• Pediatric Infection and Vaccine
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• v.21 no.2
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• pp.81-95
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• 2014

Purpose: Hematopoietic cell transplantation (HCT) recipients are vulnerable to invasive infection by Haemophilus influenzae type b (Hib) and Streptococcus pneumoniae (Sp). This study was performed to evaluate immune responses after Hib and Sp vaccination in Korean pediatric HCT recipients. Methods: Patients were prospectively enrolled at Samsung Medical Center during 2009-2011. ELISA tests to detect anti-PRP IgG antibody and antibodies to Sp serotypes 4, 6B, 9V, 14, 18C, 19F, and 23F were performed at the Center for Vaccine Evaluation and Study, Ewha Medical Research Institute. Results: Ten patients (two allogeneic, eight autologous recipients) with median age 5.4 years (range 2.7-12.2 years) were enrolled. Before Hib vaccination, 60% of patients' anti-PRP IgG titers were below $0.15{\mu}g/mL$. After vaccination, 100% of patients' anti-PRP IgG titers increased above $0.15{\mu}g/mL$ (cut-off value for detection) and $1.0{\mu}g/mL$ (cut-off value for seroprotection). For pneumococcus, in 2-5 year-old patients, pre-vaccination geometric mean concentrations (GMCs) of IgG for six serotypes (4, 6B, 9V, 14, 18C, and 23F) were below $0.35{\mu}g/mL$ and at 5 months post-vaccination GMCs of IgG for all seven serotypes increased to above $0.35{\mu}g/mL$. In patients older than 5 years, pre-vaccination GMCs of IgG for four serotypes (4, 9V, 14, and 23F) were below $0.35{\mu}g/mL$ and at 3 months post-vaccination GMCs of IgG for all seven serotypes increased to above $0.35{\mu}g/mL$. Conclusion: Most HCT recipients had low or no protective antibodies to Hib and Sp before vaccination, but showed good immune responses to protective levels after vaccination.

• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.725-729
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• 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 might be an effective option in patients with EBV-HLH with progressive CNS disease. Moreover, the addition of rituximab to RIC appears to be safe and effective in suppressing EBV in the patients with EBV-HLH.

• Journal of Korean Academy of Fundamentals of Nursing
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• v.13 no.3
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• pp.428-436
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• 2006

Purpose: To investigate the levels of uncertainty and anxiety at admission and discharge to the isolation unit for patients undergoing hematopoietic stem cell transplantation (HSCT). Method: The data were collected from 60 patients who were admitted to the department of HSCT. Uncertainty and anxiety were assessed using the Mishel Uncertainty in Illness Scale (MUIS) and Anxiety Inventory (STAI). Collected data were analyzed using the SAS program. Results: The mean score for anxiety at admission was 2.20, and at discharge 2.10. The mean score for uncertainty at admission was 2.19, and at discharge 2.07. The anxiety at admission the group with physical discomfort was higher than that of group without physical discomfort. A positive relationship was found between anxiety and uncertainty at admission and at discharge. The major variables were expectation for cure and physical discomfort, explaining 25.87% of the anxiety at admission. The major variable was expectation for cure, explaining 20.94% of the uncertainty at admission. Conclusion: Front the above results, it can be concluded that support and consideration are required to reduce anxiety of the inpatient in single room.

• Journal of Korean Academy of Fundamentals of Nursing
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• v.13 no.3
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• pp.437-446
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• 2006

Purpose: This study was done to identify the level of stress, coping, and depression among hemopoietic stem cell transplantation patients who received care in an outpatient center. Method: Data were collected from 81 patients who underwent hemopoietic stem cell transplantation at C University S Hospital between August 2005 and February 2006. Results: Stress and depression were significantly higher following hemopoietic stem cell transplantation in women, and in patients who were worse off financially or who were in bad health. The highest item of stress was 'economic burden for treatment'. There were significantly higher levels of emotion-focused coping among patients who had a spouse and who received motivation from the doctor. The highest item for problem-focused coping was 'try to look at the bright side of life'. The highest items for emotion-focused coping were to 'have faith that treatment will be finished quickly' and 'believe that your situation will improve'. Stress was significantly correlated with depression among these patients. Conclusion: It is necessary to develop nursing interventions to enhance positive coping and to decrease stress and depression among patients who have a hemopoietic stem cell transplantation.

• Asian Oncology Nursing
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• v.8 no.1
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• pp.40-49
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• 2008

Purpose: This descriptive study was to investigate the quality of life in patients with hematopoietic stem cell transplantation (HSCT) from June 1 to October 13, 2007. Method: The survey was conducted in 6 different university hospitals which located in Seoul and Jeollanam-do province using the Functional Assessment of Cancer Therapy-BMT Scale (FACT-BMT) version 4. We collected a total of 155 questionnaires and analyzed 149 among them. Results: The average score of quality of life was 2.53 out of 5. Physical well being score was highest among sub-domains, followed by emotional well-being, additional concerns, social/family well-being, and functional well-being. Study subjects worried that their conditions would get worse. However study subjects didn't regret having been received HSCT. Age, duration from HSCT, age at diagnosis, income, readmission, HSCT type, educational background, marital status, and the level of activities of daily living were related to quality of life. Conclusions: The findings of this study indicates that the HSCT survivor's quality of life issue is still important and have to be investigated repeatedly in the future. That is necessary for generalizing QOL outcomes for clinical use. We also suggest to develop interventions to improve QOL.

• Journal of Korean Academy of Nursing
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• v.44 no.5
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• pp.542-551
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• 2014

Purpose: This study was done to examine the incidence of oral mucositis in hematopoietic stem cell transplantation patients and to identify factors influencing oral mucositis and patient outcomes according to severity. Methods: In this retrospective study, data were collected from electronic medical records of 222 patients who had received hematopoietic stem cell transplantation. Oral mucositis was evaluated using WHO's assessment scale. Data were analyzed using Chi-square test, Fisher exact test, Spearman's correlation, Ordinal logistic regression, ANOVA and Kruskal-Wallis test. Results: A total of 69.8% of the patients evaluated developed oral mucositis (grade II and over). As a results of ordinal regression, factors influencing oral mucositis severity were found to be diagnosis, type of transplantation, oxygen inhalation and the number of antiemetics administration before transplantation. The severity of oral mucositis was found to increase the days of hospitalization, days of TPN administration, days of using antibiotics and the number and dosage of analgesics. Conclusion: The results would help predict severity of oral mucositis in hematopoietic stem cell transplantation patients and suggest that provision of appropriate nursing assessment and oral care would improve patient outcomes.

• Journal of the Korean society of biological therapies in psychiatry
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• v.24 no.3
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• pp.142-155
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• 2018

Insomnia in patients with hematopoietic stem cell transplantation(HSCT) has been underdiagnosed and undertreated. This study reviewed the frequency, characteristics, physical and psychological effects, and treatments of insomnia in HSCT patients to highlight clinical importance in this specialized population. Furthermore, the authors intended to suggest a model that would conceptualize insomnia in the context of HSCT. In the pre-transplant period, about half of patients with HSCT suffered from sleep disturbance. A substantial number of patients experienced distressing insomnia during the HSCT procedure and recovered to the level of the pre-transplant period. However, sleep disruption could be a chronic symptom in HSCT survivors and could negatively impact quality of control, cancer-related fatigue(CRF), immune function, and psychological distress. The 3P's model(Predisposing, Precipitating, Perpetuating) explains insomnia in cancer population and could be also relevant to HSCT patients with specific consideration of CRF, graft-versus-host diseases, specific properties of hematological disease, and protective isolated milieu. Effective treatment of insomnia in HSCT includes non-pharmacological(e.g., cognitive behavioral therapy, environmental modification) and pharmacological interventions. The decision of pharmacological treatment should be based on the issue of safety due to high risk of potential drug-drug interactions. Screening, treatment, and further research of insomnia in HSCT patients using validated subjective and/or objective measures are warranted.

• Clinical and Experimental Pediatrics
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• v.49 no.12
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• pp.1329-1339
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• 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 University. This study was a retrospective analysis of the medical records of 15 patients who received the second HSCT after initial graft. Results : Among eight patients with nonmalignant diseases, two patients underwent the second HSCT because of primary graft failure and five because of late graft rejection. The remaining Fanconi anemia patient was re-transplanted due to development of AML. Two patients died and one experienced primary graft failure, but is still alive. The Kaplan-Meier 5-year overall survival rate was 75 percent and the disease free survival rate was 62.5 percent in nonmalignant diseases. All malignant patients underwent second transplants because of relapses. Four died of relapse and one of treatment-related complications. The Kaplan-Meier 2-year overall and event free survival rate was 28.6 percent each in malignant diseases. Conclusion : Second HSCT for graft dysfunction of nonmalignant disease seems to be feasible and should be considered as a standard practice. The relapse of malignant diseases remains a big obstacle even after the second HSCT, although a small portion of patients might be salvaged. Further investigation of novel therapeutic strategies, as well an the understanding of the biology should be explored.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.24 no.1
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• pp.10-16
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• 2024

Congenital Disorders of Glycosylation (CDG) represent a complex group of inherited metabolic disorders resulting from defects in multiple pathways of glycosylation, a critical biochemical process for protein functionality and cellular communication. This review provides a comprehensive overview of CDG, including its history, epidemiology, classification, diagnostic complexities, and therapeutic developments. Despite advancements in understanding CDG and identifying over 160 subtypes, challenges remain due to the diverse clinical manifestations and multi-systemic involvement. Targeted therapy is available for only a few CDGs, but promising treatments are being investigated. Ongoing research is vital to developing targeted treatments and improving patient outcomes.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.247-255
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• 2002

Purpose : This study was undertaken to obtain basic data about the megakaryocyte colony formation of fetal liver cells by using immunocytochemical staining and ex vivo culture with growth factors. Methods : The mononuclear cells were isolated from fetal liver and bone marrow with idiopathic thrombocytopenic purpura(ITP) and pancytopenia. These mononuclear cells were cultured in $MegaCult^{TM}-C$(Stem Cell Tech, Canada) media in the presence of growth factors and CFU-Megakaryocyte( CFU-Mk) colonies were counted on day 12. The expansion of CD34+ and CD41+ cell was analyzed by flow cytometry after 5 days incubation using flask culture. Results : The numbers of CFU-Mk colonies of mononuclear cells obtained from fetal liver in the 11th week gestational age were more than those in the 19th week specimens; growth factors could not enhance the colony expansion in all cases. Total numbers of CFU-Mk colony of fetal liver cells were higher than bone marrow from ITP or pancytopenia groups. The numbers of pure or large CFU-Mk colonies of fetal liver cells were also higher than bone marrow specimens. The rate of CD34+ cell expression of fetal liver was increased after flask culture and the enhancement effect of epression was seen only in cases which added thrombopoietin. The rate of CD41+ cell expression of fetal liver was increased after incubation, but the enhancement effect of growth factors was unclear. Conclusion : This study revealed good results about the megakaryocyte colony assay of fetal liver mononuclear cells using $MegaCult^{TM}-C$ media. This study suggests that the fetal liver could be a good source of megakaryocytic progenitor cells for clinical application in hematopoietic stem cell transplantation.