• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.23-29
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• 2018

Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is a rare autosomal recessive urea cycle disorder characterized by hyperammonemia. CPS1D is caused by mutations in the CPS1 gene on chromosome 2q35. Based on the age of onset, there are two phenotypes: the neonatal type and the delayed-onset type. The severity of clinical manifestation depends on the degree of CPS1 residual enzymatic activity, and can result in hyperammonemia and neurological dysfunction. We report a case of CPS1D in a neonate who developed vomiting, decreased consciousness and hyperammonemia at 25th day after birth. She showed excellent response to treatment including hydration, ammonia-lowering drugs and a low-protein diet without hemodialysis. Her growth, development and neurological outcomes were fair at the last follow-up at 17 months of age.

• Journal of Chest Surgery
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• v.44 no.2
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• pp.137-141
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• 2011

Background: There is controversy about the benefit of surgical correction of an atrial septal defect (ASD) in patients over 60 years old. The purpose of this study was to determine whether surgical treatment is beneficial in those 60 years of age or older. Materials and Methods: We reviewed the clinical course of 57 patients (mean age: $63.54{\pm}5.59$ years) diagnosed with an isolated secundum ASD after the age of 60. The 24 patients (group A) who underwent surgical repair were compared with the 33 patients (group B) who were treated non-surgically. The mean follow-up period was $6.8{\pm}4.5$ years. Results: One operative death, 5 late deaths (20.8%) in group A, and 9 deaths (27.3%) in group B occurred in the study period. Symptomatic improvement was noted in 18 patients (75%) of group A after surgery. However 13 patients (39.4%) of group B showed symptomatic improvement during the follow-up period (p=0.012). The incidence of new atrial arrhythmia of the two groups was significantly different (16.7% vs 36.7%, p=0.038). The actuarial 10 year survival rate was 79% in group A and 73% in group B. Conclusion: Although surgical correction of ASD did not increase survival in patients over 60 years old, the surgical outcomes of ASD showed low operative mortality and resulted in symptomatic improvement in the majority of these patients. This study has shown the benefits of surgical closure of ASD even in advanced age in comparison to medical treatment.

• Journal of Chest Surgery
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• v.46 no.4
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• pp.274-278
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• 2013

Background: Coronary involvement in Takayasu's arteritis is a rare but fatal disease. The aim of this study was to evaluate the early and mid-term results of Takayasu's arteritis patients who underwent coronary artery bypass grafting (CABG). Materials and Methods: Of 2,280 patients who underwent isolated CABG from January 1998 to June 2012, Takayasu's arteritis was identified in 5 patients. There were 3 female patients, and the mean age was $58{\pm}9$ years. Takayasu's arteritis was diagnosed during preoperative evaluation for coronary artery disease in 4 patients, and the initial manifestation was angina pectoris in 4 patients. All of the patients underwent anaortic off-pump CABG (OPCAB) using the in situ left or right internal thoracic arteries (ITA); 3 patients had severe stenosis of the proximal left subclavian artery and the in situ right ITA was used instead. Medical treatment for inflammatory arteritis during the perioperative and follow-up period was performed if indicated. Early, 1-year, and 5-year angiographic results and clinical outcomes were analyzed. Results: There was no surgical mortality, and all of the patients were discharged without complications on postoperative $8{\pm}2$ days. Early postoperative (postoperative $2{\pm}1$ days) angiography demonstrated a graft patency of 100% (12 of 12 distal anastomoses). One-year ($13{\pm}3$ months) angiography was performed in 4 patients, and all of the grafts were patent (100%, 9 of 9 distal anastomoses). Conclusion: By performing anaortic OPCAB in patients with Takayasu's arteritis, we were able to avoid complications associated with manipulating an atherosclerotic and severely calcified ascending aorta. The early and mid-term graft patency of OPCAB in Takayasu's arteritis was maintained when concomitant with medical treatment.

• Clinics in Shoulder and Elbow
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• v.16 no.2
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• pp.123-129
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• 2013

Purpose: The purpose of this study is to compare the clinical and radiological outcomes of two surgical treatments, transacromial pin fixation and hook plate fixation, for unstable distal clavicle fractures. Materials and Methods: Twenty four patients with Neer type II distal clavicle fractures who underwent surgery with transacromial pin fixation (Group I: 12 patients) and hook plate fixation (Group II: 12 patients) were included. Reduction and union were evaluated using plain radiographs. Functional evaluation was performed according to UCLA score and Constant-Murley score at last follow-up. Results: All 24 cases showed bone union. Complete union took an average of 10 weeks for group I and 11.7 weeks for group II. Average UCLA score was 33 (group I) and 32.8 (group II). Average Constant score was 88.5 (group I) and 88.8 (group II). No significant difference for each variable was observed between the two groups. Conclusion: For the surgical treatment of Neer type II distal clavicle fractures, transacromial pin fixation and hook plate fixation are both useful methods.

• Korean Journal of Head & Neck Oncology
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• v.19 no.2
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• pp.127-132
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• 2003

Objectives: The best treatment for the malignant parotid tumor still remains to be defined, and a better knowledge about the tumor features that predict the treatment result is needed. The aim of this study is to evaluate the treatment outcomes and to suggest the optimal treatment modality for the parotid cancer. Materials and Methods: The clinicopathologic characteristics of 113 patients who were treated for parotid cancer from January 1990 to December 2002 were retrospectively analysed. Univalate analyses were performed to establish the prognostic influence of pateint age, gender, tumor size, histologic grade and lymph node metastasis. Results: The mean age was 46.4 years old (15-81 years) and. The male to female ratio was 1 : 1.1. The chief complaint was a palpable mass in 85%, pain was in 12.4% and facial nerve palsy was accompanied with 2.7%. The mean tumor size was 3.5cm in diameter. The most common malignant tumor was mucoepidermoid carcinoma (33.6%), followed by acinic cell carcinoma (15%), adenoid cystic carcinoma (11%), carcinoma expleomorhpic adenoma (11%), basal cell carcinoma (7%). The most common operative procedure was total parotidectomy (47.8%) and various types of cervical lymph node dissection were added in 69.9%. Postoperative radiotherapy was done in 61.1 %. Postoperative complications developed in 54 cases (47.8%), including 46 cases (40.7%) of facial nerve palsy and 9 cases (8%) of Frey's syndrome. Recurrences developed in 21 cases (18.6%) and deaths in 15 (13.3%). Cumulative survival at 5 year was 75.4%. Univariate analysis of clinical factors showed that histologic grade and positive cervical lymph node significantly influenced survival (p<0.05). Conclusion: These results suggests that the radical resection with lymph node dissection and postopertaive XRT would be necessary to improve the survival of the patients with high grade cancer or positive lymphnode metastasis.

• Journal of Hospice and Palliative Care
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• v.12 no.4
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• pp.177-193
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• 2009

The assessment of patient status in palliative medicine is essential for determining treatments and for clinical outcomes. The objective of assessment tools is to raise the quality of care for individual patients and their families. There are a number of tools available to assess pain, non-pain symptoms and quality of life. The tools are either uni-dimensional or multi-dimensional measures. Unfortunately, however, no single tool is recommended to be a superior to others in symptoms or quality of life assessment. Therefore, to select an appropriate assessment tool, one should consider the time frame and unique characteristics of tools depending on purpose and setting. The combination of prognostic index is highly recommended in prognostication, and web-based prognostic tools are available. Recently, a new objective prognostic score has been constructed through multicenter study in Korea. It does not include clinicalestimates of survival, but includes new objective prognostic factors, therefore, anyone can easily use it. For beginners in palliative medicine, relatively easy-to-use tools would be convenient. We recommend Eastern Cooperative Oncology Group performance status to assess functional status, numeric rating scale for pain assessment and the Korean version of brief pain inventory for initial pain assessment. Asking directly with numeric rating scale or the Korean version of MD Anderson Symptom Inventory would be desirable to assess various symptoms together. We think that European Organization Research and Treatment Quality of Life Questionnaire Core 15 for Palliative Care is good to assess the quality of life, while Objective Prognostic Score is convenient as prognostic index for beginners.

• Journal of Pharmacopuncture
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• v.15 no.2
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• pp.31-35
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• 2012

Background and Objectives: Non-small-cell lung cancer (NSCLC) represents approximately 80% of all lung cancers. Unfortunately, at their time of diagnosis, most patients have advanced to unresectable disease with a very poor prognosis. The oriental herbal medicine HangAm-Plus (HAP) has been developed for antitumor purposes, and several previous studies have reported its therapeutic effects. In this study, the efficacy of HAP was evaluated as a third-line treatment for advanced-stage IIIb/IV NSCLC. Methods: The study involved six patients treated at the East- West Cancer Center (EWCC) from April 2010 to October 2011. Inoperable advanced-stage IIIb/IV NSCLC patients received 3,000 or 6,000 mg of HAP on a daily basis over a 12-week period. Computed tomography (CT) scans were obtained from the patients at the time of the initial administration and after 12 weeks of treatment. We observed and analyzed the patients overall survival (OS) and progression-free survival (PFS). Results: Of the six patients, three expired during the study, and the three remaining patients were alive as of October 31, 2011. The OS ranged from 234 to 512 days, with a median survival of 397 days and a one-year survival rate of 66.7%. In the 12-week-interval chest CT assessment, three patients showed stable disease (SD), and the other three showed progressive disease (PD). The PFS of patients ranged from 88 to 512 days, the median PFS being 96 days. Longer OS and PFS were correlated with SD. Although not directly comparable, the OS and the PFS of this study were greater than those of the docetaxel or the best supportive care group in other studies. Conclusion: HAP may prolong the OS and the PFS of inoperable stage IIIb/IV NSCLC patients without significant adverse effects. In the future, more controlled clinical trials with larger samples from multi-centers should be conducted to evaluate the efficacy and the safety of HAP.

• Journal of Pharmacopuncture
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• v.21 no.3
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• pp.151-158
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• 2018

Objectives: We investigated the efficacy and safety of miniscalpel acupuncture (MA) for knee osteoarthritis (KOA) in an assessor-blinded randomized controlled pilot trial; this would provide information for a large-scale randomized controlled trial. Methods: Participants (n = 24) were recruited and randomly allocated to the MA group (experimental) or acupuncture group (control). The MA group received treatment once a week for 3 weeks (total of 3 treatments), while the acupuncture group received treatment two times per week for 3 weeks (total of 6 treatments). The primary outcome was pain as assessed by a visual analogue scale (VAS). The secondary outcomes (intensity of current pain, stiffness, and physical function) were assessed using the short-form McGill Pain Questionnaire (SF-MPQ) and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC). Assessments were performed at baseline, 1, 2, and 3 during treatment and at week 5 (2 weeks after the end of treatment). Results: Of the 24 participants, 23 completed the study. Both groups showed significant improvements in VAS, SF-MPQ, and WOMAC. However, there were no significant differences between the MA and acupuncture groups. No serious adverse event occurred and blood test results were within normal limits. Conclusion: Our results suggest that although both MA and acupuncture provide similar effects with regard to pain control in patients with KOA, MA may be more effective in providing pain relief because the same relief was obtained with fewer treatments. A large-scale clinical study is warranted to further clarify these findings.

• Journal of Oral Medicine and Pain
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• v.38 no.1
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• pp.77-86
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• 2013

In order to evaluate the effect of conservative treatment for Temporomandibular Disorders(TMD), 137 patients were subjected at the Department of Oral Medicine, Pusan National University Dental Hospital from June 2012 to Sept. 2012. They were treated conservatively with behavioral therapy, physical therapy, medication and occlusal stabilizing splint therapy. Subjective symptoms and clinical findings were investigated to evaluate and compare the patients' status after 3 months treatment. The results were as follows; 1. Pain, Noise, LOM(Limitation of motion) and MCO(Maximum comfortable opening) measurements of TMD were markedly improved after conservative treatments including behavior therapy, physical therapy, medication and splint therapy. 2. There was no difference in treatment outcomes after conservative treatments when the subjects were classified and compared according to gender and chronicity. 3. Conservative treatment including stabilization splint produced better results than physical therapy with medication. 4. After 3 months of treatment, pain and LOM were significantly improved in the MD(Muscle disorder) group. Pain, LOM and noise were significantly improved in the DD(Disc displacements) group. In the OA(Osteoarthritis) group, pain, noise, LOM and MCO were significantly improved.

• Tuberculosis and Respiratory Diseases
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• v.81 no.2
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• pp.99-105
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• 2018

Acute exacerbation(s) of chronic obstructive pulmonary disease (AECOPD) tend to be critical and debilitating events leading to poorer outcomes in relation to chronic obstructive pulmonary disease (COPD) treatment modalities, and contribute to a higher and earlier mortality rate in COPD patients. Besides pro-active preventative measures intended to obviate acquisition of AECOPD, early recovery from severe AECOPD is an important issue in determining the long-term prognosis of patients diagnosed with COPD. Updated GOLD guidelines and recently published American Thoracic Society/European Respiratory Society clinical recommendations emphasize the importance of use of pharmacologic treatment including bronchodilators, systemic steroids and/or antibiotics. As a non-pharmacologic strategy to combat the effects of AECOPD, noninvasive ventilation (NIV) is recommended as the treatment of choice as this therapy is thought to be most effective in reducing intubation risk in patients diagnosed with AECOPD with acute respiratory failure. Recently, a few adjunctive modalities, including NIV with helmet and helium-oxygen mixture, have been tried in cases of AECOPD with respiratory failure. As yet, insufficient documentation exists to permit recommendation of this therapy without qualification. Although there are too few findings, as yet, to allow for regular andr routine application of those modalities in AECOPD, there is anecdotal evidence to indicate both mechanical and physiological benefits connected with this therapy. High-flow nasal cannula oxygen therapy is another supportive strategy which serves to improve the symptoms of hypoxic respiratory failure. The therapy also produced improvement in ventilatory variables, and it may be successfully applied in cases of hypercapnic respiratory failure. Extracorporeal carbon dioxide removal has been successfully attempted in cases of adult respiratory distress syndrome, with protective hypercapnic ventilatory strategy. Nowadays, it is reported that it was also effective in reducing intubation in AECOPD with hypercapnic respiratory failure. Despite the apparent need for more supporting evidence, efforts to improve efficacy of NIV have continued unabated. It is anticipated that these efforts will, over time, serve toprogressively decrease the risk of intubation and invasive mechanical ventilation in cases of AECOPD with acute respiratory failure.