No abstract available.
Granuloma* ; Leprosy, Lepromatous*

Hypothalamic hamartoma is a congenital anomaly presenting precocious puberty, gelastic seizure and mental retardation. Other possible accompanying anomalies are cleft palate, tetralogy of Fallot, skeletal malformation and anal malformation. But the patient describing here manifested gelastic seizure only with isosignal mass of 1.5cm in diameter situated just behind optic chiasm in T1WI and T2WI of MRI. His EEG showed multifocal spike and sharp waves at left frontal and central area with asymmetric sleep spindles. The seizure has been refractory to conventional antiepileptic drugs. He is now on Lamotrigine, a newly developed antiepileptic drug available.
Anticonvulsants ; Arteriovenous Malformations ; Cleft Palate ; Electroencephalography ; Hamartoma* ; Humans ; Intellectual Disability ; Magnetic Resonance Imaging ; Optic Chiasm ; Paraplegia ; Puberty, Precocious ; Seizures* ; Spine ; Tetralogy of Fallot

We experienced a case of arteriovenous hemangioma showing Dariers sign on the forehead of a 43-year-old man. He presented with a single, 1 * 1.5cm sized, violaceous, asymptomatic nodule with a history of an intermittent wheal at the lesional site. This skin lesion showed Dariers sign clinically and proliferation of mast cells histopathologically with the punch biopsy specirnen suggesting urticaria pig- mentosa. Howerer, we could diagnose it as a arteriovenous hemangioma on complete excision, as the specimen showed arteriovenous proliferation with increased mast cells in a perivascular pattern. This case showed confusing clinical signs and showed the importance of complete excision for the diagnosis of a small skin tumor especially when a vascular proliferating tumor is suspected.
Adult ; Biopsy ; Diagnosis ; Forehead ; Hemangioma* ; Humans ; Mast Cells ; Skin ; Urticaria

No abstract available.
Neural Tube Defects* ; Neural Tube* ; Prenatal Diagnosis*

No abstract available.
Dermatitis, Atopic* ; Food Hypersensitivity ; Milk Hypersensitivity* ; Milk*

Narcotics and Some Neuromuscular blockers are used for open heart sugery anesthesia. Rapid induction of intravenous narcotics techniques may cause some reductjon in blood pressure and systemic vascular resistance, however, the selection of a narcotic and neuromuscular blocker influences subsequent Hemodynamic responses. 72 patients having open heart surgery were investigated using three combination of a narcotic and neuromuscular blocker: Group I (fentanyl 50 ug/kg, pancuronium 100 ug/kg): Group II (fentanyl 50 ug/kg, Vecuronium 80 ug/kg) Group III (sufentanil 10 u/kg, vecuronium 80 u/kg), each combination being administered over 2 minutes. Hemodynamic functions were then monitored for 10 minutes before tracheal intubation. 1) Significant changes included increases in heart nate in the groups receiving pancuronium and decrease in those receiving vecuronium. 2) In all groups mean arterial pressure initially decreased: systemic vascular resistance index decreased significantly in all groups except sufentanil and vecuronium group. 3) Cardiac index decreased significantly only in ufentanil-Vecuronium groups. Time of onset of neuromuscular blockade did not differ among the three groups, but transient chest wall rigidity occurred significantly more often with sufentanil than with fentanyl. Overall, the fentanyl-Pancuronium combination afforded the greatest hemodynamic stability, whereas the sufentanil-Vecuronium Combination proved least Satisfactory because of bradycardia, hypotension, increase of chest wall rigidity.
Anesthesia* ; Arterial Pressure ; Blood Pressure ; Bradycardia ; Cardiovascular System* ; Fentanyl ; Heart* ; Hemodynamics ; Humans ; Hypotension ; Intubation ; Narcotics ; Neuromuscular Blockade ; Neuromuscular Blocking Agents ; Pancuronium ; Sufentanil* ; Thoracic Surgery ; Thoracic Wall ; Vascular Resistance ; Vecuronium Bromide*

OBJECTIVE: Patient' desire of transfusion free surgery has been increasing due to blood transfusion risks. We analyzed the perioperative parameters and perioperative management of transfusion free surgical treatment in Soonchunhyang University Seoul Hospital. METHODS: Operation quantity and blood unstoring count from blood bank between 2000 and 2012 were collected from chronological records. Perioperative parameters including preoperative hemoglobin level, postoperative hemoglobin level, and lowest hemoglobin level were collected from medical records. Perioperative blood management such as acute normovolemic hemodilution, intraoperative blood cell salvage, or hematinic agents and complication were assessed. RESULTS: A total of 3,088 patients underwent transfusion free surgery at Soonchunhyang University Seoul Hospital between 2000 and 2012. Postoperative hemoglobin level <5.0 g/dL were 33 patients. Four patients expired at postoperative period with serious perioperative complications. Average of expired patient's hemoglobin was 3.22 g/dL and overall mortality was 0.12%. Operation was increased as years go by. The amount of blood use bank wasn't increased in general patients with transfusion. CONCLUSION: Careful perioperative blood management for transfusion free surgical treatment was responsible for safety and results in good clinical outcomes. Overall transfusion rate was decreased in spite of increasing operation quantity.
Blood Banks ; Blood Transfusion ; Bloodless Medical and Surgical Procedures ; Hemin ; Hemodilution ; Humans ; Korea ; Medical Records ; Mortality ; Operative Blood Salvage ; Perioperative Care ; Postoperative Period ; Seoul

BACKGROUND: The etiology of chronic urticaria (CU) remains unknown in most patients. Possible causes in some cases include food, but the role of allergy to food antigens in patients with CU remains controversial. OBJECTIVE: The aim of this study was to evaluate the association between food allergy and CU. METHODS: Korean patients with CU were assessed for a previous history of food allergy that caused symptoms of CU. Blood samples were taken from 350 patients to measure food allergen-specific IgE. Based on history and laboratory results, open oral food challenge (OFC) tests were performed. RESULTS: Of 350 participants, 46 (13.1%) claimed to have experienced previous food hypersensitivity. Pork (n=16) was the main food mentioned, followed by beef (n=7), shrimp (n=6), and mackerel (n=6). We found that 73 participants (20.9%) had elevated levels of food-specific IgE, with pork (n=30), wheat (n=25), and beef (n=23) being the most common. However, when the open OFC tests were conducted in 102 participants with self-reported food hypersensitivity or raised levels of food-specific IgE, only four participants showed a positive reaction to pork (n=3) or crab (n=1). CONCLUSION: Although some participants claimed to have a history of CU related to food intake, when an open OFC test was conducted, few of them had positive results. We therefore conclude that food allergy is an uncommon cause of chronic CU.
Eating ; Food Hypersensitivity* ; Humans ; Hypersensitivity ; Immunoglobulin E ; Perciformes ; Prevalence ; Red Meat ; Triticum ; Urticaria*

PURPOSE: This study was aimed at determining whether tri-iodinated compounds with greater solubility and low osmolality, Na or meglumine ioxaglate(Hexabrix 320, Guerbet Lab., France) had deleterious effects on renal function after cardiac angiography for the evaluation of congenital heart disease(CHD) and determining whether correction of dehydration using iv hydration given before cardiac angiography were effective in prventing renal dysfunction. METHODS: For the study of radiocontrast media induced nephrotoxicity, 21 children with various CHDs admitted to the hospital for the evaluation of their CHDs. None of them had any evidence of renal dysfunction before study. They were devided into 2 groups, one was low-dose group(ioxaglate given, < 2.0 mL/kg) and the other one was high-dose group(>2.0 mL/kg). Renal function studies including serum creatinine(Scr), fractional excretion of sodium(FENa) and urinary beta2-microglobulin excreton(mg of beta2- microglobulin per gm creatinine) were done before and after ioxaglate administration. For the study of hydration effect on radiocontrast media induced nephrotoxicity, 9 children with CHDs were subjected. Thery were hydated with 5% D/S 1 hr before cardiac angiography using high-dose ioxaglate(> 2.0 mL/kg) and maintained on fluid therapy during the examination. Renal function studies were done before and after ioxaglate administration. Statistical analyses were done using Wilcoxon signed rank test. RESULTS: 1) Scr level and FENa were not increased significantly after administration of ioxaglate in both of low-dose and high-dose group. 2) A significant increase of urinary beta2M per creatinine excretion(mg/gm creatinine) was observed after administration of ioxaglate n the high-dose group(0.24 vs 0.57, p<0.01) but, not in the low-dose group(0.28 vs 0.35, p>0.05) 3) Hydration before ioxaglate administration made urinary B2M per creatinine excretion insignificant even in patients who received high-dose ioxaglate, more than 3.0 ml/kg(0.22 vs 0.27, p>0.55). 4) Uric acid and/or calcium oxalate crystaluria appeared after ioxaglate administration, particularly in patients with dehydration. CONCLUSIONS: Ioxaglate, a low osmolality ionic dimer contrast media, could induce renal tubular dysfunction, which depended on dose used. Correction of dehydration before ioxaglate administration significantly reduced the risk of ioxaglate induced nephrotoxicity.
Acute Kidney Injury ; Angiography ; Calcium Oxalate ; Child ; Contrast Media* ; Creatinine ; Dehydration ; Fluid Therapy ; Heart ; Humans ; Ioxaglic Acid ; Meglumine ; Osmolar Concentration ; Solubility ; Uric Acid

True midfacial deficiency is defined as a hypoplasia of various components of midface such as maxilla, orbit, zygoma and nasal bone. For treatment of these anomalies Le Fort III osteotomy and its modifications have been used traditionally. Le Fort III osteotomy is the method which advances maxilla with nasal bone and zygomatic bone at a time. At first midfacial osteotomy was introduced by Gillies to treatment of dentofacial deformity in 1950. In 1967 Tessier designed Le Fort III osteotomy according to Le Fort III midfacial fracture line and popularized to treat midfacial deficiency using coronal incision to appoach osteotomy sites. This is a case of patient who had mandibular prognathism with midfacial deficiency with severe discrepancy in maxillomandibular interrelation. First we performed Le Fort III osteomomy for zygomaticomaxillary advancement, and then carried out simultaneous two jaw surgery with Le Fort I osteotomy and BSSRO three months after first surgery.
Dentofacial Deformities ; Humans ; Maxilla ; Nasal Bone ; Orbit ; Orthognathic Surgery ; Osteotomy* ; Prognathism* ; Zygoma