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The cellular identity of pancreatic polypeptide (Ppy)-expressing γ-cells, one of the rarest pancreatic islet cell-type, remains elusive. Within islets, glucagon and somatostatin, released respectively from α- and δ-cells, modulate the secretion of insulin by β-cells. Dysregulation of insulin production raises blood glucose levels, leading to diabetes onset. Here, we present the genetic signature of human and mouse γ-cells. Using different approaches, we identified a set of genes and pathways defining their functional identity. We found that the γ-cell population is heterogeneous, with subsets of cells producing another hormone in addition to Ppy. These bihormonal cells share identity markers typical of the other islet cell-types. In mice, Ppy gene inactivation or conditional γ-cell ablation did not alter glycemia nor body weight. Interestingly, upon β-cell injury induction, γ-cells exhibited gene expression changes and some of them engaged insulin production, like α- and δ-cells. In conclusion, we provide a comprehensive characterization of γ-cells and highlight their plasticity and therapeutic potential. The cellular identity and function of the pancreatic polypeptide (Ppy)-producing γ-cells are incompletely understood. Here the authors show that these cells are heterogeneous and display adaptive plasticity to engage in insulin production following β-cell injury, but loss of the Ppy gene or γ-cells in mice does not affect weight or glycemia under basal conditions.

(1) Receiving an oncological diagnosis involves countless physical and mental challenges for those who become ill. In addition to this, developmental activities are put on hold when a parent becomes ill, significantly impairing children’s normal growth and development. The purpose of this review is to highlight the psychological impact of cancer on children, with particular attention to differences based on the age of the child and the stage of the parent’s disease. (2) Articles published on PubMed up until October 2023 were searched. Qualitative and quantitative studies were included in this review after an evaluation of the full text. The study selection process was undertaken by two researchers, and articles for which there was unanimous agreement between researchers were included in the review. (3) Children’s psycho-emotional responses differ based on their age and the disease stage. In general, good communication and a supportive family environment that understands everyone’s needs seem to constitute important protective factors that favor the adaptation of the entire family to the disease. (4) The family, as an evolutionary system, finds itself facing phases typical of development. Knowing the variables that intervene in the process of adaptation to the disease will allow us to design specific and differentiated clinical interventions based on the needs of not only the patient but also the entire family.

Breast cancer is a potential traumatic event associated with psychological symptoms, but few studies have analysed its impact in under-50 women. Emotional processing is a successful function in integrating traumatic experiences. This work analysed the relationship between emotional processing and psychological symptoms during three phases of treatment (before hospitalization, counselling after surgery and adjuvant therapy) in 50 women under the age of 50 with breast cancer. Mixed-effects models tested statistical differences among phases. There were significant differences in symptoms during the treatments: the levels of anxiety decrease from T1 to T3 (0.046), while those of hostility increase (<0.001). Emotional processing is a strong predictor of all symptoms. Clinical implications are discussed.

Background Noonan Syndrome (NS) is a rare multisystemic disorder with heterogeneous phenotypic manifestations. The aim of this study was to analyse rates of survival, hospitalisation, surgeries and prescriptions in children born with NS in the first 10 years of life. Methods This is a multi-centre population-based cohort study. Data on 175 liveborn children diagnosed with NS from 11 EUROCAT congenital anomaly registries were linked to healthcare databases. Each registry applied a common data model to standardise data and run common syntax scripts to produce aggregated results which were pooled using random effects meta-analyses. Results Mortality rates were high in the first year of life with 5.4% (95%CI 1.5%-10.1%) of children dying before the age of 1 year with a further 2% dying up to age 5. In the first year, 87.9% (95%CI 75.3%-94.3%) of children were hospitalized and the median Length Of hospital Stay (LOS) was 15.3 days (95%CI 9.3–21.2). After the first year, the proportion of children hospitalized remained higher than 70%, but the LOS decreased to 1.3 days per year. In the first 5 years, 65.2% of children underwent a median of two surgical procedures. The median age at first surgery was 29 weeks. The proportion of children with an antibiotic prescription increased from 53.6% at age 1 to 62.4% yearly until 4 years of age. Conclusions Children with NS have high mortality and morbidity not only in the first year of life but also up to five years of age. This study evaluated the health burden of NS and provided information for clinicians, health-care providers and families.

Purpose Since long-term survivorship is now a reality for an increasingly number of people with a history of cancer, understanding their quality of life (QoL) can inform health care policy as well as help supporting individual patients. This study was aimed to quantify QoL of this specific population in comparison with data provided for both the general population and cancer patients and to assess QoL association with several sociodemographic, clinical, and psychological variables. Methods Three hundred fourteen Italian long-term cancer survivors (people who have been free from cancer and cancer treatments for at least 5 years) completed a battery of questionnaires including the SF12 for QoL assessment. Results Both physical and mental functioning were higher than those among Italian cancer patients but lower than those of the Italian general population ( p  < .001). Poorer QoL (physical and mental functioning) was associated more often with psychological conditions (as anxiety and depression) than with sociodemographic and cancer-related variables. Conclusions These data support an ongoing specific interest in the QoL of long-term cancer survivors and suggest the need for further study of multidimensional functioning in this population.

Several studies have highlighted the key role of chronic inflammation in breast cancer development, progression, metastasis, and therapeutic outcome. These processes are mediated through a variety of cytokines and hormones that exert their biological actions either locally or distantly via systemic circulation. Recent findings suggest that positive psychosocial experiences, including psychotherapeutic interventions and therapeutic mind-body protocols, can modulate the inflammatory response by reducing the expression of genes/proteins associated with inflammation and stress-related pathways. Our preliminary results indicate that a specific mind-body therapy (MBT-T) could induce a significant reduction of the release of different cytokines and chemokines, such as SCGFβ, SDF-1α, MCP3, GROα, LIF, and IL-18, in the sera of breast cancer patients compared to a control group, suggesting that MBT-T could represent a promising approach to improve the wellness and outcome of breast cancer patients.

Background/aimPrader-Willi syndrome (PWS) and Angelman syndrome (AS) are rare imprinting disorders caused by the aberrant expression of 15q11.2-q13 imprinted genes. Due to their rarity, data on health outcomes during infancy are limited. This EUROlinkCAT study aimed to investigate major health outcomes of children with these chromosomal disorders.MethodsData of children born in 1995–2014 and diagnosed with PWS (n=150) or AS (n=46), collected by 11 population-based congenital anomaly registries, were linked to local electronic healthcare and mortality databases and analysed.ResultsChildren with PWS had a survival rate of 94% (95% CI 89.5% to 98.7%) by 10 years of age. Nearly all children (99.5%, 95% CI 97.6% to 99.9%) with PWS required hospitalisation during the first year of life with a median length of stay of 25 days; a high proportion continued to need hospital care later in life (93.2% at 1–4 years and 79.6% at 5–9 years) with shorter stays (1.2 and 0.5 days per year, respectively). In comparison, no deaths occurred among children with AS by 10 years of age. Fewer children with AS required hospitalisation in the first year of life (59.0%, 95% CI 39.6% to 74.0%); as they grew older, the proportion admitted was 68% (95% CI 40.0% to 85.0%) at 5–9 years. Children with PWS and AS underwent first surgery at approximately 1.8 years and 2.5 years, respectively.ConclusionsThis study provides valuable evidence for improving family counselling and promoting an adequate healthcare support system.

Tumour necrosis factor (TNF)-inhibition therapy is a recommended treatment option in patients with ankylosing spondylitis who do not respond to non-steroidal anti-inflammatory drugs.2,3 In view of the effectiveness of TNF-inhibition therapy for ankylosing spondylitis, we strongly believe that it is not ethical to do a trial of the new anti-interleukin-17A monoclonal antibody secukinumab versus placebo.

Insulin-producing β-cells in pancreatic islets are regulated by systemic cues and, locally, by adjacent islet hormone-producing ‘non-β-cells’ (namely α-cells, δ-cells and γ-cells). Yet whether the non-β-cells are required for accurate insulin secretion is unclear. Here, we studied mice in which adult islets are exclusively composed of β-cells and human pseudoislets containing only primary β-cells. Mice lacking non-β-cells had optimal blood glucose regulation, enhanced glucose tolerance, insulin sensitivity and restricted body weight gain under a high-fat diet. The insulin secretion dynamics in islets composed of only β-cells was comparable to that in intact islets. Similarly, human β-cell pseudoislets retained the glucose-regulated mitochondrial respiration, insulin secretion and exendin-4 responses of entire islets. The findings indicate that non-β-cells are dispensable for blood glucose homeostasis and β-cell function. These results support efforts aimed at developing diabetes treatments by generating β-like clusters devoid of non-β-cells, such as from pluripotent stem cells differentiated in vitro or by reprograming non-β-cells into insulin producers in situ. Pancreatic islet β-cells can regulate insulin secretion in vivo, even in the absence of non-β-cells.