제 1형 신경섬유종증에 동반된 흉강 내악성 말초신경초종 1례

김영미(Young Mi Kim),전소은(So Eun Jeon),이병기(Byung Ki Lee),이윤진(Yoon Jin Lee),남상욱(Sang Ook Nam),임영탁(Young Tak Lim) 대한소아신경학회 2011 대한소아신경학회지 Vol.19 No.2

Malignant peripheral nerve sheath tumor (MPNST) is a rare neoplasm and the main cause of the mortality in neurofibromatosis type 1 (NF 1). MPNSTs have been found mostly in the head and neck and the upper or lower extremities with intrathoracic MPNSTs being uncommon. PET has been a useful diagnostic modality of MPNSTs in NF 1. We present a 17-year-old girl patient with NF 1. She was admitted with chronic cough and shortness of breath caused by a huge mediastinal mass. An 18FDG-PET study revealed intense uptake at the mediastinal mass. She underwent surgery to lessen respiratory symptoms, and the mass was histologically diagnosed as an intrathoracic MPNST. 악성 말초신경초종은 드문 악성 종양이나 신경섬유종증 제 1형 환자에서 호발하며, 말초신경이나 심부에 발생한 신경섬유종에서 발생할 수 있다. 18FDG-PET는 신경섬유종증 제 1형 환자에서 발생한 악성말초신경초종을 진단하는데 유용하다. 저자들은 신경섬유종증 제 1형을 가진 17세 여아에서 18FDG-PET 등의 검사를 사용하여 흉강 내에 발생한 악성말초신경초종을 진단하고 치료한 경험을 보고하는 바이다.

소아 특발혈소판감소 자색반병 환자에서 항핵항체 검사의 양성률 및 임상적 의의

전소은,박성식,임영탁,Jun, So Eun,Park, Seong Sik,Lim, Young Tak 대한소아청소년과학회 2008 Clinical and Experimental Pediatrics (CEP) Vol.51 No.11

목 적: SLE에서는 진단 당시 및 경과 중에 약 15-20%에서 혈소판 감소가 발생하며, 5-16%에서는 혈소판 감소증이 첫 임상증상으로 발생할 수 있으므로 소아 ITP와 SLE와의 연관성이 강력히 제기되고 있다. 한편 항핵항체는 여러 자가면역질환의 선별검사로 주로 이용되고 있는데, 특히 자가면역질환의 임상증상이 발현되기 수개월 혹은 수년 전에 발현될 수 있다. 따라서 저자들은 소아 ITP로 진단된 환자들에서 항핵항체 검사의 양성률 및 임상적 의의를 조사하고자 본 연구를 시작하였다. 방 법: 2003년 1월부터 2006년 12월까지 부산대학교병원 소아청소년과에서 급성 ITP로 진단 받고, 진단 당시에 항핵항체 검사를 시행하였던 77예의 환아를 대상으로 하였다. 항핵항체 검사는 정량 및 정성 검사를 시행하였고, 1:40 이상을 양성으로 판정하였다. 항핵항체 검사 상 양성인 환자들은 1년 이상 역가를 주기적으로 추적관찰 하였다. 결 과: 1) 진단 당시 항핵항체 검사를 시행한 77예의 ITP 환자 중 남아는 38예(49.4%), 여아는 39예(50.5%)였으며, 평균 연령은 4.5세였고, 16예(20.8%)에서 항핵항체 검사 상 양성을 보였다. 2) 항핵항체 양성인 군에서 항핵항체의 정량 역가의 분포는 1:80-1:1,280으로 1:640이 16명 중 5명(30.7%)으로 가장 많았고, 항핵항체의 형은 homogeneous 형이 16명 중 6명(37.5%)으로 가장 많았다. 3) 항핵항체가 양성인 군의 평균 연령은 9.3세로 음성인 군의 3.3세에 비해 진단 당시 평균 연령이 의미 있게 높았다(P<0.05). 4) 항핵항체가 음성인 군의 남녀비는 비슷하였던 반면 항핵항체가 양성인 군은 여아가 81.3%로 현저히 높았다(P<0.05). 5) 진단 당시 혈소판 정중값은 항핵항체 양성군에서는 $18,400/{\mu}L$, 음성군에서는 $21,800/{\mu}L$으로 두 군 사이에 의미 있는 차이가 없었다. 6) 진단 당시 항핵항체의 역가 및 형은 만성 ITP 및 SLE로의 진행과 의의있는 관련이 없었다. 7) 급성 ITP 진단 당시 항핵항체 양성이었던 환자 중 1명이 추적관찰 41개월 후 SLE로 최종 진단되었다. 결 론: 소아 급성 ITP 환자에서 ANA 양성인 경우가 비교적 흔한 것으로 나타났으며, 만성 ITP와 SLE로의 진행에 대한 연관성은 더 많은 환자를 대상으로 한 연구를 통하여 밝혀져야 할 것이다. Purpose : An association between idiopathic thrombocytopenic purpura (ITP) and systemic lupus erythematosus (SLE) has been recognized for decades because thrombocytopenia is the first manifestation in some patients with SLE. However, the risk of later development of SLE in childhood ITP is currently unknown. We retrospectively evaluated the incidence and clinical significance of the positive antinuclear antibody (ANA) in children with acute ITP. Methods : This study was retrospectively performed to review the clinical and laboratory characteristics in 77 children diagnosed to have acute ITP and admitted to the Pusan National University Hospital between January 2003 and December 2006. Patients tested positive for ANA were regularly followed-up for at least 12 months for symptoms indicative of SLE. Results : Seventy-seven children were included in the study; 38 males (49.4%) and 39 females (50.5%), the mean age was 4.5 years. Sixteen (20.8%) ITP patients had a positive ANA, with a median titer of 1:320. The mean age of the patients with positive ANA was 9.3 years, which is much older than 3.3 years for patients with negative ANA (P<0.05). The positive ANA group was predominantly female (81.3%) compared to the negative ANA group (P<0.05). There was no statistically significant difference in mean platelet counts between both groups. No statistically significant difference was found in ANA positivity and progression to chronic ITP or SLE. After the median follow-up of 32 months, SLE was diagnosed only in one ITP patient with positive ANA. Conclusion : Our data demonstrated that ANA positivity is often found in children with acute ITP. Large-scale studies should be considered to determine the significance of ANA positivity in childhood ITP for the later development of SLE.

Burkholderia cepacia 패혈증 2례

박성식,안성연,박수은,임영탁,장철훈,Park, Seong Shik,Ahn, Sung Ryon,Park, Su Eun,Lim, Young Tak,Chang, Chul Hun 대한소아감염학회 2001 Pediatric Infection and Vaccine Vol.8 No.2

저자들은 급성 림프구성 백혈병으로 진단받고 항암 약물 치료 중인 10세 남아와 선천성 수신증으로 진단받고 도뇨관 유치 후 배뇨성 방광 요도 조영술을 촬영한 신생아에서 발생된 Burkholderia cepacia 패혈증을 혈액 배양 검사로 진단하고 이에 감수성이 있는 항생제 투여 후 임상적으로 호전을 보였기에 문헌고찰과 함께 보고하는 바이다. Burkholderia cepacia, a widespread gram-negative environmental bacillus associated with nosocomial infection, is considered to be of relatively low virulence and rarely to cause invasive disease in immunocompromised patients. Nosocomial infections resulting from the use of contaminted medication, antiseptics and instruments have also been reported in otherwise healthy hosts. We experienced two cases of B. cepacia sepsis in 10 year-old male who was medicated with the anticancer drugs for the treatment of acute lymphoblastic leukemia(ALL) and in 15 day-old newborn who was examined with voiding vesicourethrography(VCUG) for the evaluation of congenital hydronephrosis. The organism isolated from serial blood culture in ALL patient and from serial blood culture and urine culture in newborn examined with VCUG. The former ALL patient improved after antibacterial medication of imipenem and the latter newborn improved after treatment with imipenem and trimethoprim-sulfamethoxazole.

13세 여아에서 발생한 총담관낭 I형에서 기원한 담관암 1례

안성연,남상욱,박재홍,임영탁,이준우,이창훈,Ahn, Sung-Ryon,Nam, Sang-Ook,Park, Jae-Hong,Lim, Young-Tak,Lee, Jun-Woo,Lee, Chang-Hun 대한소아소화기영양학회 2002 Pediatric gastroenterology, hepatology & nutrition Vol.5 No.1

저자들은 갑작스럽게 발생한 우상복부 동통을 주소로 내원한 13세 여아에서 간에 다발성으로 전이가 된 I형 총담관낭으로부터 발생한 담관암 1례를 경험하였기에 보고하는 바이다. Choledochal cyst is a rare developmental malformation of the biliary tree and has serious problem of transformation to malignancy. The development of cholangiocarcinoma related to choledochal cyst increases as the age of patient increases and is more common in Orientals with female predominance of 2.5 times. Prevalence rate of cholangiocarcinoma is various from 2.5 to 15.6% in adult with choledochal cyst, but very low in children. We experienced a case of cholangiocarcinoma with multiple liver metastases arising from type I choledochal cyst in a 13-year-old girl who complained of sudden onset of right upper quadrant abdominal pain.

최근 5년간(1996-2000) 부산지역 소아 백혈병 환자에 대한 역학적 연구

문재훈,이순용,신종범,박재선,이영호,임영탁,박수은,Moon, Jae Hoon,Lee, Soon Yong,Sinn, Jong Beom,Park, Jae Sun,Lee, Young Ho,Lim, Young Tak,Park, Su Eun 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.10

목 적 : 소아 백혈병은 소아 악성종양 중 빈도가 가장 높으며 타 질환에 비하면 아직도 사망률이 높기 때문에 발생률에 대한 기초자료는 대단히 중요하다. 저자들은 1996년부터 2000년까지 5년 동안 부산지역에서 발생한 소아 백혈병 환자들에 대한 역학적 연구를 시도하였다. 방 법 : 해당기간 동안 부산시내 4개 대학병원과 11개 종합병원의 소아 입원 환자들 중에서 백혈병으로 최초 진단된 133명(남아 87명, 여아 46명)에 대하여 역학적인 조사를 하여 시행하였다. 결 과 : 1) 1996-2000년의 5년 동안 부산지역에서 발생한 소아 백혈병 환자수는 총 133명이며 연간 환자수는 20-33명의 범위였고 연평균 26.6명(남아 17.4명, 여아 9.2명)이었다. 남녀 성비를 보면 1.89 : 1로 남아가 훨씬 많았다. 2) 이 기간 동안 15세 이하의 부산시 소아인구 100,000명당 연령 및 성별 정정 연간 발생률을 보면 2.37-4.53의 범위로 평균 3.29이었으며 남아의 경우는 2.47-5.29의 범위로 평균 4.05이었고 여아는 0.76-3.36의 범위로 평균 2.43이었다. 3) 연령 특정 연간 발생률은 0-4세군이 3.78, 5-9세군이 3.08, 10-14세군이 3.08로 0-4세군에서 가장 높았다. 4) 소아 백혈병의 병형별 분포는 ALL이 71.4%, AML이 23.3%, CML이 4.5%이었고, 병형별 남녀 성비를 보면, ALL에서는 1.97 : 1로 남아가 거의 두 배나 많았고 AML에서는 1.21 : 1로 남아가 약간 많았다. CML은 6명 전원이 남아였다. 5) 연령군에 따른 소아 백혈병의 병형 분포를 보면, 급성 림프구성 백혈병은 5-9세 군에서, 급성 골수성 백혈병은 0-4세군과 10-14세군에서 공히 가장 많았다. 6) 백혈병의 병형별 남녀 성비를 보면, 급성 림프구성 백혈병은 1.97 : 1, 급성 골수성 백혈병은 1.2 : 1이었으며 만성 골수성 백혈병은 전원 남아였다. 결 론: 1996-2000년의 5년 동안 부산지역에서 발생한 소아 백혈병 환자들의 연령 및 성별 조정 연간 발생률은 평균 3.29였다. 저자들의 이와 같은 성적은, 이와 관련된 문헌들과 비교해 볼 때, 부산지역 소아 백혈병의 발생률이 1981년부터 지난 20년간 완만하게 증가되었음 시사하였다. Purpose : For the control of childhood leukemia, of which the mortality is still high, the basic data for the incidence has a great importance. The authors analyzed the data from 133 new patients with childhood leukemia between 1996-2000 in Busan, Korea. Methods : The data were obtained from 133 new cases(87 males and 46 females from 0 to 15 years old) of childhood leukemia who were residents of Busan and who were admitted to the 4 university hospitals and 11 general hospitals from 1996 to 2000. Results : The total number of the new childhood leukemia patients was 133 between 1996-2000; the average annual number of new patients was 26.6. The age-and-sex adjusted annual incidence rate (/100,000) was in the range of 2.37-4.53(male 2.47-5.29, female 0.76-3.36) with an average of 3.29 (male 4.05, female 2.43). Age-specific annual incidence rate(/100,000) was 3.78 in the 0-4 year age group, 3.51 in the 5-9 year age group and 3.08 in the 10-14 year age group. Of the major types of childhood leukemia, the distribution of ALL was average 71.4%, of AML 23.3%, and of CML 4.5%. Of the major types of leukemia by age range, ALL showed highest in the 5-9 year age group, while AML in 0-4 and 10-14 year age groups. Sex-ratio(male to female) of major type of leukemia was 1.97 : 1 and 1.21 : 1, in ALL and AML groups, respectively, while all were male in CML. Conclusion : The average age-and-sex adjusted annual incidence rate(/100,000) of childhood leukemia in Busan from 1996 to 2000 was 3.29. Compared to data in related articles, this data suggests a steady increase in the incidence of childhood leukemia in the Busan area over the last 20 years since 1981.

소아 철결핍성 빈혈 환아에서 혈소판 증가증

임영탁 부산대학교 병원 암연구소 2008 부산대병원학술지 Vol.- No.24

Purpose : Reactive thrombocytosis is observed in many disorder in children such as infection, malignancy, kawasaki disease, acute blood loss and post-splenectomy state. Iron deficiency anemia (IDA) is also a cause of reactive thrombocytosis in children, but the mechanisms are still unknown. I searched the incidence and progress of thrombocytosis in patients with IDA in children. Subject and Methods : Total 80 patients were diagnosed and treated with IDA in Department of Pediatrics, Pusan National University Hospital from January 2000 to December 2005. Thrombocytosis was defined as a platelet count >450x10^(9)/ L. The patients who had diseases causing reactive thrombocytosis except IDA were excluded in this study. A retrospective analysis of case records was undertaken for children with IDA. Results : 1) Thirty children (37.5%) had thrombocytosis and 50 children (62.5%) had a normal platelet count but there is no children who had thrombocytopenia. 2) The mean age of patients who had thrombocytosis was significantly younger than patients without thrombocytopenia (3.44 yr vs 7.43 yr). 3) Most patients (56.7%) who had thrombocytosis showed only moderate elevation of platelets, but there are 3 patients (10.0%) whose platelet counts >700x10^(9)/ L. 4) The incidence of thrombocytosis does not changed whether the patients had gastrointestinal occult bleeding or not. 5) Platelet count was progressively decreased with iron treatment and was nearly normalized after 1 month of treatment. 6) There are no complications of thrombocytosis. Conclusions : IDA is one of the most frequent causes of reactive thrombocytosis in children. Reactive thrombocytosis induced by IDA have usually a benign course and need no specific treatment except iron.

소아 만성 특발성 혈소판 감소성 자반증에서 고용량 경구용 Dexamethasone 치료의 효과

곽정원,임영탁 부산대학교 병원 암연구소 2006 부산대병원학술지 Vol.- No.20

Purpose : The treatment of children with chronic idiopathic thrombocytopenic purpura (ITP) and clinically important thrombocytopenia remain a challenge. The major aim of therapeutic intervention is to prevent serious and potentially fatal bleeding, with minimal side effects. The effectiveness of pulsed high-dose oral dexamethasone therapy in children with chronic ITP is evaluated. Subject and Methods : Seventeen children (11 boys, 6 girls) who were refractory conventional standard therapies were included in the study. Dexamethasone was administered orally at a dosage of 40 mgj1.73m'jday for 4 days every 4 week in six courses. Results : At the end of the 6 cycles, 3 patients had complete response (17.6%), 3 patients had partial response (17.6%). Age at the time of treatment, gender, mean duration of chronic ITP and mean platelet count at the time of treatment were not associated with a different rate of response. The major side effects observed were emotional changes, pururitus, sleep disturbance, facial flush, increased appetite, gastrointestinal symptoms and headache. None of them had side effects that were severe enough to warrant discontinuing therapy. Conclusion : Although treatment with pulsed high-dose dexamethasone is not always effective in children with chronic ITP, we think that this therapeutic regimen deserved trial in children with chronic ITP because of its relatively minimal side effects and low cost.

골수이형성 증후군과 급성 골수성 백혈병에서 혈청 항p53 항체와 p53 단백 과발현의 임상적 의의

박정선,박태희,박태성,정주섭,임영탁,이은엽,조군제 大韓血液學會 2001 大韓血液學會誌 Vol.36 No.1

급성 및 만성 백혈병에서 p53 단백 과잉발현과 혈청 p53 항체의 임상적 의의

박정선,박태희,이은,임영탁,정주섭엽,조군제 대한임상병리학회 2000 대한임상병리학회지 Vol.20 No.3