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The Charter of the Rights of the Dying Child was formulated as a professional guide for caring the child in the final stages. The study examines the nurses' degree of agreement with the Charter's principles and their perception of the implementation of those principles in hospital.A multicenter, cross-sectional study to observe the nurses' positions about the 10 rights outlined in the Charter, using an online questionnaire in 5 pediatric hospitals in northern Italy.A total of 119 nurses (44.9%) completed the questionnaire. The majority (range: 86.6-100%) expressed their agreement with the Charter's principles (Likert ≥4). Lower ratings were reported in Charter's principles implementation items (range: 42.9-89.1%). Being older and working in a smaller hospital lead the nurses to overlook the child's right to be informed and to be given the opportunity to make decisions about his/her own life and death (p = 0.02, p 0.01). Postgraduate training induced greater awareness of the dying child (p = 0.01).This study highlights the value of the Charter of the Rights of the Dying Child as a reference guideline for nurses working in pediatric hospitals. Better training is important to improve the nurse's communication skills and the pediatric palliative care should be offered to all families that have a child with incurable disease mostly in the end of life.

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.

Purpose The death of a child is a devastating and tragic event for all those involved. This charter aims to help healthcare workers and people assisting terminally ill children to recognize some important rights of the child, with some related suggestions. We consider it important to have a trace of this process, based on the skillfulness of long-lasting experts. Methods In September 2012, a group of professionals working with children affected by incurable illness in Italy launched a project to formulate the charter. Trieste is the city where the group of professionals first met to start the project. The first step was a detailed literature search on the topic, the second step was an extensive discussion among the professionals (writing committee) to prepare a first draft; later (third step) the draft was revised by 38 experts in different areas, including patient and family representatives, and lastly (fourth step) the final version of the charter was prepared. Results We developed a document containing 10 rights and corresponding duties that could be applied to any clinical situation or circumstances and used as a guide by professionals and families caring for children in the terminal stages of an illness. Conclusions The Trieste Charter proposes fundamental rights for children who are approaching the end of their lives. The charter will have achieved its purpose when every person caring for a dying child is capable of staying near the child until the last moments of his or her life, prepared to accept his or her death, ensuring both respect and dignity.

Bronchopulmonary dysplasia (BPD) is a common adverse outcome of prematurity, causing severe morbidity and mortality. The cytokine macrophage migration inhibitory factor (MIF) has been recently shown to favor murine fetal lung development. In this prospective study, we evaluate the expression of MIF in the lung and in the serum of preterm infants (n = 50) and investigate whether the −173 G/C MIF promoter polymorphism is associated with the risk of BPD (n = 103). MIF was highly expressed in lung tissue from preterm infants. Serum MIF levels were measured by ELISA at d 1 after birth. MIF levels were increased [median (interquartile range), 71.01 (44.9–162.3) ng/mL], particularly in those infants with RDS [110.4 (59.4–239.2) ng/mL] compared with healthy adults [2.4 (1.2–5.0) ng/mL], (p < 0.001). The MIF −173*C allele, which predisposes to higher MIF production, was associated with a lower incidence of BPD (OR, 0.2; 95% CI, 0.04–0.93), independently from mechanical ventilation and oxygen exposure (p = 0.03). In conclusion, these data show that MIF expression is increased in lung and serum of preterm infants and suggest that the high producing MIF −173*C allele may be a protective factor for BPD.

We investigated the association of mannose-binding lectin (MBL) serum levels with nosocomial sepsis (NS), their changes overtime during infection, their relation with pathogens, with the MBL2 genotype and their relationship with mortality. In a prospective observational study, we included 365 critically ill neonates: 261 had no infection and 104 had at least 1 septic event. The median MBL serum concentration was significantly lower in infected than in noninfected neonates (p < 0.001). Low MBL levels on admission increased the risk of infection, independently from gestational age and invasive procedures. The median peak MBL level during infection was higher than the median level on admission (p < 0.001) and was correlated with it (r2 = 0.83, p < 0.001). Moreover, MBL levels on admission were not associated with death (OR = 0.80, 95% CI = 0.56–1.14, p = 0.21). Similarly, no association was found between MBL peak levels during infection and death among infected neonates (OR = 1.10, 95% CI = 0.78–1.57, p = 0.57). In 127 neonates (42 infected) genotyped for exon-1 and -221 promoter MBL2 variants, we did not find significant difference in the frequencies of MBL2 genotypes between infected and noninfected neonates. Moreover, no association was found between MBL2 genotypes and death.

Recent progress in neonatal care has greatly improved the prognosis and the probabilities of survival in very sick or very preterm neonates and has modified the concept and limits of the so called viability. However, in some circumstances, when the death of the baby can only be postponed temporarily, at the price of severe suffering, or when survival is associated with severe disabilities and an intolerable life for the child and the family, then it might not be appropriate to utilize all the armamentarium of neonatal intensive care. In such circumstances, limitation of intensive treatments (withholding or withdrawal) generally invasive and painful, could represent a more human and reasonable alternative. The ethical principles underlying those decisions, the most frequent situations occurring in practice, the role of parents in the decision-making process, and the opinions and behavior of neonatologists from many European intensive care units will be examined and discussed.

Background.Nosocomial infections are still a major cause of morbidity and mortality among neonates admitted to neonatal intensive care units (NICUs).Objective.To describe the epidemiology of nosocomial infections in NICUs and to assess the risk of nosocomial infection related to the therapeutic procedures performed and to the clinical characteristics of the neonates at birth and at admission to the NICU, taking into account the time between the exposure and the onset of infection.Design.A multicenter, prospective cohort study.Patients and Setting.A total of 1,692 neonates admitted to 6 NICUs in Italy were observed and monitored for the development of nosocomial infection during their hospital stay.Methods.Data were collected on the clinical characteristics of the neonates admitted to the NICUs, their therapeutic interventions and treatments, their infections, and their mortality rate. The cumulative probability of having at least 1 infection and the cumulative probability of having at least 1 infection or dying were estimated. The hazard ratio (HR) for the first infection and the HR for the first infection or death were also estimated.Results.A total of 255 episodes of nosocomial infection were diagnosed in 217 neonates, yielding an incidence density of 6.9 episodes per 1,000 patient-days. The risk factors related to nosocomial infection in very-low-birth-weight neonates were receipt of continuous positive airway pressure (HR, 3.8 [95% confidence interval {CI}, 1.7-8.1]), a Clinical Risk Index for Babies score of 4 or greater (HR, 2.2 [95% CI, 1.4-3.4]), and a gestational age of less than 28 weeks (HR, 2.1 [95% CI, 1.2-3.8]). Among heavier neonates, the risk factors for nosocomial infection were receipt of parenteral nutrition (HR, 8.1 [95% CI, 3.2-20.5]) and presence of malformations (HR, 2.3 [95% CI, 1.5-3.5]).Conclusions.Patterns of risk factors for nosocomial infection differ between very-low-birth-weight neonates and heavier neonates. Therapeutic procedures appear to be strong determinants of nosocomial infection in both groups of neonates, after controlling for clinical characteristics.

Aim: To validate an Italian parental questionnaire designed to evaluate the neuropsychological and behavioural developmental status of 4-year-olds and identify children in need of further evaluation. Methods: The questionnaire (Questionario per la valutazione dello Sviluppo di bambini a 4 anni – Genitori (QS4-G) ) consisted of 93 questions divided into 10 areas: language, visual-motor abilities, memory/attention, fine and gross motor and self-help abilities, lateralisation, social skills, stress, sleep, alimentation and evacuation. It was distributed to 263 parents of 4-year-olds: 94 healthy preterm (gestational age

Mannose binding lectin (MBL) is a soluble pattern recognition receptor of innate immunity that binds a wide range of pathogens and exerts opsonic effects. We investigated the association between serum MBL levels and development of sepsis in infants admitted to neonatal intensive care units (NICUs). Serum MBL levels on admission were measured by enzyme-linked immunosorbent assay (ELISA) in 206 neonates consecutively admitted to an NICU of whom 138 did not develop hospital-acquired sepsis and 68 did. Of these 68, 40 had confirmed sepsis with positive blood cultures, 19 clinically suspected sepsis, with negative blood cultures, and nine had clinically suspected sepsis with blood culture yielding coagulase-negative staphylococci (CoNS). Serum MBL levels on admission were significantly lower in infants with sepsis [0.45 microg/mL; interquartile range (IQR) 0.09-1.68], particularly in those with confirmed sepsis (0.17 microg/mL; IQR 0.05-0.96), compared with infants without sepsis (1.45 microg/mL; IQR 0.43-3.52), and infants with CoNS-positive blood culture (1.70 microg/mL: IQR 0.85-3.60). After adjusting for duration of exposure gestational age (GA) and birth weight (BW), the association of low MBL levels with development of sepsis was maintained [odds ratio (OR) = 0.52; 95% confidence interval (CI): 0.36-0.75]. The measurement of serum MBL levels on admission in NICU may help to identify neonates at higher risk of developing sepsis.

Aim: A number of social and health aspects in Italy show remarkable geographic dishomogeneity. We investigated if this phenomenon involves the outcome of very low birth-weight infants (VLBWI). Methods: This is a multi-centre nation-based survey among all Italian NICUs. The number of VLBWI admitted to each NICU in 2001 by birth-weight classes of 250 g, their inborn/outborn status and survival at discharge were registered through ad hoc questionnaires. The data were analysed for the whole country and for three geographic areas (North, Centre and South). Results: A total of 4679 VLBWI in 125 units were surveyed (0.88% of live births in Italy in 2001). The median number of infants admitted was 34 per NICU (interquartile range 16,52), without significant differences among the three geographic areas. The inborn rate was 80.7% (86.5% in the North, 83.7% in the Centre and 74.6% in the South). The mortality rate was 19.6% (15.6% in the North, 19.3% in the Centre and 23.4% in the South). Adjusted relative risk of death between Southern and Northern regions was 1.48 (95% CI 1.30–1.68), and that between outborn and inborn infants was 1.20 (95% CI 1.04–1.37). Conclusions: The differences in mortality among geographic areas suggest a state of socio-sanitary deprivation in the Southern regions, as well as different models of organisation and quality of perinatal care.

The assessment of lung volumes, particularly functional residual capacity (FRC), is crucial for understanding lung development during infancy in CDH patients. Aim To evaluate changes in lung function during infancy in subjects with CDH treated with a “gentle ventilation” technique and delayed surgery strategy in the neonatal period. Methods 13 CDH infants were studied twice and compared with a population of 28 healthy infants (HI). Tidal-Volume (Vt), respiratory rate (RR) and time to peak expiratory flow/expiratory time ratio (tPTEF/Te) were measured with an ultrasonic flow meter; Compliance (Crs) and Resistance (Rrs) of the respiratory system were studied with the single occlusion technique; FRC and Lung Clearance Index (LCI), were assessed with the sulfur hexafluoride (SF6) wash-in/wash-out technique. The differences between the first (T1) and second (T2) measurement in the CDH group were assessed by the Student's t-test for paired values. For each set of measurement (T1 and T2) the values were compared with HI by Student's t-test. Results Mean age at test was 7.5 ± 5.2 months for HI, 4.5 ± 2.5 at T1 and 11.9 ± 4.5 months at T2 for CDH infants. At T1 there were no significant differences between CDH infants and HI in Vt, Crs, and FRC, while tPTEF/te ratio was lower and RR, Rrs, and LCI were higher in CDH patients than in HI. At T2 Vt, Crs, and FRC remained normal in CDH patients as well as RR that, at this time was not different between CDH and healthy infants; tPTEF/te remained below and Rrs and LCI remained above normal ranges, indicating a persistent impairment in lower airways patency. Conclusions Lung function in infants with severe CDH is characterized by a persistent impairment in airways patency and significant inhomogeneity of ventilation, suggesting a peripheral bronchial obstruction even if the other lung function tests are within normal ranges. Pediatr Pulmonol. 2007; 42:600–604. © 2007 Wiley-Liss, Inc.

Closure of the ductus arteriosus (DA) is due to functional constriction followed by wall remodeling, with neointimal formation caused by proliferation and migration of smooth muscle cells (SMCs) from the media to subendothelium. CD44 is a surface cell proteoglycan family. Its isoform, CD44-v6, is only minimally expressed in SMCs in the media of normal arteries, but is highly expressed in SMCs in the intima and media of injured arteries (e.g., atherosclerosis). Twenty-two autopsy DA specimens, 11 from full-term babies (age range 2 days to 5 months) and 11 from premature babies (age range 3 days to 5 months), with varying degrees of ductal wall remodeling, were evaluated by immunohistochemistry using antiactin, antifibronectin-extradomain A, anti-leukocyte common antigen, anti-CD44, and anti-CD44-v6. In DA with wall remodeling, synthetic antifibronectin-extradomain A-positive SMCs were evident at the neointimal mounds, and the SMCs were highly positive for the CD44-v6 isoform, irrespective of gestational age at birth. Conversely, SMCs of either closed DAs or persistently patent DAs were CD44-v6 negative. In conclusion, the present data provide evidence that closure of DA involves synthetic SMCs highly positive for CD44-v6, and patent or closed DAs are populated by CD44-v6-negative SMCs.

Recent advances in neonatal care have greatly improved the chances for survival of very sick and/or very preterm neonates and have in fact changed the concept and the limits of viability. However, in some situations, when the infant’s demise can only be postponed at the price of great suffering or when survival is associated with severe disabilities and an intolerable life for the patient and the parents, it may be unwise to employ the full armamentarium of modern neonatal intensive care. In those circumstances withholding or withdrawing mechanical ventilation and other life-saving, though invasive and painful, procedures might be a better option. This review examines the ethical principles underlying those difficult decisions, the most frequent circumstances where they should be considered, the role of parents and other parties in the decision-making process and the reported behavior of neonatologists in many American and European neonatal intensive care units.

Summary Radiological examination of the abdomen is critical to the diagnosis of necrotising enterocolitis (NEC). Previous studies on the reproducibility of radiological findings have been limited in size and based only on infants with the disease. We conducted a study among a sample of high-risk infants with and without the diagnosis of NEC: (1) to measure the degree of inter-observer agreement of NEC radiological diagnosis and signs and (2) identify a profile of radiological signs which led the observers to make a diagnosis of NEC. We collected 297 X-rays from a sample of 57 newborns admitted in 1999 to neonatal intensive care and neonatal surgery units in Rome, Italy. Three specialists in paediatric radiology examined the films independently and without any clinical information about patients. The analyses were conducted on a total of 891 forms filled in by the observers. Kappa values were calculated to measure the inter-observer reliability. To identify the profiles of radiological signs, a multidimensional analysis, binary segmentation, was carried out. The reproducibility of radiographic signs was 0.55 (P

The neonatal EEG remains one of oldest, yet most valuable, diagnostic and prognostic tests in neonates. The goals of this study were to determine the relationships between the morphology, frequency, and distribution of ictal discharges in the neonatal EEG with age, EEG background activity, and etiology. A total of 156 ictal events were evaluated in 11 preterm (PT) and 25 fullterm (FT) infants. Most of the infants had severe abnormalities of background activity although ictal discharges occurred on both normal and abnormal backgrounds. There was a trend for a closer relationship between behavioral changes during the electroencephalographic seizure when the background activity was normal or moderately abnormal than when background activity was severely abnormal. In both PT and FT infants, the most common site of seizure origin was the temporal lobe. FT infants commonly had sharp waves, spikes, sharp and slow waves, and spike and slow waves at the onset of the ictus while rhythmic delta activity was most common in the PT infants. PT infants typically had a regional onset to the ictus whereas FT infants most frequently had a focal onset. Duration of the ictal events was similar in PT and FT infants and a change in morphology or frequency of the discharges was common during propagation of the ictal discharges in both age groups. There was not a clear relationship between onset, morphology, frequency, or propagation patterns and etiology in either the PT or FT infants. Our results demonstrate that while the type of ictal discharge is related to gestational age, there is a rich variety in the onset, morphology, and frequency of the ictal discharges in both PT and FT infants and that neonatal ictal patterns lack a close correlation with underlying pathology.

The clinical records a years cohort of 280 newborn infants consecutively hospitalized for 48 h or more in our neonatal intensive-care unit (NICU) were reviewed. Information on the infants' conditions during the first 12h of life, and on the procedures used in the NICU, were collected. Statistical significance was tested by univariate analysis with the chi(2) test and by multivariate logistic regression analysis with the software program SPSS (Version 10). Over the one-year period reviewed, 90 hospital-acquired infections (HAIs) were contracted; 55 (19.6%) of infants had at least one infection during their stay. The overall in-hospital mortality was 7.1%, and mortality was higher in infants in whom at least one infection developed than in non-infected infants (12.7 vs. 5.8% P=0.13). Very low birthweight infants (VLBW1,501 g) who had more severe clinical conditions on admission [clinical risk index for babies (CRIB) score/=5] had an almost two-fold higher risk of contracting a HAI. In the multivariate regression analysis, the onset of a HAI was strongly associated with a low gestational age and the presence of an intravascular catheter. HAIs frequently complicate hospitalization in NICUs and are associated with increased mortality. Our findings also suggest that CRIB could be predictive for the risk of infection in VLBW infants.

Summary Background The ethical issue of foregoing life-sustaining treatment for newborn infants at high risk of death or severe disability is extensively debated, but there is little information on how physicians in different countries actually confront this issue to reach end-of-life decisions. The EURONIC project aimed to investigate practices as reported by physicians themselves. Methods The study recruited a large, representative sample of 122 neonatal intensive-care units (NICUs) by census (in Luxembourg, the Netherlands, and Sweden) or stratified random sampling (in France, Germany, the UK, Italy, and Spain) with an overall response rate of 86%. Physicians' practices of end-of-life decision-making were investigated through an anonymous, self-administered questionnaire. 1235 completed questionnaires were returned (response rate 89%). Findings In all countries, most physicians reported having been involved at least once in setting limits to intensive care because of incurable conditions (61–96%); smaller proportions reported such involvement because of a baby's poor neurological prognosis (46–90%). Practices such as continuation of current treatment without intensification and withholding of emergency manoeuvres were widespread, but withdrawal of mechanical ventilation was reported by variable proportions (28–90%). Only in France (73%) and the Netherlands (47%) was the administration of drugs with the aim of ending life reported with substantial frequency. Age, length of professional experience, and the importance of religion in the physician's life affected the likelihood of reporting of non-treatment decisions. Interpretation A vast majority of neonatologists in European NICUs have been involved in end-of-life limitation of treatments, but type of decision-making varies among countries. Cultur-related and other country-specific factors are more relevant than characteristics of individual physicians or units in explaining such variability.

During the past 20 years the most important indicators of maternal and child health have improved considerably in many parts of the world, with the notable exception of countries affected by the HIV/AIDS epidemic. Nevertheless, it is estimated that out of 130 million babies that are born alive every year, almost 8 million die in their first year of life and more than 4 million die in the first 4 weeks after birth; furthermore, every year over 3,3 million fetuses are stillborn and one third of fetuses die during delivery [1].

The psycho-relational problems in Neonatal Intensive Care Units (NICU) are complex and multifaceted and have only recently been properly addressed. Some specific factors make communication in NICU particularly problematic; the baby's clinical condition, the emotional and working conditions of the medical staff, the emotional state of the parents and the setting of the NICU and the interaction of multiple professional figures with the parents. The purpose of communication in NICUs is not only to inform parents of their child's clinical condition; the medical and nursing staff must also educate and guide parents so that they can actively participate in caring for their child and become true "partners" with the medical team in the decision-making process. Furthermore, the staff must also use their communication skills to understand and contain the anxieties and emotions of parents, supporting and comforting them through the most critical moments of their child's illness and possibly even bereavement. Given the number and complexity of the interpersonal exchanges that take place in the NICU, the risk of misunderstanding, misinterpretation and conflict is high. One could say that the interpersonal aspect is an area where the risk of iatrogenesis is elevated. It is recognized that poor staff-family interactions not only reflect negatively on the baby's care and are a source of distress and discontent for the parents, but are also a major cause of medico-legal litigation and increase the incidence of "burnout". Therefore, specific training of the staff in communication is essential if the optimal results, obtained through modern technology, are not to be invalidated.

'Idiopathic' hyperbilirubinaemia in the first 4 days of life was studied in 431 unselected healthy full-term (gestational age > or = 37 weeks) singleton Sardinian infants with birthweight > or = 2500 g. All infants were free from malformations or any disease requiring treatment other than jaundice, they were ABO and Rh compatible with their mothers and were not G6PD deficient. The serum bilirubin level was > 11.9 mg/dl (204 mumol/l) and > 14.9 mg/dl (256 mumol/l) in 37.1% and 15.3% of the study subjects. The vast majority of the infants (94%) were breast fed; no difference in the incidence of jaundice was found between breast-fed and bottle-fed infants. A logistic regression analysis indicated that high alpha-fetoprotein concentrations in cord blood, history of neonatal jaundice in previous full-term siblings, delayed first meconium passage and weight loss were associated with jaundice, defined as a serum bilirubin level > 11.9 mg/dl. These results suggest that the high rate of neonatal hyperbilirubinaemia in Sardinia is mostly related to constitutional and possibly hereditary factors.

To validate an Italian parental questionnaire designed to evaluate the neuropsychological and behavioural developmental status of 4-year-olds and identify children in need of further evaluation.The questionnaire (Questionario per la valutazione dello Sviluppo di bambini a 4 anni - Genitori (QS4-G) ) consisted of 93 questions divided into 10 areas: language, visual-motor abilities, memory/attention, fine and gross motor and self-help abilities, lateralisation, social skills, stress, sleep, alimentation and evacuation. It was distributed to 263 parents of 4-year-olds: 94 healthy preterm (gestational age33 weeks and/or1500 g, without major neurosensory damage); 44 children with developmental disorders and 125 children with typical development. Cognitive and neuropsychological evaluations were performed using standardised tests.The internal consistency of the areas was adequate (Cronbach's alpha: 0.69-0.79). The correlation coefficients (r=|0.30|-|0.68|) with standardised tests (Griffiths, Vineland and neuropsychological tests) indicated a good concurrent validity. The receiver operating characteristic curve, for predicting a Griffiths Quotient less than 81, showed an area under the curve of 0.90 and a high diagnostic and discriminatory capacity (sensitivity of 0.88 and specificity of 0.84) for the optimal cut-off (value 48.4).The QS4-G seems to be a valid tool for identifying 4-year-old children at risk for low or borderline cognitive development and/or problematic behaviour who need a complete assessment. It can describe individual neuropsychological profiles. QS4-G is not a diagnostic tool. It is useful for outcome studies in preterm children and in other pathologies. It could also be useful for preschooler prevention programmes.

The first article of the Universal Declaration of Human Rights (UN, 1948) reads: “all human beings are born free and equal in dignity and rights”. According to the International Convention on the R...

The availability of novel technologies, such as tandem-mass-spectrometry (MS/MS) and DNA analysis, has expanded tremendously the number of genetic conditions that can be diagnosed through neonatal screening programs at birth, including conditions that cannot be treated nor prevented, or that will become manifest only later in life, or that identify individuals that are only at an increased risk of multifactorial conditions. This has increased the number and complexity of ethical problems related to newborn screening programs, creating considerable confusion and generating controversies and ethical concerns. The experience so far gained indicates that, besides the incomplete knowledge of many aspects of the conditions to be identified, the majority of screening programs do not pay sufficient attention to the problems of communication, information and counselling of the parents. Therefore, communication must be substantially improved if we wish to increase the efficiency of such programs and avoid possible unwanted side effects. Furthermore, ethical issues should receive more attention and consideration for a better and more complete understanding of the overall impact of neonatal screening programs. This more extensive and ethically correct approach should allow us to find an optimal equilibrium between the potential benefits and the possible damages deriving from neonatal screening programs.

Aims: To evaluate to which extent pregnancies of very short duration ( Methods: Area-based study in the 57 maternity units of the Lazio Region (Italy), years 2003–2004, including: 1012 live births (gestational age 22–31 weeks), 261 fetal losses (22–31 weeks) and 209 induced abortions (22–25 weeks). Variables associated with access to a level III unit were evaluated through multivariable logistic regression models. Results: 83.7% of all pregnancies Conclusions: Regionalization of perinatal care in Lazio is not satisfactory. Concentration of high-risk deliveries in level III centers is good, but in utero transfer is insufficient. This study can help to define the effectiveness of different organizational systems on access to locally available perinatal facilities and to optimize general organizational patterns of perinatal care.

An international project (EURONIC) was carried out to explore the end-of-life decision-making process in a large, representative sample of neonatal intensive care units (NICUs) in eight western European countries: France, Germany, Great Britain, Italy, Luxembourg, the Netherlands, Spain and Sweden. Structured questionnaires were used to record data on NICU organization and policies, and to survey staff views and practices regarding ethical decision-making. One hundred and twenty-two NICUs were recruited by census or random sampling (response rate 86%); 1235 physicians and 3115 nurses completed the staff questionnaire (response rates 89 and 85%, respectively). This paper focuses on the physicians' answers. In all countries but Italy, most physicians reported having been involved at least once in setting limits to intensive care because of a baby's incurable condition and/or poor neurological prognosis. Adopted strategies varied between countries. Practices such as the continuation of current treatment without intensifying it and the withholding of emergency manoeuvres appeared widespread. In contrast, the frequency of doctors reporting withdrawal of mechanical ventilation was highest in the Netherlands (93%), Sweden (91%) and the Great Britain (88%), intermediate in France and Germany, and lowest in Spain and Italy (34 and 21%, respectively). Conclusion: Ethically problematic clinical cases are approached differently in the various countries. The findings of this study may provide an opportunity for physicians to review their practices critically, in light of how other colleagues proceed, and foster an open discussion about these difficult issues.

Despite improvements in clinical management, mortality of congenital diaphragmatic hernia (CDH) remains high. Early prediction of mortality risk helps in comparing strategies and/or performances of different centers. Birth weight (BW), Apgar Score at 5 minutes, and modified McGoon Index (MGI) calculated by the ratio between the diameters of pulmonary arteries and the descending aorta have been used to determine mortality of CDH.The purpose of this study is to evaluate the relationship between early detectable variables and survival in newborns with CDH intubated at birth, managed with "gentle" ventilation and delayed surgery.All medical records of patients affected by high-risk CDH and treated with a standardized protocol at Bambino Gesù Children's Hospital, Rome, Italy, between January 2002 and September 2004 were reviewed. Prenatal diagnosis, gestational age, BW, sex, side of hernia, and MGI were recorded on admission. The relationship with mortality of each variable was evaluated by univariate analysis. Subsequently, a predictive model of mortality was developed using a logistic regression: the explanatory variables, BW, and MGI were dichotomized in high (HBW and HMGI) and low (LBW and LMGI) according to the best cutoff found with receiver-operating characteristic curves.Thirty-four newborns with CDH, treated with a standardized protocol, were studied. The main characteristics of the 34 patients were BW, 2886 g (1500-3620 g); gestational age, 37.7 weeks (32-42 weeks); male/female, 22/12; right/left, 8/26; prenatal diagnosis, 29; MGI, 1.31 (0.9-1.85). Only BW and MGI were significantly (P.05) associated with mortality at the univariate analysis. The best cutoff values were 2755 g for BW (sensitivity, 70%; specificity, 74%) and 1.25 for MGI (sensitivity, 73%; specificity, 78%). Using these limits, BW and MGI resulted independently associated with mortality in the multivariate analysis. Using the 4 possible combinations, the LBW associated with the LMGI presented the highest prediction of mortality (80%).Birth weight and MGI, variously combined, were predictive of mortality. Because they are not influenced by subsequent modalities of care, they can be considered as valid early severity scores in CDH and used for comparing strategies and/or performances of different centers.

Background Cardiorespiratory stabilization is recommended before surgical repair of congenital diaphragmatic hernia (CDH) because surgery may induce a transitory deterioration of chest compliance and gas exchange. It is not known if surgical intervention can affect cerebral circulation and oxygenation. Aim The aim of the study was to assess noninvasively, by near-infrared spectroscopy, the possible changes in cerebral hemodynamics and oxygenation associated with surgical repair of CDH. Subjects Twenty-five newborns with severe CDH (birth weight, 3057 ± 354 g; gestational age, 37.8 ± 1.8 weeks; male/female newborns, 15/10; left/right CDH, 19/6) were sedated, paralyzed, and mechanically ventilated by conventional gentle ventilation and surgically corrected at a median age of 2.7 days (min-max, 2-14 days) after cardiorespiratory stabilization. Methods Heart rate (HR [beats per minute]), preductal transcutaneous oxygen saturation (tcSao 2 [%]), carbon dioxide tension (tcPco 2 [Torr]), and mean arterial blood pressure (mm Hg) were continuously monitored. Inspired fractional oxygen concentration (Fio 2 ) was adjusted to maintain and preductal tcSao 2 of greater than 80%, whereas the ventilator's settings were kept unchanged throughout the surgical procedure. Cerebral hemodynamics was assessed by near-infrared spectroscopy (NIRO 300, Hamamatsu Photonics, Japan), recording continuously and noninvasively the relative changes in concentration of oxygenated ( Δ O 2 Hb [ μ mol/L]), deoxygenated ( Δ HHb [ μ mol/L]), and total ( Δ tHb [ μ mol/L]) hemoglobin; the tissue oxygenation index (TOI [%]) was also calculated (TOI = O 2 Hb/O 2 Hb + HHb). Total hemoglobin concentration is considered to be representative of cerebral blood volume. Arterial blood gases were also measured at the beginning ( T 1 ) and at the end of surgery ( T 2 ). For all measurements, results at T 1 and at T 2 , as well as the differences between T 1 and T 2 , have been expressed as means or medians and SDs or 95% confidence intervals or ranges. The differences between T 1 and T 2 were considered statistically significant for a P value of less than .05 by the Student t test for paired values. Results At T 1 , mean tcSao 2 % was 94.1 % (SD, 4.6) with a Fio 2 of 0.25 (SD, 0.1); at T 2 , to obtain similar values of tcSao 2 (93.4%; SD, 4.4), it was necessary to increase the Fio 2 to 0.37 (SD, 0.14; P T 1 was 149.5 beats per minute (SD, 9.1) and increased significantly ( P T 2 (165.2 beats per minute; SD, 14.2). Mean arterial blood pressure was 54.7 mm Hg (SD, 7.7) at T 1 and did not change appreciably at T 2 (55.6 mm Hg; SD, 8.1). Moreover, tcPco 2 did not change significantly during the procedure (mean tcPco 2 = 49.9 Torr [SD, 12.8] at T 1 and 57.3 mm Hg [SD, 17.9] at T 2 ). O 2 Hb and tHb decreased ( P P Δ [SD]: Δ O 2 Hb= −10.9 μ mol/L [9.7], Δ tHb = −7.5 μ mol/L [11.7], and Δ HHb = −3.5 μ mol/L [6.8]). Mean TOI was 70% at T 1 (normal values >60%) and decreased significantly at T 2 (mean Δ TOI = −6.1% [SD, 10.6]). In all infants, the greatest changes occurred when the viscera were positioned into the abdomen. Conclusions Notwithstanding the initial cardiorespiratory stabilization, surgical repair of CDH was associated with a rise in HR and oxygen requirement and a drop in cerebral tHb and O 2 Hb, suggesting a reduction in cerebral blood volume and oxygenation. These events were probably due to the combined effects of an increase in right to left shunting (as indicated by the increased oxygen requirement) and a decrease in venous return (possibly due to compression of the inferior vena cava by the viscera positioned into the abdomen). These preliminary results reinforce the importance of achieving a good cardiorespiratory stability before undertaking surgical correction of CDH to minimize the possible interference of the procedure with cerebral circulation and oxygenation.

Treatment of long gap esophageal atresia (EA) is still a major challenge. Gastric transposition and colon interposition are the 2 most popular choices for esophageal replacement, but there is general agreement that the child's own esophagus is the best. The aim of the study was to critically evaluate the feasibility and outcome of primary repair of long gap EA with or without tracheoesophageal fistula (TEF) by direct esophago-esophageal anastomosis as the only technique.Seventy-one neonates with EA+/-TEF were considered. Nineteen cases were classified as long gap (or =3 cm). All infants underwent either primary or shortly delayed repair. In the latter group, a gastrostomy was performed along with an x-ray evaluation of the gap a few days before surgery (mean age, 46.4 days). To avoid disruptive anastomotic force, all infants were kept paralyzed and mechanically ventilated for an additional 6 days after esophageal anastomosis. Before starting feeding, postoperative esophagogram was done on day 7. Endoscopy was done routinely, starting 1 month after surgery; pH monitoring was conventionally performed at 1 year of age or even earlier, should gastroesophageal reflux disease (GERD) be suspected. Follow-up ranged from 11 months to 7 years.In all 19 long gap EA infants an esophago-esophageal anastomosis was performed. Six of them (31%) required an anterior esophageal flap to bridge residual gap. Complications included minor anastomotic leak in 2 cases and anastomotic stricture (5mm) in 12 (80%) cases, which were treated with an average of 5 dilatations (1 of which with resection of the stricture). GERD occurred in 8 cases (53.3%), of which, 3 required fundoplication. None of the patients had esophageal swallowing difficulties or persistent dysphagia. Two children experienced food aversion. Mean hospital stay was 66.2 (22 to 230) days. There were 4 deaths (very low birth weight, 1; associated anomalies, 1; and late sepsis, 2).Considering heat gap determination remains imprecise, it seems possible to conclude that in a well-established tertiary care level referral center: (1) long gap EA could be treated successfully with primary repair and anastomosis; (2) strictures and GER represent the most frequent postoperative problem, but additional procedures required seem "acceptable" to maintain the patient's own esophagus and avoid replacement; (3) esophageal substitution in long gap EA should be reserved for cases in which a previous attempt of esophageal reconstruction failed.

Contains fulltext : 57784.pdf (Publisher’s version ) (Closed access) OBJECTIVE: To present the views of a representative sample of neonatal doctors and nurses in 10 European countries on the moral acceptability of active euthanasia and its legal regulation. DESIGN: A total of 142 neonatal intensive care units were recruited by census (in the Netherlands, Sweden, Hungary, and the Baltic countries) or random sampling (in France, Germany, Italy, Spain, and the United Kingdom); 1391 doctors and 3410 nurses completed an anonymous questionnaire (response rates 89% and 86% respectively). MAIN OUTCOME MEASURE: The staff opinion that the law in their country should be changed to allow active euthanasia "more than now". RESULTS: Active euthanasia appeared to be both acceptable and practiced in the Netherlands, France, and to a lesser extent Lithuania, and less acceptable in Sweden, Hungary, Italy, and Spain. More then half (53%) of the doctors in the Netherlands, but only a quarter (24%) in France felt that the law should be changed to allow active euthanasia "more than now". For 40% of French doctors, end of life issues should not be regulated by law. Being male, regular involvement in research, less than six years professional experience, and having ever participated in a decision of active euthanasia were positively associated with an opinion favouring relaxation of legal constraints. Having had children, religiousness, and believing in the absolute value of human life showed a negative association. Nurses were slightly more likely to consider active euthanasia acceptable in selected circumstances, and to feel that the law should be changed to allow it more than now. CONCLUSIONS: Opinions of health professionals vary widely between countries, and, even where neonatal euthanasia is already practiced, do not uniformly support its legalisation.

We compared the effectiveness of a single dose and a three-day course of antibiotic prophylaxis in preventing bacterial infections in high-risk neonates. The study was a prospective, randomized controlled trial conducted in a 20-bed tertiary referral neonatal intensive care unit (NICU). A series of 130 neonates admitted consecutively to the NICU, fulfilling risk factors for infection, were assigned at random to receive intravenous antibiotic prophylaxis with ampicillin and netilmicin either in two daily doses for 72 h (three-day-administration group, 67 infants) or in a single bolus injection on admission (bolus group, 63 infants). Hospital-acquired infection, the main outcome measure, was defined as infection that developed at least 48 h after admission, and vertical infection (maternally transmitted) was considered to be present when clinical symptoms and abnormal laboratory findings became evident within 48 h of birth. Infections were considered as suspected when clinical and laboratory findings of infection were present, without positive cultures, and as confirmed when positive cultures were also present. No significant differences were found between the two groups of neonates studied in mean birth weight, gestational age or postnatal age on admission. The incidence of vertical infection was similar in the two groups (16/67, 23.9% vs. 14/63, 22.2%). Of the 130 newborns studied, 29 (22.3%) acquired at least one nosocomial infection during their NICU stay; total hospital-acquired infections, calculated as the incidence density of infection (the number of infective episodes divided by the number of days in the NICU), were less frequent among newborns who received the three-day course than the bolus (relative risk 0.69). This difference, although not statistically significant, depended on the different incidence density of confirmed nosocomial infections rather than on suspected infections (relative risk 0.59; 95% confidence interval 0.32-1.09; P=0.1). There were no significant differences between the two groups in overall mortality. A single bolus administration on admission is therefore likely to be as effective as a three-day course of antibiotic prophylaxis in preventing bacterial infection in high-risk infants admitted to an NICU.

Objectifs: 1) Definir le meilleur devenir des hernies congenitales diaphragmatiques severes (CDH). 2) Evaluer de maniere critique les deces pour identifier de possible criteres d'exclusion de l'ECMO. 3) Identifier les CDH qui pourraient beneficier de l'ECMO. Materiels et methodes: 63 hernies de la coupole diaphragmatique considerees comme severes: 35 (55,6%) survivants et 28 (44,4%) non survivants divises en 2 groupes en fonction de l'âge du deces: groupe I, deces a moins de 24 heures et groupe Il, deces au-dela de 24 heures. Les 3 groupes etaient compares en terme de diagnostic prenatal, de polyhydramnios, d'âge gestationnel, de poids de naissance, de pneumothorax, des meilleures valeures de la PaCO 2 et de la PaO 2 post-ductale et de signes cliniques et electro-cardiographiques d'une hypertension arterielle pulmonaire persistante et de la severite de l'hypoplasie pulmonaire (rapport du poids corporel au poids pulmonaire a l'autopsie). Resultats: PaCO 2 , PaO 2 et le degre de l'hypoplasie pulmonaire sont significativement plus graves dans le groupe I compare au groupe Il et aux survivants. PaCO 2 et PaO 2 dans le groupe II ne different pas significativement de ceux des survivants. Conclusion: Dans les hernies de coupole diaphragmatiques, il est possible: 1) d'obtenir un taux de survie de 56% sans ECMO, 2) d'identifier des patients (groupe I = 27%) avec hypoplasie pulmonaire, qui mourront meme avec l'ECMO, 3) d'identifier des patients (groupe II = 17%) qui pourraient beneficier de l'ECMO.

We investigated the prognostic indicators in ten hyperammonemic neonates: four treated by continuous arteriovenous hemodialysis (CAVHD), four with continuous venovenous hemodialysis (CVVHD), and two with hemodialysis (HD). Plasma ammonium levels decreased significantly within the first 24 h irrespective of dialysis modality (from 1419 to 114 micromol/l, median values; P0.0001). CVVHD achieved the highest ammonium clearance. HD provided highest ammonium extraction but clearance was hampered by severe hemodynamic instability. Five patients had a good outcome (normal at follow-up of 9-59 months), five had poor outcome (four died and one has severe neurological damage). Total coma duration was shorter in patients who had a good outcome (47+/-11 vs 78+/-13 h; P=0.02). Remarkably, only coma duration before dialysis determined this difference (22.2+/-10.1 vs 48.8+/-11.2 h; P=0.02). In cases with good outcome, coma duration was33 h, whereas the others exceeded this limit. The prognosis was not related to dialysis modality, rapidity in reducing ammonium levels or to the underlying metabolic defect. In conclusion, results showed CVVHD to be the optimal modality for extracorporeal ammonium detoxification. However, the most relevant indicator for prognosis was coma duration before the start of dialysis. Therefore, major efforts should be made to refer patients quickly to highly specialized centers.

As care improves many neonates with life-threatening disorders now survive. Nonetheless Nosocomial Infections (NI) are still a major cause of morbidity and mortality in NICUs. A prospective multicentric surveillance study was conducted in six Italian NICUs to describe the epidemiologic profile and determinants of NI in NICU. 1692 neonates, consecutively admitted to the NICUs from July 2000 to October 2002 and monitored for the development of NI were enrolled into the study. The standard definition criteria for NI formulated by the Centers for Disease Control in Atlanta were used. The cumulative probability and hazard ratios (HR) for the first episode of infection were estimated by the Kaplan-Meier method and the Cox model. A total of 217 neonates had 255 episodes of NI. The incidence rate of NI was 7 per 1000 patient-days. The cumulative probability of first infection was 20% (95% CI, 7.50–23.30) and 27.6% (95 CI, 23.20–32.80) at 30 and 60 days after admission to the NICU. After adjustment for the severity of illness, the main risk factors related to NI in very-low-birth-weight neonates (VLBW) were surgical procedures (HR 2.69;95% CI 0.60–12.08), nasal ventilation (CPAP) (HR, 2.51; 95% CI, 0.93–6.76), continuous enteral feeding (HR 1.89; 95% CI, 0.20–17.50), mechanical ventilation (HR, 1.70; 95%CI, 0.72–4.00) and intravenous infusions (HR 1.46; 95% CI, 0.32–6.52). Among neonates with a birth weight over 1500 g, risk factors for NI were parenteral nutrition with lipid emulsion (HR, 12.41; 95% CI, 4.19–36.78), surgical procedures (HR 2.78; 95% CI, 0.82–9.44), and intravenous infusions (HR, 2.63; 95% CI, 0.27–25.53). Risk factors for NI were related more to the severity of illness than to healthcare procedures in VLBW babies and more to medications among neonates weighing more than 1500 g at birth.

60 Birthweight and Mcgoon Index Predict Outcome in Newborn Infants with Congenital Diaphragmatic Hernia (CDH).

Background: CDH is still characterized by high morbidity and mortality, mostly due to lung hypoplasia. Recently, gentle ventilation and delayed surgery have been suggested to improve mortality and long-term pulmonary sequelae. The assessment of lung volumes, particularly functional residual capacity (FRC), is crucial for understanding lung development during the first year of life. The sulphur hexafluoride (SF6) wash-in / wash-out technique together with an ultrasonic flow meter, can measure both FRC and lung clearance index (LCI), a very sensitive and stable index of ventilation homogeneity. Aim: To evaluate changes in lung function during the first two years of life in infants with CDH requiring mechanical ventilation and surgically corrected in the neonatal period. Methods: Ten CDH infants (means±SD: BW=3082±570 g and GA=39.1±1.7 wks, M/F=8/2, Left/Right CDH 8/2) surgically corrected in the neonatal period were studied twice during the first two years of life, with at least six months interval between measurements. Tidal Volume (Vt), Respiratory Rate (RR) and time to peak expiratory flow/expiratory time ratio (tPTEF/Te, an index of lower airways patency) were measured with an ultrasonic flow meter; Compliance (Crs) and Resistance (Rrs) of the respiratory system were studied with the single occlusion technique; FRC and LCI were assessed with the multiple breath SF6 wash-in/wash-out technique. The differences between the first and second measurement were considered statistically significant for p

Background: MBL is a serum lectin, synthesised by the liver, that is part of the innate immune system; it behaves as an acute phase reactant and plays an auxiliary rather than a critical role in host defence. Individuals with MBL deficiency may experience an increased number of infections, particularly in the course of immunodeficiency (e.g. chemotherapy). A MBL concentration of 0.5 mcg/ml has been suggested as a cut-off level for MBL deficiency. Serum MBL levels in term neonates are similar to those in adults, but values in preterms are approximately 50% lower. HAI are an important cause of neonatal morbidity and mortality. Known risk factors account only in part of the (inter)individual variability of the frequency and severity of neonatal infections. Part of this variability could be related to individual differences in the maturation of the innate immune system.

202 Cerebral Haemodynamics and Lung Mechanics in Preterm Infants During Synchronized Intermittent Mandatory Ventilation (SIMV) and Synchronized Intermittent Positive Pressure Ventilation (SIPPV), with and Without Volume Guaranteed (VG)

EDITOR—Murray et al include pain relief for dying people among the essential health interventions that should be accessible for all, in developing countries.1 Even developed countries, however, have different pain relief policies. In Italy, where medicinal morphine consumption for 2000 ranked among the lowest in Europe,2 a …

Modulation of lung matrix metalloproteinases (MMPs) and their inhibitors (TIMPs) through the GTP-dependent signal transduction pathway

The Effects of Broncho-Alveolar Lavage (BAL) on Lung Mechanics in Ventilated Newborns Infants