Primary Bile Duct Diffuse Large B-cell Lymphoma Diagnosed by Repeated Endoscopic Ultrasound-guided Fine-needle Aspiration and Endoscopic Retrograde Cholangiopancreatography.

A 54-year-old man was admitted with obstructive jaundice. Computed tomography showed common bile duct stricture and a tumor around the celiac artery. Repeated endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) and endoscopic retrograde cholangiopancreatography (ERCP) as well as a laparotomic biopsy around the celiac artery were diagnostically unsuccessful. Since the bile duct stricture progressed, EUS-FNA and ERCP were performed a third time, finally leading to the diagnosis of diffuse large B-cell lymphoma. The treatment plan and prognosis of obstructive jaundice differ greatly depending on the disease. It is important to conduct careful follow-up and repeated histological examinations with appropriate modifications until a diagnosis is made.

Late-onset Wilson Disease in a Patient Followed-up for Nonalcoholic Fatty Liver Disease.

A 73-year-old woman was referred to our hospital for persistent liver dysfunction. When the patient was 45 years old, her youngest sister had been diagnosed with Wilson disease (WD). The patient therefore underwent several family screening tests, all of which were unremarkable. She had an annual medical checkup and was diagnosed with liver dysfunction and fatty liver at 68 years old. A liver biopsy and genetic testing were performed, and she was diagnosed with WD; chelation therapy was then initiated. In patients with hepatic disorders and a family history of WD, multiple medical examinations should be conducted, as the development of WD is possible regardless of age.

Adult-onset Still's Disease Diagnosed during Puerperium with Liver Dysfunction in Pregnancy and Liver Failure of the Infant.

Most cases of liver dysfunction in pregnancy are pregnancy-related, but the onset of systemic autoimmune diseases is also differentiated. A 24-year-old woman presented with liver dysfunction at 28 weeks' gestation with suspected autoimmune hepatitis and started taking ursodeoxycholic acid. She gave birth prematurely at 35 weeks' gestation, and the infant presented with pancytopenia and liver failure but survived because of liver transplantation. Since the patient had major symptoms during the puerperium, she was diagnosed with adult-onset Still's disease. When encountering a patient with liver dysfunction during pregnancy, we should also consider the onset of autoimmune diseases.

Analysis of Disaster-Related Deaths in the Great East Japan Earthquake: A Retrospective Observational Study Using Data from Ishinomaki City, Miyagi, Japan.

Disaster-related deaths are of two types: direct and indirect. Preventable disaster-related deaths reported in the Great East Japan Earthquake (GEJE) included a large number of indirect deaths. This study aimed to investigate the data on disaster-related deaths in the GEJE in Ishinomaki City, Miyagi Prefecture, and to clarify the scope of disaster-related deaths to help future disaster preparedness. A retrospective observational study was conducted using public data on disaster-related deaths from March 2011 to January 2021, available at Ishinomaki City Hall. Descriptive and Cox regression analyses were conducted. The most common direct cause of disaster-related deaths was respiratory diseases, which were more common among those aged less than three months and over 60 years. Suicide was common among those aged under 60 years, and the proportion increased more than six months after the disaster. The risk of death was significantly higher among those who needed nursing care than among those independent in daily living. The results indicate that measures should be taken for the elderly and those who need care from an early phase after the disaster. The analysis of data on disaster-related deaths in other affected municipalities may provide further evidence to help reduce disaster-related deaths.

Pruritus is common in patients with chronic liver disease and is improved by nalfurafine hydrochloride.

Pruritus is known to be a common complication in hepatitis patients, but the exact frequency and degree are not fully elucidated. Thus, we evaluated pruritus of 450 patients with chronic liver disease at our hospital. Pruritus was observed in 240 (53%) of the patients. Pruritus was significantly associated with males (OR = 1.51, P = 0.038) and patients with alkaline phosphatase (ALP) ≥ 200 U/L (OR = 1.56, P = 0.0495) and was significantly less in HBsAg-positive patients (OR = 0.449, P = 0.004). Seasonally, there was no difference in the frequency of pruritus between summer and winter. Of the 24 refractory pruritus patients treated with nalfurafine, 17 (71%) indicated improvement of itch, which is defined as a decrease in the visual analog scale score ≥ 30 mm. Pruritus was improved by nalfurafine both during daytime and nighttime in the Kawashima's scores evaluation. All patients who received nalfurafine exhibited improved Kawashima's scores ≥ 1 point during the daytime or nighttime. In conclusion, pruritus occurred in > 50% of patients with chronic liver disease, and predictors of pruritus were males and ALP ≥ 200 U/L. Nalfurafine may be useful for pruritus, regardless of whether daytime or nighttime.

A Diagnostically Challenging Case of Autoimmune Pancreatitis Due to Contamination of the Pathological Specimen with Early Gastric Cancer.

A 73-year-old man with worsened diabetes underwent abdominal computed tomography and was diagnosed with localized enlargement of the pancreatic tail. Based on the suspicion of autoimmune pancreatitis, endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) was performed. A cytologic examination showed findings suggestive of adenocarcinoma. Due to discrepancies between the imaging and pathological findings, esophagogastroduodenoscopy was performed. An extensive early gastric cancer lesion was detected in the posterior wall of the gastric corpus. It was therefore likely that puncturing through the tumor resulted in the contamination with cancer tissue. The possibility of a concomitant malignancy should be considered in EUS-FNA, and thorough examinations should be conducted.

Crohn's Disease with Mesalazine Allergy that Was Difficult to Differentiate from Comorbid Ulcerative Colitis.

An 18-year-old man diagnosed with ileocolonic Crohn's disease with circumferential strictures of the ascending colon started treatment with mesalazine and subsequently underwent right hemicolectomy. After surgery, the patient was started on adalimumab, and the clinical course was favorable. Nine months postoperatively, colonoscopy revealed granular mucosa with circumferential and continuous involvement from the transverse colon down to the rectum, findings which resembled ulcerative colitis. Mesalazine allergy was suspected, and the inflammatory findings resolved after discontinuing mesalazine. In patients of inflammatory bowel disease receiving mesalazine with an atypical clinical course, the possibility of mesalazine allergy must be borne in mind.

Gangliocytic Paraganglioma with Carcinoma of the Ampulla of Vater.

The patient was a "73" -year-old woman who visited our hospital with the chief complaint of weight loss. Upper gastrointestinal endoscopy revealed an enlarged ampulla of Vater, and a biopsy led to a diagnosis of Group "4" gastric carcinoma; suspicious of adenocarcinoma. There were no findings suggesting invasion into the muscle layer of duodenum, despite tumor mass formation being observed in the sphincter of Oddi. We performed endoscopic papillectomy for both diagnostic and therapeutic purposes. Pathologically, a well-differentiated adenocarcinoma existed in the superficial layer of the mucous membrane of the papilla of Vater, and gangliocytic paraganglioma was present in the deep portion. The resected margins of both lesions were negative.

The role of Gαq/Gα11 signaling in intestinal epithelial cells.

Intestinal homeostasis and the coordinated actions of digestion, absorption and excretion are tightly regulated by a number of gastrointestinal hormones. Most of them exert their actions through G-protein-coupled receptors. Recently, we showed that the absence of Gαq/Gα11 signaling impaired the maturation of Paneth cells, induced their differentiation toward goblet cells, and affected the regeneration of the colonic mucosa in an experimental model of colitis. Although an immunohistochemical study showed that Gαq/Gα11 were highly expressed in enterocytes, it seemed that enterocytes were not affected in Int-Gq/G11 double knock-out intestine. Thus, we used an intestinal epithelial cell line to examine the role of signaling through Gαq/Gα11 in enterocytes and manipulated the expression level of Gαq and/or Gα11. The proliferation was inhibited in IEC-6 cells that overexpressed Gαq/Gα11 and enhanced in IEC-6 cells in which Gαq/Gα11 was downregulated. The expression of T-cell factor 1 was increased according to the overexpression of Gαq/Gα11. The expression of Notch1 intracellular cytoplasmic domain was decreased by the overexpression of Gαq/Gα11 and increased by the downregulation of Gαq/Gα11. The relative mRNA expression of Muc2, a goblet cell marker, was elevated in a Gαq/Gα11 knock-down experiment. Our findings suggest that Gαq/Gα11-mediated signaling inhibits proliferation and may support a physiological function, such as absorption or secretion, in terminally differentiated enterocytes.

A case of a glomus tumor of the stomach resected by laparoscopy endoscopy cooperative surgery.

A 56-year-old woman who was found to have a submucosal tumor (SMT) of the stomach in a medical check-up was admitted to our hospital for a detailed investigation of the SMT. Upper gastrointestinal endoscopy revealed an SMT of 20mm at the anterior wall of the antrum of the stomach. Endoscopic ultrasonography showed a hyperechoic tumor in the fourth layer of the stomach wall. CT examination showed a strongly enhancing tumor on arterial phase images and persistent enhancement on portal venous phase images. Laparoscopy endoscopy cooperative surgery was performed with a diagnosis of SMT of the stomach highly suspicious of a glomus tumor. Immunohistochemistry revealed expression of α-SMA but no expression of desmin, c-kit, CD34, or S-100. The tumor was finally diagnosed as a glomus tumor of the stomach.