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Epidermolysis bullosa is a term for a heterogeneous group of rare disorders, characterized by extensive blistering of the skin and mucous membranes as the most prominent features. So far association of the disease with extra-cutaneous manifestations and complications has been pointed out. We present an unusual case of a neonate with severe complications of epidermolysis bullosa, associated with bilateral clubfoot. [ABSTRACT FROM AUTHOR]

Orofacial granulomatosis is a condition that manifests clinically as painless labial enlargement, perioral and mucosal edema, oral ulcers, and gingivitis. It is characterized by non-necrotizing granulomatous inflammation of the oral and maxillofacial region. When the swelling only affects the lips, the pathology is called Miescher's granulomatous cheilitis; however, when it also causes facial paresis and lingua plicata, it is known as Melkersson-Rosenthal syndrome. We report a case that was successfully treated with a combination of a local (intralesional) steroid, a systemic antibiotic, and a systemic steroid. After 6 months of therapy, we observed improvement in gingival hyperplasia and buccal mucosa and lip edema., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2023.)

Healthcare service quality and hospitalized patient satisfaction in Albania have never truly received the necessary attention. The aim of this study is the assessment of key elements of service quality and their relationship with patients' satisfaction with receiving healthcare at public health institutions in Albania. The study examines five key dimensions of quality such as responsiveness, reliability, assurance, tangibles, and staff empathy to assess properly the healthcare quality and patient satisfaction in Albania based on the SERVQUAL instrument, which measures the differences between patient's perceptions of service quality received, and their expectations in terms of these five dimensions. A total of 800 randomly selected patients were interviewed in the main cities of Albania in public hospitals. A respective literature review was also a necessity to determine not only the appropriate methodology to be applied, together with the right quality dimensions to use but also for a better understanding of the link between service quality and patient satisfaction. The determination of the main quality factors which contribute to patient satisfaction, as well as, their order of importance, is another key aspect of the study, which finalizes with appropriate recommendations that might help quality improvement in future. The analysis shows that overall patient satisfaction is affected in public hospitals by all dimensions of service quality. Therefore, patients' perspective is extremely important in assessing healthcare service quality and should be taken into consideration by healthcare managers and policymakers in Albania, whenever significant reforms will be undertaken to improve the quality of services in this sector., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Kalaja and Krasniqi.)

Objective: Identifying components of immuneparesis, a hallmark of chronic liver failure, is crucial for our understanding of complications in cirrhosis. Various suppressor CD4 + T cells have been established as potent inhibitors of systemic immune activation. Here, we establish the presence, regulation and mechanism of action of a suppressive CD4 + T cell subset expressing human leucocyte antigen G (HLA-G) in patients with acute decompensation of cirrhosis (AD)., Design: Flow cytometry was used to determine the proportion and immunophenotype of CD4 + HLA-G + T cells from peripheral blood of 20 healthy controls (HCs) and 98 patients with cirrhosis (28 with stable cirrhosis (SC), 20 with chronic decompensated cirrhosis (CD) and 50 with AD). Transcriptional and functional signatures of cell-sorted CD4 + HLA-G + cells were delineated by NanoString technology and suppression assays, respectively. The role of immunosuppressive cytokine interleukin (IL)-35 in inducing this population was investigated through in vitro blockade experiments. Immunohistochemistry (IHC) and cultures of primary human Kupffer cells (KCs) were performed to assess cellular sources of IL-35. HLA-G-mediated T cell suppression was explored using neutralising antibodies targeting co-inhibitory pathways., Results: Patients with AD were distinguished by an expansion of a CD4 + HLA-G + CTLA-4 + IL-35 + immunosuppressive population associated with disease severity, clinical course of AD, infectious complications and poor outcome. Transcriptomic analyses excluded the possibility that these were thymic-derived regulatory T cells. IHC analyses and in vitro cultures demonstrate that KCs represent a potent source of IL-35 which can induce the observed HLA-G + phenotype. These exert cytotoxic T lymphocyte antigen-4-mediated impaired responses in T cells paralleled by an HLA-G-driven downregulation of T helper 17-related cytokines., Conclusion: We have identified a cytokine-driven peripherally derived suppressive population that may contribute to immuneparesis in AD., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY. Published by BMJ.)

This study compared pedestrian behaviors in five countries (Estonia, Greece, Kosovo, Russia, and Turkey) and investigated the relationships between these behaviors and values in each country. The study participants were 131 pedestrians for Estonia, 249 for Greece, 112 for Kosovo, 176 for Russia, and 145 for Turkey. The principal component analyses revealed that the four-factor structure of the Pedestrian Behavior Scale (PBS) was highly consistent across the five countries. ANCOVA results revealed significant differences between countries on the PBS items and scale scores. Specifically, Greek and Turkish participants reported transgressive pedestrian behaviors more frequently than Estonian, Kosovar, and Russian pedestrians while Kosovar participants reported transgressive pedestrian behaviors less frequently than Estonian pedestrians. In addition, Turkish and Russian pedestrians reported lapses and aggressive behaviors more frequently than Estonian, Greek, and Kosovar pedestrians. Finally, Turkish and Estonian pedestrians reported positive behaviors more frequently than Kosovar pedestrians. Unexpectedly, the regression analyses showed that values have varying effects on pedestrian behavior in the five countries. That is, context or country may determine the effect of values on pedestrian behaviors. The results are discussed in relation to the previous literature., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Purpose: To determine the 5-year success rate for Baerveldt glaucoma implant (BGI) in patients who received the implant as a primary (primary BGI, used as the initial surgical procedure) or secondary (secondary BGI, used after trabeculectomy) by a single surgeon between year 1994 and 2010., Patients and Methods: A total of 117 eyes from patients who underwent BGI placement as a primary or secondary procedure were included in this study. Demographics, previous history, type of glaucoma, intraocular pressure (IOP), visual acuity (VA), glaucoma medication use, and early and late postoperative complications were collected. Postoperative data were collected at day 1, 30, 60, and every year until the last visit (minimum of 2 y). IOP was the main outcome measure. Secondary outcome measures included VA, glaucoma medication use, and early and late postoperative complications. Overall success rates were calculated for primary and secondary BGI using Kaplan-Meier survival analysis. Differences between survival curves were determined using log-rank test. Risk factors for success were defined by Cox proportional-hazards regression model., Results: At the 5-year follow-up, the overall success rates (determined as IOP between 6 and 21 mm Hg) in the primary and secondary BGI were 58% (49/79) and 53% (20/38), respectively (P=0.56). The overall success rate dropped by an average 10% and 13% per year for the primary and secondary BGI groups, respectively (P=0.05). The complete success rates at the 5-year follow-up for primary and secondary BGI were 24% (19/79) and 13% (5/38), respectively, whereas the qualified success rates were 34% (27/79) and 39% (15/38), respectively. There was a significant decrease in IOP [from 31.23 (±9.51) to 12.86 (±4.84) mm Hg (P<0.001) for the primary BGI group, and from 26.35 (±7.22) to 13.92 (±5.90) mm Hg (P<0.001) for the secondary BGI group]. There was also a significant drop in medication use but a significant worsening in VA in both groups (P<0.05) likely from cataract. The difference in the incidence of postoperative complications was not statistically significant between the groups., Conclusions: Although, the success rates were similar for the primary and secondary BGI at the 5-year follow-up, the drop in the success rate per year was significantly higher in the secondary BGI group. In contrast, both procedures had similar incidence of postoperative complications.

Background: Relationships between in-vitro fertilization (IVF), gestational hypertension, and pregnancy outcomes are demonstrated in a number of studies. However, it is still debated if IVF treatment or specific characteristics of infertile patients are responsible for worse obstetrical and neonatal outcomes., Objective: The aim of this study was to investigate maternal characteristics associated with hypertensive disorders (HD) in IVF conceived pregnancies and to assess the obstetrical and neonatal outcomes., Materials and Methods: In this observational, cross-sectional study, 207 pregnant women who underwent IVF treatment were consecutively divided into two groups: a group that had no HD during pregnancy (IVF group) and a group that had HD during pregnancy (IVF+HD group). Maternal, obstetrical and neonatal data of the two groups were compared., Results: Some maternal characteristics were significantly higher in IVF+HD compared to IVF group such as: older age (p=0.0001), primiparity (p=0.038), obesity (p=0.0001), and cigarette smoking (p=0.0001). There were no significant differences between the groups in regard to obstetrical outcomes besides premature rupture of membranes time that was significantly higher in IVF+HD group compared to IVF group (p=0.036). In regard to neonatal outcomes, the only statistically significant difference was in the 5 th min Apgar score, which was higher in IVF+HD group compared to IVF group without HD (p=0.002)., Conclusion: With regard to significantly higher differences in maternal characteristics of IVF conceived pregnancies complicated with HD, compared to uncomplicated ones, development of a specific national prevention measure for HD of IVF conceived pregnancies in Kosovo is strongly suggested. In addition, setting up of a national registry is recommended in order to evaluate the outcomes of IVF treatments properly., Competing Interests: All contributing authors declare no conflict of interest.

Purpose: To investigate the lymphatic vascular microvessel density (LVD) and the blood vascular microvessel density (MVD) and their distribution in excised leaking blebs after mitomycin C trabeculectomy and normal conjunctiva specimens., Materials and Methods: LVD and MVD in normal human conjunctiva (n=8) and excised blebs in the hypocellular stroma and peribleb tissue (conjunctiva adjacent to hypocellular bleb tissue) (n=8) were evaluated by immunohistochemistry using antibodies raised against Lymphatic Vessel Endothelial Receptor 1 (D2-40, lymphatic endothelium) and CD34 (vascular endothelium). LVD and MVD counts were performed by light microscopy in 5 fields at ×20 magnification by 3 observers. Differences were determined using Mann-Whitney U test (P<0.05 was considered significant)., Results: The leaking blebs showed typical epithelial-stromal domes with areas of acellular stroma covered by attenuated epithelium and surrounded by normal conjunctival epithelium and a dense scar-like matrix replacing the substantia propria. The LVD and MVD were significantly reduced to nil in the hypocellular conjunctival stroma of the excised blebs compared with normal conjunctiva (21.42 vs. 1.16, P<0.002 and 24.28 vs. 1, P<0.008, respectively). The LVD and MVD was also reduced (2- to 2.5-fold) in the peribleb stroma when compared with normal conjunctiva specimens., Conclusions: In this study we show reduced LCD and MVD in the hypocellular and peribleb stroma. These results may suggest a role of these vessels in an altered immune response in leaking blebs leading to an increased risk for blebitis.