Semantic variant primary progressive aphasia (svPPA), a well-known subtype of the frontotemporal dementia often shows peculiar clinical features and structural neuroimage findings. To strengthen the accuracy of a clinical diagnosis, amyloid positron emission tomography-computed tomography (PET-CT) might be helpful. However, in patients with late-onset svPPA, an admixture of the various neuropathology would interfere with diagnostic approach. Herein, we report a case of late-onset bilateral svPPA showing a regional amyloid deposition on PET-CT scan.

Background: For acute ischemic stroke (AIS) patients with history of prior stroke (PS) and diabetes mellitus (DM), intravenous recombinant tissue plasminogen activator (IV-tPA) therapy in the 3- to 4.5-hour window is off-label in Korea. This study aimed to assess the safety and efficacy of IV-tPA in these patients. Methods: Using data from a prospective multicenter stroke registry between January 2009 and March 2021, we identified AIS patients who received IV-tPA in the 3- to 4.5-hour window, and compared the outcomes of symptomatic intracranial hemorrhage (SICH), 3-month mortality, 3-month modified Rankin Scale (mRS) score 0-1 and 3-month mRS distribution between patients with both PS and DM (PS/DM, n=56) versus those with neither PS nor DM, or with only one (non-PS/DM, n=927). Results: The PS/DM group versus the non-PS/DM group was more likely to have a prior disability, hypertension, hyperlipidemia, coronary heart disease and less likely to have atrial fibrillation. The PS/DM and the non-PS/DM groups had comparable rates of SICH (0% vs. 1.7%; p>0.999) and 3-month mortality (10.7% vs. 10.2%; p=0.9112). The rate of 3-month mRS 0-1 was non-significantly lower in the PS/DM group than in the non-PS/DM group (30.4% vs. 40.7%; adjusted odds ratio [95% confidence interval], 0.81 [0.41-1.59]). Conclusions In the 3- to 4.5-hour window, AIS patients with PS/DM, as compared to those with non-PS/DM, might benefit less from IV-tPA. However, given the similar risks of SICH and mortality, IV-tPA in the late time window could be considered in patients with both PS and DM.

Background: Atopic dermatitis (AD) is a heterogeneous disease with different age of onset, disease course, clinical symptoms, severity, and risk of comorbidity. The characteristics of children with AD also vary by age or country. However, little is known about the clinical characteristics of AD in Korean school-aged children and adolescents. Furthermore, there are few studies on phenotypic differences according to onset age. This study aimed to explore the clinical characteristics and phenotypes according to onset age and severity of AD in children and adolescents in Korea. Methods: AD patients aged 6–18 years who presented to 18 hospitals nationwide were surveyed.The patients were examined for disease severity by pediatric allergy specialists, and data on history of other allergic diseases, familial allergy history, onset age, trigger factors, lesion sites,treatment history and quality of life were collected. The results of the patient’s allergy test were also analyzed. The patients were classified into infancy-onset (< 2 years of age), preschoolonset (2–5 years of age), and childhood-onset (≥ 6 years of age) groups. Study population was analyzed for clinical features according to onset-age groups and severity groups. Results: A total of 258 patients with a mean age of 10.62 ± 3.18 years were included in the study. Infancy-onset group accounted for about 60% of all patients and presented significantly more other allergic diseases, such as allergic rhinitis and asthma (P = 0.002 and P = 0.001, respectively). Food allergy symptoms and diagnoses were highly relevant to both earlier onset and more severe group. Inhalant allergen sensitization was significantly associated with both infancy-onset group and severe group (P = 0.012 and P = 0.024, respectively). A family history of food allergies was significantly associated with infancyonset group (P = 0.036). Severe group was significantly associated with a family history of AD, especially a paternal history of AD (P = 0.048 and P = 0.004, respectively). Facial (periorbital, ear, and cheek) lesions, periauricular fissures, hand/foot eczema, and xerosis were associated with infancy-onset group. The earlier the onset of AD, the poorer the quality of life (P = 0.038). Systemic immunosuppressants were used in only 9.6% of the patients in the severe group. Conclusion This study analyzed the clinical features of AD in Korean children and adolescents through a multicenter nationwide study and demonstrated the phenotypic differences according to onset age and severity. Considering the findings that the early-onset group is more severe and accompanied by more systemic allergic diseases, early management should be emphasized in young children and infants.

With increasing need to prevent serious food allergy reactions, Korean food allergen labeling regulation has been revised repeatedly. This paper aims to summarize current statuses of food allergen labeling in Korea and foreign countries and to analyze the issue of food allergen labeling regulation. Korean food labeling regulation currently requires 19 items and 22 foods to be reported on labels (eggs, milk, buckwheat, peanut, soybean, wheat, mackerel, crab, shrimp, pork, peach, tomato, sulfite, walnut, chicken, beef, squid, shellfish, and pine nut). However, some common food triggers (for example, almond, cashew nut, and kiwi fruit) are not included in the current labeling regulation. Another issue is that the Korean labeling regulation has not yet been fully implemented for nonprepacked foods; thus, consumers still have difficulty in correctly identifying allergenic ingredients in food. It should be assessed whether warning statements for cross-contamination are reasonable. To prevent the occurrence of serious reactions from accidental ingestion, efforts must be made to solve recently raised issues including the items required to be listed on food labels, the system of standards for labeling and display methods.
Anacardium ; Arachis ; Chickens ; Decapodiformes ; Eating ; Fagopyrum ; Food Hypersensitivity ; Food Labeling ; Hypersensitivity ; Juglans ; Korea ; Lycopersicon esculentum ; Milk ; Nuts ; Perciformes ; Prunus dulcis ; Prunus persica ; Red Meat ; Shellfish ; Soybeans ; Triticum

Food allergy has emerged as an important public health problem affecting people of all ages in many countries. The prevalence varies according to age, geographic regions, and ethnicity. For several years, many studies have suggested that the prevalence of food allergy is increasing at an alarming rate, for unclear reasons. Conversely, some studies have also provided findings that sensitization to common food allergens did not increase. Increased recognition rather than an actual increase in patients with IgE-mediated food allergy might lead to the increases in the prevalence of self-reported or physician-diagnosed food allergy. It is also noted that the prevalence of food allergy differs even in the same region according to the study design, i.e., hospital-based or community-based studies. Despite these limitations, epidemiologic data are important because they provide useful information on diagnosis, treatment, and prevention of food allergy. This review focuses on advances in the epidemiology of food allergy in Korean children.
Allergens ; Child ; Diagnosis ; Epidemiology ; Food Hypersensitivity ; Humans ; Prevalence ; Public Health

PURPOSE: Although anaphylaxis is recognized as an important, life-threatening condition, data are limited regarding its triggers in different age groups. We aimed to identify anaphylaxis triggers by age in Korean children. METHODS: We performed a retrospective review of medical records for children diagnosed with anaphylaxis between 2009 and 2013 in 23 secondary or tertiary hospitals in South Korea. RESULTS: A total of 991 cases (mean age=5.89±5.24) were reported, with 63.9% involving patients younger than 6 years of age and 66% involving male children. Food was the most common anaphylaxis trigger (74.7%), followed by drugs and radiocontrast media (10.7%), idiopathic factors (9.2%), and exercise (3.6%). The most common food allergen was milk (28.4%), followed by egg white (13.6%), walnut (8.0%), wheat (7.2%), buckwheat (6.5%), and peanut (6.2%). Milk and seafood were the most common anaphylaxis triggers in young and older children, respectively. Drug-triggered anaphylaxis was observed more frequently with increasing age, with antibiotics (34.9%) and nonsteroidal anti-inflammatory drugs (17.9%) being the most common causes. CONCLUSIONS: The most common anaphylaxis trigger in Korean children was food. Data on these triggers show that their relative frequency may vary by age.
Anaphylaxis* ; Anti-Bacterial Agents ; Arachis ; Child* ; Contrast Media ; Egg White ; Epidemiology ; Fagopyrum ; Humans ; Juglans ; Korea ; Male ; Medical Records ; Milk ; Retrospective Studies* ; Seafood ; Tertiary Care Centers ; Triticum

No abstract available.

Oral food challenge is a definitive diagnostic test for immediate and occasionally delayed adverse reaction to foods. The gold standard for diagnosing food allergy is still the double-blind, placebo-controlled food challenge, but it is time-consuming, expensive and troublesome for physician and patients. Open oral food challenge controlled by trained personnel is useful and sufficient methods when concern of bias is low. We aimed to provide a practical guideline for oral food challenge in children for the diagnosis of suspected food allergy or the evaluation of food tolerance. We considered reasons, types, indications, contraindications, risks, benefits, detailed methods, practical performance, interpretations of test results, and treatments for the adverse reactions of oral food challenge.
Bias (Epidemiology) ; Child ; Diagnostic Tests, Routine ; Food Hypersensitivity ; Humans

Many patients assume that allergic reactions against foods are responsible for triggering or worsening their allergic symptoms. Therefore, it is important to identify patients who would benefit from an elimination diet, while avoiding unnecessary dietary restrictions. The diagnosis of food allergy depends on the thorough review of the patients's medical history, results of supplemented trials of dietary elimination, and in vivo and in vitro tests for measuring specific IgE levels. However, in some cases the reliability of such procedures is suboptimal. Oral food challenges are procedures employed for making an accurate diagnosis of immediate and occasionally delayed adverse reactions to foods. The timing and type of the challenge, preparation of patients, foods to be tested, and dosing schedule should be determined on the basis of the patient's history, age, and experience. Although double-blind, placebo-controlled food challenges(DBPCFC) are used to establish definitively if a food is the cause of adverse reactions, they are time-consuming, expensive and troublesome for physician and patients. In practice, An open challenge controlled by trained personnel is sufficient especially in infants and young children. The interpretation of the results and follow-up after a challenge are also important. Since theses challenges are relatively safe and informative, controlled oral food challenges could become the measure of choice in children.
Appointments and Schedules ; Child ; Diet ; Follow-Up Studies ; Food Hypersensitivity ; Humans ; Hypersensitivity ; Immunoglobulin E ; Infant

BACKGROUND/AIMS: Fabry disease is an X-linked recessive and progressive disease caused by alpha-galactosidase A (alpha-GaL A) deficiency. We sought to assess the prevalence of unrecognized Fabry disease in dialysis-dependent patients and the efficacy of serum globotriaosylceramide (GL3) screening. METHODS: A total of 480 patients of 1,230 patients among 17 clinics were enrolled. Serum GL3 levels were measured by tandem mass spectrometry. Additionally, we studied the association between increased GL3 levels and cardiovascular disease, cerebrovascular disease, or left ventricular hypertrophy. RESULTS: Twenty-nine patients had elevated serum GL3 levels. The alpha-GaL A activity was determined for the 26 patients with high GL3 levels. The mean alpha-GaL A activity was 64.6 nmol/hr/mg (reference range, 45 to 85), and no patient was identified with decreased alpha-GaL A activity. Among the group with high GL3 levels, 15 women had a alpha-GaL A genetics analysis. No point mutations were discovered among the women with high GL3 levels. No correlation was observed between serum GL3 levels and alpha-GaL A activity; the Pearson correlation coefficient was 0.01352 (p = 0.9478). No significant correlation was observed between increased GL3 levels and the frequency of cardiovascular disease or cerebrovascular disease. CONCLUSIONS: Fabry disease is very rare disease in patients with end-stage renal disease. Serum GL3 measurements as a screening method for Fabry disease showed a high false-positive rate. Thus, serum GL3 levels determined by tandem mass spectrometry may not be useful as a screening method for Fabry disease in patients with end stage renal disease.
Adult ; Aged ; Fabry Disease/blood/*diagnosis ; Female ; Humans ; Kidney Failure, Chronic/blood/*therapy ; Male ; Middle Aged ; *Renal Dialysis ; Trihexosylceramides/*blood ; alpha-Galactosidase/genetics/metabolism