Supravalvular aortic stenosis may be defined as an obstructive congenital deformity of the ascending aorta which originates just distal to the level of the origins of the coronary arteries. It may be localized or diffuse, and includes a wide spectrum of pathologic changes. A 16 years old school boy was admitted because of exertional dyspnea for 6 years. Clinical diagnosis of supravalvular and valvular aortic stenosis with bicuspid aortic valves and myocardial hypertrophy was made by echocardiography and angiocardiography. Surgical correction was performed successfully. We presented a case of supravalvular and valvular aortic stenosis with a review of literatures.
Adolescent ; Angiocardiography ; Aorta ; Aortic Stenosis, Supravalvular ; Aortic Valve ; Aortic Valve Stenosis* ; Bicuspid ; Congenital Abnormalities ; Coronary Vessels ; Diagnosis ; Dyspnea ; Echocardiography ; Humans ; Hypertrophy ; Male

PURPOSE: To evaluate the effects of wearing rigid gas permeable (RGP) contact lenses on the topographic changes in keratoconus. METHODS: Seventy-seven keratoconic eyes that wore multicurve RGP contact lenses and 30 keratoconic eyes that wore no contact lenses were retrospectively analyzed. The mean follow-ups were 22.6 and 20.5 months in the lens-wearing and control groups, respectively. Visual acuity, comfort, daily wearing time, and corneal staining were evaluated for both groups. The changes in topographic indices were compared between the lens-wearing and control groups. RESULTS: Multicurve RGP lens corrected logarithm of the minimum angle of resolution visual acuity of the lens-wearing group significantly improved from -0.016+/-0.065 to -0.032+/-0.10 at follow-up (p=0.05). In the lens-wearing group with advanced keratoconus, the Sim Kmax, Sim Kmin, apical power, astigmatic index, and anterior elevation significantly decreased from 57.68+/-4.26 diopter (D), 50.50+/-2.32 D, 62.79+/-5.11 D, 7.20+/-0.55 D and 67.36+/-16.30 microm to 55.51+/-4.28 D, 49.62+/-3.26 D, 60.31+/-5.41 D, 5.90+/-0.51 D and 60.61+/-16.09 microm, respectively (paired t-test, p<0.05). The irregularity index of 3 mm did not significantly change. Meanwhile, in the control group, the apical power and irregularity index increased from 55.56+/-7.25 D and 3.06+/-1.68 D to 57.11+/-7.75 D and 3.25+/-1.71 D, respectively (paired t-test, p=0.008, p=0.01). CONCLUSIONS: Properly fitted multicurve RGP contact lenses are not likely to contribute to the progression of keratoconus.
Adult ; Astigmatism/etiology/pathology/therapy ; *Contact Lenses ; Cornea/*pathology ; *Corneal Topography ; Disease Progression ; Female ; Follow-Up Studies ; Humans ; Keratoconus/complications/pathology/*therapy ; Male ; Prognosis ; Prosthesis Design ; Retrospective Studies ; Time Factors ; Young Adult

PURPOSE: In order to know the clinical characteristics of pediatric optic neuritis in children, an analysis was performed on 23 patients with onset of the disease before the age of 16 years from 1995 to 2000. METHODS: The degree of initial visual loss, subsequent visual recovery, magnetic resonance images and associated diseases were reviewed retrospectively. RESULTS: There was no female predilection in the study group (43%), with a mean age of 8.9 years at presentation. Involvement was bilateral in 87% of patients. A preceding febrile illness within 2 weeks of the onset of visual symptoms was reported in 39% of patients. Of the 43 involved eyes of 23 patients, disc swelling was present in 51%, and central/cecocentral scotoma in 58% of the involved eyes. With a mean follow-up of 14 months, 79% of the patients had final vision of > or = 20/40. Twenty-four (96%) of 25 eyes receiving intravenous steroid treatment, and 10 (56%) out of 18 eyes without intravenous steroids, showed a final visual acuity p> or =20/40. Only one patient (4%) with multifocal signal abnormalities around the periventricular white matter developed multiple sclerosis. CONCLUSIONS: Most of the children with optic neuritis showed bilateral involvement with papillitis. The visual prognosis was relatively good and was particularly better in the patients receiving intravenous steroid treatment than in those without any treatment, however this study was not controlled. Although a longer follow-up period is required, the development of multiple sclerosis is rare in children with optic neuritis.
Child* ; Female ; Follow-Up Studies ; Humans ; Multiple Sclerosis ; Optic Neuritis* ; Papilledema ; Prognosis ; Retrospective Studies ; Scotoma ; Steroids ; Visual Acuity

Recent epidemiologic and clinical students have shown that plasma cholesterol and triglyceride levels are independent risk factors for coronary heart disease. However, there is not much data on the characteristics of anthropometry and lipid profiles of hypercholesterolemic patients with hypertiglyceridemia. In this study, 112 hypercholesterolemic subjects(T-C 240mg/dl) were divided into two groups by their plasma triglyceride levels. We compared the anthropometric measurements and lipid profiles of the subjects between the two groups : the simples hypercholesterolemic group(SHC, TG<200mg/dl) and the combined hypercholesterolemic group(CHC, TG 250mg/dl). The distribution of the subjects into the SHC and CHC groups was 36.6% and 47.3%, respectively. The frequency of the CHC patients decreased with age. The subjects in this group had higher weight, BMI, HWR, cricumferences of mid arm, waist, hip and thigh, and skinfold thicknesses of biceps and triceps than those of the SHC subjects. The difference of plasma total cholesterol level was mainly due to the difference of VLDL-C levels. These differences resulted in the CHC subjects having higher atherogenic indexes and T-C/HDL-C ratios than those of the SHC subjects. Also, the former had higher Apo-B and insulin levels than those the latter. However, blood pressure, fasting blood glucose and HDL-C levels were not significantly different between the two groups. These results suggest that hypercholesterolemic patients with hypertriglyceridemia have riskier lipid profiles for CHD than those of patients with normal triglyceridemia. They also indicate that CHC is closely associated with glucose resistance syndrome(obesity, hyperglycemia, hyperinsulinemia and hypertriglyceridemia), and more prevalent in young people.
Anthropometry* ; Apolipoproteins B ; Arm ; Blood Glucose ; Blood Pressure ; Cholesterol ; Coronary Disease ; Fasting ; Glucose ; Hip ; Humans ; Hypercholesterolemia* ; Hyperglycemia ; Hyperinsulinism ; Hypertriglyceridemia ; Insulin ; Obesity ; Plasma* ; Risk Factors ; Skinfold Thickness ; Thigh ; Triglycerides

PURPOSE: In order to prospectively investigate the causative mechanisms of visual loss in 42 patients referred to rule out optic neuropathy or diagnosed with optic neuropathy in other hospitals. METHODS: Thorough history taking and ophthalmologic examinations were performed. In some patients, evaluation of extraocular movement, nerve fiber layer photography, contrast sensitivity, visual evoked potential, electroretinogram and visual field were tested. RESULTS: Functional visual loss was most common(18 patients), followed by retinopathy(nine patients). Three patients suffered from amblyopia and glaucoma, respectively, two from keratoconus, pseudopapilledema, miscorrected refractive error, and Leber's congenital amaurosis, respectively, and one from cortical visual impairment. In the differential diagnosis of optic neuropathy, history taking and ophthalmologic examinations including pupillary light reflex, color vision test, nerve fiber layer photography, funduscopy and visual field test were useful. Especially the color vision test was very simple, but useful. CONCLUSION: Clinical entities including functional visual loss and retinopathy should be considered when diagnosing visual loss of unknown etiology.
Amblyopia ; Blindness ; Color Vision ; Contrast Sensitivity ; Diagnosis, Differential* ; Evoked Potentials, Visual ; Glaucoma ; Humans ; Keratoconus ; Nerve Fibers ; Optic Nerve Diseases* ; Photography ; Prospective Studies ; Reflex ; Refractive Errors ; Vision Disorders ; Visual Field Tests ; Visual Fields

The amplification of c-myc oncogene was evaluated in 42 cases of ovarian carcinomas to correlate with clinical parameters. Using oligonucleotide primers, sequences from the c-myc exon-3 gene and from a control gene, tissue plasminogen activator (tPA), were amplified simultaneously by polymerase chain reaction (PCR). After the products of differential PCR (d-PCR) were electrophoresed, slot blot hybridization was performed, and hybridized with P32 dATP-labeled myc and tPA oligonucleotide probes and then autoradiographed. The signal intensities of the two products were quantitated by densitometry and the ratios of two products (c-myc/tPA) were measured. The ovarian carcinomas showed significantly increased amplification of c-myc oncogene Oligonucleoti compared to normal control group (p<0.05). 15 of 42 cases (35.7%) showed various degrees of the MYC gene amplification up to 27 folds in various histologic types of ovarian carcinomas. No significant differences of the MYC gene amplification according to histologic subtypes, tumor action) grades and clinical stages of ovarian carcinomas were present.
Densitometry ; DNA Primers ; Genes, myc ; Oligonucleotide Probes ; Oncogenes* ; Polymerase Chain Reaction* ; Tissue Plasminogen Activator

The purpose of this study was to evaluate the canal configuration after shaping by ProFile, ProTaper and K-Flexofile in simulated resin canals with different angles of curvature. Three types of instruments were used : ProFile, ProTaper, K-Flexofile. Simulated root canals, which were made of epoxy resin, were prepared by ProFile, ProTaper with rotary instrument using a crown-down pressureless technique, and hand instrumentation was performed by K-Flexofile using a step-back technique. All simulated canals were prepared up to size 25 file at end-point of preparation. Pre and post instrumentation images were recorded with Scanner. Assessment of canal shape was completed with Image Analysis program. Measurements were made at 1, 2, 3, 4, 5, 6, 7, 8, 9 and 10 mm from the apex. At each level, outer canal width, inner canal width, total canal width, and amount of transportation from original axis were recorded. Instrument deformation and fracture were recorded. Data were analyzed by means of one-way ANOVA analysis of variance and the Sheffe's test. The result was that ProFile and ProTaper maintain original canal shape regardless of the increase of angle of curvature than K-Flexofile. ProFile show significantly less canal transportation and maintained original canal shape better than ProTaper.
Axis, Cervical Vertebra ; Dental Instruments ; Dental Pulp Cavity ; Transportation

PURPOSE: To investigate the clinical characteristics of herpetic keratitis in Korea. METHODS: A retrospective analysis was performed on 90 eyes of 84 patients who were clinically diagnosed with herpetic keratitis and were followed for at least 4 months or more. Information on prior herpetic keratitis, type of keratitis, time to remission, recurrence rate, administration of oral acyclovir, final visual acuity was reviewed. Remission time and recurrence rate were compared according to types of herpetic keratitis and the application of oral acyclovir in epithelial, stromal or endothelial keratitis; and the relation of history of previous herpetic keratitis, recurrence and final vision, was analyzed. RESULTS: Sex ratio (M:F) was 1.31 and the mean age was 54.6 years. Of 90 eyes, the proportion of infectious epithelial keratitis, stromal keratitis, endothelitis and neurotrophic ulcer was 51.1, 17.8, 25.6 and 5.6%, respectively. The mean remission time was 1.92+/-1.01, 4.13+/-5.05, 5.52+/-5.08, 4.00+/-1.00, respectively (p=0.001, one-way Anova). Recurrence occurred in 21 (42.9%) eyes of 49, which were followed up for more than 12 months, in a year after the previous attack. The rates of recurrence of infectious epithelial keratitis, stromal keratitis and endothelitis were 25.0%, 63.6% and 53.8%, respectively. Oral acyclovir neither shortens the remission nor prevents the recurrence. The percentage of final vision over 20/40 in infectious epithelial keratitis, stromal keratitis and endothelitis was 81.1, 57.1 and 60.0%, respectively. The final vision was worse in the group with a history of herpetic keratitis. CONCLUSIONS: Stromal keratitis and endothelitis showed a higher recurrence rate and longer remission time than infectious epithelial keratitis. Because recurrent and severe keratitis may result in corneal opacity and vision loss, aggressive and proper treatment is needed. However, these cases are often resistant to therapy.
Acyclovir ; Corneal Opacity ; Humans ; Keratitis ; Keratitis, Herpetic* ; Korea* ; Recurrence ; Retrospective Studies ; Sex Ratio ; Ulcer ; Visual Acuity

Exposure to light can induce photoreceptor cell death and exacerbate retinal degeneration. In this study, mice with genetic knockout of several genes, including rhodopsin kinase (Rhok-/-), arrestin (Sag-/-), transducin (Gnat1-/-), c-Fos (c-Fos-/-) and arrestin/transducin (Sag-/-/Gnat1-/-), were examined. We measured the expression levels of thousands of genes in order to investigate their roles in phototransduction signaling in light-induced retinal degeneration using DNA microarray technology and then further explored the gene network using pathway analysis tools. Several cascades of gene components were induced or inhibited as a result of corresponding gene knockout under specific light conditions. Transducin deletion blocked the apoptotic signaling induced by exposure to low light conditions, and it did not require c-Fos/AP-1. Deletion of c-Fos blocked the apoptotic signaling induced by exposure to high intensity light. In the present study, we identified many gene transcripts that are essential for the initiation of light-induced rod degeneration and proposed several important networks that are involved in pro- and anti-apoptotic signaling. We also demonstrated the different cascades of gene components that participate in apoptotic signaling under specific light conditions.
Animals ; Apoptosis/radiation effects ; G-Protein-Coupled Receptor Kinase 1/genetics ; GTP-Binding Protein alpha Subunits/genetics ; *Gene Expression Profiling ; Genes, fos/genetics ; Light/adverse effects ; Light Signal Transduction/*genetics/physiology/radiation effects ; Mice ; Mice, Knockout ; Oligonucleotide Array Sequence Analysis ; Retina/metabolism/pathology/radiation effects ; Retinal Degeneration/etiology/*genetics/physiopathology ; Transducin/genetics

A fibromatosis or desmoid tumor of the breast is an extremely rare benign tumor, which is often presented clinically and radiologically as a malignant tumor. Although benign, fibromatosis or desmoid tumors can grow aggressively in local infiltrating patterns. The recurrence is relatively common; hence a wide excision with a clear margin around the tumor is necessary. We report a case of a young female patient with a recurring fibromatosis of the breast after a local excision.
Breast ; Breast Neoplasms ; Female ; Fibroma ; Fibromatosis, Aggressive ; Humans ; Recurrence