• Neonatal Medicine
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• v.25 no.1
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• pp.49-52
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• 2018

Arterial tortuosity syndrome (ATS) is a very rare autosomal recessive connective tissue disease characterized by generalized elongation and tortuosity of the medium- to large-sized arteries, and other systemic connective tissue manifestations. To date, this disease entity has not been reported in Korea. We report a case of ATS diagnosed in a neonate who presented with severe elongation and tortuosity of the aorta and its major branches, as well as the intracranial arteries. Additionally, the patient presented with a tortuous dilatation of the inferior vena cava, an aneurysmal dilatation of the extra-hepatic bile ducts, and an inguinal and sliding hiatal hernia. The diagnosis was confirmed using DNA sequencing analysis, and the patient demonstrated a compound heterozygosity for two novel mutations (c.738delG [p.Gln247Serfs*33] and c.362T>C [p.Ile121Thr]) in exon 2 of the SLC2A10 gene. Genetic analysis also confirmed that both parents were heterozygous carriers of the responsible mutations. Owing to such clinical manifestations, ATS is often misdiagnosed as other connective tissue diseases including Loeys-Dietz syndrome, Marfan syndrome, and Ehlers-Danlos syndrome. In patients presenting with a high index of suspicion, thorough clinical evaluation and screening for ATS including computed tomography or magnetic resonance angiography and target gene analysis are necessary for early diagnosis and management.

• International Journal of Oral Biology
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• v.46 no.1
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• pp.51-59
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• 2021

Periodontal disease is an inflammatory disease that affects the destruction of the bone supporting the tooth and connective tissues surrounding it. Periodontal ligament fibroblasts (PDLFs) induce overexpression of matrix metalloproteinase (MMP) involved in periodontal disease's inflammatory destruction. Osteoclasts take part in physiological bone remodeling, but they are also involved in bone destruction in many kinds of bone diseases, including osteoporosis and periodontal disease. This study examined the effect of baicalin on proteolytic enzymes' production and secretion of inflammatory cytokines in PDLFs and RAW 264.7 cells under the lipopolysaccharide (LPS)-induced inflammatory conditions. Baicalin inhibited the expression of the protein, MMP-1 and MMP-2, without affecting PDLFs' cell viability, suggesting its possibility because of the inhibition of phosphorylation activation of mitogen-activated protein kinase's p38, and the signal transduction process of nuclear factor κB (NFκB)-related protein. Also, baicalin reduced the expression of MMP-8 and MMP-9 in RAW 264.7 cells. This reduction is thought to be due to the inhibition of the signal transduction process of NFκB-related proteins affected by inhibiting p65RelA phosphorylation. Also, baicalin inhibited the secretion of nitric oxide and interleukin-6 induced by LPS in RAW 264.7 cells. These results suggest that baicalin inhibits connective tissue destruction in periodontal disease. The inhibition of periodontal tissue destruction may be a therapeutic strategy for treating inflammatory periodontal-diseased patients.

• The Journal of the Korean bone and joint tumor society
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• v.10 no.2
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• pp.124-129
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• 2004

The phosphaturic mesenchymal tumor mixed connective tissue variant (PMTMCT) is an extremely rare disease, and is frequently associated with oncogenic osteomalacia showing an paraneoplastic syndrome, which is characterized by phosphaturia, hypophosphatemia, normocalcemia and decreased levels of 1,25-dihydroxyvitamin D3 associated with a tumor. We experienced a 45-year-old female who had a soft tissue tumor on her right buttock causing oncogenic osteomalacia, which was satisfactorily treated by surgical excision of the mass.

• Clinical and Experimental Pediatrics
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• v.60 no.3
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• pp.77-85
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• 2017

Purpose: Fever is one of the most common symptoms in children. In previous studies, infectious disease was the most common cause of pediatric fever of unknown origin (FUO). The aim of this study is to investigate the etiology, clinical characteristics and prognosis of pediatric FUO in 21 century with more diagnostics available and to analyze the factors for certain disease categories. Methods: Among the children under 18 years old who were hospitalized at Samsung Medical Center from January 2000 to December 2014, the patients who met the criteria including fever of ${\geq}38.0^{\circ}C$ for longer than ${\geq}14days$ and failure to reach a diagnosis after one week of investigations were included. Results: Total 100 patients were identified. Confirmed diagnosis was achieved in 57 patients (57%). Among them, infectious diseases (n=19, 19%) were most common, followed by connective tissue diseases (n=15, 15%), necrotizing lymphadenitis (n=8, 8%), and malignancies (n=7, 7%). Children with fever duration over 28 days had a trend for higher frequency of connective tissue diseases (28.3%) except undiagnosed etiology. The symptoms such as arthritis, lymph node enlargement and only fever without other symptoms were significantly related with connective tissue diseases, necrotizing lymphadenitis and undiagnosed respectively (P<0.001). Ninety-two patients have become afebrile at discharge and 1 patient died (1%). Conclusion: Almost half of our patients were left without diagnosis. Although it has been known that infectious disease was most common cause of pediatric FUO in the past, undiagnosed portion of FUO have now increased due to development of diagnostic techniques for infectious diseases.

• Clinical and Experimental Pediatrics
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• v.51 no.8
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• pp.886-891
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• 2008

Mixed connective tissue disease (MCTD) is characterized by diverse symptoms including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, and dermatomyositis, associated with high titers of antibodies to extractable nuclear antigen (ENA), especially anti-ribonucleoprotein (anti-RNP) antibody. Since the first report of 25 cases with MCTD in adults, there have been only a few cases of MCTD reported in children. Here, we report a rare childhood case of MCTD in a 7-year-old girl presenting initially with Raynaud's phenomenon, swollen hands, and ulceration of the right index finger tip followed by alopecia and arthritis during follow-up.

• Journal of agricultural medicine and community health
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• v.23 no.2
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• pp.243-250
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• 1998

This study was analyzed through the reports which published on the subject matter of Su Dong-Myun from 1994 to 1997 and the medical record of patient in Su-Dong Myun health subcenter. The result are as follow: 1. The number of population in Su-Dong Myun(study area) was 5,475 in 1994, 5,707 in 1995, 6,079 in 1996 and 6,253 in 1997. In composition rate of population, "65 and over" of age group only showed markedly increasing tendancy from 9.8% in l995 to 10.2% in 1997. However, the rest of all age group showed decreasing tendancy. 2. Annual utilization rate showed decreasing tendancy, such as 247 in 1994. 203 in 1995, 146 in 1996, and 140 in 1997 per 1000 population. But visiting time is increasing tendency, such as 3.1 in 1994, 2.8 in 1995. 2.4 in 1996 and 3.4 in 1997 per disease case. 3. Age specific annual utilization rate, all age showed decreasing tendancy in the age group of "0-14", "15-44" and "45-64", however showed increasing tendency in the age group of "65 and over" from 1994 to 1997. 4. The major disease were disease of Respiratory system. Gastrointestinal system. Musculoskeletal system and Connective tissue. Skin and Subcutaneous tissue and Circulatory system The disease of Musculoskeletal system and Connective tissue and Circulatory system are increasing.

• The korean journal of orthodontics
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• v.28 no.3 s.68
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• pp.431-440
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• 1998

While periodontal disease results in the extrusion of upper incisors with interdental spacing in adult patient, the intrusive movement should be executed for the rehabilitation in terms of esthetics and function. The present study was peformed to investigate the periodontal response following orthodontic intrusion of teeth with infrabony pocket. Bone defect and periodontal disease were produced adjacent to the both maxillary second lateral incisors in four adult dogs. Four weeks later, a flap operation was performed to eliminate inflammation and a reference notch was made on the root surface at the level of bottom of each defect. Two weeks after periodontal surgery, 4 weeks of intrusion and subsequent another 4 weeks of retention was executed on the right side of incisors while left incisors served as the control. Through the histologic analysis, following results were obtained. 1. Histologic section of control side showed the reduction of pocket depth after flap operation. However, the status of long junctional epithelium was observed while new connective tissue attachment occurred in a small area adjacent newly formed cementum 2. Epithelial attachment was less around the intruded incisor while new connective tissue attachment was much more compared to the control side. 3. Plenty of cells were observed in the connective tissue around the reference notch of the intruded incisor and the degree of the new cementum formation was higher in the intruded incisor than the control. The results of the present study suggested that an orthodontic intrusion of periodontally involved and extruded incisors could be carried out with a desirable periodontal response, provided that inflammation is under control.

• Journal of Periodontal and Implant Science
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• v.30 no.3
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• pp.527-538
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• 2000

Several extraction cases with advanced bone loss as a result of periodontal disease, root or labial bone fracture, extensive caries, and periapical lesions occur esthetic, functional problems and severe bone loss. Therefore, to treat these cases used several surgical methods and socket preservation among this therapies have been evaluated simple, effective and good prognosis in the implant placement. Socket preservation therapy have been used with barrier membranes or/and graft materials. Deproteinized bovine bone mineral have been evaluated ideal grafting materials. Recently, calcium-phosphate thin film coated bovine bone powders were developed in our country, but the study for these material wasn't reported. When two types of xenograft materials were implanted in extraction sockets of Beagle dogs, the effects of these were analyzed after 4 weeks and 8 weeks histological views. The results of this study were as follows. 1. In control groups, 4 weeks after implantation, the extraction sockets were filled with connective tissue which has dilated vessels and epithelial growth. And after 8 weeks, irregular connective bundles were observed. But new bone formation was not seen. 2. In Bio-Oss groups, epithelial growth was not seen and bone powder was covered with connective tissue fiber. New bone formation was found around the interproximal bone. There was no special change seen after 8 weeks, connective tissue fibers became more regular, and bone growth near bone powder was not made well. 3. In Ca-P BBP groups, epithelial cells didn't grow in the extraction sockets, there was a lot of new bone made around the bone powder after 8 weeks, new bone around bone powder was replaced with mature bone. It is thought that bone powder grafting into the extraction sockets is very useful for conservation of ridge, and Ca-P BBP is more effective in bone formation than Bio-Oss.

• Journal of Chest Surgery
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• v.34 no.8
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• pp.626-629
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• 2001

Congenital polyvalvular disease is a connective tissue disorder affecting more than one heart valve with variable involvement of the entire valvular and subvalvular apparatus. It is frequently associated with the Trisomy 18 and trisomy 13-15 or ventricular septal defect and patent ductus arteriosus. We present an isolated case of congenital polyvalvular disease in a new born baby with a review of the pertinent literatures, which has not been described in Korea. The mass was discovered as a right atrial mass in the prenatal ultrasonography and it was thought to be either a hematoma or a myxoma in the preoperative echocardiography. Microscopic examination of the surgically resected mass showed irregular thickening, nodulation, and additional features of calcification and ossification in the valvular connective tissue on the body of anterior and septal leaflet of tricuspid valve. Congenital polyvalvular disease should be included in the differential diagnosis in cases showing valvular calcification or ossification in the fetal echocardiography.

• Journal of Society of Preventive Korean Medicine
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• v.3 no.2
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• pp.101-128
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• 1999

A study on disease treated at oriental medical treatment facilities (OMTF) and patients' satisfaction levels was conducted in order to figure out why the patients visited oriental medical doctors and the level of satisfaction of the patients for the services offered to them by oriental medical doctors. This study was performed from March 2 through May 31, 1998 by interviewing 1.532 persons living in major and small cities in korea. The results obtained were summarized as follows; 1. The general characteristics of subjects. The highest portion of each part was, 66.9% female, persons in the age group of over 60's 22.7%, high school graduated 34.9%, house wife 30.8%, The married 65.0%, Buddhist 36.9%, maj or city residents 60.2%, company covered insurance benefiter 39.0% and etc. 2. 40.5% of subjects visited OMTF for skeletal and connective tissue diseases. 21.5% for digestive system diseases. 16.2% for respiratory system diseases. 13.3% for circulatory system diseases and 9.0% for neurological problems. 3. 42.7% of males visited OMTF for skeletal and connective tissue diseases, which were the highest and respiratory system disorders, digestive system disorders, circulatory system disorders and neurological diseases in order. 39.4% of females visited OMTF for skeletal and connective tissue disorders which were the highest and other conditions such as digestive system, circulatory, respiratory, and neurological disorders in order. 4. The males with circulatory system disorders were treated by herbal medicine, combination of herbal medicine and acupuncture, only in order. The females with the some conditions above were treated by combination of herbal medicine and herbal medical and acupuncture only in order. The males and females with respiratory system and digestive system diseases were treated by herbal medicine, combination of herbal medicine and acupuncture only in order. But the males and females with skeletal and connective tissue diseases were by acupuncture are the highest in order. 5. The females and persons in the age group of over 60' s and house wife. the not married, the unhealthy persons, residents living in small cities, the persons with high income by medical treatments frequency in circulatory system diseases are the highest. 6, The females, middle school graduated and the married, persons in the age group of over 60's, unemployed, sales and service industry workers, Buddhists, major city residents, the unhealthy persons, the persons with middle income by medical treatments frequency in respiratory system diseases are the highest. 7. The females, persons in the age group of over 60's, under graduated or elementary school graduated, the unemployed and house wife, the unmarried, Buddhists, major city residents, the unhealthy persons, the persons with low income by medical treatments frequency in digestive system diseases are the highest. 8. The males, major city residents, old ages, under graduated or elementary school graduated, go earn officials, people grown in small city, the persons who had health insurance policies, the persons with low income, the unhealthy persons by medical treatments frequency in skeletal and connective tissue disorders diseases. 9. 50.8% of the respondents said that the treatments at the OMTF were very effective. 47.7% of them said that the treatments were effective. The males, persons in the age group of 40's, high school graduates, official workes, the married, the persons who did not have religion, major city residents, the persons who had health insurance policies, the persons with high income and the healthy persons said that the treatment effects at OMTF were satisfactory. 10. The patients' satisfaction rate for OMTF on each disease is, 1st. Musculo-Skeletal system(most satisfied), 2nd. the pregnancy & delivery complications, 3rd. Eye & ophthalmics, 4th. Respiratory system, 5th. Mental & bodily disorder, 6th. Digestive system and etc. 11. The factors affect OMTF are age, satisfaction for OMTF, current disease, religion, efficiency of Oriental Medicine, health condition and etc. This explained power of variable were 39.0%. 12. The satisfied factors for OMTF is correlate to educational level, and economical variables.