Mucopolysaccharidosis (MPS) are a group of lysosomal storage disorders that are caused by the deficiency of enzymes involving in the catabolism of glycosaminoglycan (GAGs). GAGs incompletely degraded accumulate in many sites, damaging tissues and cells, leading to a variety of clinical manifestations. Many of these manifestations are painful, but few data are available in the literature concerning the prevalence, etiology, and pathogenesis of pain in children with MPS. This review, through the analysis of the data available the in literature, underscores the relevant prevalence of pain in MPSs’ children, provides the instruments to discern the etiopathogenesis of the disease and of pain, illustrates the available molecules for the management of pain and the possible advantages of non-pharmacological pain therapy in MPSs’ patients.

In recent years the emergence of new types of patient, clinical situations, technological frontiers and "health" objectives have changed considerably the needs of ill children, this also concerns pediatric palliative care (PPC). In Italy, despite the introduction of legislation (Law 38/2010) stipulating the right of children and families to access appropriate services for pain control and pediatric palliative care, the availability of these services is still limited. The aim of this study is to highlight, through a review of the existing data and published literature, the critical issues that obstacle the planning and development of PPC services in Italy. Four main areas identified were: socio-cultural setting; types of patients and nature of diseases requiring PPC; training for PPC providers; regulatory and political issues. This type of analysis can provide the rational for advancing proposals and developing supportive, corrective and implementation strategies.

Neonates are highly susceptible to bacterial infections, which represent a major source of mortality and morbidity in this age category. It is recognized that β2 integrins play a critical role in innate immunity by mediating leukocyte vascular adhesion, transmigration and bacterial phagocytosis. Therefore, we aimed to assess if the impaired immune functions seen in newborns may derive, in part, from a transient insufficient β2 integrin expression. In the present study we measured baseline lymphocyte function-associated antigen-1 (LFA-1 or CD11a/CD18), macrophage-1 antigen (MAC-1 or CD11b/CD18) and leukocyte integrin p150-95 (CD11c/CD18) expression on cord blood, and on the third day of life in a cohort of 35 healthy neonates, compared with a control group of 12 healthy adults. For any of the three β2 integrins, the expression on polymorphonuclear cells was significantly lower on cord blood than in adults and increased from birth to day 3. We also compared superoxide radical (SR) production in these neonates with 28 non-smoking adults. SR production in response to integrin stimulation by Zymosan was significantly lower at birth than in adults, and it decreased further in the third day of life. These findings suggest that innate immune impairment in newborns may be, in part, accounted for by a lower β2 integrin expression on phagocytes in the neonatal period, but also by a functional impairment of free radical production.