Split hand split foot(SHSF) is a rare human developmental defect characterized by mi-ssing digits, fusion of remaining digits, and a deep median cleft in the hands and feet. Cy-togenetic studies of deletions and translocations associated with this disorder have indicated that an autosomal dominant split hand/split foot locus maps to 7q21-q22. We have experienced a case of ectrodactyly in a 25-year-old primigravida woman and her baby and reported out our experience with a review of related literature.
Adult ; Female ; Fetus* ; Foot ; Hand ; Human Development ; Humans

No abstract available.
Paget Disease, Extramammary*

We examined effects of interleukin 1 alpha (IL1 alpha) and phorbol 12, 13 dibutyrate (PDB), an activator of protein kinase C, on mRNA for Prostaglandin H synthase (PGHS) and prostanoid production in cultured ovine meningeal fibroblasts. Immuno- and morphologically-identified fibroblasts were derived from cerebral cortex and white matter from fetal lambs (approximately 120 days gestation) and grown to confluence on glass coverslips in 12 well plates. Levels of prostaglandin F2alpha and the stable hydrolysis product of prostacyclin (i.e., 6-keto-PGF1alpha) were determined using enzyme immunoassay. Relative amounts of mRNA were determined by in situ hybridization using ovine cDNA for PGHS1. IL1alpha (10 ng/ml) increased mRNA levels over baseline by 62 +/- 19% (p<0.05) at 60 min., 37 +/- 12% (NS) at 120 min., and 36 +/- 18 % (NS) at 240 min (n= 12). Levels of 6-keto-PGF1alpha were 148 +/- 18 pg/ml during baseline, 246 +/- 41 pg/ml at 60 min., 248 +/- 40 pg/ml at 120 min., and 259 +/- 62 pg/ml at 240 min (all p<0.05) (n=12). PGF2alpha was increased although it wasn't statistically significant. However, IL1alpha decreased PGE2 level significantly (all p<0.05). PDB (10-6 M) increased mRNA levels over baseline by 25+/-6% after 30 min., 40 +/- 6% after 60 min., and 20 +/- 8% after 90 min. (n=9) (all p<0.05). Levels of 6-keto-PGF1alpha were 200 +/- 43 pg/ml during baseline, 202 +/- 43 pg/ml after 30 min. (NS), 268 +/- 58 pg/ml after 60 min. (p<0.05), and 296 +/- 60 pg/ml after 90 min. (p<0.05) (n=9). Levels of PGF2alpha were 178 +/- 26 pg/ml during baseline, 300 +/- 30 pg/ml after 30 min., 299 +/- 35 pg/ml after 60 min., and 355 +/- 32 pg/ml after 90 min (all p <0.05) (n=6). Actinomycin-D (1 mg/ml) prevented increases in mRNA, 6-keto-PGF1alpha, and PGF2alpha at 60 min. for both I-L1 alpha and PDB. We conclude that cerebral fibroblasts are avid producers of prostanoids, and that enhanced production of PGHS is responsible for augmented PGF2alpha and prostacyclin production in the presence of an activator of protein kinase C and for decreased PGE2 and increased prostacyclin production in the presence of IL1alpha.
Cerebral Cortex ; Dinoprost ; Dinoprostone ; DNA, Complementary ; Epoprostenol ; Fibroblasts* ; Glass ; Hydrolysis ; Immunoenzyme Techniques ; In Situ Hybridization ; Interleukin-1alpha ; Prostaglandin-Endoperoxide Synthases* ; Prostaglandins ; Protein Kinase C ; RNA, Messenger*

Bone involvement is a common finding in many types of lymphomas, particularly in advanced stages. However, cranial vault affliction has been regarded as an exceedingly rare presentation. Here, we report the case of a patient with cranial vault lymphoma who presented with a scalp mass. An 81-year-old woman presented with a gradually growing and non-painful frontal scalp mass that she noticed one month before admission. It was a flatly elevated, round mass measuring about 6x4x4 cm. Computed tomography and magnetic resonance imaging of the brain revealed a contrast-enhancing intracranial extradural mass at the counter-location of the scalp mass. The superior sagittal sinus was involved at the tumor site. Cerebral angiography showed that the tumor feeding vessels originated from the bilateral external carotid arteries. An operation was performed and the tumors were removed together with the involved bone. The pathologic diagnosis was malignant diffuse large B-cell type lymphoma. The patient was transferred to the Hemato-Oncology department for chemotherapy. Primary lymphoma of the cranial vault with scalp mass is very rare but it should be considered in the differential diagnosis of scalp masses. Although the results of reported cases are variable, the combination of surgery, radiation, and chemotherapy appears to offer favorable outcomes.
Aged, 80 and over ; B-Lymphocytes ; Brain ; Carotid Artery, External ; Cerebral Angiography ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Female ; Humans ; Lymphoma* ; Magnetic Resonance Imaging ; Scalp ; Superior Sagittal Sinus

Multiple primary or secondary malignancies after anticancer therapy were recently reported to be increasing in frequency. The authors describe a case of metachronous metastatic pulmonary basaloid carcinoma to the central nervous system that was discovered after chemotherapy and radiation therapy for cervical uterine carcinoma. Two different types of cancer developed within some interval. There's the possibility that a secondary pulmonary neoplasm developed after the chemotherapy and radiotherapy conducted as cervical cancer treatment.
Central Nervous System ; Drug Therapy* ; Lung Neoplasms ; Neoplasm Metastasis* ; Neoplasms, Second Primary ; Radiotherapy ; Uterine Cervical Neoplasms

BACKGROUND: Hyponatremia in the elderly, one of the most common disorders seen in the emergency room (ER), has been shown to have a poor prognosis if treated improperly. However, it often is a challenge to detect because of its varying symptoms. This study aimed to improve the diagnosis and management of severe hyponatremia in the ER by analyzing clinical characteristics and patterns according to patient age. METHODS: Of the patients who visited the ER at Asan Medical Center in Seoul from May 1 to October 2006, 151 patients whose blood sodium concentration was 120 mEq/L or lower were examined in terms of gender, age, history, major symptoms, history of diuretic uses, and blood tests. RESULTS: The study group included 91 males and 60 females. Of these, 81 (53.6%) were middle-aged and 70 (46.4%) were elderly. For underlying diseases, 58 (38.4%) had liver disease and 79 (52.3%) had cancer. In the elderly group, more patients (21, 30%) visited the ER with neurological disturbances than with gastrointestinal symptoms (13, 18.6%); and more elderly patients (14, 20%) presented with serious hyponatremia (blood sodium concentration 110 or lower) than middle-aged patients (6, 7.4%). CONCLUSION: In the elderly, many patients visiting the ER did not have any underlying disease but were suffering from more severe hyponatremia. The major symptoms observed included neurological disturbances such as impaired consciousness and generalized weakness. The elderly can easily develop serious hyponatremia with accompanying neurologic disturbances. The emergency physician, having a high level of suspicion, should make a quick diagnosis and manage these patients appropriately.
Aged ; Consciousness ; Emergencies ; Female ; Hematologic Tests ; Humans ; Hyponatremia ; Liver Diseases ; Male ; Prognosis ; Sodium ; Stress, Psychological

OBJECTIVES: Few disciplines in medicine encompasses such wide diagnostic and treatment modalities as cardiology. A traditional text and education system is limited in its efficacy to convey such variety. In order to address this problem, a comprehensive computer-aided education system that compiles and links the current knowledge of cardiovascular diseases is proposed. An availability of such system through CD-ROM and world wide web will contribute undoubtedly to the promotion and betterment of current understandings of cardiology all over the world. METHODS AND RESULTS:1) The information about the cardiovascular diseases has been complied and organized. Multimedia medical information includes text, audio features that allow the users to study actual sounds, and visual aids (slides, real-time demonstration, and schematic illustrations).2) Cardiology education system can be approached according to "Disease module" or "Laboratory test module". One can readily look up the disease of interest and see the results of different tests. Or one can choose the laboratory test or physical examination and study the differences among the diseases. "Self test module" is also available to help user's self assessment of his/her understanding of cardiovascular diseases.3) This cardiology education program is conveniently stored in CD-ROM and can also be accessible through the world wide web (http://med.chungbuk.ac.kr/~mccho/ web-cardiology/alogo.html). CONCLUSION: A multimedia approach for the study of cardiovascular diseases has been developed and is now avaliable in CD-ROM. The same information can be obtained from our home page through the world wide web.
Audiovisual Aids ; Cardiology* ; Cardiovascular Diseases ; CD-ROM ; Education* ; Internet* ; Multimedia ; Physical Examination ; Self-Assessment

Polycythemia is a condition in which the red blood cell count is increased due to an inherited or acquired mutation, a physiologic response to hypoxia, autonomous erythropoietin production, or deliberate erythropoietin administration. Higher testosterone levels appear to act as a stimulus for erythropoiesis and testosterone replacement therapies have rarely been reported as causes of polycythemia. We report here a case of a 51-year-old man with polycythemia that was caused by long-acting testosterone undecanoate (Nebido(R)).
Anoxia ; Erythrocyte Count ; Erythropoiesis ; Erythropoietin ; Humans ; Middle Aged ; Polycythemia ; Testosterone

No abstract available.
Niemann-Pick Disease, Type A*

BACKGROUND AND OBJECTIVES: Mechanisms of restenosis following successful coronary angioplasty (PTCA) are knownasvascularsmoothmuscle cells(VSMCs)proliferationandmigration, elastic recoil or vascular wall remodeling. Paclitaxel whose effect on the stabilization of microtubles leads to cell death is highly lipophilic, permitting easy pass through cell membrane, and has a long-term antiproliferative effect. This study was performed to evaluate effect of paclitaxel on VSMCs proliferation and whether locally delivered paclitaxel can prevent stenosis and neointimal formation in rat carotid artery injury model. MATERIALS AND METHODS: Cultured VSMCs were exposed to sequential concentrations of paclitaxel in vitro, and proliferation inhibition was analyzed with 3H-thymidine incorporation. Paclitaxel of a suitable concentration was applied to the endothelium-denuded carotid artery of Fisher 344 inbred rats for 20 minutes. Angiogram and morphometric analysis of carotid artery was performed after 2 weeks. RESULTS: 3H-thymidine incorporation in cultured VSMCs was decreased dose-dependently from the concentration of 0.1 micromol/L (2,454+/-149cpm/ microgram protein) to 100 micromol/L (1,323+/-69cpm/ microgram protein) of paclitaxel by single and 20-minute exposure in the presence of platelet-derived growth factor (p<0.005). In the absence of platelet-derived growth factor, the decrement of 3H-thymidine incorporation was evident above the concentration of 5 micromol/L of paclitaxel. To evaluate in vivo effect, paclitaxel (0.1 or 1 micromol/L) was administered into the endothelium-denuded carotid artery by balloon injury and incubated for 20 minutes. Percent stenoses (32.2+/-9.8%) of paclitaxel-treated group was less than those (46.3+/-7.5%) of control group on histologic analysis (p<0.01). Paclitaxel-treated group also had wider lumen on carotid angiogram and less neointimal thickening than control on histologic examination (p<0.005). CONCLUSION: Proliferation of VSMCs was effectively inhibited and neointimal formation and luminal stenosis was prevented in rat carotid artery injury model by single, brief and local delivery of low-dose paclitaxel. This strategy could be applied to clinical settings for the prevention of restenosis after PTCA.
Angioplasty ; Animals ; Carotid Arteries* ; Carotid Artery Injuries ; Cell Death ; Cell Membrane ; Constriction, Pathologic ; Neointima ; Paclitaxel* ; Phenobarbital ; Platelet-Derived Growth Factor ; Rats*