• Journal of the korean academy of Pediatric Dentistry
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• v.47 no.3
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• pp.352-358
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• 2020

Glanzmann's thrombasthenia (GT) is a rare, autosomal recessive inherited congenital disorder, characterized by impaired blood coagulation due to platelet dysfunction. It was first reported by the pediatrician Glanzmann in 1918. GT affects both males and females, and it is more common in regions of the Middle East, India, and France, where intermarriage is common. It has an incidence of about 1 in 1,000,000 people. In South Korea, according to the Division of Rare Diseases, Korea Centers for Disease Control and Prevention, around 200 cases have been reported in 2018. Clinical symptoms include petechia, ecchymosis, epistaxis, and gingival bleeding. The spontaneous loss of deciduous teeth can result in excessive bleeding with that blood transfusion should be considered. Preventing hemorrhages and hemostasis are most important factors in dental treatment. Local bleeding can be controlled by compression, but platelet transfusion can be required by prolonged bleeding. Pediatric dentists can minimize the gingival bleeding by control of the oral hygiene to prevent gingivitis and dental caries. The importance of oral hygiene and periodic recall check-up should be emphasized. During dental treatment, the examination and the treatment plan of patient should be modified to prevention of hemorrhages carefully. A 6-year-old girl with GT was referred for the treatment of dental caries, and resin restoration was performed under nitrous oxide inhalation sedation. After treatment, compression was required for the bleeding control.

• Clinical and Experimental Pediatrics
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• v.51 no.5
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• pp.546-550
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• 2008

In this untreated classic phenylketonuria (PKU) case, mental retardation is severe; however, there have been individuals- like the mother of this case- who have escaped mental retardation and all the other potential sequelae of phenylketonuria, despite having high blood phenylalanine levels, and very poor dietary control. It appears that they have nearly normal brain phenylalanine levels despite high blood phenylalanine (Phe) levels. A number of studies have now demonstrated considerable variability in blood vs. brain phenylalanine levels in phenylketonuria patients. Outcome of phenylketonuria appears to be related to brain phenylalanine levels. We report a case of "undiagnosed" maternal phenylketonuria syndrome. A female infant had low birth weight (2,400 g) with microcephaly. We examined her family and discovered that her mother was an undiagnosed phenylketonuria patient with a borderline intelligence quotient (IQ). The infant's sister, six years old, was diagnosed with phenylketonuria at the age of four years was mentally retarded and had received an operation for cleft lip and palate. the sister had also had a low birth weight (2,300 g). Her sister and mother were compound heterozygotes (mother: R243Q/Y325X; sister: Y325X/P407S). The infant and father were heterozygous carriers (baby: R243Q/ -; father: P407S/ - ).

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.8 no.2
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• pp.118-121
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• 2012

Down syndrome (DS) is a genetic disease known as trisomy 21. Congenital cardiac anomalies are present in about 40% of DS patients. Dental anomalies are also common among DS patients. In DS patients, canine impaction is 10 times more frequent and transposition of maxillary canine and first premolar is 50 times more common than in general population. A female DS patient with congenital heart disease was diagnosed as having a transposed impacted maxillary canine. Sectional fixed appliance with Nance holding arch was used for the orthodontic treatment. After space was regained for the eruption of the canine, orthodontic button was attached using flap operation with closed technique. Traction and alignment of the tooth followed. To prevent endocarditis, prophylactic antibiotics were prescribed for the recommended dental procedures. Total treatment time was 25 months and no complication was found.

• Investigative Magnetic Resonance Imaging
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• v.18 no.4
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• pp.290-302
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• 2014

Purpose : Susceptibility-weighted magnetic resonance (MR) sequence is three-dimensional (3D), spoiled gradient-echo pulse sequences that provide a high sensitivity for the detection of blood degradation products, calcifications, and iron deposits. This pictorial review is aimed at illustrating and discussing its main clinical applications. Materials and Methods: SWI is based on high-resolution, 3D, fully velocity-compensated gradient-echo sequences using both magnitude and phase images. To enhance the visibility of the venous structures, the magnitude images are multiplied with a phase mask generated from the filtered phase data, which are displayed at best after post-processing of the 3D dataset with the minimal intensity projection algorithm. A total of 200 patients underwent MR examinations that included SWI on a 3 tesla MR imager were enrolled. Results: SWI is very useful in detecting multiple brain disorders. Among the 200 patients, 80 showed developmental venous anomaly, 22 showed cavernous malformation, 12 showed calcifications in various conditions, 21 showed cerebrovascular accident with susceptibility vessel sign or microbleeds, 52 showed brain tumors, 2 showed diffuse axonal injury, 3 showed arteriovenous malformation, 5 showed dural arteriovenous fistula, 1 showed moyamoya disease, and 2 showed Parkinson's disease. Conclusion: SWI is useful in detecting occult low flow vascular lesions, calcification and microbleed and characterising diverse brain disorders.

• Journal of the Korean Society of Food Science and Nutrition
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• v.45 no.8
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• pp.1099-1106
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• 2016

The objective of this study was to evaluate the immunomodulatory activity of crude polysaccharide separated from Cudrania tricuspidata leaf. C. tricuspidata polysaccharide (CTP) was extracted by ethanol precipitation. Immunomodulation activity was tested in macrophage cells (RAW 264.7 and bone-marrow derived macrophage) and splenocytes. CTP treatment significantly increased cell proliferation up to $250{\mu}g/mL$ in both RAW 264.7 and bone-marrow derived macrophages. In this concentration range (below $250{\mu}g/mL$), nitric oxide and cytokine [tumor necrosis factor $(TNF)-{\alpha}$ and interleukin (IL)-6] production also significantly increased. Similarly, splenocyte proliferation dosedependently increased except for the $1,000{\mu}g/mL$ treated group. Regarding cytokine production activity in splenocytes, CTP treatment significantly increased production of Th 1 type cytokines [interferon $(IFN)-{\gamma}$] production but not Th 2 type cytokines (IL-4). Therefore, the results indicate that CTP may have a potential effect on immunomodulatory activity in various immune cells, and this is useful for development of immune enhancing adjuvant materials as a natural ingredient.

• Journal of Korean Physical Therapy Science
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• v.5 no.3
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• pp.643-650
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• 1998

Facial nerve is subject to injury at any point in the course from the cerebral cortex to the motor end plate in the face, so many etiologic varieties of facial paralysis may be encountered, including trauma, viral infection and the idiopathic. Authors have studied 39 cases of facial paralysis which had experienced of treatment in our department from March, 1996 to March, 1997 at Dong San Medical Center. The results obtained are as follows : 1) The highest age incidence showed 10 cases(24.6%) in 3rd decade 2) Among the total of 39 cases, male were 21 cases(53.8%) and female were 18cases(46.2%). 3) At the involved side, left side were 19 cases(48.7%) and right side were 18 cases(46.2%). 4) The causes of facial paralysis were; idiopathic (Bell's palsy) in 19 cases(48.7%) infectious in 6 cases(15.4%) neoplastic in 6 cases(15.4%) traumatic in 5 cases(12.9%) metabolic in 2 cases(5.1%) congenital in 1 cases(2.6%) 5) In time interval between onset of symptom and treated initial date, 26 cases(66.7%) in below 10 days and 8 cases(20.5%) in 11-20 days 6) Correlation of recovery rate according to the treated duration did not differ significantly 7)Result after treatment were satisfactory

• Reproductive and Developmental Biology
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• v.32 no.1
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• pp.1-7
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• 2008

The SCID-repopulation cells(SRCs) assay has been widely used to determine the self-renewal capacity of hematopoietic stem cells (HSCs). In this study, we tested the repopulating efficiency of porcine bone marrow derived hematopoietic stem cells using nonobese diabetic/severe combined immunodieficient (NOD/SCID) mice which was inherited immunodeficiency mire with defect of T cells, B cells, and low activity of NK cells. We transplanted porcine bone marrow hematopoietic stem/progenitor cells with intraperitoneal injection into neonate NOD/SCID mice. We confirmed efficient reconstitution activity of inoculated porcine hematopoietis cells in variety of organs of NOD/SCID mice. Interestingly, pig $CD3^+$ T lymphocytes detected with high level in liver($15.6{\pm}3.7%$), spleen($5.6{\pm}3.0%$), thymus($1.5{\pm}1.3%$), and BM($2.3{\pm}0.9%$), respectively. These data imply that microenvironment of neonate NOD/SCID mice is very efficient for proliferation and differentiation of porcine T cells, and can be useful for the study of T cells development and renogeneic organ transplantation.

• Journal of Life Science
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• v.27 no.4
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• pp.482-499
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• 2017

Allergic diseases have increased over the past several decade worldwide including developing countries. Allergic inflammatory responses are caused by Th (T helper)2 immune responses, triggered by allergen ingestion by antigen presenting cells such as dendritic cells (DCs). Intestinal microorganisms control the metabolism and physiological functions of the host, contribute to early immune system maturation during the early life, and homeostasis and epithelial integrity during life. Bifidobacteria have strain-specific immunostimulatory properties in the Th1/Th2 balance, inhibit TSLP (thymic stromal lymphopoietin) and IgE expression, and promote Flg (Filaggrin) and FoxP3 (Treg) expression to alleviate allergies. In addition, unmethylated CpG motif ODN (oligodeoxynucleotides) is recognized by TLR (toll-like receptors)9 of B cells and plasmacytoid dendritic cells (pDCs) to induce innate and adaptive immune responses, while the butyrate produced by Clostridium butyricum activates the GPR (G-protein coupled receptors)109a signaling pathway to induce the expression of anti-inflammatory gene of pDCs, and directly stimulates the proliferation of thymically derived regulatory T (tTreg) cells through the activation of GPR43 or inhibits the activity of HADC (histone deacetylase) to differentiate naive $CD4^+$ T cells into pTreg cells through the histone H3 acetylation of Foxp3 gene intronic enhancer.

• Journal of Digital Convergence
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• v.14 no.9
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• pp.379-387
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• 2016

The purpose of this study was to examine the relationship between maternal age and infant mortality in Korea(n=617,867). Data of Korean vital statistics linked National Infant Mortality Survey conducted on births in 1999 were used in this study. The odds ratios (ORs) of infant death by maternal age were estimated with the multiple logistic regression model, adjusting for gender, plurality, birth order, low birth weight, and congenital malformation. After adjusting for the relevant variables, the odds of infant death in the group of maternal age with less than 20 years(Odds ratio [OR], 5.29, 95% confidence interval [CI], 3.51-7.98), 20-24 years(OR, 1.44, 95% CI, 1.23-1.69), 35-39 years(OR, 1.28, 95% CI, 1.11-1.46), and more than 40 years(OR, 1.94, 95% CI, 1.53-2.45) was higher than that of reference group(25-29 years). In conclusion, the higher infant mortality in the group of maternal age with less than 25 years and more than 35 years in Korea appears to be due to higher proportion of low birth weight and pre-term birth. Prenatal care intervention programs which tackle biological factors for advanced maternal age and address socio-economic problems and social stigma for early maternal age should be devised.

• Korean Journal of Food Science and Technology
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• v.49 no.4
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• pp.453-458
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• 2017

This study compared the immuno-modulatory effects of ethanol extracts (A. muricata L. ethanol extracts, ALE) and crude polysaccharide fraction (A. muricata L. crude polysaccharide fraction, ALP) from Annona muricata L. in macrophages. Immuno-modulatory activity was determined by assessing cell viability, nitric oxide (NO) production, inducible NO synthase (iNOS) expression and cytokine production in RAW 264.7 a macrophage cell line. Both ALE and ALP treatment did not affect cytotoxicity, and ALP treatment significantly increased NO production. Additionally, cytokine production [tumor necrosis factor ($TNF-{\alpha}$; $2909.04{\pm}4.1pg/mL$), interleukin (IL)-6; $662.84{\pm}5.3pg/mL$, and $IL-1{\beta}$; $852.37{\pm}2.2pg/mL$), was highly increased in the ALP ($250{\mu}g/mL$) treated group compared to the ALE ($250{\mu}g/mL$) treated group ($TNF-{\alpha}$; $1564.50{\pm}6.1pg/mL$, IL-6; $517.24{\pm}4.1pg/mL$ and $IL-1{\beta}$; $237.23{\pm}1.8pg/mL$). Moreover, ALP treatment considerably increased the expression of mitogen-activated protein kinase (MAPKs) and nuclear $factor-{\kappa}B$ ($NF-{\kappa}B$) in the macrophages. Therefore, ALP can induce macrophage activation through MAPK and $NF-{\kappa}B$ signaling and this can be a potential candidate for development of nutraceuticals.