Kitakyushu City Yahata Hospital

ABSTRACTBackground and ObjectiveGenetic polymorphisms have been associated with susceptibility to interstitial lung diseases including idiopathic pulmonary fibrosis (IPF). We have now examined the relation between single nucleotide polymorphisms (SNPs) and clinical course, including acute exacerbation (AE), in addition to disease susceptibility for IPF and unclassifiable idiopathic interstitial pneumonias (IIPs).MethodsDNA samples were collected from 223 IPF patients and 160 unclassifiable IIP patients included in a prospective, multicentre observational study in Japan. Nonfibrotic control subjects (n = 379) were selected from a previous study. Genotyping of TERT rs2736100, TERC rs1881984, MUC5B rs35705950, DSP rs2076295, and AKAP13 rs62025270 was conducted with commercial assays. The association between these SNPs and disease susceptibility, prognosis, or the cumulative incidence of AE was assessed.ResultsTERT rs2736100 and MUC5B rs35705950 were significantly associated with IPF risk, and DSP rs2076295 was linked to prognosis and the incidence of AE in IPF patients. TERT rs2736100 was significantly associated with prognosis in IPF patients and the incidence of AE in patients with unclassifiable IIPs. No individuals with the TT genotype of MUC5B rs35705950 were identified, and the proportion of those with the T allele was low (minor allele frequency of 0.005 in control subjects). In addition, no individuals with the minor allele (A) of AKAP13 rs62025270 were detected.ConclusionConsideration of genetic polymorphisms has the potential to facilitate prediction not only of prognosis but also of AE in individuals with IIPs, providing a foundation for the development of personalised management strategies based on genetic profiles.

Endovascular treatment (EVT) for large ischemic stroke is associated with a risk of hemorrhagic transformation (HT). The severity of ischemic stress can be assessed using the apparent diffusion coefficient (ADC), with a high ADC indicating viability. This study aimed to examine whether a low ADC is associated with HT following EVT, based on a low Alberta Stroke Program Early Computed Tomography Score (ASPECTS) and diffusion-weighted imaging (DWI). This retrospective cohort study included consecutive patients with acute large ischemic stroke (ASPECTS-DWI ≤ 5) who underwent EVT with successful recanalization between April 2014 and March 2023. The most frequent ADC (peak ADC) in the ischemic core and DWI lesion volume were assessed. Patients with and without HT following EVT were compared to evaluate the associated between a low ADC and HT. Seventy-eight patients were enrolled, 25 (32%) of whom experienced HT after EVT. Parenchymal hematomas were observed in 17 patients (22%). Statistically significant differences in peak ADC and onset-to-recanalization time were observed between patients with and without HT (p = 0.0036 and p = 0.0151, respectively). In multivariate analysis, a low peak ADC was associated with HT (odds ratio [OR], 0.839; p = 0.018), but onset-to-recanalization time was not. A threshold peak ADC resulting in HT was calculated as < 480 × 10-6 mm2/s. A low peak ADC was associated with HT after EVT for a large ischemic core. The threshold of the peak ADC that resulted in HT was < 480 × 10-6 mm2/s. These results suggest that evaluating the ischemic core is necessary to confirm EVT.

The Japanese Society on Thrombosis and Hemostasis (JSTH) published the first-ever disseminated intravascular coagulation (DIC) guidelines in 2009. Fifteen years later, the JSTH developed new guidelines covering DIC associated with various underlying conditions. These guidelines were developed in accordance with the GRADE system to determine the strength of the recommendations and certainty of the evidence. This article was drafted as Part 1 of an overall DIC guideline covering various underlying conditions, with sepsis as the subject. In this section, seven key clinical issues (questions) are set. Question 1, regarding DIC diagnosis, introduces several diagnostic criteria, such as the JAAM-2, ISTH overt, SIC, and JSTH DIC criteria and recommends choosing the appropriate diagnostic criteria for DIC based on an understanding of their diagnostic properties. For pharmacotherapy in DIC patients with sepsis, we recommend the administration of antithrombin (Question 2) and recombinant thrombomodulin (Question 3) (both GRADE 1B). However, we do not make a clear recommendation regarding the administration of heparin (Question 6) and serine protease inhibitors (Question 7) because of the lack of evidence. Combination therapy, order of administration, and other administration methods for antithrombin and recombinant thrombomodulin are proposed as important future research questions (Questions 4 and 5).

A low-grade appendiceal mucinous neoplasm (LAMN) is a rare condition, occurring in 0.08-4.1% of appendectomy cases. Although this condition is frequently asymptomatic, it may occasionally result in acute complications, including torsion and rupture. We report the case of a 33-year-old male who presented with upper abdominal pain. Contrast-enhanced computed tomography (CECT) revealed a 3 cm cystic lesion in the appendix with 360° torsion at its base. Laparoscopic surgery confirmed the torsion, and the appendix was successfully resected without mucin spillage. Histopathological examination confirmed the diagnosis of LAMN. The postoperative course was uneventful, and the patient was discharged on postoperative day four. At 2.5 years follow-up, no recurrence was observed. This case highlights the rarity of LAMN torsion and the challenges in its preoperative diagnosis. The use of advanced imaging techniques, such as CECT, is crucial in identifying the torsion preoperatively, enabling timely surgical intervention. Furthermore, the successful laparoscopic resection without mucin spillage demonstrates the importance of meticulous surgical technique in preventing complications such as Pseudomyxoma peritonei (PMP), ensuring a favorable prognosis.

Porcelain gallbladder is a rare condition characterized by an extensive calcification of the gallbladder wall. It is associated with an increased risk of gallbladder cancer and elevated levels of tumor marker CA19-9, which is typically seen in pancreatic and biliary cancers. We present a case of a 60-year-old woman who presented with upper abdominal pain and nausea. Imaging revealed both a porcelain gallbladder and common bile duct stones. Initially, the patient underwent endoscopic retrograde cholangiopancreatography (ERCP) for the removal of the common bile duct stones. Notably, following the removal of the stones, the patient's CA19-9 levels were significantly elevated, reaching 6076 U/mL. Subsequently, she underwent a laparoscopic cholecystectomy. Histopathological examination and immunostaining for CA19-9 were performed on the resected specimen. Histopathology revealed marked fibrosis, calcification, and xanthogranulomatous inflammation of the gallbladder wall without evidence of malignancy. Immunostaining showed strong CA19-9 positivity in the inflamed gallbladder mucosal epithelium. Postoperatively, the patient's CA19-9 levels returned to normal (17 U/mL). This case highlights that a benign porcelain gallbladder can present with abnormally high CA19-9 levels, potentially mimicking malignancy. Reports of CA19-9 elevation in porcelain gallbladder are limited, and in most cases, significant CA19-9 elevation has been associated with malignancy. This case also demonstrates that benign conditions such as biliary obstruction, chronic inflammation, and xanthogranulomatous cholecystitis can cause significant elevations in CA19-9 levels. Therefore, careful differentiation and comprehensive evaluation are crucial for accurate diagnosis and appropriate management in such cases.

Introduction: The number of deceased organ donors remains very low (PMP 1.2) in Japan. The author, a neurosurgeon with extensive experience in deceased donors, has personally advocated for organ donation for eighteen years and observed the impact of official promotions on motivating emergency doctors. Methods: The author analyzed annual data of deceased donors and investigated the relationship between official promotion policies and the responses of emergency doctors, both positive and negative, towards donation. Also, the author has given lectures to the medical university students about the issues of organ donations and observed their responses. Results: Since fifteen years ago, a policy introducing the concept of terminal care to organ donation has been implemented by transplantation societies and the Japan Organ Transplantation Network. This policy asserted that donation was a human right, aimed at fulfilling the wishes of donors, and provided care for the grieving families. It emphasized that donation was fundamentally for the donors themselves, not the recipients. This policy was adopted by emergency medicine societies. Although there are 900 approved institutes for organ donation in Japan, only 10-20% of them actively participated, resulting in 120-150 donors annually. The remaining 80-90% showed no interest, yielding zero donors historically. Some leaders in emergency societies mentioned their lack of awareness or reluctance to engage in donation efforts, including education of medical students. The author concluded the following points: 1. Both proponents and opponents of donation failed to recognize the existence of awaiting recipients amidst emergency treatments. 2. The policy of terminal care or human rights motivated less than 20% of them, indicating its limitations. 3. Emotional responses among medical staff arose due to the policy’s focus on emotional aspects of medicine. Conversely, the author’s lectures emphasizing the professional obligation of medical staff to aid waiting recipients received positive responses, suggesting the effectiveness of a dry and professional approach in the long term. Conclusion: Motivating emergency doctors toward organ donation in Japan proves challenging. Future promotion activities should adopt dry and professional approaches rather than emotional ones.

要旨【目的】PECARN頭部外傷ルールは，clinically important traumatic brain injury（ciTBI）の低リスク群に頭部CT不要と判断するのに役立つ。しかし，中間リスク群に頭部CTを撮影するか，院内経過観察するかに関して臨床判断に迷うことがあり，ciTBIのリスク因子を探索した。【対象】国内6施設での多施設共同前方視的観察研究データベースの二次解析を行った。2016年6月から2017年9月に研究参加施設を受診した16歳未満の頭部外傷患者のうち，中間リスク群を対象とした。2歳未満と2歳以上に分けて，ciTBIとの関連を単変量ロジスティック回帰分析にて検討した。【結果】中間リスク群1,377例中，2歳未満の1.2%（7/607），2歳以上の0.65%（5/770）にciTBIを認めた。2歳未満での月齢［オッズ比（OR）0.74，95%信頼区間（CI）0.62–0.90］・2項目以上該当（OR 5.25，95% CI 1.15–23.9），2歳以上での前頭部以外の皮下血腫（OR 13.1，95% CI 1.3–640）がciTBIに関連した。【結語】PECARN中間リスク群において，2歳未満での低月齢・2項目以上該当，2歳以上での前頭部以外の皮下血腫のciTBI発生リスクが高い可能性が示唆された。

BackgroundEndovascular treatment of large ischemic cores is challenging. The severity of ischemic stress is assessed using the apparent diffusion coefficient (ADC). We aimed to evaluate the ADC in patients with a low Alberta Stroke Program Early CT [Computed Tomography] Score using diffusion‐weighted imaging and whether it correlates with clinical outcomes.MethodsThis study included consecutive patients with acute large ischemic stroke (Alberta Stroke Program Early CT Score‐diffusion‐weighted imaging ≤5) who underwent endovascular treatment with successful recanalization between April 2014 and March 2023. The most frequent ADC (peak ADC) and diffusion‐weighted imaging lesion volumes were assessed. The primary outcome was the 3‐month modified Rankin Scale (mRS) score. Good (mRS score, 0–3) and poor clinical outcomes (mRS score, 4–6) were compared to confirm whether ADC was associated with clinical outcomes.ResultsIn total, 78 patients were enrolled in this study; 30 had an mRS score of 0 to 3 at 3 months. The peak ADC in these patients was significantly higher than that in patients with mRS scores of 4 to 6 (P = 0.0002). In multivariate analysis, peak ADC was strongly associated with good clinical outcomes (odds ratio, 1.231; P = 0.0135) rather than onset‐to‐recanalization time and ischemic core volume. The optimal peak ADC threshold for discriminating between the mRS groups was 520×10−6 mm2/s with a sensitivity of 75% and a specificity of 73%. Good clinical outcomes were more frequently observed in patients with peak ADC ≥520×10−6 mm2/s (P<0.0001).ConclusionIn large ischemic cores, diffusion‐weighted imaging lesions with peak ADCs ≥520×10−6 mm2/s are associated with favorable outcomes. Evaluation of the ischemic core is necessary to confirm endovascular treatment.

This study assessed the concordance between line blot (LB) and immunoprecipitation (IP) assays for detecting myositis-specific antibodies (MSAs) in idiopathic inflammatory myopathies (IIMs) and their association with IIM subtypes. One hundred patients with IIM were enrolled, and MSA was detected using LB and IP. The IIM subtypes, including immune-mediated necrotizing myopathy-like, anti-tRNA synthetase syndrome-like, and clinically amyopathic dermatomyositis-like, were clinically diagnosed. The validity and reliability of the LB compared with the IP were evaluated. Optimal cutoff levels for LB were determined using various statistical methods including Cohen κ, Gwet's AC, diagnostic odds ratios, and receiver operating characteristic analysis. Line blot exhibited lower specificity and accuracy than IP in predicting IIM subtypes. Some MSAs performed better at higher LB cutoff values. Anti-signal recognition particle antibodies showed poor performance in predicting the immune-mediated necrotizing myopathy-like subtype using LB. Raising the cutoffs improved the reliability of anti-threonyl-tRNA synthetase and anti-signal recognition particle antibodies. Anti-histidyl-tRNA synthetase antibodies performed well at lower positivity, whereas diagnostic odds ratios increased for anti-transcription intermediary factor 1γ and anti-nuclear matrix protein 2 with higher cutoffs. Inconsistencies between LB and IP have been observed in patients with IIM. Individual optimal cutoffs for MSA by LB correlating with IP were determined. Rheumatologists should consider the differences between LB and IP results when classifying IIM subtypes.