No abstract available.
DNA Topoisomerases* ; DNA*

Neurologic complications after open-heart surgery are not only relatively common but also often fatal. In order to know the clinical characteristics and causative risk factors, we carried out the retrospective study on 655 patients who had been admitted and undergone OHS at Seoul National University Children's Hospital during 2-year period from July 1990 to June 1992. The results were as follows; 1) The incidence of neurologic complications after OHS was 4.7% (31 cases) of the total 655 cases. 2) In the viewpoint of the pre-op diagnosis, neurologic complications developed in 20 cases (7.8%) among cyanotic congenital heart disease and in 11 cases (2.9%J among acyanotic CHD. 3) In the viewpoint of the type of operation, the incidence of neurologic complications was 33.3% in Jatene procedure and 12.1% in Fontan procedure. 4) Compared with the group who didn't develp neurologic complications (624 cases), cardiopulmonary bypass time, aorta cross clamp time, and total arrest time were longer and the degree of hypothermia was lower in the group who had neurologic complications (31 cases), which are statistically significant each other(p<0.01). 5) In 25 cases (80.6%), neurologic complications occurred within 72 hours after OHS. 6) Clinical manifestations of neurologic complications were seizures (26 cases, 84%), consciousness change (13 cases, 42%), hemiplegia (1 case), and decreased muscle tone (1 case). 7) Of the 31 cases, 16 cases showed more than one abnormal finding among EEG, Brain CT, or Brain MRI. 8) The possible etiologies of neurologic complications were diffuse hypoxic ischemic encephalopathy (16 cases, 51.6%), focal or multiple ischemic stoke that was thought to be due to microembolisms (3 cases, 9.7%), electrolyte or acid-base imbalance (8 cases, 25.8%), and the remainders were unknown (4 cases. 12.9%) 9) In the course of neurologic complications, 11 cases (35.5%) expired, 14 cases(45.2%) recovered at discharge, 3 cases (9.7%) were controlled at discharge, and 2 cases (6.5%) had long-term sequela(one spastic quadriplegia and one hemiparesis). 10) The cause of death among mortality cases were mainly due to brain damage (7 cases), while 4 cases had other direct causes such as heart failure, respiratory failure, or sepsis.
Acid-Base Imbalance ; Aorta ; Brain ; Cardiopulmonary Bypass ; Cause of Death ; Child* ; Consciousness ; Diagnosis ; Electroencephalography ; Fontan Procedure ; Heart Defects, Congenital ; Heart Failure ; Hemiplegia ; Humans ; Hypothermia ; Hypoxia-Ischemia, Brain ; Incidence ; Magnetic Resonance Imaging ; Mortality ; Muscle Hypotonia ; Quadriplegia ; Respiratory Insufficiency ; Retrospective Studies ; Risk Factors ; Seizures ; Seoul ; Sepsis

BACKGROUND: Fanconi's anemia (FA) is an autosomal recessive disease characterized by aplastic anemia, pre-malignancy, congenital malformations and chromosome breakage syndromes. As up to 30% of patients have no detectable congenital anomalies, the modern diagnosis of FA rests on chromosomal breakage of patient's cells induced by chemical clastogens such as diepoxybutane (DEB) or mitomycin-C (MMC). METHODS: We have done chromosome breakage test to differentiate FA from 11 aplastic anemia, three Diamond-Blackfan syndrome, three myelodysplastic syndrome, one acute leukemia with congenital anomaly and three siblings of FA. The peripheral blood lymphocytes from each individual were co-cultured in phytohemagglutinin-containing medium by the three methods, i.e., DEB treated, MMC treated and un-treated. RESULTS: Five cases were found to have increased chromosomal breakages to DEB and MMC, confirming diagnosis of FA. Other 21 cases showed no increased chromosomal breakages. No overlap was found between FA group and others (P<0.01). In one FA, there was no increased spontaneous breakage, but increased breakage to DEB and MMC. Of five FA, one case showed no congenital anomalies. CONCLUSIONS: Chromosme breakage test was shown to be simple, reliable and useful in ascertaining the diagnosis of FA.
Anemia, Aplastic ; Chromosome Breakage* ; Diagnosis* ; Fanconi Anemia* ; Humans ; Leukemia ; Lymphocytes ; Mitomycin ; Mutagens ; Myelodysplastic Syndromes ; Siblings

No abstract available.

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: To report a rare case of corneal edema caused by amantadine. CASE SUMMARY: A 35-year-old man was diagnosed with hypoxic brain damage caused by ventricular fibrillation. The patient showed Parkinsonism and was started on treatment with amantadine. Thirty-seven months after the commencement of amantadine treatment, the patient suffered a corneal ulcer in his right eye, which healed with opacity and thinning after medical treatment. After healing, slit-lamp examination revealed a bilateral, epithelial and stromal edema without obvious guttae and keratic precipitates. The corneal edema did not improve with topical treatment of 5% NaCl and 0.02% fluorometholone in both eyes. Three months after leaving the hospital, the patient's corrected visual acuity decreased to 0.2 (-2.0 Dsph -0.5 Dcyl Ax 90) in the right eye and 0.4 (-0.75 Dsph -2.0 Dcyl Ax 90) in the left eye. Amantadine medication was discontinued after discussion with the patient's neurologist. At the 1-month follow-up, corneal examination revealed resolution of the epithelial and stromal edema in both eyes. Corrected visual acuity was improved to 0.5 (-1.5 Dsph) in the right eye and 0.7 (-1.0 Dsph -1.0 Dcyl Ax 90) in the left eye. CONCLUSIONS: In cases of corneal edema without an obvious causative disease, the patient's systemic medication list must be reviewed and amantadine should be considered as a possible cause.
Adult ; Amantadine ; Corneal Edema ; Corneal Ulcer ; Edema ; Endothelium, Corneal ; Eye ; Fluorometholone ; Follow-Up Studies ; Humans ; Hypoxia, Brain ; Parkinsonian Disorders ; Ventricular Fibrillation ; Visual Acuity

PURPOSE: To investigate the efficacy of a computerized visual acuity test, the SNU visual acuity test for children. METHODS: Fifty-six children, ranging from 1 to 5 years of age, were included. In a dark room, children gazed at and followed a circular dot with 50% contrast moving at a fixed velocity of 10 pixels/sec on a computer monitor. Eye movement was captured using a charge coupled device camera and was expressed as coordinates on a graph. Movements of the eye and dot were superimposed on a graph and analyzed. Minimum visualized dot diameters were compared to the Teller visual acuity. RESULTS: Ten eyes (8.9%) of six children failed to perform the Teller visual acuity test, and two eyes (1.8%) of one patient failed to perform the SNU visual acuity test. The observed Teller visual acuity and SNU visual acuity were significantly correlated (p < 0.001). Visual angle degrees converted from the Teller visual acuity and SNU visual acuity were also significantly correlated (p < 0.001). CONCLUSION: The SNU visual acuity using moving targets correlated well with Teller visual acuity and was more applicable than the Teller acuity test. Therefore, the SNU visual acuity test has potential clinical applications for children.
Child, Preschool ; Diagnosis, Computer-Assisted/*methods ; Female ; Humans ; Infant ; Male ; Prospective Studies ; Vision Disorders/*diagnosis ; Vision Tests/*methods ; *Visual Acuity

This technique in the conscious volunteers to study the neuromuscular blockade was described in which two different muscle relaxants of ED95X10(-1) in 20ml 0.9% saline were injected simultaneously into one arm and the contralateral arm below the occlusion site of circulation with tourniquet, and then two different neuromuscular blockades were compared following tourniquet released after 80% twitch depression. The benefits of this technique were obtained as following; 1) Simplicity, saving and safety from easily to set up and using low dose of muscle relaxant. 2) Expected results from pure effects of muscle relaxant without any drug interactions. 3) Time shortening of experimental study. 4) Saving time and experiments from simultaneously comparison of control with study group in one experiment. 5) The study of pure receptor binding with muscle relaxant, since a presumed plasma concentration of muscle relaxants is near or actually zero after release of the tourniquet when fresh blood had flushed the limb. Conclusively bilateral isolated forearm technique is elegant method to study the biophase binding interacted of relaxants and cholinerceptor.
Arm ; Depression ; Drug Interactions ; Extremities ; Forearm* ; Neuromuscular Blockade* ; Neuromuscular Blocking Agents ; Plasma ; Tourniquets ; Volunteers

No abstract available.
Catheters* ; Cystic Adenomatoid Malformation of Lung, Congenital* ; Fetal Therapies* ; Lung*

BACKGROUND: Several methods have been used to evaluate the engraftment and to monitor residual disease after bone marrow transplantation (BMT). Among them, karyotyping have been useful in gauging engraftment following opposite sex BMT. More recently, fluorescence in situ hybridization (FISH) has also been applied to determine engraftment and residual status. In order to establish the utility of this method in clinical practice, we have evaluated the data from FISH and several methods. METHODS: We performed FISH using chromosome X alpha-satellite probe (Oncor , USA) on twenty eight peripheral blood and nine bone marrow nuclear cells from eleven patients who underwent sex mis-matched transplant and from a patient who had a loss of X chromosome. RESULTS: In nine patients with well engrafted BMT, signals of host cells showed less than 5% in all patients, evaluated 21-210 days post-transplant. Mixed chimerism was detected in six patients; transiently in early post-transplant period in four, in a patient with engraftment failure, and in a patient with relapse, respectively. CONCLUSION: FISH using X probe is a rapid, quantitative and sensitive 'interphase cytogenetic method' for the evaluation of engraftment and monitoring of residual disease following sex mis-matched BMT or BMT in a patient with a loss of X chromosome; It is especially useful in early post-transplant period when ony a few cells are available during severe cytopenia.
Bone Marrow Transplantation* ; Bone Marrow* ; Chimerism ; Cytogenetics ; Fluorescence* ; Humans ; In Situ Hybridization* ; Karyotyping ; Recurrence ; X Chromosome