• Journal of Oral Medicine and Pain
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• v.32 no.4
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• pp.397-402
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• 2007

Sjogren's syndrome is a chronic autoimmune disorder of the exocrine glands with associated lymphocytic infiltrates of the affected glands. Primary SS presents alone as xerostomia, keratoconjunctivitis sicca and patotid gland enlargement, secondary SS occurs in connection with other autoimmune disorder such as rheumatitoid arthritis, systemic lupus erythematosus, or progressive systemic sclerosis. Among many other systemic complication, lymphoma is considered seriously. Patient with SS had a 44 times higher relative risk of lymphoma, and clinically identifiable lymphoma occurs in approximately 5% of patients with SS. So, patients with SS should be closely monitored. In dental office, diagnosis of SS is important in view of high risk of lymphoma. When a dentist diagnose and manage dry mouth, he or she should consider possibility of SS all the times and have knowledge of diagnostic criteria of SS.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.9-17
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• 2015

Glycogen storage disease (GSD) type Ia is rare inborn metabolic disorder, caused by glucose-6-phosphatase deficiency. It characterized by hepatomegaly, hypoglycemia, lactic acidosis, hypertriglyceridemia, and hyperuricemia and it is usually manifested in the infantile period. In addition, it is also associated with growth failure, pubertal delay, anemia, platelet dysfunction, osteopenia, and pulmonary hypertension. Hepatocellular adenoma and renal dysfunction are frequent late complications. Delayed diagnosis and inappropriate therapy lead to many complications such as growth failure, osteoporosis, refractory gout, renal failure, hepatocellular carcinoma (HCC), and pulmonary hypertension. Here, two Korean sisters diagnosed with GSD Ia, aged 33 and 36 respectively, were described and compared to recent articles about four adults with late diagnosis of GSD Ia. One sister had typical manifestations of GSD Ia including short stature (height, 145 cm), multiple hepatic adenoma, chronic kidney disease stage IV, and severe osteoporosis, whereas the older sister had normal stature (162 cm), one tiny hepatic nodule, and normal renal function. Direct sequencing of G6PC in two sisters identified a homozygous splicing mutation, c.645G>T, which is a prevalent mutation in Korea. Interestingly, our cases and four adults from recent reports had asymptomatic mild hypoglycemia and various manifestations including renal failure, HCC, fatty liver, or uncontrolled hyperlipidemia. These adult cases represent not only heterogenous phenotype to genotype within family members with GSD Ia but also long-term complications such as gouty arthritis, renal failure, and osteoporosis in untreated adult GSD Ia patients. In addition, lactic academia and hypertriglyceridemia are good markers of GSD Ia to distinguish from metabolic disease.

• Clinics in Shoulder and Elbow
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• v.2 no.2
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• pp.126-132
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• 1999

Purpose : To evaluate the functional and radiographic outcome of the modified Bosworth method in the surgical treatment of acute type V acromioclavicular joint dislocation. Materials and Methods: From June 1995 to May 1998, 20 patients were operated on for acute and complete acromioclavicular dislocation(Rockwood type V). The operative technique includes fixation of the coracoclavicular joint with Bosworth screw or 6.5mm cancellous screw and imbrication of trapezius and deltoid muscles. The average age was 34 years(range, 19 to 51 years). These 20 patients with an average follow-up of 18months, were evaluated clinically using the UCLA scoring system. Additional radiographical assessment was performed with stress radiographs. Results: Excellent or good clinical results were obtained in 95%(19 cases). And the average coracoclavicular interval ratio was decreased from 3.31(2.2-6.0) to 1.13(1-1.4) in stress radiographs. There were 4 cases of hetero­topic calcification postoperatively but there was no correlation with clinical result. Posttraumatic A-C joint arthritis was developed in one case. In that case, the distal clavicular resection was done under the arthroscopic technique. Conclusion: The severe displacement observed with type V injuries is incompatible with normal shoulder function if the shoulder is left in its displaced position. In type V injuries, significant damage to the deltoid and trapezius musculature and overlying fascia occurs, therefore open reduction and good fixation must be obtained with imbrication of trapezius and deltoid muscles. In our type V acute complete acromioclavicular dislocation, the modified Bosworth technique provides excellent results with a low complication rate.

• Tuberculosis and Respiratory Diseases
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• v.46 no.6
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• pp.869-878
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• 1999

Intravesical instillation of the bacillus Calmentte-Gu$\acute{e}$rin(BCG), an attenuated strain of Mycobacterium bovis, is an approved method for the treatment of superficial bladder cancer. Because BCG is a living organism, the potential for infection exists. BCG is generally well tolerated, with complications in less than 5% of those treated with use of current practices. The most frequent symptoms of toxicity associated with intravesical BCG immunotherapy include bladder irritation, frequency, and dysuria. Systemic reactions are less common but more serious than local side effects, and include fever, chills, malaise, rash, hepatitis, pneumonitis, arthritis and sepsis. In rare cases, BCG treatment can result in a systemic infection that requires antituberculous therapy. The pulmonary toxicity that results from intravesical BCG treatment is generally characterized by one of two types : systemic allergic reaction with pulmonary reticulonodular opacities depicted on chest radiographs with cellular findings consisting of activated lymphocytes, and actual BCG mycobacteremia with a miliary pattern depicted on chest radiographs and granuloma formation which rarely results in positive acid-fast stain or culture results. Recently we experienced two types of pulmonary complications following intravesical BCG immunotherapy in patients with superficial bladder cancer. We report two cases with a review of literatures.

• Research in Community and Public Health Nursing
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• v.12 no.3
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• pp.639-646
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• 2001

The purpose of this study is to analyze the trend of nursing research related to family support and to provide an effective direction for future research by conducting a review of the Korean nursing literature from 1980 to 2000. A total of 73 studies were analyzed with the following results. 1) The number of studies related to family support has been rapidly increasing since 1984, and 77% of those were conducted in the 1990s. 48 studies, 65% of those analyzed. were degree-based research including 4 doctoral theses (4%) and 45 master's theses (62%). 2) Based on the objective frame of study types developed by Lee et al. (991), the types of studies were analyzed: 56 studies, 76.6% of those reviewed, belonged to factor-relating: 9 studies (12.4%) belonged to factor-isolating: 7 studies (9.6%) belonged to situation-producing: and only 1 study (1.4%) belonged to situation-relating research, respectively. 3) In terms of research design, most studies of analyzed (90.4%) were conducted by non-experimental design, and 7 studies (9.6%) were conducted by experimental design. But, qualitative research related to family support was not found. 4) In terms of research subjects, target populations of experimental studies were patients with arthritis, mental disorder, tuberculosis, coronary artery disease and hypertension. In correlational studies, 51 studies, 69.9% of those analyzed, were conducted to patients with renal failure, cancer and hemiplegia, and 5 studies (6.8%) were conducted to aged people and adolescents. 5) Study concepts used in correlational studies were quality of life, anxiety and depression, self-esteem, hope, role behavior, compliance and self-care activities. The quality of life was most often used among studies with 12.5% of those analyzed. 6) Nursing interventions, including diet therapy, family involving education and supportive care, used in experimental studies mostly showed positive effects on client involvement and home health improvement. However, the nursing interventions of mental health rehabilitation and resistance exercise showed neutral and negative results. The findings of this study provide evidence that more empirical research including experimental or qualitative studies should be conducted actively to improve nursing practice related to family support. Also, to promote more diverse nursing interventions, a family assessment tool especially for Korean families needs to be developed.

• Journal of Oral Medicine and Pain
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• v.36 no.1
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• pp.39-51
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• 2011

The aims of this study were to investigate whether the facial skeletal patterns previously reported to be related to temporomandibular disorder (TMD) in other studies could be consistently observed in the TMD patients diagnosed according to Research Diagnostic Criteria for Temporomandibular Disorder (RDC/TMD) Axis I and evaluate its usability in the orthodontic clinics to examine the patients with TMD related symptoms. The clinical records and radiographs of female patients who visited the TMD and Orofacial Pain Clinic of Seoul National University Dental Hospital and were diagnosed as TMD were consecutively filed for this study. Patients were clinically examined and diagnosed according to the revised diagnostic algorithms of RDC/TMD Axis I and the lateral cephalogram, panoramic orthopantomogram, temporomandibular joint (TMJ) orthopantomogram, and transcranial radiograph of each patient were taken and digitalized. The data of patients who were under 18 years of age or had any systemic disease, trauma history involving the TMJ, or skeletal deformity at the time of the first examination were excluded. The remaining data of 96 female patients were finally analyzed. The obtained results were as follows: 1. There are no significant differences of cephalometric measurements between RDC I (muscle disorders) diagnostic groups. 2. Only the articular angle of the RDC group IIc (disk displacement without reduction without limited opening) patients was larger than patients of the no diagnosis of RDC II group (disk displacement). 3. Larger articular angle and smaller facial height ratio were observed in RDC IIIc group (osteoarthrosis) compared to IIIa group (arthralgia). Larger articular angle, larger Bjork sum, smaller posterior facial height, and smaller facial height ratio were observed in RDC group IIIc compared to no diagnosis of RDC III group (arthralgia, arthritis, and arthrosis). 4. According to the results of cephalometric analysis in simplified RDC groups, smaller overjet was observed in muscle disorders (MD) group. Facial height ratio and IMPA were smaller and articular angle was larger in disk displacements (DD) group than in no diagnosis of DD group. In arthrosis (AR) group, posterior facial height, and facial height ratio were smaller, and articular angle, gonial angle, facial convexity, FMA, Bjork sum, and ANB were larger than in no diagnosis of AR group. In joint pain (JP) group, only posterior facial height was smaller than no diagnosis of JP group. In conclusion, Facial morphologic patterns showing posterior-rotated mandible and lower posterior facial height is related to RDC group II and III diagnosis of the TMJ in female TMD patients. RDC/TMD Axis I diagnosis can provide a good clinical diagnostic tool for the standardized examination of the TMJ in orthodontic clinics.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.63-67
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• 2005

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis, characterized by cutaneous palpable purpura, gastrointestinal(GI) symptoms, arthritis and renal involvement. In general, the prognosis is determined by GI complication as well as the severity of nephritis. In this study, we analyzed the statistical relationship between the GI symptom and other clinical findings for assessing the prognosis, and evaluated abdominal ultrasonographic findings for early diagnosis of this disease with atypical clinical presentation and early detection of serious GI complications. Methods : One hundred seventy seven patients with HSP in the Department of Pediatrics, Wonkwang University Hospital from January 1994 to June 2004, were enrolled. We retrospectively analyzed charts about clinical and abdominal ultrasonographic findings, and classified our patients into two groups(GI-Sx(-), GI-Sx(+)) for statistical analysis. Results : The ratio of female to male is 1.5 : 1. The peak age incidence was five to eight years in 95 cases(53%). The GI symptoms appeared in 117 cases(66%), which include abdominal pain 115 (98 %), tenderness 45(38%), nausea and vomiting 35(30%), bloody stool 10(8.5%), diarrhea four(3.4%), rebound tenderness four(3.4%), and also intussusception and appendicitis were complicated in five and two cases respectively. GI-Sx(+) group had an increased risk of renal involvement and relapse than the GI-Sx(-) group. But there were no relationships about sex and age incidence, or other clinical and laboratory findings between two groups. Ultrasonographic findings in 98 patients with GI symptoms included small bowel thickening in 70 cases(71%) in which duodenum, jejunum and ileum were involved in 71%, 45.7%, 40% respectively, small bowel dilatation in 41 cases(42%), lymph node swelling in 46 cases(47%), and ascites in 25 cases(25.5%). Conclusion : GI symptoms in patients with HSP suggested increased risk of renal involvement and relapse. Abdominal ultrasonography could be helpful in the early diagnosis on atypical clinical presentation and early detection of serious GI complication in these patients.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.898-902
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• 2003

Purpose : To determine the clinical association of diagnostic criteria and the prevalence of autoantibodies in newly diagnosed children with juvenile dermatomyositis(JDM). Methods : Thirty-two children with JDM were identified at Seoul National University Children's Hospital from March 1985 to March 1999 by retrospective review. The diagnosis of JDM was based on the criteria proposed by Bohan and Peter. We investigated for the presence of several autoantibodies: antinuclear(ANA), double-stranded DNA, anti-Sm, anti-ribonucleoprotein(RNP), anti-SSA/ SSB, anti-Jo1, anti-Scl-70 antibodies and rheumatoid factor(RF). Results : Sex ratio and age at diagnosis were similar to data published in other studies. All the newly diagnosed children with JDM had a typical rash(100%) and proximal muscle weakness(100%); 17(53%) had muscle pain or tenderness; 10(31%) calcinosis; eight(25%) dysphagia; eight(25%) arthritis, and seven(22%) fever. Muscle enzymes were elevated in 90% of the patients. Of the 27 patients who had an electromyogram, 20(70%) had diagnostic results. Sixteen(70%) of biopsied patients had appropriated results for JDM. Patients were negative for all autoantibodies except ANA and RF. ANA and RF were detected in 47% and 7% of the patients respectively. Conclusion : Although the sensitivity of the criteria proposed by Bohan and Peter is superior, each of these criteria has possible confounding factors. Additional criteria may be needed for early diagnosis of JDM. Based on our findings of autoantibodies in JDM, we do not recommend routine testing for autoantibodies in children with typical JDM.

• Pediatric Infection and Vaccine
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• v.17 no.2
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• pp.108-113
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• 2010

Purpose : The aim of this study was to identify clinical availability of serum procalcitonin (PCT) compared with C-reactive protein (CRP) in prediction of bacterial infection in children. Methods : A retrospective study was conducted with children who had been admitted to the Department of Pediatrics with bacterial and viral infection between April 2008 and March 2009 and children who were admitted with Juvenile rheumatoid arthritis (JRA) between August 2007 and July 2009. Serum PCT levels were measured using an enzyme-linked fluorescent assay. Results : The study population included 10 patients with bacterial infection (group I), 69 with viral infection (group II), and 35 with JRA (group III). Mean PCT levels were significantly higher in group I than in group II or group III (P<0.05). Mean CRP levels were significantly higher in group I than in group II (P<0.05); however, mean CRP levels were not significantly higher in group I than in group III (P>0.05). Using a cutoff of 0.5 ng/mL for PCT and 8 mg/L for CRP, sensitivity and specificity in distinguishing between group I and the other groups were 60.0% and 92.3% for PCT and 60.0% and 40.1% for CRP, respectively. Positive and negative predictive values were 42.9% and 96.0% for PCT and 10.0% and 92.6% for CRP, respectively. Conclusion : Measurement of PCT concentrations appears to be more useful than CRP for distinguishing between bacterial infection and non-bacterial diseases in children.

• Clinics in Shoulder and Elbow
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• v.14 no.1
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• pp.59-66
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• 2011

Purpose: We wanted to evaluate the clinical and radiological outcomes and the prognosis of various surgical treatments for the distal clavicle fracture with an acromioclavicular joint injury. Materials and Methods: A retrospective study of 21 patients with a minimum of 12 months follow up was done. We classified acromioclavicular (AC) injury into type I (only intra-articular fracture (IAF), 5 cases), type II (IAF with widening of the AC joint > 7 mm, 9 cases) and type III (IAF with AC joint superior subluxation > 50%, 7 cases). The distal clavicle fractures were fixed using plate (9 cases), mini screws (1 case), K wire and tension band wiring (10 cases) and transarticular pinning (1 case). Acromioclavicular or coracoacromial ligament reconstruction was not done in all the cases. Results: In 20 of 21 cases, bone union was achieved at an average of 8.4 weeks. Traumatic arthritis (5 cases), AC joint widening (4 cases) and AC joint subluxation (2 cases) were noted at the last follow up. The average UCLA score was 32.6 in the type I AC joint injuries, 34 in type II and 34.1 in type III. There was no relationship between the clinical outcomes and the preoperative AC joint injury pattern, postoperative traumatic arthritis, AC joint widening or AC joint subluxation (p>0.05). Conclusion: Satisfactory results were achieved by acute reduction and firm fixation of the distal clavicle fracture with AC joint injury. There was no relationship between the pattern of AC joint injury, the residual radiologic findings and the functional outcome.