Kagawa University Hospital

Bone-avid tracers, such as 99mTc-pyrophosphate (PYP) and 99mTc-hydroxymethylene diphosphonate (HMDP), facilitate the noninvasive diagnosis of transthyretin cardiac amyloidosis (ATTR-CA). In this study, we compared these two methods. We retrospectively reviewed 83 patients with suspected or diagnosed cardiac amyloidosis (mean age, 79years; 11 women) who underwent PYP and HMDP scintigraphy. Early (1h) imaging was available for of 72/50 patients with PYP/HMDP, and all patients underwent delayed (3h) imaging. Visual grading (Dorbala score), heart-to-contralateral lung (H/CL) ratios, and relative segmental uptake (RSU) derived from SPECT (3h) polar map analysis using the quantitative perfusion SPECT software were compared. visual scores showed substantial agreement. The H/CL ratio showed a strong correlation. Bland-Altman analysis revealed fixed and proportional biases, with discrepancies increasing at higher uptake levels. The median H/CL values were comparable at 1h, whereas HMDP showed slightly higher delayed values. Receiver operating characteristic analysis indicated excellent and good diagnostic accuracy at 1 and 3h, respectively. Using routine diagnostic criteria, SPECT visual assessment demonstrated comparable diagnostic performance between PYP and HMDP. RSU analysis in 34 patients demonstrated similar segmental uptake distributions for both tracers, with basal and septal predominance, consistent with the apical-sparing patterns described previously. PYP and HMDP demonstrated comparable diagnostic accuracies for ATTR-CA. PYP has been extensively validated and is suitable for early imaging, whereas HMDP has a slightly higher delayed uptake and lower blood pool activity. When interpreted using routine diagnostic criteria, SPECT provided equivalent diagnostic performance for PYP and HMDP. Segmental analyses confirmed the preservation of the characteristic apical sparing across the tracers. Both tracers are clinically interchangeable when standardized acquisition and interpretation protocols are applied.

Glucocorticoids (GC) comprise a cornerstone in the treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV); however, prolonged GC exposure leads to substantial toxicity and immune system-related complications. Hence, identifying treatment strategies that enable early GC withdrawal while maintaining disease control is of clinical importance. To investigate the proportion and characteristics of patients achieving GC-free clinical remission (GFCR) 48 weeks after treatment initiation in patients with newly diagnosed microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) and examine their long-term clinical outcomes (up to week 96). We conducted a retrospective cohort study using clinical data from a multicenter, nationwide registry in Japan comprising patients with newly diagnosed MPA or GPA and at least 48 weeks of follow-up. GFCR was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 with complete GC withdrawal. Univariable and multivariable logistic regression analyses were used to identify independent predictors of GFCR at week 48, and a sensitivity analysis using a matched cohort was performed to validate robustness. A total of 728 patients were enrolled in the registry, of whom 544 were followed for ≥ 48 weeks; among them, 29 (5.3%) achieved GFCR at week 48. Use of rituximab (RTX) for induction therapy and avacopan within 48 weeks were independently associated with achieving GFCR (multivariable analysis, model 1: RTX: odds ratio [OR]: 3.9, 95% confidence interval [CI]: 1.5–10.0; avacopan: OR: 24.3, 95% CI: 5.8–101.9; both p < 0.01). Conversely, methylprednisolone pulse therapy was associated with a lower likelihood of achieving GFCR (multivariable analysis, model 1: OR: 0.08, 95% CI: 0.02–0.44; p < 0.01). Patient’s demographic and baseline disease characteristics were not predictive of GFCR. In the long-term (weeks 48 through 96), the rates of death, relapse, and serious infection were similar regardless of GFCR status at week 48. The use of RTX for induction therapy and avacopan within 48 weeks is independently associated with achieving GFCR at week 48 in patients with MPA and GPA, supporting their potential as key components of GC-sparing strategies in AAV. Prospective studies are needed to confirm these results and optimize treatment algorithms to ensure disease control and minimize GC-related toxicity.

About 80% of neonates with hypoxic-ischaemic encephalopathy (HIE) have cardiovascular dysfunction and are vulnerable to late-onset injury due to disrupted cerebral blood flow and metabolism. We previously reported the neuroprotective effect of hydrogen (H2) gas. This study investigated the effects of H2 gas inhalation on cardiac function in piglets after hypoxia-ischaemia (HI). Seventeen piglets ≤ 24h old were subjected to HI insult for approximately 40min and, after 10min of resuscitation, were divided into an HI group (n = 10) and an HI-H2 gas inhalation group (2.2%-2.7% H2 gas for 6h; n = 7). We examined biventricular single stroke volume (SV) and cardiac output (CO) within 6h after HI insult using transthoracic echocardiography. The HI group (n = 10) showed a transient increase in SV and CO followed by a decline. In contrast, the HI-H2 group (n = 7) experienced a increase in right ventricular SV and CO, with a mild decrease in left ventricular parameters. Notably, right CO were significantly higher at 5h after insult in the HI-H2 group (p = 0.023). Hydrogen gas inhalation may help preserve right ventricular performance after asphyxia.

550 Background: Biliary tract cancer (BTC) is an aggressive malignancy with limited treatment options and a poor prognosis. Immune checkpoint inhibitor combined with gemcitabine and cisplatin (GC + ICI) demonstrated overall survival (OS) improvement for unresectable biliary tract cancer (BTC) in the TOPAZ-1 and KEYNOTE-966 trials. In Japan, gemcitabine, cisplatin, and S-1 (GCS) also showed superiority over GC. However, the optimal first-line regimen across real-world settings remains uncertain. We conducted a multicenter retrospective study to evaluate the efficacy and safety of GC+ICI and GCS in routine practice. Methods: We retrospectively collected data from seven Japanese institutions in patients with BTC treated with GC+ICI (durvalumab or pembrolizumab) or GCS as first-line chemotherapy between April 2019 and January 2025. Inclusion criteria were as follows: an ECOG performance status of 0–2, no prior systemic therapy for biliary tract cancer, and adequate organ function. Primary endpoints were progression-free survival (PFS) and OS, with overall response rate (ORR) and disease control rate (DCR) as secondary endpoints. Survival outcomes were estimated using the Kaplan–Meier method. Safety was evaluated according to CTCAE v5.0. Results: A total of 148 patients were included: 91 in the GC+ICI group (median follow-up 15.1 months) and 57 in the GCS group (median follow-up 26.5 months). In the GC+ICI group, 92% of patients received durvalumab and 8% received pembrolizumab. Median age were 73 (40–84) and 69 (42–83) years, respectively. ECOG PS 0/1/2 were 64%/34%/2% in GC+ICI and 58%/40%/2% in GCS. Tumor sites differed substantially: intrahepatic/perihilar/extrahepatic/gallbladder/ampulla were 18%/23%/22%/33%/0% in GC+ICI and 40%/18%/9%/28%/5% in GCS. ORR was 34% with GC+ICI and 33% with GCS, while DCR was 76% 74%. Median PFS was 6.9 months (95% CI 6.0–7.3) with GC+ICI and 5.5 months (95% CI 3.5–8.3) with GCS. Median OS was 14.3 months (95% CI 10.6–16.4) for GC+ICI and 9.9 months (95% CI 8.5–13.5) for GCS. Grade 3–4 adverse events occurred in 50% and 44% of patients in the GC+ICI and GCS groups, respectively. In the GC+ICI group, immune-related adverse events were observed in 19.8% of patients, with 4.4% experiencing grade 3–4 events. Treatment discontinuation due to toxicity was observed in 12% and 9%. Conclusions: The present multicenter retrospective study demonstrated that the GC+ICI and GCS regimens achieved comparable objective response rates. There was a tendency toward improved survival in the GC+ICI group. Given the consideration of discrepancies in patient characteristics and treatment era, the findings of this study offer substantial real-world evidence to inform first-line treatment strategies for patients with unresectable BTC.

This study aimed to compare the utility of 3’-deoxy-3’-¹⁸F-fluorothymidine (FLT) positron emission tomography (PET) and 2-deoxy-2-¹⁸F-fluoro-D-glucose (FDG) PET in predicting pathological response to neoadjuvant chemoradiotherapy (nCRT) in patients with resectable and borderline resectable pancreatic ductal adenocarcinoma (PDAC), and retrospectively evaluated 21 patients who underwent FLT and FDG PET before and after nCRT. The maximum standardised uptake value (SUVmax) of the primary tumour was measured pre- and post-nCRT, and the percentage reduction in SUVmax (%ΔSUVmax) was calculated. Pathological response was assessed using the Evans grading system, with Grades I–IIa defined as non-responders and Grades IIb–IV as responders. Mann-Whitney U tests and receiver operating characteristic (ROC) analyses were performed. Three patients with blood glucose levels ≥ 200 mg/dL at pre-nCRT FDG PET were excluded from FDG PET analysis. Surgical specimens classified nine patients as responders and 12 as non-responders. SUVmax_pre-nCRT did not differ significantly between groups for either modality. SUVmax_post-nCRT was significantly lower in responders for both FLT (P = 0.001) and FDG (P = 0.009) PET. A significant %ΔSUVmax difference was observed only with FLT PET (P = 0.002). Areas under the curve for predicting response to nCRT were: FLT SUVmax_post-nCRT (0.90), FLT %ΔSUVmax (0.89), and FDG SUVmax_post-nCRT (0.86). FLT PET demonstrated comparable performance to FDG PET in assessing response to nCRT in resectable and borderline resectable PDAC, with potential as a complementary or alternative imaging modality in neoadjuvant settings given its independence from glycaemic status and biological proliferation specificity.

Anencephaly is a fatal neural tube defect often linked to folic acid deficiency and undetected in resource-limited settings. We report a case in rural Uganda where portable ultrasound revealed anencephaly with polyhydramnios in a patient who has been pregnant six times, with one term delivery, four preterm deliveries, and no abortions, and currently has one living child (G6P1401). Early detection of this anomaly enabled surgical planning and maternal counseling before stillbirth. This case highlights the impact of malnutrition and the importance of portable ultrasound use to improve prenatal care in underserved regions of the world.

Introduction: Early detection of duodenal neoplasms is crucial because of the complications associated with their treatment and their increasing recognition. However, standardised protocols for duodenal observation during screening esophagogastroduodenoscopy (EGD) have not been established. This study was performed to survey the current practical status of duodenal observation and to evaluate the clinical relevance of our systematic photo-documentation protocol, known as the Seven Pictures Rule (7 PR). Methods: A cross-sectional survey using a web-based self-administered questionnaire was conducted among endoscopists in a regional area of Japan. The questionnaire included items on respondents’ professional background, duodenal observation practices during screening EGD (observed locations, observation time, ampulla visualisation, and patient discomfort), as well as awareness, application, and perceived effects of the 7 PR, and preferences for future protocols. Data were anonymised and analysed descriptively. Results: Of the 99 endoscopists included, 96.7% reported observing the duodenum based on their own individual methods. The intentional observation rates by location were highest in the following order: bulb (99%), ampulla in descending (94%), contralateral ampulla in descending (84%), superior duodenal angle (77%), contralateral superior duodenal angle (79%), and transverse duodenum (33%). However, only 14% of respondents reported successfully observing the ampulla in more than 75% of cases. While 62% were aware of the 7 PR, only one-fourth had implemented it in practice. Notably, 43% of respondents familiar with the 7 PR felt that it had led to an increased detection of duodenal neoplasms. Conclusions: Current duodenal observation practices vary among endoscopists. The establishment of a systematic photo-documentation protocol such as the 7 PR may serve as a useful strategy for improving the detection of duodenal neoplasms. Further optimisation of the protocol is warranted to support broader adoption in clinical settings.