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Kikuchi-Fujimoto disease is a rare and benign disease, characterized by regional cervical lymphadenopathy with fever of unknown origin, affecting mainly young women. This self-limiting disease of unknown etiology has rarely been reported in children, so it has been frequently confused with malignancies. We describe a case of a child who was initially mistaken for malignant lymphoma because of multiple lymph nodes with 18-fluorodeoxyglucose uptake on positron emission tomography and was finally diagnosed with Kikuchi-Fujimoto disease by excisional biopsy of the affected lymph node.

We retrospectively investigated the outcomes of HLA-matched unrelated BMT (MU-BMT, n = 13) and HLA-identical sibling donor BMT (MS-BMT, n = 17) for childhood AML in CR1 between June 2002 and August 2005. Engraftment of neutrophil and platelet did not differ between the two transplant groups. The cumulative incidence of grade II-IV acute GVHD and any chronic GVHD at three yr was not different between MS-BMT and MU-BMT. Of the 30 patients, four patients experienced relapses (three with MS-BMT, one with MU-BMT) and four patients died of transplant-related complications (two with MS-BMT, two with MU-BMT). A total of 23 patients survived with a median follow-up of 43.2 months. The Kaplan-Meier estimates for EFS rates at three yr were 71% and 77% for MS-BMT and MU-BMT, respectively, and the OS rates were 76% and 77% for MS-BMT and MU-BMT, respectively. The outcome of HLA-matched unrelated BMT is comparable to that of HLA-identical sibling BMT for childhood AML in CR1. HLA-matched unrelated BMT may be recommended for patients who have AML in CR1 without an HLA-matched sibling donor.

Imatinib is effective for a variety of hematologic and nonhematologic malignancies. Well-known ocular side effects are periorbital edema, epiphora, extraocular muscle palsy, and blepharoconjunctivitis. However, optic disc edema has not been reported as a complication of imatinib mesylate. Herein, we describe a patient with chronic myeloid leukemia (CML) who developed optic disc edema during treatment with imatinib. A 14-year-old Korean girl was referred to ophthalmology for a 3-week history of photopsia in both eyes. She had started taking imatinib daily 2 months previously for CML. At the initial exam, slit lamp showed optic disc edema in both eyes, even though visual acuity was 20/20 and other optic nerve function evaluations were within normal limits. We recommended to the oncologist discontinuation of the imatinib treatment. When the patient was followed for 1 week after stopping imatinib treatment, the frequency of photopsia decreased and the optic disc edema improved. Because a second examination 3 weeks after discontinuation of imatinib revealed much improved optic disc edema, she restarted the imatinib treatment. No ocular side effects have been noted so far. Optic disc edema should be considered one of the complications associated with imatinib. We propose discontinuation of the treatment for a short period when optic disc edema occurs.

The purpose of this study was to investigate the effect of a Pholiota adiposa extract on fat mass in hyperlipidemic mice fed on a high-fat diet. The water extracts from P. adiposa (ASI 24018) were not affected in the total triglyceride contents and epididymal fat mass in mice fed on a high-fat diet, but the retroperitoneal fat mass decreased significantly. This result suggests that the P. adiposa extract may be a potential candidate for use as a functional food that can act as a prophylactic against hyperlipidemia. However, the P. adiposa extract showed no effect in the total triglyceride contents and epididymal fat mass.

목적: 국내 로타바이러스 백신 도입 전후로 8년간 입원한 로타바이러스 위장관염의 역학 및 임상 양상의 변화를 파악하고자 하였고, 최근 2년간 입원 환자를 대상으로 로타바이러스 예방접종의 효과에 대하여 분석하였다. 방법: 2006년 1월부터 2013년 12월까지 급성 위장관염으로 입원한 5세 미만의 5,697명의 환아 중 로타바이러스 항원 검사에 양성을 보인 1,165명의 연도 및 계절별추이를 분석하였다. 그리고 2011년 12월부터 2013년 12월까지 급성 위장관염으로 입원한 환아들 중 460명을 대상으로 설문조사를 실시하여 로타바이러스 백신 접종 여부, 접종 횟수, 증상 등에 대하여 파악하였고, 이들 중 로타바이러스 항원검사에서 양성이 확인된 124명을 대상으로 예방접종 시행 여부에 따른 증상 및 검사실 결과를 비교 분석하였다. 결과: 1) 로타바이러스 위장관염의 연령별 분포는 2세미만의 환아가 전체의 64.5%를 차지하였다. 연도별 로타바이러스 위장관염 발생 수는 2010년부터 급격히 감소하였다. 연도별 월별 분포는 2008년까지는 12월에 발생이 증가하기 시작하여 1-2월에 정점을 이루었으나, 2010년 이후로는 1-2월에 발생이 증가하기 시작하여 3-4월에 정점을 이루는 양상으로, 백신 도입 이후 로타바이러스의 활동의 시작, 계절적 정점이 늦춰지는 양상을 보였다. 2)로타바이러스 예방접종을 시행하지 않은 환자군에서 로타바이러스 위장관염 발병률이 높았다(P

Purpose In this study, we analyzed a cohort of children with chronic graft-versus-host disease (GvHD) according to the NIH consensus classification (NCC) in order to observe whether global assessment at diagnosis correlates with GvHD-specific endpoints. We then studied the clinical course of these patients, specifically with regards to episodes of GvHD exacerbation requiring treatment escalation. Materials and Methods Recipients of either allogeneic bone marrow transplantation (BMT) or peripheral blood stem cell transplantation (PBSCT) from January 2006 to August 2008 at the Department of Pediatrics, The Catholic University of Korea were evaluated for chronic GvHD, which was diagnosed according to the NCC. The course of chronic GvHD in these patients was then followed. Results Of 59 evaluable patients, 23 developed chronic GvHD for a cumulative incidence of 39.3%. Upon multivariate analysis, previous acute GvHD (≥grade II) had a significant impact on chronic GvHD incidence. With a median duration of systemic treatment for chronic GvHD of 501 days, no significant relationship was found between initial global severity of chronic GvHD and either duration of immunosuppressive treatment or final clinical response to treatment. Fifteen patients (65%) experienced at least one episode of chronic GvHD exacerbation during the period of follow-up, with a median of four exacerbations in the subgroup of patients who experienced such events. Lung GvHD resulted in the highest number of exacerbations per diagnosed patient, followed by oral GvHD. Conclusion Analysis of this small cohort indicates that global assessment as proposed by the NCC may have limited correlations with GvHD-specific endpoints, possibly due to the favorable response of children to treatment.

Congenital leukemia is uncommon and excluding transient myeloproliferation associated with Down syndrome, makes up approximately 1% of childhood leukemias. A newborn boy was born with multiple subcutaneous nodules and large purpuric papules. Skin biopsy revealed proliferation of atypical hematologic cells in the dermis. Bone marrow morphology was consistent with acute myeloid leukemia (M5) and cytogenetic studies revealed t(8;16) and t(17;19) double translocation. Although prognosis of congenital leukemia is known to be dismal, recent reports showed spontaneous remissions. With the fear of chemotherapy-related toxicity, to treat or not to treat may be a dilemma both to parents and pediatricians. We report our experience and review the literature.

We investigated the outcome of idarubicin plus N(4)-behenoyl-1-beta-D-arabinofuranosyl cytosine (BHAC)-based chemotherapy (BHAC group, n=149) compared to idarubicin plus cytarabine-based chemotherapy (cytarabine group, n=191) for childhood acute myeloid leukemia (AML). Between January 1996 and December 2005, 340 children with AML from 5 university hospitals in Korea received the BHAC-based or cytarabine-based chemotherapy, with or without hematopoietic stem cell transplantation. After induction therapy, 264 (77.6%) of 340 children achieved a complete remission (CR) and 43 (12%) achieved a partial remission (PR). The CR rate in the BHAC group was higher than in the cytarabine group (85.2% vs. 71.7%, P=0.004). However, the overall response rate (CR+PR) was not different between the two groups (93.3% vs. 87.9%, P=0.139). The 5-yr estimates of overall survival (OS) of children in the two groups were similar (54.9% for the BHAC group vs. 52.4% for the cytarabine group, P=0.281). Although the results were analyzed according to the treatment type and cytogenetic risk, the OS showed no significant difference between the BHAC group and the cytarabine group. In the present study, the clinical outcomes of the BHAC-based chemotherapy, consisting of BHAC, idarubicin, and 6-TG, are comparable to that of the cytarabine-based chemotherapy for childhood AML.

Purpose:To characterize the pathogens and their antibiotic susceptibilities in more than 24-month-old children with urinary tract infection (UTI) and to study the Escherichia coli antimicrobial susceptibility trend. Methods:We retrospectively reviewed the record of more than 24-month-old children with UTI between January 2003 and December 2008. Positive results for 1 bacterial species with a colony count of ≥10 CFU/mL was considered statistically significant. We analyzed uropathogens and their antibiotic susceptibilities. To investigate E. coli antibiotic susceptibility trend, we compared 2 study periods (group A: 2003-2005 versus group B: 2006-2008) using the chi-square test for trend. Results:In all, 63 bacterial isolates were identified in children with febrile UTI. The most common pathogen was E. coli (77.8%). There was no difference in the resistance patterns of uropathogens during the 2 study periods (P>0.05). Antibiotic susceptibility of the E. coli isolates to aztreonam, cefotetan, cefotaxime, ceftriaxone, cefepime, amikacin, and imipenem was >90% to trimethoprim/sulfamethoxazol, 49% and to ampicillin and ampicillin/sulbactam, 20-25%. Over the 2 study period, the E. coli susceptibilities to most antibiotics did not change: the susceptibility to cefuroxime increased from 74.1% to 95.5% (P=0.046) and that to ciprofloxacin increased from 59.3% to 86.4% (P=0.039). Conclusion:Empirical treatment with trimethoprim/sulfamethoxazole, ampicillin, and ampicillin/sulbactam alone appeared to be insufficient in childhood UTI because of the high resistance of E. coli and other gram-negative uropathogens. Antibiotics for empirical therapy should be selected based on the sensitivity and resistance pattern of uropathogens found in a particular region.

Purpose : This study aimed to determine the frequencies of CD4＋CD25＋ T cells in donor graft and peripheral blood CD4＋CD25＋ T cells in recipients after hematopoietic stem cell transplantation (HSCT) and their association with graft-versus-host disease (GVHD). Methods : Seventeen children who underwent HSCT were investigated. CD4＋CD25＋ T cells in samples from donor grafts and recipient peripheral blood were assessed by flow cytometry at 1 and 3 months after transplantation. Results : CD4＋CD25＋ T cell frequencies in the grafts showed no significant difference between patients with and without acute GVHD (0.90% vs. 1.06%, P=0.62). Absolute CD4＋CD25＋ T cell number in grafts were lower in patients with acute GVHD than in those without acute GVHD (6.18×105/kg vs. 25.85×105/kg, P=0.09). Patients without acute GVHD showed a significant decrease in peripheral blood CD4＋CD25＋ T cell percentage at 3 months compared to those at 1 month after HSCT (2.11% vs. 1.43%, P=0.028). However, in patients with acute GVHD, CD4＋CD25＋ T cell percentage at 3 months was not different from the corresponding percentage at 1 month after HSCT (2.47% vs. 2.30%, P=0.5). Conclusion : The effect of frequencies of CD4＋CD25＋ T cells in donor grafts on acute GVHD after HSCT could not be identified, and the majority of peripheral blood CD4＋CD25＋ T cells in patients who underwent HSCT may be activated T cells related to acute GVHD rather than regulatory T cells. Further studies with additional markers for regulatory T cells are needed to validate our results.

Purpose : In this study, we retrospectively analyzed the clinical outcomes of patients who underwent allogeneic hematopoietic stem cell transplantation (HSCT) grafted from HLA-matched parents. Methods : Seven children with acute leukemia (4 acute lymphoblastic leukemia, 3 acute myeloid leukemia) in first complete remission received allogeneic HSCT from their respective parents at the St. Marys Hospital between April, 1999 and October, 2005. The median age of patients at transplantation was 5 years (range, 1-11 years; 2 male, 5 female) and the median age of donors was 35 years (range, 30-41 years; 5 male, 2 female). We investigated the clinical outcomes such as engraftment, acute and chronic graft-versus-host disease (GVHD), transplant-related morbidity and mortality, relapse and survival. Results : Median time from transplantation to last follow-up was 69.5 months (range, 18.8-96.5 months). All patients were successfully engrafted, with a median time of 11 days (range, 10-16 days) and 26 days (range, 13-39 days) for neutrophil and platelet recovery, respectively. Grade II acute GVHD occurred in 3, and grade III acute GVHD in 1 of 7 recipients. Extensive chronic GVHD developed in 2, and limited chronic GVHD in 1 of 7 recipients. Death from transplant-related complications occurred in 1, and relapse occurred in 1 of 7 recipients. Estimated 5-year overall survival was 83±15%. Conclusion : The clinical outcomes of recipients who underwent HSCT from HLA-matched parents were comparable to those of patients who received HSCT grafted from HLA-matched sibling donors in childhood leukemia. HLA typing of parents, as well as siblings will increase the likelihood of finding an HLA-matched family donor for patients who need HSCT.

Purpose: The role of allogeneic stem cell transplantation (SCT) from unrelated donor for childhood acute myeloid leukemia (AML) in first remission remains unclear because of its high transplant-related mortality. In this study, we investigated the role of SCT from high-resolution HLA-matched unrelated donor for childhood acute myeloid leukemia in first remission by comparing the outcomes of SCT from HLA-identical sibling donors and high-resolution HLA-matched unrelated donors. Patients and Method: Between June 2002 and August 2005, 40 children who have AML in first complete remission received SCT at Saint Mary’s hospital in Korea. In SCT from 17 sibling donors (M-SBMT), low-resolution typing (two-digit level) for HLA-A, -B, -C and -DRB1 was performed and all of donor-recipient pairs were 8/8 matches. Unrelated donors and recipients were typed for HLA-A, -B, and -DRB1 by high-resolution molecular typing (four-digit level). Of the 23 unrelated donor-recipients pairs, 14 were 6/6 matches (M-UBMT) and 9 were 5/6 matches (MM-UBMT). Conditioning regimen consisted of busulfan i.v. and cyclophosphamide. All patients received cyclosporin A and short-course methotrexate for GVHD prophylaxis. Results: Of the 40 patients, 27 survived event-free with a median follow-up of 38 months (range, 23–57 months). Median times for neutrophil engraftment were 12 days(range, 11–15 days), 11 days(range, 10–16 days) and 12 days(range, 11–15 days) for M-SBMT, M-UBMT and MM-UBMT, respectively. The number of Grade II-IV acute GVHD was 5 (29%), 7 (50%) and 6 (67%) in the M-SBMT, M-UBMT and MM-UBMT, respectively. One patient in MM-UBMT developed grade III acute GVHD. The number of patients who developed chronic GVHD was 2, 6 and 4 in the M-SBMT, M-UBMT and MM-UBMT,respectively. The number of relapsed patients after SCT was 3, 2 and 1 in the M-SBMT, M-UBMT and MM-UBMT and the number of non-relapse death was 2, 2 and 4, respectively. The Kaplan-Meier estimates for event-free survival at 3 years were 70 ± 11%, 71 ± 12% and 44 ± 17% for M-SBMT, M-UBMT and MM-UBMT, respectively without statistical significance (p=0.38). The Kaplan-Meier estimates for overall survival at 3 years were 76 ± 10%, 71 ± 12% and 44 ± 17% for M-SBMT, M-UBMT and MM-UBMT,respectively (p=0.25). Conclusion: In this study, we showed the outcome of SCT from high-resolpution HLA-matched unrelated donors was comparable with that of SCT from HLA-identical sibling donors in childhood AML in first remission. Therefore, high-resolution HLA-matched unrelated SCT might be recommended in patients without HLA-matched sibling donor in childhood AML in first remission.

Purpose : Due to its high potency against leukemic blasts, our institution has opted for the use of dexamethasone during acute lymphoblastic leukemia (ALL) remission induction, but in our most recent treatment protocol, CMCPL-2005, we shortened the length of steroid treatment from 4 to 3 weeks. We compared both the rates of remission induction and significant complications observed during induction with CMCPL-2005, with those noted for our previous protocol, CMCPL-2001. Methods : We retrospectively reviewed the records of patients diagnosed with ALL from January, 2001 to December, 2006 at the Department of Pediatrics, St. Mary's Hospital, the Catholic University of Korea. Data concerning age, sex, WBC count at diagnosis, immunophenotype, cytogenetic traits, and risk group were collected for each patient. Results of remission induction treatment were compared between the two patient groups. Infection and other major complications resulting from treatment were investigated according to NCI toxicity criteria. Results : A total of 141 and 88 patients received remission induction under CMCPL-2001 and CMCPL-2005 respectively. In the CMCPL-2001 group, 136 (96%) achieved complete remission while 82 (93%) achieved CR in the CMCPL-2005 group. Patients in the CMCPL-2005 group were more likely to undergo remission induction without experiencing major complications. However, with regards to steroid related toxicities such as infection, no significant differences were noted. Conclusion : We shortened the length of steroid administration from four to three weeks, yet found the remission induction rate to be comparable to that of our previous regimen. However, rates of steroid related toxicities such as infectious complications remain unchanged despite shortened exposure to dexamethasone.

Purpose: In this study, we analyzed the short term change of thyroid function, incidence and risk factors of thyroid dysfunction soon after allogeneic hematopoietic stem cell transplantation(HSCT) in children. Method: We enrolled 80 pediatric patients following allogeneic HSCT, at the Catholic HSCT center between January, 2004 and February, 2006. Serum TSH(thyroid stimulating hormone), total serum thyroxine, total serum triiodothyronine levels were systematically measured in 80 patients before and 1 month, 6 month and 12 month after HSCT. Results: Thyroid function was statistically decreased at 1 month after HSCT( P < 0.001). Thyroid dysfunction at 1 month was observed in 43(54%) of 80 patients, 31(39%) of whom presented with euthyroid sick syndrome. Thyroid dysfunction was normalized within 1 year after HSCT(Table). In univariate analysis, malignant disease and presence of acute GvHD(grade ≥II) were risk factors for euthyroid sick syndrome( P = 0.04, 0.01). In multivariate analysis, we could not detect an independent risk factor for euthyroid sick syndrome( P = 0.19, 0.06). Conclusion: The present study suggests that the incidence of thyroid dysfunction is high after allogeneic HSCT. Therefore, regular monitoring of thyroid hormone levels after HSCT is required. | | Pre HSCT | Post HSCT 1 month | Post HSCT 6 month | Post HSCT 12 month | |:-----------:| --------- | ----------------- | ----------------- | ------------------ | | T3 (ng/dL) | 161 ± 27 | 100 ± 35 | 123 ± 35 | 124 ± 35 | | T4 (μg/dL) | 8.7 ± 1.6 | 6.5 ± 2.4 | 7.3 ± 1.8 | 7.7 ± 1.6 | | TSH (mIU/L) | 2.9 ± 1.9 | 1.2 ± 1.3 | 2.4 ± 1.8 | 2.1 ± 1.3 | Short-term changes in thyroid function after HSCT

Background: Several approaches using antibodies to CD33 in the treatment of relapsed or refractory AML have been attempted, including the use of gemtuzumab ozogamicin. We decided to conduct a pilot study in pediatric patients with CD33 positive relapsed or refractory AML of targeted immunotherapy with GO combined with FLAG-IDA, to determine whether this combination would be effective and well tolerated. Patients and Methods: Eligibility criteria included CD33 positive relapsed or refractory AML and baseline serum bilirubin and creatinine ≤ 1.5 mg/ml. The patients received GO on day 1 of the course. FLAG-IDA consists of idarubicin, fludarabine, cytarabine, and G-CSF. Toxicity was assessed using the National Cancer Institute common toxicity criteria. Complete response (CR) was defined as less than 5% blasts on a bone marrow aspirate of adequate cellularity showing evidence of trilineage regeneration with a peripheral ANC to 1.0×109/l and platelet count to 100×109l. CRp was defined as a response with incomplete platelet recovery but platelet transfusion independence. Overall response (OR) was calculated by adding CR and CRp rates. Results: Between September 2005 and January 2006, five patients received GO with FLAG-IDA up to 3 infusions. All patients received GO doses of 3 mg/m2 for induction which reduced to 0.75–1.25 mg/m2 for consolidation. Median age was 9.7 (3.3–16.1) years and there were 4 boys and 1 girl. The median interval between initial diagnosis and GO administration was 1.2 (0.5–3.1) years. Three patients were relapsed state after stem cell transplantations (2 UCBT, 1 sibling BMT), 1 patient was refractory state and 1 patient was relapsed state after chemotherapy. Most common infusion-related reactions were fever and transient elevations of liver transaminases and bilirubin were noted, but VOD was not observed. The median number of days required to reach an ANC of more than 0.5×109/l were 30 (21–51) days and spontaneous platelet recovery to more than 20×109/l was 48 (9–60) days. Overall response rates were 80% and 1 patient was refractory state and 1 patient died of TMA after subsequent UBMT. Conclusion: GO with FLAG-IDA was a valuable treatment option for children with relapsed or refractory CD33 positive AML. No cases of VOD occurred in our series, even after SCT in two responders. GO with FLAG-IDA was well tolerated, even during repeated infusions. Treatment responses and outcomes UPN Course ANC, recovery 0.5×109/l, 1×109/l (d) PLT, revocery 20×109/l, 100×109/l (d) Response Further Treatment Treatment outcomes 1 1 37, 41 49, NR CRp None Alive with disease free (342 days) 2 48, 51 60, NR CRp 3 51, 58 51, 143 CR 2 1 30, 31 35, 40 CRp UBMT Died of TMA (251 days) 2 33, 35 48, NR CR 3 1 21, 24* 9, 16* CR DLI Alive with disease free (289 days) 4 1 31, 31 NR, NR Refractory Supportive treatment Alive with refractory AML(251 days) 2 27, NR NR, NR Refractory 5 1 24, 25 28, 31 CR 2nd UCBT Alive with disease free (214 days) 2 23, 24 26, 27 CR

Purpose:In this study, we analyzed the short term changes of thyroid function, incidence and risk factors of thyroid dysfunction soon after allogeneic hematopoietic stem cell transplantation (HSCT) in children. Methods:We enrolled 80 pediatric patients following allogeneic HSCT, at the Catholic HSCT center between January, 2004 and February, 2006. Serum TSH (thyroid stimulating hormone), total serum thyroxine and total serum triiodothyronine levels were systematically measured in 80 patients before the HSCT, and at 1 month, 6 months and 12 months after HSCT. Results:Thyroid function statistically decreased at 1 month after HSCT(P