The proper assessment of needs and outcomes in pediatric palliative care (PPC) is imperative to ensure the best possible service to patients and families. However, given the multidimensional nature of PPC, the low number of patients in this setting, the heterogeneity of diseases, the presence of cognitive impairment in many patients, and the physiological development of children, outcomes can be complex and difficult to measure. Consequently, in this context, the use of standardized and validated tools to assess the needs of children and families, to assess symptom severity, and to estimate the quality of PPC service represent a current need. Even if efforts have been made to standardize approaches and tools for palliative care in adults, to our knowledge, a similar comprehensive assessment of PPC has not yet been conducted to date. This narrative review provides an overview and discusses the evaluation of tools currently applied in PPC, with an educational intent for healthcare providers. We found that several instruments are available to assess different dimensions of PPC. We proposed a classification into eligibility tools, patient and family needs assessment tools, and care assessment tools. At present, two main eligibility tools exist, the PaPaS Scale and the ACCAPED Scale questionnaire. Most of the tools for patient and family needs assessment have not been specifically validated in the PPC setting, and many may be more readily applied in research settings rather than in daily practice. Similar considerations can be made for tools assessing QoL, while tools assessing PPC service quality seem to be easily applied. Efforts to develop new specific tools and validate existing ones are undoubtedly advocated. However, in the patient’s best interest, PPC healthcare providers should start using available tools, regardless of their validation status.

Pediatric palliative care (PPC) is defined as “the active care of the child’s body, quality of life, mind and spirit, also giving support to the family”. PPC should be established once a diagnosis of life-limiting or life-threatening disease is reached and should continue as long as necessary. Therefore, pediatric palliative care (PPC) can continue for years, also given the improved care approaches for children with life-limiting or life-threatening diseases. Over time, the child may grow to become a young adult, and when this happens, the transition to adult healthcare services must be undertaken. This article discusses possible interventions, fostering an efficient transition from pediatric to adult palliative care. A narrative review presents issues, experiences, and existing programs. A “Perspectives” section presents opinions and proposals by the authors. The transition process is not limited to a change from pediatric to adult services. Rather, it includes the entire process of the development of the child and requires interdisciplinary management with proper planning and collaboration among professionals of pediatric and adult teams.

Sleep problems constitute a common and heterogeneous complaint in pediatric palliative care (PPC), where they often contribute to disease morbidity and cause additional distress to children and adolescents and their families already facing the burden of life-threatening and life-limiting conditions. Despite the significant impact of sleep problems, clinical evidence is lacking. The application of general pediatric sleep recommendations appears insufficient to address the unique challenges of the PPC dimension in terms of disease variability, duration, comorbidities, complexity of needs, and particular features of sleep problems related to hospice care. Therefore, we initiated an international project aimed at establishing a multidisciplinary consensus. A two-round Delphi approach was adopted to develop recommendations in the areas of Definition, Assessment/Monitoring, and Treatment. After selecting a panel of 72 worldwide experts, consensus (defined as ≥75% agreement) was reached through an online survey. At the end of the two voting sessions, we obtained 53 consensus recommendations based on expert opinion on sleep problems in PPC. This study addresses the need to personalize sleep medicine's approach to the palliative care setting and its peculiarities. It provides the first international consensus on sleep problems in PPC and highlight the urgent need for global guidance to improve sleep-related distress in this vulnerable population and their caregivers. Our findings represent a crucial milestone that will hopefully enable the development of guidelines in the near future.

Pediatric dystonias are associated with a broad spectrum of etiologies, resulting in a heterogeneous patient population in whom clinical presentation, evolution, and therapeutic needs may differ. These neurological symptoms are particularly common in children and adolescents with life-limiting and life-threatening conditions requiring pediatric palliative care (PPC). The impact on the child's quality of life is significant, as is distress for caregivers. Addressing and alleviating dystonia is key to providing good palliative care; however, there is limited evidence. A greater recognition and management of dystonia in this setting is urgently needed to provide appropriate interventions and care. To develop a standardized approach to dystonia in PPC. A two-round Delphi process explored the views of experts on the definition, assessment, monitoring, and treatment of dystonia in PPC. Professionals from different backgrounds and disciplines were invited worldwide. The final panel comprised 71 participants who completed a multi-statement online questionnaire. Fifty-three items were endorsed, providing expert, consensus-based recommendations. The limited clinical knowledge of childhood dystonia represents a challenge, especially in children with palliative care needs. This study is a first international consensus on dystonia in PPC and offers novel approaches to improving the dystonia-related burden and advancing clinical practice in this vulnerable population.

Summary: Narcolepsy type 1 (NT1) is a central disorder of hypersomnolence often arising in childhood and adolescence. NT1 has a significant, but poorly defined, psychological impact. We aimed to investigate the psycho‐social functioning of children and adolescents with NT1. We performed a cross‐sectional, child and parent‐reported questionnaire survey in 37 children and adolescents (6–17 years) with NT1, compared with age‐ and sex‐matched controls. Questionnaires (SSHS, ESS‐CHAD, CDI, MASC, CBCL, CRS‐R, and SNAP‐IV) evaluated various aspects of behavioural and emotional profiles, sleep habits, and daytime sleepiness. Subsequently, NT1 intra‐group analysis was performed to investigate the effect of sex (males vs females) and pharmacological treatment (treated vs non‐treated) on psychological features. The NT1 questionnaires total scores were then correlated with the clinical characteristics (age, body mass index [BMI], ESS‐CHAD score, cerebrospinal hypocretin‐1 [Hcrt‐1] levels, and diagnostic delay). Patients with NT1 showed a higher tendency to depressive symptoms, anxiety, somatisation, inattention, hyperactivity, oppositional/defiant problems, and other maladaptive behaviours compared with controls. Among NT1 patients, females showed a higher propensity to anxiety, and non‐treated patients displayed higher depressive symptoms. Psychological symptoms increased with age, BMI, and daytime sleepiness in patients with NT1, while a younger age was associated with more frequent somatisation symptoms. Lower cerebrospinal Hcrt‐1 levels correlated with poorer social competencies, daily activities, and inattention. Diagnostic delay was associated with a higher impact of depressive symptoms and behavioural problems. NT1 in children and adolescents is associated with poorer functioning in multiple psychological domains calling for a multidisciplinary approach and monitoring to reduce disease burden and to prevent psychiatric consequences.

Stretch syncope (SS) is a benign, uncommon, distinct condition described mainly in adolescent males. It is responsible for paroxysmal events started by stereotyped stretching actions with neck hyperextension, culminating in alteration of consciousness. Motor manifestations are often present and may be associated with a generalized slowing of the electroencephalographic activity, challenging the diagnosis. Despite a few cases reported in the literature, different mechanisms have been implied in the pathogenesis, involving both local and systemic hemodynamic phenomena. Here, we report on an 8-year-old girl with self-induced SS, providing new insights into the related neurophysiological profile and discussing the possible etiology. Our evidence of transient and dynamic vascular impairment supports the hypothesis of SS as a multifactorial disorder.