• Pediatric Infection and Vaccine
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• v.11 no.2
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• pp.212-215
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• 2004

The complement system is important in the generation of the normal inflammatory response and in host defense against systemic infection. Therefore, inherited or acquired deficiency of complement is associated with an increased frequency of infection. As a major effector of the complement cascade, the membrane attack complex is responsible for direct complement dependent serum bactericidal activity. Especially late complement component deficiency has a markedly increased risk of meningococcal infection and is subject to recurrent infection. We experienced a patient who had recurrent meningococcal meningitis and septicemia. The patient was 13-years old boy and he had a recurrent episode after 20 months. At second admission, we examined complement level and C7 deficiency was confirmed. He was treated without complication. We report a case of deficiency of C7 with recurrent meningococcal meningitis and septicemia.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.101-103
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• 2005

Meningococcal disease is not rarely associated with abnormalities of the complement system. We experienced a case of C9 deficiency with meningococcal meningitis from a 12-year-old girl. Identification of complement deficiency has implications for management, including family studies, prophylaxis, vaccination, and altered threshold for infection screening and treatment.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.45-51
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• 2006

Hypocomplementemia is found in all types of membranoproliferative glomerulonephritis (MPGN) but not in all patients. Hypocomplementemia can be ascribed to at least two circulating complement reactive modalities. The activation of the classical pathway produced by circulating immune complexes and the presence in the blood of anticomplement autoantibodies, called 'nephritic factor'(NF). The activation of the classical pathway by circulating immune complexes is probably the major mechanism responsible for hypocomplementemia in idiopathic MPGN type I. Nephritic factors have been shown to be responsible for the hypocomplementemia in both MPGN type II and III. NFa is probably the major mechanism responsible for the hypocomplementemia of idiopathic MPGN type II. NFt appears to be solely responsible for the hypocomplementemia in MPGN type III. Judging from the complement profile, NFt also may be present in some patients with MPGN type I. Although infection by meningococcus has been associated with deficiency of any of the plasmatic proteins of complement, it more commonly involves deficiency of the terminal components of the complement pathway(C5-C9). We experienced a patient who had MPGN and meningococcal meningitis. We examined the complement level and significantly lower levels of C3, C5 were found persistently. C7 was low at first and it returned to normal range after 2 months. C9 was normal at first, and was low after 2 months. This is the first reported case in which MPGN with meningococcal meningitis occurred.

• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.721-724
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• 2009

Meningococcal infections can be associated with abnormalities of the complement system, which contains 5 terminal complement proteins. Furthermore, deficiencies in 1 of these 5, complement component 7 (C7), leads to the loss of complement lytic function, and affected patients show increased susceptibility to recurrent meningococcal meningitis and systemic Neisseria gonorrhoeae infection. In September 2003, an 11-year-old female patient presented at our outpatient department with high fever, lower leg pain, headache, and petechiaes. She rapidly progressed to coma but later achieved full recovery due to prompt treatment. Her final diagnosis was meningococcal sepsis and arthritis. Her elder brother also had a similar bacterial meningoencephalitis history, which encouraged us to perform analyses for complement component and gene mutations. Resultantly, both the brother and sister were found to have the same mutation in the C7 gene. Subsequently, vaccinations of the meningococcal vaccine meningococcal vaccine ($Menomune^{(R)}$) were administered. However, in September 2006, the brother expired due to acute micrococcus meningoencephalitis. At present, the 16-year-old female patient is healthy. Here, we report a Korean family with a hereditary C7 deficiency with susceptibility to meningococcal infections due to C7 gene mutation.

• Journal of Veterinary Science
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• v.22 no.3
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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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• v.52 no.6
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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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• v.25 no.1
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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.

• Journal of the Korean Professional Engineers Association
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• v.33 no.1
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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

• The Journal of the Society of Korean Medicine Diagnostics
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• v.18 no.1
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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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• v.16 no.3
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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.