• Clinics in Shoulder and Elbow
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• 제11권2호
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• pp.189-192
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• 2008

흉쇄 관절의 화농성 관절염은 드문 질환으로 정맥주사 약물 남용, 당뇨 그리고 외상 등과 같은 선행성 요인과 연관되어 주로 발생한다. 진단이 지연될 경우 종격동염, 흉벽 농양 등과 같은 위험한 합병증이 발생할 수 있으므로 전산화 단층촬영이나 자기공명영상 검사 등을 시행하여야 하고 종격동염 및 흉벽 농양 등과 같은 합병증 발생 시는 흉쇄 관절 절제술을 고려하여야 한다. 저자들은 감염 유발 소인이 전혀 없는 건강한 52세 남자에서 발생하였으며 균 배양 검사상 포도상구균으로 동정되었고 감수성 항생제인 cefminox(첫 4주는 정맥주사, 그 후 2주는 경구투여)만으로 치유되었던 일차성 흉쇄 관절 화농성 관절염에 대해 보고하는 바이다.

• Asian Pacific Journal of Cancer Prevention
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• 제16권6호
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• pp.2391-2395
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• 2015

Objective: The aim of this retrospective study was to evaluate the feasibility and efficacy of response to continuous-infusion ifosfamide and doxorubicin combination as second-line chemotherapy for patients with recurrent or refractory osteosarcoma. Materials and Methods: Eighteen recurrent or refractory osteosarcoma patients who were treated with continuous-infusion ifosfamide and doxorubicin combination between May 1999 and April 2011 were included in the analysis. Ifosfamide at $12g/m^2$ was administered by intravenous continuous infusion over 3 days, and doxorubicin $60mg/m^2$ was administered as an intravenous bolus injection on day 1. The combination therapy was repeated every 3 weeks. Treatment was continued until evidence of disease progression or unacceptable toxicity. Results: The patients (ages 7-53 years) received a total of 42 cycles of chemotherapy (median: 2 courses; range: 2-5 courses). The overall response rate was 0% and the disease control rate was 22.3%, with four patients having stable disease. The median time to progression and overall survival time were 2 months (range: 2-5 months) and 9 months (range: 3-29 months), respectively. Major severe toxicities were leucopenia 7 (38.9%), nausea and vomiting 3 (16.7%) and alopecia 9 (50%). There were no treatment-related deaths. Conclusions: In our experience, continuous-infusion ifosfamide and doxorubicin combination therapy at this dosage and schedule was found to be well tolerated and moderate effective, which could be considered as salvage therapy for patients with recurrent or refractory osteosarcoma. Further assessment is necessary to confirm the safety and efficacy of this treatment.

• Childhood Kidney Diseases
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• 제14권2호
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• pp.230-235
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• 2010

경정맥 고용량 스테로이드 충격요법(IMPT)의 부작용으로는 고혈압, 동성 서맥, 심방심실 전도장애, 심방 세동, 심방 조동, 심실 빈맥 등의 부정맥, 구토, 구역질 등의 소화기 장애, 백내장, 저칼륨혈증, 그리고 감염성 질환 등이 있다. 그중, 저칼륨혈증은 IMPT를 받는 환자의 17% 정도에서 경미하게 나타날 수 있다. 저칼륨혈증이 신수질의 요농축 능력을 저하시켜 다뇨가 발생할 수 있다는 사실은 이미 알려져 있지만, IMPT후 경미한 저칼륨혈증으로 인해 심한 야간뇨과 다뇨증이 발생하였다는 보고는 별로 없다. 이에 본 저자들은 다량의 단백뇨와 혈뇨를 보이는 HSP 신염환자에게 세 차례의 IMPT 시행 후 환아에게 발생한 경미한 저칼륨혈증으로 인한 심한 야간뇨와 다뇨증의 발생을 경험하였기에 이에 보고하는 바이다.

• Pediatric Infection and Vaccine
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• 제8권1호
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• pp.101-106
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• 2001

목 적 : 홍역은 바이러스성 질환으로 강한 전염성을 가지고 있으며, 최근 홍역이 대유행을 하고 있으나, 대증 요법만으로는 효과적인 치료를 할 수 없어 강력한 항 염증작용과 면역 조절 기능을 가진 정주용 인면역글로부린(intravenous-gammaglobulin)을 투여하여, 현저하고 신속한 임상 증상의 호전과 급성기 염증 반응의 소실을 경험하였기에 보고하는 바이다. 방 법 : 2000년 1월부터 2001년 2월까지 안양 메트로병원에 홍역으로 입원한 112명 중 치료군으로 선정된 68명에게 정주용 인면역글로부린(400~500 mg/kg)을 한번에 투여하였고, 대조군 44명은 대증요법과 수액제제 등으로 치료하여 두 군간의 발열 기간, 홍반성 구진의 소실 시기, 입원 기간 그리고 CRP의 출현 시기 등을 비교 분석하였다. 결 과 : 환자들의 평균 연령은 $7.9{\pm}3.6$세였고, 남녀 비는 1.0 : 1.6으로 여아가 많았으며, 입원 중 발열 기간은 치료군에서 평균 $2.4{\pm}1.2$일, 대조군은 $5.7{\pm}2.4$일, 홍반성 구진의 소실 시기는 치료군에서는 평균 $4.5{\pm}1.3$일, 대조군은 $6.9{\pm}2.4$일, 평균 입원 기간은 치료군에서 $3.5{\pm}1.3$일, 대조군은 $7.8{\pm}3.2$일로 치료군에서 의미 있게 신속한 임상 증상의 호전을 보였고, 입원기간도 현저하게 짧았다(P<0.05). CRP(정상 <5 mg/L)는 치료 전에는 두 군간에 차이가 없었으나 치료 후 5일째에 측정한 수치가 치료군에서는 평균 $6.2{\pm}1.4mg/L$로 대부분 정상이 되었지만 대조군은 $12.4{\pm}4.2mg/L$로 대조군에서 유의하게 높게 측정되었다(P<0.05). 결 론 : 중증 감염의 치료에 사용하는 정주용 인면역글로부린은 비교적 안전하며 부작용이 거의 없고 현저하고 급속한 치료 효과를 얻을 수 있어 급성기 홍역 환자를 치료하는데 대단히 유용하다고 생각되었다.

• Journal of Genetic Medicine
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• 제20권1호
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• pp.6-14
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• 2023

Fabry disease (FD), a rare X-linked lysosomal storage disorder, is caused by mutations in the α-galactosidase A gene gene encoding α-galactosidase A (α-Gal A). The functional deficiency of α-Gal A results in progressive accumulation of neutral glycosphingolipids, causing multi-organ damages including cardiac, renal, cerebrovascular systems. The current treatment is comprised of enzyme replacement therapy (ERT), oral pharmacological chaperone therapy and adjunctive supportive therapy. ERT has been introduced 20 years ago, changing the outcome of FD patients with proven effectiveness. However, FD patients have many unmet needs. ERT needs a life-long intravenous therapy, inefficient bio-distribution, and generation of anti-drug antibodies. Migalastat, a pharmacological chaperone, augmenting α-Gal A enzyme activity only in patients with mutations amenable to the therapy, is now available for clinical practice. Furthermore, these therapies should be initiated before the organ damage becomes irreversible. Development of novel drugs aim at improving the clinical effectiveness and convenience of therapy. Clinical trial of next generation ERT is underway. Polyethylene glycolylated enzyme has a longer half-life and potentially reduced antigenicity, compared with standard preparations with longer dosing interval. Moss-derived enzyme has a higher affinity for mannose receptors, and seems to have more efficient access to podocytes of kidney which is relatively resistant to reach by conventional ERT. Substrate reduction therapy is currently under clinical trial. Gene therapy has now been started in several clinical trials using in vivo and ex vivo technologies. Early results are emerging. Other strategic approaches at preclinical research level are stem cell-based therapy with genome editing and systemic mRNA therapy.

• Journal of Audiology & Otology
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• 제24권3호
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• pp.127-132
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• 2020

Background and Objectives: In this study, we compared the outcomes of patients with idiopathic sudden sensorineural hearing loss who underwent steroid treatment with or without hyperbaric oxygen (HBO) therapy and were followed-up in our clinic. Subjects and Methods: Patients were divided into two groups according to their treatment regimen. Steroid group received intravenous 1 mg/kg methylprednisolone which was due to be completed in 2-3 weeks with decreasing doses, and five doses of 0.5 mL intratympanic dexamethasone. Steroid+HBO group received the same steroid treatment with the addition of HBO therapy. The audiologic results of both treatment groups were compared after considering the patients' risk factors. Results: There was no significant difference between the steroid and Steroid+HBO groups in terms of hearing gain and degree of recovery, both at all degrees of hearing loss, and in severe and profound hearing loss. Hearing gain was similar when evaluated by audiogram type and admission time in both treatment groups. Conclusions: We found that the addition of HBO therapy to systemic plus intratympanic steroid treatment did not affect hearing gain at all degrees of hearing loss in this study. Furthermore, audiogram type and admission time did not affect hearing gain between the two groups.

• 대한청각학회지
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• 제24권3호
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• pp.127-132
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• 2020

Background and Objectives: In this study, we compared the outcomes of patients with idiopathic sudden sensorineural hearing loss who underwent steroid treatment with or without hyperbaric oxygen (HBO) therapy and were followed-up in our clinic. Subjects and Methods: Patients were divided into two groups according to their treatment regimen. Steroid group received intravenous 1 mg/kg methylprednisolone which was due to be completed in 2-3 weeks with decreasing doses, and five doses of 0.5 mL intratympanic dexamethasone. Steroid+HBO group received the same steroid treatment with the addition of HBO therapy. The audiologic results of both treatment groups were compared after considering the patients' risk factors. Results: There was no significant difference between the steroid and Steroid+HBO groups in terms of hearing gain and degree of recovery, both at all degrees of hearing loss, and in severe and profound hearing loss. Hearing gain was similar when evaluated by audiogram type and admission time in both treatment groups. Conclusions: We found that the addition of HBO therapy to systemic plus intratympanic steroid treatment did not affect hearing gain at all degrees of hearing loss in this study. Furthermore, audiogram type and admission time did not affect hearing gain between the two groups.

• Childhood Kidney Diseases
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• 제5권2호
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• pp.117-124
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• 2001

목 적 : 1990년 Mendoza등이 스테로이드 저항성 신증후군에서 Mendoza protocol에 의한 장기간의 methylprednisolone pulse therapy (이하 MP 요법)가 관해를 유도하고 유지시키는 효과적인 치료방법인 것으로 보고한 이후, 스테로이드 저항성 신증후군에서 이 치료법의 효과에 관한 보고는 있어 왔지만 스테로이드 의존성 신증후군과 2차성 신증후군에서 이 치료법의 효과에 관한 보고는 아직까지 없는 실정이다. 본 연구에서는 면역억제제 또는 세포독성물질로도 스테로이드 의존성을 없애거나 재발율을 낮출 수 없었던 스테로이드 의존성 미세변화형 신증후군과 난치성 국소성 분절성 사구체 경화증 및 2차성 신증후군에서 Mendoza protocol에 따른 장기간의 MP 요법의 치료 효과를 알아보고자 하였다. 대상 및 방법 : 1997년부터 2000년사이에 세브란스병원 소아과에서 신생검에 의해 1차성 또는 2차성 신증후군을 진단받고 면역억제제 또는 세포독성 물질 치료로 스테로이드 의존성을 없애거나 재발률을 낮출 수 없었던 스테로이드 의존성 미세변화형 신증후군과 기존의 스테로이드 및 면역억제제 치료에 반응을 보이지 않았던 난치성 국소성 결절성 사구체 경화증 및 2차성 신증후군 환아 21명을 대상으로 하여 Mendoza protocol에 따라 82주 동안 MP요법을 시행하였다. 결 과 : 대상 환아를 신조직검사 소견에 따라 분류하면 1차성 신증후군이 16례($76\%$)로 이중 미세변화형 신증후군이 10례, 국소성 분절성 사구체 경화증이 6례였으며, 2차성 신증후군은 5례($24\%$)였다. 스테로이드 의존성 미세변화형 신증후군 10례 모두 ($100\%$)에서 MP요법 시작후 평균 $18{\pm}9일$ 내에 완전관해가 되었으나 전례에서 치료중 또는 치료후에 재발하였고, 연간 평균 재발횟수가 치료전에 $2.1{\pm}1.0회$, 치료중에 $1.4{\pm}0.9회$로 치료전보다 감소하였으나 통계적 의미는 없었고 치료후에는 $2.7{\pm}1.2회$로 치료전보다 증가하였다. 국소성 분절성 사구체 경화증 6례중 3례($50\%$)에서 완전관해, 1례($17\%$)에서 부분관해가 유도되었고 2례($33\%$)에서 여전히 반응하지 않았으며, 치료후 평균 $1.2{\pm}0.7$년간 추적관찰 기간 동안 관해된 4례($57\%$)중 3례($50\%$)에서 관해가 유지되었고 1례에서 재발하였다. 2차성 신증후군 5례중 4례($80\%$)에서 완전관해, 1례($20\%$)에서 부분 관해가 유도되었고 치료후 평균 $1.7{\pm}0.6$년간 추적 관찰 기간 동안 재발한 예는 없었다. MP요법의 부작용으로는 21례중 10례($48\%$)에서 methylprednisolone 정맥주사시 일시적인 고혈압이 있었다. 결 론 : Mendoza protocol에 의한 장기간의 IV-methylprednisolone pulse therapy는 난치성 국소성 분절성 사구체경화증과 2차성 신증후군의 관해유도와 관해유지에는 효과가 있는 것으로 생각되었으며 면역억제제 또는 세포독성물질로도 스테로이드 의존성을 없애거나 재발율을 낮출 수 없었던 스테로이드 의존성 미세변화형 신증후군의 치료에서는 스테로이드 의존성 혹은 재발율의 변화를 나타내지 못하였다. 따라서 장기간의 MP 요법은 스테로이드 및 면역억제제 치료에 저항성을 보이는 1차성 또는 2차성 신증후군의 치료에 시도해볼만한 치료법으로 생각된다.

• Journal of Korean Biological Nursing Science
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• 제4권2호
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• pp.113-125
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• 2002

This study is to evaluate contamination of the 3-way stopcocks connected to infusion set, which is used to the patients admitted to emergency room in a general hospital in D city. The data were collected from Oct. 1, 2001 to Feb. 25, 2002. First of all, in order to select microorganisms, From the 50 patients were randomized, Coagulase Negative Staphylococcus, Staphylococcus aureus, micrococcus, Pseudomonas aeruginosa, Acinetobacter detected. Coagulase Negative Staphylococcus, Staphylococcus aureus, micrococcus were determined to be evaluated in this study. As a result, 8 of the patients were Coagulase Negative Staphylococcus positive(>15 colonies), 4 were Staphylococcus aureus positive(>15 colonies). 1 was micrococcus positive(>15 colonies). Among the patients who were Coagulase Negative Staphylococcus positive 112(average) colonies were detected on the first day, 429 were on the second day, and 563 were on the third day. In case of patients of Staphylococcus aureus positive, 85(average) colonies were detected on the first day, 151 were on the second day, and the 203 were on the third day. CNS was cultured using API kit for the 8 patients who were in CNS positive. One case was detected Staphylococcus capitis, another one case was Staphylococcus chromogenes. Two cases were Staphylococcus xylosus, another two were Staphylococcus hominis, and the remainer were Staphylococcus epidermidis. As a result, the API codes of two Staphylococcus epidermidis had shown the same pattern, and the resistance patterns of the them were the same, too. As a result of resistance test among 5 patients who have shown that the same resistance pattern in 02SAK1, 02SAK5, 02SAK2, 02SAK4. As a result of this study, aseptic technique of 3-way stopcock intravenous therapy can protect infections, and it is needed the sterilization of the 3-way stopcock just before injection using disinfectants. It needs to improve the 3 way stopcock change intervals from 48 hours.

• Korean Journal of Radiology
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• 제23권6호
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• pp.664-673
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• 2022

Objective: To evaluate the performance of baseline clinical characteristics and pretherapeutic histogram parameters derived from T2 mapping of the extraocular muscles (EOMs) in the prediction of treatment response to intravenous glucocorticoid (IVGC) therapy for active and moderate-to-severe thyroid-associated ophthalmopathy (TAO) and to investigate the effect of fat-suppression (FS) in T2 mapping in this prediction. Materials and Methods: A total of 79 patients clinically diagnosed with active, moderate-to-severe TAO (47 female, 32 male; mean age ± standard deviation, 46.1 ± 10 years), including 43 patients with a total of 86 orbits in the responsive group and 36 patients with a total of 72 orbits in the unresponsive group, were enrolled. Baseline clinical characteristics and pretherapeutic histogram parameters derived from T2 mapping with FS (i.e., FS T2 mapping) or without FS (i.e., conventional T2 mapping) of EOMs were compared between the two groups. Independent predictors of treatment response to IVGC were identified using multivariable analysis. Receiver operating characteristic (ROC) curve analysis was performed to evaluate the predictive performance of the prediction models. Differences between the models were examined using the DeLong test. Results: Compared to the unresponsive group, the responsive group had a shorter disease duration, lower kurtosis (FS-kurtosis), lower standard deviation, larger 75th, 90th, and 95th (FS-95th) T2 relaxation times in FS mapping and lower kurtosis in conventional T2 mapping. Multivariable analysis revealed that disease duration, FS-95th percentile, and FS-kurtosis were independent predictors of treatment response. The combined model, integrating all identified predictors, had an optimized area under the ROC curve of 0.797, 88.4% sensitivity, and 62.5% specificity, which were significantly superior to those of the imaging model (p = 0.013). Conclusion: An integrated combination of disease duration, FS-95th percentile, and FS-kurtosis was a potential predictor of treatment response to IVGC in patients with active and moderate-to-severe TAO. FS T2 mapping was superior to conventional T2 mapping in terms of prediction.