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Case Report
Unrelated stem cell transplantation after reduced-intensity conditioning plus rituximab for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with CNS involvement
Hee Jo Baek, Hoon Kook, Dong Kyun Han, Min-Cheol Lee, Tae Woong Jeong, Tai Ju Hwang
Clin Exp Pediatr. 2009;52(6):725-729.   Published online June 15, 2009
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) with central nervous system (CNS) involvement is usually fatal unless stem cell transplant (SCT) is offered. However, SCT with conventional intensity conditioning is associated with high transplant-related mortality. We describe our experience with unrelated SCTs after reduced-intensity conditioning (RIC) for patients with EBV-HLH with progressive CNS disease. This approach was associated with minimal toxicities and...
Original Article
The Clinical Characteristics and Gene Analysis of Korean Gaucher Disease with Central Nervous System Involvement
Hoi Seok Choi, Sung Hwan Kim, Hiroyuki Ida, Hyun Ju Kim
Clin Exp Pediatr. 2000;43(5):611-618.   Published online May 15, 2000
Purpose : We performed the study to determine the clinical characteristics and natural history of Gaucher disease with CNS involvement in Korea as well as to determine whether gene analysis would be helpful in the identification of neuronopathic Gaucher disease. Methods : We described the initial symptom, clinical manifestations, age of onset, clinical neurophysiologic findings and brain MRI findings from the...
Case Report
A Case of Ectopic CD7(+) Congenital Monocytic Leukemia
Na Yeon Kim, Dong Seok Lee, Jin Hwa Jeong, Sung Min Cho, Doo Kwun Kim, Sung Min Choi, Woo Taek Kim, Kyung Im Ha
Clin Exp Pediatr. 1999;42(7):1003-1007.   Published online July 15, 1999
Congenital acute leukemia is a rare disorder with approximately 200 cases reported. It is defined as a childhood leukemia occurring at birth or before 1 month of age at a rate of 1%. Acute leukemias are generally classified according to morphology, cytochemistry and cell surface marker expression. Most leukemias conform to an ordered lineage-specific pattern of gene expression, but a...
A case of Incontinentia Pigmenti.
Wan Seob kim, Yong Woo Choi, Myung Ho Lee, Chul Wan Lim
Clin Exp Pediatr. 1985;28(9):940-944.   Published online September 30, 1985
A case of incontinentia pigmenti with CNS involvement was presented. And literatures have been reviewed. The patient was a 7 month-old female had been affected with numerous dark brownishinear streaks, irregular macules, and splashes of brown to slate gray pigmentation on the entire body, especially both trunk, axillary, and extremity. The chief complaint was severe vomiting and comatous mental state for...
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