We herein report an unusual case of Erdheim-Chester disease (ECD), a rare non-Langerhans cell histiocytosis, and emphasize its unique presentation and diagnostic challenges. Our patient exhibited uncommon symptoms and significant organ involvement, particularly pancreatic enlargement that is not typically associated with ECD. Contrast-enhanced harmonic endoscopic ultrasonography (CEH-EUS) and EUS-fine needle aspiration (EUS-FNA) play crucial roles in the comprehensive assessment of the disease, demonstrating their superiority in identifying and characterizing elusive ECD lesions. This is the first report to document pancreatic lesions in patients with ECD evaluated using CEH-EUS. EUS-FNA is valuable for diagnosing rare diseases, including ECD, with diffuse pancreatic enlargement.

Intrahepatic cholangiocarcinoma (ICC), a severe liver cancer, makes up to 20% of all hepatic malignancies and is difficult to diagnose early due to its often asymptomatic nature. This case report documents a rare presentation of ICC with multiple diffuse nodules not previously recorded in medical literature. A 65-year-old man with no significant medical history presented with back pain, anorexia, and significant weight loss. Elevated tumor markers and enlarged lymph nodes were observed, though imaging did not reveal a primary liver mass. Diagnostic efforts, including computed tomography and positron emission tomography scans and biopsies of lymph nodes and bone marrow, suggested adenocarcinoma of unknown primary origin. A definitive diagnosis was only made post-mortem, revealing multiple diffuse nodules in the liver identified as ICC, marking a rare presentation without a primary mass. This case highlights the diagnostic challenges posed by atypical ICC manifestations, where typical imaging does not indicate a primary mass, delaying diagnosis and treatment. The findings emphasize the importance of considering ICC in differential diagnoses in cases of unknown primary adenocarcinoma with liver involvement. The discovery of ICC with diffusely infiltrative nodules underscores the necessity for comprehensive diagnostic evaluations in patients presenting with nonspecific systemic symptoms and abnormal liver findings.

Background and Aims: Direct percutaneous endoscopic jejunostomy (DPEJ) is an alternative method of enteral feeding to percutaneous endoscopic gastrostomy (PEG). Although long-term outcomes of PEG have been reported, little is known regarding the outcomes of DPEJ. Methods: A retrospective cohort study was conducted including 115 and 651 consecutive attempts of DPEJ and PEG, respectively, in a total of 766 elderly patients between April 2004 and March 2019. Patients' clinical background, procedural and long-term outcomes, survival analysis, and cause of death were analyzed. Results: Successful placement rates were 93.9% and 97.1% for DPEJ and PEG, respectively. There was no significant difference in procedure-related adverse events (AEs) between the DPEJ and PEG groups. Rates of pneumonia, vomiting, and upper GI bleeding were significantly lower, whereas those of fistula enlargement and ileus were significantly higher in the DPEJ group as long-term AEs. The median survival periods were 694 and 734 days for DPEJ and PEG, respectively, with no significant differences between the 2 groups. Multivariate analysis revealed that age 80 years old or older, C-reactive protein level of 1.0 mg/dL or higher, and the presence of diabetes were independent risk factors for mortality after DPEJ. Respiratory tract infection was the primary cause of death in both groups. Conclusions: DPEJ is considered a safe and feasible method of access for enteral feeding as well as PEG. Although the survival period after DPEJ may be expected to be as long as that with PEG, DPEJ-specific AEs should be kept in mind on long-term feeding.

Objective As direct jejunal feeding often causes great fluctuation in glucose levels, continuous or slow infusion is recommended for jejunal tube-fed patients. However, continuous feeding results in prolonged immobility and the loss of activities of daily living. We investigated whether or not intermittent feeding of a low-carbohydrate high-monounsaturated fatty acid (LC/HM) nutrient formula reduces glucose fluctuation in patients who have undergone jejunotomy. Methods Ten bed-ridden non-diabetic patients receiving enteral feeding via a jejunostomy tube were enrolled in this study. LC/HM formula and standard control formula were infused in cross-over order for each patient at a speed of 160 kcal/h. Blood glucose levels were monitored by a continuous glucose monitoring system during the investigation period. Results The mean and standard deviation of the glucose concentrations and mean amplitude of glucose excursion (MAGE) were markedly lower while receiving LC/HM formula than while receiving control standard formula (104 vs. 136 mg/dL, 18.1 vs. 58.1 mg/dL, 50.8 vs. 160 mg/dL, respectively). The post-infusion hyperglycemia [area under the curve (AUC) ＞140 mg/dL] and peak value of the glucose level were also significantly lower in patients fed LC/HM than the control (25.7 vs. 880 mg・h/dL and 153 vs. 272 mg/dL, respectively). Reactive hypoglycemia (AUC ＜70 mg/dL) was also significantly lower (0.63 vs. 16.7 mg・h/dL) and the minimum value of the glucose level higher (78.4 vs. 61.8 mg/dL) in patients fed LC/HM than the control. Conclusion The LC/HM formula is considered to markedly inhibit glycemic spikes and prevent rebound hypoglycemia in patients who receive enteral feeding after jejunostomy.

A 74-year-old woman was admitted to the hospital with epigastric pain, severe nausea and vomiting, and diarrhea that had started 3 days previously. She had eaten raw Ayu fish 4 days before admission. An abdominal contrast-enhanced computed tomography scan revealed the presence of gas in the portal vein and remarkable thickening of the gastric wall. In many cases, the gas in the portal vein indicates the existence of intestinal necrosis. Esophagogastroduodenoscopy showed a submucosal tumor-like elevation in the gastric corpus. She was diagnosed with sepsis and phlegmonous gastritis (PG) with hepatic portal venous gas (HPVG) caused by Aeromonas hydrophila, which was detected in her stool. The patient was treated with antibiotics and discharged from the hospital 23 days after admission in a stable condition. When caused by PG, HPVG is not necessarily considered a poor prognostic factor and is expected to be treatable with medication. However, patients should be closely monitored for signs of a life-threatening pathology such as intestinal necrosis.