No abstract available.
Anaphylaxis* ; Basophils*

PURPOSE: Chronic urticaria (CU) has a substantial impact on the quality of life. Little clinical data on the prognosis of CU has been reported. This study aimed to investigate the control status and remission rate of CU and to explore potential predictors of good responses to the treatment during a 6-month treatment period. METHODS: A total of 75 patients with chronic spontaneous urticaria (CSU) were enrolled from 3 university hospitals in Korea. Urticaria control state was classified into 2 groups: group I (remission and well-controlled) and group II (partly and uncontrolled). CU-specific quality of life (CU-QoL) and the urticaria activity score (UAS) were measured before and after the treatment. Autologous serum skin test (ASST), and anti-nuclear and anti-thyroid antibodies were measured at the enrollment into the study. Aspirin intolerance was confirmed by an oral provocation test. RESULTS: Of 59 patients completing the study, 21 (35.6%) arrived at well-controlled status and only 2 (3.4%) achieved remission, whereas 26 (44.1%) remained at partly controlled status and 10 (16.9%) were at uncontrolled status. Mean changes in CU-QoL (36.5+/-2.7 vs 20.6+/-4.3, P=0.017) and UAS (-7.9+/-0.8 vs -3.0+/-1.0, P=0.001) were significantly different between groups I and II. The presence of serum autoantibodies and aspirin intolerance had no influence on the control of urticaria in this study. However, ASST positivity was identified as a significant predictor of CU control in multivariate analysis (OR=6.106, P=0.017). CONCLUSIONS: The proportion of CSU patients that achieved remission or a well-controlled state was 39% for the 6 months of stepwise treatment. Longer observations are necessary to assess the exact prognosis of CSU. ASST results may be a useful parameter for predicting a better response to treatment and both UAS and CU-QoL are helpful to monitor therapeutic response.
Antibodies ; Aspirin ; Autoantibodies ; Hospitals, University ; Humans ; Korea ; Multivariate Analysis ; Observational Study* ; Prognosis ; Prospective Studies* ; Quality of Life ; Skin Tests ; Urticaria*

Anaphylaxis is a severe and life-threatening systemic reaction. Despite the extensive evaluation to determine the cause, 30%-60% of cases of anaphylaxis in adults remain idiopathic. Recently, omalizumab treatment has been postulated to treat refractory idiopathic anaphylaxis. We report a case of idiopathic anaphylaxis treated with omalizumab and investigated its pharmacological mechanism. A 66-year-old female presented to our clinic with recurrent anaphylaxis. She suffered from anaphylaxis 2-3 times a month for 6 months. She had past medical history of nonallergic bronchial asthma. History was carefully undertaken and anaphylaxis was not related to any specific foods, drugs, exercise, and insect bites. Serum specific IgE antibodies to common food allergens showed negative results. Oral provocation tests to food additives revealed to be negative. To screen systemic mastocytosis and mast cell activating syndrome, baseline tryptase level was checked, and it was within normal range. From comprehensive evaluation, she was diagnosed as having idiopathic anaphylaxis. She could not tolerate oral medications due to gastrointestinal discomfort, therefore, omalizumab treatment (150 mg, monthly) was started. After 6 months of treatment, anaphylaxis did not occur with complete remission status. To evaluate the pharmacological mechanism of omalizumab treatment, basophil histamine releasability test was performed. Histamine releasability induced by anti-IgE did not change after 6 months of treatment, while that induced by calcium inophore decreased. Omalizumab treatment can induce remission or favorable effects on idiopathic anaphylaxis, which may be derived from increased threshold of mast cell degranulation. Long-term studies in a larger cohort will be needed to confirm its efficacy.
Adult ; Aged ; Allergens ; Anaphylaxis* ; Antibodies ; Asthma ; Basophils ; Calcium ; Cohort Studies ; Female ; Food Additives ; Histamine ; Humans ; Immunoglobulin E ; Insect Bites and Stings ; Mast Cells ; Mastocytosis, Systemic ; Reference Values ; Tryptases ; Omalizumab

This erratum is being published to correct of author name.

Various foods can induce anaphylaxis. However, mushrooms-induced anaphylaxis has not been reported in Korea. We report a patient with past anaphylactic episode caused by mushroom ingestion, confirmed by the skin test and specific IgE antibody to mushrooms. A 17-year-old girl with asthma was referred to our department due to itchy throat, dyspnea, and urticaria within 10 minutes after ingestion of a soup containing Oyster mushrooms. She presented an itching throat after ingestion of cooked mushrooms 3 years before the visit. She had an elevated serum IgE level (205 kU/L) and was sensitized to house dust mites. Skin prick tests with mushroom extracts showed a strong positive on Oyster and King Oyster mushrooms as well as Pyogo mushroom. The specific IgE antibody to each mushroom measured by enzyme-linked immunosorbent assay showed significant positive results to Oyster and Pyogo mushroom extracts, but was negative on King Oyster mushroom. We educated her to avoid eating Oyster and Pyogo mushrooms for preventing recurrence, whereas we couldn't perform oral challenge tests.
Adolescent ; Agaricales* ; Anaphylaxis* ; Asthma ; Dyspnea ; Eating* ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Immunoglobulin E ; Korea ; Ostreidae ; Pharynx ; Pleurotus ; Pruritus ; Pyroglyphidae ; Recurrence ; Skin ; Skin Tests ; Urticaria

Kartagener syndrome is characterized by the triad of situs inversus, bronchiectasis, and chronic paranasal sinusitis. Recurrent sinopulmonary infection, the major determinant for diagnosing immunodeficiency, is the most common clinical manifestation of the disease. A 17-year-old female patient presented with dyspnea, cough, sputum, nasal congestion, and rhinorrhea for more than 5 years. Nasal symptoms and dyspnea had not been controlled by intermittent treatment with mucolytics and antibiotics from primary clinics since 3 months before visiting our clinic. Chest X-ray and computed tomography showed situs inversus, dextrocardia and bronchiectasis. Paranasal sinus series revealed mucosal thickening and haziness on both maxillary sinus. Serum immunoglobulin (Ig) G4 was decreased, but total IgG was within normal range. Under the diagnosis of Kartagener syndrome with IgG4 deficiency, monthly intravenous IgG (IVIG) treatment was performed for 6 months. Her symptoms were well controlled and the frequency of antibiotics use was markedly decreased. We report a patient having the Kartagener syndrome with IgG4 deficiency that was successfully controlled with a 6-month-treatment of IVIG.
Adolescent ; Anti-Bacterial Agents ; Bronchiectasis ; Cough ; Dextrocardia ; Dyspnea ; Estrogens, Conjugated (USP) ; Expectorants ; Female ; Humans ; Immunoglobulin G ; Immunoglobulins ; Immunoglobulins, Intravenous ; Kartagener Syndrome ; Maxillary Sinus ; Reference Values ; Sinusitis ; Situs Inversus ; Sputum ; Thorax

PURPOSE: Allergic rhinitis (AR) is a common and increasing disease in which Dermatophagoides (D.) farinae is one of the most common causative allergens. The aims of this study were to confirm the presence of locally produced antibodies to D. farinae in nasal secretions between nasal provocation test (NPT)-positive and -negative groups of AR patients, to evaluate their relationships with the levels of inflammatory mediators, and to determine adaptive and innate immune responses in nasal mucosa. METHODS: Sixty AR patients sensitive to house dust mites confirmed by skin prick test or serum specific IgE to D. farinae underwent NPT for D. farinae. Nasal packs were placed in both nasal cavities of the patients for 5 minutes to obtain nasal secretions after NPT. The levels of total IgE, specific IgE to D. farinae, eosinophil cationic protein (ECP), and tryptase in nasal secretions were detected by using ImmunoCAP. The levels of specific IgE, IgA, and secretory IgA antibodies to D. farinae in nasal secretions were measured by using ELISA. The levels of IL-8, VEGF, IL-25, and IL-33 were also measured by using ELISA. RESULTS: High levels of total IgE, specific IgE, specific IgA, and secretory IgA to D. farinae, as well as inflammatory mediators, such as ECP, IL-8, VEGF and tryptase, were detected in nasal secretions, although the differences were not statistically significant between the NPT-positive and NPT-negative groups. Levels of all immunoglobulins measured in this study significantly correlated with ECP, IL-8, and VEGF (P<0.05), but not with tryptase (P>0.05). IL-33 and IL-25 were also detected, and IL-25 level significantly correlated with IL-8 (r=0.625, P<0.001). CONCLUSIONS: These findings confirmed the presence of locally produced specific antibodies, including D. farinae-specific IgE and IgA, in nasal secretions collected from D. farinae-sensitive AR patients in both the NPT-positive and NPT-negative groups, and close correlations were noted between antibodies and nasal inflammatory mediators, including such as ECP, IL-8 and VEGF, indicating that locally produced antibodies may be involved in the nasal inflammation of AR.
Allergens ; Antibodies* ; Enzyme-Linked Immunosorbent Assay ; Eosinophil Cationic Protein ; Humans ; Immunity, Innate ; Immunoglobulin A ; Immunoglobulin A, Secretory ; Immunoglobulin E ; Immunoglobulins ; Inflammation ; Interleukin-8 ; Nasal Cavity ; Nasal Mucosa ; Nasal Provocation Tests ; Pyroglyphidae ; Rhinitis* ; Skin ; Tryptases ; Vascular Endothelial Growth Factor A

PURPOSE: To understand the incidence of retinopathy of prematurity (ROP) in preterm infants with birth weights more than 1,500 g or gestational age 30 weeks, and/or unstable clinical course, we investigated the highest gestational age and birth weight of preterm infants who require ROP treatment and those who do not. METHODS: The subjects were preterm infants admitted in Samsung medical center between January 1, 2000 and December 31, 2013. We retrospectively reviewed the medical records of 847 premature infants whose birth weights were more than 1,000 g. RESULTS: Of the 847 infants, 105 (12.4%) had stage 1 ROP, 54 (6.4%) had stage 2, 31 (3.7%) had stage 3, 0 had stage 4, and 2 (0.2%) had stage 5 ROP. Thirty-three (3.9%) of the 847 infants developed stage 3-5 ROP. Twenty (2.4%) of these 33 (3.9%) stage 3-5 ROP infants required treatment. Among the stage 1-3 ROP infants who did not require treatment, the highest gestational age was 37(+1) weeks (stage 1) and birth weight was 2,362 g (stage 1). Among the stage 3-5 ROP infants who needed treatment, the highest gestational age was 32 weeks and birth weight was 1,495 g. CONCLUSIONS: Newborn infants with gestational age more than 38 weeks or birth weight more than 2,400 g did not develop ROP even if they had an unstable clinical course. In our study, no preterm infants with gestational age more than 33 weeks or birth weight more than 1,500 g required ROP treatment.
Birth Weight* ; Gestational Age ; Humans ; Incidence ; Infant* ; Infant, Newborn ; Infant, Premature ; Medical Records ; Retinopathy of Prematurity* ; Retrospective Studies

Good's syndrome (GS) is a rare cause of acquired combined B- and T-cell immunodeficiency in adults. GS is also known as a rare form of paraneoplastic syndrome of thymoma that may persist after thymectomy. Some reported GS cases have been related to various diseases as a result of humoral immunodeficiency, but no report to date has related GS to cytomegalovirus (CMV) retinitis in Korea. We report two cases of CMV retinitis as a manifestation of GS with severe cellular immunodeficiency. In the first case, a 61-year-old woman was diagnosed with GS manifesting as CMV retinitis combined with coincident pulmonary tuberculosis and soft-tissue tuberculosis. In the second case, a 56-year-old man had CMV retinitis and CMV pneumonia. Both patients had a history of thymoma and had received total thymectomies, and were diagnosed with GS a few years thereafter.
Adult ; Cytomegalovirus ; Cytomegalovirus Retinitis ; Female ; Humans ; Immunologic Deficiency Syndromes ; Korea ; Middle Aged ; Paraneoplastic Syndromes ; Pneumonia ; Retinitis ; T-Lymphocytes ; Thymectomy ; Thymoma ; Tuberculosis ; Tuberculosis, Pulmonary

BACKGROUND/AIMS: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are predominantly known as medication-induced diseases. However, at our institution, we have experienced more cases of non-drug-related SJS and TEN than expected. Therefore, we studied the difference between non-drug-related and drug-related SJS and TEN in terms of clinical characteristics and prognoses. METHODS: The etiologies, clinical characteristics, and treatment outcomes for 82 adult patients with SJS and TEN were retrospectively reviewed. RESULTS: A total of 71 patients (86.6%) were classified as having SJS, and the other 11 patients (13.4%) were classified as having TEN. Drug-related cases were more common (43, 52.4%) than non-drug-related cases (39, 47.6%). Anticonvulsants (12/82, 14.6%) and antibiotics (9/82, 11%) were the most common causative medications. Anemia (p = 0.017) and C-reactive protein of > or = 5 mg/dL (p = 0.026) were more common in the drug-related cases than in the non-drug-related cases. Intravenous steroid therapy was used as the main treatment regimen (70/82, 85.4%). Of the 82 patients, 8 (9.8%) died during the clinical course. A univariate analysis for mortality showed statistical significance for the following: kidney function abnormality, pneumonia, hemoglobin of < 10 g/dL, and combined underlying diseases. In a multivariate analysis, only pneumonia was statistically significant (odds ratio, 25.79; p = 0.009). CONCLUSIONS: Drugs were the most frequent cause of these diseases. However, non-drug-related causes also contributed to a significant proportion of cases. Physicians should keep this in mind when documenting patient history. In addition, early recognition and treatment may be important for better outcomes.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Chi-Square Distribution ; Epidermal Necrolysis, Toxic/diagnosis/*etiology/mortality/*therapy ; Female ; Humans ; Logistic Models ; Male ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Republic of Korea ; Risk Assessment ; Risk Factors ; Stevens-Johnson Syndrome/chemically induced/diagnosis/*etiology/mortality/*therapy ; Survival Analysis ; Treatment Outcome ; Young Adult