new PSC risk loci by extended analysis of a PSC GWAS. FUT2 genotype needs to be taken into account when assessing the influence from microbiota on biliary pathology in PSC. PMID:22521342

  8. Extraordinary elevated CA19-9 in patient with common bile duct stones and cholangitis Case report

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    Ali Mokhtarifar

    2012-05-01

    Full Text Available Serum Carbohydrate Antigen, CA19-9, is a carbohydrate antigen whose usage -as a tumor marker- in pancreatic and bile ducts malignancies is commonly increasing. Sensitivity and specificity of this antigen in the diagnosis of malignancies is 70-90% and 68-91%, respectively. CA19-9 can also rise in other disorders and in few of benign conditions including cholangitis, due to other causes, e.g. choledocholithiasis. However, in such cases the increase is not significant and it is usually below 1000 u/ml. Values more than 10000 u/ml are limited to few case reports in articles. The case under study was a 66 year old man hospitalized because of epigastric and right upper quadrant pain, fever, jaundice, acholic stool, weight loss of about 6 kg, and hyperchromic urine. Physical examination revealed that the case was icteric and febrile with a mild RUQ tenderness on percussion. Laboratory tests showed marked elevation of bilirubin and CA19-9 (Total bilirubin: 36 mg/dl, Direct: 19.5 mg/dl CA19-9 :44777u/ml, Normal: 0-40 u/ml, but the enzymes of the liver and alkaline phosphatase only increased to two times the normal level. Sonography showed dilated common bile duct with four stones within it. Four weeks after stones extraction and renewal of bile current CA19-9 fell to 60 u/ml and returned to normal range (0-40 2 months later and during this period the patient was totally asymptomatic. As a result, in patients with biliary obstruction, a diagnosis of malignancy cannot be made solely on the basis of increased concentration of CA19-9, even in case of high clinical suspicion and the interpretation of this increase must be done with more caution and after lab follow-up following removal of the obstruction.

  9. Sclerosing cholangitis by cytomegalovirus in highly active antiretroviral therapy era

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    Carmen Hidalgo-Tenorio

    2013-10-01

    Full Text Available Sclerosing colangitis (SC due to cytomegalovirus (CMV is very rare. It has been described mainly in immunocompromised patients. Currently, in HIV infected patients it is exceptional. The most of cases belong to pre-highly active antiretroviral therapy (pre-HAART and those cases were in stage AIDS with less than 100 CD4/μl. The most frequently involved pathogen in pre-HAART period was Cryptosporidium parvum (30-57% and CMV (10-30%; in late HAART period this information are unaware. CMV has been implicated as a possible etiological agent in primary SC partly because of the ability to cause liver damage and its relationship with smooth muscle antibodies. The most effective treatment for SC was the combination of antiretroviral therapy and endoscopic retrograde cholangiopancreatography with sphincterotomy and stent placement. Following, we present the first case of late HAART period which describes a SC extrahepatic without papillary stenosis with CMV as the only cause and clinical presentation of HIV infection in a woman with 177 CD4/μl.

  10. ANSWER: A patient with Charcot’s triad

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    Faezah AMIN

    2012-12-01

    Full Text Available (Refer to page 349Answer: Acute cholangitisCholangitis is the term used for the inflammation of bile duct. There are two main forms of cholangitis: Primary Sclerosing Cholangitis(PSC and acute cholangitis (also known as ascending cholangitis. Acute cholangitis is bacterial infection within the bile duct, usually as a complication of biliary obstruction.

  11. Pancreatite aguda devida a hematoma intramural do duodeno por uso de anticoagulante Acute pancreatitis due to intramural hematoma of the duodenum by use of anticoagulant therapy

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    Samer FARHOUD

    2001-01-01

    Full Text Available Racional - A hemorragia intramural espontânea do duodeno causada por complicações da terapêutica anticoagulante é rara e seu tratamento controverso. Objetivo -- Apresentar a experiência advinda do tratamento de doente com essa condição clínica. Relato do caso - Expõe-se o caso de uma mulher de 71 anos de idade, que há 3 meses fazia uso de anticoagulante oral para tratamento de trombose venosa profunda dos membros inferiores. Apresentou-se com cefaléia e dores abdominais intensas no andar superior do abdome, associadas a náuseas e vômitos. Os exames laboratoriais e de imagem comprovaram o diagnóstico de surto agudo de pancreatite, decorrente de hematoma intramural de duodeno. Os valores de protrombina (49,7 s e o sangramento de tecidos moles cervicais e urinário, sugeriam complicação da terapêutica anticoagulante. Resultados - A terapêutica conservadora foi efetiva, tendo a doente recebido alta, assintomática, no 10º dia de internação. Conclusão - É recomendado o emprego do anticoagulante em doses menores nos doentes de risco e adequado controle dos parâmetros da coagulação. Acredita-se ser ideal a conduta conservadora e recomenda-se a cirurgia somente nos casos que evoluem com complicações.Background - Spontaneous intramural hemorrhage of the duodenum due to anticoagulant therapy is rare and the treatment is controversial. Objective - To present the acquired knowledge with the treatment of these disease. Case report - A 71-year-old women receiving for a 3 month period an anticoagulant therapy presented cervical bleeding of soft tissues and symptoms of acute pancreatitis and high small bowel obstruction. Early noninvasive diagnosis by computed tomographic scan was possible and conservative therapy proved successful in complete resolution of the pancreatitis and obstructive symptoms, with resumption of oral intake in the fourth day of treatment. The frequency of bleeding in high risk patients during warfarin therapy

  12. Determination of behavioral reactions of a child of 3-6 ages group to be hospitalized due to an acute illnessDetermination of behavioral reactions of a child of 3-6 ages group to be hospitalized due to an acute illness

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    Nesrin Şen Celasin

    2010-02-01

    Full Text Available The study is executed with mothers of children aged 3-6 (n=170 whose children were hospitalized for the first time between the dates of 15.07.2003 and 15.06.2006, who were reachable by phone and accepted to participate in the study aiming determination of behavioral reactions of a child of 3-6 ages group to be hospitalized due to an acute illness.In this study, for data gathering "Personal Information Form" including 15 questions and "Inquiry Form of Behavior Changes of 3-6 Ages Group Children After Being Hospitalized" with 30 questions were used. Date gathering forms were carried out as pre-test by using face-to-face interview method with mothers of 3-6 aged children who were hospitalized for the first time and were in first 12 hours of hospitalization. "Inquiry Form of Behavior Changes of 3-6 Ages Group Children After Being Hospitalized" was re-carried out with mothers by phone 1 month after children being discharged from hospital.In analyzing of datas statistical programme of SPSS 13.0 for Windows was used. In statistical evaluation; number-percent dispersion, Wilcoxon Sing Rank test and Paired Sample-t test were used.According to the results obtained from the study, 57.6% of children are male with age average of 4,46±1,18 and 52.3% of them were hospitalized due to Gastroentestinal System Illnesses. A significant difference was determined between average points of behavior changes of 3-6 ages group children hospitalized due to an acute illness before hospitalized (10,735±4,882 and after being discharged from hospital (15,0476±4,306. In the study, it is observed that there are some behavioral changes in children after being hospitalized such as being cranky before going to bed and during eating, disquiet, bed-wetting, seperation anxiety, excessive attachment to a parent, to need help even for the things he/she could accomplish, to have fear from new environments, people or objects, bad temper attacks, fear of doctor/nurse and hospital, fear

  13. Abdome agudo perfurativo por corpo estranho em paciente com situs inversus totalis Perfurative acute abdomen due to foreign body in patient with situs inversus totalis

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    André Vitorio Câmara Oliveira

    2008-12-01

    dyspnea, dehydration (++/4++, blood pressure= 70X40mmHg, anicteric, tense abdomen, diffusely painful, distended and hypertympanic, hydroaerial sounds absent. The thorax X-ray evidenced cardiac dextroversion, pneumoperitoneum bilaterally with gastric bubble on the right. Due to the general serious condition and confirmed diagnosis of acute abdomen perforation, a hydroelectrolytic vein replacement was held and the patient was submitted to an exploratory celiotomy, observing fecaloid diffuse peritonitis and perforation of sigmoid colon for foreign body taken away followed by mechanical cleaning, washing peritoneal cavity, Hartmann retossigmoidectomy, and bilateral drainage. CONCLUSION: Although a rare entity, the diagnosis of situs inversus totalis must be suspected in a physical examination and confirmed through image examinations, since the anatomic variations can disrupt the performance of clinical surgery.

  14. Three Ulcerative Colitis Susceptibility Loci Are Associated with Primary Sclerosing Cholangitis and Indicate a Role for IL2, REL, and CARD9

    NARCIS (Netherlands)

    M. Janse; L.E. Lamberts; L. Franke; S. Raychaudhuri; E. Ellinghaus; K.M. Boberg; E. Melum; T. Folseraas; E. Schrumpf; A. Bergquist; E. Bjornsson; J. Fu; H.J. Westra; H.J.M. Groen; R.S.N. Fehrmann; J. Smolonska; L.H. Berg; R.A. Ophoff; R.J. Porte; T.J. Weismueller; J. Wedemeyer; C. Schramm; M. Sterneck; R. Guenther; F. Braun; S. Vermeire; L. Henckaerts; C. Wijmenga; C.Y. Ponsioen; S. Schreiber; T.H. Karlsen; A. Franke; R.K. Weersma

    2011-01-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts. Both environmental and genetic factors contribute to its pathogenesis. To further clarify its genetic background, we investigated susceptibility loci recently ide

  15. 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

  16. 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

    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...... 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...... by using the competing risk regression analysis. RESULTS: Among the 439 PSC patients included, 353 (80%) had IBD at the time of Ltx and 15 (3%) patients developed de novo IBD post-Ltx. The median duration of IBD was 15 (0-50) years at the time of Ltx and follow-up after Ltx was 5 (0-20) years. Ninety...

  17. 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.

  18. 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.

  19. 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.

  20. 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.

  1. 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

  2. Acute lower respiratory tract infection due to respiratory syncytial virus in a group of Egyptian children under 5 years of age

    OpenAIRE

    El-kholy Amany A; El-anany Mervat G; Mansi Yasmeen A; Fattouh Aya M; El-karaksy Hanaa M

    2011-01-01

    Abstract Background and aim Respiratory syncytial virus (RSV) is one of the most important causes of acute lower respiratory tract infections (ALRTI) in infants and young children. This study was conducted to describe the epidemiology of ALRTI associated with RSV among children ≤ 5 years old in Egypt. Patients and Methods We enrolled 427 children ≤ 5 years old diagnosed with ALRTI attending the outpatient clinic or Emergency Department (ED) of Children Hospital, Cairo University during a one-...

  3. Endoscopic Management of Afferent Loop Syndrome after a Pylorus Preserving Pancreatoduodenecotomy Presenting with Obstructive Jaundice and Ascending Cholangitis

    OpenAIRE

    Kim, Jae Kyung; Park, Chan Hyuk; Huh, Ji Hye; Park, Jeong Youp; Park, Seung Woo; Song, Si Young; Chung, Jaebock; Bang, Seungmin

    2011-01-01

    Afferent loop syndrome is a rare complication of gastrojejunostomy. Patients usually present with abdominal distention and bilious avomiting. Afferent loop syndrome in patients who have undergone a pylorus preserving pancreaticoduodenectomy can present with ascending cholangitis. This condition is related to a large volume of reflux through the biliary-enteric anastomosis and static materials with bacterial overgrowth in the afferent loop. Patients with afferent loop syndrome after pylorus pr...

  4. A Unique Use of a Double-Pigtail Plastic Stent: Correction of Kinking of the Common Bile Duct Due to a Metal Stent

    OpenAIRE

    Kuwatani, Masaki; Kawakami, Hiroshi; Abe, Yoko; Kawahata, Shuhei; Kawakubo, Kazumichi; Kubo, Kimitoshi; Sakamoto, Naoya

    2015-01-01

    A 72-year-old man with jaundice by ampullary adenocarcinoma was treated at our hospital. For biliary decompression, a transpapillary, fully covered, self-expandable metal stent (FCSEMS) was deployed. Four days later, the patient developed acute cholangitis. Endoscopic carbon dioxide cholangiography revealed kinking of the common bile duct above the proximal end of the FCSEMS. A 7-F double-pigtail plastic stent was therefore placed through the FCSEMS to correct the kink, straightening the comm...

  5. Acute Kidney Disease Due to Excessive Vitamin C Ingestion and Remote Roux-en-Y Gastric Bypass Surgery Superimposed on CKD.

    Science.gov (United States)

    Sunkara, Vasu; Pelkowski, Timothy D; Dreyfus, Darren; Satoskar, Anjali

    2015-10-01

    A 69-year-old woman presented with acute kidney failure of unknown cause that ultimately required dialysis. Kidney biopsy revealed the diagnosis of oxalate nephropathy. In retrospect, the patient had several risk factors for this entity, including excessive vitamin C intake, a remote history of Roux-en-Y gastric bypass for weight loss, and chronic kidney disease. This presentation of multiple risk factors for oxalate nephropathy is especially relevant to patients and physicians considering the increase in the United States of vitamin C supplementation use and gastric bypass surgery. It is important for physicians to maintain an awareness of this diagnosis and its risk factors.

  6. A successfully thrombolysed acute inferior myocardial infarction due to type A aortic dissection with lethal consequences: the importance of early cardiac echocardiography

    Directory of Open Access Journals (Sweden)

    Bousoula Eleni

    2011-08-01

    Full Text Available Abstract Thrombolysis, a standard therapy for ST elevation myocardial infarction (STEMI in non-PCI-capable hospitals, may be catastrophic for patients with aortic dissection leading to further expansion, rupture and uncontrolled bleeding. Stanford type A aortic dissection, rarely may mimic myocardial infarction. We report a case of a patient with an inferior STEMI thrombolysed with tenecteplase and followed by clinical and electrocardiographic evidence of successful reperfusion, which was found later to be a lethal acute aortic dissection. Prognostic implications of early diagnosis applying transthoracic echocardiography (TTE are described.

  7. A bypass case due to an acute inferior myocardial infarction caused by vascular occlusion of the left subclavian artery and left anterior descending artery

    Directory of Open Access Journals (Sweden)

    Altas Y

    2016-07-01

    Full Text Available Yakup Altas, Ali Veysel Ulugg Department of Cardiology, Diyarbakir Gazi Yasargil Education and Research Hospital, Diyarbakir, Turkey Abstract: ST segment elevation is the most common electrocardiographic finding in acute myocardial infarction. ST elevation in chest leads generally represents left anterior descending artery occlusion, while elevation in DII and III, and aVF represents right coronary and circumflex artery occlusion. A female patient aged 66 years was admitted to our emergency service with ST elevation in leads DIII and aVF. A diagnosis of acute inferior myocardial infarction was made. The patient’s history included coronary artery bypass graft involving the left internal mammary artery to the left anterior descending coronary artery and aorta to the right coronary artery. The patient was taken to the cardiac catheterization laboratory for primary percutaneous coronary intervention and a lesion in the left anterior descending artery was identified. Additionally, the left subclavian artery was totally occluded. Following intervention to the lesion, the patient was discharged on day 4 of admission. Keywords: electrocardiography, myocardial infarction, coronary circulation

  8. 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.

  9. 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

  10. 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

  11. Clonal Characteristics of Circulating B Lymphocyte Repertoire in Primary Biliary Cholangitis.

    Science.gov (United States)

    Tan, Yan-Guo; Wang, Yu-Qi; Zhang, Ming; Han, Ying-Xin; Huang, Chun-Yang; Zhang, Hai-Ping; Li, Zhuo-Min; Wu, Xiao-Lei; Wang, Xiao-Feng; Dong, Yan; Zhu, Hong-Mei; Zhu, Shi-da; Li, Hong-Mei; Li, Ning; Yan, Hui-Ping; Gao, Zu-Hua

    2016-09-01

    Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by elevated serum anti-mitochondrial Ab and lymphocyte-mediated bile duct damage. This study was designed to reveal the clonal characteristics of B lymphocyte repertoire in patients with PBC to facilitate better understanding of its pathogenesis and better management of these patients. Using high-throughput sequencing of Ig genes, we analyzed the repertoire of circulating B lymphocytes in 43 patients with PBC, and 34 age- and gender-matched healthy controls. Compared with healthy controls, PBC patients showed 1) a gain of 14 new clones and a loss of 8 clones; 2) a significant clonal expansion and increased relative IgM abundance, which corresponded with the elevated serum IgM level; 3) a significant reduction of clonal diversity and somatic hypermutations in class-switched sequences, which suggested a general immunocompromised status; 4) the reduction of clonal diversity and enhancement of clonal expansion were more obvious at the cirrhotic stage; and 5) treatment with ursodeoxycholic acid could increase the clonal diversity and reduce clonal expansion of the IgM repertoire, with no obvious effect on the somatic hypermutation level. Our data suggest that PBC is a complex autoimmune disease process with evidence of B lymphocyte clonal gains and losses, Ag-dependent ogligoclonal expansion, and a generally compromised immune reserve. This new insight into the pathogenesis of PBC opens up the prospect of studying disease-relevant B cells to better diagnose and treat this devastating disease. PMID:27430717

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

    Science.gov (United States)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

  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. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  19. 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。

  20. 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

  1. 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.

  2. 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.

  3. 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.

  4. 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.

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

    Science.gov (United States)

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

    Science.gov (United States)

    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. PMID:27393766

  7. Acute renal failure in obstructive diseases of the extrahepatic biliary ducts.

    Science.gov (United States)

    Acalovschi, I; Chirileanu, T

    1984-01-01

    A series of 46 patients with obstructive disease of the bile ducts complicated by acute renal failure (ARF) is presented. The patients exhibited obstructive jaundice with prevalence of conjugated bilirubine. In 80% of the cases biliary obstruction was associated with cholangitis. Disturbances of the liver function (from mild cholestasis to biliary cirrhosis) were also present. The renal damage was due to biliary disorders and was either present on admission (33 cases) or developed postoperatively (13 cases). Most of the patients presented nonoliguric ARF as well as poor perfusion resulting from decreased circulating blood volume (dehydration and electrolyte loss). Among the criteria used to determine the type of ARF, the urinary/plasma creatinine ratio less than 10 and urinary/plasma osmolarity ratio less than 1.1 were the most valuable. Management of ARF by dialysis alone was not satisfactory. Attention is called to the surgical treatment of the biliary disorder as being essential to prognosis. Patients not treated by radical surgery died in proportion of 87 to 100%. From the rest of 18 patients in whom the operation provided an adequate biliary drainage, in 15 the renal function was restored and 12 survived. Better prognosis in these patients was dependent not only on the ability to cure the cholestasis and infection, but on the early surgical treatment. The ultimate prognosis depends on the improvement of the liver function.

  8. Acute lower respiratory tract infection due to respiratory syncytial virus in a group of Egyptian children under 5 years of age

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    El-kholy Amany A

    2011-04-01

    Full Text Available Abstract Background and aim Respiratory syncytial virus (RSV is one of the most important causes of acute lower respiratory tract infections (ALRTI in infants and young children. This study was conducted to describe the epidemiology of ALRTI associated with RSV among children ≤ 5 years old in Egypt. Patients and Methods We enrolled 427 children ≤ 5 years old diagnosed with ALRTI attending the outpatient clinic or Emergency Department (ED of Children Hospital, Cairo University during a one- year period. Nasopharyngeal aspirates were obtained from the patients, kept on ice and processed within 2 hours of collection. Immunoflourescent assay (IFA for RSV was performed. Results 91 cases (21.3% had viral etiology with RSV antigens detected in 70 cases (16.4%. The RSV positive cases were significantly younger than other non-RSV cases (mean age 8.2 months versus 14.2 months, p Conclusion RSV is the most common viral etiology of ALRTI in children below 5 years of age, especially in young infants below 6 months of age. It is more prevalent in winter and tends to cause severe infection.

  9. Effectiveness of a drinking-motive-tailored emergency-room intervention among adolescents admitted to hospital due to acute alcohol intoxication: A randomized controlled trial

    NARCIS (Netherlands)

    Wurdak, M.; Wolstein, J.; Kuntsche, E.N.

    2016-01-01

    The aim of this study is to develop and test the effectiveness of a drinking-motive-tailored intervention for adolescents hospitalized due to alcohol intoxication in eight cities in Germany between December 2011 and May 2012 against a similar, non-motive-tailored intervention. In a randomized contro

  10. Topographic Locomotive Analysis of 99mTc-HDP Uptake of Acute Rhabdomyolysis and Musculotendinous Unit Injury due to Excessive Swimming Exercise in Novice: A Case Report

    International Nuclear Information System (INIS)

    Rhabdomyolysis (RM), also referred to as myonecrosis, is not an uncommon disorder of skeletal muscle, the incidence of which is on the increase as endurance tests, sports and body build have become popular. RM is divided into diffuse muscle fiber damage and musculotendinous unit (MTU) injury. A recent study by Crenshaw et al. revealed that muscular fiber damage caused by racing was associated with elevated intra-muscular pressure, capillary damage, and ischchemia. It is to be remembered that myocytes are mainly destroyed in RM whereas perimysial connective is predominantly damaged in myositis ossificans. When muscle fibers disrupt myoglobin escapes into extracellular fluid and plasma resulting in myoglobinemia and often acute renal failure. Plasma creatine phosphokinase level becomes elevated. We report a case of strenuous swimming-related RM that occurred in the muscles of the shoulder girdles and chest wall analyzed using magnified 9:9mTc-HDP bone scan. Of interest magnified bone scan of RM in the present case showed not only ordinary muscular injury but also MTU injury. MRI is useful in the study of soft tissue injury and in recent years sonography has also become increasingly used. As mentioned 99mTc-HDP bone scan, especially magnification scan, sensitively depicts metabolic change that occurred in injured muscles and tendons. Unless injury is trivial the bone scan nearly always reveals pathological uptake in a damaged muscle, MTU, and/or tendinous insertion permitting the topographic distinction of injury. Thus, the diagnosis of myolysis, MTU injury, and enthesitis can specifically be made when one uses magnification technique

  11. Effectiveness and safety of noninvasive positive-pressure ventilation for severe hypercapnic encephalopathy due to acute exacerbation of chronic obstructive pulmonary disease:a prospective case-control study

    Institute of Scientific and Technical Information of China (English)

    ZHU Guang-fa; ZHANG Wei; ZONG Hua; XU Qiu-fen; LIANG Ying

    2007-01-01

    Background Although severe encephalopathy has been proposed as a possible contraindication to the use of noninvasive positive-pressure ventilation(NPPV),increasing clinical reports showed it was effective in patients with impaired consciousness and even coma secondary to acute respiratory failure,especially hypercapnic acute respiratory failure(HARF).To further evaluate the effectiveness and safety of NPPV for severe hypercapnic encephalopathy,a prospective case-control study was conducted at a university respiratory intensive care unit(RICU)in patients with acute exacerbation of chronic obstructive pulmonary disease(AECOPD)during the past 3 years.Methods Forty-three of 68 consecutive AECOPD patients requiring ventilatory support for HARF were divided into 2 groups,which were carefully matched for age,sex,COPD course,tobacco use and previous hospitalization history,according to the severity of encephalopathy,22 patients with Glasgow coma scale(GCS)0.05),but group A needed an average of 7 cmH2O higher of maximal pressure support during NPPV,and 4,4 and 7 days longer of NPPV time,RICU stay and hospital stay respectively than group B(P<0.05 or P<0.01).NPPV therapy failed in 12 patients(6 in each group)because of excessive airway secretions(7 patients),hemodynamic instability(2),worsening of dyspnea and deterioration of gas exchange(2),and gastric content aspiration(1).Conclusions Selected patients with severe hypercapnic encephalopathy secondary to HARF can be treated as effectively and safely with NPPV as awake patients with HARF due to AECOPD;a trial of NPPV should be instituted to reduce the need of endotracheal intubation in patients with severe hypercapnic encephalopathy who are otherwise good candidates for NPPV due to AECOPD.

  12. 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

  13. Effectiveness of a drinking-motive-tailored emergency-room intervention among adolescents admitted to hospital due to acute alcohol intoxication — A randomized controlled trial

    OpenAIRE

    Wurdak, M.; Wolstein, J; Kuntsche, E.N.

    2015-01-01

    The aim of this study is to develop and test the effectiveness of a drinking-motive-tailored intervention for adolescents hospitalized due to alcohol intoxication in eight cities in Germany between December 2011 and May 2012 against a similar, non-motive-tailored intervention. In a randomized controlled trial, 254 adolescents received a psychosocial intervention plus motive-tailored (intervention group; IG) or general exercises (control group; CG). Adolescents in the IG received exercises in ...

  14. Rapid parenteral rehydration in children with dehydration due to acute diarrheal disease Hidratación parenteral rápida en pacientes deshidratados por enfermedad diarreica aguda

    Directory of Open Access Journals (Sweden)

    Myriam Bastidas

    1989-03-01

    Full Text Available

    Between May and July 1987, we studied 36 children with second or third degree dehydration secondary to acute diarrheal disease of less than one week duration; they had no serious associated problems. Parenteral rehydration was carried out with a solution similar in composition to the one recommended by the World Health Organization for Oral Rehydration Therapy (ORT. Rehydration was achieved in 30 patients within 6 hours and In 3 more within 12 hours; there were no cases of hypernatremia or hyperkalemia. It is concluded that parenteral rehydration with a solution similar to the one employed for ORT is an adequate alternative when oral rehydration is not indicated in children with diarrheal disease.

    Entre mayo y julio de 1987 se estudiaron 36 niños que ingresaron al Hospital Infantil de Medellín con deshidratación de segundo o tercer grado, secundaria a enfermedad diarreica de evolución menor de una semana y sin enfermedad grave asociada. La hidratación se llevó a cabo parenteralmente empleando una mezcla de composición similar a la que recomienda la Organización Mundial de la Salud para la Terapia de Rehidratación Oral (TRO. Se logró la hidratación en un lapso de 6 horas en 30 de los 36 pacientes y en 3 más en las siguientes 6 horas; no se produjeron casos de hipernatremia ni de hiperkalemia. Se concluye que la hidratación parenteral, con una solución de composición similar a la de la TRO, es una alternativa adecuada cuando no está indicada la hidratación oral del niño con enfermedad diarreica.

  15. Acidosis láctica por metformina desencadenada por una insuficiencia renal aguda Metformin-induced lactic acidosis due to acute renal failure

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    M.D. Macías-Robles

    2011-04-01

    Full Text Available La acidosis láctica es una complicación grave pero infrecuente asociada al empleo de metformina. Se discuten los mecanismos fisiopatológicos implicados en la acidosis láctica, con especial atención al papel potencial del fármaco. Presentamos un caso severo de este efecto secundario de la metformina en una paciente con diabetes tipo 2 que ingresó en el Servicio de Urgencias Hospitalario por un cuadro de insuficiencia renal aguda. El diagnóstico quedó apoyado por unos niveles séricos elevados de la biguanida, procedimiento escasamente utilizado en la práctica clínica. El tratamiento consiste en suspender la administración del fármaco e iniciar de forma inmediata la hemodiálisis con bicarbonato, lo cual proporciona un tratamiento sintomático y etiológico al eliminar del suero tanto el lactato como el antidiabético oral. Los síntomas de la acidosis láctica por metformina son inespecíficos y el comienzo es sutil, lo que hace necesario un alto nivel de sospecha para establecer un diagnostico precoz.Lactic acidosis is a serious but uncommon side effect of metformin use. We discuss the pathophysiological mechanisms of lactic acidosis with particular regard to the role played by the drug as a potential cause of the entity. We report on a severe case of this kind of drug toxicity in a patient with type 2 diabetes mellitus, admitted to the emergency department with acute renal failure symptoms. The diagnosis was supported by elevated serum levels of the biguanide, a procedure scarcely used in clinical practice. The management of this complication consists in drug discontinuation and hemodialysis with bicarbonate that provides symptomatic and ethiological treatment by removing both the lactate and the hypoglycemic agent from the serum. Since the symptoms of metformin-associated lactic acidosis are unspecific and its onset is subtle, a high level of suspicion is needed to establish an early diagnosis.

  16. Clinically recurrent primary sclerosing cholangitis following liver transplantation: a time course.

    Science.gov (United States)

    Campsen, Jeffrey; Zimmerman, Michael A; Trotter, James F; Wachs, Michael; Bak, Thomas; Steinberg, Tracy; Kam, Igal

    2008-02-01

    Orthotopic liver transplantation (OLT) is the treatment of choice for patients with end-stage primary sclerosing cholangitis (PSC). This study sought to chronicle the natural history of PSC recurrence following OLT and identify clinical variables that may contribute to disease reemergence. From 1988 to 2006, 1102 OLTs were performed in 1032 adults at the University of Colorado Health Sciences Center. Of these, 130 patients (12.6%) with PSC received 146 allografts. Recurrence was defined by a clinically worsening examination and radiographic evidence. A total of 9 potential predictors were considered, using both bivariate log rank and multivariate Cox analysis, including: age > 55, gender, surgical technique (piggyback technique), presence of inflammatory bowel disease, intact colon before transplant, or cholangiocarcinoma (CCA), cold ischemia time, sirolimus-based immunosuppression, and graft type. The 1, 5, and 10-year recurrence-free survival was 91%, 76%, and 61%, respectively. The crude incidence of disease recurrence was 22 of 130 patients or 16.9%. Patients' risk of recurrent PSC at 1, 5, and 10 years was 2%, 12%, and 20%, respectively (mortality censored). Of the 22 patients that developed recurrent disease, 7 received a second transplant. Of the 9 factors considered, the presence of CCA prior to OLT is significantly predictive of disease recurrence [risk ratio (RR) = 3.77; P = 0.0038]. Once a patient was diagnosed with recurrent disease, the median survival without receiving a second transplant was 39.1 months (95% confidence interval: 27.6-50.6 months). In conclusion, recurrent PSC following OLT is a formidable but protracted problem following OLT. Patients may require a second transplant following reemergent disease with reasonable survival benefit. PMID:18236392

  17. 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.

  18. 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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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

  1. 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.

  2. 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.

  3. 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

    2016-01-01

    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

  4. 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.

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

    Science.gov (United States)

    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.

  6. 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.

  7. 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.

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

    Science.gov (United States)

    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

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

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    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.

  10. 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.

  11. Mutational Characterization of the Bile Acid Receptor TGR5 in Primary Sclerosing Cholangitis

    Science.gov (United States)

    Hov, Johannes R.; Keitel, Verena; Laerdahl, Jon K.; Spomer, Lina; Ellinghaus, Eva; ElSharawy, Abdou; Melum, Espen; Boberg, Kirsten M.; Manke, Thomas; Balschun, Tobias; Schramm, Christoph; Bergquist, Annika; Weismüller, Tobias; Gotthardt, Daniel; Rust, Christian; Henckaerts, Liesbet; Onnie, Clive M.; Weersma, Rinse K.; Sterneck, Martina; Teufel, Andreas; Runz, Heiko; Stiehl, Adolf; Ponsioen, Cyriel Y.; Wijmenga, Cisca; Vatn, Morten H.; Stokkers, Pieter C. F.; Vermeire, Severine; Mathew, Christopher G.; Lie, Benedicte A.; Beuers, Ulrich; Manns, Michael P.; Schreiber, Stefan; Schrumpf, Erik; Häussinger, Dieter; Franke, Andre; Karlsen, Tom H.

    2010-01-01

    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 = 8.5×10−7), 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. PMID:20811628

  12. Small duct primary sclerosing cholangitis without inflammatory bowel disease is genetically different from large duct disease

    Science.gov (United States)

    Næss, Sigrid; Björnsson, Einar; Anmarkrud, Jarl A.; Al Mamari, Said; Juran, Brian D.; Lazaridis, Konstantinos N.; Chapman, Roger; Bergquist, Annika; Melum, Espen; Marsh, Steven G. E.; Schrumpf, Erik; Lie, Benedicte A.; Boberg, Kirsten Muri; Karlsen, Tom H.; Hov, Johannes R.

    2014-01-01

    Background & aims Small duct primary sclerosing cholangitis (PSC) is phenotypically a mild version of large duct PSC, but it is unknown whether these phenotypes share aetiology. We aimed to characterize their relationship by investigating genetic associations in the HLA complex, which represent the strongest genetic risk factors in large duct PSC. Methods Four classical HLA loci (HLA-A, HLA-B, HLA-C, HLA-DRB1) were genotyped in 87 small duct PSC patients, 485 large duct PSC patients and 1117 controls across three geographical regions. Results HLA-DRB1*13:01 (OR=2.0, 95% CI 1.2–3.4, P=0.01) and HLA-B*08 (OR=1.6, 95% CI 1.1–2.4, P=0.02) were significantly associated with small duct PSC compared with healthy controls. Based on the observed frequency of HLA-B*08 in small duct PSC, the strongest risk factor in large duct PSC, an estimated 32% (95% CI 4%–65%) of this population can be hypothesized to represent early stages or mild variants of large duct PSC. This subgroup may be constituted by small duct PSC patients with inflammatory bowel disease (IBD), which greatly resembled large duct PSC in its HLA association. In contrast, small duct PSC without IBD was only associated with HLA-DRB1*13:01(P=0.03) and was otherwise distinctly dissimilar from large duct PSC. Conclusions Small duct PSC with IBD resembles large duct PSC in its HLA association and may represent early stages or mild variants of large duct disease. Different HLA associations in small duct PSC without IBD could indicate that this subgroup is a different entity. HLA-DRB1*13:01 may represent a specific risk factor for inflammatory bile duct disease. PMID:24517468

  13. Ultrasound and Point Shear Wave Elastography in Livers of Patients with Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Mjelle, Anders Batman; Mulabecirovic, Anesa; Hausken, Trygve; Havre, Roald Flesland; Gilja, Odd Helge; Vesterhus, Mette

    2016-09-01

    Point shear wave elastography (pSWE) is an ultrasound-based method for non-invasive quantification of liver fibrosis. The objective of this study was to explore liver pSWE in patients with primary sclerosing cholangitis (PSC) for assessment of fibrosis. Fifty-five non-transplant patients with PSC (38 males, 17 females; mean age: 46.4 y) were included and compared with 24 matched controls. Median (range) PSC duration was 8.1 (0-33) y. Ultrasonographic scanning followed by liver stiffness measurement by pSWE was performed using a conventional ultrasound system (Philips iU22). Signs of liver fibrosis on B-mode were identified in 21 patients (38%). Splenomegaly was found in 19 patients (35%) and ascites in two patients (4%). Successful pSWE measurements were achieved in the right liver lobe of all individuals and in the left liver lobe of 36 patients (65.5%). PSC patients had significantly higher median shear wave velocity (SWV) than controls in the right liver (median [range] SWV 1.26 [0.73-2.57] m/s vs. 1.09 [0.88-1.25] m/s, p liver lobe and spleen did not differ between PSC patients and controls. Our findings indicate that PSC patients have increased median SWV, indicating more fibrosis compared with controls; however, a wide range of SWV values were obtained among PSC patients, possibly reflecting the various stages in disease development. PMID:27262519

  14. 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.

  15. 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 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 as independent risk factors.Our data strongly suggest that biliary 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. PMID:27124059

  16. 头孢唑肟钠致急性溶血性贫血%Acute hemolytic anemia due to ceftizoxime sodium

    Institute of Scientific and Technical Information of China (English)

    张寒钰

    2015-01-01

    A 70-year-old man with pneumonia received an Ⅳ infusion of ceftizoxime sodium 3 g in 0.9% sodium chloride 250 ml twice daily.Twenty minutes after the first infusion, he developed low back pain, chills and confusion.He presented with moderately jaundiced skin and sclera.Laboratory tests revealed the following results : white blood cell (WBC) count 11 × 109/L, neutrophil 0.8, red blood cell (RBC) count 3.2 × 1012/L, hemoglobin 91 g/L, reticulocytes 0.081, total bilirubin 98 μmol/L, indirect bilirubin 80 μmol/L.Direct Coomb's test(+).He developed strong tea-colored urine.Routine urine tests showed the following results: urine bilirubin (+) , occult blood (+ + +), 0-2 red cells per high power field.Ceftizoxime sodium-induced acute hemolytic anemia was considered.He received hydration and alkalizing of urine.Ceftizoxime sodium was switched to fosfomycin.On day 5, the jaundice disappeared and the color of urine returned to normal.Laboratory tests showed the following results: WBC count 4 × 109/L,RBC count 4.2 × 1012/L, hemoglobin 131 g/L, reticulocytes 0.015, total bilirubin 17 μmol/L, indirect bilirubin 10 μmol/L, urine bilirubin (-), occult blood (-), 0-1 red cells per high power field.%1例70男性患者因肺炎给予头孢唑肟钠3 g加入0.9%氯化钠注射液250 ml静脉滴注,2次/d.首次给药后约20 min,患者突发腰痛、寒战、意识模糊.体格检查见皮肤巩膜中度黄染.实验室检查:外周血白细胞计数11×109/L,中性粒细胞0.8,红细胞计数3.2×1012/L,血红蛋白91 g/L,网织红细胞计数0.081,总胆红素98 μmol/L,间接胆红素80 μmol/L,直接抗人球蛋白试验阳性;尿呈浓茶色,胆红素(+),潜血(+++),红细胞0~2个/HP.考虑头孢唑肟钠致急性药物性溶血性贫血.给予水化及碱化尿液治疗.停用头孢唑肟钠,改用磷霉素抗感染.第5天复查,患者黄染消失、尿色恢复正常,白细胞计数4×109/L,红细胞计数4.2 ×1012/L,血红蛋白131 g

  17. Effectiveness of a drinking-motive-tailored emergency-room intervention among adolescents admitted to hospital due to acute alcohol intoxication - A randomized controlled trial.

    Science.gov (United States)

    Wurdak, Mara; Wolstein, Jörg; Kuntsche, Emmanuel

    2016-06-01

    The aim of this study is to develop and test the effectiveness of a drinking-motive-tailored intervention for adolescents hospitalized due to alcohol intoxication in eight cities in Germany between December 2011 and May 2012 against a similar, non-motive-tailored intervention. In a randomized controlled trial, 254 adolescents received a psychosocial intervention plus motive-tailored (intervention group; IG) or general exercises (control group; CG). Adolescents in the IG received exercises in accordance with their drinking motives as indicated at baseline (e.g. alternative ways of spending leisure time or dealing with stress). Exercises for the CG contained alcohol-related information in general (e.g. legal issues). The data of 81 adolescents (age: M = 15.6, SD = 1.0; 42.0% female) who participated in both the baseline and the follow-up were compared using ANOVA with repeated measurements and effect sizes (available case analyses). Adolescents reported lower alcohol use at the four-week follow-up independently of the kind of intervention. Significant interaction effects between time and IG were found for girls in terms of drinking frequency (F = 7.770, p effect sizes of drinking frequency (d = - 1.18), binge drinking (d = - 1.61) and drunkenness (d = - 2.87) were much higher than the .8 threshold for large effects. Conducting psychosocial interventions in a motive-tailored way appears more effective for girls admitted to hospital due to alcohol intoxication than without motive-tailoring. Further research is required to address the specific needs of boys in such interventions. (German Clinical Trials Register, DRKS ID: DRKS00005588). PMID:26844193

  18. Original single-incision laparoscopic cholecystectomy for acute inflammation of the gallbladder

    Institute of Scientific and Technical Information of China (English)

    Kazunari Sasaki; Goro Watanabe; Masamichi Matsuda; Masaji Hashimoto

    2012-01-01

    AIM:To investigate the safety and feasibility of our original single-incision laparoscopic cholecystectomy (SILC) for acute inflamed gallbladder (AIG).METHODS:One hundred and ten consecutive patients underwent original SILC for gallbladder disease without any selection criteria and 15 and 11 of these were diagnosed with acute cholecystitis and acute gallstone cholangitis,respectively.A retrospective review was performed not only between SILC for AIG and non-AIG,but also between SILC for AIG and traditional laparoscopic cholecystectomy (TLC) for AIG in the same period.RESULTS:Comparison between SILC for AIG and nonAIG revealed that the operative time was longer in SILC for AIG (97.5 min vs 85.0 min,P =0.03).The open conversion rate (2/26 vs 2/84,P =0.24) and complication rate (1/26 vs 3/84,P =1.00) showed no differences,but a need for additional trocars was more frequent in SILC for AIG (5/24 vs 3/82,P =0.01).Comparison between SILC for AIG and TLC for AIG revealed no differences based on statistical analysis.CONCLUSION:Our original SILC technique was adequately safe and feasible for the treatment of acute cholecystitis and acute gallstone cholangitis.

  19. A case of cholestatic autoimmune hepatitis and acute liver failure: an unusual hepatic manifestation of mixed connective tissue disease and Sjögren's syndrome.

    OpenAIRE

    Min, J. K.; Han, N. I.; Kim, J. A; Lee, Y. S.; Cho, C.S.; Kim, H. Y.

    2001-01-01

    Although hepatomegaly is reported to occur occasionally in patients with mixed connective tissue disease (MCTD) or Sjögren's syndrome (SS), autoimmune liver diseases such as primary biliary cirrhosis, sclerosing cholangitis, and autoimmune hepatitis in association with MCTD or SS have rarely been described. We report a case of severe cholestatic autoimmune hepatitis presenting with acute liver failure in a 40-yr-old female patient suffering from MCTD and SS. The diagnosis of MCTD and SS was m...

  20. 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

  1. Systemic corticosteroids and early administration of antiviral agents for pneumonia with acute wheezing due to influenza A(H1N1pdm09 in Japan.

    Directory of Open Access Journals (Sweden)

    Koichiro Kudo

    Full Text Available BACKGROUND: Pneumonia patients with wheezing due to influenza A(H1N1pdm09 were frequently treated with systemic corticosteroids in Japan although systemic corticosteroid for critically ill patients with pneumonia caused by influenza A(H1N1pdm09 has been controversial. Applicability of systemic corticosteroid treatment needs to be evaluated. METHODS/PRINCIPAL FINDINGS: We retrospectively reviewed 89 subjects who were diagnosed with influenza A(H1N1pdm09 and admitted to a national hospital, Tokyo during the pandemic period. The median age of subjects (45 males was 8 years (range, 0-71. All subjects were treated with antiviral agents and the median time from symptom onset to initiation of antiviral agents was 2 days (range, 0-7. Subjects were classified into four groups: upper respiratory tract infection, wheezing illness, pneumonia with wheezing, and pneumonia without wheezing. The characteristics of each group was evaluated. A history of asthma was found more frequently in the wheezing illness (55.6% and pneumonia with wheezing (43.3% groups than in the other two groups (p = 0.017. Corticosteroid treatment was assessed among subjects with pneumonia. Oxygen saturation was lower in subjects receiving corticosteroids (steroid group than in subjects not receiving corticosteroids (no-steroid group (p<0.001. The steroid group required greater oxygen supply than the no-steroid group (p<0.001. No significant difference was found by the Kaplan-Meier method between the steroid and the no-steroid groups in hours to fever alleviation from the initiation of antiviral agents and hospitalization days. In logistic regression analysis, wheezing, pneumonia and oxygen saturation were independent factors associated with using systemic corticosteroids. CONCLUSION: Patients with wheezing and a history of asthma were frequently found in the study subjects. Systemic corticosteroids together with early administration of antiviral agents to pneumonia with wheezing and

  2. 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

  3. 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

  4. Recurrent primary sclerosing cholangitis in the Adult-to-Adult Living Donor Liver Transplantation Cohort Study: Comparison of risk factors between living and deceased donor recipients.

    Science.gov (United States)

    Gordon, Fredric D; Goldberg, David S; Goodrich, Nathan P; Lok, Anna S F; Verna, Elizabeth C; Selzner, Nazia; Stravitz, R Todd; Merion, Robert M

    2016-09-01

    Primary sclerosing cholangitis (PSC) recurs in 15%-25% of patients transplanted for PSC. In the United States, PSC transplant patients are more likely to receive an organ from a living donor (LD) than patients without PSC. Our aims were to (1) compare risk of PSC recurrence in LD versus deceased donor recipients and (2) identify risk factors for PSC recurrence. There were 241 living donor liver transplantations (LDLTs) and 65 deceased donor liver transplantation (DDLT) patients transplanted between 1998 and 2013 enrolled in the Adult-to-Adult Living Donor Liver Transplantation Cohort Study who were evaluated. PSC recurrence risk for LDLT and DDLT recipients was compared using Kaplan-Meier survival curves and log-rank tests. Cox models were used to evaluate PSC risk factors. Overall PSC recurrence probabilities were 8.7% and 22.4% at 5 and 10 years after liver transplantation (LT), respectively. The risk of PSC recurrence was not significantly different for DDLT versus LDLT recipients (P = 0.36). For DDLT versus LDLT recipients, unadjusted 5- and 10-year PSC recurrence was 9.4% versus 9.5% and 36.9% versus 21.1%. Higher laboratory Model for End-Stage Liver Disease (MELD) score at LT, onset of a biliary complication, cholangiocarcinoma, and higher donor age were associated with increased risks of PSC recurrence: for MELD (hazard ratio [HR] = 1.06; 95% confidence interval [CI] 1.02-1.10 per MELD point, P = 0.002); for biliary complication (HR, 2.82; 95% CI, 1.28-6.25; P = 0.01); for cholangiocarcinoma (HR, 3.98; 95% CI, 1.43-11.09; P = 0.008); for donor age (per 5-years donor age; HR, 1.17; 95% CI, 1.02-1.35; P = 0.02). Factors not significantly associated with PSC recurrence included the following: first-degree relative donor (P = 0.11), post-LT cytomegalovirus infection (P = 0.38), and acute rejection (P = 0.22). Risk of recurrent PSC was not significantly different for DDLT and LDLT recipients. Biliary complications

  5. Therapeutic Observation of Acupuncture plus Ozone Injection for Neuralgia Due to Acute Herpes Zoster%针刺配合臭氧穴位注射治疗急性带状疱疹神经痛疗效观察

    Institute of Scientific and Technical Information of China (English)

    王乐荣; 张中会

    2016-01-01

    目的:观察针刺配合臭氧穴位注射治疗急性带状疱疹神经痛的临床疗效。方法将60例急性带状疱疹神经痛患者随机分为治疗组和对照组,每组30例。对照组采用静脉点滴阿昔洛韦注射液、肌肉注射甲钴胺注射液及外用阿昔洛韦软膏治疗。治疗组在对照组治疗基础上采用针刺配合臭氧穴位注射治疗。观察两组患者止疱时间、结痂时间、脱痂时间及治疗前后VAS评分变化情况,并比较两组临床疗效。结果两组止疱时间、结痂时间、脱痂时间比较,差异均具有统计学意义(P<0.01,P<0.05)。两组治疗后VAS评分与同组治疗前比较,差异均具有统计学意义(P<0.01)。治疗组治疗后VAS评分与对照组比较,差异具有统计学意义(P<0.01)。治疗组总有效率为96.7%,对照组为83.3%,两组比较差异具有统计学意义(P<0.05)。结论针刺配合臭氧穴位注射是一种治疗急性带状疱疹神经痛的有效方法。%Objective To observe the clinical efficacy of acupuncture plus ozone injection in treating neuralgia due to acute herpes zoster.Method Sixty patients with neuralgia due to acute herpes zoster were randomized into a treatment group and a control group, 30 cases in each group. The control group was intervened by intravenous injection of Acyclovir, muscular injection of Mecobalamin, and external application of Acyclovir cream. The treatment group was intervened by acupuncture plus zone injection in addition to the treatment given to the control group. The time taken for the blisters to cease, crust, and decrust was observed, as well as the change of Visual Analogue Scale (VAS), and the clinical efficacies were compared between the two groups. Result There were significant inter-group differences in comparing the time for the blisters to cease, crust and decrust (P<0.01, P<0.05). The VAS scores were significantly changed after treatment in both groups (P<0.01). After treatment, the

  6. Loperamide-Induced Acute Pancreatitis

    OpenAIRE

    Halla Vidarsdottir; Hanna Vidarsdottir; Pall Helgi Moller; Einar Stefan Bjornsson

    2013-01-01

    Acute pancreatitis is a common disease leading to hospitalizations, most often caused by gallstones or alcohol. We present a case of a patient diagnosed with acute pancreatitis considered to be due to loperamide treatment for diarrhea.

  7. 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.

  8. 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

  9. Secondary Sclerosing Cholangitis in Critically Ill Patients: Clinical Presentation, Cholangiographic Features, Natural History, and Outcome: A Series of 16 Cases.

    Science.gov (United States)

    Leonhardt, Silke; Veltzke-Schlieker, Wilfried; Adler, Andreas; Schott, Eckart; Eurich, Dennis; Faber, Wladimir; Neuhaus, Peter; Seehofer, Daniel

    2015-12-01

    Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is an important differential diagnosis in patients presenting with cholestasis and PSC-like cholangiographic changes in endoscopic retrograde cholangiography (ERC). As a relatively newly described entity, SSC-CIP is still underdiagnosed, and the diagnosis is often delayed. The present study aims to improve the early detection of SSC-CIP and the identification of its complications.A total of 2633 records of patients who underwent or were listed for orthotopic liver transplantation at the University Hospital Charité, Berlin, were analyzed retrospectively. The clinical presentation and outcome (mean follow-up 62.7 months) of the 16 identified SSC-CIP cases were reviewed.Cholestasis was the first sign of SSC-CIP. GGT was the predominant enzyme of cholestasis. Hypercholesterolemia occurred in at least 75% of the patients. SSC-CIP provoked a profound weight loss (mean 18 kg) in 94% of our patients. SSC-CIP was diagnosed by ERC in all patients. The 3 different cholangiographic features detected correspond roughly to the following stages: (I) evidence of biliary casts, (II) progressive destruction of intrahepatic bile ducts, and (III) picture of pruned tree. Biliary cast formation is a hallmark of SSC-CIP and was seen in 87% of our cases. In 75% of the patients, the clinical course was complicated by cholangiosepsis, cholangitic liver abscesses, acalculous cholecystitis, or gallbladder perforation. SSC-CIP was associated with worse prognosis; transplant-free survival was ∼40 months (mean).Because of its high rate of serious complications and unfavorable prognosis, it is imperative to diagnose SSC-CIP early and to differentiate SSC-CIP from other types of sclerosing cholangitis. Specific characteristics enable identification of SSC-CIP. Early cooperation with a transplant center and special attention to biliary complications are required after diagnosis of SSC-CIP. PMID:26656347

  10. Pre-treatment ferritin level and alveolar-arterial oxygen gradient can predict mortality rate due to acute/subacute interstitial pneumonia in dermatomyositis treated by cyclosporine a/glucocorticosteroid combination therapy: a case control study [corrected].

    Directory of Open Access Journals (Sweden)

    Kentaro Isoda

    Full Text Available BACKGROUND: Acute/subacute interstitial pneumonia in dermatomyositis (DM-A/SIP is a disease associated with a poor prognosis that resists treatment with glucocorticosteroids (GC and progresses rapidly in a period of weeks to months to death. We retrospectively studied outcomes, prognostic factors, and their relations with survival rate in patients with DM-A/SIP treated with early cyclosporine A (CSA/GC combination therapy and 2-hour postdose blood concentration monitoring. METHODS: This study comprised 32 DM-A/SIP patients who were simultaneously treated with CSA and prednisolone. Clinical and laboratory findings were compared between those who died due to DM-A/SIP and those surviving 24 weeks after beginning of therapy. Prognostic factors were extracted, and their relations with the survival rate were evaluated. RESULTS: Of the 32 DM-A/SIP patients, 25 survived, 5 died of DM-A/SIP, and 2 died of infections. In those who died due to DM-A/SIP, ferritin level and the alveolar-arterial oxygen gradient were significantly increased compared with the survivors (P<0.001 and P = 0.002, respectively. Multivariate analyses showed that ferritin and alveolar-arterial oxygen gradient were independent prognostic factors of poor outcome. The survival rate 24 weeks after beginning of treatment was significantly lower in those with a ferritin level of ≥ 600 ng/ml and alveolar-arterial oxygen gradient of ≥ 45 Torr (P<0.001 and P<0.001, respectively. All patients with both prognostic factors died, and the outcome was significantly poorer in these patients than in those with one or neither of the prognostic factors (P<0.001. CONCLUSIONS: We identified pre-treatment high serum ferritin level and high alveolar-arterial oxygen gradient as poor prognostic factors in DM-A/SIP patients undergoing early CSA/GC combination therapy and showed that the outcomes were poor in patients with both factors.

  11. Three ulcerative colitis susceptibility loci are associated with primary sclerosing cholangitis and indicate a role for IL2, REL and CARD9

    OpenAIRE

    Janse, Marcel; Lamberts, Laetitia E.; Franke, Lude; Raychaudhuri, Soumya; Ellinghaus, Eva; MuriBoberg, Kirsten; Melum, Espen; Folseraas, Trine; Schrumpf, Erik; Bergquist, Annika; Bjornsson, Einar; Fu, Jingyuan; Westra, Harm Jan; Groen, Harry JM; Fehrmann, Rudolf SN

    2011-01-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts. Both environmental and genetic factors contribute to its pathogenesis. To further clarify its genetic background, we investigated susceptibility loci recently identified for ulcerative colitis (UC) in a large cohort of 1186PSC patients and 1748 controls. Single nucleotide polymorphisms (SNPs) tagging 13 UC susceptibility loci were initially genotyped in 854...

  12. 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.

  13. 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

  14. Acute Bronchitis

    Science.gov (United States)

    ... of bronchitis: acute and chronic. Most cases of acute bronchitis get better within several days. But your cough ... that cause colds and the flu often cause acute bronchitis. These viruses spread through the air when people ...

  15. 急性脑病起病的晚发枫糖尿症%Acute encephalopathy due to late-onset maple syrup urine disease in a school boy

    Institute of Scientific and Technical Information of China (English)

    屈素清; 杨丽彩; 栾佐; 杜侃; 杨辉

    2012-01-01

    枫糖尿症(maple syrup urine disease,MSUD)是一种较常见的氨基酸代谢障碍性疾病.患者通常在新生儿期至婴幼儿期发病,学龄期以急性脑病起病的病例未见报道.本文报道1例MSUD患儿,8岁6个月首次发病,以感染后急性脑病症状为主要临床表现,于发病第2天时来院,一般化验发现代谢性酸中毒、血尿酸增高、脑脊液蛋白质降低,头颅磁共振扫描显示双侧小脑齿状核、脑干、双侧丘脑、壳核、尾状核及双侧大脑半球皮层呈现长T1,长T2信号,头颅弥散频谱(DWI)示以上部位呈明显高信号.血代谢检查示:血亮氨酸/异亮氨酸、缬氨酸显著高于正常范围;尿液代谢结果示:2-羟基异戊酸、2-羟基丁酸、2-酮异戊酸和2-酮异己酸亦显著增高,提示MSUD.同时游离肉碱明显降低,提示继发性肉碱缺乏、酮症.经控制氨基酸摄入,予大剂量维生素B1、葡萄糖、左旋肉碱静脉点滴,入院后第5天病情明显好转,第7天病情完全恢复,未遗留任何中枢神经系统后遗症.根据患儿治疗效果考虑为硫胺有效型.继续给予维生素B1口服,控制蛋白质饮食(每日1 g/kg)治疗1个半月后复查,血、尿代谢结果完全正常,复查头颅MRI结果明显改善.对以急性脑病起病的病例,要警惕遗传代谢性疾病的可能.如能及时诊断、正确治疗,可以有效地预防中枢神经系统后遗症的发生,改善预后.%Maple syrup urine disease is a common amino acids metabolic disease. In most patients, onset occurs in the neonatal period and infancy. In this study, the case of a school boy with acute encephalopathy due to late-onset maple syrup urine disease is summarized. The boy (8.5 years) was admitted because of acute encephalopathy after suffering from infection for two days at the age of eight and a half years. Metabolic acidosis, hyperuricemia and decreased protein level in cerebrospinal fluid were found by general laboratory tests. Magnetic

  16. Cholangiocarcinoma Secondary to Primary Sclerosing Cholangitis in Explanted Livers: A Single-Center Study in the South of Iran

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    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.

  17. 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

  18. 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.

  19. 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

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

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    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.