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Shinsaku Imashuku,1 Naoko Kudo,1 Kagekatsu Kubo,1 Katsuyasu Saigo,1 Nanako Okuno,2 Kaoru Tohyama21Division of Hematology, Takasago-seibu Hospital, Takasago, Japan; 2Department of Laboratory Medicine, Kawasaki Medical School, Kurashiki, JapanBackground: Acquired hemophilia A is rarely found in association with myeloproliferative neoplasms, such as the JAK2 kinase V617F mutation-positive chronic neutrophilic leukemia (CNL).Case report: An 80-year-old Japanese male was diagnosed with acquired hemophilia A. He had compartment-like symptoms due to soft tissue hemorrhage in his left forearm and right lower extremity. A blood examination showed neutrophilia with a white blood cell count of 31,900/µL (91.9% neutrophils), an activated partial thromboplastin time of 69.0 seconds, coagulation factor VIII (FVIII) < 1.0%, and anti-FVIII inhibitor, 190 BU/mL. The bleeding episodes were controlled with intravenous activated prothrombin complex concentrate (FEIBA®) followed by recombinant factor VIIa (NovoSeven®). In addition, oral prednisolone (maximum dose, 30 mg/day) plus four doses of rituximab effectively suppressed anti-FVIII inhibitor levels while simultaneously reducing the neutrophil count. CNL with the JAK2 kinase V617F mutation was identified as the underlying disease.Conclusion: This report describes the effectiveness of a combination of prednisolone and rituximab in managing acquired hemophilia A in an elderly man with a rare case of JAK2 kinase V617F mutation-positive CNL.Keywords: acquired hemophilia A, chronic neutrophilic leukemia, JAK2 kinase, V617 mutation, rituximab

Probe-based confocal laser endomicroscopy is an endoscopic technique that enables in vivo histological evaluation using fluorescent pigment. The ability to diagnostically differentiate between benign and malignant biliary disease using the “CholangioFlexTM”, a dedicated biliary device, has been reported. However, the Miami and Paris classifications, used as diagnostic criteria, mainly evaluate findings in the submucosa, and visualizing the epithelium as the main site of lesions remains difficult. To address this problem, we verified the imaging findings and diagnostic ability of three types of probes: CholangioFlexTM, GastroFlexTM, and AlveoFlexTM. With GastroFlexTM, the clear mucosal epithelium was observed, and differential diagnoses as benign/malignant could be made based on epithelial findings. GastroFlexTM may be a good first-choice probe for probe-based confocal laser endomicroscopy of biliary diseases, and a new diagnostic classification based on bile duct epithelial findings may provide useful criteria independent of the Miami or Paris classifications.

Evaluates full-scale thermal solidification processes implemented in lightweight aggregate, slag and brick in Tokyo, Japan. Key issues of interest; Analysis of pertinent topics and relevant issues; Implications on sludge management for the 21st century.

Brick making from 100 percent of incinerated sewage sludge ash is developed. There are already 8 full scale plants in Japan. All of them employ high pressure molding of incinerated sewage sludge ash and firing at about 1000 deg. C. The end products are widely accepted for public works such as flooring of plazas or pedestrian walk ways. Early in the history of the development, overall quality of the sewage brick was not competitive. They were subject to get moss growth on the surface because of its high moisture content, or whitening because of leaching of Calcium Carbonate. The problems were solved by increasing the temperature of the firing process and by chemical coating. The manufacturing cost of one sewage brick is around two dollars and the retail price is one dollar. [ABSTRACT FROM AUTHOR]

In 1990, Shiga Prefectural Government started to operate a full scale sludge melting plan. The plant has the capacity for handling 40 tondewatered sewage sludge cake per day. The slag has been reused for construction material, primarily as a substitute for coarse aggregate.Six years operation showed that dewatered cake could be reduced to 1/14 in weight and to 1/7 in volume by this melting and solidificationprocess. The operation cost of the process was $125.4 for each ton of dewatered sewage sludge cake with 65% moisture content, which represented approximately 5-7% sewerage user's charge. [ABSTRACT FROM AUTHOR]

Background: Median arcuate ligament syndrome describes a clinical presentation associated with direct compression of the celiac artery by the median arcuate ligament. Decreased blood flow from the celiac artery and increased flow from superior mesenteric artery causes blood flow in the pancreatic arcade to increase, and aneurysms can form. We report our experience with six cases of retroperitoneal hemorrhage induced by median arcuate ligament syndrome in patients during the COVID-19 period., Case Presentation: The time from the onset of COVID-19 to that of abdominal pain in the patients ranged from 3 to 9 days. None of the patients required oxygen for COVID-19. We used contrast-enhanced computed tomography to diagnose retroperitoneal hemorrhage and formation of a visceral pseudoaneurysm or aneurysm due to median arcuate ligament syndrome. Five patients underwent transcatheter arterial embolization. One patient suffered complications of duodenal stenosis and another suffered portal vein thrombosis., Conclusions: Retroperitoneal hemorrhage associated with median arcuate ligament syndrome is rare. However, it might be triggered by COVID-19 disease, resulting in arterial disruption and hemorrhage., Competing Interests: Dr. Yasuyuki Kuwagata is an Editorial Board member of AMS Journal and a co‐author of this article. To minimize bias, they were excluded from all editorial decision‐making related to the acceptance of this article for publication., (© 2024 The Author(s). Acute Medicine & Surgery published by John Wiley & Sons Australia, Ltd on behalf of Japanese Association for Acute Medicine.)

Background and Study Aims: Endoscopic ultrasonography (EUS)-guided ethanol injection (EI) has recently been introduced as one of the management strategies for pancreatic neuroendocrine neoplasms (PNENs). However, its role as a surgical alternative is unclear. We evaluated the efficacy and safety of EUS-EI in treating small PNENs through a prospective multicentre study., Patients and Methods: Patients with Grade 1 tumours of ≤15 mm confirmed by pathology were included. The primary endpoint assessed efficacy and safety, measuring complete ablation using computed tomography at 1 and 6 months, prevention of adverse events (AEs) within 1 month, severe pancreatic fistula at 1 month, and diabetes mellitus (DM) incidence/worsening at 6 months. The composite endpoint of EUS-EI was compared with that of historical results of a study based on surgical treatment., Results: Twenty-five patients with PNENs, with a median tumour size of 10.1 mm, were treated using EUS-EI. Seventy-six percent of the patients achieved the composite primary endpoint (19/25) (95% confidence interval [CI]=54.9%-90.6%), a proportion significantly higher than that of surgical treatment (P=0.0083). Regarding efficacy, 88% (22/25) of the patients achieved complete ablation at 1 and 6 months (95% CI=68.8%-97.5%). Regarding safety, 96% (24/25) of the patients had no severe AEs within 1 month (95% CI=79.7%-99.9%). No patients had severe pancreatic fistulas at 1 month, and 84% (21/25) of the patients had no incidence or exacerbation, or both, of DM at 6 months (95% CI=63.9%-95.5%)., Conclusion: EUS-EI is safe and could be a potent treatment option for patients with small PNENs., Competing Interests: The authors declare that they have no conflict of interest., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

Objective Adverse events such as bile leakage and bleeding are among the issues that need to be resolved in EUS-guided choledochoduodenostomy (EUS-CDS). To overcome this problem, we developed a new EUS-CDS technique using a 19-G Franseen needle without tract dilation. This study aimed to evaluate the safety and efficacy of the new EUS-CDS technique. Methods This single-center retrospective study included 20 consecutive patients who underwent EUS-CDS for primary drainage using a 19-G Franseen needle between March 2020 and May 2023. The primary endpoint was the technical success rate of EUS-CDS without tract dilation. Results The technical success rate of EUS-CDS was 20/20 (100%). None of the patients required any additional tract dilation, such as by using a balloon or electric cautery. The median procedure time was 7.8 [range, 3.2-19.4] min. No early adverse events were observed. Conclusion The 19-G Franseen needle appeared to have a sufficient dilatory effect during puncturing. This EUS-CDS technique appears to be safe and effective and has the advantages of no adverse events and it is also a simplified procedure, which suggests its potential for widespread use in primary drainage.

In this report, we describe a very rare case of metastatic epithelioid hemangio-endothelioma (EHE) originating from other organs such as the lung and requiring craniotomy due to subsequent hemorrhage. A 50-year-old man was diagnosed with EHE in the bilateral lungs, the mediastinum, and the right adrenal gland 8 years earlier. One year earlier, he had developed spinal metastasis. Six months earlier, a screening brain MRI had revealed multiple brain metastases of tumor. He developed subcortical hemorrhage from the tumor in the right parietal lobe and successfully underwent removal of hematoma and tumor. Histopathological examinations revealed EHE. Metastatic EHE is very rare but may be at high risk of intracranial hemorrhage. It is quite important to consider the possibility of brain metastasis and subsequent bleeding when treating patients with EHE., (© 2024 Japanese Society of Neuropathology.)

Alveolar rhabdomyosarcoma (ARMS) is a malignant tumor with skeletal muscle differentiation that usually occurs in soft tissues of the extremities or trunk. To date, only a few cases of primary gastric ARMS have been reported. Herein, we describe a case of ARMS in a man in his 80s and present a literature review. A submucosal tumor-like lesion was detected endoscopically. Histologically, the tumor cells exhibited a poorly differentiated morphology with hyperchromatic nuclei. Neuroendocrine markers, such as synaptophysin and CD56, were positive, and neuroendocrine carcinoma (NEC) was considered a differential diagnosis. However, both myogenic markers and OLIG2, which reflect FAX3 or FAX7 fusion, were positive, leading to a diagnosis of ARMS. Primary gastric ARMS is an extremely rare condition. Better awareness of this entity and its similarity to NEC is necessary for appropriate diagnosis and treatment., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Okuno et al.)

Background/aims: Dilation of the tract before stent deployment is a challenging step in endoscopic ultrasound-guided pancreatic duct drainage (EUS-PDD). In this study, we examined the effectiveness and safety of a novel spiral dilator, Tornus ES (Asahi Intec), for EUS-PDD., Methods: This was a retrospective, single-arm, observational study at Aichi Cancer Center Hospital. The punctured tract was dilated using a Tornus ES dilator in all EUS-PDD cases. Our primary endpoint was the technical success rate of initial tract dilation. Technical success was defined as successful fistula dilation using a Tornus ES followed by successful stent insertion. Secondary endpoints were procedure times and early adverse events., Results: A total of 12 patients were included between December 2021 and March 2023. EUS-PDD was performed in 11 patients for post-pancreaticoduodenectomy anastomotic strictures and one patient with pancreatitis with duodenal perforation. The technical success rates of stent insertion and fistula dilation using a Tornus ES dilator was 100%. The median procedure time was 24 minutes. No remarkable adverse events related to the procedure were observed, apart from fever, which occurred in 2 patients., Conclusions: Tract dilation in EUS-PDD using a Tornus ES is effective and safe.

Background: Multidisciplinary care for Chronic Kidney Disease (CKD) has been reported to be effective in preventing deterioration of renal function and avoiding hemodialysis induction using a central venous catheter., Methods: We included 171 patients who received dialysis at our department between October 2014 and June 2017. Patients were divided into two groups: an inpatient group who received inpatient multidisciplinary care for CKD (educational hospitalization) prior to outpatient collaborative care from their family physician and nephrologist, and a non-inpatient group who did not receive such care. We compared factors related to dialysis induction., Results: There was no significant difference in eGFR between the groups at the start of observation. The mean time from the start of observation to dialysis induction (inpatient group vs. non-inpatient group; 40.8 ± 2.8 vs. 23.9 ± 3.0 months, respectively; P < 0.001) and the rate of hemodialysis induction using a central venous catheter (22.5 vs. 47.1%, respectively; P = 0.002) were significantly different between the groups. Survival analysis showed that the time to dialysis induction was significantly longer in the inpatient group (P = 0.0001). Multivariate analysis revealed that educational hospitalization (odds ratio = 0.30 [95% CI 0.13, 0.67]) was significantly associated with hemodialysis induction using a central venous catheter., Conclusion: Educational hospitalization prior to outpatient collaborative care is beneficial for preventing hemodialysis induction using a central venous catheter and postponing dialysis induction., (© 2024. The Author(s).)