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Background:Even though IgG4-related disease has gained increased attention worldwide, the diagnosis remains challenging. IgG4-related sclerosing cholangitis (IgG4-SC) is not well described in the western hemisphere and may mimic cholangiocarcinoma (CC), especially when occurring without other symptoms such as, e.g. concurrent pancreatitis or retroperitoneal fibrosis. We present a case to add further information to the diagnosis and treatment of this challenging disease.Case report:A 60-year-old male patient presented with painless jaundice. Prior medical history showed diabetes mellitus type I, high blood pressure, and deep vein thrombosis. Diagnostic investigations were strongly suspicious of a Klatskin tumor, although biopsies were inconclusive. The tumor marker Carbohydrate Antigen 19-9 (CA 19-9) was elevated. Prior to the recommended surgery, the patient had two second opinions in two different university hospitals, both arguing for surgery as well. The patient received hilar resection with right hemihepatectomy. During the postoperative course, some major complications occurred, i.e. recurrent pleural effusion, abscess in the liver resection area, sepsis, ileus, and restricted liver metabolism. Treatment with prednisolone did not show any improvement. Approximately 3 months after surgery, the patient died in consequence of acute respiratory failure. Histology showed no signs of CC, but IgG4-SC could be diagnosed.Conclusion:In the case of preoperative signs of CC, differential diagnosis of IgG4-SC needs to be considered, in particular, in cases with missing histologic proof of malignant disease.

Background The aim of the study was to evaluate the current opinion and clinical decision-making process of international pancreatologists, and to systematically identify key study questions regarding the diagnosis and treatment of chronic pancreatitis (CP) for future research. Methods An online survey, including questions regarding the diagnosis and treatment of CP and several controversial clinical case vignettes, was send by e-mail to members of various international pancreatic associations: IHPBA, APA, EPC, ESGE and DPSG. Results A total of 288 pancreatologists, 56% surgeons and 44% gastroenterologists, from at least 47 countries, participated in the survey. About half (48%) of the specialists used a classification tool for the diagnosis of CP, including the Mayo Clinic (28%), Mannheim (25%), or Buchler (25%) tools. Overall, CT was the preferred imaging modality for evaluation of an enlarged pancreatic head (59%), pseudocyst (55%), calcifications (75%), and peripancreatic fat infiltration (68%). MRI was preferred for assessment of main pancreatic duct (MPD) abnormalities (60%). Total pancreatectomy with auto-islet transplantation was the preferred treatment in patients with parenchymal calcifications without MPD abnormalities and in patients with refractory pain despite maximal medical, endoscopic, and surgical treatment. In patients with an enlarged pancreatic head, 58% preferred initial surgery (PPPD) versus 42% initial endoscopy. In patients with a dilated MPD and intraductal stones 56% preferred initial endoscopic ± ESWL treatment and 29% preferred initial surgical treatment. Conclusion Worldwide, clinical decision-making in CP is largely based on local expertise, beliefs and disbeliefs. Further development of evidence-based guidelines based on well designed (randomized) studies is strongly encouraged.

ZusammenfassungKinderendoskopie ist ein typisches Beispiel für die Verzahnung von getrennten Fachgebieten. Sie wird in Deutschland unterschiedlich gehandhabt. Die meisten Endoskopien liegen in der Hand der Gastroenterologen aus der Erwachsenenmedizin, da Pädiater mit endoskopischer Erfahrung nicht flächendeckend vorgehalten werden können. Auf der anderen Seite existieren Zentren mit hoher Expertise in der Hand erfahrener Kindergastroenterologen. Über die Besonderheiten der Endoskopie beim Kind gibt es kaum Literatur. In diesem Artikel haben sich Pädiater und Gastroenterologen aus der Erwachsenenmedizin zusammengefunden, um die Besonderheiten der Endoskopie im Kindesalter zu beschreiben und nachvollziehbar anwendbar zu machen. Ziel ist es, Erfahrungen aus den Besonderheiten der Kindergastroenterologie zusammenzufassen, um sowohl Pädiatern als auch kinderendoskopisch tätigen Gastroenterologen in ihrer Tätigkeit eine Hilfestellung zu geben.

Primary sclerosing cholangitis (PSC) is associated with the development of cholangiocarcinoma (CC) in approximately 9% of patients. Neither cholangiography nor endoscopic tissue sampling can reliably distinguish between CC and benign dominant bile duct stenosis. The aim of the present study was to assess the value of intraductal ultrasonography (IDUS) in distinguishing between benign and malignant dominant stenoses in PSC patients.Forty PSC patients with dominant bile duct stenoses were studied prospectively. Transpapillary IDUS and endoscopic tissue sampling were performed in addition to endoscopic retrograde cholangiography (ERC). Cholangiography and IDUS findings were classified as malignant or benign by the investigators. Final diagnosis of malignant stenosis was based on positive histology and/or cytology, whereas a benign character was assumed in cases of negative tissue sampling and uneventful extended clinical follow-up.Eight PSC patients (20%) had dominant bile duct stenoses caused by CC, whereas 32 out of 40 patients (80%) had benign dominant bile duct stenoses. IDUS was significantly superior to ERC for detection of malignancy in terms of sensitivity (87.5% versus 62.5%, p=0.05), specificity (90.6% versus 53.1%, p0.001), accuracy (90% versus 55%, p0.001), positive predictive value (70% versus 25%, p0.001), and negative predictive value (96.7% versus 85%, p=0.049).Transpapillary IDUS significantly increases the ability to distinguish malignant from benign dominant bile duct stenoses in patients with PSC.

The Stretta procedure is an endoluminal radiofrequency energy delivery system for the treatment of gastroesophageal reflux disease (GERD). The purpose of this study was to present, for the first time, the Stretta treatment experience from European centers.Sixty patients with a history of GERD from six European centers underwent Stretta treatment from May 2001 to June 2003. All patients were at least partly responsive to daily proton-pump inhibitors. Esophageal motility, endoscopy, and ambulatory 24-h pH studies were done in all patients at baseline and 6 and 12 months after treatment. We evaluated medication use, satisfaction, GERD-health-related quality of life, 24-h pH-metry, manometry, and endoscopy.Sixty patients (31 M, 29 F, mean age 47+/-13 years, mean years of GERD 7.4+/-7.2) were treated with the Stretta procedure. At 12 months after treatment, 75% of the patients needed no medication or less medication than before treatment. They were more satisfied with their symptom control and had statistically significantly fewer GERD symptoms (mean lower esophageal sphincter (LES) pressure improved from 14.8+/-9.1 to 16.7+/-10.0 mmHg, p=0.002 and mean total reflux time from 16.7+/-12.8 to 8.8+/-6.6%, p=0.001). Quality of life (mean score decreased from 19.2+/-9.0 to 6.6+/-7.3, p0.0001) and overall physical and mental health also improved significantly.The experience with the Stretta procedure performed at centers in Europe confirms that it is well tolerated and effective in the treatment of GERD. It has a favorable impact on medication requirements, LES pressure, esophageal acid exposure, and GERD symptom scores. The Stretta procedure should be considered for patients who are not satisfied with drug therapy, and who are considering anti-reflux surgery.

Background and aims: Histamine is known as a regulator of gastrointestinal functions, such as gastric acid production, intestinal motility, and mucosal ion secretion. Most of this knowledge has been obtained from animal studies. In contrast, in humans, expression and distribution of histamine receptors (HR) within the human gastrointestinal tract are unclear. Methods: We analysed HR expression in human gastrointestinal tissue specimens by quantitative reverse transcription-polymerase chain reaction and immunostaining. Results: We found that H1R, H2R, and H4R mRNA were expressed throughout the gastrointestinal tract, while H3R mRNA was absent. No significant differences in the distribution of HR were found between different anatomical sites (duodenum, ileum, colon, sigma, and rectum). Immunostaining of neurones and nerve fibres revealed that H3R was absent in the human enteric nervous system; however, H1R and H2R were found on ganglion cells of the myenteric plexus. Epithelial cells also expressed H1R, H2R and, to some extent, H4R. Intestinal fibroblasts exclusively expressed H1R while the muscular layers of human intestine stained positive for both H1R and H2R. Immune cells expressed mRNA and protein for H1R, H2R, and low levels of H4R. Analysis of endoscopic biopsies from patients with food allergy and irritable bowel syndrome revealed significantly elevated H1R and H2R mRNA levels compared with controls. Conclusions: We have demonstrated that H1R, H2R and, to some extent, H4R, are expressed in the human gastrointestinal tract, while H3R is absent, and we found that HR expression was altered in patients with gastrointestinal diseases.

Background In this prospective case series, endoscopic management of pancreatic pseudocysts and abscesses was investigated following an EUS-guided 1-step procedure for initial transmural access. Methods Endoscopic drainage of pancreatic pseudocysts and abscesses was performed in 35 patients (mean age, 51 years; range, 21-81 years) by using interventional echoendoscopes (FG38UX and EG3830UT; Pentax-Hitachi, Lubbecke, Germany). Interventions were performed by using a 1-step device consisting of a needle-wire suitable for cutting current, a 5.5F dilator, and an 8.5F plastic endoprosthesis (Giovannini Needle Wire Oasis, Cook Endoscopy, Winston-Salem, NC). Results Endoscopic stent placement was successful in 33 of 35 patients (94%), whereas repeated needle passages were unsuccessful in 2 cases (pancreatic pseudocyst wall, 7 mm). No procedure-related complications, such as bleeding, perforation, or pneumoperitoneum, were observed. All subsequent complications, such as ineffective drainage (9%), stent occlusion (12%), or cyst infection (12%), were managed endoscopically. Fourteen patients (43%) demonstrated sustained clinical improvement and cyst resolution upon placement of the initial 8.5F transmural drain. Ten patients (30%) did not reveal a 50% reduction in cyst size on day 3, but cyst resolution was achieved by additional endoscopic cyst irrigation. Nine patients (27%) with primary or secondary cyst infection underwent endoscopic balloon dilation and prolonged endoscopic drainage procedures to achieve cyst resolution. The overall resolution rate was 88%, with a recurrence rate of 12%, based on a mean follow-up period of 24 months. Conclusion This 1-step EUS-guided technique with a needle-wire device provides safe transmural access and allows subsequent effective endoscopic management of pancreatic pseudocysts and abscesses.

Tumors of the liver hilum frequently cause obstructive cholestasis. When a curative resection of the tumor is impossible, palliative bile drainage is indicated. A hepatojejunostomy is performed if conservative treatment fails or if irresectability is proven during an initial laparotomy. In patients with peritoneal carcinosis and mesentery retraction, a hepatogastrostomy may represent a helpful alternative. An experimental study was designed to compare the bile drainage effectiveness of a hepatogastrostomy versus a hepatojejunostomy. Two-month-old outbred piglets were used in all experiments. The animals were randomized into three groups (hepatojejunostomy, hepatogastrostomy alone, hepatogastrostomy and proton pump inhibitors). Obstructive cholestasis was induced by common bile duct ligation; hepatojejunostomy and hepatogastrostomy were performed 2 weeks later. The serum bilirubin levels were monitored weekly. All animals were killed 4 weeks after the drainage operation. Following a hepatojejunostomy (n = 5) all animals showed decreasing cholestasis parameters. All animals (n = 3) died within 3–5 days after a hepatogastrostomy due to gastrointestinal bleeding caused by gastric ulcers and ulcers of the liver surface. The administration of pantoprazole prevented these bleeding complications. In animals treated by hepatogastrostomy and proton pump inhibitors (n = 5), bile drainage effectiveness was similar to that following hepatojejunostomy. A hepatogastrostomy represents an alternative treatment option for surgical bile drainage with a similar effectiveness to that of a hepatojejunostomy. To prevent postoperative gastrointestinal bleeding, proton pump inhibitors should be used.

Dysregulated erythropoietin (EPO) plasma levels may play a role in the pathophysiology of chronic liver disease (CLD) because chronic anaemia is frequently observed in patients with liver cirrhosis. We aimed to identify the factors contributing to EPO regulation in patients with CLD.Plasma EPO concentrations were correlated with clinical and laboratory parameters in 111 CLD patients and 220 healthy controls.Anaemia, though generally mild, was common in CLD patients, and thrombocytopenia and previous bleeding episodes were observed in two-thirds of the patients. Plasma EPO levels were significantly elevated in CLD patients (P0.001). EPO increased according to Child's stages of cirrhosis, independently of the aetiology of CLD. EPO correlated with haemoglobin (r= -0.498, P0.001). Additionally, EPO independently correlated with markers of liver dysfunction, e.g. prothrombin time, albumin concentration or cholinesterase activity, and platelet count. EPO was also significantly elevated in patients with a current bleeding tendency and with prior gastrointestinal haemorrhages. EPO levels were increased in patients with impaired pulmonary function, e.g. decreased diffusion capacity, vital capacity or hyperventilation. Interestingly, plasma interleukin-6 (IL-6) concentrations positively correlated with EPO (r=0.277, P = 0.003), suggesting a possible mechanism of EPO upregulation in patients with CLD through IL-6 dependent pathways, e.g. binding of STAT transcription factors in the putative EPO promoter region.EPO is upregulated in patients with chronic liver diseases in response to anaemia, bleeding complications, impaired pulmonary function, thrombocytopenia and liver dysfunction. IL-6 dependent pathways could be involved in mediating elevated EPO levels in CLD patients.

Background and aims: Chromosomal instability is one of the most consistent markers of sporadic colorectal cancer in humans. There is growing evidence that telomere shortening is one of the mechanisms leading to chromosomal instability and cancer initiation. Methods: To test this hypothesis, the telomere length of colorectal epithelial cells and cells from connective tissue was determined at the adenoma-carcinoma transition at the cellular level by quantitative fluorescence in situ hybridisation. Results: Our study showed that the telomere fluorescence intensity of epithelial cells was significantly weaker at the earliest morphologically definable stage of carcinoma—high grade dysplasia with minimal invasive growth—compared with the surrounding adenoma. In contrast, cells from connective tissue had a similar telomere signal intensity at the carcinoma stage compared with the adenoma, and in turn cells from connective tissue had overall significantly stronger telomere fluorescence signals compared with epithelial cells. Conclusions: These results demonstrate that short telomeres of epithelial cells characterise the adenoma-carcinoma transition during human colorectal carcinogenesis, suggesting that carcinomas arise from cells with critical short telomeres within the adenoma. Since the adenoma-carcinoma transition in colorectal cancer is characterised by an increase in chromosomal instability and anaphase bridges, our data support the hypothesis that short telomeres initiate colorectal cancer by induction of chromosomal instability.

Background: Premature stent clogging is the major limitation with plastic stents used in the treatment of malignant biliary structures. A pilot study suggested improved duration of patency of the Tannenbaum stent compared with polyethylene stents. The aim of this prospective, multicenter randomized trial was to compare the Tannenbaum Teflon stent with a conventional polyethylene endoprosthesis (Cotton-Leung biliary stent set) for the treatment of malignant biliary strictures. Methods: Patients over age 18 years with symptoms caused by nonhilar malignant biliary strictures were enrolled. Patients were randomized to receive a 10F Tannenbaum or polyethylene stent after a guidewire was passed beyond the stricture. One hundred six patients (mean age 72 years and 71 years, respectively) were enrolled (54 Tannenbaum, mean age 72 years; 52 polyethylene, mean age 71 years). Results: Tannenbaum and polyethylene stent placement was successful in, respectively, 100% and 96% of procedures without complications. The mean (SD) 90-day stent patency of the Tannenbaum stent was 67% (7%) compared with 73% (7%) for the polyethylene stents. Conclusions: The present study demonstrated no difference in ease of implantation or stent patency between Tannenbaum and polyethylene stents. (Gastrointest Endosc 2002;55:354-8.)

BACKGROUND AND AIMS—The development of cholangiocarcinoma (CCC) is a complication of primary sclerosing cholangitis (PSC). To date, no reliable factors have been described which can define those PSC patients at high risk for the development of CCC and the clinical diagnosis of CCC in PSC patients is difficult. Therefore, molecular markers of cholangiocarcinogenesis, such as K-ras mutations, may improve the early diagnosis of CCC or the timing of liver transplantation. METHODS—K-ras mutations were analysed by enriched polymerase chain reaction/restriction fragment length polymorphism in the bile fluid of 56 PSC patients and 20 patients with other cholestatic diseases. To assess the value of K-ras mutations as a risk factor for cholangiocarcinogenesis, patients were prospectively investigated over a mean period of 31.5 months. RESULTS—In contrast with the control group, 17 (30%) patients with PSC revealed K-ras mutations in bile fluid. The mean Mayo score was not significantly different between PSC patients with (mean score 0.70) and without (mean score 0.13; p=0.2) K-ras mutations. In contrast with the group of PSC patients without K-ras mutations, four CCCs and two dysplasia were diagnosed in the group of patients with K-ras mutations during the follow up investigation (p

BACKGROUNDThe β chemokine monocyte chemotactic protein 3 (MCP-3) has chemoattractant and activating capabilities in monocytes, lymphocytes, eosinophils, and basophils.AIMSTo investigate MCP-3 expression in inflammatory conditions of the human intestinal mucosa.PATIENTSForty five colon biopsy specimens from 18 patients with inflammatory bowel disease (IBD; 16 specimens from inflamed and 10 from non-inflamed areas) and 19 control patients were examined.METHODSImmunohistochemical staining and reverse transcription polymerase chain reaction (RT-PCR) were used for MCP-3 detection in tissue sections. Intestinal epithelial cell lines (HT-29, Caco-2, T-84) were stimulated with interleukin (IL) 1β, IL-6, and tumour necrosis factor α (TNF-α) and examined for MCP-3 protein and mRNA expression using immunocytochemistry and RT-PCR, respectively.RESULTSIn tissue sections, MCP-3 protein was detected predominantly in epithelial cells, both in patients with IBD and in controls. MCP-3 staining was particularly pronounced at sites of active mucosal inflammation. The intensity of MCP-3 staining was positively correlated with the extent of epithelial destruction. In intestinal epithelial cell lines, MCP-3 mRNA was expressed, whereas MCP-3 protein was not consistently detected.CONCLUSIONSOur data show that MCP-3 protein is present in normal and inflamed intestinal tissue. MCP-3 production is substantially enhanced in areas of active inflammation, suggesting an immunoregulatory role of MCP-3 in intestinal inflammation.

Objective To assess the feasibility, morbidity, mortality, and clinical success rate of surgical reconstruction of the biliary system in patients with ischemic-type biliary lesions in their liver graft. Summary background data After liver transplantation, strictures in the biliary tree with secondary sludge formation can occur in the absence of vascular problems. Jaundice, pruritus, and recurrent cholangitis are predominant clinical features leading to considerable morbidity. Interventional measures are the first-line treatment but are frequently only of transient success. Retransplantation is usually considered when interventional treatment is not effective. Methods Surgical exploration and reconstruction was performed in 17 patients with ischemic-type biliary strictures at a median of 2 years after liver transplantation. Findings during surgery, surgical strategies, and postsurgical courses are described. Clinical symptoms and biochemical parameters of cholestasis and liver function were analyzed in the postsurgical course. Results During surgery, all 17 patients were found to have strictures or sclerotic changes involving the hepatic bifurcation and extrahepatic bile duct. Sludge or stones were present in nine patients. In 14 patients with viable bile ducts proximal to the bifurcation, surgical reconstruction was performed by resection of the bifurcation and hepaticojejunostomy. In three patients with more extensive biliary destruction, portoenterostomy with or without peripheral hepatojejunostomy was performed. The prevalence rate of biliary infection at surgery was 93%; the predominant organisms were Candida and enterococci. The perioperative mortality rate was 0%. Clinical symptoms and biochemical parameters became normal or were considerably improved in 14 of 16 patients (88%). Conclusions The hepatic bifurcation seems to be a predominant site for ischemic-type biliary changes after liver transplantation. Surgical treatment by resection of the bifurcation and reconstruction by high hepaticojejunostomy is a safe and highly effective approach leading to cure or persistent major improvement in most patients.

Mucous membrane pemphigoid is a rare, autoimmune blistering disease characterized by erosions with membrane-like lesions. Standard treatment is corticosteroids. We report on a patient with pharyngeal-laryngeal mucositis and esophageal lesions with secondary esophageal stenosis. Tapering of steroids repeatedly induced recurrence of symptoms and lesions. An immunosuppressive therapy including mycophenolat mofetil and tacrolimus was initiated. With this therapy regimen a stable remission and successful steroid tapering could be achieved.

Die Endoskopie ist die Methode der ersten Wahl zur Diagnostik und zum Staging von Varizen im Osophagus und Magen. Besonders blutungsgefahrdet sind Varizen, die groslumig sind, punktformige Aussackungen der Wand und geschlangelte Venolen haben. Diese qualitativen Kriterien sind neben der Anzahl und Lokalisation von Varizen besonders wichtig, um ein Blutungsrisiko besser einschatzen zu konnen. Weitere diagnostische Verfahren, z.B. die Endosonographie, Farbdopplersonographie, die Varizendruckmessung, Computer- und Magnetresonzanztomographie haben sich fur die Varizendiagnostik nicht sicher etabliert. Die radiologische Kontrastdarstellung ist durch die Endoskopie weitgehend verdrangt worden. Neben der Diagnostik befst sich diese Arbeit uberwiegend mit den therapeutischen Strategien sowie der Primar- und Sekundarprophylaxe von Blutungen bei Osophagus- und Magenvarizen.

BACKGROUND: The clinical relevance of gastrointestinal food allergy in adults is largely unknown because the mechanisms are poorly understood and the diagnosis is difficult to confirm. AIMS: To improve the diagnostic means for confirming intestinal food allergy on an objective basis, a new colonoscopic allergen provocation (COLAP) test was developed. PATIENTS: The COLAP test was performed in 70 adult patients with abdominal symptoms suspected to be related to food allergy, and in five healthy volunteers. METHODS: During the COLAP test, the caecal mucosa was challenged endoscopically with three food antigen extracts, a buffer control, and a positive control (histamine). The mucosal weal and flare reaction was registered semiquantitatively 20 minutes after challenge, and tissue biopsy specimens were examined for mast cell and eosinophil activation. RESULTS: No severe systemic anaphylactic reactions were found in response to intestinal challenge. The COLAP test was positive to at least one food antigen in 54 of 70 patients (77%), whereas no reaction in response to antigen was found in healthy volunteers. Antigen induced weal and flare reactions were correlated with intestinal mast cell and eosinophil activation, as well as with patients' history of adverse reactions to food, but not with serum concentrations of total or specific IgE or skin test results. CONCLUSION: The COLAP test may be a useful diagnostic measure in patients with suspected intestinal food allergy and may provide a new tool for the study of underlying mechanisms.

To improve the diagnosis of intestinal allergy, we developed a colonoscopic allergen provocation (COLAP) test.The cecal mucosa was challenged with three food antigen extracts, a buffer control and a positive control (histamine). The mucosal wheal and flare reaction was registered semiquantitatively 20 min after challenge, and selected tissue biopsies were examined for mast cell and eosinophil activation by immunohistochemistry and electron microscopy. The COLAP test was performed on 70 adult patients with abdominal symptoms suspected to be related to food allergy and in 5 healthy volunteers. In parallel, skin prick tests were performed and IgE was measured in serum.97 out of 210 antigen challenges performed in the patient group induced a significant wheal and flare reaction of the mucosa (46%), whereas no reaction in response to antigen was observed in healthy volunteers. Antigen-induced wheal and flare reactions were dependent on patients' histories of adverse reactions to food, but not on serum levels of specific IgE or skin test results. Degranulation of mast cells was observed in almost all tissues in which food antigens caused a wheal and flare reaction. Eosinophil activation was also highly correlated with the extent of the wheal and flare reaction (r(s) = 0.86).The data suggest that the COLAP test may be a useful diagnostic means in patients with suspected intestinal food allergy and a new tool for the study of underlying mechanisms.

Background and Study Aims : In a subgroup of patients, primary sclerosing cholangitis (PSC) is complicated by high-grade focal strictures of the bile ducts, and this can have an unfavorable influence on the natural course of the disease. The aim of this study was to evaluate the efficacy and safety of endoscopic treatment in this selected patient group. Patients and Methods : Twelve symptomatic patients with primary sclerosing cholangitis and major ductal strictures were included in a prospective study of endoscopic treatment. All patients were managed by repeated angioplasty-type balloon dilation and nasobiliary catheter perfusion. A minimum of two treatment sessions was used, and therapy was continued until satisfactory reopening of the strictures was obtained. Routine endoscopic follow-up was performed after three, six, 12, 18, and 24 months, and then at yearly intervals. The efficacy of therapy was assessed by evaluating clinical symptoms, laboratory data, and cholangiograms. Results : The long-term follow-up averaged 23 months (range : 12-50 months). Two to nine (mean : three) treatment sessions were required to obtain satisfactory reopening of major biliary strictures. Eight patients showed considerable and sustained improvement. The mean serum bilirubin, alkaline phosphatase, γ-glutamyl-transpeptidase, and alanine aminotransferase levels fell significantly by 73% (P=0.0164), 46% (P=0.0022), 55% (P=0.0022), and 58% (P=0.0022), respectively. The average radiographic stricture score before treatment was 3.2 ± 0.7 (mean ± SD), and after treatment decreased to 1.9 ± 0.8 (P= 0.0033). Three patients required liver transplantation seven, 12, and 40 months after the initiation of endoscopic treatment, due to a deterioration in hepatic function or an inability to exclude complex biliary malignancy. No major procedure-related side effects were observed. Conclusions : Our results suggest that the endoscopic treatment of PSC patients with dominant bile duct strictures is effective, safe, and well-tolerated. However, it is important not to overlook the potential development of cholangiocarcinoma.

Background/Aims Timely diagnosis of biliary atresia (BA) requires key investigations that are less invasive but as accurate as possible. Non-invasive imaging preselects patients before explorative laparotomy is performed. The purpose of this prospective study was to evaluate the accuracy of endoscopic retrograde cholangiopancreaticography (ERCP) in these patients and to discuss its relevance to future diagnostic guidelines in neonatal jaundice. Methods Over a 7-year period, ERCP was routinely performed in cholestatic patients less than 6months of age suspected for an extrahepatic origin of cholestasis, most likely BA. Endoscopic diagnosis was correlated with intraoperative findings. Results In 140 consecutive patients (mean age: 60days; weight: 4kg), ERCP excluded BA in 34 (25%) but failed in 18 newborns (13%) for technical reasons. The average procedure time was 23min, and no severe complications occurred. Explorative laparotomy was performed in 106 patients and revealed BA in 80 cases. In this series, the sensitivity of ERCP for diagnosing biliary atresia was 92% and specificity was 73%. Conclusions In preselected patients, ERCP is not an alternative to non-invasive imaging, but it avoids unnecessary surgical procedures in almost 25% of the cases. Hence, ERCP is recommended prior to explorative laparotomy in all patients suspected for BA.

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with varying severity and progression. This study describes the natural history of PSC patients and evaluates the prognostic significance of clinical, biochemical, and cholangiographic findings constructing a novel prognostic model.A population of 273 German PSC patients was studied with a median follow-up time of 76 months (range 1-280 months). Survival curves were analyzed by the Kaplan-Meier method, and prognostic significance of clinical, biochemical, and cholangiographic features recorded at the time of diagnosis was evaluated by multivariate analysis using Cox proportional-hazards regression models.The estimated median survival from the time of diagnosis to death or time of liver transplantation was 9.6 yr. One hundred eight (39.6%) patients underwent liver transplantation. Hepatobiliary malignancies were found in 39 (14.3%) patients of the entire PSC population. Age, low albumin, persistent bilirubin elevation longer than 3 months, hepatomegaly, splenomegaly, dominant bile duct stenosis, and intra- and extrahepatic ductal changes at the time of diagnosis were found to be independent risk factors correlating with poor prognosis and were used to construct a new prognostic model.A persistent bilirubin elevation for longer than 3 months from the time of diagnosis could be identified as a novel marker correlating with a poor outcome. A new prognostic model was developed to predict progression of PSC, which may be useful in timing of liver transplantation.

Primary sclerosing cholangitis (PSC) confers a high risk of development of hepatobiliary carcinoma (HBC). The aim of the study was to identify indicators and risk factors for developing HBC in PSC patients.Thirty-nine PSC patients with HBC at time of HBC diagnosis were compared with 101 PSC patients without HBC at time of acceptance for liver transplantation.Eighteen of these patients (46.2%) developed HBC within one year after diagnosis of PSC. In PSC patients with HBC male gender, nicotine abuse, long duration of inflammatory bowl disease (IBD), clinical symptoms, elevation of CA 19-9, as well as dominant bile duct stenosis were significantly more frequent (p0.05) compared with the PSC control group. A cross-validated sensitivity and specificity of 85% and 97%, respectively, for the detection of HBC was obtained using the following parameters: weight loss, elevation of CA 19-9or= 200 kU/l, and dominant bile duct stenosis.HBC is not necessarily a late complication of end-stage PSC. A long history of IBD, male gender, and nicotine abuse are risk factors for the development of HBC. In particular, CA 19-9, body-weight and dominant bile duct stenosis are valuable indicators in detecting HBC in PSC patients.

Die Leberhilusnekrose mit nekrotisierender Cholangitis ist ein Befund, der gelegentlich als Komplikation einer Lebertransplantation zu finden und zumeist Folge eines arteriellen Verschlusses ist. Die Prognose ist ungunstig und das Vorgehen chirurgisch. Wir berichten uber einen 17jahrigen jungen Mann, der aus voller Gesundheit heftige abdominelle Schmerzen entwickelte. Die Initialtherapie erfolgte unter dem Bild einer biliaren Pankreatitis, im Verlauf zeigte sich aber ein Abszess in der Leberpforte, eine eitrige Cholangitis und eine Perforation des Ductus hepatocholedochus. Da sich die Symptomatik unter konservativer Therapie besserte, wurde auf ein chirurgisches Vorgehen primar verzichtet und eine endoskopische Sanierung geplant. In der ERCP zeigte sich eine schwere destruierende zentrale Gallengangsnekrose und freier Kontrastmittelaustritt im Bereich der Leberpforte und Entleerung von Sludge. Die zentralen Gallengange waren nicht sondierbar. Beim Versuch der Spulung bildetet sich ein duodenales Paravasat aus. Im weiteren wurde dieses Paravasat endosonografisch transduodenal per Stenting drainiert. Es erfolgte dann ein perkutaner Zugang zum Gallenwegssystem von rechts, ein Vorkommen nach intestinal durch die Abszessregion war primar nicht moglich. Deswegen fuhrten wir ein cholangioskopisches Rendezvous-Manover von intestinal und perkutan durch, konnten dann unter Sicht einen endoskopisch eingefuhrten Fuhrungsdraht fassen und perkutan ausleiten. Damit wurde die Platzierung einer Yamakawa-Drainage moglich. Uber diesen Zugang wurde in 2–3 Monatsabstanden unter cholangioskopischer Sicht die Region von Detritus gereinigt. Inzwischen, nach 12 Monaten, hat sich die Kontinuitat des Gallengangs weitestgehend wiederhergestellt und das Gallenwegssystem ist von Detritus bereinigt. Morphologisch bietet sich das Bild einer sekundar sklerosierenden Cholangitis. Der Patient ist unter diesen Masnahmen beschwerdefrei. Zusammenfassend lag hier eine zentrale Lebernekrose unklarer Genese vor, die bei initialem Ansprechen auf konservative Therapie einer endoskopischen Therapie erfolgreich zugefuhrt werden konnte.

BACKGROUND AND STUDY AIMS In adults, general anesthesia is usually only provided during endoscopic retrograde cholangiopancreatography (ERCP) when prior attempts using conscious sedation have failed. It was hypothesized that in our hospital, other factors might be associated with general anesthesia for ERCP. The aim of this study was therefore to assess the indications for ERCP under general anesthesia, and to evaluate the underlying diseases, type, and efficacy of ERCP under general anesthesia in comparison with conscious sedation. PATIENTS AND METHODS We retrospectively analyzed 1,056 ERCPs that had been carried out with the patients under general anesthesia or conscious sedation. The indications for general anesthesia were recorded, and the underlying diseases, the type and success of the interventions, and the causes of premature ERCP termination in both groups were assessed. RESULTS Eighteen percent of the ERCPs were performed under general anesthesia and 82% under conscious sedation. The indications for general anesthesia were related to the type of procedure planned (46%), premature termination of ERCP under conscious sedation (28%), and other reasons. Patients with primary sclerosing cholangitis and liver transplant recipients received general anesthesia more frequently (general anesthesia vs. conscious sedation, 36% vs. 16%, P < 0.0001 and 22% vs. 13%, P = 0.003). Conscious sedation was provided more frequently in patients with neoplasms and cholelithiasis (21% vs. 12%, P = 0.004 and 13% vs. 3%, P < 0.001). Painful dilations were performed more frequently with the patients under general anesthesia (60% vs. 19%, P < 0.001), whereas major papillotomies were preferably performed with conscious sedation (34% vs. 21%, P = 0.006). More interventions per ERCP were performed with the patient under general anesthesia compared to conscious sedation (P < 0.001), during the same time (51 +/- 28 min vs. 52 +/- 26 min, P = 0.39). With conscious sedation, the ERCP failure rate was double that with general anesthesia (7% vs. 14%, P = 0.012), mainly due to inadequate conscious sedation (61%). CONCLUSIONS The frequent use of general anesthesia for ERCP at our institution is related to the underlying diseases, which are frequently treated with complex and painful ERCP procedures. The efficacy of ERCP with general anesthesia supports a continued preference for general anesthesia rather than conscious sedation when complex and painful interventional ERCP procedures are planned.