Levamisole has been used for nephrotic syndrome due to its immunostimulating, immunomodulating, and steroid-sparing effects. Agranulocytosis, a serious side effect of levamisole, was rare and mostly associated with autoimmune disease, neoplastic disease and HLA B27 except one case in a nephrotic syndrome who was treated with high-dose(5mg/kg QOD) levamisole. This 15 year-old girl with steroid dependent nephrotic syndrome, who was negative for HLA B27, was treated with the usual recommended dose of levamisole(2.5mg/kg QOD). She developed agranulocytosis after 5 weeks of therapy and completely recovered in 11 days after discontinuation of levamisole.
Adolescent ; Agranulocytosis* ; Autoimmune Diseases ; Child* ; Female ; Humans ; Levamisole ; Nephrotic Syndrome*

PURPOSE: To evaluate the effect f nebulized budensonide and intramuscular dexamethasone in relieving laryngeal obstruction of croup. METHODS: Forty patients with moderate croup (croup scores of 3 to 7 out of 17) were randomly assigned to budesonide group [1 mg(2 ml) of nebulized budesonide, N=15], dexamethasone group [intramuscular dexamethasone(0.5 mg/kg) and nebulized saline, N=10] and control group(2 ml of nebulized saline, N=15). The changes of croup scores, heart rate, respiratory rate, and arterial oxygen saturation(SaO2) were observed at post-treatment of 2, 24, 36 and 48 hours. The duration of admission and the rates of improvement to the croup score of < or = 1 at the post-treatments were compared. RESULTS: Croup scores at 12, 24 and 36 hours post treatments were 1.7+/-0.8, 1.1+/-0.7 and 0.8+/-0.7 in budensonide group and 2.2+/-0.7, 1.2+/-0.8 and 1.0+/-0.5 in dexamethasone group respectively, which were significantly lower rate, and SaO2 were not significantly different among the three groups. Improved rates to croup score of <1 in budesonide group at post-treatments of 12, 24, and 36 jpirs were 53%, 73% and 87%, which were significantly higher than 0%, 13%, and 47% in control group (p<0.05). Improved rate to croup score of < or = 1 in dexamethasone group was 60^ which was significantly hgier than 13% in control group only at post-treatment of 24 hours (p<0.05). CONCLUSION: Nebulized budesonide and intramusculan dexamethasone are both effective in reducing obstructive symptoms in patients with moderate croup. The effect of nebulized budesonide was more rapid than that of intramuscular dexamethasone.
Budesonide* ; Croup ; Dexamethasone* ; Heart Rate ; Humans ; Oxygen ; Respiratory Rate

BACKGROUND: Korean National Health Insurance reimburses factor VIII (FVIII) and factor IX (FIX) clotting factor concentrate (CFC) infusions to discrepant activity levels, allowing elevation of FVIII activity to 60 IU/dL and FIX to 40 IU/dL. We aimed to assess hemostatic response to these target levels using global hemostatic assays. METHODS: We enrolled 34 normal healthy men, 34 patients with hemophilia A, and 36 with hemophilia B, with residual factor activity of 3 IU/dL or less and without inhibitors. Patients with hemophilia A and B received injected CFCs according to reimbursement guidelines. Fifteen minutes after injection, we assessed hemostatic response with global hemostatic assays: thrombin generation assay (TGA), thromboelastography (TEG), and clot waveform analysis (CWA). RESULTS: Normal healthy men and patients with hemophilia A and B were 36.7, 37.2, and 35.1 years old, respectively. FVIII and recombinant FIX concentrate doses were 28.8 IU/kg and 43.6 IU/kg. Post-infusion FVIII activity rose from 0.5 IU/dL to 69.4 IU/dL, while FIX activity rose from 1.4 IU/dL to 46.8 IU/dL. Post-infusion peak thrombin concentrations in hemophilia A and B were 116.6 nM/L and 76.4 nM/L (P < 0.001). Post-infusion endogenous thrombin potential (ETP) in hemophilia A and B was 1349.8 nM/min and 915.6 nM (P < 0.001). TEG index of hemophilia A and B was 0.11 and −0.51 (P=0.006). CONCLUSION: Current reimbursed doses for FIX concentrates are insufficient to achieve hemostatic responses comparable to those after reimbursed doses for FVIII concentrates in terms of peak thrombin concentration, ETP, and TEG index.
Factor IX* ; Factor VIII* ; Hemophilia A ; Hemophilia B ; Humans ; Male ; National Health Programs ; Thrombelastography ; Thrombin

Tracheal bronchus is an uncommon anomaly in which an ectopic bronchus originates directly from the supracarinal trachea. It is usually an asymptomatic anatomical variant incidentally found on computed tomography or bronchoscopy. However, it can present with symptoms, such as chronic cough, wheezing, atelectasis, and recurrent pneumonia. We report a case of tracheal bronchus diagnosed in the neonatal period, in which the term baby presented with respiratory distress and persistent pulmonary hypertension of the newborn after birth, but no other congenital anomaly was found on further evaluation.
Bronchi* ; Bronchoscopy ; Cough ; Female ; Humans ; Hypertension, Pulmonary* ; Infant ; Infant, Newborn* ; Parturition ; Persistent Fetal Circulation Syndrome ; Pneumonia ; Pulmonary Atelectasis ; Respiratory Sounds ; Trachea

The incidence of pregnancy in a rudimentary uterine horn is very rare. We experienced a case of unruptured rudimentary uterine horn pregnancy in the first trimester. The patient was diagnosed by laparoscopy and removed by laparoscopic resection of the pregnant rudimentary uterine horn. We report our case with brief review of literatures.
Animals ; Diagnosis* ; Female ; Horns* ; Humans ; Incidence ; Laparoscopy* ; Pregnancy Trimester, First ; Pregnancy*

PURPOSE: Biliary tract disease is one of the most common causes of acute abdominal pain in elderly patients, but there is still some debate over treatment. The aim of this study is to determine the safety and surgical outcomes of laparoscopic cholecystectomy (LC) for benign gallbladder disease in octogenarian patients. METHODS: We selected 42 patients of 80 years or older who underwent LC or open-converted cholecystectomy (OC) from February 1992 to November 2006. We evaluated clinicopathological features, treatment modalities, and surgical outcomes retrospectively. RESULTS: The patients included 17 males and 25 females. The mean age of the patients was 83.3 and 83.7, respectively. Right upper quadrant pain was the most common symptom, present in 85.7% of the patients. Gallstones were present in 85.7%. ASA class II and III comprised 92.9% of the patients. Comorbidities included hypertension, cardiac disease, pulmonary disease, and diabetes mellitus. Preoperatively biliary drainage was performed in 12 cases (endoscopic drainage in 8, percutaneous drainage in 4 cases). Preoperative cholecystostomy was performed in 5 cases. Emergency operations were more frequent than elective surgery (25 vs. 17). Mean hospital stay of LC patients was 5 days, whereas that of OC patients was 13 days. Open conversion rate was 16.7%. No surgical mortality was present and postoperative morbidity included acute myocardial infarction in one patient and wound infection in one patient. CONCLUSION: LC in octogenarian patients was safe. However, since the incidence of comorbidities is high in these patients, great care should be taken to evaluate and manage the comorbidities before surgery.
Abdominal Pain ; Aged ; Aged, 80 and over ; Biliary Tract Diseases ; Cholecystectomy ; Cholecystectomy, Laparoscopic ; Cholecystostomy ; Comorbidity ; Diabetes Mellitus ; Drainage ; Emergencies ; Female ; Gallbladder Diseases ; Gallstones ; Heart Diseases ; Humans ; Hypertension ; Incidence ; Length of Stay ; Lung Diseases ; Male ; Myocardial Infarction ; Wound Infection

PURPOSE: This study was conducted to develop Web-based multimedia contents for supporting student nurses' clinical practice on critical care, and to evaluate learners' responses. METHODS: Based on the steps of Assessment, Design, Development, Implementation, & Evaluation(ADDIE) model, a total of 13 self-directed learning modules including live lectures and real video clips were developed through faculty collaboration of nine nursing colleges in Gwangju and Chonnam province. The finally developed multimedia contents were published on the Web of the learning management system at a local e-learning center. RESULTS: The Web contents were evaluated after self-learning by 81 junior college nursing students who were encouraged to study it at their own pace during their two-week clinical practice at a medical or surgical intensive care unit of a university hospital and two hospitals. The knowledge (t = -27.66, p < .001) and self-evaluated clinical performance level(t = 7.54, p < .001) were significantly increased after learning of the Web contents and clinical practice, and satisfaction level that measured post-test only was 4.0 out of 5 point. CONCLUSION: The use of Web contents for critical care need to be extended as a complimentary material in a class room lecture or clinical practice of students to increase their self-learning ability and understandings of clinical knowledge and situation.
Computer-Assisted Instruction ; Cooperative Behavior ; Critical Care ; Humans ; Critical Care ; Learning ; Lectures ; Multimedia ; Students, Nursing

10% of labeled tumor cells) of TNF receptor 1 (TNFR1), the protein product of TNFRSF1A gene, was correlated with sarcomatoid dedifferentiation and was an independent predictive factor of clinically unfavorable response and shorter survivals in separated TKI-treated ccRCC cohort.CONCLUSION: TNF-α signaling may play a role in TKI resistance, and TNFR1 expression may serve as a predictive biomarker for clinically unfavorable TKI responses in ccRCC.]]>
Biomarkers ; Carcinoma, Renal Cell ; Cohort Studies ; Dataset ; Drug Resistance ; Gene Expression ; Gene Expression Profiling ; Heterografts ; Humans ; Immunohistochemistry ; Protein-Tyrosine Kinases ; Receptors, Tumor Necrosis Factor ; Receptors, Tumor Necrosis Factor, Type I ; Tumor Necrosis Factor-alpha

Purpose: Tyrosine kinase inhibitors (TKI) targeting vascular endothelial growth factor receptor (VEGFR) signaling pathways have been used for metastatic clear cell renal cell carcinoma (mCCRCC), but resistance to the drug develops in most patients. We aimed to explore the underlying mechanism of the TKI resistance with regard to programmed death-ligand 1 (PD-L1) and to investigate signaling pathway associated with the resistant mechanism. Materials and Methods: To determine the mechanism of resistance, 10 mCCRCC patients from whom tumor tissues were harvested at both the pretreatment and the TKI-resistant post-treatment period were included as the discovery cohort, and their global gene expression profiles were compared. A TKI-resistant renal cancer cell line was established by long-term treatment with sunitinib. Results: Among differentially expressed genes in the discovery cohort, increased PD-L1 expression in post-treatment tissues was noted in four patients. Pathway analysis showed that PD-L1 expression was positively correlated with the mammalian target of rapamycin (mTOR) signaling pathway. The TKI-resistant renal cancer cells showed increased expression of PD-L1 and mTOR signaling proteins and demonstrated aggressive tumoral behaviour. Treatment with mTOR inhibitors down-regulated PD-L1 expression and suppressed aggressive tumoral behaviour, which was reversed with stimulation of the mTOR pathway. Conclusion These results showed that PD-L1 expression may be increased in a subset of VEGFR-TKI–resistant mCCRCC patients via the mTOR pathway.

BACKGROUND: Mechanism and predictive biomarkers for tyrosine kinase inhibitor (TKI) resistance of advanced clear cell renal cell carcinoma (ccRCC) have not been fully evaluated. METHODS: We performed gene expression profiling on samples from an acquired TKI resistance cohort that consisted of 10 cases of TKI-treated ccRCC patients with matched tumor tissues harvested at pre-treatment and TKI-resistant post-treatment periods. In addition, a public microarray dataset from patient-derived xenograft model for TKI-treated ccRCC (GSE76068) was retrieved. Commonly altered pathways between the datasets were investigated by Ingenuity Pathway Analysis using commonly regulated differently expressed genes (DEGs). The significance of candidate DEG on intrinsic TKI resistance was assessed through immunohistochemistry in a separate cohort of 101 TKI-treated ccRCC cases. RESULTS: TNFRSF1A gene expression and tumor necrosis factor (TNF)-α pathway were upregulated in ccRCCs with acquired TKI resistance in both microarray datasets. Also, high expression (> 10% of labeled tumor cells) of TNF receptor 1 (TNFR1), the protein product of TNFRSF1A gene, was correlated with sarcomatoid dedifferentiation and was an independent predictive factor of clinically unfavorable response and shorter survivals in separated TKI-treated ccRCC cohort. CONCLUSION TNF-α signaling may play a role in TKI resistance, and TNFR1 expression may serve as a predictive biomarker for clinically unfavorable TKI responses in ccRCC.