• Childhood Kidney Diseases
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• 제25권2호
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• pp.117-121
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• 2021

Chronic kidney disease (CKD)-mineral and bone disorder (CKD-MBD) is a common complication of CKD, often accompanied by extra-skeletal calcification in adult patients. As increased vascular calcification is predicted to increase cardiovascular mortality and morbidity, the revised Kidney Disease: Improving Global Outcomes guidelines recommend avoiding calcium-containing phosphate chelators. However, extra-skeletal calcification is less commonly noticed in pediatric patients. Here, we report our experience of such a complication in pediatric patients receiving maintenance peritoneal dialysis. Extra-skeletal calcification was noticed at the corneas, pelvic cavity, and soft tissues of the lower leg in 4 out of 32 patients on maintenance peritoneal dialysis. These patients experienced the aggravation of extra-skeletal calcifications during peritoneal dialysis, and 2 of them underwent excisional operations. It is required to monitor extra-skeletal calcifications in children on kidney replacement therapy.

• Journal of mucopolysaccharidosis and rare diseases
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• 제2권1호
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• pp.5-7
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• 2016

Mucolipidoses II and III alpha/beta (ML II and ML III) are lysosomal disorders in which the essential mannose-6-phosphate recognition marker is not synthesized onto lysosomal hydrolases and other glycoproteins. The disorders are caused by mutations in GNPTAB, which encodes two of three subunits of the heterohexameric enzyme, N-acetylglucosamine-1-phosphotransferase ML II, recognizable at birth, often causes intrauterine growth impairment and sometimes the prenatal "Pacman" dysplasia. The main postnatal manifestations of ML II include gradual coarsening of neonatally evident craniofacial features, early cessation of statural growth and neuromotor development, dysostosis multiplex and major morbidity by hardening of soft connective tissue about the joints and in the cardiac valves. Fatal outcome occurs often before or in early childhood. ML III with clinical onset rarely detectable before three years of age, progresses slowly with gradual coarsening of the facial features, growth deficiency, dysostosis multiplex, restriction of movement in all joints before or from adolescence, painful gait impairment by prominent hip disease. Cognitive handicap remains minor or absent even in the adult, often wheelchair-bound patient with variable though significantly reduced life expectancy. As yet, there is no cure for individuals affected by these diseases. So, clinical manifestations and conservative treatment is important. This review aimed to highlight the extra-skeletal clinical problems in ML II and III.

• 대한치과교정학회지
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• 제51권6호
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• pp.387-396
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• 2021

Objective: To identify optimal areas for the insertion of extra-alveolar miniscrews into the infrazygomatic crest (IZC) and mandibular buccal shelf (MBS), using cone beam computed tomography (CBCT) imaging in patients with different craniofacial patterns. Methods: CBCT reconstructions of untreated individuals were used to evaluate the IZC and MBS areas. The participants were divided into three groups, based on the craniofacial pattern, namely, brachyfacial (n = 15; mean age, 23.3 years), mesofacial (n = 15; mean age, 19.24 years), and dolichofacial (n = 15; mean age, 17.79 years). In the IZC, the evaluated areas were at 11, 13, and 15 mm above the buccal cusp tips of the right and left first molars. In the MBS, the evaluated areas were at the projections of the first molars' distal roots and second molars' mesial and distal roots, at a 4- and 8-mm distance from the cementoenamel junction. Intergroup comparisons were performed with analysis of variance and the Tukey test. Results: There was no statistically significant difference in the IZC bone thickness among the groups. For MBS bone availability, some comparisons revealed no difference; meanwhile, other comparisons revealed increased MBS bone thickness in the brachyfacial (first molars distal roots) and dolichofacial (second molars mesial and distal roots) patterns. Conclusions: There was no significant difference in the IZC bone thickness among the groups. The facial skeletal pattern may affect the availability of ideal bone thickness for the insertion of extra-alveolar miniscrews in the MBS region; however, this variability is unlikely to be clinically meaningful.

• 대한골관절종양학회지
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• 제8권2호
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• pp.48-53
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• 2002

상피암의 인접 골격근으로의 직접 전파는 많이 보고되었으나, 골격근으로의 원격전이는 드물고 특히 다발성 전이는 매우 드문 것으로 알려져 있다. 골격근내의 가변적인 혈류량, 근수축이나 난류성 혈류 등과 같은 물리적인 요인, 골격근내의 산성 환경, 젖산, 세포외 기질의 단백분해효소 억제인자 등이 골격근으로의 원격 전이를 억제하는 원인으로 제시되고 있다. 저자들은 최근 다발성 골격근 전이를 동반한 신장암(이행성 상피세포암) 1례를 경험하였기에 이를 보고하는 바이다.

• 대한소아치과학회지
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• 제37권4호
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• pp.537-544
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• 2010

교정치료에 있어서 고정원은 진단 및 치료계획에서부터 치료 종료 단계까지 항상 염두에 두어야 하는 중요한 요소이다. 전통적으로 고정원의 조절을 위하여 차등력, 차등모멘트, 구내 고정원, 구외 고정원 등의 다양한 방법들이 사용되어져 왔다. 그러나 이러한 방법들은 원치 않는 치아의 이동이 발생할 수 있고 환자의 협조도가 필요하다는 한계가 있다. 따라서 환자의 협조도나 주변 치아에 의존하지 않는 골격성 고정원(skeletal anchorage)이 전통적인 방법들의 단점을 극복할 수 있는 대안으로 제시되었다. 골격성 고정원의 종류로는 implant, onplant, miniplate, miniscrew 등이 있다. 이 중에서 miniscrew는 환자의 협조도 감소, 술식의 간편성, 저렴한 비용, 식립부위의 다양성 등의 장점을 가지고 있어 교정치료 시 유용하게 이용될 수 있다. 본 증례는 이소성 맹출 경로를 보이는 상악 견치와 매복된 하악 견치의 견인, 정출된 상악 전치의 압하에 miniscrew를 이용하여 양호한 결과를 보였기에 보고하는 바이다.

• 구강회복응용과학지
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• 제26권3호
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• pp.349-357
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• 2010

골격성 III급 부정교합을 가진 환자에서의 보철 치료는 환자의 전신적요인과 기타 구강내, 외적인 요인을 고려하여 정확한 치료 계획하에 시행되어야 한다. 성인에 있어서 III급 악골관계는 외과적 수술과 교정치료가 선행된 후 보철수복이 진행되도록 계획하는 경우가 일반적이지만 환자의 전신적 요인 등으로 인하여 수술이 불가능한 경우가 있을 수 있다. 이러한 경우에서는 보철로 인한 저작기능의 회복시 좀 더 안정적으로 교합을 유지, 지속할 수 있는 치료가 필요하다. 본 증례에서는 전신질환으로 인하여 수술이 불가한 III급 악골관계를 가진 환자에서 고정성 보철 수복을 통하여 경제적, 시간적으로 효율적이고 기능과 심미를 충족시키는 임상적 결과를 얻었기에 보고하고자 한다.

• 대한골관절종양학회지
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• 제4권1호
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• pp.22-29
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• 1998

A limb-sparing operation has a definitive role in the treatment of osteosarcoma in the lower extremity of skeletally-immature patients. After a limb-sparing operation, leg length discrepancy remains as a major disability that should be corrected. This study was designed to suggest methods of tumor resection and proper timing of leg length equalization in skeletally immature osteosarcoma patients. From September 1990 to January 1998, we reviewed eight osteosarcoma patients in an immature skeletal age. There were 4 males and 4 females, and their mean duration of follow-up was 50.37 months (range : 25 to 88 months). Mean skeletal age was 8 years (range : 8 months to 11 years). The patients were classified according to the methods of tumor resection ; intercalary resection in 1 case, transepiphyseal resection in 1, intra-articular resection in 5, and extra-articular resection in 1. The results were as follows ; 1. The leg lengthening was begun when a patient's leg length discrepancy reached 4-5cm. 2. The age of final lengthening with permanent reconstruction was 14 years in males and 12 years in females (about 2 years before skeletal maturity). 3. When reconstruction was performed with a temporary spacer, the site of lengthening Was in the soft tissue, not in bone, and then a permanant reconstruction was done. 4. Reconstruction with a biologic spacer to preserve the joint function was a reasonable method for equalization of leg length. In conclusion, the appropriate choice of reconstructive method and the age at which to correct the leg length discrepancy in a skeletally-immature osteosarcoma patients are important factors for maintaining leg length at full maturity.

• Journal of Korean Biological Nursing Science
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• 제12권1호
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• pp.1-7
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• 2010

Purpose: This research is to promote an walking exercise program for the subjects to continue exercise by mitigating stresses with increase in increase their physiological index. Methods: The research design was a non-equivalent control group, quasiexperimental study. The research has been executed from August 1st to September 30th 2008 by operating walking exercise program to 50 subjects. Only 24 patients who continued this program to the last were could be investigated. Results: Among the subjects who participated in the program had shown significant differences in weight and BMI. Despite of an insignificant change in skeletal muscle mass, fat mass, body fat ratio and WHR, average value for skeletal muscle mass showed an increasing tendency, while the average values for fat mass, body fat ratio and WHR showed a decreasing tendency. Conclusion: In conclusion, walking exercise program was design for the subjects to be able to exercise without an extra cost for a special tool to exercise with an positive effect on increasing health problem.

• 대한치과교정학회지
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• 제19권1호
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• pp.219-243
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• 1989

The purpose of this study was to evaluate the effects of the chincap therapy on the craniofacial structure in persons with skeletal Class III malocclusion. The patients selected for this study were treated with extra-oral chincap therapy only. Both control and treatment samples were obtained from Seoul National University Hospital where these longitudinal data were gathered. 55 treated patients and 14 control patients were studied. The mean ages at the 1st evaluation was 8 years 3 months in the treatment sample and 9 years 4 months in the control sample. The duration of chincap therapy was variable but averaged 2 years of treatment. Post-treatment observation procedeeded for 1 year 2 months. Active treatment and post treatment effects were evaluated. The results were as follows: 1. Neither significant restraint nor acceleration of growth was found in the cranial base and maxilla during treatment. 2. A distal rotation of the mandibular complex was seen. 3. Some amount of restraint of growth was found in mandibular body length, ramus height, mandibular length during treatment. 4. The genial angle was reduced. 5. After removal of the chin-cap, forward displacement of the mandible took place.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제37권
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• pp.29.1-29.7
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• 2015

Facial asymmetry is found in patients with or without cosmetic facial alterations. Some patients have facial asymmetry that manifests underlying skeletal problems, while others have only limited soft-tissue facial asymmetry. Orthognathic surgery brings about a dermatic change, as soft tissue covers underlying bones. Limited soft-tissue asymmetry, meanwhile, is difficult to correct. The treatment modalities for the creation or restoration of an esthetically pleasing appearance were autogenous fat grafts, cartilage graft, and silicon injections. A young female patient had right-side facial asymmetry. The clinical assessment involved visual inspection of the face and palpation to differentiate soft tissue and bone. Although the extra-oral examination found facial asymmetry with skin atrophy, the radiographic findings revealed no mandibular atrophy or deviation. She was diagnosed as localized scleroderma with muscle spasm. In conclusion, facial asymmetry patients with skeletal asymmetry can be esthetically satisfied by orthognathic surgery; however, facial atrophy patients with skin or subdermal tissue contraction need treatment by cosmetic dermatological surgery and orthodontic correction.