Noonan syndrome and pregnancy outcomes

Background: Noonan syndrome is a genetic disorder with high prevalence of congenital heart defects, such as pulmonary stenosis, atrial septal defect and hypertrophic cardiomyopathy. Scarce data exists regarding the safety of pregnancy in patients with Noonan syndrome, particularly in the context of maternal cardiac disease. Study design: We performed a retrospective chart review of patients at Yale-New Haven Hospital from 2012 to 2020 with diagnoses of Noonan syndrome and pregnancy. We analysed medical records for pregnancy details and cardiac health, including echocardiograms to quantify maternal cardiac dysfunction through measurements of pulmonary valve peak gradient, structural heart defects and interventricular septal thickness. Results: We identified five women with Noonan syndrome (10 pregnancies). Three of five patients had pulmonary valve stenosis at the time of pregnancy, two of which had undergone cardiac procedures. 50% of pregnancies (5/10) resulted in pre-term birth. 80% (8/10) of all deliveries were converted to caesarean section after a trial of labour. One pregnancy resulted in intra-uterine fetal demise while nine pregnancies resulted in the birth of a living infant. 60% (6/10) of livebirths required care in the neonatal intensive care unit. One infant passed away at 5 weeks of age. Conclusions: The majority of mothers had pre-existing, though mild, heart disease. We found high rates of prematurity, conversion to caesarean section, and elevated level of care. No maternal complications resulted in long-term morbidity. Our study suggests that women with Noonan syndrome and low-risk cardiac lesions can become pregnant and deliver a healthy infant with counselling and risk evaluation.

Organization and management of nursing services in NICU, levels of transport

Neonatal Intensive Care is defined as, “care for medically unstable and critically ill newborns requiring constant nursing, complicated surgical procedures, continual respiratory support, or other intensive interventions.” A NICU is a unit that provides high quality skilled care to critically ill neonates by offering facilities for continuous clinical, biochemical and radio logical monitoring and use of life support systems with the aim of improving survival of these babies. Intermediate care includes care of ill infants requiring less constant nursing care, but does not exclude respiratory support. Care of ill infants requiring less constant nursing care, but does not exclude respiratory support. When an intensive care nursery is available, the intermediate nursery serves as a “step down unit” from the intensive care area.

Prelabor rupture of membranes at term: A possible hematological triage in addition to vagino-rectal beta-hemolytic streptococcus screening for early labor induction

Introduction A potential complication of term prelabor rupture of membranes (term PROM) is chorioamnionitis with an increased burden on neonatal outcomes of chronic lung disease and cerebral palsy. The purpose of the study was to analyze the efficacy of a standing clinical protocol designed to identify women with term PROM at low risk for chorioamnionitis, who may benefit from expectant management, and those at a higher risk for chorioamnionitis, who may benefit from early induction. Material and methods This retrospective study enrolled all consecutive singleton pregnant women with term PROM. Subjects included women with at least one of the following factors: white blood cell count ≥ 15×100/μL, C-reactive protein ≥ 1.5 mg/dL, or positive vaginal swab for beta-hemolytic streptococcus. These women comprised the high risk (HR) group and underwent immediate induction of labor by the administration of intravaginal dinoprostone. Women with none of the above factors and those with a low risk for chorioamnionitis waited for up to 24 hours for spontaneous onset of labor and comprised the low-risk (LR) group. Results Of the 884 consecutive patients recruited, 65 fulfilled the criteria for HR chorioamnionitis and underwent immediate induction, while 819 were admitted for expectant management. Chorioamnionitis and Cesarean section rates were not significantly different between the HR and LR groups. However, the prevalence of maternal fever (7.7% vs. 2.9%; p = 0.04) and meconium-stained amniotic fluid was significantly higher in the HR group than in LR group (6.1% vs. 2.2%; p = 0.04). This study found an overall incidence of 4.2% for chorioamnionitis, 10.9% for Cesarean section, 0.5% for umbilical artery blood pH < 7.10, and 1.9% for admission to the neonatal intensive care unit. Furthermore, no confirmed cases of neonatal sepsis were encountered. Conclusions A clinical protocol designed to manage, by immediate induction, only those women with term PROM who presented with High Risk factors for infection/inflammation achieved similar maternal and perinatal outcomes between such women and women without any risks who received expectant management. This reduced the need for universal induction of term PROM patients, thereby reducing the incidence of maternal and fetal complications without increasing the rate of Cesarean sections.

Parent experience of HIE and hypothermia: A call for trauma informed care

Purpose/Background: Therapeutic hypothermia (TH) is the standard treatment for hypoxic ischemic encephalopathy (HIE). We surveyed parents of infants treated with TH about their experiences of communication and parental involvement in the Neonatal Intensive Care Unit (NICU). Methods/Approach: A 29-question anonymous survey was posted on a parent support website (https://www.hopeforhie.org) and sent to members via e-mail. Responses from open-ended questions were analyzed using thematic analysis. Results: 165 respondents completed the survey and 108 (66%) infants were treated with TH. 79 (48%) respondents were dissatisfied/neutral regarding the quality of communication in the NICU, whereas 127 (77%) were satisfied/greatly satisfied with the quality of parental involvement in the NICU. 6 themes were identified: 1) Setting for communication: Parents preferred face to face meetings with clinicians. 2) Content and clarity of language: Parents valued clear language (use of layman’s terms) and being explicitly told the medical diagnosis of HIE. 3) Immediate and Longitudinal Emotional Support: Parents required support from clinicians to process the trauma of the birth experience and hypothermia treatment. 4) Clinician time and scheduling: Parents valued the ability to join rounds and other major conversations about infant care. 5) Valuing the Parent Role: Parents desired being actively involved in rounds, care times and decision making. 6) Physical Presence and Touch: Parents valued being physically present and touching their baby; this presence was limited by COVID-related restrictions. Conclusion: We highlight stakeholder views on parent involvement and parent-clinician communication in the NICU and note significant overlap with principles of Trauma Informed Care: safety (physical and psychological), trustworthiness and transparency, peer support, collaboration and mutuality, and empowerment, voice and choice. We propose that a greater understanding and implementation of these principles may allow the medical team to more effectively communicate with and involve parents in the care of infants with HIE in the NICU.

Ventilator-Free Days in Neonatal Ventilator-Associated Pneumonia

Objective To compare the ventilator-free days (VFDs) at day 28 and the short-term outcomes in neonates with and without ventilator-associated pneumonia (VAP and non-VAP groups). Study Design We performed a cohort study in a Thai neonatal intensive care unit between 2014 and 2020 to identify the VFDs in VAP and non-VAP neonates. Univariate and multivariate analyses were performed. Results The incidences of VAP rates were 5.76% (67/1,163 neonates) and 10.86 per 1,000 (92/8,469) ventilator days. The medians (interquartile ranges) of gestational age and birth weight in the VAP vs non-VAP groups were 31 (27–35) vs 34 (30–38) weeks, and 1,495 (813–2,593) vs 2,220 (1,405–2,940) grams (p < 0.001 both), respectively. The medians (interquartile ranges) of VFDs at 28 days in the VAP and non-VAP groups were 5 (0–16) and 24 (20–26) days (<i>p</i> < 0.001). From the univariate analysis, the lower VFDs, longer ventilator days, and higher rates of moderate to severe bronchopulmonary dysplasia (BPD), postnatal steroids for BPD, length of stay, and daily hospital cost in the VAP group were significantly higher than in the non-VAP group. From the multivariate analysis, the VAP group had significantly lower VFDs (regression coefficient = -10.99, standard error = 1.11, <i>p</i> < 0.001) and higher BPD (adjusted risk ratio 18.70; 95% confidence interval 9.17–39.5, <i>p</i> < 0.001) than the non-VAP group. <b>Conclusion</b> Neonatal VAP lead to lower VFDs and a higher frequency of BPD. A multimodal strategy with a VAP prevention bundle care should be used in indicated cases to reduce the occurrence of neonatal VAP.

Epidemiological characteristics of neonates born to mothers infected with COVID-19: A single-centre observational study

BACKGROUND: COVID-19 disease can affect women at any stage of pregnancy, and newborns could become infected with SARS-CoV-2 through vertical or horizontal transmission. Little is known about SARS-CoV-2 infection in neonates born to mothers with COVID-19. Experts emphasize the importance of ensuring the safety of newborns without compromising the benefits of early contact with the mother. The aim of the study was to investigate the epidemiological characteristics of newborns born to mothers infected with SARS-CoV-2. METHODS: Observational, prospective cohort study was conducted in the intensive care unit of the perinatal center (Georgia). Information was collected by reviewing and personal observations of medical histories of newborns born to mothers infected with SARS-CoV-2. RESULTS: The study included 38 newborns with suspected (n = 16; 42.1%) and confirmed (n = 22; 57.9%) COVID-19 infection cases, treated in the neonatal intensive care unit. The study did not reveal the risk of vertical transmission of SARS-CoV-2 infection and confirmed a fairly large rate of horizontal transmission of infection (n = 25; 66%). Skin-to-skin mother care was performed in 68.7% of newborns, 26.3% received exclusive maternal or donated breast milk during hospital stay. CONCLUSION: Prevention of horizontal transmission of infection in newborns should be a priority. It is recommended skin-to-skin mother care and maternal or donated breast milk during hospital stay, taking into account the health of the mother and the newborn, following the rules of hygiene and use of the mask by the infected mother.

Survival and mortality of preterm neonates in a neonatal intensive care unit in Northern Ethiopia: a retrospective cohort study

The purpose of this study was to assess the predictors of preterm neonatal survival in a neonatal intensive care unit (NICU). A cohort study was conducted retrospectively on 1017 preterm neonates using medical records from January 2014 through December 2018. The Kaplan–Meier model was used to estimate mean survival time and cumulative survival probability. Furthermore, Multivariable Cox regression analysis was run to identify predictors of preterm neonatal mortality using an adjusted hazard ratio (AHR) at P < 0.05 and 95% confidence interval (CI). During the follow-up period in the NICU, the mean survival time of the preterm neonates was 47 (95% CI (43.19–48.95)) days. Compound presentation (AHR = 2.29, 95% CI (1.23–4.24)), perinatal asphyxia (AHR = 2.83, 95% CI (1.75–4.58)), respiratory distress syndrome (AHR = 3.01, 95% CI (1.80–5.01)), 1-min APGAR score (AHR = 0.78, 95% CI (0.62–0.98)), and birth weight (AHR = 0.32, 95% CI (0.17–0.58)) were found to be significant predictors of time to preterm neonatal mortality. In conclusion, the survival probability of preterm neonates showed a considerable decrement in the first week of life. Fetal presentation, gestational age, birth weight, 1-min APGAR score, perinatal asphyxia and respiratory distress syndrome found as independent predictors of preterm neonatal mortality.

Protecting brains and saving futures guidelines: A prospective, multicenter, and observational study on the use of telemedicine for neonatal neurocritical care in Brazil

Background Management of high-risk newborns should involve the use of standardized protocols and training, continuous and specialized brain monitoring with electroencephalography (EEG), amplitude integrated EEG, Near Infrared Spectroscopy, and neuroimaging. Brazil is a large country with disparities in health care assessment and some neonatal intensive care units (NICUs) are not well structured with trained personnel able to provide adequate neurocritical care. To reduce this existing gap, an advanced telemedicine model of neurocritical care called Protecting Brains and Saving Futures (PBSF) Guidelines was developed and implemented in a group of Brazilian NICUs. Methods A prospective, multicenter, and observational study will be conducted in all 20 Brazilian NICUs using the PBSF Guidelines as standard-of-care. All infants treated accordingly to the guidelines during Dec 2021 to Nov 2024 will be eligible. Ethical approval was obtained from participating centers. The primary objective is to describe adherence to the PBSF Guidelines and clinical outcomes, by center and over a 3-year period. Adherence will be measured by quantification of neuromonitoring, neuroimaging exams, sub-specialties consultation, and clinical case discussions and videoconference meetings. Clinical outcomes of interest are detection of seizures during hospitalization, use of anticonvulsants, inotropes, and fluid resuscitation, death before hospital discharge, length of hospital stay, and referral of patients to specialized follow-up. Discussion The study will provide evaluation of PBSF Guidelines adherence and its impact on clinical outcomes. Thus, data from this large prospective, multicenter, and observational study will help determine whether neonatal neurocritical care via telemedicine can be effective. Ultimately, it may offer the necessary framework for larger scale implementation and development of research projects using remote neuromonitoring. Trial registration NCT03786497, Registered 26 December 2018, https://www.clinicaltrials.gov/ct2/show/NCT03786497?term=protecting+brains+and+saving+futures&draw=2&rank=1.