• Pediatric Infection and Vaccine
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• 제11권2호
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• pp.212-215
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• 2004

보체계는 우리 몸에서 감염에 대한 초기 방어를 담당하는데 그중 후기 보체 성분의 결핍시 수막구균의 감염에 쉽게 노출된다. 특히 재발 감염된 수막구균 혈증 환자에서 보체계의 검사가 반드시 필요할 것으로 생각되는 바이다. 저자들은 재발성 수막구균 혈증으로 치료받은 환자에서 C7 결핍증을 경험하였기에 이를 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제48권1호
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• pp.101-103
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• 2005

저자들은 12세 여아로 수막구균 수막염에 이환 후 시행한 보체 검사에서 C9 결핍을 확인한 1례를 경험하였다. 수막구균 수막염에 이환된 환아에 대한 보체 검사는 보체 결핍을 갖는 환자와 가족 내 위험이 있는 사람을 찾아내어, 향후 예방접종 등의 예방과 감염 관리에 필요하다.

• Childhood Kidney Diseases
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• 제10권1호
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• pp.45-51
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• 2006

저자들은 C3의 감소와 육안적 혈뇨를 보인 환아에서 현미경적 혈뇨가 지속되면서 8주 후 시행한 검사에서도 C3의 감소가 지속되어 MPGN을 의심하고 신생검 예정이었다. 추적 관찰 중 수막구균 뇌수막염이 동반되었고, C3와 C5의 지속적인 감소와 C7과 C9의 일시적인 감소를 확인하였으며, 신생검에서 1형 MPGN으로 확인되었다. 본 증례의 아쉬운 점은 nephritic factor의 존재나 factor H의 결핍을 입증하여야 하나 아직까지 국내에서는 시행되지 않아 확인은 하지 못하였다. 앞으로 국내에서도 nephritic factor나 다양한 보체계(complement profile)에 대한 검사들이 시행되어야 할 것이다. 이제까지 국내에서는 후기 보체 결핍에 의하여 수막구균 뇌수막의 발생 증례 보고는 2004년 이후 여러 차례 있었지만, MPGN 환자에서 수막구균의 감염은 국내에 보고된 바가 없다. 본 증례를 통해 저보체혈증이 지속되는 일부의 MPGN 환자에서는 수막구균 감염 위험성이 존재하며, nephritic factor 검사와 함께 후기 보체 결핍에 대한 검사가 필요하다고 생각한다. 또한 수막구균성 뇌수막염에 이환된 모든 환자에 대해서 보체 검사를 시행하고 이런 환자에서 주기적인 소변 검사를 통하여 이상이 있을시 MPGN의 가능성을 고려하여 신생검을 고려하여야 할 것이다.

• Clinical and Experimental Pediatrics
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• 제52권6호
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• pp.721-724
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• 2009

수막구균(Meningococcus) 질환은 보체계의 이상과 관련이 있을 수 있다. 보체 7번은 5개의 말단 보체 단백질(terminal complement protein) 중 하나로 이것이 결핍되면 보체의 세포를 용해하는 작용을 잃게 되어 반복적인 감염, 특히 수막구균 감염에 대한 감수성이 증가한다. 2003년 9월 고열, 하지 동통, 두통과 점상 출혈로 외래에 내원한 11세 된 여자 환자가 입원 후 급격히 혼수 상태로 빠졌으나 즉각적인 치료로 결국 완전히 회복되었다. 환자의 최종 진단은 수막구균성 패혈증과 관절염이었다. 환자의 오빠도 비슷한 세균성 뇌수막염 가족력이 있어 저자들은 보체계 검사와 유전자 돌연변이(gene mutation)에 대해 분석하였고, 환자와 환자의 오빠는 보체 7번 유전자의 exon 4에 G394C에 돌연변이가 있는 선천성 보체 7번 결핍 환자였고, 아빠는 보인자로 밝혀졌다. 저자들은 예방적으로 4가 수막구균 백신(tetravalent polysaccharide meningococcal vaccine, $Menomune^{(R)}$ A/C/Y/W-135, Aventis Pasteur, Canada)을 3년마다 접종하기로 하였고 2004년 10월에 처음으로 접종하였다. 그러나 2006년 9월 오빠는 급성 세균성 뇌수막염(meningoencephalitis)으로 사망하였다. 이에 저자들은 2년마다 예방적 접종을 하기로 하였고, 환자는 2008년 9월에 3번째로 접종하였으며 16세 된 환자는 현재까지 건강한 상태이다. 저자들은 수막구균 감염과 보체 7번 유전자 네번째 intron의 3' 말단 splice acceptor 위치에 G to T 돌연변이(g.IVS4-1G> T)가 있는 선천성 보체 7번 결핍을 가진 한국인 한 가족을 보고하는 바이다.

• Journal of Veterinary Science
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• 제22권3호
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• pp.36.1-36.12
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• 2021

Background: Mouse hepatitis virus (MHV) A59 is a highly infectious pathogen and starts in the respiratory tract and progresses to systemic infection in laboratory mice. The complement system is an important part of the host immune response to viral infection. It is not clear the role of the classical complement pathway in MHV infection. Objectives: The purpose of this study was to determine the importance of the classical pathway in coronavirus pathogenesis by comparing C1qa KO mice and wild-type mice. Methods: We generated a C1qa KO mouse using CRISPR/Cas9 technology and compared the susceptibility to MHV A59 infection between C1qa KO and wild-type mice. Histopathological and immunohistochemical changes, viral loads, and chemokine expressions in both mice were measured. Results: MHV A59-infected C1qa KO mice showed severe histopathological changes, such as hepatocellular necrosis and interstitial pneumonia, compared to MHV A59-infected wild-type mice. Virus copy numbers in the olfactory bulb, liver, and lungs of C1qa KO mice were significantly higher than those of wild-type mice. The increase in viral copy numbers in C1qa KO mice was consistent with the histopathologic changes in organs. These results indicate that C1qa deficiency enhances susceptibility to MHV A59 systemic infection in mice. In addition, this enhanced susceptibility effect is associated with dramatic elevations in spleen IFN-γ, MIP-1 α, and MCP-1 in C1qa KO mice. Conclusions: These data suggest that C1qa deficiency enhances susceptibility to MHV A59 systemic infection, and activation of the classical complement pathway may be important for protecting the host against MHV A59 infection.

• Clinical and Experimental Pediatrics
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• 제52권6호
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• pp.649-654
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• 2009

The immune system is comprised of cells and molecules whose collective and coordinated response to the introduction of foreign substance is referred to as the immune response. Defense against microbes is mediated by the early reaction (innate immunity) and the late response (adaptive immunity). Innate immunity consists of the epithelial barrier, phagocytes, complement and natural killer cells. Adaptive immunity, a more complex defense reaction, consists of activation of later-developed lymphocytes that, when stimulated by exposure to infectious agents, increase in magnitude and defensive capabilities with each successive exposure. In this review we discuss recent advances in important primary immune deficiency disorders of innate immunity (chronic granulomatous disease, leukocyte adhesion deficiency) and adaptive immunity (severe combined immune deficiency, Wiskott- Aldrich syndrome).

• Childhood Kidney Diseases
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• 제25권1호
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• pp.29-34
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• 2021

C3 glomerulonephritis (C3GN), a rare condition associated with dysregulation of the alternative pathway of the complement system, is histopathologically characterized by isolated or dominant C3 deposition in the renal glomeruli. We report a case of C3GN associated with anti-complement factor H (CFH) autoantibodies and CHF-related protein deficiency in an adolescent male. A 16-year-old adolescent male was admitted to a hospital with a 1-month history of generalized edema prior to presentation. Persistent microscopic hematuria and low serum C3 levels were incidentally detected at 7 and 10 years of age, respectively. Laboratory test results revealed hypoalbuminemia, nephrotic-range proteinuria, microscopic hematuria, and normal serum creatinine levels. The serum C3 and C4 levels were 17 mg/dL (normal 80-150 mg/dL) and 22 mg/mL (17-40 mg/mL), respectively. Renal biopsy showed typical features of C3GN. Further investigations revealed positive results on plasma anti-CFH autoantibody testing and a homozygous deletion of CFHR1 and CFHR3, which encode CFH-related proteins 1 and 3, respectively. Proteinuria persisted despite treatment with intravenous methylprednisolone, mycophenolate mofetil, and angiotensin-receptor blocker; however, his renal function remained stable. In conclusion, anti-CFH autoantibodies serve as important contributors to C3GN. This is the first case report that describes C3GN in an adolescent Korean male with anti-CFH autoantibodies and homozygous CFHR1 and CFHR3 deletion.

• 기술사
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• 제33권1호
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• pp.61-67
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• 2000

Soysauce that has a long history, is a traditional food in the Orient area, and is supporesed the region of origination was northern of Korean peninsula or southern of Manchuria. After ward, it is convinced that spreaded to China continent, Korean peninsula md Japan. I have published a book named "Jang it′s History Culture and Industry" in 1999. in which illustrated the history of soysauce, but I cannot explain the details of history of Japanese soysauce because of deficiency of the data, so I hope a complement. Let me report a translation of KAWATA′s study, in here

• 대한한의진단학회지
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• 제18권1호
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• pp.1-10
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• 2014

Objectives Visual inspection is the first diagnostic method in Oriental medicine, and visual inspection of eyes is the one among them. This study was written in order to complement further understanding on visual inspection of eyes. Methods 1. Out of 102 photographs submitted to the Society of HyungSang Medicine in 2009, 27 portrait pictures were selected as samples in blind by 2 clinicians. The samples were copied to make 54 sample pictures, and then randomly assigned to 4 clinicians. 2. The 4 clinicians evaluated the 54 samples for excess and deficiency of the eyes. The results were recorded as 5-points-scale, and their average and standard deviation was calculated. 3. Intra and inter class reliability test were measured using SPSS 13. Results For intra- and inter-class correlation coefficient (ICC) values were measured as 0.654~0.967 and 0.756~ 0.783 respectively, with the P-value below 0.05. Out of 27 originally selected samples, 7 pictures were selected as Deficiency Samples (with 3 pictures of male and 4 of females), and 20 as Excess Samples (with 4 of male and 16 of female). Among them, Sample No. 1, 9, 22, and 26 were selected as models of 'Excessive Eyes' for females, no. 4 and 5 as 'Very Excessive Eyes' for male and females, and no. 15 as 'Moderate Eyes' for females. Conclusion This study is the first attempt of quantitative measurement of excess and deficiency using the Visual Inspection of eyes by the visual inspection experts. Still, additional studies are needed regarding the relationship visual inspection methods have with existing standards of diagnosis.

• Journal of Nutrition and Health
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• 제16권3호
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• pp.193-199
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• 1983

Clinical and epidemiologic data point to a causal interrelationship between nutritional deficiency and infectious illness. Both are major contributors to childhood morbidity and mortality, particulary in underprivileged population groups. Protein-calorie malnutrition depress a variety of immune funtions. Delayed hypersensitivity and number of T-lymphocytes are consistently reduced. The interrelationship between nutritional status and immune response was studied in 80 children aged under 6 years. According to the anthropometric assessment based on weight for height for age, 36 children were classified as normal, 22 as morderate malnutrition, and 22 as severe malnutrition. The following determinations were made : hemeglobin, hematocrit, serum albumin, immunoglobulin G, complement 3, and WBC levels. Results indicate that levels of Hb, Hct, serum albumin, and C3 concentration were decreased in moderate and severe malnutrition children. However, levels of IgG and WBC were not affected by the nutritional status. It is suggested that nutritional status has more profound effects on complement system than humoral immunity.