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Clinically amyopathic dermatomyositis (CADM) patients frequently develop refractory interstitial lung disease (ILD), with a poor prognosis. We aimed to verify the efficacy and safety of plasma exchange (PE) treatment for ILD in CADM. A retrospective case–control study was conducted to compare clinical outcomes with and without PE treatment in CADM-ILD patients refractory to combination therapy of high-dose glucocorticoids, calcineurin inhibitors, and cyclophosphamide. Among 19 enrolled patients, 11 were further treated with PE. We compared survival rates and other clinical characteristics. PE consisted of either fresh-frozen plasma or albumin as a replacement solution. Basal clinical characteristics at diagnosis, including age, gender, serum ferritin, Krebs von den Lungen-6 (KL-6), C-reactive protein, and respiratory function tests, did not differ between the two groups. The survival rate for treatment with PE was higher than for treatment without PE (91% and 50%, respectively, p < 0.05). Among PE-treated patients, anti-melanoma differentiation-associated gene-5 (anti-MDA-5) antibody titre, ferritin, and KL-6 as serological activity markers were sustainably reduced only after initiating PE. Therapeutic intervention with PE reduced the frequency of exacerbation of ILD requiring methylprednisolone pulse therapy. The occurrence of bacterial, fungal, and cytomegalovirus infection did not differ between the groups with and without PE, and adverse events associated with PE resolved with appropriate intervention. Combination therapy with PE was associated with an improved survival rate, and may be effective for the management of refractory ILD in CADM patients. A personalized therapeutic strategy including PE could be introduced for fatal rapidly progressive ILD. [ABSTRACT FROM AUTHOR]

The I Treponema pallidum i hemagglutinin quantification test performed before biopsy revealed high antibody titres (1000 or more) and rapid plasma reagin (RPR) indicated 200.0 RPR units (R.U). The patient in this manuscript has given written informed consent to the publication of his case details. As a result, high level of clinical suspicion should be maintained in order to be able to diagnosis syphilis at any stage.2 This case also demonstrates the importance of proactively performing a biopsy and routine syphilis serum test at prebiopsy. [Extracted from the article]

Summary: Prostaglandin D2 (PGD2) plays an important role in atopic dermatitis (AD), and 11,15‐dioxo‐9α‐hydroxy‐2,3,4,5‐tetranorprostan‐1,20‐dioicacid (PGDM) is a major metabolite of PGD2. We investigated the relationship between urinary PGDM levels and severity of paediatric AD. In total, 31 patients with AD and 21 healthy controls (HCs) without AD were recruited, and urinary PGDM levels were measured. Of the 31 patients with AD, 14 were reassessed for urinary PGDM after topical steroid therapy. There was no difference in urinary PGDM levels between patients with AD and HCs. Although there was a significant positive correlation between the SCORing Atopic Dermatitis (SCORAD) index and the serum level of thymus and activation‐regulated chemokine (TARC), the urinary PGDM levels did not correlate with either SCORAD or serum TARC. Moreover, both SCORAD and serum TARC were significantly improved by topical steroid therapy; however, urinary PGDM levels were not changed. In conclusion, the level of urinary PGD2 metabolites in children with AD is substantially the same as that in HCs even if the disease is severe. [ABSTRACT FROM AUTHOR]

Werner syndrome (WS) is an autosomal recessive disorder characterized by physical signs and symptoms, including premature aging and scleroderma-like skin changes. The gene responsible for WS is the WRN gene. A significant proportion of WS-related malignant tumours are non-epithelial types, and the incidence of oral squamous cell carcinoma (SCC) is rare. A case of oral SCC of the lower alveolus and gingiva arising in a 63-year-old woman with WS is reported here. Biopsy confirmed moderately differentiated SCC. Surgical resection was performed and there was no recurrence or metastasis at the 3-year follow-up. Mutation analysis using next-generation sequencing, detected no mutations in the genes encoding the molecules strongly involved in the development of oral SCC, such as TP53 or PIK3CA. No obvious mutations were detected. Based on the results of the study, the results of mutation analysis suggest that this case might be genetically different from the common mechanisms of SCC in the oral cavity. [ABSTRACT FROM AUTHOR]

Introduction: Social withdrawal, or hikikomori, is one of Japan's most serious psychosocial issues. The concept gained international attention around 2010 and widespread psychiatric epidemiological studies have since been conducted. Objectives: With an understanding of the extensive range of hikikomori circumstances as a spectrum, we aimed to quantitatively measure the severity of hikikomori in adolescent subjects, an age group considered particularly susceptible to the condition, and to identify factors associated with its severity. Methods:We selected population demographics, socioeconomic data, and psycho-behavioral characteristics as factors related to hikikomori and explored their associations with hikikomori severity using crosssectional analysis. Subjects were a patient group of middle school students examined as outpatients at a psychiatric clinic during adolescence for a chief complaint of hikikomori and a control group of middle school students matched for sex and age. Subjects' parents completed a questionnaire pertaining to their child's hikikomori symptoms and living environment along with the Child Behavior Checklist (CBCL). The data collected was then statistically analyzed. Results: T-test results demonstrated that scores for all CBCL syndrome scales were significantly higher in the patient group, but no scores fell within the clinical range. Multiple regression analysis revealed that being anxious/depressed, somatic complaints, lack of communication between parents, and overuse of the Internet were statistical predictors of hikikomori severity. Conclusions: It may be possible to prevent hikikomori from becoming severe if the above predictors are used to identify highrisk individuals requiring active intervention while hikikomori is at an early stage. [ABSTRACT FROM AUTHOR]

Ligand-gated chloride channels (LGICs) are important targets for insecticides and parasiticides. Genes encoding subunits of two LGICs, a glutamate-gated chloride channel (MdGluCl-α) and a γ-aminobutyric acid (GABA)-gated chloride channel (MdRdl), were cloned from house-flies ( Musca domestica L.). These genes were first expressed independently in Xenopus laevis oocytes by cRNA injection in order to investigate the pharmacology of these ligand-gated channels using two-electrode voltage-clamp electrophysiology. It was found thatl-glutamate and GABA activated the MdGluCl-α homo-oligomers with an EC50 value of 30 µm and the MdRdl homo-oligomers with an EC50 value of 101 µm, respectively. Both channels were chloride ion-permeable, and the MdRdl channel was more sensitive to chloride channel blockers, such as γ-hexachlorocyclohexane (γ-HCH), fipronil and picrotoxinin, than the MdGluCl-α channel. MdGluCl-α required only 1–2 days of incubation after cRNA injection to be expressed in oocytes, whereas 4–7 days of incubation was necessary to achieve MdRdl expression. However, when the cRNA of MdGluCl-α was injected at a dose of 1% (w/w) 1 day after the injection of the cRNA of MdRdl, a significant increase in the current amplitude of responses to GABA was observed, and the incubation period necessary for MdRdl expression became shorter. These results suggest that MdGluCl-α assists in the expression of MdRdl when the two are coexpressed. [ABSTRACT FROM AUTHOR]

Silicone gel sheet treatment is widely used to treat hypertrophic scars and keloids since it is easily applied and prevents scar pain and itching. We used Cica-Care ® silicone gel sheets in the conservative treatment of six patients for 24 weeks and recorded pain, itching, redness, and scar elevation every 4 weeks. We also investigated the number of mast cells and Fas antigen expression in the lesional skin (one patient) before and after treatment. The pain and itching clearly decreased after 4 weeks of the silicone gel sheeting and disappeared after 12 weeks. Twelve weeks were required for a reduction in scar redness and elevation. After 24 weeks, a decrease in the number of mast cells and the enhanced expression of Fas antigen by lesional fibroblasts were observed. Thus, silicone gel sheeting is effective and safe, especially with more severe symptoms of pain and itching possibly induced by mediators derived from increased mast cells. [ABSTRACT FROM AUTHOR]

Acute ovalbumin-induced arthritis (OIA), which is mediated by Arthus reaction to ovalbumin (OVA) in the joint space, can be induced by immunization of rats with OVA followed by the intraarticular injection of OVA. Because oral administration of antigen induces immunological unresponsiveness, we studied for the first time the effects of oral administration of OVA on acute OIA. The oral administration of OVA before immunization significantly suppressed the development of acute OIA, in accordance with decreases in both the anti-OVA IgG antibody production and in vitro lymphocyte proliferative responses to OVA. However, the oral administration of OVA after immunization did not show any decrease in antibody production or in vitro lymphocyte proliferation to OVA, or in the severity of acute OIA. These results indicate that the induction of oral tolerance to OVA in OIA is possible by oral administration of OVA before, but not after, immunization with the antigen. This supports the concept of using antigen feeding as a treatment for certain humoral immune-mediated diseases. [ABSTRACT FROM AUTHOR]

Kawasaki disease is an inflammatory disease of unknown cause that causes panvasculitis, including coronary arteritis. Polymorphonucleocytosis in the early stage of the illness suggests the implication of neutrophils in the pathogenesis of the disease. In the acute phase of Kawasaki disease, mRNA expression of prostaglandin H2 synthase (PHS)-2, as determined by reverse transcription-polymerase chain reaction, was markedly enhanced, and thromboxane A2 (TXA2)-synthesizing activity was increased in polymorphonuclear leukocytes (PMNL). This up-regulation of PHS-2 was suppressed by ulinastatin (a neutrophil-elastase inhibitor) treatment. Lipopolysaccharide-induced enhancement of PHS-2 mRNA was also inhibited by therapeutic doses of ulinastatin in vitro by use of PMNL from healthy volunteers. Thus, ulinastatin inhibits arachidonate PHS metabolism by inhibiting new induction of PHS-2 at the mRNA level, which is a novel pharmacologic action of this substance. Ulinastatin treatment is possibly an additional therapeutic approach to Kawasaki disease. [ABSTRACT FROM AUTHOR]