• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.2
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• pp.39-44
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• 2023

Hereditary tyrosinemia type 1 (HT-1) is a metabolic disorder caused by biallelic pathogenic variants in the fumarylacetoacetate hydrolase (FAH) gene, which impairs the function of the FAH enzyme, resulting in the accumulation of tyrosine's toxic metabolites in hepatocytes and renal tubular cells. As a consequence, individuals with HT-1 exhibit symptomatic manifestations. Rapid diagnosis and treatment of HT-1 can prevent short-term death and long-term complications. A 15-day-old boy presented to the outpatient department with elevated levels of tyrosine on his newborn screening tests conducted at the age of 3 and 10 days, respectively. Further blood tests revealed increased levels of alpha-fetoprotein and amino acids including tyrosine and threonine. Urine organic acid tests indicated a significant elevation in tyrosine metabolites, as well as the presence of succinylacetone (SA), which led to the diagnosis of HT-1. Two pathogenic and likely pathogenic variants of FAH compatible with HT-1 were also detected. He began a tyrosine-restricted diet at one month old and received nitisinone (NTBC) at two months old. With continued treatment, the patient's initially elevated AFP level, detection of SA in the urine, and mild hepatomegaly showed improvement. During four years and seven months of treatment, there were no exceptional complications apart from an increase in tyrosine levels and a delay in speech. We report a case of tyrosinemia type 1 detected through newborn screening, treated with dietary restriction and NTBC, with a good prognosis.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.194-198
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• 2009

Purpose : Active perioperative intervention and improvement on surgical technique has decreased the mortality rate of total anomalous pulmonary venous connection (TAPVC); however, when complicated with pulmonary venous obstruction, operative mortality is still high. The purpose of this study was to investigate the clinical course of TAPVC. Methods : Twenty-seven patients who were diagnosed with TAPVC (without other complex heart anomalies) by echocardiogram at Kyungpook National University Hospital from January 1994 to February 2008 were included. Results : Mean age at diagnosis was $28.1{\pm}33.4$ days (1-126 days). Sites of drainage were supracardiac type (15), cardiac (6), infracardiac (5), and mixed (1). Seven patients had pulmonary venous obstruction: 5 with supracardiac type, 1 with cardiac, and 1 with infracardiac. Intraoperative trans-esophageal echocardiograms were performed in 14 patients (58.3%). The operative mortality was 16.7% (4 of 24) and overall hospital mortality (including deaths without operation) was 22.2% (6 of 27). There were 5 postoperative pulmonary venous obstructions. The sites of obstruction were anastomotic in 3 of 5 (60%) patients, and ostial pulmonary vein in the other 2 (40%) patients. Three patients who presented with anastomotic pulmonary venous obstruction underwent reoperation, but all the patients were found to have pulmonary venous anastomotic obstruction. The other 2 patients with ostial pulmonary vein obstruction who had no significant symptoms were diagnosed by routine echocardiographic examination during follow-up. Conclusion : In TAPVC patients, early diagnosis and aggressive surgical management will improve prognosis, and we must pay attention to early and late pulmonary vein restenosis through intraoperative trans-esophageal echocardiogram and peri- and post-operative echocardiographic follow-up examinations.

• Tuberculosis and Respiratory Diseases
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• v.43 no.1
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• pp.54-62
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• 1996

Background: $^{99m}Tc$ MIBI(Methoxyisobutylisonitrile complex), a member of the isonitrile class of coordination compounds, is a lipophilic cation presently under investigation for clinical use as myocardial perfusion imaging agent and is widely used to detect myocardial infarction. Preliminary reports indicate that $T_1$-201 accumulate in human neoplasm and several authors reported $^{99m}Tc$ MIBI may also localized in primary malignant tumor and metastatic deposits from lung cancer. We evaluated the uptake of $^{99m}Tc$ MIBI in lung cancer and localization of mediastinal and other site metastasis, and compared the benign lesion of the lung. Method: Thirty four patients of lung cancer and ten patients of benign lung lesion were studied with chest CT and $^{99m}Tc$ MIBI Lung SPECT. $^{99m}Tc$ MIBI uptake ratio was assessed by TR/NL(Lung lesion/ Normal area), HT/NL (Heart/Normal area) and HT/TR(Heart/Lung lesion). Results: 1) All lung cancer patients showed increased uptakes of $^{99m}Tc$ MIBI in malignant lung lesion and Tc-99m MIBI uptake was also increased in mediastinal and lymph node metastasis except two cases. 2) There was significant different ratio of TR/NL between malignant and benign lesion, $3.79{\pm}1.82$ and $1.67{\pm}0.63$ on planar images, respectively(p<0.001). 3) There was no significant difference of $^{99m}Tc$ MIBI uptake ratio between squamous cell carcinoma, small cell carcinoma and adeno carcinoma($3.64{\pm}1.66$, $3.57{\pm}0.72$, $4.31{\pm}2.28$ respectively). Conclusion: $^{99m}Tc$ MIBI lung SPECT was useful in the localization of tumor and mediastinal or other site metastatic lesion in lung cancer and also in the differential diagnosis between benign and malignant lesion.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.20 no.2
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• pp.133-138
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• 1998

Choanal atresia may be membrane or bony, unilateral or bilateral. Approximately 90% of the choanal atresia are bony type. Unilateral choanal atresia often eludes the diagnosis because of the absence of subjective symptoms in the neonatal period. However, bilateral choanal atresia presents at birth with cyclic respiratory distress aggravated by feedings. So complete bilateral choanal atresia is considered as a neonatal emergency. Examinations for the diagnosis of chonal atresia include 1) attempt at passing a rubber catheter or probe through the patient s nose, 2) mirror examination of the nasopharynx, 3) digital examination of the nasopharynx, 4) X-ray examination after installation of radiopaque materal into the nasal cavity. But, computed tomography has become accepted method for evaluation of choanal atresia. Surgical repair of choanal atresia is accomplished via transnasal or transpalatal approach. Advantages of the transpalatal approach are improved exposure and the preservation of mucosal flap along the newly formed apertures. On the other hand, the transpalatal approach carries the risk of injury to the greater palatine neurovascular complex, and requires longer operative time. After careful physical and radiographic examinations, we accomplished the surgical repair of the complete bony bilateral choanal atresia via transpalatal approach without complications.

• Journal of Chest Surgery
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• v.42 no.4
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• pp.532-536
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• 2009

Bypass surgery is performed for insuring continuity of a blood supply that is restricted due to obstruction of the native blood supply. Two types of surgery are commonly carried out: one is anatomic bypass and the other is extraanatomic bypass. Especially, extraanatomic bypass surgery is performed in patients who are a high risk for performing anatomical bypass surgery. The risk factors for anatomical bypass surgery are old age, infection, previous surgery and trauma. A 96-year-old patient underwent extraanatomic bypass surgery from the right femoral artery to the left femoral artery due to total occlusion of the left iliac artery, and 7 years after that operation, a pseudoaneurysm abruptly and spontaneously appeared from the bypass suture site without any predisposing factor. The lesion was revealed to be an organizing thrombus.

• Tuberculosis and Respiratory Diseases
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• v.46 no.5
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• pp.709-717
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• 1999

Background & Method : Bronchiolitis obliterans organizing pneumonia(BOOP) is a specific clinicopathologic condition characterized by chronic inflammatory interstitial infiltrates. Cryptogenic form of BOOP presents subacute clinical course of flu-like illness, such as cough, fever, dyspnea with exertion and other constitutional symptoms. Pathologically it shows the presence of granulation tissue filling the lumen of terminal and respiratory bronchioles, extending into distal airspaces. Recently, we reviewed 24 cases of idiopathic type of BOOP, 5 cases of our hospital and another 19 cases on Korean literatures, and compared with reviewed data from foreign literatures. Results : Mean age was 54 years old and there was female preponderance in domestic reports. Their common presenting symptoms were dyspnea and cough, and mean duration of illness was 41 days. On chest examination, inspiratory crackle was a common finding. The laboratory findings were nonspecific except hypoxemia. Lung function studies revealed restrictive defect or combined obstructive and restrictive pattern in most patients. Bilateral patchy and nonsegmental alveolar opacities constituted characterized radiographic finding, highlighted on high resolution computed tomogram. It showed a favorable prognosis with an excellent responsiveness to corticosteroid therapy. The clinical features and laboratory findings were similar between domestic and foreign cases except female preponderance in Korean cases. Conclusion : If the clinical course is atypical or pregressive under proper treatment, clinicians should reevaluate clinical features and radiographic findings under the consideration of BOOP. Tissue confirmation would be recommended for the definitive diagnosis of BOOP.

• Journal of the Korean Society of Marine Environment & Safety
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• v.24 no.6
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• pp.726-734
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• 2018

The total number of Sea Area Utilization Consultation in the environment management sea areas reviewed from 2015 to 2017 were 60. The number of development projects in the environment conservation sea areas decreased steadily, but special management sea areas increased. Development types in environment management sea areas showed that artificial structure installation was the highest ratio, followed by fishery port development and construction of habor and coastal maintenance and sea water intake and draining. By comparing the trend of water quality changes using marine environment information system (MEIS) data in the environment management areas from 2006 to 2017, COD showed no significant changes but the environment conservation sea areas increased slightly, and the concentration of TN and TP decreased. Gwangyang and Masan bays in the special management sea areas and Gamak bay in the environment conservation sea areas displayed oxygen deficient mass in the summer. As the use of development projects of the environment management sea areas are performed continuously, an analysis of the status of sufficient water quality changes is necessary for environmental impact assessment (Sea Area Utilization Consultation) in the marine environment and should be evaluated mainly for management of contamination by diagnosing thoroughly water quality effects and the pollution of sediment. Especially, the water quality goal for the purpose of designation in each of the environment management sea areas is set clearly, connection with pollution source control and the total pollution load management system (TPLMS) should be proposed and measured to reduce the amount of contaminated water.

• Journal of Yeungnam Medical Science
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• v.11 no.1
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• pp.35-41
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• 1994

Newborns with ileal atresia freqently present with abdominal distension, bilious vomiting. and failure to pass meconium. Diagnosis is usually established on plain x-ray of the abdomen by the findings of distended small bowel loops and air-fluid levels. In the period of October 1988 to February 1994, 8 patients with congenital ileal atresia were operated and the following results were obtained. 1. Eight patients were comprised of 4 males and 4 females, the ratio of male and female was 1 : 1. 2. Six patients(75%) had been admittted to our hospital during three days of life. 3. Congenital ileal atresia was in 8 cases : Type I in two(25%), Type II in two(25%), Type IIIa in three(37.5%), Type IIIb in one(12.5%). 4. There was one premature patient who was small for gestational age. 5. Overall, abdominal distension and bilious vomiting occurring in seven patients, were frequent presenting complaints. 6. Diagnosis was possible with clinical symptom and simple abdomen. 7. Operative treatment was undertaken as soon as the diagnosis was made. In seven cases a primary end-to-end anastomosis was performed after resection of dilated proximal loop. 8. A total of four associated congenital anomalies were found in one patient. 9. Postoperative complications occurred in three cases(37.5%).

• Proceedings of the KIEE Conference
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• 1993.07a
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• pp.504-506
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• 1993

The Joint Photographic Experts Group (JPEG) standard was proposed by the International Standardization Organization (ISO/SC 29/WG 10) and the CCITT SG VIII as an international standard for digital continuous-tone still image compression. The JPEG standard has been widely accepted in electronic imaging, computer graphics, and multi-media applications, however, due to the lossy character of the JPEG compression its application in the field of medical imaging has been limited. In this paper, the JPEG standard was applied to a series of head sections of magnetic resonance (MR) images (256 gray levels, $256{\times}256$ size) and its performance was investigated. For this purpose, DCT-based sequential mode of the JPEG standard was implemented using the CL550 compression chip and progressive and lossless coding was implemented by software without additional hardware. From the experiment, it appears that the compression ratio of about 10 to 20 was obtained for the MR images without noticeable distortion. It is also noted that the error signal between the reconstructed image by the JPEG and the original image was nearly random noise without causing any special-pattern-related artifact. Although the coding efficiency of the progressive and hierarchical coding is identical to that of the sequential coding in compression ratio and SNR, it has useful features In fast search of patient Image from huge image data base and in remote diagnosis through slow public communication channel.

• Journal of vocational education research
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• v.36 no.1
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• pp.1-22
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• 2017

This study examined the process of admission selection of vocational high schools and meister high schools and how the process have been utilized by schools to select appropriate students and by students to make decisions for application in consideration of their interests, aptitudes, and career paths. To address the purpose of this study, Focus Group Interviews(FGI) were conducted with three groups of participants: vocational high schools and meister high schools teachers, high school freshmen' parents, and middle school teachers who had many students advanced to vocational and meister high schools. The findings indicated that during the promotion process, schools made efforts to attract students with higher GAP and provided information mainly regarding employment rate instead of curriculum or program of majors. Since schools usually visited middle schools where higher GPA students were, not all students had balanced opportunities of high school promotions and enough information about majors. For the application process, the schools placed a high value on scholastic performance while students applied high schools considering their interests, aptitudes, and career paths. In terms of admission type and criteria, the high school teachers reported no big differences between special admission and general admission while students took advantage from the special admission process which was less competitive than general admission. Based on the findings, a number of suggestions were made in order to improve the process of admission selection of vocational high schools and meister high schools.