• IMMUNE NETWORK
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• 제22권5호
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• pp.37.1-37.16
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• 2022

Autoimmune diseases are caused by a dysfunction of the acquired immune system. In a subset of autoimmune diseases, B cells escaping immune tolerance present autoantigen and produce cytokines and/or autoantibodies, resulting in systemic or organ-specific autoimmunity. Therefore, B cell depletion with monoclonal Abs targeting B cell lineage markers is standard care therapy for several B cell-mediated autoimmune disorders. In the last 5 years, genetically-engineered cellular immunotherapies targeting B cells have shown superior efficacy and long-term remission of B cell malignancies compared to historical clinical outcomes using B cell depletion with monoclonal Ab therapies. This has raised interest in understanding whether similar durable remission could be achieved with use of genetically-engineered cell therapies for autoimmunity. This review will focus on current human clinical trials using engineered cell therapies for B cell-associated autoimmune diseases.

• 한국임상약학회지
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• 제14권1호
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• pp.11-23
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• 2004

Although most children with idiopathic nephrotic syndrome respond to corticosteroid therapy, many responders show steroid dependency and frequent relapse. In these children, one of the major problems is the serious side effects resulting from continuous steroid therapy. Thus, this study was conducted to assess the therapeutic efficacy and safety of six-month cyclosporine treatment with the low-dose deflazacort therapy in children with nephrotic syndrome. Thirty children with steroid dependence (SD), frequent relapse (FR) and steroid resistance (SR) were enrolled in this study. They were treated with 6-month oral cyclosporine $(Cypol-N^{(R)})$ plus the low-dose deflazacort $(Calcort^{(R)})$ therapy at Samsung Medical Center from September 2002. The dosage of cyclosporine was started at 5 mg/kg/day and was monthly adjusted to maintain clinical remission and/or a trough blood level, while deflazacort dosage was reduced gradually. Clinical evaluation and monitoring of cyclosporine toxicity were performed every $2\sim4$ weeks. Outcomes were compared to the latest sir-month period of steroid only therapy before cyclosporine treatment. Student's t-test and ANOVA were used for statistical analysis. Out of 28 children with SD and FR, 23 $(82.1\%)$ sustained remission, and 5 $(17.9\%)$ experienced 1 or 2 relapses during therapy. Out of 2 children with SR, 1 child sustained remission, and 1 child showed no response. The mean duration of remission and occurrence of relapse were significantly improved (p <.0001). In addition, the mean dosage of steroid was significantly reduced (p=.003). Although a number of adverse effects occurred in this study, they were not so serious as to necessitate discontinuation of the therapy. No nephrotoxicity was observed. Twenty out of the 28 children who had been in remission relapsed after withdrawal of cyclosporine. Fifteen of these children showed relapse within a month. These results demonstrated that the combination of cyclosporine with the low-dose deflazacort was efficient and safe in children with SD and FR during the six-month treatment. However, further studies are necessary in order to resolve the problem of high relapse rate after discontinuation of cyclosporine.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권1호
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• pp.72-79
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• 2019

Purpose: The efficacy of exclusive enteral nutrition (EEN) with a polymeric diet has not been confirmed in Korean pediatric patients with Crohn's disease (CD). This study aimed to compare the effectiveness of EEN with a specific polymeric diet ($Encover^{(R)}$) and corticosteroids (CSs) for the induction of remission in Korean pediatric CD patients. Methods: We retrospectively compared data from 51 pediatric CD patients who underwent induction therapy with EEN (n=19) or CSs (n=32) at Severance Children's Hospital or Incheon St. Mary's Hospital in Korea. The primary endpoint of this study was the rate of clinical remission, defined as a Pediatric Crohn's Disease Activity Index (PCDAI) score <10, after 8 weeks of induction treatment. Clinical, laboratory, and growth data at post-induction as well as their changes from baseline were also compared between groups. Results: After 8 weeks of induction therapy, clinical remission rates were 78.9% (15/19) and 65.6% (21/32) in the EEN and CS groups, respectively (p=0.313). No significant differences in PCDAI scores, laboratory variables, and growth parameters were noted between the two groups at post-induction. However, significant changes in albumin levels at post-induction were observed in the EEN group compared to the CS group (p=0.038). Conclusion: Our results suggest that the effectiveness of EEN with a polymeric diet and CSs for induction therapy did not differ in Korean pediatric CD patients. EEN with a polymeric diet is a good first-line treatment option for the induction of remission in these patients.

• 한국임상약학회지
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• 제22권3호
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• pp.251-259
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• 2012

Ulcerative colitis (UC) is characterized by a life-long chronic course with remissions and exacerbations. Use of biological therapies may reduce or delay the surgical procedures in patients with UC. The aim of this study was to determine the impact of infliximab (IFX) use on the rate of remission, surgical interventions, and the effect on quality of life in patients with moderate to severe UC. Literature was searched for studies that investigated the efficacy of IFX on the rate of remission, colectomy and quality of life (QoL) between January 1990 and June 2012 at MEDLINE, January 1988 and June 2012 at EMbase and others. Eleven trials were included in the meta-analysis; divided into placebo controlled 8 trials and intravenous corticosteroid controlled group 3 trials. In comparison to placebo control groups, patients who received IFX had an odds ratio (OR) of 3.712 (95% CI: 2.714, 5.079) for the short-term clinical remission, and 3.053 (95% CI: 2.044, 4.559) for the rate of long-term remission. In colectomy rate and quality of life (QoL), odds ratio were 0.566(95% CI: 0.387, 0.827) and 0.658 (0.505, 0.811) respectively. Any adverse reactions including infections, infusion reaction, rash and arthralgia were equivalent in both groups. Compared with intravenous corticosteroid controlled group, patients who received IFX had lower remission rate with short-term odds ratio 0.227 (95% CI: 0.033, 1.556) and long-term odds ratio 1.054 (95% CI: 0.317, 3.502) respectively. However, statistical significance was not showed with both two analyses. The higher adverse drug reaction (ADR) rates were occurred in the corticosteroid controlled groups. 73.3% of patients treated corticosteroid reported Cushing-like syndrome with moon face. In conclusion, IFX does increase remission rate and decrease the rate of colectomy in patients with UC without elevating any adverse reactions significantly. IFX also improves QoL in moderate to severe UC patients. It would not exceed the efficacy of intravenous corticosteroid, whereas intravenous corticosteroid also reported high rate of adverse reactions.

• Clinical and Experimental Pediatrics
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• 제58권6호
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• pp.206-210
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• 2015

Purpose: The use of a 12-week steroid regimen (long-term therapy, LT) for the first episode of idiopathic nephrotic syndrome (NS) reportedly induces a more sustained remission and lower relapse rate than previous regimens, including an 8-week steroid regimen (short-term therapy, ST). Here, we assessed the potential for selective application of 2 steroid regimens (LT vs. ST) based on the days to remission (early responders [ER] vs. late responders [LR]) for the first idiopathic NS episode in children. Methods: Patients were divided into 4 subgroups (ST+ER, ST+LR, LT+ER, and LT+LR) according to the initial steroid regimen used and rapidity of response; the baseline characteristics, relapse rates, and cumulative percentage of children with sustained remission were then compared among the 4 subgroups. Results: Fifty-four children received ST, and the remaining 45 children received LT. As observed in previous studies, children receiving LT showed significantly lower relapse rates during the first year after the first NS episode than those receiving ST. The ST+ER group showed significantly lower relapse rates during the first one year and two years after the first NS episode than the the ST+LR group, whereas there were no significant differences of the relapse rates and duration to the first relapse between the ST+ER and LT+ER groups. Conclusion: We suggest that the initial steroid regimen in idiopathic NS patients can be shortened according to the duration to remission i.e., LT in patients achieving remission after the first week of steroid therapy, and ST in those achieving remission within the first week of steroid therapy.

• Clinical and Experimental Pediatrics
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• 제54권8호
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• pp.317-321
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• 2011

Children who suffer from steroid-resistant nephrotic syndrome (SRNS) require aggressive treatment to achieve remission. When intravenous high-dose methylprednisolone fails, calcineurin inhibitors, such as cyclosporine and tacrolimus, are used as the first line of treatment. A significant number of patients with SRNS progress to end-stage renal disease if remission is not achieved. For these children, renal replacement therapy can also be problematic; peritoneal dialysis may be accompanied by significant protein loss through the peritoneal membrane, and kidney allograft transplantation may be complicated by recurrence of SRNS. Plasmapheresis and rituximab were initially used for treatment of recurrent SRNS after transplantation; these are now under consideration as rescue therapies for refractory SRNS. Although the prognosis of SRNS is complicated and unfavorable, intensive treatment in the early stages of the disease may achieve remission in more than half of the patients. Therefore, timely referral of pediatric SRNS patients to pediatric nephrology specialists for histological and genetic diagnosis and treatment is highly recommended.

• Journal of Korean Biological Nursing Science
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• 제10권2호
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• pp.170-175
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• 2008

Purpose: The purpose of this study was to predict treatment outcome of chemotherapy compared with and bone marrow transplantation in acute leukemia patients. Methods: We respectively reviewed the characteristics of subjects, cytokine, complete remission time and survival time of 111 patients with acute leukemia, admitted in St. Mary's hospital, between July 2007 and August 2008. Results: The complete remission rate with chemotherapy group was 70.8% and bone marrow transplantation group was 54.3% but without statistically significance. The prognostic factors related with survival is classification of acute leukemia and complete remission time. Conclusion: This study suggests a need for nursing research and nursing intervention for acute leukemia patients.

• Journal of Chest Surgery
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• 제25권8호
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• pp.844-849
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• 1992

Between 1979 and 1991 thymectomy was performed on 31 patients with myasthenia gravis at the department of thoracic and cardiovascular surgery, Keimyung University medical school. All patients were treated by transsternal thymectomy. During follow-up period that ranged from a month to 10.7 years[mean 2 years], the remission rate for the entire group was 16.1 percent and an additional 71.0 percent had improvement [87.1 percent benefited]. In those 8 patients with thymoma, the remission rate was 12.5 percent with 75.0 percent of the patients benefiting from operations The remainimg 23 patients fared better the operations: 17.4 percent had remission and a total of 91.4 percent benefited. There was no mortality, I concluded that most patients with myasthenia gravis will benefit from thymectomy, and that the improvement persists over an extended period of time in a high percentage of patients.

• Asian Pacific Journal of Cancer Prevention
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• 제17권12호
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• pp.5251-5256
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• 2016

Background: Diagnostic karyotyping analysis is routinely used in acute myeloid leukemia (AML) clinics. Categorization of patients into risk stratified groups (favorable, intermediate and adverse) according to cytogenetic findings can serve as a valuable independent prognostic factor. Method and Material: A retrospective descriptive study was conducted based on the patient records of newly diagnosed non-M3 AML young adult cases undergoing standard 3+7 i.e, Daunorubicin and Ara-C (DA) as remission induction chemotherapy. Diagnostic cytogenetic analysis reports were analyzed to classify the patients into risk stratified groups according to South West Oncology Group criteria and prognostic significance was measured with reference to achievement of haematological remission after 1st induction chemotherapy. Results:A normal karyotype was commonly expressed, found in 47.2% of patients, while 65% (n=39) appeared to have intermediate risk cytogenetics, and 13.3% (n=8) adverse or unclassified findings. Favourable cytogenetics was least frequent in the patient cohort, accounting for only 8.3 % (n=5).The impact of cytogenetic risk groups on achievement of haematological remission was evaluated by applying Pearson Chi-square, and was found to be non-significant (df=12, p=0.256) but when the outcomes of favourable risk groups with intermediate, adverse and unclassified findings compared, results were highly significant (df=6, p=0.000) for each comparison. In patients of the favourable cytogenetic risk group, HR?? was reported in 40% (n=2/5), as compared to 62.2% (n=23/37) in the intermediate cytogenetic risk group, 57.1% (n=4/7) in the adverse cytogenetic risk group and 28.6% (n=2/7) in hte unclassified cytogenetic risk group. Conclusion: Cytogenetic risk stratification for AML cases following criteria provided by international guidelines did not produce conclusive results in our Pakistani patients. However, we cannot preclude an importance as the literature clearly supports the use of pretreatment karyotyping analysis as a significant predictive marker for clinical outcomes. The apparent differences between Pakistani and Western studies indicate an urgent need to develop risk stratification guidelines according to the specific cytogenetic makeup of South Asian populations.

• Journal of Chest Surgery
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• 제18권4호
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• pp.872-880
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• 1985

Myasthenia gravis is a neuromuscular transmission disorder characterized by fatigue and weakness of voluntary muscles. Although the pathogenesis is known as reduction of available acetylcholine receptors at neuromuscular junctions by autoimmune attack, the thymic role in myasthenia gravis is still unclear and under investigation. But thymectomy in the management of myasthenia gravis has become increasingly important since the first successful operation with remission of symptoms in 1939 by Blalock. From January 1983 to June 1985, authors performed 17 thymectomies for patients with myasthenia gravis. Among them, 12 patients were free from thymoma [Croup A] and 5 were coupled with thymoma [Group B]. The results were as follows: 1] Sex distribution was 11 females and 6 males. Mean age of the patients was 32.2 year old. Sex and age distribution by the Group A and B are shown Table 1. 2] Clinical manifestations of ocular symptoms were seen in 5 patients [88.2%], extremity weakness in 13 patients, bulbar weakness in 12 patients and dyspnea in 6 patients. According to the Osserman`s classification, 5 patients were in group IIA, 6 in IIB and 6 in IIC. 3] Pre-operatively, all patients were positive response to the anti-cholinesterase test and 12 patients [92.3%] revealed positive findings in electromyography [EMC] which was done in 13 patients. 4] The postoperative complications were respiratory distress in 3 patients, myasthenic crisis in 2 patients and wound disruption in one patients. 5] Pathologic examination of the thymus showed hyperplasia in 10 patients [90%] and thymoma in 5 patients, of which 4 were mixed type with invasion to the adjacent tissues and one lymphocytic type without invasion. Normal thymus was noticed in only 2 patients. 6] In postoperative evaluations, among the 12 patients c free from thymoma [Group A], complete remission of symptoms was noticed in 3 patients and improvement in 7 patients. But among the 5 patients coupled with thymoma [Group B], only one patients showed improvement [Table 8]. Therefore, remission and clinical improvement were noticed in 11 patients [64.7%] of the all and complete remission was noticed in 3 patients [17.6%].