Dyke-Davidoff-Masson syndrome is a rare clinical syndrome consisting of hemiatrophy of cerebral hemisphere, enlargement of ventricles and prominent pneumatization of petrous pyramid of the temporal bone. Its clinical manifestations are hemiparesis, several types of seizure, mental retardation and other neurologic deficits. We experienced a case of Dyke-Davidoff-Masson syndrome in a four months old boy who visited to our department due to right side upper extremity weakness and infantile spasm. The brain magnetic resonance imaging showed marked hemiatrophy of left cerebral hemisphere and enlargement of left lateral ventricle. So with the review of literatures, we report a case of Dyke-Davidoff-Masson syndrome with infantile spasm.
Brain ; Cerebrum ; Humans ; Infant ; Infant, Newborn ; Intellectual Disability ; Lateral Ventricles ; Magnetic Resonance Imaging ; Male ; Neurologic Manifestations ; Paresis ; Petrous Bone ; Seizures ; Spasms, Infantile* ; Temporal Bone ; Upper Extremity

BACKGROUND: The high concentration of plasma total homocysteine is recently considered an independent risk factor for atherosclerosis. The purpose of this study was to provide reference ranges for plasma homocysteine levels and to investigate the relationship between plasma homocysteine and cardiovascular risk factors in healthy Korean men. METHODS: Anthropometric parameters, alcohol intake, cigarette use and nutrient intake were determined in 166 healthy men within a wide age range(30-69 yr). Serum levels of lipids, glucose and insulin levels during oral glucose tolerance test(OGTT), plasma amino acid concentrations and levels of antioxidant nutrients and enzymes were also measured. Hyperhomocysteinemia was defined as plasma homocysteine levels above the 90th percentile(> or =15micromol/L) of respective plasma homocysteine distribution in study subjects. Characteristics of hyperhomocysteinemic men(n=16) were compared to normohomocys- teinemic men(n=16) matched for age and body mass index. RESULTS: Plasma total homocysteine values ranged from 2.4 to 38.1micromol/L, a skewed, right-tailed distribution. The homocysteine levels of 25th, 50th and 75th percentile were 7.02, 9.61 and 12.4micromol/L, respectively. The mean concentration of plasma total homocysteine was 10.7micromol/L. Plasma total homocysteine level was positively correlated to body mass index, serum cholesterol and triglyceride levels and alcohol intake, but negatively correlated to serum bata-carotene concentration. In multivariate analysis, serum triglyceride level was the strongest determinant of plasma total homocysteine concentration. There were no significant differences between two groups in waist to hip ratio, alcohol intake, cigarette use, blood pressure and serum levels of glucose and insulin during OGTT. Hyperhomocysteinemic men had significantly higher mean values of serum triglyceride(258mg/dl), total cholesterol(226mg/dl), and LDL-cholesterol(140mg/dl) than normohomocysteinemic men. Hyperhomocysteinemic men showed a decrease in lipid corrected values of serum bata-carotene and alpha-tocopherol and plasma concentrations of serine and taurine, when compared to normohomocysteinemic men. The mean intakes of vitamin B6, folate, vitamin B12 and bata-carotene tended to decline by 25-30% in hyper- homocysteinemic group, when compared to normohomocysteinemic group. CONCLUSION: Our results indicate that healthy Korean men with hyperhomocys- teinemia show signs of hyperlipidemia and decreased antioxidants nutrients and these factors increase risk for coronary artery disease. In addition, hyperhomocysteinemia may affect amino acid metabolism related to homocysteine.
alpha-Tocopherol ; Antioxidants ; Atherosclerosis ; Blood Pressure ; Body Mass Index ; Cholesterol ; Coronary Artery Disease ; Folic Acid ; Glucose ; Glucose Tolerance Test ; Homocysteine* ; Humans ; Hyperhomocysteinemia ; Hyperlipidemias ; Insulin ; Male ; Metabolism ; Multivariate Analysis ; Plasma* ; Reference Values ; Risk Factors* ; Serine ; Taurine ; Tobacco Products ; Triglycerides ; Vitamin B 12 ; Vitamin B 6 ; Waist-Hip Ratio

No abstract available.
Endocrinology* ; Metabolism*

This erratum is being published to revise the website address of the Korean Reference Genome Database (KRGDB) and correct two typographical errors in the article.

No abstract available.
Humans ; Infant, Newborn* ; Streptococcus pneumoniae* ; Streptococcus*

Septo-optic dysplasia, as first described by de Morsier, is a rare developmental anomaly of absent or hypoplastic septum pellucidum, a primitive optic vesicle, and dysplasia of the optic nerve, chiasm, and optic tracts. It is a common cause of hypopituitarism in children and has variable hypothalamic-pituitary dysfunction. We experienced a case of septo-optic dysplasia in 5 month old female baby who presented with developmental delay and seizure. A magnetic resonance imaging of the brain showed absence of the septum pellucidum and the fundoscopic examination disclosed the absence of left optic disc and atrophy of right optic disc.
Atrophy ; Brain ; Child ; Female ; Humans ; Hypopituitarism ; Infant ; Magnetic Resonance Imaging ; Optic Nerve ; Seizures ; Septo-Optic Dysplasia* ; Septum Pellucidum ; Visual Pathways

No abstract available.
Humans ; Peripheral Nervous System Diseases* ; Pneumoconiosis*

BACKGROUND: Although current dietary guidelines recommend to increase the consumption of whole grain, these recommendations are mainly derived from the belief that replacing fats with carbohydrate may reduce risk of coronary artery disease (CAD) by improving serum lipids. Our objective was to evaluate whether the isocalorical replacement of refined rice with whole grain reduce CAD risk factors such as insulin demand and lipid peroxidation in CAD patients. METHODS: Thirty-eight male patients with CAD were provided with 70 g powder of whole grain (220 kcal) for 16 weeks, replacing cooked refined rice as a carbohydrate source of breakfast. An oral glucose tolerance test (OGTT) was performed in all subjects to determine the effect of whole grain consumption on serum concentrations of insulin and glucose in CAD patients with different degree of glucose tolerance. RESULTS: With the substitution of whole grain for refined rice, serum glucose concentrations decreased by 24% without altering body weight and energy intake. Estimates of daily fiber and vitamin E intakes increased by 24% and 50%, respectively. Whole grain consumption in CAD patients without diabetes decreased fasting glucose (22%) and the area under the curve (AUC) for insulin (26%) and glucose (19%) during an OGTT. CAD patients with diabetes also showed reductions in fasting glucose (27%) and AUC for glucose (25%) during the OGTT, compared with baseline values. Whole grain consumption reduced plasma malondialdehyde and homocysteine and urinary 8-epi-prostaglandin F 2alpha concentrations by about 30%. Lipid-corrected concentrations of alpha-carotene, retinol, alpha- and gamma-tocopherol and lycopene increased by 22-46%, compared with baseline values. Whole grain consumption decreased the percentage composition of w6 fatty acids of serum phospholipid increased by 14%. CONCLUSION: The replacement of refined rice with whole grain as a carbohydrate source of a meal showed significant beneficial effects on glucose, insulin and homocysteine concentrations and lipid peroxidation in CAD patients. These effects are likely to substantially reduce the risk factors of CAD and diabetes in CAD patients.
Area Under Curve ; Blood Glucose ; Body Weight ; Breakfast ; Edible Grain* ; Coronary Artery Disease* ; Coronary Vessels* ; Energy Intake ; Fasting ; Fats ; Fatty Acids ; gamma-Tocopherol ; Glucose ; Glucose Tolerance Test ; Homocysteine* ; Humans ; Insulin* ; Lipid Peroxidation* ; Male ; Malondialdehyde ; Meals ; Nutrition Policy ; Plasma* ; Risk Factors ; Vitamin A ; Vitamin E ; Vitamins

PURPOSE: The aim of this study was to compare the color stability, water sorption and cytotoxicity of thermoplastic acrylic resin for the non-metal clasp dentures to those of thermoplastic polyamide and conventional heat-polymerized denture base resins. MATERIALS AND METHODS: Three types of denture base resin, which are conventional heat-polymerized acrylic resin (Paladent 20), thermoplastic polyamide resin (Bio Tone), thermoplastic acrylic resin (Acrytone) were used as materials for this study. One hundred five specimens were fabricated. For the color stability test, specimens were immersed in the coffee and green tee for 1 and 8 weeks. Color change was measured by spectrometer. Water sorption was tested after 1 and 8 weeks immersion in the water. For the test of cytotoxicity, cell viability assay was measured and cell attachment was analyzed by FE-SEM. RESULTS: All types of denture base resin showed color changes after 1 and 8 weeks immersion. However, there was no significant difference between denture base resins. All specimens showed significant color changes in the coffee than green tee. In water sorption test, thermoplastic acrylic resin showed lower values than conventional heat-polymerized acrylic resin and thermoplastic polyamide resin. Three types of denture base showed low cytotoxicity in cell viability assay. Thermoplastic acrylic resin showed the similar cell attachment but more stable attachment than conventional heat-polymerized acrylic resin. CONCLUSION: Thermoplastic acrylic resin for the non-metal clasp denture showed acceptable color stability, water sorption and cytotoxicity. To verify the long stability in the mouth, additional in vitro studies are needed.
Cell Survival ; Coffee ; Denture Bases ; Dentures* ; Immersion ; Mouth ; Nylons ; Water*

Gynandroblastoma is an extremely rare sex cord-stromal tumor with both female (granulosa cell tumor) and male (Sertoli-Leydig cell tumor) elements. Juvenile granulosa cell tumors are also very rare and are so named because they usually occur in children and adolescents. A 71-year-old woman with right upper quadrant abdominal pain visited our hospital. Pelvic computed tomography showed a large multilocular cystic mass, suspected to be of ovarian origin. We performed a total abdominal hysterectomy (total abdominal hysterectomy was performed) with bilateral salpingo-oophorectomy. A 13-cm multilocular cystic mass with serous fluid was observed in her right ovary. Upon microscopic examination, the solid component of the mass showed both Sertoli-Leydig cell and juvenile granulosa cell differentiation, which we diagnosed as gynandroblastoma. Gynandroblastoma with a juvenile granulosa cell tumor component is extremely rare and, until now, only six cases have been reported in the English literature. We report the first gynandroblastoma with a juvenile granulosa cell tumor component diagnosed in an elderly patient, along with a literature review.
Abdominal Pain ; Adolescent ; Aged ; Child ; Female ; Granulosa Cell Tumor ; Granulosa Cells ; Humans ; Hysterectomy ; Male ; Ovary ; Postmenopause ; Sex Cord-Gonadal Stromal Tumors