Pheochromocytoma is originated from chromaffin cell of sympathetic nervous system and associated with other disease, such as neurofibromatosis, duodenal carcinoid, medullary thyroid cancer and parathyroid adenoma. Especially, pheochromocytoma is developed more than 50% in neurofibromatosis associated with hypertension. In such cases, several clinical features documented as more frequent bilateral phochromocytoma, more associated with other neuroendocrine tumors and thus more poor prognosis. We can observe the sustained hypertension despite of surgical resection of tumors in pheochromocytoma cases. One of the possible reason of post operative sustained hypertension is the pheochromocytoma originated from minor organ of Zukerkandl that was not resected during operation. Untreated or delayed treated cases with pheochromocytoma were often expired by complication of hypertension such as cerebrovascular hemorrhage, myocardial infarction, etc. Thus, in neurofibromatosis with hypertension, screening of pheochromocytorna is very important for the early detection of tumor and more favorable prognosis. Recently, We experienced a case of neurofibromatosis associated with bilateral pheochromocy-toma expired by cerebral hemorrhage during operation, so we report the case with literature review.
Carcinoid Tumor ; Cerebral Hemorrhage ; Chromaffin Cells ; Hemorrhage ; Hypertension ; Mass Screening ; Myocardial Infarction ; Neuroendocrine Tumors ; Neurofibromatoses* ; Parathyroid Neoplasms ; Pheochromocytoma* ; Prognosis ; Sympathetic Nervous System ; Thyroid Neoplasms

A clinico-pathologic study with an immunohistochemical examination for c-erbB-2 expression in 54 cases of ductal carcinoma in situ and 16 cases of Paget's disease of the breast was performed. c-erbB-2 oncoprotein overexpression was observed in 45% (24/54) and 88% (14/16) of ductal carcinoma in situ and Paget's disease, respectively. The overexpression of c-erbB-2 oncoprotein was significantly correlated with the nuclear grade of tumors and inversely with the status of the estrogen receptor. c-erbB-2 was positive in 4 out of 5 patients with metastasis to axillary lymph nodes and 3 out of 4 patients who died of the disease. Prognostic significance of c-erbB-2 oncoprotein in ductal carcinoma in situ was highly suggested. The expression of c-erbB-2 oncoprotein in Paget's disease was well correlated with coexisting infiltrating or in situ ductal carcinoma. The high positive rate of c-erbB-2 oncoprotein in ductal carcinoma with Paget's disease could be understood with a recent hypothesis that c-erbB-2 oncoprotein is involved in promotion of cell motility and the spread of carcinoma cells.
Neoplasm Metastasis

PURPOSE: The purpose of this study is to prove the effects of topical anesthetics (EMLA) to control pain in preterm infants during invasive procedure (percutaneous central venous catheterization, PCVC). METHODS: A total of 18 preterm infants of birth weight <2,000 g and gestational age <36 weeks born at EulJi University Hospital, at Daejeon in 2010 were randomly included in this study. EMLA was applied in nine preterm infants 40 minutes before starting PCVC and placebo was applied in another nine preterm infants. Based on PIPP (preterm infant pain profile), we evaluated their heart rate, oxygen saturation before, 15 seconds during and 30 seconds after procedure. Behavioral responses were also recorded for 2 minutes after procedure. RESULTS: Experimental group showed significantly less behavioral response during procedure (P=0.005) and 2 min after procedure (P=0.001). Also, experimental group showed less increase in heart rate (P=0.033) and reduction of behavioral state (P=0.017). CONCLUSION: Despite limitation of small size in this study, experimental group showed lower pain score compared with placebo control group during catheterization. In neonatal care unit (NICU), we recommend the use of topical anesthetics such as EMLA to control pain during invasive procedures.
Anesthetics ; Birth Weight ; Catheterization ; Catheterization, Central Venous ; Catheters ; Central Venous Catheters ; Gestational Age ; Heart Rate ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Lidocaine ; Oxygen ; Prilocaine

BACKGROUND: During clinical practice we found that left ventricular systolic function(LVSF) has been normalized in some patients with cardiomyopathy. We investigated the echocardiographic and clinical factors affecting normalization of LVSF in these patients. METHOD: The patients with LV systolic dysfunction(EF<40%) were evaluated with echocardiography, coronary angiography and/or 201-Thallium SPECT and follow-up echocardiography(FUE) one year later. They had no coronary, valvular, congenital heart diseases. Consecutive 50 patients with improved LVSF(EF> or =55%) in FUE were defined to Group 1(mean age 57+/-16, male 21, female 29, mean follow-up 18+/-6 month) and another consecutive 50 patients with sustained decreased LVSF(EF<40%) and no increment of EF over 10% in FUE were defined to Group 2(mean age 56+/-14, male 32, female 18, mean follow-up 20+/-6 month). RESULTS: By univariate analysis, significant factors affecting normalization of LVSF were female sex, non-smoker, first experience of dyspnea, absence of bundle branch block in ECG, end-diastolic dimension of LV(LVEDD), end-diastolic volume of LV(LVEDV), LA size, less sphericity, presence of pericardial effusion, peak and end systolic wall stress. By multivariate analysis, LVEDD(Group 1: 61+/-7, Group 2: 71+/-7mm, p<0.001), LVEDV(Group 1: 139+/-59, Group 2: 190+/-51ml, p<0.01), absence of bundle branch block in ECG and 1st attack of symptom were significant. By Receiver operating characteristics curve analysis, area under curve of LVEDD and LVEDV were 0.859(95%CI: 0.775-0.920) and 0.805(95%CI: 0.681-0.896), respectively. LVEDD< or =64mm predicted normalization of LVSF with a sensitivity 76% and a specificity 86%. CONCLUSION: Determination of cardiac dimension and volume by echocardiography is very important to predicting normalization of LV systolic function in primary myocardial disease. And this results suggest that myocardial structural integrity may be important for recovery of LV function in clinical setting.
Bundle-Branch Block ; Cardiomyopathies* ; Coronary Angiography ; Dyspnea ; Echocardiography* ; Electrocardiography ; Female ; Follow-Up Studies ; Heart Diseases ; Humans ; Male ; Multivariate Analysis ; Pericardial Effusion ; ROC Curve ; Sensitivity and Specificity ; Statistics as Topic ; Tomography, Emission-Computed, Single-Photon

No abstract available.
Dermatomyositis*

No abstract available.
Abruptio Placentae* ; Female ; Pregnancy

No abstract available.
Abruptio Placentae* ; Female ; Pregnancy

BACKGROUND: Sevelamer is associated with reduced complications of chronic kidney disease-mineral bone disorder (CKD-MBD) resulted from hyperphosphatemia, which may contribute mortality, in CKD patients with dialysis. So far clinical outcomes of sevelamer on mortality and risk of cardiovascular mortality related to CKD-MBD are debating. Purpose of this study was to evaluate the effectiveness of sevelamer HCl on mortality of secondary hyperparathyroidism (SHPT), risk of cardiovascular mortality and, frequency of osteopathy in end stage renal disease (ESRD) patients with dialysis. METHODS: We retrospectively reviewed the electronic medical records of 536 patients with ESRD, who were admitted for moderate to severe SHPT, for 36 months. 75 patients who met inclusion criteria were evaluated for the efficacy of sevelamer (mean serum iPTH = 487.5 pg/mL). RESULTS: Sevelamer intervention was not associated with increased three-year survival time compared with non-sevelamers group [average survival month: 30.4 months in sevelamer group, 26.8 months in non-sevelamer group, p = 0.463]. Sevelamer intervention was not associated with significant mortality benefit and cardiovascular mortality benefit as compared to non-sevelamer group [sevelamer group: non-sevelamer group, all-cause mortality (iPTH > 600 pg/mL): 14.3% (1/34): 20% (1/41) p = 0.962, OR = 0.935, 95% CI, 0.058-14.98, heart disease mortality: 6.67% (2/30): 0% (0/32) p = 0.138]. Sevelamer was not associated with significantly lower cumulative incidence of osteopathy compared to non-sevelamer group (sevelamer group: non-sevelamer group, 5.9% (2/34):9.8% (4/41); p = 0.538; OR = 0.578; 95% CI, 0.099-3.367). CONCLUSION: Sevelamer was not associated with decreased all-cause mortality and risk of cardiovascular mortality compared to non-sevelamer group in ESRD patients with SHPT.
Cardiovascular Diseases ; Dialysis* ; Electronic Health Records ; Heart Diseases ; Humans ; Hyperparathyroidism, Secondary ; Hyperphosphatemia ; Incidence ; Kidney Failure, Chronic ; Kidney* ; Mortality ; Retrospective Studies ; Sevelamer*

Myotonic dystrophy is a multisystemic disorder inherited as an autosomal dominant trait. The characteristic clinical features include the presence of myotonia, atrophy of the muscles of the face and the sternocleidomastoids and numerous nonmusclar manifestations such as cataracts, frontal baldness, gonadal dysfunctions and cardiac abnormalities. We experienced one case of myotonic dystrophy with prolonged atrial flutter in 30-year-old male who was admitted because of palpitation. We present this case with reviewing literatures.
Adult ; Alopecia ; Atrial Flutter* ; Atrophy ; Cataract ; Gonads ; Humans ; Male ; Muscles ; Myotonia ; Myotonic Dystrophy*

STATEMENT OF PROBLEM: There are two methods of color choice for the esthetic restoration. One is visual shade matching which draws a comparison between shade guide and teeth in dentist's own eye and the other is using a digital shade analysis system recently introduced. Although the visual shade matching has a lot of problems, decision of color by this visual shade matching and the ways of expression for the decided color are still applicable to clinical dentistry. PURPOSE: This study is designed to investigate shade guides used in the dental clinics and laboratories have the same value using ShadeEye-NCC(R). dental chroma meter (Shofu Inc., Kyoto, Japan) using shade guide are evaluated. MATERIAL AND METHODS: At the first experiment, eight Vita Lumin Vacuum shade guides (Vident Inc., California, USA) were collected from the dental clinics. A1 and B1 shade tabs are chosen and the colors are analyzed five times each in both tooth and porcelain modes by digital shade analysis system, ShadeEye-NCC(R). In the second experiment, twelve Vita shade guides using practically in the dental clinics and laboratories were collected and also A1 and B1 shade tabs are chosen and the colors of A1 and B1 are analyzed one time each in both tooth and porcelain modes by ShadeEye-NCC(R). RESULTS AND CONCLUSION: There were significant differences among eight shade guides in terms of shade (chroma), value and hue in both of A1 and B1 (P < .05). Shade guides using in present both dental clinics and laboratories did not show significant differences, except A1 in the porcelain mode, it showed significant differences (P < .05) in the shade even though the shade tab has the same name.
California ; Dental Clinics ; Dental Porcelain ; Dentistry ; Eye ; Quinolinium Compounds ; Tooth ; Vacuum