Infant Mortality by Selected Maternal Characteristics and Race and Hispanic Origin in the United States, 2019-2021.

Objectives- This report presents infant mortality rates for selected maternal characteristics (prepregnancy body mass index, cigarette smoking during pregnancy, receipt of Special Supplemental Nutrition Program for Women, Infants, and Children (WIC) benefits during pregnancy, timing of prenatal care, and source of payment for delivery) for the five largest maternal race and Hispanic-origin groups in the United States for combined years 2019-2021. Methods-Descriptive tabulations based on data from the linked birth/infant death files for 2019-2021 are presented. The linked birth/infant death file is based on birth and death certificates registered in all 50 states and the District of Columbia. Infant mortality rates are presented for each maternal race and Hispanic-origin group overall and by selected characteristics. Results-Infant mortality rates varied across the five largest maternal race and Hispanic-origin groups and by selected maternal characteristics. For most race and Hispanic-origin groups, mortality rates were higher among infants of women with prepregnancy obesity compared with those of women who were normal weight, and were higher for infants of women who smoked cigarettes during pregnancy, received late or no prenatal care, or were covered by Medicaid as the source of payment for delivery. Overall, mortality rates were higher for infants of women who received WIC during pregnancy, but results varied across race and Hispanic-origin groups. Mortality rates for the maternal characteristics examined were generally highest among infants of Black non-Hispanic and American Indian and Alaska Native non-Hispanic women and lowest for Asian non-Hispanic women.

Explaining the experience of breastfeeding in women with gestational diabetes and designing and implementing an educational program based on planned behavior theory: a combined exploratory study protocol.

BACKGROUND: Gestational diabetes is a type of carbohydrate intolerance that is diagnosed for the first time during pregnancy. Researches have shown that gestational diabetes is associated with many negative prenatal and birth outcomes. Because of the complications such as infant death, several diabetics' mothers plan to stop breastfeeding. Research findings indicate a decrease in breastfeeding in mothers with gestational diabetes/ or contradictory tissues regarding the factors affecting the breastfeeding behavior of mothers with gestational diabetes and a special program to promote breastfeeding for these mothers based on the social and cultural conditions of Iranian society. The present study aims to design an interventional program with a mixed qualitative study based on the theory of planned behavior (PBT) to prevent the decrease of breastfeeding in diabetic mothers. METHODS/DESIGN: A mixed methods exploratory design will be used to conduct this study in 3 phases. The first phase (qualitative): The purpose of the first phase is to understand the experience of breastfeeding mothers who had gestational diabetes, which will be done using the directed content analysis method. The purposive sampling will be used on pregnant mothers with gestational diabetes 30-34 weeks and mothers with infants (up to 6 months) with a history of gestational diabetes. The second phase include designing and implementing an educational program based on the PBT: Education will be conducted based on the needs assessment of the qualitative phase, the opinions of the focus group, and the literature review, then the breastfeeding behavior will be measured using the survey tool of "breastfeeding drop". The third phase: Interventional quantitative phase: The sample size will be carried out by a pilot study, then a designed program as an educational intervention for teaching breastfeeding behavior based on the PBT for 30-34 weeks pregnant mothers with gestational diabetes will be implemented during 3-4 sessions and breastfeeding behavior will be evaluated after delivery. DISCUSSION: This is the first mixed-method study in Iran that led to implement an interventional program based on the theory of planned behavior. Because of the complications such as infant death, several diabetics' mothers plan to stop breastfeeding. We hope that the result of this research will be a step in solving breastfeeding problems in mothers with gestational diabetes.

Gestational diabetes is a type of carbohydrate intolerance diagnosed for the first time during pregnancy. The rate of gestational diabetes has increased along with the increase in the prevalence of diabetes risk factors. It is associated with multiple prenatal and birth outcomes. Despite the incomparable benefits of breastfeeding providing health to gestational diabetes mothers, the rate of breastfeeding is low. This is the first study in Iran using a mixed method approach in 3 phases to implement an educational interventional program with a qualitative study directed by theory of planned behavior (TPB) to prevent the decrease of breastfeeding in mothers with gestational diabetes. The purpose of first phase of study (qualitative) is to understand women's experience of gestational diabetes, which will be done using the directed content analysis method. The purposive sampling will be used for pregnant mothers with gestational diabetes at 30­34 weeks and mothers with infants (up to 6 months). The second phase is designing and implementing an educational program based on the PBT: Educational aims and content will be prepared based on the need achieved in the qualitative phase, the opinions of the focus group with experts? Mothers? Whom? And the literature review, then the breastfeeding behavior will be measured using the survey tool "breastfeeding drop". The third phase includes: Interventional quantitative phase an educational program will be implemented for training breastfeeding behavior based on the theory of PBT for 30­34 weeks pregnant mothers with gestational diabetes. This program will be administered as an educational intervention during 3­4 sessions and breastfeeding behavior will be evaluated after delivery.

Team experiences of the root cause analysis process after a sentinel event: a qualitative case study.

BACKGROUND: Root cause analysis (RCA) is a systematic approach, typically involving several stages, used in healthcare to identify the underlying causes of a medical error or sentinel event. This study focuses on how members of a Norwegian RCA team experience aspects of an RCA process and whether it complies with the Norwegian RCA method. METHOD: Based on a sentinel event in which a child died unexpectedly during childbirth in a Norwegian hospital in 2021, the following research questions are addressed: 1. What was the RCA team's experience of the RCA process? 2. Was there compliance with the Norwegian RCA method in this case? A case study was chosen out of the desire to understand complex social phenomena and to allow in-depth focus on a case. RESULTS: The result covered three main themes. The first theme related to the hospital's management system and aspects of the case that made it challenging to follow all recommendations in the Norwegian RCA guidelines. The second theme encompassed external and internal assessment. The RCA team was composed of members with methodological and medical expertise. However, the police's involvement in the case made it complex for the team to carry out the process. The third and final theme covered intrapersonal challenges RCA team members faced. Team members experienced various challenges during the RCA process, including being neutral, dealing with role-related challenges, grappling with ambivalence, and managing the additional time burden and resource constraints. As anticipated in the RCA guidelines, the team's ability to remain neutral was tested. CONCLUSION: The findings of this study can help stakeholders better comprehend how an inter-professional RCA teamwork intervention can affect a healthcare organization and enhance the teamwork experience of healthcare staff while facilitating improvements in work processes and patient safety. Additionally, these results can guide stakeholders in creating, executing, utilizing, and educating others about RCA processes.

[Genetic analysis of an infant death due to a paternally derived FOXF1 somatic-gonadal mosaic variant].

OBJECTIVE: To investigate the genetic characteristics and cause of death for an infant with alveolar capillary dysplasia and pulmonary vein misalignment (ACD/MPV). METHODS: An infant with ACD/MPV diagnosed at the Affiliated Maternity and Child Health Care Hospital of Nantong University in September 2022 was selected as the study subject. Clinical data of the infant were collected. Whole exome sequencing (WES) was carried out to detect genetic variants in the skin tissue, and Sanger sequencing was performed for verifying the candidate variants in the parents. Droplet digital PCR (ddPCR) was used to determine the mosaicism ratio of the variant in different germ layer-derived samples from the father. RESULTS: The infant had died within 2 days after birth due to hypoxemia and respiratory distress. WES revealed that she has harbored a c.433C>T nonsense variant in exon 1 of the FOXF1 gene, which was unreported previously. Sanger sequencing has verified the variant in the infant, with her mother's locus being the wild-type and a minor variant peak noted in her father. ddPCR indicated that the mosaic ratio of the c.433C>T variant in the father's sperm was 27.18%, with the mosaic ratios of the variant in tissues originating from the three germ layers ranging from 11% to 28%. CONCLUSION: The c.433C>T variant derived from the paternal germline and somatic mosaicism of the FOXF1 gene had probably predisposed to the neonatal death of this infant. ddPCR is an effective method for detecting mosaic variants.

Cervical Pessary for Prevention of Preterm Birth in Individuals With a Short Cervix: The TOPS Randomized Clinical Trial.

Importance: A short cervix as assessed by transvaginal ultrasound is an established risk factor for preterm birth. Study findings for a cervical pessary to prevent preterm delivery in singleton pregnancies with transvaginal ultrasound evidence of a short cervix have been conflicting. Objective: To determine if cervical pessary placement decreases the risk of preterm birth or fetal death prior to 37 weeks among individuals with a short cervix. Design, Setting, and Participants: We performed a multicenter, randomized, unmasked trial comparing a cervical pessary vs usual care from February 2017 through November 5, 2021, at 12 centers in the US. Study participants were nonlaboring individuals with a singleton pregnancy and a transvaginal ultrasound cervical length of 20 mm or less at gestations of 16 weeks 0 days through 23 weeks 6 days. Individuals with a prior spontaneous preterm birth were excluded. Interventions: Participants were randomized 1:1 to receive either a cervical pessary placed by a trained clinician (n = 280) or usual care (n = 264). Use of vaginal progesterone was at the discretion of treating clinicians. Main Outcome and Measures: The primary outcome was delivery or fetal death prior to 37 weeks. Results: A total of 544 participants (64%) of a planned sample size of 850 were enrolled in the study (mean age, 29.5 years [SD, 6 years]). Following the third interim analysis, study recruitment was stopped due to concern for fetal or neonatal/infant death as well as for futility. Baseline characteristics were balanced between participants randomized to pessary and those randomized to usual care; 98.9% received vaginal progesterone. In an as-randomized analysis, the primary outcome occurred in 127 participants (45.5%) randomized to pessary and 127 (45.6%) randomized to usual care (relative risk, 1.00; 95% CI, 0.83-1.20). Fetal or neonatal/infant death occurred in 13.3% of those randomized to receive a pessary and in 6.8% of those randomized to receive usual care (relative risk, 1.94; 95% CI, 1.13-3.32). Conclusions and Relevance: Cervical pessary in nonlaboring individuals with a singleton gestation and with a cervical length of 20 mm or less did not decrease the risk of preterm birth and was associated with a higher rate of fetal or neonatal/infant mortality. Trial Registration: ClinicalTrials.gov Identifier: NCT02901626.

Estrategias de mejora de calidad en lactancia en recién nacidos muy bajo peso: revisión del tema / Breastfeeding quality improvement strategies in very low birth weight newborns / Revisao das estratégias de melhoria da qualidade na amamentação de recém-nascidos de muito baixo peso

El abordaje nutricional en los recién nacidos de muy bajo peso al nacimiento constituye un desafío en la práctica clínica de los neonatólogos, y muchas veces se aborda fuera del período crítico. Existe evidencia contundente de que la optimización nutricional precoz impacta en forma directamente proporcional en la sobrevida y sobrevida sin morbilidades mayores para este grupo. La implementación de lactancia materna precoz en este contexto debe ser una prioridad del equipo asistencial, siendo la mejora de calidad una herramienta de demostrada utilidad para mejorar los resultados en términos de mortalidad y morbilidad neonatal.

The nutritional approach of the very low birth weight infant poses a great challenge to most neonatologists in their clinical practice, and it is frequently delayed until de newborn is in stable clinical conditions. Currently, scientific evidence supports that early nutritional optimization impacts directly on this group's survival and on their survival without major morbidities. Initiatives fostering early breastfeeding should be prioritized by the healthcare team. Quality improvement has shown to be a very useful resource to improve outcomes regarding neonatal mortality and morbidities.

A abordagem nutricional do recém-nascido de muito baixo peso representa um grande desafio para a maioria dos neonatologistas em sua prática clínica, sendo frequentemente postergada até que o recém-nascido esteja em condições clínicas estáveis. Atualmente, evidências científicas sustentam que a otimização nutricional precoce impacta diretamente na sobrevivência desse grupo e na sobrevivência sem maiores morbidades. Iniciativas de incentivo ao aleitamento materno precoce devem ser priorizadas pela equipe de saúde. A melhoria da qualidade tem se mostrado um recurso muito útil para melhorar os desfechos em relação à mortalidade e morbidades neonatais.

Protecting Infants from Sudden Unexpected Infant Death: Guidelines for Interventions during the Perinatal Period from the French National College of Midwives.

With 300-400 annual deaths in France, sudden unexpected infant death (SUID) is the leading cause of mortality in France among infants from the end of their first through their 12th month of life. These clinical practice guidelines aim to identify strategies for (future) parents to prevent avoidable SUIDs. They are based on a narrative literature review and an analysis of the existing reports and guidelines available on the topic in 2019-2020. In summary, it is recommended that parents ensure that their infants sleep on their back on a firm, empty surface in a sufficiently ventilated environment, share the parental bedroom, and be breastfed and vaccinated. All of these actions create protective factors against SUID. Conversely, parents should know that several factors increase the risk of SUID: unsafe sleep, maternal smoking, passive smoking after birth, exposure to alcohol or other psychoactive substances, and excessive heat. Precis: Sudden Unexpected Infant Death prevention includes infants being breastfed and vaccinated, sleeping on their back, on a firm, empty surface, near parents, and that they are not overheated.

Effect of comprehensive smoke-free legislation on neonatal mortality and infant mortality across 106 middle-income countries: a synthetic control study.

BACKGROUND: There are few quantitative studies into the effect of comprehensive smoke-free legislation on neonatal and infant mortality in middle-income countries. We aimed to estimate the effects of implementing comprehensive smoke-free legislation on neonatal mortality and infant mortality across all middle-income countries. METHODS: We applied the synthetic control method using 1990-2018 country-level panel data for 106 middle-income countries from the WHO, World Bank, and Penn World datasets. Outcome variables were neonatal (age 0-28 days) mortality and infant (age 0-12 months) mortality rates per 1000 livebirths per year. For each middle-income country with comprehensive smoke-free legislation, a synthetic control country was constructed from middle-income countries without comprehensive smoke-free legislation, but with similar prelegislation trends in the outcome and predictor variables. Overall legislation effect was the mean average of country-specific effects weighted by the number of livebirths. We compared the distribution of the legislation effects with that of the placebo effects to assess the likelihood that the observed effect was related to the implementation of smoke-free legislation and not merely influenced by other processes. FINDINGS: 31 (29%) of 106 middle-income countries introduced comprehensive smoke-free legislation and had outcome data for at least 3 years after the intervention. We were able to construct a synthetic control country for 18 countries for neonatal mortality and for 15 countries for infant mortality. Comprehensive smoke-free legislation was followed by a mean yearly decrease of 1·63% in neonatal mortality and a mean yearly decrease of 1·33% in infant mortality. An estimated 12 392 neonatal deaths in 18 countries and 8932 infant deaths in 15 countries were avoided over 3 years following the implementation of comprehensive smoke-free legislation. We estimated that an additional 104 063 infant deaths (including 95 850 neonatal deaths) could have been avoided over 3 years if the 72 control middle-income countries had introduced this legislation in 2015. 220 (43%) of 514 placebo effects for neonatal mortality and 112 (39%) of 289 for infant mortality were larger than the estimated aggregated legislation effect, indicating a degree of uncertainty around our estimates. Sensitivity analyses showed results that were consistent with the main analysis and suggested a dose-response association related to comprehensiveness of the legislation. INTERPRETATION: Implementing comprehensive smoke-free legislation in middle-income countries could substantially reduce preventable deaths in neonates and infants. FUNDING: Dutch Heart Foundation, Lung Foundation Netherlands, Dutch Cancer Society, Dutch Diabetes Research Foundation, Netherlands Thrombosis Foundation, Health Data Research UK.

Risk of adverse fetal outcomes following nonobstetric surgery during gestation: a nationwide population-based analysis.

BACKGROUND: Literature suggests that nonobstetric surgery during gestation is associated with a higher risk of spontaneous abortion, prematurity, and a higher cesarean section rate, but the direct impact on fetal outcomes is still unclear. In this study, we aimed to investigate whether nonobstetric surgery during pregnancy is associated with negative fetal outcomes by analysing a nation-wide database in Taiwan. METHODS: This population-based retrospective observational case-control study was based on the linkage of Taiwan's National Health Insurance Research Database, Birth Reporting Database, and Maternal and Child Health Database between 2004 and 2014. For every pregnancy with nonobstetric surgery during gestation, four controls were randomly matched according to maternal age and delivery year. We estimated adjusted odds ratios (aOR) and 95% confidence intervals (CIs) of adverse fetal outcomes with the non-surgery group as the reference. The primary outcomes involved stillbirth, prematurity, low birth weight, low Apgar scores, and neonatal and infant death. RESULTS: Among 23,721 identified pregnancies, 4,747 underwent nonobstetric surgery. Pregnancies with nonobstetric surgery had significantly higher risks of prematurity (aOR: 1.46; 95% CI: 1.31-1.62), lower birth weight (aOR: 1.49; 95% CI: 1.33-1.67), Apgar scores < 7 (1 min, aOR: 1.58; 95% CI: 1.33-1.86; 5 min, aOR: 1.34; 95% CI: 1.03-1.74), neonatal death (aOR: 2.01; 95% CI: 1.18-3.42), and infant death (aOR: 1.69; 95% CI: 1.12-2.54) than those without nonobstetric surgery after adjustment for socioeconomic deprivation, hospital level, and other comorbidities. Surgery performed in the third trimester was associated with a significantly increased rate of prematurity (aOR: 1.38; 95% CI: 1.03-1.85), but lower rates of stillbirth (aOR: 0.1; 95% CI: 0.01-0.75) and Apgar score < 7 at the 5th minute (aOR: 0.2; 95% CI: 0.05-0.82), than surgery performed in the first trimester. CONCLUSIONS: Pregnancies with nonobstetric surgery during gestation were associated with increased risks of prematurity, low birth weight, low Apgar scores, neonatal and infant death, longer admission, and higher medical expenses than those without surgery. Furthermore, surgery in the third trimester was associated with a higher rate of prematurity than surgery performed in the first trimester. TRIAL REGISTRATION: Not applicable.

Evaluation of neural tube defects from 2014 to 2019 in Turkey.

OBJECTIVE: The aim of this study is to determine the frequency of neural tube defects (NTDs) and to examine the epidemiological characteristics of NTD related deaths in Turkey. METHODS: This nationwide descriptive study was included NTD related infant deaths, termination of pregnancy for fetal anomaly (ToPFA) and stillbirth cases registered in Death Notification System between 2014 and 2019, and patients diagnosed with NTD in the 2018 birth cohort. FINDINGS: In the 2018 birth cohort, there were 3475 cases of NTD at birth (27.5 per 10,000). The fatality rates for live-born babies with NTD in this cohort were 13.5% at first year, and 15.6% at the end of March, 2022. NTDs were associated with 11.7% of ToPFA cases, 2.5% of stillbirths and 2.8% of infant deaths in 2014-2019. NTD related stillbirth rate was 1.74 per 10,000 births, while NTD related ToPFA rate and infant mortality rate were 0.61 and 2.70 per 10,000 live births respectively. NTD-related stillbirth and infant mortality rate were highest in the Eastern region (3.64 per 10,000 births; 4.65 per 10,000 live births respectively), while ToPFA rate was highest in the North and West regions (1.17 and 0.79 per 10,000 live births respectively) (p < 0.05). Prematurity and low birth weight were the variables with the highest NTD related rates for stillbirths (11.26 and 16.80 per 10,000 birth), ToPFA (9.25 and 12.74) per 10,000 live birth), and infant deaths (13.91 and 20.11 per 10,000 live birth) (p < 0.05). CONCLUSION: NTDs are common and have an important place among the mortality causes in Turkey. Primary prevention through mandatory folic acid fortification should be considered both to reduce the frequency of NTD and related mortality rates.

Reducing maternal and child mortality in rural Ghana.

The lack of health infrastructure in developing countries to provide women with modern obstetric care and universal access to maternal and child health services has largely contributed to the existing high maternal and infant deaths. Access to basic obstetric care for pregnant women and their unborn babies is a key to reducing maternal and infants´ deaths, especially at the community-level. This calls for the strengthening of primary health care systems in all developing countries, including Ghana. Financial access and utilization of maternal and child health care services need action at the community-level across rural Ghana to avoid preventable deaths. Financial access and usage of maternal and child health services in rural Ghana is poor. Lack of financial access is a strong barrier to the use of maternal and child health services, particularly in rural Ghana. The sustainability of the national health insurance scheme is vital in ensuring full access to care in remote communities.

Assessing the Burden of Infant Deaths Due to Herpes Simplex Virus, Human Immunodeficiency Virus, and Congenital Syphilis: United States, 1995 to 2017.

BACKGROUND: Despite advances in diagnosis and treatment, neonatal infection with herpes simplex virus (HSV) has a high case fatality rate. The national burden of neonatal HSV and associated deaths is unknown because this condition is not nationally notifiable. We investigated trends in HSV-related infant deaths compared with infant deaths from congenital syphilis (CS) and human immunodeficiency virus (HIV). METHODS: Linked birth-death files for infant deaths from 1995 to 2017 were obtained from the National Center for Health Statistics. These files include infants who were born alive and died in the first 365 days of life and exclude stillbirths. We searched death certificates for disease codes indicating HSV, CS, or HIV, and calculated the frequency and rate of deaths for each infection, overall, by infant sex, and birthing parent age and race/ethnicity. RESULTS: Nationally, 1591 deaths related to the infections of interest were identified: 1271 related to HSV (79.9%), 234 to HIV (14.7%), and 86 to CS (5.4%). Herpes simplex virus-related deaths increased significantly from 0.83/100,000 live births (95% confidence interval [CI], 0.57-1.17) in 1995 to 1.77 (95% CI, 1.37-2.24) in 2017. In contrast, HIV-related deaths declined: 1.64/100,000 (95% CI, 1.27-2.10) in 1995 to 0.00 in 2017. There was a median of 3 CS-related deaths/year, with elevated frequencies in 1995 to 1996 and 2017 (n = 8). Herpes simplex virus-related death rates were elevated among infants born to birthing parents younger than 20 years (4.17/100,000; 95% CI, 3.75-4.59) and to Black parents (2.86/100,000; 95% CI, 2.58-3.15). CONCLUSIONS: Nationally, HSV-related infant deaths exceeded those caused by HIV and CS and seem to be increasing. Our findings underscore the need for an effective HSV vaccine, test technologies enabling rapid identification of infants exposed to HSV at delivery, and a focus on equity in prevention efforts.

Mystery of a Family with Recurrent Male Infant Deaths- Solved by Autopsy and Molecular Tests.

The authors report a case of a six weeks old boy who presented with acute febrile illness and progressive abdominal distension. There was a significant family history of early male sibling deaths. Autopsy showed multiorgan abscesses. Molecular test revealed final diagnosis of the child.

A novel pathogenic variant of BRAT1 gene causes rigidity and multifocal seizure syndrome, lethal neonatal.

INTRODUCTION: Lethal neonatal rigidity and multifocal seizure syndrome (RMFSL) is a severe autosomal recessive epileptic encephalopathy characterized by microcephaly, rigidity, intractable focal seizures, apnea, and bradycardia at or soon after birth. RMFSL is related to BRCA1-associated ATM activator 1 (BRAT1) gene mutations. METHODS: An Iranian couple with history of infant death due to RMFSL was referred to our genetics lab for specialized genetic counseling and testing. Whole Exome Sequencing (WES) was applied. Following WES, Sanger sequencing was performed to confirm the candidate variant. RESULT: A novel nonsense variant (c.2041G > T, p. E681X) was identified in exon 14 of the BRAT1 gene. Based on the American College of Medical Genetics and Genomics guideline this variant was classified as a pathogenic variant. CONCLUSION: This research expands the spectrum of BRAT1 pathogenic variants in RMFSL syndrome and demonstrates the utility of WES in genetic diagnostic.

Óbitos pós-cirúrgicos em neonatos com malformações congênitas do aparelho digestivo ou osteomuscular / Post-surgical deaths in neonates with congenital malformations in the digestive or musculoskeletal systems / Muertes post-quirúrgicas en neonatos con malformaciones congénitas digestivas o musculoesqueléticas

Objetivo: Analisar a ocorrência de óbitos pós-cirúrgicos em recém-nascidos com malformação do aparelho digestivo ou osteomuscular em uma maternidade de referência. Método: Estudo exploratório, retrospectivo, de abordagem quantitativa, realizado em uma maternidade de referência localizada em Teresina ­ PI. Os dados foram coletados do Tabwin e de fichas de investigação de óbito infantil de neonatos nascidos em 2016 e 2017 e analisados no software Statistical Package for the Social Sciences. Resultados: O tipo de malformação mais prevalente do aparelho digestivo e osteomuscular entre os neonatos que foram a óbito após cirurgia foi o ânus imperfurado (41%) e a gastrosquise (64,2%), respectivamente. O choque séptico, seguido da insuficiência renal aguda foram os fatores determinantes dos óbitos analisados. Conclusão: O diagnóstico precoce é o fator primordial para redução da morbimortalidade de neonatos acometidos por malformações congênitas, uma vez que contribui para o direcionamento e planejamento dos cuidados imprescindíveis a esses pacientes

Objective: To analyze the occurrence of post-surgical deaths in newborns with malformation in the digestive or musculoskeletal systems in a reference maternity hospital. Method: This is an exploratory and retrospective study, with a quantitative approach, conducted in a reference maternity located in Teresina ­ PI. Data were collected from Tabwin and infant death investigation forms of neonates born in 2016 and 2017 and analyzed through the Statistical Package for the Social Sciences software. Results: The most prevalent type of malformation of the digestive and musculoskeletal systems among neonates who died after surgery was the imperforate anus (41%) and gastroschisis (64.2%), respectively. Septic shock, followed by acute kidney failure, constituted the determining factors of the analyzed deaths. Conclusion: Early diagnosis is the key factor for reducing morbidity and mortality in neonates affected by congenital malformations, as it contributes to the targeting and planning of care actions essential for these patients

Objetivo: Analizar la ocurrencia de muertes post-quirúrgicas en recién nacidos con malformación digestiva o musculoesquelética en una maternidad de referencia. Método: Estudio exploratorio, retrospectivo, con enfoque cuantitativo, realizado en una maternidad de referencia ubicada en Teresina - PI. Los datos se recopilaron de Tabwin y de registros de investigación de muerte infantil de neonatos en 2016 y 2017 y se analizaron utilizando el programa Statistical Package for the Social Sciences. Resultados: El tipo de malformación digestiva y musculoesquelética más frecuente entre los neonatos que murieron después de la cirugía fue el ano imperforado (41%) y la gastrosquisis (64,2%), respectivamente. El shock séptico, seguido de insuficiencia renal aguda, constituyeron los factores determinantes de las muertes analizadas. Conclusión: El diagnóstico temprano es el factor principal para reducir la morbimortalidad en los neonatos afectados por malformaciones congénitas, ya que contribuye a la dirección y planificación de la atención esencial para estos pacientes

Education of family members to support weaning to solids and nutrition in later infancy in term-born infants.

BACKGROUND: Education of family members about infant weaning practices could affect nutrition, growth, and development of children in different settings across the world. OBJECTIVES: To compare effects of family nutrition educational interventions for infant weaning with conventional management on growth and neurodevelopment in childhood. SEARCH METHODS: We used the standard strategy of Cochrane Neonatal to search the Cochrane Central Register of Controlled Trials (CENTRAL; 2018, Issue 5), MEDLINE via PubMed (1966 to 26 June 2018), Embase (1980 to 26 June 2018), and the Cumulative Index to Nursing and Allied Health Literature (CINAHL; 1982 to 26 June 2018). We searched clinical trials databases, conference proceedings, and references of retrieved articles. We ran an updated search from 1 January 2018 to 12 December 2019 in the following databases: CENTRAL via CRS Web, MEDLINE via Ovid, and CINAHL via EBSCOhost. SELECTION CRITERIA: We included randomised controlled trials that examined effects of nutrition education for weaning practices delivered to families of infants born at term compared to conventional management (standard care in the population) up to one year of age. DATA COLLECTION AND ANALYSIS: Two review authors independently identified eligible trial reports from the literature search and performed data extraction and quality assessments for each included trial. We synthesised effect estimates using risk ratios (RRs), risk differences (RDs), and mean differences (MDs), with 95% confidence intervals (CIs). We used the GRADE approach to assess the certainty of evidence. MAIN RESULTS: We included 21 trials, recruiting 14,241 infants. Five of the trials were conducted in high-income countries and the remaining 16 were conducted in middle- and low-income countries. Meta-analysis showed that nutrition education targeted at improving weaning-related feeding practices probably increases both weight-for-age z scores (WAZ) (MD 0.15 standard deviations, 95% CI 0.07 to 0.22; 6 studies; 2551 infants; I² = 32%; moderate-certainty evidence) and height-for-age z scores (0.12 standard deviations, 95% CI 0.05 to 0.19; 7 studies; 3620 infants; I² = 49%; moderate-certainty evidence) by 12 months of age. Meta-analysis of outcomes at 18 months of age was heterogeneous and inconsistent in the magnitude of effects of nutrition education on WAZ and weight-for-height z score across studies. One trial that assessed effects of nutrition education on growth at six years reported an uncertain effect on change in height and body mass index z score. Two studies investigated effects of nutrition education on neurodevelopment at 12 to 24 months of age with conflicting results. No trials assessed effects of nutrition education on long-term neurodevelopmental outcomes. AUTHORS' CONCLUSIONS: Nutrition education for families of infants may reduce the risk of undernutrition in term-born infants (evidence of low to moderate certainty due to limitations in study design and substantial heterogeneity of included studies). Modest effects on growth during infancy may not be of clinical significance. However, it is unclear whether these small improvements in growth parameters in the first two years of life affect long-term childhood growth and development. Further studies are needed to resolve this question.

The outcome of pregnancy in women with cystic fibrosis: a UK population-based descriptive study.

OBJECTIVE: To estimate the incidence of cystic fibrosis in pregnancy and to explore obstetric and neonatal outcomes. DESIGN: A population-based descriptive study using the methodology of the UK Obstetric Surveillance System (UKOSS). SETTING: All consultant-led maternity units in the UK. POPULATION: All pregnant women with a diagnosis of cystic fibrosis who booked for antenatal care in a UK obstetric unit between March 2015 and February 2017. METHODS: Prospective case collection identified using UKOSS monthly notification. MAIN OUTCOME MEASURES: Incidence, maternal morbidity, maternal mortality, gestation at delivery, neonatal mortality, neonatal morbidity. RESULTS: We report 71 pregnancies over a 2-year period. There was one early miscarriage, four terminations and three sets of twins, resulting in the live birth of 69 infants. There were no maternal deaths. One infant died following spontaneous preterm birth at 29 weeks' gestation. The mean gestation at delivery was 36.2 completed weeks. The mean birthweight centile for gestational age was the 61st centile. We report a positive correlation between both maternal lung function (FEV1 ) and mean gestation at delivery, and between FEV1 and mean birthweight centile for gestational age. CONCLUSIONS: Pregnancy outcomes are generally good in women with cystic fibrosis. Successful pregnancy is possible even in those women with FEV1 <60% predicted, although such women have higher chance of preterm delivery and a smaller baby. TWEETABLE ABSTRACT: Pregnant women with cystic fibrosis who have poorer lung function at the beginning of pregnancy have a higher risk of having a premature or smaller baby.

Miscarriage, stillbirth, and infant death in an oil-polluted region of the Niger Delta, Nigeria: A retrospective cohort study.

OBJECTIVE: To determine the association between oil pollution and miscarriage, stillbirth, and infant death in the Niger Delta region of Nigeria. METHODS: A retrospective cohort study was undertaken of pregnant women (aged 18-45 years) who attended selected health facilities in regions with high and low exposure to oil pollution from May 14, 2018, to September 27, 2018. A multistage sampling technique was used to randomly select a representative of women with high and low exposure to oil pollution. An interviewer-administered questionnaire was used for data collection. Bivariate and multivariable logistic regression analyses were employed to adjust for confounding factors of miscarriage, stillbirth, and infant death. RESULTS: In total, 1564 pregnant women were included in the study. Women with high exposure to oil pollution were more likely to experience stillbirth (odds ratio [OR] 1.806; 95% confidence interval [CI] 1.177-2.770) and infant death (OR 2.162; 95% CI 1.409-3.317). However, after adjusting for potential confounders, only infant death was associated with high exposure (adjusted OR 1.843; 95% CI 1.146-2.962). No association was found between miscarriage and high exposure to oil pollution. CONCLUSION: Women with high exposure to oil pollution are at higher risk of infant death.

Novel frameshift variant in MYL2 reveals molecular differences between dominant and recessive forms of hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is characterized by thickening of the ventricular muscle without dilation and is often associated with dominant pathogenic variants in cardiac sarcomeric protein genes. Here, we report a family with two infants diagnosed with infantile-onset HCM and mitral valve dysplasia that led to death before one year of age. Using exome sequencing, we discovered that one of the affected children had a homozygous frameshift variant in Myosin light chain 2 (MYL2:NM_000432.3:c.431_432delCT: p.Pro144Argfs*57;MYL2-fs), which alters the last 20 amino acids of the protein and is predicted to impact the most C-terminal of the three EF-hand domains in MYL2. The parents are unaffected heterozygous carriers of the variant and the variant is absent in control cohorts from gnomAD. The absence of the phenotype in carriers and the infantile presentation of severe HCM is in contrast to HCM associated with dominant MYL2 variants. Immunohistochemical analysis of the ventricular muscle of the deceased patient with the MYL2-fs variant showed a marked reduction of MYL2 expression compared to an unaffected control. In vitro overexpression studies further indicate that the MYL2-fs variant is actively degraded. In contrast, an HCM-associated missense variant (MYL2:p.Gly162Arg) and three other MYL2 stop-gain variants (p.E22*, p.K62*, p.E97*) that result in loss of the EF domains are stably expressed but show impaired localization. The degradation of the MYL2-fs can be rescued by inhibiting the cell's proteasome function supporting a post-translational effect of the variant. In vivo rescue experiments with a Drosophila MYL2-homolog (Mlc2) knockdown model indicate that neither the MYL2-fs nor the MYL2:p.Gly162Arg variant supports normal cardiac function. The tools that we have generated provide a rapid screening platform for functional assessment of variants of unknown significance in MYL2. Our study supports an autosomal recessive model of inheritance for MYL2 loss-of-function variants in infantile HCM and highlights the variant-specific molecular differences found in MYL2-associated cardiomyopathy.

Timing of postnatal steroids for bronchopulmonary dysplasia: association with pulmonary and neurodevelopmental outcomes.

OBJECTIVE: To determine the associations between age at first postnatal corticosteroids (PNS) exposure and risk for severe bronchopulmonary dysplasia (BPD) and neurodevelopmental impairment (NDI). STUDY DESIGN: Cohort study of 951 infants born <27 weeks gestational age at NICHD Neonatal Research Network sites who received PNS between 8 days of life (DOL) and 36 weeks' postmenstrual age was used to produce adjusted odds ratios (aOR). RESULTS: Compared with infants in the reference group (22-28 DOL-lowest rate), aOR for severe BPD was similar for children given PNS between DOL 8 and 49 but higher among infants treated at DOL 50-63 (aOR 1.77, 95% CI 1.03-3.06), and at DOL ≥64 (aOR 3.06, 95% CI 1.44-6.48). The aOR for NDI did not vary significantly by age of PNS exposure. CONCLUSION: For infants at high risk of BPD, initial PNS should be considered prior to 50 DOL for the lowest associated odds of severe BPD.