Waardenburg syndrome is a very rare auditory-pigment syndrome with autosomal dominant inheritance. In his first report in 1951, Waardenburg characterized this syndrome as dystopia canthorum, a high broad nasal bridge, synophrys[confluent eyebrows], heterochromia iridid, a white forelock or early graying, and sensory neural deafness. We observed a case of Type 2 Waardenburg syndrome with openangle glaucoma and, therefore report with literature review.
Deafness ; Glaucoma ; Glaucoma, Open-Angle* ; Waardenburg Syndrome* ; Wills

PURPOSE: It is reported that 65% of diabetic male patients over 60 of their age are impotent. However, there is no report concerning the effect of serum glucose control on erectile dysfunction in diabetic patient. This study was performed to evaluate the effects of serum glucose control on erectile dysfunction. MATERIALS AMD METHODS: The development of Diabetes mellitus was defined that the serum glucose level was above 200mg/dL in random test at 25 weeks. We designed the experimental specimen into 3 groups; group I: diabetic OLETF (Otsuka Long Evans Tokushima Fatty) rats without blood sugar control (n=10), group II: diabetic OLETF rats with blood sugar control with 4U insulin subcutaneous injection daily from 26 to 46 weeks (n=10), group III: diabetic OLETF rats with blood sugar control with 10U insulin subcutaneous injection daily from 26 to 46 weeks (n=10). We estimated the collagen content of penile tunica albuginea with the scanning electron microscopy and observed the endothelial layers of corpus cavernosum with the transmission electron microscopy in each group. RESULTS: The serum glucose level was 249 +/- 4.1mg/dL, 234 +/- 2.7mg/dL, 215 +/- 3.0mg/dL respectively in group I, II, III, and decreased in order of group II, III. The differences among groups were statistically significant (p<0.05). The thickness of collagen bundles of penile tunica albuginea was 0.98 +/- 0.1 micrometer, 0.94 +/- 0.3 micrometer, 0.92 +/- 0.1 micrometer respectively in group I, II, III, and decreased in order of group II, III. The differences among groups were statistically significant (p<0.05). The endothelial layers of corpus cavernosum were decreased in II, III. CONCLUSIONS: The serum glucose control in diabetic rats decreases the collagen con tents in penile tunica albuginea and the endothelial layers of corpus cavernosum.
Animals ; Blood Glucose* ; Collagen* ; Diabetes Mellitus ; Endothelium ; Erectile Dysfunction ; Humans ; Injections, Subcutaneous ; Insulin ; Male ; Microscopy, Electron, Scanning ; Microscopy, Electron, Transmission ; Rats* ; Rats, Inbred OLETF

Dissecting aortic aneurysm is an emergency condition which requires prompt diagnosis and management. From January 1992 to December 1996, 54 patients were admitted to our department. Patients were categorized according to the Stanford classification of aortic dissection and investigation of the clinical records utilized the retrospective method. Male constituted the majority, according for 38(70.4%) of the total number of patients, whereas females accounted for 16(29.6%). Mean age was 52.1+/-2.2 years old (range:25~82). Clinical findings included chest pain in 46 cases(85.2%), renal failure in 11(20.4%), aortic insufficiency in 10(18.5%) and stroke in 9(16.7%). Predisposing factors were hypertension in 48 cases(88.9%), Marfan's syndrome in 5(9.3%) and diabetes mellitus in 1(1.8%). 21 patients(type A:12, type B:9) underwent surgical treatment. There were three(2 in type A and 1 in type B) deaths in the surgical treatment group and nine(5 in type A and 4 in type B) deaths in the medical treatment group. These results reflect our current policy in the management of dissecting aortic aneurysm.
Aortic Aneurysm* ; Causality ; Chest Pain ; Classification ; Diabetes Mellitus ; Diagnosis ; Emergencies* ; Emergency Service, Hospital* ; Female ; Humans ; Hypertension ; Male ; Marfan Syndrome ; Renal Insufficiency ; Retrospective Studies ; Stroke

OBJECTIVES: Cognitive psychological models propose that auditory hallucinations arise from a problem with monitoring one's auditory verbal imagery. Most auditory hallucinations are derogatory in content and accompany negative emotions. If auditory verbal imagery plays an critical role in the pathogenesis of auditory hallucination, it must be influenced by negative emotions. This study was aimed at understanding the influence of negative emotions on the development of hallucinations by investigating the way by which negative emotions have influence on cortical activity induced by auditory verbal imagery. METHODS: For both normal subjects and patients with schizophrenia, quantitative electroencephalography(Q-EEG) was applied during the auditory verbal imagery tasks using a two word list. The one word list accompanied negative emotion and the other accompanied neutral emotions. The difference of EEG activity between two tasks was compared by paired t-test. WE also compare the difference of the influence of negative emotions between normal subjects and patients with schizophrenia. RESULTS: In normal subjects, amplitude of beta wave was increased in temporal area such as TCP1, and, the amplitude of theta frequency wave was decreased in right hemisphere such as FP2, F4, C4, CP2, P4, But, in the schizophrenia group, there were no significant differences. CONCLUSION: These results may suggest that auditory verbal imagery with negative emotion requires more activation in left temporal area, but, appropriate activation may not achieved in schizophenia patients. So, the possibility that the resultant disturbance of verbal self monitoring may be related to auditory hallucination is suggested in this study.
Electroencephalography ; Hallucinations ; Humans ; Models, Psychological ; Schizophrenia*

OBJECTIVE: Amniocentesis for the diagnosis of prenatal genetic abnormalities is now the standard care for women who are at special risk. because the application of population-based maternal serum screening to prenatal diagnosis is now widespread, we can estimate the trend change of amniocentesis indications. METHODS: Four hundred twenty eight women who were attending the antenaltal clinic of Hanyang University Hospital had a amniocentesis between January 1992 and June 1997. The result were analyzed in reference to indication of amniocentesis, gestational age, pregnancy outcome and karyotype. RESULT: The major indications were abnormal maternal serum marker(53.3%), advanced maternal age(23.8%), previous fetal chromosomal anomaly(7.9%) and the most common age distribution at amniocentesis was 25-29 years(37.9%). The pregnancy outcome was full-term delivery(84.2%), preterm delivery(13.7%), spontaneous abortion(1.4%) and termination of pregnancy(0.7%). Among the 33 cases(7.7%) of abnormal karyotype, structural aberration was 21 cases(4.9%) and numerical aberration was 12 cases(2.8%). Among the numerical aberration, six cases of trisomy 21, five cases of Klinefelter syndrome, and one case of Turner syndrome were found. Among the structural aberration, insertion was most common(nine cases), and seven cases of inversion, four cases of translocation and one case of deletion were found. CONCLUSION: This is a report of genetic amniocentesis, with analysis of the indication, gestational age, karyotype results and complication.
Abnormal Karyotype ; Age Distribution ; Amniocentesis* ; Diagnosis ; Down Syndrome ; Female ; Gestational Age ; Humans ; Karyotype ; Klinefelter Syndrome ; Mass Screening ; Pregnancy ; Pregnancy Outcome ; Prenatal Diagnosis* ; Turner Syndrome

OBJECTIVE: Amniocentesis for the diagnosis of prenatal genetic abnormalities is now the standard care for women who are at special risk. because the application of population-based maternal serum screening to prenatal diagnosis is now widespread, we can estimate the trend change of amniocentesis indications. METHODS: Four hundred twenty eight women who were attending the antenaltal clinic of Hanyang University Hospital had a amniocentesis between January 1992 and June 1997. The result were analyzed in reference to indication of amniocentesis, gestational age, pregnancy outcome and karyotype. RESULT: The major indications were abnormal maternal serum marker(53.3%), advanced maternal age(23.8%), previous fetal chromosomal anomaly(7.9%) and the most common age distribution at amniocentesis was 25-29 years(37.9%). The pregnancy outcome was full-term delivery(84.2%), preterm delivery(13.7%), spontaneous abortion(1.4%) and termination of pregnancy(0.7%). Among the 33 cases(7.7%) of abnormal karyotype, structural aberration was 21 cases(4.9%) and numerical aberration was 12 cases(2.8%). Among the numerical aberration, six cases of trisomy 21, five cases of Klinefelter syndrome, and one case of Turner syndrome were found. Among the structural aberration, insertion was most common(nine cases), and seven cases of inversion, four cases of translocation and one case of deletion were found. CONCLUSION: This is a report of genetic amniocentesis, with analysis of the indication, gestational age, karyotype results and complication.
Abnormal Karyotype ; Age Distribution ; Amniocentesis* ; Diagnosis ; Down Syndrome ; Female ; Gestational Age ; Humans ; Karyotype ; Klinefelter Syndrome ; Mass Screening ; Pregnancy ; Pregnancy Outcome ; Prenatal Diagnosis* ; Turner Syndrome

OBJECTIVE AND METHODS: To determine correlations among ambulatory renin activity, ambulatory blood pressure and microalbumin excretion rate, 66 Korean essential hypertensives were studied after 4 week wash-out period. The ambulatory blood pressure was monitored every 30 minutes and mean BP were calculated automatically. Urinary microalbumin excretion rate(UAER) and ambulatory plasma renin activity(aPRA) collected at mid-day were measured by radioimmunoassays. Subjectives were divided into 2 groups by aPRA value(2ng/ml/hr). RESULT: 14 cases were high renin group and 52 cases low renin group. The mean BP were 148.83/94.69mmHg in low renin group, and 146.57/98.07mmHg in high-renin group without difference. UAER were not different also between both groups. 23.07%(4/14) of non-dippers were included in high renin group and 25.58%(12/52) in low renin group without statistical difference. The aPRA was significantly related to UAER and systolic and diastolic mean blood pressure. Also UAER was related significantly to day mean blood pressures. CONCLUSION: Thus aPRA is thought to be a meaningful indicator to predict hypertensive renal target organ damage as well as blood pressure measured with 24-hr ABPM.
Blood Pressure* ; Hypertension* ; Plasma* ; Radioimmunoassay ; Renin*

Using as assay for jejunal crypt stem cell survival, dose-response curves for the reproductive capacity of crypt stem cells mouse jejunum exposed to multifractionated gamma-ray irradiation(single, 2, 3, 4, 5, 6, 7, 8, 10,12, and 16 fractions) were analyzed and single-dose survival curve of these cells was constructed. The following conclusion were drawn: 1) Survival curves for higher numbers of dose fractions were displaced to higher dose, and characterized by increasingly shallower slopes. 2) The single-dose survival curve had broad shoulder, Dq=460 cGy, remaining near-exponential over initial dose range 0 to 300 cGy, with initial slope 1Do=474 cGy 3) At fractionated dose in the range of 180 to 450 cGy, the average recovered dose per fraction interval was approximately 50% of the dose per fraction. 4) The value of a/b ratio by using of linear regression analysis for the reciprocal dose plots was 8.3Gy which lied in the range of 6-14Gy for early-reacting tissues. 5) The linear-quadratic model for dose-response formula offers valid approximations for all doses to be used in radiotherapy, only two parameters to be determined, and considerable convenience in practical applications.
Animals ; Jejunum ; Linear Models ; Mice* ; Radiotherapy ; Shoulder ; Stem Cells

BACKGROUND: Evening primrose oil(EPO) is a rich source of cis-linoleic acid and gammalinolenic acid(GLA) and has been used as a therapeutic agent in various skin diseases such as atopic dermatitis. OBJECTIVE: The purpose of this study was to evaluate the suppressive effect of EPO on murine contact sensitivity. METHODS: BALB/c mice were divided into 3 groups, positive control, experimental and negative control groups: the positive control group represents a group of mice which were sensitized and challenged with DNFB, the experimental group represents EPO-pretreated positive control group and the negative control group represents a group of mice which were challenged only. The changes of ear thickness were measured, and H & E staining and immunohistochemical staining for ICAM-1 expression of ear skin were performed to evaluate the histological changes of each group. RESULTS: The Pretreatment of mice with EPO resulted in suppression of contact sensitivity by more than 82%. On H & E staining, only a mild inflammatory reaction was observed in the dermis. Also ICAM-1 expression of keratinocytes, the intensity of the staining was significantly decreased in the experimental group compared with positive group. CONCLUSIONS: Our study indicates that EPO was able to suppress the induction of contact sensitivity.
Animals ; Dermatitis, Atopic ; Dermatitis, Contact* ; Dermis ; Dinitrofluorobenzene ; Ear ; Intercellular Adhesion Molecule-1 ; Keratinocytes ; Mice ; Oenothera biennis* ; Skin ; Skin Diseases

Patau syndrome, or Trisomy 13 is one of the most common autosomal aberration associtated with multiple congenital abnormalities. We report a case with trisomy 13 mosacism which was found during an amniocentesis performed due to the age of the mother and abnormal nuchal translucency. The clinical features of fetus included cleft lip and palate, low set ears, polydactily, small ""micro"" penis, and Rocker-bottom feet. After termination of the pregnancy, the fetus was sent for an autopsy. The autopsy report was compatible with the gross findings and pulmonary hypoplasia, microophthalmia, hypoplasia of left ventricle of heart were found.
Amniocentesis ; Autopsy ; Cleft Lip ; Congenital Abnormalities ; Ear ; Fetus ; Foot ; Heart ; Heart Ventricles ; Humans ; Male ; Mothers ; Nuchal Translucency Measurement ; Palate ; Penis ; Pregnancy* ; Trisomy