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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...
Facial palsy as the presenting symptom of acute myeloid leukemia in children: Three cases with stem cell transplantatio
Hee Jo Baek, Dong Kyun Han, Young Ok Kim, Ic Sun Choi, Tai Ju Hwang, Hoon Kook
Clin Exp Pediatr. 2009;52(6):713-716.   Published online June 15, 2009
Facial palsy as the presenting symptom of leukemia is very rare, especially in acute myeloid leukemia. A review of the medical literature identified reports on 8 children with AML who had facial paralysis as the presenting sign. Whole brain irradiation (WBI) has been applied in most cases. We present the cases of 3 such children. Achieving a remission without WBI,...
Original Article
Distribution of CD4＋CD25＋ T cells and graft-versus-host disease in human hematopoietic stem cell transplantation
Dae Hyoung Lee, Nak Gyun Chung, Dae Chul Jeong, Bin Cho, Hack Ki Kim
Clin Exp Pediatr. 2008;51(12):1336-1341.   Published online December 15, 2008
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...
Clinical outcomes of hematopoietic stem cell transplantation from HLA-matched parental donor in childhood acute leukemia
Eun Young Cha, Moon Hee Lee, Jae Wook Lee, Young Joo Kwon, Dae Hyoung Lee, Young-Shil Park, Nak Gyun Chung, Dae Chul Jeong, Bin Cho, Hack Ki Kim
Clin Exp Pediatr. 2008;51(1):67-72.   Published online January 15, 2008
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,...
A pilot study of neuroprotection with umbilical cord blood cell transplantation for preterm very low birth weight infants
Kyu Young Chae, Kyu Hyung Lee, So-Hee Eun, Byung Min Choi, Baik-Lin Eun, Hoon-Chul Kang, Myung Jae Chey, Nam Keun Kim, Doyeun Oh
Clin Exp Pediatr. 2007;50(9):882-890.   Published online September 15, 2007
Purpose : Preterm very low birth weight infant have high rate of adverse neurodevelopmental sequale. Recently, there have been lots of reports that human umbilical cord blood transplantation ameliorates functional deficits in animal models as hypoxic ischemic injury. This pilot study was undertaken to determine the clinical efficacy and safety of autologous umbilical cord blood cell transplantation for preventing neurodevelopmental...
Review Article
Acute liver failure in children
Kyung Mo Kim
Clin Exp Pediatr. 2007;50(9):841-847.   Published online September 15, 2007
Acute liver failure (ALF) is a very rare but devastating illness in children. Specific treatment to recovery is often not available, and the underlying cause of the liver failure is often unknown and diverse especially in children. Liver transplantation has increased the chance of survival; however it needs an optimal timing to reach the best result which is not familiar...
Hematopoietic stem cell transplantation : overview for general pediatrician
Tai Ju Hwang
Clin Exp Pediatr. 2007;50(7):613-621.   Published online July 15, 2007
Hematopoietic stem cell transplantation (HSCT) has expanded and evolved substantially in the last decades to treat various malignant and nonmalignant diseases. However, the conditioning regimen can lead to transplantation related death by major organ dysfunction, severe infection and bleeding. In the allogeneic setting, graft versus host disease may also develop, making post-transplant management complex. To overcome these problems, new stem...
Aplastic anemia
Hack Ki Kim
Clin Exp Pediatr. 2007;50(6):519-523.   Published online June 15, 2007
Aplastic anemia is a rare disease, which is characterized by pancytopenia and hypocellular bone marrow without infiltration of abnormal cells or fibrosis. The incidence in Asia is higher than in the West and new cases are diagnosed at a rate of 5.1 per million pediatric populations per year in Korea. The pathophysiology is understood roughly by defective hematopoiesis, impaired bone...
Original Article
Comparison of growth and neuropsychological function after treatment for hematologic and oncologic diseases in monozygotic twins
Gi Hwan Kim, Hoon Kook, Hee Jo Baek, Dong Kyun Han, Eun Song Song, Young Kook Jo, Ic Sun Choi, Young Ok Kim, Chan Jong Kim, Young Jong Woo, Soo Jin Yang
Clin Exp Pediatr. 2007;50(2):182-189.   Published online February 15, 2007
Purpose : The primary purpose of this study was to evaluate the growth and neuropsychologic function following treatments for pediatric hematologic and oncologic diseases. Healthy monozygotic twins served as ideal controls for comparison to exclude possible confounding factors. Methods : Seven children treated with various hematologic and oncologic diseases were included in the study: acute lymphoblastic leukemia (ALL; n=2), Diamond-Blackfan anemia...
Second allogeneic hematopoietic stem cell transplantation in children to overcome graft failure or relapse after initial transplant
Dong-Yeon Kim, Do Kyun Kim, Soo Young Kim, Seok Joo Kim, Dong Gyun Han, Hee Jo Baek, Hoon Kook, Tai-Ju Hwang
Clin Exp Pediatr. 2006;49(12):1329-1339.   Published online December 15, 2006
Purpose : Failure of hematopoietic stem cell transplantation(HSCT) may be encountered in practice because of either relapse of the malignancy or dysfunction of the graft. Second HSCT may be the only option for some patients whose initial HSCT failed. Methods : From May, 1991 to December, 2004, 115 HSCTs were performed at the Pediatric Blood & Marrow Transplantation Center, Chonnam National...
Short-term follow up of thyroid function after pediatric hematopoietic stem cell transplantation
Seon-Ju Lee, Jae-Wook Lee, Dae-Hyoung Lee, Young-Joo Kwon, Young-Shil Park, Hui Sung Hwang, Sun Young Kim, Ji Kyoung Park, Pil-Sang Jang, Min-Ho Jung, Nak-Gyun Chung, Dae-Chul Jeong, Bin Cho, Hack-Ki Kim, Byung-Churl Lee
Clin Exp Pediatr. 2006;49(11):1211-1215.   Published online November 15, 2006
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...
Factors affecting hematologic recovery and infection in high-dose chemotherapy and autologous stem cell transplantation in patients with high-risk solid tumor
Jung Hyun Lee, Bo Lyun Lee, Soo Hyun Lee, Keon Hee Yoo, Ki Woong Sung, Hye Lim Jung, Eun Joo Cho, Hong Hoe Koo
Clin Exp Pediatr. 2006;49(10):1079-1085.   Published online October 15, 2006
Purpose : The purpose of this study was to evaluate factors affecting hematologic recovery and infection in high-dose chemotherapy(HDCT) and autologous stem cell transplantation(ASCT) in patients with high-risk solid tumor. Methods : From January 2004 to December 2005, 72 HDCTs and ASCTs were applied to children with high-risk solid tumor at Samsung Medical Center. Medical records of these 72 HDCTs and...
CMV antigenemia following pediatric hematopoietic stem cell transplantation : risk factors and outcomes
Eun-Young Cho, Young-Shil Park, Dae-Hyung Lee, Ji Kyoung Park, Sangrhim Choi, Sun Young Kim, Pil-Sang Jang, Dong-Gun Lee, Nak-Gyun Chung, Jong Hyun Kim, Dae-Chul Jeong, Bin Cho, Jae Gyun Hur, Jin Han Kang, Hack-Ki Kim
Clin Exp Pediatr. 2006;49(2):173-180.   Published online February 15, 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 pre- emptive 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....
Allogeneic Hematopoietic Stem Cell Transplantation in Juvenile Myelomonocytic Leukemia
Keon Hee Yoo, Dong Kil You, Soo Hyun Lee, Ki Woong Sung, Eun Joo Cho, Hong Hoe Koo
Clin Exp Pediatr. 2005;48(2):178-185.   Published online February 15, 2005
Purpose : The purpose of this study was to evaluate the outcome of children with juvenile myelomonocytic leukemia(JMML) treated with allogeneic hematopoietic stem cell transplantation(allo- HSCT). Methods : Eleven JMML patients aged 8-39 months underwent allo-HSCT. The sources of grafts were unrelated donors(n=7), HLA-matched siblings(n=3) and an HLA 1-antigen mismatched familial donor. All patients had received chemotherapy ?13-cis-retinoic acid(CRA) before...
Case Report
Isolated Intracranial Granulocytic Sarcoma as a Relapse Following Unrelated Bone Marrow Transplantation for Myelodysplastic Syndrome in a 1 Year-Old Infant
Kyung Ran Son, Hoon Kook, So Youn Kim, Hee Jo Back, Seok Joo Kim, Ha Young Noh, Mi Jeong Kim, Ic Sun Choi, Shin Jeong, Jong Hee Nam, Tai Ju Hwang
Clin Exp Pediatr. 2004;47(9):1008-1012.   Published online September 15, 2004
Isolated relapse of myeloid leukemia as a granulocytic sarcoma(GS) following allogeneic bone marrow transplantation(BMT) is very rare manifestation, and usually associated with a poor prognosis. We report a case of isolated intracranial GS in an infant with myelodysplastic syndrome(MDS) following unrelated BMT. A 7 month-old girl was diagnosed with refractory anemia with excess blasts (RAEB). During observation for a couple...
Original Article
Neurologic Complications after Hematopoietic Stem Cell Transplantation in Children
Moon Young Jeong, Young Kuk Cho, So Youn Kim, Young Ok Kim, Chan Jong Kim, Hoon Kook, Young Jong Woo, Tai Ju Hwang
Clin Exp Pediatr. 2004;47(9):978-985.   Published online September 15, 2004
Purpose : Neurologic complications(NC) after hematopoietic stem cell transplantation(HSCT) are important because of high mortality and severe sequelae. We analyzed the incidence, manifestation and risk factors of NC in 61 children undergoing HSCT. Methods : We retrospectively analyzed NC in 61 cases(42 male and 19 female, aged one to 16 years) given HSCT between 1996 and 2003 due to hematologic malignacies,...
Medical Lecture Course
Regulatory T Cells and Allogeneic Transplantation
Dae Chul Jeong
Clin Exp Pediatr. 2004;47(9):919-925.   Published online September 15, 2004
Allogeneic organ or hematopoietic stem cell transplantation(HSCT) is the treatment of choice for end-stage organ diseases or various hematologic disorders. The induction of alloantigen specific T cell tolerance and its maintenance are critical for preventing immune responses, including graft rejection or graft-versus-host disease(GVHD) in allogeneic transplantation. CD4＋ T cells are classified as immune functions : Th1 CD4＋ cells for cellular...
Original Article
Clinical Experience of 60 Pediatric Renal Transplantations at a Single Center
Sun Youn Lee, Hyewon Han, Young Seo Park, Duck Jong Han
Clin Exp Pediatr. 2004;47(1):55-65.   Published online January 15, 2004
Purpose : Renal transplantation for the management of end-stage renal disease(ESRD) in children is now the optimal treatment. We analyzed the clinical courses and characteristics of pediatric renal transplantation in Asan Medical Center. Methods : We reviewed the charts of 60 cases of renal transplantation under the age of 18, admitted to Asan Medical Center, from Oct. 1990 to May 2003....
Case Report
A Successful Transplant of HLA-3 Loci Mismatched Umbilical Cord Blood into a Patient with Acute Myeloid Leukemia from an Unrelated Donor
Byoung Chul Kwon, Kyung Mi Shin, Sung Chul Won, Chuhl Joo Lyu, Chang Hyun Yang, Hyun Ok Kim
Clin Exp Pediatr. 2002;45(7):912-916.   Published online July 15, 2002
Cord blood is a useful source of allogeneic hematopoietic stem cells for bone marrow reconstitution. The number of umbilical cord blood transplants is increasing worldwide. In this a case 15- month-old boy with acute myeloid leukemia was treated with umbilical cord blood transplant from an HLA-3 loci mismatched unrelated donor. Granulocyte recovery greater than 500/mm3 occurred at day 49, and...
Original Article
Short-term Results of Hematopoietic Stem Cell Transplantation for Children with Myelodysplastic Syndrome
Jin Lee, Soh Yeon Kim, Bin Cho, Pil Sang Jang, Nak Gyun Chung, Hack Ki Kim
Clin Exp Pediatr. 2002;45(3):370-375.   Published online March 15, 2002
Purpose : In most cases, myelodysplastic syndrome(MDS) transforms into a more aggressive state or acute myelogenous leukemia; it's prognosis is very poor. It is believed that hematopoietic stem cell transplantation(HSCT) is the only curative treatment of MDS, but available data in children are very sparse. In this report, the short term outcome of HSCT in childhood MDS was analyzed. Methods :...
Long-term Clinical Follow-up of Korean Children with Wilson Disease; Twenty Years` Experience
Jin Soo Moon, Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2001;44(2):127-138.   Published online February 15, 2001
Purpose : To investigate the clinical features of long-term follow-up, and the treatment responses in Korean children with Wilson disease(WD). Methods : The clinical data from ninety-two children with WD, diagnosed at Seoul National University Hospital from 1976 to 1999, were reviewed. Mean duration of follow-up was 5¡¾4.5 years. We analysed the patient's records based on the clinical manifestations, laboratory findings,...
Effect of Liver Transplantation in Children with Fulminant Hepatitis
Young Mee Seo, Bo Hwa Choi, Kyung Mo Kim, Kwang Min Park, Young Joo Lee, Sung Gyu Lee
Clin Exp Pediatr. 2000;43(4):535-542.   Published online April 15, 2000
Purpose : Liver transplantation(LT) is regarded as an important management option for fulminant hepatitis(FH), which is associated with considerable mortality under conservative management. The aim of this study was to evaluate the outcome of children with FH according to management. Methods : We reviewed medical records of patients presented with FH from January 1994 until April 1999. The children were...
Case Report
A Case of Ornithine Transcarbamylase Deficiency Successfully Treated with Protein Restriction and Living Related Liver Transplantation
Bong Seong Kim, Kyung Mo Kim, Han-Wook Yoo, Sung Gyu Lee
Clin Exp Pediatr. 1999;42(6):868-873.   Published online June 15, 1999
Ornithine transcarbamylase deficiency(OTCD), the most common inborn error of the urea cycle, is inherited in X-linked manner. In affected hemizygote males, OTCD manifests hyperammonemic coma that often leads to death during the newborn period. Our patient was at high risk for inborn error of urea cycle metabolism, since his two elder brothers died a few days after birth due to...
Original Article
Living Related Donor Liver Transplantation in Children : Indication and Clinical Outcome
Kyung Mo Kim, Sung gyu Lee, Young Joo Lee, Kwang Min Park, Seong Chul Kim, Hoon Bae Chun, Eun Sil Yoo, Sang Hoon Han, Kyung Suk Ko, Mon Gyu Lee, Kyu Taek Choi, Jae Kon Ko, Hyung Nam Moon
Clin Exp Pediatr. 1998;41(5):622-632.   Published online May 15, 1998
The Correlation between CD34+ Cell and Nucleated RBC Counts in Human Cord Blood
Seong Man Lee, Kyu Chan Kim, Sang Woo Kim
Clin Exp Pediatr. 1996;39(10):1422-1428.   Published online October 15, 1996
Purpose : Human umbilical cord blood as a potential source of hematopoietic stem cell for stem cell transplantation in children has recently been advocated. Clinical application of cord blood transplantation, however, requires adequate blood volume and number of stem cells. Currently, the number of stem cells in the cord blood is usually measured by flowcytometry, which requires strict quality control and high costs. Here,...
Stem Cell Transplantation in Umbilical Cord Blood(II) Characteristics of T-Lymphocyte Subpopulation in Umbilical Cord Blood
Kyung Ha Ryu, So Young Ahn
Clin Exp Pediatr. 1995;38(6):819-826.   Published online June 15, 1995
Purpose : Homatopoietic stem cells, which have the ability to undergo self-renewal as well as to develop into multipotential differentiations, have been consistantly demonsteraten in human umbilical cord blood. Recently, there have been a few studies using the umbilical cord blood as a source of stem cell transplantation. However it remains unclear whether umbilical cord blood in safe with regards to...
Stem Cell Transplantation in Umbilical Cord Blood(I) Expansion Effects of Stem Cells in Umbilical Cord Blood with Various Hematopoietic Growth Factors
Kyung Ha Ryu, Myung Hee Chung
Clin Exp Pediatr. 1995;38(5):675-684.   Published online May 15, 1995
Stem cell transplantation is used for the patients lacking the capability of production of hem-atopoitic cells. Recently, there have been a few studies using the umbilical cord blood as a sou-rce of stem cells, however it remains unclear whether umhhilical cord blood contains sufficient amount to reconstitute the patient. Our study was designed to know that umbilical cord blood is proper...
Treatment of Aplastic Anemia
Nak Gyun Chung, Kyu Jin Bhang, Woo Gun Choi, Hack Ki Kim, Kyong Su Lee, Man Kyu Yang
Clin Exp Pediatr. 1993;36(8):1059-1066.   Published online August 15, 1993
We performed this study to evaluate the therapeutic responses of bone marrow transplantation and immunosuppressive therapy for aplastic anemia in 103 cases(68 severe and 35 moderate). Among them, 15 cases with severe aplastic anemia having HLA-identical sibling donor underwent bone marrow transplantation, and cases (53 severe and 35 moderate cases) without available HLA-identical sibling donor had received immunosuppressive therapy using...
Case Report
Effect of Cultured Thymic Epithelium Transplantation in a Patients with Severe Combined Immunodeficiency
Soo Kyung Yun, Byoung Ho Cha, Woo Seung Jeoun, Dong Soo Kim
Clin Exp Pediatr. 1992;35(10):1443-1448.   Published online October 15, 1992
Severe combined immunodeficiency disease is caused by a group of genetic disease characterized by abnormal function of T and B lymphocytes. The treatment of choice for severe combined immunodeficiency disease is a HLA mached bone marrow transplant, but transplantation of cultured thymus epithelium was used to treat severe combined immunodeficiency disease in case of that mached, related bone...
A Case of Primary Hyperparathyroidism in Infancy
Mi Jung Park, Ho Seong Kim, Duk Hi Kim
Clin Exp Pediatr. 1992;35(7):1008-1013.   Published online July 15, 1992
Primary hyperparathyroidism is very rare and often fatal disease in childhood. We experienced a case in which a 4 months old male patient complaning of lethargy, hypotonia, respiratory difficulty showed typical laboratory and radiological findings compatible to primary hyperparathyroidism. He was successfully treated with total parathyroidectoimy with partial parathyroid autotrans-plantation. Thus the case of primary hyperparathyroidism in infancy is presented with...
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