• Title/Summary/Keyword: Complement 7 deficiency

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A Case of Deficiency of the Seventh Component of Complement with Recurrence of Meningococcal Meningitis and Septicemia (C7 결핍증과 연관된 재발성 수막구균 혈증 1례)

  • Lee, Jong-Seung;Yoo, Jung-Min;Yoo, Soo-Jung;Ko, Tae-Sung;Yoo, Han-Wook
    • 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.

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A Korean familial case of hereditary complement 7 deficiency (선천성 보체 7번 결핍을 가진 한국인 한 가족)

  • Kim, Moon Kyu;Lee, Kyung Yul;Lee, Jun Hwa
    • 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.

A Case of Membranoproliferative Glomerulonephritis Associated with Complement Deficiency and Meningococcal Meningitis (수막구균 뇌수막염과 보체 결핍이 동반된 막증식성 사구체신염 1례)

  • Kwon Sang-Mi;Park Kwan-Kyu;Lee Gyeong-Hoon
    • 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.

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C3 Glomerulonephritis associated with Anti-complement Factor H Autoantibodies in an Adolescent Male: A Case Report

  • Hyun, HyeSun;Kang, Hee Gyung;Cho, UiJu;Ha, Il-Soo;Cheong, Hae Il
    • 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.

A Study on Reliability and Evaluate Deficiency and Excess on Visual Inspection of Eyes (안진(眼診)을 통한 허실(虛實) 평가 및 신뢰도 연구)

  • Seo, Jae-Ho;Choi, Jin-Yong;Oh, Whan-Sup;Park, Young-Bae;Park, Young-Jae
    • 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.

A Study on Routine Formulas and Downgraders of Request Act in High School English Textbooks

  • Yang, Eun-Mi
    • English Language & Literature Teaching
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    • v.11 no.2
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    • pp.111-134
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    • 2005
  • This paper examines high school English textbooks to ascertain if they appropriately reflect the kinds and frequencies of routine formulas and downgraders of request act used by English native speakers. It is important to present authentic routine formulas in textbooks for students to acquire proper, efficient and safe communication strategies to communicate with other English speakers. For the analysis, currently available 7 series of 21 high school English textbooks under the $7^{th}$ National Curriculum were selected. Each series of textbooks contains 3 school grade textbooks as High School English, High School English I, and High School English II. The results show that the high school English textbooks generally demonstrate a secund reflection of the English native speakers' use of request strategies and downgraders. That is, the textbooks were found to have presented mostly casual forms of routine formulas while they have not presented sufficient coverage of elaborated polite routine formulas for requesting which English native speakers frequently use. The presence of some kinds of the frequently used downgraders was also very small in proportion in the textbooks. More effort should be given to complement the deficiency in this area by teachers and researchers.

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Characterization of a gene encoding ornithine carbamoyltransferase from rice

  • Islam Sikdar, Shafiqul;Kim, Jung-Sup
    • Journal of Plant Biotechnology
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    • v.36 no.4
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    • pp.397-402
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    • 2009
  • Ornithinine carbamoyltransferase (OTC) is an enzyme that catalyzes the key step in arginine biosynthesis in bacteria and plants. OTC is also involved in the urea cycle and deficiency of the enzyme in human leads to disease. The argF gene encoding OTC has been reported in many bacteria and few plants. Here we report the characterization of a gene encoding OTC from rice (OsOTC). Analysis of a cDNA sequence from rice revealed that the full-length open reading frame of OsOTC consisted of 367 amino acids, corresponding to a protein of approximately 39.7 kDa. The predicted amino acid sequence of OsOTC harbor distinct five OTC signature sites and is highly homologous to that of enzymes of plants, animals and many bacterial OTCs. Expression of OsOTC in argF mutants of Escherichia coli showed that the gene was able to functionally complement to the mutant. These results suggest that the OsOTC encode a protein for ornithine carbamoyltransferase in rice.

Case of Combined Acute Poststreptococcal Glomerulonephritis and Acute Invasive Meningococcal Disease (A군 연구균 감염 후 급성 사구체 신염과 동반된 침습성 수막구균감염증 1례)

  • Jeon, Hosang;Hahn, Wonho;Park, Dongkyun;Cha, Sungho
    • Pediatric Infection and Vaccine
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    • v.11 no.1
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    • pp.126-130
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    • 2004
  • Neisseria meningitidis is a leading cause of meningitis and septicemia in children and young adults. Meningococcemia, the most severe form of infection, represents a part of the various spectrum of the illness, and its clinical manifestations varied from mild fever and occult bacteriemia to fulminant catastrophic events(e.g., sepsis, shock, and death) within a few hours after onset of symptoms. Complement deficiencies, either congenital or acqired, increase the risk for invasive meningococcal disease. Since C3 plays a critical role in the complement cascade, congenital C3 deficiency or conditions that decrease C3(e.g., systemic lupus erythematosus, cirrhosis, nephritis, C3 nephritic factor) increase the risk for invasive disease due to pyogenic bacteria including N. meningitidis. We had experienced a case of meningococcemia and meningococcal meningitis presenting with mild fever, petechiae, and purpura. The patient was a 7-year old male. He had mild fever, vomiting, headache, nuchal rigidity, petechiae and purpura on both lower extremities, which spreaded to the whole body. His blood culture grew Neisseria meningitidis. The diagnosis of meningococcal meningitis was confirmed by examination of cerebrospinal fluid. The clinical symptoms of the illness were improved after treatment of antibiotics(cefotaxime) for 12 days. However the patient had developed generalized edema, microscopic hematuria & proteinuria on the third day after admission. High ASO titer and low serum C3 level were also noted. The microscopic hematuria returned to normal about 2 months after discharge. The C3 level returned to normal range about 3~4 months after discharge. we report a case of combined acute poststreptococcal glomerulonephritis and acute invasive meningococcal disease with reference review.

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Nutrition Survey of Children of a Day Care Center in the Low Income Area of Pusan I. A Study on Nutrient Intake and Nutritional Status (부산 시내 일부 저소득층 유아원 원아의 영양실태에 관한 연구 I. 영양섭취실태 및 건강실태 조사)

  • 이정숙
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.22 no.1
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    • pp.27-33
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    • 1993
  • A nutrition survey of 99 children, 3 to 6 years old, in the day care center, situated in Kamman-dong of Pusan, as the location of one of the socioeconomically vulnerable groups, was undertaken between July 1 and 14, 1992, to investigate dietary intake and nutritional status. The results are summarized as follows. Hematocrit value was 38.9%. Mean content of serum iron was 53.5$\mu\textrm{g}$/100$m\ell$ and 17.2% of the subjects were below the borderline of anemia. Mean contents of serum total protein, serum albumin and serum complement $C_3$were at an acceptable level, But, 27% of the subjects were below the borderline of protein deficiency, Energy intake of the children was 68.6~87.5% of the RDAs and mean protein intake was 94.1~114.1% of the recommendation. Low intake of Ca, Fe, vitamin A, B$_1$, B$_2$, C, niarin were also found. The survey found 10% of the subjects skipped a meal once a day, The children intake 3.3~5.5 kinds of the food every meal. The following percentages of the children intake below 75% of the RDAs ; 68% of the children for Fe, 60.2% for vitamin C, 58.3% for niacin, 58.2% for Ca, 46.6% for energy, 37.9% of vitamin B$_2$, 37.8% of vitamin B$_1$.

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The Meridian Interpretation of Atopic Dermatitis Phases (아토피 피부염의 발생시긱별 변화에 대한 경락학적 해석)

  • Cho Yong-ju;Kim Jin-ju
    • The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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    • v.17 no.1
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    • pp.1-15
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    • 2004
  • The different growth steps of human show the different energetic phenomenon such as the strength of triple energizer, the sale of extra meridian, and the stability of 12 meridians. So we have to understand the physiological and pathological phenomena on this point of view. Especially atopic dermatitis means the loss of adaptability under the lack of genuine energy. If you approach to this disease not with any perception of excess or deficiency of the human genuine energy but with simple symptom enumeration or partial diagnosis, it will be dangerous. So we must approach to this disease with more concrete and objective body-centered standard. Thus when we cure and diagnose infants with this disease, we should focus on stablizing the "Energy of Earth(土)" digestive ability, under the consideration of inducement to the enough "Byun-Jeung-Hu(變蒸候)" and the function of Triple Energizer. Through this process we can lead infants to the beginning equipment state(始全). Until 7 years old, we should stabilize the Link Vessel(維脈) by clarifying interior and exterior classification of Nutritive Element and Defensive Energy to control the child's energy of metal(金), water(水), wood(木) and fire(火) in the basis of the energy of earth(土). And also we should stabilize the Heel Vessel(?脈) through the Water and Fire complement each other to control the movements and to control sleep and awakening. Through this process, we can lead children to the basal equipment state(本全). After then, until adolescent phase, we should control 12 meridian and 8 extra meridian by leading vital function to be harmonized and prosperous on the basis of the meridian to reach the mature equipment state(旣全). Adult atopic dermatitis should be controlled through conciliation between meridian and internal organs by differentiating male from female.

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