A pair of 19-year-old female identical twins was referred to our hospital with progressive visual loss. They exhibited bilateral chorioretinal atrophy involving the midperiphery on fundoscopy and fluorescein angiography. Bilateral visual field constriction was noted on dynamic Goldmann perimetry, and a markedly impaired response was observed on both photopic and scotopic electroretinograms. Cystoid macular edema was identified in both eyes on optical coherence tomography. Plasma levels of ornithine were elevated. Based on these observations, the patients were diagnosed with gyrate atrophy of the choroid and retina. The clinical diagnosis was confirmed by mutation analysis of the ornithine-delta-aminotransferase (OAT) gene. Patients were treated with a pyridoxine supplement (300 mg/day) and an arginine-restricted diet to lower plasma levels of ornithine, which were successfully reduced without progression of chorioretinal atrophy for 15 months. Our report describes the first case of gyrate atrophy in the Korean population diagnosed by OAT gene analysis and treated with vitamin B6 dietary supplementation.
DNA/*analysis ; DNA Mutational Analysis ; Diagnosis, Differential ; Electroretinography ; Female ; Fluorescein Angiography ; Fundus Oculi ; Gyrate Atrophy/*diagnosis/enzymology/genetics ; Humans ; *Mutation ; Ornithine-Oxo-Acid Transaminase/*genetics/metabolism ; Tomography, Optical Coherence ; Visual Acuity ; Young Adult

PURPOSE: To evaluation the accuracy of the IOL power calculation formulae measured by IOL Master(R) and applanation ultrasonography for the Tecnis ZCB00 IOL. METHODS: We performed a retrospective study of 170 eyes in 121 patients who underwent cataract surgery in our hospital with AMO Tecnis ZCB00 IOL.s. The SRK/T formula was used to predict the patient's implanted IOL power. Differences in the predicted refractive errors between IOL Master(R) and ultrasonography were analyzed and factors attributed to the differences were also analyzed. Three months after cataract surgery, mean numeric error and mean absolute error were analyzed. RESULTS: SRK/II and SRK/T formulas calculated using ultrasonography showed differences compared to the same formulas calculated using IOL Master(R), in which hyperopic shift was also demonstrated. No definite factor was attributed to the differences between the 2 methods. Although the 3 formulas of IOL Master(R) showed no significant difference in refractive errors, the SRK/T formula calculated using IOL Master(R) showed the least mean absolute and numeric errors. CONCLUSIONS: IOL Master(R) is considered more suitable when determining proper AMO Tecnis ZCB00 IOL power in cataract surgery. The hyperopic shift should be considered when calculating the IOL power using only ultrasonography.
Cataract ; Ultrasonography* ; Humans ; Lenses, Intraocular* ; Refractive Errors ; Retrospective Studies ; Ultrasonography

PURPOSE: To manage a critically ill patient effectively, an emergency physician must make an accurate assessment of the patient's hemodynamic status. Although the hemodynamic status can be accurately measured through the use of a pulmonary artery catheter, this device has significant limitations in the emergency department. Instead, the most commonly used method of measuring cardiac output (CO) in the emergency department is transthoracic echocardiography (TTE). However, TTE has been reported to underestimate the CO. Impedance cardiography (ICG) is an established and accurate technique for noninvasive determination of hemodynamic parameters. Thus, this study aimed to assess the agreement between CO measurements made by using TTE and ICG. METHODS: In 20 healthy volunteers, the hemodynamic parameters (stroke volume and CO) obtained by TTE were compared with the parameters derived from simultaneous measurements by ICG. RESULTS: The mean stroke volumes and COs were 63.9+/-11.6 ml and 4.1+/-0.7 L/min, respectively, by TTE, and 77.2+/-10.2 ml and 5.0+/-0.6 L/min by ICG. The correlation coefficients for stroke volume and CO between TTE and ICG were 0.77 and 0.64. The mean difference in stroke volume between TTE and ICG was -13.3+/-6.4 ml (-19.5+/-10.5 %), and the mean difference in CO was -0.88+/-0.48 L/min (-20.1+/-11.6 %). CONCLUSION: In healthy volunteers, the comparison between TTE and ICG showed a good correlation for stroke volume and CO. However, the mean differences in stroke volume and CO between TTE and ICG were -19.5+/-10.5 % and -20.1+/-11.6 %, respectively.
Cardiac Output* ; Cardiography, Impedance ; Catheters ; Critical Illness ; Echocardiography* ; Emergencies ; Emergency Service, Hospital ; Healthy Volunteers ; Hemodynamics ; Humans ; Male* ; Pulmonary Artery ; Stroke Volume ; Volunteers*

PURPOSE: In the present study, the incidence and risk factors of lens-iris diaphragm retropulsion syndrome (LIDRS) were evaluated. METHODS: Patients who underwent cataract surgery using phacoemulsification between June 2014 and December 2014 were included in the study. The preoperative ocular biometric and intraoperative surgical parameters were examined. The incidence of LIDRS and various risk factors were analyzed using an independent t-test, Pearson's chi-square test, and univariable and multivariable logistic regression analyses. RESULTS: Among 124 eyes of 124 patients, 100 (80.6%) had no LIDRS and 24 (19.4%) had LIDRS. LIDRS occurred in 13 of 31 vitrectomized eyes (41.9%) and 11 of 93 non-vitrectomized eyes (11.8%). Based on univariable analysis, age (odds ratio [OR], 0.920; p = 0.001), vitrectomized eye (OR, 5.038; p = 0.001), spherical equivalent (OR, 0.778; p < 0.001), axial length (OR, 1.716; p < 0.001), anterior chamber depth (OR, 3.328; p = 0.037), and 3.0 mm vs. 2.2 mm incision size (OR, 4.964; p = 0.001) were statistically significant risk factors associated with the development of LIDRS. Conditional multivariable logistic regression showed that vitrectomized eye (OR, 3.865; 95% confidence interval [CI], 1.201 to 12.436; p = 0.023), long axial length (OR, 1.709; 95% CI, 1.264 to 2.310; p = 0.001), and 3.0 vs. 2.2 mm incision size (OR, 3.571; 95% CI, 1.120 to 11.393; p = 0.031) were significant independent risk factors associated with LIDRS. CONCLUSIONS: LIDRS is a relatively common occurrence and was found to be associated with vitrectomized eye, long axial length, and larger incision size. Evaluating risk factors prior to cataract surgery can help reduce associated morbidity.
Anterior Chamber ; Cataract ; Diaphragm* ; Humans ; Incidence* ; Iris ; Logistic Models ; Phacoemulsification* ; Risk Factors*

PURPOSE: To evaluate the relations between progression of glaucoma in visual field and peripapillary area change in normal tension glaucoma (NTG). METHODS: We respectively evaluated 66 patients (66 eyes) with normal tension glaucoma; these patients were classified as progressive glaucoma group and non-progressive glaucoma group by visual field test. Interobserver and intraobserver agreement was evaluated for qualitative assessment. Zone beta change was measured by Image J (National Institute of Health, Bethesda, USA) by two methods. One method used paired t-test and the other method used CV (correlation of variation) to define significant progression of zone beta. RESULTS: 46 patients (46 eyes) were classified as progressive glaucoma and 20 patients (20 eyes) were classified as non-progressive glaucoma. There was no statistically significant different change of zone beta between two groups and there were no changes of zone beta over coefficient of variation (CV). CONCLUSIONS: The measurement of change of zone beta seems to be of no use in recognition of progression in normal tension glaucoma.
Atrophy ; Glaucoma ; Humans ; Low Tension Glaucoma ; Visual Field Tests ; Visual Fields

PURPOSE: A number of studies have examined the B-type natriuretic peptide level in dialysis patients and in patients with lesser degrees of renal insufficiency. However, relationships between BNP and renal function are unknown. We sought to assess the diagnostic utility of BNP in differentiating congestive heart failure (CHF) from non-congestive heart failure (non-CHF) in patients with renal insufficiency. METHODS: BNP levels were obtained in 395 patients presenting to our emergency department with dyspnea. Of those 395 patients, 48 patients showed renal insufficiency. Patients transferred to other hospitals and those in a donot- resuscitate state were excluded. RESULTS: In patients with acute renal failure, patients with CHF (n=8) had BNP levels of 360+/-254 pg/ml whereas patients with non-CHF (n=3) had BNP levels of 114+/-103 pg/ml; however, this difference was not statistically significant. In patients with chronic renal failure, patients with CHF (n=22) had BNP levels of 1147+/-635 pg/ml, which was significantly higher than the BNP levels of 459+/-508 pg/ml for patients with non-CHF (n=7) (p=0.01). The area under the receiver operating curve, which plots sensitivity versus specificity for BNP levels in separating congestive heart failure from non-congestive heart failure in patients with chronic renal failure, was 0.805 (p=0.01). The diagnostic accuracy of BNP at a cutoff of 600 pg/ml was 76 %. CONCLUSION: The BNP cut-off value for diagnosis of CHF in patients with chronic renal failure is 600 pg/ml.
Acute Kidney Injury ; Diagnosis* ; Dialysis ; Dyspnea ; Emergency Service, Hospital ; Estrogens, Conjugated (USP)* ; Heart Failure* ; Humans ; Kidney Failure, Chronic ; Natriuretic Peptide, Brain* ; Renal Insufficiency ; Sensitivity and Specificity

PURPOSE: To assess the refractive change and prediction error after temporary intraocular lens (IOL) removal in temporary polypseudophakic eyes using IOL power calculation formulas and Gills' formula. METHODS: Four consecutive patients (7 eyes) who underwent temporary IOL explantation were enrolled. Postoperative refractions calculated using IOL power calculation formulas (SRK-II, SRK-T, Hoffer-Q, Holladay, and the modified Gills' formula for residual myopia and residual hyperopia) were compared to the manifest spherical equivalents checked at 1 month postoperatively. RESULTS: The mean ages of temporary piggyback IOL implantation and IOL removal were 6.71 +/- 3.68 months (range, 3 to 12 months) and 51.14 +/- 18.38 months (range, 29 to 74 months), respectively. The average refractive error was -13.11 +/- 3.10 diopters (D) just before IOL removal, and improved to -1.99 +/- 1.04 D after surgery. SRK-T showed the best prediction error of 1.17 +/- 1.00 D. The modified Gills' formula for myopia yielded a relatively good result of 1.47 +/- 1.27 D, with only the variable being axial length. CONCLUSIONS: Formulas to predict refractive change after temporary IOL removal in pediatric polypseudophakia were not as accurate as those used for single IOL implantation in adult eyes. Nonetheless, this study will be helpful in predicting postoperative refraction after temporary IOL removal.
Cataract/*congenital ; *Cataract Extraction ; *Device Removal ; Female ; Humans ; Hyperopia/etiology/*surgery ; Infant ; Lens Implantation, Intraocular/*methods ; Lenses, Intraocular ; Male ; Myopia/etiology/*surgery ; Prospective Studies

PURPOSE: To assess the refractive change and prediction error after temporary intraocular lens (IOL) removal in temporary polypseudophakic eyes using IOL power calculation formulas and Gills' formula. METHODS: Four consecutive patients (7 eyes) who underwent temporary IOL explantation were enrolled. Postoperative refractions calculated using IOL power calculation formulas (SRK-II, SRK-T, Hoffer-Q, Holladay, and the modified Gills' formula for residual myopia and residual hyperopia) were compared to the manifest spherical equivalents checked at 1 month postoperatively. RESULTS: The mean ages of temporary piggyback IOL implantation and IOL removal were 6.71 +/- 3.68 months (range, 3 to 12 months) and 51.14 +/- 18.38 months (range, 29 to 74 months), respectively. The average refractive error was -13.11 +/- 3.10 diopters (D) just before IOL removal, and improved to -1.99 +/- 1.04 D after surgery. SRK-T showed the best prediction error of 1.17 +/- 1.00 D. The modified Gills' formula for myopia yielded a relatively good result of 1.47 +/- 1.27 D, with only the variable being axial length. CONCLUSIONS: Formulas to predict refractive change after temporary IOL removal in pediatric polypseudophakia were not as accurate as those used for single IOL implantation in adult eyes. Nonetheless, this study will be helpful in predicting postoperative refraction after temporary IOL removal.
Cataract/*congenital ; *Cataract Extraction ; *Device Removal ; Female ; Humans ; Hyperopia/etiology/*surgery ; Infant ; Lens Implantation, Intraocular/*methods ; Lenses, Intraocular ; Male ; Myopia/etiology/*surgery ; Prospective Studies

PURPOSE: To report the first case of lattice corneal dystrophy, gelsolin type in Korea. CASE SUMMARY: A 61-year-old man visited our clinic with severe dry eye symptom in both eyes. Clinical examination revealed in both eyes a visual acuity of 0.7 without correction and intraocular pressure of 18 mm Hg. On slit-lamp examination, both corneas had scattered lattice lines at various depths within the stroma with punctate epithelial erosions. The patient had characteristic features of Meretoja syndrome, including cranial neuropathy characterized by dermatochalasis and facial weakness, and was positive for the gelsolin mutation according to DNA analysis. This is the first description of a patient with lattice corneal dystrophy, gelsolin type in Korea. CONCLUSIONS: This is the first description of a patient with lattice corneal dystrophy, gelsolin type in Korea and demonstrates the importance of recognizing the systemic and ophthalmic features for appropriate management of the condition.
Amyloidosis ; Cornea ; Corneal Dystrophies, Hereditary ; Cranial Nerve Diseases ; DNA ; Eye ; Gelsolin ; Humans ; Intraocular Pressure ; Korea ; Visual Acuity

BACKGROUND: It is known that there is a pronounced circardian periodicity for the time of onset of acute myocardial infarction(AMI), with prominent increase in incidence of onset in the morning hours. However, the characteristic circardian variability in AMI is blunted in patients receiving beta-blockers or aspirin therapy before their presentation with AMI. These findings are attributed to the increase in platelet aggregability, blood coagulability, and plasma catecholamine that change coronary tone and myocardial oxygen demand. We hypothesize that, in addition to above physiologic and biochemical parameters, morphologic patterns of the coronary artery lesions are related to the development of circardian variation in AMI. METHOD: Subjects were 160 patients with AMI(male 92, female 68, mean age 56.9 +/-10.5 years old). Patients were classified by the time of onset of typical chest pain(AMI) by 6-hour interval from mid-night. Circardian variability of onset of AMI was compared with clinical findings and coronary angiographic findings. RESULTS: Incidence of onset of AMI was most frequent in the morning hours(6AM-noon,42.5%). There was no difference in degree of stenosis, lesion length, incidence of intraluminal thrombus, among 3 subgroups of AMI according to time of attack. Morning hour group had more frequent ulceration of coronary lesion than that of other groups(22.4% vs. 5.4%, p<0.01), and less frequent calcified lesion than that of other groups(3.0% vs 5.4%, p<0.05). Normal or minimal coronary artery lesion, that is Iess than 25% stenosis, was more frequent in the morning hour group comparing to that of other groups(11.9% vs. 9.78%). Eccentric stenosis(15.7% vs, 11,1%) and diffuse irregular lesion(25.5% vs. 16.7%) tended to be more frequent in the morning hour group. There were no differences in sex, age, incidence of hypertension, cigarette smoking, diabetes, degree of alcohol ingestion, ejection fraction, maximal CK value, preinfarction angina duration, past history of MI, and in incidence of arrhythmia. CONCLUSIONS: There were more ulcerative coronary atherosclerotic lesions, but fewer calcified coronary lesions in the morning group than in afternoon and night group. These findings indicate that morphology of coronary artery lesions may play a role in causing circardian variation in AMI.
Angina, Unstable ; Arrhythmias, Cardiac ; Aspirin ; Blood Platelets ; Constriction, Pathologic ; Coronary Vessels* ; Eating ; Female ; Humans ; Hypertension ; Incidence ; Myocardial Infarction* ; Oxygen ; Periodicity ; Plasma ; Smoking ; Thorax ; Thrombosis ; Ulcer