Moyamoya syndrome and 6p chromosome rearrangements: Expanding evidences of a new association.

Background: Moyamoya syndrome represents an etiologically heterogeneous cerebral evolutive angiopathy. It can be associated with both well-characterized and recently described genetic conditions with mendelian inheritance.

Case Report: We report the case of a moyamoya angiopathy in a prematurely born girl affected by congenital heart defect, mild facial dysmorphism, mild neurodevelopmental delay and borderline cognitive profile, associated to a de novo complex rearrangement involving the terminal segment of the short arm of chromosome 6.

Pain therapy, pediatric palliative care and end-of-life care: training, experience, and reactions of pediatric residents in Italy.

Unlabelled: Pediatric palliative care represents the ideal response to life-limiting and life-threatening diseases and requires a specific and multidisciplinary training. This study aims at evaluating in Italy the training programs offered in pain therapy and pediatric palliative care, the exposure, and the personal experience concerning end-of-life care management. The data have been obtained through a survey addressed to all the residents specializing in pediatrics in Italy.

The management of sickle cell pain in the emergency department: a priority for health systems.

Objectives: Pain is the most common reason for admission to the Emergency Department (ED) in patients with sickle cell disease (SCD). It can be associated with severe complications and impairs quality of life. Pain management in the ED should be well-defined and aggressive.

Procedural pain in children: education and management. The approach of an Italian pediatric pain center.

Pain management should be warranted for all children in every situation. Italian legislation proposes a model for pain assistance based on specialized tertiary centers which provide direct clinical management for complex cases and assure continuous cooperation with hospitals and family pediatricians for managing painful conditions every day. The Procedural Pain Service of the University of Padua Department of Pediatrics applies such model for procedural pain management.

Procedural pain management in Italy: learning from a nationwide survery involving centers of the Italian Association of Pediatric Hematology-Oncology.

Procedural pain is an important aspect of care in pediatrics, and particularly in pediatric oncology where children often consider this to be the most painful experience during their illness. Best recommended practice to control procedural pain includes both sedative-analgesic administration and non-pharmacological treatments, practiced in an adequate and pleasant setting by skilled staff. A nationwide survey has been conducted among the Italian Centers of Pediatric Hematology-Oncology to register operators' awareness on procedural pain, state of the art procedural pain management, operators' opinions about pain control in their center, and possible barriers impeding sedation-analgesia administration.

The management of procedural pain at the Italian Centers of Pediatric Hematology-Oncology: state-of-the-art and future directions.

Purpose: The quality of life of children with cancer can be affected by the experience of cancer-related pain, treatment-related pain, procedural pain, generalized pain, and long-term chronic pain, and the consequences may be permanent. Treatment-related pain and procedural pain are often reportedly the most painful experiences relating to their illness. Procedural pain treatment is therefore now considered essential.

Congenital central hypoventilation syndrome and hypoglycaemia.

Unlabelled: Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder typically presenting in infants with an impaired automatic control of breathing, particularly during sleep, and often associated with variable patterns of autonomic nervous system dysregulations. We studied three children who had CCHS associated with episodes of severe hypoglycaemia and hyperinsulinaemia; we discuss the possible relationship with impaired dopamine-beta-hydroxylase function.

Conclusion: Hypoglycaemia and hyperinsulinaemia might be suspected in children with CCHS presenting with seizures and hyperhydrosis; though, further studies are needed to confirm this association.

The opinion of clinical staff regarding painfulness of procedures in pediatric hematology-oncology: an Italian survey.

Background: Beliefs of caregivers about patient's pain have been shown to influence assessment and treatment of children's pain, now considered an essential part of cancer treatment. Painful procedures in hematology-oncology are frequently referred by children as the most painful experiences during illness. Aim of this study was to evaluate professionals' beliefs about painfulness of invasive procedures repeatedly performed in Pediatric Hemato-Oncology Units.