• 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.

• Journal of Chest Surgery
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• v.41 no.3
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• pp.347-353
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• 2008

Background: Esophageal perforation is an emergency that. requires early diagnosis and effective treatment. A delay in diagnosis and treatment. significantly increases morbidity and mortality. Material and Method: Thirty-seven patients with esophageal perforation were surgically treated at our institutions between January 1990 and December 2006. We retrospectively reviewed the results of surgical treatment for esophageal perforation to understand the risk factors affecting survival inpatients. Result: Patients ranged in age from 21 to 87 years, with an average age of $52.7{\pm}16.98$ years. Thirty-one of the patients were men and six were women. There were 23 patients (62%) with spontaneous perforations, 10 patients (27%) with a traumatic perforation, and 4 patients (11%) with an iatrogenic perforation. The site of esophageal perforation was the cervical esophagus in 5 patients, the thoracic esophagus in 31 patients, and the abdominal esophagus in one patient. Twenty-nine patients underwent primary closure of the perforation and five patients had T-tube drainage. Exclusion-diversion procedures were performed in two patients and an esophagectomy was performed in one patient. There were six cases of mortality (16.22%) and 25 cases of postoperative complications in 15 patients (40.5%). Patients that were treated later than 24 hours after detection of the perforation showed a statistically significant high morbidity and mortality rate (p<0.05). Conclusion: The most important risk factor of esophageal perforation was the time interval between detection of the perforation and the initiation of treatment. A prompt diagnosis and effective treatment are necessary to decrease morbidity and mortality.

• The Korean Journal of Nuclear Medicine
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• v.37 no.6
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• pp.355-363
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• 2003

Purpose: Regional contractility can be calculated using the regional volume change of left ventricle measured by gated myocardial SPECT image and curve of central artery pressure obtained from radial artery pressure data. In this study, a program to obtain the regional contractility was developed, and reproducibility of regional contractility measurement was assessed. Materials and Methods: Seven patients(male:female=5:2, $58{\pm}11.9$ years) with coronary artery diseases underwent gated Tc-99m MIBI myocardial SPECT twice without delay between two scans. Regional volume change of left ventricle was estimated using CSA (Cardiac SPECT Analyzer) software developed in this study. Regional contractility was iteratively estimated from the time-elastance curve obtained using the time-pressure curve and regional time-volume curve. Reproducibility of regional contractility measurement assessed by comparing the contractility values measured twice from the same SPECT data and by comparing those measured from the pair of SPECT data obtained from a same patient. Results: Measured regional contractility was $3.36{\pm}3.38{mm}Hg/mL$ using 15-segment model, $3.16{\pm}2.25{mm}Hg/mL$ using 7-segment model, and $3.11{\pm}2.57{mm}Hg/mL$ using 5-segment model. The harmonic average of regional contractility value was almost identical to the global contractility. Correlation coefficient of regional contractility values measured twice from the same data was greater than 0.97 for all models, and two standard deviations of contractility difference on Bland Altman plot were 1.5%, 1.0%, and 0.9% for 15-, 7-, and 5-segment models, respectively. Correlation coefficient of regional contractility values measured from the pair of SPECT data obtained from a same patient was greater than 0.95 for all models, and two standard deviations on Bland Altman plot were 2.2%, 1.0%, and 1.2%. Conclusion: Regional contractility of left ventricle measured using developed software in this study was reproducible. Regional contractility of left ventricle will be a new useful index for myocardial function after analysis of the clinical data.

• The Journal of the Korean bone and joint tumor society
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• v.14 no.2
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• pp.163-171
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• 2008

Purpose: We analyze the characteristics of soft tissue sarcomas presented with hematoma, which were misdiagnosed as simple hematoma initially and the proper management were delayed. Materials and Methods: The 7 patients with histologically proven soft tissue sarcoma with hematoma presented since February 1997 were evaluated retrospectively. Neither patient had a medical history of bleeding tendency nor anticoagulant therapy. Two of them had minor traumas. There were 2 men and 5 women. Average follow up period was 58 months. MRI findings, provided treatments and oncologic outcome were reviewed with the reference of related articles. Results: Retrospective review of initial MR images revealed deep seated intramuscular masses with focal solid enhanced nodules at the peripheral margin. The diagnoses were delayed at least 1 month in 3 of them which included 2 cases of simple hematoma evacuation without biopsy initially. After histologic diagnosis of soft tissue sarcoma, wide resections were performed in 4 cases. one patient underwent above knee amputation and the remained 2 patients were managed with wide resection followed by amputation due to local recurrence. At last follow up there were CDF and NED in 2 cases, respectively and AWD in 3 cases. Conclusion: To avoid the delay of diagnosis and treatment of soft tissue sarcomas presented with hematoma, high degree of clinical suspicion, careful analysis of MR images and early biopsy were important.

• The Korean Journal of Nuclear Medicine
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• v.32 no.6
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• pp.490-496
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• 1998

Purpose: We evaluated brain perfusion SPECT findings of MELAS syndrome and mitochondrial myopathy in correlation with MR imaging in search of specific imaging features. Materials and Methods: Subjects were five patients (four females and one male; age range, 1 to 25 year) who presented with repeated stroke-like episodes, seizures or developmental delay or asymptomatic but had elevated lactic acid in CSF and serum. Conventional non-contrast MR imaging and Tc-99m-ethyl cysteinate dimer (ECD) brain perfusion SPECT were Performed and imaging features were analyzed. Results: MRI demonstrated increased T2 signal intensities in the affected areas of gray and white matters mainly in the parietal (4/5) and occipital lobes (4/5) and in the basal ganglia (1/5), which were not restricted to a specific vascular territory. SPECT demonstrated decreased perfusion in the corresponding regions of MRI lesions. In addition, there were perfusion defects in parietal (1 patient), temporal (2), and frontal (1) lobes and basal ganglia (1) and thalami (2). In a patient with mitochondrial myopathy who had normal MRI, decreased perfusion was noted in left parietal area and bilateral thalami. Conclusion: Tc-99m ECD SPECT imaging in patients with MELAS syndrome and mitochondrial myopathy showed hypoperfusion of parieto-occipital cortex, basal ganglia, thalamus and temporal cortex, which were not restricted to a specific vascular territory. There were no specific imaging features on SPECT. The significance of abnormal perfusion on SPECT without corresponding MR abnormalities needs to be evaluated further in larger number of patients.

• The Korean Nurse
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• v.29 no.2
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• pp.48-65
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• 1990

The government has adopted a policy to introduce Home Health Care Program, and has established a three stage plan to implement it. The three stage plan is : First, to amend Article 54 (Nurses for Different Types of Services) of the Regulations for Implementing the Law of Medical Services; Second, to tryout the new system through pilot projects established in public hospitals and clinics; and third, to implement at all hospitals and equivalent medical institutions. In accordance with the plan, the Regulation has been amend and it was promulgated on January 9,1990, thus establishing a legal ground for implementing the policy. Subsequently, however, the Medical Association raised its objection to the policy, causing a delay in moving into the second stage of the plan. Under these circumstances, a study was conducted by collecting and evaluating the opinions of physicians, nurses, non-medical personnel and patients on the need and expected result from the home health care for the purpose of help facilitating the implementation of the new system. As a result of this study, it was revealed that: 1. Except the physicians, absolute majority of all other three groups - nurses, non-medical personnel and patients -gave positive answers to all 11 items related to the need for establishing a program for Home Health Care. Among the physicians, the opinions on the need for the new services were different depending on their field of specialty, and those who have been treating long term patients were more positive in supporting the new system. 2. The respondents in all four groups held very positive view for the effectiveness and the expected result of the program. The composite total of scores for all of 17 items, however, re-veals that the physicians were least positive for the- effectiveness of the new system. The people in all four groups held high expectation on the system on the ground that: it will help continued medical care after the discharge from hospitals; that it will alleviate physical and economic burden of patient's family; that it will offer nursing services at home for the patients who are suffering from chronic disease, for those early discharge from hospital, or those who are without family members to look after the patients at home. 3. Opinions were different between patients( who will receive services) and nurses (who will provide services) on the types of services home visiting nurses should offer. The patients wanted "education on how to take care patients at home", "making arrangement to be admitted into hospital when need arises", "IV injection", "checking blood pressure", and "administering medications." On the other hand, nurses believed that they can offer all 16 types of services except "Controlling pain of patients", 4. For the question of "what types of patients are suitable for Home Health Care Program; " the physicians, the nurses and non-medical personnel all gave high score on the cases of "patients of chronic disease", "patients of old age", "terminal cases", and the "patients who require long-term stay in hospital". 5. On the question of who should control Home Health Care Program, only physicians proposed that it should be done through hospitals, while remaining three groups recommended that it should be done through public institutions such as public health center. 6. On the question of home health care fee, the respondents in all four groups believed that the most desireable way is to charge a fixed amount of visiting fee plus treatment service fee and cost of material. 7. In the case when the Home Health Care Program is to be operated through hospitals, it is recommended that a new section be created in the out-patient department for an exclusive handling of the services, instead of assigning it to an existing section. 8. For the qualification of the nurses for-home visiting, the majority of respondents recommended that they should be "registered nurses who have had clinical experiences and who have attended training courses for home health care". 9. On the question of if the program should be implemented; 74.0% of physicians, 87.5% of non-medical personnel, and 93.0% of nurses surveyed expressed positive support. 10. Among the respondents, 74.5% of -physicians, 81.3% of non-medical personnel and 90.9% of nurses said that they would refer patients' to home health care. 11. To the question addressed to patients if they would take advantage of home health care; 82.7% said they would if the fee is applicable to the Health Insurance, and 86.9% said they would follow advises of physicians in case they were decided for early discharge from hospitals. 12. While 93.5% of nurses surveyed had heard about the Home Health Care Program, only 38.6% of physicians surveyed, 50.9% of non-medical personnel, and 35.7% of patients surveyed had heard about the program. In view of above findings, the following measures are deemed prerequisite for an effective implementation of Home Health Care Program. 1. The fee for home health care to be included in the public health insurance. 2. Clearly define the types and scope of services to be offered in the Home Health Care Program. 3. Develop special programs for training nurses who will be assigned to the Home Health Care Program. 4. Train those nurses by consigning them at hospitals and educational institutions. 5. Government conducts publicity campaign toward the public and the hospitals so that the hospitals support the program and patients take advantage of them. 6. Systematic and effective publicity and educational programs for home heath care must be developed and exercises for the people of medical professions in hospitals as well as patients and their families. 7. Establish and operate pilot projects for home health care, to evaluate and refine their programs.

• Journal of Yeungnam Medical Science
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• v.14 no.1
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• pp.245-261
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• 1997

Slipped capital femoral epiphysis(SCFE) is a disorder in which there is a gradual or acute disruption through the capital physeal plate. The physiolysis is through a widened zone of hypertrophy, which is weakened due to altered chondrocytic maturation and endochondral ossification. The cause or causes of SCFE remain uncertain. The association of obesity and adolescent age with growth rate are predisposing factors. The possibility that most patients with subclinical hormonal abnormality were proved. The goal of treatment of slipped capital femoral epiphysis is to restore the function of the hip and delay the development of degenerative osteoarthrosis by prevention of additional displacement of the epiphysis. We report 10 patients(12hips) with SCFE who were treated by surgical means and followed along for more than one year, at Yeungnam University Hospital, from 1989 to 1996. There were six boys and four girls. The average age at operation was 11.8 years. Seven cases occurred in the left hip, one case in the right and 2 cases had bilateral involvement, five cases had a history of minor trauma on affected hip. Among hormonally studied six patients, panhypopituitarism patient was one case; decreased testosterone, two; decreased growth hormone, two; and decreased thyroid hormone, one. According to clinical stage, two cases were the acute type; five cases, acute on chronic type; and three cases, chronic type. On the radiological grades of slipping, mild slippage were nine hips; moderate, one; and severe, two. The eleven hips were treated by pin fixation in situ, and one, by cuneiform osteotomy. On the average follow-up of 2.6 years, ten hips were excellent or good functional results, two hips were failure.

• The Korean Journal of Emergency Medical Services
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• v.15 no.3
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• pp.31-44
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• 2011

Purpose : The purpose of emergency medical services(EMS) is to save human lives and assure the completeness of the body in emergency situations. Those who have been qualified on medical practice to perform such treatment as there is the risk of human life and possibility of major physical and mental injuries that could result from the urgency of time and invasiveness inflicted upon the body. In the emergency medical activities, 119 emergency medical technicians mainly perform the task but they are not able to perform such task independently and they are mandatory to receive medical direction. The purpose of this study is to examine the recognition and request for medical direction by 119 emergency medical technicians in order to provide basic information on the development of medical direction program suitable to the characteristics of EMS as well as for the studies on EMS for the sake of efficient operation of pre-hospital EMS. Method : Questionnaire via e-mail was conducted during July 1-31, 2010 for 675 participants who are emergency medical technicians, nurses and other emergency crews in Gyeongbuk. The effective 171 responses were used for the final analysis. In regards to the emergency medical technicians' scope of responsibilities defined in Attached Form 14, Enforcement regulations of EMS, t-test analysis was conducted by using the means and standard deviation of the level of request for medical direction on the scope of responsibilities of Level 1 & Level 2 emergency medical technicians as the scale of medical direction request. The general characteristics, experience result, the reason for necessity, emergency medical technicians & medical director request level, medical direction method, the place of work of the medical director, feedback content and improvement plan request level were analyzed through frequency and percentage. The level of experience in medical direction and necessity were analyzed through ${\chi}^2$ test. Results : In regards to the medical direction experience per qualification, the experience was the highest with 53.3% for Level 1 emergency medical technicians and 80.3% responded that experience was helpful. As for the recognition on the necessity of medical direction, 71.3% responded as "necessary" and it turned out to be the highest of 76.9% in nurses. As for the reason for responding "necessary", the reason for reducing the risk and side-effects from EMS for patients was the largest(75.4%), and the reason of EMS delay due to the request of medical direction was the highest(71.4%) for the reason for responding "not necessary". In regards to the request level of the task scope of emergency medical technicians, injection of certain amount of solution during a state of shock was the highest($3.10{\pm}.96$) for Level 1 emergency rescuers, and the endotracheal intubation was the highest($3.12{\pm}1.03$) for nurses, and the sublingual administration of nitroglycerine(NTG) during chest pain was the highest($2.62{\pm}1.02$) for Level 2 emergency medical technicians, and regulation of heartbeat using AED was the highest($2.76{\pm}.99$) for other emergency crews. For the revitalization of medical direction, the improvement in the capability of EMS(78.9%) was requested from emergency crew, and the ability to evaluate the medical state of patient was the highest(80.1%) in the level of request for medical director. The prehospital and direct medical direction was the highest(60.8%) for medical direction method, and the emergency medical facility was the highest(52.0%) for the placement of medical director, and the evaluation of appropriateness of EMS was the highest(66.1%) for the feedback content, and the reinforcement of emergency crew(emergency medical technicians) personnel was the highest(69.0%) for the improvement plan. Conclusion : The medical direction is an important policy in the prehospital EMS activity because 119 emergency medical technicians agreed the necessity of medical direction and over 80% of those who experienced medical direction said it was helpful. In addition, the simulation training program using algorithm and case study through feedback are necessary in order to enhance the technical capability of ambulance teams on the item of professional EMS with high level of request in the task scope of emergency medical technicians, and recognition of medical direction is the essence of the EMS field. In regards to revitalizing medical direction, the improvement of the task performance capability of 119 emergency medical technicians and medical directors, reinforcement of emergency medical activity personnel, assurance of trust between emergency medical technicians and the emergency physician, and search for professional operation plan of medical direction center are needed to expand the direct medical direction method for possible treatment beforehand through the participation by medical director even at the step in which emergency situation report is received.

• Pediatric Infection and Vaccine
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• v.14 no.1
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• pp.43-46
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• 2007

Purpose : Neonatal bacterial meningitis has been known that its mortality and morbidity is high and its neurologic complications are common. This study was performed to review of the etiology of neonatal bacterial meningitis in Busan, Korea. Methods : We retrospectively analyzed 21 medical records of patients who were diagnosed neonatal bacterial meningitis in four university hospitals in Busan from January 1997 to December 2005. We analyzed age at onset of symptoms, symptoms at admission, causative organisms, and complications. Results : There were 11 male patients and 10 female patients. One premature neonate was included. 7 patients had symptoms at 2-3 weeks after birth and 6 patients at 1-2 weeks after birth. 18 cases had fever, 8 cases had seizure and some had poor feeding, lethargy and irritability. The most common organism causing neonatal bacterial meningitis was Group B streptococcus (GBS). 11 cases were due to GBS, 3 cases due to E. coli, 3 cases were due to Klebsiella pneumoniae, and 2 cases due to Streptococcus pneumoniae. one Listeria monocytogenes and one Enterobacter cloacae were included. 18 patients were alive and 3 patients died. Mental retardation, developmental delay and epilepsy occurred in a living patient. Conclusions : Although this was a retrospective study, we found that Group B streptococcus (GBS) was the most common organism causing neonatal bacterial meningitis and this data is nearly same as others in the world.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.4
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• pp.750-756
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• 2008

Supernumerary teeth are frequently found in the anterior portion of the maxilla and develop as a result of abnormal proliferation of the dental lamina during tooth germ formation, caused by genetic or environmental factors. They may result in various complications, such as eruption interference, displacement, rotation of adjacent teeth, diastema, eruption into the nasal cavity, and development of dentigerous cyst. The optimal time for surgical extraction of supernumerary teeth has been a controversial issue. Someone prefer early surgical extraction because supernumerary teeth can cause eruption interference and displacement of adjacent teeth, eventually altering occlusion. Others prefer to delay surgical extraction until $8{\sim}10$ years of age in consideration of root maturation of the adjacent teeth and also patient's behavior. When surgical extraction of supernumerary teeth is postponed, there is possibility that impacted supernumerary teeth in the inverted or horizontal position move toward the nasal cavity, hard palate, or premolar area. When such intraosseous tooth migration is combined with the vertical growth of the maxilla, surgical approach becomes even harder. Therefore, possibility of intraosseous tooth migration should be considered as an important factor when deciding appropriate time for surgical extraction. We are presenting cases of mesiodens which showed intraosseous migration during $6{\sim}7$ years of follow-up period since the first diagnosis had been made at the $2{\sim}3$ years of age.