No abstract available.
Anaphylaxis* ; Basophils*

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

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

Kaposi sarcoma in acquired immunodeficiency syndrome (AIDS) is rarely seen in Korea and the incidence has declined in the era of highly active antiretroviral therapy (HAART). Visceral involvement in patients having AIDS with Kaposi sarcoma is more common than in patients infected with non-human immunodeficiency virus. Visceral involvement may be asymptomatic and usuallydoes not require specific therapy. Chemotherapy is usually used in symptomatic or rapidly progressive disease, and interferon- alpha can be applied as an alternative treatment option. Compared to cutaneous Kaposi sarcoma in AIDS, only three cases of gastrointestinal Kaposi sarcoma in patients with AIDS have been reported in Korea, and no experience with interferon therapy for AIDS-associated visceral Kaposi sarcoma has been reported. We report a case of gastrointestinal Kaposi sarcoma in a patient with AIDS who had combined treatment with interferon-alpha and HAART.
Acquired Immunodeficiency Syndrome ; Antiretroviral Therapy, Highly Active ; Ciprofloxacin ; Drug Resistance ; Humans ; Incidence ; Interferon-alpha ; Interferons ; Korea ; Nalidixic Acid ; Sarcoma, Kaposi ; Typhoid Fever ; Viruses

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

BACKGROUND/AIMS: Frequent pathogens of nosocomial meningitis were investigated and the adequacy of empiric antibiotic therapy was assessed. Outcomes of nosocomial meningitis were also evaluated. METHODS: Ninety-one patients, who were diagnosed and treated for nosocomial meningitis at a single tertiary hospital in Daegu, Korea for 10 years, were included. Medical record and electronic laboratory data on the causative pathogens, antibiotics used, and outcomes were retrospectively investigated. RESULTS: Coagulase-negative Staphylococcus (40.9%) was the most common pathogen, followed by Acinetobacter (32.5%). Both were cultured as a single organism in cerebrospinal fluid (CSF). Seventy-eight patients (85.7%) had infections related to external ventricular drains (EVD). The most common empirical antibiotics were extended-spectrum beta-lactam antibiotics plus vancomycin (35/91, 38.6%). Of the 27 patients who had cultured Acinetobacter in CSF, 10 (37%) were given the wrong empirical antibiotic treatment. Seven of the 27 patients (26.9%) with cultured Acinetobacter died, and overall mortality of the 91 patients was 16.5%. In the multivariate analysis, the presence of combined septic shock (p < 0.001) and a persistent EVD state (p = 0.021) were associated with a poor prognosis. CONCLUSIONS: Acinetobacter is one of the leading pathogens of nosocomial meningitis and may lead to inadequate coverage of empiric antibiotic therapy due to increasing resistance. An EVD should be removed early in cases of suspected nosocomial meningitis, and carbapenem might be required for the poor treatment response.
Acinetobacter/classification/*isolation & purification ; Acinetobacter Infections/cerebrospinal fluid/diagnosis/*drug therapy/*microbiology ; Adult ; Aged ; Aged, 80 and over ; Anti-Bacterial Agents/*therapeutic use ; Cerebrospinal Fluid/microbiology ; Cross Infection/cerebrospinal fluid/diagnosis/*microbiology/mortality/*therapy ; Drug Resistance, Bacterial ; Female ; Humans ; Logistic Models ; Male ; Meningitis, Bacterial/cerebrospinal fluid/diagnosis/*drug therapy/*microbiology/mortality ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Republic of Korea ; Retrospective Studies ; Risk Assessment ; Risk Factors ; Staphylococcal Infections/cerebrospinal fluid/diagnosis/*drug therapy/*microbiology/mortality ; Staphylococcus/classification/*isolation & purification ; Time Factors ; Treatment Outcome ; Young Adult

This erratum is being published to correct of author name.

BACKGROUND/AIMS: Chronic urticaria (CU) is defined as itchy wheals lasting 6 weeks or more. As the aged population increases worldwide, it is essential to identify the specific features of this disease in the elderly population. METHODS: We investigated the prevalence and clinical features of CU in elderly patients. Medical records of 837 CU patients from the outpatient Allergy Clinic of Ajou University Hospital, Korea were analyzed retrospectively. Patients with chronic spontaneous urticaria according to the EAACI/GA2LEN/EDF/WAO guidelines were included. Patients older than 60 years were defined as elderly. RESULTS: Of the 837 patients, 37 (4.5%) were elderly. In elderly versus nonelderly CU patients, the prevalence of atopic dermatitis (AD) was significantly higher (37.8% vs. 21.7%, respectively; p = 0.022), while that of aspirin intolerance was lower (18.9% vs. 43.6%, respectively; p = 0.003) in terms of comorbid conditions. The prevalences of serum specific immunoglobulin E antibodies to staphylococcal enterotoxin A and staphylococcal enterotoxin B were considerably higher in elderly CU patients with AD than in those without AD (37.5% vs. 0%, respectively). CONCLUSIONS: Elderly patients with CU had a higher prevalence of AD. Therefore, there is a need to recognize the existence of AD in elderly CU patients.
Adolescent ; Adult ; Age Factors ; Aged ; Aged, 80 and over ; Aging ; Antibodies, Bacterial/blood ; Biological Markers/blood ; Child ; Chronic Disease ; Comorbidity ; Dermatitis, Atopic/diagnosis/epidemiology ; Enterotoxins/immunology ; Female ; Hospitals, University ; Humans ; Immunoglobulin E/blood ; Male ; Middle Aged ; Outpatient Clinics, Hospital ; Prevalence ; Republic of Korea/epidemiology ; Retrospective Studies ; Risk Factors ; Severity of Illness Index ; Urticaria/blood/*diagnosis/*epidemiology/immunology ; Young Adult

PURPOSE: Anaphylaxis is a rapidly progressive allergic reaction that requires precise recognition and immediate management. However, health care providers, awareness of anaphylaxis has not been acknowledged. The aim of this study is to investigate the extent of knowledge and principal management skills on anaphylaxis among medical personnel and students. METHODS: We performed a questionnaire survey on knowledge, education, and managing skills for anaphylaxis to physicians, nurses, health personnel, and medical students in Ajou University Medical Center, from 26 June to 31 October, 2014. The survey contained 2 main sections: questions about demographic data and 2 types of questionnaire (type I for all participants and type II for only medical staffs) for self-assessment on anaphylaxis. RESULTS: A total of 1,615 participants (128 doctors, 828 nurses, 436 students, and 223 health personnel) completed the survey. For questionnaire I, the percentages of correct answers in doctors, nurses, medical students, and health personnel were 77.5%, 56.4%, 47.8%, and 28.0% respectively, showing significant differences between groups (P<0.001). For questionnaire II, 93% of doctors and 75.6% of nurses indicated epinephrine as the drug of choice, and 79.7% of doctors and 71.3% of nurses selected the correct intramuscular route. More than 3 quarters of the doctors (80.5%) selected epinephrine within the first 5 steps of treatment, but only 48% included epinephrine within the first 3 steps. CONCLUSION: Our study showed considerable lack of knowledge on anaphylaxis among health care providers, especially on the specific management steps of anaphylaxis. As significant gaps on overall knowledge of anaphylaxis were observed between different groups of medical personnel, regular education should be implemented for each department in the health care setting.
Academic Medical Centers ; Anaphylaxis* ; Delivery of Health Care* ; Education ; Epinephrine ; Health Personnel* ; Humans ; Hypersensitivity ; Self-Assessment ; Students, Medical*