Chordoma arising from the notochordal remnants is a rare primary bone tumor in the cervicosacral region and is even more unusual in the thoracic region. The authors experienced a case of thoracic chordoma and report its CT and MR findings.
Chordoma* ; Notochord

Chordoma arising from the notochordal remnants is a rare primary bone tumor in the cervicosacral region and is even more unusual in the thoracic region. The authors experienced a case of thoracic chordoma and report its CT and MR findings.
Chordoma* ; Notochord

BACKGROUND: Hereditary spherocytosis(HS) is a clinically and biochemically very heterogeneous disorder The purpose of this study is to detect erythrocyte membrane protein abnormalities by SDS-PAGE and to investigate the frequency of erythrocyte membrane protein defects in hereditary spherocytosis and correlation between some of the hereditary spherocytosis biochemical subsets and the selected clinical phenotype. METHODS: We evaluated the clinical and laboratory characteristics of 14 normal healthy persons and 23 hereditary spherocytosis patients and 8 their family members. The patients were divided into three groups based on clinical and hematological severity(mild, typical, severe). In addition to routine hematologic determlnatlons, osmotic fragility and autohemolysis, RBC membrane protein analysis were performed in all patients by densitometric tracing of SDS-PAGE(sodium dodecyl sulphate polyacrylamide gel electrophoresis) stained by Coomassle blue utilizing both the discontinuous buffer system of Laemmli with acrylamide linear gradient from 4% to 12% and the continuous buffer system of Fairbank with exponential gradient of acrylamide from 3.5% to 17%. RESULTS: 1) The patients could be seperated into three classes of different clinical severity as mild(3 cases), moderate(16 cases) and severe(4 cases) on the clinical feature. 2) Eighteen patients(82.6%) among 23 hereditary spherocytosis revealed abnormal erythrocyte membrane protein and we detected six patients(26.1%) with spectrin deficiency combined with ankyrin reduction, 4 patients(17.4%) with ankyrin deficiency, 4 patients(17.4%) with isolated spectrin deficiency and 3 patients(13.0%) with band 3 deficiency. Five HS patients(21.7%) showed normal RBC membrane protein. 3) Eight HS and their family members showed same RBC membrane protein deficiency. 4) The type and degree of RBC membrane protein reduction were variale with spectrin at 66~94%, with ankyrin at 48~82% of normal levels. These showed that each patient had different clinical severities according to different RBC membrane protein levels and type. CONCLUSION: RBC membrane protein abnormalities were observed in 82.6% of HS patients. The combined spectrin and ankyrin deficiency is the most common molecular defect in HS. The clinical severity and biochemical expression is heterogeneous. SDS-PAGE analysis of RBC membrane protein was provided the diagnosis of RBC membrane defects and basic molecular studies. We believed that the early identification of the biochemical defect responsible for HS is important because it is helpful starling point for the identification of the primary molecular defect, and it could help to anticipate the clinical outcome of the disease. For these reasons, we consider the SDS-PAGE of the red cell membrane to be of crucial importance for a complete evaluation of children with HS. Further studies with more cases would be to clarify the correlation between clinical and biochemical phenotypes.
Acrylamide ; Ankyrins ; Cell Membrane ; Child ; Diagnosis ; Electrophoresis, Polyacrylamide Gel* ; Erythrocyte Membrane* ; Erythrocytes* ; Erythrocytes, Abnormal ; Humans ; Membrane Proteins ; Membranes ; Osmotic Fragility ; Phenotype ; Population Characteristics* ; Spectrin ; Starlings

Excessive production of nitric oxide (NO) and proinflammatory cytokines from activated microglia play an important role in human neurodegenerative disorders. Here, we investigated whether celastrol, which has been used as a potent anti-inflammatory and anti-oxidative agent in Chinese medicine, attenuates excessive production of NO and proinflammatory cytokines such as TNF-alpha and IL-1beta in LPS-stimulated BV-2 cells, a mouse microglial cell line. We report here that the LPS-elicited excessive production of NO, TNF-alpha, and IL-1beta in BV-2 cells was largely inhibited in the presence of celastrol, and the attenuation of inducible iNOS and these cytokines resulted from the reduced expression of mRNAs of iNOS and these cytokines, respectively. The molecular mechanisms that underlie celastrol-mediated attenuation were the inhibition of LPS-induced phosphorylation of MAPK/ERK1/2 and the DNA binding activity of NF-kappaB in BV-2 cells. The results indicate that celastrol effectively attenuated NO and proinflammatory cytokine production via the inhibition of ERK1/2 phosphorylation and NF-kappaB activation in LPS-activated microglia. Thus, celastrol may be an effective therapeutic candidate for use in the treatment of neurodegenerative human brain disorders.
Animals ; Cell Line ; Cytokines/*biosynthesis/drug effects ; Gene Expression Regulation, Enzymologic/drug effects/immunology ; Inflammation/immunology ; Inflammation Mediators/immunology ; Mice ; Microglia/*drug effects/immunology ; Mitogen-Activated Protein Kinases/*physiology ; NF-kappa B/metabolism/*physiology ; Nitric Oxide/*metabolism ; Nitric Oxide Synthase Type II/biosynthesis/drug effects ; RNA, Messenger/analysis ; Signal Transduction/*drug effects/physiology ; Transcription, Genetic/drug effects/immunology ; Triterpenes/*pharmacology

While intussusception can occur at any age, the disease is most often seen in children and no etiologic factor can be seen in most cases of childhood intussusception. In contrast, the adult intussusception is rare and usually has an identifiable causes such as benign tumor, malignant tumor, sarcoma, Meckel's diverticulum and congenital anomaly. Especially, adult intussusception due to primary malignant lymphoma of small intestine is rare clinical condition. Here, we describe the case of a 49-year-old male patient with ileo-ileo-colic intussusception due to primary malignant lymphoma of the small intestine. The clinical, radiographic and pathologic findings are described with brief review of the literature.
Adult* ; Child ; Humans ; Intestine, Small* ; Intussusception* ; Lymphoma* ; Male ; Meckel Diverticulum ; Middle Aged ; Sarcoma

Tuberculoma of the spinal cord is one of rare lesions and usually produced by secondary lesion of tuberculosis. The first tuberculoma of spinal cord was described in 1830 by E.R.A. Serre and the first operative removal was performed in 1909 by Krauss and Mcguire. The most common age of tuberculoma is younger age group, especially under 30 years old and male is more frequent. The clinical symptom is rapid course and symptomatic Traid are motor, sensory, and sphincter disturbance, but early involvement is shpincter dist urbance. The authors present a case of intradural extramedullary tuberculoma of the spinal cord which was located at the thoracic region. The 63 years old female patient has complained the upper back pain and paraplegia by spontaneous onset before 3 days. On admission, paraplegia, urinary incontinence and weakness of anal sphincter were found. There were revealed within normal limit on simple flims of chest and thoracic spine. The color of the C.S.F. was xanthochromic, the amount of the protein was increased, and Quecken sted test was not patent. The contrast media was obliterated at the lower margin of 8th thoracic level and upper margin of 10th thoracic level, on myelogram by puncture of cisterna magna and lumbar region. We performed total laminectomy from T8 to T10 and removed a ID EM tuberculoma. We found white yellowish extramedullary tumor at the right postebrane. The extramedullary tumor was intermingling with dorsal nerve root on T9. The right dentate ligament and dorsal nerve root were cut and tumor was completely removed. Macroscopically the gross feature were 1.5 by 2 cm in size, white-yellowish color, hard and irregular round shape. The microscopic finding were showing multifocal granulomas with caseous necrosis. The granuloma was composed of epitheloid cells, lymphocytes and Langhans' giant cell. Post-operative recovery was very satisfactory.
Adult ; Anal Canal ; Back Pain ; Cisterna Magna ; Contrast Media ; Female ; Giant Cells ; Granuloma ; Humans ; Laminectomy ; Ligaments ; Lumbosacral Region ; Lymphocytes ; Male ; Middle Aged ; Necrosis ; Paraplegia ; Punctures ; Spinal Cord* ; Spine ; Thorax ; Tuberculoma* ; Tuberculosis ; Urinary Incontinence

BACKGROUND/AIMS: HBV infection can be seen after organ transplantation. The presence of anti-Bs in serum means protection from HBV infection. If amino acids were mutated in 'a' determinant which was a common antigenic epitope of HBsAg, escape from humoral immunity can occur. Recently, in chronic HBV infected patients who received liver transplantation but reinfected by HBV, many authors reported mutations in 'a' determinant sequence. However, in renal transplantation, there were few reports about HBV infection and 'a' determinant mutation after transplantation. Therefore, we studied the incidence of HBV reinfection after renal transplantation and also tried to analyze 'a' determinant sequence in those patients. METHODS: We reviewed HBsAg-egative patients who received renal transplantation in our hospital, but turned HBsAg positive after transplantation. We selected two patients who were anti-Bs positive before transplantation but turned HBsAg positive after transplantation, and analyzed 'a' determinant of amino acid sequence of these patients. RESULTS: Among 1682 patients who were HBsAg negative before transplantation, 21 patients were turned HBsAg positive after transplantation. Among them, 6 patients were anti-Bs positive before transplantation. Sequence analysis of the 'a' determinant amino acid in two patients whose HBsAg turned positive after transplantation revealed no evidence of mutation in comparison with previously reported subtype 'a' determinant sequences. CONCLUSION: In renal transplantation, HBV could be reinfected in patients who had been anti-Bs positive before transplantation even without mutation in 'a' determinant region.
Amino Acid Sequence ; Amino Acids ; Hepatitis B Surface Antigens ; Humans ; Immunity, Humoral ; Incidence ; Kidney Transplantation* ; Liver Transplantation ; Organ Transplantation ; Sequence Analysis* ; Transplants ; United Nations

OBJECTIVES: To investigate if vitamin D receptor (VDR) gene polymorphisms and circulating vitamin D levels are associated with pelvic floor disorders (PFDs). METHODS: In this case-control study, 25-hydroxy-vitamin D (25[OH]D) serum levels were analyzed in 47 females with PFDs and 87 healthy females (controls), respectively. The VDR gene polymorphisms were determined by using polymerase chain reaction and performing digestions with 4 restriction enzymes i.e., ApaI, TaqI, FokI, and BsmI. Vitamin D levels of patients were divided into <20 ng/mL, 20 to 30 ng/mL, and ≥30 ng/mL categories. RESULTS: Our correlative analysis of VDR polymorphisms as a function of the presence of PFD showed that ApaI and BsmI polymorphisms were significantly associated with PFD in vitamin-D-deficiency and insufficiency groups (P < 0.05). Mean vitamin D levels did not differ between the PFD case (13.01 ± 0.84 ng/mL) and control (15.11 ± 1.04 ng/mL) groups (P > 0.05). However, there was a significant difference in the distribution of vitamin D levels between study group and controls using Pearson's χ2 test (<20 ng/mL, 20–30 ng/mL, and >30 ng/mL: 87.2%, 12.8%, and 0% in the study group and 75.9%, 16.1%, and 8.0% in controls, respectively, P < 0.05). Taken together, our observations suggest that vitamin D levels could be associated with PFDs and that 2 polymorphisms (i.e., ApaI and BsmI) in the VDR gene may contribute to an increased prevalence of PFDs in women with insufficient levels of vitamin D. CONCLUSIONS: Examining vitamin D levels and performing a VDR genotype analysis may be helpful for assessing PFD risk.
Case-Control Studies ; Female ; Genotype ; Humans ; Pelvic Floor Disorders ; Pelvic Floor ; Polymerase Chain Reaction ; Prevalence ; Receptors, Calcitriol ; Vitamin D Deficiency ; Vitamin D ; Vitamins

The newly revised 2018 Organ Injury Scale (OIS) has a similar format to the previous American Association for Surgery and Trauma (AAST) Emergency General Surgery Grading System, dividing the criteria for grading solid organ damage into three groups; imaging, operation, and pathology. The most significant alteration in the OIS system 2018 revision is the incorporation of multidetector CT (MDCT) findings of vascular injury including pseudoaneurysm and arteriovenous fistula. Similar to the previous OIS, the highest of the three criteria is assigned the final grade. In addition, if multiple grade I or II injuries are present, one grade is advanced for multiple injuries up to grade III. This pictorial essay demonstrates the MDCT findings of solid organ injury grades based on the 2018 OIS system.

The newly revised 2018 Organ Injury Scale (OIS) has a similar format to the previous American Association for Surgery and Trauma (AAST) Emergency General Surgery Grading System, dividing the criteria for grading solid organ damage into three groups; imaging, operation, and pathology. The most significant alteration in the OIS system 2018 revision is the incorporation of multidetector CT (MDCT) findings of vascular injury including pseudoaneurysm and arteriovenous fistula. Similar to the previous OIS, the highest of the three criteria is assigned the final grade. In addition, if multiple grade I or II injuries are present, one grade is advanced for multiple injuries up to grade III. This pictorial essay demonstrates the MDCT findings of solid organ injury grades based on the 2018 OIS system.