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Dae Chul Jeong,
Nack Gyun Chung,
Hyoung Jin Kang,
Hong Hoe Koo,
Hoon Kook,
Soon Ki Kim,
Sun Young Kim,
Heung Sik Kim,
Hwang Min Kim,
Kyung Duk Park, [......],
Soon Yong Lee,
Young Ho Lee,
Young Tak Lim,
Yeon Jung Lim,
Hye Lim Jung,
Bin Cho, Yong Mook Choi,
Jeong Ok Hah,
Tai Ju Hwang,
Hack Ki Kim
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ABSTRACT: Aplastic anemia (AA) is a rare hematologic disease characterized by pancytopenia and hypocellular marrow. The Korean Society of Pediatric Hematology Oncology investigated retrospectively the incidence, survival, and transfusion independency according to treatment strategies in AA.
All the questionnaires were sent to members for medical records. We collected and analyzed 702 available data.
The male and female ratio was 1.2, and the median age at diagnosis was 9.3 years. The annual incidence of Korean children with AA was 5.16 per million per year. Constitutional anemia was diagnosed in 44 children. In acquired AA, causes were identified in 39 children. Severe AA (SAA) at initial diagnosis was more common than nonsevere AA. The overall survival was 47.8% with supportive care, 68.1% with immunosuppressive therapy (IST), and 81.8% with hematopoietic stem cell transplantation. In IST, response rate was 65.7%, and relapse rate after response was 54.4% within a median of 23.0 months. The factors with overall survival were severity of disease in supportive care, severity and response in IST, donor type, graft failure, and posttransplant events in hematopoietic stem cell transplantation.
Long-term outcome in AA was dependent on treatment strategies. These Korean results may help research and prospective international clinical trials for childhood AA.
Journal of Pediatric Hematology/Oncology 02/2011; 33(3):172-8. · 1.16 Impact Factor
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Ki Woong Sung,
Hyo Seop Ahn,
Bin Cho, Yong-Mook Choi,
Nack Gyun Chung,
Tai Ju Hwang,
Ho Joon Im,
Dae Chul Jeong,
Hyoung Jin Kang,
Hong Hoe Koo,
Hoon Kook,
Hack Ki Kim,
Chuhl Joo Lyu,
Jong Jin Seo,
Hee Young Shin,
Keon Hee Yoo,
Sung Chul Won,
Kun Soo Lee
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ABSTRACT: The efficacy of tandem high-dose chemotherapy and autologous stem cell rescue (HDCT/ASCR) was investigated in patients with high-risk neuroblastoma. Patients over 1 yr of age who were newly diagnosed with stage 4 neuroblastoma from January 2000 to December 2005 were enrolled in The Korean Society of Pediatric Hematology-Oncology registry. All patients who were assigned to receive HDCT/ASCR at diagnosis were retrospectively analyzed to investigate the efficacy of single or tandem HDCT/ASCR. Seventy and 71 patients were assigned to receive single or tandem HDCT/ASCR at diagnosis. Fifty-seven and 59 patients in the single or tandem HDCT group underwent single or tandem HDCT/ASCR as scheduled. Twenty-four and 38 patients in the single or tandem HDCT group remained event free with a median follow-up of 56 (24-88) months. When the survival rate was analyzed according to intent-to-treat at diagnosis, the probability of the 5-yr event-free survival+/-95% confidence intervals was higher in the tandem HDCT group than in the single HDCT group (51.2+/-12.4% vs. 31.3+/-11.5%, P=0.030). The results of the present study demonstrate that the tandem HDCT/ASCR strategy is significantly better than the single HDCT/ASCR strategy for improved survival in the treatment of high-risk neuroblastoma patients.
Journal of Korean medical science 05/2010; 25(5):691-7. · 0.84 Impact Factor
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Hee Young Shin,
Hyoung Jin Kang,
Eun Sil Park,
Hyoung Soo Choi,
Hyo Seop Ahn,
Sun Young Kim,
Nak Gyun Chung,
Hack Ki Kim,
So Youn Kim,
Hoon Kook, [......],
Kun Soo Lee,
Keon Hee Yoo,
Hong Hoe Koo,
Mee Jung Lee,
Jong Jin Seo,
Hyung Nam Moon,
Thad Ghim,
Chuhl Joo Lyu,
Won Sik Lee, Yong Mook Choi
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ABSTRACT: Hyperuricemia accompanying tumor lysis syndrome is a serious complication in neoplasia with rapid proliferation and destruction. To confirm the efficacy of recombinant urate oxidase (rasburicase) and its safety profile, a phase IV compassionate use prospective study was performed in Korean pediatric patients with hematologic malignancies.
Rasburicase was administered at 0.2 mg/kg/day once daily for 3-5 days (twice daily allowed during the first 72 hr) by intravenous route for hyperuricemia (uric acid > 7.5 mg/dl). The study period was 5 weeks and consisted of a baseline assessment within 48 hr before the administration of rasburicase, 3-5 days of assessment during treatment and a follow-up assessment at 4 weeks after its final administration.
The uric acid endpoint (< or =7.0 mg/dl) was reached in 97.3% (36/37) of the patients and uric acid levels were significantly reduced in all patients (P < 0.001). Drug related toxicities were mild and reversible without any grade 4 or serious adverse event associated with rasburicase.
This study confirms that rasburicase is a safe and effective agent for the treatment of hyperuricemia associated with hematologic malignancies in pediatric patients.
Pediatric Blood & Cancer 04/2006; 46(4):439-45. · 1.89 Impact Factor
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ABSTRACT: Extraskeletal myxoid chondrosarcoma is a rare soft-tissue sarcoma that usually occurs in deep soft tissues, especially those of the proximal extremities and limb girdles, but is rare in children. We present an unusual case of a tumor arising in the perineum and involving the bulbous urethra in a 21-month-old boy. The patient was treated with surgical excision and urethroplasty followed by combination chemotherapy.
International Journal of Urology 07/2004; 11(6):436-9. · 1.75 Impact Factor
