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Objective: Acute aortic dissection is a rare but frequently fatal aortic catastrophe with high morbidity and mortality. Especially in pregnant patients, acute dissection is often misdiagnosed putting two lives on the line. Due to its scarcity, only case reports have been reported. The aim of this study is to analyze the time of aortic dissection during the course of pregnancy and the outcome of emergency surgery in pregnant women with and without hereditary connective tissue disorder. Methods: We retrospectively reviewed all acute aortic dissections (type A and B) who underwent emergency aortic surgery at our institution between 1994 and 2022 and identified 13 patients with acute aortic dissection during pregnancy or directly postpartum. Mann-Whitney U and Fisher's exact tests were used for statistical analysis. Results: Of the 13 included patients, 5 had a genetic syndrome. These patients were significantly younger at the time of dissection and at an earlier stage of pregnancy (second trimester). Even though operative and in-house mortality was zero, we lost one patient on postoperative day 14 due to rupture of the aortic root after transfer to another hospital. Survival of neonates was 77% including two aborted pregnancies. Conclusions: Surgical treatment of acute aortic dissection during pregnancy can be performed with excellent operative mortality for the mothers and satisfying survival of their neonates. In patients with genetic syndrome, dissection occurs during the early second trimester, whereas non-syndromic patients experience acute dissection in the late third trimester. Long-term follow-up is essential for timely re-intervention, if needed.
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BACKGROUND: During pregnancy, women's bodies undergo rapid body weight and size changes within a relatively short period. Pregnancy may therefore, be associated with an increased vulnerability for developing body image dissatisfaction linked to adverse health outcomes for the mother (e.g., depression, eating disorders) and child (e.g., impaired self-regulation, childhood obesity). The present study aims to examine the prevalence and trajectories of body image dissatisfaction during pregnancy and postpartum and its relationship to pre-pregnancy BMI. This is the first study that investigates prevalence rates of body image dissatisfaction not only ante- but also postpartum, and that compares trajectories of women with normal weight and overweight. METHODS: A prospective longitudinal design with a quantitative approach was applied. Healthy pregnant women (N = 136) answered paper-pencil or online questionnaires at four time points (18th-22nd and 33rd-37th week of gestation, 3 and 6 months postpartum). Body image dissatisfaction was assessed using the German version of the Body Shape Questionnaire (BSQ) and the Eating Disorder Examination Questionnaire (EDE-Q). Both questionnaires are considered reliable and valid measures of several aspects of body image, and the BSQ allows for calculating prevalence rates by providing cut-off values. Using not just one but two body image questionnaires, trajectories of body image dissatisfaction can be compared. Pre-pregnancy BMI was assessed retrospectively via self-reported weight and height. RESULTS: The proportion of women reporting elevated levels of body image dissatisfaction was 6.6% (n = 9) in the second trimester, 2.9% (n = 4) in the third trimester, 11.0% (n = 15) three months postpartum, and 10.3% (n = 14) six months postpartum. Repeated measures ANOVA revealed that body image dissatisfaction significantly decreased from pre-pregnancy to pregnancy, remained stable during pregnancy, and returned to pre-pregnancy levels three to six months postpartum. Mixed between-within ANOVA showed that the overweight/obese group reported significantly higher levels of body image dissatisfaction at each measurement point except during the third trimester than women in the normal weight group. Significant but small interaction effects between time and pre-pregnancy BMI were found. CONCLUSIONS: The results revealed that approximately every tenth woman is affected by body image dissatisfaction after childbirth. Women with a higher BMI level before pregnancy are particularly at risk of experiencing body image dissatisfaction. Healthcare providers should screen for body image dissatisfaction, in particular after childbirth, and inform affected women about possible adverse health outcomes and treatment options. Study limitations concern the drop-out rate of 51.4% and the retrospective and self-reported assessment of pre-pregnancy BMI. Future studies should include additional assessment points in the first trimester and more than six months postpartum and try to include a matched control group of non-pregnant women to compare prevalence rates and trajectory of body image dissatisfaction.
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Insatisfacción Corporal , Imagen Corporal , Índice de Masa Corporal , Periodo Posparto , Humanos , Femenino , Embarazo , Periodo Posparto/psicología , Adulto , Insatisfacción Corporal/psicología , Estudios Prospectivos , Imagen Corporal/psicología , Estudios Longitudinales , Encuestas y Cuestionarios , Adulto Joven , Sobrepeso/psicología , Sobrepeso/epidemiologíaRESUMEN
The sFlt-1/PlGF ratio is an established tool in clinical practice, where it is part of a diagnostic algorithm and informs the prognosis of preeclampsia (PE). Maternal and gestational comorbidities can affect the performance of the sFlt-1/PlGF ratio and its constituent elements, and a good understanding of the potential pitfalls is required. The objective of this paper was to provide a current narrative review of the literature on the diagnostic and predictive performance of the sFlt-1/PlGF ratio in specific patient cohorts. Potential factors which can negatively affect the clinical interpretability and applicability of the sFlt-1/PlGF ratio include chronic kidney disease, twin pregnancy, and maternal obesity. Pathophysiological mechanisms related to these factors and disorders can result in different concentrations of sFlt-1 and/or PlGF in maternal blood, meaning that the use of standard cut-off values in specific cohorts can lead to errors. To what extent the cut-off values should be adapted in certain patient cohorts can only be clarified in large prospective cohort studies. This applies to the use of the ratio both for diagnosis and prognosis.
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Background: Placenta accreta spectrum (PAS) can be the cause of major morbidity and its optimal management is still controversial. The aim of this study was to compare the traditional one-step surgery with a two-step surgical approach in which the placenta is left in situ and the second final operation is delayed to minimise blood loss. Methods: We conducted a single-centre retrospective cohort study including all patients managed for PAS between 2007 and 2023. The number of units of red blood cells (RBCs) needed during surgery was the primary outcome used to compare these two approaches. Results: A total of 43 cases were included in this analysis. Twenty of these were managed with the delayed two-step surgical approach, whereas 23 received one-step surgery. The median estimated blood loss during surgery was 2000 mL and 2800 mL for two-step and one-step surgery, respectively (p = 0.095). In the two-step surgical approach, the median number of RBC units transfused during surgery was significantly lower (p = 0.049) and the odds ratio for needing more than four units of RBCs was 0.28 (95%-CI: 0.08-0.98, p = 0.043). A longer interval between the caesarean section and the second operation showed a trend toward lower blood loss (p = 0.065) and was associated with a significantly lower number of RBC units needed during surgery (p = 0.019). Conclusions: Two-step surgery for the treatment of PAS was safe in our cohort and could lead to a reduction in blood transfusion. Leaving the placenta in situ and delaying the final operation represents a possible alternative to traditional caesarean hysterectomy.
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Low molecular weight heparins (LMWH) are used to prevent or treat thromboembolic events during pregnancy. While studies suggest an overall protective effect of LMWH in preeclampsia (PE), their use in preeclampsia remains controversial. LMWH may convey beneficial effects in preeclampsia independent of their anti-coagulant activity, possibly by inhibiting inflammation. Here we evaluated whether LMWH inhibit placental thrombo-inflammation and trophoblast NLRP3 inflammasome activation. Using an established procoagulant extracellular vesicle (EV)-induced and platelet-dependent preeclampsia-like mouse model, we show that LMWH reduces pregnancy loss and trophoblast inflammasome activation, restores altered trophoblast differentiation and improves trophoblast proliferation in-vivo and in-vitro. Moreover, LMWH inhibits platelet independent trophoblast NLRP3 inflammasome activation. Mechanistically, LWMH activates via Heparin binding epidermal growth factor (HBEGF) signaling the PI3-Kinase-AKT pathway in trophoblasts thus preventing inflammasome activation. In human preeclampsia placental explants, inflammasome activation and PI3-Kinase-AKT signaling events were reduced with LMWH treatment compared to those without LMWH treatment. Thus, LMWH inhibits sterile inflammation via the HBEGF signaling pathway in trophoblasts and ameliorates preeclampsia-associated complications. These findings suggest that drugs targeting the inflammasome may be evaluated in preeclampsia and identify a signaling mechanism through which LMWH ameliorates preeclampsia, thus providing a rationale for the use of LMWH in preeclampsia.
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In the following casuistry, a denied advanced pregnancy was discovered during the diagnosis of an oncological disease. Faced with a life-threatening condition, the patient urged late termination of the pregnancy and was introduced to psychological counselling in order to find a viable and ethically justifiable solution. Strategies for crisis intervention and supportive approaches in the patient's care as well as interdisciplinary collaboration are presented and discussed.
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Grupo de Atención al Paciente , Humanos , Femenino , Embarazo , Adulto , Complicaciones Neoplásicas del Embarazo/psicología , Complicaciones Neoplásicas del Embarazo/terapia , Negación en Psicología , Comunicación Interdisciplinaria , Consejo , Aborto Inducido/psicología , Colaboración Intersectorial , Intervención en la Crisis (Psiquiatría)RESUMEN
PURPOSE: The physical health and development of an individual are influenced by multiple parameters and shaped by internal and external factors during pregnancy. However, it is unclear whether there is an association between maternal lipid concentrations in the third trimester of pregnancy and infant serum lipids as well as anthropometric growth, and whether these factors are influenced by the socioeconomic status (SES) of the mothers. METHODS: Between 2011 and 2021, 982 mother-child pairs were recruited in the LIFE-Child study. To investigate the influence of prenatal factors, pregnant women at the 24th and 36th week of gestation as well as children at the age of 3, 6 and 12 months were examined and serum lipids determined. Socioeconomic status (SES) was assessed using the validated Winkler Index. RESULTS: A higher maternal BMI was associated with a significantly lower Winkler score and a higher infant weight, height, head circumference and BMI from birth up to the 4th-5th week of life. In addition, the Winkler Index correlates with maternal HDL cholesterol and ApoA1 levels. There was no relation between the delivery mode and the maternal BMI or SES. For the maternal HDL cholesterol concentration in the third trimester, an inverse relation to children's height, weight, head circumference and BMI up to the first year of life as well as the chest and abdominal circumference to an age of 3 months was found. Children born to mothers with dyslipidemia in pregnancy tended to have a worse lipid profile than those born to normolipidemic mothers. CONCLUSION: Serum lipid concentrations and anthropometric parameters of children in the first year of life are affected by multiple factors like maternal BMI, lipid levels and SES.
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Madres , Clase Social , Lactante , Humanos , Femenino , Embarazo , HDL-Colesterol , Antropometría , Peso al Nacer , Índice de Masa CorporalRESUMEN
PURPOSE: The fetal membranes are essential for the maintenance of pregnancy, and their integrity until parturition is critical for both fetal and maternal health. Preterm premature rupture of the membranes (pPROM) is known to be an indicator of preterm birth, but the underlying architectural and mechanical changes that lead to fetal membrane failure are not yet fully understood. The aim of this study was to gain new insights into the anatomy of the fetal membrane and to establish a tissue processing and staining protocol suitable for future prospective cohort studies. METHODS: In this proof of principle study, we collected fetal membranes from women undergoing vaginal delivery or cesarean section. Small membrane sections were then fixed, stained for nucleic acids, actin, and collagen using fluorescent probes, and subsequently imaged in three dimensions using a spinning disk confocal microscope. RESULTS: Four fetal membranes of different types were successfully processed and imaged after establishing a suitable protocol. Cellular and nuclear outlines are clearly visible in all cases, especially in the uppermost membrane layer. Focal membrane (micro) fractures could be identified in several samples. CONCLUSION: The presented method proves to be well suited to determine whether and how the occurrence of membrane (micro) fractures and cellular jamming correlate with the timing of membrane rupture and the mode of delivery. In future measurements, this method could be combined with mechanical probing techniques to compare optical and mechanical sample information.
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Rotura Prematura de Membranas Fetales , Nacimiento Prematuro , Femenino , Recién Nacido , Embarazo , Humanos , Cesárea , Estudios Prospectivos , Corion , Membranas Extraembrionarias , Microscopía ConfocalRESUMEN
BACKGROUND: We investigated possible parameters that could predict the need for obstetric maneuvers, the duration of the active second stage of labor (i.e., the duration of active pushing), and short-term neonatal outcome in vaginal breech births. MATERIALS AND METHODS: We performed a retrospective analysis of 268 successful singleton vaginal breech births in women without previous vaginal births from January 2015 to August 2022. Multivariable regression was used to investigate associations between maternal and fetal characteristics (including antepartum magnetic resonance (MR) pelvimetry) with obstetric maneuvers, the duration of active second stage of labor, pH values, and admission to the neonatal unit. Models for the prediction of obstetric maneuvers were built and internally validated. RESULTS: Obstetric maneuvers were performed in a total of 130 women (48.5%). A total of 32 neonates (11.9%) had to be admitted to the neonatal unit. The intertuberous distance (ITD) (p < 0.001), epidural analgesia (p < 0.001), and birthweight (p = 0.026) were associated with the duration of active second stage of labor. ITD (p = 0.028) and birthweight (p = 0.011) were also independently associated with admission to the neonatal unit, while pH values below 7.10 dropped significantly (p = 0.0034) if ITD was ≥13 cm. Furthermore, ITD (p < 0.001) and biparietal diameter (p = 0.002) were independent predictors for obstetric maneuvers. CONCLUSIONS: ITD is independently associated with the duration of active second stage of labor. Thus, it can predict suboptimal birth mechanics in the last stage of birth, which may lead to the need for obstetric maneuvers, lower arterial pH values, and admission to the neonatal unit. Consequently, MR pelvimetry gives additional information for practitioners and birthing people preferring a vaginal breech birth.
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Presentación de Nalgas , Parto Obstétrico , Segundo Periodo del Trabajo de Parto , Humanos , Femenino , Embarazo , Segundo Periodo del Trabajo de Parto/fisiología , Estudios Retrospectivos , Adulto , Recién Nacido , Parto Obstétrico/métodos , Parto Obstétrico/estadística & datos numéricos , Resultado del Embarazo/epidemiología , Factores de Tiempo , Peso al Nacer , Concentración de Iones de HidrógenoRESUMEN
We performed longitudinal examinations of the arterial retinal microvasculature using Adaptive Optics Retinal Imaging in a 30-year-old healthy woman with twin pregnancy from the 23rd week of gestation (wog) to three days postpartum. Two blinded graders recorded the average wall-to-lumen ratio (WLR) of the examined retinal artery. There was a significant increase in the mean WLR over the course of pregnancy followed by a decreasing WLR from the 37th wog. The demonstrated changes in WLR may be an expression of vascular remodeling and adaptation to volume load which indicates that pregnancy can be viewed as a cardiovascular stress test.
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Hipertensión , Preeclampsia , Arteria Retiniana , Femenino , Humanos , Embarazo , Adulto , Presión Sanguínea , CorazónRESUMEN
BACKGROUND: With improved operative techniques pregnancy rates have been rising in patients with anomalies of the extrophy-epispadias-complex, including also female patients with bladder extrophy. Specific risks around pregnancy need to be addressed sufficiently beforehand. CASE PRESENTATION: An unplanned pregnancy was detected at 34 weeks in a 39-year old White female patient with former complex bladder extrophy. Decades after her operation she had not received any follow-up medical care and believed to be unable to conceive due to her anomaly. Thus no contraceptive matters were taken. The patient had lived in a stable relationship with regular sexual intercourse for many years. Until 34 weeks the pregnancy was uncomplicated, but then uterine prolapse and signs of beginning pre-eclampsia appeared, and a healthy girl was born with cesarean section. CONCLUSION: As patients with bladder extrophy and other anomalies from the extrophy-epispadias-complex reach adolescence/adulthood, they need continuous medical follow-up and transition of care to adult surgery and gynecology in order to address specific aspects of sexual health, reproduction, contraception, and also cancer screening. In the presented case lack of transition of care resulted in an unplanned and complicated pregnancy.
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Extrofia de la Vejiga , Epispadias , Complicaciones del Embarazo , Adulto , Adolescente , Humanos , Embarazo , Femenino , Lactante , Extrofia de la Vejiga/cirugía , Extrofia de la Vejiga/complicaciones , Embarazo no Planeado , Cesárea/efectos adversos , Epispadias/complicaciones , Epispadias/cirugía , Complicaciones del Embarazo/etiologíaRESUMEN
In this prospective, monocentric study, we investigated the potency of a novel three-dimensional (3D) body scanner for external pelvic assessment in birth planning for intended vaginal breech delivery. Between April 2021 and June 2022, 73 singleton pregnancies with intended vaginal birth from breech presentation (>36.0 weeks of gestation) were measured using a pelvimeter by Martin, a three-dimensional body scanner, and MR-pelvimetry. Measures were related to vaginal birth and intrapartum cesarean section. A total of 26 outer pelvic dimensions and 7 inner pelvic measurements were determined. The rate of successful vaginal breech delivery was 56.9%. The AUC (area under the curve) of the obstetric conjugate (OC) measured by MRI for predicting the primary outcome was 0.62 (OR 0.63; p = 0.22), adjusted for neonatal birth weight 0.66 (OR 0.60; p = 0.19). Of the 22 measured 3D body scanner values, the ratio of waist girth to maternal height showed the best prediction (AUC = 0.71; OR 1.27; p = 0.015). The best predictive pelvimeter value was the distantia spinarum with an AUC of 0.65 (OR = 0.80). The 3D body scanner technique is at least equal to predict successful vaginal breech delivery compared to MRI diagnostics. Further large-scale, prospective studies are needed to verify these results.
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PURPOSE: During lactation, bone turnover increases, reflecting the mobilization of Calcium from maternal skeletal stores and resulting in bone loss. However, mechanisms are not yet fully understood, and previous studies have been comparatively small. We aim to assess bone metabolism during lactation by comparing bone-metabolism-related-parameters between large cohorts of lactating and nonlactating women. METHODS: In a retrospective cohort study, we recruited 779 postpartum women and 742 healthy, nonpregnant, nonlactating controls. Postpartum women were examined 3 and 6 months after delivery and retrospectively assigned to either the exclusively breastfeeding (exc-bf) group if they had exclusively breastfed or the nonexclusively breastfeeding (nonexc-bf) group if they had not exclusively breastfed up to the respective visit. Serum levels of PTH, Estradiol, total Calcium, Phosphate, and bone turnover markers (ßCTX, P1NP, Osteocalcin) were compared between the groups. RESULTS: Bone turnover markers were significantly increased in exc-bf and nonexc-bf women compared with the controls (all ps < .001). ßCTX was approximately twice as high in exc-bf women than in the controls. PTH levels were marginally higher in exc-bf (p < .001) and nonexc-bf women (p = .003) compared with the controls (6 months). Estradiol was suppressed in exc-bf women compared with the controls (p < .001, 3 months). CONCLUSION: Exc-bf and even nonexc-bf states are characterized by an increase in bone formation and resorption markers. The PTH data distribution of exc-bf, nonexc-bf, and control groups in the underpart of the reference range suggest that lactational bone loss is relatively independent of PTH.
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Calcio , Lactancia , Femenino , Humanos , Estudios Retrospectivos , Hormona Paratiroidea , Remodelación Ósea , Estradiol , Densidad ÓseaRESUMEN
Transforming growth factor beta-1 (TGFß1) is an adipokine secreted from adipose tissue, placental tissue and immune cells with a role in cell proliferation, cell apoptosis and angiogenic proliferation. The role of TGFß1 in pregnancy and child growth and the source of cord TGFß1 are yet unknown. In this study, we sought to clarify the correlation of TGFß1 levels with parameters of intrauterine growth and child growth during the first year of life, and to determine whether their source is primarily of fetal or maternal origin. Serum samples and anthropometric measurements were obtained from the LIFE Child cohort of 79 healthy mother-child pairs. Measurements were conducted using enzyme-linked immunosorbent assays. Statistical analyses including Mann-Whitney U-test, correlation analyses and linear regression analyses were performed using GraphPad Prism and R. TGFß1 levels were significantly higher in cord than in maternal serum, suggesting a fetal origin. Multivariate regression analyses revealed strong positive associations between cord TGFß1 levels at birth and child weight at U6. Furthermore, cord TGFß1 was significantly correlated with child weight at approximately one year of age. An increase of 10,000 pg/mL in cord TGFß1 concentrations at birth was associated with a higher body weight of 201 g at roughly one year of age when adjusted for sex.
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With a prevalence of 0,01-0,03%, acute fatty liver in pregnancy (AFLP) is a rare and dangerous complication of pregnancy and is difficult to distinguish from other, sometimes more common, pregnancy diseases such as HELLP syndrome, aHUS and TTP because of its mostly non-specific symptoms. Due to its rarity, AFLP is often not obvious to the obstetrician as a possible differential diagnosis. Yet early diagnosis and the fastest possible delivery is the only causal therapy and is important for the mortality rate. In the present manuscript, the pathophysiology, diagnosis and therapy of acute fatty liver in pregnancy are highlighted for the clinical routine based on case descriptions from three university hospitals, and reference is made to possible findings that are helpful in establishing the diagnosis. The angiogenic preeclampsia marker sFlt-1 plays a role and provides new opportunities to consider pathophysiological approaches.
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Hígado Graso , Síndrome HELLP , Preeclampsia , Complicaciones del Embarazo , Embarazo , Femenino , Humanos , Hígado Graso/diagnóstico , Hígado Graso/terapia , Hígado Graso/epidemiología , Complicaciones del Embarazo/diagnóstico , Complicaciones del Embarazo/terapia , Complicaciones del Embarazo/epidemiología , Preeclampsia/diagnóstico , Síndrome HELLP/diagnóstico , Síndrome HELLP/terapiaRESUMEN
To promote the expansion of interprofessional training objectives in the curriculum of health professions curriculum at the Medical Faculty, University of Leipzig, the interprofessional teaching project between the Department of Obstetrics, the Skills and Simulation Centre and the School of Midwifery was selected to promote innovative teaching projects, supported by the University of Leipzig [https://www.stil.uni-leipzig.de/] grant "StiL - Studying in Leipzig". Using scenarios with simulated patients, students were to recall and apply theoretically learned procedures and immediate measures in an obstetric emergency under supervision and to communicate these clearly in the team. Final-year medical students from the Medical Faculty (n=15) and midwifery students (n=17) from the vocational school went through teaching situations together, in which two simulation scenarios (shoulder dystocia and postpartum haemorrhage) were implemented. The aim of the project was to integrate interprofessional collaboration into training and to learn together under simulated conditions in the Skills and Simulation Center protected environment. The following questions was intended to be clarified in the project in addition to the establishment of a sub-professional teaching unit What do students benefit most from in interprofessional teaching units? Are there differences between midwifery and medical students? Is the learning success the same for team-communicative and professional learning goals? To clarify the questions, an evaluation was carried out using an exploratory questionnaire with a Likert scale. All students particularly liked the exchange and contact with other professional groups, the communicative aspect and situational action in unforeseen emergency situations. The participants stated that they had benefited from both interprofessional teaching units, in terms of team communication as well as in professional terms. However, medical students experienced significantly higher cognitive overload regarding prior acquired knowledge compared to vocational midwifery students. Overall, the team communication learning objectives were more difficult to fulfill.
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Educación Médica , Partería , Embarazo , Femenino , Humanos , Partería/educación , Urgencias Médicas , Curriculum , EstudiantesRESUMEN
INTRODUCTION: Most births in Germany take place in a clinical setting. Midwife-led units have been offered in Germany since 2003 as an addition to the primarily physician-led obstetric care. The purpose of this study was to analyze differences regarding medical parameters between a midwife-led and a primarily physician-led unit in a level 1 perinatal center. MATERIAL AND METHODS: Between 12/2020 and 12/2021, all births begun in the midwife-led unit were retrospectively analyzed and compared to a physician-led control cohort. Outcome measures were defined as obstetric interventions, delivery mode and duration, delivery position, and maternal and neonatal outcome. RESULTS: The percentage of deliveries started in the midwife-led unit out of the total birth rate was 4.8% (n=132). Most transfers were made for more effective analgesia (52.6%). Among medically indicated transfers (n=30, 39.5%), transfers due to CTG abnormalities and failure to progress in labor after rupture of membranes predominated. 43.9% (n=58) of patients gave birth successfully in the midwife-led unit. The rate of episiotomy was significantly higher in the primarily physician-led unit compared to the successful midwife-led unit (p=0.019). CONCLUSION: Birth in a midwife-led unit within a perinatal center can be considered an equivalent alternative to primarily physician-led birth for low-risk pregnant women.
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Adipokines are signaling proteins involved in metabolic, endocrinological, vascular and immunogenic processes. Associations of various adipokines with not only insulin resistance but also with increased insulin sensitivity, increased systolic blood pressure, and atherosclerosis highlight the significance of adipokines in several components of metabolic syndrome and metabolic diseases in general. As pregnancy presents a unique metabolic state, the role of adipokines in pregnancy, and even in various pregnancy complications, appears to be key to elucidating these metabolic processes. Many studies in recent years have attempted to clarify the role of adipokines in pregnancy and gestational pathologies. In this review, we aim to investigate the changes in maternal adipokine levels in physiological gestation, as well as the association of adipokines with pregnancy pathologies, such as gestational diabetes mellitus (GDM) and preeclampsia (PE). Furthermore, we will analyze the association of adipokines in both maternal serum and cord blood with parameters of intrauterine growth and various pregnancy outcomes.
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X-linked hypohidrotic ectodermal dysplasia (XLHED) is a rare genetic disorder characte-rised by abnormal development of the skin and its appendages, such as hair and sweat glands, the teeth, and mucous glands of the airways, resulting in serious, sometimes life-threatening complications like hyperthermia or recurrent respiratory infections. It is caused by pathogenic variants of the ectodysplasin A gene (EDA). Most affected males are hemizygous for EDA null mutations that lead to the absence or inactivity of the signalling protein ectodysplasin A1 (EDA1) and, thus, to the full-blown phenotype with inability to perspire and few if any teeth. There are currently no long-term treatment options for XLHED. ER004 represents a first-in-class protein replacement molecule designed for specific, high-affinity binding to the endogenous EDA1 receptor (EDAR). Its proposed mechanism of action is the replacement of missing EDA1 in yet unborn patients with XLHED. Once bound to EDAR, ER004 activates the EDA/NFκB signalling pathway, which triggers the transcription of genes involved in the normal development of multiple tissues. Following preclinical studies, named-patient use cases demonstrated significant potential of ER004 in affected males treated in utero during the late second and third trimesters of pregnancy. In order to confirm these results, we started the EDELIFE trial, a prospective, open-label, genotype-match controlled, multicentre clinical study to investigate the efficacy and safety of intra-amniotic ER004 administration as a prenatal treatment for male subjects with XLHED. This article summarises the rationale, the study protocol, ethical issues of the trial, and potential pitfalls.