Powder diffraction patterns of the zeolite mesolite ($Na_{5.33}Ca_{5.33}Al_{16}Si_{24}O_{80}{\cdot}21.33H_2O$), with a natrolite framework topology were measured as a function of pressure up to 5.0 GPa using a diamond-anvil cell and a $200{\mu}m$-focused monochromatic synchrotron X-ray. Under the hydrostatic conditions mediated by pore-penetrating alcohol and water mixture, the elastic behavior of mesolite is characterized by continuous volume expansion between ca. 0.5 and 1.5 GPa, which results from expansion in the ab-plane and contraction along the c-axis. Subsequent to this anomalous behavior, changes in the powder diffraction patterns suggest possible reentrant order-disorder transition. The ordered layers of sodium- and calcium-containing channels in a 1:2 ratio along the b-axis attribute to the $3b_{natrolite}$ cell below 1.5 GPa. When the volume expansion is completed above 1.5 GPa, such characteristic ordering reflections disappear and the $b_{natrolite}$ cell persists with marginal volume contraction up to ca. 2.5 GPa. Further increase in pressure leads to progressive volume contraction and appears to generate another set of superlattice reflections in the $3c_{natrolite}$ cell. This suggests that mesolite in the pressure-induced hydration state experiences order-disorder-order transition involving the motions of sodium and calcium cations either through cross-channel diffusion or within the respective channels.
CD47 (integrin-associated protein), a multi-spanning transmembrane protein expressed in all cells including red blood cells (RBCs) and leukocytes, interacts with signal regulatory protein ${\alpha}$ ($SIRP{\alpha}$) on macrophages and thereby inhibits phagocytosis of RBCs. Recently, we generated a novel C57BL/6J CD47 knockout ($CD47^{-/-}$ hereafter) mouse line by employing a CRISPR/Cas9 system at Center for Mouse Models of Human Disease, and here report their hematological phenotypes. On monitoring their birth and development, $CD47^{-/-}$ mice were born viable with a natural male-to-female sex ratio and normally developed from birth through puberty to adulthood without noticeable changes in growth, food/water intake compared to their age and sex-matched wild-type littermates up to 26 weeks. Hematological analysis revealed a mild but significant reduction of RBC counts and hemoglobin in 16 week-old male $CD47^{-/-}$ mice which were aggravated at the age of 26 weeks with increased reticulocyte counts and mean corpuscular volume (MCV), suggesting hemolytic anemia. Interestingly, anemia in female $CD47^{-/-}$ mice became evident at 26 weeks, but splenomegaly was identified in both genders of $CD47^{-/-}$ mice from the age of 16 weeks, consistent with development of hemolytic anemia. Additionally, helper and cytotoxic T cell populations were considerably reduced in the spleen, but not in thymus, of $CD47^{-/-}$ mice, suggesting a crucial role of CD47 in proliferation of T cells. Collectively, these findings indicate that our $CD47^{-/-}$ mice have progressive hemolytic anemia and splenic depletion of mature T cell populations and therefore may be useful as an in vivo model to study the function of CD47.
Parkinson's disease (PD) is a progressive neurodegenerative disease that mainly affects motor system with clinical features such as bradykinesia, rigidity, tremor and abnormal posture. PD is characterized by the death of dopaminergic neurons in the substantia nigra pars compacta, which is associated with accumulation of oxidative stress and dysregulation of intracellular signaling pathway. Quercetin-3-O-glucuronide (Q3GA), a major metabolite of quercetin, has been reported to have neuroprotective effects. In this study, we examined the neuroprotective effect of Q3GA against 1-methyl-4-phenyl pyridinium ($MPP^+$)-induced neurotoxicity of PD and the underlying molecular mechanisms in SH-SY5Y cells. MTT and LDH assay showed that Q3GA significantly decreased $MPP^+$-induced cell death, which is accompanied by a reduction in poly (ADP-ribose) polymerase (PARP) cleavage. Furthermore, it attenuated $MPP^+$-induced intracellular reactive oxygen species (ROS) with the reduction of Bax/ Bcl-2 ratio. Moreover, Q3GA significantly increased the phosphorylation of Akt and cAMP response element binding protein (CREB), but it has no effects on the phosphorylation of extracellular signal-regulated kinase (ERK). Taken together, these results demonstrate that Q3GA significantly attenuates $MPP^+$-induced neurotoxicity through ROS reduction and Akt/CREB signaling pathway in SH-SY5Y cells. Our findings suggest that Q3GA might be one of the potential candidates for the prevention and/or treatment of PD.
Charcot-Marie-Tooth disease (CMT) is a slowly progressive hereditary degenerative disease and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated disorder characterized by weakness and sensory deficits. The purpose of this study was to analyze and compare the electrophysiological characteristics observed in sensory nerve conduction studies (SNCS) of both diseases. A retrospective study of 65 patients with a diagnosis of CIDP (N=35) and CMT type I (N=30) was performed. This study analyzed No potentials ratio, distal compound nerve action potential (dCNAP) of various nerve types, and a correlation coefficient analysis of the sensory nerve conduction velocity (SNCV). As a result, I found that CMT 1 was more severe systemic demyelinating and axonal polyneuropathy better than CIDP (P<0.05). In a quantitative analysis of dCNAP and SNCV, especially sural nerve was the most severe nerve injury observed in both diseases. In correlation and scatter plot analysis, CMT 1 showed relatively high correlations compared to CIDP based on the correlation coefficient analysis (Fisher's Z test) of SNCV. The results of this study suggested that CMT 1 showed the slowness in SNCV, one of the characteristics of demyelinating polyneuropathy, and this slowing had a uniform pattern. In conclusion, electrophysiological characteristic of SNCS may be useful in the diagnosis and research between patients with CMT 1 and CIDP.
Park, So-Jung;Hong, Sang-Bum;Lim, Chae-Man;Koh, Youn-Suck;Huh, Jin-Won
Quality Improvement in Health Care
/
v.27
no.2
/
pp.18-29
/
2021
Purpose: Patients with hematologic malignancy (HM) typically have a high mortality rate when their condition deteriorates. The chronic progressive course of the disease makes it difficult to assess the effect of intervention on acute events. We investigated the effectiveness of a rapid response team (RRT) on in-hospital mortality in patients with HM. Methods: We retrospectively analyzed the data of patients with HM who admitted to the medical intensive care unit between 2006 and 2015. Clinical outcomes before and after RRT implementation were evaluated. Results: A total of 228 patients in the pre-RRT period and 781 patients in the post-RRT period were included. The overall in-hospital mortality was 55.4%. Patients in the post-RRT period had improved survival; however, they required more vasopressor therapy, continuous renal replacement therapy, and extracorporeal membrane oxygenation. Multivariate analysis revealed that in-hospital mortality was associated with RRT activation (hazard ratio [HR], 0.634; 95% confidence interval [CI], 0.498-0.807; p < .001), neurological disease (HR, 2.007; 95% CI, 1.439-2.800; p < .001), sequential organ failure assessment score (HR, 1.085; 95% CI, 1.057-1.112; p < .001), need for continuous renal replacement therapy (HR, 1.608; 95% CI, 1.206-1.895; p< .001), mechanical ventilation (HR, 1.512; 95% CI, 1.206-1.895; p< .001), vasopressor (HR, 1.598; 95% CI, 1.105-2.311; p = .013), and extracorporeal membrane oxygenation (HR, 1.728; 95% CI, 1.105-2.311; p = .030). Conclusion: RRT activation may be associated with improved survival in patients with HM.
Kyung Joon Jo;SeongHee Ho;Yun Jeong Hong;Jee Hyang Jeong;SangYun Kim;Min Jeong Wang;Seong Hye Choi;SeungHyun Han;Dong Won Yang;Kee Hyung Park
Dementia and Neurocognitive Disorders
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v.23
no.1
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pp.22-29
/
2024
Background and Purpose: Alzheimer's disease (AD) is a neurodegenerative disease characterized by a progressive decline in cognition and performance of daily activities. Recent studies have attempted to establish the relationship between AD and sleep. It is believed that patients with AD pathology show altered sleep characteristics years before clinical symptoms appear. This study evaluated the differences in sleep characteristics between cognitively asymptomatic patients with and without some amyloid burden. Methods: Sleep characteristics of 76 subjects aged 60 years or older who were diagnosed with subjective cognitive decline (SCD) but not mild cognitive impairment (MCI) or AD were measured using Fitbit® Alta HR, a wristwatch-shaped wearable device. Amyloid deposition was evaluated using brain amyloid plaque load (BAPL) and global standardized uptake value ratio (SUVR) from fluorine-18 florbetaben positron emission tomography. Each component of measured sleep characteristics was analyzed for statistically significant differences between the amyloid-positive group and the amyloid-negative group. Results: Of the 76 subjects included in this study, 49 (64.5%) were female. The average age of the subjects was 70.72±6.09 years when the study started. 15 subjects were classified as amyloid-positive based on BAPL. The average global SUVR was 1.598±0.263 in the amyloid-positive group and 1.187±0.100 in the amyloid-negative group. Time spent in slow-wave sleep (SWS) was significantly lower in the amyloid-positive group (39.4±13.1 minutes) than in the amyloid-negative group (49.5±13.1 minutes) (p=0.009). Conclusions: This study showed that SWS is different between the elderly SCD population with and without amyloid positivity. How SWS affects AD pathology requires further research.
Background : An acceleration or deceleration of the heart rate (HR), which reflects autonomic effects, is observed before the onset of paroxysmal atrial fibrillation (PAF). The purpose of this study was to assess the discrepancy in the autonomic interactions before the onset of PAF for different patterns of change in the HR. Materials and Methods : From 105 Holter tapes with the PAF recorded, 55 episodes (42 patients, 34 men, $58{\pm}12$ years) of PAF (>5 min), preceded by a sinus rhythm for more than 1 hour, were selected and submitted to time-domain and frequency-domain HR variability analyses. Fifty-five episodes were divided into 2 groups: group A PAF (n=30) with acceleration of the HR during the last 2 minutes before the PAF and group B (n=25) with deceleration of the HR. Results : A significant linear decrease in the mean R-R interval was observed in group A ($924{\pm}30$ to $835{\pm}28ms$, $P$=0.001) and an increase from $831{\pm}32$ to $866{\pm}31ms$ in group B PAF episodes ($P$=0.046). In the frequency-domain analyses, the LF/HF ratio exhibited a progressive linear increase before the PAF in group A ($P$=0.005). The HF normalized units (HFnu) and natural logarithm-transformed HF ($In$HF) values decreased from $30.8{\pm}4.0$ to $16.1{\pm}1.8$ ($P$=0.003) and $4.49{\pm}0.25$ to $4.07{\pm}0.22$ ($P$=0.001), respectively. Contrary to the results in group A, a significant increase in the HF components (HFnu and $In$HF) (from $22.6{\pm}3.2$ to $30.2{\pm}4.0$, $P$=0.005, and $4.27{\pm}0.27$ to 4.75 0.33, $P$=0.001, respectively) and a resultant decrease in the LF/HF ratio were observed in group B PAF episodes. No significant changes were observed in the LF components in either PAF group. Conclusion : Autonomic stimuli leading to an acceleration or deceleration of the HR before the onset of AF are due to parasympathetic modulation. Parasympathetic modulation plays a key role in the initiation of PAF.
Kim In-Ah;Choi Ihl-Bhong;Jang Ji-Young;Kang Ki-Mun;Jho Seung-Ho;Kim Hyung-Tae;Lee Kyung-Jin;Choi Chang-Rak
Korean Journal of Head & Neck Oncology
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v.14
no.2
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pp.156-163
/
1998
Background & Objectives: Frameless fractionated stereotactic radiotherapy(FFSRT) is a modification of stereotactic radiosurgery(SRS) with radiobiologic advantage of fractionation without losing mechanical accuracy of SRS. Local recurrence of head and neck cancer at or near skull base benefit from reirradiation. Main barrier to successful palliation is dose limitation secondary to normal tissue tolerance. We try to evaluate the efficacy and safety of FFSRT as a new modality of reirradaton in these challenging patients. Materials & Methods: Seven patients with recurrent head & neck cancer involving at or near skull base received FFSRT from September 1995 to November 1997. Six patients with nasopharyngeal cancer had received induction chemotherapy and curative radiation therapy. One patient with maxillary sinus cancer had received total maxillectomy and postoperative radiation therapy as a initial treatment. Follow-up ranged from 11 to 32 months with median of 24 months. Three of 7 patients received hyperfractionated radiation therapy(1.1-1.2Gy/fraction, bid, total 19.8-24Gy) just before FFSRT. All patients received FFSRT(3-5Gy/fraction, total 15-30Gy/5-10fractions). Chemotherapy(cis-platin $100mg/m^2$) were given concurrently with FFSRT in four patients. Second course of FFSRT were given in 4 patients with progression or recurrence after initial FFSRT. Because IF(irregularity factor; ratio of surface area of target to the surface area of sphere with same volume as a target) is too big to use conventional stereotactic RT using multiple arc method for protection of radiation damage to critical normal tissue, all patients received FFSRT with conformal method using irregular static ports. Results: Five of 7 patients showed complete remission in follow-up CT &/or MRI. Three of these five patients who developed marginal, in-field, and out-field recurrences, respectively. Another one of complete responders has been dead of G-I bleeding without evidence of local recurrence. One partial responder who showed progressive disease 15 months after initial FFSRT has received additional FFSRT, and then he is well-being with symptomatic improvement. One minmal responder who showed progression of locoregional disease 9 months after $1^{st}$ FFSRT has received 2nd FFSRT, and then he is alive with stable disease. Five of 7 case had showed direct invasion to skull base and had complaint headache and various symptoms of cranial nerve involvement. Four of these five case showed improvement of neurologic symptoms after FFSRT. No significant neurologic complicaltion related to FFSRT was observed during follow-up periods. Tumor volumes were ranged from 3.9 to 50.7 cc and surface area ranged from 16.1 to $114.9cm^2$. IF ranged from 1.21 to 1.74. The average ratio of volume of prescription isodose shell to target volume was 1.02 that indicated the improvement of target coverage and dose distribution with FFSRT with conformal method compared to target coverage with FFSRT with multiple arc method. Conclusion: Our initial experience suggests that FFSRT with conformal method was relatively effective and safe modality in the treatment of recurrent head and neck cancer involving at or near skull base. Treatment benefit included good palliation of symptoms and reasonable radiographic response. However, more experience and additional follow-up are needed to better assess its ultimate role in treating these challenging patients.
Background : Idiopathic pulmonary fibrosis (IPF) is a fatal progressive fibrous disease of the lung of unknown etiology. Recently it has been classified into several distinct entities on the basis of pathologic and clinical characteristics, ie : usual interstitial pneumonia (UIP), desquamative interstitial pneumonia (DIP), acute interstitial pneumonia (AIP), bronchiolitis obliterans with organizing pneumonia (BOOP), and nonspecific interstitial pneumonia (NSIP). IPF is now applied only for UIP, which has the worst prognosis. The previous reports of 3-5 year median survival appears to be overoptimistic because other types with better prognosis like NSIP or BOOP might have been included. Therefore, this study was performed to determine the clinical course and the prognostic factors of UIP as diagnosed by surgical lung biopsy. Methods : The subjects were 72 UIP patients (age $58.2{\pm}11.6$ years, M : F=45 : 27, median follow up period : 18.1 months (0.7-103.6) diagnosed by surgical lung biopsy at the Asan Medical Center (68 patients) and the Paik Hospital in Seoul (4 patients). Clinical scores (level of dyspnea : 1-20 points), radiologic score (honeycombing : HC score 0-5 points, ground glass : GG score 0-5 points), and physiologic scores (FVC : 1-12 points, $FEV_1$ : 0-3 points, TLC : 0-10 points, $D_{LCO)$ : 0-5 points, $AaDO_2$ : 0-10 points) were summed into a total CRP score. Results : 1) The one year survival rate was 78.3%, while the rate for three year survival was 58.1%, and the median survival period was 42.5months. 2) Short term (1 year) prognosis : The patients who died within one year of diagnosis (14 patients) had the higher initial total CRP score ($28.6{\pm}8.3$ vs. $16.6{\pm}9.7$) than those who lived longer than one year (46 patients). The difference in the total CRP score was attributed to the symptom score ($8.4{\pm}2.1$ vs. $5.7{\pm}3.9$) and the physiologic score ($15.7{\pm}7.1$ vs. $6.7{\pm}5.7$) including FVC, $D_{LCO)$ and $AaDO_2$. 3) Long-term (3year) prognosis : The total CRP score ($12.2{\pm}6.7$ vs. $28.7{\pm}7.9$ : including symptom score, FVC, $D_{LCO)$ and $AaDO_2$) at the time of diagnosis were also different for the long-term survivors and those who lived less than 3 years. 4) Cox regression analysis showed $D_{LCO)$ (${\geq}$60%) (Hazard ratio : 4.56, 95% CI : 2.30-16.04) was the independent prognostic factors of UIP (P<0.05). Conclusion : These results suggest that $D_{LCO)$ at the time of diagnosis seem to be a prognostic markers of biopsy-proven UIP.
The conventional method for assessment of liquefaction potential proposed by Seed and Idriss has been widely used in most countries because of simplicity of tests. Even though various data such as stress, strain, stress path, and excess pore water pressure can be obtained from the dynamic test, especially, two simple experimental data such as the maximum deviatoric stress and the number of cycles at liquefaction have been used in the conventional assessment. In this study, a new detailed assessment for liquefaction potential to reflect both characteristics of real earthquake motion and dynamic soil resistance is proposed and verified. In the assessment, the safety factor of the liquefaction potential at a given depth of a site can be obtained by the ratio of a resistible cumulative plastic shear strain determined through the performance of the conventional cyclic test and a driving cumulative plastic shear strain calculated from the shear strain time history through the ground response analysis. The last point to cumulate the driving plastic shear strain to initiate soil liquefaction is important for this assessment. From the result of cyclic triaxial test using real earthquake motions, it was concluded that liquefaction under the impact-type earthquake loads would initiate as soon as a peak loading signal was reached. The driving cumulative plastic shear strain, therefore, can be determined by adding all plastic shear strains obtained from the ground response analysis up to the peak point. Through the verification of the proposed assessment, it can be concluded that the proposed assessment for liquefaction potential can be a progressive method to reflect both characteristics of the unique soil resistance and earthquake parameters such as peak earthquake signal, significant duration time, earthquake loading type, and magnitude.
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