Kaposi's sarcoma-associated herpesvirus: clinical, diagnostic, and epidemiological aspects

Kaposi's sarcoma-associated herpesvirus (KSHI) is one of the few viruses proven to be associated with tumorigenesis in humans. Its causal association with all clinical and epidemiological variants of Kaposi's sarcoma (KS) is well established. KSHV is also involved in the pathogenesis of primary effusion lymphoma (PEL) and a subset of multicentric Castleman's disease (MCD). Possible associations of KSHV with other clinical settings have been extensively examined. The findings from several of these studies are contradictory and are yet to be resolved. Concentrated effort over the last decade, since the initial discovery of KSHV, led to the development of several experimental systems that resulted in a better comprehension of the biological characteristics of KSHV and set the stage for the understanding of mechainisms by which diseases are induced by the virus. The development of molecular, histological, and serological tools for KSHV diagnosis allowed researchers to track the transmission and to study the epidemiology of KSHV. These assays have been applied, in particular in ambiguous cases, in order to confirm clinically and pathologically based diagnoses. Here, we review the advances in the clinical, experimental, diagnostic, and epidemiological research of KSHV.     

A review of factors influencing participation in social and community activities for wheelchair users

Objective: To systematically identify factors associated with participation in social and community activities for adult wheelchair users (WCUs). Data sources: PubMed/MEDLINE, CINAHL, PsycINFO and EMBASE. Study selection: Quantitative and qualitative peer-reviewed publications were included, which were written in English, reported original research and investigated factors associated with social and community participation in adult WCUs. Data extraction: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were used. Factors were organized using the International Classification of Functioning, Disability and Health (ICF). Data synthesis: Thirty-five studies were selected: two of power WCUs, 10 of manual WCUs and 23 of both. Six qualitative studies, ranging in quality from 8/10 to 9/10 and 29 quantitative studies were included, ranging in quality from 4/15 to 11/15. Fifteen body function, 4 activity, 5 participation, 15 environmental and 14 personal factors were found to be associated with social and community participation. Conclusions: Social and community participation of WCUs is associated with factors from all ICF domains. Wheelchair factors, accessibility, skills with wheelchair use, pain, finances and education are modifiable factors frequently reported to be associated with participation. Experimental research focusing on modifiable factors is needed to further our understanding of factors influencing participation among WCUs.

• Implications for Rehabilitation

• Wheelchair factors, including comfort and durability, are associated with participation and may be targeted in clinical intervention.

• Wheelchair skills are clinically modifiable and have been shown to improve participation in manual wheelchair users.

• Body functions (e.g. confidence, depression and fatigue) and personal factors (e.g. finances and level of education) may be considered for clinical intervention.

     

Identification of a human endogenous LTR-like sequence using HIV-1 LTR specific primers

Using «consensus» primers derived from the LTR region of 15 HIV-1 isolates, a fragment of 583 bp was amplified from human DNA. Even though specificity was confirmed by Southern blot analysis with a conserved LTR oligonucleotide probe, no significant homologies were detected to either retroviral regions or human or non-human published sequences. Nevertheless, when used as a probe, the 583-bp fragment identified a unique DNA sequence in the human genome on chromosome 1, and cross-reactive sequences in monkey, but not mouse, DNA. This novel, unique and conserved sequence of 583 bp was used to isolate a human HS-1 clone in which the structural property of a viral LTR could be identified.     

Staging Systems for Newly Diagnosed Myeloma Patients Undergoing Autologous Hematopoietic Cell Transplantation: The Revised International Staging System Shows the Most Differentiation between Groups

The Revised International Staging System (R-ISS) and the International Myeloma Working Group 2014 (IMWG 2014) are newer staging systems used to prognosticate multiple myeloma (MM) outcomes. We hypothesized that these would provide better prognostic differentiation for newly diagnosed multiple myeloma (MM) compared with ISS. We analyzed the Center for International Blood and Marrow Transplant Research database from 2008 to 2014 to compare the 3 systems (N?=?628) among newly diagnosed MM patients undergoing upfront autologous hematopoietic cell transplantation (AHCT). The median follow-up of survivors was 48 (range, 3 to 99) months. The R-ISS provided the greatest differentiation between survival curves for each stage (for overall survival [OS], the differentiation was 1.74 using the R-ISS, 1.58 using ISS, and 1.60 using the IMWG 2014) . Univariate analyses at 3 years for OS showed R-ISS I at 88% (95% confidence interval [CI], 83% to 93%), II at 75% (95% CI, 70% to 80%), and III at 56% (95% CI, 3% to 69%; P < .001). An integrated Brier score function demonstrated the R-ISS had the best prediction for PFS, though all systems had similar prediction for OS. Among available systems, the R-ISS is the most optimal among available prognostic tools for newly diagnosed MM undergoing AHCT. We recommend that serum lactate dehydrogenase and cytogenetic data be performed on every MM patient at diagnosis to allow accurate prognostication.     

Feature rich,but user-friendly: Speech pathologists’ preferences for computer-based aphasia therapy

Purpose: High-intensity language therapy has been shown to provide greater outcomes for people with aphasia (PWA). Unfortunately, a number of issues including the ageing population and a lack of rurally-based clinicians prevent high-intensity interventions. Computer-based therapies are a potential solution to the issues of intensity and accessibility; however, this service delivery model is not commonly used. A possible reason behind the poor uptake is that current computer-based aphasia therapy (CBAT) programs may not meet the needs of speech-language pathologists (SLPs). This study investigated the preferences of SLPs with regard to the features desired in their ideal CBAT program.

Method: Phenomenological research methodology was used to explore the preferences of 10 SLPs. Data were analysed using qualitative thematic analysis.

Result: Desired features were grouped into five themes: therapy activities, stimuli, cues, access and progress data. A range of sub-themes were also identified.

Conclusion: The wide range of desirable features found in this study may reflect the extent to which current CBAT programs are considered to be useful, but perhaps do not meet the needs of users. The study’s findings provide useful information for future CBAT developers to create programs with high clinician usability.     

Alu‐Alu mediated intragenic duplications in IFT81 and MATN3 are associated with skeletal dysplasias

Skeletal dysplasias are a diverse group of rare Mendelian disorders with clinical and genetic heterogeneity. Here, we used targeted copy number variant (CNV) screening and identified intragenic exonic duplications, formed through Alu‐Alu fusion events, in two individuals with skeletal dysplasia and negative exome sequencing results. First, we detected a homozygous tandem duplication of exon 9 and 10 in IFT81 in a boy with Jeune syndrome, or short‐rib thoracic dysplasia (SRTD) (MIM# 208500). Western blot analysis did not detect any wild‐type IFT81 protein in fibroblasts from the patient with the IFT81 duplication, but only a shorter isoform of IFT81 that was also present in the normal control samples. Complementary zebrafish studies suggested that loss of full‐length IFT81 protein but expression of a shorter form of IFT81 protein affects the phenotype while being compatible with life. Second, a de novo tandem duplication of exons 2 to 5 in MATN3 was identified in a girl with multiple epiphyseal dysplasia (MED) type 5 (MIM# 607078). Our data highlights the importance of detection and careful characterization of intragenic duplication CNVs, presenting them as a novel and very rare genetic mechanism in IFT81‐related Jeune syndrome and MATN3‐related MED.     

The burden of surgery and postoperative complications in children with inflammatory bowel disease

Background

Pediatric inflammatory bowel disease (IBD) may be associated with a higher burden of surgery and postoperative complications. This study aimed to measure the burden in pediatric IBD over a 20-year period in a large tertiary referral center.