• Childhood Kidney Diseases
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• v.5 no.2
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• pp.109-116
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• 2001

Purpose Long- term use of steroid, cyclophosphamide and cyclosporin, which are frequently used in the therapy of SDNS, might cause severe side effects. Recently, the immune-modulator levamisole has been tried as a substitute therapy and it has been reported as a method with less side effects and more effectiveness. We started this research in order to observe the effects of levamisole and compare it to other therapy results. Patients and Methods : We chose 16 steroid dependent nephrotic syndrome children, those who had shown frequent relapse during the immunocompromised therapy period. Mean age was $9.1{\pm}1.4$ years in children and the male to female ratio was 15:1. All of subjects were diagonized with MCNS and had received cyclophosphamide or cyclosporin before receiving levamisole. Levamisole at a dose of 2.5mg/kg was used every other day for 1 year and the relapse rate was observed. Results : On average of 14 days after treatment, complete remission was visible in all of the children, and the relapse percentage was $50\%$, which represents 8 children, while remaining 8 children representing $50\%$ of the cases showed no relapse during treatment. During the levamisole therapy period, tile average relapse rate was reduced significantly from $2.18{\pm}0.9/year\;to\;0.77{\pm}0.9/year$(P=0.027). Also the average relapse rate after the therapy was reduced to $1.34{\pm}1.1/year$, which was a significant level compared to the level before treatment(P=0.003). There was no significant difference in terms of duration of remission maintenance. Duration of remission maintenance showed an average of $12.2{\pm}9.1$ months before the use of levamisole, but it was also $10.1{\pm}6.9$ month after therapy. No other side effects such as leukopenia, skin disease and other clinically significant symptoms appeared at all during therapy. Conclusion : The long-term medication of levamisole for the therapy of SDNS children is thought to be able to maintain stable remission by reducing the relapse frequency without causing severe side effects. Further study with a broader range of subjects is required to eluccidate the long-term effects of this treatment. (J. Korean Soc Pediatr Nephrol 2001;5 : 109-16)

• Journal of Life Science
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• v.19 no.9
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• pp.1226-1231
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• 2009

Enzyme substitution with recombinant phenylalanine ammonia-lyase (PAL, EC 4.3.1.5) is currently being explored for treatment of phenylketonuria (PKU), an autosomal recessive genetic disorder with mutations of the gene encoding phenylalanine-4-hydroxylase (EC 1.14.16.1). However, oral administration of PAL is limited because of proteolytic digestion in the gastrointestinal tract. The aim of this study was to determine the biochemical properties of PAL and delinate the susceptibility of wild-type PAL to pancreatic proteolysis by exploring several mutants, and to develop therapeutic drugs with PAL for PKU. The specific activity of PAL was assayed and its optimal pH, temperature stability, and intestinal protease susceptibility were investigated. Its $V_{max}$ values for phenylalanine and tyrosine were 1.77 and $0.47{\mu}mol$/ min/mg protein, respectively, and its $K_m$ values were $4.77{\times}10^{-4}$ and $4.37{\times}10^{-4}\;M$, respectively. PAL showed an optimal pH at 8.5, corresponding to the average pH range of the small intestine. It showed no loss of activity at $-80^{\circ}C$ for 5 months and possessed 93.4% of its activity under $4^{\circ}C$ for 4 wks. PAL was susceptible to chymotrypsin digestion and, to a lesser extent, to trypsin, elastase, carboxypeptidase A, and B. The trypsin and chymotrypsin cleaving sites were mutated to investigate protection from pancreatic digestion and the specific activities of these mutants were evaluated. The six mutants displayed low specific activities compared to the wild-type, suggesting that the primary trypsin and chymotrypsin cleaving sites may be essential for catalytic reaction. The PAL mutants could therefore be applied as a pretreatment modality without susceptibility to proteolytic attack, however, additional modification for enhancing enzymatic activity is needed to reduce the Phe levels effectively.

• Journal of Chest Surgery
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• v.41 no.4
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• pp.430-438
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• 2008

Background: All the patients with mechanical valves require warfarin therapy in order to prevent them from developing thromboembolic complications. According to the ACC/AHA practice guidelines, after AVR with bileaflet mechanical prostheses in patients with no risk factors, warfarin is indicated to achieve an INR of 2.0 to 3.0. After MVR with any mechanical valve, warfarin is indicated to achieve an INR of 2.5 to 3.5. But in our clinical experience, bleeding complications (epistaxis, hematuria, uterine bleeding, intracerebral hemorrhage etc.) frequently developed in patients who maintained their INR within this value. So, we retrospectively reviewed the patients with bileaflet mechanical heart valve prosthesis and we determined the optimal anticoagulation value. Material and Method: From January 1984 to February 2007, 311 patients have been followed up at a national medical center. We classified the AVR patients (n=60) into three groups as follows: an INR from 1.5 to 2.0 in Group I, an INR from 2.0 to 2.5 in Group II and an INR from 2.5 to 3.0 in Group III. We classified the MVR (n=171) and DVR (n=80) patients into four groups as follows: an INR from 1.5 to 2.0 in Group I, an INR from 2.0 to 2.5 in Group II, an INR from 2.5 to 3.0 in Group III and an INR from 3.0 to 3.5 in Group III. We compared the groups for their thromboembolic and bleeding complications by means of the Kaplan Meier method. Result: In the AVR patients, 2 thromboembolic complications and 4 bleeding complications occurred and the log rank test failed to identify any statistical significance between the groups for thethromboembolic complication rate, but groups I and II had lower bleeding complication rates than did group III. Thirteen thromboembolic complication and 15 bleeding complication occurred in the MVR and DVR patients, and the log rank test also failed to identify statistical significance between the groups for the thromboembolic complication rate, but groups I and II had lower bleeding complication rates that did groups III and IV. Conclusion: The thromboembolic complication rate was not statistically different between groups I and II and groups III and IV, but the bleeding complication rates of groups I and II were lower than those of groups III and IV. So this outcome encouraged us to continue using our low intensive anticoagulation regime, that is, an INR of 1.5 to 2.5.

• Clinical and Experimental Pediatrics
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• v.51 no.2
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• pp.162-169
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• 2008

Purpose : Oxcabarzepine (OXC), newly recommended antiepileptic drug, has been prescribed for patients with partial seizures and generalized tonic clonic seizures in Korea from 1999. There are limited reports about an efficacy of OXC therapy in epileptic children in Korea. This study evaluated the efficacy and safety of OXC in the light of our experience. Methods : The patients, who had visited the pediatric neurology clinic of Korea University Guro Hospital from January 2001 to December 2006, were included. The data of 144 patients who were administrated OXC as monotherapy or polytherapy, was summarized retrospectively and we evaluated the efficacy and safety of OXC. Results : After 6 months of OXC therapy, 77 patients (53.5%, n=144) achieved seizure freedom, 48 patients (33.3%) experienced >50% improvement. After 12 months of OXC therapy, cessation of seizure was observed in 88 patients (61.1%, n=133), and 27 patients (18.8%) manifested an improvement. Monotherapy group showed superior efficacy to polytherapy one. The frequent side effects of OXC were drowsiness (20.1%), headache (12.5%), dizziness (9.7%) and rash (8.3%). They did not related to patient's age or sex, and dosage of OXC. Twenty four patients (16.7%) experienced hyponatremia, but which were neither symptomatic nor significant one. Conclusion : The efficacy and safety of OXC in our patients were excellent and had less significant side effects than established international one. We expect this report contributes toward OXC therapy in epileptic children.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.256-260
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• 2009

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease affecting mostly children. This disorder is characterized by recurrent episodes of hemoptysis, bilateral diffuse pulmonary infiltrates, and iron-deficiency anemia. An acute fulminant alveolar hemorrhage can be fatal due to respiratory failure, while chronic hemorrhage leads to hemosiderin-laden macrophages and pulmonary fibrosis. Genetic, autoimmune, allergic, environmental, and metabolic mechanisms of pathogenesis have been suggested, but the etiology of IPH remains unknown. We report on a 9-year-old girl with idiopathic pulmonary hemosiderosis who showed seasonal recurrences without cause.

• The Journal of Internal Korean Medicine
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• v.37 no.3
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• pp.539-547
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• 2016

Myelodysplastic syndrome (MDS) is a typical myeloid malignancy characterized by cell dysplasia in bone marrow. Currently, there is no therapeutic treatment for MDS. The only available therapies either relieve symptoms or prevent the development of acute myeloid leukemia (AML). This study aimed to report the effects of traditional Korean medicine (TKM) on MDS by presenting two case reports. The patient in Case 1 was diagnosed with primary MDS and regularly received acupuncture treatments and herbal medicine. In Case 2, a patient with ovarian cancer was diagnosed with secondary MDS, which resulted from an adverse reaction to chemotherapy. This patient took herbal medicine for four years and was hospitalized three times. In order to have their condition evaluated, both patients underwent regular blood tests. The patient in Case 1, who showed blood transfusion dependency, received only two blood transfusions after TKM treatment, and the person’s health condition was stable as of January 2016 without any signs of AML development. The patient in Case 2 also has stable health condition. TKM treatment effectively treated their MDS symptoms and improved their general health conditions without any adverse effects. It also prevented the rapid development of AML and maximized the effects of conservative therapy.

• Journal of The Korean Society of Clinical Toxicology
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• v.7 no.2
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• pp.164-171
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• 2009

Purpose: The number of patients who take warfarin is growing and so is the number of complications. Hemorrhage is the major complication, but the clinical characteristics and outcomes have not been determined for Korean patients. Therefore, we tried to evaluate the characteristics of the patients with hemorrhagic complications after taking warfarin as anticoagulation therapy. Methods: We retrospectively reviewed the medical records of the patients who visited the emergency room with bleeding complications after taking warfarin anticoagulation at the out-patient clinic for 1 year from 1 st January 2008. We compared between two groups (the major hemorrhage group vs. the minor hemorrhage group) according to the clinical criteria, the unstable vital signs that required blood transfusion, transfusion more than 2 units of blood, the need for further laboratory follow-up, the need for interventional treatment and the development of critical complications or death due to bleeding. Results: There were 150 patients who met the criteria and had acute hemorrhagic complications (the major group: 90 patients and the minor group: 60 patients). In the major hemorrhage group, the frequent sites of bleeding were the gastro-intestinal system (40 patients), lung (14 patients) and intracranium (7 patients). At the emergency room, the major group showed a higher initial INR of the activated prothrombin time than did the minor group (p=0.02). The bleeding sites of the fatal cases were the gastro-intestinal system (3 patients), lung (3 patients) and intracranium (3 patients), but the percentage of fatality was the highest for intracranium bleeding. Conclusion: In the major hemorrhage group, gastrointestinal bleeding was the most frequent complication and fatality was the highest for intracranium bleeding. An initially higher INR showed a greater risk of major bleeding, but not more fatalities.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.10 no.2
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• pp.244-251
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• 1999

Self-injurious behavior is often showed in mental retardation, especially in autism. Self-injurious behavior has been regarded as a symptom cluster rather than a disease but it is an emergent clinical situation that can directly affect mortality. This case is about a refractory autistic patient who showed a self-injurious behavior of hitting the head repetitively. He was hospitalized and was treated by pharmacotherapy and behavior therapy and for this reason this clinical experience is reported with literature review. The patient is a 7-year old boy who was ward admitted from 1999 April 20 till July 10 into OO hospital OO ward because of self-injurious behavior. During the 12 weeks he had admission treatment. As for the pharmacotherapy, haloperidol was dosed up from 0.5mg to 1.0mg from the 4th week and combination drug therapy was done during the admission with naltrexone 25-50mg. As for the behavioral therapy, Differential Reinforcement of Other behavior was used and regular play therapy was done. To remove the physical restraint, headgear and hard sleeve was used. Currently, OPD follow up treatment is being done and haloperidol 0.5mg and naltrexone 50mg is maintained. The patient’s mother is educated and play therapy is done an hour daily at home. When the patient was released form the hospital, self-injurious behavior was decreased more than the moderate state and remission state is still being maintained at the outpatient clinic.

• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.701-708
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• 1996

Background : To evaluate the effect of the balloon dilatation in tuberculous bronchial stenosis, we performed balloon dilatation in 13 cases which had airway obstruction in main bronchus with the impairment of pulmonary function. Material and Methods: Thirteen women with tuberculous bronchial stenosis(9cases : left main bronchus, 4 cases: right main bronchus) underwent fluoroscopically guided balloon dilatation under the local anesthesia. Among the these patient, 9 cases were active endobronchial tuberculosis, and 4 cases were inactive. Immediate and long term follow-up(average 15.6months) assessments were done focused on change on PIT. The increase of FVC or FEV1 more than 15% after the procedure was considered effective. Complications after dilatation were evaluated in all patients. Result : 1) There were an decrease of self-audible wheezing in 75%(6/8), improvement of dyspnea in 62.5%(5/8), improvement of cough and expectoration in 50%(3/6), and improvement of chest discomfort in 50%(1/2). 2) Significant improvement of PFT was noted in 42.9%(3/7) of which respiratory symptoms duration was below 6 months. 8m, significant improvement of PFT was noted in only 25%(1/4) of which respiratory symptoms duration was above 12 months. 3) Active stage was 69.2%(9/13) and inactive was 30.8%(4/13). There was an significant improvement of PFT in 44.4%(4/9) of active stage, but, only 25%(1/4) of inactive stage was improved. 4) In 61.5%(8/13), FVC and FEV1 were increased to 35.5%, and 22.2% at post-dilatation 7 days. After 1 month later, FVC and FEV1 were increased to 54.7%, and 31.8% in 5 cases(38.5%). 4 cases in which long-term follow-up(average 19.8months) was possible the improvement of FVC, and FEV1 were 30.5%, and 10.1%. 5) Just after balloon dilatation therapy, transient leukocytosis or fever was noted in 30.8%(4/13), and blood-tinged sputum was noted in 30.8%(4/13). However, serious complication, such as pneumothorax, pneumomediastinum or mediastinitis, was not noted. Conclusion : We conclude that tuberculous bronchial stenosis, which is on active stage, and short dulation of respiratory symptoms was more effective on balloon dilatation than inactive stage or long duration of respiratory symptoms. Furthermore, balloon dilatation is easier, much less invasive and expensive than open surgery. and cryotherapy or photoresection. Because of these advantage, we think that balloon dilatation could be the first choice for treating bronchial stenosis and could be done at first in early stage if unresponsiveness with steroid therapy is observed.

• Tuberculosis and Respiratory Diseases
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• v.60 no.4
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• pp.419-425
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• 2006

Background : Severe community-acquired pneumonia (CAP) can develop into respiratory failure that requires mechanical ventilation (MV), which is associated with a higher rate of mortality. It was recently reported that a hydrocortisone infusion in severe CAP patients was associated with a significant reduction in the length of the hospital stay and mortality. This study evaluated efficacy of a hydrocortisone infusion for patients with severe CAP requiring MV. Methods : From February 2005 to July 2005, 13 patients (M : F = 10 : 3, mean age: $68.6{\pm}14.1years$), who were diagnosed with severe CAP and required MV, were enrolled in this study. Hydrocortisone was administered as an intravenous 200mg loading bolus, which was followed by an infusion at a rate of 10mg/hour for 7 days. The control group was comprised of patients with severe CAP requiring MV but in whom corticosteroid was not used before study period. The clinical and physiologic parameters on or by day 8 and the outcome in the hydrocortisone infusion group were compared with those in the control group. Results : 1) There was no significant difference in age, gender ratio, SAPS II, SOFA score, temperature, leukocyte count, PaO2/FiO2 (P/F) ratio, the number of patients with P/F ratio < 200, chest radiograph score, lung injury score and catecholamine-dependent septic shock between the hydrocortisone infusion group and control group at day 1. 2) At day 8, the proportion of patients with an improvement in the P/F ratio ${\geq}100$ and the chest radiograph score was significantly higher in the hydrocortisone infusion group than in the control group (61.5% vs. 15.4%, 76.9% vs. 23.1%, p< 0.05). However, there was no significant difference in the other clinical and physiologic parameters. 3). There was no significant difference in the duration of the MV, ICU stay, hospital stay and 10th and 30th day mortality between the two groups. Conclusion : Hydrocortisone infusion for patients with severe CAP requiring invasive mechanical ventilation may be effective in improving the level of oxygenation and the chest radiograph score.