• Clinical and Experimental Pediatrics
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• v.56 no.4
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• pp.176-181
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• 2013

Purpose: Apical muscular ventricular septal defects (MVSDs), especially in small infants, can be difficult to manage using surgical and percutaneous closure. An intraoperative perventricular procedure is a good option for closing apical MVSDs in small children with or without associated cardiac anomalies. We evaluated the results of hybrid perventricular closure of apical MVSDs performed using an Amplatzer duct occluder (ADO). Methods: We retrospectively reviewed the medical records of 5 patients who underwent hybrid perventricular closure of MVSDs with ADOs, from March 2006 to May 2011. The median patient age at the time of the procedure was 12 months (range, 25 days to 25 months), and the median body weight was 9.1 kg (range, 4.3 to 15 kg). Two patients had multiple ventricular septal defects (VSDs; additional perimembranous VSD in 1 patient and multiple MVSDs in the other) and 3 patients had associated cardiac anomalies; complete transposition of the great arteries in 1 patient and an atrial septal defect in 2 patients. All the procedures were performed on beating hearts, exception in 1 case. The ADO selected for the aortic side was at least 1 to 2 mm larger than the largest VSD in the left ventricle side. Results: The procedure was successful in all patients and each device was well positioned. During the median follow-up of 2.4 years, a small residual VSD was noted in 2 patients who had multiple VSDs and no leakage was seen in the other 3 patients. Conclusion: Perventricular closure of MVSD with an ADO is a good option for patients with apical MVSD. However, careful manipulation is important, especially in the case of small infants.

• Clinical and Experimental Pediatrics
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• v.59 no.2
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• pp.59-64
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• 2016

Purpose: Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea. Methods: Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated. Results: Their median age at the time of diagnosis was 2.5 months (range, 0-20 months). The median follow-up period was 25.5 months (range, 0-94 months). The median length at birth was 50.0 cm (range, 48-53 cm); however, height became more prominent over time, and the patients were taller than the 97th percentile at the time of the study. None of the patients had any relevant family history. Four of the 5 patients who underwent DNA sequencing had a fibrillin 1 gene mutation. All the patients with echocardiographic data of the aortic root had a z score of >2. All had mitral and tricuspid valve prolapse, and various degrees of mitral and tricuspid regurgitation. Five patients underwent open-heart surgery, including mitral valve replacement, of whom two required multiple operations. The median age at mitral valve replacement was 28.5 months (range, 5-69 months). Seven patients showed congestive heart failure before surgery or during follow-up, and required multiple anti-heart failure medications. Four patients died of heart failure at a median age of 12 months. Conclusion: The prognosis of infantile MFS is poor; thus, early diagnosis and timely cautious treatment are essential to prevent further morbidity and mortality.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.3
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• pp.1449-1452
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• 2016

Background: Adult rhabdomyosarcoma (RMS) is a rare and aggressive disease with limited data compared with pediatric RMS. The goal of this study was to determine the treatment outcome and identify factors related to survival outcome in Thailand. Materials and Methods: Adult patients (${\geq}15$ years old) with the pathological diagnosis of RMS between 1985 and 2010 were reviewed. The data were retrospectively reviewed from the pathological results and medical records from Srinagarind Hospital, Khon Kaen University. Results: A total of 34 patients were identified in the study. The median age at diagnosis was 35.5 years and the most common primary sites were extremity and the head and neck region. The incidence of pleomorphic RMS increased with age and none was found in those aged younger than 20 years old. The median survival time was 9.33 months (95%CI: 5.6-13.1). The 1- and 5- year survival rates were 38.2% (22.3-54.0) and 20.6% (9.1-35.3). On multivariate analysis, age and size of tumor did not predict better outcome while chemotherapy and surgery were significantly associated with longer survival. Conclusions: Outcome of adult RMS was poor. Surgery and chemotherapy are strongly associated with better prognosis and multimodality treatment should be incorporated in the clinic.

• Journal of Gastric Cancer
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• v.20 no.1
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• pp.106-114
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• 2020

Breast metastases of extramammary malignant neoplasms are rare, with an incidence of 0.3%-2.7% among all malignant mammary tumors. Breast metastases from gastric carcinoma are very rare (<0.1%), and this event is even rarer during pregnancy. Herein, we describe a 39-year-old Caucasian woman with a history of an Epstein-Barr virus-associated gastric carcinoma (EBVaGC) that was characterized by prominent tumor infiltrating lymphocytes. Three years after undergoing radical surgery, the patient developed bilateral breast nodules during her pregnancy. A breast biopsy was performed, and histology confirmed a diagnosis of EBVaGC; tumor cells showed positivity for cytokeratin 8/18 and E-cadherin, and negativity for cytokeratin 7, cytokeratin 20, cytokeratin 5/6, caudal type homebox 2, androgen receptor, mammaglobin, gross cystic disease fluid protein-15, and estrogen and progesterone receptors. We also discuss the main diagnostic pitfalls. To our knowledge, this is the first report of an EBVaGC with lymphoid stroma that developed breast metastases during pregnancy.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.7
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• pp.3377-3380
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• 2016

The proportion of lung cancer patients under 50 years old is small at approximately 5-10%, but as with patients older than 50, the number is on the rise. Although lung cancer treatment strategies have undergone extensive transformation in recent years based on the presence or absence of oncogenic driver mutations, there are few reports regarding these mutations in the young or the relationship between clinical setting and prognosis. Therefore, we conducted a study of clinical features in 36 patients under the age of 50 who were diagnosed with primary lung cancer from October 2008 to November 2015. The 22 patients in stages I through III A underwent operations, and all 17 whose lung cancer were detected through screening were candidates for surgery. Gene analysis was conducted for 26 (72.2%); 10 (38.5%) were positive for EGFR gene mutations, and ALK gene translocation was present in 4 (15.4%). In stage IV patients, the median progression free survival (PFS) in the ALK translocation positive and negative patients was 518 days and 130 days, respectively, and the median overall survival (OS) was not reached and 280 days, respectively. A trend toward extended PFS (p=0.203) and OS (p=0.056) was observed in patients positive for ALK translocation. We must strive for early detection by increasing screening rates and evaluate oncogenic driver mutations important for prognosis of lung cancer in the young.

• Journal of Korean Neurosurgical Society
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• v.59 no.5
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• pp.512-517
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• 2016

Objective : The pathophysiology of idiopathic Chiari malformation (CM) type 1 is disturbance of free cerebrospinal fluid (CSF) flow and restoration of normal CSF flow is the mainstay of treatment. Additional migration of the medulla oblongata in pediatric patients is referred to as CM type 1.5, but its significance in adult patients is unknown. This study is to compare surgical outcomes of adult idiopathic CM type 1.5 with that of type 1. Methods : Thirty-eight consecutive adult patients (M : F=11 : 27; median, 33.5; range, 18-63) with syringomyelia due to idiopathic CM type 1 were reviewed. Migration of the medulla oblongata was noted in 13 patients. The modified McCormick scale (MMS) was used to evaluate functional status before and one year after surgery. All patients underwent foramen magnum decompression and duroplasty. Factors related to radiological success (${\geq}50%$ decrease in the diameter of the syrinx) were investigated. The follow-up period was $72.7{\pm}55.6$ months. Results : Preoperative functional status were MMS I in 11 patients and MMS II in 14 of CM type 1 and MMS I in 8 and II in 5 of CM type 1.5. Of patients with MMS II, 5/14 patients in group A and 3/5 patients in group B showed improvement and there was no case of deterioration. Radiological success was achieved in 32 (84%) patients and restoration of the cisterna magna (p=0.01; OR, 46.5) was the only significant factor. Conclusion : Migration of the medulla oblongata did not make a difference in the surgical outcome when the cisterna magna was restored.

• Journal of Korean society of Dental Hygiene
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• v.17 no.6
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• pp.1067-1080
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• 2017

Objectives: This study analyzed the perception towards clinical practice education content held by dental hygiene students in dental institutions and their perceived importance of dental hygienists' clinical duties. Methods: The subjects of this study were 182 dental hygienists who were working at dental institutions in Seoul, Gyeonggi, and Chungcheong areas. A survey was conducted with a self-administered questionnaire. In the questionnaire, the clinical practice contents were classified into observation, preparation, and performance, and the importance of clinical duty was measured with a 3-point scale. For the clinical practice contents and the importance of duty, descriptive statistics and chi-square test were performed, and the study results were analyzed using STATA 11.0. Results: With regard to clinical practice contents, observation was mainly performed in oral & maxillofacilal radiology, preventive dentistry, periodontal medicine and oral medicine. In primary care and infection control, practice and observation were mainly performed. In the department of orthodontics and pediatric dentistry, observation and preparation were mainly conducted, while in oral surgery, conservative dentistry observation, preparation and practice were all conducted. With regard to clinical practice contents according to the dental institution, there were statistically significant differences in the type of dental institution and the duty (p>0.05). In terms of the importance of dental hygienist's duty, infection control, toothbrushing education for each patient, removal of plaque, and patient education after surgery were considered important. Conclusions: For clinical practice of the dental hygiene department, the education contents should be standardized in accordance with the importance of the dental hygienist's duty, a protocol for operation of practice should be developed, and a method of standardization of evaluation should be sought in the future.

• Clinical and Experimental Pediatrics
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• v.50 no.7
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• pp.606-612
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• 2007

Since the introduction of chemotherapy for the treatment of childhood leukemia more than 50 years ago, the results of childhood cancer have improved dramatically. The 5-year survival rate of disease, many of which were uniformly fatal in the prechemotherapy era, reached to more than 75%. This remarkable improvement in survival is a direct result of the incorporation of chemotherapeutics into treatment regimens that previously relied only on surgery or radiotherapy for the primary tumor. The multimodality approach, which integrates surgery and radiotherapy to control local disease with chemotherapy to eradicate systemic or metastatic disease, has become the standard approach to treating most childhood cancers. The overall improvement in outcomes in childhood solid tumors has been related to the development of multidisplinary cooperative studies that has permitted the development of well-designed tumor treatment protocols characterized by uniform staging criteria, sharing informations in pathologic classification, uniform methods for tumor markers, oncogenes, and other biologic and genetic factors. Important advances in the biologic study of cancer and its genetic basis led to a number of observations that impact directly on the management of childhood solid tumors. Identification of specific genes, oncogenes, tumor markers, and other biologic and pathologic factors plays an important role in both staging and clarifying the risk categorization of individual patients. Treatment of the patient is influenced by the recognition of specific risk factors. This knowledge has resulted in a change in the approach to care based not only on staging criteria, but also on risk-based management. This concept uses various risk factors of outcomes. Risk-based management allows for each patient to maximize survival, minimize long-term morbidity and improve the quality of life, especially for children's growth and development.

• Journal of Gastric Cancer
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• v.14 no.1
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• pp.39-46
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• 2014

Purpose: According to current guidelines, perioperative chemotherapy is an integral part of the treatment strategy for advanced gastric cancer. Randomized controlled studies have been conducted in order to determine whether perioperative chemotherapy leads to improved R0 resection rates, fewer recurrences, and prolonged survival. The aim of our project was to critically appraise three major studies to establish whether perioperative chemotherapy for advanced, potentially resectable gastric cancer can be recommended on the basis of their findings. Materials and Methods: We analyzed the validity of the three most important studies (MAGIC, ACCORD, and EORTC) using a standardized questionnaire. Each study was evaluated for the study design, patient selection, randomization, changes in protocol, participating clinics, preoperative staging, chemotherapy, homogeneity of subjects, surgical quality, analysis of the results, and recruitment period. Results: All three studies had serious shortcomings with respect to patient selection, homogeneity of subjects, changes in protocol, surgical quality, and analysis of the results. The protocols of the MAGIC and ACCORD-studies were changed during the study period because of insufficient recruitment, such that carcinomas of the lower esophagus and the stomach were examined collectively. In neither the MAGIC study nor the ACCORD study did patients undergo adequate lymphadenectomy, and only about half of the patients in the chemotherapy group could undergo the treatment specified in the protocol. The EORTC study had insufficient statistical power. Conclusions: We concluded that none of the three studies was sufficiently robust to justify an unrestrained recommendation for perioperative chemotherapy in cases of advanced gastric cancer.

• Journal of Periodontal and Implant Science
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• v.36 no.1
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• pp.265-272
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• 2006

Dental plaque, a biofilm consisting of more than 500 different bacterial species, is an etiological agent of human periodontal disease, It is therefore important to characterize interactions among periodontopathic microorganisms in order to understand the microbial pathogenesis of periodontal disease. Previous data have suggested a synergistic effect of tow major periodontal pathogens Porphyromonas gingivalis and Tannerella forsythia in the periodontal lesion. In the present study, to better understand interaction between P. gingivalis and T. forsythia, the coaggregation activity between these bacteria was characterized. The coaggregation activity was observed by a direct visual assay by mixing equal amount (1 ${\times}$ $10^9$)of T. forsythia and P. gingivaJis cells. It was found that the first aggregates began to appear after 5-10 min, and that the large aggregates completely settled within 1 h. Electron and epifluorescence microscopic studies confirmed cell-cell contact between two bacteria. The heat treatment of P. gingivalis completely blocked the activity, suggesting an involvement of a heat-labile component of P. gingivalis in the interaction. On the other hand, heat treatment of T. forsythia significantly increased the coaggregation activity; the aggregates began to appear immediately. The coaggregation activity was inhibited by addition of protease, however carbohydrates did not inhibit the activity, suggesting that coaggregation is a protein-protein interaction. The results of this study suggest that coaggregation between P. gingivalis and T. forsythia is a result of cell-cell physical contact, and that coaggregation is mediated by a heat-labile component of P. gingivalis and T. forsythia component that can be activated on heat treatment.