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Objectives: Pregnancies in women with Fontan circulation are on the rise, and they are known to imply high maternal and fetal complication rates. The altered hemodynamic profile of univentricular circulation affects placental development and function. This study describes placental sonomorphologic appearance and Doppler examinations and correlates these to histopathologic findings and pregnancy outcomes in women with Fontan circulation. Methods: A single-center retrospective analysis of pregnancies in women with Fontan circulation was conducted between 2018 and 2023. Maternal characteristics and obstetric and neonatal outcomes were recorded. Serial ultrasound examinations including placental sonomorphologic appearance and Doppler studies were assessed. Macroscopic and histopathologic findings of the placentas were reviewed. Results: Six live births from six women with Fontan physiology were available for analysis. Prematurity occurred in 83% (5/6 cases) and fetal growth restriction and bleeding events in 66% (4/6 cases) each. All but one placenta showed similar sonomorphologic abnormalities starting during the late second trimester, such as thickened globular shape, inhomogeneous echotexture, and hypoechoic lakes, resulting in a jelly-like appearance. Uteroplacental blood flow indices were within normal range in all women. The corresponding histopathologic findings were non-specific and consisted of intervillous and subchorionic fibrin deposition, villous atrophy, hypoplasia, or fibrosis. Conclusions: Obstetric and perinatal complication rates in pregnancies of women with Fontan circulation are high. Thus, predictors are urgently needed. Our results suggest that serial ultrasound examinations with increased awareness of the placental appearance and its development, linked to the Doppler sonographic results of the uteroplacental and fetomaternal circulation, may be suitable for the early identification of cases prone to complications. [ABSTRACT FROM AUTHOR]

Background: To report on prophylactic therapy for hyperfibrinolysis with tranexamic acid (TXA) during expectant management (EM) in the placenta accreta spectrum (PAS). Methods: This is a monocentric retrospective study of women with PAS presenting at our hospital between 2005 and 2021. All data were retrospectively collected through the departmental database. Results: 35 patients with PAS were included. EM was planned in 25 patients prior to delivery. Complete absorption of the retained placenta was seen in two patients (8%). Curettage was performed in 14 patients (56%). A hysterectomy (HE) was needed in seven (28%) patients; 18 patients (72%) underwent uterus-preserving treatment without severe complications. The mean duration of EM was 107 days. The mean day of onset of hyperfibrinolysis and beginning of TXA treatment was day 45. The mean nadir of fibrinogen level before TXA was 242.4 mg/dL, with a mean drop of 29.7% in fibrinogen level. Conclusions: Our data support EM as a safe treatment option in PAS. Hyperfibrinolysis can be a cause of hemorrhage during EM and can be treated with TXA. To our knowledge, this is the first cohort of patients with EM of PAS in whom coagulation monitoring and use of TXA have been shown to successfully treat hyperfibrinolysis. [ABSTRACT FROM AUTHOR]

To summarize the evidence on non-hemorrhagic adrenal infarction (NHAI) and identify questions arising in diagnosis and management, cases in the PubMed database were merged with our case series. A total of 92 publications were retrieved, 15 of which reported on NHAI during pregnancy. Including the four in our case series, 24 cases have been described so far. Severe, unremitting pain requiring opioid analgesia was the leading symptom, often combined with nausea and vomiting. Laboratory results were non-contributory in most cases. Diagnosis was established via MRI in nine cases (37.5%) and via CT in six (25%); nine patients (37.5%) underwent both investigations. Location was predominantly on the right side (n = 16, 66.7%). In addition to analgesia, anticoagulation with heparin was commenced in 18 cases (75%). When thrombophilia screening was performed, major thrombogenic polymorphisms were detected in six cases (33.3%). One woman developed signs of adrenal insufficiency. The reported perinatal outcome was unremarkable. Unilateral NHAI has emerged as a rare but important cause of severe abdominal pain in pregnancy. The threshold to perform an MRI in pregnant women with characteristic clinical findings should be low. To prevent fetal radiation exposure, diagnostic imaging via CT should be avoided. In addition to symptomatic treatment with opioid analgesia, initiation of anticoagulant treatment should be strongly considered. [ABSTRACT FROM AUTHOR]

Background: Portal hypertension in pregnancy is characterized by an increased perinatal and maternal complication rate. The purpose of this study was to evaluate the perinatal and maternal outcomes of these high-risk pregnancies at our tertiary center. Methods: We identified pregnancies with portal hypertension in our departmental database for the years 2013 to 2021. The medical history and perinatal and maternal data were extracted from medical records. Results: Eleven cases were identified. In pregnancy, delivery and postpartum, complications occurred in 72.7% of cases and included among others ascites, subclavian thrombosis, variceal-ligation-induced ulcer bleeding and postoperative hemorrhage. The cesarean delivery rate was 72.7% (n = 8); five of these were done for obstetric or fetal indications. The rate of preterm birth and admissions to neonatal intensive care unit were high (54.5% and 45.5%, respectively). Conclusions: Our case series substantiates the high maternal and perinatal complication rates seen in portal hypertension. The prevention of thromboembolic and bleeding complications was the main challenge. Care by an interdisciplinary team of experts is crucial for a successful perinatal and maternal outcome. [ABSTRACT FROM AUTHOR]

Lifestyle during pregnancy impacts the health of the mother and child. However, the extent to which physical activity affects maternal biomarkers and factors that might influence birth weight remains unclear. We analysed data from two lifestyle interventions in which the effects of an exercise programme (2x/week, 60–90 min) on the course of pregnancy with regard to adipokines and offspring were evaluated. A total of 70 women participated in this study (45, intervention group; 25, control group). Anthropometric data and maternal fasting serum leptin and resistin levels were measured at three time points (approximately 14th (T1), 24th (T2), and 36th (T3) weeks of gestation). Neonatal/child data were retrieved from screening examinations. Independent of the intervention, we found a positive correlation between the fat mass at T1 and both leptin and resistin levels at all time points. Leptin level was significantly higher in the control group at T3; however, no differences between the groups were found for resistin. The birth weight was influenced by the birth length, fat mass at T1/T3, and resistin level at T2. The BMI-SDS at one year of age was influenced by maternal fat-free mass at T3 and resistin at T1/T2. Even if these results can only be interpreted cautiously, lifestyle interventions during pregnancy are important in promoting maternal and child health. Further randomised controlled trials and translational studies are warranted to clarify the underlying mechanisms. [ABSTRACT FROM AUTHOR]

For healthy women entering labor after an uneventful pregnancy, advantages of birth in midwife-led models of care have been demonstrated. We aimed to study the level of awareness regarding care in alongside midwifery units (AMU), factors involved in the decision for birth in obstetrician-led units (OLU), and wishes for care and concerns about birth in women registering for birth in OLU who would have been eligible for care in AMU. Healthy women with a term singleton cephalic fetus after an uneventful pregnancy course booking for birth in OLU were prospectively recruited. Data were collected by questionnaire. In total, 324 questionnaires were analyzed. One quarter (23.1%) of participants never had heard of care in AMU. Two thirds (64.2%) of women had made their choice regarding model of care before entering late pregnancy; only 16.4% indicated that health professionals had the biggest impact on their decision. One-to-one care and the availability of a pediatrician were most commonly quoted wishes (30.8 and 34.0%, respectively), and the occurrence of an adverse maternal or perinatal event the greatest concern (69.5%). Although the majority of respondents had some knowledge about care in AMU, expressed wishes for birth matching core features of AMU and concerns matching those of OLU, a decision for birth in OLU was taken. This finding may be a result of lack of knowledge about details of care in AMU; additionally, wishes and concerns may be put aside in favor of other criteria. [ABSTRACT FROM AUTHOR]

The Objective of our study was to investigate the influence of dietary (dGDM) and insulin-dependent (iGDM) gestational diabetes (GDM) on BDNF blood levels of corresponding maternal-neonatal pairs and compare them to pregnancies unaffected by GDM. Blood samples from 293 maternal-neonatal pairs were analyzed. Statistical analysis was performed using multiple regression analysis for association of log-transformed maternal and neonatal BDNF levels in relation to GDM, gestational age, neonatal sex, and mode of delivery. This was followed by a 2:1 matching of healthy and diabetic pairs. Maternal and neonatal BDNF levels were lowest in the iGDM group, followed by the dGDM group and healthy controls (maternal: healthy 665 ± 562 (26–2343) pg/mL vs. dGDM 593 ± 446 (25–1522) pg/mL vs. iGDM 541 ± 446 (68–2184) pg/mL; neonate: healthy 541 ± 464 (9.5–2802) pg/mL vs. dGDM 375 ± 342 (1–1491) pg/mL vs. iGDM 330 ± 326 (47–1384) pg/mL). After multiple regression analysis and additional 2:1 matching neonatal log-BDNF was significantly lower (−152.05 pg/mL, p = 0.027) in neonates of mothers with GDM compared to healthy pairs; maternal log-BDNF was also lower (−79.6 pg/mL), but did not reach significance. Our study is the first to analyze BDNF in matched maternal-neonatal pairs of GDM patients compared to a metabolically unaffected control group. [ABSTRACT FROM AUTHOR]

While nutrition during pregnancy is critical for the health of both mother and child, little is known about the diet quality of women during pregnancy, its correlation with gestational weight gain (GWG)/body composition, and chosen maternal adipokines. Therefore, we evaluated the Healthy Eating Index (HEI) of 110 pregnant women and analyzed its correlation with GWG/body composition, physical activity, leptin, resistin, adiponectin, and interleukin 6 (IL-6), respectively. Diet quality was medium in 63% of women, characterized by a high intake of animal-based products. HEI was negatively influenced by pre-pregnancy obesity (β = −0.335, p = 0.004), and positively influenced by higher age (>35 yrs., β = 0.365, p ≤ 0.001), upper arm circumference (β = 0.222, p = 0.052), and total activity during the third trimester (β = 0.258, p = 0.008). GWG was associated with pre-pregnancy obesity (β = −0.512, p ≤ 0.001), thigh circumference (β = 0.342, p = 0.007), upper arm fat area (β = 0.208, p = 0.092), and maternal age group (>35 yrs. β = −0.166, p = 0.082), but not with HEI. Leptin and IL-6 displayed associations with variables representative of body composition, such as pre-pregnancy BMI, thigh circumference, upper arm fat area, and upper arm circumference, but were not influenced by HEI. Neither were adiponectin and resistin. IL-6 was also associated with total activity. In conclusion, GWG, leptin, and IL-6 were influenced by nutritional status (body composition/pre-pregnancy BMI), not by maternal diet. Physical activity level also had an impact on IL-6. Thus, efforts should be intensified to improve diet quality and participation in sports before and during pregnancy, particularly in overweight or obese women. [ABSTRACT FROM AUTHOR]

Objective: An increasing number of studies show the importance of brain-derived neurotrophic factor (BDNF) acting at the feto-placental interface, however, only a few studies describe BDNF levels in amniotic fluid (AF). Methods: In this cross-sectional, prospective study, 109 maternal blood-amniotic fluid pairs (including 66 maternal blood-fetal-blood-amniotic fluid trios) were analyzed. BDNF concentrations were measured with a commercially available immunoassay. Results: In 71 AF from 109 samples, AF-BDNF concentrations were below the lowest limit of Quantitation (LLoQ) of 1.19 pg/ml (group A), leaving 38 samples with measurable BDNF concentrations (group B). Patients in group A showed significantly higher maternal BMI before pregnancy (mean±SD 26.3± 6.7 (kg/m2) vs. 23.8 ±4.5 (kg/m2) p = 0.04) and lower maternal blood BDNF concentrations than the other group (mean±SD 510.6 ± 554.7 pg/ml vs. mean±SD 910.1± 690.1 pg/ml; p<0.0001). Spearman correlation showed a negative correlation between maternal BMI before pregnancy and maternal BDNF concentrations (r = -0.25, p = 0.01). Conclusion: Our study is the first to correlate AF-BDNF samples with the corresponding maternal and fetal blood-BDNF samples. The significant negative correlation between maternal BMI before pregnancy and maternal BDNF and AF-BDNF concentrations below the limit of detection has to be evaluated in further studies. [ABSTRACT FROM AUTHOR]

In Germany, cesarean section (CS) rates more than doubled within the past two decades. For analysis, auditing and inter-hospital comparison, the 10-Group Classification System (TGCS) is recommended. We used the TGCS to analyze CS rates in two German hospitals of different levels of care. From October 2017 to September 2018, data were prospectively collected. Unit A is a level three university hospital, unit B a level one district hospital. The German birth registry was used for comparison with national data. We performed two-sample Z tests and bootstrapping to compare aggregated (unit A + B) with national data and unit A with unit B. In both datasets (national data and aggregated data unit A + B), Robson group (RG) 5 was the largest contributor to the overall CS rate. Compared to national data, group sizes in RG 1 and 3 were significantly smaller in the units under investigation, RG 8 and 10 significantly larger. Total CS rates between the two units differed (40.7 vs. 28.4%, p<0.001). The CS rate in RG 5 and RG 10 was different (p<0.01 for both). The most relative frequent RG in both units consisted of group 5, followed by group 10 and 2a. The analysis allowed us to explain different CS rates with differences in the study population and with differences in the clinical practice. These results serve as a starting point for audits, inter-hospital comparisons and for interventions aiming to reduce CS rates. [ABSTRACT FROM AUTHOR]

Background: Carney complex (CNC) is a rare multiple endocrine neoplasia syndrome with autosomal dominant inheritance. Affected individuals present with mucocutaneous lentigines/blue nevi, cardiac and noncardiac myxomatous tumors, and multiple endocrine tumors. Mutations in PRKAR1A have been identified as genetic cause of the disease. Here, we report on pregnancy, delivery and puerperium in a woman with genetically confirmed CNC and her newborn. Case: The 31 year-old gravida 5 para 1 with CNC was referred at 26 weeks of gestation. Adrenocorticotropin-independent hypercortisolism, hyperglycemia, hypertension, low serum potassium, and osteoporotic fractures were present. Treatment with metyrapone, a reversible 11-beta-hydroxylase inhibitor, was initiated. The maternal condition improved, and a 5 weeks' pregnancy prolongation could be achieved. Elective repeat cesarean section was performed at 31 weeks of gestation for recurrent vaginal bleeding. The neonate developed transient hyponatremia necessitating hydrocortisone substitution for 2 weeks. Conclusion: In our case, treatment of CNC-associated hypercortisolism in pregnancy with metyrapone was effective. Maternal side effects did not occur. The newborn presented with transient hypocortisolism most likely due to transplacental drug effect. Our case illustrates that the treatment of rare diseases in pregnancy represents a challenge requiring interdisciplinary team work. [ABSTRACT FROM AUTHOR]

Background: Alongside midwifery-led care (AMC) was introduced in Germany in 2003. The aim of our study was to collect data about the utilization of AMC within North Rhine-Westfalia (NRW), Germany's most populous state, and to collect information regarding the approach used for implementation of AMC and providers' experiences in day-to-day work. Methods: Quantitative data were collected by questionnaire, telephone interview and comparative analysis of documents created for use in AMC. Qualitative data were compiled by questionnaire and workshop. Results: Seven obstetric departments offer AMC in NRW. On average, 3.4% of women gave birth in these AMC units, corresponding to 0.3% of all births in NRW. For the establishment of AMC, institutional, organizational, professional and formal arrangements were undertaken. An eligibility and transfer checklist was agreed upon between midwives and obstetricians. Both professions were faced with skepticism. Daily work in AMC resulted in improved teamwork between the two professions and increased job satisfaction for midwives. Acquisition of manual skills and time constraints were the major challenges. Conclusion: The low utilization of AMC is not due to the concept of care itself or difficulties with its implementation, nor to a lack of eligible women. Reasons may be insufficient awareness about AMC or a lack of interest in this type of care. [ABSTRACT FROM AUTHOR]

Background: Early infant weight development influences metabolic regulation later in life. For the prevention of obesity and metabolic diseases, it is important to understand the underlying mechanisms in detail. Objectives: This study aims to examine the effects of maternal anthropometric, sociodemographic, and lifestyle factors on maternal and cord blood leptin levels at birth and on the development of body mass index (BMI) standard deviation scores (SDS) in offspring up to 1 year of age. Methods: Seventy-six mother-child pairs were enrolled in this follow-up analysis in a cross-sectional design. Standardized questionnaires were used to collect information regarding maternal anthropometrics, lifestyle habits, and sociodemographic conditions, and newborn weight, or, rather, BMI-SDS, development during the first year of life. Results: Cord blood leptin (β = –0.222, p = 0.074), maternal leptin (β = 0.414, p = 0.001), and female sex of the offspring (β = 0.385, p = 0.003) explained 29.0% of the variance in BMI-SDS changes in the first year of life. Cord blood leptin was influenced by newborn sex (male; β = –0.220, p = 0.025) and maternal moderate-intensity physical activity in the third trimester (β = 0.265, p = 0.007, corr. R2 = 9.2%); maternal leptin was influenced by maternal prepregnancy BMI (β = 0.602, p < 0.001) and weight gain during pregnancy (β = 0.247, p = 0.004, corr. R2 = 35.5%). Conclusions: Higher maternal and lower cord blood leptin levels are associated with a higher BMI-SDS increase during the first year of life. Maternal leptin is influenced by maternal BMI and weight gain during pregnancy, and cord blood leptin is influenced by maternal physical activity; therefore, it can be suggested that an active and healthy maternal lifestyle may play a pivotal and beneficial role in the offspring's weight development. [ABSTRACT FROM AUTHOR]

Background: Syndromic brain malformations comprise a large group of anomalies with a birth prevalence of about 1 in 1,000 live births. Their etiological factors remain largely unknown. To identify causative mutations, we used whole‐exome sequencing (WES) in aborted fetuses and children with syndromic brain malformations in which chromosomal microarray analysis was previously unremarkable. Methods: WES analysis was applied in eight case‐parent trios, six aborted fetuses, and two children. Results: WES identified a novel de novo mutation (p.Gly268Arg) in ACTB (Baraitser‐Winter syndrome‐1), a homozygous stop mutation (p.R2442*) in ASPM (primary microcephaly type 5), and a novel hemizygous X‐chromosomal mutation (p.I250V) in SLC9A6 (X‐linked syndromic mentaly retardation, Christianson type). Furthermore, WES identified a de novo mutation (p.Arg1093Gln) in BAZ1A. This mutation was previously reported in only one allele in 121.362 alleles tested (dbSNP build 147). BAZ1A has been associated with neurodevelopmental impairment and dysregulation of several pathways including vitamin D metabolism. Here, serum vitamin‐D (25‐(OH)D) levels were insufficient and gene expression comparison between the child and her parents identified 27 differentially expressed genes. Of note, 10 out of these 27 genes are associated to cytoskeleton, integrin and synaptic related pathways, pinpointing to the relevance of BAZ1A in neural development. In situ hybridization in mouse embryos between E10.5 and E13.5 detected Baz1a expression in the central and peripheral nervous system. Conclusion: In syndromic brain malformations, WES is likely to identify causative mutations when chromosomal microarray analysis is unremarkable. Our findings suggest BAZ1A as a possible new candidate gene. [ABSTRACT FROM AUTHOR]

Background: An understanding of the factors that influence intestinal wall integrity during the transition from fetal to neonatal life is important to elucidate and minimize potential contributions to the development of intestinal pathologies in vulnerable newborns.Objective: To assess the factors affecting intestinal wall integrity of late preterm and term newborns at birth.Methods: The concentrations of serum intestinal fatty acid-binding protein (I-FABP) as biomarker of intestinal wall injury, superoxide dismutase (SOD), and glutathione peroxidase (Gpx) as parameters of oxidative stress, and blood gas analyses were measured in the arterial cord blood of 80 newborns, delivered by spontaneous vaginal delivery (labor group) or elective cesarean delivery (non-labor group).Results: Compared with the non-labor group, I-FABP, SOD, and Gpx levels were significantly higher in the labor group. I-FABP concentration was inversely correlated with pH and BE and positively correlated with SOD and Gpx concentrations, second stage of labor duration, and active pushing time.Conclusions: This study demonstrates that oxidative stress at birth and prolonged second stage of labor may lead to the intestinal injury, which is reflected in increased serum concentration of I-FABP. [ABSTRACT FROM AUTHOR]

Purpose: The diagnosis of acute promyelocytic leukemia (APL) in pregnancy is an uncommon, life-threatening emergency. Choice of treatment and management of complications are challenging.Methods: We report the case of a patient with diagnosis of APL at gestational age (GA) 24 + 4. We describe the interdisciplinary management during pregnancy and delivery and provide a 2-year follow-up. Existing reports on APL in pregnancy are summarized.Results: Single-agent induction therapy with all-trans retinoic acid (ATRA) was started and resulted in normalization of blood cell counts after 32 days. Vaginal delivery of a healthy baby occurred at GA 34 + 4. Consolidation therapy consisted of four courses of ATRA and arsenic trioxide (ATO). Less than 100 cases of APL in pregnancy are published. Misdiagnosis as HELLP syndrome with fatal outcome may occur. Combination therapies (ATRA-plus anthracyclines) were used in the majority of reports.Conclusions: Diagnosis and treatment of APL during pregnancy continues to be a challenge requiring interdisciplinary team work. Single-agent ATRA therapy may evolve as a safe and less-toxic treatment modality. [ABSTRACT FROM AUTHOR]