• 대한치과교정학회지
• /
• 제48권2호
• /
• pp.113-124
• /
• 2018

The purpose of this case report was to introduce the concept of orthodontic and orthopedic treatment for a growing patient with Tessier number 0 cleft. A 5-year-old boy patient with Tessier number 0 cleft presented congenitally missing maxillary central incisors (MXCI), a bony defect at the premaxilla, a constricted maxillary arch, an anterior openbite, and maxillary hypoplasia. His treatment was divided into three stages: management of the bony defect at the premaxilla and the congenitally missing MXCIs using a fan-type expansion plate, iliac bone grafting, and eruption guidance of the maxillary lateral incisors into the graft area for substitution of MXCIs; management of the maxillary hypoplasia using sequential facemask therapy with conventional and skeletal anchorage; and management of the remaining occlusal problems using fixed orthodontic treatment. The total treatment duration was 15 years and 10 months. Class I canine and Class II molar relationships and normal overbite and overjet were achieved at the end of treatment. Although the long-term use of facemask therapy resulted in significant protraction of the retrusive maxilla, the patient exhibited Class III profile because of continued mandibular growth. However, the treatment result was well maintained after 2 years of retention. The findings from this case suggest that interdisciplinary and customized approaches are mandatory for successful management of maxillary hypoplasia, bony defect, and dental problems in Tessier number 0 cleft. Moreover, considering the potential of orthognathic surgery or distraction osteogenesis, meticulous monitoring of mandibular growth until growth completion is important.

• Journal of Genetic Medicine
• /
• 제15권2호
• /
• pp.102-106
• /
• 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.

• 한국전문물리치료학회지
• /
• 제29권3호
• /
• pp.187-193
• /
• 2022

Background: Neuromuscular electrical stimulation (NMES) is a physical modality used to activate skeletal muscles for strengthening. While voluntary muscle contraction (VMC) follows the progressive recruitment of motor units in order of size from small to large, NMES-induced muscle contraction occurs in a nonselective and synchronous pattern. Therefore, the outcome of muscle strengthening training using NMES-induced versus voluntary contraction might be different, which might affect balance performance. Objects: We examined how the NMES training affected balance and proprioception. Methods: Forty-four young adults were randomly assigned to NMES and VMC group. All participants performed one-leg standing on a force plate and sat on the Biodex (Biodex R Corp.) to measure balance and ankle proprioception, respectively. All measures were conducted before and after a training session. In NMES group, electric pads were placed on the tibialis anterior, gastrocnemius, and soleus muscles for 20 minutes. In VMC group, co-contraction of the three muscles was conducted. Outcome variables included mean distance, root mean square distance, total excursion, mean velocity, 95% confidence circle area acquired from the center of pressure data, and absolute error of dorsi/plantarflexion. Results: None of outcome variables were associated with group (p > 0.35). However, all but plantarflexion error was associated with time (p < 0.02), and the area and mean velocity were 37.0% and 18.6% lower in post than pre in NMES group, respectively, and 48.9% and 16.7% lower in post than pre in VMC group, respectively. Conclusion: Despite different physiology underlying the NMES-induced versus VMC, both training methods improved balance and ankle joint proprioception.

• 대한구순구개열학회지
• /
• 제2권1_2호
• /
• pp.5-14
• /
• 1999

The aim of treatment of cleft lip and palate is to correct the cleft and associated problems surgically and thus hide the anomaly so that patients can lead normal lives. This correction involves surgically producing a face that does not attract attention, a vocal apparatus that permits intelligible speech, and a dentition that allows optimal function and esthetics. In neonatal periods, gross distortion of tissues surrounding the cleft requires considerable effort and time due to post operative functional defect and scarring and induces milk feeding problem, malocclusion of deciduous or permanent dentition, congenital missing teeth, skeletal dysplasia. The occurrence of a cleft deformity is a source of considerable shock to the parents of an afflicted baby, and the most appropriate approach is very important things. Thus we tried to analysis of dental arch, shape and size of deformity in cleft patients. The results were obtained as follows. 1. When the cast measurements of UCLP subjects at first visit it was found that the mean length was 9.29mm at the alveolar cleft width, also that was 11.7mm at the anterior width and 14mm at the posterior cleft width. 2. Comparison of UCLP group at first visit and just lip surgery, it was found that the older group showed a insignificant reduction in the width of the cleft in the alveolar, canine, and tuberosity regions. 3. The maxillary casts of the UCLP group at 6 months differ Significantly from those of the at 3 months in both length and width. but there was no statistical difference except anterior ridge length of nonclefted site. 4. Comparison at 6 months and 18 months, there was a greater change in length of the alveolar cleft width, intercanine width, and anterior cleft width. Maxillary arch became wider at both the canine region and intertuberosity region. also posterior anteroposterior length was increased but anterior AP length was decreased from 8.1mm to 7.7mm. There was meaningful increase at intertuberosity length; however, a significant reduction in width t-t'

• 대한구순구개열학회지
• /
• 제4권1호
• /
• pp.13-26
• /
• 2001

Cleft lip and/or palate is the congenital orofacial malformation most commonly occurred in humans, The disease is multifactorial and is probably caused by genetic and/or environmental factors, So, there are many problems in research concerning etiology and in treatment of the disease, Even the most practiced and sophisticated methods of surgical repair are necessarily followed by scar contraction and fibrosis, which result in skeletal defects, dental abnormalities, cosmetic disfigurement, and speech impairment, As a result, Fetal surgery can be considered but practiced rarely when the deformity is not fatal to life, And treatment of cleft palate is performed in the form of medicine projection into uterus in animal experiments, Many studies show that growth factor and its receptor emerge from the developing palate; and the epidermal growth factor receptors have a important role in craniofacial development and in palatal fusion, The palatal morphogenesis of the avine is different from the mammal's; it takes the form of physiologic cleft palate, Recently, cleft palate fusion experiment was performed when the avine were in the period of palate formation through the exogenous TGF-β3 addition, and it showed that the exogenous TGF-β3 makes fusion of divided palate through certain process when cleft palate is occurred in palatal formation, In this study, I had the conformation of the fusion of cleft palate through the addition of TGF-β in case of chicken embryo, and observed the effect of TGF-β in EGF receptor distribution, And the following is the results of this study, 1. In case of the TGF-βl and β3 addition group, there was the decrease of EGFR(Epidermal Growth Factor Receptor) immunoreactivity in mesenchymal cells beneath the medial edge epithelium and also in epithelial mesenchymal interface which is between medial edge epithelium and nasal septum in 72 hours, 2, The immunoreactivity of the control group resembles that of normal chicken embryo palate in development, 3. In the view through fluorescence confocal microscopy, there was confluence in TGF-β3 addition group, This shows that the confluence induced by exogenous TGF-β3 is related to EGFR expression in palate of chicken embryo, which is a physiologic cleft palate model.

• Journal of Korean Neurosurgical Society
• /
• 제59권5호
• /
• pp.437-441
• /
• 2016

Objective : Recently, regenerative therapies have been used in clinical trials (heart, cartilage, skeletal). We don't make use of these treatments to spinal cord injury (SCI) patients yet, but regenerative therapies are rising interest in recent study about SCI. Neural precursor/stem cell (NPSC) proliferation is a significant event in functional recovery of the central nervous system (CNS). However, brain NPSCs and spinal cord NPSCs (SC-NPSCs) have many differences including gene expression and proliferation. The purpose of this study was to investigate the influence of neural growth factor (NGF) on the proliferation of SC-NPSCs. Methods : NPSCs ($2{\times}10^4$) were suspended in $100{\mu}L$ of neurobasal medium containing NGF-7S (Sigma-Aldrich) and cultured in a 96-well plate for 12 days. NPSC proliferation was analyzed five times for either concentration of NGF (0.02 and 2 ng/mL). Sixteen rats after SCI were randomly allocated into two groups. In group 1 (SCI-vehicle group, n=8), animals received 1.0 mL of the saline vehicle solution. In group 2 (SCI-NGF group, n=8), the animals received single doses of NGF (Sigma-Aldrich). A dose of 0.02 ng/mL of NGF or normal saline as a vehicle control was intra-thecally injected daily at 24 hour intervals for 7 days. For Immunohistochemistry analysis, rats were sacrificed after one week and the spinal cords were obtained. Results : The elevation of cell proliferation with 0.02 ng/mL NGF was significant (p<0.05) but was not significant for 2 ng/mL NGF. The optical density was increased in the NGF 0.02 ng/mL group compared to the control group and NGF 2 ng/mL groups. The density of nestin in the SCI-NGF group was significantly increased over the SCI-vehicle group (p<0.05). High power microscopy revealed that the density of nestin in the SCI-NGF group was significantly increased over the SCI-vehicle group. Conclusion : SC-NPSC proliferation is an important pathway in the functional recovery of SCI. NGF enhances SC-NPSC proliferation in vitro and in vivo. NGF may be a useful option for treatment of SCI patients pending further studies to verify the clinical applicability.

• 대한치과교정학회지
• /
• 제26권3호
• /
• pp.291-299
• /
• 1996

교정력은 치아이동과 악골성장을 조절하는 기계적 자극이며, 이러한 기계적 자극에 골세포가 반응하므로써 치조골과 악골의 개조가 일어난다. 이러한 기계적 자극은 크게 압축력과 인장력으로 대별된다. 따라서 본 연구는 인장력 및 압축력의 서로다른 기계적자극이 세포활성에 미치는 차이를 알아보기 위하여 조골세포주 MC3T3-E1 세포를 24well 배양접시에 well당 $2{\times}10^4$개의 세포를 넣어 배양한 후, 밀생상태가 되었을때 Diaphragm pump을 사용하여, $25g/cm^2$ 및 $300g/cm^2$의 압축력과 $-25g/cm^2$ 및 $-300g/cm^2$의 인장력을 지속적으로 가하였다. 배양한 후 각각 4일, 6일, 10일, 14일, 18일, 20일째에 ALP활성을 측정한 결과 같은 크기의 압력에서는 인장력에 비해 압축력을 가한 경우에서 ALP활성도가 증가되었으나, 세포는 기계적 자극의 양상 즉 압축력과 인장력을 구별하여 다르게 반응을 하지는 않는 것 같았다. 인장력과 압축력 모두에서 ALP활성도는 시간이 지남에 따라 대조군 수준으로 돌아왔다. 이는 기계적 자극은 세포의 증식과 분화가 왕성한 시기에 세포활성도에 더 크게 영향을 미치는 것으로 생각되며, 압축력과 인장력에 관계없이 기계적 자극의 양이 클수록 ALP활성도의 최고치 도달시간이 지연되어, 기계적 자극의 세기는 세포 활성도에 영향을 미칠 것으로 사료된다.

• Childhood Kidney Diseases
• /
• 제18권2호
• /
• pp.92-97
• /
• 2014

목적: 비스포스포네이트는 스테로이드 유발성 골다공증을 앓고 있는 환자의 치료에 널리 사용되어 왔다. 비스포스포네이트의 사용이 증가되면서 성장 중인 뼈에서 보이는 방사선학적 이상 소견에 대한 몇몇 보고가 있었다. 그래서 소아 환자에서 비스포스포네이트를 사용하는 것에 대해서는 논란의 여지가 있다. 이번 연구는 신병증을 가진 소아 환자에서 파미드로네이트 치료를 했을 때 발생한 방사선학적 이상 소견, 특히 골간단 경화성 선을 장기간 추적 관찰한 결과를 알기 위한 것이다. 방법: 경구로 칼슘제와 파미드로네이트(용량: 매일 100 mg)를 복용한 24명의 신병증 환아(평균 복용 기간: 9개월)를 후향적으로 연구하였다. 모든 대상 환아는 신병증에 대한 치료로 장기간 스테로이드 치료를 받았기 때문에 스테로이드 유발성 골다공증을 앓고 있었다. 파미드로네이트 치료 전에 장골의 방사선 사진을 촬영하였으며 수년간 추적 관찰 하였다. 뼈의 성장 속도는 검사를 시행한 시간 간격에 따라 방사선 사진 상에서 경화성 선이 이동한 거리를 측정하여 계산하였다. 결과: 평균 추적 관찰 기간은 138개월이었다. 장골의 방사선 사진 상, 모든 환자에서 골간단의 끝부분에서 뚜렷한 경화성 선이 관찰 되었으며 이 경화성 선은 성장판에서 골간쪽으로 점점 이동하였다 경화성 선의 평균 이동 속도는 6.21 mm/년이었다. 24명 중 12명의 환자에서는 경화성선이 완전히 사라졌다. 그리고 평균 키의 성장 속도는 7.33 cm/년이었다. 결론: 이번 연구에서 장기간 동안 추적 관찰한 결과 파미드로네이트 치료와 연관된 골간단부의 경화성 선은 뼈 성장에 영향을 주지 않았으며 점점 없어지는 경향을 보였다. 그러므로 신병증을 가진 소아 환자에서 스테로이드 유발성 골다공증을 비스포스포네이트로 치료하는 것은 안전한 것으로 생각된다. 그러나 좀 더 많은 수의 환자를 대상으로 하는 추가적인 연구가 필요하다.

• 대한구순구개열학회지
• /
• 제9권2호
• /
• pp.93-100
• /
• 2006

본 증례에서는 2명의 불완전, 그리고 완전구순열 환자를 Delaire의 개념에 의하여 수술을 시행 하였다. 불완전 구순열 교정은 코 교정 후 좌우 비대칭을 해소할 수 있었으나 완전구순구개열의 환아에서는 코의 비대칭성을 회복하기 위해 동시 수술을 시행하였으나 좌우 비대칭성은 수술 후에도 관찰할 수 있었다. 본 증례의 경우 환자의 경제적 그리고 사회적 이유로 인해 수술이 지연된 환자로 수술에 난이도는 비교적 높지 않았던 경우로 비강전정부위와 비익부위, 그리고 구륜근 등의 피부 하방에 비정상적으로 배열된 근육의 박리와 재위치를 이루어주었던 경우였다. 술 후 평가를 위한, 심미, 발음, 기능과 정서적 발달 정도를 검사하여야 하나 지리적 관계로 재평가가 어려운 점이 예상된다.

• Animal Bioscience
• /
• 제34권8호
• /
• pp.1392-1402
• /
• 2021

Objective: The growth rate of pigs is related to differentiation and proliferation of muscle cells, which are regulated by growth factors and expression of growth-related genes. Thus, the objective of this study was to establish optimal culture conditions for Jeju black pig (JBP) muscle cells and determine the relationship of various factors involved in muscle growth with the proliferation of JBP muscle cells. Methods: Muscles were taken from the femur skeletal muscle of JBP embryos. After isolation of the muscle cells, cells were cultured in a 6-well plate under four different culture conditions to optimize culture conditions for JBP muscle cells. To analyze proliferation rate of JBP muscle cells, these muscle cells were seeded into 6-well plates at a density of 1.5×10⁵ cells per well and cultured for 3 days. Western blot and quantitative real-time polymerase chain reaction were applied to verify the myogenic differentiation 1 (MyoD) expression and growth-related gene expression in JBP muscle cells, respectively. Results: We established a muscle cell line from JBP embryos and optimized its culture conditions. These muscle cells were positive for MyoD, but not for paired box 7. The proliferation rate of these muscle cells was significantly higher in a culture medium containing bFGF and epidermal growth factor + basic fibroblast growth factor (EGF+bFGF) than that without a growth factor or containing EGF alone. Treatment with EGF and bFGF significantly induced the expression of MyoD protein, an important transcription factor in muscle cells. Moreover, we checked the changes of expression of growth-related genes in JBP muscle cells by presence or absence of growth factors. Expression level of collagen type XXI alpha 1 gene was changed only when EGF and bFGF were added together to culture media for JBP muscle cells. Conclusion: Concurrent use of EGF and bFGF increased the expression of MyoD protein, thus regulating the proliferation of JBP muscle cells and the expression of growth-related genes.