• Journal of the korean academy of Pediatric Dentistry
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• v.25 no.1
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• pp.225-233
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• 1998

The purpose of this study was to determine the thickness of the cortical bone at the mandibular angle in children and adolescents. The subjects were composed of 153 subjects who were 3 to 17 years of age that had not been history of bone disease or metabolic disease and no dental caries or tooth loss on mandibular molars. The subjects were divided into three age groups, group I (preschool children) was 3 to 5 years of age and grouop II (school children) was 6 to 11 years of age and group III (adolescence) was 12 to 17 years of age. Panoramic radiographs of these patients were collected and measurements of the thickness of the cortical bone were obtained at the mandibular angle. The average thickness of the cortical bone in the group I was 1.01$\pm$0.18 mm in boys and 1.07$\pm$0.22 mm in girls. In the group II was 1.23$\pm$0.18 mm in boys and 1.32$\pm$0.18 mm in girls. In the group III was 1.60$\pm$0.23 mm in boys and 1.58$\pm$0.20 mm in girls. Statistical analyses did not reveal any significant difference between the right or left side of the mandible but difference was exist between the same age groups by sex, especially in group II (p<0.01), except in group III. The greater thickness of cortical bone in the older age group was shown to be statistically significant. The correlation coefficient between age and the thickness of the cortical bone at the mandibular angle showed a high value of r=0.76.

• The Korean Journal of Nuclear Medicine
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• v.28 no.3
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• pp.377-383
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• 1994

Objective : We retrospectively analysed $^{99m}Tc$-MDP bone and $^{67}Ga$ scans to evaluate therapeutic response of bone lymphoma among patients with complete remission. Subjects and Methods : We reviewed 35 cases with an increased uptake finding $^{99m}Tc$-MDP bone scans and 16 $^{67}Ga$ scans that were follow-up studies during and after therapy. The $^{99m}Tc$-MDP bone and $^{67}Ga$ scans were graded visually from 1 to 4 in which grade 3 means same uptake density as that of normal sacroiliac articulation in bone scan and normal liver in $^{67}Ga$ scan, respectively. Results: The improvement findings during and after therapy were found in 66.0% (19/ 29) and 72.7% (24/33) with $^{99m}Tc$-MDP bone scan, 84.6% (l1/13) and 86.7% (13/15) with $^{67}Ga$ scan, respectively. The mean grades of the uptake density in $^{99m}Tc$-MDP bone scan were 3.06 before, 2.34 during, 1.75 after therapy. Those in the $^{67}Ga$ scan were 3.22 before, 1.42 during 1.30 after therapy. Conclusion. $^{67}Ga$ scans appeared more sensitive than bone scans in evaluating therapeutic response of bone lymphoma.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.13 no.2
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• pp.120-125
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• 2013

Purpose: McCune-Albright syndrome (MAS) is caused by activating mutations in the GNAS gene, resulting in peripheral precocious puberty, caf$\acute{e}$-au-lait spots, and polyostotic fibrous dysplasia (POFD). The aim of the present study was to describe the diverse clinical and endocrine characteristics of patients with MAS. Methods: Seven patients with MAS were included in this study and medical charts were reviewed retrospectively for following parameters: patient's sex and age at diagnosis, POFD, ovarian cysts, and precocious puberty. Results: The mean age at diagnosis was $5.8{\pm}4.2$ years. One patient was male (14%) and the other six patients were female (86%). Peripheral precocious puberty was associated with 6 patients (86%). Five patients manifested premature menarche as early as 2 to 5 years of age. Letrozole was administered to 4 patients, tamoxifen to one patient and GnRH agonist to one patient. Five females developed ovarian cysts. Thyroid function tests were performed in all patients and one patient showed hyperthyroidism (14%) and has been treated with methimazole. One patient presented with pseudohypoparathyroisdism, phosphaturia, calciuria suggesting hypophosphatemic rickets. Six patients (86%) revealed POFD. One patient had symptoms of optic nerve compression and secondary esotropia and 2 patients had bone pain. Conclusion: This study described clinical characteristics and endocrine complications of patients with MAS. Careful physical examinations with history taking and serial endocrine function tests should be needed to detect complications such as endocrinologic hyperfunction and POFD.

• Proceedings of the PSK Conference
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• 2003.10b
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• pp.213.3-213.3
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• 2003

Osteoporosis is the most common form of metabolic bone disease and is an important cause of morbidity and mortality in the elderly and especially in postmenopausal women. The possible beneficial action of essential fatty acids and their metabolites both in the prevention and treatment of osteoporosis needs attention. Therefore in this study, fatty acid levels of the normal groups was compared with those of patients. And then star symbol plots were drawn based on the fatty acids values of patients normalized to the corresponding normal group values to transform into visual patterns discriminating from the normal pattern. (omitted)

• Journal of Life Science
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• v.28 no.7
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• pp.874-883
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• 2018

Osteoporosis is a disease that increases the risk of fracture by decreasing the mass and strength of bone. It is caused by imbalance of osteoclast bone formation and osteoclast bone resorption. Bone formation by osteoblast is activated via bone morphogenetic proteins and runt-related transcription factor 2. $Wnt/{\beta}-catenin$ signaling and bone resorption by osteoclast are initiated by the binding of receptor activator of nuclear $factor-{\kappa}B$ ligand and receptor activator of nuclear $factor-{\kappa}B$. Menopausal women are at risk for many diseases due to hormonal imbalances, and osteoporosis is the most common metabolic disorder in 30% of postmenopausal women. When estrogen is deficient, bone resorption of osteoclasts is promoted, and the risk of osteoporosis especially increases in postmenopausal women. Hormone replacement therapy has been widely used to relieve or treat the symptoms of menopausal syndrome. However, long-term administration of hormone therapy has been associated with a high risk of side effects, such as breast cancer, ovarian cancer, and uterine cancer. Recently, phytochemicals have been actively studied as a phytoestrogen, which has an estrogen-like activity to cope with symptoms of menopausal syndrome. Therefore, in this review, we investigated the differentiation mechanism of osteoblast and osteoclast and the role of estrogen and phytoestrogen in bone metabolism in relation to previous studies.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.2
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• pp.55-62
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• 2017

Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by beta-glucosidase deficiency. An 18 month-old male with hepatosplenomegaly, anemia, thrombocytopenia, and growth retardation referred to our hospital. The patient showed neurological symptoms, such as supranuclear gaze palsy and developmental delay. Bone marrow biopsy performed to rule out malignancy and the results revealed no malignant cell; however, abnormal histiocytes suggesting storage disease was noted. Based on hepatosplenomegaly, bicytopenia and unexplained neurologic manifestations, enzyme activity and genetic analysis were conducted emergently with a strong suspicion of GD. Beta-glucosidase activity in leukocyte was decreased. GBA sequencing to confirm the diagnosis revealed compound heterozygous pathogenic variants (i.e., c.754T>A, c.887G>A), both previously reported as the cause of neuronopathic GD. Under the diagnosis of type 3 GD, the patient immediately received enzyme replacement therapy (ERT). After 17 months of ERT, the size of spleen decreased, and hemoglobin and platelet count returned to normal. In addition, the activity of chitotriosidase and angiotensin converting enzyme decreased. However, myoclonic movement and generalized seizure occurred at the age of 19 months and antiepileptic drug was started. Other neurological deterioration including supranuclear gaze palsy and developmental delay also persisted. A new therapy to overcome neurologic problems should be developed for patients with type 3 GD.

• Journal of mucopolysaccharidosis and rare diseases
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• v.4 no.1
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• pp.14-20
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• 2018

Mucopolysaccharidoses (MPSs) are a group of rare inherited metabolic disorders caused by specific lysosomal enzyme deficiencies leading to the sequential degradation of glycosaminoglycans, causing substrate accumulation in various cells and tissues and progressive multiple organ dysfunction. The rare disease medical care team at Mackay Memorial Hospital in Taiwan has been dedicated to the study of MPSs for more than 20 years. Since 1999, more than 50 academic papers focusing on MPSs have been published in international medical journals. Topics of research include the following items regarding MPSs: incidence, natural history, clinical manifestations, gene mutation characteristics, cardiac function, bone mineral density, sleep studies, pulmonary function tests, hearing assessments, percutaneous endoscopic gastrostomy, anesthetic experience, imaging analysis, special biochemical tests, laboratory diagnostics, global expert consensus conferences, prenatal diagnosis, new drug clinical trials, newborn screening, and treatment outcomes. Of these published academic research papers, more than half were cross-domain, cross-industry, and international studies with results in cooperation with experts from European, American and other Asian countries. A cross-specialty collaboration platform was established based on high-risk population screening criteria with the acronym "BECARE" (Bone and joints, Eyes, Cardiac and central nervous system, Abdomen and appearance, Respiratory system, and Ear, nose, and throat involvement). Through this platform, orthopedic surgeons, rheumatologists, ophthalmologists, cardiologists, rehabilitation physicians, gastroenterologists, otorhinolaryngologists, and medical geneticists have been educated with regards to awareness of suspected cases of MPSs patients to allow for a further confirmative diagnosis of MPSs. Because of the progressive nature of the disease, an early diagnosis and early multidisciplinary therapeutic interventions including surgery, rehabilitation programs, symptom-based treatments, hematopoietic stem cell transplantation, and enzyme replacement therapy, are very important.

• Korean Journal of Community Nutrition
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• v.15 no.3
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• pp.393-402
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• 2010

The purpose of this study was performed to evaluate the prevalence of overweight and to compare the dietary behaviors, nutrient intake and physical activities of specialized game high school students. Total of 163 subjects participated and their weight, height, waist circumference, hip circumference and bone status by a quantitative ultrasound method were measured. The subjects were surveyed by a self-administered questionnaire about general characteristics, dietary behaviors and physical activities. Nutrient intakes of the subjects were assessed by semi-quantitative food frequency questionnaire. The subjects were divided into four groups on their obesity level by BMI. The prevalence of underweight, normal, overweight and obese group was 6%, 58%, 16%, and 20% respectively. BMI was negatively correlated with bone mineral density (p < 0.01) and positively correlated with WHR (p < 0.01). The dietary guideline compliance score for "Enjoy Korean rice food style" was 2.63, followed by "Prepare food sanitarily"2.48, "Do not skip breakfast"2.29, "Eat a variety of vegetables, fruits, dairy products daily"2.25, "Drink water instead of beverage"2.10, "Choose less fried foods"2.09 and "Maintain healthy weight"1.91. The exercise frequency of walking was not significantly different between groups; however, heavy exercise frequency was significantly lower in underweight group than the other groups (p < 0.05). The energy intake was 2153 kcal, which was 81.2% of the Estimate Energy Requirement, and the intake of calcium and vitamin $B_2$ was 66.7% and 77.8% of KDRIs. Particularly, the intake of iron, vitamin A and vitamin C was about 59.4%, 52.2% and 55.4% of KDRIs and INQ was 0.71, 0.63 and 0.65 respectively, and intake of folic acid fell behind 39.1% of KDRIs and INQ was 0.46. Our study suggests that the systematic and continuous nutrition education will have to be provided at schools to improve dietary and health behaviors and prevent chronic metabolic disease for students of specialized game high school.

• Molecules and Cells
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• v.40 no.6
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• pp.393-400
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• 2017

Type 2 diabetes mellitus (T2DM) is a chronic metabolic disease characterized by lack of insulin and high glucose levels. T2DM can cause bone loss and fracture, thus leading to diabetic osteoporosis. Promoting osteogenic differentiation of osteoblasts may effectively treat diabetic osteoporosis. We previously reported that Sirtuin 1 (Sirt1), a $NAD^+$-dependent deacetylase, promotes osteogenic differentiation through downregulation of peroxisome proliferator-activated receptor (PPAR) ${\gamma}$. We also found that miR-132 regulates osteogenic differentiation by downregulating Sirt1 in a $PPAR{\beta}/{\delta}$-dependent manner. The ligand-activated transcription factor, $PPAR{\alpha}$, is another isotype of the peroxisome proliferator-activated receptor family that helps maintain bone homeostasis and promot bone formation. Whether the regulatory role of $PPAR{\alpha}$ in osteogenic differentiation is mediated via Sirt1 remains unclear. In the present study, we aimed to determine this role and the underlying mechanism by using high glucose (HG) and free fatty acids (FFA) to mimic T2DM in MC3T3-E1 cells. The results showed that HG-FFA significantly inhibited expression of $PPAR{\alpha}$, Sirt1 and osteogenic differentiation, but these effects were markedly reversed by $PPAR{\alpha}$ overexpression. Moreover, siSirt1 attenuated the positive effects of $PPAR{\alpha}$ on osteogenic differentiation, suggesting that $PPAR{\alpha}$ promotes osteogenic differentiation in a Sirt1-dependent manner. Luciferase activity assay confirmed interactions between $PPAR{\alpha}$ and Sirt1. These findings indicate that $PPAR{\alpha}$ promotes osteogenic differentiation via the Sirt1-dependent signaling pathway.

• Journal of Korean Medicine Rehabilitation
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• v.19 no.4
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• pp.135-150
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• 2009

Objectives : Osteoporosis is the most common metabolic disease of the bone, and one of the most important public health problems around the world. Obesity rate gradually increases as women grow older. The purpose of the study was to investigate the changes between bond mineral density(BMD) and body mass index(BMI) according to sex and age. Methods : We measured the bone mineral density(BMD) of lumber spine(L2-L4) and femoral neck in 10000 normal adult, using dual energy X-ray absorptiometry (DEXA; DPX-alpha). and also measured the body mass index(BMI) in 9810 normal adult. then we analysed the changes of BMD and BMI according to sex and age. Results : 1. As getting older, male's T-score of lumbar spine(L2-L4) was slowly decreased. On the other hand, female's T-score of lumbar spine was sharply dropped after 46 years old. 2. As getting older, male's T-score of femoral was slowly decreased. On the other hand, female's T-score of femoral was sharply dropped after 49 years old. 3. Irrespective of age, male's BMI keep up the extent within a definite period of numerical value. As getting older, female's BMI continued its upward trend. Conclusions : Much more attention should be needed for female patient suffering from osteoporosis and obesity than male.