일과성 Immunoglobulin G 결핍증이 있는 아토피피부염 환아에서 면역글로불린 치료

백종욱 ( Jong Uk Baek ),이혜란 ( Hae Ran Lee ),이소연 ( So Yeon Lee ) 대한천식알레르기학회 2012 천식 및 알레르기 Vol.32 No.3

Background: Some studies have reported that intravenous immunoglobulin is an effective treatment of severe atopic dermatitis. Case History: A 3-month-old by present suffered from atopic dermatitis with skin infection since the age of 50 days. He was fed on breast milk and had no familial history of allergy. Upon admission, his body weight was 6.9 kg (50 percentile), and the Severity Scoring of Atopic Dermatitis was 80. Laboratory test results were as follows: white blood cell 23.1×103/uL, eosinophil (%) 29%, immunoglobulin E 696.2 IU/mL, immunoglobulin G 126 mg/dL (176∼601 mg/dL), immunoglobulin A 14.7 mg/dL (1.3∼53 mg/dL), immunoglobulin M 31.7 mg/dL (17∼105 mg/dL). Staphylococcus aureus was detected in the erosive cheek by skin cultures. Specific immunoglobulin E levels to egg white, soy, and peanut were elevated. He was initially treated with intravenous antibiotics, wet dressing, steroid ointments, and mother`s dietary restriction. However, the skin infection and atopic dermatitis remained uncontrolled, and the immunoglobulin G level was still below the normal range. The patient was given intravenous immunoglobulin 1 g/kg/day for 2 consecutive days. Results: After 4 days, the skin infection was controlled. After 9 months, immunoglobulin G level was normalized. Conclusion: We reported a patient who was diagnosed with severe atopic dermatitis with transient immunoglobulin G deficiency and whose symptoms improved after intravenous administration of immunoglobulin.

DEU-7 Derived from Ulmus macrocarpa Improved Immune Functions in Cyclophosphamide-treated Mice

Kyung-Hwa Kang(강경화),Ji Su Go(고지수),Inhwan Lee(이상호),Sang Ho Lee(이인환),Sung Do Lee(이성도),Deok Won Kim(김덕원),Jong-Hwan Lee(이종환),HyeJin Hwang(황혜진),Sook Kyung Hyun(현숙경),Byoung Woo KIM(김병우),Chul Min Kim(김철민),Ky 한국생명과학회 2015 생명과학회지 Vol.25 No.10

고량강(Alpinia officinarum), 산초(Zanthoxylum schinifolium), 황금(Scutellaria baicalensis Georgi), 왕느릅나무(Ulmus macrocarpa Hance), 네 종류의 식물성 한방 약재의 면역증강효능을 cyclophosphamide를 처리한 동물모델을 이용하여 조사하였다. 실험은 cyclophosphamide로 면역억제를 유도한 후 네 종류의 한방 약재를 식이하여 cyclophosphamide에 의해 억제된 면역인자의 회복 여부를 조사하였다. 네 종류의 한방 약재를 동일한 방법으로 열수 추출한 후 동일한 농도로 마우스에 처리하였다. 이 중 왕느릅나무 열수 추출물(DEU-7)이 cyclophosphamide의 영향으로 면역 억제된 마우스에서 면역인자 혈중 농도를 정상치 준하는 또는 가깝게 유지하는 것으로 나타내었다. DEU-7에 의해 비장의 무게와 비장세포수는 증가하였으나 간과 흉선과 같은 다른 장기에는 통계적으로 유의한 변화가 없었다. Ex vivo 조건에서 DEU-7은 비장세포의 사멸을 지연시키는 것으로 나타났다. 중요한 면역인자인 IL-2와 IL-4 cytokine은 면역억제로 농도가 감소되었으나 DEU-7이 약 2배와 3배 향상시키는 것으로 나타났으며, 이는 정상치의 약 1/2 수준이었다. IgM과 IgG의 농도는 cyclophosphamide 처리로 정상치의 약 1/2로 떨어졌으나, DEU-7에 의해서 정상치와 동일한 농도로 증가하였다. IL-2와 IL-4 결과에서 DEU-7은 T 림프구에 영향을 줄 수 있다고 생각되며, 또한 IgM과 IgG의 결과로서 B 림프구에 영향을 줄 수 있다고 생각된다. 선천성 면역에 중요한 면역인자인 TNF-α, IL-12과 IFN-γ 역시 cyclophosphamide에 의해 농도가 감소되었으나 DEU-7에 의해 정상치에 가깝게 회복되었다. 따라서, DEU-7은 면역 억제 또는 감소된 상태를 정상 상태로 회복 또는 유지하는 기능이 있는 것으로 생각된다. The present study investigated the immunomodulatory properties of four different medicinal plants in a cyclophosphamide-treated Balb/c mouse model. One of the four plants, Ulmus macrocarpa, showed partial resistance against immune suppression induced by cyclophosphamide. The bark of U. macrocarpa, commonly known as the Chinese elm, has been used as a pharmaceutical material in Korean traditional medicine to treat bacterial inflammation and induce wound healing. In this study, water extract of U. macrocarpa, named DEU-7, was used for its immunomodulating functional activity. DEU-7 increased the weight of the spleen and the number of splenocytes but did not significantly affect the liver, kidney, and thymus in vivo. A splenocyte viability assay confirmed that DEU-7 influenced ex vivo splenocyte survival. DEU-7 also increased the levels of cytokines, such as IL-2 and IL-4, and immunoglobulins, such as IgM, IgG, and IgA. These results indicated that DEU-7 is involved in the activation of T and B lymphocytes. In addition, DEU-7 was able to maintain the production of cytokines, such as TNF-α, IL-12, and IFN-γ, in the condition of cyclophosphamide-induced immune suppression, suggesting that DEU-7 activated innate immune cells, even under immune suppression. We concluded that DEU-7 aids immunological homeostasis, thereby preventing immune suppression, and aids both innate and adaptive immune response by maintaining the levels of various cytokines and immunoglobulins. Consequently, it is worth investigating the potential of DEU-7 as a supplemental source for immune-enhancing agents.

Infliximab Treatment for Intravenous Immunoglobulin-resistant Kawasaki Disease: a Multicenter Study in Korea

Gyu Hur,Min Seob Song,Sejung Sohn,이형두,Gi Beom Kim,Hwa Jin Cho,Kyung Lim Yoon,Chan Uhng Joo,Myung Chul Hyun,Chul-HoKim 대한심장학회 2019 Korean Circulation Journal Vol.49 No.2

Background and Objectives: We investigated the status of infliximab use in intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients and the incidence of coronary artery aneurysms (CAAs) according to treatment regimens. Methods: Between March 2010 and February 2017, 16 hospitals participated in this study. A total of 102 (32.3±19.9 months, 72 males) who received infliximab at any time after first IVIG treatment failure were enrolled. Data were retrospectively collected using a questionnaire. Results: Subjects were divided into two groups according to the timing of infliximab administration. Early treatment (group 1) had shorter fever duration (10.5±4.4 days) until infliximab infusion than that in late treatment (group 2) (16.4±4.5 days; p<0.001). We investigated the response rate to infliximab and the incidence of significant CAA (z-score >5). Overall response rate to infliximab was 89/102 (87.3%) and the incidence of significant CAA was lower in group 1 than in group 2 (1/42 [2.4%] vs. 17/60 [28.3%], p<0.001). Conclusions: This study suggests that the early administration of infliximab may reduce the incidence of significant CAA in patients with IVIG-resistant KD. However, further prospective randomized studies with larger sample sizes are required.

Multicenter, Single-Arm, Phase IV Study of Combined Aspirin and High-Dose “IVIG-SN” Therapy for Pediatric Patients with Kawasaki Disease

윤경림,이해용,유정진,이재영,한미영,김기용,허준 대한심장학회 2017 Korean Circulation Journal Vol.47 No.2

Background and Objectives: Intravenous immunoglobulin-SN (IVIG-SN) is a new human immunoglobulin product. Its safety is ensured by pathogen-elimination steps comprising solvent/detergent treatment and a nanofiltration process. This multicenter clinical study was designed to evaluate the efficacy and safety of combined aspirin and high-dose IVIG-SN therapy in pediatric patients with Kawasaki disease (KD). Subjects and Methods: We evaluated coronary artery lesions (CALs) at 2 and 7 weeks after administering IVIG-SN; total fever duration; and variations in erythrocyte sedimentation rate, N-terminal pro B-type natriuretic peptide or B-type natriuretic peptide, and creatine kinase-myocardial band level before and after treatment with IVIG-SN (2 g/kg). Adverse events were monitored. Results: Forty-five patients were enrolled, three of whom were excluded according to the exclusion criteria; the other 42 completed the study. The male:female ratio was 0.91:1, and the mean age was 29.11±17.23 months. The mean fever duration before IVIG-SN treatment was 6.45±1.30 days. Although most patients had complete KD (40 patients, 90.91%), four had atypical KD (9.09%). After IVIG-SN treatment, one patient (2.38%) had CALs, which was significantly lower than the incidence reported previously (15%) (p=0.022), but not significantly different from recent data (5%). There were no serious adverse events, though 28 patients (63.64%) had mild adverse events. Three adverse drug reactions occurred in 2 patients (eczema, anemia, and increased eosinophil count), all of which were transient. Conclusion: IVIG-SN treatment in patients with KD was safe and effective.

A Patient with Common Variable Immunodeficiency Followed by Severe Aplastic Anemia Successfully Treated with Allogenic Stem Cell Transplantation

Kyu Ree Park,Junshik Hong,Min Young Baek,Kyung Hee Kim,Jeong-Yeal Ahn,Jae Hoon Lee 순천향대학교 순천향의학연구소 2015 Journal of Soonchunhyang Medical Science Vol.21 No.2

A twenty-year-old male was diagnosed with common variable immunodecifiency (CVID) according to decreased blood level of immunoglobulins along with a history of recurrent sinopulmonary infection since early childhood, no response to prior vaccination, and the absence of all other defined immunodeficiency. Eleven months after the initiation of intravenous immunoglobulin replacement, he presented with petechiae on both lower legs for three weeks. Bone marrow exam was conducted as pancytopenia was not resolved over a month and severe aplastic anemia was diagnosed. Allogenic stem cell transplantation (Allo-SCT) with reduced intensity conditioning (RIC) enabled both a clinical resolution from propensity to infection with an appropriate production of immunoglobulins and a successful rescue of hematopoiesis. This report suggests that Allo-SCT using RIC is a potentially curable option in patients with CVID complicated by SAA if adequate efforts to minimize Allo-SCT-related complications are accompanied.

대나무 분말의 항산화력과 돼지의 면역 활성에 미치는 영향

송윤오 ( Yun O Song ),추교문 ( Gyo Moon Chu ),장선희 ( Sun Hee Jang ),구애진 ( Ae Jin Goo ),고응규 ( Yeoung Gyu Ko ),하지희 ( Ji Hee Ha ),이재형 ( Jae Young Lee ),강석남 ( Suk Nam Kang ),송영민 ( Young Min Song ),조재현 ( Jae Hyeo 한국동물위생학회 2014 韓國家畜衛生學會誌 Vol.37 No.2

본 연구는 대나무 분말의 항산화 활성이 비육돈에서 면역조절 효과에 미치는 영향을 구명하기 위해 실시하였으며, 이를 위해 대나무 분말 추출물의 항산화능을 검정하기 위하여, 총페놀 함량, 플라보노이드함량 및 DHHP, ABST, hydroxyl radical 등의 소거활성능을 알아보았다. 대나무 추출물의 항산화능은 높게 나타났으며, DPPH와 ABTS, HRSA 라디칼 소거능은 12∼21%였으며, 추출물의 유효성분으로 총페놀 함량은 171 mg/g이었으며, 플라보노이드 함량은 127.mg/g으로 나타났다. 다음으로 대나무 분말이 함유된 배합사료 급여시 돼지의 면역력에 미치는 영향을 알아보았다. 처리구에는 일반사료를 급여하였고, 시험구는 일반사료에 대나무분말을 1.0%, 2.0% 및 3.0%을 첨가하여 급여하였다. 대나무분말 첨가수준에 따라 혈액의 혈구계수 중 백혈구, 적혈구, 헤마토크리트 함량에서 유의적인 수준의 차이를 나타내지 않았다. 하지만 헤모글로빈 함량이 무처리 대조구에 비교하여 증가하였으며 특히 2∼3%의 처리구에서 헤모글로빈 함량은 높게 나타났다. 혈액 내 총단백질, 혈중알부민의 농도를 분석한 결과 처리구와 무처리구간에 유의적인 차이를 나타내지 않았다. AST, ALT 함량은 대나무 분말 처리군에서 무처리군보다 약간 높게 나타나지만 정상적인 범위내에서의 변화를 보였다. immunoglobulin에서 IgA와 IgG 농도는 무처리구에 비교하여 대나무 분말 처리구에서 높게 나타나고, IgA는 처리구 간의 차이가 나타나지 않았지만 IgG 농도에서는 대나무 추출물 2%, 3% 처리구가 1% 처리구에 비해 높은 수준을 나타냈다. TNF-α의 경우 무첨가구와 첨가구 사이의 유의적인 차이가 없었지만, 대나무 분말 첨가구에서 interferon-γ의 함량은 모든 첨가구에서 무처리 군보다 유의적으로 높게 나타났다. 특히 2%, 3% 처리구가 1%의 처리구 보다도 높은 interferon-γ의 함량을 나타내었다. 이는 대나무의 페놀, 또는 플라보노이드 성분에 기초한 높은 항산화력에 의하거나, 또는 대나무에 포함된 돼지 초기 면역반응을 활성화시킬 수 있는 물질에 의한 면역활성 증강에 따른 것으로 사료된다. 결론적으로 대나무 분말의 높은 항산화력은 돼지 면역력 향상에 기여하였으며, 특히 2∼3% 대나무 분말 첨가 사료는 돼지 생리활성을 크게 증강시키는 것으로 사료된다. The present study was designed to explore the antioxidant effect of Bamboo powder and its immunoreactivity in pigs. We investigated the functional properties of Bamboo extracts by means of measuring the contents of total polyphenols and flavonoid as well as determining ABST, DPPH radical scavenging activity, and hydroxyl radical scavenging activity and anticancer activity. The total phenolic compound and flavonoids contents of Bamboo extracts were 171.25 mg/g and 127.5 mg/g, respectively. The DPPH radical, hydroxyl radical, ABST radical scavenging activity of Bamboo extracts were 17.3%, 12.5% and 21.5%, respectively. Evidenced by MTT and cell cycle assay, Bamboo dose-dependently inhibited the cell proliferation and induced G0/G1-phase arrest in CHO cells at concentrations of 100, 250, and 500 μg/ml Bamboo extracts. More than 80% of apoptotic cells were observed by staining with annexin V in 500 μg/ml Bamboo-treated CHO cells, indicating that Bamboo had potent anticancer activities. Next, to investigate the effect of Bamboo on cytokine, immunoglobulin concentration, and blood compositions, flatting pigs were fed with Bamboo powder for 38 days. Flatting pigs were divided into 4 groups; basal diet (control), basal diet supplemented with 1% Bamboo powder (T1), 2% Bamboo powder (T2), and 3% Bamboo powder (T3). The level of hemoglobin increased in the all Bamboo-fed groups compared with the normal control group. In particular, platelet levels in the all Bamboo-treated groups increased by approximately 90% compared with the levels from pig on a normal control. Serum levels of immunoglobulins (IgG, IgA) in the pigs fed Bamboo powder were modestly increased, and the interferon-γ level also was strongly increased in 2% or 3% Bamboo-fed groups compared with the levels in control groups. Together, these results demonstrated that Bamboo extracts had an effective capacity of scavenging for ABTS, DPPH, and hydroxyl radicals and showed correlation with potent phenol and flavonoid contents, thus suggesting its antioxidant potential. Moreover, administration of Bamboo in 2∼3% improved blood parameters and platelets, and especially immunity-related ones such as IgG, IgA, and interferon- γ, leading to be potential feed additives in flatting pigs.

Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome

김지수,김민재,배이영,정대철 대한소아청소년과학회 2014 Clinical and Experimental Pediatrics (CEP) Vol.57 No.4

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is causedby the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effectof lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids,immunosuppressive agents, and immunoglobulins for managing the disease and controllinghemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia andcontrolling the bleeding. However, some patients suffer from severe, life-threatening hemorrhages,when factor II levels remain very low in spite of treatment with steroids. Here, we report a case ofLAHPS in a 15-year-old girl who experienced pulmonary hemorrhage with rapid progression. She wasreferred to our hospital owing to easy bruising and prolonged bleeding. She was diagnosed with LAHPSthat presented with pancytopenia, positive antinuclear antibody, proloned prothrombin time, activatedpartial thromboplastin time, positive LAC antibody, and factor II deficiency. Her treatment includedmassive blood transfusion, high-dose methylprednisolone, vitamin K, and immunoglobulin. However,she died due to uncontrolled pulmonary hemorrhage.

Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome

Kim, Ji Soo,Kim, Min Jae,Bae, E. Young,Jeong, Dae Chul The Korean Pediatric Society 2014 Clinical and Experimental Pediatrics (CEP) Vol.57 No.4

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and controlling the bleeding. However, some patients suffer from severe, life-threatening hemorrhages, when factor II levels remain very low in spite of treatment with steroids. Here, we report a case of LAHPS in a 15-year-old girl who experienced pulmonary hemorrhage with rapid progression. She was referred to our hospital owing to easy bruising and prolonged bleeding. She was diagnosed with LAHPS that presented with pancytopenia, positive antinuclear antibody, proloned prothrombin time, activated partial thromboplastin time, positive LAC antibody, and factor II deficiency. Her treatment included massive blood transfusion, high-dose methylprednisolone, vitamin K, and immunoglobulin. However, she died due to uncontrolled pulmonary hemorrhage.

Assessment of pathogen contamination and immune quality of colostrums collected from local colostrum banks in Korea

김원일,박상열,김상진,조용일,허태영,김남수 한국임상수의학회 2013 한국임상수의학회지 Vol.30 No.4

Because colostrum is considered to be the sole source of passively acquired maternal antibodies for calves,newborn calves must consume colostrum to gain disease resistance during their early years of life. Storage of surplus colostrum from dairy cows right after calving and feeding newborn calves in deficiency of colostrum to assure adequate uptake of IgG for protection of the calf has been a common practice in the bovine production. In the current study,35 colostrums were randomly collected from 3 colostrum banks located in different regions of Korea and monitored for general bacterial contamination and major bovine pathogens. Immunoglobulin concentrations and BVDV-specific antibodies were also determined to evaluate the immune quality of the colostrums. Moderate to severe bacterial contamination (up to 72,000,000 CFU/ml) was observed in most of the colostrums collected from colostrum banks. General immune quality of the colostrums was under the satisfactory level since most of the colostrums contained less than 50 g/L of IgG, which is the minimum concentration for good quality colostrums. Therefore, colostrum for colostrum bank should be collected at the first 2-3 post-partum milkings according to proper harvesting and handling procedures to guarantee the safety and quality of colostrum. In addition, it was recommended that colostrum should be heat-treated before frozen and stored in the bank because pasteurization at 63oC for 30 min was very effective reducing the risk of disease transmission without causing significant degradation of immunoglobulins.

Macrophage Activation Syndrome Presented in a Case of Neonatal Lupus

Kang, Chang Min,Choi, Jinwha,Lee, JungHwa The Korean Society of Neonatology 2021 Neonatal medicine Vol.28 No.3

Macrophage activation syndrome (MAS) is a potentially life-threatening complication in many autoimmune diseases. Early recognition and intervention are essential for a favorable outcome. Neonatal lupus, an acquired autoimmune disease in neonates caused by the transplacental passage of maternal autoantibodies, is rare and usually self-limited. Herein, we report a case of MAS in a patient with neonatal lupus, which improved with intravenous immunoglobulin.