We report a case of congenital vellus hamartoma, which consists of numerous matured vellus hair follicles and sebaceous glands. This patient is 4-month-old male with multiple, variable sized nodules on the right epicanthsl fold area since birth. Histopathologically, some of the infundibular portion of the vellus hair shows irregular and reticulated hyperplasia. There are numerous well-formed vellus hair follicles surrounded with thick fibrous sheath and some of them are associated with rudiment sebaceous glands.
Hair ; Hair Follicle ; Hamartoma* ; Humans ; Hyperplasia ; Infant ; Male ; Parturition ; Sebaceous Glands

OBJECTIVE: To evaluate the basophil histamine releasability in response to IgE- and non- IgE-mediated stimuli in children with atopic asthma. Met: Basophil histamine releasability was measured in Dermatophagoides farinae (D. farinae)-sensitive atopic asthmatics, D.farinae-sensitive healthy atopics, non-atopic asthmatics, and healthy non-atopics. Basophils were stimulated with D.farinae, goat antihuman IgE antibody, formyl-Met-Leu-Phe(fMLP), and Calcium ionophore A23187. Histamine was measured by automated fluorometric technique. RESULTS: Sponianeous histamine release was higher in atopic asthmatics compared to healthy non-atopics. Histamine release by D.farinae and by anti-IgE antibody was higher in atopic asthmatics compared to the other groups. There was no difference in histamine release by fMLP among all groups. Histamine release by Calcium ionophore was higher in healthy atopics and non-atopic asthmatics compared to healthy non-atopics. The atopics showed correlation between histamine release by D.farinae, by anti-IgE antibody and total serum IgE levels. CONCLUSIONS: Spontaneous and IgE-mediated histamine release were related to the presence of both atopy and asthma, whereas non-IgE mediated histamine release was different depending on the stimuli.
Asthma* ; Basophils* ; Calcimycin ; Calcium ; Child* ; Dermatophagoides farinae ; Goats ; Histamine Release ; Histamine* ; Humans ; Immunoglobulin E

MELAS syndrome is a rare but distinct clinical entity belonging to a group of mitochondrial encephalomyopathies characterized by the tetrad of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. We experienced a case of MELAS syndrome in an 8 year-old boy who showed headache, pain of the eyeball, vomiting, stroke-like episodes such as visual disturbance and dysarthria, myoclonic seizure, confusion, and walking disturbance. His serum lactate level was elevated up to 48 mg/dl. MRI findings showed high signal intensities T2-weighted image and low signal intensities in T1-weighted image in the right thalamus and parietooccipital lobe and bilateral symmetric high signal intensity in T1-dweighted image in the basal ganglia. We have seen the dispersed ragged-red fibers with modified Gomori trichrome staining on light microscope, and abundant and dysmorphic mitochondria on electon microscope in the specimen of muscle biopsy. esis of SLE.
Basal Ganglia ; Biopsy ; Child ; Dysarthria ; Headache ; Humans ; Lactic Acid ; Magnetic Resonance Imaging ; Male ; MELAS Syndrome* ; Mitochondria ; Mitochondrial Encephalomyopathies ; Seizures ; Thalamus ; Vomiting ; Walking

To investigate the imbalance of the cytokine production profile of T cells from atopic asthmatics, we measured concentrations of IL-4, IL-5 and IFN-y by ELISA method in the culture supernatants of peripheral blood mononuclear cells(PBMCs) and Derrnato-phagoides pteronyssinus(Der p) J-stimulated PBMCs from Der p-sensitized atopic asthmatics, Der p-sensitized healthy atopits, non-atopic asthmatics and healthy non-atopics. The suppressive effect of IFN-y on cytokine production of Der p J-stimulated PBMCs was also examined. The PBMCs from atopics showed higher IL-4 and IL-5 production in response to PHA +TPA and higher IFN-gamma production in response to Der p Jq compared with non-atopits. The Der p J-stimulated PBMCs from atopics showed a tendency of increased IL-5 production in response to Der p J and higher IL-4 and IL-5 production in response to PHA+TPA compared with non-atopics. IL-5 production of Der p J-stimulated PBMCs from atopics was suppressed by IFN It is suggested that an imbalance in IL-4, IL-5 and IFN-y production is a feature of the atopic state. The TH2 characteristics of allergen-stimulated PBMCs could be regulated by IFN-y.
Enzyme-Linked Immunosorbent Assay ; Interleukin-4 ; Interleukin-5 ; T-Lymphocytes

The primary rhabdomyosarcoma of the brain is very rare. There are only 14 cases reported in the literatures till 1975, and the majority of them were arised in the cerebellum. The intracranial rhabdomyosarcomas may be originated from the multipotent mesenchymal cells of aberrent muscle tissue in the leptomeninges. The histological 3 types are adult pleomorphic, alveolar and embryonal type. The demonstration of cross-striation is confirmed for diagnosis of this tumor. All of these tumors tend to have a short clinical course, but the survival time seems to be slightly improved by surgical excision followed by radiotherapy. We have recently experienced one case of primary rhabdomyosarcoma in the left cerebellopontine angle of 9 year-old girl, which was confirmed by operation and complete autopsy.
Adult ; Autopsy ; Brain ; Cerebellopontine Angle ; Cerebellum ; Child ; Diagnosis ; Female ; Humans ; Radiotherapy ; Rhabdomyosarcoma*

PURPOSE: Environmental factors may increase risk for childhood asthma. The objective of this study was to investigate the association of environmental tobacco smoke (ETS) exposure and home environment with the development of wheeze and asthma. METHODS: We conducted a cross-sectional survey of 969 elementary school students. Data included questions about asthma and wheeze symptoms, exposure to tobacco smoke, and home environment including history of moving into a new house within 1 year of age after birth, use of fragrance at home, recent purchase of new furniture. Logistic regression and Pearson chi statistics were used to estimate these associations. RESULTS: ETS exposure was associated with current wheeze and nocturnal sleep disturbance. Paternal smoking over 20 cigarettes per day was associated with 4 or more episodes of wheeze during the past 1 year. However, there were no significant differences in pulmonary function, serum total IgE levels, blood eosinophil counts, and atopy between children with ETS exposure and non-exposure. Moving into a new house within 1 year of birth was associated with current asthma or wheeze. Use of fragrance at home and purchase of new furniture during the past 1 year were associated with current wheeze, current physician-diagnosed asthma, and nocturnal cough. CONCLUSION: Exposure to ETS, moving into a new house after birth, and indoor fragrance are risk factors for wheeze and asthma in schoolchildren.
Asthma ; Child ; Cross-Sectional Studies ; Eosinophils ; Humans ; Immunoglobulin E ; Interior Design and Furnishings ; Logistic Models ; Parturition ; Risk Factors ; Smoke ; Smoking ; Tobacco ; Tobacco Products

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disease due to bronchial colonization of Aspergillus fumigatus that occurs in susceptible patients with asthma or cystic fibrosis. A 10-year-old girl was referred to the Department of Pediatric Pulmonology for persistent consolidations on chest radiography. Pulmonary consolidations were observed in the right upper and left lower lobes and were not resolved with a 4-week prescription of broad-spectrum antibiotics. The patient had a history of atopic dermatitis and allergic rhinitis but no history of asthma. She had no fever but produced thick and greenish sputum. Her breathing sounds were clear. On laboratory testing, her total blood eosinophil count was 1,412/mm3 and total serum IgE level was 2,200 kU/L. Aspergillus was isolated in the sputum culture. The A. fumigatus-specific IgE level was 15.4 kU/L, and the Aspergillus antibody test was also positive. A chest computed tomography scan demonstrated bronchial wall thickening and consolidation without bronchiectasis. An antifungal agent was added but resulted in no improvement of pulmonary consolidations after 3 weeks. Pulmonary function test was normal. Methacholine provocation test was performed, revealing bronchial hyperreactivity (PC20=5.31 mg/mL). Although the patient had no history of asthma or bronchiectasis, ABPA-seropositivity was suspected. Oral prednisolone (1 mg/kg/day) combined with antifungal therapy was started. Pulmonary consolidations began decreasing after 1 week of treatment and completely resolved after 1 month. This is the first observed and treated case of seropositive ABPA in Korean children without previously documented asthma.
Anti-Bacterial Agents ; Aspergillosis, Allergic Bronchopulmonary* ; Aspergillus ; Aspergillus fumigatus ; Asthma* ; Bronchial Hyperreactivity ; Bronchiectasis ; Child* ; Colon ; Cystic Fibrosis ; Dermatitis, Atopic ; Eosinophils ; Female ; Fever ; Humans ; Hypersensitivity ; Immunoglobulin E ; Lung Diseases ; Methacholine Chloride ; Prednisolone ; Prescriptions ; Pulmonary Medicine ; Radiography ; Respiratory Function Tests ; Respiratory Sounds ; Rhinitis ; Sputum ; Thorax

PURPOSE: Atopic dermatitis (AD) is a chronic inflammatory relapsing skin disorder. Vitamin D plays a pivotal role in the development of AD, and interleukin (IL) 31 is known to be related to pruritus in AD. The aim of our study was to determine whether 25-hydroxyvitamin D (25(OH)D) levels are related to IL-31 levels or to the severity of AD. METHODS: We enrolled 91 children with AD and 32 control subjects without history or symptoms of allergic diseases. Blood was drawn to evaluate complete blood cell count, total eosinophil count (TEC), and total IgE, specific IgE to common allergens, 25(OH)D, and IL-31 levels. Serum 25(OH)D and IL-31 levels were measured using high-performance liquid chromatography and enzyme-linked immunosorbent assay, respectively. The scoring atopic dermatitis (SCORAD) index was used to evaluate the severity of AD. RESULTS: The mean 25(OH)D level was significantly lower in the AD group than in the control group; 25(OH)D decreased greatly in the moderate and severe AD groups compared with the mild AD group. Children with atopic sensitization showed significantly lower 25(OH)D levels than nonatopic children. However, serum IL-31 levels were not related to AD group, SCORAD index, or 25(OH)D levels. The SCORAD index was inversely correlated with serum 25(OH)D level and positively correlated with TECs and total IgE levels. Children with moderate and severe AD had significantly higher TECs than children with mild AD. CONCLUSION: Vitamin D is related to the severity of AD independently of IL-31.
Allergens ; Blood Cell Count ; Child* ; Chromatography, Liquid ; Dermatitis, Atopic* ; Enzyme-Linked Immunosorbent Assay ; Eosinophils ; Humans ; Immunoglobulin E ; Interleukins ; Pruritus ; Skin ; Vitamin D

PURPOSE: A nationwide outbreak of Mycoplasma pneumoniae pneumonia (MP) refractory to macrolide antibiotics occurred in Korea during 2011. Steroid therapy has been reported to be both efficacious and well tolerated in pediatric patients with refractory MP. We compared clinical features and laboratory characteristics between children with refractory MP requiring steroid treatment and those with macrolide-responsive MP and evaluated the risk factors associated with refractory MP. METHODS: We investigated 203 children who were admitted to our institution with MP from June to November 2011. Refractory MP was defined by persistent fever over 38.3degrees C with progressive pulmonary consolidation or pleural effusion despite administration of appropriate macrolide antibiotics for 5 days or longer after admission. Steroid therapy was initiated on the fifth day after admission for refractory cases. RESULTS: There were 26 patients with refractory MP requiring steroid therapy. The mean duration of steroid therapy was 5.4 days and most of the patients were afebrile within 24 hours after initiation of steroid therapy. The prevalence of refractory MP was higher in patients with pleural effusion, lobar pneumonia affecting more than 2 lobes, higher levels of serum lactate dehydrogenase, increased oxygen requirements, and longer duration of hospitalization. Atopic sensitization and history of asthma were also associated with refractory MP after adjusting for age and gender. CONCLUSION: Children with refractory MP had more severe pneumonia. Atopic sensitization and history of asthma may be risk factors for refractory MP requiring steroid therapy in Korean children.
Anti-Bacterial Agents ; Asthma* ; Child* ; Fever ; Hospitalization ; Humans ; Korea ; L-Lactate Dehydrogenase ; Mycoplasma ; Mycoplasma pneumoniae* ; Oxygen ; Pleural Effusion ; Pneumonia* ; Pneumonia, Mycoplasma* ; Prevalence ; Risk Factors

PURPOSE: Macrolide-resistant Mycoplasma pneumoniae pneumonia (MPP) is characterized by prolonged fever and radiological progression despite macrolide treatment. Few studies have examined serum procalcitonin (PCT) level in children with MPP. We aimed to investigate the association of acute inflammation markers including PCT with clinical parameters in children with MPP. METHODS: A total of 147 children were recruited. The diagnosis of MPP relied on serial measurement of IgM antibody against mycoplasma and/or polymerase chain reaction. We evaluated the relationships between C-reactive protein (CRP), PCT, and lactate dehydrogenase (LDH) levels and white blood cell (WBC) counts, and clinical severity of the disease. We used multivariate logistic regression analysis to estimate the odds ratio for prolonged fever (>3 days after admission) and hospital stay (> 6 days), comparing quintiles 2–5 of the PCT levels with the lowest quintile. RESULTS: The serum PCT and CRP levels were higher in children with fever and hospital stay than in those with fever lasting ≤ 3 days after admission and hospital stay ≤ 6 days. CRP level was higher in segmental/lobar pneumonia than in bronchopneumonia. The LDH level and WBC counts were higher in children with fever lasting for >3 days before compared to those with fever lasting for ≤ 3 days. The highest quintile of PCT levels was associated with a significantly higher risk of prolonged fever and/or hospital stay than the lowest quintile. CONCLUSION: Serum PCT and CRP levels on admission day were associated with persistent fever and longer hospitalization in children with MPP.
Bronchopneumonia ; C-Reactive Protein ; Child* ; Diagnosis ; Drug Resistance ; Fever* ; Hospitalization ; Humans ; Immunoglobulin M ; Inflammation ; L-Lactate Dehydrogenase ; Length of Stay ; Leukocytes ; Logistic Models ; Mycoplasma pneumoniae ; Mycoplasma* ; Odds Ratio ; Pneumonia ; Pneumonia, Mycoplasma* ; Polymerase Chain Reaction ; Risk Factors*