The event-free survival (EFS) for pediatric acute lymphoblastic leukemia (ALL) has shown remarkable improvement in the past several decades. In Korea also, a recent study showed 10-year EFS of 78.5%. Much of the improved outcome for pediatric ALL stems from the accurate identification of prognostic factors, the designation of risk group based on these factors, and treatment of appropriate duration... |
Severe aplastic anemia (SAA), a fatal disease, requires multiple transfusion, immunosuppressive therapy, and finally, hematopoietic stem cell transplantation (HSCT) as the definitive treatment. We hypothesized that iron overloading associated with multiple transfusions and HSCTrelated complications may adversely affect cardiac function. Left ventricular (LV) function was assessed in children after HSCT for SAA. Forty-six consecutive patients with a median age of 9.8... |
Hemorrhagic cystitis is a common stem cell transplantation-related complication. The incidence of early-onset hemorrhagic cystitis, which is related to the pretransplant conditioning regimen, has decreased with the concomitant use of mesna and hyperhydration. However, late-onset hemorrhagic cystitis, which is usually caused by the BK virus, continues to develop. Although the BK virus is the most common pathogenic microorganism of poststem... |
Varicella-zoster virus infection can lead to severe illness in immunocompromised patients. Further the mortality rate of disseminated varicella infection is extremely high particularly in immunocompromised children. We report a case of disseminated varicella infection in a child with acute lymphoblastic leukemia who was receiving chemotherapy, but was initially admitted with only for acute abdominal pain. The patient rapidly developed severe... |
The use of cyclosporine and mini-dose methotrexate (MTX) is a common strategy for graft-versus-host disease (GVHD) prophylaxis in allogeneic transplants. We investigated whether patients who receive fewer than the planned MTX doses are at increased risk for GVHD. The study cohort included 103 patients who received allogeneic transplants at the Department of Pediatrics of The Catholic University of Korea College of... |
Despite the established role of imatinib (IM) in chronic myelogenous leukemia (CML) in adults, there are few reports on its efficacy in children. In this study, we compared the outcomes of children with CML before and after the advent of IM-based treatment. The study cohort consisted of 52 patients treated for CML at the Department of Pediatrics, The Catholic University of... |
The purpose of this study was to evaluate short-term thyroid dysfunction and related risk factors in pediatric patients who underwent hematopoietic stem cell transplantation (HSCT) during childhood. We studied 166 patients (100 boys and 66 girls) who underwent HSCT at the Catholic HSCT Center from January 2004 through December 2009. The mean age at HSCT was 10.0±4.8 years. Thyroid function of... |
In children with acute leukemia, bone marrow genetic abnormalities (GA) have prognostic significance, and may be the basis for minimal residual disease monitoring. Since April 2007, we have used a multiplex reverse transcriptase-polymerase chain reaction tool (HemaVision) to detect of GA. In this study, we reviewed the results of HemaVision screening in 270 children with acute leukemia, newly diagnosed at The... |
Lymphocyte subset recovery is an important factor that determines the success of hematopoietic stem cell transplantation (HSCT). Temporal differences in the recovery of lymphocyte subsets and the factors influencing this recovery are important variables that affect a patient's post-transplant immune reconstitution, and therefore require investigation. The time taken to achieve lymphocyte subset recovery and the factors influencing this recovery were investigated... |
Improved survival of patients with childhood acute lymphoblastic leukemia (ALL) has drawn attention to the potential for late consequences of previous treatments among survivors, including metabolic syndrome. In this study, we evaluated changes in 3 parameters, namely, random blood glucose, body mass index (BMI), and Z score for BMI (Z-BMI), in children with ALL during chemotherapy and after completion of... |
The survival rate for childhood acute lymphoblastic leukemia (ALL) has improved significantly. However, overall prognosis for the 20 to 25% of patients who relapse is poor, and allogeneic hematopoietic stem cell transplantation (HSCT) offers the best chance for cure. In this study, we identified significant prognostic variables by analyzing the outcomes of allogeneic HSCT in ALL patients in second complete... |
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 : Due to its high potency against leukemic blasts, our institution has opted for the use of dexamethasone during acute lymphoblastic leukemia (ALL) remission induction, but in our most recent treatment protocol, CMCPL-2005, we shortened the length of steroid treatment from 4 to 3 weeks. We compared both the rates of remission induction and significant complications observed during induction... |
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 : 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.... |
Glucose-6-phosphate dehydrogenase(G-6-PD) deficiency is a disease that shows hemolytic anemia and jaundice caused by injury of erythrocytes. The gene of G-6-PD has 13 exons and locates in Xq28, and over 150 mutations of this gene have been reported. We experienced a G-6-PD deficienct male patient who was suffering hemolytic anemia and jaundice confirmed by measuring low G-6-PD activity in the... |
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 : This study was aimed to investigate age-related changes of CD5+ B lymphocytes in healthy Korean neonates. Methods : Sixty healthy neonatal infants were enrolled in this study; at birth(n=10), day 1(n=10), day 2(n=10), day 3(n=10), day 4(n=10) and day 5(n=10). Phenotypic analysis of CD5+ B lymphocytes was performed on blood samples using standard flow-cytometric techniques. Results : The percentage of... |
Purpose : We reviewed 100 cases of HLA-matched sibling allogeneic bone marrow transplantation( allo-BMT) in children and wish to share these results. Methods : One hundred children had undergone allo-BMT from HLA-identical siblings between Nov. 1983 and May 1998. There were 50 males and 50 females with a median age of 10 years and a median follow-up of 38 months. Out of 100 cases,... |
von Gierke disease(type Ia glycogen storage disease) is an inherited disease associated with accumulation of glycogen in the liver, kidney, intestine and erythrocytes due to the defect of glucose-6-phosphatase activity. Hepatomegaly, doll face, anemia, bleeding tendency and increased susceptability to infection are common features observed during infancy. Hypoglycemia especially fasting hypoglycemia is typical metabolic derangement in this disease, followed by metabolic acidosis, lactic acidemia, hyperlipidemia, hyperuricemia,... |
Purpose : It has been suggested that immunologic abnormalities play a role in the pathogenesis of vascular injury in Kawasaki disease and the immunologic abnormalities could be regulated by high-dose intravenous immunoglobulin(IVIG). We investigated the peripheral blood lymphocyte subsets, the serum levels of interleukin-6(IL-6) and tumor necrosis factor-α(TNF-α) and the effect of high-dose IVIG(400 mg/kg/day for 5 days) on these factors during acute stage of... |
Purpose : This study was carried out to evaluate the age and sex distribution, clinical manifestation, presence of the antithyroid antibody, the clinical outcome following antithyroid drug treatment in children with Graves' disease. Methods : A total 45 children with Graves' disease were entered into the study. Diagnosis was based on clinical manifestation, elevated thyroid function and increased homogeneous 99mTc thyroid... |
Purpose : Asthma, one of the most common of chronic cough in children, is easily diagnosed when classic triad; cough, wheezing, dyspnea are presented. However, it has become apparant that cough may be the sole manifestation of this disease. This cough variant asthma is underdiagnosed and undertreated. The clinical manifestations and subject variables of asthma especially, cough variant asthma had... |
Chromic cough is a symptom frequently encountered by the pediatrician. Although most coughs are self-limited, chronic cough ofter proves to be a frustrating problem. This study was performed at Kangnam St. Mary's Hospital from January 1,1992 to December 31, 1992, and 83 children with chronic cough persisting for longer than 3 weeks was evaluated. We categorized these patients into 5... |
The idiopathic hypereosinophilic syndrome(HES) represents a heterogenous group of disorder characterized by prolonged eosinophilia of undetectable cause and multiorgan system dysfunction. Bone marrow is the most frequentry involved organ, but the most severe clinicopathologic involvement is heart. The major cause of death in patients with the HES is cardiac dysfunction especially congestive heart failure resulted from endocardial fibrosis and restrictive cardiomyopathy. We... |
Distal renal tubular acidosis (RTA) is a biochemical syndrome as a dificiency of hydrogen ion secretion by the distal tubule and collecting duct. Owing to the nature of the defect, hyperchloremic non-anion gap metabolic acidosis and high urine pH despite severe systemic acidosis is characterized. Typical manifestations of distal RTA are growth retardation, rickets, polyuria and nephrocalcinosis. We experienced a case of... |
Scleredema is a rare skin disease of unknown cause involving the face, neck upper portion of the trunk, and proximal upper extremities. The clinical features of it are nonpitting, solid emema or stiffness of the neck, which may be sudden or insidious onset. Preceeding upper respiratory infection or diabetes mellitus have been frequently discribed to be associated with disease. A monoclonal... |