A case of idiopathic portal hypertension accompanying multiple hepatic nodular regenerative hyperplasia in a patient with systemic sclerosis.

Idiopathic portal hypertension (IPH) is one of the background diseases causing nodular regenerative hyperplasia (NRH). Furthermore, IPH patients accompanied with autoimmune diseases, such as systemic lupus erythematosus (SLE) and systemic sclerosis (SSc), are more likely to form NRH in the liver. A 76-year-old woman had been aware of the Raynaud's phenomenon and scleroderma for the past 30 years. In this case, she presented with abdominal fullness, and her imaging analysis revealed ascites and multiple liver nodules. On Gd-EOB-DTPA enhanced magnetic resonance imaging (EOB-MRI), donut-like uptake was observed in the nodules in the hepatobiliary phase. Liver biopsy of a nodule demonstrated that it was composed of hyperplastic hepatocytes without fibrous septa, and dilated sinusoids were observed beside the nodule. Conversely, background liver showed that peripheral portal veins appeared stenotic with dense fibrosis in the portal area. The final diagnosis was that multiple NRH of the liver developed in SSc patient accompanying IPH. This case suggests that NRH may be unexpectedly diagnosed in patients with autoimmune diseases accompanying IPH.

Management of endoscopic biliary stenting for choledocholithiasis: Evaluation of stent-exchange intervals.

AIM: To evaluate the best management of plastic stents in patients with choledocholithiasis who were unfit for endoscopic stone removal or surgery. METHODS: Between April 2007 and September 2017, 87 patients (median age 83.7 years) with symptomatic choledocholithiasis were treated with insertion of 7-Fr plastic stents because complete endoscopic stone retrieval was difficult, and their general condition was not suitable for surgery. Seventy of these patients agreed to regular stent management and stent exchange was carried out at every 6 mo (Group A, n = 35) or every 12 mo (Group B, n = 35). The remaining 17 patients did not accept regular stent exchange, and stents were replaced when clinical symptoms appeared (Group C). We evaluated the frequency of biliary complication and stent patency rate during follow-up periods. RESULTS: The patency rate of biliary plastic stents was 91.4% at 6 mo (Group A) and 88.6% at 12 mo (Group B), respectively. Acute cholangitis occurred in 2.9% of Group A patients and in 8.6% of Group B patients. In Group C, median stent patency was 16.3 mo, and stent exchange was carried out in 70.6% of cases because of acute cholangitis or obstructive jaundice. Although a high incidence of acute cholangitis occurred, there was no biliary-related mortality. CONCLUSION: Plastic stent exchange at 12-mo intervals is considered a safe procedure for patients with choledocholithiasis. Long-term biliary stenting increases biliary complications, but it can be an acceptable option for select patients who are medically unfit for further invasive procedures.

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

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.

Gastric adenocarcinoma of fundic gland type: Endoscopic and clinicopathological features.

Gastric adenocarcinoma of fundic gland type (GA-FG) with chief cell differentiation was recently proposed as an extremely rare type of gastric adenocarcinoma. Here, we report 4 cases of GA-FG with chief cell differentiation. Endoscopic features included a submucosal tumor shape or a flat shape, whitish discoloration and dilated vessels on the surface. The tumors were located in the upper or middle third of the stomach. All cases were preoperatively diagnosed as GA-FG by biopsy, and endoscopic submucosal dissection was performed. Resected specimens revealed well-differentiated adenocarcinomas resembling chief cells. Tumor cells were diffusely positive for pepsinogen-I, but partially positive for H(+)/K(+)-ATPase in scattered locations around the tumor margin. Despite the presence of minimal invasion of the carcinoma into the submucosal layer, which was observed in two cases, neither lymphatic nor venous invasion was detected in any of the cases. Finally, all cases showed less aggressive clinical behavior with low grade malignancy.

Case of telangiectatic/inflammatory hepatocellular adenoma arising in a patient with primary sclerosing cholangitis.

Hepatocellular adenomas (HCA) have been recently identified as a heterogeneous group, differing based on genotypic as well as morphological characteristics. HCA are most frequently found in women on oral contraception. A type of HCA, inflammatory HCA, is also known as telangiectatic HCA and was previously referred to as telangiectatic focal nodular hyperplasia. We present the first case of HCA arising from the liver with primary sclerosing cholangitis (PSC). This case is a 30-year-old man with a past medical history of PSC, ulcerative colitis and diabetes mellitus. A routine ultrasonography for PSC detected the gradually enlarged intrahepatic mass. Liver biopsy could reveal the diagnosis of telangiectatic/ inflammatory HCA by morphological and immunohistochemical analyses. Partial hepatectomy was performed and the resected liver was pathologically diagnosed as the telangiectatic/inflammatory HCA arising in PSC. This is the first case report of such an association and here we review the current developments and published work of this rare tumor and the association with an activated inflammatory related tumorogenic pathway and PSC.

Endoscopist-directed administration of propofol: a worldwide safety experience.

BACKGROUND & AIMS: Endoscopist-directed propofol sedation (EDP) remains controversial. We sought to update the safety experience of EDP and estimate the cost of using anesthesia specialists for endoscopic sedation. METHODS: We reviewed all published work using EDP. We contacted all endoscopists performing EDP for endoscopy that we were aware of to obtain their safety experience. These complications were available in all patients: endotracheal intubations, permanent neurologic injuries, and death. RESULTS: A total of 646,080 (223,656 published and 422,424 unpublished) EDP cases were identified. Endotracheal intubations, permanent neurologic injuries, and deaths were 11, 0, and 4, respectively. Deaths occurred in 2 patients with pancreatic cancer, a severely handicapped patient with mental retardation, and a patient with severe cardiomyopathy. The overall number of cases requiring mask ventilation was 489 (0.1%) of 569,220 cases with data available. For sites specifying mask ventilation risk by procedure type, 185 (0.1%) of 185,245 patients and 20 (0.01%) of 142,863 patients required mask ventilation during their esophagogastroduodenoscopy or colonoscopy, respectively (P < .001). The estimated cost per life-year saved to substitute anesthesia specialists in these cases, assuming they would have prevented all deaths, was $5.3 million. CONCLUSIONS: EDP thus far has a lower mortality rate than that in published data on endoscopist-delivered benzodiazepines and opioids and a comparable rate to that in published data on general anesthesia by anesthesiologists. In the cases described here, use of anesthesia specialists to deliver propofol would have had high costs relative to any potential benefit.

Clinical evaluation and management of caustic injury in the upper gastrointestinal tract in 95 adult patients in an urban medical center.

BACKGROUND: Caustic ingestion causes a wide spectrum of injuries; appropriate treatment varies according to the severity and extent of the injury. This retrospective study of adult patients with caustic injury presents the endoscopic findings, treatment regimen, and clinical outcome. METHODS: Over a 28-year period, 95 consecutive adult patients admitted to an urban emergency hospital for ingestion of caustic materials were studied. Each patient underwent early endoscopy and the injury was graded for severity. There were 61 men and 34 women with an average age of 37.2 years (range 17 to 81). Ingestion was due to a suicide attempt in 49 patients and accidental in 46 patients. RESULTS: Ten patients showed no mucosal damage. The remaining 85 patients had grade I superficial injury in 47 patients, grade II moderate injury in 25 patients, and deep grade III injury in 13 patients. The ingestion of strong acid or strong alkali often produced deep grade III changes while bleach, detergent, ammonia or other substances usually caused grade I injury. Operative interventions were required for 11 patients with grade III injury and 6 patients with grade II injury. Endoscopic grading was predictive for the onset of complications including late esophageal stricture. There were no complications due to endoscopy; one patient with grade III and multiple comorbidities died from multiple organ failure. CONCLUSION: Upper gastrointestinal endoscopy after caustic ingestion should be performed early to define the extent of injury and guide appropriate therapy. Grade I injuries heal spontaneously. Grade II injuries may be treated conservatively but repeat endoscopy helps define when intervention is needed. Grade III injuries ultimately require surgical intervention.

Thrombomodulin expression by THP-1 but not by vascular endothelial cells is upregulated by pioglitazone.

Thrombomodulin-protein C pathway is a major anti-thrombotic mechanism present in endothelial cells (EC), and an important modulator of inflammation. Peroxisomal proliferator activated receptor-gamma (PPARgamma) expressed in monocytes/macrophages may have a role in cell differentiation. Since the expression of thrombomodulin (TM) by monocytes is upregulated during differentiation into macrophages, we investigated the effect of pioglitazone, a thiazolidinedione (TZD) that is a synthetic ligand of PPARgamma, on the expression of TM by a human monocyte/macrophage cell line; human acute monocytic leukemia (THP-1) cells. Pioglitazone dose-dependently upregulated TM antigen expression by THP-1 cells accompanied by an upregulation of TM cofactor activity for thrombin-dependent protein C activation. Thrombomodulin mRNA expression in THP-1 cells was also upregulated by pioglitazone, whereas tissue factor (TF) mRNA expression was not induced at all. Treatment cells with a natural PPARgamma ligand, 15-deoxy-delta12,14-prostaglandin J(2) (PGJ2), also enhanced TM protein expression. PGF(2alpha) an agent known to inactivate PPARgamma, diminished the stimulatory effect of pioglitazone and PGJ2 on TM protein expression. In contrast, pioglitazone had no effect on TM antigen expression by human umbilical vein ECs. These results suggest that PPARgamma activation in macrophages may counteract potentially prothrombotic and putative inflammatory properties in activated macrophages.