Background: and Purpose Fatigue is common in demyelinating disorders of the central nervous system (CNS), including multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).We aimed to validate the usefulness of the Functional Assessment of Chronic Illness Therapy– Fatigue (FACIT-F) and the Fatigue Severity Scale (FSS) relative to the Korean version of the Modified Fatigue Impact Scale (MFIS-K) in Korean patients with MS, NMOSD, and MOGAD. Methods: There were 294 patients with MS (n=120), NMOSD (n=103), or MOGAD (n=71) enrolled in a prospective demyelinating CNS registry. Fatigue was measured using the FACIT-F, MFIS-K, and FSS. Sleep quality, quality of life, depression, and pain were evaluated using the Pittsburgh Sleep Quality Index (PSQI), 36-item Short-Form Survey (SF-36), and Beck Depression Inventory-II (BDI-II). Results: The MFIS-K, FACIT-F, and FSS scores showed high internal consistencies and strong correlations with each other in the MS, NMOSD, and MOGAD groups. The scores on all three fatigue scales were correlated with PSQI, SF-36, and BDI-II results in the three groups. The areas under the receiver operating characteristic curves for the FSS and FACIT-F were 0.834 and 0.835, respectively, for MS, 0.877 and 0.833 for NMOSD, and 0.925 and 0.883 for MOGAD. Conclusions These results suggest that the MFIS-K, FSS, and FACIT-F are useful and valuable assessment instruments for evaluating fatigue in Korean patients with MS, NMOSD, and MOGAD.

Langerhans cell histiocytosis(LCH) is a systemic disorder characterized by idiopathic proliferation of histiocytes in the reticuloendothelial system; CNS involvement outside the hypothalamus or pituitary gland is uncommon. We present a case of LCH involving the brainstem, cerebellum, and temporal lobes, and also showing hypothalamic involvement. The lesions were isointense or hypointense on T1WI and hyperintense on T2WI, and showed multifocal enhancing nodules on post-contrast CT and Gd-enhanced MRI.
Brain Stem ; Central Nervous System* ; Cerebellum ; Histiocytes ; Histiocytosis ; Histiocytosis, Langerhans-Cell* ; Hypothalamus ; Magnetic Resonance Imaging ; Mononuclear Phagocyte System ; Pituitary Gland ; Temporal Lobe

PURPOSE: To determine the efficacy of photodynamic therapy (PDT) with verteporfin (Visudyne(R), Norvatis Ophthalmics AG, Hettingen, Switzerland), a benzoporphyrin derivative, in the treatment of corneal neo-vascularization (CN) in a rabbit eye model. METHODS: CN was induced by placing instrastromal sutures in the cornea. Two weeks after suturing, verteporfin was administrated intravenously and 1 hour later, the right eye (treated group) was exposed to a laser with a 689 nm wavelength, and the left eye was used as the control. The changes in CN were analyzed using biomicroscopy and optical microscopy in twelve rabbits. RESULTS: The mean percentages of the neovascular area in the control and treated groups were 96.4 +/- 1.9% and 90.3 +/- 3.5% (P=0.009) at three days after the PDT, 88.6 +/- 4.6% and 71.6 +/- 6.2% (P<0.001) at one week, and 76.8 +/- 4.4% and 43.6 +/- 15.1% (P<0.001) at two weeks, respectively. Optical microscopy showed significant differences between the control and treated group in terms of the area and number of CN (P<0.05). CONCLUSIONS: PDT with verteporfin is a safe and effective procedure for regressing CN. However, a further study will be necessary.
Animals* ; Cornea ; Corneal Neovascularization* ; Microscopy ; Photochemotherapy* ; Rabbits ; Sutures

von Gierke disease (type Ia glycogen storage disease) is an inherited disease associated with accumulation of glycogen in the liver, kidney, intestine and erythrocytes due to the defect of glucose-6-phosphatase activity. Hepatomegaly, doll face, anemia, bleeding tendency and increased susceptability to infection are common features observed during infancy. Hypoglycemia especially fasting hypoglycemia is typical metabolic derangement in this disease, followed by metabolic acidosis, lactic acidemia, hyperlipidemia, hyperuricemia, and platelets dysfunction. We experienced a case of von Gierke disease in 6 month-old boy with doll face, hepatomegaly, fasting hypoglycemia, acidosis, anemia, hyperlipidemia, hyperuricemia, and acetonuria. Diagnosis was confirmed by light- and electron microscopic examination of liver biopsy specimen, which revealed hepatocytes filled with dense pools of glycogen and many lipid droplets. Cornstarch dietary therapy for him had favorable responses showing improvement of hypoglycemia, other metabolic derangements, and regression of hepatomegaly.
Acidosis ; Anemia ; Biopsy ; Diagnosis ; Erythrocytes ; Glucose-6-Phosphatase ; Glycogen ; Glycogen Storage Disease Type I* ; Hemorrhage ; Hepatocytes ; Hepatomegaly ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Infant ; Intestines ; Ketosis ; Kidney ; Liver ; Male ; Starch

von Gierke disease (type Ia glycogen storage disease) is an inherited disease associated with accumulation of glycogen in the liver, kidney, intestine and erythrocytes due to the defect of glucose-6-phosphatase activity. Hepatomegaly, doll face, anemia, bleeding tendency and increased susceptability to infection are common features observed during infancy. Hypoglycemia especially fasting hypoglycemia is typical metabolic derangement in this disease, followed by metabolic acidosis, lactic acidemia, hyperlipidemia, hyperuricemia, and platelets dysfunction. We experienced a case of von Gierke disease in 6 month-old boy with doll face, hepatomegaly, fasting hypoglycemia, acidosis, anemia, hyperlipidemia, hyperuricemia, and acetonuria. Diagnosis was confirmed by light- and electron microscopic examination of liver biopsy specimen, which revealed hepatocytes filled with dense pools of glycogen and many lipid droplets. Cornstarch dietary therapy for him had favorable responses showing improvement of hypoglycemia, other metabolic derangements, and regression of hepatomegaly.
Acidosis ; Anemia ; Biopsy ; Diagnosis ; Erythrocytes ; Glucose-6-Phosphatase ; Glycogen ; Glycogen Storage Disease Type I* ; Hemorrhage ; Hepatocytes ; Hepatomegaly ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Infant ; Intestines ; Ketosis ; Kidney ; Liver ; Male ; Starch

Recent genomewide association studies of large samples have identified genes that are associated with blood pressure. The Global Blood Pressure Genetics (Global BPgen) and Cohorts for Heart and Aging Research in Genome Epidemiology (CHARGE) consortiums identified 14 loci that govern blood pressure on a genomewide significance level, one of which is CASZ1 confirmed in both Europeans and Asians. CASZ1 is a zinc finger transcription factor that controls apoptosis and cell fate and suppresses neuroblastoma tumor growth by reprogramming gene expression, like a tumor suppressor. To validate the function of CASZ1 in blood pressure, we decreased Casz1 mRNA levels in mice by siRNA. Casz1 siRNA reduced mRNA levels by 59% in a mouse cell line. A polyethylenimine-mixed siRNA complex was injected into mouse tail veins, reducing Casz1 mRNA expression to 45% in the kidney. However, blood pressure in the treated mice was unaffected, despite a 55% reduction in Casz1 mRNA levels in the kidney on multiple siRNA injections daily. Even though Casz1 siRNA-treated mice did not experience any significant change in blood pressure, our study demonstrates the value of in vivo siRNA injection in analyzing the function of candidate genes identified by genomewide association studies.
Aging ; Animals ; Apoptosis ; Asian Continental Ancestry Group ; Blood Pressure ; Cell Line ; Cohort Studies ; Gene Expression ; Genome ; Heart ; Humans ; Kidney ; Mice ; Neuroblastoma ; RNA, Messenger ; RNA, Small Interfering ; Transcription Factors ; Veins ; Zinc Fingers

Objectives: : The purpose of this study is to identify the effect of office workers' stress level and stress vulnerability on heart rate variability (HRV), confirm the significance of objective stress indicators obtained through subjective stress and heart rate variability devices, and examine their effectiveness as an integrated stress measurement tool in community mental health projects. Methods: : From June to July in 2020, 929 workers participated in the stress management and mental health promotion project carried out by K hospital, and their recorded database was used for study analysis with their agreements. Multiple linear regression analysis was performed to find out the effects of general stress level, and stress vulnerability on heart rate variability. Results: : All general characteristics were found to have a significant effect on SDNN (ln), RMSSD (ln), LF (ln), and HF (ln), but work experience did not significantly affect RMSSD (ln). Stress level and stress vulnerability did not significantly affect heart rate variability, but stress perception, a sub-factor of stress level, was found to have a significant effect on RMSSD (ln) (β=0.118, p=0.023). Conclusions : In this study, stress perception, a sub-factor of stress level, was found to be a factor affecting RMSSD (ln). This indicates an association with the activity of parasympathetic nerves in stressful situations, and more follow-up studies are needed to use it as a direct indicator of chronic stress and integrated stress in the community mental health field.

Polyurethane (PU) has been widely examined and used for biomedical applications, such as catheters, blood oxygenators, stents, cardiac valves, drug delivery carriers, dialysis devices, wound dressings, adhesives, pacemaker, tissue engineering, and coatings for breast implants due to its mechanical flexibility, high tear strength, biocompatibility, and tailorable foams although bio-acceptability, biodegradability and controlled drug delivery to achieve the desired properties should be considered. Especially, during the last decade, the development of bio-based PUs has raised public awareness because of the concern with global plastic waste for creating more environmentally friended materials. Therefore, it is desirable to discuss polysaccharide (PS)-contained PU for the wound dressing and bone tissue engineering among biobased PUs because PS has several advantages, such as biocompatibility, reproducibility from the natural resources, degradability, ease of incorporation of bioactive agents, ease of availability and cost-effectiveness, and structural feature of chemical modification to meet the desired needs to overcome the disadvantages of PU itself by containing the PS into the PU.

Kidney transplantation is a ideal renal replacement therapy in the patient with end stage renal disease. It made improvement of the patients' life quality but made increase of the incidence of chances of malignant diseases of them. We report a 44-year-old male kidney recipient recently diagnosed as early gastric cancer, had diagnosed previously as malignant lymphoma 6 years ago. He received a kidney from his mother 13 years ago, and then 7 years later he was diagnosed as malignant lymphoma presented as 2.5 cm-sized solitary lung mass. Histologically, it was confirmed as diffuse large cell type lymphoma. After 2-year scheduled chemotherapy and radiation therapy, he achieved complete remission. He was diagnosed as early gastric cancer by routine Upper Gastro-Intestinal Series study. Histologically, it was defined as moderately differentiated adenocarcinoma. He underwent a subtotal gastrectomy (Billoth-II). After the operation, he preserved good graft function with no recurrence of malignancy until now. Briefing our case, a kidney recipient has been serially diagnosed different two kinds of malignancy, malignant lymphoma and early gastric cancer with interval of 7 years. He showed good responses to therapy of malignancies and excellent prognosis. We recommend specific schedule of regular malignancy screening test for kidney recipients with long duration of post-transplantation.
Adenocarcinoma ; Adult ; Appointments and Schedules ; Drug Therapy ; Gastrectomy ; Humans ; Incidence ; Kidney ; Kidney Failure, Chronic ; Kidney Transplantation ; Lung ; Lymphoma ; Male ; Mass Screening ; Mothers ; Prognosis ; Quality of Life ; Recurrence ; Renal Replacement Therapy ; Stomach Neoplasms ; Transplants

Since the risk of developing allergic disease increases in individuals exposed to allergens previously, even during the neonatal period, the immunologic status of a fetus may be important in the subsequent development of allergy. We evaluated the fetal factors to predict atopic dermatitis (AD) at 12 months in 412 infants of a COhort for Childhood Origin of Asthma and Allergic Diseases (COCOA) in the general Korean population. Cord blood mononuclear cells (CBMCs) were stimulated with ovalbumin and phytohemagglutinin and cellular proliferative response and concentrations of interleukin-13 and interferon-gamma, were measured. The risk of developing AD was greater in boys than girls (OR 1.97, 95% CI 1.26-3.09), infants delivered by cesarean section than vaginally (OR 1.93, 95% CI 1.14-3.26) and infants with than without parental history of AD (OR 2.34, 95% CI 1.29-4.24). The CBMC proliferative response to phytohemagglutinin stimulation was higher in infants with than without AD (P = 0.048), but no difference was observed in ovalbumin-stimulated cells (P = 0.771). Risk factors for the development of AD at 12 months include male gender, delivery by cesarean section and parental history of AD. Increased CBMC proliferative response to phytohemagglutinin stimulation may predict the development of AD at 12 months.
Adult ; Cell Proliferation ; Cesarean Section ; Dermatitis, Atopic/*diagnosis/metabolism ; Female ; Fetal Blood/*cytology/metabolism ; Humans ; Infant ; Interferon-gamma/metabolism ; Interleukin-13/metabolism ; Leukocytes, Mononuclear/drug effects/*metabolism ; Male ; Odds Ratio ; Ovalbumin/toxicity ; Phytohemagglutinins/toxicity ; Predictive Value of Tests ; Pregnancy ; Risk Factors ; Sex Factors