Fukuoka Higashi Medical Center

PURPOSEGermline BRCA (gBRCA) mutations confer increased sensitivity to platinum-based chemotherapy and have been associated with improved survival across several malignancies. This study investigated the effect of gBRCA mutation status on the antitumor activity of modified fluorouracil, folinic acid, irinotecan, and oxaliplatin (FOLFIRINOX; mFFX) in patients with advanced pancreatic cancer (APC).METHODSThis multicenter retrospective study was performed in 178 patients with histologically confirmed APC who received mFFX, comprising 17 gBRCA-positive and 161 gBRCA-negative individuals. Efficacy outcomes, including progression-free survival (PFS), objective response rate (ORR), and normalization rate of carbohydrate antigen 19-9 (CA19-9), were compared between groups using univariate and multivariate analyses.RESULTSMedian PFS was significantly longer in gBRCA-positive than in gBRCA-negative patients (10.6 v 5.3 months, P = .005). Independent predictors of PFS included gBRCA mutation (hazard ratio [HR], 3.65), disease extent (HR, 2.34), and previous chemotherapy (HR, 1.66). gBRCA-positive patients demonstrated a significantly higher ORR (82% v 34%, odds ratio, 0.11, P < .001) and CA19-9 normalization rate (69% v 10%, P < 0.01). ORR was associated with gBRCA status (HR, 0.04), disease extent (HR, 0.11), and treatment history (HR, 0.27). Among 68 responders, sustained shrinkage of both primary and metastatic tumors and continued CA19-9 decline were observed during the first 6 months in gBRCA-positive patients, whereas responses in gBRCA-negative patients plateaued after 3 months. Subgroup analysis revealed that most patient subgroups demonstrated superior PFS and ORR in the gBRCA-positive cohort compared with those in the gBRCA-negative cohort.CONCLUSIONgBRCA mutation status was strongly associated with enhanced radiologic and biological responses to mFFX in APC. Routine screening for gBRCA mutations may provide essential guidance in therapeutic decision making and substantially improve clinical outcomes in this population.

Middle lobe torsion following pulmonary lobectomy is an uncommon but potentially life-threatening postoperative complication that poses significant challenges for perioperative anesthetic management. We report two cases of right middle lobe torsion after right upper lobectomy in which persistent postoperative atelectasis and equivocal initial bronchoscopic findings prompted repeated bronchoscopy and contrast-enhanced computed tomography examinations, leading to early diagnosis and timely surgical intervention. Anesthetic planning for reoperation focused on appropriate lung isolation with carefully selected double-lumen tubes, anticipation of airway mucosal edema, and vigilant hemodynamic monitoring when releasing lung torsion. In the first case, irreversible torsion and compromised perfusion necessitated middle lobectomy, whereas in the second case, preserved parenchymal enhancement permitted detorsion with lobar fixation. Both patients maintained acceptable oxygenation and stable hemodynamics during anesthesia, with no evidence of reperfusion-related instability. Postoperative management involved corticosteroid administration and non-invasive positive pressure ventilation to address airway secretions and recurrent atelectasis. These cases underscore the need for heightened clinical consideration of lobar torsion in patients with persistent atelectasis despite preserved oxygenation, systematic use of imaging modalities, and anesthetic strategies tailored to dynamic airway and vascular challenges. Awareness of lobar torsion's diagnostic subtleties and perioperative risks, coupled with close multidisciplinary coordination, are essential for optimizing perioperative outcomes in this rare but critical condition.

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.

Fragility hip fracture (FHF) has been frequently seen in trauma of older people, resulted in decreased mobility and a loss of independence. This study retrospectively assessed the long-term health-related quality of life (HRQOL) and living arrangement in older patients following FHF, and evaluated the factors associating with long-term EuroQol 5D (EQ5D) for 5years or more. This study included 203 Japanese patients (male 26, female 177) with FHF over the age of 60years. We examined the long-term EQ5D and life style at final follow-up, and identified the factors affecting with EQ5D. The mean age at baseline of 79years, the mean observation duration was 6years, and the average value of EQ5D at final follow-up was 0.65. Multivariate analysis presented that EQ5D at final follow-up was significantly predicted by serum albumin, trochanteric fracture, comorbidity stroke, higher BI at post-admission, less length of stay hospital, and living at home at final follow-up. The people living at home at final follow-up (148/203 [72.9%]) was less than the people at baseline (190/203 [93.6%]). In multivariate analysis, the people living at home at final follow-up were younger age at baseline, and higher BI at post-admission. In conclusion the long-term EQ5D after FHF was decreased in patients with lower serum albumin at baseline, with trochanteric fracture, with stroke comorbidity, and with lower BI at post-admission. The people living at home after FHF were young age at baseline and high BI at post-admission.

要旨86歳の女性。畑で複数のスズメバチに頭部5か所を刺傷され，40分後に当院へ救急搬送された。搬入時，顔面と体幹に膨疹を認めたが，アナフィラキシーの診断には至らなかった。血液検査で，APTT 55秒と軽度の延長を認めたため経過観察入院を行った。搬入16時間後の血液検査で，APTTは131秒とさらに延長していたので，ハチ毒による凝固障害と判断し，ハチ毒除去目的に血液濾過を施行した。その後もAPTT延長は増悪し，PT低下，CK，AST，ALTの上昇も認めたため，搬入36時間後に血漿交換を施行した。血漿交換を施行後は，凝固能，肝機能は改善し，CKも低下した。スズメバチの多数刺傷では，刺傷直後のアナフィラキシー以外に，ハチ毒による直接的作用で多臓器不全や血小板低下を伴わない凝固障害が生じ，10か所以上の刺傷で細胞傷害が出現することが多いと報告されている。本症例はわずか5か所の刺傷で細胞障害を来したが，これに先行したAPTTの異常は特徴的で，重症化のモニタリングとして有用である可能性が考えられた。

To enhance patient outcomes, we previously developed "Hibilog", an app that allows patients to report symptoms electronically. The paper-based Symptom Illustration Scale (SIS) was adapted using stickers and emojis to evaluate patient-reported outcomes (PROs). This study aimed to validate SIS within an electronic PRO monitoring environment for metastatic breast cancer patients undergoing chemotherapy. The patients used the Electronic Patient-Reported Outcomes Monitoring (ePROM) "Hibilog" application to answer a questionnaire consisting of 18 items selected from the Patient-Reported Outcome-Common Terminology Criteria for Adverse Events (PRO-CTCAE), focusing on symptoms related to breast cancer treatment, along with the corresponding SIS questionnaire. Symptom monitoring began upon registration and continued every two weeks until the completion of the study. The primary outcome was the criterion-related validity of the SIS against PRO-CTCA using the ePROM. The secondary endpoints included the response rate, response time, and missing rates for each item. Patients (n = 75) were registered between September 2019 and March 2020. For criterion validity, the Spearman rank correlation coefficients between the PRO-CTCAE and SIS items showed high correlations (rs ≥ 0.41) for all 18 items. The κ correlation coefficient indicated a high correlation (κ > 0.41) in 11 of the 18 items (61.1%), unlike the correlation with continuous variables. In terms of response and missing rates, the SIS in ePROM demonstrated similarly high performance as our results. Additionally, the average response time was 3.0 min (SD 4.2) for SIS, with a substantially shorter response time. We conclude that SIS is a useful tool in an ePROM environment for patients with MBC undergoing chemotherapy. The clinical utility of SIS in an ePRO environment needs to be validated to develop a more accurate scale for capturing patient symptoms.

BackgroundGastrointestinal neuroendocrine tumors (GI-NETs) are slow-growing tumors with the potential for malignancy that originate from neuroendocrine cells. Therefore, early diagnosis and treatment of GI-NETs are necessary to prevent metastasis. The widespread use of colonoscopy, which allows early detection of rectal neuroendocrine tumors (rNETs) that are small enough to be treated endoscopically, has resulted in an increasing rate of endoscopic resection of rNETs. However, whether the long-term prognosis of endoscopically resected rNETs is favorable has not yet been determined. This study aimed to assess whether endoscopically resected rNETs affect the long-term prognosis of patients.MethodsWe retrospectively reviewed the medical records of 163 consecutive patients with rNETs who underwent endoscopic resection at 11 hospitals in Japan between 1999 and 2012. The primary analysis focused on 47 patients with 51 rNETs who underwent ≥ 10 years of follow-up. The secondary analysis focused on patients who underwent less than 10 years of follow-up.ResultsThe median follow-up period of patients included in the primary analysis was 12.3 years (range, 10–19.1 years). The median lesion size was 5 mm (range, 2–12). Three lesions were treated using conventional endoscopic mucosal resection (EMR). Twenty-nine lesions were treated using modified EMR. Nineteen lesions were treated using endoscopic submucosal dissection. The R1 resection rate and lymphovascular invasion rate were 15.7% and 25.5%, respectively. The curative resection (CR) rate and non-CR rate were 66.7% and 33.3%, respectively. Two patients with lesions treated with non-CR underwent radical surgery. None of the 47 patients experienced lesion recurrence during the 10-year follow-up period. Two patients whose lesions were treated with CR died of other diseases.ConclusionsDeath attributable to rNETs did not occur among patients who underwent at least 10 years of follow-up after endoscopic resection.

【背景および目的】近年摂食障害患者は増加傾向にあり，専門ではない一般小児科医も診療の機会が増えている．腎機能障害は合併症のひとつであるが，小児領域での頻度と予後は不明である．【方法】二次医療機関である当院を受診した摂食障害患者15名を対象とし，腎機能障害の頻度および，患者背景や検査値，腎機能回復までの期間を調査した．【結果】診断時年齢の中央値は9.0歳（5.4～15.2）であり，病型は回避・制限性食物摂食症が10名（66.7%）を占めた．入院時の推定糸球体濾過量の中央値は91.8（57.4～123.4）mL/min/1.73m2であり，腎機能低下を示したのは，15名中7名（46.7%）だった．腎機能低下例で，腎機能が正常化するまでに要した期間の中央値は88日（34～114）だった．【結論】小児においても摂食障害患者で腎機能障害は高頻度でみられる合併症であり，長期的なフォローアップが必要である．