Adequate assessment of plaque deposition levels in the brain of mouse models of Alzheimer disease (AD) is required in many core issues of studies on AD, including studies on the mechanisms underlying plaque pathogenesis, identification of cellular factors modifying plaque pathology, and developments of anti-AD drugs. The present study was undertaken to quantitatively evaluate plaque deposition patterns in the brains of the two popular AD models, Tg2576 and Tg-APPswe/PS1dE9 mice. Coronally-cut brain sections of Tg2576 and Tg-APPswe/PS1dE9 mice were prepared and plaque depositions were visualized by staining with anti-amyloid beta peptides antibody. Microscopic images of plaque depositions in the prefrontal cortex, parietal cortex, piriform cortex and hippocampus were obtained and the number of plaques in each region was determined by a computer-aided image analysis method. A series of optical images representing a gradual increase of plaque deposition levels were selected in the four different brain regions and were assigned in each with a numerical grade of 1-6, where +1 was lowest and +6, highest, so that plaques per unit in mm2 increased "sigmoidally" over the grading scales. Analyzing plaque depositions using the photographic plaque reference panels and a computer-aid image analysis method, it was demonstrated that the brains of Tg2576 mice started to accumulate predominantly small plaques, while the brains of Tg-APPswe/PS1dE9 mice deposited relatively large plaques.
Alzheimer Disease/genetics/*pathology ; Amyloid beta-Protein Precursor/genetics/metabolism ; Animals ; Disease Models, Animal ; Humans ; Mice ; Mice, Transgenic ; Plaque, Amyloid/*pathology

BACKGROUND: The aim of this study was to investigate the prevalence, clinical and laboratory findings of hereditary hemolytic anemia (HHA) in Korea from 1997 to 2006 and to develop the appropriate diagnostic approach for HHA. METHODS: By the use of questionnaires, information on the clinical and laboratory findings ofHHA diagnosed from 1997 to 2006 in Korea was collected and analyzed retrospectively. A total of 431 cases were enrolled in this study from 46 departments of 35 hospitals. RESULTS: The overall frequency of HHA did not change through the 10-year period for pediatrics but did show an increasing tendency for internal medicine. The overall male to female sex ratio did not show sex predominance (1.17:1), but a significant male predominance with a ratio of 1.49:1 was seen for pediatrics while a significant female predominance with a ratio of 1:1.97 was seen forinternal medicine. Of the total cases, 74.2% (282/431) were diagnosed before the age of 15 years. The etiologies of HHA were classified as red cell membrane defects, hemoglobinopathies, red cell enzyme deficiencies and unknown causes. There were 382 cases (88.6%) of red cell membrane defects with 376 cases (87.2%) of hereditary spherocytosis and 6 cases (1.4%) of hereditary elliptocytosis, 20 cases (4.6%) of hemoglobinopathies with 18 cases (4.2%) of beta-thalassemia, a case (0.2%) of alpha-thalassemia and a case (0.2%) of Hemoglobin Madrid, 7 cases (1.6%) of red cell enzyme deficiencies with 5 cases (1.2%) of glucose-6- phosphate dehydrogenase (G-6-PD) deficiency, a case (0.2%) of pyruvate kinase (PK) deficiency and a case (0.2%) of enolase deficiency, and 22 cases (5.1%) of unknown causes. The most common chief complaint in pediatric patients was pallor and that in adult patients was jaundice. In the red cell membrane defect group of patients, the level of hemoglobin was significantly higher than in adult patients. The mean corpuscular volume, mean corpuscular hemoglobin, corrected reticulocyte count, total and indirect bilirubin level and lactate dehydrogenase levels in the hemoglobinopathy group of patients were significantly lower than the values in the red cell membrane defect group of patients. The mean concentration of G-6-PD was 0.8+/-0.7U/1012RBC in the G-6-PD deficient patients, PK was 1.7U/1010 RBC in the PK deficient patient, and the level of enolase was 0.04U/g of Hb in the enolase deficient patient. CONCLUSION: The most prevalent cause of HHA in Korea during 1997 to 2006 was hereditary spherocytosis, but HHA by other causes such as hemoglobinopathy and red cell enzyme deficiency gradually increased with the development of molecular diagnostic methods and increasing general interest. However, the etiologies of HHA need to be pursued further in 5.1% of the patients. An systematic standard diagnostic approach is needed in a nationwide prospective study for correct diagnoses and appropriate management of HHA.
Adult ; alpha-Thalassemia ; Anemia, Hemolytic, Congenital* ; beta-Thalassemia ; Bilirubin ; Cell Membrane ; Diagnosis ; Elliptocytosis, Hereditary ; Erythrocyte Indices ; Female ; Hemoglobinopathies ; Humans ; Internal Medicine ; Jaundice ; Korea* ; L-Lactate Dehydrogenase ; Male ; Oxidoreductases ; Pallor ; Pathology, Molecular ; Pediatrics ; Phosphopyruvate Hydratase ; Prevalence ; Pyruvate Kinase ; Reticulocyte Count ; Retrospective Studies* ; Sex Ratio ; Surveys and Questionnaires

OBJECTIVE: This study was conducted to investigate the presence and pattern of Hyrtl anastomosis, and to examine the effect that each type has on obstetrical outcome. METHODS: This study was carried out from January of 2001 to May of 2003 on 904 randomly selected patients who had given birth by Cesarean section or vaginal delivery at Catholic University Holy Family Hospital. Immediately proceeding delivery, barium sulfate was infused into the umbilical artery. This was followed by umbilical artery dissection to grossly confirm Hyrtl anastomosis. In order to evaluate the effect that each type of anastomosis has on obstetrical outcome, we retrospectively examined chart for birth weight, Apgar score, results of cord blood gas analysis, obstetrical complications, etc. RESULTS: Out of the 904 cases studied, 99.7% possessed Hyrt's anastomosis with frequency according to type as follows: 50.8% oblique, 48.0% transverse, 1.8% of anastomosis between one umbilical artery and the branch of the other, 1.6% of fusion of branches of each umbilical artery, 0.6% of anastomosis represented by two separate vessels between umbilical arteries, 0.1% of anastomosis between branches of each umbilical artery, 0.1% lacking anastomosis, and 0.2% of a single umbilical artery. In terms of obstetrical outcome, there was no significant difference for term and preterm infants in the frequency of type of Hyrtl's anastomosis according to birth weight, maternal age, Apgar score, obstetrical complications, etc. CONCLUSION: In an attempt to verify the presence and type of Hyrtl anastomosis, 8 types of Hyrtl's anastomosis were found, of which the oblique and transverse variety were the most common. There was no significant difference in the types of Hyrtl's anastomosis in relation to obstetrical outcome. Frequency was also similar with oblique and transverse types being the most common. Further studies detailing anatomical features of and blood flow/volume through the anastomosis are required in order to better understand the effects of Hyrtl's anastomosis on obstetrical outcome.
Apgar Score ; Barium Sulfate ; Birth Weight ; Cesarean Section ; Female ; Fetal Blood ; Humans ; Infant, Newborn ; Infant, Premature ; Maternal Age ; Parturition ; Pregnancy ; Retrospective Studies ; Single Umbilical Artery ; Umbilical Arteries

BACKGROUND: Incorrect infusion of dopamine can be potentially life threatening. If the actual volume of a 100 ml intravenous bag or bottle used to mix dopamine solutions is greater than the labeled volume, overdilution of dopamine can occur, resulting in ineffective hemodynamic response. To determine the significance of dopamine overdilution induced by the excessive volume, dopamine concentration and hemodynamic effect were compared in the manually mixed dopamine and the manufactured premixed dopamine. METHODS: For 5% dextrose water (D5W) 100 ml intravenous bottle mixed with 160 mg (4 ml) of dopamine (group 1), D5W 96 ml mixed with 160 mg of dopamine (group 2), premixed dopamine with 1.6 mg/ml of concentration manufactured 2 months ago (group 3), premixed dopamine with 1.6 mg/ml of concentration manufactured 6 months ago (group 4), and D5W 100 ml intravenous bottle mixed with 160 mg (4 ml) of dopamine after removal of 4 ml dextrose water (group 5), dopamine concentration was measured by High performance liquid chromatography (HPLC). Hemodynamic data was obtained from 10 mongrel dogs for each group at baseline (T1), 15 minutes after dopamine infusion at a rate of 3 microgram/kg/min (T2), 8 microgram/kg/min (T3), and 15 microgram/kg/min (T4). RESULTS: Dopamine concentrations of group 1, 2, 3, 4, and 5 were 1.51+/- 0.09, 1.60 +/- 0.10, 1.63 +/- 0.06, 1.57+/- 0.08 and 1.57+/- 0.07 mg/ml, respectively. Group 1 showed a significantly low concentration (p< 0.05). There was no significant differences in all hemodynamic data between group 1, 2, 3, and 4. In group 1, however, there was no significant increase in both mean blood pressure at T4 and mixed venous oxygen saturation at T3 compared with T1. CONCLUSIONS: The actual volume of D5W in 100 ml intravenous bottle is greater than the labeled, and therefore can cause significant overdilution of dopamine. Premixed dopamine, however, has the same concentration and hemodynamic effects as the dopamine mixed manually but precisely.
Animals ; Blood Pressure ; Chromatography, Liquid ; Dogs ; Dopamine* ; Glucose ; Hemodynamics* ; Oxygen ; Water

The neonatal lupus syndrome is characterized by skin lesions, hepatic and hematologic abnormalities and congenital heart block. Congenital heart block which is believed to be caused by transplacental passage of the anti-Ro (SSA)/La (SSB) antibodies from mother to infant, is known to occur in 1 in 20,000 live births. In contrast to other manifestation of neonatal lupus syndrome, which usually subside within 6 months after birth, congenital heart block is a permanent and potentially fatal complication. We experienced a case of neonatal lupus syndorme with congenital complete heart block in a newborn of asyptomatic mother with anti-Ro (SSA)/ La (SSB) antibodies.
Antibodies ; Heart Block* ; Heart* ; Humans ; Infant ; Infant, Newborn ; Live Birth ; Mothers ; Parturition ; Skin

Hydranencephaly is a condition in which cerebral hemisheres are absent and reduced to fluid-filled sacs in a normal skull. Numerous causes have been proposed, but bilateral occlusion of the internal carotid arteries during early fetal development can explain most of the pathologic abnormalities. We evaluated a case of hydranencephaly by brain CT and magnetic resonance angiography. Magnetic resonance angiography showed flow within the vertebral and basilar arteries without internal carotid intracranial flow above the supraclinoid segment. A brief review of the related literature was given on this subject.
Basilar Artery ; Brain ; Carotid Artery, Internal* ; Fetal Development ; Hydranencephaly* ; Magnetic Resonance Angiography ; Skull

BACKGROUND AND OBJECTIVES: Dual-chamber pacing is commonly used as a pacing mode maintaining at-rioventricular synchrony. However, traditional technique for atrial J lead implantation is relatively time-consuming and not easy to get optimal sites for both atrial and ventricular leads, especially for less-experienced operators. We developed a new "push technique" for atrial J lead implantation. MATERIALS AND METHOD: This study included sixty-two consecutive patients (26 males 36 females, 55+/-10 years, 56 patients received DDD and 6 received AAI pacemakers) from Jan. 1992 through Dec. 1996. Atrial J leads were implanted using a "push technique", that is, simply advancing a straightened lead while holding the stylet in the pacing lead at the junction of the superior vena cava and the right atrium. We evaluated the early and long-term result of atrial J lead implantation by the "push technique". RESULTS: 1) Atrial leads were successfully inserted by the first or second trial of the "push technique" in the 62 patients (100%). The sensed P wave amplitude was 3.1+/-1.0 mV, pacing threshold 0.6+/-0.2 V at the pulse width of 0.5 ms, impedance 547.4+/-118.5 ohms at 5 V. 2) During follow-up of 28.1+/-15.7 months, significant changes in the pacing parameters and the dislodgement of atrial leads were not seen. CONCLUSION: The early and long-term result of atrial J lead implantation by the "push technique" was excellent. This "push technique" can be used as an easy alternative technique for atrial J lead implantation.
Dichlorodiphenyldichloroethane ; Electric Impedance ; Female ; Follow-Up Studies ; Heart Atria ; Humans ; Male ; Vena Cava, Superior

BACKGROUND: Reversibly dysfunctional myocardium caused by chronic reduction of coronary perfusion was named as hibernating myocardium. The clinical characteristics, however, was not well studied. METHOD: To observed the effects of revascularization on regional wall motion(RWM) abnormalities in patients with chronic ischemic heart disease, letf ventriculogram and dipyridamole stress Tl reinjection scan with reinjection imagine were done in 60 patients with angina or old myocardial infarction before and after PTCA. Subjects were divided into two groups, group A(21 patients, improved RWM after PTCA) and group B(39 patients, no change or aggravated RWM after PTCA). RESULTS: There were no significant differences in age and sex distribution between two group. Single vessel disease was more prevalent in group A (7/21, 33.3%) than in group B(1/39, 2.6%, p<0.05), and old myocardial infarction was more prevalent in group B(24/39, 61.5%) compared to group A(7/21, 33.3%, p<0.05). Reversible perfusion defects on Tl scan were found in 19 patients, 15 of whom(78.9%) showed improved RWM after PTCA. CONCLUSION: Hibernating myocardium was more prevalent in single vessel disease and angina patients. Sensitivity of Tl reinjection scan for detection of viable myocardium was 78.9%.
Coronary Artery Disease* ; Coronary Vessels* ; Dipyridamole ; Hibernation* ; Humans ; Myocardial Infarction ; Myocardial Ischemia ; Myocardium* ; Perfusion ; Sex Distribution

BACKGROUND: To evaluate the clinical efficacy of Simvastatin, a HMG-CoA reductase inhibitor, We ibsweved the changes of clinical characteristics and lipid profiles after Simvastatin administration in patients with hypercholesterolemia. METHODS AND RESULTS: Simvastatin 10mg was given once daily for 12 weeks in 35 patients (60+/-6.0 years : 14 male, 21 female) with hypercholesterolemia. High density lipoprotein-cholesterol (HDL-C) was increased from 38+-10 to 45+-9mg/dl(p<0.05). Simvastatin significantly decreased total cholesterol(TC) from 235+-15 to 181+-21mg/dl(23.0%), low-density lipoprotein cholesterol (LDL-C) from 164+-19 to 104+-18mg/dl(36.5%), TC/HDL-C from 7.0+-2.0 to 4.4+-1.1, LDL-C/HDL-C from 4.9+-1.7 to 2.5+-0.8(p<0.01 respectively). Apo B was decreased by 31%(119+-19 to 87+-15mg/dl), apo B/A1 ratio was decreased by 41%(1.2+-0.2 to 0.7+-0.2) amd lipoprotein(a) edcreased by 12%(33+-22 to 29+-17), while apo A1 was increased by 25%(104+-18 to 130+-23mg/dl, p<0.01 respectively). No patients complained of chest pain, but two had skin rashes. Creatine kinase and creatinine were not changed in all patients. CONCLUSIONS: Somvastatin is an effective and well tolerated cholesterol lowering agent in patients with hypercholesterolemia.
Apolipoprotein A-I ; Apolipoproteins ; Apolipoproteins B ; Chest Pain ; Cholesterol ; Creatine Kinase ; Creatinine ; Exanthema ; Humans ; Hypercholesterolemia* ; Lipoprotein(a) ; Lipoproteins ; Male ; Oxidoreductases ; Simvastatin*

Effusive constrictive pericarditis after open heart surgery is a rare complication occuring in 0.2% to 0.3%. Presenting symptoms after surgery are associated with right heart failure and an elevated jugular venous pressure is most common abnormal physical sign. Predisposing factors include hemorrhage, perioperative pericardial injury or inflammation, presence of postpericardiotomy syndrome and open pericardium. Early diagnosis is important because(1) if it is unrecognized, the patient may deteriorate clinically, and(2) if surgery is delayed, the patient may have an increased risk of operative death. Hereby we report a case of effusive constrictive pericarditis after ventricular septal defect repair, in which constriction physiology was suggested by Doppler echocardiography after pericardiostomy.
Causality ; Constriction ; Early Diagnosis ; Echocardiography, Doppler ; Heart Failure ; Heart Septal Defects, Ventricular* ; Hemorrhage ; Humans ; Inflammation ; Pericardial Window Techniques ; Pericarditis, Constrictive* ; Pericardium ; Physiology ; Postpericardiotomy Syndrome ; Thoracic Surgery ; Venous Pressure