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A lumen-apposing metal stent (LAMS) is a saddle-shaped stent with large flanges at both ends, thereby preventing stent migration and helping with approximation of the adjacent structures. We report the case of a 25-year-old female with remnant choledochal cyst which was successfully treated with LAMS after initial treatment failure with a plastic stent. Although complete excision of the cyst is the definite treatment of choledochal cysts, endoscopic ultrasonography-guided cystoduodenostomy can be considered in cases wherein surgery is not feasible and dysplasia is not present. LAMS may be preferred to plastic stents for effective resolution of remnant choledochal cyst and prevention of ascending infection.
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Purpose@#To analyze the efficacy and safety of preservative-containing and preservative-free 0.2% brimonidine tartrate and 0.5% timolol maleate fixed combination drug in normal tension glaucoma. @*Methods@#Fifty-one patients (84 eyes) who were diagnosed with normal tension glaucoma and with preservative-containing or preservative-free brimonidine-timolol fixed combinations alone were analyzed retrospectively from January 2017 to February 2020. Intraocular pressure (IOP) was measured four times a day (9 a.m., 11 a.m., 2 p.m., and 4 p.m.) before and at 6 months after applying eye drops. We analyzed and compared the effect of lowering IOP and the occurrence of intra or extra-ocular adverse effects. @*Results@#A significant mean IOP reduction was shown in both groups: -1.95 ± 2.50 mmHg (-12.26 ± 15.87%) in the preservative-containing group and -1.60 ± 2.06 mmHg (-10.54 ± 13.94%) in the preservative-free group at 6 months after eyedrop instillation. The IOP was lowest in both groups at 11 a.m. There were no significant differences between the two groups in lowering IOP. Serious adverse effects causing discontinuation of the eye drops were not observed. @*Conclusions@#Both preservative-containing and preservative-free brimonidine-timolol fixed combinations are effective in lowering IOP in normal tension glaucoma patients and are considered to be effective as eye drops without serious adverse effects.
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Purpose@#To observe if obesity is a representative risk factor for vision-threatening proliferative diabetes retinopathy and end stage renal disease, which are major chronic microvascular complications in Korean Type 2 diabetes patients. @*Methods@#A retrospective data analysis was conducted on 2,524,431 adults over the age of 30 years diagnosed with type 2 diabetes using the Korean National Health Insurance Service-Health Screening database from January 2009 to December 2012. The association of body mass index (BMI) and waist circumference with the occurrence of sight-threatening diabetic retinopathy and end-stage renal disease in type 2 diabetes mellitus patients were analyzed. @*Results@#Multivariable Cox regression analysis was carried out on the potential risk factors related to microvascular complications, including vision-threatening diabetic retinopathy and end-stage renal disease. BMI and microvascular complications showed a reverse linear relationship, but a larger waist circumference was associated with a greater risk of microvascular complications (incidence rate ratio [IRR] = 1.049, 95% confidence interval [CI] = 1.021-1.078; IRR = 1.087, 95% CI = 1.05-1.125, and IRR = 1.234, 95% CI = 1.182-1.289) after adjusting other risk factors. @*Conclusions@#In our study, the results showed that type 2 diabetic patients with a larger waist circumference were at greater risk for microvascular complications. Thus, in addition to the BMI, the waist circumference should be used as the obesity parameter in microvascular complication risk assessment among type 2 diabetic patients.
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Purpose@#To observe if obesity is a representative risk factor for vision-threatening proliferative diabetes retinopathy and end stage renal disease, which are major chronic microvascular complications in Korean Type 2 diabetes patients. @*Methods@#A retrospective data analysis was conducted on 2,524,431 adults over the age of 30 years diagnosed with type 2 diabetes using the Korean National Health Insurance Service-Health Screening database from January 2009 to December 2012. The association of body mass index (BMI) and waist circumference with the occurrence of sight-threatening diabetic retinopathy and end-stage renal disease in type 2 diabetes mellitus patients were analyzed. @*Results@#Multivariable Cox regression analysis was carried out on the potential risk factors related to microvascular complications, including vision-threatening diabetic retinopathy and end-stage renal disease. BMI and microvascular complications showed a reverse linear relationship, but a larger waist circumference was associated with a greater risk of microvascular complications (incidence rate ratio [IRR] = 1.049, 95% confidence interval [CI] = 1.021-1.078; IRR = 1.087, 95% CI = 1.05-1.125, and IRR = 1.234, 95% CI = 1.182-1.289) after adjusting other risk factors. @*Conclusions@#In our study, the results showed that type 2 diabetic patients with a larger waist circumference were at greater risk for microvascular complications. Thus, in addition to the BMI, the waist circumference should be used as the obesity parameter in microvascular complication risk assessment among type 2 diabetic patients.
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Background@#Acute myeloid leukemia (AML) with internal tandem duplication in FMS-like tyrosine kinase 3 (FLT3-ITD) is associated with poor outcomes. This study aimed to analyze the outcomes of pediatric AML patients with FLT3-ITD mutations in the pre-FLT3 inhibitor era. @*Methods@#We retrospectively reviewed and identified 18 patients diagnosed with non-M3 AML with FLT3-ITD mutations at Seoul National University Children’s Hospital between May 2008 and August 2019. @*Results@#The median age was 13 years (range, 6‒19 yr). The median follow-up time was 43 months (range, 6‒157 mo). Fourteen patients received BH-AC-based (N4-Behenoy1-1-β-D-arabinofuranosy1cytosine) and 4 received cytarabine-based induction chemotherapy.Complete remission (CR) was achieved in 72.2% of the patients after the first induction chemotherapy and 80% of the patients achieved CR after salvage therapy. The overall CR rate was 94% (17/18 patients). These 17 patients underwent hematopoietic stem cell transplantation (9 matched unrelated donors, 5 matched related donors, and 3 haploidentical donors). Relapse occurred in 22% of the patients. Event free survival and overall survival rates were 53.8±12.1% and 53.6±12.1%, respectively, and they were not significantly different according to the type of induction chemotherapy (P=0.690) or the type of donor (P =0.102). @*Conclusion@#This study outlines the outcomes of pediatric AML patients with FLT3-ITD-mutations in one institution over a decade. Outcomes were significantly improved in this study compared to our previous report in 2004, where RFS and EFS were 0%. This study can provide baseline data for pediatric patients in the pre-FLT3 inhibitor era.
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Background@#Acute myeloid leukemia (AML) with internal tandem duplication in FMS-like tyrosine kinase 3 (FLT3-ITD) is associated with poor outcomes. This study aimed to analyze the outcomes of pediatric AML patients with FLT3-ITD mutations in the pre-FLT3 inhibitor era. @*Methods@#We retrospectively reviewed and identified 18 patients diagnosed with non-M3 AML with FLT3-ITD mutations at Seoul National University Children’s Hospital between May 2008 and August 2019. @*Results@#The median age was 13 years (range, 6‒19 yr). The median follow-up time was 43 months (range, 6‒157 mo). Fourteen patients received BH-AC-based (N4-Behenoy1-1-β-D-arabinofuranosy1cytosine) and 4 received cytarabine-based induction chemotherapy.Complete remission (CR) was achieved in 72.2% of the patients after the first induction chemotherapy and 80% of the patients achieved CR after salvage therapy. The overall CR rate was 94% (17/18 patients). These 17 patients underwent hematopoietic stem cell transplantation (9 matched unrelated donors, 5 matched related donors, and 3 haploidentical donors). Relapse occurred in 22% of the patients. Event free survival and overall survival rates were 53.8±12.1% and 53.6±12.1%, respectively, and they were not significantly different according to the type of induction chemotherapy (P=0.690) or the type of donor (P =0.102). @*Conclusion@#This study outlines the outcomes of pediatric AML patients with FLT3-ITD-mutations in one institution over a decade. Outcomes were significantly improved in this study compared to our previous report in 2004, where RFS and EFS were 0%. This study can provide baseline data for pediatric patients in the pre-FLT3 inhibitor era.
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BACKGROUND: Wiskott-Aldrich syndrome (WAS) is a very rare disease and patients who do not receive timely treatment suffer from bleeding, infection, and malignancy. Hematopoietic stem cell transplantation (HSCT) has been recognized as an effective treatment, but the standard transplantation protocol has not been established. We report the outcomes of WAS patients who underwent HSCT in our institution. METHODS: We retrospectively studied patients who underwent HSCT at Seoul National University Children's Hospital from 2005 to 2018. Busulfan-based myeloablative conditioning regimen was used, and an intensive daily therapeutic drug monitoring (TDM) for busulfan dosing was started for effective myeloablation and to reduce toxicity since 2008. We collected and analyzed data regarding symptoms, engraftment, transplantation-related toxicities, and survival. RESULTS: Six WAS patients who received HSCT were evaluated. The median age of the patients at diagnosis was 5 years (range, 1–11). There were 2 matched unrelated donor bone marrow transplantations, 3 matched unrelated peripheral blood stem cell transplantations (PBSCT), and 1 haploidentical PBSCT. No patient experienced engraftment failure. Three patients developed grades II to IV acute graft-versus-host disease (GVHD). Two patients had veno-occlusive disease (VOD). Two patients died (due to VOD and acute GVHD). The 5-year overall survival was 66.7% with 8 years of median follow-up. Particularly, a patient who underwent haploidentical PBSCT using targeted busulfan is alive with a follow-up duration of 3 years after HSCT. CONCLUSION: In conclusion, WAS patients may be cured with HSCT with targeted busulfan-based myeloablative conditioning. But, long-term and multi-center studies are needed.
Subject(s)
Humans , Bone Marrow , Busulfan , Diagnosis , Drug Monitoring , Follow-Up Studies , Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Hematopoietic Stem Cells , Hemorrhage , Rare Diseases , Retrospective Studies , Seoul , Stem Cell Transplantation , Unrelated Donors , Wiskott-Aldrich SyndromeABSTRACT
BACKGROUND: Androgenetic alopecia (AGA) has a negative impact on self-image and decrease in quality of life. However, relatively few men have sought treatment for AGA. Improvement in treatment willingness is important for maintaining long-term management in patients with AGA. OBJECTIVES: We aimed to identify the prevalence of patients' perception of hair loss and evaluate various factors that affect the treatment willingness in patients with AGA. METHODS: We conducted a population-based cross-sectional survey of 503 patients with AGA (329 men, 174 women). We collected the various demographic data, family history of AGA, history of past treatment, self-perception of hair loss and treatment willingness using structured questionnaires. Then, we provided the knowledge about AGA to the half of subjects and compared the treatment willingness between educated group and nond-educated group. RESULTS: Two-hundred and forty-seven out of 503 patients (49.1%) did not have illness perception and 262 out of 503 patients (52.1%) did not have treatment willingness in future. The patients with perception of hair loss, accurate information on hair loss and severe hair loss showed 1.745-fold, 1.700-fold, and 2.078-fold higher tendency of receiving treatment in future. CONCLUSION: Our findings imply that patients with perception and greater understanding of AGA tend to pursue treatment for AGA. Thus, these elements should be taken into account when treating patients. In addition, emphasis on education is needed to increase public awareness of the AGA.
Subject(s)
Humans , Male , Alopecia , Cross-Sectional Studies , Education , Hair , Prevalence , Quality of Life , Self ConceptABSTRACT
BACKGROUND: Soft tissue clear cell sarcoma is a rare tumor which originates from neural crest cells. Due to its rarity and lack of established treatment, the prognosis of clear cell sarcoma is poor. Here, we reviewed the clinical data and outcome of patients diagnosed with soft tissue clear cell sarcoma in our institution.METHODS: A retrospective study was conducted on pediatric patients who were treated for pathologically confirmed soft tissue clear cell sarcoma at the Seoul National University Hospital, between January 2000 and July 2017.RESULTS: Six patients (3 boys and 3 girls) were diagnosed with soft tissue clear cell sarcoma at a median age of 14 years 4 months (range 11 years 7 months - 19 years 3 months). The median size of the tumor was 5.6 cm (range, 0.6 cm to 7.9 cm). The most frequent symptom was pain (67%), and the most common primary site was the lower limb (67%). Three patients (50%) presented with metastases at diagnosis. Four patients underwent chemotherapy with various therapeutic combinations. Four patients received surgical resection. Only one patient received local radiotherapy. One patient died of primary refractory disease, three patients relapsed, while the remaining two survive event-free.CONCLUSION: Soft tissue clear cell sarcoma is a rare and highly aggressive tumor, for which there is no established treatment. All surviving patients received surgery, indicating that surgery is a key treatment modality. Further genetic studies of soft tissue clear cell sarcoma are needed to find a better treatment strategy.
Subject(s)
Humans , Diagnosis , Drug Therapy , Korea , Lower Extremity , Neoplasm Metastasis , Neural Crest , Pediatrics , Prognosis , Radiotherapy , Retrospective Studies , Sarcoma, Clear Cell , Seoul , Treatment OutcomeABSTRACT
Glimepiride, a third generation sulfonylurea, is an antihyperglycemic agent widely used to treat type 2 diabetes mellitus. In this study, an untargeted urinary metabolomic analysis was performed to identify endogenous metabolites affected by glimepiride administration. Urine samples of twelve healthy male volunteers were collected before and after administration of 2 mg glimepiride. These samples were analyzed by liquid chromatography-tandem mass spectrometry (LC-MS/MS), and then subjected to multivariate data analysis including principal component analysis and orthogonal partial least squares discriminant analysis. Through this metabolomic profiling, we identified several endogenous metabolites such as adenosine 3′, 5′-cyclic monophosphate (cAMP), quercetin, tyramine, and urocanic acid, which exhibit significant metabolomic changes between pre- and posturine samples. Among these, cAMP, which is known to be related to insulin secretion, was the most significantly altered metabolite following glimepiride administration. In addition, the pathway analysis showed that purine, tyrosine, and histidine metabolism was affected by pharmacological responses to glimepiride. Together, the results suggest that the pharmacometabolomic approach, based on LC-MS/MS, is useful in understanding the alterations in biochemical pathways associated with glimepiride action.
Subject(s)
Humans , Male , Adenosine , Diabetes Mellitus, Type 2 , Histidine , Insulin , Least-Squares Analysis , Mass Spectrometry , Metabolism , Metabolomics , Principal Component Analysis , Quercetin , Statistics as Topic , Tyramine , Tyrosine , Urocanic Acid , VolunteersABSTRACT
This study describes the development of an analytical method to determine sumatriptan levels in human plasma using high performance liquid chromatography (HPLC) coupled with triple quadrupole tandem mass spectrometry (MS/MS) and its application to a pharmacokinetic study in healthy Korean volunteers. A single 50 mg dose of sumatriptan was orally administered to twelve healthy volunteers (nine women and three men). The HPLC-MS/MS analytical method was validated with respect to its specificity, linearity, sensitivity, accuracy, precision, recovery, and stability. The calibration curve was linear over a concentration range of 0.3–100 ng/mL (r > 0.999). The lower limit of quantitation for sumatriptan in plasma was 0.3 ng/mL. The accuracy and precision of the analytical method were acceptable within 15% at all quality control levels. We compared plasma concentration-time curves as well as pharmacokinetic parameters such as the area under the curve (AUC) and maximum plasma concentration (C(max)). Both the mean AUC and C(max) of sumatriptan were 1.56 times higher in women than in men. These differences could be largely explained by the difference in body weight (44%) between women and men. The outcomes may provide insights into developing appropriate individualized treatment strategies.
Subject(s)
Female , Humans , Male , Area Under Curve , Body Weight , Calibration , Chromatography, Liquid , Healthy Volunteers , Methods , Plasma , Quality Control , Sensitivity and Specificity , Spectrum Analysis , Sumatriptan , Tandem Mass Spectrometry , VolunteersABSTRACT
PURPOSE: This study aimed to assess the risk of developing cardiovascular disease (CVD) and to evaluate the effects of intervention for CVD prevention in construction workers. METHODS: A total of 497 workers participated the health status survey and finally 90 workers were analyzed for evaluation of the intervention with the one-group pretest-posttest design. The data were collected from the questionnaires and health examinations in the healthcare service for road constructor's CVD prevention as secondary analysis. The intervention of the healthcare was composed of CVD prevention education for all workers and face to face counselling for the high risk group of CVD risk during 6 months in workplace. Data were analyzed with descriptive statistics, χ² test, Wilcoxon signed rank test, and paired t-test. RESULTS: The moderate and high risk groups of CVD were 9.7% and 0.8%. After the intervention, the physical activity (MET-min/week) significantly increased (Z=-5.46, p<.001). But, there were no significant differences in blood pressure, fasting glucose, body mass index, and CVD risk appraisals between pre and post intervention. CONCLUSION: The findings imply that it is necessary to develop the health promotion program for construction workers which fully reflects the characteristics of individuals and the organization.
Subject(s)
Blood Pressure , Body Mass Index , Cardiovascular Diseases , Delivery of Health Care , Education , Fasting , Glucose , Health Promotion , Motor ActivityABSTRACT
BACKGROUND: Soft tissue clear cell sarcoma is a rare tumor which originates from neural crest cells. Due to its rarity and lack of established treatment, the prognosis of clear cell sarcoma is poor. Here, we reviewed the clinical data and outcome of patients diagnosed with soft tissue clear cell sarcoma in our institution. METHODS: A retrospective study was conducted on pediatric patients who were treated for pathologically confirmed soft tissue clear cell sarcoma at the Seoul National University Hospital, between January 2000 and July 2017. RESULTS: Six patients (3 boys and 3 girls) were diagnosed with soft tissue clear cell sarcoma at a median age of 14 years 4 months (range 11 years 7 months - 19 years 3 months). The median size of the tumor was 5.6 cm (range, 0.6 cm to 7.9 cm). The most frequent symptom was pain (67%), and the most common primary site was the lower limb (67%). Three patients (50%) presented with metastases at diagnosis. Four patients underwent chemotherapy with various therapeutic combinations. Four patients received surgical resection. Only one patient received local radiotherapy. One patient died of primary refractory disease, three patients relapsed, while the remaining two survive event-free. CONCLUSION: Soft tissue clear cell sarcoma is a rare and highly aggressive tumor, for which there is no established treatment. All surviving patients received surgery, indicating that surgery is a key treatment modality. Further genetic studies of soft tissue clear cell sarcoma are needed to find a better treatment strategy.
Subject(s)
Humans , Diagnosis , Drug Therapy , Korea , Lower Extremity , Neoplasm Metastasis , Neural Crest , Pediatrics , Prognosis , Radiotherapy , Retrospective Studies , Sarcoma, Clear Cell , Seoul , Treatment OutcomeABSTRACT
BACKGROUND/OBJECTIVES: Bone formation and bone resorption continuously occur in bone tissue to prevent the accumulation of old bone, this being called bone remodeling. Osteoblasts especially play a crucial role in bone formation through the differentiation and proliferation. Therefore, in this study, we investigated the effects of Scytosiphon lomentaria extract (SLE) on osteoblastic proliferation and differentiation in MC3T3-E1 cells. MATERIALS/METHODS: A cell proliferation assay, alkaline phosphatase (ALP) activity assay, alizarin red staining and protein expression analysis of osteoblastic genes were carried out to assess the osteoblastic proliferation and differentiation. RESULTS: The results indicated that treatment of SLE promoted the proliferation of MC3T3-E1 cells and improved ALP activity. And, SLE treatment significantly promoted mineralized nodule formation compared with control. In addition, cells treated with SLE significantly upregulated protein expression of ALP, type 1 collagen, bone morphogenetic protein 2, runt-related transcription factor 2, osterix, and osteoprotegerin. CONCLUSIONS: The results demonstrate that SLE promote differentiation inducement and proliferation of osteoblasts and, therefore may help to elucidate the transcriptional mechanism of bone formation and possibly lead to the development of bone-forming drugs.
Subject(s)
Alkaline Phosphatase , Bone and Bones , Bone Morphogenetic Protein 2 , Bone Remodeling , Bone Resorption , Cell Proliferation , Collagen Type I , Osteoblasts , Osteogenesis , Osteoprotegerin , Transcription FactorsABSTRACT
We developed an ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS) method for the determination of acetaminophen concentration in human plasma. Following protein precipitated extraction, the analytes were separated and analyzed using an UPLC-MS/MS in the multiple reaction monitoring (MRM) mode with the respective [M+H]+ ions, m/z 152.06 → 110.16 for acetaminophen and m/z 180.18 → 138.12 for phenacetin (internal standard, IS). The method showed a linear response from 1 to 100 µg/mL (r > 0.9982). The limit of quantitation for acetaminophen in plasma was 1 µg/mL. The intra- and inter-day accuracy ranged in the ranges of 94.40–99.56% and 90.00–99.20%, respectively. The intra- and inter-day precision ranged in the ranges of 2.64–10.76% and 6.84–15.83%, respectively. This method was simple, reliable, precise and accurate and can be used to determine the concentration of acetaminophen in human plasma. Finally, this fully validated method was successfully applied to a pharmacokinetic study of acetaminophen in healthy volunteers following oral administration.
Subject(s)
Humans , Acetaminophen , Administration, Oral , Healthy Volunteers , Ions , Mass Spectrometry , Phenacetin , PlasmaABSTRACT
BACKGROUND: Clear cells are defined as cells that contain abundant, clear, lightly eosinophilic cytoplasm. On rare occasions, clear cell changes have been observed in actinic keratosis (AK). OBJECTIVE: We aimed to evaluate the cytoplasmic contents of clear cells and the clinicohistopathological features of clear cell AK. METHODS: From 456 cases of histopathologically confirmed AK, we selected 30 cases (6.6%) with greater than 10% of clear cells in the epidermis. We collected the demographic data and clinical features for all specimens. In addition, the specimens were divided into five histopathologic types: hypertrophic, atrophic, Bowenoid, acantholytic, and pigmented. Clear cell distribution was classified into 3 categories: upper, middle, and lower areas of the epidermis. Periodic acid-Schiff (PAS), PAS diastase (d-PAS), and alcian blue staining were carried out, and immunoperoxidase stainings were performed for S-100, HMB45, and cardio embryonic antigen (CEA) to evaluate the origin of the clear cytoplasm. RESULTS: The cheek was the most common site of involvement. The most frequent lesion color was erythematous (66.7%). Scale and telangiectasia were found in the majority of patients (90% and 66.7%, respectively). The most common histopathological type was Bowenoid, found in 21 cases. This was followed by hypertrophic in 7 cases and pigmented in 2 cases. Seventeen specimens (56.7%) stained positive for PAS and negative for d-PAS. All of the PAS-positive specimens were Bowenoid AK. All specimens stained negative for alcian blue and immunoperoxidase staining results were negative for S-100, HMB 45 and CEA. CONCLUSION: We conclude that clear cells in AK most likely originate due to an accumulation of glycogen or due to hydropic degeneration. The presence of glycogen in clear cells may induce adnexal differentiation to an atypical keratinocyte in AK.
Subject(s)
Humans , Actins , Alcian Blue , Amylases , Cheek , Cytoplasm , Eosinophils , Epidermis , Glycogen , Keratinocytes , Keratosis, Actinic , TelangiectasisABSTRACT
Heat shock protein 90 (HSP90) is involved in conformational and structural maturation of signalling molecules and transcription factors in immune reaction. HSP90 inhibitors induce immune modulation via anti-inflammatory effect, regulating humoral and cellular immune responses. Therefore, HSP90 inhibitors potentially useful target for the autoimmune disease and chronic inflammatory diseases.