OBJECTIVES: Hemophilia B has been known to result from more than 500 kinds of mutations. And it is difficult to find out a mutation specific for each family. Therefore, linkage analysis of DNA polymorphism within or near the factor IX gene has been frequently used in the clinical practice for molecular genetic diagnosis of hemophilia B. But the ethnic variation makes more difficult to apply useful markers in Caucasian population. To investigate the usefulness of the MseI and HhaI polymorphism in Korean population, we analysed the MseI and HhaI polymorphism. METHODS: Forty-five normal Korean and thirteen parents of the hemophilia B patients, using PCR and restriction enzyme analysis. RESULTS: The heterozygosity rate of MseI polymorphism was 49.7% and that of HhaI polymorphism was 25.5%. CONCLUSION: These data indicated that PCR-based analysis of MseI and HhaI polymorphism of factor IX was useful in molecular genetic diagnosis of hemophilia B in Korean population.
Diagnosis* ; DNA* ; Factor IX* ; Hemophilia A* ; Hemophilia B* ; Humans ; Molecular Biology* ; Parents ; Polymerase Chain Reaction ; Prenatal Diagnosis ; Restriction Mapping

Epiblepharon is characterized by horizontal fold of skin adjacent to lid margin. It is a common congenital anomaly, especially in Orientals. In comparison with it, entropion is characterized by an absent lid crease and an inward rolling of the entire eyelid margin. The incidence of the latter was not reported but it was said to be very rare. Not with standing that, congenital entropion has been diagnosed more frequently than epiblepharon in Korea. Supposing that the cases diagnosed as entropion in Korea might fall under the category of epiblepharon on the basis of the authors' clinical experience, the clinical evaluation of the 190 cases operated under the diagnosis of congenital entropion at Seoul National University Hospital from January 1976 to May 1986 was done. The result was that 147 cases(77.4%) were thought to be epiblepharon. So more careful examination might be necessary in diagnosing epiblepharon or entropion in Orientals.
Diagnosis ; Entropion ; Eyelids ; Incidence ; Korea ; Seoul ; Skin

We noticed a group of thoracolumbar and lumbar spine fractures showing a unique fracture pattern that consisted of 1) superior disc injury, 2) crush fracture of the upper half of the vertebral body, 3) sagittal fracture of the lower half of the vertebral body, 4) bone fragments in the spinal canal, and 5) lamina fracture. Some of these fracture patterns were present in type B burst fracture of Denis classification. The purpose of this study was to compare the difference between crush-cleavage fracture and type B burst fracture in the viewpoint of neural canal involvement and neurologic status, and functional outcome with surgical treatment. Ten cases were identified as crush-cleavage fractures in 22 type B burst fractures during a 5 year period from Mar. 1989 to Jun. 1993 at the Department of Orthopedic Surgery of Chonbuk National University Hospital. Four out of 10 crush-cleavage fractures and 4 out of 12 type B burst fractures were paraparetic. Crush-cleavage fracture must be an unstable thoracolumbar comminuted fracture, but there were no significant differences in the neural canal involvement, incidence of neurologic deficit, and in the functional outcome, compared with type B burst fracture (P>0.05). It could be classified as a progressed form of type B burst fracture in Denis classification, or as a burst-split fracture in Magerl classification in the anatomical viewpoint. In conclusion, crush-cleavage fracture must be a variant of burst fracture.
Classification ; Fractures, Comminuted ; Incidence ; Jeollabuk-do ; Neural Tube ; Neurologic Manifestations ; Orthopedics ; Spinal Canal ; Spine

Liver has a dual blood supply from portal vein and hepatic artery. Hepatocellular carcinoma receive their blood supply almost exclusively from hepatic artery. Thus, the concept of treating hepatocellular carcinoma by chemoembolization through these arteries is very effective. However, there may be several collateral or parasitic vessels feeding them in case of huge tumor or previous chemoembolization. We experierced a case of huge tumor involving right upper posterior portion of liver fed by 9th, 10th, 11th right posterior intercostal arteries and an anomalous hepatic artery. We tried chemoembolization with Adriamycin-Lipiodol suspension and Gelfoam material through the right posterior intercostal arteries to treat the lesion. After the procedure, the patient(55 years old female) became paraplegic with voiding and defecation difficulty which could be due to spinal cord infarction .by anterior spinal arteri. al occlusion caused by embolic material through the artery of Adamkiewicz from a posterior intercostal artery. She recovered completely after 20 days of treatment.
Arteries* ; Carcinoma, Hepatocellular ; Defecation ; Gelatin Sponge, Absorbable ; Hepatic Artery ; Infarction ; Liver ; Portal Vein ; Spinal Cord Injuries* ; Spinal Cord*

PURPOSE: To study the refractive outcome of cataract surgery employing partial coherence interferometry (PCI) and to compare this outcome with that of A-scan ultrasound in a prospective study of 96 eyes of 96 patients that underwent phacoemulsification with intraocular lens (IOL) implantation. METHODS: The SRK-T formula was employed, using PCI (IOL Master(R): the only commercially available model) and A-scan ultrasound data, to predict patients' implanted IOL power. Four to six weeks after cataract surgery, the refractive outcome was determined, and results from the two different biometry methods were compared. RESULTS: Ninety-six patients (mean age: 67.64, SD: 9.91) underwent phacoemulsification with IOL implantation. The optical axial length obtained using the IOL Master(R) was significantly longer (p<0.001, Student's t-test) than the axial length obtained via by A-scan ultrasound, 24.29 (SD 1.80) mm vs. 24.19 (1.75) mm. When using the IOL Master(R), the mean prediction error (PE; planned target of refraction - postoperative refraction) was 0.30 (0.60) D, and the mean absolute prediction error (APE) was 0.51 (0.44) D. When using A-scan ultrasound, the mean PE was 0.01 (0.64) D, and the mean APE was 0.47 (0.43) D. The difference in mean APE between the two biometry methods was not statistically significant (p=0.236, Wilcoxon signed rank test). Among the eyes with an axial length greater than 25 mm, as determined by A-scan ultrasound, the difference in the mean APE was not statistically significant (0.48 (0.87) vs. 0.58 (0.61), p=0.094). Likewise, among the eyes for which with axial length measured by A-scan ultrasound longer than IOL Master(R), the difference in the mean APE error was not statistically significant, (0.33 (0.30) vs. 0.46 (0.41), p=0.110). CONCLUSIONS: IOL power calculation using the PCI is as accurate as that using A-scan ultrasound for predicting the postoperative refractive state of patients who have undergone cataract surgery.
Biometry ; Cataract ; Hominidae ; Humans ; Interferometry ; Lenses, Intraocular ; Phacoemulsification ; Prospective Studies ; Ultrasonography*

The ileal atresia has been commonly accepted to be caused by a vascular accident during early intrauterine period. Thanks to the development of anesthesia, surgical techniques, postoperative supportive treatment and the hyperalimentation technique, the motality rate of this disease has been decreased. In spite of these developments, premature and very low birth weight babies should be given more attention because they still have a high motality rate. The authors report a successful treatment of ileal atresia in a 1,200g premature female infant. The proximal distended and hypertrophied intestine was resected. The distal small bowel was transected using a oblique line to create a fish-mouth. An end to end anastomosis was performed. We gave postoperative care, such as incubator care, fluid therapy, gastric decomprssion and total parenteral nutrition(TPN). TPN was initiated on postoperative one day. Calories were increased from 60cal/kg to 90cal/kg. TPN was stopped on the postoperative 24 day when the patient tolerated full strength milk feeding. Eighteen months postoperatively, she is healthy and her weight is 9.0kg(9 percentile).
Anesthesia ; Female ; Fluid Therapy ; Humans ; Incubators ; Infant ; Infant, Newborn* ; Infant, Very Low Birth Weight ; Intestines ; Milk ; Postoperative Care

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

In order to investigate the effect of head mounted display (HMD) on the human eye, the authors performed ophthalmologic examination on normal persons, dry eye patients and strabismus patients before and after use of Glastron, i-glasses, LGHMD(PT),LGHMD10 for two hours once or for five consecutive days, respectively. There were no substantial changes in visual acuity, color vision, intraocu-lar pressure, and tear secretion before and after use of HMD. There were transient changes in terms of corneal curvature, refraction, stereopsis, the angle of strabismus, and accommodation. There existed no difference according to the kinds of HMD. In conclusion, there was no permanent ocular changes with use of four kinds of HMD for two hours once or for five consecutive days.
Color Vision ; Depth Perception ; Head* ; Humans* ; Strabismus ; Tears ; Visual Acuity

The use of steroids has long been reported many side effects. Steroid-induced mental disorder is one of many complications associated with corticosteroid therapy. Steroid psychosis is not common disease but it may be more popular due to high steroid therapy such as pulse therapy or high dose steroid therapy. Euphoria, irritability, insomnia, and hallucination are predominating symptoms. The dosage, duration of the treatment may not be correlated with the time of the onset, duration, severity, or type of mental disturbances, but the risk of developing psychosis is increased to the high dose of steroid. It is usually reversible on dose reduction or discontinuation of the drug.
Euphoria ; Hallucinations ; Mental Disorders ; Psychotic Disorders* ; Sleep Initiation and Maintenance Disorders ; Steroids

No abstract available.
Diagnosis*