Journal of the korean academy of Pediatric Dentistry
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v.47
no.1
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pp.93-98
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2020
Lesch-Nyhan syndrome is a rare X-linked, recessively inherited disorder of purine metabolism, caused by complete absence of the enzyme hypoxanthine-guanine phosphoribosyl transferase. This syndrome is characterized by 3 major features: neurological dysfunction, hyperuricemia, and cognitive and behavioral disturbances (e.g., self-mutilation, which begins at 2 to 3 years of age). Uncontrollable self-mutilation begins with biting of the perioral tissues and extends into patterns such as finger biting and head hitting. This report describes the case of a 31-month-old boy who was diagnosed with Lesch-Nyhan syndrome with severe lip injuries caused by self-mutilative behaviors. The behaviors were blocked with a semi-fixed lip bumper for a short period. The device was applied to the patient on the day of the visit without the requirement for an oral impression. It was easy to manage oral hygiene and adjust the device because it was detachable by clinicians and guardians. Therefore, a semi-fixed lip bumper may be useful as an interim appliance to block selfmutilative behaviors in children with Lesch-Nyhan syndrome.
Objectives : The oriental medicine Jangwonhwan, a boiled extract of 12 medicinal herbs/mushrooms, has been prescribed to patients with cognitive dysfunction, as originally described in the Korean medical text, DonguiBogam(amnesia chapter). Recently, a modified formula of Jangwonhwan (LMK02-Jangwonhwan) consisting of seven medicinal plants/mushrooms, was shown to reduce the ${\beta}$-amyloid deposition in the brain of Tg-APPswe/PS1dE9 mouse model for Alzheimer's disease. The toxicity of LMK02-Jangwonhwan was investigated in SD rats, by a daily oral administration for 13 weeks and NOAEL(No observed adverse effect dose), a definite toxic dose and target organ, as well. Methods : Quality control of the tablet form of LMK02-Jangwonhwan was established by estimating the indicative components, Ginsenoside Rg3 of Red Ginseng and Decursin of Angelicagigas Nakai. The toxicity of LMK02-Jangwonhwan was investigated in 6 week old, specific pathogen free (SPF), Sprageu-Dawley rats by oral administration. Each test group consisted of 10 male and 10 female rats. The groups received doses of 500, 1,000 or 2,000 mg/kg/day of test substance for 13 weeks. The clinical signs, death rate, body weight, food consumption, ophthalmic examination, urinalysis, hematological and serum biochemistry, organ weight and pathological changes were examined and compared with those of the control group. Results : The 13-week repeated oral treatment doses didn't result in any specific symptoms or death. There were no significant changes in the rat's weight and food consumption. Further, ophthalmic examination, urinalysis, hematological, serum biochemistry test and organ weight revealed no significant differences. Conclusions : The no-observed-adverse-effect level(NOAEL) of LMK02 for male and female Sprague-Dawley rats was determined as 2,000mg/kg/day and the target organ wasn't confirmed. Because no significant adverse effects were observed, the target organ could not be determined.
Journal of Physiology & Pathology in Korean Medicine
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v.24
no.6
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pp.1034-1041
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2010
The oriental medicine Jangwonhwan, which is a boiled extract of 12 medicinal herbs/mushroom, has been prescribed for patients with cognitive dysfunction and it is originally from the Korean medical text, DonguiBogam(amnesia chapter). Recently, a modified recipe of Jangwonhwan (LMK02-Jangwonhwan) consisting of seven medicinal plants/mushroom, was shown to reduce ${\beta}$-amyloid deposition in the brain of Tg-APPswe/PS1dE9 mouse model of Alzheimer disease. The toxicity of LMK02 was investigated in SD rats by oral repeated adminstration for 4 weeks and we tried to determine test does for 13 weeks repeated study. Quality control of tablet form of LMK02 was established by estimating indicative components, Ginsenoside Rg3 of Red Ginseng and Decursin of Angelicagigas Nakai. The toxicity of LMK02 was investigated in 6 weeks old specific pathogen free (SPF) Sprageu-Dawley rats by oral administration. Each test group were consist of 5 male and 5 female and they received doses of 500, 1,000 and 2,000 mg/kg/day of test substance for 4 weeks. The clinical signs, death rate, body weight, food consumption, ophthalmic examination, urinalysis, hematological and serum biochemistry, organ weight and pathological changes were examined and compared with those of control group. Urinalysis : We observed increase of PRO(p<0.01), SG(p<0.01) in female rats of 1,000 mg/kg/day and 2,000 mg/kg/day(p<0.01). Also, we observed increase of pH and KET in female rats of 1,000 mg/kg/day(p<0.05) and of 2,000 mg/kg/day(p<0.01). WBC in female rats in 1,000 mg/kg/day and 2,000 mg/kg/day were on increase. Hematological test : We observed increase of MCV in male rats of 250 mg/kg/day. (p<0.05) Serum biochemistry test : We found increase of CHO in female rats of 2,000 mg/kg/day(p<0.05). During the experimental period, there were no animals dead or moribund. There were no treatment related changes of general symptom, food and water consumption, organ weight and autopsy According to the results of 4-week repeated dose range finding study, the highest dose was established as 1000 mg/kg for 13-week repeated dose toxicity study and we determined to put 2 more groups by common ratio two.
This study was conducted to develop a new method for enhancing ginsenoside $Rg_3$, which is abundant in black ginseng. The cognition-enhancing effect of black ginseng extract was investigated via the assay of acetylcholinesterase (AChE) activity. Black ginseng I was prepared through the traditional method (by steaming and drying nine times repetitions). Black ginseng II, on the other hand, was prepared by steaming the ginseng three times at $120^{\circ}C$ for 30 min after soaking it in grape juice for 24 h. The ginsenosides of white, red, and black ginseng I, and II were investigated using the HPLC method, respectively. In black ginseng II, the ginsenoside $Rg_3$ contents, which cannot be found in white ginseng, amounted to 10.91 mg/g, approximately 18 times more than that in red ginseng. In the in-vivo study, black ginseng extract (200 mg/kg, p.o.) inhibited the AChE activity after 24 h by a single administration in the brain. Thus, the new manufacturing method for black ginseng was found to more effective in the conversion of ginsenoside $Rg_3$ compared to the traditional method. Black ginseng may also have the effect of preventing the cognitive impairment induced by cholinergic dysfunction.
Evidence suggests that even low-dose irradiation can lead to progressive cognitive decline and memory deficits, which implicates, in part, hippocampal dysfunction in both humans and experimental animals. This study examined whether diethyldithiocarbamate (DDC) could attenuate memory impairment, using passive avoidance and object recognition test, and suppression of hippocampal neurogenesis, using the TUNEL assay and immunohistochemical detection with markers of neurogenesis (Kiel 67 (Ki-67) and doublecortin (DCX)) in adult mice treated with gamma radiation (0.5 or 2 Gy). DDC was administered intraperitonially at a dosage of 1,000 $mg{\cdot}kg^{-1}$ of body weight at 30 min. before irradiation. In passive avoidance and object recognition memory test, the mice, trained for 1 day after acute irradiation (2 Gy) showed significant memory deficits compared with the sham controls. The number of TUNEL-positive apoptotic nuclei in the dentate gyrus (DG) was increased 12 h after irradiation. In addition, the number of Ki-67- and DCX-positive cells were significantly decreased. DDC treatment prior to irradiation attenuated the memory defect, and blocked the apoptotic death. DDC may attenuate memory defect in a relatively low-dose exposure of radiation in adult mice, possibly by inhibiting a detrimental effect of irradiation on hippocampal neurogenesis.
Shin, Su Bin;Jang, Seok Ju;Lee, Na Gyeong;Choi, Byung Tae;Shin, Hwa Kyoung
Journal of Life Science
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v.32
no.7
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pp.550-566
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2022
Stroke is among the leading causes of death and long-term physical and cognitive disabilities worldwide, affecting an estimated 15 million people annually. The pathophysiological process of stroke is complicated by multiple and coordinated events. The breakdown of the blood-brain barrier (BBB) in people with stroke can significantly contribute to the development of ischemic brain injury. Therefore, BBB disruption is recognized as a hallmark of stroke; thus, it is important to develop novel therapeutic strategies that can protect against BBB dysfunction in ischemic stroke. Traditional medicines are composed of natural products, which represent a promising source of new ingredients for the development of conventional medicines. Indeed, several studies have shown the effectiveness of Korean medicine on stroke, highlighting the value of Korean medicinal treatment for ischemic stroke. This review summarizes the current information and underlying mechanisms regarding the ameliorating effects of the formula, decoction, herbs, and active components of traditional Korean medicine on cerebral ischemia-induced BBB disruption. These traditional medicines were shown to have protective effects on the BBB in many cellular and animal ischemia models of stroke, and experiments in various animal species, such as mice and rats. In addition, they showed brain-protective effects by protecting the BBB through the regulation of tight junction proteins and matrix metalloproteinase-9, reducing edema, neuroinflammation, and neuronal cell death. We hope that this review will help promote further investigation into the neuroprotective effects of traditional Korean medicines and stimulate the performance of clinical trials on Korean herbal medicine-derived drugs in patients with stroke.
Purpose: This study proposes a concert oriented music therapy (COMT) program which takes into account the personal and social factors of depression, and its clinical effects were validated on elderly persons with visual impairments who were susceptible to depression. The experiment was conducted on 34 elderly persons with visual impairments, randomly allocated to either the experimental group (n = 15) or the control group (n = 19). The COMT program was applied 20 times in total to the experimental group for 110 minutes every other week, for a duration of 10 months. The depression level was evaluated using the Korean form of the Geriatric Depression Scale (KGDS). The experimental results showed that the average KGDS score in the experimental group decreased by 4.1 points (19.4%), from 21.0 to 16.9, whereas it increased by 1.16 points (5.8%), from 19.95 to 21.11, in the control group. The reduction in the depression level was significant for the following 4 depression factors : decreased social interest (40%), negative thinking and the unhappiness feeling (20%), emotional discomfort (16.3%), and cognitive dysfunction (7.6%), whereas it was negligible in the factors of physical weakening and decreased vitality. The COMT program reduced the KGDS scores of the experimental group regardless of their experience in musical performance. In conclusion, the proposed COMT program proved to be efficacious in alleviating depression in elderly persons with visual impairments. In particular, it proved highly effective in helping with decreased social interest, namely, the social factor of depression which is not properly considered in existing music therapy methods.
Hyuk Sung Kwon;Seong-Ho Koh;Seong Hye Choi;Jee Hyang Jeong;Hae Ri Na;Chan Nyoung Lee;YoungSoon Yang;Ae Young Lee;Jae-Hong Lee;Kyung Won Park;Hyun Jeong Han;Byeong C. Kim;Jinse Park;Jee-Young Lee;Kyu-Yong Lee;Sangjae Kim
Dementia and Neurocognitive Disorders
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v.22
no.3
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pp.100-108
/
2023
Background and Purpose: The efficacy and safety of GV1001 have been demonstrated in patients with moderate-to-severe Alzheimer's disease (AD). In this study, we aimed to further demonstrate the effectiveness of GV1001 using subscales of the Severe Impairment Battery (SIB), which is a validated measure to assess cognitive function in patients with moderate-to-severe AD. Methods: We performed a post hoc analysis of data from a 6 month, multicenter, phase 2, randomized, double-blind, placebo-controlled trial with GV1001 (ClinicalTrials.gov, NCT03184467). Patients were randomized to receive either GV1001 or a placebo for 24 weeks. In the current study, nine subscales of SIB-social interaction, memory, orientation, language, attention, praxis, visuospatial ability, construction, and orientation to name-were compared between the treatment (GV1001 1.12 mg) and placebo groups at weeks 12 and 24. The safety endpoints for these patients were also determined based on adverse events. Results: In addition to the considerable beneficial effect of GV1001 on the SIB total score, GV1001 1.12 mg showed the most significant effect on language function at 24 weeks compared to placebo in both the full analysis set (FAS) and per-protocol set (PPS) (p=0.017 and p=0.011, respectively). The rate of adverse events did not differ significantly between the 2 groups. Conclusions: Patients with moderate-to-severe AD receiving GV1001 had greater language benefits than those receiving placebo, as measured using the SIB language subscale.
To know the feasibility of the coronary artery bypass graft (CABG) for multivessel coronary artery disease with purely bilateral internal thoracic arteries (ITAs), we analyzed the short-term clinical results and the coronary angiography of the patients. Material and Method: From March 2001 to June 2002, four hundred and five patients underwent CABG. Purely bilateral ITAs were used in 159 patients (39.3%). We analyzed these patients retrospectively The mean age of these patients was $61.2{\pm}8.5$ (range: 30 ~80) years and there were 123 male patients. The preoperative risk factors were as follows: diabetes in 54 patients (34.0%), history of acute myocardiac infarction within 4 weeks in 29 (18.2%), and emergency operation in 6 (3.8%). Off-pump CABG was carried out in 128 patients (80.5%). Associated procedures were mitral valvuloplasty (5), aortic valve replacement (3), Dor procedure (1), and so on. Result: The mean number of distal anastomoses was $3.1{\pm}0.9$ (range: 2~6), the mean duration of hospital stay was $8.4{\pm}4.5$ days. There was one (0.6%) operative death. Except for one early death, no other patients suffered from low cardiac output. The other postoperative complications were occurred as follows: reoperation due to bleeding in 3 patients, perioperative myocardiac infarction in 1, transient cardiac arrest in 2, transient cognitive dysfunction in 7, and transient ischemic attack in 1, and deep sternal wound infection in 1 patient. Recently, early postoperative angiography was performed in 19 patients who had triple vessel disease. The total number of distal anastomosis was 78 (mean $4.1{\pm}0.8$/patient). All distal anastomosis sites were patent, but competition flow was observed at the bypass sites where the native coronary artery stenosis was not significant. Conclusion: The CABG with purely bilateral ITAs for triple vessel disease was performed safely. The early patency rate was relatively good in small number of patients. However the long-term patency rate and the functional study to evaluate the sites where competition flow was observed should be followed.
Purpose: The purpose of this study was to evaluate alterations of regional cerebral blood flow (CBF) and cerebrovascular reserve (CVR), and correlation between these alternations and cognitive dysfunction in patients with chronic traumatic brain injury (TBI) and normal brain MRI findings. Materials and Methods: Thirty TBI patients and 19 healthy volunteers underwent rest/acetazolamide brain SPECT using Tc-99m HMPAO. Korean-Wechsler Adult Intelligence scale test was also performed in the patient group. Statistical analysis was performed with statistical parametric mapping software (SPM'97) Results: CBF was diminished in the left hemisphere including Wernicke's area in all patients with lower verbal scale scores. In addition, a reduction in CBF in the right frontal, temporal and parietal cortices was related with depressed scores in information, digital span, arithmetic and similarities. In patients with lower performance scale scores, CBF was mainly diminished in the right hemisphere including superior temporal and supramarginal gyri, premotor, primary somatomotor and a part of prefrontal cortices, left frontal lobe and supramarginal gyrus. CVR was diminished in sixty-four Brodmann's areas compared to control. A reduction in CVR was demonstrated bilaterally in the frontal and temporal lobes in patients with lower scores in both verbal and performance tests, and in addition, both inferior parietal and occipital lobes in information subset. Conclusion: Alterations of CBF and CVR were demonstrated in the symptomatic TBI patients with normal MRI finding. These alterations were correlated with the change of intelligence, of which the complex functions are subserved by multiple interconnected cortical structures.
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