Renal transplantation is the treatment of choice for children with end-stage renal disease. The outcome of pediatric kidney transplantation has improved dramatically in recent years, with lower acute rejection rates, superior graft survival, and low mortality. These improvements have allowed increased attention to other aspects of care for long-term survivors. Taking this into consideration, this review article will focus on... |
Cardiopulmonary arrest has long been accepted as an unquestionable definition of death. An advent of cardiopulmonary resuscitation and artificial ventilation along with the development of organ transplantation has prompted the emergence of the concept of brain death. The criteria for brain death are based mainly on the clinical examination of coma, apnea and total loss of brain stem function. Although organ transplantation by... |
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 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,... |
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... |
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,... |
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... |
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 (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 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... |
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... |
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... |
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... |
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... |
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.... |
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... |
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... |
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,... |
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... |
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.... |
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... |
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 :... |
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,... |
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... |
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... |
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,... |
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 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... |
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... |
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