No abstract available.
Amputees* ; Energy Metabolism* ; Humans ; Lower Extremity*

Juvenile nasopharyngeal angiofibroma (JNA) is a highly vascular and locally invasive tumor that occurs almost exclusively in adolescent boys. JNA accounts for 0.05-0.5% of all head and neck tumors. Pre-operative embolization is useful for reducing intraoperative blood loss and the risk of incomplete excision of the tumor. A case of angiofibroma limited to the left posterior and superior nasal cavity and nasopharynx was excised transnasally under endoscopic control after pre-operative embolization with Gelfoam(r). The tumor was completely removed without complications, and there was no evidence of any residual tumor or recurrence observed during one year of endoscopic follow-up. It can be suggested that for the limited lesions of angiofibroma, the option of a transnasal endoscopic approach could be cautiously applied by experienced surgeons.
Adolescent ; Angiofibroma ; Follow-Up Studies ; Head ; Humans ; Nasal Cavity ; Nasopharyngeal Neoplasms ; Nasopharynx ; Neck ; Neoplasm, Residual ; Recurrence

Thromboembolic complications (TECs) are clinically important sequelae of nephrotic syndrome (NS). The incidence of TECs in children is approximately 2%-5%. The veins are the most commonly affected sites, particularly the deep veins in the legs, the inferior vena cava, the superior vena cava, and the renal veins. Arterial thrombosis, which is less common, typically occurs in the cerebral, pulmonary, and femoral arteries, and is associated with the use of steroids and diuretics. Popliteal artery thrombosis in children has been described in cases of traumatic dissection, osteochondroma, Mycoplasma pneumoniae infection, and fibromuscular dysplasia. We report of a 33-month-old girl with bilateral iliac and popliteal arterial thrombosis associated with steroid-resistant NS due to focal segmental glomerulosclerosis. Her treatment involved thrombectomy and intravenous heparinization, followed by oral warfarin for 8 months. Herein, we report a rare case of spontaneous iliac and popliteal arterial thrombosis in a young child with NS.
Child* ; Child, Preschool ; Diuretics ; Female ; Femoral Artery ; Fibromuscular Dysplasia ; Glomerulosclerosis, Focal Segmental* ; Heparin ; Humans ; Iliac Artery ; Incidence ; Leg ; Mycoplasma pneumoniae ; Nephrotic Syndrome ; Osteochondroma ; Pneumonia, Mycoplasma ; Popliteal Artery ; Renal Veins ; Steroids ; Thrombectomy ; Thrombosis* ; Veins ; Vena Cava, Inferior ; Vena Cava, Superior ; Warfarin

BACKGROUND: There were several studies for the incidence of gastroesophageal reflux associated with the laryngeal mask airway(LMA), but the results of those studies were much different much different from one another. The aim of this study was to compare the incidence of gastroesophageal reflux and regurgitation of gastric contents between the LMA and the endotracheal tube(ETT). METHOD: Ninety patients scheduled for elective orthopedic surgery with a standardized general anesthetic technique were randomly allocated to receive either a LMA(n-49) or a ETT(n=41) for airway management. The esophageal manometry was carried out for the exclusion of esophageal motility disorders and the 24-hour ambulatory pH metry was done from one day before the operation. A methylene blue(50mg) capsule was swallowed just before the induction and the simultaneous recordings of pH were maintained during anesthesia. At the end of anesthesia, the episodes of regurgitation of gastric contents above hypopharynx were analyzed by the pharyngeal blue staining and the pH metric data were analyzed for the detection of gastroesophageal relux episodes during anesthesia. RESULTS: There was no significant difference in the incidence of gastroesophageal relux(pH< or =4) between two groups; only two patients in LMA and three patients in ETT had reflux episodes during the removal or arousal phase. There was no episode of the pharyngeal blue staining in both group. All of the gastroesophageal reflux patients in both group developed a cough or straining during those phases. There was no clinical evidence of aspiration of gastric contents in both group. CONCLUSION: In comparison with ETT, use of LMA does not appear to increase the incidence of gastroesophageal reflux and regurgitation above hypophryngeal level in positive pressure ventilating patients during long surgical procedures. Therefore, the risk of aspiration in LMA will not be much more than ETT.
Airway Management ; Anesthesia ; Arousal ; Cough ; Esophageal Motility Disorders ; Gastroesophageal Reflux ; Humans ; Hydrogen-Ion Concentration ; Hypopharynx ; Incidence ; Laryngeal Masks* ; Manometry ; Masks* ; Orthopedics

Congenital aniridia is a rare ocular malformation that presents with severe hypoplasia of the iris and various ocular manifestations. Most cases of congenital aniridia are known to be related to mutations in the paired box gene-6 (PAX6), which is an essential gene in eye development. Herein, we report a familial case of autosomal dominant congenital aniridia with four affected members in 3 consecutive generations and describe the detailed ophthalmologic findings for one of these members. As expected, mutational analysis revealed a nonsense mutation (p.Ser122*) in the PAX6 gene. Thus, our findings reiterate the importance of PAX6 mutations in congenital aniridia.
Aniridia* ; Codon, Nonsense ; Family Characteristics ; Genes, Essential ; Humans ; Iris ; WAGR Syndrome ; Wilms Tumor

Ultrasound-guided fine needle aspiration biopsy (USFNA) is a very useful procedure for the diagnosis and management of thyroid diseases. It is very safe procedure and complications have been rarely reported. We experienced a case of an extensive hemorrhage after USFNA of the thyroid nodule in a patient undergoing long-term aspirin therapy. We emphasize that it is very important to obtain a patient history carefully before performing a USFNA.
Aspirin* ; Biopsy* ; Biopsy, Fine-Needle* ; Diagnosis ; Hemorrhage* ; Humans ; Thyroid Diseases ; Thyroid Gland* ; Thyroid Nodule*

Syringocystadenocarcinoma papilliferum (SCACP) is a rare malignant adnexal neoplasm, which is considered as a malignant counterpart of syringocystadenoma papilliferum (SCAP). Clinically, SCACP appears as a nodule, inflammatory plaque, or tumor. The lesion is usually covered with crusts, which are formed by secretion of the apocrine epithelial cells. Histologically, SCACP resembles SCAP, with cystic papillomatous invaginations connected to the skin surface by funnel-shaped structures lined by infundibular epithelium. The stroma of the tumor consists of a dense inflammatory infiltrate of plasma cells and lymphocytes. SCACP differs from SCAP in terms of the architectural and cytological features of the tumor cells, and is characterized by higher nuclear cytoplasmic ratio, nuclear irregularity, coarse chromatin, and increased mitotic activity. However, the immunohistochemical findings of SCACP vary. Since only 49 cases of SCACP have been reported in the English literature, the clinical and histologic characteristics of SCACP have not been fully established. Further studies on the diagnostic criteria for SCACP are warranted. Here, we report a rare case of SCACP and present a review of other relevant literature.
Chromatin ; Cytoplasm ; Epithelial Cells ; Epithelium ; Lymphocytes ; Plasma Cells ; Skin ; Sweat Gland Neoplasms

Infection of cattle with bovine leukemia virus (BLV) has been observed and reported worldwide, including in Korea. The onsite identification of infected cattle would help decreasing and eradicating BLV infections on farms. Here, we present a new immunochromatographic assay that employs monoclonal antibodies (MAbs) for the detection of antibodies against BLV in the field. BLV envelope glycoprotein (gp)51 was expressed in E. coli, and MAbs against recombinant BLV gp51 were generated for the development of an immunochromatographic assay to detect BLV antibodies in cattle. The sensitivity and specificity of the assay were determined by comparing these results with those obtained from a standard enzyme linked immunosorbent assay (ELISA). A total of 160 bovine sera were used to evaluate the new immunochromatographic assay. Using ELISA as a reference standard, the relative specificity and sensitivity of this assay were determined to be 94.7% and 98%, respectively. Because of its high sensitivity and specificity, this BLV antibody detection assay would be suitable for the onsite identification of BLV infection in the field.
Agriculture ; Animals ; Antibodies* ; Antibodies, Monoclonal ; Cattle ; Deltaretrovirus Antibodies ; Deltaretrovirus Infections ; Enzootic Bovine Leukosis* ; Enzyme-Linked Immunosorbent Assay ; Glycoproteins ; Immunochromatography* ; Korea ; Leukemia Virus, Bovine* ; Sensitivity and Specificity

The prognosis for patients with primary central nervous system (CNS) lymphoma (PCNSL) who relapse after the initial response is usually poor. A standard treatment for relapsed PCNSL has not yet been identified because of the heterogeneity of the therapies employed and the lack of large, prospective clinical trials. We describe a 46-year-old relapsed PCNSL patient who was successfully treated with intraventricular applications of rituximab to minimize neurotoxicity, 2 cycles of salvage chemotherapy with etoposide, ifosfamide, and cytarabine (VIA) regimen and high-dose chemotherapy with autologous stem cell rescue. The high-dose chemotherapy consisted of bischloroethylnitrosourea, etoposide, cytarabine, and melphalan (BEAM) regimen. Partial remission was detected after intraventricular rituximab therapy and the patient has been in complete remission without evidence of neurotoxicity for 28 months after high-dose chemotherapy with autologous stem cell rescue. This case indicates a new appropriate treatment guideline in relapsed PCNSL patient after initial intensive chemo-radiotherapy.
Antibodies, Monoclonal/*therapeutic use ; Central Nervous System Neoplasms/*drug therapy/*therapy ; Cytarabine/therapeutic use ; Etoposide/therapeutic use ; Female ; Humans ; Ifosfamide/therapeutic use ; Lymphoma, Non-Hodgkin/*drug therapy/*therapy ; Middle Aged ; Stem Cell Transplantation/*methods

PURPOSE: The organisms causing peritonitis and their antibiotic sensitivities vary in different regions and centers, and these data are necessary to establish regional treatment guidelines. The aim of this study was to investigate the changes in incidence and characteristics of the organisms that cause peritonitis in children undergoing peritoneal dialysis (PD) during recent 10 years. METHODS: We retrospectively collected and analyzed the data from medical records of 110 children on PD during the period from 2000 to 2010. RESULTS: One hundred and forty episodes of peritonitis have occurred in 57 patients. The overall incidence of peritonitis was 0.43 episodes/patient.year, and similar incidence have been maintained since 2003. Sixty percent of the patients experienced peritonitis within 1 year of PD, and all patients commencing PD in infancy experienced peritonitis. Gram positive (G (+)), gram negative (G (-)) organisms and fungi were cultured in 58%, 38%, and 4.1% respectively and cultures were negative in 13.6%. Staphylococcus was the most common G (+) organism, and Pseudomonas and Acinetobacter were 2 most frequent G (-) organisms isolated. Fifty-six percent of the G (+) organisms were sensitive to first generation cephalosporin and 91% of G (-) pathogens were sensitive to ceftazidime. Methicillin-resistance rate was not higher in children less than 2 years of age than in those more than 2 years. CONCLUSION: An additional breakthrough has to be made to further reduce the incidence of peritonitis. Treatment guideline customized for peritonitis in Korean children on PD need to be established through a nationwide co-work.
Acinetobacter ; Ceftazidime ; Child ; Fungi ; Humans ; Incidence ; Medical Records ; Peritoneal Dialysis ; Peritonitis ; Pseudomonas ; Retrospective Studies ; Staphylococcus