• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.40 no.3
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• pp.140-146
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• 2014

Pigmented villonodular synovitis (PVNS) is a benign but locally aggressive and destructive disease originating in the synovial membranes. It is a proliferative disorder of unknown etiology. Involvement of the temporomandibular joint (TMJ) is very rare. Computed tomography clearly reveals areas of lytic bone erosion and sclerosis, and also clearly defines the extent of the tumor which is the focal areas of hyperdensity within the soft-tissue mass. Magnetic resonance images invariably show profound hypointensity on both T1- and T2-weighted sequences due to hemosiderin pigmentation. Additionally, high signal intensity on T2-weighted images may indicate cystic loculation of the joint fluid. This case study describes a rare case of PVNS of the TMJ with bone destruction of the mandibular condyle. Complete surgical excision of the lesion was performed through a preauricular approach with temporal extension. During the 10-year follow-up, two more operations were performed due to local recurrence and the fracture of the reconstruction plate. Total joint reconstruction with Biomet was finally performed, and the absence of disease was confirmed with a biopsy report showing fibrosis with hyalinization and mild inflammation of the excised soft tissue from the old lesion.

• Journal of Genetic Medicine
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• v.6 no.1
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• pp.91-94
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• 2009

Werner syndrome (WRN), or adult progeria, is a very rare, autosomal recessive disorder characterized by the appearance of accelerated aging, including cataracts, gray hair, skin atrophy, and atherosclerosis. This syndrome is caused by mutations in the WRN gene and had a high risk of a spectrum of rare neoplasms including: i) non-epithelial malignant or pre-malignant tumors/conditions, osteosarcomas and soft tissue sarcomas, malignant melanomas, myeloid leukemia and myelodysplastic syndrome; ii) an epithelial neoplasm, thyroid carcinoma, and iii) meningiomas. Recently, authors experienced a case of Werner syndrome complicated by bone metastasis of rhabdomyosarcoma in a 20-year old Korean man. The patient revealed a painful mass on his right knee and progeroid features, short stature, scalp alopecia, abnormal dentition, craniofacial disproportion, hypothyroidsm, cataracts and osteoporosis. The onset of symptoms of Werner syndrome generally precedes any later symptoms of associated conditions, such as malignant tumor. Therefore, early recognition of Werner syndrome is important to assist identification of malignant tumors at an early stage in this patient group.

• The Korean Journal of Nuclear Medicine
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• v.25 no.1
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• pp.81-86
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• 1991

Reflex sympathetic dystrophy syndrome (RSDS), known also as Sudeck's atrophy, is an uncommon disorder recognized by its distinctive symptom complex consisting of pain and tenderness, vasomotor instability, swelling, and dystrophic skin changes and radiologic changes. The present study has been carried out to prospectively establish scintigraphic diagnostic criteria for RSDS using three-phase radionuclide bone scintigraphy (TPBS). In addition, the usefulness in the evaluation of treatment of RSDS was assessed. Patients included were 6 men and 7 women with the age ranging from 25 to 63 years (average 47 years). Diagnosis was based on typical clinical symptoms and signs as described above. Associated clinical conditions in these patients were cerebral infarction (4 patients), lung cancer (2 patients), trauma (1 patient), lymphoma (1 patient), and unknown cause (5 patients). All patients showed diffuse radionuclide accumulation in juxtaarticular region on the delayed static image and 11 patients showed diffusely increased activities also on scintiangiogram and blood-pool image. Fillow-up TPBS after corticosteroid therapy in 4 patients revealed near normal return of abnormal radionuclide accumulations in the affected hand. TPBS is an useful test for the diagnosis of as well as the evaluation of the therapeutic effects of RSDS.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.46 no.4
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• pp.220-227
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• 2020

Objectives: Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder. These patients lose their teeth at a young age and are in need of prosthetic rehabilitation. The aim of this systematic review was to assess the success of dental implant placement in these patients. Materials and Methods: An electronic search was performed in PubMed Central, Scopus, and Web of Science using the keyword "Papillon-Lefèvre syndrome" AND "dental implant" OR "prosthodontics". Articles reporting implant placement in patients with PLS until July 2019 were included. Results: Assessment of the included 11 articles reporting 15 cases showed 136 implant placements in these patients. Implant failure occurred in 3 patients (20 implants). The peri-implantitis and failure rate was higher in the maxilla. Meta-analysis showed the probability of failure to be 7% (95% confidence interval [CI] 0%-31%) for maxillary implants and 2% (95% CI 0%-9%) for mandibular implants. The follow-up time ranged between 1 and 20 years. Healing after bone graft and implant placement in these patients was uneventful. Conclusion: Dental implants may be a viable treatment option for PLS patients. Implantation can help preserve alveolar bone if the patients' immunological and growing conditions are well-considered and proper oral hygiene and compliance with the maintenance program are continued.

• International Journal of Oral Biology
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• v.43 no.3
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• pp.141-146
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• 2018

Periodontitis is generally a chronic disorder characterized by breakdown of tooth-supporting tissues, producing dentition loss. Porphyromonas gingivalis (P. gingivalis), a Gramnegative anaerobic rod, is one of the major pathogens associated with periodontitis. Neutrophils are first line defense cells in the oral cavity that play a significant role in inflammatory response. Xylitol is a known anti-caries agent and has anti-inflammatory effects. In this study, we conducted experiments to evaluate anti-inflammatory effects of xylitol on P. gingivalis infected neutrophils for possible usage in prevention and treatment of periodontal infections. P. gingivalis was intraperitoneally injected and peritoneal lavage was collected for cytokine determination. For in vitro study, neutrophils were collected from mouse peritoneal cells after zymosan injection or bone marrow cells. Neutrophils were stimulated with live P. gingivalis and ELISA was used to determine the effect of xylitol on P. gingivalis induced cytokine production. $IL-1{\beta}$, IL-6, $TNF-{\alpha}$ concentration and neutrophil population in the peritoneal lavage was increased in P. gingivalis-infected mouse. Peritoneal cells infected with live P. gingivalis revealed significantly increased production of $IL-1{\beta}$, IL-6 and $TNF-{\alpha}$ at multiplicity of infection of 10. Neutrophils from bone marrow and peritoneal lavage revealed increased production of $IL-1{\beta}$, IL-6 and $TNF-{\alpha}$. Xylitol significantly mitigated P. gingivalis induced cytokine production in neutrophils. Findings indicate that xylitol is an anti-inflammatory agent in neutrophils infected with live P. gingivalis, that suggests its use in periodontitis management.

• Journal of Genetic Medicine
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• v.15 no.2
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• pp.102-106
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• 2018

Aggrecan is a proteoglycan in the extracellular matrix of growth plate and cartilaginous tissues. Aggrecanopathy has been reported as a genetic cause not only for severe skeletal dysplasia but also for autosomal dominant short stature with normal to advanced bone age. We report a novel heterozygous mutation of ACAN in a Korean family with proportionate short stature identified through targeted exome sequencing. We present a girl of 4 years and 9 months with a family history of short stature over three generations. The paternal grandmother is 143 cm tall (-3.8 as a Korean standard deviation score [SDS]), the father 155 cm (-3.4 SDS), and the index case 96.2 cm (-2.9 SDS). Evaluation for short stature showed normal growth hormone (GH) peaks in the GH provocation test and a mild delayed bone age for chronological age. This subject had clinical characteristics including a triangular face, flat nasal bridge, prognathia, blue sclerae, and brittle teeth. The targeted exome sequencing was applied to detect autosomal dominant growth palate disorder. The novel variant c.910G>A (p.Asp304Asn) in ACAN was identified and this variant was found in the subject's father using Sanger sequencing. This is the first case of Korean familial short stature due to ACAN mutation. ACAN should be considered for proportionate idiopathic short stature, especially in cases of familial short stature.

• Journal of the Korean Society of Food Science and Nutrition
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• v.30 no.6
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• pp.1253-1259
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• 2001

The purpose of this study was to investigate the effects of green tea catechin on change of bone tissue in long-term cadmium treated rats. Sprague-Dawley male rats weighing 100$\pm$10 g were randomly assigned to one normal group and three cadmium treated groups. Cadmium groups were classified to catechin free diet group (Cd-0C group), 0.25% catechin diet group (Cd-0.25C group) and 0.5% catechin diet group (Cd-0.5C group) according to the levels of catechin supplement. Animals were raised for 20 weeks. Cadmium were supplied as drinking water of 50 ppm Cd$^{2+}$. Effects of catechin were analyzed on changes of bony tissue in long-term cadmium treated rats by determining the accumulated cadmium in bone and bone mineral density and micro- photographs of bony tissue. The cadmium accumulation of tibia and femur were higher in Cd-treated groups than in normal group, but they was lowered by catechin supplementation. The bone mineral density (BMD) of tibia and femur in Cd-0C group was significantly lower than in normal group, but it of catechin supplemetation group was similar to normal group. Microphological changers were appeared under a light microscope and an electro microscope reveal no structural changes in bony spicules, marrow cell distribution and cellular morphology in all groups. The bone weight and length tend to decrease in Cd-0C groups. Catechin supplementation in long-term cadmium treated rats depressed the cadmium accumulation in bony tissue that led to improve the bone mineral density in tibia and femur.r.

• The Journal of Korean Obstetrics and Gynecology
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• v.26 no.2
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• pp.46-65
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• 2013

Purpose: Generally menstrual disorder caused by functional deficiency of ovary is not treated clinically because it manifests as normal process of growth. In this study we collected experimental group having several or severe menstrual disorder and we tried to find out whether there is any relationship between menstrual disorders and the function of Thoroughfare and Conception vessels or not. Methods: First, this study was researched to learn the meaning and the symptoms of 'the emaciation of Thoroughfare and Conception vessels', and to learn the relationship between menstrual disorders and 'the emaciation of Thoroughfare and Conception vessels' through literature review. And we set up a experimental group who have two or more menstrual disorders or severe degree of menstrual disorders(N=97) and control group who don't have menstrual disorders(N=97) as a result of the menstruation survey. Afterwards we conducted the DSOM in both experimental group and control group. Results and Conclusions: The meaning of 'the emaciation of Thoroughfare and Conception vessels' is deficiency of qi and blood from the Thoroughfare and Conception vessels. As a result, the female reproductive system is difficult to be worked properly with deficiency of skin, muscles, bone, and vessels. Symptoms of the emaciation of Thoroughfare and Conception vessels are classified into drying, skinny body, feeling of cold, menstrual disorders, pain and personality traits. In this study, severity of drying, skinny body, feeling of cold, menstrual disorders, pain and personality traits is significantly higher in experimental group than in control group. Like this, symptoms of 'the emaciation of Thoroughfare and Conception vessels' appear with menstrual disorders because disorder of circulation is occurred by qi and blood deficiency of Thoroughfare and Conception vessels. It is appropriate to identify this case as 'the emaciation of Thoroughfare and Conception vessels' pattern and necessary to treat actively through regulating qi and blood in advance.

• Journal of Nutrition and Health
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• v.44 no.1
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• pp.29-40
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• 2011

The purpose of this study was to examine the association among bone mineral density (BMD), biochemical bone markers, nutrients, and salt intake in premenopausal and postmenopausal women. We evaluated 431 subjects who visited a health promotion center of a university hospital between January 2008 and July 2009. We excluded those who were taking medications or who had an endocrine disorder affecting osteoporosis. The subjects were divided into premenopausal (n = 283) and postmenopausal (n = 143) women. We evaluated the correlation among BMD of the lumbar spine, femoral neck, and total femoral, as well as biochemical bone markers, hormone, serum profiles, general characteristics, nutrient intakes, and food intake frequencies. From a stepwise multiple regression analysis, lumbar spine BMD was positively correlated with weight (p < 0.001) and negatively correlated with osteocalcin (OC)(p < 0.001), Femoral neck BMD was positively correlated with weight (p < 0.001) and negatively correlated with C-telopeptide (CTx) and alkaline phosphatase (ALP)(p < 0.001, p < 0.05). In premenopausal women, femoral total BMD was positively correlated with BMI (p < 0.001) and negatively correlated with CTx (p < 0.001). In postmenopausal women, lumbar spine BMD was positively correlated with calcium intake (p < 0.01) and negatively correlated with sodium intake (p < 0.01). Femoral neck and femoral total BMD were both positively correlated with weight (p < 0.001), and femoral neck BMD was negatively correlated with age and ALP (p < 0.001, p < 0.05). Femoral total BMD was negatively correlated with age and OC (p < 0.001, p < 0.01). These results suggest that reducing sodium intake may play an important role delaying bone resorption and preventing a decrease in BMD.

• Molecules and Cells
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• v.38 no.9
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• pp.806-813
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• 2015

Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by mutations in the glucocerebrosidase gene (GBA), which encodes the lysosomal enzyme glucosylceramidase (GCase). Deficiency in GCase leads to characteristic visceral pathology and lethal neurological manifestations in some patients. Investigations into neurogenesis have suggested that neurodegenerative disorders, such as GD, could be overcome or at least ameliorated by the generation of new neurons. Bone marrowderived mesenchymal stem cells (BM-MSCs) are potential candidates for use in the treatment of neurodegenerative disorders because of their ability to promote neurogenesis. Our objective was to examine the mechanism of neurogenesis by BM-MSCs in GD. We found that neural stem cells (NSCs) derived from a neuronopathic GD model exhibited decreased ability for self-renewal and neuronal differentiation. Co-culture of GBA-deficient NSCs with BM-MSCs resulted in an enhanced capacity for self-renewal, and an increased ability for differentiation into neurons or oligodendrocytes. Enhanced proliferation and neuronal differentiation of GBA-deficient NSCs was associated with elevated release of macrophage colony-stimulating factor (M-CSF) from BM-MSCs. Our findings suggest that soluble M-CSF derived from BM-MSCs can modulate GBA-deficient NSCs, resulting in their improved proliferation and neuronal differentiation.