Fibrous dysplasia in the orbit and cranial base may involve the optic canal. Although fibrous dysplasia is benign, it may produce a mass effect along the course of the optic nerve which can then induce visual disturbance as well as contour deformities of the skull and facial bone. The treatment of fibrous dysplasia in the orbit and cranial base is to resect the lesion as much as possible and then reconstruct immediately. As well, if there is any evidence of optic canal involvement and disease progression, the treatment of fibrous dysplasia may include optic nerve decompression. It is generally understood that some patients experience improvement of visual function after optic nerve decompression. We performed radical excision and reconstruction by means of autogenous calvarial bone graft and methylmethacrylate in 7 cases. The autogenous calvarial bone was used to reconstruct the orbit. The methylmethacrylate was used to reconstruct bony defect in the temporal area. The orbit was reconstruced into one block which was made of autogenous calvarial bone with a microplate and screw. This method is superior compared to the previous multifragment wiring method with regard to stability, operation time, and appearance. The patients in our series showed satisfactory appearance. In 6 cases, we performed optic nerve decompression. Therapeutic optic nerve decompression was done in 3 cases and prophylatic optic nerve decompression was done in the others. Following therapeutic optic nerve decompression, visual acuity was improved in 2 cases while the others showed a decrease in visual acuity. There was no change of visual acuity and visual field in 1 case after prophylactic optic nerve decompression. However, the others showed decrements in visual acuity or visual field. Therefore, we believe that more attention should be paid during optic nerve decompression procedure and strict indications to that procedure should be applied.
Congenital Abnormalities ; Decompression* ; Disease Progression ; Facial Bones ; Humans ; Methylmethacrylate ; Optic Nerve ; Orbit* ; Skull ; Skull Base* ; Transplants ; Visual Acuity ; Visual Fields

Wilson's disease is an autosomal recessive disorder marked by disruptions in copper metabolism which leads to accumulation of copper in the liver, brain, cornea, and other tissues. Manifestations of this disease are more likely to be hepatic during early childhood and neurologic in adolescent. In addition, abnormalities that develop during disease progression may result in other manifestations such as hematologic, endocrine, or renal findings. Here we report a 13-year-old girl who presented with hypocalcemic tetany shortly after being diagnosed with Wilson's disease. Despite aggressive calcium, magnesium, and vitamin D replacement therapy, the hypocalcemia and hypomagnesemia did not promptly respond. Mineral levels in the blood took longer than 3 weeks to normalize. We speculate that a parathyroid insufficiency and disrupted vitamin D metabolism caused by copper accumulation and hepatic dysfunction resulted in hypocalcemic tetany.
Adolescent ; Brain ; Calcium ; Copper ; Cornea ; Disease Progression ; Hepatolenticular Degeneration ; Humans ; Hypocalcemia ; Hypoparathyroidism ; Liver ; Magnesium ; Tetany ; Vitamin D ; Vitamin D Deficiency

The purpose of this study was comparing the serial change of serum creatinine and Resistive Index (RI) after renal transplantation. From Jul. 1995 to Feb. 1997, we examined Doppler sonography for 32 out of 99 patients, who were undertaken renal transplantation, whose resistive index were taken at POD #1 and POD #5 and named initial resistive index. The late resistive index were examined when they discharged or when the acute rejection was suspicious. The RI rejection group, which was consist of 22 out of 32 patients, were selected whose late resistive index were increased more 0.05 than inital resistive index. The RI control group, which was consist of 10 out of 32 patients, was selected whose late resistive index were not more 0.05 than initial resistive index. The group which was consist of 21 patient, had over 1.8 mg/dL of serum creatinine. The rest, 11 patients, had not more than 1.8 mg/dL of serum creatinine. The Student's T-test was used for the statistical analysis. In the RI rejection group, late RI value (0.77+/-0.04) was significantly higher than initial RI (p<0.05) and the increase of RI in the acute rejection group were significantly greater than that in the control group (p<0.05). With the serial changes of late RI more 0.05 than initial RI, we obtained 85.0% of sensitivity and 58.3% of specificity for the prediction of the acute rejection. In the patients with the serum creatinine over 1.8 mg/dL, the sensitivity was 85.0% and the specificity was 66.7%. In the patients with the serum creatinine over 1.8 mg/dL and increased RI over 0.05, the sensitivity was 94.1% and specificity was 66.7%. The result shows that serial changes of duplex Doppler scan and the level of serum creatinine after renal transplantation were not enough to predict acute rejection. But the correlation of the result show more higher sensitivity. We suggest to be able to use the Doppler sonography and serial changes of serum creatinine level with the other clinical findings to predict the acute rejection after renal transplantation.
Creatinine* ; Humans ; Kidney Transplantation* ; Sensitivity and Specificity

PURPOSE: The aim of the study was to determine steroid sulfatase (STS) expression in endometrial cancer patients and its correlation with disease prognosis. MATERIALS AND METHODS: We conducted a retrospective study in 59 patients who underwent surgery with histologically confirmed endometrial cancer from January 2000 to December 2011 at Hanyang University Hospital. Immuno-histochemical staining of STS was performed using rabbit polyclonal anti-STS antibody. RESULTS: Sixteen of the 59 patients (27.1%) were positive for STS expression. Disease free survival (DFS) was 129.83±8.67 [95% confidence interval (CI): 112.84-146.82] months in the STS positive group (group A) and 111.06±7.17 (95% CI: 97.01-125.10) months in the STS negative group (group B) (p=0.92). Overall survival (OS) was 129.01±9.38 (95% CI: 110.63-147.38) months and 111.16±7.10 (95% CI: 97.24-125.07) months for the groups A and B, respectively (p=0.45). Univariate analysis revealed that FIGO stage and adjuvant therapy are significantly associated with DFS and OS. However, in multivariate analysis, FIGO stage and adjuvant therapy did not show any statistical significance with DFS and OS. STS was also not significantly associated with DFS and OS in univariate and multivariate analysis. CONCLUSION: STS expression was not significantly associated with DFS and OS, despite positive STS expression in 27% of endometrial cancer patients. Therefore, the role of STS as a prognostic factor in patients with endometrial cancer remains unclear and requires further research.
Adult ; Aged ; Biomarkers, Tumor ; Combined Modality Therapy ; Disease-Free Survival ; Endometrial Neoplasms/mortality/*surgery ; Female ; Gene Expression Regulation, Neoplastic ; Humans ; Middle Aged ; Neoplasm Staging ; Prognosis ; Retrospective Studies ; Steryl-Sulfatase/*metabolism ; Uterine Neoplasms/mortality/pathology/*surgery

PURPOSE: The tumor of lacrimal sac is very rare. We report an experience of a 63-year-old female who had a squamous cell carcinoma of the lacrimal sac and discuss its diagnosis and overall management. METHODS: A 63-year-old woman came to our clinic with a 2-month history of a growing mass in the region of the left medial canthus, left eye tearing. Examination revealed left epiphora and swelling on the left medial canthus and the left lacrimal sac, which was firm. Lacrimal irrigation indicated an obstruction. A computed tomographic scan demonstrated a well enhancing soft tissue mass in medial aspect of left orbit and no evidence of invasion of rectus muscles and adjacent bony destruction. The mass was excised by dacryocystectomy and subsequently, required 200 cGy a day, 5 times a week, 6000 cGy of radiotherapy for 6 weeks. RESULTS: The patient was finally diagnosed with keratinizing, squamous cell carcinoma that was proven by histopathologic finding. There was no evidence of recurrence of the tumor in lacrimal sac throughout 24 months of follow-up period.
Carcinoma, Squamous Cell* ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Lacrimal Apparatus Diseases ; Middle Aged ; Muscles ; Orbit ; Radiotherapy ; Recurrence ; Tears

Curcumin exerts a protective effect in cerebral ischemia through its anti-oxidant and anti-inflammatory activities. gamma-enolase is a glycolytic enzyme expressed in neurons that is known to exerts a neuroprotective effect. We investigated whether curcumin regulates gamma-enolase expression in focal cerebral ischemic injury in rats. Middle cerebral artery occlusion (MCAO) was performed to induce focal cerebral ischemia. Adult male rats were injected intraperitoneally with either vehicle or curcumin (50 mg/kg) 1 h after MCAO and cerebral cortex tissues were isolated 24 h after MCAO. We found that MCAO-induced injury resulted in a reduction in gamma-enolase expression in vehicle-treated animals using a proteomics approach. However, this reduction was attenuated in animals with MCAO treated with curcumin. Reverse-transcription PCR and Western blot analyses also showed that curcumin treatment prevented the MCAO injury-induced reduction in gamma-enolase expression. The results of this study suggest that curcumin exerts its neuroprotective function in focal cerebral ischemia by regulating the expression of gamma-enolase.
Adult ; Animals* ; Blotting, Western ; Brain Ischemia ; Cerebral Cortex ; Curcumin* ; Humans ; Infarction, Middle Cerebral Artery ; Male ; Middle Cerebral Artery* ; Models, Animal* ; Neurons ; Neuroprotective Agents ; Phosphopyruvate Hydratase* ; Polymerase Chain Reaction ; Proteomics ; Rats

Although venous thrombosis is one of the most frequent complications of malignant disease including multiple myeloma, massive pulmonary embolism is an uncommon complication. Free-floating intracardiac thrombus has been rarely reported as a cause of acute pulmonary embolism in patients with multiple myeloma. We report a case presenting acute pulmonary embolism due to free-floating right atrial thrombus in a patient with multiple myeloma.
Humans ; Multiple Myeloma ; Pulmonary Embolism ; Thrombosis ; Venous Thrombosis

Objective: We investigated the effects of fusidic acid (FA) on human cervical, thyroid, and breast carcinoma cell lines to determine the potential usefulness of FA in cancer treatment. Methods: Six cancer cell lines (cervical cancer: Caski, HeLa; thyroid cancer: 8505C, TPC1; and breast cancer: MCF-7, MDA-MB-231) were treated with FA. Furthermore the changes in cell growth, cell cycle duration, and extent of apoptosis were analyzed. Results: After FA treatment, the cancer cells showed a decrease in growth rate. In the cell death assay, the cell populations were similar in each cell type after treatment with FA, indicating that growth inhibition by FA was not related to the induction of apoptosis. FA induced cell cycle arrest at a dose that inhibited growth rate, which varied in different cell types. G0/G1 phase arrest occurs in breast cancer, S phase arrest in 8505C thyroid cancer, and G2/M phase arrest in cervical cancer. These results indicate that FA reduces growth rates by inducing cell cycle arrest. Conclusion FA treatment can interfere with cell proliferation by inducing cell cycle arrest in human cervical, thyroid, and breast carcinoma cell lines. Thus, FA can be useful in treating human cervical, thyroid, and breast carcinomas.

BACKGROUND/AIMS: Endoscopic retrograde cholangiopancreatography (ERCP) is considered a high-risk procedure in patients with previous acute coronary syndrome (ACS); however, clinical studies are rare in the literature. The aim of this study was to investigate the safety and efficacy of ERCP in patients with previous ACS. METHODS: We retrospectively reviewed the medical records of patients with previous ACS who underwent ERCP between January 2007 and August 2012. The clinical characteristics, ERCP diagnoses, treatment results, and complications were analyzed. RESULTS: Fifty patients underwent ERCP an average of 41.6 months after ACS. The most common indication for ERCP was calculous cholangitis. After deep biliary cannulation, endoscopic sphincterotomy, biliary stone removal and endoscopic biliary drainage were successfully performed. Immediate postsphincterotomy bleeding occurred in seven patients, which was successfully controlled using endoscopic therapy. Elevation of cardiac troponin I levels were observed in three patients (6%) before ERCP, and all of these patients were diagnosed with pancreatobiliary disease combined with recurrent ACS, which was treated with coronary artery stent insertion (n=2) and balloon angioplasty (n=1). CONCLUSIONS: Therapeutic ERCP is effective and safe in patients with previous ACS. Cardiac troponin I elevation should be considered a warning sign for recurrent ACS in patients who undergo ERCP.
Acute Coronary Syndrome/*blood/complications/therapy ; Aged ; Aged, 80 and over ; Ampulla of Vater/*surgery ; Angioplasty, Balloon, Coronary ; Carcinoma/*surgery ; Cholangiopancreatography, Endoscopic Retrograde/*methods ; Cholangitis/etiology/*surgery ; Cholelithiasis/complications/*surgery ; Common Bile Duct Neoplasms/*surgery ; Drainage ; Female ; Humans ; Male ; Middle Aged ; Recurrence ; Retrospective Studies ; Risk Assessment ; Sphincterotomy, Endoscopic ; Stents ; Troponin I/*blood

OBJECTIVE: Our purpose was to establish a birth defects monitoring system in Korea by multi-center study. This novel study was initiated in 1999, and extended to detect the frequencies and trends of birth defects in Korea. METHODS: Six centers participated in this study. The actively ascertained surveillance data was collected from May 1999 to November 2001. RESULTS: Of the 65,653 births included in this study, 1,143 (1.7%) had birth defects. About one third of them were terminated. While disease of the genitourinary tract was more frequent in isolated defects, cardiovascular disease was more frequent in combined defects (19.7% and 21.7%, respectively). Chromosomal anomalies were detected 23.5 per 10,000 births. And it showed increasing tendency for 3 years. CONCLUSION: We could establish multi-center monitoring system for birth defects successfully. But, many of the problems arising in the collection of accurate, valid, and comparable epidemiological data about birth defects have not yet been overcome. It appears that the development of joint projects at national level is essential for upgrading the quality and usefulness of this study.
Cardiovascular Diseases ; Congenital Abnormalities* ; Joints ; Korea* ; Parturition*