• The Journal of the Korean bone and joint tumor society
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• v.16 no.1
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• pp.1-7
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• 2010

Purpose: With advances in various treatment modalities, limb salvage surgery has been commonly used in osteosarcoma of extremities. An alternative method for skeletal reconstruction is reimplantation of the tumor bearing bone following extracorporeal irradiation (ECI). We report the long-term results of ECI autograft in aspect of the oncological and functional outcomes, and complications. Materials and Methods: We retrospectively reviewed 31 osteosarcoma patients who underwent reconstruction with ECI between July 1995 and January 2006. There were 24 males and 7 females with a mean age of 24 (7-74 years) and a mean follow-up of 117 months (17-177 months). Twenty-five cases were reconstructed with ECI autograft, 6 cases with ECI autograft-prosthesis composite. The pathologic subtypes were conventional in 29 cases, periosteal in 1 case, and parosteal in 1 case. The most common location of tumor was distal femur (15 cases) followed by humerus (3), proximal fibula (3) and proximal tibia (3). Musculoskeletal Tumor Society (MSTS) score was used for functional evaluation. The overall survival rate, local recurrence, complications were analyzed. Results: The overall survival rate was 80.6% and the disease-free survival rate was 64.5%. Five patients died of distant metastasis. One patient required above-knee amputation due to local recurrence. All of them, twenty-three complications occurred, which included nonunion in 7 cases, deep infection in 5 cases, joint instability in 4 cases, metal failure in 2 cases, Limb-length discrepancy (LLD) in 2 cases, periprosthetic fracture in 1 case, epiphyseal collapse in 1, local recurrence in 1 case. The mean MSTS functional score was 62.5%. Conclusion: Extracorporeal irradiated autograft can be achieved relatively good result in aspect of oncological and functional aspect, but is needed to be additional research about occurring many complications. The reconstruction with ECI after intercalary or fragmentary resection is effective reconstruction in aspect of oncological and functional result, complications.

• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.3
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• pp.488-495
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• 2001

Treatment of class II malocclusions require distalization of maxillary molars into class I relationship. Intraarch distal molar movement techniques have recently assumed an important role in young patients. In this study, the dental and skeletal effects of the pendulum appliance were evaluated by means of cephalometric radiographs. The samples were consisted of 19 patients: 11 females and 8 males, mean age $11.68{\pm}1.52$ years. Measurements were obtained from cephalometric prior to and the day of removal of the pendulum appliance. Treatment changes were analyzed. The following results were obtain. 1. The pendulum appliance produced $2.94{\pm}1.54mm$ distal molar movement with a mean intrusion of $1.17{\pm}0.97mm$, mean period $18.13{\pm}7.95$ weeks. 2. The anchor tooth was $1.34{\pm}1.40mm$ forward movement and $0.48{\pm}0.99mm$ extrusion, and labial tilting of incisors. 3. The angle between palatal plane and mandibular plane increased significantly. 4. There was no significant difference in according to 2nd molar position. 5. Total movement was consisted of 74% distal movement of 1st molar and 26% forward movement of the anchor tooth.

• The korean journal of orthodontics
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• v.32 no.5 s.94
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• pp.327-342
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• 2002

Soft tissue profile is a critical area of interest in the development of an orthodontic treatment and diagnosis. The purpose of this study was to determine the facial profile preference of diversified group and to investigate the relationship between most Preferred facial Profile and existing soft tissue reference lines. A survey instrument of constructed facial silhouettes was evaluated by 894 lay person. The silhouettes had varied nose, lips, chin and soft tissue subnasale point. Seven sets of facial type were computer-generated by an orthodontist to represent distinct facial types. The varied facial profiles were graded on the basis of most preferred to least preferred. Every facial profile were measured by soft tissue reference lines(Ricketts E-line, Burstone B-line) to observe the most preferred facial profile. The results as follows: 1. In reliability test, the childhood group showed lower value than other groups, which means that this group has no concern on facial profile preference. 2. It appears that sexual and age difference made no significant difference in selecting the profile 3. An agreement to least preferred facial profile was higher than an agreement to most preferred facial profile. 4. Coefficient of concordance (Kendall W) was higher in the twentieth group. It means that a profile preference of the twentieth is distinct. 5. A lip protrusion (to Ricketts E-line and Burstone B-line) of most preferred facial profile was similar to measurements of previous study that investigate skeletal and soft tissue of esthetic facial profile of young Korean. So these reference lines can be used valuably in clinics. 6. Profile of excessive lip protrusion or retrusion to E-line & B-line was least preferred. 7. Most preferred profile of all respondents group was straight profile. Profile that showing convex profile was not pre(erred and the least preferred profile was concave profile.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.3
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• pp.99-106
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• 2018

Mucolipidosis type III (pseudo-Hurler polydystrophy) is a mucolipids degrading disorder caused by a mutation in the GNPTAB gene and is inherited by autosomal recessive. It is diagnosed by examining highly concentrated mucolipids in blood and the diagnosis can be confirmed by genetic testing. Mucolipidosis type III is a rare and progressive metabolic disorder. Its initial signs and symptoms usually occur around 3 years of age. Clinical manifestations of the disease include slow growth, joint stiffness, arthralgia, skeletal abnormalities, heart valve abnormalities, recurrent respiratory infection, distinctive facial features, and mild intellectual disability. Here, we are presenting two siblings of mucolipidosis type III, a 4-year-old female and a 2 years and 7 months old male with features of delayed growth and coarse face. The diagnosis was confirmed by [c.2715+1G>A(p.Glu906Leufs*4), c.2544del(p.Glu849Lysfs*22)] mutation in targeted gene panel sequencing. In this case, c.2544del is a heterozygote newly identified mutation in mucolipidosis type III and was not found in the control group including the genome aggregation database. And it is interpreted as a pathogenic variant considering the association with phenotype. Here, we report a Korean mucolipidosis type III patients with novel mutations in GNPTAB gene who have been treated since early childhood. Owing to recent development of molecular genetic techniques, it was possible to make early diagnosis and treatment with pamidronate was initiated appropriately in case 1. In addition to these supportive therapies, efforts must be made to develop fundamental treatment for patients with early diagnosis of mucolipidosis.

• Journal of Life Science
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• v.33 no.3
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• pp.277-286
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• 2023

Sarcopenia is the age-related loss of muscle mass and function. It is a natural part of aging and can lead to decreased mobility and increased frailty. The ubiquitin-proteasome pathway, which is involved in muscle protein degradation, is closely linked to sarcopenia. Germinated Rhynchosia nulubilis hydrolysate (GRH) has been reported to have anti-inflammatory and antioxidant properties, but there have been no reports on its inhibitory effect on muscle reduction. However, no study has yet explored the relationship between GRH and muscle loss inhibition. In this study, we evaluated the effects of GRH on muscle atrophy inhibitory activity in dexamethasone (Dexa)-induced muscle atrophy C2C12 myotubes and mouse models. Moreover, we identified a molecular pathway underlying the effects of GRH on skeletal muscle. May Grunwald-Giemsa staining showed that the length and area of myotubes increased in the groups treated with GRH. In addition, the GRH-treated group significantly reduced the expression of muscle ring finger protein 1 and muscular atrophy F-box (MAFbx) in the Dexa-induced muscular atrophy C2C12 model. GRH also improved muscle strength in C57BL/6 mice with Dexa-induced muscle atrophy, resulting in prolonged running exhaustive time and increased grip strength. We found that muscle strengthening by GRH was correlated with a decreased expression of the MAFbx gene in mouse muscle tissue. In conclusion, GRH can attenuate Dexa-induced muscle atrophy by inhibiting the ubiquitin-proteasome pathway via downregulation of the MAFbx gene expression.

• Journal of the Korean Orthopaedic Association
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• v.54 no.6
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• pp.547-556
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• 2019

Purpose: This study evaluated the results of surgical treatment for residual or recurrent deformity after the conservative treatment of idiopathic clubfoot. Materials and Methods: Fifty-one cases (32 patients), who were followed up to skeletal maturity, were reviewed retrospectively. The mean age at the last follow-up was 18.7 years. The surgical options included selective or comprehensive soft tissue release, tendon lengthening and transfer, and various types of osteotomy. The radiology measurements included the talocalcaneal angle and talo-first metatarsal angle in the anteroposterior (AP) view, and the talocalcaneal angle and calcaneal pitch in the lateral view. The radiology measurements were compared with the normal values for adults. The clinical evaluations were made using the ankle-hindfoot score and the midfoot score of the American Orthopaedic Foot and Ankle Society (AOFAS): excellent (>85), good (71-85), pair (56-70), and poor (<56). Results: At the last follow-up, the percentages of the 51 cases, whose parameter values fell within the normal ranges were as follows: in the AP view, 41.2% (talocalcaneal angle); and 90.2% (talo-first metatarsal angle). In the lateral view, the percentage was 84.3% (talocalcaneal angle). For the calcaneal pitch, the percentages were 61%. The mean AOFAS score was 88.1±10.7 on the ankle-hindfoot score and 86.7±11.5 on the midfoot score. Conclusion: The long-term outcome of patients with idiopathic clubfoot, who underwent surgical treatment after conservative treatment, was found to be 43%-90% of the normal range of radiographic indices. Clinically, the mean AOFAS scores were "excellent". Therefore, a satisfactory result can be obtained by analyzing the elements of deformity more accurately and then using the selective operation method, even if the non-surgical correction method fails.