급성 신손상을 동반한 가와사키 쇼크증후군 1예

최재홍 ( Jae Hong Choi ),김윤주 ( Yoon-joo Kim ),김영돈 ( Young Don Kim ),한경희 ( Kyoung Hee Han ) 대한소아감염학회 2017 Pediatric Infection and Vaccine Vol.24 No.2

가와사키병은 다른 원인이 없는 발열과 함께 피부점막에 특징적인 병변을 보이는, 소아에서는 비교적 흔한 질환으로 일반적으로 급성기에는 혈역학적으로 안정적이다. 저자들은 심한 저혈압과 함께 급성 신손상이 있는 상태에서 내원한 8세 남아가 치료 중에 가와사키병으로 진단된 증례를 경험하였다. 최근에는 가와사키병 초기에 불안정한 혈역학적 상태를 가와사키병 쇼크증후군이라는 개념으로 정의하고 있다. 환아는 면역글로불린 치료 후 임상적으로 회복되었으나 관상동맥 확장 합병증이 확인되어 아스피린을 복용하고 있으며, 가와사키병의 합병증으로서는 비교적 드문 고혈압이 확인되어 베타 차단제를 사용하고 있다. 본 증례를 통해 가와사키병 쇼크증후군의 임상 양상을 알고, 혈관염이라는 가와사키병의 특성을 고려해볼 때 혈압 모니터링의 중요성을 깨닫게 되었다. Kawasaki disease (KD) is an acute febrile mucocutaneous lymph node syndrome that commonly presents with stable hemodynamic status during the acute phase. An 8-year-old boy initially presented with severe hypotension and acute kidney injury. He was placed in the intensive care unit and was diagnosed with KD. Observed clinical features were defined as KD shock syndrome. His coronary artery was dilated during the subacute phase. Furthermore, he was given anti-hypertensive medications, owing to hypertension as an unusual complication of KD. We knew the importance of monitoring for blood pressure considering vasculitis as an aspect of the main pathogenesis of KD.

가와사끼병에서 저나트륨혈증의 관련인자에 대한 임상적 고찰

김수연,최준석,허재경,김현정 대한소아청소년과학회 2009 Clinical and Experimental Pediatrics (CEP) Vol.52 No.3

Purpose : Clinical and laboratory findings predict a severe outcome for mucocutaneous lymph node syndrome. This study aimed to define the clinical characteristics of Kawasaki disease (KD) patients with hyponatremia and to determine the factors associated with its development. Methods : Retrospective studies were performed on 114 KD patients who received an initial high-dose intravenous immunoglobulin (IVIG, single 2 g/kg/dose) within 10 days of fever onset from January 2006 to February 2008. These patients were divided into 2 groups. Group 1 consisted of 30 (26.3%) patients with hyponatremia, and group 2 consisted of 84 (73.6%) patients without hyponatremia. Clinical manifestations, laboratory results, and echocardiographic findings were compared between the groups. Results : Group 1 patients were more likely to have a coronary artery lesion (53.3% versus 20.2%, P=0.005) and suffered from diarrhea (41.3% versus 14.1%, P=0.007). There was a higher incidence of cardiovascular involvement in group 1 patients, including coronary dilatation (46.6%), valvular regurgitation (13.3%), pericardial effusion (6.7%) and medium-sized aneurysm (6.7%). There were no coronary aneurysms in group 2 patients. Serum C-reactive protein (CRP) was significantly higher in patients with hyponatremia (12.2±7.79 mg/dL versus 7.3±4.7 mg/dL, P=0.003) and IVIG-resistant patients were more common in group 1 (13.3% versus 3.6%). Conclusion : These results indicate that hyponatremia in KD occurs in patients exhibiting severe inflammation and was significantly associated with the development of coronary disease. Further studies will be necessary to confirm the pathogenic mechanisms of hyponatremia in KD patients. 목 적 : 가와사끼병의 급성기에 심장 합병증을 미리 예측하고자 하는 연구가 꾸준히 계속되고 있으나 아직까지 확실하게 인정되고 있는 지표는 없는 실정이다. 이에 저자들은 저나트륨혈증이 가와사끼병 급성기에 나타나는 빈도와 다른 임상적 요인 및 관상동맥 합병증과의 연관성을 알아보고자 하였다. 방 법 : 2006년 1월부터 2008년 2월까지 만 2년간 을지대학병원 소아과에 입원하여 가와사끼병으로 치료받았던 114명의 환자를 대상으로 후향적으로 조사하였다. 나이와 성별, 발열부터 면역글로불린 투여시까지의 기간, 치료부터 해열까지의 기간, 입원 당시의 백혈구수, 혈소판수,알부민, ESR, CRP, AST, ALT치와 무균성 농뇨 및 심초음파 결과 등을 저나트륨혈증군(1군) (n=30)과 정상 나트륨군(2군) (n=84)으로 나누어 비교 분석하였다. 통계 처리는 t-test와 교차분석, logistic 회귀 분석을 사용하였다. 결 과 : 1) 1군의 환자들에게서 설사 증상이 나타났던 경우가 40%로 2군의 환자들보다 유의하게 많았으며 농뇨나 혈뇨의 빈도는 두 군간에 유의한 차이가 없었다. 2) 1군 환아들에서의 CRP 수치가 평균 12.2 mg/dL로 2군 환아들에서의 7.3 mg/dL에 비하여 유의하게 높았다. 3) 1군에서 13.3%의 환아에서 1회의 IVIG 치료 후에도 발열이 지속되어 IVIG의 재투여가 필요하였으며 2군의 환아들에 비해 3배 가량 많았다. 4) 1군 중 53.3%에서 관상 동맥의 병변이 있었으며 2군의 20.2%에 비해 유의하게 많았으며 1군 중 2명(6.7%)에서 중등도 크기의 관상동맥류가 있었다. 결 론 : 가와사끼병에서 입원 당시에 저나트륨혈증이 있거나 면역글로불린 투여 이후에도 저나트륨혈증이 지속될 때 관상 동맥의 합병증이 더 많이 발생할 가능성을 염두에 두고 치료에 임하는 것이 좋을 것으로 사료되는 바이다.

경부 림프절염로 발현된 가와사키병과 급성 편측 경부 림프절염의 비교

이훈상 ( Hoon Sang Lee ),김지용 ( Ji Yong Kim ),송보경 ( Bo Kyung Song ),김용우 ( Yong-woo Kim ),박수은 ( Su Eun Park ) 대한소아감염학회 2016 Pediatric Infection and Vaccine Vol.23 No.3

목적: 본 연구는 경부 림프절 비대로 시작된 가와사키병과 급성 편측 경부 림프절염 환자에서 임상 양 상, 혈액검사 소견, 경부 컴퓨터단층촬영 소견에 차이가 있는지 확인하고자 하였다. 방법: 2010년 1월부터 2014년 12월까지 발열과 경부 림프절 비대로 부산대학교 어린이병원에 내원하여 경부 컴퓨터단층촬영을 시행한 환자 총 372명 중에 가와사키병으로 진단된 28명의 환자군과 편측 경부 림프절염 환자군 28명을 후향적으로 비교하였다. 결과: 경부 림프절염으로 발현된 가와사키병과 급성 편측 경부 림프절염에서 발열 기간, 항생제 사용, 경부 림프절 크기에는 차이가 없었다. 혈액검사 소견에서 CLKD 환자에서 AUCL 환자와 비교해서 총 백혈구 수, 호중구 수, 적혈구 침강 속도, C-반응단백 수치가 더 높은 것으로 나타났다(P<0.05). 경부 컴퓨터단층촬영 소견에서 후인두부 부종 소견은 두 군 간에 차이가 없었다(P =0.686). 결론: CLKD와 AUCL을 조기에 구별할 수 있는 특징적인 소견은 없는 것으로 보이기 때문에 발열과 경 부 림프절 비대가 있는 환자에서 항생제 치료에도 증상 개선이 없다면 가와사키병을 고려해야겠다. Purpose: This study aimed to identify the differential clinical, laboratory, and imaging characteristics between patients with cervical lymphadenitis as first presentation of Kawasaki disease (CLKD) and those with acute unilateral cervical lymphadenitis (AUCL). Methods: We surveyed 372 patients who visited Pusan National University Children`s Hospital because of fever and cervical lymph node enlargement, and underwent neck computed tomography (CT) from January 2010 to December 2014. We compared 28 confirmed cases of Kawasaki disease and 28 cases of AUCL based on a retrospective review of the medical records of the patients. Results: Patients with CLKD and AUCL showed no differential clinical characteristics in terms of the duration of fever, antibiotic use, or the size of lymph nodes. Patients with CLKD had higher white blood cell count, absolute neutrophil count, erythrocyte sedimentation rate, and C-reactive protein levels (P <0.05) than those of patients with AUCL. The presence of retropharyngeal edema on neck CT was similar between the groups (64% vs. 33%, P =0.686). Conclusions: CLKD and AUCL showed no differentiating clinical and radiological characteristics; hence, Kawasaki disease should be the presumptive diagnosis in patients with fever and cervical lymph node enlargements who fail to respond to antibiotic treatment.

발열과 림프절 종대를 보인 환자에서 화농성 경부 림프절염과 가와사키병의 감별 진단

장호민 ( Homin Jang ),하은교 ( Eun Gyo Ha ),김희진 ( Hee Jin Kim ),이택진 ( Taek-jin Lee ) 대한소아감염학회 2016 Pediatric Infection and Vaccine Vol.23 No.3

목적: 경부 림프절 종대를 먼저 보인 가와사키병(node-first presented Kawasaki disease [NFKD]) 에 대한 임상 연구를 통해 세균 경부 림프절염(bacterial cervical lymphadenitis [BCL]), 전형적인 가와사키병(Kawasaki disease [KD])과 구별되는 특징을 찾고자 하였다. 방법: 2007년부터 2015년까지 BCL, NFKD, 전형적인 KD로 입원한 환자들의 의무기록을 검토하여 인구학적 특성, 임상 양상, 혈액검사 및 영상의학적 검사 결과를 후향적으로 비교 분석하였다. 결과: 22명의 BCL, 37명의 NFKD, 132명의 전형적인 KD 환자가 연구에 포함되었다. BCL과 NFKD의 비교에서 BCL군의 입원 기간이 더 길었고 NFKD군은 양측, 다발형 림프절 비대를 보이면서 혈소판 수 가 적고 호중구 백분율과 CRP 수치가 높았다. NFKD와 전형적인 KD의 비교에서 NFKD군의 나이가 더 많았고 백혈구수, 호중구 백분율, 호중구 수, CRP 수치가 높고 혈소판 수와 ALT 수치가 낮았다. 결론: 발열과 경부 림프절 종대가 있는 환자에서 호중구 백분율과 CRP 수치가 높고 혈소판 수치가 높지 않으면서 양측, 다발형 경부 림프절 비대를 보인다면, KD의 진단 및 치료가 지연되지 않도록 NFKD 진단을 신속하게 고려하여야 한다. Purpose: This study identified the characteristics differentiating node-first presentation of Kawasaki disease (NFKD) from bacterial cervical lymphadenitis (BCL) and typical Kawasaki disease (KD). Methods: From July 2007 to June 2015, the medical records of patients with BCL, NFKD, and typical KD were retrospectively reviewed. We analyzed and compared the demographic, clinical, laboratory, and imaging characteristics of the cohorts. Results: Twenty-two patients with BCL, 37 with NFKD, and 132 with typical KD were included in this study. Patients with BCL had longer durations of hospitalization than patients with NFKD. Bilateral and multiple enlarged cervical lymph nodes were associated more with NFKD than BCL. Compared with BCL patients, NFKD patients had lower platelet counts, higher percentages of neutrophils, and higher C-reactive protein (CRP) levels. NFKD patients were older and presented with higher white blood cell counts, percentages of neutrophils, absolute neutrophil counts, and CRP levels as well as lower platelet counts and alanine aminotransferase levels than typical KD patients. Conclusions: In febrile patients with cervical lymphadenopathy, the combination of bilateral and multiple enlarged nodes, low platelet count, high percentage of neutrophils, and high CRP levels should prompt consideration of NFKD for prevention of delayed diagnosis of KD.

Elevated Serum Levels of IL-21 in Kawasaki Disease

배윤정,김미현,이해용,어영,남궁미경,차병호,전진경 대한천식알레르기학회 2012 Allergy, Asthma & Immunology Research Vol.4 No.6

Purpose The serum level of immunoglobulin (Ig)E has been reported to be elevated in patients with Kawasaki disease (KD). We investigated whether interleukin (IL)-21, rather than IL-4, could be related to elevated serum levels of IgE in KD. Methods Sera from 48 patients with KD and 12 controls with high fever were collected to determine the level of IgE using an immunoassay system and the levels of IL-4 and IL-21 were determined using enzyme-linked immunosorbent assay kits. Results The median IL-21 level of KD patients was significantly elevated, at 499.5 pg/mL (range: <62.5-1,544 pg/mL), whereas that of controls was <62.5 pg/mL (<62.5-825 pg/mL; P<0.001). The median IL-4 level of KD patients was not elevated (4.0 pg/mL; 2.1-7.6 pg/mL). The median level of total IgE in KD patients was 58.0 IU/mL (5-1,109 IU/mL). No statistically significant correlation was found between IL-21 and total IgE levels (Spearman's R=0.2; P=0.19). Conclusions Patients with KD have elevated levels of IL-21 in the serum. IL-21 may play a role in the pathogenesis of KD. Keywords: Interleukin-21, immunoglobulin E, mucocutaneous lymph node syndrome.

경부임파선 비대가 첫 증상인 가와사키병과 세균성 경부임파선염의 감별진단에서N-terminal pro-brain natriuretic peptide의 역할

황일웅,이동욱,김재우,박세훈,이정원,문형준,최재형,이현정,정윤현,김현수,전덕호 대한응급의학회 2018 대한응급의학회지 Vol.29 No.1

Purpose: Kawasaki disease (KD) is an acute, self-limited, febrile disease. For cases of KD in which the first symptom is cervical lymphadenopathy (node-first presentations of KD, NFKD), it is frequently misdiagnosed as bacterial cervical lymphadenitis (BCL). Therefore, we evaluated the usefulness of N-terminal pro-brain natriuretic peptide (NT-proBNP) to differentiate between NFKD and BCL. Methods: This is a retrospective, observational study. Patients were divided into three groups, KD as 1st diagnosis, NFKD, and BCL. The laboratory and demographic data, intravenous immunoglobulin (IVIG) administration time and total febrile duration, length of hospital stay, and number of coronary artery complications were then compared for each group. Results: A total of 451 patients were diagnosed as KD and 45 patients as BCL. Of the 451 KD patients, 417 (92.5%) were KD as 1st diagnosis, and 34 (7.5%) were NFKD. White blood cell count, absolute neutrophil count, C-reactive protein, erythrocyte sedimentation rate, and NT-proBNP differed significantly between NFKD and BCL. Variables that differed significantly were analyzed using a receiver operating characteristic curve, which revealed that NT-proBNP had the largest area under curve (0.944). Additionally, IVIG administration time, total febrile duration and length of hospital stay differed between KD as 1st diagnosis and NFKD. Conclusion: It is difficult to differentiate NFKD from BCL, so proper treatment and length of hospital stay were delayed. NT-proBNP is very useful for differentiating NFKD and BCL. Therefore, in cases of BCL with a long febrile period without reacting general treatments, the NT-proBNP test can be considered.

가와사끼병의 급성기 치료에서 고용량 아스피린과 고용량 이부프로펜 사용 효과 비교 분석

금승운,오연균,김종덕,유승택 대한소아청소년과학회 2009 Clinical and Experimental Pediatrics (CEP) Vol.52 No.8

Purpose : We evaluated the effectiveness of treatment and cardiac complications of replacing a high dose of aspirin with a high dose of ibuprofen for children in acute phase of Kawasaki disease. We also analyzed the possibility of replacing a high dose of aspirin with a high dose of ibuprofen to prevent complications such as Reye’s syndrome caused by aspirin. Methods : One hundred eight children with Kawasaki disease were admitted in the pediatrics department from January 1, 2004 to December 31, 2008. Echocardiography and laboratory tests were performed during diagnosis, and the children were followed-up at 6-8 weeks after the diagnosis. We retrospectively analyzed their characteristics and clinical results. Results : The children were assigned to receive either a high dose of aspirin with intravenous immunoglobulin (IVIG) (aspirin group) or a high dose of ibuprofen with IVIG (ibuprofen group). A total of 55 and 53 children were included in the aspirin and ibuprofen groups, respectively. The mean defervescence period was 6.5±2.1 days in the aspirin group, and 6.9±1.9 days in the ibuprofen group (P=0.309). The number of failed treatments, during and after treatment, was 8 in the aspirin group and 10 in the ibuprofen group (P=0.547). There were 11 initial cardiac complications in the aspirin group, and 14 in the ibuprofen group, but children who showed improvement at follow-up was 7 and 13 in the aspirin and ibuprofen groups, respectively (P= 0.133). Laboratory findings were also improved in both groups. Conclusion : We can be considered whether we will replace high dose of aspirin by high dose of ibuprofen in acute phase of Kawasaki disease. Therefore, we can prevent the severe complications of aspirin use, such as Reye’s syndrome. 목 적 : 합병증으로 관상동맥 질환을 일으킬 수 있는 가와사끼병의 급성기 치료에 있어 정맥용 면역 글로불린과 함께 투여하는 고용량 아스피린을 고용량 이부프로펜으로 대체하여 투약시 효과와 가와사끼병의 합병증 발생 빈도를 비교 및 분석함으로서 Reye 증후군과 같은 합병증을 유발할 수 있는 아스피린을 이보다 부작용이 적은 이부프로펜과 같은 다른 비스테로이드 항염제로의 대체 가능성을 분석하였다. 방 법 : 2004년 1월 1일부터 2008년 12월 31일까지 익산 원광대학병원 소아과에 108명의 소아가 가와사끼병으로 진단받았다. 심장 초음파 검사와 혈액 검사를 진단 시점과 진단 약 6-8주 후 시행하여 비교하였다. 본 저자들은 대상 환아들의 특성과 검사소견을 기초로 후향적으로 비교분석하였다. 결 과 : 총 108명의 환아들을 정맥용 면역글로불린 투여 및 고용량 아스피린을 사용했던 군(아스피린 군)과 정맥용 면역글로불린 투여 및 고용량 이부프로펜을 사용했던 군(이부프로펜 군)으로 분류하였다. 아스피린 군은 55명이었고 이부프로펜 군은 53명이었다. 평균 해열 기간은 발열 후 아스피린 군 6.5±2.1일, 이부프로펜 군 6.9±1.9일이었다(P=0.309). 치료 중이나 후 치료실패 환아는 아스피린 군 8명, 이부프로펜 군 10명이었다(P=0.547). 재발한 환아는 아스피린 군 2명, 이부프로펜 군 1명이었다(P= 0.580). 혈액 검사에서도 두 군에서 호전된 소견을 보였다. 결 론 : 본 저자들은 가와사끼병 급성기에 있어 정맥용 면역 글로불린과 더불어 사용하고 있는 고용량 아스피린 투약에 있어 이부프로펜과 같은 비스테로이드 항염제를 대체하여 투약할 수 있을 것으로 생각되며 이에 Reye 증후군과 같은 아스피린의 치명적인 합병증을 예방할 수 있을 것이다.

6개월 이하 영아에서의 가와사끼병의 역학적 연구

박용원,한지환,박인숙,김창휘,차성호,마재숙,이준성,권태찬,이상범,김철호,이흥재,윤용수 대한소아청소년과학회 2008 Clinical and Experimental Pediatrics (CEP) Vol.51 No.12

Purpose:The aim of this study was to investigate the epidemiologic status of Kawasaki disease (KD) in infants ≤6 months of age. Methods:For the epidemiologic study of KD in Korea, data from 22,674 KD patients were collected from 1997 to 2005 on a 3-year basis by a retrospective survey. From this survey, data of 1,739 KD patients ≤6 months of age were analyzed and compared with those of KD patients >6 months of age. Results:A total of 1,739 patients ≤6 months of age, including 1,088 males and 651 females, represented 7.7% of total KD patients. These subjects included 22 patients aged <1 month, 171 patients aged 1-2 months, 304 patients aged 2-3 months, 407 patients aged 3-4 months, 372 patients aged 4-5 months, and 463 patients aged 5-6 months. Their mean age of onset was 4.3 months, and the male-to-female ratio was 1.67:1. Incidences of coronary arterial (CA) abnormalities (21.0% versus 18.7%) and CA aneurysms (4.7% versus 3.1%) detected by echocardiography showed differences between patients with KD younger and older than 6 months, respectively. Conclusion:Comparison of data from KD patients >6 months old with data from 1,739 KD patients ≤6 months old showed significantly higher incidences of CA abnormalities and CA aneurysms in the younger patients. (Korean J Pediatr 2008;51: 1320-1323) Purpose:The aim of this study was to investigate the epidemiologic status of Kawasaki disease (KD) in infants ≤6 months of age. Methods:For the epidemiologic study of KD in Korea, data from 22,674 KD patients were collected from 1997 to 2005 on a 3-year basis by a retrospective survey. From this survey, data of 1,739 KD patients ≤6 months of age were analyzed and compared with those of KD patients >6 months of age. Results:A total of 1,739 patients ≤6 months of age, including 1,088 males and 651 females, represented 7.7% of total KD patients. These subjects included 22 patients aged <1 month, 171 patients aged 1-2 months, 304 patients aged 2-3 months, 407 patients aged 3-4 months, 372 patients aged 4-5 months, and 463 patients aged 5-6 months. Their mean age of onset was 4.3 months, and the male-to-female ratio was 1.67:1. Incidences of coronary arterial (CA) abnormalities (21.0% versus 18.7%) and CA aneurysms (4.7% versus 3.1%) detected by echocardiography showed differences between patients with KD younger and older than 6 months, respectively. Conclusion:Comparison of data from KD patients >6 months old with data from 1,739 KD patients ≤6 months old showed significantly higher incidences of CA abnormalities and CA aneurysms in the younger patients. (Korean J Pediatr 2008;51: 1320-1323)

가와사키병에서의 TNF-alpha 유전자의 다형성 및 관상동맥 합병증과의 연관성

김세화,윤장원,이영혁,천은정 대한소아청소년과학회 2009 Clinical and Experimental Pediatrics (CEP) Vol.52 No.4

Purpose : The purpose of this study was to investigate the polymorphisms of the TNF-alpha promotor gene, its susceptibility to Kawasaki disease (KD) and to assess whether the TNF-alpha promotor gene polymorphism was related the risk of coronary artery lesions (CALs). Methods : From January 2003 to January 2007, 51 children (30 boys and 21 girls) with KD and 48 children forming an age-matched control group were studied. DNA from the peripheral blood of all the children was sampled, and the DNA polymorphisms of the 5' flanking regions of the TNF-alpha promoter gene at position -308 [guanine (G) to adenine (A)] were determined by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Then, the relationship between KD and the TNF-alpha promotor gene polymorphisms was evaluated. Results : The A allele frequency of the -308 site of the TNF-alpha promotor gene was 17.6% (9/51) for children with KD and 6.8% (3/48) for the control group children, but this result was not statistically significant. Twenty-four patients experienced CALs within 60 days after the onset of symptoms. KD children with TNF-alpha -308 A allele had lower frequencies of CALs (12.5% versus 22.2%, P>0.05). Conclusion : The DNA polymorphism of the -308 site TNF-alpha gene was not associated with susceptibility to KD and a risk of CALs. Multicenter, large-scale randomized controlled trials are needed for further study. 목 적 : 저자들은 가와사키병 환아에서 TNF-alpha 유전자의 다형성을 조사함으로써 가와사키병과 유전자 다형성의 관련 여부를 알아보고, 또한 관상동맥 병변의 발생과 연관이 있는지를 살펴보려 하였다. 방 법 : 2003년 1월부터 2007년 1월까지 가와사키병 환아 51명과 대조군 48명을 대상으로 TNF-alpha 촉진자의 단일 유전자 다형성을 살펴보았으며, 가와사키병 환아 중 관상동맥 병변이 있는 24명(관상동맥병변군)과 관상동맥 이상이 없었던 27명(정상관상동맥군)에서의 유전자 다형성을 또한 비교하였다. 말초 혈액에서 DNA를 추출하여 TNF-alpha 유전자 -308 부위의 촉진자에 위치한 2개의 단일 염기 서열 G/A에 대한 대립 유전자의 다형성을 Polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) 방법으로 분석하였다. 결 과 : 가와사키병 환아군에서의 -308번 TNF-alpha 유전자의 다형성은 51명 중 9명으로 17.6%였고, 정상 대조군에서는 48명 중 3명으로 6.8%로 가와사키병 환아군에서 높았으나 통계학적으로 유의성은 없었다. 가와사키병 환아 중 관상동맥병병군 24명 중 3명인 12.5%에서 유전자 다형성이 있었고 정상관상동맥군은 27명 중 6명인 22.2%로 정상관상동맥군에서 더 높은 빈도 이었으나 통계학적으로 유의하지 않았다. 결 론 : 본 연구에서는 가와사키병 환아의 TNF-alpha의 다형성과 가와사키병의 발병과의 관련성이 통계학적으로 유의한 수준은 아니지만 가와사키병 환아에서 G/A 빈도수가 17.6%으로 대조군에서 6.8% 보다 다소 높게 나타난 결과를 얻었고 앞으로 많은 수의 환아를 대상으로 한다면 유의한 차이가 있을 것으로 생각되므로 향후 대규모의 지속적인 연구가 필요할 것이다.

A case of adolescent Kawasaki disease with Epstein-Barr virus-associated infectious mononucleosis complicated by splenic infarction

최병삼,권보상,김기범,노정일,최정연,윤용수,배은정,전윤경,천정은 대한소아청소년과학회 2009 Clinical and Experimental Pediatrics (CEP) Vol.52 No.9

Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects children. There are few reports that describe the Epstein-Barr virus (EBV) as the possible infectious agent of KD. Here, we describe a case of KD in a 15- year-old boy complicated with giant coronary artery aneurysms, pericardial effusion, and splenic infarction. The clinical course of KD was refractory to intravenous gamma globulin and aspirin. Our patient also showed typical findings of concomitant EBV-associated infectious mononucleosis, such as hepatosplenomegaly and generalized lymphadenopathy, with EBV-positive atypical lymphoid hyperplasia. He improved dramatically after receiving intravenous methylprednisolone followed by oral prednisolone. Ultimately, the coronary artery aneurysms remained as the only sequelae. We report a rare case of adolescent KD with EBV-associated infectious mononucleosis and splenic infarction.