• 제목/요약/키워드: IgG deficiency

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Hyper IgM Syndrome 환자에서 얻은 림프절 및 말초혈액 B세포의 특성 (Characterization of B Cells of Lymph Nodes and Peripheral Blood in a Patient with Hyper IgM Syndrome)

  • 김동수;신경미;양우익;신전수;송창화;조은경
    • Clinical and Experimental Pediatrics
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    • 제46권2호
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    • pp.128-136
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    • 2003
  • 목 적 : Hyper IgM syndrome(HIGM)은 크게 세 부류로 나누는데, CD40L 분자의 돌연변이로 초래되며 X-linked로 유전되는 형태를 HIGM1이라고 하고, 상염색체성 열성 형태로 유전되면서 CD40L는 정상적으로 표현되는 형태로 activation-induced cytidine deaminase(AID) 유전자에 이상 때문에 오는 경우를 HIGM2로 분류하고 있다. 다른 한 부류는 X-linked HIGM 증후군의 매우 드문 한 형태로서 발한 저하성 외배엽 이형성증이 동반된 경우로, 이 질환은 전사인자인 nuclear factor ${\kappa}B$의 활성화에 관여하는 nuclear factor ${\kappa}B$ essential modulator를 coding하는 유전자의 돌연변이 때문에 오는 것으로 알려져 있다. 연구자들은 HIGM2와 유사하지만 AID 유전자에 변이는 없는 새로운 형태의 HIGM 환자의 말초 B세포를 이용하여 병인을 조사하고자 본 연구를 시도하였다. 방 법 : 환자의 말초혈액 단핵구를 분리하고, EBV로 immortalization을 시켜 Cycle TEST PLUS DNA Reagent kit를 이용하여 cell cycle을 분석하였다. 환자의 말초혈액 T 세포에서 CD40L의 표현을 immunostaining으로 알아보고, RNA를 추출하여 RT-PCR을 하고 direct sequencing을 통하여 CD40L 유전자의 돌연변이 부위를 찾아보았다. 아울러 AID 유전자의 돌연변이를 찾기 위하여 같은 방법으로 sequencing하고 조사하였다. 환자의 림프절을 병리학적인 검사를 시행하고 CD3, CD23, CD40, Fas-L, bcl-2, BAX의 표현을 알아보기 위하여 immunostaining을 실시하였다. 결 과: 림프절의 광학적 소견은 반응성 여포증식의 소견을 보였으며, 여포와 단구양 증식은 B-세포 표시인자인 L26에 양성을 보였고, 대부분의 형질세포는 IgM에 양성을 보였다. 여포는 CD40, Fas, BAX에 양성반응을 보이고, bcl-2와 Fas-L에 음성반응을 보였다. 말초혈액 B 림프구를 이용하여 cell cycle을 분석한 결과 정상(17.9%)에 비하여 G2/mitosis phase(M3 in figure)가 현저하게 감소(8.5%)되어 있는 양상을 보여주고 있으며, IL-4로 자극한 경우에는 정상인에서 보여주는 양상으로 회복되는 양상을 볼 수 있었다. 단핵수에서 CD40L의 표현은 정상이었고 CD40L 유전자에도 돌연변이는 발견할 수 없었으며, HuAID 유전자에도 돌연변이를 발견할 수 없었다. 결 론 : 환자의 말초혈액 B림프구를 통한 연구 결과, 기존에 보고 되어진 HIGM2 형태와 임상적으로는 비슷하지만, 정상적인 AID유전자를 보이고, G2/mitosis phase가 정상에 비하여 감소된, 새로운 형태의 HIGM이라고 여겨진다.

아연 섭취 수준이 고지방 식이로 유도된 비만쥐의 면역 기능에 미치는 영향 (Effects of Zn Intake on Immune Responses in High Fat Diet-induced Obese Rats)

  • 김현숙;승정자
    • 한국식품영양과학회지
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    • 제28권2호
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    • pp.464-470
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    • 1999
  • The purpose of this study was to investigate the effect of zinc intake on immune responses in high fat diet induced obese rats. The immune status was assessed by the measurements of immunoglobulins (IgG, A, and M) production by SRBC(sheep red blood cell) with mitogen in vivo. The delayed type hypersensitivity(DTH) response was also measured as an index of cell mediated immunity. The re sults are summarized as follows : 1) There were no significant differences in the feed intake, weight gain and feed efficiency ratio of obese rats by the different dietary zinc levels. 2) White blood cell (WBC) counts were significantly affected as the dietary zinc levels decreased. The capacity of Ig M production in obese rats was significantly higher in normal zinc group than that of low and high zinc group. Cell mediated immune response evaluated by means of DTH testing has also been found to be highly impaired by zinc deficiency and overload. From these observations, it was suggested that adequate levels of zinc may promote the immune function of obese individuals. The relationship and its functional role of the zinc in obesity remains to be further studied.

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사람 혈청 트란스페린수용체의 단클론 항체를 이용한 방사면역측정과 철영양상태의 진단 (Immunoradiometric Assay using Monoclonal Antibody Against Human Serum Transferrin Receptor for Diagnosis of Iron Deficiency)

  • 김승렬
    • Journal of Nutrition and Health
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    • 제29권9호
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    • pp.971-980
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    • 1996
  • The soluble transferrin receptor(TfR) in human serum has been shown recently to be a truncated form of intact membrane bound receptor containing most of the extracellular domain. We purfied the transferin-free TfR from human serum by immounoaffinity chromatography which produced the single protein identity in high resolution gel chormatography. The monoclonal antibodies(MAb) against purifed serum TfR were produced by fusion of spleen cells o fimmunized Balb/c mice and SP2 cells. Ten hybrids producing MAb specific for serum TfR were identifed and determine their iostypes. A immunoraddiometric assay (IRMA) for serum TfR was established using two monoclonal IgG1 antibodies as the coating and indicator antibodies on the bosis of their suitability in sandwich IRMA of serum TfR. The mean serum TfR levels in the 15 normal male, 15 normal female, and 19 iron-deficient subjects were 5.4$\pm$0.98, 4.6$\pm$0/76, and 18.0$\pm$12.8mg/1, respectively, and the difference in mean values between normal and iron deficient subjects was significant(p=0.0005). There existed the inverse logarithmic relationship(r=-0.9336, p<0.0001) between the serum TfR and ferritin levels.

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Production of Monoclonal Antibody to the Infective Stage of Cryptosporidium Infection in AIDS Patients

  • Cho, Myung-Hwan
    • Journal of Microbiology and Biotechnology
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    • 제7권3호
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    • pp.189-193
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    • 1997
  • Cryptosporidium parvum has been recognized as a significant cause of life-threatening diarrhea in Acquired ImmunoDeficiency Syndrome (AIDS) patients. Clinical diagnosis of cryptosporidial infections has been primarily based on the detection of infective stage, oocysts, in stools. Anti-Cryptosporidium oocyst monoclonal antibody (mAb), IgG2a, recognizing an antigen of 97 kDa was generated to be used for diagnosis of Cryptosporidium infection in AIDS patients using an immunofluorecence. It appeared to react with the surface antigens. Transmission electron micrographs of the infective stage of Cryptosporidium recognized by this mAb demonstrated sporolulated oocysts, which measure $4~6{\mu}m$, and sporozoites excysting from oocysts.

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6세미만 도시 주변거주 어린이의 면역능력에 따르는 영양상태 판정에 관한 연구 (A Survey of Nutritional-Immunologic Interactions in the Children Under 6 Years Old in the Suburbs of Seoul)

  • 이인실;김연중;김화영;김숙희;홍영자
    • 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.

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Value of spray-dried egg in pig nursery diets

  • Song, Minho;Kim, Sheena;Kim, Younghwa;Park, Juncheol;Kim, Younghoon
    • 농업과학연구
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    • 제42권3호
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    • pp.207-213
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    • 2015
  • High-quality protein ingredients have been used in nursery diets, in spite of expensive ingredients, to minimize nutritional deficiency and disease problems. Recent dramatic increases in prices of protein products for nursery diets have exacerbated the challenge. Spray-dried egg may be a part of the solutions. Therefore, this review describes the value of spray-dried egg in nursery diets as a high-quality protein source. Spray-dried egg is egg by-product and is produced by only eggs without shell that are below the USDA Grade B standards. Spray-dried egg is an excellent nutrient source: 1) highly digestible, 2) excellent balance of amino acids, 3) rich content of fat, and 4) high metabolizable energy. These can be attributed to growth of nursery pigs. Beyond the provision of bioavailable nutrients, spray-dried egg also may provide specific physiological benefits. Spray-dried egg contains 1) immunoglobulin antibodies (IgY: IgG in egg yolk) that may attach to intestinal pathogens and excrete them and 2) lysozymes antimicrobial protein that can damage bacteria cell wall. Thereby feeding spray-dried egg may reduce concentration of intestinal pathogen and thus improve potential gut health or enteric disease resistance in nursery pigs. This is important for physiologically immature weaned pigs. Based on these benefits, spray-dried egg is believed to have the same benefits as spray-dried plasma protein and milk products in diets for nursery pigs. Therefore, it is suggested that spray-dried egg has a great potential as a valuable protein source in nursery diets.

Early Growth Response-1 Plays a Non-redundant Role in the Differentiation of B Cells into Plasma Cells

  • Oh, Yeon-Kyung;Jang, Eunkyeong;Paik, Doo-Jin;Youn, Jeehee
    • IMMUNE NETWORK
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    • 제15권3호
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    • pp.161-166
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    • 2015
  • Early growth response (Egr)-1 is a $Cys_2-His_2-type$ zincfinger transcription factor. It has been shown to induce survival and proliferation of immature and mature B cells, respectively, but its role in the differentiation of B cells into plasma cells remains unclear. To examine the effects of Egr-1 deficiency on the activation of B cells, naive B cells from $Egr1^{-/-}$mice and their wild-type (WT) littermates were activated to proliferate and differentiate, and then assayed by FACS. Proportions of cells undergoing proliferation and apoptosis did not differ between $Egr1^{-/-}$ and WT mice. However, $Egr1^{-/-}$ B cells gave rise to fewer plasma cells than WT B cells. Consistently, $Egr1^{-/-}$ mice produced significantly lower titer of antigen-specific IgG than their WT littermates upon immunization. Our results demonstrate that Egr-1 participates in the differentiation program of B cells into plasma cells, while it is dispensable for the proliferation and survival of mature B cells.

폐렴 구균 농흉으로 수술적 치료까지 받은 범저감마글로불린혈증 1례 (A Case of Panhypogammaglobulinemia in Patient with Pneumococcal Empyema)

  • 최재원;오승정;조병수;차성호;최용묵
    • Pediatric Infection and Vaccine
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    • 제2권2호
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    • pp.194-199
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    • 1995
  • Most boys afflicted with panhypogammaglobulinemia, also known as X-linked agammaglobulinemia, remain healthy during the first 6 to 12 months of life because of protection by maternally transmitted IgG antibodies. Thereafter, they repeatedly acquire infections with high-grade pathogens, such as pneumococci, streptococci, and hemophilus unless given antibiotics or immunoglobulin replacement therapy. We experienced a case of panhypogammaglobulinemia in a 4 years old boy. He had been suffered from recurrent upper respiratory tract infection, otitis media and pneumonia since late infancy. He was admitted due to right pleural effusion with pneumonia, and streptococcus pneumoniae was isolated from pleural fluid and blood cultures. His immune status revealed panhypogammaglobulinemia and deficiency in mature B lymphocyte. He was treated with appropriate antibiotics therapy, but showed poor responses. He was transferred to department of thoracic surgery, and received minithoracotomy (decortication) operation. He was successfully treated with operation, antibiotics, and IV gammaglobulin infusions. Now he is being followed with periodic IV gammaglobulin replacement therapy.

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면역결핍 모델에서 β-1,3/1,6-glucan과 유산균을 이용한 in vivo 면역 활성 조절 효과 (Immunomodulatory effects of β-1,3/1,6-glucan and lactic acid bacteria in LP-BM5 murine leukemia viruses-induced murine acquired immune deficiency syndrome)

  • 김민수;김중수;류민정;김기홍;황권택
    • 한국식품저장유통학회지
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    • 제24권8호
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    • pp.1158-1167
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    • 2017
  • 본 연구에서는 LP-BM5 murine AIDS virus에 감염된 면역 결핍동물 모델을 이용하여 실험기간 10주 동안 ${\beta}$-1,3/1,6-glucan, 유산균 및 ${\beta}$-1,3/1,6-glucan+유산균의 식이가 면역활성에 미치는 영향을 조사하였다. 그 결과 LP-BM5 murine AIDS virus에 감염으로 면역능이 떨어진 T세포 증식능은 홍삼대조군과 비교하여도 유의적으로 감소된 T 세포증식능을 증가시키는 것으로 나타났고, B 세포 증식능은 감염 대조군에 비하여 유의적으로 증가된 B 세포 증식능을 감소되었다. cytokine 생성능에서는 Th1 type cytokine중에서 IL-2, IL-12, IL-15는 감염대조군에 비하여 분비량을 증가시키는 것을 확인할 수 있었고, IFN-${\gamma}$는 유산균과 ${\beta}$-1,3/1,6-glucan을 각각 처리군이 혼합처리군보다 증식능이 증가하였다. TNF-${\alpha}$는 감염대조군에 비하여 유의적으로 감소하였다. Th2 cytokine 들의 분비량 측정에서 IL-4, IL-6, IL-10 측정 결과 감염대조군에서 유의적으로 억제되어 Th1/Th2 type cytokine 발현을 조절하여 면역항상성을 유지하는 것으로 보였다. 면역글로블린 분비량측정에서 IgE, IgA, IgG 모두 감염대조군에 비하여 유의적으로 떨어지는 것으로 나타났다. 이로서LP-BM5 murine AIDS virus에 감염된 면역 결핍동물 모델에 ${\beta}$-1,3/1,6-glucan+유산균군을 혼합처리로 면역조절의 효능이 있음을 확인할 수 있었다.

거대세포 바이러스 간염이 동반된 장성 선단 피부염 1례 (A Case of Acrodermatitis Enteropathica Associated with CMV Hepatitis)

  • 김광렬;박재옥;신상만
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제2권2호
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    • pp.233-239
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    • 1999
  • Acrodermatitis enteropethica (AE) is a rare autosomal recessive disorder of zinc absorption leading to chronic diarrhea and characteristic skin lesion. The term is also applied to any acquired zinc deficiency state resulting in the same clinical pictures. We experienced one case of AE in 1 month old male infant who had bacterial enterocolitis. The skin around mouth, anus, eyes, ears, hands and legs became reddish, vesicular and eczematoid. Serum zinc level was decreased to $51.4\;{\mu}g/dL$ (N=70~150). Endoscopic finding revealed pale gastric mucosa and villous atrophy of small intestine. Biopsy finding of small intestine showed no villi due to mucosal atrophy. On 13 day of admission jaundice with DIC were noted and AST & ALT were elevated to 110 & 36.8 IU/L, respectively. Diarrhea was improved but jaundice and liver function were not recovered until discharge from hospital. After discharge when the patient was 4 months of age serum bilirubin and AST/ALT had not been normalized. CMV shell vial culture of urine and CMV Ig G antibody were positive. So intravenous ganciclovir injection of 7.5 mg/kg, two times a day for 2 weeks and then 10 mg/kg/day for 3 months was done from 4 to 6 months of age. No virus was found in the urine and AST & ALT were normalized at 2 months after stopping ganciclovir treatment.

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