• Genomics & Informatics
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• 제18권1호
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• pp.6.1-6.9
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• 2020

Acute leukemia represents the most common pediatric malignancy comprising diverse subtypes with varying prognosis and treatment outcomes. New and targeted treatment options are warranted for this disease. Patient-derived xenograft (PDX) models are increasingly being used for preclinical testing of novel treatment modalities. A novel approach involving targeted error-corrected RNA sequencing using ArcherDX HemeV2 kit was employed to compare 25 primary pediatric acute leukemia samples and their corresponding PDX samples. A comparison of the primary samples and PDX samples revealed a high concordance between single nucleotide variants and gene fusions whereas other complex structural variants were not as consistent. The presence of gene fusions representing the major driver mutations at similar allelic frequencies in PDX samples compared to primary samples and over multiple passages confirms the utility of PDX models for preclinical drug testing. Characterization and tracking of these novel cryptic fusions and exonal variants in PDX models is critical in assessing response to potential new therapies.

• Clinical and Experimental Pediatrics
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• 제54권4호
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• pp.169-175
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• 2011

Purpose: Acute internal hemorrhage is an occasionally life-threatening complication in pediatric cancer patients. Many therapeutic approaches have been used to control bleeding with various degrees of success. In this study, we evaluated the efficacy of selective internal iliac artery embolization for controlling acute intractable bleeding in children with malignancies. Methods: We retrospectively evaluated the cases of 6 children with various malignancies (acute lymphoblastic leukemia, acute myelogenous leukemia, chronic myelogenous leukemia, T-cell prolymphocytic leukemia, Langerhans cell histiocytosis, and rhabdomyosarcoma), who had undergone selective arterial embolization (SAE) of the internal iliac artery at the Chonnam National University Hwasun Hospital between January 2004 and December 2009. SAE was performed by an interventional radiologist using Gelfoam$^{(R)}$ and/or Tornado$^{(R)}$ coils. Results: The patients were 5 boys and 1 girl with median age of 6.9 years (range, 0.7-14.8 years) at the time of SAE. SAE was performed once in 4 patients and twice in 2, and the procedure was unilateral in 2 and bilateral in 4. The causes of hemorrhage were as follows: hemorrhagic cystitis (HC) in 3 patients, procedure-related internal iliac artery injuries in 2 patients, and tumor rupture in 1 patient. Initial attempt at conservative management was unsuccessful. Of the 6 patients, 5 (83.3%) showed improvement after SAE without complications. Conclusion: SAE may be a safe and effective procedure for controlling acute intractable hemorrhage in pediatric malignancy patients. This procedure may obviate the need for surgery, which carries an attendant risk of morbidity and mortality in cancer patients with critical conditions.

• Advances in pediatric surgery
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• 제7권1호
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• pp.59-63
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• 2001

소아 ALL 환자 2례에서 천공성 맹장염을 경험하였다. 주요 증상으로 고열, 복통, 복부팽만 설사가 있었으며, 혈액 검사에서 백혈구 감소증 및 혈소판 감소증이 있었다. 진단은 초음파검사와 콤푸터 단층촬영으로 비정상적인 장벽 비후를 봄으로 가능하였다. 1예는 수술적 치료를 함으로 1예는 내과적 치료로 좋은 결과를 얻었다. 백혈병환자에서 항암 치료 중 고열, 하복부 동통이 있고 백혈구 감소증이 있을 경우는 맹장염의 기능성을 생각하고 조기에 초음파 검사 또는 컴퓨터 단층촬영을 하여 적절히 대처하여야 할 것으로 생각된다. 그리고 비록 천공성 맹장염이라도 증상이 국소적 일 때는 내과적 치료를 시도해 볼 필요가 있다고 생각된다.

• Asian Pacific Journal of Cancer Prevention
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• 제16권11호
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• pp.4609-4614
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• 2015

Background: In recent decades, the prognosis for childhood leukemia has improved, especially for acute lymphoblastic leukemia (ALL). In Thailand, though, the survival rate for ALL is unimpressive. In 2006, standard national protocols for childhood leukemia treatment were implemented. We herein report the outcome of the ALL national protocols and explanations behind discrepancies in outcomes between institutions. Materials and Methods: Between March 2006 and February 2008, 486 children with ALL from 12 institutions were enrolled in the Thai national protocols. There were 3 different protocols based on specific criteria: one each for standard risk, high risk and Burkitt's ALL. We classified participating centers into 4 groups of institutions, namely: medical schools in Bangkok, provincial medical schools, hospitals in Bangkok and provincial hospitals. We also evaluated supportive care, laboratory facilities in participating centers, socioeconomics, and patient compliance. Overall and event-free survival were determined for each group using the Kaplan Meier method. Statistical differences were determined using the log-rank test. Previous outcomes of Thai childhood ALL treatment between 2003 and 2005 served as the historic control. Results: Five-year overall survival of ALL treated using the Thai national protocol was 67.2%; an improvement from the 63.7% of the 12-institute historical control (p-value=0.06). There were discrepancies in event-free survival of ALL between centers in Bangkok and up-country provinces (69.9% vs 51.2%, p-value <0.01). Socioeconomics and patient compliance were key elements in determining the outcome (65.5% vs 47.5%, 59.4% vs 42.9%) (p-value < 0.02). Conclusions: Implementation of standard national protocols for childhood leukemia in Thailand did not significantly improve the outcome of ALL. Factors leading to better outcomes included (a) improvement of treatment compliance (b) prevention of treatment abandonment and (c) financial support to the family.

• Pediatric Infection and Vaccine
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• 제27권2호
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• pp.134-139
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• 2020

표피 포도알균은 사람 피부에 있는 정상균이나, 체내 이물질을 가진 사람이나 면역 저하자에게는 심각한 감염을 일으킬 수 있다. 13세 남자가 발열, 근육통으로 입원하였고 두피, 팔과 다리에 통증이 있는 결절성 병변이 만져졌다. 혈액검사에서 범혈구 감소증과 모세포 80% 소견을 보였고 급성 골수성 백혈병으로 진단되었다. 전신 자기공명영상 검사에서 가장자리 조영 증강을 보이는 다발 낭성 병변이, 초음파 검사에서 에코성 낭성 병변과 그 내부의 에코성 결절이 팔과 다리의 근육 내부에서 관찰되어, 낭미충증이 강력히 의심되었다. 그러나 초음파 유도하 조직 검사에서 농양이 확인되었고, 조직 배양검사에서 표피 포도알균이 동정되었다. 저자들은 백혈병 환자에서 낭미충증으로 오인되었던 표피 포도알균에 의한 전신 다발 병변이 발생한 예를 경험하였기에 보고하는 바이다.

• Journal of Korean Neurosurgical Society
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• 제50권3호
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• pp.235-239
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• 2011

Radiation therapy has been widely applied for cancer treatment. Childhood acute lymphocytic leukemia (ALL), characterized by frequent central nervous system involvement, is a well documented disease for the effect of prophylactic cranio-spinal irradiation. Irradiation, however, acts as an oncogenic factor as a delayed effect and it is rare that glioblastoma multiforme develops during the remission period of ALL. We experienced a pediatric radiation-induced GBM patient which developed during the remission period of ALL, who were primarily treated with chemotherapeutic agents and brain radiation therapy for the prevention of central nervous system (CNS) relapse. Additionally, we reviewed the related literature regarding on the effects of brain irradiation in childhood and on the prognosis of radiation induced GBM.

• Clinical and Experimental Pediatrics
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• 제55권7호
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• pp.249-253
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• 2012

We report a case of a 13-year-old girl with acute lymphoblastic lymphoma- leukemia, who presented with a cardiac metastasis in the right ventricle, resulting in a pulmonary embolism. At the time of her leukemia diagnosis, a cardiac mass was incidentally found. The differential diagnosis for this unusual cardiac mass included cardiac tumor, metastasis, vegetation, and thrombus. Empirical treatment was initiated, including anticoagulation and antibiotics. She underwent plasmapheresis and was administered oral prednisolone for her leukemia. Five days later, she experienced sudden hemodynamic collapse and required extracorporeal membrane oxygenation insertion and emergency surgery. These interventions proved futile, and the patient died. Pathology revealed that the cardiac mass comprised an aggregation of small, round, necrotic cells consistent with leukemia. This is the first known case of acute lymphoblastic leukemia presenting as a right ventricular mass, with consequent fatal acute pulmonary embolism. A cardiac mass in a child with acute leukemia merits investigation to rule out every possible etiology, including vegetation, thrombus, and even a mass of leukemic cells, which could result in the fatal complication of pulmonary embolism.

• 대한방사선기술학회지:방사선기술과학
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• 제38권1호
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• pp.17-21
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• 2015

소아백혈병의 치료방법 인 조혈모세포이식법의 전처치 방법으로서 항암제와 함께 대표적으로 사용되고 있는 전신방사선조사에 대하여 치료를 시행하기 전 정량적인 인체장기에 대한 선량을 평가하고자 하였다. 모의실험 프로그램 MCNPX를 사용하여 가상의 공간에서 소아용 모의피폭체를 대상으로 조직보상체의 재질을 변화시켜 실험을 진행하였다. 그 결과 첫째, 평균피부선량은 조직보상체의 재질에 따라 Plexiglass는 74.60 mGy/min, Al은 73.96 mGy/min, Cu는 72.26 mGy/min, Pb의 경우 67.90 mGy/min을 보였다. 둘째, 심부선량은 조직보상체 재질에 상관없이 갑상선, 생식선, 소화기계, 머리, 폐, 신장의 순으로 높게 나타났다. 끝으로 조직보상체와 환자와의 거리는 50 cm 이격시켰을 때가 이상적인 것으로 분석되었다. 본 연구결과를 토대로 할 때, 조직보상체 Al, Cu, Pb은 현재 사용되고 있는 Plexiglass 재질을 대체할 수 있을 것으로 판단된다.

• Childhood Kidney Diseases
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• 제13권2호
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• pp.278-281
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• 2009

소아에서 혈액암의 경우 간비종대 없이 신장 비대만을 보이는 경우는 매우 드물다. 저자들은 한 달간 급속히 진행하는 복부 팽만을 주소로 병원에 온 9개월 된 남아에서, 간비종대가 없이 양측 거대신 소견만을 보이는 급성 림프구성 백혈병을 경험하였기에 그 임상상을 보고하는 바이다.

• Pediatric Infection and Vaccine
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• 제19권3호
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• pp.162-167
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• 2012

RSV는 2세 미만의 소아에서 급성 하기도 감염으로 인한 입원의 주원인이다. 특히 미숙아, 선천성 심폐질환, 면역결핍이 동반된 경우 주요 위험군으로 알려져 있다. 고위험군의 소아에서 중증 RSV 감염의 경우 리바비린 흡입요법이 허가가 되어 있으나 고비용, 안전성 등의 문제로 국내 임상에서의 사용은 매우 제한적인 실정이다. 저자들은 8개월 여환이 급성 림프구성 백혈병으로 관해유도요법 항암치료 중 발생한 RSV 폐렴으로 기흉, 기종격동, 호흡부전이 동반된 중증 감염을 경험하였다. 정맥용 면역글로불린, 경구 리바비린 투여에 반응이 없어 리바비린 흡입 치료를 시행하였고, 이후 임상적 호전을 경험하였기에 보고하는 바이다.