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.

PURPOSE: Age-based causes and clinical characteristics of immediate-type food allergy (FA) have not been sufficiently studied. Therefore, we investigated age-dependent clinical profiles of FA in Korean children through an extensive multicenter investigation. METHODS: Using a case report form developed by the authors, a retrospective medical record review was performed of patients (0-18 years old) diagnosed with immediate-type FA between September 2014 and August 2015 in 14 tertiary hospitals in Korea. RESULTS: A total of 1,353 children and adolescents, 93% younger than 7 years, were enrolled in the present study, and 1,661 cases of immediate-type FA were recorded in these patients. The 7 major causative foods were cow's milk (28.1%), hen's eggs (27.6%), wheat (7.9%), walnuts (7.3%), peanuts (5.3%), buckwheat (1.9%), and shrimps (1.9%). Categorizing the patients into 4 age groups revealed that the most common causative food was different for each age group: cow's milk (<2 years), walnuts (2–6 years), walnuts (7–12 years), and buckwheat (13-18 years). The onset time of symptoms was less than 10 minutes in 49%, between 10 and 30 minutes in 17%, and between 30 minutes and 2 hours in 34% of cases. Food-induced anaphylaxis was reported in 506 (30.5%) out of 1,661 cases, and the 7 major causes of food-induced anaphylaxis was cow's milk (27.5%), hen's eggs (21.9%), wheat (11.3%), walnuts (10.5%), peanuts (5.9%), buckwheat (4.2%), and pine nuts (3.0%). The proportion of anaphylaxis was highest in the patients allergic to buckwheat (67.7%), followed by those allergic to pine nuts (57.7%), walnuts (43.8%), wheat (43.5%), and peanuts (34.1%). CONCLUSIONS: The 5 major causative foods of immediate-type FA in Korean children were cow's milk, hen's eggs, wheat, walnuts, and peanuts. The distribution of causative foods was considerably distinctive according to different age groups. Anaphylaxis was reported in 30.5% of immediate-type FA cases.
Adolescent ; Anaphylaxis ; Arachis ; Child* ; Eggs ; Fagopyrum ; Food Hypersensitivity* ; Humans ; Juglans ; Korea ; Medical Records ; Milk ; Nuts ; Ovum ; Retrospective Studies ; Tertiary Care Centers ; Triticum

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

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: In this study, we used high-resolution DNA typing to investigate the distribution of HLA alleles and haplotypes in Koreans. METHODS: HLA-A, -B, -C, and -DRB1 alleles were genotyped at the allelic (4-digit) level in 474 healthy Koreans. HLA genotyping was performed in two steps. Initially, serologic typing or generic-level DNA typing was performed using the PCR-sequence-specific oligonucleotide method, and then allelic DNA typing (exons 2 and 3 for class I, and exon 2 for DRB1) was carried out using the PCR-single-strand conformation polymorphism method or sequence-based typing. HLA allele and haplotype frequencies and linkage disequilibrium values were calculated by the maximum likelihood method using a computer program developed for the 11th International Histocompatibility Workshop. RESULTS: A total of 21 HLA-A, 40 HLA-B, 22 HLA-C, and 29 HLA-DRB1 alleles were found in Koreans. The most frequent alleles in each locus with frequencies of > or =10% were, in decreasing order of frequency, as follows: A*24:02, A*02:01, A*33:03; B*51:01; C*01:02, C*03:03; and DRB1*09:01. The numbers of two- and three-locus haplotypes with frequencies of >0.5% were as follows: 44 A-C, 42 B-C, 51 A-B, 52 B-DRB1, 42 A-C-B, and 34 A-B-DRB1. Thirteen A-B-DRB1 haplotypes with frequencies of > or =1.0% comprised 26.0% of the total haplotypes. The six most common haplotypes were as follows: A*33:03-B*44:03-DRB1*13:02 (3.7%), A*33:03-B*44:03-DRB1*07:01 (3.0%), A*33:03-B*58:01-DRB1*13:02 (3.0%), A*24:02-B*07:02-DRB1*01:01 (2.8%), A*30:01-B*13:02-DRB1*07:01 (2.3%), and A*11:01-B*15:01-DRB1*04:06 (2.2%). CONCLUSIONS: The information obtained in this study can be used as basic data for Koreans in the fields of organ transplantation, disease association, and anthropologic studies.
Alleles ; Asian Continental Ancestry Group/*genetics ; DNA Fingerprinting/methods ; Gene Frequency ; Genetic Variation ; Genotype ; HLA-A Antigens/*genetics ; HLA-B Antigens/*genetics ; HLA-C Antigens/*genetics ; HLA-DR Antigens/*genetics ; Haplotypes ; Humans ; Republic of Korea

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

Polymorphisms of the prion protein gene (PRNP) havebeen detected in several cervid species. In order toconfirm the genetic variations, this study examined theDNA sequences of the PRNP obtained from 33 captivesika deer (Cervus nippon laiouanus) in Korea. A total ofthree single-nucleotide polymorphisms (SNPs) at codons100, 136 and 226 in the PRNP of the sika deer wereidentified. The polymorphic site located at codon 100 hasnot been reported. The SNPs detected at codons 100 and226 induced amino acid substitutions. The SNP at codon136 was a silent mutation that does not induce any aminoacid change. The genotype and allele frequencies weredetermined for each of the SNPs.
Animals ; Base Sequence ; DNA/chemistry/genetics ; Deer/*genetics ; Genetic Variation ; Molecular Sequence Data ; Polymerase Chain Reaction/veterinary ; Polymorphism, Single Nucleotide ; Prions/*genetics ; Sequence Analysis, DNA

PURPOSE: To establish the feasibility of high dose ifosfamide, carboplatin, and etoposide (ICE) chemotherapy followed by autologous stem cell transplantation (ASCT) in patients with high-risk or metastatic breast cancer. MATERIALS AND METHODS: High-risk breast cancer is defined as 10 or more involved axillary lymph nodes (n=3) or stage III (n=2). Patients with metastatic cancer have relapsed diseases after curative resection (n=10) or initially metastatic lesion (n=1). Colony stimulating factor with either cyclophosphamide or combination chemotherapy was administered to mobilize the stem cells. High dose chemotherapy consisted of ifosfamide 16 g/m2, carboplatin 1.8 g/m2, and etoposide 0.75 g/m2 (dose I) and later modified to ifosfamide 12 g/m2, carboplatin 1.35 g/m2, and etoposide 1.2 g/m2 (dose II). RESULTS: The median duration of grunulocyte nadir (<500/ microliter) was 11 (10~17) days and platelet transfusion dependency (<20,000/ microliter) was 11 (7~53) days in 14 patients who achieved engraftment. One out of 5 patients with high-risk breast cancer relapsed after high dose therapy. Two patients remain disease-free at 18th and 40th months. Two among the 4 patients treated with dose I died due to treatment-related complications. The responses of metastatic diseases to ICE chemotherapy were 1 continuing CR, 1 CR, 1 PR, 4 SD and 3 PD in 10 evaluable patients. CONCLUSION: High dose ICE chemotherapy, especially dose II and ASCT were feasible in high-risk or metastatic breast cancer.
Breast Neoplasms* ; Breast* ; Carboplatin* ; Colony-Stimulating Factors ; Cyclophosphamide ; Drug Therapy* ; Drug Therapy, Combination ; Etoposide* ; Humans ; Ice ; Ifosfamide* ; Lymph Nodes ; Platelet Transfusion ; Stem Cell Transplantation* ; Stem Cells*

In the present studies, changes of the glial fibrillary acidic protein (GFAP) expression in the astrocytes of the rat hippocampal formation were examined in response to the bilateral carotid artery occlusion for 10 minutes along with a decrease of mean arterial blood pressure (MABP) to 50 mmHg. Their relations to neuronal viability were also studied by H&E staining. In early postischemic period, mild increase of the GFAP expression was observed and this was not only confined to the mild-necrotic (CA3 and dentate gyrus) regions but also in the non-necrotic regions (CA1 and subiculum) at postischemic 8 h. This suggest that astrocytosis during early postischemic period may be resulted from nonspecific reaction associated with changes in brain environment. In contrast, in late phase of the postischemia, a marked increase of the GFAP expression was observed at day 4. Moreover, cell bodies were significantly larger and many prominent and numerous processes were observed, suggesting that this may also contribute to the significant increase in the GFAP expression. Importantly, these cellular changes were only confined to the regions of massive necrosis such as subiculum and inner granular cell layer of dentate gyrus and were not observed in the non-necrotic regions (except CA1). In contrast, the GFAP expression in astrocytes were returned to control levels in mildly damaged CA3 region by 4 days. Thus reactive astrocytosis with upregulation of the GFAP in the late postischemic period with structural transformation in the regions of massive necrosis may contribute to the damages in the neighboring neurons.
Animals ; Arterial Pressure ; Astrocytes ; Brain ; Carotid Arteries ; Dentate Gyrus ; Glial Fibrillary Acidic Protein ; Gliosis* ; Hippocampus* ; Necrosis ; Neurons ; Rats* ; Up-Regulation

There is a controversy in patella retention or resurfacing in total knee arthroplasty (TKA) till today. Authors studied 52 cases of 45 patients recieved total knee arthroplasty with patella retention (Group 1, twenty-five cases) or patella resurfacing (Group 2, twenty-seven cases) in patients with osteoarthritis. All operations were done by senior surgeon and the implants used were LCS type (33 cases), AMK type (12 cases), Genesis type (5 cases) and Tricon M type (2 cases). The indications of patella retention were small patella, nearly normal articular cartilage, minimal pre-operative patellofemoral pain, poor patellar bone quality and young patients. Patella retention were performed only with LCS prosthesis, which patella groove of the femoral component is deep and anatomical. The operative approaches used were medial parapatella approach in neutral or varus knee and lateral parapatella approch for severe valgus knee and tilting or lateral subluxation of the patella. The mean follow-up period was 24 months (range 12 months to 5 years). The method for clinical evaluation was Hospital for Special Surgery (HSS) knee scoring (consisted of pain, function, range of motion). The method for radiographical evaluation was Knee societys radiologic evaluation system for tibial and femoral alignment, and Keblishs method for patello-femoral congruence. The clinical HSS knee score was average 90.9 points in both groups, and 91.9 points in group 1, 90.0 points in group 2, and there was no statistically difference in HSS knee score between the two groups (P<0.05). The radiologic results had no significant difference in alignment and patello-femoral congruence between the two groups (P<0.05). But the complications were one case of patella fracture, one patella tendon rupture, one anterior instability and two cases of infection in group 2. In conclusion, the results of both groups were satisfactory. We think that it have relation to strict selection of the patients for patella retension and the use of prosthesis that the patella groove of femoral component is deep and anatomical.
Arthroplasty* ; Cartilage, Articular ; Follow-Up Studies ; Humans ; Knee* ; Osteoarthritis ; Patella* ; Patellar Ligament ; Prostheses and Implants ; Rupture