Successful use of tofacitinib in epidermolysis bullosa pruriginosa.

This case report describes 26-year-old woman who had multiple clusters of pale-pink lichenoid papules since childhood and the accompanying itching was intense. Skin biopsy revealed obvious fissures had formed under the epidermis. The patient was diagnosed with epidermolysis bullosa pruriginosa and was successfully treated with tofacitinib.

Circular RNA circ_0000034 upregulates STX17 level to promote human retinoblastoma development via inhibiting miR-361-3p.

OBJECTIVE: Retinoblastoma (RB) is a common intraocular tumor of infancy and childhood. Circular RNAs (circRNAs) are related to the development of RB. The purpose of this research was to reveal the functional mechanism of circRNA circ_0000034 in RB. MATERIALS AND METHODS: Quantitative Real-Time Polymerase Chain Reaction (qRT-PCR) and Western blot were applied to determine the levels of genes. MTT assay and flow cytometry were employed to assess cell proliferation and apoptosis rate, respectively. Furthermore, cell migratory and invasive abilities were measured using the transwell assay. Mouse xenograft was conducted to analyze the effect of circ_0000034 on tumor growth in vivo. Besides, the interaction between miR-361-3p and circ_0000034 or syntaxin 17 (STX17) was predicted by starBase, and then, confirmed by the Dual-Luciferase reporter assay and RNA immunoprecipitation (RIP) assay. RESULTS: The levels of circ_0000034 and STX17 were increased and miR-361-3p level was decreased in RB tissues and cells. Circ_0000034 knockdown suppressed cell proliferation, migration, invasion, autophagy, and tumor growth, and induced apoptosis in RB. Circ_0000034 targeted miR-361-3p and miR-361-3p bound to STX17. Circ_0000034 overexpression and miR-361-3p knockdown reversed the effect of miR-361-3p upregulation and STX17 depletion on the growth of RB cells, respectively. Besides, circ_0000034 elevated STX17 level by repressing miR-361-3p expression. CONCLUSIONS: We demonstrated that circ_0000034 knockdown suppressed the development of RB by the modulation of miR-361-3p/STX17 axis. Our findings provided a theoretical basis for the treatment of RB.

Factors related to skeletal relapse in the two-jaw surgery treatment of mandibular prognathism.

This study identified factors contributing to skeletal relapse in the two-jaw surgery treatment of mandibular prognathism. A set of three standardized lateral cephalograms (T1: before surgery, T2: immediately after surgery, T3: final follow-up after surgery) were obtained from 35 patients. The surgical changes were defined as follows: postsurgical immediate change (T2-T1), postoperative stability (T3-T2) and the final surgical change (T3-T1). The occlusal plane and gonial angles were also measured. Relapse was defined as the reverse movements of the menton point (Me) and point A, with the null hypothesis stating that Me and point A do not significantly change at the postoperative stability (T3-T2). A paired t test and Pearson's correlation were used for statistical analysis. The immediate postoperative changes (T2-T1) in Me and point A were significant, and were measured to be 8.5mm backward and 3.0mm forward, respectively. Additionally, the occlusal plane and gonial angles significantly increased by 2° and decreased by 2°, respectively. The final postoperative changes (T3-T1) in Me and point A were also significant, and were measured to be 5.2mm backward and 2.5 forward, respectively; the occlusal plane and gonial angles also increased nonsignificantly by 0.6° and 0.7°, respectively. Upon investigating postoperative stability (T3-T2), Me was measured to be significantly 3.3mm forward and 1.4mm upward, whereas point A was measured to be nonsignificantly 0.5mm backward and 0.9mm upward. Therefore, the null hypothesis was rejected. Pearson's correlation showed that horizontal Me (T3-T2) and point A (T3-T2) were significantly correlated with the amounts of setback Me (T2-T1) and advancement A (T2-T1), respectively. In conclusion, skeletal relapses are significantly correlated with the amounts of mandibular setback and maxillary advancement.

The impact of hepatitis B virus infection and vaccination on the development of non-Hodgkin lymphoma.

Hepatitis B virus (HBV) infection has been documented as a risk factor for non-Hodgkin lymphoma (NHL). However, there are few large cohort studies, and there is no report about the impact of HBV vaccination. We conducted this study to evaluate these issues. We used the nationwide cohort of the Taiwan National Health Insurance Research Database for 1997-2013. We compared the incidence and the risk of developing NHL and CD20+ aggressive lymphoma between HBV and non-HBV cohorts. The hazard ratios (HRs) were computed using Cox proportional hazards models. We matched these two large cohorts to reconfirm the data. We also compared the incidence of NHL between cohorts born before and after the inception of universal HBV vaccination. We found that HBV infection increased the risk for developing NHL and CD20+ aggressive lymphoma, with HRs of 4.14 and 5.52, with a higher incidence of 17.07 and 13.9 per 100 000 person-years, respectively, compared to the non-HBV cohort. The incidence of NHL in the cohort born in the era before universal HBV vaccination was higher with 1.85 per 100 000 person-years compared to 0.74 in the cohort born later aged younger than 20. Our study confirms that HBV confers a greater risk for developing NHL, especially CD20+ aggressive lymphoma. The impact of HBV vaccination is protective against lymphoma development in the teenagers in an endemic area, but longer follow-up is needed for older age.

Multimodal imaging including spectral-domain optical coherence tomography and confocal near-infrared reflectance for characterization of lacquer cracks in highly myopic eyes.

PURPOSE: To compare multimodal imaging in detecting lacquer cracks in highly myopic eyes, and to correlate these findings with those of spectral-domain optical coherence tomography (SD-OCT). METHODS: An observational case series study. Patients with a refractive error worse than -8 diopters and lacquer cracks were recruited. The rates of detection of the lacquer cracks using multimodal imaging including near-infrared reflectance (NIR) imaging, fundus autofluorescence (FAF) imaging, and fluorescence angiography (FA) were compared. The characteristic findings of multimodal imaging were correlated with those of SD-OCT. RESULTS: NIR imaging was more sensitive (92.9%) in detecting lacquer cracks than either FAF (12.5%) or FA (67.9%). Lacquer cracks showed hyperreflectance on NIR, and they were consistently associated with a continuous retinal pigment epithelium-Bruch's membrane complex, thinner choroid, and acoustic shadows on SD-OCT. CONCLUSIONS: NIR imaging is superior to blue laser light (FAF and FA) imaging in detecting lacquer cracks. SD-OCT in combination with NIR located primary pathological lacquer cracks in the intact retinal pigment epithelium-Bruch's membrane complex as well as thinner choroid. These findings indicate that multimodal cSLO and SD-OCT imaging allow for detecting of lacquer cracks in highly myopic eyes.

Refractive errors after the use of bevacizumab for the treatment of retinopathy of prematurity: 2-year outcomes.

PURPOSE: To evaluate the refractive outcomes in children treated after intravitreal injection of bevacizumab (IVB) for retinopathy of prematurity (ROP). METHODS: A retrospective, bi-centre study of 34 patients (64 eyes) was conducted. The patients were divided into three groups, patients received intravitreal IVB (IVB group), patients received combined IVB and laser treatment (IVB + Laser group), or patients received lens-sparing vitrectomy (IVB + LSV group). Cycloplegic refraction and axial length (AXL) were evaluated at 2 years old. RESULTS: The prevalences of myopia and high myopia were 47.5 and 10.0% in the IVB group, respectively, which were lower than those in the IVB + Laser (82.4 and 29.4%) and IVB + LSV (all 100%) groups (P = 0.001 and P < 0.001). The prevalences of emmetropia in the IVB group, IVB + Laser group, and IVB + LSV group were 50, 5.9, and 0% (P = 0.001). The AXL were similar among all groups. CONCLUSIONS: At the 2-year follow-up, severe ROP patients treated with IVB alone were more likely to remain emmetropic and had lower prevalences of myopia and high myopia. The development of high myopia in severe ROP patients could not be explained by AXL changes but may be associated with abnormalities in the anterior segment.

Association of IGF1R polymorphisms with the development of HBV-related hepatocellular carcinoma.

Although the involvement of insulin-like signaling in cancer has been well documented in various types of cancers, the association between the genetic variants in the insulin-like signaling and the development of hepatitis B virus (HBV)-related hepatocellular carcinoma (HCC) remains unclear. In this study, a total of 498 individuals including 173 HBV related cirrhosis patients, 171 HBV-related HCC patients, and 154 healthy controls were enrolled. Sixteen single nucleotide polymorphisms (SNPs) in IGF1, IGF2, IGF1R and IGF2R have been genotyped by employing SNaPshot assays. We found A/A genotype at rs3743251 of IGF1R was negatively associated with HBV related HCC [odds ratio (OR) = 0.38, 95% confidence interval (CI) = 0.20-0.72, P = 0.037]; A/G genotype decreased the risk of portal vein thrombosis (OR = 0.38, 95%CI = 0.18-0.82, P = 0.01). These results indicate that rs3743251 polymorphism in IGF1R is associated with the susceptibility of HBV-related HCC.

Clinical characteristics of optic neuritis in Taiwanese children.

PURPOSE: To document the etiology, clinical presentation, and visual prognosis of optic neuritis in Taiwanese children. METHODS: Retrospectively reviewed children younger than 18 years old with optic neuritis in Chang Gung Memorial Hospital and Chang Gung Children's Hospital from 1998 to 2009. RESULTS: There were 24 children (38 eyes) with optic neuritis in that period. Overall, 14 patients (58.3%) were female and 10 patients (41.7%) were male. In total, 14 patients (58.3%) had bilateral involvement, and 10 patients (41.7%) had unilateral involvement. Out of 38 eyes, 24 (63.2%) had disc swelling. Out of 24 patients, 21 (87.5%) underwent intravenous steroid therapy (10 to 30 mg/kg/day) for 3-5 days, and followed by an oral taper. Out of 24 patients, 20 (83.3%) achieved final visual acuity (VA) of 20/40 or better. However, a poor visual outcome (four patients) (VA<20/40) was correlated with pale disc at presentation (P=0.002, Pearson χ (2)-test) and age older than 10 years (P=0.012, Fisher's exact test). Five patients were diagnosed with acute disseminated encephalomyelitis (ADEM) (21%), and three patients were diagnosed with multiple sclerosis (MS) (12.5%). Patients with ADEM did not have a better visual outcome than patients with MS (P=0.643, Fisher's exact test). CONCLUSIONS: Visual recovery from optic neuritis was favorable in Taiwanese children. A poor visual outcome was correlated with pale disc at presentation and patients' age older than 10 years. ADEM is the most common associated systemic disease; MS is relatively rare.

Association between folate status, diabetes, antihypertensive medication and age-related cataracts in elderly Taiwanese.

OBJECTIVE: To evaluate possible risk factors for cataract in elderly Taiwanese, and to investigate whether the relationship between age and cataract in older persons is modified by other cataract-associated risk factors. PARTICIPANTS: A cross-sectional study of 661 males and 645 females aged ≥ 65 yrs was conducted as part of the Elderly Nutrition and Health Survey in Taiwan (1999-2000) (Elderly NAHSIT). METHODS: Self-reported cataracts were defined as any incidence of cataract that was diagnosed by a physician and treated by anticataractic drugs based on the medical history section of the Elderly NAHSIT. Potential risk factors for cataract were determined by multiple logistic regression analysis of data obtained from the health examination, blood biochemistry and interviewer-administered questionnaires. RESULTS: Results showed that the prevalence of self-reported cataract increased with age and was significantly higher in older women than in older men. Cataracts were associated with age, diabetes, antihypertensive medication and folate insufficiency in older men, and with age and antihypertensive medication in older women. Folate insufficiency remained associated with cataract in older men who had adequate vitamin B2, B6 and B12 status. Folate insufficiency was associated with cataract after adjustment for other risk factors in older men aged ≥ 75 yrs, while in older men aged 65-74 yrs, only diabetes and antihypertensive medication remained associated with cataract. In addition, age ≥ 75 yrs remained a risk factor for cataract in those without diabetes, not taking hypertensive medication and with normal folate status. Further analysis showed that the strength of the association between age ≥ 75 yrs in older men and cataracts was increased about 1.5-fold when combined with folate insufficiency (interaction p= 0.0198), and increased about 1.8-fold when combined with use of antihypertensive medication (interaction p = 0.0214). CONCLUSION: Our results suggest that the combination of age ≥ 75 yrs in older men with either folate insufficiency or use of antihypertensive medication had an additive effect on the risk of cataract. Maintenance of good folate status should be emphasized to reduce the risk of cataract in the Taiwanese elderly, especially men.

Intravitreal triamcinolone acetonide vs bevacizumab for treatment of macular oedema secondary to branch retinal vein occlusion.

PURPOSE: To compare the short-term visual and morphological results of intravitreal triamcinolone acetonide vsintravitreal bevacizumab for eyes with macular oedema secondary to branch retinal vein occlusion (BRVO). DESIGN: Retrospective interventional consecutive case series. METHODS: We reviewed the clinical records of 29 patients (29 eyes) who had macular oedema due to BRVO with minimum follow-up of 6 months. A total of 16 patients were treated with intravitreal injection of 4 mg/0.1ml triamcinolone acetonide. The other 13 patients received intravitreal bevacizumab of 1.25 mg in 0.05 ml. Baseline visual acuity, macular thickness, and intraocular pressure were recorded. Final visual acuity, final macular thickness, intraocular pressure, and adverse events were also recorded throughout the follow-up. RESULTS: All patients completed at least 6 months of follow-up. There were significant improvement in visual acuity and showed significant macular oedema decrease in optical coherence tomography examination in both the two groups postoperatively. However the therapeutic effects showed no statistically significant difference between these two groups with regard to visual results (F=6.012, P=0.083) and macular thickness decline (F=0.007, P=0.570). Seven eyes developed recurrent macular oedema and received reinjections of triamcinolone acetonide or bevacizumab. CONCLUSION: These short-term results indicate that intravitreal injection of triamcinolone acetonide or bevacizumab can both improve visual acuity and decrease macular oedema temporarily in eyes with BRVO. However, the therapeutic effects of intravitreal triamcinolone acetonide showed no significant differences compared with intravitreal bevacizumab with regard to anatomical and functional outcomes but seemed to cause more adverse events than bevacizumab.

Paediatric retinal detachment: comparison of high myopia and extreme myopia.

AIMS: To compare the clinical features and surgical outcomes of paediatric retinal detachment (RD) in high myopia and extreme myopia. METHODS: The clinical charts of 107 children who experienced RD and had a spherical equivalent (SE) of at least 6.00 dioptres (D) were reviewed. The patients were separated into a high myopia group (SE -6.0 to -10.0 D) and extreme myopia group (SE >-10.0 D). RD characteristics and outcomes were compared between these two groups. RESULTS: There were significant differences between the two groups in total RD (p<0.001), the presence of posterior staphyloma (p<0.001) and some types of breaks. More eyes in the extreme myopia group required vitrectomy after the initial RD repair. In the high myopia group, retinal reattachment was achieved in 79 eyes (97.5%) at the end of the intervention, whereas in the extreme myopia group, retinal reattachment was achieved in 22 eyes (73.3%). Multiple logistic regression showed that a higher refractive error was the only negative predictor of surgical outcome (p = 0.026). CONCLUSION: Due to differences in aetiologies, clinical characteristics, required surgical procedure after initial repair, surgical and functional outcomes, paediatric RD with extreme myopia should be addressed differently from paediatric RD with high myopia.

Effects of a calcineurin inhibitor, tacrolimus, on glutamate-dependent potentiation in pelvic-urethral reflex in anesthetized rats.

Effects of tacrolimus, a protein phosphatase 2B inhibitor, on the reflex plasticity between the pelvic afferent nerve fibers and the urethra were examined in urethane-anesthetized rats. Repetitive stimulation (1 Hz) induced a potentiation (0.9+/-0.2 and 10.5+/-1.6 spikes in control and repetitive stimulation groups, respectively, P<0.01, N=10) in the activities of the pelvic-urethral reflex. Intrathecal tacrolimus (0.1 mM, 10 microl, bolus) blocked repetitive stimulation-induced potentiation in pelvic-urethral reflex activities (3.2+/-0.9 spikes in tacrolimus group versus 10.5+/-1.6 spikes in repetitive stimulation group, P<0.01, N=10). Glutamate (intrathecal, 0.1 mM, 10 microl, bolus) and N-methyl-D-aspartic acid (intrathecal, 0.1 mM, 10 microl, bolus) both reversed the blocking effects exerted by tacrolimus on repetitive stimulation-induced pelvic-urethral reflex potentiation (15.0+/-1.4 spikes in glutamate group and 11.4+/-1.4 spikes in N-methyl-D-aspartic acid group versus 3.2+/-0.9 spikes in tacrolimus-treated repetitive stimulation group, P<0.01, N=7). In addition, the reversal effect elicited by these two agonists of glutamate receptors showed no statistical difference (P=NS, N=7). All these results demonstrated that tacrolimus could block glutamatergic N-methyl-D-aspartic acid receptor-mediated potentiation in pelvic-urethral reflex activities. This finding may be pathologically relevant in patients who take tacrolimus as immunosuppressant therapy. Whether tacrolimus will induce urine incontinence in such patients or not needs further investigation.