• 제목/요약/키워드: cause of defects

검색결과 622건 처리시간 0.03초

Hunter 증후군 환아의 치과적 관리: 증례보고 (DENTAL MANAGEMENT OF PATIENT WITH HUNTER SYNDROME (MUCOPOLYSACCHARIDOSIS TYPE II) : A CASE REPORT)

  • 이민정;김재곤;양연미;백병주;송희정
    • 대한소아치과학회지
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    • 제39권4호
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    • pp.412-417
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    • 2012
  • 뮤코다당증(mucopolysaccharidosis : MPS)은 glycosaminoglycans (GAGs)의 분해에 필요한 라이소좀 효소의 결함으로 인해 야기되는 질병으로 GAGs의 대사산물이 세포의 라이소좀 내에 축적되어 점차적으로 세포, 조직 그리고 기관의 기능 이상을 초래해 육체적, 정신적인 퇴행을 보이며, 심한 경우, 조기에 사망하게 되는 다양한 임상양상을 보이는 질환이다. 임상형은 어떠한 효소가 결핍되느냐에 따라 I형에서 IX형으로 분류한다. 치과적인 증상으로는 맹출 지연, 법랑질 저형성증, 왜소치, 부정교합, 하악 과두의 결함, 치은증식, 그리고 함치성낭 같은 여포성낭이 보고되었다. 뮤코다당증 환자는 심혈관계와 호흡계 기능이 취약하므로 치과 치료를 하는 경우, 전신 상태에 대한 주의사항과 뮤코다당증 환자에서 나타날 수 있는 구강 내 증상에 대해 미리 숙지하고, 의학적으로 환아의 전신 상태 변화에 민감하게 대처해가며 치과 진료를 시행해야 한다. 이 증례는 구강 내 다양한 임상증상을 보이는 제2형 뮤코다당증 환아의 치과 진료 후, 다소의 지견을 얻었기에 이를 보고하는 바이다.

A novel BTK gene mutation, c.82delC (p.Arg28 Alafs*5), in a Korean family with X-linked agammaglobulinemia

  • Lee, Jeongeun;Rhee, Minhee;Min, Taek Ki;Bang, Hae In;Jang, Mi-Ae;Kang, Eun-Suk;Kim, Hee-Jin;Yang, Hyeon-Jong;Pyu, Bok Yang
    • Clinical and Experimental Pediatrics
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    • 제59권sup1호
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    • pp.49-52
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    • 2016
  • X-linked agammaglobulinemia (XLA) is a hereditary humoral immunodeficiency that results from Bruton's tyrosine kinase (BTK ) gene mutations. These mutations cause defects in B-cell development, resulting in the virtual absence of these lymphocytes from the peripheral circulation. Consequently, this absence leads to a profound deficiency of lg all isotypes, and an increased susceptibility to encapsulated bacterial infections. A 15-month-old Korean boy presented with recurrent sinusitis and otitis media after 6 months of age, and had a family history of 2 maternal uncles with XLA. Laboratory tests revealed a profound deficiency of Ig isotypes, and a decreased count of $CD19^+$ B cells in the peripheral circulation. Based on his family history and our laboratory test results, he was diagnosed with XLA. We performed BTK gene analysis of peripheral blood samples obtained from family members to confirm the diagnosis. Mutational analysis revealed a novel hemizygous frameshift mutation (c.82delC, p.Arg28Alafs*5), in the BTK gene. His mother and maternal grandmother were heterozygous carriers of this mutation and his two maternal uncles were hemizygous at the same position. After XLA diagnosis, intravenous immunoglobulin (400 mg/kg, monthly) treatment was initiated; recurrent sinusitis and otitis media were subsequently brought under control. To our knowledge, this is the first reported case of a Korean pedigree with a novel mutation in the BTK gene.

무수치 표백술 후 잔존 과산화수소수 제거를 위한 수종의 치수강 세척제의 효과에 관한 정량적 연구 (A QUANTITATIVE STUDY ON THE DEGRADING EFFECT OF THE VARIOUS IRRIGATING AGENTS IN THE ELIMINATION OF RESIDUAL HYDROGEN PEROXIDE FOLLOWING WALKING BLEACHING)

  • 금기연;한원섭;정일영;이승종;이찬영;오병훈
    • Restorative Dentistry and Endodontics
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    • 제23권2호
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    • pp.656-669
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    • 1998
  • Hydrogen peroxide at high concentration during walking bleaching may cause damage to the tooth structure and to the surrounding periodontal tissues and may develop external root resorption. Clinically, It is so important to find a method of prevention or minimization of these complications. The efficacy of various chamber-irrigating agents to eliminate residual hydrogen peroxide after walking bleaching was examined and compared with water rinse in this study. Extracted human 46 premolars without any cementoenamel junction defects were treated endodontically and based with IRM to 1 mm below CEJ and totally bleached 3 times for each tooth with 30% hydrogen peroxide and sodium perborate. Upon completion of the 3rd walking bleaching procedure, the cervical portion and pulp chamber of each group of teeth were irrigated with catalase, 70% ethylalcohol, acetone, and distilled water. And then, a radicular hydrogen peroxide penetration was measured with spectrophotometer immediately after each bleaching and following treatment with each chamber-irrigating agents, and the significance of their eliminating efficacy of residual hydrogen peroxide was analyzed by Kruskal-Wallis test. The results were obtained as follows. 1. Cervical root penetration of hydrogen peroxide was increased as the bleaching procedure was repeated(P<.01). 2. The most effective irrigant that removed residual hydrogen peroxide was the catalase, and the least effective one was water rinsing (P<.01).; there was no significant difference between the acetone and ethanol group. 3. The Irrigation with antioxidant enzyme or water-displacement solutions can eliminate residual oxygen radicals from the pulp chamber effectively after walking bleaching. So, these agents can reduce adverse effects such as cervical external resorption and periapical inflammation and prevent residual $O_2$ from impeding composite resin polymerization, thus increase the bonding strength of composite resin. This, in turn reduces microleakage and discoloration of the esthetic restoration, extending its service-life.

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R.F. Sputtering 방법에 의한 상변화형 광디스크의 $(ZnS)_{1-x}(SiO_2)_x$ 보호막 형성에 미치는 전극거리의 영향 (The Effects of Electrode Distance on the Formation of $(ZnS)_{1-x}(SiO_2)_x$ Protective Films in Phase Change Optical Disk by R.F. Sputtering Method)

  • 이준호;김도훈
    • 한국재료학회지
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    • 제9권12호
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    • pp.1245-1251
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    • 1999
  • 상변화형 광디스크는 직접 반복기록에 의한 고속기록, 고밀도화가 가능하고 높은 전송속도, 재생신호의 C/N (carrier to noise) 비가 좋은 장점을 가지고 있으나 반복되는 열에너지에 의한 디스크의 변형과 소거도의 저하, 기록 반복성의 저하가 문제가 된다. 이러한 반복성의 저하를 개선하기 위해 적절한 디스크의 구조와 기록막의 상하부에 유전체 보호막인 ZnS-$SiO_2$ 박막층을 삽입하였다. 박막 제조시 많은 실험변수의 제어를 위해 다꾸찌 방법을 통하여 타겟 R.F. Power 200W, 기판 R.F. Power 20W, 아르곤 압력 4mTorr, 전극거리 6cm의 최적조건을 얻을수 있었다. TEM과 XRD분석 결과, 전극거리가 가까워질수록 높은 증착속도로 인하여 미세한 조직구조를 가지고 있으며, 일정거리 이상 가까워지면 막의 morphology에 나쁜 영향을 끼침을 알 수 있었다. 이러한 막의 morphology의 영향으로 투과율이 감소하는 것을 확인할 수 있었다. AFM과 SEM분석에서는 전극거리가 가까워질수록 높은 증착속도로 인하여 morphology에 나쁜 영향을 끼치고 있음을 확인할 수 있었다. 최적조건에서 증착한 박막은 우수한 morphology를 가진 초미세구조의 치밀하고 결함이 없는 박막이었다. 이 박막은 상변화형 광디스크에서 열적 변형을 억제하고, 열전도를 감소시켜 우수한 유전체 보호피막의 역할을 할 수 있다. 그리고, 전극거리가 ZnS결정립의 크기와 증착속도, morphology에 미치는 영향에 대해 고찰하였다.

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유리 조직 이식의 분석 (The Analysis of Free Flap)

  • 최상묵;홍성범;정찬민;서인석
    • Archives of Reconstructive Microsurgery
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    • 제5권1호
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    • pp.35-45
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    • 1996
  • After transplantation of groin free flap was sucessed by the Daniel and Taylor in 1973, the reconstruction of plastic surgery was extensive and universal due to rapidly developement of anatomic study of the donor site and technique of microvascular surgery. The free tissue transfers is possible to be early activity and rehabilitation by one stage operation. It currently available allow transfer of specific tissue quality as bone, muscle, nerve to achieve a functional and cosmetic result as well as the most favorable secondary defect. But free flaps require critical, skillful technique and lengthy operating time. Also it has disadvantage of donor site morbity at the large tissue transfer. Authors were transferred with 107 cases in 103 patients from May 1987 to June 1996, and then we analysed free tissue transfer to acquire more increased sucess rate, satisfactory functional and cosmetic results. The sexual distribution was male prominent in 79 cases(76.7%), female in 24(23.3%) and age was variable distribution from 3 to 76 years old. The cause of defects was most prevalent in trauma of traffic and industrial accident in 51 cases(49%). The common recipient site were lower extremities in 47 cases(43.9%), upper extremities in 28 cases(26.5%), head and neck in 25 cases(23.4%), and trunk in 7 cases(6.5%). The type of transfer were free skin flaps in 46 cases(43%), free muscle or musculocutaneous flaps in 31 cases(29%), free vasculized or osteocutaneous flaps in 10 cases(9.3%), and specilized free flaps in 20 cases(18.7%). The anastomosis of artery was end to end anastomosis in 94 cases(87.9%), end to side anastomosis in 13 cases(12.1%) and all vein was end to end anastomosis. The number of anastomosed vessels were one artery one vein in 62 cases(57.9%), one artery two vein in 45 cases(42.1%) and vein graft was performed only one case. The postoperative mornitoring were used with temperature, color of flap, capillary refilling time, ultrasonogram, bone scan, doppler, and endoscopy. The reexploration was performed in 9 cases(8.4%), and then flap was loss in 3 cases(2.8%). Accordingly overall success rate was 97.2%. The postoperative complication was early vascular occlusion, hematoma, partial necrosis and late bulkiness, scarring, color dismatch etc. Therefore, free tissue transfer is the preferred method of treatment, even through conventional local and distant flaps are available.

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면역 기능의 이상이 발견되지 않은 영아에서의 침습성 녹농균 감염증 2례 (Two Cases of Invasive Pseudomonas aeruginosa Infection that Developed in the Apparently Immunocompetent Infants)

  • 강민재;김소희;김남희;이진아;은병욱;최은화;이환종
    • Pediatric Infection and Vaccine
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    • 제13권2호
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    • pp.180-185
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    • 2006
  • 침습적 Pseudomonas 감염은 대개 면역 저하 환자들에서 발생하며, 이는 높은 사망률과 관련이 있다. 그러나 드물게는 면역력이 정상인 숙주에서도 발생할 수 있다. 첫 번째 증례는 괴저성 농창과 Pseudomonas 패혈증이 발생한 5개월 여아이며, 두번째 증례는 9개월 남아에서 지역사회 감염으로 발생한 폐렴과 Pseudomonas 패혈증이다. 두 환아 모두에서 녹농균이 배양검사로 증명되어 감수성 있는 항생제를 사용하였으며, T 림프구, B 림프구, 보체, 식세포 등 면역력에 대한 검사는 모두 정상이었다. 저자들은 이전에 건강했던 영아들에서 발생한 침습성 녹농균 감염증 2례를 경험하였기에 이를 보고하는 바이다.

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가류 촉진제 블루밍에 의한 전투기 연료필터의 막힘 (Blocking of Fuel Filter in Aircraft by an Accelerator Blooming)

  • 김익식;황영하;손경숙;이중훈;손병훈
    • Elastomers and Composites
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    • 제49권1호
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    • pp.1-12
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    • 2014
  • 전투기 연료필터의 막힘은 조종사 생명과 전투기의 손실에 큰 영향을 줄 수 있다. 따라서 결함 연구는 사고를 미연에 방지하는데 매우 중요하다. 전투기 연료필터는 제트 연료에 포함된 다양한 불순물을 여과하는 중요한 기능을 수행한다. 연료 필터는 시간당 1,330 파운드의 유속으로 공급되는 제트 연료에 함유된 10 ${\mu}m$ 크기 이하의 불순물을 여과한다. 필터는 운영시간 기준 500 시간마다 교체되어야 한다. 그러나 500 시간이 도달하기 훨씬 전에 연료 필터의 막힘 현상으로 경고등이 켜지고 있다. 이와 같은 유사한 결함이 최근에 반복적으로 발생하고 있다. 그러므로, 이 연구에서는 전투기의 연료 필터 막힘에 대한 원인을 FT-IR microcopy, FE-SEM/EDS, 총 황함량 분석기와 같은 다양한 분석기술을 이용하여 조사하였다. 결론적으로, 연료 필터를 막히게 한 물질은 내부 연료 탱크용 실란트에서 기인한 가류 촉진제로 확인되었다. 그리고 연료 탱크내에서 가류 촉진제 블루밍 현상에 의해 연료필터를 막히게 하는 물질의 형성에 대한 메커니즘을 제시하였다.

Research on the Multi-electrode Plasma Discharge for the Large Area PECVD Processing

  • Lee, Yun-Seong;You, Dae-Ho;Seol, You-Bin
    • 한국진공학회:학술대회논문집
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    • 한국진공학회 2012년도 제42회 동계 정기 학술대회 초록집
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    • pp.478-478
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    • 2012
  • Recently, there are many researches in order to increase the deposition rate (D/R) and improve film uniformity and quality in the deposition of microcrystalline silicon thin film. These two factors are the most important issues in the fabrication of the thin film solar cell, and for the purpose of that, several process conditions, including the large area electrode (more than 1.1 X 1.3 (m2)), higher pressure (1 ~ 10 (Torr)), and very high frequency regime (VHF, 40 ~ 100 (MHz)), have been needed. But, in the case of large-area capacitively coupled discharges (CCP) driven at frequencies higher than the usual RF (13.56 (MHz)) frequency, the standing wave and skin effects should be the critical problems for obtaining the good plasma uniformity, and the ion damage on the thin film layer due to the high voltage between the substrate and the bulk plasma might cause the defects which degrade the film quality. In this study, we will propose the new concept of the large-area multi-electrode (a new multi-electrode concept for the large-area plasma source), which consists of a series of electrodes and grounds arranged by turns. The experimental results with this new electrode showed the processing performances of high D/R (1 ~ 2 (nm/sec)), controllable crystallinity (~70% and controllable), and good uniformity (less than 10%) at the conditions of the relatively high frequency of 40 MHz in the large-area electrode of 280 X 540 mm2. And, we also observed the SEM images of the deposited thin film at the conditions of peeling, normal microcrystalline, and powder formation, and discussed the mechanisms of the crystal formation and voids generation in the film in order to try the enhancement of the film quality compared to the cases of normal VHF capacitive discharges. Also, we will discuss the relation between the processing parameters (including gap length between electrode and substrate, operating pressure) and the processing results (D/R and crystallinity) with the process condition map for ${\mu}c$-Si:H formation at a fixed input power and gas flow rate. Finally, we will discuss the potential of the multi-electrode of the 3.5G-class large-area plasma processing (650 X 550 (mm2) to the possibility of the expansion of the new electrode concept to 8G class large-area plasma processing and the additional issues in order to improve the process efficiency.

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광범위한 안와파열골절에서 Titanium Mesh Plate와 Porous Polyethylene (Medpor®) 동시 사용의 유용성 (Treatment of Blow-out Fractures Using Both Titanium Mesh Plate and Porous Polyethylene (Medpor®))

  • 구자혜;원창훈;동은상;윤을식
    • 대한두개안면성형외과학회지
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    • 제11권2호
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    • pp.85-90
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    • 2010
  • Purpose: The goals of a blow-out fracture reconstruction are to restore the osseous continuity, provide support for the orbital contents and prevent functional and anatomic defects. Over the past several years, a range of autogenous and synthetic implants have been used extensively in orbital reconstructions. None of these implants have any absolute indications or contraindications in certain clinical settings. However, in extensive blow-out fractures, it is difficult to restore support of the orbital contents, which can cause more complications, such as enophthalmos. This study examined the clinical outcomes of extensive or comminuted blow-out fractures that were reconstructed by the simultaneous use of a titanium mesh plate and $Medpor^{(R)}$. Methods: Eighty six patients with extensive orbital fractures, who were admitted between March 1999 and February 2007, were reviewed retrospectively. The patients' chart and CT were inspected for review. Twenty three patients were operated on with both a titanium mesh plate (Matrix MIDFACE pre-formed orbital plate, Synthes, USA) and $Medpor^{(R)}$ (Porex, GA, USA). The patients underwent pre-operative CT scans to evaluate the fracture site and measure the area of the fracture. A transconjunctival approach was used, and titanium mesh plates were inserted subperiosteally with screw fixation. $Medpor^{(R)}$ was inserted above the titanium mesh plate. The patients were evaluated post-operatively for enophthalmos, diplopia, sensory disturbances and eyeball movement for a period of at least 6 months. Results: No implant-related complications were encountered during the follow-up period. Enophthalmos occurred in 1 patient, 1 patient had permanent sensory disturbance, and 3 patients complained of ocular pain and fatigue, which recovered without treatment. Although there were no significance differences between groups, the use of 2 implants had fewer complications. Therefore, it can be an alternative method for treating blow out fractures. Conclusion: The use of both a titanium mesh plate and $Medpor^{(R)}$ simultaneously may be a safe and acceptable technique in the reconstruction of extensive blow-out fractures.

Quality Characteristics of Functional Fermented Sausages Added with Encapsulated Probiotic Bifidobacterium longum KACC 91563

  • Song, Min-Yu;Van-Ba, Hoa;Park, Won-Seo;Yoo, Ja-Yeon;Kang, Han-Byul;Kim, Jin-Hyoung;Kang, Sun-Moon;Kim, Bu-Min;Oh, Mi-Hwa;Ham, Jun-Sang
    • 한국축산식품학회지
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    • 제38권5호
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    • pp.981-994
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    • 2018
  • The present study aimed at evaluating the utilization possibility of encapsulated probiotic Bifidobacterium longum for production of functional fermented sausages. The B. longum isolated from the feces samples of healthy Korean infants encapsulated with glycerol as a cryprotectant was used for fermented sausages production as a functional bacterial ingredient, and its effect was also compared with those inoculated with commercial starter culture (CSC). Results showed that most inoculated encapsulated B. longum (initial count, 5.88 Log CFU/g) could survive after 4 days fermentation (5.40 Log CFU/g), and approximately a half (2.83 Log CFU/g) of them survived in the products after 22 days of ripening. The products inoculated with encapsulated B. longum presented the lowest lipid oxidation level, while had higher total unsaturated fatty acid content and more desirable n-6/n-3 fatty acids than those inoculated with CSC or non-inoculated control. Moreover, the odor and taste scores in the samples made with B. longum were comparable to those in the treatment with CSC. The inoculation with the B. longum had no effects on the biogenic amine contents as well as did not cause defects in color or texture of the final products. Thus, the encapsulation could preserve the probiotic B. longum in the meat mixture, and the encapsulated B. longum could be used as a functional ingredient for production of healthier fermented meat products.