• Childhood Kidney Diseases
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• v.5 no.2
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• pp.117-124
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• 2001

Purpose : Since Mendoza(1990)'s report that long term methylprednisolone pulse therapy by Mendoza protocol (MP therapy) is a good treatment option in focal segmental glomerulosclerosis(FSGS), there have been reports of the effects of this therapy in steroid-resistant nephrotic syndrome. However, no studies have been performed on the effects of MP therapy in steroid- dependent nephrotic syndrome and secondary nephrotic syndrome. In this study, we investigated the effects of long term MP therapy in primary and secondary nephrotic syndrome in which previous treatment options were not effective. Methods : We chose 10 children who were diagnosed with steroid-dependent minimal change nephrotic syndrome(SD-MCNS), who had shown frequent relapse during the immunocompromised or cytotoxic therapy Period, and 6 children with FSGS and 5 children with secondary nephrotic syndrome children, who had shown no response during the previous therapy period. We treated these patients according to Mendoza protocol involving infusions of high doses of methylprednisolone, often in combination with oral cyclophosphamide for 82 weeks. Results : In all the 10 children with SD-MCNS, complete remission was visible on average of $18{\pm}9$ days after MP therapy was started. However, all these children relapsed during or after MP therapy. In these children, the mean relapse rate prior to MP therapy was $2.1{\pm}1.0$ relpases/year, which was reduced to $1.4{\pm}0.9$ relapses/year during MP therapy(P>0.05) and rose to $2.7{\pm}1.0$ relapse/year after MP therapy. Of the 6 children with FSGS, 4 children($67\%$) showed complete remission, of whom 3 children($50\%$) remained in the remission status during the follow up period, $1.2{\pm}0.7$ years, after the end of MP therapy. 2 children($33\%$) showed no response. All of the 5 children with secondary nephrotic syndrome showed remission and remained in the remissiom status during the follow up period, $1.7{\pm}0.6$ years The only side effect of MP therapy was transient hypertension in 10 children of ail subjects during the intravenous infusion of methylprednisolone. Conclusion : We conclude that although long term MP therapy is not effective in the treatment of SD-MCNS, it is an effective therapy against intractable FSGS and secondary nephrotic syndrome. (J Korean Soc Pediatr Nephrol 2001 ; 5 : 117-24)

• Journal of Sasang Constitutional Medicine
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• v.24 no.2
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• pp.61-70
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• 2012

1. Objectives : The aim of this study was to report significant improvement of chronic diarrhea and hematochezia after treatment with Dojeokgangki-tang in a Soyangin Patient with Crohn's Disease. 2. Methods : The patient's subjective and objective symptoms were observed daily throughout the hospitalization period, and the Crohn's disease activity index(CDAI) was calculated to gauge the progress or lack of progress. 3. Results : The symptoms of diarrhea and hematochezia disappeared by the end of the hospitalization period without recurrence, and the CDAI score dropped from 92.5 to 47. 4. Conclusions : A patient with Crohn's disease, who was in the state of repetitive relapse and remission after long term clinical courses was treated with Dojeokgangki-tang in a short period and the the symptoms of diarrhea and hematochezia disappeared without recurrence. Then the patient could stop taking corticosteriods and taper off immune-suppressing drugs.

• Clinical and Experimental Pediatrics
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• v.54 no.3
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• pp.106-110
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• 2011

In pediatric patients with acute lymphoblastic leukemia (ALL), the Philadelphia chromosome translocation is uncommon, with a frequency of less than 5%. However, it is classified as a high or very high risk, and only 20-30% of Philadelphia chromosome-positive (Ph+) children with ALL are cured with chemotherapy alone. Allogeneic hematopoietic stem cell transplantation from a closely matched donor cures 60% of patients in first complete remission. Recent data suggest that chemotherapy plus tyrosine kinase inhibitors (TKIs) may be the initial treatment of choice for Ph+ ALL in children. However, longer observation is required to determine whether long-term outcome with intensive imatinib and chemotherapy is indeed equivalent to that with allogeneic related or alternative donor hematopoietic stem cell transplantation (HSCT). Reports on the use of second-generation TKIs in children with Ph+ ALL are limited. A few case reports have indicated the feasibility and clinical benefit of using dasatinib as salvage therapy enabling HSCT. However, more extensive data from clinical trials are needed to determine whether the administration of second-generation TKIs in children is comparable to that in adults. Because Ph+ ALL is rare in children, the question of whether HSCT could be a dispensable part of their therapy may not be answered for some time. An international multicenter study is needed to answer the question of whether imatinib plus chemotherapy could replace sibling allogeneic HSCT in children with Ph+ ALL.

• Tuberculosis and Respiratory Diseases
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• v.81 no.1
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• pp.29-41
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• 2018

Lung cancer is one of the most commonly diagnosed cancers and the leading cause of cancer-related deaths worldwide. Although progress in the treatment of advanced non-small cell lung cancer (NSCLC) has been made over the past decade, the 5-year survival rate in patients with lung cancer remains only 10%-20%. Obviously, new therapeutic options are required for patients with advanced NSCLC and unmet medical needs. Cancer immunotherapy is an evolving treatment modality that uses a patient's own immune systems to fight cancer. Theoretically, cancer immunotherapy can result in long-term cancer remission and may not cause the same side effects as chemotherapy and radiation. Immunooncology has become an important focus of basic research as well as clinical trials for the treatment of NSCLC. Immune checkpoint inhibitors are the most promising approach for cancer immunotherapy and they have become the standard of care for patients with advanced NSCLC. This review summarizes basic tumor immunology and the relevant clinical data on immunotherapeutic approaches, especially immune checkpoint inhibitors in NSCLC.

• Annals of Clinical Neurophysiology
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• v.9 no.2
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• pp.59-62
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• 2007

Electroencephalogram (EEG) is an indispensable tool for diagnosis of epilepsy and is the only assisting barometer of complete remission of epilepsy, which means prolonged, persistent suppression of cortical excitement in epileptic focus in addition to the clinical control of epileptic seizure. The specific morphologies or distribution of epileptic form discharges give us good information for the classification of seizure or epilepsy and epileptic syndromes, which consists of "Pros." in terms of diagnostic approach. In contrast, the EEG as a tool for long-term follow up might be limited due to the various clinical situation of each patient, which consists of "Cons." in terms of the usefulness of EEG for clinical decision. "Cons." aspect of EEG, which clinicians are more frequently coped with than those of "Pros", is an obstacle of utilization of follow up EEG in clinical practice. This is an overview about controversies in usefulness of EEG and the detailed aspects of "Pros." and "Cons." of EEG for clinical decision will be discussed following two articles. We tried to make consensus for the usefulness of EEG especially in the situation of "Cons." with plausible guideline.

• Korean journal of aerospace and environmental medicine
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• v.31 no.3
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• pp.82-83
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• 2021

Chronic myeloid leukemia (CML) is myeloproliferative neoplasm associated with a characteristic chromosomal translocation (bcr-abl) called Philadelphia chromosome which plays a key role in the pathogenesis. Approximately 85% of patients with CML are in the chronic phase at the time of diagnosis. During this phase, patients are well tolerated and have few symptoms. But untreated, over the course of several years progresses to an accelerated phase and ultimately to a blast crisis, the terminal phase. CML is largely treated with targeted drug therapy called tyrosine-kinase inhibitors (TKIs) which have led to dramatically improved long-term survival rates since 2001. These drugs became standard treatment of this disease and allow most patients to have much better quality of life when compared to the former chemotherapy drugs and the bone marrow transplantation. Imatinib (Gleevec or Glivec, Norvatis) was the first of these TKIs and found to inhibit the progression of CML in the majority of patients (65%-75%) sufficiently to achieve remission. Since the advent of imatinib, CML has become the first neoplasm in which a medical treatment can give to the patient a normal life expectancy.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.48-56
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• 1999

Purpose : This multicenter collaboratory study was conducted to see the therapeutic efficacy and side effect of cyclosporine A (Cipol-$N^{(R)}$, Chong Kun Dang) on children with idiopathic nephrotic syndrome who experienced frequently relapsing (FR), steroid dependent (SD), or steroid resistant (SR) pattern. Patients and methods : Thirty-nine children with SD/FR NS and 3 children with SR NS were enrolled in the study. After induction of remission (SD/FR NS) with steroid or after 4 weeks of steroid therapy (SR NS), cyclosporine A was started in a dose of 4-5 mg/Kg/day in two divided dose and steroid (prednisolone or equivalent dose of deflazacort) was tapered slowly. During 16 weeks of study period, monthly check up of physical examination and various laboratory tests including BUN, creatinine, Ccr and cyclosporine blood level were done. Results : Out of 39 children with SD/FR NS, 35($89.7\%$) maintained sustained remission and at 4 weeks after therapy, values of serum protein, albumin, cholesterol, and 24 hours urinary protein excretion showed normal values. Two out of 3 children with SR NS showed and sustained remission with cyclosporine A therapy. Side reaction to cyclosporine A therapy showed hypertrichosis in 8 cases and hyperuricemia in 5 cases. However, other laboratory tests including CBC, liver profile, BUN, creatinine and GFR (creatinine clearance utilizing 24 hour urine) did not show any abnormalities during the 16 weeks of study period. Conclusion : Cyclosporine A (Cipoi-$N^{(R)}$ Chong Kun Dang) can be utilized quite effectively on children with SD/FR or SR NS and further trial of cyclosporine A on long-term basis (1-2 year period) is needed to determine it's efficacy and side effect (especially nephrotoxicity) of long-term administration of cyclosporine A.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.178-184
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• 2007

Purpose : Since the first report by Mendoza in 1990, there have been several studies reporting that long-term intravenous methylprednisolone(MP) pulse therapy combined with cyclosporin A(CsA) or cyclophosphamide might be beneficial for the treatment of steroid resistant focal segmental glometulosclerosis(FSGS). We investigated the therapeutic effect of long-term MP pulse therapy without CsA or cyclophosphamide on steroid resistant FSGS. Methods : The medical records of the 10 steroid resistant FSGS patients who were treated with MP pulse therapy by the Mendoza protocol without CsA or cyclophosphamide in our hospital were retrospectively reviewed. Results : The median age at onset was 2.6 years(range 1.1-10.6 years) and the median age at the initiation of therapy was 5.7 years(range 1.8-20 years). The median duration of follow-up was 35 months(range 4-132 months). At the end of therapy, 5 patients achieved complete remission(50%) and 2 partial remission(20%), one of whom relapsed after the therapy. Three patients did not respond to the therapy, two of whom progressed to end-stage renal failure during the therapy eventually requiring kidney transplantation. Conclusion : Intravenous long-term MP pulse therapy without CsA or cyclophosphamide by the Mendoza protocol may be effective in a subset of patients with steroid-resistant FSGS.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.1
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• pp.28-35
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• 2008

Purpose: The aim of this study is to report the efficacy of infliximab, a monoclonal antibody directed against tumor necrosis factor alpha which is used for both treatment of refractory pediatric Crohn disease (CD) and induction of remission. Methods: Among pediatric patients who were diagnosed with CD at Samsung Medical Center between March 2001 and August 2007, a total of 16 patients were given infliximab to treat conventional therapyresistant refractory CD and severe active CD for induction of remission. Patients needing maintenance therapy were treated with an infliximab infusion every 8 weeks, and fistulizing CD patients occasionally received the infusion upon the condition that a fistula developed. The efficacy of treatment was assessed by comparing the Pediatric Crohn Disease Activity Index (PCDAI), Hct, ESR, CRP, and serum albumin levels using paired t-test. Results: The male/female ratio was 13:3, and the median age was 13 years (range, 21 months~15 years). The patients included 7 cases of therapy-resistant refractory CD, 7 cases of severe active CD, and 2 cases of fistulizing CD. Mean PCDAI before infliximab therapy was 34.19${\pm}$14.96, and mean follow-up PCDAI within 2 to 4 weeks after the last infusion was significantly lower, at 6.88${\pm}$10.31 (p=0.000). Hematological markers such as ESR (p=0.000), serum albumin (p=0.016), and CRP (p=0.009) also improved significantly after infusion. Remission was achieved in 2 of 4 patients refractory to conventional therapy. Among 3 steroid-dependent patients, 2 were able to discontinue steroid therapy, and dose reduction was possible in 1 patient. Remission after top-down therapy without prior use of other immunomodulators was achieved in 6 weeks in all 7 of the patients who had severe CD. Nine of ten refractory fistulizing CD patients also showed improvement after infliximab therapy. Conclusion: Infliximab was effective in pediatric refractory CD for induction of remission and maintenance therapy, as well as in severe CD for top-down induction therapy. Furthermore, infliximab has contributed to steroid cessation and dose reduction. Long-term follow-up evaluation is needed to determine safety and efficacy of infliximab in the future.

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.37-47
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• 2002

Purpose: We reviewed the clinical manifestations, responsiveness to treatment, and prognosis in children with nephrotic syndrome. Methods: Medical records of 159 children with idiopathic nephrotic syndrome who were admitted to the pediatric department of Chonbuk National University Hospital from January 1979 to December 2000 w ere retrospectively reviewed. Results: There were 32 females and 127 males. The most common age group was between 3 and 5 years of age among the 159 children with nephrotic syndrome. Generalized edema ($75.5\%$), scrotal edema ($20.1\%$), upper respiratory infection ($19.5\%$), and ascites ($28.3\%$) were frequently observed. After the initial steroid therapy, diuresis occurred within tile first two weeks in 138 children, and proteinuria disappeared within the first two weeks in 105 children. Among 159 patients who received initial daily steroid therapy, 110 children were in complete remission, 29 children were in partial remission and 20 children were in poor response state. Hematuria, hypertension and elevated serum creatinine were more frequently observed in the partial and the poor response groups than in the complete remission group. Among 107 children who were followed up for more than one year, 78 children were in complete remission and 55 children were relapsed within the first one year after steroid therapy. Renal biopsy was undertaken in 76 children and 53 children had minimal change nephrotic syndronm. Conclusion: Our study showed that illost children with idiopathic nephrotic syndrome have a good responsiveness to steroid therapy and even most children show frequent relapse during 1st year after remission, long term prognosis is excellent.