In case of Wilson's disease complicated with hemolytic anemia and fulminant hepatic failure; plasma exchange or liver transplantation should be considered. We report an 11 year-old male with fulminant Wilson's disease who developed hemolytic crisis. He was recovered by exchange transfusion after 6 times of plasma exchange.
Anemia, Hemolytic ; Child* ; Hepatolenticular Degeneration* ; Humans ; Liver Failure, Acute ; Liver Transplantation ; Male ; Plasma Exchange

The GroEL heat-shock protein from Fusobacterium nucleatum, a periodontopathogen, activates risk factors for atherosclerosis in human microvascular endothelial cells (HMEC-1) and ApoE-/- mice. In this study, we analyzed the signaling pathways by which F. nucleatum GroEL induces the proinflammatory factors in HMEC-1 cells known to be risk factors associated with the development of atherosclerosis and identified the cellular receptor used by GroEL. The MAPK and NF-kappaB signaling pathways were found to be activated by GroEL to induce the expression of interleukin-8 (IL-8), monocyte chemoattractant protein 1 (MCP-1), intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule 1 (VCAM-1), E-selectin, and tissue factor (TF). These effects were inhibited by a TLR4 knockdown. Our results thus indicate that TLR4 is a key receptor that mediates the interaction of F. nucleatum GroEL with HMEC-1 cells and subsequently induces an inflammatory response via the MAPK and NF-kappaB pathways.
Animals ; Atherosclerosis ; Chemokine CCL2 ; E-Selectin ; Endothelial Cells ; Fusobacterium ; Fusobacterium nucleatum ; Heat-Shock Proteins ; Humans ; Intercellular Adhesion Molecule-1 ; Interleukin-8 ; Mice ; NF-kappa B ; Periodontitis ; Risk Factors ; Thromboplastin ; Toll-Like Receptor 4 ; Toll-Like Receptors ; Vascular Cell Adhesion Molecule-1

Envelope glycoprotein 1 (G1) and glycoprotein 2 (G2) of Hantaan (HTN) virus are believed to be major viral antigens that can induce neutralizing immunity against HTN virus infection. The purpose of this study is to clone and express G1 gene in an E. coli expression system. The truncated G1 gene (amino acid residues 35 to 123) of the HTN virus strain 76-118 was amplified by polymerase chain reaction (PCR). The 0.28 kb PCR product was cloned into pCR2.1 vector and named as pCGS1. The truncated G1 gene was excised from the pCGS1 and subcloned into the BamHI and SalI sites of pGEX-4T-2 and named pGGS1. The nucleotide sequence of the 0.28 kb truncated G1 gene was determined. It is revealed four non-silent nucleotide substitutions between the published sequence of strain HTN virus strain 76-118 and our stock of HTN virus strain 76-118 (passaged several times in our laboratory). The first G1 mutation was found to constitute an A to G nucleotide substitution, giving raise to an asparagine to serine mutation at residue 64. The second G1 mutation was found to constitute an A to C nucleotide substitution, giving raise to an lysine to threonine mutation at residue 112. The third G1 mutation was found to constitute an A to C nucleotide substitution, giving raise to an lysine to threonine mutation at residue 112. The fourth G1 mutation was found to constitute an G to A nucleotide substitution, giving raise to an glutamic acid to lysine mutation at residue 117. The truncated G1 gene was expressed as a 37 kDa protein fused to glutathione-S-transferase (GST). The GST fusion protein was purified by Glutathione Sepharose 4B affinity chromatography and reacted with the sera from patients of hemorrhage fever with renal syndrome (HFRS). One of 12 serum samples from HFRS patients was reactive with the 37 kDa fusion protein strongly. Three sera reacted moderately with the fusion protein. Six sera reacted only weakly with the protein, while remaing two were non-reactive. Control sera from patients with scrub typhus leptospirosis, or negative HFRS did not react with the recombinant fusion protein.
Antigens, Viral ; Asparagine ; Base Sequence ; Blotting, Western ; Chromatography, Affinity ; Clone Cells ; Fever ; Glutamic Acid ; Glutathione ; Glycoproteins* ; Hantaan virus* ; Hemorrhage ; Hemorrhagic Fever with Renal Syndrome ; Humans ; Leptospirosis ; Lysine ; Polymerase Chain Reaction ; Scrub Typhus ; Sepharose ; Serine ; Threonine

MspTL is the major surface protein of Treponema lecithinolyticum associated with periodontitis and endodontic infections. Our recent investigation revealed that MspTL induces proinflammatory cytokines and intercellular adhesion molecule 1 in THP-1 cells and periodontal ligament cells. In this study we conducted oligonucleotide microarray analysis to investigate the global transcriptional regulation in THP-1 cells stimulated with purified recombinant MspTL. MspTL upregulated the expression of 90 genes in THP-1 cells at least four fold, and the functions of these genes were categorized into adhesion, apoptosis/antiapoptosis, cell cycle/growth/differentiation, chemotaxis, cytoskeleton organization, immune response, molecular metabolism, proteolysis, signaling, and transcription. The majority of the modified genes are known to be NF-kappaB-responsive and interferon-stimulated genes (ISGs). The expression of 12 selected genes was confirmed by real-time RT-PCR. Because prostaglandin E2 (PGE2) is an important inflammatory mediator and Cox-2 was found to be induced by MspTL in the microarray analysis, we determined the level of PGE2 in the culture supernatants of MspTL-treated cells and found that MspTL significantly increased PGE2. Our results provide insight into the gene regulation of host cells in response to MspTL, and may contribute to the understanding of the molecular mechanism in periodontitis.
Chemotaxis ; Cytokines ; Cytoskeleton ; Dinoprostone ; Intercellular Adhesion Molecule-1 ; Microarray Analysis ; Monocytes ; Oligonucleotide Array Sequence Analysis ; Periodontal Ligament ; Periodontitis ; Proteolysis ; Treponema ; Up-Regulation

Impetigo herpetiformis is a rare and acute pustular eruption that is often accompanied by fever and leukocytosis, which occurs during the 3rd trimester of pregnancy and usually resolves after delivery. In general, impetigo herpetiformis is regarded as a variant of psoriasis that has histopathologic features of pustular psoriasis. The lesions typically begin in the flexural areas, but rarely involve the mucous membranes. The face, palms, and soles are commonly spared. However, we report a 29-year-old woman with impetigo herpetiformis showing mucosal, face and palm involvement, atypical skin manifestation.
Female ; Fever ; Humans ; Impetigo ; Leukocytosis ; Mucous Membrane ; Pregnancy ; Psoriasis ; Skin ; Skin Manifestations

Few recent reports have indicated that Mycobacterium massiliense causes various infections including respiratory infection. However, there is scarce information on the clinical significance, natural history of the infection, and therapeutic strategy. This report describes a case of an immunocompetent old man infected by M. massiliense that causes acute respiratory failure. In light of the general courses of non-tuberculous mycobacterium infections, rapid progression and fatality are very rare and odd. In addition, we discuss the biological and pathological properties of M. massiliense with the review of cases reported previously including our fatal one.
Light ; Mycobacterium ; Mycobacterium Infections ; Mycobacterium Infections, Nontuberculous ; Natural History ; Respiratory Insufficiency

No abstract available.
Baths* ; Dermatitis, Atopic* ; Water*

BACKGROUND: The average duration of diabetes and predictive factors of macrovascular complications in Korean diabetic patients remain to be elucidated. This study examines the average duration of diabetes up to the onset of macrovascular complications and clinically important factors of early development of these complications in Korean type 2 diabetic patients. METHODS: Clinical characteristics in type 2 diabetics with (n = 121) and without macrovascular complications (n = 115) were analyzed. In addition, early onset (< or = 5 years, n = 54) and late onset groups (> 5 years, n = 67) were compared, as were the clinical characteristics between male and female patients in the macrovascular complications group. RESULTS: The average duration of diabetes was 8.7 +/- 7.8 years in the macrovascular complications group. Average age, systolic and diastolic blood pressures and smoking history were all higher in the macrovascular complications group than the control group. However, HbA1c levels and prevalence of microvascular complications were higher in the controls. Average age was lower in the early onset group and many more patients of that group had a smoking history. In the analysis based on sex, marcrovascular complications developed earlier in male patients. In addition, the prevalence of family history of diabetes was higher in males and 77.8% of male patients had a smoking history (female: 3.4%). CONCLUSION: Our study confirms that older age, high blood pressure and smoking history are major risk factors for the development of macrovascular complications. Moreover, a smoking history in males can be both risk and predictive factors for earlier development of macrovascular complications in Korean type 2 diabetic patients. We also found that several clinical characteristics including age, family history of diabetes, hypertension and smoking history, vary between the sexes, and these findings can provide useful indices for the prevention of macrovascular complications.
Diabetes Complications ; Diabetes Mellitus, Type 2 ; Female ; Humans ; Hypertension ; Male ; Prevalence ; Risk Factors ; Smoke ; Smoking

PURPOSE: Pelvic floor muscle exercise (PFME) is a therapeutic option for urinary incontinence (UI). However, studies of the efficacy of PFME on UI in patients with cognitive impairment (CI) are lacking. Therefore, we evaluated the effect of PFME on UI in elderly women with mild CI. METHODS: A total of 150 women with mild CI or Alzheimer disease and UI were screened using the International Consultation on Incontinence Questionnaire-Short Form (ICIQ-SF). Cognitive function and behavioral symptoms were evaluated by the Mini-Mental State Examination and Barthel's Activities of Daily Living. The patients were randomly divided into a control group (n=46) and a PFME group (n=52, 6 sessions of PFME for 12 weeks). The primary outcome was the change in UI episodes measured with a frequency volume chart (FVC). The secondary outcomes were other FVC parameters and the ICIQ-SF scores. RESULTS: A total of 82 women (control group: 40 and PFME group: 42) completed the study. After 12 weeks of PFME, the mean number of UI episodes per 24 hours decreased by 1.6 (from 3.3 to 1.7) in the PFME group and by 0.5 (from 3.4 to 2.9) in the control group (P < 0.001 between groups). The mean number of micturition episodes and total ICIQ-SF scores improved in the PFME group to a significantly greater extent than in the control group (P<0.001). CONCLUSIONS: Supervised PFME can be a good therapeutic option for improving UI in elderly women with CI.
Activities of Daily Living ; Aged* ; Alzheimer Disease ; Behavioral Symptoms ; Cognition ; Cognition Disorders* ; Female ; Humans ; Pelvic Floor* ; Urinary Incontinence* ; Urination

BACKGROUND: The acidic pH of the stratum corneum (SC) is important for epidermal permeability barrier homeostasis. Acidification of the skin surface has been suggested as a therapeutic strategy for skin disorders such as atopic dermatitis (AD). OBJECTIVE: We performed an animal study to evaluate the usefulness of acidification of SC for inhibition of AD lesions and to find out if the therapeutic effect of vinegar is attributable to its herbal contents, rather than its acidity. METHODS: Five groups of six oxazolone-treated (Ox)-AD mice were treated for three weeks with creams of different acidity: vehicle cream alone (pH 5.5), neutralized vinegar cream (pH 7.4), pH 5.0 vinegar cream, pH 3.5 vinegar cream, and pH 3.5 hydrogen chloride (HCl) cream. Also, we have compared two groups of Ox-AD mice treated with pH 5.5 vehicle cream or pH 5.5 vinegar cream. RESULTS: Ox-AD mice treated with acidic creams exhibited fewer AD-like lesions, had significantly lower eczema scores, decreased basal by transepidermal water loss (TEWL), and increased SC hydration compared to the groups given only vehicle and neutral cream. There was no significant difference between the acidic vinegar and HCl groups. Between the groups treated with vehicle and pH 5.5 vinegar cream, there was no difference in eczema score, basal TEWL and SC hydration. CONCLUSION: Application of topical acids, regardless of their source materials, inhibits the development of AD lesions by maintenance of skin surface pH and skin barrier function in murine model.
Acetic Acid ; Animals ; Dermatitis, Atopic* ; Eczema ; Homeostasis ; Hydrochloric Acid ; Hydrogen-Ion Concentration ; Mice ; Permeability ; Skin* ; Water