• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.4
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• pp.593-599
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• 2001

Recently, tempormandibular disorder(TMD) shows an tendency to increase every year. TMD is a collective term embracing a number of clinical problems that involve the masticatory musculature, temporomandibular joint(TMJ) and associated structures, or both. TMD, viewed in distribution of age, often occurred from late teens to late twenties. But recently, the age of occurrence tends to be lower. Accordingly, early diagnosis of tempormandibular disorder is very important. In this study, we measured the maximum mouth opening which is simple and easy to carry out as a way of TMD diagnosis. In this study, the maximum mouth opening was examined for 1,775 children from 4 to 12 years of age. We compared the relationship between the maximum mouth opening with the age, height, and weight. The results obtained were summarized as follows; 1. The mean maximum mouth openings of 4, 8, and 12 year of age were respectively 40.16mm, 47.32mm, 50.54mm for male, 39.79mm, 44.85mm, 48.09mm for female. 2. The maximum mouth opening increased with age, and the values were greater in male than in female. 3. The maximum mouth opening increased with height, and the values were eater in male than in female except between 105cm and 115cm 4. The maximum mouth opening increased with weight, and the values were eater in male than in female. 5. The correlation between the maximum mouth opening with the age, height, and weight was all significant, and height showed the highest correlation.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Journal of Chest Surgery
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• v.39 no.2 s.259
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• pp.127-133
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• 2006

Background: In the studies of the distribution of time to death in trauma patients, the early deaths within several hours after injury were a large component of total trauma deaths. Due to the development of trauma system, overall mortality of trauma was decreased, but trauma is still the major cause of deaths. Material and Method: From January 1994 to December 2003, trauma patients who had been admitted and had expired at tertiary hospital were enrolled. There was a total of 400 cases, a retrospective study was done to determine the distribution of trauma mortality according to the part of the body that were severely injured part and compared the difference between early deaths within 6 hours and late deaths after 6 hours. We also analysed the risk factors of early deaths due to trauma. Result: In severe injury to the head and abdomen, the distribution of mortality was bimodal. But, in severe chest injuries, the distribution was log-shape and most early deaths were almost of trauma related. The average of GCS were 5.86$\pm$4.15 for the early deaths and 8.24$\pm$5.02 for the late deaths (p < 0.05). The AIS of thorax were 2.66$\pm$1.87 for the early deaths and 1.55$\pm$1.76 for late deaths. The risk factors for early mortality were non-EMS transportation (odds ratio 3.474), high AIS (odds ratio 1.491) and GCS (odds ratio 0.859). Conclusion: In trauma patients, the causes of early mortality were severe brain injury and massive hemorrhage. Also severe chest injuries were the major cause of the early deaths in truama. Early diagnosis of chest injury can frequently be missed in the acute trauma setting. Therefore, high index of suspicion, a careful examination, and aggressive surgical treatment are important in multiple trauma patients.

• Journal of Chest Surgery
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• v.39 no.1 s.258
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• pp.18-27
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• 2006

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly, but is one of the most common causes of myocardial ischemia which would result in high mortality within the first year of life. This is our early result of the surgical management for these patients. Material and Method: From June 1989 to July 2003, 6 patients with ALCAPA and one patient with ARCAPA (Anomalous origin of the Right coronary artery from the pulmonary artery) underwent surgical repair. We have reviewed the all medical records, electrocardiogram, chest X-ray and echocardiography retrospectively. Result: Three of the patients were boys and four were girls. The median age at the operation was 5.4 months (Range: 3$\∼$33 months). The average body weight of at the operation was 6.7 kg (Range: 3.7$\∼$11.3 kg). A mean follow up period was 18 months. Only 3 patients were initially diagnosed as ALCAPA. And 3 patients had moderate mitral regurgitation. Immediate coronary artery reimplantation on diagnosis with the aim of restoring a two-coronary system circulation was done. The average bypass time was 114$\pm$37 minutes, and the average aortic cross clamping time was 55$\pm$22 minutes. The average stay of intensive care unit was 5$\pm$3 days, the mean mechanical ventilator time was 38$\pm$45 hours and the hospital stay after operation was 12$\pm$5 days. There were significant improvements in electrocardiogram and chest X-ray of the all patients except one late death patient. The ventricular function showed almost normal recovery after operation; the EF (Ejection Fraction) increased from 41.2$\pm$ 10.3$\%$ to 60.5$\pm$ 15.8$\%$ within 1 month and to 59.8$\pm$13.9$\%$ within 1 year after operation, the SF (Shortening Fraction) increased from 23.6$\pm$4.7$\%$ to 38.6$\pm$8.4$\%$ within 1 month and to 37.4$\pm$7.9$\%$ within 1 year after operation, LVEDDI (Left Ventricular End-diastolic Dimension Index) decreased from 100.8$\pm$25.6 mm/$m^{2}$ to 90.3$\pm$ 19.2 mm/$m^{2}$ within f month and to 79.3$\pm$ 15.8 mm/$m^{2}$ within 1 year after operation. Concomitant mitral repair was done in two patients with anterior mitral leaflet prolapse. In every patient, mitral valve showed less than mild regurgitation during follow up. One late death occurred in which patient Dor procedure was applied 10 months after initial operation due to the dilated cardiomyopathy Conclusion: In the management of this rare and could be fatal Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), early suspicion and correct diagnosis is of most important. But, after diagnosis, immediate restoration of 2 coronary systems could result in good outcome.

• Journal of Chest Surgery
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• v.40 no.4 s.273
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• pp.288-291
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• 2007

Background: Myxoma makes up close to 50% of adult primary cardiac tumors, and this mainly occurs in the left atrium, and rarely in the right atrium or ventricle. The patients clinically present with symptoms of hemodynamic obstruction, embolization or constitutional changes. Diagnosis is currently established most appropriately with 2-D echocardiography. Surgical resection of myxoma is a safe and effective treatment, Material and Method: We reviewed our clinical experience in the diagnosis and management of 57 cases of cardiac myxoma that were seen over a 20-year period from July 1984 to July 2004. Result: The mean age of the patients was $53.5{\pm}14.0$ years (range: 12 to 76 years). There were 38 (67%) females and 19 (33%) males. The preoperative symptoms included dyspnea on exertion in 27 patients, palpitation in 4, chest pain in 9 and syncopal episode in 4. The diagnosis was made by echocardiography alone in 51, and by combination of echocardiography, CT and angiography in 6. The tumor attachment sites were the interatrial septum in 50, the mital valve annulus in 3 and the left atrial wall in cases, The tumor was excised successfully via biatriotomy in 33 (58%), left atriotomy in 15 (26%), the septal approach via right atriotomy in 3, Inverted T incision in 3 and the extended septal approach in 3. The follow-up time ranged from 1 to 229 months (mean follow-up: $84.0{\pm}71.3$ months). There were no early and late deaths and no recurrence during the follow-up period except for follow-up loss in 5 patients. Conclusion: It's concluded that excision of cardiac myxoma is curative and the long-term survival is excellent. Immediate surgical treatment was indicated because of the high risk of embolization or of sudden cardiac death. Radical tumor excision may prevent recurrences.

• Journal of the Korean Society of Radiology
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• v.83 no.1
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• pp.149-161
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• 2022

Purpose To investigate the correlation between computer-aided diagnosis (CAD) parameters in 3-tesla (T) MRI and pathologic immunohistochemical (IHC) markers in invasive carcinoma of no special type (NST). Materials and Methods A total of 94 female who were diagnosed with NST carcinoma and underwent 3T MRI using CAD, from January 2018 to April 2019, were included. The relationship between angiovolume, curve peak, and early and late profiles of dynamic enhancement from CAD with pathologic IHC markers and molecular subtypes were retrospectively investigated using Dwass, Steel, Critchlow-Fligner multiple comparison analysis, and univariate binary logistic regression analysis. Results In NST carcinoma, a higher angiovolume was observed in tumors of higher nuclear and histologic grades and in lymph node (LN) (+), estrogen receptor (ER) (-), progesterone receptor (PR) (-), human epidermal growth factor 2 (HER2) (+), and Ki-67 (+) tumors. A high rate of delayed washout and a low rate of delayed persistence were observed in Ki-67 (+) tumors. In the binary logistic regression analysis of NST carcinoma, a high angiovolume was significantly associated with a high nuclear and histologic grade, LN (+), ER (-), PR (-), HER2 (+) status, and non-luminal subtypes. A high rate of washout and a low rate of persistence were also significantly correlated with the Ki-67 (+) status. Conclusion Angiovolume and delayed washout/persistent rate from CAD parameters in contrast enhanced breast MRI correlated with predictive IHC markers. These results suggest that CAD parameters could be used as clinical prognostic, predictive factors.

• Journal of Chest Surgery
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• v.29 no.9
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• pp.983-988
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• 1996

Aneurysm of the sinus of Valsalva is fairly rare. Between 1987 and 1994, we operated on a total of 12 cases of aneurysm of the sinus of Valsalva at the Inje University Seoul Paik Hospital. This represents 0.7% of all cardiac operation under cardiopulmonary bypass. There were eight male and four female patients aged from 8 to 38 years(mean, 19.2 years). 8 of 12 patients had ruptured aneurysms. The origin of aneurysm of the sinus of Valsalva was the right coronary sinus in 10(83. 3%), and the noncoronary sinus in 2(16.6%). In ruptured aneurysm, the origin was the right oronary sinus in 6, and the noncoronary sinus in 2. The aneurysms originating from the right coronary sinus ruptured into the right ventricle in 5, and into both the right atrium and right ventricle in 1. The aneurysms originating from the noncoronary sinus ruptured into the right ventricle in 1, and into the right atrium in 1. Associated congenital cardiac defects included ventricular septal defect in 10(83.3%) patients 39 cases of these were associated with the aneurysms of the right coronary sinus), aortic regurgi- tation in 3 (all of these had an additional ventricular septal defect), mitral regurgitation in 1, and double chambered right ventricle in 1. No hospital deaths occurred, although one late death occurred as a result of endocarditis 15 months after the first operation. The mean follow-up period was 29 months, range from 4 to 60 months. Eleven patients except one late death were in New York Heart Association class 1. Due to the low mortality risk o( an operation for aneur sm of the sinus of Valsalva, a ruptured aneurysm of the sinus of Valsalva should be corrected surgically when the diagnosis is mane, and unruptured aneurysm of the sinus of Valsava with complication should also be operated. In most cases the aorta was opened to examine the morphology of the aneurysm and the aortic cusps, and an associated aortic valve defect should be corrected simultaneously.

• Advances in pediatric surgery
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• v.9 no.1
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• pp.45-51
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• 2003

A nationwide survey on choledochal cyst was undertaken among 39 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and the case registration form for each patient during the five year period of 1997 to 2001. Three hundred and forty eight patients were registered from 32 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1:3.4. The age of patients on diagnosis was $49.0{\pm}44.4$ months. The geographic distribution was 34.8% in Seoul and Kyoungki-do, 33.3% in Kyoungsang-do, 17.9% in Cholla-do, and 8.5% in Choongchung-do, in order of frequency. The three common clinical presentations were abdominal pain (63.8%), vomiting (35.3%), and jaundice (29.1%). Only seven patients (2%) presented with classic triad, and 25 patients were diagnosed by antenatal ultrasonographic examination. According to the Todani Classification, 238 patients (7l.3%) were type 1, 3 (0.9%) type 11, and 93 (27.8%) type IV. At the time of the operation, three important associated conditions were choledocholithiasis in 45 patients (15.1%), liver fibrosis (Grade 1-4) in 35, and previous operative procedure for biliary diseases in 10. Associated anomalies were observed in 13 patients (3.8%). Three hundred thirty nine (98.8%) of 343 lesions were treated by Cyst excision and Roux-Y hepaticoiejunostomy. One hundred seventy-six patients had an anomalous arrangement of the pancreatobiliary ductal system (APBD): APBD was not in 92 patients, biliary duct joined to the pancreatic duct in 51, and pancreatic duct joined to the biliary duct in 26. There were 8.5% early, and 7.7% late phase operative complications. The major complications were bleeding, anastomotic leakage, and acute pancreatitis. The combination of acute abdomen and choledochal cyst may suggest spontaneous rupture. Because of the development of late intrahepatic bile duct stones, long term follow up after cyst excision and hepaticojejunostomy is required. The optimal time of surgical intervention should also be considered in the situation of routine use of antenatal ultrasonographic examination. This is the first review of the choledochal cyst in Korea and provides baseline data for future comparisons.

• 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.

• Journal of Acupuncture Research
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• v.25 no.3
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• pp.127-138
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• 2008

Objectives : This study was designed to evaluate the general distribution and the efficacy of oriental medical treatment for Lumbar Disc Herniation. Subjects and Methods : The 137 patients who had a diagnosis of HIVD by Lumbar-CT of Lumbar-MRI and admitted to Dunsan oriental medical hospital in Daejon university from January 2005 to December 2007 were observed. They were analyzed according to the distribution of sex, age, the period of disease, causing factor, The patient-condition on admission, the symptom on admission, Admission day, Herniation type of the disc and the treatment efficacy was evaluated respectively. Results : 1. Male was more than female in the ratio of 1 : 1.174, and forties 28% the most, the acutest phase 48% the most, reason unknown 30% the most, Grade III 42% the most, Back and Leg radiate pain 65% the most, the day of 6-10 29% the most, protruded disc type 48% the most. 2. In the total treatment result, the good was 48%, the excellent 28%, the fair 15%, the poor 9% in order. 3. the "effective rate"(the percentage of positive effective treatment case) of each distribution, the thirties and fifties 100% the most, subacute phase 100% the most, exercise, traffic accident 100% the most respectively, Grade III 93% the most, only low back pain 97% the most, the day of 16-20 100% the most, Extruded disc 100% the most. Conclusions : Total effective rate was 91%. We have Known the efficacy of oriental medical treatment for HIVD, was good and early treatment was better than late treatment.