Idiopathic myelofibrosis (IMF), which is characterized by marrow fibrosis, leukoerythroblastic anemia, teardrop poikilocytosis and splenomegaly due to extrumedullary hematopoiesis, has known to have no form of therapy. On the ground of the possibility of reversing collagen deposion in IMF using 1, 25dihydroxycholecalciferol [1, 25(OH)2D3], we report here our observations of 5 patients (M:F=1:4) with IMF before and after treatment with 0.5 microgram/day of alfacalcidol, precursor of 1, 25(OH)2D3. In 3 fo 5 patients the hemoglobin rose and in 4 of 5 the platelet count increased. Follow-up marrow examination revealed that marrow trephine reticulin fibrosis decreased according as the amelioration of clinical and laboratory findings. But these did not persist except one patient in spite of the sustained use of alfacalcidol. Our results suggest that alfacalcidol may have a therapeutic role in some patients with IMF. More extensive studies will be clarify the action of alfacalcidol in IMF.
Anemia, Myelophthisic ; Bone Marrow ; Child* ; Collagen ; Fibrosis ; Follow-Up Studies ; Hematopoiesis ; Humans ; Platelet Count ; Primary Myelofibrosis* ; Reticulin ; Splenomegaly

Hydranencehaly is an abnormality consisting of complete or almost absence of the cerebral hemispheres, which are replaced by a large fluid-filled cavity. The midbrain and brain stem are relatively intact, and rudiments of frontal, temporal and occipital cortex may be present. We diagnosed it through the evaluation of clinical features, prenatal US (intrauterine pregnancy, 37 wks) and brain CT. Authors have experienced a case of hydranencephaly combined with twin pregnancy and reported with a brief literature review.
Brain ; Brain Stem ; Cerebrum ; Humans ; Hydranencephaly* ; Mesencephalon ; Pregnancy ; Pregnancy, Twin* ; Twins*

This study was conducted to assess the relationship between estimated glomerular filtration rate (eGFR) and bone mineral density (BMD) in Korean postmenopausal women with mild renal dysfunction. A total of 328 postmenopausal women who underwent BMD measurement during health check-up was investigated. BMD was measured in lumbar spine (L1-L4), femoral neck, total proximal femur and femoral trochanteric areas by dual energy radiography absorptiometry and renal function was estimated by eGFR using Cockcroft-Gault equation. Of the 328 subjects, 317 (96.6%) had an eGFR > or =60 mL/min/1.73 m2. By using simple linear regression analysis, age, height, weight and eGFR were significantly associated with BMD for the 4 aforementioned anatomic sites, while serum levels of creatinine and blood urea nitrogen did not influence BMD. When multiple regression analyses were applied, age and body weight still had significant associations with BMD at 4 different anatomic sites (P < 0.001). A significant association of eGFR with BMD remained in the lumbar spine, femoral neck and proximal total femur (P < 0.05) but not in the trochanteric area (P = 0.300). Our study suggests that a decline of renal function is associated with lower BMD in the lumbar spine, femoral neck and total proximal femur areas in Korean menopausal women with mild renal dysfunction.
Absorptiometry, Photon ; Aged ; Blood Urea Nitrogen ; *Bone Density ; Creatinine/blood ; Female ; Femur Neck/physiology ; *Glomerular Filtration Rate ; Humans ; Kidney Diseases/*physiopathology ; Kidney Function Tests ; Lumbar Vertebrae/physiology ; Middle Aged ; Osteoporosis, Postmenopausal/*physiopathology ; Republic of Korea

Actinomycosis causes a chronic suppurative, granulomatous disease which is characterized by extensive abscess formation, and sulfur granule formation. Actinomycosis may present different clinical forms: cervicofacial, thoracic, abdominal and cerebral actinomycosis. The diagnosis can only be made after surgery. In general, patients with abdominal actinomycosis have undergone abdominal surgery. We report four cases of primary appendiceal actinomycosis presenting as acute appendicitis without history of abdomen surgery.
Abdomen ; Abscess ; Actinomycosis* ; Appendicitis* ; Diagnosis ; Humans ; Sulfur

The systematic study of products from bacteria and fungi has led to the development of two immunosuppressive drugs, cyclosporin A and FK 506 (tacrolimus) which are useful to suppress adaptive immune responses to the grafted tissue. However, they affect all immune responses indiscriminately and are both toxic to kidneys and other organs. To facilitate the development of immunosuppressor to block the T cell receptor (TcR)-mediated signaling cascade specifically, a novel Jurkat T cell transfectants, JK NFAT-SEAP were generated in which the expression of the secreted alkaline phosphatase (SEAP) is driven by the multiple NFAT binding sites plus minimal IL-2 promoter. Upon stimulation with ionomycin or anti-TcR mAb OKT3 in the presence of PMA, these transfectants secreted high level of SEAP into the medium, which was conveniently analyzed by SEAP analysis. The secretion of SEAP was effectively inhibited by cyclosporin A or FK 506 at the concentration of [10 ' ug/ml], [10 ug/ml] respectively. JK NFAT-SEAP transfectants will provide two major advantages for the development of a novel immunosuppressor. First, analysis of SEAP secreted into the culture medium by SEAP analysis enables us to test a large number of samples within a short period of time. Second, Usage of IL-2 promoter for the expression of SEAP makes us identify bioproducts to target specifically on TcR-mediated signaling pathway.
Alkaline Phosphatase ; Bacteria ; Binding Sites ; Cell Line* ; Cyclosporine ; Fungi ; Interleukin-2 ; Ionomycin ; Kidney ; Mass Screening* ; Muromonab-CD3 ; Receptors, Antigen, T-Cell ; T-Lymphocytes* ; Tacrolimus ; Transplants

The purpose of the present study is to evaluate the stress distribution on the length and diameter of the miniscrew and cortical bone width. Three dimensional finite element models were made of diameter 1.2mm, 1.6mm, 2.0mm and length 6.0mm, 8.0mm, 10.0mm, 12.0mm and cortical bone width 1.0mm. Also, another three dimensional finite element models were made of diameter 1.2mm, 1.6mm, 2.0mm and length 8.0mm and cortical bone width 1.0mm, 1.5mm, 2.0mm, 2.5mm. Two-hundred grams horizontal force were applied on the center of the miniscrew head and at that stress distribution and its magnitude had been analyzed by ANSYS, which is three dimensional finite element analysis program. The obtained results were as follows : 1. The comparison of the maximum von-Mises stress in the miniscrew showed that as the diameter increases from 1.2mm to 2.0mm stress has been decreased, while on the same diameter stress was not changed regardless of the length change. 2. The comparison of the maximum von-Mises stress in the cortical and cancellous bone showed that as the diameter increases from 1.2mm to 2.0mm stress has been decreased, while on the same diameter stress was not changed regardless of the length change. 3. In the analysis of the stress distribution in the cortical and cancellous bone, the most of the stress had been absorbed in the cortical bone, and did not transmitted much to the cancellous bone. 4. In the analysis of the maximum von-Mises stress according to the cortical bone width, the same diameter of the miniscrew showed a constant stress value regardless of the cortical bone width change. The above results suggest that the maintenance of the miniscrew is more reliable on diameter than length of the miniscrew.
Finite Element Analysis ; Head

PURPOSE: Infection with Shiga-like toxin (SLT)-producing Escherichia coli, an emerging human pathogen found particularly in young children under 5 years of age, causes a spectrum of illnesses with high morbidity and mortality, ranging from diarrhea to hemorrhagic colitis and hemolytic uremic syndrome. Host mediators play an important role in the pathogenesis of SLT-I toxicity. The experiments described here were designed to investigate the effect of SLT-I on TNF- alpha production and to understand the effect of TNF-alpha on GB3 expression. We also further examine the relationship between the Gb3 level and the differential susceptibility of cells to the cytotoxic action of SLT-I. METHODS: The effect of purified SLT-1 from E. coli O157 : H7 (ATCC 43890) on tumor necrosis factor-alpha (TNF- alpha) production in Raw264.7 cells was investigated. Many mediators regulate endothelial cell membrane expression of the glycolipid globotriaosyleramide (Gb3), which serves as the toxin receptor, suggesting that the host response to the toxin or other bacterial products may contribute to pathogenesis by regulating target cell sensitivity to the toxins. Therefore, the relationships between Gb3 expression and cytotoxicity against SLT-I on three types of cells were evaluated. RESULTS: Detectable levels of TNF-alpha were produced as early as six hours after induction and continued to increase during 48 hours by SLT-I. It was also found that Vero cells and dendritic cells (DC2.4 cells) expressed high levels of Gb3, 83% and 68%, respectively, and that Raw264.7 cells had a low level of Gb3 (29%) and appeared refractory to cytotoxicity against SLT-I. Vero cells and DC2.4 cells expressing high levels of Gb3 were highly susceptible to SLT-I. Furthermore, macrophages showed a resistance to SLT-I cytotoxicity, despite the fact that Gb3 expression was enhanced. CONCLUSION: These results strongly suggest that the expression of Gb3 is necessary but not sufficient to confer sensitivity of macrophages to SLT-I and further underpin the important role of SLT-I and its Gb3 receptors in the pathogenesis of E. coli O157 infection.
Child ; Colitis ; Dendritic Cells ; Diarrhea ; Endothelial Cells ; Escherichia coli ; Hemolytic-Uremic Syndrome ; Humans ; Macrophages ; Membranes ; Mortality ; Shiga Toxin 1* ; Tumor Necrosis Factor-alpha ; Vero Cells

Breast cancer is the most common type of female cancer. Tamoxifen, a selective estrogen receptor modulator, is widely used to decrease breast cancer recurrence and mortality among patients. However, it also increases the risk of endometrial cancer. This study aimed to assess knowledge and decisional conflict regarding tamoxifen use. Between June and October 2014, breast cancer patients using tamoxifen were consecutively screened and requested to complete a survey including the EQ-5D, Satisfaction with Decision Scale (SWD), Decisional Conflict Scale (DCS), and a self-developed, 15-item questionnaire measuring tamoxifen-related knowledge. The study sample comprised 299 patients. The mean total knowledge score was 63.4 of a possible 100.0 (range, 13.3-93.3). While 73.9% of the participants knew that tamoxifen reduces the risk of breast cancer recurrence, only 57.9% knew that the drug increases endometrial cancer risk. A higher education level (> or =college) was associated with a higher, total knowledge score (beta = 4.291; P = 0.017). A higher knowledge score was associated with a decreased DCS score (beta = -0.366; P < 0.001). A higher SWD score was also associated with decreased decisional conflict (beta = -0.178; P < 0.001). In conclusion, the breast cancer patients with higher levels of tamoxifen-related knowledge showed lower levels of decisional conflict regarding tamoxifen use. Clinicians should provide the exact information about tamoxifen treatment to patients, based on knowledge assessment results, so as to aid patients' decision-making with minimal conflict.
Adult ; Aged ; Antineoplastic Agents, Hormonal/adverse effects/therapeutic use ; Breast Neoplasms/*drug therapy/epidemiology ; Consent Forms/*statistics & numerical data ; Decision Making ; Endometrial Neoplasms/*chemically induced/epidemiology/prevention & control ; Female ; Health Knowledge, Attitudes, Practice ; Health Surveys ; Humans ; Middle Aged ; Patient Education as Topic/*statistics & numerical data ; Patient Participation/statistics & numerical data ; Prevalence ; Republic of Korea ; Risk Assessment ; Tamoxifen/*adverse effects/*therapeutic use

Neisseria cinerea is bacteria known as non-pathogenic strain. However, in rare cases, it can cause opportunistic infections. Those diseases caused by N. cinerea include neonatal ophthalmia, proctitis, pneumonia, peritonitis in patients with continuous ambulatory peritoneal dialysis, endocarditis and meningitis. In this report, we describe a patient with septic arthritis and skin abscess of finger joints that was caused by N. cinerea. A 27-year-old man visited the hospital due to swelling, redness and pain of proximal interphalangeal joint of the left second finger. After blood culture test, ceftriaxone was administered on admission and debridement was performed the affected joints. N. cinerea was identified in the blood culture. The patient was improved with ceftriaxone.
Abscess* ; Adult ; Arthritis, Infectious* ; Bacteria ; Ceftriaxone ; Debridement ; Endocarditis ; Endophthalmitis ; Finger Joint ; Fingers ; Humans ; Joints ; Meningitis ; Neisseria cinerea* ; Opportunistic Infections ; Peritoneal Dialysis, Continuous Ambulatory ; Peritonitis ; Pneumonia ; Proctitis ; Skin* ; Soft Tissue Infections

PURPOSE: To investigate the changes occurring in portal hemodynamics in patients with esophageal and gastric varices, according to variceal type, before and after TIPS. MATERIALS AND METHODS: Between January 1994 and June 1999, we evaluated 22 of 44 patients who had undergone TIPS and endoscopy on admission. In these 22, hepatic venous and main portal venous pressure were measured. On the basis of endoscpic findings, the esophageal and gastric varices were classified as one of three types. Changes in portal hemodynamics in relation to the diameter of the portal vein, mean portosystemic gradient before and after TIPS, delta MPSG, and the presence of hepatic encephalopathy and gastrorenal shunt were all evaluated. RESULTS: Endoscopy indicated that there were ten Type-I cases, nine Type-II, and three Type-III. The diameter of the main portal vein was 14.95 +/-1.79 mm in Type I cases, and 13.35 +/-1.59 mm in Type II. Before TIPS, main portal venous pressure was 31.40 +/-6.79 mmHg (Type I) and 22.80 +/-4.26 mmHg (Type II), and the mean portosystemic gradient was 16.10 +/-7.0 mmHg (Type I), and 11.20 +/-5.36 mmHg (Type II). After TIPS, the pressure readings were 25.70 +/-7.60 mmHg (Type I) and 17.80 +/-6.52 mmHg (Type II), while those relating to were 10.80 +/-4.94 mmHg (Type I) and 5.25 +/-3.67 mmHg (Type II). delta MPSG was 6.04 +/-2.98 mmHg (Type I) and 5.91 +/-3.98 mmHg (Type II). Angiography revealed that the gastrorenal shunt was Type I in 10% of cases, Type II in 77%, and Type III in 33%. Hepatic encephalopathy after TIPS occured in three Type-I cases, three-Type- II, and two Type-III. CONCLUSION: The diameter of the main portal vein was significantly smaller, and portal venous pressure and mean portosystemic gradient before and after TIPS significantly lower in patients with dominant gastric varices than in those with dominant esophageal varices (p<0.05). Gastrorenal shunt was more frequent among patients with dominant gastric varices. No difference in the incidence of hepatic encephalopathy after TIPS was noted between those with dominant gastric varices and those with the esophageal variety.
Angiography ; Endoscopy ; Esophageal and Gastric Varices* ; Hemodynamics* ; Hepatic Encephalopathy ; Humans ; Incidence ; Portacaval Shunt, Surgical ; Portal Pressure ; Portal Vein ; Portasystemic Shunt, Surgical* ; Reading