선천거대결장증이 늦게 발현한 10세 남자에서 발생한 복강구획증후군

정가은,장필상 대한소아응급의학회 2021 대한소아응급의학회지 Vol.8 No.2

Abdominal compartment syndrome (ACS) involves adverse physiologic consequences arising from the increased intraabdominal pressure, leading to high mortality. However, this syndrome has been scarcely reported in pediatric emergency settings. We describe a 10-year-old boy with ACS presenting with painful abdominal distension, oliguria, and dyspnea. Despite the absence of known congenital anomalies, he had undergone frequent episodes of constipation since 5 years of age, and had not defecated for recent 2 months. With computed tomography scans showing the entire colorectal distension, his manifestations were considered to have stemmed from congenital aganglionic megacolon, which had gone undetected. This case underlines the needs for considering ACS and consequent surgical decompression in a child with severe abdominal distension.

Outcome and Prognostic Factors for ETV6/RUNX1 Positive Pediatric Acute Lymphoblastic Leukemia Treated at a Single Institution in Korea

이재욱,김성구,장필상,정낙균,정대철,김명신,조빈,김학기 대한암학회 2017 Cancer Research and Treatment Vol.49 No.2

Purpose ETV6/RUNX1 (+) acute lymphoblastic leukemia (ALL), which is the most common genetic subtype of pediatric ALL, has a favorable prognosis. In this study, we analyzed the outcome of ETV6/RUNX1 (+) ALL patients treated at our institution with the aim of identifying significant prognostic variables. Materials and Methods Sixty-three patients were diagnosed with ETV6/RUNX1 (+) ALL from 2005 to 2011. Prognostic variables studied included minimal residual disease (MRD) as detected by ETV6/RUNX1 (+) fusion, and the presence of additional cytogenetic abnormalities. Results The 5-year event-free survival was 84.1±4.6%, with 10 patients relapsing at a median of 28.3 months from diagnosis for a 5-year cumulative incidence of relapse of 15.9±4.6%. Multivariate analysis revealed that the presence MRD, as detected by real-time quantitative- polymerase chain reaction or fluorescence in situ hybridization for ETV6/RUNX1 fusion at end of remission induction, and the presence of additional structural abnormalities of 12p (translocations or inversions) negatively affected outcome. Despite treatment such as allogeneic hematopoietic cell transplantation, eight of the 10 relapsed patients died from disease progression for overall survival of 82.5±6.9%. Conclusion ETV6/RUNX1 (+) ALL may be heterogeneous in terms of prognosis, and variables such as MRD at end of remission induction or additional structural abnormalities of 12p could define a subset of patients who are likely to have poor outcome.

흡인성 폐렴이 반복된 뇌성마비 환자에서의공장창냄술(Jejunostomy) 시행 1례

김효진,이근영,장필상,김동언,김영훈,김지일,이혜경,김진택 대한 소아알레르기 호흡기학회 2005 Allergy Asthma & Respiratory Disease Vol.15 No.2

Cerebral palsy(CP) usually arises in the prenatal period, interferes with normal development of the brain and presents as a disorder of motor dysfunction. It is a common cause of severe neurologic disability in children with incidence of 2 per 1000 live births in Canada. Presently, with the persistent increase in premature births and improved care in the perinatal period, the incidence of CP is strikingly augmented. According to neurologically impaired motor ability, they commonly have feeding difficulties, like swallowing, leading to frequent aspiration pneumonia and gastroesophageal reflux. Therefore, improvement of feeding techniques should be an urgent requirement to correct the risk of poor growth. We report a case of CP due to kernicterus who have recurrent pneumonia and growth failure, experience a jejunostomy to overcome the feeding problems. 저자들은 흡인성 폐렴이 자주 반복되는 뇌성마비 환자에서 공장창냄술을 시행한 후 튜브 식이요법을 계속하여 환자의 영양상태를 호전시키고 흡인성 폐렴 방지에 효과가 있었기에 이를 문헌고찰과 함께 보고하는 바이다.

Differing Outcomes of Patients with High Hyperdiploidy and ETV6-RUNX1 Rearrangement in Korean Pediatric Precursor B Cell Acute Lymphoblastic Leukemia

이재욱,김성구,장필상,정낙균,조빈 대한암학회 2021 Cancer Research and Treatment Vol.53 No.2

Purpose Recent cooperative trials in pediatric acute lymphoblastic leukemia (ALL) report long-term event-free survival (EFS) of greater than 80%. In this study, we analyzed the outcome and prognostic factors for patients with precursor B cell ALL (n=405) diagnosed during a 10-year period (2005-2015) at our institution.Materials and MethodsAll patients were treated with a uniform institutional regimen based on four risk groups, except for steroid type; patients diagnosed up till 2008 receiving dexamethasone, while subsequent patients received prednisolone. None of the patients received cranial irradiation in first complete remission.ResultsThe 10-year EFS and overall survival was 76.3%±2.3% and 85.1%±1.9%. Ten-year cumulative incidence of relapse, any central nervous system (CNS) relapse and isolated CNS relapse was 20.8%±2.2%, 3.7%±1.1% and 2.5%±0.9% respectively. A comparison of established, good prognosis genetic abnormalities showed that patients with high hyperdiploidy had significantly better EFS than those with ETV6-RUNX1 rearrangement (10-year EFS of 91.2%±3.0% vs. 79.5%±4.4%, p=0.033). For the overall cohort, male sex, infant ALL, initial CNS involvement, and Philadelphia chromosome (+) ALL were significant factors for lower EFS in multivariate study, while high hyperdiploidy conferred favorable outcome. For high and very high risk patients (n=231), high hyperdiploidy was the only significant factor for EFS in multivariate study.ConclusionRegarding good prognosis genetic abnormalities, patients with high hyperdiploidy had significantly better outcome than ETV6-RUNX1 (+) patients. High hyperdiploidy was a major, favorable prognostic factor in the overall patient group, as well as the subgroup of patients with higher risk.

전신성 홍반성 루푸스 환아에서 병발한 혈구 탐식성 조직구 증식증 1례

황자영,노석만,이진,장필상,김영훈,김진택,이준성,Hwang, Ja Young,No, Suk Man,Lee, Jin,Jang, Pil Sang,Kim, Young Hoon,Kim, Jin Tack,Lee, Joon Sung 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.10

본 저자들은 전신성 홍반성 루푸스와 미만성 증식성 사구체신염으로 진단된 남아의 치료과정 중 발열과 혈구 감소증이 지속된 환아에서 이차성 혈구 탐식성 조직구 증식증을 경험하였기에 문헌 고찰과 함께 보고하는 바이다. Hemophagocytic lymphohistiocytosis is a reactive disorder characterized by a generalized non-malignant histiocytic proliferation with prominent hemophagocytosis by stimulated histiocytes in the bone marrow and reticuloendothelial systems resulting in pancytopenia and liver dysfunction. Several diseases including infection, malignancy and autoimmune disease are known to be causative disorders. This case demonstrated histiocytic hemophagocytosis in the bone marrow, resulting in pancytopenia during treatment of systemic lupus erythematosus and did not show any underlying disease.

영유아의 급성 설사에서 Lactobacillus reuteri의 치료 효과

엄태훈,오은영,김영훈,이현승,장필상,김동언,김진택,이병철 대한소아청소년과학회 2005 Clinical and Experimental Pediatrics (CEP) Vol.48 No.9

Purpose : Certain strains of lactobacilli are known to accelerate recovery from acute diarrhea. Lactobacillus reuteri is isolated from human breast milk and a commonly occurring Lactobacillus species with therapeutic potential in acute diarrhea. The purpose of the present study was to investigate the therapeutic effect of L. reuteri in acute diarrhea in young children. Methods : Fifty patients between 6 and 36 months of age hospitalized with acute diarrhea (rotavirus in 40 percent) were randomized into two groups to receive either 108 colony-forming units of L. reuteri or a matching placebo, twice a day for their length of hospitalization, or for up to 5 days. Antidiarrheal drugs were not prescribed to either group. The clinical outcome of diarrhea was evaluated. Results : The mean duration of watery diarrhea after initiation of treatment was 2.3 days for the L. leuteri group(n=25) vs. 2.9 days for the placebo group(n=25)(P=0.072). By the second day of treatment, watery diarrhea persisted in 64 percent of patients receiving L. reuteri, compared to 84 percent of those receiving placebo(P=0.006). On the second day, the mean frequency of watery diarrhea was 1.9 in the L. leuteri group and 3.4 in the placebo(P=0.046). Also, vomiting continued to the second day in 16 percent of patients receiving L. reuteri and 40 percent of those recieving placebo(P= 0.031). Conclusion : L. reuteri is effective as a therapeutic agent in acute diarrhea in children. 목 적 : Lactobacilli의 일부 균주는 급성 설사의 회복을 도와준다. Lactobacillus reuteri는 인간에서 비롯된 균주로 위장관내에 자연적으로 집락형성을 한다. 특히 유아설사를 일으키는 로타바이러스를 억제하여 영유아 설사를 완화시켜주고 장내 다른 유익한 유산균 수를 증가시켜준다는 보고가 있다. 본 연구에서는 경구로 L. reuteri를 투여하였을 때 급성 설사에서 치료적 효과가 있는지 여부를 확인하고자 하였다.방 법 : 급성 설사로 입원한 6개월에서 36개월 사이의 영유아 50명(40% 로타바이러스)을 대상으로 하였다. 환아들을 각 25명씩 무작위로 108 colony-forming unit(CFU)를 투여하는 L. reuteri군과 위약을 투여하는 위약군으로 나누어 하루에 2회 L. reuteri나 위약을 입원 기간 동안 혹은 최대한 5일간 투여하였다. 임상적인 평가로 입원 후 수양성 설사 기간, 입원 기간, 수양성 설사 일수 및 횟수, 구토 일수 등을 조사하였다.결 과 : 설사 후 수양성 설사의 평균 기간은 L. reuteri군에서 2.3일이었으며 위약군에서 2.9일로 L. reuteri군에서 짧았으나 통계적 차이가 없었다(P=0.072). 치료 2병일째 수양성 설사는 L. reuteri군에서 64%이었으며 위약군에서 84%로 통계학적 차이가 있었다(P=0.006). 치료 2병일째 평균 수양성 설사횟수는 L. reuteri군에서 1.9회이었으며 위약군에서 3.4회로 통계학적 차이가 있었다(P=0.046). 치료 2병일째 구토는 L. reuteri군에서 16%이었으며 위약군에서 40%로 통계학적 차이가 있었다(P= 0.031). 결 론:이상의 결과는 Lactobacillus reuteri가 영유아 설사에 치료효과가 있다는 것을 시사한다.

Outcome of Intensive Therapy for Children with Relapsed Acute Myeloid Leukemia: A Single Institution Korean Study

이재욱,유재원,김성구,장필상,정낙균,조빈 대한암학회 2022 Cancer Research and Treatment Vol.54 No.4

Purpose Approximately 30%-40% of pediatric acute myeloid leukemia (AML) patients relapse. In this study, we analyzed the outcome and prognostic factors of relapsed AML patients who had previously received first-line therapy at our institution. Materials and Methods The study group consisted of 50 patients who had been diagnosed with AML from April 2009 to December 2018, and then showed first relapse. Thirty-two of the patients (64%) had previously received allogeneic hematopoietic stem cell transplantation (HSCT) in first complete remission (CR). Results Forty-five of the patients (90%) received intensive chemotherapy upon diagnosis of relapse, and 76% (34/45) of these patients achieved a second CR. Estimated 5-year overall survival for these 45 patients was 44.9%±7.6%. Time from diagnosis to relapse, extramedullary involvement (EMI) at diagnosis, core binding factor AML, and complex karyotype were significant prognostic factors; in multivariate study, both time from diagnosis to relapse and EMI at diagnosis proved significant. There was no difference in 5-year disease-free survival between patients previously treated with chemotherapy only and those who received HSCT in first CR (52.4%±14.9% vs. 52.6%±11.5%). Of the 19 patients who achieved second CR after previous allogeneic HSCT in first CR and subsequent relapse, 11 were treated with chemotherapy only, and seven survive disease-free. Conclusion Intensive therapy allowed for long-term survival in 40%-50% of patients, and 50% of patients who achieved second CR, regardless of prior treatment modalities in first CR. Intensive treatment may allow for salvage of a significant portion of patients with relapsed pediatric AML. Purpose Approximately 30%-40% of pediatric acute myeloid leukemia (AML) patients relapse. In this study, we analyzed the outcome and prognostic factors of relapsed AML patients who had previously received first-line therapy at our institution.Materials and Methods The study group consisted of 50 patients who had been diagnosed with AML from April 2009 to December 2018, and then showed first relapse. Thirty-two of the patients (64%) had previously received allogeneic hematopoietic stem cell transplantation (HSCT) in first complete remission (CR).Results Forty-five of the patients (90%) received intensive chemotherapy upon diagnosis of relapse, and 76% (34/45) of these patients achieved a second CR. Estimated 5-year overall survival for these 45 patients was 44.9%±7.6%. Time from diagnosis to relapse, extramedullary involvement (EMI) at diagnosis, core binding factor AML, and complex karyotype were significant prognostic factors; in multivariate study, both time from diagnosis to relapse and EMI at diagnosis proved significant. There was no difference in 5-year disease-free survival between patients previously treated with chemotherapy only and those who received HSCT in first CR (52.4%±14.9% vs. 52.6%±11.5%). Of the 19 patients who achieved second CR after previous allogeneic HSCT in first CR and subsequent relapse, 11 were treated with chemotherapy only, and seven survive disease-free.Conclusion Intensive therapy allowed for long-term survival in 40%-50% of patients, and 50% of patients who achieved second CR, regardless of prior treatment modalities in first CR. Intensive treatment may allow for salvage of a significant portion of patients with relapsed pediatric AML.

Immune reconstitution after allogeneic hematopoietic stem cell transplantation in children: a single institution study of 59 patients

김현오,오현진,이재욱,장필상,정낙균,조빈,김학기 대한소아청소년과학회 2013 Clinical and Experimental Pediatrics (CEP) Vol.56 No.1

Purpose: Lymphocyte subset recovery is an important factor that determines the success of hematopoietic stem cell transplantation (HSCT). Temporal differences in the recovery of lymphocyte subsets and the factors influencing this recovery are important variables that affect a patient’s posttransplant immune reconstitution, and therefore require investigation. Methods: The time taken to achieve lymphocyte subset recovery and the factors influencing this recovery were investigated in 59 children who had undergone HSCT at the Department of Pediatrics,The Catholic University of Korea Seoul St. Mary’s Hospital, and who had an uneventful follow-up period of at least 1 year. Analyses were carried out at 3 and 12 months post-transplant. An additional study was performed 1 month post-transplant to evaluate natural killer (NK) cell recovery. The impact of preand post-transplant variables, including diagnosis of Epstein-Barr virus (EBV) DNAemia posttransplant,on lymphocyte recovery was evaluated. Results: The lymphocyte subsets recovered in the following order: NK cells, cytotoxic T cells, B cells,and helper T cells. At 1 month post-transplant, acute graft-versus-host disease was found to contribute significantly to the delay of CD16+/56+ cell recovery. Younger patients showed delayed recovery of both CD3+/CD8+ and CD19+ cells. EBV DNAemia had a deleterious impact on the recovery of both CD3+ and CD3+/CD4+ lymphocytes at 1 year post-transplant. Conclusion: In our pediatric allogeneic HSCT cohort, helper T cells were the last subset to recover. Younger age and EBV DNAemia had a negative impact on the post-transplant recovery of T cells and B cells.

Evaluation of risk for graft-versus-host disease in children who receive less than the full doses of mini-dose methotrexate for graft-versus-host disease prophylaxis in allogeneic hematopoietic stem cell transplantation

엄숙경,최혜윤,이재욱,장필상,정낙균,정대철,조빈,김학기 대한소아청소년과학회 2013 Clinical and Experimental Pediatrics (CEP) Vol.56 No.11

Purpose: The use of cyclosporine and mini-dose methotrexate (MTX) is a common strategy for graftversus-host disease (GVHD) prophylaxis in allogeneic transplants. We investigated whether patients who receive fewer than the planned MTX doses are at increased risk for GVHD. Methods: The study cohort included 103 patients who received allogeneic transplants at the Department of Pediatrics of The Catholic University of Korea College of Medicine, from January 2010to December 2011. MTX was administered on days 1, 3, 6, and 11 after transplant at a dose of 5 mg/m2 each. Within the cohort, 76 patients (74%) received all 4 doses of MTX [MTX(4) group], while 27patients (26%) received 0–3 doses [MTX(0–3) group]. Results: Although there was no difference in neutrophil engraftment between the 2 groups, platelet engraftment was significantly faster in the MTX(4) group (median, 15 days), compared to the MTX(0–3) group (median, 25 days; P =0.034). The incidence of grades II–IV acute GVHD was not different between the MTX(4) and MTX(0–3) groups (P =0.417). In the multivariate study, human leukocyte antigen mismatch was the most significant factor causing grades II–IV acute GVHD (P =0.002),followed by female donor to male recipient transplant (P =0.034). No difference was found between the MTX(4) and MTX (0–3) groups regarding grades III–IV acute GVHD, chronic GVHD, and disease-free survival. Conclusion: Our results indicate that deviations from the full dose schedule of MTX for GVHD prophylaxis do not lead to increased incidence of either acute or chronic GVHD.

조혈모세포이식 후 중환자실 치료를 받은 소아 환자의 예후 예견 인자 분석

최혜선,이은정,이재욱,장필상,정낙균,조빈,정대철,김학기 대한중환자의학회 2011 Acute and Critical Care Vol.26 No.4

Background: Pediatric Index of Mortality 2 (PIM2) is a useful scoring system for the prediction of prognosis, and Oncological Pediatric Risk of Mortality (O-PRISM) for ICU support in children with HSCT. We investigated prognostic prediction and risk factors for survival through early detection of admission to ICU after HSCT. Methods: We reviewed retrospectively medical records of children cared for in ICU after HSCT between 2004 and 2010. Patients who died within 2 hours after admittance to ICU were excluded. We analyzed the worst parameters in ICU by a t-test, Cox-regression, multiple logistic regression and a receiver operating characteristics curve (ROC). Results: 54 children, with fifty-five disease events, were admitted to ICU after HSCT. Sixteen children were diagnosed with high-risk disease status and 8 with non-malignant diseases. Stem cells were sourced from 14 matched siblings, 22 unrelated donors and 19 cord bloods. The median duration in ICU was 8.5 days (0.5−110). The reasons for admission to ICU were 32 pulmonary, 14 neurologic, and 9 hemodynamic events. Six patients (11.1%) survived after intensive care. The factor indicating discharge with survival was mental status (p = 0.04), although other factors included FiO_2, prothrombin time, potassium, and pupil reflex in univariate analysis. In multiple logistic regression, there were significant factors of PaCO_2 (p = 0.028), O-PRISM (p = 0.039), and PIM2 (p = 0.004) for prognosis. For prediction of prognosis, O-PRISM (p = 0.019) was superior to PIM2 (p = 0.435) in intensive care children after HSCT. Conclusions: O-PRISM might be a predictable scoring system for children with ICU support, and the Glasgow coma scale and PaCO_2 were more reliable prognostic factors in the post-HSCT period.