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Hemophagocytic Syndrome Presenting as Severe Acute Hepatitis (중증 급성 간염으로 발현한 혈구탐식증후군에 관한 연구)

  • Ryu, Jeong Min;Chang, Soo Hee;Kim, Joon Sung;Lee, Joo Hoon;Lee, Mi Jeong;Park, Kie Young;Kim, Kyung Mo;Seo, Jong Jin;Moon, Hyung Nam;Ghim, Thad;Chi, Hyun Sook
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.8 no.2
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    • pp.213-221
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    • 2005
  • Purpose: Hemophagocytic syndrome (HPS) is characterized by persistent high fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, and/or hypofibrinogenemia. Hepatic manifestations including overt hepatic failure and fulminant hepatitis are common in HPS. Liver transplantation (LT) should be considered in a case of fulminant hepatitis by other than HPS, but LT is contraindicated and complete cure is possible by chemotherapy in HPS. Therefore, we conducted this study to define the characteristics of HPS presenting as severe acute hepatitis. Methods: Among the total of 23 patients diagnosed as HPS by bone marrow examination between 1994 and 2005 in Asan Medical Center, 11 cases presented as severe acute hepatitis were enrolled in this study. We analyzed the clinical features, laboratory findings and outcome retrospectively. Results: Seven (64%) of the 11 children with HPS and hepatitis were referred to pediatric gastroenterologist at first. The mean age of onset was 50 months. There was no case with family history of primary HPS. Epstein-Barr virus was positive in 4, and herpes Simplex virus was positive simultaneously in 1 case. As the presenting symptoms and signs, fever was present in 10, hepatosplenomagaly was noted in all and jaundice in 10. Anemia was observed in 10, thrombocytopenia in 10, leukopenia in 8, hypertriglyceridemia in 9, hypofibrinogenemia in 8 and hyperferritinemia in 7 cases, respectively. Nine children received chemotherapy including etopside. The overall mortality rate was 72% (8/11). Conclusion: HPS, which needs chemotherapy, should be considered as a cause of severe acute hepatitis especially when accompanied with prolonged high fever and cytopenias.

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The Results of Radiation Therapy in Locally Advanced Non-Small Cell Lung Cancer (국소적으로 진행된 비소세포폐암의 방사선치료 성적)

  • Kim Mi Sook;Yoo Seoung Yul;Cho Chul Koo;Yoo Hyung Joon;Kim Jae Young;Shim Jae Won;Lee Choon Taek;Kang Yoon Koo;Kim Tae You
    • Radiation Oncology Journal
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    • v.15 no.3
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    • pp.233-241
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    • 1997
  • Purpose : This study was done to evaluate the survival rate and Prognostic factors of patients with inoperable non-small cell lung cancer(NSCLC) treated with radiation therapy. Materials and Methods A retrospective analysis was undertaken of 62 Patients who had inoperable NSCLC treated with radiation therapy from January 1991 through December 1993. According to AJCC slaging, stage IIIA was 14 patients and stage IIIB was 48 patients. Forty Gy to 70.2Gy to the primary tumor site was delivered with daily fractions of 1.8Gy or 2Gy, 5days per week. Thirty-seven patients received neoadjuvant chemotherapy. Results : Complete, partial and no response to radiation therapy were 3 patients, 34 Patients and 25 patients, respectively The median survival period of all patients was 11 month. One rear survival rate, 2 year survival rateand 5 rear survival rate for all patients were 45.0%, 14.3%, and 6.0% respectively The median survival period was 6.5 months in stage IIIA and 13 months in stage IIIB. One year survival rates were 28.6% in stage IIIA and 50.3% in stage IIIB In univariaite analysis, prognostic factors affecting survival were T-s1aging, AJCC staging, and response after radiation therapy (P<0.05) . Pretreatment peformance status affected survival but was not statistically significant (0.050.1). In multivariate analysis, Pathology and response to radiation therapy are independently significant prognostic factor. T stage was marginally significant (P=0.0809). During follow-up duration, distant metastasis developed in 20 patients-bone metastasis in 10 patients, brain metastasis in 3 patients, liver mentastasis in 3 patients, contralateral lung metastasis in 1 patients and multiple metastases in 3 patients. Conclusion : Conventional radiotherapy alone or combined chemoradiotherapy are unlikely to achieve long term survival in patients with NSCLC. Surgery after concurrent chemoradiotherapy is Ivied to improve the local control in our hospital

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Results of Radiation Alone Versus Neoadjuvant Chemotherapy and Radiation in Locally Advanced Stage of Uterine Cervical Cancer (진행된 자궁경부암에서 방사선치료 단독과 항암 화학요법 및 방사선치료 병용요법의 결과)

  • Kim, Jin-Hee;Choi, Tae-Jin;Kim, Ok-Bae
    • Radiation Oncology Journal
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    • v.15 no.3
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    • pp.255-262
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    • 1997
  • Purpose : This is retrospective study to compare the results of radiation therapy alone and neoadjuvant chemotherapy and radiation in advanced stage of uterine cervical cancer. Materials and Methods : Seventy-six Patients who were treated with definitive radiation therapy for locally advanced cervical cacinoma between June 1988 and December 1993 at the department of radiation oncology, Keimyung University Dong-san Hospital. Thirty six patients were treated with radiation therapy alone and forty patients were treated with cisplatin based neoadjuvant chemotherapy and radiation therapy. According to FIGO staging system. there were 48 patients in stage IIb, 3 patients in stage IIIa, 23 patients in stage lIIb and two patients in stage IVa with median age of 53 years old. Follow-up periods ranged from 7 to 95 months with median 58 months. Results : Complete response (CR) rate were $86.1\%$ in radiation alone group and $80\%$ in chemoradiation group. There was no statistical difference in CR rate between the two groups. Overall five-year survival rate was $67.3\%$. According to stage, overall five-rear survival rates were $74\%$ in stage IIb, $66.7\%$ in stage IIIa, $49.8\%$ in stage IIb, $50\%$ in stage IVa. According to treatment modality overall five year survival rates were $74.1\%$ in radiation alone and $61.4\%$ in chemoradiation group (P=0.4) Five rear local failure free survival rates were $71.5\%$ in radiation alone group and $60\%$ in chemoradiation group (P=0.17). Five year distant metastasis free survival rates were $80.7\%$ in radiation aione group and $89.9\%$ in chemoradiation group (P=0.42). Bone marrow suppression (more than noted in 3 cases of radiaion alone group and 1 case of chemoradiation group. Grade II retal complication was noted in 5 patients of radiation group and 4 patients In chemoradiation group. Bowel obstruction treated with conservative treatment (1 patient) and bowel perforation treated with surgery (1 patient) were noted in radiation alone group. There was no statistical difference in complication between two groups. Conclusion : There was no statistical difference in survival, failure and complication between neoadjuvant chemotherapy and radiation versus radiation alone in locally advanced uterine cervical carcinoma.

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Results of MRI Evaluation for the Fatty Masses (지방 종괴의 진단에 대한 MRI의 판별 능력)

  • Seo, Jae-Sung;Ahn, Jong-Chul;Kim, Jeong-Rae;Choi, Jun-Hyuk;Cho, Kil-Ho;Shin, Duk-Seop
    • The Journal of the Korean bone and joint tumor society
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    • v.11 no.1
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    • pp.25-31
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    • 2005
  • Purpose: This study was designed to know the usefulness of the MRI to distinguish lipoma and well differentiated liposarcoma (WDL). Materials and methods: 47 lipomatous tumors with MRI were reviewed among the 107 lipomatous tumors operated in our department. MRI examinations and their corresponding pathology reports were compared to determine sensitivity, specificity, diagnostic ability, positive predictable value and negative predictable value. Statistical analysis was performed to know the relationship between malignancy of the tumor (WDL) with the age and gender of the patients, and location, depth, size and the enhancement of tumors in MRI. Results: Among 28 lipoma in MRI examinations, 26 were proved as lipoma in pathology, and only 6 were WDL from 19 suspicious lesions in MRI, and others were proved as lipoma variants mostly. The varieties of lipoma variants were fibrolipoma, angiolipoma, spindle cell lipoma, lipoblastoma and angiomyolipoma. The sensitivity, specificity, diagnostic ability, positive predictable value and negative predictable value of MRI were 100%, 68 %, 72%, 31% and 100% in WDL, and 90%, 89%, 89%, 93% and 84% in lipoma. Among the variants to distinguish WDL and lipoma, the size of tumor and enhancement in MRI were significant statistically (p<0.05). Conclusion: MRI was highly sensitive in detection of WDL and highly specific in detection of simple lipoma. The size of tumor and enhancement in MRI were significant variants to distinguish WDL and lipoma. When MRI finding is non-specific, it is more likely to represent one of lipoma variants.

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Eight cases of incidentally diagnosed as subclinical rickets (우연히 발견된 무증상 구루병 8예)

  • Seo, Ji-Young;Kim, Curie;Lee, Hee-Woo;Ahn, Young-Min
    • Clinical and Experimental Pediatrics
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    • v.51 no.8
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    • pp.812-819
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    • 2008
  • Purpose : Vitamin D plays a key role in bone mineralization of the skeleton and vitamin D deficiency can lead to rickets. It is well known that vitamin D deficiency is common in breast fed infants. Of these patients, clinically, some have no signs of rickets, but laboratory and radiographic findings are diagnostic for vitamin D deficiency rickets (subclinical vitamin D deficiency rickets). The purpose of this study is to clarify current causes and ways to prevent this disease. Methods : We reviewed the clinical and laboratory characteristics of children who were incidentally diagnosed as subclinical rickets during treatment of other disease such as pneumonia, gastroenteritis, urinary tract infection at Eulji Hospital, Seoul, Korea from March, 2003 to July 2007. Results : Eight patients (six boys and two girls) were diagnosed with subclinical vitamin D deficiency rickets. The mean age of the patients was $12.6{\pm}5.8months$, and they were diagnosed from January to July. The associated diseases were pneumonia, urinary tract infection, acute gastroenteritis, and iron deficiency anemia. All patients were breast-fed. Two showed growth failure. The mean serum alkaline phosphatase was $1995.8{\pm}739.5IU/L$, the mean calcium count was $9.5{\pm}0.6mg/dL$, and the mean phosphorus content was $3.6{\pm}1.5mg/dL$. The mean intact parathyroid hormone was $214.8{\pm}155.9pg/mL$ (reference range, 9-65), the mean 1,25-dihydroxyvitamin D was $82.4{\pm}49.3pg/mL$ (reference range, 2070), and the mean 25-hydroxyvitamin D was $29.6{\pm}10.6ng/mL$ (reference range, 1030). A radiographic examination showed cupping, fraying, and flaring of metaphyses in all patients. Six patients were administered calcitriol (400 IU/day) for three months. A consequent radiographic and laboratory examination showed improvement. The first two patients were initially diagnosed with metaphyseal dysplasia, without the detection of vitamin D deficiency and they spontaneously improved without vitamin D supplements. However, two years later, they showed mild scoliosis and metaphyseal dysplasia, respectively. Conclusion : Breast-feeding without supplementation involves high risk of vitamin D deficiency. Some infants may also develop rickets; therefore, such groups should be considered for vitamin D supplementation.

Comparison of Single-Incision, Single-Bundle Posterior Cruciate Ligament Reconstruction Using Allograft Tendon (동종건을 이용한 단일 절개, 단일 다발 후방십자인대 재건술의 비교)

  • Kyung, Hee-Soo;Cheon, Sang-Ho;Park, Kyung-Hyun;Jung, Jae-Wook
    • Journal of the Korean Arthroscopy Society
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    • v.16 no.2
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    • pp.107-113
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    • 2012
  • Purpose: The purpose of this study was to evaluate the posterior cruciate ligament (PCL) reconstruction with single bundle, single-incision technique using Achilles tendon and tibialis anterior allograft with ligament remnant preservation. Materials and Methods: Twenty six patients underwent PCL reconstruction was included. There were 21 males and 5 females. Mean age was 32 years. Used graft was a fresh frozen Achilles tendon allograft (group I, 14 cases) and tibialis anterior allograft (group II, 12 cases). Arthroscopic PCL reconstruction was performed using transtibial, single-incision and single bundle technique with remnant preserving as possible. For clinical evaluation, range of motion, posterior drawer test, Lysholm score, Tegner activity scale, International Knee Documentation Committee (IKDC) grade and posterior stress radiograph were used. The mean follow-up period was 21.6 months (12-40 months). Associated injuries were 5 medial collateral ligament injuries, which were treated by conservative method. Results: Range of motion (ROM) was returned to normal range in 24 cases, but ROM deficit under $10^{\circ}$ flexion was 2 cases at final follow-up period. Preoperative posterior drawer test was 17 cases in grade II and 9 cases in grade III. At final follow-up 13 cases returned within normal grade, 7 cases grade I and 6 cases grade II posterior instability. Lysholm mean score was improved from preoperatively 62 to 90 at final follow-up period. Tegner activity mean scale improved from preoperatively 3.5 to 5.6 at final follow-up period. IDKC grade was grade A was 3 cases, grade B 17 cases, grade C 6 cases. In posterior stress radiograph, posterior displacement was improved from mean 12 mm preoperative to 4.5 mm at final follow-up. There were no statistical differences between two groups in clinical evaluations. There were two cases of re-rupture of graft at the bone-tendon junction in group I. Conclusion: We had successful results of PCL reconstruction with single-incision, single bundle technique using Achilles and tibialis anterior allograft without difference between two groups in patients with PCL injury. There were more re-rupture of graft in Achilles tendon group.

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Postirradiation Changes of White Blood Cells and Lymphocyte Subpopulations in Cancer Patients (암환자의 방사선치료에서 흉부 및 전골반강 조사직후 백혈구 및 림프구아헝 변화에 대한 연구)

  • Ahn, Sung-Ja;Chung, Woong-Ki;Nam, Taek-Keun;Nah, Byung-Sik;Noh, Young-Hee
    • Radiation Oncology Journal
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    • v.14 no.1
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    • pp.53-59
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    • 1996
  • Purpose : Radiation-induced alteration in the immune function is well known phenomenon in cancer patients. Our purpose is to evaluate the extent of immune suppression immediately after mediastinal or pelvic irradiation, which include significant volume of active bone marrow in adults. Materials and Methods'48 cancer patients with mediastinal(N=29) and pelvic irradiation(N=19) were the basis of this analysis. Age ranged from 36 to 76 and mean and median value was 57 years, respectively Sex ratio was 1.3(M: F=27/21). The immunological parameters were the complete blood cell(CBC) with differential cell(D/C) count, T cell subset(CD3, CD4, CD8 CDl9), NK cell test(CDl6, CD56), and serum immunoglobulin(IgG, IgA, IgM) level. Results : The mean value of white blood cell(WBC) was reduced from 7017 to 4470 after irradiation(p=0.0000). In the differential count, the number of lymphocyte, neutrophil, and basophil was markedly reduced with statistical significance(p<0.01) and the number of monocyte was not changed and, on the contrary, that of eosinophil was increased by irradiation. In the lymphocyte subpopulation analysis, the number of all subpopulations, CD3(T cell), CD4(helper T cell), CD8(suppressor T cell), CDl6(NK cell), CDl9(B, cell) was reduced with statistical significance. The mean ratio of CD4 to CD8 in all patients was 1.09 initially and reduced to 0.99 after radiotherapy(p=0.34) , but the proportional percentage of all subpopulations was not changed except CD19(B cell) after irradiation. In the immunoglobulin study, initial values of Ig G, Ig A, and Ig M were relatively above the normal range and the only Ig M was statistically significantly reduced after radiotherapy(p=0.02). Conclusion : Mediastinal and pelvic irradiation resulted in remarkable suppression of lymphocyte count in contrast to the relatively good preservation of other components of white blood cells. But the further study on the functional changes of lymphocyte after radiotherapy may be necessary to conclude the effects of the radiation on the immunity of the cancer Patients.

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Hyperfractionation Radiation Therapy in Advanced Head and Neck Cancer (진행된 두경부암에서 다분할 방사선치료)

  • Kim, Jin-Hee;Ye, Ji-Won
    • Radiation Oncology Journal
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    • v.21 no.2
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    • pp.112-117
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    • 2003
  • Purpose: The effects of hypefractionation radiation therapy, such as the failure pattern and survival, on the treatment results in advanced stage head and neck cancer were studied. Materials and Methods: Between September 1990 and October 1998, 24 patients with advanced stage (III, IV) head and neck cancers, were treated using hyperfractionation radiation therapy in the Department of Radiation Oncology at the Keimyung University Dongsan Medical Center. The male to female ratio was 7 : 1, and the age range from 38 to 71 years with the median of 56 years. With regard to the TNM stage, 11 patients were stage III and 13 were stage IV. The sites of primary cancer were the nasopharynx in six, the hypopharynx in 6, the larynx in five, the oropharynx in three, the maxillary sinus in three, and the oral cavity in one patient. The radiotherapy was delivered by 6 MV X-ray, with a fraction size of 1.2 Gy at two fractions a day, with at least 6 hours inter-fractional interval. The mean total radiation doses was 72 Gy, (ranging from 64.4 to 75.8 Gy). Follow-up periods ranged between 3 and 136 months, with the median of 52 months. Results: The overall survival rates at 3 and 5 years in all patients were 66.7$\%$, and 52.4$\%$. The disease-free survival rates at 3 and S years (3YDFS, 5YDFS) in all patients were 66.7$\%$ and 47.6$\%$. The 3YDFS and 5YDFS in stage III patients were 81.8$\%$ and 63.6$\%$, and those in stage IV patients were 53.8$\%$ and 32.3$\%$. Ten patients were alive with no local nor distant failures at the time of analyses. Six patients (25$\%$) died due to distant metastasis and 12.5$\%$ died due to local failure. Distant metastasis was the major cause of failure, but 2 patients died due to unknown failures and 3 of other diseases. The distant metastasis sites were the lung (3 patients), the bone (1 patient), and the liver (2 patients). One patient died of second esophageal cancer. There were no severe late complications, with the exception of 1 osteo-radionecrosis of the mandible 58 months after treatment. Conclusion: Although this study was peformed on small patients group, we considered hypefractionated radiation therapy for the treatment of advanced stage head and neck cancer might improve the disease free survival and decrease the local failure with no increase in late complications despite of the slight Increase in acute complications.

Incidence of Osteoporosis in Patients with COPD According to Different Methods of Glucocorticoid Administration (만성페쇄성폐질환 환자에서 스테로이드 투여 방법에 따른 골다공증의 발생빈도)

  • Lee, Yang Deok;Lee, Kang Hyu;Lee, Heung Bum;Lee, Yong Chul;Rhee, Yang Keun
    • Tuberculosis and Respiratory Diseases
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    • v.54 no.1
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    • pp.15-21
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    • 2003
  • Background : There are many risk factors for osteoporosis in patients with chronic obstructive pulmonary disease(COPD). These include smoking, a low body mass index, insufficient exercise, and the use of glucocortcoids. However, there is lack of data on the incidence of osteoporosis according to the different glucocorticoid administration methods in patients with COPD. This study compared the incidence of osteoporosis according to the different administration methods of glucocorticoid. Methods : A matched case-controlled study (gender, age, cumulative steroid dose and pack-years of smoking) was conducted. Forty-five patients with documented COPD for at least a 3 year duration and a cumulative glucocorticoid dose above 1,000 mg were enrolled in study. The patients were classified into the following three groups. First, fifteen patients received continuous inhaled glucocorticoid with intermittent oral steroids but had no admission history due to an acute exacerbation(Group I). Secondly, fifteen patients received a multiple course of oral steroids with additional inhaled glucocorticoid but had no admission history due to their acute exacerbation(Group II). Lastly, fifteen patients received intermittent oral or inhaled glucocorticoids and had an admission history due to the acute exacerbation with intravenous steroid treatment for at least 2 weeks per year(Group III). The enrolled patients had apulmonary function test and bone densitometry performed at the lumbar spine and femoral neck. Results : The patients from Group III had significantly high incidence of osteoporosis in the lumbar and femoral neck compared to Group I and Group II (p<0.01). Conclusion : The incidence of osteoporosis in patients with COPD appears to be strongly affected by the method of steroid administration. This result suggests that intravenous steroid administration is strongly associated with the risk of osteoporosis.

CMV antigenemia following pediatric hematopoietic stem cell transplantation : risk factors and outcomes (소아 조혈모세포 이식 후 거대세포 바이러스 항원혈증 발생 : 위험인자와 임상 경과)

  • Cho, Eun-Young;Park, Young-Shil;Lee, Dae-Hyung;Park, Ji Kyoung;Choi, Sangrhim;Kim, Sun Young;Jang, Pil-Sang;Lee, Dong-Gun;Chung, Nak-Gyun;Kim, Jong-Hyun;Jeong, Dae-Chul;Cho, Bin;Hur, Jae Gyun;Kang, Jin Han;Kim, Hack Ki
    • Clinical and Experimental Pediatrics
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    • v.49 no.2
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    • pp.173-180
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    • 2006
  • Purpose : Cytomegalovirus(CMV) infection still remains as a major cause of morbidity and mortality after stem cell transplantation. In this study, we analyzed the results of antigenemia-guided preemptive therapy among children with allogeneic hematopoietic stem cell transplantation to determine the incidence and risk factors associated with CMV antigenemia, and evaluated the efficacy of the CMV antigenemia based preemptive therapy. Methods : We enrolled 213 pediatric patients following allogeneic hematopoietic stem cell transplantation(HSCT), at the Catholic HSCT center between October 1998 and December 2003. Pre-emptive ganciclovir was started when more than 5 CMV Ag-positive cells were detected in matched sibling HSCT, and when any Ag-positive cells were seen in unrelated allogenic HSCT. Results : CMV antigenemia was observed in 88(41.3 percent) of 213 patients on median day 28(day 11-99). In univariated analysis, use of unrelated donors(other than siblings), age of recipient(more than 5 years at transplant) at transplantation, the presence of recipient CMV-IgG before transplantation, TBI-based conditioning regimen and the presence of acute GvHD(grade ${\geq}II$) were the risk factors for positive CMV antigenemia. In multivariate analysis, unrelated bone marrow transplantation, positive recipient CMV serology and acute GvHD(grade ${\geq}II$) were the independent risk factors for positive CMV antigenemia. Conclusion : Risk factors of CMV infection in children were CMV serostatus of the recipient, the source of stem cells, and acute graft-versus-host disease. The pre-emptive therapy based on CMV antigenemia was effective in the prevention of CMV disease.