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Background Perinatal palliative care (PPC) is increasingly recognized as an essential component of quality maternity and neonatal care. Despite international calls for structured programs, little is known about how PPC is provided in everyday healthcare systems and how parents and professionals experience it. This study explored PPC practices in Flanders (Belgium), focusing on challenges and opportunities for improving care. Methods We conducted a qualitative study using semi-structured face-to-face interviews with healthcare professionals experienced in PPC, and with parents who had lost a child in the perinatal period. A total of 22 healthcare professionals and 18 parents participated. Framework analysis was employed to systematically analyze the interview data. The findings were compared with the results of a recent systematic review which synthesized existing literature and outlined the key components of PPC. Results In the absence of a formal PPC team, considerable effort was required to coordinate care and ensure effective information transfer between hospitals and departments. Palliative care training was largely informal, with most learning occurring on the job. In contrast to findings reported in the international literature, interview data revealed that external organizations were frequently and systematically involved in PPC. The recognizability of healthcare providers was identified as a key factor in parental psychosocial support. Parents’ expressions of gratitude, respect, and satisfaction were experienced by caregivers as highly supportive. In terms of shared decision-making, several parents expressed a need for clearer explanations of medical scenarios, more directive support from physicians, and early exploration of their preferred decision-making style. Simultaneously, both parents and professionals reported feeling limited by legal constraints. Additional needs included improved communication amongst all involved, greater structural support and infrastructure adapted to palliative care needs. Conclusions Both parents and healthcare providers pointed out the need for a multidisciplinary team to coordinate PPC across time and disciplines. Parents expressed more dissatisfaction with communication than with the purely medical aspects of care. The importance of caregiver introductions and role clarity, dedicated PPC training and experienced staff was stressed. A neutral, non-directive approach to decision-making did not suit all parents, underscoring the need to tailor support to individual preferences.

Background Perinatal palliative care (PPC) provides essential support for families and healthcare providers at the end-of-life. Despite clear global need, structured PPC programs are not widely established. Aim To develop a PPC program that supports families confronted with a severe perinatal diagnosis for their (unborn) child, with a focus on evidence-based development of such program, ensuring care is effective, consistent, and aligned with best practices. Methods We systematically designed a PPC program following a 7-step method, using Bleijenberg’s extended Medical Research Council framework. We conducted a qualitative interview study with 22 healthcare providers and 18 bereaved parents from six hospitals to identify care gaps alongside an integrative review study of international programs. These combined findings informed the development of a PPC prototype program, intended to be integrated in routine practice. Subsequently, we refined the program, identified barriers to implementation, and explored opportunities for contextual tailoring through six participatory workshops involving both healthcare providers and bereaved parents at three hospitals. In these sessions we reviewed the preliminary care components, identified practical challenges, and adapted the model to align with local workflows, staffing realities, and documentation systems. Results Five core components were developed: Dedicated fixed PPC Team with care coordinators Formal/Specialized 1,5 day training covering essential PPC aspects Stepwise PPC Approach with a structured PPC plan including individualized care pathways and centralized resources and information sharing Proactive psychological support for both families and staff Structured Team Debriefings Key barriers to implementation included fragmented documentation systems, limited staffing and time resources, and challenges in cross-departmental coordination. Discussion We developed a PPC program based on existing international evidence and current PPC practices. This approach ensured both relevance and practical applicability. The program design is a strength, as it is grounded in evidence and shaped through active involvement of parents and healthcare providers, with attention to contextual tailoring. The program is now ready for pilot testing to assess feasibility and acceptability in clinical practice. Future implementation will require institutional support, contextual adaptation, and ongoing evaluation to ensure sustainable integration into perinatal care.

Background End-of-life decisions with potential life-shortening effect in neonates and infants are common. We aimed to evaluate how often and in what manner neonatologists consult with parents and other healthcare providers in these cases, and whether consultation is dependent on the type of end-of-life decision made. Methods Based on all deaths under the age of one that occurred between September 2016 and December 2017 in Flanders, Belgium, a nationwide mortality follow-back survey was performed. The survey asked about different types of end-of-life decisions, and whether and why parents and/or other healthcare providers had or had not been consulted. Results Response rate was 83% of the total population. End-of-life decisions in neonates and infants were consulted both with parents (92%) and other healthcare providers (90%), and agreement was reached between parents and healthcare providers in most cases (96%). When medication with an explicit life-shortening intent was administered parents were always consulted prior to the decision; however when medication without explicit life-shortening intention was administered parents were not consulted in 25% of the cases. Conclusions Shared decision-making between parents and physicians in case of neonatal or infant end-of-life decision-making is the norm in daily practice. All cases without parental consultation concerned non-treatment decisions or comfort medication without explicit life-shortening intention where physicians deemed the medical situation clear and unambiguous. However, we recommend to at least inform parents of medical options, and to explore other possibilities to engage parents in reaching a shared decision. Physicians consult other healthcare providers before making an end-of-life decision in most cases.

Background The death of a child before or shortly after birth is frequently preceded by an end-of-life decision (ELD). Population-based studies of incidence and characteristics of ELDs in neonates and infants are rare, and those in the foetal-infantile period (> 22 weeks of gestation – 1 year) including both neonates and stillborns, are non-existent. However, important information is missed when decisions made before birth are overlooked. Our study protocol addresses this knowledge gap. Methods First, a new and encompassing framework was constructed to conceptualise ELDs in the foetal-infantile period. Next, a population mortality follow-back survey in Flanders (Belgium) was set up with physicians who certified all death certificates of stillbirths from 22 weeks of gestation onwards, and infants under the age of a year. Two largely similar questionnaires (stillbirths and neonates) were developed, pilot tested and validated, both including questions on ELDs and their preceding decision-making processes. Each death requires a postal questionnaire to be sent to the certifying physician. Anonymity of the child, parents and physician is ensured by a rigorous mailing procedure involving a lawyer as intermediary between death certificate authorities, physicians and researchers. Approval by medical societies, ethics and privacy commissions has been obtained. Discussion This research protocol is the first to study ELDs over the entire foetal-infantile period on a population level. Based on representative samples of deaths and stillbirths and applying a trustworthy anonymity procedure, the research protocol can be used in other countries, irrespective of legal frameworks around perinatal end-of-life decision-making.

Parenteral nutrition (PN) is a standard of care for preterm infants in the first postnatal days. The European Society of Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) has updated their guideline recommendations on PN in 2018. However, data on actual 2018 guideline adherence in clinical practice are sparse. In this retrospective study, conducted at the neonatal intensive care unit (NICU) of Ghent University Hospital, we analyzed the ESPGHAN 2018 PN guideline adherence and growth for 86 neonates admitted to the NICU. Analyses were stratified by birth weight (<1000 g, 1000 to <1500 g, ≥1500 g). We documented the provisions for enteral nutrition (EN) and PN, and we tested the combined EN and PN provisions for ESPGHAN 2018 adherence. The nutrition protocols showed a high adherence to PN guidelines in terms of carbohydrate provisions, yet lipid provisions for EN and PN often exceeded the recommended maximum of 4 g/kg/d; although, PN lipid intakes maxed out at 3.6 g/kg/d. Protein provisions tended to fall below the recommended minimum of 2.5 g/kg/d for preterm infants and 1.5 g/kg/d for term neonates. The energy provisions also tended to fall below the minimum recommendations, especially for neonates with a birth weight (BW) < 1000 g. Over a mean PN duration of 17.1 ± 11.4 d, the median weekly Fenton Z-scores changes for length, weight, and head circumference were positive for all BW groups. Future studies have to assess how protocols adapt to current guidelines, and how this affects short- and long-term growth across different BW groups. In conclusion, the reported findings provide real-world evidence regarding the effect of ESPGHAN 2018 PN guideline adherence, and they demonstrate how standardized neonatal PN solutions can safeguard stable growth during NICU stays.

This study shows an association between a broad range of phenotypes and either deletion or duplication of a genomic segment at chromosome 1q21.1, suggesting a fundamental role of the deletion or duplication in early development and challenging the notion that a specific mutation disposes toward a specific disorder or syndrome. This study shows an association between a broad range of phenotypes and either deletion or duplication of a genomic segment at chromosome 1q21.1, suggesting a fundamental role of the deletion or duplication in early development. Recent advances in technologies such as comparative genomic hybridization (CGH; see Glossary) allow for the routine detection of submicroscopic deletions and duplications. Several studies of persons with mental retardation or congenital anomalies of unknown cause have led to the identification of new genomic disorders. 1 – 10 Classically, criteria that have been applied to determine whether a given rearrangement is causative include de novo appearance of the deletion or duplication in an affected individual (i.e., it is not present in unaffected parents), recurrence of the same or an overlapping event in similarly affected persons, and absence of the deletion or duplication in . . .

Critically ill neonates present clinical and ethical challenges. The deaths of these infants are often preceded by possibly life-shortening end-of-life decisions (ELD), including non-treatment decisions or pain and/or symptom relief medication. Recent empirical information about this practice is scarce.

ObjectivesNeonatology has undergone important clinical and legal changes; however, the implications for end-of-life decision-making in seriously ill neonates to date are unknown. Our aim was to examine changes in prevalence and characteristics of end-of-life decisions (ELDs) in neonatology.MethodsWe performed a nationwide mortality follow-back survey in August 1999 to July 2000 and September 2016 to December 2017 in Flanders, Belgium. Data were linked to information from death certificates. For each death under the age of 1, physicians were asked to complete an anonymous questionnaire about which ELDs were made preceding death.ResultsThe response rate was 87% in 1999–2000 (253/292) and 83% in 2016–2017 (229/276). The proportion of deaths of infants born before 26 weeks’ gestation was increased (14% vs 34%, p=0.001). Prevalence of ELDs remained stable at 60%, with non-treatment decisions occurring in about 35% of all deaths. Use of medication with an explicit life-shortening intention was prevalent in 7%–10% of all deaths. In early neonatal death (<7 days old) medication with an explicit life-shortening intention decreased from 12% to 6%, in late neonatal death (7–27 days old), it increased from 0% to 26%, and in postneonatal death (>27 days old), it increased from 2% to 10%.ConclusionsOver a timespan of 17 year, the prevalence of neonatal ELDs has remained stable. A substantial number of deaths was preceded by the intentionally hastening of death by administrating medication. While surveying solely the physician perspective in this paper, there is a need for an open multidisciplinary debate, including, for example, nursing staff and family members, based on clinical as well as ethical and jurisdictional reflections to discuss the need for international guidelines.

Congenital high airway obstruction syndrome (CHAOS) is a rare prenatal diagnosis consisting of a typical fetal triad of large hyperechogenic lungs, flattened or inverted diaphragms and ascites. Most cases are sporadic with unknown incidence. Before attempts of fetoscopic fetal salvage or ex utero intrapartum treatment (EXIT) are considered, additional malformations must be carefully excluded as CHAOS may be part of various monogenic conditions or chromosomal disorders. We report an unique family with autosomal dominant inheritance of CHAOS and variable expression in the affected father and two affected children. It is concluded that minor expression in one of the parents may be an important indicator for genetic counseling in CHAOS and management of future pregnancies.

This study provides evidence for a critical role of RIPK1 in suppressing caspase-8-mediated cell death and maintaining intestinal homeostasis independently of its kinase activity. RIPK1 both activates and inhibits cell death Receptor-interacting protein 1 kinase (RIPK1) is involved in the activation of various cell death pathways and in the control of inflammatory signalling. Two separate groups reporting in this issue use contrasting techniques to show that as well as promoting cell death, RIPK1 has a paradoxical function in supporting the survival of mouse epithelial cells that is independent of its kinase function. RIPK1 suppresses epithelial cell apoptosis and necroptosis by preventing FADD/caspase-8-mediated apoptosis and RIPK3-dependent necroptosis. These findings, together with genetic data, suggest that RIPK1 is a master regulator of epithelial cell survival, homeostasis and inflammation in the intestine and the skin. Receptor interacting protein kinase 1 (RIPK1) has an essential role in the signalling triggered by death receptors and pattern recognition receptors 1 , 2 . RIPK1 is believed to function as a node driving NF-κB-mediated cell survival and inflammation as well as caspase-8 (CASP8)-dependent apoptotic or RIPK3/MLKL-dependent necroptotic cell death. The physiological relevance of this dual function has remained elusive because of the perinatal death of RIPK1 full knockout mice 3 . To circumvent this problem, we generated RIPK1 conditional knockout mice, and show that mice lacking RIPK1 in intestinal epithelial cells (IECs) spontaneously develop severe intestinal inflammation associated with IEC apoptosis leading to early death. This early lethality was rescued by antibiotic treatment, MYD88 deficiency or tumour-necrosis factor (TNF) receptor 1 deficiency, demonstrating the importance of commensal bacteria and TNF in the IEC Ripk1 knockout phenotype. CASP8 deficiency, but not RIPK3 deficiency, rescued the inflammatory phenotype completely, indicating the indispensable role of RIPK1 in suppressing CASP8-dependent apoptosis but not RIPK3-dependent necroptosis in the intestine. RIPK1 kinase-dead knock-in mice did not exhibit any sign of inflammation, suggesting that RIPK1-mediated protection resides in its kinase-independent platform function. Depletion of RIPK1 in intestinal organoid cultures sensitized them to TNF-induced apoptosis, confirming the in vivo observations. Unexpectedly, TNF-mediated NF-κB activation remained intact in these organoids. Our results demonstrate that RIPK1 is essential for survival of IECs, ensuring epithelial homeostasis by protecting the epithelium from CASP8-mediated IEC apoptosis independently of its kinase activity and NF-κB activation.