Provision of care for children with medical complexity in tertiary hospitals in England : qualitative interviews with health professionals

Background Due to medical and technological advancements, children with medical complexity are a growing population. Although previous research has identified models of care and experiences when caring for this population, the majority are the USA or Canadian based. Therefore, the aim was to identify models of care for children with medical complexity and barriers and facilitators to delivering high-quality care for this population from a 'free at point of care' national health service. Method Qualitative semistructured interviews were conducted with hospital clinicians across England and analysed using a thematic framework approach. Results Thirty-seven clinicians from 11 hospital sites were interviewed. In 6 of the hospital sites, there were 14 services identified. Majority of services had a variety of components, some shared and some unique to the individual service. Clinicians faced barriers and facilitators when caring for this population as demonstrated across five categories. Conclusions There is limited guidance and evidence on the most effective and efficient models for providing care for this population. It is not possible to determine what a service should look like as there is no consensus on the most appropriate model of care as shown in this study. Due to their complex needs, this population require coordination to ensure high standards of care. However, this was not always possible as clinicians faced barriers such as time constraints, silo thinking and a lack of available housing

On the nature of Globular and Open Clusters (GOC): a study of M16, M67, M3 & M71

A new study of two open clusters, M16 and M67, and two globular clusters, M3 and M71 is presented, exploring data taken with the WFC/INT and the Hubble Space Tele- scope (HST) in a variety of broad-band filters. A dedicated, fully functioning reduction pipeline was constructed to reduce the data used in this study. Employing SExtractor, photometry is conducted in both g and i band filters to obtain Hertzsprung-Russell (HR) diagrams which are fitted with isochrones to determine the average age, distance, metallicity and extinction of each cluster. We find that M16 is ≈ 2 kpc away, with a high dust extinction of E(B-V)≈ 0.5. For M67 the distance was found to be ≈ 0.7 kpc and the cluster is less affected by extinction, showing E(B-V)≈ 0.2. M3 is consistent with being 13 kpc away and it shows little to no extinction. For M71 the distance was found to be ∼ 3kpc and we estimate a moderate extinction of E(B-V)≈ 0.25. The re- sults of this study highlight the stark contrast between open and globular clusters. We find that M16 and M67 (open clusters) have average ages of < 10 Myrs and 3.5 Gyrs, respectively. Globular clusters are found to be much older with ages of 9.5Gyrs and 11 Gyrs for M3 and M71, respectively. It was found that open clusters contained stars that are on average more metal-poor, with lower metallicities of [Fe/H]≈ −0.8 to [Fe/H]≈ −0.5, compared to [Fe/H]≈ −0.5 and [Fe/H]≈ 0.05 for M16 and M67, re- spectively. Future work could be undertaken to correct for issues found in this study, and investigate/infer the metallicities of the birth clouds

Fetal Testosterone Predicts Sexually Differentiated Childhood Behavior in Girls and in Boys

ABSTRACT—Mammals, including humans, show sex differences in juvenile play behavior. In rodents and nonhuman primates, these behavioral sex differences result, in part, from sex differences in androgens during early development. Girls exposed to high levels of androgen prenatally, because of the genetic disorder congenital adrenal hyperplasia, show increased male-typical play, suggesting similar hormonal influences on human development, at least in females. Here, we report that fetal testosterone measured from amniotic fluid relates positively to male-typical scores on a standardized questionnaire measure of sextypical play in both boys and girls. These results show, for the first time, a link between fetal testosterone and the development of sex-typical play in children from the general population, and are the first data linking high levels of prenatal testosterone to increased male-typical play behavior in boys. Sexual differentiation of the mammalian brain occurs under the control of gonadal hormones, particularly androgens, during early development (De Vries &amp; Simerly, 2002; Ehrhardt &amp

Australia and New Zealand renal gene panel testing in routine clinical practice of 542 families

Genetic testing in nephrology clinical practice has moved rapidly from a rare specialized test to routine practice both in pediatric and adult nephrology. However, clear information pertaining to the likely outcome of testing is still missing. Here we describe the experience of the accredited Australia and New Zealand Renal Gene Panels clinical service, reporting on sequencing for 552 individuals from 542 families with suspected kidney disease in Australia and New Zealand. An increasing number of referrals have been processed since service inception with an overall diagnostic rate of 35%. The likelihood of identifying a causative variant varies according to both age at referral and gene panel. Although results from high throughput genetic testing have been primarily for diagnostic purposes, they will increasingly play an important role in directing treatment, genetic counseling, and family planning

Delivering motivational interviewing early post stroke: standardisation of the intervention

Background We applied Motivational Interviewing (MI) techniques, early after stroke, to facilitate psychological adjustment to life post-stroke. In our trial, MI-plus-usual-care increased the likelihood of normal mood at 3-months post-stroke, compared to usual-care alone. Whilst appropriate training, manuals, and supervision may increase adherence to core principles of this complex intervention, unintended variability in implementation inevitably remains. We aimed to explore the impact of variability on participant outcome. Methods Using our trial data (411 participants), we explored variation in MI delivery, examining: therapist characteristics (stroke care expertise/knowledge, psychology training); MI content (fidelity to MI techniques assessed with Motivational Interviewing Treatment Integrity code, describing therapist behaviours as MI-consistent, MI-neutral or MI-inconsistent); and MI dose (number/duration of sessions). Results The four MI therapists (two nurses/two psychologists) had varying expertise and MI delivery. Across therapists, mean average session duration ranged 29.5–47.8 min. The percentage of participants completing the per-protocol four sessions ranged 47%–74%. These variations were not related to participant outcome. There were uniformly high frequencies (>99%) of MI-consistent and MI-neutral interactions, and low frequencies (<1%) of MI-inconsistent interactions. Conclusions Variation in therapist characteristics and MI dose did not affect participant outcome. These may have been tolerated due to high fidelity to MI principles

Consultant-led UK paediatric palliative care services: Professional configuration, services, funding

Objectives: To systematically gather information on the professional team members, services provided, funding sources and population served for all consultant-led specialised paediatric palliative care (SPPC) teams in the UK. Methods: Two-part online survey. Results: Survey 1: All 17 medical leads from hospital-based or hospice-based SPPC teams responded to the survey (100% response rate). Only six services met the NICE guidance for minimum SPPC team. All services reported providing symptom management, specialist nursing care, end-of-life planning and care, and supporting discharges and transfers to home or hospice for the child's final days-hours. Most services also provided care coordination (n=14), bereavement support (n=13), clinical psychology (n=10) and social work-welfare support (n=9). Thirteen had one or more posts partially or fully funded by a charity. Survey 2: Nine finance leads provided detailed resource/funding information, finding a range of statutory and charity funding sources. Only one of the National Health Service (NHS)-based services fully funded by the NHS. Conclusions: One-third of services met the minimum criteria of professional team as defined by NICE. Most services relied on charity funding to fund part or all of one professional post and only one NHS-based service received all its funding directly from the NHS

Biallelic RIPK1 mutations in humans cause severe immunodeficiency, arthritis, and intestinal inflammation.

RIPK1 (receptor-interacting serine/threonine kinase 1) is a master regulator of signaling pathways leading to inflammation and cell death and is of medical interest as a drug target. We report four patients from three unrelated families with complete RIPK1 deficiency caused by rare homozygous mutations. The patients suffered from recurrent infections, early-onset inflammatory bowel disease, and progressive polyarthritis. They had immunodeficiency with lymphopenia and altered production of various cytokines revealed by whole-blood assays. In vitro, RIPK1-deficient cells showed impaired mitogen-activated protein kinase activation and cytokine secretion and were prone to necroptosis. Hematopoietic stem cell transplantation reversed cytokine production defects and resolved clinical symptoms in one patient. Thus, RIPK1 plays a critical role in the human immune system

Head Position in Stroke Trial (HeadPoST)- sitting-up vs lying-flat positioning of patients with acute stroke: study protocol for a cluster randomised controlled trial

Background Positioning a patient lying-flat in the acute phase of ischaemic stroke may improve recovery and reduce disability, but such a possibility has not been formally tested in a randomised trial. We therefore initiated the Head Position in Stroke Trial (HeadPoST) to determine the effects of lying-flat (0°) compared with sitting-up (≥30°) head positioning in the first 24 hours of hospital admission for patients with acute stroke. Methods/Design We plan to conduct an international, cluster randomised, crossover, open, blinded outcome-assessed clinical trial involving 140 study hospitals (clusters) with established acute stroke care programs. Each hospital will be randomly assigned to sequential policies of lying-flat (0°) or sitting-up (≥30°) head position as a ‘business as usual’ stroke care policy during the first 24 hours of admittance. Each hospital is required to recruit 60 consecutive patients with acute ischaemic stroke (AIS), and all patients with acute intracerebral haemorrhage (ICH) (an estimated average of 10), in the first randomised head position policy before crossing over to the second head position policy with a similar recruitment target. After collection of in-hospital clinical and management data and 7-day outcomes, central trained blinded assessors will conduct a telephone disability assessment with the modified Rankin Scale at 90 days. The primary outcome for analysis is a shift (defined as improvement) in death or disability on this scale. For a cluster size of 60 patients with AIS per intervention and with various assumptions including an intracluster correlation coefficient of 0.03, a sample size of 16,800 patients at 140 centres will provide 90 % power (α 0.05) to detect at least a 16 % relative improvement (shift) in an ordinal logistic regression analysis of the primary outcome. The treatment effect will also be assessed in all patients with ICH who are recruited during each treatment study period. Discussion HeadPoST is a large international clinical trial in which we will rigorously evaluate the effects of different head positioning in patients with acute stroke. Trial registration ClinicalTrials.gov identifier: NCT02162017 (date of registration: 27 April 2014); ANZCTR identifier: ACTRN12614000483651 (date of registration: 9 May 2014). Protocol version and date: version 2.2, 19 June 2014

Managing LUTS in primary care: qualitative study of GPs’ and patients’ experiences

Background: Lower urinary tract symptoms (LUTS) are common in older men and impact considerably on their quality of life. Management can be complex, and although most LUTS could be treated effectively in primary care, referrals to urology outpatients are increasing. The experiences of General Practitioners (GPs) and patients relating to the management of LUTS have not yet been fully explored. Aim: To explore GPs’ experiences of managing LUTS, together with patients’ experiences of and preferences for treatment in primary care. Design and setting: Qualitative telephone interviews with GPs and male patients presenting to primary care with bothersome LUTS. Method: Eleven GPs and 25 male patients were purposively sampled from 20 GP practices in 3 UK regions. Interviews were conducted between May 2018 and January 2019 and analysed using a framework approach. Results: Difficulty establishing causes and differentiating between prostate and bladder symptoms were key challenges to the diagnosis of LUTS in primary care, making treatment often a process of trial and error. Pharmacological treatments were commonly ineffective and often caused side-effects. Despite this, patients were generally satisfied with GP consultations and expressed a preference for treatment in primary care. Conclusion: Managing LUTS in primary care is a more accessible option for patients. Given the challenges of LUTS diagnosis, an effective diagnostic tool for use by GPs would be beneficial. Ensuring bothersome LUTS are not dismissed as a normal part of ageing is essential in improving patients’ quality of life. Greater exploration of the role of non-pharmacological treatments is needed