Navigating narcolepsy: exploring coping strategies and their association with quality of life in patients with narcolepsy type 1.

Narcolepsy type 1 (NT1) is a chronic neurological disorder characterized by symptoms such as excessive daytime sleepiness, sudden sleep episodes, disrupted nocturnal sleep, cataplexy, sleep paralysis, and hypnagogic hallucinations, which significantly impact the overall well-being and quality of life of individuals. While psychological factors have gained attention, there is limited research on the coping strategies employed by patients with NT1 and their association with quality of life. This study aimed to compare coping strategies in patients with NT1 and controls, as well as assess the relationship between coping strategies and quality of life in patients with NT1. A total of 122 individuals diagnosed with NT1 and 138 controls were enrolled in this cross-sectional study. Participants completed questionnaires assessing coping strategies and health-related quality of life. A Mann-Whitney U test was conducted to compare the use of different coping strategies by patients with NT1 and controls. Spearman's rho correlation was performed to examine the association between coping strategies and quality of life in the NT1 group. Results showed that patients with NT1 exhibited differences in the use of coping strategies compared to controls. They reported lower use of active coping, planning, instrumental, and emotional social support, and higher use of behavioral and mental disengagement. Denial and behavioral disengagement were significantly and negatively associated with quality of life. Identifying coping strategies and their association with quality of life may aid in the development of tailored interventions aimed at improving the adoption of effective coping strategies and reducing the use of maladaptive coping strategies.

Eating disorders in narcolepsy type 1: Evidence from a cross-sectional Italian study.

Narcolepsy type 1 is a chronic central disorder of hypersomnolence, and it is frequently accompanied by overweight, but the association between narcolepsy type 1 and eating disorders is controversial. Our study aims to compare patients with narcolepsy type 1 and controls on the symptomatology of eating disorders and to evaluate the association between clinical factors. This is a cross-sectional study, with consecutive recruitment of patients with narcolepsy type 1 attending the Outpatient Clinic for Narcolepsy at the IRCCS Istituto delle Scienze Neurologiche di Bologna (Italy) for routine follow-up visits. Healthy subjects from general populations were recruited as controls. Patients underwent a questionnaire-based assessment using the Eating Disorder Examination Questionnaire (EDE-Q), Binge Eating Scale (BES), Italian Night Eating Questionnaire (I-NEQ), Epworth Sleepiness Scale (ESS), and Narcolepsy Severity Scale (NSS). One hundred and thirty-eight patients with narcolepsy type 1 and 162 controls were enrolled. This study showed that individuals with narcolepsy type 1 reported higher scores on the EDE-Q, I-NEQ, and a higher body mass index (BMI) than the controls. The logistic regression analysis results, with EDE-Q positivity as a dependent variable, demonstrate a significant association with antidepressant drugs, female sex, and the use of sodium oxybate. We found an association between antidepressant drug consumption, the NSS total score, and female sex with BES positivity as the dependent variable. The logistic regression analysis for I-NEQ positivity found an association with antidepressant drug use. This study shows that patients with narcolepsy type 1 frequently present with comorbid eating disorder symptomatology, mainly night eating syndrome. Investigating the possible presence of eating disorders symptomatology through questionnaires is fundamental during the assessment of patients with narcolepsy type 1.

Suicidal ideation in female individuals with fibromyalgia and comorbid obesity: prevalence and association with clinical, pain-related, and psychological factors.

OBJECTIVE: Individuals with fibromyalgia report alarming levels of suicidal ideation, and comorbidity with other chronic health conditions such as obesity-a risk factor for suicidal ideation per se-could further complicate the clinical picture. The aim of this study is to determine, in a sample of women with fibromyalgia and comorbid obesity, the prevalence of suicidal ideation and to evaluate clinical, pain-related and psychological factors associated with suicidal ideation. METHODS: In total, 156 female individuals with fibromyalgia and obesity were recruited and completed a series of self-report measures that assessed (i) the level of pain intensity, (ii) depressive symptomatology, (iii) sleep quality, and (iv) pain catastrophizing. Suicidal ideation was evaluated by item #9 of the Beck Depression Inventory. In addition, information regarding previous suicide attempts and current opioid use was collected. RESULTS: 3n sum, 7.8% of participants reported presence of suicidal ideation. According to the results of the multiple logistic regression, depressive symptomatology, sleep quality, and pain catastrophizing were associated with the presence of suicidal ideation. DISCUSSION: The presence of suicidal ideation in our sample was significantly associated with depressive symptomatology, sleep quality, and pain catastrophizing. Our findings are the first to suggest a unique (ie, independent of depressive symptomatology, and sleep quality) association between pain catastrophizing and suicidal ideation in the context of fibromyalgia and comorbid obesity. In order to prevent and reduce suicidal ideation, these factors should be assessed and targeted in interventions for pain management. Future research should investigate the extent to which addressing depressive symptoms, sleep quality, and pain catastrophizing reduces suicidal ideation.

Phenomenology and psychiatric correlates of pseudocataplexy.

STUDY OBJECTIVES: Pseudocataplexy is a rare functional neurological disorder that mimics cataplexy, pathognomonic for narcolepsy type 1 (NT1). We describe the psychiatric comorbidity and personality traits of patients with pseudocataplexy versus NT1 cases. METHODS: The case-control observational study enrolled consecutive patients with pseudocataplexy and a control group of age-matched consecutive NT1 patients. The diagnostic work-up included a structured interview, 48-hour polysomnography, multiple sleep latency test, cataplexy provoking test, and hypocretin-1 measurement in cerebrospinal fluid.All participants were administered Beck Depression Inventory, State-Trait Anxiety Inventory, Patient Health Questionnaire-15 (PHQ-15), Personality Inventory for DSM-5 brief form, and quality of life (QoL) measurement by 36-item Short Form health survey (SF-36). RESULTS: Fifteen patients with pseudocataplexy and 30 with NT1 were included. Despite the suspicion of possible cataplexy, none of the pseudocataplexy participants fulfilled international diagnostic criteria for NT1. Pseudocataplexy patients presented higher rates of moderate state anxiety (40% vs. 10%, p=0.018), medium level of somatic symptoms, defined by PHQ-15 score >10 (66.7% vs. 16.7%, p=0.003), and a trend towards moderate-to-severe depressive symptoms (33.3% vs. 10%, p=0.054) compared to NT1. No significant differences in personality traits emerged. Pseudocataplexy patients had worse QoL profile in almost all SF-36 domains including physical (mean±SD: 37.7±9.88 vs. 51.13±7.81, p<0.001) and mental (mean±SD: 33.36±12.69 vs.42.76±11.34, p=0.02) summary scores. CONCLUSIONS: Patients with pseudocataplexy present more severe psychiatric symptoms and a lower QoL profile in comparison with patients with NT1. The severe somatoform and affection impairment in pseudocataplexy may explain the poorer QoL and should require a tailored therapeutic approach.

Association between adverse childhood experiences and suicidal behavior in schizophrenia spectrum disorders: A systematic review and meta-analysis.

Assessing and managing suicide behaviors is highly relevant to individuals with schizophrenia spectrum disorders. Our study aims to assess the association between adverse childhood experiences and suicidal behaviors in individuals with schizophrenia spectrum disorders. We included observational studies comparing the probability of suicide behaviors in adults with schizophrenia spectrum disorders exposed and unexposed to adverse childhood experiences. Odds ratio estimates were obtained by pooling data using a random-effects pairwise meta-analysis. Standardized criteria were used to assess the strength of the association of the pooled estimate. We found 21 eligible studies reporting outcomes for 6257 individuals from 11 countries. The primary outcome revealed an association between any suicidal behavior and adverse childhood experiences, which resulted "highly suggestive" according to validated Umbrella Criteria. Similarly, a positive association was confirmed for suicidal ideation and suicide attempt and for any subtype of adverse childhood experience. This meta-analysis showed that exposure to adverse childhood experiences strongly increases the probability of suicide behaviors in people with schizophrenia spectrum disorders.

Daily time use among individuals with schizophrenia spectrum disorders and unaffected controls: Results from the DiAPAson multicentric project.

OBJECTIVE: In the framework of daily time use, physical activity, and interpersonal relationships in patients with schizophrenia project, we aimed to investigate (a) within and between-group differences in daily time use of individuals with schizophrenia spectrum disorders (SSDs) and unaffected controls, stratifying them by age, sex, and employment status; (b) the associations between daily time use, the severity of psychiatric symptoms, and psychosocial functioning amongst those with SSD. METHOD: From October 2020 to October 2021, 306 outpatients and 312 individuals living in residential facilities (RFs) with SSD were recruited from 37 centers across Italy and compared on a measure of daily time use with 113 people unaffected by mental health problems. Statistical analyses included chi-squared tests, analysis of variance tests, t tests, Pearson's correlations, and nonparametric corresponding tests. RESULTS: Individuals with SSD spent significantly more time in sedentary activities, leisure, and religious activities than unaffected controls, independent of age, sex, and employment status. Unaffected controls and outpatients spent more time engaged in productive activities than patients in RFs. Among the latter group, time spent in productive activities decreased significantly after 45 years of age, while time spent in self-care activities increased. Spending time engaged in sedentary activities was associated with greater severity of psychiatric symptoms and lower levels of functioning. CONCLUSIONS AND IMPLICATIONS FOR PRACTICE: This study provides a deep understanding of how individuals with SSD spend their time and how this is associated with the severity of their mental health problems. These findings highlight the need for proactive rehabilitation programs to promote productive occupation and social inclusion of people with SSD. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

Managing leukapheresis in adult and pediatric patients eligible for chimeric antigen receptor T-cell therapy: suggestions from an Italian Expert Panel.

Chimeric antigen receptor (CAR) T-cell therapy relies on T cells engineered to target specific tumor antigens such as CD-19 in B-cell malignancies. In this setting, the commercially available products have offered a potential long-term cure for both pediatric and adult patients. Yet manufacturing CAR T cells is a cumbersome, multistep process, the success of which strictly depends on the characteristics of the starting material, i.e., lymphocyte collection yield and composition. These, in turn, might be affected by patient factors such as age, performance status, comorbidities, and previous therapies. Ideally, CAR T-cell therapies are a one-off treatment; therefore, optimization and the possible standardization of the leukapheresis procedure is critical, also in view of the novel CAR T cells currently under investigation for hematological malignancies and solid tumors. The most recent Best Practice recommendations for the management of children and adults undergoing CAR T-cell therapy provide a comprehensive guide to their use. However, their application in local practice is not straightforward and some grey areas remain. An Italian Expert Panel of apheresis specialists and hematologists from the centers authorized to administer CAR T-cell therapy took part in a detailed discussion on the following: 1) pre-apheresis patient evaluation; 2) management of the leukapheresis procedure, also in special situations represented by low lymphocyte count, peripheral blastosis, pediatric population <25 kg, and the COVID-19 outbreak; and 3) release and cryopreservation of the apheresis unit. This article presents some of the important challenges that must be faced to optimize the leukapheresis procedure and offers suggestions as to how to improve it, some of which are specific to the Italian setting.

Life satisfaction in young adults 10 or more years after hematopoietic stem cell transplantation for childhood malignant and nonmalignant diseases does not show significant impairment compared with healthy controls: a case-matched study.

Patients undergoing hematopoietic stem cell transplantation (HSCT) may experience physical and psychological deterioration that impairs their life satisfaction (LS). This study focused on LS in long-term survivors at 10 or more years after HSCT. Fifty-five patients (39 males, median age 25 years) undergoing allogeneic HSCT for childhood malignant (n = 52) or nonmalignant diseases (n = 3) were enrolled. A control group of 98 young adults (59 males, median age 24 years) was considered. A questionnaire with a modified Satisfaction Life Domain Scale was administered. We assessed such domains as education, employment, leisure time, social relationships, and perception of physical status with a 30-item questionnaire. To investigate the association between the domains and the probability of diminished LS, we performed a logistical procedure using the maximum likelihood method. Predictive factors of LS were adjusted for sociodemographic variables. In the multivariate analysis, the participant's level of LS was not significantly correlated with sociodemographic factors or with HSCT status. The same analysis showed a slight trend in favor of the control group (P = .06) for body perception. Our data suggest that the patients who undergo HSCT in childhood have no significant difference in long-term LS compared with healthy controls.

Management of poor peripheral blood stem cell mobilization: incidence, predictive factors, alternative strategies and outcome. A retrospective analysis on 2177 patients from three major Italian institutions.

CD34+ peripheral blood hematopoietic stem cells (HSC) are usually collected following mobilization therapy accomplished by using growth factors (GF) such as rHuG-CSF or rHuGM-CSF with or without chemotherapy. A target dose of yielded CD34+ is usually prescribed by the attending physician depending on different protocols, which may include single or double transplantation. HSC collection usually is performed when at least 20 CD34+ HSC/microL are detected by means of flow cytometry. A cumulative dose of at least 2 x 10(6)/Kg/bw CD34+ HSC has been considered as the threshold to allow a prompt and persistent hematopoietic recovery. Unfortunately, this goal is not achieved by the totality of patients undergoing mobilization regimen. In fact, 5-46% of patients who underwent mobilization therapy fail HSC collection due to very low peripheral blood HSC CD34+ count. Patients' characteristics, including age, sex, stage of the underlying disease (complete or partial remission), diagnosis, previously administered radio/chemotherapy regimens, time-lapse from last chemotherapy before mobilization and mobilization schedule (including dose of GF) were considered as possibly predictive of poor or failed mobilization. We performed a retrospective analysis in 2177 patients from three large Italian academic institutions to assess the incidence of poor mobilizers within our patients' series. Therefore, a patient who fails a first mobilization (and when an HLA-compatible related on unrelated donor is not available) could undergo a second attempt either with different mobilization schedule or by using different GF, such as stem cell factor, growth hormone (GH), or more recently newly introduced drugs such as AMD3100, alone or in combination with rHuG- or -rHuGM-CSF. Thus, we investigated the fate of those who failed a first mobilization and subsequently underwent a second attempt or alternative therapeutic approaches.

Extracorporeal photochemotherapy for the treatment of chronic graft-versus-host disease: trend for a possible cell dose-related effect?

Extracorporeal photochemotherapy (ECP) has been progressively introduced into the treatment of both acute and chronic graft-versus-host disease (cGvHD) over the last decade. Nevertheless, its mechanisms of action, as well as the optimal treatment schedule, have not yet been defined. We retrospectively analyzed 25 patients with cGvHD unresponsive to conventional treatments who underwent ECP from 1997 until 2005. The impact of various factors (such as treated and infused nucleated cells, time from transplantation and cGvHD onset, and time from cGvHD and ECP treatment) on the probability of no response to ECP was therefore investigated. A positive response to ECP was achieved in 80% of the patients, after a median of 19 ECP treatments (with a range of 8-38). Eighteen out of the 20 patients responsive to the treatment maintained their response for a median of 30 months. We mainly focused on clinical response and yield composition. The analysis on mononuclear cell (MNC) dose suggested that an increase of MNC dose/kg b.w. (body weight) induced a decrease in the odds of treatment failure, and that, if the MNC dose infused was at least 100 x 10(6)/kg b.w. per ECP treatment, a more positive and longer-lasting response was achieved. Moreover, the mean dose of treated and infused monocytes x 10(6)/kg b.w./ECP did not account for a clear dose-related effect. These findings may eventually result in a more patient-tailored approach to ECP. Prospective multicenter trials should be designed to investigate the real impact of MNC dose on ECP responsiveness.

Platelet activation during plasma-reduced multicomponent PLT collection: a comparison between COBE Trima and Spectra LRS turbo cell separators.

BACKGROUND: The wide diffusion of multicomponent collection in donor apheresis has led to the yielding of different components, such as plasma-reduced platelet-pheresis at high PLT concentration. We investigated whether this collection modality could induce more PLT activation compared to standard plateletpheresis. STUDY DESIGN AND METHODS: Forty-one plateletpheresis collections (20 Trima and 21 Spectra LRS Turbo v.7.0, COBE) were evaluated. Donor, procedure, and product data were recorded. ADP, collagen, and U46619 (a thromboxane-A2 analog)-induced PLT aggregation was investigated in basal (donor) and final (plateletpheresis unit) samples. The expression of PLT activation marker P-selectin (CD62P) was studied using flow cytometry in basal and final samples. In all cases, P-selectin was investigated in final samples after stimulation with ADP to assess for a possible further release of the antigen. Four additional plateletpheresis procedures were performed in donors from Group A, using the traditional, nonplasma-reduced program. RESULTS: Plateletpheresis obtained by means of the Trima device showed a lower response to in-vitro induced PLT aggregation and a higher percentage of P-selectin-expressing PLT when compared to products obtained using the Spectra device. Moreover, P-selectin release after ADP stimulation was reduced in plateletpheresis units obtained using the Trima device. These differences disappeared when a nonplasma-reduced collection program was used. In-vivo evaluation did not detect any difference between plateletpheresis obtained by means of the two cell separators. CONCLUSIONS: Plateletpheresis units obtained by means of multicomponent collection show a higher degree of PLT activation compared to traditional plateletpheresis procedures when high-concentration plasma-reduced products are collected. Randomized clinical studies are needed to assess the real impact of these findings in terms of in-vivo efficacy of plasma-reduced plateletpheresis units.

[Turnover of bone metabolism and prevalence of vertebral fractures in adrenal incidentalomas]. / Valutazione del turnover osseo e delle fratture vertebrali in incidentalomi surrenalici.

Aim of the study was to evaluate, in a homogeneous group of patients with adrenal incidentalomas (AI), calcium-phosphorus metabolism alterations, bone mineral density (BMD) and the prevalence of vertebral fractures. We selected 46 patients with adrenal incidentalomas (26M, 20F; age: M = 61 +/- 14, F = 65 +/- 10 years, BMI: M = 26.2 +/- 4 Kg/m2, F = 28.8 +/- 4 Kg/m2) compared with 84 normal subjects (NS) (44M, 40F; age: M = 60 +/- 10, F = 62 +/- 8 years; BMI: M = 27 +/- 2 Kg/m2, F = 28.1 +/- 4.5 Kg/m2). In all subjects we estimated calcium-phosphorus parameters. Our results showed that in 46 patients with AI there were a significant reduction of BMD-LS (0.915 +/- 0.176 g/cm2; p = 0.01) and of BMD-FN (0.710 +/- 0.129; p = 0.034) respect to those of NS (respectively: 0.994 +/- 0.14 9 g/cm2; 0.758 +/- 0.117 g/cm2). BMD-LS of the 20 women with AI (0.864 +/- 0.157 g/cm2, p = 0.01) was significantly reduced compared with the 40 female normal subjects (0.904 +/- 0.148 g/cm2); BMD-LS of the 26 men with AI (0.967 +/- 0.187 g/cm2; p = 0.048) was significantly reduced compared with the male normal subjects (1.048 +/- 0.133 r/cm2; p = 0.048). The MXA showed vertebral fractures in 15 (75%) of 20 patients with AI (2 patients were osteoporotic, 9 osteopenic and 4 normal at the MXA scans). In 20 women with AI, compared with female NS, we found a significant reduction of serum 25 OH D3 (p = 0.024) levels and a significant increase of plasma i-PTH (P = 0.04) value; and we found a negative correlation between plasma i-PTH and 25 OH D3 values (r = -0.451; p > 0.045). In conclusion, we demonstrated that patients with non-functioning adrenal incidentalomas present calcium-phosphorus metabolism alterations, associated at a reduction of BMD and an increase of vertebral fractures.

Hoffman's syndrome: muscle stiffness, pseudohypertrophy and hypothyroidism.

Primary hypothyroidism is a chronic and insidious disease caused by failure of thyroid hormone production. We observed a 38-year-old woman admitted to our hospital due to progressive proximal weakness, muscle pain and fatigue during mild exercise. Laboratory tests showed features of rhabdomyolysis and hypothyroidism. After examination of the thyroid, we reached a diagnosis of Hashimoto's thyroiditis and hypothyroid myopathy. Hypothyroidism should be considered as a differential diagnosis of creatine kinase elevation; actually, neuromuscular symptoms and signs occur in most newly diagnosed patients with thyroid diseases. Hypothyroidism presenting as muscle stiffness and pseudohypertrophy is called 'Hoffman's syndrome'.