• Title/Summary/Keyword: Multiple Defects

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A novel PRF1 gene mutation in a fatal neonate case with type 2 familial hemophagocytic lymphohistiocytosis

  • Kim, Jae Yeon;Shin, Jeong Hee;Sung, Se In;Kim, Jin Kyu;Jung, Ji Mi;Ahn, So Yoon;Kim, Eun Sun;Seo, Ja-Young;Kang, Eun-Sook;Kim, Sun-Hee;Kim, Hee-Jin;Chang, Yun Sil;Park, Won Soon
    • Clinical and Experimental Pediatrics
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    • v.57 no.1
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    • pp.50-53
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    • 2014
  • Hemophagocytic lymphohistiocytosis (HLH) occurs in the primary form (genetic or familial) or secondary form (acquired). The familial form of HLH (FHL) is a potentially fatal autosomal recessive disorder that occurs because of constitutional defects in cell-mediated cytotoxicity. Here, we report a fatal neonatal case of type 2 FHL (FHL2) that involved a novel frameshift mutation. Clinically, the newborn presented with severe sepsis-like features and required mechanical ventilation and continuous venovenous hemodiafiltration. Flow cytometry analysis showed marked HLH and complete absence of intracytoplasmic perforin expression in cytotoxic cells; therefore, we performed molecular genetic analyses for PRF1 mutations, which showed that the patient had a compound heterozygous mutation in PRF1, that is, c.65delC ($p.Pro22Argfs^*2$) and c.1090_1091delCT ($p.Leu364Glufs^*93$). Clinical and genetic assessments for FHL are required for neonates with refractory fever and progressive multiple organ failure, particularly when there is no evidence of microbiological or metabolic cause.

Reconstruction of the Lower Extremity with the Microsurgical Technique (미세수술을 이용한 하지 재건술)

  • Hahn, Soo-Bong;Jeon, Chang-Hoon
    • Archives of Reconstructive Microsurgery
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    • v.3 no.1
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    • pp.32-39
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    • 1994
  • One hundred and sixty patients had reconstructive surgery of the lower extremity with the microsurgical technique at the department of orthopaedic surgery, Yonsei University College of Medicine from 1982 to 1989. There were ninty-six cases of men and sixty-four cases of women, in which the mean age was 23.8 years. These patients were followed for 21.4 months. The causes were 114 cases from traffic accidents, 18 cases from tumors, 12 cases from machinery injuries, 5 cases from burns, 2 cases from explosive injuries, and 9 cases from other reasons. There were 55 cases of scapular flap, 35 cases of groin flap, 23 cases of free vascularized osteocutaneous flap, 18 cases of parascapular flap, 9 cases of combined scapular and latissimus dorsi flaps, and 8 cases of segmental resection and rotationplasty. Success in reconstructive surgery with the microsurgical technique was achieved in one hundred and thirty four cases, and function and cosmetic results were excellent. Free vascularized flap with development of the microsurgical technique has taken an important role in reconstruction of large extremity defects where skin graft and distant flap were not applicable. Reconstruction of the lower extremity with the microsurgical technique is indicated with free vascularized osteocutaneous flap when there is a large defect of bone, a need for injured nerve replacement, and in the case of needed multiple staged operations. In these instences, this technique is regarded as simple one-staged reconstructive surgery.

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

  • Kang, Min Jae;Kim, So Hee;Kim, Nam Hee;Lee, Jin-A;Eun, Byung Wook;Choi, Eun Hwa;Lee, Hoan Jong
    • Pediatric Infection and Vaccine
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    • v.13 no.2
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    • pp.180-185
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    • 2006
  • Invasive Pseudomonas infections most often occur in the immunocompromised patients and are associated with high mortality rate. Rarely this disease may develop in healthy infants and children. We report two cases of invasive Pseudomonas aeruginosa infections that were diagnosed in otherwise healthy infants. The first case was a previously healthy 5-month-old infant with ecthyma gangrenosum and septicemia. She presented with fever, swelling of left periorbital area and multiple erythronodular skin lesions. Each skin lesion formed a black eschar surrounded by an erythematous areola over time. Cultures of blood, urine and discharge from skin lesions grew P. aeruginosa. On the day of visit, she showed pancytopenia which was normalized after 10 days. The patient responded well to the management with ceftazidime and tobramycin. The other case was a previously healthy 9-month-old infant with community-acquired pneumonia. He was referred from an outside hospital with fever and cough. Chest x-ray revealed pneumonic infiltrations on both lower lungs with pleural effusion on the right side. Cultures of blood and pleural fluid grew P. aeruginosa. Chest CT performed on the ninth day demonstrated pneumatoceles, lung abscess and necrosis of lung parenchyma. He was managed with ceftazidime and amikacin for 50 days. No residual pulmonary complications were noted during the three month follow-up. Laboratory results to evaluate immunologic defects of phagocytic cells, complement components and T- and B-lymphocytes were all within normal range in both patients. It should be kept in mind that Pseudomonas can be, though uncommon, a cause of community-acquired invasive infections in the previously healthy infants.

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A Case of Acute Respiratory Distress Syndrome with Pulmonary Embolism Induced by Injection of Silicone at Vaginal Wall (질벽의 실리콘 액 주사에 의한 폐색전종 및 급성 호흡곤란 증후군 1예)

  • Kang, Moon-Bo;Kim, Seong-Tae;Lee, Jung-Gu;Seo, Chan-Jong;Lee, Hwa-Eun;Jeong, Jung-Bae;Kim, Sung-Gwon;Kim, Chul;Park, Jeong-Woong;Jeong, Seong-Whan;Nam, Gui-Hyun
    • Tuberculosis and Respiratory Diseases
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    • v.46 no.3
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    • pp.414-419
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    • 1999
  • Silicone fluid(polydimethylsiloxane) is widely used in breast augmentation and other cosmetic procedures because of little incidence of complications and low mortality rate. However, local reaction following silicone injections can be occurred sometimes leading to serious complications. Especially, illicit silicone injections have resulted in severe reactions within the pulmonary area, and some have resulted in acute respiratory distress syndrome subsequently. We experienced a case of acute respiratory distress syndrome induced by subcutaneous injections of silicone at vaginal wall. The patients was 39-year-old, previously healthy woman who had complained of dyspnea related to silicone injection at vaginal wall. Chest X-ray and chest CT scan show diffuse air consolidation with ground glass opacities and perfusion lung scan revealed likelihood of pulmonary embolism as showing multiple perfusion defects. We report a case of acute respiratory distress syndrome occured after silicone injection with review of literature.

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A Comparative Analysis on Slope Stability Using Specific Catchment Area Calculation (비 집수면적 산정기법에 따른 사면 안정성 비교·분석)

  • Lee, Gi-Ha;Oh, Sung-Ryul;An, Hyun-Uk;Jung, Kwan-Sue
    • Journal of Korea Water Resources Association
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    • v.45 no.7
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    • pp.643-656
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    • 2012
  • There has been an increase for the landslide areas and restoration expenses due, in large part, to the increased locally heavy rains caused by recent climate change as well as the reckless development. This study carried out a slope stability analysis by the application of distributed wetness index, using the GIS-based infinite slope stability model, which took the root cohesion effect into consideration, for part of Mt. Umyeon in Seoul, where landslide occurred in July 2011, in order to compensate the defects of existing analysis method, and subsequently compared its result with the case on the exploitation of lumped wetness index. In addition, this study estimated the distributed wetness index by methodology, applying three methods of specific catchment area calculation: single flow direction (SFD), multiple flow direction (MFD), and infinity flow direction (IFD), for catchment area, one of the variables of distributed wetness indices, and finally implemented a series of comparative analysis for slope stability by methodology. The simulation results showed that most unstable areas within the study site were dominantly located in cutting-area surroundings along with the residential area and the mountaintop and unstable areas of IFD and lumped wetness index method were similar while SFD and MFD provided smaller unstable areas than the two former methods.

Petrological Characteristics and Nondestructive Deterioration Assessments for Foundation Stones of the Sebyeonggwan Hall in Tongyeong, Korea (통영 세병관 초석의 암석학적 특성 및 비파괴 손상평가)

  • Han, Doo Roo;Kim, Sung Han;Park, Seok Tae;Lee, Chan Hee
    • Economic and Environmental Geology
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    • v.54 no.2
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    • pp.199-212
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    • 2021
  • The Sebyeonggwan Hall (National Treasure No. 305) is located on the Naval Headquarter of Three Provinces in Tongyeong, and it has partly undergone with several rebuilding, remodeling, repairing and restorations since it's the first establishment in Joseon Dynasty (AD 1605) of ancient Korea. This study focuses on 50 foundation stones that comprise the Sebyeonggwan. These stones are made of six rock types and currently have various shapes of the surface damages. As the foundation stones, the dominant rock type was dacitic lapilli tuffs, and provenance-based interpretation was performed to supply alternative stones for conservation. Most of the provenance rocks for foundation stones showed highly homogeneity with their corresponding stones of petrography, mineralogy and magnetic susceptibility. According to surface deterioration assessments, the most serious damages of the stones were blistering and scaling. The deterioration mechanism was identified through the analysis of inorganic contaminants, and the primary reason is considered salt weathering caused by sea breeze and other combined circumstances. Based on the mechanical durability of the stones, there was no foundation stone that required the replacement of its members attributed to the degradation of the rock properties, but conservation treatment is considered necessary to delay superficial damage. The foundation stones are characterized by a combined outcome of multiple petrological factors that caused physical damage to surfaces and internal defects. Therefore, it's required to diagnosis and monitoring the Sebyeonggwan regularly for long-term preservation.

Intrawound Vancomycin Powder Application for Preventing Surgical Site Infection Following Cranioplasty

  • Seong Bin Youn;Gyojun Hwang;Hyun-Gon Kim;Jae Seong Kang;Hyung Cheol Kim;Sung Han Oh;Mi-Kyung Kim;Bong Sub Chung;Jong Kook Rhim;Seung Hun Sheen
    • Journal of Korean Neurosurgical Society
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    • v.66 no.5
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    • pp.536-542
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    • 2023
  • Objective : Surgical site infection is the most detrimental complication following cranioplasty. In other surgical fields, intrawound vancomycin powder application has been introduced to prevent surgical site infection and is widely used based on results in multiple studies. This study evaluated the effect of intrawound vancomycin powder in cranioplasty compared with the conventional method without topical antibiotics. Methods : This retrospective study included 580 patients with skull defects who underwent cranioplasty between August 1, 1998 and December 31, 2021. The conventional method was used in 475 (81.9%; conventional group) and vancomycin powder (1 g) was applied on the dura mater and bone flap in 105 patients (18.1%; vancomycin powder group). Surgical site infection was defined as infection of the incision, organ, or space that occurred after cranioplasty. Surgical site infection within 1-year surveillance period was compared between the conventional and vancomycin powder groups with logistic regression analysis. Penalized likelihood estimation method was used in logistic regression to deal with zero events. All local and systemic adverse events associated with topical vancomycin application were also evaluated. Results : Surgical site infection occurred in 31 patients (5.3%) and all were observed in the conventional group. The median time between cranioplasty and detection of surgical site infection was 13 days (range, 4-333). Staphylococci were the most common organisms and identified in 25 (80.6%) of 31 cases with surgical site infections. The surgical site infection rate in the vancomycin powder group (0/105, 0.0%) was significantly lower than that in the conventional group (31/475, 6.5%; crude odds ratio [OR], 0.067; 95% confidence interval [CI], 0.006-0.762; adjusted OR, 0.068; 95% CI, 0.006-0.731; p=0.026). No adverse events associated with intrawound vancomycin powder were observed during the follow-up. Conclusion : Intrawound vancomycin powder effectively prevented surgical site infections following cranioplasty without local or systemic adverse events. Our results suggest that intrawound vancomycin powder is an effective and safe strategy for patients undergoing cranioplasty.

Establishment of a deep learning-based defect classification system for optimizing textile manufacturing equipment

  • YuLim Kim;Jaeil Kim
    • Journal of the Korea Society of Computer and Information
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    • v.28 no.10
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    • pp.27-35
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    • 2023
  • In this paper, we propose a process of increasing productivity by applying a deep learning-based defect detection and classification system to the prepreg fiber manufacturing process, which is in high demand in the field of producing composite materials. In order to apply it to toe prepreg manufacturing equipment that requires a solution due to the occurrence of a large amount of defects in various conditions, the optimal environment was first established by selecting cameras and lights necessary for defect detection and classification model production. In addition, data necessary for the production of multiple classification models were collected and labeled according to normal and defective conditions. The multi-classification model is made based on CNN and applies pre-learning models such as VGGNet, MobileNet, ResNet, etc. to compare performance and identify improvement directions with accuracy and loss graphs. Data augmentation and dropout techniques were applied to identify and improve overfitting problems as major problems. In order to evaluate the performance of the model, a performance evaluation was conducted using the confusion matrix as a performance indicator, and the performance of more than 99% was confirmed. In addition, it checks the classification results for images acquired in real time by applying them to the actual process to check whether the discrimination values are accurately derived.

Research on Bridge Maintenance Methods Using BIM Model and Augmented Reality (BIM 모델과 증강현실을 활용한 교량 유지관리방안 연구)

  • Choi, Woonggyu;Pa Pa Win Aung;Sanyukta Arvikar;Cha, Gichun;Park, Seunghee
    • KSCE Journal of Civil and Environmental Engineering Research
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    • v.44 no.1
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    • pp.1-9
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    • 2024
  • Bridges, which are construction structures, have increased from 584 to 38,405 since the 1970s. However, as the number of bridges increases, the number of bridges with a service life of more than 30 years increases to 21,737 (71%) by 2030, resulting in fatal accidents due to basic human resource maintenance of facilities. Accordingly, the importance of bridge safety inspection and maintenance measures is increasing, and the need for decision-making support for supervisors who manage multiple bridges is also required. Currently, the safety inspection and maintenance method of bridges is to write down damage, condition, location, and specifications on the exterior survey map by hand or to record them by taking pictures with a camera. However, errors in notation of damage or defects or mistakes by supervisors are possible, typos, etc. may reduce the reliability of the overall safety inspection and diagnosis. To improve this, this study visualizes damage data recorded in the BIM model in an AR environment and proposes a maintenance plan for bridges with a small number of people through maintenance decision-making support for supervisors.

Cannabidiol Promotes Osteogenic Differentiation of Bone Marrow Mesenchymal Stem Cells in the Inflammatory Microenvironment via the CB2-dependent p38 MAPK Signaling Pathway

  • Lin Li;Jin Feng;Lei Sun;Yao-wei Xuan;Li Wen;Yun-xia Li;Shuo Yang;Biao Zhu;Xiao-yu Tian;Shuang Li;Li-sheng Zhao;Rui-jie Dang;Ting Jiao;Hai-song Zhang;Ning Wen
    • International Journal of Stem Cells
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    • v.15 no.4
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    • pp.405-414
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    • 2022
  • Background and Objectives: Chronic inflammation of bone tissue often results in bone defects and hazards to tissue repair and regeneration. Cannabidiol (CBD) is a natural cannabinoid with multiple biological activities, including anti-inflammatory and osteogenic potential. This study aimed to investigate the efficacy and mechanisms of CBD in the promotion of bone marrow mesenchymal stem cells (BMSCs) osteogenic differentiation in the inflammatory microenvironment. Methods and Results: BMSCs isolated from C57BL/6 mice, expressed stem cell characteristic surface markers and presented multidirectional differentiation potential. The CCK-8 assay was applied to evaluate the effects of CBD on BMSCs' vitality, and demonstrating the safety of CBD on BMSCs. Then, BMSCs were stimulated with lipopolysaccharide (LPS) to induce inflammatory microenvironment. We found that CBD intervention down-regulated mRNA expression levels of inflammatory cytokines and promoted cells proliferation in LPS-treated BMSCs, also reversed the protein and mRNA levels downregulation of osteogenic markers caused by LPS treatment. Moreover, CBD intervention activated the cannabinoid receptor 2 (CB2) and the p38 mitogen-activated protein kinase (MAPK) signaling pathway. While AM630, a selective CB2 inhibitor, reduced phosphorylated (p)-p38 levels. In addition, AM630 and SB530689, a selective p38 MAPK inhibitor, attenuated the enhancement of osteogenic markers expression levels by CBD in inflammatory microenvironment, respectively. Conclusions: CBD promoted osteogenic differentiation of BMSCs via the CB2/p38 MAPK signaling pathway in the inflammatory microenvironment.