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Bor-Luen Chiang

Publications and source records attributed to Bor-Luen Chiang.

At least 55 records · Page 3Linked to original sources
PubMed2005

Treatment with etanercept for patients with juvenile rheumatoid arthritis in Taiwan--a preliminary report.

Tumor necrosis factor (TNF) is a major inflammatory cytokine involved in the pathogenesis of juvenile rheumatoid arthritis (JRA). Etanercept, approved in the United States and in Europe for use in patients with rheumatoid arthritis (RA) and JRA, is an effective inhibitor of TNF that has been shown to provide rapid and sustained improvement in both diseases. Here we report the preliminary results of etanercept use in 3 cases of JRA with poor response to traditional therapy including non-steroidal anti-inflammatory drugs and disease-modifying antirheumatic drugs. Two of the patients had polyarticular JRA and 1 had systemic JRA. Etanercept was administered at a dosage of 0.4 mg/kg (maximum 25 mg) subcutaneously twice a week. Clinical as well as inflammatory parameter improvement was noted after use of etanercept in all cases. The preliminary results of etanercept use in these 3 cases showed significant clinical benefit without obvious adverse effects.

Antirheumatic Agents↗
PubMed2005

Clinical manifestations and inflammatory cytokine responses in patients with severe acute respiratory syndrome.

BACKGROUND AND PURPOSE: Severe acute respiratory syndrome (SARS) is a highly transmissible disease with significant morbidity and mortality. Death from SARS is most often due to rapidly progressive respiratory compromise (acute respiratory distress syndrome, ARDS) and subsequent multi-organ dysfunction. However, the mechanisms evoking respiratory distress and a fulminant systemic response remain unclear. In order to elucidate the pathogenic mechanisms of SARS, we analyzed clinical manifestations and levels of serum cytokines of SARS patients. METHODS: Fourteen hospitalized patients with a diagnosis of SARS-associated coronavirus infection at National Taiwan University Hospital from March to May 2003 were included. Data on clinical manifestations, parameters of laboratory tests, complications and final outcomes of patients were collected retrospectively. Serial plasma inflammatory cytokines, including interleukin (IL)-1beta (IL-1beta), IL-6, IL-8 and tumor necrosis factor-alpha (TNF-alpha) of preserved serum were measured by enzyme immunoassay. RESULTS: All 14 patients had fever, dry cough and dyspnea. Twelve were intubated during hospitalization. The median duration from onset of fever to the nadir level or most severe condition was 9 days for hypoxia, 7 days for lymphocytopenia, 6.5 days for thrombocytopenia, 9.5 days for maximal pulmonary infiltrates; to peak serum levels was 9 days for C-reactive protein (CRP), 10.5 days for IL-6, 13.5 days for IL-8 and 12 days for TNF-alpha; to defervescence was 13 days. There was no significant elevation of serum IL-1beta levels in any of the 14 patients. There were no significant differences in peak levels of IL-6, IL-8 and TNF-alpha between patients with and without ARDS. The 8 patients who died tended to have higher peak levels of serum TNF-alpha compared to those who survived (14 vs 9.1 pg/mL; p = 0.06). CONCLUSION: Rapid elevation of inflammatory cytokines-IL-6, IL-8 and TNF-alpha might play a role in the development of SARS-related ARDS. The timing of elevations in inflammatory cytokines and CRP is correlated with progression of pulmonary infiltrates of SARS patients. The peak level of serum TNF-alpha tends to be higher in patients who die of SARS than in those who survive. Our results indicate that CRP and TNF-alpha might be used as prognostic markers of SARS.

Adult↗
PubMed2004

Induction of IL-8 release in lung cells via activator protein-1 by recombinant baculovirus displaying severe acute respiratory syndrome-coronavirus spike proteins: identification of two functional regions.

The inflammatory response and the intracellular signaling pathway induced by severe acute respiratory syndrome (SARS)-coronavirus (CoV) were studied in lung epithelial cells and fibroblasts. SARS-CoV spike (S) protein-encoding plasmid induced activations of IL-8 promoter and AP-1, but not NF-kappaB in these cells. Mutation of the AP-1, not the kappaB site, abolished the SARS-CoV S protein-induced IL-8 promoter activity. IL-8 release was effectively induced by vAtEpGS688, a baculovirus exhibiting the aa 17-688 fragment of S protein, and this induction was attenuated by the angiotensin-converting enzyme 2 Ab. Recombinant baculovirus expressing different deletion and insertion fragments identified the functional region of S protein from aa 324-688 (particularly the N-terminal aa 324-488 and the C-terminal aa 609-688), which is responsible for IL-8 production. Activations of AP-1 DNA-protein binding and MAPKs after vAtEpGS688 transduction were demonstrated, and SARS-CoV S protein-induced IL-8 promoter activity was inhibited by the specific inhibitors of MAPK cascades. These results suggested that the S protein of SARS-CoV could induce release of IL-8 in the lung cells via activations of MAPKs and AP-1. The identification of the functional domain for IL-8 release will provide for the drug design on targeting specific sequence domains of S protein responsible for initiating the inflammatory response.

Baculoviridae↗
PubMed2004

Analysis of childhood reactive arthritis and comparison with juvenile idiopathic arthritis.

There is currently no agreement on how to classify and diagnose reactive arthritis (ReA) and what kind of clinical and laboratory findings are specific for the diagnosis. This study retrospectively analyzed the initial clinical manifestations and laboratory findings in children diagnosed with ReA and juvenile idiopathic arthritis (JIA). A comparison was also made between these two groups to see if there were differences. A retrospective chart review was performed and 44 patients diagnosed with ReA and 80 patients with JIA were enrolled in this study. Their initial clinical manifestations and laboratory findings were also analyzed and compared. The initial clinical manifestations in ReA were analyzed including the demographic data, the preceding infection history, the duration of the infectious episode to the onset of arthritis, the duration of arthritic symptoms, and the involved joint pattern. Comparison of the initial laboratory findings between patients with ReA and JIA showed significant differences between erythrocyte sedimentation rates (ESR) in the first hour, platelet counts (p < 0.05), and ESR in the second hour (p = 0.052). Further, comparing ReA with the subtypes of JIA, significant differences were noted between ReA and the systemic type in terms of hemoglobin level, platelet counts, C-reactive protein, and first and second hour ESR (p < 0.05). However, if compared with the polyarticular or pauciarticular type, only the platelet counts showed any significant statistical difference (p < 0.05). This study summarizes clinical experiences in ReA. The differences in laboratory findings of ReA and JIA may provide a clue in making a differential diagnosis.

Adolescent↗
PubMed2004

The association between transforming growth factor-beta gene promoter C-509T polymorphism and Chinese children with Henoch-Schönlein purpura.

Many cytokines, including transforming growth factor-beta (TGF-beta) and tumor necrosis factor-alpha (TNF-alpha), are involved in the inflammatory process of Henoch-Schönlein purpura (HSP). The objective of this study was to investigate whether TGF-beta C-509T and TNF-alpha G-308A polymorphisms are associated with childhood HSP. The loci of interest were amplified from genomic DNA using specific primers and polymerase chain reaction, and these two polymorphisms were compared between Chinese children with HSP and healthy controls. The disease severities evaluated and expressed as symptom score of patients with different genotypes were also compared. The TGF-beta -509 TT genotype was more common in children with HSP than controls (31% vs. 8%, P =0.03, odds ratio=4.95). The allelic frequencies of TGF-beta -509, genotypic and allelic frequencies of TNF-alpha -308 were not significantly different. Patients with the TT genotype had more severe clinical presentations than non-TT (TC+CC) patients (4.1+/-0.42 vs. 2.7+/-0.31, P =0.018). These results suggest that the TT genotype of the C-509T polymorphism of the TGF-beta gene might be related to the susceptibility of Chinese children to HSP and to the severity of this disease.

Adolescent↗
PubMed2004

Retrospective analysis of the renal outcome of pediatric lupus nephritis.

The aim of this study was to analyze the renal outcome of pediatric lupus nephritis in the past two decades. We retrospectively reviewed the medical records of patients who fulfilled the 1987 American Rheumatism Association revised criteria for systemic lupus erythematosus who were followed up at the National Taiwan University Hospital between 1980 and 2001. All new patients who were under 18 years of age at the time of diagnosis were enrolled and were followed up until death, loss to follow-up, or till the end of 2002. The response to the treatment and renal outcome were analyzed. Seventy-two children (64 girls and 8 boys) were enrolled in the study. The mean age at diagnosis was 13.93+/-0.35 years (mean +/- SEM). The mean duration of follow-up was 7.12+/-0.51 years. The 5-year renal survival rate (survival without dialysis or transplantation) was 63.13% and the 10-year survival rate was 53.54%. It was significantly better in patients receiving cyclophosphamide (CYC) pulse therapy. The 5-year survival rate for these patients was 87.82% and the 10-year survival rate was 81.06%. The renal survival curve was better in the CYC pulse therapy group than in the no CYC pulse therapy group, with p=0.0022. The duration between the diagnosis of lupus nephritis and end-stage renal disease (ESRD) was significantly longer in the CYC group (9.66+/-1.32 yrs) than in the no CYC group (3.24+/-0.94 yers), p=0.036. In the multivariate analysis, risk factors of developing ESRD were failure to achieve complete remission, higher serum creatinine at the initiation of treatment, and not receiving CYC pulse therapy. The renal survival was significantly better in the CYC pulse therapy group. The CYC pulse therapy was recommended in pediatric lupus nephritis patients and every effort should be made to achieve complete remission.

Adolescent↗
PubMed2004

Risk of infection in hospitalised children with systemic lupus erythematosus: a 10-year follow-up.

The objective of this study was to investigate infection and sepsis in hospitalised children with systemic lupus erythematosus (SLE). Between 1991 and 2000 we reviewed 72 episodes of suspected infection in 42 children with SLE from all hospitalised children with SLE at a medical center in Taiwan. Data comprised clinical and laboratory characteristics of SLE at the time of infection. Infections were identified and categorised as minor or major. A total of 125 patients (110 girls, 15 boys) were admitted 557 times. Forty-two patients had infections, giving an infection rate of 33.6%. There were a total of 72 infections including 20 proven infections; 61 cases of these were minor infections and 11 cases of these were major infections (10 cases were sepsis). Four patients died because of sepsis. By univariate analysis, major infection (sepsis) was significantly associated with a high SLE disease activity index score, lower complement levels and higher anti-DNA titres. In conclusion, our data confirm that infection is common in hospitalised children with SLE. Sepsis, most frequent in major infections, is associated with disease activity and causes significant mortality. These facts should be borne in mind when children with SLE are hospitalised.

Adolescent↗
PubMed2004

Treatment of murine lupus using nucleosomal T cell epitopes identified by bone marrow-derived dendritic cells.

OBJECTIVE: Systemic lupus erythematosus (SLE) is characterized by the existence of a heterogeneous group of autoantibodies directed against intact nuclear structures, such as nucleosomes. The most prominent of these autoantibodies are those directed against double-stranded DNA (dsDNA) and histones. The majority are of the IgG isotype and show affinity maturation, both of which are known hallmarks of T cell help. Much evidence suggests that the nucleosome is a major candidate autoantigen in SLE. In this study, a novel strategy was used to identify the critical CD4+ T cell autoepitopes in nucleosomes. In addition, peptide-based therapy was then performed in a lupus animal model. METHODS: Bone marrow (BM)-derived dendritic cells (DCs) were used to examine the self-T cell responses against nucleosomes and to characterize the T cell epitope(s) of nucleosomes in (NZB x NZW)F1 (BWF1) mice. RESULTS: Several potential auto-T cell epitopes of core histone proteins (H2A, H2B, H3, and H4) were identified. Nucleosome-pulsed BM-derived DCs elicited release of interleukin-4 and interferon-gamma, representing a Th0 (i.e., mixed Th1 and Th2) pattern of cytokine production. In addition, intradermal immunization of BWF1 mice with the H3(111-130) peptide not only suppressed the level of anti-dsDNA and anti-single-stranded DNA IgG, but also significantly delayed the progress of glomerulonephritis in lupus-prone BWF1 mice. CONCLUSION: These results will help in understanding how pathogenic autoimmune responses develop in spontaneous SLE. This may potentially open the way to T cell-based immunotherapy for lupus.

Animals↗
PubMed2004

Waning immunity to plasma-derived hepatitis B vaccine and the need for boosters 15 years after neonatal vaccination.

Neonatal immunization with hepatitis B (HB) vaccine is highly effective; however, more needs to be learned about the duration of protection and indications for boosters. We measured antibody to HB core antigen (anti-HBc), HB surface antigen (HBsAg), and pre- and postbooster titers of HBsAg antibody (anti-HBs) 15 years after primary neonatal immunization with plasma-derived HB vaccines in 2 cohorts of 15-year-old children. Group A consisted of 78 children who were born to HB e antigen-positive HBsAg carrier mothers and had developed protective levels of anti-HBs antibodies (> or =10 mIU/mL) following HB immunization. Group B consisted of 113 apparently healthy children whose anti-HBs titers after vaccination were unknown. Anti-HBs was undetectable (antibody titer <10 mIU/mL) in 29.9% in group A and 62.4% in group B (P < .001). Anti-HBc was detected in 33.3 % in group A and 4.4 % in group B (P < .001). After a single booster dose of HB vaccine, 2.7% in group A and 3.3% in group B remained anti-HBs-negative. A blunted serological response was noted in approximately 20% in both groups. One HBsAg carrier was detected in group A (1.3%) and 4 in group B (3.5%). Fifteen years after neonatal immunization with plasma-derived HB vaccine, a large proportion of children exhibited waning immunity. This poses the risk of breakthrough infection. A single booster augmented the serological response to the vaccine in most but not all subjects. In conclusion, our findings suggest that one or more booster immunizations are needed in seronegative subjects by at least 15 years following neonatal immunization with plasma-derived HB vaccine.

Adolescent↗
PubMed2004

Dendritic cells modulated by cytokine-expressing adenoviruses alleviate eosinophilia and airway hyperresponsiveness in an animal model of asthma.

BACKGROUND: It has been found that TH1-related cytokines can decrease the accumulation of eosinophils in lung tissue and relieve airway constriction. OBJECTIVE: Dendritic cells (DCs) have been found to prime naive T-helper cells efficiently. In this study, DCs infected with TH1 cytokine-expressing adenovirus can be used to induce antigen-specific TH1 cells for treatment in an animal model of asthma. METHODS: Cytokine gene-modulated DCs pulsed with ovalbumin antigen (OVA) were injected intravenously into naive mice 1 week before sensitization with OVA antigen. The mice were then monitored for OVA-specific IgE, airway inflammatory cell infiltration, and airway hyperresponsiveness in the study. RESULTS: Significant levels of IL-12 or IL-18 were expressed by Ad-IL-12 or Ad-IL-18 infected, bone marrow-derived DCs. Ad-IL-12 and Ad-IL-18 co-infected DCs effectively, decreasing sera anti-OVA IgE antibody levels, lung eosinophilia, and airway hyperresponsiveness. CONCLUSION: We concluded that DCs modulated by TH1-prone cytokine-expressing adenoviruses can alleviate TH2-type airway inflammation in a murine model and can provide possible therapeutic application for DCs in asthma.

Adenoviridae↗
PubMed2004

Correlation between age and allergens in pediatric atopic dermatitis.

BACKGROUND: There has been significant progress recently in the clinical investigation of atopic dermatitis (AD), especially in the recognition of aeroallergens as aggravating factors in AD. However, sensitization to food allergens and aeroallergens in children with AD has not been fully studied. OBJECTIVE: To evaluate the correlation between age and allergens in children with AD. METHODS: A retrospective cross-sectional study was performed between January 1, 1999, and December 31, 2002, on 262 children with a definitive diagnosis of AD aged 0 to 16 years. Comparison of 3 age groups (<2 years, 2-5 years, and >5 years) with the correlation of food allergens and aeroallergens using the CAP-FEIA test was made using linear regression and logistic regression analyses. RESULTS: In children younger than 2 years, AD was associated with food allergens. In children aged 2 to 5 years, both food allergens and aeroallergens played an important role. In children older than 5 years, only elevated aeroallergen specific IgE levels were noted (P < .02). Age correlated negatively with food allergens and positively with aeroallergens after adjusting for sex (P < .04). Sensitization to aeroallergens may correlate with acquisition of the respiratory symptoms of the atopic state. CONCLUSIONS: Food allergens may be the major trigger of AD in early life, after which the role of environmental aeroallergens become more important and may be associated with respiratory sensitization.

Adolescent↗
PubMed2004

Epidemiologic features of Kawasaki disease in Taiwan, 1996-2002.

OBJECTIVE: Kawasaki disease (KD) is the most common acquired heart disease in children worldwide. The incidence of KD varies among different countries, with Asian countries supposedly having higher incidences than Western countries. However, the incidence of KD in Taiwan has not been well investigated. METHODS: Since the implementation of Taiwan's National Health Insurance (NHI) in 1995, NHI has covered health care for >96% its population. Using the NHI database from 1996 to 2002, we investigated epidemiologic features of KD (International Classification of Diseases, Ninth Revision, code 446.1), the rate of coronary artery aneurysm formation (International Classification of Diseases, Ninth Revision, code 414.11), and the change in incidence during the recent 7 years. We also compared the annual incidences in Taiwan with those reported by other countries. RESULTS: During the 7-year study period, KD occurred most frequently in the summer and least frequently in winter. It is interesting that the highest peak occurred in the summer of 1998 at the same time that Taiwan's enterovirus 71 epidemic was occurring. Ninety-one percent of KD cases occurred in children who were <5 years old, and the male-to-female ratio was 1.70:1. Recurrence of KD was found in 1.3% (94 of 7305) of these children, and coronary artery aneurysm was found in 7.3% (536 of 7305). The annual incidence per 100,000 children was 146 in children <1 year old, 98 in 1-year-old children, 51 in 2-year-old children, 28 in 3-year-old children, 19 in 4-year-old children, and 5.3 in 5- to 9-year-old children; the incidence of KD decreased with increased age. The overall incidence was 66 cases per 100,000 children <5 years old from 1996 to 2002 with the annual incidence not differing significantly during the 7-year study period. CONCLUSIONS: KD in Taiwan occurs more frequently in boys and in the summer months. During the 7-year study period, the annual KD incidence in Taiwan of 66/100000 in children <5 years old was the second highest in the world after Japan.

Adolescent↗
PubMed2004

Patient data, early SARS epidemic, Taiwan.

Of the first 10 patients in the epidemic of severe acute respiratory syndrome (SARS) in Taiwan, 4 were closely associated with a SARS patient in an airplane. Loose stools or diarrhea, hemophagocytosis syndrome, and high serum levels of interleukin (IL)-6, IL-8, and tumor necrosis factor-a associated with lung lesions were found in all 10 patients.

Adult↗
PubMed2004

Different familial association patterns of autoimmune diseases between juvenile-onset systemic lupus erythematosus and juvenile rheumatoid arthritis.

The aim of this study was to determine if the prevalence of autoimmune disorders in the relatives of patients with systemic lupus erythematosus (SLE) is greater than that of relatives of patients with juvenile rheumatoid arthritis (JRA). Interviews were used to obtain histories of the following autoimmune disorders among living or deceased first-, second-, and third-degree relatives of 91 SLE and 110 JRA families: ankylosing spondylitis, SLE, rheumatoid arthritis (RA), JRA, multiple sclerosis, juvenile dermatomyositis, Sjögren's syndrome, myasthenia gravis, psoriasis, and thyroid diseases. There were statistically significant differences between the SLE and JRA probands in mean age and gender ratio (19.1 +/- 4.8 vs 14.0 +/- 5.5 years; M (male)/F (female): 17/74 vs 62/48, p<0.005). The prevalence rate of autoimmune diseases in relatives of SLE families (20.9%) was greater than in JRA families (11.8%), but not statistically significantly so. The mean age (18.0 +/- 5.3 vs 14.0 +/- 4.3 years), mean age at diagnosis (13.4 +/- 4.3 vs 7.9 +/- 3.9 years) and gender ratio (F/M, 16/3 vs 5/8) of the patients with affected relatives between these 2 groups all had statistically significant differences. A higher prevalence of SLE in relatives was found in SLE families than in JRA cases. Furthermore, this study revealed a higher incidence of autoimmune disorders among second- and third-degree relatives of SLE or JRA probands versus first-degree ones, especially sisters (including 1 pair of twins) and the maternal aunt in SLE families. These data demonstrate that the prevalence of autoimmune disorders in the relatives of patients with SLE is greater than those of relatives of patients with JRA. This suggests that clinically different autoimmune phenotypes may share common susceptibility genes, which may act as risk factors for autoimmunity.

Adolescent↗
PubMed2004

The hyperimmunoglobulin E syndrome.

Hyperimmunoglobulin E syndrome is a primary immunodeficiency disease characterized by markedly high titers of serum immunoglobulin E (IgE), chronic eczema, recurrent staphylococcal infections, pneumatoceles, reduced neutrophil chemotaxis, and variable impaired T cell function. There are no clinical tools for diagnosis and definitive laboratory investigation. Variability of presentation makes it easy to confuse the diagnosis with that of severe atopy or other rare immunodeficiencies. We report a case of a 6-year-old boy with hyperimmunoglobulin E syndrome with recurrent methicillin-resistant Staphylococcus aureus furunculosis. Physical examination revealed a peculiar facial appearance, pruritic dermatitis, and furunculosis over the scalp, neck, and back. Laboratory investigation revealed mild leukocytosis with eosinophilia, a very high immunoglobulin E level, defective neutrophil chemotaxis, and impaired lymphocyte proliferation to anti-CD3/CD28 monoclonal antibodies. The boy was discharged without incident after 2 weeks of antibiotic therapy and debridement.

Chemotaxis, Leukocyte↗
PubMed2004

A patient diagnosed with pauciarticular juvenile rheumatoid arthritis after a mechanical prosthetic valve replacement due to aortic regurgitation.

Valvular heart disease is a rare complication of juvenile rheumatoid arthritis (JRA), with most cases associated with polyarticular JRA. The aortic valve is most commonly affected, and valvular involvement occurs months or years after the onset of JRA. Reported cases of valvular heart disease in patients with JRA in a pauciarticular pattern are rare. We report a case of severe aortic insufficiency in a 12-year-old boy who underwent aortic valve replacement before diagnosis of JRA with a pauciarticular pattern.

Aortic Valve↗
PubMed2004

Kikuchi-Fujimoto disease in children: clinical features and disease course.

Kikuchi-Fujimoto disease (KFD) is a rare cause of cervical lymphadenitis which mostly affects Asian women but is sometimes observed in the pediatric population. This study analyzed the clinical manifestations and disease course in children with KFD. Retrospective chart review and telephone interview were used to collect data for 13 children (8 boys and 5 girls) with a diagnosis of KFD from January 1988 to January 2003. Involvement of the posterior cervical lymph nodes was found in 12 patients, leukopenia in 9, and all patients had C-reactive protein less than 5 mg/dL. A high antinuclear antibody titer was associated with a more protracted and complicated course. Five of the 13 patients had new symptoms compatible with an autoimmune process during follow-up, with neurological symptoms the most common. In conclusion, the risk of evolution into an autoimmune syndrome in pediatric KFD patients is high, and careful long-term observation is mandatory.

Adolescent↗