We report a case of Waardenburgs syndrome in 18-year-old male patient who has total deafness of the right ear, heterochromia irides, hypopigmented patches on the face and trunk, and disseminated lentigines on the entire body surface since birth. His 51-year-old mother also has total deafness, heterochromia irides, white foreloek, and disseminated lentigines on the entire body surface. The patient with this disorder may complain of pigmentary abnormalities, and we believe dermatologists should pay more attention to systemic signs of the congenital pigmentary disorders.
Adolescent ; Deafness ; Ear ; Humans ; Lentigo ; Male ; Middle Aged ; Mothers* ; Parturition ; Waardenburg Syndrome*

No abstract available.
Estrogens* ; Humans ; Receptors, Estrogen* ; Thyroid Diseases* ; Thyroid Gland*

No abstract available.
Microvascular Decompression Surgery*

The subarachnoidal or epidural opioid reveals excellent analgesic effect for postoperative pain and intractable cancer pain, but its side effects such as nausea, vomiting, voiding difficulty, pruritus and respiratory failure limit its use. There were many studies for decreasing frequency and severity of side effects and reinforcing the analgesic effect of opioid by administrating other drugs. Clonidine is one of such drugs which is able to be administered epidurally with opioids for that purpose. We studied the changes of cardiovascular response, analgesic and sedative effect according to the dose of epidural clonidine. The analgesic effect of epidural clonidine was investigated in 30 patients who underwent anal surgery with epidural anesthesia using 15 ml of 1.5~2 % lidocaine.The time of maximal intensity of pain after disapperance of injected lidocaine was checked. Thirty patients were divided into three groups randomly. In group 1 (n=10), the dose of epidural clonidine was 50 ug; Group 2 (n=10) was 150 ug; Group 3 (n=10) was 450 ug. Changes in the arterial pressure, pulse rate, sedation state and SpO2 were observed before and during 60 minutes after epidural clonidine administration. And the analgesic effect was assessed by measuring VAS pain score. Blood pressures and pulse rates decreased according to increase of dosage of clonidine. Group 1 showed the analgesic effect of 34%, group 2 showed 77% and group 3 showed 81% at 60 minutes after administration. Sedation effect was seen in group 2 and 3 but SpO2 was not decreased significantly. We thought that the respiratory depression of epidural clonidine was not so significant to limit the use for the postoperative pain control. We conclude that it is better to administer clonidine with opioids epidurally than clonidine slone to get better analgesic effect and less sedative effect, because the analgesic effect of epidural clonidine increases according to increase of dosage but the sedative effect increases also.
Analgesics, Opioid ; Anesthesia, Epidural ; Arterial Pressure ; Clonidine* ; Heart Rate ; Humans ; Hypnotics and Sedatives* ; Lidocaine ; Nausea ; Pain, Postoperative ; Pruritus ; Respiratory Insufficiency ; Vomiting

Mediastinal fibrosis is pathologically characterized by chronic inflammation and fibrosis of mediastinal soft tissue. Mediastinal fibrosis is local expression of a family of systemic fibrosing syndroms. This can result in compression of adjacent mediastinal structures. Idiopathic fibrosing syndromes include retroperitoneal fibrosis, sclerosing cholangitis of the orbit and fibrosis of the thyroid gland(Riedel's struma). The cause of these disorders is obscure, in some instance there is an underlying malignancy, infection, history of drug ingestion, or trauma with retoperitoneal bleeding. Treatment of mediastinal fibrosis depends on structures involved by the fibrotic process. The disease is self limited in most case or improved by steroids uses. We experienced a case of idopathic solerosing mediastinitis with orbital fibrous dysplasia of unknown cause, which was confirmed by open lung biopsy, so reported it with a review of literature.
Biopsy ; Cholangitis, Sclerosing ; Eating ; Fibrosis* ; Hemorrhage ; Humans ; Inflammation ; Lung ; Mediastinitis ; Orbit ; Retroperitoneal Fibrosis ; Steroids ; Thyroid Gland

PURPOSE: The aim of this study was to establish a measurement standard for the weight-bearing axial alignment of the lower extremities when performing a lower extremity reconstruction. MATERIALS AND METHODS: Among 274 patients (147 males and 127 females) in their 20s to 30s without any physical disabilities, and who had undergone weight-bearing full leg standing radiographs, 188 patients (100 males and 88 females) were examined this study. The data was analyzed further according to their age and gender using the radiographic value on the coronal and sagittal alignment of the lower extremities. RESULTS: The weight bearing ratio was 38.47+/-10.52% on the coronal plane and 36.11+/-7.88% on the sagittal plane. Both the mechanical axis and the the weight bearing axis were not identical due to their 0.68+/-0.89degrees difference on average. The most adjacent point of the femoral condyles to the tibial plateau on the sagittal plane was assigned to the center of the knee joint. The difference between the mechanical axis and weight bearing axis was 0.04degrees, which was not significant. CONCLUSION: There is a difference between the mechanical axis, which consists of both a line from the center of the femoral head to the center of knee joint and a line from the center of the knee joint to the center of ankle joint, and the weight bearing axis, which is linked directly from the center of the femoral head to the center of the ankle joint. Furthermore, the weight bearing axis passes through the anteromedial part in the knee joint. The center of the knee joint is defined as the most adjacent point of both femoral condyle to the tibial plateau on the sagittal plane.
Adult ; Ankle Joint ; Axis, Cervical Vertebra ; Head ; Humans ; Knee Joint ; Leg ; Lower Extremity ; Male ; Weight-Bearing

PURPOSES: The causes of altered mental status (AMS) are varied. Trauma accounts for 6.5%-36.3% of all AMS cases, and the mortality in these instances ranges from 20~80.5%. Multiple injury severity scoring systems exist to assist in the prognosing of trauma patients. We investigated the degree of correlation between injury severity scoring systems and prognoses of patients with AMS due to trauma. METHODS: We reviewed the medical records of 188 patients admitted to the emergency department of Konyang University Hospital who had trauma with AMS. We investigated the clinical characteristics of these patients and evaluated the usefulness of four injury severity scoring systems: revised trauma score (RTS), injury severity score (ISS), new injury severity score (NISS), and trauma and injury severity score (TRISS). RESULTS: The average scores of ISS and NISS were higher and RTS and TRISS were lower in the non-survivor group than in the survivor group (p<0.01). The area under the curve (AUC) for the receiver operating characteristic (ROC) curve was 0.725 for ISS and 0.733 for NISS, but these scoring systems had only a fair predictive ability. Hosmer-Lemeshow statistics showed no predictive ability differences for death between the four scoring systems. The predictive ability of all injury severity scoring systems to identify survivors was high (88.1~94.0%). In contrast, their predictive ability to identify non-survivors was low (29.6%~63.0%). CONCLUSIONS: In the use of RTS, ISS, NISS, and TRISS for evaluating trauma patients with AMS, none of the four systems exhibited any distinguishing predictive features. All failed to reliably predict non-survivors, but all showed good predictive value for identifying survivors.
Emergency Service, Hospital ; Humans ; Injury Severity Score ; Medical Records ; Mortality ; Multiple Trauma ; Prognosis ; ROC Curve ; Survivors

The retinal vein occlusion is one of the most common vision-threatening retinal diseases, and both the hemi-central and central retinal vein occlusion might induce severe visual loss. There have been many studies on the role of abnormally produced oxygen free radicals and excitatory amino acids in the mechanism of ischemic retinal injury. Many free radical scavengers, such as superoxide dismutase (SOD), catalase, and EGB 761 were reported to reduce the extent of ischemic retinal injury in animal experiments. Ascorbic acid is also a well-known free radical scavenger, but its in vivo therapeutic effects on retinal ischemia has not been studied, yet. We induced experimental retinal vein occlusion in 48 albino rat eyes; half of major veins in 24 eyes (group A) and total veins in 24 eyes (group B), with rose bengal dye injection followed by argon-green laser photocoagulation. The occlusion of veins was confirmed with fluorescein angiography. We injected ascorbic acid intraperitoneally just after venous occlusion in 12 eyes for each group. Rats were sacrificed after I week (group A) or 2 weeks (group B) following experiment and histological examination was done. In both groups, inner retina was less damaged than outer retina. The density of retinal ganglion cells was reduced to a less degree in eyes of rats treated with ascorbic acid in group A(P=0.0318). These data support the idea that the reactive free adicals are involved in the mechanism of ischemic retinal injury and thus the ascorbic acid may attenuate the ischemic retinal damage.
Animal Experimentation ; Animals ; Ascorbic Acid* ; Catalase ; Excitatory Amino Acids ; Fluorescein Angiography ; Free Radical Scavengers ; Free Radicals ; Ischemia ; Light Coagulation ; Models, Animal ; Oxygen ; Rats ; Retina ; Retinal Diseases ; Retinal Ganglion Cells ; Retinal Vein Occlusion* ; Retinal Vein* ; Retinaldehyde* ; Rose Bengal ; Superoxide Dismutase ; Veins

Posterior reversible encephalopathy syndrome (PRES) is characterized by headache, vomiting, confusion, and seizure. In addition, PRES is associated with reversible bilateral cortical and subcortical edema on occipital lobe or parieto-occipital lobe. Eclampsia is a rare condition to pregnant and puerperal women and one of common causes of the PRES. The clinical and radiologic manifestations can be resolved without irreversible complication by early diagnosis and appropriate treatment. The authors report a case of eclamptic encephalopathy associated with PRES, in which an 18-year-old woman had clinical manifestations of visual disturbance, headache, and tonic-clonic seizure at 34 hours after vaginal delivery. High signal intensities are seen in both parieto-occipital lobes and left basal ganglia on fluid attenuated inversion recovery (FLAIR) images and T2 weighted images performed at emergency room. But no significant signal change in both parieto-occipital lobes on diffusion weighted images (DWI). Because seizure was repeated, then anticonvulsant was administered at intensive care unit. On the second day, the clinical manifestations were resolved as blood pressure was normalized. The FLAIR imaging and DWI sequences can play an important role in the diagnosis of PRES.
Adolescent ; Basal Ganglia ; Blood Pressure ; Diagnosis ; Diffusion ; Early Diagnosis ; Eclampsia ; Edema ; Emergency Service, Hospital ; Female ; Headache ; Humans ; Intensive Care Units ; Occipital Lobe ; Posterior Leukoencephalopathy Syndrome* ; Pregnancy ; Seizures ; Vomiting

To evaluate the clinical significance of anomalous pancreaticobiliary ductal union(PBDU), we analysed 11 cases(0.19%) of anomalous PBDU among 5675 cases performed ERCP from Jan. 1973 to Aug. 1992. According to the classification of Kimura et al., 4 cases were type 1(P-C union) and 7 cases type 2(C-P union). The length of common channel ranged from 1.5 cm to 3.1 cm(mean 1.9 cm). The common associated disease were choledochal cyst(7 cases), carcinoma of the gallbladder(5 cases) and biliary stone(3 cases). Four cases of carcinoma of the gallbladder were type 1. The incidence of anomalous PBDU was significantly higher in cases with carcinoma of the gallbladder(5 cases among 49 cases) than in case without carcinoma of the gallbladder(6 cases among 5626 cases). And the incidence of gallbladder carcinoma(45%) among the 11 cases of anomalous PBDU was significantly higher than that(0.78%) among those who did not have anomalous PBDU. In conclusion, the common associated diseases with the anomalous PBDU were choledochal cyst, carcinoma of the gallbladder and stone, and the anomalous PBDU may be risk factor in the development of carcinoma of the gllbladder.
Cholangiopancreatography, Endoscopic Retrograde* ; Choledochal Cyst ; Classification ; Gallbladder ; Incidence ; Risk Factors