Hyper-IgE syndrome (HIES) is a very rare primary immune deficiency characterized by elevated serum IgE levels, recurrent bacterial infections, chronic dermatitis, and connective tissue abnormalities. Autosomal dominant (AD) HIES involves a mutation in signal transducer and activator of transcription 3 (STAT3) that leads to an impaired T(H)17 response. STAT3 signaling is also involved in the function of RORγt⁺ type 3 innate lymphoid cells (ILC3s) and RORγt⁺T(H)17 cells. The aim of this study was to investigate the role of innate immune cells such as innate lymphoid cells (ILCs), granulocytes, and monocytes in a patient with HIES. Peripheral blood mononuclear cells (PBMCs) from a patient with HIES and three age-matched healthy controls were obtained for the analysis of the innate and adaptive immune cells. The frequencies of ILCs in PBMCs were lower in the patient with HIES than in the controls. Moreover, granulocyte-macrophage colony-stimulating factor (GM-CSF) and IL-17A produced by ILC3s in PBMCs were lower in the patient with HIES than the controls. Compared with the controls, classical monocytes (CD14⁺CD16(low)), which have a high antimicrobial capability, were also lower in the patient with HIES, while non-classical monocytes (CD14(low)CD16⁺) as well as intermediate monocytes (CD14⁺CD16(intermediate)) were higher. Taken together, these results indicate that the impaired immune defense against pathogenic microbes in the patient with HIES might be partially explained by functional defects in ILC3s and inflammatory monocytes.
Bacterial Infections ; Connective Tissue ; Cytokines ; Dermatitis ; Granulocyte-Macrophage Colony-Stimulating Factor ; Granulocytes ; Humans ; Immunity, Innate ; Immunoglobulin E ; Interleukin-17 ; Job Syndrome* ; Lymphocytes* ; Monocytes* ; STAT3 Transcription Factor

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare but potentially fatal drug-induced systemic hypersensitivity response characterized by erythematous eruption, fever, leukocytosis with eosinophilia, and internal organ involvement. Antitubercular agents are potential causative agents for DRESS syndrome but difficult to verify as a culprit drug, since antitubercular agents are coadministered as a combination regimen. A 42-year-old female with endobronchial tuberculosis was diagnosed with DRESS syndrome after 4-week treatment of isoniazid, rifampicin, ethambutol, and pyrazinamide with prednisolone 50 mg. All the antitubercular agents were stopped and replaced with levofloxacin, cycloserine, p-aminosalicylic acid, and kanamycin. However, severe exacerbation of DRESS syndrome compelled the patient to discontinue the administration of the second-line antitubercular agents. Two months later, the patient underwent a patch test for all the antitubercular agents which had been used, and the results showed positivity to isoniazid and cycloserine. We report a rare case of DRESS syndrome that reacted to cycloserine as well as isoniazid. Development of coreactivity to other drugs should be differentiated with a flare-up reaction in the management of DRESS syndrome.
Adult ; Aminosalicylic Acid ; Antitubercular Agents ; Cycloserine ; Drug Hypersensitivity Syndrome ; Eosinophilia* ; Ethambutol ; Female ; Fever ; Humans ; Hypersensitivity ; Isoniazid ; Kanamycin ; Leukocytosis ; Levofloxacin ; Patch Tests ; Prednisolone ; Pyrazinamide ; Rifampin ; Tuberculosis

In this paper, some parts of Fig. 1 and dose information of methylprednisolone on page 113 were misprinted.

BACKGROUND: Reference intervals need to be established according to age. We established reference intervals of hematology and chemistry from community-based healthy 1-yr-old children and analyzed their iron status according to the feeding methods during the first six months after birth. METHODS: A total of 887 children who received a medical check-up between 2010 and 2014 at Boramae Hospital (Seoul, Korea) were enrolled. A total of 534 children (247 boys and 287 girls) were enrolled as reference individuals after the exclusion of data obtained from children with suspected iron deficiency. Hematology and clinical chemistry analytes were measured, and the reference value of each analyte was estimated by using parametric (mean±2 SD) or nonparametric methods (2.5-97.5th percentile). Iron, total iron-binding capacity, and ferritin were measured, and transferrin saturation was calculated. RESULTS: As there were no differences in the mean values between boys and girls, we established the reference intervals for 1-yr-old children regardless of sex. The analysis of serum iron status according to feeding methods during the first six months revealed higher iron, ferritin, and transferrin saturation levels in children exclusively or mainly fed formula than in children exclusively or mainly fed breast milk. CONCLUSIONS: We established reference intervals of hematology and clinical chemistry analytes from community-based healthy children at one year of age. These reference intervals will be useful for interpreting results of medical check-ups at one year of age.
Breast Feeding ; Clinical Chemistry Tests/*standards ; Female ; Hematologic Tests/*standards ; Humans ; Infant ; Iron/*blood/standards ; Male ; Reference Values ; Republic of Korea

Blueberry muffin rashes occur in various diseases including TORCH syndrome, transfusion reactions, leukemia, hereditary spherocytosis and neonatal sepsis. We report a case of congenital CMV(cytomegalovirus) infection showing blueberry muffin skin lesions which revealed dermal erythropoiesis. Even though these cutaneous findings were nonspecific, they could provide a valuable clue in approach the congenital viral infection in the perinatal period.
Blueberry Plant ; Erythropoiesis* ; Exanthema ; Leukemia ; Sepsis ; Skin* ; Transfusion Reaction

BACKGROUND: There are some previous reports about partial D in Korea. However, the frequency of the partial D in Korea is still unknown. In this study, subtypes of partial D were analyzed by the use of various commercially available anti-D reagents. METHODS: We collected 273 cases determined as RhD negative by RhD typing using the tube method with monoclonal IgM/IgG anti-D reagent (Bioscot. Livingston, UK) from 80,062 cases that were screened between January 2004 and August 2007. The cases were divided into three periods (I, II, III), according to the manufacturers and numbers of anti-D reagents used. A weak D test was performed by using the tube method with various anti-D reagents. The cases with different reactivity between anti-D reagents were determined as partial D, and further analyzed the subtypes by reactivity patterns according to the target epitope of anti-D reagents. An additional test using the ID-Partial D Typing Card (DiaMed, Cressier, Switzerland) was conducted during period III. RESULTS: Five cases showed reactivity patterns of weak D and 16 cases showed reactivity patterns of partial D. Ten cases of partial D were typed as DVI and three cases were typed as DFR. During period III, five cases were typed as DVI and one case was typed as DFR. These results were different from the results obtained with the use of the ID-Partial D Typing Card. CONCLUSION: DVI, which is the most common subtype of partial D, is also common in Korea. Therefore, RhD typing and a weak D test should be performed using combined anti-D reagents that enable the differentiation of DVI from other subtypes.
Indicators and Reagents* ; Korea

PURPOSE: To evaluate intracranial control after surgical resection according to the adjuvant treatment received in order to assess the optimal radiotherapy (RT) dose and volume. MATERIALS AND METHODS: Between 2003 and 2015, a total of 53 patients with brain oligometastases from non-small cell lung cancer (NSCLC) underwent metastasectomy. The patients were divided into three groups according to the adjuvant treatment received: whole brain radiotherapy (WBRT) ± boost (WBRT ± boost group, n = 26), local RT/Gamma Knife surgery (local RT group, n = 14), and the observation group (n = 13). The most commonly used dose schedule was WBRT (25 Gy in 10 fractions, equivalent dose in 2 Gy fractions [EQD2] 26.04 Gy) with tumor bed boost (15 Gy in 5 fractions, EQD2 16.25 Gy). RESULTS: The WBRT ± boost group showed the lowest 1-year intracranial recurrence rate of 30.4%, followed by the local RT and observation groups, at 66.7%, and 76.9%, respectively (p = 0.006). In the WBRT ± boost group, there was no significant increase in the 1-year new site recurrence rate of patients receiving a lower dose of WBRT (EQD2) <27 Gy compared to that in patients receiving a higher WBRT dose (p = 0.553). The 1-year initial tumor site recurrence rate was lower in patients receiving tumor bed dose (EQD2) of ≥42.3 Gy compared to those receiving <42.3 Gy, although the difference was not significant (p = 0.347). CONCLUSIONS: Adding WBRT after resection of brain oligometastases from NSCLC seems to enhance intracranial control. Furthermore, combining lower-dose WBRT with a tumor bed boost may be an attractive option.
Appointments and Schedules ; Brain* ; Carcinoma, Non-Small-Cell Lung ; Humans ; Lung Neoplasms* ; Lung* ; Metastasectomy ; Neoplasm Metastasis ; Radiotherapy* ; Radiotherapy, Adjuvant ; Recurrence ; Retrospective Studies*

PURPOSE: The purpose of this study was to compare the clinical and functional outcomes in patients with primary base of tongue (BOT) cancer who received definitive radiotherapy (RT) or surgery followed by radiotherapy (SRT). MATERIALS AND METHODS: Between January 2002 and December 2016, 102 patients with stage I-IVB primary BOT cancer underwent either definitive RT (n=46) or SRT (n=56), and treatment outcomes were compared between two groups. The expression of p16 was also analyzed. RESULTS: The RT group had more patients with advanced T stage (T3-4) disease (58.7% vs. 35.7%, p=0.021) and who received chemotherapy (91.3% vs. 37.5%, p < 0.001) than the SRT group. At a median follow up of 36.9 months (range, 3.3 to 181.5 months), the 5-year overall survival (OS) and disease-free survival (DFS) were 75.5% and 68.7%, respectively. With respect to treatment group, the 5-year OS and DFS in the RT and SRT groups did not differ significantly (OS, 68.7% vs. 80.5%, p=0.601; DFS, 63.1% vs. 73.1%, p=0.653). In multivariate analysis, OS differed significantly according to p16 expression (p16-negative vs. p16-positive; hazard ratio [HR], 0.145; 95% confidence interval [CI], 0.025 to 0.853; p=0.033). Regarding DFS, p16 expression (p16-negative vs. p16-positive; HR, 0.164; 95% CI, 0.045 to 0.598; p=0.006) showed a significant effect in multivariate analysis. Functional defects (late grade ≥ 3 dysphagia or voice alteration) were more frequently reported in the SRT than in the RT group (16.1% vs. 2.2%, p=0.021). CONCLUSION: Despite advanced disease, patients in the RT group showed comparable survival outcomes and better functional preservation than those in the SRT group.
Chemoradiotherapy* ; Deglutition Disorders ; Disease-Free Survival ; Drug Therapy ; Follow-Up Studies ; Humans ; Multivariate Analysis ; Organ Preservation ; Radiotherapy ; Radiotherapy, Adjuvant* ; Tongue Neoplasms* ; Tongue* ; Treatment Outcome ; Voice

Occupational asthma is induced by many agents, including herbal materials, that are exposed in working places. Although there are a few case reports for occupational allergy induced by herbal materials, there is none for that induced by Wonji (Polygala tenuifolia). This study was conducted to evaluate clinical characteristics and immunologic mechanism of Wonji-induced asthma in a exposed-worker. A patient who complained of asthma and rhinitis symptoms, and who had worked in a herbal manufacturing factory for 8 yr, underwent a skin prick test with crude extract of Wonji under the impression of occupational asthma induced by the agent. The patient had a strong positive response to the extract on the skin prick test. Allergen bronchial challenge to the extract demonstrated a typical dual response. Serum specific IgE level to the extract was higher in the patient than in healthy controls, and ELISA inhibition test revealed complete inhibition of IgE binding with the extract, but no inhibition with Der p 2 or mugwort extracts. Six IgE binding components to the extract (10, 25, 28, 36, 50, and 90 kDa) were detected using SDS-PAGE and immunoblot analysis. These findings suggest that Polygala tenuifolia, a herbal material, can induce IgE-mediated bronchoconstriction in exposed workers.
Allergens ; Asthma/*chemically induced ; Dose-Response Relationship, Drug ; Electrophoresis, Polyacrylamide Gel ; Enzyme-Linked Immunosorbent Assay ; Humans ; Immunoblotting ; Immunoglobulin E/chemistry ; Male ; *Medicine, Herbal ; Middle Aged ; Occupational Exposure ; Pollen/chemistry ; Polygala/metabolism ; Protein Binding ; Research Support, Non-U.S. Gov't ; Rhinitis/*chemically induced ; Time Factors

PURPOSE: Delayed hypersensitivity reaction can occur in a couple of hours to several days after injection of iodine-based contrast media (ICM). ICM-related delayed type hypersensitivity is not common but increasing as rapid growth of ICM use. Nevertheless, objective data on delayed type hypersensitivity are still scarce worldwide including Korea. This study was performed to investigate the clinical features of ICM-induced delayed hypersensitivity in Korean patients. METHODS: We retrospectively reviewed the electronic medical records of patients diagnosed with delayed hypersensitivity to ICM from January 2009 to December 2012 at Seoul National University Hospital and analyzed the data to identify the clinical characteristics of these patients. RESULTS: A total of 44 cases were diagnosed as delayed-type hypersensitivity to ICM. The mean age was 54 years, and 70.5% were female. The mean number of previous ICM exposure was 3.8, and skin reactions were the most common symptoms. In 45% of patients, hypersensitivity reaction developed on the first exposure to ICM. Among the 27 patients exposed to ICM again, hypersensitivity reactions recurred in only 4 patients (14.8%). There was no difference of recurrence rate according to the use of premedication or the change in ICM. CONCLUSION: In this study, we observed a female predominance and a low recurrence rate in delayed hypersensitivity to ICM. Premedication and ICM change was not effective in preventing recurrence of delayed type reactions.
Contrast Media* ; Electronic Health Records ; Female ; Humans ; Hypersensitivity* ; Hypersensitivity, Delayed ; Korea ; Premedication ; Recurrence ; Retrospective Studies ; Seoul ; Skin