159 research outputs found

    Management of hereditary angioedema in pediatric, pregnant, and breast-feeding patients: An expert opinion

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    Hereditary angioedema (HAE) is a rare inherited condition marked by recurrent skin and submucosal edema. HAE is caused by a C1 inhibitor deficiency or decreased C1 inhibitor function. The initial attack may occur during childhood or pregnancy, with symptoms ranging from classic angioedema to nonspecific stomach cramps. In this review, we discuss strategies for children and pregnant women to manage HAE attacks effectively and safely in light of the recent increase in HAE diagnosis. To begin, aggressive work-up is necessary to confirm HAE–1/2 and to determine the most effective countermeasures. Secondly, in the event of an acute attack, plasma-derived C1-inhibitor is the first line of defense for children and pregnant women. Icatibant is also appropriate for use, except in pregnant women. Fresh frozen plasma (FFP) may be suggested as an alternative. Thirdly, proactive measures to prevent HAE attacks should be considered whenever a procedure is performed that may result in an exacerbation. Finally, FFP, attenuated androgen and antifibrinolytic agents are recommended for long-term prophylaxis in South Korea where the C1-inhibitor is scarce. However, when making a decision, it is necessary to consider both the efficacy and the risk of adverse effects. For proper management, written action plans and first-aid kits are required. The action plans should be customized to the patients’ unique circumstances.ope

    Diagnosis and treatment of hereditary angioedema: An expert opinion

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    Hereditary angioedema (HAE) is a rare disease, but it severely interrupts daily life activities and can sometimes be life-threatening. Therefore, early diagnosis and prompt treatment of HAE attacks are critical. Physicians should be aware of how to diagnose and manage HAE to prepare not to miss a diagnosis when treating HAE patients. Physicians must also carry out tests to confirm the diagnosis of HAEs caused by C1 inhibitor deficiency (type 1) or C1 inhibitor dysfunction (type 2) in patients with recurrent angioedema. In addition, recent studies revealed another type of HAE which is not related to C1 inhibitor (normal C1 inhibitor HAE). Once HAE is confirmed, patients and their caregivers should be given with short-term and long-term treatment plans to relieve or prevent HAE attacks. HAE requires life-long measures, including psychological support for patients and self-management education.ope

    Soluble CD93 in allergic asthma

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    CD93 has been shown critical roles in inflammatory and immune diseases. However, in allergic asthma, the potential roles of soluble CD93 (sCD93) have not been well studied. We conducted house dust mite (HDM) stimulation with Der p 1 in BEAS-2B and U937 cells, followed by treatment with dexamethasone or small interfering RNA against CD93. A HDM-induced murine allergic asthma model was also established. We estimated the power of sCD93 to predict allergic asthma in a retrospective post-hoc analysis containing 96 human samples. HDM-stimulated BEAS-2B cells showed increased mRNA expression levels of IL-6, IL-8, IL-33, TSLP, and CD93. The CD93 level in culture supernatants steadily increased for 24 h after allergen stimulation, which was significantly suppressed by both dexamethasone and CD93 silencing. CD93 silencing increased IL-6 and TSLP, but not IL-33 levels in culture supernatants. HDM-induced asthma mice showed significant airway hyperresponsiveness and inflammation with Th2 cytokine activation, along with decreased CD93 expression in bronchial epithelial cells and lung homogenates but increased serum CD93 levels. The sCD93 level in asthma patients was significantly higher than that in healthy controls and could predict asthma diagnosis with moderate sensitivity (71.4%) and specificity (82.4%) (AUC = 0.787, P < 0.001). The level of sCD93 which has potential role to predict asthma significantly increased after HDM stimulation via IL-6 and TSLP in vitro and in vivo.ope

    Expert opinion: The clinical usefulness of skin tests prior to the administration of beta-lactam antibiotics

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    An allergy skin test is used to diagnose certain allergies by identifying sensitized allergens. In other words, it is a test for patients who are already sensitized to certain allergens. Because of the prevailing perception that beta-lactam allergy can be dangerous and potentially lethal, the intradermal test has long been routinely performed before use to screen beta-lactam allergy in Korea. The prevalence of penicillin allergy is estimated to be 1% to 2%. However, only 14% of the subjects with perceived penicillin allergy is considered to have true penicillin allergy. Moreover, it is difficult to justify performing a skin test on subjects who are very unlikely to be sensitized to beta-lactam, such as those who never used beta-lactam or never experienced allergy after previous use of beta-lactam. Therefore, allergists recommend beta-lactam skin testing in those who have allergy after the use of beta-lactam. Nevertheless, many hospitals in Korea are conducting routine skin tests on patients regardless of a history of beta-lactam allergy, which are not clinically validated but consume considerable human and material resources. False-positive results can consequently result in inappropriate labeling of beta-lactam allergy, leading to the unnecessary restriction of medication prescriptions and the increase in medical expenses. Herein, the drug allergy working group affiliated with the Korean Academy of Asthma, Allergy, and Clinical Immunology announces an expert opinion on the preuse beta-lactam skin test for subjects without a history of beta-lactam allergy based on the objective evidence from the literature and clinical relevance.ope

    Long-Term Prognosis of Asthma-Bronchiectasis Overlapped Patients: A Nationwide Population-Based Cohort Study

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    Purpose: Asthma and bronchiectasis are common chronic respiratory diseases, and their coexistence is frequently observed but not well investigated. Our aim was to study the effect of comorbid bronchiectasis on asthma. Methods: A propensity score-matched cohort study was conducted using the National Health Insurance Service-Health Screening Cohort database. From 2005 to 2008, 8,034 participants with asthma were weighted based on propensity scores in a 1:3 ratio with 24,099 participants without asthma. From the asthma group, 141 participants with overlapped bronchiectasis were identified, and 7,892 participants had only asthma. Clinical outcomes of acute asthma exacerbation(s) and mortality rates were compared among the study groups. Results: The prevalence of bronchiectasis (1.7%) was 3 times higher in asthmatics than in the general population of Korea. Patients who had asthma comorbid with bronchiectasis experienced acute exacerbation(s) more frequently than non-comorbid patients (11.3% vs. 5.8%, P = 0.007). Time to the first acute exacerbation was also shorter in the asthmatics with bronchiectasis group (1,970.9 days vs. 2,479.7 days, P = 0.005). Although bronchiectasis was identified as a risk factor for acute exacerbation (adjusted odds ratio, 1.73; 95% confidence interval [CI], 1.05-2.86), there was no significant relationship between bronchiectasis and all-cause or respiratory mortality (adjusted hazard ratio [aHR], 1.17; 95% CI, 0.67-2.04 and aHR, 0.81; 95% CI, 0.11-6.08). Conclusions: Comorbid bronchiectasis increases asthma-related acute exacerbation, but it does not-raise the risk of all-cause or respiratory mortality. Close monitoring and accurate diagnosis of bronchiectasis are required for patients with frequent exacerbations of asthma.ope

    The First Case Series of Cryopyrin-Associated Periodic Syndrome in Korea

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    Cryopyrin-associated periodic syndrome (CAPS) is a hereditary autoinflammatory syndrome caused by mutations in NLRP3 (encoding cryopyrin), which presents with fever, fatigue and arthralgia. Thus far, however there have been no reports of CAPS in Korea. Herein, we report 3 cases of CAPS for the first time in Korea. The first case, a 28-year-old man with recurrent urticaria, arthralgia and fever induced by cold, all of which were observed in his father, showed elevated erythrocyte sedimentation rate and C-reactive protein. He exhibited a p.Gly303Asp variant of the NLPR3 gene. The second case, a 2-year-old girl who had recurrent urticaria, arthritis and oral and genital ulcers, was positive for HLA B51 and a p.Glu569Lys mutation in exon 3 of the NLRP3 gene. Administration of anakinra greatly improved her symptoms. The third case, a 4-year-old boy who presented with recurrent urticaria, arthralgia, and fever, exhibited a p.Val72Met mutation in exon 1 of the NLRP3 gene. Administration of tocilizumab improved all of his symptoms. This small case series suggests that clinicians consider CAPS and conduct genetic studies when arthralgia and fever are accompanied by urticaria in Korea.ope

    Nicotine Dependence and Stress Susceptibility in E-Cigarette Smokers: The Korea National Health and Nutrition Examination Survey 2013-2017

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    Background: E-cigarettes are steadily gaining popularity in Korea. However, the characteristics of e-cigarette smokers, especially nicotine dependence and stress susceptibility, have not been evaluated in comparison to those of non-smokers or combustible cigarette smokers in Korea. Methods: In this study, 28,059 participants from the Korea National Health and Nutrition Examination Survey (2013-2017) were classified into the following three groups: non-smokers, smokers (current smokers and ex-smokers of combustible cigarettes only), and e-smokers (current smokers and ex-smokers of e-cigarettes regardless of combustible cigarette use). Results: Among the participants, 16,980 (60.5%), 9,247 (33.0%), and 1,832 (6.4%) subjects were non-smokers, smokers, and e-smokers, respectively. E-smokers were younger, more educated, and had a higher household income than non-smokers or smokers. The number of e-smokers who smoked within 5 minutes of waking up (31.5% vs. 19.8%, p<0.001) and who planned to quit smoking within 6 months (39.1% vs. 35.7%, p<0.05) was greater than that of smokers. E-smokers perceived stress as "very much" (7.0% vs. 4.4%, p<0.001) and "a lot" (29.1% vs. 20.5%, p<0.001) compared to non-smokers. Suicidal ideation (6.5% vs. 4.7%, p<0.001), plans (2.4% vs. 1.3%, p<0.001), and attempts (1.1% vs. 0.5%, p<0.001) were higher in e-smokers than in non-smokers. Depressive episodes in 1 year (14.2% vs. 11.4%, p<0.05) and suicidal plans (2.4% vs. 1.8%, p<0.05) were more frequent among e-smokers than among smokers. Conclusion: E-smokers were younger, more educated, and had a higher income, but they were more dependent on nicotine and susceptible to stress than non-smokers and smokers. Smoking cessation counseling should be tailored according to the characteristics of e-smokers.ope

    Recent advances in diagnostic technologies in lung cancer

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    The increase in lung cancer incidence of Korea has been dampened since 2000; however, increased human lifespan, interest in health care and the widespread implementation of health examinations have resulted in a considerable rise in detection of small lesions that need to be differentiated from lung cancer. Detection of lung cancer at an early stage rather than at a symptomatic advanced stage is also increasing, suggesting that there are increasing diagnostic demands for small peripheral lung lesions. The development of new molecular diagnostics, including next generation sequencing, companion diagnostics that accompany development of new anti-cancer drugs, and re-biopsy for application of new therapeutic modality accelerate the development of lung cancer diagnostics. In this review, we extensively describe the current available diagnostic tools in lung cancer.ope

    Application of Impulse Oscillometry in Adult Asthmatic Patients With Preserved Lung Function

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    Purpose: It is difficult to assess airway obstruction using spirometry in adult asthmatic patients with preserved lung function. Impulse oscillometry (IOS) can detect not only airway resistance but also reactance. Therefore, IOS may be useful in assessing pulmonary function in such patients. We investigated the applicability of IOS for asthma patients with preserved lung function. Methods: Between 2015 and 2018, 1,248 adult asthmatic patients suspected of having asthma who visited the Allergy and Asthma Center of Severance Hospital underwent both spirometry and IOS. Consequently, 784 patients had asthma, 111 had chronic obstructive lung disease (COPD) or asthma-COPD overlap, and 7 had parenchymal lung disease. The remaining 346 patients had chronic cough without underlying lung or airway disease. Among the 784 asthmatic patients, 191 with decreased lung function (predicted forced expiratory volume in 1 second [FEV1] < 80%) were excluded. Propensity score matching was performed to adjust baseline characteristics between 346 non-asthmatic and 593 asthmatic patients with preserved lung function. Subsequently, we compared the spirometry and IOS parameters between the 329 asthmatic and 329 non-asthmatic patients. Results: Multiple logistic regression analysis showed that the area of reactance (AX) was associated with asthma with preserved lung function. In receiver operating characteristic (ROC) curve analysis, the area under the curve (AUC) of AX (AUC = 0.6823) for asthma was not significantly different from that of FEV1 (AUC = 0.6758). However, the AUC of a combination of AX and FEV1 (AUC = 0.7437) for asthma was significantly higher than that of FEV1 alone. The cutoff value of AX was 0.51 kPa/L in univariate ROC analysis. Conclusions: AX is associated with adult asthma with preserved lung function. Performing spirometry together with IOS is more beneficial than performing spirometry alone for diagnosing asthma in adult patients with preserved lung function.ope

    Reliability of Portable Spirometry Performed in the Korea National Health and Nutrition Examination Survey Compared to Conventional Spirometry

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    Background: The Korea National Health and Nutrition Examination Survey (KNHANES) is a well-designed survey to collect national data, which many researchers have used for their studies. In KNHANES, although portable spirometry was used, its reliability has not been verified. Methods: We prospectively enrolled 58 participants from four Korean institutions. The participants were classified into normal pattern, obstructive pattern, and restrictive pattern groups according to their previous spirometry results. Lung function was estimated by conventional spirometry and portable spirometry, and the results were compared. Results: The intraclass correlation coefficients of forced vital capacity (FVC) (coefficient, 9.993; 95% confidence interval [CI], 0.988-0.996), forced expiratory volume in 1 second (FEV1) (coefficient, 0.997; 95% CI, 0.995-0.998), FEV1/FVC ratio (coefficient, 0.995; 95% CI, 0.992-0.997), and forced expiratory flow at 25-75% (FEF25-75%; coefficient, 0.991; 95% CI, 0.984-0.994) were excellent (all p<0.001). In the subgroup analysis, the results of the three parameters were similar in all groups. In the overall and subgroup analyses, Pearson's correlation of all the parameters was also excellent in the total (coefficient, 0.986-0.994; p<0.001) and subgroup analyses (coefficient, 0.915-0.995; p<0.001). In the paired t-test, FVC, FEV1/FVC, and FEF25-75% estimated by the two instruments were statistically different. However, FEV1 was not significantly different. Conclusion: Lung function estimated by portable spirometry was well-correlated with that estimated by conventional spirometry. Although the values had minimal differences between them, we suggest that the spirometry results from the KNHANES are reliable.ope
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