Base of Skull & Spinal Canal Narrowing in an Adolescent with Autosomal Recessive Hypophosphatemic Rickets Type 2.

Autosomal recessive hypophosphatemic rickets type 2 (ARHR2) is an uncommon hereditary form of rickets characterised by chronic renal phosphate loss and impaired bone mineralisation. This results from compound heterozygous or homozygous pathogenic variants in ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1), a key producer of extracellular inorganic pyrophosphate (PPi) and an inhibitor of fibroblast growth factor23 (FGF23). ENPP1 deficiency impacts FGF23 and increases its activity.

Impact of stopping burosumab treatment at the end of skeletal growth in adolescents with X-linked hypophosphatemia (XLH).

Many adolescents with X-linked hypophosphatemia (XLH) currently have to stop treatment with burosumab at the end of skeletal growth. We describe the experience of a cohort of adolescents with XLH before, during, and after stopping burosumab (median treatment duration 37.5 months).

Social contact patterns following the COVID-19 pandemic: a snapshot of post-pandemic behaviour from the CoMix study.

The COVID-19 pandemic led to unprecedented changes in behaviour. To estimate if these persisted, a final round of the CoMix social contact survey was conducted in four countries at a time when all societal restrictions had been lifted for several months. We conducted a survey on a nationally representative sample in the UK, Netherlands (NL), Belgium (BE), and Switzerland (CH).

Small Molecule Antagonists of the DNA Repair ERCC1/XPA Protein-Protein Interaction.

The DNA excision repair protein ERCC1 and the DNA damage sensor protein, XPA are highly overexpressed in patient samples of cisplatin-resistant solid tumors including lung, bladder, ovarian, and testicular cancer. The repair of cisplatin-DNA crosslinks is dependent upon nucleotide excision repair (NER) that is modulated by protein-protein binding interactions of ERCC1, the endonuclease, XPF, and XPA. Thus, inhibition of their function is a potential therapeutic strategy for the selective sensitization of tumors to DNA-damaging platinum-based cancer therapy.

A dual-receptor T-cell platform with Ab-TCR and costimulatory receptor achieves specificity and potency against AML.

Chimeric antigen receptor T-cell (CAR T) therapy has produced remarkable clinical responses in B-cell neoplasms. However, many challenges limit this class of agents for the treatment of other cancer types, in particular the lack of tumor-selective antigens for solid tumors and other hematological malignancies, such as acute myeloid leukemia (AML), which may be addressed without significant risk of severe toxicities while providing sufficient abundance for efficient tumor suppression. One approach to overcome this hurdle is dual targeting by an antibody-T-cell receptor (AbTCR) and a chimeric costimulatory signaling receptor (CSR) to 2 different antigens, in which both antigens are found together on the cancer cells but not together on normal cells.

Surge of lower respiratory tract group A streptococcal infections in England in winter 2022: epidemiology and clinical profile.

Background: Following low incidence of invasive group A streptococcal (iGAS) infections during the COVID-19 pandemic, marked increases were noted in many countries during 2022, particularly in children. In November 2022, severe presentations of lower respiratory tract infections (LRTIs), including empyema, were notified by clinicians across the UK. UKHSA investigated this rise with the aim of informing clinical management and public health response.

Did anonymity in self-administered questionnaires improve disclosure of sensitive information during the 2022 mpox outbreak in England? An observational study.

Background: Between May 6, 2022, and Jan 16, 2023, 3555 mpox cases were reported in England, predominantly in gay, bisexual, and other men who have sex with men. Initially, the UK Health Security agency administered questionnaires to laboratory-detected cases via telephone calls. From June, 2022, cases were requested by text or email to complete the questionnaire online, with optional anonymous completion.

Assessing the strengths and limitations of quantitative micromorphometry for the forensic examination of small arms propellant towards brand identification.

Smalls arms propellants (SAP) also known as canister powders are readily accessible and cost-effective materials that firearms enthusiasts can acquire for the legitimate assembly of ammunition. These attributes also make SAPs advantageous for the construction of improvised explosive devices (IEDs). Thus, there is a need to develop robust metrics for the characterization of propellants to provide investigative leads as well as for comparisons between known and recovered residues.

Preliminary investigation of a significant national exceedance in the United Kingdom, August 2023 and ongoing.

Routine laboratory surveillance has identified an unprecedented and ongoing exceedance of spp. across the United Kingdom, notably driven by transmission, since 14 August 2023. Information from 477 reported cases in England and Wales, followed up with a standardised exposure questionnaire as of 25 September 2023, identified foreign travel in 250 (54%) of 463 respondents and swimming in 234 (66%) of 353 cases.

Utilising qualitative data for social network analysis in disaster research: opportunities, challenges, and an illustration.

An abundance of unstructured and loosely structured data on disasters exists and can be analysed using network methods. This paper overviews the use of qualitative data in quantitative social network analysis in disaster research. It discusses two types of networks, each with a relevant major topic in disaster research-that is, (i) whole network approaches to emergency management networks and (ii) personal network approaches to the social support of survivors-and four usable forms of qualitative data.

Mortality among populations affected by armed conflict in northeast Nigeria, 2016 to 2019.

Armed conflict, displacement and food insecurity have affected Adamawa, Borno, and Yobe states of northeast Nigeria (population ≈ 12 million) since 2009. Insecurity escalated in 2013 to 2015, but the humanitarian response was delayed and the crisis' health impact was unquantified due to incomplete death registration and limited ground access. We estimated mortality attributable to this crisis using a small-area estimation approach that circumvented these challenges.

The influence of COVID-19 risk perception and vaccination status on the number of social contacts across Europe: insights from the CoMix study.

Background: The SARS-CoV-2 transmission dynamics have been greatly modulated by human contact behaviour. To curb the spread of the virus, global efforts focused on implementing both Non-Pharmaceutical Interventions (NPIs) and pharmaceutical interventions such as vaccination. This study was conducted to explore the influence of COVID-19 vaccination status and risk perceptions related to SARS-CoV-2 on the number of social contacts of individuals in 16 European countries.

Longitudinal social contact data analysis: insights from 2 years of data collection in Belgium during the COVID-19 pandemic.

Background: During the COVID-19 pandemic, the CoMix study, a longitudinal behavioral survey, was designed to monitor social contacts and public awareness in multiple countries, including Belgium. As a longitudinal survey, it is vulnerable to participants' "survey fatigue", which may impact inferences.

Methods: A negative binomial generalized additive model for location, scale, and shape (NBI GAMLSS) was adopted to estimate the number of contacts reported between age groups and to deal with under-reporting due to fatigue within the study.

Impact of tiered measures on social contact and mixing patterns of in Italy during the second wave of COVID-19.

Background: Most countries around the world enforced non-pharmaceutical interventions against COVID-19. Italy was one of the first countries to be affected by the pandemic, imposing a hard lockdown, in the first epidemic wave. During the second wave, the country implemented progressively restrictive tiers at the regional level according to weekly epidemiological risk assessments.

COVID-19 vaccine hesitancy and social contact patterns in Pakistan: results from a national cross-sectional survey.

Background: Vaccination is a key tool against COVID-19. However, in many settings it is not clear how acceptable COVID-19 vaccination is among the general population, or how hesitancy correlates with risk of disease acquisition. In this study we conducted a nationally representative survey in Pakistan to measure vaccination perceptions and social contacts in the context of COVID-19 control measures and vaccination programmes.

Social contact patterns during the COVID-19 pandemic in 21 European countries - evidence from a two-year study.

Background: Most countries have enacted some restrictions to reduce social contacts to slow down disease transmission during the COVID-19 pandemic. For nearly two years, individuals likely also adopted new behaviours to avoid pathogen exposure based on personal circumstances. We aimed to understand the way in which different factors affect social contacts - a critical step to improving future pandemic responses.

Effect of risk status for severe COVID-19 on individual contact behaviour during the SARS-CoV-2 pandemic in 2020/2021-an analysis based on the German COVIMOD study.

Background: One of the primary aims of contact restriction measures during the SARS-CoV-2 pandemic has been to protect people at increased risk of severe disease from the virus. Knowledge about the uptake of contact restriction measures in this group is critical for public health decision-making. We analysed data from the German contact survey COVIMOD to assess differences in contact patterns based on risk status, and compared this to pre-pandemic data to establish whether there was a differential response to contact reduction measures.

Comparative transmission of SARS-CoV-2 Omicron (B.1.1.529) and Delta (B.1.617.2) variants and the impact of vaccination: national cohort study, England.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) Omicron variant (B.1.1.

COVID-19 booster vaccination uptake and infection breakthrough amongst health care workers in Wales: A national prospective cohort study.

Background: From September 2021, Health Care Workers (HCWs) in Wales began receiving a COVID-19 booster vaccination. This is the first dose beyond the primary vaccination schedule. Given the emergence of new variants, vaccine waning vaccine, and increasing vaccination hesitancy, there is a need to understand booster vaccine uptake and subsequent breakthrough in this high-risk population.

Reduced time to imaging, length of stay, and hospital charges following implementation of a novel postoperative pathway for craniotomy.

Objective: The authors created a postoperative postanesthesia care unit (PACU) pathway to bypass routine intensive care unit (ICU) admissions of patients undergoing routine craniotomies, to improve ICU resource utilization and reduce overall hospital costs and lengths of stay while maintaining quality of care and patient satisfaction. In the present study, the authors evaluated this novel PACU-to-floor clinical pathway for a subset of patients undergoing craniotomy with a case time under 5 hours and blood loss under 500 ml.

Methods: A single-institution retrospective cohort study was performed to compare 202 patients enrolled in the PACU-to-floor pathway and 193 historical controls who would have met pathway inclusion criteria.