Sometimes, it is difficult to distinguish Churg-Strauss syndrome from idiopathic hypereosinophilic syndrome and there may be overlap syndrome in the differential diagnosis of systemic vasculitis with hypereosinophilia. Recently, we experienced a 42-year-old female patient who presented signs and symptoms of cardiac failure and neuropathy with peripheral hypereosinophilia. She had no history of asthma. She had erythematous skin lesions and distal digit necrosis. The cause of hypereosinophilia could not be identified. Skin and nerve biopsy revealed vasculitis with eosinophilic infiltration. Cardiac failure improved dramatically with steroid, inotropics and diuretics. Other symptoms including digital necrosis also improved. During steroid, tapering peripheral eosinophilia recurred. For maintenance therapy, we added daily cyclophosphamide to every-other-day prednisolone therapy. We report the case with a review of the literature.
Adult ; Asthma ; Biopsy ; Churg-Strauss Syndrome* ; Cyclophosphamide ; Diagnosis, Differential ; Diuretics ; Eosinophilia ; Eosinophils ; Female ; Heart Failure ; Humans ; Hypereosinophilic Syndrome* ; Necrosis ; Prednisolone ; Skin ; Systemic Vasculitis ; Vasculitis

BACKGROUND: Bronchial hbyperresponsiveness (BHR) is a key feature of asthma, and may precede the development of asthma. Genetically determined and acquired factors may contribute to development of BHR. OBJECTIVE: To evaluate expression of bronchial responsiveness to methacholine according to age, sex, smoking habit, and atopy in general population without bronchial asthma, a cross sectional study was performed. METHOD: A total of 1,190 general population who composed of 408 subjects with age less than 19 years (young age group), 621 subjects with age from 20 to 40 years (middle age group), and 161 subjects with age more than 41 years (old age group) were enrolled. Evaluations were made by a questionnaire, serum IgE level and skin prick test to common inhalant allergens, and methacholine bronchial provocation test (MBPT). Bronchial responsiveness were evaluated by positive rate of MBPT (PC,p-methacholine 4 25mg/ml), and slope of dose- response curve (slope, %fall of FEV, / log[last concentration of methacholine, mg/ml]). RESULT: Positive rate of MBPT was 11.0%, and slope (mean+SE) was 10.6+0.2 %/mg/ml. Postive rate of MBPT was more prevalent in the young age group than in middle and old age groups (19.6% vs. 6.6% vs. 6.2%, p<0.05), and slope was higher in young age group than in other groups (14.4+0.4 vs. 8.6+0.3 vs. 8.9+0.5 %/mg/ml, p<0.05). No significant differences in positive rate of MBPT and slope were noted according to sex in young and old age groups. However, in the middle age group, slope was higher in females than in males (9.5+0.4 vs. 7.9+ 0.3 %/mg/ml, p<0.05). No significant differences of slope was observed according to smoking habit in males of middle age group, but in males of old age group, the slope was higher in subjects with smoking habit than those without it (9.6+0.8 vs. 6.5+0.9 %/mg/ml, p<0.05). Significant relationship was observed between geometric value of serum IgE level and slope(r=0. 152, p=0.009). The postive rate of MBPT and slope were significantly higher in subjects with positive skin rea,ctivity to common inhalant allergens than those without it (14.3% vs. 8.6%, p ( 0.05; 11.8+0.4 vs. 9.8+0.3 %/mg/ml, p<0.05). The difference of bronchial responsiveness according to skin reactivity was observed in young and middle age groups, but not in old age group. CONCLUSION: Bronchial responsiveness to methacholine is significantly higher in children than in adults, in middle-aged females than in middle-aged males. Atopy and smoking may have a dif ferent role to determine the bronchial responsiveness depending upon age and sex.
Adult ; Allergens ; Asthma* ; Bronchial Provocation Tests ; Child ; Female ; Humans ; Immunoglobulin E ; Male ; Methacholine Chloride* ; Middle Aged ; Skin ; Smoke ; Smoking ; Surveys and Questionnaires

Eosinophilic gastroenteritis is an unusual disease characterized by eosinophilic infiltration of gastrointestinal tract, peripheral eosinophilia, and gastrointestinal symptoms. There are mucosal, muscular, and subserosal types, of which the subserosal type is the rarest. We experienced a 32-year old male patient with the subserosal type of eosinophilic gastroe-nteritis, who was presented with abdominal distension. The ascitic fluid revealed increased eosinophil count. With oral prednisolone, diarrhea and ascites rapidly disappeared and eosinophils in peripheral blood returned to normal. We report this case with a review of relevant literature.
Adult ; Ascites* ; Ascitic Fluid ; Diarrhea ; Eosinophilia ; Eosinophils* ; Gastroenteritis* ; Gastrointestinal Tract ; Humans ; Male ; Prednisolone

BACKGROUND: Increased IgE antibody responses to inhalant allergens and bronchial hyperresponsiveness are important phenotypes in development of asthma. Although heredity reported to be important in expression of these phenotypes in twin and family studies, genetic factor(s) controlling these phenotypes is unknown. OBJECTIVE: To evaluate whether genetic factor in chromosome 11q13 may control the expression of IgE responses to common inhalant allergens and bronchial hyperresponsiveness, linkage analysis between these phenotypes and gene marker of chromosome 11q13 was investigated. MATERIALS AND METHODS: The phenotyping and genotyping using microsatellite marker (D11S97) were performed in 77 probands with bronchial asthma and 80 their sibs. The linkage analysis between these phenotypes and the genotype was evaluated by affected or quantitative trait locus (QTL) sib-pair analysis. RESULTS: Positive skin test responses to inhalant allergens were 55/77(71.4%) in probands and 44/79(55.6%) in sibs, respectively. Positive bronchial provocation test responses to methacholine were 27/61(44.3%) in sibs, geometric mean of PC20-methacholine were 5.2 mg/ ml in probands and 39.4 mg/ml in sibs, respectively, and slope of dose response curve(mean+- SE, %/mg/ml) were 11.3 +- 3.22 in probands and 1.97 +- 0.5 in sibs, respectively. Of 34 sib-pairs with positive skin test responses to allergens, two D11S97 alleles were shared by 21(61.8% ) sib -pairs, one allele by 11(32.3% ) sib-pairs, and no identical allele by two(5.9% ) sib-pairs. In affected sib-pairs, sharing rate of the alleles was 77.9%, which indicates linkage of the phenotype and genotype(p<0.001). Of 25 sib-pairs with bronchial hyperresponsiveness to methacholine, two D11S97 alleles were shared by seven(28%) sib-pairs, one allele by 11(44%) sib-pairs, and no identical allele by seven(28% ) sib-pairs. In affected sib-pairs, sharing rate of the alleles was 50%, which indicates no linkage between the phenotype and genotype(p) 0.05). Differences of geometric value(mean +- SE) of PC-methacholine and slope of dose response curve(mean +- SE, %/mg/ml) were 1.11+- 0.17 and 8.33+- 3.35 in sib-pairs sharing two alleles, respectively, 0.99 +- 0.14 and 14.27+-5.75 in sib-pairs sharing one allele, respectively, and 0.57+-0.13 and 3.64+-1.62 in sib-pairs sharing no allele, respectively. There was no difference of the above values among the three groups. CONCLUSION: The expression of skin reactivity to common inhalant allergens was linked to gene marker of chromosome 11q13, not with bronchial responsiveness to methacholine.
Alleles ; Allergens* ; Antibody Formation ; Asthma ; Bronchial Provocation Tests ; Child* ; Genotype ; Heredity ; Humans ; Immunoglobulin E ; Methacholine Chloride ; Microsatellite Repeats ; Phenotype ; Quantitative Trait Loci ; Skin Tests ; Skin*

BACKGROUND: It is known that total serum IgE levels closely corrleate with prevaience of asthma regardless of atopic status. Although heredity is reported to be important in expression of total serum IgE in twin studies, genetic factor controlling this phenotype is controversial. Objective .' To evaluate whether genetic factor in chromosome 1 1q13 may control the expression of tatal serum IgE level, linkage analysis between this phenotype and gene marker of chromosome 11q13 was investigated. MATERIAL AND METHOD: Total serum IgE level and the genotype of chromosome 11q13 with microsatellite marker (D11597) was determined in 73 probands of asthmatic chiMren and 76 their sibs. Statistical significance of linkage was evaluated by affected and quantitative trait locus (QTL) sib-pair analysis. RESULT: In 20 affected sib-pairs with total serum IgE level higher than 305 IU/ml (geometric mean plus two folds SD in 53 normal controls), two D11S97 alleles were shared by ten sib-pairs, one allele by nine sib-pairs, and no allele by one sib-pairs. Sharing rate of the alleles in affect,ed sib-pairs, was 72.5%, which indicates linkage of the phenotype and genotype (x=4. 27, p=0.03). In 35 sib-pairs with total serum IgE level higher than 170 IU/ml (geometric mean plus one fold SD in 53 normal controls), two D11S97 alleles were shared by 16 sib-pairs, one allele by 15 sib-pairs, and no allele by four sib-pairs. The shar ing rate of the alleles in affected sibpairs, was 67.1%, which indicates linkage of the phenotype and the genotype(x=4. 24, p=0.03). Difference of geometric value of total serum IgE levels between probands and their sibs wa,s smaller in 32 sib-pairs sharing two alleles than in 32 those sharing one allele and 12 those with no identical allele (0.45+0.07 vs. 0.52+0.07 vs. 0.89 +0.21). CONCLUSION: The expression of total serum IgE level was linked to gene marker of chromosome 11q13.
Alleles ; Asthma ; Child* ; Chromosomes, Human, Pair 1 ; Genotype ; Heredity ; Humans ; Immunoglobulin E* ; Microsatellite Repeats ; Phenotype ; Quantitative Trait Loci

OBJECTIVE: Cerebral varices (CVs) without an arteriovenous shunt, so called nonfistulous CVs, are very rare, and their etiology and natural course are not well understood. The aim of this study is to evaluate the clinical outcomes of nonfistulous CVs by the analysis of 39 cases.METHODS: From 2000 to 2015, 22 patients with 39 nonfistulous CVs (≥5 mm) were found by searching the medical and radiologic records of our institute. Clinical data and radiological data including numbers, sizes and locations of CVs and associated anomalies were retrospectively collected and analyzed. Previously reported cases in literature were reviewed as well.RESULTS: The mean age of the patients was 21 years (range, 0–78 years). On average, 1.8±1.2 CVs were found per patient. CVs were categorized as either fusiform or saccular depending on their shapes. Two patients had saccular type CVs, seventeen patients had fusiform types, and three patients had both fusiform and saccular CVs. Eight patients had associated compromise of the vein of Galen and the straight sinus. Four of those patients had sinus pericranii, as well. Five patients had CVs that were distal draining veins of large developmental venous anomalies. One patient had associated migration anomaly, and two patients had Sturge- Weber syndrome. Six patients with an isolated cerebral varix were observed. Of the 39 CVs in 22 patients, 20 lesions in 14 patients were followed up in outpatient clinics with imaging studies. The average follow-up duration was 6.6 years. During this period, no neurological events occurred, and all the lesions were managed conservatively.CONCLUSION: Nonfistulous CVs seemed to be asymptomatic in most cases and remained clinically silent. Hence, we suggest conservative management.
Ambulatory Care Facilities ; Brain Stem Infarctions ; Central Nervous System Vascular Malformations ; Central Nervous System Venous Angioma ; Cerebral Veins ; Follow-Up Studies ; Humans ; Retrospective Studies ; Sinus Pericranii ; Varicose Veins ; Veins

OBJECTIVE: Cross-sectional survey was performed to evaluate prevalence of allergic rhinitis and its causative allergens in people in rural area of Cheju Isl~and. Subject and METHOD: A total of 1,236 people with 1,055 children and 181 adults in rural area of Cheju Island were enrolled in this study. In children, 544 were males and 511 were females, and their mean age was 12.3(ranging from 7 to 16) years. In adults, 78 were males and 103 were females, and their mean age was 50.1 (ratging from 18 to 87) years. Evaluations included a questionnaire survey, and skin prick test with extracts of Japanese cedar (Cryptomera japonica, J cedar) pollen and citrus red mite (Panonychus citri, CRM) as well as eleven common inhalant allergens. RESULTS: Prevalence of chronic nasal symptoms was 40.6% (429/1,055) in children, and 45.8 % (83/181) in adults. Prevalence of allergic rhinitis based on positive nasal symptoms on the questionnaire and positive skin response to one or more of the 13 inhalant allergens were 15.4 % (159/1.035) in children, and 19.3% (35/181) in adults. Of 1,035 children, skin-test response was positive in 37.6%, and common sensitizing allergens in order of decreasing frequency were Dermatophagoides pteronpssinus (DP) (26.6%), D. farinae (DF)(22.6%), CRM(14.2%), cockroach(11.3%) and J cedar(9.7%). Of 181 adults, skin-test response was positive in 25.9%, and t,he most common sensitizing allergen was CRM(16.5%), followed by cockroach(11.0%), DP(9. 9%), DF(9.4%), and J cedar(6.6%). Of 159 children with allergic rhinitis, the common sensitizing allergens in order of deereasing frequency were DP(79.2%), DF(67.3%), CRM(35.8%), J cedar(32.7%), and cockroach(27.0%). Of 35 adults with allergic rhinitis, the most common sensitizing allergen was CRM(68.6%), followed by DP(40%), DF(34.3%), cockroach(34.3%), and J cedar(28.9%). CONCLUSION: Allergic rhinitis is a very common disease with a prevalence of 15.4% in children and 19.3% in adults in rural area of Cheju island, and common sensitizing allergens include house dust mite, citrus red mite, cockroach, and Japanese cedar pollen.
Adult ; Allergens* ; Child ; Citrus ; Cockroaches ; Cross-Sectional Studies ; Cryptomeria ; Female ; Humans ; Jeju-do* ; Male ; Mites ; Pollen ; Prevalence* ; Pyroglyphidae ; Rhinitis* ; Skin ; Surveys and Questionnaires

OBJECTIVES: To prevent progression to fatal asthma it is essential to assess the severity of asthma and treat adequetely. But there was no objective index to evaluate the severity of airway inflammation in asthmatic patients, So we evaluated that serum ECP concentration is a objective parameter of airway inflammation in patients with acute severe asthmatic attack. METHODS: We measured the parameters of asthamtic severtity(clinical parameter, FEV1, arterial blood gas, peripheral blood eosinophil counts, and serum ECP levels) before and after antiasthmatic treatment in 9patients with acute severe asthmatic attack, And we compared serum ECP concentrations of severe asthmatic patients with those of control patients with mild to moderate severity. RESULTS: 1) The serum ECP levels in patients with acute asthmatic attacks compared to patients with mild to moderate, asthmatic severity were significantly increased. 2) The serum ECP levels and pheriperal blood eosinophil counts 2hours after antiasthmatic treatment were significantly lower than the value before treatment, but the total scores of clinical parameter, FEV1, and scores of arterial blood gases were not significantly different from the basal values, CONCLUSION: It is suggested that the serum ECP level is a objective parameter as a response to antiasthmatic treatment as well as a severity of airway inflammation in patients with acute asthmatic attack.
Asthma ; Eosinophil Cationic Protein* ; Eosinophils ; Gases ; Humans ; Inflammation

BACKGROUND: Prevalence of bee venom allergy in Korea is unknown, OBJECTIVE: We performed a cross-sectional study to evaluate the prevalence of bee venom allergy and importance of risk factors, such as age, sex, and atopy, in the development of the allergic reaction in children and adults living in rural area of Cheju Island. Swbject and METHOD: The prevalence was investigated in subjects with 1,054 children and 370 adults (701 male, 723 female), aged 7-87 years, living in rural area of Cheju Island. A history particularly focused on the systemic reactions after the bee stings was taken in all subjects. Skin prick test,s to common inhalant allergens, including house dust mites, fungi, tree, grass, mugwort and ragweed pollen, cockroach, animal epithelium, Japanese cedar pollen, and citrus red mite were performed. RESULT: The prevalence of bee venom allergy was 5.8% (mild systemic reactions 5.0%, severe systemic reactions 0.8% ). The prevalence was higher in adults than in children (10.2% vs. 5.0%, g = 12.0, p<0.01). Among the children, the prevalerlce wB.S Signifcantly higher ill male than in female (8.6% vs. 1.3%, x=28.7, p<0.01), but no significant diffecence was noted between male and female in adults (10.0% vs. 10.3%, p>0.05). The prevalence of bee venom allergy was not significantly different between subjects with positive skin reaction and those with negative skin reaction to inhalant allergens (6.4% vs. 5.4% ), Conclasion'. The prevlence of bee venom allergy in inhabitants of rural area of Cheju Island is 5.8% (mild systemic,' 5.0%, severe systemic reaction. 0.8%), and bee venom allergy was more common in adults than in children, and in male children than in female children.
Adult* ; Allergens ; Ambrosia ; Animals ; Artemisia ; Bee Venoms* ; Bees* ; Bites and Stings ; Child* ; Citrus ; Cockroaches ; Cross-Sectional Studies ; Cryptomeria ; Epithelium ; Female ; Fungi ; Humans ; Hypersensitivity* ; Jeju-do* ; Korea ; Male ; Mites ; Poaceae ; Pollen ; Prevalence* ; Pyroglyphidae ; Risk Factors ; Skin