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Background: Children with medical complexity need complex assistance, that considerably affects caregivers’ quality of life. They often need multiple medications, with a consequent relevant risk of errors or poor compliance. Galenic (or compounded) drugs are blended in the pharmacy’s laboratory worldwide according to different rules and tailoring the patient’s needs. While their use may sometimes simplify these therapies, little is known about parents’ attitude about this issue. Aim: This study aimed at investigating the complexity of the daily therapy management and exploring the parents’ opinions about galenic compounds. Design: Parents were interviewed by using a structured questionnaire. Setting: Children followed by the Pediatric Palliative Care Network in Friuli Venezia Giulia, Italy, were included from November 2021 to April 2022. Those diagnosed with malignancies were excluded, since therapies are mainly administered through a central venous catheter. Results: Thirty-four parents were interviewed. Fourteen patients took drugs orally, one via nasogastric tube (NGT), 18 via gastrostomy, and one orally + NGT. The mean number of drugs taken every day was six (2–14), in mean 10 (3–18) administrations, that overall required a mean of 44 (8–180) minutes to be delivered. Twenty-eight parents used galenic compounds, and 24 reported relevant advantages, because of a ready-to-use and safe formulation. Conclusions: The therapy management of children with medical complexity relies on parents. Galenic compounds may improve both patients’ and caregivers’ quality of life, either in terms of shorter time of administration or smaller risk of errors. Therefore, their use should be encouraged worldwide, according to the different reference rules.

Object: Hydrocephalus is one of the main complications of brain tumors in children, being present in about 50% of cases at the time of the tumor diagnosis and persisting up to 10–40% of cases after surgical resection. This is a single-institution retrospective study on the variables that may predict the need for treatment of persistent hydrocephalus in pediatric patients presenting with a brain tumor. Methods: Retrospective case note review of 43 newly diagnosed brain tumors in children referred between April 2012 and January 2018 to our regional pediatric neuro-oncology service was carried out. Diagnosis of hydrocephalus was carried out using both preoperative and postoperative MRI to determine Evans’ index (EI) and the fronto-occipital horn ratio (FOHR) from each scan. Simple logistic regression was used to analyze categorical variables as appropriate. A p value Results: Forty-three children were analyzed, 26 males and 17 females with a median age at diagnosis 10.4 years (IQR: 5.2–13.5). Hydrocephalus was present in 22/43 children (51%) preoperatively; in 8/22 children (36%) with hydrocephalus undergoing tumor resection, hydrocephalus persisted also in the postoperative period. An EI >0.34 (p = 0.028) and an FOHR >0.46 (p = 0.05) before surgery were associated with a higher prevalence of persistent hydrocephalus and therefore to the need for a cerebrospinal fluid drain device in the postoperative phase. Conclusion: Preoperative identification of children at risk for developing persistent hydrocephalus would avoid delays in planning the permanent cerebrospinal fluid drain devices. This study finds that an EI >0.34 and an FOHR >0.46 at diagnosis could impact on the therapeutic management of children with hydrocephalus associated with brain tumors. Prospective and larger-scale studies are needed to standardize this approach.

Background. While hematological symptoms are considered difficult to manage in a Pediatric Palliative Care setting, home may still represent a safe and convenient place for transfusions in patients with advanced malignancy or chronic conditions. This research focuses on the safety and feasibility of a home transfusion program.Methods. This is a case series of patients between 0 and 18 years diagnosed with advanced malignancy or incurable chronic conditions and eligible to Pediatric Palliative Care who received home platelet or packed red cell transfusions. For all patients, we recorded adverse events such as acute hemolytic reactions, allergic reactions, or any emergency condition requiring hospital admission, equipment failure, blood product transport or storage errors, errors in patient identification, and personnel safety issues. We explored parental satisfaction with a Likert-type questionnaire and short open questions.Results. We reviewed 101 transfusion procedures for six patients in Pediatric Palliative Care performed by the Regional Pediatric Palliative Care network between 2014 and 2020. We did not report any adverse effects. Families reported satisfaction and a sense of safety and positively evaluated the opportunity of having transfusion at home to minimize the disruption in everyday life. The cost analysis resulted in a consistent saving for the Regional Health System.Conclusion. This study supports the safety and feasibility of home transfusion in Pediatric Palliative Care. (C) 2021 American Academy of Hospice and Palliative Medicine. Published by Elsevier Inc. All rights reserved.

Infective uvulitis is a rare condition in children. In this report, we describe the case of a 4-year old-patient who presented a group A Streptococcus pharyngitis with uvulitis. No signs of epiglottitis were detected at nasal fibroscopy. She recovered rapidly with intravenous antibiotic therapy and 2 days of corticosteroid. Uvulitis is usually caused by group A Streptococcus or Haemophilus influentiae, but also other bacteria can be detected. Uvulitis can be isolated, or it can occur with epiglottitis and become an emergency.

We report the case of a girl with a novel mutation of the COL4A gene (c.2716+2T>C) presenting microcephaly, parenchymal hemorrhages, lissencephaly, and bilateral cataracts, associated with agenesis of the corpus callosum and hypoplasia of the optic nerve. COL4A1, located on chromosome 13, encodes the α1 chain of type IV collagen, a key component of the basement membrane in various organs, such as eye, brain, kidneys, and muscles. Different mutations have been described and may remain asymptomatic or determine porencephaly, cerebral hemorrhages, renal cysts, hematuria, and dysgenesis of the anterior segment of the eye.

Aim Children and adolescents affected by somatization and somatic symptom disorder commonly refer to emergency services. Due to the absence of specific guidelines for the emergency setting and to a possible lack of knowledge, these patients are at risk of being unrecognized and mismanaged. This study aims at proposing a clinical practice to approach and manage these patients and their families in the emergency setting. Methods This manuscript derived from the work of a research group of italian pediatric emergency physicians and anesthesiologists, with an expertise in pain management, members of the PIPER group. The research group reviewed the literature about psychosomatic pain and somatic symptom disorder and developed a clinical practice specific for the pediatric emergency setting. Results The manuscript provides information about the main clinical features shared by patients with psychosomatic pain and about current diagnostic criteria and appropriate management in the emergency setting. Furthermore, it highlights the possible pitfalls in which the emergency physician may run into dealing with these patients. Conclusion This clinical practice should be seen as a starting point toward a better understanding of patients with psychosomatic pain and a standardization of care in the pediatric emergency setting.

Objective The aims of the present study were: (1) to review the literature on long-lasting cognitive sequelae in children treated for Posterior Fossa Tumor and (2) to investigate anatomic functional relations in a case series of 7 children treated for PFT using magnetic resonance imaging (MRI) post-processing methods. Methods We retrospectively analyzed MRIs of children who underwent complete surgical resection of PFT and performed extensive neuropsychological evaluation. Tumor, ventricular volumes, and VPS insertion site were drawn on T1 volumetric MRI scans and normalized to a pediatric template. Children showed worse performances on tasks tapping executive functions, memory, visuo-motor precision, and expressive language. Results Volumes of interest related to these functions showed a maximum overlap on the left vermis and the lateral ventricle enlargement, except for impaired narrative fluency –which was associated with left lateral ventricle enlargement– and narrative memory –which was related to the right vermis and the enlarged fourth ventricle. Conclusion Results suggest that anatomic functional relations in children treated for PFT are related to a combination of different pathophysiological factors.

Background Cerebellar tumor survivors often exhibit neuropsychological deficits that could be related to alterations in cerebro-cerebellar networks. This is a pilot study designed to understand if diffusion tensor imaging (DTI)-based tractography is able to identify possible correlations between cerebellar white matter structure and cognitive outcome in children on long-term follow-up for posterior fossa (PF) tumors who were thoroughly assessed for neuropsychological functioning. Methods DTI-based tractography was performed in pediatric patients with PF tumors. Fractional anisotropy (FA) and volumetric measurements of spinocerebellar, dentorubrothalamocortical and corticopontocerebellar tracts were analyzed. Cognitive and neuropsychological functioning was assessed by the Wechsler Intelligence Scale for Children-IV Edition (WISC-IV) and the Developmental Neuropsychological Assessment (NEPSY II). The associations between Full-Scale Intelligence Quotient (FSIQ), NEPSY-II scores, and fiber tracts were tested by the Spearman rank correlation coefficient. Results Seven patients (median age at diagnosis five years, range, 3-13) treated for medulloblastoma (2/7; 29%) and pilocytic astrocytoma (5/7; 71%) were retrospectively evaluated. All children had complete surgery. The median FSIQ was 84 (range, 67-93). Patients presented with several deficits on many NEPSY-II tasks; in particular, memory was impaired in nearly half of them. FSIQ and neurocognitive tasks significantly correlated with specific corticopontocerebellar tracts. Conclusion Children on follow-up for PF tumor showed scattered cognitive impairments, including deficits in long-term and immediate memory. Tractography allowed us to describe a possible association between the integrity of cerebellar pathways and neurocognitive performance, suggesting that the myelinization of these fibers may represent an indicator for the development of long-term cognitive sequelae.

Objective Pain control is a priority in patient evaluation. Despite the proliferation of guidelines, pain is still underassessed and undertreated, especially in children. To improve efficiency and to adhere to best medical practice, our triage software was upgraded; it included mandatory pain scoring for the admission of a child to the pediatric emergency service, thereby limiting the chances of overlooking a child experiencing pain. We conducted this study to verify the effect of routine versus mandatory pain scoring on pain management under the hypothesis that mandatory pain assessment would improve reevaluation and pain treatment. Methods This retrospective case-control study was conducted in our Pediatric Emergency Department (ED). We collected data with regard to pain assessment and reassessment at triage and during the entire stay in the ED, and also with regard to the drugs eventually administered. We reviewed the charts of 1274 patients admitted with the older triage software and those of 1262 patients admitted with the newer triage software (intervention group). Results Pain was evaluated significantly more frequently in the intervention group at triage, during the medical evaluation, and at discharge. In the intervention group, a smaller percentage of patients were treated for pain at both triage and during their stay in the ED (P=0.078 and 0.048). Pain reassessment resulted lower in the intervention group (P Discussion Mandatory pain assessment improved the pain evaluation rate. This did not, however, translate into better treatment and management of pain in the pediatric emergency setting.

Background Many biological and inflammatory markers have been proposed as having a prognostic value at diagnosis of Hodgkin lymphoma (HL), but very few have been validated in paediatric patients. We explored the significance of these markers in a large population of 769 affected children. Patients and methods By using the database of patients enrolled in A.I.E.O.P. (Associazione Italiana di Emato-Oncologia Pediatrica) trial LH2004 for paediatric HL, we identified 769 consecutive patients treated with curative intent from 1st June 2004 to 1st April 2014 with ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine), or hybrid COPP/ABV (cyclophosphamide, vincristine, prednisone, procarbazine, doxorubicin, bleomycin and vinblastine) regimens. Results On multivariate analysis with categorical forms, the 5-year freedom from progression survival was significantly lower in patients with stage IV or elevated value of platelets, eosinophils and ferritin at diagnosis. Furthermore, stage IV and eosinophils seem to maintain their predictive value independently of interim (after IV cycles of chemotherapy) positron emission tomography. Conclusion Using the combination of four simple markers such as stage IV and elevated levels of platelets, ferritin and eosinophils, it is possible to classify the patients into subgroups with very different outcomes.

The aim of this study is to compare the prevalence of sleep disorders (SD) between children treated for brain tumors and healthy children, and to define the type of SD. A case-control study was performed from October 2014 to April 2015. Inclusion criteria were patients between 2 and 16 years with “cases” defined as children affected by central nervous system tumors at least 3 months after the end of treatment (surgery and/or radiotherapy and/or chemotherapy) at the time of evaluation and “controls” as healthy children. Children’s sleep quality was assessed with a questionnaire administered to parents (Child’s Sleep Habits Questionnaire, CSHQ). A total score greater than 41 is suggestive for the presence of disturbed sleep. The risk of SD was estimated by the odds ratio (OR) and their 95% confidence intervals (95% CI) through logistic regression models. Twenty-nine cases and 87 controls (in a 1:3 model) were enrolled, for a total of 116 subjects. The prevalence of SD resulted of 82.8% among cases and 64.4% in controls. A statistically significant difference between the two groups (OR 2.65; 95% CI 0.92–7.65) was not reached. Analyzing singular disturbances, parasomnias and night awakenings showed a statistically significant difference between the two groups (OR 4.32; 95% CI 1.08–17.34). Our study revealed a trend toward SD in children with brain tumor when compared to healthy population. Hovewer, analyzing specific subtypes of SD some significant differences were obtained. A significant difference was obtained only for specific subtypes of SD. Further investigations could better define the real burden of SD.

The aim of this study is to describe the symptoms and signs of central nervous system (CNS) tumors in a pediatric population and to assess the time interval between the onset of the disease and the time of the diagnosis. A retrospective observational study was conducted at our Oncology Pediatric Unit between January 2000 and November 2011. We included 75 children between 5 months and 16 years (mean age of 7.8 ± 4.7 years), with male to female ratio of 3:2. The tumor localization was supratentorial in 51% of cases, and the most frequent histological type was low-grade astrocytoma (48%). Presenting symptoms were headache (31%), vomiting (31%), seizures (21%), and behavioral change (11%). The most common symptoms at diagnosis were headache (51%), vomiting (51%), visual difficulties (37%), seizures (24%), and behavioral change (21%). By the time of diagnosis, neurologic examination was altered in 68% of our patients. Vomiting (44%) and behavioral change (44%) were the most frequent symptoms in children under 4 years of age, headache (61%) and vomiting (54%) in children older than 4 years. The median interval between symptoms’ onset and diagnosis was 4 weeks (range 0 to 314 weeks). A longer symptom interval was associated with younger age, infratentorial localization and low-grade tumors. The differences in symptom intervals between the different age, location, and grade groups were not statistically significant. Survival probability was influenced by tumor grade but not by diagnostic delay or age of the child. Headache and vomiting are the earliest and commonest symptoms in children with brain tumors. Visual symptoms and signs and behavioral change are often present. Abnormalities in neurological examination are reported in most of the children. Intracranial hypertension symptoms suggest the need for a neurological clinical examination and an ophthalmological assessment.

Occlusion of blood vessels in various parts of the body occurs continually in sickle cell disease (SCD). Most important acute features of SCD are: recurrent painful vaso-occlusive crisis, acute chest syndrome, acute splenic sequestration, priapism and anemia

A case of a non-anaplastic pleomorphic xanthoastrocytoma (PXA) presenting with leptomeningeal dissemination (LMD) affecting a 9-year-old girl is presented.The neoplasia in this young girl had the otherwise classical clinical features of PXA: the relatively advanced paediatric age of the patient, the seizures as presenting sign; the primary site in the temporal lobe; and the MRI findings of the partially solid and cystic superficial lesion. Only the tumour involvement of the chiasma and the infundibulus was a relatively unusual finding. In a 5-year period, the tumour underwent malignant transformation, bringing the child to death because of the primary tumour progression. However, the leptomeningeal deposits remained unchanged throughout the clinical course.To our knowledge, this is the first case of a non-anaplastic PXA presenting with disseminated disease. Thus, it was thought important to describe this case in order to add further information regarding the spectrum of the presenting clinical features of this rare neoplasm and the phenomenon of LMD of non-malignant glioma.