• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.3
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• pp.148-154
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• 2016

A urea cycle disorder is a condition caused by a defect of the enzymes in the urea cycle, and deficiency of ornithine transcarbamylase (OTC), which converts carbamoyl phosphate and ornithine into citrulline, is the most common type of the disorder. OTC deficiency induces the accumulation of precursors of urea, ammonia, and glutamine, leading to neurological symptoms including hypotonia, respiratory failure, seizure, lethargy, and coma and sometimes to death. Because OTC deficiency is inherited in an X-linked manner, typical symptoms such as vomiting, poor feeding, and lethargy appear mainly in male neonates. We recently had a case that presented with neonatal onset lethargy, vomiting, and apnea in a 4-day-old boy. He was diagnosed with OTC deficiency by biochemical phenotype, including hyperammonemia and an increased orotic acid level in the urine. Genetic analysis of the OTC gene showed a novel mutation c.780_781insCAGGCAGTGT (p.Ile261Glnfs*35). He was treated for hyperammonemia using continuous venovenous hemofiltration (CVVH) at 118 hours after birth. After 4 days of CVVH, his consciousness and blood ammonia concentration were normalized, and he was discharged at the age of 53 days. At around 12 months of age, bilateral femur fractures and osteomyelitis occurred in this patient. Two months after the fracture, he died of septic shock, insulin-resistant hyperglycemia, and multi-organ failure.

• Journal of Oral Medicine and Pain
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• v.34 no.3
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• pp.295-301
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• 2009

Recently, bisphosphonate-associated osteonecrosis of the jaw(BONJ) is added to the list of diseases of the oromaxillofacial region. BONJ is defined as exposed bone in the jaw that does not heal within 8 weeks after identification, in a patient who has been received to bisphosphonates and has not taken radiation therapy to the craniofacial region. Bisphosphonates binded to bone mineral are concentrated in highly active remodeling site, reside in the skeleton for a long time, and do a role as powerful inhibitors of bone resorption. As the patients receiving bisphosphonates therapy grow in number, the patients of BONJ would go on increasing in Korea. We would like to present two patients who were suspected to BONJ, describe the outline of BONJ, and mention importance of our understanding about BONJ. BONJ is rare disease, but once it develops, its prognosis is very poor. Our adequate understanding of BONJ is necessary to prevent it and cope with it properly.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.46 no.4
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• pp.240-249
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• 2020

Objectives: Although the side effects of radiation therapy vary from mucositis to osteomyelitis depending on the dose of radiation therapy, to date, an experimental animal model has not yet been proposed. The aim of this study was to develop an animal model for assessing complications of irradiated bone, especially to quantify the dose of radiation needed to develop a rat model. Materials and Methods: Sixteen Sprague-Dawley rats aged seven weeks with a mean weight of 267.59 g were used. Atraumatic extraction of a right mandibular first molar was performed. At one week after the extraction, the rats were randomized into four groups and received a single dose of external radiation administered to the right lower jaw at a level of 14, 16, 18, or 20 Gy, respectively. Clinical alopecia with body weight changes were compared and bony volumetric analysis with micro-computed tomography (CT), histologic analysis with H&E were performed. Results: The progression of the skin alopecia was different depending on the irradiation dose. Micro-CT parameters including bone volume, bone volume/tissue volume, bone mineral density, and trabecular spaces, showed no significant differences. The progression of osteoradionecrosis (ORN) along with that of inflammation, fibrosis, and bone resorption, was found with increased osteoclast or fibrosis in the radiated group. As the radiation dose increases, osteoclast numbers begin to decrease and osteoclast tends to increase. Osteoclasts respond more sensitively to the radiation dose, and osteoblasts are degraded at doses above 18 Gy. Conclusion: A standardized animal model clinically comparable to ORN of the jaw is a valuable tool that can be used to examine the pathophysiology of the disease and trial any potential treatment modalities. We present a methodology for the use of an experimental rat model that incorporates a guideline regarding radiation dose.

• Journal of Periodontal and Implant Science
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• v.38 no.sup2
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• pp.317-324
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• 2008

Purpose: Aggregatibacter actinomycetemcomitans was associated with localized aggressive periodontitis, endocarditis, meningitis, and osteomyelitis. The cytolethal distending toxin (CDT) of A. actinomycetemcomitans was considered as a key factor of these diseases is composed of five open reading frames (ORFs). Among of them, An enzymatic subunit of the CDT, CdtB has been known to be internalized into the host cell in order to induce its genotoxic effect. However, CdtB can not be localized in host cytoplasm without the help of a heterodimeric complex consisting of CdtA and CdtC. So, some studies suggested that CdtC functions as a ligand to interact with GM3 ganglioside of host cell surface. The precise role of the CdtC protein in the mechanism of action of the holotoxin is unknown at the present time. The aim of this study was to generate recombinant CdtC proteins expression from A. actinomycetemcomitans, through gene cloning and protein used to investigate the function of Cdt C protein in the bacterial pathogenesis. Materials and Methods: The genomic DNA of A. actinomycetemcomitans Y4 (ATCC29522) was isolated using the genomic DNA extraction kit and used as template to yield cdtC genes by PCR. The amplifed cdtC genes were cloned into T-vector and cloned cdt C gene was then subcloned to pET28a expression vector. The pET28a-cdtC plasmid expressed in BL21 (DE3) Escherichia coli system. Diverse conditons were tested to opitimize the expression and purification of functional CdtC protein in E. coli. Results: In this study we reconstructed CdtC subunit of A. actinomycetemcomitans Y4 and comfirmed the recombinant CdtC expression by SDS-PAGE and Western Blotting. The expression level of the recombinant CdtC was about 2% of total bacterial proteins. Conclusion: The lab condition of procedure for the purification of functionally active recombinant CdtC protein is established. The active recombinant CdtC protein will serve to examine the role of CdtC proteins in the host recognition and enzyme activity of CDT and investigate the pathological process of A. actinomycetemcomitans in periodontal disease.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.40
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• pp.27.1-27.8
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• 2018

Purpose: Various complications occur when a maxillofacial fracture is malunionized or improperly resolved. Malocclusion is the most common complication, followed by facial deformity, temporomandibular joint disorder (TMD), and neurological symptoms. The purpose of this study was to evaluate the dental treatment of postoperative complications after maxillofacial fracture. Materials and methods: In this study, nine patients with a postoperative complication after maxillofacial fracture who had been performed the initial operation from other units and were referred to the authors' department had been included. Of the nine patients, six had mandibular fractures, one had maxillary fractures, one had maxillary and mandibular complex fractures, and one had multiple facial fractures. All the patients had tooth fractures, dislocations, displacements, and alveolar bone fractures at the time of trauma, but complications occurred because none of the patients underwent preoperative and postoperative dental treatment. Malocclusion and TMD are the most common complications, followed by dental problems (pulp necrosis, tooth extrusion, osteomyelitis, etc.) due to improper treatment of teeth and alveolar bone injuries. The patients were referred to the department of dentistry to undergo treatment for the complications. One of the nine patients underwent orthognathic surgery for a severe open bite. Another patient underwent bone reconstruction using an iliac bone graft and vestibuloplasty with extensive bone loss. The other patients, who complained of moderate occlusal abnormalities and TMDs such as mouth-opening limitation, underwent occlusal treatment by prosthodontic repair and temporomandibular joint treatment instead of surgery. Results: One patient who underwent orthognathic surgery had complete loss of open bite and TMD after surgery. One patient who underwent reconstruction using an iliac bone graft had a good healing process. Other patients were treated with splint, injection, and physical therapy for mouth-opening limitation and temporomandibular joint pain. After treatment, the TMDs were resolved, but the remaining occlusal abnormalities were resolved with prosthetic restoration. Conclusions: Considering the severity of malocclusion and TMJ symptom and the feasibillity of reoperation, nonsurgical methods such as orthodontic and prosthodontic treatments and splint therapy can be used to manage the dental and TMD complication after the trauma surgery. However, reoperation needs to be strongly considered for severe malocclusion and TMD problem.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.46 no.3
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• pp.191-196
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• 2020

Objectives: Beyond the original application approved by the U.S. Food and Drug Administration, recombinant human bone morphogenetic protein-2 (rhBMP-2) is used for medication-related osteonecrosis of the jaw (MRONJ) treatment because of its bone remodeling enhancement properties. The purpose of the study was to investigate the bone formation effect of rhBMP-2/absorbable collagen sponge (ACS) in patients with MRONJ. Materials and Methods: In this retrospective cohort study, 26 female patients diagnosed with MRONJ and who underwent mandibular sequestrectomy at Ajou University Dental Hospital from 2010 to 2018 were included. The experimental group was composed of 18 patients who received rhBMP-2/ACS after sequestrectomy, while the control group was composed of 8 patients who did not receive rhBMP-2/ACS after sequestrectomy. A total dose of 0.5 mg of rhBMP-2 was used in the experimental group at a concentration of 0.5 mg/mL. Follow-up panoramic X-rays were taken immediately after the surgery and more than 6 months after the surgery. Using those X-rays, a radiographic index of bone defect area was calculated using the modified Ihan Hren method, which measures radiographic density of the normal bone and the defect site. Results: This study suggests that rhBMP-2 contributes to new bone formation. The mean radiographic index immediately after surgery and more than 6 months after the surgery for the experimental group was 68.4% and 79.8%, respectively. The mean radiographic index immediately after surgery and more than 6 months after the surgery for the control group was 73.4% and 76.7%, respectively (Wilcoxon signed rank test, P>0.05). The mean radiographic index increased 11.4% in the experimental group and 3.27% in the control group (Mann-Whitney U-test, P<0.05). Conclusion: Based on the results, use of rhBMP-2/ACS on bone defect sites after sequestrectomy could be a successful strategy for treatment of MRONJ patients.

• The Journal of the Korean bone and joint tumor society
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• v.9 no.1
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• pp.18-23
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• 2003

Purpose: Delay in the diagnosis of the primary malignant bone tumors may critically influence the chance of the patients' survival and the limb sparing, but the primary malignant bone tumors are so rare that most doctors have little experience in these challenging diseases. The purpose of the current study is to examine the initial symptoms of osteosarcoma and chondrosarcoma, and to shorten the delay of diagnosis. Materials and Methods: The data was based on the questionnaires and the medical records from 139 patients whose histological diagnoses were confirmed in the authors' institution. There were 108 patients of osteosarcoma and 31 patients of chondrosarcoma. Eighty-six were male and fifty-three were female. The mean age of the patients was 20.2 years in osteosarcoma, and 42.4 years in chondrosarcoma. Results: The most common symptom that the patient consult the doctor was pain (93.5% of osteosarcoma patients and 61.3% of chondrosarcoma patients). Among them, 76.2% of osteosarcoma and 57.8% of chondrosarcoma patients complained the night pain. The history of trauma was evident in 48.5% of osteosarcoma patients and one patient of chondrosarcoma. At the first medical visit, the malignant bone tumor was suspected in 61.1% of osteosarcoma and 64.5% of chondrosarcoma patients. Fracture was the most common misdiagnosis in osteosarcoma (16.7%), and the osteomyelitis in chondrosarcoma (19.4%). Initial radiographic examination and the adult age were shown to increase the rate of correct diagnosis of both diseases (p<0.05). Patient's delay and doctor's delay were significantly longer in chondrosarcoma patients than in osteosarcoma. Initial radiography led to shorten the doctor's delay, and the axial location of the tumor lengthened the doctor's delay. Trauma and the young age were believed to shorten the patient's delay. Conclusion: Careful history taking, including the night pain and trauma, would be mandatory for the early diagnosis of the primary malignant bone tumors, and the initial radiographic examination and periodic follow-up can increase the rate of correct diagnosis.

• Journal of Chest Surgery
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• v.42 no.2
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• pp.220-225
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• 2009

Background: Sternoclavicular septic arthritis manifests serious complications such as abscess, osteomyelitis, mediastinitis and empyema; therefore, a prompt diagnosis and appropriate treatment are necessary. Material and Method: The treatment results of eight patients with sternoclavicular septic arthritis and who had been surgically treated at our institutions between September 2005 and July 2008 were retrospectively reviewed. The surgical treatment they underwent was en bloc resection, including partial resection of the sternum, the clavicular head and the 1st rib. Result: The patients ranged in age from 40 to 74 years with an average of $55.1{\pm}10.3$ years. Five were men and three were women. There were 6 patients with spontaneous sternoclavicular septic arthritis and 2 patients had their condition induced by central venous catheters. The pathogens isolated from the patients’ blood and wounds were MRSA (3), Streptococcus intermedius (1), Streptococcus agalactiae (1) and Pseudomonas luteola (1). One patient expired from aggravation of preoperative sepsis on POD 31. Conclusion: The life-threatening complications from sternoclavicular septic arthritis can progress and lead to death unless appropriate treatment is administered. A prompt diagnosis, appropriate antibiotics therapy and effective surgical treatment such as radical en bloc resection can reduce the morbidity and mortality of this malady.

• Journal of Home Health Care Nursing
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• v.3
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• pp.53-66
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• 1996

Home Health Care is one part of the total health care system. It includes health care services that link the hospital to the community. While it is important for early discharge patients, home care is also important for people with chronic illnesses or handicapping conditions. In 1989 the Korean government passed a law that opened the way for formal development of home health care services beginning with education programs to certify nurses for home care, and then demonstration home care services. Part of the mandate of the demonstration projects was evaluation of home care services. This study was done in order to provide basic data that would contribute to the development of records that could be used for evaluation through a retrospective audit and to examine the care that had been given in Home Care at Wonju Christian Hospital over a twenty year period from 1974 to 1994. The purposes of the study were : to identify to characteristics of the clients who had received home care, to identify the reasons for client referrals, to identify the nursing problems of these clients, to identify the nursing care provided to these clients, and to identify differences in these areas over the twenty year period. The study was a descriptive study involving a retrospective audit of the client records. Demographic data on all clients were included : 4,171 clients from 2,564 families. Data on referrals, nursing diagnosis and nursing interventions were from even numbered records which had a patient problem list included in the record, 2,801 clients, Frequencies and ANOVA were used in the analysis. The results of the study showed that the majority of the clients were from Wonju city /county. There were more women than men related to the high number of postpartum clients(1,300). The high number of postparttum clients and newborns was also evident in the age distribution. An the number of maternal-child clients decreased over the 20 years, the mean age of the clients increased significantly. Other factors also contributed to this change ; as increasing number of clients with brain injuries or with cancer, and fewer children with burns, osteomyelitis and tuberculosis. There was a decrease in the mean number of visits and mean length of coverage, reflecting a movement towards a short term acute care model. The number of new clents dropped sharply after 1985. The reasons for this are : the development of other treatment alternatives for clients, the establishment of an active wellbaby clinic, many more options plus a decreasing number of new cases of Hansen's Disase, and insurance that allows people with burns to be kept in hospital until skin grafts are healed. Socioeconomic changes have resulted in an increase in the number of cases of cancer, stroke, head injuries following car accidents, and of diabetes. Of the 2,801 client records, 2,541(60.9%) contained a written referral but for 1,802 it contained only the medical diagnosis. The number of records with a referral requesting specific nursing care was 739(29.1%). Many family members who were identified as in need of nursing care had no written referral. Analysis of the patient problem list showed that 41.9% of the enteries were nursing diagnoses. Others incuded medical diagnosis, symptoms, and plans. The most frequently used diagnoses were alteration in nutrition, less than body requirements(115 entries), alteration in skin integrity(114), knowledge deficit(111), pain(78), self-care deficit(66), and alteration in pattern of urinary elimination(50). These are reflected in the NANDA categories for which the highest number of diagnosis was in the Exchanging pattern(446), followed by Moving(178), Feeling(136) and Knowing (115). Analysis of the frequency of interventions showed that exercise and teaching about exercise was the most frequent intervention, followed by teaching concering the need for follow-up care, checking vital signs, managing nutritional problems, managing catheters, giving emotional support, changing dressings, teaching about medication, teaching (subject not specified), teaching about diet, IM and IV medications or fluid, and skin care, in that order. Recommendations included: development of a record that would allow for efficient recording of frequently used nursing diagnoses and nursing interventions: expansion of the catchment area for Home Care at Wonju Christian Hospital ; expansion of the service to provide complication prevention, rehabilitation services, and support to increase the health maintenance /health promotion of the people being served as well as providing client dentered care ; and development of a clinical record that will allow efficient data collection from records, even though the recording is done by a variety of health care providers.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.967-972
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• 2002

Purpose : A retrospective study was undertaken to evaluate the usefulness of low risk criteria for identifying febrile infants younger than three months unlikely to have serious bacterial infection. Methods : We conducted a retrospective study of 527 infants younger than three month with a axillary temperature ${\geq}37.4^{\circ}C$. If they met the following all four criteria, appear well, WBC $5,000-20,000/mm^3$, urine stick WBC(-) and nitrite(-), CSF WBC < $10/mm^3$, they were considered at low risk for serious bacterial infection(SBI). SBI was defined as a positive culture of urine, blood, or cerebrospinal fluid. The sensitivity, specificity, negative predictive value and positive predictive value of the low risk criteria were calculated. Results : Of 527 febrile infants, 110(21.0%) had serious bacterial infections. The 2.7% who met the low risk criteria had SBI and negative predictive value was 97.3%. SBI was diagnosed in 103 infants(38.6%) who didn't meet the low risk criteria including urinary tract infection(78.6%), most commonly, bacteremia(16.5%), bacterial meningitis(8.7%), Salmonella gastroenteritis(1%), osteomyelitis( 1%), septic arthritis of hip joint(1%). There were no differences in the sensitivity and negative predictive value according to the monthly-age-group. Conclusion : This low risk criteria to identify infants unlikely to have SBI early is available, however low risk infants must be carefully observed.