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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.

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PURPOSE OF REVIEW: The recent developments and clinical applications of natural orifice translumenal endoscopic surgery (NOTES)-procedures and technologies are going to be presented. RECENT FINDINGS: In experimental as well as clinical settings, NOTES-procedures are predominantly performed in hybrid technique. Current experimental studies focus on the implementation of new surgical approaches as well as on the training of procedures. One emphasis in the clinical application is transrectal and transanal interventions. Transanal total mesorectal excision is equivalent to laparoscopic procedures but with the benefit of an even less invasive access. Transvaginal cholecystectomy can achieve results that are comparable to surgeries that are performed with laparoscopic techniques alone. An analysis of the German NOTES-Register concerning appendectomies as well as the national performance of NOTES-interventions in Switzerland is presented. Apart from intraabdominal approaches, several centers proclaim transoral thyroidectomies and transoral mediastinoscopies. SUMMARY: NOTES-procedures are performed in animal experiments as well as in clinical setting although with less frequency. At this time, hybrid techniques using rigid instruments are mainly applied.

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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 6 months 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: 60 days; weight: 4 kg), ERCP excluded BA in 34 (25%) but failed in 18 newborns (13%) for technical reasons. The average procedure time was 23 min, 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.

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OBJECTIVE: 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. MATERIAL AND METHODS: 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. RESULTS: 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, p<0.0001) and overall physical and mental health also improved significantly. CONCLUSIONS: 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.

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OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

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OBJECTIVE: 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. MATERIAL AND METHODS: 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. RESULTS: 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 (p<0.05) 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 19-9 >or= 200 kU/l, and dominant bile duct stenosis. CONCLUSIONS: 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.

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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, Lübbecke, 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.

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PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

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