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Chromosome 22q11.2 deletion syndrome (22q11.2DS) is the most common microdeletion syndrome in humans and can present with highly variable clinical manifestations. Immune deficiencies occur because of thymic hypoplasia or aplasia.This retrospective study included patients diagnosed with 22q11.2DS at a medical center between 2000 and 2021. We analyzed the association between clinical phenotypes, immunological abnormalities, age, and outcomes.Eighty-seven patients with 22q11.2DS had a median diagnostic age of 1.78 months. Patients presented with congenital heart disease (CHD; 86.2%), major infections (75.9%), and failure to thrive (FTT; 58.6%). Autoimmunity, neuropsychiatric disorders, and hypoparathyroidism were significantly associated. Neonatal seizures were associated with early diagnosis before 2 months (OR 8.56, 95% CI 1.21-60.58, P = 0.032). Immunological abnormalities included lymphopenia (93.1%), T lymphopenia (71.9%), CD4CHD, major infection, and FTT were common manifestations and poor prognostic factors. Autoimmunity, neuropsychiatric disorders, and hypoparathyroidism were significantly associated. Although T lymphopenia may improve with age, patients with 22q11.2DS require lifelong monitoring for immune dysregulation.

Kidney transplantation is one of the treatments for end-stage renal disease in lupus patients. The impact of lupus activity on graft survival is a concern. In this study, we aimed to analyze graft survival and factors affecting outcome of kidney transplantation in 45 lupus patients. The graft survival rate was 98, 98, 88, 85 and 78% at 1, 5, 10, 15 and 20 years, respectively. Hypertension, positive penal reactive antibodies against HLA class 2 antigens, retransplant, younger age of lupus nephritis onset, lower postoperative C4 level and the presence of HBsAg and/or anti-HBe antibody showed significantly negative correlation on graft survival by univariate logistic regression. The only factor that is significantly associated with graft failure after adjusting above factors by multiple regression is the presence of HBsAg and/or anti-HBe antibody (p = .0161). Serological markers for activity including C3, C4 and anti-dsDNA antibody before kidney transplantation and recurrent lupus nephritis were not significantly associated with graft failure. In conclusion, in lupus patients underwent kidney transplantation, preoperative serological markers for lupus activity and recurrent lupus nephritis did not affect the graft outcome. However, hepatitis B virus serology might be a single predictor for graft failure in lupus patients.

Atopic dermatitis (AD) occurs in exclusively breastfed infants. As fatty acids have some immunomodulatory effect, we aimed to investigate the influence of fatty acid compositions in breast milk (BM) on the development of AD in exclusively breastfed infants.We enrolled two- to four-month-old exclusively breastfed infants. The objective SCORing Atopic Dermatitis (objSCORAD) was evaluated. The lipid layer of BM was analyzed by gas chromatography for fatty acid levels. Medical charts were reviewed.Forty-seven AD infants and 47 healthy controls were enrolled. The objSCORAD was 20.5 ± 1.7 (shown as mean ± SEM) in the AD group. The age, sex, parental atopy history, and nutrient intake of mothers were not significantly different between two groups. The palmitate and monounsaturated fatty acid (MUFA) levels in BM positively correlated with objSCORAD, while caprylate, acetate, and short-chain fatty acid (SCFA) levels negatively correlated with objSCORAD (p = .031, .019, .039, .013, .022, respectively). However, the butyrate levels in BM were not significantly different. The caprylate and acetate levels in BM were significantly associated with the presence of infantile AD (p = .021 and .015, respectively) after adjusting for age, sex, parental allergy history, MUFA, palmitate, and SCFA levels in BM. ObjSCORAD in infancy was significantly associated with persistent AD (p = .026) after adjusting for age, sex, parental atopy history, caprylate, palmitate, MUFA, acetate, and SCFA levels in BM.Caprylate and acetate levels in BM for exclusively breastfed infants were negatively associated with objSCORAD. Lower caprylate and acetate in BM might be the risk factors for infantile AD, while butyrate in BM was not associated with infantile AD.

Chronic granulomatous disease (CGD) is a rare primary immunodeficiency disease characterized by defective neutrophil killing of microbial pathogens and recurrent infections. We aimed to investigate the clinical, genetic features, treatment, and outcomes in patients with CGD.Pediatric patients diagnosed with CGD from a medical center in Taiwan were enrolled from January 1999 to Oct 2021.Nine pediatric patients with CGD were enrolled: six X-linked (XL) CGD with CYBB gene mutations, three autosomal recessive (AR) CGD with two NCF1 and one CYBA gene mutations. The median age of onset and age of diagnosis was 0.92 and 2.64 years, respectively. Patients with XL-CGD had a younger age of onset (4.6 months vs. 1.83 years, P = 0.06) and age of diagnosis (1.71 vs. 8.86 years, P = 0.024) than AR-CGD patients. The most common sites of infections were skin and soft tissue abscesses. The most common pathogens were Staphylococcus, Serratia, and Salmonella spp. Prophylactic antibiotics, anti-fungal agents, and interferon-gamma (IFN-γ) were given in 9 (100%), 7 (77.8%), and 8 (88.9%) patients, respectively. The mean duration of IFN-γ usage was 5.15 years. One male patient with XL-CGD was successfully treated with hematopoietic stem cell transplantation at 2.2 years. The mortality rate was 11.1%, and the estimated overall survival at 20 years was 66.7%.Staphylococcus aureus, Serratia marcescens, and Salmonella infections are important in Taiwanese CGD patients. Patients with XL-CGD have early disease onset. IFN-γ prophylaxis and prophylactic anti-microbial agents might have an effect on alleviating the infection episodes in CGD patients.

Childhood asthma is an inflammatory disease with heterogeneous outcomes. We sought to determine the impact of total IgE, blood eosinophil, allergen sensitization, and inhaled corticosteroid (ICS) on longitudinal outcomes and to identify characteristics for discriminating different outcomes.We conducted a retrospective study in 383 childhood asthma patients and another 313 patients with blood eosinophil data only receiving regular program-based visits from September 1, 2004, to December 31, 2018. Peak expiratory flow (PEF) variability, PEF predicted %, asthma severity, and asthma control at each visit were assessed as clinical outcomes.Our data show that the percentage of blood eosinophils was significantly associated with increased asthma severity (OR: 1.043, 95% CI: 1.002-1.086, P = 0.0392). Mold sensitization was significantly associated with asthma severity (OR: 2.2485, 95% CI: 1.3253-3.8150, P = 0.0027). Characteristics including sensitization status plus ICS dosage had the best area under the receiver operating characteristic curve (AUC) value for predicting longitudinal PEF predicted % (0.6609), PEF variability (0.6885), asthma severity (0.5918), and asthma control (0.6441), respectively.We showed that the risk for adverse clinical outcomes at follow-up differed between serum IgE, blood eosinophil, and allergen sensitization identified at baseline. Sensitization and ICS dosage were predictive characteristics of long-term clinical outcomes.The unique aspects of the study are its longitudinal assessment of patients receiving guideline-based asthma management program to help characterize the stability of the clinical outcomes over time. Characteristics including allergen sensitization and ICS dosage demonstrated an improved capability for distinguishing between better and worse clinical outcomes. Through longitudinal serial assessment, this study indicates the risk for adverse clinical outcomes differed between children with serum IgE/blood eosinophil/allergen sensitization characterized at baseline.

Background: The aim of this study was to evaluate whether breastfeeding should be discontinued for exclusively breast-fed infants with atopic dermatitis (AD). Methods: Eighty-seven exclusively breast-fed infants with AD were enrolled in a prospective observational study. The infants were divided into 3 groups: breastfeeding only (BM group), partial breastfeeding and partial partially hydrolyzed whey formula (pHF-W) (Partial group) and pHF-W only (DC group). The extent and severity of AD were evaluated with the Patient-Oriented SCORing Atopic Dermatitis (PO-SCORAD) index at enrollment and 3 and 6 months later. Results: There were no significant differences in parental atopy history, PO-SCORAD scores, and medication scores at baseline. At month 3 and 6, the PO-SCORAD scores were significantly decreased in all groups. PO-SCORAD scores at month 3 and 6 and at the last time point when topical corticosteroids were given were significantly different among the groups. Stepwise multiple linear regression analysis showed that baseline PO-SCORAD scores and stopping breastfeeding were significantly associated with month 3 PO-SCORAD scores (p

Background: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease. SLE patients are vulnerable to infections. Infections might mimic and even trigger SLE flares. To distinguish acute infection from activity flare always remains a clinical challenge.We aim to explore the approach to differentiate active infection from disease activity in pediatric SLE patients.Methods: Fifty pediatric SLE patients presenting with 185 clinical episodes were collected.The associations betweenrelevant data encompassing both clinical and laboratory parameters and the outcome groups were analyzed using generalized estimating equations (GEEs)facilitatingthe analysis of data collected in longitudinal and repeated-measures designs.We also used a multinomial logistic regression model for simultaneous prediction of different outcome groups. Finally, we ran the GLIMMIX procedure to track trends of changes in parameters over time.Results: These 185 episodes were divided into 4 outcome groups: infected-active (n=102), infected-inactive (n=11), noninfected-active (n=59), and noninfected-inactive (n=13) episodes. Multivariate GEE analysis showed that SDI, SLEDAI-2K, neutrophil‐to‐lymphocyte ratio (NLR), hemoglobin, platelet, RDW-to-platelet ratio (RPR), and C3 are predictive of flare (combined calculated AUC of 0.8964 and with sensitivity of 82.2 % and specificity of 90.9%). Multivariate GEE analysis showed that SDI, fever temperature, CRP, procalcitonin, lymphocyte percentage, NLR, hemoglobin, and renal score in SLEDAI-2k are predictive of infection (combinedcalculated AUC of 0.7886 and with sensitivity of 63.5% and specificity of 89.2%). We developed regression equations for simultaneous prediction of 4 different outcome groups.We could differentiate noninfected-active from infected-active episodes by different change patterns of ESR, NLR, lymphocyte, C3, and C4 over time.Conclusions: The proposed approach could differentiateflares from infections in pediatric SLE patients. Combination of parameters from four different domains simultaneously, including inflammation (CRP, ESR, PCT), hematology (Lymphocyte percentage, NLR, PLR), complement (C3, C4), and clinical status (SLEDAI, SDI), is effective to make appropriate judgement and treatment decisions.

BackgroundThough outcome differences between children and adults with immunoglobulin A vasculitis (IgAV) has been well documented, it remains unclear if disease features in pediatric IgAV patients vary with onset age. We aimed to explore clinical features and prognosis of pediatric IgAV stratified by onset age.MethodsWe retrospectively reviewed records of patients under 18 years old diagnosed with IgAV from January 1999 to December 2018 in one tertiary medical center in Taiwan. Patients were grouped by onset age: ≤ 6 years old, 6–12 years old (> 6, ≤ 12), and 12–18 years old (> 12, ResultsThere were 484 IgAV patients, with an onset age of 6.10 (4.72–8.58) (median (IQR)) years old. There were 234 (48.3%) patients ≤6 years old, 210 (43.4%) 6–12 years old, and 40 (8.3%) 12–18 years old. One hundred and thirty (26.9%) patients had renal involvement, which was more frequent in older children (≤ 6 years old, 18.4%; 6–12 years old, 31.0%; 12–18 years old, 55.0%;p p p ConclusionPediatric IgAV with different onset ages are associated with distinct clinical manifestations and outcomes. The risk of developing corticosteroid dependence, refractory disease and renal involvement increased with onset age.

BackgroundChildhood asthma is a heterogeneous disease. The relationship of total serum IgE and peripheral blood eosinophil with childhood asthma are controversial. To determine whether total serum IgE and peripheral blood eosinophil are associated with long-term lung function, asthma severity and asthma control in children.MethodsWe conducted a longitudinal retrospective study in 383 childhood asthma patients with total serum IgE and peripheral blood eosinophil obtained at baseline. Peak expiratory flow (PEF) variability, predicted PEF percentage, asthma severity, asthma control test (ACT) score, and asthma control from each follow-up visit were recorded as phenotype outcomes. We correlated serum IgE and blood eosinophil with phenotype outcomes using generalized estimating equations. Additionally, we compared expression of microRNA in peripheral blood cells from nonallergic versus allergic patients.ResultsTotal serum IgE was positively associated with asthma severity (OR = 1.0014, P = 0.0071). Peripheral blood eosinophil was positively related to PEF variability (OR = 1.0052, P = 0.0002). Association of inhaled corticosteroids (ICS) dose with phenotype outcomes were not significant. Compared with allergic patients, children with nonallergic asthma had higher asthma severity. We identified six interaction pathways specific to IgE from eight top differentially expressed microRNAs. ConclusionsBeside asthma control, total serum IgE is predictive of long-term asthma severity and peripheral blood eosinophil is closely related to PEF variability. We suggest that total serum IgE might be involved in modulating disease severity of both allergic and nonallergic asthma while peripheral blood eosinophilia reflects the status of longitudinal lung function.

Background: Growing pains is a benign, non-inflammatory pain syndrome of childhood, characterized by bilateral recurrent leg pain. Studies regarding the clinical and laboratory profile of growing pains are limited, especially in East Asia. The aim of this study was to analyze the clinical features, laboratory findings, and follow-up of children with growing pains.Methods: Patients under 18 years of age with growing pains in National Taiwan University Children’s Hospital between April 2006 and April 2019 were enrolled. Clinical features, laboratory data and medication records were analyzed. Changes of body height and weight before and after the symptoms were assessed by the paired samples t-test.Results: After excluding 31 children with musculoskeletal diseases, 276 patients with a final diagnosis of growing pains were included, comprising 152 boys (55%) and 124 girls (45%). The mean age was 4.9 ± 2.7 years. Bilateral and lower limbs pain were present in 232 (84%) and all cases, respectively. There were 6 out of 16 patients (38%) with elevated alkaline phosphatase, and 3 out of 50 (6%) with positive antinuclear antibodies in low titers. There were no significant changes in body height and weight percentiles or z-scores at 0.5, 1, and 2 years after diagnosis, compared with baseline values. Symptomatic treatments were used in 33% of patients.Conclusions: Growing pains is a common benign leg pain syndrome in children. Alkaline phosphatase elevation is commonly detected. It is not associated with rapid changes of body height and weight within 2 years of experiencing growing pains.

Background: Epidemiological findings showed the increased risk of allergic rhinitis (AR) and asthma in the children with preceding atopic dermatitis (AD). In this study, we aimed to observe the development of allergic diseases in infantile AD patients. Methods: We followed up the prospective observational cohort enrolling two-to four-month-old exclusively breastfed infants. The presence of physician-diagnosed asthma, AR and AD at age 3 was recorded with the laboratory tests for atopic sensitization. Results: Fifty infantile AD patients and 48 healthy controls were enrolled. The sex, age and parental atopy history were not significantly different between the two groups. At age 3, 21 (42%) patients had persistent AD in the infantile AD group while only 2 (4.2%) patients had newly diagnosed AD in the control group (p

Background Childhood asthma comprises different phenotypes with complex pathophysiology. Different asthma phenotypes evoke various clinical symptoms and vary in their responses to treatments. Methods We applied k-means clustering algorithm of twelve objective laboratory tests among 351 asthmatic children enrolled in the Taiwanese Consortium of Childhood Asthma Study (TCCAS). We constructed gene expression profiles of peripheral blood mononuclear cells (PBMC) from children with different asthma phenotypes. Results Five distinct phenotypes of childhood asthma were identified and can be characterized by either eosinophil-predominant or neutrophil-predominant inflammatory characteristics. In the gene expression profile analysis, significant differences were noted for neutrophil-predominant asthma, compared with samples from all the other asthma phenotypes. The vast majority of the differentially expressed genes in neutrophil-predominant asthma was associated with corticosteroid response. From an independent inhaled corticosteroid (ICS) response cohort, we also found neutrophils could be activated in this severe asthma phenotype and neutrophil-predominant asthma may be associated with corticosteroid nonresponsiveness. Conclusion Phenotype clustering of childhood asthma can be helpful to identify clinically relevant patients and reveal different inflammatory characteristics in asthmatic children. Neutrophil-predominant asthma is the most severe asthma phenotype with poor corticosteroid response. Gene expression profile of different asthma phenotypes not only improve our knowledge of childhood asthma, but also can guide asthma precision medicine.

Backgrounds Behçet’s disease (BD) is a rare vasculitic disorder affecting all sizes of vessels. Among BD patients, 4 to 25% of patients with diagnosed age younger than 16 years old are defined as juvenile BD (JBD). This study aimed to evaluate the clinical manifestations and treatments of patients with JBD, with a particular focus on the effectiveness and safety of anti-tumor necrosis factor (TNF)-alpha therapy. Methods We retrospectively reviewed data of all patients diagnosed with JBD at age of 16 years or younger in a tertiary hospital in Taiwan. The clinical manifestations, laboratory data, treatments, disease courses, and clinical outcomes were evaluated. The effectiveness of anti-TNF-alpha therapy was measured based on changes in Behçet’s Disease Current Activity Form (BDCAF) scores, prednisolone dosages and the immunosuppression load scores. Results Fifty-five patients were included in the study. The median age at disease onset was 11 years. The most common clinical presentation was recurrent oral aphthous ulcers (100%), followed by genital ulceration (69.1%), skin lesions (36.4%), gastrointestinal symptoms (29.1%), ocular involvement (27.3%), and arthralgia (27.3%). Ninety-one percent of the patients fulfilled the International Criteria for Behçet’s Disease, and 36.4% met the Paediatric Behçet’s Disease criteria. The most frequently used medications were prednisolone (74.5%) and colchicine (54.5%). Six patients with refractory or severe JBD received anti-TNF-alpha therapy. These patients were diagnosed at a younger age compared with those who did not receive anti-TNF-alpha therapy (7.5 vs 13 years; P = 0.012), the BDCAF scores reduced significantly at the 1st month, the 6th month and 1 year after the treatment. They did not use steroids after the first year of treatment, and, after treatment for 6 months, their immunosuppression load scores reduced significantly. Due to the limited case numbers, literature reviews of anti-TNF-alpha therapy for refractory JBD were conducted, which had a total 18 JBD patients receiving anti-TNF-alpha therapy, of which fifteen patients had favorable outcomes after treatment with minimal side effects. Conclusions Anti-TNF-alpha therapy may be necessary for JBD patients with refractory disease courses. Anti-TNF-alpha therapy was effective and safe in these patients, especially regarding its corticosteroid- and immunosuppressive drug-sparing effects.

Background Henoch-Schonlein purpura (HSP), the most common form of vasculitis in children, predominantly involves the small vessels of the skin, the gastrointestinal (GI) tract, joints, and kidneys. GI involvement is the most severe short-term complication, in contrast, renal disease is the most troublesome long-term complication. Meanwhile, although the disease course of HSP is usually benign in children, recurrence still occurs in a subset of patients. Studies documenting the incidence and predictive factors of recurrent HSP, and biomarkers of the various manifestations of HSP are limited. Objectives We aimed to delineate characteristics and biomarkers of HSP patients with GI or renal involvement, and hoped to clarify the incidence and predictive markers of recurrent HSP Methods We retrospectively reviewed the medical records of 40 patients admitted in one tertiary medical center in Taipei, Taiwan over a 5-year period with a diagnosis of Henoch-Schonlein purpura. Incidence and risk factors for GI involvement, renal involvement, and recurrent HSP were analyzed. The Mann-Whitney U test and Fisher exact test were utilized for statistical assessment. Results From January 1, 2005 to December 31, 2010, 40 HSP patients aged Conclusion HSP patients with GI involvement had higher segmented neutrophil percentage, consistent with previous studies proposing neutrophil-lymphocyte ratio as a biomarker for GI involvement. Patients with renal involvement had a later age of onset, and the relapse rate was higher in this subpopulation but not statistically significant. References [1] Hong, S. H., C. J. Kim and E. M. Yang (2018). “Neutrophil-to-lymphocyte ratio to predict gastrointestinal bleeding in Henoch: Schonlein purpura.” Pediatr Int 60(9): 791-795. [2] Lei, W. T., P. L. Tsai, S. H. Chu, Y. H. Kao, C. Y. Lin, L. C. Fang, S. D. Shyur, Y. W. Lin and S. I. Wu (2018). “Incidence and risk factors for recurrent Henoch-Schonlein purpura in children from a 16-year nationwide database.” Pediatr Rheumatol Online J 16(1): 25. Disclosure of Interests None declared

Background Our previous study showed that the discontinuation of breastfeeding could improve atopic dermatitis (AD) symptoms in exclusively breastfed infants. As vitamins A and D are influential on the immune system, we aimed to analyze the association of vitamin A and D levels in breast milk (BM) with AD. Methods We enrolled two- to four-month-old exclusively breastfed infants. The objective SCORing Atopic Dermatitis (objSCORAD) was evaluated. The lipid layer of BM was extracted and analyzed by liquid chromatography for vitamin A and D levels. Medical charts were reviewed for the clinical course of AD. Results Forty-five AD patients and 45 healthy controls were enrolled. The objSCORAD was 20.54 ± 1.73 (shown as mean ± SEM) in the AD group. The sex, parental atopy history, nutrient intake of mothers, and vitamin A levels in BM were not significantly different between the two groups. The 25-(OH) D3 level in BM was significantly lower in the AD group than in the control group (1.72 ± 0.30 and 3.95 ± 0.64 ng/mL, respectively; P = .001). The 25-(OH) D3 level negatively correlated with objSCORAD (P = .003). The only factor that is significantly associated with persistent AD is the objSCORAD in infancy (P = .003) after adjusting for age, sex, parental atopy history, and 25-(OH) D3 level by multiple regression. Conclusion Vitamin D levels in BM for exclusively breastfed infants were negatively associated with objSCORAD. Lower vitamin D levels in BM might be a risk factor for infantile AD.

Background It has been reported that physical activity is limited in children with asthma. The aims of this study were to compare and quantify the physical activity levels between asthmatic children and their healthy peers. Factors associated with limitation of physical activity in asthmatic children were also investigated. Methods A total of 120 asthmatic children and 262 age-matched healthy controls were enrolled in this study. Clinical phenotype including severity and lung function were obtained from medical records. A questionnaire addressing physical activity, asthma diagnosis, symptoms, parental health beliefs, physician's advice and, community resources was accomplished by children and their parents. The physical activity levels of children with and without asthma were compared. Factors that might limit the activity level were analyzed. Results Children with asthma were less active than their peers. The significant difference was between normal controls and moderate-to-severe asthmatic children, but not children with mild asthma. Among children with asthma, physical activity was associated with the severity level but not parental health beliefs, physician's advice, or the convenience for physical activity. Children with moderate or severe persistent asthma were more likely to be inactive. Conclusion Children with asthma had a lower level of physical activity, particularly those with moderate-to-severe asthma. To achieve an appropriate level of physical activity, improvement of asthma management and control is considered important.

Background:Systemic lupus erythematosus (SLE) is a life-threatening autoimmune disease that affects many women of child-bearing age, with potentially severe consequences on pregnancy outcome. SLE flare-ups may occur during pregnancy and the post-partum period. Previous studies documenting the incidence and risk factors of flare-up of SLE during pregnancy and puerperium had partially discordant results.Objectives:We aimed to delineate the pregnancy complications of women with SLE, as well as neonatal outcomes of their offspring, and hoped to clarify the incidence and risk factors of SLE flare-ups during pregnancy and puerperium.Methods:We retrospectively reviewed the medical records of SLE patients with previous records of pregnancies in our institution. Flare events during pregnancy and puerperium were documented. The pregnancy outcomes recorded include live births, intra-uterine fetal death (IUFD), premature delivery (< 36 weeks of gestational age), NICU admission, and small for gestational age (SGA, thpercentile). Univariate logistic regression was performed to determine the factors associated with disease relapse and pregnancy outcomes.Results:From January, 2000 to December, 2019, a total of 94 SLE patients with 139 pregnancies were identified. The overall live birth rate was 92.4% (134/145). Forty-six (34.3%) of the neonates were delivered prematurely. Forty-six (34.3%) of them were SGA. The admission rate to the neonatal intensive care unit was 25% (30/120). Nine (6.4%) were diagnosed to have SLE during pregnancy. The flare rate during pregnancy was 20% while post-partum 9.4%. The majority of the relapses during pregnancy occurred in the second trimester (46.2%), followed by the first trimester (30.8%), and the third trimester (23.1%). Low complement C3 (C3 < 80mg/dl), thrombocytopenia (PLT < 100*103/uL) at conception, and low serum albumin level at the first trimester were associated with antepartum flare. Presence of disease flare and pre-eclampsia in pregnancy, and low serum albumin level at conception were significantly associated with premature delivery.Conclusion:Low complement C3 and thrombocytopenia at conception, and low serum albumin level at the first trimester were associated with disease flare-up during pregnancy. Patients with relative low serum albumin level at conception, or presence of eclampsia or disease flare-up during pregnancy had a higher risk of premature delivery.References:[1]Shaharir SS, Mohamed Said MS, Mohd R, et al. Predictors of SLE relapse in pregnancy and post-partum among multi-ethnic patients in Malaysia.PloS one2019;14(9):e0222343.[2]Bundhun PK, Soogund MZ, Huang F. Impact of systemic lupus erythematosus on maternal and fetal outcomes following pregnancy: A meta-analysis of studies published between years 2001-2016.Journal of autoimmunity2017;79:17-27.Disclosure of Interests: :None declared

Objective The anti-inflammatory and cardiovascular protective effects of statin for patients with systemic lupus erythematosus (SLE) are not clear. We tested the hypothesis that statin use is associated with reduced mortality and morbidity in SLE patients with hyperlipidemia. Methods We included 4095 patients with SLE and hyperlipidemia from the entire population using the Taiwan National Health Insurance Research Database between 1997 and 2008. A total of 935 matching sets (1:2) of patients who had never used lipid-lowering medications and statin users were included in the nested matched cohort. Cox proportional hazards regression was used to calculate the hazard ratios (HR) and 95% confidence intervals (CI) for the association between statin and all-cause mortality, coronary artery disease (CAD), cerebrovascular disease (CVD) and end-stage renal disease (ESRD), conditional for matching sets in the matched cohort. Results The multivariate adjusted hazard ratios (HR) for statin users, as compared with patients had never used lipid-lowering medications, were 0.67 (95% CI, 0.54 to 0.83) for death from any cause. High-dose statins (>365 cumulative defined daily dose) significantly reduced risk of all-cause mortality (HR 0.44, 95% CI 0.32 to 0.60); CAD (HR 0.20, 95% CI 0.13 to 0.31); CVD (HR 0.14, 95% CI 0.08 to 0.25); and ESRD (HR 0.22, 95% CI, 0.16 to 0.29), with similar results in the nested matched study. Conclusion Statin therapy in SLE patients with hyperlipidemia may reduce the risk of mortality, cardiovascular disease and ESRD. The effect of statins needs to be demonstrated in large prospective studies with long-term follow-up.

BACKGROUND AND OBJECTIVES: Sleep disturbance is common in patients with atopic dermatitis (AD). However, studies have largely been questionnaire-based, and the pathophysiology remains unclear. The aims of this study were to determine objective characteristics of sleep disturbance in children with AD and explore contributing factors and clinical predictors. METHODS: Sleep parameters were measured by actigraphy and polysomnography in 72 patients with AD and 32 controls ages 1 to 18 years. Urinary 6-sulfatoxymelatonin levels, serum cytokines, and total and allergen-specific immunoglobulin E (IgE) levels were also measured. RESULTS: The patients with AD had significantly reduced sleep efficiency, longer sleep onset latency, more sleep fragmentation, and less nonrapid eye movement sleep. Results from actigraphy correlated well with those from polysomnography. The AD disease severity was associated with sleep disturbance (r = 0.55−0.7), and a Scoring Atopic Dermatitis index of ≥48.7 predicted poor sleep efficiency with a sensitivity of 83.3% and a specificity of 75% (area under the curve = 0.81, P = .001). Lower nocturnal melatonin secretion was significantly associated with sleep disturbance in the patients with AD. Other correlates of sleep disturbance included pruritus, scratching movements, higher total serum IgE levels, and allergic sensitization to dust mite and staphylococcal enterotoxins. CONCLUSIONS: Poor sleep efficiency is common in children with AD and can be predicted by the Scoring Atopic Dermatitis index. Melatonin and IgE might play a role in the sleep disturbance. Further studies are required to explore the mechanisms and clinical implications, and actigraphy could serve as a useful evaluating tool.

Gastrointestinal (GI) involvement in childhood Behçet disease (BD) is not well understood. We aimed to clarify the intestinal presentation in children with BD.Medical records of 85 children with recurrent oral ulcers between 1990 and 2010 at the National Taiwan University Hospital were reviewed retrospectively. Twenty of them who fulfilled the Mason and Barnes criteria for the diagnosis of childhood BD were enrolled. The clinical and laboratory characteristics were analyzed.Among 20 patients, the median age at diagnosis was 13.2 years. The common presentations included oral ulcers (100%), genital ulcers (70%), skin lesions (65%), and GI symptoms (50%). Five of 10 patients with GI symptoms received endoscopic examinations and all had ulcers. Divided by the age of 10, patients younger than 10 years tended to have higher rates of GI symptoms initially and intestinal ulcers (P = 0.002 and 0.015, respectively). Platelet count was significantly lower in young patients (P = 0.0151). Patients without GI symptoms had higher rates of skin involvement than patients with GI symptoms (P = 0.019).Young children with BD tended to have more GI presentations. For children with BD younger than 10 years having GI symptoms, endoscopic examinations may be considered.

ObjectiveTo clarify the epidemiology of asthma admission in a nationwide survey, we analyze the characteristics of hospitalized patients in Taiwan.MethodsData regarding asthma-associated hospitalizations were obtained from the National Health Insurance Research Database of Taiwan from January 2001 to December 2002. Patient characteristics in terms of severity, mortality, and cost were stratified by age, gender, and presence of comorbidities.ResultsA total of 59,983 hospitalizations with International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code 493.xx and asthma-related treatment were included in the current analysis. When stratified by age, the number and duration of hospitalizations were distributed in J-shape distribution, with the nadir located in the range of 8–18 years of age. The severity of asthma was found to increase with age (p

Background/Purpose Increased IgE and eosinophil levels are frequently observed in cutaneous inflammation and are thought to provoke the occurrence of urticaria. However, the relationship of these factors with the disease duration of urticaria remains unclear. The aim of this study was to compare serum total IgE levels, specific IgE sensitization rates and eosinophil percentages between acute and chronic urticaria in children. Methods A total of 165 patients (104 with acute and 61 with chronic urticaria) from a tertiary referral hospital were enrolled in this study. Serum levels of total IgE, prevalence of sensitization to food and aeroallergens and blood eosinophil percentages were compared by the disease duration of urticaria. Results There were no statistical differences in total IgE production, positive sensitization to specific allergens and eosinophil percentages between the patients with acute and chronic urticaria. There is a higher prevalence of sensitization to aeroallergens than food allergens in children with urticaria. In terms of gender, males had significantly higher serum IgE levels than females. Conclusion Boys potentially have a higher serum IgE expression than girls children with urticaria. IgE levels and eosinophil percentages are not good indicators for a prolonged course of urticaria. The prevalence of sensitization to aeroallergens was significantly higher than that of food allergens in children with urticaria. Routine laboratory analysis for common allergens is not appropriate, and it could be a feasible approach to detect a predilection for atopy when respiratory infections are causative factors of urticaria occurrence.

Tai-Chi-Chuan (TCC) is an exercise of low-to-moderate intensity which is suitable for asthmatic patients. The aim of our study is to investigate improvements of the lung function, airway inflammation, and quality of life of asthmatic children after TCC. Participants included sixty-one elementary school students and they were divided into asthmatic (n=29) and nonasthmatic (n=32) groups by the International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire. Among them, 20 asthmatic and 18 nonasthmatic children volunteered to participate in a 60-minute TCC exercise weekly for 12 weeks. Baseline and postintervention assessments included forced expiratory volume in one second (FEV1), forced vital capacity (FVC), peak expiratory flow rate (PEFR), fractional exhaled nitric oxide (FeNO) level, and Standardised Pediatric Asthma Quality of Life Questionnaire (PAQLQ(S)). After intervention, the level of FeNO decreased significantly; PEFR and the FEV1/FVC also improved significantly in both asthmatic group and nonasthmatic group after TCC. The asthmatic children also had improved quality of life after TCC. The results indicated that TCC could improve the pulmonary function and decrease airway inflammation in both children with mild asthma and those without asthma. It also improves quality of life in mild asthmatic children. Nevertheless, further studies are required to determine the effect of TCC on children with moderate-to-severe asthma.

Summary Background Henoch–Schonlein purpura (HSP) is a common IgA-mediated vasculitis in children. The antigenic target for IgA is to be determined. Objective To test whether β2-glycoprotein I (β2GPI) is an antigenic target for IgA in childhood HSP, and to evaluate the clinical implications and pathogenic role of such IgA autoantibodies. Methods The reactivity of patients’ plasma samples and purified polyclonal IgA with β2GPI, β2GPI-derived peptides and endothelial cells was tested by enzyme-linked immunosorbent assay. The association between clinical manifestations and IgA anti-β2GPI antibodies was also analysed. Finally, IgA-mediated cytotoxicity on endothelial cells was further evaluated. Results At the acute stage, patients with HSP had significantly higher plasma levels of IgA antibodies against β2GPI than healthy controls [reference units (RU) 1·14 ± 0·8 vs. 0·42 ± 0·24, P

Background/Purpose To investigate the clinical manifestations, disease activity and prognosis in different types of vasculitis. Methods The charts of pediatric patients with vasculitis diagnosed from December 1997 to December 2007 were retrospectively reviewed. The first clinical manifestations and laboratory results were recorded at the time of diagnosis, and outcome evaluations with history of flare-ups were analyzed. Results A total of 508 vasculitis patients were included in this study, of whom 124 had Henoch–Schonlein purpura (HSP), and 351 had Kawasaki disease (KD). Hematuria was observed in 79% of recurrent HSP patients at the time of diagnosis, and was associated with an increased risk of relapse ( p = 0.000). In Kawasaki disease, the clinical symptoms with erythematous changes in Bacille Calmette-Guerin scars and coronary artery dilatation were more prominent in patients younger than 1 year old, and lymphadenopathy was more common in patients older than 1 year old ( p = 0.001). The risk of coronary dilatation was significant in the patients with an initial presentation of thrombocytosis, and greater in patients younger than 1 year old ( p = 0.027). Thrombocytopenia was more prominent in vasculitis-associated autoimmune diseases. Marked lymphocytosis with increased C-reactive protein levels was significantly noted in urticarial vasculitis patients compared with HSP patients in multivariate logistic regression analysis. Conclusion Vasculitis disease activity and prognosis were associated with initial laboratory results and clinical manifestations. Further large-scale clinical trials are warranted to validate these findings.

Cite this as: Y.-T. Lin, C.-T. Wang, P.-S. Chao, J.-H. Lee, L.-C. Wang, H.-H. Yu, Y.-H. Yang and B.-L. Chiang, Clinical & Experimental Allergy, 2011 (41) 516–525. Summary Background The effect of staphylococcal superantigens (SsAgs) on cutaneous lymphocyte-associated antigen (CLA)+ CD4+ Foxp3+ T cells of atopic dermatitis (AD) patients is unknown. Objective To compare the effects of SsAgs on the ratio, function, and apoptosis of CCR6+ subtype and CCR6− subtype of CLA+ CD4+ Foxp3+ T cells among AD patients, asthma/allergic rhinitis (AR) patients without AD, and healthy subjects. Methods Using immunofluorescence staining followed by flow cytometric analysis, we analysed peripheral blood mononuclear cells cultured with or without staphylococcal enterotoxin B (SEB) stimulation in 20 AD patients, 20 asthma/AR patients without AD, and 20 healthy subjects. Results SEB decreased CCR6+/CCR6− ratio in CLA+ CD4+ Foxp3+ T cells from AD patients and increased CCR6+/CCR6− ratio in those from healthy subjects. SEB induced the production of type 2 T helper cell (Th2) cytokine interleukin (IL)-5 in CCR6− subtype and anti-inflammatory cytokine IL-10 in CCR6+ subtype of CLA+ CD4+ Foxp3+ T cells. CLA+ CD4+ Foxp3+ T cells from AD patients produced more IL-5 and less IL-10 after SEB stimulation than those from healthy subjects. CCR6− subtype of CLA+ CD4+ Foxp3+ T cells from AD patients and CCR6+ subtype of those cells from healthy subjects were more resistant to SEB-induced caspase-3 activation than the other subtype and those from other subjects. Conclusions and Clinical Relevance Despite a phenotype of regulatory T cells, skin-homing CD4+ Foxp3+ T cells of AD patients exert effector Th2-like function after SsAgs stimulation, which may aggravate allergic skin inflammation.

IgA nephropathy (IgAN) is the most common glomerulonephritis in the world. The hallmark of IgAN is underglycosylation in the hinge region of IgA1. Increasing evidence supports the underglycosylated IgA-containing immune-complex including IgG antibodies against the glycans of the hinge region of IgA1 are key factors for mesangial deposition and then trigger inflammation and glomerular injury. The polymeric IgA is produced after aberrant mucosal IgA response. The displacement of mucosal B cells to systemic lymphoid organs and bone marrow may arise from abnormal trafficking of lymphocytes along the mucosa-bone marrow axis involving changes of chemokines and adhesion molecules. This review will summarize the works on the genetics, the mucosal and systemic IgA immune response, mechanism of underglycosylation of IgA1, and the pathological effect of mesangial IgA deposition in IgAN.