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

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*

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

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

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

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

PURPOSE: Urinary lithiasis is uncommon in children, however, it may lead to chronic renal insufficiency and even end stage renal disease. The etiology of stone formation in children is largely unknown; although the most common causes are known to be associated with congenital anomalies of the genito-urinary(G-U) tract, urinary tract infections(UTI), and metabolic diseases. METHODS: A total of 73 children(male:female=42:31, mean age 6.6+/-5.3 years) presented with urinary lithiasis between Sep. 1998 and Jul. 2007 at Seoul National University Children's Hospital. The medical records were reviewed retrospectively. RESULTS: The most common presenting symptoms were gross hematuria(28/73, 38%) and flank or abdominal pain(23/73, 32%). The stones were located in the upper urinary tract in 48 patients(66%), in the bladder in 18(24%), and in both the bladder and upper urinary tract in 2 (3%). Congenital anomalies of the G-U tract with/without UTI were detected in 30 children (41%), hypercalciuria with/without hypercalcemia in 15(20%), and other metabolic diseases in 8(11%). In 17 patients(23%), no underlying cause of stone formation was detected. The majority of stones were infected stones(24/36, 67%), which were followed by calcium stones(8/36, 22%), uric acid stones(3/36, 8%), and cystine stones(1/36, 3%). Thirty-four patients(46%) underwent surgical procedures and/or extracorporeal shockwave lithotripsy for stone removal, and 13(18%) passed stones spontaneously with/without medical management. Stones recurred in 6 patients(8%): 4 with neurogenic bladder augmented by ileocystoplasty, 1 with cystinuria, and 1 with unknown etiology. CONCLUSION: The common causes of urinary lithiasis in children were congenital anomalies of the G-U tract with/without UTI and metabolic disorders including hypercalciuria/hypercalcemia. For the management of stones, minimally invasive procedures should be chosen on the basis of accompanying symptoms and the composition, locations and etiology of stones.
Calcium ; Child* ; Cystine ; Cystinuria ; Humans ; Hypercalcemia ; Hypercalciuria ; Kidney Failure, Chronic ; Lithotripsy ; Medical Records ; Metabolic Diseases ; Recurrence ; Renal Insufficiency, Chronic ; Retrospective Studies ; Seoul ; Uric Acid ; Urinary Bladder ; Urinary Bladder, Neurogenic ; Urinary Tract ; Urolithiasis*

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

BACKGROUND: The purpose of the present study was to determine the effect of positive end-expiratory pressure on intraocular pressure under general anesthesia. METHODS: Contact tonometer HA-1 (Kowa, Japan) was used to measure the intraocular pressures of 22 subjects at zero end-expiratory pressure and positive end-expiratory pressure of 15 cmH2O under general anesthesia. The data were statistically analyzed by paired t-test. RESULTS: There is no statistically significant difference between intraocular pressure of zero end-expiratory pressure and positive end-expiratory pressure of 15 cmH2O in a population with normal basal ocular tonometry. CONCLUSIONS: Mechanical ventilation with positive end-expiratory pressure of 15 cmH2O under general anesthesia dose not present a clinically important significant risk for intraocular pressure increase in a population with normal basal ocular tonometry.
Anesthesia, General* ; Intraocular Pressure* ; Positive-Pressure Respiration* ; Respiration, Artificial* ; Tonometry, Ocular