No abstract available.
Primary Myelofibrosis*

BACKGROUND: Parkinson disease (PD) is caused by selective cell death of dopaminergic neurons in the substantia nigra. An early onset form of PD, autosomal recessive juvenile parkinsonism has been associated with a mutation in the parkin gene. The function of parkin is known to remove misfolding proteins and protect cell death. We aimed to investigate the role of parkin against oxidative stress in neuronal cells. METHODS: Parkin knockout embryonic stem cells (PKO ES cells) were differentiated into neurons by adherent monolayer culture method. Oxidative stress was induced by the treatment of 1-methyl-4-phenylpyridinium (MPP+) in neurons derived from wild type and PKO ES cells, and cell viability was examined by MTT assay. After exposure to MPP+, Tuj1-positive cell population was compared between PKO and wild type cells by fluorescence activated cell sorter (FACS) analysis. The activated caspase3 protein level was also measured by Western blot analysis, FACS and immunocytochemistry. RESULTS: There was no difference in the efficiency of neuronal differentiation between wild type and PKO ES cells. After exposure to MPP+, no significant differences were found in cell viability and Tuj1-positive cell population between the two groups determined by MTT assay and FACS analysis, respectively. The activated caspase3 protein levels examined by Western blot analysis, FACS and immunocytochemistry were not changed in PKO cells compared with those of wild type cells after MPP+ treatment. CONCLUSION: These results suggest that PKO neuronal cells including dopaminergic neurons are not sensitive to caspase3-dependent cell death pathway during the response against MPP+-induced oxidative stress.
1-Methyl-4-phenylpyridinium ; Blotting, Western ; Cell Death ; Cell Survival ; Dopaminergic Neurons ; Embryonic Stem Cells ; Fluorescence ; Immunohistochemistry ; Neurons ; Oxidative Stress* ; Parkinson Disease ; Parkinsonian Disorders ; Substantia Nigra

Over the physiologic range of heart rate, the duration of electrical systole(QT) is shorter than that of electromechanical systole(QS2), which reflects direct physiologic link between electrical repolarization and mechanical events of the cardiac cycle. The present study designed to test usefulness of systolic time intervals and QT-QS2(QT minus QS2) value as complication predictors, which may predict prognostic status of myocardial infarction. The studied subjects were consisted of 33 healthy adults as control(22 men, 11 women) and 35 patients with myocardial infarction. And studied patients were divided into 3 groups; group I of 14 patients with or without complication in acute stage, group II of 11 uncomplicated patients under recovery of chronic status, group III of 10 complicated patients under recovery or chronic status. The observation periods were 3 to 13 days in group I, 5 to 96 weeks in group II and III after their onset. The results were follows; 1) The normal ranges were defined as mean+/-2 standard deviations of the measured values of control, that of QTc were shorter than 431ms of man and 458ms of woman, that of QS2 I were longer than 503ms of man and 514ms of woman, that of PEP/LVET ratio was less than 0.40, that of QT-QS2 value was less than 0 ms respectively. 2) QTc of all patient groups were significantly prolonged compared with that of control, and that of group III was significantly longer than that of group II(481+/-25 vs 432+/-32, p<0.005). 3) The changes of QS2 I were not significant in all patient groups compared with QS2 I of control. 4) QT-QS2 value showed QT>QS2 in group I and III. and QTQS2 had positive predictive value of 82% for predicting probability of previous complicated status. We concluded that QT-QS2 value, including PEP/LVET ratio and QTc, could be used as complication predictors. But it is necessary to examine these indicators prospectively to confirm its clinical applicability.
Adult ; Female ; Heart Rate ; Humans ; Male ; Myocardial Infarction* ; Reference Values ; Sensitivity and Specificity ; Systole*

Two cases of giant cell tumor of bone diagnosed by fine needle aspiration cytology are decribed. Case 1 was a 28-year -old male who had pain sense for one year at the right distal thigh. His radiologic finding revealed a destructive cortical lesion with soft tissue extension at medial side of epiphysis of the distal femur. Case 2 was a 21-year-old female complaining pain at left distal forearm for eight months and showed a well-demarcated expansile osteolytic lesion with multisepatation, and cortical destruction at epiphysis and metaphysis of the left distal radius on the X-ray. Fine needle aspiration of each lesion was performed. The aspirate of the case 1 reveated moderate cellularity, which was composed of scattered giant cells of osteoclastic type and small round to oval monotonous stromal cells in large areas. Giant cells were evenly distributed in single or small groups and had irregular but abundant cytoplasms with 10 to 20 nuclei in the center. The nuclei showed ovoid shape, fine granular chromatin, and a small but conspicuous nucleolus, Stromal cells were dispersed in isolated pattern or sometimes aggregated in clusters and showed the same nuclei as those of giant cells and scanty cytoplasms, Comparing to case 1, case 2 had a more translucent abundant cytoplasm in the giant cells and more spindled stromal cells, All two cases revealed neither nuclear atypism nor increased abnormal mitoses in both giant and stromal cells, suggesting no evidence of malignancy. Therafter the lesions were treated with excision and curettage and histologically confirmed as giant cell tumors of the bone.
Biopsy, Fine-Needle* ; Chromatin ; Curettage ; Cytoplasm ; Epiphyses ; Female ; Femur ; Forearm ; Giant Cell Tumor of Bone ; Giant Cell Tumors* ; Giant Cells* ; Humans ; Male ; Mitosis ; Osteoclasts ; Radius ; Stromal Cells ; Thigh ; Young Adult

Amyloidosis is a disorder of protein metabolism in which amyloid can be accumulated in various tissues of the body. Primary localized amyloidosis can affect the bulbar or palpebral conjuctiva, Tenon`s capsule, tarsus, limbus, lacrimal gland, or orbit. Especially secondary localized amyloidosis of the cornea can occur after trauma. scar, and corneal opacity or can be associated with a chronic ocular inflammatory disorder. The authors diagnosed the secondary localized amyloidosis that developed in a 53-years-old female patient who had undergone penetrating keratoplasty. The diagnosis was confirmed by hematoxylin-eosin and Congo red staining, and transmission electron microscopic examination.
Amyloid ; Amyloidosis* ; Ankle ; Cicatrix ; Congo Red ; Cornea ; Corneal Opacity ; Corneal Transplantation* ; Diagnosis ; Female ; Humans ; Keratoplasty, Penetrating ; Lacrimal Apparatus ; Metabolism ; Orbit

Hyperthyroidism is a common condition which is initially managed with an antithyroid drug, either propylthiouracil or methimazole. Adverse reactions occur in 1-5% of patients taking antithyroid medications, most commonly transient, benign leukopenia and skin rash. Agranulocytosis and liver dysfunction are reactions which occur infrequently. Severe hepatic toxicity combined with agranulocytosis has been known as rare complication. Experimental studies have demonstrated that immunological mechanisms play an important role. While most patients are completely recovered after stop of medication, some patients can show grave prognosis. We report a case of propylthiouracil induced hepatitis with agranulocytosis.
Agranulocytosis* ; Exanthema ; Hepatitis* ; Humans ; Hyperthyroidism ; Leukopenia ; Liver Diseases ; Methimazole ; Prognosis ; Propylthiouracil

We conducted a retrospective study of 19 patients(21 eyes) that were confirmed as dermoid by pathologic study, from July, 1987 to June. 1992 at St. Mary's Hospital. Catholic University Medical College. The follow-up period was at least over 6 months We studied on age and sex distribution of the patients, site and portion of involvement and postoperative complications. The most frequent involved age was below 9 years of age(8 patients, 42.0%). The most common site of involvement was the conjunctiva(10 eyes, 47.6%). The most frequent involvement portion was inferiotemporal portion(15 eyes, 71.4%) We did not find any recurrence or remarkable postoperative complications.
Dermoid Cyst* ; Follow-Up Studies ; Humans ; Postoperative Complications ; Recurrence ; Retrospective Studies ; Sex Distribution

OBJECTIVE: The aim of this study was to test the psychometric properties of Korean version of 10 items of Drug Attitude Inventory (KDAI-10). METHODS: A total of 157 stabilized schizophrenic patients were entered into the study after completion of screening tests. They completed the KDAI-10. And then they were interviewed with various scales such as Subjective Response Scale (SRS), Positive and Negative Syndrome Scale (PANSS), Extrapyramidal Side Effects Rating Scale (ESRS), and Global Assessment of Functioning (GAF). Correlations of KDAI-10 with these scales were tested. RESULTS: The Cronbach's alpha used to measure the internal consistency of the scale, was high (0.83). Principal component analysis with varimax rotation produced 2 factors, which explained 50.37% of the total variances. The First factor including 6 items was the positive subjective feelings and the second factor including 4 items was the negative subjective feelings, which were identical to the original authors'. Individual item correlations with total scores were all statistically significant (r=0.48-0.72, p<.001). And correlations of KDAI-10 with SRS, PANSS, ESRS and GAF showed that they were significantly correlated. CONCLUSION: Although the results were not quite satisfactory, KDAI-10 was demonstrated to have good reliability and construct validity.
Humans ; Mass Screening ; Principal Component Analysis ; Psychometrics* ; Schizophrenia ; Weights and Measures

BACKGROUND: Polycystic ovary syndrome (PCOS) is the most common endocrinopathy causing anovulation in women of childbearing age. It has been well established that estrogen receptor-alpha knockout (ERalphaKO) mice display several pathologic ovarian phenotypes of PCOS. The aims of this study were to determine ovarian pathology in new ERalphaKO mice using a CreloxP approach and intra-ovarian ERalpha function as regulating key aspects of PCOS. METHODS: ERalphaKO mice, which were deficient in exon 3 of the ERalpha gene, were used. Immunohistochemical studies were done on ovaries of control and ERalphaKO mice using antibodies specific to ERalpha, ERbeta, inhibin-alpha, and alpha-smooth muscle actin (SMA), as well as histochemical staining using Sudan black-B. RESULTS: All ovaries of ERalphaKO mice were larger than control mouse ovaries and displayed a disrupted theca-interstitial tissue organization, multiple atretic follicles and multiple hemorrhagic cysts. None of the ERalphaKO mouse ovaries showed a corpus luteum. In addition, heavy deposition of Sudan black-B positive foamy cells was seen. The theca externa of preantral immature follicles and hemorrhagic cysts showed strong expression of alpha-SMA. CONCLUSIONS: ERalphaKO mice show hemorrhagic polycystic ovaries and hyperplasia of the theca externa. This study demonstrates that the ERalpha is the functional key to the pathogenesis of PCOS.
Actins ; Animals ; Anovulation ; Antibodies ; Corpus Luteum ; Estrogen Receptor alpha ; Estrogen Receptor beta ; Estrogens ; Exons ; Female ; Humans ; Hyperplasia ; Immunohistochemistry ; Mice ; Mice, Knockout ; Muscles ; Ovarian Follicle ; Ovary ; Phenotype ; Polycystic Ovary Syndrome ; Sudan ; Theca Cells

We present a rare case of spindle cell oncocytoma (SCO) of the sella turcica with malignant histologic features and rapid progression. A 42-year-old woman experienced bilateral blurred vision and was preoperatively misdiagnosed as having a pituitary macroadenoma on magnetic resonance imaging. After surgery, SCO was diagnosed by the histopathologic features of interlacing fascicles of spindle tumor cells with finely granular, eosinophilic cytoplasm. Focal anaplastic changes and necrosis were present. Immunohistochemically, the tumor cells were positive for vimentin, epithelial membrane antigen, S-100, galectin-3, and thyroid transcription factor 1. Four months later, the tumor had progressed, and second surgery with adjuvant radiotherapy was performed; the patients remains under observation. In this report, we proposed distinctive radiologic features for differential diagnosis between SCO and other pituitary tumors.