• The Journal of the Korean bone and joint tumor society
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• v.14 no.1
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• pp.17-24
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• 2008

Purpose: Chondroblastoma is a rare benign bone tumor which occurs most frequently at epiphysis of long bones. This study analyzed the difference between patients with chondroblastoma either on their epiphysis or apophysis. Materials and Methods: We reviewed 19 patients with chondroblastoma who visited our hospital from August 1987 to August 2005. The mean follow up period was five years. Fifteen patients were male and 4 patients were female. The mean age of the patients was 17.6 years. The treatment consisted of either curettage alone, curettage with bone graft or curettage with cementation. We retrospectively compared the difference between one chondroblastoma originating from the epiphysis and the other chondroblastoma originating from the apophysis in terms of age predilection, duration of symptoms, size of tumor, status of the physis, presence of pathologic fracture, recurrence rate and accompanying aneurismal bone cyst. Results: Among the 19 patients, 11 patients had chondroblastoma at the epiphysis, and 8 at the apophysis. Distal femur was the most common site for epiphysis lesions while the greater trochanter was the most common site for lesions arising at the apophysis. The mean age was 14.2 years in the epiphysis group and 22.3 years in the apophysis group. Chondroblastoma occurred after closure of the physis in 3 out of 11 cases in the epiphysis group and in all 8 cases in the apophysis group showing a statistical significance between the groups in terms of status of the physis at onset. Size of the lesion was bigger in the apophysis group with statistical significance. Conclusion: Chondroblastoma is known to occur frequently at the epiphysis of long bones, but our study shows that in patients over 20 years old it occurs more frequently at the apophysis which needs to be considered when making the proper diagnosis. There was a significant difference between the two groups in terms of the age of occurrence, status of physis and size of tumor while there was none in terms of the duration of symptoms, presence of pathologic fracture, recurrence rate and presence of accompanying aneurismal bone cyst.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.75-80
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• 2005

Purpose : Internal tibial torsion is prevalent in East Asian countries such as Korea and Japan, where sitting on the floor is common behavior. Internal tibial torsion or excessive lateral tibial torsion may cause esthetical, functional, or psychological problems and also may induce degenerative arthritis in older age. The purpose of this study is to measure the tibial torsion in children of the Jeju area. Methods : Tibial torsion was measured in 1,042 lower extremities of 521 children from one to 12 years of age. The values of transmalleolar angles were analyzed for each age group divided by 6 months. Quadratic and linear regression models were used to fit patterns of changes in mean values of transmalleolar angles. The age at seven, which provides the highest coefficient of determination for quadratic regression analysis, was used as a cut-off point to fit different statistical models. Results : The mean transmalleolar angle was $0.10{\pm}5.79^{\circ}$ in all children,$ 0.90{\pm}5.49^{\circ}$ in males, and $-0.80{\pm}5.97^{\circ}$ in females. The value was $4.25{\pm}4.04$ in 1 year of age, gradually decreased to the lowest level of $-1.98^{\circ}$ in four years and seven months of age, increased again with age until it reached $0.67{\pm}1.10^{\circ}$ at seven years of age, and stayed at that level thereafter. Conclusion : Internal tibial torsion in infancy is known to correct spontaneously in the normal developing process. But in this study, the mean transmalleolar angle in children of Jeju area annually decreased after one year of age; to the lowest angle at four years and seven months of age; increased again gradually to the age of seven; and persisted in that level, about $10^{\circ}$ less than western children, not correcting further thereafter. These findings suggest tibial torsion might be caused by lifestyle, especially sitting on feet. To prevent abnormalities of joints and gaits, early diagnosis of tibial torsion in childhood and posture correction or early treatment when needed, seems to be necessary.

• Journal of Chest Surgery
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• v.29 no.7
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• pp.769-776
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• 1996

The application of video-assisted thoracic surgery (VATS) in the examination of the thoracic cavity can be a new option in patients with mediastinal tumor because it provides outstanding visibility of the structures of the mediastinum. By clear viewing through the thoracoscope, a mediastinal tumor can be biopsied or resected, depending on the findings during an operation. We reviewed all patients who underwent curative or diagnostic operations from March 1990 to August 1995 under the impression of a mediastinal mass. The total number of patients were 113 with 59 males and 54 females. Group A underwent resection of tu or by thoracotomy(38 patients: 18 males, 20 females), and group B underwent resection of tumor by VATS (36 patients : 20 males and 16 females). Seven patients in group B were excluded because they underwent thoracotomy due to pleural adhesion or intra-operative bleeding ; therefore, the true VATS group numbered 29 cases. Group C underwent Iymph node biopsy by VATS(33 patients'16 males, 17 females), and group D(6 patients: 5 males, 1 female) underwent Iymph node biopsy through anterior mediastinotomy. The mean age in group A was 36.2 years compared to 41.3 years In group B. We compared operation time, frequency of injection for pain control, duration of chest tube insertion, postoperative hospital stay, and diagnostic yield. In group A, they were 164 minutes, 3.4 times, 5.2 days, and 11.3 days, respectively, in comparison to 152 minutes, 2 times, 4.7 days, and 8.3 days, respectively, in group B. These data revealed that the day of discharge was significantly shorter in group B (p valu : 0.03). In group C, the mean age was 45.8 years (range 1 ∼70). The operation time was from 30 to 335 minutes (mean 105), pain control was required from 0 to 15 times(mean 3.2), and a chest tube was needed for 1 to 36 days (mean 6.1). In group D, mean age was 53.3 years, operation time 121 minutes, pall control injec- tion frequency 2.6 times, and mean chest tube duration 10.5 days. The diagnostic yield in group C was 8 oyo compared to 100 oyo in group D although the number of patients in group D is small.

• Clinical and Experimental Pediatrics
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• v.49 no.8
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• pp.851-856
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• 2006

Purpose : The purpose of this study is to find out the diagnostic significance of serum bile acid on total parenteral nutrition induced cholestasis in premature infants. Methods : Infants without cholestasis were classified into postnatal days and each change of serum bile acid was measured and analyzed. Also, the serum direct bilirubin, serum bile acid, ${\gamma}$-glutamic acid transferase, and alkaline phosphatase of premature infants with total parenteral nutrition induced cholestasis were measured for comparison and analysis of their correlation. Results : Changes of serum bile acid analysis after birth showed no significant difference between boys and girls, between premature infants and term infants without cholestasis. Serum bile acid levels are constant after two weeks after birth in neonates without cholestasis. In premature infants with total parenteral nutrition induced cholestasis, the increase of serum direct bilirubin over 2 mg/dL was $34.9{\pm}18.3$ days after birth, and the increase of serum bile acid was $28.1{\pm}18.3$ days. Its increase was about 1 week faster than serum direct bilirubin, however, there was no statistical significance(P=0.114). Comparing analysis of serum bile acid, ${\gamma}$-glutamic acid transferase, and alkaline phosphatase, serum bile acid showed the highest correlation to serum direct bilirubin(r=0.487, P=0.000). Conclusion : Serum bile acid is an important parameter of total parenteral nutrition induced cholestasis in premature infants and will be useful for early diagnosis and treatment.

• Journal of Gifted/Talented Education
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• v.11 no.3
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• pp.131-153
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• 2001

The importance of identifying gifted children during early childhood is becoming recognized. Nonetheless, most researchers preferred to study the primary and secondary levels where children are already and more clearly demonstrating what talents they have, and where more reliable predictions of gifted may be made. Comparatively lisle work has been done in this area. When we identify giftedness during early childhood, we have to consider the potential of the young children rather than on actual achievement. Giftedness during early childhood is still developing and less stable than that of older children and this prevents us from making firm and accurate predictions based on children's actual achievement. Dynamic assessment, based on Vygotsky's concept of the zone of proximal development(ZPD), suggests a new idea in the way the gifted young children are identified. In light of dynamic assessment, for identifying the potential giftedness of young children. we need to involve measuring both unassisted and assisted performance. Dynamic assessment usually consists of a test-intervene-retest format that focuses attention on the improvement in child performance when an adult provides mediated assistance on how to master the testing task. The advantages of the dynamic assessment are as follows: First, the dynamic assessment approach can provide a useful means for assessing young gifted child who have not demonstrated high ability on traditional identification method. Second, the dynamic assessment approach can assess the learning process of young children. Third, the dynamic assessment can lead an individualized education by the early identification of young gifted children. Fourth, the dynamic assessment can be a more accurate predictor of potential by linking diagnosis and instruction. Thus, it can make us provide an educational treatment effectively for young gifted children.

• Journal of Chest Surgery
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• v.32 no.4
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• pp.388-393
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• 1999

Background: Correct preoperative staging of esophageal cancer is a prerequisite for adequate treatment. We prospectively compared the accuracy of positron emission tomography (PET) with [fluorine-18]FDG in the staging of esophageal cancer to that of computed tomography (CT). Material and Method: The findings of FDG PET and of chest CT including lower neck and the upper abdomen of 20 biopsy-proven squamous cell carcinoma patients (male, 19; female, 1; mean age, 61) were compared with the pathologic findings obtained from a curative esophagectomy with lymph node dissection. Result: The sensitivities of FDG PET and CT for diagnosis of primary tumor were the same, 90.0% (18/20). Both FDG PET and CT failed to show the primary tumor in 2 of 20 patients; one had a 1cm sized carcinoma in situ and the other had T1 stage cancer. By using the results of the pathologic examinations of 193 removed lymph node groups, we calculated the diagnostic sensitivities, specificities and accuracies of PET and CT (*$\chi$2 p < 0.005). Sensitivity** Specificity Accuracy* PET 55.6%(30/54) 97.1%(135/139) 85.5%(165/193) CT 13.0%(7/54) 98.6%(137/139) 74.6%(144/193) One of four patients with a false-positive for PEThad had active pulmonary tuberculosis. Among the 24 tumor involved lymph node groups, PET failed to show tumor metastasis in 5 lymph node groups abutting the tumor and in 14 lymph node groups located where the decay correction was not performed. Conclusion: Based on the above findings, it is suggested that [F-18]FDG-PET is superior to CT in the detection of nodal metastases and in the staging of patients with esophageal cancer.

• Tuberculosis and Respiratory Diseases
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• v.55 no.5
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• pp.478-487
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• 2003

Background : There have been several studies showing that the angiotensin II and angiotensin converting enzyme(ACE) contributes to the apoptosis of alveolar epithelial cells in idiopathic interstitial pneumonia and the activation of fibroblasts during the process of pulmonary fibrosis. These results suggest that the pulmonary fibrosis can be inhibited by the angiotensin II receptor antagonist(AGIIRA). This study was performed to identify the therapeutic effect of AGIIRA in idiopathic pulmonary fibrosis(IPF). Method : Thirteen patients with IPF, who were diagnosed with an open lung biopsy(6 patients) and furfilling the ATS criteria(7 patients) between March 1999 and October 2001 at the Gachon medical center, were enrolled in this study. Of these patients, eight patients were treated with a regimen including AGIIRA(AT group), and five were treated without AGIIRA(NT group). The pulmonary function tests and dyspnea(ATS scale) were measured at diagnosis and 1 year after treatment. All the data was collected to analyze the therapeutic effect of AGIIRA on the patients with IPF. Results : The AT group contained 8 patients(M:F=4:4) and the NT group contained 5 patients(M:F=3:2). There was no significant difference in the serum angiotensin II level between the two groups($202.5{\pm}58.5$ vs $163.7{\pm}47.3pg/ml$, p>0.05). The AT group showed an upward trend in TLC(+3%), FVC(+4%), FEV1(+3%) and DLco(+2%) compared to the NT group(TLC(-14%), FVC(-3%), FEV1(-4%) except for DLco(+5%)). The dyspnea score in the AT group improved significantly but not in the NT group. Conclusion : These results suggest that the angiotensin II receptor antagonist may have an effect on stabilizing IPF.

• Clinical and Experimental Pediatrics
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• v.45 no.5
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• pp.646-653
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• 2002

Purpose : The incidence of type 2 diabetes mellitus in children has been increasing worldwide recently, which is thought to be related to the increasing prevalence of obesity. We investigated to evaluate the incidence and the characteristics of type 2 diabetes mellitus in children and also analysed the relationship between intrauterine growth retardation and type 2 diabetes mellitus. Methods : We investigated 25 children diagnosed as type 2 diabetes mellitus between March 1990 and December 2000. The analysis was performed retrospectively with medical records based on the clinical characteristics and laborotory findings. Results : Incidence of type 1 and type 2 diabetes mellitus in children has been increasing since 1990. We demonstrated an increase in the percentage of type 2 diabetes mellitus children from 5.3% in 1990 to 21.0% in 2000. Sixty eight percent of patients(17/25) were classified as obese group. Initial symptoms at first visit were polyuria, polydipsia and polyphagia 48%(12/25), asymptomatic glycosuria 40% (10/25), weight loss 8%(2/25) and obesity 4%(1/25). The mean age at diagnosis was $12.9{\pm}1.8$ years. 64%(16/25) of patients had positive family history of type 2 diabetes mellitus. Autoanti-bodies were positive in 18.1%(4/22) of patients. Twenty eight percent (7/25) of patients had an associated disease and two patients had fatty liver in association with obesity. Treatment consisted of diet, exercise, education and oral hypoglycemic agents. Three patients were treated with insulin as well as oral hypoglycemic agents because of poor blood glucose control. Long-term diabetic complications occurred in 4 patients. Intrauterine growth retardtion was found in 34.6%(9/25); 88.9% (8/9) of these patients were non-obese group. Conclusion : The increase in the incidence of type 2 diabetes mellitus in children is thought to be related to the increasing prevalence of obesity. The non-obese group of patients might be associated with intrauterine growth retardation.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.21-30
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• 2005

Purpose : Metabolic bone diseases have been mai or problems in children with renal diseases and steroid treatment is the main precipitating factor reducing bone mineral density(BMD). This study was performed to assess the prevalence of osteoporosis and to evaluate the clinical factors associated with decreased BMD in children with renal diseases. Methods : Forty-four children with renal diseases who were diagnosed at the Pediatric no phrology division of Ajou University hospital since Oct. 1994 were included. Using a new quantitative ultrasound device, BMD and the prevalence of osteoporosis were evaluated. The clinical and serological data were analyzed in association with decreased BMD. Results : A total of 44 patients were evaluated. The age at initial diagnosis was 6.7$\pm$4.2 years. At the time of evaluation, the chronological and bone age was 9.3$\pm$4.2 years and 8.2 $\pm$ 4.6 years, respectively. The renal diseases included nephrotic syndrome 24(54.5%), Henoch Schonlein purpura nephritis 7(15.9%), IgA nephropathy 6(13.9%), reflux nephropathy(RN) 2 (4.5%), and other renal disease 5(%). The prevalence of osteoporosis was 11%. There was no difference in the clinical factors between the long-term and the short-term treated steroid groups. Conclusion : The prevalence of osteoporosis was 12% in 44 children with renal diseases No significant factor was found in association with decreased BMD and there was no relationship between osteoporosis and steroid usage duration or cumulative dose. A new quantitative ultrasound, which is relatively easy to perform, especially in children, is expected to be in common use and will enable clinicians to evaluate metabolic bone disorders with ease.

• Journal of Life Science
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• v.29 no.7
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• pp.800-808
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• 2019

Charcot-Marie-Tooth disease (CMT) is a group of rare peripheral neuropathies characterized by progressive muscle weakness and atrophy and areflexia in the upper and lower extremities. The most common subtype of CMT is CMT1A, which is caused by a tandem duplication of the PMP22 gene in the 17p12 region. Patients with CMT1A show a loose genotype-phenotype correlation, which suggests the existence of secondary genetic or association factors. Recently, polymorphisms of rs71428439 (n.83A>G) and rs2292832 (n.86T>C) in the MIR149 have been reported to be associated with late onset and mild phenotypic CMT1A severity. The aim of this study was to examine the intrafamilial heterogeneities of clinical phenotypes according to the genotypes of these two SNPs in MIR149. For this study, we selected 6 large CMT1A families who showed a wide range of phenotypic variation. This study suggested that both SNPs were related to the onset age and severity in the dominant model. In particular, the AG+GG (n.83A>G) and TC+CC genotypes (n.86T>C) were associated to late onset and mild symptoms. Motor nerve conduction velocity (MNCV) was not related to the MIR149 genotypes. These results were consistent with the previous studies. Therefore, we suggest that the rs71428439 and rs2292832 variants in MIR149 may serve as genetic modifiers of CMT1A intrafamilial phenotypic heterogeneity, as they have a role in the unrelated patients. This is the first study to show an association using large families with variable clinical CMT1A phenotypes. The results will be helpful in the molecular diagnosis and treatment of patients with CMT1A.