Oguchi's disease is an unusual form of congenital stationary night blindness that is characterized by a peculiar grey-white discoloration of the retina that gives a metallic sheen. After prolonged dark adaptation, the unusual fundus discoloration disappeared and the retina slowly reverted to its original metallic color after exposure to the light. A 20-year-old man was referred for study because of a complaint of night blindness since childhood. As results of diagnostic work up, especially based on typical ocular fundus finding, dark adaptometry and electrophysiologic studies of the retina the authors have diagnosed as a Oguchi's disease. We report this case with the review of the literature.
Dark Adaptation ; Humans ; Night Blindness ; Retina ; Young Adult

Both "caowu", the root of Aconitum kusnezoffii, "chuanwu", the main root of Aconitum carmichaeli are believed to possess anti-inflammatory, analgesics and cardiotonic effects. They have been used to treat conditions such as rheumatism, arthritis, bruises, fractures, hemiplegia. They contain the highly toxic C19 diterpenoid alkaloids of aconitine, mesaconitine and hypaconitine. After ingestion, patients may present with neurological, cardiovascular, gastrointestinal sign and symptoms. A case of thirty six-year-olds healthy women with aconitine poisoning due to caowu that is described presented as transient tetraplegia, arrhythmia and hypocalcemia.
Aconitine* ; Aconitum ; Alkaloids ; Analgesics ; Arrhythmias, Cardiac ; Arthritis ; Contusions ; Eating ; Female ; Hemiplegia ; Humans ; Hypocalcemia* ; Poisoning* ; Quadriplegia* ; Rheumatic Diseases

PURPOSE: To evaluate the optical quality after laser in situ keratomileusis (LASIK) or serial photorefractive keratectomy (PRK) using a double-pass system and to follow the recovery of optical quality after laser vision correction. METHODS: This study measured the visual acuity, manifest refraction and optical quality before and one day, one week, one month, and three months after laser vision correction. Optical quality parameters including the modulation transfer function, Strehl ratio and intraocular scattering were evaluated with a double-pass system. RESULTS: This study included 51 eyes that underwent LASIK and 57 that underwent PRK. The optical quality three months post-surgery did not differ significantly between these laser vision correction techniques. Furthermore, the preoperative and postoperative optical quality did not differ significantly in either group. Optical quality recovered within one week after LASIK but took between one and three months to recover after PRK. The optical quality of patients in the PRK group seemed to recover slightly more slowly than their uncorrected distance visual acuity. CONCLUSIONS: Optical quality recovers to the preoperative level after laser vision correction, so laser vision correction is efficacious for correcting myopia. The double-pass system is a useful tool for clinical assessment of optical quality.
Adult ; Female ; Humans ; *Keratomileusis, Laser In Situ ; Male ; Middle Aged ; Myopia/*surgery ; *Photorefractive Keratectomy ; Postoperative Complications ; Prospective Studies ; *Recovery of Function ; Treatment Outcome ; *Visual Acuity ; Young Adult

Patients with chronic renal failure(CRF) suffer from muscular dysfunction of varying degrees. 31P-nuclear magnetic resonance(NMR) spectroscopic studies of skeletal muscle have shown that these patients have less energy reservoir. Chronic metabolic acidosis has been known to decrease intracellular pH in rat muscle. We investigated the effect of correction of metabolic acidosis with sodium bicarbonate on muscle energy metabolism. Eight patients(6M, 2F) with CRF(creatinine clearance <20ml/min/1.73m2) who were clinically stable and had serum bicarbonate level less than 18mEq/L were included. The median age was 48 years (range; 29-56 years). The patients were treated with daily doses of 3 gram sodium bicarbonate for 3 months. Phosphocreatine to inorganic phosphate ratio(PCr/Pi) and intracellular pH were measured with 31P-NMR spectroscopy, at resting, nadir and maximum recovery state after all-out exercise, before and after correction of acidosis. Plasma bicarbonate level increased from 16.2+/-0.9 to 21.9+/-1.1mmol/L(p<0.05). Creatinine clearance, hematocrit and serum albumin did not change during the study period. The exercise capacity, as measured by the time holding the weight, did not improve with sodium bicarbonate treatment. PCr/Pi ratio at resting state was 7.0+/-1.9 before treatment, which did not change after treatment, 6.2+/-0.8, although plasma bicarbonate level increased significantly. The ratio reached bottom immediately after the all-out exercise(the nadir state) and then recovered toward preexercise value. The PCr/Pi ratio at nadir and maximum recovery state were not different between before and after treatment. The intracellular pH became more acidotic immediately after exercise, but no difference was found between before and after sodium bicarbonate treatment in resting state, nadir or maximum recovery state. With our results, we conclude that the partial correction of acidosis does not improve energy reserve and serum albumin in CRF patients.
Acidosis* ; Animals ; Creatinine ; Energy Metabolism* ; Hematocrit ; Humans ; Hydrogen-Ion Concentration ; Kidney Failure, Chronic* ; Muscle, Skeletal ; Phosphocreatine ; Plasma ; Rats ; Serum Albumin ; Sodium Bicarbonate ; Spectrum Analysis

PURPOSE: To evaluate the role of bile duct obstuction in the development of atrophy of the liver, using ananimal model. MATERIALS AND METHODS: Seven rabbits were divided into two groups : group 1(n=5), in which therewas selective bile duct ligation, and group 2(n=2), which underwent a sham operation. Each group was evaluated using CT for changes in hepatic volume after selective bile duct ligation or a sham operation. In group I, the diameter of dilated bile duct was measured 2, 4, 8, 12 and 16 weeks after bile duct ligation, while gross andhistologic change were evaluated in all cases. RESULTS: In group 1, bile duct dilatation was seen on CT two weeks after selective bile duct ligation, and did not change significantly during follow-up. In four of five cases, CT revealed no evidence of significant atrophy of the involved segment. Pathologic specimens, however, revealed dilatation of the bile duct, periductal fibrosis, infiltration of chronic inflammatory cells, and periportalfibrosis. One of five cases showed segmental liver atrophy after selective bile duct ligation. In addion to the above pathologic findings, there was obstruction of the portal vein by foreign body reaction. In group 2, no evidence of dilated bile duct or liver atrophy was revealed by CT or pathologic specimen after a sham operation. CONCLUSION: During long-term follow-up of 16 weeks, obstruction of the bile duct did not play a major role in the development of lobar atrophy in the rabbit.
Animals ; Atrophy ; Bile Ducts* ; Bile* ; Dilatation ; Fibrosis ; Follow-Up Studies ; Foreign-Body Reaction ; Ligation* ; Liver ; Portal Vein ; Rabbits

PURPOSE: To evaluate the CT and US features of the colonic villous tumors. MATERIALS AND METHODS: We retrospectively reviewed the CT findings of 11 cases with histologically proved colonic villous tumor. CT parameters evaluated were morphological appearances and enhancing pattern (size, shape, margin, presence or absence of fronds, bowel wall thickening). CT features of six cases with malignant change were compared with five tumors without malignant change. US features available in 10 patients were also analyzed. RESULTS: On CT, thetumors showed irregular margin(n=9), presence of fronds(n=6), lobulated shape(n=11), with pericolonic invasion(n=1). Six cases with malignant change were larger(mean, 6.8cm in diameter) than those without malignant change(mean, 3.3 cm). US features in 10 cases were intraluminal mass(n=5), colonic wall thickening(n=5), with variable echogenicity. CONCLUSIONS: Colonic villous tumor appeared as a nonspecific mass on CT and US with adifficulty in distinguishing from colon carcinoma.
Colon* ; Humans

PURPOSE: To evaluate whether the presence of a peripheral echogenic rim surrounding internal low echogenicityis helpful in the diagnosis of atypical hepatic hemangioma. MATERIALS AND METHODS: Within a two-year period, aretrospective review of the sonographic appearances of 24 atypical hemangiomas in 21 patients was performed. Diagnosis was made by dynamic and delayed enhanced CT, MR imaging or clinical follow up for one year, including follow-up sonogram. The sonographic appearances of these hemangiomas were analyzed for pattern and thickness ofthe echogenic rim, internal echo pattern, shape, and size. Additionally, forty six lesions of hepatoma, metastasis, abscess and intrahepatic cholangiocarcinoma confirmed by pathology within the same period were also evaluated for the presence of echogenic rim. RESULTS: Twenty-three of 24 lesions showed an echogenic rim ; these were thick in nine cases, and thin in 14 cases. The thickness of the rim was either uniform(n=10) or eccentric(n=13). The average maximum diameter of atypical hemangioma was 4.4cm (range of diameters, 1.5-12cm). The internal echo pattern was partially or entirely hypoechoic in 15 lesions, homogeneously isoechoic in three, and mixed insix cases. Twenty-one lesions were round, and the shape of three was irregular. The other forty six masses including hepatocellular carcinoma, metastases, cholangiocarcinoma and hepatic abscesses did not show an echogenicrim. CONCLUSIONS: The presence of a sonographic echogenic rim in a hepatic mass may help in the diagnosis ofhepatic hemangioma.
Abscess ; Carcinoma, Hepatocellular ; Cholangiocarcinoma ; Diagnosis ; Follow-Up Studies ; Hemangioma* ; Humans ; Liver Abscess ; Magnetic Resonance Imaging ; Neoplasm Metastasis ; Pathology ; Ultrasonography*

PURPOSE: Chest pain is common in children and adolescents and is a reason for referral to pediatric cardiologists. Although most cases of chest pain in these age groups are benign and do not require treatment, timely diagnosis is important not to miss life-threatening diseases requiring prompt treatment. We investigated certain clinical characteristics that may be useful in the diagnosis of such critical diseases. METHODS: Patient medical records between July 2006 and September 2013 were retrospectively examined. We included 517 patients who presented with chest pain to the Department of Pediatrics at Kyung Hee University Hospital in Gangdong. RESULTS: Most cases of chest pain were idiopathic in origin (73.6%), followed by cases with respiratory (9.3%), musculoskeletal (8.8%), cardiac (3.8%), gastrointestinal (2.9%), and psychiatric (1.4%) causes. In 6 patients (1.2%) with air-leak syndrome including pneumothorax or pneumomediastinum, the pain was abrupt, continuous, and lasted for a short period of 1-2 days after onset in the older adolescents. Of the patients with cardiac pain, 13 had cardiac arrhythmias (65.0%), 6 had congenital heart diseases (30%), and 1 had coronary aneurysms caused by Kawasaki disease (5.0%). One patient with atrial flutter had only symptoms of syncope and chest pain. CONCLUSION: The abrupt, continuous chest pain of a short duration in the older children was characteristic of air-leak syndrome. In patients with pneumomediastinum, radiological diagnosis was difficult without careful examination. Combined syncope should not be neglected and further cardiac workup is essential in such patients.
Adolescent* ; Arrhythmias, Cardiac ; Atrial Flutter ; Chest Pain* ; Child* ; Coronary Aneurysm ; Diagnosis ; Heart Diseases ; Humans ; Mediastinal Emphysema ; Medical Records ; Mucocutaneous Lymph Node Syndrome ; Musculoskeletal Diseases ; Pediatrics ; Pneumothorax ; Referral and Consultation ; Retrospective Studies ; Syncope ; Thorax*

BACKGROUND: To avoid complications of homologous transfusion, many methods are used in patients who undergo an operation, but the autologous transfusion is most popular. This retrospective study was done to evaluate the applicability of an autologous transfusion in an orthopedic spinal surgery. METHODS: The cases of 239 autologous transfusion and 85 homologous transfusions in patients who had spinal surgery due to spinal stenosis were reviewed, and the differences in homologous transfusion, postoperative drainage and complications in both groups were compared. Both groups were analysed and compared by the T-test and Mann-Whitmann rank sum test. RESULTS: In the homologous transfusion group, 4.6 +/- 2.7 units of RBC products were used in 85 patients. In the autologous transfusion group more than 2 techniques of preoperative deposit, intraoperative autotansfusion by cell saver, acute normovolemic hemodilution, and postoperative autotransfusion were used, and 3.6 +/- 2.4 units of RBC products were infused to 49 out of 239 patients (P < 0.001). A postoperative hematoma occured in 2 patients after a homologous transfusion but there were no cases in autologous transfusions. CONCLUSION: Consequently much of the homologous transfusion could be saved by using an autologous transfusion, and smaller amounts of postoperative drainge occured, so the author could confirm the benefit of autologous transfusion.
Blood Transfusion, Autologous* ; Drainage ; Hematoma ; Hemodilution ; Humans ; Orthopedics ; Retrospective Studies* ; Spinal Stenosis

Annuloaortic ectasia, cystic medial degeneration of the afflicted aortic wall leading to progressive dilatation, is often accompanied by Marfan's syndrome. Some portions of intimal flap is commonly demonstrated along the aorta in the noninvasive diagnosis of aortic dissection. We report the first case of circumscribed aortic dissection developed in a 28 year old obese non-Marfanoid patient. He was transferred after thrombolytic therapy at a community hospital because of severe chest pain and ST segment elevation. Transthoracic echocardiography showed markedly dilated aortic root, moderate amount of pericardial effusion, mild aortic regurgitation in spite of normal regional wall motion of left ventricle. Intimal flap, characteristic of aortic dissection, was not seen with computed tomography. Intimal tear was demonstrated just above aortic valve only by transesophageal echocardiography. Two parallel intimal tear and small circumscribed dissection was demonstrated by autopsy.
Adult ; Aorta ; Aortic Valve ; Aortic Valve Insufficiency ; Autopsy ; Chest Pain ; Diagnosis ; Dilatation ; Dilatation, Pathologic* ; Echocardiography ; Echocardiography, Transesophageal ; Heart Ventricles ; Hospitals, Community ; Humans ; Marfan Syndrome ; Pericardial Effusion ; Thrombolytic Therapy