Epithelioid smooth muscle tumor is relatively rare and potentially malignant, especially in retroperitoneum. Distinct criteria for malignancy still have not been clarified in this epithelioid variant arising in retroperitoneum. We report a deceptively benign-appearing epithelioid leiomyosarcoma in a 50-year-old female. She was admitted with abdominal discomfort and dysuria. Abdominal CT showed a well-demarcated, 10 10 cm sized, solid mass in retroperitoneum. Concomitant metastatic lesions were noted in right lung field. Surgical excision of retroperitoneal mass and right lung lobectomy were performed. The retroperitoneal mass showed yellowish-tan, well-delineated and lobulated appearance. Histologically, this tumor was composed of predominantly epithelioid, round to oval cells with distinct clear cytoplasm and slightly atypical nuclei. Mitosis was rare (0~1/50 HPF). Lung lesions were morphologically similar to that of retroperitoneum.
Cytoplasm ; Dysuria ; Female ; Humans ; Leiomyosarcoma* ; Lung ; Middle Aged ; Mitosis ; Smooth Muscle Tumor ; Tomography, X-Ray Computed

No abstract available.
Blindness, Cortical* ; Diagnosis* ; Magnetic Resonance Imaging* ; Pregnancy*

Encephaloceles are congenital defects in the skull through which menings and brain tissues herniated. Basal encephaloceles are found in 1 in 40,000 live births. Transsphenoidal basal encephaloceles are very rare, accounting for only 5% of all basal encephaloceles. Currently, most encephaloceles are diagnosed antenatally and present at birth. Postnatally, infants may present with CSF rhinorrhea, recurrent meningitis, headache, hypothalamic- pituitary dysfunction. Some, particularly trasnssphenoidal encephaloceles are often clinically occult and diagnosis of the disease may be postponed up to adulthood. Surgical indications and approaches for transsphenoidal encephaloceles have been remained controversial. We report a case of CSF rhinorrhea and recurrent meningitis caused by transsphenoidal encephalocele.
Brain ; Congenital Abnormalities ; Diagnosis ; Encephalocele* ; Headache ; Humans ; Infant ; Live Birth ; Meningitis ; Meningitis, Bacterial* ; Parturition ; Skull

Background/Aims: Asthma is not a single disease but, rather, a heterogeneous inf lammatory disorder with various pathogenic mechanisms. We analyzed the associations between the cellular profile of sputum and the serum levels of inflammatory mediators/cytokines in a cohort of adult asthmatics. Methods: We recruited 421 adult asthmatic patients. All subjects were classified into four groups according to their sputum cellular profiles: G1, eosinophilic; G2, mixed granulocytic; G3, neutrophilic; and G4, paucigranulocytic. Serum levels of cytokines and mediators including periostin, eosinophil-derived neurotoxin (EDN), S100A9, and folliculin were quantified. Results: Among 421 patients, G1 accounted for 149 (35.4%), G2 for 71 (16.9%), G3 for 155 (36.8%), and G4 for 46 (10.9%). Serum periostin and EDN levels were significantly higher in G1 (p = 0.004, and p = 0.031) than in the others. Serum S100A9 levels were elevated in G2 and G3 (p = 0.008). Serum folliculin levels differed significantly among the four groups, with the highest level in G4 (p = 0.042). To identify G1 from G1 plus G2 groups, the optimal serum cut-off levels were 1.71 ng/mL for periostin, and 1.61 ng/mL for EDN. When these two parameters were combined, the sensitivity was 76.0% and the specificity was 64.3% (area under the curve, 0.701; p = 0.004). Conclusions The serum periostin and EDN levels may be used as predictors to discriminate the eosinophilic asthma group from patients having eosinophilic or mixed granulocytic asthma, and the serum folliculin level is significantly elevated in patients with paucigranulocytic asthma compared to those with different inflammatory cell profile.

No abstract available.
Sertoli-Leydig Cell Tumor*

PURPOSE: Assessment of the severity and clinical course of asthma is important for effective disease control. Cognitive and physical impairments occur due to the aging process, which may impact on asthma control. We aimed to evaluate the impact of cognitive function on the assessment of asthma control in older asthmatics as a prospective interventional trial. METHODS: A total of 50 mild to moderate asthmatics over 60 years of age were enrolled. Three questionnaires were used: the asthma control test (ACT), the asthma-specific quality of life, and the Korean version of the Short Form of the Geriatric Depression Scale. The Seoul neuropsychological screening battery-dementia version (SNSB-D), Korean version of the Mini-Mental Status Examination, and the Seoul instrumental activities of daily living scale were applied for neuropsychological assessment. RESULTS: The mean patient age was 67.0±4.9 years, and 30 patients (60.0%) were female. The sensitivity and specificity of the ACT for determining well-controlled asthma were 91.7% and 39.5%, respectively. Regarding neuropsychological assessment, 22 patients (44%) had mild cognitive impairment, 4 (8.7%) had dementia, and 17 (34%) had depression. Total SNSB-D score was significantly higher in patients with an ACT score of ≥20 (P=0.015). The ACT scores were significantly associated with SNSB-D results in analyses adjusted for age, sex, education duration, lung function, and depression (P=0.004). CONCLUSION: We found significant positive correlations between cognitive functions, as measured by SNSB-D, and asthma control status, as measured by ACT scores, in older patients with asthma. Therefore, cognitive impairment may be associated with poor asthma control in older asthmatics.
Activities of Daily Living ; Aging ; Asthma* ; Cognition ; Cognition Disorders* ; Dementia ; Depression ; Education ; Female ; Humans ; Lung ; Mass Screening ; Mild Cognitive Impairment ; Prospective Studies ; Quality of Life ; Sensitivity and Specificity ; Seoul

PURPOSE: The IL-4 and IL-4 receptor alpha (IL-4Ralpha) genes are the key candidate genes for atopy and asthma susceptibility. Exposure to wheat flour can cause IgE sensitization and respiratory symptoms in bakery workers. Therefore, we hypothesized that IL-4 and IL-4Ralpha single nucleotide polymorphisms (SNPs) may be involved in the pathogenic mechanism of baker's asthma. METHODS: Clinical and genetic data from 373 bakery workers were analyzed. A survey questionnaire, spirometry, and skin prick tests with wheat flour were performed. Serum-specific IgE, IgG1, and IgG4 to wheat flour were determined using ELISA. Five candidate IL-4 (-729 T>G, 589 T>C, and 33 T>C) and IL-4Ralpha (Ile75Val A>G and Gln576Arg A>G) SNPs were genotyped and analyzed. RESULTS: Workers with the G allele of IL-4Ralpha Ile75Val A>G had a significantly higher prevalence of work-related lower respiratory symptoms than those with the AA genotype (P=0.004, 16.0% vs. 2.9%). In the skin prick test, workers with the AA genotype of IL-4Ralpha Gln576Arg A>G had a significantly higher positive rate to wheat flour (P=0.015, 8.2% vs. 1.1%) than those with AG/GG genotype. No significant associations were found in the three genetic polymorphisms of IL-4. For the predicted probabilities, workers with the AA genotype of Gln576Arg A>G had a higher prevalence of IgG1 and IgG4 in response to wheat flour, according to increased exposure intensity (P=0.001 for IgG1 and P=0.003 for IgG4). CONCLUSIONS: These findings suggest that the IL-4Ralpha Ile75Val and Gln576Arg polymorphisms may be associated with work-related respiratory symptom development.
Alleles ; Asthma ; Enzyme-Linked Immunosorbent Assay ; Flour ; Genotype ; Immunoglobulin E ; Immunoglobulin G ; Interleukin-4 ; Polymorphism, Genetic ; Polymorphism, Single Nucleotide ; Prevalence ; Receptors, Interleukin-4 ; Skin ; Spirometry ; Triticum ; Surveys and Questionnaires

PURPOSE: The IL-4 and IL-4 receptor alpha (IL-4Ralpha) genes are the key candidate genes for atopy and asthma susceptibility. Exposure to wheat flour can cause IgE sensitization and respiratory symptoms in bakery workers. Therefore, we hypothesized that IL-4 and IL-4Ralpha single nucleotide polymorphisms (SNPs) may be involved in the pathogenic mechanism of baker's asthma. METHODS: Clinical and genetic data from 373 bakery workers were analyzed. A survey questionnaire, spirometry, and skin prick tests with wheat flour were performed. Serum-specific IgE, IgG1, and IgG4 to wheat flour were determined using ELISA. Five candidate IL-4 (-729 T>G, 589 T>C, and 33 T>C) and IL-4Ralpha (Ile75Val A>G and Gln576Arg A>G) SNPs were genotyped and analyzed. RESULTS: Workers with the G allele of IL-4Ralpha Ile75Val A>G had a significantly higher prevalence of work-related lower respiratory symptoms than those with the AA genotype (P=0.004, 16.0% vs. 2.9%). In the skin prick test, workers with the AA genotype of IL-4Ralpha Gln576Arg A>G had a significantly higher positive rate to wheat flour (P=0.015, 8.2% vs. 1.1%) than those with AG/GG genotype. No significant associations were found in the three genetic polymorphisms of IL-4. For the predicted probabilities, workers with the AA genotype of Gln576Arg A>G had a higher prevalence of IgG1 and IgG4 in response to wheat flour, according to increased exposure intensity (P=0.001 for IgG1 and P=0.003 for IgG4). CONCLUSIONS: These findings suggest that the IL-4Ralpha Ile75Val and Gln576Arg polymorphisms may be associated with work-related respiratory symptom development.
Alleles ; Asthma ; Enzyme-Linked Immunosorbent Assay ; Flour ; Genotype ; Immunoglobulin E ; Immunoglobulin G ; Interleukin-4 ; Polymorphism, Genetic ; Polymorphism, Single Nucleotide ; Prevalence ; Receptors, Interleukin-4 ; Skin ; Spirometry ; Triticum ; Surveys and Questionnaires

No abstract available.
Skin*

The genetic mechanism of aspirin intolerant acute urticaria (AIAU) is unknown. To demonstrate an association between the beta2 adrenergic receptor (ADRB2) polymorphism and the phenotype of AIAU, one hundred fourteen patients with AIAU, 110 patients with aspirin intolerant chronic urticaria (AICU), and 498 normal healthy controls (NC) based on a Korean population were enrolled. The genotype of ADRB2 at 46 A > G was analyzed using a direct sequencing method. The ADRB2 polymorphism at 46 A > G showed a significant difference between AIAU and NC; the frequency of the major genotype was significantly higher in the AIAU group (p= 0.017 in recessive model), while no differences were noted in allele and genotype frequencies between AICU and NC. In conclusion, the ADRB2 (46 A > G) gene polymorphism may contribute to the development of the phenotype of AIAU.
Adult ; Aspirin/*adverse effects ; Female ; Gene Frequency ; Genotype ; Humans ; Male ; Middle Aged ; Phenotype ; *Polymorphism, Single Nucleotide ; Receptors, Adrenergic, beta-2/*genetics ; Urticaria/chemically induced/*genetics/pathology