Moyamoya disease is a particular disease of the circle of Willis which is characterized by its progressive obstruction and associated with different cerebral vascular malformations and infrequently associated with aneurysms. A 23 year old Korean woman was admitted to the Department of Neurosurgery, Korea University Hospital, because of stuporous mentality and Rt. Sided hemiplegia. She fell down and almost immediately lost consciousness on the day morning of admission. Neurological findings included neck stiffness, moderately bilateral dilated pupils, Rt. Sided hemiplegia, positive bilateral Babinski's sign and positive Kernig's sign. Lumbar angiogram revealed grossly bloody cerebrospinal fluid. Carotid angiogram revealed marked stenosis of supraclinoid portion of intracranial internal carotid arteries with extensive collateral small arterial net work at the base of the brain. Small aneurismal sac was visualized at the distal end of the small arterial nerwork probably distal portion of posterior choroidal artery. Brain computed tomography showed hematoma at the posterior portion of left side thalamic area and in the left lateral ventricle. The patient had operation for removal of hematoma and died on the 2nd day of postoperation. Based on this case, mechanisms of formation of aneurysms in moyamoya disease are discussed.
Aneurysm ; Arteries ; Brain ; Carotid Artery, Internal ; Cerebrospinal Fluid ; Choroid ; Circle of Willis ; Consciousness ; Constriction, Pathologic ; Female ; Hematoma ; Hemiplegia ; Humans ; Intracranial Aneurysm* ; Korea ; Lateral Ventricles ; Moyamoya Disease* ; Neck ; Neurosurgery ; Pupil ; Reflex, Babinski ; Stupor ; Vascular Malformations ; Young Adult

This study introduces the Clinical practice guidelines (CPGs) appraisal system by the Korean Academy of Medical Sciences (KAMS). Quality management policies for CPGs vary among different countries, which have their own cultures and health care systems. However, supporting developers in guideline development and appraisals using standardized tools are common practices. KAMS, an organization representing the various medical societies of Korea, has been striving to establish a quality management system for CPGs, and has established a CPGs quality management system that reflects the characteristics of the Korean healthcare environment and the needs of its users. KAMS created a foundation for the development of CPGs, set up an independent appraisal organization, enacted regulations related to the appraisals, and trained appraisers. These efforts could enhance the ability of each individual medical society to develop CPGs, to increase the quality of the CPGs, and to ultimately improve the quality of the information available to decision-makers.
Guideline Adherence/*standards ; *Models, Organizational ; Practice Guidelines as Topic/*standards ; Quality Assurance, Health Care/*standards ; Republic of Korea

BACKGROUND: QT dispersion(QTd) has been shown to be ventricular electrical instability, especially predictor of ventricular arrythmia and indicator of antiarrythmic effect. It was reported that there was a relationship between acute myocardial infarction and increased QTd in that QTd is dependent of the degree of reperfusion as well as the site and size of infarction. In this study, we intended to verify a significant association between myocardial ischemia and QTd by comparing the changes in QTd with or without chest pain in patients with unstable angina who had proven myocardial ischemic changes. METHOD: We studied 20 patients (12 men and 8 women : mean age, 58+/-3.4 years) with unstable angina who had proven myocardial ischemic changes and perfusion defect by 24 hour Holter monitoring, Treadmill test, or coronary angiography. Each case was measured QTd during chest patin and resting state 24 hours after chest pain. All standard 12-lead ECGs were recorded at a speed of 25 mm/sec and examined retrospectively by one observer. QTd corrected for heart rate (QTcd) was calculated by Bazett's formula. The difference of QTd was assessed by comparing by paired t-test. RESULTS: The mean values of QTd were 117.9+/-49.9 msec and 69.7+/-30.2 msec with existence and the absence of chest pain. There was significant increment of QTd when the paients with unstable angina had chest pain(p<0.01). QTcd also significantly increased with the mean value of 119.7+/-57.1 and 74.9+/-36.6 msec (p=0.015). CONCLUSIONS: The results of this study clarified the change of QTd with myocardial ischemia. We expect QTd using a single, noninvasive method to indicate that the chest pain is induced by myocardial ischemic changes. For the furture, it may be possible to study as to the significance of QTd as a predictor of cardiovascular accidents in patients with unstable angina by measuring the serial QTd.
Angina, Unstable ; Arrhythmias, Cardiac ; Chest Pain ; Coronary Angiography* ; Coronary Stenosis* ; Coronary Vessels* ; Electrocardiography ; Electrocardiography, Ambulatory ; Exercise Test ; Female ; Heart Rate ; Humans ; Infarction ; Male ; Myocardial Infarction ; Myocardial Ischemia ; Perfusion ; Reperfusion ; Retrospective Studies ; Thorax

Background: Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. Methods: We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. Results: A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. Conclusion This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.