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Joondon Lee,Jinseok Kim,Byeongzu Ghang,정우성 대한내과학회 2023 The Korean Journal of Internal Medicine Vol.38 No.3
Background/Aims: The occurrence of gout attacks at the start of uric acid lowering treatment worsens compliance. We aimed to determine the appropriate dose of febuxostat to reduce the occurrence of gout attacks during the initial treatment period. Methods: We retrospectively analyzed the data of patients diagnosed with gout who underwent treatment at Jeju National University Hospital between May 2018 and May 2020. Results: Two-hundred and twenty-seven patients were included, with a mean age of 53.2 ± 16.4 years, and 219 (96.5%) were male. The patients were divided into two groups according to the starting dose of febuxostat (20 mg vs. 40 mg). There were no significant differences in mean age, disease duration, colchicine, estimated glomerular filtration rate (eGFR), initial uric acid levels, and presence of subcutaneous tophi between the two groups. Gout attacks occurred more frequently in the 20 mg group than in the 40 mg group during the first 3 months of treatment (32.0% vs. 14.3%, p = 0.002), particularly CKDduring the first month (21.3% vs. 7.5%, p = 0.005). Multivariate logistic regression analysis was conducted adjusting for the effects of disease duration, the presence of subcutaneous tophi, eGFR, and initial uric acid levels. A febuxostat starting dose of 40 mg (odds ratio, 0.464; 95% confidence interval [CI], 0.246 to 0.862; p = 0.015) and anti-inflammatory prophylaxis (odds ratio, 0.359; 95% CI, 0.158 to 0.813; p = 0.014) were found to be independent factors associated with a gout attack. Conclusions: Starting uric acid lowering treatment with febuxostat 40 mg rather than 20 mg may reduce the incidence of gout attacks in the early period of treatment in Korean patients with gout.
Ehlers–Danlos syndrome VIII with novel C1R variant accompanying white matter changes
Go Hun Seo,Yoon-Myung Kim,Byeongzu Ghang,Gu-Hwan Kim,Beom Hee Lee 대한의학유전학회 2019 대한의학유전학회지 Vol.16 No.1
Ehlers–Danlos syndrome (EDS) VIII is an autosomal dominant inherited connective tissue disorder characterized by intrac-table periodontal inflammation, absence of gingiva, pretibial plaques, skin hyperextensibility, joint hypermobility, and tissue fragility with onset in the childhood or adolescence. In a recent report, heterozygous variants of the C1R or C1S related to the classical complement pathway were identified in families with history of EDS VIII. The current report describes a Korean 34-year-old female carrying a novel missense variant of C1R c.925T>G (p.Cys309Gly) and exhibiting early severe periodonti-tis, skin fragility, and joint hypermobility. The patient also had frontal, parietal, and temporal white matter brain lesions without definite vascular abnormalities on brain magnetic resonance imaging, which have not been surveyed meticulously in EDS VIII. Considering the genetic alteration of classic complement pathways in this condition, it is necessary to carefully observe multisystemic inflammation processes such as changes in brain white matter.
Ehlers-Danlos syndrome VIII with novel C1R variant accompanying white matter changes
Seo, Go Hun,Kim, Yoon-Myung,Ghang, Byeongzu,Kim, Gu-Hwan,Lee, Beom Hee Korean Society of Medical Genetics and Genomics 2019 대한의학유전학회지 Vol.16 No.1
Ehlers-Danlos syndrome (EDS) VIII is an autosomal dominant inherited connective tissue disorder characterized by intractable periodontal inflammation, absence of gingiva, pretibial plaques, skin hyperextensibility, joint hypermobility, and tissue fragility with onset in the childhood or adolescence. In a recent report, heterozygous variants of the C1R or C1S related to the classical complement pathway were identified in families with history of EDS VIII. The current report describes a Korean 34-year-old female carrying a novel missense variant of C1R c.925T>G (p.Cys309Gly) and exhibiting early severe periodontitis, skin fragility, and joint hypermobility. The patient also had frontal, parietal, and temporal white matter brain lesions without definite vascular abnormalities on brain magnetic resonance imaging, which have not been surveyed meticulously in EDS VIII. Considering the genetic alteration of classic complement pathways in this condition, it is necessary to carefully observe multisystemic inflammation processes such as changes in brain white matter.
Immunoglobulin Binding Protein 1 as a Potential Urine Biomarker in Patients with Lupus Nephritis
Lee, Eun-Ju,Kwon, Oh Chan,Ghang, Byeongzu,Lim, Doo-Ho,Kim, Do Hoon,Hong, Seokchan,Lee, Chang-Keun,Yoo, Bin,Kim, Yong-Gil MDPI AG 2019 INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES Vol.20 No.10
<P>We evaluated the role of immunoglobulin binding protein 1 (IGBP1), a phosphoprotein associated with the B cell receptor (BCR) complex, as a urine biomarker in lupus nephritis (LN). The IGBP1 concentrations in plasma and urine of patients with LN, systemic lupus erythematosus (SLE) without nephritis and healthy controls were estimated by ELISA. IGBP1 expression in the kidneys of LN patients and transplantation donors was detected by immunohistochemistry. Microarray-based global gene expression profile of HK-2 cells with IGBP1 knock-down and fluorescence-activated cell sorting (FACS) for intracellular IGBP1 expression in human peripheral blood mononuclear cells (PBMCs) was performed. Urine IGBP1 levels were elevated significantly in LN patients, and it correlated with the clinical activity indices (complement 3 (C3) level, anti-dsDNA antibodies titer, SLE Disease Activity Index-2000 (SLEDAI-2K) and histological activity index. IGBP1 expression was increased in LN patients as compared to the donors and was detected mainly in the tubules by histopathology. In microarray analysis, several genes related to SLE pathogenesis (PPME1, ROCK2, VTCN1, IL-17R, NEU1, HLA-DM, and PTX3) responded to siRNA-mediated IGBP1 silencing. In FACS, IGBP1 was expressed mainly in the CD14+ cells. The overall expression of IGBP1 in PBMCs was higher in LN patients as compared with that in SLE patients without nephritis. Conclusively, urinary IGBP1 may be a novel biomarker reflecting the clinical and histological activities in LN.</P>
Ehlers–Danlos syndrome VIII with novel C1R variant accompanying white matter changes
Go Hun Seo,Yoon-Myung Kim,Byeongzu Ghang,Gu-Hwan Kim,Beom Hee Lee 대한의학유전학회 2019 대한의학유전학회지 Vol.16 No.1
Ehlers–Danlos syndrome (EDS) VIII is an autosomal dominant inherited connective tissue disorder characterized by intrac-table periodontal inḀammation, absence of gingiva, pretibial plaques, skin hyperextensibility, joint hypermobility, and tissue fragility with onset in the childhood or adolescence. In a recent report, heterozygous variants of the C1R or C1S related to the classical complement pathway were identiἀed in families with history of EDS VIII. The current report describes a Korean 34-year-old female carrying a novel missense variant of C1R c.925T>G (p.Cys309Gly) and exhibiting early severe periodonti-tis, skin fragility, and joint hypermobility. The patient also had frontal, parietal, and temporal white matter brain lesions without deἀnite vascular abnormalities on brain magnetic resonance imaging, which have not been surveyed meticulously in EDS VIII. Considering the genetic alteration of classic complement pathways in this condition, it is necessary to carefully observe multisystemic inḀammation processes such as changes in brain white matter.
( Soo Min Ahn ),( Seokchan Hong ),( Doo-ho Lim ),( Byeongzu Ghang ),( Yong-gil Kim ),( Chang-keun Lee ),( Bin Yoo ) 대한내과학회 2019 The Korean Journal of Internal Medicine Vol.34 No.2
Background/Aims: Acute transverse myelitis (ATM) is a severe complication of systemic lupus erythematosus (SLE). This study evaluated the clinical factors related to outcome in patients with SLE-associated ATM. Methods: The medical records of patients diagnosed with SLE-associated ATM between January 1995 and January 2015 were reviewed. The patients were divided into two groups based on improvement of neurological deficits after treatment: favorable response group and unfavorable response group. During follow-up, the recurrence of ATM was also analyzed. Results: ATM was identified in 16 patients with SLE. All of the patients were treated with high doses of methylprednisolone (≥ 1 mg/kg daily). Although 12 patients (75%) recovered (favorable response group), four (25%) had persistent neurologic deficits (unfavorable response group) after the treatment. Compared to the favorable response group, significantly higher Systemic Lupus Erythematosus Disease Activity Index-2000, lower complement levels and initial severe neurologic deficits were found in the unfavorable response group. Among the 12 favorable response patients, five (41.7%) experienced recurrence of ATM during the follow-up. Patients (n = 5) who experienced relapse had a shorter duration of high-dose corticosteroid treatment (13.2 days vs. 32.9 days, p = 0.01) compared to patients who did not relapse. The mean duration of tapering-off the corticosteroid until 10 mg per day was significantly longer in non-relapse group (151.3 ± 60.8 days) than in relapse group (63.6 ± 39.4 days, p = 0.013). Conclusions: Higher disease activity in SLE and initial severe neurologic deficits might be associated with the poor outcome of ATM. Corticosteroid slowly tapering-off therapy might be helpful in preventing the recurrence of ATM.
( Doo-ho Lim ),( Yong-gil Kim ),( Tae Sun Shim ),( Kyung-wook Jo ),( Byeongzu Ghang ),( Soo Min Ahn ),( Seokchan Hong ),( Chang-keun Lee ),( Bin Yoo ) 대한내과학회 2017 The Korean Journal of Internal Medicine Vol.32 No.6
Background/Aims: Nontuberculous mycobacteria (NTM) infection has been increasing worldwide in both general population and immunocompromised patients, which has also been reported in rheumatoid arthritis (RA) patients. This study aimed to identify the incidence and clinical characteristics of NTM infection in RA patients living in tuberculosis (TB) infection endemic area. Methods: We performed a retrospective analysis of NTM infection cases in our RA registry at a tertiary referral center from January 1995 to December 2013. The clinical features of them were compared to those of 52 TB infection patients from same registry. Results: Among 1,397 patients with RA, NTM infection was newly developed in 26 patients and the incidence of NTM infection was 164.8 per 100,000 patient-years. The Mycobacterium avium complex was the most frequent isolate (76.9%). None of the NTM infections had extrapulmonary involvement, which was rather common in TB infection (26.9%). Patients with NTM infection were older, received higher cumulative steroid doses, and had higher rates of past TB infection history and concomitant interstitial lung disease (ILD) than cases with TB infection. Conclusions: In South Korea, NTM infection is not rare in RA patients, and infection rates are growing. Physicians should be cautious about NTM infection in patients with a history of TB infection or concomitant ILD, even living in TB endemic area.
( Seokjung Jo ),( Eun Hye Oh ),( Jae Yong Lee ),( Yun Kyung Cho ),( Oh Chan Kwon ),( Byeongzu Ghang ),( Yong-gil Kim ) 대한류마티스학회 2017 대한류마티스학회지 Vol.24 No.2
A 36-yr-old woman with systemic lupus erythematosus was admitted in our center because of thrombocytopenia that was being treated with corticosteroids. She was prescribed a four-day course of intravenous immunoglobulin (IVIG) infusion. After three days of IVIG infusion, she developed aseptic meningitis with severe pleocytosis in the cerebrospinal fluid, followed by acute kidney injury. These complications resolved completely with conservative management. Here, we report these rare complications of IVIG and the outcome. (J Rheum Dis 2017;24:119-122)