• 제목/요약/키워드: Immunoglobulin G4-Related Disease

검색결과 39건 처리시간 0.026초

Rapidly resolved IgG4-related retroperitoneal fibrosis after steroid pulse therapy

  • Jeung, Soomin;Kim, Hyosang;Seo, Yuri;Yoon, Hee-Young;Lee, Nah Kyum;Park, Shinhee;Seo, Bomi;Park, Su-Yeon;Park, Su-Kil
    • Journal of Yeungnam Medical Science
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    • 제33권1호
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    • pp.40-43
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    • 2016
  • Retroperitoneal fibrosis (RF) is a disorder characterized by the presence of a retroperitoneal mass and concurrent systemic inflammation. Some cases of RF are recognized as belonging to the spectrum of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoids are highly effective for treatment of retroperitoneal fibrosis, although the optimal dose and duration of therapy have not been established. An initial dose of prednisone (40-60 mg) daily is usually administered with a tapering scheme. We report on a 55-year-old man diagnosed with IgG4-related RF and successfully treated with a 3-day course of daily 250 mg (4 mg/kg) intravenous methylprednisolone, which resulted in the prompt resolution of urinary obstruction and systemic symptoms.

Clinical and Radiological Features of Diffuse Lacrimal Gland Enlargement: Comparisons among Various Etiologies in 91 Biopsy-Confirmed Patients

  • Sae Rom Chung;Gye Jung Kim;Young Jun Choi;Kyung-Ja Cho;Chong Hyun Suh;Soo Chin Kim;Jung Hwan Baek;Jeong Hyun Lee;Min Kyu Yang;Ho-Seok Sa
    • Korean Journal of Radiology
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    • 제23권10호
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    • pp.976-985
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    • 2022
  • Objective: To compare the clinical and radiological features of various etiologies of chronic diffuse lacrimal gland enlargement. Materials and Methods: We retrospectively reviewed 91 consecutive patients who underwent surgical biopsy for chronic diffuse lacrimal gland enlargement and were diagnosed with non-specific dacryoadenitis (DA) (n = 42), immunoglobulin G4-related dacryoadenitis (IgG4-RD) (n = 33), and lymphoma (n = 16). Data on patient demographics, clinical presentation, and CT imaging findings (n = 73) and MRI (n = 43) were collected. The following radiologic features of lacrimal gland enlargement were evaluated: size, unilaterality, wedge sign, angle with the orbital wall, heterogeneity, signal intensity, degree of enhancement, patterns of dynamic contrast-enhanced, and apparent diffusion coefficient value. Radiological features outside the lacrimal glands, such as extra-lacrimal orbital involvement and extra-orbital head and neck involvement, were also evaluated. The clinical and radiological findings were compared among the three diseases. Results: Compared to the DA and IgG4-RD groups, the lymphoma group was significantly older (mean 59.9 vs. 46.0 and 49.4 years, respectively; p = 0.001) and had a higher frequency of unilateral involvement (62.5% vs. 31.0% and 15.2%, respectively; p = 0.004). Compared to the IgG4-RD and lymphoma groups, the DA group had significantly smaller lacrimal glands (2.3 vs. 2.8 and 3.3 cm, respectively; p < 0.001) and a lower proportion of cases with a wedge sign (54.8% vs. 84.8% and 87.5%, respectively; p = 0.005). The IgG4-RD group showed more frequent involvement of the extra-orbital head and neck structures, including the infraorbital nerve (36.4%), paranasal sinus (72.7%), and salivary gland (58.6%) compared to the DA and lymphoma groups (4.8%-28.6%) (all p < 0.005). Conclusion: Patient age, unilaterality, lacrimal gland size, wedge sign, and extra-orbital head and neck involvement differed significantly different between lymphoma, DA, and IgG4-RD. Our results will be useful for the differential diagnosis and proper management of chronic lacrimal gland enlargement.

Development and Characterization of an Anti-Acne Gel Containing Siamese Crocodile (Crocodylus siamensis) Leukocyte Extract

  • Phupiewkham, Weeraya;Lu, Qiumin;Payoungkiattikun, Wisarut;Temsiripong, Threeranan;Jangpromma, Nisachon;Lai, Ren;Klaynongsruang, Sompong
    • Journal of Microbiology and Biotechnology
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    • 제28권5호
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    • pp.707-717
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    • 2018
  • Leukocytes are reportedly the first line of the innate immune defense and essential for the control of common bacterial infections. Therefore, in this work, the antibacterial activity of crocodile leukocyte extract against Propionibacterium acnes was evaluated, and we also characterized the related activity of skin infection. The leukocyte extract showed the minimum inhibitory concentration to be $100{\mu}g/ml$ to P. acnes. SEM imaging demonstrated that the leukocyte extract adversely affected P. acnes cell permeability in a concentration-dependent manner. Furthermore, the crocodile leukocyte extract could significantly reduce proinflammatory markers and decrease inflammatory signs in infected mouse ears. The crude leukocyte extract was further purified using FPLC and RP-HPLC. The resulting fraction F5 was indicated as the anti-acne peptide-containing fraction. The molecular mass of the peptide contained in F5 was calculated to be 4,790.5 Da. N-Terminal sequencing revealed the amino acid sequence as GPEPVPAIYQ, which displays similarities to immunoglobulin A and leucine-rich repeat neuronal protein. This is the first reported amino acid sequence of a crocodile leukocyte extract that possesses anti-acne activity. To attempt to use it in a prototype cosmetic, an anti-acne gel containing crude crocodile leukocyte extract was formulated, resulting in seven gel formulations (G1, G2, G3, G4, G5, G6, and G7). The formulations G5, G6, and G7 exhibited 2-fold higher anti-acne activity than G1-G4. Investigation of accelerating stability studies of anti-acne gel formulations G5, G6, and G7 demonstrated that a low storage temperature ($4^{\circ}C$) is suitable for maintaining the physical properties and biological activity of the anti-acne gel products.

Deoxynivalenol- and zearalenone-contaminated feeds alter gene expression profiles in the livers of piglets

  • Reddy, Kondreddy Eswar;Jeong, Jin young;Lee, Yookyung;Lee, Hyun-Jeong;Kim, Min Seok;Kim, Dong-Wook;Jung, Hyun Jung;Choe, Changyong;Oh, Young Kyoon;Lee, Sung Dae
    • Asian-Australasian Journal of Animal Sciences
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    • 제31권4호
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    • pp.595-606
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    • 2018
  • Objective: The Fusarium mycotoxins of deoxynivalenol (DON) and zerolenone (ZEN) cause health hazards for both humans and farm animals. Therefore, the main intention of this study was to reveal DON and ZEN effects on the mRNA expression of pro-inflammatory cytokines and other immune related genes in the liver of piglets. Methods: In the present study, 15 six-week-old piglets were randomly assigned to the following three different dietary treatments for 4 weeks: control diet, diet containing 8 mg DON/kg feed, and diet containing 0.8 mg ZEN/kg feed. After 4 weeks, liver samples were collected and sequenced using RNA-Seq to investigate the effects of the mycotoxins on genes and gene networks associated with the immune systems of the piglets. Results: Our analysis identified a total of 249 differentially expressed genes (DEGs), which included 99 upregulated and 150 downregulated genes in both the DON and ZEN dietary treatment groups. After biological pathway analysis, the DEGs were determined to be significantly enriched in gene ontology terms associated with many biological pathways, including immune response and cellular and metabolic processes. Consistent with inflammatory stimulation due to the mycotoxin-contaminated diet, the following Kyoto encyclopedia of genes and genomes pathways, which were related to disease and immune responses, were found to be enriched in the DEGs: allograft rejection pathway, cell adhesion molecules, graft-versus-host disease, autoimmune thyroid disease (AITD), type I diabetes mellitus, human T-cell leukemia lymphoma virus infection, and viral carcinogenesis. Genome-wide expression analysis revealed that DON and ZEN treatments downregulated the expression of the majority of the DEGs that were associated with inflammatory cytokines (interleukin 10 receptor, beta, chemokine [C-X-C motif] ligand 9), proliferation (insulin-like growth factor 1, major facilitator superfamily domain containing 2A, insulin-like growth factor binding protein 2, lipase G, and salt inducible kinase 1), and other immune response networks (paired immunoglobulin-like type 2 receptor beta, Src-like-adaptor-1 [SLA1], SLA3, SLA5, SLA7, claudin 4, nicotinamide N-methyltransferase, thyrotropin-releasing hormone degrading enzyme, ubiquitin D, histone $H_2B$ type 1, and serum amyloid A). Conclusion: In summary, our results demonstrated that high concentrations DON and ZEN disrupt immune-related processes in the liver.

Association between periodontal bacteria and degenerative aortic stenosis: a pilot study

  • Kataoka, Akihisa;Katagiri, Sayaka;Kawashima, Hideyuki;Nagura, Fukuko;Nara, Yugo;Hioki, Hirofumi;Nakashima, Makoto;Sasaki, Naoki;Hatasa, Masahiro;Maekawa, Shogo;Ohsugi, Yujin;Shiba, Takahiko;Watanabe, Yusuke;Shimokawa, Tomoki;Iwata, Takanori;Kozuma, Ken
    • Journal of Periodontal and Implant Science
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    • 제51권4호
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    • pp.226-238
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    • 2021
  • Purpose: Although several reports have described the relationship between periodontal disease and cardiovascular disease, information about the association between periodontal disease and the progression of degenerative aortic stenosis (AS) is lacking. Therefore, we performed a retrospective, single-center, pilot study to provide insight into this potential association. Methods: Data from 45 consecutive patients (19 men; median age, 83 years) with mild or moderate degenerative aortic stenosis were analyzed for a mean observation period of 3.3±1.9 years. The total amount of Aggregatibacter actinomycetemcomitans and Porphyromonas gingivalis and titers of serum immunoglobulin G (IgG) against periodontal bacteria and high-sensitivity C-reactive protein (hs-CRP) were evaluated. Aortic valve area (AVA), maximal velocity (Vmax), mean pressure gradient (mean PG), and the Doppler velocity index (DVI) were evaluated. The change in each parameter per year ([ParameterLATEST-ParameterBASELINE]/Follow-up Years) was calculated from the retrospective follow-up echocardiographic data (baseline vs. the most recently collected data [latest]). Results: No correlation was found between the concentration of periodontopathic bacteria in the saliva and AS status/progression. The anti-P. gingivalis antibody titer in the serum showed a significant positive correlation with AVA and DVI. Additionally, there was a negative correlation between the anti-P. gingivalis IgG antibody titer and mean PG. The hs-CRP concentration showed positive correlations with Vmax and mean PG. Meanwhile, a negative correlation was observed between the anti-P. gingivalis IgG antibody titer and ΔAVA/year and Δmean PG/year. The hs-CRP concentration showed positive correlations with Vmax and mean PG, and it was significantly higher in patients with rapid aortic stenosis progression (ΔAVA/year <-0.1) than in their counterparts. Conclusions: Our results suggest that periodontopathic bacteria such as A. actinomycetemcomitans and P. gingivalis are not directly related to the status/progression of degenerative AS. However, inflammation and a lower immune response may be associated with disease progression.

Selection of Vaccinia Virus-Neutralizing Antibody from a Phage-Display Human-Antibody Library

  • Shin, Yong Won;Chang, Ki-Hwan;Hong, Gwang-Won;Yeo, Sang-Gu;Jee, Youngmee;Kim, Jong-Hyun;Oh, Myoung-don;Cho, Dong-Hyung;Kim, Se-Ho
    • Journal of Microbiology and Biotechnology
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    • 제29권4호
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    • pp.651-657
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    • 2019
  • Although smallpox was eradicated in 1980, it is still considered a potential agent of biowarfare and bioterrorism. Smallpox has the potential for high mortality rates along with a major public health impact, eventually causing public panic and social disruption. Passive administration of neutralizing monoclonal antibodies (mAbs) is an effective intervention for various adverse reactions caused by vaccination and the unpredictable nature of emerging and bioterrorist-related infections. Currently, vaccinia immune globulin (VIG) is manufactured from vaccinia vaccine-boosted plasma; however, this production method is not ideal because of its limited availability, low specific activity, and risk of contamination with blood-borne infectious agents. To overcome the limitations of VIG production from human plasma, we isolated two human single-chain variable fragments (scFvs), (SC34 and SC212), bound to vaccinia virus (VACV), from a scFv phage library constructed from the B cells of VACV vaccine-boosted volunteers. The scFvs were converted to human IgG1 (VC34 and VC212). These two anti-VACV mAbs were produced in Chinese Hamster Ovary (CHO) DG44 cells. The binding affinities of VC34 and VC212 were estimated by competition ELISA to $IC_{50}$ values of $2{\mu}g/ml$ (13.33 nM) and $22{\mu}g/ml$ (146.67 nM), respectively. Only the VC212 mAb was proven to neutralize the VACV, as evidenced by the plaque reduction neutralization test (PRNT) result with a $PRNT_{50}$ of ~0.16 mg/ml (${\sim}1.07{\mu}M$). This VC212 could serve as a valuable starting material for further development of VACV-neutralizing human immunoglobulin for a prophylactic measure against post-vaccination complications and for post-exposure treatment against smallpox.

단일 기관에서 진단받은 가와사끼병 환아 1,000례의 통계학적 분석 (Statistical Analysis of 1,000 Cases of Kawasaki Disease Patients Diagnosed at a Single Institute)

  • 황대환;신경미;최경민;최재영;설준희;김동수
    • Clinical and Experimental Pediatrics
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    • 제48권4호
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    • pp.416-424
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    • 2005
  • 목 적 : 가와사끼병 환아의 특성과 관상동맥 이상, IVIG에 대한 비반응 및 병의 재발 등에 대해 관련된 인자를 찾아보고자 하였다. 방 법 : 1990년 9월부터 2003년 12월까지 본원에 내원, 가와사끼 병으로 진단받고 IVIG을 투여한 1,000명을 대상으로 후향적으로 분석하였다. 내원시 관상동맥 확장군과 관상동맥 정상군을 비교하였고 IVIG 1회 투여에 대한 반응군과 비반응군 및 재발군과 비재발군의 비교도 시행하여 두 군간에 의미있는 요인을 찾아보려고 하였으며 재발된 환아를 대상으로 처음 발병시와 두번째 발병시의 각종 자료를 분석하였고 두 군간에 관상동맥 이상이 생기는 정도도 비교해 보았다. 또한 첫 IVIG을 투여하기 전까지의 발열기간과 상관비례하는 인자를 알아보려 하였다. 결 과 : 1) 관상동맥 확장은 16%에서 관찰되었으며 중등도 이상(${\geq}5mm$)의 관상동맥 확장 및 중증(${\geq}8mm$) 관상동맥 확장의 빈도는 각각 2%와 0.5%로 나타났다. 또한 입원당시 관상동맥 이상의 빈도는 1년간 추적관찰 했을 때와도 비슷한 수준을 보였다. 2) 첫 IVIG을 투여하기 전까지의 발열기간이 길수록(7일 이상), 첫 IVIG 투여 종료 후부터 최종적으로 열이 소실될 때까지의 기간이 길수록, 남아일수록, 내원 당시 검사상 Hgb(10 g/dL이하), Hct(30% 이하)수치가 낮을수록, WBC 수치($12,000/mm^3$ 이상)및 과립구 분율이 높을수록, CRP 값이 높을수록(10 mg/dL 이상), Harada's score가 높을수록(4점 이상) 관상동맥 이상군과 관련이 있었다. 3) IVIG 한번 투여에 반응이 없었던 경우는 8%였으며 내원당시 검사수치상 WBC 수치가 높을수록($12,000/mm^3$ 이상), 과립구 분율이 높을수록, CRP 수치가 높을수록(10 mg/dL 이상), SGPT가 높을수록, Harada's score가 높을수록(4점 이상), 농뇨를 보일수록 1회의 IVIG 투여에 반응이 없었다. IVIG을 두 번이상 투여한 군에서는 한번 투여에 반응이 있었던 군보다 향후 재발율이 더 높았다. 또한 중증도 이상의 관상동맥 확장의 경우는 두 번 이상 투여하게 된 군에서 의미있게 높았다. 4) 재발은 6%에서 나타났고 재발을 예측할 수 있는 지표는 없었으며 재발한 환아들에서 관상동맥 이상의 빈도 또한 별 차이가 없었다. 5) 재발된 환아들의 처음 발병시와 두 번째 발병시의 비교에서, 두 번째 발병시에 WBC 수치와 CRP 값은 첫 번째 발병시 보다 낮았고 첫 IVIG 투여 전까지의 발열기간도 낮았으나 두 군간에 관상동맥 이상이 생기는 정도에는 차이가 없었다. 6) 첫 IVIG을 투여하기 전까지의 발열기간과의 상관 인자 조사에서는, WBC 수치와 CRP 값, Harada's score의 경우 양의 상관관계를 보였고 Hgb의 경우 음의 상관관계를 보였다. 결 론 : 내원 당시 높은 WBC 수치와 CRP 값 및 Harada'score는 관상동맥 병변 발생 및 IVIG 투여에 대한 비반응과 관련이 있으며 또한 첫 IVIG을 투여하기 전까지의 발열기간과도 상관관계가 있었다. 따라서 되도록이면 조기에 발견하여 IVIG을 투여하는 것이 비록 비반응률을 낮추지는 못하더라도 관상동맥 병변 발생의 위험률은 낮출 수 있을 것으로 생각된다. 또한 IVIG 비반응군에서는 차후 재발률이 더 높았으며 중등도 이상의 관상동맥 확장이 더 많이 발견되었으므로 이들을 대상으로 한 집중적인 치료와 관리가 도입될 필요가 있다. 재발을 예측할 수 있는 기타 지표는 찾아볼 수 없었으며 따라서 보호자의 교육을 통한 조기 발견 및 치료에 지속적으로 노력을 기울여야 할 것이고 재발한 환아들에서 관상동맥 이상의 빈도가 증가하지 않은 점은 현재 이루어지고 있는 이러한 노력의 성과로 여겨진다.

가와사끼병에서 C-반응 단백질과 적혈구 침강속도의 불일치 및 정맥내 면역글로불린 치료 후의 변화 (C-reactive Protein and Erythrocyte Sedimentation Rate Discrepancies and Variations after Intravenous Immunoglobulin Therapy in Kawasaki Disease)

  • 이윤숙;이지현;홍영미;손세정
    • Pediatric Infection and Vaccine
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    • 제23권1호
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    • pp.25-30
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    • 2016
  • 목적: 가와사끼병에서 C-반응 단백질(CRP)과 적혈구 침강속도(ESR)의 불일치 및 정맥내 면역글로불린(IVIG) 치료 후의 변화를 보고자 하였다. 방법: 총 123명의 가와사끼병 환자의 의무기록를 후향적으로 분석하였다. 염증성 지표들을 IVIG 치료 전과 치료 후 48-72시간에 검사하였다. 불일치는 $CRP{\geq}10mg/dL$와 ESR <50 mm/hr, 또는 CRP <10 mg/dL와 $ESR{\geq}50mm/hr$로 정의하였고 전자를 1 군으로, 후자를 2군으로 구분하였다. 결과: 전체 123명 중 36명(29.2%)에서 불일치를 보였는데 1군은 25명(20.3%)이었고, 2군은 11명(8.9%)이었다. 1군에서 15명은 발열기간이 5일 미만(초기 발현자)이었고, 10명은 5일 이상(후기 발현자)이었다. 2군에서는 6명이 초기 발현자, 5명이 후기 발현자였다. ESR 값은 초기 발현자보다 후기 발현자에서 더 높았다($26.3{\pm}19.3mm/hr$ vs. $34.3{\pm}21.0mm/hr$, P=0.029). IVIG 치료 후에는 증가된 CRP를 포함한 여러 염증성 지표들은 정상으로 돌아왔으나 ESR은 오히려 $37.4{\pm}21.9mm/hr$에서 $48.0{\pm}22.7mm/hr$로 증가하였다(n=36, P=0.051). 결론: CRP와 ESR 값의 불일치는 발열기간과 연관이 있으리라 추정된다. 이 두 중요 염증성 지표가 불일치할 수 있으므로 급성 가와사끼병의 치료 전 질병의 염증 활성도를 평가하기 위해서는 이 두 지표를 동시에 검사하는 것이 필요하며, IVIG 치료 후의 ESR은 치료 반응의 지표로 적합하지 않다.

중증 지역사회획득 폐렴환자의 예후에 영향을 미치는 면역지표에 대한 연구 (Prospective Study of the Immunologic Factors Affecting the Prognosis of Severe Community-Acquired Pneumonia)

  • 황재경;이호명;송광식;박계영;박정웅;박재경;정성환;안정열;서일혜;남귀현
    • Tuberculosis and Respiratory Diseases
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    • 제50권4호
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    • pp.437-449
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    • 2001
  • 연구배경 : 중증 지역사회획득 폐렴환자에서 어떤 면역학적 요인이 중요한 예후 인자로 작용하는지와 그에 대한 예방과 개선방법을 알아보기 위해 연구를 시행하였다. 방 법 : 중증 폐렴환자 23명을 임상경과에 따라 회복군(n=16)과 사망군(n=7) 으로 나눈 후, 말초혈액에서 전혈구검사, 혈청 생화학검사, 면역글로블린, 보체, 림프구 아세포군 검사, 객담 및 혈액배양검사, 소변검사, 흉부 x-선 검사등을 시행하였다. 결 과 : 1) 양 군에서 말초혈액검사상 림프구감소증 소견이 있었다(림프구 평균 회복군 $995.6{\pm}505.7/mm^3$, 사망군 $624.0{\pm}287.6/mm^3$, p=0.18). 2) 양 군 모두 T 림프구가 감소되었고, 특히 사망군의 T 림프구수가 통계학적으로 유의하게 감소된 소견을 보여주었다(회복군 $723.6{\pm}406.5/mm^3$, 사망군 $295.9{\pm}203.0/mm^3$, p<0.05). 3) 혈청 생화학 검사상 단백질, 알부민이 정상치에 비해 감소되었으며(protein/ albumin 회복군 $6.0{\pm}1.0/2.7{\pm}0.7$, 사망군 $5.2{\pm}1.5/2.3{\pm}0.8$ 단위 : $g/d{\ell}$), 혈중 요소질소 수치는 회복군 $18.9{\pm}9.8mg/d{\ell}$, 사망군 $41.7{\pm}30.0mg/d{\ell}$으로 사망군에서 통계학적으로 의미있게 증가되었고(p<0.05), 혈청 크레아티닌은 회복군 $1.0{\pm}0.3mg/d{\ell}$, 사망군 $1.8{\pm}1.0mg/d{\ell}$으로, 사망군이 역시 의미있게 증가되어 있음을 보여주었다(p<0.05). 4) 면역글로블린 G는 회복군보다 사망군에서 유의하게 감소되어 있었다(IgG 회복군 $1433.0{\pm}729.5mg/d{\ell}$, 사망군 $849.1{\pm}373.1mg/d{\ell}$, p<0.05). 5) 보체 $C_3$, $C_4$의 수치가 모두 정상보다 떨어져 있었으며, 회복군에 비해 사망군에서 감소를 보였으나 통계학적 의의는 없었다. 6) Cytokine 연구 결과는 TNF-$\alpha$, IFN-$\gamma$, IL-2, IL-10을 측정하였으나 두 군간에 유의한 차이는 없었다. 7) 만성 간질환, 당뇨, 만성 폐쇄성 폐절환이 양 군에서 주요 기저질환이였다. 결 론 : 이상의 결과는 중증 지역사회획득 폐렴환자에서 T 림프구수치 특히, $T_8$ 림프구의 감소, $T_4/T_8$ 비율의 증가, 면역글로블린 G 감소, 혈중 요소질소 수치 증가, 혈청 크레아티닌이 증가된 소견은 나쁜 예후인자와 관련되는 것으로 추정된다.

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