• Journal of Korean Neurosurgical Society
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• 제67권2호
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• pp.237-248
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• 2024

Objective : Postoperative data on Cushing's disease (CD) are equivocal in the literature. These discrepancies may be attributed to different series with different criteria for remission and variable follow-up durations. Additional data from experienced centers may address these discrepancies. In this study, we present the results obtained from 96 endoscopic transsphenoidal surgeries (ETSSs) for CD conducted in a well-experienced center. Methods : Pre- and postoperative data of 96 ETSS in 87 patients with CD were included. All cases were handled by the same neurosurgical team between 2014 and 2022. We obtained data on remission status 3-6 months postoperatively (medium-term) and during the latest follow-up (long-term). Additionally, magnetic resonance imaging (MRI) and pathology results were obtained for each case. Results : The mean follow-up duration was 39.5±3.2 months. Medium and long-term remission rates were 77% and 82%, respectively. When only first-time operations were considered, the medium- and long-term remission rates were 78% and 82%, respectively. The recurrence rate in this series was 2.5%. Patients who showed remission between 3-6 months had higher long-term remission rates than did those without initial remission. Tumors >2 cm and extended tumor invasion of the cavernous sinus (Knosp 4) were associated with lower postoperative remission rates. Conclusion : Adenoma size and the presence/absence of cavernous sinus invasion on preopera-tive MRI may predict long-term postoperative remission. A tumor size of 2 cm may be a supporting criterion for predicting remission in Knosp 4 tumors. Further studies with larger patient populations are necessary to support this finding.

• Journal of Yeungnam Medical Science
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• 제40권1호
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• pp.12-22
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• 2023

Graves disease (GD) is the most common cause of hyperthyroidism, accounting for more than 90% of cases in Korea. Patients with GD are treated with any of the following: antithyroid drugs (ATDs), radioactive iodine (RAI) therapy, or thyroidectomy. Most patients begin treatment with ATDs, and clinical guidelines suggest that the appropriate treatment period is 12 to 18 months. While RAI treatment and surgery manage thyrotoxicosis by destroying or removing thyroid tissue, ATDs control thyrotoxicosis by inhibiting thyroid hormone synthesis and preserving the thyroid gland. Although ATDs efficiently control thyrotoxicosis symptoms, they do not correct the main etiology of GD; therefore, frequent relapses can follow. Recently, a large amount of data has been collected on long-term ATDs for GD, and low-dose methimazole (MMZ) is expected to be a good option for remission. For the long-term management of recurrent GD, it is important to induce remission by evaluating the patient's drug response, stopping ATDs at an appropriate time, and actively switching to surgery or RAI therapy, if indicated. Continuing drug treatment for an extended time is now encouraged in patients with a high possibility of remission with low-dose MMZ. It is also important to pay attention to the quality of life of the patients. This review aimed to summarize the appropriate treatment methods and timing of treatment transition in patients who relapsed several times while receiving treatment for GD.

• Annals of Clinical Neurophysiology
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• 제9권2호
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• pp.51-58
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• 2007

Autoimmune myasthenia gravis (MG) is the neuromuscular junction disorder mostly caused by antibody against the acetylcholine receptor (AChR antibody) at the muscle endplate. The goal of treatment is to induce and maintain remission, i.e., absence of symptoms, with the least cost-to-benefit ratio. Although corticosteroids are effective in inducing remission in most patients, they have numerous potentially serious adverse effects with their long-term use. In addition, some patients do not respond or are intolerant to the conventional treatment. In this article, we discuss the difficulties encountered in long-term immunosuppressive treatment of MG, and review useful tips for the use of corticosteroids. Long-term immunosuppressive agents that can be used in steroid-refractory or -dependent patients will be reviewed with their safety profiles and efficacy in MG.

• Clinical and Experimental Pediatrics
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• 제46권10호
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• pp.1013-1018
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• 2003

목 적 : 천식 환자에서 특징적으로 존재하는 BHR의 기전으로 호산구성 기도 염증 반응이 중요시 생각되고 있다. 그러나 장기간 천식 관해 상태인 청소년에서 BHR이 지속되는 기전에 대해서는 아직 명확하지 않은 실정이다. 본 연구는 장기간 천식 관해 된 청소년에서의 BHR이 호산구성 기도 혈액내 총 호산구 증가와 혈청 ECP 농도의 증가와 연관되어 있는지 알아보고자 시행하였다. 방 법 : 서울대학교 어린이병원 소아과 외래에서 아토피성 천식으로 진단되었던 청소년 중 임상적으로 장기간 관해(최근 2년간 천식 증상이 없고 치료가 필요 없었던 경우)되었으나 BHR이 잔존하는 35명과 이들과 서로 대응하는 BHR을 갖고 있으면서 현증을 보이는 아토피 천식 청소년 35명을 대상으로 하였다. 이 두 군간의 총 호산구 수와 혈청 ECP 농도를 비교하고 각 군에서 총 호산구 수 및 혈청 ECP 농도와 $PC_{20}$ 사이에 상관 관계가 있는 지 분석하였다. 결 과 : 장기간 관해 된 군의 혈액 내 호산구 수와 혈청 ECP 농도의 평균값이 현증을 보이는 군보다 통계적으로 유의하게 낮았다($273{\pm}108$ vs. $365{\pm}178/{\mu}L$; $16.3{\pm}9.4$ vs. $26.5{\pm}15.1{\mu}g/L$, both, P<0.05). 현증을 보이는 군에서는 메타콜린 $PC_{20}$에 따라 혈액 내 호산구 수 및 혈청 ECP 농도가 각각 통계적으로 유의한 음의 상관관계를 보였으나(r=-0.385, P=0.022; r=-0.439, P=0.008) 장기간 관해 된 군에서는 유의한 상관관계를 보이지 않았다(r=-0.292, P=0.089; r=-0.243, P=0.159). 결 론 : 이와 같은 소견은 장기간 관해시의 BHR은 호산구성 염증과 연관성이 없으며 현증 천식에서의 BHR과는 다른 기전으로 설명될 수 있을 것으로 추정된다. 장기간 천식 관해 된 청소년에서 BHR이 잔존하는 기전에 대하여 정확히 밝히기 위해서 향후 BAL, 조직 검사, 유도 객담 등을 이용한 연구를 시행할 필요가 있다.

• 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제24권1호
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• pp.7-18
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• 2021

Purpose: The long-term efficacy and safety of infliximab (IFX) in children with ulcerative colitis (UC) have not been well-evaluated. Here, we reviewed the long-term durability and safety of IFX in our single center pediatric cohort with UC. Methods: This retrospective study included 20 children with UC who were administered IFX. Results: For induction, 5 mg/kg IFX was administered at weeks 0, 2, and 6, followed by every 8 weeks for maintenance. The dose and interval of IFX were adjusted depending on clinical decisions. Corticosteroid (CS)-free remission without dose escalation (DE) occurred in 30% and 25% of patients at weeks 30 and 54, respectively. Patients who achieved CS-free remission without DE at week 30 sustained long-term IFX treatment without colectomy. However, one-third of the patients discontinued IFX treatment because of a primary nonresponse, and one-third experienced secondary loss of response (sLOR). IFX durability was higher in patients administered IFX plus azathioprine for >6 months. Four of five patients with very early onset UC had a primary nonresponse. Infusion reactions (IRs) occurred in 10 patients, resulting in discontinuation of IFX in four of these patients. No severe opportunistic infections occurred, except in one patient who developed acute focal bacterial nephritis. Three patients developed psoriasis-like lesions. Conclusion: IFX is relatively safe and effective for children with UC. Clinical remission at week 30 was associated with long-term durability of colectomy-free IFX treatment. However, approximately two-thirds of the patients were unable to continue IFX therapy because of primary nonresponse, sLOR, IRs, and other side effects.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권1호
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• pp.31-36
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• 2014

Purpose: Infliximab (IFX) is considered safe and effective for the treatment of ulcerative colitis (UC) in both adults and children. The aim of this study was to evaluate the short- and long-term clinical course of IFX in Korean children with UC. Methods: Pediatric patients with UC who had received IFX infusions between November 2007 and May 2013 at Samsung Medical Center were retrospectively investigated. The clinical efficacy of IFX treatment was evaluated at 8 weeks (short term) and 54 weeks (long term) after the initiation of IFX treatment using the Pediatric Ulcerative Colitis Activity Index (PUCAI). The degree of response to IFX treatment was defined as complete response (PUCAI score=0), partial response (decrement of PUCAI score${\geq}20$ points), and non-response (decrement of PUCAI score <20 points). Adverse events associated with IFX treatment were also investigated. Results: Eleven pediatric patients with moderate to severe UC had received IFX. The remission rate after IFX treatment was 46% (5/11) and 82% (9/11) at 8 weeks and 54 weeks after IFX treatment, respectively. All patients who were steroid-dependent before treatment with IFX achieved remission at 54 weeks and were able to stop treatment with corticosteroids, while all steroid-refractory patients failed to achieve remission at 54 weeks after treatment with IFX. Conclusion: Response to IFX treatment after 8 weeks may predict a favorable long-term response to IFX treatment in Korean pediatric UC patients.

• Clinical and Experimental Pediatrics
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• 제54권3호
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• pp.95-105
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• 2011

Since the successful introduction of all-trans-retinoic acid (ATRA) and its combination with anthracycline-containing chemotherapy, the prognosis for acute promyelocytic leukemia (APL) has markedly improved. With ATRA and anthracycline-based-chemotherapy, the complete remission rate is greater than 90%, and the long-term survival rate is 70-89%. Moreover, arsenic trioxide (ATO), which was introduced for APL treatment in 1994, resulted in excellent remission rates in relapsed patients with APL, and more recently, several clinical studies have been designed to explore its role in initial therapy either alone or in combination with ATRA. APL is a rare disease in children and is frequently associated with hyperleukocytosis, which is a marker for higher risk of relapse and an increased incidence of microgranular morphology. The frequency of occurrence of the promyelocytic leu-kemia/retinoic acid receptor-alpha (PML/$RAR{\alpha}$) isoforms bcr 2 and bcr 3 is higher in children than in adults. Although recent clinical studies have reported comparable long-term survival rates in patients with APL, therapy for APL in children is challenging because of the risk of early death and the potential long-term cardiac toxicity resulting from the need to use high doses of anthracyclines. Additional prospective, randomized, large clinical trials are needed to address several issues in pediatric APL and to possibly minimize or eliminate the need for chemotherapy by combining ATRA and ATO. In this review article, we discuss the molecular pathogenesis, diagnostic progress, and most recent therapeutic advances in the treatment of children with APL.

• 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.

• Asian Pacific Journal of Cancer Prevention
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• 제17권6호
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• pp.2805-2810
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• 2016

Background: Differentiated thyroid cancer is the most common endocrine malignancy with a generally good prognosis. Knowing long-term outcomes of each patient helps management planning. The study was conducted to develop and validate a clinical prognostic score for predicting disease remission in patients with differentiated thyroid cancer based on patient, tumor and treatment factors. Materials and Methods: A retrospective cohort study of 1,217 differentiated thyroid cancer patients from two tertiary-care hospitals in the Northeast of Thailand was performed. Associations between potential clinical prognostic factors and remission were tested by Cox proportional-hazards analysis in 852 patients (development cohort). The prediction score was created by summation of score points weighted from regression coefficients of independent prognostic factors. Risks of disease remission were estimated and the derived score was then validated in the remaining 365 patients (validation cohort). Results: During the median follow-up time of 58 months, 648 (76.1%) patients in the development cohort had disease remission. Five independent prognostic factors were identified with corresponding score points: duration from thyroid surgery to $^{131}I$ treatment (0.721), distant metastasis at initial diagnosis (0.801), postoperative serum thyroglobulin level (0.535), anti-thyroglobulin antibodies positivity (0.546), and adequacy of serum TSH suppression (0.293). The total risk score for each patient was calculated and three categories of remission probability were proposed: ${\leq}1.628$ points (low risk, 83% remission), 1.629-1.816 points (intermediate risk, 87% remission), and ${\geq}1.817$ points (high risk, 93% remission). The concordance (C-index) was 0.761 (95% CI 0.754-0.767). Conclusions: The clinical prognostic scoring model developed to quantify the probability of disease remission can serve as a useful tool in personalized decision making regarding treatment in differentiated thyroid cancer patients.