PURPOSE: To describe the abnormal signal intensity seen on MRI of the brain in Wilson disease. MATERIALS AND METHODS: Eight patients (7 male and 1 female, 10 to 33 years of age ) with Wilson disease were studied with a 0.5TMRI system. Patients were divided into symptomatic and asymptomatic groups, and MR imaging was compared withclinical data. RESULTS: In 93 lesions, signal intensity was abnormal ; there was involvement of the pallidus(24lesions, 26%), the midbrain (20, 22%), the pons(14, 15%), the putamen (13, 14%), the thalamus(6, 7%), thepituitary gland (4, 4%), the caudate nuclei (4, 4%), the internal capsule (4, 4%), and the dentate nucleus (4,4%). In the putamen, all lesions but one were bilateral, and there was symmetric distribution. The four patientswith neurologic symptoms had 69 lesions and the remaining four without such symptoms had 24 lesions. OnT2-weighted images, high signal intensity was seen in all lesions but two, and on T1-weighted images, this wasseen in 24 lesions. All lesions of the pituitary gland showed high signal intensity on T1-weighted images.CONCLUSION: Lesions were frequently seen in the globus pallidus, midbrain, pons and putamen, and were more commonin patients with neurologic symptoms.
Brain* ; Cerebellar Nuclei ; Female ; Globus Pallidus ; Hepatolenticular Degeneration* ; Humans ; Internal Capsule ; Magnetic Resonance Imaging* ; Male ; Mesencephalon ; Neurologic Manifestations ; Pituitary Gland ; Pons ; Putamen

BACKGROUND: In the majority of cases, sternal instability and wound infection concomitantly present after a cardiac operation following conventional median sternotomy, and these complications have a major influence on the postoperative course. The aim of this study is to compare the results of the different sternal wiring techniques on sternal infection. MATERIAL AND METHOD: Between April 2004 and December 2008, 157 adult patients underwent cardiac operation through a median sternotomy. 86 patients who had undergone standard peristernal wiring were included in group A, whereas 71 patients who had undergone modified Robicsek sternal wiring were included in group B. The incidences of sternal wound complications in the two groups were assessed. RESULT: The mean age of the group B patients was older than that of the group A patients (61+/-10 years vs 57+/-13 years). The incidence of preoperative left ventricular dysfunction (ejection fraction <30%), chronic obstructive pulmonary disease, renal failure requiring dialysis and diabetes mellitus were significantly higher in Group B, whereas the other perioperative risk factors for infection were not significantly different between the two groups. Two patients in group A experienced superficial wound infection, whereas 4 patients in group B displayed superficial wound infection, but the difference was not statistically significant (p=0.255). Yet poststernotomy deep sternal wound infection appeared in 6 patients of group A, whereas none of the patients in group B displayed this malady. CONCLUSION: The modified Robicsek sternal wiring technique showed greater sternal stability even for the patient with a high risk for infection, and the technique caused a lower incidence of deep sternal wound infection.
Adult ; Diabetes Mellitus ; Dialysis ; Humans ; Incidence ; Pulmonary Disease, Chronic Obstructive ; Renal Insufficiency ; Risk Factors ; Sternotomy ; Sternum ; Ventricular Dysfunction, Left ; Wound Infection

An 81-year-old woman was admitted to our hospital with bilateral chest wall mass in the infrascapular region. Considering the possible diagnosis of a malignant chest wall tumor at such location, we performed excision and biopsy. Both masses were histologically diagnosed as elastofibroma. We experienced this rare disease, bilateral elastofibroma, so we report this case with a bibliography.
Aged, 80 and over ; Biopsy ; Diagnosis ; Female ; Humans ; Rare Diseases ; Thoracic Wall

OBJECTIVE: To investigate whether GnRH-agonist (GnRH-Ag) using in IVF-ET affects apoptosis of human granulosa-luteal cells and expression of peripheral benzodiazepine receptor (PBR) protein involved in the apoptosis of the cells. METHODS: Granulosa-luteal cells obtained during oocyte retrieval were cultured and treated with 10(-5) M GnRH-Ag. Apoptosis of the cells by the treatment was confirmed using DNA fragmentation analysis 24 h after culture. The presence of PBR protein within the cells was examined by immunofluorescence staining and the expression of the protein was analyzed by Western blotting. In addition, it was measured for progesterone and nitric oxide (NO) produced by granulosa-luteal cells after GnRH-Ag treatment. To evaluate the relationship between NO production and PBR expression, sodium nitroprusside (SNP) as a NO donor was added in media and investigated the expression of PBR protein by Western blotting. RESULTS: Apoptosis increased in the granulosa-luteal cells 24 h after GnRH-Ag treatment, whereas the expression of PBR protein significantly decreased. Furthermore, the production of progesterone and nitric oxide (NO) by the cells significantly fell from 12 h after the treatment. In the results of Western blotting after SNP treatment, the expression of PBR protein increased in the treatment with SNP alone to the granulosa-luteal cells, but was suppressed in the treatment with GnRH-Ag and SNP. Additionally, the staining result of PBR protein in the cells showed the even distribution of it through the cell. CONCLUSION: These results demonstrate that GnRH-Ag treatment induces apoptosis, decreasing expression of PBR protein and NO production in human granulosa-luteal cells. The present study suggests that one of the apoptosis mechanism of human granulosa-luteal cells by GnRH-Ag might be a signal transduction pathway via NO and PBR.
Apoptosis* ; Blotting, Western ; DNA Fragmentation ; Female ; Fluorescent Antibody Technique ; Humans* ; Luteal Cells* ; Nitric Oxide ; Nitroprusside ; Oocyte Retrieval ; Progesterone ; Receptors, GABA-A ; Signal Transduction ; Tissue Donors

PURPOSE: To evaluate the effects of various factors on the variability of retinal nerve fiber layer (RNFL) thickness measurements using the Stratus optical coherence tomography (OCT) in normal and glaucomatous eyes. METHODS: Four hundred seventy-four subjects (103 normal eyes and 371 glaucomatous eyes) were scanned to determine the RNFL thickness measurements using the Stratus OCT. Measurements were obtained twice during the same day. The standard deviation (SD) was used to compare the variability in RNFL thickness measurements of the normal subjects to that of the glaucomatous patients. Multivariate regression analysis was used to evaluate which covariates were independent predictors of SD in overall mean RNFL thickness. RESULTS: The mean SD of all RNFL thickness measurements was larger in the glaucoma group except in one sector. In the multivariate regression analysis, the average signal strength (SS) and the relative SS change (difference in SS between initial and repeat scans, divided by initial SS) were independent predictors of the SD in the RNFL thickness measurements (partial R2 = 0.018, 0.013; p = 0.016, 0.040, respectively). CONCLUSIONS: Glaucomatous eyes tend to be more variable than normal eyes in RNFL thickness measurement using the Straus OCT. The average SS and the relative SS changes appear to correlate with the variability in RNFL thickness measurement. Therefore, the results of the RNFL analysis should not be interpreted independently of these factors.
Aged ; Female ; Glaucoma/*pathology ; Humans ; Male ; Middle Aged ; Multivariate Analysis ; Nerve Fibers/*pathology ; Predictive Value of Tests ; Reference Values ; Regression Analysis ; Reproducibility of Results ; Retina/*cytology/*pathology ; Tomography, Optical Coherence/*methods/*standards

PURPOSE: To assess the characteristic CT findings of the angiomatous polyp. MATERIALS AND METHODS: Fivecases of pathologically-proven angiomatous polyp were retrospectively evaluated. All underwent CT scanning, butin only four cases were postcontrast CT scans obtained. In analysing CT findings we focused on adjacent bonychange, and the extent and enhancement pattern of the mass. RESULTS: All but one case involved the maxillarysinus, showing thickening of the posterolateral wall and erosion or destruction of the medial wall. As forinvolvement of the anterior wall of this sinus, bony destruction was seen in one case, and thickening in three. Infour cases the tumor involved the maxillary sinus and nasal cavity, and two cases showed nasopharyngeal extension.No case involved the pterygopalatine fossa, however. On contrast enhanced CT scans(n=4), all cases showedenhancement as strong as blood vessels, and a multiple focal punctate or tubular pattern. CONCLUSION: Angiomatouspolyp tends to show bone thickening rather than bone destruction, not to involve the pterygopalatine fossa, and toreveal a strong punctate or tubular enhancement pattern. These findings may be helpful in the differentialdiagnosis of angiomatous polyp from other tumors such as maxillary cancer, angiofibroma and nasal polyp
Angiofibroma ; Blood Vessels ; Maxillary Sinus ; Nasal Cavity* ; Nasal Polyps ; Polyps* ; Pterygopalatine Fossa ; Retrospective Studies ; Tomography, X-Ray Computed

BACKGROUND: Malignant pleural effusion is a common condition in neoplastic patients and palliative therapy is the usual treatment. Talc has been generally accepted to be the most effective sclerosant for chemical pleurodesis, but the optimal route of administration remains controversy. We compared the results of video-assisted thoracoscopic talc poudrage (VTP) with administering a bedside talc slurry through a chest tube (BTS) for the treatment of malignant pleural effusion. MATERIAL AND METHOD: From December 2004 to May 2006, 20 patients with malignant pleural effusion underwent chemical pleurodesis via VTP (group A, n=10), and BTS (group B, n=10). RESULT:The durations of chest tube placement after the procedure were 7.0+/-4.0 days (group A) and 6.7+/-3.6 days (group B). The hospital stays were 24.3+/-9.4 days (group A) and 30.7+/-21.5 days (group B), respectively. The symptoms of dyspnea were much more improved in group A (p-value=0.014) after discharge (mean f/u group A=8.5+/-2.2 months, group B 8.0+/-7.4 months). The collapsed portions of lung were better expanded in group A than in group B (p-value= 0.011). CONCLUSION: We recommend VTP for the selected patients with malignant pleural effusion because of the advantages of dissecting the fibrous peel to relieve the atelectasis and dyspnea, and excising the pleura for diagnosis with direct viewing of the lesion.
Chest Tubes ; Diagnosis ; Dyspnea ; Humans ; Length of Stay ; Lung ; Palliative Care ; Pleura ; Pleural Effusion ; Pleural Effusion, Malignant* ; Pleurodesis ; Pulmonary Atelectasis ; Talc* ; Thoracoscopy

PURPOSE: This study aimed to examine the following questions: to what extent do patients and caregivers perceive their family members to be avoidant of communication regarding patient's cancer, and to what extent do these perceptions interrelate; and how do such perceptions influence their own and each other's communication behaviors, communication outcome, mental health, and quality of life. MATERIALS AND METHODS: A national survey was performed with 990 patient-caregiver dyads (participation rate, 76.2%). To examine the dyadic interaction, we developed linked patient and family member questionnaires, including the Family Avoidance of Communication about Cancer (FACC) scale. RESULTS: The mean scores (standard deviations) of patient- and caregiver-perceived FACC were low at 10.9 (15.5) and 15.5 (17.5), respectively (p < 0.001), and concordance was low, a well (Spearman's rho, 0.23). Patient-perceived FACC was associated with lower levels of disclosure and behaviors of holding back communication, as well as lower levels of mental health outcome and quality of life. The same was true for caregivers (all p < 0.05). Patient-perceived FACC was associated with caregiver holding back, caregiver's depression level, and caregiver quality of life (all p < 0.05). Both patient- and caregiver-perceived FACC were independently associated with communication difficulty within the family. CONCLUSION: Future research would benefit from the measurement of FACC from both patients and caregivers, and promote family intervention to enhance openness to communication, which would be helpful for improving mental health and quality of life for both patients and caregivers.
Caregivers ; Depression ; Disclosure ; Humans ; Mental Health ; Quality of Life

PURPOSE: To determine the therapeutic effect of transcatheter embolization in the treatment of splanchnicpseudoaneurysm. MATERIALS AND METHODS: This study involved eleven patients who underwent embolization for thetreatment of splanchnic pseudoaneurysm. Nine were men and two were women ; their ages ranged from 8 to 74 (mean,51) years. The etiology of these cases included postoperative pseudoaneurysm(n=4), pancreatitis(n=3), stabinjury(n=1), and suspected infection(n=1), while two cases were uncertain. The locations of the pseudoaneurysmwere the splenic artery(n=4), the gastroduodenal artery(n=3), the hepatic artery(n=2), the celiac artery(n=1), andboth the right renal and lumbar artery(n=1). All patients underwent angiography prior to embolization. Thematerials used during embolization were a microcoil, a 5-cm metallic guide wire, and a detachable balloon. RESULTS: Embolization was successful in all eleven cases. Among nine cases in which follow-up was possible, angiographywas performed in four, and five cases of thrombus were confirmed by abdominal CT. Three of these nine patientsunderwent re-embolization. One patient underwent elective surgery for a pseudocyst due to pancreatitis. CONCLUSION: Transcatheter embolization is a safe and convenient modality for the treatment of splanchnic pseudoaneurysm.
Aneurysm ; Aneurysm, False* ; Angiography ; Female ; Follow-Up Studies ; Humans ; Male ; Pancreatitis ; Thrombosis ; Tomography, X-Ray Computed

Infected aneurysms are uncommon, frequently fatal lesions. "True" fungus-infected aneurysms are even rarer. Fungal infections have high morbidity and mortality. However, diagnosis is frequently difficult, since the symptoms are non-specific and standard diagnostic procedures are often insensitive. We experienced a patient with persistent fever and negative blood cultures. The patient was immunocompetent and had no risk factors, and was diagnosed with a fungus-infected aneurysm based on computed tomography and vascular surgery. The vascular tissue revealed some narrow-based budding yeast within the thrombus, suggesting Candida infection. Seventeen cases of infected aneurysm of the abdomen were reported in Korea from 1988 to 2007, although none were "true" fungus-infected aneurysms, making this the first fungus-infected aneurysm of the abdomen in Korea. Prompt diagnostic procedures and aggressive treatment modalities are necessary for patients with occult infection and negative blood cultures, regardless of their immunocompetence, because of the high morbidity and mortality of this condition.
Abdomen ; Aneurysm ; Aneurysm, Infected ; Aorta, Abdominal ; Candida ; Fever ; Fungi ; Humans ; Immunocompetence ; Korea ; Risk Factors ; Saccharomycetales ; Thrombosis