• Title/Summary/Keyword: Immunoglobulin G4-related disease

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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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    • v.33 no.1
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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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    • v.23 no.10
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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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    • v.28 no.5
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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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    • v.31 no.4
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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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    • v.51 no.4
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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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    • v.29 no.4
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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.

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

  • Hwang, Dae Hwan;Sin, Kyoung Mi;Choi, Kyong Min;Choi, Jae Young;Sul, Jun Hee;Kim, Dong Soo
    • Clinical and Experimental Pediatrics
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    • v.48 no.4
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    • pp.416-424
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    • 2005
  • Purpose : To find the risk factors associated with coronory artery lesions, non-responsiveness to intravenous immunoglobulin(IVIG) treatment, and recurrences in Kawasaki disease patients. Methods : We retrospectively analyzed 1,000 Kawasaki disease patients who were admitted to Yonsei University Medical Center from September 1990 to December 2003. We compared between responder and non-responder groups to IVIG treatment as well as between relapsed and non-relapsed groups, and as to the relapsed group, we also compared variables between patients in their first and second attack states. Finally, factors associated with longer-fever duration from disease onset were evaluated. Results : Longer fever durations before and after IVIG treatment, male sex, lower Hgb and Hct level, higher WBC count and segmented WBC proportion, and higher CRP and Harada's score were related with coronary artery lesions. Non-responsiveness was related to higher WBC count, segmented WBC proportion, CRP, SGPT, Harada's score, and pyuria. Moderate-to-severe coronary artery dilatations and recurrences were more commonly seen among the non-responder group. No significant predictive factors for recurrence were found. In the relapsed group, lower WBC count, CRP, and shorter fever duration from disease onset were observed in their second attack state. Fever duration from disease onset showed positive correlation with WBC count, CRP, and Harada's score and negative correlation with Hgb levels. Conclusion : Higher WBC count, CRP, and higher Harada's score were related to both higher incidences of coronary artery lesions and non-responsiveness to IVIG treatment, and these factors were also related with longer fever duration. Non-responders to IVIG treatment showed higher recurrence rate and more moderate-to-severe coronary artery dilatations than responders.

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

  • Lee, Yoon Suk;Lee, Jihyen;Hong, Young Mi;Sohn, Sejung
    • Pediatric Infection and Vaccine
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    • v.23 no.1
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    • pp.25-30
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    • 2016
  • Purpose: We undertook this study to investigate discrepancies in C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) values, and variations following intravenous immunoglobulin (IVIG) therapy in Kawasaki disease (KD). Methods: A total of 123 KD patients were retrospectively enrolled. Patients were treated with IVIG 2 g/kg at 2 to 9 days after disease onset. We obtained white blood cell (WBC) count, percentage of neutrophils (% neutrophils), CRP, ESR, and N-terminal pro-brain natriuretic peptide (NT-proBNP) values before and 48 to 72 hours after IVIG treatment. Discrepancy was defined as $CRP{\geq}10mg/dL$ and ESR <50 mm/hr (Group 1), or CRP <10 mg/dL and $ESR{\geq}50mm/hr$ (Group 2). Results: Thirty-six of 123 subjects (29.2%) had a discrepancy: 25 (20.3%) in Group 1 and 11 (8.9%) in Group 2. In Group 1, 15 patients (60%) had fever for <5 days (early presenter) and 10 (40%) had fever for ${\geq}5days$ (late presenter). There were six early presenters (55%) and five late presenters (45%) in Group 2. Late presenters had higher ESR than early presenters ($34.3{\pm}21.0mm/hr$ vs. $26.3{\pm}19.3mm/hr$, P=0.029). After IVIG treatment, elevated WBC count, % neutrophils, CRP, and NT-proBNP levels normalized. In contrast, ESR increased from $37.4{\pm}21.9mm/hr$ to $48.0{\pm}22.7mm/hr$ (n=36, P=0.051). Conclusions: A discrepancy may be related to the duration of fever. Due to discrepancies in CRP and ESR values in acute KD, both should be measured to assess the degree of inflammatory activity before IVIG treatment. After IVIG treatment, the ESR should not be used as a marker of response to therapy in KD.

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

  • Hwang, Jae-Kyung;Lee, Ho-Moeng;Song, Kwang-Sik;Park, Gye-Young;Park, Jeong-Woong;Park, Jae-Kyung;Jeong, Seong-Hwan;Ahn, Jeong-Yeal;Seo, Yiel-Hea;Nam, Gui-Hyun
    • Tuberculosis and Respiratory Diseases
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    • v.50 no.4
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    • pp.437-449
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    • 2001
  • Background : In the severe community-acquired pneumonia, it has been known that the immune status is occasionally suppressed. This study was performed to identify the immunologic markers related with the prognostic factors in severe community-acquired pneumonia. Methods : 23 patients with severe community-acquired pneumonia were involved in this study, and divided into survivor (16) and nonsurvivor (7) groups. In this study, the medical history, laboratory tests(complete blood counts, routine chemistry profile, immunoglobulins, complements, lymphocyte subsets, cytokines, sputum and blood culture, urine analysis), and chest radiographs were scrutinized. Results : 1) Both groups had lymphopenia(total lymphocyte count $995.6{\pm}505.7/mm^3$ in the survivor and $624.0{\pm}287.6/mm^3$ in the nonsurvivor group). 2) The T-lymphocyte count of the nonsurvivor group($295.9{\pm}203.0/mm^3$) was lower than the survivor group($723.6{\pm}406.5/mm^3$) (p<0.05). 3) The total serum protein(albumin) was $6.0{\pm}1.0(2.7{\pm}0.7)\;g/d{\ell}$ in the survivor and $5.2{\pm}1.5(2.3{\pm}0.8)g/d{\ell}$ in the nonsurvivor group. The BUN of the nonsurvivor group($41.7{\pm}30.0mg/d{\ell}$) was higher than that of the survivor group($18.9{\pm}9.8mg/d{\ell}$)(p<0.05). The creatinine concentration was higher in the nonsurvivor group($1.8{\pm}1.0mg/d{\ell}$) than that in the survivor group($1.0{\pm}0.3mg/d{\ell}$)(p<0.05). 4) The immunoglobulin G level was higher in the survivor group($1433.0{\pm}729.5mg/d{\ell}$) than in the nonsurvivor group($849.1{\pm}373.1mg/d{\ell}$) (p<0.05). 5) The complement $C_3$ level was $108.0{\pm}37.9mg/d{\ell}$ in the survivor group and $88.0{\pm}32.1mg/d{\ell}$ in the nonsurvivor group. 6) A cytokine study showed an insignificant difference in both groups. 7) Chronic liver disease, DM, and COPD were major underlying diseases in both groups. Conclusion : These results suggest that decreased a T-lymphocyte count and immunoglobulin G level, and an increased BUN and creatinine level may be associated with the poor prognosis of severe community-acquired pneumonia.

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