Abnormalities in the number and function of platelets may contribute to thromboembolic complications in patients with essential thrombocythemia(ET). Physicians often use hydroxyurea which reduces the platelet count for the initial treatment of ET. A 74-year-old man with ET was presented with headache, dysarthria, and right hemiparesis 10 months after discontinuation of hydroxyurea. Initial platelet count was 450x103/microliter and we gave him heparin. However, his platelet count rised upto 1,019x103/microliter within 4 days. He was on hydroxyurea 1.5 g/day and his symptoms improved with decrease of platelet count. We report a case of left MCA(middle cerebral artery) multifocal infarction in ET after discontinuation of hydroxyurea.
Aged ; Cerebral Infarction* ; Dysarthria ; Headache ; Heparin ; Humans ; Hydroxyurea* ; Infarction ; Paresis ; Platelet Count ; Thrombocythemia, Essential*

The anatomical structure of vermiform appendix was studied for 124 Korean adult cadavers and the articles were reviewed with special reference to its position and length. The results are as follows ; 1. On the frequency of positional types based on Wakeley (1933), postileal type was recorded most frequently (37.7%) and retrocecal type was found in 19.7% of cases, showing the difference from other ethnic groups whose most frequent position was retrocecal or pelvic type. 2. In the direction of appendix in relation to the clock, the direction between 1 and 3 o'clock was most frequently found. 3. Appendix of fixed form was found in 22.1% of cases, that of free form 77.9%. In free form, postileal type was found most frequently, but retrocecal type was met most frequently in fixed form. 4. The length of appendix was 5.95±1.64 cm (male ; 6.14±1.67 cm ; female 5.59±1.51 cm). Neither significant difference between sex nor positional type was noted. 5. Positions of appendix are seemed to be set up during fetal stage. The Ethnic variation in the positions of appendix is thought to be determined by inherited physical anthropological factor, not by postnatal one.
Adult* ; Appendix* ; Cadaver ; Ethnic Groups ; Female ; Humans

Nevoid basal cell carcinoma syndrome (NBCC syndrome) is a very rare autosomal dominant dermatopathy characterized by a primary triad with variable secondary anomalies. The chief features include nevoid basal cell carcinomas, one or more skeletal anomalies, and multiple odontogenic keratocysts of the jaw. We report a case of NBCC syndrome in a 43-year old male who had multiple nevoid basal cell carcinomas on the retroauricular area, face, chest wall, and back, which have been present since childhood. Skull x-rays revealed relatively well-circumscribed cystic, radiolucent lesions on bilateral rami of both upper and lower jaws, calcification of the falx cerebri, agenesis of the right coronoid process, and a bifid chin. The cystic lesions were histologically confirmed as keratocysts.
Child ; Male ; Female ; Humans

Carcinoma, Merkel Cell*

Maspin is a recently described gene with tumor suppressor activity. The gene product is a 42 kD protein with homology to the serpin family of protease inhibitors and may play a role as an inhibitor of tumor cell invasion. The prior observation that invasive breast cancers and their metastases showed decreased maspin protein expression by immunostaining supports this speculation. However, the role of maspin in breast cancer progression has not been studied in detail. We, therefore, studied maspin protein expression in a series of hyperplasia, atypical ductal hyperplasia, intraductal carcinoma and invasive carcinomas. Immunohistochemical staining (IHC) for maspin was performed on paraffin sections of 136 breast specimens using a commercially available monoclonal antibody. Among the 106 cases studied were 36 moderate/florid ductal hyperplasia, 11 atypical ductal hyperplasia (ADH), 29 intraductal carcinoma (IDC) (4 low grade, 13 intermediate grade, 12 high grade) and 30 invasive ductal carcinomas. Thirty cases of normal breast were also studied as control group. IHC stains were scored using a semiquantitative scoring system. The mean IHC scores for maspin for normal, moderate/florid hyperplasia, atypical ductal hyperplasia, intraductal carcinoma, and invasive carcinoma were 5.51 1.30, 7.36 0.72, 3.82 1.60, 4.48 2.69, 3.97 3.30, respectively. These scores for each category were statistically significant (p<0.05), except between ADH and IDC. Maspin protein expression was increased in most cases of moderate/florid hyperplasia, while maspin expression was more heterogeneous in ADH and IDC. In high grade IDC, maspin protein expression was stronger than low and intermediate grade IDC, and this suggests the possibility of a compensatory cellular response against the forces driving further tumor progression. Two thirds of invasive ductal carcinomas expressed maspin protein weakly and focally. All metastatic carcinomas of lymph nodes were negative for maspin. It is possible that high grade IDC with strong maspin expression may represent a subset less likely to progress to invasive cancer. This speculation merits investigation in clinical outcome studies.
Breast Neoplasms ; Breast* ; Carcinoma, Ductal* ; Carcinoma, Intraductal, Noninfiltrating* ; Coloring Agents ; Humans ; Hyperplasia* ; Lymph Nodes ; Neoplasm Metastasis ; Outcome Assessment (Health Care) ; Paraffin ; Protease Inhibitors

The activation of the insulin-like growth factor-I-receptor system (IGF-IR) has recently emerged as critical events in transformation and tumorigenicity of several human tumors. In this study we investigated the expression of IGF-IR in 33 colorectal adenomas, 88 primary colorectal carcinomas, and 30 normal colonic mucosa adjacent to the carcinoma. Immunohistochemical staining (IHC) for IGF-IR was performed on paraffin embedded sections using an anti-IGF-IR rabbit polyclonal antibody. IHC stains for IGF-IR were scored using a semiquantitative scoring system. The relationship of IGF-IR staining to clinicopathologic variables and proliferating cell nuclear antigen (PCNA) staining was also analysed. The mean IHC scores for IGF-IR of normal glands, adenoma, intramucosal carcinoma, node-negative carcinoma, and node-positive carcinoma were 0.41 0.96, 0.76 1.23, 2.0 1.48, 2.83 2.0 and 5.93 1.58, respectively. These scores for each category were statistically significant except between normal glands and adenoma and between intramucosal carcinoma and node-negative carcinomas. The mean PCNA indexes of normal glands, adenoma, intramucosal carcinoma, node-negative carcinoma, and node-positive carcinoma were 2.48 2.60, 6.94 11.03, 27.21 11.42, 43.36 9.9 and 57.60 10.01, respectively. The PCNA index for each category was statistically significant except between normal and adenoma. IGF-IR scores and PCNA indexes were higher with tumor progression and also correlated each other (sr=0.65, p=0.0001). Higher IGF-IR scores and PCNA indexes were seen in tumors with advanced stage, infiltrative growth pattern, poor differentiation, nerve invasion, lymphovascular invasion, and moderate fibrosis. Our results suggest that IGF-IR plays an important role in tumorigenicity and tumor progression.
Adenoma* ; Colon ; Colorectal Neoplasms ; Coloring Agents ; Fibrosis ; Humans ; Immunohistochemistry ; Mucous Membrane ; Paraffin ; Proliferating Cell Nuclear Antigen

Hormone replacement therapy combined with progestogens induces changes in effect of estrogen on serum lipid levels and it has been known that the changes depend on a type and dosage of progestogen. It is also known that progestational agent induces positive ch-ange in bone mineral density. To study the effects of progestogen on lipoprotein and bone metabolism, we administ- ered conjugated equine estrogen 0.625 mg alone to 50 postmenopausal women, in combinat- ion with medroxy- progesterone acetate 5 mg to 40 postmenopausal women. The data demonstrated a beneficial effect in lipoprotein profiles in both groups. Total cholesterol in two groups decreased from the baseline values, LDL-cholesterol decreased significantly by 4.8 % in group I and 16.2 % in group II(p < 0.05), HDL-cholesterol increa- sed significantly by 11.3 % in group I and 14.7 % in group II(p < 0.05), triglyceride incre- ased slightly in both groups. Bone mineral density of femur was maintained and BMD of vertebrae increased by 1.1 % in group I and 2.0 % in group II, but it is not statistically significant. The differences of changes between two groups were not statistically significa- nt. Our results suggest that medroxyprogesterone acetate have no adverse effect on HDL -cholesterol and have no additive effect on bone mineral density in hormone replacement therapy.
Bone Density* ; Cholesterol ; Estrogens* ; Female ; Femur ; Hormone Replacement Therapy ; Humans ; Lipoproteins ; Medroxyprogesterone Acetate ; Metabolism ; Progesterone ; Progestins ; Spine ; Triglycerides

PURPOSE: We performed this study to describe the findings of MR Myelography(MRM) of herniated disc disease, spinal stenosis and spinal tumor and to evaluate the usefulness of the MRM in comparison to MRI. MATERIALS AND METHODS: MRI and MRM were performed in 31 patients with herniated disc disease(12 patients), spinal stenosis(11 patients) and spinal tumor(8 patients). MRI and MRM were done with 1.5-T Signa MR, using fat suppressed heavily T2-weighted fast spin echo technique. We retrospectively analyzed MRM images about the thecal sac indentation, compression or displacement of the nerve root, extent and degree of narrowing of spinal canal, relationship between spinal tumor and spinal cord. MRM findings were compared with MRI in all cases. RESULTS: In 18 herniated disc cases of 12 patients, focal filling defect with cutoff or displacement of the nerve root in eight cases of paracentral herniated disc was seen. Cutoff and displacement of the nerve root were more clearly delineated on MRM than rvlRI. In the patients of spinal stenosis(11 cases), hourglass deformity of the thecal sac or complete spinal block of the subarachnoid space was clearly demonstrated. The extent and severity of spinal stenosis were more accurately evaluated on MRM than MRI. MRM finding of intramedullary tumor(3 cases) was enlargement of spinal cord. Five cases of intradural extramedullary tumor showed intradural filling defect, which caused contralateral displacement of the spinal cord with meniscus sign on inferior margin of the mass. CONCLUSION: MRM shows characteristic findings of herniated disc disease, spinal stenosis and spinal tumor. MRM yields excellent definition of the thecal sac, nerve roots and nerve root sleeves in relation to herniated disc and may be more accurate in evaluation of the degree and extent of spinal stenosis than MRI.
Congenital Abnormalities ; Constriction, Pathologic ; Humans ; Intervertebral Disc Displacement ; Magnetic Resonance Imaging ; Myelography* ; Retrospective Studies ; Spinal Canal ; Spinal Cord ; Spinal Diseases ; Spinal Stenosis ; Subarachnoid Space

We report a patient with progressive supranuclear palsy (PSP) with his serial photographs before the onset of ocular symptoms and after the onset with two year intervals. These photographs show his progressive eyeball deviations toward complete exotropia. There were no effective voluntary eyeball movements, Bell's phenomenon, doll's eye movements, and vestibulo-ocular reflexes. These signs indicate the involvement of the oculomotor nuclear complex by the disease. We suggest that PSP may cause not only 'supranuclear' but also 'nuclear' complete ophthalmoplegia with exodeviation of the eyes.
Aged ; Exotropia/diagnosis ; Eye Movements ; Humans ; Magnetic Resonance Imaging ; Male ; Ophthalmoplegia/*diagnosis ; Supranuclear Palsy, Progressive/*diagnosis ; Time Factors ; Vision, Binocular

BACKGROUND: Trisomy 16 in mice is considered to be an animal model for Down's syndrome in human. We studied the morphologic characteristics of the heart and the edema, and their significance to the teratogenesis in this animal model. METHODS: A total of 30 dams were sacrificed to bear 125 (61.9%) normal fetuses 35 (17.3%) abnormal fetuses and 42 (20.8%) resorptions. Cytogenetic study and morphological examination were performed using microdissection agar-mount sectioning histologic examination and scanning electron microscope. RESULTS: The crown-rump length was significantly shorter in abnormal (trisomic fetuses) than the normal (eusomic) fetuses. Trisomic fetuses showed massive edema at the back from the vertex to the lumbar area. Four-chamber view section of the agar-mount and histologic section showed a common atrioventricular valve bridging the left and the right atrioventricular junction. Scanning electron microscopic examination on atrioventricular valves showed three types of atrioventricular valves:five cases with common atrioventricular orifice, three cases with partitioned atrioventricular orifice and two cases with atresia of the right atrioventricular orifice. CONCLUSION: This study confirmed the presence of massive edema and cardiac anomalies in the mouse with trisomy 16. But there was morphologic diversity of cardiac anomaly in this model.
Animals ; Crown-Rump Length ; Cytogenetics ; Down Syndrome ; Edema* ; Endocardial Cushion Defects ; Fetus* ; Heart ; Humans ; Hydrops Fetalis ; Mice ; Microdissection ; Models, Animal ; Pathology* ; Teratogenesis ; Trisomy*