No abstract available.
Child* ; Humans ; Proteinuria*

Spontaneous pneumomediastnum is a very rare complication of systemic autoimmune diseases. The precise mechanism of pneumomediastinum in dermatomyositis is not well known. Pulmonary alveoli rupture secondary to interstitial pneumonitis or pulmonary infarctions consequent upon vasculitis are the suggested mechanisms. Among the idiopathic inflammatory myopathies, dermatomyositis and polymyositis show similar clinical manifestations except skin lesions. But pneumomediastinum occurs exclusively in cases with dermatomyositis, not in case with polymyositis. In a literature review, patients with dermatomyositis and pneumomediastinum had some characteristic features. As compared with dermatomyositis without pneumomediastinum, CK level was normal in about half and concomitance of interstitial lung disease and cutaneous vasculitis were very frequent. We experienced a case of dermatomyositis with spontaneous pneumomediastinum and subcutaneous emphysema. Thus we report that with a review of the literature and analysis of reported cases.
Autoimmune Diseases ; Dermatomyositis* ; Humans ; Lung Diseases, Interstitial ; Mediastinal Emphysema* ; Myositis ; Polymyositis ; Pulmonary Alveoli ; Pulmonary Infarction ; Rupture ; Skin ; Subcutaneous Emphysema* ; Vasculitis

PURPOSE: Features of epidermal growth factor receptor (EGFR) expression in osteosarcoma and in vitro efficacies of EGFR inhibitors against osteosarcoma cells were evaluated. MATERIALS AND METHODS: Thirty biopsy samples of osteosarcoma patients were retrospectively analyzed for EGFR protein expression by immunohistochemistry. Relationships between EGFR expression and clinicopathologic characteristics and treatment outcomes were evaluated. Four osteosarcoma cell lines were analyzed for EGFR and p-EGFR expression by western blotting. Efficacies of gefitinib and BIBW2992 on osteosarcoma cells were evaluated using a 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide assay. Tyrosine kinase domains in exons 18 to 21 were sequenced and gene expression analyses of EGFR and PTEN were performed in four osteosarcoma cell lines. RESULTS: EGFR protein was expressed in 27 (90%) samples (6 low, 12 intermediate, 9 high) and in three cell lines. Intermediate or high staining for EGFR was related to a tumor volume<150 mL (p<0.001) and histologic subtype other than osteoblastic type (p=0.03). However, EGFR expression was not associated with histologic response to preoperative chemotherapy or survival. Gefitinib and BIBW 2992 did not have any significant inhibitory effect on cell viabilities. DNA sequencing analysis revealed three osteosarcoma cell lines have single base changes at codon 2361 of exon 20 (G to A), without affecting translation results. Furthermore, no mutation was found to be associated with constitutive EGFR activation. CONCLUSION: In the present study, gefitinib and BIBW2992 were not effective against osteosarcoma cells. However, as osteosarcoma cells express EGFR, further studies are necessary to explore the potential of other therapeutic agents targeting EGFR.
Biopsy ; Blotting, Western ; Cell Line ; Cell Survival ; Codon ; Epidermal Growth Factor ; Exons ; Gene Expression ; Humans ; Immunohistochemistry ; Osteoblasts ; Osteosarcoma ; Protein-Tyrosine Kinases ; Quinazolines ; Receptor, Epidermal Growth Factor ; Retrospective Studies ; Sequence Analysis, DNA ; Tetrazolium Salts ; Thiazoles

PURPOSE: Parenteral nutrition (PN) not only provides nutritional support but also plays a crucial role in the treatment of children with intestinal failure. The aim of this study was to evaluate the clinical significance and clinical outcomes of long-term PN. METHODS: Retrospective cohort study was conducted using the medical records of patients treated at Seoul National University Children's Hospital. This study included 19 patients who received PN for over six months. Most patients received home PN. RESULTS: The indications for PN included short bowel syndrome, chronic intestinal pseudo-obstruction, and intractable diarrhea of infancy. The median age of PN initiation was 1.3 years, and the median treatment duration was 2.9 years. Two patients were weaned from PN; 14 continued to receive PN with enteral feedings; and 3 patients died. The overall survival rates at 2 and 5 years were 93.3% and 84.0%, respectively. The incidence of catheter-related bloodstream infections was 2.7/1,000 catheter-days and was associated with younger age at PN initiation and lower initial height Z-score. Six patients developed catheter-related central vein thrombosis, with an incidence of 0.25/1,000 catheter-days. Eleven patients experienced PN-associated liver disease (PNALD), and one patient underwent multi-visceral transplant. The patients with PNALD exhibited lower final heights and body weight Z-scores. All patients experienced micronutrient deficiencies transiently while receiving PN. CONCLUSION: PN is an important and safe treatment for pediatric intestinal failure. PNALD was linked to final anthropometric poor outcomes. Micronutrient deficiencies were common. Anthropometric measurements and micronutrient levels must be monitored for successful PN completion.
Body Weight ; Catheter-Related Infections ; Child ; Cholestasis ; Cohort Studies ; Diarrhea ; Humans ; Incidence ; Intestinal Pseudo-Obstruction ; Liver Diseases ; Medical Records ; Micronutrients ; Nutritional Support ; Parenteral Nutrition ; Retrospective Studies ; Seoul ; Short Bowel Syndrome ; Survival Rate ; Thrombosis ; Veins

PURPOSE: The aim of the present study was to investigate the clinical features and outcome of eosinophilic gastroenteritis (EGE) in children. METHODS: Our study enrolled 24 children who were diagnosed with EGE from 1993 to 2014 at the Department of Pediatrics, Seoul National University Children's Hospital. The patients' clinical manifestations, treatments, and outcomes were reviewed from the medical records. RESULTS: The mean age at diagnosis was 5.3 years. Most patients had gastrointestinal symptoms including diarrhea (54.2%) and abdominal pain (45.8%). Peripheral eosinophilia was present in 91.7% of the patients. Thirteen patients (54.2%) showed anemia, and 15 patients (62.5%) had hypoalbuminemia. EGE was classified as mucosal, subserosal, or muscular in 75.0%, 20.8%, and 4.2% of cases, respectively. Three patients showed gastroduodenal ulcers upon endoscopic analysis. A history of allergy was reported in 13 patients, including atopic dermatitis, allergic rhinitis, and asthma. Five patients (20.8%) improved with food restrictions. Among the 19 patients treated with steroids, 11 (57.9%) discontinued steroid treatment without subsequent relapse, 4 (21.1%) relapsed after ceasing steroid treatment, and 4 (21.1%) showed no response to steroids. Two patients who were resistant to steroids underwent therapeutic surgery. The presence of gastroduodenal ulcers was significantly associated with relapse and steroid resistance. CONCLUSION: A high suspicion of EGE is warranted when children have nonspecific gastrointestinal symptoms and peripheral eosinophilia. Most patients improved with food restrictions or steroid treatment, although one-third of patients showed a relapse or steroid resistance.
Abdominal Pain ; Anemia ; Asthma ; Child* ; Dermatitis, Atopic ; Diagnosis ; Diarrhea ; Eosinophilia ; Eosinophils* ; Gastroenteritis* ; Humans ; Hypersensitivity ; Hypoalbuminemia ; Medical Records ; Pediatrics ; Peptic Ulcer ; Recurrence ; Rhinitis ; Seoul ; Steroids ; Ulcer

OBJECTIVE: To compare correlations between pulmonary function test (PFT) results and different reconstruction algorithms and to suggest the optimal reconstruction protocol for computed tomography (CT) quantification of low lung attenuation areas and airways in healthy individuals. MATERIALS AND METHODS: A total of 259 subjects with normal PFT and chest CT results were included. CT scans were reconstructed using filtered back projection, hybrid-iterative reconstruction, and model-based IR (MIR). For quantitative analysis, the emphysema index (EI) and wall area percentage (WA%) were determined. Subgroup analysis according to smoking history was also performed. RESULTS: The EIs of all the reconstruction algorithms correlated significantly with the forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) (all p < 0.001). The EI of MIR showed the strongest correlation with FEV1/FVC (r = −0.437). WA% showed a significant correlation with FEV1 in all the reconstruction algorithms (all p < 0.05) correlated significantly with FEV1/FVC for MIR only (p < 0.001). The WA% of MIR showed the strongest correlations with FEV1 (r = −0.205) and FEV1/FVC (r = −0.250). In subgroup analysis, the EI of MIR had the strongest correlation with PFT in both ever-smoker and never-smoker subgroups, although there was no significant difference in the EI between the reconstruction algorithms. WA% of MIR showed a significantly thinner airway thickness than the other algorithms (49.7 ± 7.6 in ever-smokers and 49.5 ± 7.5 in never-smokers, all p < 0.001), and also showed the strongest correlation with PFT in both ever-smoker and never-smoker subgroups. CONCLUSION: CT quantification of low lung attenuation areas and airways by means of MIR showed the strongest correlation with PFT results among the algorithms used, in normal subjects.
Emphysema ; Forced Expiratory Volume ; Lung* ; Pulmonary Emphysema ; Respiratory Function Tests* ; Smoke ; Smoking ; Tomography, X-Ray Computed ; Vital Capacity

OBJECTIVE: To evaluate the accuracy of emphysema volume (EV) and airway measurements (AMs) produced by various iterative reconstruction (IR) algorithms and virtual monoenergetic images (VME) at both low- and standard-dose settings. MATERIALS AND METHODS: Computed tomography (CT) images were obtained on phantom at both low- (30 mAs at 120 kVp) and standard-doses (100 mAs at 120 kVp). Each CT scan was reconstructed using filtered back projection, hybrid IR (iDose4; Philips Healthcare), model-based IR (IMR-R1, IMR-ST1, IMR-SP1; Philips Healthcare), and VME at 70 keV (VME70). The EV of each air column and wall area percentage (WA%) of each airway tube were measured in all algorithms. Absolute percentage measurement errors of EV (APEvol) and AM (APEWA%) were then calculated. RESULTS: Emphysema volume was most accurately measured in IMR-R1 (APEvol in low-dose, 0.053 ± 0.002; APEvol in standard-dose, 0.047 ± 0.003; all p < 0.001) and AM was the most accurate in IMR-SP1 on both low- and standard-doses CT (APEWA% in low-dose, 0.067 ± 0.002; APEWA% in standard-dose, 0.06 ± 0.003; all p < 0.001). There were no significant differences in the APEvol of IMR-R1 between low- and standard-doses (all p > 0.05). VME70 showed a significantly higher APEvol than iDose4, IMR-R1, and IMR-ST1 (all p < 0.004). VME70 also showed a significantly higher APEWA% compared with the other algorithms (all p < 0.001). CONCLUSION: IMR was the most accurate technique for measurement of both EV and airway wall thickness. However, VME70 did not show a significantly better accuracy compared with other algorithms.
Emphysema* ; Tomography, X-Ray Computed

Laparoscopic splenectomy (LS) has been reserved for intractable and relapsing immune thrombocytopenic purpura (ITP) despite medical treatment. With further experiences of LS in ITP, we investigated long term outcomes of LS, especially newly developed morbidities, and tried to find predictive factors for favorable outcomes. From August 1994 to December 2004, fifty-nine patients whose follow-up period was more than 12 months after LS were investigated. After a long-term follow-up (median 54 months, range 12.5-129 months), a complete response (CR) was found in 28 patients (47.5%), partial response in 24 (40.7%), and no response in 7 (11.9%). The relapse rate during follow-up periods was 15.2%. The rapid response group (p=0.017), in which the platelet count increased more than twice of the preoperative platelet count within 7 days after LS, relapsing after medical treatment (p=0.02), and the satisfactory group as the initial result of LS (p=0.001) were significant for predicting CR in univariate analysis, but only the initial satisfactory group was an independent predictive factor for CR in multivariate analysis (p=0.036, relative risk=6419; 95% CI, 1.171-35.190). Infections were the most frequent morbidities during the follow-up period, which were treated well without mortality. LS is a safe and effective treatment modality for ITP. Active referral to surgery might be required, considering complications and treatment results related to long-term use of steroid-based medications.
Adolescent ; Adult ; Aged ; Female ; Follow-Up Studies ; Humans ; Laparoscopy/*methods ; Male ; Middle Aged ; Multivariate Analysis ; Purpura, Thrombocytopenic, Idiopathic/*surgery/therapy ; Recurrence ; Splenectomy/*methods ; Time Factors ; Treatment Outcome

BACKGROUND: Biomarkers for cancer have several potential clinical uses, including the following: early cancer detection, monitoring for recurrence prognostication, and risk stratification. However, no biomarker has been shown to have adequate sensitivity and specificity. Many investigators have tried to validate biomarkers for the early detection and recurrence of lung cancer. To evaluate plasma G-CSF as such a biomarker, protein levels were measured and were found to correlate with the clinicopathological features of primary lung tumors. METHODS: Between December 2006 and May 2008, 100 patients with histologically-validated primary lung cancer were enrolled into this study. To serve as controls, 127 healthy volunteers were enrolled into this study. Plasma G-CSF levels were measured in lung cancer patients using the sandwich ELISA system (R & D inc.) prior to treatment. RESULTS: The mean plasma G-CSF levels were 12.2+/-0.3 pg/mL and 46.0+/-3.8 pg/mL (mean+/-SE) in the normal and in the cancer groups, respectively. In addition, plasma G-CSF levels were higher in patients with early lung cancer than in healthy volunteers (p<.001). Plasma G-CSF levels were higher in patients who were under 65 years old or smokers. Within the cancer group, plasma G-CSF levels were higher in patients with non small cell lung cancer than in patients with small cell lung cancer (p<.05). Overall, plasma G-CSF levels were shown to increase dependent upon the type of lung cancer diagnsosed. In the order from highest to lowest, the levels of plasma G-CSF tended to decrease in the following order: large cell carcinoma, squamous cell carcinoma, adenocarcinoma, and bronchioloalveolar carcinoma. Plasma G-CSF levels tended to be higher in patients with advanced TNM stage than in localized TNM stage (I, II Adenocarcinoma ; Adenocarcinoma, Bronchiolo-Alveolar ; Adrenal Glands ; Biomarkers ; Carcinoma, Large Cell ; Carcinoma, Squamous Cell ; Enzyme-Linked Immunosorbent Assay ; Granulocyte Colony-Stimulating Factor ; Humans ; Lung ; Lung Neoplasms ; Neoplasm Metastasis ; Plasma ; Recurrence ; Research Personnel ; Small Cell Lung Carcinoma