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Previous reports focusing on cefiderocol susceptibility against Stenotrophomonas maltophilia have included a large number of noninvasive or colonized isolates, and data focusing on invasive S. maltophilia strains are still lacking. We retrospectively investigated the cefiderocol susceptibility of stored S. maltophilia strains that caused bacteremia at two Japanese hospitals. The MIC 50 and MIC 90 were 0.06 μg/mL and 0.25 μg/mL, respectively, while the susceptibility rate was 99.3% (current CLSI breakpoint criteria). Our results provide the MIC distribution of bacteremic S. maltophilia isolates in Japan and show the preserved cefiderocol susceptibility of S. maltophilia among clinically invasive pathogenic strains., Competing Interests: Declarations. Ethical approval: Not applicable. This article does not contain any studies with human participants or animals performed by any of the authors. Consent to publish: The authors consent to the publication of the manuscript. Competing interests: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Cutis laxa constitutes a diverse group of connective tissue diseases, both inherited and acquired, characterized by loose skin and varying systemic involvement, including pulmonary lesions. While cutis laxa has been linked to conditions like emphysema, asthma, and bronchiectasis, the specific pathological and radiological characteristics underlying pulmonary complications related to cutis laxa remain unclear., Case Presentation: A 36-year-old woman, diagnosed with cutis laxa at birth, presented to our outpatient clinic with severe obstructive ventilatory impairment, evident in pulmonary function tests (expiratory volume in one second (FEV 1 )/forced vital capacity (FVC): 34.85%; %residual volume [RV]: 186.5%; %total lung capacity [TLC]: 129.2%). Pulmonary function tests also indicated small airway disease (%FEF50%, 7.9%; %FEF75%, 5.7%; and %FEF25-75%, 6.8%). Computed tomography (CT) revealed the lack of normal increase in lung attenuation on expiratory CT scan, with no discernible emphysematous changes. Exome sequencing was performed to confirm the association between the pulmonary lesions and cutis laxa, revealing a frameshift variant in exon 30 of the elastin gene (ELN). Further analysis employing a parametric response map revealed a longitudinal increase in the percentage of functional small airway disease (fSAD) from 37.84% to 46.61% over the 8-year follow-up, despite the absence of overt changes in CT findings, specifically the lack of normal increase in lung attenuation on expiratory CT scan. Over the same follow-up interval, there was a modest reduction of 25.6 mL/year in FEV 1 coupled with a significant increase in %RV. Pulmonary function test metrics, reflective of small airway disease, exhibited a continual decline; specifically, %FEF50%, %FEF75%, and %FEF25-75% diminished from 7.9% to 7.0%, 5.7% to 4.6%, and 6.8% to 5.4%, respectively., Conclusions: This case highlighted an instance of autosomal dominant cutis laxa arising from a frameshift variant in exon 30 of ELN, accompanied by small airway disease. Comprehensive investigation, utilizing quantitative CT analysis, revealed a longitudinal increase in fSAD percentage with a mild reduction in FEV 1 . These findings indicate that elastin deficiency may not only diminish elastic fibers in the skin but also be implicated in small airway disease by impacting components of the extracellular matrix in the lungs., (© 2024. The Author(s).)

The site of enterohepatic Helicobacter colonization/infection in humans is still unknown. We report microbiologically and histopathologically confirmed H. fennelliae localization in the large intestine in an immunocompromised patient in Japan. This case contributes to better understanding of the life cycle of enterohepatic Helicobacter species.

Background: Liver cirrhosis is the irreversible fibrosis of the liver and causes refractory ascites and hepatic encephalopathy, which might not respond to treatment. Living donor liver transplantation (LDLT) is an effective treatment for patients with cirrhosis. However, post-LDLT patients are prone to muscle atrophy and sarcopenia. Therefore, physiotherapy of post-LDLT patients is essential for preventing the progression of sarcopenia. Recently, rehabilitation using neuromuscular electrical stimulation (NMES) has been reported to be useful for preventing the progression of sarcopenia. Similarly, nutrition therapy is essential for post-LDLT patients because these patients frequently experience malnutrition. However, the effects of combined NMES and nutrition therapy on post-LDLT patients remain unknown., Methods/design: This open-label, randomized, parallel-group study will compare the effects of combined therapy with NMES and branched-chain amino acids (BCAA) with those of NMES alone in patients with decompensated cirrhosis after LDLT. After LDLT, 50 patients with decompensated cirrhosis will be randomly assigned to receive NMES with BCAA or NMES without BCAA. The duration of the intervention will be 3 months. To analyze the change in skeletal muscle mass, InBody 770 body composition and body water analysis and ultrasonography will be performed before LDLT and 4 weeks and 12 weeks post-LDLT. The primary endpoint is changes in the skeletal muscle mass from baseline to 3 months. Important secondary endpoints are the changes in the skeletal muscle mass from baseline to 1 month and changes in the quadriceps strength from baseline to 1 month., Discussion: The results of this study are expected to provide evidence regarding the effect of NMES combined with BCAA therapy on the skeletal muscle of post-LDLT patients., Trial Registration: Japan Registry of Clinical Research jRCTs071190051 . Registered on February 26, 2020.

Background: Triple combination therapy involving long-acting muscarinic antagonists long-acting β2 agonists, and inhaled corticosteroids has recently become an option for maintenance treatment of COPD. Some add-on clinical trials have reported the benefits of these combinations. However, the process to step up to triple therapy varies for individual cases., Methods: Keio University and affiliated hospitals conducted an observational COPD cohort study, recruiting patients diagnosed as having COPD by pulmonary physicians and those referred for investigation of possible COPD. Their prescription history and clinical course were retrospectively analyzed based on the physicians' medical records and patient questionnaires. This study was registered with UMIN (UMIN000003470, April 10, 2010)., Results: A total of 95 of the 445 COPD patients (21%) were treated with inhaled corticosteroids/long-acting β2 agonists/long-acting muscarinic antagonists as maintenance therapy, including 12 in COPD Grade I, 31 in Grade II, 38 in Grade III, and 14 in Grade IV, based on the Global Initiative for Chronic Obstructive Lung Disease spirometric grading. For more than half of the patients on triple therapy, the treatment had been intensified due to unsatisfactory improvement of symptoms, and 32% were treated with triple therapy due to comorbid asthma. In contrast, there were COPD patients whose therapy was maintained after starting with triple therapy because of their serious conditions or concurrent exacerbation at diagnosis (8%)., Conclusion: Triple therapy was often prescribed in the real-life management of COPD, even in patients whose airflow limitation was not severe. To better control symptoms was the major reason for choosing triple therapy, regardless of the severity of COPD, in Japan.

Background and Objective: Osteoporosis is an important systemic comorbidity of chronic obstructive pulmonary disease (COPD). However, neither its mechanisms nor its risk factors have been fully elucidated. With regard to genetic factors, low-density lipoprotein receptor-related protein 5 (LRP5) A1330V is known to be associated with osteoporosis in the general population, but the influence of this polymorphism in COPD is unknown. The aim of this study was to investigate the potential risk factors of COPD-related bone loss and fracture., Methods: Keio University and affiliated hospitals have enrolled an observational cohort to investigate the management of COPD comorbidities. To assess risk factors for osteopenia and osteoporosis, bone mineral density (BMD) of the hip and lumbar spine, presence of vertebral fracture, quantitative data on emphysema and airway wall on computed tomography, as well as LRP5 genotype were analysed in patients with or at risk for COPD (n = 270)., Results: The percentage of subjects with osteoporosis (T-score ≤ -2.5), osteopenia (T-score between -1 and -2.5) and a normal BMD (T-score ≥ -1) was 15.2%, 35.9% and 48.9%, respectively. T-score was significantly decreased in subjects with LRP5 TT genotype (n = 15) compared with that in those with CC/CT genotype (n = 255) (-1.83 vs. -0.98, P = 0.0167). On multivariate logistic regression analysis, female gender (odds ratio (OR) 10.4; P < 0.0001), severe emphysema (OR 2.3; P = 0.013) and LRP5 TT genotype (OR 3.7; P = 0.031) independently increased the risk of osteopenia/osteoporosis., Conclusions: This study confirmed the complex pathophysiology of COPD-related osteoporosis, including the influence of gender, clinical phenotype and genetic factors., (© 2014 Asian Pacific Society of Respirology.)

Introduction: Obturator hernia is an important cause of small bowel obstruction and is associated with difficult diagnosis and high mortality., Materials and Methods: We reviewed 22 cases of intestinal obstruction due to obturator hernia that necessitated surgical intervention over the last 17 years and evaluated the changes in the number of patients based on the census data., Results: All cases were elderly females. The Howship-Romberg sign was present in 15 patients (68%). Computed tomography (CT) correctly diagnosed 13/15 (87%) of cases in which it was performed prospectively. The median time to surgery was 7.5+/-5.2 days for those patients with bowel resection, compared to 0.8+/-1.3 days for those without (P <0.05). The number of patients with obturator hernia has increased with an ageing society., Conclusions: The rising incidence of obturator hernia is probably linked to an ageing society. CT of the pelvis was helpful in obtaining the correct diagnosis. The shorter the delay between admission and surgery, the lower the bowel resection rate. Laparotomy at an early stage is recommended in such patients as it leads to reduced morbidity and mortality.

From 1965 to 1985, 1,150 patients underwent gastrectomy for carcinoma of the stomach invading beyond the submucosa. One thousand, one hundred and forty-one patients were studied for five years or longer. The patients were classified into two groups--those treated from 1965 to 1974 (n = 622) and those treated from 1975 to 1985 (n = 519). In the more recent group, there was a significant increase in the number of patients more than 70 years of age, in the number of female patients, of carcinomas present in the upper two-thirds of the stomach, in tumors exceeding 10 centimeters and diffusely infiltrative type and in early cancer-simulating type of advanced carcinoma in gross appearance and in undifferentiated type adenocarcinomas in histology. Although differences in the incidence of patients treated by radical procedures, such as extensive lymph node dissection and combined resection of adjacent organs, were not statistically significant, the incidence for those who underwent total gastrectomy was significantly increased. The over-all long term survival rate remained unchanged during 1965 to 1985 (five year survival rate of 31.3 versus 32.8 per cent). However, there was a significantly longer survival period for patients who underwent total gastrectomy (five year survival rate of 22.9 versus 28.3 per cent) (p less than 0.05). Much of the improvement can be attributed to an increase in the detection of relatively small advanced carcinomas and of advanced carcinomas of the stomach simulating early carcinoma in the upper one-third of the stomach. Therefore, we emphasize the need to diagnose advanced carcinoma of the stomach when the lesion is in an earlier stage of disease.