Prematurity is becoming a real public health issue as more and more children are being born prematurely, alongside a higher prevalence of neurodevelopmental disorders. Early intervention programs in Neonatal Intensive Care Units (NICUs) correspond to these uni- or multi-sensorial solicitations aiming to prevent and detect complications in order to support the development of preterm infants. This article aims to distinguish sensory intervention programs according to the gradient of the type of solicitations, uni- or multi-modal, and according to the function of the person who performs these interventions. Uni-sensorial interventions are essentially based on proprioceptive, gustatory, or odorant solicitations. They allow, in particular, a reduction of apneas that support the vegetative states of the preterm infant. On the other hand, the benefits of multi-sensory interventions seem to have a longer-term impact. Most of them allow the support of the transition from passive to active feeding, an increase in weight, and the improvement of sleep-wake cycles. These solicitations are often practiced by caregivers, but the intervention of parents appears optimal since they are the main co-regulators of their preterm child’s needs. Thus, it is necessary to co-construct and train the parents in this neonatal care.
The sequelae of extremely preterm birth have an impact on the quality of life (QoL) of these children. Standardized assessment of their QoL is rarely done in France. The aim of this study is to examine among all the types of physicians involved in the management of children born extremely preterm, their knowledge, use in routine practice and expectations concerning QoL assessment of these children using standardized questionnaires.
Generalized Arterial Calcification of Infancy (GACI) is a heritable ectopic mineralization disorder resulting in diffuse arterial calcifications and/or stenosis, mostly caused by mutations in the ENPP1 gene. Here we present a case report of GACI in a male infant with a new familial mutation of the ENPP1 gene and the clinical outcome after biphosphonates therapy. The clinical presentation was characterized by a severe early-onset of hypertension refractory to multiple therapy. To investigate this atypical hypertension, a renal Doppler ultra-sonography was performed and diffuse echo-bright arteries were detected; then a low-dose whole-body computed tomography demonstrated extensive arterial calcifications, suggesting GACI. A novel homozygous mutation c.784A > G (p.Ser262Gly) was detected in the ENPP1 gene. The infant was administered four courses of bisphosphonates: arterial calcifications were found to decrease but severe refractory hypertension was persistent. Although GACI can be a rapidly fatal illness and frequently results in death in infancy, the patient was 24 months of age at the time of writing this report. Three points of interest: the first one is to remind clinicians of this rare and atypical etiology in neonates with severe hypertension and in fetuses with cardiomyopathy and non-immune hydrops fetalis. The second point is the identification of a novel mutation in the ENPP1 gene associated with a clinical presentation of GACI. The third point is the fairly favourable outcome of our patient after bisphosphonates therapy, with calcifications regression but not hypertension.
Objective: To evaluate, in very preterm infants, the hemoglobin (Hb) levels during the first 24 h and the neurodevelopment outcomes at 24 months of corrected age. Design, setting, and patients: We conducted a secondary analysis of the French national prospective and population-based cohort EPIPAGE-2. The eligible study participants were live-born singletons who were born before 32 weeks of gestational age, with early Hb levels who were admitted to the neonatal intensive care unit. Main outcome measures: The early Hb levels for an outcome survival at 24 months of corrected age without neurodevelopmental impairment were measured. The secondary outcomes were survival at discharge and without severe neonatal morbidity. Results: Of the 2158 singletons of 26 weeks with Hb of <15.5 g/dL (n = 703) were associated with a poor outcome at 24 months (OR 1.9; CI: [1.5–2.4] p < 0.01). Conclusions: Early low Hb levels are associated with major neonatal morbidities in VP singletons, but not with neurodevelopment outcomes at two years of age, except in male infants of >26 Weeks GA.
The outcome of very preterm infants is marked by the development of complications that can have an impact on the quality of life of the children and their families. The concept of quality of life and its evaluation in the long term raise semantic and ethical problems for French physicians in perinatal care. Our reflection aims to gain a better understanding of the representations surrounding quality of life in neonatal medicine. If French physicians hesitate to face this concept (through self-interest and apprehension), it is because the debate has become more complex. Formerly, the dilemma was between respect for life versus quality of life. Today, although this dilemma is still with us, the questions raised by French physicians show us that autonomy is given increasing importance. The equation to be solved now contains three variables: respect for life, well-being, autonomy. So we find ourselves between three positions and no longer two: respect for life (the ethics of conviction), quality of life based on autonomy (rationalist and secular deontologism), and quality of life based on the differential between well-being and suffering (utilitarianism). A solution could lie in consequentialism, which integrates the consequences for future generations in terms of both safeguarding of autonomy and quality of life, and puts the sacredness of life in second place but without sacrificing it. By evaluating their future quality of life, we can better respond to the needs of these children.