Exploring the lived experiences and care challenges of formal paid caregivers for people with intellectual disability and dementia.

A greater number of people with intellectual disability are living into older age and are at increased risk of developing conditions such as dementia. Caring for a person with dementia presents several challenges for formal caregivers due to the progressive nature of the disease. An interpretive phenomenological analysis was used to understand the lived experiences of a purposive sample of formal caregivers in caring for people with intellectual disability and dementia. Discussions from 14 individual interviews generated data were analysed. Four key super-ordinate themes emerged which were: (1) recognising early indicators and diagnosis, (2) post diagnostic support, (3) coping with change and (4) need for future development. Themes reflected the experiences, barriers to dementia diagnosis and provide a valuable insight into the challenges faced by formal caregivers in providing aged care services.

Suicide and Suicidality in Children and Adolescents with Chronic Skin Disorders: A Systematic Review.

Suicide in young children is rare; the incidence increases towards the end of adolescence. Skin disorders confer a high prevalence of psychiatric and psycho-logical comorbidities. However, published research on suicidal behaviour in adolescents and children with skin disorders is sparse. The aim of this study was to identify the prevalence of suicidal behaviour in children and adolescents under 18 years of age with chronic skin disorders and associated contributing risk factors. MEDLINE, PsycINFO, EMBASE, CINAHL and Cochrane databases were searched from inception to October 2020 for suicide or suicide attempts in patients under 18 years old with chronic skin disorders. The study protocol was logged on PROSPERO (CRD42020083528). Returned texts were reviewed independently by 2 authors. Bias was assessed according to Joanna Briggs Institute criteria. Five studies met the inclusion criteria; 4 cross-sectional surveys and 1 retrospective matched-cohort study. A total of 31,641 patients with acne, atopic dermatitis, body dysmorphic disorder or psoriasis were identified. Prevalence of suicidal ideation was 0.45% (psoriasis) to 67% (body dysmorphic disorder). The prevalence of suicidal attempts ranged from 0.08% (psoriasis) to 21.9% (acne). Patients with acne or atopic dermatitis had significantly increased odds ratio for suicidal attempts. Meta-analysis could not be performed owing to the heterogeneity and sparsity of data. Suicidal risk in skin disorders amongst adolescents and children under the age of 18 years old is broad and complex. The suicidal risk remained after adjusting for depression, suggestive of an alternative mechanism.

British Association of Dermatologists guidelines for the management of adults with delusional infestation 2022.

The overall objective of the guideline is to provide up-to-date, evidence-based recommendations for the management of delusional infestation (DI) in adults. Linked Comment: I. Coulson. Br J Dermatol 2022; 187:457.

Why sexual health clinics are important in the 2020s.

To make services more accessible, acceptable and affordable, sexual health service delivery models have embraced innovation, technology, outreach and decentralisation. In particular, some routine high-volume services, like asymptomatic testing for sexually transmitted infections (STIs), can be delivered in general practice, online or in non-clinical settings. On the surface, sexual health clinics, like hospitals or other primary care clinics, might appear to be operating on a model that has not changed significantly in recent times. However, globally sexual healthcare needs are rising both in volume and complexity, not all of which can be adequately met through decentralised care. Sexual health clinics themselves are the site of considerable innovation. The importance of sexual health clinics in the diagnosis and treatment of symptomatic STIs is likely to increase with the increasing burden of disease, the complexity of treatment guidelines and the emergence of new infections. Services essential to patient health such as immediate or complex clinical care, partner notification and safeguarding, and activities essential to the health system like research, training and supervision require expertise to be located where it can be accessed and maintained at reasonable cost. We do not know whether increasing some services outside existing models can safely compensate for reducing other services inside them.

Δ9-tetrahydrocannabinol and 2-AG decreases neurite outgrowth and differentially affects ERK1/2 and Akt signaling in hiPSC-derived cortical neurons.

Endocannabinoids regulate different aspects of neurodevelopment. In utero exposure to the exogenous psychoactive cannabinoid Δ9-tetrahydrocannabinol (Δ9-THC), has been linked with abnormal cortical development in animal models. However, much less is known about the actions of endocannabinoids in human neurons. Here we investigated the effect of the endocannabinoid 2-arachidonoyl glycerol (2AG) and Δ9-THC on the development of neuronal morphology and activation of signaling kinases, in cortical neurons derived from human induced pluripotent stem cells (hiPSCs). Our data indicate that the cannabinoid type 1 receptor (CB1R), but not the cannabinoid 2 receptor (CB2R), GPR55 or TRPV1 receptors, is expressed in young, immature hiPSC-derived cortical neurons. Consistent with previous reports, 2AG and Δ9-THC negatively regulated neurite outgrowth. Interestingly, acute exposure to both 2AG and Δ9-THC inhibited phosphorylation of serine/threonine kinase extracellular signal-regulated protein kinases (ERK1/2), whereas Δ9-THC also reduced phosphorylation of Akt (aka PKB). Moreover, the CB1R inverse agonist SR 141716A attenuated the decrease in neurite outgrowth and ERK1/2 phosphorylation induced by 2AG and Δ9-THC. Taken together, our data suggest that hiPSC-derived cortical neurons express CB1Rs and are responsive to exogenous cannabinoids. Thus, hiPSC-neurons may represent a good cellular model for investigating the role of the endocannabinoid system in regulating cellular processes in developing human neurons.

Strategies and resources for nurse leaders to use to lead with empathy and prudence so they understand and address sources of anxiety among nurses practising in the era of COVID-19.

AIMS: Identify strategies and resources for nurse leaders to use to lead with empathy and prudence to improve quality of care and to ease the psychological toll on nurses caring for patients with COVID-19. BACKGROUND: In a 2020 report, clinicians caring for patients during the COVID-19 pandemic said their healthcare leaders needed to: 'hear me, protect me, prepare me, support me, and care for me'. These words provide an action plan for nurse leaders to communicate, educate and support nurses to practice competently and safely (physically and mentally) in the context of COVID-19. DESIGN: Discursive paper. METHOD: Identification and inclusion of relevant international evidence with clinical discussion. FINDINGS: Nurse leaders can mobilise system and individual level strategies and resources to support nurses to manage pandemic-related issues including: anxiety due to the risk of infection, supporting anxious children, mitigating moral injury; providing safe and quality nursing care for patients with COVID-19 and end-of-life care as needed; supporting relatives who cannot be present with a dying relative and care for grieving relatives and colleagues. We categorise a selection of evidence-based, online sources providing current COVID-19 information, practice updates and resources to develop personalised self-care plans to ease anxiety and support renewal and resilience. CONCLUSIONS: Nurse leaders must ensure adequate PPE supply, upskill nurses to provide safe, quality care for patients with COVID-19 and promote restorative self-care plans. RELEVANCE TO CLINICAL PRACTICE: The strategic actions nurse leaders take today can positively impact nurses' well-being and ability to provide safe and quality care for patients in the context of COVID-19.

Ultrastructural defects in stereocilia and tectorial membrane in aging mouse and human cochleae.

The aging cochlea is subjected to a number of pathological changes to play a role in the onset of age-related hearing loss (ARHL). Although ARHL has often been thought of as the result of the loss of hair cells, it is in fact a disorder with a complex etiology, arising from the changes to both the organ of Corti and its supporting structures. In this study, we examine two aging pathologies that have not been studied in detail despite their apparent prevalence; the fusion, elongation, and engulfment of cochlear inner hair cell stereocilia, and the changes that occur to the tectorial membrane (TM), a structure overlying the organ of Corti that modulates its physical properties in response to sound. Our work demonstrates that similar pathological changes occur in these two structures in the aging cochleae of both mice and humans, examines the ultrastructural changes that underlie stereocilial fusion, and identifies the lost TM components that lead to changes in membrane structure. We place these changes into the context of the wider pathology of the aging cochlea, and identify how they may be important in particular for understanding the more subtle hearing pathologies that precede auditory threshold loss in ARHL.

Disruption of SorCS2 reveals differences in the regulation of stereociliary bundle formation between hair cell types in the inner ear.

Behavioural anomalies suggesting an inner ear disorder were observed in a colony of transgenic mice. Affected animals were profoundly deaf. Severe hair bundle defects were identified in all outer and inner hair cells (OHC, IHC) in the cochlea and in hair cells of vestibular macular organs, but hair cells in cristae were essentially unaffected. Evidence suggested the disorder was likely due to gene disruption by a randomly inserted transgene construct. Whole-genome sequencing identified interruption of the SorCS2 (Sortilin-related VPS-10 domain containing protein) locus. Real-time-qPCR demonstrated disrupted expression of SorCS2 RNA in cochlear tissue from affected mice and this was confirmed by SorCS2 immuno-labelling. In all affected hair cells, stereocilia were shorter than normal, but abnormalities of bundle morphology and organisation differed between hair cell types. Bundles on OHC were grossly misshapen with significantly fewer stereocilia than normal. However, stereocilia were organised in rows of increasing height. Bundles on IHC contained significantly more stereocilia than normal with some longer stereocilia towards the centre, or with minimal height differentials. In early postnatal mice, kinocilia (primary cilia) of IHC and of OHC were initially located towards the lateral edge of the hair cell surface but often became surrounded by stereocilia as bundle shape and apical surface contour changed. In macular organs the kinocilium was positioned in the centre of the cell surface throughout maturation. There was disruption of the signalling pathway controlling intrinsic hair cell apical asymmetry. LGN and Gαi3 were largely absent, and atypical Protein Kinase C (aPKC) lost its asymmetric distribution. The results suggest that SorCS2 plays a role upstream of the intrinsic polarity pathway and that there are differences between hair cell types in the deployment of the machinery that generates a precisely organised hair bundle.

Treatments for primary delusional infestation.

BACKGROUND: Primary delusional infestation (DI) is a primary psychiatric disorder characterised by delusions and abnormal tactile sensations. The pathophysiology is undecided and treatment includes both pharmacological and non-pharmacological options. There is currently no Cochrane Review of the treatments used. Primary DI is a diagnosis often encountered by both dermatologists and psychiatrists, with a large associated disease burden. OBJECTIVES: To evaluate the effectiveness of different treatments in primary delusional infestation (DI). SEARCH METHODS: On 24 December 2014 and 19 March 2019, we searched the Cochrane Schizophrenia Group's Study-Based Register of Trials including registries of clinical trials. SELECTION CRITERIA: Randomised controlled trials involving the treatment of adults with primary DI. DATA COLLECTION AND ANALYSIS: Two review authors independently screened and assessed studies for inclusion using pre-specified inclusion criteria. MAIN RESULTS: We did not identify any studies for inclusion. AUTHORS' CONCLUSIONS: Currently there is no evidence from RCTs available to compare treatment of primary DI with placebo. We cannot, therefore, make any conclusions regarding the effects of treatments (pharmacological or non-pharmacological) for primary DI. This lack of evidence for treatment of primary DI has implications for research and practice. Robust randomised trials are indicated.

A patient with delusional infestation by proxy: Issues for vulnerable adults.

A 57-year-old Caucasian female presented to our clinic with her 23-year-old son, who was profoundly autistic. Our patient was convinced that her son had an infestation with fibers and believed that he had developed the condition as a young child. She described the symptoms of the infestation in her son's skin on his behalf, as he was not able to communicate. She identified dietary factors as a causative factor and wanted them removed from his diet. Her son had seborrhoeic dermatitis on examination, with no evidence of an infestation. Our patient later identified that fibers were coming out of her skin also. Her medical history included a road traffic accident resulting in back pain. Blood, urine, and microbiological investigations were normal. She was diagnosed with delusional infestation (DI) by proxy, and was started on risperidone. We present an interesting case of a patient with DI by proxy, in which the delusional beliefs of the mother have been projected onto her son. Issues of safeguarding vulnerable adults are raised in such cases, suggesting the crucial role of the physician in ensuring patient safety. DI by proxy has been reported in patients with children and animals, but we believe this is the first report of DI by proxy involving a vulnerable adult.

Absence of plastin 1 causes abnormal maintenance of hair cell stereocilia and a moderate form of hearing loss in mice.

Hearing relies on the mechanosensory inner and outer hair cells (OHCs) of the organ of Corti, which convert mechanical deflections of their actin-rich stereociliary bundles into electrochemical signals. Several actin-associated proteins are essential for stereocilia formation and maintenance, and their absence leads to deafness. One of the most abundant actin-bundling proteins of stereocilia is plastin 1, but its function has never been directly assessed. Here, we found that plastin 1 knock-out (Pls1 KO) mice have a moderate and progressive form of hearing loss across all frequencies. Auditory hair cells developed normally in Pls1 KO, but in young adult animals, the stereocilia of inner hair cells were reduced in width and length. The stereocilia of OHCs were comparatively less affected; however, they also showed signs of degeneration in ageing mice. The hair bundle stiffness and the acquisition of the electrophysiological properties of hair cells were unaffected by the absence of plastin 1, except for a significant change in the adaptation properties, but not the size of the mechanoelectrical transducer currents. These results show that in contrast to other actin-bundling proteins such as espin, harmonin or Eps8, plastin 1 is dispensable for the initial formation of stereocilia. However, the progressive hearing loss and morphological defects of hair cells in adult Pls1 KO mice point at a specific role for plastin 1 in the preservation of adult stereocilia and optimal hearing. Hence, mutations in the human PLS1 gene may be associated with relatively mild and progressive forms of hearing loss.

Exploring the Psychological Profile of Patients with Delusional Infestation.

Delusional infestation (DI) is an uncommon psychiatric disorder in which patients present with the false and fixed belief (i.e. a delusion) that their skin and/or their environment is infested despite objective evidence to the contrary. Within psychodermatology specialist clinics there is a high rate of DI referrals. What is not known is the level of psychiatric and psychological co-morbidities associated with DI and whether psychiatric or psychological assessment would be warranted. One-hundred and thirty-eight adult patients with DI attending an outpatient psychodermatology clinic were given 3 standardised questionnaires. The results showed that 81% had a poor quality of life; 52% with anxiety, 41.6% with depression and 49% with appearance-related concerns. This study indicates high levels of psychiatric and psychological disorders in DI which require assessment and appropriate intervention.

Quality of care provided in two Scottish rural community maternity units: a retrospective case review.

BACKGROUND: Women in Scotland with uncomplicated pregnancies are encouraged by professional bodies and national guidelines to access community based models of midwife-led care for their labour and birth. The evidence base for these guidelines relates to comparisons of predominantly urban birth settings in England. There appears to be little evidence available about the quality of the care during the antenatal, birth and post birth periods available for women within the Scottish Community Maternity Unit (CMU) model. The research aim was to explore the safety and effectiveness of the maternity services provided at two rural Community Maternity Units in Scotland, both 40 miles by main road access from a tertiary obstetric unit. METHODS: Following appropriate NHS and University ethical approval, an anonymous retrospective review of consecutive maternity records for all women who accessed care at the CMUs over a 12 month period (June 2011 to May 2012) was undertaken in 2013 -14. Data was extracted using variables chosen to provide a description of the socio-demographics of the cohort and the process and outcomes of the care provided. Data were analysed using descriptive statistics. RESULTS: Regarding effectiveness, the correct care pathway was allocated to 97.5% of women, early access to antenatal care achieved by 95.7% of women, 94.8% of women at one CMU received continuity of carer and 78.6% of those clinically eligible accessed care in labour. 11.9% were appropriately transferred to obstetrician-led care antenatally and 16.9% were transferred in labour. All women received one-to one care in labour and 67.1% of babies born at the CMUs were breastfed at birth. Regarding safety, severe morbidity for women was rare, perineal trauma of 3rd degree tear occurred for 0.3% of women and 1.0% experienced an episiotomy. Severe post partum haemorrhage occurred for 0.3% of women. Babies admitted to the Neonatal unit were discharged within 48 hrs. CONCLUSION: These findings support the recommendations of professional bodies and national guidelines. Maternity service provision at rural CMUs achieved a consistently high standard of safety and effectiveness when measured against national standards and international evidence.

Exploring the concept of "caring cultures": A critical examination of the conceptual, methodological and validity issues with the "caring cultures" construct.

AIMS AND OBJECTIVES: To critically examine the conceptual, methodological and validity issues with the "caring cultures" construct. BACKGROUND: Post the Francis Report, "caring cultures" and alternative terminology such as "culture/s of care/caring/compassionate care" have gained prominence in the literature, especially within a UK policy context. However, in order to understand the value these "caring cultures" hold in terms of clinical practice, the concept itself first needs to be understood. DESIGN: A discussion and critical examination of the concept of "caring cultures" and associated terminology. METHODS: Grey literature, database, library and reference list searches were conducted. RESULTS: It is implied that "caring cultures" influence patient care. However, evidence which verifies this assertion is limited. In this article, the concept of "caring cultures" is deconstructed and its validity explored. An alternative to "caring cultures" is proposed in terms of research, whereby the concept of culture is instead explored in detail, on a microsystem level, using appropriate methodology. CONCLUSION: The concept of "caring cultures", although attractive in terms of its apparent simplicity, is not considered the most useful nor appropriate phrases in terms of advancing research. Instead, research which examines the established concept of "culture" in relation to outcomes such as patient care, doing so with an appropriate methodology, is viewed as a more suitable alternative. RELEVANCE TO CLINICAL PRACTICE: Clarifying concepts and terminology relating to "caring cultures" is essential for research to progress and the impact of culture on clinical practice to be better understood.

Aide-memoire diaries in longitudinal research.

BACKGROUND: Diaries can be used as contemporaneous tools for gathering data, frequently in combination with interviews. However, diaries can also be used by study participants as personal records to improve their recall during interviews, without having to be collected for analysis. AIM: To discuss the use of diaries as aide-memoires by participants in a study exploring women's views and experiences of the quality of maternity care they received in community maternity units in Scotland. DISCUSSION: The use of aide-memoire diaries was an effective way to enhance the quality of data collected during interviews. CONCLUSION: This use of diaries would help longitudinal research in different contexts, including long-term care, as an aide-memoire to help participants recall their experiences throughout their care. IMPLICATIONS FOR PRACTICE: Aide-memoire diaries provide a private platform for contemporaneously and honestly recording participants' reflective accounts throughout their experiences of care.

Psychodermatology in Clinical Practice: Main Principles.

Psychodermatology is a newer and emerging subspecialty of dermatology, which bridges psychiatry, psychology, paediatrics and dermatology. It has become increasingly recognised that the best outcomes for patients with psychodermatological disease is via a multidisciplinary psychodermatology team. The exact configuration of the multidisciplinary team is, to some extent, determined by local expertise. In addition there is a growing body of evidence that it is much more cost effective to manage patients with psychodermatological disease in dedicated psychodermatology clinics. Even so, despite this evidence, and the demand from patients (and patient advocacy groups), the delivery and establishment of psychodermatology services is very sporadic globally. Clinical and academic expertise in psychodermatology is emerging in dermatology and other (often peer-reviewed) literature. Organisations such as the European Society for Dermatology and Psychiatry champion clinical and academic advances in psychodermatology, whist also enabling training of health care professionals in psychodermatology. Emiliano Panconesi, to whom this supplement is dedicated, was at the forefront of psychodermatology research and was a founding member of ESDaP.

Connexin30-mediated intercellular communication plays an essential role in epithelial repair in the cochlea.

A role for connexin (Cx)30 in epithelial repair following injury was examined in the organ of Corti, the sensory epithelium of the cochlea. In this tissue, lesions caused by loss of the sensory hair cells are closed by the supporting cells that surround each one. Gap junctions in which Cx30 is the predominant connexin are large and numerous between supporting cells. In mice carrying a deletion in the gene (Gjb6) that encodes Cx30, the size and number of gap junction plaques, and the extent of dye transfer, between supporting cells was greatly reduced compared with normal animals. This corresponded with unique peculiarities of the lesion closure events during the progressive hair cell loss that occurs in these animals in comparison with other models of hair cell loss, whether acquired or as a result of a mutation. Only one, rather than all, of the supporting cells that contacted an individual dying hair closed the lesion, indicating disturbance of the co-ordination of cellular responses. The cell shape changes that the supporting cells normally undergo during repair of the organ of Corti did not occur. Also, there was disruption of the migratory activities that normally lead to the replacement of a columnar epithelium with a squamous-like one. These observations demonstrate a role for Cx30 and intercellular communication in regulating repair responses in an epithelial tissue.

Children with a mother with delusional infestation--implications for child protection and management.

Delusional infestation (DI) is a reportedly uncommon psychocutaneous condition in which an individual holds a fixed, false belief of pathogenic infestation of the skin or body contrary to medical evidence. There are reports of such a delusion being shared with family members or close friends (folie a deux/famillie), but the implications of DI induced in children have not been discussed. We describe the case of a mother with DI whose children subsequently shared her belief. Implications for child protection and management are explored.

Selective ablation of pillar and deiters' cells severely affects cochlear postnatal development and hearing in mice.

Mammalian auditory hair cells (HCs) are inserted into a well structured environment of supporting cells (SCs) and acellular matrices. It has been proposed that when HCs are irreversibly damaged by noise or ototoxic drugs, surrounding SCs seal the epithelial surface and likely extend the survival of auditory neurons. Because SCs are more resistant to damage than HCs, the effects of primary SC loss on HC survival and hearing have received little attention. We used the Cre/loxP system in mice to specifically ablate pillar cells (PCs) and Deiters' cells (DCs). In Prox1CreER(T2)+/-;Rosa26(DTA/+) (Prox1DTA) mice, Cre-estrogen receptor (CreER) expression is driven by the endogenous Prox1 promoter and, in presence of tamoxifen, removes a stop codon in the Rosa26(DTA/+) allele and induces diphtheria toxin fragment A (DTA) expression. DTA produces cell-autonomous apoptosis. Prox1DTA mice injected with tamoxifen at postnatal days 0 (P0) and P1 show significant DC and outer PC loss at P2-P4, that reaches ∼70% by 1 month. Outer HC loss follows at P14 and is almost complete at 1 month, while inner HCs remain intact. Neural innervation to the outer HCs is disrupted in Prox1DTA mice and auditory brainstem response thresholds in adults are 40-50 dB higher than in controls. The hearing deficit correlates with loss of cochlear amplification. Remarkably, in Prox1DTA mice, the auditory epithelium preserves the ability to seal the reticular lamina and spiral ganglion neuron counts are normal, a key requirement for cochlear implant success. In addition, our results show that cochlear SC pools should be appropriately replenished during HC regeneration strategies.

Pituitary adenylate cyclase-activating polypeptide (PACAP) inhibits the slow afterhyperpolarizing current sIAHP in CA1 pyramidal neurons by activating multiple signaling pathways.

The slow afterhyperpolarizing current (sIAHP ) is a calcium-dependent potassium current that underlies the late phase of spike frequency adaptation in hippocampal and neocortical neurons. sIAHP is a well-known target of modulation by several neurotransmitters acting via the cyclic AMP (cAMP) and protein kinase A (PKA)-dependent pathway. The neuropeptide pituitary adenylate cyclase activating peptide (PACAP) and its receptors are present in the hippocampal formation. In this study we have investigated the effect of PACAP on the sIAHP and the signal transduction pathway used to modulate intrinsic excitability of hippocampal pyramidal neurons. We show that PACAP inhibits the sIAHP , resulting in a decrease of spike frequency adaptation, in rat CA1 pyramidal cells. The suppression of sIAHP by PACAP is mediated by PAC1 and VPAC1 receptors. Inhibition of PKA reduced the effect of PACAP on sIAHP, suggesting that PACAP exerts part of its inhibitory effect on sIAHP by increasing cAMP and activating PKA. The suppression of sIAHP by PACAP was also strongly hindered by the inhibition of p38 MAP kinase (p38 MAPK). Concomitant inhibition of PKA and p38 MAPK indicates that these two kinases act in a sequential manner in the same pathway leading to the suppression of sIAHP. Conversely, protein kinase C is not part of the signal transduction pathway used by PACAP to inhibit sIAHP in CA1 neurons. Our results show that PACAP enhances the excitability of CA1 pyramidal neurons by inhibiting the sIAHP through the activation of multiple signaling pathways, most prominently cAMP/PKA and p38 MAPK. Our findings disclose a novel modulatory action of p38 MAPK on intrinsic excitability and the sIAHP, underscoring the role of this current as a neuromodulatory hub regulated by multiple protein kinases in cortical neurons.