Loeys-Dietz syndrome (LDS) is a rare autosomal dominant disorder whose clinical phenotype overlaps with that of Marfan syndrome (MFS) and vascular Ehlers-Danlos syndrome (vEDS), including aortic and/or arterial aneurysms, skeletal abnormalities, and spontaneous pneumothorax. Although the pathological features of MFS- and vEDS-associated pulmonary lesions have been described, the pulmonary pathology of LDS remains virtually unknown. Herein, we report the detailed histopathological features of LDS-associated pulmonary vascular abnormalities and pneumothorax. A 32-year-old Japanese woman underwent pulmonary surgery for spontaneous pneumothorax. She had undergone aortic valve surgery 7 years earlier, and genetic testing identified a heterozygous germline missense variant in TGFBR2 (c.1150 A > C, p.Asn384His). Histologically, the resected lung showed a ruptured bulla/bleb. Localized distal acinar emphysema was present in continuity with the bullous lesion. In the remaining parenchyma, the alveolar septa exhibited a distinctive proliferation of irregularly dilated and tortuous capillaries, reminiscent of pulmonary capillary hemangiomatosis. Hemosiderin-laden macrophages were conspicuous in some air spaces. In addition, several pulmonary muscular arteries and veins showed irregular dilatation and tortuosity, with elastic fibers that were fragmented and disorganized. Aberrant TGF-β signaling, together with deranged matrix formation, may underlie both the dilated, tortuous vasculature and the emphysematous/bullous changes leading to pneumothorax in the LDS lung.
BACKGROUND AND AIMS:Calcified coronary plaques frequently harbor lipid-rich components, yet identifying residual lipid burden within calcification remains challenging. This study aimed to evaluate the ability of optical coherence tomography (OCT) to identify lipidic calcification, utilizing near-infrared spectroscopy intravascular ultrasound (NIRS-IVUS) as the reference standard. METHODS:We retrospectively analyzed patients undergoing PCI for de novo calcified lesions (IVUS arc ≥90°) assessed by both NIRS-IVUS and OCT. Lipidic calcification was defined as a lesion with a maximum lipid core burden index in 4 mm (maxLCBI4mm) ≥400, reflecting lesion-level lipid burden within a calcified plaque segment, rather than lipid strictly co-localized with calcium. On OCT, the "attenuated calcium index" was calculated as the mean angle of calcification exhibiting an invisible trailing edge across the 20-mm lesion segment. We compared clinical and imaging characteristics between vessels with and without lipidic calcification. RESULTS:Among 102 patients, 38 lesions (37.3%) exhibited lipidic calcification. While clinical demographics were comparable between groups, OCT analysis revealed significantly greater lipid length, attenuated calcium index, and prevalence of cholesterol crystals in the lipidic calcification group. Multivariable analysis identified the attenuated calcium index (OR 1.03; 95% CI 1.01-1.05) and cholesterol crystals (OR 7.50; 95% CI 2.68-20.9) as independent predictors of lipidic calcification. Furthermore, among calcified nodules, eruptive nodules demonstrated a significantly higher maxLCBI4mm than non-eruptive ones (401 vs. 208; p = 0.035). CONCLUSIONS:The OCT-derived attenuated calcium index and cholesterol crystals are distinct markers associated with lipidic calcification. Additionally, eruptive calcified nodules appear to harbor a greater lipid burden. OCT facilitates the identification of lipid-rich phenotypes even within complex calcified lesions.
To date, no study has focused exclusively on D3 lymph node dissection for mid-transverse colon cancer. The present study compared surgical and oncological results following extended right hemicolectomy (exRHC) and transverse colectomy (TC) with standardized D3 lymphadenectomy in patients with locally advanced mid-transverse colon cancer. Patients with stage II or III mid-transverse colon cancer who underwent D3 lymphadenectomy with central ligation of the middle colic artery (MCA) between January 2013 and December 2022 were divided into two groups by surgical procedure. We compared patient short- and long-term outcomes between the groups. Eighty-three patients (exRHC, n = 56; TC, n = 27) were analyzed. Overall complication rates (35.7
PURPOSE:To investigate the safety and efficacy of mini-endoscopic combined intrarenal surgery (ECIRS) in older patients with renal and ureteral stones. METHODS:Consecutive patients with renal or ureteral stones who underwent mini-ECIRS were retrospectively analyzed at three Japanese tertiary care institutions between 2015 and 2021. Data on patient backgrounds, stone characteristics, and postoperative indications were collected and evaluated. After matching preoperative and intraoperative factors in older patients and other groups, postoperative factors were assessed using univariate analysis. RESULTS:The final analysis included data from 1303 single-session mini-ECIRS of 1432 cases collected. The patients were divided into two groups: those aged > 75 and those aged < 75 years, with 121 and 1182 patients. From each group, 112 cases matched for eight factors, performance status, body mass index, sex, hydronephrosis, percutaneous nephrostomy, pyuria, preoperative urinary tract infection, and preoperative ureteral stenting, were selected. The univariate analysis was performed on postoperative factors between the groups of matched older and matched non-older patients, and there was no significant difference in any factor, including postoperative hospitalization duration, complications, and stone-free rate (SFR). CONCLUSIONS:To the best of our knowledge, this multicenter cohort study is the first to compare the efficacy and safety of the mini-ECIRS between older patients and other groups. It was discovered that surgery could be performed with a similar quality even in patients aged 75 and older compared to that in other groups.
Aim:Femoral artery sheath insertion is a common resuscitation procedure; however, most research on complications focuses on cardiovascular medicine. No studies have examined sheath insertion complications in emergency or intensive care settings where fluoroscopy is unavailable. This study investigated complications associated with femoral artery sheath insertion in non-fluoroscopic resuscitation. Methods:This retrospective, single-center observational study included patients who underwent femoral artery sheath insertion for resuscitation under non-fluoroscopic conditions between April 2014 and August 2024 and subsequently underwent computed tomography. Patients were categorized into the non-complication and complication groups. Vascular and skin insertion positions were measured using computed tomography, and patient backgrounds were compared between groups. Results:The study included 151 patients in the non-complication group and 16 in the complication group, yielding a complication rate of 9.6%. A significant difference was observed in sheath insertion position (p < 0.001). The proportion of sheath insertions at the level of the femoral head was higher in the non-complication group, whereas insertions below the femoral head were more frequent in the complication group. Conclusion:Femoral artery sheath insertion without fluoroscopy during resuscitation was associated with a 9.6% complication rate, with the complication group having a higher rate of vascular puncture below the femoral head than the non-complication group.
