CD4 and CD8 coexpressed T-lymphocytosis in adult onset Still's disease.
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Adult↗
Biomedical subjects
K S Song
Publications and source records attributed to K S Song.
Exogenous lipoid pneumonia: high-resolution CT findings.
The aim of this study was to assess high-resolution computed tomography (HRCT) findings of exogenous lipoid pneumonia. High-resolution computed tomography was obtained in 25 patients with proven exogenous lipoid pneumonia resulting from aspiration of squalene (derived from shark liver oil). Diagnosis was based on biopsy (n = 9), bronchoalveolar lavage (n = 8), or sputum cytology and clinical findings (n = 8). The clinical history of taking squalene was confirmed in all patients. The CT findings were classified into three patterns: diffuse ground-glass opacity, consolidation, and interstitial abnormalities. Distribution of the abnormalities, duration of taking squalene, predisposing factors for aspiration, and route of administration were analyzed. Ten patients showed diffuse ground-glass opacity pattern. Seven of 10 patients had predisposing conditions such as unconsciousness, pharyngeal dysmotility, or motor disturbances, and 6 patients had a recent history of taking large amount of squalene through nasal route. Seven patients who had consolidation pattern had a history of taking squalene for several months and did not have any predisposing factor. All of the 5 patients who had a pattern of interstitial abnormalities had a history of taking squalene longer than 1 year and showed segmental distribution of interstitial thickening with interposing ground-glass opacities. Three patients simultaneously had two different patterns at different lobes of the lung. The HRCT findings of lipoid pneumonia are ground-glass opacities, consolidation, and interstitial abnormalities. These HRCT findings with appropriate inquiries could be useful for diagnosis of exogenous lipoid pneumonia.
Adult↗
Role of CD95/Fas and its ligand in the regulation of the growth of human CD34(++)CD38(-) fetal liver cells.
The functional significance of CD95/Fas expressed by candidate hematopoietic stem cells (HSCs) from human fetal liver was studied by testing the effect of agonistic anti-CD95 monoclonal antibody (mAb) CH-11 and soluble CD95 ligand (sCD95L) on the growth of CD34(++)CD38(-)lineage cells in vitro. Candidate fetal HSCs exhibited a dose-dependent proliferative response to CH-11 as well as to sCD95L when combined with kit ligand (KL) + interleukin 3 (IL-3) under serum-deprived culture conditions. CH-11 mAb increased, in a synergistic fashion, the number of myeloid colony-forming unit culture (CFU-C) generated by candidate HSCs in liquid cultures with the cytokine combinations KL + IL-3, KL + granulocytemacrophage colony-stimulating factor, and KL + IL-6. CH-11 mAb and sCD95L also enhanced erythropoiesis supported by KL + IL-3 + erythropoietin (Epo). Furthermore, sCD95L was able to increase the number of megakaryocytes, granulocytes, and CD34- cells generated in the presence of KL + IL-3 + Epo + thrombopoietin. An analysis performed using Western blotting revealed that the membrane-bound CD95L (mCD95L) was expressed by both immature (total CD34+/++) and mature (CD34-) hematopoietic lin(-) FL cells. Among the CD34(++)lin(-)cells, both the freshly isolated CD38+ and CD38 subsets as well as CD95+ and CD95- cells constitutively expressed mCD95L, demonstrating that the CD95/CD95L system represents a paracrine and potentially autocrine regulator of early hematopoiesis. To study the role of the endogenously produced CD95L, we determined the effects of a neutralizing anti-CD95L NOK-1 on the growth of candidate HSCs. By blocking the endogenous CD95L with NOK-1 mAb, we observed an increase in CFU-C generated by candidate HSCs. We conclude that the endogenous CD95L has an inhibitory effect on fetal candidate HSCs, which can be blocked by sCD95L and CH-11 mAb.
ADP-ribosyl Cyclase↗
Prevalence of anti-beta(2) glycoprotein-I antibody in patients with primary or secondary immune thrombocytopenia.
We determined the prevalence of antibodies to beta(2) glycoprotein-I (beta(2) GPI) in sera of 57 patients with primary or secondary immune thrombocytopenia positive for platelet antibody (PAIgG) and in 16 healthy control subjects. Both isotypes (IgG and IgM) of anti-beta(2) GPI were detected by means of semiquantitative ELISA and platelet antibodies were measured by means of flow cytometry using FITC-labeled anti-human IgG. Six (10.5%) of 57 patients with immune thrombocytopenia had anti-beta(2) GPI, whereas none of the 16 healthy subjects had anti-beta(2) GPI. Four were positive for IgM anti-beta(2) GPI, one for IgG anti-beta(2) GPI, and one was positive both for IgM and IgG anti-beta(2) GPI. There was no significant association between the presence of anti-beta(2) GPI and the severity of thrombocytopenia. Our findings suggest that some patients with immune thrombocytopenia have anti-beta(2) GPI in addition to the increased PAIgG; however, the results do not support a major role for anti-beta(2) GPI in the pathogenesis of immune thrombocytopenia.
Journal Article↗
History of the Korean GLPs and the activities and perspectives of the Korean Society of GLP.
The Korean Society of Good Laboratory Practice (KSGLP) was established Dec. 10, 1998. The objectives of the KSGLP are to enhance the quality of domestic studies and the level of GLP compliance, in safety testing, and to promote information exchange among its members. The activities of KSGLP include: offering workshops and symposiums, linking with related governmental organizations, collecting GLP related information and providing the information to the related organizations, building international networks to collect information and to establish relationship, developing training materials and publishing periodicals, and other business necessary to achieve the objectives of the KSGLP. The KSGLP achieved its goals within a short period of time by offering workshops and symposia, and by providing important GLP related information in newspapers or via the KSGLP's internet homepage (www.ksglp.or.kr). The main role of the KSGLP will be to disseminate GLP technology nationwide. The KSGLP would like to help many labs that are preparing their facilities for GLP compliance. Further, the KSGLP is hoping to share GLP experiences with other members.
Drug Industry↗
Primary intracranial leiomyoma. Case illustration.
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Adult↗
von Willebrand disease with G4022A mutation (vWd Sungnam): a case report.
A 10-year-old male patient affected by type 2 von Willebrand disease (vWD) and his family members were investigated by hemostatic and molecular genetic studies. The propositus, who experienced frequent bleeding episodes, was characterized by a normal level of von Willebrand factor (vWF) antigen (54%), reduced vWF ristocetin cofactor activity (5%), decreased factor VIII clotting activity (25%) and absent high molecular weight multimers in the plasma. An exon 28 fragment coding for the A1 and A2 domains was amplified by polymerase chain reaction and sequenced. We found a heterozygous mutation (G4022A), producing an additional PstI restriction site, which resulted in the substitution of Arg578Gln. Family studies, including the parents and a brother, were negative for this mutation and vWF abnormalities were not observed. We confirmed that G to A mutation in the region of the platelet glycoprotein Ib binding domain of vWF causes the qualitative type 2 defect in von Willebrand disease.
Alanine↗
Incidence and possible reasons for discordant results between positive FDP and negative D-dimer latex assays in clinical specimens.
In general, FDP and D-dimer values have a correlation in clinical conditions associated with disseminated intravascular coagulation(DIC) or coagulation activation. However, there are some patients with discordant results who demonstrate elevated FDP and negative D-dimer results by latex agglutination assays. The incidence and possible reasons for the discordance between FDP and D-dimer results were investigated through simultaneous measurements (n = 763) from clinical patients with suspected DIC or coagulation activation. 24.8% (189/763) of samples with elevated FDP were negative for D-dimer assays by the latex agglutination method. Further detailed analysis on randomly-selected discordant samples (n = 41) revealed that the most common reason for the discordance was the lower sensitivity of the semiquantitative latex agglutination method for D-dimer, compared with quantitative enzyme or other latex immunoassay. The other contributing factors to the discordance were accelerated fibrinogenolysis without secondary fibrinolysis, elevated soluble fibrin monomer and rheumatoid factor.
Blood Coagulation Disorders↗
Apolipoprotein E polymorphism in non-diabetic patients with acute coronary syndrome.
Since a decade ago, apolipoprotein (apo) E polymorphism has been focussed as a risk factor for cardiovascular disease. ApoE plays a central role as a receptor ligand for the uptake of lipoproteins from the circulation. There was an agreement on apoE polymorphism being one of the major risk factors for coronary artery disease (CAD) by its effects on lipid profiles. However, the effects of apoE have not been noted in all populations and conflicting results in the risk of CAD have been noted. Recently, in situ expression of apoE on the atherosclerotic plaque has been studied. We, therefore, investigated the effects of apoE genotype on patients with acute coronary syndrome, including unstable angina and acute myocardial infarction, in non-diabetic patients. While we could not find significant risk effects of apoE on coronary artery disease and lipid profiles on simple comparison with the normal control group, we could find significantly decreased frequencies of apo epsilon 3 allele in patients with acute coronary syndrome compared with stable angina patients (77.8% vs 88.8%). We suggest that the apoE genotype could be associated with acute coronary events in CAD and further study with in situ biochemical methods will be needed on the effects of apoE polymorphism on plaque stability.
Apolipoproteins E↗
Absence of the prothrombin gene variant in Koreans.
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Genetic Variation↗
Caveolae, plasma membrane microdomains for alpha-secretase-mediated processing of the amyloid precursor protein.
Caveolae are plasma membrane invaginations where key signaling elements are concentrated. In this report, both biochemical and histochemical analyses demonstrate that the amyloid precursor protein (APP), a source of Abeta amyloid peptide, is enriched within caveolae. Caveolin-1, a principal component of caveolae, is physically associated with APP, and the cytoplasmic domain of APP directly participates in this binding. The characteristic C-terminal fragment that results from APP processing by alpha-secretase, an as yet unidentified enzyme that cleaves APP within the Abeta amyloid sequence, was also localized within these caveolae-enriched fractions. Further analysis by cell surface biotinylation revealed that this cleavage event occurs at the cell surface. Importantly, alpha-secretase processing was significantly promoted by recombinant overexpression of caveolin in intact cells, resulting in increased secretion of the soluble extracellular domain of APP. Conversely, caveolin depletion using antisense oligonucletotides prevented this cleavage event. Our current results indicate that caveolae and caveolins may play a pivotal role in the alpha-secretase-mediated proteolysis of APP in vivo.
Amyloid Precursor Protein Secretases↗
Prolyl endopeptidase inhibitors from caryophylli flos.
Three prolyl endopeptidase inhibitors were isolated and identified as luteolin, quercetin and beta-sitosterol-3-O-beta-D-glucopyranoside with IC50 of 0.17, 0.19 and 27.5 ppm, respectively. The inhibition of two flavonoids were non-competitive with substrate. Twenty authentic flavonoids were tested in order to investigate structure-activity relationship. No significant relationship was found in them, however, catechol moiety of B-ring and 7-OH group in flavonoid skeleton were seemed to be responsible for the stronger activity.
Hydrolysis↗
Vestibular schwannoma with repeated intratumoral hemorrhage.
A symptomatic hemorrhage from a vestibular schwannoma is extremely rare, despite its relatively high incidence in the cerebellopontine angle. Much more unusual are repeated intratumoral hemorrhages. The authors report on such a rare case of repeated intratumoral hemorrhage from a vestibular schwannoma, presenting with sudden exacerbation of headache, nausea and vomiting. This second hemorrhage was confirmed by CT scan on the day of the onset of symptomatic exacerbation, which was 9 days after the initial CT scan, showing an enlarged amount of blood within the tumor. The MRI scan confirmed this CT finding. After removal, the tumor proved to be a typical vestibular schwannoma of mixed Antoni types A and B. Thin-dilated vessels with hemorrhage and hemosiderin-laden macrophages were observed. The patient's postoperative course was uneventful. A total of 28 reported cases of a single intratumoral hemorrhage in vestibular schwannomas, and one reported case of repeated hemorrhage are reviewed.
Adult↗
Mediastinal teratoma: CT differentiation of ruptured and unruptured tumors.
OBJECTIVE: The purpose of this study was to differentiate ruptured from unruptured mediastinal teratomas using CT. MATERIALS AND METHODS; CT findings in 17 cases of surgically resected mediastinal teratomas were reviewed retrospectively. Preoperative rupture was found in seven patients during surgery. We compared the clinical symptoms and CT findings of ruptured tumors with those of unruptured tumors. On CT, we evaluated size, wall thickness, location of the mass, presence or absence of internal septation, homogeneity of the internal components of each compartment, calcification or fat within the mass, and ancillary findings in adjacent structures. RESULTS: Severe symptoms (chest pain or hemoptysis) were more commonly found in ruptured (71%) than in unruptured tumors. All ruptured mediastinal teratomas had a tendency to display inhomogeneity of the internal components, whereas 90% of unruptured masses showed homogeneous densities of internal components in each compartment of the mass. Ancillary CT findings in ruptured tumors included fat-containing masses in adjacent lung parenchyma in two patients, consolidation or atelectasis in the adjacent lung in three patients, pericardial effusion in one patient, and pleural effusion in four patients. CONCLUSION: In cases of mediastinal teratoma, CT findings of inhomogeneity of the internal components and changes in the adjacent lung parenchyma, pleura, or pericardium can be used as signs of tumor rupture.
Adult↗
Hereditary protein C deficiency with recurrent thrombosis: identification of a missense mutation (C6218T).
Protein C is the central component of a major anti-thrombotic regulatory system and individuals with hereditary protein C deficiency (PCD) tend to have an increased risk of thromboembolism. During the last several years, mutations causing PCD have been identified in Western countries and in Japanese. In the present study, we report a case of hereditary PCD with a missense mutation (C6218T) in a 44 year old female with recurrent pulmonary thromboembolism. The protein C activity (35%) and antigen (58%) levels in this patient were decreased. Furthermore, we have identified the same molecular defect and PCD in other asymptomatic family members including proband's mother and two daughters and one son. To our knowledge, this is the first case of hereditary PCD with identified genetic defect in the Korean population, which may be one of hot spots for mutation in the protein C gene.
Adult↗
Usual interstitial pneumonia: relationship between disease activity and the progression of honeycombing at thin-section computed tomography.
The authors estimate the relationship between the progression of honeycombing and disease activity of usual interstitial pneumonia (UIP) by open lung biopsy specimen and ground-glass opacity on thin-section computed tomography (CT). Open lung biopsy specimens and the initial and follow-up thin-section CT of 29 patients with proven UIP are reviewed. Follow-up thin-section CTs were performed from 2 to 61 months (mean, 15.3 months) after biopsy. The interval between the initial CT and open lung biopsy was from 2 to 30 days (mean, 10.0 days). Areas of ground-glass opacity and honeycombing were quantified respectively on each CT slice by using a 0%-100% scale with 10% increments. Each open lung biopsy specimen was scored semiquantitatively for alveolar desquamation, alveolar septal inflammation, inflammatory airway narrowing, obstructive pneumonitis, and lymphoid nodules. Patients were classified into either a mild or severe activity group according to the median value of the pathologic score and the median value of the area of ground-glass opacity. The authors compared the progression of honeycombing on follow-up thin-section CT between the groups. The progression of honeycombing in UIP was significantly faster in the severe activity group than in the mild group according to the pathologic score (p = 0.003) and the area of ground-glass opacity (p = 0.0024). In patients with UIP, more active inflammation of the pulmonary interstitium results in faster progression of honeycombing in long-term follow-up.
Adult↗
Recombinant expression of caveolin-1 in oncogenically transformed cells abrogates anchorage-independent growth.
Caveolae are plasma membrane-attached vesicular organelles. Caveolin-1, a 21-24-kDa integral membrane protein, is a principal component of caveolae membranes in vivo. Both caveolae and caveolin are most abundantly expressed in terminally differentiated cells: adipocytes, endothelial cells, and muscle cells. Conversely, caveolin-1 mRNA and protein expression are lost or reduced during cell transformation by activated oncogenes such as v-abl and H-ras (G12V); caveolae are absent from these cell lines. However, its remains unknown whether down-regulation of caveolin-1 protein and caveolae organelles contributes to their transformed phenotype. Here, we have expressed caveolin-1 in oncogenically transformed cells under the control of an inducible-expression system. Regulated induction of caveolin-1 expression was monitored by Western blot analysis and immunofluorescence microscopy. Our results indicate that caveolin-1 protein is expressed well using this system and correctly localizes to the plasma membrane. Induction of caveolin-1 expression in v-Abl-transformed and H-Ras (G12V)-transformed NIH 3T3 cells abrogated the anchorage-independent growth of these cells in soft agar and resulted in the de novo formation of caveolae as seen by transmission electron microscopy. Consistent with its antagonism of Ras-mediated cell transformation, caveolin-1 expression dramatically inhibited both Ras/MAPK-mediated and basal transcriptional activation of a mitogen-sensitive promoter. Using an established system to detect apoptotic cell death, it appears that the effects of caveolin-1 may, in part, be attributed to its ability to initiate apoptosis in rapidly dividing cells. In addition, we find that caveolin-1 expression levels are reversibly down-regulated by two distinct oncogenic stimuli. Taken together, our results indicate that down-regulation of caveolin-1 expression and caveolae organelles may be critical to maintaining the transformed phenotype in certain cell populations.
3T3 Cells↗
Mutational analysis of the properties of caveolin-1. A novel role for the C-terminal domain in mediating homo-typic caveolin-caveolin interactions.
Caveolin is a principal structural component of caveolae membranes in vivo. Recently, a family of caveolin-related proteins has been identified; caveolin has been retermed caveolin-1. Caveolin family members share three characteristic properties: (i) detergent insolubility at low temperatures; (ii) self-oligomerization; and (iii) incorporation into low density Triton-insoluble fractions enriched in caveolae membranes. Here, we have used a deletion mutagenesis approach as a first step toward understanding which regions of caveolin-1 contribute to its unusual properties. Two caveolin-1 deletion mutants were created that lack either the C-terminal domain (Cav-1DeltaC) or the N-terminal domain (Cav-1DeltaN); these mutants were compared with the behavior of full-length caveolin-1 (Cav-1FL) expressed in parallel. Our results show that the N-terminal domain and membrane spanning segment are sufficient to form high molecular mass oligomers of caveolin-1. However, a complete caveolin-1 molecule is required for conveying detergent insolubility and incorporation into low density Triton-insoluble complexes. These data indicate that homo-oligomerization and an intact transmembrane are not sufficient to confer detergent insolubility, suggesting an unknown role for the C-terminal domain in this process. To better understand the role of the C-terminal domain, this region of caveolin-1 (residues 135-178) was expressed as a glutathione S-transferase fusion protein in Escherichia coli. Purified recombinant glutathione S-transferase-C-Cav-1 was found to stably interact with full-length caveolin-1 but not with the two caveolin-1 deletion mutants. These results suggest that the C-terminal domain interacts with both the N-terminal and C-terminal domains of an adjacent caveolin-1 homo-oligomer. This appears to be a specific homo-typic interaction, because the C-terminal domain of caveolin-1 failed to interact with full-length forms of caveolin-2 and caveolin-3. Homo-typic interaction of the C-terminal domain with an adjacent homo-oligomer could provide a mechanism for clustering caveolin-1 homo-oligomers while excluding other caveolin family members. This type of lateral segregation event could promote caveolae membrane formation and contribute to the detergent insolubility of caveolins-1, -2, and -3.
Animals↗