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Precise patterning of biomaterials has widespread applications, including drug release, degradable implants, tissue engineering, and regenerative medicine. Patterning of protein-based microstructures using UV-photolithography has been demonstrated using protein as the resist material. The Achilles heel of existing protein-based biophotoresists is the inevitable wide molecular weight distribution during the protein extraction/regeneration process, hindering their practical uses in the semiconductor industry where reliability and repeatability are paramount. A wafer-scale high resolution patterning of bio-microstructures using well-defined silk fibroin light chain as the resist material is presented showing unprecedent performances. The lithographic and etching performance of silk fibroin light chain resists are evaluated systematically and the underlying mechanisms are thoroughly discussed. The micropatterned silk structures are tested as cellular substrates for the successful spatial guidance of fetal neural stems cells seeded on the patterned substrates. The enhanced patterning resolution, the improved etch resistance, and the inherent biocompatibility of such protein-based photoresist provide new opportunities in fabricating large scale biocompatible functional microstructures.

Background Inflammatory bowel diseases [IBD] are chronic and pervasive conditions of the gastrointestinal tract with a rising incidence in paediatric and young adult populations. Evidence suggests that psychological disorders might be associated with relapse of disease activity. This study aims to evaluate the efficacy of short-term psychodynamic psychotherapy [STPP] in addition to standard medical therapy [SMT] in maintaining clinical remission in adolescents and young adults [AYA] with quiescent IBD, compared with SMT alone. Methods A two-arm, single-centre, randomised, controlled trial was conducted in 60 IBD AYA in clinical remission. Patients were randomised to receive an 8-week STPP + SMT [ n  = 30] or SMT alone [ n  = 30]. The primary outcome was the steroid-free remission rate at 52 weeks after treatment. Secondary outcomes included the overall hospitalisation rate within 52 weeks after treatment, and medication adherence obtained from patient's electronic medical records. Results Intention-to-treat analysis showed significant improvement in maintaining disease remission rates in the 8-week STPP + SMT group compared with the control one. The proportion of patients maintaining steroid-free remission at 52 weeks was higher in patients in STTP group [93.1%] compared with patients randomised to control group [64.3%; p  = 0.01]. There were no significant differences in secondary outcomes, except for depression reduction in STPP + SMT group. Conclusions An 8-week STPP intervention in addition to SMT effectively increases the steroid-free remission rates in AYA with quiescent IBD. Results do not support effects for other secondary outcomes, except for depression reduction. [ABSTRACT FROM AUTHOR]

Background: Italy initiated elexacaftor/tezacaftor/ivacaftor (ETI) for people with cystic fibrosis (pwCF) in July 2021. It has led to dramatic improvements in lung function, BMI, sweat chloride, and respiratory symptoms. However, few data are available on side effects or effects on a broad range of outcomes., Research Question: How does ETI affect mental health, cognitive processing, neuropsychological side effects, GI symptoms, and health-related quality of life over time?, Study Design and Methods: This was a prospective, "real-world" longitudinal study. Participants were recruited consecutively and evaluated at initiation (T0) and after 1 month, 3 months, and 6 months of starting treatment. Assessments included depression (nine-item Patient Health Questionnaire), anxiety (seven-item Generalized Anxiety Disorder), cognition (Symbol Digit Modalities Test), GI Symptom Tracker, and health-related quality of life (Cystic Fibrosis Questionnaire-Revised). Based on literature, an ad hoc questionnaire was developed to assess side effects: insomnia, headache, memory problems, "brain fog," and concentration problems. Following descriptive analyses, longitudinal data were analyzed by using mixed models for repeated measures, controlling for age and sex when appropriate., Results: Ninety-two consecutive pwCF (female/male, 46/46; mean age, 25.4 years) participated. FEV 1 increased initially and then remained stable. BMI also increased significantly from T0 to 6 months (P < .01). Depression improved from T0 to 1 month (P < .001); however, no changes in anxiety were found. Cognitive processing improved from T0 to subsequent assessments. Positive changes were reported on the GI Symptom Tracker for stools and adherence challenges, although no changes were found for abdominal pain and digestion. Side effects occurred in 10% to 29%, with no reduction over time; insomnia increased significantly across time. Female participants reported more side effects than male participants (ie, insomnia, headache, concentration problems, brain fog)., Interpretation: This prospective study evaluated the effects of ETI using multiple measures. Significant improvements were found in many domains; however, side effects were reported by a substantial proportion of pwCF, with no improvements over time. Female participants reported more side effects than male participants. pwCF should be followed up systematically to assess the frequency of side effects after starting this new modulator., Competing Interests: Financial/Nonfinancial Disclosures The authors have reported to CHEST the following: S. G. has received consulting fees from Vertex Pharmaceuticals. A. Q. has received consulting fees from Vertex Pharmaceuticals. F. Majo has received consulting fees from Vertex Pharmaceuticals. None declared (F. B., G. R. P., F. Milo, L. C., E. M., F. A., S. B., R. C., A. G. F., P. T.)., (Copyright © 2023 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Background: Elexacaftor-tezacaftor-ivacaftor (ETI) represents a significant step forward in cystic fibrosis (CF) care and could change the course of CF lung disease and quality of life for many people with CF (PwCF). However, several PwCF cannot benefit from these modulators because their rare mutations are not eligible for treatment. This study aimed to investigate the lived experiences of PwCF who are not eligible for ETI., Methods: Data were collected through semi-structured interviews with 13 individuals with CF that were not eligible for ETI. Thematic analysis was used to identify the key themes of their experiences., Results: Two main themes and six subthemes were identified. The first main theme (being deemed ineligible for ETI) had four subthemes (disappointment, information, happiness, and concerns). The second main theme (coping with a life without ETI) had two subthemes (lack of hope and continued hope)., Conclusions: PwCF who are not eligible for ETI experience intense disappointment and conflicting emotions that can influence their decision-making linked to diminishing/renewal hope. Integrated care, including mental health monitoring programs, should be provided to these patients to aid them in overcoming their disappointment and to improve their coping., Competing Interests: Declaration of Competing Interest The Authors declare that there is no conflict of interest., (Copyright © 2022 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

Introduction: Individuals with chronic respiratory diseases and caregivers are at higher risk for depression and anxiety. Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are both rare genetic diseases, characterized by recurrent respiratory infections. This study compared depression and anxiety in people with PCD (pwPCD) and CF (pwCF), and caregivers, using the screening tools recommended in the CF guidelines., Methods: Patient Health Questionnaire (PHQ-9) and Generalized Anxiety Disorder (GAD-7) were administered to a PCD and CF sample. Given that PCD is extremely rare, they were matched on age and sex to pwCF at a 1:2 ratio. Similar procedures were performed with parents., Results: A total of 63 patients and 129 caregivers participated: 21 pwPCD and 42 pwCF (ages 12-34 years) plus 43 caregivers of pwPCD and 86 caregivers of pwCF. A high percentage of patients scored above the cut-off for depression (PCD: 33%; CF: 43%) and anxiety (PCD and CF both: 43%), mostly mild. Similarly, a high percentage of caregivers scored above the cut-off for depression (PCD: 42-54%; CF: 45-46%) and anxiety (PCD: 47-54%; CF: 39-56%). Suicidal ideation was endorsed by 9.5% of pwPCD, 20% of mothers and 10% of fathers and 5% of pwCF, 3% of mothers, but no fathers., Conclusion: A large percentage of patients and caregivers reported elevated psychological distress and suicidal ideation. Addressing psychological symptoms is critical given they are associated with poor adherence, missed clinic visits, increased inflammation and worse quality of life. Mental health screening and treatment should be integrated into PCD care., Competing Interests: Declaration of competing interest There are no conflicts of interest., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Background: The spread of COVID-19 was associated with increased stress and new mental health concerns for people with cystic fibrosis (pwCF), already at increased risk for depression and anxiety. This study assessed stress and mental health in adolescents and young adults with CF 1 year from when the pandemic began., Methods: Sixty-six pwCF (mean age = 24; range 14-36) completed a new measure of the impact of COVID-19 (COVID-19 Exposure and Family Impact Scale-Adolescence and Young Adult; CEFIS-AYA); the Patient Health Questionnaire-9, and the Generalized Anxiety Disorder-7. The Italian translation of the CEFIS-AYA was performed., Results: On the CEFIS-AYA, the mean Exposure score was 5.2 (SD = 2.6) out of 28. The mean Impact score was 1.8 (SD = 0.7; negative valence &gt; 2.5). Individuals were more sedentary and undertaking less exercise. Average stress rating was 5.9 (SD = 2), indicating moderate stress. No significant differences were found between those who did (N = 12) and who did not have a COVID infection (N = 54). A high percentage of participants scored above the clinical cut-off for depression (45%) and anxiety (41%), with a low proportion reporting moderate-severe symptomatology., Conclusion: After 1 year, the pandemic was having a less significant impact on patients' daily lives. Sedentary activity and reduced exercise were common. Despite expectations that this group was particularly vulnerable, depression and anxiety scores were similar to the rates described for this population before the pandemic. Overall, these results suggested that pwCF are highly resilient and nearly 1 year after the onset of COVID-19, have returned to similar daily activities and emotional health., (© 2022 Wiley Periodicals LLC.)

Aims: To synthesize qualitative studies of subjective experience of pregnancy in people with cystic fibrosis., Design: Thematic synthesis of qualitative studies., Data Sources: We searched PUBMED, CINAHL, EMBASE. PsicINFO and Social Sciences Citation Index for qualitative studies published in English, which reported on pregnancy in people with cystic fibrosis. Searches were carried out in March 2021, updated in June 2022., Review Methods: Studies that met the inclusion criteria were appraised for quality using the Critical Appraisal Skills Programme (CASP) for qualitative research. Data were extracted from the studies, analyse and synthesise using thematic synthesis approach., Results: Thirteen studies were included in the review and 'Walking on a wire' framework was conceptualized. We found three analytical themes: (1) desire for information related to pregnancy in cystic fibrosis, (2) factors at play in decision-making related to pregnancy for people with cystic fibrosis and (3) pregnancy experience and eight descriptive themes: (1) information topics, (2) CF healthcare team/PwCF as a provider of reproductive health information, (3) information timing, (4) barriers to information delivery, (5) barriers to decision-making and stresses relating to the process of trying to conceive, (6) environmental factors, (7) coping with challenges, and (8) moving towards parenthood., Conclusion: For people with cystic fibrosis, pregnancy is a complex pathway: the amount of knowledge about cystic fibrosis and sexual and reproductive health, barriers to pregnancy and environmental factors provides the background to decision-making. Moreover, coping with pregnancy is a challenging experience, where they have to mediate between the physical and emotional implications of planning a pregnancy and the limitations imposed by the chronic health conditions., Impact: Understanding the psychological experiences of people with cystic fibrosis (PwCF) will improve future research and practice. Education about sexual and reproductive health and psychosocial care programmes are necessary to help PwCF deal with the challenges related to pregnancy. Hospitals should enhance the development of specific programmes to promote the well-being of individuals with CF who are planning a pregnancy., (© 2022 John Wiley & Sons Ltd.)

Background: Delirium, bed immobilization, and heavy sedation are among the major contributors of pediatric post-intensive care syndrome. Recently, the Society of Critical Care Medicine has proposed the implementation of daily interventions to minimize the incidence of these morbidities and optimize children functional outcomes and quality of life. Unfortunately, these interventions require important clinical and economical efforts which prevent their use in many pediatric intensive care units (PICU). Aim: First, to evaluate the feasibility and safety of a PICU bundle implementation prioritizing delirium screening and treatment, early mobilization (<72 h from PICU admission) and benzodiazepine-limited sedation in a human resource-limited PICU. Second, to evaluate the incidence of delirium and describe the early mobilization practices and sedative drugs used during the pre- and post-implementation periods. Third, to describe the barriers and adverse events encountered during early mobilization. Methods: This observational study was structured in a pre- (15th November 2019-30th June 2020) and post-implementation period (1st July 2020-31st December 2020). All patients admitted in PICU for more than 72 h during the pre and post-implementation period were included in the study. Patients were excluded if early mobilization was contraindicated. During the pre-implementation period, a rehabilitation program including delirium screening and treatment, early mobilization and benzodiazepine-sparing sedation guidelines was developed and all PICU staff trained. During the post-implementation period, delirium screening with the Connell Assessment of Pediatric Delirium scale was implemented at bedside. Early mobilization was performed using a structured tiered protocol and a new sedation protocol, limiting the use of benzodiazepine, was adopted. Results: Two hundred and twenty-five children were enrolled in the study, 137 in the pre-implementation period and 88 in the post-implementation period. Adherence to delirium screening, benzodiazepine-limited sedation and early mobilization was 90.9, 81.1, and 70.4%, respectively. Incidence of delirium was 23% in the post-implementation period. The median cumulative dose of benzodiazepines corrected for the total number of sedation days (mg/kg/sedation days) was significantly lower in the post-implementation period compared with the pre-implementation period: [0.83 (IQR: 0.53-1.31) vs. 0.74 (IQR: 0.55-1.16), p = 0.0001]. The median cumulative doses of fentanyl, remifentanil, and morphine corrected for the total number of sedation days were lower in the post-implementation period, but these differences were not significant. The median number of mobilizations per patient and the duration of each mobilization significantly increased in the post-implementation period [3.00 (IQR: 2.0-4.0) vs. 7.00 (IQR: 3.0-12.0); p = 0.004 and 4 min (IQR: 3.50-4.50) vs. 5.50 min (IQR: 5.25-6.5); p < 0.0001, respectively]. Barriers to early mobilization were: disease severity and bed rest orders (55%), lack of physicians' order (20%), lack of human resources (20%), and lack of adequate devices for patient mobilization (5%). No adverse events related to early mobilization were reported in both periods. Duration of mechanical ventilation and PICU length of stay was significantly lower in the post-implementation period as well as the occurrence of iatrogenic withdrawal syndrome. Conclusion: This study showed that the implementation of a PICU liberation bundle prioritizing delirium screening and treatment, benzodiazepine-limited sedation and early mobilization was feasible and safe even in a human resource-limited PICU. Further pediatric studies are needed to evaluate the clinical impact of delirium, benzodiazepine-limited sedation and early mobilization protocols on patients' long-term functional outcomes and on hospital finances., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Di Nardo, Boldrini, Broccati, Cancani, Satta, Stoppa, Genuini, Zampini, Perdichizzi, Bottari, Fischer, Gawronski, Bonetti, Piermarini, Recchiuti, Leone, Rossi, Tabarini, Biasucci, Villani, Raponi, Cecchetti and Choong.)

Background: Cystic fibrosis (CF) is a chronic, life-threatening condition that results in life-long morbidity and premature mortality. CF has a significant impact on healthy siblings' adaptation and well-being. Siblings of patients with a chronic disease may experience psychological difficulties, such as anxiety, depression symptoms and troubles in adaptation. This study aimed to explore the participants' experiences of growing up with a CF patient diagnosed at a paediatric age and their adaptation strategies., Methods: We enrolled eight CF siblings (adolescents and young adults) in a 6-month focus group sessions programme. Each session had two phases (psychoeducational and experiential). Transcripts were analysed and grouped through grounded theory analysis to elaborate on data-driven theory., Results: We identified 14 subthemes by which the raw data could be organized. All the subthemes were gathered together according to the axial coding process into six themes (illness, changes, communication, avoidance, normalization, and protection and care). We then grouped the six themes into two main themes ('Growing up with a CF brother or sister' and 'Finding the right distance') and conceptualized the grounded theory 'Keeping the right distance'. The participants described the evolving process of maintaining a balance between the illness of their brother or sister, family organization and their own needs., Conclusions: Knowing siblings' experiences and their common strategies to deal with the experience of having a brother or sister with a chronic health condition may be useful to ensure more tailored and specific interventions., (© 2021 John Wiley & Sons Ltd.)

Introduction: Coronavirus disease 2019 (COVID-19) emerged in China, leading to worldwide morbidity and mortality, including depression and anxiety. As the pandemic spread throughout Italy, mental health concerns increased for people with cystic fibrosis (pwCF), who are at greater risk. The aim was to pilot a Telehealth Psychological Support Intervention for pwCF and caregivers to reduce stress, depression, and anxiety during the lockdown in Italy in March 2020., Methods: This intervention utilized cognitive behavioral skills (e.g., cognitive reframing). Participants included 16 pwCF and 14 parents, who completed four individual telehealth sessions with a psychologist. Stress ratings, Patient Health Questionnaire and General Anxiety Disorder, PHQ-8 and GAD-7, were completed, in addition to Feasibility and Satisfaction ratings., Results: Ratings of stress significantly decreased from pre- to post-testing for pwCF (paired t(14) = -4.06, p < .01) and parents (paired t = -5.2, p < .001). A large percentage of both groups scored in the clinical range for depression and anxiety at baseline (pwCF: depression/anxiety = 71%; parents: depression = 57%; anxiety = 79%); a large proportion (20%-40%) reported moderate to severe symptomatology. Significant reductions in depression for pwCF were found (pre: M = 8.0 to post: M = 4.7; paired t(14) = 2.8, p < .05) but not anxiety (pre: M = 6.9 to post: M = 5.6, t(14) = 1.2, p = NS-non-significant). Parental depression decreased for parents (pre: M = 6.4 to post: M = 5.1, t(14) = -2.5, p < .05), but not anxiety (pre: M = 8.1 to post: M = 7.9, t(14) = -0.2, p = NS). Feasibility and Satisfaction were positive., Conclusion: This telehealth intervention yielded reductions in stress and depression for participants. Anxiety did not significantly decrease, possibly because COVID was ongoing. This feasible, satisfactory intervention was effective for improving mental health., (© 2021 Wiley Periodicals LLC.)

Living with cystic fibrosis (CF) exposes patients to the risk of developing anxiety and depression, with therapeutic compliance reduction, hospitalization increase, and quality of life and health outcomes deterioration. As pulmonary infections represent the major cause of morbidity and mortality in patients with CF, environmental contamination due to droplet dispersion and the potential transmission from environment to such patients should be prevented. Therefore, in-person contact, including group-based psychotherapy, are strongly discouraged. Nevertheless, group sharing of disease-related experiences represents a way to recover the inner resources essential for dealing with a chronic pathology. Keeping in mind the guidelines for infection control, the aim of this study is to evaluate the risk of the dissemination of microorganisms in a restricted environment where patients with CF attend group psychotherapy sessions. Five patients, selected according to their microbiological status, attended 32 group-based psychological/psychoanalytic meetings. Before each session, they were asked to observe the infection control recommendations. Microbiological environmental monitoring (MEM) has been performed to evaluate both air and surface contamination. As reported, a strict observation of standard precautions allows one to avoid environmental contamination by pathogens of the CF respiratory tract. Although infection control guidelines discourage group-based psychological/psychoanalytic interventions, our observations report the feasibility and safety of group psychotherapy when strict precautions are taken.

Background: Individuals with chronic respiratory conditions are at-risk for depression and anxiety. In the largest mental health screening study of over 6000 people with cystic fibrosis (CF) and 4000 parent caregivers (TIDES, 2014), rates of symptomatology were two to three times higher than in the general population. International guidelines recommend annual screening of mental health. This is the first study to implement these guidelines in one of the largest CF Centers in Italy., Methods: All individuals with CF, 12 and older (n = 167) and caregivers of children with CF (n = 186), birth to 18, were screened. Health outcome data were also collected (i.e FEV 1 , BMI, pulmonary exacerbations, CF-related diabetes). Prevalence data and associations between psychological symptoms and health outcomes were examined., Results: A high percentage of patients and parent caregivers reported scored above the clinical cut-off for depression and anxiety (37%-48% of adolescents, 45%-46% of adults, 49%-66% of mothers and fathers). Most scores fell in the mild range, however, over 30% were in the moderate to severe range. Elevations in depression and anxiety were correlated. Adolescents who had more pulmonary exacerbations reported higher anxiety. Adults with recent events of hemoptysis reported higher symptoms of depression., Conclusions: Symptoms of depression and anxiety were elevated in both individuals with CF and parents. Implementation of mental health screening was critical for identifying those in need of psychological interventions. These results strongly suggest that mental health should be integrated into physical health care for those with complex, chronic respiratory conditions, including COPD, PCD., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: Cystic fibrosis, like other chronic diseases, is a risk factor for the development of elevated symptoms of depression and anxiety. The objective of this study was to investigate the prevalence of anxiety and depression in Italian patients with CF and their parents., Methods: The Hospital Anxiety and Depression Scale (HADS) and Center for Epidemiologic Studies Depression Scale (CES-D) questionnaires were administered to a sample of patients and their parents recruited at the cystic fibrosis centers in Italy., Results: Elevated levels of anxiety were higher in mothers than in fathers, and also higher in female patients than in male patients. A correlation between elevated levels of anxiety/depression and geographical area also emerged. Patient anxiety (OR 2.33) and depression (OR 4.09) were significantly associated with forced expiratory volume in one second (FEV1) <40% and forced vital capacity (FVC) <80% (OR 1.60 and 1.61, respectively)., Conclusions: Cystic fibrosis increases the risk of developing anxiety and depression in female patients and in mothers. Geographical differences were observed, with higher anxiety and depression in southern Italy for parents, but not for patients. Anxiety and depression levels also depend on clinical status. Pediatr Pulmonol. 2016;51:1311-1319. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)