Sample records for acute cholangitis due

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

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


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

  2. Assessment of acute cholangitis by MR imaging

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


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

  3. Pathogenic aspects of acute cholangitis

    V. Borisenko


    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

  4. A focus on acute cholecystitis and acute cholangitis

    Massimo Sartelli; Cristian Tranà


    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.

  5. A focus on acute cholecystitis and acute cholangitis

    Massimo Sartelli


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

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

    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)


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

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

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


    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.

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

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


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

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

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


    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.

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

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


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

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

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


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

  12. Endoscopic management of acute cholangitis in elderly patients

    Naresh Agarwal; Barjesh Chander Sharma; Shiv K Sarin


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

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

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


    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.

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

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


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

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

    Yaseen Alastal


    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.

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

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


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

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

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


    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.

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



    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

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

    Pochamana Phisalprapa; Varayu Prachayakul


    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...

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

    Kambiz Yazdanpanah


    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.

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

    Takeuchi H


    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

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

    LINXiu-zhen; GONGYan-ling; WANGHong-bo


    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.

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

    Tohda, Gen; Ohtani, Masahiro; Dochin, Masaki


    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

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

    Manley C Uy


    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

  5. Reasonable options and controversies of laparoscopic treatment for acute cholangitis%腹腔镜手术治疗急性胆管炎合理选择及争议

    赵海鹰; 刘金钢


    急性胆管炎治疗选择与诊断、分级密切相关。中、重度急性胆管炎须立即行胆道引流,国内外指南均推荐首选内镜下鼻胆管引流术(ENBD),经皮经肝胆道引流术(PTCD)与外科手术为备选治疗方案。在对急性胆管炎准确分级前提下,行急诊腹腔镜胆总管探查术(LCBDE)治疗胆总管结石引起的轻、中度急性胆管炎安全可靠。急诊行LCBDE时,轻度胆管炎病人的术中情况与择期手术基本相同,特殊情况少见;中度胆管炎病人可能发生出血量偏大、胆管壁菲薄或偏厚缝合困难等情况,须酌情处理,必要时中转开腹手术。对于重度胆管炎病人,应尽可能避免手术引流,如果ENBD和PTCD失败或存在禁忌证时,可考虑行开腹胆道引流术。%Treatment option of acute cholangitis is closely related to diagnosis and severity assessment. For moderate and severe acute cholangitis,early biliary drainage should be performed. Both of China and aboard guidelines recommend endoscopic naso-biliary drainage(ENBD)as the first choice, and suggest that percutaneous transhepatic cholangial drainage(PTCD)and surgical operation may be considered as alternative methods. With the current technology and accurate severity assessment in acute cholangitis, emergency laparoscopic common bile duct exploration (LCBDE) can be performed safely and effectively for patients with mild and moderate acute cholangitis caused by common bile duct stones. For mild acute cholangitis, emergency LCBDE operation is the same as elective operation. For moderate acute cholangitis,massive bleeding and difficult suture due to thin or thick biliary wall may occur, which need special management even conversion to open operation when necessary. Surgical biliary drainage should be avoided in severe cholangitis. When ENBD and PTCD failed, or contraindications occurred,open surgical biliary drainage should be performed.

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

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


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

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

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


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

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

    王文龙; 郑英键


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

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

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


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

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

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


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

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

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


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

  12. A patient presenting with cholangitis due to Stenotrophomonas maltophilia and Pseudomonas aeruginosa successfully treated with intrabiliary colistine

    Pablo N. Pérez


    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.

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

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


    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.

  14. Acute chylous peritonitis due to acute pancreatitis.

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


    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.

  15. Acute chylous peritonitis due to acute pancreatitis

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


    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.

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

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


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

  17. Liver sinusoidai endothelial cell injury by neutrophils in rats with acute obstructive cholangitis

    Jian-Ping Gong; Chuan-Xin Wu; Chang-An Liu; Sheng-Wei Li; Yu-Jun Shi; Xu-Hong Li; Yong Peng


    AIM: The objective of this study is to elucidate the potentialrole of poly-morphonuclear neutrophils (PMN) in thedevelopment of such a sinusoidal endothelial cell (SEC)injury during early acute obstructive cholangitis (AOC) inrats.METHODS: Twenty one Wistar rats were divided into threegroups: the AOC group, the bile duct ligated group (BDLgroup), and the sham operation group (SO group ) . Thecommon bile duct (CBD) of rats in AOC group was duallyligated and 0.2 mi of the E. coli O111 B4 (5 × 109 cfu/ml)suspension was injected into the upper segment, in BDLgroup, only the CBD was ligated and in SO group, neitherinjection of E. coil suspension nor CBD ligation was done,but the same operative procedure. Such group consisted ofseven rats, all animals were killed 6 h after the operation.Morphological changes of the liver were observed underlight and electron microscope. Expression of intercellularadhesion molecule-1 (ICAM-1) mRNA in hepatic tissue wasdetermined with reverse transcription polymerase chainreaction ( RT-PCR ). The serum levels of alanineaminotransferase (ALT) were determined with anutoanalygerand cytokine-induced neutrophil chemoattractant (ClNC)was determined by enzyme-linked immunosorbent assay( ELISA).RESULTS: Neutrophils was accumulated in the hepaticsinusoids and sinusoidal endothelial cell injury existed inAOC group. In contrast, in rats of BDL group, all thefeatures of SEC damage were greatly reduced. Expressionof ICAM- 1 mRNA in hepatic tissue in three groups were 7.54±0.82, 2.87 ± 0.34, and 1.01 ± 0.12, respectively. Therewere significant differences among three groups ( P< 0.05).The serum ClNC levels in the three groups were 188 ± 21 ng@L-1 , 94 ± 11 ng@ L-1 , and 57 ± 8 ng@ L-1 , respectively. Therewere also significant differences among the three groups ( P< 0.05). Activity of the senum ALT was 917 ± 167 nkat@ L1 , 901 ±171 nkat@ L-1, and 908 ± 164 nkat@L-1, respectively, ( P> 0.05).CONCLUSION: Hepatic SEC injury occurs earlier

  18. TG13 miscellaneous etiology of cholangitis and cholecystitis.

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


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

  19. Sclerosing cholangitis

    ... 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 ...

  20. 化脓性胆管炎的研究进展%Research progress of acute suppurative cholangitis



    急性化脓性胆管炎(acute suppurative cholangitis,ASC)是由于急性胆管梗阻并继发化脓性感染所致,是肝外胆道梗阻的一种严重并发症.本病起病急骤,病情复杂而进展迅速,并发症多而且病死率高,是良性胆道疾患引起病人死亡的主要病症,已引起临床普遍重视.本文对急性化脓性胆管炎的病因、病理变化、诊断标准以及治疗等研究进展作一综述.

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


    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.

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

    Zhuo YANG


    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.

  3. Feline cholecystitis and acute neutrophilic cholangitis: clinical findings, bacterial isolates and response to treatment in six cases.

    Brain, Philip H; Barrs, Vanessa R; Martin, Patricia; Baral, Randolph; White, Joanna D; Beatty, Julia A


    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.

  4. D-penicillamine for primary sclerosing cholangitis

    Klingenberg, S L; Chen, Weikeng


    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....

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

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


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

  6. Risk Factors for Multi-Drug Resistant Pathogens and Failure of Empiric First-Line Therapy in Acute Cholangitis

    Reuken, Philipp A.; Torres, Dorian; Baier, Michael; Löffler, Bettina; Lübbert, Christoph; Lippmann, Norman; Stallmach, Andreas; Bruns, Tony


    Background Acute cholangitis (AC) requires the immediate initiation of antibiotic therapy in addition to treatment for biliary obstruction. Against a background of an increasing prevalence of multi-drug resistant (MDR) bacteria, the risk factors for the failure of empiric therapy must be defined. Methods Using a pathogen-based approach, 1764 isolates from positive bile duct cultures were retrospectively analyzed to characterize the respective pathogen spectra in two German tertiary centers. Using a patient-based approach, the clinical and laboratory data for 83 patients with AC were assessed to identify risk factors for AC with pathogens resistant to the applied empiric therapy. Results Bile cultures were predominantly polymicrobial, and empiric antibiotic therapies did not cover the full biliary pathogen spectrum in 78% of cases. MDR bacteria were isolated from the bile of 24/83 (29%) patients. The univariate risk factors for biliary MDR bacteria were male sex, nosocomial AC, prior antibiotic exposure and prior biliary stenting, of which biliary stenting was the only independent risk factor according to multivariate analysis (OR = 3.8; 95% CI 1.3–11.0, P = 0.013). Although there were no significant differences in survival or hospital stay in AC patients with and without detected biliary MDR pathogens, the former more often had a concomitant bloodstream infection (58% vs. 24%; P = 0.019), including those involving MDR pathogens or fungi (21% vs. 2%; P = 0.007). Conclusion Patients with biliary stents who develop AC should receive empiric therapy covering enterococci and extended-spectrum beta-lactamase (ESBL)-producing Enterobacteriaceae. These patients are at an increased risk for bloodstream infections by MDR pathogens or fungi. PMID:28076388

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

    Hong Yu; Shuo-Dong Wu


    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.

  8. Riskfactorsandincidenceofacutepyogenic cholangitis

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


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

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

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


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

  10. Clinicomicrobiological analysis of patients with cholangitis

    S M Shenoy


    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.

  11. Diagnosis and treatment for acute suppurative cholangitis%急性化脓性胆管炎诊断及治疗

    令狐恩强; 李雪


    @@ 急性化脓性胆管炎(acute suppurative cholangitis,ASC)是指各种原因导致胆管急性梗阻后,胆管内压力升高和细菌感染引起的急性化脓性炎症;临床表现为右上腹部疼痛、发热、黄疸,严重者可以出现肝损害、感染性休克、败血症、肝肾综合征、呼吸衰竭等多器官系统衰竭等临床情况.本病起病急,发展快,病死率高.近年来,随着内镜技术的不断发展,该病的病死率明显下降,得到了比较满意的治疗效果.

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

    Zulfu Bayhan


    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

  13. Acute flaccid paralysis due to rabies

    J B Ghosh


    Full Text Available Two clinical forms of rabies are recognize: i a furious form associated with classical signs of excitation or phobic symptoms, ii Dumb rabies (paralytic rabies characterized by progressive paralysis without an initial furious phase wherein distinction from Guillain-Barrι Syndrome may be difficult. Paralytic rabies is more common in persons who have received postexposure vaccination. We report here the diagnostic dilemma of two cases of acute flaccid paralysis due to rabies.

  14. Monitoring and treatment of acute obstructive suppurative cholangitis%急性梗阻性化脓性胆管炎患者的监测及处理

    杨维良; 张好刚


    急性梗阻性化脓性胆管炎(acute obstructive suppurative cholangitis,AOSC)多为胆管结石、胆道蛔虫或蛔虫残骸阻塞胆管继发胆道细菌感染所致。1983年中华医学会外科学会重庆胆石病研究会议上,确认AOSC在病因、病理上并非是一种特殊的类型,只是一个疾病的过程,因此将此种严重感染性疾病称为急性重症型胆管炎(acute cholangitis severe type,ACST),实质在临床上AOSC与ACST可互为通用,多数医生认为AOSC比ACST更符合临床病理实际过程。

  15. Acute renal failure due to rhabdomyolyisis

    Nieto-Ríos, John Fredy


    Full Text Available Acute renal failure is a frequent cause of morbidity and mortality in emergency, hospitalization and critical care services. In 15 % of cases it is due to rhabdomyolysis, in which there is breakdown of skeletal muscle with massive necrosis and leakage of muscle cell contents into the circulation. It has many different etiologies. The rhabdomyolysis-induced acute kidney injury results from the combination of several mechanisms, including tubular obstruction, vasoconstriction and oxidative stress. The most important therapeutic measures are: Aggressive repletion of fluids, forced diuresis and avoidance of exposure to nephrotoxic substances. In cases of severe uremia, metabolic acidosis, hiperkalemia or fluid overload it is necessary to start renal replacement therapy. As a rule, kidney function is completely recovered, but these patients have higher risk of future chronic kidney disease.

  16. Acute hepatitis due to brucellosis: case report

    Nevil AYKIN


    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.

  17. Autoimmune pancreatitis and cholangitis

    Niraj; Jani; James; Buxbaum


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

  18. Acute oral poisoning due to chloracetanilide herbicides.

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


    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.

  19. Emergency thyroidectomy: Due to acute respiratory failure

    Zulfu Bayhan


    CONCLUSION: Respiratory failure due to giant nodular goiter is a life-threatening situation and should be treated immediately by performing awake endotracheal intubation following emergency total thyroidectomy.

  20. Cardiogenic shock due to acute tramadol intoxication.

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


    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.

  1. Glucocorticosteroids for primary sclerosing cholangitis

    Chen, Weikeng; Gluud, C


    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....

  2. Glucocorticosteroids for primary sclerosing cholangitis

    Giljaca, Vanja; Poropat, Goran; Stimac, Davor


    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...

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

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


    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.

  4. Primary sclerosing cholangitis

    Marina G Silveira


    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.

  5. Primary sclerosing cholangitis

    Chapman Roger


    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.

  6. Cutting edge issues in primary sclerosing cholangitis.

    Bowlus, Christopher L


    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.

  7. Acute small bowel obstruction due to chicken bone bezoar

    Vetpillai P


    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

  8. Primary sclerosing cholangitis and pregnancy

    Casper Q. Kammeijer


    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.

  9. 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



    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。

  10. Bile acids for primary sclerosing cholangitis

    Chen, Weikeng; Gluud, C


    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....

  11. Acute adrenal insufficiency due to primary antiphospholipid antibody syndrome

    Kishore Kumar Behera


    Full Text Available Introduction: We report a case of acute adrenal insufficiency (AAI in a patient with antiphospholipid syndrome (APS. Case Report: A 44-year-old female patient presented to us with acute abdominal pain associated with recurrent vomiting and giddiness. On examination, her blood pressure was 80/50 mm Hg. Systemic examination was normal. Further evaluation revealed hypocortisolemia with elevated plasma adrenocorticotropin hormone indicative of primary adrenal insufficiency. Her abdominal computed tomography scan showed features of evolving bilateral adrenal infarction. Etiological work-up revealed prolonged activated thromboplastin time, which didn′t correct with normal plasma, her anti-cardiolipin antibody and lupus anticoagulant were also positive. She was diagnosed to have APS with adrenal insufficiency and she was started on intravenous steroids and heparin infusion. Conclusion: AAI due to the APS can present with acute abdominal pain followed by hypotension. A high index of suspicion is needed to make the correct diagnosis and to initiate appropriate treatment.

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

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


    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.

  13. Unusual Presentation of Primary Sclerosing Cholangitis

    BC Pineau


    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.

  14. Primary Sclerosing Cholangitis

    A Stiehl


    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.

  15. Acute Anterolateral Myocardial Infarction Due to Aluminum Phosphide Poisoning

    Bita Dadpour


    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

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

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


    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.

  17. Acute Pancreatitis and Splenic Vein Thrombosis due to Hypertriglyceridemia

    Ercan Gündüz


    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.

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

    Kaymakamzade, Bahar; Eker, Amber


    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.

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

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


    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.

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

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


    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.

  1. Small Bowel Perforation due to Gossypiboma Caused Acute Abdomen

    Tahsin Colak


    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.

  2. Pregnancy in primary sclerosing cholangitis

    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


    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

  3. Chronic neck pain disability due to an acute whiplash injury.

    Nederhand, Marc J; Hermens, Hermie J; IJzerman, Maarten J; Turk, Dennis C; Zilvold, Gerrit


    Several theories about musculoskeletal pain syndromes such as whiplash-associated disorder (WAD) suggest that pain and muscle activity interact and may contribute to the chronicity of symptoms. Studies using surface electromyography (sEMG) have demonstrated abnormal muscle activation patterns of the upper trapezius muscles in the chronic stage of WAD (grade II). There are, however, no studies that confirm that these muscle reactions are initiated in the acute stage of WAD, nor that these muscle reactions persist in the transition from acute neck pain to chronic neck pain disability. We analyzed the muscle activation patterns of the upper trapezius muscles in a cohort of 92 subjects with acute neck pain due to a motor vehicle accident (MVA). This cohort was followed up in order to evaluate differences in muscular activation patterns between subjects who have recovered and those subjects who have not recovered following an acute WAD and developed chronic neck pain. sEMG parameters were obtained at 1, 4, 8, 12, and 24 weeks after an MVA. The level of muscle reactivity (the difference in pre- and post-exercise EMG levels) and the level of muscle activity during an isometric and a dynamic task were used as EMG parameters. The results revealed no elevated muscle reactivity either in the acute stage, or during the follow-up period. The results of both the isometric and dynamic task, showed statistically significant different EMG levels between four neck pain disability subgroups (analysis of variance reaching P-levels of 0.000), with an inverse relationship between the level of neck pain disability and EMG level. Furthermore, follow-up assessments of the EMG level during these two tasks, did not show a time related change. In conclusion, in subjects with future disability, the acute stage is characterized by a reorganization of the muscular activation of neck and shoulder muscles, possibly aimed at minimizing the use of painful muscles. This change of motor control, is in

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

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


    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.


    Tagle, Martín; Bussalleu, Alejandro


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

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

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


    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.

  7. Acute metheamoglobinemia due to nitrobenzene poisoning: Case series

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


    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

  8. Gastric perforation due to foreign body ingestion mimicking acute cholecystitis.

    Henneman, Daniel; Bosman, Willem-Maarten; Ritchie, Ewan D; van den Bremer, Jephta


    An 82-year-old man presented with signs and symptoms that were suggestive of acute cholecystitis. He underwent a laparoscopic cholecystectomy. During the intervention, a wooden foreign body was removed from the infiltrated omentum, probably after it had perforated the gastric antrum. The gastric perforation had led to a secondary infection of the gallbladder. The presumed gastric perforation was treated conservatively, and the patient recovered well and was discharged after 7 days. Secondary inflamed gallbladders are rare; the current case is, to the best of our knowledge, the first case reporting a secondary infection of the gallbladder due to a gastric perforation. Clinicians should be aware of possible ingestion of foreign bodies in elderly patients wearing dental prosthetic devices.

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

    Perković-Vukčević Nataša


    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

  10. MRCP findings in recurrent pyogenic cholangitis

    Jain, Manoj [Department of Radiology, AMRI Hospitals, Salt Lake, JC-16 and 17 Salt Lake City, Kolkata 700091 (India)], E-mail:; Agarwal, Ajay [Department of Radiology, AMRI Hospitals, Salt Lake, JC-16 and 17 Salt Lake City, Kolkata 700091 (India)], E-mail:


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

  11. Bile acids for primary sclerosing cholangitis

    Poropat, Goran; Giljaca, Vanja; Stimac, Davor


    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...

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

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


    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.

  13. An Acute Disseminated Encephalomyelitis Case due to Mycobacterium tuberculosis

    Hale Turan Özden


    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

  14. Endoscopy therapy on acute purlent cholangitis%急性化脓性胆管炎的内镜治疗现状

    李国华; 吕农华


    急性化脓性胆管炎(acute suppurative cholangitis,ASC)是指各种原因导致胆管急性梗阻后,胆管内压力升高和细菌感染引起的急性化脓性炎症。临床表现为右上腹部疼痛、发热、黄疸,严重者可以出现肝损害、感染性休克、败血症、肝肾综合征、呼吸衰竭等多器官系统衰竭等。本病起病急,发展快,病死率高。近年来,随着内镜技术的不断发展,该病的病死率明显下降。

  15. Acute acalculous cholecystitis due to an incarcerated epigastric hernia.


    A case of a 96 year old woman with an incarcerated gallbladder in an epigastric hernia causing acute acalculous cholecystitis is described. At operation the gallbladder was replaced in its anatomical site and the hernia was repaired. This unique case of acute acalculous cholecystitis demonstrates cystic duct obstruction and cystic vessel compression as possible aetiologies for this pathology.

  16. Damage control surgery for acute obstructire cholangitis%损伤控制技术在急性化脓性梗阻性胆管炎中的应用

    汪东文; 简明; 鲁建国; 宋军; 刘凡; 杜锡林; 樊巍巍


    目的 探讨损伤控制性外科(DCS)技术治疗急性化脓性梗阻性胆管炎(AOSC)的方法和疗效.方法 回顾性分析我们2001年10月至2008年10月收治的72例AOSC患者的控制性手术和后续治疗的情况.结果 72例患者均经DCS治疗.经外科重症监护病房(SICU)复苏后,20例患者痊愈,49例接受再次确定性手术.全组痊愈67例(93.1%),再次确定性手术后胆肠吻合口漏1例(1.4%),胆道出血2例(2.8%),肝断面及膈下感染3例(4.2%),均经相应治疗痊愈;放弃后续治疗3例(4.2%),死亡2例(2.8%).结论 对AOSC患者应根据不同的病因,选择适当的DCS术式和再次确定性手术,其疗效是满意的.%Objective To explore the clinical efficacy of damage control surgery (DCS)in the treatment of acute obstructive suppurative cholangitis ( AOSC ).Methods The clinical data of 72 AOSC patients who were admitted to our hospitals between Oct 2001 and Oct 2008 and underwent DCS were retrospectively analyzed.Results Among the 72 patients,20 were cured and 49 received re - operations after surgical intensive care unit( SICU )resuscitation.The cure rate reached 93.1 %.There was choleintestinal anostomotic stoma fistula in 1 patient, hemobilia in 2 and subdiaphragmatic and resectional surface infections in 3 after re - operations, and these patients were eventually cured by corresponding treatments.Three patients abandoned the treatment after the operation.Mortality rate was 2.8%.Conclusion DCS is effective in the treatment of AOSC.

  17. Diffuse Alveolar Hemorrhage due to Acute Mitral Valve Regurgitation.

    Marak, Creticus P; Joy, Parijat S; Gupta, Pragya; Bukovskaya, Yana; Guddati, Achuta K


    Diffuse alveolar hemorrhage (DAH) can be caused by several etiologies including vasculitis, drug exposure, anticoagulants, infections, mitral valve stenosis, and regurgitation. Chronic mitral valve regurgitation (MR) has been well documented as an etiological factor for DAH, but there have been only a few cases which have reported acute mitral valve regurgitation as an etiology of DAH. Acute mitral valve regurgitation can be a life-threatening condition and often requires urgent intervention. In rare cases, acute mitral regurgitation may result in a regurgitant jet which is directed towards the right upper pulmonary vein and may specifically cause right-sided pulmonary edema and right-sided DAH. Surgical repair of the mitral valve results in rapid resolution of DAH. Acute MR should be considered as a possible etiology in patients presenting with unilateral pulmonary edema, hemoptysis, and DAH.

  18. Diffuse Alveolar Hemorrhage due to Acute Mitral Valve Regurgitation

    Creticus P. Marak


    Full Text Available Diffuse alveolar hemorrhage (DAH can be caused by several etiologies including vasculitis, drug exposure, anticoagulants, infections, mitral valve stenosis, and regurgitation. Chronic mitral valve regurgitation (MR has been well documented as an etiological factor for DAH, but there have been only a few cases which have reported acute mitral valve regurgitation as an etiology of DAH. Acute mitral valve regurgitation can be a life-threatening condition and often requires urgent intervention. In rare cases, acute mitral regurgitation may result in a regurgitant jet which is directed towards the right upper pulmonary vein and may specifically cause right-sided pulmonary edema and right-sided DAH. Surgical repair of the mitral valve results in rapid resolution of DAH. Acute MR should be considered as a possible etiology in patients presenting with unilateral pulmonary edema, hemoptysis, and DAH.

  19. Chemotherapy-induced sclerosing cholangitis

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


    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.

  20. Acute generalized exanthematous pustulosis due to insect bites?

    Yasmeen J Bhat


    Full Text Available 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 as the etiological agent in these cases of AGEP which are the first such reported cases in India.

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

    Köklü, Erkan, E-mail:; 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)


    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.

  2. Primary sclerosing cholangitis and liver transplantation

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


    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.

  3. Autoantibodies in primary sclerosing cholangitis

    Johannes Roksund Hov; Kirsten Muri Boberg; Tom H Karlsen


    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.

  4. Etiopathogenesis of primary sclerosing cholangitis

    Roger Chapman; Sue Cullen


    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.

  5. Ampullary duodenal diverticulum and cholangitis

    Joaquim Mendes Castilho Netto

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

  6. Acute Gastric Bleeding Due to Giant Hyperplastic Polyp

    Bulent Aksel


    Full Text Available Hyperplastic gastric polyps account for the majority of benign gastric polyps. The vast majority of these lesions are small, asymptomatic and found incidentally on radiologic or endoscopic examination. Giant hyperplastic gastric polyps are uncommon and most of them are asymptomatic. We report a case of a 66-year-old woman who admitted because of acute gastric bleeding. The gastrin levels were within normal ranges. Esophagogastroduodenoscopy showed 12 cm pedunculated and multiple lobulated hyperplastic polyps arising from antrum with signs of diffuse oozing. The patient is treated by subtotal gastrectomy with Roux-Y gastrojejunostomy. Histological examination showed the presence of ulcers and regeneration findings with the contemporary occurrence of hyperplastic polyp. Giant hyperplastic gastric polyp should be kept in mind in the differential diagnosis of acute upper gastrointestinal bleeding.

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

    Rashmee Patil


    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.

  8. An unusual case of reversible acute kidney injury due to chlorine dioxide poisoning.

    Bathina, Gangadhar; Yadla, Manjusha; Burri, Srikanth; Enganti, Rama; Prasad Ch, Rajendra; Deshpande, Pradeep; Ch, Ramesh; Prayaga, Aruna; Uppin, Megha


    Chlorine dioxide is a commonly used water disinfectant. Toxicity of chlorine dioxide and its metabolites is rare. In experimental studies, it was shown that acute and chronic toxicity were associated with insignificant hematological changes. Acute kidney injury due to chlorine dioxide was not reported. Two cases of renal toxicity due to its metabolites, chlorate and chlorite were reported. Herein, we report a case of chlorine dioxide poisoning presenting with acute kidney injury.

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

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


    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.

  10. Acute Otitis due to Vibrio fluvialis after Swimming

    Ping-Jen Chen


    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.

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

    Loabat Geranpayeh


    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.

  12. Acute cortical blindness due to posterior reversible encephalopathy.

    Nguyen-Lam, Jenny; Kiernan, Matthew C


    An acutely hypertensive 55 year-old male experienced seizures and cortical blindness post-operatively. CT scans demonstrated hypointensities in the occipital lobes bilaterally. MRI revealed symmetrical bilateral hyperintense signals in the same region, involving both grey and white matter. Thromboembolic screening investigations including vertebral artery doppler studies were normal and echocardiography demonstrated borderline left ventricular hypertrophy. A diagnosis of posterior reversible encephalopathy syndrome (PRES) was reached and there was complete resolution of blindness with antihypertensive therapy. This case supports the vasogenic theory of PRES which suggests that sustained high grade fluctuations in blood pressure lead to a reduction in cerebral vascular autoregulatory function. The resultant failure of compensatory vasoconstriction to prevent hyperperfusion causes fluid to extravasate into the occipital lobes, which in the present case resulted in cortical blindness.

  13. Pulmonary hypertension due to acute respiratory distress syndrome

    S.A. Ñamendys-Silva


    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.

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

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


    @@ 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.

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

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


    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

  16. Acute respiratory infection due to : current status of diagnostic methods

    Loens, K.; Goossens, H.; Ieven, M


    Abstract Because of the absence of well-standardized both in-house and FDA-approved commercially available diagnostic tests, the reliable diagnosis of respiratory infection due to Mycoplasma pneumoniae remains difficult. In addition, no formal external quality assessment schemes which would allow to conclude about the performance of M. pneumoniae diagnostic tests exist. In this review, the current state of knowledge of M. pneumoniae-associated respiratory infections in the context ...

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

    Yahya Ayhan Acar


    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.

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

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


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

  19. Papillary muscle rupture due to acute myocardial infarction followed by cardiogenic shock, pulmonary edema, and acute renal failure

    Ilić Radoje


    Full Text Available Aim. The case of successful surgical treatment of anterolateral papillary muscle rupture due to acute myocardial infarction with cardiogenic shock, pulmonary edema and acute renal failure. Case report. A 62-year old male from Belgrade with chest pain, hypotension and a new heart murmur refused hospitalization at the Military Medical Academy. On the third day of his illness he was readmitted to MMA as an emergency due to hemoptysis. Examination revealed mitral valve anterolateral papillary muscle rupture. The patient, with signs of cardiogenic shock and acute renal failure, was immediately operated on. The surgery was performed using extracorporeal circulation. An artificial mitral valve was implanted, and myocardial revascularization accomplished with one venous graft of the left anterior descending artery. On the second postoperative day, hemodialysis was carried out due to acute renal failure. On the 28th postoperative day, the patient was discharged from the hospital being hemodynamically stable with normal renal function and balanced anticoagulation. The case is interesting in terms of unrecognized papillary muscle rupture that led to the development of cardiogenic shock, hemoptysis and acute renal failure. Conclusion. Papillary muscle rupture is a fatal complication of acute myocardial infarction. Early recognition and urgent surgical intervention were lifesaving in the case of complete papillary muscle rupture. Surgical treatment, regardless of high risk, is the procedure of choice.

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

    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


    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

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

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


    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.

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

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


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

  3. Acute colonic obstruction due to benign prostatic hypertrophy.

    Mac Giobuin, S


    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.

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

    Sarkar, Souvik; Bowlus, Christopher L


    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.

  5. Diffusion MRI features of acute encephalopathy due to stopping steroid medication abruptly in congenital adrenal hyperplasia

    Asli Serter


    Full Text Available Congenital adrenal hyperplasia (CAH is characterized by adrenal steroid biosynthesis defect. Steroid replacement therapy should be performed regularly in these patients. Adrenal crisis may be present in acute stress due to increased cortisol requirements or in steroid deficiency due to stopping steroid medication abruptly. In patients with acute adrenal insufficiency, severe hypotension or hypovolemic shock occurs typically. Acute encephalopathy can be seen due to hypoxia, hypervolemia, or hypoglycemia. Diffusion restriction can be seen in cortical-subcortical regions of frontal and parieto-occipital lobes and in splenium of corpus callosum. In CAH patients with neurologic symptoms, Diffusion weighted images (DWI is very important in the diagnosis and follow-up of acute encephalopathy.

  6. Acute pancreatitis due to ascariasis: a sonographic pictorial essay on four cases.

    Lynser, Donboklang; Thangkhiew, R S; Laloo, Demitrost; Hek, M D; Marbaniang, Evarisalin; Tariang, Satisfy


    Ascariasis is the commonest helminthic disease to infect humans. Due to their wandering nature, the roundworms from the second part of the duodenum migrate through the biliary opening into the hepatobiliary and pancreatic ducts. Ascariasis is the most common parasitic cause of pancreatitis in endemic region. Pancreatitis can result due to pancreatic ascariasis, biliary ascariasis or both. Pancreatitis due to ascariasis can be severe and life-threatening. We present a pictorial essay of acute pancreatitis due to ascariasis on four cases.

  7. An unusual cause of acute kidney injury due to oxalate nephropathy in systemic scleroderma.

    Mascio, Heather M; Joya, Christie A; Plasse, Richard A; Baker, Thomas P; Flessner, Michael F; Nee, Robert


    Oxalate nephropathy is an uncommon cause of acute kidney injury. Far rarer is its association with scleroderma, with only one other published case report in the literature. We report a case of a 75-year-old African-American female with a history of systemic scleroderma manifested by chronic pseudo-obstruction and small intestinal bacterial overgrowth (SIBO) treated with rifaximin, who presented with acute kidney injury with normal blood pressure. A renal biopsy demonstrated extensive acute tubular injury with numerous intratubular birefringent crystals, consistent with oxalate nephropathy. We hypothesize that her recent treatment with rifaximin for SIBO and decreased intestinal transit time in pseudo-obstruction may have significantly increased intestinal oxalate absorption, leading to acute kidney injury. Oxalate nephropathy should be considered in the differential diagnosis of acute kidney injury in scleroderma with normotension, and subsequent evaluation should be focused on bowel function to include alterations in gut flora due to antibiotic administration.

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

    Bjarte Fosby; Tom H Karlsen; Espen Melum


    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.

  9. A case report of acute acalculous cholecystitis due to Salmonella Paratyphi B complicated by biliary peritonitis.

    Benjelloun, El Bachir; Chbani, Leila; Toughrai, Iman; Ousadden, Abdelmalek; Mazaz, Khalid; Taleb, Kahlid Ait


    Non-typhoidal salmonella are a rare case of acute acalculouscholecystitis (AAC). Salmonella Paratyphi B, which accounts for one of the less invasive NTS serotypes, has rarely been reported to cause cholecystitis. We describe a case of 65-year old previously healthy man, who present with signs of acute abdomen, due to biliary peritonitis as a complication of acute acalculouscholecystitis caused by Salmonella paratyphi B. Our case illustrates the potential severity of infection with Salmonella Paratyphi B especially in older patient. High index of awarenessshould be considered in endemic areas.

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

    Metin Çeliker


    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.

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

    Boulyana, M.


    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...

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

    Massa Zantah


    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.

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

    Canas Nuno


    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.

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

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


    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....

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

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


    @@ 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.

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

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


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

  17. IgG4 Related Sclerosing Cholangitis

    D. Joshi


    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.

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

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


    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.

  19. IgG4-associated cholangitis.

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


    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.

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

    M. Boulyana


    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.

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

    Kipp, Ryan; Raval, Amish N


    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.

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

    Patel, Utpal; Sriram, Krishnan


    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.

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

    Erarslan, Elife; Yüksel, Ilhami; Haznedaroglu, Serap


    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.

  4. Acute renal failure due to bilateral uric acid lithiasis in infants.

    Kaya, Mete; Boleken, M Emin; Soran, Mustafa; Kanmaz, Turan; Yücesan, Selçuk


    Acute renal failure (ARF) is one of the complications of urolithiasis, but the role of medical treatment to relieve urinary obstruction in children with ARF is uncertain. We report on infants with acute obstructive uric acid lithiasis. We describe presentation features as well as diagnosis methods and medical treatment in five infants who were admitted to our institution with ARF due to uric acid lithiasis. The medical treatments for all patients were fluid liberalization, urine alkalinization, and oral allopurinol. Two children underwent urinary diversion. Within 8 h, urine output improved in all patients, and the stones passed spontaneously. All obstructed kidneys were relieved with medical treatment, and no renal sequel remained. So this series has showed a role of medical therapy in acute obstructive uric acid lithiasis.

  5. Adjuvant Use of Ivabradine in Acute Heart Failure due to Myocarditis

    Jennifer Franke


    Full Text Available We report two cases of young men in whom acute heart failure due to myocarditis was diagnosed. The patients had been transferred to the intensive care unit (ICU with commencing symptoms of acute heart failure and consecutive multiorgan failure for further treatment and to evaluate the indication for implantation of a ventricular assist device or for high urgent orthotopic heart transplantation. In both patients, the If-channel inhibitor ivabradine was administered off-label to provide selective heart rate reduction, and thus support hemodynamic stabilization. Though currently considered off-label use in patients suffering from severe hypotension and acute heart failure, the use of ivabradine may beneficially influence outcome by allowing optimization of the patient's heart rate concomitant to initial measures of clinical stabilization.

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

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


    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.


    Thulaseedharan Nallaveettil


    Full Text Available BACKGROUND Acute liver failure is a heterogeneous entity and its prognosis varies with the aetiology. In India and other developing countries, hepatitis A virus is an important cause of acute liver failure. The prognostic factors and outcome of such patients should be studied separately. AIM OF THE STUDY To study the outcome of patients with acute liver failure due to hepatitis A treated with intensive supportive care and to determine the prognostic factors predicting the transplant free survival. MATERIALS AND METHODS In this observational study, all patients admitted in our hospital with ALF due to hepatitis A virus infection during the period of 3 years from January 1st 2013 to December 31st 2015 were selected; 40 patients satisfied the inclusion and exclusion criteria. Detailed history taking, physical examination, haematological and biochemical investigations were performed. The day-to-day progress and treatment given until discharge or death were recorded. RESULTS Overall mortality in acute liver failure due to hepatitis A was 30%. Transplant free survival was 100% in patients with grade I and II encephalopathy, 66.6% in grade III encephalopathy and 22.2% in grade IV encephalopathy (P less than 0.001. Extrahepatic manifestations were observed in 29 patients (72.5%, the most common was thrombocytopenia in 22 patients (55% followed by acute kidney injury in 12 patients (30%. CONCLUSIONS The grade of hepatic encephalopathy was the single most important factor that determined the prognosis. Patients with grade I and II encephalopathy had 100% spontaneous survival rate.

  8. Autoimmune Cholangitis: A Variant Syndrome of Autoimmune Hepatitis

    Brij Sharma; Sujeet Raina; Rajesh Sharma


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

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

    Rita Jermyn


    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.

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

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


    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

  11. [Acute respiratory insufficiency due to COPD: invasive mechanical ventilation or not?].

    Kant, K Merijn; Djamin, Remco S; Belderbos, Huub N A; van den Berg, Bart


    The decision to move to a form of mechanical ventilation in patients with acute respiratory failure due to an acute exacerbation of COPD is influenced by expectations about survival and quality of life after discharge from the ICU. Physicians tend to be too pessimistic about the survival outcome of an ICU stay with invasive mechanical ventilation. The forced expiratory volume in 1 second (FEV1) is not an adequate prognostic parameter. In order to prevent undertreatment of patients with respiratory failure due to an exacerbation of COPD, knowledge of prognostic parameters and quality of life in these patients is very important. End of life care should be integrated into the standard care of COPD patients.

  12. Malnutrition and inflammation in acute kidney injury due to earthquake-related crush syndrome


    Abstract Background Malnutrition and inflammation are common and serious complications in patients with acute kidney injury (AKI). However, the profile of these complications in patients with AKI caused by crush syndrome (CS) remains unclear. This study describes the clinical characteristics of malnutrition and inflammation in patients with AKI and CS due to the Wenchuan earthquake. Methods One thousand and twelve victims and eighteen healthy adults were recruited to the study. They were divi...

  13. Unusual presenting of acute aortic dissection due to penetrating atheromatous ulcer.

    Atas, Halil; Durmus, Erdal; Sunbul, Murat; Birkan, Yasar; Ozben, Beste


    Penetrating atheromatous ulcer (PAU) is an atherosclerotic ulcer penetrating the internal elastic lamina of the aortic wall causing a hematoma within the media layer of aorta. They are commonly located in the descending aorta of the elderly and hypertensive patients. They may rarely be complicated by aortic dissection. We report a relative young normotensive patient presenting with acute aortic dissection due to PAU located in the ascending aorta.

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

    Akram, Javed; Josefsson, Pernilla; Rømeling, Frans


    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.

  15. Diffusion MRI features of acute encephalopathy due to stopping steroid medication abruptly in congenital adrenal hyperplasia

    Asli Serter; Alpay Alkan; Demet Demirkol


    Diffusion MRI features of acute encephalopathy due to stopping steroid medication abruptly in congenital adrenal hyperplasia Asli Serter, Alpay Alkan and Demet Demirkol Annals of Indian Academy of Neurology. 18.3 (July-September 2015): p342. Copyright: COPYRIGHT 2015 Medknow Publications and Media Pvt. Ltd. Full Text: Byline: Asli. Serter, Alpay. Alkan, Demet. Demirkol Congenital adrenal hyperplasia (CAH) is characterized by adrenal steroid biosynthesis defect. Ste...

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

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


    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.

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

    Peter J. McGuire


    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

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

    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)


    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.

  19. An unusual presentation of primary sclerosing cholangitis

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


    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.

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

    Hakan Ozkan


    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.

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

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


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

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

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


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

  3. Acute post-infectious cerebellar ataxia due to co-infection of human herpesvirus-6 and adenovirus mimicking myositis.

    Naselli, Aldo; Pala, Giovanna; Cresta, Federico; Finetti, Martina; Biancheri, Roberta; Renna, Salvatore


    Acute cerebellar ataxia (ACA) is a relatively common neurological disease in children. Most common types of ACA are acute post-infectious (APCA) and acute disseminated encephalomyelitis (ADEM). Less common but important causes include opsoclonus-myoclonus syndrome (OMS) and acute cerebellitis. Cerebellar neoplasms and acute hydrocephalus are additional causes of paediatric ataxia. APCA is the most common cause of ACA in children, comprising about 30-50% of total cases. This is a report about an immunocompetent 4-yrs-old male affected by APCA, due to co-infection by human herpesvirus-6 (HHV-6) and adenovirus, with symptoms mimicking myositis.

  4. Laparoscopic exploration of the common bile duct and removal of dead worm in a patient of cholangitis after endoscopic retrograde cholangiopancreatography failure

    Chalkoo Mushtaq


    Full Text Available We describe a dead ascaris-induced extrahepatic bilary obstruction in a young female who presented with acute cholangitis. The dead ascaris was removed by laparoscopic exploration of common bile duct after endoscopic retrograde cholangiopancreatography failure. Patient had an uneventful hospital course after the procedure and was discharged afebrile after 3 days of hospital stay.

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

    Hyung Hun Kim


    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.

  6. Acute airway obstruction due to spontaneous intrathyroid hemorrhage precipitated by anticoagulation therapy

    Laxmi Kokatnur


    Full Text Available Acute airway compromise due to hemorrhage in of thyroid gland is a rare life-hreating condition. The increasing use of anticoagulants for various reasons is likely increased the occurrence of this this complication. We describe an elderly patient on anticoagulation for atrial fibrillation, which developed swelling on the right side of neck causing acute airway obstruction requiring emergency intubation for airway protection. Computed tomographic scan showed massive intrathyroid hemorrhage along with substernal extension. She had supratherapeutic INR which was appropriately corrected emergently. She underwent resection of the thyroid gland which showed multinodular goiter without any evidence of malignancy. Our case illustrates the rare but lethal bleeding complication of anticoagulants in critical anatomical area and we request physicians should be wary of similar conditions.

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

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


    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.

  8. Evolving concepts in primary sclerosing cholangitis.

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


    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.

  9. 内镜介入治疗急性化脓性胆管炎对 HMGBl、sTREM -1和 hs - CRP 的影响%Effect of endoscopic therapy on HMGB1,sTREM - 1 and hs - CRP in the treatment for acute suppurative cholangitis

    张毅; 沈锋


    目的:观察比较内镜介入和开腹治疗急性化脓性胆管炎的临床疗效及对血清高迁移率族蛋白 B1(HMGBl)、可溶性髓样细胞触发受体-1( sTREM -1)和高敏 C 反应蛋白(hs - CRP)的影响。方法按照手术方式不同将82例急性化脓性胆管炎患者分为2组,观察组47例予以内镜介入治疗,对照组35例予以开腹手术治疗。观察2组休克纠正、腹痛缓解、下床活动、胃肠功能恢复及住院时间和并发症发生率,检测2组治疗前和治疗后3 d 白细胞计数(WBC)、丙氨酸氨基转移酶( ALT)、总胆红素( TBil)、HMGB1、sTREM -1和 hs - CRP水平。结果观察组休克纠正、腹痛缓解、下床活动、胃肠功能恢复和住院时间均明显短于对照组(P 均﹤0.05),并发症发生率明显低于对照组( P ﹤0.05)。2组治疗前 WBC、ALT、TBil、HMGB1、sTREM -1和 hs - CRP 水平比较差异均无统计学意义(P 均﹥0.05),2组治疗后各指标水平均较治疗前明显降低(P 均﹤0.05),且观察组各指标水平明显低于对照组( P 均﹤0.05)。结论内镜介入治疗急性化脓性胆管炎具有创伤小、恢复快、黄疸消退快等优点,且可明显减轻机体的炎症反应。%Objective It is to investigate the efficacy of endoscopic therapy and open surgery in treatment of acute suppu-rative cholangitis,and their impact on high mobility group box - 1 protein(HMGBl),soluble triggering receptor expressed on myloid cells - 1(sTREM - 1)and high-sensitive C - reactive protein(hs - CRP). Methods 82 patients with acute suppura-tive cholangitis were divided into observation group(47 cases)and control group(35 cases)according to different modus ope-randi,observation group were performed with endoscopic therapy,and control group were gaved laparotomy. Shock recovery, abdominal pain,out-of-bed activity,recovery of gastrointestinal function and hospital stay and complication rates were

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

    Larsen Erling


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

  11. Acute stenting and thromboaspiration in basilar artery occlusions due to embolism from the dominating vertebral artery

    Nedeltchev, K.; Remonda, L.; Brekenfeld, C.; Ozdoba, C.; Schroth, G. [Institute of Diagnostic and Interventional Neuroradiology, University of Bern, Inselspital, Freiburgstrasse 4, 3010, Bern (Switzerland); Do, D.D. [Institute of Angiology, University of Bern, Inselspital, Bern (Switzerland); Arnold, M.; Mattle, H.P. [Department of Neurology, University of Bern, Inselspital, Bern (Switzerland)


    Intra-arterial thrombolysis (IAT) is the only treatment that has demonstrated benefit in patients with acute basilar artery occlusions (ABAO). IAT may be difficult to perform when access to the occluded basilar artery (BA) is prevented by pathology of the vertebral arteries (VA). We report on two patients with ABAO due to embolism from the dominating VA. Catheter navigation through the occluded VA and thromboaspiration enabled access to the BA. Thromboaspiration alone or in addition to IAT resulted in a complete recanalization of the BA and a favorable clinical outcome. A stent was deployed at the site of the stenosis in the VA either prior to or immediately after BA recanalization. (orig.)

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

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


    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)

  13. [Acute respiratory insufficiency due to severe lung injury - ARDS and ALI].

    Pfeifer, M


    As a consequence of the novel therapeutic option of mechanical ventilation in early intensive care medicine, the acute respiratory distress syndrome (ARDS) was defined as a disease entity of its own representing the most severe form of acute lung injury (ALI). Since its first description four decades ago, our knowledge about the aetiology, physiology, histology and epidemiology of this lethal pulmonary complication of severe acute diseases such as pneumonia or sepsis has been increasing steadily. The initial major therapeutic advances were due to improvements in intensive care medical procedures and monitoring. The large ARDS Network clinical trial on the magnitude of tidal volume impressively demonstrated the feasibility of targeted clinical trials in patients with ARDS that provide robust evidence in this field. This clinical trial, as well as following large-scale trials in ARDS patients, led to significant changes of ventilation therapy and therapeutic strategies that improve the outcome of this disease entity. Advances in the standardisation of care for ARDS patients involving innovative therapeutic procedures such as extracorporeal gas exchange systems will lead to a further improvement in ARDS management and outcome. Modern pulmonary medicine can play a pivotal role in this process and can contribute its rich experiences in all areas of the respiratory system.

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

    Singhal, Sameer; Wagh, D D; Kashikar, Shivali; Lonkar, Yeshwant


    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.

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

    Sameer Singhal; DD Wagh; Shivali Kashikar; Yeshwant Lonkar


    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.

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

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


    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.

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

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


    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.

  18. Biochemical and biomolecular aspects of oxidative stress due to acute and severe hypoxia in human muscle tissue.

    Corbucci, G G; Sessego, R; Velluti, C; Salvi, M


    Mitochondrial oxidative stress was investigated in severe and acute hypoxia and in reperfusion applied to human muscle tissues. The biochemical and biomolecular relationship between the response of the respiratory-chain enzymic complexes and the metabolism of specific hypoxia stress proteins (HSP) suggest an adaptive mechanism which antagonizes the oxidative damage due to acute and severe tissue hypoxia.

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

    Paul, Ian M


    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.

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

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


    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.

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

    van den Berg, A; Jansen, PLM


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

  2. Genetic epidemiology of primary sclerosing cholangitis


    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).

  3. Clinical features and management of primary sclerosing cholangitis

    Marina G Silveira; Keith D Lindor


    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.

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

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


    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.

  5. Readmissions due to acute biliary edematous pancreatitis in patients without cholecystectomy

    Eva Barreiro-Alonso

    Full Text Available Objectives: Analyzing the readmission of patients with acute biliary edematous pancreatitis (ABEP without cholecystectomy despite a previous episode of mild acute gallstone pancreatitis or lithiasic cholecystitis. Calculating the health costs associated with the non-performance of cholecystectomy. Materials and methods: Prospective observational study conducted at a tertiary hospital (Hospital de Cabueñes. Gijón, Asturias. Spain from July to November 2014. The study has consecutively included inpatients suffering from ABEP who: a had suffered a previous episode of mild acute gallstone pancreatitis or cholecystitis at least 2 weeks before readmission; and b had not undergone cholecystectomy despite the lack of contraindications. Results: During the research period, 9 patients (7 females and 2 males with a mean age of 65.3 years (standard deviation [SD] 19.2 were readmitted. The median number of days between the previous episode of ABEP or cholecystitis and the readmission was 114 days (interquartile range [IQR] 111.0. Reported median overall length of hospital stay was 10 days (IQR = 2.0. Patients underwent a mean of 2.8 (SD = 1.2 ultrasound scans, 1.3 (SD = 0.9 abdominal and pelvic CT, 0.8 (SD = 1.0 MRCP and 0.2 (SD = 0.4 ERCP. The mean cost per patient for each readmission, including hospital stay (143.0 €/day, Emergency Service (332.31 € and tests performed was 2,381.70 €/patient. Conclusions: Not performing a cholecystectomy within two weeks after a first episode of mild ABEP or cholecystitis contributes to patient readmission due to recurrent pancreatitis, resulting in avoidable treatment costs.

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

    G.M. Tronconi


    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.

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

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


    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.

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

    Sangeeta Kini


    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.

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

    Weig Thomas


    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.

  10. Unilateral pulmonary edema: a rare initial presentation of cardiogenic shock due to acute myocardial infarction.

    Shin, Jeong Hun; Kim, Seok Hwan; Park, Jinkyu; Lim, Young-Hyo; Park, Hwan-Cheol; Choi, Sung Il; Shin, Jinho; Kim, Kyung-Soo; Kim, Soon-Gil; Hong, Mun K; Lee, Jae Ung


    Cardiogenic unilateral pulmonary edema (UPE) is a rare clinical entity that is often misdiagnosed at first. Most cases of cardiogenic UPE occur in the right upper lobe and are caused by severe mitral regurgitation (MR). We present an unusual case of right-sided UPE in a patient with cardiogenic shock due to acute myocardial infarction (AMI) without severe MR. The patient was successfully treated by percutaneous coronary intervention and medical therapy for heart failure. Follow-up chest Radiography showed complete resolution of the UPE. This case reminds us that AMI can present as UPE even in patients without severe MR or any preexisting pulmonary disease affecting the vasculature or parenchyma of the lung.

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

    Ömer Engin


    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 post operative day.


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


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

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

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


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

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


    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

  15. Acutely exacerbated hypertension and increased inflammatory signs due to radiation treatment for metastatic pheochromocytoma

    Teno, Shinichi; Tanabe, Akiyo; Nomura, Kaoru; Demura, Hiroshi [Tokyo Women`s Medical Coll. (Japan)


    Hypertension and norepinephrine hypersecretion in a 59-year-old woman suffering from malignant pheochromocytoma with multiple metastases were appropriately controlled with {alpha}- and {beta}- blockers, and {alpha}-methyltyrosine ({alpha}-MT), a catecholamine-synthesis inhibitor. Metastasized vertebrae were treated with external radiation to relieve pain, but this treatment had to be interrupted at a total dose of 20 Gy because the patient suffered acutely exacerbated hypertension (200/110 mmHg), tachycardia (160 beats/min) and a low-grade fever. Simultaneously her serum levels of LDH, potassium, urea nitrogen, creatinine, white blood cell count, CRP and norepinephrine were significantly increased, suggesting that this episode was due to radiation-induced tissue destruction and the leakage of catecholamines and possibly interleukin-6, a cytokine mediating inflammation which is reportedly present in pheochromocytoma. The marked hypertension was controlled by continuous iv administration of phentolamine and propranolol. Although radiation therapy effectively relieves pain due to neoplasmic metastasis to the bone, physicians should be aware that life-threatening complications such as the above occur in malignant pheochromocytoma. Sufficient pretreatment with adrenergic blocking agents and/or {alpha}-MT and careful monitoring of the patient`s general condition during radiation therapy, even at a low dose, are highly recommended. (author)

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

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


    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.

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

    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


    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.

  18. Primary sclerosing cholangitis - the Norwegian experience.

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


    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.

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

    Singh, Siddharth; Talwalkar, Jayant A


    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.

  20. Serum levels and roles of high mobility group box-1 protein in patients with acute suppurative cholangitis%血清高迁移率族蛋白B1在急性化脓性胆管炎发生中的作用及意义研究

    郭继中; 张婷; 谢震雄; 蒋莉莎; 陆国民; 夏敏


    Objective To observe the serum levels of high mobility group box-1 protein (HMGB1)in patients with acute cholangitis (AC) and to investigate contributions of HMGB1 in AC.Methods Serum HMGB1 concentrations were determined by an enzyme-linked immunosorbent assay in 30 patients with AC of severe type (ACST) and 42 patients with mild acute cholangitis at the time of admission (within 72 h after the onset).A total of 50 healthy subjects were recruited as the control group.Fluorescent quantitative PCR (FQPCR) was used to detect the HMGB1 mRNA expression and the relationship between serum HMGB1 levels and clinical factors was analyzed.Results The serum HMGB1 levels in healthy control group,mild group and ACST group were (1.82 ± 0.64) μg/L,(10.46 ± 3.75) μg/L,(18.89 ± 6.86) μg/L,respectively.The mean value of serum HMGB1 level in mild group was significantly higher than that in control group,while significantly lower than that in ACST group (P < 0.05).Compared to the control group,the HMGB1 mRNA level in patients of AC increased significantly and the level of ACST group was higher than that of mild group.The serum HMGB1 levels of patients with positive bile or/and blood cultures were higher than that of negative.After emergency endoscopic nasal biliary drainage,the serum HMGB1 levels of patients significantly decreased compared to preoperational (P < 0.05).The HMGB1 levels were significantly positively correlated with white cell counts,C-reactive protein (CRP),total serum bilirubin,direct bilirubin and alkaline phosphatase (ALP).By logistic regression analysis,serum HMGB1 levels had correlation with severity of disease.Conclusion Serum HMGB1 levels significantly increased in patients with AC and the serum concentrations of ACST group were higher than those of mild group.Serum HMGB1 level has a correlation with sepsis.ENBD could lower its serum levels.Serum HMGB1 has predictive value to severity of disease.%目的 检测急性化脓性胆管炎(ASC)患者

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

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


    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.

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

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


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

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

    Yinka K. Davies


    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.

  4. Acute Liver Failure Due to Budd-Chiari Syndrome in the Setting of Cardiac Synovial Sarcoma

    Stine, Jonathan G.; Newton, Kelly; Vinayak, Ajeet G


    Primary malignant tumors of the heart, specifically cardiac sarcomas, are rare and mainly diagnosed at autopsy. Acute Budd-Chiari syndrome is a recognized cause of acute liver failure and has been associated with several rare cardiac tumors: atrial myxoma, caval rhabdomyosarcoma, and primary cardiac adenocarcinoma. We present the first case of a fatal, highly differentiated cardiac synovial sarcoma that presented as acute liver failure from Budd-Chiari syndrome.

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

    Patompong Ungprasert


    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. [Primary-care morbidity and true morbidity due to acute respiratory infections].

    Pérez Rodríguez, A E; González Ochoa, E; Bravo González, J R; Carlos Silva, L; Linton, T


    The present work presents the study of morbidity due to acute respiratory infections (ARI) in areas of the town of Lisa in Ciudad Habana, and Isla Juventud (Cuba), to characterize different aspects of morbidity measured by health care attendance and to measure true morbidity. About 90% of consultations for ARI were first-time consultations, while their ratio to further consultations was 5.3. True morbidity rates (TMR), obtained trough active research, ranged from 110.4 to 163.4 cases per 1000 inhabitants, considerably higher than morbidity rates measured by primary care consultations (MRPCC) in the same time period. The true morbidity index (TMI), as measured by the ratio of the two previous rates, ranged from 5 to 15. A high proportion (47.6%) of cases reported no medical care attendance. These results provide approximate estimates of true morbidity in the study area, and allow the establishment of a new control program, also improving epidemiologic surveillance within primary care activities.

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

    Shyam A. Patel


    Full Text Available 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.

  8. Transcriptome signature in young children with acute otitis media due to non-typeable Haemophilus influenzae.

    Liu, Keyi; Chen, Linlin; Kaur, Ravinder; Pichichero, Michael E


    Non-typeable Haemophilus influenzae (NTHi) causes acute otitis media (AOM) in young children. In our recent paper in Microbes and Infection we described the transcriptome signature elicited from PBMCs at onset of AOM caused by Streptococcus pneumoniae. In the current study we found very different results with NTHi AOM infections; 5.1% of 29 187 genes were differentially regulated by more than 2-fold at the onset of AOM compared with the pre-infection healthy state in the same children. Among the 1487 transcripts, 100 genes associated with the immune defense response were specifically analyzed. About half of the differentially regulated genes associated with antibacterial activity and the cell-mediated immune response were activated and half were suppressed. The important signatures for NTHi in children suggested that the balance of the immune response was toward suppression. Moreover, 90% of the genes associated with a pro-inflammatory cytokine response were down-regulated. The genes associated with the classic complement pathway were down-regulated, although the alternative complement pathway genes were up-regulated. These results provide the first human transcriptome data identifying gene expression in the immune response to be predominantly down-regulated at the onset of AOM due to NTHi.

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

    M.F.R. Silva


    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

  10. [Peripheral nerve disease associated with acute renal failure due to bromate poisoning].

    Deshimaru, M; Miyagawa, T; Sumiyoshi, S; Nomura, Y


    A case of 21 year old male with neuropathy caused by renal insufficiency was present. He had taken bromate (mixed powder of potassium bromate and sodium bromate) for the purpose of suicide and suffered from acute renal insufficiency and hard of hearing. Renal dysfunction improved gradually by peritoneal dialysis and hemodialysis. However, on the 32th day after the onset, burning pain appeared in the bilateral feets. Following this, he began to complain of the disturbances of superficial and deep sensory below the ankle jerks and the weakness of his toes. Considering the clinical features, we supposed that the disturbance of the peripheral nerve was caused by uremia due to taking bromate. N. suralis was biopsied on the 80th day after the onset and examined electron microscopically. Electroscopical findings was as follows. Degeneration of the Schwann cells and irregularity or destruction of the myelin sheaths were observed. The axoplasm of the myelinated nerve fiber were relatively preserved as compared with the changes of the myelin sheaths. In the unmyelinated nerve fibers, cavity formations were observed. The findings of regeneration were not observed. From the electron microscopical findings, we speculate that the changes of the Schwann cells and the myelin sheaths are primary resulting from the disturbance of the metabolism of the Schwann cells. We speculate that anemia and hypoproteinemia caused by bromate disturbed regeneration.

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

    Ponsioen, Cyriel Y


    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%.

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

    Mehmet Husamettin Akkucuk


    Full Text Available 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.

  13. A case of heart failure due to alcoholic cardiomyopathy combined with acute pulmonary embolism.

    Xiao, Feng; Yuan, Wei; Li, Xiaorong; Wang, Gannan; Jiang, Ting; Wang, Weiwei; Zhang, Jinsong; Li, Ping; Qi, Lianwen; Chen, Yan


    It has not been reported that cases of alcoholic cardiomyopathy (ACM) combined with acute pulmonary embolism (PE). We hereby present a case of a 48-year-old male with ACM with significant enlargement of the heart and heart failure is described. Then, the patient was seized with acute PE which was confirmed by specific examination and his symptoms.

  14. Destructive Cholangitis in an Adult Jack Russell Terrier

    Atsushi Kodama


    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. [Acute renal failure due to obstructive ureteral stone associated with norovirus gastroenteritis in an infant with congenital solitary kidney].

    Kato, Taiki; Hamano, Atsushi; Kawamura, Hideki


    We report a 35 month-old boy with acute renal failure caused by an obstructive ureteral stone associated with norovirus gastroenteritis. He visited his family physician because of fever, abdominal pain and vomiting. He was diagnosed as acute gastroenteritis. The symptoms relieved once, but abdominal pain and vomiting recurred two days after the visit and the volume of urine decreased. He was diagnosed as norovirus gastoenteritis and acute renal failure which was unresponsive to fluid replacement. Ultrasound study of the abdomen showed a solitary kidney with mild hydronephrosis. He was then admitted to our hospital. He was finally diagnosed as acute postrenal failure due to obstructive ureteral stone with left solitary kidney by abdominal computer tomography (CT). We performed transurethral catheterization immediately. The creatinine and blood urea nitrogen returned to normal level in 2 days. The CT performed on the 28th day post operation showed disappearance of the stone after uric alkalization. Recently, some cases of postrenal failure due to bilateral obstructive ureteral stones, mainly ammonium acid urate stones, associated with viral gastroenteritis were reported. As clinical features, they are common in boys three years or younger after an episode of rotavirus gastroenteritis with high uric acid concentration. By far, the most common cause of acute renal failure in patients with severe gastroenteritis is prerenal failure resulting from hypovolemia. But postrenal cause due to bilateral obstructive stones should be taken in a consideration.

  16. An Uncommon Presentation of Spontaneous Rectus Sheath Hematoma with Acute Kidney Injury due to Obstructive Uropathy and Prerenal Azotemia

    Eleni Paschou


    Full Text Available Rectus Sheath Hematoma (RSH represents an unusual entity which is characterized by acute abdominal pain and tender palpable abdominal mass usually, among elderly patients receiving anticoagulant therapy. We report the case of an 81-year-old woman admitted to our department due to acute abdominal pain and oligoanuria. The patient had recently been hospitalized due to acute myocardial infarction (AMI and atrial fibrillation (AF and received both anticoagulant and antiplatelet therapies. The radiological assessments revealed an extended Rectus Sheath Hematoma and bilateral hydronephrosis. Treatment of the hematoma required cessation of anticoagulants and antiplatelet agents, immobilization, blood and fresh frozen plasma transfusion, and administration of vasopressors. The patient recovered gradually and was discharged home fifteen (15 days later.

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

    Padillo Francisco J


    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.

  18. Malnutrition and inflammation in acute kidney injury due to earthquake-related crush syndrome

    Zhang Yue


    Full Text Available Abstract Background Malnutrition and inflammation are common and serious complications in patients with acute kidney injury (AKI. However, the profile of these complications in patients with AKI caused by crush syndrome (CS remains unclear. This study describes the clinical characteristics of malnutrition and inflammation in patients with AKI and CS due to the Wenchuan earthquake. Methods One thousand and twelve victims and eighteen healthy adults were recruited to the study. They were divided into five groups: Group A was composed of victims without CS and AKI (904 cases; Group B was composed of patients with CS and AKI who haven't received renal replacement therapy (RRT (57 cases; and Group C was composed of patients with CS and AKI receiving RRT (25 cases; Group D was composed of earthquake victims with AKI but without CS (26 cases; and Group E was composed of 18 healthy adult controls. The C-reactive protein (CRP, prealbumin, transferrin, interleukin-6 and TNF-α were measured and compared between Group E and 18 patients from Group C. Results The results indicate that participants in Group C had the highest level of serum creatinine, blood urea nitrogen and uric acid. Approximately 92% of patients with CS who had RRT were suffering from hypoalbuminemia. The interleukin-6 and CRP levels were significantly higher in patients with CS AKI receiving RRT than in the control group. Patients in Group C received the highest dosages of albumin, plasma or red blood cell transfusions. One patient in Group C died during treatment. Conclusions Malnutrition and inflammation was common in patients with earthquake-related CS and had a negative impact on the prognosis of these subjects. The results of this study indicate that the use of RRT, intensive nutritional supplementation and transfusion alleviated the degree of malnutrition and inflammation in hemodialysis patients with crush syndrome.

  19. Central line-related bacteraemia due to Roseomonas mucosa in a neutropenic patient with acute myeloid leukaemia in Piraeus, Greece.

    Christakis, G B; Perlorentzou, S; Alexaki, P; Megalakaki, A; Zarkadis, I K


    A case of central venous catheter-related bacteraemia due to Roseomonas mucosa in a neutropenic patient with acute myelogenous leukaemia is reported. The patient was successfully treated with amikacin and piperacillin-tazobactam. The clinical isolate was identified as R. mucosa by 16S rRNA gene sequencing.

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

    Mohamad N


    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

  1. Acute hepatitis due to dengue virus in a chronic hepatitis patient

    Souza, L J; Coelho, J.M.C. de O.; Silva,E. J.; Abukater,M.; Almeida,F.C.R.; A. S. Fonte; L.A Souza


    We present a case of acute hepatitis caused by dengue virus, with a significant increase in aspartate transferase and alanine transferase levels in a chronic hepatitis patient attended at the Cane Sugar Planters Hospital of Campos dos Goytacazes, RJ.

  2. Transportable Life Support for Treatment of Acute Lung Failure Due to Smoke Inhalation and Burns


    Batchinsky AI, Cancio LC, Chung KK. Acute respiratory distress syndrome in wartime military burns: application of the Berlin criteria. J Trauma Acute...Respiratory Distress Syndrome in Burns: Application of the Berlin Definition Definition. Critical Care Med. 2013; 41(12):A53. Scaravilli V, Kreyer S...distress syndrome secondary to inhalation of chlorine gas. J Trauma 2006;60 (5)(5):944-957. 18. Batchinsky AI, Weiss WB, Jordan BS, Dick EJ, Jr

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

    Janice M. Lage


    Full Text Available 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 fetal demise.

  4. Acute immune-mediated thrombocytopenia due to oxaliplatin administration: a case report.

    Pietrantonio, Filippo; Di Bartolomeo, Maria; Buzzoni, Roberto; Bajetta, Emilio


    Drug-induced acute thrombocytopenia is an extremely rare side effect that may occur immediately after oxaliplatin infusion. This potentially fatal reaction is immune mediated and can be anticipated by mild hemorrhagic signs during previous administrations. This is the first report of acute thrombocytopenia occurring during adjuvant treatment of colorectal cancer with oxaliplatin. Clinicians should be aware of this adverse event in order to prevent possible serious consequences and stop further oxaliplatin administration.

  5. Damage control using percutaneous transhepatic biliary drainage in acute cholangitis of severe type secondary to intrahepatic choledocholithiasis%经皮经肝胆管引流在肝内梗阻性重症胆管炎损伤控制中的临床价值

    吴武军; 杜立学; 杨俊武; 何凯亮; 孙华; 刘晓刚; 胡海田


    目的 探讨经皮经肝胆管引流(PTCD)在结石所致肝内梗阻性重症胆管炎(ACST)治疗中的临床价值.方法 回顾性分析2009年12月至2013年1月收治的8例PTCD后再行手术的肝内梗阻型ACST患者的临床资料.结果 8例患者均在X线引导下成功进行PTCD.当日引流胆汁100 ~400 ml,所有患者24h内一般情况改善,48 h后胆红素下降约25~ 100 μmol/L.3例血小板数量<20×109/L的患者引流72 h后血小板回升至>50×109/L.所有患者均成功接受手术,其中2例患者行胆道探查+T管引流术,2例患者同时行左半肝切除,3例行胆肠吻合术.8例中7例痊愈,1例肝功能衰竭、胆红素持续> 200 μmol/L的患者长期带管.手术前带管时间7d~3个月,PTCD管均保持通畅,无死亡及出血、胆漏、腹膜炎等严重并发症发生.结论 PTCD用于治疗肝内梗阻型ACST成功率较高,创伤小,耐受性好,引流效果可靠,能充分降低胆道压力,降低手术风险,为手术创造了机会和条件.%Objective To study the safety and efficacy of damage control using percutaneous transhepatic biliary drainage (PTBD) in acute cholangitis of severe type (ACST) secondary to intrahepatic choledocholithiasis.Methods The clinical data of 8 patients who received PTBD after hospital admission followed by conventional surgery for ACST when their general condition improved were retrospectively studied.Results All patients received PTBD successfully and the amount of bile drained was 100-400 ml in the first day.The general condition of these 8 patients became better after 24 h and the total bilirubin decreased for about 25-100 mmol/L after 48 h.Three patients with a platelet count of less than 20 × 109/L showed an improved count to more than 50 × 109/L 72 h after PTBD.All patients were operated at different times after the PTBD:2 received T-tube drainage,3 T-tube drainage combined with left hepatectomy,and 3 choledochojejunostomy.Seven patients recovered

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



    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

  7. Cholangitis with septic shock caused by Raoultella planticola.

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


    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.

  8. Secondary sclerosing cholangitis in a critically ill patient.

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


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

  9. Immune-mediated diseases in primary sclerosing cholangitis

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


    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

  10. Novel developments in IBD-related sclerosing cholangitis

    C.Y. Ponsioen


    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

  11. Distinctive inflammatory bowel disease phenotype in primary sclerosing cholangitis

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


    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

    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


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

    O'Toole, Aoibhlinn


    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.

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

    Mitchell, Robert M S; Byrne, Michael F


    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.

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

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


    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.

  16. New insights into autoimmune cholangitis through animal models.

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


    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.

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

    Mikić Sandra Stefan


    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

  18. Acute kidney injury, hyperbilirubinemia, and ischemic skin necrosis due to massive sulindac overdose.

    Vaughn, John L; Shah, Kejal V; Ghossein, Maroun M; Meyer, William L; Kirkpatrick, Robert B


    Sulindac is a long-acting nonsteroidal anti-inflammatory drug (NSAID) widely used for the management of osteoarthritis, rheumatoid arthritis, ankylosing sponydlitis, and acute gouty arthritis. Reports of sulindac toxicity in the literature are rare. We report the case of a 22-year old male with a history of bipolar disorder who was brought to the emergency department after ingesting approximately 15 g of sulindac in a suicide attempt. He was found to have acute kidney injury and hyperbilirubinemia. Despite aggressive fluid resuscitation, his renal function progressively worsened requiring the initiation of hemodialysis. Ten days following ingestion of sulindac, he began to develop ischemic skin changes with a gangrenous appearance in his hands and feet. He continued to receive supportive treatment, and his acute kidney injury, hyperbillirubinemia, and ischemic skin necrosis eventually resolved. Clinicians should be aware of this long-acting NSAID and its ability to cause prolonged multisystem organ dysfunction.

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

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


    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 (DIC). Renal biopsy showed acute tubular necrosis. Later, the consumed substance was proven to be mercuric chloride. His renal failure improved over time, and his creatinine normalized after 2 months. PMID:27194836

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

    Kim Dong-Min


    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. [Acute kidney failure due to kidney cortex necrosis. 2 clinical cases of surviving patients].

    Fuenzalida, E


    A 22 year old female developed preeclampsia with fetal death in utero. After cesarean section she developed uterine inertia and acute hemorrhagic anemia complicated by sepsis, disseminated intravascular coagulation and total anuria for 4 weeks. She was treated with hemodialysis. The second patient, a 49 year old man developed sepsis and intravascular coagulation after a dog bite. Acute renal failure with a 3 week total anuria followed. He was initially treated with peritoneo dialysis. Renal biopsy showed evidence of renal cortical necrosis in both patients.

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

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


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

    Jaballah Sakhri


    Full Text Available 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 severe acute pancreatitis when using tamoxifen therapy. If tamoxifen is suspected as the probable causative agent, rechallenge with this drug should be prohibited.

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

    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


    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


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


    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.

  6. Acute Respiratory Failure due to Alveolar Hemorrhage after Exposure to Organic Dust

    Sun Mi Choi


    Full Text Available Diffuse alveolar hemorrhage (DAH is associated with severe outcomes. We report a case of acute respiratory failure that required mechanical ventilation and was clinically and pathologically diagnosed as DAH related to exposure to organic dust. A 39-year-old man, who had visited a warehouse to grade beans for purchase, was referred to our hospital for impending respiratory failure. His initial radiographic examinations revealed diffuse bilateral ground-glass opacities in his lungs and bronchoalveolar lavage resulted in progressively bloodier returns, which is characteristic of DAH. He underwent bedside open lung biopsy of his right lower lobe in the intensive care unit. Biopsy results revealed DAH and organization with accumulation of hemosiderin-laden macrophages and a few fibroblastic foci. The patient was treated with empirical antibiotics and high-dose corticosteroids and successfully weaned from mechanical ventilation. DAH might be considered in the differential diagnosis of patients with acute respiratory failure after exposure to organic particles.

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

    Girish V Badarkhe


    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.

  8. Acute kidney injury due to bilateral urolithiasis in pregnancy: a case report

    Vineet Mishra


    Full Text Available Kidney stones are very common and unfortunately do not spare the pregnant population. Anatomical and pathophysiological changes occur in the pregnant females that alter the risk for development of urolithiasis. Acute renal colic during pregnancy is associated with significant potential risks to both mother and fetus. Diagnosis is often challenging because good imaging options without radiation use are limited. Management of diagnosed urolithiasis is unique in the pregnant population and requires multi-disciplinary care. Herein, we report a case of pregnancy which occurred in a state of pre-existing bilateral renal calculi with compromised renal function which subsequently developed into acute kidney injury, and requiring definitive management in the form of PCNL after termination of pregnancy. [Int J Reprod Contracept Obstet Gynecol 2016; 5(12.000: 4486-4490

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

    Muhlis Bal


    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.

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

    Bjurlin, Marc A


    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.

  11. Acute Muscle Trauma due to Overexercise in an Otherwise Healthy Patient with Cystic Fibrosis

    Henning Neubauer


    Full Text Available Cystic fibrosis (CF is one of the most common inherited diseases and is caused by mutations in the CFTR gene. Although the pulmonary and gastrointestinal manifestations of the disease remain in the focus of treatment, recent studies have shown expression of the CFTR gene product in skeletal muscle cells and observed altered intramuscular Ca2+ release dynamics in CFTR-deficient animal models. Physical exercise is beneficial for maintaining fitness and well-being in CF patients and constitutes one aspect of modern multimodal treatment, which has considerably increased life span and reduced morbidity. We report on a case of acute muscle trauma resulting from excessive dumbbell exercise in a young adult with cystic fibrosis and describe clinical, laboratory and imaging characteristics of acute exercise-induced muscle injury.

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

    Bal, Muhlis; Atar, Yavuz; Salturk, Ziya; Ateş, Ahmet Hakan; Yağcı, Serkan; Coşkun Bal, Gökçen


    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.

  13. Acute Abdomen Due to Penicillium marneffei: An Indicator of HIV Infection in Manipur State.

    Ghalige, Hemanth Sureshwara; Sahoo, Biswajeet; Sharma, Sanjeeb; Devi, Khuraijam Ranjana; Singh Th, Sudhir Chandra


    Opportunistic infection in HIV disease often present to clinicians in an atypical manner testing clinical acumen. Here, we report a case of Penicilliosis marneffei (PM) infection presenting to surgical emergency as acute abdomen with undiagnosed HIV status in advanced AIDS, chief complaints being prolonged fever and diffuse abdominal pain. Radiologic imaging showed non-specific mesenteric and retroperitoneal lymphadenopathy. Fine needle aspiration cytology (FNAC) of the lymph node was done and subjected to direct microscopy, gram staining and culture on Sabouraud's dextrose agar (SDA) which showed Penicillium marneffei. He was then treated with intravenous amphotericin. This case is reported for its rarity and unusual presentation to sensitise clinicians and microbiologists to consider PM as an aetiology in acute abdomen in high risk individuals, more so, in patients from north-east India.

  14. Acute kidney injury due to bilateral urolithiasis in pregnancy: a case report


    Kidney stones are very common and unfortunately do not spare the pregnant population. Anatomical and pathophysiological changes occur in the pregnant females that alter the risk for development of urolithiasis. Acute renal colic during pregnancy is associated with significant potential risks to both mother and fetus. Diagnosis is often challenging because good imaging options without radiation use are limited. Management of diagnosed urolithiasis is unique in the pregnant population and requi...

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


    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 b...

  16. Acute urinary retention in women due to urethral calculi: A rare case


    We present a case of a 51-year-old woman with acute urinary retention caused by a urethral calculus. Urethral calculi in women are extremely rare and are usually formed in association with underlying genitourinary pathology. In this case, however, no pathology was detected via thorough urological evaluation. We discuss the pathogenesis, clinical presentation and treatment of urethral calculi. To our knowledge, this is the second reported case of a primary urethral calculus in a female with an...

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

    Julia McNabb-Baltar


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

  18. Xanthogranulomatous cholangitis causing obstructive jaundice: A case report

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


    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. IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis

    Allon Kahn


    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.

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

    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


    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.

  1. A case report of acute pediatric bacterial meningitis due to the rare isolate, Pseudomonas putida

    Grishma V. Kulkarni


    Acute bacterial meningitis (ABM) is the medical emergency which warrants an early diagnosis and an aggressive therapy. Despite the availability of the potent newer antibiotics, the mortality caused by ABM and its complications remain high in India, ranging from 16% to 32%. The aim of this case report is to present the rare isolation ofPseudomonas putida from cerebrospinal lfuid sample. Besides this, the author also emphasizes the importance of correctly identifying the organism and thus the selection of the most accurate antibiotic from the susceptibility proifle to allow for early recovery and to improve the patient outcome and survival.

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

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


    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...... of two animals. During the infection, blood was collected for cultivation, and after the animals were killed from day 5 to 15, they were necropsied and tissues were sampled for histopathology. Animals receiving =500 CFU/kg BW were free of lesions. Pigs inoculated with 5000 and 50 000 CFU/kg BW only...

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

    Zwirewich Charles


    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

  4. Recurrent pyogenic cholangitis : angiographic findings and its significance

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


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

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

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


    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.

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

    Cleva Roberto de


    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.

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

    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


    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.

  8. Medical neglect death due to acute lymphoblastic leukaemia: an autopsy case report.

    Usumoto, Yosuke; Sameshima, Naomi; Tsuji, Akiko; Kudo, Keiko; Nishida, Naoki; Ikeda, Noriaki


    We report the case of 2-year-old girl who died of precursor B-cell acute lymphoblastic leukaemia (ALL), the most common cancer in children. She had no remarkable medical history. She was transferred to a hospital because of respiratory distress and died 4 hours after arrival. Two weeks before death, she had a fever of 39 degrees C, which subsided after the administration of a naturopathic herbal remedy. She developed jaundice 1 week before death, and her condition worsened on the day of death. Laboratory test results on admission showed a markedly elevated white blood cell count. Accordingly, the cause of death was suspected to be acute leukaemia. Forensic autopsy revealed the cause of death to be precursor B-cell ALL. With advancements in medical technology, the 5-year survival rate of children with ALL is nearly 90%. However, in this case, the deceased's parents preferred complementary and alternative medicine (i.e., naturopathy) to evidence-based medicine and had not taken her to a hospital for a medical check-up or immunisation since she was an infant. Thus, if she had received routine medical care, she would have a more than 60% chance of being alive 5 years after diagnosis. Therefore, we conclude that the parents should be accused of medical neglect regardless of their motives.

  9. Role of carglumic acid in the treatment of acute hyperammonemia due to N-acetylglutamate synthase deficiency

    Häberle J


    Full Text Available Johannes HäberleKinderspital Zürich, Abteilung Stoffwechsel, Zürich, SwitzerlandAbstract: N-acetylglutamate synthase (NAGS deficiency is a rare inborn error of metabolism affecting ammonia detoxification in the urea cycle. The product of NAGS is N-acetylglutamate which is the absolutely required allosteric activator of the first urea cycle enzyme carbamoylphosphate synthetase 1. In defects of NAGS, the urea cycle function can be severely affected resulting in fatal hyperammonemia in neonatal patients or at any later stage in life. NAGS deficiency can be treated with a structural analog of N-acetylglutamate, N-carbamyl-L-glutamate, which is available for enteral use as a licensed drug. Since NAGS deficiency is an extremely rare disorder, reports on the use of N-carbamyl-L-glutamate are mainly based on single patients. According to these, the drug is very effective in treating acute hyperammonemia by avoiding the need for detoxification during the acute metabolic decompensation. Also during long-term treatment, N-carbamyl-L-glutamate is effective in maintaining normal plasma ammonia levels and avoiding the need for additional drug therapy or protein-restricted diet. Open questions remain which concern the optimal dosage in acute and long-term use of N-carbamyl-L-glutamate and potential additional disorders in which the drug might also be effective in treating acute hyperammonemia. This review focuses on the role of N-carbamyl-L-glutamate for the treatment of acute hyperammonemia due to primary NAGS deficiency but will briefly discuss the current knowledge on the role of N-carbamyl-L-glutamate for treatment of secondary NAGS deficiencies.Keywords: carglumic acid, carbamylglutamate, N-carbamyl-L-glutamate, N-acetylglutamate synthase deficiency, NAGS deficiency, hyperammonemia

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

    Almdal, T; Schroeder, T; Ranek, L


    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....

  11. [An elderly woman with macrocytic anemia and acute high-output heart failure following acute bleeding due to a gastric ulcer].

    Matsuzaki, Gen; Ashida, Terunao; Akanuma, Masao; Fujii, Jun; Seko, Yoshinori


    Although macrocytic anemia can develop in patients with acute blood loss, such anemia in very old patients is uncommon. In this report, we describe the course of an 89-year-old woman who had a rapid recovery from macrocytic anemia by medication only after acute blood loss due to a gastric ulcer. She had been treated with antihypertensive drugs for the previous 28 years at our outpatient clinic, and was admitted because of acute anemia 6 days after she had experienced tarry stool. Her hemoglobin (Hb) count and mean corpuscular volume (MCV) were 8.4 g/dl, and 103 fl, respectively. A gastroscopic examination indicated that the tarry stool originated from a fresh gastric ulcer. She was treated with an iron preparation, a diuretic and a proton pump inhibitor. The anemia was rapidly improved to Hb 10.5 g/dl and MCV 106 fl in one week, and to Hb 14.5 g/dl and MCV 99 fl in 4 weeks. At admission, she had slight pleural effusion and slight edema associated with an increase in her plasma brain natriuretic peptide (BNP) level (323 pg/ml), and her left ventricular ejection fraction was 76% based on the echocardiography findings, which are signs of the high-output heart failure without remarkable left ventricular diastolic dysfunction (E/e': 11.2). However, these signs improved rapidly, and her BNP level thereafter decreased to 114 pg/ml within four weeks.

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

    Manuel Klein


    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

  13. Mechanism of nano silver stents in the treatment of acute cholangitis of severe type and the antibacterial property of nano silver stents%纳米银支架治疗重症胆管炎的机制及抗菌性研究

    呼增吉; 贾长库; 韩霖; 符誉; 黄小龙


    目的:研究内镜下纳米银支架治疗急性重症胆管炎(ACST)的机制及纳米银支架的抗菌性。方法将20头猪随机分为纳米银支架组(n=8)、普通支架组(n=8)和对照组(n=4)。对照组采用内镜下逆行胆管造影(ERC),胆总管内注入大肠杆菌,乳头口用钛夹夹闭,封闭乳头。支架组在造模成功后行ERC+支架置入,支架末端在乳头口处以钛夹固定。观察术后猪的一般情况,分别于术前、术后1d、3d、5d采集猪血检测其白细胞计数、谷丙转氨酶(ALT)、碱性磷酸酶(ALP)、血清胆红素(TB)、血清肿瘤坏死因子(TNF-α)和白细胞介素-1(IL-1),观察猪死亡后的胆管病理改变。结果三组猪术前的各项观察指标比较差异均无统计学意义(P>0.05);术后对照组的白细胞及分类、ALT、ALP、TB、TNF-α和IL-1水平均逐步上升,且在各时点均明显高于两支架组,差异均有统计学意义(P<0.05);纳米银支架组和普通支架组在术后第五天白细胞计数及分类、血清TNF-α和IL-1比较差异均有统计学意义(P<0.05)。结论细菌感染和胆道梗阻是ACST的主要病因,内镜下置入支架治疗ACST,微创有效,纳米银支架有抗菌性。%Objective To study the mechanism of nano silver stents in the treatment of acute cholangitis of severe type (ACST) and the antibacterial property of nano silver stents under endoscope. Methods Twenty pigs were randomly divided into nano silver stent group (n=8), ordinary stent group (n=8) and control group (n=4). The control group was treated by endoscopic retrograde biliary imaging (ERC), followed by injecting E. coli through bile duct and enclosing papilla with titanium clips. The nano silver stent group and ordinary stent group were treated by ERC com-bined with stent implantation, followed by enclosing papilla with titanium clips. The peripheral blood samples were collected pre-operatively and at 1 day, 3 day, 5 day post

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

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


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

    Wollina, Uwe; Graf, Andreas; Hanisch, Volkmar


    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.

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

    Maria de Lourdes Teixeira Masukawa


    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.

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

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


    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.

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

    Warkaa Al Shamkani


    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.

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

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


    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.

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

    N. V. García-Talavera Espín


    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.

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

    Shinya Hayashi


    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.

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

    Sakaguchi, Kohsaku; KOBASHI, HARUHIKO; Takaki,Akinobu; Kato, Jun; Nawa,Toru; Tatsukawa, Masashi; ISHIKAWA, SHIN; Iwasaki, Yoshiaki; Miyake,Yasuhiro; Shiratori, Yasushi


    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. Reversible acute axonal polyneuropathy associated with Wernicke-Korsakoff syndrome: impaired physiological nerve conduction due to thiamine deficiency?

    Ishibashi, S; Yokota, T; Shiojiri, T; Matunaga, T; Tanaka, H; Nishina, K; Hirota, H; Inaba, A; Yamada, M; Kanda, T; Mizusawa, H


    Acute axonal polyneuropathy and Wernicke-Korsakoff encephalopathy developed simultaneously in three patients. Nerve conduction studies (NCS) detected markedly decreased compound muscle action potentials (CMAPs) and sensory nerve action potentials (SNAPs) with minimal conduction slowing; sympathetic skin responses (SSRs) were also notably decreased. Sural nerve biopsies showed only mild axonal degeneration with scattered myelin ovoid formation. The symptoms of neuropathy lessened within two weeks after an intravenous thiamine infusion. CMAPs, SNAPs, and SSRs also increased considerably. We suggest that this is a new type of peripheral nerve impairment: physiological conduction failure with minimal conduction delay due to thiamine deficiency.

  4. Acute intestinal obstruction due to taenia saginata infestation: a case report

    Soleimani A


    Full Text Available "n Normal 0 false false false EN-US X-NONE AR-SA MicrosoftInternetExplorer4 st1":*{behavior:url(#ieooui } /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0cm 5.4pt 0cm 5.4pt; mso-para-margin:0cm; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:Arial; mso-bidi-theme-font:minor-bidi;} Background: Infection with Taenia saginata or taeniasis is an uncommon parasitic infection in Iran with a prevalence rate of 2-3% and it is more seen in the northern parts of the country. Epigastric pain, nervousness, dizziness, nausea and loss of appetite may be the only presenting symptoms but secondary appendicitis, acute intestinal obstruction and necrosis of the pancreas are its serious and rare complications."n"nCase presentation : A 62-year old woman was admitted to Imam Khomeini Hospital with signs of acute abdomen. She had a past history of infection with hydatid cyst and its subsequent surgery, eight years ago. At the time of admission, she suffered from persistent abdominal pain and loss of appetite for two years. Despite having the epidemiological evidence of working along the banks of rivers contaminated with human sewage and working on farms fertilized with human waste and presence of signs hinting at the disease, parasitic infection had not been considered in its diagnosis."n"nConclusion: Although signs and symptoms of taeniasis are non-specific but a complete history, physical examination and detailed patient notes, especially by considering epidemiological factors, are

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

    André V. Lomar


    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.

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

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


    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.

  7. [Lactose intolerance in hospitalized infants with acute diarrhea due to classic enteropathogenic Escherichia coil (EPEC)].

    Moreira, C R; Fagundes-Neto, U


    Three hundred and eleven hospitalized weaned infants with acute diarrhea, all under 12 months of age, were studied in order to evaluate the development of lactose intolerance and its association with age, nutritional status, birth weight, dehydration and enteropathogenic agents identified in fecal samples. After been rehydrated the infants received whole cow' milk assuring the intake of 100 kcal/kg per day. Lactose intolerance was defined according t the following criteria: 1) persistence of diarrhea associated with weight loss during 48 hours, 2) development of vomiting and/or abdominal distention associated with excretion of carbohydrate in feces and/or acids tools, 3) metabolic acidosis associated with abdominal distention at anytime of refeeding period. Lactose intolerance was detected in 52.1% (162/311) of the patients and it was significantly associated with age under 6 months (P < 0.01), birth weight under 3000 grams (P < 0.01), development of dehydration (P < 0.01) and with enteropathogenic Escherichia coli (EPEC) serotypes infection (P < 0.01).

  8. Acute decrease in the stiffness of resting muscle belly due to static stretching.

    Taniguchi, K; Shinohara, M; Nozaki, S; Katayose, M


    The purpose of the study was to examine the acute effect of static stretching exercise on the resting stiffness of gastrocnemius muscle belly. Ten healthy young adults performed standing wall stretching in dorsiflexion for 1 min at a time and repeated five times. Before and after stretching, the shear modulus was measured in medial and lateral heads of the resting gastrocnemius muscle with ultrasound shear-wave elastography. After the stretching, dorsiflexion range of motion (ROM) of the ankle joint increased (P stretching, shear modulus decreased (P stretching across muscle heads. The decrease in shear modulus returned in 20 min after stretching. In the comparison group of 10 additional subjects, the standing intervention without stretching had no influence on these measures. There was a negative correlation between dorsiflexion ROM and shear modulus in either head before and after stretching. The results demonstrate the transient decreases in the stiffness of the resting gastrocnemius muscle belly and indicate that joint flexibility is greater in individuals with lower resting stiffness of the muscle belly.

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

    Boby Varkey Maramattom


    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.

  10. Ileum perforation due to accidental chicken bone ingestion a rare cause of the acute abdomen

    Doklestić Krstina S.


    Full Text Available Ingestion of foreign bodies is not an uncommon occurrence, but most of them will pass through the gastrointestinal tract without consequences. Complication such as perforation is rare. We present a case of small bowel perforation secondary to the accidental ingestion of a chicken bone. The patient presented with abdominal pain, constipation and vomiting. Clinical examination confirmed generalized abdominal tenderness and rebound tenderness. Abdominal radiography showed multiple dilated loops of small bowel, and abdominal ultrasound (US showed inflammatory changes on small bowel loops, with free fluid and fluid collection around intestinal loops. The patient underwent an emergency laparotomy. Intra operative findings revealed diffuse fibro purulent peritonitis with abscess between central small bowels loops. At about 60 cm from Bauchini valve we found a perforation of ileum at the anti-mesenteric site caused by a sharp chicken wishbone. The patient was treated with resection of the ileum segment (10 cm and primary end-to-end anastomosis. Even that intestinal perforation by a foreign body is rare, physicians should consider possibility of intestinal perforation by a foreign body in the differential diagnosis of acute abdomen in patients presenting with abdominal pain.

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

    Lidegran, Marika; Jorulf, Haakan [Department of Paediatric Radiology, Astrid Lindgren Children' s Hospital, Karolinska Hospital, Karolinska Institute, 17176 Stockholm (Sweden); Palmer, Kenneth; Linden, Viveka [Department of ECMO, Astrid Lindgren Children' s Hospital, Karolinska Hospital, Karolinska Institute, Stockholm (Sweden)


    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.)

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

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


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

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

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


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

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

    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


    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.

  15. A case of acute spinal intradural hematoma due to spinal anesthesia

    Josu M Avecillas-Chasn; Jordi A Matias-Guiu; Gustavo Gomez; Javier Saceda-Gutierrez


    Spinal intradural hematoma is a rare complication of diagnostic lumbar puncture or spinal anesthesia. This complication could be overlooked with devastating neurological consequences due to a delay in diagnosis. Here, we reported a case of a patient with a lumbar spinal intradural hematoma as a result of a difficult spinal anesthesia.

  16. A diagnostic conundrum: acute interstitial nephritis due to armodafinil versus acute cellular rejection in a renal transplant recipient--a case report.

    Baradhi, K M; Gohh, R


    Acute interstitial nephritis is a well-recognized cause of acute kidney injury in native kidneys. While the most common etiology being drug-induced, other causes are infectious, autoimmune, and idiopathic forms of disease. Drug-induced acute interstitial nephritis is not only uncommon in renal transplant recipients but is difficult to diagnose as it mimics acute cellular rejection histologically. We have described herein a renal transplant recipient with acute kidney injury to highlight the difficulties to distinguish acute interstitial nephritis from acute cellular rejection.

  17. Prognosis of patients in coma after acute subdural hematoma due to ruptured intracranial aneurysm.

    Torné, Ramon; Rodríguez-Hernández, Ana; Romero-Chala, Fabián; Arikan, Fuat; Vilalta, Jordi; Sahuquillo, Juan


    Acute subdural hematomas (aSDH) secondary to intracranial aneurysm rupture are rare. Most patients present with coma and their functional prognosis has been classically considered to be very poor. Previous studies mixed good-grade and poor-grade patients and reported variable outcomes. We reviewed our experience by focusing on patients in coma only and hypothesized that aSDH might worsen initial mortality but not long-term functional outcome. Between 2005 and 2013, 440 subarachnoid hemorrhage (SAH) patients were admitted to our center. Nineteen (4.3%) were found to have an associated aSDH and 13 (2.9%) of these presented with coma. Their prospectively collected clinical and outcome data were reviewed and compared with that of 104 SAH patients without aSDH who presented with coma during the same period. Median aSDH thickness was 10mm. Four patients presented with an associated aneurysmal cortical laceration and only one had good recovery. Overall, we observed good long-term outcomes in both SAH patients in coma with aSDH and those without aSDH (38.5% versus 26.4%). Associated aSDH does not appear to indicate a poorer long-term functional prognosis in SAH patients presenting with coma. Anisocoria and brain herniation are observed in patients with aSDH thicknesses that are smaller than those observed in trauma patients. Despite a high initial mortality, early surgery to remove the aSDH results in a good outcome in over 60% of survivors. Aneurysmal cortical laceration appears to be an independent entity which shows a poorer prognosis than other types of aneurysmal aSDH.

  18. Bacteremia due to imipenem-resistant Roseomonas mucosa in a child with acute lymphoblastic leukemia.

    Michon, Anne-Laure; Saumet, Laure; Bourdier, Alice; Haouy, Stéphanie; Sirvent, Nicolas; Marchandin, Hélène


    Roseomonas are described as opportunistic pathogens rarely involved in human infections. Their identification requires molecular methods and their antimicrobial susceptibility pattern varies according to the species. We report the first case of bacteremia due to Roseomonas mucosa in a child with leukemia and reviewed pediatric cases of Roseomonas infection, for which undoubted strain identification was available. Favorable outcome was observed despite resistance to numerous β-lactams that may account for delayed effective treatment, suggesting the low virulence of Roseomonas in children. Here, the strain also displayed unusual resistance to imipenem, highlighting the possible acquisition of additional resistance by this pathogen.

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

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


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

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

    Tharindu Vithanage


    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.

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

    Nao Okuda


    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.

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

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


    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.

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

    James H. Tabibian


    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.

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

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


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

  5. Ulcerative Colitis associated with Sclerosing Cholangitis and Autoimmune Hepatitis

    Hoduț Andrei


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

  6. Cancer Risk and Surveillance in Primary Sclerosing Cholangitis.

    Folseraas, Trine; Boberg, Kirsten Muri


    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.

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

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


    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

  8. Acute silicosis in teflon-coated pan manufacturing due to metal sandblasting.

    Köksal, Nurhan; Kahraman, Hasan


    Sandblasting is one of the occupational causes of silicosis. This report details three cases diagnosed as silicosis caused by sandblasting in Teflon-coated pan manufacturing: Case 1--A 24-year-old man admitted with dyspnea and cough; Case 2--An 18-year-old man admitted with shortness of breath and fever; and Case 3--A 25-year-old man admitted with dyspnea and weight loss. Chest examinations of the first and second cases revealed crackles in both lungs, but the third case was normal, no crackles. Chest x-rays showed bilateral reticulonodular densities and hilar enlargement in all cases. They were clinically and radiologically diagnosed as silicosis due to occupational exposure. All cases had worked in the sandblasting unit at a Teflon-coated pan manufacturing factory for one to three years. Silicosis is a preventable occupational lung disease, but no effective treatment is available for the disease yet. Improving workplace conditions is the most effective way to prevent silicosis.

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

    Di Nardo Matteo


    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.

  10. Positive influence of being overweight/obese on long term survival in patients hospitalised due to acute heart failure.

    Simona Littnerova

    Full Text Available Obesity is clearly associated with increased morbidity and mortality rates. However, in patients with acute heart failure (AHF, an increased BMI could represent a protective marker. Studies evaluating the "obesity paradox" on a large cohort with long-term follow-up are lacking.Using the AHEAD database (a Czech multi-centre database of patients hospitalised due to AHF, 5057 patients were evaluated; patients with a BMI 25 kg/m2. Data were adjusted by a propensity score for 11 parameters.In the balanced groups, the difference in 30-day mortality was not significant. The long-term mortality of patients with normal weight was higher than for those who were overweight/obese (HR, 1.36; 95% CI, 1.26-1.48; p<0.001. In the balanced dataset, the pattern was similar (1.22; 1.09-1.39; p<0.001. A similar result was found in the balanced dataset of a subgroup of patients with de novo AHF (1.30; 1.11-1.52; p = 0.001, but only a trend in a balanced dataset of patients with acute decompensated heart failure.These data suggest significantly lower long-term mortality in overweight/obese patients with AHF. The results suggest that at present there is no evidence for weight reduction in overweight/obese patients with heart failure, and emphasize the importance of prevention of cardiac cachexia.

  11. Effects of ocean acidification on the brown alga Padina pavonica: decalcification due to acute and chronic events.

    Gil-Díaz, Teba; Haroun, Ricardo; Tuya, Fernando; Betancor, Séfora; Viera-Rodríguez, María A


    Since the industrial revolution, anthropogenic CO₂ emissions have caused ocean acidification, which particularly affects calcified organisms. Given the fan-like calcified fronds of the brown alga Padina pavonica, we evaluated the acute (short-term) effects of a sudden pH drop due to a submarine volcanic eruption (October 2011-early March 2012) affecting offshore waters around El Hierro Island (Canary Islands, Spain). We further studied the chronic (long-term) effects of the continuous decrease in pH in the last decades around the Canarian waters. In both the observational and retrospective studies (using herbarium collections of P. pavonica thalli from the overall Canarian Archipelago), the percent of surface calcium carbonate coverage of P. pavonica thalli were contrasted with oceanographic data collected either in situ (volcanic eruption event) or from the ESTOC marine observatory data series (herbarium study). Results showed that this calcified alga is sensitive to acute and chronic environmental pH changes. In both cases, pH changes predicted surface thallus calcification, including a progressive decalcification over the last three decades. This result concurs with previous studies where calcareous organisms decalcify under more acidic conditions. Hence, Padina pavonica can be implemented as a bio-indicator of ocean acidification (at short and long time scales) for monitoring purposes over wide geographic ranges, as this macroalga is affected and thrives (unlike strict calcifiers) under more acidic conditions.

  12. Effects of ocean acidification on the brown alga Padina pavonica: decalcification due to acute and chronic events.

    Teba Gil-Díaz

    Full Text Available Since the industrial revolution, anthropogenic CO₂ emissions have caused ocean acidification, which particularly affects calcified organisms. Given the fan-like calcified fronds of the brown alga Padina pavonica, we evaluated the acute (short-term effects of a sudden pH drop due to a submarine volcanic eruption (October 2011-early March 2012 affecting offshore waters around El Hierro Island (Canary Islands, Spain. We further studied the chronic (long-term effects of the continuous decrease in pH in the last decades around the Canarian waters. In both the observational and retrospective studies (using herbarium collections of P. pavonica thalli from the overall Canarian Archipelago, the percent of surface calcium carbonate coverage of P. pavonica thalli were contrasted with oceanographic data collected either in situ (volcanic eruption event or from the ESTOC marine observatory data series (herbarium study. Results showed that this calcified alga is sensitive to acute and chronic environmental pH changes. In both cases, pH changes predicted surface thallus calcification, including a progressive decalcification over the last three decades. This result concurs with previous studies where calcareous organisms decalcify under more acidic conditions. Hence, Padina pavonica can be implemented as a bio-indicator of ocean acidification (at short and long time scales for monitoring purposes over wide geographic ranges, as this macroalga is affected and thrives (unlike strict calcifiers under more acidic conditions.

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

    Burcu Camcıoğlu


    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.

  14. Acute intestinal obstruction due to Kalimate, a potassium-lowering agent: a case report and literature review.

    Tongyoo, Assanee; Sriussadaporn, Ekkapak; Limpavitayaporn, Palin; Mingmalairak, Chatchai


    Sodium polystyrene sulfonate (Kayexalate) and calcium polystyrene sulfonate (CPS, Kalimate) are commonly used to reduce serum potassium. There were some published evidences of severe gastrointestinal complications from the administration of these agents such as colonic necrosis with or without perforation and acute obstruction. The authors reported a 52-year-old male patient being critically ill from severe soft tissue infection of the right leg and sepsis. Hyperkalemia had occurred due to renal insufficiency and required several doses of Kalimate to reduce the serum potassium level. Subsequently, the patient developed complete intestinal obstruction and an exploratory laparotomy was performed. The intra-operative findings were distended stomach and the small bowel contained a large amount of intraluminal affected Kalimate that was removed via gastrotomy and enterotomy. These findings suggested that the inspissated Kalimate could lead to significant obstruction of the gastrointestinal tract in some groups of patient.

  15. Point-of-Care Ultrasound in Necrotizing Acute Pancreatitis Complicated by Perforated Ileum Due to Nonocclusive Mesenteric Ischemia

    Sonia López-Cuenca


    Full Text Available Necrotizing acute pancreatitis is the most severe form of pancreatitis, and it is a potentially life-threatening condition. Its diagnosis and severity are based on radiological signs. Although computed tomography is the most used imaging tool, ultrasound can be a quick and useful technique in emergency and intensive care scenarios. The use of abdominal ultrasound is generally limited to ruling out cholecystitis. Bowel gas can limit the accuracy of pancreatic imaging. When the pancreas is visualized, ultrasound can reveal pancreatic enlargement, echotextural changes, and peripancreatic fluid. We present a patient with necrotizing pancreatitis who developed peritonitis due to ileal perforation, where the use of ultrasound as a bedside imaging technique was very useful.

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

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


    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.

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

    Haja Mydin H


    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

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

    Hannon, M J


    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

  19. Comparison of computed tomography findings between bacteremic and non-bacteremic acute pyelonephritis due to Escherichia coli

    Seon Jung Oh; Bo-Kyung Je; Seung Hwa Lee; Won Seok Choi; Doran Hong; Sung-Bum Kim


    AIM:To identify computed tomography(CT)findings that are associated with the presence of bacteremia in patients with acute pyelonephritis(APN)due to Escherichia coli(E.coli).METHODS:The clinical data and contrast-enhanced CT findings of 128 patients who were diagnosed with APN due to E.coli and showed renal abnormality on contrast-enhanced CT between January 2003 and November 2013 were retrospectively reviewed.The patients were divided into two groups according to the presence of bacteremia:The bacteremia group and the non-bacteremia group.The abnormality on contrast-enhanced CT were categorized into 5 renal and 4 extrarenal CT findings and compared between the two groups using the χ~2 test and multivariate logistic regression.RESULTS:Among the 128 patients,34 patients(26.6%)were classified into the bacteremia group and 94 patients(73.4%)into the non-bacteremia group.There was no statistically significant difference in gender between the two groups(P = 0.09),but the age of thepatients in the bacteremia group was higher than that of the patients in the non-bacteremia group(P < 0.01).Compared to the non-bacteremia group,1 renal CT finding such as urothelial thickening and 3 extrarenal CT findings such as diffuse peritoneal thickening,cystitis and pulmonary congestion were more frequently observed in the bacteremia group with statistical significance.The logistic regression analysis revealed that CT findings,including urothelial thickening,diffuse peritoneal thickening,cystitis and pulmonary congestion were suggested as the predictive CT findings of bacteremic APN.CONCLUSION:On CT,urothelial thickening,diffuse peritoneal thickening,cystitis,and pulmonary congestion are more frequently observed in patients with bacteremic APN due to E.coli.

  20. Associating pancreaticostomy and biliary-irrigation for staged pancreaticoduodenectomy approach to pancreatic intraductal papillary mucinous neoplasm with recurrent cholangitis and severe jaundice

    Dai, Chao; Lou, Siyuan; Zhou, Fan


    Abstract Patient concerns: A 63-year-old man was hospitalized with history of abdominal pain since more than 1 year, and that of fever with chills since 2 weeks. Diagnoses: Based on the laboratory investigations and radiologic findings, a preliminary diagnosis of pancreatic intraductal papillary mucinous neoplasm (IPMN) with recurrent cholangitis and severe jaundice was made. Interventions: An initial attempt at endoscopic and image-guided drainage proved unsuccessful. Due to cholangitis, liver dysfunction, and hypoalbuminemia, the patient was deemed to be medically unfit for radical surgery. Therefore we considered a novel strategy of associating pancreaticostomy and biliary-irrigation for staged pancreaticoduodenectomy (APBSP). In the first stage, biliary tract double irrigation (endoscopic nasobiliary drainage and T-tube) in combination with pancreaticostomy was performed, which alleviated the symptoms and helped improve the general condition of the patient. In the second stage, radical pancreaticoduodenectomy was performed. Outcomes: Over a follow-up period of 23 months, no recurrence occurred. Lessons: In this report, we present a previously unreported treatment strategy for pancreatic IPMN with recurrent cholangitis and jaundice. The innovative treatment approach may help advance the understanding and management of this condition. PMID:27902614

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

    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.


    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

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

    Trauner Michael


    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.

  3. Management of Primary Sclerosing Cholangitis: Conventions and Controversies

    Natasha Chandok


    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.

  4. Primary sclerosing cholangitis: Updates in diagnosis and therapy

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


    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.

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

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


    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.

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

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


    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

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

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


    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.

  8. Acute Abdomen in the 17th Week of Twin Pregnancy due to Ovarian Torsion – A Late Complication of IVF

    Habek, D.; Bauman, R.; Rukavina Kralj, L.; Hafner, T.; Turudic, T.; Vujisic, S.


    Background: A 32-year-old woman with tubal factor infertility due to bilateral laparoscopic salpingectomy conceived twins with in vitro fertilization (IVF). She developed moderate ovarian hyperstimulation syndrome which was treated with anticoagulant therapy. The subsequent course of the twin pregnancy was normal until the 17th week of gestation when she presented to hospital because of a sharp pain in the right lower abdomen which ceased after admission. Case: Except for a single incident of vomiting, patient had no other subjective symptoms. The clinical examination showed tenderness of the lower right abdominal segment on palpation. The surgeon and the urologist found no signs of an acute surgical or urologic condition, and laboratory findings were within normal reference ranges for pregnant women. Two days after admission the pain reappeared; it was now much stronger and colic-like. The pain was initially located supraumbilically but subsequently spread diffusely across the lower abdomen. Abdominal guarding was present and laboratory findings showed an increase in inflammatory parameters. An enlarged and edematous right ovary was found on transvaginal ultrasound. Conclusion: Exploratory laparotomy via a vertical midline abdominal transection revealed a torqued necrotic right ovary with elements of inflammation and inflammatory adhesions involving the entire pelvis. The patient underwent right-sided ovariectomy and adhesiolysis. Recovered was normal and the patient was delivered of healthy twins in the 37th week of gestation. PMID:28017976

  9. [Case report: Löffler's syndrome due to Ascaris lumbricoides mimicking acute bacterial community--acquired pneumonia].

    Acar, Ali; Oncül, Oral; Cavuşlu, Saban; Okutan, Oğuzhan; Kartaloğlu, Zafer


    In this study we present a patient with Loeffler's syndrome caused by Ascaris lumbricoides who presented with the clinical findings of community-acquired pneumonia (CAP). Our patient, who was twenty-five years old, and who had had symptoms such as coughing, expectorating, dyspnea and fever for approximately ten days, was hospitalized. We auscultated polyphonic rhonchuses at the both hemithoraxes. A chest X-ray revealed bilateral lower zone patch consolidation. Acute bacterial community acquired pneumonia (CAP) was diagnosed due to these findings and empirical antibiotic treatment was begun. Repeated sputum Gram stains were negative, and both sputum and blood cultures were sterile. A sputum smear was negative for acid-fast bacilli. The patient's fever and respiratory complaint did not respond to the empirical antibiotics therapy. During the course of advanced investigations, we measured peripheric eosinophilia, and high levels of total Eo and total IgE, and observed Ascaris lumbricoides eggs during stool examination. The patient was given a diagnosis of Loeffler's syndrome. Thereupon the patient was treated successfully with one dose of albendazol 400 mg. In conclusion, we suggest that Loeffler's syndrome must be considered early in the differential diagnosis for CAP when peripheric eosinophilia is seen in patients if they live in an endemic area for parasitic disease.

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

    陈振甫; 周文秀; 高举先; 孙江桥


    @@ 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).

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

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


    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.

  12. Rapidly fatal community-acquired pneumonia due to Klebsiella pneumoniae complicated with acute myocarditis and accelerated idioventricular rhythm.

    Chuang, Tzu-Yi; Lin, Chou-Jui; Lee, Shih-Wei; Chuang, Chun-Pin; Jong, Yuh-Shiun; Chen, Wen-Jone; Hsueh, Po-Ren


    We describe a previously healthy 52-year-old man with rapidly fatal community-acquired pneumonia caused by Klebsiella pneumoniae. The patient developed acute renal dysfunction, accelerated idioventricular rhythm (acute myocarditis), lactic acidosis and septic shock. He died within 15 hours after admission despite intravenous levofloxacin (750 mg daily) and aggressive medical treatment.

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

    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:; Kim, Myung-Hwan [Dept. of Internal Medicine, Univ. of Ulsan Coll. of Medicine, Asan Medical Center, Seoul (Korea, Republic of)


    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.

  14. [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].

    Nakajima, Hirokazu; Sawaguchi, Hirochiyo; Nakajima, Shigenori


    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.

  15. First case of atypical takotsubo cardiomyopathy in a bilateral lung-transplanted patient due to acute respiratory failure.

    Ghadri, Jelena R; Bataisou, Roxana D; Diekmann, Johanna; Lüscher, Thomas F; Templin, Christian


    Takotsubo cardiomyopathy which is characterised by a transient left ventricular wall motion abnormality was first described in 1990. The disease is still not well known, and as such it is suggested that an emotional trigger is mandatory in this disease. We present the case of a 51-year old female patient seven years after bilateral lung transplantation, who developed acute respiratory distress syndrome and subsequently suffered from atypical takotsubo cardiomyopathy with transient severe reduction of ejection fraction and haemodynamic instability needing acute intensive care treatment. Acute respiratory failure has emerged as an important physical trigger factor in takotsubo cardiomyopathy. Little is known about the association of hypoxia and takotsubo cardiomyopathy which can elicit a life-threatening condition requiring acute intensive care. Therefore, experimental studies are needed to investigate the role of hypoxia in takotsubo cardiomyopathy.

  16. Acute ischemic stroke in a child with cyanotic congenital heart disease due to non-compliance of anticoagulation

    Mohammad, Misbahuddin; James, Anish F.; Qureshi, Raheel S.; Saraf, Sapan; Ahluwalia, Tina; Mukherji, Joy Dev; Kole, Tamorish


    BACKGROUND: Stroke is a common presentation in geriatric patients in emergency department but rarely seen in pediatric patients. In case of acute ischemic stroke in pediatric age group, management is different from that of adult ischemic stroke where thrombolysis is a good op. METHODS: We report a case of a 17-year-old male child presenting in emergency with an episode of acute ischemic stroke causing left hemiparesis with left facial weakness and asymmetry. The patient suffered from cyanotic congenital heart disease for which he had undergone Fontan operation previously. He had a history of missing his daily dose of warfarin for last 3 days prior to the stroke. RESULTS: The patient recovered from acute ischemic stroke without being thrombolyzed. CONCLUSION: In pediatric patients, acute ischemic stroke usually is evolving and may not require thrombolysis. PMID:25215056

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

    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


    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.

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

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


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

  19. An acute hemolytic transfusion reaction due to the "anti-c" rhesus antibody: A case report emphasizing the role of transfusion medicine

    Deepti Sachan


    Full Text Available Rhesus (Rh mediated hemolytic transfusion reactions (HTR are usually immunoglobulin G mediated and delayed onset. Rh antibodies being the cause of acute HTR (AHTR and intravascular hemolysis are still under debate. We report here a case of a 53-year-old male who developed AHTR due to "anti-c" antibodies within 3 h of blood transfusion, precipitating fatal acute liver failure in a patient with hepatitis C related chronic liver disease. This case emphasizes the need of inclusion of antibody screening in routine pretransfusion testing as well as a critical role of transfusion medicine specialists for early diagnosis and minimizing transfusion-related morbidity and mortality.

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

    Aburano, Tamio; Takayama, Teruhiko; Shuke, Noriyuki


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

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

    Yong Ku


    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.

  2. [Acute traumatic spinal cord injury and cardiovascular complications due to neurogenic shock: a possible threat for functional recovery].

    van de Meent, H; Vos, P E; Schreuder, H W; van der Hoeven, J G


    Three men aged 18, 18 and 24 years, developed hypotension and bradycardia following an acute traumatic cervical or thoracic spinal cord injury. After treatment in intensive care and 1-12 months of rehabilitation they still suffered from considerable neurological disorders. Hypotension and bradycardia are common phenomena following acute traumatic cervical and thoracic spinal cord injury. Awareness of cardiovascular complications as a possible threat for functional recovery and adequate insight in the neurological cause of hypotension and bradycardia are important issues in the acute treatment of patients with spinal cord injury. It seems sensible to admit these patients to a medium-care or intensive-care department where they can be monitored and treated by a specialised team in accordance with an adequate protocol.

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

    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)


    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.)

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

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


    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.

  5. Magnetic resonance cholangiopancreatography in primary sclerosing cholangitis in children

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


    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.)

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

    Khaderi, Saira A; Sussman, Norman L


    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.

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

    Tanaka, Atsushi; Takikawa, Hajime


    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.

  8. Gallbladder emptying in patients with primary sclerosing cholangitis

    Karouk Said; Nick Edsborg; Nils Albiin; Annika Bergquist


    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.

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

    Goldaracena Nicolas


    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.

  10. Two cases of cystic artery pseudoaneurysm rupture due to acute cholecystitis with gallstone impaction in the neck.

    Kaida, Shogo; Arahata, Kyouko; Itou, Asako; Takarabe, Sakiko; Kimura, Kayoko; Kishikawa, Hiroshi; Nishida, Jiro; Fujiyama, Yoshiki; Takigawa, Yutaka; Matsui, Junichi


    A cystic artery aneurysm is a rare cause of hemobilia. Herein, we report two cases of acute cholecystitis with a ruptured cystic artery pseudoaneurysm. Two patients (a 69-year-old man and an 83-year-old man) were admitted to our hospital because of acute cholecystitis with gallstone impaction in the neck. Percutaneous transhepatic gallbladder drainage (PTGBD) was performed for both patients. After a few days of PTGBD, gallbladder hemorrhage was observed. Abdominal angiography showed cystic artery aneurysm. A transcatheter arterial embolization was therefore performed, followed by an open cholecystectomy.

  11. Acute inferior ST segment elevation myocardial infarction due to chemotherapy in a young man with testicular cancer

    Veysel Tosun


    Full Text Available Testicular cancer is the most common malignancy in men between 15 and 29 years of age. cisplatin, etoposide and bleomycin combination chemotherapy remains the mainstay of testicular cancer treatment. Acute myocardial infarction is a rare complication of these chemotherapeutics. In this case report, we present a case of 36-year-old male with acute inferior ST-segment elevation myocardial infarction after bleomycin injection. Because the patient had no significant risk factors for coronary artery disease, the infarction was likely caused by the chemotherapy regimen.

  12. 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

    Winnekendonk Guido


    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

  13. [Acute traumatic spinal cord injury and cardiovascular complications due to neurogenic shock: a possible threat for functional recovery

    Meent, H. van de; Vos, P.E.; Schreuder, H.W.B.; Hoeven, J.G. van der


    Three men aged 18, 18 and 24 years, developed hypotension and bradycardia following an acute traumatic cervical or thoracic spinal cord injury. After treatment in intensive care and 1-12 months of rehabilitation they still suffered from considerable neurological disorders. Hypotension and bradycardi

  14. [The use of extracorporeal membrane oxygenation (ECMO) in the treatment of acute respiratory distress syndrome due to pandemic influenza].

    Knapik, Piotr; Przybylski, Roman; Nadziakiewicz, Paweł; Koba, Rafał; Maciejewski, Tomasz; Borowicz, Marcin; Włoczka, Grzegorz; Pawlak, Szymon; Zembala, Marian


    Pandemic influenza particularly often is associated with symptoms of acute respiratory failure, and in case of refractory hypoxemia patients may have indications for the extracorporeal membrane oxygenation (ECMO). The paper presents a case of a pandemic influenza virus infection, where classical indications for veno-venous ECMO occured. Practical aspects of this kind of treatment in the intensive care unit are discussed.

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

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


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

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

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


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

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

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


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

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

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


    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.


    Liliany P. Repizo


    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.

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

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


    @@ 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.

  1. De Garengeot's hernia: an unusual right groin mass due to acute appendicitis in an incarcerated femoral hernia.

    Salkade, Parag R; Chung, Alexander Y F; Law, Y M


    The presence of an acutely inflamed vermiform appendix in a femoral hernia sac is extremely rare; the condition is termed De Garengeot's hernia. Here we describe an elderly patient for whom preoperative computed tomography aided the diagnosis of this rare entity. This Chinese woman had presented with a painful right groin mass. The patient successfully underwent an emergency appendicectomy and primary femoral hernia repair. Once diagnosed, it is imperative to follow key surgical principles to limit the spread of infection.

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

    Abdulbaki, Abdulrahman; Kocherla, Cyrus; Modi, Kalgi


    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.

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

    Abdulrahman Abdulbaki


    Full Text Available 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.

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

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


    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.

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

    Rachel Cherian Koshy


    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.

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

    Vega, Jorge; Díaz, Rienzi


    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.

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

    Camilla Cicognani


    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.

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

    Marcelo Corti


    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.

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

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


    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.

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

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


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

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

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


    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...

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

    Boonstra, K.


    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

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

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


    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...

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

    H.J.F. Hoogstraten (Hubert)


    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

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

    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


    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

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

    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.


    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

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

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


    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

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

    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


    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

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

    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.


    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

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

    Claessen, M.M.H.


    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

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

    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.


    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

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

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


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

  3. Variability of hemodynamic responses to acute digitalization in chronic cardiac failure due to cardiomyopathy and coronary artery disease.

    Cohn, K; Selzer, A; Kersh, E S; Karpman, L S; Goldschlager, N


    Eight patients with chronic congestive heart failure (four with cardiomyopathy and four with ischemic heart disease) underwent hemodynamic studies during acute administration of digoxin, given intravenously in two 0-5 mg doses 2 hours apart. Observations were made before administration of digitalis (control period) and serially therafter for 4 hours after the first dose. Resting mean cardiac index and pulmonary arterial wedge pressure were as follows: 2.0 liters/min per m2 and 23 mm Hg (control period); 2.1 and 24 (at 1 hour); 2.0 and 23 (at 2 hours); 2.7 and 19 (at 3 hours); and 2.3 and 20 (at 4 hours). Exercise responses of mean cardiac index and pulmonary arterial wedge pressure in five patients were: 3.1 liters/min per m2 and 36 mm Hg (control period); 3.2 and 33 (at 1 hour); 3.2 and 28 (at 2 hours); 3.1 and 27 (at.3 hours); and 3.4 and 31 (at 4 hours). The pulmonary arterial wedge pressure remained elevated during exercise in all cases. Arrhythmias were seen in five patients after administration of 0.5 mg of digoxin. Hemodynamic improvement at 4 hours involving both reduced filling pressure and increased blood flow was observed in only two patients at rest and in one additional patient during exercise. Acute deterioration of cardiac function (elevated pulmonary arterial wedge pressure of decreased cardiac index) occurred 30 minutes after administration of digoxin in four patients, concomitantly with increased systemic resistance. In six patients, a peak hemodynamic effect appeared 1 to 1 1/2 hours after administration of digoxin, with partial or total loss of initial benefit by 2 and 4 hours. In previously performed studies observations have seldom exceeded 1 hour; the results of this 4 hour study suggest that, in patients with cardiomyopathy or coronary artery disease and chronic congestive heart failure, acute digitalization does not necessarily lead to consistent, marked or lasting hemodynamic improvement. Thus, current concepts of the use of digitalis is

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

    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.

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

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


    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.

  6. Alcohol consumption in patients with primary sclerosing cholangitis

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


    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

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

    Mahendra K Patel


    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.

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

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


    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.

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

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


    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.

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

    Fahmi Fahmi


    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.

  11. A Cluster of CNS Infections Due to B. cereus in the Setting of Acute Myeloid Leukemia: Neuropathology in 5 Patients.

    Vodopivec, Ivana; Rinehart, Elizabeth M; Griffin, Gabriel K; Johncilla, Melanie E; Pecora, Nicole; Yokoe, Deborah S; Feske, Steven K; Milner, Danny A; Folkerth, Rebecca D


    Bacillus cereus typically causes a self-limited foodborne gastrointestinal (GI) illness. Severe invasive infection occurs rarely, mainly among immunocompromised hosts. We describe a cluster of B. cereus infections among 5 patients with acute myeloid leukemia and chemotherapy-induced neutropenia. The initial case presented with occipital lobe abscess and was found on biopsy to have organisms consistent with Bacillus species. Within 1 week, a second patient died of fulminant brain swelling and hemorrhage. Neuropathologic autopsy and culture revealed B. cereus; hospital infection control and public health officials were notified. Three more patients died within the subsequent 9 months (2 patients had rapid massive hemorrhage and many bacilli reminiscent of Bacillus anthracis infection, and 1 patient had sparse bacilli, petechial hemorrhages, and border zone infarcts). Blood cultures yielded positive results in 3 of 5 cases. A possible route of infection was hematogenous dissemination via GI mucosal breaches (GI symptoms occurred in 3 of 5 cases, and postmortem GI ulceration was found in 3 of 4 cases). Bacilli were seen in 2 of 3 GI ulcerations. Epidemiologic work-up, including a site visit conducted by the Centers for Disease Control and Prevention, did not identify a clear common source but suggested the possibility of bananas as a food source. Bacillus cereus causes a rapidly progressive, hemorrhagic meningoencephalitis with high mortality among patients with neutropenia. Neuropathologists can play a key role in the detection of outbreaks.

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

    Omkar U. Vaidya


    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.

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

    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


    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.

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

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


    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.

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

    Karli J Moncrief


    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.

  16. The effectiveness of a rotavirus vaccine in preventing hospitalizations and deaths presumably due to acute infectious diarrhea in Brazilian children: a quasi-experimental study

    Estêvão Teles


    Full Text Available INTRODUCTION: Rotavirus is the main etiologic agent of acute infectious diarrhea in children worldwide. Considering that a rotavirus vaccine (G1P8, strain RIX4414 was added to the Brazilian vaccination schedule in 2006, we aimed to study its effectiveness and safety regarding intestinal intussusception. METHODS: A quasi-experimental trial was performed in which the primary outcome was the number of hospitalizations that were presumably due to acute infectious diarrhea per 100,000 children at risk (0-4 years old. The secondary outcomes included mortality due to acute infectious diarrhea and the intestinal intussusception rates in children in the same age range. We analyzed three scenarios: Health Division XIII of the State of São Paulo (DRS XIII from 2002 to 2008, the State of São Paulo, and Brazil from 2002 to 2012. RESULTS: The averages of the hospitalization rates for 100,000 children in the pre- and post-vaccination periods were 1,413 and 959, respectively, for DRS XIII (RR=0.67, 312 and 249, respectively, for the State of São Paulo (RR=0.79, and 718 and 576, respectively, for Brazil (RR=0.8. The mortality rate per 100,000 children in the pre- and post-vaccination periods was 2.0 and 1.3, respectively, for DRS XIII (RR=0.66, 5.5 and 2.5, respectively, for the State of São Paulo (RR=0.47, and 15.0 and 8.0, respectively, for Brazil (RR=0.53. The average annual rates of intussusception for 100,000 children in DRS XIII were 28.0 and 22.0 (RR=0.77 in the pre- and post-vaccination periods, respectively. CONCLUSIONS: A monovalent rotavirus vaccine was demonstrated to be effective in preventing the hospitalizations and deaths of children that were presumably due to acute infectious diarrhea, without increasing the risk of intestinal intussusception.

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

    杨景震; 霍英杰; 张玉; 袁静; 赵永强; 邹翠洁


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

  18. Deposition of C3, the terminal complement complex and vitronectin in primary biliary cirrhosis and primary sclerosing cholangitis

    Garred, P; Lyon, H; Christoffersen, P


    Characteristics of primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are bile duct destruction and portal inflammation. Increased levels of circulating complement activation products are also present. This raises the possibility of involvement of complement-dependent cytoto......Characteristics of primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are bile duct destruction and portal inflammation. Increased levels of circulating complement activation products are also present. This raises the possibility of involvement of complement...

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

    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


    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.

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

    Jeong-Sook Lim


    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.

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

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


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

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

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


    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.

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

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


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

  4. [Treating severe acute anemia due to vaginal bleeding in the Jehovah's Witness: a report of 2 cases].

    Gredilla, E; Pérez-Ferrer, A; Canser, E; Alonso, E; Martínez Serrano, B; Gilsanz, F


    For reasons of religious belief, Jehova's Witnesses do not accept blood transfusions or the infusion of blood products. In situations in which severe, life-threatening anemia develops, patient refusal to receive a transfusion can create serious ethical and legal problems. The principle of patient autonomy, which implies the freedom to accept or reject treatment, comes into conflict with the physician's obligation to safeguard the patient's life using all means possible. We report 2 cases of severe anemia in Jehova's Witnesses. One was due to menorrhagia and the other to postpartum bleeding. The physician should be aware of alternatives to infusion of blood products and know how to cope with an unexpected critical event in these patients. The measures we took were effective in our patients. In the case of menorrhagia, hormone treatment is effective when the woman wishes to preserve the ability to conceive and avoid surgery (endometrial ablation and hysterectomy). In postpartum bleeding refractory to conservative treatment, selective embolization of bleeding vessels may make it unnecessary to resort to more aggressive treatment, such as obstetric hysterectomy.

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

    Brian; Story; Michael; Gluck


    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...

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

    Arvind Rajamani


    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.

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

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


    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.

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

    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)


    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.)

  9. Asymptomatic De Novo Inflammatory Bowel Disease Late After Liver Transplantation for Primary Sclerosing Cholangitis: A Case Report.

    Åberg, F; Abdulle, A; Mäkelä, A; Nissinen, M


    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.

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

    Zümrüt Başbakkal


    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

  11. Acute Cholangitis following Intraductal Migration of Surgical Clips 10 Years after Laparoscopic Cholecystectomy

    Natalie E. Cookson


    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.

  12. Acute disseminated encephalomyelitis without optic neuritis followed by optic neuritis in a child due to the sudden cessation of steroid therapy.

    Ryu, Won Yeol; Sohn, Eun Jung; Kwon, Yoon Hyung; Jeung, Woo Jin; Ahn, Hee Bae; Park, Woo Chan; Rho, Sae Heun


    Acute disseminated encephalitis (ADEM) is an autoimmune demyelinating disorder of the central nervous system that usually occurs in children after viral infection or vaccination. It is not uncommon for ADEM to be accompanied by optic neuritis. However, ADEM followed by optic neuritis is a rare. We report the case of a 6-year-old girl who initially presented with ADEM (without optic neuritis) due to a live measles, mumps, and rubella vaccine and was treated with intravenous high-dose corticosteroids. After steroid therapy, she recovered neurologically and was not prescribed any medication, including an oral steroid taper, for use after discharge. Three weeks later, she developed unilateral optic neuritis and was again treated with steroid therapy. This is a rare case of ADEM without optic neuritis in a child, followed by optic neuritis due to the sudden cessation of steroid therapy. Further studies and follow-ups are needed to determine whether ADEM followed by optic neuritis can be considered a specific clinical form of this disorder.

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

    P. Zarogoulidis


    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.

  14. Detection and monitoring of tissue ischemia due to acute arterial obstruction in the limbs of patients with quantitative violations in consciousness, peridural anesthesia

    Ognyan Zlatev


    Full Text Available Traced and interpreted is the dynamic and correlative relations between the values of certain biochemical parameters of the homeostasis connected with cell metabolism in skeletal muscles so a verification of tissue ischemia (TI and ischemic tissue lesion (ITL can be performed. The target group is patients with quantitative violations in consciousness and peridural catheters placed for anesthesia, with the suspicion that an acute arterial obstruction in the limbs (AAOL has occurred, or one has already been verified. Test of the serum levels of CK; CK (MB; ASAT; ALAT and Myoglobin were initially performed and were monitored in dynamic. A valuation of the ASAT/ALAT index in close correlation with the other parameters and clinical course of the disease was also performed. A particular dynamic and correlation, before the operation, after the timely revascularization, during the development of ischemic gangrene and after the amputation on an optimal level, was observed. We found an acute elevation of the enzyme activity of CK; CK (MB and ASAT in the case of AAOL which generated TI. ALAT and Myoglobin remained in the reference ranges. In cases with unsuccessful, late or impossible revascularization and transition to TI and ITL (ischemic rhabdomyolysis we found progressive elevation in the levels of the parameters being monitored, including ALAT and Myoglobin. The registered values, in the patients who were not operated, before death, were 900 times above for CK, and CK (MB did not exceed 5% of CK. Over 100 times the standard value for ASAT and ALAT without and hepatic pathology and acute coronary incident. The ASAT/ALAT index is extremely positive in the group with ITL. The registered values of serum Myoglobin reached up to 70 times above the reference range. Upon, revascularization, amputation on an optimal level or an experienced gangrene, a rapid decrease in the values to the reference range for a period 24-48 hours, was observed, except for ALAT

  15. A step-by-step diagnosis of exclusion in a twin pregnancy with acute respiratory failure due to non-fatal amniotic fluid embolism: a case report

    Papaioannou Vasilios E


    Full Text Available Abstract Introduction Respiratory failure may develop during the later stages of pregnancy and is usually associated with tocolysis or other co-existing conditions such as pneumonia, sepsis, pre-eclampsia or amniotic fluid embolism syndrome. Case presentation We present the case of a 34-year-old healthy woman with a twin pregnancy at 31 weeks and 6 days who experienced acute respiratory failure, a few hours after administration of tocolysis (ritodrine, due to preterm premature rupture of the membranes. Her chest discomfort was significantly ameliorated after the ritodrine infusion was stopped and a Cesarean section was performed 48 hours later under spinal anesthesia; however, 2 hours after surgery she developed severe hypoxemia, hypotension, fever and mild coagulopathy. The patient was intubated and transferred to the intensive care unit where she made a quick and uneventful recovery within 3 days. As there was no evidence for drug- or infection-related thromboembolic or myocardial causes of respiratory failure, we conclude that our patient experienced a rare type of non-fatal amniotic fluid embolism. Conclusion In spite of the lack of solid scientific support for our diagnosis, we conclude that our patient suffered an uncommon type of amniotic fluid embolism syndrome and we believe that this report highlights the need for extreme vigilance and a high index of suspicion for such a diagnosis in any pregnant individual.

  16. Acute tamponade of the left paracorporeal pump house due to membrane defect in a patient with a Berlin Heart EXCOR biventricular assist device.

    Völz, Sebastian; Holmberg, Michael; Redfors, Bengt; Dellgren, Göran


    We report a case of acute tamponade of the left paracorporeal pump house in a patient supported by a Berlin Heart EXCOR biventricular assist device (BiVAD) caused by mechanical defect in the membrane of the arterial chamber. A 36-year old male was admitted for composite graft surgery due to a large aortic regurgitation and consecutive heart failure, decompensated postoperatively and was rescued by with an extracorporeal membrane oxygenation (ECMO) device. He was accepted for heart transplantation, and a BiVAD (Berlin Heart EXCOR) was implanted as bridge-to-transplantation. Two months after discharge, he experienced dyspnoea and received error signals from his BiVAD. Relatives released him from his BiVAD companion driver, connected him to the hand pump and transported him to our institution. On arrival, he was in cardiogenic shock and was stabilized by ECMO. Inspection of the arterial chamber revealed a wear hole and delamination of the diaphragm, which had led to a tamponade by air insufflation into the three-layer membrane. New BiVAD paracorporeal pumps were connected, and the patient was subsequently successfully transplanted. The case depicts the difficulty of diagnosis in this specific patient setting. Despite transparent design of the BiVAD chambers, the development of a chamber tamponade remained undetected until explantation of the system.

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

    Germán Málaga


    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.

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

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


    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.

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

    郭克建; 秦阳


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

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

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


    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.

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

    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)


    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.)

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

    Warren Leigh R


    Full Text Available Abstract Abscess formation of the falciform ligament is incredibly rare and perplexing when encountered for the first time. It is reported to occur in the setting of cholecystitis and cholangitis, but the pathophysiology is poorly understood. In this case report, we present a 73-year-old man with falciform ligament abscess following cholangitis from an obstructive ampullary carcinoma. The patient was referred to the Royal Adelaide Hospital from a country hospital, with progressive jaundice, anorexia and nausea. Prior to transfer, he deteriorated with cholangitis, dehydration and renal failure. On arrival, his abdomen was exquisitely tender along the course of the falciform ligament. His blood tests revealed an elevated white cell count of 14.9 x 103/μl, bilirubin of 291μmol/l and creatinine of 347 μmol/l. His CA 19-9 was markedly elevated at 35,000 kU/l. A non-contrast computed tomography (CT demonstrated gross biliary dilatation and a collection tracking along the path of the falciform ligament to the umbilicus. The patient was commenced on intravenous antibiotics and underwent an urgent endoscopic retrograde cholangiopancreatogram (ERCP with sphincterotomy and biliary stent drainage. Cholangiogram revealed a grossly dilated biliary tree, with abrupt transition at the ampulla, which on biopsy confirmed an obstructing ampullary carcinoma. Following ERCP, his jaundice and abdominal tenderness resolved. He was optimized over 4 weeks for an elective pancreaticoduodenectomy. At operation, we found abscess transformation of the falciform ligament. Copious amounts of pus and necrotic material was drained. Part of the round ligament was resected along the undersurface of the liver. Histology showed that there was prominent histiocytic inflammation with granular acellular eosinophilic components. The patient recovered slowly but uneventfully. A contrast CT scan undertaken 2 weeks post-operatively (approximately 7 weeks after the initial CT revealed

  3. Effects of gentamicin monotherapy for the initial treatment of community-onset complicated non-obstructive acute pyelonephritis due to Enterobacteriaceae in elderly and non-elderly women.

    Wie, S-H; Kim, H W; Chang, U-I


    Aminoglycosides may serve as fluoroquinolone-sparing or cephalosporin-sparing agents if the clinical effectiveness of aminoglycoside monotherapy is demonstrated. The purposes of this study were to investigate the clinical efficacy of gentamicin as an initial empirical antimicrobial agent and to evaluate the effects of gentamicin resistance on clinical outcomes in women with complicated non-obstructive acute pyelonephritis (APN). Medical records of 1066 women with a diagnosis of APN were reviewed retrospectively. We enrolled 275 women with community-onset complicated non-obstructive APN due to Enterobacteriaceae who received gentamicin as their initial antibiotic. Of these 275 patients, 43 had gentamicin-resistant (GM-R) Enterobacteriaceae APN, and 232 had gentamicin-susceptible (GM-S) Enterobacteriaceae APN. The early clinical success rates were 67.4% (29/43) versus 89.7% (208/232) at 72 h in the GM-R versus the GM-S groups (p 0.001). The overall clinical cure rate was 100% (43/43) and 98.7% (229/232) in the GM-R and GM-S groups, respectively. The duration of hospital stay was significantly longer in the elderly, although there were no significant differences in the rates of early clinical success, final clinical cure, mortality, and time to fever clearance between the elderly and non-elderly groups. Resistance of Enterobacteriaceae to gentamicin, haematuria and serum C-reactive protein level≥20 mg/dL were independently associated with early clinical failure. Gentamicin can be an effective initial antibiotic option for empirical therapy in women with community-onset complicated APN who do not need urological interventional procedures. The use of gentamicin may contribute to a reduction of fluoroquinolone or broad-spectrum cephalosporin use in the treatment of complicated APN.

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

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


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

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

    Ignjatović Igor I.


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

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

    P J Lorenzoni


    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.

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

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


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

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

    R Enns


    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.

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

    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.


    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

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

    Wong C


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

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

    Matthias Buechter


    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.

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

    Carmen Hidalgo-Tenorio


    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.

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

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


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

  14. 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

    Nesrin Şen Celasin


    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

  15. 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

    Samer FARHOUD


    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

  16. ST-elevation acute myocardial infarction due to arterial thrombosis in a 29-year-old woman with normal coronary arteries

    Male, Eneida; Morton, Talitha; Farber, Adam


    Acute myocardial infarction (AMI) is rare in young adults. We present a case of a 29-year-old black woman who presented with an acute onset of chest pain while sleeping. Anterior wall ST-elevation AMI was diagnosed based on clinical presentation, electrocardiographic findings, and elevated cardiac biomarkers. Coronary angiography revealed a totally occluded proximal left anterior descending artery. The obstructing lesion, thrombus, was removed. There was no evidence of atherosclerotic disease or dissection. An evaluation for a hypercoagulable state was unrevealing. Echocardiography 1 year later revealed normal left ventricular wall motion and systolic function. PMID:28127135

  17. 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

    André Vitorio Câmara Oliveira


    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.

  18. Serological misdiagnosis of acute liver failure associated with echovirus 25 due to immunological similarities to hepatitis A virus and prozone effect.

    Wollersheim, Susan K; Humphries, Romney M; Cherry, James D; Krogstad, Paul


    We describe a case of acute liver failure caused by echovirus 25 (E25) in a previously healthy 2-year-old boy. Initial serological studies were consistent with hepatitis A virus (HAV), with prozone phenomenon. The similarity of E25 to HAV may obscure accurate diagnosis in some cases of hepatitis.

  19. Discordant diagnoses of acute myocardial infarction due to the different use of assays and cut-off points of cardiac troponins

    Lyck Hansen, Maria; Saaby, Lotte; Nybo, Mads


    Objectives: Several assays for the measurement of cardiac troponin (cTn) are available, but differences in their analytical performances may affect the diagnosis of acute myocardial infarction (MI). Methods: A survey was conducted at all Danish departments of clinical biochemistry at hospitals...

  20. Methotrexate-induced side effects are not due to differences in pharmacokinetics in children with down syndrome and acute lymphoblastic leukemia

    T.D. Buitenkamp (Trudy); R.A.A. Mathôt (Ron); V. de Haas (Valerie); R. Pieters (Rob); C.M. Zwaan (Christian Michel)


    textabstractBackground Children with Down syndrome have an increased risk of developing acute lymphoblastic leukemia and a poor tolerance of methotrexate. This latter problem is assumed to be caused by a higher cellular sensitivity of tissues in children with Down syndrome. However, whether differen

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

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


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

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

    Ali Shorbagi; Yusuf Bayraktar


    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.

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

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


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

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

    K.D. Chakravarty


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

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

    Buness, Cynthia; Miloh, Tamir


    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

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

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


    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.

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

    Masaki Shimizu


    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.

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

    Susan M Gilmour


    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.

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

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


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

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

    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


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

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

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


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

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

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


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

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

    Bousoula Eleni


    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.

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

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


    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.

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

    Altas Y


    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

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

    Rebecca Saich; Roger Chapman


    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

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

    Djokicc Kovac, Jelena [Center for Radiology and Magnetic Resonance Imaging, Clinical Center Serbia, Belgrade (Serbia)], e-mail:; 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)


    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

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

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


    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.

  19. Acute Myocardial Infarction Due to Coronary Artery Embolism in a 22-Year-Old Woman with Mitral Stenosis with Atrial Fibrillation Under Warfarinization: Successful Management with Anticoagulation.

    Sinha, Santosh Kumar; Jha, Mukesh Jitendra; Razi, Mahmadula; Chaturvedi, Vikash; Erappa, Yatish Besthenahalli; Singh, Shravan; Mishra, Vikas; Khanra, Dibbendhu; Singh, Karandeep


    BACKGROUND Coronary artery embolization is an exceedingly rare cause of myocardial infarction, but a few cases in association with prosthetic mechanical valves have been reported. We report a case of embolic myocardial infarction caused by a thrombus in the left atrium with deranged coagulation profile in a patient with critical mitral stenosis under warfarinization. CASE REPORT A 22-year-old woman was taken to the catheterization lab for early coronary intervention in lieu of non-ST elevation myocardial infarction. Electrocardiography showed T↓ in V1 to V4, and atrial fibrillation with controlled ventricular rate. Coronary angiography showed total occlusion of the mid-left anterior descending artery with thrombus. After upstream treatment with tirofiban, the apparent thrombus was dislodged distally while passing a BMW wire. No abnormalities were seen by intravascular ultrasound study. Echocardiography revealed critical mitral stenosis, and left atrial clot with mild left ventricular dysfunction. Coagulation profile revealed sub-therapeutic international normalized ratio levels. The sequential angiographic images, normal intravascular ultrasound study, and presence of atrial fibrillation are confirmatory of coronary embolism as the cause of myocardial infarction. Anticoagulation and treatment of acute coronary syndrome were initiated and she was referred for closed mitral valvulotomy. CONCLUSIONS Coronary artery thromboembolism as a nonatherosclerotic cause of acute coronary syndrome is rare. The treatment consists of aggressive anticoagulation, antiplatelet therapy, and interventional options, including simple wiring when possible. In this context, primary prevention in the form of patient education on optimal anticoagulation with oral vitamin K antagonist and medical advice about imminent thromboembolic risks are of extreme importance.

  20. Acute kidney failure

    Kidney failure; Renal failure; Renal failure - acute; ARF; Kidney injury - acute ... There are many possible causes of kidney damage. They include: ... cholesterol (cholesterol emboli) Decreased blood flow due to very ...

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

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


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

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

    Jeffrey S Crippin


    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.

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

    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


    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.

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

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


    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.

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

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


    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

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

    Young-Mee Lee


    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.

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

    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.

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

    Vrolijk Jan


    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.




    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。

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

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


    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

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

    Minoru Itou


    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.

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

    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.

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

    Margriet R. Timmer


    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.

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

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


    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.

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

    Christopher A. Aoki


    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.

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

    Jochen Mattner


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

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

    Acalovschi, I; Chirileanu, T


    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.

  18. AFM study shows prominent physical changes in elasticity and pericellular layer in human acute leukemic cells due to inadequate cell-cell communication

    Guz, Nataliia V.; Patel, Sapan J.; Dokukin, Maxim E.; Clarkson, Bayard; Sokolov, Igor


    Biomechanical properties of single cells in vitro or ex vivo and their pericellular interfaces have recently attracted a lot of attention as a potential biophysical (and possibly prognostic) marker of various diseases and cell abnormalities. At the same time, the influence of the cell environment on the biomechanical properties of cells is not well studied. Here we use atomic force microscopy to demonstrate that cell-cell communication can have a profound effect on both cell elasticity and its pericellular coat. A human pre-B p190BCR/ABL acute lymphoblastic leukemia cell line (ALL3) was used in this study. Assuming that cell-cell communication is inversely proportional to the distance between cells, we study ALL3 cells in vitro growing at different cell densities. ALL3 cells demonstrate a clear density dependent behavior. These cells grow very well if started at a relatively high cell density (HD, >2 × 105 cells ml-1) and are poised to grow at low cell density (LD, communication must be taken into account when studying biomechanics of cells, in particular, correlating cell phenotype and its biophysical properties.

  19. A patient with minimal change disease and acute focal tubulointerstitial nephritis due to traditional medicine: a case report and small literature review.

    Lee, Keun-Hyeun; Jeong, Han-Sol; Rhee, Harin


    Gongjin-dan (GJD) is a traditional formula that is widely used in Korea and China, and it has been used from 1345 AD in China to improve the circulation between the kidneys and the heart and to prevent all diseases. However, its adverse effects have not yet been reported. We present a patient with minimal change disease and focal tubulointerstitial nephritis associated with GJD. A 72-year-old man visited the clinic for generalized edema 20 days after starting GJD. His serum albumin level was low and nephrotic-range proteinuria was detected. A kidney biopsy showed minimal change disease and acute tubulointerstitial nephritis. After stopping GJD, a spontaneous complete remission was achieved. We discuss the possible pathogenesis of GJD-induced minimal change disease and review the adverse effects of GJD's ingredients and traditional Chinese medicines that can induce proteinuria. We report a new adverse effect of GJD, which might induce increased IL-13 production and an allergic response, leading to minimal change disease and focal tubulointerstitial nephritis.

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

    杨艳; 董莘


    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

  1. Topographic Locomotive Analysis of {sup 99m}Tc-HDP Uptake of Acute Rhabdomyolysis and Musculotendinous Unit Injury due to Excessive Swimming Exercise in Novice: A Case Report

    Bahk, Yong Whee; Jeon, Ho Seung [SungAe General Hospital, Seoul (Korea, Republic of)


    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 {sup 9}:9{sup m}Tc-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 {sup 99m}Tc-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.

  2. 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

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


    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.

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

    Myriam Bastidas


    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.

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

    M.D. Macías-Robles


    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.

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

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


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

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

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


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

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

    Li Chin-Shang


    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.

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

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


    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.

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

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


    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.

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

    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.

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

    Keitel, Verena; Reich, Maria; Häussinger, Dieter


    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.

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

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


    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

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

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


    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.

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

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


    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

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

    Hossein Ahrar


    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.

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

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


    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.

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

    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.

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

    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


    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

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

    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.

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

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


    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.