IgG4-Related Perineural Disease

Aims. To elucidate characteristics of IgG4-related disease involving the peripheral nervous system. Methods. Retrospective review of 106 patients with IgG4-related disease identified 21 peripheral nerve lesions in 7 patients. Clinicopathological and radiological features were examined. Results. Peripheral nerve lesions were commonly identified in orbital or paravertebral area, involving orbital (n = 9), optic (n = 4), spinal (n = 7), and great auricular nerves (n = 1). The predominant radiological feature was a distinct perineural soft tissue mass, ranging 8 to 30 mm in diameter. Histologically, the epineurium was preferentially involved by massive lymphoplasmacytic infiltration rich in IgG4+ plasma cells. All lesions were neurologically asymptomatic and steroid-responsive at the first presentation, but one recurrent lesion around the optic nerve caused failing vision. Conclusion. IgG4-related disease of the peripheral nervous system is characterized by orbital or paravertebral localization, perineural mass formation, and rare neurologic symptoms. The term “IgG4-related perineural disease” seems appropriate to describe this entity

Clinicopathology and prognosis of mucinous gastric carcinoma

Background/Aims: Mucinous gastric carcinoma (MGC) is a rare histopathological type of gastric carcinoma, for which the clinicopathological features and prognosis remain controversial. To clarify the clinical significance of mucinous histological type in gastric cancer, we studied clinicopathological characteristics of MGC tumors and prognosis of patients. Methodology: Forty-one patients with MGC and 1,407 patients with non-mucinous gastric carcinoma (NGC) were included in the study. Tumors were evaluated against patient gender and age, tumor location, size, and macroscopic type, depth of gastric wall invasion, lymph node metastasis, liver metastasis, peritoneal dissemination, distant metastasis, stage, and operative curability. Results: Compared with NGC tumors, MGC tumors were larger, showed more serosal invasion, were associated with a higher incidence of lymph node metastasis, and peritoneal dissemination, and tended to be at a more advanced stage. However, multivariate analysis demonstrated that the mucinous histological type was neither an independent prognostic factor nor an independent risk factor for lymph node metastasis in patients with gastric cancer. Conclusions: The mucinous histological type had no influence on patient outcome or the frequency of lymph node metastasis. MGC tumors are therefore biologically similar to those in NGC

Scientific opportunies for bERLinPro 2020+, report with ideas and conclusions from bERLinProCamp 2019

The Energy Recovery Linac (ERL) paradigm offers the promise to generate intense electron beams of superior quality with extremely small six-dimensional phase space for many applications in the physical sciences, materials science, chemistry, health, information technology and security. Helmholtz-Zentrum Berlin started in 2010 an intensive R\&D programme to address the challenges related to the ERL as driver for future light sources by setting up the bERLinPro (Berlin ERL Project) ERL with 50 MeV beam energy and high average current. The project is close to reach its major milestone in 2020, acceleration and recovery of a high brightness electron beam. The goal of bERLinProCamp 2019 was to discuss scientific opportunities for bERLinPro 2020+. bERLinProCamp 2019 was held on Tue, 17.09.2019 at Helmholtz-Zentrum Berlin, Berlin, Germany. This paper summarizes the main themes and output of the workshop

Omecamtiv mecarbil in chronic heart failure with reduced ejection fraction, GALACTIC‐HF: baseline characteristics and comparison with contemporary clinical trials

Aims: The safety and efficacy of the novel selective cardiac myosin activator, omecamtiv mecarbil, in patients with heart failure with reduced ejection fraction (HFrEF) is tested in the Global Approach to Lowering Adverse Cardiac outcomes Through Improving Contractility in Heart Failure (GALACTIC‐HF) trial. Here we describe the baseline characteristics of participants in GALACTIC‐HF and how these compare with other contemporary trials. Methods and Results: Adults with established HFrEF, New York Heart Association functional class (NYHA) ≥ II, EF ≤35%, elevated natriuretic peptides and either current hospitalization for HF or history of hospitalization/ emergency department visit for HF within a year were randomized to either placebo or omecamtiv mecarbil (pharmacokinetic‐guided dosing: 25, 37.5 or 50 mg bid). 8256 patients [male (79%), non‐white (22%), mean age 65 years] were enrolled with a mean EF 27%, ischemic etiology in 54%, NYHA II 53% and III/IV 47%, and median NT‐proBNP 1971 pg/mL. HF therapies at baseline were among the most effectively employed in contemporary HF trials. GALACTIC‐HF randomized patients representative of recent HF registries and trials with substantial numbers of patients also having characteristics understudied in previous trials including more from North America (n = 1386), enrolled as inpatients (n = 2084), systolic blood pressure < 100 mmHg (n = 1127), estimated glomerular filtration rate < 30 mL/min/1.73 m2 (n = 528), and treated with sacubitril‐valsartan at baseline (n = 1594). Conclusions: GALACTIC‐HF enrolled a well‐treated, high‐risk population from both inpatient and outpatient settings, which will provide a definitive evaluation of the efficacy and safety of this novel therapy, as well as informing its potential future implementation

『茶譜』巻七注釈

International audienc

Study of hepatic toxicity in small liver tumors after photon or proton therapy based on factors predicting the benefits of proton

Objectives: In a previous study of hepatic toxicity, the following three factors were identified to predict the benefits of proton beam therapy (PBT) for hepatocellular carcinomas (HCCs) with a maximum diameter of ≤5 cm and Child-pugh grade A (CP-A): number of tumors (1 vs ≥2), the location of tumors (hepatic hilum or others), and the sum of the diameters of lesions. The aim of this study is to analyze the association between these three factors and hepatic toxicity. Methods: We retrospectively reviewed patients of CP-A treated with PBT or photon stereotactic body radiotherapy (X-ray radiotherapy, XRT) for HCC ≤5 cm. For normal liver dose, the V5, V10, V20 (volumes receiving 5, 10, and 20 Gy at least), and the mean dose was evaluated. The albumin-bilirubin (ALBI) and CP score changes from the baseline were evaluated at 3 and 6 months after treatment. Results: In 89 patients (XRT: 48, PBT: 41), those with two or three (2-3) predictive factors were higher normal liver doses than with zero or one (0-1) factor. In the PBT group, the ALBI score worsened more in patients with 2-3 factors than those with 0-1 factor, at 3 months (median: 0.26 vs 0.02, p = 0.032) and at 6 months (median: 0.35 vs 0.10, p = 0.009). The ALBI score change in the XRT group and CP score change in either modality were not significantly different in the number of predictive factors. Conclusion: The predictive factor numbers predicted the ALBI score change in PBT but not in XRT. Advances in knowledge: This study suggest that the number of predictive factors previously identified (0-1 vs 2-3) were significantly associated with dosimetric parameters of the normal liver in both modalities. In the proton group, the number of predictive factors was associated with a worsening ALBI score at 3 and 6 months, but these associations were not found in the photon SBRT group.A correction has been published: British Journal of Radiology, Volume 97, Issue 1155, March 2024, Page 694https://doi.org/10.1093/bjr/tqad05

Endoscopic naso-pancreatic drainage for the treatment of pancreatic fistula occurring after LDLT

Pancreatic fistula is a quite rare complication in patients who undergo living donor liver transplantation (LDLT). However, in the cases that show pancreatic fistula, the limited volume of the graft and the resultant inadequate liver function may complicate the management of the fistula. As a result, the pancreatic fistula may result in the death of the patient. We present 2 cases in which endoscopic treatment was effective against pancreatic fistulas that developed after LDLT. In case 1, a 61-year-old woman underwent LDLT for primary biliary cirrhosis. Because of a portal venous thrombus caused by a splenorenal shunt, the patient underwent portal vein reconstruction, and a splenorenal shunt was ligated on postoperative day (POD) 7. The main pancreatic duct was injured during the manipulation to achieve hemostasis, thereby necessitating open drainage. However, discharge of pancreatic fluid continued even after POD 300. Endoscopic naso-pancreatic drainage (ENPD) was performed, and this procedure resulted in a remarkable decrease in drain output. The refractory pancreatic fistula healed on day 40 after ENPD. In case 2, a 58-year-old man underwent LDLT for cirrhosis caused by the hepatitis C virus. When the portal vein was exposed during thrombectomy, the pancreatic head was injured, which led to the formation of a pancreatic fistula. Conservative therapy was ineffective; therefore, ENPD was performed. The pancreatic fistula healed on day 38 after ENPD. The findings in these 2 cases show that endoscopic drainage of the main pancreatic duct is a less invasive and effective treatment for pancreatic fistulas that develop after LDLT

Treatment outcomes of stereotactic body radiation therapy using a real-time tumor-tracking radiotherapy system for hepatocellular carcinomas

Aim To report the outcomes of stereotactic body radiotherapy using a real-time tumor-tracking radiotherapy system for hepatocellular carcinoma patients. Methods From January 2005 to July 2018, 63 patients with 74 lesions with a maximum diameter = 9 was included, and 85.6% had a score of 5 or 6. Using the biological effective dose (BED) with an alpha/beta ratio of 10 (BED10), the median dose in BED10 at the reference point was 76.8 Gy (range 60-122.5 Gy). Overall survival (OS) and local control rates were assessed using the Kaplan-Meier method. Results With a median follow-up period of 24.6 months (range 0.9-118.4 months), the 1-year and 2-year OS rates were 86.8% (95% confidence interval [95% CI] 75.8-93.3) and 71.1% (57.8-81.6), respectively. The 2-year OS was 89.6% in patients with the baseline modified albumin-bilirubin (mALBI) grade =1, and 61.7% in patients with grade >= 2a. In the multivariate analysis, the mALBI grade (=1 vs. >= 2a) was a significant factor for OS (p = 0.028, 95% CI 1.11-6.18). The 1-year and 2-year local control rates were 100% (100-100%) and 92.0% (77.5-97.5%). The local control rates were significantly higher in the BED10 >= 100 Gy group than in the BED10 = 100 Gy to the reference point