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Background: Infective endocarditis is an infection of microbial origin affecting the endocardial layer of the heart, mostly impacting the heart valves. Right-sided infective endocarditis mainly affects the tricuspid valve. In some cases where surgical management is indicated the patients might not be good candidates for surgery. The AngioVac drainage cannula (AngioDynamics, Latham, NY, USA) is a novel device used in debulking and suction of intravascular material. It has been reported in the literature as a novel treatment for patients with right-sided tricuspid valve endocarditis vegetations, where their size is reduced and the efficacy of antibiotics in clearing the bloodstream infection is enhanced. Methods and results: We conducted a thorough literature review to assess the uses of the AngioVac drainage cannula in the management of right-sided infective endocarditis vegetations and lesions. We collected all reported cases where the system was used for the management of right-sided infective endocarditis and performed an encompassing review of the literature. In the review, we found 65 cases reported using the AngioVac drainage cannula for the removal of right-sided infective endocarditis vegetations. Majority of the cases were successful with no complications (87.6%); 7 (10.7%) cases were successful but there were complications: 2 reported mortalities, 1 patient had worsening TR during follow-up, 3 had recurrence of the vegetation, and 1 patient remained bacteremic. There was only 1 reported failure. Four (6.1%) patients required postprocedural valvular surgery with 3 repairs and a single valve replacement. Conclusions: The AngioVac system is a possible bailout option for surgeons managing patients with right-sided infective endocarditis vegetations who are not ideal candidates for surgery. With increased reports on its use, it could be effective at reducing the microbiological burden with minimal complications. [ABSTRACT FROM AUTHOR]

The aim of this study is to comprehensively review the topics and themes studied in destination competitiveness research and to enrich tourism researchers' literature review toolbox with the computational literature review technique. A novel computational literature review framework that can be applied to review tourism research domains characterised by an abundance of literature is developed. Enabled by the developed computational literature review framework with topic modelling, this study offers a comprehensive review of 1,130 destination competitiveness studies. The results identified several promising research topics, themes and theoretical perspectives that can be perused in future destination competitiveness studies. [ABSTRACT FROM AUTHOR]

Objectives Iatrogenic injury to the internal carotid artery (ICA) is one of the most catastrophic complications of endoscopic sinus and skull base surgery. Previous research has shown that packing with a crushed muscle graft at the injury site can be an effective management technique to control bleeding and prevent the need for ICA sacrifice. Here, we describe a novel and readily available repair donor site—an autologous lateral tongue muscle patch. Design Three representative cases of a successful repair of ICA injuries using a lateral tongue muscle patch are included in this study. The graft measured approximately 2 × 3 cm and was taken from the lateral intrinsic tongue musculature. We describe the harvest of the graft, its advantages, and the details of operative repair. Results The lateral tongue provides a large and readily accessible source of muscle within the surgical field that can be quickly harvested during an endoscopic procedure. For the first case, an expanding parasellar ICA pseudoaneurysm was managed with a tongue muscle patch and nasal packing. In the second case, a cavernous ICA injury was sustained during craniopharyngioma resection. Case three involved an ICA injury during endonasal debridement of invasive fungal rhinosinusitis. None of the patients required embolization or neurovascular stenting. Postoperative angiograms and serial computed tomography angiograms showed complete resolution of the pseudoaneurysm, and the patients continued to do well at least 1 year after repair. Conclusion Lateral tongue muscle graft is an effective and efficient method to manage ICA injuries during endoscopic endonasal surgery. Advantages include the speed of harvest, donor site being readily accessible in the surgical field, and low donor site morbidity. It should be added to the repertoire of possible donor sites for addressing catastrophic sinonasal bleeding. [ABSTRACT FROM AUTHOR]

Patients with Aarskog–Scott syndrome (AAS) have short stature, facial anomalies, skeletal deformities, and genitourinary malformations. FYVE, RhoGEF, and PH domain-containing 1 (FGD1) is the only known causative gene of AAS. However, the diagnosis of AAS remains difficult, and specific treatments are still absent. Patients suspected with AAS were recruited, and clinical information was collected. Genetic testing and functional analysis were carried out for the diagnosis. By literature review, we summarized the clinical and genetic characteristics of FGD1-related AAS and analyzed the genotype–phenotype correlation. Five patients were recruited, and four novel FGD1 variants were identified. The diagnosis of AAS was confirmed by genetic analysis and functional study. Three patients treated with growth hormone showed improved heights during the follow-up period. By literature review, clinical features of AAS patients with FGD1 variants were summarized. Regarding FGD1 variations, substitutions were the most common form, and among them, missense variants were the most frequent. Moreover, we found patients with drastic variants showed higher incidences of foot and genitourinary malformations. Missense variants in DH domain were related to a lower incidence of cryptorchidism. Conclusion: We reported four novel pathogenic FGD1 variations in AAS patients and confirmed the efficacy and safety of growth hormone treatment in FGD1-related AAS patients with growth hormone deficiency. Additionally, our literature review suggested the crucial role of DH domain in FGD1 function. What is Known: • Aarskog–Scott syndrome is a rare genetic disease, and the only known cause is the variant in FGD1 gene. The typical clinical manifestations of AAS include facial, skeletal, and urogenital deformities and short stature. What is New: • We reported four novel FGD1 variants and reported the treatment of growth hormone in FGD1-related AAS patients. Our genotype–phenotype correlation analysis suggested the crucial role of DH domain in FGD1 function. [ABSTRACT FROM AUTHOR]

Distraction osteogenesis is utilized to increase intracranial volume in the treatment of restrictive pathologies, most commonly syndromic synostosis. Children too young for open calvarial vault expansion or other systemic or local contraindications to a direct reconstructive approach benefit greatly from distraction osteogenesis, typically addressing posterior vault expansion. Wound infection, cerebrospinal fluid (CSF) leak, device failure, need for a second surgery for removal, and cost, are issues that can limit the use of this approach. These challenges are more pronounced in low- and middle-income countries (LMICs) due to lack of access to the device, the financial burden of the need for a second surgery, and the severity of the implications of infection and CSF leak. Over the last five decades, there has been an increased acceptance of bioresorbable instrumentation in craniofacial surgery. Poly L-lactic acid, polyglycolic acid, and polydioxanone are the most commonly used polymers. New resorbable fixation tools such as ultrasound-activated pins and heat-activated pins are superior to conventional bioresorbable screws in allowing attachment to thinner bone plates. In this paper, we present a review of the literature on cranial vault distraction and the use of bioresorbable materials and propose a novel design of a fully absorbable cranial distractor system using external magnetic distraction control, eliminating the need for external activation ports and a second surgery to remove the hardware. The application of this technology in LMIC settings could advance access to care and treatment options for patients with syndromic synostosis. [ABSTRACT FROM AUTHOR]

NOTCH1-related leukoencephalopathy is a new diagnostic entity linked to heterozygous gain-of-function variants in NOTCH1 that neuroradiologically show some overlap with the inflammatory microangiopathy Aicardi-Goutières syndrome (AGS). To report a 16-year-old boy harbouring a novel NOTCH1 mutation who presented neuroradiological features suggestive of enhanced type I interferon signalling. We describe five years of follow-up and review the current literature on NOTCH1-related leukoencephalopathy. Clinical evaluation, standardised scales (SPRS, SARA, CBCL, CDI-2:P, WISCH-IV and VABS-2) and neuroradiological studies were performed, as well as blood DNA analysis. For the literature review, a search was performed on Pubmed, Scopus and Web of Science up to December 2023 using the following text word search strategy: (NOTCH1) AND (leukoencephalopathy). Our patient presents clinical features consistent with other reported cases with NOTCH1 mutations but is among the minority of patients with an onset after infancy. During the five-year follow-up, we observed an increase in the severity of spasticity and ataxia. However, at the age of 16 years, our proband is still ambulatory. As for other reported patients, he manifests psychiatric features ranging from hyperactivity during childhood to anxiety and depression during adolescence. The neuroradiological picture remained essentially stable over five years. In addition to the typical findings of leukoencephalopathy with cysts and calcifications already described, we report the presence of T2-hyperintensity and T1-hypotensity of the transverse pontine fibres, enhancement in the periventricular white matter after gadolinium administration and decreased NAA and Cho peaks in the periventricular white matter on MRS. We identified a novel heterozygous variant in NOTCH1 (c.4788_4799dup), a frame insertion located in extracellular negative regulatory region (NRR)-domain as in previously published cases. Blood interferon signalling was not elevated compared to controls. This case provides further data on a new diagnostic entity, i.e., NOTCH1-related leukoencephalopathy. By describing a standardised five-year follow-up in one case and reviewing the other patients described to date, we outline recommendations relating to monitoring in this illness, emphasising the importance of psychiatric and gastroenterological surveillance alongside neurological and neuropsychological management. Studies are needed to better understand the factors influencing disease onset and severity, which are heterogeneous. [ABSTRACT FROM AUTHOR]

Objective: Through a case of deficiency of adenosine deaminase 2 (DADA2) to improve domestic clinicians' understanding of the disease, and to review the literature, promote dermatologists for clinical secondary primary lesion diagnosis. Method: Analysis of a case diagnosed with DADA2 deficiency of clinical manifestations, laboratory, imaging examination and treatment methods, and discussion through literature analysis. Results: The child with recurrent fever, limbs nodular erythema, gradually in the limbs. CT of lower limb skin showed mild edema of the spinous layer, intact basal layer, dilated vascular congestion in the superficial dermis, visible RBC extravasation, and changes of telangiectasia ring purpura were considered. Cranial magnetic resonance imaging (MRI) showed a left choroidal cleft cyst. Genetic test was the CECR1 mutation. The treatment with adalimumab was effective. Conclusion: In this case, DADA2 is the seventh case in China, and the CECR1 mutation site (c.254A> T p.N85I,c.851G>T p. G284V) was a compound heterozygous mutation. Mastering the clinical characteristics is helpful for clinicians to diagnose this disease. [ABSTRACT FROM AUTHOR]

Myeloid/lymphoid neoplasms with eosinophilia (M/LN‐eo) and tyrosine kinase (TK) gene fusions are a rare group of haematopoietic neoplasms with a broad range of clinical and morphological presentations. Paediatric cases have increasingly been recognised. Importantly, not all appear as a chronic myeloid neoplasm and eosinophilia is not always present. In addition, standard cytogenetic and molecular methods may not be sufficient to diagnose M/LN‐eo due to cytogenetically cryptic aberrations. Therefore, additional evaluation with fluorescence in‐situ hybridisation and other molecular genetic techniques (array‐based comparative genomic hybridisation, RNA sequencing) are recommended for the identification of specific TK gene fusions. M/LN‐eo with JAK2 and FLT3‐rearrangements and ETV6::ABL1 fusion were recently added as a formal member to this category in the International Consensus Classification (ICC) and the 5th edition of the WHO classification (WHO‐HAEM5). In addition, other less common defined genetic alterations involving TK genes have been described. This study is an update on M/LN‐eo with TK gene fusions with focus on novel entities, as illustrated by cases submitted to the Bone Marrow Workshop, organised by the European Bone Marrow Working Group (EBMWG) within the frame of the 21st European Association for Haematopathology congress (EAHP‐SH) in Florence 2022. A literature review was performed including paediatric cases of M/LN‐eo with TK gene fusions. [ABSTRACT FROM AUTHOR]

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease characterized by progressive degeneration of upper and lower motor neurons, with occasional involvement of the extrapyramidal system. Mutations in the sigma non-opioid intracellular receptor 1 (SIGMAR1) gene have been identified as one of the causes of ALS. Here, we present a case of a 49-year-old man diagnosed with ALS--Parkinson's disease (PD) complex. The patient exhibited bradykinesia and tremor, and whole-exome sequencing revealed homozygous mutations in the SIGMAR1 gene (c.446-2A > T). In addition, we conducted an investigation into the clinical and molecular phenotype of previously reported variants of SIGMAR1 associated with ALS. This case report aims to raise awareness among physicians regarding atypical phenotypes of amyotrophic lateral sclerosis and to encourage further research on the factors leading to SIGMAR1 mutations in patients. [ABSTRACT FROM AUTHOR]

Supply chain collaboration has been widely discussed in the literature. With this maturity comes a plethora of heterogeneous research that is difficult to manage and navigate. This paper, therefore, applies a novel literature review approach based on text mining analyzing 10,556 articles to provide an overview of previous research themes and future directions of the field. The applied method enables researchers to systematically analyze and structure larger samples of research publications. It allocates articles to thematic clusters using a visual hierarchical clustering approach and subsequently aggregates them into nine overarching themes to determine potential research and insights for practice. Developments regarding research interest and attention within these themes are examined and journals publishing the most impactful articles are identified. The paper thus contributes to the field of Supply Chain Collaboration research by mapping its evolvement over the last five years and by deriving a research agenda for the next decade. [ABSTRACT FROM AUTHOR]

Increasing congestion and the push for (i) a greener modal shift, (ii) more reliability and visibility of goods, and (iii) more sustainable activities in and related to the port are turning policymakers to the concept of the smart port. Such emerging concept describes a future state of the port that is adapted to these challenges. A smart port is a port that optimizes in-, intra- and outbound flow of goods and information, leads sustainable development and guarantees safe, resilient and secure activities through the capabilities of its (extended) port community and enabling technologies. However, turning a traditional port into a smart port is a strategic challenge for the port community at large. To contribute to this practically relevant and academic challenge, the present paper applies a literature review and the novel maturity model development methodology to develop a strategic tool. This newly-developed strategic tool (i) enhances the common understanding of the current and aspired maturity state, (ii) support decision-making of port policy and management actors and (iii) represent a maturation path for improvement for port communities. The case study results demonstrate the benefits and relevance of the tool. Finally, recommendations are made to apply this policy tool for digitalization strategy. [ABSTRACT FROM AUTHOR]

The non-orthogonal multiple access (NOMA) system is considered an important technology that enables the fifth-generation (5G) wireless systems and beyond to satisfy different requirements such as high efficiency, massive networks, sophisticated optimization, and steady quality. Also, the NOMA scheme provides advancements and attractive characteristics such as low latency, ultra-dense service, great fairness, innovative waveform architecture, efficient bandwidth utilization, and massive device connectivity compared to the earliest multiple access schemes. Therefore, the NOMA system necessitates an efficient resource allocation technique such as user pairing (UP) and power allocation (PA) schemes to achieve optimal performance. So, in this paper, we discuss the significance of resource allocation in NOMA in 5G networks and beyond in-depth. As a result, firstly, we review the classification of multiple access schemes, the various types of NOMA techniques, and the characteristics of NOMA. Then, the paper analyzes the issue of resource allocation by classifying the different resource allocation schemes in 5G. Further, a summary of the solutions to the current resource allocation issues are reviewed. Finally, we suggest future research challenges on which to focus. [ABSTRACT FROM AUTHOR]

"Sweet" hydrothorax is a rare complication of peritoneal dialysis (PD). It is characterized by the presence of peritoneal fluid in the pleural cavity. We describe the case of a 41-year-old woman who developed this complication three days after starting continuous ambulatory peritoneal dialysis (CAPD). We present the current diagnostic approach and treatment of this rare complication. [ABSTRACT FROM AUTHOR]

Carl Jung's theory of synchronicity is defined by Jung as the "occurrence of a certain psychic state with one or more external events which appear as meaningful parallels to the momentary subjective state." Fundamental to this proposal, developed with physicist Wolfgang Pauli, is the profound assertion that the acausal nature of synchronicity is "equal in rank to causality as a principle of explanation." The epistemology of this concept is explored via the exposition of novel ideas and literature review. The supportive and transformative healing value of discovering and sharing a synchronicity experience is revealed in diverse psychotherapeutic modalities ranging from Alcoholics Anonymous (AA) to analytical psychology, and via indigenous-shamanic healing practices. Attention is also devoted to reporting the positive, possibly curative nature and attributes of synchronicities that enhance the spiritual aspect of recovery from addictive process disorders and other maladies of postmodern life. [ABSTRACT FROM AUTHOR]

This study is an attempt to review relevant literature on the theme of corporate real investment decisions. We have conducted a comprehensive survey of literature on the studies published in well-reputed journals of finance, i.e., The Journal of Finance, The Review of Financial Studies, and The Journal of Financial Economics, during the years 2010 to 2022. The theoretical analysis reveals that information asymmetry, cash holdings, policy uncertainty, idiosyncratic risk, governance quality, financing diversification, financial development, managerial network, investor protection, tax policy, etc., are prominent factors influencing investment decisions. The current review analysis is useful and has certain policy implications for investment managers regarding investment decisions. It guides on the factors that can impede or boost investment volume. Our study has a novel contribution to the literature by summarizing the voluminous empirical literature arranged on physical investment decisions. [ABSTRACT FROM AUTHOR]

Realist evaluation is a method increasingly adopted to provide an understanding of how and why a program works, for whom, and under what circumstances. Initial program theories (IPT) are the crucial starting point of any realist evaluation, however descriptions about how they are developed in practice remain under-reported in the published literature. This article argues for the value of genuine research-group conversations using David Bohm's concept of dialogue in realist research. We label it the realist dialogic approach. We draw out the relational qualities as well as the contextual circumstances of dialogue through our development of IPT and interview guides for a research study on the implementation and scaling of a large-system value-based program to transform complex health services. We selected the relevant middle-range theories, conducted a literature review, and drew on informal discussions with key stakeholders, to develop IPT through research-group conversations. The benefits of this approach were: 1) development of rigorous, novel, deep and well-tailored IPT, 2) detailed understanding of the complex intervention under investigation and development of rapport and networks with participants, 3) empirically grounded Context-Mechanism-Outcome (CMO) configurations, predicated on suitable abstract and contextually-contingent middle-range theories, and 4) productive research team interactions which supported the entire research process. The challenges of this approach include: 1) establishing and retaining a sense of humility across the research team, 2) contextual circumstances can hinder dialogic relationship, and 3) time and resource heavy. This paper uses middle-range theory and ethnographic insights to advance the existing practice of realist evaluations and offer transferable lessons to other scholars considering similar approaches. Moreover, we content that the use of middle-range theory to extend the methodological literature is a novel contribution to realist work. [ABSTRACT FROM AUTHOR]

Congenital melanocytic nevus is a benign proliferation seen from birth. However, malignant transformation can be observed in later ages, so the removal of especially large and giant nevi is recommended during childhood. Nevertheless, there are no cases reported in the literature regarding excision of giant congenital melanocytic nevi in advanced age. This article presents the first case of a 39-year-old patient with a giant congenital melanocytic nevus covering 10% of the total body surface area, who underwent treatment with a 2-step operation. The nevus was located on the back, covering 10% of the total body surface area. The patient underwent en-bloc excision. A bilayer dermal matrix was applied over the fascia. Subsequently, a split-thickness skin graft was applied to the entire area. Full re-epithelialization was achieved within a total of 35 days. Thanks to the applied dermal scaffold, the area became pliable. [ABSTRACT FROM AUTHOR]

Chordomas are rare malignant neoplasms, accounting for 1–4% of all primary bone tumors. Most spinal chordomas occur in the sacrococcygeal region and the base of the skull; however, 6% of chordomas are observed in the cervical spine. In these cases, the lesion is mainly located in the midline. These tumors slowly grow before becoming symptomatic and encase the surrounding vascular and nerve structures. Patients with advanced chordoma have a poor prognosis due to local recurrence with infiltration and destruction of adjacent bone and tissues. Systemic chemotherapy options have not been fully effective in these tumors, especially for recurrent chordomas. Thus, new combinations of currently available targeted molecular and biological therapies with radiotherapy have been proposed as potential treatment modalities. Here, the present paper describes the case of a 41-year-old male with a C2–C4 chordoma located paravertebrally, who underwent surgical resection with a debulking procedure for a cervical chordoma. Computed tomography angiography revealed a paraspinal mass with bone remodeling and the MRI showed a paravertebral mass penetrating to the spinal canal with a widening of the intervertebral C2–C3 foramen. Initially, the tumor was diagnosed as schwannoma based on its localization and imaging features; however, the histopathology specimen confirmed the diagnosis of chordoma. This case study highlights the effectivity of radical surgical resection as a mainstay treatment for chordomas, discusses neuroimaging, diagnosis, and the use of currently available targeted therapies and forthcoming treatment strategies, as alternative treatment options for chordoma. [ABSTRACT FROM AUTHOR]

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Statins is widely used in clinical practice as lipid-lowering drugs and has been proven to be effective in the treatment of cardiovascular, endocrine, metabolic syndrome and other diseases. The latest preclinical evidence shows that statins have anti-proliferation, pro-apoptotic, anti-invasion and radiotherapy sensitization effects on tumor cells, suggesting that statins may become a new type of anti-tumor drugs. For a long time, mevalonate pathway has been proved to play a supporting role in the development of tumor cells. As an effective inhibitor of mevalonate pathway, statins have been proved to have a direct auxiliary anti-tumor effect in a large number of studies. In addition, anti-tumor effects of statins through ferroptosis, pyroptosis, autophagy and tumor microenvironment (TME) have also been gradually discovered. However, the specific mechanism of the antitumor effect of statins in the tumor microenvironment has not been clearly elucidated. Herein, we reviewed the antitumor effects of statins in tumor microenvironment, focusing on hypoxia microenvironment, immune microenvironment, metabolic microenvironment, acid microenvironment and mechanical microenvironment. [ABSTRACT FROM AUTHOR]

• A novel frameshift mutation (SUPT5H c.3032_3033delTG) and a novel nonsense mutation (SUPT5H c. 397C > T) causing increased Hb A 2 level was identified. • Currently reported SUPT5H mutations were compiled, in order to perform the genotype-phenotype correlation analysis. • The rare cases are instructive for clinical diagnosis and management. β-thalassemia is one of the most common monogenic disorders in areas of the tropics and subtropics, which represents a major familial and social burden to local people. The elevated Hb A 2 level, generally specified as greater than 3.5 %, is commonly used as a high efficiency index for screening of β-thalassemia carriers. However, mutations in other genes such as GATA1 and KLF1 , could also result in increased Hb A 2 level. In this study, we identified two novel variants in the SUPT5H gene: a frameshift mutation (SUPT5H : c.3032_3033delTG, p.M1011Mfs*9) and a nonsense mutation (SUPT5H : c.397C > T, p.Arg133*) in two Chinese individuals. Utilizing a combination of phenotype analysis, bioinformatics analysis, and functional analysis, we deduced that these two variants modified the SUPT5H protein's structure, thereby impacting its function and consequently leading to the heightened Hb A 2 level phenotype found in the carriers. Furthermore, through a comprehensive literature review, a mutation spectrum was consolidated for SUPT5H , an investigation into the genotype-phenotype correlation was conducted, and factors known to influence Hb A 2 levels were identified. Based on this in-depth understanding, clinicians are better equipped to carry out large scale screenings in regions with high prevalence of β-thalassemia. [ABSTRACT FROM AUTHOR]

Purpose: This article presents a review of the existing state-of-the-art literature concerning Supply Chain Management 4.0 (SCM 4.0) and identifies and evaluates the relationship between digital technologies and Supply Chain Management. Design/methodology/approach: A literature review of state-of-the-art publications in the subject field and a bibliometric analysis were conducted. Findings: The paper identifies the impact of novel technologies on the different supply chain processes. Furthermore, the paper develops a roadmap framework for future research and practice. Practical implications: The proposed work is useful for both academics and practitioners as it outlines the pillar components for every supply chain transformation. It also proposes a range of research questions that can be used as a base to guide the future research direction of the field. Originality/value: This paper presents a novel and original literature review-based study on SCM4.0 as no comprehensive review is available where bibliometric analysis, motivations, barriers and technologies' impact on different SC processes have been considered. [ABSTRACT FROM AUTHOR]

Background: Implanting hardware into surgical sites increases the rate of infection associated with these sites. Without novel efforts to reduce this rate of infection, we can expect to see an increase in the number of hardware-associated infections as more patients are implanted with these devices. These infections often necessitate the removal of these devices resulting in a significant financial and clinical burden to patients. We developed a prototype antibiotic coating using products that are both low cost and that can be sourced easily. Our study aims to test the effectiveness of this coating against bacteria commonly observed in hospital-associated infections. Methods: The antibiotic coating was prepared by combining one gram of vancomycin and 500 mg of ciprofloxacin in 50 mL of glycerol. The coating was examined for inhibition of growth of Pseudomonas aeruginosa PA14 and Staphylococcus aureus AH2486 and compared with the bacterial growth of the above bacteria in glycerol alone. The growth curves were plotted measuring the bacterial growth at 5 h intervals. Results: The results of the growth curves clearly demonstrate a lack of bacterial growth when these bacteria are combined with glycerol combined with our selected antibiotic agents. Conclusion: There appears to be a limited interest from device companies in developing new strategies for infection prevention associated with neurosurgical hardware, and we propose that this prototype will be an effective and low-cost solution to a large problem. [ABSTRACT FROM AUTHOR]

Because of the vast number of academic products with focus on sustainability being currently generated, it is becoming exceedingly difficult and time-consuming to perform accurate literature reviews that have actual relationship with such a topic and correctly reflect its state of the art. Thus, this work proposes a comprehensive Systematic Literature Review (SLR) design as a novel approach for collecting the body of contributions for any given research field in general and sustainability in particular. This design is guided by a semi-automated procedure that aims at incorporating the best automation features of earlier SLRs and involves available scientific databases, e.g., Scopus and Web of Science. The results of the proposed SLR design include bibliometric analyses, a list of researcher and institution networks, and a qualitative assessment of practical applications of the field of interest. Moreover, the proposed semi-automated approach allows for the inclusion of subject categorization based on the generation of author-density maps and keyword co-occurrence networks. For illustration, the semi-automated SLR design is implemented by thoroughly reviewing the contributions pertaining to the P-graph (Process Graph) framework with a focus on sustainability. The P-graph framework has been applied to multiple areas, such as network design, optimization, and system integration; in particular, the framework has proven to be useful for the development of sustainable systems. Upon execution, the semi-automated SLR design collected 284 contributions related to P-graph, of which 139 were classified as sustainability-related, and further partitioned into specific categories. It is expected that the proposed semi-automated SLR design will help researchers in the sustainability community accelerate the generation of works in that area by rapidly generating accurate literature reviews. [Display omitted] • Presents a novel Systematic Literature Review design with automation tools. • Integrates semi-automated solutions comprehensively in the full review process. • Includes bibliometric-, network-, and text-mining aided content analysis has a high potential for full-automation via AI tools. • Demonstrated the SLR design in the research area of P-graph and sustainability. [ABSTRACT FROM AUTHOR]

The original article can be found online at https://doi.org/10.1007/s11276-022-03175-7. B Correction to: Wireless Networks b https://doi.org/10.1007/s11276-022-03175-7 The original version of this article unfortunately contained a mistake in the author group. The correct affiliations for Dr. Mohamed Elwekeil are published with this article. [Extracted from the article]

Although fan fiction studies has historically focused overwhelmingly on (cis)female fans, research suggests that trans fans--here used as an umbrella term for gender nonnormative fans--are a significant proportion of fan fiction communities. This literature review summarizes recent studies that discuss fan fiction and trans fans, as well as research exploring various genres of fan fiction that play with gender to consider the reasons such fan fiction may appeal to trans fans specifically. [ABSTRACT FROM AUTHOR]

The Banki turbine is a crossflow turbine suitable for sites with heads below 200 m and flow rates below 10 m3/s, with maximum efficiency around 80%. Its flexible operation and easy manufacturing make it a suitable hydropower technology for different geographic areas and hydraulic contexts. However, the design procedure proposed in literature is not complete and it is quite fragmented. It lacks of effective equations to select the optimal number of blades, the optimal tip speed ratio, the rotational speed and a preliminary cost estimate. Information on runner material and blade thickness is also fragmented. In this paper, the traditional design procedures are reviewed, and a new and more complete one is proposed to overcome the above-mentioned gaps, providing new expeditious equations and data to be used in practical applications. The new equations are obtained by elaborating and generalizing literature and industrial data, presenting them in a dimensionless form. The current share of Banki turbines and their future developments and opportunities are also discussed. • The Banki turbine is a crossflow turbine with usual maximum efficiency 80%. • Literature data are elaborated to find new design equations for the blade design and rotational speed selection. • The proposed design methodology overcomes the limitations of traditional ones. • A typical cost ranges between 1000 and 6000 €/kW. [ABSTRACT FROM AUTHOR]

The ZNF142 gene on chromosome 2q35 contains ten exons and encodes a zinc finger protein 142 with 31 C2H2-type zinc fingers domain. Pathogenic variants in ZNF142 result in an autosomal recessive neurodevelopmental disorder with impaired speech and developmental delay. Here, we report two novel variants (NM_001105537: c.25C > T/c.1741C > T, p.Gln9*/p.Arg581Cys) in ZNF142 in an Iranian family identified by Whole-Exome sequencing and confirmed by Sanger sequencing. These variants are categorized as "pathogenic" and "variant of unknown significance" based on the standards for the interpretation of sequence variations recommended by ACMG, respectively. The proband is a five-year-old male born to consanguineous parents. The compound heterozygous variant (NM_001105537: c.25C > T/c.1741C > T, p.Gln9*/p.Arg581Cys) in ZNF142 was identified in the proband with moderate intellectual disability, global developmental delay, speech impairment, and seizures. This paper reported the sixth family in the world with novel pathogenic variants in the ZNF142 gene as the reason for neurodevelopmental Disorder with Impaired Speech and Hyperkinetic Movements (NEDISHM) and determining the phenotype spectrum of this disease. In this study, we also reviewed the phenotype of the former cases. In contrast to the Malaysian cases, proband in the present paper does not manifest any facial features similar to the patients in the initial study. Further studies on the NEDISHM patients could be valuable to determine the phenotype precisely. [ABSTRACT FROM AUTHOR]

The online version of the original article can be found at https://doi.org/10.1007/s10103-013-1446-7 Publisher's note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Correction to: Lasers Med Sci https://doi.org/10.1007/s10103-013-1446-7 Original text, taken from the on-line version: Following the method of Duysens, adapting only the terminology, we arrive at: 3 HT ht Graph where I i SB a,blood sb and I i SB a,Hb sb are the absorption coefficient of a blood sample and haemoglobin solution, respectively. [Extracted from the article]

Simple Summary: Molecular profiling of unclassified neoplasms has been pivotal in the characterization of new entities. Cutaneous melanocytic tumor with CRTC1::TRIM11 fusion (CMTCT) is a newly described neoplasm that falls into the broad category of diagnostically challenging dermal proliferations with melanocytic differentiation. The aim of this review is to educate colleagues about the clinical, histopathologic, and molecular features of CMTCT, and provide important information on the differential diagnosis. "Cutaneous melanocytic tumor with CRTC1::TRIM11 fusion" (CMTCT) is a recently described entity belonging to the family of superficial tumors displaying melanocytic differentiation. Thirteen cases have been reported so far, on the head and neck, extremities, and trunk of adults of all ages (12 cases) and one in an 11-year-old child. Histopathologically, it is a nodular or multilobulated tumor composed of spindle and epithelioid cells arranged in nests, fascicles, or bundles that are surrounded by thin collagenous septa. By immunohistochemistry, the tumor shows variable immunoreactivity for S100-protein, SOX10, and MITF, as well as specific melanocytic markers such as MelanA and HMB-45. The neoplasm's biologic behavior remains uncertain since the reported cases are limited and the follow-up is short (median 12 months). However, local recurrence and synchronous distant metastasis after 13 years of initial resection has been described in one case. Herein, we present a comprehensive literature review of CMTCT hoping to raise awareness among the dermatopathologists of this potentially novel entity. [ABSTRACT FROM AUTHOR]

Cerebrospinal fluid (CSF) diversion is among the most commonneurosurgical procedures that are performed worldwide. It is estimated thatapproximately 30,000 ventriculostomies are performed annually in the United States.Ventriculoperitoneal (VP) shunt malfunction rate within the first year of initialimplantation has been reported to be as high as 11–25%. In patients with abdominaladhesions, infections or multiple failed VP shunts, another bodily compartment shouldbe utilized as a substitute for the peritoneal cavity for distal shunt catheter placement.Ventriculopleural (VPL) shunting for hydrocephalus was first introduced by Heile in1914. Since the inception of this idea, VPL shunts have been utilized in select patientswith varying degrees of success. There have been a number of case reports andseries documenting unique complications with VPL shunting, with pleural effusion andpneumothorax being the most common complications. In our review article, we soughtto review the development of VPL shunting, pleuropulmonary physiology, insertiontechniques for VPL shunt, complications associated with VPL shunts, and uniquestrategies to improve VPL shunt tolerance. • VPL shunting for hydrocephalus was first introduced by Heile in 1914. • Pleural effusion is the most common complication associated with VPL shunting. • VPL shunting may offer utility as an alternative treatment for CSF diversion. • The number of studies on VPL shunting remain sparse in the literature. [ABSTRACT FROM AUTHOR]

NGLY1 deficiency is a recently described autosomal recessive disorder, involved in deglycosylation of proteins, and for that reason grouped as the congenital disorders of deglycosylation together with the lysosomal storage disorders. The typical phenotype is characterized by intellectual disability, liver malfunctioning, muscular hypotonia, involuntary movements, and decreased or absent tear production. Liver biopsy demonstrates vacuolar amorphous cytoplasmic storage material. NGLY1 deficiency is caused by bi-allelic variants in NGLY1 which catalyzes protein deglycosylation. We describe five patients from two families with NGLY1 deficiency due to homozygosity for two novel NGLY1 variants, and compare their findings to those of earlier reported patients. The typical features of the disorder are present in a limited way, and there is intra-familial variability. In addition in one of the families the muscle atrophy and posture abnormalities are marked. These can be explained either as variability of the phenotype or as sign of slowly progression of features as the present affected individuals are older than earlier reported patients. [ABSTRACT FROM AUTHOR]