Alagille syndrome (AGS) is a rare autosomal dominant inherited disorder, with major clinical manifestations of bile duct paucity, cholestasis, cardiovascular anomaly, ophthalmic abnormalities, butterfly vertebrae, and dysmorphic facial appearance. It is caused by heterozygous mutations in JAG1 or NOTCH of the Notch signaling pathway presenting with variable phenotypic penetrance and involving multiple organ systems. The following case report describes a unique case of a 16-year-old female with AGS who presented with the primary complaint of renovascular hypertension. She had a medical history of ventricular septal defect and polycystic ovary syndrome. The patient had a dysmorphic facial appearance including frontal bossing, bulbous tip of the nose, a pointed chin with prognathism, and deeply set eyes with mild hypertelorism. Stenoocclusive changes of both renal arteries, celiac artery, lower part of the abdominal aorta, and left intracranial artery, along with absence of the left internal carotid artery were found on examination. Whole exome sequencing was performed and revealed a pathologic mutation of JAG1, leading to the diagnosis of AGS. Reverse phenotyping detected butterfly vertebrae and normal structure and function of the liver and gallbladder. While the representative symptom of AGS in most scenarios is a hepatic problem, in this case, the presenting clinical features were the vascular anomalies. Clinical manifestations of AGS are diverse, and this case demonstrates that renovascular hypertension might be in some cases a presenting symptom of AGS.
Noni has been used for medicinal purposes for more than 2,000 years in South Pacific Polynesia, China and India, and has been heavily ingested as an extract for its excellent antioxidant and anti-inflammatory effects. However, a recent study found that the noni extract causes digestive disorders, kidney problems, and liver diseases, which made it necessary to use it for other purposes than as an extract. In this study, we want to evaluate the potential of noni as an anti-oxidant, anti-inflammatory and anti-wrinkling agent. Methods: Noni was freeze-dried, extracted in water, and concentrated. Skin cells were treated with the noni extract for 24 hrs and then were exposed to UVB (55 mJ/cm2). After 48 hrs of incubation, pro-inflammatory cytokine, elastase, MMP-1 and type-1 procollagen levels were measured by ELISA. Results: To find out the antioxidant effect of the noni extract, the DPPH and ABTS radical scavenging activity experiments were conducted and the noni extract showed 97.0 % and 92.0 % antioxidant efficacy at 200 ㎍/mL respectively. The noni extract (50 and 100 ㎍/mL) decreased IL-6 and TNF-α in RAW 264.7 cells induced by LPS in a concentration-dependent manner. In the RT-PCR experiment involving NO production, the noni extract (50 and 100 ㎍/mL) inhibited NO production by strongly inhibiting iNOS mRNA expression, and also inhibited the elevation of MMP-1 and elastases caused by UVB irradiation by 25.0 % and 7.0 % respectively. In addition, type-1 procollagen was elevated by 20.0 % by the noni extract treatment in HaCaT cells. Conclusion: The noni extract has photoprotective ability by reducing proinflammatory mediators, elastase and MMP-1 production, and elevation of collagen synthesis. Our findings suggest that the noni extract might be a good natural substance to protect against UVB-induced premature skin aging.
Objectives : To study the characteristics and meaning of the division pharmaceutical method in the Donguibogam through analysis of formulas that apply such method. Methods : Each formula applying the division method within the Donguibogam was analyzed Results & Conclusions : In the Donguibogam, medicinals to which the division pharmaceutical method was applied were baizhu[白朮], cangzhu[蒼朮], zhiqiao[枳殼], wuzhuyu[吳茱萸], xiangfuzi[香附子], chuanlianzi[川練子], huangbo[黃柏], etc. Formulas where this method was used were few, while the four methods that were used, together with the Center represents the principle of the five phases. The formulas that applied the division method usually did not adopt the tablet or decoction form, which means they were meant to treat slowly, treating deficiency or stagnation diseases due to problems in the middle-lower body such as the Spleen, Liver and Kidney. It could be said that compared to other more common formulas, this was a unique method. The division pharmaceutical method allows for usage of a wider variety of processing methods compared to single ingredient formulas, while there is lower chance of interference among the different types of processing. Another benefit is that the properties of the main medicinal ingredient could be preserved as much as possible. In addition, the division method allows for the main ingredient and the processed ingredient to interact exclusively, after which the processed medicinal is eliminated leaving only the main ingredient. This allows for maximum improvement of the main ingredient. The division method includes the principles of formula composition as well, whose concept could be positioned between single ingredient formulas and common formulas.
Yun, Ji Young;Jeong, Jin Boo;Eo, Hyun Ji;Kwon, Kun Woo;Hong, Se Chul;Jeong, Hyung Jin;Koo, Jin Suk
The Korea Journal of Herbology
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v.27
no.6
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pp.7-13
/
2012
Objectives : Reactive oxygen species (ROS) have been associated with pathogenic processes including carcinogenesis through direct effect on DNA directly and by acting as a tumor promoter. Therefore, it has been regarded that ROS may be a major target for cancer prevention. The root of Paeonia lactiflora pall (PL), a traditional Chinese herb, has been a component of effective prescriptions for treatment of liver disease. Also, there are some reports about the antioxidant activities of the extracts from PL. However, little has been known about the effects of PL against oxidative damage. This work aimed to elucidate the anti-oxidant effects of Paeonia lactiflora pall (PL) in the non-cellular system and cellular system. Methods : Antioxidant activities of PL were evaluated by hydroxyl radical scavenging assay and $Fe^{2+}$ chelating assay. Anti-oxidative effect of PL was evaluated by ${\varphi}X$-174 RF I plasmid DNA cleavage assay in non-cellular system. In addition, DNA migration assay, expression level of phospho-H2AX, MTT assay and lipid peroxidation assay were performed for evaluate the anti-oxidative effect of PL in cellular system. Results : PL had a dose-dependent hydroxyl radical scavenging and $Fe^{2+}$ chelating capacity. In addition, PL inhibited oxidative DNA and cell damage induced by hydroxyl radical in non-cellular system and cellular system. Conclusion : Taken together, P. lactiflora pall may be possible for the application to a potential drug for treating the oxidative diseases such as cancer.
The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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v.35
no.3
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pp.1-14
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2022
Background : Tinnitus is common disorder with many possible causes and a symptom of many different diseases but has no effective treatment. Most of tinnitus patients experience the disorder to a degree that their quality of life and productivity are impaired. Especially tinnitus accompanied hearing loss causes severe discomfort in patients. Objectives : 1. Through symptoms of tinnitus, we tried to find out the relationship of Heo-sil(虛實) and Zuang fu bian zheng(臟腑辨證) among tinnitus patients accompanied Hearing Loss. 2. By testing THI, we tried to evaluate the quality of life of tinnitus patients group and tried to compare the quality of life between high-narrow tone and low-wide tone tinnitus patients group. 3. In the future, this study was planned to help monitor the prognosis and determine the progress of treatment for tinnitus patients who also have hearing loss. Methods : 43 patients were recruited in this study who was ill with tinnitus accompanied hearing loss. They wrote out the questionnaire about tinnitus and the Tinnitus Handicap Inventory(THI). Results : 1. In tinnitus patients, there were more men than women accompanied hearing loss. The rate of visits was higher as the age increased. 2. Most of the patients who visited the hospital had sensitive hearing loss. 3. In high-narrow tone tinnitus group, phlegm fire congesting-type are more uncomfortable than spleen-stomach weakness-type. 4. In low-wide tone tinnitus group, liver qi depression was the most uncomfortable. Conclusions : The present study suggests that tinnitus pattern may be helpful in differentiating patients with tinnitus, and the effectiveness of treatment can be predicted through differentiation. Zuang fu bian zheng(臟腑辨證) and Distinguishing between Heo(虛) and Sil(實) is expected to have positive effects on tinnitus accompanied hearing loss treatment.
Children and adolescents with coronavirus disease 2019 (COVID-19) generally have mild symptoms. Severe infection due to severe acute respiratory syndrome coronavirus 2 (SARSCoV-2) involving multiorgan dysfunction is rare in this population. Herein, we present an unusual case of severe SARS-CoV-2 infection with multiorgan involvement followed by multisystem inflammatory syndrome in children (MIS-C) in a vaccinated 16-year-old boy. The patient was unconscious on initial presentation, and had severe paralytic ileus. On laboratory examination, there was severe metabolic acidosis, lymphocytopenia, thrombocytopenia, elevated inflammatory markers, elevated liver enzymes, and evidence of acute kidney injury with proteinuria and hematuria. His symptoms improved with the administration of remdesivir and dexamethasone. The patient briefly experienced MIS-C 2 weeks after the diagnosis of COVID-19, but the patient was discharged without any complications.
Purpose: Systemic health has a profound effect on dental treatment. The aim of this study was to evaluate peri-implant bone loss and health screening data to discover factors that may influence peri-implant diseases. Methods: This study analyzed the panoramic X-rays of patients undergoing health screenings at the Health Promotion Center at Seoul St. Mary's Hospital in 2018, to investigate the relationship between laboratory test results and dental data. The patients' physical data, such as height, weight, blood pressure, hematological and urine analysis data, smoking habits, number of remaining teeth, alveolar bone level, number of implants, and degree of bone loss around the implant, were analyzed for correlations. Their associations with glycated hemoglobin, glucose, blood urea nitrogen (BUN), creatinine, and severity of periodontitis were evaluated using univariate and multivariate regression analysis. Results: In total, 2,264 patients opted in for dental health examinations, of whom 752 (33.2%) had undergone dental implant treatment. These 752 patients had a total of 2,658 implants, and 129 (17.1%) had 1 or more implants with peri-implant bone loss of 2 mm or more. The number of these implants was 204 (7%). Body mass index and smoking were not correlated with peri-implant bone loss. Stepwise multivariate regression analysis revealed that the severity of periodontal bone loss (moderate bone loss: odds ratio [OR], 3.154; 95% confidence interval [CI], 1.175-8.475 and severe bone loss: OR, 7.751; 95% CI, 3.003-20) and BUN (OR, 1.082; 95% CI, 1.027-1.141) showed statistically significant predictive value. The severity of periodontitis showed greater predictive value than the biochemical parameters of blood glucose, renal function, and liver function. Conclusions: The results of this study showed that periodontal bone loss was a predictor of peri-implant bone loss, suggesting that periodontal disease should be controlled before dental treatment. Diligent maintenance care is recommended for patients with moderate to severe periodontal bone loss.
Background: Rb3 is a ginsenoside with anti-inflammatory properties in many cell types and has been reported to attenuate inflammation-related metabolic diseases such as insulin resistance, nonalcoholic fatty liver disease, and cardiovascular disease. However, the effect of Rb3 on podocyte apoptosis under hyperlipidemic conditions, which contributes to the development of obesity-mediated renal disease, remains unclear. In the current study, we aimed to investigate the effect of Rb3 on podocyte apoptosis in the presence of palmitate and explore its underlying molecular mechanisms. Methods: Human podocytes (CIHP-1 cells) were exposed to Rb3 in the presence of palmitate as a model of hyperlipidemia. Cell viability was assessed by MTT assay. The effects of Rb3 on the expression of various proteins were analyzed by Western blotting. Apoptosis levels were determined by MTT assay, caspase 3 activity assay, and cleaved caspase 3 expression. Results: We found that Rb3 treatment alleviated the impairment of cell viability and increased caspase 3 activity as well as inflammatory markers in palmitate-treated podocytes. Treatment with Rb3 dosedependently increased PPARδ and SIRT6 expression. Knockdown of PPARδ or SIRT6 reduced the effects of Rb3 on apoptosis as well as inflammation and oxidative stress in cultured podocytes. Conclusions: The current results suggest that Rb3 alleviates inflammation and oxidative stress via PPARδ-or SIRT6-mediated signaling, thereby attenuating apoptosis in podocytes in the presence of palmitate. The present study provides Rb3 as an effective strategy for treating obesity-mediated renal injury.
So-Yeon Han;Hye-Jeong Park;Jeong-Yong Park;Seo-Hyun Yun;Mi-Ji Noh;Soo-Yeon Kim;Tae-Won Jang;Jae-Ho Park
Proceedings of the Plant Resources Society of Korea Conference
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2021.04a
/
pp.54-54
/
2021
The Melanoma Research Coalition reported melanoma affects humans of various races. This study was conducted to confirm the inhibitory effect of melanogenesis in B16 F10 cells of Nypa fruticans Wurmb of ethyl acetate fraction (NEF). Nypa fruticans Wurmb is an important component of the East Asian mangrove vegetation. It belongs to Araceae family. Traditionally, N. fruticans was used to treat various diseases such as asthma, sore throat, liver disease, a pain reliever, and can also be used as sedative and carminative. The present study, the inhibitory effect on melanogenesis was determined by Western blotting and RT-qPCR. The level of expression of tyrosinase, TRP-1, and TRP-2 is regulated by microphthalmia-associated transcription factor (MITF) and cAMP, and cAMP affects the activity of protein kinase A (PKA). Activated PKA stimulates the phosphorylation of cAMP-reactive element-binding protein (CREB) in the nucleus, thereby increasing the amount of MITF expression and enhancing melanogenesis. Western blotting and RT-qPCR analysis showed that NEF treatment decreased the expression of tyrosinase. Similarly, TRP-1 and TRP-2 levels were decreased, which were decreased significantly at compared with the untreated control. Also, NEF attenuated the IBMX mediated increase in the intracellular cAMP level and the phosphorylation of PKA. In conclusion, NEF significantly inhibited the expressions of melanogenesis through cAMP/PKA/CREB signaling pathways.
Ji-Yong Jung;Joong Hyun Shim;Su Hae Cho;Il-Hong Bae;Seung Ha Yang;Jinsick Kim;Hye Won Lim;Dong Wook Shin
Biomolecules & Therapeutics
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v.32
no.2
/
pp.224-230
/
2024
Pinitol (3-O-Methyl-D-chiro-inositol) has been reported to possess insulin-like effects and is known as one of the anti-diabetic agents to improve muscle, liver, and endothelial cells. However, the beneficial effects of pinitol on the skin are not well known. Here, we investigated whether pinitol had effects on human dermal fibroblasts (HDFs), and human dermal equivalents (HDEs) irradiated with ultraviolet A (UVA), which causes various damages including photodamage in the skin. We observed that pinitol enhanced wound healing in UVA-damaged HDFs. We also found that pinitol significantly antagonized the UVA-induced up-regulation of matrix metalloproteinase 1 (MMP1), and the UVA-induced down-regulation of collagen type I and tissue inhibitor of metalloproteinases 1 (TIMP1) in HDEs. Electron microscopy analysis also revealed that pinitol remarkably increased the number of collagen fibrils with regular banding patterns in the dermis of UVA-irradiated human skin equivalents. Pinitol significantly reversed the UVA-induced phosphorylation levels of ERK and JNK but not p38, suggesting that this regulation may be the mechanism underlying the pinitol-mediated effects on UVA-irradiated HDEs. We also observed that pinitol specifically increased Smad3 phosphorylation, which is representative of the TGF-β signaling pathway for collagen synthesis. These data suggest that pinitol exerts several beneficial effects on UVA-induced damaged skin and can be used as a therapeutic agent to improve skin-related diseases.
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