• Maxillofacial Plastic and Reconstructive Surgery
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• 제34권3호
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• pp.220-225
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• 2012

Wegener's granulomatosis (WG) is an autoimmune disease of unknown etiology characterized by the triad of necrotizing granulomatous lesion in the upper and lower respiratory tracts or both, disseminated vasculitis involving both small arteries and veins, and necrotizing glomerulonephritis. The most common oral lesions associated with WG are ulceration and strawberry gingivitis. A 47-years old man in medical care associated WG was consulted our Division of Oral and Maxillofacial Surgery for the chief complaint of toothaches. Pre-operative panorama showed the alveolar radiolucency and the loss of lamina dura regarding the left upper teeth. An oropharyngeal magnetic resonance imaging also revealed the increased bone marrow signal intensity on the left maxilla. Under the impression of maxillary osteonecrosis due to WG, maxillary saucerization with removal of involved teeth was performed. We obtained good results and report the first case of WG in Korea, with the review of literatures regarding oral and general systemic features.

• Pediatric Infection and Vaccine
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• 제23권1호
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• pp.67-71
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• 2016

결절성 홍반은 소아에서 가장 흔한 피하지방층의 염증으로 하지의 압통을 동반한 결절을 특징으로 한다. 이 질환은 다양한 원인에 의해 발생되지만 공통된 조직소견을 보여준다. 저자들은 임상적 소견 및 혈청학적 방법으로 마이코플라즈마 폐렴균에 의한 결절성 홍반을 진단하였고 조직병리적인 접근을 통해 결절성 홍반의 면역병인에 대해 고찰해 보았다. 조직병리소견은 제 4형 면역반응 염증을 보였으며 이는 다양한 원인에 의한 결절성 홍반의 발생 기전을 이해하는데 도움을 줄 것이다.

• Clinical and Experimental Pediatrics
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• 제48권1호
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• pp.81-84
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• 2005

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP)는 피부, 관절, 위장관 그리고 신장을 침범하는 혈관염으로 혈소판 감소증이 없는 자반증을 전형적인 증상으로 하는 질환으로 그 원인에 대하여 여러 가지 가설들이 있지만 아직 확실치 않다. HSP에서 신장 침범은 이차적으로 오는 혈관염으로 임상적으로 혈뇨와 단백뇨의 증상을 보이고 병리학적 소견은 IgA 신염과 매우 유사하다. 그 외에도 HSP 신염은 다양한 형태의 사구체 신염을 보이는데 막성 사구체 신염은 드물다. 이에 저자들은 HSP의 임상 양상을 가지면서 혈청 내 보체의 감소, 단백뇨 및 고혈압을 보이고 조직학적으로 막성 사구체 신염 소견을 보인 6세 환아를 경험하였기에 이를 문헌 고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제56권1호
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• pp.13-18
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• 2013

Purpose: Kawasaki disease (KD) is a systemic vasculitis and affects many organ systems. It often presents sterile pyuria, microscopic hematuria, and proteinuria due to renal involvement. The aims of this study were to define clinical characteristics of acute KD patients with pyuria and to analyze meaning of pyuria in KD. Methods: The medical records and laboratory findings including serum and urine test of 133 patients with KD admitted to Yeungnam University Hospital from March 2006 to December 2010 were reviewed retrospectively. Results: Forty patients had sterile pyuria and their clinical characteristics including age, gender and body weight were not significantly different with those who did not have pyuria. Fever duration after treatment was significantly longer in KD patients with pyuria. Erythrocyte sedimentation rate, C-reactive protein and serum concentration of alanine aminotransferase were significantly higher in patients with pyuria. Hyponatremia and coronary artery lesion were seen more often in patients with pyuria but there was no significant difference. Also serum blood urea nitrogen was significantly higher in KD patients with pyuria. Urine ${\beta}_2$-microglobulin was elevated in both patients groups and showed no difference between two groups. Conclusion: We found more severe inflammatory reaction in KD patients with pyuria. We also found elevation of some useful parameters like ${\beta}_2$-microglobulin that indicate renal involvement of KD through the urine test. Careful management and follow up will need for KD patients with pyuria and it is necessary in the future to study the specific parameters for renal involvement of KD.

• IMMUNE NETWORK
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• 제2권4호
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• pp.202-207
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• 2002

Background: Kawasaki disease is an acute febrile illness with systemic vasculitis which primarily affects children, We examined the production of leptin in plasma and gene expressions of CXC chemokines in peripheral blood mononuclear cells from patients with Kawasaki disease. Methods: Consecutive 39 samples from 13 patients according to the different clinical stages (acute, subacute, convalescent) of Kawasaki disease were collected. The plasma leptin levels according to clinical stages of Kawasaki disease were examined by ELISA and the expression of IP-10, Mig and IL-8 mRNAs in 39 samples (13 samples of each stage) from 13 cases were examined by RT-PCR. Results: There were not significant changes of plasma leptin levels according to the clinical stages of Kawasaki disease. The mean values of plasma leptin concentrations during each of the stages (n=13, p>0.05, pg/ml) were $335.8{\pm}549.0$ in acute, $358{\pm}347.6$ in subacute, and $443.6{\pm}645.9$ in convalescent stage. The mRNAs of IP-10, Mig, and IL-8 were expressed in 13/13 (100%), 2/13 (15%), 9/13 (69%) during acute stage, 13/13 (100%), 6/13 (46%), 13/13 (100%) during subacute stage, and 13/13 (100%), 4/13 (31%), 10/13 (77%) during the convalescent stage, respectively. In three patients, the production of leptin and expression of IP-10 mRNA were dramatically decreased according to the process of the clinical stages. In five patients with prominent cervical lymphadenopathy, the expression of IL-8 mRNA during the subacute stage was more elevated than the acute and convalescent stages. Conclusion: This data suggests that the production of leptin and the gene expressions of IP-10, Mig and IL-8 seem to have no significant correlation to the clinical stages of Kawasaki disease. However, expression patterns of IP-10, Mig and IL-8 mRNA may be related to the specific clinical manifestations, and the expression of IP-10 may also be correlated to leptin levels with pericardial involvement.

• 한국임상약학회지
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• 제27권3호
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• pp.127-135
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• 2017

Background: Kawasaki disease (KD) is an acute febrile, systemic vasculitis as a leading cause of acquired heart disease in children. Intravenous immunoglobulin G (IVIG) and aspirin are the standard initial therapy in the treatment of acute KD. The purpose of this study was to investigate drug utilization in children with KD, and to compare "IVIG + high-dose aspirin" and "IVIG + moderate-dose aspirin" in preventing cardiac complications. Methods: We analyzed pediatric patient sample data compiled by the Health Insurance Review & Assessment Service from 2010 to 2015. We identified patients with KD using the KCD-6 code of M30.3. We excluded patients in chronic phase or ${\geq}1$0 years. We also excluded patients who were diagnosed KD in November or December. Drug utilization pattern were assessed in acute KD patients and 30-day and 60-day cardiac complications were investigated between "IVIG + high-dose aspirin" group and "IVIG + moderate-dose aspirin" group. Results: In acute phase, IVIG was administered to 95.8% patients, and 57.1% patients were prescribed moderate-dose aspirin and 25% patients were with high-dose aspirin. Steroid use was rapidly increased from 4.0% in 2010 to 11.3% in 2015. Both 30-day and 60-day cardiac complications occurred less in "IVIG + high-dose aspirin" group compared to "IVIG + moderate-dose aspirin" group, but not statistically significant (0.9% vs 1.8%, p=0.252 for 30-day complication rate; 1.5% vs 2.7%. p=0.073 for 60-day complication rate). Conclusion: We were not able to demonstrate which aspirin therapy is superior for preventing cardiac complications in acute KD patients and further research is warranted.

• Clinical and Experimental Pediatrics
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• 제58권11호
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• pp.434-439
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• 2015

Purpose: Kawasaki disease involves acute febrile systemic vasculitis that can cause a variety of symptoms by affecting various organs. Here, we aimed to evaluate the prevalence, causes, and prognosis of sensorineural hearing loss (SNHL) occurring in children with Kawasaki disease. Methods: Patients who were diagnosed with Kawasaki disease and received inpatient treatment in the Pediatrics Department at one of three university hospitals in Daegu city from February 2012 to September 2012 were enrolled in the study. The clinical features, hematological results, echocardiography results, audiometry results, and aspirin and salicylic acid serum levels of the patients were evaluated. Results: Of the 59 children enrolled in the study, three showed mild bilateral SNHL on audiometry tests conducted after 48 hours of defervescence; these patients demonstrated normal patterns of recovery on follow-up tests 8 weeks later. Aspirin serum levels were significantly higher in the SNHL group after 48 hours of afebrile condition with high dose aspirin intake (P=0.034). However, no significant differences were found in other laboratory tests or for fever duration (P>0.05). Upon echocardiography, coronary artery abnormality was observed in 9 cases, but none of these patients showed hearing loss. Conclusion: The results indicate that SNHL in children with Kawasaki disease might occur during treatment of the acute phase; this SNHL usually involves mild bilateral hearing loss and recovers naturally. However, this study suggests that determination of the causes and clinical implications of hearing loss in Kawasaki disease requires long-term follow-up studies with more cases.

• Pediatric Infection and Vaccine
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• 제24권1호
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• pp.60-64
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• 2017

급성 음낭증은 갑작스런 음낭의 부종과 통증을 주소로 하는 음낭의 병적 상태를 말하는 것으로 가와사키의 드문 합병증으로 알려져 있다. 저자들은 급성 음낭증을 동반한 가와사키병으로 진단된 2개월 남자 환아를 경험하였으며, 환아는 정맥 면역글로불린 및 아스피린 투여 후 발열과 가와사키병의 임상 증상들이 호전되었고 2달째에 고환 초음파검사에서 급성 음낭증의 호전을 확인하고 심장 초음파 검사에서 관상동맥 합병증이 보이지 않아 치료를 종료하였다.

• Childhood Kidney Diseases
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• 제18권1호
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• pp.42-46
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• 2014

가와사끼병은 전신성 혈관염을 일으키는 질환중의 하나로 여러 장기들을 침범할 수 있다. 신장증세로는 농뇨, 혈뇨, 단백뇨, 간질성 신염, 급성 신부전증, 용혈성 요독 증후군, 신반흔 등이 있다. 가와사끼병의 신장침범에 대한 병리기전은 아직 알려져 있지 않지만, 자가면역질환으로 인한 것으로 사려된다. 가와사끼병이 요로감염 이 후에 발병한다는 몇몇 보고들이 있었다. 하지만, 이미 보고된 논문들에 포함된 많은 요로감염 환자들은 신장방광 초음파, DMSA 스캔이나 배뇨중 요도방광조영술 등을 모두 받은 경우는 없었다. 이에 저자들은 급성 신우신염이 재발한 후 불완전 가와사끼병이 발생한 고도의 방광요관역류가 있는 8개월 남아를 보고하는 바이다. 급성 신우신염은 가와사끼병의 초기 증세일 수 있다. 그런 경우, 환아가 가와사끼병으로 확진되더라도 요로감염 진료지침에 따라 요로기형에 대한 이미지 검사를 시행할 필요가 있다고 생각한다.

• The Korean Journal of Pain
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• 제20권2호
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• pp.255-257
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• 2007

Giant cell arteritis, which is also referred to as temporal arteritis, is defined as a systemic vasculitis in individuals over 50 years of age. Here, we report a case of giant cell arteritis combined with oculomotor nerve palsy. An 81-year old female patient experienced a headache for 10 days in her left temporoparietal area, that was characterized by a continuous dull ache and heaviness with intermittent shooting and lancinating pain. Her symptoms persisted in spite of receiving strong analgesics in another hospital. Upon physical examination, she was found to have marked tenderness over the left temporal area, especially along the path of the temporal artery as well as limitation of adduction, supraduction and infraduction of the left eyeball. At the time of admission, her erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were 52 mm/hr and 3.94 mg/dl. In addition her brain MRI revealed no specific findings. Giant cell arteritis was suspected based on the clinical symptoms and signs as well as the elevated ESR and CRP. Oral steroid therapy started was started with an initial dose of 40 mg of prednisolone per day that was gradually tapered to 5 mg a day for 2 weeks. Her headache subsided one day after the steroid therapy and oculomotor nerve palsy was markedly improved after 2 weeks of the therapy. After 2 months she had recovered completely from her symptoms.