• Title/Summary/Keyword: CGD

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Chronic Granulomatous Disease on Jeju Island, Korea

  • Cho, Moonjae;Shin, Kyung-Sue
    • Journal of Genetic Medicine
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    • v.10 no.1
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    • pp.1-6
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    • 2013
  • Chronic granulomatous disease (CGD) is a rare inherited disorder of a defective NADPH oxidase enzyme, resulting in very low or no production of superoxide and subsequent reactive oxygen species. Consequently, patients with CGD are highly susceptible to severe bacterial and fungal infections. CGD is a genetically heterogeneous disease caused by defects in any one of the genes encoding the NADPH oxidase components. CGD generally affects about 3-4 per 1,000,000 individuals; thus, it is surprising that the prevalence of CGD on Jeju Island is 34.3 per 1,000,000 individuals. At present, 20 patients with CGD from 14 unrelated families on Jeju Island have been identified; nine males and 11 females. All patients with CGD tested on Jeju Island had an identical and homozygous mutation (c.7C>T in CYBA, p.Q3X in $p22^{phox}$). Therefore, all patients were autosomal recessive form of CGD. This strongly suggests that the unique and identical mutation in CYBA may be inherited from a common proband. Using mutation-specific primers to detect the mutated allele in CYBA, the frequency of subjects carrying a mutated allele was 1.3% of enrolled subjects from Seogwipo City. Further studies are necessary to elucidate how frequently this mutant allele occurs in the population on Jeju Island. Additionally, it is important to construct a national registry system to understand the pathophysiology of CGD and develop a strategy for long-term therapy.

Concomitant use of corticosteroid and antimicrobials for liver abscesses in patients with chronic granulomatous disease

  • Shin, Kyung-Sue;Lee, Mu Suk
    • Clinical and Experimental Pediatrics
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    • v.59 no.4
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    • pp.196-201
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    • 2016
  • Chronic granulomatous disease (CGD) is a rare inherited disorder caused by defective nicotinamide adenine dinucleotide phosphate oxidase enzyme and characterized by recurrent bacterial and fungal infections. Although liver abscess is a common manifestation of CGD, its management in CGD patients is not well-defined. In addition, the generalized guidelines for treating liver abscesses do not necessarily apply to CGD patients. Corticosteroids are commonly used to control granulomatous complications, such as inflammatory gastrointestinal and genitourinary lesions, in patients with CGD, Corticosteroids have also been used in combination with antimicrobials to treat refractory infections in patients with CGD. Because corticosteroids are capable of suppressing symptomatic inflammation, all potential infections must be adequately controlled prior to corticosteroid initiation. We report 3 typical CGD cases with liver abscesses refractory to conventional treatments that were successfully treated with the concomitant use of corticosteroid and antimicrobials. It remains unclear whether corticosteroid therapy is required for liver abscesses in CGD refractory to conventional treatments. However, based on our observations, use of corticosteroids in combination with optimal antimicrobials should be considered for refractory liver abscesses in CGD.

컴퓨터와 산업디자인과의 고찰

  • Park, Chan-Beom;Kim, In-Su
    • Electronics and Telecommunications Trends
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    • v.4 no.3
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    • pp.133-145
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    • 1989
  • 우리는 컴퓨터와 인간이 교환하는 모든 시각 정보 개념을 주로 컴퓨터 Graphics로 총칭하고 있으나, 실제적으로 CAD와 CGD의 2가지 분야로 구분하여야 하는데, CAD와 CGD는 이용매체의 유사함으로 인하여 두 용어사이에 자주 혼동을 가져온 것 같다. CAD는 공학중심으로 CGD는 미학중심으로 이루어졌는데, 현 추세에 있어서 상호보완적, 의존적으로 발달되고 있는 바 CAID의 발생이 그것이다. 본문에서는 우리 연구소에서 주로 전자분야에 적용하고 있는 CAD시스팀 영역외에 산업디자인 분야에서의 컴퓨터를 이용한 CGD 및 CAD(CAID) 시스팀의 발전 동향 및 기술동향을 소개함으로써 산업디자인에 있어서의 그래픽 디자인의 이해를 돕고자 한다.

A Case of Esophageal Obstruction Complicated in a Patient with Chronic Granulomatous Disease - Esophageal obstruction in Chronic Granulomatous Disease (만성육아종질환 환자에서 합병된 식도 폐쇄 사례 -만성육아종질환의 육아종성 식도 폐쇄)

  • Kim, Ji Soo;Hwang, Jinsol;Choi, Young Hun;Kim, Woo Sun;Kim, Joong Gon
    • Pediatric Infection and Vaccine
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    • v.21 no.1
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    • pp.53-58
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    • 2014
  • Chronic granulomatous disease (CGD) is an inherited immunodeficient disease characterized by recurrent infections and granuloma formation. Granulomatous obstruction of esophagus is one of the rare complications of CGD. The use of steroids and antimicrobials for esophageal obstruction by granuloma in CGD patients has been controversial due to the possibility of concomitant inapparent infection. We report a case of esophageal obstruction in an 8-year-old CGD patient showing the poor response to antibiotics therapy. However, dramatic improvement of symptoms and radiologic findings of esophageal obstruction were achieved after steroid therapy. One month after discontinuation of steroid, esophageal obstruction recurred and the patient was re-treated with steroid. After that time, he experienced one more recurrence of esophageal obstruction. This symptom subsided after antibiotics therapy without steroid and he has been followed up to the present without further relapse.

Herbal Medicine and Local Acupuncture Point Stimulation Administration and Their Clinical Effect on Quality of Life for Cervicogenic Dizziness (CGD) Patient: A CARE Guideline Compliant Case Report (경추성 현훈 환자의 복합 한의진료 경과 및 삶의 질 변화 : CARE 가이드라인에 따른 증례보고)

  • Kim, Eunmi;Jo, Hee-Geun
    • The Journal of Internal Korean Medicine
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    • v.41 no.3
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    • pp.414-423
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    • 2020
  • Background: This study examines the clinical effect on quality of life of herbal medicine and local acupuncture point stimulation administration for a cervicogenic dizziness (CGD) patient. This case report was described in accordance with CARE guidelines. Case Report: A 25 year-old female patient suspected to be suffering from CGD was examined. The patient was treated with herbal medicine and local acupuncture point stimulation. We used the Headache Impact Test-6 (HIT-6), Dizziness Handicap Inventory (DHI), Pittsburgh Sleep Quality Index (PSQI), 36-Item Short Form Health Survey Version 2 (SF-36v2), and verbal numerical rating scale (VNRS) to assess the patient's symptoms. As new herbal medicine and local acupuncture point stimulation were applied, dizziness, headache, and neck pain symptoms improved. Quality of life affected by the symptoms significantly improved. Adverse effects were not observed. Conclusions: This study may suggest that herbal medicine and local acupuncture point stimulation may be effective therapy for the treatment of typical symptoms in patients with CGD.

Trench Power MOSFET using Separate Gate Technique for Reducing Gate Charge (Gate 전하를 감소시키기 위해 Separate Gate Technique을 이용한 Trench Power MOSFET)

  • Cho, Doohyung;Kim, Kwangsoo
    • Journal of IKEEE
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    • v.16 no.4
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    • pp.283-289
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    • 2012
  • In this paper, We proposed Separate Gate Technique(SGT) to improve the switching characteristics of Trench power MOSFET. Low gate-to-drain 전하 (Miller 전하 : Qgd) has to be achieved to improve the switching characteristics of Trench power MOSFET. A thin poly-silicon deposition is processed to form side wall which is used as gate and thus, it has thinner gate compared to the gate of conventional Trench MOSFET. The reduction of the overlapped area between the gate and the drain decreases the overlapped charge, and the performance of the proposed device is compared to the conventional Trench MOSFET using Silvaco T-CAD. Ciss(input capacitance : Cgs+Cgd), Coss(output capacitance : Cgd+Cds) and Crss(reverse recovery capacitance : Cgd) are reduced to 14.3%, 23% and 30% respectively. To confirm the reduction effect of capacitance, the characteristics of inverter circuit is comprised. Consequently, the reverse recovery time is reduced by 28%. The proposed device can be fabricated with convetional processes without any electrical property degradation compare to conventional device.

Assessment of Coastal Ground Water Discharge for Complex Coastlines (해안선의 형태를 고려한 지하수 해안 유출량 평가 기법)

  • Han, Su-Young;Hong, Sung-Hoon;Park, Nam-Sik
    • Journal of Korea Water Resources Association
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    • v.37 no.11
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    • pp.939-947
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    • 2004
  • Amount of ground water discharging through coastline(CGD) is small compared to the amount of river discharges. However, CGD is important for gound water development in coastal areas and for contamination of coastal waters by nutrients transported by ground water. In the previous study, a method was proposed to estimate CGD from simple data such as ground water levels at observation wells and hydraulic conductivities. In this study, the method is enhanced by taking into account complexity of coastlines. A new method is proposed to estimate average hydraulic gradient, length of coastline, and average thickness of freshwater The new method is verified against numerical solutions. It is shown that the method is able to estimate CGD from complex coastlines more accurately than the previous method.

Long-term outcome of patients with p22phox-deficient chronic granulomatous disease on Jeju Island, Korea

  • Kang, Hyun Sik;Hwang, Geol;Shin, Kyung-Sue
    • Clinical and Experimental Pediatrics
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    • v.58 no.4
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    • pp.129-135
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    • 2015
  • Purpose: This study investigated the long-term clinical outcomes of patients with $p22^{phox}$-deficient chronic granulomatous disease (CGD) on Jeju Island and retrospectively evaluated the effects of interferon-gamma (IFN-${\gamma}$) prophylaxis. Methods: The medical records of 15 patients with CGD were retrospectively reviewed. The efficacy of IFN-${\gamma}$ prophylaxis was evaluated by comparing the frequency of severe infections before and after starting continuous prophylaxis with IFN-${\gamma}$. Results: At the time of the analysis, 14 patients were alive, with a median age of 14.3 years. The diagnosis of CGD was made at a median age of 2.4 years, and the median age at onset of severe infection was 0.3 years. Thirteen of the 15 patients had their first severe infection within the first year of life. The overall incidence of severe infection was 1.36 infections per patient-year; pneumonia, suppurative lymphadenitis, and skin and subcutaneous abscesses were the most common infections. Aspergillus species were the most frequently isolated microorganisms, present in 15.8% of isolates. IFN-${\gamma}$ did not significantly change the rate of severe infection. The survival rate for patients after 2 years of age was 93%; there was a prolonged survival plateau beyond the age of 2. Conclusion: Compared with cases of X-linked CGD reported in other studies, patients with CGD on Jeju Island did not show obviously different clinical manifestations, but they had a significantly higher survival rate. Further studies with a substantially longer period of observation, and with more patients under intensive surveillance are necessary to elucidate the prophylactic efficiency of IFN-${\gamma}$.

Geochemistry of Granites in the Southern Gimcheon Area of Korea (김천남부에 분포하는 화강암류의 지구화학)

  • 윤현수;홍세선
    • The Journal of the Petrological Society of Korea
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    • v.12 no.1
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    • pp.16-31
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    • 2003
  • The granites in the southern Gimcheon area can be divided into two parts, marginal hornblende biotite granodiorite (Mgd) and central biotite granodiorite to granite (Cgd). Mgd and Cgd are gray in color and display gradational contact relations and are mainly composed of coarse-grained and medium-grained rocks, respectively. Mgd has more frequent and larger mafic enclaves than Cgd, and the two granites partly show parallel foliation at thire contact with gneisses. From representative samples of the granites, K-Ar biotite ages of 197∼207 Ma were obtained. Considering the blocking temperature of biotite, it is suggested that the emplacement age of the granitic magma was probably late Triassic. The anorthite contents of plagioclases in Mgd display less variation than those of Cgd, indicating that Mgd crystallized within a narrow range of temperatures. In the Al$\_$total/-Mg diagram, the biotites from the granites plot within the subalkaline field, and the smooth slope indicates differentiation from a single magma. All amphiboles from the granites belong to magnesio-hornblende. The linear trends of major oxides, AFM and Ba-Sr-Rb indicate that Mgd and Cgd were fractionally differentiated from a single granitic magma body crystallizing from the margin inwards. The relations of modal (Qz+Af) vs. Op, K$_2$O vs. Na$_2$O, Fe$_2$ $O_3$ vs. FeO, Fe$\^$+3/(Fe$\^$+3/+Fe$\^$+2/) and K/Rb vs. Rb/Sr show that they belong to I-type and magnetite-series granitic rocks developed by the progressive melting products of fixed sources. REE data, normalized to chondrite value, have trends of enriched LREE and depleted HREE together with weakly negative Eu anomalies.

A Study on the Alternative ARL Using Generalized Geometric Distribution (일반화 기하분포를 이용한 ARL의 수정에 관한 연구)

  • 문명상
    • Journal of Korean Society for Quality Management
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    • v.27 no.4
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    • pp.143-152
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    • 1999
  • In Shewhart control chart, the average run length(ARL) is calculated using the mean of a conventional geometric distribution(CGD) assuming a sequence of identical and independent Bernoulli trials. In this, the success probability of CGB is the probability that any point exceeds the control limits. When the process is in-control state, there is no problem in the above assumption since the probability that any point exceeds the control limits does not change if the in-control state continues. However, if the out-of-control state begins and continues during the process, the probability of exceeding the control limits may take two forms. First, once the out-of-control state begins with exceeding probability p, it continues with the same exceeding probability p. Second, after the out-of-control state begins, the exceeding probabilities may very according to some pattern. In the first case, ARL is the mean of CGD with success probability p as usual. But in the second case, the assumption of a sequence of identical and independent Bernoulli trials is invalid and we can not use the mean of CGD as ARL. This paper concentrate on that point. By adopting one generalized binomial distribution(GBD) model that allows correlated Bernoulli trials, generalized geometric distribution(GGD) is defined and its mean is derived to find an alternative ARL when the process is in out-of-control state and the exceeding probabilities take the second form mentioned in the above. Small-scale simulation is performed to show how an alternative ARL works.

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