Angiomyolipoma is considered by many authors to be a hamartoma, occurring in a sporadic form or in association with tuberous sclerosis. This lesion consists of thick walled blood vessels, smooth muscle, and mature adipose tissue in varying amounts. We have experienced a case of the angiomyolipoma composed of monotypic epithelioid cells. The patient was a 69-year-old female. Clinically, there was no evidence of tuberous sclerosis. Microscopically, the tumor was composed of polygonal cells with abundant eosinophilic granular or clear cytoplasm, pleomorphic nuclei, prominent nucleoli, and multinucleated giant cells. The tumor cells showed positive reaction for HMB45, CD68, smooth muscle actin, and S-100, and negative reaction for epithelial membrane antigen, cytokeratin, vimentin, desmin, CD34, estrogen receptor, and progesterone receptor. Ultrastructual analysis showed the presence of glycogen, mitochondria, and other microorganelles in neoplastic cells. Melanosome or premelanosome was not identified.
Actins ; Adipose Tissue ; Aged ; Angiomyolipoma* ; Blood Vessels ; Cytoplasm ; Desmin ; Eosinophils ; Epithelioid Cells ; Estrogens ; Female ; Giant Cells ; Glycogen ; Hamartoma ; Humans ; Keratins ; Kidney* ; Melanosomes ; Mitochondria ; Mucin-1 ; Muscle, Smooth ; Receptors, Progesterone ; Tuberous Sclerosis ; Vimentin

OBJECTIVE: This study aimed to evaluate attention, memory and executive function in patients with narcolepsy. METHODS: This study included 23 narcoleptic patients whose diagnosis were confirmed by the International Classification of Sleep Disorders(ICSD) at Chonnam National University Hospital Sleep Disorders Clinic or an other hospital in Korea, from 2005 to 2008, as well as 23 normal controls. All participants were given an IQ test for Korean-Wechsler Adult Intelligence Scale and several neuropsychological function tests (the d2 test for attention function, the Rey Complex Figure Test for nonverbal memory, the Korean-California Verbal Learning Test [K-CVLT] for verbal memory, and the Wisconsin Card Sorting Test for executive function). Clinical features of narcoleptic patients, including the frequency of excessive daytime sleepiness, cataplexy, sleep paralysis and hypnagogic hallucination, were investigated by a structured clinical interview administered by a neuropsychiatist. Excessive daytime sleepiness was evaluated by the Epworth sleepiness scale. RESULTS: Characteristic symptoms of narcolepsy observed in this study included excessive daytime sleepiness (n=23, 100.0%), cataplexy (n=19, 82.6%), hypnagogic hallucination (n=5, 21.7%) and sleep paralysis (n=12, 52.2%). In nocturnal polysomnographic findings, stage 2 sleep and REM latency were found to be significantly decreased in narcoleptic patients compared with the control group, and were accompanied by significant increases in stage 1 sleep. Narcoleptic patients had lower scores than the control group on total number, Total Number-Total Error, Concentration Performance and Fluctuation Rate on the d2 test, which measures attention. Also, there were significant differences between the performance of patient and control groups on the B list of the K-CVLT, which measures verbal memory. CONCLUSION: Narcoleptic patients showed decreased attention and verbal memory performance compared to the control group; however, in many areas, narcoleptic patients still demonstrated normal cognitive function.
Adult ; Cataplexy ; Executive Function ; Hallucinations ; Humans ; Intelligence ; Korea ; Memory ; Narcolepsy ; Sleep Paralysis ; Sleep Wake Disorders ; Verbal Learning ; Wisconsin

No abstract available.
Aneurysm* ; Arteries* ; Cardiopulmonary Bypass* ; Circulatory Arrest, Deep Hypothermia Induced*

PURPOSE: The aim of this study was to investigate the effect of the maternal hypertension on the lipid peroxidation and antioxidant status of the newborn rat lung. METHODS: We used adult female SHR(essential hypertension, 1b, A, 6-8 weeks), Sprague-Dawley white rats as a model of hypertensive dams. Breeding was accomplished by allowing female and male rats to cohabit overnight. The animals were allowed to spontaneously deliver at term(21.5d). The pups delivered from the hypertensive dams grouped into HBP group. Control group consisted of the pups delivered from normal dams. The content of malondialdehyde(MDA) and activity of superoxide dismutase(SOD) and glutathione(GSH) in the lung tissues were analysed at birth and 24 hours later in both groups. RESULTS: The body weight of the HBP group was significantly lower than the control group at birth. At birth and 24 hours after birth, MDA content and SOD activity were significantly increased in the lung tissues of HBP group compared to control group. There were no significant differences in GSH activity between the two groups. After 24 hours, the elevated MDA value was significantly decreased, and SOD activity was significantly elevated in HBP group but there were no significant changes in the MDA value, SOD or GSH activity in the control group. CONCLUSION: These results support the possibility that maternal hypertension during pregnancy is associated with an increased lipid peroxidation and that compensatory activation of antioxidant system works in the fetal rat lung.
Adult ; Animals ; Body Weight ; Breeding ; Female ; Glutathione ; Humans ; Hypertension* ; Infant, Newborn* ; Lipid Peroxidation* ; Lung* ; Male ; Malondialdehyde ; Parturition ; Pregnancy ; Rats* ; Rats, Sprague-Dawley ; Superoxide Dismutase ; Superoxides

To report a novel mutation within the CHST6 gene, as well as describe light and electron microscopic features of a case of macular corneal dystrophy. A 59-year old woman with macular corneal dystrophy in both eyes who had decreased visual acuity underwent penetrating keratoplasty. Further studies including light and electron microscopy, as well as DNA analysis were performed. Light microscopy of the cornea revealed glycosaminoglycan deposits in the keratocytes and endothelial cells, as well as extracellularly within the stroma. All samples stained positively with alcian blue, colloidal iron, and periodic acid-Schiff. Electron microscopy showed keratocytes distended by membrane-bound intracytoplasmic vacuoles containing electron-dense fibrillogranular material. These vacuoles were present in the endothelial cells and between stromal lamellae. Some of the vacuoles contained dense osmophilic whorls. A novel homozygous mutation (c.613 C>T [p.Arg205Trp]) was identified within the whole coding region of CHST6. A novel CHST6 mutation was detected in a Korean macular corneal dystrophy patient.
Corneal Dystrophies, Hereditary/diagnosis/*genetics/metabolism ; Corneal Keratocytes/ultrastructure ; DNA/*genetics ; DNA Mutational Analysis ; Female ; Humans ; Microscopy, Electron ; Middle Aged ; *Mutation, Missense ; Pedigree ; Polymerase Chain Reaction ; Republic of Korea ; Sulfotransferases/*genetics/metabolism

No abstract available.
Sparganosis*

It was well received that well grounded behavioral theories were important in the development of effective nutrition education programs, but there are only a few programs available for Korean women. The objective of this study was to develop nutrition education programs for childbearing-aged women in Korea. Based on the findings of the needs assessment for the program and theoretical backgrounds, we developed behaviorally oriented tailored nutrition education program including motivation (MT), modifying (MD) and maintenance (MA) stages. The key concepts of the stages were motivation promotion for MT, increasing behavioral capabilities for MD, and strengthening self-management and building favorable environmental condition for MA. The education program was intended to be need in individual nutrition counseling, but it could be well used for group education by developing materials using the relevant contents. The primary users of the program were nutrition educators, however it could be also used by clients as needed. The introductory chapter provided dietary assessment tools and nutrition education tips. MT chapter included subjects such as nutritional status screening, costs of inappropriate nutrition and weight management, benefits of eating right, and activities for motivation promotion. MD stage chapter dealt with topics of healthy weight, knowledges and skills for better eating habits and physical activity status, and activities related to setting tailored behavioral objectives. MA stage focused on facilitating self management skills and building helping relationships. Each stage underlined activities using various educational tools in order to promote active participation of the client (s). For better use of this program, it was recommended to conduct program validation study.
Counseling ; Eating ; Education* ; Female ; Humans ; Korea* ; Mass Screening ; Motivation ; Motor Activity ; Needs Assessment ; Nutritional Status ; Self Care

Clear cell sarcoma(CCS) is an uncommon soft tissue sarcoma that occurs in tendons and aponeuroses, usually of the lower extremities and is believed to be of neural crest origin that have a capability to produce melanin. These tumors commonly metastasize and have a very poor prognosis. The fine needle aspiration cytologic finding of CCS is not well documented. We recently experienced a case of CCS. The patient was a 54-year-old male with painful swelling of the right inguinal area. Fine needle aspiration cytology revealed polygonal or fusiform tumor cells with clear or granular cytoplasm and vesicular nuclei containing one or two nucleoli. Im munohistochemical staining for S-100 protein and HMB-45 revealed strong positivity, and variable developing stages of premelanosomes were observed by electron microscopy in the excised specimen.
Biopsy, Fine-Needle* ; Cytoplasm ; Humans ; Lower Extremity ; Male ; Melanins ; Microscopy, Electron ; Middle Aged ; Neural Crest ; Prognosis ; S100 Proteins ; Sarcoma ; Sarcoma, Clear Cell* ; Tendons

PURPOSE: Genetic polymorphisms in antioxidant defense and detoxification genes may modulate the levels of oxidative stress biomarkers. METHODS: A total of 301 healthy preschool-aged children in the Seoul and Kyung-gi areas were recruited. DNA was extracted from blood for genotyping of manganese superoxide dismutase (Mn-SOD) Val16Ala, glutathione S-transferase (GST) P1 Ile105Val, GSTT1 present/null, and GSTM1 present/null polymorphisms by PCR-restriction fragment length polymorphism or multiplex PCR analyses. In addition to a questionnaire survey, the levels of urinary 8- hydroxyl-2-deoxiguanosine (8-OHdG) and plasma malondialdehyde (MDA) were measured by ELISA. RESULTS: Significantly higher urinary 8-OHdG concentrations were observed in GSTP1 Ile/Val + Val/Val genotype (p = 0.030), and tended to be higher in Mn-SOD Val/Val genotype (p = 0.065). On the other hand, exposure to environmental tobacco smoking (ETS) and interaction between ETS and gene polymorphisms did not significantly influence either urinary 8-OHdG concentrations or serum MDA. CONCLUSION: Based on our findings, GSTP1 Ile/Val gene polymorphisms might modulate the levels of oxidative stress biomarkers in healthy preschool children.
Biomarkers ; Child ; Child, Preschool* ; DNA ; Enzyme-Linked Immunosorbent Assay ; Genotype ; Glutathione Transferase ; Gyeonggi-do ; Hand ; Humans ; Malondialdehyde ; Multiplex Polymerase Chain Reaction ; Oxidative Stress ; Plasma ; Polymorphism, Genetic ; Seoul ; Smoking ; Superoxide Dismutase*

BACKGROUND: Bisphosphonate is used in osteoporosis treatment to repress osteoclast activity, which then decreases levels of osteocalcin (OC). OC, a protein secreted by osteoblasts and released from the bone matrix during osteoclastic bone resorption, has been found to control blood glucose levels by increasing insulin production and sensitivity. The question addressed in this study is whether decreasing OC through bisphosphonate treatment will provoke a change in glucose homeostasis. METHODS: Eighty-four patients with osteoporosis were treated with once-weekly risedronate 35 mg and cholecalciferol 5,600 IU. We measured fasting plasma glucose (FPG), insulin, and undercarboxylated (Glu) and carboxylated (Gla) OC levels at baseline and after 16 weeks. To estimate insulin resistance (IR) and beta-cell function (B)%, homeostasis model assessment (HOMA)-IR and HOMA-B% were also calculated, respectively. RESULTS: The mean FPG level in total subjects increased significantly from 5.3 to 5.5 mmol/L, but no changes in blood glucose were noted in the 24 subjects with impaired fasting glucose. Glu and Gla OC levels declined significantly after treatment. No correlations were observed between changes in OC and changes in glucose, however. CONCLUSIONS: Bisphosphonate treatment for osteoporosis reduced OC, but this change was not associated with changes in glucose metabolism.
Blood Glucose ; Bone Matrix ; Bone Resorption ; Cholecalciferol ; Etidronic Acid ; Fasting ; Glucose ; Homeostasis ; Humans ; Insulin ; Insulin Resistance ; Osteoblasts ; Osteocalcin ; Osteoclasts ; Osteoporosis ; Plasma ; Risedronate Sodium