Clinical and immunological profiles in 17 Japanese patients with drug-induced pemphigus studied at Kurume University.

BACKGROUND: Drug-induced pemphigus (DIP) shows clinical, histopathological and immunological features of pemphigus. However, little is known about immunological profiles in DIP. OBJECTIVES: To characterize clinical and immunological profiles in patients with DIP. METHODS: We studied 17 Japanese patients with DIP who were treated at Kurume University Hospital or who consulted from other hospitals between 1997 and 2012. Complicated diseases, clinical and histopathological manifestations, responsible drugs and findings in immunofluorescence, enzyme-linked immunosorbent assays (ELISAs), immunoblotting (IB) and prognosis were analysed. RESULTS: Eight of the 17 patients with DIP showed pemphigus foliaceus-like appearance, three showed pemphigus herpetiformis-like appearance, and six showed atypical bullous lesions. Responsible drugs were thiol-containing drugs in 16 patients (bucillamine in nine cases, d-penicillamine in four cases, and cetapril, thiopronine and captopril in one patient each), and a nonthiol drug, sulfasalazine, in one patient. By ELISAs and/or IB analyses, nine patients reacted only with desmoglein 1 (Dsg1), four reacted with Dsg1 and Dsg3, and four showed no specific reactivity. By IB of normal human epidermal extracts, in addition to positive reactivity with Dsg1, four patients with no detectable malignancy showed paraneoplastic pemphigus-like reactivity with the 210-kDa envoplakin and the 190-kDa periplakin. Four cases showed anti-Dsg3 antibodies without mucosal lesions. While 11 cases recovered after discontinuation of the causative drugs, six patients had a very protracted or intractable disease course, and might develop true pemphigus. CONCLUSIONS: The present study indicated that the majority of the patients with DIP studied showed a pemphigus foliaceus-type phenotype with anti-Dsg1 autoantibodies, caused by thiol-containing drugs.

[Video-assisted thoracic surgery for pneumothorax in patients over fifty years of age].

BACKGROUND: Pneumothorax in middle-aged and elderly patients has various different features from young patients. We set out to investigate the outcome and usefulness of video-assisted thoracic surgery (VATS) in this group. PATIENTS AND METHODS: From January 1993 to August 2010, 168 patients underwent a total of 178 thoracoscopic surgeries. There were 160 men and 8 women with mean age of 67 (range, 50 to 85). The average duration of thoracic drainage before surgery was 7.4 days. We excised only responsible lesions as minimum degree of dissection as possible. When patients have intractable air leakage and chest computed tomography (CT) indicates severe pleural adhesions, pleurography should be performed to locate the site of air leakage and determine surgical approaches. One hundred and seventy patients were treated with the lateral approach, while 8 patients were successfully treated with the anterior approach. One hundred and fifty-one patients were treated with VATS alone, and 27 patients were treated with the combination of VATS and small thoracotomy. The duration of chest tube drainage, hospital stay, post-operative complication, outcome and recurrence were assessed. RESULTS: The air leakages stopped and all patients except for 2 patents were discharged without drainage tubes. Postoperative drainage time was 4.5 +/- 3.4 days. Postoperative hospital stay was 9.9 +/- 13.9 days. One patient died caused by perioperative myocardial infarction. Seventeen patients died of other diseases. Seven patients needed local hospital treatments and the recurrence rate was 3.9%. CONCLUSION: VATS for pneumothorax in patients over 50 years old is very useful by appropriate perioperative managements.

In vitro bactericidal activity against periodontopathic bacteria by electrolyzed ion-reduced water.

As typical periodontopathic bacteria, Porphyromonas gingivalis (P. gingivalis) and Aggregatibacter actinomycetemcomitans (A. actinomycetemcomitans) were exposed to electrolyzed ion-reduced water (ERI) and ERI containing 1% sodium carboxymethylcellulose (CMC-Na) (ERI-1% CMC-Na), and the time course of their bactericidal action was evaluated. More than 99% of each bacteria species were killed after exposure to each solution for 15 sec. In addition, 1% CMC-Na, which was added to prolong bactericidal action, did not affect the bactericidal action of ERI. Its bactericidal action was concentration-dependent. No viable P. gingivalis bacteria were observed at a concentration of 15% of the undiluted solution and no viable A. actinomycetemcomitans bacteria were observed at a concentration of 50%, indicating differences in the bactericidal action of ERI for the two bacteria species. These results suggest that ERI may be extremely useful in preventing and treating periodontal diseases.

Structural transformations and anomalous viscosity in the B2O3 melt under high pressure.

Liquid B2O3 represents an archetypical oxide melt with a superhigh viscosity at the melting temperature. We present the results of the in situ x-ray diffraction study and the in situ viscosity measurements of liquid B2O3 under high pressure up to 8 GPa. Additionally, the 11B solid state NMR spectroscopy study of B2O3 glasses quenched from the melt at five different pressures has been carried out. Taken together, the results obtained provide understanding of the nature of structural transformations in liquid B2O3. The fraction of the boroxol rings in the melt structure rapidly decreases with pressure. From pressures of about 4.5 GPa, four-coordinated boron states begin to emerge sharply, reaching the fraction 40%-45% at 8 GPa. The viscosity of the B2O3 melt along the melting curve drops by 4 orders of magnitude as the pressure increases up to 5.5 GPa and remains unchanged on further pressure increase.

Intradermal injections of polyarginine-containing immunogenic antigens preferentially elicit Tc1 and Th1 activation and antitumour immunity.

Background We previously have shown that nona-arginine protein transduction domain (R9-PTD) induced efficient protein-antigen (Ag) transduction of dendritic cells (DCs) in vitro, resulting in the efficient induction of strong Ag-specific immune responses mediated by CD8+ and CD4+ T cells and in superior antitumour effects in vivo in cancer-bearing mice. Objectives The Ag-specific immune responses caused by intradermal (i.d.) injections of R9-PTD-containing protein Ags without DC preparation were investigated. We also investigated the antitumour effects by intratumoral (i.t.) injections of rR9-containing protein Ags. Methods Synthesized SIINFEKL peptide, or recombinant ovalbumin fusion proteins (rOVA, rR9-OVA), were directly injected into abdominal skin in naïve C57BL/6 mice. OVA-specific cytotoxic T lymphocyte (CTL) activity, serum IgG titre and cytokine profiles were investigated. Histopathological analyses were also performed. In a cancer vaccination model, EG.7 (OVA-cDNA transfectants thymoma) cells were inoculated intradermally in C57BL/6 mice, and the antitumour effects were evaluated by i.t. injections of rR9-OVA in a treatment setting. Results i.d. injections of rR9-OVA into naïve C57BL/6 mice elicited OVA-specific CTLs and produced IgG2-dominant immunoglobulin. The i.d. injections of rR9-OVA also induced inflammatory cell infiltrates containing neutrophils, monocytes and lymphocytes, as well as production of inflammatory cytokines such as interferon (IFN)-gamma, interleukin-2 and IFN-inducible protein 10, with presenting SIINFEKL epitopes on major histocompatibility complex (MHC) class I molecules at the injection area. i.t. injections of rR9-OVA into EG.7 tumour mass significantly suppressed tumour growth, and these effects were completely abrogated by the depletion of CD8+ T cells. These antitumour effects were superior to those elicited by i.t. injections of rR9-OVA-treated DCs. Conclusions i.d. injections of rR9-containing immunogenic Ag without adjuvants simultaneously induce dual immunological effects: the induction of Tc1- and Th1-dominant immune responses, and the induction of inflammatory and CTL-mediated immune responses at the injection area by expressing Ag epitopes on MHC class I molecules as targets. This simple vaccination approach with R9-PTD-containing fusion proteins might be useful as prophylactic immunotherapy for cancer or infectious diseases.

[Surgically treated chest wall schwannoma without entering the pleural space utilizing ultrasonography].

We report a case of surgically treated chest wall schwannoma without entering the pleural space utilizing ultrasonography. A 19-year-old woman was admitted for an abnormal shadow on a routine health checkup. Roentgenologic examination of the chest showed a 2 cm left chest wall mass. Chest computed tomography revealed a heterogeneous mass with obtuse angles characteristic of a pleural-based lesion. Under general anesthesia, the patient was placed in the right decubitous position. Chest wall ultrasonography defined a mass in the intercostal space. A 2.5 cm skin incision was made on the tumor. The tumor arising from the intercostal nerve was surgically removed without entering the pleural space. The pathological examination revealed a benign schwannoma. Postoperative course was uneventful and she discharged 2 days later. Six years later the patient is well without recurrence.

Viscosity behavior spanning four orders of magnitude in As-S melts under high pressure.

Under compression, the As-S liquids are subject to transformations, including polymerization and metallization. We have measured the viscosity of the As-S liquids under high pressures. As a result, large viscosity variations by 4-5 orders of magnitude have been revealed. The viscosity values of the As-S liquids are moderate in the molecular state, very high in the covalent state, and low in the metallic state. Therefore, predicting the viscosity behavior in other melts under pressure is possible, which is of considerable importance for the physics of glass transition, geophysics, and material science.

Pressure-induced phase transitions of AX(2)-type iron pnictides: an ab initio study.

An investigation into the high-pressure behavior of AX(2)-type iron pnictides was conducted using first-principles calculations based on density functional theory within the generalized gradient approximation. Our results demonstrate that a phase transition from the marcasite to the CuAl(2) occurs at 108 GPa for FeP(2), at 92 GPa for FeAs(2), and at 38 GPa for FeSb(2), accompanying a semiconductor-to-metal crossover. A linear relationship between bulk moduli and the inverse specific volume is proposed to be B(0) = 17 498/V(0)-45.9 GPa for the marcasite-type phase and B(0) = 31 798/V(0)-67.5 GPa for the CuAl(2)-type phase. According to the observed structural evolutions, we claim that the regular marcasite transforms to the CuAl(2)-type phase and the anomalous marcasite transforms to the pyrite-type phase at high pressures.

[Multiple calcifying fibrous pseudotumor of the pleura].

A newly recognized distinctive fibrous soft tissue lesion called "calcifying fibrous pseudotumor" (CFPT) was recently described in the soft tissue of the extremities, trunk, scrotum, groin, neck, or axilla. But CFPT orgining from the pleura is rare. A 44-year-old woman was admitted to our hospital for an investigation of a chest radiographic abnormality. The complete resection was performed through mini-thoracotomy utilizing video assisted thoracoscopic surgery. Microscopically, the lesion was mostly composing dense collagenous tissue and scattered calcifications. The postoperative course was uneventful and no recurrence is observed 18 months after operation. We report succsessful surgical treatment for multiple CFPTs.

[Adult Bochdalek hernia with volvulus of the stomach].

We report a rare case of Bochdalek hernia, congenital posterolateral diaphragmatic hernia with volvulus of the stomach, in an adult A 74-year-old man was admitted to our hospital complaining of sudden abdominal pain and vomiting. Roentgenologic examination of the chest showed air above the left diaphragm, and the mediastinum was displaced to the right. Upper gastrointestinal series revealed volvulus of the stomach in which the pylorus was displaced to the left. The surgical repair was done through left thoracotomy with combining laparoscopy and thoracoscopy without surgical complications, 1 year later the patient is asymptomatic.

Ganglioside complexes containing GQ1b as targets in Miller Fisher and Guillain-Barre syndromes.

BACKGROUND: Serum antibodies to GQ1b are associated with Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) with ophthalmoplegia. Antibodies to ganglioside complexes (GSCs) have not yet been examined in a large population of patients with MFS or GBS. This study aimed to determine the clinical significance of antibodies to GSCs in MFS and GBS. METHODS: The study investigated serum anti-GSC antibodies and the clinical features in 64 MFS patients, 53 GBS patients with ophthalmoplegia (GBS-OP(+)) and 53 GBS patients without ophthalmoplegia (GBS-OP(-)). RESULTS: Thirty patients with MFS (47%), 25 with GBS-OP(+) (47%) and none with GBS-OP(-) had antibodies to GSCs containing GQ1b or GT1a. Patients with MFS and GBS-OP(+) were subdivided according to the antibody reactivities; patients with antibodies specific to GQ1b and/or GT1a (without anti-GSCs antibodies) were placed in Group 1, those with antibodies against GSCs with a total of two sialic acids in the terminal residues, such as GQ1b/GM1, were placed in Group 2, and those with antibodies against GSCs with a total of three sialic acids in the terminal residue, such as GQ1b/GD1a, were placed in Group 3. In MFS, sensory disturbances were infrequent in Group 2 compared with the other groups (p<0.0001). Antibodies specific to GQ1b were observed more often in MFS than in GBS-OP(+) (p = 0.0002). CONCLUSIONS: IgG antibodies to GSCs containing GQ1b or GT1a were closely associated with the development of ophthalmoplegia in GBS, as well as MFS. Both GQ1b and clustered epitopes of GSCs containing GQ1b or GT1a may be prime target antigens for MFS and GBS-OP(+).

[Esophago-bronchial fistula confirmed with bronchoscopy utilized an indocyanine green].

The patient was a 54-year-old female with both headache and vomit presented to the emergency room. Endoscopic examination revealed an advanced esophageal cancer located on the middle thoracic esophagus. Histological analysis revealed squamous cell carcinoma. The clinical stage was diagnosed as T4N2M0 and this case was treated by the chemoradiation. She presented progressive moist cough after chemoradiotherapy. Esophagography demonstrated esophago-bronchial fistula (EBF). EBF was not detected by routine broncoscopy. To confirm fistula, we were performed the bronchoscopy which utilized an indocyanine green. Contrast media colored green were over from the superior segmental bronchus in a bronchoscope. The bronchoscope which utilized an indocyanine green is effective for EBF.

Transforming growth factor-beta(2) polymorphisms are associated with childhood atopic asthma.

BACKGROUND: Transforming growth factor (TGF)-beta plays an important role in the regulation of airway inflammation and remodelling in asthma. Recent studies suggest that TGF-beta(2) is a predominant isoform expressed in severe asthma and it is also associated with airway remodelling. OBJECTIVE: To determine whether the polymorphisms in TGF-beta(2) are associated with childhood atopic bronchial asthma in a Japanese population. METHODS: We identified a total of eight polymorphisms and characterized the linkage disequilibrium (LD) mapping of the gene. Three variants in the promoter and 3'UTR were genotyped, and we conducted an association study of TGF-beta(2) (childhood atopic asthma n=297, normal controls n=555). An association analysis of these variants and an expression and functional analysis were performed. RESULTS: 3'UTR 94862T >A was found to be significantly associated with the risk of childhood atopic asthma (P=0.00041). The -109-->ACAA ins promoter variant was also associated with the risk of childhood atopic asthma (P=0.0037). TGF-beta(2) expression was observed in both the normal and asthmatic bronchial epithelium, and both real-time PCR and an ELISA showed a significant basal and TGF-beta(1)-induced TGF-beta(2) expression in the bronchial epithelial cell line BEAS2B. Furthermore, the promoter variant -109-->ACAA ins increased the TGF-beta(2) promoter-reporter activity in BEAS2B cells. CONCLUSIONS: Our data suggest that TGF-beta(2) may therefore be involved in the development of childhood atopic asthma by means of functional genetic polymorphism. The polymorphisms in TGF-beta(2) may become important information for asthma susceptibility in children.

Anti-ganglioside complex antibodies associated with severe disability in GBS.

Ganglioside complexes (GSCs) are known as target antigens in Guillain-Barré syndrome (GBS). To elucidate the clinical importance of the anti-GSC antibodies in GBS, we investigated serum antibodies to GSCs containing two of the gangliosides, GM1, GD1a, GD1b and GT1b, and analyzed clinical features of anti-GSC-positive GBS patients. Thirty-nine (17%) of 234 GBS patients had IgG anti-GSC antibodies. Anti-GSC-positive GBS had antecedent gastrointestinal infection and lower cranial nerve deficits more frequently than control GBS. The presence of antibody specificity to GD1a/GD1b and/or GD1b/GT1b was significantly associated with severe disability and a requirement for mechanical ventilation.

Nonviscous metallic liquid Se.

Viscosity is one of the fundamental physical properties of liquids; for different melts it varies in an extremely wide range. Selenium is among the first elementary substances to have manifested, at compression, a phase transformation in the liquid state accompanied by melt metallization. Direct measurements by means of a real-time radiography show that the viscosity of liquid Se under pressure drops by 500 times to a very low level of 8 mPa s. This is the first case of viscosity measurements being performed both for a relatively viscous semiconducting state and a low-viscous metallic state of the same liquid substance. The viscosity of the Se melt strongly decreases with pressure along the melting curve in a semiconducting state and experiences a further significant drop at melt metallization. A similar phenomenon is expected to be observed in many chalcohenide, halogenide, and oxide melts.

[New forceps for a sutureless patch technique using sheet materials].

Intraoperative air leaks are often treated by a sutureless patch technique using a fibrin tissue-adhesive collagen fleece or synthetic materials with fibrin sealant. Intraoperatively, the technique of using these materials has been troublesome, therefore air leaks fail to close. Akicette has been applied as an easy sutureless patch technique for air leaks. Seven air leaks (5 were lung cancers, 2 were spontaneous pneumothorax) were treated with Akicette. Akicette is simple to operate with, and can easily carry sheet materials. Using Akicette, the sheet materials tightly adhered onto surrounding the pleural defect without any sliding on the wet lung surface. Akicette is feasible tool for a sutureless patch technique and is useful to easily place the sheet materials.

Anti-ganglioside complex antibodies in Miller Fisher syndrome.

BACKGROUND: Some ganglioside complexes (GSCs) are target antigens for serum antibodies in patients with Guillain-Barré syndrome (GBS). Anti-GSC antibodies may be associated with particular clinical features of GBS. OBJECTIVE: To investigate antibodies to GSCs in the sera of patients with Miller Fisher syndrome (MFS) characterised by elevation of the IgG anti-GQ1b antibody. RESULTS: In all, 7 of 12 (58%) consecutive patients with MFS were found to have IgG antibodies to GSCs containing GQ1b, of whom 5 had IgG antibodies to GQ1b-GM1 complex (GQ1b/GM1) and 2 had antibodies to GQ1b/GD1a; 4 of 5 patients without sensory symptoms had anti-GQ1b/GM1 antibodies. CONCLUSIONS: At least three different specificities in MFS-associated antibodies, GQ1b-specific, anti-GQ1b/GM1-positive and anti-GQ1b/GD1a-positive, were observed. In patients with MFS not only GQ1b itself but also clustered epitopes of GSCs, including GQ1b, may be considered to be prime target antigens for serum antibodies. A tendency to escape sensory disturbances is shown by anti-GQ1b/GM1-positive MFS.

Intraspinal sarcoidosis: clinical features, MR imaging and electrophysiological study.

We presented the case of a 58-year-old woman with spinal sarcoidosis at the C4-C6 level. The lesion itself as visualized with magnetic resonance imaging, and its neurological findings responded favorably to treatment with prednisolone. Comparison of pre- and post-treatment values for somatosensory evoked potential (SEP) and central motor conduction time (CMCT) also showed improvement. Analysis of SEP and CMCT can be a useful tool for following up spinal sarcoidosis.

[Stenting in obstruction of superior vena cava; clinical experience with the self-expanding endovascular prosthesis].

From August 1997 to December 2002, 14 consecutive patients with superior vena cava syndrome with the self-expanding endovascular prosthesis. Diagnoses were adenocarcinoma in 6, small cell carcinoma in 4, squamous cell carcinoma in 1, metastatic lung cancer in 2, and invasive thymoma in 1. Atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) were measured on their admission and perioperative period. Expecting only 1 patient complete symptomatically relieved within 3 days of stent implantation. Superior vena cava pressure or radial pressure of the stent was sufficient to relieve obstruction. Preoperative ANP level were normal, BNP level were increased. Postoperatively both ANP level and BNP level were slightly increased under intravenous dopamine hydrochloride. Implantation of the self-expanding stent endovascular prosthesis for superior vena cava syndrome provides rapid symptomatic relief and improves the patient's quality of life.