Sabrina Congedi

Background: Scant data are available on nutritional status in bilateral lung transplant (BLT) candidates. Methods: All consecutive recipients admitted to the intensive care unit (ICU) of the University Hospital of Padua (February 2016–2020) after bilateral-lung transplant (BLT) were retrospectively screened. Data collected: (i) nutritional indices (body mass index (BMI), albumin level, prognostic nutritional index (PNI), mini nutritional assessment short-form (MNA-SF)); and (ii) muscular indices (creatinine height index (CHI)), skeletal muscle index (SMI), densitometry of paravertebral muscles on chest CT). Results: 108 BLT recipients were enrolled: 55% had a normal BMI, 83% had serum albumin levels > 35 g/L; high PNI and MNA-SF scores were recorded in most of patients. A total of 74% had a “normal or slightly reduced protein state“ according to the CHI score; 17% were identified as “sarcopenic” according to muscle densitometry (Hu < 30). Lower serum albumin was associated with longer invasive mechanical ventilation days (IMV) and ICU length of stay (p-value for non-linearity < 0.01). PNI and BMI were also associated with an increased ICU length of stay (p-value for non-linearity < 0.01). Conclusions: Most of the BLT recipients had normal nutritional and sarcopenia status. Pre-transplant albumin values correlated with the duration of IMV; serum albumin, PNI and BMI were associated with ICU stay. No nutritional or muscle parameters predicted re-intubation, 30-days rejection and overall length of hospital stay.

Introduction: Pial arteriovenous fistulas (pAVF) are rare vascular malformations, especially in children and newborns. In neonates, the most common symptom is congestive heart failure. Case presentation: We report a case of an asymptomatic preterm newborn incidentally diagnosed with pAVF during a routine cranial ultrasound (cUS) on the third day of life. Cerebral magnetic resonance (MRI) confirmed the diagnosis. A wait-and-see approach was chosen by the multidisciplinary team. The cUS and the MRI on day 14 of life showed the spontaneous resolution of the lesion. Conclusions: This case underlines the challenges in identifying pAVF in the first weeks of life and demonstrates a possible positive outcome for affected neonates.

Background: Endotracheal intubation in neonates is challenging and requires a high level of precision, due to narrow and short airways, especially in preterm newborns. The current gold standard for endotracheal tube (ETT) verification is chest X-ray (CXR); however, this method presents some limitations, such as ionizing radiation exposure and delayed in obtaining the radiographic images, that point of care ultrasound (POCUS) could overcome. Primary Objective: To evaluate ultrasound efficacy in determining ETT placement adequacy in preterm and term newborns. Secondary Objective: To compare the time required for ultrasound confirmation vs. time needed for other standard of care methods. Search Methods: A search in Medline, PubMed, Google Scholar and in the Cochrane Central Register of Controlled Trials (CENTRAL) was performed. Our most recent search was conducted in September 2021 including the following keywords: “newborn”, “infant”, “neonate”, “endotracheal intubation”, “endotracheal tube”, “ultrasonography”, “ultrasound”. Selection Criteria: We considered randomized and non-randomized controlled trials, prospective, retrospective and cross-sectional studies published after 2012, involving neonatal intensive care unit (NICU) patients needing intubation/intubated infants and evaluating POCUS efficacy and/or accuracy in detecting ETT position vs. a defined gold-standard method. Three review authors independently assessed the studies' quality and extracted data. Main Results: We identified 14 eligible studies including a total of 602 ETT evaluations in NICU or in the delivery room. In about 80% of cases the gold standard for ETT position verification was CXR. Ultrasound was able to identify the presence of ETT in 96.8% of the evaluations, with a pooled POCUS sensitivity of 93.44% (95% CI: 90.4–95.75%) in detecting an appropriately positioned ETT as assessed by CXR. Bedside ultrasound confirmation was also found to be significantly faster compared to obtaining a CXR. Conclusion: POCUS appears to be a fast and effective technique to identify correct endotracheal intubation in newborns. This review could add value and importance to the use of this promising technique.

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.

Background. Despite pain being a crucial aspect of urgent-emergency care, the attention of healthcare workers towards this symptom in the Emergency Department (ED) is still inadequate. The aims of this study were to assess children's and their adults accompanier's satisfaction regarding pain management in Italian ED, considering healthcare workers' attention to the symptom as well as the appropriateness and efficacy of treatments received. Methods. Questionnaires were administered face-to-face by trained interviewers over the period of one weekend in 29 Italian ED. The questionnaires were addressed to children 3-16 years old, assigned a green code at triage, and their adult accompaniers. Results. Overall, 1581 questionnaires were administered (923 to parents or caregivers, 658 to children). The reported level of attention to pain by the care team was high (57.20%) as was the adults' satisfaction with the pain management (95.01%); a high level of satisfaction was related to the healthcare workers' attention to pain. According to the adults, 73.56% of the healthcare professionals collected accurate information about pain; this was confirmed by the children. Pain was managed by a doctor-and-nurse team in less than half the cases (43.77%). Therapy efficacy was reportedly poor: only 14.01% of children were pain-free when leaving the ED. Conclusions. Despite increasing understanding of pain and the availability of guidelines and effective analgesics, efficient pain relief in paediatric patients in the ED remains an unfulfilled goal: half of children with pain did not receive any treatments. Therefore the quality of pain management in the ED needs to be improved.

Mucopolysaccharidoses (MPSs) are a group of rare, genetic lysosomal storage disorders. They are caused by deficiencies of the lysosomal enzymes involved in the degradation of glycosaminoglycans (GAGs). Pain is a common feature in mucopolysaccharidoses. However, the pathophysiology of pain in this group of diseases is still unclear and genesis of pain is multifactorial. Currently, poor data about pain management in these patients are available. Here, we present our clinical experience in complex pain management in three children with MPS.

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.

We describe a case of a paediatric patient affected by mandibular fibrous dysplasia (FD) with severe and chronic pain who was successfully treated with zoledronic acid (ZOL): a third-generation bisphosphonate. Further research is needed to assess its safety and efficacy as a treatment option for FD in the paediatric population.

We report our clinical experience on the effect of Scrambler Therapy (ST) for a child with acute mixed pain refractory to pharmacological treatment. ST, recently proposed as an alternative treatment for chronic neuropathic pain in adults, is a noninvasive approach to relieve pain, by changing pain perception at brain level. It is safe and has no side effects. Further research is needed to assess its efficacy for acute pain and for paediatric population.